PMID- 32007400 OWN - NLM STAT- MEDLINE DCOM- 20201113 LR - 20201113 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 34 IP - 1 DP - 2020 Feb TI - Raynaud's phenomenon. PG - 101474 LID - S1521-6942(19)30170-6 [pii] LID - 10.1016/j.berh.2019.101474 [doi] AB - Raynaud's phenomenon (RP) is common, affecting approximately 5% of the population, and is important to the rheumatologist because it is often the presenting symptom of connective tissue disease, especially of systemic sclerosis (SSc)-spectrum disorders. RP therefore provides a window of opportunity for early diagnosis. When RP is associated with SSc it is particularly challenging to treat. This review begins with a discussion of some of the recent advances in our understanding of the pathogenesis of RP: it is through increased understanding of the complex pathophysiology of RP that we are most likely to develop new therapies. The following questions are then addressed (with three clinical scenarios demonstrating key principles of assessment and management): 1. How can we predict underlying connective tissue disease in the patient presenting with Raynaud's? 2. How can we measure severity of Raynaud's? 3. What are the latest advances in treatment of connective tissue disease-related digital vasculopathy? CI - Copyright © 2019. Published by Elsevier Ltd. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: ariane.herrick@manchester.ac.uk. FAU - Wigley, Fredrick M AU - Wigley FM AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, USA. Electronic address: fwig@jhmi.edu. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20200129 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - *Connective Tissue Diseases/complications/diagnosis/therapy MH - Diagnostic Tests, Routine MH - Early Diagnosis MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Scleroderma, Systemic/complications/diagnosis/therapy OTO - NOTNLM OT - Autoantibodies OT - Capillaroscopy OT - Connective tissue disease OT - Pathogenesis OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Treatment COIS- Declaration of Competing Interest ALH has undertaken consultancy work for Boehringer-Ingelheim, Gesynta and Camurus, has received research funding from Actelion and Gesynta, and speaker's fees from Actelion. FMW has received research support from Corbus, Boehringer-Ingelheim, Cumberland, GlaxoSmithKline, Scleroderma Lung Study III, and Kadmon. EDAT- 2020/02/03 06:00 MHDA- 2020/11/18 06:00 CRDT- 2020/02/03 06:00 PHST- 2020/02/03 06:00 [pubmed] PHST- 2020/11/18 06:00 [medline] PHST- 2020/02/03 06:00 [entrez] AID - S1521-6942(19)30170-6 [pii] AID - 10.1016/j.berh.2019.101474 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2020 Feb;34(1):101474. doi: 10.1016/j.berh.2019.101474. Epub 2020 Jan 29. PMID- 33199324 OWN - NLM STAT- MEDLINE DCOM- 20210512 LR - 20240426 IS - 1473-4893 (Electronic) IS - 1470-2118 (Print) IS - 1470-2118 (Linking) VI - 20 IP - 6 DP - 2020 Nov TI - Raynaud's phenomenon. PG - 580-587 LID - 10.7861/clinmed.2020-0754 [doi] AB - Raynaud's phenomenon (RP) is a common vasospastic condition which affects ~5% of the general population. The majority of individuals have primary RP; however, Raynaud's can also occur secondary to a broad range of underlying medical conditions and drug therapies. RP is a cardinal feature in patients with systemic sclerosis and is often the earliest symptom of the disease. Unlike primary RP, patients with secondary RP can develop persistent digital ischaemia, including ulcers and gangrene. Patients require a comprehensive clinical assessment and investigation, in particular, the detection of autoantibodies and nailfold capillaroscopic abnormalities. Non-pharmacological management is indicated in all patients. There are a wide range of available drug therapies to treat RP, including when complicated by digital ulceration, and surgical intervention is sometimes required. Future research is needed to understand the complex pathogenesis of RP and to measure the impact and severity of RP to develop optimised approaches to management. CI - © Royal College of Physicians 2020. All rights reserved. FAU - Haque, Ashraful AU - Haque A AD - Royal Hallamshire Hospital, Sheffield, UK. FAU - Hughes, Michael AU - Hughes M AD - Royal Hallamshire Hospital, Sheffield, UK michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Journal Article PL - England TA - Clin Med (Lond) JT - Clinical medicine (London, England) JID - 101092853 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Humans MH - *Raynaud Disease/diagnosis/therapy MH - *Scleroderma, Systemic/complications/diagnosis/therapy PMC - PMC7687329 OTO - NOTNLM OT - Raynaud's phenomenon OT - digital ulcers OT - systemic sclerosis EDAT- 2020/11/18 06:00 MHDA- 2021/05/13 06:00 PMCR- 2020/11/01 CRDT- 2020/11/17 06:11 PHST- 2020/11/17 06:11 [entrez] PHST- 2020/11/18 06:00 [pubmed] PHST- 2021/05/13 06:00 [medline] PHST- 2020/11/01 00:00 [pmc-release] AID - S1470-2118(24)03427-4 [pii] AID - clinmedicine [pii] AID - 10.7861/clinmed.2020-0754 [doi] PST - ppublish SO - Clin Med (Lond). 2020 Nov;20(6):580-587. doi: 10.7861/clinmed.2020-0754. PMID- 27509103 OWN - NLM STAT- MEDLINE DCOM- 20160816 LR - 20220408 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 375 IP - 6 DP - 2016 Aug 11 TI - Raynaud's Phenomenon. PG - 556-65 LID - 10.1056/NEJMra1507638 [doi] FAU - Wigley, Fredrick M AU - Wigley FM AD - From the Division of Rheumatology (F.M.W.) and the Department of Anesthesiology and Critical Care Medicine (N.A.F.), Johns Hopkins University School of Medicine, Baltimore. FAU - Flavahan, Nicholas A AU - Flavahan NA AD - From the Division of Rheumatology (F.M.W.) and the Department of Anesthesiology and Critical Care Medicine (N.A.F.), Johns Hopkins University School of Medicine, Baltimore. LA - eng PT - Journal Article PT - Review PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Cold Temperature/adverse effects MH - Diagnosis, Differential MH - Hand/pathology MH - Humans MH - Iloprost/therapeutic use MH - *Raynaud Disease/diagnosis/etiology/physiopathology/therapy MH - Vasoconstriction/physiology MH - Vasodilator Agents/*therapeutic use EDAT- 2016/08/11 06:00 MHDA- 2016/08/17 06:00 CRDT- 2016/08/11 06:00 PHST- 2016/08/11 06:00 [entrez] PHST- 2016/08/11 06:00 [pubmed] PHST- 2016/08/17 06:00 [medline] AID - 10.1056/NEJMra1507638 [doi] PST - ppublish SO - N Engl J Med. 2016 Aug 11;375(6):556-65. doi: 10.1056/NEJMra1507638. PMID- 25673314 OWN - NLM STAT- MEDLINE DCOM- 20170123 LR - 20170123 IS - 1827-1839 (Electronic) IS - 0392-9590 (Linking) VI - 35 IP - 2 DP - 2016 Apr TI - Raynaud's Syndrome: a neglected disease. PG - 117-21 AB - Raynaud's Syndrome is a frequent manifestation of digital ischemia which occurs or is aggravated upon exposure to cold temperatures or emotional distress. Primary Raynaud is a benign disease which predominantly affects younger women and is transient without serious sequelae. In contrast, secondary Raynaud is usually one of the manifestations of systemic disease and is, in addition to symptoms of the basic disease, associated with ischemic lesions. The diagnosis of primary Raynaud is mostly based on the clinical presentation. In secondary Raynaud, additional investigating techniques including imaging investigations and laboratory tests for the detection of underline disease are needed. Treatment is based on lifestyle modification, which includes smoking cessation, avoiding low outside temperatures, avoiding the use of vibrating tools and limiting repeated hand actions. Drug treatment consists of calcium-channel blockers, nitroglycerine ointments, prostacyclins and various new drugs such as endothelin receptor antagonists, phosphodiesterase inhibitors and serotonin receptor antagonists. Most of these drugs are effective in less than 50% of treated patients and do not completely abolish vasospastic attacks, but reduce the severity and frequency of attacks. The prostacyclin derivate iloprost is the most promising drug in the management of secondary Raynaud's disease. Other therapeutic procedures including chemical or surgical sympathectomy are obsolete and without any long-term positive effects. FAU - Poredos, Pavel AU - Poredos P AD - Department of Vascular Diseases, University Medical Centre Ljubljana, Ljubljana, Slovenia - pavel.poredos@kclj.si. FAU - Poredos, Peter AU - Poredos P LA - eng PT - Journal Article PT - Review DEP - 20150212 PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - DCR9Z582X0 (Epoprostenol) RN - G59M7S0WS3 (Nitroglycerin) RN - JED5K35YGL (Iloprost) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Epoprostenol/therapeutic use MH - Humans MH - Iloprost/*therapeutic use MH - Ischemia/*drug therapy MH - Life Style MH - Neglected Diseases MH - Nitroglycerin/therapeutic use MH - Raynaud Disease/*diagnosis/*drug therapy MH - Smoking Cessation MH - Vasodilator Agents/*therapeutic use EDAT- 2015/02/13 06:00 MHDA- 2017/01/24 06:00 CRDT- 2015/02/13 06:00 PHST- 2015/02/13 06:00 [entrez] PHST- 2015/02/13 06:00 [pubmed] PHST- 2017/01/24 06:00 [medline] AID - R34Y9999N00A150013 [pii] PST - ppublish SO - Int Angiol. 2016 Apr;35(2):117-21. Epub 2015 Feb 12. PMID- 31420815 OWN - NLM STAT- MEDLINE DCOM- 20200406 LR - 20210110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 12 DP - 2019 Dec TI - Raynaud's phenomenon-an update on diagnosis, classification and management. PG - 3317-3330 LID - 10.1007/s10067-019-04745-5 [doi] AB - Raynaud's phenomenon (RP) is used to describe a symptom complex caused by digital vascular compromise. RP is a clinical diagnosis. The typically episodic nature of RP has resulted in a reliance upon patient self-report for diagnosis. The term 'primary RP' is generally applied when no underlying pathology can be demonstrated. Whilst 'primary RP' is currently considered a distinct disorder, there is evidence that the term may comprise several entities that include a functional vasospastic disorder, a physiologically appropriate thermoregulatory response, subclinical atherosclerosis and 'cold intolerance'. Optimal management may differ depending on cause. The term 'secondary RP' encompasses a broad range of rheumatological, haematological, endocrinological and vascular pathology. RP can range from relatively benign but intrusive vasospasm, to the progressive obliterative microangiopathy of systemic sclerosis (SSc), in which severe digital ischaemia can threaten tissue viability. SSc has formed the focus of much of the research into RP but, consistent with most medical symptom complexes, the aetiopathogenesis of RP varies greatly dependent on cause. Vasospasm within the digital macro- and microvasculature occurs in SSc, but digital ischaemia is further compounded by a progressive obliterative microangiopathy. Recent work exploring the patient experience of SSc-RP is challenging the 'episodic' paradigm of 'Raynaud's', with important implications for clinical trials utilising diary-based patient-reported outcome instruments for assessing Raynaud's symptoms. This review shall examine the causes, pathogenesis, clinical features, classification and management of RP. A practical approach to the evaluation and management of RP is outlined, highlighting important knowledge gaps and unmet research needs where applicable. Key Points • Raynaud's phenomenon is a symptom complex related to digital vascular compromise secondary to broad-ranging pathology. • Raynaud's phenomenon, as currently classified, likely encompasses a number of aetiopathogenic processes. • Raynaud's phenomenon causes significant disease-related morbidity in autoimmune rheumatic diseases such as systemic sclerosis. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, BA1 1RL, UK. JohnPauling@nhs.net. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. JohnPauling@nhs.net. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Pope, Janet E AU - Pope JE AD - University of Western Ontario, London, ON, Canada. LA - eng PT - Journal Article PT - Review DEP - 20190816 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM CIN - Clin Rheumatol. 2019 Dec;38(12):3689-3690. doi: 10.1007/s10067-019-04785-x. PMID: 31641890 MH - Disease Management MH - Humans MH - Raynaud Disease/classification/diagnosis/*etiology/therapy OTO - NOTNLM OT - Assessment OT - Classification OT - Management OT - Pathogenesis OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2019/08/20 06:00 MHDA- 2020/04/09 06:00 CRDT- 2019/08/18 06:00 PHST- 2019/06/18 00:00 [received] PHST- 2019/08/07 00:00 [accepted] PHST- 2019/08/01 00:00 [revised] PHST- 2019/08/20 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2019/08/18 06:00 [entrez] AID - 10.1007/s10067-019-04745-5 [pii] AID - 10.1007/s10067-019-04745-5 [doi] PST - ppublish SO - Clin Rheumatol. 2019 Dec;38(12):3317-3330. doi: 10.1007/s10067-019-04745-5. Epub 2019 Aug 16. PMID- 31707892 OWN - NLM STAT- MEDLINE DCOM- 20200330 LR - 20200330 IS - 1759-7390 (Electronic) IS - 1750-8460 (Linking) VI - 80 IP - 11 DP - 2019 Nov 2 TI - Raynaud's phenomenon. PG - 658-664 LID - 10.12968/hmed.2019.80.11.658 [doi] AB - Raynaud's phenomenon is a common vasospastic condition which carries a significant burden of pain and hand-related disability (Hughes and Herrick, 2016). The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% (Garner et al, 2015). Raynaud's phenomenon can occur either as a primary ('idiopathic') phenomenon or secondary to a wide range of underlying medical conditions and drug causes. Therefore, hospital-based specialists are frequently involved in the care of patients with Raynaud's phenomenon and need to be aware of associated conditions and prescribed medications for Raynaud's phenomenon. In particular, Raynaud's phenomenon is often the earliest manifestation of an underlying autoimmune connective tissue disease (e.g. systemic sclerosis). A comprehensive clinical assessment is required including performing targeted investigations (e.g. nailfold capillaroscopy and systemic sclerosis-associated autoantibodies). Patient education and lifestyle adaptations is first-line treatment for Raynaud's phenomenon. There is a wide range of pharmacological options including oral and intravenous drug therapies available to treat Raynaud's phenomenon. Surgical intervention is sometimes required for refractory Raynaud's phenomenon and tissue ischaemia. This review describes the clinical manifestations of Raynaud's phenomenon including potential secondary causes and presents an approach to assessment and management. FAU - Devgire, Vikrant AU - Devgire V AD - Core Medical Trainee, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield. FAU - Hughes, Michael AU - Hughes M AD - Consultant Rheumatologist, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF. LA - eng PT - Journal Article PT - Review PL - Singapore TA - Br J Hosp Med (Lond) JT - British journal of hospital medicine (London, England : 2005) JID - 101257109 SB - IM MH - Diagnostic Tests, Routine MH - Fingers/blood supply MH - Humans MH - Ischemia/etiology MH - Medical History Taking/methods MH - Physical Examination/methods MH - Raynaud Disease/diagnosis/*etiology/therapy MH - Scleroderma, Systemic/complications MH - Skin Ulcer/complications MH - Thermography/methods EDAT- 2019/11/12 06:00 MHDA- 2020/03/31 06:00 CRDT- 2019/11/12 06:00 PHST- 2019/11/12 06:00 [entrez] PHST- 2019/11/12 06:00 [pubmed] PHST- 2020/03/31 06:00 [medline] AID - 10.12968/hmed.2019.80.11.658 [doi] PST - ppublish SO - Br J Hosp Med (Lond). 2019 Nov 2;80(11):658-664. doi: 10.12968/hmed.2019.80.11.658. PMID- 38704280 OWN - NLM STAT- MEDLINE DCOM- 20240824 LR - 20240909 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 38 IP - 1 DP - 2024 Mar TI - Painful Raynaud's mimics. PG - 101948 LID - S1521-6942(24)00019-6 [pii] LID - 10.1016/j.berh.2024.101948 [doi] AB - Raynaud's syndrome is a common finding in many autoimmune conditions. Accurately diagnosing Raynaud's, and differentiating it from mimicking conditions, is imperative in rheumatologic diseases. Raynaud's syndrome and Raynaud's mimickers, especially painful Raynaud's mimickers, can prove a diagnostic challenge for the practicing rheumatologist. Painful Raynaud's mimickers can lead to increased patient stress and unnecessary medical work up; Healthcare providers need to be aware of Raynaud's mimickers when evaluating patient concerns of skin color changes and pain. The present narrative review aims to highlight Raynaud's syndrome, important painful mimickers that may be seen, diagnosis, and updated management recommendations. CI - Copyright © 2024 Elsevier Ltd. All rights reserved. FAU - Zahn, Carleigh AU - Zahn C AD - Department of Internal Medicine, Division of Rheumatology, University of Michigan, 300 North Ingalls Building - Rm 7C27, Ann Arbor, MI, 48109, USA. Electronic address: czahn@med.umich.edu. FAU - Puga, Cindy AU - Puga C AD - Cedars Sinai Internal Medicine Residency, 8700 Beverly Blvd, Becker Bldg. B105 A, Los Angeles, CA, 90048, USA. Electronic address: cindy.puga@cshs.org. FAU - Malik, Aroosa AU - Malik A AD - Department of Internal Medicine, Division of Vascular Medicine, University of Michigan, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109, USA. Electronic address: aroosamm@med.umich.edu. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Internal Medicine, Division of Rheumatology, University of Michigan, 300 North Ingalls Building - Rm 7C27, Ann Arbor, MI, 48109, USA. Electronic address: khannad@med.umich.edu. LA - eng PT - Journal Article PT - Review DEP - 20240503 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/physiopathology MH - Diagnosis, Differential MH - Pain/diagnosis OTO - NOTNLM OT - Acrocyanosis OT - Chilblains / pernio OT - Complex regional pain syndrome OT - Erythromelalgia OT - Lupus chilblains OT - Paraneoplastic acral vascular syndrome OT - Raynaud's disease OT - Raynaud's mimickers OT - Raynaud's phenomena OT - Raynaud's syndrome OT - Thoracic outlet syndrome OT - Thromboangiitis obliterans COIS- Declaration of competing interest None of the authors have relevant declarations related to this narrative review. EDAT- 2024/05/05 07:47 MHDA- 2024/08/26 12:39 CRDT- 2024/05/04 21:57 PHST- 2024/03/30 00:00 [received] PHST- 2024/04/04 00:00 [accepted] PHST- 2024/08/26 12:39 [medline] PHST- 2024/05/05 07:47 [pubmed] PHST- 2024/05/04 21:57 [entrez] AID - S1521-6942(24)00019-6 [pii] AID - 10.1016/j.berh.2024.101948 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2024 Mar;38(1):101948. doi: 10.1016/j.berh.2024.101948. Epub 2024 May 3. PMID- 27311222 OWN - NLM STAT- MEDLINE DCOM- 20160802 LR - 20240331 IS - 0026-6620 (Print) IS - 0026-6620 (Linking) VI - 113 IP - 2 DP - 2016 Mar-Apr TI - A Review of Raynaud's Disease. PG - 123-6 AB - Raynaud's phenomenon is a relatively common but often unrecognized clinical syndrome causing characteristic color changes in the digits as a result of vasospasm. This may occur after exposure to a cold environment, emotional stress, or from other physical or medication exposures. Differentiating between primary and secondary Raynaud's is important as secondary Raynaud's can be complicated by digital ischemia and gangrene whereas primary Raynaud's is generally a benign condition. Referral to a rheumatologist is recommended to help evaluate for an underlying rheumatologic condition and to guide future therapy. FAU - Temprano, Katherine K AU - Temprano KK LA - eng PT - Journal Article PT - Review PL - United States TA - Mo Med JT - Missouri medicine JID - 0400744 SB - IM MH - Humans MH - Raynaud Disease/*diagnosis/physiopathology/therapy PMC - PMC6139949 EDAT- 2016/06/18 06:00 MHDA- 2016/08/03 06:00 PMCR- 2016/03/01 CRDT- 2016/06/18 06:00 PHST- 2016/06/18 06:00 [entrez] PHST- 2016/06/18 06:00 [pubmed] PHST- 2016/08/03 06:00 [medline] PHST- 2016/03/01 00:00 [pmc-release] AID - ms113_p0123 [pii] PST - ppublish SO - Mo Med. 2016 Mar-Apr;113(2):123-6. PMID- 17218139 OWN - NLM STAT- MEDLINE DCOM- 20070412 LR - 20260128 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 74 IP - 1 DP - 2007 Jan TI - Raynaud's phenomenon. PG - e1-8 AB - Vascular acrosyndromes constitute a common reason for physician visits. They are associated with connective tissue disease; for example, 90% of patients with scleroderma experience Raynaud's phenomenon. The rheumatologist must strive to establish the diagnosis, to identify a potential underlying cause, and to prescribe effective treatment when the symptoms are incapacitating. Raynaud's phenomenon is the acrosyndrome most commonly encountered by rheumatologists. The diagnosis of Raynaud's phenomenon rests on clinical grounds. Nailfold capillaroscopy and immunological tests are useful chiefly for determining the cause. Calcium-channel antagonists are the treatment of reference for Raynaud's phenomenon. Drugs introduced over the last few years for severe refractory forms include prostacyclin and its derivatives, endothelin receptor antagonists, and phosphodiesterase inhibitors. These drugs were developed as a result of new knowledge on the pathogenesis of Raynaud's phenomenon. Acrocyanosis, which is extremely common, and erythromelalgia are the other main vascular acrosyndromes. FAU - Gayraud, Martine AU - Gayraud M AD - Internal Medicine Department, Institut Mutualiste Motnsouris, 42 boulevard Jourdan, 75014 Paris, France. martine.gayraud@imm.fr LA - eng PT - Journal Article PT - Review DEP - 20061204 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Hematologic Agents) RN - 0 (Nitrates) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Prostaglandins) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Angiotensin Receptor Antagonists MH - Connective Tissue Diseases/complications MH - Endocrine System Diseases/complications MH - Endothelin Receptor Antagonists MH - Female MH - Hematologic Agents/therapeutic use MH - Humans MH - Male MH - Neoplasms/complications MH - Nitrates/therapeutic use MH - Phosphodiesterase Inhibitors/therapeutic use MH - Prostaglandins/therapeutic use MH - Raynaud Disease/*diagnosis/etiology/*therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Vascular Diseases/complications MH - Vasodilator Agents/therapeutic use MH - Wounds and Injuries/complications RF - 55 EDAT- 2007/01/16 09:00 MHDA- 2007/04/14 09:00 CRDT- 2007/01/16 09:00 PHST- 2005/11/02 00:00 [received] PHST- 2006/07/26 00:00 [accepted] PHST- 2007/01/16 09:00 [pubmed] PHST- 2007/04/14 09:00 [medline] PHST- 2007/01/16 09:00 [entrez] AID - S1297-319X(06)00246-6 [pii] AID - 10.1016/j.jbspin.2006.07.002 [doi] PST - ppublish SO - Joint Bone Spine. 2007 Jan;74(1):e1-8. doi: 10.1016/j.jbspin.2006.07.002. Epub 2006 Dec 4. PMID- 27421220 OWN - NLM STAT- MEDLINE DCOM- 20170707 LR - 20220408 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 30 IP - 1 DP - 2016 Feb TI - Raynaud's phenomenon. PG - 112-32 LID - S1521-6942(16)30001-8 [pii] LID - 10.1016/j.berh.2016.04.001 [doi] AB - Raynaud's phenomenon (RP) is a major cause of pain and disability in patients with autoimmune connective tissue diseases (CTDs), particularly systemic sclerosis (SSc). The clinician must perform a comprehensive clinical assessment in patients with RP to differentiate between primary (idiopathic) and secondary RP, in particular (for rheumatologists), secondary to an autoimmune CTD, as both the prognosis and treatment may differ significantly. Key investigations are nailfold capillaroscopy and testing for autoantibodies (in particular, those associated with SSc). Patients with RP and either abnormal nailfold capillaroscopy or an SSc-specific antibody (and especially with both) have a high risk of transitioning to an autoimmune CTD. Both nailfold capillaroscopy and autoantibody specificity may help the clinician in predicting organ-based complications. The management of CTD-associated RP requires a multifaceted approach to treatment, including patient education and conservative ('non-drug') measures. Patients with CTD-associated RP often require pharmacological treatment, which in the first instance is usually a calcium channel blocker, although other agents can be used. There is an increasing tendency to use phosphodiesterase type 5 inhibitors early in the treatment of CTD-associated RP. Oral therapies are commonly associated with side effects (often due to systemic vasodilation) that may result in failure of dose escalation and/or permanent discontinuation. Intravenous prostanoid therapy and surgery (e.g., botulinum toxin injection and digital sympathectomy) can be considered in severe RP. Patients with CTD-associated RP can develop a number of ischaemic digital complications (primarily ulcers and critical ischaemia), which may be associated with significant tissue loss. Future research is required to increase the understanding of the pathogenesis and natural history of RP (to drive therapeutic advances), and to explore/develop drug therapies, including those that target the mechanisms mediating cold-induced vasoconstriction, and locally acting therapies free of systemic side effects. CI - Copyright © 2016. Published by Elsevier Ltd. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: ariane.herrick@manchester.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20160511 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/immunology MH - Connective Tissue Diseases/*complications MH - Humans MH - Microscopic Angioscopy MH - Prognosis MH - Raynaud Disease/diagnosis/*etiology MH - Scleroderma, Systemic/*complications MH - Sensitivity and Specificity OTO - NOTNLM OT - Connective tissue disease OT - Digital ischaemia OT - Raynaud's phenomenon OT - Scleroderma OT - Systemic sclerosis OT - Treatment EDAT- 2016/07/17 06:00 MHDA- 2017/07/08 06:00 CRDT- 2016/07/17 06:00 PHST- 2016/07/17 06:00 [entrez] PHST- 2016/07/17 06:00 [pubmed] PHST- 2017/07/08 06:00 [medline] AID - S1521-6942(16)30001-8 [pii] AID - 10.1016/j.berh.2016.04.001 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2016 Feb;30(1):112-32. doi: 10.1016/j.berh.2016.04.001. Epub 2016 May 11. PMID- 38678551 OWN - NLM STAT- MEDLINE DCOM- 20240428 LR - 20250612 IS - 1788-6120 (Electronic) IS - 0030-6002 (Linking) VI - 165 IP - 17 DP - 2024 Apr 28 TI - [Raynaud's syndrome, 2024]. PG - 643-651 LID - 10.1556/650.2024.33026 [doi] AB - In this article, we review the latest findings of Raynaud’s syndrome from the last 13 years after our previous paper published in 2011. We describe the main characteristics, the available diagnostic and therapeutic options of Raynaud’s syndrome. In this review, we place particular emphasis on highlighting the open questions and unclear points of the topic. We discuss in great detail the available research options hoping to encourage future research to further understand Raynaud’s syndrome. Orv Hetil. 2024; 165(17): 643–651. FAU - Fábián, Balázs AU - Fábián B AD - 1 Debreceni Egyetem, Általános Orvostudományi Kar, Magatartástudományi Intézet Debrecen, Móricz Zs. krt. 22., 4032 Magyarország. FAU - Csiki, Zoltán AU - Csiki Z AD - 1 Debreceni Egyetem, Általános Orvostudományi Kar, Magatartástudományi Intézet Debrecen, Móricz Zs. krt. 22., 4032 Magyarország. LA - hun PT - Journal Article PT - Review TT - Raynaud-szindróma, 2024. DEP - 20240428 PL - Hungary TA - Orv Hetil JT - Orvosi hetilap JID - 0376412 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis OTO - NOTNLM OT - Raynaud-szindróma OT - Raynaud’s syndrome OT - diagnosis OT - diagnózis OT - terápia OT - therapy EDAT- 2024/04/28 21:03 MHDA- 2024/04/28 21:04 CRDT- 2024/04/28 12:12 PHST- 2024/02/01 00:00 [received] PHST- 2024/02/29 00:00 [accepted] PHST- 2024/04/28 21:04 [medline] PHST- 2024/04/28 21:03 [pubmed] PHST- 2024/04/28 12:12 [entrez] AID - 10.1556/650.2024.33026 [doi] PST - epublish SO - Orv Hetil. 2024 Apr 28;165(17):643-651. doi: 10.1556/650.2024.33026. Print 2024 Apr 28. PMID- 28895508 OWN - NLM STAT- MEDLINE DCOM- 20180116 LR - 20180116 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 46 IP - 6 DP - 2017 Oct TI - ESVM guidelines - the diagnosis and management of Raynaud's phenomenon. PG - 413-423 LID - 10.1024/0301-1526/a000661 [doi] AB - Regarding the clinical diagnosis of Raynaud's phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud's phenomenon. This consensus document starts with a clarification about the definition and terminology of Raynaud's phenomenon and covers the differential and aetiological diagnoses as well as the symptomatic treatment. FAU - Belch, Jill AU - Belch J AD - 1 University of Dundee School of Medicine, Dundee, United Kingdom. FAU - Carlizza, Anita AU - Carlizza A AD - 2 Azienda Ospedaliera S.Giovanni-Addolorata, Rome, Italy. FAU - Carpentier, Patrick H AU - Carpentier PH AD - 3 Grenoble University Hospital, Grenoble, France. FAU - Constans, Joel AU - Constans J AD - 4 Hopital St Andre, Bordeaux, France. FAU - Khan, Faisel AU - Khan F AD - 1 University of Dundee School of Medicine, Dundee, United Kingdom. FAU - Wautrecht, Jean-Claude AU - Wautrecht JC AD - 5 Cliniques universitaires de Bruxelles, Brussels, Belgium. FAU - Visona, Adriana AU - Visona A AD - 6 Angiology Unit, Azienda ULSS 2, Marca Trevigiana, Treviso, Italy. FAU - Heiss, Christian AU - Heiss C AD - 7 Department of Cardiology, Pulmonology and Vascular Medicine, Düsseldorf, Germany. FAU - Brodeman, Marianne AU - Brodeman M AD - 8 Division of Angiology, Medical University, Graz, Austria. FAU - Pécsvárady, Zsolt AU - Pécsvárady Z AD - 9 Head of 2nd Dept. of Internal Medicine, Vascular Center, Flor Ferenc Teaching Hospital, Kistarcsa, Hungary. FAU - Roztocil, Karel AU - Roztocil K AD - 10 Institute of Clinical and Experimental Medicine, Prague, Czech Republic. FAU - Colgan, Mary-Paula AU - Colgan MP AD - 11 St. James's Hospital and Trinity College, Dublin, Ireland. FAU - Vasic, Dragan AU - Vasic D AD - 12 Clinical Centre of Serbia, Belgrade, Serbia. FAU - Gottsäter, Anders AU - Gottsäter A AD - 13 Department of Vascular Diseases, Skåne University Hospital, Sweden. FAU - Amann-Vesti, Beatrice AU - Amann-Vesti B AD - 14 Clinic for Angiology, University Hospital Zurich, Switzerland. FAU - Chraim, Ali AU - Chraim A AD - 15 Department of Vascular Surgery, Cedrus Vein and Vascular Clinic, Lviv Hospital, Lviv, Ukraine. FAU - Poredoš, Pavel AU - Poredoš P AD - 16 University Medical Centre Ljubljana, Slovenia. FAU - Olinic, Dan-Mircea AU - Olinic DM AD - 17 Medical Clinic no. 1, Iuliu Hatieganu, University of Medicine and Pharmacy, Cluj-Napoca, Romania. FAU - Madaric, Juraj AU - Madaric J AD - 18 National Institute of Heart and Vascular Diseases, Bratislava, Slovakia. FAU - Nikol, Sigrid AU - Nikol S AD - 19 Asklepios Klinik St. Georg, Klinische und Interventionelle Angiologie, Hamburg, Germany. FAU - Herrick, Ariane L AU - Herrick AL AD - 20 Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom. FAU - Sprynger, Muriel AU - Sprynger M AD - 21 Cardiology-Vascular Medicine, Cardiology Department, University Hospital Liège, Liège, France. FAU - Klein-Weigel, Peter AU - Klein-Weigel P AD - 22 Helios Klinik Berlin-Buch, Klinik für Angiologie, Berlin, Germany. FAU - Hafner, Franz AU - Hafner F AD - 23 Division of Angiology, Medical University, Graz, Austria. FAU - Staub, Daniel AU - Staub D AD - 24 Department of Angiology, University Hospital Basel, Switzerland. FAU - Zeman, Zan AU - Zeman Z AD - 25 Department of Clinical Cardiology and Angiology, Hospital Bulovka, Prague, Czech Republic. LA - eng PT - Journal Article PT - Practice Guideline PT - Review DEP - 20170912 PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Consensus MH - Humans MH - Predictive Value of Tests MH - Raynaud Disease/classification/*diagnosis/epidemiology/*therapy MH - Risk Factors MH - Terminology as Topic MH - Treatment Outcome OTO - NOTNLM OT - Raynaud’s OT - hand arm vibration OT - systemic sclerosis OT - vasospasm EDAT- 2017/09/13 06:00 MHDA- 2018/01/18 06:00 CRDT- 2017/09/13 06:00 PHST- 2017/09/13 06:00 [pubmed] PHST- 2018/01/18 06:00 [medline] PHST- 2017/09/13 06:00 [entrez] AID - 10.1024/0301-1526/a000661 [doi] PST - ppublish SO - Vasa. 2017 Oct;46(6):413-423. doi: 10.1024/0301-1526/a000661. Epub 2017 Sep 12. PMID- 27917635 OWN - NLM STAT- MEDLINE DCOM- 20170629 LR - 20181202 IS - 0008-7335 (Print) IS - 0008-7335 (Linking) VI - 155 IP - 6 DP - 2016 Fall TI - [Raynaud's phenomenon]. PG - 310-318 AB - Raynaud's phenomenon (RP) is a very common sign which can usually be seen across all medical specialties. It is characterized by episodic color changes of acral parts of the body (palor, cyanosis, rubor) lasting from a few minutes to hours, which are usually triggered by cold temperature and/or stress. The primary RP occurs alone, without concomitant diseases, is usually benign and has favorable prognosis. Secondary RP occurs in a variety of diseases with a very variable progression and prognosis, mostly unfavorable one due to the development of ischemic tissue necrosis and gangrene. This work provides a comprehensive overview of the history, current knowledge about the epidemiology and pathogenesis and the recommended evaluation and treatment of RP. FAU - Tomčík, Michal AU - Tomčík M LA - cze PT - Journal Article PT - Review TT - Raynaudův fenomén. PL - Czech Republic TA - Cas Lek Cesk JT - Casopis lekaru ceskych JID - 0004743 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Antihypertensive Agents) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Antihypertensive Agents/therapeutic use MH - Humans MH - Physical Examination/*methods MH - Raynaud Disease/*diagnosis/*drug therapy MH - Rheumatic Diseases/diagnosis OTO - NOTNLM OT - Raynaud's phenomenon OT - capillaroscopy treatment. OT - primary OT - secondary EDAT- 2016/12/06 06:00 MHDA- 2017/07/01 06:00 CRDT- 2016/12/06 06:00 PHST- 2016/12/06 06:00 [entrez] PHST- 2016/12/06 06:00 [pubmed] PHST- 2017/07/01 06:00 [medline] AID - 59466 [pii] PST - ppublish SO - Cas Lek Cesk. 2016 Fall;155(6):310-318. PMID- 37698069 OWN - NLM STAT- MEDLINE DCOM- 20240104 LR - 20250724 IS - 2702-3648 (Electronic) IS - 2702-3648 (Linking) VI - 124 IP - 1 DP - 2024 Jan 1 TI - Raynaud's phenomenon. PG - 43-44 LID - 10.1515/jom-2023-0139 [doi] FAU - Humpel, Olivia AU - Humpel O AD - Lake Erie College of Osteopathic Medicine, Bradenton, FL, USA. FAU - Hostoffer, Robert AU - Hostoffer R AD - Allergy/Immunology Associates Inc., Mayfield Heights, OH, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230913 PL - Germany TA - J Osteopath Med JT - Journal of osteopathic medicine JID - 101776472 SB - IM MH - Humans MH - *Raynaud Disease/complications/diagnosis EDAT- 2023/09/12 06:42 MHDA- 2024/01/04 11:44 CRDT- 2023/09/12 05:43 PHST- 2023/06/21 00:00 [received] PHST- 2023/07/25 00:00 [accepted] PHST- 2024/01/04 11:44 [medline] PHST- 2023/09/12 06:42 [pubmed] PHST- 2023/09/12 05:43 [entrez] AID - jom-2023-0139 [pii] AID - 10.1515/jom-2023-0139 [doi] PST - epublish SO - J Osteopath Med. 2023 Sep 13;124(1):43-44. doi: 10.1515/jom-2023-0139. eCollection 2024 Jan 1. PMID- 25322727 OWN - NLM STAT- MEDLINE DCOM- 20180426 LR - 20250728 IS - 1752-8526 (Electronic) VI - 2014 DP - 2014 Oct 14 TI - Raynaud's phenomenon (secondary). LID - 1125 [pii] AB - INTRODUCTION: Raynaud's phenomenon is episodic vasospasm of the peripheral vessels. It presents as episodic colour changes of the digits (sometimes accompanied by pain and paraesthesia), usually in response to cold exposure or stress. The classic triphasic colour change is white (ischaemia), then blue (de-oxygenation), then red (reperfusion). Raynaud's phenomenon can be primary (idiopathic) or secondary to several different conditions and causes. When secondary (e.g., to systemic sclerosis), it can progress to ulceration of the fingers and toes. This review deals with secondary Raynaud's phenomenon. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of surgical interventions in complicated secondary Raynaud's phenomenon? We searched: Medline, Embase, The Cochrane Library, and other important databases up to March 2014 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found two studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review, we present information relating to the effectiveness and safety of the following interventions: botulinum toxin, simple debridement/surgical toilet of ulcers, peripheral sympathectomy (digital, digital plus sympathectomy of the ulnar and/or radial artery, ligation of the ulnar artery), cervical/thoracic sympathectomy, arterial reconstruction (venous graft, arterial graft, balloon angioplasty), and amputation. FAU - Herrick, Ariane AU - Herrick A AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Muir, Lindsay AU - Muir L LA - eng PT - Journal Article PT - Systematic Review DEP - 20141014 PL - England TA - BMJ Clin Evid JT - BMJ clinical evidence JID - 101294314 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - Amputation, Surgical MH - Botulinum Toxins/therapeutic use MH - Debridement MH - Humans MH - Peripheral Nerves/surgery MH - Raynaud Disease/drug therapy/surgery/*therapy MH - Sympathectomy MH - Ulcer/surgery PMC - PMC4200538 EDAT- 2014/10/18 06:00 MHDA- 2018/04/27 06:00 PMCR- 2016/10/14 CRDT- 2014/10/18 06:00 PHST- 2014/10/18 06:00 [entrez] PHST- 2014/10/18 06:00 [pubmed] PHST- 2018/04/27 06:00 [medline] PHST- 2016/10/14 00:00 [pmc-release] AID - 1125 [pii] PST - epublish SO - BMJ Clin Evid. 2014 Oct 14;2014:1125. PMID- 25878215 OWN - NLM STAT- MEDLINE DCOM- 20160303 LR - 20150529 IS - 1477-0377 (Electronic) IS - 1358-863X (Linking) VI - 20 IP - 3 DP - 2015 Jun TI - Raynaud's phenomenon. PG - 269-71 LID - 10.1177/1358863X15579122 [doi] FAU - Ratchford, Elizabeth V AU - Ratchford EV AD - Johns Hopkins Center for Vascular Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA evr@jhmi.edu. FAU - Evans, Natalie S AU - Evans NS AD - Section of Vascular Medicine, Cleveland Clinic, Cleveland, OH, USA. LA - eng PT - Journal Article DEP - 20150415 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - Risk Factors OTO - NOTNLM OT - Raynaud’s phenomenon OT - vasospasm EDAT- 2015/04/17 06:00 MHDA- 2016/03/05 06:00 CRDT- 2015/04/17 06:00 PHST- 2015/04/17 06:00 [entrez] PHST- 2015/04/17 06:00 [pubmed] PHST- 2016/03/05 06:00 [medline] AID - 1358863X15579122 [pii] AID - 10.1177/1358863X15579122 [doi] PST - ppublish SO - Vasc Med. 2015 Jun;20(3):269-71. doi: 10.1177/1358863X15579122. Epub 2015 Apr 15. PMID- 19268319 OWN - NLM STAT- MEDLINE DCOM- 20090917 LR - 20100623 IS - 0025-7753 (Print) IS - 0025-7753 (Linking) VI - 132 IP - 18 DP - 2009 May 16 TI - [Raynaud's phenomenon]. PG - 712-8 LID - 10.1016/j.medcli.2008.11.017 [doi] AB - Raynaud's phenomenon is a frequent reason for seeking of medical attention, since it affects 3-5% of the population. It is characterized by sudden, transient and recurrent episodes of pallor and/or digital cyanosis, after exposure to cold or stressful situations. No known underlying illness is identified in over 80% of cases and consequently these cases are classified as primary Raynaud's phenomenon. Connective tissue diseases, particularly systemic sclerosis, are the main causes of the phenomenon. Once a complete clinical and physical evaluation rule out other causes, a nailfold capillaroscopy and antinuclear antibodies determination are the most useful adjunctive tests. Mild Raynaud's phenomenon can be managed almost exclusively with conservative non-pharmacological lifestyle modifications. However, if a patient develops a severe vascular condition a suitable vasodilator treatment is needed. When critical digital ischemia develops, intravenous treatment with prostaglandin analogues and surgery may be useful. FAU - Tolosa Vilella, Carles AU - Tolosa Vilella C AD - Servicio de Medicina Interna, Hospital de Sabadell, Institut Universitari Parc Taulí, Universidad Autónoma de Barcelona, Barcelona, España. ctolosa@tauli.cat FAU - Simeón Aznar, Carmen Pilar AU - Simeón Aznar CP FAU - Gabarró Julià, Lourdes AU - Gabarró Julià L LA - spa PT - English Abstract PT - Journal Article PT - Review TT - El fenómeno de Raynaud. DEP - 20090306 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM CIN - Med Clin (Barc). 2010 Apr 10;134(10):470; author reply 470-1. doi: 10.1016/j.medcli.2009.06.067. PMID: 20022066 MH - Humans MH - *Raynaud Disease/classification/diagnosis/etiology/therapy RF - 55 EDAT- 2009/03/10 09:00 MHDA- 2009/09/18 06:00 CRDT- 2009/03/10 09:00 PHST- 2008/09/30 00:00 [received] PHST- 2008/11/05 00:00 [accepted] PHST- 2009/03/10 09:00 [entrez] PHST- 2009/03/10 09:00 [pubmed] PHST- 2009/09/18 06:00 [medline] AID - S0025-7753(08)00234-0 [pii] AID - 10.1016/j.medcli.2008.11.017 [doi] PST - ppublish SO - Med Clin (Barc). 2009 May 16;132(18):712-8. doi: 10.1016/j.medcli.2008.11.017. Epub 2009 Mar 6. PMID- 39615000 OWN - NLM STAT- MEDLINE DCOM- 20250113 LR - 20250711 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 44 IP - 1 DP - 2025 Jan TI - A systematic review of botulinum toxin as a treatment for Raynaud's disease secondary to scleroderma. PG - 81-96 LID - 10.1007/s10067-024-07237-3 [doi] AB - Raynaud's phenomenon (RP) is a vasospastic disorder that affects the small blood vessels in the extremities such as the hands, feet, fingers or toes. It is a debilitating condition that can severely impact the patient's quality of life. Botulinum toxin (BTX) has been examined as a treatment option for RP, but its effect has been inconclusive. A systematic review has been conducted to determine the current evidence of BTX as a treatment for RP secondary to scleroderma. Major clinical databases Medline, Embase (via Ovid), the Cochrane Central Library, ClinicalTrials.gov, EU Clinical Trials Register and the ISRCTN registry were systematically searched from its inception to 27 November 2023 for studies describing BTX and RP. Standard mean differences of Quick-DASH scores, visual analogue scale pain (VAS-P) score and Raynaud's condition score (RCS) are reported with BTX treatment with a random-effect model. A total of 890 entries were retrieved. Of these, 19 met the inclusion criteria, and all studies were included for analysis. There was a significant effect (p = 0.03) with Quick-DASH score and VAS-P score (p < 0.00001) but a non-significant effect (p = 0.37) with RCS. BTX is a therapeutic option in the treatment of RP secondary to scleroderma; however, the evidence published so far is not sufficient to credit it as a revolutionary first line treatment. More research is needed to establish dosing, techniques and core outcome measures for BTX in RP. CI - © 2024. The Author(s). FAU - Pang, Calver AU - Pang C AUID- ORCID: 0000-0001-9914-4706 AD - Department of Surgical Biotechnology, Division of Surgery & Interventional Science, Faculty of Medical Sciences, University College London, London, UK. calverpang@doctors.org.uk. AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. calverpang@doctors.org.uk. FAU - Iakovou, Despina AU - Iakovou D AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. AD - West Suffolk NHS Foundation Trust, Suffolk, UK. FAU - Fraser, Danny AU - Fraser D AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. FAU - Leurent, Baptiste AU - Leurent B AD - Department of Statistical Science, University College London, London, UK. FAU - Awad, Laura AU - Awad L AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. FAU - Langridge, Benjamin AU - Langridge B AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. FAU - Butler, Peter AU - Butler P AD - Department of Plastic and Reconstructive Surgery, Royal Free London NHS Foundation Trust, London, UK. LA - eng PT - Journal Article PT - Systematic Review DEP - 20241130 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - EC 3.4.24.69 (Botulinum Toxins) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Humans MH - *Raynaud Disease/drug therapy/etiology MH - *Scleroderma, Systemic/complications MH - *Botulinum Toxins/therapeutic use MH - Treatment Outcome MH - *Botulinum Toxins, Type A/therapeutic use MH - Quality of Life PMC - PMC11729122 OTO - NOTNLM OT - Botulinum toxin OT - Raynaud’s disease OT - Scleroderma OT - Systematic review COIS- Declarations. Disclosures: None. EDAT- 2024/12/01 15:20 MHDA- 2025/01/13 12:36 PMCR- 2024/11/30 CRDT- 2024/11/30 11:14 PHST- 2024/09/11 00:00 [received] PHST- 2024/11/11 00:00 [accepted] PHST- 2024/11/07 00:00 [revised] PHST- 2025/01/13 12:36 [medline] PHST- 2024/12/01 15:20 [pubmed] PHST- 2024/11/30 11:14 [entrez] PHST- 2024/11/30 00:00 [pmc-release] AID - 10.1007/s10067-024-07237-3 [pii] AID - 7237 [pii] AID - 10.1007/s10067-024-07237-3 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Jan;44(1):81-96. doi: 10.1007/s10067-024-07237-3. Epub 2024 Nov 30. PMID- 33412961 OWN - NLM STAT- MEDLINE DCOM- 20220105 LR - 20220105 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 50 IP - 5 DP - 2021 Sep TI - Immunoglobulin G(4)-related disease presenting with Raynaud's phenomenon. PG - 409-410 LID - 10.1080/03009742.2020.1849789 [doi] FAU - Nishioka, H AU - Nishioka H AD - Department of General Internal Medicine, Kobe City Medical Center General Hospital, Kobe, Japan. FAU - Mizuno, Y AU - Mizuno Y AD - Department of General Internal Medicine, Kobe City Medical Center General Hospital, Kobe, Japan. LA - eng PT - Letter DEP - 20210108 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Immunoglobulin G) SB - IM MH - Humans MH - Immunoglobulin G MH - Immunoglobulin G4-Related Disease/*complications MH - *Raynaud Disease/diagnosis/etiology EDAT- 2021/01/09 06:00 MHDA- 2022/01/06 06:00 CRDT- 2021/01/08 05:40 PHST- 2021/01/09 06:00 [pubmed] PHST- 2022/01/06 06:00 [medline] PHST- 2021/01/08 05:40 [entrez] AID - 10.1080/03009742.2020.1849789 [doi] PST - ppublish SO - Scand J Rheumatol. 2021 Sep;50(5):409-410. doi: 10.1080/03009742.2020.1849789. Epub 2021 Jan 8. PMID- 36300739 OWN - NLM STAT- MEDLINE DCOM- 20221028 LR - 20221028 IS - 2458-9446 (Electronic) IS - 1300-0012 (Linking) VI - 34 IP - 4 DP - 2022 Oct TI - Spinal cord stimulator for the treatment of ischemic pain-Burger's disease and Raynaud's disease: A report of two cases and literature review. PG - 316-321 LID - 10.14744/agri.2020.29053 [doi] AB - Ischemic pain is the main symptom of a group of diseases that result in inadequate blood flow to the extremities and ischemia. In this symptomatology, two major diseases are distinguished: Critical vascular disease and Raynaud's phenomenon. Critical vascular disease background of atherosclerosis caused by diabetes mellitus or hypertension. Raynaud phenomenon is divided into primary and secondary form. The primary form is due to vasospasm and there is no underlying cause. Secondary form is associated with underlying connective tissue or rheumatic diseases, peripheral vascular diseases such as thromboangitis obliterans (Burger's disease). Clinical findings in Raynaud's disease are vasomotor changes with cold exposure such as bruising, coldness, painful paresthesias, and ulcers due to chronic ischemia. Clinic presentation in critical ischemic disease is intermittent claudication for earlier stage and resting pain, gangrene, necrosis, and trophic changes were added in advanced stages. The treatment of the Raynaud 's disease in early stage is medical and conservative. In case of advanced stage ischemic vascular disease, medical treatment resistant pain, insufficient response to endovascular treatment, and inoperabl cases, interventions such as sympathectomy and spinal cord stimulation (SCS) can be applicable. SCS reduces vascular resistance through vasodilator mediators and increases blood flow. SCS also suppresses sympathetic vasoconstriction, increases tissue vascularity, reduces tissue damage, provides ulcer healing and pain reduction. In this report, we demonstrated that persistent Raynaud's disease and advanced stage Burger's disease were successfully treated with SCS. FAU - Ertilav, Esra AU - Ertilav E AD - Division of Algology, Department of Neurology, Adnan Menderes University Faculty of Medicine, Aydın, Türkiye. FAU - Aydın, Osman Nuri AU - Aydın ON AD - Division of Algology, Department of Anesthesiology and Reanimation, Adnan Menderes University Faculty of Medicine, Aydın, Türkiye. LA - eng PT - Case Reports PT - Journal Article PT - Review TT - İskemik ağrı-Burger hastalığı ve Raynaud hastalığı tedavisinde spinal kord stimülatörü uygulaması: 2 olgu sunumu ve literatür taraması. PL - Turkey TA - Agri JT - Agri : Agri (Algoloji) Dernegi'nin Yayin organidir = The journal of the Turkish Society of Algology JID - 9426197 RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - *Raynaud Disease/complications/therapy/diagnosis MH - Ischemia/complications/drug therapy MH - Vasodilator Agents MH - Pain/etiology MH - Spinal Cord EDAT- 2022/10/28 06:00 MHDA- 2022/10/29 06:00 CRDT- 2022/10/27 07:33 PHST- 2022/10/27 07:33 [entrez] PHST- 2022/10/28 06:00 [pubmed] PHST- 2022/10/29 06:00 [medline] AID - 10.14744/agri.2020.29053 [doi] PST - ppublish SO - Agri. 2022 Oct;34(4):316-321. doi: 10.14744/agri.2020.29053. PMID- 30737060 OWN - NLM STAT- MEDLINE DCOM- 20190813 LR - 20190813 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 62 DP - 2019 Apr TI - Raynaud's phenomenon and inflammatory bowel disease: The possible role of microcirculation. PG - e16 LID - S0953-6205(19)30045-7 [pii] LID - 10.1016/j.ejim.2019.02.001 [doi] FAU - Bernardino, Vera R AU - Bernardino VR AD - Unidade de Doenças Auto-Imunes, Internal Medicine Department 7.2, Hospital Curry Cabral, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal. Electronic address: verarodriguesb@gmail.com. FAU - Rodrigues, Ana Catarina AU - Rodrigues AC AD - Unidade de Doenças Auto-Imunes, Internal Medicine Department 7.2, Hospital Curry Cabral, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal. FAU - Panarra, António AU - Panarra A AD - Unidade de Doenças Auto-Imunes, Internal Medicine Department 7.2, Hospital Curry Cabral, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal. LA - eng PT - Letter DEP - 20190206 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 MH - Adult MH - Capillaries/pathology MH - Female MH - Humans MH - Inflammatory Bowel Diseases/*complications MH - Male MH - Microcirculation MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/pathology OTO - NOTNLM OT - Crohn's disease OT - Inflammatory bowel disease OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - Ulcerative colitis EDAT- 2019/02/10 06:00 MHDA- 2019/08/14 06:00 CRDT- 2019/02/10 06:00 PHST- 2018/12/09 00:00 [received] PHST- 2019/02/01 00:00 [accepted] PHST- 2019/02/10 06:00 [pubmed] PHST- 2019/08/14 06:00 [medline] PHST- 2019/02/10 06:00 [entrez] AID - S0953-6205(19)30045-7 [pii] AID - 10.1016/j.ejim.2019.02.001 [doi] PST - ppublish SO - Eur J Intern Med. 2019 Apr;62:e16. doi: 10.1016/j.ejim.2019.02.001. Epub 2019 Feb 6. PMID- 23342877 OWN - NLM STAT- MEDLINE DCOM- 20130215 LR - 20150826 IS - 0370-629X (Print) IS - 0370-629X (Linking) VI - 67 IP - 12 DP - 2012 Dec TI - [Raynaud's phenomenon]. PG - 655-9 AB - Raynaud's phenomenon is a vascular acrosyndrome caused by a variety of diseases. There is a distinction between the idiopathic Raynaud's disease, the secondary types and the suspicious idiopathic Raynaud's phenomenon. FAU - Piérard-Franchimont, C AU - Piérard-Franchimont C AD - Unilab Lg, Service de Dermatopathologie, CHU de Liège, Belgique. claudine.franchimont@ulg.ac.be FAU - Piérard, G E AU - Piérard GE FAU - Hermanns-Lê, T AU - Hermanns-Lê T LA - fre PT - English Abstract PT - Journal Article TT - La vignette diagnostique de l'étudiant. Le phénomène de Raynaud. PL - Belgium TA - Rev Med Liege JT - Revue medicale de Liege JID - 0404317 SB - IM MH - Adult MH - Humans MH - Raynaud Disease/classification/*diagnosis/physiopathology EDAT- 2013/01/25 06:00 MHDA- 2013/02/16 06:00 CRDT- 2013/01/25 06:00 PHST- 2013/01/25 06:00 [entrez] PHST- 2013/01/25 06:00 [pubmed] PHST- 2013/02/16 06:00 [medline] PST - ppublish SO - Rev Med Liege. 2012 Dec;67(12):655-9. PMID- 30047433 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20260127 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 36 IP - 4 DP - 2018 Jul-Aug TI - Raynaud's phenomenon: Current concepts. PG - 498-507 LID - S0738-081X(18)30066-X [pii] LID - 10.1016/j.clindermatol.2018.04.007 [doi] AB - Raynaud's phenomenon (RP) is a transient, acral, vasospastic phenomenon that manifests with characteristic color changes. This vasospasm, classically triggered by cold temperatures, may also be driven by shifts in temperature, climate, or emotional state. Primary RP (PRP) is a common condition without severe sequelae. Secondary RP (SRP), which may be driven by vascular, autoimmune, hematologic, or endocrine etiologies, can result in digital ulceration, irreversible ischemia and necrosis, and secondary infection. This review delineates the clinical manifestations of both primary and secondary RP, as well as the current understanding of RP epidemiology and pathogenesis. Proper examination, including nailfold capillary microscopy, and laboratory workup for secondary causes of RP are also discussed. The traditional armamentarium of therapies used for RP, as well as newer medical and surgical options, is also summarized with particular regard to the clinical evidence for their efficacy. CI - Copyright © 2018. Published by Elsevier Inc. FAU - Stringer, Thomas AU - Stringer T AD - The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York, USA. FAU - Femia, Alisa N AU - Femia AN AD - The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York, USA. Electronic address: alisa.femia@nyumc.org. LA - eng PT - Journal Article PT - Review DEP - 20180410 PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Anticoagulants) RN - 0 (Antihypertensive Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Calcium Channel Blockers) RN - 0 (Endothelin Receptor Antagonists) RN - DCR9Z582X0 (Epoprostenol) RN - 0 (Neuromuscular Agents) RN - G59M7S0WS3 (Nitroglycerin) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Administration, Cutaneous MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Anticoagulants/therapeutic use MH - Antihypertensive Agents/therapeutic use MH - Botulinum Toxins, Type A/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Endothelin Receptor Antagonists/therapeutic use MH - Epoprostenol/therapeutic use MH - Humans MH - Hyperthermia, Induced MH - Neuromuscular Agents/therapeutic use MH - Nitroglycerin/administration & dosage MH - Patient Education as Topic MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Raynaud Disease/complications/*diagnosis/epidemiology/*therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Sympathectomy MH - Vasodilator Agents/therapeutic use EDAT- 2018/07/27 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/07/27 06:00 PHST- 2018/07/27 06:00 [entrez] PHST- 2018/07/27 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] AID - S0738-081X(18)30066-X [pii] AID - 10.1016/j.clindermatol.2018.04.007 [doi] PST - ppublish SO - Clin Dermatol. 2018 Jul-Aug;36(4):498-507. doi: 10.1016/j.clindermatol.2018.04.007. Epub 2018 Apr 10. PMID- 30856621 OWN - NLM STAT- MEDLINE DCOM- 20200630 LR - 20200630 IS - 1423-0151 (Electronic) IS - 1011-7571 (Print) IS - 1011-7571 (Linking) VI - 28 IP - 4 DP - 2019 TI - An Atypical Presentation of Raynaud's Disease. PG - 394-396 LID - 10.1159/000499495 [doi] AB - OBJECTIVE: A 57-year-old female with a 33-year history of constant hand discoloration and paronychia had undergone multiple evaluations with a failure to find a diagnosis. She continues to undergo an evolving treatment regimen and diagnostic workup in an effort to find a long-eluded diagnosis. CLINICAL PRESENTATION: She began to develop superficial ulcerations over the proximal phalanx of her fingers, often pruritic and erythematous, with pain and edema. INTERVENTION: She has since been managed with nifedipine and sildenafil and intermittent stellate ganglion blocks. CONCLUSION: Despite still lacking a formal diagnosis, her constellation of symptoms is most likely the result of an atypical presentation of Raynaud's disease. CI - © 2019 The Author(s) Published by S. Karger AG, Basel. FAU - Viswanath, Omar AU - Viswanath O AD - Valley Anesthesiology and Pain Consultants, Phoenix, Arizona, USA, viswanoy@gmail.com. AD - Department of Anesthesiology, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona, USA, viswanoy@gmail.com. AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, Nebraska, USA, viswanoy@gmail.com. FAU - Peck, Jacquelin AU - Peck J AD - Department of Anesthesiology, Mt. Sinai Medical Center of Florida, Miami Beach, Florida, USA. FAU - Gill, Jatinder S AU - Gill JS AD - Department of Anesthesia, Critical Care, and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20190312 PL - Switzerland TA - Med Princ Pract JT - Medical principles and practice : international journal of the Kuwait University, Health Science Centre JID - 8901334 SB - IM MH - Female MH - Fingers MH - Humans MH - Middle Aged MH - Raynaud Disease/*complications/*diagnosis/therapy PMC - PMC6639579 OTO - NOTNLM OT - Chilblain’s disease OT - Fabry’s disease OT - Paronychia OT - Raynaud’s disease OT - Stellate ganglion COIS- The authors have no conflicts of interest to disclose. EDAT- 2019/03/12 06:00 MHDA- 2020/07/01 06:00 PMCR- 2019/03/12 CRDT- 2019/03/12 06:00 PHST- 2018/08/30 00:00 [received] PHST- 2019/03/11 00:00 [accepted] PHST- 2019/03/12 06:00 [pubmed] PHST- 2020/07/01 06:00 [medline] PHST- 2019/03/12 06:00 [entrez] PHST- 2019/03/12 00:00 [pmc-release] AID - 000499495 [pii] AID - mpp-0028-0394 [pii] AID - 10.1159/000499495 [doi] PST - ppublish SO - Med Princ Pract. 2019;28(4):394-396. doi: 10.1159/000499495. Epub 2019 Mar 12. PMID- 38334051 OWN - NLM STAT- MEDLINE DCOM- 20240412 LR - 20251225 IS - 1477-0377 (Electronic) IS - 1358-863X (Print) IS - 1358-863X (Linking) VI - 29 IP - 2 DP - 2024 Apr TI - Advanced Raynaud's disease: A vascular medicine-initiated team-based approach and nationwide cohort analysis. PG - 120-122 LID - 10.1177/1358863X231220609 [doi] FAU - Kaushik, Milan AU - Kaushik M AUID- ORCID: 0000-0002-1860-5978 AD - Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA. AD - Harvard Medical School, Boston, MA, USA. FAU - Beyer, Sebastian E AU - Beyer SE AD - Department of Medicine, Division of Cardiology, Weill Cornell Medical College, New York-Presbyterian Hospital, New York, NY, USA. FAU - Nashel, Jennifer AU - Nashel J AD - Harvard Medical School, Boston, MA, USA. AD - Division of Rheumatology, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Kholdani, Cyrus AU - Kholdani C AD - Harvard Medical School, Boston, MA, USA. AD - Division of Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Dowlatshahi, Arriyan S AU - Dowlatshahi AS AD - Harvard Medical School, Boston, MA, USA. AD - Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Secemsky, Eric A AU - Secemsky EA AUID- ORCID: 0000-0003-3861-3163 AD - Harvard Medical School, Boston, MA, USA. AD - Division of Cardiology, Beth Israel Deaconess Medical Center, Boston, MA, USA. AD - Department of Medicine, Richard A and Susan F Smith Center for Outcomes Research in Cardiology, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Carroll, Brett J AU - Carroll BJ AUID- ORCID: 0000-0002-6704-5663 AD - Harvard Medical School, Boston, MA, USA. AD - Division of Cardiology, Beth Israel Deaconess Medical Center, Boston, MA, USA. AD - Department of Medicine, Richard A and Susan F Smith Center for Outcomes Research in Cardiology, Beth Israel Deaconess Medical Center, Boston, MA, USA. LA - eng GR - K23 HL150290/HL/NHLBI NIH HHS/United States PT - Journal Article DEP - 20240209 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/therapy MH - Cohort Studies MH - *Cardiology PMC - PMC12723403 MID - NIHMS2129935 OTO - NOTNLM OT - Raynaud’s phenomenon OT - multidisciplinary team OT - sympathectomy OT - vascular medicine COIS- Declaration of conflicting interestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Brett Carroll: Bristol Myers Squibb, institutional research support; Koya Medical, scientific advisory board. Eric Secemsky: research grants to BIDMC (Beth Israel Deaconess Medical Center): NIH/NHLBI K23HL150290, Food & Drug Administration, SCAI, BD, Boston Scientific, Cook, Abbott/CSI, Laminate Medical, Medtronic and Philips; consulting/speaking: Abbott/CSI, BD, Boston Scientific, Cook, Cordis, Heartflow, InfraRedx, Medtronic, Philips, RapidAI, Shockwave, and VentureMed. The remaining authors with no disclosures. EDAT- 2024/02/09 06:42 MHDA- 2024/04/12 06:44 PMCR- 2025/12/23 CRDT- 2024/02/09 05:53 PHST- 2024/04/12 06:44 [medline] PHST- 2024/02/09 06:42 [pubmed] PHST- 2024/02/09 05:53 [entrez] PHST- 2025/12/23 00:00 [pmc-release] AID - 10.1177/1358863X231220609 [doi] PST - ppublish SO - Vasc Med. 2024 Apr;29(2):120-122. doi: 10.1177/1358863X231220609. Epub 2024 Feb 9. PMID- 33566754 OWN - NLM STAT- MEDLINE DCOM- 20220222 LR - 20220222 IS - 1477-0377 (Electronic) IS - 1358-863X (Linking) VI - 26 IP - 1 DP - 2021 Feb TI - Raynaud's phenomenon and related vasospastic disorders. PG - 56-70 LID - 10.1177/1358863X20983455 [doi] AB - Raynaud's phenomenon, which is characterized by episodic digital pallor, cyanosis and rubor upon exposure to cold environment or to stress, is relatively common, although the prevalence depends on the climate. Still, it is under-diagnosed, under-treated, and often confused with other conditions. Primary Raynaud's phenomenon (i.e., Raynaud disease) must be distinguished from secondary Raynaud's phenomenon (i.e., Raynaud syndrome) as long-term morbidity and outcomes differ vastly between the two conditions. Additionally, the practitioner must differentiate between Raynaud's phenomenon and related vascular disorders, such as acrocyanosis, pernio, and livedo reticularis. In this article, we review differences between the conditions and suggest an approach to diagnosis and treatment strategy for these disorders. FAU - Choi, Eunjung AU - Choi E AD - Heart and Vascular Center, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA. FAU - Henkin, Stanislav AU - Henkin S AUID- ORCID: 0000-0001-5968-1216 AD - Heart and Vascular Center, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA. LA - eng PT - Journal Article PT - Review PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/epidemiology/therapy OTO - NOTNLM OT - Raynaud’s OT - acrocyanosis OT - livedo reticularis OT - pernio OT - vasospasm EDAT- 2021/02/11 06:00 MHDA- 2022/02/23 06:00 CRDT- 2021/02/10 17:11 PHST- 2021/02/11 06:00 [pubmed] PHST- 2022/02/23 06:00 [medline] PHST- 2021/02/10 17:11 [entrez] AID - 10.1177/1358863X20983455 [doi] PST - ppublish SO - Vasc Med. 2021 Feb;26(1):56-70. doi: 10.1177/1358863X20983455. PMID- 40876770 OWN - NLM STAT- MEDLINE DCOM- 20251119 LR - 20251119 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 370 IP - 6 DP - 2025 Dec TI - Cardiovascular disease and inpatient complications in Raynaud's syndrome: Propensity score analysis. PG - 546-551 LID - S0002-9629(25)01177-2 [pii] LID - 10.1016/j.amjms.2025.08.020 [doi] AB - BACKGROUND: Raynaud's Syndrome (RS) is a vasospastic disorder characterized by intermittent episodes of arterial vasospasm of the extremities. The relationship between RS and cardiovascular outcomes is not well studied, particularly among hospitalized patients. This study aims to examine the relationship between RS and clinical outcomes among hospitalized patients. METHODS: The National Inpatient Sample (NIS) database was used to identify patients with RS using the ICD-10 code (RS: I73.0). Propensity score matching was performed to balance baseline demographic and clinical characteristics between patients with and without RS. CVD, rheumatological diseases, and inpatient outcomes were compared between the two groups before and after matching. RESULTS: RS patients had significantly higher rates of ischemic heart disease (22.9 % vs. 19.5 %; p < 0.01), heart failure (19.2 % vs. 14.5 %; p < 0.01), and pulmonary arterial hypertension (12.4 % vs. 4.0 %; p < 0.01). Rheumatological co-morbidities, including systemic sclerosis (14.6 % vs. 0.2 %; p < 0.01) and systemic lupus erythematosus (14.2 % vs. 0.8 %; p < 0.01), were more prevalent in RS patients. Inpatient complications, particularly acute decompensated heart failure (5.9 % vs. 4.5 %; p < 0.01), were more common in RS patients. CONCLUSIONS: Patients with RS had a higher prevalence of cardiovascular and rheumatological diseases and were associated with inpatient complications. These findings emphasize the importance of comprehensive cardiovascular and rheumatological disease risk assessment and inpatient monitoring in RS patients to optimize outcomes. CI - Copyright © 2025. Published by Elsevier Inc. FAU - Alzahrani, Talal AU - Alzahrani T AD - Department of Medicine, College of Medicine, Taibah University. Electronic address: tzahrani@taibahu.edu.sa. LA - eng PT - Journal Article DEP - 20250826 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Humans MH - *Raynaud Disease/complications/epidemiology MH - Female MH - Male MH - Middle Aged MH - Propensity Score MH - *Cardiovascular Diseases/epidemiology/etiology MH - Adult MH - Aged MH - Inpatients MH - Hospitalization MH - Retrospective Studies OTO - NOTNLM OT - Cardiovascular disease OT - Inpatient outcomes OT - Raynaud’s syndrome OT - Rheumatological disease COIS- Declaration of competing interest There are no conflicts of interest. EDAT- 2025/08/29 00:45 MHDA- 2025/11/20 00:29 CRDT- 2025/08/28 19:17 PHST- 2025/02/10 00:00 [received] PHST- 2025/08/22 00:00 [revised] PHST- 2025/08/25 00:00 [accepted] PHST- 2025/11/20 00:29 [medline] PHST- 2025/08/29 00:45 [pubmed] PHST- 2025/08/28 19:17 [entrez] AID - S0002-9629(25)01177-2 [pii] AID - 10.1016/j.amjms.2025.08.020 [doi] PST - ppublish SO - Am J Med Sci. 2025 Dec;370(6):546-551. doi: 10.1016/j.amjms.2025.08.020. Epub 2025 Aug 26. PMID- 18692160 OWN - NLM STAT- MEDLINE DCOM- 20090630 LR - 20081020 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 8 IP - 1 DP - 2008 Oct TI - Treatment of Raynaud's phenomenon. PG - 62-8 LID - 10.1016/j.autrev.2008.07.002 [doi] AB - Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia, and, rarely, ulceration of the fingers and toes. Primary or idiopathic Raynaud's phenomenon (Raynaud's disease) occurs without an underlying disease. Secondary Raynaud's phenomenon (Raynaud's syndrome) occurs in association with an underlying disease. Initially conservative, non-pharmacologic approach is important for these patients, although pharmacologic therapy may ultimately be necessary. Advances in vascular physiology have showed the role of the endothelium as well as endothelium-independent mechanisms in the altered vasoregulation of Raynaud's phenomenon. This has opened promising therapeutic avenues, and it is likely that therapies targeted towards specific pathophysiologic steps become available in the near future. FAU - García-Carrasco, Mario AU - García-Carrasco M AD - Systemic Autoimmune Diseases Research Unit, HGR #36, Instituto Mexicano del Seguro Social, Puebla, Mexico. FAU - Jiménez-Hernández, Mario AU - Jiménez-Hernández M FAU - Escárcega, Ricardo O AU - Escárcega RO FAU - Mendoza-Pinto, Claudia AU - Mendoza-Pinto C FAU - Pardo-Santos, Rodrigo AU - Pardo-Santos R FAU - Levy, Roger AU - Levy R FAU - Maldonado, Claudio Galarza AU - Maldonado CG FAU - Chávez, Gonzalo Pérez AU - Chávez GP FAU - Cervera, Ricard AU - Cervera R LA - eng PT - Journal Article PT - Review DEP - 20080808 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) SB - IM MH - Anti-Inflammatory Agents, Non-Steroidal/*therapeutic use MH - Calcium Channel Blockers/*therapeutic use MH - Clinical Trials as Topic MH - Cold Temperature/adverse effects MH - Humans MH - Life Style MH - Raynaud Disease/*therapy MH - Sympathectomy, Chemical MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use RF - 40 EDAT- 2008/08/12 09:00 MHDA- 2009/07/01 09:00 CRDT- 2008/08/12 09:00 PHST- 2008/07/02 00:00 [received] PHST- 2008/07/12 00:00 [accepted] PHST- 2008/08/12 09:00 [pubmed] PHST- 2009/07/01 09:00 [medline] PHST- 2008/08/12 09:00 [entrez] AID - S1568-9972(08)00114-6 [pii] AID - 10.1016/j.autrev.2008.07.002 [doi] PST - ppublish SO - Autoimmun Rev. 2008 Oct;8(1):62-8. doi: 10.1016/j.autrev.2008.07.002. Epub 2008 Aug 8. PMID- 36331170 OWN - NLM STAT- MEDLINE DCOM- 20221107 LR - 20221130 IS - 1603-6824 (Electronic) IS - 0041-5782 (Linking) VI - 184 IP - 43 DP - 2022 Oct 24 TI - [Not Available]. LID - V04220293 [pii] AB - Raynaud's phenomenon (RP) is a vasospastic condition of the extremities in response to cold or stress, affecting approximately 3% to 5% of the population. While most patients have primary RP, the condition can also occur secondary to a variety of underlying medical conditions. RP may be the presenting symptom of connective tissue diseases, especially systemic sclerosis, and RS may therefore provide an opportunity for early diagnosis and treatment. This review addresses the causes, clinical features, diagnostic workup, and treatment possibilities of RS. FAU - Næser, Esben Uggerby AU - Næser EU AD - Diagnostisk Center, Regionshospitalet Silkeborg. AD - Led- og Bindevævssygdomme, Aarhus Universitetshospital. FAU - Søndergaard, Klaus AU - Søndergaard K AD - Led- og Bindevævssygdomme, Aarhus Universitetshospital. LA - dan PT - English Abstract PT - Journal Article PT - Review TT - Raynaud's phenomenon. PL - Denmark TA - Ugeskr Laeger JT - Ugeskrift for laeger JID - 0141730 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Connective Tissue Diseases/complications MH - *Scleroderma, Systemic/complications/diagnosis/therapy MH - Early Diagnosis EDAT- 2022/11/05 06:00 MHDA- 2022/11/08 06:00 CRDT- 2022/11/04 08:33 PHST- 2022/11/04 08:33 [entrez] PHST- 2022/11/05 06:00 [pubmed] PHST- 2022/11/08 06:00 [medline] AID - V04220293 [pii] PST - ppublish SO - Ugeskr Laeger. 2022 Oct 24;184(43):V04220293. PMID- 34864903 OWN - NLM STAT- MEDLINE DCOM- 20220708 LR - 20220711 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 7 DP - 2022 Jul 6 TI - Comment on: Causes of Raynaud's phenomenon and the predictive laboratory and capillaroscopy features for the evolution to a definite connective tissue disease. PG - e188-e189 LID - 10.1093/rheumatology/keab884 [doi] FAU - Yin, Hanlin AU - Yin H AUID- ORCID: 0000-0003-0706-3998 AD - Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Lu, Liangjing AU - Lu L AUID- ORCID: 0000-0001-9116-6038 AD - Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. LA - eng PT - Comment PT - Editorial PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2022 May 5;61(5):1975-1985. doi: 10.1093/rheumatology/keab668. PMID: 34463711 CIN - Rheumatology (Oxford). 2022 Jul 6;61(7):e190-e191. doi: 10.1093/rheumatology/keab885. PMID: 34864926 MH - *Connective Tissue Diseases/complications MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/etiology EDAT- 2021/12/06 06:00 MHDA- 2022/07/09 06:00 CRDT- 2021/12/05 21:37 PHST- 2021/10/02 00:00 [received] PHST- 2021/10/06 00:00 [accepted] PHST- 2021/12/06 06:00 [pubmed] PHST- 2022/07/09 06:00 [medline] PHST- 2021/12/05 21:37 [entrez] AID - 6448780 [pii] AID - 10.1093/rheumatology/keab884 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Jul 6;61(7):e188-e189. doi: 10.1093/rheumatology/keab884. PMID- 31721965 OWN - NLM STAT- MEDLINE DCOM- 20200103 LR - 20220411 IS - 1806-9282 (Electronic) IS - 0104-4230 (Linking) VI - 65 IP - 10 DP - 2019 TI - Raynaud's phenomenon in the occupational context. PG - 1314-1320 LID - S0104-42302019001001314 [pii] LID - 10.1590/1806-9282.65.10.1314 [doi] AB - OBJECTIVE: To review articles that evaluated the prevalence of Raynaud's phenomenon of occupational origin. METHODS: The search for articles was carried out in the Medline (via PubMed), Embase, Web of Science, Scientific Electronic Library Online (SciELO), and Latin America and Caribbean Health Sciences Literature (Lilacs) databases. RESULTS: 64 articles were obtained from the electronic search; 18 articles met the eligibility criteria. All studies discussed the exposure to vibrations in the upper limbs. In 6 of them, the thermal issue was directly or indirectly addressed. No studies have addressed exposure to vinyl chloride. CONCLUSIO: In general, a higher prevalence of Raynaud's phenomenon was found among vibratory tool operators compared to non-exposed workers, with an increase in the number of cases the higher the level of vibration and the time of exposure. Cold is a triggering and aggravating factor of the Raynaud phenomenon and seems to play an important role in the emergence of vascular manifestations of the hand-arm vibration syndrome. FAU - Cordeiro, Rafael Alves AU - Cordeiro RA AUID- ORCID: 0000-0002-5695-9136 AD - . Divisão de Medicina Ocupacional, Instituto Oscar Freire, Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, SP, Brasil. FAU - Andrade, Rogério Muniz de AU - Andrade RM AUID- ORCID: 0000-0003-4013-3812 AD - . Divisão de Medicina Ocupacional, Instituto Oscar Freire, Faculdade de Medicina da Universidade de São Paulo (USP), São Paulo, SP, Brasil. LA - eng PT - Journal Article PT - Review DEP - 20191107 PL - Brazil TA - Rev Assoc Med Bras (1992) JT - Revista da Associacao Medica Brasileira (1992) JID - 9308586 RN - WD06X94M2D (Vinyl Chloride) SB - IM MH - Cold Temperature/adverse effects MH - Hand-Arm Vibration Syndrome/complications MH - Humans MH - Occupational Diseases/epidemiology/*etiology MH - Occupational Exposure/*adverse effects MH - Prevalence MH - Raynaud Disease/epidemiology/*etiology MH - Risk Factors MH - Vinyl Chloride/adverse effects EDAT- 2019/11/14 06:00 MHDA- 2020/01/04 06:00 CRDT- 2019/11/14 06:00 PHST- 2019/03/12 00:00 [received] PHST- 2019/03/31 00:00 [accepted] PHST- 2019/11/14 06:00 [entrez] PHST- 2019/11/14 06:00 [pubmed] PHST- 2020/01/04 06:00 [medline] AID - S0104-42302019001001314 [pii] AID - 10.1590/1806-9282.65.10.1314 [doi] PST - epublish SO - Rev Assoc Med Bras (1992). 2019 Nov 7;65(10):1314-1320. doi: 10.1590/1806-9282.65.10.1314. eCollection 2019. PMID- 22641907 OWN - NLM STAT- MEDLINE DCOM- 20120627 LR - 20120530 IS - 0035-2640 (Print) IS - 0035-2640 (Linking) VI - 62 IP - 4 DP - 2012 Apr TI - [Raynaud's phenomenon]. PG - 569-75 FAU - Marie, Isabelle AU - Marie I AD - Département de médecine interne, CHU de Rouen, 76031 Rouen Cedex, France. isabelle.marie@chu-rouen.fr LA - fre PT - Journal Article PT - Review TT - Phénomène de Raynaud. Orientation diagnostique. PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Humans MH - Medical History Taking MH - Raynaud Disease/*diagnosis/etiology MH - Risk Factors MH - Vascular Diseases/complications/diagnosis EDAT- 2012/05/31 06:00 MHDA- 2012/06/28 06:00 CRDT- 2012/05/31 06:00 PHST- 2012/05/31 06:00 [entrez] PHST- 2012/05/31 06:00 [pubmed] PHST- 2012/06/28 06:00 [medline] PST - ppublish SO - Rev Prat. 2012 Apr;62(4):569-75. PMID- 32881655 OWN - NLM STAT- MEDLINE DCOM- 20210527 LR - 20210527 IS - 1875-533X (Electronic) IS - 0929-8673 (Linking) VI - 28 IP - 12 DP - 2021 TI - Repurposing Cilostazol for Raynaud's Phenomenon. PG - 2409-2417 LID - 10.2174/0929867327666200903114154 [doi] AB - Raynaud 's Phenomenon (RP) results from exaggerated cold-induced vasoconstriction. RP patients suffer from vasospastic attacks and compromised digital blood perfusion leading to a triple color change at the level the fingers. Severe RP may cause ulcers and threaten tissue viability. Many drugs have been used to alleviate the symptoms of RP. These include calcium-channel blockers, cGMP-specific phosphodiesterase type 5 inhibitors, prostacyclin analogs, and angiotensin receptor blockers. Despite their variety, these drugs do not treat RP but rather alleviate its symptoms. To date, no drug for RP has been yet approved by the U.S Food and Drugs Administration. Cilostazol is a selective inhibitor of phosphodiesterase-III, originally prescribed to treat intermittent claudication. Owing to its antiplatelet and vasodilating properties, cilostazol is being repurposed as a potential drug for RP. This review focuses on the different lines of action of cilostazol serving to enhance blood perfusion in RP patients. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - El-Hachem, Nehme AU - El-Hachem N AD - Laboratory of Medical Genetics, Institute of Experimental Cardiology, National Medical Research Center of Cardiology, Beirut, Lebanon. FAU - Fardoun, Manal M AU - Fardoun MM AD - Department of Biology, American University of Beirut, Beirut, Lebanon. FAU - Slika, Hasan AU - Slika H AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - Baydoun, Elias AU - Baydoun E AD - Department of Biology, American University of Beirut, Beirut, Lebanon. FAU - Eid, Ali H AU - Eid AH AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Med Chem JT - Current medicinal chemistry JID - 9440157 RN - 0 (Calcium Channel Blockers) RN - N7Z035406B (Cilostazol) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Cilostazol/therapeutic use MH - *Drug Repositioning MH - Fingers MH - Humans MH - *Raynaud Disease/drug therapy OTO - NOTNLM OT - Cardiovascular disease OT - Cold-induced vasoconstriction OT - Raynaud's phenomenon OT - cilostazol OT - digital ischemia OT - drug repurposing EDAT- 2020/09/04 06:00 MHDA- 2021/05/28 06:00 CRDT- 2020/09/04 06:00 PHST- 2020/04/30 00:00 [received] PHST- 2020/07/16 00:00 [revised] PHST- 2020/07/21 00:00 [accepted] PHST- 2020/09/04 06:00 [pubmed] PHST- 2021/05/28 06:00 [medline] PHST- 2020/09/04 06:00 [entrez] AID - CMC-EPUB-109647 [pii] AID - 10.2174/0929867327666200903114154 [doi] PST - ppublish SO - Curr Med Chem. 2021;28(12):2409-2417. doi: 10.2174/0929867327666200903114154. PMID- 22248476 OWN - NLM STAT- MEDLINE DCOM- 20121119 LR - 20131121 IS - 1538-3687 (Electronic) IS - 0003-9942 (Linking) VI - 69 IP - 5 DP - 2012 May TI - Syncope and Raynaud's disease. PG - 608-13 AB - OBJECTIVE: To investigate an association between syncope and Raynaud's disease (RD), its clinical features, and the effect of treatment with nifedipine. DESIGN: One-year prospective study of new outpatients after 3 initial clinical observations. SETTING: Neurology clinics at Chelsea and Westminster, Royal Free, Barnet, and Edgware Hospitals. PATIENTS: Ten women and 1 man. The group had a mean (SD) age of 33 (17) years. Mean (SD) follow-up was 24 (36) months. INTERVENTION: Treatment with nifedipine. OUTCOME MEASURES: Observed vs expected frequency of syncope in RD, temporal relation between syncope and Raynaud's phenomenon, clinical features, and response to nifedipine treatment. RESULTS: Eight additional patients with syncope and RD were identified from 603 new patients (1.3%); we had expected only 1 patient to be identified with syncope and RD (P=.003). A chance association between RD and migraine with recurrent syncope was unlikely (P=.01). The prevalence of RD in patients with syncope with migraine was higher than expected (P=.03), but that of migraine in patients with RD was not (P=.2). All 11 patients had 5 or more syncopal episodes for a median of 2 years (range, 0.1-62 years). Three patients had previous diagnoses of nonepileptic attacks. Syncope was preceded by or contemporaneous with Raynaud's phenomenon in 10 patients (P=.02). Nine patients had migraine; headache was contemporaneous with syncope in 4 patients as expected by chance (P=1.0). In all patients, syncope was preceded by brainstem or vertebrobasilar symptoms, and it ceased after treatment with nifedipine. Raynaud's disease and migraine improved less. CONCLUSIONS: The association of syncope to RD was unrelated to chance or migraine. The temporal relation between syncope and Raynaud's phenomenon but not headache was statistically significant. Treatment with nifedipine stopped recurrent syncope in all patients. Syncope related to RD may result from brainstem ischemia. Unexplained recurrent syncope should prompt screening for RD. FAU - Guiloff, Roberto J AU - Guiloff RJ AD - West London Neurosciences Centre, Charing Cross Hospital, Imperial College Healthcare National Health Service Trust, London, England. r.guiloff@imperial.ac.uk FAU - Rajakulendran, Sanjeev AU - Rajakulendran S FAU - Angus-Leppan, Heather AU - Angus-Leppan H LA - eng PT - Journal Article PL - United States TA - Arch Neurol JT - Archives of neurology JID - 0372436 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Female MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Nifedipine/*therapeutic use MH - Raynaud Disease/*complications/*drug therapy MH - Syncope/*complications/*drug therapy MH - Vasodilator Agents/*therapeutic use MH - Young Adult EDAT- 2012/01/18 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/01/18 06:00 PHST- 2012/01/18 06:00 [entrez] PHST- 2012/01/18 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - archneurol.2011.1168 [pii] AID - 10.1001/archneurol.2011.1168 [doi] PST - ppublish SO - Arch Neurol. 2012 May;69(5):608-13. doi: 10.1001/archneurol.2011.1168. PMID- 21983952 OWN - NLM STAT- MEDLINE DCOM- 20111220 LR - 20211020 IS - 1995-1892 (Print) IS - 1680-0745 (Electronic) IS - 1015-9657 (Linking) VI - 22 IP - 5 DP - 2011 Sep-Oct TI - Raynaud's phenomenon. PG - 233 FAU - Solomons, H D AU - Solomons HD LA - eng PT - Letter PL - South Africa TA - Cardiovasc J Afr JT - Cardiovascular journal of Africa JID - 101313864 RN - 0 (Vasodilator Agents) SB - IM MH - Fingers/*blood supply MH - Humans MH - *Raynaud Disease/diagnosis/drug therapy/etiology/physiopathology MH - Risk Factors MH - *Vasoconstriction MH - Vasodilator Agents/therapeutic use PMC - PMC3721961 EDAT- 2011/10/11 06:00 MHDA- 2011/12/21 06:00 PMCR- 2011/10/01 CRDT- 2011/10/11 06:00 PHST- 2011/10/11 06:00 [entrez] PHST- 2011/10/11 06:00 [pubmed] PHST- 2011/12/21 06:00 [medline] PHST- 2011/10/01 00:00 [pmc-release] PST - ppublish SO - Cardiovasc J Afr. 2011 Sep-Oct;22(5):233. PMID- 32149407 OWN - NLM STAT- MEDLINE DCOM- 20210708 LR - 20210708 IS - 1097-4679 (Electronic) IS - 0021-9762 (Linking) VI - 76 IP - 9 DP - 2020 Sep TI - Alexithymia and emotion regulation in patients with Raynaud's disease. PG - 1696-1704 LID - 10.1002/jclp.22947 [doi] AB - OBJECTIVES: Besides cold, emotional distress is the most important trigger of Raynaud's disease (RD) attacks, although little is known about the factors that contribute to the effectiveness of coping with emotional distress. The aim of the present study was to explore alexithymia and emotion regulation and their relationship with depression and quality of life among patients with RD. METHODS: Total of 110 patients (mean age 53.65; 96 women) with RD completed self-report measures. RESULTS: Alexithymia was associated with adverse emotional regulation skills, depression, and quality of life impairment. Detailed analysis revealed that there are significant differences between alexithymic and nonalexithymic patients in emotion regulation. CONCLUSION: Alexithymia is a significant factor affecting health status in RD since it plays a significant role in emotion regulation. A multidisciplinary approach is essential to improve treatment outcome by identifying patients with high alexithymia, and to improve their emotional regulation skills. CI - © 2020 Wiley Periodicals, Inc. FAU - Fábián, Balázs AU - Fábián B AUID- ORCID: 0000-0002-1073-568X AD - Faculty of Public Health, Institute of Behavioural Sciences, University of Debrecen, Debrecen, Hungary. AD - Doctoral School of Health Sciences, University of Debrecen, Debrecen, Hungary. FAU - Csiki, Zoltán AU - Csiki Z AD - Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. FAU - Bugán, Antal AU - Bugán A AD - Faculty of Public Health, Institute of Behavioural Sciences, University of Debrecen, Debrecen, Hungary. LA - eng PT - Journal Article DEP - 20200309 PL - United States TA - J Clin Psychol JT - Journal of clinical psychology JID - 0217132 SB - IM MH - Adult MH - Affective Symptoms/*psychology MH - Aged MH - Depression/psychology MH - *Emotional Regulation MH - Female MH - Humans MH - Male MH - Middle Aged MH - Quality of Life/psychology MH - Raynaud Disease/*psychology/therapy MH - Self Report OTO - NOTNLM OT - Raynaud's disease OT - alexithymia OT - depression OT - emotion regulation OT - quality of life EDAT- 2020/03/10 06:00 MHDA- 2021/07/09 06:00 CRDT- 2020/03/10 06:00 PHST- 2020/03/10 06:00 [pubmed] PHST- 2021/07/09 06:00 [medline] PHST- 2020/03/10 06:00 [entrez] AID - 10.1002/jclp.22947 [doi] PST - ppublish SO - J Clin Psychol. 2020 Sep;76(9):1696-1704. doi: 10.1002/jclp.22947. Epub 2020 Mar 9. PMID- 34864926 OWN - NLM STAT- MEDLINE DCOM- 20220708 LR - 20220711 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 7 DP - 2022 Jul 6 TI - Comment on: Causes of Raynaud's phenomenon and the predictive laboratory and capillaroscopy features for the evolution to a definite connective tissue disease: reply. PG - e190-e191 LID - 10.1093/rheumatology/keab885 [doi] FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AUID- ORCID: 0000-0003-0522-7186 AD - Division of Rheumatology, Department of Internal Medicine. FAU - Ajri, Mehrnoush AU - Ajri M AUID- ORCID: 0000-0001-9066-3155 AD - Department of Internal Medicine. FAU - Hamidi, Sahand AU - Hamidi S AD - Department of Cardiology, Shiraz University of Medical Sciences, Shiraz, Iran. LA - eng PT - Comment PT - Editorial PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2022 May 5;61(5):1975-1985. doi: 10.1093/rheumatology/keab668. PMID: 34463711 CON - Rheumatology (Oxford). 2022 Jul 6;61(7):e188-e189. doi: 10.1093/rheumatology/keab884. PMID: 34864903 MH - *Connective Tissue Diseases/complications MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/etiology EDAT- 2021/12/06 06:00 MHDA- 2022/07/09 06:00 CRDT- 2021/12/05 21:38 PHST- 2021/11/08 00:00 [received] PHST- 2021/11/12 00:00 [accepted] PHST- 2021/12/06 06:00 [pubmed] PHST- 2022/07/09 06:00 [medline] PHST- 2021/12/05 21:38 [entrez] AID - 6448781 [pii] AID - 10.1093/rheumatology/keab885 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Jul 6;61(7):e190-e191. doi: 10.1093/rheumatology/keab885. PMID- 22390864 OWN - NLM STAT- MEDLINE DCOM- 20120425 LR - 20131121 IS - 0030-6002 (Print) IS - 0030-6002 (Linking) VI - 153 IP - 11 DP - 2012 Mar 18 TI - [Raynaud's syndrome, 2011]. PG - 403-9 LID - 10.1556/OH.2012.29321 [doi] AB - Raynaud's phenomenon is characterized by intense vasospasm of the digital arteries on cold exposure or emotional stress, leading to well-defined colour changes in the skin of the fingers. Behind the clinical manifestations, there is an imbalance between vasoconstrictor and vasodilator factors. It may be primary or secondary to an underlying condition, including autoimmune diseases. Physical examination, nail fold capillaroscopy and immunological tests can differentiate primary forms from secondary ones. The treatment is based on preventing exposure to cold, emotional stress and the administration of certain drugs and, if attacks are present, vasodilators, prostaglandin analogues and anticoagulants may be given. This review focuses on the characteristics of Raynaud's phenomenon and the available diagnostic and therapeutic options. FAU - Takáts, Alajos AU - Takáts A AD - Semmelweis Egyetem, Általános Orvostudományi Kar I. Sebészeti Klinika Budapest. FAU - Garai, Ildikó AU - Garai I FAU - Papp, Gábor AU - Papp G FAU - Hevér, Tímea AU - Hevér T FAU - Csiki, Emese AU - Csiki E FAU - András, Csilla AU - András C FAU - Csiki, Zoltán AU - Csiki Z LA - hun PT - English Abstract PT - Journal Article PT - Review TT - Raynaud-szindróma, 2011. PL - Hungary TA - Orv Hetil JT - Orvosi hetilap JID - 0376412 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Angiography MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Body Temperature MH - Calcium Channel Blockers/therapeutic use MH - Diagnosis, Differential MH - Humans MH - Nitroglycerin/therapeutic use MH - Plethysmography MH - *Raynaud Disease/classification/diagnosis/epidemiology/etiology/physiopathology/therapy MH - Rheology MH - Vasodilator Agents/therapeutic use EDAT- 2012/03/07 06:00 MHDA- 2012/04/26 06:00 CRDT- 2012/03/07 06:00 PHST- 2012/03/07 06:00 [entrez] PHST- 2012/03/07 06:00 [pubmed] PHST- 2012/04/26 06:00 [medline] AID - V30J7U018140631X [pii] AID - 10.1556/OH.2012.29321 [doi] PST - ppublish SO - Orv Hetil. 2012 Mar 18;153(11):403-9. doi: 10.1556/OH.2012.29321. PMID- 21117349 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20101201 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 58 DP - 2010 May TI - Raynaud's phenomenon. PG - 309-13 AB - Raynauds phenomena (RP) is a commonly encountered clinical manifestation which may be primary or secondary to underlying disease. There is imbalance between vasoconstricting and vasodilating factors. Physical examination, nailfold capillaroscopy and immunological tests can differentiate primary from secondary RP. Treatment involves prevention of RP so that permanent ischemic damage i.e. gangrene can be avoided. Avoidance of exposure to cold, emotional stress and certain drugs is mandatory and if attacks are occurring then vasodilators, prostaglandin analogues, anticoagulants and antiplatelet drugs may be added. An attempt has been made to guide the clinician to diagnose and treat a patient of RP through this article. FAU - Saigal, Renu AU - Saigal R AD - Department of Medicine, Sawai Man Singh Medical College and Hospital, Jaipur, India. FAU - Kansal, Amit AU - Kansal A FAU - Mittal, Manoop AU - Mittal M FAU - Singh, Yadvinder AU - Singh Y FAU - Ram, Hari AU - Ram H LA - eng PT - Journal Article PT - Review PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Autoimmune Diseases/complications MH - Humans MH - Prognosis MH - *Raynaud Disease/classification/diagnosis/etiology/physiopathology/therapy EDAT- 2010/12/02 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/12/02 06:00 PHST- 2010/12/02 06:00 [entrez] PHST- 2010/12/02 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2010 May;58:309-13. PMID- 39063426 OWN - NLM STAT- MEDLINE DCOM- 20240727 LR - 20250711 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 21 IP - 7 DP - 2024 Jun 28 TI - Raynaud's Phenomenon of the Nipple: Epidemiological, Clinical, Pathophysiological, and Therapeutic Characterization. LID - 10.3390/ijerph21070849 [doi] LID - 849 AB - Raynaud's phenomenon of the nipple is a possible cause of pain and breastfeeding cessation in lactating women. However, there are still few studies on the characterization of this manifestation. Thus, we aim to develop a systematic review of the literature carried out between January 1992 and January 2024 in PubMed, Scopus, Web of Science, Virtual Health Library (VHL), and Portal de Periódicos da CAPES. Of the 438 articles, 19 met the eligibility criteria. The findings were divided by heuristic questions into two groups: "Epidemiological, pathophysiological, and clinical characterization of Raynaud's Phenomenon of the nipple" and "Treatment of Raynaud's Phenomenon of the nipple". Raynaud's phenomenon of the nipple is commonly primary, being more prevalent in the postpartum period, in women with a mean age of 32 years. The main triggers appear to be stress and temperature change. Generally, it is associated with a change in color and pain during breastfeeding. A calcium channel blocker was the most used medication with or without non-pharmacological measures. FAU - Moreira, Thaís Gomes AU - Moreira TG AD - School of Medicine, Universidade Federal do Cariri (UFCA), Barbalha 63048-080, Ceará, Brazil. FAU - Castro, Giovana Mamede AU - Castro GM AD - School of Medicine, Universidade Federal do Cariri (UFCA), Barbalha 63048-080, Ceará, Brazil. FAU - Gonçalves Júnior, Jucier AU - Gonçalves Júnior J AUID- ORCID: 0000-0001-5077-7959 AD - School of Medicine, Universidade Federal do Cariri (UFCA), Barbalha 63048-080, Ceará, Brazil. LA - eng PT - Journal Article PT - Systematic Review DEP - 20240628 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 SB - IM MH - Humans MH - *Raynaud Disease/epidemiology/physiopathology MH - *Nipples/physiopathology MH - Female MH - Breast Feeding MH - Adult PMC - PMC11276586 OTO - NOTNLM OT - Raynaud phenomenon OT - breastfeeding OT - systematic review OT - treatment COIS- The authors declare no conflicts of interest. EDAT- 2024/07/27 10:44 MHDA- 2024/07/28 14:52 PMCR- 2024/06/28 CRDT- 2024/07/27 01:15 PHST- 2024/06/06 00:00 [received] PHST- 2024/06/25 00:00 [revised] PHST- 2024/06/27 00:00 [accepted] PHST- 2024/07/28 14:52 [medline] PHST- 2024/07/27 10:44 [pubmed] PHST- 2024/07/27 01:15 [entrez] PHST- 2024/06/28 00:00 [pmc-release] AID - ijerph21070849 [pii] AID - ijerph-21-00849 [pii] AID - 10.3390/ijerph21070849 [doi] PST - epublish SO - Int J Environ Res Public Health. 2024 Jun 28;21(7):849. doi: 10.3390/ijerph21070849. PMID- 16609626 OWN - NLM STAT- MEDLINE DCOM- 20060621 LR - 20191109 IS - 0398-0499 (Print) IS - 0398-0499 (Linking) VI - 31 IP - 1 DP - 2006 Feb TI - [Heredity and genetic aspects of Raynaud's disease]. PG - 10-5 AB - The pathophysiology of primary Raynaud's phenomenon (Raynaud's disease) remains uncertain but the transmission of this primary microcirculatory dysregulation seems strongly influenced by genetic factors. For a long time, physicians have found that the hereditary factor plays an important role in the genesis of Raynaud's disease. Familial analysis and twin studies have confirmed the role of an hereditary factor. It seems heterogeneous but pedigree analysis indicates the possibility of an autosomal dominant transmission influenced by sex, in some families, allowing an approach called "reverse genetic" based on linkage analysis. Such an approach has focused on few loci but sequencing of candidate genes for genetic mutations remains negative. Given the supposed heterogeneity of the genetic transmission of Raynaud's disease, diversification of strategies in molecular genetics is suitable with reference to techniques applied to multifactorial heredity. FAU - Pistorius, M A AU - Pistorius MA AD - Service de Médecine Interne et Vasculaire, CHU Hôtel-Dieu, Place Alexis Ricordeau, 44000 Nantes. FAU - Planchon, B AU - Planchon B FAU - Schott, J J AU - Schott JJ FAU - Lemarec, H AU - Lemarec H LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Aspects héréditaires et génétiques de la maladie de Raynaud. PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Female MH - Humans MH - Male MH - Microcirculation MH - Pedigree MH - Raynaud Disease/*genetics RF - 31 EDAT- 2006/04/13 09:00 MHDA- 2006/06/22 09:00 CRDT- 2006/04/13 09:00 PHST- 2006/04/13 09:00 [pubmed] PHST- 2006/06/22 09:00 [medline] PHST- 2006/04/13 09:00 [entrez] AID - MDOI-JMV-01-2006-31-1-0398-0499-101019-200517601 [pii] AID - 10.1016/s0398-0499(06)76512-x [doi] PST - ppublish SO - J Mal Vasc. 2006 Feb;31(1):10-5. doi: 10.1016/s0398-0499(06)76512-x. PMID- 19065406 OWN - NLM STAT- MEDLINE DCOM- 20091002 LR - 20091117 IS - 1745-3062 (Electronic) IS - 1745-3054 (Linking) VI - 31 IP - 4 DP - 2008 Dec TI - Raynaud's phenomenon. PG - 148-9 LID - 10.1080/17453050802595349 [doi] FAU - Betton, Carly AU - Betton C AD - University Hospitals Birmingham NHS Foundation, Trust, UK. carly.betton@uhb.nhs.uk FAU - Rowland, Katy AU - Rowland K LA - eng PT - Journal Article PL - England TA - J Vis Commun Med JT - Journal of visual communication in medicine JID - 101254059 SB - IM MH - Fingers/*blood supply MH - Humans MH - *Medical Illustration MH - Photography MH - Raynaud Disease/*physiopathology EDAT- 2008/12/10 09:00 MHDA- 2009/10/03 06:00 CRDT- 2008/12/10 09:00 PHST- 2008/12/10 09:00 [pubmed] PHST- 2009/10/03 06:00 [medline] PHST- 2008/12/10 09:00 [entrez] AID - 906497168 [pii] AID - 10.1080/17453050802595349 [doi] PST - ppublish SO - J Vis Commun Med. 2008 Dec;31(4):148-9. doi: 10.1080/17453050802595349. PMID- 39392515 OWN - NLM STAT- MEDLINE DCOM- 20241121 LR - 20241205 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 43 IP - 12 DP - 2024 Dec TI - Development and validation of the RQLQ: a Raynaud's disease-specific measure of health-related quality of life. PG - 3963-3972 LID - 10.1007/s10067-024-07175-0 [doi] AB - INTRODUCTION/OBJECTIVES: The aim of this study was to develop and validate the Raynaud Specific Quality of Life Questionnaire (RQLQ) for assessing health-related quality (HRQOL) of life in patients with Raynaud's disease (RD). METHOD: The questionnaire was developed and validated in three stages. Initially, semi-structured interviews with 28 RD patients identified domains of everyday life affected by RD, leading to the creation of the initial RQLQ. In the first quantitative stage, 101 patients completed the RQLQ, and exploratory factor analysis assessed dimensionality and factor structure. After removing poorly performing items, the final RQLQ was tested with 102 patients. This stage also evaluated convergent, divergent, and discriminant validity, as well as internal reliability. RESULTS: From the interviews, 135 items were generated, with factor analysis refining the measure to 29 items across five subscales, showing good internal consistency. The RQLQ demonstrated significant correlations with self-rated quality of life and physical and mental health outcomes, confirming convergent and divergent validity. It also showed discriminant validity for different levels of disease activity. CONCLUSIONS: The RQLQ is the first specific HRQOL measure for RD patients, proving to be a psychometrically sound, reliable, and valid tool for clinical research and practice. Key Points • The Raynaud Specific Quality of Life Questionnaire (RQLQ) is an important scale that evaluates the quality of life of patients with Raynaud's disease. • The questionnaire showed good validity and reliability a capturing disease-specific quality of life. • This tool may aid in clinical research and practice. CI - © 2024. The Author(s). FAU - Fábián, Balázs AU - Fábián B AUID- ORCID: 0000-0002-1073-568X AD - Department of Behavioural Sciences, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. fabian.balazs@med.unideb.hu. FAU - Csiki, Zoltán AU - Csiki Z AD - Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. FAU - Bugán, Antal AU - Bugán A AD - Clinical Psychology Center of Clinical Center, University of Debrecen, Debrecen, Hungary. LA - eng PT - Journal Article PT - Validation Study DEP - 20241011 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - *Quality of Life MH - *Raynaud Disease/psychology/diagnosis MH - Female MH - Male MH - Middle Aged MH - Reproducibility of Results MH - Surveys and Questionnaires MH - Adult MH - *Psychometrics MH - Aged MH - Factor Analysis, Statistical PMC - PMC11582172 OTO - NOTNLM OT - Health-related quality of life OT - Questionnaire OT - Raynaud’s disease OT - Reliability OT - Validity COIS- Declarations. Institutional review board: The study was conducted in accordance with the Declaration of Helsinki and approved by the University of Debrecen Clinical Centre’s Regional and Institutional Ethics Committee and the Scientific (registration number, RKEB/IKEB 4822–2017). Informed consent: Informed consent was obtained from all subjects involved in the study. Disclosures: None. EDAT- 2024/10/11 12:26 MHDA- 2024/11/21 18:21 PMCR- 2024/10/11 CRDT- 2024/10/11 11:04 PHST- 2024/06/14 00:00 [received] PHST- 2024/10/03 00:00 [accepted] PHST- 2024/08/27 00:00 [revised] PHST- 2024/11/21 18:21 [medline] PHST- 2024/10/11 12:26 [pubmed] PHST- 2024/10/11 11:04 [entrez] PHST- 2024/10/11 00:00 [pmc-release] AID - 10.1007/s10067-024-07175-0 [pii] AID - 7175 [pii] AID - 10.1007/s10067-024-07175-0 [doi] PST - ppublish SO - Clin Rheumatol. 2024 Dec;43(12):3963-3972. doi: 10.1007/s10067-024-07175-0. Epub 2024 Oct 11. PMID- 18656283 OWN - NLM STAT- MEDLINE DCOM- 20080930 LR - 20080916 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 59 IP - 4 DP - 2008 Oct TI - Raynaud's phenomenon: pathogenesis and management. PG - 633-53 LID - 10.1016/j.jaad.2008.06.004 [doi] AB - Raynaud's phenomenon is a common clinical disorder for which patients frequently seek the expertise and care of dermatologists. It is manifested by recurrent vasospasm of the fingers and toes, often associated with exposure to cold temperature or emotional stress. The phenomenon is named after Maurice Raynaud, who, as a medical student, defined the first case in 1862 as episodic, symmetric, acral vasospasm characterized by pallor, cyanosis, suffusion, and a sense of fullness or tautness, which may be painful. Despite more than 140 years of research, the pathophysiology of Raynaud's phenomenon continues to elude investigators. Accordingly, although many pharmacologic treatments have been reported, there is still no cure or gold standard therapy. Further, response to treatment varies and is difficult to predict. Recently, there has been renewed interest in finding the pathogenetic mechanisms of Raynaud's phenomenon, an effort that has led to more potential targeted therapeutics. The purpose of this review is to discuss recent breakthroughs in the pathogenesis and treatment of Raynaud's phenomenon. FAU - Bakst, Richard AU - Bakst R AD - Ronald O. Perelman Department of Dermatology, New York University Medical Center, New York, New York 10016, USA. FAU - Merola, Joseph F AU - Merola JF FAU - Franks, Andrew G Jr AU - Franks AG Jr FAU - Sanchez, Miguel AU - Sanchez M LA - eng PT - Journal Article PT - Review DEP - 20080724 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Reactive Oxygen Species) SB - IM MH - Adult MH - Fibrinolysis MH - Humans MH - Platelet Activation MH - Raynaud Disease/diagnosis/etiology/*physiopathology/*therapy MH - Reactive Oxygen Species/metabolism MH - Sympathectomy RF - 162 EDAT- 2008/07/29 09:00 MHDA- 2008/10/01 09:00 CRDT- 2008/07/29 09:00 PHST- 2008/05/06 00:00 [received] PHST- 2008/05/28 00:00 [revised] PHST- 2008/06/03 00:00 [accepted] PHST- 2008/07/29 09:00 [pubmed] PHST- 2008/10/01 09:00 [medline] PHST- 2008/07/29 09:00 [entrez] AID - S0190-9622(08)00653-1 [pii] AID - 10.1016/j.jaad.2008.06.004 [doi] PST - ppublish SO - J Am Acad Dermatol. 2008 Oct;59(4):633-53. doi: 10.1016/j.jaad.2008.06.004. Epub 2008 Jul 24. PMID- 38109761 OWN - NLM STAT- MEDLINE DCOM- 20231220 LR - 20241122 IS - 2164-554X (Electronic) IS - 2164-5515 (Print) IS - 2164-5515 (Linking) VI - 19 IP - 3 DP - 2023 Dec 15 TI - Special focus on the association between COVID-19 vaccination and Raynaud's phenomenon in autoimmune disease. PG - 2295077 LID - 10.1080/21645515.2023.2295077 [doi] LID - 2295077 FAU - Bao, Zheng AU - Bao Z AD - Department of Clinical Laboratory, Xiangshan Red Cross Taiwan Compatriot Hospital, Xiangshan, Zhejiang, China. FAU - Liu, Jinlin AU - Liu J AD - Department of Clinical Laboratory, South China Hospital, Medical School, Shenzhen University, Shenzhen, China. FAU - Yu, Diao AU - Yu D AD - Department of Clinical Laboratory, Zhejiang Provincial People's Hospital Bijie Hospital, Bijie, Guizhou, China. LA - eng PT - Comment PT - Letter DEP - 20231218 PL - United States TA - Hum Vaccin Immunother JT - Human vaccines & immunotherapeutics JID - 101572652 RN - 0 (COVID-19 Vaccines) SB - IM CON - Hum Vaccin Immunother. 2023 Dec 31;19(1):2199653. doi: 10.1080/21645515.2023.2199653. PMID: 37067070 MH - Humans MH - *Autoimmune Diseases MH - *COVID-19/prevention & control MH - COVID-19 Vaccines/adverse effects MH - *Raynaud Disease MH - Vaccination PMC - PMC10730199 OTO - NOTNLM OT - Association OT - COVID-19 vaccination OT - Raynaud’s phenomenon OT - autoimmune disease patients COIS- No potential conflict of interest was reported by the author(s). EDAT- 2023/12/18 18:41 MHDA- 2023/12/20 06:42 PMCR- 2023/12/18 CRDT- 2023/12/18 17:13 PHST- 2023/12/20 06:42 [medline] PHST- 2023/12/18 18:41 [pubmed] PHST- 2023/12/18 17:13 [entrez] PHST- 2023/12/18 00:00 [pmc-release] AID - 2295077 [pii] AID - 10.1080/21645515.2023.2295077 [doi] PST - ppublish SO - Hum Vaccin Immunother. 2023 Dec 15;19(3):2295077. doi: 10.1080/21645515.2023.2295077. Epub 2023 Dec 18. PMID- 37169136 OWN - NLM STAT- MEDLINE DCOM- 20230811 LR - 20230816 IS - 1532-2165 (Electronic) IS - 1078-5884 (Linking) VI - 66 IP - 2 DP - 2023 Aug TI - Is there any role for surgical management of primary Raynaud's disease? PG - 291 LID - S1078-5884(23)00365-9 [pii] LID - 10.1016/j.ejvs.2023.05.004 [doi] FAU - Budge, James AU - Budge J AD - St George's Vascular Institute, St George's University Hospitals NHS Foundation Trust, London, UK; St George's University of London, London, UK. Electronic address: james.budge@stgeorges.nhs.uk. FAU - Azhar, Bilal AU - Azhar B AD - St George's Vascular Institute, St George's University Hospitals NHS Foundation Trust, London, UK; St George's University of London, London, UK. FAU - Desai, Mital AU - Desai M AD - St George's Vascular Institute, St George's University Hospitals NHS Foundation Trust, London, UK; St George's University of London, London, UK. LA - eng PT - Comment PT - Letter DEP - 20230509 PL - England TA - Eur J Vasc Endovasc Surg JT - European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery JID - 9512728 SB - IM CON - Eur J Vasc Endovasc Surg. 2023 Aug;66(2):278-279. doi: 10.1016/j.ejvs.2023.04.003. PMID: 37054875 MH - Humans MH - *Raynaud Disease/surgery EDAT- 2023/05/12 01:07 MHDA- 2023/08/11 06:42 CRDT- 2023/05/11 19:33 PHST- 2023/04/17 00:00 [received] PHST- 2023/05/03 00:00 [accepted] PHST- 2023/08/11 06:42 [medline] PHST- 2023/05/12 01:07 [pubmed] PHST- 2023/05/11 19:33 [entrez] AID - S1078-5884(23)00365-9 [pii] AID - 10.1016/j.ejvs.2023.05.004 [doi] PST - ppublish SO - Eur J Vasc Endovasc Surg. 2023 Aug;66(2):291. doi: 10.1016/j.ejvs.2023.05.004. Epub 2023 May 9. PMID- 38640572 OWN - NLM STAT- MEDLINE DCOM- 20240603 LR - 20250128 IS - 1878-3279 (Electronic) IS - 0171-2985 (Linking) VI - 229 IP - 3 DP - 2024 May TI - Association of neutrophil extracellular trap levels with Raynaud's phenomenon, glomerulonephritis and disease index score in SLE patients from Brazil. PG - 152803 LID - S0171-2985(24)00021-4 [pii] LID - 10.1016/j.imbio.2024.152803 [doi] AB - Neutrophil extracellular traps (NETs) are cell-extruded DNA strands coated with neutrophils' nuclear proteins and enzymes from cytotoxic granules, produced by NETosis, a cell death pathway. They perform an important defensive role in innate immunity, but their increased production and/or inefficient degradation expose new antigens, such as DNA or citrullinated histone peptides, triggering autoimmunity. This study aimed to access possible associations between serum NETs levels with epidemiological, clinical, and serological data from a well-characterized SLE Brazilian patients' cohort. NET levels were evaluated in one hundred seventy serum samples of patients with Systemic Lupus Erythematosus (SLE) using an Immunoassay. Univariate and multivariate binary logistic regression used clinical patients' data as independent variables. Parametric and non-parametric tests compared log10 base serum NET levels transformed between patients' groups. SLE patients were also dichotomized into "High serum NET levels" and "Low serum NET levels" groups. All analyses were performed in R language 4.1.2, and p < 0.05 were considered significant. Increased susceptibility for high serum NET levels was observed in SLE patients with Raynaud's phenomenon (OR = 2.30, 95 % CI = 1.06-5.21 and p = 0.039), independently of any other risk factor. Also, SLE patients with Raynaud's phenomenon presented higher mean NET serum levels (mean = -0.13 vs. -0.51, p = 0.01). In addition, higher mean NET serum levels were associated with glomerulonephritis (mean = -0.45 vs. -0.12, p = 0.03). Ultimately, the SLEDAI index scored higher in the high NETs serum levels group (median = 2.0 vs. 0.0, p = 6 × 10(-3)). The formation of NETs might be implicated in Raynaud's phenomenon, glomerulonephritis, and disease index score in SLE patients. Our results highlight the importance of serum NET levels as a possible therapeutical target to modulate the clinical course of SLE. CI - Copyright © 2024 The Authors. Published by Elsevier GmbH.. All rights reserved. FAU - Delabio Auer, Eduardo AU - Delabio Auer E AD - Postgraduate Program in Genetics. Department of Genetics, Federal University of Paraná (UFPR), Centro Politécnico, Jardim das Américas, 81531-990, Curitiba, Paraná, Brazil. FAU - Bumiller-Bini Hoch, Valéria AU - Bumiller-Bini Hoch V AD - Postgraduate Program in Genetics. Department of Genetics, Federal University of Paraná (UFPR), Centro Politécnico, Jardim das Américas, 81531-990, Curitiba, Paraná, Brazil. FAU - Borges da Silva, Emiliano AU - Borges da Silva E AD - Federal University of Paraná (UFPR), Centro Politécnico, Jardim das Américas, 81531-990 Curitiba, PR, Brazil. FAU - Ricci Zonta, Yohan AU - Ricci Zonta Y AD - São Paulo State University (UNESP), Medical School of Botucatu, Laboratory of Immunopathology and Infectious Agents - LIAI, UNIPEX - Experimental Research Unity, Sector 5, Botucatu, SP, Brazil. FAU - Alarcão Dias-Melicio, Luciane AU - Alarcão Dias-Melicio L AD - São Paulo State University (UNESP), Medical School of Botucatu, Laboratory of Immunopathology and Infectious Agents - LIAI, UNIPEX - Experimental Research Unity, Sector 5, Botucatu, SP, Brazil; São Paulo State University (UNESP), Medical School of Botucatu, Department of Pathology, Botucatu, SP, Brazil. FAU - Larocca Skare, Thelma AU - Larocca Skare T AD - Mackenzie Evangelical School of Medicine Paraná-Curitiba, Paraná, Brazil. FAU - F Picceli, Vanessa AU - F Picceli V AD - Hospital Geral de Curitiba - Exército Brasileiro - Curitiba, Paraná, Brazil. FAU - Messias-Reason, Iara José AU - Messias-Reason IJ AD - Laboratory of Immunopathology, Department of Clinical Pathology, Federal University of Paraná, Curitiba, Brazil. FAU - Boldt, Angelica Beate Winter AU - Boldt ABW AD - Postgraduate Program in Genetics. Department of Genetics, Federal University of Paraná (UFPR), Centro Politécnico, Jardim das Américas, 81531-990, Curitiba, Paraná, Brazil. Electronic address: angelicaboldt@gmail.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20240405 PL - Netherlands TA - Immunobiology JT - Immunobiology JID - 8002742 RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Extracellular Traps/metabolism/immunology MH - Female MH - Male MH - Brazil/epidemiology MH - Adult MH - *Lupus Erythematosus, Systemic/blood/diagnosis/immunology MH - *Raynaud Disease/etiology/blood/immunology MH - Middle Aged MH - Neutrophils/immunology MH - Severity of Illness Index MH - Glomerulonephritis/blood/immunology/diagnosis MH - Young Adult MH - Biomarkers/blood OTO - NOTNLM OT - Glomerulonephritis OT - NETs OT - Raynaud's syndrome OT - SLE OT - SLEDAI COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/04/20 00:42 MHDA- 2024/06/04 00:45 CRDT- 2024/04/19 18:02 PHST- 2023/07/23 00:00 [received] PHST- 2024/02/28 00:00 [revised] PHST- 2024/04/04 00:00 [accepted] PHST- 2024/06/04 00:45 [medline] PHST- 2024/04/20 00:42 [pubmed] PHST- 2024/04/19 18:02 [entrez] AID - S0171-2985(24)00021-4 [pii] AID - 10.1016/j.imbio.2024.152803 [doi] PST - ppublish SO - Immunobiology. 2024 May;229(3):152803. doi: 10.1016/j.imbio.2024.152803. Epub 2024 Apr 5. PMID- 37541711 OWN - NLM STAT- MEDLINE DCOM- 20230807 LR - 20230807 IS - 1557-9859 (Electronic) IS - 0025-7125 (Linking) VI - 107 IP - 5 DP - 2023 Sep TI - Cold Hands or Feet: Is It Raynaud's or Not? PG - 829-844 LID - S0025-7125(23)00060-3 [pii] LID - 10.1016/j.mcna.2023.04.005 [doi] AB - Raynaud's phenomenon is an exaggerated response to cold stimuli that may be primary or secondary. The diagnosis relies on patient history and physical examination to distinguish RP from other vasomotor dysfunction (e.g. acrocyanosis, pernio, small fiber neuropathy with vasomotor symptoms, and complex regional pain syndrome). Achenbach syndrome, or spontaneous venous hemorrhage, may also be mistaken for RP but is a self-limiting phenomenon. Laboratory evaluation and vascular diagnostic testing may identify SRP causes. Regardless of etiology, treatment includes warming with trigger avoidance, and consideration of vasodilators (eg. calcium channel, alpha-1 blockers). SRP with digital ulceration may require PDE5i, endothelin-1 receptor blockers, and prostanoids. Refractory cases may require pneumatic arterial pumps, botulinum toxin administration, or surgical sympathectomy. CI - Copyright © 2023 Elsevier Inc. All rights reserved. FAU - Kadian-Dodov, Daniella AU - Kadian-Dodov D AD - Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, 1190 Fifth Avenue, Box 1030, New York, NY 10029, USA. Electronic address: daniella.kadian-dodov@mountsinai.org. LA - eng PT - Journal Article PT - Review DEP - 20230604 PL - United States TA - Med Clin North Am JT - The Medical clinics of North America JID - 2985236R SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy OTO - NOTNLM OT - Cold-induced vasospasm OT - Raynaud OT - Raynaud’s disease OT - Raynaud’s phenomenon OT - Vasospasm EDAT- 2023/08/05 05:41 MHDA- 2023/08/07 06:42 CRDT- 2023/08/04 20:56 PHST- 2023/08/07 06:42 [medline] PHST- 2023/08/05 05:41 [pubmed] PHST- 2023/08/04 20:56 [entrez] AID - S0025-7125(23)00060-3 [pii] AID - 10.1016/j.mcna.2023.04.005 [doi] PST - ppublish SO - Med Clin North Am. 2023 Sep;107(5):829-844. doi: 10.1016/j.mcna.2023.04.005. Epub 2023 Jun 4. PMID- 26098320 OWN - NLM STAT- MEDLINE DCOM- 20150812 LR - 20150623 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 44 IP - 3 DP - 2015 May TI - Raynaud’s phenomenon - assessment and differential diagnoses. PG - 166-77 LID - 10.1024/0301-1526/a000426 [doi] AB - Raynaud’s phenomenon (RP) is characterised by paroxysmal reversible episodes of vasospasm, usually involving peripheral small vessels of the fingers or toes and resulting in a triple-colour change starting with pallor and followed by cyanosis and erythema. Attacks are typically triggered by cold or emotional stress. The diagnosis of RP can be made on the basis of the patient’s clinical symptoms. Primary RP occurs without underlying disease and is considered a benign condition. A normal erythrocyte sedimentation rate, negative testing for antinuclear antibodies, normal nailfold capillaries and the absence of structural micro- or macrovascular damage and other diseases lead to the diagnosis of primary RP. Digital photoplethysmography and pulse contour analysis can be used as an additional tool to exclude structural macro- or microvascular disease. In contrast, secondary RP is associated with other diseases, mainly connective tissue diseases such as systemic sclerosis. If there is a suspicion of secondary RP, a thorough laboratory and vascular assessment is required to make the diagnosis of underlying disease. Acrocyanosis and erythromelalgia are additional functional vascular disorders that can be easily distinguished when patients are carefully assessed for their history and clinical symptoms. FAU - Linnemann, Birgit AU - Linnemann B AD - 1 Medical Practice of Angiology and Haemostaseology, Praxis am Grüneburgweg, Frankfurt, Germany. FAU - Erbe, Matthias AU - Erbe M LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Diagnosis, Differential MH - Humans MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/*diagnosis/epidemiology/physiopathology MH - Risk Factors OTO - NOTNLM OT - Raynaud’s phenomenon OT - acrocyanosis OT - digital photoplethysmography OT - erythromelalgia OT - microvascular disease OT - nailfold capillaroscopy EDAT- 2015/06/23 06:00 MHDA- 2015/08/13 06:00 CRDT- 2015/06/23 06:00 PHST- 2015/06/23 06:00 [entrez] PHST- 2015/06/23 06:00 [pubmed] PHST- 2015/08/13 06:00 [medline] AID - 10.1024/0301-1526/a000426 [doi] PST - ppublish SO - Vasa. 2015 May;44(3):166-77. doi: 10.1024/0301-1526/a000426. PMID- 36933784 OWN - NLM STAT- MEDLINE DCOM- 20230626 LR - 20231221 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 90 IP - 4 DP - 2023 Jul TI - Coexistence of erythromelalgia and Raynaud's phenomenon. PG - 105561 LID - S1297-319X(23)00040-4 [pii] LID - 10.1016/j.jbspin.2023.105561 [doi] FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH 44195, United States. Electronic address: chattes@ccf.org. LA - eng PT - Letter DEP - 20230317 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Humans MH - *Erythromelalgia/complications/diagnosis MH - *Raynaud Disease/complications/diagnosis OTO - NOTNLM OT - Erythromelalgia OT - Mixed connective tissue disease OT - Raynaud's phenomenon OT - Reactive hyperemia OT - Vasospasm EDAT- 2023/03/19 06:00 MHDA- 2023/06/26 06:41 CRDT- 2023/03/18 20:31 PHST- 2022/11/25 00:00 [received] PHST- 2023/02/18 00:00 [revised] PHST- 2023/03/01 00:00 [accepted] PHST- 2023/06/26 06:41 [medline] PHST- 2023/03/19 06:00 [pubmed] PHST- 2023/03/18 20:31 [entrez] AID - S1297-319X(23)00040-4 [pii] AID - 10.1016/j.jbspin.2023.105561 [doi] PST - ppublish SO - Joint Bone Spine. 2023 Jul;90(4):105561. doi: 10.1016/j.jbspin.2023.105561. Epub 2023 Mar 17. PMID- 24418302 OWN - NLM STAT- MEDLINE DCOM- 20140513 LR - 20220331 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 13 IP - 6 DP - 2014 Jun TI - Raynaud's phenomenon: from molecular pathogenesis to therapy. PG - 655-67 LID - S1568-9972(14)00002-0 [pii] LID - 10.1016/j.autrev.2013.12.001 [doi] AB - Raynaud's phenomenon (RP) is a well defined clinical syndrome characterized by recurrent episodes of digital vasospasm triggered by exposure to physical/chemical or emotional stress. RP has been classified as primary or secondary, depending on whether it occurs as an isolated condition (pRP) or is associated to an underlying disease, mainly a connective tissue disease (CTD-RP). In both cases, it manifests with unique "triple" (pallor, cyanosis and erythema), or "double" color changes. pRP is usually a benign condition, while sRP can evolve and be complicated by acral digital ulcers and gangrene, which may require surgical treatment. The pathogenesis of RP has not yet been entirely clarified, nor is it known whether autoantibodies have a role in RP. Even so, recent advances in our understanding of the pathophysiology have highlighted novel potential therapeutic targets. The aim of this review is to discuss the etiology, epidemiology, risk factors, clinical manifestations, recently disclosed pathogenic mechanisms underlying RP and their correlation with the available therapeutic options, focusing primarily on pRP and CTD-RP. CI - Copyright © 2014 Elsevier B.V. All rights reserved. FAU - Prete, Marcella AU - Prete M AD - Internal Medicine, University of Bari Medical School, I-70124 Bari, Italy. FAU - Fatone, Maria Celeste AU - Fatone MC AD - Internal Medicine, University of Bari Medical School, I-70124 Bari, Italy. FAU - Favoino, Elvira AU - Favoino E AD - Rheumatological and Autoimmune Systemic Diseases Units, University of Bari Medical School, I-70124 Bari, Italy. FAU - Perosa, Federico AU - Perosa F AD - Rheumatological and Autoimmune Systemic Diseases Units, University of Bari Medical School, I-70124 Bari, Italy. Electronic address: federico.perosa@uniba.it. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20140110 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Animals MH - Connective Tissue Diseases/etiology/therapy MH - Diagnosis, Differential MH - Humans MH - Oxidative Stress MH - Raynaud Disease/*etiology/physiopathology/*therapy MH - Risk Factors OTO - NOTNLM OT - Autoantibodies OT - Connective tissue disease OT - Raynaud's phenomenon OT - Smooth muscle cells OT - Systemic sclerosis OT - Targeted therapy EDAT- 2014/01/15 06:00 MHDA- 2014/05/14 06:00 CRDT- 2014/01/15 06:00 PHST- 2013/12/02 00:00 [received] PHST- 2013/12/24 00:00 [accepted] PHST- 2014/01/15 06:00 [entrez] PHST- 2014/01/15 06:00 [pubmed] PHST- 2014/05/14 06:00 [medline] AID - S1568-9972(14)00002-0 [pii] AID - 10.1016/j.autrev.2013.12.001 [doi] PST - ppublish SO - Autoimmun Rev. 2014 Jun;13(6):655-67. doi: 10.1016/j.autrev.2013.12.001. Epub 2014 Jan 10. PMID- 25511234 OWN - NLM STAT- MEDLINE DCOM- 20160303 LR - 20210402 IS - 1573-2665 (Electronic) IS - 0141-8955 (Linking) VI - 38 IP - 2 DP - 2015 Mar TI - Raynaud's phenomenon associated with Fabry disease. PG - 367-8 LID - 10.1007/s10545-014-9799-z [doi] FAU - Germain, Dominique P AU - Germain DP AD - University of Versailles, UFR des Sciences de la Santé Simone Veil, UMR 1179, Montigny, 78180, France, dominique.germain@rpc.aphp.fr. FAU - Atanasiu, Oana I AU - Atanasiu OI FAU - Akrout-Marouene, Jouda AU - Akrout-Marouene J FAU - Benistan, Karelle AU - Benistan K LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141216 PL - United States TA - J Inherit Metab Dis JT - Journal of inherited metabolic disease JID - 7910918 RN - EC 3.2.1.22 (GLA protein, human) RN - EC 3.2.1.22 (alpha-Galactosidase) SB - IM MH - Fabry Disease/*complications/diagnosis/genetics MH - Genetic Predisposition to Disease MH - Humans MH - Male MH - Middle Aged MH - Mutation MH - Phenotype MH - Raynaud Disease/diagnosis/*etiology MH - Risk Factors MH - alpha-Galactosidase/genetics EDAT- 2014/12/17 06:00 MHDA- 2016/03/05 06:00 CRDT- 2014/12/17 06:00 PHST- 2014/08/20 00:00 [received] PHST- 2014/11/23 00:00 [accepted] PHST- 2014/11/17 00:00 [revised] PHST- 2014/12/17 06:00 [entrez] PHST- 2014/12/17 06:00 [pubmed] PHST- 2016/03/05 06:00 [medline] AID - 10.1007/s10545-014-9799-z [doi] PST - ppublish SO - J Inherit Metab Dis. 2015 Mar;38(2):367-8. doi: 10.1007/s10545-014-9799-z. Epub 2014 Dec 16. PMID- 24926630 OWN - NLM STAT- MEDLINE DCOM- 20140925 LR - 20141120 IS - 0040-5957 (Print) IS - 0040-5957 (Linking) VI - 69 IP - 2 DP - 2014 Mar-Apr TI - [Pharmacology of Raynaud's phenomenon]. PG - 115-28 LID - 10.2515/therapie/2013068 [doi] AB - Raynaud's phenomenon (RP) is characterised by transient ischaemia in the extremities in response to cold or emotions. It can be primary (idiopathic) or secondary to an underlying disease. The pathophysiology of RP is multifactorial and complex. Microvascular impairment is a hallmark of the disease. The objective of this work is to review the different pharmacological treatments currently used in the management of RP, from their mechanism of action to the available evidence regarding their efficacy. We also propose to discuss potential pharmacological targets such as the potentiation of the nitric oxide pathway, or the inhibition of the RhoA-Rho kinase pathway. The last part of this review deals with drug-induced RP. Among various medications, beta-blockers, interferons, tyrosine-kinase inhibitors or cytotoxic agents such as bleomycin are involved. CI - © 2014 Société Française de Pharmacologie et de Thérapeutique. FAU - Roustit, Matthieu AU - Roustit M AD - UMR 1042, Inserm & Université Grenoble-Alpes, Grenoble, France - Unité de pharmacologie clinique, Centre d'Investigation clinique, Inserm CIC003, CHU de Grenoble, Grenoble, France. FAU - Khouri, Charles AU - Khouri C AD - Centre régional de Pharmacovigilance, CHU de Grenoble, Grenoble, France. FAU - Blaise, Sophie AU - Blaise S AD - UMR 1042, Inserm & Université Grenoble-Alpes, Grenoble, France - Clinique universitaire de Médecine vasculaire, CHU de Grenoble, Grenoble, France. FAU - Villier, Céline AU - Villier C AD - Centre régional de Pharmacovigilance, CHU de Grenoble, Grenoble, France. FAU - Carpentier, Patrick AU - Carpentier P AD - Clinique universitaire de Médecine vasculaire, CHU de Grenoble, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - UMR 1042, Inserm & Université Grenoble-Alpes, Grenoble, France - Unité de pharmacologie clinique, Centre d'Investigation clinique, Inserm CIC003, CHU de Grenoble, Grenoble, France. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Pharmacologie du phénomène de Raynaud. DEP - 20140612 PL - France TA - Therapie JT - Therapie JID - 0420544 RN - 0 (Adrenergic alpha-1 Receptor Antagonists) RN - 0 (Calcium Channel Blockers) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins) SB - IM MH - Adrenergic alpha-1 Receptor Antagonists/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Drug Discovery/trends MH - Drug-Related Side Effects and Adverse Reactions/drug therapy MH - Endothelin Receptor Antagonists MH - Humans MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Prostaglandins/therapeutic use MH - Raynaud Disease/*drug therapy/etiology EDAT- 2014/06/14 06:00 MHDA- 2014/09/26 06:00 CRDT- 2014/06/14 06:00 PHST- 2013/09/16 00:00 [received] PHST- 2013/09/19 00:00 [accepted] PHST- 2014/06/14 06:00 [entrez] PHST- 2014/06/14 06:00 [pubmed] PHST- 2014/09/26 06:00 [medline] AID - th132196 [pii] AID - 10.2515/therapie/2013068 [doi] PST - ppublish SO - Therapie. 2014 Mar-Apr;69(2):115-28. doi: 10.2515/therapie/2013068. Epub 2014 Jun 12. PMID- 27129065 OWN - NLM STAT- MEDLINE DCOM- 20160629 LR - 20160430 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 45 IP - 3 DP - 2016 TI - Raynaud's phenomenon and digital ischaemia--pharmacologic approach and alternative treatment options. PG - 201-12 LID - 10.1024/0301-1526/a000526 [doi] AB - The primary goal of therapy is to reduce the frequency and intensity of Raynaud's attacks and to minimize the related morbidity rather than to cure the underlying condition. Treatment strategies depend on whether Raynaud's phenomenon (RP) is primary or secondary. All patients should be instructed about general measures to maintain body warmth and to avoid triggers of RP attacks. Pharmacologic intervention can be useful for patients with severe and frequent RP episodes that impair the patient's quality of life. Calcium channel blockers are currently the most prescribed and studied medications for this purpose. There has been limited evidence for the efficacy of alpha-1-adrenergic receptor antagonists, angiotensin receptor blockers, topical nitrates or fluoxetine to treat RP. The intravenously administered prostacyclin analogue iloprost can reduce the frequency and severity of RP attacks and is considered a second-line therapy in patients with markedly impaired quality of life, critical digital ischaemia and skin ulcers who are at risk for substantial tissue loss and amputation. Phosphodiesterase inhibitors (e.g., sildenafil) can also improve RP symptoms and ulcer healing whereas endothelin-1 receptor antagonists (e.g., bosentan) are mainly considered treatment options in secondary prevention for patients with digital skin ulcers related to systemic sclerosis. However, their use in clinical practice has been limited by their high cost. Antiplatelet therapy with low-dose aspirin is recommended for all patients who suffer from secondary RP due to ischaemia caused by structural vessel damage. Anticoagulant therapy can be considered during the acute phase of digital ischaemia in patients with suspected vascular occlusive disease attributed to the occurrence of new thromboses. In patients with critical digital ischaemia, consideration should be given to hospitalisation, optimisation of medical treatment in accordance with the underlying disease and evaluation for a secondary, possibly reversible process that is causing or aggravating the clinical symptoms. FAU - Linnemann, Birgit AU - Linnemann B AD - 1 Medical Practice of Angiology and Haemostaseology, Praxis am Grüneburgweg, Frankfurt/Main, Germany. FAU - Erbe, Matthias AU - Erbe M AD - 1 Medical Practice of Angiology and Haemostaseology, Praxis am Grüneburgweg, Frankfurt/Main, Germany. LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 RN - 0 (Anticoagulants) RN - 0 (Platelet Aggregation Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Anticoagulants/adverse effects/*therapeutic use MH - Fingers/*blood supply MH - Humans MH - Ischemia/diagnosis/physiopathology/*therapy MH - Platelet Aggregation Inhibitors/adverse effects/*therapeutic use MH - Raynaud Disease/diagnosis/physiopathology/*therapy MH - Treatment Outcome MH - Vasodilator Agents/adverse effects/*therapeutic use OTO - NOTNLM OT - Raynaud’s phenomenon OT - calcium channel blocker OT - endothelin-1 receptor antagonist OT - iloprost OT - phosphodiesterase inhibitors OT - vasodilator EDAT- 2016/04/30 06:00 MHDA- 2016/06/30 06:00 CRDT- 2016/04/30 06:00 PHST- 2016/04/30 06:00 [entrez] PHST- 2016/04/30 06:00 [pubmed] PHST- 2016/06/30 06:00 [medline] AID - 10.1024/0301-1526/a000526 [doi] PST - ppublish SO - Vasa. 2016;45(3):201-12. doi: 10.1024/0301-1526/a000526. PMID- 40400145 OWN - NLM STAT- MEDLINE DCOM- 20250522 LR - 20250530 IS - 1643-3750 (Electronic) IS - 1234-1010 (Print) IS - 1234-1010 (Linking) VI - 31 DP - 2025 May 22 TI - Association Between Risk of Relapse and Type of Surgical Procedure for Raynaud's Disease. PG - e947488 LID - 10.12659/MSM.947488 [doi] LID - e947488 AB - BACKGROUND Raynaud's syndrome, a vascular dysfunction disorder characterized by paroxysmal spasms of small arteries in the extremities, has long attracted attention in the medical field. Despite the absence of a comprehensive understanding of its etiology, this condition is considered to be associated with impaired vascular endothelial function, neuromodulation disorders, and genetic factors. MATERIAL AND METHODS We conducted an analysis of data from 110 eligible patients, with approval from the Ethics Committee of our hospital. We assessed medical records, the Visual Analog Scale (VAS), Pittsburgh Sleep Quality Index (PSQI), Disabilities of the Arm, Shoulder, and Hand (DASH) scores, and adverse events linked to relapse. Survival analyses were carried out using the Kaplan-Meier method. Univariate and multivariate analyses were employed to identify factors associated with RD and to construct a prognostic line chart for postoperative recurrence. RESULTS In the 6-month survival analysis model, the following factors demonstrated statistical significance in multivariate analysis: primary disease (HR: 1.718; 95% CI: 1.044-2.829), surgical approach (HR: 0.454; 95% CI: 0.272-0.760), perfusion index difference (HR: 0.870; 95% CI: 0.76-0.994), and peripheral temperature difference (HR: 0.755; 95% CI: 0.615-0.928). In the 1-year survival analysis model, statistically significant factors in multivariate analysis included primary disease (HR: 1.881; 95% CI: 1.125-3.145), surgical approach (HR: 0.489; 95% CI: 0.291-0.821), perfusion index difference (HR: 0.866; 95% CI: 0.759-0.989), and peripheral temperature difference (HR: 0.757; 95% CI: 0.618-0.928). CONCLUSIONS Independent risk factors for postoperative recurrence of Reynaud's syndrome include the primary disease, surgical approach, changes in perfusion indices, and changes in peripheral temperature. FAU - Zhi, Tong AU - Zhi T AD - Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Xu, Miao AU - Xu M AD - Department of Anesthesiology and Pain Research Center, The Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. FAU - Kuang, Jiao AU - Kuang J AD - Department of Anesthesiology and Pain Research Center, The Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. FAU - Wei, Shirong AU - Wei S AD - Department of Anesthesiology and Pain Research Center, The Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. FAU - Yao, Ming AU - Yao M AD - Department of Anesthesiology and Pain Research Center, The Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. FAU - Ni, Huadong AU - Ni H AD - Department of Anesthesiology and Pain Research Center, The Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. LA - eng PT - Journal Article DEP - 20250522 PL - United States TA - Med Sci Monit JT - Medical science monitor : international medical journal of experimental and clinical research JID - 9609063 SB - IM MH - Humans MH - *Raynaud Disease/surgery/physiopathology MH - Female MH - Male MH - Recurrence MH - Middle Aged MH - Adult MH - Risk Factors MH - Kaplan-Meier Estimate MH - Aged MH - Prognosis PMC - PMC12108146 COIS- Conflict of interest: None declared EDAT- 2025/05/22 06:28 MHDA- 2025/05/22 06:29 PMCR- 2025/05/22 CRDT- 2025/05/22 01:23 PHST- 2025/05/22 06:29 [medline] PHST- 2025/05/22 06:28 [pubmed] PHST- 2025/05/22 01:23 [entrez] PHST- 2025/05/22 00:00 [pmc-release] AID - 947488 [pii] AID - 10.12659/MSM.947488 [doi] PST - epublish SO - Med Sci Monit. 2025 May 22;31:e947488. doi: 10.12659/MSM.947488. PMID- 20022066 OWN - NLM STAT- MEDLINE DCOM- 20100715 LR - 20100405 IS - 0025-7753 (Print) IS - 0025-7753 (Linking) VI - 134 IP - 10 DP - 2010 Apr 10 TI - [On Raynaud's phenomenon]. PG - 470; author reply 470-1 LID - 10.1016/j.medcli.2009.06.067 [doi] FAU - Olivé, Alejandro AU - Olivé A FAU - García-Melchor, Emma AU - García-Melchor E LA - spa PT - Comment PT - Letter TT - A propósito del fenómeno de Raynaud. DEP - 20091221 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM CON - Med Clin (Barc). 2009 May 16;132(18):712-8. doi: 10.1016/j.medcli.2008.11.017. PMID: 19268319 MH - False Negative Reactions MH - False Positive Reactions MH - Humans MH - Raynaud Disease/*diagnosis/etiology MH - Risk Factors EDAT- 2009/12/22 06:00 MHDA- 2010/07/16 06:00 CRDT- 2009/12/22 06:00 PHST- 2009/06/25 00:00 [received] PHST- 2009/06/30 00:00 [accepted] PHST- 2009/12/22 06:00 [entrez] PHST- 2009/12/22 06:00 [pubmed] PHST- 2010/07/16 06:00 [medline] AID - S0025-7753(09)01427-4 [pii] AID - 10.1016/j.medcli.2009.06.067 [doi] PST - ppublish SO - Med Clin (Barc). 2010 Apr 10;134(10):470; author reply 470-1. doi: 10.1016/j.medcli.2009.06.067. Epub 2009 Dec 21. PMID- 28992170 OWN - NLM STAT- MEDLINE DCOM- 20171012 LR - 20181113 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 56 IP - suppl_5 DP - 2017 Sep 1 TI - Points to consider-Raynaud's phenomenon in systemic sclerosis. PG - v45-v48 LID - 10.1093/rheumatology/kex199 [doi] AB - RP is an exaggerated vasospastic response to cold or emotion. Randomized, double-blind, placebo-controlled trials with either parallel group or cross-over trials should be mainly considered. Cross-over design, which is good for early phase trials of immediate or very short-term outcomes, is important in a condition as heterogeneous as RP: a wash-out period between treatment arms should always be included to minimize the possibility of a period (carry-over) effect. Duration of RP trials is usually constrained by the need to complete these over a single season, usually winter when the weather is colder. For cross-over trials, each treatment arm tends to be 4 weeks or less. Frequency and duration of attacks, and the Raynaud's Condition Score are widely used outcome measures. There is increasing interest in physiological laboratory endpoints, for example laser Doppler imaging at least for early phase trials. CI - © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS, University of Genova, Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital. AD - Faculty of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Furst, Daniel E AU - Furst DE AD - Department of Rheumatology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Medicine University of Michigan, University of Michigan Scleroderma Program, Ann Arbor, MI. FAU - Herrick, Ariane L AU - Herrick AL AD - The University of Manchester, Salford Royal NHS Foundation Trust, Manchester and NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, UK. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Clinical Trials as Topic/*methods MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - Scleroderma, Systemic/*complications PMC - PMC5850109 OTO - NOTNLM OT - Raynaud’s phenomenon OT - clinical trials OT - connective tissue diseases OT - digital ulcers OT - gangrene OT - microcirculation OT - nailfold capillaroscopy OT - rheumatic diseases OT - systemic sclerosis OT - vasodilators EDAT- 2017/10/11 06:00 MHDA- 2017/10/13 06:00 PMCR- 2018/09/01 CRDT- 2017/10/10 06:00 PHST- 2015/07/01 00:00 [received] PHST- 2017/10/10 06:00 [entrez] PHST- 2017/10/11 06:00 [pubmed] PHST- 2017/10/13 06:00 [medline] PHST- 2018/09/01 00:00 [pmc-release] AID - 4345757 [pii] AID - kex199 [pii] AID - 10.1093/rheumatology/kex199 [doi] PST - ppublish SO - Rheumatology (Oxford). 2017 Sep 1;56(suppl_5):v45-v48. doi: 10.1093/rheumatology/kex199. PMID- 31712897 OWN - NLM STAT- MEDLINE DCOM- 20191202 LR - 20200119 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 78 IP - 10 DP - 2019 Dec TI - [Raynaud's phenomenon : Practical management]. PG - 967-978 LID - 10.1007/s00393-019-00723-z [doi] AB - Raynaud's phenomenon (RP) is a frequent and painful vasospasm of small arteries localized in acral body regions (most frequently the fingers). The more frequent so-called primary RP is caused merely by a functional dysregulation of the tonus of vascular walls. In contrast, the rarer secondary RP is additionally associated with structural abnormalities of blood vessels. Knowledge of RP is important for rheumatologists because secondary RP can be associated with the presence or development of severe underlying diseases, especially with systemic sclerosis. Thus, the rheumatologist has to be aware of this condition. In this article the diagnostic procedures and the most important treatment approaches are summarized. FAU - Drerup, C AU - Drerup C AD - Klinik für Hautkrankheiten - Allgemeine Dermatologie und Venerologie, Universitätsklinikum Münster, Von-Esmarch-Str. 58, 48149, Münster, Deutschland. FAU - Maier, A AU - Maier A AD - Rheumatologisches Kompetenzzentrum Nordwestdeutschland, Klinik für Rheumatologie, St. Josef-Stift Sendenhorst, Westtor 7, 48324, Sendenhorst, Deutschland. FAU - Ehrchen, J AU - Ehrchen J AD - Klinik für Hautkrankheiten - Allgemeine Dermatologie und Venerologie, Universitätsklinikum Münster, Von-Esmarch-Str. 58, 48149, Münster, Deutschland. jan.ehrchen@ukmuenster.de. LA - ger PT - Journal Article TT - Raynaud-Phänomen : Praktisches Management. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Humans MH - *Raynaud Disease/complications/therapy MH - Rheumatologists MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Acral circulatory disorder OT - Capillaroscopy OT - Cyanosis OT - Rheumatic diseases OT - Systemic sclerosis EDAT- 2019/11/13 06:00 MHDA- 2019/12/04 06:00 CRDT- 2019/11/13 06:00 PHST- 2019/11/13 06:00 [pubmed] PHST- 2019/12/04 06:00 [medline] PHST- 2019/11/13 06:00 [entrez] AID - 10.1007/s00393-019-00723-z [pii] AID - 10.1007/s00393-019-00723-z [doi] PST - ppublish SO - Z Rheumatol. 2019 Dec;78(10):967-978. doi: 10.1007/s00393-019-00723-z. PMID- 38442964 OWN - NLM STAT- MEDLINE DCOM- 20240307 LR - 20260306 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 17 IP - 3 DP - 2024 Mar 5 TI - Raynaud's phenomenon associated with psoriatic arthritis. LID - 10.1136/bcr-2023-258215 [doi] LID - e258215 AB - We report a case of Raynaud's phenomenon in a patient with psoriatic arthritis (PsA). A middle-aged right-handed housewife presented with complaints of severely painful hand discolouration for 1 week, which usually worsened with cold exposure. She was diagnosed with PsA 6 months earlier. Her PsA was well controlled with weekly methotrexate. Physical examination showed no features of scleroderma or skin necrosis of her right hand. Both radial pulses were strong and symmetrical. Her nailfolds were visibly normal. The extractable nuclear antigen panel and other blood investigations were negative for scleroderma and other possible causes of secondary Raynaud's phenomenon. Occupational or environmental factors were also excluded. Dermatoscope examination of the nailfolds revealed some areas of dilated capillary loops, areas of vascular sparing and proximal nail fold telangiectasia. The diagnosis of secondary Raynaud's phenomenon was made, and an oral calcium channel blocker was started. The patient had significant improvement in symptoms shortly afterwards. CI - © BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Che Rahim, Mohd Jazman AU - Che Rahim MJ AUID- ORCID: 0000-0003-1721-6329 AD - Internal Medicine, Hospital Universiti Sains Malaysia, Kota Bahru, Kelantan, Malaysia drjazman@usm.my. AD - Universiti Sains Malaysia - Kampus Kesihatan, Kota Bharu, Kelantan, Malaysia. FAU - Lim, Jo Anne AU - Lim JA AD - Internal Medicine, Hospital Universiti Sains Malaysia, Kota Bahru, Kelantan, Malaysia. AD - Universiti Sains Malaysia - Kampus Kesihatan, Kota Bharu, Kelantan, Malaysia. FAU - Wan Ghazali, Wan Syamimee AU - Wan Ghazali WS AUID- ORCID: 0000-0002-7872-4581 AD - Internal Medicine, Hospital Universiti Sains Malaysia, Kota Bahru, Kelantan, Malaysia. AD - Universiti Sains Malaysia - Kampus Kesihatan, Kota Bharu, Kelantan, Malaysia. LA - eng PT - Case Reports PT - Journal Article DEP - 20240305 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Calcium Channel Blockers) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Female MH - Middle Aged MH - Humans MH - *Arthritis, Psoriatic/complications/diagnosis/drug therapy MH - *Raynaud Disease/complications/diagnosis MH - Calcium Channel Blockers MH - Hand MH - Methotrexate MH - *Scleroderma, Localized PMC - PMC10916094 OTO - NOTNLM OT - Dermatology OT - Rheumatology COIS- Competing interests: None declared. EDAT- 2024/03/06 00:42 MHDA- 2024/03/07 06:43 PMCR- 2026/03/05 CRDT- 2024/03/05 20:53 PHST- 2024/03/07 06:43 [medline] PHST- 2024/03/06 00:42 [pubmed] PHST- 2024/03/05 20:53 [entrez] PHST- 2026/03/05 00:00 [pmc-release] AID - 17/3/e258215 [pii] AID - bcr-2023-258215 [pii] AID - 10.1136/bcr-2023-258215 [doi] PST - epublish SO - BMJ Case Rep. 2024 Mar 5;17(3):e258215. doi: 10.1136/bcr-2023-258215. PMID- 34420003 OWN - NLM STAT- MEDLINE DCOM- 20211028 LR - 20221129 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 33 IP - 6 DP - 2021 Nov 1 TI - Raynaud's phenomenon and digital ulcers: advances in evaluation and management. PG - 453-462 LID - 10.1097/BOR.0000000000000826 [doi] AB - PURPOSE OF REVIEW: The aim of this review is to give an update on advances in evaluation and management of systemic sclerosis (SSc)-related Raynaud's phenomenon and digital ulceration, focusing on reports from the last 18 months. The increasing recognition of the huge impact of Raynaud's phenomenon and of digital ulceration on the everyday lives of patients with SSc has sparked enthusiasm internationally to develop better outcome measures and better treatments, and so a review is timely. RECENT FINDINGS: There have been recent advances in the development of patient reported outcome instruments [e.g. the Hand Disability in Systemic Sclerosis-Digital Ulcers (HDISS-DU) instrument] and also in noninvasive imaging techniques, including thermography and laser Doppler methods. Improved outcome measures will facilitate future clinical trials, both early phase proof-of-concept and later phase trials. New insights have been gained into mechanisms of action and methods of administration of 'conventional' therapies, for example phosphodiesterase inhibitors and intravenous prostanoids. New treatment approaches are being investigated, including topical and procedural therapies. SUMMARY: Clinicians can look forward to seeing these advances translating into clinical benefit over the next 5 years. To help ensure this, they should strive whenever possible to recruit patients with SSc-related digital vasculopathy into observational studies and clinical trials. CI - Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Fingers MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Scleroderma, Systemic/complications/diagnosis/therapy MH - *Skin Ulcer/diagnosis/etiology/therapy MH - Ulcer EDAT- 2021/08/23 06:00 MHDA- 2021/10/29 06:00 CRDT- 2021/08/22 21:03 PHST- 2021/08/23 06:00 [pubmed] PHST- 2021/10/29 06:00 [medline] PHST- 2021/08/22 21:03 [entrez] AID - 00002281-202111000-00002 [pii] AID - 10.1097/BOR.0000000000000826 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2021 Nov 1;33(6):453-462. doi: 10.1097/BOR.0000000000000826. PMID- 26949933 OWN - NLM STAT- MEDLINE DCOM- 20171120 LR - 20250626 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 82 IP - 1 DP - 2016 Jul TI - Drug-induced Raynaud's phenomenon: beyond β-adrenoceptor blockers. PG - 6-16 LID - 10.1111/bcp.12912 [doi] AB - AIM: Drug-induced Raynaud's phenomenon (RP) has long been associated with the use of different drugs, including cancer chemotherapy or β-adrenoceptor blockers. However, sources report extremely variable prevalence and the level of evidence for each class is heterogeneous. Moreover, new signals are emerging from case reports and small series. Our objective was therefore to review available evidence about this adverse drug effect and to propose a mechanistic approach of drug-induced RP. METHODS: A systematic review of English and French language articles was performed through Medline (1946-2015) and Embase (1974-2015). Further relevant papers were identified from the reference lists of retrieved articles. RESULTS: We identified 12 classes of drugs responsible for RP, with a variety of underlying mechanisms such as increased sympathetic activation, endothelial dysfunction, neurotoxicity or decreased red blood cell deformability. Cisplatin and bleomycin were associated with the highest risk, followed by β-adrenoceptor blockers. Recent data suggest a possible involvement of tyrosine kinase inhibitors (TKI), through an unknown mechanism. CONCLUSION: Drug-induced RP is a probably underestimated adverse drug event, with limited available evidence regarding its prevalence. Although rare, serious complications like critical digital ischaemia have been reported. When these treatments are started in patients with a history of RP, careful monitoring must be made and, if possible, alternative therapies that do not alter peripheral blood flow should be considered. CI - © 2016 The British Pharmacological Society. FAU - Khouri, Charles AU - Khouri C AD - Pôle Santé Publique, Pharmacovigilance, CHU Grenoble-Alpes, F-38000, Grenoble. FAU - Blaise, Sophie AU - Blaise S AD - HP2, Univ. Grenoble Alpes, F-38000, Grenoble. AD - HP2, INSERM, F-38000, Grenoble. AD - CHU Grenoble-Alpes, Clinique de Médecine Vasculaire, F-38000, Grenoble. FAU - Carpentier, Patrick AU - Carpentier P AD - CHU Grenoble-Alpes, Clinique de Médecine Vasculaire, F-38000, Grenoble. FAU - Villier, Céline AU - Villier C AD - Pôle Santé Publique, Pharmacovigilance, CHU Grenoble-Alpes, F-38000, Grenoble. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - HP2, Univ. Grenoble Alpes, F-38000, Grenoble. AD - HP2, INSERM, F-38000, Grenoble. AD - Pôle Recherche, Pharmacologie Clinique Inserm CIC1406, CHU Grenoble-Alpes, F-38000, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AD - HP2, Univ. Grenoble Alpes, F-38000, Grenoble. AD - HP2, INSERM, F-38000, Grenoble. AD - Pôle Recherche, Pharmacologie Clinique Inserm CIC1406, CHU Grenoble-Alpes, F-38000, Grenoble, France. LA - eng PT - Journal Article PT - Systematic Review DEP - 20160407 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - 0 (Adrenergic beta-Antagonists) SB - IM MH - Adrenergic beta-Antagonists/*adverse effects MH - Animals MH - Drug-Related Side Effects and Adverse Reactions/*epidemiology/physiopathology MH - Humans MH - Prevalence MH - Raynaud Disease/*chemically induced/physiopathology PMC - PMC4917788 OTO - NOTNLM OT - Raynaud's phenomenon OT - peripheral vasoconstriction OT - β-adrenoceptor blockers EDAT- 2016/03/08 06:00 MHDA- 2017/11/29 06:00 PMCR- 2017/07/01 CRDT- 2016/03/08 06:00 PHST- 2016/01/21 00:00 [received] PHST- 2016/02/12 00:00 [revised] PHST- 2016/02/14 00:00 [accepted] PHST- 2016/03/08 06:00 [entrez] PHST- 2016/03/08 06:00 [pubmed] PHST- 2017/11/29 06:00 [medline] PHST- 2017/07/01 00:00 [pmc-release] AID - BCP12912 [pii] AID - 10.1111/bcp.12912 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2016 Jul;82(1):6-16. doi: 10.1111/bcp.12912. Epub 2016 Apr 7. PMID- 35064670 OWN - NLM STAT- MEDLINE DCOM- 20220420 LR - 20220531 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 72 IP - 3 DP - 2022 Apr 19 TI - Carpal tunnel syndrome and Raynaud's phenomenon: a narrative review. PG - 170-176 LID - 10.1093/occmed/kqab158 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) is a recognized symptom associated with carpal tunnel syndrome (CTS) and is also the vascular manifestation of hand arm vibration syndrome (HAVS). The symptoms of HAVS and CTS are such that there is a significant possibility of misdiagnosis and an incorrect attribution of vascular and sensory symptoms. An understanding of the relationship between RP and CTS is essential when undertaking health surveillance of vibration-exposed workers presenting with combined vascular and sensory symptoms. AIMS: To clarify the relationship between CTS and Raynaud's phenomenon. METHODS: A systematic search was undertaken of studies that reviewed links between CTS and Raynaud's phenomenon (RP). RESULTS: A total of 4170 papers were identified, with 21 articles that were then reviewed in full, including 1 meta-analysis of 8 studies. Eighteen papers, not included in the meta-analysis, were found including 3 case control studies, 9 case reports, 2 prospective studies and 4 retrospective reviews. Papers were reviewed on the basis of the diagnostic criteria used for CTS and RP. Our review of the literature confirms a substantial body of evidence of a relationship between RP and CTS. CONCLUSIONS: It is recommended that assessment of vibration exposed individuals who report concurrent RP and separate sensory symptoms suggestive of, or compatible with CTS, should formally exclude CTS before attributing symptoms to HAVS. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Cooke, Roger AU - Cooke R AD - Institute of Occupational and Environmental Medicine, University of Birmingham, Birmingham, United Kingdom. FAU - Lawson, Ian AU - Lawson I AD - Occupational Health Physician (ret'd), Belper, UK. FAU - Gillibrand, Susanna AU - Gillibrand S AD - Independent Occupational Physician, Southampton,UK. FAU - Cooke, Andrew AU - Cooke A AD - Clarity Healthcare Ltd, Stockport, UK. LA - eng PT - Journal Article PT - Review PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - *Carpal Tunnel Syndrome/diagnosis/etiology MH - *Hand-Arm Vibration Syndrome/complications/diagnosis MH - Humans MH - Prospective Studies MH - *Raynaud Disease/diagnosis/etiology MH - Retrospective Studies MH - *Vascular Diseases MH - Vibration/adverse effects OTO - NOTNLM OT - Raynaud’s OT - carpal tunnel syndrome OT - hand arm vibration OT - vibration white finger EDAT- 2022/01/23 06:00 MHDA- 2022/04/21 06:00 CRDT- 2022/01/22 08:36 PHST- 2022/01/23 06:00 [pubmed] PHST- 2022/04/21 06:00 [medline] PHST- 2022/01/22 08:36 [entrez] AID - 6513858 [pii] AID - 10.1093/occmed/kqab158 [doi] PST - ppublish SO - Occup Med (Lond). 2022 Apr 19;72(3):170-176. doi: 10.1093/occmed/kqab158. PMID- 32672856 OWN - NLM STAT- MEDLINE DCOM- 20210428 LR - 20210428 IS - 1529-8019 (Electronic) IS - 1396-0296 (Linking) VI - 33 IP - 6 DP - 2020 Nov TI - Public interest in Raynaud's phenomenon: A Google Trends analysis. PG - e14017 LID - 10.1111/dth.14017 [doi] AB - Raynaud's phenomenon is a common disorder affecting body extremities and other vascular beds. As infodemiological methods have developed, online search tools could be used to explore the public interest of the disease. The study aimed to determine the annual trend, seasonal pattern, and associated topics of Raynaud's phenomenon. Google Trends was used to collect the data. "Raynaud syndrome" was selected as the search term. Data on monthly relative search volume (RSV) were collected from four selected countries (United States, United Kingdom, Australia, and New Zealand) and globally. Related topics were obtained, and annual-related topics were also collected for analysis. The maximum RSV appeared in January 2019, and the minimum value was observed in August 2011. The peak for RSV occurred in winter, and the bottom appeared in summer. In top related topics, "Maurice Raynaud" was the most related. In rising related topics, disease manifestations and autoimmune connective diseases were highly concerned. For annual-related topics, associated diseases were attracting more attention over time. The population is interested in related diseases, pathogenesis, and treatment. There was a peak in winter for searching and supported the importance of season variation on the impact of Raynaud's phenomenon. CI - © 2020 Wiley Periodicals LLC. FAU - Wang, Yuanzhuo AU - Wang Y AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. AD - Eight-Year MD Program, Peking Union Medical College, Beijing, China. FAU - Zhang, Hanlin AU - Zhang H AUID- ORCID: 0000-0001-5065-4086 AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. AD - Eight-Year MD Program, Peking Union Medical College, Beijing, China. FAU - Zheng, Qingyue AU - Zheng Q AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. AD - Eight-Year MD Program, Peking Union Medical College, Beijing, China. FAU - Tang, Keyun AU - Tang K AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. AD - Eight-Year MD Program, Peking Union Medical College, Beijing, China. FAU - Sun, Qiuning AU - Sun Q AUID- ORCID: 0000-0002-1912-341X AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. LA - eng PT - Journal Article DEP - 20200806 PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 SB - IM MH - Australia/epidemiology MH - Humans MH - *Raynaud Disease/diagnosis/epidemiology OTO - NOTNLM OT - Google Trends OT - Raynaud's phenomenon OT - infodemiological study OT - public interest EDAT- 2020/07/17 06:00 MHDA- 2021/04/29 06:00 CRDT- 2020/07/17 06:00 PHST- 2020/05/26 00:00 [received] PHST- 2020/06/26 00:00 [revised] PHST- 2020/07/11 00:00 [accepted] PHST- 2020/07/17 06:00 [pubmed] PHST- 2021/04/29 06:00 [medline] PHST- 2020/07/17 06:00 [entrez] AID - 10.1111/dth.14017 [doi] PST - ppublish SO - Dermatol Ther. 2020 Nov;33(6):e14017. doi: 10.1111/dth.14017. Epub 2020 Aug 6. PMID- 26869017 OWN - NLM STAT- MEDLINE DCOM- 20180720 LR - 20260127 IS - 1995-9133 (Electronic) IS - 1684-1182 (Linking) VI - 50 IP - 6 DP - 2017 Dec TI - The role of endothelial microparticles in autoimmune disease patients with Raynaud's phenomenon. PG - 857-862 LID - S1684-1182(16)00011-6 [pii] LID - 10.1016/j.jmii.2015.12.010 [doi] AB - BACKGROUND AND AIM: Raynaud's phenomenon (RP) is a microvascular disorder characterized by episodic peripheral vasospasm and ischemia and is commonly found in patients with autoimmune diseases (AID). The vasomotor homoeostasis and endothelial cells damage are involved in RP. Endothelial microparticles (EMPs) may act as a biomarker for endothelial damage. The aim of this study is to investigate the correlation between the levels of microparticles (MPs) and microvasculopathy in AID with RP. METHODS: Thirty-seven patients with AID and RP (RP group) and 27 patients with AID but without RP (non-RP group) were enrolled. The microvasculopathy score of RP was graded by nailfold capillary microscopy. The plasma levels of MPs were measured by flow cytometry utilizing specific labels for endothelial MPs (CD105 and CD144) and annexin V staining for phosphatidylserine bearing-MPs (annexin V+MPs). RESULTS: The levels of circulating EMPs (CD105+ p = 0.005, CD144+ p = 0.004), and the annexin V+ MPs (p < 0.001) were significantly elevated in the RP group compared with the non-RP group. Moreover, the high microvasculopathy scores were closely related with annexinV+ MPs levels in the RP group (p = 0.041). CONCLUSIONS: Levels of circulating EMPs and annexin V+ MPs are elevated in AID patients with RP indicate the endothelial damage and endothelial dysfunctions. In addition, levels of annexin V+ MPs can predict the severity of microvasculopathy in AID with RP. CI - Copyright © 2016. Published by Elsevier B.V. FAU - Wu, Yeong-Jian Jan AU - Wu YJ AD - Department of Internal Medicine, Division of Allergy, Immunology and Rheumatology, Chang Gung Memorial Hospital at Keelung, College of Medicine, Chang Gung University, Taoyuan, Taiwan, ROC. FAU - Hua, Chung-Ching AU - Hua CC AD - Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, Chang Gung Memorial Hospital at Keelung, Taoyuan, Taiwan, ROC. FAU - Chen, Ji-Yih AU - Chen JY AD - Department of Internal Medicine, Division of Allergy, Immunology and Rheumatology, Chang Gung Memorial Hospital at Linkou, College of Medicine, Chang Gung University, Taoyuan, Taiwan, ROC. FAU - Chang, Yao-Wen AU - Chang YW AD - Graduate Institute of Biomedical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan, ROC. FAU - Tseng, Jo-Chi AU - Tseng JC AD - Department of Emergency Medicine, Chang Gung Memory Hospital at Linkou, Taoyuan, Taiwan, ROC. Electronic address: jochi77@cgmh.org.tw. LA - eng PT - Journal Article DEP - 20160112 PL - England TA - J Microbiol Immunol Infect JT - Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi JID - 100956211 RN - 0 (Annexin A5) RN - 0 (Antigens, CD) RN - 0 (Biomarkers) RN - 0 (Cadherins) RN - 0 (Endoglin) RN - 0 (Cadherin 5) RN - 0 (ENG protein, human) SB - IM MH - Annexin A5/*blood MH - Antigens, CD/blood MH - Autoimmune Diseases/blood/complications/*pathology MH - Biomarkers/blood MH - Cadherins/blood MH - Cell-Derived Microparticles/*pathology MH - Endoglin/blood MH - Endothelial Cells/*pathology MH - Female MH - Humans MH - Microvessels/cytology/*pathology MH - Middle Aged MH - Raynaud Disease/blood/complications/*pathology MH - Cadherin 5 OTO - NOTNLM OT - Raynaud's phenomenon OT - annexin V OT - autoimmune disease OT - endothelial microparticles OT - microvasculopathy EDAT- 2016/02/13 06:00 MHDA- 2018/07/22 06:00 CRDT- 2016/02/13 06:00 PHST- 2015/03/02 00:00 [received] PHST- 2015/09/17 00:00 [revised] PHST- 2015/12/29 00:00 [accepted] PHST- 2016/02/13 06:00 [pubmed] PHST- 2018/07/22 06:00 [medline] PHST- 2016/02/13 06:00 [entrez] AID - S1684-1182(16)00011-6 [pii] AID - 10.1016/j.jmii.2015.12.010 [doi] PST - ppublish SO - J Microbiol Immunol Infect. 2017 Dec;50(6):857-862. doi: 10.1016/j.jmii.2015.12.010. Epub 2016 Jan 12. PMID- 29327980 OWN - NLM STAT- MEDLINE DCOM- 20181011 LR - 20220410 IS - 1557-9034 (Electronic) IS - 1092-6429 (Linking) VI - 28 IP - 6 DP - 2018 Jun TI - Effects of Endoscopic Thoracic Sympathectomy on Raynaud's Disease. PG - 726-729 LID - 10.1089/lap.2017.0634 [doi] AB - INTRODUCTION: Raynaud's disease is a disorder that is characterized by attacks of pain, cyanosis, redness, and numbness in the upper extremities caused by vasospasm of digital arteries due to cold or emotional stress. We aimed at demonstrating our experiences with endoscopic thoracic sympathectomy (ETS) in the treatment of Raynaud's disease. METHODS: From 48 patients who underwent ETS for various reasons at our department between January 2014 and January 2015, we reviewed 9 patients with Raynaud's disease (18.7%) with respect to their demographic characteristics such as gender and age, postoperative complications, short-term results, side effects, recurrence of symptoms, and long-terms results. RESULTS: The symptoms and findings reappeared and the number and dosage of the drugs used returned to their preoperative levels in 66.6% of the patients at month 6, and in all patients except 1 at the end of the 1st year. CONCLUSION: ETS should be considered an ultimate choice for patients with Raynaud's disease who have treatment-resistant severe symptoms and serious complications, disturbed social and daily lives, and impaired quality of life, and all patients should be properly informed before the surgery about the possibility of a high rate of recurrence. FAU - Karapolat, Sami AU - Karapolat S AD - Department of Thoracic Surgery, Karadeniz Technical University Medical School , Trabzon, Turkey . FAU - Turkyilmaz, Atila AU - Turkyilmaz A AD - Department of Thoracic Surgery, Karadeniz Technical University Medical School , Trabzon, Turkey . FAU - Tekinbas, Celal AU - Tekinbas C AD - Department of Thoracic Surgery, Karadeniz Technical University Medical School , Trabzon, Turkey . LA - eng PT - Journal Article DEP - 20180112 PL - United States TA - J Laparoendosc Adv Surg Tech A JT - Journal of laparoendoscopic & advanced surgical techniques. Part A JID - 9706293 SB - IM MH - Adult MH - Endoscopy/adverse effects/*methods MH - Female MH - Humans MH - Male MH - Postoperative Complications/epidemiology MH - Quality of Life MH - Raynaud Disease/*surgery MH - Recurrence MH - Sympathectomy/adverse effects/*methods MH - Thoracic Surgical Procedures/adverse effects/*methods MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - Raynaud's disease OT - quality of life OT - recurrence OT - sympathectomy EDAT- 2018/01/13 06:00 MHDA- 2018/10/12 06:00 CRDT- 2018/01/13 06:00 PHST- 2018/01/13 06:00 [pubmed] PHST- 2018/10/12 06:00 [medline] PHST- 2018/01/13 06:00 [entrez] AID - 10.1089/lap.2017.0634 [doi] PST - ppublish SO - J Laparoendosc Adv Surg Tech A. 2018 Jun;28(6):726-729. doi: 10.1089/lap.2017.0634. Epub 2018 Jan 12. PMID- 20407628 OWN - NLM STAT- MEDLINE DCOM- 20110127 LR - 20260128 IS - 1178-2048 (Electronic) IS - 1176-6344 (Print) IS - 1176-6344 (Linking) VI - 6 DP - 2010 Apr 15 TI - Recent achievements in the management of Raynaud's phenomenon. PG - 207-14 AB - Raynaud's phenomenon is a clinical disorder with episodic digital ischemic vasospasm triggered by cold- or emotional-stress. It was first mentioned by Maurice Raynaud in 1862 describing "a local asphyxia of the extremities" and was further divided into primary Raynaud's disease and secondary Raynaud's phenomenon, which is often related to connective tissue diseases, but also physical or chemical strain. Though pathophysiology of Raynaud's phenomenon is still poorly understood, systemic and local vascular effects are most likely to be involved in primary Raynaud's disease. In secondary Raynaud's phenomenon additional abnormalities in vascular structure and function may play the major role. Thus, medical treatment of Raynaud's phenomenon remains unsatisfactory, due to limited understanding of pathophysiological mechanisms. This review addresses current evidence for medical treatment of primary and secondary Raynaud's phenomenon with regard to pathophysiological mechanisms as well as future perspectives. FAU - Baumhäkel, Magnus AU - Baumhäkel M AD - Klinik für Innere Medizin III, Kardiologie, Angiologie und Internistische Intensivmedizin, Universitätsklinikum des Saarlandes, Homburg/Saar, Germany. magnus@baumhaekel.de FAU - Böhm, Michael AU - Böhm M LA - eng PT - Journal Article PT - Review DEP - 20100415 PL - New Zealand TA - Vasc Health Risk Manag JT - Vascular health and risk management JID - 101273479 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Vasodilator Agents) RN - EC 2.7.11.1 (rho-Associated Kinases) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Algorithms MH - Angiotensin Receptor Antagonists/therapeutic use MH - Endothelin Receptor Antagonists MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use MH - Raynaud Disease/etiology/physiopathology/*therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Vasoconstriction/physiology MH - Vasodilation/physiology MH - Vasodilator Agents/therapeutic use MH - rho-Associated Kinases/antagonists & inhibitors PMC - PMC2856576 OTO - NOTNLM OT - Raynaud’s disease OT - mechanisms OT - treatment EDAT- 2010/04/22 06:00 MHDA- 2011/01/29 06:00 PMCR- 2010/04/15 CRDT- 2010/04/22 06:00 PHST- 2010/03/27 00:00 [received] PHST- 2010/04/22 06:00 [entrez] PHST- 2010/04/22 06:00 [pubmed] PHST- 2011/01/29 06:00 [medline] PHST- 2010/04/15 00:00 [pmc-release] AID - vhrm-6-207 [pii] AID - 10.2147/vhrm.s5255 [doi] PST - epublish SO - Vasc Health Risk Manag. 2010 Apr 15;6:207-14. doi: 10.2147/vhrm.s5255. PMID- 17103176 OWN - NLM STAT- MEDLINE DCOM- 20070801 LR - 20260128 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 27 IP - 6 DP - 2007 Apr TI - Citalopram-induced Raynaud's phenomenon. PG - 599-601 FAU - Peiró, A M AU - Peiró AM AD - Clinical Pharmacology Unit, Hospital General de Alicante, c/Pintor Baeza s/n, 03010, Alicante, Spain. peiro_ana@gva.es FAU - Margarit, C AU - Margarit C FAU - Torra, M AU - Torra M LA - eng PT - Case Reports PT - Journal Article DEP - 20061114 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Analgesics) RN - 0DHU5B8D6V (Citalopram) RN - 0 (Selective Serotonin Reuptake Inhibitors) SB - IM MH - Adult MH - Analgesics/therapeutic use MH - Citalopram/*adverse effects MH - Depressive Disorder/*drug therapy MH - Female MH - Humans MH - Neuralgia/drug therapy/*psychology MH - Raynaud Disease/*chemically induced MH - Selective Serotonin Reuptake Inhibitors/*adverse effects EDAT- 2006/11/15 09:00 MHDA- 2007/08/02 09:00 CRDT- 2006/11/15 09:00 PHST- 2006/09/21 00:00 [received] PHST- 2006/10/06 00:00 [accepted] PHST- 2006/11/15 09:00 [pubmed] PHST- 2007/08/02 09:00 [medline] PHST- 2006/11/15 09:00 [entrez] AID - 10.1007/s00296-006-0254-9 [doi] PST - ppublish SO - Rheumatol Int. 2007 Apr;27(6):599-601. doi: 10.1007/s00296-006-0254-9. Epub 2006 Nov 14. PMID- 17608774 OWN - NLM STAT- MEDLINE DCOM- 20080103 LR - 20151119 IS - 1600-0420 (Electronic) IS - 1395-3907 (Linking) VI - 85 IP - 8 DP - 2007 Dec TI - Fluorescein-induced Raynaud's phenomenon. PG - 910-1 FAU - Blaise, Pierre AU - Blaise P FAU - Ribbens, Clio AU - Ribbens C FAU - Rakic, Jean-Marie AU - Rakic JM LA - eng PT - Case Reports PT - Letter DEP - 20070630 PL - Denmark TA - Acta Ophthalmol Scand JT - Acta ophthalmologica Scandinavica JID - 9507578 RN - 0 (Fluorescent Dyes) RN - TPY09G7XIR (Fluorescein) SB - IM MH - Aged MH - Fluorescein/administration & dosage/*adverse effects MH - Fluorescein Angiography/adverse effects MH - Fluorescent Dyes/administration & dosage/*adverse effects MH - Humans MH - Injections, Intravenous MH - Male MH - Raynaud Disease/*chemically induced MH - Retinal Vein Occlusion/diagnosis EDAT- 2007/07/05 09:00 MHDA- 2008/01/04 09:00 CRDT- 2007/07/05 09:00 PHST- 2007/07/05 09:00 [pubmed] PHST- 2008/01/04 09:00 [medline] PHST- 2007/07/05 09:00 [entrez] AID - AOS953 [pii] AID - 10.1111/j.1600-0420.2007.00953.x [doi] PST - ppublish SO - Acta Ophthalmol Scand. 2007 Dec;85(8):910-1. doi: 10.1111/j.1600-0420.2007.00953.x. Epub 2007 Jun 30. PMID- 38174479 OWN - NLM STAT- MEDLINE DCOM- 20240105 LR - 20240111 IS - 1998-4022 (Electronic) IS - 0028-3886 (Linking) VI - 71 IP - 6 DP - 2023 Nov-Dec TI - Spontaneous Regression of Painless Carotid Artery Dissection in a Patient With Raynaud's Disease. PG - 1276-1277 LID - 10.4103/0028-3886.391341 [doi] FAU - An, Min Ho AU - An MH AD - College of Medicine, Catholic Kwandong University, Incheon, South Korea. FAU - Ku, Bon D AU - Ku BD AD - Department of Neurology, International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Incheon; Institute for Cognitive Intervention, International St. Mary's Hospital, South Korea. FAU - An, Sang Joon AU - An SJ AD - Department of Neurology, International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Incheon, South Korea. LA - eng PT - Letter PL - India TA - Neurol India JT - Neurology India JID - 0042005 SB - IM MH - Humans MH - Remission, Spontaneous MH - *Raynaud Disease/complications MH - *Carotid Artery Diseases MH - Carotid Arteries COIS- None EDAT- 2024/01/04 11:44 MHDA- 2024/01/05 06:42 CRDT- 2024/01/04 04:48 PHST- 2024/01/05 06:42 [medline] PHST- 2024/01/04 11:44 [pubmed] PHST- 2024/01/04 04:48 [entrez] AID - ni_2023_71_6_1276_391341 [pii] AID - 10.4103/0028-3886.391341 [doi] PST - ppublish SO - Neurol India. 2023 Nov-Dec;71(6):1276-1277. doi: 10.4103/0028-3886.391341. PMID- 16902282 OWN - NLM STAT- MEDLINE DCOM- 20061205 LR - 20161124 IS - 1018-8665 (Print) IS - 1018-8665 (Linking) VI - 213 IP - 2 DP - 2006 TI - Do the telangiectases of hereditary hemorrhagic telangiectasia and the calcinosis, Raynaud's disease, sclerodactyly, telangiectasia variant of scleroderma have a common etiology? PG - 81-2 FAU - Braverman, Irwin M AU - Braverman IM LA - eng PT - Comment PT - Editorial PL - Switzerland TA - Dermatology JT - Dermatology (Basel, Switzerland) JID - 9203244 RN - 0 (Antigens, CD) RN - 0 (ENG protein, human) RN - 0 (Endoglin) RN - 0 (Receptors, Cell Surface) SB - IM CON - Dermatology. 2006;213(2):88-92. doi: 10.1159/000093846. PMID: 16902284 MH - Antigens, CD/metabolism MH - Calcinosis/diagnosis/*etiology/metabolism MH - Diagnosis, Differential MH - Endoglin MH - Humans MH - Raynaud Disease/diagnosis/*etiology/metabolism MH - Receptors, Cell Surface/metabolism MH - Scleroderma, Limited/diagnosis/*etiology/metabolism MH - Skin/metabolism/*pathology MH - Telangiectasia, Hereditary Hemorrhagic/diagnosis/*etiology/metabolism EDAT- 2006/08/12 09:00 MHDA- 2006/12/09 09:00 CRDT- 2006/08/12 09:00 PHST- 2006/08/12 09:00 [pubmed] PHST- 2006/12/09 09:00 [medline] PHST- 2006/08/12 09:00 [entrez] AID - 93844 [pii] AID - 10.1159/000093844 [doi] PST - ppublish SO - Dermatology. 2006;213(2):81-2. doi: 10.1159/000093844. PMID- 27269655 OWN - NLM STAT- MEDLINE DCOM- 20190705 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 84 IP - 2 DP - 2017 Mar TI - Raynaud's phenomenon of the tongue. PG - 231 LID - S1297-319X(16)30064-1 [pii] LID - 10.1016/j.jbspin.2016.02.031 [doi] FAU - Grémain, Vincent AU - Grémain V AD - Department of Internal Medicine, Rouen University Hospital, Rouen, 1, rue Germont, 76031 Rouen cedex, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, 76183 Rouen cedex, France. FAU - Richard, Laetitia AU - Richard L AD - Department of Internal Medicine, Rouen University Hospital, Rouen, 1, rue Germont, 76031 Rouen cedex, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, 76183 Rouen cedex, France. FAU - Langlois, Vincent AU - Langlois V AD - Department of Internal Medicine, Rouen University Hospital, Rouen, 1, rue Germont, 76031 Rouen cedex, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, 76183 Rouen cedex, France. FAU - Marie, Isabelle AU - Marie I AD - Department of Internal Medicine, Rouen University Hospital, Rouen, 1, rue Germont, 76031 Rouen cedex, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, 76183 Rouen cedex, France. Electronic address: isabelle.marie@chu-rouen.fr. LA - eng PT - Case Reports PT - Journal Article DEP - 20160603 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Adult MH - Female MH - Humans MH - Rare Diseases MH - Raynaud Disease/*complications/*diagnosis MH - Risk Assessment MH - Scleroderma, Systemic/*complications/diagnosis MH - Severity of Illness Index MH - Tongue/*physiopathology EDAT- 2016/06/09 06:00 MHDA- 2019/07/06 06:00 CRDT- 2016/06/09 06:00 PHST- 2016/02/08 00:00 [received] PHST- 2016/02/26 00:00 [accepted] PHST- 2016/06/09 06:00 [pubmed] PHST- 2019/07/06 06:00 [medline] PHST- 2016/06/09 06:00 [entrez] AID - S1297-319X(16)30064-1 [pii] AID - 10.1016/j.jbspin.2016.02.031 [doi] PST - ppublish SO - Joint Bone Spine. 2017 Mar;84(2):231. doi: 10.1016/j.jbspin.2016.02.031. Epub 2016 Jun 3. PMID- 34415235 OWN - NLM STAT- MEDLINE DCOM- 20211025 LR - 20211025 IS - 2242-3982 (Electronic) IS - 1239-9736 (Print) IS - 1239-9736 (Linking) VI - 80 IP - 1 DP - 2021 Dec TI - Occupational noise exposure and Raynaud's phenomenon: a nested case-control study. PG - 1969745 LID - 10.1080/22423982.2021.1969745 [doi] LID - 1969745 AB - The primary aim of this study was to determine if self-reported occupational noise exposure was associated with Raynaud's phenomenon. In northern Sweden, a nested case-control study was performed on subjects reporting Raynaud's phenomenon (N=461), and controls (N=763) matched by age, sex and geographical location. The response rate to the exposure questionnaire was 79.2%. The study showed no statistically significant association between occupational noise exposure and reporting Raynaud's phenomenon (OR 1.10; 95% CI 0.83-1.46) in simple analyses. However, there was a trend towards increasing OR for Raynaud's phenomenon with increasing noise exposure, although not statistically significant. Also, there was a significant association between noise exposure and hearing loss (OR 2.76; 95% CI 2.00-3.81), and hearing loss was associated with reporting Raynaud's phenomenon (OR 1.52; 95% CI 1.03-2.23) in a multiple regression model. In conclusion, self-reported occupational noise exposure was not statistically significantly associated with Raynaud's phenomenon, but there was a dose-effect trend. In addition, the multiple model showed a robust association between hearing loss and Raynaud's phenomenon. These findings offer some support for a common pathophysiological background for Raynaud's phenomenon and hearing loss among noise-exposed workers, possibly through noise-induced vasoconstriction. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Abu Mdaighem, Mahmoud AU - Abu Mdaighem M AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Pettersson, Hans AU - Pettersson H AUID- ORCID: 0000-0001-7077-2389 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Int J Circumpolar Health JT - International journal of circumpolar health JID - 9713056 SB - IM MH - Case-Control Studies MH - Humans MH - *Noise, Occupational/adverse effects MH - *Occupational Exposure/adverse effects MH - *Raynaud Disease/diagnosis/epidemiology/etiology MH - Surveys and Questionnaires PMC - PMC8381957 OTO - NOTNLM OT - (Mesh): Raynaud disease OT - Sweden OT - hand-arm vibration syndrome OT - hearing loss OT - heredity OT - noise OT - occupational COIS- No potential conflict of interest was reported by the author(s). EDAT- 2021/08/21 06:00 MHDA- 2021/10/26 06:00 PMCR- 2021/01/01 CRDT- 2021/08/20 12:13 PHST- 2021/08/20 12:13 [entrez] PHST- 2021/08/21 06:00 [pubmed] PHST- 2021/10/26 06:00 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 1969745 [pii] AID - 10.1080/22423982.2021.1969745 [doi] PST - ppublish SO - Int J Circumpolar Health. 2021 Dec;80(1):1969745. doi: 10.1080/22423982.2021.1969745. PMID- 38771559 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20260508 IS - 1668-3501 (Electronic) IS - 0325-0075 (Linking) VI - 122 IP - 5 DP - 2024 Oct 1 TI - Raynaud's phenomenon of the nipple during breastfeeding: A case series. PG - e202310280 LID - 10.5546/aap.2023-10280.eng [doi] AB - Raynaud's phenomenon consists of excessive contraction of the blood vessels in response to various stimuli; although it usually affects the extremities, other locations are less frequently involved. This study focused on describing the characteristics of a series of women with Raynaud's phenomenon of the nipple. Through medical record review and direct communication with patients, data from 12 women diagnosed with Raynaud's phenomenon of the nipple between 2016 and 2023 were collected and analyzed. The following variables were assessed: age, symptoms, triggering factors, treatment, and duration of symptoms. In this case series, Raynaud's phenomenon of the nipple in breastfeeding women was more common among primiparous women around 10 days after delivery; pain was severe and, in most cases, improved with local and/or drug treatment, and did not limit the duration of breastfeeding. CI - Sociedad Argentina de Pediatría. FAU - Eymann, Alfredo AU - Eymann A AUID- ORCID: 0000-0001-7509-3721 AD - Department of Clinical Pediatrics; Hospital Italiano de Buenos Aires, City of Buenos Aires, Argentina. FAU - Pontoriero Daroni, Julieta AU - Pontoriero Daroni J AUID- ORCID: 0009-0006-8801-3632 AD - Department of Clinical Pediatrics; Hospital Italiano de Buenos Aires, City of Buenos Aires, Argentina. FAU - Brinci, Mabel AU - Brinci M AD - Department of Neonatology; Hospital Italiano de Buenos Aires, City of Buenos Aires, Argentina. FAU - Cámera, Soledad AU - Cámera S AUID- ORCID: 0009-0000-7602-9254 AD - Department of General Medicine; Hospital Italiano de Buenos Aires, City of Buenos Aires, Argentina. LA - eng LA - spa PT - Case Reports PT - Journal Article TT - Fenómeno de Raynaud en el pezón durante la lactancia: serie de casos. DEP - 20240523 PL - Argentina TA - Arch Argent Pediatr JT - Archivos argentinos de pediatria JID - 0372460 SB - IM MH - Humans MH - *Breast Feeding MH - Female MH - *Raynaud Disease/etiology/diagnosis MH - *Nipples/blood supply MH - Adult MH - Young Adult MH - Retrospective Studies MH - Time Factors OTO - NOTNLM OT - Raynaud’s disease OT - breastfeeding OT - nipples OT - pain OT - vasoconstriction EDAT- 2024/05/21 12:45 MHDA- 2024/08/01 18:43 CRDT- 2024/05/21 11:16 PHST- 2024/08/01 18:43 [medline] PHST- 2024/05/21 12:45 [pubmed] PHST- 2024/05/21 11:16 [entrez] AID - 10.5546/aap.2023-10280.eng [doi] PST - ppublish SO - Arch Argent Pediatr. 2024 Oct 1;122(5):e202310280. doi: 10.5546/aap.2023-10280.eng. Epub 2024 May 23. PMID- 17352512 OWN - NLM STAT- MEDLINE DCOM- 20070731 LR - 20220318 IS - 0012-6667 (Print) IS - 0012-6667 (Linking) VI - 67 IP - 4 DP - 2007 TI - The diagnosis and treatment of Raynaud's phenomenon: a practical approach. PG - 517-25 AB - Raynaud's phenomenon is a common disorder with vasospasm of the digital arteries causing pallor with cyanosis and/or rubor. It can be primary (idiopathic), where it is not associated with other diseases, or secondary to several diseases or conditions, including connective tissue diseases, such as scleroderma and systemic lupus erythematosus. Raynaud's is often mild enough to not require treatment; however, with secondary Raynaud's there is not only vasospasm but also fixed blood vessel defects so the ischaemia can be more severe. Complications can include digital ulcers and could, rarely, lead to amputation. Treatment is often non-pharmacological including avoiding cold and smoking cessation. Calcium channel antagonists, such as nifedipine, are often considered when treatment is needed; however, adverse effects of these drugs can include hypotension, vasodilatation, peripheral oedema and headaches. Other treatments have been studied in randomised, controlled trials including classes of drugs, such as angiotensin II inhibitors, selective serotonin reuptake inhibitors, phosphodiesterase-5 inhibitors (e.g. sildenafil), nitrates (topical or oral; the latter can be limited by adverse effects, such as flushing, headache and hypotension), and for more serious Raynaud's or its complications prostacyclin agonists may be used. There are two large studies that demonstrate that endothelin receptor blockade with bosentan can reduce the number of new digital ulcers in scleroderma patients. However, it does not affect the healing period. Thus, Raynaud's is common and often requires non-pharmacological treatment. When secondary Raynaud's is suspected, such as Raynaud's with an older age at onset or other features of connective tissue disease, then an appropriate history, physical examination and laboratory tests may be indicated to reach an appropriate diagnosis. There have been advances in pharmacological treatment, but some of the treatments are limited by adverse effects. FAU - Pope, Janet E AU - Pope JE AD - Department of Medicine, Division of Rheumatology, London, Ontario, Canada. janet.pope@sjhc.london.on.ca LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Drugs JT - Drugs JID - 7600076 RN - 0 (Calcium Channel Blockers) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Humans MH - Raynaud Disease/diagnosis/physiopathology/*therapy MH - Smoking Cessation RF - 30 EDAT- 2007/03/14 09:00 MHDA- 2007/08/01 09:00 CRDT- 2007/03/14 09:00 PHST- 2007/03/14 09:00 [pubmed] PHST- 2007/08/01 09:00 [medline] PHST- 2007/03/14 09:00 [entrez] AID - 6743 [pii] AID - 10.2165/00003495-200767040-00003 [doi] PST - ppublish SO - Drugs. 2007;67(4):517-25. doi: 10.2165/00003495-200767040-00003. PMID- 35809802 OWN - NLM STAT- MEDLINE DCOM- 20240115 LR - 20250128 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 90 IP - 2 DP - 2024 Feb TI - Part II: The treatment of primary and secondary Raynaud's phenomenon. PG - 237-248 LID - S0190-9622(22)02249-6 [pii] LID - 10.1016/j.jaad.2022.05.067 [doi] AB - Raynaud phenomenon (RP) presents with either primary or secondary disease, and both have the potential to negatively impact patient quality of life. First-line management of RP should include lifestyle modifications in all patients. Some patients with primary RP and most with secondary RP require pharmacologic therapies, which may include calcium channel blockers, topical nitrates, phosphodiesterase 5 inhibitors, or endothelin antagonists. Additional approaches to treatment for those with signs of critical ischemia or those who fail pharmacologic therapy include botulinum toxin injection and digital sympathectomy. Herein, we describe in detail the treatment options for patients with RP as well as provide treatment algorithms for each RP subtype. CI - Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Curtiss, Paul AU - Curtiss P AD - Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. FAU - Svigos, Katerina AU - Svigos K AD - Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. FAU - Schwager, Zachary AU - Schwager Z AD - Department of Dermatology, Lahey Hospital and Medical Center, Burlington, Massachusetts. FAU - Lo Sicco, Kristen AU - Lo Sicco K AD - Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. Electronic address: Kristen.losicco@nyulangone.org. FAU - Franks, Anrdew G Jr AU - Franks AG Jr AD - Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York; Division of Rheumatology, Department of Internal Medicine, New York University Grossman School of Medicine, New York, New York. LA - eng PT - Journal Article PT - Review DEP - 20220706 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Calcium Channel Blockers) RN - 0 (Nitrates) SB - IM MH - Humans MH - *Quality of Life MH - Calcium Channel Blockers/therapeutic use MH - Nitrates/therapeutic use MH - *Raynaud Disease/diagnosis/etiology/therapy OTO - NOTNLM OT - Raynaud phenomenon OT - connective tissue disease OT - medical dermatology OT - scleroderma OT - systemic lupus COIS- Conflicts of interest None disclosed. EDAT- 2022/07/10 06:00 MHDA- 2024/01/15 12:42 CRDT- 2022/07/09 19:26 PHST- 2022/01/31 00:00 [received] PHST- 2022/05/15 00:00 [revised] PHST- 2022/05/25 00:00 [accepted] PHST- 2024/01/15 12:42 [medline] PHST- 2022/07/10 06:00 [pubmed] PHST- 2022/07/09 19:26 [entrez] AID - S0190-9622(22)02249-6 [pii] AID - 10.1016/j.jaad.2022.05.067 [doi] PST - ppublish SO - J Am Acad Dermatol. 2024 Feb;90(2):237-248. doi: 10.1016/j.jaad.2022.05.067. Epub 2022 Jul 6. PMID- 17124789 OWN - NLM STAT- MEDLINE DCOM- 20070405 LR - 20190922 IS - 1547-1896 (Print) IS - 0893-7400 (Linking) VI - 19 IP - 11 DP - 2006 Nov TI - The patient with cold hands: understanding Raynaud's disease. PG - 34-8 AB - The availability of new treatments for Raynaud's disease, as with any medical condition, depends on how convincing the results of clinical research prove to be. The validity and reliability of research in Raynaud's disease is subject to two major constraints: the seasonal and intermittent nature of the condition limits the amount of time patients can be studied continuously, and researchers have failed to agree on objective outcome measures. Vascular physiology is, and no doubt is destined to remain, a dynamic and technology-driven clinical realm. Despite the wide array of promising treatments, the best and most basic management of Raynaud's disease seems to be behavioral and at least partly pharmaceutical. The two biggest behavioral factors are nicotine use and exposure to cold environments. Giving up nicotine can be a daunting challenge for long-term smokers and chewing-tobacco users. Avoiding cold environments may be easier said than done, especially for patients who work outdoors or in air-conditioned spaces. Perhaps the best treatment for Raynaud's disease is a reliable diagnosis and the positive prognosis that comes with it. Most patients have a stable course, and nearly half will actually improve with time and steady reassurance. FAU - Jackson, Carol Mackenzie AU - Jackson CM AD - Internal Medicine Associates, Crozer-Chester Medical Center, Upland, PA, USA. LA - eng PT - Journal Article PL - United States TA - JAAPA JT - JAAPA : official journal of the American Academy of Physician Assistants JID - 9513102 MH - Humans MH - Prognosis MH - Raynaud Disease/*physiopathology/*therapy EDAT- 2006/11/28 09:00 MHDA- 2007/04/06 09:00 CRDT- 2006/11/28 09:00 PHST- 2006/11/28 09:00 [pubmed] PHST- 2007/04/06 09:00 [medline] PHST- 2006/11/28 09:00 [entrez] AID - 10.1097/01720610-200611000-00006 [doi] PST - ppublish SO - JAAPA. 2006 Nov;19(11):34-8. doi: 10.1097/01720610-200611000-00006. PMID- 21868066 OWN - NLM STAT- MEDLINE DCOM- 20120723 LR - 20220318 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 41 IP - 4 DP - 2012 Feb TI - Botulinum toxin A treatment of Raynaud's phenomenon: a review. PG - 599-603 LID - 10.1016/j.semarthrit.2011.07.006 [doi] AB - OBJECTIVES: Botulinum toxin A has conventionally been used in the upper extremity to treat spasticity resulting from stroke, paraplegia, and dystonia. Recently, it has been used to relieve symptoms of vasospasm in Raynaud's phenomenon. This review summarizes the current literature on botulinum toxin A in the treatment of Raynaud's phenomenon and examines the proposed mechanisms of action, suggested techniques of administration, and clinical efficacy. METHODS: An Ovid MEDLINE search from 1950 to September 2010 was performed to identify any reports on the use of Botulinum toxin in the treatment of Raynaud's disease or associated vasoconstrictive disorders. All studies pertaining to "Raynaud's disease," "Raynaud's," or "vasoconstriction" were queried and meshed with a secondary search of studies pertaining to "botox" or "botulinum toxin type A." These reports were meshed and subsequently limited to human studies. All studies that met criteria were included and their outcomes evaluated and summarized. RESULTS: Since 2004, there have been 5 studies that have evaluated the use of Botulinum Toxin A for the treatment of Raynaud's. In each study, patients received a range of botulinum toxin injections (10-100 units) in their fingers and hands. The studies have many limitations (lack of controls, variable severity of disease, variability of dosing) but all report favorable clinical results. All showed overall improvement in patient pain as well as a reduction in soft tissue ulceration. CONCLUSIONS: Initial reports on the use of botulinum toxin A for Raynaud's phenomenon are promising. Larger controlled trials with improved study design are warranted. A better understanding of the mechanism of action, appropriate dose and dose frequency, and the efficacy relative to other medical and surgical treatments requires investigation. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Iorio, Matthew L AU - Iorio ML AD - The Curtis National Hand Center, Baltimore, MD, USA. FAU - Masden, Derek L AU - Masden DL FAU - Higgins, James P AU - Higgins JP LA - eng PT - Journal Article PT - Review DEP - 20110824 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/*therapeutic use MH - Humans MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Treatment Outcome EDAT- 2011/08/27 06:00 MHDA- 2012/07/24 06:00 CRDT- 2011/08/27 06:00 PHST- 2010/11/04 00:00 [received] PHST- 2011/07/12 00:00 [revised] PHST- 2011/07/16 00:00 [accepted] PHST- 2011/08/27 06:00 [entrez] PHST- 2011/08/27 06:00 [pubmed] PHST- 2012/07/24 06:00 [medline] AID - S0049-0172(11)00200-9 [pii] AID - 10.1016/j.semarthrit.2011.07.006 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2012 Feb;41(4):599-603. doi: 10.1016/j.semarthrit.2011.07.006. Epub 2011 Aug 24. PMID- 17668693 OWN - NLM STAT- MEDLINE DCOM- 20070821 LR - 20200103 IS - 0204-8043 (Print) IS - 0204-8043 (Linking) VI - 48 IP - 3-4 DP - 2006 TI - Raynaud's phenomenon in common rheumatic diseases. PG - 22-8 AB - Raynaud's phenomenon (RP) is caused by a reversible spasm of the smallest arteries and the arterioles of the fingers and toes. Two forms of RP have been described: primary and secondary. Secondary RP is often present in patients with rheumatic diseases. This review presents the characteristics of the clinical pattern, the immunological profile and the capillaroscopic pattern in patients with primary and secondary RP in common rheumatic diseases. Attention is paid to standard examinations usually used in RP patients. Primary RP appears to be more common in women beginning usually at puberty. This disorder is caused by vasospasm; no abnormalities of the endothelium are observed. Primary RP shows benign progression. Laboratory tests - erythrocyte sedimentation rate (ESR) and antinuclear antibody test (ANA) - are normal. Capillaroscopy is normal too. Secondary RP tends to begin later in life. Of the rheumatic diseases, scleroderma is the one that is the most often associated with RP (in 90-95% of all cases). The pathogenesis of secondary RP in scleroderma is explained with abnormalities of the endothelium through different mechanisms. Motor ulcerations are frequently observed. Anticentromeric and antitopoizomeric antibodies get positive. Capillaroscopy appears to be very important for the early diagnosis and prognosis of scleroderma. The capillaroscopic pattern is abnormal - enlarged capillaries, hemorrhages and avascular areas are observed. Part of the patients with systemic lupus erythematosus, Sjogren's syndrome and polymyositis / dermatomyositis develops secondary RP and it usually shows benign progression. RP is the main symptom in mixed connective tissue disease and trophic abnormalities of the fingers are frequently observed. Elevated anti-U1-RNP antibody titers and an abnormal capillaroscopic pattern are specific for the condition. In older patients isolated RP may represent a paraneoplastic manifestation. RP is frequently found in the rheumatologic practice. The differentiation of the primary form from the secondary one is essential because of the differences concerning the heaviness of the disorder, the prognosis and the therapeutic approach. FAU - Lambova, Sevdalina N AU - Lambova SN AD - Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria. FAU - Kuzmanova, Stefka I AU - Kuzmanova SI LA - eng PT - Journal Article PT - Review PL - Bulgaria TA - Folia Med (Plovdiv) JT - Folia medica JID - 2984761R RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Disease Progression MH - Humans MH - Microscopic Angioscopy/methods MH - Raynaud Disease/*complications/immunology/physiopathology MH - Rheumatic Diseases/*complications/immunology/physiopathology RF - 35 EDAT- 2007/08/03 09:00 MHDA- 2007/08/22 09:00 CRDT- 2007/08/03 09:00 PHST- 2007/08/03 09:00 [pubmed] PHST- 2007/08/22 09:00 [medline] PHST- 2007/08/03 09:00 [entrez] PST - ppublish SO - Folia Med (Plovdiv). 2006;48(3-4):22-8. PMID- 35809798 OWN - NLM STAT- MEDLINE DCOM- 20240115 LR - 20250128 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 90 IP - 2 DP - 2024 Feb TI - Part I: Epidemiology, pathophysiology, and clinical considerations of primary and secondary Raynaud's phenomenon. PG - 223-234 LID - S0190-9622(22)02250-2 [pii] LID - 10.1016/j.jaad.2022.06.1199 [doi] AB - Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to cold or stress. Secondary RP (SRP) has been described in association with a variety of rheumatologic and nonrheumatologic diseases, environmental exposures, and/or medications. While both primary RP and SRP may impact the quality of life, SRP may lead to permanent and potentially devastating tissue destruction when undiagnosed and untreated. It is therefore crucial for dermatologists to distinguish between primary and secondary disease forms early in clinical evaluation, investigate potential underlying causes, and risk stratify SRP patients for the development of associated autoimmune connective tissue disease. The epidemiology, pathogenesis, and clinical presentation and diagnosis of both forms of RP are described in detail in this review article. CI - Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Curtiss, Paul AU - Curtiss P AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. FAU - Svigos, Katerina AU - Svigos K AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. FAU - Schwager, Zachary AU - Schwager Z AD - Department of Dermatology, Lahey Hospital and Medical Center, Burlington, Massachusetts. FAU - Lo Sicco, Kristen AU - Lo Sicco K AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York. Electronic address: Kristen.losicco@nyulangone.org. FAU - Franks, Andrew G Jr AU - Franks AG Jr AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York; Department of Internal Medicine, Division of Rheumatology, New York University School of Medicine, New York, New York. LA - eng PT - Journal Article PT - Review DEP - 20220707 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Humans MH - Quality of Life MH - *Connective Tissue Diseases/diagnosis/epidemiology/complications MH - *Raynaud Disease/diagnosis/epidemiology MH - Hand MH - Upper Extremity OTO - NOTNLM OT - Raynaud's phenomenon OT - connective tissue disease OT - erythematosus OT - medical dermatology OT - scleroderma OT - systemic lupus COIS- Conflicts of interest None disclosed. EDAT- 2022/07/10 06:00 MHDA- 2024/01/15 12:42 CRDT- 2022/07/09 19:26 PHST- 2022/03/04 00:00 [received] PHST- 2022/06/21 00:00 [revised] PHST- 2022/06/23 00:00 [accepted] PHST- 2024/01/15 12:42 [medline] PHST- 2022/07/10 06:00 [pubmed] PHST- 2022/07/09 19:26 [entrez] AID - S0190-9622(22)02250-2 [pii] AID - 10.1016/j.jaad.2022.06.1199 [doi] PST - ppublish SO - J Am Acad Dermatol. 2024 Feb;90(2):223-234. doi: 10.1016/j.jaad.2022.06.1199. Epub 2022 Jul 7. PMID- 35699336 OWN - NLM STAT- MEDLINE DCOM- 20220629 LR - 20240928 IS - 1531-6963 (Electronic) IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 34 IP - 4 DP - 2022 Jul 1 TI - Practical management of Raynaud's phenomenon - a primer for practicing physicians. PG - 235-244 LID - 10.1097/BOR.0000000000000877 [doi] AB - PURPOSE OF REVIEW: Raynaud's phenomenon (RP) is a common vasospastic condition that results in digital hypoperfusion in response to cold and/or emotional stress and is associated with significant pain and disability. The aim of our review is to provide a practical approach for clinicians to inform assessment and management of patients with RP. RECENT FINDINGS: Autoantibodies and nailfold capillaroscopy are key investigations to stratify the risk of progression to systemic sclerosis (SSc) in patients RP, which was recently confirmed in the multicenter, very early diagnosis of systemic sclerosis (VEDOSS) project. Research has explored the complex lived-patient experience of RP including digital vasculopathy in SSc and has highlighted the need for outcome measure development to facilitate research in the field. Pharmacological treatment strategies vary significantly internationally and there is continued interest in developing surgical approaches. SUMMARY: We provide a practical and up-to-date approach to inform the assessment and management of patients with RP including guidance on drug initiation and escalation. Calcium channel blockers are first-line treatment and can be initiated by primary care physicians. We also highlight second-line drug therapies used for refractory RP and the potential role for surgical intervention. CI - Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved. FAU - Ramahi, Ahmad AU - Ramahi A AD - Division of Rheumatology, Department of Medicine. AD - University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. FAU - Hughes, Michael AU - Hughes M AD - Tameside Hospital, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne. AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, Department of Medicine. AD - University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review DEP - 20220609 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Humans MH - Microscopic Angioscopy MH - Multicenter Studies as Topic MH - *Physicians MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Scleroderma, Systemic/complications/diagnosis/therapy PMC - PMC9246963 MID - NIHMS1798395 COIS- Acknowledgements None of the authors or professional colleagues has any conflict of interest. EDAT- 2022/06/15 06:00 MHDA- 2022/06/30 06:00 PMCR- 2023/07/01 CRDT- 2022/06/14 08:14 PHST- 2022/06/15 06:00 [pubmed] PHST- 2022/06/30 06:00 [medline] PHST- 2022/06/14 08:14 [entrez] PHST- 2023/07/01 00:00 [pmc-release] AID - 00002281-202207000-00008 [pii] AID - 10.1097/BOR.0000000000000877 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2022 Jul 1;34(4):235-244. doi: 10.1097/BOR.0000000000000877. Epub 2022 Jun 9. PMID- 27494080 OWN - NLM STAT- MEDLINE DCOM- 20170117 LR - 20250626 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 34 Suppl 100 IP - 5 DP - 2016 Sep-Oct TI - Raynaud's syndrome in children: systematic review and development of recommendations for assessment and monitoring. PG - 200-206 AB - OBJECTIVES: To develop recommendations for investigation and monitoring of children with Raynaud's syndrome, based on paediatric evidence collated by a systematic review. METHODS: A systematic review was undertaken to establish the paediatric evidence for assessment and monitoring of Raynaud's syndrome. An expert panel including members of the Paediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement. RESULTS: The expert panel recommended testing anti-nuclear antibody (ANA), more specific antibodies associated with connective tissue disease and nail-fold capillaroscopy in all children presenting with Raynaud's syndrome as data suggests these can be risk factors for evolution into a connective tissue disease. The frequency of follow-up recommended depends on presence of these risk factors with the aim to detect evolving connective tissue disease early in high risk individuals. Those with no abnormalities on capillaroscopy and negative autoantibodies were deemed low risk of progression, whereas those with ANA positivity, specific autoantibodies and/or nailfold capillary changes were deemed high risk and more frequent follow-up was recommended. CONCLUSIONS: Recommendations, primarily based on consensus opinion, were agreed regarding investigation and monitoring of children who present with Raynaud's syndrome. Further prospective studies are needed to better define the risk factors for progression to connective tissue disease. FAU - Pain, Clare E AU - Pain CE AD - Alder Hey Children's NHS Foundation Trust, Liverpool, UK. FAU - Constantin, Tamás AU - Constantin T AD - Semmelweis University, Budapest, Hungary. FAU - Toplak, Natasa AU - Toplak N AD - University Children's Hospital Ljubljana, Slovenia. FAU - Moll, Monica AU - Moll M AD - University Children's Hospital, Tuebingen, Germany. FAU - Iking-Konert, Christof AU - Iking-Konert C AD - Universitätsklinikum Hamburg-Eppendorf, Germany. FAU - Piotto, Daniella P AU - Piotto DP AD - Universidade Federal de São Paulo, Brazil. FAU - Aktay Ayaz, Nuray AU - Aktay Ayaz N AD - Istanbul Kanuni Sultan Süleyman Education and Research Hospital, Turkey. FAU - Nemcova, Dana AU - Nemcova D AD - Charles University, Prague, Czech Republic. FAU - Hoeger, Peter H AU - Hoeger PH AD - Department of Paediatric Dermatology, Cath. Children's Hospital, Wilhelmstift, Hamburg, Germany. FAU - Cutolo, Maurizio AU - Cutolo M AD - University of Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Ghent University Hospital, Belgium. FAU - Foeldvari, Ivan AU - Foeldvari I AD - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany. foeldvari@t-online.de. CN - Paediatric Rheumatology European Society (PRES) Juvenile Scleroderma Working Group. LA - eng PT - Journal Article PT - Practice Guideline PT - Systematic Review DEP - 20160727 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Age Factors MH - Antibodies, Antinuclear/blood MH - Biomarkers/blood MH - Child MH - Consensus MH - Disease Progression MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*standards MH - Pediatrics/*standards MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/blood/*diagnosis/therapy MH - Rheumatology/*standards MH - Risk Factors MH - Serologic Tests/*standards EDAT- 2016/10/18 06:00 MHDA- 2017/01/18 06:00 CRDT- 2016/08/06 06:00 PHST- 2016/01/29 00:00 [received] PHST- 2016/05/24 00:00 [accepted] PHST- 2016/10/18 06:00 [pubmed] PHST- 2017/01/18 06:00 [medline] PHST- 2016/08/06 06:00 [entrez] AID - 10375 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):200-206. Epub 2016 Jul 27. PMID- 17003081 OWN - NLM STAT- MEDLINE DCOM- 20070108 LR - 20101118 IS - 1542-6270 (Electronic) IS - 1060-0280 (Linking) VI - 40 IP - 11 DP - 2006 Nov TI - Renin-angiotensin system mediators and Raynaud's phenomenon. PG - 1998-2002 AB - OBJECTIVE: To review the use of angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) in the treatment of Raynaud's phenomenon (RP). DATA SOURCES: Biomedical literature was accessed through July 2006 via PubMed, the Iowa Drug Information System (IDIS), and Cumulative Index to Nursing and Allied Health Literature (CINAHL) Plus. PubMed database terms included Raynaud's disease, angiotensin-converting enzyme inhibitors, and angiotensin II type 1 receptor blockers [pharmacological action]; IDIS terms included hypotensive agents-ace inhib 24080200, raynaud's syndrome 443.0, and hypotensive agents-angioten II 24080400; and CINAHL Plus terms included Raynaud's disease, angiotensin-converting enzyme inhibitors, losartan, and irbesartan. STUDY SELECTION AND DATA EXTRACTION: All clinical trials published in English that reported both subjective and objective outcomes of efficacy were reviewed. DATA SYNTHESIS: Several small, short-term studies have evaluated captopril, enalapril, and losartan in the treatment of RP. The studies of ACE inhibitors have found conflicting results in their ability to improve digit blood flow and reduce both frequency and severity of RP attacks. Two studies have focused on the use of losartan for RP treatment, with both finding a statistically significant reduction in attacks and one showing improvement in symptoms of RP in comparison with the commonly utilized calcium-channel blocker, nifedipine. Most of the studies were short term (< or =12 wk) and included a limited number of patients (<60). CONCLUSIONS: ACE inhibitors and ARBs may provide some minor benefits in the relief of RP, although no definite evidence exists to suggest that they are superior to traditionally used treatments such as calcium-channel blockers. Larger, randomized controlled trials of longer duration are needed to compare the effectiveness of ACE inhibitors and ARBs with conventional treatment. FAU - Wood, Heidi M AU - Wood HM AD - College of Pharmacy, The University of Iowa, Iowa City, IA 52242-1009, USA. FAU - Ernst, Michael E AU - Ernst ME LA - eng PT - Journal Article PT - Review DEP - 20060926 PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Receptors, Angiotensin) SB - IM MH - *Angiotensin Receptor Antagonists MH - Angiotensin-Converting Enzyme Inhibitors/pharmacology/*therapeutic use MH - Humans MH - Raynaud Disease/*drug therapy/metabolism MH - Receptors, Angiotensin/metabolism MH - Renin-Angiotensin System/*drug effects/physiology RF - 11 EDAT- 2006/09/28 09:00 MHDA- 2007/01/09 09:00 CRDT- 2006/09/28 09:00 PHST- 2006/09/28 09:00 [pubmed] PHST- 2007/01/09 09:00 [medline] PHST- 2006/09/28 09:00 [entrez] AID - aph.1H201 [pii] AID - 10.1345/aph.1H201 [doi] PST - ppublish SO - Ann Pharmacother. 2006 Nov;40(11):1998-2002. doi: 10.1345/aph.1H201. Epub 2006 Sep 26. PMID- 40506673 OWN - NLM STAT- MEDLINE DCOM- 20250825 LR - 20250825 IS - 1568-5608 (Electronic) IS - 0925-4692 (Print) IS - 0925-4692 (Linking) VI - 33 IP - 7 DP - 2025 Jul TI - Cold responses and hormonal echoes: a comprehensive view of Raynaud's vascular dysfunction. PG - 3637-3651 LID - 10.1007/s10787-025-01792-0 [doi] AB - Raynaud's phenomenon is a peripheral vascular disorder characterized by exaggerated vasoconstrictive response to certain stimuli, most typically cold exposure and emotional stress. Interestingly, Raynaud's phenomenon incidence is significantly higher in premenopausal females compared to age-matched males, highlighting a role of the female hormone, estrogen, in Raynaud's phenomenon pathogenesis. Indeed, estrogen plays a fundamental role in potentiating the expression and function of α(2C) adrenoceptor (α(2C)-AR), the sole mediator of local cooling-induced vasoconstriction. Due to the mosaic nature of Raynaud's phenomenon involving vascular, hormonal, and neuronal factors, as well as due to the lack of an appropriate animal model, the pathogenesis of Raynaud's phenomenon is not fully elucidated. Consequently, despite various therapeutic approaches aimed at mitigating symptoms of Raynaud's phenomenon, a definitive treatment for Raynaud's phenomenon is quite challenging and remains an unmet need. Therefore, a better understanding of the underlying pathophysiologic mechanisms of Raynaud's phenomenon is crucial to better delineate pharmacotherapeutic targets to help fight this elusive disease. In this paper, we dissect the molecular and cellular mechanisms underlying Raynaud's phenomenon and its risk factors, and we shed more light on the role of estrogen. We also explore traditional and current therapeutic approaches, including pharmacologic and non-pharmacologic treatments. In addition, we discuss how the advancement in molecular research offered promising avenues of Raynaud's phenomenon treatment, namely drug repurposing and molecular targeting. Nonetheless, enhanced awareness, precaution, and good patient compliance are critically important in preventing the progression of Raynaud's phenomenon and reducing its severity. CI - © 2025. The Author(s). FAU - Fardoun, Manal AU - Fardoun M AD - Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - El Ghawi, Odette AU - El Ghawi O AD - Faculty of Medicine, University of Balamand, El Koura, Lebanon. FAU - Dib, Christie AU - Dib C AD - Faculty of Medicine, University of Balamand, El Koura, Lebanon. FAU - Jaradi, Leen AU - Jaradi L AD - Faculty of Medicine, University of Balamand, El Koura, Lebanon. FAU - Chaddad, Marie Therese AU - Chaddad MT AD - Faculty of Medicine, University of Balamand, El Koura, Lebanon. FAU - Dehaini, Hassan AU - Dehaini H AD - Case Western Reserve University, The MetroHealth System, Cleveland, OH, USA. FAU - Eid, Ali H AU - Eid AH AUID- ORCID: 0000-0003-3004-5675 AD - Department of Basic Medical Sciences, College of Medicine, QU Health, Qatar University, PO Box 2713, Doha, Qatar. ali.eid@qu.edu.qa. LA - eng PT - Journal Article PT - Review DEP - 20250612 PL - Switzerland TA - Inflammopharmacology JT - Inflammopharmacology JID - 9112626 RN - 0 (Estrogens) RN - 0 (Receptors, Adrenergic, alpha-2) SB - IM MH - Humans MH - *Raynaud Disease/physiopathology/metabolism/drug therapy MH - Animals MH - *Cold Temperature/adverse effects MH - *Estrogens/metabolism MH - Vasoconstriction/physiology MH - Female MH - Receptors, Adrenergic, alpha-2/metabolism MH - Male MH - Risk Factors PMC - PMC12354549 OTO - NOTNLM OT - Adrenergic receptors OT - Arterioles OT - Cold-induced constriction OT - Peripheral vascular disease OT - Thermoregulation COIS- Declarations. Conflict of interest: The authors declare that they have no conflicts of interest. EDAT- 2025/06/13 00:27 MHDA- 2025/08/31 20:01 PMCR- 2025/06/12 CRDT- 2025/06/12 23:22 PHST- 2025/04/09 00:00 [received] PHST- 2025/05/06 00:00 [accepted] PHST- 2025/08/31 20:01 [medline] PHST- 2025/06/13 00:27 [pubmed] PHST- 2025/06/12 23:22 [entrez] PHST- 2025/06/12 00:00 [pmc-release] AID - 10.1007/s10787-025-01792-0 [pii] AID - 1792 [pii] AID - 10.1007/s10787-025-01792-0 [doi] PST - ppublish SO - Inflammopharmacology. 2025 Jul;33(7):3637-3651. doi: 10.1007/s10787-025-01792-0. Epub 2025 Jun 12. PMID- 29658285 OWN - NLM STAT- MEDLINE DCOM- 20180702 LR - 20180702 IS - 0030-6002 (Print) IS - 0030-6002 (Linking) VI - 159 IP - 16 DP - 2018 Apr TI - [Quality of life of patients with Raynaud's disease]. PG - 636-641 LID - 10.1556/650.2018.31034 [doi] AB - INTRODUCTION: Raynaud's disease is characterized by episodic vasospastic attacks and digital ischemia usually followed by pain, numbness and cold. Despite the severity of the symptoms, the investigation of the quality of life in this disease received less attention yet. AIM: The aim of the study was to examine how the disease affects the patients' quality of life. METHOD: Semi-structured interviews were made with 28 patients diagnosed with Raynaud's disease. RESULTS: Almost every domain of quality of life is negatively affected. The somatic symptoms cause significant suffering, they are accompanied by loss of functionality; frequent preventive actions are needed; furthermore they affect job performance, commuting and sleep quality. Emotional and cognitive burdens and negative changes in interpersonal relationships were found. CONCLUSION: The findings of this study show that the disease is present as significant hardship in every aspect of daily life. Because of the decrease in the quality of life and the psychological burdens caused by this chronic disease, not only the basic medical care, but psychological treatment is also indicated. Orv Hetil. 2018; 159(16): 636-641. FAU - Fábián, Balázs AU - Fábián B AD - Klinikai és Egészségpszichológiai Tanszék, Magatartástudományi Intézet, Debreceni Egyetem, Népegészségügyi Kar Debrecen, Pf. 45, 4032. FAU - Csiki, Zoltán AU - Csiki Z AD - Belgyógyászati Klinika, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen. FAU - Bugán, Antal AU - Bugán A AD - Klinikai és Egészségpszichológiai Tanszék, Magatartástudományi Intézet, Debreceni Egyetem, Népegészségügyi Kar Debrecen, Pf. 45, 4032. LA - hun PT - Journal Article TT - Raynaud-szindrómás betegek életminőségének jellemzői. PL - Hungary TA - Orv Hetil JT - Orvosi hetilap JID - 0376412 RN - 0 (Calcium Channel Blockers) SB - IM MH - Adaptation, Psychological MH - Adult MH - Calcium Channel Blockers/administration & dosage MH - *Cost of Illness MH - Family Relations MH - Female MH - Humans MH - Male MH - Quality of Life/*psychology MH - Raynaud Disease/complications/drug therapy/*psychology MH - Stress, Psychological/etiology/*psychology OTO - NOTNLM OT - Raynaud-szindróma OT - Raynaud’s disease OT - quality of life OT - stress OT - stressz OT - életminőség EDAT- 2018/04/17 06:00 MHDA- 2018/07/03 06:00 CRDT- 2018/04/17 06:00 PHST- 2018/04/17 06:00 [entrez] PHST- 2018/04/17 06:00 [pubmed] PHST- 2018/07/03 06:00 [medline] AID - 10.1556/650.2018.31034 [doi] PST - ppublish SO - Orv Hetil. 2018 Apr;159(16):636-641. doi: 10.1556/650.2018.31034. PMID- 28092215 OWN - NLM STAT- MEDLINE DCOM- 20180309 LR - 20180410 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 13 IP - 7 DP - 2017 Jul TI - Therapeutic implications from the pathogenesis of Raynaud's phenomenon. PG - 723-735 LID - 10.1080/1744666X.2017.1279052 [doi] AB - Raynaud's phenomenon (RP) can be either primary (idiopathic) or secondary to a number of different diseases/conditions, when vasopasm can be superimposed upon structural vascular abnormality or a hyperviscosity state and may then lead to severe ischaemia with tissue damage. Treatment must be tailored to the individual. Areas covered: This review discusses how increased understanding of the pathogenesis of RP has driven and is driving new approaches to therapy, and how we are now better able to predict which patients presenting with RP are likely to have an underlying disease requiring specific intervention. Medline searches (1946 to August 2016) were conducted for 'Raynaud's' in combination with relevant terms including different drugs. All papers identified were English language, with abstracts. Expert commentary: Randomised controlled trials of RP present particular challenges. The major aim must continue to be development of safe, effective treatments for patients across the spectrum of RP. FAU - Herrick, Ariane L AU - Herrick AL AD - a Division of Musculoskeletal and Dermatological Sciences , The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK. AD - b NIHR Manchester Musculoskeletal Biomedical Research Unit , Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK. LA - eng PT - Journal Article PT - Review DEP - 20170203 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - Animals MH - Blood Vessels/*abnormalities MH - Diagnosis, Differential MH - Environmental Exposure/adverse effects MH - Expert Testimony MH - Humans MH - Ischemia/etiology/*prevention & control MH - Precision Medicine MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/complications/diagnosis/*drug therapy MH - Thrombophilia MH - Treatment Outcome OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital vasculopathy OT - pathogenesis OT - randomised controlled trials OT - systemic sclerosis OT - treatment EDAT- 2017/01/17 06:00 MHDA- 2018/03/10 06:00 CRDT- 2017/01/17 06:00 PHST- 2017/01/17 06:00 [pubmed] PHST- 2018/03/10 06:00 [medline] PHST- 2017/01/17 06:00 [entrez] AID - 10.1080/1744666X.2017.1279052 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2017 Jul;13(7):723-735. doi: 10.1080/1744666X.2017.1279052. Epub 2017 Feb 3. PMID- 28256352 OWN - NLM STAT- MEDLINE DCOM- 20180430 LR - 20180430 IS - 0736-4679 (Print) IS - 0736-4679 (Linking) VI - 52 IP - 6 DP - 2017 Jun TI - Post-Traumatic Raynaud's Phenomenon: A Case Report. PG - e237-e238 LID - S0736-4679(17)30001-X [pii] LID - 10.1016/j.jemermed.2017.01.001 [doi] AB - BACKGROUND: Raynaud's phenomenon has multiple etiologies, ranging from occupational causes to systemic disease. Most occupational causes of Raynaud's phenomenon usually present with vascular compromise. CASE REPORT: A 41-year-old Chinese woman presented to the emergency department with progressive pain and bluish discoloration over her right index finger after minor trauma. The clinical examination revealed discoloration over multiple fingertips on both hands. She was diagnosed with Raynaud's phenomenon with possible underlying systemic disease. Additional laboratory workup led to the diagnosis of systemic lupus erythematosus with complex regional pain syndrome. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: It is rare for the emergency physician to diagnose Raynaud's phenomenon in the setting of minor trauma. It is important to diagnose this condition because of its potential complications. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Cheok, Liang Jie AU - Cheok LJ AD - Family Medicine, National Health Group Polyclinics, Singapore. FAU - Ooi, Chee Kheong AU - Ooi CK AD - Emergency Department, Tan Tock Seng Hospital, Singapore. LA - eng PT - Case Reports PT - Journal Article DEP - 20170227 PL - United States TA - J Emerg Med JT - The Journal of emergency medicine JID - 8412174 SB - IM MH - Adult MH - Cyanosis/etiology MH - Emergency Service, Hospital/organization & administration MH - Female MH - Finger Injuries/*complications/physiopathology MH - Fingers/abnormalities/anatomy & histology/physiopathology MH - Humans MH - Microscopic Angioscopy/methods MH - Pain/etiology MH - Photoplethysmography/methods MH - Raynaud Disease/*etiology OTO - NOTNLM OT - Raynaud's phenomenon OT - systemic lupus erythematosus OT - trauma EDAT- 2017/03/04 06:00 MHDA- 2018/05/01 06:00 CRDT- 2017/03/04 06:00 PHST- 2016/06/22 00:00 [received] PHST- 2016/12/27 00:00 [revised] PHST- 2017/01/04 00:00 [accepted] PHST- 2017/03/04 06:00 [pubmed] PHST- 2018/05/01 06:00 [medline] PHST- 2017/03/04 06:00 [entrez] AID - S0736-4679(17)30001-X [pii] AID - 10.1016/j.jemermed.2017.01.001 [doi] PST - ppublish SO - J Emerg Med. 2017 Jun;52(6):e237-e238. doi: 10.1016/j.jemermed.2017.01.001. Epub 2017 Feb 27. PMID- 23440376 OWN - NLM STAT- MEDLINE DCOM- 20131018 LR - 20211021 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 72 IP - 2 DP - 2013 Mar TI - [Capillaroscopy. An update]. PG - 145-50 LID - 10.1007/s00393-012-1069-6 [doi] AB - This article reports relevant innovations 2 years after the publication of the German consensus on nomenclature and procedure of capillaroscopy in this journal. Standardization: certified training courses in capillaroscopy under the patronage of the Rheumatism Academy have reached over 300 rheumatologists nationwide. Trained investigators clearly show greater agreement on findings than untrained experts even with years of experience. Normal findings were detected using the published terminology in a large cohort. Scientific application: the number of publications on capillaroscopy has risen significantly, prospective studies of smaller cohorts and the comparison to other methods regarding systemic sclerosis, Raynaud's disease and also miscellaneous diseases. A large prospective multicenter European study on the capillaroscopic skin ulcer risk index (CSURI) was conducted with German participation. The role as a screening tool has been confirmed by the majority of the studies. For systemic lupus erythematosus it is also increasingly being used in the diagnostic work-up as an additional investigation. Practical application: capillaroscopy has a solid and indispensable role in the diagnostic algorithm of Raynaud's disease and early diagnosis of systemic sclerosis. Videocapillaroscopy and simple USB microscopes are increasingly being used besides traditional light microscopy. FAU - Richter, J AU - Richter J AD - Poliklinik für Rheumatologie, Heinrich Heine-Universität Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland. FAU - Iking-Konert, C AU - Iking-Konert C FAU - Schneider, M AU - Schneider M FAU - Sander, O AU - Sander O LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Kapillarmikroskopie. Ein Update. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Capillaries/pathology MH - Connective Tissue Diseases/*diagnosis/therapy MH - Curriculum/standards MH - Education, Medical, Continuing MH - Germany MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/therapy MH - Microscopic Angioscopy/*instrumentation/*methods MH - Middle Aged MH - Raynaud Disease/diagnosis/therapy MH - Reference Values MH - Rheumatology/education MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/therapy MH - Skin Ulcer/diagnosis MH - Terminology as Topic MH - Video Recording/instrumentation/methods EDAT- 2013/02/27 06:00 MHDA- 2013/10/19 06:00 CRDT- 2013/02/27 06:00 PHST- 2013/02/27 06:00 [entrez] PHST- 2013/02/27 06:00 [pubmed] PHST- 2013/10/19 06:00 [medline] AID - 10.1007/s00393-012-1069-6 [doi] PST - ppublish SO - Z Rheumatol. 2013 Mar;72(2):145-50. doi: 10.1007/s00393-012-1069-6. PMID- 28523890 OWN - NLM STAT- MEDLINE DCOM- 20180404 LR - 20180404 IS - 1525-1470 (Electronic) IS - 0736-8046 (Linking) VI - 34 IP - 3 DP - 2017 May TI - Handy Hints About Raynaud's Phenomenon in Children: A Critical Review. PG - 235-239 LID - 10.1111/pde.13129 [doi] AB - Raynaud's phenomenon (RP) is a vasospastic disorder characterized by recurrent self-limited episodes of skin pallor, cyanosis, and hyperemia caused by paroxysmal spasms in the small arteries of the fingers and toes and can occur in any age group. Hands, feet, nose, ears, and nipples can be affected. The diagnosis is made clinically, assessing varying degrees of ischemia in the involved areas of skin, but this transient ischemia may also herald the onset of connective tissue disease. Investigation is recommended when RP starts in childhood to exclude an underlying autoimmune condition and close follow-up for its development. Management of RP in children includes conservative and pharmacologic treatments. CI - © 2017 Wiley Periodicals, Inc. FAU - Rigante, Donato AU - Rigante D AD - Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - Fastiggi, Michele AU - Fastiggi M AD - Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - Ricci, Francesco AU - Ricci F AUID- ORCID: 0000-0002-8388-9268 AD - Institute of Dermatology, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - D'Errico, Francesca AU - D'Errico F AD - Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - Bracci, Benedetta AU - Bracci B AD - Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - Guerriero, Cristina AU - Guerriero C AD - Institute of Dermatology, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. LA - eng PT - Journal Article PT - Review PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 SB - IM MH - Child MH - Child, Preschool MH - *Comorbidity MH - Connective Tissue Diseases/diagnosis/epidemiology MH - Conservative Treatment/methods MH - Female MH - Fingers/blood supply/*physiopathology MH - Humans MH - Male MH - Pediatrics MH - Peripheral Vascular Diseases/*diagnosis/pathology/therapy MH - Raynaud Disease/*diagnosis/epidemiology/therapy MH - Regional Blood Flow EDAT- 2017/05/20 06:00 MHDA- 2018/04/05 06:00 CRDT- 2017/05/20 06:00 PHST- 2017/05/20 06:00 [entrez] PHST- 2017/05/20 06:00 [pubmed] PHST- 2018/04/05 06:00 [medline] AID - 10.1111/pde.13129 [doi] PST - ppublish SO - Pediatr Dermatol. 2017 May;34(3):235-239. doi: 10.1111/pde.13129. PMID- 32700966 OWN - NLM STAT- MEDLINE DCOM- 20210625 LR - 20210625 IS - 1556-8342 (Electronic) IS - 1556-8253 (Linking) VI - 15 IP - 11 DP - 2020 Nov TI - Drug Treatment of Raynaud's Phenomenon of the Nipple. PG - 686-688 LID - 10.1089/bfm.2020.0198 [doi] FAU - Anderson, Philip O AU - Anderson PO AD - Division of Clinical Pharmacy, Skaggs School of Pharmacy and Pharmaceutical Sciences, University of California, San Diego, San Diego, California, USA. LA - eng PT - Journal Article PT - Review DEP - 20200717 PL - United States TA - Breastfeed Med JT - Breastfeeding medicine : the official journal of the Academy of Breastfeeding Medicine JID - 101260777 RN - 0 (Calcium Channel Blockers) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - G59M7S0WS3 (Nitroglycerin) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Breast Diseases/diagnosis/*drug therapy MH - Breast Feeding MH - Calcium Channel Blockers/administration & dosage/*therapeutic use MH - Female MH - Humans MH - Nifedipine/administration & dosage/*therapeutic use MH - *Nipples MH - Nitroglycerin/*administration & dosage/therapeutic use MH - Phosphodiesterase 5 Inhibitors/administration & dosage/*therapeutic use MH - Raynaud Disease/diagnosis/*drug therapy EDAT- 2020/07/24 06:00 MHDA- 2021/06/29 06:00 CRDT- 2020/07/24 06:00 PHST- 2020/07/24 06:00 [pubmed] PHST- 2021/06/29 06:00 [medline] PHST- 2020/07/24 06:00 [entrez] AID - 10.1089/bfm.2020.0198 [doi] PST - ppublish SO - Breastfeed Med. 2020 Nov;15(11):686-688. doi: 10.1089/bfm.2020.0198. Epub 2020 Jul 17. PMID- 21435165 OWN - NLM STAT- MEDLINE DCOM- 20120112 LR - 20220310 IS - 1533-2500 (Electronic) IS - 1530-7085 (Linking) VI - 11 IP - 5 DP - 2011 Sep-Oct TI - 25. Ischemic pain in the extremities and Raynaud's phenomenon. PG - 483-91 LID - 10.1111/j.1533-2500.2011.00460.x [doi] AB - Two important groups of disorders result from an insufficient blood supply to the extremities: critical vascular disease and the Raynaud's phenomenon. The latter can be subdivided into a primary and a secondary type. Critical ischemic disease is often caused by arteriosclerosis due to hypertension or diabetes. Primary Raynaud's is idiopathic and will be diagnosed as such if underlying systemic pathology has been excluded. Secondary Raynaud's is often a manifestation of a systemic disease. It is essential to try to establish a diagnosis as soon as possible in order to influence the evolution of the disease. A sympathetic nerve block can be considered in patients with critical ischemic vascular disease after extensive conservative treatment, preferably in the context of a study (2B±). If this has insufficient effect, spinal cord stimulation can be considered in a selected patient group (2B±). In view of the degree of invasiveness and the costs involved, this treatment should preferably be applied in the context of a study and with the use of transcutaneous pO(2) measurements. In case of primary Raynaud's, life style changes are the first step. Sympathectomy can be considered as a treatment of Raynaud's phenomenon (2C+), but only after multidisciplinary evaluation of the patient and in close consultation with the patient's rheumatologist, vascular surgeon or internist. CI - © 2011 The Authors. Pain Practice © 2011 World Institute of Pain. FAU - Devulder, Jacques AU - Devulder J AD - Department of Anesthesiology and Multidisciplinary Pain Centre, University Hospital Ghent, Ghent, Belgium. FAU - van Suijlekom, Hans AU - van Suijlekom H FAU - van Dongen, Robert AU - van Dongen R FAU - Diwan, Sudhir AU - Diwan S FAU - Mekhail, Nagy AU - Mekhail N FAU - van Kleef, Maarten AU - van Kleef M FAU - Huygen, Frank AU - Huygen F LA - eng PT - Journal Article PT - Review DEP - 20110325 PL - United States TA - Pain Pract JT - Pain practice : the official journal of World Institute of Pain JID - 101130835 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/therapeutic use MH - Electric Stimulation Therapy MH - Humans MH - Ischemia/*complications MH - Neuromuscular Agents/therapeutic use MH - Pain/diagnosis/epidemiology/*etiology/physiopathology MH - Pain Management/*methods MH - Raynaud Disease/*complications/diagnosis/epidemiology/physiopathology/*therapy MH - Spinal Cord EDAT- 2011/03/26 06:00 MHDA- 2012/01/13 06:00 CRDT- 2011/03/26 06:00 PHST- 2011/03/26 06:00 [entrez] PHST- 2011/03/26 06:00 [pubmed] PHST- 2012/01/13 06:00 [medline] AID - 10.1111/j.1533-2500.2011.00460.x [doi] PST - ppublish SO - Pain Pract. 2011 Sep-Oct;11(5):483-91. doi: 10.1111/j.1533-2500.2011.00460.x. Epub 2011 Mar 25. PMID- 32794364 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1529-8019 (Electronic) IS - 1396-0296 (Linking) VI - 33 IP - 6 DP - 2020 Nov TI - Botulinum toxin type A in the treatment of Raynaud's phenomenon. PG - e14182 LID - 10.1111/dth.14182 [doi] AB - Raynaud's phenomenon is a vasospastic disorder clinically characterized by cold or stress-induced discoloration of the skin, pain and ulcers of the fingers or toes. Although this phenomenon might be self-limiting, there is a subgroup of patients requiring a therapeutic approach. The majority of patients do well on conservative measures; however, there is also a subgroup requiring systemic treatment. The efficacy of these systemic treatments is currently not thoroughly investigated. Furthermore, no uniform guidelines exist regarding the choice for a treatment option. In the past several years, several reports have shown the benefits of botulinum toxin for the treatment of Raynaud's phenomenon. In this case series, we report our experience with botulinum toxin type A in the treatment of Raynaud's phenomenon. CI - © 2020 Wiley Periodicals LLC. FAU - Habib, S Meelad AU - Habib SM AUID- ORCID: 0000-0001-9582-5002 AD - Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands. AD - Department of Dermatology, Dermateam at Bravis Hospital, Bergen op Zoom, The Netherlands. FAU - Brenninkmeijer, Elian E A AU - Brenninkmeijer EEA AD - Department of Dermatology, Centrum Oosterwal, Alkmaar, The Netherlands. FAU - Vermeer, Maarten H AU - Vermeer MH AD - Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Velthuis, Peter J AU - Velthuis PJ AD - Department of Dermatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands. LA - eng PT - Journal Article DEP - 20200910 PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A MH - Fingers MH - Humans MH - Pain MH - *Raynaud Disease/diagnosis/drug therapy MH - Toes OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin OT - pain OT - vasospastic EDAT- 2020/08/15 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/08/15 06:00 PHST- 2020/07/15 00:00 [received] PHST- 2020/08/03 00:00 [revised] PHST- 2020/08/06 00:00 [accepted] PHST- 2020/08/15 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/08/15 06:00 [entrez] AID - 10.1111/dth.14182 [doi] PST - ppublish SO - Dermatol Ther. 2020 Nov;33(6):e14182. doi: 10.1111/dth.14182. Epub 2020 Sep 10. PMID- 24801303 OWN - NLM STAT- MEDLINE DCOM- 20140625 LR - 20140507 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 139 IP - 20 DP - 2014 May TI - [Differential diagnoses of Raynaud's phenomenon]. PG - 1064-9 LID - 10.1055/s-0034-1370036 [doi] AB - Raynaud's phenomenon (RP) is characterized by repeated vasospastic attacks of the distal extremities induced by cold, humidity, vibrations or emotional stress. It typically presents a triphasic colour change from white (palor; vasoconstriction) to blue (cyanosis) and red (reactive hyperaemia). The symptoms are based on a primary RP in 90 %. Secondary RP is a symptom of an underlying disease. RP has to be distinguished from other colour changes of the distal extremities like acrocyanosis, erythromelalgia, perniosis and Chilblain-Lupus. Patients history, clinical examination, ANA, ESR/CRP and nailfold capillaroscopy are essential for the early diagnosis of an underlying disease. The initiation of angiologic tests is important in patients with digital ulcers, necrosis or gangrene. Important differential diagnoses in secondary RP are autoimmune rheumatic diseases like systemic sclerosis and systemic lupus erythematodes as well as vascular diseases like arterial occlusions and compression syndromes or concomitant medication (i. e. beta-blocker). CI - © Georg Thieme Verlag KG Stuttgart · New York. FAU - Ahrazoglu, M AU - Ahrazoglu M AD - Klinik und Poliklinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln. FAU - Moinzadeh, P AU - Moinzadeh P AD - Klinik und Poliklinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln. FAU - Hunzelmann, N AU - Hunzelmann N AD - Klinik und Poliklinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln. LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Differenzialdiagnosen des Raynaud-Syndroms. DEP - 20140506 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Arterial Occlusive Diseases/*diagnosis MH - Diabetes Mellitus, Type 1/*diagnosis MH - Diagnosis, Differential MH - Humans MH - Physical Examination/*methods MH - Raynaud Disease/*diagnosis MH - Rheumatic Diseases/*diagnosis EDAT- 2014/05/08 06:00 MHDA- 2014/06/26 06:00 CRDT- 2014/05/08 06:00 PHST- 2014/05/08 06:00 [entrez] PHST- 2014/05/08 06:00 [pubmed] PHST- 2014/06/26 06:00 [medline] AID - 10.1055/s-0034-1370036 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2014 May;139(20):1064-9. doi: 10.1055/s-0034-1370036. Epub 2014 May 6. PMID- 36124934 OWN - NLM STAT- MEDLINE DCOM- 20230828 LR - 20230828 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 33 IP - 5 DP - 2023 Aug 25 TI - Arm heating to relieve Raynaud's phenomenon in systemic sclerosis: A single-arm multicentre prospective clinical trial. PG - 968-974 LID - 10.1093/mr/roac116 [doi] AB - OBJECTIVES: Raynaud's phenomenon, one of the major symptoms of systemic sclerosis (SSc), is difficult to treat. Although it is empirically considered that warming is a beneficial technique, there is no supportive evidence. We conducted a multicentre study to evaluate whether continuous heating of the arm alleviates Raynaud's phenomenon in SSc. METHODS: A pair of disposable warmers was applied to the upper arm near the elbow of patients with SSc. Two weeks of non-warmer application were followed by 2 weeks of warmer application, which was repeated twice. The Raynaud Condition Score (RCS), number of episodes, and duration of Raynaud's phenomenon were recorded. The differences in the mean RCS, frequency, and duration of Raynaud's phenomenon between the warmer application and non-application periods were analysed. RESULTS: Twenty-eight patients were included in the analysis. The average RCS was 1.98 and 2.66 during the warmer application and non-application periods, respectively. The change between the two periods was statistically significant by paired t-test. In addition, the frequency and total duration of Raynaud's phenomenon in the warmer application period were significantly lower than those in the non-application period. CONCLUSIONS: Heating of the upper arm near the elbow is effective in alleviating Raynaud's phenomenon in SSc. CI - © Japan College of Rheumatology 2022. Published by Oxford University Press. FAU - Watanabe, Akane AU - Watanabe A AUID- ORCID: 0000-0001-8451-9501 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Osaka, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Osaka, Japan. FAU - Shima, Yoshihito AU - Shima Y AUID- ORCID: 0000-0002-2523-2938 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Osaka, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Osaka, Japan. FAU - Takahashi, Hiroki AU - Takahashi H AD - Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Hokkaido, Japan. FAU - Akiyama, Yuji AU - Akiyama Y AD - Division of Rheumatology, Department of Internal Medicine, Ogawa Red Cross Hospital, Saitama, Japan. AD - Department of Rheumatology and Applied Immunology, Saitama Medical University, Saitama, Japan. FAU - Kodera, Masanari AU - Kodera M AD - Department of Dermatology, Japan Community Health Care Organization Chukyo Hospital, Aichi, Japan. FAU - Jinnin, Masatoshi AU - Jinnin M AD - Department of Dermatology, Wakayama Medical University Graduate School of Medicine, Wakayama, Japan. FAU - Azuma, Naoto AU - Azuma N AD - Department of Diabetes, Endocrinology and Clinical Immunology, Hyogo Medical University School of Medicine, Hyogo, Japan. FAU - Ishii, Koji AU - Ishii K AD - Department of Rheumatology, Oita Red Cross Hospital, Oita, Japan. FAU - Kumanogoh, Atsushi AU - Kumanogoh A AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Osaka, Japan. LA - eng PT - Journal Article PT - Multicenter Study PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Humans MH - Prospective Studies MH - Heating MH - *Scleroderma, Systemic/therapy/drug therapy MH - *Raynaud Disease/etiology/therapy OTO - NOTNLM OT - Clinical trial OT - Raynaud’s phenomenon OT - continuous heating OT - systemic sclerosis OT - warmer EDAT- 2022/09/21 06:00 MHDA- 2023/08/28 06:42 CRDT- 2022/09/20 06:23 PHST- 2022/07/11 00:00 [received] PHST- 2022/09/08 00:00 [revised] PHST- 2022/09/13 00:00 [accepted] PHST- 2023/08/28 06:42 [medline] PHST- 2022/09/21 06:00 [pubmed] PHST- 2022/09/20 06:23 [entrez] AID - 6705070 [pii] AID - 10.1093/mr/roac116 [doi] PST - ppublish SO - Mod Rheumatol. 2023 Aug 25;33(5):968-974. doi: 10.1093/mr/roac116. PMID- 26734864 OWN - NLM STAT- MEDLINE DCOM- 20160601 LR - 20240522 IS - 1806-4841 (Electronic) IS - 0365-0596 (Print) IS - 0365-0596 (Linking) VI - 90 IP - 6 DP - 2015 Nov-Dec TI - Systemic lupus erythematosus and Raynaud's phenomenon. PG - 837-40 LID - S0365-05962015000600837 [pii] LID - 10.1590/abd1806-4841.20153881 [doi] AB - BACKGROUND: Patients with systemic lupus erythematosus seem to belong to different serological and clinical subgroups of the disease. Genetic background can cause the appearance of these subgroups. OBJECTIVE: To determine whether Brazilian patients who have systemic lupus erythematosus and Raynaud's phenomenon differ from those who do not. METHODS: Retrospective analysis of 373 medical records of systemic lupus erythematosus patients studied for demographic, clinical and serological data. A comparative analysis was performed of individuals with and without RP. RESULTS: There was a positive association between Raynaud's phenomenon and age at diagnosis (p=0.02), presence of anti-Sm (p=0.01) antibodies and anti-RNP (p<0.0001). Furthermore, a negative association was found between Raynaud's phenomenon and hemolysis (p=0.01), serositis (p=0.01), glomerulonephritis (p=0.0004) and IgM aCL (p=0.004) antibodies. CONCLUSION: Raynaud's phenomenon patients appear to belong to a systemic lupus erythematosus subset with a spectrum of clinical manifestations located in a more benign pole of the disease. FAU - Heimovski, Flavia Emilie AU - Heimovski FE AD - Faculdade Evangélica do Paraná, Curitiba, PR, Brazil. FAU - Simioni, Juliana A AU - Simioni JA AD - Faculdade Evangélica do Paraná, Curitiba, PR, Brazil. FAU - Skare, Thelma Larocca AU - Skare TL AD - Faculdade Evangélica do Paraná, Curitiba, PR, Brazil. LA - eng PT - Comparative Study PT - Journal Article PL - Spain TA - An Bras Dermatol JT - Anais brasileiros de dermatologia JID - 0067662 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/analysis MH - Brazil MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/immunology/pathology/*physiopathology MH - Male MH - Medical Records MH - Middle Aged MH - Raynaud Disease/immunology/pathology/*physiopathology MH - Retrospective Studies MH - Statistics, Nonparametric MH - Young Adult PMC - PMC4689071 COIS- Conflict of Interest: None EDAT- 2016/01/07 06:00 MHDA- 2016/06/02 06:00 PMCR- 2015/11/01 CRDT- 2016/01/07 06:00 PHST- 2014/07/26 00:00 [received] PHST- 2015/02/17 00:00 [accepted] PHST- 2016/01/07 06:00 [entrez] PHST- 2016/01/07 06:00 [pubmed] PHST- 2016/06/02 06:00 [medline] PHST- 2015/11/01 00:00 [pmc-release] AID - S0365-05962015000600837 [pii] AID - 10.1590/abd1806-4841.20153881 [doi] PST - ppublish SO - An Bras Dermatol. 2015 Nov-Dec;90(6):837-40. doi: 10.1590/abd1806-4841.20153881. PMID- 36357109 OWN - NLM STAT- MEDLINE DCOM- 20221114 LR - 20241111 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 15 IP - 11 DP - 2022 Nov 10 TI - Lingual Raynaud's phenomenon: a rare presentation. LID - 10.1136/bcr-2022-251988 [doi] LID - e251988 AB - A woman in her mid-60s presented to transient ischaemic attack (TIA) clinic with a 3-year history of intermittent sensory changes and white discolouration affecting the left side of her tongue. Following extensive investigation, a provisional diagnosis of posterior circulation TIA was made, and the patient was commenced on clopidogrel therapy. Despite anti-platelet treatment, she continued to have identical episodic symptoms. She was referred to the rheumatology team for assessment of possible underlying autoimmune pathology. On rheumatology assessment, the patient reported colour changes on the tongue, associated with numbness, followed by paraesthesia of the affected area. A comprehensive assessment excluded secondary causes and a diagnosis of primary Raynaud's phenomenon of the tongue was made. The diagnosis of TIA was revoked. This case illustrates a rare presentation of a common condition and highlights the sensory symptoms which are associated with Raynaud's phenomenon. CI - © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Biddle, Kathryn AU - Biddle K AUID- ORCID: 0000-0001-5267-200X AD - Rheumatology, St George's Healthcare NHS Trust, London, UK kathryn.biddle@nhs.net. FAU - Kaul, Arvind AU - Kaul A AD - Rheumatology, St George's Healthcare NHS Trust, London, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20221110 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Female MH - Humans MH - *Ischemic Attack, Transient/complications MH - *Raynaud Disease/etiology MH - Tongue PMC - PMC9660575 OTO - NOTNLM OT - Connective tissue disease OT - Rheumatology COIS- Competing interests: None declared. EDAT- 2022/11/11 06:00 MHDA- 2022/11/15 06:00 PMCR- 2024/11/10 CRDT- 2022/11/10 21:06 PHST- 2022/11/10 21:06 [entrez] PHST- 2022/11/11 06:00 [pubmed] PHST- 2022/11/15 06:00 [medline] PHST- 2024/11/10 00:00 [pmc-release] AID - 15/11/e251988 [pii] AID - bcr-2022-251988 [pii] AID - 10.1136/bcr-2022-251988 [doi] PST - epublish SO - BMJ Case Rep. 2022 Nov 10;15(11):e251988. doi: 10.1136/bcr-2022-251988. PMID- 26602360 OWN - NLM STAT- MEDLINE DCOM- 20170424 LR - 20170424 IS - 1885-1398 (Electronic) IS - 1699-258X (Linking) VI - 12 IP - 6 DP - 2016 Nov-Dec TI - Raynaud's phenomenon in children. PG - 342-344 LID - S1699-258X(15)00153-9 [pii] LID - 10.1016/j.reuma.2015.08.008 [doi] AB - Raynaud's Phenomenon is caused by spasm of the small arteries and arterioles of the fingers. It is triggered by various stimuli including exposure to cold or a stressful event. It may be symmetrical or wrap one end. The appearance of this entity in children is rare. We report the case of a 4 year old male consultation health center by episodes of coldness, pallor and pain in both feet. CI - Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Ortega Vicente, Elena AU - Ortega Vicente E AD - Médico especialista en Pediatría, CS Tórtola, Valladolid, España. Electronic address: elenaovicente@gmail.com. FAU - Garrido Redondo, Mercedes AU - Garrido Redondo M AD - Médico especialista en Pediatría, CS Tórtola, Valladolid, España. LA - eng LA - spa PT - Case Reports PT - Journal Article TT - Fenómeno de Raynaud en pediatría. DEP - 20151119 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 SB - IM MH - Child, Preschool MH - Foot MH - Humans MH - Male MH - Raynaud Disease/*diagnosis OTO - NOTNLM OT - Blush OT - Capilaroscopia OT - Capillaroscopy OT - Cianosis OT - Cyanosis OT - Fénomeno de Raynaud OT - Paleness OT - Palidez OT - Pediatrics OT - Pediatría OT - Raynaud's Phenomenon OT - Rubor EDAT- 2015/11/26 06:00 MHDA- 2017/04/25 06:00 CRDT- 2015/11/26 06:00 PHST- 2015/06/09 00:00 [received] PHST- 2015/08/07 00:00 [revised] PHST- 2015/08/18 00:00 [accepted] PHST- 2015/11/26 06:00 [pubmed] PHST- 2017/04/25 06:00 [medline] PHST- 2015/11/26 06:00 [entrez] AID - S1699-258X(15)00153-9 [pii] AID - 10.1016/j.reuma.2015.08.008 [doi] PST - ppublish SO - Reumatol Clin. 2016 Nov-Dec;12(6):342-344. doi: 10.1016/j.reuma.2015.08.008. Epub 2015 Nov 19. PMID- 22315243 OWN - NLM STAT- MEDLINE DCOM- 20120514 LR - 20260128 IS - 1756-1833 (Electronic) IS - 0959-8138 (Linking) VI - 344 DP - 2012 Feb 7 TI - Diagnosis and management of Raynaud's phenomenon. PG - e289 LID - bmj.e289 [pii] LID - 10.1136/bmj.e289 [doi] FAU - Goundry, Beth AU - Goundry B AD - Department of Rheumatology, University Hospitals of Leicester NHS Trust, Leicester LE1 5WW, UK. FAU - Bell, Laura AU - Bell L FAU - Langtree, Matthew AU - Langtree M FAU - Moorthy, Arumugam AU - Moorthy A LA - eng PT - Journal Article PT - Review DEP - 20120207 PL - England TA - BMJ JT - BMJ (Clinical research ed.) JID - 8900488 RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Endothelin Receptor Antagonists MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - Referral and Consultation MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2012/02/09 06:00 MHDA- 2012/05/15 06:00 CRDT- 2012/02/09 06:00 PHST- 2012/02/09 06:00 [entrez] PHST- 2012/02/09 06:00 [pubmed] PHST- 2012/05/15 06:00 [medline] AID - bmj.e289 [pii] AID - 10.1136/bmj.e289 [doi] PST - epublish SO - BMJ. 2012 Feb 7;344:e289. doi: 10.1136/bmj.e289. PMID- 39198722 OWN - NLM STAT- MEDLINE DCOM- 20240829 LR - 20241210 IS - 1471-244X (Electronic) IS - 1471-244X (Linking) VI - 24 IP - 1 DP - 2024 Aug 28 TI - Raynaud's phenomenon on initiation of Lithium therapy: a case report. PG - 586 LID - 10.1186/s12888-024-06007-4 [doi] LID - 586 AB - Lithium Carbonate is an effective treatment for affective disorders, but has a range of side effects. This case report highlights a rare side effect of Raynaud's phenomenon following initiation of Lithium therapy in a patient with recurrent depressive disorder. He was commenced on Lithium therapy to treat severe treatment resistant depression with psychotic symptoms when alternative treatments trialled were ineffective. He had no other risk factors or known aetiological causes for development of Raynaud's phenomenon. Symptoms resolved on discontinuation of Lithium and re-emerged on recommencement. Previous case series have shown Lithium effectively treating vasospastic disorders such as cluster headache and Raynaud's phenomenon. However, a paradoxical reaction to those previously described was induced in this case. CI - © 2024. The Author(s). FAU - Boland, Cailín AU - Boland C AD - Consultant Psychiatrist, Cork, Ireland. cailin.boland@gmail.com. FAU - Adams, Emma AU - Adams E AD - Consultant Psychiatrist, Mid-East Kildare Mental Health Services, Kildare, Ireland. FAU - Wong, Man Ching AU - Wong MC AD - Consultant Psychiatrist, Finglas CMHT, CHO9, Dublin, Ireland. FAU - Denihan, Cian AU - Denihan C AD - Consultant Psychiatrist, Dublin, Ireland. LA - eng PT - Case Reports PT - Journal Article DEP - 20240828 PL - England TA - BMC Psychiatry JT - BMC psychiatry JID - 100968559 RN - 0 (Antimanic Agents) RN - 2BMD2GNA4V (Lithium Carbonate) SB - IM MH - Humans MH - Male MH - Antimanic Agents/administration & dosage/adverse effects MH - Depressive Disorder, Treatment-Resistant/drug therapy MH - *Lithium Carbonate/administration & dosage/adverse effects MH - *Raynaud Disease/chemically induced PMC - PMC11361196 OTO - NOTNLM OT - Adverse effects OT - Depression OT - Depressive disorder OT - Lithium Carbonate OT - Raynaud disease COIS- The authors declare no competing interests. EDAT- 2024/08/31 09:53 MHDA- 2024/08/31 09:54 PMCR- 2024/08/28 CRDT- 2024/08/28 23:46 PHST- 2023/06/30 00:00 [received] PHST- 2024/08/08 00:00 [accepted] PHST- 2024/08/31 09:54 [medline] PHST- 2024/08/31 09:53 [pubmed] PHST- 2024/08/28 23:46 [entrez] PHST- 2024/08/28 00:00 [pmc-release] AID - 10.1186/s12888-024-06007-4 [pii] AID - 6007 [pii] AID - 10.1186/s12888-024-06007-4 [doi] PST - epublish SO - BMC Psychiatry. 2024 Aug 28;24(1):586. doi: 10.1186/s12888-024-06007-4. PMID- 29526628 OWN - NLM STAT- MEDLINE DCOM- 20180531 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 17 IP - 5 DP - 2018 May TI - The role of capillaroscopy and thermography in the assessment and management of Raynaud's phenomenon. PG - 465-472 LID - S1568-9972(18)30049-1 [pii] LID - 10.1016/j.autrev.2017.11.036 [doi] AB - Most patients with Raynaud's phenomenon (RP) have "benign" primary RP (PRP), but a minority have an underlying cause, for example a connective tissue disease such as systemic sclerosis (SSc). Secondary RP can be associated with structural as well as functional digital vascular changes and can be very severe, potentially progressing to digital ulceration or gangrene. The first step in management is to establish why the patient has RP. This short review discusses the role of nailfold capillaroscopy and thermography in the assessment of RP. Nailfold capillaroscopy examines microvascular structure, which is normal in PRP but abnormal in most patients with SSc: the inclusion of abnormal nailfold capillaries into the 2013 classification criteria for SSc behoves clinicians diagnosing connective tissue disease to be familiar with the technique. For those without access to the gold standard of high magnification videocapillaroscopy, a low magnification dermatoscope or USB microscope can be used. Thermography measures surface temperature and is therefore an indirect measure of blood blow, assessing digital vascular function (abnormal in both PRP and SSc). Until now, the use of thermography has been mainly confined to specialist centres and used mainly in research: this may change with development of mobile phone thermography. CI - Copyright © 2018 Elsevier B.V. All rights reserved. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: ariane.herrick@manchester.ac.uk. FAU - Murray, Andrea AU - Murray A AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20180309 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Aged MH - Female MH - Humans MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/*diagnostic imaging/therapy MH - Thermography/*methods OTO - NOTNLM OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - Themography EDAT- 2018/03/13 06:00 MHDA- 2018/06/01 06:00 CRDT- 2018/03/13 06:00 PHST- 2017/11/19 00:00 [received] PHST- 2017/11/26 00:00 [accepted] PHST- 2018/03/13 06:00 [pubmed] PHST- 2018/06/01 06:00 [medline] PHST- 2018/03/13 06:00 [entrez] AID - S1568-9972(18)30049-1 [pii] AID - 10.1016/j.autrev.2017.11.036 [doi] PST - ppublish SO - Autoimmun Rev. 2018 May;17(5):465-472. doi: 10.1016/j.autrev.2017.11.036. Epub 2018 Mar 9. PMID- 37838733 OWN - NLM STAT- MEDLINE DCOM- 20231023 LR - 20231122 IS - 1471-2482 (Electronic) IS - 1471-2482 (Linking) VI - 23 IP - 1 DP - 2023 Oct 14 TI - Efficacy analysis of minimally invasive surgery for Raynaud's syndrome. PG - 313 LID - 10.1186/s12893-023-02225-x [doi] LID - 313 AB - BACKGROUND: Raynaud's syndrome (RS), also referred to as Raynaud's phenomenon, is a vasospastic disorder causing episodic color changes in extremities upon exposure to cold or stress. These manifestations, either primary Raynaud's phenomenon (PRP) or associated with connective tissue diseases like systemic sclerosis (SSc) as secondary Raynaud's phenomenon (SRP), affect the quality of life. Current treatments range from calcium channel blockers to innovative surgical interventions, with evolving efficacy and safety profiles. METHODS: In this retrospective study, patients diagnosed with RS were selected based on complete medical records, ensuring homogeneity between groups. Surgeries involved microscopic excision of sympathetic nerve fibers and stripping of the digital artery's adventitia. Postoperative care included antibiotics, analgesia, oral nifedipine, and heat therapies. Evaluation metrics such as the VAS pain score and RCS score were collected bi-weekly. Data analysis was conducted using SPSS 26.0, with significance set at p < 0.05. RESULTS: In total, 15 patients formed the experimental group, with five presenting fingertip soft tissue necrosis and ten showing RS symptoms. Comparative analysis of demographic data between experimental and control groups, both containing 15 participants, demonstrated no significant age and gender difference. However, the "Mean Duration of RP attack" in the experimental group was notably shorter (9.47 min ± 0.31) than the control group (19.33 min ± 1.79). The RS Severity Score also indicated milder severity for the experimental cohort (score: 8.55) compared to the control (score: 11.23). Postoperative assessments at 2, 4, and 6 weeks revealed improved VAS pain scores, RCS scores, and other measures for the experimental group, showing significant differences (p < 0.05). One distinctive case showcased a variation in the common digital nerve and artery course in an RS patient. CONCLUSION: Our retrospective analysis on RS patients indicates that microsurgical techniques are safe and effective in the short term. As surgical practices lean towards minimally invasive methods, our data supports this shift. However, extensive, prospective studies are essential for conclusive insights. CI - © 2023. BioMed Central Ltd., part of Springer Nature. FAU - Yu, Fengwei AU - Yu F AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Liu, Yongtao AU - Liu Y AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. 1752703560@qq.com. FAU - Zhang, Chengnian AU - Zhang C AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Pang, Botao AU - Pang B AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Zhang, Daijie AU - Zhang D AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Zhao, Wei AU - Zhao W AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Li, Xuecheng AU - Li X AD - Hand Microsurgery, Binzhou Medical University Hospital, Binzhou, 256600, China. FAU - Yang, Weiqiang AU - Yang W AD - The First Clinical School of Binzhou Medical University, Binzhou, 256600, China. LA - eng GR - SLCZDZK-0303/Specialty construction foundation: The clinical specialty construction foundation of Shandong Province (2020)/ GR - SLCZDZK-0303/Specialty construction foundation: The clinical specialty construction foundation of Shandong Province (2020)/ PT - Journal Article DEP - 20231014 PL - England TA - BMC Surg JT - BMC surgery JID - 100968567 SB - IM EIN - BMC Surg. 2023 Nov 16;23(1):347. doi: 10.1186/s12893-023-02265-3. PMID: 37974138 MH - Humans MH - Retrospective Studies MH - *Quality of Life MH - Prospective Studies MH - *Raynaud Disease/surgery/complications MH - Minimally Invasive Surgical Procedures/adverse effects MH - Pain/complications PMC - PMC10576400 OTO - NOTNLM OT - Adventitial release OT - Hand OT - Microsurgery OT - Raynaud’s syndrome OT - Surgical treatment OT - Sympathectomy COIS- The authors declare no competing interests. EDAT- 2023/10/15 05:46 MHDA- 2023/10/23 01:18 PMCR- 2023/10/14 CRDT- 2023/10/14 23:26 PHST- 2023/07/15 00:00 [received] PHST- 2023/10/08 00:00 [accepted] PHST- 2023/10/23 01:18 [medline] PHST- 2023/10/15 05:46 [pubmed] PHST- 2023/10/14 23:26 [entrez] PHST- 2023/10/14 00:00 [pmc-release] AID - 10.1186/s12893-023-02225-x [pii] AID - 2225 [pii] AID - 10.1186/s12893-023-02225-x [doi] PST - epublish SO - BMC Surg. 2023 Oct 14;23(1):313. doi: 10.1186/s12893-023-02225-x. PMID- 39591227 OWN - NLM STAT- MEDLINE DCOM- 20241126 LR - 20250609 IS - 2072-6651 (Electronic) IS - 2072-6651 (Linking) VI - 16 IP - 11 DP - 2024 Nov 1 TI - Botulinum Toxin for the Treatment of Raynaud's Conditions of the Hand: Clinical Practice Updates and Future Directions. LID - 10.3390/toxins16110472 [doi] LID - 472 AB - Raynaud's conditions of the hand, referred to commonly as Raynaud's phenomenon, both primary and secondary, represents a spectrum of disorders affecting the digits, characterised by recurrent episodes of vasospasm that result in a triad of symptoms: pain, pallor, and cyanosis. Various therapies, ranging from conservative hand therapy techniques to surgical sympathectomy, have been explored with inconsistent results. Recently, the local administration of botulinum toxin type-A (BTX-A) has re-emerged as a treatment option for this condition. This review delves into the mechanistic pathways of BTX-A therapy, optimal dosing concentrations, administration techniques, and its safety profile. A critical analysis of published studies to date demonstrates varied clinical efficacy of BTX-A in Raynaud's conditions based on patient-reported outcome measures and objective measures of outcomes assessment. Thus, in order to accurately assess the clinical effectiveness of BTX-A in future robust studies, this review emphasises the importance of streamlining patient selection to minimise heterogeneity in disease severity, optimising recruitment to ensure adequate statistical power, and establishing sensitive outcome measures to monitor response and discern treatment efficacy. Additionally, addressing concerns such as minimising antibody resistance, extending the duration of treatment effects on tissues, and exploring new modalities to assess hand perfusion will be focal points for future research and BTX-A drug development. FAU - O'Donohoe, Patrick AU - O'Donohoe P AUID- ORCID: 0000-0002-7323-1454 AD - Department of Plastic & Reconstructive Surgery, St. Vincent's University Hospital, D04T6F4 Dublin, Ireland. AD - Department of Surgery, Royal College of Surgeons in Ireland, D02YN77 Dublin, Ireland. FAU - McDonnell, Jake AU - McDonnell J AD - Department of Surgery, Royal College of Surgeons in Ireland, D02YN77 Dublin, Ireland. FAU - Wormald, Justin AU - Wormald J AD - Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford OX1 2DJ, UK. AD - Department of Plastic & Reconstructive Surgery, John Radcliffe Hospital, Oxford OX3 9DU, UK. FAU - Aljohmani, Lylas AU - Aljohmani L AD - Department of Plastic & Reconstructive Surgery, St. Vincent's University Hospital, D04T6F4 Dublin, Ireland. FAU - Cronin, Kevin AU - Cronin K AD - Department of Plastic & Reconstructive Surgery, Mater Misericordiae University Hospital, D07R2WY Dublin, Ireland. FAU - Durcan, Laura AU - Durcan L AD - Department of Rheumatology, Beaumont Hospital, D09V2N0 Dublin, Ireland. FAU - Kennedy, Oran AU - Kennedy O AUID- ORCID: 0000-0003-3343-2873 AD - Tissue Engineering Research Group, Department of Anatomy & Regenerative Medicine, Royal College of Surgeons in Ireland, D02YN77 Dublin, Ireland. AD - Trinity Center for Biomedical Engineering, Trinity College Dublin, D02PN40 Dublin, Ireland. AD - Advanced Materials and Bioengineering Research Centre (AMBER), D02PN40 Dublin, Ireland. FAU - Dolan, Roisin AU - Dolan R AUID- ORCID: 0000-0003-0455-7343 AD - Department of Plastic & Reconstructive Surgery, St. Vincent's University Hospital, D04T6F4 Dublin, Ireland. AD - UCD School of Medicine & Medical Sciences, University College Dublin, D04V1W8 Dublin, Ireland. LA - eng PT - Journal Article PT - Review DEP - 20241101 PL - Switzerland TA - Toxins (Basel) JT - Toxins JID - 101530765 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Neuromuscular Agents) SB - IM MH - Humans MH - *Raynaud Disease/drug therapy MH - *Botulinum Toxins, Type A/therapeutic use/administration & dosage MH - *Hand MH - Neuromuscular Agents/therapeutic use MH - Treatment Outcome PMC - PMC11598569 OTO - NOTNLM OT - Raynaud’s disease OT - Raynaud’s phenomenon OT - botulinum toxin OT - patient-reported outcomes COIS- The authors declare no conflicts of interest. EDAT- 2024/11/26 18:22 MHDA- 2024/11/26 18:23 PMCR- 2024/11/01 CRDT- 2024/11/26 13:23 PHST- 2024/10/08 00:00 [received] PHST- 2024/10/29 00:00 [revised] PHST- 2024/10/30 00:00 [accepted] PHST- 2024/11/26 18:23 [medline] PHST- 2024/11/26 18:22 [pubmed] PHST- 2024/11/26 13:23 [entrez] PHST- 2024/11/01 00:00 [pmc-release] AID - toxins16110472 [pii] AID - toxins-16-00472 [pii] AID - 10.3390/toxins16110472 [doi] PST - epublish SO - Toxins (Basel). 2024 Nov 1;16(11):472. doi: 10.3390/toxins16110472. PMID- 22039719 OWN - NLM STAT- MEDLINE DCOM- 20111229 LR - 20111101 IS - 0035-2640 (Print) IS - 0035-2640 (Linking) VI - 61 IP - 7 DP - 2011 Sep TI - [Raynaud's phenomenon, disease or syndrome?]. PG - 899-903 AB - Raynaud's phenomenon is a common symptom. More often it is usually an idiopathic and benign condition. But it can be an early manifestation of a connective tissue disease especially scleroderma and primary Sjogren's syndrom. Thus it is necessary to develop reasonable screening model. If the vasomotor symptoms are localized, a diagnosis of secondary Raynaud's phenomenon is highly probable and the main etiology is an arterial disease. Occupational arterial lesions are a particularly aspect of secondary Raynaud's phenomenon. Calcium channel blockers are the reference for the symptomatic treatment of Raynaud's phenomenon. In severe secondary forms, intravenous iloprost infusion is effective. New drugs as endothelin antagonist and phospodiesterase type 5 inhibitors are still to be evaluated. FAU - Fiessinger, Jean-Noël AU - Fiessinger JN AD - Hôpital européen Georges-Pompidou, service de médecine vasculaire et HTA, 75908 Paris Cedex 15. jean-noel.fiessinger@egp.aphp.fr LA - fre PT - English Abstract PT - Journal Article TT - Phénomène de Raynaud, maladie ou syndrome? PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Humans MH - Raynaud Disease/*diagnosis/etiology/therapy EDAT- 2011/11/02 06:00 MHDA- 2011/12/30 06:00 CRDT- 2011/11/02 06:00 PHST- 2011/11/02 06:00 [entrez] PHST- 2011/11/02 06:00 [pubmed] PHST- 2011/12/30 06:00 [medline] PST - ppublish SO - Rev Prat. 2011 Sep;61(7):899-903. PMID- 17917543 OWN - NLM STAT- MEDLINE DCOM- 20080201 LR - 20071005 IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 19 IP - 6 DP - 2007 Nov TI - Current drug therapy for scleroderma and secondary Raynaud's phenomenon: evidence-based review. PG - 611-8 AB - (1) Scleroderma and secondary Raynaud's phenomenon are frequently associated with increased morbidity for which no specific standardised treatment guidelines exist. (2) Current therapies for scleroderma target the immune system, with the goal of reducing inflammation and secondary tissue injury and fibrosis. Therapy targeting underlying vascular disease is designed to improve the symptoms of Raynaud's phenomenon and to reduce ischemic injury to involved organs. (3) Few controlled trials of therapy used for scleroderma are completed, and current treatments are largely based on organ-specific therapy and uncontrolled case series suggesting disease modification. (4) Recent randomised, controlled trials in scleroderma demonstrate promising results in the treatment of interstitial lung disease with cyclophosphamide, and vascular disease of the lungs and digits with endothelin receptor antagonists, the phosphodiesterase inhibitor sildenafil and prostacyclins, while trials with methotrexate show only modest benefit in controlling scleroderma-associated skin disease. (5) Prostacyclins are a therapeutic option in patients with secondary Raynaud's phenomenon. Modest benefits have also been shown with alpha1-antagonists and calcium channel blockers, while the effect of ACE inhibitors has been variable. Some data suggest some benefits to the use of the phosphodiesterase inhibitor sildenafil, the serotonin uptake inhibitor fluoxetine and the angiotensin receptor inhibitor losartan. FAU - Henness, Sheridan AU - Henness S AD - Wolters Kluwer Health | Adis, Auckland, New Zealand. FAU - Wigley, Fredrick M AU - Wigley FM LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Clinical Trials as Topic MH - Evidence-Based Medicine MH - Humans MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Systemic/*complications/*drug therapy RF - 51 EDAT- 2007/10/06 09:00 MHDA- 2008/02/02 09:00 CRDT- 2007/10/06 09:00 PHST- 2007/10/06 09:00 [pubmed] PHST- 2008/02/02 09:00 [medline] PHST- 2007/10/06 09:00 [entrez] AID - 00002281-200711000-00015 [pii] AID - 10.1097/BOR.0b013e3282f13137 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2007 Nov;19(6):611-8. doi: 10.1097/BOR.0b013e3282f13137. PMID- 35135008 OWN - NLM STAT- MEDLINE DCOM- 20220707 LR - 20220810 IS - 1526-4637 (Electronic) IS - 1526-2375 (Linking) VI - 23 IP - 7 DP - 2022 Jul 1 TI - Sympathetic Blocks for Raynaud's Phenomena in Pediatric Rheumatological Disorders. PG - 1211-1216 LID - 10.1093/pm/pnac015 [doi] AB - OBJECTIVE: Sympathetic blocks are invaluable to prevent morbidity from Raynaud's phenomenon (RP). RP may occur in children with rheumatological disorders and causes severe pain, discoloration of digits, gangrene, and auto-amputation. We describe the planning and execution of sympathectomy blocks in children with rheumatological disorders presenting with RP. METHODS: With upper-limb involvement, ultrasound-guided stellate ganglion block (USGB) was given with ropivacaine and clonidine. When all four limbs were involved, intrathecal block with bupivacaine and clonidine was also given. RESULTS: A total of 68 sympathectomy blocks were performed: 28 bilateral USGBs, two unilateral USGBs, and 10 intrathecal injections. Multiple interventions in a single day were frequently required. For safety, all USGBs were performed with an ultrasound with strict adherence to local anaesthetic volume was maintained, with periprocedure monitoring of 2-3 hours. All blocks were performed by an experienced specialist. All children reported immediate pain relief with prevention of major amputation. CONCLUSION: With meticulous planning, monitoring, and precautions, sympathectomy of limbs in pediatric rheumatological disorders with RP can be safely undertaken. Bilateral stellate ganglion block with ultrasound is safe in children, and clonidine is a useful adjunct for vasodilation and prolongation of the effect of sympathectomies in children. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. FAU - Punj, Jyotsna AU - Punj J AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Garg, Heena AU - Garg H AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Gomez, Gaurav AU - Gomez G AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Bagri, Narendra Kumar AU - Bagri NK AD - Department of Paediatrics, Subunit of Paediatric Rheumatology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Thakur, Jay Prakash AU - Thakur JP AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Singh, Longjam Darendrajit AU - Singh LD AUID- ORCID: 0000-0002-7461-1328 AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Jain, Dhruv AU - Jain D AUID- ORCID: 0000-0002-0343-8126 AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Darlong, V AU - Darlong V AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. FAU - Pandey, R AU - Pandey R AD - Department of Anaesthesiology, Pain Medicine and Critical Care, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. LA - eng PT - Journal Article PL - England TA - Pain Med JT - Pain medicine (Malden, Mass.) JID - 100894201 RN - MN3L5RMN02 (Clonidine) SB - IM MH - *Autonomic Nerve Block MH - Child MH - Clonidine/therapeutic use MH - Humans MH - Pain/complications MH - *Raynaud Disease/etiology/surgery MH - *Rheumatic Diseases/complications OTO - NOTNLM OT - Children OT - Intrathecal OT - Pediatric OT - Raynaud’s Phenomenon OT - Rheumatology Disorders OT - Stellate Ganglion Block OT - Sympathectomy EDAT- 2022/02/09 06:00 MHDA- 2022/07/08 06:00 CRDT- 2022/02/08 20:23 PHST- 2021/10/22 00:00 [received] PHST- 2022/01/08 00:00 [revised] PHST- 2022/01/18 00:00 [accepted] PHST- 2022/02/09 06:00 [pubmed] PHST- 2022/07/08 06:00 [medline] PHST- 2022/02/08 20:23 [entrez] AID - 6522132 [pii] AID - 10.1093/pm/pnac015 [doi] PST - ppublish SO - Pain Med. 2022 Jul 1;23(7):1211-1216. doi: 10.1093/pm/pnac015. PMID- 35239979 OWN - NLM STAT- MEDLINE DCOM- 20221115 LR - 20230222 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 61 IP - 12 DP - 2022 Dec TI - Raynaud's phenomenon and splinter hemorrhage: an early telltale sign of cannabis arteritis. PG - e480-e483 LID - 10.1111/ijd.16143 [doi] FAU - Palaniappan, Vijayasankar AU - Palaniappan V AD - Department of Dermatology, Venereology and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India. FAU - Sadhasivamohan, Anusuya AU - Sadhasivamohan A AD - Department of Dermatology, Venereology and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India. FAU - Karthikeyan, Kaliaperumal AU - Karthikeyan K AD - Department of Dermatology, Venereology and Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Pondicherry, India. LA - eng PT - Comment PT - Letter DEP - 20220303 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 SB - IM CON - J Cutan Med Surg. 2018 Mar/Apr;22(2):194-199. doi: 10.1177/1203475417738971. PMID: 29056081 MH - Humans MH - *Cannabis MH - *Raynaud Disease/diagnosis/etiology MH - *Arteritis MH - *Skin Diseases MH - Hemorrhage EDAT- 2022/03/04 06:00 MHDA- 2022/11/16 06:00 CRDT- 2022/03/03 17:15 PHST- 2021/12/17 00:00 [revised] PHST- 2021/10/31 00:00 [received] PHST- 2022/02/06 00:00 [accepted] PHST- 2022/03/04 06:00 [pubmed] PHST- 2022/11/16 06:00 [medline] PHST- 2022/03/03 17:15 [entrez] AID - 10.1111/ijd.16143 [doi] PST - ppublish SO - Int J Dermatol. 2022 Dec;61(12):e480-e483. doi: 10.1111/ijd.16143. Epub 2022 Mar 3. PMID- 19689931 OWN - NLM STAT- MEDLINE DCOM- 20100329 LR - 20131121 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 9 IP - 3 DP - 2010 Jan TI - Homocysteine and Raynaud's phenomenon: a review. PG - 181-7 LID - 10.1016/j.autrev.2009.08.004 [doi] AB - Raynaud's phenomenon, categorized as primary and secondary when occurring isolated or in association with an underlying disease, respectively, is a paroxysmal and recurrent acral ischemia resulting from an abnormal arterial vasospastic response to cold or emotional stress. The key issue in the pathogenesis of Raynaud's phenomenon is presumed to be a dysregulation in the mechanisms of vascular motility resulting in an imbalance between vasodilatation and vasoconstriction. Homocysteine, a non-protein forming sulphured amino acid proposed as an independent risk factor for atherothrombosis in the general population, clearly demonstrated to produce vascular damage through mechanisms also including endothelial injury and modifications in circulating mediators of vasomotion. The rationale for homocysteine involvement in the pathogenesis of Raynaud's phenomenon led some authors to investigate the possible association between mild hyperhomocysteinemia and such a vascular disturbance, particularly in the course of connective tissue disease. Here we review data regarding this putative association and the supposed mechanisms involved, also discussing the emblematic case of a patient with new-onset severe Raynaud's phenomenon and markedly elevated homocysteinemia. CI - Copyright 2009 Elsevier B.V. All rights reserved. FAU - Lazzerini, Pietro Enea AU - Lazzerini PE AD - Department of Clinical Medicine and Immunological Sciences, University of Siena, Italy. pietroenea@yahoo.it FAU - Capecchi, Pier Leopoldo AU - Capecchi PL FAU - Bisogno, Stefania AU - Bisogno S FAU - Cozzalupi, Mauro AU - Cozzalupi M FAU - Rossi, Pier Carlo AU - Rossi PC FAU - Pasini, Franco Laghi AU - Pasini FL LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20090815 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0LVT1QZ0BA (Homocysteine) SB - IM MH - Adult MH - Animals MH - Female MH - Homocysteine/*blood MH - Humans MH - Raynaud Disease/*blood/immunology RF - 40 EDAT- 2009/08/20 09:00 MHDA- 2010/03/30 06:00 CRDT- 2009/08/20 09:00 PHST- 2009/07/29 00:00 [received] PHST- 2009/08/10 00:00 [accepted] PHST- 2009/08/20 09:00 [entrez] PHST- 2009/08/20 09:00 [pubmed] PHST- 2010/03/30 06:00 [medline] AID - S1568-9972(09)00143-8 [pii] AID - 10.1016/j.autrev.2009.08.004 [doi] PST - ppublish SO - Autoimmun Rev. 2010 Jan;9(3):181-7. doi: 10.1016/j.autrev.2009.08.004. Epub 2009 Aug 15. PMID- 27465880 OWN - NLM STAT- MEDLINE DCOM- 20170712 LR - 20220410 IS - 1542-6270 (Electronic) IS - 1060-0280 (Linking) VI - 50 IP - 12 DP - 2016 Dec TI - Evidence for the Use of Epoprostenol to Treat Raynaud's Phenomenon With or Without Digital Ulcers. PG - 1060-1067 AB - OBJECTIVE: To review the evidence for using intravenous (IV) epoprostenol to treat Raynaud's phenomenon (RP). DATA SOURCES: The databases MEDLINE (1946 to March 2016), PubMed, and International Pharmaceutical Abstracts were searched using the terms epoprostenol, Flolan, Raynaud's disease, and CREST syndrome. Further literature sources were identified by reviewing article citations. STUDY SELECTION AND DATA EXTRACTION: All English-language, clinical trials and case series evaluating IV epoprostenol for the management or treatment of RP were included. Lower-quality evidence were incorporated due to limited information. DATA SYNTHESIS: Seven small uncontrolled studies/case series, 1 small placebo controlled study, and 1 larger randomized trial were identified and included. There was no consistent measurement of efficacy utilized, but improvements in hand temperature, RP attack duration and frequency were commonly associated with IV epoprostenol treatment (5 trials). There were conflicting data regarding effect sustainability, with 5 trials showing long-term effects and 3 showing immediate effects. Fewer ischemic ulcers developed during treatment with IV epoprostenol in 1 trial compared to conventional treatment. Ulcer healing ocurred in 2 trials. Common adverse effects included hypotension, headache, flushing, gastrointestinal symptoms, and jaw pain. CONCLUSIONS: Available evidence supports the use of IV epoprostenol for treatment of severe RP in patients refractory or intolerant to standard therapies. The dose, titration schedule, and duration of IV epoprostenol utilized in studies varied, but a conservative approach to initiation should be considered. Patients who do not respond to intermittent infusions and have severe digital ischemia may require more aggressive regimens. CI - © The Author(s) 2016. FAU - Cruz, Joseph E AU - Cruz JE AD - Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA Joseph.Cruz@Rutgers.edu. AD - Department of Pharmacy, Englewood Hospital Medical Center, Englewood, NJ, USA. FAU - Ward, Ashley AU - Ward A AD - Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA. FAU - Anthony, Shannon AU - Anthony S AD - Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA. FAU - Chang, Susanna AU - Chang S AD - Montefiore Medical Center, Bronx, NY, USA. FAU - Bae, Hyun Billy AU - Bae HB AD - Englewood Hospital Medical Center, Englewood, NJ, USA. FAU - Hermes-DeSantis, Evelyn R AU - Hermes-DeSantis ER AD - Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA. AD - Department of Pharmacy, Robert Wood Johnson University Hospital, New Brunswick, NJ, USA. LA - eng PT - Journal Article PT - Review DEP - 20160726 PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Adult MH - Clinical Trials as Topic MH - Epoprostenol/administration & dosage/adverse effects/*therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Infusions, Intravenous MH - Ischemia/complications/drug therapy MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Ulcer/complications/*drug therapy OTO - NOTNLM OT - Flolan OT - PGI2 OT - Raynaud’s disease OT - epoprostenol OT - ischemic ulcers OT - prostacyclin OT - systemic sclerosis EDAT- 2016/11/05 06:00 MHDA- 2017/07/14 06:00 CRDT- 2016/07/29 06:00 PHST- 2016/11/05 06:00 [pubmed] PHST- 2017/07/14 06:00 [medline] PHST- 2016/07/29 06:00 [entrez] AID - 1060028016660324 [pii] AID - 10.1177/1060028016660324 [doi] PST - ppublish SO - Ann Pharmacother. 2016 Dec;50(12):1060-1067. doi: 10.1177/1060028016660324. Epub 2016 Jul 26. PMID- 40272049 OWN - NLM STAT- MEDLINE DCOM- 20250909 LR - 20250909 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 64 IP - 10 DP - 2025 Oct TI - An Evidence-Based Review of Perniosis (Chilblains): Therapeutic Strategies and Integration With Raynaud's Syndrome Management. PG - 1781-1787 LID - 10.1111/ijd.17812 [doi] AB - Perniosis (chilblains) is an uncommon inflammatory condition characterized by erythematous to violaceous papules or plaques typically affecting acral surfaces. The pathogenesis remains poorly understood, and its management remains challenging due to the limited evidence available. This comprehensive review examines evidence-based therapies for primary and secondary perniosis, informed by observational studies, case series, and trials on similar conditions such as Raynaud's phenomenon. We synthesize treatment options for managing inflammation, alleviating symptoms, and preventing recurrence, including topical and systemic vasodilators, corticosteroids, selective serotonin reuptake inhibitors (SSRIs), antimalarials, and antithrombotic therapies. A focus is placed on systemic vasodilators, including nifedipine, and topical therapies like nitroglycerin, which have shown efficacy in improving peripheral perfusion. Additionally, corticosteroids are used to address inflammation, although their effectiveness is debated in randomized controlled trials. Antimalarials and SSRIs, notably fluoxetine, also show promise, particularly for secondary perniosis. Despite the absence of high-quality randomized controlled trials, therapeutic recommendations have emerged from case reports and studies on related vascular conditions. The role of botulinum toxin, though under investigation, remains inconclusive for primary perniosis, but may be considered in cases of secondary perniosis or coexisting vascular issues. This review underscores the need for individualized treatment approaches and highlights the importance of future research to refine management strategies and enhance patient outcomes. CI - © 2025 the International Society of Dermatology. FAU - Sharifzadeh, Afsheen AU - Sharifzadeh A AUID- ORCID: 0000-0002-9941-5227 AD - Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA. AD - Dermatology Department, NYC Health + Hospitals/Metropolitan, New York, New York, USA. FAU - Smith, Gideon P AU - Smith GP AD - Harvard Medical School, Boston, Massachusetts, USA. LA - eng PT - Journal Article PT - Review DEP - 20250424 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - 0 (Vasodilator Agents) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Antimalarials) RN - 0 (Adrenal Cortex Hormones) RN - 0 (Fibrinolytic Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Humans MH - *Raynaud Disease/complications/drug therapy/therapy MH - Vasodilator Agents/therapeutic use MH - *Chilblains/drug therapy/therapy/etiology MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Antimalarials/therapeutic use MH - Evidence-Based Medicine MH - Adrenal Cortex Hormones/therapeutic use MH - Fibrinolytic Agents/therapeutic use MH - Nitroglycerin/therapeutic use OTO - NOTNLM OT - Raynaud's phenomenon OT - chilblains OT - evidence‐based therapies OT - pernio OT - perniosis OT - skin inflammation OT - systemic therapies for chilblains OT - systemic vasodilators OT - therapeutic guidelines in perniosis OT - vascular perfusion improvement EDAT- 2025/04/24 12:30 MHDA- 2025/09/09 12:31 CRDT- 2025/04/24 08:14 PHST- 2025/03/24 00:00 [revised] PHST- 2025/02/06 00:00 [received] PHST- 2025/04/15 00:00 [accepted] PHST- 2025/09/09 12:31 [medline] PHST- 2025/04/24 12:30 [pubmed] PHST- 2025/04/24 08:14 [entrez] AID - 10.1111/ijd.17812 [doi] PST - ppublish SO - Int J Dermatol. 2025 Oct;64(10):1781-1787. doi: 10.1111/ijd.17812. Epub 2025 Apr 24. PMID- 29512213 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 32 IP - 7 DP - 2018 Jul TI - The role of the dermatologist in Raynaud's phenomenon: a clinical challenge. PG - 1120-1127 LID - 10.1111/jdv.14914 [doi] AB - Raynaud's phenomenon (RP) is a functional vascular disorder involving extremities. In his practice, the dermatologist may frequently encounter RP which affects mainly women and is categorized into a primary benign form and a secondary form associated with different diseases (infections, drugs, autoimmune and vascular conditions, haematologic, rheumatologic and endocrinologic disorders). Still today, the differential diagnosis is a clinical challenge. Therefore, a careful history and a physical examination, together with laboratory tests and nailfold capillaroscopy, is mandatory. RP is generally benign, but a scheduled follow-up for primary RP patients should be established, due to risk of evolution to secondary RP. A combination of conservative measures and medications can help in the management of RP. The importance of avoiding all potential physical, chemical and emotional triggers, as well as quitting smoking, should be strongly suggested to the patient. As first-line treatment, dihydropyridine calcium channel blockers should be used. If this approach is not sufficient, prostacyclin derivatives, phosphodiesterases inhibitors and endothelin receptor antagonists can be considered as second-line treatment. In cases of acute ischaemia, nifedipine and intravenous prostanoids are helpful. In refractory cases, botulinum injections have shown a significant benefit. The approach to the RP patients requires therefore a coordinated care of specialists together with the primary care physician. CI - © 2018 European Academy of Dermatology and Venereology. FAU - Matucci-Cerinic, C AU - Matucci-Cerinic C AD - Dermatology Clinic - ASF, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. FAU - Nagaraja, V AU - Nagaraja V AD - Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. FAU - Prignano, F AU - Prignano F AD - Dermatology Clinic - ASF, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. FAU - Kahaleh, B AU - Kahaleh B AD - Department of Medicine, Division of Rheumatology, University of Toledo, Toledo, USA. FAU - Bellando-Randone, S AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20180411 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 SB - IM MH - *Dermatology MH - Fingers/*pathology MH - Gangrene/prevention & control MH - Humans MH - Ischemia/diagnosis/drug therapy/etiology MH - Microscopic Angioscopy MH - Necrosis/prevention & control MH - *Physician's Role MH - Raynaud Disease/complications/*diagnosis/diagnostic imaging/*therapy EDAT- 2018/03/08 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/03/08 06:00 PHST- 2017/09/22 00:00 [received] PHST- 2018/02/09 00:00 [accepted] PHST- 2018/03/08 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/03/08 06:00 [entrez] AID - 10.1111/jdv.14914 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2018 Jul;32(7):1120-1127. doi: 10.1111/jdv.14914. Epub 2018 Apr 11. PMID- 36192079 OWN - NLM STAT- MEDLINE DCOM- 20221005 LR - 20221103 IS - 1556-1380 (Electronic) IS - 1556-0864 (Linking) VI - 17 IP - 10 DP - 2022 Oct TI - Tumor-Associated Raynaud's Phenomenon Exacerbated by Administration of Immune Checkpoint Inhibitors. PG - 1233-1234 LID - S1556-0864(22)00343-4 [pii] LID - 10.1016/j.jtho.2022.07.004 [doi] FAU - Sumi, Toshiyuki AU - Sumi T AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan; Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan. Electronic address: tsh715@gmail.com. FAU - Michimata, Haruhiko AU - Michimata H AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan; Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan. FAU - Nagayama, Daiki AU - Nagayama D AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan; Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan. FAU - Koshino, Yuta AU - Koshino Y AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan; Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan. FAU - Watanabe, Hiroki AU - Watanabe H AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan. FAU - Yamada, Yuichi AU - Yamada Y AD - Department of Pulmonary Medicine, Hakodate Goryoukaku Hospital, Hokkaido, Japan. FAU - Chiba, Hirofumi AU - Chiba H AD - Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan. LA - eng PT - Journal Article PL - United States TA - J Thorac Oncol JT - Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer JID - 101274235 RN - 0 (Immune Checkpoint Inhibitors) SB - IM MH - Humans MH - Immune Checkpoint Inhibitors MH - *Lung Neoplasms/drug therapy MH - *Raynaud Disease/chemically induced/drug therapy OTO - NOTNLM OT - Immune checkpoint inhibitors OT - Lung cancer OT - Raynaud's phenomenon OT - Squamous cell carcinoma EDAT- 2022/10/04 06:00 MHDA- 2022/10/06 06:00 CRDT- 2022/10/03 21:03 PHST- 2022/03/29 00:00 [received] PHST- 2022/06/11 00:00 [revised] PHST- 2022/07/06 00:00 [accepted] PHST- 2022/10/03 21:03 [entrez] PHST- 2022/10/04 06:00 [pubmed] PHST- 2022/10/06 06:00 [medline] AID - S1556-0864(22)00343-4 [pii] AID - 10.1016/j.jtho.2022.07.004 [doi] PST - ppublish SO - J Thorac Oncol. 2022 Oct;17(10):1233-1234. doi: 10.1016/j.jtho.2022.07.004. PMID- 21704575 OWN - NLM STAT- MEDLINE DCOM- 20111207 LR - 20220410 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 64 IP - 11 DP - 2011 Nov TI - Classification of Raynaud's disease based on angiographic features. PG - 1503-11 LID - 10.1016/j.bjps.2011.05.017 [doi] AB - Accurate diagnosis and timely management are crucial to avoid an ischaemic consequence in Raynaud's disease. There is, however, no objective classification of this disorder which guides surgical planning in refractory cases. We propose a new classification system to achieve this. From 2003 to 2009, we treated 178 patients (351 hands) who underwent surgical intervention due to an ischaemic consequence. We analysed the angiographic features of the arterial supply of the hand at three levels: (1) radial or ulnar, (2) palmar arch and common digital and (3) digital vessels. Subsequent surgical interventions were tailored according to disease types, and these included combinations of: digital sympathectomy, balloon angioplasty and end-to-end interposition venous or arterial grafting. We classified Raynaud's disease into six types: type I and II involve the radial or ulnar arteries. Type I (27.3%) showed complete occlusion, while type II (26.2%) involved partial occlusion. Type IIIa (27.1%) showed tortuous, narrowed or stenosed common digital and digital vessels. Type IIIb (1.4%) is a subset which involved the digital vessel of the index finger related to exposure to prolonged vibration. Type IV and V showed global involvement from the main to digital vessels. Type IV (13.7%) showed diffused tortuosity, narrowing and stenosis. Type V (4.3%) is the most severe, with paucity of vessels and very scant flow. Nearly half (47%) of the patients had associated systemic disease. This new classification provides objective and valuable information for decision making regarding choice of surgical procedures for the treatment of patients with Raynaud's disease which had failed conservative therapy. CI - Copyright © 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Kim, Youn Hwan AU - Kim YH AD - Department of Plastic and Reconstructive Surgery, College of Medicine, Hanyang University, 17 Haengdang-Dong, Seongdong-Gu, Seoul 133-792, South Korea. FAU - Ng, Siew-Weng AU - Ng SW FAU - Seo, Heung Seok AU - Seo HS FAU - Chang Ahn, Hee AU - Chang Ahn H LA - eng PT - Journal Article DEP - 20110624 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 RN - 0 (Contrast Media) RN - 4419T9MX03 (Iohexol) RN - 712BAC33MZ (iopromide) SB - IM CIN - J Plast Reconstr Aesthet Surg. 2012 Jul;65(7):986; author reply 986-7. doi: 10.1016/j.bjps.2011.12.041. PMID: 22333496 MH - Adult MH - Aged MH - Angiography/*methods MH - Angioplasty, Balloon MH - Contrast Media MH - Female MH - Hand/*blood supply/diagnostic imaging/surgery MH - Humans MH - Iohexol/analogs & derivatives MH - Male MH - Middle Aged MH - Raynaud Disease/*classification/*diagnostic imaging/surgery MH - Retrospective Studies MH - Sympathectomy MH - Vascular Surgical Procedures EDAT- 2011/06/28 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/06/28 06:00 PHST- 2011/01/12 00:00 [received] PHST- 2011/04/04 00:00 [revised] PHST- 2011/05/15 00:00 [accepted] PHST- 2011/06/28 06:00 [entrez] PHST- 2011/06/28 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - S1748-6815(11)00256-7 [pii] AID - 10.1016/j.bjps.2011.05.017 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2011 Nov;64(11):1503-11. doi: 10.1016/j.bjps.2011.05.017. Epub 2011 Jun 24. PMID- 32768463 OWN - NLM STAT- MEDLINE DCOM- 20201215 LR - 20201215 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 132 DP - 2020 Nov TI - Secondary Raynaud's phenomenon is associated with microvascular peripheral endothelial dysfunction. PG - 104040 LID - S0026-2862(20)30100-X [pii] LID - 10.1016/j.mvr.2020.104040 [doi] AB - Previous studies in patients with Raynaud's phenomenon (RP) have found an association between microvascular abnormalities assessed by nail fold capillaroscopy and macrovascular peripheral endothelial dysfunction (PED), but the association between RP and nitric oxide related (NO) microvascular PED is not yet established. We performed a retrospective cross-sectional analysis of patients who were referred to Mayo Clinic between 2006 and 2014 for routine cardiovascular evaluation and who underwent evaluation of Reactive Hyperemia Peripheral Arterial Tonometry (index <2 consistent with PED). Identification of the presence of RP was determined by retrospective chart review. Six hundred sixty six individuals were included in this study (mean age 51.9 ± 13.5 years, 411 (61.3%) women), 637 (95.1%) individuals did not have RP (control group), and 29 (4.3%) had secondary RP. Only 4 patients had primary RP and were thus excluded from the final analyses. In a multivariate analysis adjusting for age, sex, smoking status, and use of statins we found a significant association between secondary RP and microvascular PED in all patients (Odds ratio: 2.45; 95% confidence interval 1.13-5.34; P = 0.0236) that remained significant in women after stratifying by sex. Secondary RP is associated with microvascular PED, detected using a non-invasive NO-dependent method. Early detection of microvascular PED could help in identifying individuals with secondary RP who are at risk for developing connective tissue disease as well as CVD. CI - Copyright © 2020 Elsevier Inc. All rights reserved. FAU - Taher, Riad AU - Taher R AD - Division of Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Sara, Jaskanwal D AU - Sara JD AD - Division of Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Toya, Takumi AU - Toya T AD - Division of Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Shepherd, Roger AU - Shepherd R AD - Division of Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Moder, Kevin AU - Moder K AD - Division of Rheumatology, Department of Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Lerman, Lilach O AU - Lerman LO AD - Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Lerman, Amir AU - Lerman A AD - Division of Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. Electronic address: lerman.amir@mayo.edu. LA - eng PT - Journal Article DEP - 20200806 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 31C4KY9ESH (Nitric Oxide) SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Endothelium, Vascular/metabolism/*physiopathology MH - Female MH - Humans MH - Hyperemia/physiopathology MH - Male MH - Manometry MH - *Microcirculation MH - Microvessels/metabolism/*physiopathology MH - Middle Aged MH - Nitric Oxide/metabolism MH - Raynaud Disease/diagnosis/metabolism/*physiopathology MH - Retrospective Studies MH - Upper Extremity/*blood supply OTO - NOTNLM OT - Cardiovascular disease OT - Endothelial dysfunction OT - Novel risk factor OT - Raynaud's phenomenon OT - Vascular health COIS- Declaration of competing interest The authors have no conflicts of interest to disclose. EDAT- 2020/08/10 06:00 MHDA- 2020/12/16 06:00 CRDT- 2020/08/10 06:00 PHST- 2020/06/17 00:00 [received] PHST- 2020/07/09 00:00 [accepted] PHST- 2020/08/10 06:00 [pubmed] PHST- 2020/12/16 06:00 [medline] PHST- 2020/08/10 06:00 [entrez] AID - S0026-2862(20)30100-X [pii] AID - 10.1016/j.mvr.2020.104040 [doi] PST - ppublish SO - Microvasc Res. 2020 Nov;132:104040. doi: 10.1016/j.mvr.2020.104040. Epub 2020 Aug 6. PMID- 35944349 OWN - NLM STAT- MEDLINE DCOM- 20220908 LR - 20250728 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 56 DP - 2022 Oct TI - Treatment of resistant Raynaud's phenomenon with single-port thoracoscopic sympathicotomy: One-year follow-up. PG - 152065 LID - S0049-0172(22)00116-0 [pii] LID - 10.1016/j.semarthrit.2022.152065 [doi] AB - OBJECTIVE: Follow-up of patients with treatment-resistant Raynaud's phenomenon (RP) one-year after single-port thoracoscopic sympathicotomy (SPTS). METHODS: Eight patients (six males, two females, median age of 45 years) with treatment-resistant RP underwent left-sided SPTS at the third rib (R3), unilaterally. Questionnaires were taken, and number and duration of RP attacks were reported over a 2-week period. Perfusion was assessed with a cooling and recovery procedure at baseline and one year after SPTS. Furthermore, laser speckle contrast analysis, pulse wave velocity, heart rate variability and nailfold capillary microscopy were performed. RESULTS: One year after SPTS the duration of the attacks of was reduced with 1.9 h in the left hand versus 0.3 h in the right hand. Furthermore, three aspects of the questionnaire showed a significant improvement (role limitations due to physical health (p = 0.017), pain (p = 0.027) and physical functioning (p = 0.025)). The total area under the curve of the total cooling and recovery procedure of the left hand was larger one year after surgery (101 (75-140) at baseline versus 118 (95-190) one year post-operatively, p = 0.012), implying a better perfusion in the fingers. This was mainly due to the improvement during the recovery phase (21 (1-41) at baseline versus 38 (24-43) one year post-operatively, p = 0.028). CONCLUSION: One year after unilateral R3 SPTS the benefit with regard to the majority of outcome variables persisted, though some effects seem to attenuate. Long-term effects and long-term follow-up results will be investigated in an on-going study. CLINICAL TRIAL REGISTRATION NUMBER: NCT02680509. CI - Copyright © 2022. Published by Elsevier Inc. FAU - Kuijpers, Michiel AU - Kuijpers M AD - Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - van de Zande, Saskia C AU - van de Zande SC AD - Department of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. Electronic address: s.c.van.de.zande@umcg.nl. FAU - van Roon, Anniek M AU - van Roon AM AD - Department of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - van Roon, Arie M AU - van Roon AM AD - Department of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - Stel, Alja J AU - Stel AJ AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - Smit, Andries J AU - Smit AJ AD - Department of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - Bouma, Wobbe AU - Bouma W AD - Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - DeJongste, Mike J L AU - DeJongste MJL AD - University Medical Center Groningen and University of Groningen, Groningen, the Netherlands. FAU - Mariani, Massimo A AU - Mariani MA AD - Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - Klinkenberg, Theo J AU - Klinkenberg TJ AD - Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. FAU - Mulder, Douwe J AU - Mulder DJ AD - Department of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands. LA - eng SI - ClinicalTrials.gov/NCT02680509 PT - Journal Article DEP - 20220709 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Capillaries MH - Female MH - Fingers/blood supply MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - *Pulse Wave Analysis MH - *Raynaud Disease/drug therapy/surgery OTO - NOTNLM OT - Blood perfusion OT - Raynaud's diary OT - Raynaud's phenomenon OT - Sympathicotomy COIS- Declaration of Competing Interest The authors have declared no conflicts of interest. EDAT- 2022/08/10 06:00 MHDA- 2022/09/09 06:00 CRDT- 2022/08/09 18:18 PHST- 2022/05/02 00:00 [received] PHST- 2022/06/28 00:00 [revised] PHST- 2022/07/05 00:00 [accepted] PHST- 2022/08/10 06:00 [pubmed] PHST- 2022/09/09 06:00 [medline] PHST- 2022/08/09 18:18 [entrez] AID - S0049-0172(22)00116-0 [pii] AID - 10.1016/j.semarthrit.2022.152065 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Oct;56:152065. doi: 10.1016/j.semarthrit.2022.152065. Epub 2022 Jul 9. PMID- 35120748 OWN - NLM STAT- MEDLINE DCOM- 20220428 LR - 20220428 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 98 DP - 2022 Apr TI - Primary Raynaud's phenomenon, very early and early scleroderma tips for a timely diagnosis. PG - 111-112 LID - S0953-6205(22)00048-6 [pii] LID - 10.1016/j.ejim.2022.01.036 [doi] FAU - Sirufo, Maria Maddalena AU - Sirufo MM AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy; Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04 Teramo, Italy. FAU - Magnanimi, Lina Maria AU - Magnanimi LM AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy. FAU - Ginaldi, Lia AU - Ginaldi L AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy; Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04 Teramo, Italy. FAU - De Martinis, Massimo AU - De Martinis M AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy; Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04 Teramo, Italy. Electronic address: demartinis@cc.univaq.it. LA - eng PT - Comment PT - Letter DEP - 20220201 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 SB - IM CON - Eur J Intern Med. 2022 Mar;97:4-7. doi: 10.1016/j.ejim.2021.12.012. PMID: 34969594 MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Systemic/complications/diagnosis OTO - NOTNLM OT - Capillaroscopy OT - Connective tissue disease OT - Microcirculation OT - Microvascular abnormalities OT - NVC OT - Nailfold videocapillaroscopy OT - Raynaud's phenomenon OT - Scleroderma OT - Systemic sclerosis EDAT- 2022/02/06 06:00 MHDA- 2022/04/29 06:00 CRDT- 2022/02/05 05:36 PHST- 2022/01/17 00:00 [received] PHST- 2022/01/21 00:00 [accepted] PHST- 2022/02/06 06:00 [pubmed] PHST- 2022/04/29 06:00 [medline] PHST- 2022/02/05 05:36 [entrez] AID - S0953-6205(22)00048-6 [pii] AID - 10.1016/j.ejim.2022.01.036 [doi] PST - ppublish SO - Eur J Intern Med. 2022 Apr;98:111-112. doi: 10.1016/j.ejim.2022.01.036. Epub 2022 Feb 1. PMID- 41259640 OWN - NLM STAT- MEDLINE DCOM- 20251119 LR - 20251130 IS - 2317-6385 (Electronic) IS - 1679-4508 (Print) IS - 1679-4508 (Linking) VI - 23 DP - 2025 TI - Raynaud's phenomenon during treatment with lisdexamfetamine: risk of cerebral vasospasm? PG - eRC1439 LID - S1679-45082025000100515 [pii] LID - 10.31744/einstein_journal/2025RC1439 [doi] LID - eRC1439 AB - Lisdexamfetamine, a prodrug used to treat Attention Deficit/Hyperactivity Disorder in children, adolescents, and adults, is an inactive substance that is converted into its active form (dextroamphetamine) after being metabolized. This conversion primarily occurs in the bloodstream through enzymatic cleavage following active absorption from the gastrointestinal lumen. The active metabolite then stimulates the central nervous system by increasing the levels of dopamine and norepinephrine in the brain. It was discovered in 1996 by New River Pharmaceuticals and approved by the FDA in 2007 for the treatment of Attention Deficit/Hyperactivity Disorder in children. In this article, two cases of secondary Raynaud's phenomenon due to the use of lisdexamfetamine are described. Raynaud's disease is the primary form, occurring in the absence of an underlying cause, and differs from secondary Raynaud's phenomenon, which is associated with various medical conditions and pharmacological agents. In the cases reported in this study, the phenomenon occurred during treatment with lisdexamfetamine, where it is listed as an uncommon adverse effect in the prescribing information. In both patients presented in this report, discontinuation of the medication led to resolution of the phenomenon within a few days. This report highlights the fact that one of the patients reported episodes of dizziness during Raynaud's phenomenon, drawing attention to the potential associated complications. FAU - Pitliuk, Rubens AU - Pitliuk R AUID- ORCID: 0000-0001-6212-9983 AD - Hospital Israelita Albert Einstein, São Paulo, SP, Brazil. FAU - Fucci, Tatyanny Paula Pinto da Costa Santos AU - Fucci TPPDCS AUID- ORCID: 0000-0001-8186-8415 AD - Nutricionist, São Paulo, SP, Brazil. LA - eng PT - Case Reports PT - Journal Article DEP - 20251024 PL - Brazil TA - Einstein (Sao Paulo) JT - Einstein (Sao Paulo, Brazil) JID - 101281800 RN - 0 (Central Nervous System Stimulants) RN - TZ47U051FI (Dextroamphetamine) RN - SJT761GEGS (Lisdexamfetamine Dimesylate) SB - IM MH - Female MH - Humans MH - Attention Deficit Disorder with Hyperactivity/drug therapy MH - *Central Nervous System Stimulants/adverse effects MH - *Dextroamphetamine/adverse effects MH - *Lisdexamfetamine Dimesylate/adverse effects MH - *Raynaud Disease/chemically induced MH - Adult PMC - PMC12539819 EDAT- 2025/11/19 18:27 MHDA- 2025/11/19 18:28 PMCR- 2025/10/03 CRDT- 2025/11/19 14:33 PHST- 2024/11/25 00:00 [received] PHST- 2025/04/01 00:00 [accepted] PHST- 2025/11/19 18:28 [medline] PHST- 2025/11/19 18:27 [pubmed] PHST- 2025/11/19 14:33 [entrez] PHST- 2025/10/03 00:00 [pmc-release] AID - S1679-45082025000100515 [pii] AID - einstein_journal/2025RC1439 [pii] AID - 10.31744/einstein_journal/2025RC1439 [doi] PST - epublish SO - Einstein (Sao Paulo). 2025 Oct 24;23:eRC1439. doi: 10.31744/einstein_journal/2025RC1439. eCollection 2025. PMID- 39216492 OWN - NLM STAT- MEDLINE DCOM- 20240925 LR - 20260331 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 6 IP - 10 DP - 2024 Oct TI - Outdoor temperatures and Raynaud's phenomenon in patients with systemic sclerosis. PG - e655-e657 LID - S2665-9913(24)00243-1 [pii] LID - 10.1016/S2665-9913(24)00243-1 [doi] FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, 16132 Genoa, Italy; University of Genova, 16132 Genoa, Italy; IRCCS Ospedale Policlinico San Martino, Largo Rosanna Benzi, Genoa, Italy. Electronic address: mcutolo@unige.it. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center, Technologiepark-Zwijnaarde, Ghent, Belgium. FAU - Hysa, Elvis AU - Hysa E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, 16132 Genoa, Italy; Department of Experimental Medicine, University of Genova, 16132 Genoa, Italy; University of Genova, 16132 Genoa, Italy. LA - eng PT - Comment PT - Letter DEP - 20240828 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 SB - IM CON - Lancet Rheumatol. 2024 Oct;6(10):e684-e692. doi: 10.1016/S2665-9913(24)00189-9. PMID: 39216493 MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Systemic/complications MH - Temperature MH - Female MH - Middle Aged MH - Male COIS- We declare no competing interests. EDAT- 2024/09/01 16:20 MHDA- 2024/09/26 04:18 CRDT- 2024/08/31 18:53 PHST- 2024/08/01 00:00 [received] PHST- 2024/08/06 00:00 [accepted] PHST- 2024/09/26 04:18 [medline] PHST- 2024/09/01 16:20 [pubmed] PHST- 2024/08/31 18:53 [entrez] AID - S2665-9913(24)00243-1 [pii] AID - 10.1016/S2665-9913(24)00243-1 [doi] PST - ppublish SO - Lancet Rheumatol. 2024 Oct;6(10):e655-e657. doi: 10.1016/S2665-9913(24)00243-1. Epub 2024 Aug 28. PMID- 21115465 OWN - NLM STAT- MEDLINE DCOM- 20110923 LR - 20110118 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 50 IP - 2 DP - 2011 Feb TI - Beyond Raynaud's phenomenon hides very early systemic sclerosis: the assessment of organ involvement is always mandatory. PG - 250-1 LID - 10.1093/rheumatology/keq374 [doi] FAU - Czirják, László AU - Czirják L FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M LA - eng PT - Comment PT - Editorial DEP - 20101128 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2011 Feb;50(2):317-23. doi: 10.1093/rheumatology/keq176. PMID: 20562195 MH - Early Diagnosis MH - Humans MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis/physiopathology MH - Scleroderma, Systemic/*diagnosis/physiopathology MH - Time Factors EDAT- 2010/12/01 06:00 MHDA- 2011/09/29 06:00 CRDT- 2010/12/01 06:00 PHST- 2010/12/01 06:00 [entrez] PHST- 2010/12/01 06:00 [pubmed] PHST- 2011/09/29 06:00 [medline] AID - keq374 [pii] AID - 10.1093/rheumatology/keq374 [doi] PST - ppublish SO - Rheumatology (Oxford). 2011 Feb;50(2):250-1. doi: 10.1093/rheumatology/keq374. Epub 2010 Nov 28. PMID- 34322228 OWN - NLM STAT- MEDLINE DCOM- 20210803 LR - 20240402 IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 9 DP - 2020 TI - Impact of global warming on Raynaud's phenomenon: a modelling study. PG - 829 LID - 10.12688/f1000research.24939.1 [doi] LID - 829 AB - Background: Raynaud's phenomenon is induced by excessive vasoconstriction of the peripheral microcirculation in response to environmental factors, essentially cold, but also stress or emotions. The objective of the present study is to evaluate the impact of global warming on the worldwide prevalence and severity of Raynaud's phenomenon over the 21 (st) century. Method: We first estimated the correlation between average temperature and prevalence and severity of Raynaud's phenomenon. Then, we mapped the prevalence and the severity of Raynaud's phenomenon worldwide at Christmas 1999 using historical data and, using climate projections from the Inter-Sectoral Impact Model Intercomparison Project, we predicted the prevalence and severity of Raynaud's phenomenon at Christmas 2099 according to four greenhouse-gas emission scenarios. Results: The prevalence of Raynaud's phenomenon in the general population is expected to decrease by 0.5% per degree Celsius increase. Furthermore, patients are expected to suffer from one less attack per week for each increase of 2.5 degrees Celsius.  Conclusions: Our study shows that global warming may have a significant impact on the prevalence and the severity of Raynaud's phenomenon over the 21 (st) century. However, as expected, this will greatly depend on the level of greenhouse-gas emissions. CI - Copyright: © 2020 Khouri C et al. FAU - Khouri, Charles AU - Khouri C AUID- ORCID: 0000-0002-8427-8573 AD - Clinical pharmacology, Grenoble Alpes University Hospital, Grenoble, France. AD - Centre Regional de pharmacovigilance, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2, U1042, University Grenoble Alpes, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AD - Clinical pharmacology, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2, U1042, University Grenoble Alpes, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Clinical pharmacology, Grenoble Alpes University Hospital, Grenoble, France. AD - Centre Regional de pharmacovigilance, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2, U1042, University Grenoble Alpes, Grenoble, France. LA - eng SI - Dryad/10.5061/dryad.c670tq2 PT - Journal Article DEP - 20200730 PL - England TA - F1000Res JT - F1000Research JID - 101594320 SB - IM MH - Cold Temperature MH - *Global Warming MH - Humans MH - *Raynaud Disease/epidemiology MH - Temperature PMC - PMC8220350 OTO - NOTNLM OT - Raynaud's phenomenon OT - global warming COIS- Competing interests: CK has nothing to disclose; MR reports grants from Bioprojet, grants from Pfizer France, grants from GIRCI Rhône-Alpes-Auvergne, grants from Association des Sclérodermiques de France, outside the submitted work; JLC reports grants from BIOPROJET, grants from Pfizer France, grants from GIRCI Rhône-Alpes-Auvergne, grants from Association des Sclérodermiques de France, outside the submitted work. EDAT- 2021/07/30 06:00 MHDA- 2021/08/04 06:00 PMCR- 2020/07/30 CRDT- 2021/07/29 06:50 PHST- 2020/07/23 00:00 [accepted] PHST- 2021/07/29 06:50 [entrez] PHST- 2021/07/30 06:00 [pubmed] PHST- 2021/08/04 06:00 [medline] PHST- 2020/07/30 00:00 [pmc-release] AID - 10.12688/f1000research.24939.1 [doi] PST - epublish SO - F1000Res. 2020 Jul 30;9:829. doi: 10.12688/f1000research.24939.1. eCollection 2020. PMID- 28580711 OWN - NLM STAT- MEDLINE DCOM- 20190627 LR - 20260127 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 83 IP - 10 DP - 2017 Oct TI - Fluoxetine and Raynaud's phenomenon: friend or foe? PG - 2307-2309 LID - 10.1111/bcp.13314 [doi] FAU - Khouri, Charles AU - Khouri C AUID- ORCID: 0000-0002-8427-8573 AD - Grenoble Alps University Hospital, Pharmacovigilance Unit, F-38000, Grenoble, France. AD - Grenoble Alps University Hospital, Clinical Pharmacology Department, INSERM CIC1406, F-38000, Grenoble, France. FAU - Gailland, Thomas AU - Gailland T AD - Grenoble Alps University Hospital, Pharmacovigilance Unit, F-38000, Grenoble, France. FAU - Lepelley, Marion AU - Lepelley M AD - Grenoble Alps University Hospital, Pharmacovigilance Unit, F-38000, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AUID- ORCID: 0000-0003-4475-1626 AD - Grenoble Alps University Hospital, Clinical Pharmacology Department, INSERM CIC1406, F-38000, Grenoble, France. AD - Univ. Grenoble Alpes, UMR 1042-HP2, INSERM, F-38000, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Grenoble Alps University Hospital, Clinical Pharmacology Department, INSERM CIC1406, F-38000, Grenoble, France. AD - Univ. Grenoble Alpes, UMR 1042-HP2, INSERM, F-38000, Grenoble, France. LA - eng PT - Letter DEP - 20170604 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - 01K63SUP8D (Fluoxetine) RN - 0 (Selective Serotonin Reuptake Inhibitors) SB - IM MH - Fluoxetine/pharmacology/*therapeutic use MH - Humans MH - Raynaud Disease/*drug therapy/physiopathology MH - Selective Serotonin Reuptake Inhibitors/pharmacology/*therapeutic use MH - Treatment Outcome PMC - PMC5595930 OTO - NOTNLM OT - Raynaud's phenomenon OT - fluoxetine OT - systemic sclerosis EDAT- 2017/06/06 06:00 MHDA- 2019/06/30 06:00 PMCR- 2018/10/01 CRDT- 2017/06/06 06:00 PHST- 2017/01/11 00:00 [received] PHST- 2017/04/07 00:00 [revised] PHST- 2017/04/16 00:00 [accepted] PHST- 2017/06/06 06:00 [pubmed] PHST- 2019/06/30 06:00 [medline] PHST- 2017/06/06 06:00 [entrez] PHST- 2018/10/01 00:00 [pmc-release] AID - BCP13314 [pii] AID - 10.1111/bcp.13314 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2017 Oct;83(10):2307-2309. doi: 10.1111/bcp.13314. Epub 2017 Jun 4. PMID- 34323017 OWN - NLM STAT- MEDLINE DCOM- 20220405 LR - 20220405 IS - 1754-9485 (Electronic) IS - 1754-9477 (Linking) VI - 66 IP - 3 DP - 2022 Apr TI - Magnetic resonance characterisation of primary Raynaud's phenomenon. PG - 419-422 LID - 10.1111/1754-9485.13293 [doi] AB - The objective findings of phalangeal T2-weighted hyperintense and T1-weighted hypointense bone marrow signal on MRI without features of seronegative arthropathy or osteomyelitis may assist clinicians in making a diagnosis in the appropriate clinical context. CI - © 2021 The Royal Australian and New Zealand College of Radiologists. FAU - White, Roland Z AU - White RZ AUID- ORCID: 0000-0002-4455-609X AD - Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - School of Medicine, University of Adelaide, Adelaide, South Australia, Australia. FAU - Nguyen, Thi AU - Nguyen T AD - Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - Benson Radiology, Adelaide, South Australia, Australia. AD - Radiology Department, Flinders Medical Centre, Adelaide, South Australia, Australia. FAU - Sampson, Matthew J AU - Sampson MJ AD - Benson Radiology, Adelaide, South Australia, Australia. AD - School of Medicine, Flinders University, Adelaide, South Australia, Australia. LA - eng PT - Journal Article DEP - 20210728 PL - Australia TA - J Med Imaging Radiat Oncol JT - Journal of medical imaging and radiation oncology JID - 101469340 SB - IM MH - Extremities MH - Humans MH - Magnetic Resonance Imaging MH - Magnetic Resonance Spectroscopy MH - *Osteomyelitis MH - *Raynaud Disease/diagnostic imaging OTO - NOTNLM OT - MRI OT - Raynaud’s OT - bone marrow oedema OT - cyanosis OT - pallor EDAT- 2021/07/30 06:00 MHDA- 2022/04/06 06:00 CRDT- 2021/07/29 07:13 PHST- 2021/04/30 00:00 [received] PHST- 2021/07/11 00:00 [accepted] PHST- 2021/07/30 06:00 [pubmed] PHST- 2022/04/06 06:00 [medline] PHST- 2021/07/29 07:13 [entrez] AID - 10.1111/1754-9485.13293 [doi] PST - ppublish SO - J Med Imaging Radiat Oncol. 2022 Apr;66(3):419-422. doi: 10.1111/1754-9485.13293. Epub 2021 Jul 28. PMID- 29426682 OWN - NLM STAT- MEDLINE DCOM- 20200520 LR - 20210525 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 15 IP - 6 DP - 2019 Nov-Dec TI - Retrospective view of primary Raynaud's phenomenon in childhood. PG - e92-e95 LID - S1699-258X(17)30317-0 [pii] LID - 10.1016/j.reuma.2017.12.012 [doi] AB - OBJECTIVES: Primary Raynaud's phenomenon (PRP) manifests as episodes of transient spasms of peripheral blood vessels. To elucidate the clinical clues and laboratory characteristics will facilitate the identification of PRP. METHODS: A retrospective data collection of clinical and laboratory characteristics of 58 children with PRP was performed between January 2007 and December 2016. RESULTS: A positive ANA test at lower titers <1:100 was detected in 24.1% of the patients. There was a significant relationship between presence of ANA positivity and migraine in female patients with PRP (p=0.01; p=0.020 respectively). The most common accompanying disorder was migraine which was detected in 37.9% of all patients with PRP. Hemoglobin and serum ferritin levels were significantly lower in PRP patients with migraine (p=0.045; p<0.05, respectively). Additionally, the mean platelet volume (MPV) measurements were significantly higher in patients with migraine compared to those without migraine (p=0.045; p<0.05 respectively). DISCUSSION: There is limited data concerning childhood PRP. For the first time we showed a high frequency of migraine in childhood PRP. Anemia and high MPV could be the underlying triggering factors of these two episodic diseases. CI - Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Turan, Enes AU - Turan E AD - Uludag University Medical Faculty, Department of Pediatrics, Gorukle, 16059 Bursa, Turkey. FAU - Kilic, Sara Sebnem AU - Kilic SS AD - Uludag University Medical Faculty, Department of Pediatric Immunology-Rheumatology, Gorukle, 16059 Bursa, Turkey. Electronic address: sebnemkl@uludag.edu.tr. LA - eng LA - spa PT - Journal Article DEP - 20180206 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Adolescent MH - Child MH - Female MH - Humans MH - Male MH - Raynaud Disease/complications/*diagnosis MH - Retrospective Studies MH - Young Adult OTO - NOTNLM OT - Anemia OT - Fenómeno de Raynaud OT - Migraine OT - Migraña OT - Raynaud's phenomenon EDAT- 2018/02/11 06:00 MHDA- 2020/05/21 06:00 CRDT- 2018/02/11 06:00 PHST- 2017/03/14 00:00 [received] PHST- 2017/12/09 00:00 [revised] PHST- 2017/12/21 00:00 [accepted] PHST- 2018/02/11 06:00 [pubmed] PHST- 2020/05/21 06:00 [medline] PHST- 2018/02/11 06:00 [entrez] AID - S1699-258X(17)30317-0 [pii] AID - 10.1016/j.reuma.2017.12.012 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2019 Nov-Dec;15(6):e92-e95. doi: 10.1016/j.reuma.2017.12.012. Epub 2018 Feb 6. PMID- 38282510 OWN - NLM STAT- MEDLINE DCOM- 20240711 LR - 20240711 IS - 1469-1833 (Electronic) IS - 1352-4658 (Linking) VI - 52 IP - 4 DP - 2024 Jul TI - Psychological factors in symptom severity and quality of life in Raynaud's phenomenon. PG - 426-439 LID - 10.1017/S1352465823000620 [doi] AB - BACKGROUND: Despite emotional stress being recognised as a key trigger for Raynaud's phenomenon episodes, research in the area is still in its infancy. AIMS: This study investigated the role of psychological factors relating to symptom severity and quality of life, and differences between Raynaud's types (primary and secondary) to further inform the development of intervention in this field. METHOD: A cross-sectional design was used. Two hundred and ten adults with Raynaud's completed an online questionnaire measuring stress, anxiety, depression, anxiety sensitivity, beliefs about emotions, symptom severity and quality of life. RESULTS: Primary and secondary Raynaud's groups differed in anxiety (p < .004), symptom severity (p < .001) and quality of life (p < .001). Stepwise multiple regressions indicated anxiety and Raynaud's type explained 23% variance in hand symptom severity (p < .001); anxiety, Raynaud's type and anxiety sensitivity explained 29% variance in symptom severity (global impact, p < .001); depression, Raynaud's type and anxiety sensitivity explained 32% variance in quality of life (p < .001). CONCLUSIONS: Results highlight the importance of psychological factors in Raynaud's phenomenon, indicating possible targets for treatment. Interventions such as cognitive behavioural therapy, which target both physical and psychological wellbeing, bear some promise as an adjuvant therapy for this group. FAU - Irving, Dulcie AU - Irving D AUID- ORCID: 0000-0002-6005-8648 AD - Department of Psychology, University of Bath, Bath, UK. FAU - Daniels, Jo AU - Daniels J AUID- ORCID: 0000-0003-3067-9416 AD - Department of Psychology, University of Bath, Bath, UK. LA - eng PT - Journal Article DEP - 20240129 PL - United States TA - Behav Cogn Psychother JT - Behavioural and cognitive psychotherapy JID - 9418292 SB - IM MH - Humans MH - *Quality of Life/psychology MH - *Raynaud Disease/psychology/therapy MH - Female MH - Male MH - Cross-Sectional Studies MH - Adult MH - Middle Aged MH - Surveys and Questionnaires MH - *Severity of Illness Index MH - *Anxiety/psychology MH - *Depression/psychology MH - Stress, Psychological/psychology/complications OTO - NOTNLM OT - Anxiety OT - Beliefs OT - Depression OT - Quality of life OT - Raynaud’s phenomenon OT - Symptoms EDAT- 2024/01/29 06:44 MHDA- 2024/07/11 12:42 CRDT- 2024/01/29 03:30 PHST- 2024/07/11 12:42 [medline] PHST- 2024/01/29 06:44 [pubmed] PHST- 2024/01/29 03:30 [entrez] AID - S1352465823000620 [pii] AID - 10.1017/S1352465823000620 [doi] PST - ppublish SO - Behav Cogn Psychother. 2024 Jul;52(4):426-439. doi: 10.1017/S1352465823000620. Epub 2024 Jan 29. PMID- 30207278 OWN - NLM STAT- MEDLINE DCOM- 20190116 LR - 20220413 IS - 1879-291X (Electronic) IS - 0301-5629 (Print) IS - 0301-5629 (Linking) VI - 44 IP - 10 DP - 2018 Oct TI - Photoacoustic Oxygenation Quantification in Patients with Raynaud's: First-in-Human Results. PG - 2081-2088 LID - S0301-5629(18)30179-0 [pii] LID - 10.1016/j.ultrasmedbio.2018.04.017 [doi] AB - The purpose of this study was to investigate the use of photoacoustic imaging for quantifying fingertip oxygenation as an approach to diagnosing and monitoring Raynaud's phenomenon. After 30 min of acclimation to room temperature, 22 patients (7 patients with secondary Raynaud's associated to Scleroderma and 15 healthy controls) provided informed consent to undergo fingertip Doppler imaging and high-frequency photoacoustic imaging before and 5, 15 and 30 min after cold stimulus (submerged hand in a 15 °C water bath for 1 min). High-frequency ultrasound and photoacoustic imaging was performed on the nail bed of each patient's second through fifth finger on their dominant hand, using a Vevo 2100 LAZR system with an LZ-250 probe (Fujifilm VisualSonics, Toronto, ON, Canada) in oxy-hemoglobin quantification mode. During each exam, volumetric data across a 3-mm span of data was acquired to produce a volumetric image of percent oxygenation and hemoglobin concentration. Changes in fingertip oxygenation between Raynaud's patients and healthy volunteers were compared, using receiver operator characteristic (ROC) analysis. Photoacoustic signal was detected in both the nail bed and nailfold in all study participants. Doppler ultrasound resulted in poor differentiation of Raynaud's patients from healthy volunteers, with an area under the ROC curve (A(z)) of 0.51. Photoacoustic imaging demonstrated improved accuracy at baseline (A(z) = 0.72), which improved when quantifying normalized changes after cold stimulus (A(z) = 0.89 5-min post stimulus, A(z) = 0.91 15-min post stimulus, and A(z) = 0.85 after stimulus). Oxygenation levels derived using photoacoustic imaging are able to identify patients with Raynaud's and safely evaluate their response to a cold stimulus over time. CI - Copyright © 2018. Published by Elsevier Inc. FAU - Eisenbrey, John R AU - Eisenbrey JR AD - Department of Radiology, Thomas Jefferson University, Philadelphia, PA, USA. Electronic address: John.Eisenbrey@jefferson.edu. FAU - Stanczak, Maria AU - Stanczak M AD - Department of Radiology, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Forsberg, Flemming AU - Forsberg F AD - Department of Radiology, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Mendoza-Ballesteros, Fabian A AU - Mendoza-Ballesteros FA AD - Department of Medicine, Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA, USA; Scleroderma Center and Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, PA. FAU - Lyshchik, Andrej AU - Lyshchik A AD - Department of Radiology, Thomas Jefferson University, Philadelphia, PA, USA. LA - eng GR - S10 OD010408/OD/NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20180706 PL - England TA - Ultrasound Med Biol JT - Ultrasound in medicine & biology JID - 0410553 RN - S88TT14065 (Oxygen) SB - IM MH - Adult MH - Cold Temperature MH - Female MH - Fingers/blood supply/diagnostic imaging MH - Humans MH - Male MH - Middle Aged MH - Oxygen/*metabolism MH - Photoacoustic Techniques/*methods MH - Raynaud Disease/*diagnostic imaging/metabolism MH - Regional Blood Flow MH - Ultrasonography, Doppler/*methods PMC - PMC8994565 MID - NIHMS1784929 OTO - NOTNLM OT - Photoacoustic imaging OT - Raynaud's OT - Tissue oxygenation EDAT- 2018/09/13 06:00 MHDA- 2019/01/17 06:00 PMCR- 2022/04/09 CRDT- 2018/09/13 06:00 PHST- 2017/12/07 00:00 [received] PHST- 2018/04/16 00:00 [revised] PHST- 2018/04/23 00:00 [accepted] PHST- 2018/09/13 06:00 [entrez] PHST- 2018/09/13 06:00 [pubmed] PHST- 2019/01/17 06:00 [medline] PHST- 2022/04/09 00:00 [pmc-release] AID - S0301-5629(18)30179-0 [pii] AID - 10.1016/j.ultrasmedbio.2018.04.017 [doi] PST - ppublish SO - Ultrasound Med Biol. 2018 Oct;44(10):2081-2088. doi: 10.1016/j.ultrasmedbio.2018.04.017. Epub 2018 Jul 6. PMID- 35723612 OWN - NLM STAT- MEDLINE DCOM- 20221220 LR - 20230104 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 52 IP - 1 DP - 2023 Jan TI - Paradoxical Raynaud's phenomenon following iloprost infusion in a patient with systemic sclerosis. PG - 91-92 LID - 10.1080/03009742.2022.2082054 [doi] FAU - Bertelle, D AU - Bertelle D AUID- ORCID: 0000-0002-5901-7742 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Bertoldo, E AU - Bertoldo E AUID- ORCID: 0000-0001-5678-3161 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Bixio, R AU - Bixio R AUID- ORCID: 0000-0001-9335-3371 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Rossini, M AU - Rossini M AUID- ORCID: 0000-0001-9692-2293 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. LA - eng PT - Journal Article DEP - 20220620 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - JED5K35YGL (Iloprost) SB - IM MH - Humans MH - Iloprost/adverse effects MH - *Raynaud Disease/chemically induced/drug therapy MH - *Scleroderma, Systemic/complications/drug therapy EDAT- 2022/06/21 06:00 MHDA- 2022/12/21 06:00 CRDT- 2022/06/20 10:23 PHST- 2022/06/21 06:00 [pubmed] PHST- 2022/12/21 06:00 [medline] PHST- 2022/06/20 10:23 [entrez] AID - 10.1080/03009742.2022.2082054 [doi] PST - ppublish SO - Scand J Rheumatol. 2023 Jan;52(1):91-92. doi: 10.1080/03009742.2022.2082054. Epub 2022 Jun 20. PMID- 39623044 OWN - NLM STAT- MEDLINE DCOM- 20241203 LR - 20241205 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 14 IP - 1 DP - 2024 Dec 2 TI - Radial cortex transverse distraction help to treat the Raynaud's phenomenon. PG - 29984 LID - 10.1038/s41598-024-81612-7 [doi] LID - 29984 AB - Raynaud's phenomenon is a condition that causing vasospasm of the digital extremities and have a great influence on the quality of patients life. Distraction osteogenesis is accompanied with vascularization and regeneration of surrounding soft tissues. There have been no previous reports of using radial cortex transverse distraction for the treatment of RP. Since January 2019 to January 2023, 6 patients with RP accepted radial cortex transverse distraction. They were failed to conventional treatment for at least six months before the surgery. All the patients were followed-up over 1 year. The bone cortex healed without osteomyelitis or fractures. The ulcer of finger healed in one month. The pain and skin pale released after the surgery. The VAS score declined from 6 to 2 and the finger SpO(2) improved. Radial cortex transverse distraction facilitated finger ulcer healing, salvage the hand and improved Raynaud symptoms. These findings suggest that radial cortex transverse distraction may be an effective procedure to treat the Raynaud's phenomenon.Level of Evidence II Therapeutic study. CI - © 2024. The Author(s). FAU - Ma, Zhihu AU - Ma Z AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. FAU - Qiao, Li AU - Qiao L AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. FAU - Cui, Yidong AU - Cui Y AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. AD - Department of Orthopedic Surgery, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, China. FAU - Zhu, Ruowen AU - Zhu R AD - Shandong First Medical University Taian Compus, Taian, China. FAU - Zhang, Xingsheng AU - Zhang X AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. AD - Department of Orthopedic Surgery, The Eighth People's Hospital of Jinan, Jinan, China. FAU - Zhu, Lei AU - Zhu L AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. zhuleijn@aliyun.com. FAU - Xu, Qingjia AU - Xu Q AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. FAU - Pei, Yantao AU - Pei Y AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. FAU - Wang, Gang AU - Wang G AD - Department of Hand Surgery, Department of Orthopedics, Qilu Hospital of Shandong University, #107 Wenhua Xilu, Jinan, China. LA - eng GR - ZR2023MH368/Natural Science foundation of Shandong Province/ PT - Journal Article DEP - 20241202 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Humans MH - *Raynaud Disease/surgery/therapy MH - Female MH - Male MH - Adult MH - Osteogenesis, Distraction/methods MH - Fingers/surgery MH - Middle Aged MH - Treatment Outcome MH - Young Adult MH - Radius/surgery PMC - PMC11612199 OTO - NOTNLM OT - Osteotomy OT - Radial cortex transverse distraction OT - Raynaud’s phenomenon OT - Vasospasm COIS- Declarations. Competing interests: The authors declare no competing interests. Ethical approval: This study was approved by the Ethics Committee of Qilu Hospital of Shandong University. All procedures performed in this study involving human participants followed the relevant guidelines and regulations of the Declaration of Helsinki. Informed consent: All patients in our study were anonymous. Informed consent was obtained from all individual participants included in the study. EDAT- 2024/12/03 00:22 MHDA- 2024/12/03 06:24 PMCR- 2024/12/02 CRDT- 2024/12/02 23:36 PHST- 2024/03/31 00:00 [received] PHST- 2024/11/14 00:00 [accepted] PHST- 2024/12/03 06:24 [medline] PHST- 2024/12/03 00:22 [pubmed] PHST- 2024/12/02 23:36 [entrez] PHST- 2024/12/02 00:00 [pmc-release] AID - 10.1038/s41598-024-81612-7 [pii] AID - 81612 [pii] AID - 10.1038/s41598-024-81612-7 [doi] PST - epublish SO - Sci Rep. 2024 Dec 2;14(1):29984. doi: 10.1038/s41598-024-81612-7. PMID- 35227144 OWN - NLM STAT- MEDLINE DCOM- 20220401 LR - 20220519 IS - 1473-4877 (Electronic) IS - 0300-7995 (Linking) VI - 38 IP - 4 DP - 2022 Apr TI - The shadow zone of capillaroscopy in the classification of the Raynaud's phenomenon. PG - 637-639 LID - 10.1080/03007995.2022.2047538 [doi] AB - Raynaud's phenomenon (RP) is a frequent clinical finding in the general population that can be observed across multiple medical specialties and nailfold videocapillaroscopy is a first line imaging tool to early differenziate primitive and secondary forms of RP. According to the criteria of LeRoy and Medsger normal nailfold capillaries characterize a primary RP. The recognition of vascular alterations at capillaroscopy is the key to a correct framing of the phenomenon. Capillaroscopy is of significant practical value as it allows a reliable predictive assessment of the developmental risk of the disorder. However, to date, the variety of nomenclature for subjects affected by RP generates uncertainty in patient management and in the possibility of comparing studies. The capillaroscopic findings have a very broad range of normality and a significant presence of non specific microvascular abnormalities are reported also in patients with primary RP. The presence of these non specific vascular changes can make it difficult to differenziate primary and secondary RP. Here we highlight some critical points in the capillaroscopic distinction of primary and secondary RP and relaunch the debate on the classification of the RP because it is likely that what we identify today as the primary RP (pRP) collects different clinical entities with different prognostic significance and different therapeutic needs. FAU - Sirufo, Maria Maddalena AU - Sirufo MM AUID- ORCID: 0000-0003-1006-7121 AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the diagnosis and treatment of Osteoporosis, AUSL 04, Teramo, Italy. FAU - Magnanimi, Lina Maria AU - Magnanimi LM AUID- ORCID: 0000-0003-2056-9483 AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy. FAU - Ginaldi, Lia AU - Ginaldi L AUID- ORCID: 0000-0003-1841-2807 AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the diagnosis and treatment of Osteoporosis, AUSL 04, Teramo, Italy. FAU - De Martinis, Massimo AU - De Martinis M AUID- ORCID: 0000-0003-4253-1312 AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the diagnosis and treatment of Osteoporosis, AUSL 04, Teramo, Italy. LA - eng PT - Journal Article DEP - 20220308 PL - England TA - Curr Med Res Opin JT - Current medical research and opinion JID - 0351014 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Prognosis MH - *Raynaud Disease/diagnostic imaging OTO - NOTNLM OT - Capillaroscopy OT - NVC OT - Raynaud’s phenomenon OT - connective tissue disease OT - microcirculation OT - microvascular abnormalities EDAT- 2022/03/02 06:00 MHDA- 2022/04/02 06:00 CRDT- 2022/03/01 05:42 PHST- 2022/03/02 06:00 [pubmed] PHST- 2022/04/02 06:00 [medline] PHST- 2022/03/01 05:42 [entrez] AID - 10.1080/03007995.2022.2047538 [doi] PST - ppublish SO - Curr Med Res Opin. 2022 Apr;38(4):637-639. doi: 10.1080/03007995.2022.2047538. Epub 2022 Mar 8. PMID- 30133174 OWN - NLM STAT- MEDLINE DCOM- 20200228 LR - 20200228 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 71 IP - 8 DP - 2019 Aug TI - Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient-Reported Assessments of Disease Severity. PG - 1119-1126 LID - 10.1002/acr.23729 [doi] AB - OBJECTIVE: Assessment of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) is reliant on self-report. The Raynaud's Condition Score (RCS) diary assumes discrete episodic RP attacks, although not all SSc patients identify with this paradigm. We investigated the clinical associations of SSc-RP symptom characteristics and the evolution of SSc-RP symptoms with disease progression. METHODS: A cross-sectional study at UK and US sites captured digital color changes of SSc-RP and patients' ability to identify with diagrammatic representations (and descriptive stems) of 4 distinct theoretical SSc-RP patterns (progressing severity A through D) reflecting progressively severe SSc-RP experiences. SSc-RP self-management and symptom evolution were explored. Patient demographics, the clinical phenotype, the Scleroderma Health Assessment Questionnaire (SHAQ), the 2-week RCS diary, and patient and physician global assessments were collected. RESULTS: We enrolled 107 patients with SSc (with questionnaires returned by 94). A higher number of self-reported digital color changes of SSc-RP were associated with increased SSc-RP symptom severity but not with the SSc clinical phenotype. Patients could identify with distinct patterns of SSc-RP. These patterns were associated with disease duration, global disease severity, and conceptually linked physician and patient assessments of peripheral vascular severity (e.g., SHAQ RP subscale and RCS diary parameters), but not with conceptually unrelated outcomes (e.g., SHAQ breathing subscale). SSc-RP characteristics and symptom severity evolve during the disease course. CONCLUSION: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischemia in patients with advanced SSc could influence diary-based approaches to assessing SSc-RP, with implications for future clinical trials. CI - © 2018, American College of Rheumatology. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, and University of Bath, Bath, UK. FAU - Reilly, Elizabeth AU - Reilly E AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, and University of Bath, Bath, UK. FAU - Smith, Theresa AU - Smith T AD - University of Bath, Bath, UK. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Salt Lake Regional Veterans Affairs Medical Center and University of Utah, Salt Lake City. LA - eng PT - Journal Article DEP - 20190703 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Disease Progression MH - Female MH - Humans MH - Male MH - Middle Aged MH - Patient Reported Outcome Measures MH - Raynaud Disease/*diagnosis/*etiology MH - Scleroderma, Systemic/*complications MH - Self Report MH - *Severity of Illness Index MH - Surveys and Questionnaires EDAT- 2018/08/23 06:00 MHDA- 2020/02/29 06:00 CRDT- 2018/08/23 06:00 PHST- 2018/04/18 00:00 [received] PHST- 2018/08/14 00:00 [accepted] PHST- 2018/08/23 06:00 [pubmed] PHST- 2020/02/29 06:00 [medline] PHST- 2018/08/23 06:00 [entrez] AID - 10.1002/acr.23729 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2019 Aug;71(8):1119-1126. doi: 10.1002/acr.23729. Epub 2019 Jul 3. PMID- 31969418 OWN - NLM STAT- MEDLINE DCOM- 20200916 LR - 20220122 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 13 IP - 1 DP - 2020 Jan 21 TI - Raynaud's phenomenon in paediatric age. LID - 10.1136/bcr-2019-233596 [doi] LID - e233596 FAU - Esteireiro, Ana Sofia AU - Esteireiro AS AUID- ORCID: 0000-0003-1603-0602 AD - Paediatrics Department, Hospital de Caldas da Rainha, Centro Hospitalar do Oeste, Caldas da Rainha, Portugal esteireira@gmail.com. FAU - Bicho, Anabela AU - Bicho A AD - Paediatrics Department, Hospital de Caldas da Rainha, Centro Hospitalar do Oeste, Caldas da Rainha, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20200121 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*analysis MH - Child MH - Female MH - Humans MH - Raynaud Disease/*diagnosis PMC - PMC7035820 OTO - NOTNLM OT - connective tissue disease OT - paediatrics OT - rheumatology OT - systemic lupus erythematosus COIS- Competing interests: None declared. EDAT- 2020/01/24 06:00 MHDA- 2020/09/17 06:00 PMCR- 2022/01/21 CRDT- 2020/01/24 06:00 PHST- 2020/01/24 06:00 [entrez] PHST- 2020/01/24 06:00 [pubmed] PHST- 2020/09/17 06:00 [medline] PHST- 2022/01/21 00:00 [pmc-release] AID - 13/1/e233596 [pii] AID - bcr-2019-233596 [pii] AID - 10.1136/bcr-2019-233596 [doi] PST - epublish SO - BMJ Case Rep. 2020 Jan 21;13(1):e233596. doi: 10.1136/bcr-2019-233596. PMID- 19053879 OWN - NLM STAT- MEDLINE DCOM- 20090720 LR - 20190917 IS - 1744-7658 (Electronic) IS - 1354-3784 (Linking) VI - 18 IP - 1 DP - 2009 Jan TI - Phosphodiesterase-5 inhibitors for the treatment of Raynaud's: a novel indication. PG - 23-9 LID - 10.1517/13543780802525100 [doi] AB - BACKGROUND: Raynaud's phenomenon is a common clinical condition characterized by significant morbidity, especially in patients who have an underlying connective tissue disorder. These patients suffer from a worse clinical phenotype that at times can lead to digital vessel occlusion. Current therapies are not optimal, and better therapeutic options are needed. OBJECTIVE: To describe the up-to-date data available on the use of phosphodiesterase 5 inhibitors in the treatment of Raynaud's phenomenon. METHODS: We performed a literature search of PubMed from 1990 - 2008, as well as a search of the abstracts presented at the American College of Rheumatology scientific meeting and the European League Against Rheumatism scientific meeting for the years 2001 - 2007. The search terms used were 'Raynaud's', 'phosphodiesterase 5 inhibitors', ' tadalafil', 'sildenafil' and 'vardenafil'. RESULTS/CONCLUSION: Based on current data from small clinical trials, open-label pilot studies and case series and reports, phosphodiesterase 5 inhibitors may help some patients with very serious Raynaud's. A large, well-conducted multicenter, double-blind study is needed to determine the benefit and risk of these agents in Raynaud's phenomenon. FAU - De LaVega, Alfonso J AU - De LaVega AJ AD - Gregorio Marañon Hospital, Universidad Complutense, Madrid Spain. FAU - Derk, Chris T AU - Derk CT LA - eng PT - Journal Article PT - Review PL - England TA - Expert Opin Investig Drugs JT - Expert opinion on investigational drugs JID - 9434197 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) SB - IM MH - Clinical Trials as Topic MH - Cyclic Nucleotide Phosphodiesterases, Type 5/metabolism MH - Humans MH - *Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/*therapeutic use MH - Raynaud Disease/*drug therapy/*enzymology/physiopathology RF - 46 EDAT- 2008/12/05 09:00 MHDA- 2009/07/21 09:00 CRDT- 2008/12/05 09:00 PHST- 2008/12/05 09:00 [pubmed] PHST- 2009/07/21 09:00 [medline] PHST- 2008/12/05 09:00 [entrez] AID - 10.1517/13543780802525100 [doi] PST - ppublish SO - Expert Opin Investig Drugs. 2009 Jan;18(1):23-9. doi: 10.1517/13543780802525100. PMID- 32282567 OWN - NLM STAT- MEDLINE DCOM- 20211124 LR - 20211124 IS - 2327-6924 (Electronic) IS - 2327-6886 (Linking) VI - 33 IP - 4 DP - 2020 Apr 8 TI - Raynaud's phenomenon of the nipple: Ensuring timely diagnosis. PG - 271-277 LID - 10.1097/JXX.0000000000000407 [doi] AB - Breast milk is the perfect first food for infants, yet many women face challenges that prematurely discontinue their breastfeeding. Nipple pain is the second most common reason women stop breastfeeding. As primary care providers, nurse practitioners (NPs) are in the ideal position to support women both prenatally and postpartum. To help these patients, the NP must recognize conditions affecting nursing mothers early, as well as provide interventions and referrals in a timely fashion. A common but frequently overlooked condition is Raynaud's phenomenon of the nipple (RPN), a vasospastic disorder that causes moderate to severe nipple pain and is often misdiagnosed. The purpose of this article is to address a gap in the literature regarding this phenomenon, prevent missed diagnoses of RPN, and to promote higher rates of successful breastfeeding. CI - Copyright © 2020 American Association of Nurse Practitioners. FAU - Pereira, Marina AU - Pereira M AD - Rady Faculty of Health Sciences, College of Nursing, University of Manitoba, Winnipeg, Manitoba, Canada. FAU - Thable, Angela AU - Thable A LA - eng PT - Journal Article DEP - 20200408 PL - United States TA - J Am Assoc Nurse Pract JT - Journal of the American Association of Nurse Practitioners JID - 101600770 MH - Female MH - Humans MH - Mothers MH - Nipples MH - *Nurse Practitioners MH - Pain MH - *Raynaud Disease/diagnosis COIS- Competing interests: The authors report no conflicts of interest. EDAT- 2020/04/14 06:00 MHDA- 2021/11/25 06:00 CRDT- 2020/04/14 06:00 PHST- 2019/09/24 00:00 [received] PHST- 2020/01/28 00:00 [accepted] PHST- 2020/04/14 06:00 [pubmed] PHST- 2021/11/25 06:00 [medline] PHST- 2020/04/14 06:00 [entrez] AID - 01741002-202104000-00004 [pii] AID - 10.1097/JXX.0000000000000407 [doi] PST - epublish SO - J Am Assoc Nurse Pract. 2020 Apr 8;33(4):271-277. doi: 10.1097/JXX.0000000000000407. PMID- 36343456 OWN - NLM STAT- MEDLINE DCOM- 20221122 LR - 20221129 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 57 DP - 2022 Dec TI - Raynaud's phenomenon secondary to central nervous system stimulants: A therapeutic role of α-1 adrenergic blockers? PG - 152115 LID - S0049-0172(22)00166-4 [pii] LID - 10.1016/j.semarthrit.2022.152115 [doi] FAU - Upadhya, Shubhra AU - Upadhya S AD - Department of Internal Medicine, Division of General Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, NM 87106, United States. FAU - Nunez, Sharon E AU - Nunez SE AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, MSC 10 5550, 5th FL ACC, Albuquerque, NM 87106, United States. FAU - Muruganandam, Maheswari AU - Muruganandam M AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, MSC 10 5550, 5th FL ACC, Albuquerque, NM 87106, United States. FAU - McElwee, Matthew K AU - McElwee MK AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, MSC 10 5550, 5th FL ACC, Albuquerque, NM 87106, United States. FAU - Emil, N Suzanne AU - Emil NS AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, MSC 10 5550, 5th FL ACC, Albuquerque, NM 87106, United States. FAU - Sibbitt, Wilmer L Jr AU - Sibbitt WL Jr AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, MSC 10 5550, 5th FL ACC, Albuquerque, NM 87106, United States. Electronic address: wsibbitt@salud.unm.edu. LA - eng PT - Comment PT - Letter DEP - 20221026 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Central Nervous System Stimulants) RN - 0 (Adrenergic Antagonists) SB - IM CON - Semin Arthritis Rheum. 2021 Dec;51(6):1200-1204. doi: 10.1016/j.semarthrit.2021.09.002. PMID: 34655948 CIN - Semin Arthritis Rheum. 2022 Dec;57:152114. doi: 10.1016/j.semarthrit.2022.152114. PMID: 36356481 MH - Humans MH - *Central Nervous System Stimulants MH - *Raynaud Disease/drug therapy/etiology MH - Adrenergic Antagonists COIS- Declaration of Competing Interest None of the authors declare a competing interest. EDAT- 2022/11/08 06:00 MHDA- 2022/11/23 06:00 CRDT- 2022/11/07 18:18 PHST- 2022/09/08 00:00 [received] PHST- 2022/10/20 00:00 [accepted] PHST- 2022/11/08 06:00 [pubmed] PHST- 2022/11/23 06:00 [medline] PHST- 2022/11/07 18:18 [entrez] AID - S0049-0172(22)00166-4 [pii] AID - 10.1016/j.semarthrit.2022.152115 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Dec;57:152115. doi: 10.1016/j.semarthrit.2022.152115. Epub 2022 Oct 26. PMID- 18463765 OWN - NLM STAT- MEDLINE DCOM- 20090115 LR - 20181201 IS - 1972-6007 (Electronic) IS - 0009-9074 (Linking) VI - 159 IP - 2 DP - 2008 Mar-Apr TI - Oxidant/antioxidant status in patients with Raynaud's disease. PG - 77-81 AB - OBJECTIVE: Despite several studies concerning the oxidative stress in Raynaud's phenomenon secondary to systemic sclerosis (secondary Raynaud, SR), little is known regarding the relationship between Raynaud's disease (primary Raynaud, PR) and oxidative stress at present. The aim of the present research was to study the parameters of oxidative stress in PR patients and to compare them to those of SR patients. MATERIALS AND METHODS: Serum hydroperoxide (Hydrop) level, serum total antioxidant capacity (TAC) and serum total thiol (-SHp) barrier were measured in 19 patients with SR, 15 patients with PR and 14 healthy control subjects (CS). The statistical contrasts were performed via one-way ANOVA. RESULTS: The serum Hydrop concentrations were significantly higher in SR (p = 0.0043) and PR (p = 0.0038) groups in comparison with CS. A significant decrease in serum TAC level in SR (p = 0.00005) and PR (p = 0.00128) patients in respect to the CS was revealed. No significant change in the -SHp barrier in SR and PR patients in respect to CS has been demonstrated. However, there were no significant differences in serum Hydrop and TAC levels between the groups of patients. CONCLUSIONS: The study of serum oxidative stress parameters, in terms of Hydro and TAC levels may be used as a routine and rapid test to verify the oxidative stress status in SR and PR patients and to undertake a strategy of treatments by a supplementation of antioxidant molecules. FAU - Biondi, R AU - Biondi R AD - Department of Internal Medicine and Dermatology Unit, Perugia University School of Medicine, S. Maria General Hospital, Terni, Italy. FAU - Coaccioli, S AU - Coaccioli S FAU - Lattanzi, S AU - Lattanzi S FAU - Puxeddu, A AU - Puxeddu A FAU - Papini, M AU - Papini M LA - eng PT - Journal Article PL - Italy TA - Clin Ter JT - La Clinica terapeutica JID - 0372604 RN - 0 (Antioxidants) RN - 0 (Multienzyme Complexes) RN - 0 (Oxidants) RN - 0 (Sulfhydryl Compounds) RN - 11062-77-4 (Superoxides) RN - BBX060AN9V (Hydrogen Peroxide) RN - EC 1.6.- (NADH oxidase) RN - EC 1.6.- (NADH, NADPH Oxidoreductases) SB - IM MH - Adult MH - Antioxidants/*analysis MH - Cells, Cultured/drug effects/metabolism MH - Female MH - Fibroblasts/metabolism MH - Humans MH - Hydrogen Peroxide/*blood/metabolism MH - Male MH - Middle Aged MH - Multienzyme Complexes/metabolism MH - NADH, NADPH Oxidoreductases/metabolism MH - Oxidants/*blood MH - Oxidative Stress MH - Raynaud Disease/*blood/etiology MH - Scleroderma, Localized/blood/complications MH - Scleroderma, Systemic/blood/complications MH - Sulfhydryl Compounds/*blood MH - Superoxides/metabolism EDAT- 2008/05/09 09:00 MHDA- 2009/01/16 09:00 CRDT- 2008/05/09 09:00 PHST- 2008/05/09 09:00 [pubmed] PHST- 2009/01/16 09:00 [medline] PHST- 2008/05/09 09:00 [entrez] PST - ppublish SO - Clin Ter. 2008 Mar-Apr;159(2):77-81. PMID- 16732585 OWN - NLM STAT- MEDLINE DCOM- 20060714 LR - 20080118 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 54 IP - 6 DP - 2006 Jun TI - Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: results of ten years of prospective surveillance. PG - 1974-81 AB - OBJECTIVE: To assess the early signs, risk factors, and rate of transition from primary Raynaud's phenomenon (primary RP) to secondary RP. METHODS: A clinical sample of 307 consecutive patients with RP was included in a prospective followup study. After an initial screening, 244 patients were classified as having primary RP, of whom 236 were followed up for a mean +/- SD of 11.2 +/- 3.9 years. Patients classified according to the screening as having suspected secondary RP underwent an extended screening program annually until transition to secondary RP occurred. RESULTS: The initial prevalence of secondary RP was 11%. The annual incidence of transition to suspected secondary RP was 2%, and the annual incidence of transition to secondary RP was 1%. Overall, 46 patients were classified as having suspected secondary RP, and 23 of these later were classified as having secondary RP. Older age at onset of RP (hazard ratio 2.59, 95% confidence interval [95% CI] 1.40-4.80), shorter duration of RP at enrollment (hazard ratio 0.87, 95% CI 0.81-0.94), and abnormal findings on thoracic outlet test (hazard ratio 2.69, 95% CI 1.12-6.48) were associated with an increased risk for transition to secondary RP. Compared with patients with suspected secondary RP, those diagnosed as having secondary RP had a higher number and earlier occurrence of pathologic findings. Furthermore, antinuclear antibodies at a titer of > or = 1:320 and positive findings in specific serologic subsets were associated with a significantly increased risk for developing a connective tissue disease. CONCLUSION: Patients diagnosed initially as having primary RP may actually comprise 1 of 3 groups: those with idiopathic RP, those with a rather benign disease course, and those with a more severe course of the disease. FAU - Hirschl, Mirko AU - Hirschl M AD - Hanuschkrankenhaus, Vienna, Austria. Mirko.Hirschl@wgkk.sozvers.at FAU - Hirschl, Katharina AU - Hirschl K FAU - Lenz, Matthias AU - Lenz M FAU - Katzenschlager, Reinhold AU - Katzenschlager R FAU - Hutter, Hans-Peter AU - Hutter HP FAU - Kundi, Michael AU - Kundi M LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antibodies, Antinuclear) SB - IM CIN - Arthritis Rheum. 2007 Jun;56(6):2102-3; author reply 2103-4. doi: 10.1002/art.22636. PMID: 17530659 CIN - Perspect Vasc Surg Endovasc Ther. 2007 Sep;19(3):341-2. doi: 10.1177/1531003507301668. PMID: 17911571 MH - Adult MH - Antibodies, Antinuclear/blood MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/classification/*diagnosis/immunology/pathology EDAT- 2006/05/30 09:00 MHDA- 2006/07/15 09:00 CRDT- 2006/05/30 09:00 PHST- 2006/05/30 09:00 [pubmed] PHST- 2006/07/15 09:00 [medline] PHST- 2006/05/30 09:00 [entrez] AID - 10.1002/art.21912 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Jun;54(6):1974-81. doi: 10.1002/art.21912. PMID- 22270434 OWN - NLM STAT- MEDLINE DCOM- 20120325 LR - 20220311 IS - 1873-233X (Electronic) IS - 0029-7844 (Linking) VI - 119 IP - 2 Pt 2 DP - 2012 Feb TI - Raynaud's phenomenon of the nipple. PG - 447-449 LID - 10.1097/AOG.0b013e31822c9a73 [doi] AB - BACKGROUND: Raynaud's phenomenon is a well-described pathologic state in which there is episodic vasospasm followed by vasodilation. It is described most commonly in the digits but also has been shown to affect the nipple vasculature. Raynaud's phenomenon of the nipple may result in discontinuation of breastfeeding secondary to pain and disruption of the maternal-infant bonding process. CASES: We present the cases of two patients with painful breastfeeding associated with color changes of the nipple. Owing to a clinical presentation similar to fungal infections, the patients were treated repeatedly with antifungals before the correct diagnosis was made. Symptoms resolved with a course of nifedipine. CONCLUSION: Increased awareness in the obstetric field will lead to appropriate diagnoses, earlier treatment and relief, and more successful breastfeeding experiences. FAU - Wu, Mae AU - Wu M AD - From the Department of Obstetrics and Gynecology, National Naval Medical Center, and the Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland. FAU - Chason, Rebecca AU - Chason R FAU - Wong, Michelle AU - Wong M LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Obstet Gynecol JT - Obstetrics and gynecology JID - 0401101 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Feeding MH - Female MH - Humans MH - Nifedipine/therapeutic use MH - Nipples/*physiopathology MH - Pain/etiology MH - Raynaud Disease/complications/*diagnosis/drug therapy MH - Vasodilator Agents/therapeutic use MH - Young Adult EDAT- 2012/01/25 06:00 MHDA- 2012/03/27 06:00 CRDT- 2012/01/25 06:00 PHST- 2012/01/25 06:00 [entrez] PHST- 2012/01/25 06:00 [pubmed] PHST- 2012/03/27 06:00 [medline] AID - 00006250-201202001-00012 [pii] AID - 10.1097/AOG.0b013e31822c9a73 [doi] PST - ppublish SO - Obstet Gynecol. 2012 Feb;119(2 Pt 2):447-449. doi: 10.1097/AOG.0b013e31822c9a73. PMID- 32755067 OWN - NLM STAT- MEDLINE DCOM- 20210617 LR - 20210617 IS - 1524-4741 (Electronic) IS - 1075-122X (Linking) VI - 26 IP - 10 DP - 2020 Oct TI - Nipple pain: Raynaud's beyond fingers and toes. PG - 2045-2047 LID - 10.1111/tbj.13991 [doi] AB - Raynaud's phenomenon of the nipple (RPN) is a cause of nipple pain scarcely reported in the literature and frequently missed by physicians. We present a case of RPN in a pregnant mother who sought breast surgical consultation for episodic nipple pain. Review of the literature reveals RPN is predominant in lactating and pregnant patients and missed diagnosis can cause cessation of breastfeeding or mistreatment with antifungals. Clinical suspicion should be raised if symptoms are precipitated by cold, associated with color change, occur during pregnancy or breastfeeding, or with a history of Raynaud's. Treatment is generally supportive, with nifedipine used for severe cases. CI - © 2020 Wiley Periodicals LLC. FAU - Di Como, Joseph AU - Di Como J AD - Breast Health Center, Women and Infants Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA. FAU - Tan, Sydney AU - Tan S AUID- ORCID: 0000-0002-2178-1139 AD - Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA. FAU - Weaver, Micaela AU - Weaver M AD - Breast Health Center, Women and Infants Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA. FAU - Edmonson, David AU - Edmonson D AD - Breast Health Center, Women and Infants Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA. FAU - Gass, Jennifer S AU - Gass JS AD - Breast Health Center, Women and Infants Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200804 PL - United States TA - Breast J JT - The breast journal JID - 9505539 SB - IM MH - *Breast Neoplasms MH - Female MH - Humans MH - Lactation MH - Nipples MH - Pregnancy MH - *Raynaud Disease/diagnosis/etiology MH - Toes OTO - NOTNLM OT - Raynaud's phenomenon OT - Raynaud's phenomenon of the nipple OT - lactation OT - nipple pain EDAT- 2020/08/06 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/08/06 06:00 PHST- 2020/07/09 00:00 [received] PHST- 2020/07/10 00:00 [accepted] PHST- 2020/08/06 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/08/06 06:00 [entrez] AID - 10.1111/tbj.13991 [doi] PST - ppublish SO - Breast J. 2020 Oct;26(10):2045-2047. doi: 10.1111/tbj.13991. Epub 2020 Aug 4. PMID- 41367919 OWN - NLM STAT- MEDLINE DCOM- 20251210 LR - 20251212 IS - 1664-2392 (Print) IS - 1664-2392 (Electronic) IS - 1664-2392 (Linking) VI - 16 DP - 2025 TI - Artifactual hypoglycemia caused by Raynaud's phenomenon: A case report with literature review. PG - 1695633 LID - 10.3389/fendo.2025.1695633 [doi] LID - 1695633 AB - Finger-stick glucose monitoring is commonly used in the clinical management of diabetes as a tool to obtain a reliable estimate of venous glucose levels. However, it should be noted that discrepancies can arise in certain situations between the finger-stick glucose value and venous blood glucose concentration. We present herein the case of a 76-year-old woman with dermatomyositis presenting with artifactual hypoglycemia, in which finger-stick glucose monitoring exhibited false-low values due to Raynaud's phenomenon. Despite the low glucose level (<54 mg/dL) on finger-stick glucose monitoring, she was asymptomatic, and occasional laboratory blood tests failed to detect apparent hypoglycemia. We suspected artifactual hypoglycemia to be caused by Raynaud's phenomenon, and consistently, switching the blood sampling site from the finger to the earlobe ameliorated the discrepancy against the actual venous glucose levels. Given the prevalence of steroid-induced diabetes in patients with Raynaud's phenomenon, clinicians should be aware that finger-stick glucose monitoring can present false-low values due to Raynaud's phenomenon, thus avoiding unnecessary investigations searching for the cause of "hypoglycemia," or conversely, preventing underestimation of the actual hyperglycemia. CI - Copyright © 2025 Nishioka, Yamamotoya, Itoda, Ichikawa, Nishiyama, Takubo, Nagasawa, Kosuda, Egashira, Fujishiro, Watanabe and Ishihara. FAU - Nishioka, Hidenori AU - Nishioka H AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Yamamotoya, Takeshi AU - Yamamotoya T AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Itoda, Yuki AU - Itoda Y AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Ichikawa, Chikako AU - Ichikawa C AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Nishiyama, Mai AU - Nishiyama M AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Takubo, Masahiro AU - Takubo M AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Nagasawa, Akiko AU - Nagasawa A AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Kosuda, Minami AU - Kosuda M AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Egashira, Fujiko AU - Egashira F AD - Department of Diabetes and Metabolism, Nihon University School of Medicine Itabashi Hospital, Tokyo, Japan. FAU - Fujishiro, Midori AU - Fujishiro M AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Watanabe, Kentaro AU - Watanabe K AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. FAU - Ishihara, Hisamitsu AU - Ishihara H AD - Division of Diabetes and Metabolic Diseases, Nihon University School of Medicine, Tokyo, Japan. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20251124 PL - Switzerland TA - Front Endocrinol (Lausanne) JT - Frontiers in endocrinology JID - 101555782 RN - 0 (Blood Glucose) SB - IM MH - Humans MH - Female MH - *Raynaud Disease/complications/blood MH - Aged MH - *Hypoglycemia/etiology/blood/diagnosis MH - *Blood Glucose Self-Monitoring/methods MH - *Artifacts MH - Blood Glucose/analysis MH - Dermatomyositis/complications/blood PMC - PMC12683289 OTO - NOTNLM OT - Raynaud’s phenomenon OT - artifactual hypoglycemia OT - false-low glucose value OT - finger-stick glucose monitoring OT - pseudo-hypoglycemia COIS- MF received funding from Eli Lilly. KW received funding from the Japan Diabetes and Novo Nordisk Pharma Foundation. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. EDAT- 2025/12/10 06:28 MHDA- 2025/12/10 06:29 PMCR- 2025/11/24 CRDT- 2025/12/10 04:49 PHST- 2025/08/30 00:00 [received] PHST- 2025/11/03 00:00 [accepted] PHST- 2025/12/10 06:29 [medline] PHST- 2025/12/10 06:28 [pubmed] PHST- 2025/12/10 04:49 [entrez] PHST- 2025/11/24 00:00 [pmc-release] AID - 10.3389/fendo.2025.1695633 [doi] PST - epublish SO - Front Endocrinol (Lausanne). 2025 Nov 24;16:1695633. doi: 10.3389/fendo.2025.1695633. eCollection 2025. PMID- 32686771 OWN - NLM STAT- MEDLINE DCOM- 20211103 LR - 20211103 IS - 1804-7521 (Electronic) IS - 1213-8118 (Linking) VI - 165 IP - 1 DP - 2021 Mar TI - Infrared thermography and capillaroscopy in the diagnosis of Raynaud's phenomenon. PG - 90-98 LID - 10.5507/bp.2020.031 [doi] AB - AIMS: Raynaud's phenomenon (RP) is a relatively common disease. There are two distinct forms of RP - primary (PRP), where no other associated diseases are present, and secondary (SRP), where RP is associated with other diseases. It can be challenging to differentiate between RP and other diseases through medical history alone, due to the episodic nature of RP. Objective analysis of anamnestic data was performed in our study using infrared thermography (IRT) and a cold pressor test (CPT). Capillaroscopy was performed to assess morphological changes in the acral circulation. METHODS: Patients with a history of cold hands were included in the study. IRT was performed before and after the CPT, and then capillaroscopy was performed. The results (including epidemiologic data) were statistically evaluated. RESULTS: A total of 150 patients were included in the study. Summarisation of the results from the IRT and capillaroscopy determined the final diagnosis - 4.7% acrocyanosis, 10.7% physiologic findings, 31.3% PRP, 29.3% borderline SRP and 24% SRP. The coldest fingers following the CPT were, in most patients, the 2(nd) and 3(rd) fingers. The correlation between the presence of connective tissue disease and the diagnosis of borderline SRP and SRP was significant (P=0.0001). CONCLUSIONS: Using the combination of the IRT and capillaroscopy in the diagnostic algorithm for RP has its justification. IRT distinguishes healthy patients from patients with RP, and capillaroscopy can then be used to differentiate PRP from SRP. IRT can also detect which fingers are more affected, and then these can direct the focus of capillaroscopy. FAU - Sternbersky, Jan AU - Sternbersky J AD - Department of Dermatology and Venereology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic. FAU - Tichy, Martin AU - Tichy M AD - Department of Dermatology and Venereology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic. FAU - Zapletalova, Jana AU - Zapletalova J AD - Department of Medical Biophysics, Faculty of Medicine and Dentistry, Palacky University Olomouc, Czech Republic. LA - eng PT - Journal Article DEP - 20200715 PL - Czech Republic TA - Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub JT - Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia JID - 101140142 SB - IM MH - Adult MH - Female MH - Humans MH - Infrared Rays MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - *Thermography OTO - NOTNLM OT - Raynaud's phenomenon OT - capillaroscopy OT - cold pressor test OT - connective tissue disease OT - infrared thermography EDAT- 2020/07/21 06:00 MHDA- 2021/11/04 06:00 CRDT- 2020/07/21 06:00 PHST- 2019/09/04 00:00 [received] PHST- 2020/07/07 00:00 [accepted] PHST- 2020/07/21 06:00 [pubmed] PHST- 2021/11/04 06:00 [medline] PHST- 2020/07/21 06:00 [entrez] AID - 10.5507/bp.2020.031 [doi] PST - ppublish SO - Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2021 Mar;165(1):90-98. doi: 10.5507/bp.2020.031. Epub 2020 Jul 15. PMID- 31023622 OWN - NLM STAT- MEDLINE DCOM- 20200429 LR - 20200429 IS - 1958-5578 (Electronic) IS - 0040-5957 (Linking) VI - 74 IP - 6 DP - 2019 Dec TI - French translation and linguistic validation of the Raynaud's condition score. PG - 627-631 LID - S0040-5957(19)30057-5 [pii] LID - 10.1016/j.therap.2019.03.002 [doi] AB - The Raynaud's condition score is a 11-point scale severity score used in Raynaud's phenomenon clinical trials since 1998. The Raynaud's condition score diary has been recommended for use in clinical trials assessing efficacy of interventions on scleroderma related Raynaud's phenomenon. However, this score has never been formally validated in French. We thus performed a translation and a linguistic validation of the Raynaud's condition score through a forward/backward translations process followed by an expert review and cognitive patient interviews. The translations led to a French version of the Raynaud's condition score that was linguistically valid, and conceptually equivalent to the original English version. This "Score de Raynaud" will be usable to perform and harmonize clinical trials in French-speaking patients with secondary Raynaud's phenomenon. CI - Copyright © 2019 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved. FAU - Khouri, Charles AU - Khouri C AD - Pharmacovigilance unit, Grenoble Alpes university hospital, 38000 Grenoble, France; Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, 38000 Grenoble, France; Inserm, UMR 1042-HP2, university Grenoble Alpes, 38000 Grenoble, France. Electronic address: CKhouri@chu-grenoble.fr. FAU - Blaise, Sophie AU - Blaise S AD - Inserm, UMR 1042-HP2, university Grenoble Alpes, 38000 Grenoble, France; Department of vascular medicine, Grenoble university hospital, 38000 Grenoble, France. FAU - Guigui, Alicia AU - Guigui A AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, 38000 Grenoble, France. FAU - Cracowski, Claire AU - Cracowski C AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, 38000 Grenoble, France. FAU - Allanore, Yannick AU - Allanore Y AD - Service de rhumatologie A, hôpital Cochin, université Paris Descartes, 75014 Paris, France. FAU - Hachulla, Eric AU - Hachulla E AD - Service de médecine interne, Centre de référence des maladies auto-immunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), hôpital Claude Huriez, CHU de Lille, 59045 Lille, France. FAU - Senet, Patricia AU - Senet P AD - Service de dermatologie et allergologie, hôpital Tenon, groupe hospitalier universitaire Paris Est, Assistance publique-Hôpitaux de Paris, 75020 Paris, France. FAU - Roustit, Mathieu AU - Roustit M AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, 38000 Grenoble, France; Inserm, UMR 1042-HP2, university Grenoble Alpes, 38000 Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, 38000 Grenoble, France; Inserm, UMR 1042-HP2, university Grenoble Alpes, 38000 Grenoble, France. LA - eng PT - Journal Article PT - Validation Study DEP - 20190402 PL - France TA - Therapie JT - Therapie JID - 0420544 SB - IM MH - *Diagnostic Techniques, Cardiovascular/standards MH - France MH - Humans MH - Interviews as Topic MH - *Language MH - Linguistics MH - *Patient Outcome Assessment MH - Raynaud Disease/*diagnosis/pathology MH - Research Design MH - Scleroderma, Systemic/diagnosis/*therapy MH - Severity of Illness Index MH - *Translating MH - Treatment Outcome OTO - NOTNLM OT - Raynaud's phenomenon OT - Systemic scleroderma EDAT- 2019/04/27 06:00 MHDA- 2020/04/30 06:00 CRDT- 2019/04/27 06:00 PHST- 2019/01/21 00:00 [received] PHST- 2019/03/15 00:00 [accepted] PHST- 2019/04/27 06:00 [pubmed] PHST- 2020/04/30 06:00 [medline] PHST- 2019/04/27 06:00 [entrez] AID - S0040-5957(19)30057-5 [pii] AID - 10.1016/j.therap.2019.03.002 [doi] PST - ppublish SO - Therapie. 2019 Dec;74(6):627-631. doi: 10.1016/j.therap.2019.03.002. Epub 2019 Apr 2. PMID- 35764895 OWN - NLM STAT- MEDLINE DCOM- 20221101 LR - 20260127 IS - 1590-3478 (Electronic) IS - 1590-1874 (Linking) VI - 43 IP - 11 DP - 2022 Nov TI - Teriflunomide-induced Raynaud's phenomenon: a serious adverse event, previously unreported. PG - 6593-6594 LID - 10.1007/s10072-022-06239-w [doi] FAU - Riancho, Javier AU - Riancho J AUID- ORCID: 0000-0001-7929-1055 AD - Service of Neurology. Hospital Sierrallana-IDIVAL, Barrio Ganzo S/N 39300, Torrelavega, Spain. javier.riancho86@gmail.com. AD - Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. javier.riancho86@gmail.com. AD - Centro de Investigación en Red de Enfermedades Neurodegenerativas, CIBERNED, Instituto Carlos III, Madrid, Spain. javier.riancho86@gmail.com. FAU - Delgado-Alvarado, Manuel AU - Delgado-Alvarado M AD - Service of Neurology. Hospital Sierrallana-IDIVAL, Barrio Ganzo S/N 39300, Torrelavega, Spain. AD - Centro de Investigación en Red de Enfermedades Neurodegenerativas, CIBERNED, Instituto Carlos III, Madrid, Spain. FAU - Riancho-Zarrabeitia, Leyre AU - Riancho-Zarrabeitia L AD - Service of Rheumatology, Hospital Sierrallana, IDIVAL, Torrelavega, Spain. LA - eng PT - Letter DEP - 20220629 PL - Italy TA - Neurol Sci JT - Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology JID - 100959175 RN - 0 (Crotonates) RN - 0 (Toluidines) RN - 0 (Hydroxybutyrates) RN - 1C058IKG3B (teriflunomide) RN - 0 (Nitriles) SB - IM MH - Humans MH - *Raynaud Disease/chemically induced MH - Crotonates/adverse effects MH - Toluidines/adverse effects MH - Hydroxybutyrates MH - Nitriles OTO - NOTNLM OT - Multiple sclerosis OT - Raynaud OT - Teriflunomide EDAT- 2022/06/29 06:00 MHDA- 2022/11/02 06:00 CRDT- 2022/06/28 23:32 PHST- 2022/05/17 00:00 [received] PHST- 2022/06/24 00:00 [accepted] PHST- 2022/06/29 06:00 [pubmed] PHST- 2022/11/02 06:00 [medline] PHST- 2022/06/28 23:32 [entrez] AID - 10.1007/s10072-022-06239-w [pii] AID - 10.1007/s10072-022-06239-w [doi] PST - ppublish SO - Neurol Sci. 2022 Nov;43(11):6593-6594. doi: 10.1007/s10072-022-06239-w. Epub 2022 Jun 29. PMID- 26932288 OWN - NLM STAT- MEDLINE DCOM- 20170215 LR - 20191113 IS - 1573-3971 (Print) IS - 1573-3971 (Linking) VI - 9 IP - 4 DP - 2013 TI - From Raynaud's Phenomenon to Very Early Diagnosis of Systemic Sclerosis- The VEDOSS approach. PG - 245-8 AB - Systemic sclerosis (SSc) is a heterogeneous chronic autoimmune disease that is very difficult to diagnose in the early phase, because of the low sensitivity of the classification criteria currently used to identify patients without skin involvement, with an important delay on the therapy that is often started when internal organ involvement has already irreversible. The biggest challenge in the fight against SSc is to detect valid predictors of disease so as to treat patients since the earliest stages of disease. Raynaud's phenomenon (RP), antinuclear antibodies (ANA) positivity, and puffy fingers have been recently indicated as the"red flags", the main elements to suspect SSc and then to perform further tests to confirm the diagnosis in particular nailfold video capillaroscopy and evaluation of specific disease antibodies (anticentromere and anti-topoisomerase I). Particularly, RP is the earliest, even if aspecific sign of SSc, and more and more attention should be paid to its early identification in order to reduce the diagnostic delay. Besides, the time gap between the onset of RP and the diagnosis should be considered as a "window of opportunity" for SSc patients, through which the physician can act with effective drugs able to block or at least slow the progression of the disease. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department Rheumatology AVC, Department Biomedicine AOUC, Division Rheumatology AOUC, Department Medicine Denothe Centre, University of Florence, Italy, Villa Monna Tessa, Viale Pieraccini 18, 50139 Firenze Italy. cerinic@unifi.it. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Early Diagnosis MH - Humans MH - Raynaud Disease/*diagnosis/etiology MH - Scleroderma, Systemic/complications/*diagnosis EDAT- 2013/01/01 00:00 MHDA- 2017/02/16 06:00 CRDT- 2016/03/03 06:00 PHST- 2016/03/03 06:00 [entrez] PHST- 2013/01/01 00:00 [pubmed] PHST- 2017/02/16 06:00 [medline] AID - CRR-EPUB-60155 [pii] AID - 10.2174/157339710904140417124819 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2013;9(4):245-8. doi: 10.2174/157339710904140417124819. PMID- 27870974 OWN - NLM STAT- MEDLINE DCOM- 20171109 LR - 20171109 IS - 1874-1754 (Electronic) IS - 0167-5273 (Linking) VI - 228 DP - 2017 Feb 1 TI - Myocardial perfusion in peripheral Raynaud's phenomenon. Evaluation using stress cardiovascular magnetic resonance. PG - 444-448 LID - S0167-5273(16)33783-4 [pii] LID - 10.1016/j.ijcard.2016.11.242 [doi] AB - BACKGROUND: Peripheral Raynaud's phenomenon (RP) is either primary (PRP), without any coexisting disease or secondary (SRP), due to connective tissue diseases (CTD). We hypothesized that adenosine stress cardiovascular magnetic resonance (CMR) can assess myocardial perfusion in a population of PRP and SRP. PATIENTS-METHODS: Twenty CTDs, aged 30.6±7.5yrs., 16F/4M, including 9 systemic sclerosis (SSc), 4 systemic lupus erythematosus (SLE), 3 mixed connective tissue disease (MCTD), 2 polymyositis (PM) and 2 rheumatoid arthritis (RA), with SRP, under treatment with calcium blockers, were evaluated by stress CMR and compared with age-sex matched PRP and controls. All RP patients were under treatment with calcium blockers. Stress perfusion CMR was performed by 1.5T system using 140mg/kg/min adenosine for 4min and 0.05mmol/kg Gd-DTPA for first-pass perfusion. A rest perfusion was performed with the same protocol. Late gadolinium enhanced (LGE) images were acquired after another dose of Gd-DTPA. RESULTS: In both PRP, SRP, the myocardial perfusion reserve index (MPRI) was significantly reduced compared with the controls (1.7±0.6 vs 3.5±0.4, p<0.001 and 0.7±0.2 vs 3.5±0.4, p<0.001, respectively). Furthermore, in SRP, MPRI was significantly reduced, compared with PRP (0.7±0.2 vs 1.7±0.6, p<0.001). Subendo-cardial LGE=8.2±1.7 of LV mass was revealed in 1 SLE, 1MCTD and 2 SSc, but in none of PR patients. CONCLUSIONS: MPRI reduction is common in both PRP and SRP, but it is more severe in SRP, even if RP patients are under treatment with calcium blockers. Occult fibrosis may coexist with the reduced MPRI in SRP but not in PRP. CI - Copyright © 2016 Elsevier Ireland Ltd. All rights reserved. FAU - Mavrogeni, Sophie AU - Mavrogeni S AD - Onassis Cardiac Surgery Center, Athens, Greece. Electronic address: soma13@otenet.gr. FAU - Bratis, Konstantinos AU - Bratis K AD - Onassis Cardiac Surgery Center, Athens, Greece. FAU - Koutsogeorgopoulou, Loukia AU - Koutsogeorgopoulou L AD - Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece. FAU - Karabela, Georgia AU - Karabela G AD - Athens Navy Hospital, Athens, Greece. FAU - Savropoulos, Efthymios AU - Savropoulos E AD - Athens Navy Hospital, Athens, Greece. FAU - Katsifis, Gikas AU - Katsifis G AD - Athens Navy Hospital, Athens, Greece. FAU - Raftakis, John AU - Raftakis J AD - Asklipion Hospital, Athens, Greece. FAU - Markousis-Mavrogenis, George AU - Markousis-Mavrogenis G AD - Onassis Cardiac Surgery Center, Athens, Greece. FAU - Kolovou, Genovefa AU - Kolovou G AD - Onassis Cardiac Surgery Center, Athens, Greece. LA - eng PT - Journal Article DEP - 20161112 PL - Netherlands TA - Int J Cardiol JT - International journal of cardiology JID - 8200291 SB - IM MH - Adult MH - Coronary Circulation/*physiology MH - Exercise Test/*methods MH - Female MH - Humans MH - Magnetic Resonance Imaging, Cine/*methods MH - Male MH - Myocardial Ischemia/*diagnosis/etiology/physiopathology MH - Myocardium/*pathology MH - Raynaud Disease/complications/*diagnosis/physiopathology MH - Reproducibility of Results OTO - NOTNLM OT - Myocardial fibrosis OT - Myocardial ischemia OT - Raynaud's phenomenon OT - Stress cardiovascular magnetic resonance EDAT- 2016/11/22 06:00 MHDA- 2017/11/10 06:00 CRDT- 2016/11/22 06:00 PHST- 2016/08/06 00:00 [received] PHST- 2016/11/06 00:00 [revised] PHST- 2016/11/10 00:00 [accepted] PHST- 2016/11/22 06:00 [pubmed] PHST- 2017/11/10 06:00 [medline] PHST- 2016/11/22 06:00 [entrez] AID - S0167-5273(16)33783-4 [pii] AID - 10.1016/j.ijcard.2016.11.242 [doi] PST - ppublish SO - Int J Cardiol. 2017 Feb 1;228:444-448. doi: 10.1016/j.ijcard.2016.11.242. Epub 2016 Nov 12. PMID- 33965340 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20211101 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 35 IP - 3 DP - 2021 Sep TI - Botulinum toxin in the management of primary and secondary Raynaud's phenomenon. PG - 101684 LID - S1521-6942(21)00026-7 [pii] LID - 10.1016/j.berh.2021.101684 [doi] AB - Raynaud's phenomenon (RP) is common in rheumatic diseases. In the setting of systemic sclerosis (SSc), it can be complicated by digital ischemia that includes ulceration and gangrene. Systemic adverse effects may preclude the use of oral or topical vasodilators for the treatment of RP and its complications. In this article, we review effectiveness/efficacy of botulinum toxin injection in primary and secondary RP. We discuss botulinum toxin formulations, dosage, sites of administration, and adverse effects. The evidence for botulinum toxin in the treatment of primary and SSc-associated RP is promising. Consistency across patient populations, treatment options (botulinum serotype, dose, and injection site), and outcome measures will be essential for further research. CI - Copyright © 2021 Elsevier Ltd. All rights reserved. FAU - Ennis, Daniel AU - Ennis D AD - Mary Pack Vasculitis Clinic, Division of Rheumatology, Department of Medicine, Vancouver General Hospital, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: Daniel.Ennis@vch.ca. FAU - Ahmad, Zareen AU - Ahmad Z AD - Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada. Electronic address: zareen.ahmad@sinaihealth.ca. FAU - Anderson, Melanie A AU - Anderson MA AD - University Health Network Library and Information Services, Toronto, Ontario, Canada. Electronic address: melanie.anderson@uhn.ca. FAU - Johnson, Sindhu R AU - Johnson SR AD - Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, Toronto Western Hospital, Toronto, Ontario, Canada; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. Electronic address: Sindhu.Johnson@uhn.ca. LA - eng PT - Journal Article PT - Review DEP - 20210505 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - *Botulinum Toxins MH - Humans MH - *Raynaud Disease/drug therapy/etiology MH - *Rheumatic Diseases MH - *Scleroderma, Systemic/complications/drug therapy MH - *Skin Ulcer OTO - NOTNLM OT - Botox OT - Botulinum toxin OT - Raynaud's phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of competing interest Dr. Johnson has been a site investigator for trials supported by Bayer, Boehringer Ingelheim, Corbus, GSK, Roche, Merck and served in advisory boards sponsored by Boehringer Ingelheim and Ikaria. EDAT- 2021/05/10 06:00 MHDA- 2021/11/03 06:00 CRDT- 2021/05/09 20:32 PHST- 2021/05/10 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] PHST- 2021/05/09 20:32 [entrez] AID - S1521-6942(21)00026-7 [pii] AID - 10.1016/j.berh.2021.101684 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101684. doi: 10.1016/j.berh.2021.101684. Epub 2021 May 5. PMID- 27154994 OWN - NLM STAT- MEDLINE DCOM- 20170123 LR - 20181113 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2016 DP - 2016 May 6 TI - Corkscrew collaterals in Raynaud's syndrome. LID - 10.1136/bcr-2016-215841 [doi] LID - bcr2016215841 FAU - Fujii, Yuichi AU - Fujii Y AD - Department of Cardiovascular Medicine, JR Hiroshima Hospital, Hiroshima, Japan. FAU - Teragawa, Hiroki AU - Teragawa H AD - Department of Cardiovascular Medicine, JR Hiroshima Hospital, Hiroshima, Japan. FAU - Kihara, Yasuki AU - Kihara Y AD - Department of Cardiovascular Medicine, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan. FAU - Higashi, Yukihito AU - Higashi Y AD - Department of Human Genetics, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20160506 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Angiography/*methods MH - Cold Temperature MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Raynaud Disease/*diagnostic imaging/etiology MH - Ultrasonography, Doppler, Duplex/*methods PMC - PMC4885451 EDAT- 2016/05/08 06:00 MHDA- 2017/01/24 06:00 PMCR- 2018/05/06 CRDT- 2016/05/08 06:00 PHST- 2016/05/08 06:00 [entrez] PHST- 2016/05/08 06:00 [pubmed] PHST- 2017/01/24 06:00 [medline] PHST- 2018/05/06 00:00 [pmc-release] AID - bcr-2016-215841 [pii] AID - 10.1136/bcr-2016-215841 [doi] PST - epublish SO - BMJ Case Rep. 2016 May 6;2016:bcr2016215841. doi: 10.1136/bcr-2016-215841. PMID- 22821334 OWN - NLM STAT- MEDLINE DCOM- 20131030 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 4 DP - 2013 Apr TI - Late appearance and exacerbation of primary Raynaud's phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3,035 patients. PG - 921-6 LID - 10.1007/s00296-012-2484-3 [doi] AB - To assess the prognostic value of the age at onset of Raynaud's (RP) and of a history of exacerbation of RP attacks for the development of connective tissue disease (CTD) in patients initially found to have primary Raynaud's. 3,035 patients with primary RP (2,702 women and 333 men) were followed for an average of 4.8 years (range from 1 to 10 years). At baseline and every 6 months, they were screened for signs and symptoms of CTD. At 4.8 years of follow-up, 54.7 % patients remained as primary RP, 8.1 % had developed suspected secondary RP, and 37.2 % had developed a definite CTD. Primary RP patients had an earlier onset of RP (mean age of 32.2 years) than those with suspected (mean age 36.5 years, P = .007) or definite secondary RP associated with CTD (mean age of 39.8 years, P = .004). RP beginning before the age of forty was not significantly associated with the development of CTD. Conversely, the appearance of RP after the age of 40 was significantly associated with the development of CTD (P = .00001). Worsening of RP attacks predicted the development of CTD, especially systemic sclerosis (relative risk [RR] of 1.42), scleroderma overlap syndrome (RR of 1.18), and mixed CTD (RR of 1.18). Patients whose onset of RP occurred past 40 years of age and those with worsening RP attacks were at risk for the future development of CTD. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AD - Institute of Rheumatology, Belgrade, Serbia. dolijan@eunet.rs FAU - Damjanov, Nemanja S AU - Damjanov NS FAU - Vujasinovic Stupar, Nada Z AU - Vujasinovic Stupar NZ FAU - Radunovic, Goran L AU - Radunovic GL FAU - Stojanovic, Roksanda M AU - Stojanovic RM FAU - Babic, Dragan AU - Babic D LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120722 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Age of Onset MH - Connective Tissue Diseases/diagnosis/*etiology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/complications/*diagnosis MH - Retrospective Studies MH - Risk MH - Severity of Illness Index EDAT- 2012/07/24 06:00 MHDA- 2013/10/31 06:00 CRDT- 2012/07/24 06:00 PHST- 2012/02/08 00:00 [received] PHST- 2012/07/07 00:00 [accepted] PHST- 2012/07/24 06:00 [entrez] PHST- 2012/07/24 06:00 [pubmed] PHST- 2013/10/31 06:00 [medline] AID - 10.1007/s00296-012-2484-3 [doi] PST - ppublish SO - Rheumatol Int. 2013 Apr;33(4):921-6. doi: 10.1007/s00296-012-2484-3. Epub 2012 Jul 22. PMID- 18437399 OWN - NLM STAT- MEDLINE DCOM- 20080926 LR - 20260128 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 67 IP - 3 DP - 2008 May TI - [Primary and secondary Raynaud's phenomenon]. PG - 211-7; quiz 218-9 LID - 10.1007/s00393-008-0282-9 [doi] AB - The term Raynaud's phenomenon describes an abnormal vasospastic response to cold or emotional stress. It is a common condition with a prevalence of 3-5% of the population. Clinically, Raynaud's phenomenon manifests as sharply demarcated colour changes of the skin of the digits that is often accompanied by paraesthesia. Raynaud's phenomenon can be subdivided into primary, or idiopathic, and secondary forms, in the latter of which associated diseases or causes can be identified. The pathogenesis of the disease is incompletely understood. Pathologic changes have been observed primarily in vascular smooth muscle cells, endothelial cells and perineuronal microvasculature. Current therapeutic strategies include supportive treatments, topical therapeutic approaches and systemic medication. Drug therapies with proven efficacy include calcium channel blockers, prostacyclin analogues, fluoxetine, losartan and sildenafil. FAU - Distler, J H W AU - Distler JH AD - Medizinische Klinik 3 und Institut für klinische Immunologie, Universität Erlangen-Nürnberg, Universitätsstrasse 29, 91054 Erlangen. joerg.distler@uk-erlangen.de LA - ger PT - English Abstract PT - Journal Article TT - Primäres und sekundäres Raynaud-Phänomen. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Angiotensin II Type 1 Receptor Blockers) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Prostaglandins I) RN - 0 (Serotonin Antagonists) RN - 0 (Selective Serotonin Reuptake Inhibitors) SB - IM MH - Angiotensin II Type 1 Receptor Blockers/adverse effects/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/adverse effects/therapeutic use MH - Calcium Channel Blockers/adverse effects/therapeutic use MH - Combined Modality Therapy MH - Endothelium, Vascular/pathology MH - Humans MH - Life Style MH - Muscle, Smooth, Vascular/pathology MH - Phosphodiesterase Inhibitors/adverse effects/therapeutic use MH - Prostaglandins I/therapeutic use MH - Raynaud Disease/*diagnosis/*etiology/pathology/therapy MH - Risk Factors MH - Serotonin Antagonists/adverse effects/therapeutic use MH - Selective Serotonin Reuptake Inhibitors/adverse effects/therapeutic use EDAT- 2008/04/26 09:00 MHDA- 2008/09/27 09:00 CRDT- 2008/04/26 09:00 PHST- 2008/04/26 09:00 [pubmed] PHST- 2008/09/27 09:00 [medline] PHST- 2008/04/26 09:00 [entrez] AID - 10.1007/s00393-008-0282-9 [doi] PST - ppublish SO - Z Rheumatol. 2008 May;67(3):211-7; quiz 218-9. doi: 10.1007/s00393-008-0282-9. PMID- 36422688 OWN - NLM STAT- MEDLINE DCOM- 20221128 LR - 20221128 IS - 1788-6120 (Electronic) IS - 0030-6002 (Linking) VI - 163 IP - 47 DP - 2022 Nov 20 TI - [Factors determining the quality of life in patients with Raynaud's syndrome]. PG - 1880-1885 LID - 10.1556/650.2022.32647 [doi] AB - INTRODUCTION: Raynaud's disease is a vasospastic phenomenon affecting acral areas, which manifests itself in characteristic color changes. Symptoms are affected by mundane things like stress or temperature. There are also differences in the presence and progression of the disease in terms of gender, age, health-damaging behaviors (e.g., smoking) and occupation. OBJECTIVE: The aim of our study was to examine how the risk factors assumed in the literature affect the quality of life of patients with Raynaud's disease. METHOD: 110 people diagnosed with Raynaud's disease completed a questionnaire on disease-specific quality of life and risk factors. RESULTS: There was a significant difference between the groups with good and less good quality of life in terms of age (p<0.001), education (p<0.01), type of diagnosis (p<0.001), duration of illness (p<0.001), headache frequency (p<0.01), the influence of cold (p<0.05) and emotions (p<0.01). The groups currently working (p<0.01), drinking coffee more often (p<0.05), attributing less influence to emotions (p<0.001) and cold (p<0.01) had a better quality of life. According to the regression analysis, the type of diagnosis, the duration of the disease, the influencing role of emotions and cold are the most important predictors of Raynaud-specific quality of life. CONCLUSION: Our results draw attention to the role of factors that potentially affect the long-term development of the quality of life, thereby identifying the possible focuses of prevention. Orv Hetil. 2022; 163(47): 1880-1885. FAU - Hajnal, Réka Krisztina AU - Hajnal RK AD - 1 Szent Borbála Kórház, I. Belgyógyászat Tatabánya Magyarország. FAU - Csatári, László AU - Csatári L AD - 2 Debreceni Egyetem, Általános Orvostudományi Kar, Magatartástudományi Intézet Debrecen, Móricz Zs. út 22., 4032 Magyarország. FAU - Fábián, Balázs AU - Fábián B AD - 2 Debreceni Egyetem, Általános Orvostudományi Kar, Magatartástudományi Intézet Debrecen, Móricz Zs. út 22., 4032 Magyarország. FAU - Csiki, Zoltán AU - Csiki Z AD - 3 Debreceni Egyetem, Általános Orvostudományi Kar, Belgyógyászati Klinika Debrecen Magyarország. LA - hun PT - English Abstract PT - Journal Article TT - Az életminőséget meghatározó tényezők Raynaud-szindrómában. DEP - 20221120 PL - Hungary TA - Orv Hetil JT - Orvosi hetilap JID - 0376412 SB - IM MH - Humans MH - *Quality of Life/psychology MH - *Raynaud Disease/psychology MH - Surveys and Questionnaires MH - Risk Factors MH - Emotions OTO - NOTNLM OT - Raynaud-szindróma OT - Raynaud’s syndrome OT - quality of life OT - risk factors OT - rizikótényezők OT - életminőség EDAT- 2022/11/25 06:00 MHDA- 2022/11/29 06:00 CRDT- 2022/11/24 11:24 PHST- 2022/08/12 00:00 [received] PHST- 2022/09/02 00:00 [accepted] PHST- 2022/11/24 11:24 [entrez] PHST- 2022/11/25 06:00 [pubmed] PHST- 2022/11/29 06:00 [medline] AID - 10.1556/650.2022.32647 [doi] PST - epublish SO - Orv Hetil. 2022 Nov 20;163(47):1880-1885. doi: 10.1556/650.2022.32647. Print 2022 Nov 20. PMID- 25543422 OWN - NLM STAT- MEDLINE DCOM- 20150312 LR - 20141229 IS - 0255-2930 (Print) IS - 0255-2930 (Linking) VI - 34 IP - 10 DP - 2014 Oct TI - [Case of Raynaud's disease]. PG - 960 FAU - Gao, Xi-yan AU - Gao XY FAU - Sun, Cui-ying AU - Sun CY LA - chi PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China TA - Zhongguo Zhen Jiu JT - Zhongguo zhen jiu = Chinese acupuncture & moxibustion JID - 8600658 SB - IM MH - Acupuncture Points MH - Acupuncture Therapy MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*therapy EDAT- 2014/12/30 06:00 MHDA- 2015/03/13 06:00 CRDT- 2014/12/30 06:00 PHST- 2014/12/30 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2015/03/13 06:00 [medline] PST - ppublish SO - Zhongguo Zhen Jiu. 2014 Oct;34(10):960. PMID- 29350104 OWN - NLM STAT- MEDLINE DCOM- 20181026 LR - 20181202 IS - 1819-6357 (Electronic) IS - 1993-2820 (Print) IS - 1819-6357 (Linking) VI - 13 IP - 1 DP - 2018 Dec TI - The importance of perfusion index monitoring in evaluating the efficacy of stellate ganglion blockage treatment in Raynaud's disease. PG - 1422666 LID - 10.1080/19932820.2017.1422666 [doi] LID - 1422666 AB - Stellate ganglion blockage (SGB) is a method used for treating Raynaud's phenomenon (RP). This study primarily aimed to determine whether the perfusion index (PI) can be used an alternative to Horner's signs in evaluating the efficacy of SGB in patients diagnosed with RP. In a total of 40 patients, aged 18-65 years and diagnosed with primary RP, SGB was applied for 5 days on the same side with the 2-finger method, using 6 mL of 5% levobupivacaine at the 7th cervical vertebra level. The PI values were recorded from the distal end of the 2nd finger of the upper extremity on the side applied with the block at baseline and at 5, 15, 30, 60 and 120 min. The onset time of Horner findings was recorded. The PI values and visual analogue scale (VAS) pain scores were recorded pre-treatment and after 2 weeks.When the PI values of the 40 patients were examined, a 62.7% increase was observed from baseline to the first session at 5 min (p < 0.05). When all sessions were evaluated, a statistically significant increase was determined in the PI values measured at 5, 15, 30, 60 and 120 min compared with the baseline PI values. There was a statistically significant decrease in the post-treatment VAS pain scores and a statistically significant increase in the post-treatment PI values (p < 0.05). By eliminating peripheral vasospasm with the application of SGB in patients with RP, the distal artery blood flow and PI are increased. PI measurement is a more objective method and therefore could be used as an alternative to Horner findings in evaluating the success of SGB. PI is a non-invasive and simple measurement and also an earlier indicator in evaluating the success of SGB than Horner's signs. FAU - Şahin, Ömer Fatih AU - Şahin ÖF AUID- ORCID: 0000-0003-1953-9121 AD - a Department of Anesthesiology and Reanimation , Diyarbakır Bismil State Hospital , Bismil , Diyarbakır. FAU - Tarıkçı Kılıç, Ebru AU - Tarıkçı Kılıç E AD - b Department of Anesthesiology and Reanimation , Ümraniye Training and Research Hospital , Istanbul. FAU - Aksoy, Yakup AU - Aksoy Y AUID- ORCID: 0000-0002-1681-7459 AD - a Department of Anesthesiology and Reanimation , Diyarbakır Bismil State Hospital , Bismil , Diyarbakır. FAU - Kaydu, Ayhan AU - Kaydu A AUID- ORCID: 0000-0002-7781-8885 AD - c Department of Anesthesiology and Reanimation , Diyarbakır Selahaddin Eyyübi State Hospital , Diyarbakır. FAU - Gökçek, Erhan AU - Gökçek E AD - c Department of Anesthesiology and Reanimation , Diyarbakır Selahaddin Eyyübi State Hospital , Diyarbakır. LA - eng PT - Journal Article PL - United States TA - Libyan J Med JT - The Libyan journal of medicine JID - 101299403 RN - 0 (Anesthetics, Local) RN - A5H73K9U3W (Levobupivacaine) RN - Y8335394RO (Bupivacaine) SB - IM MH - Adult MH - Anesthetics, Local MH - *Autonomic Nerve Block MH - Bupivacaine/analogs & derivatives MH - Female MH - Humans MH - Levobupivacaine MH - Male MH - Middle Aged MH - Oximetry MH - Pain/etiology MH - Pain Measurement MH - Raynaud Disease/complications/physiopathology/*therapy MH - Regional Blood Flow MH - *Stellate Ganglion MH - Time Factors MH - Treatment Outcome MH - Young Adult PMC - PMC5774394 OTO - NOTNLM OT - Raynaud’s phenomenon OT - perfusion index OT - stellate ganglion blockage; pain; Horner's sign COIS- No potential conflict of interest was reported by the authors. EDAT- 2018/01/20 06:00 MHDA- 2018/10/27 06:00 PMCR- 2018/01/19 CRDT- 2018/01/20 06:00 PHST- 2018/01/20 06:00 [entrez] PHST- 2018/01/20 06:00 [pubmed] PHST- 2018/10/27 06:00 [medline] PHST- 2018/01/19 00:00 [pmc-release] AID - 1422666 [pii] AID - 10.1080/19932820.2017.1422666 [doi] PST - ppublish SO - Libyan J Med. 2018 Dec;13(1):1422666. doi: 10.1080/19932820.2017.1422666. PMID- 34789379 OWN - NLM STAT- MEDLINE DCOM- 20220125 LR - 20220125 IS - 1866-0452 (Electronic) IS - 1866-0452 (Linking) VI - 118I processed the file.I'm waiting for feedback tomorrow IP - 35-36 DP - 2021 Sep 6 TI - Raynaud's Phenomenon in the Breast Nipple Area. PG - 605 LID - arztebl.m2021.0282 [pii] LID - 10.3238/arztebl.m2021.0282 [doi] FAU - Koschinski-Möller, Beate AU - Koschinski-Möller B LA - eng PT - Comment PT - Journal Article PL - Germany TA - Dtsch Arztebl Int JT - Deutsches Arzteblatt international JID - 101475967 SB - IM CON - Dtsch Arztebl Int. 2021 Apr 09;118(Forthcoming):arztebl.m2021.0023. doi: 10.3238/arztebl.m2021.0023. PMID: 33632387 MH - Humans MH - *Nipples MH - *Raynaud Disease/diagnosis PMC - PMC8647287 EDAT- 2021/11/19 06:00 MHDA- 2022/01/27 06:00 PMCR- 2021/09/01 CRDT- 2021/11/18 05:55 PHST- 2021/11/18 05:55 [entrez] PHST- 2021/11/19 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] PHST- 2021/09/01 00:00 [pmc-release] AID - arztebl.m2021.0282 [pii] AID - 10.3238/arztebl.m2021.0282 [doi] PST - ppublish SO - Dtsch Arztebl Int. 2021 Sep 6;118I processed the file.I'm waiting for feedback tomorrow(35-36):605. doi: 10.3238/arztebl.m2021.0282. PMID- 25826330 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20181023 IS - 0717-6384 (Electronic) IS - 0717-6384 (Linking) VI - 15 IP - 2 DP - 2015 Mar 9 TI - Is iloprost effective in secondary Raynaud's phenomenon? PG - e6082 LID - 10.5867/medwave.2015.02.6082 [doi] AB - Patients with systemic sclerosis frequently have Raynaud's phenomenon and digital ischemic ulcers. Iloprost, a synthetic prostacyclin analogue, may be effective in these cases. Searching in Epistemonikos database, which is maintained by screening 20 databases, we identified three systematic reviews including seven randomized trials. We combined the evidence using meta-analysis and generated a summary of findings table following the GRADE approach. We concluded iloprost may lead to little or no difference in the frequency or severity of secondary Raynaud, and it is associated to adverse effects and important costs. FAU - Lustig, Nicole AU - Lustig N AD - Programa de Salud Basada en Evidencia, Facultad de Medicina, Pontificia Universidad Católica de Chile; Departamento de Medicina Interna, Facultad de Medicina, Pontificia Universidad Católica de Chile. Address: Facultad de Medicina Pontificia Universidad Católica de Chile, Lira 63, Santiago Centro, Chile. Email: radagabriel@epistemonikos.org. FAU - Rada, Gabriel AU - Rada G AD - Programa de Salud Basada en Evidencia, Facultad de Medicina, Pontificia Universidad Católica de Chile; Departamento de Medicina Interna, Facultad de Medicina, Pontificia Universidad Católica de Chil; GRADE working group; The Cochrane Collaboration; Fundación Epistemonikos. LA - eng LA - spa PT - Journal Article PT - Meta-Analysis DEP - 20150309 PL - Chile TA - Medwave JT - Medwave JID - 101581949 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Databases, Factual MH - Humans MH - Iloprost/adverse effects/*therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/*complications/drug therapy MH - Vasodilator Agents/adverse effects/therapeutic use EDAT- 2015/04/01 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/04/01 06:00 PHST- 2015/04/01 06:00 [entrez] PHST- 2015/04/01 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - e6082 [pii] AID - 10.5867/medwave.2015.02.6082 [doi] PST - epublish SO - Medwave. 2015 Mar 9;15(2):e6082. doi: 10.5867/medwave.2015.02.6082. PMID- 33325078 OWN - NLM STAT- MEDLINE DCOM- 20210621 LR - 20210621 IS - 1447-0756 (Electronic) IS - 1341-8076 (Linking) VI - 47 IP - 2 DP - 2021 Feb TI - Raynaud's phenomenon as a presenting manifestation of ovarian cancer: A case report. PG - 855-859 LID - 10.1111/jog.14609 [doi] AB - Raynaud's phenomenon (RP) is characterized by episodes of vasospasm affecting the hands and feet. Paraneoplastic RP, as a single presenting symptom is rarely seen in cases of ovarian cancer (OC), and thus may lead to misdiagnosis. We present a case of paraneoplastic RP in a patient with high-grade serous OC. A 66-year-old female presented with dyspnea and bilateral peripheral cyanosis involving her fingers. CA125 was elevated (423 U/mL). CT revealed a pleural effusion on the left side, suspicious omental lesions and ascites. Omental biopsy and pleural cytology demonstrated high-grade serous OC. Neoadjuvant chemotherapy (carboplatin/paclitaxel) resulted in objective improvement in finger ischemia and complete regression of vasospastic features. However, the patient's disease was refractory to post-surgical treatment and eventually she deceased of multiple organ failure. To conclude, RP may be a presenting symptom of OC. It is important to determine the underlying disease and develop an effective treatment strategy. CI - © 2020 Japan Society of Obstetrics and Gynecology. FAU - Matanes, Emad AU - Matanes E AD - Department of Obstetrics and Gynecology, Rambam Health Care Campus, Haifa, Israel. AD - Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. FAU - Boulus, Sari AU - Boulus S AD - Department of Obstetrics and Gynecology, Rambam Health Care Campus, Haifa, Israel. AD - Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. FAU - Lauterbach, Roy AU - Lauterbach R AD - Department of Obstetrics and Gynecology, Rambam Health Care Campus, Haifa, Israel. AD - Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. FAU - Matanis, Jawad AU - Matanis J AD - Department of Anesthesiology, Rambam Health Care Campus, Haifa, Israel. FAU - Reiss, Ari AU - Reiss A AD - Department of Obstetrics and Gynecology, Rambam Health Care Campus, Haifa, Israel. AD - Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. FAU - Amit, Amnon AU - Amit A AD - Department of Obstetrics and Gynecology, Rambam Health Care Campus, Haifa, Israel. AD - Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. LA - eng PT - Case Reports PT - Journal Article DEP - 20201215 PL - Australia TA - J Obstet Gynaecol Res JT - The journal of obstetrics and gynaecology research JID - 9612761 SB - IM MH - Aged MH - Carcinoma, Ovarian Epithelial MH - Female MH - Fingers MH - Humans MH - Ischemia MH - *Ovarian Neoplasms/complications/diagnosis MH - *Raynaud Disease/diagnosis/etiology OTO - NOTNLM OT - Raynaud's phenomenon OT - Raynaud's phenomenonchemotherapy OT - gynecologic oncology OT - ovarian cancer OT - paraneoplastic EDAT- 2020/12/17 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/12/16 05:40 PHST- 2020/05/07 00:00 [received] PHST- 2020/09/19 00:00 [revised] PHST- 2020/12/07 00:00 [accepted] PHST- 2020/12/17 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/12/16 05:40 [entrez] AID - 10.1111/jog.14609 [doi] PST - ppublish SO - J Obstet Gynaecol Res. 2021 Feb;47(2):855-859. doi: 10.1111/jog.14609. Epub 2020 Dec 15. PMID- 41076468 OWN - NLM STAT- MEDLINE DCOM- 20251113 LR - 20251113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 12 DP - 2025 Dec TI - Critical discrepancies in oximetry among patients with Raynaud's phenomenon. PG - 4995-5002 LID - 10.1007/s10067-025-07738-9 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is associated with a high frequency of pulmonary complications, making oxygen saturation critical for monitoring purposes. However, there are significant difficulties, such as skin thickening and edema, digital ulcers, and Raynaud's phenomenon (RP), which may lead to inaccurate and unreliable conventional digital readings. The objective of the present study is to assess the concordance of oxygen saturation at different anatomical sites, including the forehead, which has been proven to be unaffected by vascular changes, such as RP. METHODS: Patients with SSc and RP were included. Pulse oximetry was recorded for each patient using a transmittance pulse oximeter on the index finger and pinna; a reflectance pulse oximeter was also used in the supraciliary region and on the dorsum of the hand. These measurements were obtained both with and without RP, and the concordance between them was subsequently determined using the Bland-Altman plot and Lin's coefficient. Additional potential relationships with other factors that may alter the measurement and the rate of unsuccessful measurements at each site were also analyzed. RESULTS: Sixty-nine patients were included. One third (37%) had interstitial lung disease. Measurements taken in the presence of RP on the fingers, dorsum of the hand, and pinna showed mean oxygenation values of 5%, 7%, and 1.5% lower than measurements taken on the forehead. Lin's coefficients were between 0.1 and 0.2, indicating low concordance. No variations were observed for finger edema and skin thickness. According to the heteroscedasticity analysis, these differences are more marked when saturation levels fall below 93%. Furthermore, 14% of patients had unsatisfactory measurements on the fingers in the presence of RP, although none were observed on the forehead, associated with diffuse skin disease (OR 11.6, 95% CI 1.46-9.1, p = 0.02). CONCLUSION: Concordance, measured using different methods, is low across other sites, with greater significance at lower oximetry levels. This affects the study and follow-up of patients with pulmonary complications, such as interstitial lung disease or pulmonary hypertension. Forehead measurements are not affected by vasoconstriction induced by RP and can be an excellent option for patient follow-up. Key Points • Oximetry values measured on fingers affected by Raynaud's phenomenon differ significantly (5%) from those measured on the forehead. • The difference between finger oximetry measurements varies heterogeneously and is significantly greater when values are below 93%. • In patients with Raynaud's phenomenon, finger oximetry is often not feasible due to low perfusion (14%), a limitation not observed with forehead measurements. • Forehead oximetry is not affected by Raynaud's phenomenon and is therefore recommended in patients with this condition. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Ramos, Roberto AU - Ramos R AD - Departamento de Medicina Interna, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Bustos, Lina María AU - Bustos LM AD - Departamento de Medicina Interna, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Peña, Juan Sebastián AU - Peña JS AD - Departamento de Medicina Interna, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Medina, Juan Pablo AU - Medina JP AD - Departamento de Medicina Interna, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Recalde, Julia AU - Recalde J AD - Departamento de Reumatología, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Pérez, Manuel AU - Pérez M AD - Departamento de Reumatología, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Correa, Alejandro AU - Correa A AD - Departamento de Reumatología, Universidad Nacional de Colombia, Bogotá, Colombia. FAU - Molina, Sebastián AU - Molina S AD - Departamento de Reumatología, Universidad Nacional de Colombia, Bogotá, Colombia. AD - Departamento de Reumatología, Hospital Universitario Nacional, Bogotá, Colombia. FAU - Cajas, Luis Javier AU - Cajas LJ AUID- ORCID: 0000-0002-4912-7657 AD - Departamento de Reumatología, Universidad Nacional de Colombia, Bogotá, Colombia. ljaviercs.77@hotmail.com. AD - Departamento de Reumatología, Hospital Universitario Nacional, Bogotá, Colombia. ljaviercs.77@hotmail.com. LA - eng PT - Journal Article DEP - 20251012 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - S88TT14065 (Oxygen) SB - IM MH - Humans MH - *Raynaud Disease/complications/diagnosis/blood MH - *Oximetry/methods MH - Female MH - Male MH - Middle Aged MH - Aged MH - Adult MH - *Scleroderma, Systemic/complications MH - Fingers/blood supply MH - Oxygen Saturation MH - Oxygen/blood OTO - NOTNLM OT - Oximetry OT - Raynaud disease OT - Systemic sclerosis COIS- Declarations. Ethics approval and consent to participate: This study received approval from an ethics committee in compliance with legal requirements. All patients provided informed consent for participation. Consent for publication: Not applicable, only anonymized data will be published; no identifying information will be disclosed. Disclosures: None. EDAT- 2025/10/12 00:34 MHDA- 2025/11/13 06:29 CRDT- 2025/10/11 23:14 PHST- 2025/06/10 00:00 [received] PHST- 2025/09/29 00:00 [accepted] PHST- 2025/09/26 00:00 [revised] PHST- 2025/11/13 06:29 [medline] PHST- 2025/10/12 00:34 [pubmed] PHST- 2025/10/11 23:14 [entrez] AID - 10.1007/s10067-025-07738-9 [pii] AID - 10.1007/s10067-025-07738-9 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Dec;44(12):4995-5002. doi: 10.1007/s10067-025-07738-9. Epub 2025 Oct 12. PMID- 34559483 OWN - NLM STAT- MEDLINE DCOM- 20211025 LR - 20211025 IS - 2155-7780 (Electronic) IS - 2155-7780 (Linking) VI - 23 IP - 5 DP - 2021 Sep 23 TI - Rare Side Effects of Stimulants: Raynaud's Phenomenon. LID - 20l02857 [pii] LID - 10.4088/PCC.20l02857 [doi] FAU - Gupta, Mayank AU - Gupta M AD - Clarion Psychiatric Center, Clarion Pennsylvania. AD - Corresponding author: Mayank Gupta, MD, Clarion Psychiatric Center, 2 Hospital Dr, Clarion, PA 16214 (mayank6nov@gmail.com). LA - eng PT - Journal Article DEP - 20210923 PL - United States TA - Prim Care Companion CNS Disord JT - The primary care companion for CNS disorders JID - 101547532 RN - 0 (Central Nervous System Stimulants) SB - IM MH - *Central Nervous System Stimulants/adverse effects MH - *Drug-Related Side Effects and Adverse Reactions MH - Humans MH - *Raynaud Disease/chemically induced EDAT- 2021/09/25 06:00 MHDA- 2021/10/26 06:00 CRDT- 2021/09/24 12:35 PHST- 2021/09/24 12:35 [entrez] PHST- 2021/09/25 06:00 [pubmed] PHST- 2021/10/26 06:00 [medline] AID - 20l02857 [pii] AID - 10.4088/PCC.20l02857 [doi] PST - epublish SO - Prim Care Companion CNS Disord. 2021 Sep 23;23(5):20l02857. doi: 10.4088/PCC.20l02857. PMID- 21183379 OWN - NLM STAT- MEDLINE DCOM- 20111207 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 78 IP - 4 DP - 2011 Jul TI - Management of Raynaud's phenomenon and digital ulcers in systemic sclerosis. PG - 341-6 LID - 10.1016/j.jbspin.2010.11.005 [doi] AB - Raynaud's phenomenon (RP) and digital ulcers (DU) are the clinical manifestations of vasculopathy in systemic sclerosis. Both interfere with hand function and hold the possibility of severe complications, thus adversely influencing patients' quality of life. Managing RP and DU is often a challenge for the treating physician, who has to establish a treatment plan based upon knowledge of the current therapeutic options. The first step is to differentiate primary from secondary RP, where combining history and physical examination with diagnostic modalities, such as nailfold capillaroscopy, aids in reaching the correct diagnosis. Next a wide range of treatment options is offered nowadays, starting from first-line agents, as calcium channel blockers, to the more targeted-ones, like endothelin receptor antagonists. Research and clinical experience with each agent are reviewed in the text, as well as the combinations that more recently gain field in the treatment of DU. CI - Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Botzoris, Vasileios AU - Botzoris V AD - Department of Internal Medicine, Rheumatology Clinic, Medical School, University of Ioannina, 45110 Ioannina, Greece. FAU - Drosos, Alexandros A AU - Drosos AA LA - eng PT - Journal Article PT - Review DEP - 20101222 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Calcium Channel Blockers) RN - 0 (Cardiovascular Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Enzyme Inhibitors) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins I) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - Q326023R30 (Bosentan) SB - IM MH - Bosentan MH - Calcium Channel Blockers/therapeutic use MH - Cardiovascular Agents/*therapeutic use MH - Endothelin Receptor Antagonists MH - Enzyme Inhibitors/therapeutic use MH - Fingers MH - Humans MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Prostaglandins I/therapeutic use MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/complications/*drug therapy/physiopathology MH - Skin Ulcer/*drug therapy/etiology/physiopathology MH - Sulfonamides/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2010/12/25 06:00 MHDA- 2011/12/13 00:00 CRDT- 2010/12/25 06:00 PHST- 2010/09/07 00:00 [received] PHST- 2010/11/05 00:00 [accepted] PHST- 2010/12/25 06:00 [entrez] PHST- 2010/12/25 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - S1297-319X(10)00295-2 [pii] AID - 10.1016/j.jbspin.2010.11.005 [doi] PST - ppublish SO - Joint Bone Spine. 2011 Jul;78(4):341-6. doi: 10.1016/j.jbspin.2010.11.005. Epub 2010 Dec 22. PMID- 37067070 OWN - NLM STAT- MEDLINE DCOM- 20230420 LR - 20241122 IS - 2164-554X (Electronic) IS - 2164-5515 (Print) IS - 2164-5515 (Linking) VI - 19 IP - 1 DP - 2023 Dec 31 TI - Temporal association between COVID-19 vaccination and Raynaud's phenomenon: A case series. PG - 2199653 LID - 10.1080/21645515.2023.2199653 [doi] LID - 2199653 AB - COVID-19 vaccine-related adverse events are mostly minor to moderate, and serious events are rare. Single cases of Raynaud's phenomenon (RP) in temporal proximity to COVID-19 vaccination have been reported. Demographic data, medical history, and detailed information regarding vaccination status and RP characteristics were obtained from patients with confirmed RP after COVID-19 vaccination. Fifteen participants reported the initial manifestation of RP, which occurred in 40% after the first, in 33% after the second, and in 27% after the third vaccination. RP development and occurrence of episodes were not linked to any specific vaccine type. New onset of disease was observed in 40% of the vaccinees after BNT162b2, in 33% after mRNA-1273, and in 27% after ChAdOx1 vaccination. Three out of four participants with preexisting RP prior to COVID-19 vaccination reported aggravation in frequency and intensity after immunization. Although COVID-19 vaccination is pivotal in controlling the pandemic, the observed temporal association between vaccine administration and RP occurrence warrants global activities to support pharmacovigilance for the detection of adverse reactions, one of which may include RP. FAU - Lisy, Marcus AU - Lisy M AUID- ORCID: 0000-0002-2473-9841 AD - Department of Dermatology, Medical University of Vienna, Vienna, Austria. FAU - Urban, Nikolaus AU - Urban N AUID- ORCID: 0000-0002-4052-1092 AD - Department of Dermatology, Medical University of Vienna, Vienna, Austria. FAU - Brunner-Ziegler, Sophie AU - Brunner-Ziegler S AD - Division of Angiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Weber, Benedikt AU - Weber B AUID- ORCID: 0000-0002-7217-4590 AD - Department of Dermatology, Medical University of Vienna, Vienna, Austria. FAU - Bauer, Wolfgang M AU - Bauer WM AUID- ORCID: 0000-0002-0155-1176 AD - Department of Dermatology, Medical University of Vienna, Vienna, Austria. FAU - Dassler, Eva AU - Dassler E AD - Division of Angiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Koppensteiner, Renate AU - Koppensteiner R AD - Division of Angiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Handisurya, Alessandra AU - Handisurya A AUID- ORCID: 0000-0001-9823-7644 AD - Department of Dermatology, Medical University of Vienna, Vienna, Austria. LA - eng PT - Journal Article DEP - 20230417 PL - United States TA - Hum Vaccin Immunother JT - Human vaccines & immunotherapeutics JID - 101572652 RN - 0 (BNT162 Vaccine) RN - 0 (COVID-19 Vaccines) SB - IM CIN - Hum Vaccin Immunother. 2023 Dec 15;19(3):2295077. doi: 10.1080/21645515.2023.2295077. PMID: 38109761 MH - Humans MH - BNT162 Vaccine MH - *COVID-19/prevention & control MH - *COVID-19 Vaccines/adverse effects MH - *Raynaud Disease/diagnosis MH - Vaccination/adverse effects PMC - PMC10116941 OTO - NOTNLM OT - BNT162b2 vaccine OT - COVID-19 vaccination OT - COVID-19 vaccine OT - ChAdOx1 vaccine OT - Raynaud’s phenomenon OT - Raynaud’s syndrome OT - adverse effect OT - mRNA-1273 vaccine COIS- No potential conflict of interest was reported by the author(s). EDAT- 2023/04/18 06:00 MHDA- 2023/04/20 10:17 PMCR- 2023/04/17 CRDT- 2023/04/17 07:32 PHST- 2023/04/20 10:17 [medline] PHST- 2023/04/18 06:00 [pubmed] PHST- 2023/04/17 07:32 [entrez] PHST- 2023/04/17 00:00 [pmc-release] AID - 2199653 [pii] AID - 10.1080/21645515.2023.2199653 [doi] PST - ppublish SO - Hum Vaccin Immunother. 2023 Dec 31;19(1):2199653. doi: 10.1080/21645515.2023.2199653. Epub 2023 Apr 17. PMID- 36316060 OWN - NLM STAT- MEDLINE DCOM- 20230118 LR - 20230412 IS - 1001-9294 (Print) IS - 1001-9294 (Linking) VI - 37 IP - 4 DP - 2022 Dec 31 TI - Multidisciplinary Treatment for Severe Secondary Raynaud's Phenomenon: A Case Report. PG - 353-358 LID - 10.24920/004078 [doi] AB - Raynaud's phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers. Secondary Raynaud's phenomenon is often more severe and could even lead to finger ulceration, making it particularly complicated to treat. We describe a case of severe Raynaud's phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman. The patient was also diagnosed with hypothyroidism, epilepsy, and secondary soft tissue infection of the right middle and ring fingers. After careful multidisciplinary consultation and discussion, the patient received vasodilation, anticoagulation, thyroxine supplementation, stellate ganglion block, hyperbaric oxygen therapy and debridement. The patient responded well to the medication, avoiding amputation or obviously dysfunction. Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud's phenomenon and could improve the prognosis and satisfaction of patient effectively. FAU - Leng, Cui-Bo AU - Leng CB AD - Department of Anesthesiology, Affiliated Qingdao Central Hospital of Qingdao University ;Second Affiliated Hospital of Medical College of Qingdao University, Qingdao, Shandong 266042, China. FAU - Lin, Guan-Jun AU - Lin GJ AD - Department of Cardiovascular Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong 266000, China. FAU - Cao, Hong AU - Cao H AD - Department of Anesthesiology, Affiliated Qingdao Central Hospital of Qingdao University ;Second Affiliated Hospital of Medical College of Qingdao University, Qingdao, Shandong 266042, China. FAU - Liu, Zi-Jia AU - Liu ZJ AD - Department of Anesthesiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences ;Peking Union Medical College, Beijing 100730, China. LA - eng PT - Case Reports PT - Journal Article PL - China TA - Chin Med Sci J JT - Chinese medical sciences journal = Chung-kuo i hsueh k'o hsueh tsa chih JID - 9112559 SB - IM MH - Female MH - Humans MH - Aged MH - *Hypothyroidism/complications MH - *Raynaud Disease/etiology/therapy/diagnosis OTO - NOTNLM OT - multidisciplinary OT - secondary Raynaud’s phenomenon OT - stellate ganglion block EDAT- 2022/11/01 06:00 MHDA- 2023/01/19 06:00 CRDT- 2022/10/31 21:03 PHST- 2022/11/01 06:00 [pubmed] PHST- 2023/01/19 06:00 [medline] PHST- 2022/10/31 21:03 [entrez] AID - 4078 [pii] AID - 10.24920/004078 [doi] PST - ppublish SO - Chin Med Sci J. 2022 Dec 31;37(4):353-358. doi: 10.24920/004078. PMID- 26400642 OWN - NLM STAT- MEDLINE DCOM- 20160831 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 34 IP - 12 DP - 2015 Dec TI - Primary care assessment of capillaroscopy abnormalities in patients with Raynaud's phenomenon. PG - 2135-40 LID - 10.1007/s10067-015-3062-3 [doi] AB - Raynaud's phenomenon is a clinical symptom that can commonly present to a primary care provider or generalist. Proper identification of an underlying connective tissue disease in a patient with Raynaud's could allow for the prevention of possible critical digital ischemia. Capillaroscopy is a tool which can identify abnormalities associated with connective tissue disease. Patients presenting with a complaint of Raynaud's phenomenon were assessed with capillaroscopy. In twenty consecutive Raynaud patients, 8 digits were assessed by a ×200 magnification dermatoscope and an image was obtained. Each image was assessed for the following abnormalities: drop-out (<9 capillaries in 1 mm); microhemorrhage; dilated loops; and neoangiogenesis. These 160 images were then shown to 20 primary care physicians, who assessed these same abnormalities. The interrater reliability, a measure of agreement, of individual primary care providers with the expert provider was assessed using kappa statistics. Three raters had slight agreement (in the range 0 to 0.20), one rater had fair agreement (0.21 to 0.40), 11 raters had moderate agreement (0.41 to 0.60), five raters had substantial agreement (0.61 to 0.80), and no rater had almost perfect agreement (0.81 to 1.00) (14). The total agreement from the 20 primary care providers (n = 3,156) was moderate (Κ = 0.50, 95 % CI 0.49, 0.55). For the four providers with the slight to fair interrater reliabilities, the most common disagreement was providing a positive diagnosis when the expert rater diagnosed the digit negative. Ten of the twenty primary care providers provided at least one additional diagnosis following an abnormal diagnosis (n = 35 digits or 35 % of the 1556 abnormal ratings by the primary care providers). The four providers with the poorest interrater reliabilities were not among the ten providers who participated in making these additional specific diagnoses. These providers achieved the moderate agreement with the expert provider for diagnoses of microhemorrhage (Κ = 0.64, 95 % CI 0.57, 0.70), but fair agreement with the expert provider for diagnoses of dilated (Κ = 0.27, 95 % CI 0.20, 0.34) and neoangiogenesis (Κ = 0.22, 95 %CI 0.13, 0.31). Capillaroscopy is a potentially contributive clinical exam skill that could assist primary care providers and generalists in identifying and qualifying changes associated with the common presentation of Raynaud's disease. However, formal training is needed to ensure accuracy and reproducibility. Furthermore, training and scoring systems should address time constraints of busy primary care practitioners. FAU - Overbury, Rebecca AU - Overbury R AD - Departments of Internal Medicine and Pediatrics, University of Utah, Salt Lake City, UT, USA. FAU - Murtaugh, Maureen A AU - Murtaugh MA AD - Department of Internal Medicine, Division of Epidemiology, Salt Lake Veterans Affair Medical Center, University of Utah, Salt Lake City, UT, USA. FAU - Fischer, Aryeh AU - Fischer A AD - Division of Rheumatology, University of Colorado School of Medicine, Denver, CO, USA. FAU - Frech, Tracy M AU - Frech TM AD - Department of Internal Medicine, Division of Rheumatology, Salt Lake Veterans Affair Medical Center, University of Utah, Salt Lake City, UT, USA. Tracy.Frech@hsc.utah.edu. LA - eng GR - 5UL1TR001067-02/TR/NCATS NIH HHS/United States GR - 8UL1TR000105/TR/NCATS NIH HHS/United States GR - UL1RR025764/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150923 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Capillaries/*pathology MH - Dilatation, Pathologic/pathology MH - Female MH - Hemorrhage/*pathology MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Neovascularization, Pathologic/*pathology MH - Observer Variation MH - *Physicians, Primary Care MH - Raynaud Disease/*diagnosis/pathology MH - Reproducibility of Results MH - Sensitivity and Specificity OTO - NOTNLM OT - Diagnostic tests OT - Methodology OT - Raynaud’s syndrome OT - Rheumatic diseases OT - Scleroderma OT - Systemic sclerosis EDAT- 2015/09/25 06:00 MHDA- 2016/09/01 06:00 CRDT- 2015/09/25 06:00 PHST- 2015/03/09 00:00 [received] PHST- 2015/08/18 00:00 [accepted] PHST- 2015/08/14 00:00 [revised] PHST- 2015/09/25 06:00 [entrez] PHST- 2015/09/25 06:00 [pubmed] PHST- 2016/09/01 06:00 [medline] AID - 10.1007/s10067-015-3062-3 [pii] AID - 10.1007/s10067-015-3062-3 [doi] PST - ppublish SO - Clin Rheumatol. 2015 Dec;34(12):2135-40. doi: 10.1007/s10067-015-3062-3. Epub 2015 Sep 23. PMID- 29068146 OWN - NLM STAT- MEDLINE DCOM- 20190916 LR - 20190916 IS - 1365-2796 (Electronic) IS - 0954-6820 (Linking) VI - 283 IP - 3 DP - 2018 Mar TI - Chronic inflammation predicts long-term mortality in patients with Raynaud's phenomenon. PG - 293-302 LID - 10.1111/joim.12705 [doi] AB - BACKGROUND: Subclinical chronic inflammation could be the driving force behind the recently revealed association between abnormal nailfold capillaries as well as autoantibodies and long-term mortality in patients with incipient Raynaud's phenomenon. Whether laboratory markers that reflect a chronic inflammatory process are directly related to mortality in Raynaud's phenomenon is not known. METHODS: In total, 2958 patients with incipient Raynaud's phenomenon without previously known connective tissue disease (CTD) were enrolled. At their initial presentation, laboratory tests for C-reactive protein (CRP), leucocytes, fibrinogen and the haemoglobin concentration were obtained. In addition, nailfold capillaries and antinuclear antibodies (ANA) were assessed. Patients' mortality was recorded through a median follow-up period of 9.3 years. RESULTS: Baseline CRP, fibrinogen and haemoglobin concentration were associated with long-term mortality in an individual analysis of patients with incipient Raynaud's phenomenon. In a multivariable model including patients' age, nailfold capillaries and ANA, a low haemoglobin concentration remained independently related to future mortality. Amongst potential predictors for mortality in patients with Raynaud's phenomenon, a low haemoglobin concentration was most strongly related to patients' mortality risk. CONCLUSION: In Raynaud's phenomenon, laboratory markers that can be attributed to a chronic inflammatory state independently yield prognostic information in addition to the presence of abnormal nailfold capillaries and ANA. Amongst all prognostic markers, the haemoglobin concentration is most strongly related to patients' mortality in Raynaud's phenomenon. CI - © 2017 The Association for the Publication of the Journal of Internal Medicine. FAU - Mueller, M AU - Mueller M AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Gschwandtner, M E AU - Gschwandtner ME AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Gamper, J AU - Gamper J AD - Center for Medical Statistics, Informatics and Intelligent Systems, Medical University of Vienna, Vienna, Austria. FAU - Giurgea, G-A AU - Giurgea GA AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Kiener, H P AU - Kiener HP AD - Division of Rheumatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria. FAU - Perkmann, T AU - Perkmann T AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Koppensteiner, R AU - Koppensteiner R AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. FAU - Schlager, O AU - Schlager O AUID- ORCID: 0000-0001-6934-5355 AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Vienna, Austria. LA - eng PT - Journal Article DEP - 20171114 PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Adult MH - Austria/epidemiology MH - Autoantibodies/*blood MH - Biomarkers/blood MH - C-Reactive Protein/*metabolism MH - Cause of Death/trends MH - Female MH - Follow-Up Studies MH - *Forecasting MH - Humans MH - Inflammation/*blood/immunology/mortality MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/blood/immunology/*mortality MH - Retrospective Studies MH - Survival Rate/trends OTO - NOTNLM OT - Raynaud OT - epidemiology OT - inflammation OT - microcirculation OT - prognosis EDAT- 2017/10/27 06:00 MHDA- 2019/09/17 06:00 CRDT- 2017/10/26 06:00 PHST- 2017/10/27 06:00 [pubmed] PHST- 2019/09/17 06:00 [medline] PHST- 2017/10/26 06:00 [entrez] AID - 10.1111/joim.12705 [doi] PST - ppublish SO - J Intern Med. 2018 Mar;283(3):293-302. doi: 10.1111/joim.12705. Epub 2017 Nov 14. PMID- 28641553 OWN - NLM STAT- MEDLINE DCOM- 20180925 LR - 20180925 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 14 IP - 1 DP - 2018 Apr 20 TI - Capillaroscopy in Routine Diagnostics: Potentials and Limitations. PG - 5-11 LID - 10.2174/1573397113666170615084229 [doi] AB - BACKGROUND: Nailfold capillaroscopy is a safe and useful investigational tool that allows an early detection and a qualitative description of the microvascular abnormalities in patients with Raynaud's phenomenon secondary to scleroderma-spectrum disorders. Nowadays, the role of capillaroscopy in the diagnosis of systemic sclerosis is well known. Capillaroscopy has been included in the new 2013 classification criteria for systemic sclerosis and is considered a key investigation in the very early phases of the disease. CONCLUSION: Because of its potential value in monitoring disease progression and treatment response, nailfold capillaroscopy may also have a role in the management of overt systemic sclerosis. RESULTS: Its application in scleroderma-spectrum disorders in which a microvascular component is suspected may also provide new insights into their pathophysiology. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Pini, Milano, Dept. of Clinical Sciences & Community Health, Universita degli Studi di Milano, Milano, Italy. FAU - Smith, Vanessa AU - Smith V AD - University of Ghent, Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genova, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genova, Italy. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Humans MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/diagnostic imaging MH - Scleroderma, Localized/diagnostic imaging MH - Scleroderma, Systemic/*diagnostic imaging OTO - NOTNLM OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - autoantibodies OT - biomarkers OT - classification criteria OT - connective tissue diseases OT - differential diagnosis OT - imaging OT - microcirculation OT - systemic sclerosis OT - systemic sclerosis. EDAT- 2017/06/24 06:00 MHDA- 2018/09/27 06:00 CRDT- 2017/06/24 06:00 PHST- 2017/02/17 00:00 [received] PHST- 2017/02/17 00:00 [revised] PHST- 2017/05/16 00:00 [accepted] PHST- 2017/06/24 06:00 [pubmed] PHST- 2018/09/27 06:00 [medline] PHST- 2017/06/24 06:00 [entrez] AID - CRR-EPUB-84105 [pii] AID - 10.2174/1573397113666170615084229 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2018 Apr 20;14(1):5-11. doi: 10.2174/1573397113666170615084229. PMID- 30003585 OWN - NLM STAT- MEDLINE DCOM- 20191011 LR - 20210109 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 84 IP - 10 DP - 2018 Oct TI - Proton pump inhibitors and Raynaud's phenomenon: is there a link? PG - 2443-2444 LID - 10.1111/bcp.13697 [doi] FAU - Khouri, Charles AU - Khouri C AUID- ORCID: 0000-0002-8427-8573 AD - Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France. AD - Clinical Pharmacology Department, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2 Laboratory, Grenoble Alpes University, Grenoble, France. FAU - Revol, Bruno AU - Revol B AUID- ORCID: 0000-0003-2931-2940 AD - Pharmacovigilance Unit, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2 Laboratory, Grenoble Alpes University, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Clinical Pharmacology Department, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2 Laboratory, Grenoble Alpes University, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AUID- ORCID: 0000-0003-4475-1626 AD - Clinical Pharmacology Department, Grenoble Alpes University Hospital, Grenoble, France. AD - HP2 Laboratory, Grenoble Alpes University, Grenoble, France. LA - eng PT - Letter DEP - 20180712 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - 0 (Histamine H2 Antagonists) RN - 0 (Proton Pump Inhibitors) RN - 63CV1GEK3Y (N,N-dimethylarginine) RN - 94ZLA3W45F (Arginine) RN - EC 3.5.- (Amidohydrolases) RN - EC 3.5.3.18 (dimethylargininase) SB - IM MH - Adverse Drug Reaction Reporting Systems/statistics & numerical data MH - Amidohydrolases/antagonists & inhibitors/metabolism MH - Arginine/analogs & derivatives/blood/metabolism MH - Gastroesophageal Reflux/drug therapy MH - Histamine H2 Antagonists/*adverse effects MH - Humans MH - Pharmacovigilance MH - Proton Pump Inhibitors/*adverse effects MH - Raynaud Disease/blood/chemically induced/*epidemiology PMC - PMC6138507 OTO - NOTNLM OT - Raynaud's phenomenon OT - drug safety OT - proton pump inhibitors EDAT- 2018/07/14 06:00 MHDA- 2019/10/12 06:00 PMCR- 2019/10/01 CRDT- 2018/07/14 06:00 PHST- 2018/03/09 00:00 [received] PHST- 2018/06/15 00:00 [revised] PHST- 2018/06/18 00:00 [accepted] PHST- 2018/07/14 06:00 [pubmed] PHST- 2019/10/12 06:00 [medline] PHST- 2018/07/14 06:00 [entrez] PHST- 2019/10/01 00:00 [pmc-release] AID - BCP13697 [pii] AID - 10.1111/bcp.13697 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2018 Oct;84(10):2443-2444. doi: 10.1111/bcp.13697. Epub 2018 Jul 12. PMID- 34315746 OWN - NLM STAT- MEDLINE DCOM- 20210729 LR - 20230728 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 14 IP - 7 DP - 2021 Jul 27 TI - I am blue: a toddler's Raynaud's phenomenon. LID - 10.1136/bcr-2021-243848 [doi] LID - e243848 AB - Raynaud's phenomenon (RP) is a well-known disorder of self-limiting paroxysmal vasospasms occurring in small arteries of the digits, in the order of skin pallor (white), followed by cyanosis (blue), ending with hyperemia (red). These designative triphasic colour changes with exposure to cold, or emotional response is diagnostic in adults. RP is a very rare phenomenon in the young paediatric population as noted by Nigrovic et al with 123 patients <19 year old in a large children's centre over 10 years and only 4 patients being <2 years old, with 69% of these being primary RP. To our knowledge, this is the youngest documented case of Raynaud's disease that has not required treatment. CI - © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Jeong, Yae Sul AU - Jeong YS AUID- ORCID: 0000-0003-3885-6069 AD - Pediatric Emergency Medicine, Beaumont Health, Royal Oak, Michigan, USA hazelline88@gmail.com. FAU - Menoch, Margaret AU - Menoch M AD - Pediatric Emergency Medicine, Beaumont Health, Royal Oak, Michigan, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210727 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Adult MH - Child MH - Child, Preschool MH - Humans MH - *Raynaud Disease/diagnosis MH - Young Adult PMC - PMC8317084 OTO - NOTNLM OT - emergency medicine OT - paediatrics OT - rheumatology OT - vasculitis COIS- Competing interests: None declared. EDAT- 2021/07/29 06:00 MHDA- 2021/07/30 06:00 PMCR- 2023/07/27 CRDT- 2021/07/28 05:45 PHST- 2021/07/28 05:45 [entrez] PHST- 2021/07/29 06:00 [pubmed] PHST- 2021/07/30 06:00 [medline] PHST- 2023/07/27 00:00 [pmc-release] AID - 14/7/e243848 [pii] AID - bcr-2021-243848 [pii] AID - 10.1136/bcr-2021-243848 [doi] PST - epublish SO - BMJ Case Rep. 2021 Jul 27;14(7):e243848. doi: 10.1136/bcr-2021-243848. PMID- 17595712 OWN - NLM STAT- MEDLINE DCOM- 20070628 LR - 20070525 IS - 0741-6245 (Print) IS - 0741-6245 (Linking) VI - 25 IP - 5 DP - 2007 May TI - Raynaud's disease. Cold hands, slowed blood flow. PG - 7 LA - eng PT - Journal Article PL - United States TA - Mayo Clin Health Lett JT - Mayo Clinic health letter (English ed.) JID - 8507508 MH - Female MH - Humans MH - Life Style MH - Male MH - Raynaud Disease/*drug therapy/physiopathology/prevention & control MH - Regional Blood Flow/physiology MH - Sex Factors EDAT- 2007/06/29 09:00 MHDA- 2007/06/29 09:01 CRDT- 2007/06/29 09:00 PHST- 2007/06/29 09:00 [pubmed] PHST- 2007/06/29 09:01 [medline] PHST- 2007/06/29 09:00 [entrez] PST - ppublish SO - Mayo Clin Health Lett. 2007 May;25(5):7. PMID- 32327024 OWN - NLM STAT- MEDLINE DCOM- 20210111 LR - 20210111 IS - 2375-6322 (Electronic) IS - 2375-6314 (Linking) VI - 91 IP - 5 DP - 2020 May 1 TI - You're the Flight Surgeon. PG - 459-461 LID - 10.3357/AMHP.5575.2020 [doi] AB - Warneke JA, Pavelites JJ. You're the flight surgeon: Raynaud's phenomenon/hand arm vibration syndrome. Aerosp Med Hum Perform. 2020; 91(5):459-461. LA - eng PT - Journal Article PL - United States TA - Aerosp Med Hum Perform JT - Aerospace medicine and human performance JID - 101654770 SB - IM MH - Aerospace Medicine MH - *Carpal Tunnel Syndrome MH - Hand/*physiopathology MH - Humans MH - Military Personnel MH - *Raynaud Disease EDAT- 2020/04/25 06:00 MHDA- 2021/01/12 06:00 CRDT- 2020/04/25 06:00 PHST- 2020/04/25 06:00 [entrez] PHST- 2020/04/25 06:00 [pubmed] PHST- 2021/01/12 06:00 [medline] AID - 10.3357/AMHP.5575.2020 [doi] PST - ppublish SO - Aerosp Med Hum Perform. 2020 May 1;91(5):459-461. doi: 10.3357/AMHP.5575.2020. PMID- 31847013 OWN - NLM STAT- MEDLINE DCOM- 20200417 LR - 20200417 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 144 IP - 25 DP - 2019 Dec TI - [Maurice Raynaud (1834-1881) and the Mystery of "Raynaud's Phanomenon"]. PG - 1778-1783 LID - 10.1055/a-0869-9899 [doi] AB - Maurice Raynaud first described color changes and symptoms of the fingers due to cold-induced vasospasm and restricted blood flow in his medical school thesis in 1862. Raynaud's phenomenon is common and exists as an uncomplicated primary Raynaud phenomenon and a Raynaud phenomenon secondary to underlying diseases and medication. Mechanisms contributing to altered vasoconstrictor activity are endothelial and not-endothelial. Cold-induced vasospasm is probably a thermoregulatory problem and effects are mediated by sympathetic activity and selective stimulation of alpha(2c)-adrenoreceptors. CI - © Georg Thieme Verlag KG Stuttgart · New York. FAU - Koehler, Ulrich AU - Koehler U FAU - Portig, Irene AU - Portig I FAU - Hildebrandt, Olaf AU - Hildebrandt O FAU - Koehler, Niklas Alexander AU - Koehler NA LA - ger PT - Historical Article PT - Journal Article TT - Maurice Raynaud (1834–1881) und das Mysterium „Raynaud-Phänomen“. DEP - 20191217 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Fingers/physiopathology MH - France MH - History, 19th Century MH - Humans MH - Male MH - Physicians/*history MH - *Raynaud Disease/history/physiopathology COIS- Die Autoren geben an, dass kein Interessenkonflikt besteht. EDAT- 2019/12/18 06:00 MHDA- 2020/04/18 06:00 CRDT- 2019/12/18 06:00 PHST- 2019/12/18 06:00 [entrez] PHST- 2019/12/18 06:00 [pubmed] PHST- 2020/04/18 06:00 [medline] AID - 10.1055/a-0869-9899 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2019 Dec;144(25):1778-1783. doi: 10.1055/a-0869-9899. Epub 2019 Dec 17. PMID- 23618525 OWN - NLM STAT- MEDLINE DCOM- 20130729 LR - 20130530 IS - 1097-6809 (Electronic) IS - 0741-5214 (Linking) VI - 57 IP - 6 DP - 2013 Jun TI - Current medical and surgical management of Raynaud's syndrome. PG - 1710-6 LID - S0741-5214(13)00598-3 [pii] LID - 10.1016/j.jvs.2013.03.012 [doi] AB - Raynaud's syndrome (RS) is characterized by episodic digital ischemia induced by cold or emotional stress. Pathophysiologic mechanisms include temporary vasospasm and fixed digital artery obstruction. A number of pharmacologic and invasive therapies have been studied to treat RS symptoms; however, there are no specific treatments that are currently approved by the U.S. Food and Drug Administration specifically for RS. Of the available pharmacologic agents, calcium-channel blockers remain the preferred initial treatment for vasospastic RS, although many vasodilators have been studied and found to be efficacious. Vasodilators are less effective in treating digital artery obstruction, and no treatments have been found to be universally beneficial, although the phosphodiesterase V inhibitors have been gaining in popularity. Invasive therapies may have a role in selective cases. In this review, the current evidence of treatment for RS is summarized. CI - Copyright © 2013 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved. FAU - Landry, Gregory J AU - Landry GJ AD - Division of Vascular Surgery, Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Ore 97239-3098, USA. landryg@ohsu.edu LA - eng PT - Journal Article PT - Review DEP - 20130423 PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Algorithms MH - Humans MH - Raynaud Disease/*drug therapy/*surgery EDAT- 2013/04/27 06:00 MHDA- 2013/07/31 06:00 CRDT- 2013/04/27 06:00 PHST- 2012/12/06 00:00 [received] PHST- 2013/02/28 00:00 [revised] PHST- 2013/03/09 00:00 [accepted] PHST- 2013/04/27 06:00 [entrez] PHST- 2013/04/27 06:00 [pubmed] PHST- 2013/07/31 06:00 [medline] AID - S0741-5214(13)00598-3 [pii] AID - 10.1016/j.jvs.2013.03.012 [doi] PST - ppublish SO - J Vasc Surg. 2013 Jun;57(6):1710-6. doi: 10.1016/j.jvs.2013.03.012. Epub 2013 Apr 23. PMID- 38128831 OWN - NLM STAT- MEDLINE DCOM- 20240325 LR - 20250408 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 90 IP - 4 DP - 2024 Apr TI - Depression in patients with Raynaud's phenomenon: A case-control study in the National Institutes of Health's All of Us Research Program. PG - 857-859 LID - S0190-9622(23)03387-X [pii] LID - 10.1016/j.jaad.2023.12.015 [doi] FAU - Chen, Gloria F AU - Chen GF AD - Yale School of Medicine, New Haven, Connecticut. FAU - Shaw, Katharina S AU - Shaw KS AD - Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. FAU - Xu, Suzanne AU - Xu S AD - Yale School of Medicine, New Haven, Connecticut. FAU - Hashemi, Kimberly B AU - Hashemi KB AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Castillo, Rochelle L AU - Castillo RL AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Vleugels, Ruth Ann AU - Vleugels RA AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Cohen, Jeffrey M AU - Cohen JM AD - Department of Dermatology, Yale School of Medicine, New Haven, Connecticut; Section of Biomedical Informatics and Data Science, Yale School of Medicine, New Haven, Connecticut. Electronic address: jeffrey.m.cohen@yale.edu. LA - eng GR - OT2 OD026549/OD/NIH HHS/United States GR - OT2 OD026554/OD/NIH HHS/United States GR - OT2 OD026557/OD/NIH HHS/United States GR - OT2 OD026556/OD/NIH HHS/United States GR - OT2 OD026550/OD/NIH HHS/United States GR - OT2 OD026552/OD/NIH HHS/United States GR - OT2 OD026553/OD/NIH HHS/United States GR - OT2 OD026548/OD/NIH HHS/United States GR - OT2 OD026551/OD/NIH HHS/United States GR - OT2 OD026555/OD/NIH HHS/United States GR - U2C OD023196/OD/NIH HHS/United States GR - U24 OD023121/OD/NIH HHS/United States GR - U24 OD023176/OD/NIH HHS/United States GR - U24 OD023163/OD/NIH HHS/United States GR - OT2 OD023205/OD/NIH HHS/United States GR - OT2 OD023206/OD/NIH HHS/United States GR - OT2 OD025277/OD/NIH HHS/United States GR - OT2 OD025315/OD/NIH HHS/United States GR - OT2 OD025337/OD/NIH HHS/United States GR - OT2 OD025276/OD/NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20231219 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - United States/epidemiology MH - Humans MH - Case-Control Studies MH - Depression MH - *Population Health MH - National Institutes of Health (U.S.) MH - *Raynaud Disease/complications/epidemiology OTO - NOTNLM OT - Raynaud’s phenomenon OT - depression OT - epidemiology COIS- Conflicts of interest Dr Cohen serves on a data and safety monitoring board (DSMB) for Advarra. The remaining authors have no conflicts of interest to declare. EDAT- 2023/12/22 00:42 MHDA- 2024/03/25 06:44 CRDT- 2023/12/21 19:29 PHST- 2023/08/11 00:00 [received] PHST- 2023/10/09 00:00 [revised] PHST- 2023/12/11 00:00 [accepted] PHST- 2024/03/25 06:44 [medline] PHST- 2023/12/22 00:42 [pubmed] PHST- 2023/12/21 19:29 [entrez] AID - S0190-9622(23)03387-X [pii] AID - 10.1016/j.jaad.2023.12.015 [doi] PST - ppublish SO - J Am Acad Dermatol. 2024 Apr;90(4):857-859. doi: 10.1016/j.jaad.2023.12.015. Epub 2023 Dec 19. PMID- 37706545 OWN - NLM STAT- MEDLINE DCOM- 20240706 LR - 20240828 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 34 IP - 4 DP - 2024 Jul 6 TI - Heating of the neck or elbows alleviates Raynaud's phenomenon but has different effects on different types of patients with systemic sclerosis. PG - 750-755 LID - 10.1093/mr/road091 [doi] AB - OBJECTIVES: We previously reported that heating of the neck or elbows alleviated Raynaud's phenomenon in patients with systemic sclerosis and upregulated capillary extension factor angiopoietin-1 (Angpt-1) in the fingertips. In this study, we investigated which cases responded better to the effect of heating of the neck or elbows. METHODS: The pre- to postheating change in the visual analogue scale (ΔVAS) for Raynaud's phenomenon was examined for correlation with age, disease duration, autoantibodies, disease types, corticosteroid dose, capillaroscopic nailfold capillary damage, fingertip Angpt-1 concentrations at baseline, and increased rate of Angpt-1 concentration. RESULTS: The ΔVAS for elbow heating correlated positively with the baseline Angpt-1 concentration, whereas opposite correlation was observed for neck heating. The other items did not significantly correlate with the ΔVAS; however, the ΔVAS for elbow heating tended to be larger in patients with advanced capillary damage, whereas an opposite trend was observed for neck heating. CONCLUSIONS: Elbow and neck heating alleviated Raynaud's phenomenon to a similar extent, but their mechanism was different. Heating of the elbows had a greater effect on patients with advanced capillary damage and lower fingertip Angpt-1 concentrations. CI - © Japan College of Rheumatology 2023. Published by Oxford University Press. FAU - Shima, Yoshihito AU - Shima Y AUID- ORCID: 0000-0002-2523-2938 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Watanabe, Akane AU - Watanabe A AUID- ORCID: 0000-0001-8451-9501 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Inoue, Nobuto AU - Inoue N AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Maruyama, Tetsuya AU - Maruyama T AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Kunitomo, Eiji AU - Kunitomo E AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Kumanogoh, Atsushi AU - Kumanogoh A AUID- ORCID: 0000-0003-4749-7117 AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. LA - eng PT - Journal Article PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Angiopoietin-1) SB - IM MH - Humans MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/complications/drug therapy MH - Female MH - Middle Aged MH - Male MH - Adult MH - Aged MH - *Neck MH - Elbow MH - Angiopoietin-1 MH - Treatment Outcome OTO - NOTNLM OT - Angiopoietin-1 OT - Raynaud’s phenomenon OT - capillary OT - heating OT - systemic sclerosis EDAT- 2023/09/14 12:41 MHDA- 2024/07/06 20:43 CRDT- 2023/09/14 08:31 PHST- 2023/06/27 00:00 [received] PHST- 2023/09/06 00:00 [accepted] PHST- 2024/07/06 20:43 [medline] PHST- 2023/09/14 12:41 [pubmed] PHST- 2023/09/14 08:31 [entrez] AID - 7273664 [pii] AID - 10.1093/mr/road091 [doi] PST - ppublish SO - Mod Rheumatol. 2024 Jul 6;34(4):750-755. doi: 10.1093/mr/road091. PMID- 24034762 OWN - NLM STAT- MEDLINE DCOM- 20151112 LR - 20161021 IS - 1578-8865 (Electronic) IS - 1138-3593 (Linking) VI - 39 IP - 6 DP - 2013 Sep TI - [Methylphenidate and secondary Raynaud's phenomenon]. PG - 330-4 LID - S1138-3593(12)00233-X [pii] LID - 10.1016/j.semerg.2012.07.011 [doi] AB - Raynaud's phenomenon is a clinical disease characterized by episodic attacks of vasoconstriction of the arteries and arterioles of the extremities such as fingers and toes, sometimes the ears and nose, in response to cold or emotional stimuli. A classic attack is the pallor of the distal extremity, followed by cyanosis and redness, accompanied by paresthesia, usually as heat. When it occurs without apparent cause is called primary Raynaud's phenomenon. When associated with other disease, is called secondary Raynaud's phenomenon. The secondary table is associated with increased frequency of rheumatic diseases of collagen. They can also present certain drugs that cause vasoconstriction, such as ergotamine, beta-adrenergic antagonists, contraception and sympathomimetic drugs. Regarding the latter, we present a case of Raynaud's phenomenon secondary to methylphenidate in a 14 years. CI - Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved. FAU - Iglesias Otero, M AU - Iglesias Otero M AD - Medicina de Familia y Comunitaria, Centro de Salud de Padrón, Gerencia de Gestión Integrada de Santiago de Compostela, Servicio Gallego de Salud, España. FAU - Portela Romero, M AU - Portela Romero M FAU - Bugarín González, R AU - Bugarín González R FAU - Ventura Victoria, M A AU - Ventura Victoria MA LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Metilfenidato y fenómeno de Raynaud secundario. DEP - 20121104 PL - Spain TA - Semergen JT - Semergen JID - 9610769 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) SB - IM MH - Adolescent MH - Central Nervous System Stimulants/*adverse effects MH - Humans MH - Male MH - Methylphenidate/*adverse effects MH - Raynaud Disease/*chemically induced OTO - NOTNLM OT - Fenómeno de Raynaud primario OT - Fenómeno de Raynaud secundario OT - Methylphenidate OT - Metilfenidato OT - Primary Raynaud's phenomenon OT - Secundary Raynaud's phenomenon EDAT- 2013/09/17 06:00 MHDA- 2015/11/13 06:00 CRDT- 2013/09/17 06:00 PHST- 2012/07/05 00:00 [received] PHST- 2012/07/26 00:00 [revised] PHST- 2012/07/30 00:00 [accepted] PHST- 2013/09/17 06:00 [entrez] PHST- 2013/09/17 06:00 [pubmed] PHST- 2015/11/13 06:00 [medline] AID - S1138-3593(12)00233-X [pii] AID - 10.1016/j.semerg.2012.07.011 [doi] PST - ppublish SO - Semergen. 2013 Sep;39(6):330-4. doi: 10.1016/j.semerg.2012.07.011. Epub 2012 Nov 4. PMID- 30671625 OWN - NLM STAT- MEDLINE DCOM- 20191002 LR - 20200309 IS - 1432-1173 (Electronic) IS - 0017-8470 (Linking) VI - 70 IP - 2 DP - 2019 Feb TI - [Raynaud's phenomenon : Practical management for dermatologists]. PG - 131-141 LID - 10.1007/s00105-018-4353-9 [doi] AB - Raynaud's phenomenon (RP) is a painful vasospasm of small arteries, localised in fingers and toes. Typically these body parts turn white (ischemia), then blue (deoxygenation) and then red (reperfusion). Two different types of RP exist: the common primary RP without underlying disease and the rare secondary RP, mostly associated with rheumatoid diseases such as systemic sclerosis. Thus, the dermatologist has to be aware of this condition. In this article the clinical criteria, differential diagnoses, diagnostic considerations and treatment options are discussed. FAU - Drerup, Christian AU - Drerup C AD - Klinik für Hautkrankheiten, Allgemeine Dermatologie und Venerologie, Universitätsklinikum Münster, Von-Esmarch Str. 58, 48149, Münster, Deutschland. FAU - Ehrchen, Jan AU - Ehrchen J AD - Klinik für Hautkrankheiten, Allgemeine Dermatologie und Venerologie, Universitätsklinikum Münster, Von-Esmarch Str. 58, 48149, Münster, Deutschland. jan.ehrchen@ukmuenster.de. LA - ger PT - Journal Article TT - Raynaud-Phänomen : Praktisches Management durch den Dermatologen. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 SB - IM MH - *Dermatologists MH - Diagnosis, Differential MH - Fingers MH - Humans MH - *Raynaud Disease/etiology/therapy MH - Scleroderma, Systemic/complications OTO - NOTNLM OT - Connective tissue disease OT - Cyanosis OT - Ischemia OT - Nailfold capillaroscopy OT - Systemic sclerosis EDAT- 2019/01/24 06:00 MHDA- 2019/10/03 06:00 CRDT- 2019/01/24 06:00 PHST- 2019/01/24 06:00 [pubmed] PHST- 2019/10/03 06:00 [medline] PHST- 2019/01/24 06:00 [entrez] AID - 10.1007/s00105-018-4353-9 [pii] AID - 10.1007/s00105-018-4353-9 [doi] PST - ppublish SO - Hautarzt. 2019 Feb;70(2):131-141. doi: 10.1007/s00105-018-4353-9. PMID- 21885977 OWN - NLM STAT- MEDLINE DCOM- 20120120 LR - 20220318 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 23 IP - 6 DP - 2011 Nov TI - Contemporary management of Raynaud's phenomenon and digital ischaemic complications. PG - 555-61 LID - 10.1097/BOR.0b013e32834aa40b [doi] AB - PURPOSE OF REVIEW: The present review gives an update of the current management of Raynaud's phenomenon and its ischaemic complications (digital ulceration and critical ischaemia) and discusses possible further developments in the next 5-10 years. New approaches to therapy are being driven by increased understanding of pathophysiology and by increased international networking of clinicians and scientists, facilitating clinical trials. RECENT FINDINGS: Key points include phosphodiesterase inhibitors most likely confer benefit, although clinical trials have given somewhat conflicting results, and have been short-term; a new topical, easy-to-use glyceryl trinitrate preparation has been shown to improve Raynaud's Condition Score; the endothelin-1 receptor antagonist bosentan has now been shown to reduce the number of new systemic sclerosis (SSc)-related digital ulcers in two multinational clinical trials; and although statin therapy is likely to confer benefit in SSc-related Raynaud's phenomenon, further research is required to confirm this. SUMMARY: New therapeutic approaches in patients who do not respond to more traditionally used vasodilators include phosphodiesterase inhibitors and (for those with recurrent SSc-related digital ulcers) endothelin-1 receptor antagonism. Several other potential new therapies are being researched. Optimal management of digital ulceration is multidisciplinary including tissue viability and (sometimes) surgical input. FAU - Herrick, Ariane L AU - Herrick AL AD - The University of Manchester, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Salford, UK. ariane.herrick@manchester.ac.uk LA - eng GR - Arthritis Research UK/United Kingdom GR - Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Antioxidants) RN - 0 (Endothelin A Receptor Antagonists) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Platelet Aggregation Inhibitors) RN - 31C4KY9ESH (Nitric Oxide) RN - 94ZLA3W45F (Arginine) SB - IM MH - Antioxidants/therapeutic use MH - Arginine/metabolism MH - Endothelin A Receptor Antagonists MH - Fingers/blood supply MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use MH - Ischemia/*etiology/*therapy MH - Nitric Oxide/metabolism MH - Platelet Aggregation Inhibitors/therapeutic use MH - Raynaud Disease/*complications/metabolism/*therapy MH - Signal Transduction MH - Skin Ulcer/*etiology/*therapy MH - Toes/blood supply EDAT- 2011/09/03 06:00 MHDA- 2012/01/21 06:00 CRDT- 2011/09/03 06:00 PHST- 2011/09/03 06:00 [entrez] PHST- 2011/09/03 06:00 [pubmed] PHST- 2012/01/21 06:00 [medline] AID - 10.1097/BOR.0b013e32834aa40b [doi] PST - ppublish SO - Curr Opin Rheumatol. 2011 Nov;23(6):555-61. doi: 10.1097/BOR.0b013e32834aa40b. PMID- 30552281 OWN - NLM STAT- MEDLINE DCOM- 20191104 LR - 20231005 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 8 IP - 12 DP - 2018 Dec 14 TI - Behaviour change interventions for the management of Raynaud's phenomenon: a systematic literature review. PG - e024528 LID - 10.1136/bmjopen-2018-024528 [doi] LID - e024528 AB - OBJECTIVES: Raynaud's phenomenon (RP) is a significant cause of morbidity. Vasodilator medications cause unwanted adverse effects, with behavioural and lifestyle changes forming the mainstay of self-management; this is difficult to implement successfully. The objectives of this study were to evaluate the efficacy of behaviour change interventions for RP and identify learning points for future treatment development. DESIGN: Systematic literature review and narrative synthesis of findings. DATA SOURCES: EMBASE, MEDLINE, Cochrane and PsycINFO were searched for eligible studies on 22 August 2017. ELIGIBILITY CRITERIA: Randomised controlled trials (RCTs) of behaviour change interventions with at least one control comparator arm. DATA EXTRACTION AND SYNTHESIS: Study selection, data extraction and risk of bias were assessed independently by two reviewers, reaching consensus with a third when necessary. Primary outcomes of interest included severity/impact, frequency and duration of RP episodes, pain, disability, adverse events and study withdrawal. RESULTS: Of 638 articles retrieved, eight studies fulfilled criteria for inclusion. Biofeedback was the active behaviour change treatment arm for seven studies, with one study reporting a behavioural intervention. Studies were published 1978-2002; six were USA-based studies, one German and one Swedish. Using Cochrane Risk of Bias assessment, studies were assessed to be overall at high risk of bias, with the exception of one large RCT. The total sample included 495 participants (study median=29), with a median age of 39.5 years and preponderance towards females (73%). Five studies reported significant effects in primary outcomes of interest; however, due to missing data, relative efficacy of interventions could not be reliably assessed. CONCLUSIONS: There is no evidence to support or refute claims of the efficacy of behaviour change interventions for the management of RP. There remains a strong case for developing and testing behaviour change interventions that focus on self-management; however, theoretical development and advancement in trial quality is imperative to underpin future work. PROSPERO REGISTRATION NUMBER: CRD42017049643. CI - © Author(s) (or their employer(s)) 2018. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Daniels, Jo AU - Daniels J AUID- ORCID: 0000-0003-3067-9416 AD - Department of Psychology, The University of Bath, Bath, UK. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. AD - Department of Pharmacy and Pharmacology, The University of Bath, Bath, UK. FAU - Eccleston, Christopher AU - Eccleston C AD - Centre for Pain Research, University of Bath, Bath, UK. AD - Department of Clinical and Health Psychology, Ghent University, Ghent, Belgium. LA - eng PT - Journal Article PT - Systematic Review DEP - 20181214 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - *Behavior Therapy MH - Humans MH - Life Style MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*psychology/*therapy MH - *Self-Management PMC - PMC6303561 OTO - NOTNLM OT - behaviour-change OT - raynaud’s OT - raynaud’s phenomenon OT - systematic review COIS- Competing interests: None declared. EDAT- 2018/12/16 06:00 MHDA- 2019/11/05 06:00 PMCR- 2018/12/14 CRDT- 2018/12/16 06:00 PHST- 2018/12/16 06:00 [entrez] PHST- 2018/12/16 06:00 [pubmed] PHST- 2019/11/05 06:00 [medline] PHST- 2018/12/14 00:00 [pmc-release] AID - bmjopen-2018-024528 [pii] AID - 10.1136/bmjopen-2018-024528 [doi] PST - epublish SO - BMJ Open. 2018 Dec 14;8(12):e024528. doi: 10.1136/bmjopen-2018-024528. PMID- 21898061 OWN - NLM STAT- MEDLINE DCOM- 20120703 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 3 DP - 2012 Mar TI - The association of Raynaud's syndrome with carpal tunnel syndrome: a meta-analysis. PG - 569-74 LID - 10.1007/s00296-011-2122-5 [doi] AB - Carpal tunnel syndrome (CTS) has traditionally been included among the diseases associated with Raynaud's syndrome (RS). The prevalence of RS in patients suffering from CTS is not well defined. The objective of this paper was to assess the prevalence of RS in patients with CTS-a meta-analysis of published data was performed. The PubMed database of the National Library of Medicine and ISI Web of Knowledge was used for studies dealing with RS and CTS. The studies provided sufficient data to estimate the prevalence of RS in patients of CTS. A forest plot was determined by the revealed prevalence. Statistical analysis was based on methods for a random effects meta-analysis and a finite mixture model for proportions. Publication bias was investigated with the linear regression test (Egger's method). A meta-regression was conducted by the year of publication. Eight eligible studies, contributing data on 675 subjects, were included in this meta-analysis. For CTS, a pooled prevalence of 15.5% and 95% CI (95% CI 0.043, 0.318) were obtained. Statistically publication bias was present (P value 0.143). A mixture model analysis found five latent classes. The meta-regression indicated that the estimated prevalence increased when the year of commencement increased, too. Within the decade (1957-1967), the odds ratio increased from 1 (95% CI 1.065, 1.112) to 2.340 (95% CI 1.886, 2.903). Despite some heterogeneity, there is a possible indication of an association between RS and patients with CTS. FAU - Hartmann, Peter AU - Hartmann P AD - Charité University Hospital, Berlin, Germany. peter.hartmann@live.de FAU - Mohokum, Melvin AU - Mohokum M FAU - Schlattmann, Peter AU - Schlattmann P LA - eng PT - Journal Article PT - Meta-Analysis PT - Review DEP - 20110907 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Carpal Tunnel Syndrome/*complications/epidemiology/physiopathology MH - Comorbidity MH - Databases, Bibliographic MH - Humans MH - Models, Statistical MH - Prevalence MH - Raynaud Disease/*complications/epidemiology/physiopathology EDAT- 2011/09/08 06:00 MHDA- 2012/07/04 06:00 CRDT- 2011/09/08 06:00 PHST- 2011/04/07 00:00 [received] PHST- 2011/08/22 00:00 [accepted] PHST- 2011/09/08 06:00 [entrez] PHST- 2011/09/08 06:00 [pubmed] PHST- 2012/07/04 06:00 [medline] AID - 10.1007/s00296-011-2122-5 [doi] PST - ppublish SO - Rheumatol Int. 2012 Mar;32(3):569-74. doi: 10.1007/s00296-011-2122-5. Epub 2011 Sep 7. PMID- 38739175 OWN - NLM STAT- MEDLINE DCOM- 20250422 LR - 20250513 IS - 1432-1971 (Electronic) IS - 0172-0643 (Linking) VI - 46 IP - 4 DP - 2025 Apr TI - Pediatric Primary Raynaud's Phenomenon: A Comprehensive Cardiovascular Analysis. PG - 908-913 LID - 10.1007/s00246-024-03514-9 [doi] AB - Our aim in this study is to evaluate the cardiovascular findings of pediatric patients with primary Raynaud's phenomenon (RP) and to determine if there are any pathological findings. Our study included 42 pediatric patients aged between 7 and 18 who were diagnosed with primary RP and did not have any additional underlying structural vascular disease or secondary rheumatological conditions. The control group consisted of 30 healthy volunteers aged 7-18 years, matched by age and sex, without any additional diseases. We evaluated demographic, clinical, and laboratory findings, echocardiographic and capillaroscopic features, as well as carotid intima-media thickness. Compared to the control group, pediatric patients with primary RP showed increased A wave velocity and E/E' ratio parameters in the left ventricle, indicating diastolic dysfunction of the heart. The isovolumetric relaxation time (IVRT) was prolonged in both the left and right ventricles, and the E/A ratio decreased in the left ventricle. The myocardial performance index (MPI), indicating both systolic and diastolic dysfunction, increased in both ventricles. Additionally, the aortic stiffness index, aortic elastic modulus (Ep), and left carotid intima-media thickness (CIMT) significantly increased, while distensibility decreased in pediatric patients with primary RP compared to the control group. The cardiovascular evaluation of pediatric patients with primary RP revealed that diastolic dysfunction is likely present in both the left and right heart. Additionally, based on the aorta and carotid intima measurements, it is suggested that pediatric patients with primary RP are at risk for developing atherosclerosis. CI - © 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. FAU - Özpınar, Şeyma AU - Özpınar Ş AD - Department of Pediatrics, Faculty of Health Sciences, Kanuni Sultan Süleyman Education and Research Hospital, Istanbul, Turkey. drseymapolat@gmail.com. FAU - Bornaun, Helen AU - Bornaun H AD - Department of Pediatric Cardiology, Faculty of Health Sciences, Kanuni Sultan Süleyman Education and Research Hospital, Istanbul, Turkey. FAU - Sönmez, Hafize Emine AU - Sönmez HE AD - Department of Pediatric Rheumatology, Kocaeli University, Kocaeli, Turkey. FAU - Doğan, Sümeyra AU - Doğan S AD - Department of Radiology, Faculty of Health Sciences, Kanuni Sultan Süleyman Education and Research Hospital, Istanbul, Turkey. FAU - Sönmez, Süleyman AU - Sönmez S AD - Department of Radiology, Faculty of Health Sciences, Kanuni Sultan Süleyman Education and Research Hospital, Istanbul, Turkey. FAU - Harman, Halil AU - Harman H AD - Department of Rheumatology, Faculty of Health Sciences, Kanuni Sultan Süleyman Education and Research Hospital, Istanbul, Turkey. LA - eng PT - Journal Article DEP - 20240513 PL - United States TA - Pediatr Cardiol JT - Pediatric cardiology JID - 8003849 SB - IM MH - Humans MH - Child MH - *Raynaud Disease/physiopathology/complications/diagnosis MH - Male MH - Female MH - Adolescent MH - Carotid Intima-Media Thickness MH - Echocardiography MH - Case-Control Studies MH - Vascular Stiffness MH - *Heart Ventricles/diagnostic imaging/physiopathology OTO - NOTNLM OT - Antinuclear antibodies OT - Capillaroscopy OT - Echocardiography OT - Raynaud phenomenon COIS- Declarations. Conflict of interest: The authors declare no conflict of interest. EDAT- 2024/05/13 12:54 MHDA- 2025/03/12 18:17 CRDT- 2024/05/13 11:04 PHST- 2024/02/18 00:00 [received] PHST- 2024/04/29 00:00 [accepted] PHST- 2025/03/12 18:17 [medline] PHST- 2024/05/13 12:54 [pubmed] PHST- 2024/05/13 11:04 [entrez] AID - 10.1007/s00246-024-03514-9 [pii] AID - 10.1007/s00246-024-03514-9 [doi] PST - ppublish SO - Pediatr Cardiol. 2025 Apr;46(4):908-913. doi: 10.1007/s00246-024-03514-9. Epub 2024 May 13. PMID- 31364293 OWN - NLM STAT- MEDLINE DCOM- 20200224 LR - 20200224 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 17 IP - 7 DP - 2019 Jul TI - Systemic sclerosis - the dermatological perspective. PG - 716-728 LID - 10.1111/ddg.13887 [doi] AB - Systemic scleroderma/systemic sclerosis (SSc) is an inflammatory connective tissue disease clinically characterized by two major subtypes: limited and diffuse SSc. While both conditions present with Raynaud's phenomenon (paroxysmal digital ischemia), diffuse SSc is associated with rapid disease progression and early - prognostically relevant - involvement of internal organs. Treatment is challenging. In addition to general lifestyle modifications, measures include treatments aimed at improving circulation as well as immunosuppressive and immunomodulatory drugs. However, these agents are effective only in terms of slowing disease progression. CI - © 2019 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. FAU - Sticherling, Michael AU - Sticherling M AD - Department of Dermatology, University Medical Center, Erlangen, Germany. LA - eng PT - Journal Article PT - Review PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 RN - 0 (Immunosuppressive Agents) SB - IM MH - Disease Progression MH - Humans MH - Immunosuppressive Agents MH - Life Style MH - Raynaud Disease/complications/*therapy MH - Scleroderma, Systemic/classification/complications/epidemiology/*therapy EDAT- 2019/08/01 06:00 MHDA- 2020/02/25 06:00 CRDT- 2019/08/01 06:00 PHST- 2019/05/07 00:00 [received] PHST- 2019/06/21 00:00 [accepted] PHST- 2019/08/01 06:00 [entrez] PHST- 2019/08/01 06:00 [pubmed] PHST- 2020/02/25 06:00 [medline] AID - 10.1111/ddg.13887 [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2019 Jul;17(7):716-728. doi: 10.1111/ddg.13887. PMID- 35445832 OWN - NLM STAT- MEDLINE DCOM- 20220504 LR - 20230322 IS - 1435-1250 (Electronic) IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 81 IP - 4 DP - 2022 May TI - [Nailfold capillaroscopy-Principles and clinical application]. PG - 313-322 LID - 10.1007/s00393-022-01200-w [doi] AB - Nailfold capillaroscopy is a rapid and easily applicable differential diagnostic technique that allows direct visualization of the microcirculation. Abnormal findings in nailfold capillaroscopy are closely associated with connective tissue diseases, such as systemic sclerosis. The clinical manifestation of impaired microcirculation is Raynaud's phenomenon, which is a classical symptom of connective tissue diseases. Nailfold capillaroscopy is increasingly used in various fields of medicine, therefore it is important to define methods for the acquisition and analysis of the results of nailfold capillary and to have a uniform definition of abnormal capillaries. This article discusses image acquisition and analysis, various capillaroscopic techniques, normal and abnormal capillaroscopic features and their significance, scoring systems and reliability of image acquisition and interpretation. CI - © 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature. FAU - Hasseli-Fräbel, R AU - Hasseli-Fräbel R AD - Medizinische Klinik und Poliklinik II, Universitätsklinikum Gießen, Justus-Liebig-Universität Gießen, Gießen, Deutschland. FAU - Hermann, W AU - Hermann W AD - Abteilung für Rheumatologie und Klinische Immunologie, Kerckhoff-Klinik GmbH, Benekestr. 2-8, 61231, Bad Nauheim, Deutschland. w.hermann@kerckhoff-klinik.de. FAU - Sander, O AU - Sander O AD - Poliklinik und Funktionsbereich für Rheumatologie, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität, Düsseldorf, Deutschland. FAU - Triantafyllias, K AU - Triantafyllias K AD - RZ Rheumazentrum Rheinland-Pfalz, Bad Kreuznach, Deutschland. LA - ger PT - Journal Article TT - Kapillarmikroskopie – Grundlagen und klinische Anwendung. DEP - 20220421 PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM CIN - Dermatologie (Heidelb). 2023 Jan;74(1):53-54. doi: 10.1007/s00105-022-05095-1. PMID: 36633635 MH - Capillaries/diagnostic imaging MH - *Connective Tissue Diseases/diagnostic imaging MH - Humans MH - Microscopic Angioscopy/methods MH - Nails/blood supply/diagnostic imaging MH - *Raynaud Disease/diagnosis MH - Reproducibility of Results MH - *Scleroderma, Systemic/diagnosis PMC - PMC9022415 OTO - NOTNLM OT - Connective tissue disease OT - Inflammatory rheumatic diseases OT - Microangiopathy OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2022/04/22 06:00 MHDA- 2022/05/06 06:00 PMCR- 2022/04/21 CRDT- 2022/04/21 12:01 PHST- 2022/03/11 00:00 [accepted] PHST- 2022/04/22 06:00 [pubmed] PHST- 2022/05/06 06:00 [medline] PHST- 2022/04/21 12:01 [entrez] PHST- 2022/04/21 00:00 [pmc-release] AID - 10.1007/s00393-022-01200-w [pii] AID - 1200 [pii] AID - 10.1007/s00393-022-01200-w [doi] PST - ppublish SO - Z Rheumatol. 2022 May;81(4):313-322. doi: 10.1007/s00393-022-01200-w. Epub 2022 Apr 21. PMID- 38290781 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20241120 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 8 DP - 2024 Aug 1 TI - Raynaud's phenomenon as an adverse event associated with Bruton tyrosine kinase inhibitor. PG - e222-e223 LID - 10.1093/rheumatology/keae053 [doi] FAU - Deligeorgakis, Dimitrios AU - Deligeorgakis D AUID- ORCID: 0000-0002-1379-4888 AD - Fourth Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. FAU - Adamichou, Christina AU - Adamichou C AUID- ORCID: 0000-0001-7374-7795 AD - Fourth Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. LA - eng PT - Case Reports PT - Letter PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - EC 2.7.10.2 (Agammaglobulinaemia Tyrosine Kinase) RN - 0 (Pyrimidines) RN - 0 (Tyrosine Kinase Inhibitors) SB - IM MH - Humans MH - Male MH - Middle Aged MH - *Agammaglobulinaemia Tyrosine Kinase/antagonists & inhibitors MH - Pyrimidines/adverse effects MH - *Raynaud Disease/chemically induced/diagnosis MH - *Tyrosine Kinase Inhibitors/adverse effects EDAT- 2024/01/31 00:42 MHDA- 2024/08/01 06:42 CRDT- 2024/01/30 21:13 PHST- 2024/01/18 00:00 [accepted] PHST- 2024/08/01 06:42 [medline] PHST- 2024/01/31 00:42 [pubmed] PHST- 2024/01/30 21:13 [entrez] AID - 7593759 [pii] AID - 10.1093/rheumatology/keae053 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Aug 1;63(8):e222-e223. doi: 10.1093/rheumatology/keae053. PMID- 21147732 OWN - NLM STAT- MEDLINE DCOM- 20110118 LR - 20181023 IS - 1756-1833 (Electronic) IS - 0959-8138 (Linking) VI - 341 DP - 2010 Dec 8 TI - Raynaud's phenomenon and macrocytic anaemia. PG - c6011 LID - bmj.c6011 [pii] LID - 10.1136/bmj.c6011 [doi] FAU - Flower, V AU - Flower V AD - Royal National Hospital for Rheumatic Disease, Bath, UK. FAU - Pauling, J D AU - Pauling JD FAU - Shipley, J A AU - Shipley JA FAU - McHugh, N J AU - McHugh NJ LA - eng PT - Case Reports PT - Journal Article DEP - 20101208 PL - England TA - BMJ JT - BMJ (Clinical research ed.) JID - 8900488 SB - IM MH - Anemia, Hemolytic, Autoimmune/diagnosis/etiology MH - Anemia, Macrocytic/*complications MH - Coombs Test MH - Cyanosis/diagnosis/*etiology/therapy MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*complications MH - Referral and Consultation MH - Thermography EDAT- 2010/12/15 06:00 MHDA- 2011/01/19 06:00 CRDT- 2010/12/15 06:00 PHST- 2010/12/15 06:00 [entrez] PHST- 2010/12/15 06:00 [pubmed] PHST- 2011/01/19 06:00 [medline] AID - bmj.c6011 [pii] AID - 10.1136/bmj.c6011 [doi] PST - epublish SO - BMJ. 2010 Dec 8;341:c6011. doi: 10.1136/bmj.c6011. PMID- 33058653 OWN - NLM STAT- MEDLINE DCOM- 20210118 LR - 20210118 IS - 2101-017X (Electronic) IS - 0035-2640 (Linking) VI - 70 IP - 5 DP - 2020 May TI - [Acrosyndromes (Raynaud's phenomenon, erythermalgia, acrocyanosis, frostbite, digital]. PG - e147-e153 FAU - Andrès, Emmanuel AU - Andrès E AD - Service de médecine interne, diabète et maladies métaboliques, clinique médicale B, Hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France. FAU - Serraj, Khalid AU - Serraj K AD - Service de médecine interne, diabète et maladies métaboliques, clinique médicale B, Hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France. AD - Service de médecine interne-hématologie, CHU Mohammed-VI, 59000 Oujda, Maroc. LA - fre PT - Journal Article TT - Acrosyndromes (phénomène de Raynaud, érythermalgie, acrocyanose, engelures, ischémie digitale). PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Cyanosis/diagnosis/etiology MH - *Erythromelalgia MH - *Frostbite/diagnosis/therapy MH - Humans MH - *Raynaud Disease/diagnosis OTO - NOTNLM OT - Peripheral vascular diseases OT - Raynaud's disease COIS- E. Andrès déclare avoir participé à des interventions ponctuelles pour les entreprises Novartis, BMS, Pfizer, Boehringer et Air Liquide. K. Serraj déclare avoir participé à des interventions ponctuelles (et avoir été pris en charge, à l’occasion de déplacement ) pour les entreprises Novartis, Roche, Janssen. EDAT- 2020/10/16 06:00 MHDA- 2021/01/20 06:00 CRDT- 2020/10/15 17:48 PHST- 2020/10/15 17:48 [entrez] PHST- 2020/10/16 06:00 [pubmed] PHST- 2021/01/20 06:00 [medline] PST - ppublish SO - Rev Prat. 2020 May;70(5):e147-e153. PMID- 32324110 OWN - NLM STAT- MEDLINE DCOM- 20200930 LR - 20200930 IS - 2578-5826 (Electronic) IS - 2578-5826 (Linking) VI - 43 IP - 3 DP - 2020 Sep TI - 'Narrow-sense' and 'broad-sense' vascular abnormalities of systemic sclerosis. PG - 107-114 LID - 10.1080/25785826.2020.1754692 [doi] AB - Systemic sclerosis (SSc) induces skin thickening and numerous symptoms involving the entire body. Collagen deposition, immune disorder, and vascular abnormalities is currently estimated to be three major causal factors involved in the respective conditions. Vascular abnormalities usually develop in the initial phase of this disease, and may exist in all phases; therefore, they markedly influence the patient's quality of life. This article reviews recent findings about 'narrow-sense' vascular lesions (including Raynaud's phenomenon, skin ulcers, nailfold bleedings, pitting scars, telangiectasia, and pulmonary hypertension) and 'broad-sense' vascular lesions (such as calcinosis or erectile dysfunction). Affected blood vessels can be classified into arteriole/small artery and capillary blood vessels. Furthermore, pathological changes include the proliferation of the vascular endothelial or smooth muscle cells, lumen stenosis by collagen accumulation of the vascular intima, vasodilation or fragility, and apoptosis. There may be interaction between vascular lesions, autoimmune disorder, and collagen deposition. Thus, various symptoms of this disease may be controlled through the treatment of vascular lesions. FAU - Jinnin, Masatoshi AU - Jinnin M AD - Department of Dermatology, Wakayama Medical University Graduate School of Medicine, Wakayama, Japan. LA - eng PT - Journal Article PT - Review DEP - 20200423 PL - England TA - Immunol Med JT - Immunological medicine JID - 101736847 RN - 9007-34-5 (Collagen) SB - IM MH - Calcinosis/etiology MH - Collagen/metabolism MH - Endothelial Cells/pathology MH - Erectile Dysfunction/etiology MH - Female MH - Humans MH - Hypertension, Pulmonary/etiology MH - Male MH - Myocytes, Smooth Muscle/pathology MH - *Quality of Life MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/etiology MH - Telangiectasis/etiology MH - Vascular Diseases/*etiology OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ulcers OT - nailfold bleedings EDAT- 2020/04/24 06:00 MHDA- 2020/10/02 06:00 CRDT- 2020/04/24 06:00 PHST- 2020/04/24 06:00 [pubmed] PHST- 2020/10/02 06:00 [medline] PHST- 2020/04/24 06:00 [entrez] AID - 10.1080/25785826.2020.1754692 [doi] PST - ppublish SO - Immunol Med. 2020 Sep;43(3):107-114. doi: 10.1080/25785826.2020.1754692. Epub 2020 Apr 23. PMID- 30155665 OWN - NLM STAT- MEDLINE DCOM- 20191001 LR - 20191001 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 38 IP - 12 DP - 2018 Dec TI - Use of nitroglycerin ointment to treat primary and secondary Raynaud's phenomenon: a systematic literature review. PG - 2209-2216 LID - 10.1007/s00296-018-4119-9 [doi] AB - Raynaud's phenomenon (RP) is a microvascular condition in which reversible ischemic attacks occur in the extremities. Due to the unpredictable nature of these attacks, pharmacologic agents that can be administered on as-needed basis are currently being sought after. Topical nitrates are well suited for as-needed use, and several different formulations have been studied for the treatment of RP, including ointments, gels, patches, and tapes. However, these different dosage forms are not all equal in terms of safety and efficacy, and not every one is commercially available for use in clinical practice. Nitroglycerin ointment is commercially available, and it has less systemic side effects than other topical formulations. Since its role in the treatment of RP has not yet been completely established, we performed a systematic search of Medline, Embase, and the Cochrane Central Register of Controlled Trials to evaluate its safety and efficacy. A total of 1125 studies were identified, and 7 were included in our review. Although the included studies used different measures of efficacy, the majority reported positive responses to nitroglycerin ointment. The benefit of nitroglycerin ointment in the treatment of RP may be further realized through more robust investigation. FAU - Qiu, Olivia AU - Qiu O AUID- ORCID: 0000-0001-5859-4313 AD - Ernest Mario School of Pharmacy, 160 Frelinghuysen Rd., Piscataway, NJ, 08854, USA. FAU - Chan, Theresa AU - Chan T AUID- ORCID: 0000-0001-6781-0751 AD - Ernest Mario School of Pharmacy, 160 Frelinghuysen Rd., Piscataway, NJ, 08854, USA. FAU - Luen, Matthew AU - Luen M AUID- ORCID: 0000-0003-2079-6280 AD - Massachusetts College of Pharmacy and Health Sciences, 13516 Bonnie Dale Drive, Gaithersburg, MD, 20878, USA. FAU - Cruz, Joseph E AU - Cruz JE AUID- ORCID: 0000-0002-8083-8692 AD - Overlook Medical Center, 99 Beauvoir Avenue, Summit, NJ, 07901, USA. FAU - Hermes-DeSantis, Evelyn R AU - Hermes-DeSantis ER AUID- ORCID: 0000-0001-5658-3899 AD - Ernest Mario School of Pharmacy, 160 Frelinghuysen Rd., Piscataway, NJ, 08854, USA. ehermesd@pharmacy.rutgers.edu. LA - eng PT - Journal Article PT - Systematic Review DEP - 20180822 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Ointments) RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Administration, Cutaneous MH - Humans MH - Microcirculation/*drug effects MH - Nitroglycerin/*administration & dosage/adverse effects MH - Ointments MH - Raynaud Disease/diagnosis/*drug therapy/epidemiology/physiopathology MH - Treatment Outcome MH - Vasodilation/*drug effects MH - Vasodilator Agents/*administration & dosage/adverse effects OTO - NOTNLM OT - Nitroglycerin OT - Ointment OT - Raynaud’s phenomenon OT - Topical therapy EDAT- 2018/08/30 06:00 MHDA- 2019/10/02 06:00 CRDT- 2018/08/30 06:00 PHST- 2018/05/31 00:00 [received] PHST- 2018/08/02 00:00 [accepted] PHST- 2018/08/30 06:00 [pubmed] PHST- 2019/10/02 06:00 [medline] PHST- 2018/08/30 06:00 [entrez] AID - 10.1007/s00296-018-4119-9 [pii] AID - 10.1007/s00296-018-4119-9 [doi] PST - ppublish SO - Rheumatol Int. 2018 Dec;38(12):2209-2216. doi: 10.1007/s00296-018-4119-9. Epub 2018 Aug 22. PMID- 35139518 OWN - NLM STAT- MEDLINE DCOM- 20220623 LR - 20220706 IS - 2504-2106 (Electronic) IS - 2504-2092 (Linking) VI - 29 IP - 3 DP - 2022 TI - Topical Rosmarinus officinalis L. in Systemic Sclerosis-Related Raynaud's Phenomenon: An Open-Label Pilot Study. PG - 242-248 LID - 10.1159/000522507 [doi] AB - BACKGROUND: An effective and well-tolerated topical treatment of Raynaud's phenomenon is needed. The aim of this pilot study was to determine change in skin temperature and self-reported warmth perception from topical rosemary essential oil in patients with systemic sclerosis and secondary Raynaud's phenomenon. PATIENTS AND METHODS: Twelve patients with progressive systemic sclerosis and Raynaud's phenomenon were consecutively enrolled in an open-label pilot study at a university outpatient rheumatology clinic. Each patient received an application of olive oil to both hands as a control and 3 h later an application of a 10% essential oil of Rosmarinus officinalis L. Clinical severity and subjective warmth perception were assessed; skin temperature was measured by infrared thermography. RESULTS: Skin temperature increased significantly after both olive oil and rosemary oil but differences between oils did not reach significance. Self-reported warmth perception increased after rosemary oil but not after olive oil. No adverse effects were observed. CONCLUSION: Topical rosemary essential oil increased warmth perception in patients with systemic sclerosis-related Raynaud's phenomenon but did not increase finger skin temperature more than the olive oil control. CI - © 2022 S. Karger AG, Basel. FAU - Vagedes, Jan AU - Vagedes J AD - ARCIM Institute, Filderstadt, Germany. AD - Department of Neonatology, University Hospital Tübingen, Tübingen, Germany. AD - Department of Pediatrics, Filderklinik, Filderstadt, Germany. FAU - Henes, Jörg AU - Henes J AD - Department of Internal Medicine II, University Hospital Tübingen, Tübingen, Germany. FAU - Deckers, Bernhard AU - Deckers B AD - ARCIM Institute, Filderstadt, Germany. FAU - Vagedes, Katrin AU - Vagedes K AD - ARCIM Institute, Filderstadt, Germany. FAU - Kuderer, Silja AU - Kuderer S AD - ARCIM Institute, Filderstadt, Germany. FAU - Helmert, Eduard AU - Helmert E AD - ARCIM Institute, Filderstadt, Germany. FAU - von Schoen-Angerer, Tido AU - von Schoen-Angerer T AD - ARCIM Institute, Filderstadt, Germany. AD - Department of Pediatrics, Fribourg Hospital HFR, Fribourg, Switzerland. LA - eng PT - Clinical Trial PT - Journal Article TT - Topische Anwendung von Rosmarinus officinalis L. bei Raynaud-Phänomen im Zusammenhang mit systemischer Sklerose: Eine unverblindete Pilotstudie. DEP - 20220209 PL - Switzerland TA - Complement Med Res JT - Complementary medicine research JID - 101698453 RN - 0 (Oils, Volatile) RN - 0 (Olive Oil) SB - IM MH - Humans MH - *Oils, Volatile/therapeutic use MH - Olive Oil/therapeutic use MH - Pilot Projects MH - *Raynaud Disease/complications/etiology MH - *Rosmarinus MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Raynaud phenomenon OT - Rosemary essential oil OT - Rosmarinus officinalis OT - Scleroderma OT - Systemic sclerosis EDAT- 2022/02/10 06:00 MHDA- 2022/06/24 06:00 CRDT- 2022/02/09 20:08 PHST- 2021/03/30 00:00 [received] PHST- 2022/02/04 00:00 [accepted] PHST- 2022/02/10 06:00 [pubmed] PHST- 2022/06/24 06:00 [medline] PHST- 2022/02/09 20:08 [entrez] AID - 000522507 [pii] AID - 10.1159/000522507 [doi] PST - ppublish SO - Complement Med Res. 2022;29(3):242-248. doi: 10.1159/000522507. Epub 2022 Feb 9. PMID- 37054875 OWN - NLM STAT- MEDLINE DCOM- 20230811 LR - 20230816 IS - 1532-2165 (Electronic) IS - 1078-5884 (Linking) VI - 66 IP - 2 DP - 2023 Aug TI - Endovascular Radiofrequency Ablation of Nerves for Treatment of Raynaud's Phenomenon. PG - 278-279 LID - S1078-5884(23)00293-9 [pii] LID - 10.1016/j.ejvs.2023.04.003 [doi] FAU - Xueguang, Lin AU - Xueguang L AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. FAU - Shuai, Jiang AU - Shuai J AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. FAU - Bo, Chen AU - Bo C AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. FAU - Ying, Deng AU - Ying D AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. FAU - Bo, Yu AU - Bo Y AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. FAU - Jingdong, Tang AU - Jingdong T AD - Department of General Surgery, Shanghai Pudong Hospital, Fudan University Pudong Medical Centre, Shanghai Key Laboratory of Vascular Lesions Regulation and Remodelling, Shanghai, China. Electronic address: drtangjingdong@126.com. LA - eng PT - Letter DEP - 20230411 PL - England TA - Eur J Vasc Endovasc Surg JT - European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery JID - 9512728 SB - IM CIN - Eur J Vasc Endovasc Surg. 2023 Aug;66(2):291. doi: 10.1016/j.ejvs.2023.05.004. PMID: 37169136 MH - Humans MH - *Raynaud Disease/therapy MH - *Radiofrequency Ablation OTO - NOTNLM OT - Endovascular radiofrequency ablation OT - Raynaud’s phenomenon OT - Stockholm workshop staging EDAT- 2023/04/14 06:00 MHDA- 2023/08/11 06:43 CRDT- 2023/04/13 19:23 PHST- 2022/10/12 00:00 [received] PHST- 2023/03/15 00:00 [revised] PHST- 2023/04/03 00:00 [accepted] PHST- 2023/08/11 06:43 [medline] PHST- 2023/04/14 06:00 [pubmed] PHST- 2023/04/13 19:23 [entrez] AID - S1078-5884(23)00293-9 [pii] AID - 10.1016/j.ejvs.2023.04.003 [doi] PST - ppublish SO - Eur J Vasc Endovasc Surg. 2023 Aug;66(2):278-279. doi: 10.1016/j.ejvs.2023.04.003. Epub 2023 Apr 11. PMID- 27541182 OWN - NLM STAT- MEDLINE DCOM- 20170605 LR - 20181202 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 28 IP - 6 DP - 2016 Nov TI - Recent advances in the pathogenesis and management of Raynaud's phenomenon and digital ulcers. PG - 577-85 LID - 10.1097/BOR.0000000000000332 [doi] AB - PURPOSE OF REVIEW: Systemic sclerosis (SSc)-related digital vasculopathy can progress from severe Raynaud's phenomenon to digital ulceration, is a major cause of pain and disability, and impacts negatively on quality of life. Current treatments are often ineffective and poorly tolerated. This review summarises some of the progress which has been made in the last 12 to 18 months in terms of our understanding of disease process, measurement and treatment. RECENT FINDINGS: The most important findings include that we can now better predict which patients with SSc are most likely to develop digital ulcers. In terms of treatment, a multicentre trial showed that the phosphodiesterase inhibitor sildenafil confers some benefit in SSc-related digital ulceration. Topical therapies are being explored: iontophoresis of vasodilators increases local blood flow, and in an avian model, VEGF121 fibrin applied in a gel matrix improved wound healing. SUMMARY: Progress is being made. Advances in our understanding of SSc-related vasculopathy continue to lead to exploration of new treatment approaches. Clinical trials and observational studies are challenging, but are being facilitated by developments in outcome measures and improved infrastructures and networking, allowing trials in much larger numbers of patients than have previously been possible. FAU - Herrick, Ariane L AU - Herrick AL AD - aCentre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester bNIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, UK. LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (VEGFA protein, human) RN - 0 (Vascular Endothelial Growth Factor A) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Fingers MH - Humans MH - Pain/etiology MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Systemic/complications MH - Sildenafil Citrate/therapeutic use MH - Skin Ulcer/*drug therapy/*etiology MH - Vascular Endothelial Growth Factor A/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2016/08/20 06:00 MHDA- 2017/06/06 06:00 CRDT- 2016/08/20 06:00 PHST- 2016/08/20 06:00 [entrez] PHST- 2016/08/20 06:00 [pubmed] PHST- 2017/06/06 06:00 [medline] AID - 10.1097/BOR.0000000000000332 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2016 Nov;28(6):577-85. doi: 10.1097/BOR.0000000000000332. PMID- 35504485 OWN - NLM STAT- MEDLINE DCOM- 20230718 LR - 20251231 IS - 1097-6787 (Electronic) IS - 0190-9622 (Print) IS - 0190-9622 (Linking) VI - 89 IP - 2 DP - 2023 Aug TI - Part II: Cutaneous manifestations of peripheral vascular disease. PG - 211-226 LID - S0190-9622(22)00708-3 [pii] LID - 10.1016/j.jaad.2021.05.077 [doi] AB - In this Part 2 of a 2-part continuing medical education series, we review the epidemiology of peripheral vascular disease, its association with cutaneous symptoms, and the diagnosis and evaluation of cutaneous features of vascular disorders. As peripheral vascular disease becomes more prevalent globally, it is essential for dermatologists to become competent at accurately recognizing and diagnosing cutaneous manifestations and directing individuals to receive appropriate care and treatment. CI - Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Raja, Aishwarya AU - Raja A AD - Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York. FAU - Karch, Jamie AU - Karch J AD - Department of Medicine, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire. FAU - Shih, Allen F AU - Shih AF AD - Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts. FAU - De La Garza, Henriette AU - De La Garza H AD - Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts. FAU - De Zepeda Diaz, Antonio Jesus AU - De Zepeda Diaz AJ AD - Department of Medicine, Boston University School of Medicine at Boston Medical Center, Boston, Massachusetts. FAU - Maymone, Mayra B C AU - Maymone MBC AD - Department of Dermatology, The Warren Alpert Medical School, Brown University, Providence, Rhode Island. FAU - Phillips, Tania J AU - Phillips TJ AD - Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts. FAU - Secemsky, Eric AU - Secemsky E AD - Smith Center for Outcomes Research, Departments of Cardiology and Medicine, Beth Israel Deaconess Medical Center, Boston, Massachusetts. FAU - Vashi, Neelam AU - Vashi N AD - Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts; Department of Dermatology, US Department of Veteran Affairs, Boston Health Care System, Boston, Massachusetts. Electronic address: nvashi419@gmail.com. LA - eng GR - K23 HL150290/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Review DEP - 20220430 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Humans MH - *Peripheral Vascular Diseases/diagnosis/epidemiology/etiology MH - Skin/blood supply MH - *Skin Diseases/diagnosis/epidemiology/etiology MH - *Raynaud Disease/diagnosis PMC - PMC12743645 MID - NIHMS2129973 OTO - NOTNLM OT - Raynaud’s OT - Raynaud’s disease OT - Raynaud’s phenomenon OT - acrocyanosis OT - chronic venous disease OT - chronic venous insufficiency OT - deep vein thrombosis OT - erythromelalgia OT - livedo racemosa OT - livedo reticularis OT - lymphedema OT - peripheral artery disease OT - peripheral vascular disease OT - pernio OT - superficial vein thrombosis OT - vascular disease COIS- Conflicts of interest None disclosed. EDAT- 2022/05/04 06:00 MHDA- 2023/07/18 13:08 PMCR- 2025/12/28 CRDT- 2022/05/03 19:26 PHST- 2020/11/23 00:00 [received] PHST- 2021/04/29 00:00 [revised] PHST- 2021/05/19 00:00 [accepted] PHST- 2023/07/18 13:08 [medline] PHST- 2022/05/04 06:00 [pubmed] PHST- 2022/05/03 19:26 [entrez] PHST- 2025/12/28 00:00 [pmc-release] AID - S0190-9622(22)00708-3 [pii] AID - 10.1016/j.jaad.2021.05.077 [doi] PST - ppublish SO - J Am Acad Dermatol. 2023 Aug;89(2):211-226. doi: 10.1016/j.jaad.2021.05.077. Epub 2022 Apr 30. PMID- 32402430 OWN - NLM STAT- MEDLINE DCOM- 20200914 LR - 20200914 IS - 2542-4513 (Electronic) IS - 2542-4513 (Linking) VI - 45 IP - 3 DP - 2020 May TI - Paraneoplastic Raynaud's phenomenon associated to astrocytoma. PG - 161-164 LID - S2542-4513(20)30227-3 [pii] LID - 10.1016/j.jdmv.2020.03.001 [doi] FAU - Bilancini, S AU - Bilancini S AD - J.F. Merlen Research Center for vascular diseases, Via Mola-Vecchia, 4, 03100 Frosinone, Italy. Electronic address: silviasilvietta@libero.it. FAU - Lucchi, M AU - Lucchi M AD - J.F. Merlen Research Center for vascular diseases, Via Mola-Vecchia, 4, 03100 Frosinone, Italy. Electronic address: maxlucchi@libero.it. FAU - Lucchi, G AU - Lucchi G AD - J.F. Merlen Research Center for vascular diseases, Via Mola-Vecchia, 4, 03100 Frosinone, Italy. Electronic address: gabriella.lucchi@libero.it. FAU - Carbone, I AU - Carbone I AD - Department of Radiological Sciences, Oncology and Pathology, ICOT Hospital, "Sapienza" University of Rome, Via Franco-Faggiana, 1668, 04100 Latina, Italy. Electronic address: iacopo.carbone@uniroma1.it. FAU - Bellini, D AU - Bellini D AD - Department of Radiological Sciences, Oncology and Pathology, ICOT Hospital, "Sapienza" University of Rome, Via Franco-Faggiana, 1668, 04100 Latina, Italy. Electronic address: bellinidavide29@gmail.com. FAU - Polidoro, A AU - Polidoro A AD - Internal Medicine Unit, Department of Medico-Surgical Sciences and Biotechnologies, ICOT Hospital, "Sapienza" University of Rome, Via Franco-Faggiana, 1668, 04100 Latina, Italy. Electronic address: alessandro.polidoro@uniroma1.it. FAU - Ciacciarelli, M AU - Ciacciarelli M AD - Internal Medicine Unit, Department of Medico-Surgical Sciences and Biotechnologies, ICOT Hospital, "Sapienza" University of Rome, Via Franco-Faggiana, 1668, 04100 Latina, Italy. Electronic address: marco.ciacciarelli@uniroma1.it. LA - eng PT - Case Reports PT - Letter DEP - 20200327 PL - France TA - J Med Vasc JT - Journal de medecine vasculaire JID - 101709200 SB - IM MH - Adult MH - Astrocytoma/*complications/diagnostic imaging/surgery MH - Brain Neoplasms/*complications/diagnostic imaging/surgery MH - Female MH - Humans MH - Paraneoplastic Syndromes/diagnosis/*etiology MH - Raynaud Disease/diagnosis/*etiology MH - Treatment Outcome OTO - NOTNLM OT - Astrocytoma OT - Brain tumor OT - Paraneoplastic acral vascular syndrome OT - Raynaud's phenomenon EDAT- 2020/05/14 06:00 MHDA- 2020/09/15 06:00 CRDT- 2020/05/14 06:00 PHST- 2020/01/05 00:00 [received] PHST- 2020/01/23 00:00 [accepted] PHST- 2020/05/14 06:00 [entrez] PHST- 2020/05/14 06:00 [pubmed] PHST- 2020/09/15 06:00 [medline] AID - S2542-4513(20)30227-3 [pii] AID - 10.1016/j.jdmv.2020.03.001 [doi] PST - ppublish SO - J Med Vasc. 2020 May;45(3):161-164. doi: 10.1016/j.jdmv.2020.03.001. Epub 2020 Mar 27. PMID- 25887938 OWN - NLM STAT- MEDLINE DCOM- 20160929 LR - 20151123 IS - 1665-1731 (Electronic) IS - 1665-1731 (Linking) VI - 85 IP - 4 DP - 2015 Oct-Dec TI - [Raynaud's phenomenon secondary to nebivolol]. PG - 342-3 LID - S1405-9940(15)00028-2 [pii] LID - 10.1016/j.acmx.2015.02.002 [doi] FAU - V-Ibarra, Nuria AU - V-Ibarra N AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. FAU - de Lara, Gregorio AU - de Lara G AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. FAU - Pernias, Vicente AU - Pernias V AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. FAU - Nuñez, Laura AU - Nuñez L AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. FAU - García de Burgos, Fernando AU - García de Burgos F AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. FAU - Morillas, Pedro AU - Morillas P AD - Servicio de Cardiología, Hospital General Universitario de Elche, Elche, Alicante, España. Electronic address: pedromorillas68@gmail.com. LA - spa PT - Case Reports PT - Letter TT - Fenómeno de Raynaud secundario a nebivolol. DEP - 20150414 PL - Mexico TA - Arch Cardiol Mex JT - Archivos de cardiologia de Mexico JID - 101126728 RN - 0 (Antihypertensive Agents) RN - 030Y90569U (Nebivolol) SB - IM MH - Adult MH - Antihypertensive Agents/*adverse effects MH - Female MH - Humans MH - Nebivolol/*adverse effects MH - Raynaud Disease/*chemically induced EDAT- 2015/04/19 06:00 MHDA- 2016/09/30 06:00 CRDT- 2015/04/19 06:00 PHST- 2014/07/30 00:00 [received] PHST- 2015/02/15 00:00 [revised] PHST- 2015/02/16 00:00 [accepted] PHST- 2015/04/19 06:00 [entrez] PHST- 2015/04/19 06:00 [pubmed] PHST- 2016/09/30 06:00 [medline] AID - S1405-9940(15)00028-2 [pii] AID - 10.1016/j.acmx.2015.02.002 [doi] PST - ppublish SO - Arch Cardiol Mex. 2015 Oct-Dec;85(4):342-3. doi: 10.1016/j.acmx.2015.02.002. Epub 2015 Apr 14. PMID- 38290764 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20241231 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 8 DP - 2024 Aug 1 TI - Raynaud's of the gut? Systemic sclerosis-associated microvascular ischaemic colitis responsive to intravenous iloprost. PG - e219-e221 LID - 10.1093/rheumatology/keae052 [doi] FAU - Pratap, Krishan AU - Pratap K AUID- ORCID: 0000-0002-1093-5999 AD - Department of Gastroenterology, St Vincent's Hospital, Sydney, NSW, Australia. FAU - Penglase, Ross AU - Penglase R AUID- ORCID: 0000-0002-4061-4301 AD - Department of Rheumatology, St Vincent's Hospital, Sydney, NSW, Australia. FAU - Roper, Edward AU - Roper E AD - Histopath Diagnostic Specialists, Sydney, NSW, Australia. FAU - Stoita, Alina AU - Stoita A AUID- ORCID: 0000-0001-9460-2149 AD - Department of Gastroenterology, St Vincent's Hospital, Sydney, NSW, Australia. LA - eng PT - Case Reports PT - Letter PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - JED5K35YGL (Iloprost) RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - *Colitis, Ischemic/drug therapy/diagnostic imaging MH - *Iloprost/therapeutic use/administration & dosage MH - *Raynaud Disease/drug therapy/etiology MH - *Scleroderma, Systemic/complications/drug therapy MH - *Vasodilator Agents/therapeutic use/administration & dosage EDAT- 2024/01/31 00:42 MHDA- 2024/08/01 06:41 CRDT- 2024/01/30 21:12 PHST- 2024/01/21 00:00 [accepted] PHST- 2024/08/01 06:41 [medline] PHST- 2024/01/31 00:42 [pubmed] PHST- 2024/01/30 21:12 [entrez] AID - 7593758 [pii] AID - 10.1093/rheumatology/keae052 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Aug 1;63(8):e219-e221. doi: 10.1093/rheumatology/keae052. PMID- 27594391 OWN - NLM STAT- MEDLINE DCOM- 20170606 LR - 20170606 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 45 IP - 5 DP - 2016 Sep TI - Nailfold capillaroscopy microscopy - an interdisciplinary appraisal. PG - 353-64 LID - 10.1024/0301-1526/a000553 [doi] AB - Nailfold capillaroscopy is a method of great diagnostic value in the differential diagnosis of primary versus secondary Raynaud´s phenomenon, of systemic sclerosis versus other so called connective tissue diseases and of additional diagnostic value in other entities. Rheumatologists, dermatologists, and angiologists in Germany have convened in an interdisciplinary working group in which they synergistically combined their expertise to develop a common nomenclature and standards for the technical performance of nailfold capillary microscopy. The article gives an overview of historical and technical aspects of capillaroscopy, morphologic findings, and disease-specific patterns. It also provides a critical appraisal of its significance in the diagnosis and sequelae of these interdisciplinarily-managed diseases including its performance in children and gives an excursion in the potential perspectives of capillaroscopy in less common indications. FAU - Klein-Weigel, Peter Franz AU - Klein-Weigel PF AD - 1 Department for Angiology, HELIOS Klinikum Berlin-Buch, Berlin, Germany. FAU - Sunderkötter, Cord AU - Sunderkötter C AD - 2 Department of Dermatology, University Hospital, Münster, Germany. FAU - Sander, Oliver AU - Sander O AD - 3 Policlinic for Rheumatology and Hiller Research Centre for Rheumatology, University Hospital, Düsseldorf, Germany. LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Connective Tissue Diseases/*diagnosis/epidemiology/physiopathology/therapy MH - Diagnosis, Differential MH - Humans MH - Interdisciplinary Communication MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/*diagnosis/epidemiology/physiopathology/therapy OTO - NOTNLM OT - Capillaroscopy OT - Raynaud`s phenomenon OT - connective tissue diseases OT - dermatomyositis OT - systemic lupus erythematosus OT - systemic sclerosis EDAT- 2016/09/07 06:00 MHDA- 2017/06/07 06:00 CRDT- 2016/09/06 06:00 PHST- 2016/09/06 06:00 [entrez] PHST- 2016/09/07 06:00 [pubmed] PHST- 2017/06/07 06:00 [medline] AID - 10.1024/0301-1526/a000553 [doi] PST - ppublish SO - Vasa. 2016 Sep;45(5):353-64. doi: 10.1024/0301-1526/a000553. PMID- 21427808 OWN - NLM STAT- MEDLINE DCOM- 20111025 LR - 20110323 IS - 1427-440X (Print) IS - 1427-440X (Linking) VI - 56 IP - 1 DP - 2010 TI - [Raynaud's phenomenon: pathogenesis and prevalence]. PG - 11-4 AB - Raynaud's phenomenon is defined as occasional ischemia of the distal parts of the extremities. Ischemia may be idiopathic as in primary Raynaud's disease or instigated by a comorbidity as in Raynaud's syndrome. Opinions on the etiopathogenesis of Raynaud's phenomenon have changed during recent years. Research has shown that enhanced vascular reactivity is attributable more to local factors and less to abnormalities in the central nervous system. Local factors are classified as vascular, nervous, and intravascular. Changes in our understanding of the etiopathogenesis of Raynaud's phenomenon have resulted in modified therapeutic guidelines. The present work reviews current opinions on the etiopathogenesis of Raynaud's phenomenon. FAU - Mikulska, Danuta AU - Mikulska D AD - Katedra i Klinika Chorób Skórnych i Wenerycznych Pomorskiej Akademii Medycznej w Szczecinie, al. Powstańców Wlkp. 72, 70-111 Szczecin. LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Objaw Raynauda--patogeneza i wystepowanie. PL - Poland TA - Ann Acad Med Stetin JT - Annales Academiae Medicae Stetinensis JID - 7506854 SB - IM MH - Causality MH - Humans MH - Prevalence MH - Raynaud Disease/*epidemiology EDAT- 2010/01/01 00:00 MHDA- 2011/10/26 06:00 CRDT- 2011/03/25 06:00 PHST- 2011/03/25 06:00 [entrez] PHST- 2010/01/01 00:00 [pubmed] PHST- 2011/10/26 06:00 [medline] PST - ppublish SO - Ann Acad Med Stetin. 2010;56(1):11-4. PMID- 33866879 OWN - NLM STAT- MEDLINE DCOM- 20211124 LR - 20211124 IS - 1938-9116 (Electronic) IS - 1538-5744 (Linking) VI - 55 IP - 7 DP - 2021 Oct TI - Novel Combined Approach for Digital Necrosis Secondary to Raynaud's Phenomenon. PG - 766-771 LID - 10.1177/15385744211005663 [doi] AB - The presence of severe Raynaud's Phenomenon (RP), with permanent pain or digital necrosis is a rare condition. Cervical sympathectomy or distal sympathectomy or A botulinum toxin have demonstrated efficacy after medical treatment failure. We report the case of a 38-year-old female patient with an acute onset of severe RP in both hands secondary to systemic sclerosis. Medical treatment failed, so a novel approach by a combination of a modified distal sympathectomy and injection of A botulinum toxin on digital neuromuscular bundles was performed. Remission of the pain occurred immediately after the procedure and 45 days later she had complete healing of the digital wounds and recovered full mobilization of both hands. The patient remained asymptomatic 6 month after the procedure, and a Doppler ultrasound showed tri-phasic flows distal to the surgical site. This novel technique is described, and a brief review of the literature is performed. FAU - Vegas, Diego Herrera AU - Vegas DH AUID- ORCID: 0000-0001-7669-2988 AD - Centro de Estudios Médicos e Investigaciones Clínicas (C.E.M.I.C.), Buenos Aires, Argentina. FAU - Fabiani, Mario Alejandro AU - Fabiani MA AUID- ORCID: 0000-0002-8548-6833 AD - Tecnologico de Monterrey, School of Medicine and Health Sciences, Monterrey, Nuevo León, México. FAU - Gonzalez-Urquijo, Mauricio AU - Gonzalez-Urquijo M AUID- ORCID: 0000-0001-5101-1541 AD - Tecnologico de Monterrey, School of Medicine and Health Sciences, Monterrey, Nuevo León, México. FAU - Bignotti, Agustín AU - Bignotti A AD - Centro de Estudios Médicos e Investigaciones Clínicas (C.E.M.I.C.), Buenos Aires, Argentina. FAU - Seré, Ignacio AU - Seré I AD - Centro de Estudios Médicos e Investigaciones Clínicas (C.E.M.I.C.), Buenos Aires, Argentina. FAU - Salvadores, Pablo AU - Salvadores P AD - Centro de Estudios Médicos e Investigaciones Clínicas (C.E.M.I.C.), Buenos Aires, Argentina. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210419 PL - United States TA - Vasc Endovascular Surg JT - Vascular and endovascular surgery JID - 101136421 RN - 0 (Acetylcholine Release Inhibitors) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Acetylcholine Release Inhibitors/*administration & dosage MH - Adult MH - Botulinum Toxins, Type A/*administration & dosage MH - Combined Modality Therapy MH - Female MH - Fingers/*blood supply MH - Humans MH - Injections MH - Necrosis MH - Raynaud Disease/diagnosis/etiology/physiopathology/*therapy MH - Recovery of Function MH - Scleroderma, Systemic/*complications/diagnosis MH - Severity of Illness Index MH - *Sympathectomy MH - Treatment Outcome MH - Wound Healing OTO - NOTNLM OT - Raynaud’s phenomenon OT - a botulinum toxin OT - cervical sympathectomy OT - distal sympathectomy OT - scleroderma EDAT- 2021/04/20 06:00 MHDA- 2021/11/25 06:00 CRDT- 2021/04/19 05:26 PHST- 2021/04/20 06:00 [pubmed] PHST- 2021/11/25 06:00 [medline] PHST- 2021/04/19 05:26 [entrez] AID - 10.1177/15385744211005663 [doi] PST - ppublish SO - Vasc Endovascular Surg. 2021 Oct;55(7):766-771. doi: 10.1177/15385744211005663. Epub 2021 Apr 19. PMID- 29425538 OWN - NLM STAT- MEDLINE DCOM- 20180911 LR - 20260127 IS - 2542-4513 (Electronic) IS - 2542-4513 (Linking) VI - 43 IP - 1 DP - 2018 Feb TI - [Etiological profile of secondary Raynaud's phenomenon in an internal medicine department. About 121 patients]. PG - 29-35 LID - S2542-4513(17)30328-0 [pii] LID - 10.1016/j.jdmv.2017.11.005 [doi] AB - INTRODUCTION: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. METHODS: A descriptive retrospective study including patients with secondary Raynaud's phenomenon followed in a tertiary internal medicine department between 2000 and 2013. RESULTS: We included 121 patients. The sex ratio M/F was 0.16. The mean age at the onset of Raynaud's phenomenon was 41.7 years. The average age of patients at the time of the etiologic diagnosis was 47.3 years. The mean delay between Raynaud's phenomenon onset and the first consultation was 41.33 months. Raynaud's phenomenon involved hands in all cases and feet in 16.10% of cases with a typical form in most cases (41.4%). Complications (digital ulcers and scars) were noted in 32.23% of cases. Nail fold capillaroscopy showed scleroderma pattern in 49.52% of patients. Antinuclear antibodies were positive in 88.49% of patients. Interstitial lung disease was reported in 54.04% of cases. Connective tissue diseases were diagnosed in 86.77% of patients. Other secondary Raynaud's phenomenon causes were vasculitis (6.61%), atherosclerosis (1.65%) and medical or professional causes (1.65%). The most frequent one cause systemic sclerosis (n=61, 98%) followed by systemic lupus erythematosus (11.57%) and primary Sjögren syndrome (6.61%). CONCLUSION: In our study, the Raynaud's phenomenon was most frequently secondary to connective tissue diseases. This may be a selection bias because our department is a third-line unit where patients are often referred for systemic disease suspicion. CI - Copyright © 2017 Elsevier Masson SAS. All rights reserved. FAU - Ben Salem, T AU - Ben Salem T AD - Service de médecine interne, La Rabta, Tunis, Tunisie. FAU - Tougorti, M AU - Tougorti M AD - Service de médecine interne, La Rabta, Tunis, Tunisie. Electronic address: molka.tougorti@gmail.com. FAU - Bziouech, S AU - Bziouech S AD - Service de médecine interne, La Rabta, Tunis, Tunisie. FAU - Lamloum, M AU - Lamloum M AD - Service de médecine interne, La Rabta, Tunis, Tunisie. FAU - Khanfir, M AU - Khanfir M AD - Service de médecine interne, La Rabta, Tunis, Tunisie. FAU - Ben Ghorbel, I AU - Ben Ghorbel I AD - Service de médecine interne, La Rabta, Tunis, Tunisie. FAU - Houman, M H AU - Houman MH AD - Service de médecine interne, La Rabta, Tunis, Tunisie. LA - fre PT - Journal Article TT - Profil étiologique d’un phénomène de Raynaud secondaire dans un service de médecine interne. À propos de 121 patients. DEP - 20171220 PL - France TA - J Med Vasc JT - Journal de medecine vasculaire JID - 101709200 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/blood MH - Connective Tissue Diseases/complications/epidemiology/immunology MH - Female MH - Hospital Departments/statistics & numerical data MH - Humans MH - Internal Medicine MH - Lung Diseases, Interstitial/complications/epidemiology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Plaque, Atherosclerotic/complications/epidemiology MH - Raynaud Disease/diagnostic imaging/epidemiology/*etiology MH - Retrospective Studies MH - Tertiary Care Centers/statistics & numerical data MH - Tunisia/epidemiology MH - Vasculitis/complications/epidemiology MH - Young Adult OTO - NOTNLM OT - Connective tissue disease OT - Lupus érythémateux systémique OT - Maladie systémique OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Sclérodermie systémique OT - Sjögren Syndrome OT - Syndrome de Sjögren OT - Systemic lupus erythematosus OT - Systemic sclerosis EDAT- 2018/02/10 06:00 MHDA- 2018/09/12 06:00 CRDT- 2018/02/10 06:00 PHST- 2016/11/05 00:00 [received] PHST- 2017/11/23 00:00 [accepted] PHST- 2018/02/10 06:00 [entrez] PHST- 2018/02/10 06:00 [pubmed] PHST- 2018/09/12 06:00 [medline] AID - S2542-4513(17)30328-0 [pii] AID - 10.1016/j.jdmv.2017.11.005 [doi] PST - ppublish SO - J Med Vasc. 2018 Feb;43(1):29-35. doi: 10.1016/j.jdmv.2017.11.005. Epub 2017 Dec 20. PMID- 30004597 OWN - NLM STAT- MEDLINE DCOM- 20190724 LR - 20191219 IS - 1468-3083 (Electronic) IS - 0926-9959 (Print) IS - 0926-9959 (Linking) VI - 33 IP - 3 DP - 2019 Mar TI - The clinical effects of l-arginine and asymmetric dimethylarginine: implications for treatment in secondary Raynaud's phenomenon. PG - 497-503 LID - 10.1111/jdv.15180 [doi] AB - Secondary Raynaud's phenomenon (RP) is often the sentinel clinical finding in systemic sclerosis and may precede systemic disease by several years. Altered nitric oxide metabolism plays a critical role in both fibrosis and severe secondary RP phenotypes in these patients. Increased flux through inducible nitric oxide synthase (iNOS) drives cutaneous fibrosis. Failure of flux through endothelial nitric oxide synthase (eNOS) contributes to increased vasoconstriction and decreased vasorelaxation. The underproduction of nitric oxide by eNOS is in part due to increased levels of asymmetric dimethylarginine (ADMA), an endogenous competitive inhibitor of nitric oxide synthase. The inhibitory effects of increased ADMA levels may be counteracted increasing serum l-arginine, which is often an effective treatment strategy in these patients. As such, l-arginine-based therapies should be considered in managing secondary RP, particularly given their favourable safety and tolerability profile. While there is no established dosing regimen, studies of oral l-arginine in secondary RP suggest that divided dosing may begin at 1-2 g/day and may be titrated up to 10 g/day. Conversely, primary RP is not associated with increased ADMA production which likely accounts for the failure of l-arginine trials to show benefit in primary RP. CI - © 2018 European Academy of Dermatology and Venereology. FAU - Curtiss, P AU - Curtiss P AUID- ORCID: 0000-0001-8850-6011 AD - Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA. FAU - Schwager, Z AU - Schwager Z AD - Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA. FAU - Lo Sicco, K AU - Lo Sicco K AD - Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA. FAU - Franks, A G Jr AU - Franks AG Jr AD - Skin Lupus & Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA. AD - Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, NY, USA. LA - eng GR - Frances & Benjamin Benenson Foundation/ GR - Lynn & William M. Silverman Foundation/ PT - Journal Article PT - Review DEP - 20180828 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 RN - 31C4KY9ESH (Nitric Oxide) RN - 63CV1GEK3Y (N,N-dimethylarginine) RN - 94ZLA3W45F (Arginine) RN - EC 1.14.13.39 (Nitric Oxide Synthase Type III) MH - Arginine/*analogs & derivatives/blood/*therapeutic use MH - Humans MH - Nitric Oxide/*metabolism MH - Nitric Oxide Synthase Type III/metabolism MH - Raynaud Disease/*blood/*drug therapy/etiology MH - Scleroderma, Systemic/complications/*metabolism MH - Vasodilation PMC - PMC6916181 EDAT- 2018/07/14 06:00 MHDA- 2019/07/25 06:00 PMCR- 2019/12/17 CRDT- 2018/07/14 06:00 PHST- 2018/02/05 00:00 [received] PHST- 2018/07/06 00:00 [accepted] PHST- 2018/07/14 06:00 [pubmed] PHST- 2019/07/25 06:00 [medline] PHST- 2018/07/14 06:00 [entrez] PHST- 2019/12/17 00:00 [pmc-release] AID - JDV15180 [pii] AID - 10.1111/jdv.15180 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2019 Mar;33(3):497-503. doi: 10.1111/jdv.15180. Epub 2018 Aug 28. PMID- 34636314 OWN - NLM STAT- MEDLINE DCOM- 20220412 LR - 20220412 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 18 IP - 1 DP - 2022 TI - Evaluation of Botulinum Toxin Type A and its Potential Effect on Exacerbated Raynaud's Phenomenon in Hospitalized Scleroderma Patients. PG - 48-57 LID - 10.2174/1573397117666211012105611 [doi] AB - BACKGROUND/AIMS: Raynaud's phenomenon by episodically reversible constriction of the arteries in the fingers and toes causes pain, numbness, sores, and gangrene. However, the treatment of Raynaud's phenomenon is one of the clinical issues. Recent studies have shown that botulinum toxin is considered a potential and effective therapeutic option for improving finger blood circulation in patients with Raynaud's syndrome. In this study, we sought to investigate the therapeutic effect of botulinum toxin type A on exacerbated Raynaud's phenomenon in patients with scleroderma. METHODS: In this prospective study, 11 patients with systemic scleroderma who were referred due to aggravated Raynaud's were included. For all patients, questionnaires were filled up, and physical examination was performed separately for both treatment and control hands, and then similar volumes of botulinum toxin type A (Botox) and normal saline were randomly injected. RESULTS: The results showed that there was a significant difference in Raynaud's score (P = 0.001), Quick-Dash score (P = 0.01), Mc-Cabe cold score (P = 0.003), the mean frequency of recurrences arracks (P = 0.01), pain (0.005) (P = 0), skin color (P = 0.01), and duration of Raynaud's phenomenon (P = 0.006) between the intervention and control groups after two months. CONCLUSION: Following Botox injection, a significant improvement in terms of various Raynaud's parameters as well as the clinical manifestations was observed in the intervention group. Together, botulinum toxin type A could retrieve the hand function, the cold sensitivity, and the painful feeling caused by Raynaud's syndrome. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Seyedmardani, Seyed Mostafa AU - Seyedmardani SM AD - Internal Medicine Department, Urmia University of Medical Sciences, Urmia, Iran. FAU - Aghdashi, Mir Amir AU - Aghdashi MA AD - Internal Medicine Department, Urmia University of Medical Sciences, Urmia, Iran. FAU - Soltani, Shaida AU - Soltani S AD - Internal Medicine Department, Urmia University of Medical Sciences, Urmia, Iran. FAU - Zonouz, Golshan Kamali AU - Zonouz GK AD - Emergency Medicine Department, Urmia University of Medical Sciences, Urmia, Iran. LA - eng PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/therapeutic use MH - Hand MH - Humans MH - Pain MH - Prospective Studies MH - *Raynaud Disease/diagnosis/drug therapy/etiology MH - *Scleroderma, Localized OTO - NOTNLM OT - Botox OT - botulinum toxin A OT - parameters OT - raynaud's syndrome OT - scleroderma OT - therapeutic intervention EDAT- 2021/10/13 06:00 MHDA- 2022/04/13 06:00 CRDT- 2021/10/12 08:41 PHST- 2020/11/20 00:00 [received] PHST- 2021/02/23 00:00 [revised] PHST- 2021/06/21 00:00 [accepted] PHST- 2021/10/13 06:00 [pubmed] PHST- 2022/04/13 06:00 [medline] PHST- 2021/10/12 08:41 [entrez] AID - CRR-EPUB-118431 [pii] AID - 10.2174/1573397117666211012105611 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2022;18(1):48-57. doi: 10.2174/1573397117666211012105611. PMID- 38630277 OWN - NLM STAT- MEDLINE DCOM- 20240418 LR - 20240604 IS - 1432-069X (Electronic) IS - 0340-3696 (Linking) VI - 316 IP - 5 DP - 2024 Apr 17 TI - Efficacy and safety of botulinum toxin in treating scleroderma-associated raynaud's phenomenon: a systematic review and meta-analysis. PG - 122 LID - 10.1007/s00403-024-02864-x [doi] FAU - Elrosasy, Amr AU - Elrosasy A AD - Faculty of Medicine, Cairo University, Cairo, Egypt. AD - Medical research group of Egypt (MRGE), Negida Academy, Arlington, MA, US. FAU - Abo Zeid, Mohamed AU - Abo Zeid M AD - Faculty of Medicine, Tanta University, Tanta, Egypt. Mohamed_31059599@med.tanta.edu.eg. FAU - Cadri, Shirin AU - Cadri S AD - University of medicine and pharmacy Grigore T. Popa, Ia?i, Romania. FAU - Fahmy, Belal M AU - Fahmy BM AD - faculty of medicine, Ain shams university, Ain shams, Egypt. FAU - Elzeftawy, Mohamed A AU - Elzeftawy MA AD - Faculty of Medicine, Tanta University, Tanta, Egypt. FAU - Mohammed, Fatma AU - Mohammed F AD - Faculty of Medicine, Aswan University, Aswan, Egypt. FAU - Ramadan, Alaa AU - Ramadan A AD - Faculty of Medicine, South Valley University, Qena, Egypt. LA - eng PT - Letter PT - Meta-Analysis PT - Systematic Review DEP - 20240417 PL - Germany TA - Arch Dermatol Res JT - Archives of dermatological research JID - 8000462 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - Humans MH - *Botulinum Toxins/therapeutic use MH - *Skin Diseases/complications MH - *Raynaud Disease/drug therapy OTO - NOTNLM OT - Botulinum Toxin OT - Raynaud’s phenomena OT - Scleroderma OT - Systemic Sclerosis EDAT- 2024/04/17 12:43 MHDA- 2024/04/18 06:43 CRDT- 2024/04/17 11:14 PHST- 2023/10/25 00:00 [received] PHST- 2024/04/07 00:00 [accepted] PHST- 2024/01/26 00:00 [revised] PHST- 2024/04/18 06:43 [medline] PHST- 2024/04/17 12:43 [pubmed] PHST- 2024/04/17 11:14 [entrez] AID - 10.1007/s00403-024-02864-x [pii] AID - 10.1007/s00403-024-02864-x [doi] PST - epublish SO - Arch Dermatol Res. 2024 Apr 17;316(5):122. doi: 10.1007/s00403-024-02864-x. PMID- 36209513 OWN - NLM STAT- MEDLINE DCOM- 20230309 LR - 20231221 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 75 IP - 3 DP - 2023 Mar TI - Botulinum toxin for Raynaud's phenomenon associated with systemic sclerosis: comment on the article by Senet et al. PG - 486 LID - 10.1002/art.42379 [doi] FAU - Yin, Hanlin AU - Yin H AUID- ORCID: 0000-0003-0706-3998 AD - Department of Rheumatology Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Liu, Bei AU - Liu B AD - Department of Rheumatology Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Li, Qianqian AU - Li Q AD - Department of Rheumatology Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Yan, Qingran AU - Yan Q AUID- ORCID: 0000-0002-2781-5280 AD - Department of Rheumatology Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Lu, Liangjing AU - Lu L AUID- ORCID: 0000-0001-9116-6038 AD - Department of Rheumatology Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. LA - eng PT - Comment PT - Letter DEP - 20230119 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM CON - Arthritis Rheumatol. 2023 Mar;75(3):459-467. doi: 10.1002/art.42342. PMID: 36066501 CIN - Arthritis Rheumatol. 2023 Mar;75(3):486-487. doi: 10.1002/art.42377. PMID: 36209510 MH - Humans MH - *Botulinum Toxins MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/complications/drug therapy EDAT- 2022/10/10 06:00 MHDA- 2023/03/10 06:00 CRDT- 2022/10/09 16:59 PHST- 2022/09/10 00:00 [received] PHST- 2022/09/27 00:00 [accepted] PHST- 2022/10/10 06:00 [pubmed] PHST- 2023/03/10 06:00 [medline] PHST- 2022/10/09 16:59 [entrez] AID - 10.1002/art.42379 [doi] PST - ppublish SO - Arthritis Rheumatol. 2023 Mar;75(3):486. doi: 10.1002/art.42379. Epub 2023 Jan 19. PMID- 22286643 OWN - NLM STAT- MEDLINE DCOM- 20120529 LR - 20211203 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 52 IP - 1 DP - 2012 Jan-Feb TI - CysLT1 receptor inhibition in patients with Raynaud's phenomenon: capillaroscopic evidence of the role of leukotriene. PG - 30-2 LID - S0482-50042012000100004 [pii] AB - OBJECTIVE: To assess the effect of the leukotriene receptor inhibitor (montelukast) on vascular alterations in fingers of patients with Raynaud's phenomenon. METHODS: Patients with Raynaud's phenomenon of the hands secondary to inflammatory connective tissue disease were selected, and those with the following characteristics were excluded: smokers, arterial hypertension, and diabetes mellitus. All patients maintained their previous medications and started the use of montelukast, 10 mg/day, for 60 days. Naifold capillaroscopy of fingers was performed before the use of medication and after 30 and 60 days. Statistical analysis was performed with percentage, media, standard deviation, Fisher exact test, with 95% of confidence interval. RESULTS: The study assessed five Caucasian, female patients with Raynaud's phenomenon secondary to inflammatory connective tissue disease (three with scleroderma and two with mixed connective tissue disease), aged 42.4 ± 11.5 years, and with 9.6 ± 4.8 years of disease duration. Patients were on nifedipine and pentoxifylline, and those with mixed connective tissue disease were also on prednisone. The medications were maintained. After using montelukast for two months, nailfold capillaroscopy showed a reduction in edema and pallor, and normalization of capillary number, size, and distribution. CONCLUSION: The use of montelukast modified the capillary abnormalities observed on nailfold capillaroscopy of patients with Raynaud's phenomenon. FAU - Azevedo, Mario Newton Leitão de AU - Azevedo MN AD - Universidade Federal do Rio de Janeiro. mnazevedo@uol.com.br FAU - Bernardini, Edda Maria T AU - Bernardini EM FAU - Salles, Elizabeth F AU - Salles EF FAU - Heinzmann, Felipe AU - Heinzmann F FAU - Marconde, Frederico AU - Marconde F FAU - Mello, Tatiana AU - Mello T FAU - Gomes, Blanca Elena Rios AU - Gomes BE LA - eng LA - por PT - Journal Article PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0 (Acetates) RN - 0 (Cyclopropanes) RN - 0 (Leukotriene Antagonists) RN - 0 (Quinolines) RN - 0 (Receptors, Leukotriene) RN - 0 (Sulfides) RN - LRF7RW46ID (leukotriene D4 receptor) RN - MHM278SD3E (montelukast) SB - IM MH - Acetates/*therapeutic use MH - Adult MH - Cyclopropanes MH - Female MH - Humans MH - Leukotriene Antagonists/*therapeutic use MH - *Microscopic Angioscopy MH - Prospective Studies MH - Quinolines/*therapeutic use MH - Raynaud Disease/*diagnosis/*drug therapy MH - Receptors, Leukotriene/*drug effects MH - Sulfides EDAT- 2012/01/31 06:00 MHDA- 2012/05/30 06:00 CRDT- 2012/01/31 06:00 PHST- 2011/02/22 00:00 [received] PHST- 2011/11/02 00:00 [accepted] PHST- 2012/01/31 06:00 [entrez] PHST- 2012/01/31 06:00 [pubmed] PHST- 2012/05/30 06:00 [medline] AID - S0482-50042012000100004 [pii] PST - ppublish SO - Rev Bras Reumatol. 2012 Jan-Feb;52(1):30-2. PMID- 30392649 OWN - NLM STAT- MEDLINE DCOM- 20190304 LR - 20190304 IS - 1532-3374 (Electronic) IS - 0959-289X (Linking) VI - 36 DP - 2018 Nov TI - New onset acute pulmonary edema after methylergonovine given during cesarean delivery of a patient with undiagnosed Raynaud's disease. PG - 111-114 LID - S0959-289X(18)30034-7 [pii] LID - 10.1016/j.ijoa.2018.05.006 [doi] AB - Raynaud's disease is a medical condition in which arterial spasm causes episodes of reduced blood flow, in the setting of certain triggers such as cold weather. Patients with this condition are at risk of adverse reactions if they receive medications with vasoactive properties. Methylergonovine maleate is one drug used during cesarean delivery to treat postpartum hemorrhage due to uterine atony. By acting directly on uterine and vascular smooth muscle, it produces cardiovascular responses such as coronary vasospasm, myocardial infarction, and even cardiac arrest. However, pulmonary events have rarely been reported. We report our anesthetic management of a 36-year-old patient, with undiagnosed Raynaud's disease and undergoing cesarean delivery, who experienced new onset acute pulmonary edema after methylergonovine administration to manage postpartum hemorrhage. CI - Copyright © 2018 Elsevier Ltd. All rights reserved. FAU - Kim, I AU - Kim I AD - The Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, MD 21287, USA. Electronic address: inkim@jhmi.edu. FAU - Lindeman, K AU - Lindeman K AD - The Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, MD 21287, USA. FAU - Masear, C AU - Masear C AD - The Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, MD 21287, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20180529 PL - Netherlands TA - Int J Obstet Anesth JT - International journal of obstetric anesthesia JID - 9200430 RN - 0 (Oxytocics) RN - W53L6FE61V (Methylergonovine) SB - IM MH - Acute Disease MH - Adult MH - *Cesarean Section MH - Diagnosis, Differential MH - Female MH - Humans MH - Methylergonovine/*adverse effects MH - Oxytocics/*adverse effects MH - Pregnancy MH - Pregnancy Complications, Cardiovascular/*diagnosis MH - Pulmonary Edema/*chemically induced MH - Raynaud Disease/complications/*diagnosis OTO - NOTNLM OT - Cesarean delivery OT - Methylergonovine OT - Pulmonary edema OT - Raynaud’s disease EDAT- 2018/11/06 06:00 MHDA- 2019/03/05 06:00 CRDT- 2018/11/06 06:00 PHST- 2018/01/17 00:00 [received] PHST- 2018/05/11 00:00 [revised] PHST- 2018/05/21 00:00 [accepted] PHST- 2018/11/06 06:00 [entrez] PHST- 2018/11/06 06:00 [pubmed] PHST- 2019/03/05 06:00 [medline] AID - S0959-289X(18)30034-7 [pii] AID - 10.1016/j.ijoa.2018.05.006 [doi] PST - ppublish SO - Int J Obstet Anesth. 2018 Nov;36:111-114. doi: 10.1016/j.ijoa.2018.05.006. Epub 2018 May 29. PMID- 32152916 OWN - NLM STAT- MEDLINE DCOM- 20210409 LR - 20210409 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 39 IP - 8 DP - 2020 Aug TI - A systematic review of internet-based information for individuals with Raynaud's phenomenon and patients with systemic sclerosis. PG - 2363-2367 LID - 10.1007/s10067-020-05023-5 [doi] AB - INTRODUCTION: Patients are increasingly using internet-based information to inform healthcare utilization and treatment decisions. Our aim was to examine the quality and readability of internet-based information relating to Raynaud's phenomenon (RP) and systemic sclerosis (SSc). MATERIAL AND METHODS: A systematic review of three commonly used search engines (Google®, Yahoo®, and Bing®) using the terms "Raynaud's phenomenon" and "systemic sclerosis" separately. The first 30 websites per search engine were examined. Quality was assessed using the DISCERN questionnaire and readability by the Flesch-Kincaid Grade Level, SMOG Index, Coleman-Liau index, and Flesch Reading Ease score. RESULTS: Fifty-two studies (30 RP and 22 SSc) were included after duplicates and exclusion criteria were applied. The overall quality of information was low for both SSc and RP (1.99 & 2.21), including in relation to reliability of the literature and information on treatment choices. Readability for RP and SSc was also poor (i.e., the texts were difficult to read) across all of the four methods examined. CONCLUSION: Overall, RP and SSc internet-based information is of low quality and inadequate readability. The RP and SSc international community should strongly consider developing an information standard for internet-based resources for healthcare users. KEY POINTS: • Patients with SSc and RP are increasingly using internet/online sources of information and support. • RP represents an important opportunity for the early diagnosis of SSc. • The overall quality and readability of internet-based RP and SSc information is poor. • Internet-based RP and SSc information requires improvement to facilitate early diagnosis and inform shared decision-making. FAU - Devgire, Vikrant AU - Devgire V AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, S10 2JF, UK. FAU - Martin, Andreas Flores AU - Martin AF AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, S10 2JF, UK. FAU - McKenzie, Lorraine AU - McKenzie L AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester, UK. FAU - Sandler, Robert D AU - Sandler RD AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, S10 2JF, UK. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, S10 2JF, UK. Michael.hughes-6@postgrad.manchester.ac.uk. AD - Centre for Musculoskeletal Research, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK. Michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Journal Article PT - Systematic Review DEP - 20200309 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM EIN - Clin Rheumatol. 2020 Jul;39(7):2247. doi: 10.1007/s10067-020-05101-8. PMID: 32394217 MH - *Comprehension MH - Consumer Health Information/*standards/statistics & numerical data MH - Humans MH - *Internet MH - Raynaud Disease/diagnosis/*therapy MH - Reading MH - Scleroderma, Systemic/diagnosis/*therapy MH - Search Engine OTO - NOTNLM OT - Information OT - Internet OT - Quality OT - Raynaud’s phenomenon OT - Readability OT - Systemic sclerosis EDAT- 2020/03/11 06:00 MHDA- 2021/04/10 06:00 CRDT- 2020/03/11 06:00 PHST- 2020/01/27 00:00 [received] PHST- 2020/02/28 00:00 [accepted] PHST- 2020/02/22 00:00 [revised] PHST- 2020/03/11 06:00 [pubmed] PHST- 2021/04/10 06:00 [medline] PHST- 2020/03/11 06:00 [entrez] AID - 10.1007/s10067-020-05023-5 [pii] AID - 10.1007/s10067-020-05023-5 [doi] PST - ppublish SO - Clin Rheumatol. 2020 Aug;39(8):2363-2367. doi: 10.1007/s10067-020-05023-5. Epub 2020 Mar 9. PMID- 28960445 OWN - NLM STAT- MEDLINE DCOM- 20181023 LR - 20181023 IS - 1098-2353 (Electronic) IS - 0897-3806 (Linking) VI - 31 IP - 5 DP - 2018 Jul TI - Vascular branches from cutaneous nerve of the forearm and hand: Application to better understanding raynaud's disease. PG - 734-741 LID - 10.1002/ca.22993 [doi] AB - Cutaneous nerves have branches called vascular branches (VBs) that reach arteries. VBs are thought to be involved in arterial constriction, and this is the rationale for periarterial sympathectomy as a treatment option for Raynaud's disease. However, the branching patterns and distribution areas of the VBs remain largely unclear. The aim of the present study was to investigate the anatomical structures of the VBs of the cutaneous nerves. Forty hands and forearms were examined to assess the branching patterns and distribution areas of the VBs of the superficial branch of the radial nerve (SBRN), the lateral antebrachial cutaneous nerve (LACN), the medial antebrachial cutaneous nerve (MACN), and the palmar cutaneous branch of the ulnar nerve (PCUN). VBs reaching the radial and ulnar arteries were observed in all specimens. The branching patterns were classified into six types. The mean distance between the radial styloid process and the point where the VBs reached the radial artery was 34.3 ± 4.8 mm in the SBRN and 38.5 ± 15.8 mm in the LACN. The mean distance between the ulnar styloid process and the point where the VBs reached the ulnar artery was 60.3 ± 25.9 mm in the MACN and 43.8 ± 26.0 mm in the PCUN. This study showed that the VBs of the cutaneous nerves have diverse branching patterns. The VBs of the SBRN had a more limited distribution areas than those of the other nerves. Clin. Anat. 31:734-741, 2018. © 2017 Wiley Periodicals, Inc. CI - © 2017 Wiley Periodicals, Inc. FAU - Umemoto, K AU - Umemoto K AUID- ORCID: 0000-0001-5855-5496 AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Ohmichi, M AU - Ohmichi M AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Ohmichi, Y AU - Ohmichi Y AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Yakura, T AU - Yakura T AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Hammer, N AU - Hammer N AD - Department of Anatomy, University of Otago, Dunedin, Otago, New Zealand. FAU - Mizuno, D AU - Mizuno D AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Naito, M AU - Naito M AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. FAU - Nakano, T AU - Nakano T AD - Department of Anatomy, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan. LA - eng PT - Journal Article DEP - 20171208 PL - United States TA - Clin Anat JT - Clinical anatomy (New York, N.Y.) JID - 8809128 SB - IM MH - Aged MH - Aged, 80 and over MH - Female MH - Forearm/*blood supply/innervation MH - Hand/*blood supply/innervation MH - Humans MH - Male MH - Radial Artery/*innervation MH - Raynaud Disease/surgery MH - Ulnar Artery/*innervation OTO - NOTNLM OT - Raynaud's disease OT - innervation OT - radial artery OT - sympathectomy OT - ulnar artery EDAT- 2017/09/30 06:00 MHDA- 2018/10/24 06:00 CRDT- 2017/09/30 06:00 PHST- 2017/08/10 00:00 [received] PHST- 2017/09/15 00:00 [revised] PHST- 2017/09/27 00:00 [accepted] PHST- 2017/09/30 06:00 [pubmed] PHST- 2018/10/24 06:00 [medline] PHST- 2017/09/30 06:00 [entrez] AID - 10.1002/ca.22993 [doi] PST - ppublish SO - Clin Anat. 2018 Jul;31(5):734-741. doi: 10.1002/ca.22993. Epub 2017 Dec 8. PMID- 29408338 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20220410 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 78 IP - 6 DP - 2018 Jun TI - A systematic review and meta-analysis of the effects of topical nitrates in the treatment of primary and secondary Raynaud's phenomenon. PG - 1110-1118.e3 LID - S0190-9622(18)30172-5 [pii] LID - 10.1016/j.jaad.2018.01.043 [doi] AB - BACKGROUND: Multiple placebo-controlled trials have assessed locally applied topical nitrate preparations in treating Raynaud's phenomenon (RP). OBJECTIVES: The objective of this meta-analysis was to assess the effects of local topical nitrates in primary and secondary RP with respect to a combined end point integrating parameters of digital blood flow and clinical severity. METHODS: A systematic review was performed using MEDLINE, Embase, and the Cochrane library. Only trials comparing locally applied topical nitrates with placebo comparators were included. Studies were appraised for bias by 2 independent reviewers. RESULTS: A total of 7 placebo-controlled trials including 346 patients were used in the meta-analysis; 4 trials used nitroglycerin ointments, 2 used the nitroglycerin gel vehicle MQX-503, and 1 used compounded nitrite. The meta-analysis results supported a moderate-to-large treatment effect in RP (standardized mean difference [SMD] = 0.70; 95% CI, 0.35-1.05; P < .0001). Subgroup analyses showed a large treatment effect in secondary RP (SMD = 0.95; 95% CI, 0.25-1.65; P = .008) and moderate effect in primary RP (SMD = 0.45; 95% CI, 0.05-0.85; P = .03). LIMITATIONS: Limitations include the inclusion of multiple topical nitrate preparations and integration of different outcomes assessments. CONCLUSION: Local topical nitrates have significant efficacy in the treatment of both primary and secondary RP. CI - Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Curtiss, Paul AU - Curtiss P AD - Skin Lupus and Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Schwager, Zachary AU - Schwager Z AD - Skin Lupus and Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Cobos, Gabriela AU - Cobos G AD - Skin Lupus and Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Lo Sicco, Kristen AU - Lo Sicco K AD - Skin Lupus and Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Franks, Andrew G Jr AU - Franks AG Jr AD - Skin Lupus and Autoimmune Connective Tissue Section, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York; Division of Rheumatology, The Department of Internal Medicine, New York University School of Medicine, New York, New York. Electronic address: Andrew.Franks@nyumc.org. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20180331 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Nitrates) SB - IM MH - Administration, Topical MH - Controlled Clinical Trials as Topic MH - Female MH - Humans MH - Male MH - Nitrates/adverse effects/*therapeutic use MH - *Patient Safety MH - Prognosis MH - Raynaud Disease/*diagnosis/*drug therapy MH - Severity of Illness Index MH - Treatment Outcome OTO - NOTNLM OT - Raynaud's phenomenon OT - connective tissue disease OT - medical dermatology OT - meta-analysis OT - scleroderma EDAT- 2018/02/07 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/02/07 06:00 PHST- 2017/10/09 00:00 [received] PHST- 2018/01/21 00:00 [revised] PHST- 2018/01/27 00:00 [accepted] PHST- 2018/02/07 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/02/07 06:00 [entrez] AID - S0190-9622(18)30172-5 [pii] AID - 10.1016/j.jaad.2018.01.043 [doi] PST - ppublish SO - J Am Acad Dermatol. 2018 Jun;78(6):1110-1118.e3. doi: 10.1016/j.jaad.2018.01.043. Epub 2018 Mar 31. PMID- 24732584 OWN - NLM STAT- MEDLINE DCOM- 20160226 LR - 20150130 IS - 1827-1839 (Electronic) IS - 0392-9590 (Linking) VI - 34 IP - 1 DP - 2015 Feb TI - Antioxidant agents help primary Raynaud's phenomenon. PG - 94-5 FAU - Bruni, C AU - Bruni C AD - Division of Internal Medicine and Rheumatology, Department of Experimental and Clinical Medicine, Careggi Hospital, University of Florence, Florence, Italy - cosimobruni85@gmail.com. FAU - Lepri, G AU - Lepri G FAU - Peruzzi, F AU - Peruzzi F FAU - Bellando-Randone, S AU - Bellando-Randone S FAU - Radicati, A AU - Radicati A FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M FAU - Guiducci, S AU - Guiducci S LA - eng PT - Letter DEP - 20140414 PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 RN - 0 (Antioxidants) RN - 0 (Calcium Channel Blockers) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Antioxidants/*therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Drug Therapy, Combination MH - Female MH - Humans MH - Male MH - Microcirculation/*drug effects MH - Middle Aged MH - Nails/*blood supply MH - Nifedipine/therapeutic use MH - Raynaud Disease/diagnosis/*drug therapy/physiopathology MH - Retrospective Studies MH - Time Factors MH - Treatment Outcome MH - Vascular Remodeling/drug effects EDAT- 2014/04/16 06:00 MHDA- 2016/02/27 06:00 CRDT- 2014/04/16 06:00 PHST- 2014/04/16 06:00 [entrez] PHST- 2014/04/16 06:00 [pubmed] PHST- 2016/02/27 06:00 [medline] AID - R34Y9999N00A140009 [pii] PST - ppublish SO - Int Angiol. 2015 Feb;34(1):94-5. Epub 2014 Apr 14. PMID- 18329534 OWN - NLM STAT- MEDLINE DCOM- 20080506 LR - 20250529 IS - 0889-857X (Print) IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 34 IP - 1 DP - 2008 Feb TI - Regulation of vascular reactivity in scleroderma: new insights into Raynaud's phenomenon. PG - 81-7; vii LID - 10.1016/j.rdc.2007.12.005 [doi] AB - Because of the role of the RhoA/Rho kinase (ROCK) pathway in regulating numerous pathologic processes including vasoconstriction, vascular remodeling, and fibrosis, ROCK inhibitors may be especially beneficial in treating Raynaud's phenomenon and scleroderma. FAU - Flavahan, Nicholas A AU - Flavahan NA AD - Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Ross Research Building R370, 720 Rutland Avenue, Baltimore, MD 21205, USA. LA - eng GR - R01 HL080119/HL/NHLBI NIH HHS/United States GR - R01 OH008531/OH/NIOSH CDC HHS/United States GR - HL080119/HL/NHLBI NIH HHS/United States GR - OH008531/OH/NIOSH CDC HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 RN - 0 (Reactive Oxygen Species) RN - 0 (Receptors, Adrenergic) RN - EC 2.7.11.1 (rho-Associated Kinases) SB - IM MH - Cold Temperature/adverse effects MH - Humans MH - Muscle, Smooth, Vascular/*physiopathology MH - Raynaud Disease/enzymology/etiology/*physiopathology MH - Reactive Oxygen Species MH - Receptors, Adrenergic/physiology MH - Scleroderma, Systemic/complications/*physiopathology MH - Signal Transduction MH - Skin Temperature/physiology MH - rho-Associated Kinases/physiology PMC - PMC2336896 MID - NIHMS45309 EDAT- 2008/03/11 09:00 MHDA- 2008/05/07 09:00 PMCR- 2009/02/01 CRDT- 2008/03/11 09:00 PHST- 2008/03/11 09:00 [pubmed] PHST- 2008/05/07 09:00 [medline] PHST- 2008/03/11 09:00 [entrez] PHST- 2009/02/01 00:00 [pmc-release] AID - S0889-857X(07)00104-4 [pii] AID - 10.1016/j.rdc.2007.12.005 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2008 Feb;34(1):81-7; vii. doi: 10.1016/j.rdc.2007.12.005. PMID- 22828684 OWN - NLM STAT- MEDLINE DCOM- 20121130 LR - 20120725 IS - 2150-1149 (Electronic) IS - 1533-3159 (Linking) VI - 15 IP - 4 DP - 2012 Jul-Aug TI - Cervical spinal cord stimulation with 5-column paddle lead in Raynaud's disease. PG - 303-9 AB - OBJECTIVE: To report a case of Raynaud disease and its successful treatment with spinal cord stimulation utilizing the newly designed five-column Penta lead paddle. Specific electrode design, programming characteristics, and surgical technique are also discussed in this case. DESIGN: Case Report. SETTING: University pain management center. BACKGROUND: A 65-year-old man with Raynaud disease presented with neck and upper extremity pain. The patient also had herniation and spondylosis of the lumbar spine and intervertebral disc disease of the cervical spine. An examination revealed venous changes, chronic ulceration, and digit discoloration in upper and lower extremities. METHOD: Conservative management and pharmacological treatment were ineffective. Sympathetic block produced significant but limited improvement. Treatment with spinal cord stimulation was tried after a successful 7-day trial. RESULTS: Initial stimulation of the cervical spine with two octapolar leads at the C2 level produced greater than 75% pain improvement. However, the patient lost coverage shortly after discharge due to lead migration which could not be regained with reprogramming. A revision with Penta lead paddles produced sustainable and significant paresthesia coverage. LIMITATIONS: A case report. CONCLUSION: We report the successful application of spinal cord stimulation utilizing a five-column paddle lead in an individual with severe refractory Raynaud disease. FAU - Issa, Mohammed A AU - Issa MA AD - Yale University School of Medicine, West Haven, CT, USA. drissa80@gmail.com FAU - Kim, Chong H AU - Kim CH LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pain Physician JT - Pain physician JID - 100954394 SB - IM MH - Aged MH - Cervical Vertebrae MH - Electrodes, Implanted MH - Humans MH - Male MH - Neck Pain/etiology/therapy MH - Raynaud Disease/complications/*therapy MH - Spinal Cord Stimulation/*instrumentation/*methods EDAT- 2012/07/26 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/07/26 06:00 PHST- 2012/07/26 06:00 [entrez] PHST- 2012/07/26 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] PST - ppublish SO - Pain Physician. 2012 Jul-Aug;15(4):303-9. PMID- 29306224 OWN - NLM STAT- MEDLINE DCOM- 20181224 LR - 20191210 IS - 1300-0144 (Print) IS - 1300-0144 (Linking) VI - 47 IP - 6 DP - 2017 Dec 19 TI - Plasma urotensin II levels in primary Raynaud's phenomenon and systemic sclerosis. PG - 1687-1692 LID - 10.3906/sag-1702-23 [doi] AB - Background/aim: The pathogenesis of Raynaud's phenomenon (RP) has not yet been fully elucidated. RP is characterized by exaggerated cold-induced vasoconstriction. Urotensin II (UII) is a potent vasoconstrictor. The aim of the present study was to evaluate plasma UII levels in both primary RP and secondary RP associated with systemic sclerosis (SSc).Materials and methods: Fifteen patients with primary RP, 30 patients with RP secondary to SSc, and 30 healthy controls (HC) were included in the study. Raynaud condition scores (RCS) were determined in the primary RP and SSc groups. Modified Rodnan skin score (MRSS) was determined for the SSc patients. Plasma UII level was analyzed by the ELISA method. Results: When compared to the HC group, plasma UII level was lower in the secondary RP group, but not in the primary RP group. Plasma UII level was not directly related to RCS in either the primary or secondary RP group. Moreover, it was not correlated with MRSS in the secondary RP group.Conclusion: The results of the present study suggest that UII is not associated with primary RP. Its level was lower in the secondary RP (SSc) patients. Therefore, it can be concluded that decreased UII level is related to SSc instead of RP. FAU - Gözel, Nevzat AU - Gözel N FAU - Karataş, Ahmet AU - Karataş A FAU - Yardım, Meltem AU - Yardım M FAU - Kınacı, Mesude Seda AU - Kınacı MS FAU - Ulu, Ramazan AU - Ulu R FAU - Demircan, Fatih AU - Demircan F FAU - Kılınç, Faruk AU - Kılınç F FAU - Öz, Burak AU - Öz B FAU - Dönder, Emir AU - Dönder E FAU - Aydın, Süleyman AU - Aydın S FAU - Koca, Süleyman Serdar AU - Koca SS LA - eng PT - Comparative Study PT - Journal Article DEP - 20171219 PL - Turkey TA - Turk J Med Sci JT - Turkish journal of medical sciences JID - 9441758 RN - 0 (Biomarkers) RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (Peptide Hormones) RN - 0 (UTS2B protein, human) RN - 0 (Urotensins) RN - 9047-55-6 (urotensin II) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Biomarkers/blood MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Intracellular Signaling Peptides and Proteins MH - Male MH - Middle Aged MH - Peptide Hormones/blood MH - Raynaud Disease/*blood/physiopathology MH - Scleroderma, Systemic/*blood/physiopathology MH - Severity of Illness Index MH - Urotensins/*blood MH - Vasoconstriction/*physiology MH - Young Adult OTO - NOTNLM OT - *Urotensin II OT - *Raynaud’s phenomenon OT - *systemic sclerosis EDAT- 2018/01/08 06:00 MHDA- 2018/12/26 06:00 CRDT- 2018/01/08 06:00 PHST- 2018/01/08 06:00 [entrez] PHST- 2018/01/08 06:00 [pubmed] PHST- 2018/12/26 06:00 [medline] AID - 10.3906/sag-1702-23 [doi] PST - epublish SO - Turk J Med Sci. 2017 Dec 19;47(6):1687-1692. doi: 10.3906/sag-1702-23. PMID- 22990501 OWN - NLM STAT- MEDLINE DCOM- 20130301 LR - 20181201 IS - 1827-1839 (Electronic) IS - 0392-9590 (Linking) VI - 31 IP - 5 DP - 2012 Oct TI - Association of Raynaud's syndrome with interferons. A meta-analysis. PG - 408-13 AB - AIM: Vasospastic disorders of the digital circulation such as the Raynaud's syndrome (RS) are known side-effects of treatment of interferons. The prevalence of RS in patients during treatment with interferons agents is not well-defined. The objective of this paper was to assess the prevalence of RS in patients receiving interferons - a meta-analysis of published data was performed. METHODS: The PubMed database of the National Library of Medicine and ISI Web of Knowledge was used for studies dealing with RS and patients receiving interferons. The studies provided sufficient data to estimate the prevalence of RS in patients receiving interferons. A forest plot was determined by the revealed prevalences. Statistical analysis was based on methods for a random effects meta-analysis and a finite mixture model for proportions. Publication bias was investigated with the linear regression test (Egger's method). A meta-regression was conducted by the year of publication. RESULTS: Six eligible studies, contributing data on 183 subjects, were included in this meta-analysis. For RS in patients receiving interferons a pooled prevalence of 13.6% and 95% CI (95% CI 0.026, 0.313) was obtained. A mixture model analysis found three latent classes. Statistically, publication bias was not present (p-value 0.335). CONCLUSION: Despite some heterogeneity there is a possible indication of an association between RS and patients receiving interferons. FAU - Mohokum, M AU - Mohokum M AD - Department of Biometry and Clinical Epidemiology, Charité Universitätsmedizin Berlin, Berlin, Germany. melvin.mohokum@charite.de FAU - Hartmann, P AU - Hartmann P FAU - Schlattmann, P AU - Schlattmann P LA - eng PT - Journal Article PT - Meta-Analysis PT - Review PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 RN - 0 (Cyclopropanes) RN - 9008-11-1 (Interferons) RN - G56VK1HF36 (Milnacipran) SB - IM MH - Blood Vessels/*drug effects/physiopathology MH - Chi-Square Distribution MH - Cyclopropanes MH - Fingers/*blood supply MH - Humans MH - Interferons/*adverse effects MH - Linear Models MH - Milnacipran MH - Odds Ratio MH - Prevalence MH - Raynaud Disease/*chemically induced/epidemiology/physiopathology MH - Risk Assessment MH - Risk Factors EDAT- 2012/09/20 06:00 MHDA- 2013/03/02 06:00 CRDT- 2012/09/20 06:00 PHST- 2012/09/20 06:00 [entrez] PHST- 2012/09/20 06:00 [pubmed] PHST- 2013/03/02 06:00 [medline] AID - R34122884 [pii] PST - ppublish SO - Int Angiol. 2012 Oct;31(5):408-13. PMID- 37068571 OWN - NLM STAT- MEDLINE DCOM- 20230825 LR - 20230828 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 136 IP - 9 DP - 2023 Sep TI - Achenbach Syndrome in a Patient with Raynaud's Phenomenon. PG - e173-e174 LID - S0002-9343(23)00257-7 [pii] LID - 10.1016/j.amjmed.2023.04.006 [doi] FAU - Jimenez, Massiel E AU - Jimenez ME AD - Internal Medicine Residency, Department of Internal Medicine, Unity Hospital, Rochester Regional Health, NY. Electronic address: Mjimenezartiles@gmail.com. FAU - Ocon, Anthony AU - Ocon A AD - Division of Allergy, Immunology & Rheumatology, Rochester Regional Health, NY; Department of Medicine, University of Rochester Medical Center, NY; Clinical Faculty, Rochester Institute of Technology, NY. LA - eng PT - Case Reports PT - Journal Article DEP - 20230415 PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 SB - IM MH - Humans MH - *Raynaud Disease/complications/diagnosis MH - Syndrome MH - Hematoma MH - Patients EDAT- 2023/04/18 06:00 MHDA- 2023/08/25 06:42 CRDT- 2023/04/17 19:22 PHST- 2023/03/21 00:00 [received] PHST- 2023/04/01 00:00 [revised] PHST- 2023/04/02 00:00 [accepted] PHST- 2023/08/25 06:42 [medline] PHST- 2023/04/18 06:00 [pubmed] PHST- 2023/04/17 19:22 [entrez] AID - S0002-9343(23)00257-7 [pii] AID - 10.1016/j.amjmed.2023.04.006 [doi] PST - ppublish SO - Am J Med. 2023 Sep;136(9):e173-e174. doi: 10.1016/j.amjmed.2023.04.006. Epub 2023 Apr 15. PMID- 31651110 OWN - NLM STAT- MEDLINE DCOM- 20200402 LR - 20240328 IS - 1303-6165 (Electronic) IS - 1300-0144 (Print) IS - 1300-0144 (Linking) VI - 49 IP - 5 DP - 2019 Oct 24 TI - The frequency of Raynaud’s phenomenon in patients with methylenetetrahydrofolate reductase gene mutation and hyperhomocysteinemia. PG - 1444-1449 LID - 10.3906/sag-1903-206 [doi] AB - BACKGROUND/AIM: Raynaud’s phenomenon (RP) is not a rare health problem; global prevalence is about 3%–20%. Etiology and pathophysiology of this pathology has not been clarified. There are many precipitating factors resulting in RP. Hyperhomocysteinemia resulting from methylenetetrahydrofolate reductase (MTHFR) gene mutationmay have a role in its etiology. The aim of this study was to observe the frequency of RP in patients with MTFHR gene mutation and hyperhomocysteinemia. Possible relationships among vitamin B12, folic acid, complete blood count (leukocytes and platelets), and c-reactive protein levels and RP were also analyzed. MATERIALS AND METHODS: A total of 388 patients admitted to the internal medicine, hematology, and obstetric clinics of a university hospital between January 2012 and April 2013 ranging in age from 21 to 83 (mean age 38.16 ± 13.1) were enrolled in the study. Eighty-five (21.9%) of the patients were male and 303 (78.1%) were female. MTHFR gene mutation was analyzed in 388 patients; 52 (13.4%) were homozygous, 275 (70.9%) were heterozygous, and 61 (15.7%) were found to be negative for the MTHFR gene mutation and accepted as a control group. Vitamin B12, folic acid, complete blood count (leukocytes and platelets), and c-reactive protein levels were also analyzed. RESULTS: Homocysteine levels were higher in both heterozygous and homozygous groups (P < 0.05). RP was more frequently observed in patients with elevated homocysteine levels (P < 0.05; X2 = 14.51). There was no significant relationship in other parameters studied. CONCLUSION: RP was more frequently observed in the groups with the MTHFR mutation and hyperhomocysteinemia. Serum homocysteine levels in patients with RP may be helpful for diagnosis. CI - This work is licensed under a Creative Commons Attribution 4.0 International License. FAU - Yalçın, Kadir Serkan AU - Yalçın KS AUID- ORCID: 0000-0002-8028-1070 AD - Department of Internal Diseases, Lokman Hekim Ankara Hospital, Ankara, Turkey FAU - Koşar, Ali AU - Koşar A AUID- ORCID: 0000-0002-0720-1791 AD - Department of Hematology, Lokman Hekim Ankara Hospital, Ankara, Turkey LA - eng PT - Journal Article PT - Observational Study DEP - 20191024 PL - Turkey TA - Turk J Med Sci JT - Turkish journal of medical sciences JID - 9441758 RN - EC 1.5.1.20 (Methylenetetrahydrofolate Reductase (NADPH2)) MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Hyperhomocysteinemia/*complications/*genetics MH - Male MH - Methylenetetrahydrofolate Reductase (NADPH2)/*genetics MH - Middle Aged MH - *Mutation MH - Prospective Studies MH - Raynaud Disease/epidemiology/*etiology MH - Young Adult PMC - PMC7018243 OTO - NOTNLM OT - *Raynaud’s phenomenon OT - *hyperhomocysteinemia OT - *methylenetetrahydrofolate reductase OT - *mutation COIS- none declared EDAT- 2019/10/28 06:00 MHDA- 2020/04/03 06:00 PMCR- 2019/10/24 CRDT- 2019/10/26 06:00 PHST- 2019/03/26 00:00 [received] PHST- 2019/07/22 00:00 [accepted] PHST- 2019/10/26 06:00 [entrez] PHST- 2019/10/28 06:00 [pubmed] PHST- 2020/04/03 06:00 [medline] PHST- 2019/10/24 00:00 [pmc-release] AID - 10.3906/sag-1903-206 [doi] PST - epublish SO - Turk J Med Sci. 2019 Oct 24;49(5):1444-1449. doi: 10.3906/sag-1903-206. PMID- 32578512 OWN - NLM STAT- MEDLINE DCOM- 20220125 LR - 20220430 IS - 2295-3337 (Electronic) IS - 1784-3286 (Linking) VI - 77 IP - 1 DP - 2022 Feb TI - Paraneoplastic Raynaud's phenomenon as sign of progression: a case report on a patient with breast cancer. PG - 122-125 LID - 10.1080/17843286.2020.1782055 [doi] AB - BACKGROUND: Paraneoplastic Raynaud's phenomenon is a rare complication occurring in patients with advanced cancer. METHODS: We present a case of a patient with breast cancer and presenting with acrocyanosis evolving into necrosis as a paraneoplastic phenomenon in progressive breast cancer. RESULTS: Unless a multidisciplinary approach was taken, improvement of the symptoms was only seen with prostacyclin analogs, and cancer treatment needed to be changed because of rapidly progressing disease. CONCLUSION: Paraneoplastic Raynaud's phenomenon is a rare but severe presentation of advanced and progressive cancer. Treatment with intravenous prostacyclin analogs should be considered and a multidisciplinary approach is necessary. FAU - Vanlancker, Tine AU - Vanlancker T AUID- ORCID: 0000-0002-0253-6488 AD - Department of Medical Oncology, Ghent University Hospital, Ghent, Belgium. FAU - Stragier, Barbara AU - Stragier B AD - Department of Medical Oncology, AZ Delta Roeselare, Roeselare, Belgium. FAU - De Bock, Marlies AU - De Bock M AUID- ORCID: 0000-0001-9439-9881 AD - Department of Medical Oncology, AZ Delta Roeselare, Roeselare, Belgium. LA - eng PT - Case Reports PT - Journal Article DEP - 20200624 PL - England TA - Acta Clin Belg JT - Acta clinica Belgica JID - 0370306 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - *Breast Neoplasms/complications MH - Female MH - Humans MH - Necrosis MH - *Raynaud Disease/diagnosis/etiology OTO - NOTNLM OT - Raynaud’s phenomenon OT - acrocyanosis OT - advanced cancer OT - breast cancer OT - paraneoplastic EDAT- 2020/06/25 06:00 MHDA- 2022/01/27 06:00 CRDT- 2020/06/25 06:00 PHST- 2020/06/25 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] PHST- 2020/06/25 06:00 [entrez] AID - 10.1080/17843286.2020.1782055 [doi] PST - ppublish SO - Acta Clin Belg. 2022 Feb;77(1):122-125. doi: 10.1080/17843286.2020.1782055. Epub 2020 Jun 24. PMID- 16987835 OWN - NLM STAT- MEDLINE DCOM- 20070607 LR - 20220410 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 Suppl 3 DP - 2006 Oct TI - Successful treatment of patients with severe secondary Raynaud's phenomenon with the endothelin receptor antagonist bosentan. PG - iii45-8 AB - Secondary Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc) and other collagen vascular diseases is a serious manifestation of microvascular damage that may precede the onset of visceral and/or cutaneous sclerosis for years. Recent studies have demonstrated that the endothelin receptor antagonist bosentan prevents the development of new digital ulcers in SSc. We investigated the potential benefits of bosentan in patients with secondary RP associated with pre-scleroderma and with SSc, independent of digital ulcers. Three patients with secondary RP received bosentan 62.5 mg twice daily for 4 weeks followed by 125 mg twice daily for 12 weeks during the winter season. Pain (visual analogue scale), Raynaud's disease activity (Scleroderma Health Assessment Questionnaire), number and severity of daily Raynaud's attacks (diary) and peripheral thermoregulation (thermography) were assessed during treatment periods. Pain, Raynaud's disease activity, number and severity of Raynaud's attacks significantly decreased during treatment periods. Thermography after 16-week treatment demonstrated improved peripheral thermoregulation. Although this is a small observational study, treatment with bosentan appears to measurably reduce the daily impact of Raynaud's disease and improve peripheral thermoregulation in patients with secondary RP, independent of digital ulcers. FAU - Selenko-Gebauer, N AU - Selenko-Gebauer N AD - Division of Immunology, Allergy and Infectious Diseases (DIAID), Department of Dermatology, Medical University of Vienna, Vienna General Hospital, Austria. FAU - Duschek, N AU - Duschek N FAU - Minimair, G AU - Minimair G FAU - Stingl, G AU - Stingl G FAU - Karlhofer, F AU - Karlhofer F LA - eng PT - Clinical Trial PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM EIN - Rheumatology (Oxford). 2008 Feb;47(2):234-5 MH - Adult MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology/pathology MH - Scleroderma, Systemic/complications MH - Severity of Illness Index MH - Sulfonamides/*therapeutic use MH - Treatment Outcome EDAT- 2006/09/22 09:00 MHDA- 2008/03/26 09:00 CRDT- 2006/09/22 09:00 PHST- 2006/09/22 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2006/09/22 09:00 [entrez] AID - 45/suppl_3/iii45 [pii] AID - 10.1093/rheumatology/kel290 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii45-8. doi: 10.1093/rheumatology/kel290. PMID- 26784283 OWN - NLM STAT- MEDLINE DCOM- 20180925 LR - 20181004 IS - 1557-8992 (Electronic) IS - 1044-5463 (Linking) VI - 26 IP - 10 DP - 2016 Dec TI - Aripiprazole-Induced Raynaud's Phenomenon: An Adolescent Case. PG - 953-954 FAU - Camkurt, Mehmet Akif AU - Camkurt MA AD - 1 Department of Psychiatry, Afşın State Hospital , Kahramanmaraş, Turkey . FAU - Gunes, Serkan AU - Gunes S AD - 2 Department of Child and Adolescent Psychiatry, Mersin University School of Medicine , Mersin, Turkey . FAU - Tecimer, Ergün AU - Tecimer E AD - 3 Department of Cardiovascular Surgery, Afşın State Hospital , Kahramanmaraş, Turkey . LA - eng PT - Case Reports PT - Letter DEP - 20160119 PL - United States TA - J Child Adolesc Psychopharmacol JT - Journal of child and adolescent psychopharmacology JID - 9105358 RN - 82VFR53I78 (Aripiprazole) SB - IM MH - Adolescent MH - Aripiprazole/*adverse effects/therapeutic use MH - Humans MH - Male MH - Mood Disorders/drug therapy MH - Raynaud Disease/*chemically induced/diagnosis EDAT- 2016/01/20 06:00 MHDA- 2018/09/27 06:00 CRDT- 2016/01/20 06:00 PHST- 2016/01/20 06:00 [pubmed] PHST- 2018/09/27 06:00 [medline] PHST- 2016/01/20 06:00 [entrez] AID - 10.1089/cap.2015.0153 [doi] PST - ppublish SO - J Child Adolesc Psychopharmacol. 2016 Dec;26(10):953-954. doi: 10.1089/cap.2015.0153. Epub 2016 Jan 19. PMID- 28803485 OWN - NLM STAT- MEDLINE DCOM- 20180611 LR - 20180611 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 47 IP - 3 DP - 2018 May TI - Raynaud's phenomenon and its impact on activities in daily life during one year of follow-up in early systemic sclerosis. PG - 206-209 LID - 10.1080/03009742.2017.1350745 [doi] AB - OBJECTIVE: To investigate Raynaud's phenomenon (RP) and its impact on daily life activities during 1 year of follow-up in early systemic sclerosis (SSc). METHOD: Fourteen SSc patients with a median disease duration of 2 years were enrolled in the study. Every 7 weeks the patients completed a 7 day diary documenting the frequency and duration of RP attacks, the activity causing the attack, and how they handled the attack. The patients also recorded in the diary daily self-assessments of the difficulties with RP, using a 0-10 ordinal scale according to the Raynaud's Condition Score. RESULTS: Ninety-eight RP weekly diaries were analysed. The median number of RP attacks varied between six and nine per week, and the median score reflecting the difficulty associated with the attacks varied between 2.0 and 2.9. No difference was found in the number of attacks or the difficulties associated with them between winter, spring, and autumn. Fewer attacks and less difficulty were reported in August than in any of the other documented weeks (p < 0.05). In all diaries, all patients reported RP attacks associated with domestic activities. The use of heating devices varied during the follow-up. In February, all patients except one used such devices, while about half of the group used devices during the rest of the year. CONCLUSIONS: Difficulties resulting from RP are present and disabling all year round, which underscore the importance of intense vasoactive therapy and non-pharmacological strategies throughout the year. FAU - Sandqvist, G AU - Sandqvist G AD - a Department of Clinical Sciences Lund, Section of Rheumatology , Lund University, Skåne University Hospital , Lund , Sweden. FAU - Wollmer, P AU - Wollmer P AD - b Department of Translational Medicine , Lund University , Lund , Sweden. FAU - Scheja, A AU - Scheja A AD - a Department of Clinical Sciences Lund, Section of Rheumatology , Lund University, Skåne University Hospital , Lund , Sweden. FAU - Wildt, M AU - Wildt M AD - a Department of Clinical Sciences Lund, Section of Rheumatology , Lund University, Skåne University Hospital , Lund , Sweden. FAU - Hesselstrand, R AU - Hesselstrand R AD - a Department of Clinical Sciences Lund, Section of Rheumatology , Lund University, Skåne University Hospital , Lund , Sweden. LA - eng PT - Journal Article DEP - 20170813 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Vasodilator Agents) SB - IM MH - *Activities of Daily Living MH - Adult MH - *Disability Evaluation MH - Disease Progression MH - Exercise Therapy/*methods MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/etiology/*physiopathology/rehabilitation MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/physiopathology/rehabilitation MH - Severity of Illness Index MH - Time Factors MH - Vasodilator Agents/*therapeutic use EDAT- 2017/08/15 06:00 MHDA- 2018/06/12 06:00 CRDT- 2017/08/15 06:00 PHST- 2017/08/15 06:00 [pubmed] PHST- 2018/06/12 06:00 [medline] PHST- 2017/08/15 06:00 [entrez] AID - 10.1080/03009742.2017.1350745 [doi] PST - ppublish SO - Scand J Rheumatol. 2018 May;47(3):206-209. doi: 10.1080/03009742.2017.1350745. Epub 2017 Aug 13. PMID- 18696408 OWN - NLM STAT- MEDLINE DCOM- 20080819 LR - 20080812 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 133 IP - 34-35 DP - 2008 Aug TI - [Definition and terminology of Raynaud's syndrome]. PG - 1742-4 LID - 10.1055/s-0028-1082798 [doi] AB - Physicians have for a long time used different definitions, terminology and differential diagnoses for Raynaud's syndromes, which has resulted in diagnostic, therapeutic and prognostic problems. In order for difficulties of understanding to be prevented, the terminology should be restricted to the most common terms in international use, i. e. primary, secondary and suspected secondary Raynaud's syndrome. The characteristics of those three forms of Raynaud's syndrome are outlined. FAU - Heidrich, H AU - Heidrich H LA - ger PT - English Abstract PT - Journal Article TT - Definition und Terminologie von Raynaud-Syndromen. PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Humans MH - Raynaud Disease/*classification/*diagnosis MH - *Terminology as Topic EDAT- 2008/08/13 09:00 MHDA- 2008/08/20 09:00 CRDT- 2008/08/13 09:00 PHST- 2008/08/13 09:00 [pubmed] PHST- 2008/08/20 09:00 [medline] PHST- 2008/08/13 09:00 [entrez] AID - 10.1055/s-0028-1082798 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2008 Aug;133(34-35):1742-4. doi: 10.1055/s-0028-1082798. PMID- 16604347 OWN - NLM STAT- MEDLINE DCOM- 20070409 LR - 20211020 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 26 IP - 11 DP - 2006 Sep TI - Raynaud's syndrome: comparison of late and early onset forms using hand perfusion scintigraphy. PG - 1014-8 AB - Primary Raynaud's disease is generally a disease of younger females; however, there are cases where symptoms present over the age of 40. These cases are described as late onset. In our current prospective study we compared the characteristics of early and late onset types of primary Raynaud's in 127 patients. In addition to the collection of medical records, we performed capillary-microscopy and hand perfusion scintigraphy using Tc-99 m DTPA to evaluate the microcirculation of each patient's fingers. Regarding the spectrum of the capillary-microscopic findings, we did not find any significant difference between the early and late onset forms. However, in hand perfusion examinations done using Tc-99 m DTPA, we measured a significantly lower finger/palm ratio (FPR) in the early onset group of patients. We also observed a correlation between the duration of the disease and the FPR, as well as between the age and FPR. Longer disease duration resulted in a significantly lower FPR. On the basis of our results, we believe that late onset Raynaud's should be treated as a separate entity. Due to its different characteristics found on examination and follow-up of our patients, functional hand perfusion examination should be recommended independently of the age-related characteristics of the disease. FAU - Csiki, Z AU - Csiki Z AD - Third Department of Internal Medicine, University of Debrecen Medical and Health Science Center, Nagyerdei krt. 98, 4012 Debrecen, Hungary. csikiz@gmail.com FAU - Galuska, L AU - Galuska L FAU - Garai, I AU - Garai I FAU - Szabó, N AU - Szabó N FAU - Varga, J AU - Varga J FAU - András, Cs AU - András C FAU - Zeher, M AU - Zeher M LA - eng PT - Clinical Trial PT - Comparative Study PT - Journal Article DEP - 20060408 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Diagnosis, Differential MH - Female MH - Fingers/diagnostic imaging MH - Humans MH - Male MH - Microcirculation/physiopathology MH - Middle Aged MH - Perfusion/methods MH - Radionuclide Imaging/*methods MH - Raynaud Disease/*diagnostic imaging/epidemiology EDAT- 2006/04/11 09:00 MHDA- 2007/04/10 09:00 CRDT- 2006/04/11 09:00 PHST- 2005/08/08 00:00 [received] PHST- 2006/02/20 00:00 [accepted] PHST- 2006/04/11 09:00 [pubmed] PHST- 2007/04/10 09:00 [medline] PHST- 2006/04/11 09:00 [entrez] AID - 10.1007/s00296-006-0121-8 [doi] PST - ppublish SO - Rheumatol Int. 2006 Sep;26(11):1014-8. doi: 10.1007/s00296-006-0121-8. Epub 2006 Apr 8. PMID- 38233001 OWN - NLM STAT- MEDLINE DCOM- 20240119 LR - 20241023 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 17 IP - 1 DP - 2024 Jan 16 TI - Mottled Raynaud's phenomenon and hand-arm vibration syndrome: followed up for 10 years. LID - 10.1136/bcr-2023-257314 [doi] LID - e257314 AB - Vibration white finger is a form of secondary Raynaud's phenomenon (RP) caused by the use of handheld vibrating tools. RP usually appears on the extremities of the fingers, and its borders are well recognised. No reports have been published on 'mottled' RP in continuous observation from the onset to the disappearance of RP. A man in his 60s who had been using vibrating tools such as jackhammers and tampers for 30 years presented with sensations of coldness, burning and numbness. Whole-body cold exposure was performed outdoors in winter, and RP was photographed continuously. 'Mottled' RP can be defined as triphasic colour changes: white, blue and red. The patient was taken off work, kept warm and medicated. His symptoms improved slightly after 10 years of follow-up, but the RP did not disappear. 'Mottled' RP is rare and refractory and should be recognised as a form of RP. CI - © BMJ Publishing Group Limited 2024. Re-use permitted under CC BY. Published by BMJ. FAU - Hirano, Harukazu AU - Hirano H AD - Koyo Seikyo Clinic, Fukui Health Cooperative Association, Fukui, Japan koyo@asfu.jp. LA - eng PT - Case Reports PT - Journal Article DEP - 20240116 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Male MH - Humans MH - *Hand-Arm Vibration Syndrome/complications/diagnosis MH - Vibration/adverse effects MH - *Raynaud Disease/diagnosis/etiology MH - Fingers MH - Hypesthesia MH - *Occupational Diseases/etiology/complications PMC - PMC10806868 OTO - NOTNLM OT - Dermatological OT - General practice / family medicine OT - Occupational and environmental medicine COIS- Competing interests: None declared. EDAT- 2024/01/18 00:42 MHDA- 2024/01/19 06:42 PMCR- 2024/01/17 CRDT- 2024/01/17 20:43 PHST- 2024/01/19 06:42 [medline] PHST- 2024/01/18 00:42 [pubmed] PHST- 2024/01/17 20:43 [entrez] PHST- 2024/01/17 00:00 [pmc-release] AID - 17/1/e257314 [pii] AID - bcr-2023-257314 [pii] AID - 10.1136/bcr-2023-257314 [doi] PST - epublish SO - BMJ Case Rep. 2024 Jan 16;17(1):e257314. doi: 10.1136/bcr-2023-257314. PMID- 26453392 OWN - NLM STAT- MEDLINE DCOM- 20160412 LR - 20260305 IS - 1552-5732 (Electronic) IS - 0890-3344 (Linking) VI - 31 IP - 4 DP - 2015 Nov TI - My Nipples Hurt: Could I Have Raynaud's? PG - 675-6 LID - 10.1177/0890334415597902 [doi] FAU - Jeffers, Noelene K AU - Jeffers NK LA - eng PT - Patient Education Handout PL - United States TA - J Hum Lact JT - Journal of human lactation : official journal of International Lactation Consultant Association JID - 8709498 MH - *Breast Feeding MH - Female MH - Humans MH - Mastodynia/*etiology MH - Nipples/pathology/*physiopathology MH - Raynaud Disease/*diagnosis/etiology/therapy EDAT- 2015/10/11 06:00 MHDA- 2016/04/14 06:00 CRDT- 2015/10/11 06:00 PHST- 2015/10/11 06:00 [entrez] PHST- 2015/10/11 06:00 [pubmed] PHST- 2016/04/14 06:00 [medline] AID - 31/4/675 [pii] AID - 10.1177/0890334415597902 [doi] PST - ppublish SO - J Hum Lact. 2015 Nov;31(4):675-6. doi: 10.1177/0890334415597902. PMID- 16463792 OWN - NLM STAT- MEDLINE DCOM- 20060323 LR - 20061115 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 2 IP - 48 DP - 2006 Jan 11 TI - [Understanding Raynaud's phenomenon: towards a better treatment]. PG - 93-6 AB - The Raynaud's phenomenon seems to be well known among the health care workers. It has been identified a long time ago, as Maurice Raynaud described the phenomenon which bears his name in 1862. However its true definition is often forgotten. Moreover the Raynaud's phenomenon is easily confounded with other entities like acrocyanosis... The topic of this review has firstly been chosen in order to precise the criteria of the Raynaud's phenomenon, secondly to mention the new pathogenic aspects recently described and the new therapeutic interventions developed in this field. FAU - Bart, P A AU - Bart PA AD - Service d'immunologie et d'allergie CHUV, 10 11 Lausanne. pierre-alexandre.bart@chuv.ch FAU - Waeber, B AU - Waeber B LA - fre PT - English Abstract PT - Journal Article PT - Review TT - 2. Le phénomène de Raynaud: mieux comprendre pour mieux traiter. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 SB - IM MH - Humans MH - Raynaud Disease/physiopathology/*therapy RF - 18 EDAT- 2006/02/09 09:00 MHDA- 2006/03/24 09:00 CRDT- 2006/02/09 09:00 PHST- 2006/02/09 09:00 [pubmed] PHST- 2006/03/24 09:00 [medline] PHST- 2006/02/09 09:00 [entrez] PST - ppublish SO - Rev Med Suisse. 2006 Jan 11;2(48):93-6. PMID- 17289791 OWN - NLM STAT- MEDLINE DCOM- 20070612 LR - 20151119 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 4 DP - 2007 Apr TI - A case of Raynaud's phenomenon in mixed connective tissue disease responding to rituximab therapy. PG - 718-9 FAU - Haroon, M AU - Haroon M FAU - O'Gradaigh, D AU - O'Gradaigh D FAU - Foley-Nolan, D AU - Foley-Nolan D LA - eng PT - Case Reports PT - Letter DEP - 20070208 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) SB - IM CIN - Rheumatology (Oxford). 2007 Oct;46(10):1628-9. doi: 10.1093/rheumatology/kem208. PMID: 17766999 MH - Adult MH - Antibodies, Monoclonal/*therapeutic use MH - Antibodies, Monoclonal, Murine-Derived MH - Antirheumatic Agents/*therapeutic use MH - Female MH - Humans MH - Mixed Connective Tissue Disease/*drug therapy MH - Raynaud Disease/*drug therapy MH - Rituximab EDAT- 2007/02/10 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/02/10 09:00 PHST- 2007/02/10 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/02/10 09:00 [entrez] AID - kem003 [pii] AID - 10.1093/rheumatology/kem003 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Apr;46(4):718-9. doi: 10.1093/rheumatology/kem003. Epub 2007 Feb 8. PMID- 31310337 OWN - NLM STAT- MEDLINE DCOM- 20200713 LR - 20200713 IS - 1526-4610 (Electronic) IS - 0017-8748 (Linking) VI - 59 IP - 8 DP - 2019 Sep TI - Raynaud's Phenomenon Associated With Calcitonin Gene-Related Peptide Monoclonal Antibody Antagonists. PG - 1360-1364 LID - 10.1111/head.13596 [doi] AB - Two cases are reported of migraineurs who reported Raynaud's phenomenon (RP) exacerbated while taking monoclonal antibodies to the calcitonin gene-related peptide (CGRP) ligand (fremanezumab and galcanezumab) and 1 case of new onset RP while taking the CGRP receptor antagonist (erenumab). The prevalence of primary and secondary RP, causes of secondary RP, co-morbidity with migraine, and medications which might induce or exacerbate RP are reviewed. The pathophysiology of how CGRP monoclonal antagonists might exacerbate or induce RP is discussed. The cases suggest but do not prove causation. CI - © 2019 American Headache Society. FAU - Evans, Randolph W AU - Evans RW AD - Department of Neurology, Baylor College of Medicine, Houston, TX, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20190716 PL - United States TA - Headache JT - Headache JID - 2985091R RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (fremanezumab) RN - 55KHL3P693 (galcanezumab) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Adult MH - Aged MH - Antibodies, Monoclonal/*adverse effects MH - Antibodies, Monoclonal, Humanized/*adverse effects MH - Calcitonin Gene-Related Peptide/*antagonists & inhibitors MH - Female MH - Humans MH - Male MH - Middle Aged MH - Migraine Disorders/*drug therapy MH - Raynaud Disease/*chemically induced OTO - NOTNLM OT - Raynaud's phenomenon OT - erenumab OT - fremanezumab OT - galcanezumab OT - migraine EDAT- 2019/07/17 06:00 MHDA- 2020/07/14 06:00 CRDT- 2019/07/17 06:00 PHST- 2019/04/13 00:00 [accepted] PHST- 2019/07/17 06:00 [pubmed] PHST- 2020/07/14 06:00 [medline] PHST- 2019/07/17 06:00 [entrez] AID - 10.1111/head.13596 [doi] PST - ppublish SO - Headache. 2019 Sep;59(8):1360-1364. doi: 10.1111/head.13596. Epub 2019 Jul 16. PMID- 38536557 OWN - NLM STAT- MEDLINE DCOM- 20240701 LR - 20250104 IS - 1867-108X (Electronic) IS - 1867-1071 (Print) IS - 1867-1071 (Linking) VI - 42 IP - 7 DP - 2024 Jul TI - Non-contrast MRI of micro-vascularity of the feet and toes. PG - 785-797 LID - 10.1007/s11604-024-01553-z [doi] AB - PURPOSE: This study aimed to develop novel non-contrast MR perfusion techniques for assessing micro-vascularity of the foot in human subjects. METHODS: All experiments were performed on a clinical 3 T scanner using arterial spin labeling (ASL). Seven healthy subjects (30-72 years old, 5 males and 2 females) were enrolled and bilateral feet were imaged with tag-on and tag-off alternating inversion recovery spin labeling for determining micro-vascularity. We compared an ASL technique with 1-tag against 4-tag pulses. For perfusion, we determined signal increase ratio (SIR) at varying inversion times (TI) from 0.5 to 2 s. SIR versus TI data were fit to determine perfusion metrics of peak height (PH), time to peak (TTP), full width at half maximum (FWHM), area under the curve (AUC), and apparent blood flow (aBF) in the distal foot and individual toes. Using analysis of variance (ANOVA), effects of tag pulse and region of interest (ROI) on the mean perfusion metrics were assessed. In addition, a 4-tag pulse perfusion experiment was performed on patients with peripheral artery disease (PAD) and Raynaud's disease. RESULTS: Using our MR perfusion techniques, SIR versus TI data showed well-defined leading and trailing edges, with a peak near TI of 0.75-1.0 s and subsiding quickly to near zero by TI of 2 s, particularly when 4-tag pulses were used. When imaged with 4-tag pulse, we found significantly greater values in perfusion metrics, as compared to 1-tag pulse. The patients with PAD and Raynaud's disease showed a reduced or scattered perfusion curves compared to the healthy control. CONCLUSION: MR perfusion imaging of the distal foot shows greater SIR and perfusion metrics with the 4-tag pulse compared to the 1-tag pulse technique. This will likely benefit those with low perfusion due to aging, PAD, diabetic foot, and other vascular diseases. CI - © 2024. The Author(s) under exclusive licence to Japan Radiological Society. FAU - Bae, Won C AU - Bae WC AD - Department of Radiology, University of California-San Diego, La Jolla, CA, USA. AD - Department of Radiology, VA San Diego Healthcare System, San Diego, CA, USA. FAU - Malis, Vadim AU - Malis V AD - Department of Radiology, University of California-San Diego, La Jolla, CA, USA. FAU - Vucevic, Diana AU - Vucevic D AD - Department of Radiology, University of California-San Diego, La Jolla, CA, USA. FAU - Yamamoto, Asako AU - Yamamoto A AD - Department of Radiology, Teikyo University, Tokyo, Japan. FAU - Nakamura, Katsumi AU - Nakamura K AD - Kyoritsu Tobata Hospital, Kitakyusyu, Fukuoka, Japan. FAU - Lane, John AU - Lane J AD - Department of Surgery, University of California-San Diego, La Jolla, CA, USA. FAU - Miyazaki, Mitsue AU - Miyazaki M AUID- ORCID: 0000-0002-5690-694X AD - Department of Radiology, University of California-San Diego, La Jolla, CA, USA. mimiyazaki@health.ucsd.edu. LA - eng GR - P30 AR073761/AR/NIAMS NIH HHS/United States GR - R01 HL154092/HL/NHLBI NIH HHS/United States GR - 35938/Canon Medical Systems Corporation/ GR - R01 HL154092/NH/NIH HHS/United States PT - Journal Article DEP - 20240327 PL - Japan TA - Jpn J Radiol JT - Japanese journal of radiology JID - 101490689 SB - IM MH - Humans MH - Male MH - Middle Aged MH - Female MH - Adult MH - Aged MH - *Foot/blood supply/diagnostic imaging MH - *Toes/blood supply/diagnostic imaging MH - Peripheral Arterial Disease/diagnostic imaging/physiopathology MH - Magnetic Resonance Imaging/methods MH - Raynaud Disease/diagnostic imaging/physiopathology PMC - PMC11512541 MID - NIHMS2030613 OTO - NOTNLM OT - Arterial spin labeling OT - Blood flow perfusion OT - Diabetes OT - Foot and toe OT - Peripheral artery disease EDAT- 2024/03/27 18:45 MHDA- 2024/07/02 00:42 PMCR- 2025/01/01 CRDT- 2024/03/27 12:21 PHST- 2024/01/02 00:00 [received] PHST- 2024/02/28 00:00 [accepted] PHST- 2024/07/02 00:42 [medline] PHST- 2024/03/27 18:45 [pubmed] PHST- 2024/03/27 12:21 [entrez] PHST- 2025/01/01 00:00 [pmc-release] AID - 10.1007/s11604-024-01553-z [pii] AID - 10.1007/s11604-024-01553-z [doi] PST - ppublish SO - Jpn J Radiol. 2024 Jul;42(7):785-797. doi: 10.1007/s11604-024-01553-z. Epub 2024 Mar 27. PMID- 20506306 OWN - NLM STAT- MEDLINE DCOM- 20101109 LR - 20220330 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 62 IP - 9 DP - 2010 Sep TI - Clinical implications from capillaroscopic analysis in patients with Raynaud's phenomenon and systemic sclerosis. PG - 2595-604 LID - 10.1002/art.27543 [doi] FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester, Manchester Academic Health Science Centre, Salford Royal National Health Service Foundation Trust, Salford, UK. FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article PT - Review PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Capillaries/pathology MH - Disease Progression MH - Humans MH - Microcirculation MH - Nails MH - Raynaud Disease/complications/*diagnosis/physiopathology MH - Scleroderma, Systemic/complications/*diagnosis/physiopathology MH - Skin/*blood supply EDAT- 2010/05/28 06:00 MHDA- 2010/11/10 06:00 CRDT- 2010/05/28 06:00 PHST- 2010/05/28 06:00 [entrez] PHST- 2010/05/28 06:00 [pubmed] PHST- 2010/11/10 06:00 [medline] AID - 10.1002/art.27543 [doi] PST - ppublish SO - Arthritis Rheum. 2010 Sep;62(9):2595-604. doi: 10.1002/art.27543. PMID- 16981288 OWN - NLM STAT- MEDLINE DCOM- 20070220 LR - 20130520 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 33 IP - 10 DP - 2006 Oct TI - Anakinra prevents symptoms of familial cold autoinflammatory syndrome and Raynaud's disease. PG - 2085-7 AB - Familial cold autoinflammatory syndrome (FCAS) is a rare, hereditary disorder characterized by cold-induced inflammation. We describe the successful longterm treatment of a patient with FCAS with anakinra, an interleukin 1 receptor antagonist (IL-1Ra). The remarkable response of FCAS and associated Raynaud's disease in this patient suggests that IL-1 is an important mediator of these inflammatory diseases. Our report supports increasing evidence that anakinra plays an important role in the treatment of select chronic inflammatory diseases. FAU - Metyas, Samy K AU - Metyas SK AD - Division of Rheumatology, University of Southern California, Los Angeles, California 90033, USA. metyas@usc.edu FAU - Hoffman, Hal M AU - Hoffman HM LA - eng PT - Case Reports PT - Journal Article DEP - 20060915 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Interleukin 1 Receptor Antagonist Protein) RN - 0 (Interleukin-1) RN - 0 (Receptors, Interleukin-1) SB - IM MH - Cold Temperature/*adverse effects MH - Female MH - Humans MH - Inflammation/genetics/immunology/physiopathology/*prevention & control MH - Interleukin 1 Receptor Antagonist Protein/*therapeutic use MH - Interleukin-1/physiology MH - Middle Aged MH - Raynaud Disease/diagnosis/physiopathology/*prevention & control MH - Receptors, Interleukin-1/*antagonists & inhibitors MH - Syndrome MH - Urticaria/physiopathology/prevention & control MH - Vascular Diseases/genetics/immunology/physiopathology/*prevention & control EDAT- 2006/09/19 09:00 MHDA- 2007/02/21 09:00 CRDT- 2006/09/19 09:00 PHST- 2006/09/19 09:00 [pubmed] PHST- 2007/02/21 09:00 [medline] PHST- 2006/09/19 09:00 [entrez] AID - 06/13/0920 [pii] PST - ppublish SO - J Rheumatol. 2006 Oct;33(10):2085-7. Epub 2006 Sep 15. PMID- 37594342 OWN - NLM STAT- MEDLINE DCOM- 20230821 LR - 20231016 IS - 1952-4013 (Electronic) IS - 1167-1122 (Linking) VI - 33 IP - 3 DP - 2023 Jun 1 TI - A port-wine stain mimicking secondary Raynaud's phenomenon in a phototype V patient: clinical and videocapillaroscopic evaluation. PG - 302-304 LID - 10.1684/ejd.2023.4490 [doi] FAU - Gilmant, Céline AU - Gilmant C AD - Department of Dermatology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium, Department of Immunology-Allergology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium. FAU - Verheyden, Michel AU - Verheyden M AD - Department of Dermatology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium, Department of Dermatology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel (VUB), Jette, Brussels, Belgium. FAU - Badot, Valérie AU - Badot V AD - Department of Immunology-Allergology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium, Department of Rheumatology, CHU Brugmann, Université Libre de Bruxelles (ULB), Place A. Van Gehuchten 4, 1020 Brussels, Belgium. FAU - Richert, Bertrand AU - Richert B AD - Department of Dermatology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium. FAU - Mostmans, Yora AU - Mostmans Y AD - Department of Dermatology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium, Department of Immunology-Allergology, CHU Brugmann, Université Libre de Bruxelles, Place A. Van Gehuchten 4, 1020 Laken, Belgium. LA - eng PT - Journal Article PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 SB - IM MH - Humans MH - *Port-Wine Stain/diagnosis MH - *Hemangioma, Capillary MH - *Raynaud Disease/diagnosis EDAT- 2023/08/18 12:42 MHDA- 2023/08/21 06:42 CRDT- 2023/08/18 09:42 PHST- 2023/08/21 06:42 [medline] PHST- 2023/08/18 12:42 [pubmed] PHST- 2023/08/18 09:42 [entrez] AID - ejd.2023.4490 [pii] AID - 10.1684/ejd.2023.4490 [doi] PST - ppublish SO - Eur J Dermatol. 2023 Jun 1;33(3):302-304. doi: 10.1684/ejd.2023.4490. PMID- 40762127 OWN - NLM STAT- MEDLINE DCOM- 20250809 LR - 20250809 IS - 3050-6581 (Electronic) IS - 3050-6581 (Linking) VI - 50 IP - 3-4 DP - 2025 Aug TI - Sociodemographic and pharmacological characteristics of patients with primary and secondary Raynaud's phenomenon. A cross-sectional study. PG - 99-103 LID - S3050-6581(25)00203-1 [pii] LID - 10.1016/j.vasdi.2025.05.001 [doi] AB - Raynaud's phenomenon is a vasospastic condition that negatively affects the quality of life of patients. Symptoms may improve with the use of vasodilators and may worsen with the use of vasoconstrictors. The objective was to describe the sociodemographic and pharmacological characteristics of patients with a diagnosis of primary or secondary Raynaud's phenomenon in Colombia. This cross-sectional study investigated the use of drugs by patients diagnosed with Raynaud's phenomenon between June-2021, and June-2024, collected from a population database of 9.5 million Colombians affiliated with the Colombian health care system. Descriptive and bivariate analyses were performed. A total of 567 patients were identified. The average age was 51.9±17.9 years, and 82.5% were women. A total of 58.7% were diagnosed with primary Raynaud's phenomenon and 41.3% with secondary Raynaud's phenomenon. The most frequent secondary cause was systemic sclerosis (36.8%). A total of 58.9% of patients received pharmacological treatment, with a greater proportion among patients with secondary Raynaud's phenomenon than among those with primary Raynaud's phenomenon (71.8 vs. 49.8%; P<0.001). The most used drugs were calcium channel blockers (45.7%). A total of 9.2% of patients received drugs that can worsen Raynaud's phenomenon, most of which were non-selective β-blockers (6.9%). Primary Raynaud's phenomenon was more common than secondary Raynaud's phenomenon. Systemic sclerosis was the main cause of the secondary Raynaud's phenomenon. Calcium channel blockers were the most used medications for the management of Raynaud's phenomenon. Some patients were exposed to medications that can worsen Raynaud's phenomenon, but these medications may be necessary for the management of other conditions. CI - Copyright © 2025 The Author(s). Published by Elsevier Masson SAS.. All rights reserved. FAU - Valladales-Restrepo, Luis Fernando AU - Valladales-Restrepo LF AD - Grupo de Investigación en Farmacoepidemiología y Farmacovigilancia, Universidad Tecnológica de Pereira-Audifarma S.A, Calle 105 # 14-140, Pereira, Risaralda, Colombia; Grupo de Investigación Biomedicina, Facultad de Medicina, Fundación Universitaria Autónoma de las Américas, Pereira, Colombia; Semillero de Investigación en Farmacología Geriátrica, Grupo de Investigación Biomedicina, Facultad de Medicina, Fundación Universitaria Autónoma de las Américas, Pereira, Colombia. FAU - Henao-Martínez, Manuela AU - Henao-Martínez M AD - Semillero de Investigación en Farmacología Geriátrica, Grupo de Investigación Biomedicina, Facultad de Medicina, Fundación Universitaria Autónoma de las Américas, Pereira, Colombia. FAU - Velasquez-Quimara, Santiago AU - Velasquez-Quimara S AD - Semillero de Investigación en Farmacología Geriátrica, Grupo de Investigación Biomedicina, Facultad de Medicina, Fundación Universitaria Autónoma de las Américas, Pereira, Colombia. FAU - Giraldo-Galeano, Sebastián AU - Giraldo-Galeano S AD - Grupo de Investigación en Farmacoepidemiología y Farmacovigilancia, Universidad Tecnológica de Pereira-Audifarma S.A, Calle 105 # 14-140, Pereira, Risaralda, Colombia. FAU - Saldarriaga-Rivera, Lina María AU - Saldarriaga-Rivera LM AD - Grupo de Investigación en Farmacoepidemiología y Farmacovigilancia, Universidad Tecnológica de Pereira-Audifarma S.A, Calle 105 # 14-140, Pereira, Risaralda, Colombia. FAU - Machado-Alba, Jorge Enrique AU - Machado-Alba JE AD - Grupo de Investigación en Farmacoepidemiología y Farmacovigilancia, Universidad Tecnológica de Pereira-Audifarma S.A, Calle 105 # 14-140, Pereira, Risaralda, Colombia. Electronic address: machado@utp.edu.co. LA - eng PT - Journal Article DEP - 20250529 PL - France TA - Vasc Dis (Paris) JT - Vascular diseases (Paris, France) JID - 9919050936006676 RN - 0 (Vasodilator Agents) RN - 0 (Calcium Channel Blockers) SB - IM MH - Humans MH - Cross-Sectional Studies MH - *Raynaud Disease/drug therapy/epidemiology/diagnosis/physiopathology/etiology MH - Female MH - Male MH - Middle Aged MH - Adult MH - Colombia/epidemiology MH - Aged MH - *Vasodilator Agents/therapeutic use/adverse effects MH - Databases, Factual MH - Sociodemographic Factors MH - Socioeconomic Factors MH - Treatment Outcome MH - Calcium Channel Blockers/therapeutic use MH - Scleroderma, Systemic/epidemiology/complications/drug therapy/diagnosis OTO - NOTNLM OT - Calcium channel blockers OT - Colombia OT - Medications OT - Raynaud's phenomenon OT - Scleroderma COIS- Disclosure of interest The authors declare that they have no competing interest. EDAT- 2025/08/05 06:28 MHDA- 2025/08/10 06:16 CRDT- 2025/08/05 05:45 PHST- 2025/01/13 00:00 [received] PHST- 2025/04/19 00:00 [revised] PHST- 2025/05/05 00:00 [accepted] PHST- 2025/08/10 06:16 [medline] PHST- 2025/08/05 06:28 [pubmed] PHST- 2025/08/05 05:45 [entrez] AID - S3050-6581(25)00203-1 [pii] AID - 10.1016/j.vasdi.2025.05.001 [doi] PST - ppublish SO - Vasc Dis (Paris). 2025 Aug;50(3-4):99-103. doi: 10.1016/j.vasdi.2025.05.001. Epub 2025 May 29. PMID- 24568762 OWN - NLM STAT- MEDLINE DCOM- 20140429 LR - 20140226 IS - 1661-8157 (Print) IS - 1661-8157 (Linking) VI - 103 IP - 5 DP - 2014 Feb 26 TI - [Raynaud's phenomenon and other circulatory disorders of the fingers]. PG - 265-9 LID - 10.1024/1661-8157/a001571 [doi] AB - Raynaud's phenomenon (RP) is defined as attacks of blanking, subsequent cyanosis and rubeosis of fingers due to vasospasms in response to cold or emotional stimuli. Primary RP has no known underlying cause and occurs mainly in young and otherwise healthy women. Secondary RP goes along with various causes such as connective tissue diseases, toxic substances, drugs, physical trauma or organic finger artery occlusions, and occurs at any age and in both genders. Related affections are acrocyanosis and finger artery occlusions either due to arteriosclerosis or vasculitis. Also spontaneous finger hematoma may provoke an episode of RP. Therapeutically strict cold protection and avoidance of possible noxa is recommended besides the treatment of underlying diseases. No standard vasoactive drug has proven ideal for RP due to side effects. In cases with rest pain or ulcerations the same principles are applied as in ischemic diseases with no possibility for revascularization. FAU - Mahler, Felix AU - Mahler F AD - Medizinische Klinik, Spital Männedorf. LA - ger PT - English Abstract PT - Journal Article TT - Das Raynaud-Phänomen und andere Durchblutungsstörungen der Finger. PL - Switzerland TA - Praxis (Bern 1994) JT - Praxis JID - 101468093 SB - IM MH - Capillary Resistance/physiology MH - Diagnosis, Differential MH - Fingers/*blood supply MH - Humans MH - Ischemia/*diagnosis/etiology/therapy MH - Microscopy MH - Raynaud Disease/*diagnosis/etiology/therapy OTO - NOTNLM OT - Acrocyanosis OT - Akrozyanose OT - Fingerarterienverschlüsse OT - Kapillarmikroskopie OT - Phénomène de Raynaud OT - Raynaud Phänomen OT - Raynaud's phenomenon OT - acrocyanose OT - capillaroscopie périunguéale OT - finger artery occlusions OT - nailfold capillaroscopy OT - occlusions des artères digitales EDAT- 2014/02/27 06:00 MHDA- 2014/04/30 06:00 CRDT- 2014/02/27 06:00 PHST- 2014/02/27 06:00 [entrez] PHST- 2014/02/27 06:00 [pubmed] PHST- 2014/04/30 06:00 [medline] AID - H38130X117355680 [pii] AID - 10.1024/1661-8157/a001571 [doi] PST - ppublish SO - Praxis (Bern 1994). 2014 Feb 26;103(5):265-9. doi: 10.1024/1661-8157/a001571. PMID- 32337731 OWN - NLM STAT- MEDLINE DCOM- 20210728 LR - 20240330 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 86 IP - 11 DP - 2020 Nov TI - Drug repurposing in Raynaud's phenomenon through adverse event signature matching in the World Health Organization pharmacovigilance database. PG - 2217-2222 LID - 10.1111/bcp.14322 [doi] AB - AIMS: Several pharmacological treatments are recommended for Raynaud's phenomenon (RP) secondary to systemic sclerosis, but they only have modest efficacy. A way to efficiently identify new drugs is drug repurposing, which can be based on signature matching. The signature could be derived from chemical structures, pharmacological affinity or adverse event profiles. We propose to use the World Health Organization (WHO) pharmacovigilance database to generate repositioning hypotheses for treatments of RP through adverse event signature matching. METHODS: We first screened all drugs associated with at least 1 case of erythromelalgia, an adverse effect opposite to RP. In parallel, to define the adverse event signature of drugs recommended in secondary RP from the WHO pharmacovigilance database, we selected the 14 most representative adverse drug reactions (ADRs). Lastly, we performed a hierarchical cluster analysis to identify drugs with similar ADR signature to vasodilatory drugs used in RP. RESULTS: In total, 179 drugs were associated with erythromelalgia; they were related to 860 334 adverse events representative of RP drugs in the WHO pharmacovigilance database. Hierarchical cluster analysis allowed identification of 6 clusters. The most stable cluster contained 7 drugs, among which 5 are recommended in secondary RP, or pertain to the same drug class: epoprostenol, nifedipine, nicardipine, lacidipine and israpidine. The 2 remaining drugs were alemtuzumab and fumaric acid. CONCLUSION: Our ADR signature matching approach suggests that alemtuzumab and fumaric acid could be effective treatments of secondary RP. The latter is currently being investigated as a treatment of pulmonary hypertension in systemic sclerosis. CI - © 2020 The British Pharmacological Society. FAU - Zaza, Putkaradze AU - Zaza P AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, Grenoble, France. FAU - Matthieu, Roustit AU - Matthieu R AUID- ORCID: 0000-0003-4475-1626 AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, Grenoble, France. AD - Inserm, UMR 1042-HP2, university Grenoble Alpes, Grenoble, France. FAU - Jean-Luc, Cracowski AU - Jean-Luc C AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, Grenoble, France. AD - Inserm, UMR 1042-HP2, university Grenoble Alpes, Grenoble, France. FAU - Charles, Khouri AU - Charles K AUID- ORCID: 0000-0002-8427-8573 AD - Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, Grenoble, France. AD - Inserm, UMR 1042-HP2, university Grenoble Alpes, Grenoble, France. AD - Pharmacovigilance unit, Grenoble Alpes university hospital, Grenoble, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200514 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Drug Repositioning MH - Humans MH - Nifedipine MH - *Pharmacovigilance MH - *Raynaud Disease/drug therapy/epidemiology MH - World Health Organization PMC - PMC7576623 OTO - NOTNLM OT - Raynaud's phenomenon OT - drug repurposing OT - systemic sclerosis COIS- M.R. reports grants from Bioprojet, grants from Pfizer France, grants from GIRCI Rhône‐Alpes‐Auvergne, grants from Association des Sclérodermiques de France, outside the submitted work. J.L.C. reports grants from BIOPROJET, grants from Pfizer France, grants from GIRCI Rhône‐Alpes‐Auvergne, grants from Association des Sclérodermiques de France, outside the submitted work. Z.P. and C.K. have nothing to disclose. EDAT- 2020/04/28 06:00 MHDA- 2021/07/29 06:00 PMCR- 2021/11/01 CRDT- 2020/04/28 06:00 PHST- 2020/01/15 00:00 [received] PHST- 2020/04/02 00:00 [revised] PHST- 2020/04/15 00:00 [accepted] PHST- 2020/04/28 06:00 [pubmed] PHST- 2021/07/29 06:00 [medline] PHST- 2020/04/28 06:00 [entrez] PHST- 2021/11/01 00:00 [pmc-release] AID - BCP14322 [pii] AID - 10.1111/bcp.14322 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2020 Nov;86(11):2217-2222. doi: 10.1111/bcp.14322. Epub 2020 May 14. PMID- 20693202 OWN - NLM STAT- MEDLINE DCOM- 20101124 LR - 20100809 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 19 IP - 9 DP - 2010 Aug TI - Diagnostic algorithm for Raynaud's phenomenon and vascular skin lesions in systemic lupus erythematosus. PG - 1087-95 LID - 10.1177/0961203310374304 [doi] AB - Skin discolorations and skin lesions due to vascular pathologies are common clinical features in systemic lupus erythematosus. A variety of clinical manifestations such as Raynaud's phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis or thromboembolism. A standardized macro- and microvascular assessment is necessary to establish the correct diagnosis. We describe and illustrate common clinical features of vascular skin manifestations in systemic lupus erythematosus and present a diagnostic algorithm. FAU - Richter, J G AU - Richter JG AD - Department of Endocrinology, Diabetes and Rheumatology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany. FAU - Sander, O AU - Sander O FAU - Schneider, M AU - Schneider M FAU - Klein-Weigel, P AU - Klein-Weigel P LA - eng PT - Journal Article PT - Review PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Algorithms MH - Humans MH - Lupus Erythematosus, Systemic/complications/*diagnosis/physiopathology MH - Raynaud Disease/*diagnosis/etiology/pathology MH - Skin/pathology MH - Skin Diseases, Vascular/*diagnosis/etiology/pathology MH - Thromboembolism/diagnosis/etiology MH - Vasculitis/diagnosis/etiology EDAT- 2010/08/10 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/08/10 06:00 PHST- 2010/08/10 06:00 [entrez] PHST- 2010/08/10 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 19/9/1087 [pii] AID - 10.1177/0961203310374304 [doi] PST - ppublish SO - Lupus. 2010 Aug;19(9):1087-95. doi: 10.1177/0961203310374304. PMID- 29525756 OWN - NLM STAT- MEDLINE DCOM- 20180912 LR - 20240714 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2018 DP - 2018 Mar 9 TI - The novel use of botulinum toxin A for the treatment of Raynaud's phenomenon in the toes. LID - bcr-2017-219348 [pii] LID - 10.1136/bcr-2017-219348 [doi] LID - bcr2017219348 AB - Raynaud's phenomenon is a vasospastic disorder of the digital vessels triggered by exposure to cold or stress. It is most commonly observed in the hands, but also frequently affects the toes. We present three cases of patients with severe Raynaud's phenomenon in the toes, secondary to scleroderma. The diagnosis of Raynaud's syndrome and scleroderma was established according to the 2010 American College of Rheumatology and European League Against Rheumatism criteria. Patients were treated with 10 units of botulinum toxin injected into each foot. Two millilitres was injected into the base of each toe in both the left and right feet. Six weeks postinjection into the toes, patients reported an improvement of cold intolerance, colour change and frequency and severity of Raynaud's attacks. The effects were reported to last up to 5 months. To our knowledge, these are the first reported cases of the treatment of Raynaud's phenomenon in the toes with botulinum toxin A. CI - © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. FAU - Dhaliwal, Kiran AU - Dhaliwal K AD - UCL Centre for Nanotechnology and Regenerative Medicine, Royal Free Hospital, London, UK. AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. FAU - Griffin, Michelle AU - Griffin M AD - UCL Centre for Nanotechnology and Regenerative Medicine, Royal Free Hospital, London, UK. AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. FAU - Denton, Christopher P AU - Denton CP AD - Experimental Rheumatology Department, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK. AD - Division of Medicine, University College London, London, UK. FAU - Butler, Peter E M AU - Butler PEM AD - UCL Centre for Nanotechnology and Regenerative Medicine, Royal Free Hospital, London, UK. AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20180309 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Acetylcholine Release Inhibitors) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Acetylcholine Release Inhibitors/administration & dosage/*therapeutic use MH - Adult MH - Botulinum Toxins, Type A/administration & dosage/*therapeutic use MH - Female MH - Humans MH - Injections/methods MH - Middle Aged MH - Raynaud Disease/diagnosis/*drug therapy/etiology MH - Scleroderma, Systemic/complications/*diagnosis MH - Severity of Illness Index MH - Toes/*blood supply/pathology MH - Treatment Outcome PMC - PMC5847911 OTO - NOTNLM OT - plastic and reconstructive surgery OT - rheumatology COIS- Competing interests: None declared. EDAT- 2018/03/12 06:00 MHDA- 2018/09/13 06:00 PMCR- 2020/03/09 CRDT- 2018/03/12 06:00 PHST- 2018/03/12 06:00 [entrez] PHST- 2018/03/12 06:00 [pubmed] PHST- 2018/09/13 06:00 [medline] PHST- 2020/03/09 00:00 [pmc-release] AID - bcr-2017-219348 [pii] AID - 10.1136/bcr-2017-219348 [doi] PST - epublish SO - BMJ Case Rep. 2018 Mar 9;2018:bcr2017219348. doi: 10.1136/bcr-2017-219348. PMID- 37184860 OWN - NLM STAT- MEDLINE DCOM- 20231109 LR - 20240207 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 62 IP - 11 DP - 2023 Nov 2 TI - Mobile phone thermal imaging as an ambulatory assessment tool in Raynaud's phenomenon. PG - e311-e312 LID - 10.1093/rheumatology/kead210 [doi] FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Nazeer, Shanees AU - Nazeer S AD - Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Manning, Joanne AU - Manning J AD - Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Herrick, Ariane Loraine AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Letter PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Raynaud Disease/diagnostic imaging MH - Diagnostic Imaging MH - *Cell Phone PMC - PMC10838746 EDAT- 2023/05/15 13:06 MHDA- 2023/11/09 06:41 PMCR- 2023/05/15 CRDT- 2023/05/15 11:33 PHST- 2023/04/23 00:00 [accepted] PHST- 2023/11/09 06:41 [medline] PHST- 2023/05/15 13:06 [pubmed] PHST- 2023/05/15 11:33 [entrez] PHST- 2023/05/15 00:00 [pmc-release] AID - 7162675 [pii] AID - kead210 [pii] AID - 10.1093/rheumatology/kead210 [doi] PST - ppublish SO - Rheumatology (Oxford). 2023 Nov 2;62(11):e311-e312. doi: 10.1093/rheumatology/kead210. PMID- 34784129 OWN - NLM STAT- MEDLINE DCOM- 20211125 LR - 20211125 IS - 1545-9616 (Print) IS - 1545-9616 (Linking) VI - 20 IP - 11 DP - 2021 Nov 1 TI - OnabotulinumtoxinA for Systemic Sclerosis-associated Raynaud's Phenomenon: A Multi-Institutional Study on Accessibility and Effectiveness. PG - 1257-1259 LID - 10.36849/jdd.6135 [doi] AB - Raynaud’s phenomenon (RP), a common presenting symptom of systemic sclerosis (SSc), is a painful and debilitating condition of the digits caused by increased vascular reactivity.1,2. FAU - Kassamali, Bina AU - Kassamali B FAU - Desai, Sheena AU - Desai S FAU - Min, Michelle AU - Min M FAU - Mazori, Daniel AU - Mazori D FAU - Villa-Ruiz, Camila AU - Villa-Ruiz C FAU - Kus, Kylee AU - Kus K FAU - Cobos, Gabriela AU - Cobos G FAU - Merola, Joseph AU - Merola J FAU - LaChance, Avery AU - LaChance A FAU - Vleugels, Ruth AU - Vleugels R LA - eng PT - Journal Article PT - Multicenter Study PL - United States TA - J Drugs Dermatol JT - Journal of drugs in dermatology : JDD JID - 101160020 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A MH - Humans MH - Pain MH - *Raynaud Disease/diagnosis/drug therapy/etiology MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy EDAT- 2021/11/17 06:00 MHDA- 2021/11/26 06:00 CRDT- 2021/11/16 12:40 PHST- 2021/11/16 12:40 [entrez] PHST- 2021/11/17 06:00 [pubmed] PHST- 2021/11/26 06:00 [medline] AID - S1545961621P1257X [pii] AID - 10.36849/jdd.6135 [doi] PST - ppublish SO - J Drugs Dermatol. 2021 Nov 1;20(11):1257-1259. doi: 10.36849/jdd.6135. PMID- 16987831 OWN - NLM STAT- MEDLINE DCOM- 20070607 LR - 20080325 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 Suppl 3 DP - 2006 Oct TI - Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis. PG - iii33-5 AB - According to the so-called vascular hypothesis, Raynaud's phenomenon (RP) is one initial event in the pathophysiological cascade leading to sclerosis in systemic sclerosis (SSc). It is characterized by recurrent, reversible spasms of small arterioles and digital arteries, usually triggered by cold and emotional stress. Clinical signs of RP are a sudden pallor of single digits of fingers followed by reactive hyperaemia and in severe cases also by cyanosis. Besides imbalances between vasoconstrictive and vasodilatory processes, structural alterations of the involved vessels are fundamental to secondary RP in SSc. The latter is the reason why secondary RP in SSc, in contrast to primary RP, often leads to ischaemia and re-perfusion injuries. New insights into the pathophysiology of RP feature a special role for alpha2c-adrenoreceptors, Rho-kinase signalling pathways and soluble mediators. They have resulted in promising therapeutic options, including antagonism of endothelin receptors, inhibition of phosphodiesterases or selective blockade of alpha2c-adrenoreceptors. They should also have a positive impact on the course of SSc in general. FAU - Sunderkötter, C AU - Sunderkötter C AD - Department of Dermatology and Venereology, University Hospital Münster, von-Esmarch-Str. 58, 48149 Münster, Germany. cord.sunderkoetter@ukmunster.de FAU - Riemekasten, G AU - Riemekasten G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM EIN - Rheumatology (Oxford). 2008 Feb;47(2):234-5 MH - Fibrosis/physiopathology MH - Fingers/blood supply MH - Humans MH - Ischemia MH - Raynaud Disease/classification/etiology/*physiopathology MH - Scleroderma, Systemic/*physiopathology MH - Skin/blood supply MH - Vasoconstriction MH - Vasodilation RF - 15 EDAT- 2006/09/22 09:00 MHDA- 2008/03/26 09:00 CRDT- 2006/09/22 09:00 PHST- 2006/09/22 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2006/09/22 09:00 [entrez] AID - 45/suppl_3/iii33 [pii] AID - 10.1093/rheumatology/kel280 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii33-5. doi: 10.1093/rheumatology/kel280. PMID- 18413419 OWN - NLM STAT- MEDLINE DCOM- 20080919 LR - 20110311 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 17 IP - 4 DP - 2008 Apr TI - Raynaud's phenomenon and pulmonary arterial hypertension. PG - 355 LID - 10.1177/0961203307088290 [doi] FAU - Callejas-Rubio, J L AU - Callejas-Rubio JL FAU - López-Pérez, L AU - López-Pérez L FAU - Moreno-Escobar, E AU - Moreno-Escobar E FAU - Ortego-Centeno, N AU - Ortego-Centeno N LA - eng PT - Comment PT - Letter PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM CON - Lupus. 2007;16(7):505-8. doi: 10.1177/0961203307080629. PMID: 17670849 MH - Diagnosis, Differential MH - Echocardiography MH - Humans MH - Hypertension, Pulmonary/diagnosis/*etiology/physiopathology MH - Pulmonary Wedge Pressure/physiology MH - Raynaud Disease/*complications/diagnosis MH - Risk Factors MH - Severity of Illness Index EDAT- 2008/04/17 09:00 MHDA- 2008/09/20 09:00 CRDT- 2008/04/17 09:00 PHST- 2008/04/17 09:00 [pubmed] PHST- 2008/09/20 09:00 [medline] PHST- 2008/04/17 09:00 [entrez] AID - 17/4/355 [pii] AID - 10.1177/0961203307088290 [doi] PST - ppublish SO - Lupus. 2008 Apr;17(4):355. doi: 10.1177/0961203307088290. PMID- 27783160 OWN - NLM STAT- MEDLINE DCOM- 20170629 LR - 20181113 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 36 IP - 12 DP - 2016 Dec TI - Diagnosis of Raynaud's phenomenon by (99m)Tc-pertechnetate hand perfusion scintigraphy: a pilot study. PG - 1683-1688 AB - We assess the usefulness of (99m)Tc-pertechnetate hand perfusion scintigraphy in patients with Raynaud's phenomenon (RP). The study population consisted of 18 patients with primary RP, 25 patients with secondary RP within systemic sclerosis (SSc), and ten healthy individuals. Gamma camera dynamic first-pass study during the first 60 s and a static scintigraphy after 5 min were recorded following a bolus injection of (99m)Tc-pertechnetate via a cubital vein. Regions of interest were drawn on the summed images around the fingers and the palmar region. The fingers-to-palm ratios were then calculated. The mean fingers-to-palm ratio for dynamic study (blood flow) was 0.58 ± 0.19 for the healthy group, 0.45 ± 0.18 for the primary RP, and 0.43 ± 0.21 for the SSc patients. The mean fingers-to-palm ratio for static study (blood pool) was 0.44 ± 0.06 for the healthy group, 0.42 ± 0.06 for the primary RP, and 0.36 ± 0.07 for the SSc patients. Analysis of variance showed these differences to be significant (p = 0.039 from blood flow and p = 0.004 from blood pool). The receiver operating characteristic curve showed sensitivity of 80% and a specificity of 60% when using cutoff values of 0.40 for blood flow and sensitivity of 79% and a specificity of 70% when using cutoff values of 0.37 for blood pool. Our method is able to differentiate between patients with normal and those with abnormal microcirculation of the hands. Dynamic study separates the healthy subjects from patients with RP, while static study separates primary from secondary RP. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AUID- ORCID: 0000-0003-2366-9244 AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Resavska 69, 11000, Belgrade, Serbia. dolijan@eunet.rs. FAU - Petrovic, Nebojsa AU - Petrovic N AD - Center for Nuclear Medicine, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Vujasinovic Stupar, Nada AU - Vujasinovic Stupar N AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Resavska 69, 11000, Belgrade, Serbia. FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Resavska 69, 11000, Belgrade, Serbia. FAU - Radunovic, Goran AU - Radunovic G AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Resavska 69, 11000, Belgrade, Serbia. FAU - Babic, Dragan AU - Babic D AD - Institute for Medical Statistics and Informatics, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Sobic-Saranovic, Dragana AU - Sobic-Saranovic D AD - Center for Nuclear Medicine, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Artiko, Vera AU - Artiko V AD - Center for Nuclear Medicine, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. LA - eng PT - Journal Article DEP - 20161025 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - A0730CX801 (Sodium Pertechnetate Tc 99m) SB - IM MH - Adult MH - Aged MH - Female MH - Hand/*diagnostic imaging MH - Humans MH - Male MH - Middle Aged MH - Perfusion Imaging/*methods MH - Pilot Projects MH - Raynaud Disease/*diagnostic imaging/etiology MH - Scleroderma, Systemic/complications/*diagnostic imaging MH - Sensitivity and Specificity MH - *Sodium Pertechnetate Tc 99m OTO - NOTNLM OT - 99mTc-pertechnetate OT - Raynaud’s phenomenon OT - Scintigraphy EDAT- 2016/10/27 06:00 MHDA- 2017/07/01 06:00 CRDT- 2016/10/27 06:00 PHST- 2016/07/01 00:00 [received] PHST- 2016/10/19 00:00 [accepted] PHST- 2016/10/27 06:00 [pubmed] PHST- 2017/07/01 06:00 [medline] PHST- 2016/10/27 06:00 [entrez] AID - 10.1007/s00296-016-3584-2 [pii] AID - 10.1007/s00296-016-3584-2 [doi] PST - ppublish SO - Rheumatol Int. 2016 Dec;36(12):1683-1688. doi: 10.1007/s00296-016-3584-2. Epub 2016 Oct 25. PMID- 23212937 OWN - NLM STAT- MEDLINE DCOM- 20131204 LR - 20130101 IS - 1552-5732 (Electronic) IS - 0890-3344 (Linking) VI - 29 IP - 1 DP - 2013 Feb TI - Raynaud's phenomenon of the nipple associated with labetalol use. PG - 17-9 LID - 10.1177/0890334412467509 [doi] AB - Raynaud's phenomenon of the nipple is an unusual cause of severe nipple pain. Precipitants for Raynaud's phenomenon of the nipple are known to be cold temperatures, caffeine, and emotional stress. Nipple pain is quoted as the most common cause of cessation of breastfeeding. It is important that lactation consultants and other health care professionals are aware of Raynaud's phenomenon of the nipple and the treatment options available. This is a case of a 37-year-old woman, Gravida 2, Para 1, who was first seen in the antenatal clinic at 34 weeks gestation. A diagnosis of Raynaud's phenomenon of the nipple was made after she began taking labetalol for pregnancy-induced hypertension. The phenomenon had occurred in both of her previous pregnancies on commencing labetalol and resolved postnatally on cessation of the drug. We aim to raise awareness of both the condition itself and the potential role of labetalol in the development of Raynaud's phenomenon of the nipple. FAU - McGuinness, Naomi AU - McGuinness N AD - Department of Obstetrics and Gynaecology, St Helens and Knowsley Teaching Hospital, Prescot, UK. naomi.mcg@doctors.org.uk FAU - Cording, Vicky AU - Cording V LA - eng PT - Case Reports PT - Journal Article DEP - 20121204 PL - United States TA - J Hum Lact JT - Journal of human lactation : official journal of International Lactation Consultant Association JID - 8709498 RN - 0 (Antihypertensive Agents) RN - R5H8897N95 (Labetalol) MH - Adult MH - Antihypertensive Agents/*adverse effects/therapeutic use MH - Female MH - Humans MH - Hypertension/drug therapy MH - Labetalol/*adverse effects/therapeutic use MH - Nipples/*blood supply MH - Pregnancy MH - Raynaud Disease/*chemically induced/diagnosis EDAT- 2012/12/06 06:00 MHDA- 2013/12/16 06:00 CRDT- 2012/12/06 06:00 PHST- 2012/12/06 06:00 [entrez] PHST- 2012/12/06 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - 0890334412467509 [pii] AID - 10.1177/0890334412467509 [doi] PST - ppublish SO - J Hum Lact. 2013 Feb;29(1):17-9. doi: 10.1177/0890334412467509. Epub 2012 Dec 4. PMID- 19687085 OWN - NLM STAT- MEDLINE DCOM- 20100406 LR - 20151119 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 34 IP - 5 DP - 2009 Oct TI - Analysis of rewarming curves in Raynaud's phenomenon of various aetiologies. PG - 621-6 LID - 10.1177/1753193409102373 [doi] AB - This study investigated whether a modified Cold Provocation Test could distinguish between 86 normal subjects and 31 patients with Raynaud's phenomenon or 59 with hand arm vibration syndrome (HAVS). Of the HAVS subjects, 56 were seen for medical reports as they were involved in litigation. Their assessments were done in a different location but the same protocol was used. A standardised cold stress was used to reduce the finger temperature to 15 degrees C or less without inducing reflex hyperaemia. This test had acceptable repeatability for subjects without HAVS with an intra-class correlation of 0.7. Baseline temperature, temperature rise in the first 30 seconds and the time taken to rewarm by 5 degrees C were measured. Patients with Raynaud's phenomenon and HAVS had cooler hands than controls. HAVS patients rewarmed most in the first 30 seconds. Patients with Raynaud's phenomenon take longer to rewarm by 5 degrees C than controls or those with HAVS (P<0.001). A baseline difference of >7.5 degrees C between the temperature of the digit and that of the room is unlikely to occur in patients with Raynaud's phenomenon or HAVS. A temperature gain of > or =2.2 degrees C in the first 30 seconds on rewarming combined with a low baseline temperature strongly suggests HAVS. This modified cold provocation test may differentiate between patients with Raynaud's phenomenon, HAVS and controls but this observation requires independent verification in subjects not involved in litigation and tested in the same facility. FAU - Salem, K M AU - Salem KM AD - School of Graduate Entry Medicine & Health, Division of Vascular Medicine The University of Nottingham, Derby, UK. FAU - Baker, M AU - Baker M FAU - Hilliam, R M AU - Hilliam RM FAU - Davies, S AU - Davies S FAU - Deighton, C AU - Deighton C FAU - Bainbridge, L C AU - Bainbridge LC FAU - Manning, G AU - Manning G LA - eng PT - Controlled Clinical Trial PT - Journal Article DEP - 20090817 PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 SB - IM CIN - J Hand Surg Eur Vol. 2010 Jun;35(5):431-2; author reply 432-3. doi: 10.1177/1753193410365795. PMID: 20515996 MH - Adult MH - Age Factors MH - Aged MH - *Cold Temperature MH - Female MH - Hand MH - Humans MH - Male MH - Middle Aged MH - Physical Stimulation MH - Raynaud Disease/*diagnosis/etiology/*physiopathology MH - Recovery of Function MH - Reproducibility of Results MH - *Rewarming MH - Sex Factors MH - Smoking MH - Time Factors EDAT- 2009/08/19 09:00 MHDA- 2010/04/07 06:00 CRDT- 2009/08/19 09:00 PHST- 2009/08/19 09:00 [entrez] PHST- 2009/08/19 09:00 [pubmed] PHST- 2010/04/07 06:00 [medline] AID - 1753193409102373 [pii] AID - 10.1177/1753193409102373 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2009 Oct;34(5):621-6. doi: 10.1177/1753193409102373. Epub 2009 Aug 17. PMID- 17607716 OWN - NLM STAT- MEDLINE DCOM- 20080303 LR - 20131121 IS - 0276-3478 (Print) IS - 0276-3478 (Linking) VI - 40 IP - 8 DP - 2007 Dec TI - Anorexia nervosa and Raynaud's phenomenon: a case report. PG - 762-5 AB - OBJECTIVE: To describe and discuss potential relationships between anorexia nervosa (AN) and Raynaud's phenomenon, the course and concurrent treatment of these two disorders as they appeared simultaneously, and a potential treatment modification entailed in such concurrent therapies. BACKGROUND: Although Raynaud's phenomenon has been described during the course of AN, the associations and interactions between these two conditions are not clear. METHOD: We report the medical workup, treatment, and outcomes in a 19-year old female patient who developed Raynaud's phenomenon following the onset of AN. RESULTS: After treatment with nutritional rehabilitation, counseling, and individual and group therapy, the patient's weight, eating disorder-related behaviors, and attitudes improved significantly. Raynaud's related symptoms improved, following treatment with a calcium channel blocker and antiaggregant therapy. In conjunction with nutritional efforts to treat the patient's long-standing amenorrhea and osteopenia, the treatment team elected to also administer estrogen hormone in addition to oral calcium and vitamin D supplementation. Since oral contraceptives are to be avoided in patients with Raynaud's phenomenon who show clinical findings suggesting connective tissue disorder, the treatment team elected to treat this patient with transdermal hormone replacement therapy. CONCLUSION: The co-occurrence of AN and Raynaud's phenomenon merits close and persistent follow-up by a multidisciplinary team and may lead to alterations of usual therapeutic approaches. CI - (c) 2007 by Wiley Periodicals, Inc. FAU - Yucel, Basak AU - Yucel B AD - Department of Psychiatry, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. byucel@superonline.com FAU - Uzum, Ayse Kubat AU - Uzum AK FAU - Ozbey, Nese AU - Ozbey N FAU - Kamali, Sevil AU - Kamali S FAU - Yager, Joel AU - Yager J LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Int J Eat Disord JT - The International journal of eating disorders JID - 8111226 RN - 0 (Calcium Channel Blockers) RN - 0 (Platelet Aggregation Inhibitors) RN - I9ZF7L6G2L (Nifedipine) RN - R16CO5Y76E (Aspirin) SB - IM MH - Administration, Cutaneous MH - Adult MH - Amenorrhea/etiology/therapy MH - Anorexia Nervosa/*complications/diagnosis/therapy MH - Aspirin/therapeutic use MH - Bone Density/drug effects MH - Bone Diseases, Metabolic/etiology/therapy MH - Calcium Channel Blockers/therapeutic use MH - Combined Modality Therapy MH - Counseling MH - Estrogen Replacement Therapy MH - Family Therapy MH - Female MH - Follow-Up Studies MH - Humans MH - Nifedipine/therapeutic use MH - Nutrition Therapy MH - Platelet Aggregation Inhibitors/therapeutic use MH - Psychotherapy MH - Psychotherapy, Group MH - Raynaud Disease/diagnosis/*etiology/therapy EDAT- 2007/07/04 09:00 MHDA- 2008/03/04 09:00 CRDT- 2007/07/04 09:00 PHST- 2007/07/04 09:00 [pubmed] PHST- 2008/03/04 09:00 [medline] PHST- 2007/07/04 09:00 [entrez] AID - 10.1002/eat.20418 [doi] PST - ppublish SO - Int J Eat Disord. 2007 Dec;40(8):762-5. doi: 10.1002/eat.20418. PMID- 30651255 OWN - NLM STAT- MEDLINE DCOM- 20200316 LR - 20240426 IS - 1473-4893 (Electronic) IS - 1470-2118 (Print) IS - 1470-2118 (Linking) VI - 19 IP - 1 DP - 2019 Jan TI - Severe Raynaud's phenomenon and Sjögren's syndrome with ferocious gangrene change and auto-amputation. PG - 85 LID - 10.7861/clinmedicine.19-1-85 [doi] FAU - Huang, Pin-Hsiang AU - Huang PH AD - Division of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, Taiwan. FAU - Liao, Hsien-Tzung AU - Liao HT AD - Division of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, Taiwan; Division of Allergy, Immunology and Rheumatology, Taipei Medical University, Taipei, Taiwan; School of Medicine, National Yang-Ming University, Taipei, Taiwan htliao@vghtpe.gov.tw. FAU - Tsai, Chang-Youh AU - Tsai CY AD - Division of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, Taiwain; School of Medicine, National Yang-Ming University, Taipei, Taiwan. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Clin Med (Lond) JT - Clinical medicine (London, England) JID - 101092853 SB - IM MH - Female MH - Gangrene/*etiology/pathology MH - Humans MH - Middle Aged MH - Raynaud Disease/*complications/pathology MH - Sjogren's Syndrome/*complications/pathology MH - *Toes/pathology PMC - PMC6399625 EDAT- 2019/01/18 06:00 MHDA- 2020/03/17 06:00 PMCR- 2019/01/01 CRDT- 2019/01/18 06:00 PHST- 2019/01/18 06:00 [entrez] PHST- 2019/01/18 06:00 [pubmed] PHST- 2020/03/17 06:00 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - S1470-2118(24)01421-0 [pii] AID - clinmedicine [pii] AID - 10.7861/clinmedicine.19-1-85 [doi] PST - ppublish SO - Clin Med (Lond). 2019 Jan;19(1):85. doi: 10.7861/clinmedicine.19-1-85. PMID- 38727559 OWN - NLM STAT- MEDLINE DCOM- 20240708 LR - 20241022 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 8 IP - 2 DP - 2024 Jul 8 TI - Neurofascialvascular training for the treatment of Raynaud's phenomenon: A case report. PG - 302-309 LID - 10.1093/mrcr/rxae026 [doi] AB - Primary Raynaud's phenomenon (PRP) is characterised by episodic, reversible, and disabling vasospasms of the peripheral arteries. In the most severe cases, it can lead to ulceration of the fingers and toes. Neuro fascial VascularTraining (NFVT) is a novel therapeutic approach for treating PRP. NFVT aims to enhance peripheral circulation and stimulate the autonomic nervous system (ANS) by engaging multiple physiological mechanisms simultaneously. This integrated approach works to reduce vasospasms and alleviate associated symptoms through neurodynamic and myofascial interventions. A 54-year-old woman, who has experienced pain and hypoesthesia in her hands for 9 years, received a diagnosis of PRP without systemic sclerosis in 2014. The patient reported daily colour changes in her fingers, along with pain and a temporary decrease in tactile sensitivity. The patient engaged in ten 30-minute exercise sessions, and the clinical outcomes were assessed based on several parameters. These included the frequency and duration of vasospastic attacks, evaluated using the Raynaud Condition Score, as well as pain and tingling, measured through the daily Numeric Rating Scale. The Composite Autonomic Symptom Score (COMPASS 31) was utilised to assess dysautonomia, while the frequency of medication use and the Disabilities of the Arm, Shoulder, and Hand questionnaire were also considered. The results indicated a significant improvement in symptoms. NFVT improved symptoms and motor dysfunction in a patient with Raynaud's syndrome, demonstrating how NFVT can increase peripheral blood flow, stimulate the ANS, and improve symptoms in PRP. CI - © Japan College of Rheumatology 2024. Published by Oxford University Press. FAU - Bertacchini, Paolo AU - Bertacchini P AD - Master OMPT, University of Bologna, Bologna, Emilia-Romagna 40138, Italy. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 SB - IM MH - Humans MH - *Raynaud Disease/therapy/diagnosis/etiology MH - Female MH - Middle Aged MH - Treatment Outcome MH - Exercise Therapy/methods OTO - NOTNLM OT - Autonomic nervous system OT - blood flow circulation OT - case report OT - myofascial chains OT - neurodynamics EDAT- 2024/05/10 12:44 MHDA- 2024/07/08 18:42 CRDT- 2024/05/10 09:44 PHST- 2024/01/18 00:00 [received] PHST- 2024/04/03 00:00 [revised] PHST- 2024/05/09 00:00 [accepted] PHST- 2024/07/08 18:42 [medline] PHST- 2024/05/10 12:44 [pubmed] PHST- 2024/05/10 09:44 [entrez] AID - 7668179 [pii] AID - 10.1093/mrcr/rxae026 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2024 Jul 8;8(2):302-309. doi: 10.1093/mrcr/rxae026. PMID- 21677001 OWN - NLM STAT- MEDLINE DCOM- 20120622 LR - 20110902 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 38 IP - 9 DP - 2011 Sep TI - Raynaud's phenomenon in medical laboratory workers who work with solvents. PG - 1940-6 LID - 10.3899/jrheum.101129 [doi] AB - OBJECTIVE: To investigate whether there is an association between Raynaud's phenomenon (RP) and exposure to organic solvents in laboratory workers. METHODS: Technicians, scientists, and laboratory assistants working in histology, cytology, and transfusion medicine were surveyed about their use of solvents, particularly xylene and toluene, and about symptoms of RP. There were 341 responses. OR for having worked with solvents were calculated with logistic regression adjusted for age and sex. RESULTS: Laboratory workers who had worked with solvents had higher rates of severe RP, particularly those who had worked with xylene or toluene and either acetone (OR 8.8, 95% CI 1.9-41.1), or chlorinated solvents (OR 8.9, 95% CI 1.9-41.6), xylene or toluene and acetone compared to those who had worked with xylene or toluene but not acetone (OR 4.5, 95% CI 1.2-16.2), and similarly for chlorinated solvents (OR 4.5, 95% CI 1.2-16.3). RP symptoms occurring in the absence of cold exposure were more frequent for those who had worked with any solvent (OR 3.6, 95% CI 1.2-10.5) and just xylene or toluene (OR 2.8, 95% CI 1.1-7.3). Associations were also seen between increasing exposure to xylene or toluene and severe RP (OR 1.7, 95% CI 1.1-2.7, per 10 years) and with symptoms occurring in the absence of cold exposure (OR 1.7, 95% CI 1.2-2.5, per 10 years). CONCLUSION: We found that exposure to solvents may be associated with the development of RP, supporting previous work indicating that solvent exposure may be an etiological factor in systemic sclerosis. FAU - Purdie, Gordon L AU - Purdie GL AD - Department of Public Health, University of Otago Wellington, PO Box 7343, Wellington South, New Zealand. Gordon.Purdie@otago.ac.nz FAU - Purdie, Dianne J AU - Purdie DJ FAU - Harrison, Andrew A AU - Harrison AA LA - eng PT - Journal Article DEP - 20110615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Solvents) SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - *Medical Laboratory Personnel MH - Middle Aged MH - Occupational Diseases/*chemically induced/diagnosis/epidemiology MH - Occupational Exposure/*adverse effects MH - Raynaud Disease/*chemically induced/diagnosis/epidemiology MH - Scleroderma, Systemic/*chemically induced/diagnosis/epidemiology MH - Solvents/*poisoning EDAT- 2011/06/17 06:00 MHDA- 2012/06/23 06:00 CRDT- 2011/06/17 06:00 PHST- 2011/06/17 06:00 [entrez] PHST- 2011/06/17 06:00 [pubmed] PHST- 2012/06/23 06:00 [medline] AID - jrheum.101129 [pii] AID - 10.3899/jrheum.101129 [doi] PST - ppublish SO - J Rheumatol. 2011 Sep;38(9):1940-6. doi: 10.3899/jrheum.101129. Epub 2011 Jun 15. PMID- 20980938 OWN - NLM STAT- MEDLINE DCOM- 20110110 LR - 20101028 IS - 1091-0220 (Print) IS - 1091-0220 (Linking) VI - 14 IP - 12 DP - 2010 Dec TI - Mayo Clinic office visit. Raynaud's disease. An interview with Kevin Moder, M.D. PG - 6 FAU - Moder, Kevin AU - Moder K LA - eng PT - Interview PL - United States TA - Mayo Clin Womens Healthsource JT - Mayo Clinic women's healthsource JID - 9891120 MH - Health Education/*methods MH - *Health Knowledge, Attitudes, Practice MH - Humans MH - Patient Education as Topic MH - Raynaud Disease/*diagnosis/prevention & control/*therapy MH - United States EDAT- 2010/10/29 06:00 MHDA- 2011/01/11 06:00 CRDT- 2010/10/29 06:00 PHST- 2010/10/29 06:00 [entrez] PHST- 2010/10/29 06:00 [pubmed] PHST- 2011/01/11 06:00 [medline] PST - ppublish SO - Mayo Clin Womens Healthsource. 2010 Dec;14(12):6. PMID- 35059878 OWN - NLM STAT- MEDLINE DCOM- 20220317 LR - 20220406 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 4 DP - 2022 Apr TI - Can Raynaud's phenomenon be defined as a subtype of primary Sjögren's syndrome? Comments on "Clinical features and risk factors of Raynaud's phenomenon in primary Sjögren's syndrome". PG - 1265-1266 LID - 10.1007/s10067-021-06023-9 [doi] FAU - Chen, Caiqun AU - Chen C AUID- ORCID: 0000-0002-5314-8418 AD - Department of Chinese Pharmacy, Huangyan Hospital of Wenzhou Medical University, Taizhou First People's Hospital, 218 Hengjie Road, Taizhou, 318020, Zhejiang, China. FAU - Liang, Yan AU - Liang Y AUID- ORCID: 0000-0003-4029-1765 AD - Department of Laboratory Medicine, Changzheng Hospital, Naval Medical University, 415 Fengyang Road, Shanghai, 200003, China. liangyan0829@163.com. FAU - Yang, Zaixing AU - Yang Z AUID- ORCID: 0000-0002-7545-642X AD - Department of Laboratory Medicine, Huangyan Hospital of Wenzhou Medical University, Taizhou First People's Hospital, 218 Hengjie Road, Taizhou, 318020, Zhejiang, China. yangzaixingdiyi@163.com. LA - eng PT - Letter DEP - 20220121 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM CIN - Clin Rheumatol. 2022 Apr;41(4):1267-1268. doi: 10.1007/s10067-021-06043-5. PMID: 34984505 MH - Humans MH - *Raynaud Disease/complications MH - Risk Factors MH - *Sjogren's Syndrome/complications EDAT- 2022/01/22 06:00 MHDA- 2022/03/18 06:00 CRDT- 2022/01/21 06:33 PHST- 2021/12/02 00:00 [received] PHST- 2021/12/09 00:00 [accepted] PHST- 2021/12/07 00:00 [revised] PHST- 2022/01/22 06:00 [pubmed] PHST- 2022/03/18 06:00 [medline] PHST- 2022/01/21 06:33 [entrez] AID - 10.1007/s10067-021-06023-9 [pii] AID - 10.1007/s10067-021-06023-9 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Apr;41(4):1265-1266. doi: 10.1007/s10067-021-06023-9. Epub 2022 Jan 21. PMID- 25348785 OWN - NLM STAT- MEDLINE DCOM- 20160823 LR - 20150508 IS - 1708-539X (Electronic) IS - 1708-5381 (Linking) VI - 23 IP - 3 DP - 2015 Jun TI - A suspicious reason for Raynaud's phenomenon: Intrauterine device. PG - 327-8 LID - 10.1177/1708538114558330 [doi] AB - Primary Raynaud's phenomenon may be insistent in patients under medical therapy, and intrauterine devices may be an unnoticed reason in these patients. Fluctuations in female sex hormone status were reported to be associated with the emergence of primary Raynaud's phenomenon symptoms. The use of intrauterine devices was not reported to be associated with Raynaud's phenomenon previously. Intrauterine device may stimulate vascular hyperactivity regarding hormonal or unknown mechanisms that result in Raynaud's phenomenon. We present a postmenopausal patient who complained of primary Raynaud's phenomenon symptoms and had recovery after the removal of her copper intrauterine device. CI - © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Diken, Adem I AU - Diken AI AD - Department of Cardiovascular Surgery, Faculty of Medicine, Hitit University, Çorum, Turkey ademilkay@gmail.com. FAU - Yalçınkaya, Adnan AU - Yalçınkaya A AD - Department of Cardiovascular Surgery, Faculty of Medicine, Hitit University, Çorum, Turkey. FAU - Aksoy, Eray AU - Aksoy E AD - Department of Cardiovascular Surgery, Faculty of Medicine, Hitit University, Çorum, Turkey. FAU - Yılmaz, Seyhan AU - Yılmaz S AD - Department of Cardiovascular Surgery, Faculty of Medicine, Hitit University, Çorum, Turkey. FAU - Çağlı, Kerim AU - Çağlı K AD - Department of Cardiovascular Surgery, Faculty of Medicine, Hitit University, Çorum, Turkey. LA - eng PT - Case Reports PT - Journal Article DEP - 20141027 PL - England TA - Vascular JT - Vascular JID - 101196722 RN - 0 (Gonadal Steroid Hormones) SB - IM MH - Female MH - Gonadal Steroid Hormones/physiology MH - Hand/*physiopathology MH - Humans MH - Intrauterine Devices/*adverse effects MH - Middle Aged MH - Raynaud Disease/diagnosis/*etiology/*surgery MH - Recovery of Function/physiology OTO - NOTNLM OT - Raynaud’s phenomenon OT - intrauterine device EDAT- 2014/10/29 06:00 MHDA- 2016/08/24 06:00 CRDT- 2014/10/29 06:00 PHST- 2014/10/29 06:00 [entrez] PHST- 2014/10/29 06:00 [pubmed] PHST- 2016/08/24 06:00 [medline] AID - 1708538114558330 [pii] AID - 10.1177/1708538114558330 [doi] PST - ppublish SO - Vascular. 2015 Jun;23(3):327-8. doi: 10.1177/1708538114558330. Epub 2014 Oct 27. PMID- 33947582 OWN - NLM STAT- MEDLINE DCOM- 20210929 LR - 20210929 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 51 IP - 3 DP - 2021 Jun TI - Developing a core set of outcome measure domains to study Raynaud's phenomenon and digital ulcers in systemic sclerosis: Report from OMERACT 2020. PG - 640-643 LID - S0049-0172(21)00060-3 [pii] LID - 10.1016/j.semarthrit.2021.04.005 [doi] AB - Raynaud's phenomenon (RP) and digital ulcers (DUs) are important disease manifestations of systemic sclerosis (SSc) that can lead to significant pain and disability. It is essential when studying these disease features to utilize outcome measures that fully evaluate the complexities of RP and DUs . The Outcome Measures in Rheumatology (OMERACT) Vascular Disease in SSc Working Group is applying the OMERACT filter 2.1 to identify a core set of disease domains that encompass the full burden of SSc-related RP and DUs. Progress to date and future research plans were presented during a Special Interest Group held in December 2020. CI - Copyright © 2021 Elsevier Inc. All rights reserved. FAU - Maltez, Nancy AU - Maltez N AD - Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada. Electronic address: nmaltez@toh.ca. FAU - Hughes, Michael AU - Hughes M AD - Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Brown, Edith AU - Brown E AD - Manchester, United Kingdom. FAU - Hickey, Virginia AU - Hickey V AD - Adelaide, Australia. FAU - Park, Heiyoung AU - Park H AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institute of Health, USA. FAU - Shea, Beverley AU - Shea B AD - Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada. FAU - Herrick, Ariane L AU - Herrick AL AD - The University of Manchester and Salford Royal NHS Foundation Trust, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath, Bath, UK. FAU - Proudman, Susanna AU - Proudman S AD - Discipline of Medicine, University of Adelaide and Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia. FAU - Merkel, Peter A AU - Merkel PA AD - Division of Rheumatology, Department of Medicine, Division of Clinical Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210421 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - Outcome Assessment, Health Care MH - *Raynaud Disease/etiology MH - *Scleroderma, Systemic/complications MH - *Skin Ulcer/etiology MH - Ulcer OTO - NOTNLM OT - Digital ulcers OT - OMERACT OT - Raynaud's phenomenon OT - Systemic sclerosis COIS- Declaration of Competing Interest Dr Hughes reports speaking fees from Actelion Pharmaceuticals, Eli Lilly and Pfizer, outside of the submitted work. Dr. Pauling reports grants and personal fees from Janssen, outside the submitted work; Dr Pauling reports personal fees from Boehringer Ingelheim, Sojournix Pharma and Permeatus, Inc. Dr Proudman reports receiving funds for the following activities: advisory board: Boehringer-Ingelheim, Janssen, Gossamer. Research Support: Janssen Dr. Merkel reports receiving funds for the following activities: Consulting: AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, CSL Behring, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Jannsen, Kiniksa, Kyverna, Magenta, Novartis, Pfizer, Sparrow, Takeda, Talaris. Research Support: AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Sanofi. Royalties: UpToDate. A Herrick reports research funding from Gesynta Pharma, consultancies with Boehringer-Ingelheim, Camurus, CSL Behring, Gesynta Pharma and speaker's fees from Janssen. EDAT- 2021/05/06 06:00 MHDA- 2021/09/30 06:00 CRDT- 2021/05/05 05:49 PHST- 2021/03/28 00:00 [received] PHST- 2021/04/09 00:00 [accepted] PHST- 2021/05/06 06:00 [pubmed] PHST- 2021/09/30 06:00 [medline] PHST- 2021/05/05 05:49 [entrez] AID - S0049-0172(21)00060-3 [pii] AID - 10.1016/j.semarthrit.2021.04.005 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2021 Jun;51(3):640-643. doi: 10.1016/j.semarthrit.2021.04.005. Epub 2021 Apr 21. PMID- 24933844 OWN - NLM STAT- MEDLINE DCOM- 20140717 LR - 20140617 IS - 1540-9740 (Print) IS - 1540-9740 (Linking) VI - 12 IP - 2 DP - 2014 Mar-Apr TI - Xerosis cutis: a common predicament of Raynaud's disease. PG - 80-2 FAU - Chacon, Anna H AU - Chacon AH FAU - Franca, Katlein AU - Franca K FAU - Ledon, Jennifer AU - Ledon J FAU - Savas, Jessica AU - Savas J FAU - Nouri, Keyvan AU - Nouri K LA - eng PT - Journal Article PL - United States TA - Skinmed JT - Skinmed JID - 101168327 SB - IM MH - Humans MH - Ischemia/*etiology MH - Raynaud Disease/*complications/diagnosis/physiopathology MH - Skin/*blood supply MH - Skin Diseases/etiology/*therapy EDAT- 2014/06/18 06:00 MHDA- 2014/07/18 06:00 CRDT- 2014/06/18 06:00 PHST- 2014/06/18 06:00 [entrez] PHST- 2014/06/18 06:00 [pubmed] PHST- 2014/07/18 06:00 [medline] PST - ppublish SO - Skinmed. 2014 Mar-Apr;12(2):80-2. PMID- 30139585 OWN - NLM STAT- MEDLINE DCOM- 20200914 LR - 20200914 IS - 1578-8989 (Electronic) IS - 0025-7753 (Linking) VI - 153 IP - 3 DP - 2019 Aug 2 TI - Loss of fingerprints due to Raynaud's phenomenon. PG - 127-128 LID - S0025-7753(18)30409-3 [pii] LID - 10.1016/j.medcli.2018.06.006 [doi] FAU - Cavallasca, Javier A AU - Cavallasca JA AD - Sección de Reumatología y Enfermedades Autoinmunes Sistémicas, Hospital JB Iturraspe, Santa Fe, Argentina. Electronic address: jcavallasca@yahoo.com.ar. FAU - Riera, Julia L AU - Riera JL AD - Sección de Reumatología y Enfermedades Autoinmunes Sistémicas, Hospital JB Iturraspe, Santa Fe, Argentina. FAU - Musuruana, Jorge L AU - Musuruana JL AD - Sección de Reumatología y Enfermedades Autoinmunes Sistémicas, Hospital JB Iturraspe, Santa Fe, Argentina. LA - eng LA - spa PT - Case Reports PT - Letter TT - Borramiento de huellas dactilares debido a fenómeno de Raynaud. DEP - 20180820 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM MH - *Dermatoglyphics MH - Female MH - Fingers/*pathology MH - Humans MH - Raynaud Disease/diagnosis/*pathology MH - Young Adult EDAT- 2018/08/25 06:00 MHDA- 2020/09/15 06:00 CRDT- 2018/08/25 06:00 PHST- 2018/05/25 00:00 [received] PHST- 2018/06/06 00:00 [revised] PHST- 2018/06/14 00:00 [accepted] PHST- 2018/08/25 06:00 [pubmed] PHST- 2020/09/15 06:00 [medline] PHST- 2018/08/25 06:00 [entrez] AID - S0025-7753(18)30409-3 [pii] AID - 10.1016/j.medcli.2018.06.006 [doi] PST - ppublish SO - Med Clin (Barc). 2019 Aug 2;153(3):127-128. doi: 10.1016/j.medcli.2018.06.006. Epub 2018 Aug 20. PMID- 18567613 OWN - NLM STAT- MEDLINE DCOM- 20090209 LR - 20191210 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 58 IP - 6 DP - 2008 Sep TI - The validity of Raynaud's phenomenon symptoms in HAVS cases. PG - 431-5 LID - 10.1093/occmed/kqn075 [doi] AB - BACKGROUND: Raynaud's phenomenon, a common manifestation of the hand-arm vibration syndrome (HAVS), is typically diagnosed by a subjective history provided by employees. AIM: This study evaluates the validity of the subjective history of Raynaud's phenomenon provided by individuals applying for compensation for HAVS. METHODS: Thirty-six workers with a history of occupational hand-arm vibration exposure who were labelled as having Raynaud's phenomenon were asked to photographically document their finger symptoms before undergoing a detailed clinical assessment. Each individual was provided with a disposable camera and instructions. Returned photographs were reviewed for signs of Raynaud's phenomenon. The reliability of photograph interpretation was tested with three physicians and a non-physician. RESULTS: Inter and intra-rater reliability was very good, Kappa coefficient >0.80. Six individuals (17%) did not return cameras. Thirty individuals provided photographs and underwent a clinical evaluation. The photographs of 13 individuals (43%) did not show Raynaud's phenomenon and for four of these the diagnosis was not supported by careful symptom history. Seventeen individuals (57%) had photographic evidence of Raynaud's phenomenon. CONCLUSIONS: A presenting history of Raynaud's phenomenon in workers seeking compensation for HAVS may not be accurate since approximately half the cases are unable to provide objective photographic evidence of Raynaud's phenomenon. FAU - Youakim, Sami AU - Youakim S AD - Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. syouakim@telusplanet.net LA - eng PT - Journal Article PT - Validation Study DEP - 20080620 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM CIN - Occup Med (Lond). 2009 Jan;59(1):67. doi: 10.1093/occmed/kqn162. PMID: 19114432 MH - British Columbia MH - Hand-Arm Vibration Syndrome/*complications/diagnosis MH - Humans MH - Occupational Exposure/adverse effects MH - *Photography/methods MH - Raynaud Disease/*diagnosis/etiology MH - *Workers' Compensation EDAT- 2008/06/24 09:00 MHDA- 2009/02/10 09:00 CRDT- 2008/06/24 09:00 PHST- 2008/06/24 09:00 [pubmed] PHST- 2009/02/10 09:00 [medline] PHST- 2008/06/24 09:00 [entrez] AID - kqn075 [pii] AID - 10.1093/occmed/kqn075 [doi] PST - ppublish SO - Occup Med (Lond). 2008 Sep;58(6):431-5. doi: 10.1093/occmed/kqn075. Epub 2008 Jun 20. PMID- 36840449 OWN - NLM STAT- MEDLINE DCOM- 20230524 LR - 20230524 IS - 1525-1470 (Electronic) IS - 0736-8046 (Linking) VI - 40 IP - 3 DP - 2023 May-Jun TI - Botulinum toxin for refractory Raynaud's phenomenon: A "how to" guide for pediatric patients. PG - 587-589 LID - 10.1111/pde.15229 [doi] AB - Raynaud's phenomenon describes symptoms caused by digital vascular spasm and is classically induced by cold exposure. Severe cases can result in ulceration, necrosis, and digital autoamputation. When standard and adjunctive medical therapies fail or are contraindicated, botulinum toxin A (BTX-A) is an effective treatment option that can be added to existing regimens and should be considered before utilizing rescue therapies associated with higher risk and often higher cost. This report describes our technique, highlights considerations relevant to pediatric patients, and provides photos and videos of the procedure performed on a 16-year-old girl. CI - © 2023 Wiley Periodicals LLC. FAU - Barry, Kelly K AU - Barry KK AUID- ORCID: 0000-0003-0382-4470 AD - Dermatology Program, Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. AD - Tufts University School of Medicine, Boston, Massachusetts, USA. FAU - Reusch, Diana B AU - Reusch DB AD - Dermatology Program, Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Shahriari, Neda AU - Shahriari N AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Lonowski, Sarah AU - Lonowski S AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Dedeoglu, Fatma AU - Dedeoglu F AD - Rheumatology Program, Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Vleugels, Ruth Ann AU - Vleugels RA AD - Dermatology Program, Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230225 PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Female MH - Humans MH - Child MH - Adolescent MH - *Botulinum Toxins, Type A/therapeutic use MH - Treatment Outcome MH - *Skin Ulcer/etiology MH - *Raynaud Disease/drug therapy/diagnosis MH - Necrosis EDAT- 2023/02/26 06:00 MHDA- 2023/05/24 06:42 CRDT- 2023/02/25 04:50 PHST- 2022/08/13 00:00 [received] PHST- 2022/12/04 00:00 [accepted] PHST- 2023/05/24 06:42 [medline] PHST- 2023/02/26 06:00 [pubmed] PHST- 2023/02/25 04:50 [entrez] AID - 10.1111/pde.15229 [doi] PST - ppublish SO - Pediatr Dermatol. 2023 May-Jun;40(3):587-589. doi: 10.1111/pde.15229. Epub 2023 Feb 25. PMID- 26016221 OWN - NLM STAT- MEDLINE DCOM- 20150618 LR - 20181202 IS - 1330-0164 (Print) IS - 1330-0164 (Linking) VI - 68 IP - 3 DP - 2014 Jun TI - [Raynaud's phenomenon - first sign of malignancy: case report]. PG - 295-8 AB - Raynaud's phenomenon is a common phenomenon in the general population. It most commonly occurs in healthy individuals, in whom there is no associated illness or any other cause of Raynaud's phenomenon (primary or idiopathic Raynaud's phenomenon). Secondary Raynaud's phenomenon is common with rheumatic diseases (systemic sclerosis, systemic lupus erythematosus, primary Sjögren's syndrome, mixed connective tissue disease, etc.), occlusive vascular diseases, hematologic disorders, use of vibrating tools and use of some medications, and rarely with malignancy. We report on a patient who presented with a three-week history of painful Raynaud's attacks, which was the reason for seeking assistance of internists in emergency clinic. Upon admission to the hospital and diagnostic work-up, adenocarcinoma of the lung was found. Antinuclear antibodies (ANA), anti-dsDNA antibodies, anticardiolipin IgM and IgG antibodies were present in a lower titer. It is known that rheumatoid factor or ANA characteristic of rheumatic disease are often present in patients with paraneoplastic rheumatic syndromes, which can lead to wrong conclusions about the possible systemic connective tissue diseases and ultimately delay the correct diagnosis. The first appearance of Raynaud's phenomenon as an isolated symptom in people older than 50, with painful signs of ischemia, as in our patient, or the occurrence of asymmetric grasping fingers, especially in men, regardless of the presence of RF, ANA, anti-dsDNA or other autoantibodies, requires broader diagnostic evaluation for malignancy. FAU - Sutić, Anamarija AU - Sutić A FAU - Gračanin, Gudelj AU - Gračanin G FAU - Morović-Vergles, Jadranka AU - Morović-Vergles J LA - hrv PT - Case Reports PT - Journal Article TT - Raynaudov fenomen kao prvi znak maligne bolesti: prikaz bolesnice. PL - Croatia TA - Acta Med Croatica JT - Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti JID - 9208249 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adenocarcinoma/complications/*diagnosis MH - Antibodies, Antinuclear/*blood MH - Disease Progression MH - Early Diagnosis MH - Humans MH - Lung Neoplasms/complications/*diagnosis MH - Male MH - Middle Aged MH - Raynaud Disease/*blood/*diagnosis/etiology EDAT- 2015/05/29 06:00 MHDA- 2015/06/19 06:00 CRDT- 2015/05/29 06:00 PHST- 2015/05/29 06:00 [entrez] PHST- 2015/05/29 06:00 [pubmed] PHST- 2015/06/19 06:00 [medline] PST - ppublish SO - Acta Med Croatica. 2014 Jun;68(3):295-8. PMID- 38530533 OWN - NLM STAT- MEDLINE DCOM- 20240509 LR - 20240821 IS - 1573-9686 (Electronic) IS - 0090-6964 (Linking) VI - 52 IP - 6 DP - 2024 Jun TI - An Experimental Phototherapy Device for Studying the Effects of Blue Light on Patients with Raynaud's Phenomenon. PG - 1732-1743 LID - 10.1007/s10439-024-03487-z [doi] AB - Raynaud's phenomenon (RP) is a condition that causes decreased blood flow to areas perfused by small blood vessels (e.g., fingers, toes). In severe cases, ulceration, gangrene, and loss of fingers may occur. Most treatments focus on inducing vasorelaxation in affected areas by the way of pharmaceuticals. Recently, animal studies have shown that vasorelaxation can be induced by non-coherent blue light (wavelength ~ 430-460 nm) through the actions of melanopsin, a photoreceptive opsin protein encoded by the OPN4 gene. To study this effect in humans, a reliable phototherapy device (PTD) is needed. We outline the construction of a PTD to be used in studying blue light effects on Raynaud's patients. Our design addresses user safety, calibration, electromagnetic compatibility/interference (EMC/EMI), and techniques for measuring physiological responses (temperature sensors, laser Doppler flow sensors, infrared thermal imaging of the hands). We tested our device to ensure (1) safe operating conditions, (2) predictable, user-controlled irradiance output levels, (3) an ability for measuring physiological responses, and (4) features necessary to enable a double-blinded crossover study for a clinical trial. We also include in the Methods an approved research protocol utilizing our device that may serve as a starting point for clinical study. We introduced a reliable PTD for studying the effects of blue light therapy for patients suffering from Raynaud's phenomenon and showed that our device is safe and reliable and includes the required measurement vectors for tracking treatment effects throughout the duration of a clinical study. CI - © 2024. The Author(s) under exclusive licence to Biomedical Engineering Society. FAU - Levac, Brett AU - Levac B AUID- ORCID: 0000-0002-1182-3438 AD - Electrical and Computer Engineering, University of Texas at Austin, 2501 Speedway, Austin, 78712, USA. blevac@utexas.edu. FAU - Kerber, James AU - Kerber J AD - University of Minnesota Law School, 229 S 19th Ave, Minneapolis, MN, 55455, USA. FAU - Wagner, Emily AU - Wagner E AD - University of New England, Public Health, 716 Stevens Ave, Portland, ME, 04103, USA. FAU - Molitor, Jerry A AU - Molitor JA AD - University of Minnesota, Scleroderma Clinic, 717 Delaware St. SE, Minneapolis, MN, 55414, USA. FAU - Saliterman, Steven S AU - Saliterman SS AD - Biomedical Engineering, University of Minnesota, 312 Church St. S.E. Ste 7-105, Minneapolis, MN, 55455, USA. LA - eng PT - Journal Article DEP - 20240326 PL - United States TA - Ann Biomed Eng JT - Annals of biomedical engineering JID - 0361512 SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - Blue Light MH - *Phototherapy/instrumentation MH - *Raynaud Disease/therapy/physiopathology OTO - NOTNLM OT - Blue light OT - Laser doppler flowmetry OT - Melanopsin OT - Phototherapy OT - Raynaud’s OT - Thermography OT - Vasorelaxation EDAT- 2024/03/26 18:43 MHDA- 2024/05/10 00:43 CRDT- 2024/03/26 12:21 PHST- 2023/10/25 00:00 [received] PHST- 2024/02/28 00:00 [accepted] PHST- 2024/05/10 00:43 [medline] PHST- 2024/03/26 18:43 [pubmed] PHST- 2024/03/26 12:21 [entrez] AID - 10.1007/s10439-024-03487-z [pii] AID - 10.1007/s10439-024-03487-z [doi] PST - ppublish SO - Ann Biomed Eng. 2024 Jun;52(6):1732-1743. doi: 10.1007/s10439-024-03487-z. Epub 2024 Mar 26. PMID- 38465507 OWN - NLM STAT- MEDLINE DCOM- 20240516 LR - 20260320 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 20 IP - 6 DP - 2024 Jun TI - Re-evaluation of nailfold capillaroscopy in discriminating primary from secondary Raynaud's phenomenon and in predicting systemic sclerosis: a randomised observational prospective cohort study. PG - 665-672 LID - 10.1080/1744666X.2024.2313642 [doi] AB - BACKGROUND: Primary Raynaud's phenomenon (pRP) is difficult to distinguish from secondary (sRP). Although nailfold capillaroscopy (NFC) may detect early alterations, no universal criteria yet discriminate between pRP from sRP. OBJECTIVES: To create and validate two NFC scores that could distinguish pRP from sRP and that could predict systemic sclerosis (SSc), respectively. METHODS: We performed NFC on two separate cohorts with isolated RP, and recorded number of capillaries per field, enlarged/giant capillaries, crossed/bizarre patterns, microhemorrhages, neoangiogenesis, rarefaction, edema, blood flow velocity, stasis. By multivariate regression analysis, we evaluated the adjusted prognostic role of these features in a derivation cohort of 656 patients. Results were used to construct algorithm-based prognostic scores (A and B). These scores were then tested on a confirmation cohort of 219 patients. RESULTS: Score A was unable to discriminate sRP from pRP (low negative predictive values with high positive predictive values for any cut-point); score B was unable to discriminate progression to SSc or a SSc-spectrum disorder (low positive predictive values with high negative predictive values for lower cut-points). CONCLUSION: NFC patterns, believed as specific, showed low discriminatory power and on their own are unable to reliably discriminate sRP from pRP or predict evolution to SSc. FAU - Amaral, Marta C AU - Amaral MC AUID- ORCID: 0000-0002-6985-8852 AD - Immune response and vascular disease, iNOVA4Health, NOVA Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. AD - UDIMS - Unidade de Doenças Imuno-Mediadas Sistémicas, Departamento de Medicina IV, Hospital Prof. Doutor Fernando Fonseca, E.P.E, Amadora, Portugal. FAU - Paula, F Seguro AU - Paula FS AUID- ORCID: 0000-0003-4429-7486 AD - Immune response and vascular disease, iNOVA4Health, NOVA Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. AD - UDIMS - Unidade de Doenças Imuno-Mediadas Sistémicas, Departamento de Medicina IV, Hospital Prof. Doutor Fernando Fonseca, E.P.E, Amadora, Portugal. FAU - Caetano, Joana AU - Caetano J AD - Immune response and vascular disease, iNOVA4Health, NOVA Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. AD - UDIMS - Unidade de Doenças Imuno-Mediadas Sistémicas, Departamento de Medicina IV, Hospital Prof. Doutor Fernando Fonseca, E.P.E, Amadora, Portugal. FAU - Ames, Paul Rj AU - Ames PR AD - Immune response and vascular disease, iNOVA4Health, NOVA Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. AD - Department of Haematology, Dumfries & Galloway Royal Infirmary, Cargenbridge, Scotland, UK. FAU - Alves, J Delgado AU - Alves JD AD - Immune response and vascular disease, iNOVA4Health, NOVA Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. AD - UDIMS - Unidade de Doenças Imuno-Mediadas Sistémicas, Departamento de Medicina IV, Hospital Prof. Doutor Fernando Fonseca, E.P.E, Amadora, Portugal. LA - eng PT - Journal Article PT - Observational Study DEP - 20240311 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Capillaries/diagnostic imaging/pathology MH - Cohort Studies MH - Diagnosis, Differential MH - *Microscopic Angioscopy/methods MH - Nails/blood supply/pathology MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Systemic/diagnosis OTO - NOTNLM OT - Nailfold capillaroscopy OT - connective tissue disease OT - primary Raynaud’s phenomenon OT - raynaud’s disease OT - secondary Raynaud’s phenomenon OT - systemic sclerosis OT - videocapillaroscopy EDAT- 2024/03/11 06:43 MHDA- 2024/05/16 18:42 CRDT- 2024/03/11 05:43 PHST- 2024/05/16 18:42 [medline] PHST- 2024/03/11 06:43 [pubmed] PHST- 2024/03/11 05:43 [entrez] AID - 10.1080/1744666X.2024.2313642 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2024 Jun;20(6):665-672. doi: 10.1080/1744666X.2024.2313642. Epub 2024 Mar 11. PMID- 18208825 OWN - NLM STAT- MEDLINE DCOM- 20080305 LR - 20081204 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 2 DP - 2008 Feb TI - Sparing of the thumb in Raynaud's phenomenon. PG - 219-21 LID - 10.1093/rheumatology/kem353 [doi] AB - OBJECTIVES: To conduct a prospective study to determine which digits are affected (and whether the thumb is spared or not) in a cohort of patients with RP as assessed by symptoms and thermography and to determine whether the degree of thumb involvement differs between primary (PRP) and secondary Raynaud's phenomenon (SRP). METHODS: This was a cross-sectional study of 44 patients with RP. The following characteristics were recorded to allow comparisons between digits: symptoms of RP in each digit (graded on a scale of 'never', 'sometimes' and 'always' affected during an attack of RP) and thermography at 23 degrees C. A distal-dorsal difference (DDD) in temperature at 23 degrees C of -1 degree C or less was considered to be clinically relevant. RESULTS: Symptom scores in the thumb were significantly better, i.e. less severe than in each finger (P < 0.001). As only three participants had any finger better than the thumb, there was no power to compare whether the thumb was spared more in PRP compared with SRP. Mean DDD was significantly higher (i.e. better) in the thumb compared with each finger (P < 0.001). Although DDD scores were higher in PRP compared with SRP (P = 0.01), there was no evidence that the relative effect of the thumb differed between the two groups (P = 0.26). CONCLUSIONS: Our findings confirm that the thumbs are spared in RP, both primary and secondary, as demonstrated by both symptoms and thermography. The reasons for sparing of the thumb were not addressed in this study but raised questions regarding pathophysiology. FAU - Chikura, B AU - Chikura B AD - The Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK. docbatsi@aol.com FAU - Moore, T L AU - Moore TL FAU - Manning, J B AU - Manning JB FAU - Vail, A AU - Vail A FAU - Herrick, A L AU - Herrick AL LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) SB - IM CIN - Rheumatology (Oxford). 2008 Aug;47(8):1260; author reply 1260. doi: 10.1093/rheumatology/ken228. PMID: 18559372 MH - Adolescent MH - Adult MH - Aged MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Cross-Sectional Studies MH - Female MH - Fingers/*physiology MH - Functional Laterality MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/classification/drug therapy/etiology/*physiopathology MH - Skin Pigmentation MH - Smoking/epidemiology MH - Thumb/*physiopathology EDAT- 2008/01/23 09:00 MHDA- 2008/03/06 09:00 CRDT- 2008/01/23 09:00 PHST- 2008/01/23 09:00 [pubmed] PHST- 2008/03/06 09:00 [medline] PHST- 2008/01/23 09:00 [entrez] AID - 47/2/219 [pii] AID - 10.1093/rheumatology/kem353 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Feb;47(2):219-21. doi: 10.1093/rheumatology/kem353. PMID- 31028378 OWN - NLM STAT- MEDLINE DCOM- 20200514 LR - 20200514 IS - 1460-2393 (Electronic) IS - 1460-2725 (Print) IS - 1460-2393 (Linking) VI - 112 IP - 7 DP - 2019 Jul 1 TI - Raynaud's phenomenon and the nailfold capillaroscopic findings in a guitar player. PG - 531-533 LID - 10.1093/qjmed/hcz095 [doi] FAU - Sirufo, M M AU - Sirufo MM AD - Department of Life, Health, & Environmental Sciences, University of L'Aquila, Piazzale Salvatore Tommasi 1, 67100 L'Aquila, Italy. AD - Allergology and Clinical Immunology Unit, AUSL 04 Teramo, Italy. FAU - Ginaldi, L AU - Ginaldi L AD - Department of Life, Health, & Environmental Sciences, University of L'Aquila, Piazzale Salvatore Tommasi 1, 67100 L'Aquila, Italy. AD - Allergology and Clinical Immunology Unit, AUSL 04 Teramo, Italy. FAU - De Martinis, M AU - De Martinis M AD - Department of Life, Health, & Environmental Sciences, University of L'Aquila, Piazzale Salvatore Tommasi 1, 67100 L'Aquila, Italy. AD - Allergology and Clinical Immunology Unit, AUSL 04 Teramo, Italy. LA - eng PT - Case Reports PT - Journal Article PL - England TA - QJM JT - QJM : monthly journal of the Association of Physicians JID - 9438285 SB - IM MH - Adult MH - Capillaries/*pathology MH - Fingers MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Music MH - Occupational Diseases/*etiology MH - Raynaud Disease/*diagnosis/etiology PMC - PMC6609905 EDAT- 2019/04/28 06:00 MHDA- 2020/05/15 06:00 PMCR- 2019/04/26 CRDT- 2019/04/28 06:00 PHST- 2019/04/09 00:00 [received] PHST- 2019/04/12 00:00 [revised] PHST- 2019/04/28 06:00 [pubmed] PHST- 2020/05/15 06:00 [medline] PHST- 2019/04/28 06:00 [entrez] PHST- 2019/04/26 00:00 [pmc-release] AID - 5480689 [pii] AID - hcz095 [pii] AID - 10.1093/qjmed/hcz095 [doi] PST - ppublish SO - QJM. 2019 Jul 1;112(7):531-533. doi: 10.1093/qjmed/hcz095. PMID- 28431994 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1527-9995 (Electronic) IS - 0090-4295 (Linking) VI - 106 DP - 2017 Aug TI - Raynaud's Phenomenon: Revisiting a Rare Sign of Pheochromocytoma and Paraganglioma. PG - e3-e4 LID - S0090-4295(17)30367-9 [pii] LID - 10.1016/j.urology.2017.04.014 [doi] AB - Paraganglioma (PGL) are rare tumors arising from extra-adrenal chromaffin cells and occasionally secret catecholamines. The patient commonly presents with headache, palpitation, anxiety, diaphoresis, and episodic or sustained hypertension. Rarely patient can present with Raynaud's phenomenon. We present a case of adolescent girl who presented with isolated Raynaud's phenomenon as only manifestation of metastasis of PGL 3 years after undergoing surgical excision of normetanephrine secreting abdominal PGL. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Patil, Virendra Ashokrao AU - Patil VA AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. Electronic address: viru.patil33@gmail.com. FAU - Kasaliwal, Rajiv AU - Kasaliwal R AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. FAU - Goroshi, Manjunath R AU - Goroshi MR AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. FAU - Lila, Anurag R AU - Lila AR AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. FAU - Bandgar, Tushar AU - Bandgar T AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. FAU - Shah, Nalini S AU - Shah NS AD - Department of Endocrinology, Seth GS Medical College & KEM Hospital, Mumbai, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20170418 PL - United States TA - Urology JT - Urology JID - 0366151 SB - IM MH - Abdominal Neoplasms/*diagnostic imaging/*pathology/therapy MH - Adolescent MH - Female MH - Humans MH - Paraganglioma/*diagnostic imaging/*secondary/therapy MH - Positron Emission Tomography Computed Tomography MH - Raynaud Disease/*etiology EDAT- 2017/04/23 06:00 MHDA- 2018/12/12 06:00 CRDT- 2017/04/23 06:00 PHST- 2016/12/22 00:00 [received] PHST- 2017/04/08 00:00 [revised] PHST- 2017/04/11 00:00 [accepted] PHST- 2017/04/23 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2017/04/23 06:00 [entrez] AID - S0090-4295(17)30367-9 [pii] AID - 10.1016/j.urology.2017.04.014 [doi] PST - ppublish SO - Urology. 2017 Aug;106:e3-e4. doi: 10.1016/j.urology.2017.04.014. Epub 2017 Apr 18. PMID- 19238503 OWN - NLM STAT- MEDLINE DCOM- 20090722 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 6 DP - 2009 Jun TI - Dynamic Doppler evaluation of the hand arteries of the patients with Raynaud's disease. PG - 679-83 LID - 10.1007/s10067-009-1131-1 [doi] AB - The aim of this study was the determination of blood flow characteristics and parameters in the hand arteries of patients with primary Raynaud's phenomenon (pRP) and comparison with the results of healthy subjects. The diameter, resistive index and flow volume of the digital, ulnar and radial arteries of the patients with pRP and the control group were measured at rest and after cold provocation. The flow starting time (FST) in the digital artery and the flow normalising time (FNT) of all three arteries were also recorded after cold provocation. The diameter and flow volume of the digital and ulnar arteries of the patients were lower at rest, but resistive index was significantly high in all arteries. After cold provocation, the diameters of the radial and ulnar arteries and the flow volume of the digital arteries of the patients were significantly lower than those of the controls. The mean FST was 3.6 +/- 3.8 min for the patients and 0.9 +/- 1.2 min for the controls. The mean FNT was significantly longer in all the arteries of the patients; FNT cutoff times for the radial, ulnar and digital arteries were 6.5, 5.5 and 6.5 min, respectively. The measurements of the diameter, resistive index and flow volume of all the arteries measured before and after cold provocation as well as FST of the digital artery and FNT of all the arteries may facilitate in providing additional information in pRP patients. FAU - Toprak, Uğur AU - Toprak U AD - Ankara Numune Training and Research Hospital, Talatpasa Bulvari, Altindag, Ankara, Turkey. toprakugur@yahoo.com FAU - Selvi, Nadir Alper AU - Selvi NA FAU - Ateş, Aşkin AU - Ateş A FAU - Erhuner, Zeynep AU - Erhuner Z FAU - Bostanoğlu, Sevinç AU - Bostanoğlu S FAU - Karademir, Mehmet Alp AU - Karademir MA FAU - Karaaslan, Yaşar AU - Karaaslan Y LA - eng PT - Journal Article DEP - 20090224 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Blood Flow Velocity/physiology MH - Case-Control Studies MH - Cold Temperature/adverse effects MH - Female MH - Fingers/*blood supply/diagnostic imaging MH - Hand/*blood supply/diagnostic imaging MH - Humans MH - Male MH - Middle Aged MH - Radial Artery/diagnostic imaging/*physiopathology MH - Raynaud Disease/*diagnostic imaging/*physiopathology MH - Regional Blood Flow/*physiology MH - Ulnar Artery/diagnostic imaging/*physiopathology MH - Ultrasonography, Doppler, Color MH - Ultrasonography, Doppler, Pulsed MH - Vascular Resistance/physiology MH - Young Adult EDAT- 2009/02/25 09:00 MHDA- 2009/07/23 09:00 CRDT- 2009/02/25 09:00 PHST- 2008/05/05 00:00 [received] PHST- 2009/02/09 00:00 [accepted] PHST- 2009/01/21 00:00 [revised] PHST- 2009/02/25 09:00 [entrez] PHST- 2009/02/25 09:00 [pubmed] PHST- 2009/07/23 09:00 [medline] AID - 10.1007/s10067-009-1131-1 [doi] PST - ppublish SO - Clin Rheumatol. 2009 Jun;28(6):679-83. doi: 10.1007/s10067-009-1131-1. Epub 2009 Feb 24. PMID- 22691218 OWN - NLM STAT- MEDLINE DCOM- 20120813 LR - 20250626 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 30 IP - 2 Suppl 71 DP - 2012 Mar-Apr TI - Use of infrared thermography as an endpoint in therapeutic trials of Raynaud's phenomenon and systemic sclerosis. PG - S103-15 AB - OBJECTIVES: To perform a systematic review evaluating the use of infrared thermography (IRT) as an endpoint in clinical trials of Raynaud's phenomenon (RP). METHODS: Articles reporting the use of IRT and Raynaud's phenomenon were identified following systematic searches of PubMed, EMBase, MEDLINE and AMED databases. Articles incorporating IRT as an endpoint in a therapeutic trial were selected for full text analysis. Data extracted from articles included study design, study size, intervention, clinical and thermographic endpoints, and outcomes. Studies were scored on their methodological quality. Data analysis involved a descriptive analysis of the studies, along with a secondary analysis focusing on agreement between clinical and thermographic outcomes in the larger, better-described studies. RESULTS: Thirty-two studies evaluating 654 patients with RP were assessed. Significant heterogeneity between studies precluded any attempt at formal meta-analysis. Most studies were small (median 15.5 patients) and open-label design (19/32, 59.4%). The majority of studies (18/32, 56.3%) reported improvements in both clinical and thermographic endpoints. Thermographic parameters showing agreement with clinical endpoints in therapeutic trials included baseline hand/finger absolute temperature and parameters derived following local cold challenge, including longitudinal thermal gradients and percent re-warming. CONCLUSIONS: No single thermographic parameter has emerged as the preferred endpoint for clinical trials of RP. Objective microvascular imaging tools such as IRT have the potential to overcome the limitations of self-report assessment of RP. Future studies should continue to evaluate IRT, alongside recommended self-reports, in an attempt to validate objective microvascular assessment tools in therapeutic trials of RP. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. john.pauling@rnhrd.nhs.uk FAU - Shipley, Jacqueline A AU - Shipley JA FAU - Harris, Nigel D AU - Harris ND FAU - McHugh, Neil J AU - McHugh NJ LA - eng PT - Journal Article PT - Systematic Review DEP - 20120530 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Clinical Trials as Topic/*methods MH - Endpoint Determination MH - Hand/*blood supply MH - Humans MH - *Infrared Rays MH - *Microcirculation MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/physiopathology/*therapy MH - Research Design MH - Scleroderma, Systemic/diagnosis/physiopathology/*therapy MH - Thermography/*methods MH - Treatment Outcome EDAT- 2012/07/26 06:00 MHDA- 2012/08/14 06:00 CRDT- 2012/06/14 06:00 PHST- 2012/03/28 00:00 [received] PHST- 2012/05/15 00:00 [accepted] PHST- 2012/06/14 06:00 [entrez] PHST- 2012/07/26 06:00 [pubmed] PHST- 2012/08/14 06:00 [medline] AID - 5997 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2012 Mar-Apr;30(2 Suppl 71):S103-15. Epub 2012 May 30. PMID- 41689194 OWN - NLM STAT- In-Process DCOM- 20260306 LR - 20260608 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 65 IP - 2 DP - 2026 Feb 4 TI - Assessing digital vasculopathy in systemic sclerosis. LID - keag081 [pii] LID - 10.1093/rheumatology/keag081 [doi] AB - Digital vasculopathy (a spectrum of Raynaud's phenomenon, digital ulceration and critical ischaemia) is one of the most characteristic manifestations of systemic sclerosis (SSc). It is an area of unmet need with a major impact on quality of life: current treatments are only poorly effective. SSc-related digital vasculopathy is a result of structural as well as functional change at the level of both the microcirculation and the digital artery, explaining its severity. This review begins with a brief description of digital vasculopathy, followed by its assessment in the clinical setting, relevant to both diagnosis and monitoring of SSc. Outcome measures of Raynaud's phenomenon and of digital ulcers are then discussed, focusing on recent advances. These outcome measures are a 'hot topic' because reliable patient-reported and laboratory-based outcome measures will facilitate much needed clinical trials. Finally, some of the emerging non-invasive technologies which are providing new insights into pathophysiology are briefly described. CI - © The Author(s) 2026. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom. AD - National Institute for Health and Care Research (NIHR) Manchester Biomedical Research Centre, Manchester, United Kingdom. AD - Department of Rheumatology, Aberdeen Royal Infirmary, Foresterhill, NHS Grampian, Aberdeen and Centre for Arthritis and Musculoskeletal Health, University of Aberdeen, Aberdeen, United Kingdom. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/physiopathology/diagnosis MH - *Fingers/blood supply MH - *Raynaud Disease/etiology/diagnosis/physiopathology MH - Skin Ulcer/etiology/diagnosis MH - Ischemia/etiology/diagnosis MH - Quality of Life OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ulcers OT - nailfold capillaroscopy OT - outcome measures OT - systemic sclerosis EDAT- 2026/02/14 05:30 MHDA- 2026/03/06 13:44 CRDT- 2026/02/14 00:24 PHST- 2025/09/27 00:00 [received] PHST- 2026/01/26 00:00 [accepted] PHST- 2026/03/06 13:44 [medline] PHST- 2026/02/14 05:30 [pubmed] PHST- 2026/02/14 00:24 [entrez] AID - 8483062 [pii] AID - 10.1093/rheumatology/keag081 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 Feb 4;65(2):keag081. doi: 10.1093/rheumatology/keag081. PMID- 22711208 OWN - NLM STAT- MEDLINE DCOM- 20130312 LR - 20160526 IS - 1098-8947 (Electronic) IS - 0743-684X (Linking) VI - 28 IP - 8 DP - 2012 Oct TI - The use of noninvasive angiography for digital ischemia in Raynaud's disease. PG - 569-70 LID - 10.1055/s-0032-1315776 [doi] FAU - Rozen, Warren M AU - Rozen WM FAU - Ye, Xuan AU - Ye X FAU - Overstall, Simon AU - Overstall S FAU - Leong, James AU - Leong J LA - eng PT - Letter DEP - 20120618 PL - United States TA - J Reconstr Microsurg JT - Journal of reconstructive microsurgery JID - 8502670 SB - IM MH - Hand/*blood supply MH - Humans MH - Ischemia/*pathology MH - Magnetic Resonance Angiography/*methods MH - Raynaud Disease/*pathology EDAT- 2012/06/20 06:00 MHDA- 2013/03/13 06:00 CRDT- 2012/06/20 06:00 PHST- 2012/06/20 06:00 [entrez] PHST- 2012/06/20 06:00 [pubmed] PHST- 2013/03/13 06:00 [medline] AID - 10.1055/s-0032-1315776 [doi] PST - ppublish SO - J Reconstr Microsurg. 2012 Oct;28(8):569-70. doi: 10.1055/s-0032-1315776. Epub 2012 Jun 18. PMID- 19959302 OWN - NLM STAT- MEDLINE DCOM- 20100506 LR - 20181201 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 35 IP - 1 DP - 2010 Feb TI - [A negative first-line work-up of Raynaud's phenomenon: And what if it were cancer?]. PG - 35-7 LID - 10.1016/j.jmv.2009.10.003 [doi] AB - Raynaud's phenomenon is a transient paroxysmal vasomotor phenomenon affecting the extremities including manifestations of ischemia. It is a common phenomenon in the general population. In a routine clinical situation, the first step is to differentiate Raynaud's disease from a secondary Raynaud's phenomenon, the latter requiring complementary investigations. We report here the case of an 80-year-old woman who presented a secondary Raynaud's phenomenon. First-line investigations remained negative. A mammography was performed and revealed breast cancer. Raynaud's phenomenon disappeared after treatment of the breast carcinoma and did not recur during the 2-year follow-up. CI - Copyright 2009 Elsevier Masson SAS. All rights reserved. FAU - Auboire, L AU - Auboire L AD - Service de médecine vasculaire, CHU de Caen, avenue de la Côte-de-Nacre, 14033 Caen cedex, France. FAU - Landy, S AU - Landy S FAU - Perrot, J-Y AU - Perrot JY FAU - Maïza, D AU - Maïza D FAU - Le Hello, C AU - Le Hello C LA - fre PT - Case Reports PT - Journal Article PT - Review TT - Phénomène de Raynaud avec négativité du bilan de première intention : et si c'était un cancer ? DEP - 20091202 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 RN - 0 (Antineoplastic Agents, Hormonal) RN - 0 (Estrogens) RN - 0 (Nitriles) RN - 0 (Triazoles) RN - 0D3Q044KCA (Galantamine) RN - 2Z07MYW1AZ (Anastrozole) RN - 4G7DS2Q64Y (Progesterone) RN - CJ0O37KU29 (Verapamil) RN - KXO2KT9N0G (Pravastatin) SB - IM MH - Adenocarcinoma, Mucinous/*complications/diagnostic imaging/drug therapy/radiotherapy/surgery MH - Aged, 80 and over MH - Anastrozole MH - Antineoplastic Agents, Hormonal/therapeutic use MH - Breast Neoplasms/*complications/diagnostic imaging/drug therapy/radiotherapy/surgery MH - Combined Modality Therapy MH - Estrogens MH - Female MH - Galantamine/therapeutic use MH - Humans MH - Mammography MH - Mastectomy MH - Neoplasms, Hormone-Dependent/*complications/drug therapy/radiotherapy/surgery MH - Nitriles/therapeutic use MH - Paraneoplastic Syndromes/drug therapy/*etiology MH - Pravastatin/therapeutic use MH - Progesterone MH - Radiotherapy, Adjuvant MH - Raynaud Disease/drug therapy/*etiology MH - Seasons MH - Triazoles/therapeutic use MH - Verapamil/therapeutic use RF - 13 EDAT- 2009/12/05 06:00 MHDA- 2010/05/07 06:00 CRDT- 2009/12/05 06:00 PHST- 2009/06/11 00:00 [received] PHST- 2009/10/13 00:00 [accepted] PHST- 2009/12/05 06:00 [entrez] PHST- 2009/12/05 06:00 [pubmed] PHST- 2010/05/07 06:00 [medline] AID - S0398-0499(09)00307-2 [pii] AID - 10.1016/j.jmv.2009.10.003 [doi] PST - ppublish SO - J Mal Vasc. 2010 Feb;35(1):35-7. doi: 10.1016/j.jmv.2009.10.003. Epub 2009 Dec 2. PMID- 40465410 OWN - NLM STAT- MEDLINE DCOM- 20251003 LR - 20251005 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 10 DP - 2025 Oct 1 TI - Frequency-specific microcurrent improves hand function and Raynaud's symptoms in scleroderma: results of two pilot studies. PG - 5504-5508 LID - 10.1093/rheumatology/keaf301 [doi] AB - OBJECTIVES: To evaluate the efficacy of frequency-specific microcurrent as a treatment designed to improve hand function and alleviate Raynaud's symptoms in patients with systemic sclerosis. METHODS: Seventeen patients were treated in two separate pilot studies for 60 and 45 min, respectively, over 1 or 2 days with microcurrent frequencies specific to scarring in the hands and capillaries, and to Raynaud's disease. Efficacy was evaluated using the Cochin hand function score questionnaire before and after treatment, and a visual analogue scale to evaluate the severity of the Raynaud's symptoms. RESULTS: Improvement in hand function was observed in both studies, being significant in the second pilot study with an average improvement of 38% (95% CI 22%-55%, P = 0.002) and in both studies combined with an average improvement of 40% (95% CI 26%-55%, P = 0.0001). There was a significant improvement in mean Raynaud's VAS scores pre- and post-treatment of 18 points (out of 100), 95% CI 3.3-33 points, P = 0.016. A proportion of patients achieved very substantial improvements in hand function, with particular tasks improving from being nearly impossible to do to being able to be performed without difficulty. CONCLUSIONS: The degree of improvement in hand function obtained over a very short treatment period of 45 min to 1 h was significant. Further randomized, controlled studies are needed to confirm these results, and longer-term follow-up will be required to establish whether the changes are maintained and whether repeated treatments can produce further improvements. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Gregory, Walter M AU - Gregory WM AUID- ORCID: 0000-0003-2641-8416 AD - Division of Clinical Medicine, University of Sheffield, Sheffield, UK. FAU - Bagley, Katherine AU - Bagley K AD - Chapel Allerton Osteopath, Leeds, UK. FAU - Eng, Sookhoe AU - Eng S AD - University of Leeds Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, UK. FAU - McMakin, Carolyn AU - McMakin C AD - Fibromyalgia and Myofascial Pain Clinic of Portland, Portland, OR, USA. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - University of Leeds Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, UK. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Raynaud Disease/therapy/physiopathology/etiology MH - Pilot Projects MH - Female MH - Male MH - Middle Aged MH - *Hand/physiopathology MH - *Scleroderma, Systemic/complications/physiopathology/therapy MH - Adult MH - Treatment Outcome MH - Severity of Illness Index MH - Aged PMC - PMC12494225 OTO - NOTNLM OT - Cochin OT - FSM OT - Raynaud’s OT - capillaries OT - frequencies OT - microcurrent OT - scarring OT - scleroderma OT - sclerosis OT - skin EDAT- 2025/06/04 18:30 MHDA- 2025/10/03 18:28 PMCR- 2025/06/04 CRDT- 2025/06/04 12:12 PHST- 2025/02/27 00:00 [received] PHST- 2025/05/19 00:00 [accepted] PHST- 2025/10/03 18:28 [medline] PHST- 2025/06/04 18:30 [pubmed] PHST- 2025/06/04 12:12 [entrez] PHST- 2025/06/04 00:00 [pmc-release] AID - 8156779 [pii] AID - keaf301 [pii] AID - 10.1093/rheumatology/keaf301 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Oct 1;64(10):5504-5508. doi: 10.1093/rheumatology/keaf301. PMID- 21875018 OWN - NLM STAT- MEDLINE DCOM- 20111031 LR - 20110830 IS - 0374-1338 (Print) IS - 0374-1338 (Linking) VI - 57 IP - 2 DP - 2010 TI - Capillaroscopy as a prognostic tool for the development of connective tissue disease in patients with Raynaud's phenomenon. PG - 119-20 FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Dr. Subotica 8 11000 Belgrade, Serbia. nemanjadamjanov@yahoo.com FAU - Pavlov-Dolijanović, Slavica AU - Pavlov-Dolijanović S FAU - Zlatanović, Maja AU - Zlatanović M LA - eng PT - Journal Article PL - Croatia TA - Reumatizam JT - Reumatizam JID - 0216650 SB - IM MH - Connective Tissue Diseases/complications/*diagnosis MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Prognosis MH - Raynaud Disease/*complications EDAT- 2010/01/01 00:00 MHDA- 2011/11/01 06:00 CRDT- 2011/08/31 06:00 PHST- 2011/08/31 06:00 [entrez] PHST- 2010/01/01 00:00 [pubmed] PHST- 2011/11/01 06:00 [medline] PST - ppublish SO - Reumatizam. 2010;57(2):119-20. PMID- 30842016 OWN - NLM STAT- MEDLINE DCOM- 20190314 LR - 20190314 IS - 1555-9823 (Electronic) IS - 0003-1348 (Linking) VI - 84 IP - 8 DP - 2018 Aug 1 TI - Resolution of Raynaud's Symptoms after Parathyroidectomy. PG - e323-e324 FAU - Held, Jenny M AU - Held JM FAU - Daily, Jason AU - Daily J FAU - Kilfoil, Terrence AU - Kilfoil T FAU - Johnston, Michael AU - Johnston M LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am Surg JT - The American surgeon JID - 0370522 SB - IM MH - Female MH - Humans MH - Hypercalcemia/complications MH - Hyperparathyroidism/complications/*surgery MH - Middle Aged MH - *Parathyroidectomy MH - Raynaud Disease/*etiology MH - Remission, Spontaneous EDAT- 2019/03/08 06:00 MHDA- 2019/03/15 06:00 CRDT- 2019/03/08 06:00 PHST- 2019/03/08 06:00 [entrez] PHST- 2019/03/08 06:00 [pubmed] PHST- 2019/03/15 06:00 [medline] PST - ppublish SO - Am Surg. 2018 Aug 1;84(8):e323-e324. PMID- 26597492 OWN - NLM STAT- MEDLINE DCOM- 20170110 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 36 IP - 3 DP - 2016 Mar TI - Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies. PG - 365-9 LID - 10.1007/s00296-015-3396-9 [doi] AB - The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed. FAU - Corominas, Hèctor AU - Corominas H AD - Service of Rheumatology, Hospital de Sant Joan Despí Moisés Broggi-Hospital General Hospitalet, Consorci Sanitari Integral (CSI), Avda. Jacint Verdaguer 90, 08970, Sant Joan Despí, Barcelona, Catalonia, Spain. vancor@yahoo.com. FAU - Ortiz-Santamaría, Vera AU - Ortiz-Santamaría V AD - Service of Rheumatology, Hospital de Granollers, Granollers, Barcelona, Spain. veraortiz@hotmail.com. FAU - Castellví, Iván AU - Castellví I AD - Service of Rheumatology, Hospital Universitari de Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain. ivancastellvi@hotmail.com. FAU - Moreno, Mireia AU - Moreno M AD - Service of Rheumatology, Hospital Corporació Parc Taulí, Sabadell, Barcelona, Spain. mmoreno@tauli.cat. FAU - Morlà, Rosa AU - Morlà R AD - Service of Rheumatology, Hospital del Vendrell, El Vendrell, Tarragona, Spain. rmmorla@gmail.com. FAU - Clavaguera, Teresa AU - Clavaguera T AD - Service of Rheumatology, Hospital de Palamós, Palamós, Girona, Spain. tclava1@yahoo.es. FAU - Erra, Alba AU - Erra A AD - Service of Rheumatology, Hospital Sant Rafael, Barcelona, Spain. aerra@hsrafael.es. FAU - Martínez-Pardo, Silvia AU - Martínez-Pardo S AD - Service of Rheumatology, Hospital Universitari Mútua de Terrassa, Terrassa, Barcelona, Spain. smartinez@mutuaterrasa.cat. FAU - Ordóñez, Sergi AU - Ordóñez S AD - Service of Rheumatology, Hospital Arnau de Vilanova, Lleida, Spain. ordonezsergi@gmail.com. FAU - Santo, Pilar AU - Santo P AD - Service of Rheumatology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain. pili_santo@yahoo.es. FAU - Reyner, Patricia AU - Reyner P AD - Service of Rheumatology, Hospital de Santa Caterina, Girona, Spain. preyner1@hotmail.com. FAU - González, Maria José AU - González MJ AD - Service of Rheumatology, Hospital Universitari Quirón Dexeus, Barcelona, Spain. mj_gonzalez@hotmail.com. FAU - Codina, Oriol AU - Codina O AD - Service of Rheumatology, Hospital de Figueres, Figueres, Girona, Spain. ocodina@salutemporda.cat. FAU - Gelman, Mario Saul AU - Gelman MS AD - Service of Rheumatology, Fundació Althaia Hospital de Manresa, Manresa, Barcelona, Spain. 14979sga@comb.cat. FAU - Juanola-Roura, Xavier AU - Juanola-Roura X AD - Service of Rheumatology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain. x.juanola@bellvitgehospital.cat. FAU - Olivé, Alex AU - Olivé A AD - Service of Rheumatology, Hospital Universitari Germans Trias, Badalona, Barcelona, Spain. aolive.germanstrias@gencat.cat. FAU - Torrente-Segarra, Vicenç AU - Torrente-Segarra V AD - Service of Rheumatology, Hospital de Sant Joan Despí Moisés Broggi-Hospital General Hospitalet, Consorci Sanitari Integral (CSI), Avda. Jacint Verdaguer 90, 08970, Sant Joan Despí, Barcelona, Catalonia, Spain. vicente.torrente@sanitatintegral.org. CN - CapiCAT group LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20151123 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) SB - IM MH - Aged MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - *Microcirculation MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Predictive Value of Tests MH - Raynaud Disease/blood/*diagnosis/immunology/physiopathology MH - Regional Blood Flow MH - Sjogren's Syndrome/blood/*diagnosis/immunology/physiopathology MH - Spain MH - *Video Recording OTO - NOTNLM OT - Antibodies, antinuclear OT - Connective tissue diseases OT - Microscopic angioscopy OT - Raynaud’s disease OT - Sjögren’s syndrome OT - Systemic sclerosis EDAT- 2015/11/26 06:00 MHDA- 2017/01/11 06:00 CRDT- 2015/11/25 06:00 PHST- 2015/03/27 00:00 [received] PHST- 2015/11/12 00:00 [accepted] PHST- 2015/11/25 06:00 [entrez] PHST- 2015/11/26 06:00 [pubmed] PHST- 2017/01/11 06:00 [medline] AID - 10.1007/s00296-015-3396-9 [pii] AID - 10.1007/s00296-015-3396-9 [doi] PST - ppublish SO - Rheumatol Int. 2016 Mar;36(3):365-9. doi: 10.1007/s00296-015-3396-9. Epub 2015 Nov 23. PMID- 32181935 OWN - NLM STAT- MEDLINE DCOM- 20230511 LR - 20230511 IS - 1600-0781 (Electronic) IS - 0905-4383 (Linking) VI - 39 IP - 3 DP - 2023 May TI - Repeatability of high-resolution laser Doppler images of the hands in patients with systemic sclerosis and secondary Raynaud's phenomenon. PG - 193-203 LID - 10.1111/phpp.12549 [doi] AB - OBJECTIVES: The objective of the study was to establish the repeatability of baseline diagnostic images of the dorsum of the hands acquired using a high-resolution laser Doppler imager in patients with Raynaud's phenomenon secondary to systemic sclerosis (SSc). METHODS: The dorsal side of the hands of 22 patients (8 male 14 female), age range 29-73, median 62, with SSc and secondary Raynaud's phenomenon were imaged over two consecutive days at approximately the same time using a Moor Instruments' high-resolution laser Doppler imaging unit. The images were analysed by taking regions of interest at discrete locations in the images to calculate dimensionless values of flux (PU). Repeatability of the diagnostic investigation was assessed using methods described by Bland and Altman and by also plotting the results from visit 1 against visit 2 and calculating the line of best fit. RESULTS AND CONCLUSIONS: Based on the criteria that 95% of all measurement differences should be within a factor of 1.96 of the standard deviations of the mean values, then high-resolution laser Doppler imaging technique is probably repeatable when acquiring and analysing baseline images of patients with Raynaud's phenomenon secondary to SSc. However, a larger study with more patients is required to prove this conclusively-as only data from 19 patients were analysed (3 patients were not included due to technical issues)-and was therefore susceptible to marked clinical variations in patients presenting on different days for the investigations. CI - © 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Britton, Jason AU - Britton J AUID- ORCID: 0000-0002-3145-9274 AD - Medical Physics Department, Old Medical School, Leeds General Infirmary, Leeds, UK. LA - eng GR - Acorn Committee (Registered Charity No: 1149223)/ PT - Journal Article PL - England TA - Photodermatol Photoimmunol Photomed JT - Photodermatology, photoimmunology & photomedicine JID - 9013641 SB - IM MH - Humans MH - Male MH - Female MH - Infant, Newborn MH - Infant MH - Laser-Doppler Flowmetry/methods MH - Hand/diagnostic imaging MH - *Scleroderma, Systemic/complications/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging/etiology MH - Lasers OTO - NOTNLM OT - Laser Doppler imaging OT - Raynaud's OT - repeatability OT - scleroderma EDAT- 2020/03/18 06:00 MHDA- 2023/05/11 06:42 CRDT- 2020/03/18 06:00 PHST- 2020/02/11 00:00 [revised] PHST- 2019/08/08 00:00 [received] PHST- 2020/03/13 00:00 [accepted] PHST- 2023/05/11 06:42 [medline] PHST- 2020/03/18 06:00 [pubmed] PHST- 2020/03/18 06:00 [entrez] AID - 10.1111/phpp.12549 [doi] PST - ppublish SO - Photodermatol Photoimmunol Photomed. 2023 May;39(3):193-203. doi: 10.1111/phpp.12549. PMID- 17766999 OWN - NLM STAT- MEDLINE DCOM- 20081204 LR - 20151119 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 10 DP - 2007 Oct TI - Comment on: a case of Raynaud's phenomenon in mixed connective tissue disease responding to Rituximab therapy response. PG - 1628-9 FAU - Dunkley, L AU - Dunkley L FAU - Green, M AU - Green M FAU - Gough, A AU - Gough A LA - eng PT - Case Reports PT - Comment PT - Letter DEP - 20070901 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) SB - IM CON - Rheumatology (Oxford). 2007 Apr;46(4):718-9. doi: 10.1093/rheumatology/kem003. PMID: 17289791 MH - Adult MH - Antibodies, Monoclonal/*therapeutic use MH - Antibodies, Monoclonal, Murine-Derived MH - Antirheumatic Agents/*therapeutic use MH - Female MH - Humans MH - Mixed Connective Tissue Disease/*drug therapy MH - Raynaud Disease/*drug therapy MH - Rituximab MH - Treatment Failure EDAT- 2007/09/04 09:00 MHDA- 2008/12/17 09:00 CRDT- 2007/09/04 09:00 PHST- 2007/09/04 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2007/09/04 09:00 [entrez] AID - kem208 [pii] AID - 10.1093/rheumatology/kem208 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Oct;46(10):1628-9. doi: 10.1093/rheumatology/kem208. Epub 2007 Sep 1. PMID- 33342232 OWN - NLM STAT- MEDLINE DCOM- 20240415 LR - 20240415 IS - 1555-9823 (Electronic) IS - 0003-1348 (Linking) VI - 89 IP - 4 DP - 2023 Apr TI - Acute Digital Necrosis in a Patient With Raynaud's Phenomenon and COVID-19 Infection. PG - 1129-1132 LID - 10.1177/0003134820979788 [doi] FAU - Shih, Linden AU - Shih L AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 FAU - Ferry, Andrew M AU - Ferry AM AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 AD - Division of Plastic Surgery, Texas Children's Hospital, Houston, TX, USA. FAU - Gravina, Paula R AU - Gravina PR AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 FAU - Wang, Eric D AU - Wang ED AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 FAU - Reece, Edward M AU - Reece EM AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 AD - Division of Plastic Surgery, Texas Children's Hospital, Houston, TX, USA. FAU - Maricevich, Marco AU - Maricevich M AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 FAU - Winocour, Sebastian J AU - Winocour SJ AD - Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA. RINGGOLD: 3984 LA - eng PT - Journal Article DEP - 20201219 PL - United States TA - Am Surg JT - The American surgeon JID - 0370522 SB - IM MH - Humans MH - *COVID-19/complications MH - *Raynaud Disease/complications/diagnosis MH - Patients MH - Necrosis/etiology COIS- Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2020/12/22 06:00 MHDA- 2024/04/15 06:42 CRDT- 2020/12/21 05:30 PHST- 2024/04/15 06:42 [medline] PHST- 2020/12/22 06:00 [pubmed] PHST- 2020/12/21 05:30 [entrez] AID - 10.1177/0003134820979788 [doi] PST - ppublish SO - Am Surg. 2023 Apr;89(4):1129-1132. doi: 10.1177/0003134820979788. Epub 2020 Dec 19. PMID- 19785782 OWN - NLM STAT- MEDLINE DCOM- 20091130 LR - 20151119 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 153 DP - 2009 TI - [Raynaud's phenomenon; diagnosis and treatment]. PG - B216 AB - Primary Raynaud's phenomenon (RP) is a relatively common disorder. Most patients with primary RP have only mild symptoms and do not develop complications. Distinguishing primary from secondary RP is important with respect to complications, and for prognosis and treatment. Secondary RP mainly manifests as part of systemic connective tissue disease. About 13% of patients diagnosed initially as having primary RP develop a systemic disorder within the following few years. Both auto-immune serology including antinuclear antibodies (ANA), and capillaroscopy are important diagnostic tools if one suspects the existence or development of a systemic disorder. Calcium antagonists are the cornerstone of RP pharmacotherapy. FAU - Hofstee, Herman M A AU - Hofstee HM AD - VU Medisch Centrum, Afd. Interne Geneeskunde, Amsterdam, The Netherlands. hma.hofstee@vumc.nl FAU - Voskuyl, Alexandre E AU - Voskuyl AE FAU - Serné, Erik H AU - Serné EH FAU - Smulders, Yvo M AU - Smulders YM LA - dut PT - English Abstract PT - Journal Article PT - Review TT - Het fenomeen van Raynaud: diagnostiek en behandeling. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Antibodies, Antinuclear) RN - 0 (Calcium Channel Blockers) SB - IM MH - Antibodies, Antinuclear MH - Calcium Channel Blockers/*therapeutic use MH - Humans MH - Microscopic Angioscopy/methods MH - Raynaud Disease/complications/*diagnosis/*drug therapy RF - 35 EDAT- 2009/09/30 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/30 06:00 PHST- 2009/09/30 06:00 [entrez] PHST- 2009/09/30 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2009;153:B216. PMID- 23457400 OWN - NLM STAT- MEDLINE DCOM- 20130826 LR - 20211022 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 40 IP - 3 DP - 2013 Mar TI - Raynaud's phenomenon of the tongue. PG - 336 LID - 10.3899/jrheum.121093 [doi] FAU - Cohen, Justin C AU - Cohen JC AD - Department of Otolaryngology, New York Presbyterian Hospital, Columbia University, New York, USA. FAU - Palomba, M Lia AU - Palomba ML FAU - Morris, Luc G T AU - Morris LG LA - eng PT - Case Reports PT - Journal Article PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Aged MH - Female MH - Humans MH - Raynaud Disease/*diagnosis MH - Tongue Diseases/*diagnosis EDAT- 2013/03/05 06:00 MHDA- 2013/08/27 06:00 CRDT- 2013/03/05 06:00 PHST- 2013/03/05 06:00 [entrez] PHST- 2013/03/05 06:00 [pubmed] PHST- 2013/08/27 06:00 [medline] AID - 40/3/336 [pii] AID - 10.3899/jrheum.121093 [doi] PST - ppublish SO - J Rheumatol. 2013 Mar;40(3):336. doi: 10.3899/jrheum.121093. PMID- 31641890 OWN - NLM STAT- MEDLINE DCOM- 20191120 LR - 20210110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 12 DP - 2019 Dec TI - Raynaud's phenomenon and blood rheology: comments on the article "Raynaud's phenomenon-an update on diagnosis, classification and management". PG - 3689-3690 LID - 10.1007/s10067-019-04785-x [doi] FAU - Harris, Edward S AU - Harris ES AUID- ORCID: 0000-0002-7492-5589 AD - Department of Medicine (Rheumatology), University of Wisconsin, Madison, WI, USA. eharris5@wisc.edu. LA - eng PT - Comment PT - Letter DEP - 20191022 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM CON - Clin Rheumatol. 2019 Dec;38(12):3317-3330. doi: 10.1007/s10067-019-04745-5. PMID: 31420815 MH - Humans MH - *Raynaud Disease MH - Rheology EDAT- 2019/10/24 06:00 MHDA- 2019/11/21 06:00 CRDT- 2019/10/24 06:00 PHST- 2019/09/10 00:00 [received] PHST- 2019/09/16 00:00 [accepted] PHST- 2019/09/10 00:00 [revised] PHST- 2019/10/24 06:00 [pubmed] PHST- 2019/11/21 06:00 [medline] PHST- 2019/10/24 06:00 [entrez] AID - 10.1007/s10067-019-04785-x [pii] AID - 10.1007/s10067-019-04785-x [doi] PST - ppublish SO - Clin Rheumatol. 2019 Dec;38(12):3689-3690. doi: 10.1007/s10067-019-04785-x. Epub 2019 Oct 22. PMID- 21942753 OWN - NLM STAT- MEDLINE DCOM- 20121107 LR - 20120620 IS - 1369-1635 (Electronic) IS - 0953-7104 (Linking) VI - 23 IP - 4 DP - 2012 TI - Increased mean platelet volume in primary Raynaud's phenomenon. PG - 312-6 LID - 10.3109/09537104.2011.618563 [doi] AB - We hypothesized that mean platelet volume (MPV), a reliable marker of platelet activation, might be elevated in primary Raynaud's phenomenon (PRP) even if there was no thrombotic complication in our subjects. In this retrospective-cohort study, we examined the clinical value of MPV in 200 patients with PRP and 116 clinical controls, and measured MPV and platelet P-selectin (CD62P) in all study participants. We also evaluated the effect of age, gender, and disease duration on these platelet activation markers in PRP. MPV and CD62 positivities were significantly (p<0.001) elevated in patients with PRP compared with controls. These differences retained when patients and controls were analyzed according to age, gender, and the disease duration. In logistic regression analysis, MPV (OR: 15.8, 95% CI: 8.14-30.64, p<0.001) and CD62P (OR: 11.3, 95% CI: 4.85-26.12, p<0.001) were found to be independently associated with PRP. In conclusion, increased MPV is independently related to PRP, and its level was not influenced by age, gender, and the duration of PRP. FAU - Shemirani, Amir-Houshang AU - Shemirani AH AD - Clinical Research Center, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. shemirani1@gmail.com FAU - Nagy, Béla Jr AU - Nagy B Jr FAU - Takáts, Alajos-Tamas AU - Takáts AT FAU - Zsóri, Katalin-Szilvia AU - Zsóri KS FAU - András, Csilla AU - András C FAU - Kappelmayer, János AU - Kappelmayer J FAU - Csiki, Zoltán AU - Csiki Z LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110926 PL - England TA - Platelets JT - Platelets JID - 9208117 RN - 0 (P-Selectin) SB - IM MH - Adult MH - Blood Platelets/metabolism/*pathology MH - Case-Control Studies MH - Cell Size MH - Female MH - Humans MH - Male MH - Middle Aged MH - P-Selectin/metabolism MH - Raynaud Disease/*diagnosis/pathology MH - Young Adult EDAT- 2011/09/29 06:00 MHDA- 2012/11/08 06:00 CRDT- 2011/09/28 06:00 PHST- 2011/09/28 06:00 [entrez] PHST- 2011/09/29 06:00 [pubmed] PHST- 2012/11/08 06:00 [medline] AID - 10.3109/09537104.2011.618563 [doi] PST - ppublish SO - Platelets. 2012;23(4):312-6. doi: 10.3109/09537104.2011.618563. Epub 2011 Sep 26. PMID- 19268093 OWN - NLM STAT- MEDLINE DCOM- 20090720 LR - 20190917 IS - 0014-2565 (Print) IS - 0014-2565 (Linking) VI - 209 IP - 1 DP - 2009 Jan TI - [Treatment of Raynaud's phenomenon]. PG - 21-4 AB - Raynaud's phenomenon (RP) is a clinical picture characterized by the presence of recurrent episodes of vasospasm that is precipitated by cold or other stimuli and especially affects the fingers and/or toes. It may be primary or idiopathic or secondary and be due to many causes, among which connective diseases, and specifically scleroderma, stand out. Primary RP does not generally require drug treatment, only requiring general measures. Calcium channel antagonists continue to be the drug of first choice. However, when RP is severe and is accompanied by digital ulcers/tissue necrosis, the therapeutic regime must be individualized and combinations should be established with difference drugs such as prostanoids, bosentan, sildenafil, antiaggregants/anticoagulants, antibiotics and analgesics. FAU - Fonollosa-Pla, V AU - Fonollosa-Pla V AD - Servicio de Medicina Interna (Enfermedades Autoinmunes Sistémicas), Hospital General Universitario Vall d'Hebron, Departamento de Medicina, Universidad Autónoma de Barcelona, Barcelona, España. vfonollosa@vhebron.net FAU - Pilar Simeón-Aznar, C AU - Pilar Simeón-Aznar C FAU - Vilardell-Tarrés, M AU - Vilardell-Tarrés M LA - spa PT - English Abstract PT - Journal Article TT - Tratamiento del fenómeno de Raynaud. PL - Spain TA - Rev Clin Esp JT - Revista clinica espanola JID - 8608576 SB - IM MH - Algorithms MH - Humans MH - Raynaud Disease/*therapy EDAT- 2009/03/10 09:00 MHDA- 2009/07/21 09:00 CRDT- 2009/03/10 09:00 PHST- 2009/03/10 09:00 [entrez] PHST- 2009/03/10 09:00 [pubmed] PHST- 2009/07/21 09:00 [medline] AID - S0014-2565(09)70354-6 [pii] AID - 10.1016/s0014-2565(09)70354-6 [doi] PST - ppublish SO - Rev Clin Esp. 2009 Jan;209(1):21-4. doi: 10.1016/s0014-2565(09)70354-6. PMID- 28777521 OWN - NLM STAT- MEDLINE DCOM- 20171205 LR - 20171205 IS - 1066-2936 (Print) IS - 1066-2936 (Linking) VI - 43 IP - 7 DP - 2016 Nov-Dec TI - Blue-fingered diver: case report. PG - 835-840 AB - Although Raynaud's phenomenon is a well-known consequence of exposure to cold, neither its incidence in recreational divers nor case reports in that population have been reported in the medical literature. We present a case report of the initial manifestation of primary Raynaud's phenomenon during a warm-water scuba dive. A healthy 18-year-old Caucasian male made four open-circuit compressed air scuba dives over two days in the Florida Keys to a maximum depth of 90 feet. After two of those dives, he noted painless, blue discoloration on three digits of his left hand, unaccompanied by sensory changes, which resolved within an hour of surfacing. During a fitness-to-dive evaluation one week later, his physical examination was normal. No skin discoloration, neurological symptoms or changes to pulse or blood pressure were noted with temperature or positional provocation. Laboratory testing was normal. However, arterial Doppler measurements were severely diminished in all digits of both upper extremities with temperature provocation, and continued to be diminished five minutes after immersion, suggesting Raynaud's phenomenon. CI - Copyright© Undersea and Hyperbaric Medical Society. FAU - Cable, Rebecca AU - Cable R AD - Division of Hyperbaric Medicine, Intermountain Medical Center, Murray, Utah and Intermountain LDS Hospital, Salt Lake City, Utah U.S. FAU - Weaver, Lindell K AU - Weaver LK AD - Division of Hyperbaric Medicine, Intermountain Medical Center, Murray, Utah and Intermountain LDS Hospital, Salt Lake City, Utah U.S. AD - Department of Anesthesiology, Center for Hyperbaric Medicine and Environmental Physiology, Duke University Medical Center, Durham, North Carolina U.S. AD - University of Utah School of Medicine, Salt Lake City, Utah U.S. FAU - Roberts, Anne B AU - Roberts AB AD - Department of Anesthesiology, Center for Hyperbaric Medicine and Environmental Physiology, Duke University Medical Center, Durham, North Carolina U.S. AD - Hyperbaric Medicine Department Head, Naval Aerospace Medicine Institute, NAS Pensacola, Florida U.S. FAU - Goodman, Greg AU - Goodman G AD - Department of Vascular Surgery, Intermountain Medical Center and Mountain Medical Vascular, Murray, Utah U.S. FAU - Deru, Kayla AU - Deru K AD - Division of Hyperbaric Medicine, Intermountain Medical Center, Murray, Utah and Intermountain LDS Hospital, Salt Lake City, Utah U.S. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Undersea Hyperb Med JT - Undersea & hyperbaric medicine : journal of the Undersea and Hyperbaric Medical Society, Inc JID - 9312954 SB - IM MH - Adolescent MH - Cold Temperature MH - Diving/*adverse effects MH - *Fingers/blood supply MH - Humans MH - Male MH - Photoplethysmography MH - Raynaud Disease/diagnosis/*etiology OTO - NOTNLM OT - Raynaud's phenomenon OT - blue fingers OT - recreational diving OT - thoracic outlet syndrome COIS- The authors of this paper declare no conflicts of interest exist with this submission. EDAT- 2017/08/05 06:00 MHDA- 2017/12/06 06:00 CRDT- 2017/08/05 06:00 PHST- 2017/08/05 06:00 [entrez] PHST- 2017/08/05 06:00 [pubmed] PHST- 2017/12/06 06:00 [medline] PST - ppublish SO - Undersea Hyperb Med. 2016 Nov-Dec;43(7):835-840. PMID- 21253616 OWN - NLM STAT- MEDLINE DCOM- 20110406 LR - 20191112 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 62 IP - 4 DP - 2010 Oct-Dec TI - [The role of nailfold videocapillaroscopy in Raynaud's phenomenon monitoring and early diagnosis of systemic sclerosis]. PG - 237-47 AB - Several connective tissue diseases, in particular systemic sclerosis (SSc), have Raynaud's phenomenon (RP) as their first clinical manifestation. Primary RP represents a benign condition often observed in otherwise healthy subjects, especially women: it is due to an exaggerated response to the physiological cold-induced vasospasm, whereas the secondary form of RP is typically associated with connective tissue diseases, especially SSc. Nailfold videocapillaroscopy (NVC), particulary after the recent technological advances, is a safe and reliable method to observe the microvascular structure and its early changes, especially during the transition from primary to secondary RP. In case of SSc, by considering validated patterns and scoring systems, NVC is the main tool that rheumatologists can rely on, besides the presence of specific auto-antibodies, to perform a very early diagnosis of the disease. This implies the possibility of early treatment of SSc, with an eye of predicting and preventing its major clinical complications. FAU - Cutolo, M AU - Cutolo M AD - Dipartimento di Medicina Interna e Specialità Mediche, Università degli Studi di Genova, Genova, Italia. mcutolo@unige.it FAU - Pizzorni, C AU - Pizzorni C FAU - Meroni, M AU - Meroni M FAU - Zampogna, G AU - Zampogna G FAU - Ferrone, C AU - Ferrone C FAU - Alessandri, E AU - Alessandri E FAU - Sulli, A AU - Sulli A LA - ita PT - English Abstract PT - Journal Article PT - Review TT - La videocapillaroscopia ungueale nel monitoraggio del fenomeno di Raynaud e nella diagnosi precoce della sclerosi sistemica. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Early Diagnosis MH - Humans MH - *Microscopic Angioscopy MH - Raynaud Disease/*pathology MH - Scleroderma, Systemic/*pathology MH - Video Recording EDAT- 2011/01/22 06:00 MHDA- 2011/04/07 06:00 CRDT- 2011/01/22 06:00 PHST- 2011/01/22 06:00 [entrez] PHST- 2011/01/22 06:00 [pubmed] PHST- 2011/04/07 06:00 [medline] AID - 10.4081/reumatismo.2010.237 [doi] PST - ppublish SO - Reumatismo. 2010 Oct-Dec;62(4):237-47. doi: 10.4081/reumatismo.2010.237. PMID- 37796654 OWN - NLM STAT- MEDLINE DCOM- 20240220 LR - 20240224 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 74 IP - 1 DP - 2024 Feb 19 TI - COVID-19 associated with Raynaud's phenomenon in a vibration-exposed worker. PG - 128-130 LID - 10.1093/occmed/kqad103 [doi] AB - This is the first known case report of COVID-19-related Raynaud's phenomenon (RP) identified at routine health surveillance of a male exposed to hand-transmitted vibration. The temporal relationship with COVID infection followed the course previously reported, being a late feature associated with mild COVID, and followed by gradual spontaneous recovery. It is not known whether the RP, in this case, is solely due to COVID or represents a summative effect of COVID and vibration exposure. A prudent approach is recommended in such circumstances with limitation of vibration exposure and continuing frequent surveillance pending improvement of the RP, with further prompt investigation if the expected recovery does not occur. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Cooke, R AU - Cooke R AD - Cooke Medical Services, Sheffield, S11 8AE, UK. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Humans MH - Male MH - Vibration/adverse effects MH - *COVID-19/complications MH - Hand MH - *Raynaud Disease/complications EDAT- 2023/10/05 18:42 MHDA- 2024/02/20 11:50 CRDT- 2023/10/05 12:33 PHST- 2024/02/20 11:50 [medline] PHST- 2023/10/05 18:42 [pubmed] PHST- 2023/10/05 12:33 [entrez] AID - 7290986 [pii] AID - 10.1093/occmed/kqad103 [doi] PST - ppublish SO - Occup Med (Lond). 2024 Feb 19;74(1):128-130. doi: 10.1093/occmed/kqad103. PMID- 39152754 OWN - NLM STAT- MEDLINE DCOM- 20240817 LR - 20240817 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 8 DP - 2024 Aug TI - Comparison of serum exosome miRNA from patients with Raynaud's phenomenon with positive and negative serum antinuclear antibodies. PG - 1629-1634 LID - 10.55563/clinexprheumatol/4iiab4 [doi] AB - OBJECTIVES: To compare the microRNAs (miRNAs) contained within serum exosomes isolated from patients with Raynaud's phenomenon (RP) and negative antinuclear antibodies (ANA) to the miRNA contained in serum exosomes isolated from patients with RP and positive ANA. METHODS: Serum exosomes were isolated employing a polymer precipitation procedure. Next Generation Sequencing (NGS) was used to identify the miRNAs contained in the exosomes isolated from the two clinical cohorts and to analyse the differences in their contents. RESULTS: The NGS results identified six miRNAs that displayed significant differences in their content between serum exosomes from patients with RP with negative serum ANA compared to miRNAs contained in serum exosomes from patients with ANA-positive RP. CONCLUSIONS: A comparative analysis of miRNAs contained within serum exosomes of patients with RP and negative ANA vs. samples from patients with RP and positive ANA identified several differentially expressed miRNAs that may represent non-invasive biomarkers to assist in the identification of patients with RP at risk of evolving into systemic sclerosis. FAU - Piera-Velazquez, Sonsoles AU - Piera-Velazquez S AD - Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Fortina, Paolo AU - Fortina P AD - Cancer Genomics and Bioinformatics Laboratory, Department of Pharmacology, Physiology, and Cancer Biology, Sidney Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Jimenez, Sergio A AU - Jimenez SA AD - Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA. sergio.jimenez@jefferson.edu. LA - eng PT - Comparative Study PT - Journal Article DEP - 20240801 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) RN - 0 (MicroRNAs) RN - 0 (Biomarkers) RN - 0 (Circulating MicroRNA) SB - IM MH - Humans MH - *Raynaud Disease/blood/genetics/immunology/diagnosis MH - *Antibodies, Antinuclear/blood MH - Female MH - *Exosomes/genetics MH - Middle Aged MH - *MicroRNAs/blood/genetics MH - Male MH - Adult MH - Biomarkers/blood MH - High-Throughput Nucleotide Sequencing MH - Circulating MicroRNA/blood/genetics MH - Aged MH - Predictive Value of Tests EDAT- 2024/08/17 15:42 MHDA- 2024/08/17 15:43 CRDT- 2024/08/17 05:33 PHST- 2024/03/22 00:00 [received] PHST- 2024/06/03 00:00 [accepted] PHST- 2024/08/17 15:43 [medline] PHST- 2024/08/17 15:42 [pubmed] PHST- 2024/08/17 05:33 [entrez] AID - 21091 [pii] AID - 10.55563/clinexprheumatol/4iiab4 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Aug;42(8):1629-1634. doi: 10.55563/clinexprheumatol/4iiab4. Epub 2024 Aug 1. PMID- 27136918 OWN - NLM STAT- MEDLINE DCOM- 20170629 LR - 20181113 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 36 IP - 10 DP - 2016 Oct TI - Symptoms of Raynaud's phenomenon (RP) in fibromyalgia syndrome are similar to those reported in primary RP despite differences in objective assessment of digital microvascular function and morphology. PG - 1371-7 LID - 10.1007/s00296-016-3483-6 [doi] AB - Symptoms of Raynaud's phenomenon (RP) are common in fibromyalgia syndrome (FMS). We compared symptom characteristics and objective assessment of digital microvascular function using infrared thermography (and nailfold capillaroscopy where available) in patients with FMS (reporting RP symptoms) and primary RP. We retrospectively reviewed the outcome of microvascular imaging studies and RP symptom characteristics (captured using patient-completed questionnaire at the time of assessment) for patients with FMS (reporting RP symptoms) and patients with primary RP referred for thermographic assessment of RP symptoms over a 2-year period. Of 257 patients referred for thermographic assessment of RP symptoms between 2010 and 2012, we identified 85 patients with primary RP and 43 patients with FMS. There were no differences in RP symptom characteristics between FMS and primary RP (p > 0.05 for all comparisons). In contrast, patients with FMS had higher baseline temperature of the digits (32.1 vs. 29.0 °C, p = 0.004), dorsum (31.9 vs. 30.2 °C, p = 0.005) and thermal gradient (temperature of digits minus temperature of dorsum; +0.0 vs. -0.9 °C, p = 0.03) compared with primary RP. Significant differences between groups persisted following local cold challenge. In primary RP, patient reporting "blue" digits, bi-phasic and tri-phasic RP was associated with lower digital perfusion. In contrast, no associations between skin temperature and RP digital colour changes/phases were identified in FMS. Our findings suggest that symptoms of RP in FMS may have a different aetiology to those seen in primary RP. These findings have potential implications for both the classification of RP symptoms and the management of RP symptoms in the context of FMS. Digital colour changes reported by patients might reflect the degree of digital microvascular compromise in primary RP. FAU - Scolnik, M AU - Scolnik M AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. AD - Sección Reumatología, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. FAU - Vasta, B AU - Vasta B AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. FAU - Hart, D J AU - Hart DJ AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. FAU - Shipley, J A AU - Shipley JA AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. FAU - McHugh, N J AU - McHugh NJ AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Pauling, J D AU - Pauling JD AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath, BA1 1RL, UK. johnpauling@nhs.net. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. johnpauling@nhs.net. LA - eng PT - Journal Article DEP - 20160502 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Female MH - Fibromyalgia/*complications/physiopathology MH - Fingers/blood supply MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/complications/*diagnosis/physiopathology MH - Retrospective Studies MH - Symptom Assessment/methods PMC - PMC5020104 OTO - NOTNLM OT - Classification OT - Fibromyalgia syndrome OT - Objective OT - Raynaud’s phenomenon OT - Self-report OT - Thermography COIS- The authors declare that they have no conflict of interest relevant to the content of this work. Ethical approval This article is a retrospective analysis and does not contain any prospective studies with human participants performed by any of the authors. We received written confirmation from the National Research Ethics Service confirming that Research Ethics Committee approval was not required for a retrospective review of data obtained under normal clinical practice [under UK-wide Governance Arrangements for Research Ethics Committees (GAfREC)]. For this type of study, formal consent was not required. EDAT- 2016/05/04 06:00 MHDA- 2017/07/01 06:00 PMCR- 2016/05/02 CRDT- 2016/05/04 06:00 PHST- 2015/10/14 00:00 [received] PHST- 2016/04/16 00:00 [accepted] PHST- 2016/05/04 06:00 [entrez] PHST- 2016/05/04 06:00 [pubmed] PHST- 2017/07/01 06:00 [medline] PHST- 2016/05/02 00:00 [pmc-release] AID - 10.1007/s00296-016-3483-6 [pii] AID - 3483 [pii] AID - 10.1007/s00296-016-3483-6 [doi] PST - ppublish SO - Rheumatol Int. 2016 Oct;36(10):1371-7. doi: 10.1007/s00296-016-3483-6. Epub 2016 May 2. PMID- 34984505 OWN - NLM STAT- MEDLINE DCOM- 20220408 LR - 20220408 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 4 DP - 2022 Apr TI - Comment on: Can Raynaud's phenomenon be defined as a subtype of primary Sjögren's syndrome? Comments on "Clinical features and risk factors of Raynaud's phenomenon in primary Sjögren's syndrome" Reply. PG - 1267-1268 LID - 10.1007/s10067-021-06043-5 [doi] FAU - Wei, Lin AU - Wei L AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Liu, Yang AU - Liu Y AD - Department of Graduate School, Hebei Medical University, Shijiazhuang, 050051, China. FAU - Fengxiao, Zhang AU - Fengxiao Z AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. zfx8889@163.com. LA - eng PT - Comment PT - Letter DEP - 20220104 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM CON - Clin Rheumatol. 2021 Oct;40(10):4081-4087. doi: 10.1007/s10067-021-05749-w. PMID: 33914202 CON - Clin Rheumatol. 2022 Apr;41(4):1265-1266. doi: 10.1007/s10067-021-06023-9. PMID: 35059878 MH - Humans MH - *Raynaud Disease/complications MH - Risk Factors MH - *Sjogren's Syndrome/complications EDAT- 2022/01/06 06:00 MHDA- 2022/04/09 06:00 CRDT- 2022/01/05 06:02 PHST- 2021/12/22 00:00 [received] PHST- 2021/12/23 00:00 [accepted] PHST- 2021/12/22 00:00 [revised] PHST- 2022/01/06 06:00 [pubmed] PHST- 2022/04/09 06:00 [medline] PHST- 2022/01/05 06:02 [entrez] AID - 10.1007/s10067-021-06043-5 [pii] AID - 10.1007/s10067-021-06043-5 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Apr;41(4):1267-1268. doi: 10.1007/s10067-021-06043-5. Epub 2022 Jan 4. PMID- 21253740 OWN - NLM STAT- MEDLINE DCOM- 20140113 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 6 DP - 2013 Jun TI - Capillaroscopic pattern in paraneoplastic Raynaud's phenomenon. PG - 1597-9 LID - 10.1007/s00296-010-1715-8 [doi] AB - The capillaroscopic pattern in paraneoplastic Raynaud's phenomenon (RP) has not been investigated systematically and is not well-defined. Here, we present three case reports of patients with paraneoplastic rheumatic conditions, manifested with severe secondary RP with emphasis upon capillaroscopic findings. The first patient is a 58-year-old male with known psoriasis and psoriatic arthritis, severe RP and scleroderma-like syndrome, resulting in a paraneoplastic syndrome in the context of a lung cancer. At capillaroscopic examination classic "scleroderma" pattern, an "early" type was found. The second patient is a 48-year-old woman with an abrupt onset of paraneoplastic dermatomyositis, severe RP, and a lung cancer. The capillaroscopic examination revealed frequent dilated and giant capillaries, hemorrhages and severe microvascular disarrangement-the so-called "scleroderma-like" pattern typical of the idiopathic forms of the disease. The third patient is a 56-year-old woman with paraneoplastic dermatomyositis, secondary RP, and thyroid cancer. The capillaroscopic examination showed dilated and giant capillaries, elongated capillaries, decreased mean capillary density with avascular areas, severe disarrangement, single hemorrhages, and clear evidence of neoangiogenesis. These capillaroscopic features characteristic of the "scleroderma-like" pattern are indistinguishable from those in idiopathic dermatomyositis like in the second case. Taken together, the cases illustrate the problem that capillaroscopic patterns in paraneoplastic RP in the context of scleroderma-like syndrome and dermatomyositis appear to be indistinguishable from the microvascular changes in the respective idiopathic rheumatic diseases. FAU - Lambova, S AU - Lambova S AD - Department for Propaedeutics of Internal Medicine, Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria. sevdalina_n@abv.bg FAU - Müller-Ladner, U AU - Müller-Ladner U LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110121 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Paraneoplastic Syndromes/*pathology/physiopathology MH - Raynaud Disease/*pathology/physiopathology EDAT- 2011/01/22 06:00 MHDA- 2014/01/15 06:00 CRDT- 2011/01/22 06:00 PHST- 2010/05/16 00:00 [received] PHST- 2010/12/30 00:00 [accepted] PHST- 2011/01/22 06:00 [entrez] PHST- 2011/01/22 06:00 [pubmed] PHST- 2014/01/15 06:00 [medline] AID - 10.1007/s00296-010-1715-8 [doi] PST - ppublish SO - Rheumatol Int. 2013 Jun;33(6):1597-9. doi: 10.1007/s00296-010-1715-8. Epub 2011 Jan 21. PMID- 16978744 OWN - NLM STAT- MEDLINE DCOM- 20070116 LR - 20201209 IS - 0248-8663 (Print) IS - 0248-8663 (Linking) VI - 27 IP - 10 DP - 2006 Oct TI - [Helicobacter pylori prevalence in Raynaud's disease]. PG - 736-41 AB - PURPOSE: Recent studies have suggested that the prevalence of Helicobacter pylori may be more frequent in patients with primary Raynaud's phenomenon (PRP) compared to healthy subjects. These data prompted us to conduct this prospective study, in order to assess the prevalence of H. pylori infection in a large series of patients with PRP. METHODS: Forty consecutive patients with a definite diagnosis of PRP were included in the study. The findings in the PRP patients were compared with those of 80 age- and sex-matched healthy subjects. H. pylori infection was diagnosed using serology and urease breath test. RESULTS: The prevalence of H. pylori infection was as high as 12.5% in PRP patients using both serology and urease breath test, whereas it was found to be 16.7% and 18%, respectively, in healthy controls. CONCLUSION: As prevalence of H. pylori infection was similar in PRP patients compared to controls (P=0.53 and 0.43, respectively), our data underscore that H. pylori infection may not play a role in the genesis of PRP-related vascular complication onset. Interestingly, PRP patients exhibited more commonly digestive symptoms consistent with H. pylori infection compared to controls (P<0.05). FAU - Hervé, F AU - Hervé F AD - Département de médecine interne, CHU de Rouen-Boisguillaume, 76031 Rouen cedex, France. FAU - Cailleux, N AU - Cailleux N FAU - Benhamou, Y AU - Benhamou Y FAU - Ducrotté, P AU - Ducrotté P FAU - Lemeland, J-F AU - Lemeland JF FAU - Denis, P AU - Denis P FAU - Marie, I AU - Marie I FAU - Lévesque, H AU - Lévesque H LA - fre PT - Journal Article TT - Prévalence des infections à Helicobacter pylori au cours de la maladie de Raynaud. DEP - 20060721 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - France/epidemiology MH - Gastritis/complications/microbiology MH - Helicobacter Infections/*complications/diagnosis/epidemiology MH - Helicobacter pylori/*isolation & purification MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Prospective Studies MH - Raynaud Disease/diagnosis/epidemiology/*microbiology MH - Surveys and Questionnaires EDAT- 2006/09/19 09:00 MHDA- 2007/01/17 09:00 CRDT- 2006/09/19 09:00 PHST- 2006/05/24 00:00 [received] PHST- 2006/07/04 00:00 [accepted] PHST- 2006/09/19 09:00 [pubmed] PHST- 2007/01/17 09:00 [medline] PHST- 2006/09/19 09:00 [entrez] AID - S0248-8663(06)00274-8 [pii] AID - 10.1016/j.revmed.2006.07.003 [doi] PST - ppublish SO - Rev Med Interne. 2006 Oct;27(10):736-41. doi: 10.1016/j.revmed.2006.07.003. Epub 2006 Jul 21. PMID- 18850101 OWN - NLM STAT- MEDLINE DCOM- 20090819 LR - 20181113 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 6 DP - 2009 Apr TI - A case of MCTD overlapped by Takayasu's arteritis, presenting Raynaud's phenomenon as the initial manifestation of both diseases. PG - 685-8 LID - 10.1007/s00296-008-0717-2 [doi] AB - Raynaud's phenomenon is characteristic three-phase color change of digits that occurs when hands are exposed to cold and subsequently rewarmed. Raynaud's phenomenon has many possible causes, but evaluation tends to focus on a few notorious etiologies, such as, connective tissue diseases. Thus, having reached a diagnosis, detailed physical exam to rule out other possible causes is often not performed. The authors present a case of mixed connective tissue disease (MCTD) and Takayasu's arteritis overlap in a woman, who showed Raynaud's phenomenon as an initial manifestation. She was first diagnosed as having MCTD, but her treatment did not improve the persistent Raynaud's phenomenon. Several years later, follow-up chest CT showed underlying Takayasu's arteritis and a subsequent physical examination revealed that typical abnormalities consistent with Takayasu's arteritis were present. The authors advocate thorough history taking and complete physical examinations on a routine basis to help unearth other underlying causes. FAU - Lim, Mie Jin AU - Lim MJ AD - Inha University Hospital, Incheon, South Korea. FAU - Kwon, Seong Ryul AU - Kwon SR FAU - Kim, Sang Gu AU - Kim SG FAU - Park, Won AU - Park W LA - eng PT - Case Reports PT - Journal Article DEP - 20081011 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Autoantibodies MH - Diagnosis, Differential MH - Diagnostic Errors/*adverse effects MH - Female MH - Follow-Up Studies MH - Humans MH - Mixed Connective Tissue Disease/*diagnosis MH - Raynaud Disease/*diagnosis/etiology MH - Takayasu Arteritis/*complications MH - Time Factors EDAT- 2008/10/14 09:00 MHDA- 2009/08/20 09:00 CRDT- 2008/10/14 09:00 PHST- 2008/07/30 00:00 [received] PHST- 2008/09/07 00:00 [accepted] PHST- 2008/10/14 09:00 [pubmed] PHST- 2009/08/20 09:00 [medline] PHST- 2008/10/14 09:00 [entrez] AID - 10.1007/s00296-008-0717-2 [doi] PST - ppublish SO - Rheumatol Int. 2009 Apr;29(6):685-8. doi: 10.1007/s00296-008-0717-2. Epub 2008 Oct 11. PMID- 22516031 OWN - NLM STAT- MEDLINE DCOM- 20120921 LR - 20120611 IS - 1759-9873 (Electronic) IS - 0964-5284 (Linking) VI - 30 IP - 2 DP - 2012 Jun TI - Raynaud's phenomenon, cytokines and acupuncture: a case report. PG - 139-41 LID - 10.1136/acupmed-2011-010107 [doi] AB - A 30-year-old African-American woman diagnosed in 2006 with primary Raynaud's phenomenon (RP) was seen in the clinic in 2010 and the diagnosis confirmed excluding underlying disorders. Acupuncture was administered bilaterally at the LI4 Hegu acupuncture points for 5 min twice weekly for 2 months, which resulted in improvement in pain severity, joint stiffness and the colour of her fingers and toes. The literature reveals that acupuncture is effective in improving pain severity and joint stiffness in RP. The patient's serum proinflammatory cytokines were compared with those from an ongoing study in our institution and the results indicated that acupuncture therapy might be anti-inflammatory. Acupuncture is relatively safe and should be considered as an alternative treatment or non-pharmacological therapy for pain associated with RP. FAU - Omole, Folashade S AU - Omole FS AD - Department of Family Medicine, Morehouse School of Medicine, East Point, GA 30344, USA. fomole@msm.edu FAU - Lin, James S AU - Lin JS FAU - Chu, Tehching AU - Chu T FAU - Sow, Charles M AU - Sow CM FAU - Flood, Anthony AU - Flood A FAU - Powell, Michael David AU - Powell MD LA - eng PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20120419 PL - England TA - Acupunct Med JT - Acupuncture in medicine : journal of the British Medical Acupuncture Society JID - 9304117 RN - 0 (Cytokines) SB - IM MH - *Acupuncture Therapy MH - Adult MH - Cytokines/*immunology MH - Female MH - Humans MH - Raynaud Disease/diagnosis/immunology/*therapy EDAT- 2012/04/21 06:00 MHDA- 2012/09/22 06:00 CRDT- 2012/04/21 06:00 PHST- 2012/04/21 06:00 [entrez] PHST- 2012/04/21 06:00 [pubmed] PHST- 2012/09/22 06:00 [medline] AID - acupmed-2011-010107 [pii] AID - 10.1136/acupmed-2011-010107 [doi] PST - ppublish SO - Acupunct Med. 2012 Jun;30(2):139-41. doi: 10.1136/acupmed-2011-010107. Epub 2012 Apr 19. PMID- 24660212 OWN - NLM STAT- MEDLINE DCOM- 20150904 LR - 20151119 IS - 2327-2228 (Electronic) VI - 97 IP - 4 DP - 2014 Apr 1 TI - Post-traumatic Raynaud's phenomenon following volar plate injury. PG - 24-6 AB - Post-traumatic Raynaud's phenomenon following non-penetrating or non-repetitive injury is rare. We report a case of Raynaud's phenomenon occurring in a single digit 3 months following volar plate avulsion injury. Daily episodes of painless pallor of the digit occurred for 1 month upon any exposure to cold, resolving with warm water therapy. Symptoms resolved after the initiation of hand therapy, splinting, and range-of- motion exercises. FAU - Chodakiewitz, Yosef G AU - Chodakiewitz YG AD - The Warren Alpert Medical School, Brown University, Providence, RI. FAU - Daniels, Alan H AU - Daniels AH AD - Department of Orthopaedics, The Warren Alpert Medical School, Brown University, Providence, RI. FAU - Kamal, Robin N AU - Kamal RN AD - Department of Orthopaedics, The Warren Alpert Medical School, Brown University, Providence, RI. FAU - Weiss, Arnold-Peter C AU - Weiss AP AD - Department of Orthopaedics, The Warren Alpert Medical School, Brown University, Providence, RI. LA - eng PT - Case Reports PT - Journal Article DEP - 20140401 PL - United States TA - R I Med J (2013) JT - Rhode Island medical journal (2013) JID - 101605827 SB - IM MH - Adult MH - Humans MH - Male MH - Palmar Plate/*injuries MH - Raynaud Disease/*etiology OTO - NOTNLM OT - Raynaud’s phenomenon OT - Raynaud’s syndrome OT - Volar plate injury EDAT- 2014/03/25 06:00 MHDA- 2015/09/05 06:00 CRDT- 2014/03/25 06:00 PHST- 2014/03/25 06:00 [entrez] PHST- 2014/03/25 06:00 [pubmed] PHST- 2015/09/05 06:00 [medline] PST - epublish SO - R I Med J (2013). 2014 Apr 1;97(4):24-6. PMID- 32447423 OWN - NLM STAT- MEDLINE DCOM- 20211115 LR - 20211115 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 41 IP - 5 DP - 2021 May TI - Targeted high concentration botulinum toxin A injections in patients with Raynaud's phenomenon: a retrospective single-centre experience. PG - 943-949 LID - 10.1007/s00296-020-04606-4 [doi] AB - Raynaud's phenomenon is a vasospastic condition affecting hands and feet which may lead to rest pain, ischemic ulcers and gangrene. Botulinum toxin A has been shown to improve peripheral circulation and relieve vasospastic symptoms. Our aim was to assess our treatment outcomes following Botulinum toxin A injections in patients with Raynaud's phenomenon and to explore the importance of toxin concentration and injection sites. Retrospective chart review of patients with primary and secondary Raynaud's syndrome treated with Botulinum toxin A injections and a literature review was conducted. The toxin dose, injection sites, symptom relief, healing of ulcers and complications were assessed. A total of 30 treatment episodes over a 7½ year period were included. All patients had failed medical management. Botulinum toxin A injection was injected primarily in the vicinity of the palmar digital neurovascular bundle. The average total Botulinum toxin A dose injected was 156 U and the concentration was 50 U/ml. All patients reported an improvement in symptoms and healing of digital ulcers. One patient reported a temporary muscle weakness. Six patients had a single treatment episode with long term benefit. Systemic sclerosis patients had an average of 6-month interval between treatment episodes. Higher doses of Botulinum toxin A has been well tolerated with no long term adverse effects. Our study shows that targeted low volume higher concentration Botulinum toxin A injections are effective in treating Raynaud's phenomenon. FAU - Nagarajan, Mahalakshmi AU - Nagarajan M AUID- ORCID: 0000-0003-3445-0627 AD - Department of Plastic and Reconstructive Surgery, Saint Helens and Knowsley NHS Trust, Whiston Hospital, Warrington Road, Prescot, L35 5DR, United Kingdom. Maha.nagarajan@sthk.nhs.uk. FAU - McArthur, Paul AU - McArthur P AD - Department of Plastic and Reconstructive Surgery, Saint Helens and Knowsley NHS Trust, Whiston Hospital, Warrington Road, Prescot, L35 5DR, United Kingdom. LA - eng PT - Journal Article PT - Observational Study DEP - 20200523 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*administration & dosage/pharmacology MH - Female MH - Humans MH - Injections/methods MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*drug therapy MH - Retrospective Studies MH - Scleroderma, Systemic/complications OTO - NOTNLM OT - Botulinum toxin OT - Digital ischemic ulcers OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2020/05/25 06:00 MHDA- 2021/11/16 06:00 CRDT- 2020/05/25 06:00 PHST- 2020/04/09 00:00 [received] PHST- 2020/05/12 00:00 [accepted] PHST- 2020/05/25 06:00 [pubmed] PHST- 2021/11/16 06:00 [medline] PHST- 2020/05/25 06:00 [entrez] AID - 10.1007/s00296-020-04606-4 [pii] AID - 10.1007/s00296-020-04606-4 [doi] PST - ppublish SO - Rheumatol Int. 2021 May;41(5):943-949. doi: 10.1007/s00296-020-04606-4. Epub 2020 May 23. PMID- 18682952 OWN - NLM STAT- MEDLINE DCOM- 20090424 LR - 20220716 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 29 IP - 2 DP - 2008 Dec TI - Prevalence of Raynaud's phenomenon in healthy Turkish medical students and hospital personnel. PG - 185-8 LID - 10.1007/s00296-008-0666-9 [doi] AB - In this study, we investigated the frequency of Raynaud's phenomenon (RP) in medical students and hospital personnel and determined associated factors. Students and hospital personnel of our medical faculty (1,414 subjects; 838 females, 576 males, mean age, 27.2+/-6.6) were questioned for symptoms associated with RP, and the presence of smoking and headache. 530 subjects (37.5%) defined color changes on cold exposure. 51 subjects (3.6%) had biphasic or triphasic color changes (definite RP). The prevalence of definite RP in females (4.8%) was significantly higher than in males (1.9%) (P=0.005). The frequency of smoking subjects (45.1 vs. 28.8%) was significantly higher in patients with RP (P=0.009). There was numbness and/or paresthesia in 174 subjects (12.3%) who defined uniphasic color change on cold exposure (possible RP). Female sex and being a smoker were factors associated with RP in our study. FAU - Cakir, Necati AU - Cakir N AD - Department of Rheumatology, Trakya University Medical Faculty, 22030, Edirne, Turkey. FAU - Pamuk, Omer Nuri AU - Pamuk ON FAU - Dönmez, Salim AU - Dönmez S FAU - Barutçu, Ahmet AU - Barutçu A FAU - Diril, Hidayet AU - Diril H FAU - Odabaş, Esin AU - Odabaş E FAU - Kiliçcigil, Volkan AU - Kiliçcigil V LA - eng PT - Journal Article DEP - 20080806 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Female MH - Hospitals, University MH - Humans MH - Male MH - *Medical Staff, Hospital MH - Prevalence MH - Raynaud Disease/diagnosis/*epidemiology/physiopathology MH - Smoking/epidemiology/pathology/physiopathology MH - *Students, Medical MH - Surveys and Questionnaires MH - Turkey/epidemiology EDAT- 2008/08/07 09:00 MHDA- 2009/04/25 09:00 CRDT- 2008/08/07 09:00 PHST- 2008/02/15 00:00 [received] PHST- 2008/07/28 00:00 [accepted] PHST- 2008/08/07 09:00 [pubmed] PHST- 2009/04/25 09:00 [medline] PHST- 2008/08/07 09:00 [entrez] AID - 10.1007/s00296-008-0666-9 [doi] PST - ppublish SO - Rheumatol Int. 2008 Dec;29(2):185-8. doi: 10.1007/s00296-008-0666-9. Epub 2008 Aug 6. PMID- 16721713 OWN - NLM STAT- MEDLINE DCOM- 20060629 LR - 20060524 IS - 0012-0472 (Print) IS - 0012-0472 (Linking) VI - 131 IP - 21 DP - 2006 May 26 TI - [Raynaud's phenomenon--current diagnosis and therapy]. PG - 1223-7 FAU - Caspary, L AU - Caspary L AD - Angiologische Gemeinschaftspraxis und Gefässzentrum am Klinikum Oststadt, Hannover. LCaspary@t-online.de FAU - Creutzig, A AU - Creutzig A LA - ger PT - Journal Article PT - Review TT - Raynaud-Phänomen -- Aktuelle Diagnostik und Therapie. PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Diagnosis, Differential MH - Humans MH - Raynaud Disease/*diagnosis/etiology/*therapy RF - 15 EDAT- 2006/05/25 09:00 MHDA- 2006/06/30 09:00 CRDT- 2006/05/25 09:00 PHST- 2006/05/25 09:00 [pubmed] PHST- 2006/06/30 09:00 [medline] PHST- 2006/05/25 09:00 [entrez] AID - 10.1055/s-2006-941757 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2006 May 26;131(21):1223-7. doi: 10.1055/s-2006-941757. PMID- 35441772 OWN - NLM STAT- MEDLINE DCOM- 20220714 LR - 20221205 IS - 1529-8019 (Electronic) IS - 1396-0296 (Linking) VI - 35 IP - 7 DP - 2022 Jul TI - The efficacy of botulinum toxin A in the treatment of Raynaud's phenomenon in systemic sclerosis: A randomized self-controlled trial. PG - e15529 LID - 10.1111/dth.15529 [doi] AB - The current conservative and surgical treatments are not fully effective and have complications for Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). Botulinum toxin A (BTX-A) can be used to manage RP, but the literature mostly includes case reports and case series. Thus, we performed a randomized controlled trial to explore the efficacy of BTX-A in the treatment of RP secondary to SSc. Sixteen patients with RP secondary to SSc were recruited. One hand was randomly included in the BTX-A group and the other as control. Both hands were tested before treatment and 4 weeks later using qualitative and quantitative dermatoscopic assessments and the cold water test. Reynolds score (from 6.7 ± 4.0 to 2.9 ± 3.7, p < 0.001), T(base) (from 25.8 ± 3.0°C to 27.9 ± 2.1°C, p = 0.031) and T(change) (from 2.1 ± 1.2°C to 4.5 ± 2.1°C, p < 0.001) in the experimental group were improved, while there were no improvements in T(base) and T(change) in the control group. In the experimental group, the sum of the six dermoscopic parameters was improved after treatment (from 4.00 (3.00, 5.75) to 3.00 (2.00, 5.00), p = 0.002); the nailfold capillary pattern staging was also improved (from 2.00 (2.00, 3.00) to 2.00 (1.00, 3.00), p = 0.004). There were no improvements in the dermoscopic assessment in the control group. None of the patients reported adverse reactions such as infection, hematoma, hand muscle weakness, allergic reaction and nerve injury. In conclusion, local injection BTX-A to treat RP secondary to SSc might be safe and effective. CI - © 2022 Wiley Periodicals LLC. FAU - Du, Wei AU - Du W AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. FAU - Zhou, Mo AU - Zhou M AD - Department of Internal Medicine, China-Japan Friendship Hospital, Beijing, China. FAU - Zhang, Chunying AU - Zhang C AD - Department of Dermatology, Jilin City Hospital of Chemical Industry, Jilin, China. FAU - Sun, Qiuning AU - Sun Q AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20220427 PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/therapeutic use MH - Hand MH - Humans MH - Injections MH - *Raynaud Disease/diagnosis/drug therapy/etiology MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin A OT - dermoscopy OT - efficacy OT - systemic sclerosis EDAT- 2022/04/21 06:00 MHDA- 2022/07/15 06:00 CRDT- 2022/04/20 08:43 PHST- 2022/03/21 00:00 [revised] PHST- 2021/09/28 00:00 [received] PHST- 2022/04/18 00:00 [accepted] PHST- 2022/04/21 06:00 [pubmed] PHST- 2022/07/15 06:00 [medline] PHST- 2022/04/20 08:43 [entrez] AID - 10.1111/dth.15529 [doi] PST - ppublish SO - Dermatol Ther. 2022 Jul;35(7):e15529. doi: 10.1111/dth.15529. Epub 2022 Apr 27. PMID- 24789669 OWN - NLM STAT- MEDLINE DCOM- 20150706 LR - 20220408 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 34 IP - 11 DP - 2014 Nov TI - Effects of phosphodiesterase type 5 inhibitors on Raynaud's phenomenon. PG - 1623-6 LID - 10.1007/s00296-014-3025-z [doi] AB - Raynaud's phenomenon (RP) is commonly observed in fingers and toes of patients with connective tissue diseases (CTDs). However, existing vasodilators have very limited efficacy. In this study, phosphodiesterase type 5 inhibitors (PDE-5Is) were administered to evaluate efficacy on RP. Three patients with mixed connective tissue disease and three patients with systemic sclerosis having RP were enrolled. Oral sildenafil, vardenafil, or tadalafil was administered. The fingertip temperature was measured by thermography before and 120 min after administration. To evaluate longer effects, vardenafil was administered daily for 12 weeks; the fingertip temperature was measured by thermography before and 12 weeks after administration. As compared with the pre-administration of sildenafil, vardenafil, and tadalafil, the mean fingertip temperature increased by 2.17, 3.47, and 3.59 °C, respectively, in 120 min. In the 12-week trial with vardenafil in 3 patients, the mean fingertip temperature increased by 3.04, 7.96, and 3.32 °C from baseline in each patient. PDE-5Is significantly increased fingertip temperature within 120 min, and the effect of vardenafil lasted for 12 weeks under daily use. PDE-5Is were safe and would be an effective treatment for RP with CTDs. FAU - Kamata, Yasuyuki AU - Kamata Y AD - Division of Rheumatology and Clinical Immunology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi-ken, 329-0498, Japan, y.kamata@jichi.ac.jp. FAU - Minota, Seiji AU - Minota S LA - eng PT - Comparative Study PT - Journal Article DEP - 20140501 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Carbolines) RN - 0 (Imidazoles) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfonamides) RN - 0 (Sulfones) RN - 0 (Triazines) RN - 0 (Vasodilator Agents) RN - 5O8R96XMH7 (Vardenafil Dihydrochloride) RN - 742SXX0ICT (Tadalafil) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Administration, Oral MH - Adult MH - Aged MH - Carbolines/therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Imidazoles/therapeutic use MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/administration & dosage/*therapeutic use MH - Pilot Projects MH - Piperazines/therapeutic use MH - Prospective Studies MH - Purines/therapeutic use MH - Raynaud Disease/diagnosis/*drug therapy/enzymology/physiopathology MH - Regional Blood Flow/drug effects MH - Sildenafil Citrate MH - Skin Temperature/drug effects MH - Sulfonamides/therapeutic use MH - Sulfones/therapeutic use MH - Tadalafil MH - Thermography MH - Time Factors MH - Treatment Outcome MH - Triazines/therapeutic use MH - Vardenafil Dihydrochloride MH - Vasodilator Agents/administration & dosage/*therapeutic use EDAT- 2014/05/03 06:00 MHDA- 2015/07/07 06:00 CRDT- 2014/05/03 06:00 PHST- 2014/03/01 00:00 [received] PHST- 2014/04/13 00:00 [accepted] PHST- 2014/05/03 06:00 [entrez] PHST- 2014/05/03 06:00 [pubmed] PHST- 2015/07/07 06:00 [medline] AID - 10.1007/s00296-014-3025-z [doi] PST - ppublish SO - Rheumatol Int. 2014 Nov;34(11):1623-6. doi: 10.1007/s00296-014-3025-z. Epub 2014 May 1. PMID- 24517537 OWN - NLM STAT- MEDLINE DCOM- 20140611 LR - 20151119 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 43 IP - 3 DP - 2014 TI - Associations of nailfold capillary abnormalities and immunological markers in early Raynaud's phenomenon. PG - 226-33 LID - 10.3109/03009742.2013.847118 [doi] AB - OBJECTIVES: Nailfold capillaroscopy (NC) and laboratory tests for antinuclear antibodies (ANA) are routinely used in parallel for detection of emerging connective tissue disease (CTD) in patients with Raynaud's phenomenon (RP). The aim of this study was to assess the associations between distinct nailfold capillary abnormalities and concomitant autoantibodies in patients with incipient RP without previously known CTD. METHOD: Patients with incipient RP without previously known CTD were included in this retrospective analysis. We analysed the association of particular capillary abnormalities (reduced density, avascular fields, dilations, giant capillaries, haemorrhages, tortuosity, ramifications, oedema) with ANA and ANA subsets (anti-Scl-70, anti-CENP-B, anti-U1-RNP, anti-dsDNA, anti-SSA(Ro), anti-SSB(La), anti-Sm, and anti-Jo-1 antibodies). We also developed a score that allows the estimation of each patient's individual probability for the presence of an ANA titre ≥ 1:160. RESULTS: The final analysis comprised 2971 patients. Avascular fields, giant capillaries, reduced capillary density, and capillary oedema were closely related to an ANA titre ≥ 1:160. Both giant capillaries and avascular fields were associated with anti-Scl-70 and anti-CENP-B antibodies. Only a weak association was found between giant capillaries and anti-U1-RNP antibodies. Each patient's individual probability for the presence of an ANA titre ≥ 1:160 can be represented by a sum score comprising giant capillaries, reduced density, avascular fields, ramifications, and oedema as well as patients' sex and age. CONCLUSION: In patients with incipient RP, anti-Scl-70 and anti-CENP-B antibodies are related most specifically to distinct capillary alterations. Although a sum score can represent the patient's probability for elevated ANA titres, NC cannot substitute for immunological tests in patients with incipient RP. FAU - Schlager, O AU - Schlager O AD - Division of Angiology, Department of Medicine II, Medical University of Vienna , Austria. FAU - Kiener, H P AU - Kiener HP FAU - Stein, L AU - Stein L FAU - Hofkirchner, J AU - Hofkirchner J FAU - Zehetmayer, S AU - Zehetmayer S FAU - Ristl, R AU - Ristl R FAU - Perkmann, T AU - Perkmann T FAU - Smolen, J S AU - Smolen JS FAU - Koppensteiner, R AU - Koppensteiner R FAU - Gschwandtner, M E AU - Gschwandtner ME LA - eng PT - Comparative Study PT - Journal Article DEP - 20140212 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Age Factors MH - Antibodies, Antinuclear/*immunology MH - Area Under Curve MH - Biomarkers/analysis MH - Capillaries/*abnormalities MH - Comorbidity MH - Databases, Factual MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nail Diseases/*diagnosis/*epidemiology MH - Nails/*blood supply MH - Predictive Value of Tests MH - Prognosis MH - ROC Curve MH - Raynaud Disease/diagnosis/*epidemiology/*immunology MH - Retrospective Studies MH - Risk Assessment MH - Severity of Illness Index MH - Sex Factors EDAT- 2014/02/13 06:00 MHDA- 2014/06/12 06:00 CRDT- 2014/02/13 06:00 PHST- 2014/02/13 06:00 [entrez] PHST- 2014/02/13 06:00 [pubmed] PHST- 2014/06/12 06:00 [medline] AID - 10.3109/03009742.2013.847118 [doi] PST - ppublish SO - Scand J Rheumatol. 2014;43(3):226-33. doi: 10.3109/03009742.2013.847118. Epub 2014 Feb 12. PMID- 20071354 OWN - NLM STAT- MEDLINE DCOM- 20100610 LR - 20161125 IS - 1879-0844 (Electronic) IS - 1388-9842 (Linking) VI - 12 IP - 3 DP - 2010 Mar TI - Cardiac Raynaud's phenomenon induced by cold provocation as a predictor of long-term left ventricular dysfunction and remodelling in systemic sclerosis: 7-year follow-up study. PG - 268-75 LID - 10.1093/eurjhf/hfp198 [doi] AB - AIMS: The clinical importance of cold-induced reversible myocardial ischaemia, known as cardiac Raynaud's phenomenon (C-Raynaud), has not been established in systemic sclerosis (SSc). This prospective study investigated the impact of C-Raynaud on long-term irreversible left ventricular (LV) functional and morphologic deterioration in SSc. METHODS AND RESULTS: Fifty-one SSc patients with no clinical evidence of cardiac involvement were prospectively followed up for 7.1 +/- 2.2 years. Systolic LV dysfunction was defined as a LV ejection fraction <50%. Left ventricular remodelling was defined as an increase in LV volume during follow-up of more than 20% compared with baseline values. At the initial evaluation, C-Raynaud was found in 15 patients (29.4%). Of these, eight patients had severe C-Raynaud. None of the patients had systolic LV dysfunction. At the final evaluation, five patients had developed systolic LV dysfunction. In four of these five patients, the development of systolic LV dysfunction was associated with LV remodelling. At multivariate analysis, severe C-Raynaud was a strong independent determinant of the development of long-term systolic LV dysfunction. CONCLUSION: This study documents for the first time that severe C-Raynaud is a strong long-term predictor of systolic LV dysfunction in SSc patients. Detection of C-Raynaud is clinically important for identifying SSc patients at high risk of cardiac deterioration at latent stage. FAU - Mizuno, Reiko AU - Mizuno R AD - Department of General Medicine, Nara Medical University, 840 Shijo, Kashihara, Nara 634-8522, Japan. FAU - Fujimoto, Shinichi AU - Fujimoto S FAU - Saito, Yoshihiko AU - Saito Y FAU - Nakamura, Shinobu AU - Nakamura S LA - eng PT - Journal Article DEP - 20100112 PL - England TA - Eur J Heart Fail JT - European journal of heart failure JID - 100887595 SB - IM MH - Analysis of Variance MH - Cold Temperature/*adverse effects MH - Female MH - Humans MH - Linear Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Myocardial Ischemia/diagnostic imaging/*etiology/physiopathology MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/diagnostic imaging/*etiology/physiopathology MH - Risk Factors MH - Scleroderma, Systemic/*complications/physiopathology MH - Stroke Volume MH - Systole MH - Time Factors MH - Ultrasonography MH - Ventricular Dysfunction, Left/diagnostic imaging/*physiopathology MH - *Ventricular Remodeling EDAT- 2010/01/15 06:00 MHDA- 2010/06/11 06:00 CRDT- 2010/01/15 06:00 PHST- 2010/01/15 06:00 [entrez] PHST- 2010/01/15 06:00 [pubmed] PHST- 2010/06/11 06:00 [medline] AID - hfp198 [pii] AID - 10.1093/eurjhf/hfp198 [doi] PST - ppublish SO - Eur J Heart Fail. 2010 Mar;12(3):268-75. doi: 10.1093/eurjhf/hfp198. Epub 2010 Jan 12. PMID- 23414388 OWN - NLM STAT- MEDLINE DCOM- 20140402 LR - 20130704 IS - 1541-3144 (Electronic) IS - 0194-2638 (Linking) VI - 33 IP - 3 DP - 2013 Aug TI - Exercise using the Wii Fit plus with a child with primary Raynaud's disease and obesity: a case report. PG - 327-41 LID - 10.3109/01942638.2012.747583 [doi] AB - The purpose of this case report was to describe the use of the Nintendo® Wii Fit Plus as an alternative exercise for an 11-year-old child with primary Raynaud's disease (PRD) and obesity who was not involved in organized sports and had limited outdoor physical activity and exercise. The Wii Fit Plus exercise parameters are described as well as outcomes measured at baseline, 12 weeks, and 24 weeks. Specifically, we evaluated changes in body mass index (BMI), cardiorespiratory fitness, and health related quality of life (HRQL). Following the 24-week exercise program, the child's BMI decreased, cardiorespiratory fitness increased, and HRQL increased and were comparable to values in healthy children. These findings suggest that the Wii Fit Plus may have been an effective exercise strategy for this child. FAU - Qualls, Kathlene K AU - Qualls KK AD - Matanuska-Susitna Borough School District, Palmer, Alaska, USA. Kathlene.qualls@matsuk12.us FAU - Arnold, Sandra H AU - Arnold SH FAU - McEwen, Irene R AU - McEwen IR FAU - Jeffries, Lynn M AU - Jeffries LM LA - eng PT - Case Reports PT - Journal Article PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20130218 PL - England TA - Phys Occup Ther Pediatr JT - Physical & occupational therapy in pediatrics JID - 8109120 SB - IM MH - Body Mass Index MH - Child MH - Exercise Therapy/*methods MH - Female MH - Humans MH - Obesity/complications/*rehabilitation MH - Physical Fitness/physiology MH - Quality of Life MH - Raynaud Disease/complications/*rehabilitation MH - *Video Games EDAT- 2013/02/19 06:00 MHDA- 2014/04/03 06:00 CRDT- 2013/02/19 06:00 PHST- 2013/02/19 06:00 [entrez] PHST- 2013/02/19 06:00 [pubmed] PHST- 2014/04/03 06:00 [medline] AID - 10.3109/01942638.2012.747583 [doi] PST - ppublish SO - Phys Occup Ther Pediatr. 2013 Aug;33(3):327-41. doi: 10.3109/01942638.2012.747583. Epub 2013 Feb 18. PMID- 40726248 OWN - NLM STAT- MEDLINE DCOM- 20250917 LR - 20250917 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 90 IP - 4 DP - 2025 Aug TI - First evaluation of microvascular damage through nailfold capillaroscopy of an uncommon case of Raynaud's phenomenon in multiple sclerosis: Case report and literature review. PG - 185-190 LID - 10.1177/13860291251361329 [doi] AB - INTRODUCTION: The occurrence of Raynaud's phenomenon in patients with multiple sclerosis (MS) is infrequently documented in the literature. Some cases have been attributed to interferon-β therapy, while others have emerged in MS patients who sub-sequently developed Raynaud's phenomenon and/or systemic sclerosis. The association between microangiopathic damage and both Raynaud's phenomenon and systemic sclerosis is well-established, leading to the adoption of nailfold video-capillaroscopy (NVC) as a non invasive diagnostic tool for the initial clinical assessment of these conditions. CASE PRESENTATION: We present a case study of a 42-year-old female patient with Raynaud's phenomenon in MS. The patient underwent NVC in 2023 showing microvascular impairment and after one year and Natalizumab treatment she repeated NVC evaluation with significant improvement in previous microangiopathic changes. A comprehensive literature review regarding the relationship between MS and Raynaud's phenomenon was conducted using electronic databases (PUBMED, UpToDate, Google Scholar, ResearchGate), along with manual searches for relevant articles published up to December 2024. DISCUSSION: This case highlights the potential utility of NVC in the ongoing evaluation of microangiopathic damage in MS patients exhibiting Raynaud's phenomenon. By elucidating the overlap between these conditions, NVC may serve as a valuable tool in the clinical management of affected individuals. FAU - Mondini, Lucrezia AU - Mondini L AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Screm, Gianluca AU - Screm G AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Salton, Francesco AU - Salton F AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Confalonieri, Paola AU - Confalonieri P AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Chernovsky, Maria AU - Chernovsky M AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Trotta, Liliana AU - Trotta L AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Barbieri, Mariangela AU - Barbieri M AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - De Nes, Anna AU - De Nes A AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Maggisano, Marta AU - Maggisano M AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - D'Oria, Mario AU - D'Oria M AD - Division of Vascular and Endovascular Surgery, Department of Clinical Surgical and Health Sciences, Uni-versity of Trieste, Trieste, Italy. FAU - Hughes, Michael AU - Hughes M AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. FAU - Confalonieri, Marco AU - Confalonieri M AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, Hospital of Cattinara, Trieste, Italy. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20250729 PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 SB - IM MH - Humans MH - *Raynaud Disease/etiology/diagnosis/diagnostic imaging MH - Female MH - *Microscopic Angioscopy/methods MH - Adult MH - *Multiple Sclerosis/complications MH - *Nails/blood supply OTO - NOTNLM OT - Raynaud's phenomenon OT - microcirculation OT - multiple sclerosis OT - nailfold video-capillaroscopy COIS- Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/07/29 06:29 MHDA- 2025/09/17 12:46 CRDT- 2025/07/29 03:59 PHST- 2025/09/17 12:46 [medline] PHST- 2025/07/29 06:29 [pubmed] PHST- 2025/07/29 03:59 [entrez] AID - 10.1177/13860291251361329 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2025 Aug;90(4):185-190. doi: 10.1177/13860291251361329. Epub 2025 Jul 29. PMID- 40244470 OWN - NLM STAT- MEDLINE DCOM- 20250605 LR - 20250608 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 44 IP - 6 DP - 2025 Jun TI - Physical activity, sedentary behaviour and health-related quality of life in 929 women with primary Raynaud's phenomenon. PG - 2101-2107 LID - 10.1007/s10067-025-07416-w [doi] AB - INTRODUCTION: Women with primary Raynaud's phenomenon (RP) often experience a decreased health-related quality of life (HRQoL). A physically active lifestyle could improve vascular function and HRQoL. OBJECTIVE: This study aimed to quantify the amount and type of physical activity (PA) and sedentary behaviour, as well as HRQoL and stress, in women with primary RP from a large population-based cohort (Lifelines). METHODS: A total of 19,820 adult (≥ 18 years) women from the Lifelines cohort were included; 929 of these were classified as having RP based on the connective tissue disease (CTD) screening questionnaire. Participant characteristics, data on PA and sedentary behaviour, HRQoL and stress were retrieved from the database. RESULTS: Women with RP reported 300 min/week minutes of moderate-to-vigorous physical activity (MVPA), which was more than women without RP (255 min/week, p ≤ .001). Women (74%) with RP complied to health enhancing PA guidelines (70% of women without RP, p = .003). Sedentary time was comparable. Women with RP had a low score on almost all eight domains of the HRQoL questionnaire. The Long-term Difficulties Inventory (LDI) showed a high stress level in the RP group (p < .001). CONCLUSION: Most women with RP reported to spent a sufficient amount of time on MVPA and thus comply to health enhancing PA guidelines. The PA and sedentary behaviour of women with RP seems comparable to that of women without RP. However, HRQoL was lower and stress levels were higher in women with RP; more research is needed to elucidate the relation between PA and HRQoL in RP. Key Points • Experiencing symptoms of Raynaud's phenomenon seem no obstacle for being physically active. • Focus on women with symptoms of Raynaud's phenomenon in a large cohort. • Nearly 75% of the women with symptoms of Raynaud's phenomenon comply to the physical activity guidelines. • Women with symptoms of Raynaud's phenomenon have low levels of health-related quality of life and experience a high stress level. CI - © 2025. The Author(s). FAU - van de Zande, Saskia Corine AU - van de Zande SC AUID- ORCID: 0000-0002-3063-0260 AD - Division of Vascular Medicine, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. s.c.van.de.zande@umcg.nl. FAU - Eijkelenkamp, Karin AU - Eijkelenkamp K AD - Department of Endocrinology and Metabolic Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. FAU - Eman Abdulle, Amaal AU - Eman Abdulle A AUID- ORCID: 0000-0003-3766-2238 AD - Division of Vascular Medicine, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. FAU - Smit, Andries Jan AU - Smit AJ AD - Division of Vascular Medicine, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. FAU - Zwerver, Johannes AU - Zwerver J AUID- ORCID: 0000-0002-8499-2806 AD - Center for Human Movement Sciences, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. AD - Sports Valley, Gelderse Vallei Hospital, Ede, The Netherlands. FAU - van den Akker-Scheek, Inge AU - van den Akker-Scheek I AUID- ORCID: 0000-0002-1614-8419 AD - Department of Orthopaedics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. FAU - Mulder, Douwe Johannes AU - Mulder DJ AUID- ORCID: 0000-0003-3715-6474 AD - Division of Vascular Medicine, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. LA - eng PT - Journal Article DEP - 20250417 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - Female MH - *Quality of Life MH - *Sedentary Behavior MH - *Exercise MH - Middle Aged MH - Adult MH - *Raynaud Disease/psychology/physiopathology MH - Surveys and Questionnaires MH - Aged MH - Stress, Psychological MH - Cohort Studies PMC - PMC12141168 OTO - NOTNLM OT - Health-related quality of life OT - Physical activity OT - Raynaud's phenomenon COIS- Compliance with ethical standards. Disclosures: None. Institutional review board: The study was conducted in accordance with the Declaration of Helsinki and approved by the Medical Ethical Committee of the UMCG (protocol code 2007/152). EDAT- 2025/04/17 12:27 MHDA- 2025/06/05 12:30 PMCR- 2025/04/17 CRDT- 2025/04/17 11:17 PHST- 2025/01/08 00:00 [received] PHST- 2025/03/21 00:00 [accepted] PHST- 2025/03/20 00:00 [revised] PHST- 2025/06/05 12:30 [medline] PHST- 2025/04/17 12:27 [pubmed] PHST- 2025/04/17 11:17 [entrez] PHST- 2025/04/17 00:00 [pmc-release] AID - 10.1007/s10067-025-07416-w [pii] AID - 7416 [pii] AID - 10.1007/s10067-025-07416-w [doi] PST - ppublish SO - Clin Rheumatol. 2025 Jun;44(6):2101-2107. doi: 10.1007/s10067-025-07416-w. Epub 2025 Apr 17. PMID- 39152746 OWN - NLM STAT- MEDLINE DCOM- 20240817 LR - 20240817 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 8 DP - 2024 Aug TI - No seasonal trends in referrals for vascular investigations: insight into the diagnosis of Raynaud's phenomenon and systemic sclerosis. PG - 1699-1700 LID - 10.55563/clinexprheumatol/1eqvih [doi] FAU - Dinsdale, Graham AU - Dinsdale G AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Mandzuk, Melissa AU - Mandzuk M AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Manning, Joanne AU - Manning J AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, and Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Samaranayaka, Muditha AU - Samaranayaka M AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, and Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Letter DEP - 20240802 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/epidemiology MH - *Scleroderma, Systemic/diagnosis/epidemiology MH - *Referral and Consultation/trends MH - *Seasons MH - Female MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Adult MH - Practice Patterns, Physicians'/trends EDAT- 2024/08/17 15:43 MHDA- 2024/08/17 15:44 CRDT- 2024/08/17 05:33 PHST- 2023/09/19 00:00 [received] PHST- 2023/10/20 00:00 [accepted] PHST- 2024/08/17 15:44 [medline] PHST- 2024/08/17 15:43 [pubmed] PHST- 2024/08/17 05:33 [entrez] AID - 20362 [pii] AID - 10.55563/clinexprheumatol/1eqvih [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Aug;42(8):1699-1700. doi: 10.55563/clinexprheumatol/1eqvih. Epub 2024 Aug 2. PMID- 40619205 OWN - NLM STAT- MEDLINE DCOM- 20250706 LR - 20250706 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 21 IP - 5 DP - 2025 May TI - Some aspects of body thermoregulation, environmental temperature and vascular hypothesis in systemic sclerosis patients. PG - 501894 LID - S2173-5743(25)00071-1 [pii] LID - 10.1016/j.reumae.2025.501894 [doi] AB - In line with vascular hypothesis of systemic sclerosis (SSc), it is proposed that visceral Raynaud's phenomenon (RP)/endothelial dysfunction causes severe oxygen supply/consumption imbalance in internal organs, leading to visceral ischemic failure in early SSc, especially with cold environmental temperature (Te) and severe RP. There would also be a decrease in body thermogenesis and a decrease in heat loss caused by chronic visceral ischemia and by systemic vasculopathy, respectively. At any given time, these disorders could produce low core temperature (Tc). Hence, SSc is proposed as a candidate cause of secondary hypothermia. It is suggested that SSc could be an adaptive response in cold Te to systemic endothelial damage with secondary chronic visceral ischemia/slow metabolism. This pathophysiological mechanism is proposed in early visceral failure, prognosis, SSc phenotype according to ethnicity, and other manifestations. The impact of Te and Tc on SSc warrants further investigations. CI - Copyright © 2025 Sociedad Española de Reumatología (SER), Colegio Mexicano de Reumatología (CMR) and Elsevier España, S.L.U. All rights reserved. FAU - Olmedo Garzón, Francisco Javier AU - Olmedo Garzón FJ AD - Clínica Amstrong Internacional, Calle Guzmán el Bueno, 102, 28003 Madrid, Spain. Electronic address: jolmedogarzon@gmail.com. LA - eng PT - Journal Article PT - Review PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - *Scleroderma, Systemic/physiopathology/complications MH - *Body Temperature Regulation/physiology MH - Raynaud Disease/physiopathology/etiology MH - Cold Temperature/adverse effects MH - Hypothermia/etiology/physiopathology MH - Endothelium, Vascular/physiopathology OTO - NOTNLM OT - Body core temperature and scleroderma OT - Fenómeno de Raynaud y esclerodermia OT - Raynaud's phenomenon and scleroderma OT - Temperatura corporal central y esclerodermia OT - Temperatura y esclerodermia OT - Temperature and scleroderma EDAT- 2025/07/07 00:26 MHDA- 2025/07/07 00:27 CRDT- 2025/07/06 20:54 PHST- 2024/03/05 00:00 [received] PHST- 2025/04/10 00:00 [revised] PHST- 2025/04/30 00:00 [accepted] PHST- 2025/07/07 00:27 [medline] PHST- 2025/07/07 00:26 [pubmed] PHST- 2025/07/06 20:54 [entrez] AID - S2173-5743(25)00071-1 [pii] AID - 10.1016/j.reumae.2025.501894 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2025 May;21(5):501894. doi: 10.1016/j.reumae.2025.501894. PMID- 27998297 OWN - NLM STAT- MEDLINE DCOM- 20170512 LR - 20221207 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 10 IP - 1 DP - 2016 Dec 20 TI - Difficulties in diagnosis and treatment of severe secondary Raynaud's phenomenon in a Cameroonian woman: a case report. PG - 356 LID - 10.1186/s13256-016-1142-x [doi] LID - 356 AB - BACKGROUND: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment. CASE PRESENTATION: A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief. CONCLUSIONS: An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading. FAU - Agbor, Valirie Ndip AU - Agbor VN AD - Ibal Sub-Divisional Hospital, Oku, North West Region, Cameroon. nvagbor@gmail.com. FAU - Njim, Tsi AU - Njim T AD - Centre for Tropical Medicine and Global Health, Nuffield Department of Medicine, University of Oxford, Oxfordshire, UK. FAU - Aminde, Leopold Ndemnge AU - Aminde LN AD - Clinical Research Education, Networking and Consultancy (CRENC), Douala, Littoral, Cameroon. AD - School of Public Health, Faculty of Medicine & Biomedical Sciences, University of Queensland, Brisbane, Australia. LA - eng PT - Case Reports PT - Journal Article DEP - 20161220 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 RN - 0 (Analgesics, Opioid) RN - 0 (Anti-Bacterial Agents) RN - 0 (Calcium Channel Blockers) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 39J1LGJ30J (Tramadol) RN - BW9B0ZE037 (Sildenafil Citrate) RN - I9ZF7L6G2L (Nifedipine) RN - O6X5QGC2VB (Cloxacillin) SB - IM MH - Adult MH - Analgesics, Opioid/therapeutic use MH - Anti-Bacterial Agents/therapeutic use MH - Black People MH - Calcium Channel Blockers/therapeutic use MH - Cloxacillin/therapeutic use MH - Connective Tissue Diseases/*complications/pathology MH - *Directive Counseling MH - Female MH - Fingers/*pathology MH - Gangrene MH - Humans MH - Life Style MH - Microcirculation/*drug effects MH - Nifedipine/therapeutic use MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Raynaud Disease/complications/*pathology/therapy MH - *Risk Reduction Behavior MH - Severity of Illness Index MH - Sildenafil Citrate/therapeutic use MH - Toes/*pathology MH - Tramadol/therapeutic use MH - Treatment Outcome PMC - PMC5175299 OTO - NOTNLM OT - Cameroon OT - Connective tissue disease OT - Secondary Raynaud’s phenomenon EDAT- 2016/12/22 06:00 MHDA- 2017/05/13 06:00 PMCR- 2016/12/20 CRDT- 2016/12/22 06:00 PHST- 2016/10/03 00:00 [received] PHST- 2016/11/09 00:00 [accepted] PHST- 2016/12/22 06:00 [entrez] PHST- 2016/12/22 06:00 [pubmed] PHST- 2017/05/13 06:00 [medline] PHST- 2016/12/20 00:00 [pmc-release] AID - 10.1186/s13256-016-1142-x [pii] AID - 1142 [pii] AID - 10.1186/s13256-016-1142-x [doi] PST - epublish SO - J Med Case Rep. 2016 Dec 20;10(1):356. doi: 10.1186/s13256-016-1142-x. PMID- 23444022 OWN - NLM STAT- MEDLINE DCOM- 20130419 LR - 20141113 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 138 IP - 10 DP - 2013 Mar TI - [Raynaud's phenomenon in occupational vibration exposure]. PG - 473-6 LID - 10.1055/s-0032-1332913 [doi] AB - HISTORY AND ADMISSION FINDINGS: A 34-year-old female stonemason was referred for expert opinion. The question at issue was, whether she suffered from vibration-induced white finger disease. She was exposed to high-frequency hand-arm vibrations for many years. She reported white finger attacks at the long fingers, which were associated with cold weather. Until this point, physical findings were normal. INVESTIGATIONS: The cold water provocation test showed a slight delay of the rewarming for the long fingers of the right hand. The nailfold capillary microscopy was normal. DIAGNOSIS AND COURSE: The slight Raynaud's phenomenon was recognized as an occupational disease with a diagnosis of vibration-induced white finger disease. About three years later, the symptoms of the Raynaud's phenomenon had deteriorated, although the patient had finished working with vibrating tools. The cold water provocation test confirmed the deterioration. At this time, the patient had inflamed swellings of some joints caused by rheumatoid arthritis. CONCLUSION: The differential diagnosis of a Raynaud's phenomenon should include occupational causes. Occupational history is diagnostically indicative. If an occupational disease is assumed, a report must be filed. With respect to German social law, the deterioration of the Raynaud's phenomenon was caused by the rheumatoid arthritis, which is regarded as independent from the job. CI - © Georg Thieme Verlag KG Stuttgart · New York. FAU - Letzel, S AU - Letzel S AD - Institut für Arbeits-, Sozial- und Umweltmedizin der Universitätsmedizin der Johannes Gutenberg-Universität Mainz. FAU - Muttray, A AU - Muttray A LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Raynaud-Phänomen bei einer beruflichen Vibrationsbelastung. DEP - 20130226 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Adult MH - Diagnosis, Differential MH - Disease Progression MH - Female MH - Hand-Arm Vibration Syndrome/diagnosis/*etiology MH - Humans MH - Raynaud Disease/diagnosis/*etiology MH - Thermography EDAT- 2013/02/28 06:00 MHDA- 2013/04/23 06:00 CRDT- 2013/02/28 06:00 PHST- 2013/02/28 06:00 [entrez] PHST- 2013/02/28 06:00 [pubmed] PHST- 2013/04/23 06:00 [medline] AID - 10.1055/s-0032-1332913 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2013 Mar;138(10):473-6. doi: 10.1055/s-0032-1332913. Epub 2013 Feb 26. PMID- 34894267 OWN - NLM STAT- MEDLINE DCOM- 20220302 LR - 20220531 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 32 IP - 2 DP - 2022 Feb 28 TI - Proximal heat stress up-regulates angiopoietin-1 in fingers and reduces the severity of Raynaud's phenomenon in systemic sclerosis: a single-centre pilot study. PG - 351-357 LID - 10.1093/mr/roab014 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) is a peripheral vascular disorder that frequently occurs in systemic sclerosis (SSc). Although therapeutic heating seems reasonable given that RP is elicited by cold stimuli, the effects of heating are still unclear. We examined the effects of heating applied on various body parts in SSc patients with RP of fingers. METHODS: Fourteen SSc patients heated their neck, elbows, and wrists with disposable heating pads for 1 week each. The visual analogue scale (VAS) for RP during each heating period was compared with that of each 1-week pre-treatment interval. On the day after the expiration of each heating period, their finger temperature, the finger blood flow, and angiogenesis-related factors (vascular endothelial growth factor, endostatin, angiopoietin-1, and angiopoietin-2) obtained from the cubital vein and fingertip were measured. RESULTS: The mean VAS was significantly reduced during the heating of the neck and elbows. Fingertip blood samples showed significantly increased angiopoietin-1 after each of the heating periods and increased endostatin after wrist heating. After the termination of heating, changes in finger temperature or blood flow could not be detected. CONCLUSIONS: Heating the neck or elbows can alleviate RP in SSc. The heat up-regulates angiopoietin-1 in the fingers. CI - © Japan College of Rheumatology 2022. Published by Oxford University Press. FAU - Shima, Yoshihito AU - Shima Y AUID- ORCID: 0000-0002-2523-2938 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Watanabe, Akane AU - Watanabe A AUID- ORCID: 0000-0001-8451-9501 AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Inoue, Nobuto AU - Inoue N AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Maruyama, Tetsuya AU - Maruyama T AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Kunitomo, Eiji AU - Kunitomo E AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Hamano, Kunihiko AU - Hamano K AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Kawanishi, Takashi AU - Kawanishi T AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Takasugi, Masashi AU - Takasugi M AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd, Ibaraki, Japan. FAU - Kumanogoh, Atsushi AU - Kumanogoh A AUID- ORCID: 0000-0003-4749-7117 AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. LA - eng GR - Osaka University Graduate School of Medicine and Kobayashi Pharmaceutical Co.,Ltd/ PT - Journal Article PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Angiopoietin-1) RN - 0 (Vascular Endothelial Growth Factor A) SB - IM MH - *Angiopoietin-1/blood/metabolism MH - *Fingers MH - *Heat-Shock Response MH - Heating MH - Humans MH - Pilot Projects MH - *Raynaud Disease/etiology/therapy MH - *Scleroderma, Systemic/complications MH - Up-Regulation MH - Vascular Endothelial Growth Factor A/blood OTO - NOTNLM OT - Angiogenesis OT - Raynaud’s phenomenon OT - angiopoietin-1 OT - mild heat stress OT - systemic sclerosis EDAT- 2021/12/12 06:00 MHDA- 2022/03/03 06:00 CRDT- 2021/12/11 12:07 PHST- 2021/03/02 00:00 [received] PHST- 2021/05/07 00:00 [revised] PHST- 2021/05/24 00:00 [accepted] PHST- 2021/12/12 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2021/12/11 12:07 [entrez] AID - 6363900 [pii] AID - 10.1093/mr/roab014 [doi] PST - ppublish SO - Mod Rheumatol. 2022 Feb 28;32(2):351-357. doi: 10.1093/mr/roab014. PMID- 26715139 OWN - NLM STAT- MEDLINE DCOM- 20160825 LR - 20181113 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2015 DP - 2015 Dec 29 TI - Raynaud's phenomenon in limited cutaneous systemic sclerosis. LID - 10.1136/bcr-2015-212911 [doi] LID - bcr2015212911 FAU - Bonnecaze, Alex K AU - Bonnecaze AK AD - Department of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20151229 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Adult MH - Female MH - Fingers/pathology MH - Humans MH - Raynaud Disease/*complications/pathology MH - Scleroderma, Systemic/*complications/pathology PMC - PMC4716270 EDAT- 2015/12/31 06:00 MHDA- 2016/08/26 06:00 PMCR- 2017/12/29 CRDT- 2015/12/31 06:00 PHST- 2015/12/31 06:00 [entrez] PHST- 2015/12/31 06:00 [pubmed] PHST- 2016/08/26 06:00 [medline] PHST- 2017/12/29 00:00 [pmc-release] AID - bcr-2015-212911 [pii] AID - 10.1136/bcr-2015-212911 [doi] PST - epublish SO - BMJ Case Rep. 2015 Dec 29;2015:bcr2015212911. doi: 10.1136/bcr-2015-212911. PMID- 18799113 OWN - NLM STAT- MEDLINE DCOM- 20090205 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 4 DP - 2008 Jul-Aug TI - Inhaled iloprost for the treatment of Raynaud's phenomenon. PG - 709 FAU - Pakozdi, A AU - Pakozdi A FAU - Howell, K AU - Howell K FAU - Wilson, H AU - Wilson H FAU - Fox, S AU - Fox S FAU - Gonzalez, L AU - Gonzalez L FAU - Black, C M AU - Black CM FAU - Denton, C P AU - Denton CP LA - eng PT - Clinical Trial PT - Letter PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Administration, Inhalation MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Iloprost/*administration & dosage MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*drug therapy MH - Scleroderma, Systemic/complications MH - Vasodilator Agents/*administration & dosage EDAT- 2008/09/19 09:00 MHDA- 2009/02/06 09:00 CRDT- 2008/09/19 09:00 PHST- 2008/09/19 09:00 [pubmed] PHST- 2009/02/06 09:00 [medline] PHST- 2008/09/19 09:00 [entrez] AID - 2417 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 Jul-Aug;26(4):709. PMID- 19878974 OWN - NLM STAT- MEDLINE DCOM- 20110113 LR - 20100929 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 40 IP - 2 DP - 2010 Oct TI - Systemic sclerosis: bilateral improvement of Raynaud's phenomenon with unilateral digital sympathectomy. PG - 137-46 LID - 10.1016/j.semarthrit.2009.08.002 [doi] AB - OBJECTIVES: To demonstrate that unilateral digital sympathectomy, in patients with Raynaud's phenomenon (RP) and systemic sclerosis (SSc), may result in bilateral resolution of RP and digital ulcerations. METHODS: We report a case of SSc and RP that had bilateral benefits from unilateral digital sympathectomy. A computer-assisted Medline/PubMed search of the medical literature was performed for 1960 through June 2009 using the keywords sympathectomy, Raynaud's phenomenon, systemic sclerosis, CREST, and digital ulcers. These searches were also combined with text words unilateral, ipsilateral, bilateral, digital sympathectomy, selective sympathectomy, autonomic nervous system, hyperhidrosis, etiology, pathogenesis, hypothesis, and treatment. Only pertinent literature, primarily in the English language, was included. RESULTS: The majority of patients with SSc have RP and many suffer from digital ulcerations. Medical and behavioral management may have limited benefit and surgical intervention can be considered in recalcitrant cases, although efficacy data are sparse. We describe a man with limited SSc who underwent unilateral digital sympathectomy but manifested bilateral benefit. To our knowledge, this is the first published report of contralateral response with this procedure. The patient ultimately demonstrated these digital benefits when stressed with extreme cold and hypoxia while mountaineering. Despite the onset of high-altitude sickness and cerebral edema, his fingers remained unaffected while other mountaineers sustained severe frostbite or died of hypothermia. CONCLUSIONS: Selective unilateral sympathectomy in SSc, for RP with digital ulcerations, can result in bilateral benefits despite intense challenge with cold and hypoxia. CI - © 2010 Elsevier Inc. All rights reserved. FAU - Wasserman, Amy AU - Wasserman A AD - Division of Rheumatology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA 90095-1670, USA. FAU - Brahn, Ernest AU - Brahn E LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20091030 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Altitude MH - Fingers/innervation/pathology/*surgery MH - Humans MH - MEDLINE MH - Male MH - Mountaineering MH - Raynaud Disease/complications/pathology/*surgery MH - Regional Blood Flow MH - Scleroderma, Systemic/complications/pathology/*surgery MH - Skin Ulcer/etiology/surgery MH - Sympathectomy/*methods MH - Treatment Outcome EDAT- 2009/11/03 06:00 MHDA- 2011/01/14 06:00 CRDT- 2009/11/03 06:00 PHST- 2009/06/06 00:00 [received] PHST- 2009/08/10 00:00 [revised] PHST- 2009/08/24 00:00 [accepted] PHST- 2009/11/03 06:00 [entrez] PHST- 2009/11/03 06:00 [pubmed] PHST- 2011/01/14 06:00 [medline] AID - S0049-0172(09)00097-3 [pii] AID - 10.1016/j.semarthrit.2009.08.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2010 Oct;40(2):137-46. doi: 10.1016/j.semarthrit.2009.08.002. Epub 2009 Oct 30. PMID- 17394182 OWN - NLM STAT- MEDLINE DCOM- 20070501 LR - 20151119 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 57 IP - 3 DP - 2007 Apr 15 TI - Heritability of Raynaud's phenomenon and vascular responsiveness to cold: a study of adult female twins. PG - 524-8 FAU - Cherkas, L F AU - Cherkas LF AD - Twin Research and Genetic Epidemiology Unit, St. Thomas' Hospital, Kings College, London, UK. lynn.cherkas@gstt.nhs.uk FAU - Williams, F M K AU - Williams FM FAU - Carter, L AU - Carter L FAU - Howell, K AU - Howell K FAU - Black, C M AU - Black CM FAU - Spector, T D AU - Spector TD FAU - MacGregor, A J AU - MacGregor AJ LA - eng GR - Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Twin Study PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adult MH - Body Temperature MH - *Cold Temperature MH - Diseases in Twins MH - Female MH - Humans MH - Immersion MH - Incidence MH - Middle Aged MH - Raynaud Disease/*epidemiology/*genetics/physiopathology MH - Severity of Illness Index MH - Surveys and Questionnaires MH - *Twins MH - Twins, Monozygotic MH - Vasoconstriction/*genetics EDAT- 2007/03/31 09:00 MHDA- 2007/05/02 09:00 CRDT- 2007/03/31 09:00 PHST- 2007/03/31 09:00 [pubmed] PHST- 2007/05/02 09:00 [medline] PHST- 2007/03/31 09:00 [entrez] AID - 10.1002/art.22626 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Apr 15;57(3):524-8. doi: 10.1002/art.22626. PMID- 23720085 OWN - NLM STAT- MEDLINE DCOM- 20150817 LR - 20181023 IS - 2458-9446 (Electronic) IS - 1300-0012 (Linking) VI - 25 IP - 2 DP - 2013 TI - A case of recurrent complex regional pain syndrome accompanying Raynaud's disease: a prospective coincidence? PG - 90-2 LID - 10.5505/agri.2013.31932 [doi] AB - Complex regional pain syndrome (CPRS) and Raynaud's disease are disorders characterized by vasomotor disturbances associating with abnormal autonomic nervous system. We present a case of CRPS involving a history of recurrence and no initiating event. Raynaud's disease accompanying CRPS was diagnosed clinically in the patient. We propose that a sympathetic dysfunction underlies the pathophysiologies of both disorders and may be responsible for the coexistence of these two distinct entities. Recurrence and unknown etiology of CRPS might account for temporary alterations in sympathetic function. FAU - Kesikburun, Serdar AU - Kesikburun S AD - Department of Physical Medicine and Rehabilitation, Gülhane Military Medical Academy, Turkish Armed Forces Rehabilitation Center, Ankara, Turkey. serdarkb@gmail.com FAU - Günendi, Zafer AU - Günendi Z FAU - Aydemir, Koray AU - Aydemir K FAU - Özgül, Ahmet AU - Özgül A FAU - Tan, Arif Kenan AU - Tan AK LA - eng PT - Case Reports PT - Journal Article PL - Turkey TA - Agri JT - Agri : Agri (Algoloji) Dernegi'nin Yayin organidir = The journal of the Turkish Society of Algology JID - 9426197 SB - IM MH - Adult MH - Diagnosis, Differential MH - Extremities MH - Humans MH - Male MH - Raynaud Disease/*complications MH - Reflex Sympathetic Dystrophy/complications/*diagnosis EDAT- 2013/05/31 06:00 MHDA- 2015/08/19 06:00 CRDT- 2013/05/31 06:00 PHST- 2013/05/31 06:00 [entrez] PHST- 2013/05/31 06:00 [pubmed] PHST- 2015/08/19 06:00 [medline] AID - 10.5505/agri.2013.31932 [doi] PST - ppublish SO - Agri. 2013;25(2):90-2. doi: 10.5505/agri.2013.31932. PMID- 19369451 OWN - NLM STAT- MEDLINE DCOM- 20090805 LR - 20220408 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 6 DP - 2009 Jun TI - Raynaud's phenomenon and plasma endothelin: correlations with capillaroscopic patterns in systemic sclerosis. PG - 1235-9 LID - 10.3899/jrheum.081030 [doi] AB - OBJECTIVE: We evaluated endothelin (ET)-1 plasma levels and some clinical measures in patients with primary Raynaud's phenomenon (PRP), and in patients with systemic sclerosis (SSc) and secondary RP (SRP), in the latter according to their different nailfold videocapillaroscopy (NVC) patterns of microangiopathy (early, active, and late). METHODS: Ninety-nine patients with SSc, 49 with PRP, and 45 control subjects were studied. NVC was performed in all patients to distinguish the pattern of microvascular damage, and the morphological alterations were scored by a semiquantitative rating scale. ET-1 plasma levels were evaluated in all individuals by ELISA. RESULTS: ET-1 plasma levels were significantly higher (p=0.001) in patients with both PRP and SRP, compared to controls. A significant positive correlation (p=0.03) was found between ET-1 plasma levels and SRP duration, but not between ET-1 plasma levels and PRP duration. Significant correlations were observed in patients with SSc between ET-1 plasma levels and clinical measures (e.g., digital ulcers), as well as the score value of single NVC measures, such as the number of capillaries, "ramified" capillaries, and enlarged capillaries (p<0.05). Finally, the highest ET-1 plasma levels were found in patients with SSc showing the late pattern of microangiopathy when compared to the early pattern (p=0.03) and to controls (p=0.003). CONCLUSION: Highest ET-1 plasma levels were detected in the more advanced stage of the SSc microangiopathy, namely the late NVC pattern, characterized by capillary loss and increased tissue fibrosis; this might support the involvement of ET-1 in the progression of the microvascular/fibrotic SSc damage. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV 6, 16132 Genova, Italy. FAU - Soldano, Stefano AU - Soldano S FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Montagna, Paola AU - Montagna P FAU - Secchi, Maria Elena AU - Secchi ME FAU - Villaggio, Barbara AU - Villaggio B FAU - Seriolo, Bruno AU - Seriolo B FAU - Brizzolara, Renata AU - Brizzolara R FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article DEP - 20090415 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Endothelin-1) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Capillaries/pathology MH - Endothelin-1/*blood MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease/blood/complications/*pathology MH - Scleroderma, Systemic/blood/complications/*pathology MH - Skin/blood supply/pathology MH - Young Adult EDAT- 2009/04/17 09:00 MHDA- 2009/08/06 09:00 CRDT- 2009/04/17 09:00 PHST- 2009/04/17 09:00 [entrez] PHST- 2009/04/17 09:00 [pubmed] PHST- 2009/08/06 09:00 [medline] AID - jrheum.081030 [pii] AID - 10.3899/jrheum.081030 [doi] PST - ppublish SO - J Rheumatol. 2009 Jun;36(6):1235-9. doi: 10.3899/jrheum.081030. Epub 2009 Apr 15. PMID- 33944714 OWN - NLM STAT- MEDLINE DCOM- 20211130 LR - 20211130 IS - 2078-5135 (Electronic) VI - 111 IP - 2 DP - 2021 Feb 1 TI - Artifactual hypoglycaemia in a patient with scleroderma and Raynaud's phenomenon. PG - 13202 LID - 10.7196/SAMJ.2021.v111i2.15451 [doi] FAU - Osman, R AU - Osman R AD - Department of Emergency Medicine, Khayelitsha Hospital, Cape Town, South Africa. osman.ridwaan@gmail.com. FAU - Erasmus, E AU - Erasmus E FAU - Lahri, S AU - Lahri S FAU - Moosajee, F AU - Moosajee F LA - eng PT - Case Reports PT - Letter DEP - 20210201 PL - South Africa TA - S Afr Med J JT - South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde JID - 0404520 RN - 0 (Blood Glucose) SB - IM MH - Blood Glucose/metabolism MH - Female MH - Humans MH - Hypoglycemia/*complications/metabolism MH - Middle Aged MH - Raynaud Disease/*complications/metabolism MH - Scleroderma, Systemic/*complications/metabolism MH - South Africa EDAT- 2021/05/05 06:00 MHDA- 2021/12/01 06:00 CRDT- 2021/05/04 12:26 PHST- 2021/02/01 00:00 [received] PHST- 2021/05/04 12:26 [entrez] PHST- 2021/05/05 06:00 [pubmed] PHST- 2021/12/01 06:00 [medline] AID - 10.7196/SAMJ.2021.v111i2.15451 [doi] PST - epublish SO - S Afr Med J. 2021 Feb 1;111(2):13202. doi: 10.7196/SAMJ.2021.v111i2.15451. PMID- 41327988 OWN - NLM STAT- MEDLINE DCOM- 20251202 LR - 20251202 IS - 2791-6421 (Electronic) IS - 0041-4301 (Linking) VI - 67 IP - 5 DP - 2025 Oct 15 TI - Heart rate variability as a marker of autonomic dysfunction in children with primary Raynaud's phenomenon. PG - 711-719 LID - 10.24953/turkjpediatr.2025.6241 [doi] AB - BACKGROUND: Primary Raynaud's phenomenon (RP) is a functional vasospastic disorder triggered by cold or emotional stress, often occurring without an underlying systemic disease. As autonomic dysfunction is thought to contribute to RP pathogenesis, heart rate variability (HRV) analysis may provide insights into underlying mechanisms. This study aimed to assess autonomic nervous system activity in children with primary RP using HRV parameters. METHODS: The study included 36 primary RP patients (0-18 years) and age- and gender-matched 30 healthy controls with normal 24-hour Holter electrocardiograms (ECG). Data on demographics, laboratory results, 24-hour Holter ECG, capillaroscopy, and treatment were collected. HRV was analyzed in both the time and frequency domains. RESULTS: In the patient group, 11 (30.4%) were male, and 25 (69.6%) were female, with a median age of 15 (8-18) years. Symptom onset occurred at a median age of 14. The attack patterns were biphasic in 36.1% of patients, triphasic in 30.6%, and monophasic in 33.3%. Capillaroscopy was normal in 16 (44.4%) patients, with minor changes in 20 (55.6%). Six (16.6%) patients had positive antinuclear antibody (ANA) with no autoimmune disease diagnoses. Holter ECG monitoring results were compared with those of healthy controls (median age 15 years), showing significant differences in standard deviation of all normal-to-normal intervals (SDNN) and standard deviation of successive differences between adjacent RR intervals (SDSD) between primary RP patients and controls, but no differences in root mean square of successive differences (RMSSD) or HRV index values. CONCLUSION: Pediatric patients with primary RP showed significant autonomic changes compared to controls, though it remains unclear if these changes favor sympathetic or parasympathetic pathways. Further multicenter, prospective studies are needed to clarify these findings. FAU - Ulusoy, Samet AU - Ulusoy S AD - Department of Pediatrics, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye. FAU - Akgün, Gökmen AU - Akgün G AD - Department of Pediatric Cardiology, Kocaeli City Hospital, Kocaeli, Türkiye. FAU - Bayrak, Yunus Emre AU - Bayrak YE AD - Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye. FAU - Başar, Eviç Zeynep AU - Başar EZ AD - Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye. FAU - Sönmez, Hafize Emine AU - Sönmez HE AD - Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye. FAU - Şahin, Nihal AU - Şahin N AD - Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye. LA - eng PT - Journal Article DEP - 20251015 PL - Turkey TA - Turk J Pediatr JT - The Turkish journal of pediatrics JID - 0417505 SB - IM MH - Humans MH - Female MH - *Raynaud Disease/physiopathology/diagnosis/complications MH - Child MH - Male MH - Adolescent MH - *Heart Rate/physiology MH - Electrocardiography, Ambulatory MH - Child, Preschool MH - Case-Control Studies MH - *Autonomic Nervous System Diseases/physiopathology/diagnosis MH - Infant MH - *Autonomic Nervous System/physiopathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - antinuclear antibody OT - autonomic nervous system activity OT - capillaroscopy OT - heart rate variability COIS- The authors declare that there is no conflict of interest. EDAT- 2025/12/02 07:36 MHDA- 2025/12/02 07:37 CRDT- 2025/12/02 03:27 PHST- 2025/04/29 00:00 [received] PHST- 2025/10/03 00:00 [accepted] PHST- 2025/12/02 07:37 [medline] PHST- 2025/12/02 07:36 [pubmed] PHST- 2025/12/02 03:27 [entrez] AID - 6241 [pii] AID - 10.24953/turkjpediatr.2025.6241 [doi] PST - epublish SO - Turk J Pediatr. 2025 Oct 15;67(5):711-719. doi: 10.24953/turkjpediatr.2025.6241. PMID- 16538393 OWN - NLM STAT- MEDLINE DCOM- 20070619 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 4 DP - 2007 Apr TI - High resolution 3 Tesla contrast-enhanced MR angiography of the hands in Raynaud's disease. PG - 587-9 AB - Raynaud's disease is associated with disorders in blood circulation of the hands. The gold standard to visualise pathology of digital arteries is catheter angiography. Contrast-enhanced MR angiography (CE MRA) has developed even more as an alternative non-invasive method to digital subtraction angiography, mostly for pelvic or lower limb vessels. We report a case of primary Raynaud's disease with high-grade stenosis and an occlusion of the digital arteries. This case illustrates the benefit and efficiency of CE MRA at high fields in depicting location and extension of peripheral arterial alterations. FAU - Walcher, J AU - Walcher J AD - Department of Diagnostic Radiology and Medical Physics, University Hospital Freiburg, Hugstetter Strasse 55, 79106 Freiburg, Germany. FAU - Strecker, R AU - Strecker R FAU - Goldacker, S AU - Goldacker S FAU - Winterer, J AU - Winterer J FAU - Langer, M AU - Langer M FAU - Bley, T A AU - Bley TA LA - eng PT - Case Reports PT - Journal Article DEP - 20060315 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aortic Valve Stenosis/pathology MH - Female MH - Humans MH - Magnetic Resonance Angiography/*methods MH - Peripheral Vascular Diseases/*pathology MH - Raynaud Disease/*pathology EDAT- 2006/03/16 09:00 MHDA- 2007/06/20 09:00 CRDT- 2006/03/16 09:00 PHST- 2005/11/18 00:00 [received] PHST- 2005/12/03 00:00 [accepted] PHST- 2006/03/16 09:00 [pubmed] PHST- 2007/06/20 09:00 [medline] PHST- 2006/03/16 09:00 [entrez] AID - 10.1007/s10067-005-0172-3 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Apr;26(4):587-9. doi: 10.1007/s10067-005-0172-3. Epub 2006 Mar 15. PMID- 28514385 OWN - NLM STAT- MEDLINE DCOM- 20190529 LR - 20190529 IS - 0023-1207 (Print) IS - 0023-1207 (Linking) IP - 5 DP - 2017 TI - [The effects of diosmin and hesperidin on capillary blood flow of upper limbs in patients with secondary Raynaud's syndrome]. PG - 60-66 LID - 10.17116/hirurgia2017560-66 [doi] AB - The article discusses the effects of diosmin and hesperidin on capillary blood flow in patients with secondary Raynaud's syndrome (RS). Raynaud's syndrome a difficult problem of modern angiology, since in its development there is a large range of disorders of the capillary blood flow of the limbs. Currently, the main way of therapy in patients with secondary Raynaud's syndrome is the use of either calcium channel blockers (if angiospastic stage of the disease), or prostaglandins of group E1 (with tropho-paralytic stage of secondary RS). However, pharmacological effects of calcium channel blockers (vasodilation) and prostaglandins (vasodilation, antiproliferative, anti-inflammatory etc.) do not allow impact on all violations of capillary blood flow, occurs when the Raynaud's syndrome. In this regard, the task was to study the reaction of capillary blood flow of the fingers of the hands on the concomitant use of drugs based on diosmin and hesperidin. A prerequisite for the use of a combination of diosmin and hesperidin in the treatment of RS was based on data about their impact on the state of the venous segment of the capillary bed and perivascular oedema. To conduct the study was established two groups of patients (22 in each group). The main criterion for inclusion of patients in the study was the presence of newly identified Raynaud's syndrome of angiospastic or tropho-paralytic stage of the disease. The main exclusion criteria was the presence of necrotic stage of the disease. In the first group of patients therapy was carried out using only vasodilators. In the second group (comparison group) with the addition of a combination of diosmin and hesperidin (tablets Venarus used in a dose 500 mg 2 times per day). The condition of capillary blood flow in this study was estimated by the method of videocapillaroscopy (VCS). Statistical data processing was performed using a criterion of statistical significance (P-value). The study revealed the significant impact of a combination of diosmin and hesperidin for parameters of capillary blood flow that correlated with clinical improvements - reduction of oedema of the fingers of the hands. FAU - Zudin, A M AU - Zudin AM AD - Department of Hospital Surgery with the course of pediatric surgery, Medical Faculty of Russian People's Friendship University, Moscow. FAU - Gritsenko, A G AU - Gritsenko AG AD - Gritsenko Medico-Engineering Research Center, Moscow. FAU - Hadzhishvili, I T AU - Hadzhishvili IT AD - Department of Hospital Surgery with the course of pediatric surgery, Medical Faculty of Russian People's Friendship University, Moscow. LA - rus PT - Journal Article TT - Vozdeĭstvie diosmina i gesperidina na kapilliarnyĭ krovotok verkhnikh konechnosteĭ u patsientov s vtorichnym sindromom Reĭno. PL - Russia (Federation) TA - Khirurgiia (Mosk) JT - Khirurgiia JID - 0412765 RN - 0 (Calcium Channel Blockers) RN - 7QM776WJ5N (Diosmin) RN - E750O06Y6O (Hesperidin) SB - IM MH - *Calcium Channel Blockers/therapeutic use MH - Capillaries/drug effects MH - *Diosmin/therapeutic use MH - *Hesperidin/therapeutic use MH - Humans MH - *Raynaud Disease/drug therapy/etiology EDAT- 2017/05/18 06:00 MHDA- 2019/05/30 06:00 CRDT- 2017/05/18 06:00 PHST- 2017/05/18 06:00 [entrez] PHST- 2017/05/18 06:00 [pubmed] PHST- 2019/05/30 06:00 [medline] AID - 10.17116/hirurgia2017560-66 [doi] PST - ppublish SO - Khirurgiia (Mosk). 2017;(5):60-66. doi: 10.17116/hirurgia2017560-66. PMID- 19364728 OWN - NLM STAT- MEDLINE DCOM- 20100608 LR - 20250529 IS - 1468-2060 (Electronic) IS - 0003-4967 (Print) IS - 0003-4967 (Linking) VI - 69 IP - 3 DP - 2010 Mar TI - The minimally important difference and patient acceptable symptom state for the Raynaud's condition score in patients with Raynaud's phenomenon in a large randomised controlled clinical trial. PG - 588-91 LID - 10.1136/ard.2009.107706 [doi] AB - BACKGROUND: The Raynaud's condition score (RCS) is a validated outcome measure for Raynaud's phenomenon (RP). OBJECTIVE: To assess the minimally important difference (MID) and patient acceptable symptom state (PASS) for RCS in patients with RP. SUBJECTS: and methods Patients with active RP (n=162) (mean RCS >25 (0-100 visual analogue scale) participated in a placebo-controlled, crossover randomised clinical trial (RCT). Data from the two treatment groups were combined for this analysis. Retrospective and prospective anchors were administered during the RCT. MID groups were defined as the group who reported being somewhat better (anchor #1) and a one-step change from "unbearable" to "very severe", etc (anchor #2). Patients were considered to have achieved PASS if they rated their Raynaud's condition as "very mild" or "mild" at the last study visit. RESULTS: The mean age of participants was 48.9 years and the mean baseline RCS was 46.4 points. The RCS change score for the MID improvement group ranged from -13.9 to -14.3 points and PASS estimate was 34.0 points. CONCLUSION: The MID and PASS estimates for RCS are 14-15 points for improvement and 34 points, respectively, on a 0-100 scale in a large RCT of patients with active RP. This information can aid in interpreting RCS in future RP trials. FAU - Khanna, Puja P AU - Khanna PP AD - Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, Los Angeles, CA 90095, USA. FAU - Maranian, Paul AU - Maranian P FAU - Gregory, Jeff AU - Gregory J FAU - Khanna, Dinesh AU - Khanna D LA - eng GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - T32 AR053463/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial DEP - 20090412 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Vasodilator Agents) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Disability Evaluation MH - Epidemiologic Methods MH - Female MH - Humans MH - Male MH - Middle Aged MH - Patient Acceptance of Health Care/*psychology MH - Raynaud Disease/diagnosis/drug therapy/*psychology MH - Vasodilator Agents/therapeutic use MH - Young Adult PMC - PMC2837770 MID - NIHMS131129 EDAT- 2009/04/15 09:00 MHDA- 2010/06/09 06:00 PMCR- 2010/09/01 CRDT- 2009/04/15 09:00 PHST- 2009/04/15 09:00 [entrez] PHST- 2009/04/15 09:00 [pubmed] PHST- 2010/06/09 06:00 [medline] PHST- 2010/09/01 00:00 [pmc-release] AID - S0003-4967(24)19603-3 [pii] AID - 10.1136/ard.2009.107706 [doi] PST - ppublish SO - Ann Rheum Dis. 2010 Mar;69(3):588-91. doi: 10.1136/ard.2009.107706. Epub 2009 Apr 12. PMID- 17530659 OWN - NLM STAT- MEDLINE DCOM- 20070720 LR - 20220408 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 6 DP - 2007 Jun TI - Identification of transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon by nailfold videocapillaroscopy: comment on the article by Hirschl et al. PG - 2102-3; author reply 2103-4 FAU - Cutolo, Maurizio AU - Cutolo M FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Sulli, Alberto AU - Sulli A LA - eng PT - Comment PT - Letter PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antibodies, Antinuclear) SB - IM CON - Arthritis Rheum. 2006 Jun;54(6):1974-81. doi: 10.1002/art.21912. PMID: 16732585 MH - Antibodies, Antinuclear/blood MH - Disease Progression MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Prognosis MH - Raynaud Disease/*diagnosis/immunology/physiopathology MH - Video Recording/methods EDAT- 2007/05/29 09:00 MHDA- 2007/07/21 09:00 CRDT- 2007/05/29 09:00 PHST- 2007/05/29 09:00 [pubmed] PHST- 2007/07/21 09:00 [medline] PHST- 2007/05/29 09:00 [entrez] AID - 10.1002/art.22636 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Jun;56(6):2102-3; author reply 2103-4. doi: 10.1002/art.22636. PMID- 37537038 OWN - NLM STAT- MEDLINE DCOM- 20231108 LR - 20231114 IS - 0219-3108 (Electronic) IS - 1015-9584 (Linking) VI - 46 IP - 11 DP - 2023 Nov TI - A case report of polymyositis accompanied by Raynaud's phenomenon. PG - 5176-5177 LID - S1015-9584(23)01004-7 [pii] LID - 10.1016/j.asjsur.2023.07.020 [doi] FAU - Duan, Xiaokai AU - Duan X AD - Department of General Practice, Zhengzhou First People's Hospital, Zhengzhou, China. Electronic address: a2558040221@163.com. FAU - Yang, Shuhan AU - Yang S AD - Department of General Practice, Zhengzhou First People's Hospital, Zhengzhou, China. FAU - Zhao, Limin AU - Zhao L AD - Pathology Department, Zhengzhou First People's Hospital, Zhengzhou, China. LA - eng PT - Case Reports PT - Letter DEP - 20230802 PL - Netherlands TA - Asian J Surg JT - Asian journal of surgery JID - 8900600 SB - IM MH - Humans MH - *Raynaud Disease/etiology MH - *Polymyositis/complications OTO - NOTNLM OT - Electromyography OT - Muscle biopsy OT - Polymyositis OT - Raynaud's phenomenon COIS- Declaration of competing interest The authors declare that they have no conflicts of interest. EDAT- 2023/08/04 01:07 MHDA- 2023/11/08 06:43 CRDT- 2023/08/03 21:55 PHST- 2023/06/14 00:00 [received] PHST- 2023/07/07 00:00 [accepted] PHST- 2023/11/08 06:43 [medline] PHST- 2023/08/04 01:07 [pubmed] PHST- 2023/08/03 21:55 [entrez] AID - S1015-9584(23)01004-7 [pii] AID - 10.1016/j.asjsur.2023.07.020 [doi] PST - ppublish SO - Asian J Surg. 2023 Nov;46(11):5176-5177. doi: 10.1016/j.asjsur.2023.07.020. Epub 2023 Aug 2. PMID- 23422338 OWN - NLM STAT- MEDLINE DCOM- 20141112 LR - 20220316 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 56 IP - 2 DP - 2014 TI - Homocysteine levels in patients with primary and secondary Raynaud's phenomenon. Its association with microangiopathy severity. PG - 153-9 LID - 10.3233/CH-131681 [doi] AB - The association between hyperhomocysteinemia (HHcy) and Raynaud's phenomenon (RP) remains a matter of debate. In 18 primary RP, 23 secondary RP and 41 controls, we investigated homocysteine (Hcy) levels along with biochemical and inflammatory parameters. The Hcy levels in both primary and secondary RP were elevated when compared with controls (p < 0.05 and p < 0.01, respectively). As age was higher in secondary RP as compared with controls (p < 0.01), both primary and secondary RP were age-matched with a corresponding control group, and with Hcy maintaining its statistical significance (p < 0.05). No differences in creatinine, B12 vitamin or folic acid were observed between groups (p > 0.05), or in the prevalence of cardiovascular risk factors (p > 0.05). When patients were classified according to presence or absence of digital ulcers, as a sign of microangiopathy severity, the former showed higher Hcy levels than the latter (p = 0.035). Our results indicate that both primary and secondary RP patients show a mild increase in Hcy levels, which is not related to age, vitamin deficiencies or impaired renal function, but is related to microangiopathy severity. Therefore the association of HHcy and RP suggest that Hcy may contribute to endothelial dysregulation, which characterizes this disease. Specific studies should be designed to elucidate the pathogenesis of HHcy in these patients. FAU - Vayá, Amparo AU - Vayá A AD - Hemorheology and Hemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain. FAU - Sánchez, Fernando AU - Sánchez F AD - Hemorheology and Hemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain. FAU - Todolí, Jose AU - Todolí J AD - Internal Medicine Service, La Fe University Hospital, Valencia, Spain. FAU - Calvo, Javier AU - Calvo J AD - Rheumatology Service, General Hospital, Valencia, Spain. FAU - Alis, Rafael AU - Alis R AD - School of Medicine, Catholic University of Valencia, Spain. FAU - Collado, Susana AU - Collado S AD - Hemorheology and Hemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain. FAU - Ricart, Jose M AU - Ricart JM AD - Dermatology Service, La Fe University Hospital, Valencia, Spain. LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 0LVT1QZ0BA (Homocysteine) RN - 935E97BOY8 (Folic Acid) RN - P6YC3EG204 (Vitamin B 12) SB - IM MH - Adult MH - Aged MH - Capillaries/pathology MH - Female MH - Folic Acid/blood MH - Homocysteine/*blood MH - Humans MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/*blood/complications/pathology MH - Vitamin B 12/blood MH - Young Adult OTO - NOTNLM OT - Raynaud's phenomenon OT - homocysteine OT - microangiopathy EDAT- 2013/02/21 06:00 MHDA- 2014/11/13 06:00 CRDT- 2013/02/21 06:00 PHST- 2013/02/21 06:00 [entrez] PHST- 2013/02/21 06:00 [pubmed] PHST- 2014/11/13 06:00 [medline] AID - 935W808243336Q46 [pii] AID - 10.3233/CH-131681 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2014;56(2):153-9. doi: 10.3233/CH-131681. PMID- 32597543 OWN - NLM STAT- MEDLINE DCOM- 20210709 LR - 20210709 IS - 1097-0274 (Electronic) IS - 0271-3586 (Linking) VI - 63 IP - 9 DP - 2020 Sep TI - Blood biomarkers for vibration-induced white fingers. A case-comparison study. PG - 779-786 LID - 10.1002/ajim.23148 [doi] AB - BACKGROUND: Vibration induced white fingers (VWF) is one form of secondary Raynaud's phenomenon (RP). METHODS: Vibration exposed workers with RP and vibration exposed controls without RP participated. Blood samples were collected before and after cold challenge exposure (COP). The concentration of von Willebrand factor (vonWf), thrombomodulin (TM), serotonin (SER), endothelin-1 (ET(1) ), calcitonin gene-related peptide, or thromboxane A(2) was calculated. The diagnostic usefulness of the substances for ruling in the diagnosis of Raynaud's was evaluated. RESULTS: The cases showed a significant lower concentration of vonWf before and after COP, a significant increase of ET(1)  and a decrease of TM after COP. The diagnostic usefulness of vonWf showed a likelihood of defining a true case by 35%. CONCLUSIONS: vonWf, TM, SER, or ET(1) are suggested biomarkers for VWF. Diagnostic evaluation of vonWf showed a likelihood of defining a true case by 35% in the diagnosis of RP related to vibration. CI - © 2020 Wiley Periodicals LLC. FAU - Eriksson, Kåre AU - Eriksson K AUID- ORCID: 0000-0003-0323-1683 AD - Department of Sustainable Health, Umeå University, Umeå, Sweden. FAU - Burström, Lage AU - Burström L AD - Department of Sustainable Health, Umeå University, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AD - Department of Sustainable Health, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200629 PL - United States TA - Am J Ind Med JT - American journal of industrial medicine JID - 8101110 RN - 0 (Biomarkers) RN - 0 (Endothelin-1) RN - 0 (Thrombomodulin) RN - 0 (von Willebrand Factor) RN - 333DO1RDJY (Serotonin) RN - 57576-52-0 (Thromboxane A2) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Biomarkers/blood MH - Calcitonin Gene-Related Peptide/blood MH - Case-Control Studies MH - Endothelin-1/blood MH - Female MH - Fingers/pathology MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*diagnosis/etiology MH - Occupational Exposure/*adverse effects MH - Raynaud Disease/*diagnosis/etiology MH - Serotonin/blood MH - Thrombomodulin/blood MH - Thromboxane A2/blood MH - Vibration/*adverse effects MH - von Willebrand Factor/analysis OTO - NOTNLM OT - Raynaud's OT - biomarker OT - challenge OT - cold OT - diagnostic Likelihood ratio OT - fingers OT - vibrations OT - white EDAT- 2020/07/01 06:00 MHDA- 2021/07/10 06:00 CRDT- 2020/06/30 06:00 PHST- 2020/02/11 00:00 [received] PHST- 2020/06/10 00:00 [revised] PHST- 2020/06/17 00:00 [accepted] PHST- 2020/07/01 06:00 [pubmed] PHST- 2021/07/10 06:00 [medline] PHST- 2020/06/30 06:00 [entrez] AID - 10.1002/ajim.23148 [doi] PST - ppublish SO - Am J Ind Med. 2020 Sep;63(9):779-786. doi: 10.1002/ajim.23148. Epub 2020 Jun 29. PMID- 31524993 OWN - NLM STAT- MEDLINE DCOM- 20191121 LR - 20191121 IS - 1545-9616 (Print) IS - 1545-9616 (Linking) VI - 18 IP - 9 DP - 2019 Sep 1 TI - Botulinum Toxin in the Long-Term Treatment of Refractory Raynaud’s Phenomenon. PG - 943-945 LID - S1545961619P0943X [pii] AB - Raynaud’s phenomenon is an exaggerated physiological response of blood vessels in the distal extremities to emotional stress and cold. It can be idiopathic or secondary to a connective tissue disorder, such as scleroderma or systemic lupus erythematosus. Treatment for Raynaud’s phenomenon consists primarily of lifestyle modifications; if unsuccessful, pharmacotherapy with dihydropyridine calcium channel blockers can be added. Botulinum toxin (BTX-A) is a neurotoxic protein produced by Clostridium botulinum spores. While most widely known for its cosmetic use, BTX-A has many therapeutic utilities due to its ability to inhibit multiple neurotransmitters. In this report, we present a patient with Raynaud’s phenomenon refractory to standard therapies whose symptoms resolved after treatment with BTX-A. Follow-up with the patient after one and five years showed no relapse or recurrence of symptoms. J Drugs Dermatol. 2019;18(9):943-945. FAU - Asad, Usman AU - Asad U FAU - Austin, Brett AU - Austin B FAU - Tarbox, Michelle AU - Tarbox M FAU - Paulger, Brent AU - Paulger B LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Drugs Dermatol JT - Journal of drugs in dermatology : JDD JID - 101160020 RN - 0 (Neurotoxins) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) MH - Botulinum Toxins, Type A/*administration & dosage MH - Drug Resistance MH - Female MH - Fingers MH - Humans MH - Injections, Subcutaneous MH - Necrosis/drug therapy/etiology MH - Neurotoxins/*administration & dosage MH - Raynaud Disease/complications/*drug therapy MH - Skin/drug effects/*pathology MH - Time Factors MH - Treatment Outcome MH - Young Adult EDAT- 2019/09/17 06:00 MHDA- 2019/11/22 06:00 CRDT- 2019/09/17 06:00 PHST- 2019/09/17 06:00 [entrez] PHST- 2019/09/17 06:00 [pubmed] PHST- 2019/11/22 06:00 [medline] AID - S1545961619P0943X [pii] PST - ppublish SO - J Drugs Dermatol. 2019 Sep 1;18(9):943-945. PMID- 24622281 OWN - NLM STAT- MEDLINE DCOM- 20141118 LR - 20140326 IS - 1361-6579 (Electronic) IS - 0967-3334 (Linking) VI - 35 IP - 4 DP - 2014 Apr TI - Differential diagnosis of Raynaud's phenomenon based on modeling of finger thermoregulation. PG - 703-16 LID - 10.1088/0967-3334/35/4/703 [doi] AB - Raynaud's phenomenon (RP) is a vasospastic disorder of small arteries, pre-capillary arteries, and cutaneous arteriovenous shunts of the extremities, typically induced by cold exposure and emotional stress. RP is either primary (PRP) or secondary to connective tissue diseases such as systemic sclerosis (SSc). Early differential diagnosis is crucial in order to set the proper therapeutic strategy. To this goal, thermal infrared imaging data from 18 healthy controls (HCs) and 48 RP patients (20 PRP, 28 SSc) were processed through a model for a second-order time-invariant system with exponential critically damped dynamic response. Subject classification on the basis of the model parameters provides 100% true-positive discrimination for RP patients (PRP and SSc) and healthy, and 90% of correct classification within the group of patients. The proposed method may provide useful hints for early differential diagnosis in the assessment of RP disease. FAU - Ismail, E AU - Ismail E AD - Department of Neuroscience and Imaging and ITAB-Institute of Advanced Biomedical Technologies, University 'G. d'Annunzio'-Chieti-Pescara, Italy. FAU - Orlando, G AU - Orlando G FAU - Corradini, M L AU - Corradini ML FAU - Amerio, P AU - Amerio P FAU - Romani, G L AU - Romani GL FAU - Merla, A AU - Merla A LA - eng PT - Journal Article DEP - 20140312 PL - England TA - Physiol Meas JT - Physiological measurement JID - 9306921 SB - IM MH - *Body Temperature Regulation MH - Demography MH - Diagnosis, Differential MH - Female MH - Fingers/blood supply/*physiopathology MH - Humans MH - Male MH - Middle Aged MH - *Models, Biological MH - ROC Curve MH - Raynaud Disease/*diagnosis/*physiopathology MH - Temperature MH - Time Factors EDAT- 2014/03/14 06:00 MHDA- 2014/11/19 06:00 CRDT- 2014/03/14 06:00 PHST- 2014/03/14 06:00 [entrez] PHST- 2014/03/14 06:00 [pubmed] PHST- 2014/11/19 06:00 [medline] AID - 10.1088/0967-3334/35/4/703 [doi] PST - ppublish SO - Physiol Meas. 2014 Apr;35(4):703-16. doi: 10.1088/0967-3334/35/4/703. Epub 2014 Mar 12. PMID- 24458685 OWN - NLM STAT- MEDLINE DCOM- 20140515 LR - 20161128 IS - 0041-4131 (Print) IS - 0041-4131 (Linking) VI - 91 IP - 12 DP - 2013 Dec TI - [Raynaud's syndrome revealing hypereosinophilic syndrome]. PG - 742-5 FAU - Said, Fatma AU - Said F FAU - Kooli, Chakib AU - Kooli C FAU - Azzabi, Samira AU - Azzabi S FAU - Boukhris, Imen AU - Boukhris I FAU - Cherif, Eya AU - Cherif E FAU - Kaouech, Zouleikha AU - Kaouech Z FAU - Belhassine, Lamia AU - Belhassine L FAU - Khalfallah, Narjess AU - Khalfallah N LA - fre PT - Case Reports PT - Letter TT - Un syndrome de Raynaud révélant un syndrome hypéréosinophilique. PL - Tunisia TA - Tunis Med JT - La Tunisie medicale JID - 0413766 SB - IM MH - Adult MH - Angiography MH - Diagnosis, Differential MH - Fingers/blood supply/diagnostic imaging MH - Humans MH - Hypereosinophilic Syndrome/*complications/*diagnosis MH - Leg/blood supply/diagnostic imaging MH - Male MH - Raynaud Disease/*complications/diagnosis EDAT- 2014/01/25 06:00 MHDA- 2014/05/16 06:00 CRDT- 2014/01/25 06:00 PHST- 2014/01/25 06:00 [entrez] PHST- 2014/01/25 06:00 [pubmed] PHST- 2014/05/16 06:00 [medline] AID - /article-medicale-tunisie.php?article=2343 [pii] PST - ppublish SO - Tunis Med. 2013 Dec;91(12):742-5. PMID- 21961408 OWN - NLM STAT- MEDLINE DCOM- 20111103 LR - 20191027 IS - 0095-6562 (Print) IS - 0095-6562 (Linking) VI - 82 IP - 10 DP - 2011 Oct TI - You're the flight surgeon: Raynaud's disease. PG - 1002-3 FAU - Pack, Craig R AU - Pack CR LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Aviat Space Environ Med JT - Aviation, space, and environmental medicine JID - 7501714 SB - IM MH - Adult MH - Aerospace Medicine MH - Guidelines as Topic MH - Humans MH - Male MH - *Military Personnel MH - Raynaud Disease/*diagnosis MH - United States MH - *Work Capacity Evaluation EDAT- 2011/10/04 06:00 MHDA- 2011/11/04 06:00 CRDT- 2011/10/04 06:00 PHST- 2011/10/04 06:00 [entrez] PHST- 2011/10/04 06:00 [pubmed] PHST- 2011/11/04 06:00 [medline] AID - 10.3357/asem.3054.2011 [doi] PST - ppublish SO - Aviat Space Environ Med. 2011 Oct;82(10):1002-3. doi: 10.3357/asem.3054.2011. PMID- 26356403 OWN - NLM STAT- MEDLINE DCOM- 20151106 LR - 20181023 IS - 1997-7298 (Print) IS - 1997-7298 (Linking) VI - 115 IP - 6 DP - 2015 TI - [Chronic cerebral ischemia associated with Raynaud's syndrome]. PG - 90-96 LID - 10.17116/jnevro20151156190-96 [doi] AB - Over the last years, a number of patients with chronic cerebral ischemia has been increased significantly. Compensatory possibilities of the brain and cerebral circulatory system are so great that even serious disturbances of blood circulation could not cause clinical signs of brain dysfunction for a long time. At the same time, long-term ischemia can lead to peripheral local disturbances of microcirculation that is appears to be a first signal of the problems with homeostasis. Therefore, Raynaud's syndrome may be one of the predictors of standard symptoms of chronic cerebral ischemia (CCI). This phenomenon is explicitly considered as a sign of blood circulation impairment while the pathogenetic mechanism of vascular arterial bed instability is completely ignored. Detailed study of clinical correlations of Raynaud's syndrome in CCI would help to develop a common pharmacotherapeutic approach to its treatment. FAU - Putilina, M V AU - Putilina MV AD - Pirogov Russian National Research Medical University, Moscow. LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Zh Nevrol Psikhiatr Im S S Korsakova JT - Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova JID - 9712194 SB - IM MH - Brain Ischemia/*diagnosis/physiopathology MH - Chronic Disease MH - Female MH - Humans MH - Male MH - Microcirculation MH - Raynaud Disease/*diagnosis/physiopathology EDAT- 2015/09/12 06:00 MHDA- 2015/11/07 06:00 CRDT- 2015/09/11 06:00 PHST- 2015/09/11 06:00 [entrez] PHST- 2015/09/12 06:00 [pubmed] PHST- 2015/11/07 06:00 [medline] AID - 10.17116/jnevro20151156190-96 [doi] PST - ppublish SO - Zh Nevrol Psikhiatr Im S S Korsakova. 2015;115(6):90-96. doi: 10.17116/jnevro20151156190-96. PMID- 26966965 OWN - NLM STAT- MEDLINE DCOM- 20160526 LR - 20160312 IS - 0885-1158 (Print) IS - 0885-1158 (Linking) VI - 31 IP - 1 DP - 2016 Mar TI - Raynaud's Phenomenon in a Slap Bass Player: A Case Report. PG - 51-3 LID - 10.21091/mppa.2016.1009 [doi] AB - OBJECTIVE: Secondary Raynaud's phenomenon is a frequent condition related to occupational exposure to local vibration but has not been described in musicians. This study aims to describe cold-induced blanching of the right second and (in particular) third digits in a 67-year-old double bass player following decades of cumulative repetitive blunt trauma to the fingers from slapping the strings. METHODS: A physical examination was undertaken and systolic blood pressure measured before and after cold provocation. RESULTS: At 10 deg C the brachial systolic blood pressure was 156 mm Hg while blood pressure was immeasurable at the finger level, corresponding to a finger/brachial index of 0% of the second and third fingers. CONCLUSION: This is the first reported case of objectively verified, playing-related Raynaud's phenomenon in a musician. FAU - Jepsen, Jørgen Riis AU - Jepsen JR AD - Dep. of Occupational Medicine, Hospital of Southwestern Jutland, Østergade 81-83, DK-6700 Esbjerg, Denmark. Tel +45 23285702. joergen.riis.jepsen@svs.regionsyd danmark.dk. FAU - Simonsen, Jane Angel AU - Simonsen JA LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Med Probl Perform Art JT - Medical problems of performing artists JID - 9416269 SB - IM MH - Aged MH - Humans MH - Male MH - *Music MH - Occupational Diseases/*diagnosis/etiology MH - Occupational Exposure/*adverse effects MH - Raynaud Disease/*diagnosis/etiology MH - Vibration/*adverse effects EDAT- 2016/03/12 06:00 MHDA- 2016/05/27 06:00 CRDT- 2016/03/12 06:00 PHST- 2016/03/12 06:00 [entrez] PHST- 2016/03/12 06:00 [pubmed] PHST- 2016/05/27 06:00 [medline] AID - 10.21091/mppa.2016.1009 [doi] PST - ppublish SO - Med Probl Perform Art. 2016 Mar;31(1):51-3. doi: 10.21091/mppa.2016.1009. PMID- 34845840 OWN - NLM STAT- MEDLINE DCOM- 20220228 LR - 20240222 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 25 IP - 2 DP - 2022 Feb TI - What are the effects of vasodilators in patients with primary Raynaud's phenomenon? A Cochrane review summary with commentary. PG - 232-235 LID - 10.1111/1756-185X.14243 [doi] FAU - Coskun Benlidayi, Ilke AU - Coskun Benlidayi I AUID- ORCID: 0000-0001-6517-5969 AD - Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Cukurova University, Adana, Turkey. LA - eng PT - Comment PT - Letter DEP - 20211129 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Vasodilator Agents) SB - IM CON - Cochrane Database Syst Rev. 2021 May 17;5:CD006687. doi: 10.1002/14651858.CD006687.pub4. PMID: 33998674 MH - Humans MH - *Raynaud Disease/diagnosis/drug therapy MH - *Vasodilator Agents/adverse effects OTO - NOTNLM OT - Cochrane review summary OT - Raynaud’s phenomenon OT - therapeutics OT - vasodilator agents OT - vasodilators EDAT- 2021/12/01 06:00 MHDA- 2022/03/01 06:00 CRDT- 2021/11/30 07:10 PHST- 2021/11/04 00:00 [revised] PHST- 2021/11/01 00:00 [received] PHST- 2021/11/05 00:00 [accepted] PHST- 2021/12/01 06:00 [pubmed] PHST- 2022/03/01 06:00 [medline] PHST- 2021/11/30 07:10 [entrez] AID - 10.1111/1756-185X.14243 [doi] PST - ppublish SO - Int J Rheum Dis. 2022 Feb;25(2):232-235. doi: 10.1111/1756-185X.14243. Epub 2021 Nov 29. PMID- 34651332 OWN - NLM STAT- MEDLINE DCOM- 20220107 LR - 20220107 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 49 IP - 1 DP - 2022 Jan TI - Multiple digital ulcers on the hands and feet in anti-PL-12 antibody-positive anti-synthetase syndrome without Raynaud's phenomenon. PG - e28-e29 LID - 10.1111/1346-8138.16198 [doi] FAU - Hattori, Shoko AU - Hattori S AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. FAU - Miyagawa, Fumi AU - Miyagawa F AUID- ORCID: 0000-0002-2030-6969 AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. FAU - Mitsui, Yasuhiro AU - Mitsui Y AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. FAU - Nakashima, Ran AU - Nakashima R AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Sasai, Tsuneo AU - Sasai T AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Mimori, Tsuneyo AU - Mimori T AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. AD - Ijinkai Takeda General Hospital, Kyoto, Japan. FAU - Asada, Hideo AU - Asada H AUID- ORCID: 0000-0003-1971-9835 AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. LA - eng PT - Letter DEP - 20211014 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Antibodies, Antinuclear) RN - EC 6.- (Ligases) SB - IM MH - Antibodies, Antinuclear MH - Hand MH - Humans MH - Ligases MH - *Raynaud Disease/diagnosis MH - *Ulcer EDAT- 2021/10/16 06:00 MHDA- 2022/01/08 06:00 CRDT- 2021/10/15 06:49 PHST- 2021/09/28 00:00 [revised] PHST- 2021/09/01 00:00 [received] PHST- 2021/10/04 00:00 [accepted] PHST- 2021/10/16 06:00 [pubmed] PHST- 2022/01/08 06:00 [medline] PHST- 2021/10/15 06:49 [entrez] AID - 10.1111/1346-8138.16198 [doi] PST - ppublish SO - J Dermatol. 2022 Jan;49(1):e28-e29. doi: 10.1111/1346-8138.16198. Epub 2021 Oct 14. PMID- 19321043 OWN - NLM STAT- MEDLINE DCOM- 20090625 LR - 20151119 IS - 0393-974X (Print) IS - 0393-974X (Linking) VI - 23 IP - 1 DP - 2009 Jan-Mar TI - Plasma adrenomedullin and endothelin-1 levels are reduced and Raynaud's phenomenon improved by daily tadalafil administration in male patients with systemic sclerosis. PG - 23-9 AB - The aim of our study is to evaluate in Systemic Sclerosis (SSc) male patients the tadalafil effects on Raynaud's phenomenon and on AM and ET-1 plasma levels. In an open-label study 20 consecutive male patients with SSc were enrolled and received 10 mg of tadalafil daily for 12 weeks. The primary endpoint was the subjective reduction of frequency and duration of Raynaud's attacks measured with a 10-point Raynaud's Condition Score; the secondary aim was to modify Adrenomedullin (AM) and Endothelin-1 (ET-1) plasma levels. After the treatment Raynaud's phenomenon was improved by once-daily tadalafil (decrease of mean number of Raynaud's attacks and of Raynaud's Condition Score) and plasma AM and ET-1 levels decreased. The results of our study lead us to postulate the beneficial effect of adding long term inhibition of Phosphodiesterase type 5 to Systemic Sclerosis' therapy. FAU - Rosato, E AU - Rosato E AD - Department of Clinical Immunology and Allergy, Sapienza University of Rome, Rome, Italy. FAU - Letizia, C AU - Letizia C FAU - Proietti, M AU - Proietti M FAU - Aversa, A AU - Aversa A FAU - Menghi, G AU - Menghi G FAU - Rossi, C AU - Rossi C FAU - Torella, E AU - Torella E FAU - Cotesta, D AU - Cotesta D FAU - Petramala, L AU - Petramala L FAU - Bruzziches, R AU - Bruzziches R FAU - Spera, G AU - Spera G FAU - Pisarri, S AU - Pisarri S FAU - Salsano, F AU - Salsano F LA - eng PT - Clinical Trial PT - Journal Article PL - Singapore TA - J Biol Regul Homeost Agents JT - Journal of biological regulators and homeostatic agents JID - 8809253 RN - 0 (Carbolines) RN - 0 (Endothelin-1) RN - 0 (Phosphodiesterase Inhibitors) RN - 148498-78-6 (Adrenomedullin) RN - 742SXX0ICT (Tadalafil) SB - IM MH - Adrenomedullin/*blood MH - Adult MH - Carbolines/*administration & dosage/*therapeutic use MH - Drug Administration Schedule MH - Endothelin-1/*blood MH - Female MH - Humans MH - Male MH - Phosphodiesterase Inhibitors/administration & dosage/therapeutic use MH - Raynaud Disease/blood/*complications/*drug therapy MH - Scleroderma, Systemic/blood/*complications/drug therapy MH - Tadalafil EDAT- 2009/03/27 09:00 MHDA- 2009/06/26 09:00 CRDT- 2009/03/27 09:00 PHST- 2009/03/27 09:00 [entrez] PHST- 2009/03/27 09:00 [pubmed] PHST- 2009/06/26 09:00 [medline] AID - 4 [pii] PST - ppublish SO - J Biol Regul Homeost Agents. 2009 Jan-Mar;23(1):23-9. PMID- 22880344 OWN - NLM STAT- MEDLINE DCOM- 20120911 LR - 20120813 IS - 0029-6570 (Print) IS - 0029-6570 (Linking) VI - 26 IP - 46 DP - 2012 Jul 18-24 TI - Diagnosis and management of patients with Raynaud's phenomenon. PG - 41-6 AB - This article describes the characteristics of Raynaud's phenomenon, focusing on the role of the specialist nurse in diagnosis and management of the condition. Pharmacological and non-pharmacological treatment options are discussed, along with the importance of self-management. Advice is provided to help nurses enable patients to minimise episodes and improve symptoms. In the majority of cases, Raynaud's phenomenon is a treatable condition, and patients can learn to self-manage the disease. FAU - Brown, Susan AU - Brown S AD - Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Bath. sue.brown@rnhrd.nhs.uk LA - eng PT - Journal Article PL - England TA - Nurs Stand JT - Nursing standard (Royal College of Nursing (Great Britain) : 1987) JID - 9012906 RN - 0 (Cardiovascular Agents) MH - Cardiovascular Agents/administration & dosage/adverse effects/therapeutic use MH - Diagnostic Techniques, Cardiovascular MH - Humans MH - Physical Examination MH - Raynaud Disease/*diagnosis/*therapy MH - Self Care EDAT- 2012/08/14 06:00 MHDA- 2012/09/12 06:00 CRDT- 2012/08/14 06:00 PHST- 2012/08/14 06:00 [entrez] PHST- 2012/08/14 06:00 [pubmed] PHST- 2012/09/12 06:00 [medline] AID - 10.7748/ns2012.07.26.46.41.c9214 [doi] PST - ppublish SO - Nurs Stand. 2012 Jul 18-24;26(46):41-6. doi: 10.7748/ns2012.07.26.46.41.c9214. PMID- 19760147 OWN - NLM STAT- MEDLINE DCOM- 20100407 LR - 20151119 IS - 1573-9686 (Electronic) IS - 0090-6964 (Linking) VI - 37 IP - 12 DP - 2009 Dec TI - Finger thermoregulatory model assessing functional impairment in Raynaud's phenomenon. PG - 2631-9 LID - 10.1007/s10439-009-9788-9 [doi] AB - Raynaud's Phenomenon (RP) is a paroxysmal vasospastic disorder of small arteries, pre-capillary arteries, and cutaneous arteriovenous shunts of the extremities, typically induced by cold exposure and emotional stress. RP is either primary (PRP) or secondary to systemic sclerosis. In this study we use Control System Theory to model finger thermoregulatory processes in response to a standardized cold challenge (a diagnostic test routinely performed for differential diagnosis of RP). The proposed model is based on a homeostatic negative feedback loop, characterized by five distinct parameters which describe how the control mechanisms are activated and maintained. Thermal infrared imaging data from 14 systemic sclerosis subjects (SSc), 14 PRP, and 16 healthy control subjects (HCS) were processed. HCS presented the fastest active recovery, with the highest gain. PRP presented the slowest and weakest recovery, mostly due to passive heat exchange with the environment. SSc presented an intermediate behavior, with the longest delay of response onset. The estimated model parameters elucidated the level of functional impairment expressed in the various forms of this disease. FAU - Mariotti, Alessandro AU - Mariotti A AD - Department of Clinical Sciences and Bioimaging, "G. d'Annunzio" University, Chieti, Italy. FAU - Grossi, Giuliana AU - Grossi G FAU - Amerio, Paolo AU - Amerio P FAU - Orlando, Giuseppe AU - Orlando G FAU - Mattei, Peter A AU - Mattei PA FAU - Tulli, Antonio AU - Tulli A FAU - Romani, Gian Luca AU - Romani GL FAU - Merla, Arcangelo AU - Merla A LA - eng PT - Journal Article DEP - 20090904 PL - United States TA - Ann Biomed Eng JT - Annals of biomedical engineering JID - 0361512 SB - IM MH - *Body Temperature Regulation MH - Cold Temperature MH - Computer Simulation MH - Diagnosis, Computer-Assisted/*methods MH - Fingers/*physiopathology MH - Humans MH - Middle Aged MH - *Models, Biological MH - Raynaud Disease/*diagnosis/*therapy MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Thermography/*methods EDAT- 2009/09/18 06:00 MHDA- 2010/04/08 06:00 CRDT- 2009/09/18 06:00 PHST- 2009/04/08 00:00 [received] PHST- 2009/08/27 00:00 [accepted] PHST- 2009/09/18 06:00 [entrez] PHST- 2009/09/18 06:00 [pubmed] PHST- 2010/04/08 06:00 [medline] AID - 10.1007/s10439-009-9788-9 [doi] PST - ppublish SO - Ann Biomed Eng. 2009 Dec;37(12):2631-9. doi: 10.1007/s10439-009-9788-9. Epub 2009 Sep 4. PMID- 28358168 OWN - NLM STAT- MEDLINE DCOM- 20171212 LR - 20260127 IS - 1651-2057 (Electronic) IS - 0001-5555 (Linking) VI - 97 IP - 7 DP - 2017 Jul 6 TI - Efficacy of Botulinum Toxin B Injection for Raynaud's Phenomenon and Digital Ulcers in Patients with Systemic Sclerosis. PG - 843-850 LID - 10.2340/00015555-2665 [doi] AB - The efficacy and safety of botulinum toxin B (BTX-B) for treatment of Raynaud's phenomenon and digital ulcers in patients with systemic sclerosis was assessed. A total of 45 patients with systemic sclerosis who had Raynaud's phenomenon were blinded and divided randomly into 4 groups: a no-treatment control group, and 3 treatment groups, using 250, 1,000 or 2,000 international units (U) of BTX-B injections in the hand with more severe symptoms. Four weeks after injection, pain/numbness visual analogue scale scores and Raynaud's score in the groups treated with 1,000 and 2,000 U BTX-B were significantly lower than in the control group and the group treated with 250 U BTX-B. These beneficial effects were sustained until 16 weeks after the single injection. At 4 weeks after injection skin temperature recovery in the group treated with 2,000 U BTX-B was significantly improved. The numbers of digital ulcers in the groups treated with 1,000 and 2,000 U BTX-B were significantly lower than in the control group. In conclusion, 1,000 and 2,000 U BTX-B injections significantly suppressed the activity of Raynaud's phenomenon and digital ulcers in patients with SSc without serious adverse events. FAU - Motegi, Sei-Ichiro AU - Motegi SI AD - Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan. smotegi@gunma-u.ac.jp. FAU - Uehara, Akihito AU - Uehara A FAU - Yamada, Kazuya AU - Yamada K FAU - Sekiguchi, Akiko AU - Sekiguchi A FAU - Fujiwara, Chisako AU - Fujiwara C FAU - Toki, Sayaka AU - Toki S FAU - Date, Yuki AU - Date Y FAU - Nakamura, Tetsuya AU - Nakamura T FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article PT - Randomized Controlled Trial PL - Sweden TA - Acta Derm Venereol JT - Acta dermato-venereologica JID - 0370310 RN - 0 (Acetylcholine Release Inhibitors) RN - 0Y70779M1F (rimabotulinumtoxinB) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Acetylcholine Release Inhibitors/*administration & dosage/adverse effects MH - Aged MH - Botulinum Toxins, Type A/*administration & dosage/adverse effects MH - Female MH - Humans MH - Injections MH - Japan MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/diagnosis/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/*complications MH - Single-Blind Method MH - Skin Temperature/drug effects MH - Skin Ulcer/diagnosis/*drug therapy/etiology/physiopathology MH - Time Factors MH - Treatment Outcome MH - Wound Healing/drug effects EDAT- 2017/03/31 06:00 MHDA- 2017/12/13 06:00 CRDT- 2017/03/31 06:00 PHST- 2017/03/31 06:00 [pubmed] PHST- 2017/12/13 06:00 [medline] PHST- 2017/03/31 06:00 [entrez] AID - 10.2340/00015555-2665 [doi] PST - ppublish SO - Acta Derm Venereol. 2017 Jul 6;97(7):843-850. doi: 10.2340/00015555-2665. PMID- 24344159 OWN - NLM STAT- MEDLINE DCOM- 20140530 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 73 IP - 5 DP - 2014 May TI - Fibrosis biomarkers in isolated Raynaud's phenomenon: too little, too soon? PG - 940-1 LID - 10.1136/annrheumdis-2013-204009 [doi] FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Clinical Science & Community Health, Division of Rheumatology, Istituto Gaetano Pini, University of Milano, , Milano, Italy. FAU - Gualtierotti, Roberta AU - Gualtierotti R FAU - Schioppo, Tommaso AU - Schioppo T FAU - Orenti, Annalisa AU - Orenti A FAU - Boracchi, Patrizia AU - Boracchi P FAU - Lubatti, Chiara AU - Lubatti C FAU - Mastaglio, Claudio AU - Mastaglio C FAU - Galbiati, Valentina AU - Galbiati V FAU - Murgo, Antonella AU - Murgo A FAU - Zeni, Silvana AU - Zeni S FAU - Grossi, Claudia AU - Grossi C FAU - Borghi, Orietta AU - Borghi O FAU - Rosenberg, William AU - Rosenberg W FAU - Castelnovo, Laura AU - Castelnovo L FAU - Meroni Pier, Luigi AU - Meroni Pier L LA - eng PT - Journal Article DEP - 20131216 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Biomarkers) SB - IM MH - Adult MH - Aged MH - *Algorithms MH - Biomarkers/*blood MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Liver Cirrhosis/blood/*diagnosis/etiology MH - Male MH - Middle Aged MH - Raynaud Disease/blood/complications/*diagnosis MH - Scleroderma, Systemic/blood/*diagnosis OTO - NOTNLM OT - Autoantibodies OT - Autoimmune Diseases OT - Systemic Sclerosis EDAT- 2013/12/18 06:00 MHDA- 2014/05/31 06:00 CRDT- 2013/12/18 06:00 PHST- 2013/12/18 06:00 [entrez] PHST- 2013/12/18 06:00 [pubmed] PHST- 2014/05/31 06:00 [medline] AID - S0003-4967(24)14976-X [pii] AID - 10.1136/annrheumdis-2013-204009 [doi] PST - ppublish SO - Ann Rheum Dis. 2014 May;73(5):940-1. doi: 10.1136/annrheumdis-2013-204009. Epub 2013 Dec 16. PMID- 22089468 OWN - NLM STAT- MEDLINE DCOM- 20120501 LR - 20120102 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 39 IP - 1 DP - 2012 Jan TI - Clinical significance of Cryofibrinogenemia: possible pathophysiological link with Raynaud's phenomenon. PG - 119-24 LID - 10.3899/jrheum.110793 [doi] AB - OBJECTIVE: To describe the clinical findings and prevalence of patients with cryofibrinogenemia (CF) and to determine whether CF is associated with primary Raynaud's phenomenon. METHODS: Between June 2006 and December 2009, 227 patients were tested for CF in a single university hospital. Forty-five patients with primary Raynaud's phenomenon were tested for CF. RESULTS: A total of 117 patients with CF without cryoglobulinemia were included. The main clinical manifestations included skin manifestations (50%) and arthralgia (35%). There were 67 patients with primary CF and 50 patients with secondary CF. There was no significant difference in the mean concentration of the cryoprecipitate in primary CF as compared to the secondary form (172 ± 18.6 vs 192 ± 20.9 mg/dl, respectively; p = 0.41). Highest concentrations of cryoprecipitate were observed in those containing fibrinogen only as compared to cryoprecipitates containing fibrinogen and fibronectin (301 ± 43.5 vs 125 ± 10.6 mg/dl; p < 0.001). Patients having skin necrosis (n = 3) had significantly higher values of cryofibrinogen compared to those without necrosis (638 ± 105 vs 160 ± 10.2 mg/dl; p = 0.0046). Among the 45 patients with primary Raynaud's phenomenon, 36 had associated CF. There was no significant difference in the mean concentration of the cryoprecipitate in these patients compared to those with primary CF. CONCLUSION: There seems to be a significant correlation between cryofibrinogen concentration and the severity of the clinical signs, particularly when cryoprecipitate is composed of fibrinogen alone. CF might have a possible pathophysiological role in primary Raynaud's phenomenon. FAU - Soyfoo, Muhammad S AU - Soyfoo MS AD - Department of Rheumatology, Erasme Hospital, Université Libre de Bruxelles, 808 Route de Lennik, 1070 Brussels, Belgium. msoyfoo@ulb.ac.be FAU - Goubella, Ahmed AU - Goubella A FAU - Cogan, Elie AU - Cogan E FAU - Wautrecht, Jean-Claude AU - Wautrecht JC FAU - Ocmant, Annick AU - Ocmant A FAU - Stordeur, Patrick AU - Stordeur P LA - eng PT - Journal Article DEP - 20111115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Fibronectins) RN - 9001-32-5 (Fibrinogen) RN - Cryofibrinogenemia SB - IM MH - Cryoglobulinemia/pathology/*physiopathology MH - Female MH - Fibrinogen/metabolism MH - Fibronectins/blood MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*blood/pathology/*physiopathology MH - Retrospective Studies EDAT- 2011/11/18 06:00 MHDA- 2012/05/02 06:00 CRDT- 2011/11/18 06:00 PHST- 2011/11/18 06:00 [entrez] PHST- 2011/11/18 06:00 [pubmed] PHST- 2012/05/02 06:00 [medline] AID - jrheum.110793 [pii] AID - 10.3899/jrheum.110793 [doi] PST - ppublish SO - J Rheumatol. 2012 Jan;39(1):119-24. doi: 10.3899/jrheum.110793. Epub 2011 Nov 15. PMID- 30639703 OWN - NLM STAT- MEDLINE DCOM- 20190624 LR - 20190624 IS - 1469-0691 (Electronic) IS - 1198-743X (Linking) VI - 25 IP - 3 DP - 2019 Mar TI - Helicobacter cinaedi associated with atypical Raynaud syndrome. PG - 324-325 LID - S1198-743X(19)30005-9 [pii] LID - 10.1016/j.cmi.2019.01.001 [doi] FAU - Beauruelle, C AU - Beauruelle C AD - Département de Bactériologie-Virologie, Hygiène et Parasitologie-Mycologie, Centre Hospitalier Régional Universitaire (CHRU) de Brest, Brest, France; UMR1078 "Génétique, Génomique Fonctionnelle et Biotechnologies", INSERM, Université de Brest, EFS, IBSAM, Brest, France. Electronic address: clemence.beauruelle@chu-brest.fr. FAU - Le Bars, H AU - Le Bars H AD - Département de Bactériologie-Virologie, Hygiène et Parasitologie-Mycologie, Centre Hospitalier Régional Universitaire (CHRU) de Brest, Brest, France. FAU - Astruc, N AU - Astruc N AD - Service de Médecine Interne et Pneumologie, CHRU Brest, Brest, France. FAU - Tandé, D AU - Tandé D AD - Département de Bactériologie-Virologie, Hygiène et Parasitologie-Mycologie, Centre Hospitalier Régional Universitaire (CHRU) de Brest, Brest, France. FAU - Le Berre, R AU - Le Berre R AD - UMR1078 "Génétique, Génomique Fonctionnelle et Biotechnologies", INSERM, Université de Brest, EFS, IBSAM, Brest, France; Service de Médecine Interne et Pneumologie, CHRU Brest, Brest, France. FAU - Héry-Arnaud, G AU - Héry-Arnaud G AD - Département de Bactériologie-Virologie, Hygiène et Parasitologie-Mycologie, Centre Hospitalier Régional Universitaire (CHRU) de Brest, Brest, France; UMR1078 "Génétique, Génomique Fonctionnelle et Biotechnologies", INSERM, Université de Brest, EFS, IBSAM, Brest, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20190111 PL - England TA - Clin Microbiol Infect JT - Clinical microbiology and infection : the official publication of the European Society of Clinical Microbiology and Infectious Diseases JID - 9516420 RN - 0 (Anti-Bacterial Agents) SB - IM MH - Adult MH - Anti-Bacterial Agents/therapeutic use MH - Female MH - Helicobacter/drug effects/*isolation & purification MH - Helicobacter Infections/*diagnosis/drug therapy MH - Humans MH - Immunocompetence MH - Raynaud Disease/complications/*microbiology MH - Treatment Outcome OTO - NOTNLM OT - Arteriosclerosis OT - Helicobacter OT - Raynaud's disease EDAT- 2019/01/15 06:00 MHDA- 2019/06/25 06:00 CRDT- 2019/01/15 06:00 PHST- 2018/11/09 00:00 [received] PHST- 2018/12/31 00:00 [revised] PHST- 2019/01/03 00:00 [accepted] PHST- 2019/01/15 06:00 [pubmed] PHST- 2019/06/25 06:00 [medline] PHST- 2019/01/15 06:00 [entrez] AID - S1198-743X(19)30005-9 [pii] AID - 10.1016/j.cmi.2019.01.001 [doi] PST - ppublish SO - Clin Microbiol Infect. 2019 Mar;25(3):324-325. doi: 10.1016/j.cmi.2019.01.001. Epub 2019 Jan 11. PMID- 23609604 OWN - NLM STAT- MEDLINE DCOM- 20150811 LR - 20140415 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 56 IP - 3 DP - 2014 TI - Hemorheological profile in primary and secondary Raynaud's phenomenon. Influence of microangiopathy. PG - 259-64 LID - 10.3233/CH-131723 [doi] AB - Raynaud's phenomenon (RP) is an episodic peripheral circulatory disorder characterized by local artery spams in subjects exposed to cold or emotional stress. It is not well-established whether RP patients show an altered rheological profile, mostly due to patient classification and clinical severity. We aimed to compare the hemorheological profile in patients with primary and secondary RP with a healthy control group. Eighteen primary RP, 22 secondary RP and 22 healthy controls, were included in the study. RP patients were also divided according to the presence of digital ulcers (7 with, 33 without). Biochemical and hemorheological variables were analyzed, including glucose, triglycerides, total-cholesterol, immunoglobulins, fibrinogen, plasma viscosity, erythrocyte aggregation, erythrocyte deformability and blood viscosity. Age was higher in secondary RP as compared with primary (p = 0.049), while glucose, triglycerides IgA, IgG and plasma viscosity were higher in secondary RP than in healthy subjects (p < 0.05). RP patients with digital ulcers presented higher IgA (p = 0.012), lower erythrocyte aggregation time (p = 0.008) and a trend for higher fibrinogen levels and plasma viscosity (p = 0.064, p = 0.069, respectively). The results of the present study indicate that secondary RP patients show a mild impairment of the rheological profile that aggravates with microangiopathy severity. FAU - Vayá, Amparo AU - Vayá A AD - Hemorheology and Hemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain. FAU - Alis, Rafael AU - Alis R AD - Universitary Research Institute "Dr. Viña Giner", Molecular and Mitochondrial Medicine, Catholic University of Valencia, "San Vicente Mártir", Valencia, Spain. FAU - Romagnoli, Marco AU - Romagnoli M AD - Universitary Research Institute "Dr. Viña Giner", Molecular and Mitochondrial Medicine, Catholic University of Valencia, "San Vicente Mártir", Valencia, Spain Department of Physical Education and Sports, Catholic University of Valencia, "San Vicente Mártir", Valencia, Spain. FAU - Todolí, Jose AU - Todolí J AD - Internal Medicine Service, La Fe University Hospital, Valencia, Spain. FAU - Calvo, Javier AU - Calvo J AD - Rheumatology Service, General Hospital, Valencia, Spain. FAU - Ricart, Jose M AU - Ricart JM AD - Dermatology Service, La Fe University Hospital, Valencia, Spain. LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 SB - IM MH - Adult MH - Aged MH - Blood Viscosity MH - Female MH - Hemorheology/*physiology MH - Humans MH - Middle Aged MH - Raynaud Disease/*blood/diagnosis MH - Young Adult OTO - NOTNLM OT - Raynaud's phenomenon OT - digital ulcers OT - hemorheology EDAT- 2013/04/24 06:00 MHDA- 2015/08/12 06:00 CRDT- 2013/04/24 06:00 PHST- 2013/04/24 06:00 [entrez] PHST- 2013/04/24 06:00 [pubmed] PHST- 2015/08/12 06:00 [medline] AID - JL3686M847324352 [pii] AID - 10.3233/CH-131723 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2014;56(3):259-64. doi: 10.3233/CH-131723. PMID- 26173902 OWN - NLM STAT- MEDLINE DCOM- 20170123 LR - 20221207 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 43 IP - 1 DP - 2016 Jan TI - Beneficial effect of botulinum toxin A on Raynaud's phenomenon in Japanese patients with systemic sclerosis: A prospective, case series study. PG - 56-62 LID - 10.1111/1346-8138.13030 [doi] AB - Currently, there is no satisfactory treatment for Raynaud's phenomenon (RP) in systemic sclerosis (SSc). Recently, it has been reported that botulinum toxin A (BTX-A) injection was effective for the treatment of RP in SSc patients. The objective was to assess the efficacy and safety of BTX-A on RP in Japanese SSc patients. In the prospective, case series study, 10 Japanese SSc patients with RP received 10 U of BTX-A injections into the hand. The change in severity of RP, including the frequency of attacks/pain, color changes, duration time of RP and the severity of pain, was assessed by Raynaud's score and pain visual analog scale (VAS) at each visit during 16 weeks. The recovery of skin temperature 20 min after cold water stimulation was examined by thermography at baseline and 4 weeks after injection. The number of digital ulcers (DU) and adverse effects were assessed at each visit. BTX-A injection decreased Raynaud's score and pain VAS from 2 weeks after injection, and the suppressive effect was continued until 16 weeks after injection. Skin temperature recovery after cold water stimulation at 4 weeks after injection was significantly enhanced compared with that before injection. All DU in five patients were healed within 12 weeks after injection. Neither systemic nor local adverse effects were observed in all cases. We conclude that BTX-A injection significantly improved the activity of RP in SSc patients without any adverse events, suggesting that BTX-A may have possible long-term preventive and therapeutic potentials for RP in Japanese SSc patients. CI - © 2015 Japanese Dermatological Association. FAU - Motegi, Sei-ichiro AU - Motegi S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Yamada, Kazuya AU - Yamada K AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Toki, Sayaka AU - Toki S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uchiyama, Akihiko AU - Uchiyama A AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Kubota, Yuka AU - Kubota Y AD - Clinical Investigation and Research Unit, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Nakamura, Tetsuya AU - Nakamura T AD - Clinical Investigation and Research Unit, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Ishikawa, Osamu AU - Ishikawa O AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150715 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Aged MH - Asian People MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain/drug therapy MH - Prospective Studies MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/complications/*drug therapy MH - Skin Temperature/drug effects MH - Skin Ulcer/drug therapy/etiology OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin A OT - digital ulcer OT - systemic sclerosis EDAT- 2015/07/16 06:00 MHDA- 2017/01/24 06:00 CRDT- 2015/07/16 06:00 PHST- 2015/06/04 00:00 [received] PHST- 2015/06/11 00:00 [accepted] PHST- 2015/07/16 06:00 [entrez] PHST- 2015/07/16 06:00 [pubmed] PHST- 2017/01/24 06:00 [medline] AID - 10.1111/1346-8138.13030 [doi] PST - ppublish SO - J Dermatol. 2016 Jan;43(1):56-62. doi: 10.1111/1346-8138.13030. Epub 2015 Jul 15. PMID- 41923505 OWN - NLM STAT- MEDLINE DCOM- 20260402 LR - 20260402 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 22 IP - 956 DP - 2026 Apr 1 TI - [The Role of Nailfold Capillaroscopy in Raynaud's Phenomenon and Autoimmune Diseases]. PG - 622-626 LID - 10.53738/REVMED.2026.22.956.48523 [doi] AB - Connective tissue diseases are systemic autoimmune disorders for which diagnosis is often delayed owing to nonspecific early clinical manifestations. However, Raynaud's phenomenon is a common and early symptom that prompts etiological evaluation. In this context, nailfold capillaroscopy plays a pivotal role. It enables the detection of characteristic microvascular abnormalities, particularly the scleroderma pattern, which is strongly associated with systemic sclerosis and included in both classification and early diagnostic criteria. This review outlines the principles of capillaroscopy interpretation, its diagnostic and predictive value in systemic sclerosis, and its complementary role in other connective tissue diseases, even in the absence of acrosyndrome. FAU - Della Vedova, Ludovico AU - Della Vedova L AD - Service d'immunologie et allergie, Centre hospitalier universitaire vaudois, 1011 Lausanne. FAU - Porceddu, Enrica AU - Porceddu E AUID- ORCID: 0000-0002-6354-3980 AD - Service d'angiologie, Centre hospitalier universitaire vaudois, 1011 Lausanne. FAU - Arlettaz, Lionel AU - Arlettaz L AUID- ORCID: 0009-0003-2863-1103 AD - Service d'immunologie et allergie, Centre hospitalier universitaire vaudois, 1011 Lausanne. AD - Service d'immunologie et allergie, Institut central des hôpitaux, Hôpital du Valais, 1950 Sion. FAU - Allali, Daniéle AU - Allali D AD - Service d'allergologie et immunologie, Hôpital de la Tour, 1217 Meyrin/Genève. FAU - Mazzolai, Lucia AU - Mazzolai L AD - Service d'angiologie, Centre hospitalier universitaire vaudois, 1011 Lausanne. FAU - Ribi, Camillo AU - Ribi C AD - Service d'immunologie et allergie, Centre hospitalier universitaire vaudois, 1011 Lausanne. FAU - Horisberger, Alice AU - Horisberger A AUID- ORCID: 0000-0002-3732-8662 AD - Service d'immunologie et allergie, Centre hospitalier universitaire vaudois, 1011 Lausanne. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Rôle de la capillaroscopie dans le phénomène de Raynaud et les maladies auto-immunes. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - *Raynaud Disease/diagnosis/etiology MH - *Autoimmune Diseases/diagnosis MH - Scleroderma, Systemic/diagnosis MH - *Nails/blood supply MH - Predictive Value of Tests MH - Connective Tissue Diseases/diagnosis COIS- les auteurs n’ont déclaré aucun conflit d’intérêts en lien avec cet article. EDAT- 2026/04/02 06:30 MHDA- 2026/04/02 06:31 CRDT- 2026/04/02 03:23 PHST- 2026/04/02 06:31 [medline] PHST- 2026/04/02 06:30 [pubmed] PHST- 2026/04/02 03:23 [entrez] AID - RMS0956-005 [pii] AID - 10.53738/REVMED.2026.22.956.48523 [doi] PST - ppublish SO - Rev Med Suisse. 2026 Apr 1;22(956):622-626. doi: 10.53738/REVMED.2026.22.956.48523. PMID- 24369360 OWN - NLM STAT- MEDLINE DCOM- 20150529 LR - 20220410 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 39 IP - 8 DP - 2014 Oct TI - A prospective study of the use of botulinum toxin injections in the treatment of Raynaud's syndrome associated with scleroderma. PG - 876-80 LID - 10.1177/1753193413516242 [doi] AB - Raynaud's syndrome contributes to the pain, paraesthesia, ulceration, and gangrene of scleroderma. Botulinum toxin has been shown to improve digital perfusion in patients with Raynaud's. This is the first study to objectively assess hand function following this treatment in patients with scleroderma. Twenty patients were treated with 100 units of botulinum toxin injected into the hand. An assessment of hand function and symptoms was performed prior to injection and then 8-12 weeks later. The outcomes assessed were change in pain, appearance, cold intolerance, pinch and power grip, ranges of movement, and Disabilities of the Arm, Shoulder and Hand (DASH) score. In total, 80% of patients reported an overall improvement in their symptoms, reduction in pain, and improved DASH score and 65% reported improvement in cold intolerance. Overall, 90% showed an improvement in pinch grip and 65% an improvement in power grip. Objective parameters were statistically significantly improved; however, subjective outcomes only showed a trend. We have found botulinum toxin to be an effective treatment for Raynaud's syndrome secondary to scleroderma. CI - © The Author(s) 2013. FAU - Uppal, L AU - Uppal L AD - Department of Plastic and Reconstructive Surgery, Royal Free Hospital, London, UK lauren.uppal@me.com. FAU - Dhaliwal, K AU - Dhaliwal K AD - Department of Plastic and Reconstructive Surgery, Royal Free Hospital, London, UK. FAU - Butler, P E AU - Butler PE AD - Department of Plastic and Reconstructive Surgery, Royal Free Hospital, London, UK. LA - eng PT - Journal Article DEP - 20131224 PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Botulinum Toxins, Type A/*therapeutic use MH - Disability Evaluation MH - Female MH - Hand Strength MH - Humans MH - Injections, Intramuscular MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Prospective Studies MH - Range of Motion, Articular MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications MH - Visual Analog Scale MH - Young Adult OTO - NOTNLM OT - Botulinum toxin OT - Raynaud’s OT - scleroderma OT - sympathectomy EDAT- 2013/12/27 06:00 MHDA- 2015/05/30 06:00 CRDT- 2013/12/27 06:00 PHST- 2013/12/27 06:00 [entrez] PHST- 2013/12/27 06:00 [pubmed] PHST- 2015/05/30 06:00 [medline] AID - 1753193413516242 [pii] AID - 10.1177/1753193413516242 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2014 Oct;39(8):876-80. doi: 10.1177/1753193413516242. Epub 2013 Dec 24. PMID- 17473568 OWN - NLM STAT- MEDLINE DCOM- 20070703 LR - 20070502 IS - 0957-5235 (Print) IS - 0957-5235 (Linking) VI - 18 IP - 4 DP - 2007 Jun TI - Increased circulating platelet-leucocyte complexes in patients with primary Raynaud's phenomenon and Raynaud's phenomenon secondary to systemic sclerosis: a comparative study. PG - 297-302 AB - Platelet activation and circulating platelet-leucocyte complexes increase in vascular ischemic events and autoimmune inflammatory diseases. Platelet activation markers and platelet-leucocyte complexes were evaluated in primary Raynaud's phenomenon (RP) and in RP secondary to systemic sclerosis (SSc). Whole-blood flow cytometry was utilized to quantify CD62P, platelet microparticles (PMP), platelet-monocyte complexes (PMC) and platelet-neutrophil complexes (PNC) in primary RP and in SSc patients with secondary RP. SSc patients with secondary RP had significantly higher platelet CD62P expression than primary RP patients and controls (P = 0.017 and 0.004, respectively). Primary and secondary RP patients had higher mean PMC and PNC levels than controls (all P < or = 0.001). PMP level in SSc patients with pulmonary hypertension was significantly higher than in others (P = 0.048). All parameters were similar in SSc patients with and without digital ulcers, aspirin-users and nonusers (P > 0.05). CD62P level decreased significantly after iloprost administration in four patients with digital ulcers (16.1 +/- 17.4 vs 7.4 +/- 3.8%, P = 0.03). Our results suggest there is platelet-leucocyte complex formation in RP, and, despite antithrombotic therapy, platelet activation and platelet-leucocyte interaction are ongoing in SSc. This is important as it might have potential therapeutic implications with respect to using antiplatelet drugs in SSc. FAU - Pamuk, Gülsüm Emel AU - Pamuk GE AD - Department of Hematology, Trakya University Medical Faculty, Edirne, Turkey. gepamuk@yahoo.com FAU - Turgut, Burhan AU - Turgut B FAU - Pamuk, Omer Nuri AU - Pamuk ON FAU - Vural, Ozden AU - Vural O FAU - Demir, Muzaffer AU - Demir M FAU - Cakir, Necati AU - Cakir N LA - eng PT - Comparative Study PT - Journal Article PL - England TA - Blood Coagul Fibrinolysis JT - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JID - 9102551 RN - 0 (P-Selectin) SB - IM MH - Adult MH - Female MH - Flow Cytometry MH - Humans MH - Leukocytes/metabolism MH - Male MH - Middle Aged MH - P-Selectin/*metabolism MH - Platelet Activation/*physiology MH - Raynaud Disease/*blood/complications/physiopathology MH - Scleroderma, Systemic/*blood/complications/physiopathology EDAT- 2007/05/03 09:00 MHDA- 2007/07/04 09:00 CRDT- 2007/05/03 09:00 PHST- 2007/05/03 09:00 [pubmed] PHST- 2007/07/04 09:00 [medline] PHST- 2007/05/03 09:00 [entrez] AID - 00001721-200706000-00001 [pii] AID - 10.1097/MBC.0b013e328010bd05 [doi] PST - ppublish SO - Blood Coagul Fibrinolysis. 2007 Jun;18(4):297-302. doi: 10.1097/MBC.0b013e328010bd05. PMID- 17478872 OWN - NLM STAT- MEDLINE DCOM- 20070614 LR - 20070504 IS - 0890-3344 (Print) IS - 0890-3344 (Linking) VI - 23 IP - 2 DP - 2007 May TI - Raynaud's phenomenon of the nipples: an elusive diagnosis. PG - 191-3 AB - Pain and throbbing of the nipples associated with Raynaud's phenomenon often mimics yeast or fungal infections. Breastfeeding mothers with Raynaud's of the nipples are often treated inappropriately for organisms such as Candida Albicans with topical or systemic antifungal agents. This case report describes the eventual diagnosis of Raynaud's phenomenon of the nipples in a breastfeeding mother who was initially treated for yeast. FAU - Morino, Carolyn AU - Morino C AD - Saint Luke's South Hospital, Overland Park, KS 66213, USA. FAU - Winn, Susan M AU - Winn SM LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Hum Lact JT - Journal of human lactation : official journal of International Lactation Consultant Association JID - 8709498 MH - Adult MH - Candida albicans/isolation & purification MH - Candidiasis/diagnosis/pathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Nipples/microbiology/*pathology MH - Raynaud Disease/*diagnosis/pathology EDAT- 2007/05/05 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/05/05 09:00 PHST- 2007/05/05 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/05/05 09:00 [entrez] AID - 23/2/191 [pii] AID - 10.1177/0890334407300018 [doi] PST - ppublish SO - J Hum Lact. 2007 May;23(2):191-3. doi: 10.1177/0890334407300018. PMID- 25817568 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1440-0960 (Electronic) IS - 0004-8380 (Linking) VI - 56 IP - 3 DP - 2015 Aug TI - Clinical and image improvement of Raynaud's phenomenon after botulinum toxin type A treatment. PG - 202-5 LID - 10.1111/ajd.12326 [doi] AB - Raynaud's phenomenon is often accompanied by pain, digital ulceration and compromised daily activities. Pharmacological therapy or sympathectomies have been administered to diminish these symptoms but existing treatments are not invariably efficacious. A recent case series has described the use of botulinum toxin type A in the treatment of Raynaud's phenomenon. We report two patients with severe or mild Raynaud's phenomenon who were injected with BTX-A; both of whom experienced clinical and image improvement after treatment. CI - © 2015 The Australasian College of Dermatologists. FAU - Zhao, HongMei AU - Zhao H AD - Department of Neurology, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China. FAU - Lian, YaJun AU - Lian Y AD - Department of Neurology, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20150329 PL - Australia TA - Australas J Dermatol JT - The Australasian journal of dermatology JID - 0135232 RN - 0 (Acetylcholine Release Inhibitors) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Acetylcholine Release Inhibitors/*therapeutic use MH - Adult MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Humans MH - Magnetic Resonance Angiography MH - Raynaud Disease/diagnostic imaging/*drug therapy OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin type A EDAT- 2015/03/31 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/03/31 06:00 PHST- 2015/01/06 00:00 [received] PHST- 2015/02/11 00:00 [accepted] PHST- 2015/03/31 06:00 [entrez] PHST- 2015/03/31 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1111/ajd.12326 [doi] PST - ppublish SO - Australas J Dermatol. 2015 Aug;56(3):202-5. doi: 10.1111/ajd.12326. Epub 2015 Mar 29. PMID- 20199852 OWN - NLM STAT- MEDLINE DCOM- 20100727 LR - 20100423 IS - 1873-6513 (Electronic) IS - 0885-3924 (Linking) VI - 39 IP - 4 DP - 2010 Apr TI - Paraneoplastic Raynaud's phenomenon--good palliation after a multidisciplinary approach. PG - 779-83 LID - 10.1016/j.jpainsymman.2009.09.006 [doi] AB - Paraneoplastic Raynaud's phenomenon is a rare complication of a number of different malignancies (carcinomas, sarcomas, lymphomas, and leukemias). We present a case of paraneoplastic Raynaud's phenomenon in a patient with non-small-cell lung cancer that was associated with significant morbidity, involved a multidisciplinary approach, and eventually responded to a specialized intervention (i.e., iloprost trometamol). CI - Copyright (c) 2010 U.S. Cancer Pain Relief Committee. Published by Elsevier Inc. All rights reserved. FAU - Schildmann, Eva K AU - Schildmann EK AD - Department of Hematology, Helios Klinikum Berlin-Buch, Berlin, Germany. eva.schildmann@gmx.net FAU - Davies, Andrew N AU - Davies AN LA - eng PT - Case Reports PT - Journal Article DEP - 20100303 PL - United States TA - J Pain Symptom Manage JT - Journal of pain and symptom management JID - 8605836 SB - IM MH - Aged MH - Carcinoma, Non-Small-Cell Lung/diagnosis/*therapy MH - Humans MH - Lung Neoplasms/diagnosis/*therapy MH - Male MH - Pain Clinics MH - Palliative Care/*methods MH - Paraneoplastic Syndromes/diagnosis/*therapy MH - Raynaud Disease/diagnosis/*therapy MH - Treatment Outcome EDAT- 2010/03/05 06:00 MHDA- 2010/07/28 06:00 CRDT- 2010/03/05 06:00 PHST- 2008/09/30 00:00 [received] PHST- 2009/08/30 00:00 [revised] PHST- 2009/09/28 00:00 [accepted] PHST- 2010/03/05 06:00 [entrez] PHST- 2010/03/05 06:00 [pubmed] PHST- 2010/07/28 06:00 [medline] AID - S0885-3924(10)00079-5 [pii] AID - 10.1016/j.jpainsymman.2009.09.006 [doi] PST - ppublish SO - J Pain Symptom Manage. 2010 Apr;39(4):779-83. doi: 10.1016/j.jpainsymman.2009.09.006. Epub 2010 Mar 3. PMID- 26458705 OWN - NLM STAT- MEDLINE DCOM- 20160825 LR - 20151013 IS - 1998-4138 (Electronic) IS - 1998-4138 (Linking) VI - 11 IP - 3 DP - 2015 Jul-Sep TI - Raynaud's phenomenon in a child with medulloblastoma as a late effect of chemotherapy. PG - 666 LID - 10.4103/0973-1482.144649 [doi] AB - There are a lot of early or late side effects of chemotherapies. One of them is Raynaud's phenomenon (RP). Vascular toxicity associated with antineoplastic agents is notified in bleomycin alone therapy or in combination with cisplatin, vinblastine, and vincristine. The mechanism of RP associated with antineoplastic agents is unknown. All children receiving vinblastine, vincristine, bleomycin and cisplatin therapy, are followed and questioned about their complaint on RP. Long-term follow-up of surviving patients is recommended. Oncologists should be aware of the potential late toxic effects of antineoplastic drugs. FAU - Atas, Erman AU - Atas E AD - Department of Pediatric Oncology, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Korkmazer, Nadir AU - Korkmazer N FAU - Artik, Hatice A AU - Artik HA FAU - Babacan, Oguzhan AU - Babacan O FAU - Kesik, Vural AU - Kesik V LA - eng PT - Case Reports PT - Letter PL - India TA - J Cancer Res Ther JT - Journal of cancer research and therapeutics JID - 101249598 RN - 5J49Q6B70F (Vincristine) RN - 7BRF0Z81KG (Lomustine) RN - Q20Q21Q62J (Cisplatin) SB - IM MH - Adolescent MH - Antineoplastic Combined Chemotherapy Protocols/*adverse effects/therapeutic use MH - Cerebellar Neoplasms/*diagnosis/therapy MH - Chemoradiotherapy MH - Cisplatin/administration & dosage MH - Female MH - Fingers/blood supply MH - Humans MH - Lomustine/administration & dosage MH - Medulloblastoma/*diagnosis/therapy MH - Raynaud Disease/chemically induced/*diagnosis MH - Toes/blood supply MH - Vincristine/administration & dosage EDAT- 2015/10/16 06:00 MHDA- 2016/08/26 06:00 CRDT- 2015/10/14 06:00 PHST- 2015/10/14 06:00 [entrez] PHST- 2015/10/16 06:00 [pubmed] PHST- 2016/08/26 06:00 [medline] AID - JCanResTher_2015_11_3_666_144649 [pii] AID - 10.4103/0973-1482.144649 [doi] PST - ppublish SO - J Cancer Res Ther. 2015 Jul-Sep;11(3):666. doi: 10.4103/0973-1482.144649. PMID- 21816024 OWN - NLM STAT- MEDLINE DCOM- 20111201 LR - 20191210 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 14 IP - 3 DP - 2011 Aug TI - Increased urotensin-II activity in patients with Raynaud's phenomenon and systemic lupus erythematosus. PG - 276-81 LID - 10.1111/j.1756-185X.2011.01597.x [doi] AB - AIM: Raynaud's phenomenon (RP) commonly co-exists with systemic lupus erythematosus (SLE). The obvious pathophysiological mechanism in RP is vasoconstriction. Although the roles of certain vasoconstrictor substances, like endothelin-1, have been identified in RP, underlying mechanisms remain unclear. METHODS: In this pilot study, we researched a relatively recently identified, very potent vasoconstrictor peptide, urotensin-II (U-II), in SLE patients versus those without RP. In addition to its vasoconstrictor effect, U-II has been implicated in cardiovascular events and atherosclerosis. Increased frequencies of atherosclerosis and cardiovascular events comprise another issue in SLE patients. To address these effects, we included 15 Raynaud's (+) and 15 Raynaud's (-) SLE patients and compared both cohorts against age and sex-matched controls. RESULTS: We found significantly elevated U-II activity in both RP (+) and RP (-) SLE patients, relative to controls (P < 0.0001); however, the difference among RP (+) SLE patients was more prominent. U-II was significantly elevated in RP (+) SLE patients when compared to RP (-) SLE patients (P < 0.001). CONCLUSIONS: The results of our study suggest that, either as a cause or by-product, U-II may have some role in Raynaud's-related vasoconstriction. It also might contribute to atherosclerosis and cardiovascular diseases in SLE patients. Further studies clearly are warranted. CI - © 2011 The Authors. International Journal of Rheumatic Diseases © 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd. FAU - Buyukhatipoglu, Hakan AU - Buyukhatipoglu H AD - Department of Internal Medicine, Harran University School of Medicine, Gaziantep, Turkey. buyukhatipoglu@gmail.com FAU - Buyukaslan, Hasan AU - Buyukaslan H FAU - Pehlivan, Yavuz AU - Pehlivan Y FAU - Ceylan, Nurdan AU - Ceylan N FAU - Ulas, Turgay AU - Ulas T FAU - Tarakcioglu, Mehmet AU - Tarakcioglu M FAU - Onat, Ahmet M AU - Onat AM LA - eng PT - Comparative Study PT - Journal Article PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Biomarkers) RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (Peptide Hormones) RN - 0 (UTS2B protein, human) SB - IM MH - Adult MH - Biomarkers/blood MH - Comorbidity MH - Female MH - Humans MH - Intracellular Signaling Peptides and Proteins MH - Lupus Erythematosus, Systemic/*blood/epidemiology/physiopathology MH - Male MH - Peptide Hormones/*blood MH - Pilot Projects MH - Raynaud Disease/*blood/epidemiology/physiopathology MH - Turkey/epidemiology EDAT- 2011/08/06 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/08/06 06:00 PHST- 2011/08/06 06:00 [entrez] PHST- 2011/08/06 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 10.1111/j.1756-185X.2011.01597.x [doi] PST - ppublish SO - Int J Rheum Dis. 2011 Aug;14(3):276-81. doi: 10.1111/j.1756-185X.2011.01597.x. PMID- 26101120 OWN - NLM STAT- MEDLINE DCOM- 20150831 LR - 20150623 IS - 1175-8716 (Electronic) IS - 0028-8446 (Linking) VI - 128 IP - 1413 DP - 2015 May 1 TI - Medical image. Primary Raynaud's phenomenon. PG - 69-70 FAU - Beyong, Taso AU - Beyong T AD - Internal Medicine, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Mawdiangdiang, Shillong 793018, India. beyongtaso@gmail.com. FAU - Ete, Tony AU - Ete T FAU - Warjri, Synrang Batngen AU - Warjri SB FAU - Barman, Bhupen AU - Barman B LA - eng PT - Case Reports PT - Journal Article DEP - 20150501 PL - New Zealand TA - N Z Med J JT - The New Zealand medical journal JID - 0401067 SB - IM MH - *Cold Temperature MH - Female MH - Humans MH - Raynaud Disease/*diagnosis MH - Young Adult EDAT- 2015/06/24 06:00 MHDA- 2015/09/01 06:00 CRDT- 2015/06/24 06:00 PHST- 2015/06/24 06:00 [entrez] PHST- 2015/06/24 06:00 [pubmed] PHST- 2015/09/01 06:00 [medline] PST - epublish SO - N Z Med J. 2015 May 1;128(1413):69-70. PMID- 31838167 OWN - NLM STAT- MEDLINE DCOM- 20200227 LR - 20200227 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 19 IP - 2 DP - 2020 Feb TI - Thermography in systemic sclerosis patients and other rheumatic diseases: Diagnosis, disease activity assessment, and therapeutic monitoring. PG - 102449 LID - S1568-9972(19)30262-9 [pii] LID - 10.1016/j.autrev.2019.102449 [doi] FAU - Sciascia, Savino AU - Sciascia S AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy. FAU - Cecchi, Irene AU - Cecchi I AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy; School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Italy. FAU - Massara, Carlo AU - Massara C AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy. FAU - Rossi, Daniela AU - Rossi D AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy. FAU - Radin, Massimo AU - Radin M AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy; School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Italy. FAU - Ladehesa, Pineda Lourdes AU - Ladehesa PL AD - Department of Rheumatology, Reina Sofía University Hospital, Maimonides Biomedical Research Institute of Córdoba (IMIBIC), University of Córdoba, Córdoba, Spain. FAU - Guiñazu, Francisco AU - Guiñazu F AD - Rheumatology, Hospital of Córdoba, Córdoba, Argentina. FAU - Rubini, Elena AU - Rubini E AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy; School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Italy. FAU - Foddai, Silvia Grazietta AU - Foddai SG AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy; School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Italy. FAU - Alba, Paula AU - Alba P AD - Rheumatology, Hospital of Córdoba, Córdoba, Argentina. FAU - Escudero, Alejandro AU - Escudero A AD - Department of Rheumatology, Reina Sofía University Hospital, Maimonides Biomedical Research Institute of Córdoba (IMIBIC), University of Córdoba, Córdoba, Spain. FAU - Menegatti, Elisa AU - Menegatti E AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy; School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Italy. FAU - Roccatello, Dario AU - Roccatello D AD - Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy. Electronic address: dario.roccatello@unito.it. LA - eng PT - Letter PT - Review DEP - 20191212 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Arthritis/diagnostic imaging/therapy MH - Humans MH - Lupus Erythematosus, Systemic/diagnostic imaging/therapy MH - Raynaud Disease/diagnosis/therapy MH - Rheumatic Diseases/*diagnostic imaging/*therapy MH - Scleroderma, Systemic/*diagnostic imaging/*therapy MH - *Thermography MH - Treatment Outcome OTO - NOTNLM OT - Autoimmune disease OT - Imaging OT - Raynaud's phenomenon OT - Rheumatic disease OT - Systemic sclerosis OT - Thermography EDAT- 2019/12/16 06:00 MHDA- 2020/02/28 06:00 CRDT- 2019/12/16 06:00 PHST- 2019/08/29 00:00 [received] PHST- 2019/09/01 00:00 [accepted] PHST- 2019/12/16 06:00 [pubmed] PHST- 2020/02/28 06:00 [medline] PHST- 2019/12/16 06:00 [entrez] AID - S1568-9972(19)30262-9 [pii] AID - 10.1016/j.autrev.2019.102449 [doi] PST - ppublish SO - Autoimmun Rev. 2020 Feb;19(2):102449. doi: 10.1016/j.autrev.2019.102449. Epub 2019 Dec 12. PMID- 19690939 OWN - NLM STAT- MEDLINE DCOM- 20100330 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 12 DP - 2009 Dec TI - The treatment with N-acetylcysteine of Raynaud's phenomenon and ischemic ulcers therapy in sclerodermic patients: a prospective observational study of 50 patients. PG - 1379-84 LID - 10.1007/s10067-009-1251-7 [doi] AB - N-Acetylcysteine is useful in the short-term treatment of severe Raynaud's phenomenon and digital ulcers (DU) in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 3 years) in a prospective study of a cohort of 50 consecutive patients with SSc who received N-acetylcysteine (NAC) infusional therapy every 2 weeks. We observed a reduction of DU/patient/year (4.5 +/- 3.1 vs 0.81 +/- 0.79) and DU ulcer visual analog scale (VAS; 6.88 +/- 2.62 vs 3.20 +/- 1.80), a decrease of the Raynaud's phenomenon (RP) number attacks (7.18 +/- 3.87 vs 3 +/- 1.92), and RP VAS (6.24 +/- 1.92 vs 3.62 +/- 1.48). In this study, we did not observe serious adverse events in patients. Minor side effects were flushing (two patients) and headache (one patient). NAC infusion was generally well tolerated, and nobody had to discontinue the treatment. In conclusion, long-term therapy with NAC, in patients with SSc, has a durable effectiveness on ischemic ulcers and Raynaud's phenomenon. FAU - Rosato, Edoardo AU - Rosato E AD - Clinical Immunology Unit-Scleroderma Center, Department of Clinical Medicine, Sapienza University of Rome, Viale dell'Università 37, 00185 Rome, Italy. FAU - Borghese, Federica AU - Borghese F FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article DEP - 20090820 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Free Radical Scavengers) RN - WYQ7N0BPYC (Acetylcysteine) SB - IM MH - Acetylcysteine/administration & dosage/*therapeutic use MH - Female MH - Fingers MH - Free Radical Scavengers/administration & dosage/*therapeutic use MH - Humans MH - Infusions, Intravenous MH - Ischemia/complications/*drug therapy/pathology MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*drug therapy/pathology MH - Scleroderma, Systemic/complications/*drug therapy/pathology MH - Skin/blood supply MH - Skin Ulcer/complications/*drug therapy/pathology MH - Treatment Outcome EDAT- 2009/08/20 09:00 MHDA- 2010/03/31 06:00 CRDT- 2009/08/20 09:00 PHST- 2009/03/26 00:00 [received] PHST- 2009/08/05 00:00 [accepted] PHST- 2009/07/03 00:00 [revised] PHST- 2009/08/20 09:00 [entrez] PHST- 2009/08/20 09:00 [pubmed] PHST- 2010/03/31 06:00 [medline] AID - 10.1007/s10067-009-1251-7 [doi] PST - ppublish SO - Clin Rheumatol. 2009 Dec;28(12):1379-84. doi: 10.1007/s10067-009-1251-7. Epub 2009 Aug 20. PMID- 31619252 OWN - NLM STAT- MEDLINE DCOM- 20200408 LR - 20231014 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 17 IP - 1 DP - 2019 Oct 16 TI - Infrared thermography in children: a reliable tool for differential diagnosis of peripheral microvascular dysfunction and Raynaud's phenomenon? PG - 68 LID - 10.1186/s12969-019-0371-0 [doi] LID - 68 AB - BACKGROUND: Infrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is available on its use in children for this purpose. The first aim of the study was to assess reproducibility of thermographic examination after cold exposure by comparing inter-observer agreement in thermal imaging interpretation. The secondary aim was to evaluate whether IRT is reliable to diagnose and differentiate peripheral circulation disturbances in children. METHODS: Children with clinical diagnosis of primary Raynaud's phenomenon (PRP), secondary RP (SRP), acrocyanosis (AC) and age-matched controls underwent sequential measurements of skin temperature at distal interphalangeal (DIP) and metacarpophalangeal (MCP) joints with IRT at baseline and for 10 min after cold challenge test. Intraclass correlation coefficient (ICC) was calculated for inter-rater reliability in IRT interpretation, then temperature variations at MCP and DIP joints and the distal-dorsal difference (DDD) were analysed. RESULTS: Fourteen PRP, 16 SRP, 14 AC and 15 controls entered the study. ICC showed excellent agreement (> 0.93) for DIPs and MCPs in 192 measures for each subject. Patients with PRP, SRP and acrocyanosis showed significantly slower recovery at MCPs (p < 0.05) and at DIPs (p < 0.001) than controls. At baseline, higher temperature at DIPs and lower at MCPs was observed in PRP compared with SRP with significantly lower DDD (p < 0.001). Differently from AC, both PRP and SRP showed gain of temperature at DIPs and less at MCPs after cold challenge. PRP but not SRP patients returned to DIPs basal temperature by the end of re-warming time. Analysis of DDD confirmed that controls and PRP, SRP and AC patients significantly differed in fingers recovery pattern (p < 0.05). CONCLUSION: IRT appears reliable and reproducible in identifying children with peripheral microvascular disturbances. Our results show that IRT examination pointed out that PRP, SRP and AC patients present significant differences in basal extremities temperature and in re-warming pattern after cold challenge therefore IRT can be suggested as an objective tool for diagnosis and monitoring of disease. FAU - Martini, Giorgia AU - Martini G AUID- ORCID: 0000-0003-3801-8903 AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 2, 35128, Padova, Italy. giorgia.martini@aopd.veneto.it. FAU - Cappella, Michela AU - Cappella M AD - Pediatric Unit, Santa Maria Nuova Hospital, Reggio Emilia, Italy. FAU - Culpo, Roberta AU - Culpo R AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 2, 35128, Padova, Italy. FAU - Vittadello, Fabio AU - Vittadello F AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 2, 35128, Padova, Italy. FAU - Sprocati, Monica AU - Sprocati M AD - Pediatric Unit, Sant'Anna Hospital, Ferrara, Italy. FAU - Zulian, Francesco AU - Zulian F AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 2, 35128, Padova, Italy. LA - eng PT - Journal Article DEP - 20191016 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM MH - Adolescent MH - Case-Control Studies MH - Child MH - Child, Preschool MH - Diagnosis, Differential MH - Female MH - Humans MH - Infrared Rays MH - Male MH - Microvessels/pathology MH - Peripheral Vascular Diseases/*diagnosis/pathology MH - Raynaud Disease/*diagnosis/pathology MH - Reproducibility of Results MH - Skin Temperature MH - Thermography/*methods PMC - PMC6794834 OTO - NOTNLM OT - Acrocyanosis OT - Child OT - Diagnosis OT - Infrared thermography OT - Raynaud’s phenomenon COIS- The authors declare that they have no competing interests. EDAT- 2019/10/18 06:00 MHDA- 2020/04/09 06:00 PMCR- 2019/10/16 CRDT- 2019/10/18 06:00 PHST- 2019/06/24 00:00 [received] PHST- 2019/09/24 00:00 [accepted] PHST- 2019/10/18 06:00 [entrez] PHST- 2019/10/18 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2019/10/16 00:00 [pmc-release] AID - 10.1186/s12969-019-0371-0 [pii] AID - 371 [pii] AID - 10.1186/s12969-019-0371-0 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2019 Oct 16;17(1):68. doi: 10.1186/s12969-019-0371-0. PMID- 19577729 OWN - NLM STAT- MEDLINE DCOM- 20091002 LR - 20140730 IS - 1542-2224 (Electronic) IS - 1067-2516 (Linking) VI - 48 IP - 4 DP - 2009 Jul-Aug TI - Gradual digital lengthening with autologous bone graft and external fixation for correction of flail toe in a patient with Raynaud's disease. PG - 488-94 LID - 10.1053/j.jfas.2009.02.011 [doi] AB - Iatrogenic flail toe is a complication of hammertoe surgery that occurs when an overaggressive resection of the proximal phalanx occurs. This can cause both functional and cosmetic concerns for the patient. We present a case report of the correction of a flail second toe in a patient with Raynaud's disease. The correction was achieved by means of gradual soft tissue lengthening with external fixation and an interposition autologous bone graft digital arthrodesis. After 5 months, this 2-stage procedure lengthened, stabilized, and restored the function of the toe. LEVEL OF CLINICAL EVIDENCE: 4. FAU - Lamm, Bradley M AU - Lamm BM AD - Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening, Sinai Hospital of Baltimore, Baltimore, Maryland 21215, USA. blamm@lifebridgehealth.org FAU - Ades, Joe K AU - Ades JK LA - eng PT - Case Reports PT - Journal Article DEP - 20090509 PL - United States TA - J Foot Ankle Surg JT - The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle Surgeons JID - 9308427 SB - IM MH - Adult MH - Arthroplasty/*adverse effects MH - *Bone Lengthening MH - *Bone Transplantation MH - Female MH - Hammer Toe Syndrome/*surgery MH - Humans MH - Joint Instability/etiology/surgery MH - *Orthopedic Fixation Devices MH - Raynaud Disease/*complications/physiopathology MH - Toes/*surgery MH - Vasoconstriction EDAT- 2009/07/07 09:00 MHDA- 2009/10/03 06:00 CRDT- 2009/07/07 09:00 PHST- 2009/02/19 00:00 [received] PHST- 2009/07/07 09:00 [entrez] PHST- 2009/07/07 09:00 [pubmed] PHST- 2009/10/03 06:00 [medline] AID - S1067-2516(09)00099-4 [pii] AID - 10.1053/j.jfas.2009.02.011 [doi] PST - ppublish SO - J Foot Ankle Surg. 2009 Jul-Aug;48(4):488-94. doi: 10.1053/j.jfas.2009.02.011. Epub 2009 May 9. PMID- 30712434 OWN - NLM STAT- MEDLINE DCOM- 20191023 LR - 20191023 IS - 1097-9867 (Electronic) IS - 1083-7450 (Linking) VI - 24 IP - 6 DP - 2019 Jul TI - Evaluation of topical econazole nitrate formulations with potential for treating Raynaud's phenomenon. PG - 689-699 LID - 10.1080/10837450.2019.1578371 [doi] AB - The purpose of this work was to design and characterize a topical formulation of econazole nitrate (EN) with potential for treating Raynaud's phenomenon (RP). Four topical dosage forms (F1_topical solution, F2_HPMC or hydroxypropyl methylcellulose dispersion, F3_VersaBase(®) cream, and F4(_)Lipoderm(®) Activemax™ Cream) containing 3% w/w EN were prepared and characterized for drug content, pH, viscosity, spreadability, drug crystallinity, stability, and in vitro permeation using Franz cells across pig ear skin, and results were compared to the 1% marketed EN cream. All four formulations had acceptable physical and visual characteristics required for topical application, with 3% w/w EN. The order of amount of drug permeated from highest to lowest was F2 (10.27%) > F4 (2.47%) > F1 (2.28%) > F3 (1.47%) > marketed formulation (0.22%). Formulation F2 showed better penetration of the drug into the stratum corneum, epidermis, and dermis layers. The drug concentration in the stratum corneum and epidermis was approximately 10-20 times higher with F2 compared to the marketed formulation. All formulations were found to be stable for up to 6 months. All four EN formulations were found to be better than the 1% marketed cream. Formulation F2(_)HPMC dispersion could be further explored as a treatment option for RP. FAU - Bahl, Dherya AU - Bahl D AD - a College of Pharmacy and Pharmaceutical Sciences , The University of Toledo Health Science Campus, The University of Toledo , Toledo , OH , USA. FAU - Daftardar, Saloni AU - Daftardar S AD - a College of Pharmacy and Pharmaceutical Sciences , The University of Toledo Health Science Campus, The University of Toledo , Toledo , OH , USA. FAU - Devi Bachu, Rinda AU - Devi Bachu R AD - a College of Pharmacy and Pharmaceutical Sciences , The University of Toledo Health Science Campus, The University of Toledo , Toledo , OH , USA. FAU - Boddu, Sai H S AU - Boddu SHS AD - a College of Pharmacy and Pharmaceutical Sciences , The University of Toledo Health Science Campus, The University of Toledo , Toledo , OH , USA. AD - b Department of Pharmaceutical Sciences, College of Pharmacy and Health Sciences , Ajman University , Ajman , UAE. FAU - Altorok, Nezam AU - Altorok N AD - c Department of Medicine, Division of Rheumatology , University of Toledo , Toledo , OH , USA. FAU - Kahaleh, Bashar AU - Kahaleh B AD - c Department of Medicine, Division of Rheumatology , University of Toledo , Toledo , OH , USA. LA - eng PT - Journal Article DEP - 20190426 PL - England TA - Pharm Dev Technol JT - Pharmaceutical development and technology JID - 9610932 RN - 0 (14-alpha Demethylase Inhibitors) RN - 0 (Antifungal Agents) RN - 0 (Pharmaceutical Vehicles) RN - 3NXW29V3WO (Hypromellose Derivatives) RN - 6Z1Y2V4A7M (Econazole) SB - IM MH - 14-alpha Demethylase Inhibitors/*administration & dosage/pharmacokinetics MH - Administration, Topical MH - Animals MH - Antifungal Agents/*administration & dosage/pharmacokinetics MH - Crystallization MH - Drug Compounding/methods MH - Econazole/*administration & dosage/pharmacokinetics MH - Humans MH - Hypromellose Derivatives/chemistry MH - Pharmaceutical Vehicles/*chemistry MH - Raynaud Disease/*drug therapy/metabolism MH - Skin Absorption MH - Swine OTO - NOTNLM OT - Raynaud’s phenomenon OT - TRPM-8 OT - econazole nitrate OT - permeation OT - topical EDAT- 2019/02/05 06:00 MHDA- 2019/10/24 06:00 CRDT- 2019/02/05 06:00 PHST- 2019/02/05 06:00 [pubmed] PHST- 2019/10/24 06:00 [medline] PHST- 2019/02/05 06:00 [entrez] AID - 10.1080/10837450.2019.1578371 [doi] PST - ppublish SO - Pharm Dev Technol. 2019 Jul;24(6):689-699. doi: 10.1080/10837450.2019.1578371. Epub 2019 Apr 26. PMID- 19517112 OWN - NLM STAT- MEDLINE DCOM- 20100615 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 30 IP - 6 DP - 2010 Apr TI - Paraneoplastic Raynaud's phenomenon in a breast cancer survivor. PG - 789-92 LID - 10.1007/s00296-009-0985-5 [doi] AB - A 35-year-old woman with a history of breast cancer, treated 3 years ago with surgery, radiation, and chemotherapy presented with a rapid onset of severe Raynaud's phenomenon. On physical examination, she had digital ulcers and splinter hemorrhages; there were no signs of an underlying rheumatic condition. Laboratory evaluation revealed anemia, the presence of antinuclear antibody and slight depression in her serum complement C3 level. The remainder of her serologic evaluation, including extractable nuclear antigens, anti-double-stranded DNA antibody, antiphospholipid antibodies, rheumatoid factor, anti-neutrophil cytoplasmic antibodies, cryoglobulins, and cold agglutins, were negative. Within weeks of her presentation, she developed acute renal failure and bilateral lower extremity edema. A computed tomography scan of her abdomen and pelvis showed bulky lymphadenopathy and hydronephrosis; a pelvic lymph node biopsy revealed metastatic breast cancer. She was initially managed with passive rewarming strategies, topical antibiotics, vasodilator and anti-platelet therapy, but had a negligible response. However, once she was started on chemotherapy for her recurrent malignancy, there was a significant improvement in her Raynaud's symptoms and resolution of her digital ulcers. FAU - Allen, David AU - Allen D AD - Section of Rheumatology, Department of Medicine, University of Manitoba, Winnipeg, Canada. david.allen.w@gmail.com FAU - Robinson, David AU - Robinson D FAU - Mittoo, Shikha AU - Mittoo S LA - eng PT - Case Reports PT - Journal Article DEP - 20090611 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antineoplastic Agents) RN - 1J444QC288 (Amlodipine) SB - IM MH - Adult MH - Amlodipine/pharmacology MH - Antibodies, Antinuclear/analysis/blood MH - Antineoplastic Agents/therapeutic use MH - Breast Neoplasms/*complications/*immunology/pathology MH - Edema/etiology MH - Female MH - Fingers/innervation/pathology MH - Humans MH - Hydronephrosis/etiology MH - Lymphatic Diseases/complications MH - Neoplasm Metastasis/drug therapy/pathology MH - Paraneoplastic Syndromes/*immunology/pathology/*physiopathology MH - Pelvic Neoplasms/diagnostic imaging/secondary MH - Pelvis/diagnostic imaging/pathology MH - Raynaud Disease/*immunology/pathology/*physiopathology MH - Renal Insufficiency/etiology MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Ulcer/etiology/pathology EDAT- 2009/06/12 09:00 MHDA- 2010/06/16 06:00 CRDT- 2009/06/12 09:00 PHST- 2009/01/29 00:00 [received] PHST- 2009/05/20 00:00 [accepted] PHST- 2009/06/12 09:00 [entrez] PHST- 2009/06/12 09:00 [pubmed] PHST- 2010/06/16 06:00 [medline] AID - 10.1007/s00296-009-0985-5 [doi] PST - ppublish SO - Rheumatol Int. 2010 Apr;30(6):789-92. doi: 10.1007/s00296-009-0985-5. Epub 2009 Jun 11. PMID- 16908357 OWN - NLM STAT- MEDLINE DCOM- 20060912 LR - 20151119 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 55 IP - 3 DP - 2006 Sep TI - Sildenafil for the treatment of Raynaud's phenomenon. PG - 501-2 FAU - Heymann, Warren R AU - Heymann WR LA - eng PT - Journal Article PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Humans MH - Piperazines/*therapeutic use MH - Purines MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate MH - Sulfones MH - Vasodilator Agents/*therapeutic use EDAT- 2006/08/16 09:00 MHDA- 2006/09/13 09:00 CRDT- 2006/08/16 09:00 PHST- 2006/04/08 00:00 [received] PHST- 2006/04/10 00:00 [revised] PHST- 2006/04/12 00:00 [accepted] PHST- 2006/08/16 09:00 [pubmed] PHST- 2006/09/13 09:00 [medline] PHST- 2006/08/16 09:00 [entrez] AID - S0190-9622(06)01213-8 [pii] AID - 10.1016/j.jaad.2006.04.037 [doi] PST - ppublish SO - J Am Acad Dermatol. 2006 Sep;55(3):501-2. doi: 10.1016/j.jaad.2006.04.037. PMID- 31287178 OWN - NLM STAT- MEDLINE DCOM- 20200429 LR - 20200429 IS - 1098-2752 (Electronic) IS - 0738-1085 (Linking) VI - 39 IP - 6 DP - 2019 Sep TI - Lymphaticovenular anastomosis and venous arterialization in coexisting Raynaud's phenomenon and lymphedema: A case report. PG - 553-558 LID - 10.1002/micr.30490 [doi] AB - Raynaud's phenomenon is highly prevalent in the general population. The optimal medical management for patients with severe Raynaud's phenomenon remains unclear. Venous arterialization (VA) may be considered as a salvage procedure when no distal vessels are available for vascular reconstruction. Surgical treatments for lymphedema, including lymphovenous anastomosis (LVA), are becoming popular alternatives to conservative therapy. Here, we report on a patient with comorbid primary Raynaud's phenomenon and lymphedema in whom both VA and LVA were performed. The patient was a 60-year-old woman with an edematous right upper limb and pain and cold sensitivity in the middle, ring, and small fingers that was refractory to medication. Indocyanine green lymphography and computed tomography angiography suggested coexistence of lymphedema and primary Raynaud's phenomenon. VA and LVA were performed to reduce the risks of cellulitis and amputation. Computed tomography angiography was performed regularly after surgery to examine the arterialized venous system and Doppler echography to search for developing branches. Five months later, three branches of the arterialized veins that flowed proximally at the level of the hand and wrist were ligated. By around 1 year after surgery, the lymphedema index in the affected upper limb had improved from 116 to 103 and the patient's numerical rating scale score for intractable pain and cold sensitivity had improved from 6-7 to 1-2. We believe that the combination of VA and LVA in the early stages of primary Raynaud's phenomenon and lymphedema was effective in this case. CI - © 2019 Wiley Periodicals, Inc. FAU - Yoshida, Shuhei AU - Yoshida S AUID- ORCID: 0000-0003-4196-7237 AD - The International Center for Lymphedema, Hiroshima University Hospital, Hiroshima, Japan. FAU - Koshima, Isao AU - Koshima I AD - The International Center for Lymphedema, Hiroshima University Hospital, Hiroshima, Japan. FAU - Imai, Hirofumi AU - Imai H AD - The International Center for Lymphedema, Hiroshima University Hospital, Hiroshima, Japan. FAU - Uchiki, Toshio AU - Uchiki T AD - Plastic and Reconstructive Surgery, Hiroshima University, Hiroshima, Japan. FAU - Sasaki, Ayano AU - Sasaki A AD - Plastic and Reconstructive Surgery, Hiroshima University, Hiroshima, Japan. FAU - Fujioka, Yumio AU - Fujioka Y AD - Plastic and Reconstructive Surgery, Hiroshima University, Hiroshima, Japan. FAU - Nagamatsu, Shogo AU - Nagamatsu S AD - Plastic and Reconstructive Surgery, Hiroshima University, Hiroshima, Japan. FAU - Yokota, Kazunori AU - Yokota K AD - Plastic and Reconstructive Surgery, Hiroshima University, Hiroshima, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20190709 PL - United States TA - Microsurgery JT - Microsurgery JID - 8309230 SB - IM MH - Anastomosis, Surgical/*methods MH - Arm/blood supply MH - Comorbidity MH - Computed Tomography Angiography MH - Female MH - Fingers/blood supply MH - Follow-Up Studies MH - Humans MH - Lymphedema/diagnostic imaging/pathology/*surgery MH - Middle Aged MH - Raynaud Disease/diagnostic imaging/*pathology/*surgery MH - Vascular Surgical Procedures/*methods MH - Veins/surgery EDAT- 2019/07/10 06:00 MHDA- 2020/04/30 06:00 CRDT- 2019/07/10 06:00 PHST- 2019/04/22 00:00 [received] PHST- 2019/06/13 00:00 [revised] PHST- 2019/06/21 00:00 [accepted] PHST- 2019/07/10 06:00 [pubmed] PHST- 2020/04/30 06:00 [medline] PHST- 2019/07/10 06:00 [entrez] AID - 10.1002/micr.30490 [doi] PST - ppublish SO - Microsurgery. 2019 Sep;39(6):553-558. doi: 10.1002/micr.30490. Epub 2019 Jul 9. PMID- 21780344 OWN - NLM STAT- MEDLINE DCOM- 20120222 LR - 20181201 IS - 2651-3463 (Electronic) IS - 1300-2163 (Linking) VI - 22 IP - 2 DP - 2011 Summer TI - Raynaud’s phenomenon in a patient with schizophrenia and obsessive-compulsive disorder: a case report. PG - 132-3 FAU - Korkmaz, Sevda AU - Korkmaz S FAU - Işik, Umut AU - Işik U FAU - Korkmaz, Hasan AU - Korkmaz H LA - eng PT - Case Reports PT - Letter PL - Turkey TA - Turk Psikiyatri Derg JT - Turk psikiyatri dergisi = Turkish journal of psychiatry JID - 9425936 RN - 0 (Antidepressive Agents, Second-Generation) RN - 0 (Antipsychotic Agents) RN - 0 (GABA Modulators) RN - 0 (Vasodilator Agents) RN - 01K63SUP8D (Fluoxetine) RN - 12794-10-4 (Benzodiazepines) RN - 1J444QC288 (Amlodipine) RN - 5PE9FDE8GB (Clonazepam) RN - N7U69T4SZR (Olanzapine) SB - IM MH - Amlodipine/therapeutic use MH - Antidepressive Agents, Second-Generation/therapeutic use MH - Antipsychotic Agents/therapeutic use MH - Benzodiazepines/therapeutic use MH - Clonazepam/therapeutic use MH - Drug Therapy, Combination MH - Fluoxetine/therapeutic use MH - GABA Modulators/therapeutic use MH - Humans MH - Male MH - Middle Aged MH - Obsessive-Compulsive Disorder/*complications/drug therapy MH - Olanzapine MH - Raynaud Disease/*complications/drug therapy MH - Schizophrenia/*complications/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2011/07/26 06:00 MHDA- 2012/02/23 06:00 CRDT- 2011/07/26 06:00 PHST- 2011/07/26 06:00 [entrez] PHST- 2011/07/26 06:00 [pubmed] PHST- 2012/02/23 06:00 [medline] PST - ppublish SO - Turk Psikiyatri Derg. 2011 Summer;22(2):132-3. PMID- 22333496 OWN - NLM STAT- MEDLINE DCOM- 20120920 LR - 20181201 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 65 IP - 7 DP - 2012 Jul TI - Rationalising treatment of Raynaud's syndrome. PG - 986; author reply 986-7 LID - 10.1016/j.bjps.2011.12.041 [doi] FAU - Nikkhah, Dariush AU - Nikkhah D FAU - Rodrigues, Jeremy AU - Rodrigues J FAU - Mahajan, Ajay L AU - Mahajan AL LA - eng PT - Comment PT - Letter DEP - 20120212 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 SB - IM CON - J Plast Reconstr Aesthet Surg. 2011 Nov;64(11):1503-11. doi: 10.1016/j.bjps.2011.05.017. PMID: 21704575 MH - Angiography/*methods MH - Female MH - Hand/*blood supply MH - Humans MH - Male MH - Raynaud Disease/*classification/*diagnostic imaging EDAT- 2012/02/16 06:00 MHDA- 2012/09/21 06:00 CRDT- 2012/02/16 06:00 PHST- 2011/11/28 00:00 [received] PHST- 2011/12/19 00:00 [accepted] PHST- 2012/02/16 06:00 [entrez] PHST- 2012/02/16 06:00 [pubmed] PHST- 2012/09/21 06:00 [medline] AID - S1748-6815(12)00045-9 [pii] AID - 10.1016/j.bjps.2011.12.041 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2012 Jul;65(7):986; author reply 986-7. doi: 10.1016/j.bjps.2011.12.041. Epub 2012 Feb 12. PMID- 40839312 OWN - NLM STAT- MEDLINE DCOM- 20250821 LR - 20251015 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 45 IP - 9 DP - 2025 Aug 21 TI - Fast-track capillaroscopic progression in systemic sclerosis: a case-based review of active pattern emerging within 3 months of raynaud's phenomenon onset. PG - 203 LID - 10.1007/s00296-025-05962-9 [doi] AB - Nailfold videocapillaroscopy typically shows a gradual progression through early, active, and late scleroderma patterns over years in systemic sclerosis. Rapid evolution directly to active pattern within months of Raynaud's phenomenon onset is rarely documented. We report a 39-year-old non-smoking Caucasian woman who developed Raynaud's phenomenon in April 2025. Nailfold videocapillaroscopy performed three months later revealed an active scleroderma pattern characterized by multiple megacapillaries, microhemorrhages, and reduced capillary density, bypassing the typical early pattern phase. Antinuclear antibodies were positive at 1:320 with centromere pattern. The patient met the criteria for early systemic sclerosis (SSc) in accordance with LeRoy and Medsger, and showed high risk for progression to definite systemic sclerosis. This case demonstrates that rapid progression to active scleroderma pattern can occur within three months of Raynaud's onset, challenging the conventional understanding of capillaroscopic evolution timing. Early capillaroscopy in recent-onset Raynaud's phenomenon may identify patients at accelerated risk for systemic sclerosis development, with important implications for monitoring and early intervention strategies. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Nigro, Angelo AU - Nigro A AUID- ORCID: 0009-0001-0802-6825 AD - Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. angelonigro13@gmail.com. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20250821 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - *Raynaud Disease/etiology/diagnostic imaging MH - Female MH - *Microscopic Angioscopy MH - Adult MH - *Scleroderma, Systemic/complications/diagnostic imaging/diagnosis/physiopathology MH - Disease Progression MH - Capillaries/diagnostic imaging MH - Nails/blood supply MH - Time Factors MH - Antibodies, Antinuclear OTO - NOTNLM OT - Active scleroderma pattern OT - Early diagnosis OT - Early systemic sclerosis OT - LeRoy criteria OT - Nailfold videocapillaroscopy OT - Raynaud's phenomenon COIS- Declarations. Conflict of interest: The author declares no conflict of interest. Ethical approval: This case report was conducted in accordance with the Declaration of Helsinki. Formal ethical approval was not required for this single case report. Informed consent: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. EDAT- 2025/08/21 12:47 MHDA- 2025/08/21 12:48 CRDT- 2025/08/21 11:39 PHST- 2025/07/31 00:00 [received] PHST- 2025/08/05 00:00 [accepted] PHST- 2025/08/21 12:48 [medline] PHST- 2025/08/21 12:47 [pubmed] PHST- 2025/08/21 11:39 [entrez] AID - 10.1007/s00296-025-05962-9 [pii] AID - 10.1007/s00296-025-05962-9 [doi] PST - epublish SO - Rheumatol Int. 2025 Aug 21;45(9):203. doi: 10.1007/s00296-025-05962-9. PMID- 23238545 OWN - NLM STAT- MEDLINE DCOM- 20130129 LR - 20121214 IS - 0231-424X (Print) IS - 0231-424X (Linking) VI - 99 IP - 4 DP - 2012 Dec TI - Prothrombotic polymorphisms in patients with Raynaud's phenomenon and migraine. PG - 430-5 LID - 10.1556/APhysiol.99.2012.4.7 [doi] AB - We have investigated the prevalence and possible association of inherited prothrombotic risk factors in patients with primary Raynaud's phenomenon (PRP) and migraine. We performed genotypic analysis of FVLeiden, prothrombin G20210A, methyltetrahydrofolate reductase C677T and FXIII-A V34L mutations in these patients. Two hundred patients with primary Raynaud's phenomenon of Hungarian origin with migraine (57 female, one male, mean age of 43.8 ± 11.5 years) or without migraine (101 female, 41 male, mean age of 41.8 ± 14.5 years) were included in this study. Duration of PRP among migrainous patients was significantly longer than patients without migraine. The prevalence of methyltetrahydrofolate reductase T677 allele among patients with migraine was significantly higher than in patients without migraine (odds ratio 2.1, 95% CI: 1.4-3.3, p = 0.001). The prevalence of other thrombosis-associated alleles did not differ between patients with or without migraine. FVLeiden mutation, prothrombin G20210A mutation, and FXIII-A V34L polymorphism have no apparent effect on the occurrence of migraine in PRP. FAU - Takáts, A T AU - Takáts AT AD - Semmelweis University 1st Department of Surgery Budapest Hungary. FAU - Shemirani, Amir-Houshang AU - Shemirani AH FAU - Zsóri, K S AU - Zsóri KS FAU - András, C AU - András C FAU - Csiki, Z AU - Csiki Z LA - eng PT - Journal Article PL - Hungary TA - Acta Physiol Hung JT - Acta physiologica Hungarica JID - 8309201 RN - 0 (factor V Leiden) RN - 9001-24-5 (Factor V) RN - 9001-26-7 (Prothrombin) RN - 9013-56-3 (Factor XIII) RN - EC 1.5.1.20 (MTHFR protein, human) RN - EC 1.5.1.20 (Methylenetetrahydrofolate Reductase (NADPH2)) SB - IM MH - Adult MH - Factor V/*genetics MH - Factor XIII/*genetics MH - Female MH - Genetic Predisposition to Disease/epidemiology/genetics MH - Genotype MH - Humans MH - Male MH - Methylenetetrahydrofolate Reductase (NADPH2)/*genetics MH - Middle Aged MH - Migraine Disorders/epidemiology/*genetics MH - Polymorphism, Genetic/genetics MH - Prevalence MH - Prothrombin/*genetics MH - Raynaud Disease/epidemiology/*genetics MH - Risk Factors MH - Thrombosis/epidemiology/genetics EDAT- 2012/12/15 06:00 MHDA- 2013/01/30 06:00 CRDT- 2012/12/15 06:00 PHST- 2012/12/15 06:00 [entrez] PHST- 2012/12/15 06:00 [pubmed] PHST- 2013/01/30 06:00 [medline] AID - 3452Q07283213073 [pii] AID - 10.1556/APhysiol.99.2012.4.7 [doi] PST - ppublish SO - Acta Physiol Hung. 2012 Dec;99(4):430-5. doi: 10.1556/APhysiol.99.2012.4.7. PMID- 19177534 OWN - NLM STAT- MEDLINE DCOM- 20090414 LR - 20260518 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 61 IP - 2 DP - 2009 Feb 15 TI - Time to diagnosis in systemic sclerosis: is sex a factor? PG - 274-8 LID - 10.1002/art.24284 [doi] AB - OBJECTIVE: To determine whether sex plays a role in the time to diagnosis of systemic sclerosis (SSc). METHODS: In the Canadian Scleroderma Research Group registry, dates of onset of Raynaud's phenomenon, the first non-Raynaud's disease symptom, and diagnosis were recorded based on patient reports. Association between sex and time to diagnosis was assessed for the group as a whole and stratified based on extent of skin involvement, either limited or diffuse. RESULTS: Of the 408 patients studied (347 women, 61 men, 44% with diffuse cutaneous SSc), the time to diagnosis after the onset of Raynaud's phenomenon was significantly longer for women than men (log rank P = 0.001), but not significantly different after the onset of the first non-Raynaud's disease manifestation. In an analysis stratified by limited or diffuse status, the time to diagnosis from onset of Raynaud's phenomenon was also significantly longer for women than men with diffuse cutaneous SSc (log rank P = 0.008). A trend toward a longer period between onset of Raynaud's phenomenon and SSc diagnosis was observed in women compared with men with limited cutaneous SSc (median 4.6 years in women versus 2.1 years in men; P = 0.085), and there was no sex difference in time to diagnosis after the onset of the first non-Raynaud's disease manifestation. CONCLUSION: In SSc, the time to diagnosis is longer for women than men after the onset of Raynaud's phenomenon, suggesting that there may be possible biologic differences in the progression of disease or in the health care trajectories of men and women with early SSc. FAU - Hudson, Marie AU - Hudson M AD - Sir Mortimer B. Davis Jewish General Hospital and McGill University, Montreal, Quebec, Canada. marie.hudson@mcgill.ca FAU - Thombs, Brett AU - Thombs B FAU - Baron, Murray AU - Baron M CN - Canadian Scleroderma Research Group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Canada MH - Cross-Sectional Studies MH - Female MH - Humans MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*diagnosis MH - *Registries MH - Scleroderma, Diffuse/complications/*diagnosis MH - Scleroderma, Limited/complications/*diagnosis MH - Sex Factors MH - Time FIR - Markland, J IR - Markland J FIR - Pope, J IR - Pope J FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Docherty, P IR - Docherty P FIR - Le Clercq, S IR - Le Clercq S FIR - Khalidi, N A IR - Khalidi NA FIR - Kaminska, E IR - Kaminska E FIR - Sutton, E IR - Sutton E FIR - Smith, C D IR - Smith CD FIR - Mathieu, J-P IR - Mathieu JP FIR - Ligier, S IR - Ligier S FIR - Rahman, P IR - Rahman P EDAT- 2009/01/30 09:00 MHDA- 2009/04/15 09:00 CRDT- 2009/01/30 09:00 PHST- 2009/01/30 09:00 [entrez] PHST- 2009/01/30 09:00 [pubmed] PHST- 2009/04/15 09:00 [medline] AID - 10.1002/art.24284 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Feb 15;61(2):274-8. doi: 10.1002/art.24284. PMID- 32016657 OWN - NLM STAT- MEDLINE DCOM- 20210105 LR - 20210105 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 39 IP - 3 DP - 2020 Mar TI - Possible benefit of tadalafil cream for the treatment of Raynaud's phenomenon and digital ulcers in systemic sclerosis. PG - 963-965 LID - 10.1007/s10067-020-04966-z [doi] FAU - Fernández-Codina, Andreu AU - Fernández-Codina A AUID- ORCID: 0000-0001-7939-7837 AD - Rheumatology Division, University of Western Ontario, 268 Grosvenor Street, London, Ontario, N6A4V2, Canada. Andreu.fernandezcodina@lhsc.on.ca. AD - General Internal Medicine Department-Windsor Campus, University of Western Ontario, Windsor, Ontario, Canada. Andreu.fernandezcodina@lhsc.on.ca. FAU - Kazem, Mikameh AU - Kazem M AD - Rheumatology Division, University of Western Ontario, 268 Grosvenor Street, London, Ontario, N6A4V2, Canada. FAU - Pope, Janet E AU - Pope JE AD - Rheumatology Division, University of Western Ontario, 268 Grosvenor Street, London, Ontario, N6A4V2, Canada. LA - eng PT - Letter DEP - 20200203 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 742SXX0ICT (Tadalafil) SB - IM MH - Administration, Cutaneous MH - Adult MH - Aged MH - Female MH - Fingers/pathology MH - Humans MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/*administration & dosage MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications/drug therapy MH - Skin Cream MH - Tadalafil/*administration & dosage MH - Ulcer/*drug therapy/etiology EDAT- 2020/02/06 06:00 MHDA- 2021/01/06 06:00 CRDT- 2020/02/05 06:00 PHST- 2020/01/17 00:00 [received] PHST- 2020/01/27 00:00 [accepted] PHST- 2020/01/22 00:00 [revised] PHST- 2020/02/06 06:00 [pubmed] PHST- 2021/01/06 06:00 [medline] PHST- 2020/02/05 06:00 [entrez] AID - 10.1007/s10067-020-04966-z [pii] AID - 10.1007/s10067-020-04966-z [doi] PST - ppublish SO - Clin Rheumatol. 2020 Mar;39(3):963-965. doi: 10.1007/s10067-020-04966-z. Epub 2020 Feb 3. PMID- 17164754 OWN - NLM STAT- MEDLINE DCOM- 20070621 LR - 20061213 IS - 0392-9590 (Print) IS - 0392-9590 (Linking) VI - 25 IP - 4 DP - 2006 Dec TI - Endothelin-1, platelets and Raynaud's phenomenon. PG - 436 FAU - Gazi, I AU - Gazi I FAU - Jagroop, I A AU - Jagroop IA FAU - Mikhailidis, D P AU - Mikhailidis DP LA - eng PT - Comment PT - Letter PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 RN - 0 (Endothelin-1) SB - IM CON - Int Angiol. 2006 Jun;25(2):221-7. PMID: 16763543 MH - Blood Platelets/*physiology MH - Endothelin-1/*physiology MH - Humans MH - Raynaud Disease/blood/*etiology EDAT- 2006/12/14 09:00 MHDA- 2007/06/22 09:00 CRDT- 2006/12/14 09:00 PHST- 2006/12/14 09:00 [pubmed] PHST- 2007/06/22 09:00 [medline] PHST- 2006/12/14 09:00 [entrez] PST - ppublish SO - Int Angiol. 2006 Dec;25(4):436. PMID- 19208538 OWN - NLM STAT- MEDLINE DCOM- 20090323 LR - 20090211 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 1 DP - 2009 Jan TI - Raynaud's phenomenon as a presenting feature of hypothyroidism in an 11-year-old girl. PG - 203-4 LID - 10.3899/jrheum.080678 [doi] FAU - Batthish, Michelle AU - Batthish M FAU - Costigan, Colm AU - Costigan C FAU - Killeen, Orla G AU - Killeen OG LA - eng PT - Case Reports PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Child MH - Female MH - Humans MH - Hypothyroidism/*complications/*diagnosis MH - Raynaud Disease/*etiology EDAT- 2009/02/12 09:00 MHDA- 2009/03/24 09:00 CRDT- 2009/02/12 09:00 PHST- 2009/02/12 09:00 [entrez] PHST- 2009/02/12 09:00 [pubmed] PHST- 2009/03/24 09:00 [medline] AID - 36/1/203 [pii] AID - 10.3899/jrheum.080678 [doi] PST - ppublish SO - J Rheumatol. 2009 Jan;36(1):203-4. doi: 10.3899/jrheum.080678. PMID- 21515290 OWN - NLM STAT- MEDLINE DCOM- 20111005 LR - 20131121 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 82 IP - 1 DP - 2011 Jul TI - The effect of metoprolol alone and combined metoprolol-felodipin on the digital microcirculation of patients with primary Raynaud's syndrome. PG - 84-7 LID - 10.1016/j.mvr.2011.04.004 [doi] AB - OBJECTIVES: Calcium channel inhibitors have beneficial impact on microcirculation, but beta-blocker effect is controversial. Clinicians still do not agree on beta-blocker combination with other treatments in the management of impaired microcirculation. The aim of the present study was to describe the effects of beta-blocker metoprolol monotherapy and combined with calcium channel inhibitor felodipin on digital microcirculation in primary Raynaud's syndrome. METHODS: We enrolled in this study 46 patients suffering from both hypertension and primary Raynaud's syndrome. Fifteen patients were treated with beta-blocker monotherapy (metoprolol), 13 received combined beta-blocker and calcium channel blocker therapy (felodipin and metoprolol), while 18 patients without any medications served as controls. Measurement of digital microcirculation was carried out with laser Doppler scanner. RESULTS AND CONCLUSIONS: Our investigation concludes that the concurrent administration of beta-blockers with calcium channel inhibitors positively reduces symptoms in patients suffering from Raynaud's syndrome. CI - Copyright © 2011 Elsevier Inc. All rights reserved. FAU - Csiki, Zoltan AU - Csiki Z AD - Division of Clinical Immunology, 3rd Department of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. csikiz@gmail.com FAU - Garai, Ildiko AU - Garai I FAU - Shemirani, Amir H AU - Shemirani AH FAU - Papp, Gabor AU - Papp G FAU - Zsori, Katalin S AU - Zsori KS FAU - Andras, Csilla AU - Andras C FAU - Zeher, Margit AU - Zeher M LA - eng PT - Controlled Clinical Trial PT - Journal Article DEP - 20110415 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - GEB06NHM23 (Metoprolol) RN - OL961R6O2C (Felodipine) SB - IM MH - Adult MH - Drug Therapy, Combination/methods MH - Felodipine/pharmacology/*therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Hyperemia/physiopathology MH - Hypertension/complications/physiopathology MH - Laser-Doppler Flowmetry MH - Metoprolol/pharmacology/*therapeutic use MH - Microcirculation/*drug effects/physiology MH - Middle Aged MH - Raynaud Disease/complications/*drug therapy/physiopathology MH - Treatment Outcome EDAT- 2011/04/26 06:00 MHDA- 2011/10/06 06:00 CRDT- 2011/04/26 06:00 PHST- 2010/08/17 00:00 [received] PHST- 2011/04/08 00:00 [revised] PHST- 2011/04/09 00:00 [accepted] PHST- 2011/04/26 06:00 [entrez] PHST- 2011/04/26 06:00 [pubmed] PHST- 2011/10/06 06:00 [medline] AID - S0026-2862(11)00066-5 [pii] AID - 10.1016/j.mvr.2011.04.004 [doi] PST - ppublish SO - Microvasc Res. 2011 Jul;82(1):84-7. doi: 10.1016/j.mvr.2011.04.004. Epub 2011 Apr 15. PMID- 28321017 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20260127 IS - 1880-8026 (Electronic) IS - 0019-8366 (Print) IS - 0019-8366 (Linking) VI - 55 IP - 3 DP - 2017 Jun 8 TI - A non-invasive technique for the evaluation of peripheral circulatory functions in female subjects with Raynaud's phenomenon. PG - 275-284 LID - 10.2486/indhealth.2016-0201 [doi] AB - Japanese women now account for 43 percent of the labor force. A number of them are involved in construction, agricultural and forestry jobs. The aim of this study was to establish a non-invasive technique for the evaluation of peripheral circulatory functions in women with Raynaud's phenomenon (RP) and introduce a specific method for the assessment of vascular disturbances in females exposed to hand-transmitted vibration. The subjects of this study were 10 women with primary RP, 7 women with progressive systemic sclerosis (PSS) secondary to RP, and 17 females who were included as the control group. The evaluation of peripheral circulatory functions in all subjects was based on the values of finger blood flow (FBF) and finger skin temperature (FST) measured before, during and following a 5-min recovery period after the hand was immersed in cold water (5°C, 1 min). The measured values of FBF and FST of the primary RP group before and after the immersion test were significantly (p<0.01) lower compared to those of the control group. The technique applied in this study could be used as a non-invasive and tolerable technique to determine the digital circulatory functions in female subjects with RP. FAU - Mirbod, Seyed Mohammad AU - Mirbod SM AD - Laboratory of Scientific English Studies, Gifu Pharmaceutical University, Japan. FAU - Sugiura, Haruo AU - Sugiura H AD - Laboratory of Health and Sport Science, Gifu Pharmaceutical University, Japan. LA - eng PT - Journal Article DEP - 20170317 PL - Japan TA - Ind Health JT - Industrial health JID - 2985065R SB - IM MH - Adult MH - Cold Temperature/adverse effects MH - Female MH - Fingers/*blood supply MH - Hand MH - Humans MH - Japan MH - Laser-Doppler Flowmetry/*methods MH - Raynaud Disease/complications/*diagnosis MH - Scleroderma, Diffuse/complications/diagnosis MH - *Skin Temperature PMC - PMC5462643 OTO - NOTNLM OT - Cold immersion test OT - Finger blood flow OT - Finger skin temperature OT - Health examination OT - Raynaud’s phenomenon OT - Recovery EDAT- 2017/03/23 06:00 MHDA- 2017/12/22 06:00 PMCR- 2017/05/01 CRDT- 2017/03/22 06:00 PHST- 2017/03/23 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/03/22 06:00 [entrez] PHST- 2017/05/01 00:00 [pmc-release] AID - 2016-0201 [pii] AID - 10.2486/indhealth.2016-0201 [doi] PST - ppublish SO - Ind Health. 2017 Jun 8;55(3):275-284. doi: 10.2486/indhealth.2016-0201. Epub 2017 Mar 17. PMID- 34666100 OWN - NLM STAT- MEDLINE DCOM- 20220309 LR - 20220309 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 139 DP - 2022 Jan TI - Diagnosis of pulmonary arterial hypertension preceding the confirmation of systemic sclerosis in a patient with Raynaud's phenomenon. PG - 104267 LID - S0026-2862(21)00137-0 [pii] LID - 10.1016/j.mvr.2021.104267 [doi] FAU - Tariq, Shahna AU - Tariq S AD - Division of Rheumatology, Department of Medicine, University of Alberta, 8-130 Clinical Sciences Building, Edmonton, Alberta T6G 2B7, Canada. FAU - Tervaert, Jan Willem Cohen AU - Tervaert JWC AD - Division of Rheumatology, Department of Medicine, University of Alberta, 8-130 Clinical Sciences Building, Edmonton, Alberta T6G 2B7, Canada. FAU - Osman, Mohammed AU - Osman M AD - Division of Rheumatology, Department of Medicine, University of Alberta, 8-130 Clinical Sciences Building, Edmonton, Alberta T6G 2B7, Canada. Electronic address: mosman@ualberta.ca. LA - eng PT - Case Reports PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20211016 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Anticoagulants) RN - 0 (Antihypertensive Agents) SB - IM MH - Anticoagulants/therapeutic use MH - Antihypertensive Agents/therapeutic use MH - Capillaries/*diagnostic imaging MH - *Cardiac Catheterization MH - Female MH - Fingers/*blood supply MH - Humans MH - *Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Pulmonary Arterial Hypertension/*diagnosis/drug therapy/etiology MH - Raynaud Disease/*diagnostic imaging/drug therapy/etiology MH - Scleroderma, Systemic/complications/*diagnostic imaging/drug therapy MH - Treatment Outcome EDAT- 2021/10/20 06:00 MHDA- 2022/03/11 06:00 CRDT- 2021/10/19 20:13 PHST- 2021/03/31 00:00 [received] PHST- 2021/10/01 00:00 [revised] PHST- 2021/10/12 00:00 [accepted] PHST- 2021/10/20 06:00 [pubmed] PHST- 2022/03/11 06:00 [medline] PHST- 2021/10/19 20:13 [entrez] AID - S0026-2862(21)00137-0 [pii] AID - 10.1016/j.mvr.2021.104267 [doi] PST - ppublish SO - Microvasc Res. 2022 Jan;139:104267. doi: 10.1016/j.mvr.2021.104267. Epub 2021 Oct 16. PMID- 18329535 OWN - NLM STAT- MEDLINE DCOM- 20080506 LR - 20080310 IS - 0889-857X (Print) IS - 0889-857X (Linking) VI - 34 IP - 1 DP - 2008 Feb TI - Diagnosis and management of scleroderma peripheral vascular disease. PG - 89-114; vii LID - 10.1016/j.rdc.2007.11.006 [doi] AB - The manifestations of peripheral vascular disease in patients who have systemic sclerosis (SSc) range from episodic Raynaud's phenomenon to irreversible tissue injury with ulceration and gangrene. Structural and functional changes may occur in the microvessels, digital arteries, and sometimes more proximal vessels. This article discusses the assessment of patients who have Raynaud's phenomenon in whom an underlying scleroderma-spectrum disorder is suspected and patients who have SSc with critical digital ischemia/ulceration. Different imaging techniques, including capillaroscopy and angiography, complement the history and examination, and developments in vascular imaging should facilitate future studies of pathogenesis and treatment response. New vasoactive treatments are currently being researched and older treatments revisited; therefore new approaches to therapy will likely be developed over the next 5 to 10 years. FAU - Herrick, Ariane AU - Herrick A AD - Rheumatic Diseases Centre, University of Manchester, Salford Royal NHS Foundation Trust, Salford M6 8HD, UK. ariane.herrick@manchester.ac.uk LA - eng PT - Journal Article PT - Review PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 RN - 0 (Antioxidants) RN - 0 (Prostaglandins, Synthetic) RN - 0 (Vasodilator Agents) SB - IM MH - Angiography MH - Antioxidants/therapeutic use MH - Humans MH - Microscopic Angioscopy MH - Prostaglandins, Synthetic/therapeutic use MH - Raynaud Disease/complications/diagnosis/*drug therapy MH - *Scleroderma, Systemic/diagnosis/drug therapy MH - Vasodilator Agents/*therapeutic use RF - 128 EDAT- 2008/03/11 09:00 MHDA- 2008/05/07 09:00 CRDT- 2008/03/11 09:00 PHST- 2008/03/11 09:00 [pubmed] PHST- 2008/05/07 09:00 [medline] PHST- 2008/03/11 09:00 [entrez] AID - S0889-857X(07)00098-1 [pii] AID - 10.1016/j.rdc.2007.11.006 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2008 Feb;34(1):89-114; vii. doi: 10.1016/j.rdc.2007.11.006. PMID- 23314682 OWN - NLM STAT- MEDLINE DCOM- 20131017 LR - 20130708 IS - 1651-2057 (Electronic) IS - 0001-5555 (Linking) VI - 93 IP - 4 DP - 2013 Jul 6 TI - Freezing fingers syndrome, primary and secondary Raynaud's phenomenon: characteristic features with hand thermography. PG - 428-32 LID - 10.2340/00015555-1508 [doi] AB - The aim of the present study was to establish a thermographic model of healthy subjects' hands and compare it with a model of the hands of patients with freezing fingers syndrome, a group usually regarded as a healthy population. A further aim was to establish the thermographic parameters that distinguish primary Raynaud's phenomenon (RP) from secondary RP. The study was conducted on a group of 74 subjects, divided into 3 groups: patients with freezing hands symptoms (G1), those with primary RP (G2), and those with limited scleroderma (G3). In addition, 69 healthy volunteers served as a control group (G4). The most distinctive features of healthy subjects' hands are the thermal symmetry between left to right measurements (ΔT<0.5°C) and between mean temperatures of the metacarpus and digits (ΔT<0.5°C (1°C maximum)). A negative correlation was found between mean hands temperature and age of subjects in G4 (p<0.0001). All the temperatures observed in G4 subjects were significantly higher than among patients in G1, G2 and G3 (p<0.001). No significant differences were found between mean temperatures in G2 and G3. RP should be suspected when differences between mean temperatures of the metacarpus and digits are ≥to 3ºC. Moreover, we suggest that a cut-off point >1ºC is established for subjects with "freezing" symptoms. FAU - Chlebicka, Iwona AU - Chlebicka I AD - Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Wroclaw, Poland. FAU - Matusiak, Łukasz AU - Matusiak Ł FAU - Maj, Joanna AU - Maj J FAU - Baran, Eugeniusz AU - Baran E FAU - Szepietowski, Jacek C AU - Szepietowski JC LA - eng PT - Journal Article PL - Sweden TA - Acta Derm Venereol JT - Acta dermato-venereologica JID - 0370310 SB - IM MH - Adolescent MH - Adult MH - Aged MH - *Body Temperature MH - Case-Control Studies MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis/physiopathology MH - Scleroderma, Systemic/*diagnosis/physiopathology MH - *Thermography MH - Young Adult EDAT- 2013/01/15 06:00 MHDA- 2013/10/18 06:00 CRDT- 2013/01/15 06:00 PHST- 2013/01/15 06:00 [entrez] PHST- 2013/01/15 06:00 [pubmed] PHST- 2013/10/18 06:00 [medline] AID - 10.2340/00015555-1508 [doi] PST - ppublish SO - Acta Derm Venereol. 2013 Jul 6;93(4):428-32. doi: 10.2340/00015555-1508. PMID- 28780561 OWN - NLM STAT- MEDLINE DCOM- 20180511 LR - 20220330 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 7 IP - 8 DP - 2017 Aug 4 TI - Behaviour change interventions for the management of Raynaud's phenomenon: a systematic review protocol. PG - e017039 LID - 10.1136/bmjopen-2017-017039 [doi] LID - e017039 AB - INTRODUCTION: Raynaud's phenomenon (RP) describes excessive peripheral vasospasm to cold exposure and/or emotional stress. RP episodes are associated with digital colour changes, pain and reduced quality of life. Pharmacological interventions are of low to moderate efficacy and often result in adverse effects such as facial flushing and headaches. Recommended lifestyle and behavioural interventions have not been evaluated. The objectives of the proposed systematic review are to assess the comparative safety and efficacy of behaviour change interventions for RP and identify what we can learn to inform future interventions. METHODS AND ANALYSIS: Studies eligible for inclusion include randomised controlled trials testing behaviour change interventions with a control comparator. A comprehensive search strategy will include peer review and grey literature up until 30 April 2017. Search databases will include Medline, Embase, PsychINFO and Cochrane. Initial sifting, eligibility, data extraction, risk of bias and quality assessment will be subject to review by two independent reviewers with a third reviewer resolving discrepancies. Risk of bias assessment will be performed using Cochrane risk of a bias assessment tool with quality of evidence assessed using Grading of Recommendations Assessment, Development and Evaluation(GRADE). A meta-analysis will be performed if there are sufficient data. Two subgroup analyses are planned: primary versus secondary RP outcomes; comparison of theoretically informed interventions with pragmatic interventions. ETHICS AND DISSEMINATION: This review does not require ethical approval as it will summarise published studies with non-identifiable data. This protocol complies with the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P) guidelines. Findings will be disseminated in peer-reviewed articles and reported according to PRISMA. This review will make a significant contribution to the management of RP where no review of behaviour-change interventions currently exist. The synopsis and protocol for the proposed systematic review is registered in the International Prospective Register of Systematic Reviews (registration number CRD42017049643). CI - © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. FAU - Daniels, Jo AU - Daniels J AUID- ORCID: 0000-0003-3067-9416 AD - Department of Psychology, The University of Bath, Bath, UK. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. AD - Department of Pharmacy and Pharmacology, The University of Bath, Bath, UK. FAU - Eccelston, Christopher AU - Eccelston C AD - Centre for Pain Research, Department for Health, The University of Bath, Bath, UK. LA - eng PT - Journal Article DEP - 20170804 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - *Behavior Therapy MH - Cold Temperature/adverse effects MH - Humans MH - Patient Compliance MH - Quality of Life MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/physiopathology/*prevention & control/*therapy MH - *Research Design MH - *Review Literature as Topic MH - *Risk Reduction Behavior MH - Smoking Cessation MH - Stress, Psychological/complications MH - Systematic Reviews as Topic PMC - PMC5724227 OTO - NOTNLM OT - Raynaud's Phenomenon OT - behaviour change OT - systematic review COIS- Competing interests: None declared. EDAT- 2017/08/07 06:00 MHDA- 2018/05/12 06:00 PMCR- 2017/08/04 CRDT- 2017/08/07 06:00 PHST- 2017/08/07 06:00 [entrez] PHST- 2017/08/07 06:00 [pubmed] PHST- 2018/05/12 06:00 [medline] PHST- 2017/08/04 00:00 [pmc-release] AID - bmjopen-2017-017039 [pii] AID - 10.1136/bmjopen-2017-017039 [doi] PST - epublish SO - BMJ Open. 2017 Aug 4;7(8):e017039. doi: 10.1136/bmjopen-2017-017039. PMID- 33994482 OWN - NLM STAT- MEDLINE DCOM- 20211028 LR - 20211028 IS - 1349-6867 (Electronic) IS - 1343-1420 (Linking) VI - 68 IP - 1.2 DP - 2021 TI - Nailfold capillaroscopy : a comprehensive review on common findings and clinical usefulness in non-rheumatic disease. PG - 6-14 LID - 10.2152/jmi.68.6 [doi] AB - Nailfold video-capillaroscopy (NVC) is a useful diagnostic tool, used to early detect abnormalities in micro-circulation, providing a qualitative description of microvascular anomalies in Raynaud's phenomenon. NVC role in the diagnosis of Systemic Sclerosis is well known. In other rheumatic conditions such as connective tissue diseases, vasculitis, and arthritis, the NVC anomalies are often included in a scleroderma like pattern. The use of NVC in non-rheumatic diseases (NRD), with remarkable microvascular damage, as diabetes, is not standardized yet, although several research studies are carrying on. The aim of this article is to provide a resume of published results in order to lay the groundwork for the employment of NVC both in the diagnosis and follow up of microvascular complication in NRD. Furthermore, we mention NVC findings in pathologies without well recognize microvascular damages in their pathogenesis : micro-vessels abnormalities may suggest a different point of view. J. Med. Invest. 68 : 6-14, February, 2021. FAU - Mansueto, Natalia AU - Mansueto N AD - Department of Medical and Surgical Sciences - Rheumatology Unit, University of Foggia, Italy. FAU - Rotondo, Cinzia AU - Rotondo C AD - Department of Medical and Surgical Sciences - Rheumatology Unit, University of Foggia, Italy. FAU - Corrado, Addolorata AU - Corrado A AD - Department of Medical and Surgical Sciences - Rheumatology Unit, University of Foggia, Italy. FAU - Cantatore, Francesco Paolo AU - Cantatore FP AD - Department of Medical and Surgical Sciences - Rheumatology Unit, University of Foggia, Italy. LA - eng PT - Journal Article PT - Review PL - Japan TA - J Med Invest JT - The journal of medical investigation : JMI JID - 9716841 SB - IM MH - Humans MH - Microscopic Angioscopy MH - Nails/diagnostic imaging MH - *Raynaud Disease/diagnosis MH - *Rheumatic Diseases/diagnosis MH - *Scleroderma, Systemic/diagnosis OTO - NOTNLM OT - Raynaud’s phenomenon OT - diabetes OT - glaucoma OT - microcirculation OT - nailfold videocapillaroscopy EDAT- 2021/05/18 06:00 MHDA- 2021/10/29 06:00 CRDT- 2021/05/17 05:52 PHST- 2021/05/17 05:52 [entrez] PHST- 2021/05/18 06:00 [pubmed] PHST- 2021/10/29 06:00 [medline] AID - 10.2152/jmi.68.6 [doi] PST - ppublish SO - J Med Invest. 2021;68(1.2):6-14. doi: 10.2152/jmi.68.6. PMID- 21420982 OWN - NLM STAT- MEDLINE DCOM- 20120313 LR - 20151119 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 82 IP - 3 DP - 2011 Nov TI - Influence of the cold challenge on the discriminatory capacity of the digital distal-dorsal difference in the thermographic assessment of Raynaud's phenomenon. PG - 364-8 LID - 10.1016/j.mvr.2011.03.007 [doi] AB - OBJECTIVES: To investigate the influence of a standardised cold stress test (CST) on the thermographic 'distal-dorsal difference' (DDD) and its capacity to differentiate between disease states in the assessment of Raynaud's phenomenon (RP), and to compare the discriminatory capacity of the DDD of individual digits with composite indices of multiple digits. METHODS: Thermographic images of 55 patients with primary RP (PRP, n=27) and systemic sclerosis (SSc, n=28) who had undergone assessment of RP were retrospectively reviewed. The DDD for individual digits, and composite scores of multiple digits, were calculated at baseline (23°C), and at 10 min following CST. The discriminatory capacity of the mean DDD, and the proportion of patients with a clinically meaningful DDD of <-1°C, were assessed for individual digits and composite indices, at baseline and following cold challenge. RESULTS: There was a more pronounced decrease of the DDD (indicating reduced distal perfusion) following CST in patients with PRP compared to SSc. The disparity in response to CST between groups narrowed the differences that were present at baseline, reducing the discriminatory capacity of the DDD for all endpoints. Sparing of the thumbs occurs to a greater extent in SSc (P<0.005) compared with PRP (P<0.05) but does not facilitate differentiation between groups. Large variability of the DDD within groups precludes easy differentiation between disease states. Composite indices of multiple digits are preferable to individual digital assessment. CONCLUSIONS: The discriminatory capacity of the DDD is lost following CST. The CST may not be essential in the thermographic assessment of RP, potentially allowing greater use of thermography in clinical practise. CI - Copyright © 2011 Elsevier Inc. All rights reserved. FAU - Pauling, J D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Bath, UK. John.Pauling@rnhrd.nhs.uk FAU - Flower, V AU - Flower V FAU - Shipley, J A AU - Shipley JA FAU - Harris, N D AU - Harris ND FAU - McHugh, N J AU - McHugh NJ LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110321 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - *Body Temperature Regulation MH - Chi-Square Distribution MH - *Cold Temperature MH - Diagnosis, Differential MH - England MH - Female MH - Fingers/*blood supply MH - Humans MH - Image Interpretation, Computer-Assisted MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis/etiology/physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/physiopathology MH - Severity of Illness Index MH - *Thermography MH - Time Factors EDAT- 2011/03/23 06:00 MHDA- 2012/03/14 06:00 CRDT- 2011/03/23 06:00 PHST- 2011/01/27 00:00 [received] PHST- 2011/03/14 00:00 [accepted] PHST- 2011/03/23 06:00 [entrez] PHST- 2011/03/23 06:00 [pubmed] PHST- 2012/03/14 06:00 [medline] AID - S0026-2862(11)00044-6 [pii] AID - 10.1016/j.mvr.2011.03.007 [doi] PST - ppublish SO - Microvasc Res. 2011 Nov;82(3):364-8. doi: 10.1016/j.mvr.2011.03.007. Epub 2011 Mar 21. PMID- 40438935 OWN - NLM STAT- MEDLINE DCOM- 20250529 LR - 20250626 IS - 2242-3982 (Electronic) IS - 1239-9736 (Print) IS - 1239-9736 (Linking) VI - 84 IP - 1 DP - 2025 Dec TI - Local cold injury affecting the hand and incident Raynaud's phenomenon - a case report. PG - 2511501 LID - 10.1080/22423982.2025.2511501 [doi] LID - 2511501 AB - There is an association between local cold injuries and Raynaud's phenomenon (RP) in the scientific literature, but the time relation and anatomical correlation have not been established. During military training in an Arctic setting, a previously healthy man in his early twenties sustained a freezing cold injury affecting mainly his right index finger. He subsequently developed Raynaud's phenomenon limited to only the part of the index finger that was originally affected by the cold injury. Medical investigation also revealed findings suggestive of subclinical peripheral neuropathy. This case demonstrates that Raynaud's phenomenon can develop secondary to local cold injury affecting the hand. It also suggests that cold exposure could be related to peripheral neuropathy. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Department of Epidemiology and Global Health, Umeå University, Umeå, Sweden. LA - eng PT - Case Reports PT - Journal Article DEP - 20250529 PL - United States TA - Int J Circumpolar Health JT - International journal of circumpolar health JID - 9713056 SB - IM MH - Humans MH - Male MH - Arctic Regions MH - *Cold Injury/complications MH - Military Personnel MH - *Raynaud Disease/etiology MH - Young Adult PMC - PMC12123960 OTO - NOTNLM OT - Cold injury OT - Raynaud Disease OT - Vascular Diseases OT - military personnel OT - peripheral nervous system diseases COIS- No potential conflict of interest was reported by the author(s). EDAT- 2025/05/29 06:26 MHDA- 2025/05/29 06:27 PMCR- 2025/05/29 CRDT- 2025/05/29 05:03 PHST- 2025/05/29 06:27 [medline] PHST- 2025/05/29 06:26 [pubmed] PHST- 2025/05/29 05:03 [entrez] PHST- 2025/05/29 00:00 [pmc-release] AID - 2511501 [pii] AID - 10.1080/22423982.2025.2511501 [doi] PST - ppublish SO - Int J Circumpolar Health. 2025 Dec;84(1):2511501. doi: 10.1080/22423982.2025.2511501. Epub 2025 May 29. PMID- 18505148 OWN - NLM STAT- MEDLINE DCOM- 20080812 LR - 20181201 IS - 1545-9616 (Print) IS - 1545-9616 (Linking) VI - 7 IP - 5 DP - 2008 May TI - Treatment for Raynaud's: beyond calcium channel blockers. PG - 497-500 FAU - Bergstrom, Kendra Gail AU - Bergstrom KG FAU - Perelman, Ronald O AU - Perelman RO LA - eng PT - News PL - United States TA - J Drugs Dermatol JT - Journal of drugs in dermatology : JDD JID - 101160020 RN - 0 (Calcium Channel Blockers) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfonamides) RN - 0 (Sulfones) RN - 94ZLA3W45F (Arginine) RN - BW9B0ZE037 (Sildenafil Citrate) RN - EC 3.4.24.69 (Botulinum Toxins) RN - Q326023R30 (Bosentan) RN - XM03YJ541D (Prazosin) SB - IM MH - Arginine/administration & dosage MH - Bosentan MH - Botulinum Toxins/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Humans MH - Piperazines/therapeutic use MH - Prazosin/therapeutic use MH - Purines/therapeutic use MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate MH - Sulfonamides/therapeutic use MH - Sulfones/therapeutic use EDAT- 2008/05/29 09:00 MHDA- 2008/08/13 09:00 CRDT- 2008/05/29 09:00 PHST- 2008/05/29 09:00 [pubmed] PHST- 2008/08/13 09:00 [medline] PHST- 2008/05/29 09:00 [entrez] PST - ppublish SO - J Drugs Dermatol. 2008 May;7(5):497-500. PMID- 24705032 OWN - NLM STAT- MEDLINE DCOM- 20150724 LR - 20181202 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 65 IP - 6 DP - 2014 Mar 17 TI - Efficacy of bosentan in the treatment of Raynaud's phenomenon in patients with systemic sclerosis never treated with prostanoids. PG - 286-91 LID - 10.4081/reumatismo.2013.691 [doi] AB - The objective of this study was to evaluate the efficacy of the endothelin receptor antagonist, bosentan, in patients with Raynaud's phenomenon secondary to systemic sclerosis never treated with prostanoids and without digital ulcers. The study design is a preliminary, prospective open label trial. The patients recruited took one 62.5 mg dose of bosentan twice daily for 4 weeks, followed by 125 mg twice daily for 24 weeks. Of the 10 patients recruited, all completed the study. The reduction in Raynaud's phenomenon attacks at week 24 from the baseline was statistically significant (Δ-1.3, P=0.0126). The Raynaud's condition score showed a statistically significant improvement (Δ-1.4, P=0.0279), as did the visual analog pain scale (Δ-1.5, P=0.0016) at the 24th week. Bosentan appears to be effective and may be a valid alternative for the treatment of severe secondary Raynaud's phenomenon for patients where prostanoids therapy is contraindicated or refused. FAU - Parisi, S AU - Parisi S AD - Struttura Complessa di Reumatologia, A.O. Città della Salute e della Scienza di Torino. simone.parisi@hotmail.it. FAU - Bruzzone, M AU - Bruzzone M FAU - Centanaro Di Vittorio, C AU - Centanaro Di Vittorio C FAU - Laganà, A AU - Laganà A FAU - Peroni, C L AU - Peroni CL FAU - Fusaro, E AU - Fusaro E LA - eng PT - Clinical Trial PT - Journal Article DEP - 20140317 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Bosentan MH - Endothelin Receptor Antagonists/*therapeutic use MH - Female MH - Follow-Up Studies MH - Humans MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/diagnosis/*drug therapy MH - Sulfonamides/*therapeutic use MH - Treatment Outcome EDAT- 2014/04/08 06:00 MHDA- 2015/07/25 06:00 CRDT- 2014/04/08 06:00 PHST- 2013/08/08 00:00 [received] PHST- 2013/12/31 00:00 [accepted] PHST- 2014/04/08 06:00 [entrez] PHST- 2014/04/08 06:00 [pubmed] PHST- 2015/07/25 06:00 [medline] AID - 10.4081/reumatismo.2013.691 [doi] PST - epublish SO - Reumatismo. 2014 Mar 17;65(6):286-91. doi: 10.4081/reumatismo.2013.691. PMID- 30411414 OWN - NLM STAT- MEDLINE DCOM- 20200316 LR - 20200316 IS - 1365-2796 (Electronic) IS - 0954-6820 (Linking) VI - 285 IP - 3 DP - 2019 Mar TI - Systemic features of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations: a monogenic small vessel disease. PG - 317-332 LID - 10.1111/joim.12848 [doi] AB - BACKGROUND: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a small vessel disease caused by C-terminal truncating TREX1 mutations. The disease is typically characterized by vascular retinopathy and focal and global brain dysfunction. Systemic manifestations have also been reported but not yet systematically investigated. METHODS: In a cross-sectional study, we compared the clinical characteristics of 33 TREX1 mutation carriers (MC+) from three Dutch RVCL-S families with those of 37 family members without TREX1 mutation (MC-). All participants were investigated using personal interviews, questionnaires, physical, neurological and neuropsychological examinations, blood and urine tests, and brain MRI. RESULTS: In MC+, vascular retinopathy and Raynaud's phenomenon were the earliest symptoms presenting from age 20 onwards. Kidney disease became manifest from around age 35, followed by liver disease, anaemia, markers of inflammation and, in some MC+, migraine and subclinical hypothyroidism, all from age 40. Cerebral deficits usually started mildly around age 50, associated with white matter and intracerebral mass lesions, and becoming severe around age 60-65. CONCLUSIONS: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations is a rare, but likely underdiagnosed, systemic small vessel disease typically starting with vascular retinopathy, followed by multiple internal organ disease, progressive brain dysfunction, and ultimately premature death. CI - © 2018 The Association for the Publication of the Journal of Internal Medicine. FAU - Pelzer, N AU - Pelzer N AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Hoogeveen, E S AU - Hoogeveen ES AD - Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Haan, J AU - Haan J AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. AD - Department of Neurology, Alrijne Hospital, Leiderdorp, The Netherlands. FAU - Bunnik, R AU - Bunnik R AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Poot, C C AU - Poot CC AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - van Zwet, E W AU - van Zwet EW AD - Department of Biomedical Data Sciences, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Inderson, A AU - Inderson A AD - Department of Gastroenterology-Hepatology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Fogteloo, A J AU - Fogteloo AJ AD - Department of Internal Medicine (Acute Care), Leiden University Medical Centre, Leiden, The Netherlands. FAU - Reinders, M E J AU - Reinders MEJ AD - Department of Internal Medicine (Nephrology), Leiden University Medical Centre, Leiden, The Netherlands. FAU - Middelkoop, H A M AU - Middelkoop HAM AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. AD - Institute of Psychology, Health, Medical and Neuropsychology Unit, Leiden University, Leiden, The Netherlands. FAU - Kruit, M C AU - Kruit MC AD - Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - van den Maagdenberg, A M J M AU - van den Maagdenberg AMJM AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. AD - Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Ferrari, M D AU - Ferrari MD AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Terwindt, G M AU - Terwindt GM AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20181108 PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 RN - 0 (Phosphoproteins) RN - EC 3.1.- (Exodeoxyribonucleases) RN - EC 3.1.16.- (three prime repair exonuclease 1) SB - IM MH - Adult MH - Age of Onset MH - Exodeoxyribonucleases/genetics MH - Female MH - Humans MH - Kidney Diseases/diagnosis/etiology MH - *Leukoencephalopathies/congenital/epidemiology/physiopathology/psychology MH - Liver Diseases/diagnosis/etiology MH - Magnetic Resonance Imaging/methods MH - Male MH - Middle Aged MH - Mutation MH - Netherlands/epidemiology MH - Neuropsychological Tests MH - Phosphoproteins/genetics MH - *Raynaud Disease/diagnosis/etiology MH - *Retinal Vasculitis/diagnosis/etiology MH - *Systemic Vasculitis/diagnosis/epidemiology/etiology MH - White Matter/diagnostic imaging OTO - NOTNLM OT - Raynaud's phenomenon OT - kidney disease OT - liver disease OT - microangiopathy OT - neurology OT - thyroid disease EDAT- 2018/11/10 06:00 MHDA- 2020/03/17 06:00 CRDT- 2018/11/10 06:00 PHST- 2018/11/10 06:00 [pubmed] PHST- 2020/03/17 06:00 [medline] PHST- 2018/11/10 06:00 [entrez] AID - 10.1111/joim.12848 [doi] PST - ppublish SO - J Intern Med. 2019 Mar;285(3):317-332. doi: 10.1111/joim.12848. Epub 2018 Nov 8. PMID- 18799065 OWN - NLM STAT- MEDLINE DCOM- 20090205 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 3 Suppl 49 DP - 2008 May-Jun TI - Iloprost treatment for refractory Raynaud's phenomenon in two infants. PG - S105-7 AB - Raynaud's phenomenon (RP) is rare in young children. We describe two infants with severe RP, manifesting as fingertip necrosis, who were resistant to conventional vasodilators and were treated successfully with iloprost, a prostacyclin analogue. The application of iloprost is safe and should be considered in children with threatening ischemic digits. FAU - Shouval, D S AU - Shouval DS AD - Department of Pediatrics C, Schneider Children's Medical Center of Israel, Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. dror_sho@netvision.net.il FAU - Mukamel, M AU - Mukamel M FAU - Zulian, F AU - Zulian F FAU - Amir, J AU - Amir J FAU - Harel, L AU - Harel L LA - eng PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Female MH - Fingers/blood supply/pathology MH - Humans MH - Iloprost/*therapeutic use MH - Infant MH - Male MH - Necrosis MH - Raynaud Disease/*drug therapy/pathology MH - Vasodilator Agents/*therapeutic use EDAT- 2008/10/24 09:00 MHDA- 2009/02/06 09:00 CRDT- 2008/10/24 09:00 PHST- 2008/10/24 09:00 [pubmed] PHST- 2009/02/06 09:00 [medline] PHST- 2008/10/24 09:00 [entrez] AID - 2369 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 May-Jun;26(3 Suppl 49):S105-7. PMID- 29393078 OWN - NLM STAT- MEDLINE DCOM- 20181109 LR - 20220408 IS - 0973-3922 (Electronic) IS - 0378-6323 (Linking) VI - 84 IP - 2 DP - 2018 TI - Eosinophilic fasciitis associated with Raynaud's phenomenon, esophageal dysmotility, positive antinuclear antibody and anti-neutrophil cytoplasmic antibody. PG - 224-227 LID - 10.4103/ijdvl.IJDVL_306_16 [doi] FAU - Behera, Biswanath AU - Behera B AD - Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India. FAU - Singh, Nidhi AU - Singh N AD - Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India. FAU - Chandrashekar, Laxmisha AU - Chandrashekar L AD - Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India. FAU - Sathya, Arjuna Babu AU - Sathya AB AD - Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India. FAU - Thappa, Devinder Mohan AU - Thappa DM AD - Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India. FAU - Toi, Pampa Ch AU - Toi PC AD - Department of Pathology, JIPMER, Puducherry, India. LA - eng PT - Case Reports PT - Letter PL - United States TA - Indian J Dermatol Venereol Leprol JT - Indian journal of dermatology, venereology and leprology JID - 7701852 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Antibodies, Antinuclear) RN - 0 (Dermatologic Agents) RN - 9PHQ9Y1OLM (Prednisolone) RN - YL5FZ2Y5U1 (Methotrexate) RN - Eosinophilic Fasciitis SB - IM MH - Adult MH - *Antibodies, Antineutrophil Cytoplasmic/blood MH - *Antibodies, Antinuclear/blood MH - Dermatologic Agents/administration & dosage MH - Eosinophilia/complications/*diagnosis/drug therapy MH - Esophageal Motility Disorders/complications/*diagnosis/drug therapy MH - Fasciitis/complications/*diagnosis/drug therapy MH - Female MH - Humans MH - Methotrexate/administration & dosage MH - Prednisolone/administration & dosage MH - Raynaud Disease/complications/*diagnosis/drug therapy COIS- There are no conflicts of interest. EDAT- 2018/02/03 06:00 MHDA- 2018/11/10 06:00 CRDT- 2018/02/03 06:00 PHST- 2018/02/03 06:00 [pubmed] PHST- 2018/11/10 06:00 [medline] PHST- 2018/02/03 06:00 [entrez] AID - 224597 [pii] AID - 10.4103/ijdvl.IJDVL_306_16 [doi] PST - ppublish SO - Indian J Dermatol Venereol Leprol. 2018;84(2):224-227. doi: 10.4103/ijdvl.IJDVL_306_16. PMID- 30309971 OWN - NLM STAT- MEDLINE DCOM- 20191202 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 78 IP - 3 DP - 2019 Mar TI - Patient organisation-led initiatives can play an important role in raising awareness about Raynaud's phenomenon and encourage earlier healthcare utilisation for high-risk groups. PG - 439-441 LID - 10.1136/annrheumdis-2018-214161 [doi] FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Baker, Amy AU - Baker A AD - Scleroderma and Raynaud's UK, London, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK johnpauling@nhs.net. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. LA - eng PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20181011 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Delayed Diagnosis/psychology MH - *Health Knowledge, Attitudes, Practice MH - Humans MH - Mass Screening/methods/*psychology MH - Patient Acceptance of Health Care/*psychology MH - Raynaud Disease/*diagnosis/*psychology OTO - NOTNLM OT - epidemiology OT - patient perspective OT - systemic sclerosis COIS- Competing interests: None declared. EDAT- 2018/10/13 06:00 MHDA- 2019/12/04 06:00 CRDT- 2018/10/13 06:00 PHST- 2018/07/21 00:00 [received] PHST- 2018/09/13 00:00 [revised] PHST- 2018/09/13 00:00 [accepted] PHST- 2018/10/13 06:00 [pubmed] PHST- 2019/12/04 06:00 [medline] PHST- 2018/10/13 06:00 [entrez] AID - S0003-4967(24)00777-5 [pii] AID - 10.1136/annrheumdis-2018-214161 [doi] PST - ppublish SO - Ann Rheum Dis. 2019 Mar;78(3):439-441. doi: 10.1136/annrheumdis-2018-214161. Epub 2018 Oct 11. PMID- 39515460 OWN - NLM STAT- MEDLINE DCOM- 20241129 LR - 20250108 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 157 DP - 2025 Jan TI - Increased angiopoietin-1 improves nailfold capillary morphology in patients with systemic sclerosis. PG - 104761 LID - S0026-2862(24)00110-9 [pii] LID - 10.1016/j.mvr.2024.104761 [doi] AB - OBJECTIVE: Raynaud's phenomenon is a common symptom of systemic sclerosis. We previously reported that elbow heating increases angiopoietin-1 in the fingertips and alleviates Raynaud's phenomenon. Angiopoietin-1 levels decrease in patients with systemic sclerosis with severe capillary damage. We aimed to conduct a prospective study to confirm whether the increase in angiopoietin-1 caused by heating modifies capillary morphology. METHODS: The left ring fingers of 19 patients with systemic sclerosis were monitored six times at 4-week intervals using capillaroscopy, during which both elbows were heated using disposable heating pads for 8 weeks. Blood samples were collected from the same fingertips four times-before heating, twice during heating, and once after heating-to measure angiopoietin-1. RESULTS: In six patients, the peak increase in angiopoietin-1 occurred 4 weeks after the start of heating, whereas in seven patients, the peak value was observed 4 weeks after the termination thereof. No change in the density of the front-row capillaries was observed by capillaroscopy. The proportion of hairpin-shaped capillaries increased from 20.2 % during the preheating period to 26.6 % during the heating period (p = 0.00107). When a correlation coefficient of 0.6 or higher was set as significant, there was a strong correlation between changes in fingertip angiopoietin-1 levels and changes in the proportion of hairpin-shaped capillaries in six patients. CONCLUSION: Increased angiopoietin-1 levels in the fingertip due to elbow heating may improve the peripheral capillary morphology in patients with systemic sclerosis. CI - Copyright © 2024. Published by Elsevier Inc. FAU - Shima, Yoshihito AU - Shima Y AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. Electronic address: ryanjin@imed3.med.osaka-u.ac.jp. FAU - Watanabe, Akane AU - Watanabe A AD - Laboratory of Thermo-therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Inoue, Nobuto AU - Inoue N AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Maruyama, Tetsuya AU - Maruyama T AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Kunitomo, Eiji AU - Kunitomo E AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Matsushima, Yuji AU - Matsushima Y AD - Central R&D Laboratory, Kobayashi Pharmaceutical Co., Ltd., Ibaraki, Japan. FAU - Kumanogoh, Atsushi AU - Kumanogoh A AD - Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20241107 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Angiopoietin-1) RN - 0 (ANGPT1 protein, human) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Microscopic Angioscopy MH - Female MH - *Angiopoietin-1/blood MH - Middle Aged MH - Male MH - Prospective Studies MH - *Scleroderma, Systemic/blood/physiopathology MH - *Capillaries/pathology/physiopathology/metabolism MH - Aged MH - Time Factors MH - *Raynaud Disease/physiopathology/blood/pathology MH - *Nails/blood supply MH - Adult MH - Up-Regulation MH - Treatment Outcome MH - Biomarkers/blood OTO - NOTNLM OT - Angiopoietin-1 OT - Capillaroscopy OT - Heating OT - Raynaud's phenomenon OT - Systemic sclerosis COIS- Declaration of competing interest Research expenses and labor costs for Y.S. and A.W. were provided by Kobayashi Pharmaceutical Co., Ltd. Additionally, N.I., T.M., E.K., and Y.M. received support from Kobayashi Pharmaceutical Co., Ltd. A.K. declares no conflicts of interest regarding this paper. EDAT- 2024/11/13 13:50 MHDA- 2024/11/30 11:17 CRDT- 2024/11/08 19:25 PHST- 2024/08/05 00:00 [received] PHST- 2024/11/04 00:00 [revised] PHST- 2024/11/05 00:00 [accepted] PHST- 2024/11/30 11:17 [medline] PHST- 2024/11/13 13:50 [pubmed] PHST- 2024/11/08 19:25 [entrez] AID - S0026-2862(24)00110-9 [pii] AID - 10.1016/j.mvr.2024.104761 [doi] PST - ppublish SO - Microvasc Res. 2025 Jan;157:104761. doi: 10.1016/j.mvr.2024.104761. Epub 2024 Nov 7. PMID- 22204894 OWN - NLM STAT- MEDLINE DCOM- 20140911 LR - 20131126 IS - 1768-319X (Electronic) IS - 0294-1260 (Linking) VI - 58 IP - 6 DP - 2013 Dec TI - [Botulinum toxin type A contribution in the treatment of Raynaud's phenomenon due to systemic sclerosis]. PG - 658-62 LID - S0294-1260(11)00183-X [pii] LID - 10.1016/j.anplas.2011.11.001 [doi] AB - AIMS: Raynaud's phenomenon is a vasospastic disorder of the extremities that can lead, in the hands, to pain, disability, ischemic ulcers and digital chronic ischemia. Medical and surgical current treatments are not fully effective while causing side effects. Recent studies have emphasized the value of botulinum toxin type A (BTX A) in the management of primary Raynaud's phenomenon. The originality of Raynaud's syndrome secondary to systemic sclerosis is to combine both arterial vasospasm and sclerosis of the arterial wall, what is supposed to reduce BTX A effects. The purpose of this work is to evaluate BTX A efficiency in patients with Raynaud's phenomenon secondary to systemic sclerosis. PATIENTS AND METHOD: We performed a prospective study for 12 months. Patients with severe Raynaud's phenomenon due to systemic sclerosis were injected with BTX A in the two hands. Evolution of ischemic ulcers, QuickDASH Score, O2 partial pressure, pain were measured before and 30 days after injection. RESULTS: We treated 18 patients. Thirty days after injection, we noticed a complete healing of ulcers, QuickDASH Score was improved from 39.4 to 20, as the O2 partial pressure from 16 to 42 mmHg and the pain from VNS from 6/10 to 2/10. CONCLUSION: BTX A appears to improve significantly Raynaud's phenomenon symptomatology in patients with systemic sclerosis despite the component of arterial sclerosis. CI - Copyright © 2011 Elsevier Masson SAS. All rights reserved. FAU - Serri, J AU - Serri J AD - Service de chirurgie de la main, CHU Conception, 145, boulevard Baille, 13005 Marseille, France. FAU - Legré, R AU - Legré R FAU - Veit, V AU - Veit V FAU - Guardia, C AU - Guardia C FAU - Gay, A-M AU - Gay AM LA - fre PT - English Abstract PT - Journal Article TT - Intérêt de la toxine botulinique de type A dans le traitement des syndromes de Raynaud sévères secondaires à la sclérodermie systémique. DEP - 20111226 PL - France TA - Ann Chir Plast Esthet JT - Annales de chirurgie plastique et esthetique JID - 8305839 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*therapeutic use MH - Disability Evaluation MH - Female MH - Fingers/blood supply MH - Humans MH - Injections MH - Male MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Prospective Studies MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology MH - Visual Analog Scale MH - Young Adult OTO - NOTNLM OT - Botulinum toxin type A OT - Raynaud's phenomenon OT - Sclérodermie systémique OT - Syndrome de Raynaud OT - Systemic sclerosis OT - Toxine botulinique de type A EDAT- 2011/12/30 06:00 MHDA- 2014/09/12 06:00 CRDT- 2011/12/30 06:00 PHST- 2011/07/06 00:00 [received] PHST- 2011/11/13 00:00 [accepted] PHST- 2011/12/30 06:00 [entrez] PHST- 2011/12/30 06:00 [pubmed] PHST- 2014/09/12 06:00 [medline] AID - S0294-1260(11)00183-X [pii] AID - 10.1016/j.anplas.2011.11.001 [doi] PST - ppublish SO - Ann Chir Plast Esthet. 2013 Dec;58(6):658-62. doi: 10.1016/j.anplas.2011.11.001. Epub 2011 Dec 26. PMID- 18020090 OWN - NLM STAT- MEDLINE DCOM- 20080130 LR - 20181010 IS - 0310-057X (Print) IS - 0310-057X (Linking) VI - 35 IP - 4 DP - 2007 Aug TI - Successful long-term treatment of a patient with long-standing Raynaud's disease by an extradural bupivacaine block. PG - 618-9 FAU - Hashem, M AU - Hashem M FAU - Lewis, R AU - Lewis R LA - eng PT - Case Reports PT - Letter PL - United States TA - Anaesth Intensive Care JT - Anaesthesia and intensive care JID - 0342017 RN - 0 (Anesthetics, Local) RN - Y8335394RO (Bupivacaine) SB - IM MH - Anesthetics, Local/*administration & dosage MH - Bupivacaine/*administration & dosage MH - Female MH - Humans MH - Injections, Epidural MH - Middle Aged MH - *Nerve Block MH - Raynaud Disease/*surgery MH - Time Factors MH - Treatment Outcome EDAT- 2007/11/21 09:00 MHDA- 2008/01/31 09:00 CRDT- 2007/11/21 09:00 PHST- 2007/11/21 09:00 [pubmed] PHST- 2008/01/31 09:00 [medline] PHST- 2007/11/21 09:00 [entrez] AID - 2007198 [pii] PST - ppublish SO - Anaesth Intensive Care. 2007 Aug;35(4):618-9. PMID- 29164658 OWN - NLM STAT- MEDLINE DCOM- 20180910 LR - 20220330 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 45 IP - 3 DP - 2018 Mar TI - Successful treatment of Raynaud's phenomenon and digital ulcers in systemic sclerosis patients with botulinum toxin B injection: Assessment of peripheral vascular disorder by angiography and dermoscopic image of nail fold capillary. PG - 349-352 LID - 10.1111/1346-8138.14140 [doi] AB - We recently identified the efficacy and safety of a botulinum toxin (BTX)-A/B in Raynaud's phenomenon (RP) and digital ulcers (DU) in Japanese patients with systemic sclerosis (SSc). Detailed assessments of peripheral vascular disorder using angiography and dermoscopic images of nail fold capillaries have not been performed previously. This study aimed to evaluate the effect of BTX-B on SSc-associated peripheral vascular disorder. Two SSc patients who suffered with RP and DU were treated with a BTX-B injection, and thereafter the symptoms of RP were improved and DU healed in both patients. Furthermore, angiography showed an increased blood flow to the palm and fingers, and dermoscopic images of nail fold capillary changes showed improvement. These results suggest that a BTX-B injection may increase peripheral blood flow and improve RP and DU in SSc patients. CI - © 2017 Japanese Dermatological Association. FAU - Motegi, Sei-Ichiro AU - Motegi SI AUID- ORCID: 0000-0001-8286-0669 AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Sekiguchi, Akiko AU - Sekiguchi A AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Saito, Shintaro AU - Saito S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Ishibuchi, Hirohisa AU - Ishibuchi H AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Kishi, Chikako AU - Kishi C AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Yasuda, Masahito AU - Yasuda M AUID- ORCID: 0000-0002-2907-640X AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Ishikawa, Osamu AU - Ishikawa O AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20171122 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Acetylcholine Release Inhibitors) RN - 0Y70779M1F (rimabotulinumtoxinB) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Acetylcholine Release Inhibitors/*therapeutic use MH - Adult MH - Aged MH - Angiography MH - Botulinum Toxins, Type A/*therapeutic use MH - Capillaries/diagnostic imaging MH - Dermoscopy/methods MH - Female MH - Fingers/blood supply/diagnostic imaging MH - Humans MH - Injections MH - Microscopic Angioscopy MH - Raynaud Disease/diagnostic imaging/*drug therapy/etiology MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/diagnostic imaging/*drug therapy/etiology MH - Treatment Outcome OTO - NOTNLM OT - Raynaud's phenomenon OT - angiography OT - botulinum toxin B OT - digital ulcers OT - systemic sclerosis EDAT- 2017/11/23 06:00 MHDA- 2018/09/11 06:00 CRDT- 2017/11/23 06:00 PHST- 2017/10/03 00:00 [received] PHST- 2017/10/20 00:00 [accepted] PHST- 2017/11/23 06:00 [pubmed] PHST- 2018/09/11 06:00 [medline] PHST- 2017/11/23 06:00 [entrez] AID - 10.1111/1346-8138.14140 [doi] PST - ppublish SO - J Dermatol. 2018 Mar;45(3):349-352. doi: 10.1111/1346-8138.14140. Epub 2017 Nov 22. PMID- 40930801 OWN - NLM STAT- MEDLINE DCOM- 20250910 LR - 20250914 IS - 2051-817X (Electronic) IS - 2051-817X (Linking) VI - 13 IP - 17 DP - 2025 Sep TI - Skin transient receptor potential channels expression and microvascular reactivity to cooling in primary and secondary Raynaud's phenomenon. PG - e70557 LID - 10.14814/phy2.70557 [doi] LID - e70557 AB - Temperature-sensitive Transient Receptor Potential (TRP) channels contribute to modulating skin vascular tone. Their role in Raynaud's Phenomenon (RP) remains unknown. We aimed to investigate TRPs expression in the skin, along with microvascular reactivity to cooling in patients with primary and secondary RP, compared with healthy subjects. Skin blood flow was measured in 10 regions of interest on the dorsum of the hand before, during, and after a 30-min cooling at 8°C. Skin biopsies were performed from a subset of participants at the distal phalanx before and after cooling. RNAs were extracted, sequenced, and TRPs mRNA expression quantified. TRPs mRNA were successfully quantified, but no significant differences in their expression were observed between groups, or in response to cooling. There was a decreased perfusion at the distal phalanx over time, in secondary RP compared with primary RP (adjusted p = 0.03) or healthy subjects (adjusted p = 0.042). Gene expression profiles were more similar between primary RP and healthy subjects than with secondary RP. This study shows an impairment of microvascular reactivity to cold in secondary, but not in primary RP. Transcriptomic data suggest involvement of the NO pathway in secondary RP, while TRP channel expression appears unchanged; however, their function could be impaired, which should be further investigated. CI - © 2025 The Author(s). Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society. FAU - Guigui, A AU - Guigui A AUID- ORCID: 0000-0002-7203-8134 AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, CIC 1406, Grenoble, France. FAU - Hodaj, E AU - Hodaj E AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, CIC 1406, Grenoble, France. FAU - Rendu, J AU - Rendu J AD - Univ. Grenoble Alpes, Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Grenoble, France. FAU - Bidart, M AU - Bidart M AD - UM Génétique Moléculaire: Maladies Héréditaires et Oncologie, University Hospital Grenoble Alpes, Grenoble, France. AD - INSERM U1209, CNRS UMR 5309, Institute for Advanced Biosciences, Grenoble Alpes University, Grenoble, France. FAU - Petre, G AU - Petre G AD - UMR1205, Brain Tech Lab, Grenoble Alpes University, Grenoble, France. FAU - Pluchart, H AU - Pluchart H AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, CIC 1406, Grenoble, France. FAU - Coutton, C AU - Coutton C AD - INSERM U1209, CNRS UMR 5309, Institute for Advanced Biosciences, Grenoble Alpes University, Grenoble, France. AD - UM de Génétique Chromosomique, University Hospital Grenoble Alpes, Grenoble, France. FAU - Coste, B AU - Coste B AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, CIC 1406, Grenoble, France. FAU - Cracowski, J L AU - Cracowski JL AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Pharmacovigilance Unit, CHU Grenoble Alpes, Univ. Grenoble Alpes, Grenoble, France. FAU - Blaise, S AU - Blaise S AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Department of Vascular Medicine, CHU Grenoble Alpes, Univ. Grenoble Alpes, Grenoble, France. FAU - Roustit, M AU - Roustit M AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, CIC 1406, Grenoble, France. LA - eng GR - Association des sclerodermiques de France/ GR - CHU Grenoble Alpes/ PT - Journal Article PL - United States TA - Physiol Rep JT - Physiological reports JID - 101607800 RN - 0 (Transient Receptor Potential Channels) SB - IM MH - Humans MH - *Raynaud Disease/metabolism/physiopathology/genetics MH - Male MH - Female MH - *Skin/blood supply/metabolism MH - Adult MH - Cold Temperature MH - *Transient Receptor Potential Channels/metabolism/genetics MH - Middle Aged MH - *Microvessels/metabolism/physiopathology MH - Regional Blood Flow MH - Microcirculation PMC - PMC12422803 OTO - NOTNLM OT - Raynaud's phenomenon OT - TRP OT - microcirculation OT - systemic sclerosis COIS- Authors have no conflict of interest to declare for this study. The Institutional Review Board of Sud‐Est V, Grenoble, France (No. 6705), approved the protocols. Written informed consent was obtained from all subjects before participation. EDAT- 2025/09/11 00:28 MHDA- 2025/09/11 00:29 PMCR- 2025/09/10 CRDT- 2025/09/10 21:33 PHST- 2025/09/01 00:00 [revised] PHST- 2025/02/25 00:00 [received] PHST- 2025/09/01 00:00 [accepted] PHST- 2025/09/11 00:29 [medline] PHST- 2025/09/11 00:28 [pubmed] PHST- 2025/09/10 21:33 [entrez] PHST- 2025/09/10 00:00 [pmc-release] AID - PHY270557 [pii] AID - 10.14814/phy2.70557 [doi] PST - ppublish SO - Physiol Rep. 2025 Sep;13(17):e70557. doi: 10.14814/phy2.70557. PMID- 41523865 OWN - NLM STAT- In-Process LR - 20260604 IS - 1937-8688 (Electronic) VI - 52 DP - 2025 TI - [Castleman disease with systemic manifestation: a case report]. PG - 84 LID - 10.11604/pamj.2025.52.84.48274 [doi] LID - 84 AB - Castleman's disease is a rare lymphoproliferative disorder. It typically presents with lymphadenopathy and may be associated with sometimes severe systemic manifestations. It can occur in two clinical forms: the unicentric form, which is limited to a single lymph node or lymph node region, generally has a favorable prognosis and is primarily treated with surgical excision; and the multicentric form, which is more severe, characterized by diffuse lymph node involvement and excessive cytokine production, particularly interleukin-6 (IL-6), which accounts for the systemic symptoms. Treatment in the multicentric form includes monoclonal antibodies targeting IL-6, immunosuppressants, chemotherapy, or corticosteroids, depending on the underlying cause. The disease may be associated with human herpes virus 8 (HHV-8) infection, especially in immunocompromised patients. We report the case of a 65-year-old patient presenting with polyadenopathy and Raynaud's phenomenon. Imaging revealed multiple lymphadenopathies along with hepatosplenomegaly. An exploratory cervicotomy was performed, followed by histopathological examination and immunohistochemical analysis, which confirmed the diagnosis of multicentric Castleman disease. This clinical case underscores the importance of considering Castleman disease in the differential diagnosis of unexplained polyadenopathies, particularly when accompanied by atypical systemic manifestations. CI - Copyright: Ahlam Hmimsa et al. FAU - Hmimsa, Ahlam AU - Hmimsa A AD - Service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale, Hôpital Militaire Moulay Ismail, Meknès, Maroc. FAU - Touihem, Nabil AU - Touihem N AD - Service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale, Hôpital Militaire Moulay Ismail, Meknès, Maroc. FAU - Attifi, Hicham AU - Attifi H AD - Service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale, Hôpital Militaire Moulay Ismail, Meknès, Maroc. FAU - Hmidi, Mounir AU - Hmidi M AD - Service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale, Hôpital Militaire Moulay Ismail, Meknès, Maroc. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Maladie de Castleman avec manifestation systémique: à propos d’un cas. DEP - 20251024 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - Multi-centric Castleman's Disease SB - IM MH - Humans MH - *Castleman Disease/diagnosis/complications/pathology MH - Aged MH - *Raynaud Disease/etiology/diagnosis MH - Male MH - Diagnosis, Differential MH - *Lymphadenopathy/etiology/diagnosis MH - Hepatomegaly/etiology MH - Splenomegaly/etiology PMC - PMC12790396 OTO - NOTNLM OT - Castleman disease OT - Raynaud's phenomenon OT - case report OT - interleukin-6 OT - polyadenopathy COIS- Les auteurs ne déclarent aucun conflit d'intérêts. EDAT- 2026/01/12 06:29 MHDA- 2026/01/12 13:16 PMCR- 2025/10/24 CRDT- 2026/01/12 05:48 PHST- 2025/06/10 00:00 [received] PHST- 2025/07/29 00:00 [accepted] PHST- 2026/01/12 13:16 [medline] PHST- 2026/01/12 06:29 [pubmed] PHST- 2026/01/12 05:48 [entrez] PHST- 2025/10/24 00:00 [pmc-release] AID - PAMJ-52-84 [pii] AID - 10.11604/pamj.2025.52.84.48274 [doi] PST - epublish SO - Pan Afr Med J. 2025 Oct 24;52:84. doi: 10.11604/pamj.2025.52.84.48274. eCollection 2025. PMID- 19385450 OWN - NLM STAT- MEDLINE DCOM- 20090811 LR - 20090423 IS - 1025-9589 (Print) IS - 1025-9589 (Linking) VI - 20 IP - 2 DP - 2008 Apr-Jun TI - Efficacy of cervicothoracic sympathectomy versus conservative management in patients suffering from incapacitating Raynaud's syndrome after frost bite. PG - 21-4 AB - BACKGROUND: Raynaud's syndrome is a known complication of cold injuries. Stress, smoking and metabolic diseases may further aggravate the disease course. The purpose of this study was to determine the efficacy of Cervico-thoracic sympathectomy as compared to conservative management in severe Raynaud's syndrome after frostbite. METHODS: This non-randomized controlled trial was conducted at Railway Hospital, Rawalpindi and Islamic International Medical Complex, Islamabad between January 1999 and June 2006. All patients sustained severe cold trauma in the mountain ridges of Himalayas in Kashmir. In all cases, an informed consent was obtained from patients and families. All operations performed were free of charges. Out of the total 48 patients who developed incapacitating Raynaud's syndrome of the upper limbs after frost bite, 17 patients underwent thoracic sympathectomy through anterior supraclavicular route. Remaining 31 patients were treated conservatively and were placed in the control group. Data was collected on pre-designed proforma and assessed using SPSS (version 11). Chi-square test was applied to assess the effectiveness of the two treatment modalities. RESULTS: All operated cases initially showed improvement in symptoms and incapacitation. Among sympathectomised patients, 11 patients became symptom free and 3 patients showed mild but improved symptoms. Two patients after initial transient improvement developed incapacitating symptoms requiring further treatment, one patient developed gangrene ofdistal phalanx nine month after sympathectomy requiring amputation of the finger. Frequency of attacks and duration between the attacks reduced in all operated patients of cervical sympathectomy (p < 0.05) as compared to conservative management. CONCLUSION: Cervical sympathectomy is a very effective modality of treatment in patients having severe Raynaud's disease of upper limbs secondary to frost bite. FAU - Khan, Mohammad Iqbal AU - Khan MI AD - Department of Surgery, Islamic International Medical College, Rawalpindi, Pakistan. mikhandr@gmail.com FAU - Tariq, Mohammad AU - Tariq M FAU - Rehman, Ahmed AU - Rehman A FAU - Zafar, Afsheeen AU - Zafar A FAU - Sheen, Salman Najam AU - Sheen SN LA - eng PT - Controlled Clinical Trial PT - Journal Article PL - Pakistan TA - J Ayub Med Coll Abbottabad JT - Journal of Ayub Medical College, Abbottabad : JAMC JID - 8910750 SB - IM MH - Adolescent MH - Adult MH - Cervical Plexus/*surgery MH - Female MH - Frostbite/*complications MH - Humans MH - Male MH - Pakistan MH - Raynaud Disease/etiology/*surgery/therapy MH - Sympathectomy/*methods MH - Thoracic Nerves/*surgery MH - Time Factors MH - Young Adult EDAT- 2009/04/24 09:00 MHDA- 2009/08/12 09:00 CRDT- 2009/04/24 09:00 PHST- 2009/04/24 09:00 [entrez] PHST- 2009/04/24 09:00 [pubmed] PHST- 2009/08/12 09:00 [medline] PST - ppublish SO - J Ayub Med Coll Abbottabad. 2008 Apr-Jun;20(2):21-4. PMID- 33547102 OWN - NLM STAT- MEDLINE DCOM- 20210222 LR - 20230919 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 14 IP - 2 DP - 2021 Feb 5 TI - Sudden onset peripheral visual deficit secondary to retinal artery spasm in Raynaud's phenomenon. LID - 10.1136/bcr-2020-239954 [doi] LID - e239954 AB - A 32-year-old doctor, who has a medical history of primary Raynaud's disease and previous scotomas, presented to eye clinic with sudden onset blurring of vision (infero-nasally) with no other associated symptoms. The patient had good visual acuity bilaterally (6/6) and no anterior chamber activity or conjunctival hyperaemia. Findings consistent with a nerve fibre layer infarct were noted in the right eye, with unremarkable examination of the left eye. Optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) images were obtained, which showed an area of capillary shut down in keeping with a nerve fibre layer lesion. Previous literature pertaining to similar symptoms is sparse with symptoms such as migraines, epilepsy and visual loss being stated. This case provides further evidence of Raynaud's associated retinal artery spasm, with complete resolution at 4 weeks. We also demonstrate the accessibility of OCT and more importantly OCTA for investigation of sudden onset visual deficit. CI - © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Ansari, Yousuf AU - Ansari Y AD - Department of Ophthalmology, Queen Elizabeth Hospital Birmingham,University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK yousufansari@nhs.net. AD - Department of Ophthalmology, Birmingham and Midland Eye Centre, Sandwell and West Birmingham Hospitals NHS trust,Birmingham, UK, Birmingham, UK. FAU - Kale, Aditya Uday AU - Kale AU AUID- ORCID: 0000-0002-2186-1446 AD - Department of Ophthalmology, Queen Elizabeth Hospital Birmingham,University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK. FAU - Tallouzi, Mohammad O AU - Tallouzi MO AD - Department of Ophthalmology, Birmingham and Midland Eye Centre, Sandwell and West Birmingham Hospitals NHS trust,Birmingham, UK, Birmingham, UK. AD - Institute of Applied Health Research, University of Birmingham College of Medical and Dental Sciences, Birmingham, UK. FAU - Manna, Avinash AU - Manna A AD - Department of Ophthalmology, Queen Elizabeth Hospital Birmingham,University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20210205 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Adult MH - Diagnosis, Differential MH - Female MH - Humans MH - Raynaud Disease/*complications MH - *Retinal Artery MH - Retinal Diseases/*etiology MH - Spasm/*etiology MH - Vascular Diseases/*etiology PMC - PMC7871241 OTO - NOTNLM OT - ophthalmology OT - retina OT - rheumatology COIS- Competing interests: None declared. EDAT- 2021/02/07 06:00 MHDA- 2021/02/23 06:00 PMCR- 2023/02/05 CRDT- 2021/02/06 05:34 PHST- 2021/02/06 05:34 [entrez] PHST- 2021/02/07 06:00 [pubmed] PHST- 2021/02/23 06:00 [medline] PHST- 2023/02/05 00:00 [pmc-release] AID - 14/2/e239954 [pii] AID - bcr-2020-239954 [pii] AID - 10.1136/bcr-2020-239954 [doi] PST - epublish SO - BMJ Case Rep. 2021 Feb 5;14(2):e239954. doi: 10.1136/bcr-2020-239954. PMID- 18477675 OWN - NLM STAT- MEDLINE DCOM- 20080618 LR - 20080703 IS - 1468-3288 (Electronic) IS - 0017-5749 (Linking) VI - 57 IP - 6 DP - 2008 Jun TI - An unusual cause of abdominal fullness in a patient with Raynaud's phenomenon. Mixed connective tissue disease. PG - 733, 820 LID - 10.1136/gut.2007.124701 [doi] FAU - Hokama, A AU - Hokama A AD - First Department of Internal Medicine, University of the Ryukyus, 207 Uehara, Nishihara, Okinawa 903-0215, Japan. hokama-a@med.u-ryukyu.ac.jp FAU - Kishimoto, K AU - Kishimoto K FAU - Nakamoto, M AU - Nakamoto M FAU - Kinjo, N AU - Kinjo N FAU - Kinjo, F AU - Kinjo F FAU - Fujita, J AU - Fujita J LA - eng PT - Case Reports PT - Journal Article PL - England TA - Gut JT - Gut JID - 2985108R SB - IM MH - Adult MH - Dilatation, Pathologic/diagnosis/etiology MH - Duodenoscopy MH - Duodenum/pathology MH - Female MH - Humans MH - Mixed Connective Tissue Disease/complications/*diagnosis MH - Raynaud Disease/*complications EDAT- 2008/05/15 09:00 MHDA- 2008/06/19 09:00 CRDT- 2008/05/15 09:00 PHST- 2008/05/15 09:00 [pubmed] PHST- 2008/06/19 09:00 [medline] PHST- 2008/05/15 09:00 [entrez] AID - 57/6/733 [pii] AID - 10.1136/gut.2007.124701 [doi] PST - ppublish SO - Gut. 2008 Jun;57(6):733, 820. doi: 10.1136/gut.2007.124701. PMID- 17694282 OWN - NLM STAT- MEDLINE DCOM- 20071130 LR - 20101118 IS - 0723-5003 (Print) IS - 0723-5003 (Linking) VI - 102 IP - 8 DP - 2007 Aug 15 TI - [Novel indications for phosphodiesterase type 5 inhibitors]. PG - 617-30 AB - Phosphodiesterase type 5 (PDE5) induces the breakdown of cyclic guanosine monophosphate (cGMP) in smooth muscle cells. Hence, PDE5 inhibitors promote vasodilative effects by enhancing intracellular cGMP levels. Three PDE5 inhibitors, sildenafil, vardenafil, and tadalafil, have been approved for the treatment of "erectile dysfunction" (ED). All three show an excellent effectiveness regarding ED therapy, but differ from one another regarding their pharmacokinetic properties. Recent experimental studies and clinical trials indicate that PDE5 inhibitors are also effective in the treatment of various other diseases such as pulmonary arterial hypertension (PAH), Raynaud's disease, gastrointestinal disorders, and stroke, and furthermore exert cardioprotective effects. This review describes the cardiovascular safety of PDE5 inhibitors and provides an overview of the current literature regarding potential novel indications. FAU - Rosenkranz, Stephan AU - Rosenkranz S AD - Klinik III für Innere Medizin, Universität zu Köln, Köln. stephan.rosenkrantz@uk-koeln.de FAU - Caglayan, Evren AU - Caglayan E FAU - Erdmann, Erland AU - Erdmann E LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Neue Indikationen für Phosphodiesterase-5-Inhibitoren. PL - Germany TA - Med Klin (Munich) JT - Medizinische Klinik (Munich, Germany : 1983) JID - 8303501 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Cerebral Infarction/drug therapy MH - Clinical Trials as Topic MH - Erectile Dysfunction/drug therapy MH - Gastrointestinal Diseases/drug therapy MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Male MH - *Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/adverse effects/*therapeutic use MH - Raynaud Disease/drug therapy MH - Stroke/drug therapy MH - Vasodilator Agents/adverse effects/*therapeutic use RF - 124 EDAT- 2007/08/19 09:00 MHDA- 2007/12/06 09:00 CRDT- 2007/08/19 09:00 PHST- 2006/10/31 00:00 [received] PHST- 2007/05/25 00:00 [accepted] PHST- 2007/08/19 09:00 [pubmed] PHST- 2007/12/06 09:00 [medline] PHST- 2007/08/19 09:00 [entrez] AID - 10.1007/s00063-007-1078-4 [doi] PST - ppublish SO - Med Klin (Munich). 2007 Aug 15;102(8):617-30. doi: 10.1007/s00063-007-1078-4. PMID- 22043689 OWN - NLM STAT- MEDLINE DCOM- 20111220 LR - 20111102 IS - 0255-2930 (Print) IS - 0255-2930 (Linking) VI - 31 IP - 10 DP - 2011 Oct TI - [Acupuncture combined with finger cupping therapy for Raynaud's disease]. PG - 931 FAU - Li, Min-Lan AU - Li ML AD - liminlankuaile@163.com FAU - Liu, Jian-Yu AU - Liu JY FAU - Chen, Qiang AU - Chen Q LA - chi PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China TA - Zhongguo Zhen Jiu JT - Zhongguo zhen jiu = Chinese acupuncture & moxibustion JID - 8600658 SB - IM MH - Acupuncture Points MH - *Acupuncture Therapy MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*therapy EDAT- 2011/11/03 06:00 MHDA- 2011/12/21 06:00 CRDT- 2011/11/03 06:00 PHST- 2011/11/03 06:00 [entrez] PHST- 2011/11/03 06:00 [pubmed] PHST- 2011/12/21 06:00 [medline] PST - ppublish SO - Zhongguo Zhen Jiu. 2011 Oct;31(10):931. PMID- 27587939 OWN - NLM STAT- MEDLINE DCOM- 20170414 LR - 20221207 IS - 2413-7170 (Electronic) IS - 1029-1857 (Print) IS - 1029-1857 (Linking) VI - 26 IP - 4 DP - 2016 Jul TI - Post-traumatic Digital Gangrene Associated with Epinephrine Use in Primary Raynaud's Phenomenon: Lesson for the Future. PG - 401-4 AB - BACKGROUND: Following digital surgical procedures, the ensuing post-operative course may be complicated by the presence of underlying ischaemic or vasospastic process. In the presence of such conditions, post-operative ischaemic changes may be further exacerbated with the use of local anaesthetics in combination with epinephrine. CASE DETAILS: We report a 21 year-old female who presented with an amputated fifth digit due to a rapidly spreading gangrene which started immediately after the surgical repair of a traumatic laceration which was infiltrated with a pre-mixed solution of lignocaine and epinephrine 3 hours earlier. The patient's final diagnosis was epinephrine-associated digital gangrene in the background of primary Raynaud's Phenomenon (RP). CONCLUSION: The author reports this case in order to reiterate the importance of thorough clinical evaluation prior to the use of epinephrine in digital anaesthesia as well as to increase awareness on how primary RP can be complicated by gangrene. FAU - Sama, Carlson-Babila AU - Sama CB AD - Galactic Corps Research Group (GCRG) and Department of Clinical Sciences, Faculty of Health Sciences, University of Buea, Buea, South-West Region, Cameroon. LA - eng PT - Case Reports PT - Journal Article PL - Ethiopia TA - Ethiop J Health Sci JT - Ethiopian journal of health sciences JID - 101224773 RN - 0 (Vasoconstrictor Agents) RN - YKH834O4BH (Epinephrine) SB - IM MH - Adult MH - Amputation, Surgical/*adverse effects/methods MH - Anesthesia/*adverse effects/methods MH - Epinephrine/*adverse effects/therapeutic use MH - Female MH - Fingers/*pathology/surgery MH - Gangrene/diagnosis/*etiology MH - Humans MH - Lacerations/*surgery MH - Raynaud Disease/*complications/diagnosis MH - Vasoconstriction MH - Vasoconstrictor Agents/adverse effects MH - Young Adult PMC - PMC4992781 OTO - NOTNLM OT - Digital gangrene OT - Raynaud's phenomenon OT - epinephrine OT - trauma EDAT- 2016/09/03 06:00 MHDA- 2017/04/15 06:00 PMCR- 2016/07/01 CRDT- 2016/09/03 06:00 PHST- 2016/09/03 06:00 [entrez] PHST- 2016/09/03 06:00 [pubmed] PHST- 2017/04/15 06:00 [medline] PHST- 2016/07/01 00:00 [pmc-release] AID - jEJHS.v26.i4.pg401 [pii] AID - 10.4314/ejhs.v26i4.13 [doi] PST - ppublish SO - Ethiop J Health Sci. 2016 Jul;26(4):401-4. doi: 10.4314/ejhs.v26i4.13. PMID- 25889905 OWN - NLM STAT- MEDLINE DCOM- 20160120 LR - 20220408 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 17 IP - 1 DP - 2015 Mar 22 TI - Application of the 2013 ACR/EULAR classification criteria for systemic sclerosis to patients with Raynaud's phenomenon. PG - 77 LID - 10.1186/s13075-015-0594-5 [doi] LID - 77 AB - INTRODUCTION: We investigated how many patients, who presented with Raynaud's phenomenon (RP) and who had not been classified as systemic sclerosis (SSc), would be reclassified as SSc, if the 2013 American College of Rheumatology (ACR)/the European League Against Rheumatism (EULAR) classification criteria were used. We also analyzed the predictive values of the reclassification as SSc in those patients. METHODS: We consecutively enrolled 64 patients with RP and 60 patients with SSc. We applied the new classification criteria to them, reclassified them, and compared variables between those who were newly classified as SSc and those who were not or previously classified as SSc. RESULTS: Seventeen of 64 patients (26.5%), who presented with RP, but did not fulfill the 1980 ACR classification criteria, were newly classified as SSc by the 2013 ACR/EULAR classification criteria. The newly classified patients as SSc showed increased frequencies of sclerodactyly, digital tip ulcer, telangiectasia, abnormal nailfold capillaries and the presence of anti-centromere antibody, compared to those not and telangiectasia and anti-centromere antibody, compared to the previously classified patients. For the reclassification as SSc, the variables with independent predictive value were sclerodactyly (odds ratio (OR) 60.025), telangiectasia (OR 13.353) and the presence of anti-centromere antibody (OR 11.168). CONCLUSIONS: Overall, 26.5% of the patients, who presented with RP, but who did not fulfill the 1980 ACR classification criteria, were newly classified as SSc according to the 2013 ACR/EULAR classification criteria. Sclerodactyly, telangiectasia, and the presence of anti-centromere antibody had independent predictive value for reclassifying patients with RP as SSc. FAU - Park, Jin-Su AU - Park JS AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. mooldool@yuhs.ac. FAU - Park, Min-Chan AU - Park MC AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. mcpark@yuhs.ac. FAU - Song, Jason Jungsik AU - Song JJ AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. jsksong@yuhs.ac. FAU - Park, Yong-Beom AU - Park YB AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. yongbpark@yuhs.ac. FAU - Lee, Soo-Kon AU - Lee SK AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. sookonlee@yuhs.ac. FAU - Lee, Sang-Won AU - Lee SW AD - Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, South Korea. sangwonlee@yuhs.ac. LA - eng PT - Journal Article DEP - 20150322 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Adult MH - Aged MH - Europe/epidemiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*classification/*diagnosis/epidemiology MH - Rheumatology/*classification/standards MH - Scleroderma, Systemic/*classification/*diagnosis/epidemiology MH - United States/epidemiology PMC - PMC4384278 EDAT- 2015/04/19 06:00 MHDA- 2016/01/21 06:00 PMCR- 2015/03/22 CRDT- 2015/04/19 06:00 PHST- 2014/07/19 00:00 [received] PHST- 2015/03/11 00:00 [accepted] PHST- 2015/04/19 06:00 [entrez] PHST- 2015/04/19 06:00 [pubmed] PHST- 2016/01/21 06:00 [medline] PHST- 2015/03/22 00:00 [pmc-release] AID - 10.1186/s13075-015-0594-5 [pii] AID - 594 [pii] AID - 10.1186/s13075-015-0594-5 [doi] PST - epublish SO - Arthritis Res Ther. 2015 Mar 22;17(1):77. doi: 10.1186/s13075-015-0594-5. PMID- 25428518 OWN - NLM STAT- MEDLINE DCOM- 20150904 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 34 IP - 1 DP - 2015 Jan TI - Anti-nuclear antibodies as predictor of outcome in a multi-center cohort of Italian children and adolescents with Raynaud's phenomenon. PG - 167-9 LID - 10.1007/s10067-014-2833-6 [doi] AB - A retrospective multi-center data collection of clinical, laboratory, and treatment characteristics of 94 Caucasian children and adolescents with Raynaud's phenomenon (RP) started at a mean age of 12.8 ± 5 years, with variable involvement of hands, feet, and face, was performed for a period of 3 years. Collected data included nailfold videocapillaroscopy (NVC), lung function tests, and different laboratory tests finalized to characterize an eventual connective tissue disease (CTD), disclosed by RP itself. Twelve patients presented an early-scleroderma pattern at NVC, 1 a late-scleroderma pattern, and 58 a nonspecific pattern. Laboratory data results showed the positivity of anti-nuclear antibodies (ANA) in 29 % of patients. After this 3-year period of observation, 8 patients had developed a CTD. Our data examined by multivariate analysis, though limited to a multi-center cohort of pediatric patients with RP, strongly suggest that ANA positivity is a significant predictor of progression of RP towards a CTD. FAU - Falcini, Fernanda AU - Falcini F AD - Department of Experimental and Clinical Medicine and Department of Biomedicine, Division of Rheumatology AOUC & Transition Clinic, University of Florence, Viale Pieraccini 18, 50139, Florence, Italy, falcini@unifi.it. FAU - Rigante, Donato AU - Rigante D FAU - Candelli, Marcello AU - Candelli M FAU - Martini, Giorgia AU - Martini G FAU - Corona, Fabrizia AU - Corona F FAU - Petaccia, Antonella AU - Petaccia A FAU - La Torre, Francesco AU - La Torre F FAU - Raffaele, Carmela G L AU - Raffaele CG FAU - Matucci Cerinic, Marco AU - Matucci Cerinic M LA - eng PT - Journal Article DEP - 20141128 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Antibodies, Antinuclear/*blood MH - Child MH - Child, Preschool MH - Connective Tissue Diseases/blood/*diagnosis/immunology MH - Disease Progression MH - Female MH - Humans MH - Male MH - Raynaud Disease/blood/*immunology MH - Retrospective Studies EDAT- 2014/11/28 06:00 MHDA- 2015/09/05 06:00 CRDT- 2014/11/28 06:00 PHST- 2014/11/09 00:00 [received] PHST- 2014/11/14 00:00 [accepted] PHST- 2014/11/28 06:00 [entrez] PHST- 2014/11/28 06:00 [pubmed] PHST- 2015/09/05 06:00 [medline] AID - 10.1007/s10067-014-2833-6 [doi] PST - ppublish SO - Clin Rheumatol. 2015 Jan;34(1):167-9. doi: 10.1007/s10067-014-2833-6. Epub 2014 Nov 28. PMID- 24144459 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - Sex and time to diagnosis in systemic sclerosis: an updated analysis of 1,129 patients from the Canadian scleroderma research group registry. PG - S-10-4 AB - OBJECTIVES: A previous study found that time to diagnosis was significantly longer from onset of Raynaud's phenomenon for women compared to men with diffuse systemic sclerosis (SSc) and that, in limited SSc, it was more than twice as long for women than men. That study was limited, however, by the small number of men in disease subtype subgroups. The objective of the present study was to investigate the association of sex with time to diagnosis of SSc using a substantially larger patient sample. METHODS: Association between sex and time to diagnosis was assessed overall and stratified based on diffuse versus limited disease using Kaplan-Meier curves and Cox proportional hazards models. RESULTS: There were 1,129 patients in the study (median age=56.0 years; 978 [86.6%] women). Time to diagnosis was significantly longer for women (median=1.1 years) than men (median 0.8= years; p=0.037) with diffuse SSc following onset of Raynaud's phenomenon. There were no significant or substantive sex differences in time to diagnosis after Raynaud's onset in limited SSc or from onset of first non-Raynaud's disease manifestation in diffuse or limited SSc. CONCLUSIONS: Time to diagnosis was significantly longer for women compared to men with diffuse SSc following onset of Raynaud's phenomenon, but the difference was small and unlikely to be clinically significant. There were no differences in time to diagnosis following Raynaud's onset in limited disease or following onset of first non-Raynaud's disease manifestation in diffuse or limited disease. Overall, sex does not appear to influence time to diagnosis meaningfully. FAU - Delisle, V C AU - Delisle VC AD - Department of Educational and Counselling Psychology, McGill University and Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada. vanessa.delisle@mail.mcgill.ca. FAU - Hudson, M AU - Hudson M FAU - Baron, M AU - Baron M FAU - Thombs, B D AU - Thombs BD FAU - And The Canadian Scleroderma Research Group, A AU - And The Canadian Scleroderma Research Group A LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131011 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Age Factors MH - Aged MH - Canada MH - Delayed Diagnosis/*statistics & numerical data MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/etiology MH - *Registries MH - Scleroderma, Diffuse/complications/*diagnosis MH - Scleroderma, Limited/complications/*diagnosis MH - Scleroderma, Systemic/complications/diagnosis MH - Sex Factors EDAT- 2013/10/23 06:00 MHDA- 2015/10/31 06:00 CRDT- 2013/10/23 06:00 PHST- 2013/03/23 00:00 [received] PHST- 2013/07/19 00:00 [accepted] PHST- 2013/10/23 06:00 [entrez] PHST- 2013/10/23 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 7030 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-10-4. Epub 2013 Oct 11. PMID- 16484290 OWN - NLM STAT- MEDLINE DCOM- 20061107 LR - 20131121 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 IP - 8 DP - 2006 Aug TI - Iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol. PG - 999-1004 AB - OBJECTIVES: To evaluate the clinical efficacy and the effects on the quality of life of iloprost, a prostacyclin analogue, used according to a new protocol in patients with Raynaud's phenomenon secondary to systemic sclerosis. METHODS: In this randomized study, we treated 30 patients with iloprost, given by intravenous infusion, at progressively increasing doses (from 0.5 to 2 ng/kg/min) over a period of 6 h each day for 10 days in two consecutive weeks, with repeated cycles at regular intervals of 3 months for 18 months. The results were compared with those obtained in 30 other patients who received the same drug but with different dosing schemes. RESULTS: The total average daily duration of the attacks, the average duration of a single attack and the average daily frequency of the attacks were reduced significantly in all treatment groups, but the comparison between the groups demonstrated significant differences between patients treated with the new protocol and the others at later times (12 and 18 months). The effects on the quality of life in the group treated with the new protocol, evaluated with the Short Form-36, demonstrated a marked improvement regarding both the scale relating to the physical aspect of the illness and, especially, the scale relating to the mental aspect. CONCLUSIONS: In patients with systemic sclerosis, cyclic intravenous iloprost infusion is efficacious in the treatment of Raynaud's phenomenon. The protocol that we used, compared with others, not only has favourable clinical effects but also leads to a marked improvement in the quality of life. FAU - Milio, G AU - Milio G AD - Department of Internal Medicine and Cardiovascular Diseases, University of Palermo, Palermo, Italy. glaucomilio@libero.it FAU - Corrado, E AU - Corrado E FAU - Genova, C AU - Genova C FAU - Amato, C AU - Amato C FAU - Raimondi, F AU - Raimondi F FAU - Almasio, P L AU - Almasio PL FAU - Novo, S AU - Novo S LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20060216 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Female MH - Humans MH - Iloprost/*therapeutic use MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Prospective Studies MH - *Quality of Life MH - Raynaud Disease/*drug therapy/etiology/psychology MH - Scleroderma, Systemic/*complications/psychology MH - Severity of Illness Index MH - Time Factors MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use EDAT- 2006/02/18 09:00 MHDA- 2006/11/09 09:00 CRDT- 2006/02/18 09:00 PHST- 2006/02/18 09:00 [pubmed] PHST- 2006/11/09 09:00 [medline] PHST- 2006/02/18 09:00 [entrez] AID - kel038 [pii] AID - 10.1093/rheumatology/kel038 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Aug;45(8):999-1004. doi: 10.1093/rheumatology/kel038. Epub 2006 Feb 16. PMID- 21157301 OWN - NLM STAT- MEDLINE DCOM- 20110601 LR - 20101223 IS - 1473-5733 (Electronic) IS - 0957-5235 (Linking) VI - 22 IP - 1 DP - 2011 Jan TI - Polymorphism of clotting factors in Hungarian patients with Raynaud's phenomenon. PG - 56-9 LID - 10.1097/MBC.0b013e32834234fe [doi] AB - Patients with primary Raynaud's phenomenon may have a genetically determined risk for clotting factors that predispose them to aberrant microvascular thrombosis. We investigated the prevalence of factor V substitution of G to A at position 1691 (FVLeiden), prothrombin G20210A, and methyltetrahydrofolate reductase C677T mutations in these patients. Two hundred (158 women, 42 men, mean age of 42.4 ± 13.7 years) consecutive patients with primary Raynaud's phenomenon and 200 age-sex-matched healthy controls of Hungarian origin were included in a case-control study. The prevalence of methyltetrahydrofolate reductase C677T homozygous among patients was significantly lower than in the control group (odds ratio 0.4, 95% confidence interval 0.2-0.9, P < 0.05). The prevalence of other thrombosis-associated alleles did not differ between patients with primary Raynaud's phenomenon and control subjects. FVLeiden, prothrombin G20210A, and polymorphism, prothrombin G20210A mutations have no apparent effect on the etiology of primary Raynaud's phenomenon. FAU - Shemirani, Amir-Houshang AU - Shemirani AH AD - Central Laboratory, Erzsébet Hospital, Sátoraljaújhely, Hungary. FAU - Szomják, Edit AU - Szomják E FAU - Balogh, Emese AU - Balogh E FAU - András, Csilla AU - András C FAU - Kovács, Dóra AU - Kovács D FAU - Acs, Judit AU - Acs J FAU - Csiki, Zoltán AU - Csiki Z LA - eng PT - Journal Article PL - England TA - Blood Coagul Fibrinolysis JT - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JID - 9102551 RN - 0 (factor V Leiden) RN - 9001-24-5 (Factor V) RN - 9001-26-7 (Prothrombin) RN - EC 1.5.1.20 (Methylenetetrahydrofolate Reductase (NADPH2)) SB - IM MH - Adult MH - Case-Control Studies MH - Factor V/*genetics MH - Female MH - Humans MH - Hungary/epidemiology MH - Male MH - Methylenetetrahydrofolate Reductase (NADPH2)/*genetics MH - Middle Aged MH - *Polymorphism, Genetic MH - Prothrombin/*genetics MH - Raynaud Disease/epidemiology/*genetics MH - Thrombosis/genetics EDAT- 2010/12/16 06:00 MHDA- 2011/06/02 06:00 CRDT- 2010/12/16 06:00 PHST- 2010/12/16 06:00 [entrez] PHST- 2010/12/16 06:00 [pubmed] PHST- 2011/06/02 06:00 [medline] AID - 10.1097/MBC.0b013e32834234fe [doi] PST - ppublish SO - Blood Coagul Fibrinolysis. 2011 Jan;22(1):56-9. doi: 10.1097/MBC.0b013e32834234fe. PMID- 32480268 OWN - NLM STAT- MEDLINE DCOM- 20200924 LR - 20200924 IS - 1873-4499 (Electronic) IS - 0899-7071 (Linking) VI - 66 DP - 2020 Oct TI - Dynamic CTA and MRA in a case of severe Raynaud's phenomenon. PG - 133-136 LID - S0899-7071(20)30155-8 [pii] LID - 10.1016/j.clinimag.2020.05.010 [doi] AB - Raynaud's phenomenon (RP) is a condition where arterial spasm, usually in the fingers, causes episodes of reduced blood flow. The condition is either idiopathic (primary) or related to a connective tissue disorder or drug response (secondary). We present a case of severe RP where we performed a novel-sequenced CTA and MRA during a prolonged active episode of peripheral vasospasm. Real-time multidisciplinary consultation resulted in appropriate therapy with symptoms alleviation within hours of presentation. CI - Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved. FAU - Hislop-Jambrich, Jacqueline L AU - Hislop-Jambrich JL AD - Centre for Medical Research and Development, Canon Medical, Building C, 12-24 Talavera Road, North Ryde, NSW 2113, Australia. Electronic address: jhislop@medical.canon. FAU - Troupis, John M AU - Troupis JM AD - Medical Imaging Department, Monash Medical Centre, 246 Clayton Road, Clayton, Victoria 3168, Australia. FAU - Littlejohn, Geoffrey AU - Littlejohn G AD - Rheumatology Department, Monash Medical Centre, 246 Clayton Road, Clayton, Victoria 3168, Australia. FAU - Kuganesan, Ahilan AU - Kuganesan A AD - Medical Imaging Department, Monash Medical Centre, 246 Clayton Road, Clayton, Victoria 3168, Australia. LA - eng PT - Case Reports PT - Journal Article DEP - 20200518 PL - United States TA - Clin Imaging JT - Clinical imaging JID - 8911831 SB - IM MH - Female MH - Fingers/blood supply/physiopathology MH - Humans MH - Male MH - Raynaud Disease/*diagnostic imaging/etiology/physiopathology OTO - NOTNLM OT - CTA OT - CTP OT - MRA OT - Raynaud's phenomenon OT - Rheumatology EDAT- 2020/06/02 06:00 MHDA- 2020/09/25 06:00 CRDT- 2020/06/02 06:00 PHST- 2020/03/02 00:00 [received] PHST- 2020/04/22 00:00 [revised] PHST- 2020/05/13 00:00 [accepted] PHST- 2020/06/02 06:00 [pubmed] PHST- 2020/09/25 06:00 [medline] PHST- 2020/06/02 06:00 [entrez] AID - S0899-7071(20)30155-8 [pii] AID - 10.1016/j.clinimag.2020.05.010 [doi] PST - ppublish SO - Clin Imaging. 2020 Oct;66:133-136. doi: 10.1016/j.clinimag.2020.05.010. Epub 2020 May 18. PMID- 24034643 OWN - NLM STAT- MEDLINE DCOM- 20140505 LR - 20260128 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 140 IP - 8-9 DP - 2013 Aug-Sep TI - [Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation working group of the Société française de médecine vasculaire]. PG - 549-54 LID - S0151-9638(13)00535-8 [pii] LID - 10.1016/j.annder.2013.02.010 [doi] AB - About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up. CI - Copyright © 2013. Published by Elsevier Masson SAS. FAU - Pistorius, M-A AU - Pistorius MA AD - Service de médecine interne et vasculaire, Hôtel-Dieu, place Alexis-Ricordeau, BP 1005, 44093 Nantes cedex 01, France. Electronic address: marc.pistorius@chu-nantes.fr. FAU - Carpentier, P-H AU - Carpentier PH CN - le groupe de travail « Microcirculation » de la Société française de médecine vasculaire LA - fre PT - Consensus Statement PT - English Abstract PT - Journal Article PT - Practice Guideline TT - Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts. DEP - 20130411 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/blood MH - Connective Tissue Diseases/complications MH - Disease Progression MH - Fingers/blood supply MH - France/epidemiology MH - Humans MH - Laser-Doppler Flowmetry MH - Microcirculation MH - Microscopic Angioscopy MH - Occupational Diseases/diagnosis MH - Physical Examination/methods MH - Raynaud Disease/*diagnosis/epidemiology/etiology/pathology/physiopathology MH - Risk Factors MH - Scleroderma, Systemic/complications/diagnosis/immunology OTO - NOTNLM OT - Bilan OT - Capillaroscopie OT - Capillaroscopy OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Scleroderma OT - Sclérodermie OT - Screening EDAT- 2013/09/17 06:00 MHDA- 2014/05/06 06:00 CRDT- 2013/09/17 06:00 PHST- 2012/05/03 00:00 [received] PHST- 2012/05/09 00:00 [accepted] PHST- 2013/09/17 06:00 [entrez] PHST- 2013/09/17 06:00 [pubmed] PHST- 2014/05/06 06:00 [medline] AID - S0151-9638(13)00535-8 [pii] AID - 10.1016/j.annder.2013.02.010 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2013 Aug-Sep;140(8-9):549-54. doi: 10.1016/j.annder.2013.02.010. Epub 2013 Apr 11. PMID- 36710439 OWN - NLM STAT- MEDLINE DCOM- 20230509 LR - 20230509 IS - 1098-2752 (Electronic) IS - 0738-1085 (Linking) VI - 43 IP - 4 DP - 2023 May TI - Simultaneous bilateral distal venous arterialization between redial artery and cephalic vein in dorsal hand for Raynaud's phenomenon in both hands: A case report. PG - 397-402 LID - 10.1002/micr.31014 [doi] AB - Raynaud's phenomenon (RP) is characterized by episodic vasospasm in peripheral vessels and ischemia of the fingers. Venous arterialization is thought to induce neovascularization and increased oxygen tension. In this report, we describe a patient with RP in the fingers of both hands in whom venous arterialization achieved an acceptable result in both hands. The patient was a 62-year-old woman with a 10-year history of worsening pain and cold sensation in the tips of the index, middle, ring, and little fingers on both sides. The venous arterialization procedure was performed on both hands simultaneously at the level of the anatomical snuff box between radial artery and cephalic vein in dorsal hand. There was no need for valvectomy in the level of hands. To prevent development of the steal phenomenon in the arterialized veins, the superficial basilic and median veins of the forearm were ligated via 1 cm skin incisions. The pain and cold sensation in the fingertips of both hands remained decreased, and the nonhealing ulcers on the fingertips healed without the need for amputation. The observation period was 14 months, and the surface temperature of the fingers was increased after venous arterialization, as was the temperature of the palm and forearm. There was no problem when administering intravenous infusion into the forearm on either side postoperatively. The case showed venous arterialization was effective for RP without increasing intravenous pressure in the affected limb, and further investigation is necessary. CI - © 2023 Wiley Periodicals LLC. FAU - Yoshida, Shuhei AU - Yoshida S AD - The International Center for Lymphedema, Plastic and reconstructive Surgery, Hiroshima University Hospital, Hiroshima, Japan. FAU - Imai, Hirofumi AU - Imai H AD - The International Center for Lymphedema, Plastic and reconstructive Surgery, Hiroshima University Hospital, Hiroshima, Japan. FAU - Roh, Solji AU - Roh S AUID- ORCID: 0000-0001-6751-2439 AD - The International Center for Lymphedema, Plastic and reconstructive Surgery, Hiroshima University Hospital, Hiroshima, Japan. FAU - Mese, Toshiro AU - Mese T AD - The International Center for Lymphedema, Plastic and reconstructive Surgery, Hiroshima University Hospital, Hiroshima, Japan. FAU - Koshima, Isao AU - Koshima I AUID- ORCID: 0000-0003-4588-8157 AD - The International Center for Lymphedema, Plastic and reconstructive Surgery, Hiroshima University Hospital, Hiroshima, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20230129 PL - United States TA - Microsurgery JT - Microsurgery JID - 8309230 SB - IM MH - Female MH - Humans MH - Middle Aged MH - *Hand/surgery MH - Veins/surgery MH - Fingers/blood supply MH - Pain/etiology MH - Radial Artery/surgery MH - *Raynaud Disease/etiology/surgery EDAT- 2023/01/31 06:00 MHDA- 2023/05/09 06:42 CRDT- 2023/01/30 00:52 PHST- 2022/12/10 00:00 [revised] PHST- 2022/08/25 00:00 [received] PHST- 2023/01/13 00:00 [accepted] PHST- 2023/05/09 06:42 [medline] PHST- 2023/01/31 06:00 [pubmed] PHST- 2023/01/30 00:52 [entrez] AID - 10.1002/micr.31014 [doi] PST - ppublish SO - Microsurgery. 2023 May;43(4):397-402. doi: 10.1002/micr.31014. Epub 2023 Jan 29. PMID- 26976953 OWN - NLM STAT- MEDLINE DCOM- 20170612 LR - 20190320 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 55 IP - 6 DP - 2016 Jun TI - Digital ischaemia during cooling is independently related to nailfold capillaroscopic pattern in patients with Raynaud's phenomenon. PG - 1083-90 LID - 10.1093/rheumatology/kew028 [doi] AB - OBJECTIVE: The aim of the study was to assess the association between plethysmographically measured vasospasms during stepwise cooling and recovery, as an index for digital ischaemia, and nailfold capillaroscopic pattern (NCP) severity in patients with primary or secondary RP, including SSc. METHODS: In 381 consecutive patients with suspected RP without a history of digital ulcers, NCP (assessed by widefield videocapillaroscopy), fingertip photoelectric plethysmography during cooling and recovery and clinical characteristics were analysed. NCPs were graded as follows: normal, non-specific, early and active. The mean ischaemic time was defined as the mean time of perfusion loss during cooling and recovery of five fingers. RESULTS: In the patients with loss of perfusion during cooling and recovery, the NCP was normal in 152, non-specific in 96, early in 61 and active in 39 patients. The mean ischaemic time was positively associated with the severity of NCP, with P < 0.05 for each two- or three-grade increase and independent of underlying SSc. The difference was most pronounced during recovery. CONCLUSION: We demonstrate that the degree of vasospasm and ischaemia provoked by stepwise cooling and recovery are positively associated with NCP in patients with RP of different aetiologies and without a history of digital ulcers. CI - © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - van Roon, Anniek M AU - van Roon AM AD - Department of Internal Medicine, Division of Vascular Medicine a.m.van.roon01@umcg.nl. FAU - Smit, Andries J AU - Smit AJ AD - Department of Internal Medicine, Division of Vascular Medicine. FAU - van Roon, Arie M AU - van Roon AM AD - Department of Internal Medicine, Division of Vascular Medicine. FAU - Bootsma, Hendrika AU - Bootsma H AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. FAU - Mulder, Douwe J AU - Mulder DJ AD - Department of Internal Medicine, Division of Vascular Medicine Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. LA - eng PT - Journal Article DEP - 20160314 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Cold Temperature/adverse effects MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*diagnostic imaging/etiology MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Plethysmography/methods MH - Raynaud Disease/etiology/*physiopathology MH - Scleroderma, Systemic/*complications/physiopathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ischaemia OT - nailfold capillaroscopy OT - systemic sclerosis EDAT- 2016/03/16 06:00 MHDA- 2019/03/21 06:00 CRDT- 2016/03/16 06:00 PHST- 2015/02/06 00:00 [received] PHST- 2016/03/16 06:00 [entrez] PHST- 2016/03/16 06:00 [pubmed] PHST- 2019/03/21 06:00 [medline] AID - kew028 [pii] AID - 10.1093/rheumatology/kew028 [doi] PST - ppublish SO - Rheumatology (Oxford). 2016 Jun;55(6):1083-90. doi: 10.1093/rheumatology/kew028. Epub 2016 Mar 14. PMID- 32107950 OWN - NLM STAT- MEDLINE DCOM- 20210126 LR - 20210126 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 49 IP - 5 DP - 2020 Sep TI - Immunoglobulin G kappa multiple myeloma with the only initial manifestation of Raynaud's phenomenon. PG - 417-418 LID - 10.1080/03009742.2020.1711965 [doi] FAU - Chu, C-C AU - Chu CC AD - Division of Rheumatology/Immunology and Allergy, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. FAU - Chiu, Y-H AU - Chiu YH AD - Division of Rheumatology/Immunology and Allergy, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. FAU - Lin, M-H AU - Lin MH AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. FAU - Yeh, J-H AU - Yeh JH AD - Division of Hematology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. FAU - Chen, H-C AU - Chen HC AD - Division of Rheumatology/Immunology and Allergy, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. FAU - Kuo, S-Y AU - Kuo SY AD - Division of Rheumatology/Immunology and Allergy, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei, Taiwan. LA - eng PT - Case Reports PT - Letter DEP - 20200228 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Immunoglobulin G) SB - IM MH - Aged MH - Female MH - Humans MH - Immunoglobulin G/*immunology MH - Multiple Myeloma/*complications/immunology MH - Raynaud Disease/*etiology/immunology EDAT- 2020/02/29 06:00 MHDA- 2021/01/27 06:00 CRDT- 2020/02/29 06:00 PHST- 2020/02/29 06:00 [pubmed] PHST- 2021/01/27 06:00 [medline] PHST- 2020/02/29 06:00 [entrez] AID - 10.1080/03009742.2020.1711965 [doi] PST - ppublish SO - Scand J Rheumatol. 2020 Sep;49(5):417-418. doi: 10.1080/03009742.2020.1711965. Epub 2020 Feb 28. PMID- 34282441 OWN - NLM STAT- MEDLINE DCOM- 20220413 LR - 20220525 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 4 DP - 2022 Apr 11 TI - Clinical significance of anti-NOR90 antibodies in systemic sclerosis and idiopathic interstitial pneumonia. PG - 1709-1716 LID - 10.1093/rheumatology/keab575 [doi] AB - OBJECTIVE: Anti-NOR90 antibodies are usually found in patients with SSc; however, their clinical relevance remains obscure. We developed an ELISA for measuring them to investigate the clinical features of patients with anti-NOR90 antibodies. METHODS: Serum samples from 1252 patients with various conditions from Nagoya University Hospital and 244 patients with idiopathic interstitial pneumonia (IIP) from Tosei General Hospital were included. Anti-NOR90 antibodies were assayed by an ELISA using the recombinant protein produced by in vitro transcription/translation. RESULTS: Five (0.4%) patients in the Nagoya University Hospital cohort had anti-NOR90 antibodies. One patient with diffuse cutaneous SSc, three with limited cutaneous SSc, and one with Raynaud's disease were positive for anti-NOR90 antibodies. Anti-NOR90 antibodies were found more frequently in patients with systemic scleroderma-spectrum disorders (SSDs) than without SSDs (5/316 vs 0/936, P <0.00101) and were found more frequently in patients with SSc than without SSc (4/249 vs 0/528, P <0.0104) in the systemic autoimmune rheumatic diseases cohort. Three of the four anti-NOR90-positive SSc patients had interstitial lung disease (ILD), and two of those four had cancer. Three (1.2%) patients in the Tosei General Hospital cohort had anti-NOR90 antibodies. All three of the anti-NOR90-positive IIP patients had gastrointestinal tract involvement, and two of those three had cancer or skin lesions observed in SSc. CONCLUSIONS: Although anti-NOR90 antibodies are rarely found in clinics, our ELISA is useful for their detection. Further studies are needed to confirm the association of anti-NOR90 antibodies with ILD and cancer in SSc and IIP patients. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Yamashita, Yuta AU - Yamashita Y AD - Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya. FAU - Yamano, Yasuhiko AU - Yamano Y AD - Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan. FAU - Muro, Yoshinao AU - Muro Y AD - Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya. FAU - Ogawa-Momohara, Mariko AU - Ogawa-Momohara M AD - Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya. FAU - Takeichi, Takuya AU - Takeichi T AD - Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya. FAU - Kondoh, Yasuhiro AU - Kondoh Y AD - Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan. FAU - Akiyama, Masashi AU - Akiyama M AD - Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Cohort Studies MH - Humans MH - *Idiopathic Interstitial Pneumonias/complications MH - *Lung Diseases, Interstitial/complications MH - *Raynaud Disease/complications MH - *Scleroderma, Systemic OTO - NOTNLM OT - anti-NOR90 antibody OT - anti-nucleolar antibody OT - cancer OT - idiopathic interstitial pneumonia OT - interstitial lung disease OT - systemic autoimmune rheumatic disease OT - systemic sclerosis EDAT- 2021/07/21 06:00 MHDA- 2022/04/14 06:00 CRDT- 2021/07/20 06:58 PHST- 2021/05/06 00:00 [received] PHST- 2021/07/09 00:00 [revised] PHST- 2021/07/21 06:00 [pubmed] PHST- 2022/04/14 06:00 [medline] PHST- 2021/07/20 06:58 [entrez] AID - 6324309 [pii] AID - 10.1093/rheumatology/keab575 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Apr 11;61(4):1709-1716. doi: 10.1093/rheumatology/keab575. PMID- 25612948 OWN - NLM STAT- MEDLINE DCOM- 20170102 LR - 20170104 IS - 1603-6824 (Electronic) IS - 0041-5782 (Linking) VI - 177 IP - 2A DP - 2015 Jan 26 TI - [Raynaud's phenomenon of the papilla mammae caused by breastfeeding]. PG - 18-9 LID - V04130236 [pii] AB - In Denmark, the benefits of breastfeeding are emphasized and the Danish Health and Medicines Authority encourages women to breastfeed for at least six months. Raynaud's phenomenon can occur in any small arteriole and cause painful, temporary ischaemia. Women quit breastfeeding prematurely for a variety of reasons, pain being just one of them. In this case report we describe an incident of Raynaud's phenomenon of the nipple and describe how correct diagnosis can prevent unnecessary medical treatment and cause relief of symptoms, thus avoiding premature cessation of breastfeeding. FAU - Laursen, Jacob Brink AU - Laursen JB AD - Gynækologisk/Obstetrisk Afdeling, Hvidovre Hospital, Kettegård Allé 30, 2650 Hvidovre. jacobbrink@gmail.com. FAU - Rørbye, Christina AU - Rørbye C LA - dan PT - Case Reports PT - Journal Article TT - Ammeudløst Raynauds fænomen i papilla mammae. PL - Denmark TA - Ugeskr Laeger JT - Ugeskrift for laeger JID - 0141730 RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) SB - IM MH - Adult MH - Breast Feeding/*adverse effects MH - Calcium Channel Blockers/therapeutic use MH - Female MH - Humans MH - Nipples/*pathology MH - Pain/etiology MH - Pregnancy MH - Raynaud Disease/drug therapy/*etiology/pathology MH - Vasodilator Agents/therapeutic use EDAT- 2015/01/24 06:00 MHDA- 2017/01/04 06:00 CRDT- 2015/01/24 06:00 PHST- 2015/01/24 06:00 [entrez] PHST- 2015/01/24 06:00 [pubmed] PHST- 2017/01/04 06:00 [medline] AID - V04130236 [pii] PST - ppublish SO - Ugeskr Laeger. 2015 Jan 26;177(2A):18-9. PMID- 34953734 OWN - NLM STAT- MEDLINE DCOM- 20220503 LR - 20220503 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 18 IP - 4 DP - 2022 Apr TI - Treatment of Raynaud phenomenon and ischemic ulcers associated to systemic sclerosis with hyperbaric oxygen. PG - 246-248 LID - S2173-5743(21)00216-1 [pii] LID - 10.1016/j.reumae.2021.05.004 [doi] AB - We describe 4 patients with Raynaud's phenomenon associated with systemic sclerosis, 3 with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment. CI - Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Ahijón-Lana, María AU - Ahijón-Lana M AD - Servicio de Reumatología, Hospital Universitario Central de la Defensa Gómez Ulla, Madrid, Spain. Electronic address: mahilan@mde.es. FAU - Baragaño-Ordóñez, Elia AU - Baragaño-Ordóñez E AD - Servicio de Medicina Subacuática e Hiperbárica, Hospital Universitario Central de la Defensa Gómez Ulla, Madrid, Spain. FAU - Veiga-Cabello, Raúl AU - Veiga-Cabello R AD - Servicio de Reumatología, Hospital Universitario Central de la Defensa Gómez Ulla, Madrid, Spain. FAU - de la Cruz-Tapidor, Carmen AU - de la Cruz-Tapidor C AD - Servicio de Reumatología, Hospital Universitario Central de la Defensa Gómez Ulla, Madrid, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20211223 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - *Hyperbaric Oxygenation MH - *Raynaud Disease/complications/therapy MH - *Scleroderma, Systemic/complications/therapy MH - Ulcer/complications/drug therapy OTO - NOTNLM OT - Digital ulcers OT - Esclerosis sistémica OT - Fenómeno de Raynaud OT - Hyperbaric oxygen OT - Oxígeno hiperbárico OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Úlceras digitales EDAT- 2021/12/27 06:00 MHDA- 2022/05/04 06:00 CRDT- 2021/12/26 20:29 PHST- 2021/03/04 00:00 [received] PHST- 2021/05/19 00:00 [accepted] PHST- 2021/12/27 06:00 [pubmed] PHST- 2022/05/04 06:00 [medline] PHST- 2021/12/26 20:29 [entrez] AID - S2173-5743(21)00216-1 [pii] AID - 10.1016/j.reumae.2021.05.004 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2022 Apr;18(4):246-248. doi: 10.1016/j.reumae.2021.05.004. Epub 2021 Dec 23. PMID- 26692357 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 12 IP - 3 DP - 2016 TI - The cardiac magnetic resonance in the diagnosis of cardiac Raynaud phenomenon in a patient with systemic sclerosis: case report and review of literature. PG - 251-5 LID - 10.1586/1744666X.2016.1134320 [doi] AB - Raynaud phenomenon (RP) is the hallmark of Systemic Sclerosis (SSc). Visceral RP has also been proposed in SSc patients. Cardiac Raynaud's phenomenon (C-RP) was evaluated in a few clinical studies both as cold-induced transient myocardial ischaemia and as presence of advanced myocardial fibrosis and contraction band necrosis in autopsied patients. Until today numerous techniques, such as scintigraphy and myocardial contrast echocardiography, have been used to evaluate C-RP. In this case report for the first time we have used Cardiac Magnetic Resonance (CMR) after cold test to demonstrate the presence of the C-RP. In addition we have shown that therapy with Iloprost can be used to reduce episodes of C-RP. FAU - Quarta, Silvia AU - Quarta S AD - a Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center , Sapienza University of Rome , Rome , Italy. FAU - Galea, Nicola AU - Galea N AD - b Department of Radiological Sciences, Pathology and Oncology , Sapienza University of Rome , Rome , Italy. FAU - Gigante, Antonietta AU - Gigante A AD - a Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center , Sapienza University of Rome , Rome , Italy. FAU - Romaniello, Antonella AU - Romaniello A AD - c Department of Clinical and Molecular Medicine, Cardiology Unit , Sapienza University of Rome , Rome , Italy. FAU - Rosato, Edoardo AU - Rosato E AD - a Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center , Sapienza University of Rome , Rome , Italy. FAU - Carbone, Iacopo AU - Carbone I AD - b Department of Radiological Sciences, Pathology and Oncology , Sapienza University of Rome , Rome , Italy. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20160106 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - 0 (Antibodies, Antinuclear) RN - 0 (Platelet Aggregation Inhibitors) RN - JED5K35YGL (Iloprost) SB - IM MH - Antibodies, Antinuclear/blood MH - Female MH - Fibrosis MH - Heart/*diagnostic imaging/drug effects MH - Humans MH - Iloprost/therapeutic use MH - Ischemic Attack, Transient/complications/*diagnostic imaging/drug therapy MH - *Magnetic Resonance Imaging MH - Middle Aged MH - Myocardium/*pathology MH - Platelet Aggregation Inhibitors/therapeutic use MH - Raynaud Disease/complications/*diagnostic imaging/drug therapy MH - Recovery of Function/drug effects MH - Scleroderma, Systemic/complications/*diagnostic imaging/drug therapy OTO - NOTNLM OT - Cardiac Magnetic Resonance OT - Cardiac Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2015/12/23 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/12/23 06:00 PHST- 2015/12/23 06:00 [entrez] PHST- 2015/12/23 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1586/1744666X.2016.1134320 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2016;12(3):251-5. doi: 10.1586/1744666X.2016.1134320. Epub 2016 Jan 6. PMID- 25982864 OWN - NLM STAT- MEDLINE DCOM- 20160218 LR - 20260518 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 142 Suppl 2 DP - 2015 Jun TI - [Item 237 - UE 8 Acrosyndromes (Raynaud's phenomenon, erythermalgia, acrocyanosis, frostbite, digital ischemia)]. PG - S195-200 LID - S0151-9638(15)00161-1 [pii] LID - 10.1016/j.annder.2015.04.001 [doi] CN - CEDEF LA - fre PT - Journal Article TT - Item 237--UE 8 Acrosyndromes (phénomène de Raynaud, érythermalgie, acrocyanose, engelures, ischémie digitale). DEP - 20150513 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - *Erythromelalgia/diagnosis/therapy MH - Fingers/*blood supply MH - *Frostbite/diagnosis/therapy MH - Humans MH - *Ischemia/diagnosis/therapy MH - *Raynaud Disease/diagnosis/therapy FIR - Aractingi, S IR - Aractingi S FIR - Aubin, F IR - Aubin F FIR - Avril, M-F IR - Avril MF FIR - Bachelez, H IR - Bachelez H FIR - Bagot, M IR - Bagot M FIR - Barbaud, A IR - Barbaud A FIR - Basset-Seguin, N IR - Basset-Seguin N FIR - Béani, J-Cl IR - Béani JC FIR - Bédane, C IR - Bédane C FIR - Bérard, F IR - Bérard F FIR - Berbis, P IR - Berbis P FIR - Bernard, P IR - Bernard P FIR - Beylot-Barry, M IR - Beylot-Barry M FIR - Bodemer, C IR - Bodemer C FIR - Boraveli, F IR - Boraveli F FIR - Bourgault-Villada, I IR - Bourgault-Villada I FIR - Bursztejn, A-C IR - Bursztejn AC FIR - Cambazard, F IR - Cambazard F FIR - Carsuzaa, F IR - Carsuzaa F FIR - Caux, F IR - Caux F FIR - Chosidow, O IR - Chosidow O FIR - Couppie, P IR - Couppie P FIR - Cribier, B IR - Cribier B FIR - Crickx, B IR - Crickx B FIR - Dalle, S IR - Dalle S FIR - Delaporte, E IR - Delaporte E FIR - Dereure, O IR - Dereure O FIR - Descamps, V IR - Descamps V FIR - D'Incan, M IR - D'Incan M FIR - Doutre, M-S IR - Doutre MS FIR - Dreno, B IR - Dreno B FIR - Dupin, N IR - Dupin N FIR - Dupuy, A IR - Dupuy A FIR - Faure, M IR - Faure M FIR - Frances, C IR - Frances C FIR - Gaudy-Marqueste, C IR - Gaudy-Marqueste C FIR - Grange, F IR - Grange F FIR - Grob, J-J IR - Grob JJ FIR - Guégan, S IR - Guégan S FIR - Guillet, G IR - Guillet G FIR - Guillot, B IR - Guillot B FIR - Hadj Rabia, S IR - Hadj Rabia S FIR - Humbert, P IR - Humbert P FIR - Joly, P IR - Joly P FIR - Jullien, D IR - Jullien D FIR - Lacour, J-P IR - Lacour JP FIR - Laroche, L IR - Laroche L FIR - Lebbé, C IR - Lebbé C FIR - Leccia, M-T IR - Leccia MT FIR - Lipsker, D IR - Lipsker D FIR - Lok, C IR - Lok C FIR - Lorette, G IR - Lorette G FIR - Machet, L IR - Machet L FIR - Martin, L IR - Martin L FIR - Maruani, A IR - Maruani A FIR - Mazereeuw-Hautier, J IR - Mazereeuw-Hautier J FIR - Meunier, L IR - Meunier L FIR - Misery, L IR - Misery L FIR - Modiano, P IR - Modiano P FIR - Morand, J-J IR - Morand JJ FIR - Mortier, L IR - Mortier L FIR - Musette, P IR - Musette P FIR - Nicolas, J-F IR - Nicolas JF FIR - Passeron, T IR - Passeron T FIR - Paul, C IR - Paul C FIR - Pelletier, F IR - Pelletier F FIR - Phan, A IR - Phan A FIR - Prost, C IR - Prost C FIR - Quereux, G IR - Quereux G FIR - Richard, M-A IR - Richard MA FIR - Saiag, P IR - Saiag P FIR - Samimi, M IR - Samimi M FIR - Sbidian, E IR - Sbidian E FIR - Schmutz, J-L IR - Schmutz JL FIR - Soria, A IR - Soria A FIR - Stalder, J-F IR - Stalder JF FIR - Staumont, D IR - Staumont D FIR - Stoebner, P-E IR - Stoebner PE FIR - Taïeb, A IR - Taïeb A FIR - Thomas, L IR - Thomas L FIR - Vabres, P IR - Vabres P FIR - Vaillant, L IR - Vaillant L FIR - Verneuil, L IR - Verneuil L FIR - Viguier, M IR - Viguier M FIR - Wolkenstein, P IR - Wolkenstein P EDAT- 2015/05/20 06:00 MHDA- 2016/02/19 06:00 CRDT- 2015/05/19 06:00 PHST- 2015/05/19 06:00 [entrez] PHST- 2015/05/20 06:00 [pubmed] PHST- 2016/02/19 06:00 [medline] AID - S0151-9638(15)00161-1 [pii] AID - 10.1016/j.annder.2015.04.001 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2015 Jun;142 Suppl 2:S195-200. doi: 10.1016/j.annder.2015.04.001. Epub 2015 May 13. PMID- 20516043 OWN - NLM STAT- MEDLINE DCOM- 20100708 LR - 20100602 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 6 DP - 2010 Jun TI - Raynaud's phenomenon and hand function in patients with rheumatoid arthritis. PG - 1358-9 LID - 10.3899/jrheum.091006 [doi] FAU - Tansey, Rosamond J AU - Tansey RJ FAU - Wragg, Elizabeth A AU - Wragg EA FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Case Reports PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Arthritis, Rheumatoid/*complications/physiopathology MH - Female MH - Hand/*blood supply/physiopathology MH - Hand Strength/physiology MH - Humans MH - Hypokinesia/etiology/physiopathology MH - Middle Aged MH - Muscle Rigidity/etiology/physiopathology MH - Pilot Projects MH - Raynaud Disease/*complications/physiopathology MH - Tremor/etiology/physiopathology MH - Young Adult EDAT- 2010/06/03 06:00 MHDA- 2010/07/09 06:00 CRDT- 2010/06/03 06:00 PHST- 2010/06/03 06:00 [entrez] PHST- 2010/06/03 06:00 [pubmed] PHST- 2010/07/09 06:00 [medline] AID - 37/6/1358-a [pii] AID - 10.3899/jrheum.091006 [doi] PST - ppublish SO - J Rheumatol. 2010 Jun;37(6):1358-9. doi: 10.3899/jrheum.091006. PMID- 20656258 OWN - NLM STAT- MEDLINE DCOM- 20101105 LR - 20161020 IS - 1532-8473 (Electronic) IS - 1089-9472 (Linking) VI - 25 IP - 4 DP - 2010 Aug TI - Perianesthesia management of Raynaud's phenomenon--a case report. PG - 221-5 LID - 10.1016/j.jopan.2010.05.012 [doi] AB - This article presents a case report of a 52-year-old female patient with lung cancer presenting with Raynaud's phenomenon during thoracic surgery. Experiences and lessons learned from this case are presented. The classification, pathogenesis, and perianesthesia management of Raynaud's phenomenon are discussed. CI - (c) 2010 American Society of PeriAnesthesia Nurses. Published by Elsevier Inc. All rights reserved. FAU - Liang, Yong X AU - Liang YX AD - Department of Anesthesiology, The Affiliated Hospital of Qingdao University Medical College, Qingdao 266000, People's Republic of China. liangzi66@hotmail.com FAU - Gu, Miao N AU - Gu MN FAU - Wang, Shi D AU - Wang SD FAU - Chu, Hai C AU - Chu HC LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Perianesth Nurs JT - Journal of perianesthesia nursing : official journal of the American Society of PeriAnesthesia Nurses JID - 9610507 MH - Anesthesia/*adverse effects MH - Female MH - Humans MH - Lung Neoplasms/nursing/surgery MH - Middle Aged MH - Perioperative Nursing/*methods MH - Postoperative Complications/*nursing/*therapy MH - Raynaud Disease/etiology/*nursing/*therapy EDAT- 2010/07/27 06:00 MHDA- 2010/11/06 06:00 CRDT- 2010/07/27 06:00 PHST- 2009/11/15 00:00 [received] PHST- 2010/03/25 00:00 [revised] PHST- 2010/05/18 00:00 [accepted] PHST- 2010/07/27 06:00 [entrez] PHST- 2010/07/27 06:00 [pubmed] PHST- 2010/11/06 06:00 [medline] AID - S1089-9472(10)00243-1 [pii] AID - 10.1016/j.jopan.2010.05.012 [doi] PST - ppublish SO - J Perianesth Nurs. 2010 Aug;25(4):221-5. doi: 10.1016/j.jopan.2010.05.012. PMID- 22718854 OWN - NLM STAT- MEDLINE DCOM- 20140207 LR - 20220331 IS - 1460-2393 (Electronic) IS - 1460-2393 (Linking) VI - 106 IP - 6 DP - 2013 Jun TI - Raynaud's phenomenon of the tongue: uncommon presentation of a classical sign. PG - 583-4 LID - 10.1093/qjmed/hcs103 [doi] FAU - Lioger, B AU - Lioger B AD - Department of Internal Medicine, University hospital of Tours, 2 boulevard Tonnellé, Tours, Centre 37000, France. lioger.bertrand@wanadoo.fr FAU - Diot, E AU - Diot E LA - eng PT - Case Reports PT - Journal Article DEP - 20120619 PL - England TA - QJM JT - QJM : monthly journal of the Association of Physicians JID - 9438285 SB - IM MH - Adult MH - Female MH - Fingers MH - Humans MH - Rare Diseases/diagnosis/etiology MH - Raynaud Disease/*diagnosis/etiology MH - Scleroderma, Systemic/complications MH - Skin Ulcer/etiology MH - Tongue Diseases/*diagnosis/etiology EDAT- 2012/06/22 06:00 MHDA- 2014/02/08 06:00 CRDT- 2012/06/22 06:00 PHST- 2012/06/22 06:00 [entrez] PHST- 2012/06/22 06:00 [pubmed] PHST- 2014/02/08 06:00 [medline] AID - hcs103 [pii] AID - 10.1093/qjmed/hcs103 [doi] PST - ppublish SO - QJM. 2013 Jun;106(6):583-4. doi: 10.1093/qjmed/hcs103. Epub 2012 Jun 19. PMID- 21992245 OWN - NLM STAT- MEDLINE DCOM- 20120213 LR - 20220210 IS - 1525-1403 (Electronic) IS - 1094-7159 (Linking) VI - 14 IP - 3 DP - 2011 May-Jun TI - Spinal cord stimulation for Raynaud's syndrome: long-term alleviation of bilateral pain with a single cervical lead. PG - 229-33; discussion 233-4 LID - 10.1111/j.1525-1403.2011.00332.x [doi] AB - INTRODUCTION:   Spinal cord stimulation (SCS) has been described in a variety of neuropathic and vasospastic pain conditions including Raynaud's syndrome. METHODS:   We report here the outcome of single lead SCS in the case of a 49-year-old woman with severe Raynaud's syndrome, which had failed to respond to medical therapy. RESULTS:   With a single quadripolar cervical lead in midline position at the C2/C3 level sustained pain relief of the bilateral pain was accomplished. Pain scores sank from 7/10 to 2-3/10 on the nominal analog scale and remained stable more than nearly four years by now. CONCLUSIONS:   Treatment of bilateral pain in Raynaud's syndrome with SCS in a single technique is feasible. Advantages and disadvantages as compared with stimulation with bilateral leads are discussed. CI - © 2011 International Neuromodulation Society. FAU - Wolter, Tilman AU - Wolter T AD - Interdisciplinary Pain Centre, University Hospital Freiburg, Breisacherstrasse 64, Freiburg, Germany. tilman.wolter@uniklinik-freiburg.de FAU - Kieselbach, Kristin AU - Kieselbach K LA - eng PT - Case Reports PT - Journal Article DEP - 20110301 PL - United States TA - Neuromodulation JT - Neuromodulation : journal of the International Neuromodulation Society JID - 9804159 SB - IM MH - *Cervical Vertebrae MH - *Chronic Pain/etiology/therapy MH - *Electric Stimulation Therapy/instrumentation/methods MH - *Electrodes, Implanted MH - Female MH - Humans MH - Middle Aged MH - Pain Measurement MH - *Raynaud Disease/complications/therapy MH - Spinal Cord/*physiology MH - Thermography MH - Treatment Outcome EDAT- 2011/10/14 06:00 MHDA- 2012/02/14 06:00 CRDT- 2011/10/14 06:00 PHST- 2011/10/14 06:00 [entrez] PHST- 2011/10/14 06:00 [pubmed] PHST- 2012/02/14 06:00 [medline] AID - S1094-7159(11)60048-2 [pii] AID - 10.1111/j.1525-1403.2011.00332.x [doi] PST - ppublish SO - Neuromodulation. 2011 May-Jun;14(3):229-33; discussion 233-4. doi: 10.1111/j.1525-1403.2011.00332.x. Epub 2011 Mar 1. PMID- 32212180 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1525-1470 (Electronic) IS - 0736-8046 (Linking) VI - 37 IP - 4 DP - 2020 Jul TI - Ischemic ulcers of the toes secondary to Raynaud's phenomenon in a child successfully treated with botulinum toxin. PG - 681-683 LID - 10.1111/pde.14160 [doi] AB - Raynaud's phenomenon (RP) is an episodic vasospastic response to cold or emotional stress causing color changes and pain. These attacks can lead to digital ischemia, ulcers, and gangrene. Severe and refractory RP in children is a therapeutic challenge for clinicians because there are no standardized treatment protocols for these patients. We present a case of RP involving the toes of a child successfully treated with botulinum toxin A. CI - © 2020 Wiley Periodicals, Inc. FAU - Quintana Castanedo, Lucía AU - Quintana Castanedo L AUID- ORCID: 0000-0001-5136-841X AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - Feito Rodríguez, Marta AU - Feito Rodríguez M AUID- ORCID: 0000-0002-2645-8904 AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - Maseda Pedrero, Rocío AU - Maseda Pedrero R AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - Chiloeches Fernández, Clara AU - Chiloeches Fernández C AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - de Lucas Laguna, Raúl AU - de Lucas Laguna R AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20200324 PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A MH - Child MH - Humans MH - Pain MH - *Raynaud Disease/drug therapy/etiology MH - Toes MH - Ulcer OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin OT - digital ulcers OT - toes EDAT- 2020/03/27 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/03/27 06:00 PHST- 2020/03/27 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/03/27 06:00 [entrez] AID - 10.1111/pde.14160 [doi] PST - ppublish SO - Pediatr Dermatol. 2020 Jul;37(4):681-683. doi: 10.1111/pde.14160. Epub 2020 Mar 24. PMID- 18080433 OWN - NLM STAT- MEDLINE DCOM- 20080116 LR - 20071217 IS - 0035-2640 (Print) IS - 0035-2640 (Linking) VI - 57 IP - 15 DP - 2007 Oct 15 TI - [Diagnosis of Raynaud's phenomenon]. PG - 1707-12; quiz 1712 FAU - Marie, Isabelle AU - Marie I AD - Département de médecine interne, CHU de Rouen, 76031 Rouen. isabelle.marie@chu-rouen.fr LA - fre PT - Case Reports PT - Journal Article TT - Phénomène de Raynaud. Orientation diagnostique. PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 RN - 0 (Adrenergic beta-Antagonists) SB - IM MH - Adrenergic beta-Antagonists/adverse effects MH - Adult MH - Arteritis/complications MH - Carpal Tunnel Syndrome/complications MH - Connective Tissue Diseases/complications MH - Cumulative Trauma Disorders/complications MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply MH - Humans MH - Occupational Diseases/complications MH - Raynaud Disease/*complications/*diagnosis/etiology MH - Substance-Related Disorders/complications MH - Vascular Diseases/complications MH - Vibration/adverse effects EDAT- 2007/12/18 09:00 MHDA- 2008/01/17 09:00 CRDT- 2007/12/18 09:00 PHST- 2007/12/18 09:00 [pubmed] PHST- 2008/01/17 09:00 [medline] PHST- 2007/12/18 09:00 [entrez] PST - ppublish SO - Rev Prat. 2007 Oct 15;57(15):1707-12; quiz 1712. PMID- 28101516 OWN - NLM STAT- MEDLINE DCOM- 20170130 LR - 20181113 IS - 2314-6141 (Electronic) IS - 2314-6133 (Print) VI - 2016 DP - 2016 TI - Postocclusive Hyperemia Measured with Laser Doppler Flowmetry and Transcutaneous Oxygen Tension in the Diagnosis of Primary Raynaud's Phenomenon: A Prospective, Controlled Study. PG - 9645705 LID - 10.1155/2016/9645705 [doi] LID - 9645705 AB - The aim of this study was to measure the sensitivity and specificity of transcutaneous oxygen tension and postocclusive hyperemia testing using laser Doppler flowmetry in patients with primary Raynaud's phenomenon. One hundred patients and one hundred controls were included in the study. Baseline microvascular blood flow and then time to peak flow following occlusion were measured using laser Doppler flowmetry. Afterwards, the transcutaneous oxygen tension was recorded. The sensitivities of baseline microvascular blood flow, postocclusive time to peak flow, and transcutaneous oxygen tension were 79%, 79%, and 77%, respectively. The postocclusive time peak flow had a superior specificity of 90% and area under the curve of 0.92 as compared to 66% and 0.80 for baseline microvascular flow and 64% and 0.76 for transcutaneous oxygen tension. Time to postocclusive peak blood flow measured by laser Doppler flowmetry is a highly accurate test for differentiating patients with primary Raynaud's phenomenon from healthy controls. FAU - Maga, Paweł AU - Maga P AD - Department of Angiology, Jagiellonian University Medical College, 8 Skawińska Street, 31-066 Krakow, Poland. FAU - Henry, Brandon Michael AU - Henry BM AD - Department of Anatomy, Jagiellonian University Medical College, 12 Kopernika Street, 31-034 Krakow, Poland. FAU - Kmiotek, Elizabeth K AU - Kmiotek EK AD - Department of Anatomy, Jagiellonian University Medical College, 12 Kopernika Street, 31-034 Krakow, Poland. FAU - Gregorczyk-Maga, Iwona AU - Gregorczyk-Maga I AD - Department of Pediatric Dentistry, Institute of Dentistry, Jagiellonian University Medical College, 4 Montelupich Street, 31-155 Krakow, Poland. FAU - Kaczmarczyk, Paweł AU - Kaczmarczyk P AD - Department of Angiology, Jagiellonian University Medical College, 8 Skawińska Street, 31-066 Krakow, Poland. FAU - Tomaszewski, Krzysztof A AU - Tomaszewski KA AUID- ORCID: 0000-0001-5077-6948 AD - Department of Anatomy, Jagiellonian University Medical College, 12 Kopernika Street, 31-034 Krakow, Poland. FAU - Niżankowski, Rafał AU - Niżankowski R AD - Department of Angiology, Jagiellonian University Medical College, 8 Skawińska Street, 31-066 Krakow, Poland. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20161222 PL - United States TA - Biomed Res Int JT - BioMed research international JID - 101600173 SB - IM MH - Adolescent MH - Adult MH - Blood Gas Monitoring, Transcutaneous/methods MH - Female MH - Humans MH - *Hyperemia/blood/physiopathology MH - Laser-Doppler Flowmetry/methods MH - Male MH - Middle Aged MH - Prospective Studies MH - *Raynaud Disease/blood/physiopathology PMC - PMC5215461 COIS- The authors declare that there is no conflict of interests. EDAT- 2017/01/20 06:00 MHDA- 2017/01/31 06:00 PMCR- 2016/12/22 CRDT- 2017/01/20 06:00 PHST- 2016/07/19 00:00 [received] PHST- 2016/11/21 00:00 [accepted] PHST- 2017/01/20 06:00 [entrez] PHST- 2017/01/20 06:00 [pubmed] PHST- 2017/01/31 06:00 [medline] PHST- 2016/12/22 00:00 [pmc-release] AID - 10.1155/2016/9645705 [doi] PST - ppublish SO - Biomed Res Int. 2016;2016:9645705. doi: 10.1155/2016/9645705. Epub 2016 Dec 22. PMID- 27024550 OWN - NLM STAT- MEDLINE DCOM- 20160423 LR - 20181203 IS - 1042-1882 (Print) IS - 1042-1882 (Linking) VI - 25 IP - 3 DP - 2013 May TI - Raynaud's: coping with a medical phenomenon. PG - 6 LA - eng PT - Journal Article PL - United States TA - Johns Hopkins Med Lett Health After 50 JT - The Johns Hopkins medical letter health after 50 JID - 9802902 MH - *Adaptation, Psychological MH - Humans MH - *Raynaud Disease EDAT- 2013/05/01 00:00 MHDA- 2016/04/24 06:00 CRDT- 2016/03/31 06:00 PHST- 2016/03/31 06:00 [entrez] PHST- 2013/05/01 00:00 [pubmed] PHST- 2016/04/24 06:00 [medline] PST - ppublish SO - Johns Hopkins Med Lett Health After 50. 2013 May;25(3):6. PMID- 19171245 OWN - NLM STAT- MEDLINE DCOM- 20090423 LR - 20161125 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 136 IP - 1 DP - 2009 Jan TI - [Intravenous fluorescein and Raynaud's phenomenon]. PG - 96 LID - 10.1016/j.annder.2008.10.025 [doi] FAU - Schmutz, J-L AU - Schmutz JL AD - Service de dermatologie, hôpital Fournier, 36, quai de la Bataille, 54035 Nancy cedex, France. jl.schmutz@chu-nancy.fr FAU - Barbaud, A AU - Barbaud A FAU - Tréchot, P AU - Tréchot P LA - fre PT - Case Reports PT - Journal Article TT - Fluorescéine par voie intraveineuse et syndrome de Raynaud. DEP - 20081216 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Contrast Media) RN - TPY09G7XIR (Fluorescein) SB - IM MH - Aged MH - Contrast Media/*adverse effects MH - Fluorescein/*adverse effects MH - Humans MH - Male MH - Radiography MH - Raynaud Disease/*chemically induced MH - Retinal Artery/diagnostic imaging EDAT- 2009/01/28 09:00 MHDA- 2009/04/25 09:00 CRDT- 2009/01/28 09:00 PHST- 2009/01/28 09:00 [entrez] PHST- 2009/01/28 09:00 [pubmed] PHST- 2009/04/25 09:00 [medline] AID - S0151-9638(08)00760-6 [pii] AID - 10.1016/j.annder.2008.10.025 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2009 Jan;136(1):96. doi: 10.1016/j.annder.2008.10.025. Epub 2008 Dec 16. PMID- 23550672 OWN - NLM STAT- MEDLINE DCOM- 20130411 LR - 20130404 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 368 IP - 14 DP - 2013 Apr 4 TI - Images in clinical medicine. Primary Raynaud's phenomenon. PG - 1344 LID - 10.1056/NEJMicm1209600 [doi] FAU - Rodgers, Michael AU - Rodgers M AD - University Medical Center Groningen, Groningen, The Netherlands. m.g.g.rodgers@umcg.nl LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM MH - Adult MH - Female MH - Hand/*pathology MH - Humans MH - Raynaud Disease/*pathology EDAT- 2013/04/05 06:00 MHDA- 2013/04/12 06:00 CRDT- 2013/04/05 06:00 PHST- 2013/04/05 06:00 [entrez] PHST- 2013/04/05 06:00 [pubmed] PHST- 2013/04/12 06:00 [medline] AID - 10.1056/NEJMicm1209600 [doi] PST - ppublish SO - N Engl J Med. 2013 Apr 4;368(14):1344. doi: 10.1056/NEJMicm1209600. PMID- 22335764 OWN - NLM STAT- MEDLINE DCOM- 20120224 LR - 20171116 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 366 IP - 7 DP - 2012 Feb 16 TI - Images in clinical medicine. Lingual Raynaud's phenomenon. PG - e12 LID - 10.1056/NEJMicm1104140 [doi] FAU - Katada, Yoshinori AU - Katada Y AD - National Hospital Organization Osaka-Minami Medical Center, Kawachinagano City, Japan. kataday@omh.hosp.go.jp FAU - Tanaka, Toshio AU - Tanaka T LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM MH - Cell Phone MH - Female MH - Humans MH - Photography MH - Raynaud Disease/*pathology MH - Tongue/*pathology MH - Tongue Diseases/*pathology MH - Young Adult EDAT- 2012/02/18 06:00 MHDA- 2012/03/01 06:00 CRDT- 2012/02/17 06:00 PHST- 2012/02/17 06:00 [entrez] PHST- 2012/02/18 06:00 [pubmed] PHST- 2012/03/01 06:00 [medline] AID - 10.1056/NEJMicm1104140 [doi] PST - ppublish SO - N Engl J Med. 2012 Feb 16;366(7):e12. doi: 10.1056/NEJMicm1104140. PMID- 20390278 OWN - NLM STAT- MEDLINE DCOM- 20120509 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 31 IP - 7 DP - 2011 Jul TI - Primary and secondary Raynaud's phenomenon: does small neuropathy remain the same? PG - 977-8 LID - 10.1007/s00296-010-1491-5 [doi] FAU - Bajocchi, Gianluigi AU - Bajocchi G FAU - Terlizzi, Rossana AU - Terlizzi R FAU - Chiarolanza, Ilaria AU - Chiarolanza I FAU - Cortelli, Pietro AU - Cortelli P LA - eng PT - Comment PT - Letter PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM CON - Rheumatol Int. 2011 May;31(5):577-85. doi: 10.1007/s00296-009-1293-9. PMID: 20035332 MH - Autonomic Nervous System/*physiopathology MH - Female MH - Humans MH - *Nerve Fibers MH - Peripheral Nervous System Diseases/*physiopathology MH - Raynaud Disease/*physiopathology EDAT- 2010/04/15 06:00 MHDA- 2012/05/10 06:00 CRDT- 2010/04/15 06:00 PHST- 2010/01/27 00:00 [received] PHST- 2010/03/27 00:00 [accepted] PHST- 2010/04/15 06:00 [entrez] PHST- 2010/04/15 06:00 [pubmed] PHST- 2012/05/10 06:00 [medline] AID - 10.1007/s00296-010-1491-5 [doi] PST - ppublish SO - Rheumatol Int. 2011 Jul;31(7):977-8. doi: 10.1007/s00296-010-1491-5. PMID- 24248320 OWN - NLM STAT- MEDLINE DCOM- 20140617 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2013 DP - 2013 Nov 18 TI - Severe digital necrosis in a 4-year-old boy: primary Raynaud's or jellyfish sting. LID - 10.1136/bcr-2013-201478 [doi] LID - bcr2013201478 AB - Raynaud's phenomena is a common disorder which may be primary or secondary to some connective tissue disorders such as systemic sclerosis and systemic lupus erythematosus. Jellyfish sting is a rare but life-threatening cause of Raynaud's phenomena. Digital gangrene is reported in 3% of children with secondary Raynaud's phenomena but does not occur in children with primary Raynaud's phenomena. We report a case of a 4-year-old boy who initially presented with episodes of pain and bluish to blackish discolouration and necrosis affecting the fingers on both hands after a jellyfish sting without any sign of connective tissue disorder. FAU - Binnetoglu, Fatih Koksal AU - Binnetoglu FK AD - Pediatric Cardiology Department, Faculty of Medicine, Onsekiz Mart University, Canakkale, Turkey. FAU - Kizildag, Betul AU - Kizildag B FAU - Topaloglu, Naci AU - Topaloglu N FAU - Kasapcopur, Ozgur AU - Kasapcopur O LA - eng PT - Case Reports PT - Journal Article DEP - 20131118 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Animals MH - Bites and Stings/*complications MH - Child, Preschool MH - Fingers/*pathology MH - Gangrene/diagnosis/drug therapy/*etiology MH - Humans MH - Iloprost/administration & dosage MH - Male MH - Raynaud Disease/diagnosis/drug therapy/*etiology MH - *Scyphozoa MH - Vasodilator Agents/administration & dosage PMC - PMC3841385 EDAT- 2013/11/20 06:00 MHDA- 2014/06/18 06:00 PMCR- 2015/11/18 CRDT- 2013/11/20 06:00 PHST- 2013/11/20 06:00 [entrez] PHST- 2013/11/20 06:00 [pubmed] PHST- 2014/06/18 06:00 [medline] PHST- 2015/11/18 00:00 [pmc-release] AID - bcr-2013-201478 [pii] AID - 10.1136/bcr-2013-201478 [doi] PST - epublish SO - BMJ Case Rep. 2013 Nov 18;2013:bcr2013201478. doi: 10.1136/bcr-2013-201478. PMID- 21476099 OWN - NLM STAT- MEDLINE DCOM- 20120821 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 5 DP - 2012 May TI - Effect of biofeedback and deep oscillation on Raynaud's phenomenon secondary to systemic sclerosis: results of a controlled prospective randomized clinical trial. PG - 1469-73 LID - 10.1007/s00296-011-1882-2 [doi] AB - Our aim was to evaluate the effect of deep oscillation and biofeedback on Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). A prospective randomized study was performed in SSc patients receiving either deep oscillation (n = 10) or biofeedback (n = 8) thrice a week for 4 weeks, or patients were randomized into the waiting group untreated for vasculopathy (n = 10) in time of running the study interventions. Biofeedback resulted in an improvement of RP as determined by score reduction of visual analogue scale compared with patients of the control group (P < 0.05), whereas deep oscillation revealed a tendency for improvement (P = 0.055). The study underlines the beneficial role of physiotherapy for the treatment of SSc-related RP. FAU - Sporbeck, Birte AU - Sporbeck B AD - Department of Physical Medicine and Rehabilitation, Charité University Hospital, Charitéplatz 1, 10117 Berlin, Germany. birte.sporbeck@charite.de FAU - Mathiske-Schmidt, Kirsten AU - Mathiske-Schmidt K FAU - Jahr, Silke AU - Jahr S FAU - Huscher, Dörte AU - Huscher D FAU - Becker, Mike AU - Becker M FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Taufmann, Ines AU - Taufmann I FAU - Burmester, Gerd-Rüdiger AU - Burmester GR FAU - Pögel, Stephanie AU - Pögel S FAU - Reisshauer, Anett AU - Reisshauer A LA - eng SI - ClinicalTrials.gov/NCT00946738 PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20110408 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Analysis of Variance MH - *Biofeedback, Psychology MH - Chi-Square Distribution MH - Female MH - Germany MH - Humans MH - Linear Models MH - *Magnetic Field Therapy MH - Male MH - Middle Aged MH - *Physical Therapy Modalities MH - Prospective Studies MH - Raynaud Disease/diagnosis/etiology/*therapy MH - Scleroderma, Systemic/complications/diagnosis/*therapy MH - Severity of Illness Index MH - Time Factors MH - Treatment Outcome EDAT- 2011/04/09 06:00 MHDA- 2012/08/22 06:00 CRDT- 2011/04/09 06:00 PHST- 2010/12/21 00:00 [received] PHST- 2011/02/18 00:00 [accepted] PHST- 2011/04/09 06:00 [entrez] PHST- 2011/04/09 06:00 [pubmed] PHST- 2012/08/22 06:00 [medline] AID - 10.1007/s00296-011-1882-2 [doi] PST - ppublish SO - Rheumatol Int. 2012 May;32(5):1469-73. doi: 10.1007/s00296-011-1882-2. Epub 2011 Apr 8. PMID- 21790846 OWN - NLM STAT- MEDLINE DCOM- 20120105 LR - 20151119 IS - 1524-4725 (Electronic) IS - 1076-0512 (Linking) VI - 37 IP - 10 DP - 2011 Oct TI - Scleroderma and raynaud's phenomenon improve with high-peak power laser therapy: a case report. PG - 1531-5 LID - 10.1111/j.1524-4725.2011.02093.x [doi] FAU - St Surin-Lord, Sharleen AU - St Surin-Lord S AD - Department of Dermatology, Howard University College of Medicine, Washington, District of Columbia, USA. FAU - Obagi, Suzan AU - Obagi S LA - eng PT - Case Reports PT - Journal Article DEP - 20110725 PL - United States TA - Dermatol Surg JT - Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] JID - 9504371 SB - IM MH - Adult MH - Female MH - Humans MH - Lasers, Solid-State/*therapeutic use MH - *Low-Level Light Therapy MH - Raynaud Disease/complications/*radiotherapy MH - Scleroderma, Systemic/complications/*radiotherapy EDAT- 2011/07/28 06:00 MHDA- 2012/01/06 06:00 CRDT- 2011/07/28 06:00 PHST- 2011/07/28 06:00 [entrez] PHST- 2011/07/28 06:00 [pubmed] PHST- 2012/01/06 06:00 [medline] AID - 10.1111/j.1524-4725.2011.02093.x [doi] PST - ppublish SO - Dermatol Surg. 2011 Oct;37(10):1531-5. doi: 10.1111/j.1524-4725.2011.02093.x. Epub 2011 Jul 25. PMID- 41804625 OWN - NLM STAT- MEDLINE DCOM- 20260310 LR - 20260310 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 33 IP - 2 DP - 2025 Sep TI - A Rare Case of Paraneoplastic Raynaud's Phenomenon and Uveal Melanoma. PG - 89-91 AB - Raynaud's phenomenon (RP) presents as acral skin pallor, cyanosis, and erythema, usually after cold exposure or emotional stress. Symptoms of RP affect 3-5% of the general population, with the incidence four times higher in women than in men. Paraneoplastic RP is extremely rare and is thought to involve plasma hyperviscosity and blood hypercoagulability, which are present in patients with malignant diseases. Paraneoplastic RP often presents abruptly and, besides changes in skin color, it includes erosions, ulcerations, and necrosis, resulting in severe pain. We present a case of a 62-year-old female patient who suddenly developed symptoms of RP, characterized by periodic skin pallor without erosions or associated pain in all fingers, lasting 10-15 minutes after cold exposure. She was diagnosed with uveal melanoma three months prior and was also in a 14-year remission from invasive ductal carcinoma. Investigations confirmed positive antinuclear antibodies (ANA) with PCNA (proliferating cell nuclear antigen) and myositis-specific antibodies including anti-Jo, anti-mitochondrial antibody (AMA-M2), and anti-benzylpenicilloyl antibody (BPO). FAU - Vasari, Lara AU - Vasari L FAU - Špoljarić Carević, Sanda AU - Špoljarić Carević S AD - Sanda Špoljarić Carević, MD , Special Hospital for Medical,, Rehabilitation Naftalan, Ivanic Grad, Croatia; ssanda1977@gmail.com. FAU - Tomić Babić, Lucija AU - Tomić Babić L FAU - Bakula, Marija AU - Bakula M LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 SB - IM MH - Humans MH - *Melanoma/complications/diagnosis MH - *Raynaud Disease/etiology/diagnosis MH - Middle Aged MH - Female MH - Uveal Melanoma MH - *Uveal Neoplasms/complications/diagnosis MH - *Paraneoplastic Syndromes EDAT- 2026/03/10 07:00 MHDA- 2026/03/10 07:01 CRDT- 2026/03/10 04:51 PHST- 2026/03/10 07:01 [medline] PHST- 2026/03/10 07:00 [pubmed] PHST- 2026/03/10 04:51 [entrez] PST - ppublish SO - Acta Dermatovenerol Croat. 2025 Sep;33(2):89-91. PMID- 24794188 OWN - NLM STAT- MEDLINE DCOM- 20150831 LR - 20141216 IS - 1878-7533 (Electronic) IS - 1550-7289 (Linking) VI - 10 IP - 6 DP - 2014 Nov-Dec TI - A curious onset of Raynaud's phenomenon after gastric bypass: a case report. PG - e109-11 LID - S1550-7289(14)00020-3 [pii] LID - 10.1016/j.soard.2014.01.011 [doi] FAU - Nunes Ferreira, Afonso AU - Nunes Ferreira A AD - Faculty of Medicine, University of Lisbon, Lisbon, Portugal. Electronic address: afonsonunesferreira@gmail.com. FAU - Aldersley, Rachael AU - Aldersley R AD - Faculty of Medicine, Imperial College London, London, United Kingdom. FAU - Efthimiou, E AU - Efthimiou E AD - Chelsea and Westminster Hospital, London, United Kingdom. LA - eng PT - Case Reports PT - Journal Article DEP - 20140122 PL - United States TA - Surg Obes Relat Dis JT - Surgery for obesity and related diseases : official journal of the American Society for Bariatric Surgery JID - 101233161 SB - IM MH - Body Mass Index MH - Female MH - *Gastric Bypass MH - Humans MH - Middle Aged MH - Obesity, Morbid/*surgery MH - Raynaud Disease/*etiology OTO - NOTNLM OT - Laparoscopic Roux-en-Y gastric bypass OT - Raynaud disease OT - Raynaud phenomenon EDAT- 2014/05/06 06:00 MHDA- 2015/09/01 06:00 CRDT- 2014/05/06 06:00 PHST- 2014/01/05 00:00 [received] PHST- 2014/01/06 00:00 [accepted] PHST- 2014/05/06 06:00 [entrez] PHST- 2014/05/06 06:00 [pubmed] PHST- 2015/09/01 06:00 [medline] AID - S1550-7289(14)00020-3 [pii] AID - 10.1016/j.soard.2014.01.011 [doi] PST - ppublish SO - Surg Obes Relat Dis. 2014 Nov-Dec;10(6):e109-11. doi: 10.1016/j.soard.2014.01.011. Epub 2014 Jan 22. PMID- 17011135 OWN - NLM STAT- MEDLINE DCOM- 20070404 LR - 20061212 IS - 0306-9877 (Print) IS - 0306-9877 (Linking) VI - 68 IP - 2 DP - 2007 TI - Accompaniment of primary Raynaud's phenomenon with epilepsy: A similarity in the pathophysiologic process? PG - 462-3 FAU - Gulcan, Erim AU - Gulcan E FAU - Gulcan, Aynur AU - Gulcan A FAU - Kabay, Sibel AU - Kabay S FAU - Ozbek, Orhan AU - Ozbek O FAU - Ilhan, Demet AU - Ilhan D LA - eng PT - Case Reports PT - Letter DEP - 20060929 PL - United States TA - Med Hypotheses JT - Medical hypotheses JID - 7505668 SB - IM MH - Adult MH - Electroencephalography MH - Epilepsy/*complications/physiopathology MH - Female MH - Humans MH - Raynaud Disease/*complications/physiopathology MH - Vasoconstriction MH - Vasospasm, Intracranial/physiopathology EDAT- 2006/10/03 09:00 MHDA- 2007/04/05 09:00 CRDT- 2006/10/03 09:00 PHST- 2006/07/23 00:00 [received] PHST- 2006/07/29 00:00 [accepted] PHST- 2006/10/03 09:00 [pubmed] PHST- 2007/04/05 09:00 [medline] PHST- 2006/10/03 09:00 [entrez] AID - S0306-9877(06)00568-8 [pii] AID - 10.1016/j.mehy.2006.07.035 [doi] PST - ppublish SO - Med Hypotheses. 2007;68(2):462-3. doi: 10.1016/j.mehy.2006.07.035. Epub 2006 Sep 29. PMID- 16309469 OWN - NLM STAT- MEDLINE DCOM- 20060502 LR - 20081121 IS - 0307-6938 (Print) IS - 0307-6938 (Linking) VI - 31 IP - 1 DP - 2006 Jan TI - Infrared-monitored cold response in the assessment of Raynaud's phenomenon. PG - 6-12 AB - BACKGROUND: Evaluation of treatments for Raynaud's phenomenon (RP) requires objective response parameters in addition to clinical activity scores. Thermographic monitoring of fingertip re-warming after cold challenge has been widely used but usually requires sophisticated equipment. We have previously shown that fingertip re-warming after cold challenge follows a first-order transient response curve that can be described by a single variable, designated tau. OBJECTIVES: Here, we describe a novel device termed a duosensor, which records the tau value upon cold challenge in an automated manner. METHODS: We determined tau values in healthy probands, patients with primary or secondary RP associated with autoimmune disease and patients with scleroderma-associated RP following cold challenge, to determine assay variability, sensitivity and specificity. RESULTS: Duosensor-based thermography exhibited low intraindividual variability in healthy probands. As expected, tau values in RP patients were significantly increased compared with controls (8.08 +/- 3.65 min vs. 3.23 +/- 1.65 min). The duosensor-determined tau value yielded a specificity of 94.6% and predictive value of 95.3% for the presence of RP in a retrospective analysis of 139 patients. Furthermore, in a cohort of scleroderma patients with RP, patient self-assessment of RP severity correlated with tau values. CONCLUSIONS: Taken together, the present data suggest that tau value determination provides a suitable outcome measure for clinical studies of novel RP treatments. As the duosensor is a simple stand-alone device requiring no supporting equipment and minimal personnel attention, it should allow RP activity monitoring even in clinical settings with minimal technical infrastructure. FAU - Foerster, J AU - Foerster J AD - Clinic for Dermatology, Charité, Berlin, Germany. john.foerster@charite.de FAU - Wittstock, S AU - Wittstock S FAU - Fleischanderl, S AU - Fleischanderl S FAU - Storch, A AU - Storch A FAU - Riemekasten, G AU - Riemekasten G FAU - Hochmuth, O AU - Hochmuth O FAU - Meffert, B AU - Meffert B FAU - Meffert, H AU - Meffert H FAU - Worm, M AU - Worm M LA - eng PT - Journal Article PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 SB - IM MH - Body Temperature/physiology MH - Cohort Studies MH - *Cold Temperature MH - Fingers/*physiopathology MH - Humans MH - Infrared Rays MH - Plethysmography/instrumentation/methods MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis/physiopathology MH - Regional Blood Flow/physiology MH - Retrospective Studies MH - Scleroderma, Systemic/diagnosis/physiopathology MH - Sensitivity and Specificity MH - Severity of Illness Index MH - Thermography/methods EDAT- 2005/11/29 09:00 MHDA- 2006/05/04 09:00 CRDT- 2005/11/29 09:00 PHST- 2005/11/29 09:00 [pubmed] PHST- 2006/05/04 09:00 [medline] PHST- 2005/11/29 09:00 [entrez] AID - CED1995 [pii] AID - 10.1111/j.1365-2230.2005.01995.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2006 Jan;31(1):6-12. doi: 10.1111/j.1365-2230.2005.01995.x. PMID- 18187020 OWN - NLM STAT- MEDLINE DCOM- 20160423 LR - 20181201 IS - 0393-974X (Print) IS - 0393-974X (Linking) VI - 20 IP - 3-4 DP - 2006 Jul-Dec TI - Study on the effectiveness of a nifedipine gel for treatment of Raynaud?s phenomenon. PG - 59-65 AB - The aim of this study is to evaluate the efficacy of a nifedipine gel in patients with primary or secondary Raynaud?s phenomenon. Photopletismography was the instrumental examination test used to evaluate recovery time (time necessary for recuperation of normal capillary circulation) in 17 patients with primary or secondary Raynaud?s phenomenon before and after the application of the gel. It emerged that of the 17 patients who used the gel, in 3 cases the recovery time was reduced, in 9 cases the recovery time was cancelled (no spasm occurred), in 5 cases the recovery time was not modified. Therefore, in more than 70 percent of patients the drug had a positive effect. Besides, 50 percent of the patients referred an improvement of the subjective symptomatology with reduction of cooling, torpidity, ache and paresthesias of the fingers. The results obtained, even if related to a restricted number of patients and to a brief interval of time, show the effectiveness of this drug in patients with primary or secondary Raynaud?s phenomenon. We believe that these results, presented here for the first time, are important for investigators involved in the study of Raynaud?s disease. FAU - Foti, C AU - Foti C AD - Department of Internal Medicine, Immunology and Infectious Diseases Unit of Dermatology, University of Bari, Bari, Italy. FAU - Quaranta, D AU - Quaranta D FAU - Pepe, M L AU - Pepe ML FAU - Morea, G AU - Morea G FAU - Mastropasqua, D AU - Mastropasqua D FAU - D'Amore, M AU - D'Amore M FAU - Mastrangelo, F AU - Mastrangelo F FAU - Tete, S AU - Tete S FAU - Grassi, F R AU - Grassi FR FAU - Ballini, A AU - Ballini A FAU - Salini, V AU - Salini V FAU - De Amicis, D AU - De Amicis D FAU - Scagliusi, P AU - Scagliusi P FAU - De Lutiis, M A AU - De Lutiis MA FAU - Caraffa, A AU - Caraffa A FAU - Cerulli, G AU - Cerulli G LA - eng PT - Journal Article PL - Singapore TA - J Biol Regul Homeost Agents JT - Journal of biological regulators and homeostatic agents JID - 8809253 RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Fingers MH - Humans MH - *Nifedipine MH - Pain MH - *Raynaud Disease EDAT- 2008/01/12 09:00 MHDA- 2016/04/24 06:00 CRDT- 2008/01/12 09:00 PHST- 2008/01/12 09:00 [pubmed] PHST- 2016/04/24 06:00 [medline] PHST- 2008/01/12 09:00 [entrez] AID - 3 [pii] PST - ppublish SO - J Biol Regul Homeost Agents. 2006 Jul-Dec;20(3-4):59-65. PMID- 16741592 OWN - NLM STAT- MEDLINE DCOM- 20070403 LR - 20181113 IS - 1861-0684 (Print) IS - 1861-0684 (Linking) VI - 95 IP - 6 DP - 2006 Jun TI - Coronary vasospasm-induced acute diastolic dysfunction in a patient with Raynaud's phenomenon. PG - 344-8 AB - We present the case of a patient with severe dyspnea and Raynaud's phenomenon. We could clarify, using invasive techniques including left ventricular conductance catheterization and coronary ergonovine provocation, that isolated diastolic dysfunction induced by coronary vasospasm were responsible for the symptoms. Systolic function was not affected. Short-term infusions with the prostacyclin analogue iloprost, known to act as a disease-modifying agent in patients suffering from Raynaud's phenomenon, led to an improvement of cardiac function. Thus, episodes of dyspnea in patients with Raynaud's phenomenon might be also interpreted as a coronary ischemia equivalent, which may belong to a visceral form of Raynaud's phenomenon and which are sensitive to iloprost infusions. FAU - Tschöpe, Carsten AU - Tschöpe C AD - Charitè-University Medicine Berlin, Campus Benjamin Franklin, Department of Cardiology and Pneumology, Hindenburgdamm 30, 12200, Berlin, Germany. Carsten.Tschoepe@charite.de FAU - Westermann, Dirk AU - Westermann D FAU - Steendijk, Paul AU - Steendijk P FAU - Kasner, Mario AU - Kasner M FAU - Rudwaleit, Martin AU - Rudwaleit M FAU - Schwimmbeck, Peter L AU - Schwimmbeck PL FAU - Poller, Wolfgang C AU - Poller WC FAU - Schultheiss, Heinz-Peter AU - Schultheiss HP LA - eng PT - Case Reports PT - Journal Article DEP - 20060518 PL - Germany TA - Clin Res Cardiol JT - Clinical research in cardiology : official journal of the German Cardiac Society JID - 101264123 SB - IM MH - Coronary Vasospasm/*complications/diagnosis/therapy MH - Diastole MH - Female MH - Heart Failure/diagnosis/*etiology/therapy MH - Humans MH - Middle Aged MH - Raynaud Disease/*complications EDAT- 2006/06/03 09:00 MHDA- 2007/04/04 09:00 CRDT- 2006/06/03 09:00 PHST- 2005/08/16 00:00 [received] PHST- 2006/03/15 00:00 [accepted] PHST- 2006/06/03 09:00 [pubmed] PHST- 2007/04/04 09:00 [medline] PHST- 2006/06/03 09:00 [entrez] AID - 10.1007/s00392-006-0384-9 [doi] PST - ppublish SO - Clin Res Cardiol. 2006 Jun;95(6):344-8. doi: 10.1007/s00392-006-0384-9. Epub 2006 May 18. PMID- 23754794 OWN - NLM STAT- MEDLINE DCOM- 20140610 LR - 20140602 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 65 IP - 11 DP - 2013 Nov TI - Reliability of widefield nailfold capillaroscopy and video capillaroscopy in the assessment of patients with Raynaud’s phenomenon. PG - 1853-61 AB - OBJECTIVE: To analyze the diagnostic performance and reliability of different parameters evaluated by widefield nailfold capillaroscopy (NFC) with those obtained by video capillaroscopy in patients with Raynaud’s phenomenon (RP). METHODS: Two hundred fifty-two individuals were assessed, including 101 systemic sclerosis (SSc; scleroderma) patients,61 patients with undifferentiated connective tissue disease, 37 patients with primary RP, and 53 controls. Widefield NFC was performed using a stereomicroscope under 10–25 x magnification and direct measurement of all parameters. Video capillaroscopy was performed under 200 x magnification, with the acquirement of 32 images per individual (4 fields per finger in 8 fingers). The following parameters were analyzed in 8 fingers of the hands (excluding thumbs) by both methods: number of capillaries/mm, number of enlarged and giant capillaries, microhemorrhages, and avascular score.Intra- and interobserver reliability was evaluated by performing both examinations in 20 individuals on 2 different days and by 2 long-term experienced observers. RESULTS: There was a significant correlation (P < 0.000) between widefield NFC and video capillaroscopy in the comparison of all parameters. Kappa values and intraclass correlation coefficient analysis showed excellent intra- and interobserver reproducibility for all parameters evaluated by widefield NFC and video capillaroscopy. Bland-Altman analysis showed high agreement of all parameters evaluated in both methods. According to receiver operating characteristic curve analysis, both methods showed a similar performance in discriminating SSc patients from controls. CONCLUSION: Widefield NFC and video capillaroscopy are reliable and accurate methods and can be used equally for assessing peripheral microangiopathy in RP and SSc patients. Nonetheless, the high reliability obtained may not be similar for less experienced examiners. FAU - Sekiyama, Juliana Y AU - Sekiyama JY FAU - Camargo, Cintia Z AU - Camargo CZ FAU - Eduardo, Luís AU - Eduardo L FAU - Andrade, C AU - Andrade C FAU - Kayser, Cristiane AU - Kayser C LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Capillaries/physiopathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microcirculation/*physiology MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - ROC Curve MH - Raynaud Disease/*diagnosis/physiopathology MH - Reproducibility of Results MH - Severity of Illness Index MH - Video Recording EDAT- 2013/06/12 06:00 MHDA- 2014/06/11 06:00 CRDT- 2013/06/12 06:00 PHST- 2013/01/08 00:00 [received] PHST- 2013/05/05 00:00 [revised] PHST- 2013/05/24 00:00 [accepted] PHST- 2013/06/12 06:00 [entrez] PHST- 2013/06/12 06:00 [pubmed] PHST- 2014/06/11 06:00 [medline] AID - 10.1002/acr.22054 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2013 Nov;65(11):1853-61. doi: 10.1002/acr.22054. PMID- 24720153 OWN - NLM STAT- MEDLINE DCOM- 20151022 LR - 20260128 IS - 0024-3477 (Print) IS - 0024-3477 (Linking) VI - 136 IP - 1-2 DP - 2014 Jan-Feb TI - [Capillaroscopy in rheumatological practice--one center experience]. PG - 28-32 AB - Capillaroscopy is a method for evaluating morphological characteristics of nailfold capillaries. The simplicity, noninvasiveness and easiness-to-perform make the method accessible in everyday rheumatological practice. Raynaud's phenomenon is the main indication for performing capillaroscopy (differentiating between primary and secondary Raynaud's phenomenon) and diagnosing early stages of systemic sclerosis. According to some authors capillaroscopy should be included in the work-up algorithm for patients with puffy fingers and Raynaud's phenomenon. Other autoimmune conditions (systemic lupus erythematosus, polymyositis/dermatomyositis, mixed connective tissue disease, antiphospholipid syndrome and other diseases which affect microvasculature - diabetes mellitus, thromboangiitis obliterans) can have some abnormalities of the capillaroscopic pattern. We present the results of the capillaroscopies performed in our center during the period of one year. FAU - Baresić, Marko AU - Baresić M FAU - Anić, Branimir AU - Anić B LA - hrv PT - Journal Article TT - Kapilaroskopija u reumatoloskoj praksi--iskustvo jednog centra. PL - Croatia TA - Lijec Vjesn JT - Lijecnicki vjesnik JID - 0074253 SB - IM MH - Algorithms MH - Arthritis, Rheumatoid/*pathology/therapy MH - Capillaries/pathology MH - Connective Tissue Diseases/*pathology/therapy MH - Croatia MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/methods/*statistics & numerical data MH - Raynaud Disease/*pathology/therapy EDAT- 2014/04/12 06:00 MHDA- 2015/10/23 06:00 CRDT- 2014/04/12 06:00 PHST- 2014/04/12 06:00 [entrez] PHST- 2014/04/12 06:00 [pubmed] PHST- 2015/10/23 06:00 [medline] PST - ppublish SO - Lijec Vjesn. 2014 Jan-Feb;136(1-2):28-32. PMID- 16816957 OWN - NLM STAT- MEDLINE DCOM- 20090514 LR - 20181113 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 27 IP - 1 DP - 2006 Nov TI - A case of hepatocellular carcinoma who admitted with Raynaud's phenomenon. PG - 87-9 AB - Hepatocellular carcinoma (HCC) may be present in one of the several ways. The classical presentation is with right upper quadrant abdominal pain, abdominal swelling and weight loss. HCC may also be associated with several paraneoplastic manifestations. The mechanisms of these manifestations are not well known. The association of digital ischemia and malignancy was reported as early as 1884 and 1891. We report a case of HCC associated with Raynaund's phenomenon. FAU - Sahan, Cem AU - Sahan C AD - Internal Medicine Department, Samsun Gazi Region Hospital, Samsun, Turkey. sahancem@yahoo.com FAU - Ucer, Tanja AU - Ucer T FAU - Aksakal, Emre AU - Aksakal E LA - eng PT - Case Reports PT - Journal Article DEP - 20060701 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Aged MH - Biopsy MH - Carcinoma, Hepatocellular/*complications/*diagnosis/pathology MH - Humans MH - Liver/pathology MH - Liver Neoplasms/*complications/*diagnosis/pathology MH - Male MH - Raynaud Disease/*complications/*diagnosis EDAT- 2006/07/04 09:00 MHDA- 2009/05/15 09:00 CRDT- 2006/07/04 09:00 PHST- 2005/03/30 00:00 [received] PHST- 2006/02/20 00:00 [accepted] PHST- 2006/07/04 09:00 [pubmed] PHST- 2009/05/15 09:00 [medline] PHST- 2006/07/04 09:00 [entrez] AID - 10.1007/s00296-006-0154-z [doi] PST - ppublish SO - Rheumatol Int. 2006 Nov;27(1):87-9. doi: 10.1007/s00296-006-0154-z. Epub 2006 Jul 1. PMID- 26075287 OWN - NLM STAT- MEDLINE DCOM- 20160303 LR - 20181113 IS - 2314-7156 (Electronic) IS - 2314-8861 (Print) IS - 2314-7156 (Linking) VI - 2015 DP - 2015 TI - Uniphasic Blanching of the Fingers, Abnormal Capillaroscopy in Nonsymptomatic Digits, and Autoantibodies: Expanding Options to Increase the Level of Suspicion of Connective Tissue Diseases beyond the Classification of Raynaud's Phenomenon. PG - 371960 LID - 10.1155/2015/371960 [doi] LID - 371960 AB - In patients with Raynaud's phenomenon (RP), the role of medical history, capillaroscopy, and autoantibodies in order to provide an early diagnosis of connective tissue disease (CTD) were examined. 115 consecutive adults with uni-, bi-, or triphasic colour changes of the fingers were studied. RP was bilateral in 92.7% of patients. The middle finger was significantly more affected. A lack of association between fingers affected by RP and fingers with capillary abnormalities was observed OR = 0.75 (0.34-1.66). RP with the cyanotic phase had a higher risk at capillaroscopy to have hemorrhages OR = 4.46 (1.50-13.30) and giant capillaries OR = 24.85 (1.48-417.44). The thumb and triphasic involvement have an OR of 1.477 and 1.845, respectively. RP secondary to systemic sclerosis (SSc) had greater value of VAS pain (p = 0.011). The presence of anti-centromere antibodies was significantly associated with a higher risk of SSc (p < 0.001). 44.3% of subjects had uniphasic blanching of the fingers, and among these, 27% was diagnosed as having an overt or suspected CTD. Markers of a potential development of CTDs include severe RP symptoms, positive autoantibodies, and capillary abnormalities. These data support the proposal to not discharge patients with uniphasic blanching of the fingers to avoid missing the opportunity of an early diagnosis. FAU - Ingegnoli, Francesca AU - Ingegnoli F AUID- ORCID: 0000-0002-6727-1273 AD - Department of Clinical Science & Community Health, University of Milano, 20122 Milan, Italy ; Division of Rheumatology, Istituto Gaetano Pini, 20122 Milan, Italy. FAU - Gualtierotti, Roberta AU - Gualtierotti R AD - Department of Clinical Science & Community Health, University of Milano, 20122 Milan, Italy ; Division of Rheumatology, Istituto Gaetano Pini, 20122 Milan, Italy. FAU - Orenti, Annalisa AU - Orenti A AD - Department of Clinical Science & Community Health, University of Milano, 20122 Milan, Italy ; Medical Statistics and Biometry, 20122 Milan, Italy. FAU - Schioppo, Tommaso AU - Schioppo T AD - Division of Rheumatology, Istituto Gaetano Pini, 20122 Milan, Italy. FAU - Marfia, Giovanni AU - Marfia G AD - Laboratory of Experimental Neurosurgery and Cell Therapy, Neurosurgery Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, 20122 Milan, Italy. FAU - Campanella, Rolando AU - Campanella R AD - Laboratory of Experimental Neurosurgery and Cell Therapy, Neurosurgery Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, 20122 Milan, Italy. FAU - Mastaglio, Claudio AU - Mastaglio C AD - Rheumatology Unit, Ospedale Moriggia-Pelascini, 22015 Gravedona, Italy. FAU - Meroni, Pier Luigi AU - Meroni PL AD - Department of Clinical Science & Community Health, University of Milano, 20122 Milan, Italy ; Division of Rheumatology, Istituto Gaetano Pini, 20122 Milan, Italy. FAU - Boracchi, Patrizia AU - Boracchi P AD - Department of Clinical Science & Community Health, University of Milano, 20122 Milan, Italy ; Medical Statistics and Biometry, 20122 Milan, Italy. LA - eng PT - Journal Article DEP - 20150517 PL - Egypt TA - J Immunol Res JT - Journal of immunology research JID - 101627166 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/*immunology MH - Connective Tissue Diseases/diagnosis/*immunology/*pathology MH - Early Diagnosis MH - Female MH - Fingers/*pathology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/diagnosis/immunology/*pathology MH - Scleroderma, Systemic/diagnosis/immunology/pathology PMC - PMC4449942 EDAT- 2015/06/16 06:00 MHDA- 2016/03/05 06:00 PMCR- 2015/05/17 CRDT- 2015/06/16 06:00 PHST- 2015/02/19 00:00 [received] PHST- 2015/05/02 00:00 [revised] PHST- 2015/05/04 00:00 [accepted] PHST- 2015/06/16 06:00 [entrez] PHST- 2015/06/16 06:00 [pubmed] PHST- 2016/03/05 06:00 [medline] PHST- 2015/05/17 00:00 [pmc-release] AID - 10.1155/2015/371960 [doi] PST - ppublish SO - J Immunol Res. 2015;2015:371960. doi: 10.1155/2015/371960. Epub 2015 May 17. PMID- 27830961 OWN - NLM STAT- MEDLINE DCOM- 20180326 LR - 20260127 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 27 IP - 4 DP - 2017 Jul TI - Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc. PG - 614-617 LID - 10.1080/14397595.2016.1250332 [doi] AB - OBJECTIVE: To classify Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria (new 2013 criteria). METHODS: We assessed 120 patients who visited Kanazawa University Hospital suspected of SSc and who did not meet the 1980 ACR preliminary classification criteria for SSc. We clinically diagnosed 16 patients with primary Raynaud's disease and 104 with mild/early SSc prior to being assessed by the new 2013 criteria. RESULTS: None of the 16 patients with primary Raynaud's disease met the new 2013 criteria. On the other hand, 94 out of the 104 patients (90.3%) with mild/early SSc by our clinical diagnosis met the new 2013 criteria. Among the 94 SSc patients, sclerodactyly was detected in 58 (62%), puffy fingers in 62 (66%), abnormal nailfold capillaries in 89 (95%), Raynaud's phenomenon in 93 (99%), and SSc-related autoantibodies (Abs) in 85 (90%). The median (range) score of these 94 patients was 12 (9-14). Ten mild/early SSc patients who did not meet the new 2013 criteria had the following clinical features: puffy fingers in 1 (10%), abnormal nailfold capillaries in 8 (80%), Raynaud's phenomenon in 9 (90%), and SSc-related autoAbs in 8 (80%). The median (range) score of these 10 patients was 7 (5-8). CONCLUSION: The new 2013 criteria can classify most mild/early Japanese SSc patients, which may contribute to early treatment interventions. FAU - Ikawa, Yuka AU - Ikawa Y AD - a Department of Molecular Pathology of Skin, Faculty of Medicine , Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University , Kanazawa , Japan and. FAU - Hamaguchi, Yasuhito AU - Hamaguchi Y AD - a Department of Molecular Pathology of Skin, Faculty of Medicine , Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University , Kanazawa , Japan and. FAU - Mugii, Naoki AU - Mugii N AD - b Department of Rehabilitation , Kanazawa University Hospital , Kanazawa , Japan. FAU - Matsushita, Takashi AU - Matsushita T AD - a Department of Molecular Pathology of Skin, Faculty of Medicine , Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University , Kanazawa , Japan and. FAU - Takehara, Kazuhiko AU - Takehara K AD - a Department of Molecular Pathology of Skin, Faculty of Medicine , Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University , Kanazawa , Japan and. LA - eng PT - Journal Article PT - Observational Study DEP - 20161114 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Japan MH - Male MH - Middle Aged MH - Physical Examination/standards MH - Raynaud Disease/*classification/diagnosis MH - Rheumatology/organization & administration/standards MH - Scleroderma, Systemic/*classification/diagnosis MH - Societies, Medical/standards OTO - NOTNLM OT - New classification criteria OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2016/11/11 06:00 MHDA- 2018/03/27 06:00 CRDT- 2016/11/11 06:00 PHST- 2016/11/11 06:00 [pubmed] PHST- 2018/03/27 06:00 [medline] PHST- 2016/11/11 06:00 [entrez] AID - 10.1080/14397595.2016.1250332 [doi] PST - ppublish SO - Mod Rheumatol. 2017 Jul;27(4):614-617. doi: 10.1080/14397595.2016.1250332. Epub 2016 Nov 14. PMID- 24820143 OWN - NLM STAT- MEDLINE DCOM- 20151214 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 34 IP - 3 DP - 2015 Mar TI - The influence of Multiwave Locked System (MLS) laser therapy on clinical features, microcirculatory abnormalities and selected modulators of angiogenesis in patients with Raynaud's phenomenon. PG - 489-96 LID - 10.1007/s10067-014-2637-8 [doi] AB - The aim of this study was to investigate the influence of the Multiwave Locked System (MLS) laser therapy on clinical features, microvascular changes in nailfold videocapillaroscopy (NVC) and circulating modulators releasing as a consequence of vascular endothelium injury such as vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang-2) in patients with primary and secondary Raynaud's phenomenon. Seventy-eight RP patients and 30 healthy volunteers were recruited into the study. All patients with RP received MLS laser irradiation for 3 weeks. Clinical, NVC and laboratory investigations were performed before and after the MLS laser therapy. The serum concentration of VEGF and Ang-2 were determined by an enzyme-linked immunosorbent assay (ELISA). After 3 weeks of MLS laser therapy, the clinical improvement manifested by decreasing of the number of RP attacks, mean duration of Raynaud's attack and pain intensity in RP patients was observed. After MLS laser therapy in 65% of patients with primary and in 35% with secondary RP, an increase in the loop number and/or a reduction in avascular areas in NVC were observed. In comparison with a control group, higher serum concentration of VEGF and Ang-2 in RP patients was demonstrated. After MLS laser therapy, a reduction of Ang-2 in both groups of RP patients was found. Our results suggest that NVC may reflect microvascular changes associated with clinical improvement after MLS laser therapy in patients with primary and secondary RP. Ang-2 serum levels may be a useful marker of microvascular abnormalities in RP patients treated with MLS laser therapy. FAU - Kuryliszyn-Moskal, Anna AU - Kuryliszyn-Moskal A AD - Department of Rehabilitation, Medical University of Bialystok, M. Sklodowskiej-Curie 24A, 15-276, Bialystok, Poland, akuryl@umb.edu.pl. FAU - Kita, Jacek AU - Kita J FAU - Dakowicz, Agnieszka AU - Dakowicz A FAU - Chwieśko-Minarowska, Sylwia AU - Chwieśko-Minarowska S FAU - Moskal, Diana AU - Moskal D FAU - Kosztyła-Hojna, Bożena AU - Kosztyła-Hojna B FAU - Jabłońska, Ewa AU - Jabłońska E FAU - Klimiuk, Piotr Adrian AU - Klimiuk PA LA - eng PT - Clinical Trial PT - Journal Article DEP - 20140513 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (VEGFA protein, human) RN - 0 (VPS51 protein, human) RN - 0 (Vascular Endothelial Growth Factor A) RN - 0 (Vesicular Transport Proteins) SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - Humans MH - *Laser Therapy MH - Male MH - Microcirculation MH - Microvessels/pathology MH - Middle Aged MH - Raynaud Disease/blood/pathology/*therapy MH - Vascular Endothelial Growth Factor A/blood MH - Vesicular Transport Proteins/blood MH - Young Adult PMC - PMC4348551 EDAT- 2014/05/14 06:00 MHDA- 2015/12/15 06:00 PMCR- 2014/05/13 CRDT- 2014/05/14 06:00 PHST- 2013/12/12 00:00 [received] PHST- 2014/04/15 00:00 [accepted] PHST- 2014/03/20 00:00 [revised] PHST- 2014/05/14 06:00 [entrez] PHST- 2014/05/14 06:00 [pubmed] PHST- 2015/12/15 06:00 [medline] PHST- 2014/05/13 00:00 [pmc-release] AID - 2637 [pii] AID - 10.1007/s10067-014-2637-8 [doi] PST - ppublish SO - Clin Rheumatol. 2015 Mar;34(3):489-96. doi: 10.1007/s10067-014-2637-8. Epub 2014 May 13. PMID- 18854389 OWN - NLM STAT- MEDLINE DCOM- 20081118 LR - 20180126 IS - 1471-6771 (Electronic) IS - 0007-0912 (Linking) VI - 101 IP - 5 DP - 2008 Nov TI - Cerebral vasospasm and primary Raynaud's phenomenon. PG - 743-4 LID - 10.1093/bja/aen285 [doi] FAU - Roberts, H AU - Roberts H FAU - Tomlinson, S AU - Tomlinson S LA - eng PT - Case Reports PT - Letter PL - England TA - Br J Anaesth JT - British journal of anaesthesia JID - 0372541 SB - IM MH - Carotid Artery, Internal/diagnostic imaging MH - Female MH - Humans MH - Intracranial Aneurysm/*surgery MH - Intraoperative Complications/*diagnostic imaging MH - Middle Aged MH - Radiography MH - Raynaud Disease/*complications MH - Vasospasm, Intracranial/*etiology EDAT- 2008/10/16 09:00 MHDA- 2008/11/19 09:00 CRDT- 2008/10/16 09:00 PHST- 2008/10/16 09:00 [pubmed] PHST- 2008/11/19 09:00 [medline] PHST- 2008/10/16 09:00 [entrez] AID - S0007-0912(17)34150-8 [pii] AID - 10.1093/bja/aen285 [doi] PST - ppublish SO - Br J Anaesth. 2008 Nov;101(5):743-4. doi: 10.1093/bja/aen285. PMID- 28029677 OWN - NLM STAT- MEDLINE DCOM- 20181126 LR - 20260127 IS - 0028-2715 (Print) IS - 0028-2715 (Linking) VI - 55 IP - 204 DP - 2016 Oct-Dec TI - An Interesting Case of Basal Cell Carcinoma with Raynaud's Phenomenon Following Chronic Arsenic Exposure. PG - 100-102 AB - Arsenic is commonly known to be associated with squamous cell carcinoma. Among the lesser known associations is basal cell carcinoma and even rarer is its effect on blood vessels causing peripheral vascular disease. Here we present a case of a 55 yr old man with ulceroproliferative lesions on scalp and forehead along with several hyperpigmented patches on trunk and extremities. He had symptoms suggestive of Raynaud's phenomenon that eventually led to digital gangrene. FNAC was done which was suggestive of basal cell carcinoma. On further enquiry, he was found to reside in an arsenic endemic zone and was investigated for blood arsenic level which was elevated. Punch biopsy from different lesions from body confirmed nodular basal cell carcinoma. Presently the patient has stopped drinking water from the local tubewell. On follow-up he shows improvement of Raynaud's phenomenon and skin lesions. FAU - Gulshan, S AU - Gulshan S AD - Department of Pathology, NRS Medical College, Kolkata, India. FAU - Rahman, M J AU - Rahman MJ AD - Department of Urology, Regional Institute of Medical Sciences, Imphal, India. FAU - Sarkar, R AU - Sarkar R AD - Department of Pathology, NRS Medical College, Kolkata, India. FAU - Ghosh, S AU - Ghosh S AD - Department of Pathology, NRS Medical College, Kolkata, India. FAU - Hazra, R AU - Hazra R AD - Department of Pathology, NRS Medical College, Kolkata, India. LA - eng PT - Case Reports PT - Journal Article PL - Nepal TA - JNMA J Nepal Med Assoc JT - JNMA; journal of the Nepal Medical Association JID - 0045233 RN - 0 (Water Pollutants, Chemical) RN - N712M78A8G (Arsenic) SB - IM MH - Arsenic/blood/*toxicity MH - Biopsy MH - Basal Cell Carcinoma/*chemically induced/pathology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*chemically induced MH - Skin/drug effects/pathology MH - Skin Neoplasms/*chemically induced/pathology MH - Water Pollutants, Chemical/*toxicity MH - *Water Supply OTO - NOTNLM OT - basal cell carcinoma; chronic arsenic exposure; raynaud's phenomenon. EDAT- 2016/12/29 06:00 MHDA- 2018/11/27 06:00 CRDT- 2016/12/29 06:00 PHST- 2016/12/29 06:00 [entrez] PHST- 2016/12/29 06:00 [pubmed] PHST- 2018/11/27 06:00 [medline] PST - ppublish SO - JNMA J Nepal Med Assoc. 2016 Oct-Dec;55(204):100-102. PMID- 17160374 OWN - NLM STAT- MEDLINE DCOM- 20070223 LR - 20181113 IS - 0043-5341 (Print) IS - 0043-5341 (Linking) VI - 156 IP - 21-22 DP - 2006 Nov TI - [Psychometric investigation of Tyrolean patients with primary Raynaud's phenomenon]. PG - 574-82 AB - Primary Raynaud's phenomenon (PRP) is provoked by digital vasospasm induced by cold and emotional strain. By use of established psychometric test instruments (Freiburger Aggressionsfragebogen (FAF), Fragebogen zur Abschätzung psychosomatischen Krankheitserlebens (FAPK), Stressverarbeitungsfragebogen (SVF 120)) we examined the patients' ways of dealing with aggression and distress as well as their experience of psychosomatic illness. In all psychometric tests performed there were no statistically significant differences between the patients and the controls, indicating that patients with PRP depict no specific ways of dealing with aggression and distress, and do not experience psychosomatic illness. The subgroup of patients with affective disorders and the subgroup of patients with impaired psychological well-being, displayed a 'depressive style' of dealing with life events. Patients with major physical complaints of symptoms of Raynaud's phenomenon showed either an inability or a propensity to deny aggression. Patients with impaired psychological well-being tended to complain more often about severe physical symptoms associated with Raynaud's phenomenon. Psychometric testing in patients with primary Raynaud's phenomenon cannot be recommended without additional psychiatric diagnostic. In patients with primary Raynaud's phenomenon and psychiatric comorbidity psychometric testing can additionally provide useful clinical information. FAU - Weigel, Iris AU - Weigel I AD - Landesamt für Gesundheit und Soziales, Berlin, Germany. iweigel@gmx.de FAU - Klein-Weigel, Peter AU - Klein-Weigel P FAU - Kinzl, Johannes AU - Kinzl J FAU - Biebl, Wilfried AU - Biebl W FAU - Fraedrich, Gustav AU - Fraedrich G FAU - Heidrich, Heinz AU - Heidrich H LA - ger PT - Comparative Study PT - English Abstract PT - Journal Article TT - Psychometrische Tests bei primärem Raynaud-Phänomen--Untersuchungsergebnisse an einem Tiroler Patientenkollektiv. PL - Austria TA - Wien Med Wochenschr JT - Wiener medizinische Wochenschrift (1946) JID - 8708475 SB - IM MH - Adult MH - Aged MH - Aggression MH - Anxiety Disorders/epidemiology MH - Austria/epidemiology MH - Chi-Square Distribution MH - Comorbidity MH - Data Interpretation, Statistical MH - Female MH - Humans MH - Male MH - Mental Disorders/*diagnosis/epidemiology MH - Middle Aged MH - Mood Disorders/epidemiology MH - Psychometrics MH - Psychophysiologic Disorders/diagnosis/epidemiology/*psychology MH - Raynaud Disease/epidemiology/*psychology MH - Stress, Psychological EDAT- 2006/12/13 09:00 MHDA- 2007/02/24 09:00 CRDT- 2006/12/13 09:00 PHST- 2005/09/20 00:00 [received] PHST- 2006/04/07 00:00 [accepted] PHST- 2006/12/13 09:00 [pubmed] PHST- 2007/02/24 09:00 [medline] PHST- 2006/12/13 09:00 [entrez] AID - 10.1007/s10354-006-0338-x [doi] PST - ppublish SO - Wien Med Wochenschr. 2006 Nov;156(21-22):574-82. doi: 10.1007/s10354-006-0338-x. PMID- 19035499 OWN - NLM STAT- MEDLINE DCOM- 20090219 LR - 20081209 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 58 IP - 12 DP - 2008 Dec TI - Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. PG - 3902-12 LID - 10.1002/art.24038 [doi] AB - OBJECTIVE: To identify in patients with Raynaud's phenomenon (RP) independent markers that predict progression to definite systemic sclerosis (SSc) and to determine in patients with progression to SSc the type and sequence of microvascular damage and its relationship to SSc-specific autoantibodies. METHODS: Consecutive patients referred for evaluation of RP who had no definite connective tissue disease were evaluated for microvascular damage by nailfold capillary microscopy (NCM) and for anticentromere (anti-CENP-B), anti-Th/To, anti-topoisomerase I, and anti-RNA polymerase III (anti-RNAP III) autoantibodies by specific assays. Patients were studied prospectively. RESULTS: Of the 586 patients who were followed up for 3,197 person-years, 74 (12.6%) developed definite SSc. A characteristic sequence of microvascular damage was identified, starting with enlarged capillaries, followed by capillary loss, and then by capillary telangiectases. Definite SSc was diagnosed in close temporal relationship to capillary loss. Enlarged capillaries, capillary loss, and SSc-specific autoantibodies independently predicted definite SSc. Anti-CENP-B and anti-Th/To antibodies predicted enlarged capillaries; these autoantibodies and anti-RNAP III predicted capillary loss. Each autoantibody was associated with a distinct time course of microvascular damage. At followup, 79.5% of patients with 1 of these autoantibodies and abnormal findings on NCM at baseline had developed definite SSc. Patients with both baseline predictors were 60 times more likely to develop definite SSc. The data validated the proposed criteria for early SSc. CONCLUSION: In RP evolving to definite SSc, microvascular damage is dynamic and sequential, while SSc-specific autoantibodies are associated with the course and type of capillary abnormalities. Abnormal findings on NCM at baseline together with an SSc-specific autoantibody indicate a very high probability of developing definite SSc, whereas their absence rules out this outcome. FAU - Koenig, Martial AU - Koenig M AD - Notre-Dame Hospital, Centre Hospitalier de l'Université de Montréal, Montreal, Quebec, Canada. FAU - Joyal, France AU - Joyal F FAU - Fritzler, Marvin J AU - Fritzler MJ FAU - Roussin, André AU - Roussin A FAU - Abrahamowicz, Michal AU - Abrahamowicz M FAU - Boire, Gilles AU - Boire G FAU - Goulet, Jean-Richard AU - Goulet JR FAU - Rich, Eric AU - Rich E FAU - Grodzicky, Tamara AU - Grodzicky T FAU - Raymond, Yves AU - Raymond Y FAU - Senécal, Jean-Luc AU - Senécal JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Antibody Specificity MH - Autoantibodies/*blood MH - Decision Trees MH - Disease Progression MH - Early Diagnosis MH - Female MH - Follow-Up Studies MH - Humans MH - Incidence MH - Male MH - Microvessels/*immunology/pathology MH - Middle Aged MH - Predictive Value of Tests MH - Prospective Studies MH - Raynaud Disease/classification/epidemiology/*immunology/*pathology MH - Scleroderma, Systemic/classification/epidemiology/*immunology/*pathology MH - Seroepidemiologic Studies MH - Young Adult EDAT- 2008/11/28 09:00 MHDA- 2009/02/20 09:00 CRDT- 2008/11/28 09:00 PHST- 2008/11/28 09:00 [pubmed] PHST- 2009/02/20 09:00 [medline] PHST- 2008/11/28 09:00 [entrez] AID - 10.1002/art.24038 [doi] PST - ppublish SO - Arthritis Rheum. 2008 Dec;58(12):3902-12. doi: 10.1002/art.24038. PMID- 27045794 OWN - NLM STAT- MEDLINE DCOM- 20181107 LR - 20181107 IS - 1603-6824 (Electronic) IS - 0041-5782 (Linking) VI - 178 IP - 13 DP - 2016 Mar 28 TI - [Severe Raynaud's syndrome treated by lumbar sympathectomy]. PG - V08150658 LID - V08150658 [pii] AB - Avoiding exposure of extremities to cold combined with pharmacologic treatment usually suffice in the attempt to suppress the related symptoms of Raynaud's syndrome. This case report describes a severe case of Raynaud's syndrome affecting the lower extremities of a 16-year-old female. She was referred to a centre of vascular surgery with severe vasospasms of the feet. After failed attempts of pharmacologic treatment, a laparoscopic lumbar sympathectomy was performed with no complications and a slight reduction of symptoms three years post-surgically. FAU - Thomsen, Thomas Laden AU - Thomsen TL AD - t_thomsen@me.com. FAU - Roeder, Ole AU - Roeder O LA - dan PT - Case Reports PT - Journal Article TT - Svært Raynauds syndrom behandlet med lumbal sympatektomi. PL - Denmark TA - Ugeskr Laeger JT - Ugeskrift for laeger JID - 0141730 SB - IM MH - Adolescent MH - Female MH - Foot/pathology MH - Ganglia, Spinal/surgery MH - Humans MH - Laparoscopy/methods MH - Lumbosacral Plexus/surgery MH - Raynaud Disease/*surgery MH - *Sympathectomy EDAT- 2016/04/06 06:00 MHDA- 2018/11/08 06:00 CRDT- 2016/04/06 06:00 PHST- 2016/04/06 06:00 [entrez] PHST- 2016/04/06 06:00 [pubmed] PHST- 2018/11/08 06:00 [medline] AID - V08150658 [pii] PST - ppublish SO - Ugeskr Laeger. 2016 Mar 28;178(13):V08150658. PMID- 20376945 OWN - NLM STAT- MEDLINE DCOM- 20100420 LR - 20100318 IS - 1051-5313 (Print) IS - 1051-5313 (Linking) VI - 20 IP - 6 DP - 2010 Feb TI - Raynaud's: the big chill for fingers and toes. Blood vessel spasms can make the cold a painful experience. PG - 3 LA - eng PT - Journal Article PL - United States TA - Harv Heart Lett JT - Harvard heart letter : from Harvard Medical School JID - 9425723 MH - Fingers/*blood supply MH - Health Education/methods MH - *Health Knowledge, Attitudes, Practice MH - Humans MH - Patient Education as Topic MH - Raynaud Disease/complications/*diagnosis/*prevention & control MH - Regional Blood Flow MH - Skin Temperature MH - Toes/*blood supply EDAT- 2010/04/10 06:00 MHDA- 2010/04/21 06:00 CRDT- 2010/04/10 06:00 PHST- 2010/04/10 06:00 [entrez] PHST- 2010/04/10 06:00 [pubmed] PHST- 2010/04/21 06:00 [medline] PST - ppublish SO - Harv Heart Lett. 2010 Feb;20(6):3. PMID- 19229802 OWN - NLM STAT- MEDLINE DCOM- 20090320 LR - 20151119 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 38 IP - 1 DP - 2009 Feb TI - Circulating nerve growth factor in primary and secondary Raynaud's syndrome - results of a pilot study. PG - 39-45 LID - 10.1024/0301-1526.38.1.39 [doi] AB - BACKGROUND: In this pilot study we examined circulating concentrations of nerve growth factor (NGF) in controls and patients suffering from primary or secondary Raynaud's syndrome and determined their relation to digital vasospasm. PATIENTS AND METHODS: Eighteen controls, 16 patients with primary RP and 19 patients with systemic sclerosis (SScl) were included. Degree of vasospasm was graduated according to the degree of plethysmographically measured vasospastic reaction after cold test. A diary was handed out for documentation of the daily number and duration of RP during a period of 16 days. Circulating NGF levels were analysed by a commercial ELISA (Promega Inc., USA). RESULTS: SScl-patients were significantly older (p < 0.0001) and more severely affected by spontaneously occurring RP (p = 0.03), whereas the severity of the vasospastic reactions after a standard cold test were not significantly different between the groups (p = 0.09). Within each study group and between the study groups elevated NGF levels were observed only in SScl-patients after thermal provocations (p = < 0.05). In a correlation analysis restricted to patients with PRP or SRP, the degree of vasospasm after cold testing as well as the intensity of Raynaud's symptoms were not correlated with NGF-levels (p = n.s.). CONCLUSIONS: Our results do not support the hypothesis that NGF plays a major role in the generation of vasospasm in Raynaud's phenomenon. FAU - Klein-Weigel, P AU - Klein-Weigel P AD - DRK Kliniken, Klinik für Innere Medizin - Schwerpunkt Angiologie, Berlin, Germany. p.klein-weigel@web.de FAU - Gutsche-Petrak, B AU - Gutsche-Petrak B FAU - Humpel, C AU - Humpel C FAU - Riemekasten, G AU - Riemekasten G FAU - Ivanov, S AU - Ivanov S FAU - Heidrich, H AU - Heidrich H LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 RN - 9061-61-4 (Nerve Growth Factor) SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Cold Temperature MH - Enzyme-Linked Immunosorbent Assay MH - Fingers/*blood supply MH - Humans MH - Middle Aged MH - Nerve Growth Factor/*blood MH - Pilot Projects MH - Plethysmography MH - Raynaud Disease/*blood/etiology/physiopathology MH - Scleroderma, Systemic/blood/*complications/physiopathology MH - Severity of Illness Index MH - Time Factors MH - *Vasoconstriction MH - Young Adult EDAT- 2009/02/21 09:00 MHDA- 2009/03/21 09:00 CRDT- 2009/02/21 09:00 PHST- 2009/02/21 09:00 [entrez] PHST- 2009/02/21 09:00 [pubmed] PHST- 2009/03/21 09:00 [medline] AID - 10.1024/0301-1526.38.1.39 [doi] PST - ppublish SO - Vasa. 2009 Feb;38(1):39-45. doi: 10.1024/0301-1526.38.1.39. PMID- 22749761 OWN - NLM STAT- MEDLINE DCOM- 20121204 LR - 20260128 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 37 IP - 4 DP - 2012 Jul TI - [Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation Working Group of the French Vascular Medicine Society]. PG - 207-12 LID - 10.1016/j.jmv.2012.05.005 [doi] AB - About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up. CI - Copyright © 2012 Elsevier Masson SAS. All rights reserved. FAU - Pistorius, M-A AU - Pistorius MA AD - Service de médecine interne et vasculaire, Hôtel-Dieu, place Alexis-Ricordeau, BP 1005, 44093 Nantes cedex 01, France. marc.pistorius@chu-nantes.fr FAU - Carpentier, P-H AU - Carpentier PH CN - le groupe de travail « Microcirculation » de la Société française de médecine vasculaire LA - fre PT - Consensus Statement PT - English Abstract PT - Journal Article PT - Practice Guideline TT - Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts. DEP - 20120629 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/blood MH - Disease Susceptibility MH - France/epidemiology MH - Humans MH - Microcirculation MH - Microscopic Angioscopy MH - Nails/blood supply MH - Physical Examination MH - Raynaud Disease/*diagnosis/epidemiology/etiology/microbiology/physiopathology MH - Scleroderma, Systemic/complications/diagnosis/epidemiology MH - Symptom Assessment EDAT- 2012/07/04 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/07/04 06:00 PHST- 2012/05/03 00:00 [received] PHST- 2012/05/09 00:00 [accepted] PHST- 2012/07/04 06:00 [entrez] PHST- 2012/07/04 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - S0398-0499(12)00102-3 [pii] AID - 10.1016/j.jmv.2012.05.005 [doi] PST - ppublish SO - J Mal Vasc. 2012 Jul;37(4):207-12. doi: 10.1016/j.jmv.2012.05.005. Epub 2012 Jun 29. PMID- 22441059 OWN - NLM STAT- MEDLINE DCOM- 20130308 LR - 20220317 IS - 2042-8189 (Electronic) IS - 1478-2715 (Linking) VI - 42 IP - 1 DP - 2012 Mar TI - Digital gangrene in a patient with primary Raynaud's phenomenon. PG - 24-6 LID - 10.4997/JRCPE.2012.106 [doi] AB - Digital gangrene is not usually associated with primary Raynaud's phenomenon (RP). Its presence should therefore alert the healthcare provider to look for an alternative explanation. A 19-year-old female patient with primary RP developed digital gangrene following surgical management of acute paronychia. The possible mechanism in this patient appears to be the augmentation of the vasoconstrictive response due to the local infiltration of epinephrine mixed with lignocaine prior to the incision and drainage of her infected finger. FAU - Ravindran, V AU - Ravindran V AD - Department of Rheumatology, MES Medical College, Perinthalmanna, Kerala, India. drvinod12@gmail.com FAU - Rajendran, S AU - Rajendran S LA - eng PT - Case Reports PT - Journal Article PL - England TA - J R Coll Physicians Edinb JT - The journal of the Royal College of Physicians of Edinburgh JID - 101144324 RN - 0 (Vasoconstrictor Agents) RN - YKH834O4BH (Epinephrine) SB - IM MH - Adult MH - Connective Tissue Diseases/*diagnosis MH - Dermatologic Surgical Procedures MH - Epinephrine/*adverse effects MH - Female MH - Fingers/*pathology/surgery MH - Gangrene/*etiology MH - Humans MH - Paronychia/surgery MH - *Postoperative Complications MH - Raynaud Disease/complications/*diagnosis MH - Skin/pathology MH - Vasoconstriction MH - Vasoconstrictor Agents/*adverse effects MH - Young Adult EDAT- 2012/03/24 06:00 MHDA- 2013/03/09 06:00 CRDT- 2012/03/24 06:00 PHST- 2012/03/24 06:00 [entrez] PHST- 2012/03/24 06:00 [pubmed] PHST- 2013/03/09 06:00 [medline] AID - 10.4997/JRCPE.2012.106 [doi] PST - ppublish SO - J R Coll Physicians Edinb. 2012 Mar;42(1):24-6. doi: 10.4997/JRCPE.2012.106. PMID- 18634148 OWN - NLM STAT- MEDLINE DCOM- 20090114 LR - 20161124 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 8 DP - 2008 Aug TI - Color Doppler ultrasonography of hand and finger arteries to differentiate primary from secondary forms of Raynaud's phenomenon. PG - 1591-8 AB - OBJECTIVE: Modern ultrasound (US) equipment allows rheumatologists to directly visualize hand and finger arteries. How does US aid in diagnosis of Raynaud's phenomenon (RP)? METHODS: Color Doppler US of the proper and common palmar digital, radial, and ulnar arteries and the superficial palmar arch of both hands was performed in 135 consecutive patients who presented with suspected RP. RESULTS: US was pathologic in 63% of patients with secondary RP, in 6% with primary RP, and in none with pseudo-RP (p < 0.0001). We found 3 types of vascular pathology: Type 1 showed narrowing or chronic occlusion of some proper digital arteries; Type 2 was characterized by the same finding in all proper digital arteries; and Type 3 involved acute occlusions. Type 1 was found in 3 of 53 patients with primary RP and in 19 patients with secondary RP including 5 of 9 patients with anti-centromere positive systemic sclerosis (SSc); Type 2 occurred in 16 patients with SSc, MCTD, and dermatomyositis; and Type 3 was found in 8 patients with antiphospholipid antibody syndrome, thromboangiitis obliterans, vibration trauma, or vasculitis. The ulnar arteries were more commonly affected than the radial arteries. The 2nd radial, 3rd radial, 4th ulnar, and 5th ulnar proper palmar digital arteries were most commonly involved. CONCLUSION: Aiding in differentiating primary versus secondary RP, severe versus less severe disease, and acute versus chronic vascular occlusion, digital artery US depicts the same anatomical structures as angiography, but it is cheaper, faster, and noninvasive. FAU - Schmidt, Wolfgang A AU - Schmidt WA AD - Medical Center for Rheumatology Berlin-Buch, Berlin, Germany. w.schmidt@immanuel.de FAU - Krause, Andreas AU - Krause A FAU - Schicke, Bernd AU - Schicke B FAU - Wernicke, Dirk AU - Wernicke D LA - eng PT - Journal Article DEP - 20080715 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Connective Tissue Diseases/complications/*diagnostic imaging MH - Female MH - Fingers/blood supply/diagnostic imaging MH - Humans MH - Male MH - Middle Aged MH - Radial Artery/*diagnostic imaging MH - Raynaud Disease/complications/*diagnostic imaging/etiology MH - Trauma Severity Indices MH - Ulnar Artery/*diagnostic imaging MH - Ultrasonography EDAT- 2008/07/18 09:00 MHDA- 2009/01/15 09:00 CRDT- 2008/07/18 09:00 PHST- 2008/07/18 09:00 [pubmed] PHST- 2009/01/15 09:00 [medline] PHST- 2008/07/18 09:00 [entrez] AID - 08/13/0722 [pii] PST - ppublish SO - J Rheumatol. 2008 Aug;35(8):1591-8. Epub 2008 Jul 15. PMID- 26692039 OWN - NLM STAT- MEDLINE DCOM- 20161215 LR - 20161230 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 25 IP - 5 DP - 2016 Apr TI - Iloprost treatment in pediatric patients with complicated Raynaud's phenomenon. PG - 558-60 LID - 10.1177/0961203315622898 [doi] FAU - Cekic, S AU - Cekic S AD - Uludag University Faculty of Medicine Division of Pediatric Immunology and Rheumatology, Uludag University School of Medicine, Bursa, Turkey drsukrucekic@gmail.com. FAU - Kilic, S S AU - Kilic SS AD - Uludag University Faculty of Medicine Division of Pediatric Immunology and Rheumatology, Uludag University School of Medicine, Bursa, Turkey. LA - eng PT - Case Reports PT - Letter DEP - 20151221 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adolescent MH - Age of Onset MH - Antiphospholipid Syndrome/*complications/diagnosis MH - Child MH - Female MH - Foot/*blood supply MH - Foot Ulcer/etiology MH - Humans MH - Iloprost/*therapeutic use MH - Ischemia/diagnosis/*drug therapy/etiology/physiopathology MH - Raynaud Disease/diagnosis/*drug therapy/etiology/physiopathology MH - Regional Blood Flow MH - Treatment Outcome MH - Vasoconstriction/*drug effects MH - Vasodilator Agents/*therapeutic use MH - Wound Healing/drug effects EDAT- 2015/12/23 06:00 MHDA- 2016/12/16 06:00 CRDT- 2015/12/23 06:00 PHST- 2015/05/08 00:00 [received] PHST- 2015/11/25 00:00 [accepted] PHST- 2015/12/23 06:00 [entrez] PHST- 2015/12/23 06:00 [pubmed] PHST- 2016/12/16 06:00 [medline] AID - 0961203315622898 [pii] AID - 10.1177/0961203315622898 [doi] PST - ppublish SO - Lupus. 2016 Apr;25(5):558-60. doi: 10.1177/0961203315622898. Epub 2015 Dec 21. PMID- 22434167 OWN - NLM STAT- MEDLINE DCOM- 20120501 LR - 20120321 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 137 IP - 13 DP - 2012 Mar TI - [Recent advances in Raynaud's syndrome]. PG - 622-4 LID - 10.1055/s-0031-1298986 [doi] FAU - Klein-Weigel, P AU - Klein-Weigel P AD - Klinik für Innere Medizin - Schwerpunkt Angiologie und kardiovaskuläre Frührehabilitation, DRK Kliniken Berlin. p.klein-weigel@drk-kliniken-berlin.de LA - ger PT - Journal Article TT - Raynaud-Syndrom: Neue Erkenntnisse und Entwicklungen. DEP - 20120320 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Vasodilator Agents) SB - IM MH - Biofeedback, Psychology MH - Humans MH - Raynaud Disease/complications/*diagnosis/drug therapy/pathology/*therapy MH - Scleroderma, Systemic/complications/diagnosis MH - Vasodilator Agents/therapeutic use EDAT- 2012/03/22 06:00 MHDA- 2012/05/02 06:00 CRDT- 2012/03/22 06:00 PHST- 2012/03/22 06:00 [entrez] PHST- 2012/03/22 06:00 [pubmed] PHST- 2012/05/02 06:00 [medline] AID - 10.1055/s-0031-1298986 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2012 Mar;137(13):622-4. doi: 10.1055/s-0031-1298986. Epub 2012 Mar 20. PMID- 17881663 OWN - NLM STAT- MEDLINE DCOM- 20071029 LR - 20250214 IS - 0003-4967 (Print) IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 66 IP - 10 DP - 2007 Oct TI - Bosentan therapy for patients with severe Raynaud's phenomenon in systemic sclerosis. PG - 1398-9 FAU - Hettema, M E AU - Hettema ME FAU - Zhang, D AU - Zhang D FAU - Bootsma, H AU - Bootsma H FAU - Kallenberg, C G M AU - Kallenberg CG LA - eng PT - Letter PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*complications MH - Sulfonamides/*therapeutic use MH - Treatment Outcome PMC - PMC1994313 EDAT- 2007/09/21 09:00 MHDA- 2007/10/30 09:00 PMCR- 2010/10/01 CRDT- 2007/09/21 09:00 PHST- 2007/09/21 09:00 [pubmed] PHST- 2007/10/30 09:00 [medline] PHST- 2007/09/21 09:00 [entrez] PHST- 2010/10/01 00:00 [pmc-release] AID - S0003-4967(24)34666-7 [pii] AID - ar73684 [pii] AID - 10.1136/ard.2007.073684 [doi] PST - ppublish SO - Ann Rheum Dis. 2007 Oct;66(10):1398-9. doi: 10.1136/ard.2007.073684. PMID- 25458027 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 54 IP - 6 DP - 2014 Nov-Dec TI - [Oral N-acetylcysteine in the treatment of Raynaud's phenomenon secondary to systemic sclerosis: a randomized, double-blind, placebo-controlled clinical trial]. PG - 452-8 LID - S0482-5004(14)00151-X [pii] LID - 10.1016/j.rbr.2014.07.001 [doi] AB - OBJECTIVE: To evaluate the safety and efficacy of N-acetylcysteine (NAC) orally on digital microcirculation blood flow in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). METHODS: This was a randomized, double-blind, placebo-controlled trial in which 42 patients with SSc received oral NAC at a dose of 600mg tid (21 patients, mean age 45.6±9.5 years) or placebo (21 patients, mean age 45.0±12.7 years) for four weeks. The primary endpoint was the change in cutaneous microcirculation blood flow before and after cold stimulation measured by laser Doppler imaging (LDI) at weeks 0 and 4. The frequency and severity of RP and the number of digital ulcers were also measured at weeks 0 and 4. The adverse events were recorded in the fourth week. RESULTS: There was no significant change in digital blood flow assessed by LDI before or after cold stimulus after four weeks of NAC or placebo. Both groups showed significant improvement in the frequency and severity of RP attacks, with no difference between the two groups. At the end of the study, the placebo group had three digital ulcers, while the NAC group showed no ulcers. NAC was well tolerated and no patient discontinued the treatment. CONCLUSIONS: NAC orally at a dose of 1800mg/day showed no vasodilator effect on hands' microcirculation after four weeks of treatment in patients with RP secondary to SSc. CI - Copyright © 2014 Elsevier Editora Ltda. All rights reserved. FAU - Correa, Marcelo José Uchoa AU - Correa MJ AD - Disciplina de Reumatologia, Universidade Federal de São Paulo, São Paulo, SP, Brasil. FAU - Mariz, Henrique Ataíde AU - Mariz HA AD - Disciplina de Reumatologia, Universidade Federal de São Paulo, São Paulo, SP, Brasil. FAU - Andrade, Luís Eduardo Coelho AU - Andrade LE AD - Disciplina de Reumatologia, Universidade Federal de São Paulo, São Paulo, SP, Brasil. FAU - Kayser, Cristiane AU - Kayser C AD - Disciplina de Reumatologia, Universidade Federal de São Paulo, São Paulo, SP, Brasil. Electronic address: criskayser@terra.com.br. LA - por PT - Journal Article PT - Randomized Controlled Trial TT - N-acetilcisteína oral no tratamento do fenômeno de Raynaud secundário à esclerose sistêmica: ensaio clínico randomizado, placebo-controlado e duplo-cego. DEP - 20140827 PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0 (Free Radical Scavengers) RN - WYQ7N0BPYC (Acetylcysteine) SB - IM MH - Acetylcysteine/*administration & dosage MH - Administration, Oral MH - Double-Blind Method MH - Female MH - Free Radical Scavengers/*administration & dosage MH - Humans MH - Male MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Regional Blood Flow MH - Scleroderma, Systemic/complications OTO - NOTNLM OT - Esclerose sistêmica OT - Estresse oxidativo OT - Fenômeno de Raynaud OT - N‐acetilcisteína OT - N‐acetylcysteine OT - Oxidative stress OT - Raynaud's Phenomenon OT - Systemic sclerosis OT - Tratamento OT - Treatment EDAT- 2014/12/03 06:00 MHDA- 2016/12/15 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/03/14 00:00 [received] PHST- 2014/05/21 00:00 [revised] PHST- 2014/07/18 00:00 [accepted] PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - S0482-5004(14)00151-X [pii] AID - 10.1016/j.rbr.2014.07.001 [doi] PST - ppublish SO - Rev Bras Reumatol. 2014 Nov-Dec;54(6):452-8. doi: 10.1016/j.rbr.2014.07.001. Epub 2014 Aug 27. PMID- 19076576 OWN - NLM STAT- MEDLINE DCOM- 20090312 LR - 20171116 IS - 1460-9592 (Electronic) IS - 1155-5645 (Linking) VI - 18 IP - 12 DP - 2008 Dec TI - Raynaud's phenomenon in a child presenting as oxygen desaturation during transfusion with cold blood. PG - 1208-10 LID - 10.1111/j.1460-9592.2008.02782.x [doi] AB - We report a case of Raynaud's phenomenon (RP) triggered by transfusion of cold blood to a pediatric burn patient under general anesthesia. The child was febrile so a decision was made to not use a blood warmer. When the blood was rapidly administered the child suddenly developed 'desaturation'. The child was placed on 100% oxygen, adequate ventilation assured, and the color of his oral mucosa assessed as 'pink'. Placement of the oximeter on the opposite hand revealed 100% saturation. To our knowledge, this is the first case of apparent RP reported in a pediatric patient triggered by transfusion of cold blood. FAU - Zhang, Xiaopeng AU - Zhang X AD - Division of Pediatric Anesthesia, Department of Anesthesia and Critical Care, Harvard Medical School, The MassGeneral Hospital for Children, Boston, MA 02114, USA. FAU - Coté, Charles J AU - Coté CJ LA - eng PT - Case Reports PT - Journal Article PL - France TA - Paediatr Anaesth JT - Paediatric anaesthesia JID - 9206575 RN - S88TT14065 (Oxygen) SB - IM MH - Anesthesia, General MH - Burns/therapy MH - Cold Temperature MH - Electrocardiography MH - Fever/complications MH - Humans MH - Hypoxia/blood/*complications MH - Infant MH - Male MH - Oximetry MH - Oxygen/blood MH - Raynaud Disease/*etiology MH - *Transfusion Reaction EDAT- 2008/12/17 09:00 MHDA- 2009/03/13 09:00 CRDT- 2008/12/17 09:00 PHST- 2008/12/17 09:00 [entrez] PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/03/13 09:00 [medline] AID - PAN2782 [pii] AID - 10.1111/j.1460-9592.2008.02782.x [doi] PST - ppublish SO - Paediatr Anaesth. 2008 Dec;18(12):1208-10. doi: 10.1111/j.1460-9592.2008.02782.x. PMID- 16501880 OWN - NLM STAT- MEDLINE DCOM- 20060908 LR - 20181113 IS - 0172-0643 (Print) IS - 0172-0643 (Linking) VI - 27 IP - 2 DP - 2006 Mar-Apr TI - Carney complex presenting with Raynaud's phenomenon and erythematous macules of the extremities. PG - 297-300 AB - A 12-year-old male presented with a 6-week history of apparent digital vasculitis with color changes of the distal fingers and erythematous macules of the palms and soles. Physical examination revealed skin findings of Carney complex and an abnormal cardiac examination. Echocardiography demonstrated a large left atrial mass, which caused partial obstruction during diastole and moderate mitral valve insufficiency. Surgical excision and pathological examination of the mass confirmed the presence of a large, peduculated myxoma. This case illustrates the presentation of Carney complex with left atrial myxoma as apparent vasculitis and Raynaud's phenomenon. FAU - Mirkinson, L J AU - Mirkinson LJ AD - Department of Rheumatology, George Washington University Medical Center, Children's National Medical Center, Washington, DC 20010, USA. mirkil@holycross-health.org FAU - Ratnayaka, K AU - Ratnayaka K FAU - Sable, C A AU - Sable CA FAU - Gaskin, P R A AU - Gaskin PR LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pediatr Cardiol JT - Pediatric cardiology JID - 8003849 SB - IM MH - Child MH - Echocardiography MH - *Heart Atria MH - Heart Neoplasms/*complications/diagnostic imaging MH - Humans MH - Male MH - Myxoma/*complications/diagnostic imaging MH - Neoplastic Syndromes, Hereditary/*complications MH - Raynaud Disease/*etiology MH - Skin Diseases, Vascular/*etiology EDAT- 2006/02/28 09:00 MHDA- 2006/09/09 09:00 CRDT- 2006/02/28 09:00 PHST- 2006/02/28 09:00 [pubmed] PHST- 2006/09/09 09:00 [medline] PHST- 2006/02/28 09:00 [entrez] AID - 10.1007/s00246-005-1238-3 [doi] PST - ppublish SO - Pediatr Cardiol. 2006 Mar-Apr;27(2):297-300. doi: 10.1007/s00246-005-1238-3. PMID- 20558950 OWN - NLM STAT- MEDLINE DCOM- 20110308 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 49 IP - 12 DP - 2010 TI - Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome. PG - 1229-32 AB - Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice. FAU - Watanabe, Ryu AU - Watanabe R AD - Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan. FAU - Shirai, Tsuyoshi AU - Shirai T FAU - Tajima, Yumi AU - Tajima Y FAU - Ohguchi, Hiroto AU - Ohguchi H FAU - Onishi, Yasushi AU - Onishi Y FAU - Fujii, Hiroshi AU - Fujii H FAU - Takasawa, Naruhiko AU - Takasawa N FAU - Ishii, Tomonori AU - Ishii T FAU - Harigae, Hideo AU - Harigae H LA - eng PT - Case Reports PT - Journal Article DEP - 20100615 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Adult MH - Antibodies, Antinuclear/*biosynthesis/blood MH - Female MH - Humans MH - Infant, Newborn MH - Pregnancy MH - Pregnancy Complications, Hematologic/*blood/diagnosis MH - Purpura, Thrombotic Thrombocytopenic/*blood/complications/diagnosis MH - Raynaud Disease/*blood/complications/diagnosis EDAT- 2010/06/19 06:00 MHDA- 2011/03/09 06:00 CRDT- 2010/06/19 06:00 PHST- 2010/06/19 06:00 [entrez] PHST- 2010/06/19 06:00 [pubmed] PHST- 2011/03/09 06:00 [medline] AID - JST.JSTAGE/internalmedicine/49.3465 [pii] AID - 10.2169/internalmedicine.49.3465 [doi] PST - ppublish SO - Intern Med. 2010;49(12):1229-32. doi: 10.2169/internalmedicine.49.3465. Epub 2010 Jun 15. PMID- 16739187 OWN - NLM STAT- MEDLINE DCOM- 20060706 LR - 20250529 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 55 IP - 3 DP - 2006 Jun 15 TI - Influence of clinical features on the health status of patients with limited cutaneous systemic sclerosis. PG - 473-9 AB - OBJECTIVE: To determine the effect of limited cutaneous systemic sclerosis (lcSSc) on patients' health status, and to identify the contributions to health status of different manifestations of lcSSc. METHODS: The Short Form 36 questionnaire was completed by 213 patients with lcSSc or Raynaud's syndrome and an antinuclear antibody typical of lcSSc as part of the baseline visit of the Quinapril in Scleroderma trial. Results were analyzed after correcting for age and sex using the Welsh Health Survey. Patients' results were related to their clinical characteristics. RESULTS: The mean physical component score (PCS) was 44.0 (95% confidence interval [95% CI] 42.5, 45.5), which was lower than the population norm of 50, and the median mental component score (MCS) was 52.2 (95% CI 48.5, 54.3). Raynaud's disease visual analog scale (VAS) scores, lung function, the number of organ systems affected, and skin score were significantly correlated with PCS. The total score (TDS) of an SSc severity scale showed the highest correlation. The effect of lcSSc on PCS was worse in younger patients. Multiple regression including age demonstrated that Raynaud's disease severity could predict a reduction in PCS beyond that predicted by TDS. Raynaud's disease severity and duration of lcSSc were linked to low MCS. Arthritis reduced PCS and esophageal involvement reduced PCS and MCS. CONCLUSION: Physical health status of patients with lcSSc was reduced, with 30% of the variation predicted by TDS, age, and severity of Raynaud's disease VAS. Mental health status was not reduced in this population. FAU - Gliddon, Angela E AU - Gliddon AE AD - School of Sport, Health & Exercise Science, University of Wales, Bangor, Gwynedd, United Kingdom. a.e.gliddon@bangor.ac.uk FAU - Doré, Caroline J AU - Doré CJ FAU - Maddison, Peter J AU - Maddison PJ CN - Quins Trial Study Group LA - eng GR - MC_U122886349/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - *Health Status MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/complications/pathology/physiopathology MH - Scleroderma, Limited/*complications/*pathology/physiopathology MH - Severity of Illness Index MH - *Surveys and Questionnaires EDAT- 2006/06/02 09:00 MHDA- 2006/07/11 09:00 CRDT- 2006/06/02 09:00 PHST- 2006/06/02 09:00 [pubmed] PHST- 2006/07/11 09:00 [medline] PHST- 2006/06/02 09:00 [entrez] AID - 10.1002/art.21999 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Jun 15;55(3):473-9. doi: 10.1002/art.21999. PMID- 26986711 OWN - NLM STAT- MEDLINE DCOM- 20160429 LR - 20160502 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 45 IP - 1 DP - 2016 Jan TI - Paroxysmal finger haematoma--a benign acrosyndrome occurring in middle-aged women. PG - 57-62 LID - 10.1024/0301-1526/a000496 [doi] AB - BACKGROUND: Paroxysmal finger haematoma (PFH) is an under-recognised vascular acrosyndrome with no epidemiological description to date. The aim of this work was to evaluate the prevalence, risk factors and clinical correlates of PFH in a population-based sample of subjects and to describe their semiological characteristics. PATIENTS AND METHODS: This cross-sectional study of random samples of the general population in three geographic areas of France involved 802 subjects, 548 women and 254 men, aged 18 to 84 years. The diagnosis of PFH was made from a report by the subject of a history of recurrent haematoma in the fingers with a sudden, painful and unexpected occurrence. Diagnosis of associated conditions and evaluation of lifestyle variables were obtained through standardised medical interview and examination. RESULTS: A history of PFH was detected in 71 subjects, with a prevalence of 1.2% in men and 12.4% in women; there was no significant regional variation. Onset before 40 years of age was rare. Besides female sex and age, no socio-economical nor lifestyle risk factors were detected. PFH was associated with Raynaud phenomenon and a history of chilblains, but no link with any health threatening disease was found. In addition to the sudden onset of pain and hematoma, the main clinical features were a frequent digital swelling during the painful attack, and their predominant location on the volar side of the first and second phalanges of the third or second fingers of the dominant hand. CONCLUSIONS: PFH is a benign phenomenon, frequently found in middle-aged women, to be classified among the vascular acrosyndromes. Patients seeking medical evaluation for this disorder should be reassured. FAU - Carpentier, Patrick H AU - Carpentier PH AD - 1 Department of Vascular Medicine, Grenoble University Hospital, France. FAU - Maricq, Hildegard R AU - Maricq HR AD - 2 Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, USA. FAU - Biro, Christine AU - Biro C AD - 1 Department of Vascular Medicine, Grenoble University Hospital, France. FAU - Jiguet, Myriam AU - Jiguet M AD - 1 Department of Vascular Medicine, Grenoble University Hospital, France. FAU - Seinturier, Christophe AU - Seinturier C AD - 1 Department of Vascular Medicine, Grenoble University Hospital, France. LA - eng PT - Journal Article PT - Multicenter Study PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Cross-Sectional Studies MH - Female MH - Fingers/*blood supply MH - France/epidemiology MH - Hematoma/diagnosis/*epidemiology MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/diagnosis/*epidemiology MH - Recurrence MH - Risk Assessment MH - Risk Factors MH - Sex Distribution MH - Sex Factors MH - Young Adult OTO - NOTNLM OT - Haematoma OT - Raynaud’s disease OT - epidemiology OT - finger OT - prevalence OT - risk factors EDAT- 2016/03/18 06:00 MHDA- 2016/04/30 06:00 CRDT- 2016/03/18 06:00 PHST- 2016/03/18 06:00 [entrez] PHST- 2016/03/18 06:00 [pubmed] PHST- 2016/04/30 06:00 [medline] AID - 10.1024/0301-1526/a000496 [doi] PST - ppublish SO - Vasa. 2016 Jan;45(1):57-62. doi: 10.1024/0301-1526/a000496. PMID- 20145687 OWN - NLM STAT- MEDLINE DCOM- 20100308 LR - 20151119 IS - 1175-8716 (Electronic) IS - 0028-8446 (Linking) VI - 122 IP - 1306 DP - 2009 Nov 20 TI - Prevalence of Raynaud's phenomenon in the adult New Zealand population. PG - 55-62 AB - AIMS: To estimate the prevalence of Raynaud's phenomenon (RP) in the New Zealand adult population. METHODS: 350 adults 18 years and over, random selected from the electoral roll, were sent a postal survey based on the UK Scleroderma Study Group questionnaire. Participants were classified as having RP if they had biphasic colour changes. RESULTS: There was a 67% response rate. The prevalence of RP was estimated to be 18.8% (95% Confidence Interval (CI) 13.0%-27.1%) in females and 4.9% (95%CI 1.9%-13.0%) in males. The prevalence decreased with age. There was a higher prevalence in the warmer north of the country. People of Maori descent and in more manual occupations had more serve symptoms. Among those reporting symptoms 11% (95%CI 7%-17%) had consulted a doctor. CONCLUSION: New Zealand has high rates of RP. Few people with RP consult medical practitioners about their symptoms. FAU - Purdie, Gordon AU - Purdie G AD - Department of Public Health, University of Otago, Wellington, PO Box 7343, Wellington South, New Zealand. gordon.purdie@otago.ac.nz FAU - Harrison, Andrew AU - Harrison A FAU - Purdie, Dianne AU - Purdie D LA - eng PT - Journal Article DEP - 20091120 PL - New Zealand TA - N Z Med J JT - The New Zealand medical journal JID - 0401067 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Chi-Square Distribution MH - Confidence Intervals MH - Female MH - Health Surveys MH - Humans MH - Male MH - Middle Aged MH - New Zealand/epidemiology MH - Prevalence MH - Probability MH - Raynaud Disease/*diagnosis/*epidemiology MH - Rural Population MH - Severity of Illness Index MH - Sex Distribution MH - Surveys and Questionnaires MH - Urban Population MH - Young Adult EDAT- 2010/02/11 06:00 MHDA- 2010/03/10 06:00 CRDT- 2010/02/11 06:00 PHST- 2010/02/11 06:00 [entrez] PHST- 2010/02/11 06:00 [pubmed] PHST- 2010/03/10 06:00 [medline] PST - epublish SO - N Z Med J. 2009 Nov 20;122(1306):55-62. PMID- 17384174 OWN - NLM STAT- MEDLINE DCOM- 20070703 LR - 20070906 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 5 DP - 2007 May TI - An unusual association with Raynaud's phenomenon. PG - 894; author reply 894 FAU - Binymin, K A AU - Binymin KA LA - eng PT - Comment PT - Letter DEP - 20070323 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2007 Jan;46(1):92. doi: 10.1093/rheumatology/kel304. PMID: 17164258 MH - Humans MH - Hypopituitarism/*complications MH - Magnetic Resonance Imaging MH - Raynaud Disease/*etiology EDAT- 2007/03/27 09:00 MHDA- 2007/07/04 09:00 CRDT- 2007/03/27 09:00 PHST- 2007/03/27 09:00 [pubmed] PHST- 2007/07/04 09:00 [medline] PHST- 2007/03/27 09:00 [entrez] AID - kem035 [pii] AID - 10.1093/rheumatology/kem035 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 May;46(5):894; author reply 894. doi: 10.1093/rheumatology/kem035. Epub 2007 Mar 23. PMID- 27562035 OWN - NLM STAT- MEDLINE DCOM- 20170220 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 35 IP - 10 DP - 2016 Oct TI - Serum CXCL4 increase in primary Sjögren's syndrome characterizes patients with microvascular involvement and reduced salivary gland infiltration and lymph node involvement. PG - 2591-6 LID - 10.1007/s10067-016-3386-7 [doi] AB - CXCL4 is an antiangiogenic and immunomodulatory chemokine. We aimed to investigate serum levels of CXCL4 in primary Sjögren's syndrome (pSS), looking for associations with disease features. Thirty-nine consecutive pSS patients underwent clinical-serological assessment and nailfold videocapillaroscopy (NVC). Thirty-six patients and 30 controls affected by osteoarthritis were also investigated for serum levels of CXCL4 and soluble E-selectin (sE-selectin). CXCL4 was higher in pSS patients than in controls (1.79 [0.2-11.18] vs 1.023 ng/ml [0.02-14.45], p < 0.05), particularly in those without anti-La/SSB antibodies (2.89 [1.01-11.18] vs 1.69 ng/ml [0.2-2.72], p < 0.05), while it was lower in pSS patients with a focus score ≥1 at lip biopsy (1.44 [0.86-2.1] vs 2.24 ng/ml [1.64-3.25], p < 0.05) and clinically evident lymphadenopathy (1.53 [0.38-1.7] vs 2.08 ng/ml [1.45-3.03], p < 0.05). CXCL4 correlated with disease duration (r = 0.35, p < 0.05) and sE-selectin (r = 0.45, p < 0.01). Patients with Raynaud's phenomenon (RP) had more frequently abnormal CXCL4 levels than patients without RP (11/15 vs 3/21, p < 0.001), enlarged capillaries (14/16 vs 7/23, p < 0.001) and capillary loss at NVC (14/16 vs 6/23, p < 0.001). The hitherto unknown association of increased serum CXCL4 with features of microvascular impairment in pSS, along with the negative association with features of lymphocytic response (i.e., the absence of subset disease-specific autoantibodies, a low focus score, and the absence of lymphadenopathy) suggest clarifying the possible implication of this chemokine in pSS pathogenesis in larger studies. FAU - Vettori, Serena AU - Vettori S AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. serena.vettori@unina2.it. FAU - Irace, Rosaria AU - Irace R AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. FAU - Riccardi, Antonella AU - Riccardi A AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. FAU - Iacono, Daniela AU - Iacono D AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. FAU - Pellecchia, Luciana AU - Pellecchia L AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. FAU - Vicedomini, Lucia AU - Vicedomini L AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. FAU - Valentini, Gabriele AU - Valentini G AD - Rheumatology Unit, Department of Internal and Experimental Medicine, Second University of Naples, II Policlinico, via Pansini 5, 80131, Naples, Italy. LA - eng PT - Journal Article DEP - 20160826 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 37270-94-3 (Platelet Factor 4) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Capillaries/*pathology MH - Female MH - Humans MH - Lymph Nodes/*pathology MH - Male MH - Middle Aged MH - Platelet Factor 4/*blood MH - Raynaud Disease/*blood/etiology/pathology MH - Salivary Glands/*pathology MH - Sjogren's Syndrome/*blood/complications/pathology OTO - NOTNLM OT - CXCL4 OT - Raynaud’s phenomenon OT - Sjögren’s syndrome OT - sE-selectin EDAT- 2016/08/27 06:00 MHDA- 2017/10/14 06:00 CRDT- 2016/08/27 06:00 PHST- 2016/07/01 00:00 [received] PHST- 2016/08/14 00:00 [accepted] PHST- 2016/08/09 00:00 [revised] PHST- 2016/08/27 06:00 [entrez] PHST- 2016/08/27 06:00 [pubmed] PHST- 2017/10/14 06:00 [medline] AID - 10.1007/s10067-016-3386-7 [pii] AID - 10.1007/s10067-016-3386-7 [doi] PST - ppublish SO - Clin Rheumatol. 2016 Oct;35(10):2591-6. doi: 10.1007/s10067-016-3386-7. Epub 2016 Aug 26. PMID- 39947726 OWN - NLM STAT- MEDLINE DCOM- 20250213 LR - 20250529 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 18 IP - 2 DP - 2025 Feb 13 TI - Paraneoplastic scleroderma: a patient with a history of breast cancer presenting with sclerodactyly and Raynaud's phenomenon. LID - 10.1136/bcr-2024-261681 [doi] LID - e261681 AB - A woman in her 70s with a past history of breast cancer in remission presented with several months of dyspnoea, skin tightness and Raynaud's phenomenon. Physical examination was significant for hypertension, hypoxaemia, sclerodactyly, diffuse crackles on auscultation and mild respiratory distress. Work-up revealed elevated serum creatinine, elevated right ventricular systolic pressure on echocardiography and nuclear bone scan findings of osseous metastatic disease. Captopril was initiated with concern for scleroderma renal crisis. The scleroderma panel was positive for RNA polymerase 11 and 155. Vertebral biopsy was diagnostic of recurrent breast cancer. Through multidisciplinary discussion, rituximab was initiated, and malignancy treatment was deferred; however, the patient developed several complications and ultimately elected to pursue inpatient hospice. In the setting of recurrent breast cancer, this case is suggestive of paraneoplastic scleroderma. The presence of underlying malignancy is an important consideration in new cases of scleroderma, and multidisciplinary management is key to determining treatment preference. CI - © BMJ Publishing Group Limited 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Phillips, Emma Catherine AU - Phillips EC AUID- ORCID: 0000-0002-5427-2074 AD - Internal Medicine, Ohio State University Foundation, Columbus, Ohio, USA emmacatephillips@gmail.com. FAU - Clark, Meagan J AU - Clark MJ AD - Ohio State University Foundation, Columbus, Ohio, USA. FAU - Schirtzinger, Matthew AU - Schirtzinger M AD - Ohio State University Foundation, Columbus, Ohio, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250213 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Humans MH - Female MH - *Breast Neoplasms/complications/pathology MH - *Raynaud Disease/etiology/diagnosis MH - *Paraneoplastic Syndromes/diagnosis/etiology/complications MH - Aged MH - *Scleroderma, Systemic/diagnosis MH - Diagnosis, Differential PMC - PMC11831058 OTO - NOTNLM OT - Breast cancer OT - Rheumatology COIS- Competing interests: None declared. EDAT- 2025/02/14 00:24 MHDA- 2025/02/14 00:25 PMCR- 2025/02/13 CRDT- 2025/02/13 20:53 PHST- 2025/02/14 00:25 [medline] PHST- 2025/02/14 00:24 [pubmed] PHST- 2025/02/13 20:53 [entrez] PHST- 2025/02/13 00:00 [pmc-release] AID - 18/2/e261681 [pii] AID - bcr-2024-261681 [pii] AID - 10.1136/bcr-2024-261681 [doi] PST - epublish SO - BMJ Case Rep. 2025 Feb 13;18(2):e261681. doi: 10.1136/bcr-2024-261681. PMID- 33666068 OWN - NLM STAT- MEDLINE DCOM- 20210310 LR - 20210310 IS - 2532-5264 (Electronic) IS - 1122-0643 (Linking) VI - 91 IP - 2 DP - 2021 Mar 5 TI - A strange case of severe but fleeting hypoxemia in patient with COVID-19 infection: maybe virus-induced pulmonary Raynaud's phenomenon? LID - 10.4081/monaldi.2021.1706 [doi] AB - This case-report describes severe acute respiratory failure in a patient with a COVID-19 positive nasopharyngeal swab that spontaneously resolved within a few hours. It is speculated that the virus may have caused a fleeting pulmonary vasospasm. FAU - Barbarito, Nicola AU - Barbarito N AD - Don Gnocchi Foundation, Palazzolo Institute, Pneumology, Milan. nicola.barbarito@yahoo.it. LA - eng PT - Case Reports PT - Journal Article DEP - 20210305 PL - Italy TA - Monaldi Arch Chest Dis JT - Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace JID - 9307314 RN - 0 (Fibrin Fibrinogen Degradation Products) RN - 0 (fibrin fragment D) SB - IM MH - Aged, 80 and over MH - COVID-19/blood/*complications/*diagnosis MH - Female MH - Fibrin Fibrinogen Degradation Products/metabolism MH - Humans MH - Hypoxia/*virology MH - Raynaud Disease/diagnosis/*virology EDAT- 2021/03/06 06:00 MHDA- 2021/03/11 06:00 CRDT- 2021/03/05 06:22 PHST- 2020/11/28 00:00 [received] PHST- 2021/01/08 00:00 [accepted] PHST- 2021/03/05 06:22 [entrez] PHST- 2021/03/06 06:00 [pubmed] PHST- 2021/03/11 06:00 [medline] AID - 10.4081/monaldi.2021.1706 [doi] PST - epublish SO - Monaldi Arch Chest Dis. 2021 Mar 5;91(2). doi: 10.4081/monaldi.2021.1706. PMID- 19402237 OWN - NLM STAT- MEDLINE DCOM- 20090507 LR - 20090406 IS - 1070-910X (Print) IS - 1070-910X (Linking) VI - 16 IP - 7 DP - 2009 Mar TI - Cold fingers, cold toes? Could be Raynaud's. PG - 4-5 LA - eng PT - Journal Article PL - United States TA - Harv Womens Health Watch JT - Harvard women's health watch JID - 9423147 MH - Activities of Daily Living MH - Fingers/*blood supply MH - Health Promotion/*methods MH - Humans MH - Patient Education as Topic MH - Raynaud Disease/complications/*diagnosis/*prevention & control MH - Regional Blood Flow MH - Skin Temperature MH - Toes/*blood supply EDAT- 2009/05/01 09:00 MHDA- 2009/05/08 09:00 CRDT- 2009/05/01 09:00 PHST- 2009/05/01 09:00 [entrez] PHST- 2009/05/01 09:00 [pubmed] PHST- 2009/05/08 09:00 [medline] PST - ppublish SO - Harv Womens Health Watch. 2009 Mar;16(7):4-5. PMID- 25467391 OWN - NLM STAT- MEDLINE DCOM- 20151204 LR - 20150211 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 24 IP - 3 DP - 2015 Mar TI - Critical peripheral ischemia precipitated by severe episode of Raynaud's phenomenon in a patient with aPL-positive systemic lupus erythematosus, upon high titer anti-RNP seroconversion. PG - 327-30 LID - 10.1177/0961203314560427 [doi] AB - A 35-year-old female with long standing aPL-positive lupus without history of thromboembolic events, who has developed critical peripheral ischemia (CPI) is described. An episode of severe Raynaud's phenomenon rapidly progressed to an extensive digit-threatening ischemia, involving bilateral hands and feet. She was successfully treated with corticosteroids, anticoagulation, iloprost, sildenafil, and nifedipine. Her serological studies were remarkable for the emergence of high titer anti-RNP seroconversion and an increase in aPL titer, suggesting that these autoantibodies played a role in the pathogenesis of CPI. It is important to note that such observation should herald this potentially devastating complication of systemic lupus erythematosus. CI - © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Levy, O AU - Levy O AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine oferl@asaf.health.gov.il. FAU - Maslakov, I AU - Maslakov I AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine. FAU - Vosco, S AU - Vosco S AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine. FAU - Markov, A AU - Markov A AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine. FAU - Amit-Vazina, M AU - Amit-Vazina M AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine. FAU - Tishler, M AU - Tishler M AD - Assaf Harofeh Medical Center, Internal Medicine B, Rheumatology Unit; Tel Aviv University, Sackler School of Medicine. LA - eng PT - Case Reports PT - Journal Article DEP - 20141202 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adult MH - Extremities/*blood supply MH - Female MH - Humans MH - Ischemia/*immunology MH - Lupus Erythematosus, Systemic/*complications/immunology MH - Raynaud Disease/*complications/immunology OTO - NOTNLM OT - Anti-RNP antibodies OT - Raynaud's phenomenon OT - anticardiolipin antibodies OT - critical peripheral ischemia EDAT- 2014/12/04 06:00 MHDA- 2015/12/15 06:00 CRDT- 2014/12/04 06:00 PHST- 2014/12/04 06:00 [entrez] PHST- 2014/12/04 06:00 [pubmed] PHST- 2015/12/15 06:00 [medline] AID - 0961203314560427 [pii] AID - 10.1177/0961203314560427 [doi] PST - ppublish SO - Lupus. 2015 Mar;24(3):327-30. doi: 10.1177/0961203314560427. Epub 2014 Dec 2. PMID- 25200107 OWN - NLM STAT- MEDLINE DCOM- 20150812 LR - 20211021 IS - 1742-481X (Electronic) IS - 1742-4801 (Print) IS - 1742-4801 (Linking) VI - 11 IP - 6 DP - 2014 Dec TI - Partial fingertip necrosis following a digital surgical procedure in a patient with primary Raynaud's phenomenon. PG - 581-2 LID - 10.1111/iwj.12339 [doi] AB - Raynaud's phenomenon is a common clinical disorder consisting of recurrent, long-lasting and episodic vasospasm of the fingers and toes often associated with exposure to cold. In this article, we present a case of partial fingertip necrosis following digital surgical procedure in a patient with primary Raynaud's phenomenon. CI - © 2014 The Authors. International Wound Journal © 2014 Medicalhelplines.com Inc and John Wiley & Sons Ltd. FAU - Uygur, Safak AU - Uygur S AD - Nevsehir Government Hospital, Department of Plastic Surgery, Nevsehir, Turkey. FAU - Tuncer, Serhan AU - Tuncer S LA - eng PT - Case Reports PT - Journal Article DEP - 20140909 PL - England TA - Int Wound J JT - International wound journal JID - 101230907 SB - IM MH - Female MH - Fingers/*pathology/surgery MH - Humans MH - Necrosis/etiology/therapy MH - Postoperative Complications/*pathology MH - Raynaud Disease/*complications MH - Young Adult PMC - PMC7950774 OTO - NOTNLM OT - Digital necrosis OT - Digital surgery OT - Raynaud's disease OT - Raynaud's phenomenon EDAT- 2014/09/10 06:00 MHDA- 2015/08/13 06:00 PMCR- 2014/09/09 CRDT- 2014/09/10 06:00 PHST- 2014/07/03 00:00 [received] PHST- 2014/07/06 00:00 [accepted] PHST- 2014/09/10 06:00 [entrez] PHST- 2014/09/10 06:00 [pubmed] PHST- 2015/08/13 06:00 [medline] PHST- 2014/09/09 00:00 [pmc-release] AID - IWJ12339 [pii] AID - 10.1111/iwj.12339 [doi] PST - ppublish SO - Int Wound J. 2014 Dec;11(6):581-2. doi: 10.1111/iwj.12339. Epub 2014 Sep 9. PMID- 18559372 OWN - NLM STAT- MEDLINE DCOM- 20081204 LR - 20080717 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 8 DP - 2008 Aug TI - Comment on: Sparing of the thumb in Raynaud's phenomenon. PG - 1260; author reply 1260 LID - 10.1093/rheumatology/ken228 [doi] FAU - Binymin, K A AU - Binymin KA LA - eng PT - Comment PT - Letter DEP - 20080617 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2008 Feb;47(2):219-21. doi: 10.1093/rheumatology/kem353. PMID: 18208825 MH - Humans MH - Raynaud Disease/*physiopathology MH - Sensitivity and Specificity MH - Temperature MH - Thermography MH - Thumb/*physiopathology EDAT- 2008/06/19 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/06/19 09:00 PHST- 2008/06/19 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/06/19 09:00 [entrez] AID - ken228 [pii] AID - 10.1093/rheumatology/ken228 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Aug;47(8):1260; author reply 1260. doi: 10.1093/rheumatology/ken228. Epub 2008 Jun 17. PMID- 19996264 OWN - NLM STAT- MEDLINE DCOM- 20100219 LR - 20151119 IS - 1545-7222 (Electronic) IS - 0895-0172 (Linking) VI - 21 IP - 4 DP - 2009 Fall TI - Duloxetine in treating medically unexplained pain and Raynaud's phenomenon. PG - 475-6 FAU - Li, Cheng-Ta AU - Li CT FAU - Chou, Yuan-Hwa AU - Chou YH FAU - Su, Tung-Ping AU - Su TP LA - eng PT - Case Reports PT - Letter PL - United States TA - J Neuropsychiatry Clin Neurosci JT - The Journal of neuropsychiatry and clinical neurosciences JID - 8911344 RN - 0 (Antidepressive Agents) RN - 0 (Thiophenes) RN - 9044SC542W (Duloxetine Hydrochloride) SB - IM MH - Antidepressive Agents/therapeutic use MH - Duloxetine Hydrochloride MH - Female MH - Humans MH - Middle Aged MH - *Off-Label Use MH - Pain/*drug therapy MH - Raynaud Disease/*drug therapy MH - Thiophenes/*therapeutic use MH - Treatment Outcome EDAT- 2009/12/10 06:00 MHDA- 2010/02/20 06:00 CRDT- 2009/12/10 06:00 PHST- 2009/12/10 06:00 [entrez] PHST- 2009/12/10 06:00 [pubmed] PHST- 2010/02/20 06:00 [medline] AID - 21/4/475 [pii] AID - 10.1176/jnp.2009.21.4.475 [doi] PST - ppublish SO - J Neuropsychiatry Clin Neurosci. 2009 Fall;21(4):475-6. doi: 10.1176/jnp.2009.21.4.475. PMID- 17559484 OWN - NLM STAT- MEDLINE DCOM- 20070717 LR - 20260128 IS - 1533-2500 (Electronic) IS - 1530-7085 (Linking) VI - 7 IP - 2 DP - 2007 Jun TI - Treatment of refractory ischemic pain from chemotherapy-induced Raynaud's syndrome with spinal cord stimulation. PG - 143-6 AB - We report the successful treatment of refractory ischemic pain from cisplatin-induced Raynaud's syndrome with spinal cord stimulation after failed pharmacologic management and surgical sympathectomy. CASE REPORT: A 48-year-old man developed ischemic pain of the hands while undergoing cisplatin and gemcitabine chemotherapy for metastatic pancreatic carcinoma. After extensive pharmacologic management and surgical sympathectomy failed to provide adequate analgesia, the patient underwent a percutaneous spinal cord stimulation trial followed by permanent implantation and received significant pain relief prior to succumbing to his illness. Spinal cord stimulation provided effective therapy for refractory ischemic pain, even after failed sympathectomy. FAU - Ting, Joseph C AU - Ting JC AD - University of Texas M.D. Anderson Cancer Center, Department of Anesthesiology and Pain Medicine, Houston, Texas 77030, USA. FAU - Fukshansky, Mikhail AU - Fukshansky M FAU - Burton, Allen W AU - Burton AW LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pain Pract JT - Pain practice : the official journal of World Institute of Pain JID - 101130835 SB - IM MH - Drug-Related Side Effects and Adverse Reactions MH - Humans MH - Ischemia/etiology MH - Male MH - Middle Aged MH - Pain Measurement/methods MH - Intractable Pain/etiology/pathology/*therapy MH - Raynaud Disease/chemically induced/*complications MH - Spinal Cord/physiopathology/*radiation effects MH - Transcutaneous Electric Nerve Stimulation/*methods EDAT- 2007/06/15 09:00 MHDA- 2007/07/18 09:00 CRDT- 2007/06/15 09:00 PHST- 2007/06/15 09:00 [pubmed] PHST- 2007/07/18 09:00 [medline] PHST- 2007/06/15 09:00 [entrez] AID - PPR122 [pii] AID - 10.1111/j.1533-2500.2007.00122.x [doi] PST - ppublish SO - Pain Pract. 2007 Jun;7(2):143-6. doi: 10.1111/j.1533-2500.2007.00122.x. PMID- 35228457 OWN - NLM STAT- MEDLINE DCOM- 20220412 LR - 20220716 IS - 2185-4610 (Electronic) IS - 0016-2590 (Print) IS - 0016-2590 (Linking) VI - 68 IP - 1 DP - 2022 Apr 8 TI - Spinal cord stimulation for the treatment of pain and toe ulceration associated with systemic sclerosis: a case report. PG - 37-41 LID - 10.5387/fms.2021-33 [doi] AB - Systemic sclerosis is a complex disease characterized by extensive fibrosis, microvascular alterations, and additional sequelae. Microvascular alterations can cause painful ulcers and necrosis; however, conservative or surgical treatment is often challenging in terms of healing. The study aimed to describe a toe ulcer with systemic sclerosis and its' successful treatment with spinal cord stimulation. An 83-year-old woman, who was diagnosed with systemic sclerosis over the past decade, was distressed by a non-healing toe ulcer for an extended period of time. The patient underwent spinal cord stimulation treatment with the expectation of pain relief and an improvement in microcirculatory insufficiency. Her pain scales and microcirculation improved, and the toe ulcer healed. Furthermore, the frequency of Raynaud's symptoms was reduced, and the patient's pain decreased. There was no recurrence of the ulcer and she no longer needed a cane for walking. FAU - Ito, Hiroyuki AU - Ito H AD - Department of Plastic and Reconstructive Surgery, Komaki City Hospital. FAU - Tanei, Takafumi AU - Tanei T AD - Department of Neurosurgery, Komaki City Hospital. FAU - Sugawara, Kyoko AU - Sugawara K AD - Department of Dermatology, Komaki City Hospital. FAU - Sando, Yu AU - Sando Y AD - Department of Dermatology, Komaki City Hospital. FAU - Hori, Naohiro AU - Hori N AD - Department of Plastic and Reconstructive Surgery, Komaki City Hospital. LA - eng PT - Case Reports PT - Journal Article DEP - 20220225 PL - Japan TA - Fukushima J Med Sci JT - Fukushima journal of medical science JID - 0374626 SB - IM MH - Aged, 80 and over MH - Female MH - Humans MH - Microcirculation MH - Pain/complications MH - *Raynaud Disease/complications/therapy MH - *Scleroderma, Systemic/complications/therapy MH - *Spinal Cord Stimulation MH - Toes MH - Ulcer/complications PMC - PMC9071355 OTO - NOTNLM OT - Raynaud’s phenomenon OT - pain control OT - spinal cord stimulation OT - systemic sclerosis OT - ulcer COIS- All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (e.g., honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements) or non-financial interest (e.g., personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript. EDAT- 2022/03/02 06:00 MHDA- 2022/04/13 06:00 PMCR- 2022/01/01 CRDT- 2022/03/01 05:55 PHST- 2022/03/02 06:00 [pubmed] PHST- 2022/04/13 06:00 [medline] PHST- 2022/03/01 05:55 [entrez] PHST- 2022/01/01 00:00 [pmc-release] AID - 10.5387/fms.2021-33 [doi] PST - ppublish SO - Fukushima J Med Sci. 2022 Apr 8;68(1):37-41. doi: 10.5387/fms.2021-33. Epub 2022 Feb 25. PMID- 24532678 OWN - NLM STAT- MEDLINE DCOM- 20140717 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 73 IP - 6 DP - 2014 Jun TI - Fluorescence optical imaging as a novel technique for the visualisation of inflammation in patients with systemic sclerosis with Raynaud's phenomenon: a pilot study. PG - 1279-80 LID - 10.1136/annrheumdis-2013-204958 [doi] FAU - Pfeil, Alexander AU - Pfeil A AD - Department of Internal Medicine III, Jena University Hospital-Friedrich Schiller University Jena, , Jena, Germany. FAU - Drummer, Karl F AU - Drummer KF FAU - Oelzner, Peter AU - Oelzner P FAU - Böttcher, Joachim AU - Böttcher J FAU - Jung, Christian AU - Jung C FAU - Wolf, Gunter AU - Wolf G LA - eng PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20140214 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Coloring Agents) RN - IX6J1063HV (Indocyanine Green) SB - IM MH - Adult MH - Aged MH - Cohort Studies MH - Coloring Agents MH - Female MH - Hand MH - Humans MH - Indocyanine Green MH - Inflammation/complications/*diagnosis/immunology MH - Male MH - Middle Aged MH - Optical Imaging/*methods MH - Pilot Projects MH - Prospective Studies MH - Raynaud Disease/*diagnosis/etiology/immunology MH - Scleroderma, Limited/complications/*diagnosis/immunology MH - Young Adult OTO - NOTNLM OT - Disease Activity OT - Inflammation OT - Systemic Sclerosis EDAT- 2014/02/18 06:00 MHDA- 2014/07/18 06:00 CRDT- 2014/02/18 06:00 PHST- 2014/02/18 06:00 [entrez] PHST- 2014/02/18 06:00 [pubmed] PHST- 2014/07/18 06:00 [medline] AID - S0003-4967(24)14947-3 [pii] AID - 10.1136/annrheumdis-2013-204958 [doi] PST - ppublish SO - Ann Rheum Dis. 2014 Jun;73(6):1279-80. doi: 10.1136/annrheumdis-2013-204958. Epub 2014 Feb 14. PMID- 39655459 OWN - NLM STAT- MEDLINE DCOM- 20241210 LR - 20250331 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 27 IP - 12 DP - 2024 Dec TI - Alterations on Nailfold Videocapillaroscopy in Myelodysplastic Syndrome and Onychomycosis in a Female Smoker: Microvascular Dysfunction Without Connective Tissue Disease. PG - e70000 LID - 10.1111/1756-185X.70000 [doi] AB - A 58-year-old female smoker diagnosed with myelodysplastic syndrome (MDS) presented with Raynaud's phenomenon and a "scleroderma-like" pattern on nailfold capillaroscopy. The capillaroscopic abnormalities were observed across all fingers, including those without clinical manifestations of onychomycosis. Over a two-year follow-up, there was no evidence of clinical or serological progression toward a connective tissue disease, particularly systemic sclerosis. This case underscores the potential contributory role of MDS and smoking in the development of microvascular dysfunction, presenting as capillaroscopic abnormalities in the absence of an underlying autoimmune disorder. CI - © 2024 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Nigro, Angelo AU - Nigro A AUID- ORCID: 0009-0001-0802-6825 AD - Department of Rheumatology of Lucania-UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. LA - eng PT - Case Reports PT - Letter PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM EIN - Int J Rheum Dis. 2025 Feb;28(2):e70122. doi: 10.1111/1756-185X.70122. PMID: 39904747 MH - Humans MH - Female MH - *Microscopic Angioscopy MH - Middle Aged MH - *Myelodysplastic Syndromes/complications/diagnosis/physiopathology MH - *Raynaud Disease/physiopathology/diagnosis/etiology MH - *Onychomycosis/diagnosis/complications MH - *Nails/blood supply MH - Smokers MH - Predictive Value of Tests MH - Microcirculation MH - Smoking/adverse effects OTO - NOTNLM OT - Capillaroscopy OT - Raynaud's phenomenon OT - microvascular dysfunction OT - myelodysplastic syndrome OT - smoking OT - “scleroderma‐like” pattern EDAT- 2024/12/10 06:23 MHDA- 2024/12/10 06:24 CRDT- 2024/12/10 05:13 PHST- 2024/11/24 00:00 [revised] PHST- 2024/11/04 00:00 [received] PHST- 2024/12/02 00:00 [accepted] PHST- 2024/12/10 06:24 [medline] PHST- 2024/12/10 06:23 [pubmed] PHST- 2024/12/10 05:13 [entrez] AID - 10.1111/1756-185X.70000 [doi] PST - ppublish SO - Int J Rheum Dis. 2024 Dec;27(12):e70000. doi: 10.1111/1756-185X.70000. PMID- 17632601 OWN - NLM STAT- MEDLINE DCOM- 20070726 LR - 20071115 IS - 1175-8716 (Electronic) IS - 0028-8446 (Linking) VI - 120 IP - 1257 DP - 2007 Jun 29 TI - Medical image. A vascular phenomenon. Takayasu's arteritis with Raynaud's phenomenon. PG - U2611 FAU - Nandy, Utpal AU - Nandy U AD - Leicester Royal Infirmary, Leicester, UK. FAU - Varughese, George I AU - Varughese GI FAU - Hock, Yelin L AU - Hock YL LA - eng PT - Case Reports PT - Journal Article DEP - 20070629 PL - New Zealand TA - N Z Med J JT - The New Zealand medical journal JID - 0401067 SB - IM MH - Adult MH - Female MH - Humans MH - Raynaud Disease/*complications/*diagnosis MH - Takayasu Arteritis/*complications/*diagnosis EDAT- 2007/07/17 09:00 MHDA- 2007/07/27 09:00 CRDT- 2007/07/17 09:00 PHST- 2007/07/17 09:00 [pubmed] PHST- 2007/07/27 09:00 [medline] PHST- 2007/07/17 09:00 [entrez] PST - epublish SO - N Z Med J. 2007 Jun 29;120(1257):U2611. PMID- 16476452 OWN - NLM STAT- MEDLINE DCOM- 20060713 LR - 20131121 IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 71 IP - 2 DP - 2006 Mar TI - Exaggerated local hand sympathetic but not renin-angiotensin system activation in patients with primary Raynaud's phenomenon. PG - 128-34 AB - In Raynaud's disease (RD), an overactivity of sympathetic nervous system (SNS) was hypothesized but only indirect proofs were obtained. Complex interactions between the renin-angiotensin system (RAS) and SNS were reported without clear demonstrations of a RAS involvement. Recently, the use of ACE inhibitors and AT1 receptor antagonists in RD patients showed mixed results. The study of total and regional kinetics of tritiated noradrenaline (NA) and the measurements of local Angiotensin (Ang) I and II arterial-venous gradient were performed in 10 RD patients and 10 controls both in rest conditions and following a cold pressor test (CPT). Hand blood flow (HBF) was measured by strain-gauge plethysmography. Baseline HBF was slightly lower in patients than in controls, but during CPT, it significantly decreased only in RD patients (P < 0.01). Total (3H)-NA clearance and spillover were similar in the two groups throughout the study. On the contrary, baseline hand NA spillover was higher in RD patients than in controls and the difference further increased during CPT. Hand NA spillover was linearly related to HBF (P < 0.001). Arterial-venous Ang I and Ang II gradients were positive without difference between controls and patients throughout the study. In conclusion in RD patients, a pathological waste of NA from sympathetic nervous endings of the hand region, exaggerated by sympathetic stimulation, occurs but an enhanced local Ang II formation was not demonstrable. FAU - Coppo, Mirella AU - Coppo M AD - Cardiology 1, Department of Medical and Surgical Critical Care, University of Florence, Florence, Italy. mcoppo@unifi.it FAU - Boddi, Maria AU - Boddi M FAU - Poggesi, Loredana AU - Poggesi L FAU - Bandinelli, Manuela AU - Bandinelli M FAU - Abbate, Rosanna AU - Abbate R FAU - Gensini, Gian Franco AU - Gensini GF LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060213 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - X4W3ENH1CV (Norepinephrine) SB - IM MH - Case-Control Studies MH - Cold Temperature MH - Female MH - Hand/blood supply/*innervation/*physiopathology MH - Humans MH - Male MH - Norepinephrine/blood MH - Plethysmography MH - Raynaud Disease/*physiopathology MH - Reference Standards MH - Regional Blood Flow MH - Renin-Angiotensin System/*physiology MH - Sympathetic Nervous System/*physiopathology EDAT- 2006/02/16 09:00 MHDA- 2006/07/14 09:00 CRDT- 2006/02/16 09:00 PHST- 2005/06/21 00:00 [received] PHST- 2005/12/12 00:00 [revised] PHST- 2005/12/19 00:00 [accepted] PHST- 2006/02/16 09:00 [pubmed] PHST- 2006/07/14 09:00 [medline] PHST- 2006/02/16 09:00 [entrez] AID - S0026-2862(06)00002-1 [pii] AID - 10.1016/j.mvr.2005.12.002 [doi] PST - ppublish SO - Microvasc Res. 2006 Mar;71(2):128-34. doi: 10.1016/j.mvr.2005.12.002. Epub 2006 Feb 13. PMID- 19083682 OWN - NLM STAT- MEDLINE DCOM- 20090323 LR - 20111117 IS - 1532-9283 (Electronic) IS - 1360-8592 (Linking) VI - 12 IP - 3 DP - 2008 Jul TI - Efficacy of myofascial release techniques in the treatment of primary Raynaud's phenomenon. PG - 274-80 LID - 10.1016/j.jbmt.2007.12.002 [doi] AB - OBJECTIVE: This study investigated whether myofascial release techniques performed on upper body connective tissue could mitigate the frequency, duration or pain intensity associated with primary Raynaud's phenomenon. METHODS: Five treatments were administered over a 3-week treatment period on a 35-year-old female experiencing primary Raynaud's phenomenon for the past 12 years. A log was kept documenting frequency, duration and severity of pain. The myofascial work targeted the upper back, neck and arms according to hypothetical fascial meridian lines. RESULTS: Symptom duration was the one characteristic that showed improvement. After the first treatment, the duration of the subject's vasospastic episodes was reduced by almost half and continued to decrease throughout the 3 weeks of treatments. Neither the frequency or number of affected digits varied significantly from the pre-treatment weeks. CONCLUSIONS: The results suggest that by releasing restricted fascia, myofascial techniques may influence the duration and severity of the vasospastic episodes experienced in primary Raynaud's phenomenon. FAU - Walton, Anne AU - Walton A AD - Kiné-Concept Institute Ontario, Ontario, Canada. awalton@hotmail.com LA - eng PT - Case Reports PT - Journal Article DEP - 20080305 PL - United States TA - J Bodyw Mov Ther JT - Journal of bodywork and movement therapies JID - 9700068 SB - IM MH - Adult MH - Fascia/*physiology MH - Female MH - Fingers/blood supply MH - Humans MH - Pain/physiopathology MH - Pain Management MH - *Physical Therapy Modalities MH - Raynaud Disease/*physiopathology/*therapy MH - Sympathetic Nervous System/physiology EDAT- 2008/12/17 09:00 MHDA- 2009/03/24 09:00 CRDT- 2008/12/17 09:00 PHST- 2007/06/23 00:00 [received] PHST- 2007/08/28 00:00 [revised] PHST- 2007/12/04 00:00 [accepted] PHST- 2008/12/17 09:00 [entrez] PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/03/24 09:00 [medline] AID - S1360-8592(08)00004-1 [pii] AID - 10.1016/j.jbmt.2007.12.002 [doi] PST - ppublish SO - J Bodyw Mov Ther. 2008 Jul;12(3):274-80. doi: 10.1016/j.jbmt.2007.12.002. Epub 2008 Mar 5. PMID- 29271704 OWN - NLM STAT- MEDLINE DCOM- 20180424 LR - 20180424 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 47 IP - 2 DP - 2018 Feb TI - Incidental finding of an absent left common carotid artery. PG - 153-155 LID - 10.1024/0301-1526/a000680 [doi] AB - We describe a case of a young woman evaluated for Raynaud's phenomenon in whom an extremely rare variation, the absence of the left common carotid artery, was incidentally detected as an isolated finding. The detection of vascular anomalies may be important for future endovascular or surgical interventions. FAU - Noflatscher, Maria AU - Noflatscher M AD - 1 Department of Internal Medicine, Medical University of Innsbruck, Innsbruck, Austria. FAU - Luger, Anna Katharina AU - Luger AK AD - 2 Department of Radiology, Medical University of Innsbruck, Innsbruck, Austria. FAU - Frank, Renate AU - Frank R AD - 2 Department of Radiology, Medical University of Innsbruck, Innsbruck, Austria. FAU - Glodny, Bernhard AU - Glodny B AD - 2 Department of Radiology, Medical University of Innsbruck, Innsbruck, Austria. FAU - Gruber, Johann AU - Gruber J AD - 1 Department of Internal Medicine, Medical University of Innsbruck, Innsbruck, Austria. FAU - Marschang, Peter AU - Marschang P AD - 1 Department of Internal Medicine, Medical University of Innsbruck, Innsbruck, Austria. LA - eng PT - Case Reports PT - Journal Article DEP - 20171222 PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Adult MH - Carotid Artery, Common/*abnormalities/diagnostic imaging MH - Computed Tomography Angiography MH - Female MH - Humans MH - *Incidental Findings MH - Raynaud Disease/complications/diagnostic imaging/therapy MH - *Vascular Malformations/complications/diagnostic imaging OTO - NOTNLM OT - Carotid artery disease OT - computed tomographic angiography (CTA) OT - duplex ultrasound OT - vascular malformation EDAT- 2017/12/23 06:00 MHDA- 2018/04/25 06:00 CRDT- 2017/12/23 06:00 PHST- 2017/12/23 06:00 [pubmed] PHST- 2018/04/25 06:00 [medline] PHST- 2017/12/23 06:00 [entrez] AID - 10.1024/0301-1526/a000680 [doi] PST - ppublish SO - Vasa. 2018 Feb;47(2):153-155. doi: 10.1024/0301-1526/a000680. Epub 2017 Dec 22. PMID- 31868804 OWN - NLM STAT- MEDLINE DCOM- 20200928 LR - 20210125 IS - 2542-5641 (Electronic) IS - 0366-6999 (Print) IS - 0366-6999 (Linking) VI - 133 IP - 2 DP - 2020 Jan 20 TI - Pulmonary capillary hemangiomatosis or hepatopulmonary syndrome in a patient with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia syndrome? PG - 243-244 LID - 10.1097/CM9.0000000000000596 [doi] FAU - Shrestha, Bijaya Laxmi AU - Shrestha BL AD - Pediatric Department, Guangzhou Medical University, Guangzhou, Guangdong 510623, China. FAU - Jin, Ying-Kang AU - Jin YK AD - Pediatric Pulmonary Department, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong 510623, China. LA - eng PT - Case Reports PT - Journal Article PL - China TA - Chin Med J (Engl) JT - Chinese medical journal JID - 7513795 SB - IM MH - Calcinosis/*diagnosis MH - Child MH - Esophageal Motility Disorders/*diagnosis MH - Female MH - Hepatopulmonary Syndrome/*diagnosis MH - Humans MH - Male MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis MH - Telangiectasis/*diagnosis PMC - PMC7028180 EDAT- 2019/12/24 06:00 MHDA- 2020/09/29 06:00 PMCR- 2020/01/20 CRDT- 2019/12/24 06:00 PHST- 2019/12/24 06:00 [pubmed] PHST- 2020/09/29 06:00 [medline] PHST- 2019/12/24 06:00 [entrez] PHST- 2020/01/20 00:00 [pmc-release] AID - 00029330-202001200-00018 [pii] AID - CMJ-2019-1385 [pii] AID - 10.1097/CM9.0000000000000596 [doi] PST - ppublish SO - Chin Med J (Engl). 2020 Jan 20;133(2):243-244. doi: 10.1097/CM9.0000000000000596. PMID- 18373099 OWN - NLM STAT- MEDLINE DCOM- 20081203 LR - 20211020 IS - 0941-293X (Print) IS - 0941-293X (Linking) VI - 105 IP - 8 DP - 2008 Aug TI - [Optic atrophy, recurrent deterioration of vision and Raynaud's phenomenon]. PG - 770-3 LID - 10.1007/s00347-007-1676-9 [doi] FAU - Papageorgiou, E AU - Papageorgiou E AD - Abt. für Pathophysiologie des Sehens und Neuroophthalmologie, Universitätsaugenklinik Tübingen, Schleichstrasse 12-16, 72076, Tübingen, Deutschland. Eleni.Papageorgiou@med.uni-tuebingen.de FAU - Schiefer, U AU - Schiefer U FAU - Dörnberger, V AU - Dörnberger V FAU - Leo-Kottler, B AU - Leo-Kottler B LA - ger PT - Case Reports PT - Journal Article TT - Optikusatrophie, rezidivierende Sehverschlechterung und Raynaud-Phänomen. PL - Germany TA - Ophthalmologe JT - Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft JID - 9206148 RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Collagen Diseases/*diagnosis/drug therapy MH - Cyclophosphamide/administration & dosage MH - Diagnosis, Differential MH - Drug Therapy, Combination MH - Female MH - Fluorescein Angiography MH - Humans MH - Immunosuppressive Agents/administration & dosage MH - Optic Atrophy/*diagnosis/drug therapy MH - Optic Neuritis/*diagnosis/drug therapy MH - Prednisolone/administration & dosage MH - Raynaud Disease/*diagnosis/drug therapy MH - Sneddon Syndrome/diagnosis/drug therapy MH - Vision Disorders/*diagnosis/drug therapy MH - Visual Fields EDAT- 2008/04/01 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/04/01 09:00 PHST- 2008/04/01 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/04/01 09:00 [entrez] AID - 10.1007/s00347-007-1676-9 [doi] PST - ppublish SO - Ophthalmologe. 2008 Aug;105(8):770-3. doi: 10.1007/s00347-007-1676-9. PMID- 17169096 OWN - NLM STAT- MEDLINE DCOM- 20070119 LR - 20131121 IS - 0385-2407 (Print) IS - 0385-2407 (Linking) VI - 33 IP - 12 DP - 2006 Dec TI - Calcinosis cutis of the fingertip associated with Raynaud's phenomenon. PG - 884-6 AB - Cutaneous calcification may be divided into four major categories: (i) dystrophic; (ii) metastatic; (iii) idiopathic; and (iv) iatrogenic. Dystrophic calcification is the most common type of calcinosis cutis and is associated with a variety of diseases. It most notably occurs in connective tissue diseases. Diffuse and limited cutaneous systemic sclerosis is an example of connective tissue diseases that frequently show calcinosis. We experienced a case of fingertip calcinosis cutis associated with Raynaud's phenomenon. The patient had no previous trauma, skin lesion or systemic connective tissue disease. We propose that calcinosis cutis of the fingertip may result from chronic ischemic injury caused by Raynaud's phenomenon. FAU - Yang, Jeong Hoon AU - Yang JH AD - Department of Dermatology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea. FAU - Kim, Jee Woong AU - Kim JW FAU - Park, Hyun Su AU - Park HS FAU - Jang, Sang Jai AU - Jang SJ FAU - Choi, Jung Chul AU - Choi JC LA - eng PT - Case Reports PT - Journal Article PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - SY7Q814VUP (Calcium) SB - IM MH - Adult MH - Calcinosis/*complications/pathology MH - Calcium/analysis MH - Female MH - Fingers/*pathology MH - Follow-Up Studies MH - Hand Dermatoses/*complications/pathology MH - Humans MH - Keratosis/pathology MH - Raynaud Disease/*complications EDAT- 2006/12/16 09:00 MHDA- 2007/01/20 09:00 CRDT- 2006/12/16 09:00 PHST- 2006/12/16 09:00 [pubmed] PHST- 2007/01/20 09:00 [medline] PHST- 2006/12/16 09:00 [entrez] AID - JDE202 [pii] AID - 10.1111/j.1346-8138.2006.00202.x [doi] PST - ppublish SO - J Dermatol. 2006 Dec;33(12):884-6. doi: 10.1111/j.1346-8138.2006.00202.x. PMID- 17164258 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20070906 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 1 DP - 2007 Jan TI - Clinical vignette. An unusual association with Raynaud's phenomenon. PG - 92 FAU - Al-Deiri, Maria AU - Al-Deiri M AD - Derbyshire Royal Infirmary, Department of Rheumatology, Derby, Derbyshire, UK. FAU - Benn, Jonathan J AU - Benn JJ FAU - Deighton, Chris M AU - Deighton CM LA - eng PT - Case Reports PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2007 May;46(5):894; author reply 894. doi: 10.1093/rheumatology/kem035. PMID: 17384174 MH - Adult MH - Female MH - Humans MH - Hypopituitarism/*complications/diagnosis MH - Magnetic Resonance Imaging MH - Raynaud Disease/*etiology EDAT- 2006/12/14 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/12/14 09:00 PHST- 2006/12/14 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/12/14 09:00 [entrez] AID - 46/1/92 [pii] AID - 10.1093/rheumatology/kel304 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Jan;46(1):92. doi: 10.1093/rheumatology/kel304. PMID- 17258642 OWN - NLM STAT- MEDLINE DCOM- 20070320 LR - 20070129 IS - 0099-2399 (Print) IS - 0099-2399 (Linking) VI - 33 IP - 2 DP - 2007 Feb TI - A case report of a patient with Raynaud's phenomenon undergoing multiple endodontic procedures. PG - 187-90 AB - The pulp represents a terminal circulation. This fact, in addition to other anatomical limitations such as a large volume of pulp: blood supply ratio and the inability of pulp tissue to swell because of its dentinal confines, all may contribute to the susceptibility of the pulp to infection and necrosis. Raynaud's disease primarily affects the terminal vasculature of the fingers, toes or nose. Is there a relationship between patients with Raynaud's disease and the status of the terminal vasculature of their dental pulps? The presented case opens up the question and possibly makes a correlation between peripheral vascular disease and pulpal disease. FAU - Rankin, Charles H AU - Rankin CH AD - Department of Endodontics, Tufts University, School of Dental Medicine, Boston, Massachusetts 02111, USA. charles.rankin@tufts.edu LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Endod JT - Journal of endodontics JID - 7511484 MH - Adult MH - Dental Pulp/*blood supply MH - Dental Pulp Diseases/*etiology MH - Fibrosis/etiology/therapy MH - Humans MH - Male MH - Raynaud Disease/*complications MH - Retreatment MH - Root Canal Therapy EDAT- 2007/01/30 09:00 MHDA- 2007/03/21 09:00 CRDT- 2007/01/30 09:00 PHST- 2006/04/13 00:00 [received] PHST- 2006/08/14 00:00 [revised] PHST- 2006/08/20 00:00 [accepted] PHST- 2007/01/30 09:00 [pubmed] PHST- 2007/03/21 09:00 [medline] PHST- 2007/01/30 09:00 [entrez] AID - S0099-2399(06)00768-0 [pii] AID - 10.1016/j.joen.2006.08.010 [doi] PST - ppublish SO - J Endod. 2007 Feb;33(2):187-90. doi: 10.1016/j.joen.2006.08.010. PMID- 16531439 OWN - NLM STAT- MEDLINE DCOM- 20060816 LR - 20181201 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 IP - 7 DP - 2006 Jul TI - Treatment of severe Raynaud's phenomenon with bosentan in a patient with systemic sclerosis. PG - 911-2 FAU - Dunne, J AU - Dunne J FAU - Dutz, J AU - Dutz J FAU - Shojania, K AU - Shojania K FAU - Ng, B AU - Ng B FAU - van Eeden, S AU - van Eeden S LA - eng PT - Case Reports PT - Letter DEP - 20060310 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Bosentan MH - Female MH - Fingers/blood supply/pathology MH - Gangrene MH - Humans MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*complications MH - Sulfonamides/*therapeutic use EDAT- 2006/03/15 09:00 MHDA- 2006/08/17 09:00 CRDT- 2006/03/15 09:00 PHST- 2006/03/15 09:00 [pubmed] PHST- 2006/08/17 09:00 [medline] PHST- 2006/03/15 09:00 [entrez] AID - kei129 [pii] AID - 10.1093/rheumatology/kei129 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Jul;45(7):911-2. doi: 10.1093/rheumatology/kei129. Epub 2006 Mar 10. PMID- 23765092 OWN - NLM STAT- MEDLINE DCOM- 20140609 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 32 IP - 11 DP - 2013 Nov TI - Focal Raynaud's phenomenon of the 5th digit related to iPad reading posture. PG - 1667 LID - 10.1007/s10067-013-2302-7 [doi] FAU - Touby, Kathleen A AU - Touby KA AD - Neurosensory Sciences, Einstein Medical Center, 5401 Old York Road, Klein 405, Philadelphia, PA, 19141, USA. FAU - Newman, George C AU - Newman GC LA - eng PT - Case Reports PT - Journal Article DEP - 20130614 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Computers, Handheld MH - Fingers/*physiopathology MH - Humans MH - Posture/*physiology MH - Raynaud Disease/*diagnosis/etiology/physiopathology MH - Reading EDAT- 2013/06/15 06:00 MHDA- 2014/06/10 06:00 CRDT- 2013/06/15 06:00 PHST- 2013/03/25 00:00 [received] PHST- 2013/05/20 00:00 [accepted] PHST- 2013/05/05 00:00 [revised] PHST- 2013/06/15 06:00 [entrez] PHST- 2013/06/15 06:00 [pubmed] PHST- 2014/06/10 06:00 [medline] AID - 10.1007/s10067-013-2302-7 [doi] PST - ppublish SO - Clin Rheumatol. 2013 Nov;32(11):1667. doi: 10.1007/s10067-013-2302-7. Epub 2013 Jun 14. PMID- 22190563 OWN - NLM STAT- MEDLINE DCOM- 20120820 LR - 20120425 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 37 IP - 4 DP - 2012 May TI - Fingertip necrosis in a 6-year-old child with primary Raynaud's phenomenon. PG - 363-5 LID - 10.1177/1753193411433509 [doi] FAU - Bhat, W AU - Bhat W FAU - Akhtar, S AU - Akhtar S FAU - Knight, S AU - Knight S LA - eng PT - Case Reports PT - Letter DEP - 20111221 PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/blood MH - Child MH - Female MH - Fingers/*pathology MH - Humans MH - Necrosis MH - Raynaud Disease/*diagnosis/immunology/pathology EDAT- 2011/12/23 06:00 MHDA- 2012/08/21 06:00 CRDT- 2011/12/23 06:00 PHST- 2011/12/23 06:00 [entrez] PHST- 2011/12/23 06:00 [pubmed] PHST- 2012/08/21 06:00 [medline] AID - 1753193411433509 [pii] AID - 10.1177/1753193411433509 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2012 May;37(4):363-5. doi: 10.1177/1753193411433509. Epub 2011 Dec 21. PMID- 30219447 OWN - NLM STAT- MEDLINE DCOM- 20190102 LR - 20190102 IS - 1873-6963 (Electronic) IS - 0965-2299 (Linking) VI - 40 DP - 2018 Oct TI - Effect of topical rosemary essential oil on Raynaud phenomenon in systemic sclerosis. PG - 191-194 LID - S0965-2299(17)30606-4 [pii] LID - 10.1016/j.ctim.2017.10.012 [doi] AB - INTRODUCTION: Raynaud's phenomenon is the earliest manifestation of systemic sclerosis. Nitroglycerin gel is the only proven topical therapy. METHODS: A 53-year-old woman with systemic sclerosis had topical Rosamarinus officinalis (rosemary) oil, often used in anthroposophic medicine, applied to her hands over 3days and then, separately, olive oil. RESULTS: Thermography images showed significant warming of fingers after rosemary oil, but not after olive oil, coinciding with the patient's subjective experience. CONCLUSIONS: Topical Rosamarinus officinalis oil had a vasodilator and warming effect in a patient with systemic sclerosis and Raynaud's phenomenon. CI - Copyright © 2017 Elsevier Ltd. All rights reserved. FAU - von Schoen-Angerer, Tido AU - von Schoen-Angerer T AD - ARCIM Institute, Filderklinik, Filderstadt, Germany; Department of Pediatrics, Fribourg Hospital HFR, Fribourg, Switzerland. Electronic address: tido.von.schoenangerer@gmail.com. FAU - Deckers, Bernhard AU - Deckers B AD - ARCIM Institute, Filderklinik, Filderstadt, Germany; Department of Surgery, Filderklinik, Filderstadt, Germany. FAU - Henes, Jörg AU - Henes J AD - Department of Internal Medicine II, University Hospital Tübingen, Germany. FAU - Helmert, Eduard AU - Helmert E AD - ARCIM Institute, Filderklinik, Filderstadt, Germany. FAU - Vagedes, Jan AU - Vagedes J AD - ARCIM Institute, Filderklinik, Filderstadt, Germany; Department of Pediatrics, University Hospital Tübingen, Germany. LA - eng PT - Case Reports PT - Journal Article DEP - 20171101 PL - Scotland TA - Complement Ther Med JT - Complementary therapies in medicine JID - 9308777 RN - 0 (Oils, Volatile) RN - 8LGU7VM393 (rosemary oil) SB - IM MH - Arm/physiology MH - Female MH - Humans MH - Middle Aged MH - Oils, Volatile/*therapeutic use MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Systemic/*complications MH - Thermography OTO - NOTNLM OT - Raynaud phenomenon OT - Rosmarinus officinalis OT - Scleroderma OT - Systemic sclerosis EDAT- 2018/09/17 06:00 MHDA- 2019/01/03 06:00 CRDT- 2018/09/17 06:00 PHST- 2017/09/28 00:00 [received] PHST- 2017/10/30 00:00 [accepted] PHST- 2018/09/17 06:00 [entrez] PHST- 2018/09/17 06:00 [pubmed] PHST- 2019/01/03 06:00 [medline] AID - S0965-2299(17)30606-4 [pii] AID - 10.1016/j.ctim.2017.10.012 [doi] PST - ppublish SO - Complement Ther Med. 2018 Oct;40:191-194. doi: 10.1016/j.ctim.2017.10.012. Epub 2017 Nov 1. PMID- 30324790 OWN - NLM STAT- MEDLINE DCOM- 20190522 LR - 20190522 IS - 1565-1088 (Print) VI - 20 IP - 10 DP - 2018 Oct TI - Diagnosis of Systemic Sclerosis Based on Raynaud's Phenomenon, Capillaroscopy Findings, and Autoantibodies in the Absence of Sclerodactyly. PG - 658 FAU - Druyan, Amit AU - Druyan A AD - Rheumatology Unit, Sheba Medical Center, Tel Hashomer, Israel. AD - Department of Medicine F, Sheba Medical Center, Tel Hashomer, Israel. FAU - Lidar, Merav AU - Lidar M AD - Rheumatology Unit, Sheba Medical Center, Tel Hashomer, Israel. AD - Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. LA - eng PT - Case Reports PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/*immunology MH - Female MH - Humans MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease/*etiology MH - Scleroderma, Systemic/*diagnosis/immunology/physiopathology EDAT- 2018/10/17 06:00 MHDA- 2019/05/23 06:00 CRDT- 2018/10/17 06:00 PHST- 2018/10/17 06:00 [entrez] PHST- 2018/10/17 06:00 [pubmed] PHST- 2019/05/23 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2018 Oct;20(10):658. PMID- 17097319 OWN - NLM STAT- MEDLINE DCOM- 20070209 LR - 20061212 IS - 1078-5884 (Print) IS - 1078-5884 (Linking) VI - 33 IP - 1 DP - 2007 Jan TI - Letter to the editor re: Thorascopic sympathectomy or Raynaud's phenomenon--a long term follow-up study. Thune TH, Ladegard L & Licht PB. Eur J Vasc Endovasc Surg 32, 198-202 (2006). PG - 133 FAU - Cameron, A AU - Cameron A LA - eng PT - Comment PT - Letter DEP - 20061109 PL - England TA - Eur J Vasc Endovasc Surg JT - European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery JID - 9512728 SB - IM CON - Eur J Vasc Endovasc Surg. 2006 Aug;32(2):198-202. doi: 10.1016/j.ejvs.2006.01.017. PMID: 16564187 MH - Follow-Up Studies MH - Humans MH - Patient Selection MH - Raynaud Disease/*surgery MH - Retrospective Studies MH - *Sympathectomy MH - *Thoracoscopy MH - Treatment Outcome EDAT- 2006/11/14 09:00 MHDA- 2007/02/10 09:00 CRDT- 2006/11/14 09:00 PHST- 2006/09/01 00:00 [received] PHST- 2006/09/18 00:00 [accepted] PHST- 2006/11/14 09:00 [pubmed] PHST- 2007/02/10 09:00 [medline] PHST- 2006/11/14 09:00 [entrez] AID - S1078-5884(06)00531-4 [pii] AID - 10.1016/j.ejvs.2006.09.020 [doi] PST - ppublish SO - Eur J Vasc Endovasc Surg. 2007 Jan;33(1):133. doi: 10.1016/j.ejvs.2006.09.020. Epub 2006 Nov 9. PMID- 22108676 OWN - NLM STAT- MEDLINE DCOM- 20131030 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 4 DP - 2013 Apr TI - Association of von Willebrand factor and fibrinogen plasma levels with primary Raynaud's phenomenon in male and female patients. PG - 1083-4 LID - 10.1007/s00296-011-2236-9 [doi] FAU - Takáts, Alajos-Tamas AU - Takáts AT AD - 1st Department of Surgery, Semmelweis University, Budapest, Hungary. FAU - Shemirani, Amir-Houshang AU - Shemirani AH FAU - Zsóri, Katalin-Szilvia AU - Zsóri KS FAU - András, Csilla AU - András C FAU - Csiki, Zoltán AU - Csiki Z LA - eng PT - Journal Article DEP - 20111116 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (von Willebrand Factor) RN - 9001-32-5 (Fibrinogen) SB - IM MH - Adult MH - Female MH - Fibrinogen/*metabolism MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*blood MH - von Willebrand Factor/*metabolism EDAT- 2011/11/24 06:00 MHDA- 2013/10/31 06:00 CRDT- 2011/11/24 06:00 PHST- 2011/07/18 00:00 [received] PHST- 2011/10/22 00:00 [accepted] PHST- 2011/11/24 06:00 [entrez] PHST- 2011/11/24 06:00 [pubmed] PHST- 2013/10/31 06:00 [medline] AID - 10.1007/s00296-011-2236-9 [doi] PST - ppublish SO - Rheumatol Int. 2013 Apr;33(4):1083-4. doi: 10.1007/s00296-011-2236-9. Epub 2011 Nov 16. PMID- 20233249 OWN - NLM STAT- MEDLINE DCOM- 20100713 LR - 20131121 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 39 IP - 12 DP - 2009 Dec TI - Resolution of visual field constriction with verapamil in a patient with bilateral optic neuropathy, migraine and Raynaud's phenomenon. PG - 851-3 LID - 10.1111/j.1445-5994.2009.02080.x [doi] FAU - Chu, E R L AU - Chu ER FAU - Lee, A W AU - Lee AW FAU - Chen, C S AU - Chen CS LA - eng PT - Case Reports PT - Letter PT - Research Support, Non-U.S. Gov't PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - CJ0O37KU29 (Verapamil) SB - IM MH - Adult MH - Female MH - Humans MH - Migraine Disorders/*drug therapy MH - Optic Nerve Diseases/*drug therapy MH - Raynaud Disease/drug therapy MH - Treatment Outcome MH - Verapamil/*pharmacology/*therapeutic use MH - Visual Fields/*drug effects EDAT- 2010/03/18 06:00 MHDA- 2010/07/14 06:00 CRDT- 2010/03/18 06:00 PHST- 2010/03/18 06:00 [entrez] PHST- 2010/03/18 06:00 [pubmed] PHST- 2010/07/14 06:00 [medline] AID - IMJ2080 [pii] AID - 10.1111/j.1445-5994.2009.02080.x [doi] PST - ppublish SO - Intern Med J. 2009 Dec;39(12):851-3. doi: 10.1111/j.1445-5994.2009.02080.x. PMID- 18285649 OWN - NLM STAT- MEDLINE DCOM- 20080307 LR - 20190513 IS - 1348-9585 (Electronic) IS - 1341-9145 (Linking) VI - 50 IP - 1 DP - 2008 TI - Simultaneous observation of zero-value of FSBP% and Raynaud's phenomenon during cold provocation in vibration syndrome. PG - 75-8 FAU - Fujiwara, Yutaka AU - Fujiwara Y AD - Department of Internal Medicine, Bibai Rosai Hospital, Japan. y-fuji@dg8.so-net.ne.jp FAU - Yoshino, Satoshi AU - Yoshino S FAU - Nasu, Yoshiro AU - Nasu Y LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - J Occup Health JT - Journal of occupational health JID - 9616320 SB - IM MH - Aged MH - Blood Pressure/physiology MH - Blood Pressure Monitoring, Ambulatory/*methods MH - Cold Temperature/*adverse effects MH - Fingers/blood supply/physiopathology MH - Hand-Arm Vibration Syndrome/complications/*diagnosis MH - Humans MH - Male MH - Occupational Diseases/diagnosis MH - *Plethysmography, Impedance MH - Raynaud Disease/complications/*diagnosis EDAT- 2008/02/21 09:00 MHDA- 2008/03/08 09:00 CRDT- 2008/02/21 09:00 PHST- 2008/02/21 09:00 [pubmed] PHST- 2008/03/08 09:00 [medline] PHST- 2008/02/21 09:00 [entrez] AID - JST.JSTAGE/joh/50.75 [pii] AID - 10.1539/joh.50.75 [doi] PST - ppublish SO - J Occup Health. 2008;50(1):75-8. doi: 10.1539/joh.50.75. PMID- 20698174 OWN - NLM STAT- MEDLINE DCOM- 20101203 LR - 20100811 IS - 1427-440X (Print) IS - 1427-440X (Linking) VI - 55 IP - 3 DP - 2009 TI - [Diagnostic value of thermography in Raynaud's phenomenon associated with systemic lupus erythematosus]. PG - 23-7 AB - INTRODUCTION: The study was undertaken to determine of thermographic parameters for the assessment of Raynaud's phenomenon in patients with systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The study was done in 18 patients with SLE and 16 healthy volunteers without vasomotor disorders. Thermography was done with ThermaCAM SC500 infrared camera under conditions defined by the European Association of Thermology. Measurement of hand temperature preceded the cooling test during which the hands in latex gloves were placed in water at 20 degrees C for 1 minute. Palm temperature was measured after 0, 5, 10, and 20 minutes from removal of hands from water. The average temperature of fingers of both hands was calculated. Statistical comparison was done with Student's t-test. RESULTS: 1. The average temperature of fingers in patients with SLE was significantly different than in controls (p < 0.001). 2. The time of warming up of fingers to the starting temperature was longer than 10 minutes in SLE patients and shorter than 10 minutes in healthy controls. CONCLUSION: Thermography is useful for the diagnosis of SLE. FAU - Mikulska, Danuta AU - Mikulska D AD - Katedra i Klinika Chorób Skórnych i Wenerycznych Pomorskiej Akademii Medycznej w Szczecinie al. Powstańców Wlkp. 72, 70-111 Szczecin. LA - pol PT - Controlled Clinical Trial PT - English Abstract PT - Journal Article TT - Wartość diagnostyczna termowizji w ocenie objawu raynauda u chorych na toczeń rumieniowaty układowy. PL - Poland TA - Ann Acad Med Stetin JT - Annales Academiae Medicae Stetinensis JID - 7506854 SB - IM MH - Adult MH - Body Temperature MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/*diagnosis MH - Male MH - Middle Aged MH - Raynaud Disease/*complications/*diagnosis MH - *Thermography EDAT- 2009/01/01 00:00 MHDA- 2010/12/14 06:00 CRDT- 2010/08/12 06:00 PHST- 2010/08/12 06:00 [entrez] PHST- 2009/01/01 00:00 [pubmed] PHST- 2010/12/14 06:00 [medline] PST - ppublish SO - Ann Acad Med Stetin. 2009;55(3):23-7. PMID- 17456649 OWN - NLM STAT- MEDLINE DCOM- 20070524 LR - 20070425 IS - 1526-7598 (Electronic) IS - 0003-2999 (Linking) VI - 104 IP - 5 DP - 2007 May TI - Cerebral oxygen desaturation after cardiopulmonary bypass in a patient with raynaud's phenomenon detected by near-infrared cerebral oximetry. PG - 1034-6, tables of contents AB - Raynaud's phenomenon is characterized by episodes of arterial vasospasm precipitated by cold stress, usually affecting the digits of the hands. There is controversy about the occurrence of vasospasm in internal organ systems. In this report, we present a case of Raynaud's peripheral vasospasm accompanied by cerebral oxygen desaturation as detected by near infrared cerebral oximetry after separation from cardiopulmonary bypass. FAU - Aron, Jesse H AU - Aron JH AD - Department of Anesthesiology, SUNY Upstate Medical University, Syracuse, New York, USA. FAU - Fink, Gregory W AU - Fink GW FAU - Swartz, Michael F AU - Swartz MF FAU - Ford, Brant AU - Ford B FAU - Hauser, Michael C AU - Hauser MC FAU - O'Leary, Colleen E AU - O'Leary CE FAU - Puskas, Ferenc AU - Puskas F LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Anesth Analg JT - Anesthesia and analgesia JID - 1310650 SB - IM MH - Aged MH - Brain/*metabolism MH - Cardiopulmonary Bypass/*adverse effects MH - Female MH - Humans MH - Oximetry/*methods MH - Oxygen Consumption/physiology MH - Raynaud Disease/diagnosis/*metabolism MH - Spectroscopy, Near-Infrared/*methods EDAT- 2007/04/26 09:00 MHDA- 2007/05/26 09:00 CRDT- 2007/04/26 09:00 PHST- 2007/04/26 09:00 [pubmed] PHST- 2007/05/26 09:00 [medline] PHST- 2007/04/26 09:00 [entrez] AID - 104/5/1034 [pii] AID - 10.1213/01.ane.0000260265.53212.fe [doi] PST - ppublish SO - Anesth Analg. 2007 May;104(5):1034-6, tables of contents. doi: 10.1213/01.ane.0000260265.53212.fe. PMID- 29658213 OWN - NLM STAT- MEDLINE DCOM- 20190523 LR - 20190523 IS - 1565-1088 (Print) VI - 20 IP - 1 DP - 2018 Jan TI - Successful Use of a Reduced Dose Regimen of Rituximab in a Case of Rheumatoid Arthritis with Raynaud's Syndrome. PG - 64-65 FAU - Ivanova, Raifa AU - Ivanova R AD - Department of Internship in General Practice and Postgraduate Education, Semey State Medical University, Semey, Kazakhstan. FAU - Goremykina, Maya AU - Goremykina M AD - Department Public Health, Semey State Medical University, Semey, Kazakhstan. FAU - Glushkova, Natalya AU - Glushkova N AD - Department of Internship in General Practice and Postgraduate Education, Semey State Medical University, Semey, Kazakhstan. FAU - Vento, Sandro AU - Vento S AD - Department of Medicine, School of Medicine, Nazarbayev University, Astana, Kazakhstan. AD - University Medical Center, Astana, Kazakhstan. LA - eng PT - Case Reports PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) RN - VB0R961HZT (Prednisone) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adult MH - *Antirheumatic Agents/administration & dosage MH - *Arthritis, Rheumatoid/complications/diagnosis/physiopathology/therapy MH - Humans MH - Immunologic Tests/methods MH - Male MH - Methotrexate/*administration & dosage MH - Patient Acuity MH - Pleural Effusion/diagnostic imaging/etiology/therapy MH - Prednisone/*administration & dosage MH - Radiography/methods MH - *Raynaud Disease/diagnosis/drug therapy/physiopathology MH - Remission Induction/methods MH - Rituximab/*administration & dosage MH - Thoracentesis/methods MH - Treatment Outcome MH - Ultrasonography/methods EDAT- 2018/04/17 06:00 MHDA- 2019/05/24 06:00 CRDT- 2018/04/17 06:00 PHST- 2018/04/17 06:00 [entrez] PHST- 2018/04/17 06:00 [pubmed] PHST- 2019/05/24 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2018 Jan;20(1):64-65. PMID- 17505420 OWN - NLM STAT- MEDLINE DCOM- 20070614 LR - 20161124 IS - 1341-1098 (Print) IS - 1341-1098 (Linking) VI - 13 IP - 2 DP - 2007 Apr TI - Ectopic cystic thymoma associated with Raynaud's phenomenon. PG - 118-21 AB - Mediastinal cystic tumors are well-marginated round lesions that comprise 12% to 18% of all mediastinal masses. These lesions include a variety of diseases with overlapping radiologic appearances and variable prognoses. Pathological examinations are almost always required for differential diagnosis. We encountered a case of anterior mediastinal tumor discovered in the process of investigation of Raynaud's phenomenon. Taking into account the tumor location, a pericardial cyst was initially suspected. However, the tumor was surgically resected and histopathological examinations demonstrated thymus-like tissue in the cyst walls. Raynaud's phenomenon greatly improved after surgery. These findings suggested that cystic thymoma originated from ectopic thymic tissue and is accompanied by paraneoplastic syndrome. FAU - Nomura, Tetsuya AU - Nomura T AD - Department of Cardiovascular Medicine, Kyoto Prefectural University School of Medicine, Kyoto, Japan. FAU - Kawasaki, Tatsuya AU - Kawasaki T FAU - Tanabe, Takuji AU - Tanabe T FAU - Azuma, Akihiro AU - Azuma A FAU - Matsubara, Hiroaki AU - Matsubara H LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Ann Thorac Cardiovasc Surg JT - Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia JID - 9703158 SB - IM MH - Adult MH - Humans MH - Male MH - Raynaud Disease/*complications MH - Thymoma/*complications/diagnostic imaging/pathology MH - Thymus Neoplasms/*complications/diagnostic imaging/pathology MH - Tomography, X-Ray Computed EDAT- 2007/05/17 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/05/17 09:00 PHST- 2006/08/02 00:00 [received] PHST- 2006/09/14 00:00 [accepted] PHST- 2007/05/17 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/05/17 09:00 [entrez] AID - atcs/2007_13_2/118 [pii] PST - ppublish SO - Ann Thorac Cardiovasc Surg. 2007 Apr;13(2):118-21. PMID- 21290877 OWN - NLM STAT- MEDLINE DCOM- 20110318 LR - 20161018 IS - 0023-2130 (Print) IS - 0023-2130 (Linking) IP - 10 DP - 2010 Oct TI - [Comparative estimation of the results of open and endoscopic thoracic sympathectomy in the treatment of Raynaud's phenomenon]. PG - 16-9 AB - Comparative analysis of thoracic sympathectomy efficacy, performed for Raynaud's phenomenon (RP), was accomplished, concerning application either endoscopic or open method. In 62 patients, suffering RP, open or endoscopic thoracic sympathectomy was done in surgical and vascular departments in 1997-2007 yrs. In 32 (51.61%) patients there was performed endoscopic thoracic sympathectomy, in 30 (48.39%)--upper thoracic sympathectomy, using extrapleural supraclavicular access, according to V. N. Klimenko. Immediate results of upper thoracic sympathectomy did not differ trustworthy depending on any method or access. Late follow-up results of upper thoracic sympathectomy were worse than immediate results by 28%--in endoscopic variant and by 43% --in the open one. The positive results rate after endoscopic thoracic sympathectomy have exceeded that after conducting the open one by 15.18%. FAU - Miminoshvili, O I AU - Miminoshvili OI FAU - Perepelitsa, S V AU - Perepelitsa SV FAU - Shapovalov, I N AU - Shapovalov IN LA - rus PT - Clinical Trial PT - English Abstract PT - Journal Article PL - Ukraine TA - Klin Khir JT - Klinichna khirurhiia JID - 9516872 SB - IM MH - Adolescent MH - Adult MH - Blood Flow Velocity/physiology MH - Female MH - Fingers/blood supply MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/physiopathology/*surgery MH - Sympathectomy/*methods MH - Thoracic Surgery, Video-Assisted/*methods MH - Treatment Outcome MH - Vasodilation/physiology MH - Young Adult EDAT- 2011/02/05 06:00 MHDA- 2011/03/19 06:00 CRDT- 2011/02/05 06:00 PHST- 2011/02/05 06:00 [entrez] PHST- 2011/02/05 06:00 [pubmed] PHST- 2011/03/19 06:00 [medline] PST - ppublish SO - Klin Khir. 2010 Oct;(10):16-9. PMID- 24593173 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20181202 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 26 IP - 3 DP - 2016 TI - Effect of ambrisentan on peripheral circulation in patients with systemic sclerosis. PG - 454-7 LID - 10.3109/14397595.2014.885377 [doi] AB - Systemic sclerosis (SSc) is characterized by disturbed blood circulation. The effect of ambrisentan, an endothelin-A receptor-selective antagonist, on impaired peripheral circulation in SSc remains largely elusive. Here we show SSc patients, whose clinical symptoms such as cyanosis and Raynaud's phenomenon, were ameliorated by the treatment with ambrisentan. Additionally, objective evaluations with thermography showed improvement of hand coldness in steady-state and cold challenge tests. Ambrisentan might have a potential to improve peripheral circulation in SSc. FAU - Sumida, Hayakazu AU - Sumida H AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Asano, Yoshihide AU - Asano Y AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Hatano, Masaru AU - Hatano M AD - b Department of Cardiovascular Medicine , Graduate School of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Aozasa, Naohiko AU - Aozasa N AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Toyama, Tetsuo AU - Toyama T AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Akamata, Kaname AU - Akamata K AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Miyazaki, Miki AU - Miyazaki M AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Taniguchi, Takashi AU - Taniguchi T AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Takahashi, Takehiro AU - Takahashi T AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Ichimura, Yohei AU - Ichimura Y AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Noda, Shinji AU - Noda S AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Kuwano, Yoshihiro AU - Kuwano Y AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Yanaba, Koichi AU - Yanaba K AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. FAU - Sato, Shinichi AU - Sato S AD - a Department of Dermatology , Faculty of Medicine, The University of Tokyo , Tokyo , Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20140304 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Endothelin A Receptor Antagonists) RN - 0 (Phenylpropionates) RN - 0 (Pyridazines) RN - HW6NV07QEC (ambrisentan) SB - IM MH - Aged MH - Endothelin A Receptor Antagonists/*therapeutic use MH - Female MH - Humans MH - Middle Aged MH - Phenylpropionates/*therapeutic use MH - Pyridazines/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Treatment Outcome OTO - NOTNLM OT - Ambrisentan OT - Cyanosis OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Thermography EDAT- 2014/03/07 06:00 MHDA- 2016/12/15 06:00 CRDT- 2014/03/06 06:00 PHST- 2014/03/06 06:00 [entrez] PHST- 2014/03/07 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.3109/14397595.2014.885377 [doi] PST - ppublish SO - Mod Rheumatol. 2016;26(3):454-7. doi: 10.3109/14397595.2014.885377. Epub 2014 Mar 4. PMID- 23383543 OWN - NLM STAT- MEDLINE DCOM- 20130307 LR - 20191210 IS - 1427-440X (Print) IS - 1427-440X (Linking) VI - 57 IP - 3 DP - 2011 TI - [The usefulness of capillaroscopy for the evaluation of pulsed magnetic field therapy in patients with primary and secondary Raynaud's phenomenon]. PG - 17-22; discussion 22 AB - INTRODUCTION: Raynaud's phenomenon (RP) as a vasospastic response to cold and emotions has an impact on the quality of life. This also applies to patients with primary (idiopathic) RP. Moreover, RP may also be a factor of irreversible tissue injury manifested by ulceration or necrosis, especially in patients with connective tissue disease. Nailfold videocapillaroscopy (NVC) is a recognized method for diagnosing and monitoring of microvascular abnormalities in rheumatic diseases. The purpose of this study was to assess the usefulness of capillaroscopy for monitoring of the therapeutic effect of low-frequency pulsed magnetic field in 44 patients with RP. MATERIAL AND METHODS: Clinical examination and NVC were performed before and after 2 weeks of treatment. Low-frequency pulsed magnetic field was administered 5 days per week for 2 weeks with the Magnetronic MF-10 generator operating at 40 Hz frequency and 1.0-5.0 mT induction values. Each session lasted 10-20 minutes. RESULTS: Patients with primary and secondary RP experienced a significant decrease in the number and duration of RP episodes and reported a reduction in pain on the Visual Analog Scale. Moreover, an improvement in vascular flow and reduction in interstitial edema was seen with NVC. A correlation between capillaroscopic findings and severity of RP was also observed. CONCLUSIONS: Capillaroscopy is an effective method for evaluation of the effects of low-frequency pulsed magnetic field therapy on the microcirculation in patients with primary and secondary RP. Further prospective studies on the effect of this therapy on capillaroscopic abnormalities in RP patients are needed. FAU - Kuryliszyn-Moskal, Anna AU - Kuryliszyn-Moskal A AD - Klinika Rehabilitacji Uniwersytetu Medycznego w Białymstoku ul. M. Skłodowskiej-Curie 24A, 15-276 Białystok. FAU - Kita, Jacek AU - Kita J FAU - Dakowicz, Agnieszka AU - Dakowicz A FAU - Klimiuk, Piotr A AU - Klimiuk PA LA - pol PT - Evaluation Study PT - Journal Article TT - Przydatnoć kapilaroskopii do oceny terapii impulsowym polem magnetycznym pacjentów z pierwotnym i wtórnym objawem Raynauda. PL - Poland TA - Ann Acad Med Stetin JT - Annales Academiae Medicae Stetinensis JID - 7506854 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - *Magnetic Field Therapy MH - Male MH - Microcirculation MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Pain Measurement MH - Raynaud Disease/*diagnosis/*therapy MH - Young Adult EDAT- 2011/01/01 00:00 MHDA- 2013/03/08 06:00 CRDT- 2013/02/07 06:00 PHST- 2013/02/07 06:00 [entrez] PHST- 2011/01/01 00:00 [pubmed] PHST- 2013/03/08 06:00 [medline] PST - ppublish SO - Ann Acad Med Stetin. 2011;57(3):17-22; discussion 22. PMID- 20515996 OWN - NLM STAT- MEDLINE DCOM- 20100914 LR - 20111209 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 35 IP - 5 DP - 2010 Jun TI - Re: Salem et al. Analysis of rewarming curves in Raynaud's phenomenon of various aetiologies. J Hand Surg Eur. 2009, 34: 621-6. PG - 431-2; author reply 432-3 LID - 10.1177/1753193410365795 [doi] FAU - Proud, George AU - Proud G FAU - Burke, Frank D AU - Burke FD FAU - Lawson, Ian AU - Lawson I FAU - McGeoch, Ken AU - McGeoch K FAU - Bainbridge, L C AU - Bainbridge LC FAU - Baker, M AU - Baker M FAU - Hilliam, R M AU - Hilliam RM FAU - Davies, S AU - Davies S FAU - Deighton, C AU - Deighton C FAU - Manning, G AU - Manning G LA - eng PT - Comment PT - Letter PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 SB - IM CON - J Hand Surg Eur Vol. 2009 Oct;34(5):621-6. doi: 10.1177/1753193409102373. PMID: 19687085 MH - Hand-Arm Vibration Syndrome/complications MH - Humans MH - Raynaud Disease/*diagnosis/etiology MH - *Rewarming MH - *Skin Temperature MH - *Temperature EDAT- 2010/06/03 06:00 MHDA- 2010/09/15 06:00 CRDT- 2010/06/03 06:00 PHST- 2010/06/03 06:00 [entrez] PHST- 2010/06/03 06:00 [pubmed] PHST- 2010/09/15 06:00 [medline] AID - 35/5/431 [pii] AID - 10.1177/1753193410365795 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2010 Jun;35(5):431-2; author reply 432-3. doi: 10.1177/1753193410365795. PMID- 16960964 OWN - NLM STAT- MEDLINE DCOM- 20061213 LR - 20151119 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 33 IP - 9 DP - 2006 Sep TI - Thermographic and symptomatic effect of a single dose of sildenafil citrate on Raynaud's phenomenon in patients with systemic sclerosis: a potential treatment. PG - 1918-9 FAU - Kumar, Namita AU - Kumar N FAU - Griffiths, Bridget AU - Griffiths B FAU - Allen, John AU - Allen J LA - eng PT - Case Reports PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Adult MH - Dose-Response Relationship, Drug MH - Feasibility Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Piperazines/*administration & dosage MH - Purines MH - Raynaud Disease/diagnosis/*drug therapy/etiology MH - Scleroderma, Systemic/*complications/diagnosis/drug therapy MH - Sildenafil Citrate MH - Skin Temperature/*drug effects MH - Sulfones MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage EDAT- 2006/09/09 09:00 MHDA- 2006/12/14 09:00 CRDT- 2006/09/09 09:00 PHST- 2006/09/09 09:00 [pubmed] PHST- 2006/12/14 09:00 [medline] PHST- 2006/09/09 09:00 [entrez] AID - 0315162X--33-1918 [pii] PST - ppublish SO - J Rheumatol. 2006 Sep;33(9):1918-9. PMID- 21105471 OWN - NLM STAT- MEDLINE DCOM- 20101228 LR - 20101124 IS - 0023-2149 (Print) IS - 0023-2149 (Linking) VI - 88 IP - 2 DP - 2010 TI - [Clinical and pathogenetic significance of antibodies to antioxidative enzymes in patients with Raynaud's syndrome]. PG - 43-6 AB - Patients presenting with systemic scleroderma were found to have antibodies to antioxidative enzymes the levels of which increased with activity of the disease. Taking into account the important role of immune disorders in the development of systemic sclerosis, it can be conjectured that antienzyme antibodies may cause dysregulation of enzymatic systems. Serum antibodies were detected by the original modification of indirect enzyme immunoassay using immobilized antigenic forms of enzymes. All patients with high antibody titers presented with class II-IV Raynaud"s syndrome (RS). Pathogenetic mechanisms of RS are complicated and poorly known, it is supposed to be a multifactor pathology associated with disturbances in neural, vascular, mediator, and immune systems. Reperfusion and free oxygen radicals may also contribute to the development of ischemia in RS. The antioxidative system is known to involve a number of enzymes that neutralize pathogenic effects of free oxygen species. Disturbance of equilibrium between aggressive and protective factors under pathological conditions leads to further aggravation of tissue lesions. The presence of antienzyme antibodies in the blood of patients with primary RS may be regarded as an unfavourable prognostic factor preceding a systemic disease of connective tissue. FAU - Shilova, L N AU - Shilova LN FAU - Gontar', I P AU - Gontar' IP FAU - Zborovskaia, I A AU - Zborovskaia IA FAU - Novikova, O V AU - Novikova OV FAU - Emel'ianov, N N AU - Emel'ianov NN LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Klin Med (Mosk) JT - Klinicheskaia meditsina JID - 2985204R RN - 0 (Autoantibodies) RN - EC 1.11.1.9 (Glutathione Peroxidase) RN - EC 1.15.1.1 (Superoxide Dismutase) RN - EC 1.8.1.7 (Glutathione Reductase) SB - IM MH - Adult MH - Autoantibodies/*blood MH - Female MH - Glutathione Peroxidase/immunology MH - Glutathione Reductase/immunology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/blood/etiology/*immunology MH - Scleroderma, Systemic/blood/complications/immunology MH - Superoxide Dismutase/immunology EDAT- 2010/11/26 06:00 MHDA- 2010/12/29 06:00 CRDT- 2010/11/26 06:00 PHST- 2010/11/26 06:00 [entrez] PHST- 2010/11/26 06:00 [pubmed] PHST- 2010/12/29 06:00 [medline] PST - ppublish SO - Klin Med (Mosk). 2010;88(2):43-6. PMID- 27608801 OWN - NLM STAT- MEDLINE DCOM- 20170717 LR - 20170717 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 68 IP - 2 DP - 2016 Sep 9 TI - Critical finger ischemia and myocardial fibrosis development after sudden interruption of sildenafil treatment in a systemic sclerosis patient. PG - 109-11 LID - 10.4081/reumatismo.2016.889 [doi] AB - Systemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud's Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non -responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment. FAU - Bruni, C AU - Bruni C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze. cosimobruni85@gmail.com. FAU - Bellando-Randone, S AU - Bellando-Randone S FAU - Gargani, L AU - Gargani L FAU - Picano, E AU - Picano E FAU - Pingitore, A AU - Pingitore A FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M FAU - Guiducci, S AU - Guiducci S LA - eng PT - Case Reports PT - Journal Article DEP - 20160909 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Antirheumatic Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Antirheumatic Agents/*adverse effects/therapeutic use MH - Cardiomyopathies/etiology/*pathology MH - Fingers/*blood supply MH - Humans MH - Ischemia MH - Male MH - Middle Aged MH - Myocardium/pathology MH - Raynaud Disease/complications/*drug therapy/pathology MH - Risk Factors MH - Scleroderma, Systemic/complications/*drug therapy/pathology MH - Sildenafil Citrate/*adverse effects/therapeutic use MH - *Substance Withdrawal Syndrome MH - Time Factors EDAT- 2016/09/10 06:00 MHDA- 2017/07/18 06:00 CRDT- 2016/09/10 06:00 PHST- 2016/05/05 00:00 [received] PHST- 2016/08/01 00:00 [accepted] PHST- 2016/07/19 00:00 [revised] PHST- 2016/09/10 06:00 [entrez] PHST- 2016/09/10 06:00 [pubmed] PHST- 2017/07/18 06:00 [medline] AID - 10.4081/reumatismo.2016.889 [doi] PST - epublish SO - Reumatismo. 2016 Sep 9;68(2):109-11. doi: 10.4081/reumatismo.2016.889. PMID- 19116527 OWN - NLM STAT- MEDLINE DCOM- 20090223 LR - 20210106 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 123 IP - 1 DP - 2009 Jan TI - Reoperative digital sympathectomy in refractory Raynaud's phenomenon. PG - 36e-38e LID - 10.1097/PRS.0b013e3181905725 [doi] FAU - Dorafshar, Amir H AU - Dorafshar AH AD - Section of Plastic and Reconstructive Surgeons, University of Chicago, Chicago, Ill. (Dorafshar, Seitz, Zachary). FAU - Seitz, Iris A AU - Seitz IA FAU - Zachary, Lawrence AU - Zachary L LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM MH - Adult MH - Female MH - Fingers/*innervation/*surgery MH - Humans MH - Middle Aged MH - Raynaud Disease/*surgery MH - Refractory Period, Electrophysiological MH - Reoperation MH - Sympathectomy/*methods EDAT- 2009/01/01 09:00 MHDA- 2009/02/24 09:00 CRDT- 2009/01/01 09:00 PHST- 2009/01/01 09:00 [entrez] PHST- 2009/01/01 09:00 [pubmed] PHST- 2009/02/24 09:00 [medline] AID - 00006534-200901000-00088 [pii] AID - 10.1097/PRS.0b013e3181905725 [doi] PST - ppublish SO - Plast Reconstr Surg. 2009 Jan;123(1):36e-38e. doi: 10.1097/PRS.0b013e3181905725. PMID- 22874636 OWN - NLM STAT- MEDLINE DCOM- 20130304 LR - 20121221 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 66 IP - 1 DP - 2013 Jan TI - Breast reconstruction in the high risk patient with systemic connective tissue disease: a case series. PG - 61-6 LID - S1748-6815(12)00433-0 [pii] LID - 10.1016/j.bjps.2012.07.024 [doi] AB - INTRODUCTION: The presence of severe underlying connective tissue disease may restrict the reconstructive options offered to a woman in the event of mastectomy. Putative concerns about reconstructive surgery include the effects of connective tissue disease and immunosuppression on wound healing and donor site morbidity, and increased risks of deranged clotting and thrombophilia after free tissue transfer. There is also the possibility of an unpredictable tissue reaction after oncological resection surgery and adjuvant radiotherapy. METHODOLOGY: Here we present a review of the current sparse evidence regarding reconstructive breast surgery in this challenging group of patients. In addition we present a series of six consecutive patients with a spectrum of connective tissue disorders including combinations of longstanding Systemic Lupus Erythematosis (SLE), Rheumatoid arthritis and Raynaud's Disease who underwent successful post-mastectomy reconstruction with an extended autologous latissimus dorsi flap, along with subsequent successful correction of asymmetry and/or nipple reconstruction. RESULTS: There is a paucity of literature on this subject perhaps suggesting that surgeons are reluctant to offer reconstruction or that uptake is poor in this group. Complications related to radiotherapy and free tissue transfer in patients with severe CTD is less than may be expected. The most common complications experienced by our patients with CTD after extended ALD breast reconstruction were persistent donor site seroma, wound dehiscence and delayed haematoma formation, reflecting the abnormal inflammatory response and deranged haemostatic cascade common to connective tissue disease. However, all six patients made a full recovery from surgery without residual donor site morbidity and with an acceptable aesthetic breast reconstruction. CONCLUSION: Careful peri-operative management is crucial in this group of patients, but good outcomes are possible using a variety of reconstructive techniques. This is the first reported series of patients with severe connective tissue disease who have been managed with extended ALD breast reconstruction. The majority of complications relate to the donor site but the favourable outcomes demonstrate that the extended ALD flap remains a reliable reconstructive option for this group. CI - Copyright © 2012 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Chin, K Y AU - Chin KY AD - Burns and Plastic Surgery, Canniesburn Unit, Glasgow Royal Infirmary, Glasgow G4 0SF, UK. andycky@doctors.org.uk FAU - Chalmers, C R AU - Chalmers CR FAU - Bryson, A V AU - Bryson AV FAU - Weiler-Mithoff, E M AU - Weiler-Mithoff EM LA - eng PT - Journal Article DEP - 20120805 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 SB - IM MH - Adult MH - Arthritis, Rheumatoid/complications MH - Breast Neoplasms/*surgery MH - Carcinoma, Ductal, Breast/*surgery MH - Connective Tissue Diseases/*complications MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/complications MH - *Mammaplasty/adverse effects/methods MH - Mastectomy MH - Middle Aged MH - Muscle, Skeletal/surgery MH - Raynaud Disease/complications MH - Risk Factors MH - *Surgical Flaps/adverse effects EDAT- 2012/08/10 06:00 MHDA- 2013/03/05 06:00 CRDT- 2012/08/10 06:00 PHST- 2012/02/05 00:00 [received] PHST- 2012/06/12 00:00 [revised] PHST- 2012/07/22 00:00 [accepted] PHST- 2012/08/10 06:00 [entrez] PHST- 2012/08/10 06:00 [pubmed] PHST- 2013/03/05 06:00 [medline] AID - S1748-6815(12)00433-0 [pii] AID - 10.1016/j.bjps.2012.07.024 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2013 Jan;66(1):61-6. doi: 10.1016/j.bjps.2012.07.024. Epub 2012 Aug 5. PMID- 19051747 OWN - NLM STAT- MEDLINE DCOM- 20090128 LR - 20190724 IS - 0021-5384 (Print) IS - 0021-5384 (Linking) VI - 97 IP - 10 DP - 2008 Oct 10 TI - [Ectopic thymic carcinoma diagnosed by long-term Raynaud's phenomenon]. PG - 2546-8 FAU - Morishima, Atsuhito AU - Morishima A AD - Department of Internal Medicine, NTT West Osaka Hospital, Osaka. FAU - Maeda, Keiji AU - Maeda K FAU - Andou, Maiko AU - Andou M FAU - Nakahara, Eiko AU - Nakahara E FAU - Kuritani, Taro AU - Kuritani T FAU - Igarashi, Tsuyoshi AU - Igarashi T FAU - Takemoto, Hirotoshi AU - Takemoto H FAU - Onishi, Tyoku AU - Onishi T FAU - Okamoto, Shigeru AU - Okamoto S LA - jpn PT - Case Reports PT - Journal Article PL - Japan TA - Nihon Naika Gakkai Zasshi JT - Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine JID - 19130210R SB - IM MH - Choristoma/*complications/surgery MH - Humans MH - Male MH - Mediastinal Neoplasms/*complications/surgery MH - Middle Aged MH - Paraneoplastic Syndromes/*etiology MH - Raynaud Disease/*etiology MH - Thymoma/*complications/surgery MH - Thymus Neoplasms/*complications/surgery MH - Time Factors MH - Treatment Outcome EDAT- 2008/12/05 09:00 MHDA- 2009/01/29 09:00 CRDT- 2008/12/05 09:00 PHST- 2008/12/05 09:00 [pubmed] PHST- 2009/01/29 09:00 [medline] PHST- 2008/12/05 09:00 [entrez] AID - 10.2169/naika.97.2546 [doi] PST - ppublish SO - Nihon Naika Gakkai Zasshi. 2008 Oct 10;97(10):2546-8. doi: 10.2169/naika.97.2546. PMID- 28276952 OWN - NLM STAT- MEDLINE DCOM- 20180328 LR - 20180328 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 46 IP - 6 DP - 2017 Nov TI - Magnetic resonance imaging-detected digital artery stenosis associated with Raynaud's phenomenon in a patient with anti-signal recognition particle antibody-associated myopathy: a rare complication. PG - 503-504 LID - 10.1080/03009742.2017.1284261 [doi] FAU - Horikoshi, M AU - Horikoshi M AD - a Department of Rheumatology , Saitama Red Cross Hospital , Saitama , Japan. AD - b Department of Rheumatology , Sainokuni Higashiomiya Medical Center , Saitama , Japan. FAU - Kumagai, T AU - Kumagai T AD - b Department of Rheumatology , Sainokuni Higashiomiya Medical Center , Saitama , Japan. AD - c Department of Ophthalmology , Saitama Medical University Hospital , Saitama , Japan. FAU - Takagi, K AU - Takagi K AD - b Department of Rheumatology , Sainokuni Higashiomiya Medical Center , Saitama , Japan. LA - eng PT - Case Reports PT - Letter DEP - 20170302 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - 0 (Signal Recognition Particle) RN - 0 (Vasodilator Agents) RN - EC 2.7.3.2 (Creatine Kinase) RN - F5TD010360 (Alprostadil) SB - IM MH - Alprostadil/*administration & dosage MH - *Arteries/diagnostic imaging/physiopathology MH - Autoantibodies/blood MH - Constriction, Pathologic MH - Creatine Kinase/blood MH - Female MH - *Fingers/blood supply MH - Humans MH - Immunosuppressive Agents/administration & dosage MH - Magnetic Resonance Imaging/*methods MH - Middle Aged MH - *Polymyositis/diagnosis/drug therapy/etiology/immunology MH - *Raynaud Disease/complications/diagnosis/physiopathology MH - Signal Recognition Particle/*immunology MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage EDAT- 2017/03/10 06:00 MHDA- 2018/03/29 06:00 CRDT- 2017/03/10 06:00 PHST- 2017/03/10 06:00 [pubmed] PHST- 2018/03/29 06:00 [medline] PHST- 2017/03/10 06:00 [entrez] AID - 10.1080/03009742.2017.1284261 [doi] PST - ppublish SO - Scand J Rheumatol. 2017 Nov;46(6):503-504. doi: 10.1080/03009742.2017.1284261. Epub 2017 Mar 2. PMID- 19340395 OWN - NLM STAT- MEDLINE DCOM- 20091106 LR - 20161020 IS - 1439-7595 (Print) IS - 1439-7595 (Linking) VI - 19 IP - 4 DP - 2009 TI - A case of cold agglutinin disease in the course of treatment for polymyalgia rheumatica. PG - 427-30 LID - 10.1007/s10165-009-0167-z [doi] AB - A 60-year-old male who had been treated for polymyalgia rheumatica (PMR) with Raynaud's phenomenon was admitted to our hospital with acrocyanosis and rapid progressive anemia. Hemolytic anemia with reduction of haptoglobin and cold agglutinin was detected, and the patient had a negative Donath-Landsteiner test. A skin biopsy from his toe revealed microthromboembolism without vasculitis, resulting in the diagnosis of cold agglutinin disease (CAD). This is a first case report of CAD complicated with PMR. FAU - Nakamura, Hideki AU - Nakamura H AD - Unit of Translational Medicine, Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, Nagasaki 852-8501, Japan. nhideki@nagasaki-u.ac.jp FAU - Kamiya-Matsumoto, Kayo AU - Kamiya-Matsumoto K FAU - Kawakami, Atsushi AU - Kawakami A FAU - Ida, Hiroaki AU - Ida H FAU - Hayashi, Tomayoshi AU - Hayashi T FAU - Sato, Shinichi AU - Sato S FAU - Kamihira, Shimeru AU - Kamihira S FAU - Eguchi, Katsumi AU - Eguchi K LA - eng PT - Case Reports PT - Journal Article DEP - 20090402 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Anti-Inflammatory Agents) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Administration, Oral MH - Anemia, Hemolytic, Autoimmune/complications/*pathology MH - Anti-Inflammatory Agents/therapeutic use MH - Humans MH - Male MH - Middle Aged MH - Polymyalgia Rheumatica/complications/drug therapy/*pathology MH - Prednisolone/therapeutic use MH - Raynaud Disease/complications/drug therapy/*pathology MH - Treatment Outcome EDAT- 2009/04/03 09:00 MHDA- 2009/11/07 06:00 CRDT- 2009/04/03 09:00 PHST- 2009/01/19 00:00 [received] PHST- 2009/03/10 00:00 [accepted] PHST- 2009/04/03 09:00 [entrez] PHST- 2009/04/03 09:00 [pubmed] PHST- 2009/11/07 06:00 [medline] AID - 10.1007/s10165-009-0167-z [doi] PST - ppublish SO - Mod Rheumatol. 2009;19(4):427-30. doi: 10.1007/s10165-009-0167-z. Epub 2009 Apr 2. PMID- 17116656 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20181201 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 2 DP - 2007 Feb TI - Assessment of digital vascular structure and function in response to bosentan in patients with systemic sclerosis-related Raynaud's phenomenon. PG - 363-4 FAU - Moore, T L AU - Moore TL FAU - Vail, A AU - Vail A FAU - Herrick, A L AU - Herrick AL LA - eng PT - Clinical Trial PT - Letter DEP - 20061120 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Bosentan MH - Endothelin Receptor Antagonists MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Microcirculation/drug effects MH - Middle Aged MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*complications MH - Sulfonamides/*therapeutic use EDAT- 2006/11/23 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/11/23 09:00 PHST- 2006/11/23 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/11/23 09:00 [entrez] AID - kel383 [pii] AID - 10.1093/rheumatology/kel383 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Feb;46(2):363-4. doi: 10.1093/rheumatology/kel383. Epub 2006 Nov 20. PMID- 23171559 OWN - NLM STAT- MEDLINE DCOM- 20130114 LR - 20121122 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 156 IP - 47 DP - 2012 TI - [An elderly woman with stiffness of the thumb]. PG - A4550 AB - A 81-year-old woman presented with progressive pain, stiffness and swelling of the right thumb. She also complained of dysphagia and Raynaud's phenomenon. Physical examination revealed sclerodactyly and telangiectasia. A plain X-ray showed marked calcifications of the flexor tendon of the right thumb and esophagography demonstrated decreased motility of the lower esophagus. Additionally, anticentromere antibodies were positive. As a result, the patient was diagnosed with calcinosis as a presenting symptom of limited systemic scleroderma. FAU - Franssen, Laurens E AU - Franssen LE AD - St. Antonius Ziekenhuis, afd. Interne Geneeskunde, Nieuwegein, the Netherlands. franssen.laurens@gmail.com FAU - Franssen, Marcel J A M AU - Franssen MJ FAU - Jeurissen, Maurice E C AU - Jeurissen M LA - dut PT - Case Reports PT - English Abstract PT - Journal Article TT - Een oude vrouw met een stijve duim. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Autoantibodies) SB - IM MH - Aged, 80 and over MH - Autoantibodies/analysis MH - Centromere/immunology MH - Female MH - Humans MH - Raynaud Disease/*diagnosis/immunology MH - Scleroderma, Limited/*diagnosis/immunology MH - Thumb/*pathology EDAT- 2012/11/23 06:00 MHDA- 2013/01/15 06:00 CRDT- 2012/11/23 06:00 PHST- 2012/11/23 06:00 [entrez] PHST- 2012/11/23 06:00 [pubmed] PHST- 2013/01/15 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2012;156(47):A4550. PMID- 28188718 OWN - NLM STAT- MEDLINE DCOM- 20190715 LR - 20190715 IS - 0002-5151 (Print) IS - 0002-5151 (Linking) VI - 64 IP - 1 DP - 2017 Jan-Mar TI - [Hematopoietic stem cells transplant in patients with common variable immunodeficiency. Is a therapeutic option?]. PG - 121-125 AB - BACKGROUND: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. CASE REPORT: Twenty-six-year old woman with no family or hereditary history of primary immune deficiencies or consanguinity, with repeated episodes of otitis, sinusitis, gastroenteritis and bronchitis since childhood. At adolescence, she was diagnosed with common variable immunodeficiency; she was prescribed intravenous gamma-globulin, broad-spectrum antimicrobials and macrolides. At 22 years of age, she underwent hematopoietic stem cell transplantation owing to continued severe infections. At 4 months, post-transplantation she was diagnosed with hypothyroidism and ovarian insufficiency. During the following 3 years, she had no infections, but at 25 years of age she had immune thrombocytopenic purpura diagnosed, which persists together with Raynaud's disease and upper respiratory tract persistent infections. At the moment of this report she is being treated with intravenous gamma-globulin and receiving prophylaxis with clarithromycin, without steroids or danazol. CONCLUSIONS: Given the high rate of morbidity and mortality associated and immune reconstitution failure, hematopoietic stem cell transplantation should be carefully evaluated in patients with treatment-unresponsive infections or lymphoproliferative disorders. FAU - Cambray-Gutiérrez, Julio César AU - Cambray-Gutiérrez JC AD - Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica. Ciudad de México, México. jcesar_963@hotmail.com. FAU - Herrera-Sánchez, Diana Andrea AU - Herrera-Sánchez DA FAU - López-Pérez, Patricia AU - López-Pérez P FAU - Chávez-García, Aurora AU - Chávez-García A FAU - Yamazaki-Nakashimada, Marco Antonio AU - Yamazaki-Nakashimada MA LA - spa PT - Case Reports PT - Journal Article TT - Trasplante de médula ósea en pacientes con inmunodeficiencia común variable, ¿es una opción terapéutica? PL - Mexico TA - Rev Alerg Mex JT - Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993) JID - 9438824 RN - 0 (Anti-Bacterial Agents) RN - 0 (Immunoglobulins, Intravenous) RN - Hypothyroidism, Autoimmune SB - IM MH - Adult MH - Anti-Bacterial Agents/therapeutic use MH - Bronchiectasis/etiology MH - Combined Modality Therapy MH - Common Variable Immunodeficiency/*therapy MH - Female MH - Follow-Up Studies MH - Hashimoto Disease/etiology MH - *Hematopoietic Stem Cell Transplantation MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - Primary Ovarian Insufficiency/chemically induced MH - Purpura, Thrombocytopenic, Idiopathic/etiology MH - Raynaud Disease/etiology MH - Recurrence MH - Respiratory Tract Infections/etiology MH - Thyroiditis, Autoimmune/etiology OTO - NOTNLM OT - Common variable immunodeficiency OT - Hematopoietic progenitor cell transplant OT - Human gamma globulin EDAT- 2017/02/12 06:00 MHDA- 2019/07/16 06:00 CRDT- 2017/02/12 06:00 PHST- 2017/02/12 06:00 [entrez] PHST- 2017/02/12 06:00 [pubmed] PHST- 2019/07/16 06:00 [medline] AID - 10.29262/ram.v64i1.169 [doi] PST - ppublish SO - Rev Alerg Mex. 2017 Jan-Mar;64(1):121-125. doi: 10.29262/ram.v64i1.169. PMID- 17222301 OWN - NLM STAT- MEDLINE DCOM- 20070309 LR - 20070115 IS - 0004-8380 (Print) IS - 0004-8380 (Linking) VI - 48 IP - 1 DP - 2007 Feb TI - Unilateral limited scleroderma-like changes following formation of an arteriovenous fistula. PG - 37-9 AB - A 55-year-old man presented with a 1-year history of progressive skin thickening involving the right hand associated with Raynaud's phenomenon, sclerodactyly and painful cutaneous ulcers. An arteriovenous fistula for haemodialysis had been formed on the same arm 2 years previously. There were no symptoms or signs of systemic sclerosis or involvement of the contralateral arm. The limb was clinically ischaemic, attributed to a vascular steal phenomenon from the arteriovenous fistula, superimposed on occlusive arterial disease. A revascularization procedure was performed, which resulted in substantial improvement in the sclerodactyly, Raynaud's phenomenon and hand function. Tissue hypoxia is believed to be a contributing factor in the pathogenesis of scleroderma, and this case demonstrates scleroderma-like changes in the setting of limb ischaemia. FAU - Roberts, Hugh AU - Roberts H AD - Department of Dermatology, Alfred Hospital, Prahran, Victoria, Australia. hughroberts1@bigpond.com FAU - Curnow, Paul A AU - Curnow PA LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - Australas J Dermatol JT - The Australasian journal of dermatology JID - 0135232 SB - IM MH - *Arteriovenous Fistula MH - Diagnosis, Differential MH - Hand Dermatoses/*diagnosis/etiology/pathology MH - Humans MH - Kidney Failure, Chronic MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/etiology/pathology MH - Renal Dialysis/*adverse effects MH - Scleroderma, Systemic/*diagnosis/etiology/pathology EDAT- 2007/01/16 09:00 MHDA- 2007/03/10 09:00 CRDT- 2007/01/16 09:00 PHST- 2007/01/16 09:00 [pubmed] PHST- 2007/03/10 09:00 [medline] PHST- 2007/01/16 09:00 [entrez] AID - AJD325 [pii] AID - 10.1111/j.1440-0960.2007.00325.x [doi] PST - ppublish SO - Australas J Dermatol. 2007 Feb;48(1):37-9. doi: 10.1111/j.1440-0960.2007.00325.x. PMID- 24760690 OWN - NLM STAT- MEDLINE DCOM- 20140609 LR - 20140424 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 139 IP - 17 DP - 2014 May TI - [53-year-old patient with Raynaud's syndrome. polyneuropathia and fingertip necrosis]. PG - 883-4 LID - 10.1055/s-0034-1369928 [doi] FAU - Märker-Hermann, E AU - Märker-Hermann E AD - Klinik Innere Medizin IV (Rheumatologie, Klinische Immunologie, Nephrologie), HSK Dr. Horst Schmidt Klinik GmbH. LA - ger PT - Case Reports PT - Journal Article TT - 53-jähriger Patient mit Verfärbungen der Finger bei Kälte, Fuß- und Beinschmerzen und Fingerkuppennekrosen. DEP - 20140423 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Immunoglobulin lambda-Chains) SB - IM MH - Cryoglobulinemia/*diagnosis MH - Diagnosis, Differential MH - Fingers/*blood supply/pathology MH - Foot/innervation MH - Humans MH - *Immunoglobulin lambda-Chains MH - Ischemia/*diagnosis MH - Leg/blood supply MH - Male MH - Middle Aged MH - Necrosis MH - Paraproteinemias/*diagnosis MH - Phlebitis/diagnosis MH - Raynaud Disease/*diagnosis EDAT- 2014/04/25 06:00 MHDA- 2014/06/10 06:00 CRDT- 2014/04/25 06:00 PHST- 2014/04/25 06:00 [entrez] PHST- 2014/04/25 06:00 [pubmed] PHST- 2014/06/10 06:00 [medline] AID - 10.1055/s-0034-1369928 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2014 May;139(17):883-4. doi: 10.1055/s-0034-1369928. Epub 2014 Apr 23. PMID- 18275771 OWN - NLM STAT- MEDLINE DCOM- 20080623 LR - 20190917 IS - 0014-2565 (Print) IS - 0014-2565 (Linking) VI - 208 IP - 3 DP - 2008 Mar TI - [Patient with Raynaud's phenomenon, pleuropericardial efussion and digital infarctions]. PG - 158-60 FAU - Muñoz-Morente, A AU - Muñoz-Morente A AD - Servicio de Medicina Interna, Hospital de la Serranía, Ronda, Málaga, España. FAU - Ramos, M A Baron AU - Ramos MA FAU - Costa, M Grana AU - Costa MG FAU - Jiménez, M Maíz AU - Jiménez MM LA - spa PT - Case Reports PT - Journal Article TT - Paciente con fenómeno de Raynaud, derrame pleuropericárdico e infartos digitales. PL - Spain TA - Rev Clin Esp JT - Revista clinica espanola JID - 8608576 SB - IM MH - Aged MH - Carcinoma, Squamous Cell/*complications MH - Fingers/blood supply MH - Humans MH - Infarction/etiology MH - Lung Neoplasms/*complications MH - Male MH - Paraneoplastic Syndromes/*etiology MH - Pericardial Effusion/etiology MH - Pleural Effusion/etiology MH - Raynaud Disease/etiology EDAT- 2008/02/16 09:00 MHDA- 2008/06/24 09:00 CRDT- 2008/02/16 09:00 PHST- 2008/02/16 09:00 [pubmed] PHST- 2008/06/24 09:00 [medline] PHST- 2008/02/16 09:00 [entrez] AID - 13115825 [pii] AID - 10.1157/13115825 [doi] PST - ppublish SO - Rev Clin Esp. 2008 Mar;208(3):158-60. doi: 10.1157/13115825. PMID- 19785781 OWN - NLM STAT- MEDLINE DCOM- 20091130 LR - 20131121 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 153 DP - 2009 TI - [Diagnostic image. A breastfeeding woman with a painful, blue nipple]. PG - B343 AB - A 32-year-old breastfeeding woman presented with a painful, blue nipple due to Raynaud's syndrome. FAU - Velstra, Berit AU - Velstra B AD - Spaarne Ziekenhuis, afd. Heelkunde, Hoofddorp, The Netherlands. beritvelstra@hotmail.com FAU - Bijlsma, Taco S AU - Bijlsma TS LA - dut PT - Case Reports PT - English Abstract PT - Journal Article TT - Een borstvoedende vrouw met een pijnlijke, blauwe tepel. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Feeding MH - Calcium Channel Blockers/therapeutic use MH - Female MH - Humans MH - Nifedipine/therapeutic use MH - Nipples/*blood supply/pathology MH - Raynaud Disease/*complications/diagnosis/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2009/09/30 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/30 06:00 PHST- 2009/09/30 06:00 [entrez] PHST- 2009/09/30 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2009;153:B343. PMID- 18214409 OWN - NLM STAT- MEDLINE DCOM- 20090406 LR - 20211020 IS - 1432-1289 (Electronic) IS - 0020-9554 (Linking) VI - 49 IP - 3 DP - 2008 Mar TI - [Digital ischemia of a patient with undifferentiated large cell lung cancer]. PG - 346-8 LID - 10.1007/s00108-007-2008-5 [doi] AB - A Raynaud's phenomenon with acral ischemia till necrosis is a rare form of a paraneoplasia. We report about a woman with a metastatic lung cancer, who was admitted because of pain in her left hand with necrosis of the fingertips and of the fifth right toe. We started a treatment with the intravenous vasodilator Iloprost, but the necroses progressed to gangrene. The course of a paraneoplastic syndrome is often determined by the underlying malignoma and if the antineoplastic treatment is ineffective, the chances to treat the paraneoplasia may be limited. FAU - Schmid, I AU - Schmid I AD - Stadtspital Triemli, Zürich. FAU - Fliegner, M AU - Fliegner M LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Digitale Ischämien bei einer Patientin mit grosszelligem Lungenkarzinom. PL - Germany TA - Internist (Berl) JT - Der Internist JID - 0264620 SB - IM MH - Carcinoma, Large Cell/*complications/*diagnosis/therapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung Neoplasms/*diagnosis/therapy MH - Middle Aged MH - Paraneoplastic Syndromes/*diagnosis/therapy MH - Raynaud Disease/*diagnosis/*etiology/therapy EDAT- 2008/01/25 09:00 MHDA- 2009/04/07 09:00 CRDT- 2008/01/25 09:00 PHST- 2008/01/25 09:00 [pubmed] PHST- 2009/04/07 09:00 [medline] PHST- 2008/01/25 09:00 [entrez] AID - 10.1007/s00108-007-2008-5 [doi] PST - ppublish SO - Internist (Berl). 2008 Mar;49(3):346-8. doi: 10.1007/s00108-007-2008-5. PMID- 21400722 OWN - NLM STAT- MEDLINE DCOM- 20110609 LR - 20161020 IS - 0869-6047 (Print) IS - 0869-6047 (Linking) IP - 1 DP - 2011 TI - [The experience with thoracoscopic sympathectomy for the treatment of distal lesions of limb arteries]. PG - 18-22 AB - The study included 107 patients with distal lesions of limb arteries treated with the use of thoracoscopic sympathectomy. The best results in the early postoperative period were obtained in patients with Raynaud's disease. Modifications introduced into the methods of its treatment permitted to retain effectiveness of sympathectomy till the late postoperative period in 90% of the patients. Surgery for obliterative endarteritis and atherosclerosis was viewed as a possibility to preserve the supporting function of the limb. This purpose was attained in 73.2 and 62.5% of the patients respectively in the early and in 62 and 25% in the late postoperative periods. FAU - Karimov, Sh I AU - Karimov ShI FAU - Berkinov, U B AU - Berkinov UB FAU - Krotov, N F AU - Krotov NF FAU - Ganiev, D A AU - Ganiev DA LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Vestn Ross Akad Med Nauk JT - Vestnik Rossiiskoi akademii meditsinskikh nauk JID - 9215641 SB - IM MH - Arteriosclerosis Obliterans/diagnosis/etiology/physiopathology/*surgery MH - Endarteritis/diagnosis/etiology/physiopathology/*surgery MH - Extremities/blood supply MH - Ganglionectomy/adverse effects/standards MH - Humans MH - Raynaud Disease/diagnosis/physiopathology/*surgery MH - Risk Factors MH - Secondary Prevention MH - *Sympathectomy/adverse effects/methods/standards MH - Thoracic Nerves/surgery MH - Thoracoscopy/*methods MH - Time MH - Transcutaneous Electric Nerve Stimulation MH - Treatment Outcome EDAT- 2011/03/15 06:00 MHDA- 2011/06/10 06:00 CRDT- 2011/03/15 06:00 PHST- 2011/03/15 06:00 [entrez] PHST- 2011/03/15 06:00 [pubmed] PHST- 2011/06/10 06:00 [medline] PST - ppublish SO - Vestn Ross Akad Med Nauk. 2011;(1):18-22. PMID- 21780632 OWN - NLM STAT- MEDLINE DCOM- 20110809 LR - 20110725 IS - 1027-6661 (Print) IS - 1027-6661 (Linking) VI - 17 IP - 1 DP - 2010 TI - [Experience with thoracoscopic sympathectomy in distal lesions of lower-limb arteries]. PG - 131-5 AB - We studied the results outcomes of thoracoscopic sympathectomy in 107patients with distal lesions of lower-limb arteries. The best results in the early postoperative period were observed in patients with Raynaud's disease and syndrome, reaching 100% efficacy. Due to implementation of novelties, we managed to preserve the efficiency of sympathectomy in 90% of patients with this pathology in the remote period. In patients with obliterating endarteritis and atherosclerosis, the efficacy of the operation was regarded as a possibility to preserve the supporting function of the extremity. In the postoperative period this goal was achieved in 73.2% and 62.5% of patients, respectively, while in the remote period in 62% and 25%, respectively. FAU - Karimov, Sh I AU - Karimov ShI FAU - Berkinov, U B AU - Berkinov UB FAU - Krotov, N F AU - Krotov NF FAU - Ganiev, D A AU - Ganiev DA LA - rus PT - Comparative Study PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Angiol Sosud Khir JT - Angiologiia i sosudistaia khirurgiia = Angiology and vascular surgery JID - 9604504 SB - IM MH - Adolescent MH - Adult MH - Arteries/*innervation MH - Arteriosclerosis Obliterans/*surgery MH - Female MH - Follow-Up Studies MH - Humans MH - Leg/*blood supply MH - Male MH - Middle Aged MH - Raynaud Disease/*surgery MH - Retrospective Studies MH - Sympathectomy/*methods MH - Thoracoscopy/*methods MH - Thromboangiitis Obliterans/*surgery MH - Treatment Outcome MH - Young Adult EDAT- 2010/01/01 00:00 MHDA- 2011/08/10 06:00 CRDT- 2011/07/26 06:00 PHST- 2011/07/26 06:00 [entrez] PHST- 2010/01/01 00:00 [pubmed] PHST- 2011/08/10 06:00 [medline] PST - ppublish SO - Angiol Sosud Khir. 2010;17(1):131-5. PMID- 20954308 OWN - NLM STAT- MEDLINE DCOM- 20101215 LR - 20101015 IS - 0374-1338 (Print) IS - 0374-1338 (Linking) VI - 56 IP - 1 DP - 2009 TI - [Capillaroscopy--an insufficiently known and underused method in rheumatology]. PG - 41-4 AB - Capillaroscopy is a noninvasive and harmless morphological method for examination of the nailfold capillaries in both suspected and already diagnosed patients with systemic connective disease. The most useful aspect is in discrimination between the patients with primary and secondary Raynaud's phenomenon. Many of the mentioned features should make capillaroscopy a part of the diagnostic algorithm and follow-up in everyday rheumatologic practice. FAU - Baresić, Marko AU - Baresić M AD - Zavod za klinicku imunologiju i reumatologiju, Klinika za unutarnje bolesti, Klinicki bolnicki centar Zagreb, Kispatićeva 12, 10000 Zagreb. markobaresic@gmail.com FAU - Anić, Branimir AU - Anić B LA - hrv PT - English Abstract PT - Journal Article TT - Kapilaroskopija--nedovoljno poznata i nedovoljno iskoristena metoda u reumatologiji. PL - Croatia TA - Reumatizam JT - Reumatizam JID - 0216650 SB - IM MH - Humans MH - *Microscopic Angioscopy MH - Raynaud Disease/diagnosis MH - Rheumatic Diseases/*diagnosis MH - Scleroderma, Systemic/diagnosis EDAT- 2009/01/01 00:00 MHDA- 2010/12/16 06:00 CRDT- 2010/10/20 06:00 PHST- 2010/10/20 06:00 [entrez] PHST- 2009/01/01 00:00 [pubmed] PHST- 2010/12/16 06:00 [medline] PST - ppublish SO - Reumatizam. 2009;56(1):41-4. PMID- 18320797 OWN - NLM STAT- MEDLINE DCOM- 20080508 LR - 20151026 IS - 2067-2993 (Print) IS - 2067-2993 (Linking) VI - 56 IP - 4 DP - 2007 Oct-Dec TI - [Thoracoscopic sympathectomy for arterial ischemia of the upper extremities: a case report]. PG - 208-10 AB - INTRODUCTION: Thoracoscopic surgery reduce the morbidity of sympathectomy. Major indications of video-assisted sympathectomy (VAT) include hyperhidrosis, Raynaud's disease, causalgia, and reflex sympathetic dystrophy. Because little information is available in the national and international literature VAT sympathectomy in the treatment of upper extremities ischemia, we decided to present our first case. CLINIC CASE: The 38 years old patient was hospitalized for left upper-extremity ischemia (Raynaud's syndrome). Thoracoscopic sympathectomy was performed with resection of the main trunk proximally immediately after the stellate ganglion and distally at the level of T4 and identification and resection of 2 collateral branches of the sympathetic chain T2-T3. RESULTS: The postoperative evolution demonstrated evident clinical benefit. The thermography performed postoperative showed hyperthermia and hyper-vascularization in the left hemithorax with a difference of temperature of 2 degrees C between the two sides of the thorax. COMMENT: Before the advent of VAT, thoracic sympathectomy was performed only in highly selected patients because of its invasiveness. Now VAT sympathectomy is considered in most cases as the last resort to prevent extensive and successive amputation. Because the procedure is minimally invasive, safe, and associated with a low rate of complications, it should be considered earlier in the natural course of this disease. FAU - Paleru, Cristian AU - Paleru C AD - Clinica I Chirurgie Toracică, Institutul National de Pneumoftiziologie Marius Nasta Bucureşti. cpaleru@yahoo.com FAU - Dănăilă, Olga AU - Dănăilă O FAU - Bordîncă, Ioana AU - Bordîncă I FAU - Istrate, Adrian AU - Istrate A FAU - Bolca, Ciprian AU - Bolca C FAU - Cordoş, Ion AU - Cordoş I LA - rum PT - Case Reports PT - English Abstract PT - Journal Article TT - Simpatectomia toracoscopică în tratamentul ischemiei arteriale a membrului superior: prezentare de caz. PL - Romania TA - Pneumologia JT - Pneumologia (Bucharest, Romania) JID - 100941067 SB - IM MH - Adult MH - Humans MH - Ischemia/*surgery MH - Male MH - Raynaud Disease/*surgery MH - Sympathectomy/*methods MH - *Thoracic Surgery, Video-Assisted MH - Treatment Outcome MH - Upper Extremity/*blood supply EDAT- 2008/03/07 09:00 MHDA- 2008/05/09 09:00 CRDT- 2008/03/07 09:00 PHST- 2008/03/07 09:00 [pubmed] PHST- 2008/05/09 09:00 [medline] PHST- 2008/03/07 09:00 [entrez] PST - ppublish SO - Pneumologia. 2007 Oct-Dec;56(4):208-10. PMID- 21120563 OWN - NLM STAT- MEDLINE DCOM- 20110406 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 30 IP - 1 DP - 2011 Jan TI - Prevalence of Raynaud's phenomenon in young Greek males. PG - 57-9 LID - 10.1007/s10067-010-1621-1 [doi] AB - The aim of this paper is to study the prevalence of Raynaud's phenomenon in young males. Young males were examined prospectively in a district hospital, and laboratory tests were performed on the basis of the clinical history. Young males (3.912), age 18-28 years old, were examined. Raynaud's phenomenon was present in seven men (1,79 per 1.000, 95% CI 0.72-3.68). Three of them had at least one member in their family diagnosed with Raynaud's phenomenon. Three were smokers. All had negative immunological test. Five patients reported having severe attacks and two had only mild ischemic attacks. The treatment was conservative for all patients, two of them necessitated vasodilators. Very low prevalence of Raynaud's phenomenon was observed in this young male group compared with the previous studies. FAU - Tzilalis, Vassilios AU - Tzilalis V AD - Department of Vascular Surgery, 401 General Military Hospital of Athens, Athens, Greece. FAU - Panagiotopoulos, Nikolaos AU - Panagiotopoulos N FAU - Papatheodorou, George AU - Papatheodorou G FAU - Rallis, Efstathios AU - Rallis E FAU - Kassimos, Dimitrios AU - Kassimos D LA - eng PT - Journal Article DEP - 20101201 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Vasodilator Agents) SB - IM MH - Adolescent MH - Adult MH - Greece MH - Humans MH - Ischemia MH - Male MH - Prevalence MH - Raynaud Disease/*epidemiology MH - Rheumatology/methods MH - Smoking MH - Vasodilator Agents/therapeutic use EDAT- 2010/12/02 06:00 MHDA- 2011/04/07 06:00 CRDT- 2010/12/02 06:00 PHST- 2010/08/02 00:00 [received] PHST- 2010/11/03 00:00 [accepted] PHST- 2010/10/30 00:00 [revised] PHST- 2010/12/02 06:00 [entrez] PHST- 2010/12/02 06:00 [pubmed] PHST- 2011/04/07 06:00 [medline] AID - 10.1007/s10067-010-1621-1 [doi] PST - ppublish SO - Clin Rheumatol. 2011 Jan;30(1):57-9. doi: 10.1007/s10067-010-1621-1. Epub 2010 Dec 1. PMID- 16308344 OWN - NLM STAT- MEDLINE DCOM- 20060717 LR - 20250214 IS - 0003-4967 (Print) IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 65 IP - 7 DP - 2006 Jul TI - Low plasma protein nitrotyrosine levels distinguish primary Raynaud's phenomenon from scleroderma. PG - 952-4 AB - OBJECTIVE: To investigate the hypothesis that increased formation of reactive nitrogen species may contribute to the vascular pathology that develops in patients with connective tissue disease such as scleroderma. PATIENTS AND METHODS: The level of protein-bound nitrotyrosine in plasma was measured by stable isotope dilution gas chromatography/negative ion chemical ionisation mass spectrometry in 11 patients with primary Raynaud's phenomenon, 37 with scleroderma, 13 with chronic renal impairment, and in 23 healthy controls. RESULTS: Plasma protein-bound nitrotyrosine was markedly decreased in patients with primary Raynaud's phenomenon (mean (SEM) 0.60 (0.06) ng/mg dry protein) compared with patients with scleroderma (1.78 (0.21) ng/mg protein), chronic renal impairment (1.42 (0.17) ng/mg protein) or healthy controls (1.63+/-0.15 ng/mg protein, ANOVA p<0.001). CONCLUSION: These data suggest that there is decreased nitration of plasma proteins, or increased degradation of nitrated proteins from the circulation of patients with primary but not secondary Raynaud's phenomenon. FAU - Kingdon, E J AU - Kingdon EJ AD - Royal Free and University College Medical School, University College London (UCL), Rowland Hill Street, London NW3 2PF, UK. FAU - Mani, A R AU - Mani AR FAU - Frost, M T AU - Frost MT FAU - Denton, C P AU - Denton CP FAU - Powis, S H AU - Powis SH FAU - Black, C M AU - Black CM FAU - Moore, K P AU - Moore KP LA - eng PT - Comparative Study PT - Journal Article DEP - 20051124 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Biomarkers) RN - 0 (Blood Proteins) RN - 3604-79-3 (3-nitrotyrosine) RN - 42HK56048U (Tyrosine) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Analysis of Variance MH - Biomarkers/blood MH - Blood Proteins/*chemistry MH - Chromatography, Gas MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/blood/*diagnosis MH - Scleroderma, Systemic/blood/*diagnosis MH - Tyrosine/*analogs & derivatives/blood PMC - PMC1798203 EDAT- 2005/11/26 09:00 MHDA- 2006/07/18 09:00 PMCR- 2009/07/01 CRDT- 2005/11/26 09:00 PHST- 2005/11/26 09:00 [pubmed] PHST- 2006/07/18 09:00 [medline] PHST- 2005/11/26 09:00 [entrez] PHST- 2009/07/01 00:00 [pmc-release] AID - S0003-4967(24)20130-8 [pii] AID - ar43562 [pii] AID - 10.1136/ard.2005.043562 [doi] PST - ppublish SO - Ann Rheum Dis. 2006 Jul;65(7):952-4. doi: 10.1136/ard.2005.043562. Epub 2005 Nov 24. PMID- 23256110 OWN - NLM STAT- MEDLINE DCOM- 20140303 LR - 20181023 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 64 IP - 5 DP - 2012 Dec 11 TI - Coexistence of osteopoikilosis with seronegative spondyloarthritis and Raynaud's phenomenon: first case report with evaluation of the nailfold capillary bed and literature review. PG - 335-9 LID - 10.4081/reumatismo.2012.335 [doi] AB - Osteopoikilosis (OPK) is a rare autosomal dominant bone disorder characterized by numerous hyperostotic areas that tend to localize in periarticular osseous regions. It is usually asymptomatic and is often diagnosed incidentally during X-rays. OPK may be an isolated finding or associated with other pathologies, e.g. skin manifestations, rheumatic and/or skeletal disorders. We report a literature review and, for the first time, the coexistence of OPK with seronegative spondyloarthritis and Raynaud's phenomenon in a 48-year old female. To the best of our knowledge, this is the first case of OPK studied by videocapillaroscopy, demonstrating the absence of specific microvascular abnormalities of nailfold capillaries. FAU - Ruaro, B AU - Ruaro B AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy. FAU - Sulli, A AU - Sulli A FAU - Alessandri, E AU - Alessandri E FAU - Ravera, F AU - Ravera F FAU - Cutolo, M AU - Cutolo M LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20121211 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Arthroplasty, Replacement, Hip MH - Blood Sedimentation MH - C-Reactive Protein/analysis MH - Capillaries/pathology MH - Female MH - Humans MH - *Microscopic Angioscopy/methods MH - *Microscopy, Video MH - Middle Aged MH - Nails/*blood supply MH - Osteoarthritis, Hip/complications/diagnostic imaging/surgery MH - Osteopoikilosis/blood/*complications/diagnostic imaging MH - Pelvic Bones/diagnostic imaging MH - Radiography MH - Raynaud Disease/*complications/pathology MH - Sacroiliitis/complications/diagnostic imaging MH - Spondylarthritis/blood/*complications/diagnostic imaging EDAT- 2012/12/21 06:00 MHDA- 2014/03/04 06:00 CRDT- 2012/12/21 06:00 PHST- 2012/04/26 00:00 [received] PHST- 2012/07/02 00:00 [accepted] PHST- 2012/06/28 00:00 [revised] PHST- 2012/12/21 06:00 [entrez] PHST- 2012/12/21 06:00 [pubmed] PHST- 2014/03/04 06:00 [medline] AID - reumatismo.2012.335 [pii] AID - 10.4081/reumatismo.2012.335 [doi] PST - epublish SO - Reumatismo. 2012 Dec 11;64(5):335-9. doi: 10.4081/reumatismo.2012.335. PMID- 28957564 OWN - NLM STAT- MEDLINE DCOM- 20171006 LR - 20191210 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 56 IP - 10 DP - 2017 Oct 1 TI - Reproducibility of the scleroderma pattern assessed by wide-field capillaroscopy in subjects suffering from Raynaud's phenomenon. PG - 1780-1783 LID - 10.1093/rheumatology/kex282 [doi] AB - OBJECTIVES: The aim of this work was to study inter- and intra-observer agreement for the diagnosis of scleroderma pattern by wide-field capillaroscopy. METHODS: Images were taken from 50 patients known to have SSc and 50 controls consulting for RP who did not have SSc. These images were rated simultaneously by 11 experienced vascular medicine physicians as scleroderma pattern or not. Two weeks later, 7 of the 11 observers again rated the same images. RESULTS: Inter-observer agreement was almost perfect between the 11 observers (κ 0.86 ± 0.01), and the proportion of concordant observations was 79% (70-87). When each observer was compared with the reference, agreement was also almost perfect: κ coefficient 0.92 ± 0.03 and proportion of concordant observations 79% (70-87). Intra-observer agreement was also almost perfect: median κ coefficient 0.94 (0.78-0.96) and median proportion of concordant observations 97% (89-98). CONCLUSION: Excellent inter- and intra-observer agreement was obtained in experienced vascular physicians for the diagnosis of capillaroscopic landscape by wide-field nailfold capillary microscopy. CI - © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com FAU - Boulon, Carine AU - Boulon C AD - Service de Médecine Vasculaire, Hôpital Saint-André, Bordeaux. FAU - Blaise, Sophie AU - Blaise S AD - Service de Médecine Vasculaire, CHU de Grenoble-Alpes, La Tronche. FAU - Lazareth, Isabelle AU - Lazareth I AD - Service de Médecine Vasculaire, Hôpital Saint Joseph, Paris. FAU - Le Hello, Claire AU - Le Hello C AD - Service de Médecine Vasculaire, CHU de Saint-Etienne, Hôpital Nord, Saint-Etienne. FAU - Pistorius, Marc-Antoine AU - Pistorius MA AD - Service de Médecine Vasculaire, Hôtel Dieu, Nantes. FAU - Imbert, Bernard AU - Imbert B AD - Service de Médecine Vasculaire, CHU de Grenoble-Alpes, La Tronche. FAU - Mangin, Marion AU - Mangin M AD - Service de Médecine Vasculaire, Hôpital Saint-André, Bordeaux. FAU - Sintes, Pierre AU - Sintes P AD - Médecine Vasculaire, Saint Cloud. FAU - Senet, Patricia AU - Senet P AD - Service de Dermatologie, Médecine Vasculaire et Allergologie, Hôpital Tenon. FAU - Decamps-Le Chevoir, Joëlle AU - Decamps-Le Chevoir J AD - Service de Médecine Interne, Hôpital de la Pitié, Paris, France. FAU - Tribout, Laurent AU - Tribout L AD - Service de Dermatologie, Médecine Vasculaire et Allergologie, Hôpital Tenon. FAU - Carpentier, Patrick AU - Carpentier P AD - Service de Médecine Vasculaire, CHU de Grenoble-Alpes, La Tronche. FAU - Constans, Joël AU - Constans J AD - Service de Médecine Vasculaire, Hôpital Saint-André, Bordeaux. LA - eng PT - Journal Article PT - Multicenter Study PT - Validation Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2018 May 1;57(5):940-941. doi: 10.1093/rheumatology/kex482. PMID: 29390117 CIN - Rheumatology (Oxford). 2018 May 1;57(5):941-942. doi: 10.1093/rheumatology/kex483. PMID: 29390130 MH - Adult MH - Case-Control Studies MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/methods/*statistics & numerical data MH - Nails/blood supply/diagnostic imaging MH - Observer Variation MH - Raynaud Disease/etiology/*physiopathology MH - Reproducibility of Results MH - Scleroderma, Systemic/complications/*diagnosis OTO - NOTNLM OT - Raynaud’s phenomenon OT - nailfold capillaroscopy OT - reproducibility OT - systemic sclerosis EDAT- 2017/09/29 06:00 MHDA- 2017/10/07 06:00 CRDT- 2017/09/29 06:00 PHST- 2017/04/05 00:00 [received] PHST- 2017/09/29 06:00 [entrez] PHST- 2017/09/29 06:00 [pubmed] PHST- 2017/10/07 06:00 [medline] AID - 4049643 [pii] AID - 10.1093/rheumatology/kex282 [doi] PST - ppublish SO - Rheumatology (Oxford). 2017 Oct 1;56(10):1780-1783. doi: 10.1093/rheumatology/kex282. PMID- 19531744 OWN - NLM STAT- MEDLINE DCOM- 20091008 LR - 20220410 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 8 DP - 2009 Aug TI - Diagnosis of Raynaud's phenomenon by (99m)Tc-hydroxymethylene diphosphonate digital blood flow scintigraphy after one-hand chilling. PG - 1663-70 LID - 10.3899/jrheum.080988 [doi] AB - OBJECTIVE: We introduce the use of (99m)Tc-hydroxymethylene diphosphonate (HDP) digital blood flow scintigraphy to diagnose Raynaud's phenomenon (RP). METHODS: Fifty-seven patients with RP and 60 healthy controls were recruited. One hand was chilled by immersion into water at 4 degrees C, and then an intravenous bolus of 740 MBq of (99m)Tc-HDP was injected. The radioactivity from the second to the fifth fingers of both hands was recorded. Acquisition was performed at a rate of one frame per 2 seconds until 155 frames. We calculated 4 ratios by comparing the activity curves of the chilled hand with those of the ambient hand. RESULTS: The chilled to ambient hand ratio of the initial slope was significantly lower in the patients with RP (0.28 +/- 0.18) than in the controls (0.78 +/- 0.20) (p < 0.001). The chilled to ambient hand ratio of the first peak height, 30-second area under the curve, and blood pool uptake were also lower in the patients with RP than in controls (p < 0.001 for each). The initial slope ratio of 0.51, used as a cutoff value, showed a sensitivity of 91.2% and specificity of 93.3%. The loss of the initial spike curve, the presence of a slowly progressing radioactivity curve, and the inhomogeneous radioactivity uptake in the blood pool image in either hand were characteristic findings of the patients with RP (p < 0.001). CONCLUSION: (99m)Tc-HDP digital blood flow scintigraphy after one-hand chilling is a noninvasive, accurate, and quantitative method to evaluate RP. FAU - Kwon, Seong-Ryul AU - Kwon SR AD - Division of Rheumatology, Department of Internal Medicine; Occupational and Environmental Medicine; and Nuclear Medicine, Inha University Hospital, Incheon, Republic of Korea. FAU - Lim, Mie-Jin AU - Lim MJ FAU - Park, Shin-Goo AU - Park SG FAU - Hyun, In-Young AU - Hyun IY FAU - Park, Won AU - Park W LA - eng PT - Controlled Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090616 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Autoantibodies) RN - X89XV46R07 (Technetium Tc 99m Medronate) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoantibodies/blood MH - *Cold Temperature MH - Female MH - Fingers/*blood supply MH - Gated Blood-Pool Imaging/*methods MH - Humans MH - Immersion MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnostic imaging/immunology/physiopathology MH - Regional Blood Flow MH - *Technetium Tc 99m Medronate MH - Young Adult EDAT- 2009/06/18 09:00 MHDA- 2009/10/09 06:00 CRDT- 2009/06/18 09:00 PHST- 2009/06/18 09:00 [entrez] PHST- 2009/06/18 09:00 [pubmed] PHST- 2009/10/09 06:00 [medline] AID - jrheum.080988 [pii] AID - 10.3899/jrheum.080988 [doi] PST - ppublish SO - J Rheumatol. 2009 Aug;36(8):1663-70. doi: 10.3899/jrheum.080988. Epub 2009 Jun 16. PMID- 16391889 OWN - NLM STAT- MEDLINE DCOM- 20070109 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 6 DP - 2006 Nov TI - The usefulness of power Doppler ultrasonography in differentiating primary and secondary Raynaud's phenomenon. PG - 814-8 AB - The objectives of this study were to assess a usefulness of power Doppler ultrasonography (PDU) and compare the diagnostic value of PDU to nailfold capillaroscopy (NFC) in the patients with clinically diagnosed Raynaud's phenomenon (RP) and healthy controls. Forty-one patients with primary (n=19), secondary RP (n=22), and ten healthy controls underwent PDU and NFC examinations on the same day. Microvascularity was evaluated using PDU before and after cold challenges, and the PDU signals were qualitatively graded on a scale of 1-4. According to the change of microvascularity before and after cold challenges, the findings of PDU were classified into three groups: (1) 'pattern I' (normal microvascularity over grade 3 both before and after cold challenges), (2) 'pattern II' (decreased microvascularity to grade 1 or 2 only after cold challenge), and (3) 'pattern III' (decreased microvascularity of grade 1 or 2 both before and after cold challenges). PDU confirmed the presence of RP in all patients with clinically diagnosed RP and yielded a correct classification in 88.9% of the all persons analyzed (normal=100%, primary RP=89.5%, secondary RP=77.3%). The analysis was performed to assess the degree of agreement between the final diagnoses obtained by PDU and NFC. A good correlation rate was observed between PDU and NFC examinations in differentiating primary from secondary RP (Kappa=0.658, p<0.01). In conclusion, PDU examination with a cold challenge is a useful and reliable method to diagnose RP and discriminate between primary and secondary RP. FAU - Lee, Sang Il AU - Lee SI AD - Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School, and Research Institute of Clinical Medicine, San 2-20, Geumam-dong, Deokjin-gu, Jeonju, Jeonbuk, 561-180, Republic of Korea. goldgu@chonbuk.ac.kr FAU - Lee, Sang Yong AU - Lee SY FAU - Yoo, Wan Hee AU - Yoo WH LA - eng PT - Controlled Clinical Trial PT - Journal Article DEP - 20060104 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Autonomic Nervous System Diseases/*complications MH - Cold Temperature MH - Connective Tissue Diseases/*complications MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/blood supply/pathology MH - Raynaud Disease/*diagnostic imaging/*etiology/pathology MH - *Ultrasonography, Doppler EDAT- 2006/01/05 09:00 MHDA- 2007/01/11 09:00 CRDT- 2006/01/05 09:00 PHST- 2005/10/03 00:00 [received] PHST- 2005/11/07 00:00 [accepted] PHST- 2005/10/28 00:00 [revised] PHST- 2006/01/05 09:00 [pubmed] PHST- 2007/01/11 09:00 [medline] PHST- 2006/01/05 09:00 [entrez] AID - 10.1007/s10067-005-0167-0 [doi] PST - ppublish SO - Clin Rheumatol. 2006 Nov;25(6):814-8. doi: 10.1007/s10067-005-0167-0. Epub 2006 Jan 4. PMID- 29390117 OWN - NLM STAT- MEDLINE DCOM- 20180507 LR - 20181202 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 57 IP - 5 DP - 2018 May 1 TI - Comment on: Reproducibility of the scleroderma pattern assessed by wide-field capillaroscopy in subjects suffering from Raynaud's phenomenon. PG - 940-941 LID - 10.1093/rheumatology/kex482 [doi] FAU - Sabour, Siamak AU - Sabour S AD - Department of Clinical Epidemiology, Shahid Beheshti University of Medical Sciences, Tehran, I.R. Iran. AD - Safety Promotions and Injury Prevention Research Center, Shahid Beheshti University of Medical Sciences, Tehran, I.R. Iran. LA - eng PT - Comment PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2017 Oct 1;56(10):1780-1783. doi: 10.1093/rheumatology/kex282. PMID: 28957564 CIN - Rheumatology (Oxford). 2018 May 1;57(5):941-942. doi: 10.1093/rheumatology/kex483. PMID: 29390130 MH - Humans MH - *Microscopic Angioscopy MH - Nails MH - *Raynaud Disease MH - Reproducibility of Results MH - Scleroderma, Localized MH - Scleroderma, Systemic EDAT- 2018/02/02 06:00 MHDA- 2018/05/08 06:00 CRDT- 2018/02/02 06:00 PHST- 2017/10/07 00:00 [received] PHST- 2017/11/08 00:00 [accepted] PHST- 2018/02/02 06:00 [pubmed] PHST- 2018/05/08 06:00 [medline] PHST- 2018/02/02 06:00 [entrez] AID - 4829711 [pii] AID - 10.1093/rheumatology/kex482 [doi] PST - ppublish SO - Rheumatology (Oxford). 2018 May 1;57(5):940-941. doi: 10.1093/rheumatology/kex482. PMID- 18843783 OWN - NLM STAT- MEDLINE DCOM- 20090324 LR - 20191111 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 12 DP - 2008 Dec TI - The temporal relationship of Raynaud's phenomenon and features of connective tissue disease in rheumatoid arthritis. PG - 2329-33 AB - OBJECTIVE: In a prospective cohort study we examined the relationship between Raynaud's phenomenon (RP) onset and other connective tissue disease (CTD) characteristics in rheumatoid arthritis (RA) to determine if RP is predictive of RA severity and associated with other CTD signs, and if late onset RP in RA has an effect on prognosis compared to other patients with RA. METHODS: Using a standardized assessment, data were collected on 328 subjects with RA [mean age 60.3 +/- 0.7; 77% women; 76% erosions, 75% positive rheumatoid factor (RF)] seen at one London, Ontario, rheumatology clinic. The data included RA disease duration; presence and duration of RP; presence of nodules, joint damage, telangiectasia, and sclerodactyly; and RF status (+/-), RF value, antinuclear antibodies, and E-nuclear antibodies. RESULTS: The mean RA disease duration was 12 +/- 0.6 years. Seventy-one (22%) had RP and the mean RP duration was 9.2 +/- 1.5 years. Patients presented with RP a mean of 3.8 +/- 1.4 years after the diagnosis of RA. RP status was positively associated with the presence of sclerodactyly (p < 0.001), but not nodules or erosions. Higher RF values were associated with longer RA disease duration (p < 0.002) and longer RP duration (p < 0.01). CONCLUSION: Idiopathic RP may have a different clinical effect on RA than secondary RP; the latter is correlated with more severe RA. Sclerodactyly is associated with erosive arthritis and RP in RA. Higher RF values were indicative of increased RA and RP duration. FAU - Pope, Janet E AU - Pope JE AD - Division of Rheumatology, Department of Medicine, Schulich School of Medicine and Dentistry, The University of Western Ontario, London, Ontario, Canada. janet.pope@sjhc.london.on.ca FAU - Al-Bishri, Jamal AU - Al-Bishri J FAU - Al-Azem, Hafsah AU - Al-Azem H FAU - Ouimet, Janine M AU - Ouimet JM LA - eng PT - Journal Article DEP - 20081001 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Arthritis, Rheumatoid/*complications/*physiopathology MH - Connective Tissue Diseases/complications/physiopathology MH - Disease Progression MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/*complications/*physiopathology EDAT- 2008/10/10 09:00 MHDA- 2009/03/25 09:00 CRDT- 2008/10/10 09:00 PHST- 2008/10/10 09:00 [pubmed] PHST- 2009/03/25 09:00 [medline] PHST- 2008/10/10 09:00 [entrez] AID - 08/13/104 [pii] AID - 10.3899/jrheum.071025 [doi] PST - ppublish SO - J Rheumatol. 2008 Dec;35(12):2329-33. doi: 10.3899/jrheum.071025. Epub 2008 Oct 1. PMID- 16804700 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20191210 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 4 DP - 2006 Jul TI - [Systemic sclerosis - diagnosis and classification]. PG - 268-74 AB - Systemic sclerosis (SSc) is a polymorphic and heterogenic systemic disorder with inflammation, fibrosis and vascular damage. Early diagnosis and classification may be difficult if disease expression is oligosymptomatic (undifferentiated), presenting with only Raynaud's phenomenon or limited scleroderma. Scleroderma specific antinuclear autoantibodies, which are present early and persistently in about 90% of the patients with SSc, play an important taxonomic role. Scleroderma specific findings in nailfold capillary microscopy are sensitive and predictive for evolving SSc. An algorithm will be presented for the diagnosis and classification of SSc using clinical, capillaroscopic and serologic criteria, which are also useful for mixed or special forms of SSc. The 6th Outcome Measures in Rheumatology Clinical Trials (OMERACT) conference proposed different outcome measurements for clinical studies, however, for daily clinical practice there is as yet no consensus on status indices for disease activity, disease related damage or suitable prognostic criteria. FAU - Genth, E AU - Genth E AD - Rheumaklinik und Rheumaforschungsinstitut Aachen, Burtscheider Markt 24, 52066, Aachen, Germany. mail@ekkehard-genth.de FAU - Krieg, T AU - Krieg T LA - ger PT - Journal Article PT - Review TT - Systemische Sklerose - Diagnose und Klassifikation. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Algorithms MH - Antibodies, Antinuclear/blood MH - Diagnosis, Differential MH - Humans MH - Microscopic Angioscopy MH - Outcome Assessment, Health Care MH - Prognosis MH - Raynaud Disease/classification/diagnosis MH - Scleroderma, Systemic/classification/*diagnosis RF - 37 EDAT- 2006/06/29 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/06/29 09:00 PHST- 2006/06/29 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/06/29 09:00 [entrez] AID - 10.1007/s00393-006-0065-0 [doi] PST - ppublish SO - Z Rheumatol. 2006 Jul;65(4):268-74. doi: 10.1007/s00393-006-0065-0. PMID- 25890839 OWN - NLM STAT- MEDLINE DCOM- 20160629 LR - 20161018 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 36 IP - 9 DP - 2015 Sep TI - [Periungueal capillaroscopy: an easy and reliable method to evaluate all microcirculation diseases]. PG - 603-12 LID - S0248-8663(15)00114-9 [pii] LID - 10.1016/j.revmed.2015.03.015 [doi] AB - Periungueal capillaroscopy is a simple and reliable non-invasive technique allowing evaluation of cutaneous microcirculation. It was promoted for decades in patients with Raynaud's phenomenon in order to differentiate between the benign primary Raynaud's phenomenon and the secondary form in connective tissue diseases, especially systemic sclerosis. Nevertheless, the value of this procedure has also been shown in numerous pathologies such as diabetes or cardiovascular diseases. This literature review points to the versatility of this useful exam and its results in a large spectrum of diseases with microvascular involvement. CI - Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved. FAU - Jammal, M AU - Jammal M AD - Service de médecine interne, Hôtel Dieu de France, Beyrouth, Liban. Electronic address: mouinjammal@yahoo.fr. FAU - Kettaneh, A AU - Kettaneh A AD - Service de médecine interne, hôpital privé de Vitry, 94400 Paris, France. FAU - Cabane, J AU - Cabane J AD - Service de médecine interne, hôpital Saint-Antoine, 94400 Paris, France. FAU - Tiev, K AU - Tiev K AD - Service de médecine interne, hôpital privé de Vitry, 94400 Paris, France. FAU - Toledano, C AU - Toledano C AD - Service de médecine interne, hôpital privé de Vitry, 94400 Paris, France. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Capillaroscopie périunguéale : une évaluation simple et fiable de toute pathologie de la microcirculation. DEP - 20150415 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Connective Tissue Diseases/diagnosis MH - Diagnosis, Differential MH - Fingers/blood supply/pathology MH - Humans MH - *Microcirculation MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply/pathology MH - Raynaud Disease/diagnosis MH - Reproducibility of Results MH - Scleroderma, Systemic/diagnosis MH - Vascular Diseases/*diagnosis/pathology OTO - NOTNLM OT - Capillaroscopie OT - Capillaroscopy OT - Microcirculation OT - Phénomène de Raynaud OT - Raynaud's phenomenon EDAT- 2015/04/22 06:00 MHDA- 2016/06/30 06:00 CRDT- 2015/04/20 06:00 PHST- 2014/04/01 00:00 [received] PHST- 2014/11/24 00:00 [revised] PHST- 2015/03/13 00:00 [accepted] PHST- 2015/04/20 06:00 [entrez] PHST- 2015/04/22 06:00 [pubmed] PHST- 2016/06/30 06:00 [medline] AID - S0248-8663(15)00114-9 [pii] AID - 10.1016/j.revmed.2015.03.015 [doi] PST - ppublish SO - Rev Med Interne. 2015 Sep;36(9):603-12. doi: 10.1016/j.revmed.2015.03.015. Epub 2015 Apr 15. PMID- 37280057 OWN - NLM STAT- MEDLINE DCOM- 20240105 LR - 20241119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 1 DP - 2024 Jan 4 TI - Raynaud's phenomenon and digital ischaemia induced by ixekizumab. PG - e3-e5 LID - 10.1093/rheumatology/kead270 [doi] FAU - Paredes-Romero, M Beatriz AU - Paredes-Romero MB AUID- ORCID: 0000-0002-0911-1810 AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. AD - Universidad Europea de Madrid, Madrid, Spain. FAU - Castañeda-Estévez, Elisabet AU - Castañeda-Estévez E AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. FAU - Steiner, Martina AU - Steiner M AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. AD - Universidad Europea de Madrid, Madrid, Spain. FAU - Cobo-Ibáñez, Tatiana AU - Cobo-Ibáñez T AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. AD - Universidad Europea de Madrid, Madrid, Spain. FAU - Esteban-Vázquez, Ana AU - Esteban-Vázquez A AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. FAU - Muñoz-Fernández, Santiago AU - Muñoz-Fernández S AUID- ORCID: 0000-0002-2758-2085 AD - Rheumatology Department, Infanta Sofía University Hospital, San Sebastián de los Reyes, Madrid, Spain. AD - Universidad Europea de Madrid, Madrid, Spain. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - BTY153760O (ixekizumab) RN - 0 (Antibodies, Monoclonal, Humanized) SB - IM MH - Humans MH - *Raynaud Disease/chemically induced MH - Antibodies, Monoclonal, Humanized/adverse effects MH - Ischemia/chemically induced EDAT- 2023/06/07 01:07 MHDA- 2024/01/05 06:42 CRDT- 2023/06/06 21:03 PHST- 2023/05/26 00:00 [accepted] PHST- 2024/01/05 06:42 [medline] PHST- 2023/06/07 01:07 [pubmed] PHST- 2023/06/06 21:03 [entrez] AID - 7191018 [pii] AID - 10.1093/rheumatology/kead270 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Jan 4;63(1):e3-e5. doi: 10.1093/rheumatology/kead270. PMID- 21466606 OWN - NLM STAT- MEDLINE DCOM- 20111128 LR - 20191210 IS - 1549-8719 (Electronic) IS - 1073-9688 (Linking) VI - 18 IP - 6 DP - 2011 Aug TI - Preliminary clinical evaluation of semi-automated nailfold capillaroscopy in the assessment of patients with Raynaud's phenomenon. PG - 440-7 LID - 10.1111/j.1549-8719.2011.00104.x [doi] AB - OBJECTIVES:   Nailfold capillaroscopy is well established in screening patients with Raynaud's phenomenon for underlying SSc-spectrum disorders, by identifying abnormal capillaries. Our aim was to compare semi-automatic feature measurement from newly developed software with manual measurements, and determine the degree to which semi-automated data allows disease group classification. METHODS:   Images from 46 healthy controls, 21 patients with PRP and 49 with SSc were preprocessed, and semi-automated measurements of intercapillary distance and capillary width, tortuosity, and derangement were performed. These were compared with manual measurements. Features were used to classify images into the three subject groups. RESULTS:   Comparison of automatic and manual measures for distance, width, tortuosity, and derangement had correlations of r=0.583, 0.624, 0.495 (p<0.001), and 0.195 (p=0.040). For automatic measures, correlations were found between width and intercapillary distance, r=0.374, and width and tortuosity, r=0.573 (p<0.001). Significant differences between subject groups were found for all features (p<0.002). Overall, 75% of images correctly matched clinical classification using semi-automated features, compared with 71% for manual measurements. CONCLUSIONS:   Semi-automatic and manual measurements of distance, width, and tortuosity showed moderate (but statistically significant) correlations. Correlation for derangement was weaker. Semi-automatic measurements are faster than manual measurements. Semi-automatic parameters identify differences between groups, and are as good as manual measurements for between-group classification. CI - © 2011 John Wiley & Sons Ltd. FAU - Murray, Andrea K AU - Murray AK AD - School of Translational Medicine, The University of Manchester, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Salford, UK. andrea.murray@manchester.ac.uk FAU - Feng, Kaiyan AU - Feng K FAU - Moore, Tonia L AU - Moore TL FAU - Allen, Phillip D AU - Allen PD FAU - Taylor, Christopher J AU - Taylor CJ FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Evaluation Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Microcirculation JT - Microcirculation (New York, N.Y. : 1994) JID - 9434935 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - *Capillaries/pathology/physiopathology MH - Female MH - Humans MH - Image Processing, Computer-Assisted/*methods MH - Male MH - *Microscopic Angioscopy/instrumentation/methods MH - Middle Aged MH - *Raynaud Disease/pathology/physiopathology MH - *Software EDAT- 2011/04/07 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/04/07 06:00 PHST- 2011/04/07 06:00 [entrez] PHST- 2011/04/07 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 10.1111/j.1549-8719.2011.00104.x [doi] PST - ppublish SO - Microcirculation. 2011 Aug;18(6):440-7. doi: 10.1111/j.1549-8719.2011.00104.x. PMID- 15951918 OWN - NLM STAT- MEDLINE DCOM- 20060818 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 2 DP - 2006 Mar TI - Predictive value of nailfold capillaroscopy in patients with Raynaud's phenomenon. PG - 153-8 AB - The objective of this study was to evaluate the long-term follow-up of patients with Raynaud's phenomenon (RP) and pathological nailfold capillaroscopy (NC) in order to analyse the predictive value of specific features of capillaroscopy for the development of a connective tissue disease (CTD). From 1992 to 2002, NC alone or combined with fluorescence videomicroscopy with sodium fluorescein (NaF) was performed in 1024 consecutive patients because of RP. We analysed the follow-up and pathological features of NC in all patients who had neither clinical nor serological signs of a CTD at the time of NC. Of 308 patients with neither serological findings nor clinical signs of CTD but with RP and pathological features in NC suspicious for CTD, follow-up data were available for 133 patients. An additional NaF test had been performed in 51 (38.4%) patients. After a mean follow-up of 6.5 years (range: 1-15 years), 109 patients had developed a CTD and 24 patients did not show any clinical signs or serological markers for a CTD after a mean follow-up of 8.5 years (range: 2-15 years). There were no differences in age, duration of RP or of follow-up in patients who developed a CTD compared to patients who did not. Significantly more giant capillaries (p=0.0001), avascular fields (p=0.02) and irregular architecture (p=0.0001) had been observed in patients who had developed a CTD during the follow-up of 6.5 years. The presence of giant capillaries, avascular fields and irregular architecture of nailfold capillaries is predictive for the development of a CTD in patients with RP. FAU - Meli, Madeleine AU - Meli M AD - Division of Angiology, Department of Internal Medicine, University Hospital, Ramistrasse 100, 8091 Zurich, Switzerland. FAU - Gitzelmann, Gabriela AU - Gitzelmann G FAU - Koppensteiner, Renate AU - Koppensteiner R FAU - Amann-Vesti, Beatrice R AU - Amann-Vesti BR LA - eng PT - Journal Article DEP - 20050611 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Capillaries/*pathology MH - Child MH - Connective Tissue Diseases/etiology/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy MH - Microscopy, Video MH - Middle Aged MH - Nails MH - Predictive Value of Tests MH - Raynaud Disease/complications/*pathology EDAT- 2005/06/14 09:00 MHDA- 2006/08/19 09:00 CRDT- 2005/06/14 09:00 PHST- 2004/11/16 00:00 [received] PHST- 2005/03/23 00:00 [accepted] PHST- 2005/03/23 00:00 [revised] PHST- 2005/06/14 09:00 [pubmed] PHST- 2006/08/19 09:00 [medline] PHST- 2005/06/14 09:00 [entrez] AID - 10.1007/s10067-005-1146-1 [doi] PST - ppublish SO - Clin Rheumatol. 2006 Mar;25(2):153-8. doi: 10.1007/s10067-005-1146-1. Epub 2005 Jun 11. PMID- 27717655 OWN - NLM STAT- MEDLINE DCOM- 20181031 LR - 20181031 IS - 1578-8865 (Electronic) IS - 1138-3593 (Linking) VI - 43 IP - 4 DP - 2017 May-Jun TI - [Raynaud's phenomenon secondary to anti-glaucoma eye drops]. PG - e49-e50 LID - S1138-3593(16)30157-5 [pii] LID - 10.1016/j.semerg.2016.07.013 [doi] FAU - Bugarín-González, R AU - Bugarín-González R AD - Unidad de Asesoramiento Científico-técnico, avalia-t, Axencia de Coñecemento en Saúde (ACIS), Servicio Gallego de Salud, Santiago de Compostela, España. Electronic address: rosendo.bugarin.gonzalez@sergas.es. FAU - Portela-Romero, M AU - Portela-Romero M AD - Estrutura Organizativa de Xestión Integrada (EOXI) de Santiago de Compostela, Servicio Gallego de Salud, Santiago de Compostela, A Coruña, España. FAU - Maceira-Rozas, M C AU - Maceira-Rozas MC AD - Unidad de Asesoramiento Científico-técnico, avalia-t, Axencia de Coñecemento en Saúde (ACIS), Servicio Gallego de Salud, Santiago de Compostela, España. FAU - López-Vázquez, P AU - López-Vázquez P AD - Consellería de Sanidad, Xunta de Galicia, Santiago de Compostela, España. FAU - Bugarín-Diz, C AU - Bugarín-Diz C AD - King's College, Londres, Reino Unido. LA - spa PT - Case Reports PT - Journal Article TT - Fenómeno de Raynaud secundario a un colirio antiglaucomatoso. DEP - 20161004 PL - Spain TA - Semergen JT - Semergen JID - 9610769 RN - 0 (Antihypertensive Agents) RN - 0 (Drug Combinations) RN - 0 (Ophthalmic Solutions) RN - 0 (Sulfonamides) RN - 0 (Thiophenes) RN - 0 (dorzolamide-timolol combination) RN - 817W3C6175 (Timolol) SB - IM MH - Adult MH - Antihypertensive Agents/administration & dosage/adverse effects MH - Drug Combinations MH - Glaucoma, Open-Angle/drug therapy MH - Humans MH - Male MH - Ophthalmic Solutions MH - Raynaud Disease/*chemically induced/diagnosis MH - Sulfonamides/administration & dosage/*adverse effects MH - Thiophenes/administration & dosage/*adverse effects MH - Timolol/administration & dosage/*adverse effects EDAT- 2016/10/09 06:00 MHDA- 2018/11/01 06:00 CRDT- 2016/10/09 06:00 PHST- 2016/07/28 00:00 [received] PHST- 2016/07/29 00:00 [accepted] PHST- 2016/10/09 06:00 [pubmed] PHST- 2018/11/01 06:00 [medline] PHST- 2016/10/09 06:00 [entrez] AID - S1138-3593(16)30157-5 [pii] AID - 10.1016/j.semerg.2016.07.013 [doi] PST - ppublish SO - Semergen. 2017 May-Jun;43(4):e49-e50. doi: 10.1016/j.semerg.2016.07.013. Epub 2016 Oct 4. PMID- 25876022 OWN - NLM STAT- MEDLINE DCOM- 20160919 LR - 20181113 IS - 1680-0745 (Electronic) IS - 1995-1892 (Print) IS - 1015-9657 (Linking) VI - 26 IP - 6 DP - 2015 Nov-Dec TI - Autonomic imbalance assessed by time-domain heart rate variability indices in primary Raynaud's phenomenon. PG - 214-6 LID - 10.5830/CVJA-2015-032 [doi] AB - OBJECTIVES: The pathogenesis of primary Raynaud's phenomenon (RP) seems to be multifactorial and autonomic nervous dysfunction is one factor. Heart rate variability (HRV) is one of the most reliable parameters to demonstrate autonomic dysfunction. Our aim was to evaluate the time-domain HRV in patients with primary RP. METHODS: A time analysis of HRV was performed in patients with primary RP and age- and gender-matched healthy controls. The results of the study and control group were compared. RESULTS: Thirty patients with primary RP [all men, median (IQR) age: 21 (2) years) and 31 age- and gender-matched healthy controls (median (IQR): 21(3) years] were enrolled in the study. We found a statistically significant difference between the primary RP patients and control subjects in terms of time-domain HRV parameters (p < 0.05 for all). CONCLUSION: Our study showed the presence of autonomic nervous dysfunction of heart function in patients with primary RP. FAU - Karabacak, Kubilay AU - Karabacak K AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. FAU - Celik, Murat AU - Celik M AD - Department of Cardiology, Gulhane Military Academy of Medicine, Ankara, Turkey. FAU - Kaya, Erkan AU - Kaya E AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. FAU - Kadan, Murat AU - Kadan M AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. FAU - Arslan, Gokhan AU - Arslan G AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. FAU - Demirkilic, Ufuk AU - Demirkilic U AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. LA - eng PT - Journal Article DEP - 20150415 PL - South Africa TA - Cardiovasc J Afr JT - Cardiovascular journal of Africa JID - 101313864 SB - IM MH - Autonomic Nervous System/*physiopathology MH - Autonomic Nervous System Diseases/diagnosis/*physiopathology MH - Case-Control Studies MH - Electrocardiography, Ambulatory MH - Heart/*innervation MH - *Heart Rate MH - Humans MH - Male MH - Prognosis MH - Raynaud Disease/diagnosis/*physiopathology MH - Time Factors MH - Young Adult PMC - PMC4780019 EDAT- 2015/04/16 06:00 MHDA- 2016/09/20 06:00 PMCR- 2015/11/01 CRDT- 2015/04/16 06:00 PHST- 2014/09/07 00:00 [received] PHST- 2015/03/24 00:00 [accepted] PHST- 2015/04/16 06:00 [entrez] PHST- 2015/04/16 06:00 [pubmed] PHST- 2016/09/20 06:00 [medline] PHST- 2015/11/01 00:00 [pmc-release] AID - 10.5830/CVJA-2015-032 [doi] PST - ppublish SO - Cardiovasc J Afr. 2015 Nov-Dec;26(6):214-6. doi: 10.5830/CVJA-2015-032. Epub 2015 Apr 15. PMID- 23620555 OWN - NLM STAT- MEDLINE DCOM- 20131226 LR - 20151119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 52 IP - 11 DP - 2013 Nov TI - State of the art on nailfold capillaroscopy: a reliable diagnostic tool and putative biomarker in rheumatology? PG - 1933-40 LID - 10.1093/rheumatology/ket153 [doi] AB - Capillaroscopy is a non-invasive and safe tool to morphologically study the microcirculation. In rheumatology it has a dual use. First, it has a role in differential diagnosis of patients with RP. Second, it may have a role in the prediction of clinical complications in CTDs. In SSc, pilot studies have shown predictive associations with peripheral vascular and lung involvement hinting at a role of capillaroscopy as putative biomarker. Also and logically, in SSc, microangiopathy, as assessed by capillaroscopy, has been associated with markers of the disease such as angiogenic/static factors and SSc-specific antibodies. Moreover, morphological assessments of the microcirculation (capillaroscopy) seem to correlate with functional assessments (such as laser Doppler). Because of its clinical and research role, eyes are geared in Europe to expand the knowledge of this tool. Both the European League Against Rheumatism (EULAR) and the ACR are stepping forward to this need. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, No. 6, 16132 Genova, Italy. mcutolo@unige.it. FAU - Smith, Vanessa AU - Smith V LA - eng PT - Journal Article PT - Review DEP - 20130425 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Biomarkers) SB - IM MH - Biomarkers/blood MH - Connective Tissue Diseases/complications/diagnosis MH - Diagnosis, Differential MH - Humans MH - Microcirculation/physiology MH - Microscopic Angioscopy/methods/*trends MH - Nails/*blood supply MH - Prognosis MH - Raynaud Disease/*diagnosis/etiology MH - Scleroderma, Systemic/*diagnosis OTO - NOTNLM OT - EULAR OT - Raynaud's phenomenon OT - capillaroscopy OT - classification criteria OT - clinical complications OT - connective tissue diseases OT - differential diagnosis OT - microcirculation OT - systemic sclerosis EDAT- 2013/04/27 06:00 MHDA- 2013/12/27 06:00 CRDT- 2013/04/27 06:00 PHST- 2013/04/27 06:00 [entrez] PHST- 2013/04/27 06:00 [pubmed] PHST- 2013/12/27 06:00 [medline] AID - ket153 [pii] AID - 10.1093/rheumatology/ket153 [doi] PST - ppublish SO - Rheumatology (Oxford). 2013 Nov;52(11):1933-40. doi: 10.1093/rheumatology/ket153. Epub 2013 Apr 25. PMID- 22467473 OWN - NLM STAT- MEDLINE DCOM- 20130108 LR - 20120813 IS - 1099-0496 (Electronic) IS - 1099-0496 (Linking) VI - 47 IP - 9 DP - 2012 Sep TI - Dyspnea in a patient with Raynaud's phenomenon: the uncovering of interstitial lung disease. PG - 926-7 LID - 10.1002/ppul.22549 [doi] AB - Interstitial lung disease (ILD) can develop in patients with connective tissue disease (CTD) in the context of progressive multiorgan involvement, but ILD can also be the predominant manifestation of active CTD. A high index of suspicion for CTD in patients presenting with pulmonary disease might facilitate timely, accurate diagnosis and management. CI - Copyright © 2012 Wiley Periodicals, Inc. FAU - Coates, Anne AU - Coates A AD - Stanford University, Pediatric Pulmonary Division, Palo Alto, CA 94040, USA. coates67@stanford.edu FAU - Meehan, Richard AU - Meehan R FAU - Milla, Carlos AU - Milla C LA - eng PT - Case Reports PT - Journal Article DEP - 20120329 PL - United States TA - Pediatr Pulmonol JT - Pediatric pulmonology JID - 8510590 SB - IM MH - Adolescent MH - Connective Tissue Diseases/complications/*diagnosis MH - Dyspnea/etiology MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/etiology MH - Male MH - Raynaud Disease/etiology EDAT- 2012/04/03 06:00 MHDA- 2013/01/09 06:00 CRDT- 2012/04/03 06:00 PHST- 2011/08/25 00:00 [received] PHST- 2011/12/30 00:00 [accepted] PHST- 2012/04/03 06:00 [entrez] PHST- 2012/04/03 06:00 [pubmed] PHST- 2013/01/09 06:00 [medline] AID - 10.1002/ppul.22549 [doi] PST - ppublish SO - Pediatr Pulmonol. 2012 Sep;47(9):926-7. doi: 10.1002/ppul.22549. Epub 2012 Mar 29. PMID- 17018538 OWN - NLM STAT- MEDLINE DCOM- 20070509 LR - 20191210 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 3 DP - 2007 Mar TI - The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon. PG - 533-8 AB - OBJECTIVE: To test the hypothesis that in a patient with Raynaud's phenomenon (RP), a difference of >1 degrees C between the fingertips and the dorsum of the hand ['distal-dorsal difference' (DDD), fingers cooler] is specific for underlying structural vascular disease as occurs in systemic sclerosis (SSc), and to evaluate other thermographic parameters in the separation of secondary from primary RP. METHODS: A retrospective analysis of the case notes and thermography results of patients attending thermography, primarily over a 2-yr period. Multinomial logistic regression was used to ascertain whether thermography variables differed between groups with primary RP (56 patients), undifferentiated connective tissue disease (21 patients) and SSc (45 patients), with adjustment for age, sex and smoking. RESULTS: A DDD >1 degrees C in any finger at 30 degrees C had a positive predictive value of 70%, and a negative predictive value of 82%, in identifying the patient with RP secondary to SSc. From the results of the multinomial logistic regression, a score was derived incorporating age, number of fingers with DDD >1 degrees C at 30 degrees C and maximum rewarming gradient. This score (with a suitable cut-off) was 82% sensitive and 82% specific in identifying RP secondary to SSc, with a positive predictive value of 73% and a negative predictive value of 89%. CONCLUSION: Parameters derived from thermography (incorporating both a heat and cold challenge) aid in the prediction of SSc in patients with RP. FAU - Anderson, M E AU - Anderson ME AD - University of Liverpool Adademic Rheumatology Unit, University Hospital Aintree, Liverpool, UK. FAU - Moore, T L AU - Moore TL FAU - Lunt, M AU - Lunt M FAU - Herrick, A L AU - Herrick AL LA - eng PT - Evaluation Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20061002 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Aged MH - Diagnosis, Differential MH - Epidemiologic Methods MH - Female MH - Fingers MH - Hand MH - Humans MH - Image Processing, Computer-Assisted MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/*etiology MH - Scleroderma, Systemic/*complications/*diagnosis MH - Skin Temperature MH - Thermography EDAT- 2006/10/05 09:00 MHDA- 2007/05/10 09:00 CRDT- 2006/10/05 09:00 PHST- 2006/10/05 09:00 [pubmed] PHST- 2007/05/10 09:00 [medline] PHST- 2006/10/05 09:00 [entrez] AID - kel330 [pii] AID - 10.1093/rheumatology/kel330 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Mar;46(3):533-8. doi: 10.1093/rheumatology/kel330. Epub 2006 Oct 2. PMID- 29750280 OWN - NLM STAT- MEDLINE DCOM- 20190108 LR - 20190108 IS - 1471-8405 (Electronic) IS - 0962-7480 (Print) IS - 0962-7480 (Linking) VI - 68 IP - 5 DP - 2018 Jun 20 TI - Can sensation of cold hands predict Raynaud's phenomenon or paraesthesia? PG - 314-319 LID - 10.1093/occmed/kqy053 [doi] AB - BACKGROUND: Raynaud's phenomenon and neurosensory symptoms are common after hand-arm vibration exposure. Knowledge of early signs of vibration injuries is needed. AIMS: To investigate the risk of developing Raynaud's phenomenon and paraesthesia in relation to sensation of cold hands in a cohort of male employees at an engineering plant. METHODS: We followed a cohort of male manual and office workers at an engineering plant in Sweden for 21 years. At baseline (1987 and 1992) and each follow-up (1992, 1997, 2002, 2008), we assessed sensation of cold, Raynaud's phenomenon and paraesthesia in the hands using questionnaires and measured vibration exposure. We calculated risk estimates with univariate and multiple logistic regression analyses and adjusted for vibration exposure and tobacco usage. RESULTS: There were 241 study participants. During the study period, 21 individuals developed Raynaud's phenomenon and 43 developed paraesthesia. When adjusting the risk of developing Raynaud's phenomenon for vibration exposure and tobacco use, the odds ratios were between 6.0 and 6.3 (95% CI 2.2-17.0). We observed no increased risk for paraesthesia in relation to a sensation of cold hands. CONCLUSIONS: A sensation of cold hands was a risk factor for Raynaud's phenomenon. At the individual level, reporting a sensation of cold hands did not appear to be useful information to predict future development of Raynaud's phenomenon given a weak to moderate predictive value. For paraesthesia, the sensation of cold was not a risk factor and there was no predictive value at the individual level. FAU - Carlsson, D AU - Carlsson D AD - Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå University, Umeå, Sweden. FAU - Wahlström, J AU - Wahlström J AD - Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå University, Umeå, Sweden. FAU - Burström, L AU - Burström L AD - Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå University, Umeå, Sweden. FAU - Hagberg, M AU - Hagberg M AD - Occupational and Environmental Medicine, Sahlgrenska Academy and University Hospital, University of Gothenburg, Gothenburg, Sweden. FAU - Lundström, R AU - Lundström R AD - Department of Radiation Sciences, Umeå University, Umeå, Sweden. FAU - Pettersson, H AU - Pettersson H AD - Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå University, Umeå, Sweden. FAU - Nilsson, T AU - Nilsson T AD - Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Adult MH - Cold Temperature/adverse effects MH - Female MH - Hand/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Paresthesia/*etiology/physiopathology MH - Raynaud Disease/*diagnosis/physiopathology MH - Risk Factors MH - Sweden PMC - PMC6012203 EDAT- 2018/05/12 06:00 MHDA- 2019/01/09 06:00 PMCR- 2018/05/10 CRDT- 2018/05/12 06:00 PHST- 2018/05/12 06:00 [pubmed] PHST- 2019/01/09 06:00 [medline] PHST- 2018/05/12 06:00 [entrez] PHST- 2018/05/10 00:00 [pmc-release] AID - 4994727 [pii] AID - kqy053 [pii] AID - 10.1093/occmed/kqy053 [doi] PST - ppublish SO - Occup Med (Lond). 2018 Jun 20;68(5):314-319. doi: 10.1093/occmed/kqy053. PMID- 17666574 OWN - NLM STAT- MEDLINE DCOM- 20071004 LR - 20131121 IS - 1542-6270 (Electronic) IS - 1060-0280 (Linking) VI - 41 IP - 9 DP - 2007 Sep TI - Effect of clonazepam on Raynaud's phenomenon and fingertip ulcers in scleroderma. PG - 1544-7 AB - OBJECTIVE: To report the novel finding of a significant improvement in Raynaud's phenomenon symptoms with clonazepam in a patient with systemic sclerosis. CASE SUMMARY: A 45-year-old female with limited scleroderma and chronic renal failure was admitted to our hospital due to hyponatremia (sodium 103 mEq/L). Her hyponatremia was treated by intravenous infusion of NaCl 3%. Clonazepam, which had been prescribed previously for anxiety and insomnia, was discontinued. Three weeks after she was discharged from the hospital, the patient presented with the complaint of increased severity of Raynaud's phenomenon and digital ulcers. She told us that her fingertip ulcers had been healed while she was taking clonazepam and that episodes of Raynaud's phenomenon had increased after discontinuation of the drug. Clonazepam 1 mg twice daily was restarted, and Raynaud's phenomenon and fingertip ulcers resolved within a month. On 2 occasions after that time, we discontinued clonazepam and replaced it with alprazolam, as the patient believed alprazolam was more beneficial in alleviating anxiety. Episodes of Raynaud's phenomenon and new digital ulcers recurred on both of these occasions, and clonazepam was restarted. At the time of writing, no severe episodes of Raynaud's phenomenon or fingertip ulcers have occurred with clonazepam treatment. DISCUSSION: Raynaud's phenomenon and recurrent digital ulcers are a manifestation of vascular disease in patients with systemic sclerosis and lead to pain, impaired function, and tissue loss. Few drugs have previously been shown to affect digital ulcers in the setting of scleroderma. Our patient experienced a significant and sustained improvement in Raynaud's phenomenon and digital ulcers following the initiation of clonazepam. To our knowledge, as of March 2007, this is the first reported use of clonazepam in Raynaud's phenomenon and digital ulcer. While its therapeutic mechanism remains unclear, clonazepam may offer some advantages compared with current agents. CONCLUSIONS: We report a case of Raynaud's phenomenon and digital ulcers responding to clonazepam. Further research is warranted to test the robustness of this preliminary finding. FAU - Colakoğlu, Murat AU - Colakoğlu M AD - Departmen of Nephrology, School of Medicine, Pamukkale University, Denizli, Turkey. murcol@mynet.com FAU - Cobankara, Veli AU - Cobankara V FAU - Akpolat, Tekin AU - Akpolat T LA - eng PT - Case Reports PT - Journal Article DEP - 20070731 PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - 0 (Anti-Anxiety Agents) RN - 5PE9FDE8GB (Clonazepam) SB - IM MH - Anti-Anxiety Agents/therapeutic use MH - Clonazepam/*therapeutic use MH - Female MH - Fingers MH - Humans MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications/drug therapy MH - Ulcer/*drug therapy/etiology EDAT- 2007/08/02 09:00 MHDA- 2007/10/05 09:00 CRDT- 2007/08/02 09:00 PHST- 2007/08/02 09:00 [pubmed] PHST- 2007/10/05 09:00 [medline] PHST- 2007/08/02 09:00 [entrez] AID - aph.1K212 [pii] AID - 10.1345/aph.1K212 [doi] PST - ppublish SO - Ann Pharmacother. 2007 Sep;41(9):1544-7. doi: 10.1345/aph.1K212. Epub 2007 Jul 31. PMID- 19149638 OWN - NLM STAT- MEDLINE DCOM- 20090305 LR - 20191027 IS - 1570-1611 (Print) IS - 1570-1611 (Linking) VI - 7 IP - 1 DP - 2009 Jan TI - Neuronal regulators and vascular dysfunction in Raynaud's phenomenon and systemic sclerosis. PG - 34-9 AB - Raynaud's phenomenon (RP) results from an exaggerated cutaneous vasospastic response to cold or emotional stress. The mechanisms that lead to impaired cutaneous vascular tone are complex. The regulation of cutaneous vasoconstriction and vasodilation, involves altered sympathetic nerve activity and a host of neuronal regulators, including adrenergic and non-adrenergic, as well as REDOX signalling and other signalling such as the RhoA/ROCK pathway. This review summarises the literature concerning the regulation of vascular tone by neurohumoral factors that may be involved in RP and systemic sclerosis (SSc). FAU - Fonseca, Carmen AU - Fonseca C AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, Hampstead, London, NW3 2PF, UK. FAU - Abraham, David AU - Abraham D FAU - Ponticos, Markella AU - Ponticos M LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Vasc Pharmacol JT - Current vascular pharmacology JID - 101157208 RN - EC 2.7.11.1 (rho-Associated Kinases) RN - EC 3.6.5.2 (rhoA GTP-Binding Protein) SB - IM MH - Humans MH - Oxidation-Reduction MH - Raynaud Disease/*physiopathology MH - Scleroderma, Systemic/*physiopathology MH - Signal Transduction MH - Sympathetic Nervous System/*physiopathology MH - rho-Associated Kinases/metabolism MH - rhoA GTP-Binding Protein/metabolism RF - 66 EDAT- 2009/01/20 09:00 MHDA- 2009/03/06 09:00 CRDT- 2009/01/20 09:00 PHST- 2009/01/20 09:00 [entrez] PHST- 2009/01/20 09:00 [pubmed] PHST- 2009/03/06 09:00 [medline] AID - 10.2174/157016109787354105 [doi] PST - ppublish SO - Curr Vasc Pharmacol. 2009 Jan;7(1):34-9. doi: 10.2174/157016109787354105. PMID- 28664836 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20171221 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 Suppl 106 IP - 4 DP - 2017 Sep-Oct TI - The use of hand perfusion scintigraphy to assess Raynaud's phenomenon associated with hand-arm vibration syndrome. PG - 138-143 AB - OBJECTIVES: This study aimed to evaluate the hand perfusion scintigraphic features of hand-arm vibration syndrome (HAVS) and to compare these with the features of primary and secondary Raynaud's phenomenon (RP) associated with systemic sclerosis (SSc). METHODS: Hand perfusion scintigraphy was performed in 57 patients with primary RP, 71 patients with HAVS-related RP, and 15 patients with SSc-related RP. We calculated 6 ratios: chilled to ambient hand and wrist ratios of the first peak height, initial slope, and blood pool uptake. We analysed 3 morphologic characteristics: slow progress pattern, paradoxically increased uptake pattern, and inhomogeneous radioactivity uptake. RESULTS: All of the 71 patients with HAVS-related RP were mine workers. The chilled to ambient hand ratios of the first peak height, the initial slope, and the blood pool uptake were significantly lower in patients with HAVS-related occupational RP than in patients with primary RP. The presence of a paradoxically increased uptake pattern was significantly lower in HAVS than in primary RP. CONCLUSIONS: There were significant differences in hand perfusion scintigraphic features between primary RP and HAVS. These results suggest that the underlying pathophysiology of the two diseases differs. FAU - Lee, Kyung-Ann AU - Lee KA AD - Division of Rheumatology, Department of Internal Medicine, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea. FAU - Chung, Hyung Woo AU - Chung HW AD - Department of Nuclear Medicine, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea. FAU - Lee, Sang-Heon AU - Lee SH AD - Division of Rheumatology, Department of Internal Medicine, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea. FAU - Kim, Hae-Rim AU - Kim HR AD - Division of Rheumatology, Department of Internal Medicine, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea. kimhaerim@kuh.ac.kr. LA - eng PT - Journal Article DEP - 20170630 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Hand/*blood supply MH - Hand-Arm Vibration Syndrome/complications/*physiopathology MH - Humans MH - Male MH - Middle Aged MH - Perfusion Imaging/*methods MH - Raynaud Disease/etiology/*physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/complications MH - Young Adult EDAT- 2017/07/01 06:00 MHDA- 2017/12/22 06:00 CRDT- 2017/07/01 06:00 PHST- 2017/02/22 00:00 [received] PHST- 2017/06/19 00:00 [accepted] PHST- 2017/07/01 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/07/01 06:00 [entrez] AID - 11649 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):138-143. Epub 2017 Jun 30. PMID- 22453196 OWN - NLM STAT- MEDLINE DCOM- 20120607 LR - 20211021 IS - 1532-6535 (Electronic) IS - 0009-9236 (Print) IS - 0009-9236 (Linking) VI - 91 IP - 5 DP - 2012 May TI - Sildenafil increases digital skin blood flow during all phases of local cooling in primary Raynaud's phenomenon. PG - 813-9 LID - 10.1038/clpt.2011.302 [doi] AB - Digital skin vasoconstriction on local cooling is exaggerated in primary Raynaud's phenomenon (RP) as compared with controls. A significant part of such vasoconstriction relies on the inhibition of the nitric oxide (NO) pathway. We tested the effect of the phosphodiesterase 5 (PDE5) inhibitor sildenafil, which potentiates the effect of NO, on skin blood flow. We recruited 15 patients with primary RP, performing local cooling without sildenafil (day 1), after a single oral dose of 50 mg (day 2), and after a dose of 100 mg (day 3). Skin blood flow, skin temperature, and arterial pressure were recorded, and data were expressed as cutaneous vascular conductance (CVC). Sildenafil at 100 mg, but not 50 mg, significantly lessened the cooling-induced decrease in CVC. It also increased resting CVC and skin temperature. These data suggest that 100 mg sildenafil improves digital skin perfusion during local cooling in primary RP. The benefit of sildenafil "as required" should be confirmed in a randomized, controlled trial. FAU - Roustit, M AU - Roustit M AD - INSERM U1042, Université Joseph Fourier, Grenoble, France. MRoustit@chu-grenoble.fr FAU - Hellmann, M AU - Hellmann M FAU - Cracowski, C AU - Cracowski C FAU - Blaise, S AU - Blaise S FAU - Cracowski, J L AU - Cracowski JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120328 PL - United States TA - Clin Pharmacol Ther JT - Clinical pharmacology and therapeutics JID - 0372741 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/adverse effects/*pharmacology MH - Piperazines/adverse effects/*pharmacology MH - Purines/adverse effects/pharmacology MH - Raynaud Disease/*drug therapy/physiopathology MH - Regional Blood Flow/drug effects MH - Sildenafil Citrate MH - Skin/*blood supply MH - Skin Temperature/drug effects MH - Sulfones/adverse effects/*pharmacology PMC - PMC4000564 MID - HALMS767054 OID - NLM: HALMS767054 COIS- Conflicts of interest JLC and MR have received research grants from Pfizer for other studies. EDAT- 2012/03/29 06:00 MHDA- 2012/06/08 06:00 PMCR- 2014/04/28 CRDT- 2012/03/29 06:00 PHST- 2012/03/29 06:00 [entrez] PHST- 2012/03/29 06:00 [pubmed] PHST- 2012/06/08 06:00 [medline] PHST- 2014/04/28 00:00 [pmc-release] AID - clpt2011302 [pii] AID - 10.1038/clpt.2011.302 [doi] PST - ppublish SO - Clin Pharmacol Ther. 2012 May;91(5):813-9. doi: 10.1038/clpt.2011.302. Epub 2012 Mar 28. PMID- 41060307 OWN - NLM STAT- MEDLINE DCOM- 20260308 LR - 20260308 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 36 IP - 2 DP - 2026 Mar 5 TI - Cold fingers under the lens: unveiling microvascular differences between children with primary Raynaud's phenomenon and healthy individuals. PG - 252-260 LID - 10.1093/mr/roaf089 [doi] AB - OBJECTIVES: This study aimed to evaluate capillaroscopic findings in children with Raynaud's phenomenon (RP) referred to a paediatric rheumatology clinic and compare them to healthy controls to identify RP-related patterns. METHODS: Sixty-six patients aged 0-18 years with RP and 65 age- and sex-matched healthy controls were included. Standardized capillaroscopic assessments followed the 2020 recommendations of EULAR study group on microcirculation in rheumatic diseases. Capillaroscopic patterns of 62 primary RP patients were compared with controls. RESULTS: Two patients were diagnosed with systemic sclerosis and two with systemic lupus erythematosus. Among 62 primary RP patients (median age 14.92 years, 62.9% female), antinuclear antibody positivity was 11.29%. Capillaroscopy revealed increased apical loop diameter (18.74 ± 4.40 vs. 15.20 ± 2.98, P < .001), dilated capillaries (82.3% vs. 15.40%, P < .001), abnormal capillaries (53.2% vs. 18.5%, P < .001), microhemorrhages (17.7% vs. 1.5%, P = .002) in primary RP patients compared to controls. The predominant pattern was non-specific (56.5%) in RP patients and normal pattern in controls (87.7%, P < .001). No correlation was found between capillaroscopy patterns and antinuclear antibody positivity or medication use. CONCLUSIONS: Patients with primary RP showed a unique capillaroscopy pattern. Follow-up studies are needed to assess the proportion who may develop secondary RP and how capillaroscopic findings evolve. CI - © Japan College of Rheumatology 2025. FAU - Kavrul Kayaalp, Gülşah AU - Kavrul Kayaalp G AUID- ORCID: 0000-0001-7490-7076 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Arık, Selen Duygu AU - Arık SD AUID- ORCID: 0000-0001-7648-1195 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Akgün, Özlem AU - Akgün Ö AUID- ORCID: 0000-0001-7216-0562 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Menentoğlu, Bengisu AU - Menentoğlu B AUID- ORCID: 0000-0003-2774-9591 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Doğru, Ayşenur AU - Doğru A AUID- ORCID: 0000-0001-8091-8417 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Çakmak, Figen AU - Çakmak F AUID- ORCID: 0000-0002-1667-2480 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. FAU - Aktay Ayaz, Nuray AU - Aktay Ayaz N AUID- ORCID: 0000-0003-3594-7387 AD - Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye. LA - eng PT - Journal Article PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - *Raynaud Disease/physiopathology/diagnostic imaging MH - Female MH - Male MH - Child MH - Adolescent MH - Microscopic Angioscopy MH - Child, Preschool MH - Infant MH - Microcirculation MH - *Fingers/blood supply MH - Case-Control Studies MH - Capillaries/diagnostic imaging MH - Antibodies, Antinuclear OTO - NOTNLM OT - Raynaud’s phenomenon OT - connective tissue diseases OT - nailfold capillaroscopy OT - paediatric rheumatology OT - systemic sclerosis EDAT- 2025/10/08 17:31 MHDA- 2026/03/08 06:38 CRDT- 2025/10/08 10:43 PHST- 2025/06/20 00:00 [received] PHST- 2025/09/09 00:00 [accepted] PHST- 2026/03/08 06:38 [medline] PHST- 2025/10/08 17:31 [pubmed] PHST- 2025/10/08 10:43 [entrez] AID - 8277633 [pii] AID - 10.1093/mr/roaf089 [doi] PST - ppublish SO - Mod Rheumatol. 2026 Mar 5;36(2):252-260. doi: 10.1093/mr/roaf089. PMID- 22077642 OWN - NLM STAT- MEDLINE DCOM- 20130222 LR - 20120925 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 39 IP - 10 DP - 2012 Oct TI - Highly sensitive high-pressure liquid chromatography with ultraviolet light method detected the reduction of serum nitrite/nitrate levels after cold exposure in patients with Raynaud's phenomenon. PG - 889-90 LID - 10.1111/j.1346-8138.2011.01433.x [doi] FAU - Tanaka, Asayo AU - Tanaka A FAU - Yamazaki, Masashi AU - Yamazaki M FAU - Saito, Masuyoshi AU - Saito M FAU - Oh-I, Tsunao AU - Oh-I T FAU - Watanabe, Yasuo AU - Watanabe Y FAU - Tsuboi, Ryoji AU - Tsuboi R LA - eng PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20111114 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Nitrates) RN - 0 (Nitrites) RN - 0 (Platelet Aggregation Inhibitors) RN - 0 (Succinates) RN - 19P708E787 (sarpogrelate) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Chromatography, High Pressure Liquid/instrumentation/*methods MH - *Cold Temperature MH - Female MH - Hand/physiology MH - Humans MH - Male MH - Middle Aged MH - Nitrates/*blood MH - Nitrites/*blood MH - Platelet Aggregation Inhibitors/therapeutic use MH - Raynaud Disease/*blood/drug therapy MH - Stress, Physiological/drug effects/physiology MH - Succinates/therapeutic use MH - *Ultraviolet Rays MH - Young Adult EDAT- 2011/11/15 06:00 MHDA- 2013/02/23 06:00 CRDT- 2011/11/15 06:00 PHST- 2011/11/15 06:00 [entrez] PHST- 2011/11/15 06:00 [pubmed] PHST- 2013/02/23 06:00 [medline] AID - 10.1111/j.1346-8138.2011.01433.x [doi] PST - ppublish SO - J Dermatol. 2012 Oct;39(10):889-90. doi: 10.1111/j.1346-8138.2011.01433.x. Epub 2011 Nov 14. PMID- 30086690 OWN - NLM STAT- MEDLINE DCOM- 20181024 LR - 20181024 IS - 1661-8157 (Print) IS - 1661-8157 (Linking) VI - 107 IP - 16 DP - 2018 Aug TI - [Cold and White - Hypothenar Hammer Syndrome]. PG - 912-916 LID - 10.1024/1661-8157/a003046 [doi] AB - Cold and White - Hypothenar Hammer Syndrome Abstract. Hypothenar hammer syndrome (HHS) is a condition caused by digital ischemia as a result of repeated trauma to the little finger. Routine diagnosis should include a detailed medical history and a physical examination including Allen's test. Imaging of vascular lesions can be done initially by acral plethysmography and duplex sonography, or directly in the context of angiography (gold standard). Early diagnosis enables effective therapeutic strategies and preventing permanent sequelae. The optimal treatment options are selected depending on the intensity of symptoms, ranging from conservative methods, secondary prevention, through a local thrombolysis up to operational measures. FAU - Biskup, Ewelina AU - Biskup E AD - 1 Shanghai University of Medicine and Health Sciences, Shanghai, P.R. China. LA - ger PT - Case Reports PT - Journal Article TT - Kalt und weiss – Hypothenar-Hammer-Syndrom. PL - Switzerland TA - Praxis (Bern 1994) JT - Praxis JID - 101468093 SB - IM MH - *Accidents, Occupational MH - Adult MH - Computed Tomography Angiography MH - Diagnosis, Differential MH - Finger Injuries/*complications/*diagnosis/drug therapy MH - Fingers/*blood supply MH - Humans MH - Ischemia/*diagnosis/drug therapy/*etiology MH - Male MH - Neurologic Examination MH - Occupational Injuries/*diagnosis/drug therapy MH - Raynaud Disease/diagnosis/drug therapy MH - Thrombolytic Therapy MH - Thrombosis/diagnosis/drug therapy MH - Ulnar Artery/injuries OTO - NOTNLM OT - A. ulnaris OT - Hypothenar hammer syndrome OT - Hypothenar-Hammer-Syndrom OT - Raynaud-Phänomen OT - Raynaud’s phenomenon OT - Thrombolyse OT - digital ischemia OT - digitale Ischämie OT - thrombolysis EDAT- 2018/08/09 06:00 MHDA- 2018/10/26 06:00 CRDT- 2018/08/09 06:00 PHST- 2018/08/09 06:00 [entrez] PHST- 2018/08/09 06:00 [pubmed] PHST- 2018/10/26 06:00 [medline] AID - 10.1024/1661-8157/a003046 [doi] PST - ppublish SO - Praxis (Bern 1994). 2018 Aug;107(16):912-916. doi: 10.1024/1661-8157/a003046. PMID- 31747762 OWN - NLM STAT- MEDLINE DCOM- 20191129 LR - 20200108 IS - 0006-9248 (Print) IS - 0006-9248 (Linking) VI - 120 IP - 11 DP - 2019 TI - The relationship between Raynaud's phenomenon and Helicobacter pylori. PG - 827-831 LID - 10.4149/BLL_2019_137 [doi] AB - BACKGROUND: This study was aimed to reveal whether there is a relationship between Raynaud Phenomenon (RP) and Helicobacter Pylori (HP). MATERIAL AND METHODS: Seventy-nine patients, who had been referred to outpatient clinic with Raynaud in the last 9 years were retrospectively screened. Of these, 29 patients with access to their data and who had HP screening tests were included in the study. RESULTS: HP direct antigen was found in feces in only one of 29 patients. When we compared the results of this study to the results of previous literature, it was observed that the patients admitted with RP symptoms by a gastroenterology outpatient clinic had a higher incidence for HP (+) scanning than the patients admitted with RP symptoms by a cardiovascular surgery policlinic (CVSOC). CONCLUSION: Although previous literature reports that HP leads to RP in the group of patients referred to other outpatient clinics, the same relation is very low in the Raynaud patient group in CVSOC. Patients admitted with RP in the CVSOC shouldn't be prescribed empirical antibiotic therapy for the eradication of HP. However, as the appropriate antibiotic regimen can resolve Raynaud symptoms in the presence of a HP(+) test, it makes this scanning rational for all symptomatic patients (Tab. 1, Fig. 1, Ref. 23). FAU - Alat, I AU - Alat I LA - eng PT - Journal Article PL - Germany TA - Bratisl Lek Listy JT - Bratislavske lekarske listy JID - 0065324 SB - IM MH - Helicobacter Infections/*complications MH - Helicobacter pylori MH - Humans MH - Raynaud Disease/*complications/microbiology MH - Retrospective Studies OTO - NOTNLM OT - Helicobacter pylori OT - Raynaud phenomenon OT - Raynaud syndrome OT - vasculitis gastritis. EDAT- 2019/11/22 06:00 MHDA- 2019/11/30 06:00 CRDT- 2019/11/22 06:00 PHST- 2019/11/22 06:00 [entrez] PHST- 2019/11/22 06:00 [pubmed] PHST- 2019/11/30 06:00 [medline] AID - 10.4149/BLL_2019_137 [doi] PST - ppublish SO - Bratisl Lek Listy. 2019;120(11):827-831. doi: 10.4149/BLL_2019_137. PMID- 17169532 OWN - NLM STAT- MEDLINE DCOM- 20070327 LR - 20081121 IS - 0923-1811 (Print) IS - 0923-1811 (Linking) VI - 45 IP - 2 DP - 2007 Feb TI - A cold-response index for the assessment of Raynaud's phenomenon. PG - 113-20 AB - BACKGROUND: Quantification of Raynaud's phenomenon (RP) is a prerequisite in the evaluation of novel therapeutic strategies. Fingertip rewarming in response to local cold provocation has been used in many studies but not been systematically validated. We have previously described the time elapsed before 63% of pre-cooling temperature is reached as a RP activity index. OBJECTIVE: A comprehensive evaluation of fingertip rewarming in primary and scleroderma-associated RP. METHODS: We defined a cold-response index (CRI) as the log transformation of the 63% rewarming time upon cold challenge. RESULTS: The CRI shows high intra-individual reproducibility. The mean CRI values were (mean+/-S.D.): 2.4+/-0.3 in controls (n=53) versus 2.7+/-0.3 in RP (n=50, p<0.0001 versus controls), and 2.7+/-0.3 in scleroderma patients (n=46, p<0.0001). In addition, baseline fingertip temperature was also found to be significantly reduced both in primary as well as scleroderma-associated RP. Kinetic analysis of rewarming temperature curves demonstrates that the CRI is independent of individual rewarming patterns. Finally, the CRI decreases significantly upon a single low-level systemic hyperthermia treatment in scleroderma patients (2.68+/-0.28 before versus 2.45+/-0.33 after, p=0.0003), while the extent of cooling remained unchanged, thus demonstrating sensitivity to change. CONCLUSION: Our results provide a solid basis for using the cold-response assay as an endpoint in addition to clinical activity scores in RP treatment trials. FAU - Foerster, John AU - Foerster J AD - Klinik für Dermatologie, Charité, Charitéplatz 1, 10117 Berlin, Germany. john.foerster@charite.de FAU - Kuerth, Anja AU - Kuerth A FAU - Niederstrasser, Eyline AU - Niederstrasser E FAU - Krautwald, Esther AU - Krautwald E FAU - Pauli, Ruth AU - Pauli R FAU - Paulat, Raik AU - Paulat R FAU - Eweleit, Markus AU - Eweleit M FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Worm, Margitta AU - Worm M LA - eng PT - Controlled Clinical Trial PT - Journal Article DEP - 20061213 PL - Netherlands TA - J Dermatol Sci JT - Journal of dermatological science JID - 9011485 SB - IM MH - Adult MH - Aged MH - *Cold Temperature MH - Female MH - Fingers MH - Hot Temperature MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/*physiopathology MH - Reproducibility of Results MH - Thermography/*instrumentation/*methods/standards EDAT- 2006/12/16 09:00 MHDA- 2007/03/28 09:00 CRDT- 2006/12/16 09:00 PHST- 2006/09/27 00:00 [received] PHST- 2006/11/03 00:00 [revised] PHST- 2006/11/08 00:00 [accepted] PHST- 2006/12/16 09:00 [pubmed] PHST- 2007/03/28 09:00 [medline] PHST- 2006/12/16 09:00 [entrez] AID - S0923-1811(06)00345-8 [pii] AID - 10.1016/j.jdermsci.2006.11.006 [doi] PST - ppublish SO - J Dermatol Sci. 2007 Feb;45(2):113-20. doi: 10.1016/j.jdermsci.2006.11.006. Epub 2006 Dec 13. PMID- 21879200 OWN - NLM STAT- MEDLINE DCOM- 20120119 LR - 20140530 IS - 1581-2979 (Electronic) IS - 1318-4458 (Linking) VI - 20 IP - 1 DP - 2011 TI - Discontinuing long-term Iloprost treatment for Raynaud's Phenomenon and systemic sclerosis: a single-center, randomized, placebo-controlled, double-blind study. PG - 13-21 AB - BACKGROUND: Iloprost has been reported to reduce Raynaud`s phenomenon (RP) and to inhibit progression of systemic sclerosis (SSc). OBJECTIVE: The aim of our study was to compare monthly iloprost infusions with placebo in patients treated long-term. METHODS: Seventeen patients, six with RP and 11 with SSc on monthly treatment with iloprost, received either a 3-hour intravenous infusion of iloprost or an equal volume of placebo once per month for 4 months in a monocentric, randomized, placebo-controlled, double-blind study. Raynaud attacks as measured by diary entries, skin temperature, skin sclerosis, fist closure, mouth opening, and digital ulcers were recorded during the observation period. RESULTS: Whereas mouth opening improved significantly (p = 0.043) in the iloprost-treated group, RS improved in both patient groups. However, no significant differences were found in the outcome measures. CONCLUSION: Although iloprost influences the inflammatory cascade in SSc, no statistical differences were seen in our study, indicating that treatment strategies with iloprost should be modified. FAU - Bali, G AU - Bali G AD - MVZ Attendorn GmbH, Ostwall 94, D-57439 Attendorn. gaborbali@hotmail.com FAU - Schwantzer, G AU - Schwantzer G FAU - Aberer, F AU - Aberer F FAU - Kraenke, B AU - Kraenke B FAU - Aberer, E AU - Aberer E LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - Slovenia TA - Acta Dermatovenerol Alp Pannonica Adriat JT - Acta dermatovenerologica Alpina, Pannonica, et Adriatica JID - 9422563 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Double-Blind Method MH - Female MH - Humans MH - Iloprost/*administration & dosage MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*drug therapy MH - Scleroderma, Systemic/complications/*drug therapy MH - Vasodilator Agents/*administration & dosage EDAT- 2011/09/01 06:00 MHDA- 2012/01/20 06:00 CRDT- 2011/09/01 06:00 PHST- 2011/09/01 06:00 [entrez] PHST- 2011/09/01 06:00 [pubmed] PHST- 2012/01/20 06:00 [medline] AID - 00000212 [pii] PST - ppublish SO - Acta Dermatovenerol Alp Pannonica Adriat. 2011;20(1):13-21. PMID- 20351379 OWN - NLM STAT- MEDLINE DCOM- 20100607 LR - 20100330 IS - 0964-5284 (Print) IS - 0964-5284 (Linking) VI - 28 IP - 1 DP - 2010 Mar TI - Electroacupuncture therapy for arthralgia and Raynaud's phenomenon in a patient with systemic lupus erythematosus. PG - 49-51 LID - 10.1136/aim.2009.001529 [doi] AB - A 45-year-old woman with systemic lupus erythematosus presented with multiple arthralgia, coldness in fingers and toes, and Raynaud's phenomenon. Electroacupuncture (EA) therapy was performed in two courses (14 treatment sessions) 1 month apart. A needle was inserted in the proximal (or medial) side of the painful joint and another needle was inserted in the distal (or lateral) side of the same joint and a 50 Hz stimulus was applied (3 s bursts with 1 s gaps) for 15 min. A visual analogue scale was used to evaluate pain intensity. Cold provocation testing was conducted before and after EA sessions to determine the vasomotor response. Visual analogue scale scores were lower after EA sessions than before. Before starting EA, the skin temperature of the right mid fingertip was 27.9 degrees C and that of the left mid fingertip was 28.3 degrees C. In contrast, after the EA sessions, the skin temperature of the right mid fingertip was 34.8 degrees C and that of the left mid fingertip was 34.7 degrees C. In the last EA session, the patient reported that the cold in her fingers and toes had eased and Raynaud's phenomenon, in which nail colour tone changed from white to red, had disappeared. In the cold-provocation test, before EA, the temperature recovery rates of mid fingertips after cold exposure reached over 80% in 20 min. In contrast, after EA had been completed, the temperature recovery rate exceeded 80% in 10 min, thus the delay of temperature recovery was alleviated. FAU - Donoyama, Nozomi AU - Donoyama N AD - Course of Acupuncture and Moxibustion, Department of Health, Faculty of Health Sciences, Tsukuba University of Technology, 4-12-7, Kasuga, Tsukuba, Ibaraki 305-8521, Japan. donoyama@k.tsukuba-tech.ac.jp FAU - Ohkoshi, Norio AU - Ohkoshi N LA - eng PT - Case Reports PT - Journal Article PL - England TA - Acupunct Med JT - Acupuncture in medicine : journal of the British Medical Acupuncture Society JID - 9304117 SB - IM MH - Arthralgia/etiology/*therapy MH - Electroacupuncture/*methods MH - Female MH - Fingers/blood supply MH - Humans MH - Lupus Erythematosus, Systemic/complications/*therapy MH - Middle Aged MH - Pain Measurement MH - Raynaud Disease/etiology/*therapy MH - Skin Temperature EDAT- 2010/03/31 06:00 MHDA- 2010/06/09 06:00 CRDT- 2010/03/31 06:00 PHST- 2010/03/31 06:00 [entrez] PHST- 2010/03/31 06:00 [pubmed] PHST- 2010/06/09 06:00 [medline] AID - 28/1/49 [pii] AID - 10.1136/aim.2009.001529 [doi] PST - ppublish SO - Acupunct Med. 2010 Mar;28(1):49-51. doi: 10.1136/aim.2009.001529. PMID- 19159999 OWN - NLM STAT- MEDLINE DCOM- 20090825 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 5 DP - 2009 May TI - To compare the efficacy and safety of nifedipine sustained release with Ginkgo biloba extract to treat patients with primary Raynaud's phenomenon in South Korea; Korean Raynaud study (KOARA study). PG - 553-9 LID - 10.1007/s10067-008-1084-9 [doi] AB - This study examined the efficacy and safety of nifedipine sustained release (nifedipine SR) compared with Ginkgo biloba extract as treatment for primary Raynaud's phenomenon (RP) in Korea. Primary RP were screened and assigned to either the nifedipine SR group (Group N) or the Ginkgo biloba extract group (Group G) in the ratio of 2:1. After a run-in period of 2 weeks, patients received treatment for 8 weeks. We observed the percent improvement of the RP attack rate between before and after the 8-week treatment. Ninety-three subjects were randomly assigned. The percent improvement in Group N was 50.1% at 8 weeks after treatment, while it was 31.0% in Group G (p = 0.03). No serious adverse events occurred, and almost adverse events were mild and improved without specific treatment. nifedipine SR was more effective than Ginkgo biloba extract for treatment of primary RP in Korean patients. Both drugs were tolerable with primary RP patients. FAU - Choi, Whan-Seok AU - Choi WS AD - Department of Family Medicine, Catholic University, Seoul, Korea. FAU - Choi, Chang-Jin AU - Choi CJ FAU - Kim, Kyung-Soo AU - Kim KS FAU - Lee, Jae-Ho AU - Lee JH FAU - Song, Chan-Hee AU - Song CH FAU - Chung, Ju-Hye AU - Chung JH FAU - Ock, Sun-Myeoung AU - Ock SM FAU - Lee, Jung-Bok AU - Lee JB FAU - Kim, Chul-Min AU - Kim CM LA - eng PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20090122 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Delayed-Action Preparations) RN - 0 (Plant Extracts) RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Delayed-Action Preparations MH - Female MH - Ginkgo biloba/*metabolism MH - Humans MH - Korea MH - Male MH - Middle Aged MH - Nifedipine/*administration & dosage MH - Phytotherapy/methods MH - Plant Extracts/*administration & dosage MH - Raynaud Disease/*drug therapy/*epidemiology MH - Time Factors MH - Treatment Outcome MH - Vasodilator Agents/pharmacology EDAT- 2009/01/23 09:00 MHDA- 2009/08/26 09:00 CRDT- 2009/01/23 09:00 PHST- 2008/03/18 00:00 [received] PHST- 2008/12/27 00:00 [accepted] PHST- 2008/12/24 00:00 [revised] PHST- 2009/01/23 09:00 [entrez] PHST- 2009/01/23 09:00 [pubmed] PHST- 2009/08/26 09:00 [medline] AID - 10.1007/s10067-008-1084-9 [doi] PST - ppublish SO - Clin Rheumatol. 2009 May;28(5):553-9. doi: 10.1007/s10067-008-1084-9. Epub 2009 Jan 22. PMID- 33175296 OWN - NLM STAT- MEDLINE DCOM- 20210716 LR - 20220218 IS - 1970-9366 (Electronic) IS - 1828-0447 (Print) IS - 1828-0447 (Linking) VI - 16 IP - 4 DP - 2021 Jun TI - In systemic sclerosis patients the anxiety disorder and Raynaud's phenomenon are increased during lock down period for COVID-19 pandemic. PG - 1095-1096 LID - 10.1007/s11739-020-02557-z [doi] FAU - Gigante, Antonietta AU - Gigante A AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy. FAU - Villa, Annalisa AU - Villa A AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy. FAU - Pellicano, Chiara AU - Pellicano C AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy. FAU - Rosato, Edoardo AU - Rosato E AUID- ORCID: 0000-0002-7417-8093 AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Viale dell'Università 37, 00185, Rome, Italy. edoardo.rosato@uniroma1.it. LA - eng PT - Letter DEP - 20201111 PL - Italy TA - Intern Emerg Med JT - Internal and emergency medicine JID - 101263418 SB - IM MH - Anxiety Disorders/*epidemiology MH - COVID-19/epidemiology/*prevention & control/transmission MH - *Communicable Disease Control MH - Female MH - Humans MH - Male MH - Middle Aged MH - Psychological Distress MH - Quality of Life MH - Raynaud Disease/*psychology MH - Scleroderma, Systemic/*psychology MH - Sedentary Behavior MH - Surveys and Questionnaires PMC - PMC7656092 COIS- The authors declare no conflict of interests. EDAT- 2020/11/12 06:00 MHDA- 2021/07/17 06:00 PMCR- 2020/11/11 CRDT- 2020/11/11 12:14 PHST- 2020/08/22 00:00 [received] PHST- 2020/10/28 00:00 [accepted] PHST- 2020/11/12 06:00 [pubmed] PHST- 2021/07/17 06:00 [medline] PHST- 2020/11/11 12:14 [entrez] PHST- 2020/11/11 00:00 [pmc-release] AID - 10.1007/s11739-020-02557-z [pii] AID - 2557 [pii] AID - 10.1007/s11739-020-02557-z [doi] PST - ppublish SO - Intern Emerg Med. 2021 Jun;16(4):1095-1096. doi: 10.1007/s11739-020-02557-z. Epub 2020 Nov 11. PMID- 29909388 OWN - NLM STAT- MEDLINE DCOM- 20181107 LR - 20200615 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2018 DP - 2018 Jun 15 TI - Lymphedema secondary to limited cutaneous systemic sclerosis. LID - bcr-2017-224148 [pii] LID - 10.1136/bcr-2017-224148 [doi] LID - bcr2017224148 AB - Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema. CI - © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. FAU - Moreira, Sónia AU - Moreira S AUID- ORCID: 0000-0003-3363-0589 AD - Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Crespo, Jorge AU - Crespo J AD - Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Santos, Lèlita AU - Santos L AD - Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20180615 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 9PHQ9Y1OLM (Prednisolone) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Female MH - Humans MH - Lymphedema/*diagnostic imaging/drug therapy/etiology MH - Lymphoscintigraphy MH - Methotrexate/*therapeutic use MH - Microscopic Angioscopy MH - Middle Aged MH - Prednisolone/*therapeutic use MH - Raynaud Disease/diagnostic imaging/drug therapy/etiology MH - Scleroderma, Limited/*diagnosis/drug therapy MH - Treatment Outcome PMC - PMC6011433 OTO - NOTNLM OT - connective tissue disease OT - dermatology COIS- Competing interests: None declared. EDAT- 2018/06/18 06:00 MHDA- 2018/11/08 06:00 PMCR- 2020/06/15 CRDT- 2018/06/18 06:00 PHST- 2018/06/18 06:00 [entrez] PHST- 2018/06/18 06:00 [pubmed] PHST- 2018/11/08 06:00 [medline] PHST- 2020/06/15 00:00 [pmc-release] AID - bcr-2017-224148 [pii] AID - 10.1136/bcr-2017-224148 [doi] PST - epublish SO - BMJ Case Rep. 2018 Jun 15;2018:bcr2017224148. doi: 10.1136/bcr-2017-224148. PMID- 28343718 OWN - NLM STAT- MEDLINE DCOM- 20180416 LR - 20180416 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 144 IP - 5 DP - 2017 May TI - [Comparative evaluation of dermoscopy and capillaroscopy in Raynaud's phenomenon]. PG - 333-340 LID - S0151-9638(17)30106-0 [pii] LID - 10.1016/j.annder.2017.02.004 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) is a common cause for consultation. Capillaroscopy is a well-established technique to detect capillary abnormalities suggestive of a connective tissue disease, but it is sometimes unavailable. The aim of this study was to compare dermoscopy and capillaroscopy in the assessment of RP. METHODS: This was a prospective single-centre observational study in adult patients consulting for RP at the Hôpital Nord Franche-Comté between January 2014 and June 2015. Dermoscopy was performed at dermatological consultations and capillaroscopy was prescribed. For each capillaroscopy and dermoscopy, the following parameters were examined: normal appearance, giant capillaries, avascular areas, dystrophic capillaries or tortuosity and haemorrhages. Kappa coefficients were calculated. RESULTS: Twenty-six patients participated in this study. The kappa coefficient was 0.76 for "normal" status, 0.78 for tortuosity, 0.70 for giant capillaries, 0.48 for haemorrhage and 0.62 for avascular areas. The global kappa coefficient was 0.33. Detection of these abnormalities with capillaroscopy was significantly associated with abnormal dermoscopic status (P<0.05). The sensitivity of dermoscopy for the detection of "abnormal" capillaroscopic status was 0.87. CONCLUSION: The correlation coefficients were good. Despite poor global concordance, 80% of patients had the same status, normal or abnormal, for both capillaroscopy and dermoscopy, which resulted in the same clinical management. Dermoscopy is thus a valuable tool screening for periungual anomalies and provides support for clinical examination by the dermatologist, although the reference method continues to be capillaroscopy. CI - Copyright © 2017 Elsevier Masson SAS. All rights reserved. FAU - Moreau, J AU - Moreau J AD - Service de dermatologie, CHU de Besançon, 3, boulevard Fleming, 25000 Besançon, France. Electronic address: josephine.moreau@hotmail.fr. FAU - Dupond, A-S AU - Dupond AS AD - Service de dermatologie néphrologie, CH Montbéliard, 2, rue du Dr-Flamand, 25200 Montbéliard, France. FAU - Dan, N AU - Dan N AD - Service de dermatologie, hôpital de jour, CH Montbéliard, 2, rue du Dr-Flamand, 25200 Montbéliard, France. FAU - Untereiner, T AU - Untereiner T AD - Service de radiologie, CH Montbéliard, 2, rue du Dr-Flamand, 25200 Montbéliard, France. FAU - Vidal, C AU - Vidal C AD - Centre d'investigation clinique, CHU de Besançon, 3, boulevard Fleming, 25000 Besançon, France. FAU - Aubin, F AU - Aubin F AD - Service de dermatologie, CHU de Besançon, 3, boulevard Fleming, 25000 Besançon, France. LA - fre PT - Comparative Study PT - Journal Article PT - Observational Study TT - Évaluation comparative de la dermatoscopie et de la capillaroscopie dans le phénomène de Raynaud. DEP - 20170323 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - *Dermoscopy/methods MH - Diagnosis, Differential MH - Female MH - Hospitals, University MH - Humans MH - Male MH - *Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/pathology MH - Prospective Studies MH - Raynaud Disease/*diagnosis OTO - NOTNLM OT - Capillaroscopie OT - Capillaroscopy OT - Dermatoscopie OT - Dermoscopy OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Sclérodermie systémique OT - Systemic sclerosis EDAT- 2017/03/28 06:00 MHDA- 2018/04/17 06:00 CRDT- 2017/03/28 06:00 PHST- 2015/08/23 00:00 [received] PHST- 2017/01/15 00:00 [revised] PHST- 2017/02/20 00:00 [accepted] PHST- 2017/03/28 06:00 [pubmed] PHST- 2018/04/17 06:00 [medline] PHST- 2017/03/28 06:00 [entrez] AID - S0151-9638(17)30106-0 [pii] AID - 10.1016/j.annder.2017.02.004 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2017 May;144(5):333-340. doi: 10.1016/j.annder.2017.02.004. Epub 2017 Mar 23. PMID- 30256542 OWN - NLM STAT- MEDLINE DCOM- 20181213 LR - 20181213 IS - 2155-7780 (Electronic) IS - 2155-7780 (Linking) VI - 20 IP - 5 DP - 2018 Sep 13 TI - Lisdexamfetamine and Secondary Raynaud's Phenomenon. LID - 17l02240 [pii] LID - 10.4088/PCC.17l02240 [doi] FAU - Gnanavel, Sundar AU - Gnanavel S AD - sundar221103@yahoo.com. AD - Child and Adolescent Mental Health Services, Northumberland, Tyne and Wear NHS Foundation Trust, Morpeth, United Kingdom. LA - eng PT - Case Reports PT - Letter DEP - 20180913 PL - United States TA - Prim Care Companion CNS Disord JT - The primary care companion for CNS disorders JID - 101547532 RN - 0 (Central Nervous System Stimulants) RN - SJT761GEGS (Lisdexamfetamine Dimesylate) SB - IM MH - Adolescent MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Central Nervous System Stimulants/*adverse effects MH - Humans MH - Lisdexamfetamine Dimesylate/*adverse effects MH - Male MH - Raynaud Disease/*chemically induced EDAT- 2018/09/27 06:00 MHDA- 2018/12/14 06:00 CRDT- 2018/09/27 06:00 PHST- 2018/09/27 06:00 [entrez] PHST- 2018/09/27 06:00 [pubmed] PHST- 2018/12/14 06:00 [medline] AID - 17l02240 [pii] AID - 10.4088/PCC.17l02240 [doi] PST - epublish SO - Prim Care Companion CNS Disord. 2018 Sep 13;20(5):17l02240. doi: 10.4088/PCC.17l02240. PMID- 21467714 OWN - NLM STAT- MEDLINE DCOM- 20110721 LR - 20220321 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 50 IP - 7 DP - 2011 TI - Digital ulcers as an initial manifestation of systemic lupus erythematosus. PG - 767-9 AB - Digital ulcers (DUs) and gangrene are common skin manifestations of connective tissue diseases, especially systemic sclerosis. Raynaud's phenomenon (RP) is an almost universal manifestation of systemic sclerosis, with 95% of all patients being affected, and resulting in DUs in approximately 30% of the patients each year. Although RP may be present in approximately 25-50% of the patients, DUs are relatively rare in systemic lupus erythematosus (SLE) and never present as an initial manifestation of disease. In this case report DUs appear as the initial manifestation of SLE in a young woman with a capillaroscopic scleroderma pattern and elevated systolic pulmonary arterial pressure. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Sapienza University of Rome, Italy. FAU - Molinaro, Ilenia AU - Molinaro I FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Case Reports PT - Journal Article DEP - 20110401 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Glucocorticoids) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Capillaries/pathology MH - Female MH - *Fingers MH - Glucocorticoids/therapeutic use MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Methylprednisolone/therapeutic use MH - Raynaud Disease/diagnosis/etiology MH - Skin/blood supply MH - Skin Diseases/diagnosis/drug therapy/*etiology MH - Treatment Outcome MH - Ulcer/diagnosis/drug therapy/*etiology EDAT- 2011/04/07 06:00 MHDA- 2011/07/22 06:00 CRDT- 2011/04/07 06:00 PHST- 2011/04/07 06:00 [entrez] PHST- 2011/04/07 06:00 [pubmed] PHST- 2011/07/22 06:00 [medline] AID - JST.JSTAGE/internalmedicine/50.4617 [pii] AID - 10.2169/internalmedicine.50.4617 [doi] PST - ppublish SO - Intern Med. 2011;50(7):767-9. doi: 10.2169/internalmedicine.50.4617. Epub 2011 Apr 1. PMID- 21283969 OWN - NLM STAT- MEDLINE DCOM- 20110323 LR - 20121019 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 40 IP - 1 DP - 2011 Jan TI - Systemic sclerosis - a systematic overview: part 1 - disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance. PG - 6-19 LID - 10.1024/0301-1526/a000065 [doi] AB - Due to its high association with Raynaud's phenomenon systemic sclerosis (SSc) is probably the most common connective tissue disease seen by vascular specialists. In part 1 of our systematic overview we summarize classification concepts of scleroderma disorders, the epidemiologic and genetic burden, the complex pathophysiologic background, and the clinical features and the stage-dependent capillary microscopic features of SSc. Furthermore, we address the diagnostic recommendations propagated by the German Network for Systemic Sclerosis and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology, the European Respiratory Society, and the International Society of Heart and Lung Transplantation. FAU - Klein-Weigel, P AU - Klein-Weigel P AD - Klinik für Innere Medizin, Schwerpunkt Angiologie und kardiovaskuläre Frührehabilitation, DRK Kliniken Berlin, Germany. p.klein-weigel@drk-kliniken-berlin.de FAU - Opitz, C AU - Opitz C FAU - Riemekasten, G AU - Riemekasten G LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM CIN - Vasa. 2011 Jan;40(1):3-4. doi: 10.1024/0301-1526/a000064. PMID: 21375075 MH - Capillaries/pathology/physiopathology MH - Europe MH - Humans MH - Hypertension, Pulmonary/diagnosis/epidemiology/*etiology/physiopathology MH - Practice Guidelines as Topic MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/epidemiology/*etiology/physiopathology MH - Risk Factors MH - Scleroderma, Systemic/*classification/complications/diagnosis/epidemiology/physiopathology MH - Severity of Illness Index MH - Societies, Medical MH - Terminology as Topic MH - Ulcer/diagnosis/epidemiology/*etiology/physiopathology EDAT- 2011/02/02 06:00 MHDA- 2011/03/24 06:00 CRDT- 2011/02/02 06:00 PHST- 2011/02/02 06:00 [entrez] PHST- 2011/02/02 06:00 [pubmed] PHST- 2011/03/24 06:00 [medline] AID - 10.1024/0301-1526/a000065 [doi] PST - ppublish SO - Vasa. 2011 Jan;40(1):6-19. doi: 10.1024/0301-1526/a000065. PMID- 25189168 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20260128 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - Baseline MRA predicts the treatment response to vasodilator udenafil in patients with secondary Raynaud's phenomenon. PG - S-167-70 AB - OBJECTIVES: High-resolution MR angiography (HR-MRA) demonstrates blood flow in the digital arteries, which correlates with the severity of Raynaud's phenomenon (RP). This study investigates whether baseline HR-MRA of the hand can predict the treatment response to udenafil, a new PDE5-inhibitor, in patients with secondary RP. METHODS: Baseline MRA and Doppler ultrasound were obtained in 12 patients with secondary RP. The patients were treated with udenafil 100 mg/day for 4 weeks and changes in blood flow were measured. Blood flow on MRA was scored on a 4-point scale: 0, no visible flow; 1, visible flow to the proximal phalanx; 2, to the middle phalanx; and 3, to the distal phalanx. Peak systolic velocity (PSV) was measured to determine blood flow. Paired t-test and ANOVA were used to determine the treatment response of the different MRA scores. RESULTS: On baseline MRA, 53.3% of digital arteries had an MRA score of 0, 25.8% MRA score of 1, 9.2% MRA score of 2, and 11.6% MRA score of 3. Overall, 4-week udenafil treatment improved digital flow (p<0.05) in all MRA scores. Digital arteries with MRA score 2 showed the best response with improvement in PSV by 14.5 mm/sec (p<0.01), whereas improvement in arteries of MRA scores 1 and 3 were not better than an MRA score of 0 (all, p>0.05). CONCLUSIONS: Digital arteries with moderate blood flow observed on MRA respond best to treatment with udenalfil. Therefore, baseline MRA may help predict treatment response in patients with secondary RP. FAU - Park, J K AU - Park JK AD - Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. jinkyunpark@gmail.com. FAU - Park, E-A AU - Park EA FAU - Lee, W AU - Lee W FAU - Kim, Y K AU - Kim YK FAU - Lee, E Y AU - Lee EY FAU - Song, Y W AU - Song YW FAU - Lee, E B AU - Lee EB LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140905 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Pyrimidines) RN - 0 (Sulfonamides) RN - L5IB4XLY36 (udenafil) RN - 0 (Benzenesulfonamides) SB - IM MH - Adult MH - Cohort Studies MH - Connective Tissue Diseases/complications MH - Female MH - Fingers/*blood supply/diagnostic imaging MH - Humans MH - *Magnetic Resonance Angiography MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Prognosis MH - Pyrimidines/*therapeutic use MH - Raynaud Disease/diagnosis/*drug therapy/etiology MH - *Regional Blood Flow MH - Sulfonamides/*therapeutic use MH - Treatment Outcome MH - Ultrasonography MH - Benzenesulfonamides EDAT- 2014/09/06 06:00 MHDA- 2015/10/31 06:00 CRDT- 2014/09/06 06:00 PHST- 2014/03/27 00:00 [received] PHST- 2014/05/26 00:00 [accepted] PHST- 2014/09/06 06:00 [entrez] PHST- 2014/09/06 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 8120 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-167-70. Epub 2014 Sep 5. PMID- 17525782 OWN - NLM STAT- MEDLINE DCOM- 20070724 LR - 20111117 IS - 1533-3159 (Print) IS - 1533-3159 (Linking) VI - 10 IP - 3 DP - 2007 May TI - A case of spinal cord stimulation in Raynaud's Phenomenon: can subthreshold sensory stimulation have an effect? PG - 473-8 AB - Spinal cord stimulation is currently used to treat a variety of chronic intractable painful conditions. We report a case of severe Raynaud's phenomenon in the hands refractory to conservative treatment and responsive to diagnostic stellate ganglion block that was effectively treated with a spinal cord stimulator placed in the cervical epidural space. After capturing the affected areas with paresthesias, blood flow in the left hand and fingers significantly improved as evidenced by an increase in skin temperature, a change from cyanotic to pink appearance and concomitant reduction in pain. Moreover, the patient reported that limb ischemia and pain could be managed overnight with stimulation intensities that were below sensory perception thresholds. Thus it seems, at least in the overnight period, paresthesias were not required to maintain pain relief. This case presents a potential divergence between a requirement for paresthesias and pain relief in spinal cord stimulation therapy for the treatment of Raynaud's phenomenon. The possible role of the sympathetic nervous system in this relationship is also discussed. FAU - Benyamin, Ramsin AU - Benyamin R AD - Millennium Pain Center, Bloomington, IL 61701, USA. benyamin@millenniumpaincenter.com FAU - Kramer, Jeffery AU - Kramer J FAU - Vallejo, Ricardo AU - Vallejo R LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pain Physician JT - Pain physician JID - 100954394 SB - IM MH - Afferent Pathways/*physiology MH - Autonomic Nerve Block MH - Brachial Artery/innervation/physiopathology MH - Electric Stimulation Therapy/adverse effects/*methods/standards MH - Electrodes/standards MH - Epidural Space/anatomy & histology/physiology MH - Female MH - Hand/blood supply/innervation/physiopathology MH - Humans MH - Middle Aged MH - Neurons, Afferent/physiology MH - Pain/etiology/physiopathology MH - Pain Management MH - Paresthesia/etiology/prevention & control MH - Raynaud Disease/physiopathology/*therapy MH - Regional Blood Flow/physiology MH - Sensory Thresholds/*physiology MH - Skin Temperature/physiology MH - Spinal Cord/anatomy & histology/*physiology MH - Stellate Ganglion/physiopathology MH - Sympathetic Nervous System/physiopathology MH - Treatment Outcome EDAT- 2007/05/26 09:00 MHDA- 2007/07/25 09:00 CRDT- 2007/05/26 09:00 PHST- 2007/05/26 09:00 [pubmed] PHST- 2007/07/25 09:00 [medline] PHST- 2007/05/26 09:00 [entrez] PST - ppublish SO - Pain Physician. 2007 May;10(3):473-8. PMID- 19156432 OWN - NLM STAT- MEDLINE DCOM- 20091002 LR - 20211020 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 68 IP - 2 DP - 2009 Mar TI - [Leg ulcer in mixed connective tissue disease. Resolution during sitaxsentan therapy]. PG - 154-6 LID - 10.1007/s00393-008-0421-3 [doi] AB - Leg ulcers may result in serious morbidity in patients with connective tissue diseases and Raynaud's phenomenon (RP). We describe a 35-year-old woman with mixed connective tissue disease who suffered from leg ulcers refractory to iloprost. When the patient was treated with the selective endothelin A receptor antagonist sitaxsentan for pulmonary arterial hypertension, the ulcers improved within 4 weeks and resolved completely thereafter. In addition, severity of RP ameliorated markedly. Further evaluation of sitaxsentan in patients with connective tissue diseases suffering from ischemic skin ulcers is required. FAU - Becker, H AU - Becker H AD - Medizinische Klinik und Poliklinik D, Universitätsklinikum Münster, Albert-Schweitzer-Str. 33, 48149 Münster. beckerhe@mednet.uni-muenster.de FAU - Sunderkoetter, C AU - Sunderkoetter C FAU - Willeke, P AU - Willeke P FAU - Domschke, W AU - Domschke W FAU - Gaubitz, M AU - Gaubitz M FAU - Mohr, M AU - Mohr M LA - ger PT - Case Reports PT - Journal Article TT - Unterschenkelulzera bei Sharp-Syndrom. Abheilung unter Sitaxentan. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Antihypertensive Agents) RN - 0 (Isoxazoles) RN - 0 (Thiophenes) RN - J9QH779MEM (sitaxsentan) SB - IM MH - Adult MH - Antihypertensive Agents/administration & dosage MH - Female MH - Humans MH - Isoxazoles/*administration & dosage MH - Leg Ulcer/*complications/*drug therapy MH - Raynaud Disease/*complications/*drug therapy MH - Scleroderma, Systemic/*complications/*drug therapy MH - Thiophenes/*administration & dosage MH - Treatment Outcome EDAT- 2009/01/22 09:00 MHDA- 2009/10/03 06:00 CRDT- 2009/01/22 09:00 PHST- 2009/01/22 09:00 [entrez] PHST- 2009/01/22 09:00 [pubmed] PHST- 2009/10/03 06:00 [medline] AID - 10.1007/s00393-008-0421-3 [doi] PST - ppublish SO - Z Rheumatol. 2009 Mar;68(2):154-6. doi: 10.1007/s00393-008-0421-3. PMID- 19684154 OWN - NLM STAT- MEDLINE DCOM- 20100211 LR - 20091012 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 36 IP - 10 DP - 2009 Oct TI - Laser Doppler perfusion imaging is useful in the study of Raynaud's phenomenon and improves the capillaroscopic diagnosis. PG - 2257-63 LID - 10.3899/jrheum.090187 [doi] AB - OBJECTIVE: To investigate capillary morphology and skin blood flow of dorsal hands by nailfold videocapillaroscopy (NVC) and laser Doppler perfusion imaging (LDPI), respectively, in patients with primary Raynaud's phenomenon (PRP) and systemic sclerosis (SSc) and to compare the results with those obtained in healthy controls. METHODS: The study group consisted of 142 patients with SSc, 88 patients with PRP, and 147 healthy controls. NVC was performed in all the groups examined. In patients with SSc the capillaroscopic pattern was classified as early, active, or late group pattern. A baseline skin blood flow determination of the dorsum of the subject's hands was acquired through a low-energy 670 nm Lisca Laser Doppler Perfusion Imager. RESULTS: In the healthy controls the perfusion distribution pattern was homogeneous, with a proximal-distal perfusion gradient. In patients with PRP, the perfusion distribution pattern was homogeneous, but the proximal-distal perfusion gradient was absent. Finally, in patients with SSc the perfusion distribution pattern was dyshomogeneous and a proximal-distal gradient was absent. The minimum perfusion, mean perfusion, maximum perfusion, and standard deviation, calculated as variation by means of each measurement site, were significantly different in all the groups examinated. CONCLUSION: NVC represents the best method to analyze microvascular damage in rheumatic diseases. LDPI improves the evaluation of vascular damage in patients with SSc. The LDPI and the capillaroscopic images fully matched the definition of the various stages of vascular digital damage in SSc. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center, Sapienza University of Rome, Rome, Italy. FAU - Borghese, Federica AU - Borghese F FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article DEP - 20090814 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - Humans MH - Laser-Doppler Flowmetry/*methods MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/*physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/physiopathology MH - Skin/blood supply EDAT- 2009/08/18 09:00 MHDA- 2010/02/12 06:00 CRDT- 2009/08/18 09:00 PHST- 2009/08/18 09:00 [entrez] PHST- 2009/08/18 09:00 [pubmed] PHST- 2010/02/12 06:00 [medline] AID - jrheum.090187 [pii] AID - 10.3899/jrheum.090187 [doi] PST - ppublish SO - J Rheumatol. 2009 Oct;36(10):2257-63. doi: 10.3899/jrheum.090187. Epub 2009 Aug 14. PMID- 31209194 OWN - NLM STAT- MEDLINE DCOM- 20200102 LR - 20200102 IS - 1735-8604 (Electronic) IS - 1735-8582 (Linking) VI - 13 IP - 3 DP - 2019 May TI - Systemic Sclerosis with Focus on Scleroderma Renal Crisis. PG - 207-210 AB - Known as systemic sclerosis (SSc), this autoimmune rheumatic disease has vast pathogenesis on many organs, including kidneys. It can lead to the point where the patient's survival relies entirely on dialysis. This report has basically focused on scleroderma renal crisis (SRC), which is the most serious renal manifestation of SSc, characterized by renal failure and sudden onset of hypertension. A 44-year-old man was hospitalized with hypertension, headache, vertigo, nausea, rhinorrhea, reflux, dysphagia, dyspnea (Fc II), visual impairment, mechanical arthralgia, and edema (+3) accompanied by a rare skin lesion. Raynaud's phenomenon was also remarkable in fingers and toes. According to signs and symptoms, SSc diagnosed and the proper treatment was applied. It is of great importance that in the case of malignant hypertension in patients with scleroderma, renal crisis always be kept in mind. FAU - Pasha, Farahnaz AU - Pasha F FAU - Abazari, Setayesh AU - Abazari S FAU - Bikarannejad, Pouria AU - Bikarannejad P FAU - Zabolian, Amirhossein AU - Zabolian A AD - Young Researchers and Elite Club, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran. Fzr2000_0007@yahoo.com. LA - eng PT - Case Reports PT - Journal Article PL - Iran TA - Iran J Kidney Dis JT - Iranian journal of kidney diseases JID - 101316967 SB - IM MH - Acute Kidney Injury/*etiology/physiopathology MH - Adult MH - Edema MH - Humans MH - Hypertension/*etiology/physiopathology MH - Male MH - Myocardial Perfusion Imaging MH - Raynaud Disease/*etiology/physiopathology MH - Scleroderma, Systemic/*complications/*diagnosis EDAT- 2019/06/19 06:00 MHDA- 2020/01/03 06:00 CRDT- 2019/06/19 06:00 PHST- 2018/10/09 00:00 [received] PHST- 2019/01/25 00:00 [accepted] PHST- 2018/10/12 00:00 [revised] PHST- 2019/06/19 06:00 [entrez] PHST- 2019/06/19 06:00 [pubmed] PHST- 2020/01/03 06:00 [medline] AID - 4342/1069 [pii] PST - ppublish SO - Iran J Kidney Dis. 2019 May;13(3):207-210. PMID- 25776043 OWN - NLM STAT- MEDLINE DCOM- 20151221 LR - 20250711 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 5 IP - 3 DP - 2015 Mar 16 TI - Prevalence, risk factors and associations of primary Raynaud's phenomenon: systematic review and meta-analysis of observational studies. PG - e006389 LID - 10.1136/bmjopen-2014-006389 [doi] LID - e006389 AB - OBJECTIVE: To systematically review the literature with regard to the prevalence, incidence, risk factors and associations of primary Raynaud's phenomenon (PRP). METHOD: A systematic review of the literature of observational studies for PRP was undertaken using five electronic databases. Any studies reporting prevalence, incidence and risk factors of PRP were collected. Relative risk or OR and 95% CI were extracted or calculated to present the association between risk factors and PRP. Random effects model was used to pool the results. RESULTS: 33 articles assessing a total of 33,733 participants were included in this analysis (2 cohort, 17 cross-sectional and 14 case-control studies). The pooled prevalence of PRP was 4.85% (95% CI 2.08% to 8.71%) in the general population. The pooled annual incidence of PRP was 0.25% (95% CI 0.19% to 0.32%). Risk factors and associations for PRP included female gender (OR=1.65, 95% CI 1.42 to 1.91), family history (OR=16.6, 95% CI 7.44 to 36.8), smoking (OR=1.27, 95% CI 1.06 to 1.53), manual occupation (OR=2.66 95% CI 1.73 to 4.08), migraine (OR=4.02, 95% CI 2.62 to 6.17), cardiovascular disease (OR=1.69, 95% CI 1.22 to 2.34) and marital status (married, OR=0.60, 95% CI 0.43 to 0.83). The definition of PRP varied considerably between studies. CONCLUSIONS: This is the first systematic review of the prevalence, incidence, risk factors and associations of PRP. Further study using uniform strict criteria for the condition is required to confirm these findings, particularly the possible association with cardiovascular disease. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. FAU - Garner, Rozeena AU - Garner R AD - Department of Rheumatology, Queens Medical Centre, Nottingham, UK. FAU - Kumari, Rakesh AU - Kumari R AD - Department of Rheumatology, Queens Medical Centre, Nottingham, UK. FAU - Lanyon, Peter AU - Lanyon P AD - Department of Rheumatology, Queens Medical Centre, Nottingham, UK. FAU - Doherty, Michael AU - Doherty M AD - Department of Rheumatology, Queens Medical Centre, Nottingham, UK Department of Academic Rheumatology, University of Nottingham, Nottingham, UK. FAU - Zhang, Weiya AU - Zhang W AD - Department of Academic Rheumatology, University of Nottingham, Nottingham, UK. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20150316 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - Cold Temperature MH - Humans MH - Observational Studies as Topic MH - Prevalence MH - Raynaud Disease/complications/*epidemiology MH - Risk Factors PMC - PMC4368987 OTO - NOTNLM OT - EPIDEMIOLOGY OT - RHEUMATOLOGY OT - STATISTICS & RESEARCH METHODS EDAT- 2015/03/18 06:00 MHDA- 2015/12/22 06:00 PMCR- 2015/03/16 CRDT- 2015/03/18 06:00 PHST- 2015/03/18 06:00 [entrez] PHST- 2015/03/18 06:00 [pubmed] PHST- 2015/12/22 06:00 [medline] PHST- 2015/03/16 00:00 [pmc-release] AID - bmjopen-2014-006389 [pii] AID - 10.1136/bmjopen-2014-006389 [doi] PST - epublish SO - BMJ Open. 2015 Mar 16;5(3):e006389. doi: 10.1136/bmjopen-2014-006389. PMID- 23521585 OWN - NLM STAT- MEDLINE DCOM- 20140610 LR - 20131014 IS - 1600-0846 (Electronic) IS - 0909-752X (Linking) VI - 19 IP - 4 DP - 2013 Nov TI - Nailfold capillaroscopy in systemic sclerosis: is there any difference between videocapillaroscopy and dermatoscopy? PG - 446-9 LID - 10.1111/srt.12067 [doi] AB - BACKGROUND: Vasculopathy is known to destroy nailfold capillary pattern (NCP) in systemic sclerosis (SSc). There are several methods for the evaluation of NCP of which the most common are dermatoscopy and videocapillaroscopy (VCAP). No study has been reported in the literature comparing these two techniques for their diagnostic value. OBJECTIVE: To compare the diagnostic value of dermatoscopy and VCAP which are widely used to determine changes in the NCP in SSc patients. METHODS: A total of 382 nailfolds were visualized. NCP was evaluated in 39 SSc patients using dermatoscopy and VCAP. Defined dermatoscopic groups were matched with early, active and late phase NCP groups determined by VCAP for comparisons. RESULTS: Both dermatoscopy and VCAP demonstrated distinct NCP of SSc efficiently. According to dermatoscopic NCP, capillary dilatation, giant capillaries and disrupted vascular configuration were able to be visualized. VCAP revealed early phase NCP in N = 8 (20,5%), active phase in N = 18 (46,2%) and late phase NCP in N = 13 (33.3%) of the patients. Statistical evaluation of grouped data resulted a Cohen kappa value (K) = 0,527. Although VCAP was able to facilitate a more detailed evaluation of NCP, there was no difference between dermatoscopy and VCAP for the identification of distinct NCP in SSc. CONCLUSION: We suggest that dermatoscopy is efficient enough to identify pathognomonic changes in NCP in SSc as well as VCAP and find dermatoscopy as a very easy applicable and convenient method than VCAP although VCAP facilitates a more detailed evaluation of NCP. CI - © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Dogan, Sibel AU - Dogan S AD - Department of Dermatology, Faculty of Medicine, Hacettepe University, Sıhhiye, Ankara, Turkey. FAU - Akdogan, Ali AU - Akdogan A FAU - Atakan, Nilgün AU - Atakan N LA - eng PT - Clinical Trial PT - Comparative Study PT - Journal Article DEP - 20130325 PL - England TA - Skin Res Technol JT - Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) JID - 9504453 SB - IM MH - Adult MH - Capillaries/pathology/physiology MH - Dermoscopy/instrumentation/*methods/standards MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/instrumentation/*methods/standards MH - Microscopy, Video/instrumentation/*methods/standards MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/diagnosis/pathology/physiopathology MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis/pathology/physiopathology MH - Sensitivity and Specificity OTO - NOTNLM OT - dermatoscopy OT - nailfold OT - raynaud's phenomenon OT - scleroderma OT - videocapillaroscopy EDAT- 2013/03/26 06:00 MHDA- 2014/06/11 06:00 CRDT- 2013/03/26 06:00 PHST- 2013/02/23 00:00 [accepted] PHST- 2013/03/26 06:00 [entrez] PHST- 2013/03/26 06:00 [pubmed] PHST- 2014/06/11 06:00 [medline] AID - 10.1111/srt.12067 [doi] PST - ppublish SO - Skin Res Technol. 2013 Nov;19(4):446-9. doi: 10.1111/srt.12067. Epub 2013 Mar 25. PMID- 30916482 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20240715 IS - 2051-817X (Electronic) IS - 2051-817X (Linking) VI - 7 IP - 6 DP - 2019 Mar TI - Rapid free thiol rebound is a physiological response following cold-induced vasoconstriction in healthy humans, primary Raynaud and systemic sclerosis. PG - e14017 LID - 10.14814/phy2.14017 [doi] LID - e14017 AB - Raynaud's phenomenon (RP) is often the first sign of systemic sclerosis (SSc). Molecular mechanisms involved are incompletely understood, but reactive oxygen, nitrogen, and sulfur species are thought to play an important role in the pathogenesis of SSc. Free thiol groups play a protective role against oxidative stress and may represent an attractive therapeutic target. We aimed to investigate the effects of hypothermia-induced vasoconstriction on the responsiveness of redox-related markers. Thirty participants (n = 10/group [SSc, primary Raynaud's phenomenon (PRP), healthy controls (HC)]) were included in this study. Fingertip photoelectric plethysmography was performed during a standardized cooling and recovery experiment. Venous blood was collected at four predetermined time points. Free thiols, NO-derived species (nitros(yl)ated species, nitrite, nitrate), sulfate and endothelin-1 were measured. Lower baseline concentrations of free thiols were observed in PRP and SSc patients (HC: 5.87 [5.41-5.99] μmol/g; PRP: 5.17 [4.74-5.61]; SSc 5.28 [4.75-5.80], P = 0.04). Redox-related markers remained unchanged during cooling. However, an unexpected increase in systemic free thiol concentrations was observed in all groups during the recovery phase. The response of this marker differed between groups, with a higher increase found in SSc patients (HC Δ = 1.30 [1.48-1.17]; PRP Δ = 1.04 [1.06-1.03]; SSc Δ = 1.72 [1.13-1.49], P = 0.04). NO-derived species, sulfate and endothelin-1 levels remained unchanged throughout the recovery phase. This exploratory study sheds light on the rapid responsiveness of systemic free thiol concentrations following reperfusion, which may reflect overall redox balance. The robust response to reperfusion in SSc patients suggests that reductive systems involved in this response are functionally intact in these patients. CI - © 2019 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society. FAU - Abdulle, Amaal Eman AU - Abdulle AE AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - van Roon, Anniek M AU - van Roon AM AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Smit, Andries J AU - Smit AJ AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Pasch, Andreas AU - Pasch A AD - Department of Biomedical Research, University of Bern, Bern, Switzerland. FAU - van Meurs, Matijs AU - van Meurs M AD - Department of Critical Care, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Bootsma, Hendrika AU - Bootsma H AD - Department of Rheumatology and Clinical Immunology, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Bakker, Stephan J L AU - Bakker SJL AD - Department of Internal Medicine, Division of Nephrology, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Said, Mohammad Y AU - Said MY AD - Department of Internal Medicine, Division of Nephrology, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Fernandez, Bernadette O AU - Fernandez BO AD - Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom. FAU - Feelisch, Martin AU - Feelisch M AD - Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom. FAU - van Goor, Harry AU - van Goor H AD - Department of Pathology and Medical Biology, Section Pathology, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. FAU - Mulder, Douwe J AU - Mulder DJ AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen - University Medical Centre Groningen, Groningen, The Netherlands. LA - eng PT - Comparative Study PT - Journal Article PL - United States TA - Physiol Rep JT - Physiological reports JID - 101607800 RN - 0 (Antioxidants) RN - 0 (Sulfhydryl Compounds) SB - IM MH - Adaptation, Physiological MH - Adult MH - Aged MH - Antioxidants/*metabolism MH - Case-Control Studies MH - *Cold Temperature MH - Female MH - Humans MH - Male MH - Middle Aged MH - Oxidation-Reduction MH - Pilot Projects MH - Raynaud Disease/*blood/diagnosis/*physiopathology MH - Scleroderma, Systemic/*blood/diagnosis/*physiopathology MH - Sulfhydryl Compounds/*blood MH - Time Factors MH - *Vasoconstriction PMC - PMC6436142 OTO - NOTNLM OT - Free thiols OT - Raynaud's phenomenon OT - nitric oxide OT - redox system OT - systemic sclerosis COIS- The authors declared no conflicts of interest. EDAT- 2019/03/28 06:00 MHDA- 2020/04/28 06:00 PMCR- 2019/03/27 CRDT- 2019/03/28 06:00 PHST- 2019/01/22 00:00 [received] PHST- 2019/01/24 00:00 [revised] PHST- 2019/01/28 00:00 [accepted] PHST- 2019/03/28 06:00 [entrez] PHST- 2019/03/28 06:00 [pubmed] PHST- 2020/04/28 06:00 [medline] PHST- 2019/03/27 00:00 [pmc-release] AID - PHY214017 [pii] AID - 10.14814/phy2.14017 [doi] PST - ppublish SO - Physiol Rep. 2019 Mar;7(6):e14017. doi: 10.14814/phy2.14017. PMID- 21747356 OWN - NLM STAT- MEDLINE DCOM- 20111114 LR - 20110712 IS - 1827-1839 (Electronic) IS - 0392-9590 (Linking) VI - 30 IP - 4 DP - 2011 Aug TI - Peripheral nerve changes assessed by conduction velocity distribution in patients with primary Raynaud's phenomenon and dysautonomia. PG - 375-9 AB - AIM: Different mechanisms (neural and intravascular) are thought to be important in the pathogenesis of Raynaud's phenomenon (RP). In a previous study we confirmed autonomic nervous system impairment in patients with primary RP, but the pathogenic role of peripheral nerves remained unclear. The aim of the current study was an electrophysiological analysis of peripheral nerves using both standard conduction velocity and the conduction velocity distribution (CVD) in patients with primary RP in order to investigate the causes of dysautonomia. METHODS: We examined 34 patients with primary RP and dysautonomia and 31 sex- and age-matched controls. Standard motor and sensory conduction tests in ulnar and peroneal (sural) nerves and a CVD test in the same nerves were performed. RESULTS: Clinically, none of the patients had motor symptoms, while 35.3% of them presented sensory neuropathy. Standard neurographic tests were within the normal limits except for the significant prolongation of mean sensory latency in both examined nerves. CVD revealed significant slowing of motor conduction velocity in all the conduction values, e.g. in the 10th, 50th, and 90th percentiles of velocity. There were no differences in the width of the velocity distribution in the patient group and controls. CONCLUSION: The results of CVD indicated the presence of generalized subclinical peripheral motor nerve impairment (subclinical polyneuropathy) in patients with primary RP and dysautonomia. Based on the present and previous studies, we conclude that the mechanism of autonomic dysfunction in primary RP is mixed, resulting from both central and peripheral neural abnormalities. FAU - Koszewicz, M AU - Koszewicz M AD - Department of Neurology, Wrocław Medical University, Wrocław, Poland. magda.koszewicz@onet.pl FAU - Gosk-Bierska, I AU - Gosk-Bierska I FAU - Jerzy, G AU - Jerzy G FAU - Bilińska, M AU - Bilińska M FAU - Podemski, R AU - Podemski R FAU - Budrewicz, S AU - Budrewicz S FAU - Adamiec, R AU - Adamiec R LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 SB - IM MH - Action Potentials MH - Adult MH - Case-Control Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Neural Conduction MH - Peroneal Nerve/*physiopathology MH - Poland MH - Primary Dysautonomias/diagnosis/*physiopathology MH - Raynaud Disease/diagnosis/*physiopathology MH - Reaction Time MH - Sensation MH - Time Factors MH - Ulnar Nerve/*physiopathology MH - Young Adult EDAT- 2011/07/13 06:00 MHDA- 2011/11/15 06:00 CRDT- 2011/07/13 06:00 PHST- 2011/07/13 06:00 [entrez] PHST- 2011/07/13 06:00 [pubmed] PHST- 2011/11/15 06:00 [medline] AID - R34112596 [pii] PST - ppublish SO - Int Angiol. 2011 Aug;30(4):375-9. PMID- 31670800 OWN - NLM STAT- MEDLINE DCOM- 20200629 LR - 20210403 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 59 IP - 3 DP - 2020 Mar 1 TI - Drug initiation and escalation strategies of vasodilator therapies for Raynaud's phenomenon: can we treat to target? PG - 464-466 LID - 10.1093/rheumatology/kez522 [doi] FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. AD - Centre for Musculoskeletal Research, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan Scleroderma Program, Department of Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Editorial PT - Research Support, N.I.H., Extramural PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - Raynaud Disease/*drug therapy MH - Vasodilator Agents/*therapeutic use PMC - PMC7998338 EDAT- 2019/11/02 06:00 MHDA- 2020/07/01 06:00 PMCR- 2020/10/31 CRDT- 2019/11/01 06:00 PHST- 2019/08/28 00:00 [received] PHST- 2019/09/10 00:00 [revised] PHST- 2019/09/30 00:00 [accepted] PHST- 2019/11/02 06:00 [pubmed] PHST- 2020/07/01 06:00 [medline] PHST- 2019/11/01 06:00 [entrez] PHST- 2020/10/31 00:00 [pmc-release] AID - 5610598 [pii] AID - kez522 [pii] AID - 10.1093/rheumatology/kez522 [doi] PST - ppublish SO - Rheumatology (Oxford). 2020 Mar 1;59(3):464-466. doi: 10.1093/rheumatology/kez522. PMID- 31679095 OWN - NLM STAT- MEDLINE DCOM- 20200930 LR - 20231018 IS - 1591-9528 (Electronic) IS - 1591-8890 (Linking) VI - 20 IP - 1 DP - 2020 Feb TI - Evaluation of the influence of social, demographic, environmental, work-related factors and/or lifestyle habits on Raynaud's phenomenon: a case-control study. PG - 31-37 LID - 10.1007/s10238-019-00589-0 [doi] AB - Raynaud's phenomenon (RP) is a clinical disorder characterized by recurrent, reversible episodes of digital vasospasm. RP can be classified as primary (pRP) or secondary, depending on whether it occurs as a benign condition (not disease-associated) or is associated with other diseases, mainly of the connective tissues. In both cases, it can be triggered by environmental factors, as indicated by the increased incidence of pRP episodes following exposure to cold, vibration injury or chemicals. The purpose of this prospective case-control study was to assess, in an Italian cohort of 132 pRP patients, the association of the phenomenon with demographic, lifestyle habits, environmental and work-related factors. Compared to healthy controls, pRP was found to be inversely associated with the use of contact lenses (OR = 0.4; p = 0.004) and of chlorous-based disinfectants (OR = 0.3; p < 0.001) and directly associated with the presence of prosthesis implants (OR = 5.3; p = 0.001) and the use of hydrogen peroxide-based compounds (OR = 2.6; p = 0.002), suggesting that the latter should be avoided in RP affected patients. Multivariate and multivariable analysis confirmed the associations. Further investigations are needed to understand the mechanism(s) underlying these findings. FAU - Prete, M AU - Prete M AD - Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Science and Human Oncology (DIMO), University of Bari Medical School, Piazza G. Cesare 11, I-70124, Bari, Italy. FAU - Favoino, E AU - Favoino E AD - Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Science and Human Oncology (DIMO), University of Bari Medical School, Piazza G. Cesare 11, I-70124, Bari, Italy. FAU - Giacomelli, R AU - Giacomelli R AD - Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Afeltra, A AU - Afeltra A AD - Clinical Medicine and Rheumatology Department, Campus Bio-Medico, University of Roma, Roma, Italy. FAU - Cantatore, F P AU - Cantatore FP AD - Rheumatology Section, Department of Medical and Surgical Sciences, University of Foggia Medical School, Foggia, Italy. FAU - Bruno, C AU - Bruno C AD - Rheumatology Research Unit, Department of Health Sciences, University of Catanzaro, Catanzaro, Italy. FAU - Corrado, A AU - Corrado A AD - Rheumatology Section, Department of Medical and Surgical Sciences, University of Foggia Medical School, Foggia, Italy. FAU - Emmi, L AU - Emmi L AD - Department of Neurosciences, Psychology, Pharmacology and Child Health (NEUROFARBA), University of Firenze, Firenze, Italy. FAU - Emmi, G AU - Emmi G AD - Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy. FAU - Grembiale, R D AU - Grembiale RD AD - Rheumatology Research Unit, Department of Health Sciences, University of Catanzaro, Catanzaro, Italy. FAU - Navarini, L AU - Navarini L AD - Clinical Medicine and Rheumatology Department, Campus Bio-Medico, University of Roma, Roma, Italy. FAU - Marcoccia, A AU - Marcoccia A AD - UOSD of Ischemic Microangiopathy and Sclerodermic Ulcers, Sandro Pertini Hospital, Roma, Italy. FAU - Liakouli, V AU - Liakouli V AD - Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Riccardi, A AU - Riccardi A AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Napoli, Italy. FAU - Valentini, G AU - Valentini G AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Napoli, Italy. FAU - Perosa, F AU - Perosa F AUID- ORCID: 0000-0002-4072-4244 AD - Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Science and Human Oncology (DIMO), University of Bari Medical School, Piazza G. Cesare 11, I-70124, Bari, Italy. federico.perosa@uniba.it. CN - GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale) LA - eng PT - Journal Article DEP - 20191102 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Disinfectants) RN - BBX060AN9V (Hydrogen Peroxide) SB - IM MH - Adult MH - Case-Control Studies MH - Contact Lenses/*statistics & numerical data MH - Disinfectants/chemistry MH - Female MH - Humans MH - Hydrogen Peroxide/*adverse effects MH - Incidence MH - Italy/epidemiology MH - Life Style MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Prospective Studies MH - Prostheses and Implants/*adverse effects/statistics & numerical data MH - Raynaud Disease/*epidemiology/etiology OTO - NOTNLM OT - Contact lenses OT - Environmental factors OT - Hydrogen peroxide-based compounds OT - Lifestyle habits OT - Prosthesis implants OT - Raynaud’s phenomenon EDAT- 2019/11/05 06:00 MHDA- 2020/10/02 06:00 CRDT- 2019/11/04 06:00 PHST- 2019/05/20 00:00 [received] PHST- 2019/10/22 00:00 [accepted] PHST- 2019/11/05 06:00 [pubmed] PHST- 2020/10/02 06:00 [medline] PHST- 2019/11/04 06:00 [entrez] AID - 10.1007/s10238-019-00589-0 [pii] AID - 10.1007/s10238-019-00589-0 [doi] PST - ppublish SO - Clin Exp Med. 2020 Feb;20(1):31-37. doi: 10.1007/s10238-019-00589-0. Epub 2019 Nov 2. PMID- 25182165 OWN - NLM STAT- MEDLINE DCOM- 20141209 LR - 20211021 IS - 1471-2377 (Electronic) IS - 1471-2377 (Linking) VI - 14 DP - 2014 Sep 3 TI - Guillain-Barré syndrome presenting with Raynaud's phenomenon: a case report. PG - 174 LID - 10.1186/s12883-014-0174-3 [doi] AB - BACKGROUND: Guillain-Barré syndrome is an immune mediated acute inflammatory polyradiculo-neuropathy involving the peripheral nervous system. Commonest presentation is acute or subacute flaccid ascending paralysis of limbs. Rarely autonomic dysfunction can be the presenting feature of Guillain-Barré syndrome. Raynaud's phenomenon, although had been described in relation to many disease conditions, has not been described in association with Guillain-Barré syndrome up to date. CASE PRESENTATION: We report the first case of Guillain-Barré syndrome presenting with Raynaud's phenomenon in a 21-year-old previously well boy. New onset Raynaud's phenomenon was experienced followed by acute ascending flaccid paralysis of lower limbs and upper limbs together with palpitations and postural giddiness. Nerve conduction studies showed acute inflammatory demyelinating polyneuropathy with cerebrospinal fluid cyto-protein dissociation. He was treated with intravenous immunoglobulin and showed a satisfactory clinical recovery of muscle weakness, Raynaud's phenomenon and autonomic disturbances. CONCLUSION: Guillain-Barré syndrome presenting with Raynaud's phenomenon is not being reported in literature previously. Although the underlying mechanism is not fully understood, Raynaud's phenomenon should prompt the physician to consider Guillain-Barré syndrome with a complimentary clinical picture. FAU - Gunatilake, Sonali Sihindi Chapa AU - Gunatilake SS AD - Teaching Hospital, Kandy, Sri Lanka. sonaligunatilake@gmail.com. FAU - Wimalaratna, Harith AU - Wimalaratna H LA - eng PT - Case Reports PT - Journal Article DEP - 20140903 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Guillain-Barre Syndrome/*complications/drug therapy MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - Male MH - Raynaud Disease/*etiology MH - Young Adult PMC - PMC4236670 EDAT- 2014/09/04 06:00 MHDA- 2014/12/15 06:00 PMCR- 2014/09/03 CRDT- 2014/09/04 06:00 PHST- 2014/05/21 00:00 [received] PHST- 2014/08/29 00:00 [accepted] PHST- 2014/09/04 06:00 [entrez] PHST- 2014/09/04 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] PHST- 2014/09/03 00:00 [pmc-release] AID - s12883-014-0174-3 [pii] AID - 10.1186/s12883-014-0174-3 [doi] PST - epublish SO - BMC Neurol. 2014 Sep 3;14:174. doi: 10.1186/s12883-014-0174-3. PMID- 22711501 OWN - NLM STAT- MEDLINE DCOM- 20130722 LR - 20211021 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 43 IP - 3 DP - 2012 Dec TI - Impact of hallmark autoantibody reactivity on early diagnosis in scleroderma. PG - 249-55 LID - 10.1007/s12016-012-8331-1 [doi] AB - Raynaud's phenomenon often precedes the diagnosis of systemic sclerosis and is the first symptom of the disease in many cases. Antinuclear antibody positivity can assist in the early identification of cases of isolated Raynaud's phenomenon likely to progress to systemic sclerosis. However, the specific differences between rate of progression for different scleroderma hallmark antibodies is less clear. We review the predictive potential of ANA positivity and nailfold capillaroscopy for identifying cases of Raynaud's phenomenon which may progress to connective tissue diseases. We also have reviewed data from our own large scleroderma cohort to explore the relationship between antibody subtype and time to development of SSc. Duration of pre-existing Raynaud's phenomenon may be an important determinant of the profile of systemic sclerosis cases identified through screening. Ninety-five percent of our patients with isolated Raynaud's phenomenon, negative autoimmune serology on more than one visit and normal capillaroscopy score showed no progression to connective tissue disease. Duration of antecedent Raynaud's phenomenon differs between disease subsets and scleroderma-specific ANA patterns. FAU - Moinzadeh, Pia AU - Moinzadeh P AD - Centre for Rheumatology, Royal Free Hospital and UCL Medical School, London, UK. FAU - Nihtyanova, Svetlana I AU - Nihtyanova SI FAU - Howell, Kevin AU - Howell K FAU - Ong, Voon H AU - Ong VH FAU - Denton, Christopher P AU - Denton CP LA - eng GR - Arthritis Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*immunology MH - Disease Progression MH - Early Diagnosis MH - Humans MH - Raynaud Disease/*diagnosis/*immunology MH - Scleroderma, Localized/*diagnosis/*immunology EDAT- 2012/06/20 06:00 MHDA- 2013/07/23 06:00 CRDT- 2012/06/20 06:00 PHST- 2012/06/20 06:00 [entrez] PHST- 2012/06/20 06:00 [pubmed] PHST- 2013/07/23 06:00 [medline] AID - 10.1007/s12016-012-8331-1 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2012 Dec;43(3):249-55. doi: 10.1007/s12016-012-8331-1. PMID- 40073248 OWN - NLM STAT- MEDLINE DCOM- 20250701 LR - 20260525 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 7 DP - 2025 Jul 1 TI - Using a smartphone app to monitor Raynaud's attacks and quantify skin colour changes-towards objective outcome measures for Raynaud's. PG - 4236-4244 LID - 10.1093/rheumatology/keaf141 [doi] AB - OBJECTIVES: Our overall aim was to develop a smartphone app to collect photographic images of Raynaud's phenomenon (RP) attacks alongside patient reported outcome measures (PROMs). Specific objectives included assessing the feasibility of patients documenting RP attacks using mobile phones, developing image analysis methods to document colour change, and comparing photographic parameters with 'non-imaging' app and paper diary parameters. METHODS: In study 1, 36 patients with systemic sclerosis (SSc)-related RP photographed RP attacks over 15 days as well as completing an RP paper diary. In study 2, 40 patients with either SSc-related or primary RP used a smartphone app to collect, over 15 days, photographic images and data including frequency and severity of attacks. In both studies, mean colour change during each attack (indicating severity) was quantified by the Bhattacharyya distance. RESULTS: In study 1, 24/36 (67%) patients completed the study of whom 22 photographed at least one RP attack (median number of attacks 12.5, range 1-53); 18/24 (75%) patients preferred phone to paper diary documentation. 'Photographic' and 'paper diary' frequency (but not severity) of attacks correlated strongly, with a correlation coefficient of 0.71 (95% CI: 0.41, 0.87); P = 0.002. In study 2, 36/40 (90%) completed the study, providing 1747 usable images from 456 RP attacks. ANOVA demonstrated that RP colour change was significantly different with different values of RP attack severity (P <0.001). CONCLUSION: Collecting photographs of RP attacks and PROMS via a smartphone app was feasible and preferred by patients to data collection via paper diary, providing proof-of-concept for validation studies of app-based outcome measures for RP. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - Rheumatology Department, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Manning, Joanne AU - Manning J AD - Rheumatology Department, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Dickinson, Mark AU - Dickinson M AD - Photon Science Institute, The University of Manchester, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Rheumatology Department, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Taylor, Chris J AU - Taylor CJ AD - Centre for Imaging Sciences, Institute of Population Health, The University of Manchester, Manchester, UK. LA - eng GR - 20677/VAC_/Versus Arthritis/United Kingdom GR - NIHR/ GR - NIHR203308/Manchester Biomedical Research Centre/ GR - Department of Health and Social Care/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Raynaud Disease/etiology/diagnosis MH - *Mobile Applications MH - Female MH - Male MH - *Smartphone MH - Middle Aged MH - Photography MH - Adult MH - Patient Reported Outcome Measures MH - Feasibility Studies MH - Aged MH - Severity of Illness Index MH - *Skin Pigmentation MH - Scleroderma, Systemic/complications PMC - PMC12212912 OTO - NOTNLM OT - Raynaud’s phenomenon OT - outcome measure OT - smartphone app OT - smartphone photography OT - systemic sclerosis EDAT- 2025/03/12 18:17 MHDA- 2025/07/02 04:06 PMCR- 2025/03/12 CRDT- 2025/03/12 15:03 PHST- 2024/10/07 00:00 [received] PHST- 2025/02/13 00:00 [accepted] PHST- 2025/07/02 04:06 [medline] PHST- 2025/03/12 18:17 [pubmed] PHST- 2025/03/12 15:03 [entrez] PHST- 2025/03/12 00:00 [pmc-release] AID - 8071877 [pii] AID - keaf141 [pii] AID - 10.1093/rheumatology/keaf141 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Jul 1;64(7):4236-4244. doi: 10.1093/rheumatology/keaf141. PMID- 18604184 OWN - NLM STAT- MEDLINE DCOM- 20081017 LR - 20080707 IS - 0303-464X (Print) IS - 0303-464X (Linking) VI - 33 IP - 2 DP - 2008 Apr-Jun TI - [Nailfold capillaroscopy in the evaluation of Raynaud's phenomenon and undifferentiated connective tissue disease]. PG - 203-9 AB - BACKGROUND: Microvascular abnormalities involved in the pathogenic mechanism of several connective tissue disorders can be detected by nailfold capillaroscopy. OBJECTIVES: Evaluation of the interest of nailfold capillaroscopy results in patients with Raynaud s phenomenon or undifferentiated connective tissue disease and their correlation with diagnostic and therapeutical evolution. METHODS: Selection of capillaroscopic and laboratory results of patients with the diagnosis of Raynaud s phenomenon (without defined connective tissue disease) or undifferentiated connective tissue disease. Evaluation of the present diagnosis and treatment comparing with the ones existed at the time of capillaroscopy performance. RESULTS: 80 patients were enrolled with an age of 51.4+/-14.3 years (mean+/-SD) 78 females (97.5%) with Raynaud s phenomenon and undifferentiated connective tissue disease 27 patients (33.8%); Raynaud s Phenomenon 46 patients (57.5%); undifferentiated connective tissue disease 7 patients (8.7%). The capillaroscopic results were normal 30 patients (37.5%); minor changes tortuosity enlargement 16 patients (20.0%) major changes 34 patients (42.5%) hemorrhages 25 patients (31.3%) megacapillaries 26 patients (32.5%) avascular areas 3 patients (3.8%). The introduction of new treatments after the capillaroscopy occurred in 32 patients (40.0%) and a new diagnosis was done in 39 patients (48.8%). Major changes in capillaroscopy correlated with the change of diagnosis and the introduction of a new treatment (p<0.0001). CONCLUSION: Nailfold capillaroscopy performed in patients with isolated Raynaud s phenomenon or undifferentiated connective tissue disease has a role in the prognostic evaluation related to the possibility of an evolution of the diagnosis or to the need of the introduction of new treatments. FAU - Cortes, Sara AU - Cortes S AD - Instituto Portugues de Reumatologia, Lisboa Portugal. FAU - Clemente-Coelho, Paulo AU - Clemente-Coelho P LA - por PT - English Abstract PT - Journal Article TT - A Capilaroscopia do Leito Ungueal na Avaliação Prognóstica do Fenómeno de Raynaud e da Doença Indiferenciada do Tecido Conjuntivo. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Connective Tissue Diseases/*pathology MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*pathology MH - Raynaud Disease/*pathology EDAT- 2008/07/08 09:00 MHDA- 2008/10/18 09:00 CRDT- 2008/07/08 09:00 PHST- 2008/07/08 09:00 [pubmed] PHST- 2008/10/18 09:00 [medline] PHST- 2008/07/08 09:00 [entrez] PST - ppublish SO - Acta Reumatol Port. 2008 Apr-Jun;33(2):203-9. PMID- 24839783 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20190918 IS - 0370-8179 (Print) IS - 0370-8179 (Linking) VI - 142 IP - 3-4 DP - 2014 Mar-Apr TI - Dystrophic calcifications and Raynaud's phenomenon in an eight-year old girl. PG - 239-42 AB - INTRODUCTION: Dystrophic calcifications are the most common subtype of skin calcinosis. Tumorous soft tissue calcium deposits usually contain hydroxyapatite and amorphous calcium phosphate. Differential diagnosis of skin calcinosis encompasses Thibierge-Weissenbach syndrome, systemic sclerosis, scleroderma, CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), dermatomyositis, systemic lupus erythematosus, ad myositis ossificans progressiva. CASE OUTLINE: We present the case of an eight-year old girl with tumorous soft tissue calcium deposits and Raynaud's phenomenon. At the age of 3.5 years, our patient was admitted to Pediatric Surgery Clinic because of bilateral acrocyanosis localized at the fingertips area of hands, with the signs of vascular trauma. Therapy with vasodilators and hyperbaric oxygen treatment were completed. This therapy resulted in improvement. At the age of eight, the patient was admitted again due to intermittent, painful cramps localized in both hands. Punctiform deposits were present at the tips of fingers and toes, which looked like calcifications and were spontaneously eliminated, with the remnants of crater-shaped defects. A hard tumorous deformity localized in soft tissue was present in the extensor area of the right elbow. Laboratory indicators of inflammation were within the reference values, and antinuclear antibodies were positive. A nodus localized at the right elbow was extirpated. Pathohistological findings: connective and fat tissue with large deposits of calcium. CONCLUSION: Further follow-up of our patient is necessary due to possible development of complete picture of CREST syndrome or systemic sclerosis. FAU - Grebeldinger, Slobodan P AU - Grebeldinger SP FAU - Tomić, Jelena M AU - Tomić JM FAU - Vijatov-Djurić, Gordana V AU - Vijatov-Djurić GV FAU - Radojcić, Branka S AU - Radojcić BS FAU - Vucković, Nada M AU - Vucković NM FAU - Culafić, Jelena N AU - Culafić JN LA - eng PT - Case Reports PT - Journal Article PL - Serbia TA - Srp Arh Celok Lek JT - Srpski arhiv za celokupno lekarstvo JID - 0027440 SB - IM MH - CREST Syndrome/diagnosis MH - Calcinosis/*complications/diagnosis MH - Child MH - Connective Tissue Diseases/*complications/diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/complications/diagnosis MH - Raynaud Disease/*complications/diagnosis MH - Scleroderma, Systemic/complications/diagnosis EDAT- 2014/05/21 06:00 MHDA- 2015/10/27 06:00 CRDT- 2014/05/21 06:00 PHST- 2014/05/21 06:00 [entrez] PHST- 2014/05/21 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 10.2298/sarh1404239g [doi] PST - ppublish SO - Srp Arh Celok Lek. 2014 Mar-Apr;142(3-4):239-42. doi: 10.2298/sarh1404239g. PMID- 19755613 OWN - NLM STAT- MEDLINE DCOM- 20100211 LR - 20220410 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 36 IP - 10 DP - 2009 Oct TI - Randomized placebo-controlled crossover trial of tadalafil in Raynaud's phenomenon secondary to systemic sclerosis. PG - 2264-8 LID - 10.3899/jrheum.090270 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) is an important clinical feature of systemic sclerosis (SSc) for which consistently effective therapies are lacking. The study was designed to assess the safety, tolerability, and efficacy of tadalafil, a selective, long acting type V cyclic GMP phosphodiesterase (PDE-5) inhibitor, in this clinical syndrome. METHODS: We performed a prospective, randomized, double-blind, placebo-controlled, crossover study comparing oral tadalafil at a fixed dose of 20 mg daily for a period of 4 weeks versus placebo in women with RP secondary to SSc. RESULTS: Thirty-nine subjects completed the study and were evaluable. There were no statistically significant differences in Raynaud Condition Score (RCS), frequency of RP episodes, or duration of RP episodes between treatment groups. Placebo response was a confounding factor. Tadalafil was well tolerated. CONCLUSION: Tadalafil appears to be safe and well tolerated but lacks efficacy in comparison to placebo as a treatment for RP secondary to SSc. FAU - Schiopu, Elena AU - Schiopu E AD - University of Michigan Scleroderma Program, 24 Frank Lloyd Wright Drive, P. O. Box 481, Ann Arbor, MI 48106-5753, USA. FAU - Hsu, Vivien M AU - Hsu VM FAU - Impens, Ann J AU - Impens AJ FAU - Rothman, Jennifer A AU - Rothman JA FAU - McCloskey, Deborah A AU - McCloskey DA FAU - Wilson, Julianne E AU - Wilson JE FAU - Phillips, Kristine AU - Phillips K FAU - Seibold, James R AU - Seibold JR LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20090915 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Carbolines) RN - 0 (Phosphodiesterase Inhibitors) RN - 742SXX0ICT (Tadalafil) SB - IM MH - Adult MH - Carbolines/adverse effects/*therapeutic use MH - Cross-Over Studies MH - Dose-Response Relationship, Drug MH - Double-Blind Method MH - Female MH - Humans MH - Middle Aged MH - Phosphodiesterase Inhibitors/adverse effects/*therapeutic use MH - Pilot Projects MH - Prospective Studies MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Systemic/*complications MH - Tadalafil MH - Treatment Outcome EDAT- 2009/09/17 06:00 MHDA- 2010/02/12 06:00 CRDT- 2009/09/17 06:00 PHST- 2009/09/17 06:00 [entrez] PHST- 2009/09/17 06:00 [pubmed] PHST- 2010/02/12 06:00 [medline] AID - jrheum.090270 [pii] AID - 10.3899/jrheum.090270 [doi] PST - ppublish SO - J Rheumatol. 2009 Oct;36(10):2264-8. doi: 10.3899/jrheum.090270. Epub 2009 Sep 15. PMID- 22293245 OWN - NLM STAT- MEDLINE DCOM- 20121010 LR - 20190513 IS - 1348-9585 (Electronic) IS - 1341-9145 (Linking) VI - 54 IP - 2 DP - 2012 TI - A longitudinal study on Raynaud's phenomenon in workers using an impact wrench. PG - 96-102 AB - OBJECTIVES: The purpose of this study was to clarify the occurrence of Raynaud's phenomenon among workers using an impact wrench for a long time. METHODS: The subjects were 704 workers regularly using an impact wrench and taking special medical examinations for vibration syndrome from 1981 to 2008. Raynaud's phenomenon was observed in 39 subjects during the observation period. RESULTS: The mean operating years at the occurrence of Raynaud's phenomenon was 25.5 ± 8.3 (standard deviation) yr. The mean total operating time (geometric average) at the occurrence of Raynaud's phenomenon was 11,689 h. By the person-year method, the incidence rate of Raynaud's phenomenon was 6.27 persons per 1,000 person-years. The estimated risk of developing Raynaud's phenomenon did not increase until 12 years after starting to operate an impact wrench but increased exponentially after that. The vibration level of an impact wrench (from 4.9 m/s(2) to 22.6 m/s(2)) exceeded the occupational exposure limit value (4.9 m/s(2)). Various countermeasures, such as introducing automatically apparatus and keeping the working environment warm to protect from cold exposure, were taken at the factory. CONCLUSIONS: These findings showed that the rate of occurrence of Raynaud's phenomenon was not high, although the vibration level of the impact wrench was high. This may result from various countermeasures to prevent the occurrence of Raynaud's phenomenon. A long period of exposure to vibration had the potential to lead to the occurrence of Raynaud's phenomenon even under various countermeasures. FAU - Aiba, Yoko AU - Aiba Y AD - Occupational Health Research and Development Center, Japan Industrial Safety and Health Association, Tokyo, Japan. y-aiba@jisha.or.jp FAU - Yamamoto, Kenya AU - Yamamoto K FAU - Ohshiba, Satoshi AU - Ohshiba S FAU - Ikeda, Kazuhiro AU - Ikeda K FAU - Morioka, Ikuharu AU - Morioka I FAU - Miyashita, Kazuhisa AU - Miyashita K FAU - Shimizu, Hidesuke AU - Shimizu H LA - eng PT - Journal Article DEP - 20120131 PL - Australia TA - J Occup Health JT - Journal of occupational health JID - 9616320 SB - IM MH - Adolescent MH - Adult MH - Female MH - Humans MH - Japan/epidemiology MH - Kaplan-Meier Estimate MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Occupational Diseases/*epidemiology/etiology MH - Occupational Exposure/*adverse effects MH - Occupational Health/*statistics & numerical data MH - Proportional Hazards Models MH - Raynaud Disease/*epidemiology/etiology MH - Risk Factors MH - Syndrome MH - Time Factors MH - Vibration/*adverse effects MH - Workplace/psychology MH - Young Adult EDAT- 2012/02/02 06:00 MHDA- 2012/10/12 06:00 CRDT- 2012/02/02 06:00 PHST- 2012/02/02 06:00 [entrez] PHST- 2012/02/02 06:00 [pubmed] PHST- 2012/10/12 06:00 [medline] AID - JST.JSTAGE/joh/11-0058-OA [pii] AID - 10.1539/joh.11-0058-oa [doi] PST - ppublish SO - J Occup Health. 2012;54(2):96-102. doi: 10.1539/joh.11-0058-oa. Epub 2012 Jan 31. PMID- 37957209 OWN - NLM STAT- MEDLINE DCOM- 20231115 LR - 20231116 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 13 IP - 1 DP - 2023 Nov 13 TI - Dynamic blood flow imaging with (99m)Tc-hydroxymethylene diphosphonate as a therapeutic response marker in patients with Raynaud's phenomenon. PG - 19751 LID - 10.1038/s41598-023-47197-3 [doi] LID - 19751 AB - We evaluated the predictive value of dynamic blood flow scintigraphy with (99m)Tc-HDP (hydroxymethylene diphosphonate) for therapeutic response in patients with Raynaud's phenomenon (RP). Eighty patients who underwent dynamic blood flow scintigraphy using the one-hand chilling method were enrolled. We analyzed the quantitative variables as the ratio of chilled fingers to ambient fingers (CA(finger)), that of the chilled hand to ambient hand (CA(hand)), and that of chilled fingers to ambient palm (FPR) (CA(FPR)) at 15 and 30 s after (99m)Tc-HDP bolus injection. Total cumulative radioactivity counts for 180 s were obtained. We evaluated the clinical utility of these quantitative parameters with other clinical variables, including RP severity, therapeutic compliance, types of RP, and scintigraphic interpretation of findings in patients with RP. Fifty-two patients showed poor therapeutic response. There were significant differences between good- and poor-therapeutic responder groups in RP intensity (p = 0.003), CA(finger15s) (p = 0.008), CA(finger30s) (p = 0.002), CA(finger180s) (p = 0.011), CA(hand15s) (p = 0.008), CA(hand30s) (p = 0.007), CA(hand180s) (p = 0.017), CA(FPR30s) (p = 0.004), and CA(FPR180s) (p = 0.002). After multivariate logistic regression analysis, only CA(finger30s) (p = 0.002) had an independent predictive value of the therapeutic response. (99m)Tc-HDP dynamic blood flow scintigraphy could be helpful in predicting the therapeutic response in patients with RP. CI - © 2023. The Author(s). FAU - Yoo, Jang AU - Yoo J AD - Department of Nuclear Medicine, Veterans Health Service Medical Center, Seoul, South Korea. jang8214.yoo@gmail.com. FAU - Cheon, Miju AU - Cheon M AD - Department of Nuclear Medicine, Veterans Health Service Medical Center, Seoul, South Korea. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231113 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - X89XV46R07 (Technetium Tc 99m Medronate) RN - 15468-10-7 (hydroxymethanediphosphonic acid) RN - 0 (Diphosphonates) SB - IM MH - Humans MH - *Hand MH - Fingers MH - Radionuclide Imaging MH - Technetium Tc 99m Medronate MH - Diphosphonates MH - *Raynaud Disease/diagnostic imaging/drug therapy MH - Chills PMC - PMC10643549 COIS- The authors declare no competing interests. EDAT- 2023/11/14 00:42 MHDA- 2023/11/15 06:42 PMCR- 2023/11/13 CRDT- 2023/11/13 23:23 PHST- 2023/05/15 00:00 [received] PHST- 2023/11/10 00:00 [accepted] PHST- 2023/11/15 06:42 [medline] PHST- 2023/11/14 00:42 [pubmed] PHST- 2023/11/13 23:23 [entrez] PHST- 2023/11/13 00:00 [pmc-release] AID - 10.1038/s41598-023-47197-3 [pii] AID - 47197 [pii] AID - 10.1038/s41598-023-47197-3 [doi] PST - epublish SO - Sci Rep. 2023 Nov 13;13(1):19751. doi: 10.1038/s41598-023-47197-3. PMID- 25394956 OWN - NLM STAT- MEDLINE DCOM- 20160502 LR - 20220410 IS - 1827-1839 (Electronic) IS - 0392-9590 (Linking) VI - 34 IP - 5 DP - 2015 Oct TI - Cold climate could be an etiologic factor involved in Raynaud's phenomenon physiopathology. Epidemiological investigation from 954 consultations in general practic. PG - 467-74 AB - AIM: The physiopathology of Raynaud's phenomenon (RP) is not currently fully resolved. The cold seems to be not only an important factor triggering attacks, but also inducing RP. The aims of this study were to assess the prevalence of RP in Nantes urban district, and study the relationship between RP prevalence and cold climate. METHODS: Patients aged between 10 and 80 years old, consulting in five Nantes General Practices, from June 2011 and March 2012, were included. Patients presenting RP underwent a full clinical examination. Subjects not meeting Allen and Brown criteria benefited from at least a dosage of Anti-Nuclear Antibodies and a naifold Capillaroscopy. Climate data provided by French national weather agency allowed establishing an average of observed temperatures during the past five years and correlating them to the observed prevalence. RESULTS: Of 954 patients included, 78 had a RP, for an overall prevalence estimated at 8.2%. The prevalence among women (8.9%) was slightly higher than men (7.3%). Secondary form represented 5.1% of RP. In the RP group, 13 patients were active smokers, mean BMI was 22.3±3.2 kg/m², and only 4 patients were treated by vasoconstrictor therapy. According to French national weather agency, between 2007 and 2011, mean temperature of January in Nantes area was 5.8 °C. CONCLUSION: We confirmed that the lower winter temperatures a region experiences, the higher the prevalence of RP, thus raising the question of the physiopathological role of the cold in the induction or in the revelation of RP. FAU - Plissonneau Duquene, P AU - Plissonneau Duquene P AD - Department of Internal and Vascular Medicine, Hôtel Dieu, Nantes, France - p.plissonneau@gmail.com. FAU - Pistorius, M A AU - Pistorius MA FAU - Pottier, P AU - Pottier P FAU - Aymard, B AU - Aymard B FAU - Planchon, B AU - Planchon B LA - eng PT - Journal Article PT - Multicenter Study DEP - 20141114 PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Cold Climate/*adverse effects MH - Female MH - Fingers/*blood supply MH - France/epidemiology MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*diagnosis/*epidemiology MH - Young Adult EDAT- 2014/11/15 06:00 MHDA- 2016/05/03 06:00 CRDT- 2014/11/15 06:00 PHST- 2014/11/15 06:00 [entrez] PHST- 2014/11/15 06:00 [pubmed] PHST- 2016/05/03 06:00 [medline] AID - R34Y9999N00A140052 [pii] PST - ppublish SO - Int Angiol. 2015 Oct;34(5):467-74. Epub 2014 Nov 14. PMID- 24077899 OWN - NLM STAT- MEDLINE DCOM- 20140929 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 33 IP - 1 DP - 2014 Jan TI - Noninvasive evaluation of cardiac autonomic modulation in children with primary Raynaud’s phenomenon: a controlled study. PG - 71-5 AB - This study aimed to objectively evaluate autonomic nervous function in children with primary Raynaud’s phenomenon (PRP). Thirty-two children with PRP and 30 healthy subjects were included in the study. We analyzed heart rate variability (HRV) in the time domain by the following six standard time-domain measures: standard deviation of all normal R-R intervals during 24 h (SDNN), standard deviation of all normal R-R intervals for all 5-min segments (SDNNi), standard deviation of the average normal R-R intervals for all 5-min segments (SDANN), root mean square of the successive normal R-R interval difference, percentage of successive normal R-R intervals longer than 50 ms, and triangular index (integral of the density distribution of NN intervals divided by the maximum of the density distribution). The mean heart rate throughout 24 h was significantly higher in the PRP group than in the control group (p = 0.001). Although heart rate during the activity period was not significantly different from that during the night period, it was higher in the PRP group than in the control group (p = 0.002). In children with PRP, HRV analysis showed significantly lower values of SDNN (p = 0.01), SDNNi (p = 0.005), SDANN (p = 0.02), and HRV triangular index (p = 0.02) compared with the control group. HRV analysis for sympathovagal balance demonstrated a preponderance for the sympathetic component in patients with PRP. We conclude that all time-domain parameters evaluated in HRV analysis are significantly lower in children with PRP than in healthy subjects. FAU - Oflaz, Mehmet Burhan AU - Oflaz MB FAU - Ece, İbrahim AU - Ece İ FAU - Kibar, Ayşe Esin AU - Kibar AE FAU - Ballı, Şevket AU - Ballı Ş FAU - Alaygut, Demet AU - Alaygut D FAU - Guven, Ahmet Sami AU - Guven AS FAU - Bolat, Fatih AU - Bolat F FAU - Duksal, Fatma AU - Duksal F FAU - Cevit, Ömer AU - Cevit Ö LA - eng PT - Journal Article PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Autonomic Nervous System/*physiopathology MH - Case-Control Studies MH - Child MH - Echocardiography MH - Electrocardiography MH - Female MH - Heart Rate MH - Humans MH - Male MH - Monitoring, Physiologic MH - Raynaud Disease/diagnosis/*physiopathology MH - Sympathetic Nervous System/physiopathology EDAT- 2013/10/01 06:00 MHDA- 2014/09/30 06:00 CRDT- 2013/10/01 06:00 PHST- 2013/03/02 00:00 [received] PHST- 2013/09/12 00:00 [accepted] PHST- 2013/07/03 00:00 [revised] PHST- 2013/10/01 06:00 [entrez] PHST- 2013/10/01 06:00 [pubmed] PHST- 2014/09/30 06:00 [medline] AID - 10.1007/s10067-013-2393-1 [doi] PST - ppublish SO - Clin Rheumatol. 2014 Jan;33(1):71-5. doi: 10.1007/s10067-013-2393-1. PMID- 24583433 OWN - NLM STAT- MEDLINE DCOM- 20150512 LR - 20221207 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 53 IP - 5 DP - 2014 TI - Gemcitabine-induced hemolytic uremic syndrome mimicking scleroderma renal crisis presenting with Raynaud's phenomenon, positive antinuclear antibodies and hypertensive emergency. PG - 445-8 AB - A 58-year-old woman who received gemcitabine for advanced gallbladder cancer developed an impaired renal function, thrombocytopenia, Raynaud's phenomenon, digital ischemic changes, a high antinuclear antibody titer and hypertensive emergency that mimicked a scleroderma renal crisis. A kidney biopsy specimen demonstrated onion-skin lesions in the arterioles and small arteries along with ischemic changes in the glomeruli, compatible with a diagnosis of hypertensive emergency (malignant hypertension). The intravenous administration of a calcium channel blocker, the oral administration of an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker and the transfusion of fresh frozen plasma were effective for treating the thrombocytopenia and progressive kidney dysfunction. Gemcitabine induces hemolytic uremic syndrome with accelerated hypertension and Raynaud's phenomenon, mimicking scleroderma renal crisis. FAU - Yamada, Yuichiro AU - Yamada Y AD - Division of Nephrology and Rheumatology, Department of Internal Medicine, Aichi Medical University School of Medicine, Japan. FAU - Suzuki, Keisuke AU - Suzuki K FAU - Nobata, Hironobu AU - Nobata H FAU - Kawai, Hirohisa AU - Kawai H FAU - Wakamatsu, Ryo AU - Wakamatsu R FAU - Miura, Naoto AU - Miura N FAU - Banno, Shogo AU - Banno S FAU - Imai, Hirokazu AU - Imai H LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0W860991D6 (Deoxycytidine) RN - EC 1.17.4.- (Ribonucleotide Reductases) RN - 0 (Gemcitabine) SB - IM MH - Antibodies, Antinuclear/blood MH - Blood Pressure/physiology MH - Deoxycytidine/adverse effects/*analogs & derivatives/therapeutic use MH - Diagnosis, Differential MH - Emergencies MH - Female MH - Gallbladder Neoplasms/drug therapy MH - Hemolytic-Uremic Syndrome/blood/*chemically induced/diagnosis MH - Humans MH - Hypertension, Malignant/diagnosis/*etiology/physiopathology MH - Immunosuppressive Agents/adverse effects/therapeutic use MH - Kidney Glomerulus/pathology MH - Middle Aged MH - Raynaud Disease/blood/*diagnosis MH - Ribonucleotide Reductases/antagonists & inhibitors MH - Scleroderma, Systemic/*diagnosis MH - Gemcitabine EDAT- 2014/03/04 06:00 MHDA- 2015/05/13 06:00 CRDT- 2014/03/04 06:00 PHST- 2014/03/04 06:00 [entrez] PHST- 2014/03/04 06:00 [pubmed] PHST- 2015/05/13 06:00 [medline] AID - DN/JST.JSTAGE/internalmedicine/53.1160 [pii] AID - 10.2169/internalmedicine.53.1160 [doi] PST - ppublish SO - Intern Med. 2014;53(5):445-8. doi: 10.2169/internalmedicine.53.1160. PMID- 33848376 OWN - NLM STAT- MEDLINE DCOM- 20210803 LR - 20210803 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 48 IP - 8 DP - 2021 Aug TI - Serum levels of tissue factor pathway inhibitor: Potential association with Raynaud's phenomenon and telangiectasia in patients with systemic sclerosis. PG - 1253-1256 LID - 10.1111/1346-8138.15893 [doi] AB - Vasculopathy is a critical step of systemic sclerosis (SSc) development, bridging between autoimmune inflammation and tissue fibrosis. Impaired coagulation system is a part of SSc vasculopathy, but the role of tissue factor pathway inhibitor (TFPI), a critical regulator of the extrinsic coagulation pathway, remained unknown. Therefore, we evaluated the clinical correlation of serum TFPI levels in SSc patients. Serum TFPI levels were comparable between SSc and control participants, but SSc patients with Raynaud's phenomenon and telangiectasia had significantly lower serum TFPI levels than those without. Importantly, there was a significant positive correlation between serum TFPI levels and protein S activity. These results support the critical role of impaired coagulation system in SSc. CI - © 2021 Japanese Dermatological Association. FAU - Norimatsu, Yuta AU - Norimatsu Y AUID- ORCID: 0000-0002-7649-0806 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miyagawa, Takuya AU - Miyagawa T AUID- ORCID: 0000-0002-4471-1635 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Fukui, Yuki AU - Fukui Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Omatsu, Jun AU - Omatsu J AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Toyama, Satoshi AU - Toyama S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Awaji, Kentaro AU - Awaji K AUID- ORCID: 0000-0003-3864-5587 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Ikawa, Tetsuya AU - Ikawa T AUID- ORCID: 0000-0002-2753-9001 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Watanabe, Yusuke AU - Watanabe Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yoshizaki, Ayumi AU - Yoshizaki A AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Sato, Shinichi AU - Sato S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Asano, Yoshihide AU - Asano Y AUID- ORCID: 0000-0001-5560-9778 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. LA - eng GR - The Ministry of Health, Labor, and Welfare of Japan/ PT - Journal Article DEP - 20210413 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Lipoproteins) RN - 0 (lipoprotein-associated coagulation inhibitor) SB - IM MH - Humans MH - Lipoproteins MH - *Raynaud Disease/etiology MH - *Scleroderma, Systemic/complications/diagnosis MH - *Telangiectasis OTO - NOTNLM OT - Raynaud’s phenomenon OT - coagulation OT - systemic sclerosis OT - telangiectasia OT - tissue factor pathway inhibitor EDAT- 2021/04/14 06:00 MHDA- 2021/08/04 06:00 CRDT- 2021/04/13 17:17 PHST- 2021/03/07 00:00 [revised] PHST- 2020/11/10 00:00 [received] PHST- 2021/03/23 00:00 [accepted] PHST- 2021/04/14 06:00 [pubmed] PHST- 2021/08/04 06:00 [medline] PHST- 2021/04/13 17:17 [entrez] AID - 10.1111/1346-8138.15893 [doi] PST - ppublish SO - J Dermatol. 2021 Aug;48(8):1253-1256. doi: 10.1111/1346-8138.15893. Epub 2021 Apr 13. PMID- 40812099 OWN - NLM STAT- MEDLINE DCOM- 20250918 LR - 20250918 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 74 DP - 2025 Oct TI - Raynaud's phenomenon is associated with an increased risk of cardiovascular disease and venous thromboembolism. PG - 152799 LID - S0049-0172(25)00170-2 [pii] LID - 10.1016/j.semarthrit.2025.152799 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) is common affecting 3-5 % of the population; however, there are little data concerning vascular outcomes. We aimed to estimate risk of cardiovascular disease (CVD) and venous thromboembolism (VTE) in individuals with RP without underlying relevant systemic autoimmune rheumatic diseases (SARDs). METHODS: A cohort study using data from North American electronic healthcare organization records. RP was defined using ≥2 ICD codes (I73.0), excluding those with SARDs. Comparators had ≥2 irritable bowel syndrome (IBS) ICD codes (K58); selected as a condition with similar demographics but not typically associated with adverse CVD outcomes. Cohorts were stratified by age (<45 and ≥45 years). Co-primary outcomes were 1) major adverse cardiovascular events (MACE), and 2) VTE. Secondary outcomes included: myocardial infarction, stroke, any CVD. Risk of each outcome was compared using 1:1 propensity score matched Cox proportional hazard models. RESULTS: Among 30,088 matched pairs aged <45, hazard ratios (HR [95 % CI]) of MACE (HR 1.23 [1.07, 1.42]) and VTE (HR 1.32 [1.20, 1.46]) were higher in RP (excluding SARD) than IBS. Similarly, in 60,145 matched pairs ages ≥45, MACE (HR 1.17 [1.13, 1.22]) and VTE (HR 1.20 [1.14, 1.26]) were higher in RP. Risk of secondary outcomes was higher in RP, although estimates for DVT in both analyses and myocardial infarction in patients under 45 lacked precision. CONCLUSIONS: RP was associated with an increased risk of CVD and VTE, independent of age and traditional cardiovascular risk factors. Future research is warranted to confirm, explore pathobiological mechanisms, and develop therapeutic strategies. CI - Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biological Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK; Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK; NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. Electronic address: Michael.hughes-6@manchester.ac.uk. FAU - Ruaro, Barbara AU - Ruaro B AD - Pulmonology Unit, University of Trieste, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, Trieste, Italy. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - UTHealth Houston, Department of Medicine, Division of Rheumatology, Houston, TX, USA. FAU - Alam, Uazman AU - Alam U AD - Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK; Department of Medicine, University Hospital Aintree, Liverpool University NHS Foundation Trust, Liverpool, UK. FAU - Jude, Edward AU - Jude E AD - Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton under Lyne, UK; University of Manchester, Manchester, UK; Manchester Metropolitan University, Manchester, UK. FAU - Zhao, Sizheng Steven AU - Zhao SS AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biological Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. LA - eng PT - Journal Article DEP - 20250805 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Raynaud Disease/complications/epidemiology MH - *Venous Thromboembolism/epidemiology/etiology MH - Female MH - Male MH - Middle Aged MH - *Cardiovascular Diseases/epidemiology/etiology MH - Adult MH - Risk Factors MH - Aged MH - Cohort Studies OTO - NOTNLM OT - Cardiovascular disease OT - DVT: Myocardial infraction OT - Raynaud’s phenomenon OT - Stroke OT - Venous thromboembolism COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: MH reports Research Funding and Speaker Fees from Janssen, and Conference Support from UCB, and Consultancy Fees from Boehringer Ingelheim and Novartis, outside of the submitted work. UA has no direct COI. However, UA declares that he has received honoraria from Viatris, Grünenthal, Eli Lilly, Procter & Gamble and for educational meetings and has received investigator-led funding form Proctor & Gamble. UA has received sponsorship to educational meetings from Daiichi Sankyo and Sanofi. None of the other authors report any relevant disclosures to the manuscript. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/08/14 22:50 MHDA- 2025/09/19 00:36 CRDT- 2025/08/14 18:10 PHST- 2025/04/30 00:00 [received] PHST- 2025/07/11 00:00 [revised] PHST- 2025/07/18 00:00 [accepted] PHST- 2025/09/19 00:36 [medline] PHST- 2025/08/14 22:50 [pubmed] PHST- 2025/08/14 18:10 [entrez] AID - S0049-0172(25)00170-2 [pii] AID - 10.1016/j.semarthrit.2025.152799 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2025 Oct;74:152799. doi: 10.1016/j.semarthrit.2025.152799. Epub 2025 Aug 5. PMID- 41724373 OWN - NLM STAT- In-Process DCOM- 20260315 LR - 20260608 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 165 DP - 2026 May TI - Carbon dioxide vs. warm-water hand baths in systemic sclerosis with secondary Raynaud's syndrome - A capillaroscopy centered randomized controlled trial. PG - 104922 LID - S0026-2862(26)00022-1 [pii] LID - 10.1016/j.mvr.2026.104922 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) affects nearly all patients with systemic sclerosis (SSc) and causes substantial morbidity and functional impairment. We evaluated the immediate, segment-specific microvascular effects of a single carbon dioxide (CO₂) versus warm water hand baths in SSc with secondary RP, including healthy controls as physiological reference. METHODS: In this single-centre, randomized controlled trial, 24 SSc patients were allocated (1:1) to a 15-min CO₂ bath (2 g/L, 35 °C) or warm water bath (40-42 °C). Twelve healthy controls received a CO(2) bath. Nailfold capillaroscopy assessed vas afferens (VA), apex, and vas efferens (VE) diameters at baseline, immediately, and 10 min post-intervention. Primary outcome was immediate diameter change; secondary outcomes were persistence and between-cohort differences. RESULTS: After CO₂ baths, mean VA diameter increased by 3.31 μm (95% CI [2.23,4.38]; p < 0.001) and VE by 3.83 μm (95% CI [2.28,5.39]; p < 0.001), both exceeding changes after warm water in SSc patients (p < 0.001; p = 0.005). CO(2) induced dilation in healthy controls, but VA increase was smaller than after CO₂ in SSc (p = 0.010). At 10 min, VA remained higher in the CO₂ vs. water group (p = 0.006). No adverse events occurred. CONCLUSIONS: A single CO₂ hand bath induced greater and partly sustained nailfold capillary dilation of the arterial limb and apex than warm water in SSc-associated RP. The lack of response to warm water suggests impaired heat-induced vasodilation in SSc, whereas CO₂ may engage alternative vasodilatory mechanisms. These findings support CO₂ hand baths as a safe, low-cost, non-pharmacological adjunct. Larger trials with clinical endpoints are warranted. CI - Copyright © 2026 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Schulz, Nils AU - Schulz N AD - Justus Liebig University Giessen, Campus Kerckhoff, Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Bad Nauheim, Germany. Electronic address: n.schulz@kerckhoff-klinik.de. FAU - Hermann, Walter AU - Hermann W AD - Justus Liebig University Giessen, Campus Kerckhoff, Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Bad Nauheim, Germany. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Justus Liebig University Giessen, Campus Kerckhoff, Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Bad Nauheim, Germany. FAU - Lange, Uwe AU - Lange U AD - Justus Liebig University Giessen, Campus Kerckhoff, Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Bad Nauheim, Germany. FAU - Klemm, Philipp AU - Klemm P AD - Justus Liebig University Giessen, Campus Kerckhoff, Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Bad Nauheim, Germany. LA - eng PT - Journal Article DEP - 20260220 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 142M471B3J (Carbon Dioxide) RN - 059QF0KO0R (Water) SB - IM MH - Humans MH - *Raynaud Disease/therapy/physiopathology/etiology/diagnosis MH - *Scleroderma, Systemic/complications/therapy/physiopathology/diagnosis MH - Female MH - *Carbon Dioxide/administration & dosage MH - *Microscopic Angioscopy MH - Male MH - Middle Aged MH - *Hand/blood supply MH - Adult MH - Treatment Outcome MH - Time Factors MH - *Microcirculation MH - *Nails/blood supply MH - *Water MH - Aged MH - *Baths MH - *Capillaries/physiopathology MH - Vasodilation MH - *Hot Temperature MH - Predictive Value of Tests OTO - NOTNLM OT - Carbon dioxide OT - Nailfold capillaroscopy OT - Systemic sclerosis OT - Vasodilatation COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/02/23 01:34 MHDA- 2026/03/16 00:30 CRDT- 2026/02/22 19:29 PHST- 2026/01/07 00:00 [received] PHST- 2026/02/11 00:00 [revised] PHST- 2026/02/16 00:00 [accepted] PHST- 2026/03/16 00:30 [medline] PHST- 2026/02/23 01:34 [pubmed] PHST- 2026/02/22 19:29 [entrez] AID - S0026-2862(26)00022-1 [pii] AID - 10.1016/j.mvr.2026.104922 [doi] PST - ppublish SO - Microvasc Res. 2026 May;165:104922. doi: 10.1016/j.mvr.2026.104922. Epub 2026 Feb 20. PMID- 34346597 OWN - NLM STAT- MEDLINE DCOM- 20210823 LR - 20210823 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 165 DP - 2021 Jun 21 TI - [Sympathicotomy in patients with drug resistant Raynaud's phenomenon]. LID - D5630 [pii] AB - Raynaud's phenomenon (RP) is a disorder of the microvasculature which causes poor blood flow to the digits. This disorder is common in young females and may be associated with several underlying connective tissue diseases including systemic sclerosis. Although RP may have a tremendous impact on quality of life, treatment options are limited. Conventional medical treatment mainly consists of vasodilatory drugs, which are not effective in all patients and may induce undesired side effects. The current clinical lesson describes three patients with severe RP who all underwent a novel, minimally invasive, single-port thoracoscopic sympathicotomy (SPTS). Although this procedure seems promising in patients with treatment-resistant RP, as shown with patients A and B, future research has yet to show what the long-term effects are. FAU - van Roon, Anniek M AU - van Roon AM AD - Universitair Medisch Centrum Groningen, afd. Reumatologie en Klinische Immunologie, Groningen. AD - Contact: Anniek M. van Roon (a.m.van.roon01@umcg.nl). FAU - Eman Abdulle, Amaal AU - Eman Abdulle A AD - Universitair Medisch Centrum Groningen, afd. Interne Geneeskunde, Groningen. FAU - Zhang, Dan AU - Zhang D AD - Medisch Centrum Leeuwarden, afd. Reumatologie, Leeuwarden. FAU - Stel, Alja J AU - Stel AJ AD - Universitair Medisch Centrum Groningen, afd. Reumatologie en Klinische Immunologie, Groningen. FAU - Kuijpers, Michiel AU - Kuijpers M AD - Universitair Medisch Centrum Groningen, afd. CardiothoracaleChirurgie, Groningen. FAU - Mulder, D J AU - Mulder DJ AD - Universitair Medisch Centrum Groningen, afd. Reumatologie en Klinische Immunologie, Groningen. LA - dut PT - Journal Article TT - Sympathicotomie bij medicatieresistent fenomeen van Raynaud. DEP - 20210621 PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Pharmaceutical Preparations) SB - IM MH - Female MH - Humans MH - *Pharmaceutical Preparations MH - Quality of Life MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic EDAT- 2021/08/05 06:00 MHDA- 2021/08/24 06:00 CRDT- 2021/08/04 09:02 PHST- 2021/08/04 09:02 [entrez] PHST- 2021/08/05 06:00 [pubmed] PHST- 2021/08/24 06:00 [medline] AID - D5630 [pii] PST - epublish SO - Ned Tijdschr Geneeskd. 2021 Jun 21;165:D5630. PMID- 41537619 OWN - NLM STAT- In-Process DCOM- 20260115 LR - 20260604 IS - 0042-8787 (Print) IS - 0042-8787 (Linking) VI - 102 IP - 6 DP - 2025 TI - [Historical development and modern significance of capillaroscopy in the diagnosis and monitoring of microcirculatory disorders]. PG - 40-44 LID - 10.17116/kurort202510206140 [doi] AB - Capillaroscopy is a non-invasive method for visualizing the microcirculatory system using modern digital equipment. This review presents the historical development of the method-from the first microscopic observations of nailfold capillaries in the 17(th) century to the creation of high-precision videocapillaroscopes. Special attention is paid to the clinical application of the method in rheumatology for diagnosing systemic sclerosis and the differential diagnosis of Raynaud's phenomenon. The prospects of using capillaroscopy in neurological practice for assessing microcirculatory disorders in migraine and neuropathies are discussed. The importance of standardizing the method according to EULAR recommendations and the integration of artificial intelligence technologies for automating the analysis of capillaroscopic images is emphasized. Capillaroscopy demonstrates potential as an effective diagnostic tool in various fields of medicine. FAU - Titov, G B AU - Titov GB AUID- ORCID: 0009-0006-5603-4930 AD - Sechenov First Moscow State Medical University, Moscow, Russia. LA - rus PT - English Abstract PT - Journal Article TT - Istoricheskoe razvitie i sovremennoe znachenie kapillyaroskopii v diagnostike i monitoringe mikrotsirkulyatornykh narushenii. PL - Russia (Federation) TA - Vopr Kurortol Fizioter Lech Fiz Kult JT - Voprosy kurortologii, fizioterapii, i lechebnoi fizicheskoi kultury JID - 2984868R SB - IM MH - Humans MH - *Microscopic Angioscopy/methods/history/instrumentation MH - *Microcirculation/physiology MH - History, 17th Century MH - *Raynaud Disease/diagnosis/physiopathology/history/diagnostic imaging MH - *Scleroderma, Systemic/diagnosis/physiopathology/history/diagnostic imaging MH - History, 20th Century MH - History, 18th Century MH - History, 19th Century MH - *Migraine Disorders/physiopathology/diagnosis/diagnostic imaging/history MH - Diagnosis, Differential OTO - NOTNLM OT - capillaroscopy OT - diagnostics OT - microcirculation OT - videocapillaroscopy EDAT- 2026/01/15 16:13 MHDA- 2026/01/15 16:14 CRDT- 2026/01/15 09:21 PHST- 2026/01/15 16:14 [medline] PHST- 2026/01/15 16:13 [pubmed] PHST- 2026/01/15 09:21 [entrez] AID - 10.17116/kurort202510206140 [doi] PST - ppublish SO - Vopr Kurortol Fizioter Lech Fiz Kult. 2025;102(6):40-44. doi: 10.17116/kurort202510206140. PMID- 41417215 OWN - NLM STAT- MEDLINE DCOM- 20260417 LR - 20260417 IS - 1432-1912 (Electronic) IS - 0028-1298 (Linking) VI - 399 IP - 6 DP - 2026 Mar TI - Calcitonin gene-related peptide antagonists in Raynaud's phenomenon: a disproportionality study based on real data and drug-gene network analysis. PG - 8049-8059 LID - 10.1007/s00210-025-04877-3 [doi] AB - This study aims to investigate the potential risk and possible mechanisms of Raynaud's phenomenon (RP) associated with the use of calcitonin gene-related peptide (CGRP) antagonists through a comprehensive analysis of the FDA Adverse Event Reporting System (FAERS) database combined with drug-gene network analysis methods. In this disproportionality study, we evaluated the adverse event (AE) signal of RP associated with CGRP antagonists from the FAERS covering the quarter 2 of 2018 to quarter 1 of 2025, using four methods. The gene targets of CGRP antagonists and RP targets were predicted using multiple databases and protein-protein interactions (PPI) using the STRING database. Subsequently, Kyoto Encyclopedia of genes and genomes (KEGG) enrichment analysis was performed using R software to identify the potential mechanisms of CGRP antagonists related to RP. This study retrieved a total of 149 AE reports related to CGRP antagonists and RP reports from the FAERS database, involving 7 CGRP antagonists. Erenumab has the highest number of reports. The data mining results indicate that fremanezumab resulted in the strongest AE signals of the four methods. Protein-protein interaction (PPI) network analysis revealed key nodes of non-peptide small molecule CGRP antagonists in RP, such as the key nodes of rimegepant in RP is AKT1, EGFR, ERBB2, and others. The KEGG pathway enrichment analysis showed that the most possible mechanisms of rimegepant, atogepant, and ubrogepant induce RP is the PI3K signaling pathway. Using a novel approach, we systematically integrated the FAERS database with drug-gene network analysis. Our results not only suggest a potential risk of RP associated with CGRP antagonists but also reveal that the PI3K/AKT pathway is the underlying mechanism for non-peptide small molecules. We recommend that patients receiving these antagonists, particularly those with underlying vascular dysfunction, undergo regular monitoring for RP. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Zhu, Haibin AU - Zhu H AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. FAU - Ma, Minghua AU - Ma M AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. FAU - Tian, Weiwei AU - Tian W AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. FAU - Wu, Tingting AU - Wu T AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. FAU - Wang, Yan AU - Wang Y AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. FAU - Huo, Yan AU - Huo Y AUID- ORCID: 0009-0006-0710-3231 AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. huoyan@tongji.edu.cn. FAU - Liao, Xiaolan AU - Liao X AUID- ORCID: 0000-0001-9110-3934 AD - Department of Pharmacy, Yangpu Hospital, School of Medicine, Tongji University, 200090, Shanghai, China. 15259676260@126.com. LA - eng PT - Journal Article DEP - 20251219 PL - Germany TA - Naunyn Schmiedebergs Arch Pharmacol JT - Naunyn-Schmiedeberg's archives of pharmacology JID - 0326264 RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) RN - I5I8VB78VT (erenumab) RN - 0 (Antibodies, Monoclonal, Humanized) SB - IM MH - Humans MH - *Gene Regulatory Networks/drug effects MH - *Calcitonin Gene-Related Peptide Receptor Antagonists/adverse effects MH - *Raynaud Disease/chemically induced/genetics/drug therapy MH - *Calcitonin Gene-Related Peptide/antagonists & inhibitors MH - Adverse Drug Reaction Reporting Systems/statistics & numerical data MH - Protein Interaction Maps MH - Databases, Factual MH - Antibodies, Monoclonal, Humanized/adverse effects OTO - NOTNLM OT - Calcitonin gene-related peptide antagonists OT - Drug-gene network analysis OT - FAERS OT - Migraine OT - Raynaud’s phenomenon COIS- Declarations. Ethics approval: This study did not require institutional ethics committee approval because the analysis was carried out on an anonymized and publicly accessible pharmacovigilance database. Consent to participate: Not applicable. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2025/12/19 15:03 MHDA- 2026/04/19 17:41 CRDT- 2025/12/19 11:26 PHST- 2025/09/11 00:00 [received] PHST- 2025/11/29 00:00 [accepted] PHST- 2026/04/19 17:41 [medline] PHST- 2025/12/19 15:03 [pubmed] PHST- 2025/12/19 11:26 [entrez] AID - 10.1007/s00210-025-04877-3 [pii] AID - 10.1007/s00210-025-04877-3 [doi] PST - ppublish SO - Naunyn Schmiedebergs Arch Pharmacol. 2026 Mar;399(6):8049-8059. doi: 10.1007/s00210-025-04877-3. Epub 2025 Dec 19. PMID- 20863907 OWN - NLM STAT- MEDLINE DCOM- 20110801 LR - 20110302 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 10 IP - 5 DP - 2011 Mar TI - Two faces of the same coin: Raynaud phenomenon and digital ulcers in systemic sclerosis. PG - 241-3 LID - 10.1016/j.autrev.2010.09.008 [doi] AB - Systemic sclerosis (SSc) is characterized by wide-spread fibrosis, activation of immune system with production of autoantibodies and extensive vascular damage. Raynaud's phenomenon (RP) and digital ulcers (DU) represent two faces of the same coin in SSc vasculopathy. RP, the earliest manifestation of the vascular involvement, is due to an excessive vasospasm of digital arteries, precapillary arterioles and cutaneous arteriovenous shunts, usually in response to cold exposure or other stimuli. DU are a severe complication of microvessel involvement and also of the persistent vasospasm of RP. Thus, the management of RP and DU requires a multimodal approach using a combination of pharmacological, non-pharmacological, and surgical treatments. Currently, the treatment of these complications represents a great challenge for all physicians. CI - Copyright © 2010 Elsevier B.V. All rights reserved. FAU - Galluccio, Felice AU - Galluccio F AD - Department of Biomedicine–Division of Rheumatology AOUC, Denothe centre, University of Florence, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M LA - eng PT - Journal Article PT - Review DEP - 20100921 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/biosynthesis MH - Cold Temperature MH - Combined Modality Therapy MH - Endothelium, Vascular/physiopathology MH - Fibrosis/complications/*physiopathology/therapy MH - Humans MH - Immune System Phenomena MH - Raynaud Disease/complications/*physiopathology/therapy MH - Scleroderma, Systemic/complications/immunology/*physiopathology/therapy MH - Skin/blood supply MH - Skin Ulcer/complications/*physiopathology/therapy MH - Spasm/physiopathology MH - Ulnar Artery/physiopathology MH - Vasoconstriction EDAT- 2010/09/25 06:00 MHDA- 2011/08/02 06:00 CRDT- 2010/09/25 06:00 PHST- 2010/09/25 06:00 [entrez] PHST- 2010/09/25 06:00 [pubmed] PHST- 2011/08/02 06:00 [medline] AID - S1568-9972(10)00204-1 [pii] AID - 10.1016/j.autrev.2010.09.008 [doi] PST - ppublish SO - Autoimmun Rev. 2011 Mar;10(5):241-3. doi: 10.1016/j.autrev.2010.09.008. Epub 2010 Sep 21. PMID- 38109321 OWN - NLM STAT- MEDLINE DCOM- 20231220 LR - 20250513 IS - 2242-3982 (Electronic) IS - 1239-9736 (Print) IS - 1239-9736 (Linking) VI - 83 IP - 1 DP - 2024 Dec TI - Raynaud's phenomenon in the feet of Arctic open-pit miners. PG - 2295576 LID - 10.1080/22423982.2023.2295576 [doi] LID - 2295576 AB - The literature on Raynaud's phenomenon (RP) in the feet is scarce, especially in the occupational setting. The primary aim of our study was to investigate the occurrence of RP in the feet of miners. As part of the MineHealth project, written surveys and clinical examinations were completed by 260 Arctic open-pit miners working in northern Sweden and Norway (participation rate 53.6%). Data on RP were collected using standardised colour charts and questionnaire items. Clinical examination included assessing the perception of vibration and pain in both feet. There were eight women and three men who reported RP in the feet. Four also had RP in their hands but none acknowledged any first-degree relatives with the condition. Nine reported exposure to foot-transmitted vibration and one to hand-arm vibration. Seven showed signs of neurosensory injury in the feet. To conclude, the occurrence of RP in the feet of miners was 4.4%. Most cases with RP in the feet did not report the condition in the hands and were exposed to vibration transmitted directly to the feet. There were no reports of a hereditary component. Most cases with RP in the feet also had clinical findings suggestive of peripheral neuropathy in the feet. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Pettersson, Hans AU - Pettersson H AUID- ORCID: 0000-0001-7077-2389 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Vihlborg, Per AU - Vihlborg P AUID- ORCID: 0000-0002-4256-1880 AD - Department of Geriatrics, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. FAU - Höper, Anje Christina AU - Höper AC AUID- ORCID: 0000-0002-8962-5853 AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Tromsø, Norway. AD - Department of Community Medicine, UiT The Arctic University of Norway, Tromsø, Norway. FAU - Aminoff, Anna AU - Aminoff A AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Tromsø, Norway. AD - Department of Community Medicine, UiT The Arctic University of Norway, Tromsø, Norway. FAU - Wahlström, Jens AU - Wahlström J AUID- ORCID: 0000-0002-2359-509X AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AUID- ORCID: 0000-0003-2789-6321 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231218 PL - United States TA - Int J Circumpolar Health JT - International journal of circumpolar health JID - 9713056 SB - IM MH - Male MH - Humans MH - Female MH - *Occupational Diseases/epidemiology MH - *Raynaud Disease/epidemiology MH - Hand MH - Vibration/adverse effects MH - Pain PMC - PMC10732197 OTO - NOTNLM OT - Cold climate OT - Norway OT - Raynaud Disease OT - Sweden OT - foot-transmitted vibration OT - mining OT - peripheral nervous system diseases OT - vibration OT - white toes OT - whole-body vibration COIS- No potential conflict of interest was reported by the author(s). EDAT- 2023/12/18 18:41 MHDA- 2023/12/20 06:42 PMCR- 2023/12/18 CRDT- 2023/12/18 13:34 PHST- 2023/12/20 06:42 [medline] PHST- 2023/12/18 18:41 [pubmed] PHST- 2023/12/18 13:34 [entrez] PHST- 2023/12/18 00:00 [pmc-release] AID - 2295576 [pii] AID - 10.1080/22423982.2023.2295576 [doi] PST - ppublish SO - Int J Circumpolar Health. 2024 Dec;83(1):2295576. doi: 10.1080/22423982.2023.2295576. Epub 2023 Dec 18. PMID- 32040755 OWN - NLM STAT- MEDLINE DCOM- 20201208 LR - 20201214 IS - 1435-1250 (Electronic) IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 79 IP - 10 DP - 2020 Dec TI - [Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany]. PG - 1057-1066 LID - 10.1007/s00393-019-00743-9 [doi] AB - BACKGROUND: Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers. METHODS: In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives. RESULTS: Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower. CONCLUSION: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations. FAU - Juche, A AU - Juche A AD - Klinik für Rheumatologie, Immanuel Krankenhaus Berlin-Buch, Berlin, Deutschland. FAU - Siegert, E AU - Siegert E AD - Klinik für Rheumatologie u. klinischer Immunologie, Charité Berlin, Berlin, Deutschland. FAU - Mueller-Ladner, U AU - Mueller-Ladner U AD - Rheumatologie und klinische Immunologie, Kerckhoff-Klinik, Bad Nauheim, Deutschland. FAU - Riemekasten, G AU - Riemekasten G AD - Klinik für Rheumatologie und Immunologie, Universitätsklinikum Schleswig-Holstein, Lübeck, Deutschland. FAU - Günther, C AU - Günther C AD - Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Dresden, Deutschland. FAU - Kötter, I AU - Kötter I AD - Klinik für Rheumatologie, klinische Immunologie u. Nephrologie, Asklepios Kliniken Hamburg, Hamburg, Deutschland. FAU - Henes, J AU - Henes J AD - Zentrum für interdisziplinäre Rheumatologie, Immunologie und Autoimmunerkrankungen INDIRA und Medizinische Klinik II, Universitätsklinik Tübingen, Tübingen, Deutschland. FAU - Blank, N AU - Blank N AD - Medizinische Klinik f. Hämatologie, Onkologie u. Rheumatologie, Universitätsklinikum Heidelberg, Heidelberg, Deutschland. FAU - Voll, R E AU - Voll RE AD - Klinik für Rheumatologie u. Klinische Immunologie, Medizinische Fakultät, Universität Freiburg, Freiburg, Deutschland. FAU - Ehrchen, J AU - Ehrchen J AD - Klinik für Hautkrankheiten, allg. Dermatologie u. Venerologie, Universitätsklinikum Münster, Münster, Deutschland. FAU - Schmalzing, M AU - Schmalzing M AD - Rheumatologie/Klinische Immunologie, Medizinische Klinik II, Universitätsklinikum Würzburg, Würzburg, Deutschland. FAU - Susok, L AU - Susok L AD - Klinik für Dermatologie, Allergologie u. Venerologie der Ruhr-Universität Bochum, Krankenhaus St. Josef-Hospital Bochum, Bochum, Deutschland. FAU - Schmeiser, T AU - Schmeiser T AD - Klinik für Rheumatologie, Immunologie u. Osteologie, St. Josef Wuppertal, Wuppertal, Deutschland. FAU - Sunderkoetter, C AU - Sunderkoetter C AD - Universitätsklinik u. Poliklinik für Dermatologie u. Venerologie, Universitätsklinikum Halle (Saale), Halle (Saale), Deutschland. FAU - Distler, J AU - Distler J AD - Medizinische Klinik für Rheumatologie u. Immunologie, Universitätsklinikum Erlangen, Erlangen, Deutschland. FAU - Worm, M AU - Worm M AD - Klinik für Dermatologie, Venerologie u. Allergologie, Charité Berlin, Berlin, Deutschland. FAU - Kreuter, A AU - Kreuter A AD - Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik Oberhausen, Universität Witten/Herdecke, Oberhausen, Deutschland. FAU - Horváth, O N AU - Horváth ON AD - Klinik für Dermatologie u. Allergologie, Ludwig-Maximilians Universität München, München, Deutschland. FAU - Schön, M P AU - Schön MP AD - Klinik für Dermatologie, Venerologie u. Allergologie, Universitätsmedizin Göttingen, Göttingen, Deutschland. AD - Niedersächsisches Institut für Berufsdermatologie, Universitätsmedizin Göttingen, Göttingen, Deutschland. FAU - Korsten, P AU - Korsten P AD - Klinik für Nephrologie u. Rheumatologie, Universitätsmedizin Göttingen, Göttingen, Deutschland. FAU - Zeidler, G AU - Zeidler G AD - Klinik für internistische Rheumatologie, Orthopädie u. Rheumachirurgie, Johanniter-Krankenhaus im Fläming, Treuenbrietzen, Deutschland. FAU - Pfeiffer, C AU - Pfeiffer C AD - Klinik für Dermatologie u. Allergologie, Universitätsklinikum Ulm, Ulm, Deutschland. FAU - Krieg, T AU - Krieg T AD - Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Köln, Deutschland. FAU - Hunzelmann, N AU - Hunzelmann N AD - Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Köln, Deutschland. FAU - Moinzadeh, P AU - Moinzadeh P AD - Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Köln, Deutschland. pia.moinzadeh@uk-koeln.de. LA - ger PT - Journal Article TT - Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - *Epoprostenol/analogs & derivatives/therapeutic use MH - Fingers/blood supply MH - Germany MH - Humans MH - Inpatients MH - Quality of Life MH - *Raynaud Disease/diagnosis/drug therapy/epidemiology MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy MH - Skin/blood supply PMC - PMC7708340 OTO - NOTNLM OT - Digital ulceration OT - Iloprost OT - Raynaud’s phenomenon OT - Scleroderma OT - Systemic sclerosis EDAT- 2020/02/11 06:00 MHDA- 2020/12/15 06:00 PMCR- 2020/02/10 CRDT- 2020/02/11 06:00 PHST- 2020/02/11 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2020/02/11 06:00 [entrez] PHST- 2020/02/10 00:00 [pmc-release] AID - 10.1007/s00393-019-00743-9 [pii] AID - 743 [pii] AID - 10.1007/s00393-019-00743-9 [doi] PST - ppublish SO - Z Rheumatol. 2020 Dec;79(10):1057-1066. doi: 10.1007/s00393-019-00743-9. PMID- 36082752 OWN - NLM STAT- MEDLINE DCOM- 20221006 LR - 20221114 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 34 IP - 6 DP - 2022 Nov 1 TI - Insights into molecular and clinical characteristics of very early systemic sclerosis. PG - 351-356 LID - 10.1097/BOR.0000000000000903 [doi] AB - PURPOSE OF REVIEW: The early heterogenous presentation of systemic sclerosis (SSc), in particular without skin involvement, has been a confounding factor delaying early diagnosis. In fact, early signs of SSc as Raynaud's phenomenon and puffy fingers, are also typical of other connective tissue diseases (CTDs) such as mixed CTD and undifferentiated CTD. In the last decade, a significant effort has been dedicated in defining molecular characteristics that could be used as early SSc biomarkers. In this narrative review, we address the present situation where several clinical scenarios are in search of a correct positioning into the prescleroderma (pre-SSc) phase as well as in the very early phase of SSc. RECENT FINDINGS: Literature data showed that a part of patients classified as sine scleroderma SSc (ssSSc), mixed CTD and undifferentiated CTD may already belong to the very early phase of SSc, thus having a different pattern of progression to SSc. Recently, the very early diagnosis of systemic sclerosis (VEDOSS) criteria has been validated. SUMMARY: while the area of pre-SSc still remains fuzzy, the VEDOSS study has shown that a 'window of opportunity' does exist also for SSc. In the very next future, this may allow to start the treatment to prevent the disease progression to a more advanced fibrotic stage. CI - Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, University of Florence. AD - Division of Rheumatology and Scleroderma Unit, Department of Geriatric Medicine, AOUC, Florence, Italy. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Chapel Allerton Hospital, Leeds, UK. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence. AD - Division of Rheumatology and Scleroderma Unit, Department of Geriatric Medicine, AOUC, Florence, Italy. AD - Division of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy. LA - eng PT - Journal Article PT - Review DEP - 20220830 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Biomarkers) SB - IM MH - Biomarkers MH - Early Diagnosis MH - Fingers MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Systemic/diagnosis EDAT- 2022/09/10 06:00 MHDA- 2022/10/07 06:00 CRDT- 2022/09/09 05:52 PHST- 2022/09/10 06:00 [pubmed] PHST- 2022/10/07 06:00 [medline] PHST- 2022/09/09 05:52 [entrez] AID - 00002281-202211000-00010 [pii] AID - 10.1097/BOR.0000000000000903 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2022 Nov 1;34(6):351-356. doi: 10.1097/BOR.0000000000000903. Epub 2022 Aug 30. PMID- 34800695 OWN - NLM STAT- MEDLINE DCOM- 20220512 LR - 20220611 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 89 IP - 3 DP - 2022 May TI - Serotonin and systemic sclerosis. An emerging player in pathogenesis. PG - 105309 LID - S1297-319X(21)00182-2 [pii] LID - 10.1016/j.jbspin.2021.105309 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a complex, autoimmune disease characterized by multiple organ fibrosis and vasculopathy. Experimental and clinical evidence indicates that serotonin is crucially involved in the fibrotic process and mediates vascular manifestations such as Raynaud's phenomenon (RP) or pulmonary arterial hypertension (PAH), all key features of SSc. In this review, we summarize the current knowledge on the potential contribution of serotonin in SSc pathogenesis and provide a rationale for further investigation of this molecule as a therapeutic target. METHODS: Medline and Cochrane databases were searched from inception to April 2021 using the search terms (systemic sclerosis OR scleroderma OR Raynaud OR Pulmonary arterial hypertension) AND serotonin. RESULTS: Serotonin, a key molecule in an array of central and peripheral functions, has a multifaceted role in regulating fibrosis and vasculopathy. Experimental data suggest that serotonin drives fibrosis in the skin and visceral organs, promotes platelet aggregation, induces vasoconstriction and increases pulmonary vascular resistance. Earlier human trials regarding drugs that inhibit serotonin signaling produced mixed results. However, recent advances in the understanding of the underlying molecular mechanisms could help identify novel therapeutics targeting the serotonin pathway and inform future clinical trials. CONCLUSIONS: Serotonin may be a mediator in both fibrosis and vasculopathy. Further exploration of the potential role of serotonin in SSc is justified. CI - Copyright © 2021 Société française de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. FAU - Sagonas, Ioannis AU - Sagonas I AD - General Hospital of Laconia, Sparta, Greece. FAU - Daoussis, Dimitrios AU - Daoussis D AD - Department of Internal Medicine, Division of Rheumatology, Patras University Hospital, 26504 Rion, Patras, Greece. Electronic address: jimdaoussis@hotmail.com. LA - eng PT - Journal Article PT - Review DEP - 20211117 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 333DO1RDJY (Serotonin) SB - IM MH - Fibrosis MH - Humans MH - *Pulmonary Arterial Hypertension MH - *Raynaud Disease/etiology MH - *Scleroderma, Systemic/drug therapy MH - Serotonin/therapeutic use OTO - NOTNLM OT - Fibrosis OT - Pulmonary arterial hypertension OT - Raynaud's OT - Scleroderma OT - Serotonin OT - Systemic sclerosis OT - Vasculopathy EDAT- 2021/11/21 06:00 MHDA- 2022/05/14 06:00 CRDT- 2021/11/20 20:14 PHST- 2021/09/16 00:00 [received] PHST- 2021/11/03 00:00 [revised] PHST- 2021/11/09 00:00 [accepted] PHST- 2021/11/21 06:00 [pubmed] PHST- 2022/05/14 06:00 [medline] PHST- 2021/11/20 20:14 [entrez] AID - S1297-319X(21)00182-2 [pii] AID - 10.1016/j.jbspin.2021.105309 [doi] PST - ppublish SO - Joint Bone Spine. 2022 May;89(3):105309. doi: 10.1016/j.jbspin.2021.105309. Epub 2021 Nov 17. PMID- 24753696 OWN - NLM STAT- MEDLINE DCOM- 20150127 LR - 20220410 IS - 1598-6357 (Electronic) IS - 1011-8934 (Print) IS - 1011-8934 (Linking) VI - 29 IP - 4 DP - 2014 Apr TI - Digital thermography of the fingers and toes in Raynaud's phenomenon. PG - 502-6 LID - 10.3346/jkms.2014.29.4.502 [doi] AB - The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2℃) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11℃) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended. FAU - Lim, Mie Jin AU - Lim MJ AUID- ORCID: 0000-0002-7405-8139 AD - Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, Incheon, Korea. FAU - Kwon, Seong Ryul AU - Kwon SR AUID- ORCID: 0000-0003-1262-2790 AD - Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, Incheon, Korea. FAU - Jung, Kyong-Hee AU - Jung KH AUID- ORCID: 0000-0002-5757-5775 AD - Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, Incheon, Korea. FAU - Joo, Kowoon AU - Joo K AUID- ORCID: 0000-0002-3517-5033 AD - Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, Incheon, Korea. FAU - Park, Shin-Goo AU - Park SG AUID- ORCID: 0000-0002-1544-4486 AD - Department of Occupational and Environmental Medicine, Inha University Hospital, Incheon, Korea. FAU - Park, Won AU - Park W AUID- ORCID: 0000-0002-0004-8034 AD - Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, Incheon, Korea. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140401 PL - Korea (South) TA - J Korean Med Sci JT - Journal of Korean medical science JID - 8703518 SB - IM MH - Adult MH - Diagnosis, Differential MH - Female MH - Fingers/*physiology MH - Humans MH - Male MH - Middle Aged MH - ROC Curve MH - Raynaud Disease/*diagnosis MH - Sensitivity and Specificity MH - Skin Temperature MH - *Thermography MH - Toes/*physiology PMC - PMC3991792 OTO - NOTNLM OT - Digital Thermography OT - Raynaud Disease OT - Temperature OT - Thermography COIS- Authors declare no conflicts of interest. EDAT- 2014/04/23 06:00 MHDA- 2015/01/28 06:00 PMCR- 2014/04/01 CRDT- 2014/04/23 06:00 PHST- 2013/12/05 00:00 [received] PHST- 2014/02/14 00:00 [accepted] PHST- 2014/04/23 06:00 [entrez] PHST- 2014/04/23 06:00 [pubmed] PHST- 2015/01/28 06:00 [medline] PHST- 2014/04/01 00:00 [pmc-release] AID - 10.3346/jkms.2014.29.4.502 [doi] PST - ppublish SO - J Korean Med Sci. 2014 Apr;29(4):502-6. doi: 10.3346/jkms.2014.29.4.502. Epub 2014 Apr 1. PMID- 17098681 OWN - NLM STAT- MEDLINE DCOM- 20061213 LR - 20250529 IS - 1607-551X (Print) IS - 2410-8650 (Electronic) IS - 1607-551X (Linking) VI - 22 IP - 10 DP - 2006 Oct TI - Peripheral sympathectomy for Raynaud's phenomenon: a salvage procedure. PG - 491-9 AB - We retrospectively reviewed the effectiveness of peripheral sympathectomy for severe Raynaud's phenomenon. In this study, a total of 14 digits from six patients with chronic digital ischemic change were included. All patients had pain, ulcer, or gangrenous change in the affected digits and were unresponsive to pharmacologic or other nonsurgical therapies. In all cases, angiography showed multifocal arterial lesions, so microvascular reconstruction was unfeasible. Peripheral sympathectomy was performed as a salvage procedure to prevent digit amputation. The results were analyzed according to reduction of pain, healing of ulcers, and prevention of amputation. In 12 of the 14 digits, the ulcers healed and amputation was avoided. In the other two digits, the ulcers improved and progressive gangrene was limited. As a salvage procedure for Raynaud's phenomenon recalcitrant to conservative treatment, peripheral sympathectomy improves perfusion to ischemic digits and enables amputation to be avoided. FAU - Wang, Wen-Her AU - Wang WH AD - Division of Plastic and Reconstructive Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan. FAU - Lai, Chung-Sheng AU - Lai CS FAU - Chang, Kao-Ping AU - Chang KP FAU - Lee, Su-Shin AU - Lee SS FAU - Yang, Chih-Chiang AU - Yang CC FAU - Lin, Sin-Daw AU - Lin SD FAU - Liu, Chia-Ming AU - Liu CM LA - eng PT - Case Reports PT - Journal Article PL - China (Republic : 1949- ) TA - Kaohsiung J Med Sci JT - The Kaohsiung journal of medical sciences JID - 100960562 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*surgery MH - Retrospective Studies MH - Salvage Therapy MH - Sympathectomy/*methods PMC - PMC11917728 EDAT- 2006/11/14 09:00 MHDA- 2006/12/14 09:00 PMCR- 2009/07/13 CRDT- 2006/11/14 09:00 PHST- 2006/11/14 09:00 [pubmed] PHST- 2006/12/14 09:00 [medline] PHST- 2006/11/14 09:00 [entrez] PHST- 2009/07/13 00:00 [pmc-release] AID - S1607-551X(09)70343-2 [pii] AID - KJM2491 [pii] AID - 10.1016/S1607-551X(09)70343-2 [doi] PST - ppublish SO - Kaohsiung J Med Sci. 2006 Oct;22(10):491-9. doi: 10.1016/S1607-551X(09)70343-2. PMID- 22618491 OWN - NLM STAT- MEDLINE DCOM- 20131030 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 4 DP - 2013 Apr TI - Is there a difference in systemic lupus erythematosus with and without Raynaud's phenomenon? PG - 859-65 LID - 10.1007/s00296-012-2449-6 [doi] AB - The aim of this study was to assess the association between Raynaud's phenomenon (RP) and specific capillaroscopic findings in patients with SLE and particular clinical manifestations of the disease. A total of 79 patients with SLE were included in the study: 44 of them (43 women) with RP and 35 (32 women) age-, sex-, and disease-duration-matched patients with SLE without RP. Demographic variables, clinical manifestations, laboratory and nailfold capillaroscopy findings were compared between the two groups. Central nervous systemic involvements (P = 0.0038) and peripheral neuropathy (P = 0.0336) were significantly more common in SLE patients with RP, while secondary Sjögren's syndrome (P = 0.0363) was more common in SLE patients without RP. RP occurred in 52 % of patients before SLE onset while 48 % of patients developed RP after they had been diagnosed with SLE. Arthritis/arthralgia (P = 0.0073) was significantly more common in patients who had been diagnosed with RP before the onset of SLE, while mucosal ulcers were more common in patients who contracted RP after the onset of SLE (P = 0.0258). Enlarged capillaries (P = 0.0482), presence of avascular areas (P = 0.0476), capillary hemorrhages (P = 0.0482), and granular blood flow (P = 0.0482) were more common in patients with SLE who also suffered from RP, than in patients with SLE without RP. The frequency of normal (63.6 vs. 82.9 %, P = 0.100) and nonspecific (25 vs. 17.1 %, P = 0.5696) capillaroscopy findings were similar in either groups. Scleroderma-like pattern of capillaroscopy finding was only found in patients with RP [(11.4 %), P = 0.0482]. RP in our patients with SLE was associated with specific clinical manifestations, indicating that prognostic relevance of RP in SLE should be evaluated. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AD - Institute of Rheumatology, Belgrade, Serbia. dolijan@eunet.rs FAU - Damjanov, Nemanja S AU - Damjanov NS FAU - Vujasinovic Stupar, Nada Z AU - Vujasinovic Stupar NZ FAU - Marcetic, Danijel R AU - Marcetic DR FAU - Sefik-Bukilica, Mirjana N AU - Sefik-Bukilica MN FAU - Petrovic, Radmila R AU - Petrovic RR LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120522 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Arthralgia/*complications/physiopathology MH - Arthritis/*complications/physiopathology MH - Capillaries/physiopathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/physiopathology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/blood supply MH - Prognosis MH - Raynaud Disease/*complications/physiopathology EDAT- 2012/05/24 06:00 MHDA- 2013/10/31 06:00 CRDT- 2012/05/24 06:00 PHST- 2011/12/29 00:00 [received] PHST- 2012/05/06 00:00 [accepted] PHST- 2012/05/24 06:00 [entrez] PHST- 2012/05/24 06:00 [pubmed] PHST- 2013/10/31 06:00 [medline] AID - 10.1007/s00296-012-2449-6 [doi] PST - ppublish SO - Rheumatol Int. 2013 Apr;33(4):859-65. doi: 10.1007/s00296-012-2449-6. Epub 2012 May 22. PMID- 32320708 OWN - NLM STAT- MEDLINE DCOM- 20200923 LR - 20240329 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 130 DP - 2020 Jul TI - State-of-the-art technologies provide new insights linking skin and blood vessel abnormalities in SSc-related disorders. PG - 104006 LID - S0026-2862(20)30066-2 [pii] LID - 10.1016/j.mvr.2020.104006 [doi] LID - 104006 AB - OBJECTIVE: A key unanswered question in systemic sclerosis (SSc) is how microvascular abnormality and fibrosis inter-relate. Our aim was to use state-of-the-art non-invasive imaging methods to gain new insights into pathophysiology, comparing patients with different subtypes of SSc, including early dcSSc, not only to healthy controls but also to patients with causes of Raynaud's phenomenon not progressing to fibrosis. METHODS: Laser Doppler imaging, nailfold capillaroscopy, spectroscopy, and ultrasound measured (respectively) perfusion, microvascular structure, oxygenation/oxidative stress, and skin thickening in the hands of 265 subjects: 31 patients with primary Raynaud's phenomenon (PRP), 35 with undifferentiated connective tissue disease (UCTD), 93 with limited cutaneous SSc (lcSSc), 46 with diffuse cutaneous SSc (dcSSc, including 27 'early') and 60 healthy controls. RESULTS: Mean perfusion was reduced in SSc groups compared to controls (lcSSc 172 perfusion units [standard deviation 157], late-dcSSc 90 [145], early-dcSSc 68 [137] vs. controls 211 [146]; p = 0.0002) as was finger-oxygenation (lcSSc 12.1 [13.6] arbitrary units [AU], late-dcSSc 12.2 [8.4], early-dcSSc 11.1 [11.3] vs controls 14.9 [10.5]; p = 0.0049). Oxidative stress was increased at the hand-dorsum in SSc groups (p = 0.0007). Perfusion positively correlated with oxygenation (r = 0.23, p < 0.001), and capillary density negatively with skin thickness (r = -0.26, p < 0.001). CONCLUSION: Our findings lend support to the hypothesis that in SSc, particularly early dcSSc, (but not in PRP or UCTD), reduced perfusion (together with structural microvascular abnormality) associates with reduced oxygenation, with oxidative stress and with skin thickening/fibrosis, most likely driving a vicious cycle which ultimately results in irreversible tissue injury. Findings in skin may mirror alterations in internal organs. CI - Copyright © 2020. Published by Elsevier Inc. FAU - Dinsdale, Graham AU - Dinsdale G AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Wilkinson, Sarah AU - Wilkinson S AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Wilkinson, Jack AU - Wilkinson J AD - Centre for Biostatistics, Manchester Academic Health Science Centre, Faculty of Biology, Medicine and Health The University of Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Moore, Tonia L AU - Moore TL AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Manning, Joanne B AU - Manning JB AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Berks, Michael AU - Berks M AD - Centre for Imaging Sciences, Division of Informatics, Imaging and Data Sciences, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Marjanovic, Elizabeth AU - Marjanovic E AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Dickinson, Mark AU - Dickinson M AD - Photon Science Institute, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; The Dept of Physics and Astronomy, Unversity of Manchester, United Kingdom of Great Britain and Northern Ireland. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. Electronic address: andrea.murray@manchester.ac.uk. FAU - Murray, Andrea K AU - Murray AK AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland. LA - eng GR - DH_/Department of Health/United Kingdom GR - 19465/ARC_/Arthritis Research UK/United Kingdom PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200419 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - S88TT14065 (Oxygen) SB - IM MH - Adult MH - Blood Flow Velocity MH - Case-Control Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Laser-Doppler Flowmetry MH - Male MH - Microcirculation MH - *Microscopic Angioscopy MH - Microvessels/*diagnostic imaging/physiopathology MH - Middle Aged MH - Oxidative Stress MH - Oxygen/blood MH - Predictive Value of Tests MH - Raynaud Disease/blood/*diagnostic imaging/pathology/physiopathology MH - Regional Blood Flow MH - Scleroderma, Diffuse/blood/*diagnostic imaging/pathology/physiopathology MH - Scleroderma, Limited/blood/*diagnostic imaging/pathology/physiopathology MH - Skin/*blood supply/metabolism/pathology MH - Spectrum Analysis MH - *Ultrasonography PMC - PMC7522709 OTO - NOTNLM OT - Hypoxia OT - Imaging OT - Laser Doppler OT - Nailfold capillaroscopy OT - Oxidative stress OT - Raynaud's phenomenon OT - Spectroscopy OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare no conflicts of interests. EDAT- 2020/04/23 06:00 MHDA- 2020/09/24 06:00 PMCR- 2020/07/01 CRDT- 2020/04/23 06:00 PHST- 2020/01/17 00:00 [received] PHST- 2020/04/07 00:00 [revised] PHST- 2020/04/10 00:00 [accepted] PHST- 2020/04/23 06:00 [pubmed] PHST- 2020/09/24 06:00 [medline] PHST- 2020/04/23 06:00 [entrez] PHST- 2020/07/01 00:00 [pmc-release] AID - S0026-2862(20)30066-2 [pii] AID - 104006 [pii] AID - 10.1016/j.mvr.2020.104006 [doi] PST - ppublish SO - Microvasc Res. 2020 Jul;130:104006. doi: 10.1016/j.mvr.2020.104006. Epub 2020 Apr 19. PMID- 20430487 OWN - NLM STAT- MEDLINE DCOM- 20101102 LR - 20161018 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 31 IP - 7 DP - 2010 Jul TI - [RACAND syndrome associated with primary biliary cirrhosis]. PG - e11-3 LID - 10.1016/j.revmed.2009.04.012 [doi] AB - The acronym RACAND means the association of Raynaud's phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynaud's phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells. FAU - Abouzahir, A AU - Abouzahir A AD - Service de médecine interne B, hôpital militaire d'instruction Med-V 10000, Rabat, Maroc. FAU - Badaoui, M AU - Badaoui M FAU - Amezyane, T AU - Amezyane T FAU - Fatihi, J AU - Fatihi J FAU - Chahdi, H AU - Chahdi H FAU - Albouzidi, A AU - Albouzidi A FAU - Mahassin, F AU - Mahassin F FAU - Ghafir, D AU - Ghafir D FAU - Ohayon, V AU - Ohayon V LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Syndrome RACAND associé à une cirrhose biliaire primitive. DEP - 20100428 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Antibodies, Antinuclear/blood MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/*etiology MH - Middle Aged MH - Necrosis MH - Raynaud Disease/blood/*complications MH - Syndrome MH - Toes/*pathology EDAT- 2010/05/01 06:00 MHDA- 2010/11/03 06:00 CRDT- 2010/05/01 06:00 PHST- 2008/11/20 00:00 [received] PHST- 2009/04/14 00:00 [revised] PHST- 2009/04/28 00:00 [accepted] PHST- 2010/05/01 06:00 [entrez] PHST- 2010/05/01 06:00 [pubmed] PHST- 2010/11/03 06:00 [medline] AID - S0248-8663(10)00088-3 [pii] AID - 10.1016/j.revmed.2009.04.012 [doi] PST - ppublish SO - Rev Med Interne. 2010 Jul;31(7):e11-3. doi: 10.1016/j.revmed.2009.04.012. Epub 2010 Apr 28. PMID- 40817612 OWN - NLM STAT- MEDLINE DCOM- 20251120 LR - 20251123 IS - 1440-0960 (Electronic) IS - 0004-8380 (Print) IS - 0004-8380 (Linking) VI - 66 IP - 7 DP - 2025 Nov TI - Breast and Nipple Dermatoses During Lactation. PG - e386-e407 LID - 10.1111/ajd.14586 [doi] AB - Lactation and breastfeeding can present both psychological and physical challenges for breastfeeding mothers. In addition, many nursing mothers will also suffer from breast and nipple dermatoses during this period, compounding these difficulties. Common causes of breast and nipple dermatitis during lactation include eczema, psoriasis, mastitis, mammary Paget's disease, Raynaud's phenomenon, and herpes virus infection, all of which may arise or be exacerbated during breastfeeding. This article summarises the common causes of breast and nipple dermatitis during lactation, as well as their investigation and management. In addition, we review the safety of common dermatological medications in this population, accounting for the unique consideration of the breastfeeding infant. CI - © 2025 The Author(s). Australasian Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Australasian College of Dermatologists. FAU - Moore, Hamish AU - Moore H AUID- ORCID: 0000-0001-6815-2658 AD - Faculty of Health and Medical Sciences, University of Adelaide, Adelaide, South Australia, Australia. AD - Department of Dermatology, Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - Stevenson, Annabel AU - Stevenson A AD - Faculty of Health and Medical Sciences, University of Adelaide, Adelaide, South Australia, Australia. AD - Department of Dermatology, Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - Department of Dermatology, Queen Elizabeth Hospital, Woodville South, South Australia, Australia. LA - eng PT - Journal Article PT - Review DEP - 20250815 PL - Australia TA - Australas J Dermatol JT - The Australasian journal of dermatology JID - 0135232 SB - IM MH - Humans MH - Female MH - *Nipples MH - *Lactation MH - *Breast Feeding/adverse effects MH - *Breast Diseases/therapy/etiology/diagnosis MH - Mastitis/therapy/diagnosis MH - Eczema/therapy/diagnosis MH - Paget's Disease, Mammary/therapy/diagnosis MH - Raynaud Disease/complications/therapy MH - Psoriasis/therapy/diagnosis PMC - PMC12633704 OTO - NOTNLM OT - breast feeding OT - dermatitis OT - eczema OT - lactation OT - mastitis OT - medication safety OT - psoriasis COIS- The authors declare no conflicts of interest. EDAT- 2025/08/16 06:27 MHDA- 2025/11/20 13:38 PMCR- 2025/11/20 CRDT- 2025/08/16 02:03 PHST- 2025/07/24 00:00 [revised] PHST- 2025/04/13 00:00 [received] PHST- 2025/08/05 00:00 [accepted] PHST- 2025/11/20 13:38 [medline] PHST- 2025/08/16 06:27 [pubmed] PHST- 2025/08/16 02:03 [entrez] PHST- 2025/11/20 00:00 [pmc-release] AID - AJD14586 [pii] AID - 10.1111/ajd.14586 [doi] PST - ppublish SO - Australas J Dermatol. 2025 Nov;66(7):e386-e407. doi: 10.1111/ajd.14586. Epub 2025 Aug 15. PMID- 23270004 OWN - NLM STAT- MEDLINE DCOM- 20130311 LR - 20161125 IS - 1527-7755 (Electronic) IS - 0732-183X (Linking) VI - 31 IP - 4 DP - 2013 Feb 1 TI - Bleomycin-induced Raynaud's phenomenon after single-dose exposure: risk factors and treatment with intravenous iloprost infusion. PG - e51-2 LID - 10.1200/JCO.2012.43.2872 [doi] FAU - McGrath, Sophie E AU - McGrath SE AD - University of Surrey, Guildford, United Kingdom. FAU - Webb, Andrew AU - Webb A FAU - Walker-Bone, Karen AU - Walker-Bone K LA - eng PT - Case Reports PT - Journal Article DEP - 20121226 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - 0 (Vasodilator Agents) RN - 11056-06-7 (Bleomycin) RN - 5V9KLZ54CY (Vinblastine) RN - 7GR28W0FJI (Dacarbazine) RN - 80168379AG (Doxorubicin) RN - JED5K35YGL (Iloprost) RN - ABVD protocol SB - IM MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*therapeutic use MH - Bleomycin/*administration & dosage MH - Dacarbazine/administration & dosage MH - Doxorubicin/administration & dosage MH - Fingers/blood supply MH - Hodgkin Disease/diagnosis/diagnostic imaging/*drug therapy MH - Humans MH - Iloprost/administration & dosage/*therapeutic use MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Multimodal Imaging MH - Necrosis/chemically induced MH - Positron-Emission Tomography MH - Raynaud Disease/*chemically induced/*drug therapy MH - Remission Induction MH - Risk Factors MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage/*therapeutic use MH - Vinblastine/administration & dosage EDAT- 2012/12/28 06:00 MHDA- 2013/03/12 06:00 CRDT- 2012/12/28 06:00 PHST- 2012/12/28 06:00 [entrez] PHST- 2012/12/28 06:00 [pubmed] PHST- 2013/03/12 06:00 [medline] AID - JCO.2012.43.2872 [pii] AID - 10.1200/JCO.2012.43.2872 [doi] PST - ppublish SO - J Clin Oncol. 2013 Feb 1;31(4):e51-2. doi: 10.1200/JCO.2012.43.2872. Epub 2012 Dec 26. PMID- 22835924 OWN - NLM STAT- MEDLINE DCOM- 20130225 LR - 20181201 IS - 1885-1398 (Electronic) IS - 1699-258X (Linking) VI - 8 IP - 5 DP - 2012 Sep-Oct TI - Raynaud, digital ulcers and calcinosis in scleroderma. PG - 270-7 AB - Raynaud, digital ulcers and calcinosis are frequent manifestations of patients with systemic sclerosis. Digital ulcers are seen in more than half of the patients with scleroderma. Hospitalizations, ischemic complications and impairment of hand function are frequently observed in patients with digital ulcers, especially if treatment is delayed. Rapid and intensive treatment escalation in patients with scleroderma and refractory Raynaud's phenomenon is one of the most effective preventive action available in order to avoid the development of digital ulcers and tissue loss. CI - Copyright © 2011 Elsevier España, S.L. All rights reserved. FAU - Nitsche, Alejandro AU - Nitsche A AD - Specialist in rheumatology, Servicio de Reumatología, Hospital Alemán; Consultorio de Raynaud, Esclerodermia e Hipertensión Arterial Pulmonar, Sanatorio San José, Ciudad Autónomade Buenos Aires, Argentina. alejandro.nitsche@gmail.com LA - eng PT - Journal Article PT - Review DEP - 20120725 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 RN - 0 (Endothelin-1) RN - 0 (Platelet Aggregation Inhibitors) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - Q326023R30 (Bosentan) SB - IM MH - Bosentan MH - Calcinosis/drug therapy/*etiology/physiopathology MH - Cell Hypoxia MH - Combined Modality Therapy MH - Endothelin-1/physiology MH - Endothelium, Vascular/pathology/physiopathology MH - Fingers/blood supply/pathology MH - Foot Ulcer/drug therapy/etiology/physiopathology/surgery MH - Humans MH - Ischemia/drug therapy/etiology/physiopathology/prevention & control MH - Multicenter Studies as Topic MH - Platelet Aggregation MH - Platelet Aggregation Inhibitors/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/diagnosis/drug therapy/*etiology/therapy MH - Scleroderma, Systemic/*complications/diagnosis/drug therapy/physiopathology MH - Skin Ulcer/drug therapy/*etiology/physiopathology/surgery MH - Sulfonamides/therapeutic use MH - Thrombophilia/etiology MH - Thrombosis/etiology/prevention & control MH - Toes/blood supply/pathology MH - Vasoconstriction/physiology MH - Vasodilator Agents/therapeutic use EDAT- 2012/07/28 06:00 MHDA- 2013/02/26 06:00 CRDT- 2012/07/28 06:00 PHST- 2011/12/26 00:00 [received] PHST- 2012/02/23 00:00 [revised] PHST- 2012/02/29 00:00 [accepted] PHST- 2012/07/28 06:00 [entrez] PHST- 2012/07/28 06:00 [pubmed] PHST- 2013/02/26 06:00 [medline] AID - S1699-258X(12)00090-3 [pii] AID - 10.1016/j.reuma.2012.02.006 [doi] PST - ppublish SO - Reumatol Clin. 2012 Sep-Oct;8(5):270-7. doi: 10.1016/j.reuma.2012.02.006. Epub 2012 Jul 25. PMID- 36972928 OWN - NLM STAT- MEDLINE DCOM- 20230329 LR - 20240916 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 1 DP - 2023 Mar TI - Associations between nailfold capillary aberrations and autoantibodies in children and adults with Raynaud's phenomenon. LID - 10.1136/rmdopen-2023-003077 [doi] LID - e003077 AB - OBJECTIVE: To characterise associations between individual nailfold capillary aberrations with autoantibodies in a cross-sectional study on children and adults with Raynaud's phenomenon (RP). METHODS: Consecutive children and adults with RP and without previously known connective tissue disease (CTD) systemically underwent nailfold capillaroscopy and laboratory tests for the presence of antinuclear antibodies (ANA). The prevalence of individual nailfold capillary aberrations and ANA was assessed, and the associations between individual nailfold capillary aberrations and ANA were analysed separately in children and adolescents. RESULTS: In total, 113 children (median age 15 years) and 2858 adults (median age 48 years) with RP and without previously known CTD were assessed. At least one nailfold capillary aberration was detected in 72 (64%) of included children and in 2154 (75%) of included adults with RP (children vs adults p<0.05). An ANA titre ≥1:80, ≥1:160 or≥1:320 was observed in 29%, 21% or 16% of included children, and in 37%, 27% or 24% of screened adults, respectively. While the occurrence of individual nailfold capillary aberrations was related to the presence of an ANA titre of ≥1:80 in adults (reduced capillary density, avascular fields, haemorrhages, oedema, ramifications, dilations and giant capillaries: each p<0.001), no comparable association between nailfold capillary aberrations and ANA was observed in children with RP without previously known CTD. CONCLUSION: In contrast to adults, the association between nailfold capillary aberrations and ANA might be less pronounced in children. Further studies are warranted to validate these observations in children with RP. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Mueller, Markus AU - Mueller M AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Austria, Medical University of Vienna, Vienna, Austria. FAU - Gschwandtner, Michael E AU - Gschwandtner ME AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Austria, Medical University of Vienna, Vienna, Austria. FAU - Emminger, Wolfgang AU - Emminger W AD - Department of Pediatrics, University Children's Hospital, Wien, Austria. FAU - Kiener, Hans AU - Kiener H AD - Division of Rheumatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria. FAU - Schnaubelt, Sebastian AU - Schnaubelt S AD - Department of Emergency Medicine, Medical University of Vienna, Vienna, Austria. FAU - Giurgea, Georgiana-Aura AU - Giurgea GA AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Austria, Medical University of Vienna, Vienna, Austria. FAU - Ristl, Robin AU - Ristl R AD - Center of Medical Statistics, Informatics and Intelligent Systems, Medical University of Vienna, Vienna, Austria. FAU - Perkmann, Thomas AU - Perkmann T AUID- ORCID: 0000-0002-7976-0285 AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Koppensteiner, Renate AU - Koppensteiner R AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Austria, Medical University of Vienna, Vienna, Austria. FAU - Schlager, Oliver AU - Schlager O AUID- ORCID: 0000-0001-6934-5355 AD - Division of Angiology, Department of Medicine II, Medical University of Vienna, Austria, Medical University of Vienna, Vienna, Austria oliver.schlager@meduniwien.ac.at. LA - eng PT - Journal Article PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Humans MH - Adult MH - Child MH - Middle Aged MH - Autoantibodies MH - Capillaries MH - Cross-Sectional Studies MH - Nails/blood supply MH - *Raynaud Disease/diagnosis/etiology MH - Antibodies, Antinuclear MH - *Connective Tissue Diseases PMC - PMC10069575 OTO - NOTNLM OT - Autoantibodies OT - Autoimmune Diseases OT - Cardiovascular Diseases COIS- Competing interests: None declared. EDAT- 2023/03/28 06:00 MHDA- 2023/03/29 06:05 PMCR- 2023/03/27 CRDT- 2023/03/27 20:52 PHST- 2023/02/16 00:00 [received] PHST- 2023/03/13 00:00 [accepted] PHST- 2023/03/29 06:05 [medline] PHST- 2023/03/27 20:52 [entrez] PHST- 2023/03/28 06:00 [pubmed] PHST- 2023/03/27 00:00 [pmc-release] AID - rmdopen-2023-003077 [pii] AID - 10.1136/rmdopen-2023-003077 [doi] PST - ppublish SO - RMD Open. 2023 Mar;9(1):e003077. doi: 10.1136/rmdopen-2023-003077. PMID- 28450106 OWN - NLM STAT- MEDLINE DCOM- 20180319 LR - 20220408 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 113 DP - 2017 Sep TI - Detection of early endothelial damage in patients with Raynaud's phenomenon. PG - 22-28 LID - S0026-2862(16)30198-4 [pii] LID - 10.1016/j.mvr.2017.04.004 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) can be the first manifestation of systemic sclerosis (SSc) or other connective tissue diseases (CTDs), often preceding an overt disease by years. It is not known if markers of endothelial damage are detectable in those RP patients who subsequently develop a CTD. METHODS: We studied 82 RP patients at their first evaluation to correlate the levels of endothelial markers with the subsequent development of an overt disease 36months later. We measured plasma levels of tissue-type plasminogen activator (t-PA) and von Willebrand factor (vWF), two markers of endothelial damage, and interleukin-6 (IL-6), a pro-inflammatory cytokine. Thirty sex- and age-matched healthy subjects (HS) served as controls. RESULTS: At baseline, 67 patients showed capillaroscopic normal pattern (CNP) and 15 patients, of which 11 were very early SSc, had capillaroscopic scleroderma pattern (CSP). Plasma levels of t-PA, vWF and IL-6 were higher in patients with CNP (p=0.0001) than in HS and even much higher in patients with CSP (p=0.0001). In patients with CNP and RP of recent onset (<18months), vWF plasma levels were higher when autoantibodies were present (p=0.020). After 36months, among 48 RP patients with CNP who remained in follow-up, 24 were diagnosed as primary and 24 as secondary RP. In secondary RP, basal levels of t-PA, IL-6 and particularly vWF were higher than in primary RP (p=0.005, p=0.004, p=0.0001 respectively) and HS (p=0.0001 for all). CONCLUSIONS: Our findings indicate that markers of endothelial damage are elevated in RP patients who subsequently develop SSc or other CTDs, even in the absence of capillaroscopic abnormalities. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Gualtierotti, Roberta AU - Gualtierotti R AD - Lupus Clinic, Divisione e Cattedra di Reumatologia, Università degli Studi di Milano, Istituto G. Pini, Milan, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, Cattedra di Reumatologia, Istituto G. Pini, Milan, Italy. FAU - Griffini, Samantha AU - Griffini S AD - Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milan, Italy. FAU - Grovetti, Elena AU - Grovetti E AD - Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milan, Italy. FAU - Borghi, Maria Orietta AU - Borghi MO AD - Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, Cattedra di Reumatologia, Istituto G. Pini, Milan, IRCCS Istituto Auxologico Italiano, Milan, Italy. FAU - Bucciarelli, Paolo AU - Bucciarelli P AD - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy. FAU - Meroni, Pier Luigi AU - Meroni PL AD - Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, Cattedra di Reumatologia, Istituto G. Pini, Milan, IRCCS Istituto Auxologico Italiano, Milan, Italy. FAU - Cugno, Massimo AU - Cugno M AD - Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Milan, Italy. Electronic address: massimo.cugno@unimi.it. LA - eng PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20170425 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (IL6 protein, human) RN - 0 (Interleukin-6) RN - 0 (von Willebrand Factor) RN - EC 3.4.21.68 (Tissue Plasminogen Activator) SB - IM MH - Adult MH - Aged MH - Area Under Curve MH - Autoantibodies/blood MH - Biomarkers/blood MH - Case-Control Studies MH - Early Diagnosis MH - Endothelial Cells/*metabolism/pathology MH - Female MH - Humans MH - Interleukin-6/*blood MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - ROC Curve MH - Raynaud Disease/*blood/pathology MH - Time Factors MH - Tissue Plasminogen Activator/*blood MH - Up-Regulation MH - Young Adult MH - von Willebrand Factor/*metabolism OTO - NOTNLM OT - Autoantibodies OT - Capillaroscopy OT - Endothelial damage OT - Interleukin-6 OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Tissue-type plasminogen activator OT - Very early systemic sclerosis OT - von Willebrand factor EDAT- 2017/04/30 06:00 MHDA- 2018/03/20 06:00 CRDT- 2017/04/29 06:00 PHST- 2016/11/16 00:00 [received] PHST- 2017/04/04 00:00 [revised] PHST- 2017/04/04 00:00 [accepted] PHST- 2017/04/30 06:00 [pubmed] PHST- 2018/03/20 06:00 [medline] PHST- 2017/04/29 06:00 [entrez] AID - S0026-2862(16)30198-4 [pii] AID - 10.1016/j.mvr.2017.04.004 [doi] PST - ppublish SO - Microvasc Res. 2017 Sep;113:22-28. doi: 10.1016/j.mvr.2017.04.004. Epub 2017 Apr 25. PMID- 19083656 OWN - NLM STAT- MEDLINE DCOM- 20090318 LR - 20081216 IS - 1532-9283 (Electronic) IS - 1360-8592 (Linking) VI - 12 IP - 1 DP - 2008 Jan TI - Nerve conduction velocities in the lower extremity in patients with Raynaud's phenomenon and clinical applications. PG - 58-66 LID - 10.1016/j.jbmt.2007.10.003 [doi] AB - BACKGROUND AND PURPOSE: The purpose of the study is to study the nerve conductivity of the tibial motor, peroneal motor, peroneal sensory, and sural nerves in patients with primary and secondary Raynaud's phenomenon (RP). SUBJECTS: Twenty each: primary RP, secondary RP, and normal controls. METHODS: Electromyography using distal latency (DL) and nerve conduction velocity (NCV) as dependent variables. RESULTS: Peroneal nerve DLs were slower and NCVs were weaker for the secondary RP group compared to the primary RP group and controls. Tibial motor nerve DLs from slowest to fastest were: primary RP, secondary RP, and controls. NCV strength order was: secondary RP weakest, primary RP, and controls. DISCUSSION: Patients with secondary RP generally had the slowest DLs and the weakest NCVs, with differences most pronounced in the motor nerves. With the exception of the tibial motor nerve, patients with primary RP had similar NCVs to the control group. Neural mobilization techniques can be applied to assist with patient symptoms. FAU - Kostopoulos, Dimitrios AU - Kostopoulos D AD - DIMIPT@aol.com FAU - Rizopoulos, Konstantine AU - Rizopoulos K FAU - Vartholomeos, Nikolaos AU - Vartholomeos N LA - eng PT - Journal Article DEP - 20071128 PL - United States TA - J Bodyw Mov Ther JT - Journal of bodywork and movement therapies JID - 9700068 SB - IM MH - Adult MH - Case-Control Studies MH - Electromyography MH - Female MH - Humans MH - Lower Extremity/*physiopathology MH - Male MH - *Neural Conduction MH - Peroneal Nerve/*physiopathology MH - Raynaud Disease/*physiopathology MH - Sural Nerve/*physiopathology MH - Tibial Nerve/*physiopathology EDAT- 2008/12/17 09:00 MHDA- 2009/03/19 09:00 CRDT- 2008/12/17 09:00 PHST- 2007/08/07 00:00 [received] PHST- 2007/10/17 00:00 [revised] PHST- 2007/10/18 00:00 [accepted] PHST- 2008/12/17 09:00 [entrez] PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/03/19 09:00 [medline] AID - S1360-8592(07)00113-1 [pii] AID - 10.1016/j.jbmt.2007.10.003 [doi] PST - ppublish SO - J Bodyw Mov Ther. 2008 Jan;12(1):58-66. doi: 10.1016/j.jbmt.2007.10.003. Epub 2007 Nov 28. PMID- 30548402 OWN - NLM STAT- MEDLINE DCOM- 20190729 LR - 20190729 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 22 IP - 3 DP - 2019 Mar TI - Raynaud's phenomenon and anti-nuclear antibody are associated with pulmonary function decline in patients with dermatomyositis and polymyositis. PG - 507-515 LID - 10.1111/1756-185X.13456 [doi] AB - OBJECTIVES: To identify factors associated with deterioration of pulmonary function with disproportional decline in diffusing capacity for carbon monoxide (DLCO) relative to forced vital capacity (FVC) in patients with dermatomyositis (DM) and polymyositis (PM). METHODS: This retrospective cohort study included patients with DM and PM, in whom serial pulmonary function tests were available. Changes in FVC and DLCO over time were estimated using a linear mixed-effects model. RESULTS: A total of 103 patients were included. During follow-up, 31 (30.1%) and 37 (35.9%) had a disproportionally better (ΔDLCO/ΔFVC>mean slope + 95% CI) or a disproportionally worse (ΔDLCO/ΔFVC  1.0 and IC > 0. Intra-class and inter-class analyses were conducted to compare SDRs among CGRP inhibitors and other migraine therapies, including triptans, beta-blockers, and anticonvulsants. CGRP inhibitors demonstrated significant SDRs for Raynaud's phenomenon (ROR 19.12; 95% CI 15.44-23.69), with rimegepant, ubrogepant, and atogepant showing particularly strong signals. Intra-class analysis revealed a significant SDR only for galcanezumab (ROR 2.01; 95% CI 1.28-3.17). Inter-class analysis indicated significant SDRs for CGRP inhibitors compared to beta-blockers, anticonvulsants, and celecoxib, but not triptans. These findings underscore the importance of ongoing pharmacovigilance and further research to validate these associations and ensure patient safety. CI - © 2025. The Author(s). FAU - Lee, Nai AU - Lee N AD - College of Pharmacy, Daegu Catholic University, 13-13, Hayang-ro, Hayang-eup, Gyeongsan, Gyeongbuk, 38430, Republic of Korea. FAU - Ok, Ji Hoon AU - Ok JH AD - College of Pharmacy, Daegu Catholic University, 13-13, Hayang-ro, Hayang-eup, Gyeongsan, Gyeongbuk, 38430, Republic of Korea. FAU - Rhee, Su-Jin AU - Rhee SJ AD - Department of Pharmacy, Wonkwang University College of Pharmacy, Iksan, Republic of Korea. FAU - Kim, Yun AU - Kim Y AD - College of Pharmacy, Daegu Catholic University, 13-13, Hayang-ro, Hayang-eup, Gyeongsan, Gyeongbuk, 38430, Republic of Korea. ykim71@cu.ac.kr. LA - eng GR - 2023/Catholic University of Daegu/ GR - RS-2022-00166945/National Research Foundation of Korea/ PT - Journal Article DEP - 20250215 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) SB - IM MH - Humans MH - *Raynaud Disease/chemically induced/epidemiology MH - United States/epidemiology MH - United States Food and Drug Administration MH - *Adverse Drug Reaction Reporting Systems/statistics & numerical data MH - *Calcitonin Gene-Related Peptide/antagonists & inhibitors MH - Retrospective Studies MH - Migraine Disorders/drug therapy MH - *Calcitonin Gene-Related Peptide Receptor Antagonists/adverse effects MH - Female MH - Male PMC - PMC11830029 OTO - NOTNLM OT - Calcitonin gene-related peptide inhibitors OT - Disproportionality analysis OT - Drug safety OT - Migraine OT - Pharmacovigilance OT - Raynaud’s phenomenon COIS- Declarations. Competing interests: The authors declare no competing interests. EDAT- 2025/02/16 01:32 MHDA- 2025/02/16 01:33 PMCR- 2025/02/15 CRDT- 2025/02/15 23:18 PHST- 2024/09/01 00:00 [received] PHST- 2025/01/20 00:00 [accepted] PHST- 2025/02/16 01:33 [medline] PHST- 2025/02/16 01:32 [pubmed] PHST- 2025/02/15 23:18 [entrez] PHST- 2025/02/15 00:00 [pmc-release] AID - 10.1038/s41598-025-87421-w [pii] AID - 87421 [pii] AID - 10.1038/s41598-025-87421-w [doi] PST - epublish SO - Sci Rep. 2025 Feb 15;15(1):5675. doi: 10.1038/s41598-025-87421-w. PMID- 24016612 OWN - NLM STAT- MEDLINE DCOM- 20140624 LR - 20161013 IS - 0929-6646 (Print) IS - 0929-6646 (Linking) VI - 112 IP - 8 DP - 2013 Aug TI - Clinical applicability of quantitative nailfold capillaroscopy in differential diagnosis of connective tissue diseases with Raynaud's phenomenon. PG - 482-8 LID - S0929-6646(12)00233-1 [pii] LID - 10.1016/j.jfma.2012.02.029 [doi] AB - BACKGROUND/PURPOSE: Nailfold capillaroscopy is a useful tool to distinguish primary from secondary Raynaud's phenomenon (RP) by examining the morphology of nailfold capillaries but its role in disease diagnosis is not clearly established. The purpose of this study was to evaluate the roles of quantitative nailfold capillaroscopy in differential diagnosis of connective tissue diseases (CTDs) with RP. METHODS: The data between the year 2005 and 2009 were retrieved from the nailfold capillaroscopic database of National Taiwan University Hospital (NTUH). Only the data from the patients with RP were analyzed. The criteria for interpretation of capillaroscopic findings were predefined. The final diagnoses of the patients were based on the American College of Rheumatology classification criteria for individual diseases, independent of nailfold capillaroscopic findings. The sensitivity and the specificity of each capillaroscopic pattern to the diseases were determined. RESULTS: The data from a total of 67 patients were qualified for the current study. We found the sensitivity and specificity of scleroderma pattern for systemic sclerosis (SSc) were 89.47% and 80%, and the specificity of the early, active, and late scleroderma patterns for SSc reached 87.5%, 97.5%, and 95%, respectively. The sensitivity/specificity of systemic lupus erythematosus (SLE) pattern for SLE and polymyositis/dermatomyositis (PM/DM) pattern for PM/DM were 33.33%/95.45% and 60%/96.3%, respectively. The sensitivity/specificity of mixed connective tissue disease (MCTD) pattern for MCTD were 20%/100%. CONCLUSION: The nailfold capillaroscopic (NC) patterns may be useful in the differential diagnosis of CTDs with RP. The NC patterns for SSc and PM/DM are both sensitive and specific to the diseases, while the SLE and MCTD patterns exhibit high specificity but relatively low sensitivity. CI - Copyright © 2012. Published by Elsevier B.V. FAU - Wu, Po-Chang AU - Wu PC AD - Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan. FAU - Huang, Min-Nung AU - Huang MN FAU - Kuo, Yu-Min AU - Kuo YM FAU - Hsieh, Song-Chou AU - Hsieh SC FAU - Yu, Chia-Li AU - Yu CL LA - eng PT - Journal Article DEP - 20120814 PL - Singapore TA - J Formos Med Assoc JT - Journal of the Formosan Medical Association = Taiwan yi zhi JID - 9214933 SB - IM MH - Adult MH - Aged MH - Connective Tissue Diseases/*diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - Sensitivity and Specificity OTO - NOTNLM OT - Raynaud's phenomenon OT - connective tissue diseases OT - diagnosis OT - nailfold capillaroscopy EDAT- 2013/09/11 06:00 MHDA- 2014/06/25 06:00 CRDT- 2013/09/11 06:00 PHST- 2011/01/01 00:00 [received] PHST- 2012/02/23 00:00 [revised] PHST- 2012/02/29 00:00 [accepted] PHST- 2013/09/11 06:00 [entrez] PHST- 2013/09/11 06:00 [pubmed] PHST- 2014/06/25 06:00 [medline] AID - S0929-6646(12)00233-1 [pii] AID - 10.1016/j.jfma.2012.02.029 [doi] PST - ppublish SO - J Formos Med Assoc. 2013 Aug;112(8):482-8. doi: 10.1016/j.jfma.2012.02.029. Epub 2012 Aug 14. PMID- 25266415 OWN - NLM STAT- MEDLINE DCOM- 20161109 LR - 20161230 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 55 IP - 1 DP - 2016 Jan TI - Paclitaxel-induced diffuse cutaneous sclerosis: a case with associated esophageal dysmotility, Raynaud's phenomenon, and myositis. PG - 97-100 LID - 10.1111/ijd.12437 [doi] FAU - Winkelmann, Richard R AU - Winkelmann RR AD - Intern, O'Bleness Memorial Hospital/OUHCOM, Athens, OH, USA. FAU - Yiannias, James A AU - Yiannias JA AD - Department of Dermatology, Mayo Clinic, Scottsdale, AZ, USA. FAU - DiCaudo, David J AU - DiCaudo DJ AD - Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA. FAU - Trotter, Shannon C AU - Trotter SC AD - Department of Dermatology, The Ohio State University, Wexner Medical Center, Columbus, OH, USA. FAU - Farhey, Yolanda AU - Farhey Y AD - Department of Rheumatology, University of Cincinnati, Cincinnati, OH, USA. FAU - Griffing, W Leroy AU - Griffing WL AD - Department of Rheumatology, Mayo Clinic, Scottsdale, AZ, USA. FAU - Martorano, Lisa M AU - Martorano LM AD - Intern, University Hospitals Richmond Medical Center, Richmond, OH, USA. FAU - Winkelmann, John C AU - Winkelmann JC AD - Oncology Hematology Care, Cincinnati, OH, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20140930 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - 0 (Antineoplastic Agents, Phytogenic) RN - BG3F62OND5 (Carboplatin) RN - P88XT4IS4D (Paclitaxel) SB - IM MH - Adenocarcinoma/pathology/*therapy MH - Antineoplastic Agents, Phytogenic/administration & dosage/*adverse effects MH - Antineoplastic Combined Chemotherapy Protocols MH - Biopsy, Needle MH - Carboplatin/administration & dosage MH - Chemotherapy, Adjuvant MH - Drug Therapy, Combination MH - Esophageal Motility Disorders/chemically induced/drug therapy/pathology MH - Female MH - Humans MH - Immunohistochemistry MH - Middle Aged MH - Myositis/chemically induced/drug therapy/pathology MH - Neoplasm Invasiveness/pathology MH - Neoplasm Staging MH - Ovarian Neoplasms/pathology/*therapy MH - Ovariectomy/methods MH - Paclitaxel/administration & dosage/*adverse effects MH - Prognosis MH - Raynaud Disease/chemically induced/drug therapy/pathology MH - Risk Assessment MH - Scleroderma, Systemic/*chemically induced/pathology MH - Treatment Outcome EDAT- 2014/10/01 06:00 MHDA- 2016/11/10 06:00 CRDT- 2014/10/01 06:00 PHST- 2013/02/21 00:00 [received] PHST- 2013/06/13 00:00 [revised] PHST- 2013/07/25 00:00 [accepted] PHST- 2014/10/01 06:00 [entrez] PHST- 2014/10/01 06:00 [pubmed] PHST- 2016/11/10 06:00 [medline] AID - 10.1111/ijd.12437 [doi] PST - ppublish SO - Int J Dermatol. 2016 Jan;55(1):97-100. doi: 10.1111/ijd.12437. Epub 2014 Sep 30. PMID- 40388975 OWN - NLM STAT- MEDLINE DCOM- 20250519 LR - 20260512 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 150 IP - 12 DP - 2025 Jun TI - [Update Raynaud Phenomenon]. PG - 661-667 LID - 10.1055/a-2365-0537 [doi] AB - Several new aspects concerning Raynaud's phenomenon (RP) are highlighted. Low weight is a known factor for the manifestation of RP, but RP may also be triggered by intended or unintended weight loss. RP is more frequent in presence of depression or anxiety disorders which may be revealed by questionnaires. The socioeconomic effect of RP is small in most of the patients; as far as they aren't engaged in professions with cold exposition, ability to work is not restricted. However, if systemic sclerosis (SSc) is the underlying disease of RP, disability rates are considerable due to finger necrosis and soft tissue damages. Capillaroscopy may be a decisive tool to disclose SSc being the underlying disorder for RP. However, a pathological capillaroscopy pattern is no proof of SSc and not diagnostic if antinuclear antibodies are absent; the predictive value of puffy fingers and specific antibodies is superior. Medical treatment is restricted to severe forms of RP, which are often caused by SSc. Calcium channel blockers have the highest propagation but should not be used in case of digital ulcers. Prostanoids seem to be of greater value, phophodiesterase inhibitors have a small effect and Bosentan may reduce the appearance of new ulcers. Botulinum-A may be considered if ulcers and pain are prevailing. With all options of treatment a considerable placebo effect has to be regarded. Promising case series for several drugs were followed by disillusioning controlled studies. Autologous fat or adipose-derived stem cell grafting is a recent therapeutic approach for SSc patients with severe and disabling course of disease who cannot use their hands anymore, but the best form of application is not yet determined. CI - Thieme. All rights reserved. FAU - Caspary, Ludwig AU - Caspary L AD - Angiologische Praxis, Hannover, Deutschland. LA - ger PT - English Abstract PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review TT - Update Raynaud-Syndrom. DEP - 20250519 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/therapy/etiology MH - Scleroderma, Systemic/complications/diagnosis MH - Microscopic Angioscopy MH - Risk Factors COIS- Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht. EDAT- 2025/05/20 00:30 MHDA- 2025/05/20 00:31 CRDT- 2025/05/19 19:12 PHST- 2025/05/20 00:31 [medline] PHST- 2025/05/20 00:30 [pubmed] PHST- 2025/05/19 19:12 [entrez] AID - 10.1055/a-2365-0537 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2025 Jun;150(12):661-667. doi: 10.1055/a-2365-0537. Epub 2025 May 19. PMID- 28859256 OWN - NLM STAT- MEDLINE DCOM- 20190527 LR - 20190527 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 69 IP - 12 DP - 2017 Dec TI - Editorial: Clinical Trials in Raynaud's Phenomenon: A Spoonful of Sugar (Pill) Makes the Medicine Go Down (in Flames). PG - 2256-2258 LID - 10.1002/art.40307 [doi] FAU - Seibold, James R AU - Seibold JR AD - Medical College of Georgia, Augusta, Georgia. FAU - Wigley, Fredrick M AU - Wigley FM AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. LA - eng PT - Comment PT - Editorial PT - Research Support, Non-U.S. Gov't DEP - 20171110 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Acetamides) RN - 0 (Pyrazines) RN - 0 (Sugars) RN - 5EXC0E384L (selexipag) SB - IM CON - Arthritis Rheumatol. 2017 Dec;69(12):2370-2379. doi: 10.1002/art.40242. PMID: 29193819 MH - Acetamides MH - Adult MH - Humans MH - Pyrazines MH - *Raynaud Disease MH - *Scleroderma, Systemic MH - Sugars EDAT- 2017/09/01 06:00 MHDA- 2019/05/28 06:00 CRDT- 2017/09/01 06:00 PHST- 2017/08/25 00:00 [received] PHST- 2017/08/28 00:00 [accepted] PHST- 2017/09/01 06:00 [pubmed] PHST- 2019/05/28 06:00 [medline] PHST- 2017/09/01 06:00 [entrez] AID - 10.1002/art.40307 [doi] PST - ppublish SO - Arthritis Rheumatol. 2017 Dec;69(12):2256-2258. doi: 10.1002/art.40307. Epub 2017 Nov 10. PMID- 30670098 OWN - NLM STAT- MEDLINE DCOM- 20200330 LR - 20200330 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 59 IP - 1 DP - 2019 Jan 22 TI - Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy. PG - 5 LID - 10.1186/s42358-018-0046-4 [doi] AB - Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction. FAU - Kayser, Cristiane AU - Kayser C AUID- ORCID: 0000-0003-0543-5305 AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Rua Botucatu 740, 3° andar, São Paulo, SP, 04023-062, Brazil. cristiane.kayser@unifesp.br. FAU - Bredemeier, Markus AU - Bredemeier M AD - Rheumatology Service, Hospital Nossa Senhora da Conceição, Grupo Hospitalar Conceição, Porto Alegre, RS, Brazil. FAU - Caleiro, Maria Teresa AU - Caleiro MT AD - Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil. FAU - Capobianco, Karina AU - Capobianco K AD - Rheumatology Service, Moinhos de Vento Hospital, Porto Alegre, Brazil. FAU - Fernandes, Tatiana Melo AU - Fernandes TM AD - Rheumatology Division, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. FAU - de Araújo Fontenele, Sheila Márcia AU - de Araújo Fontenele SM AD - Departament of Medicine, Universidade Estadual do Ceará, Fortaleza, Brazil. FAU - Freire, Eutilia AU - Freire E AD - Rheumatology Service, Universidade Federal da Paraíba, João Pessoa, Brazil. FAU - Lonzetti, Lilian AU - Lonzetti L AD - Rheumatology Service, Complexo Hospitalar da Santa Casa de Misericórdia de Porto Alegre, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, Brazil. FAU - Miossi, Renata AU - Miossi R AD - Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil. FAU - Sekiyama, Juliana AU - Sekiyama J AD - Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil. FAU - de Souza Müller, Carolina AU - de Souza Müller C AD - Rheumatology Division, Hospital de Clínicas, Universidade Federal do Paraná (UFPR), Curitiba, Brazil. LA - eng PT - Journal Article PT - Practice Guideline PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20190122 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 SB - IM MH - Arthritis, Rheumatoid/diagnostic imaging MH - Brazil MH - Capillaries/diagnostic imaging/pathology MH - Dermatomyositis/diagnostic imaging/pathology MH - Early Diagnosis MH - Humans MH - Lupus Erythematosus, Systemic/diagnostic imaging/pathology MH - Microscopic Angioscopy/instrumentation/*methods/standards MH - Mixed Connective Tissue Disease/diagnostic imaging/pathology MH - Raynaud Disease/diagnostic imaging/pathology MH - Rheumatic Diseases/*diagnostic imaging/pathology MH - Rheumatology MH - Scleroderma, Systemic/diagnostic imaging/pathology MH - Societies, Medical MH - Systemic Vasculitis/diagnostic imaging MH - Terminology as Topic OTO - NOTNLM OT - Capillaroscopy OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2019/01/24 06:00 MHDA- 2020/03/31 06:00 CRDT- 2019/01/24 06:00 PHST- 2018/10/05 00:00 [received] PHST- 2018/12/20 00:00 [accepted] PHST- 2019/01/24 06:00 [entrez] PHST- 2019/01/24 06:00 [pubmed] PHST- 2020/03/31 06:00 [medline] AID - 10.1186/s42358-018-0046-4 [pii] AID - 10.1186/s42358-018-0046-4 [doi] PST - epublish SO - Adv Rheumatol. 2019 Jan 22;59(1):5. doi: 10.1186/s42358-018-0046-4. PMID- 19234022 OWN - NLM STAT- MEDLINE DCOM- 20090408 LR - 20090223 IS - 1472-0213 (Electronic) IS - 1472-0205 (Linking) VI - 26 IP - 3 DP - 2009 Mar TI - Raynaud's phenomenon--or just skin with dye? PG - 221-2 LID - 10.1136/emj.2008.062562 [doi] AB - Munchausen's syndrome is a factitious disorder resulting in unnecessary investigations and operative treatments in a small and well-defined population. Autobiographical falsification is the characteristic of the entity. The case history is presented of a 28-year-old woman admitted to the emergency department with severe pain of acute onset in her fingers and discoloration while washing dishes. She had been diagnosed with Raynaud's phenomenon and had been on antiepileptic drugs. The fingertips of both hands looked cyanotic. Radial and ulnar pulses were intact. She had argued with the personnel obtaining vital signs and had a tendency to hide her right hand, which raised the suspicion that a psychiatric disorder was the primary cause of the visit to the emergency department. A blue piece of dirt on the left shoulder had also augmented these concerns. Munchausen's syndrome was suspected after careful handshaking with hands soaked in alcohol resulted in a blue discoloration on the doctor's palm and fingers. Emergency and primary care physicians should be alert to this type of situation, with a myriad possible scenarios to be differentiated from real conditions. FAU - Serinken, M AU - Serinken M AD - Pamukkale University Medical School, Department of Emergency Medicine, 20070 Denizli, Turkey. mserinken@hotmail.com FAU - Karcioglu, O AU - Karcioglu O FAU - Turkcuer, I AU - Turkcuer I FAU - Bukiran, A AU - Bukiran A LA - eng PT - Case Reports PT - Journal Article PL - England TA - Emerg Med J JT - Emergency medicine journal : EMJ JID - 100963089 RN - 0 (Coloring Agents) SB - IM MH - Adult MH - Coloring Agents MH - Cyanosis/diagnosis MH - Diagnosis, Differential MH - Female MH - *Fingers MH - Humans MH - Munchausen Syndrome/*diagnosis/psychology MH - Raynaud Disease/*diagnosis MH - Skin/*pathology EDAT- 2009/02/24 09:00 MHDA- 2009/04/09 09:00 CRDT- 2009/02/24 09:00 PHST- 2009/02/24 09:00 [entrez] PHST- 2009/02/24 09:00 [pubmed] PHST- 2009/04/09 09:00 [medline] AID - 26/3/221 [pii] AID - 10.1136/emj.2008.062562 [doi] PST - ppublish SO - Emerg Med J. 2009 Mar;26(3):221-2. doi: 10.1136/emj.2008.062562. PMID- 39082149 OWN - NLM STAT- MEDLINE DCOM- 20250117 LR - 20250514 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 9 IP - 1 DP - 2025 Jan 16 TI - A case of Buerger's disease with vasculopathy and skin fibrosis requiring differential diagnosis from systemic sclerosis. PG - 127-130 LID - 10.1093/mrcr/rxae036 [doi] AB - Buerger's disease is characterised by peripheral ischaemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger's disease in a 51-year-old male who presented with findings resembling systemic sclerosis (SSc). The patient exhibited Raynaud's phenomenon in year 2015, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year 2018. Diagnostic testing showed positive microvasculopathy on nailfold videocapillaroscopy (NVC) and positive fibrosis on skin biopsy. Although the patient fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc, several findings in this case were atypical for SSc, including left-right asymmetry in finger involvement, nail loss, and negative autoantibody tests. Contrast-enhanced computed tomography showed poor perfusion of the right ulnar artery, and a heavy smoking history was established in the patient case. Therefore, based on Shionoya's criteria, he was diagnosed with a case of Buerger's disease confined to the upper extremity. Smoking cessation and vasodilator therapy resulted in the prompt resolution of ischaemic symptoms, skin hardening, and ulcerations. Furthermore, NVC abnormalities improved, and ulnar artery occlusion showed reperfusion on repeat testing. The present case suggests that hypoxaemia-driven microvasculopathy may contribute to vascular occlusion and skin fibrosis observed in this atypical presentation. CI - © Japan College of Rheumatology 2025. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com. FAU - Funada, Masashi AU - Funada M AUID- ORCID: 0000-0003-2586-246X AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Nakayamada, Shingo AU - Nakayamada S AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Fukuyo, Shunsuke AU - Fukuyo S AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Kubo, Satoshi AU - Kubo S AUID- ORCID: 0000-0001-9693-9263 AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. AD - Department of Molecular Targeted Therapies, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Nawata, Aya AU - Nawata A AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. AD - Department of Pathology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Fujita, Yuya AU - Fujita Y AUID- ORCID: 0000-0003-4163-9356 AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Tanaka, Yoshiya AU - Tanaka Y AUID- ORCID: 0000-0002-0807-7139 AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 SB - IM MH - Humans MH - Male MH - Middle Aged MH - *Thromboangiitis Obliterans/diagnosis/complications/pathology MH - *Scleroderma, Systemic/diagnosis MH - Diagnosis, Differential MH - *Skin/pathology MH - Fibrosis MH - Raynaud Disease/etiology MH - Microscopic Angioscopy OTO - NOTNLM OT - Buerger’s disease OT - Raynaud’s phenomenon OT - nailfold videocapillaroscopy OT - systemic sclerosis OT - thromboangiitis obliterans EDAT- 2024/07/31 06:42 MHDA- 2025/01/17 18:22 CRDT- 2024/07/31 05:13 PHST- 2024/02/08 00:00 [received] PHST- 2024/06/19 00:00 [revised] PHST- 2024/07/12 00:00 [accepted] PHST- 2025/01/17 18:22 [medline] PHST- 2024/07/31 06:42 [pubmed] PHST- 2024/07/31 05:13 [entrez] AID - 7724631 [pii] AID - 10.1093/mrcr/rxae036 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2025 Jan 16;9(1):127-130. doi: 10.1093/mrcr/rxae036. PMID- 31451935 OWN - NLM STAT- MEDLINE DCOM- 20210209 LR - 20210209 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 40 IP - 2 DP - 2020 Feb TI - Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic. PG - 295-301 LID - 10.1007/s00296-019-04427-0 [doi] AB - Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study. Clinical data from patients attending our NFC clinic were reviewed. Inclusion criteria included AICTD previous diagnosis, which included systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), Sjögren syndrome, inflammatory idiopathic myopathies (IIM), rheumatoid arthritis, undifferentiated connective tissue disease and antiphospholipid syndrome (APS). Videocap(®) version 3.0 biomicroscope was used. NFC score was determined. For statistics, SPSS software was utilized. 384 patients were included; most of them were women, with mean age of 47 years. RP was present in 91% of the patients, with greater prevalence in SSc and MCTD. Scleroderma pattern was the most prevalent NFC pattern, mainly in SSc, MCTD and IIM. Mean capillary density was reduced in IIM, SSc and MCTD. NFC score was worse in SSc, IIM and MCTD. In patients with AICTD, RP is related to microvascular damage and worse NFC score. NFC scleroderma pattern correlates with SSc classification criteria score. In MCTD, scleroderma pattern relates to myositis. SLE and APS reveal significant hemorrhages, but not related to APS antibodies. This study highlights the possible role of NFC as biomarker of AICTD, particularly in SSc and IIM. FAU - Bernardino, Vera AU - Bernardino V AUID- ORCID: 0000-0002-8492-5910 AD - Internal Medicine Department 7.2, Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Rua da Beneficência, 8, 1069-166, Lisbon, Portugal. verarodriguesb@gmail.com. FAU - Rodrigues, Ana AU - Rodrigues A AD - Internal Medicine Department 7.2, Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Rua da Beneficência, 8, 1069-166, Lisbon, Portugal. FAU - Lladó, Ana AU - Lladó A AD - Internal Medicine Department 7.2, Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Rua da Beneficência, 8, 1069-166, Lisbon, Portugal. FAU - Panarra, António AU - Panarra A AD - Internal Medicine Department 7.2, Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Rua da Beneficência, 8, 1069-166, Lisbon, Portugal. LA - eng PT - Journal Article DEP - 20190826 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Antiphospholipid Syndrome/diagnostic imaging/epidemiology/physiopathology MH - Arthritis, Rheumatoid/diagnostic imaging/epidemiology/physiopathology MH - Autoimmune Diseases/*diagnostic imaging/epidemiology/physiopathology MH - Connective Tissue Diseases/*diagnostic imaging/epidemiology/physiopathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/diagnostic imaging/epidemiology/physiopathology MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Mixed Connective Tissue Disease/diagnostic imaging/epidemiology/physiopathology MH - Myositis/diagnostic imaging/epidemiology/physiopathology MH - Portugal/epidemiology MH - Raynaud Disease/*diagnostic imaging/epidemiology/physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/diagnostic imaging/epidemiology/physiopathology MH - Sjogren's Syndrome/diagnostic imaging/epidemiology/physiopathology MH - Undifferentiated Connective Tissue Diseases/diagnostic imaging/epidemiology/physiopathology MH - Young Adult OTO - NOTNLM OT - Autoimmune disease OT - Biomarkers OT - Nailfold capillaroscopy OT - Raynaud’s phenomenon EDAT- 2019/08/28 06:00 MHDA- 2021/02/10 06:00 CRDT- 2019/08/28 06:00 PHST- 2019/06/23 00:00 [received] PHST- 2019/08/19 00:00 [accepted] PHST- 2019/08/28 06:00 [pubmed] PHST- 2021/02/10 06:00 [medline] PHST- 2019/08/28 06:00 [entrez] AID - 10.1007/s00296-019-04427-0 [pii] AID - 10.1007/s00296-019-04427-0 [doi] PST - ppublish SO - Rheumatol Int. 2020 Feb;40(2):295-301. doi: 10.1007/s00296-019-04427-0. Epub 2019 Aug 26. PMID- 24960119 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - Nailfold capillaroscopy in juvenile rheumatic diseases: known measures, patterns and indications. PG - S-183-8 AB - Nailfold capillaroscopy has become an established method in adults for the evaluation of structural abnormalities of the microcirculation associated with rheumatic disease. It is a cornerstone for the diagnostic work-up of patients with Raynaud's phenomenon and the early diagnosis of systemic sclerosis. However, this non-invasive examination may also be valuable in children and adolescents with rheumatic diseases. Based on the scarce data available, this review focuses on capillaroscopic findings in healthy children and adolescents as well as in children with juvenile systemic sclerosis, juvenile dermatomyositis, juvenile idiopathic arthritis, and Raynaud's phenomenon. In addition, it outlines the potential benefits and limitations of nailfold capillaroscopy for routine care in paediatric rheumatology. FAU - Gerhold, K AU - Gerhold K AD - Kinder und Jugendrheumatologie, Sozialpädiatrisches Zentrum, Charité-Universitätsmedizin, Berlin, Germany. kerstin.gerhold@charite.de. FAU - Becker, M O AU - Becker MO LA - eng PT - Journal Article PT - Review DEP - 20140606 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - Amyopathic dermatomyositis RN - Juvenile systemic scleroderma SB - IM MH - Adolescent MH - Autoimmune Diseases/diagnosis MH - Child MH - Dermatomyositis/*diagnosis MH - Fingers/*blood supply MH - Humans MH - Microcirculation MH - Microscopic Angioscopy/*methods MH - *Microvessels MH - Raynaud Disease/*diagnosis MH - Rheumatic Diseases/diagnosis MH - Scleroderma, Systemic/*diagnosis EDAT- 2014/06/25 06:00 MHDA- 2015/10/31 06:00 CRDT- 2014/06/25 06:00 PHST- 2013/06/24 00:00 [received] PHST- 2014/01/23 00:00 [accepted] PHST- 2014/06/25 06:00 [entrez] PHST- 2014/06/25 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 7298 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-183-8. Epub 2014 Jun 6. PMID- 39783848 OWN - NLM STAT- MEDLINE DCOM- 20250429 LR - 20260527 IS - 1097-0274 (Electronic) IS - 0271-3586 (Print) IS - 0271-3586 (Linking) VI - 68 IP - 4 DP - 2025 Apr TI - Risk of Raynaud's Phenomenon Among Workers in the Occupational Disease Surveillance System. PG - 344-357 LID - 10.1002/ajim.23700 [doi] AB - INTRODUCTION: Raynaud's phenomenon (RP) is linked to occupational exposures such as vibration, cold temperature, and chemicals. However, large cohort studies examining RP by occupation and sex are scarce. To address this gap, this study aimed to assess risk of RP by both occupation and sex in a large cohort of workers in Ontario, Canada. METHODS: Workers with accepted lost-time compensation claims were linked to physician billing records to identify diagnoses of RP between 2002 and 2020. A 3-year washout (disease-free) period was applied, and follow-up was limited to 5 years. Cox proportional hazard models were used to estimate hazard ratios (HR) and 95% confidence intervals (CI) for diagnoses of RP, adjusted for age at start of follow-up, birth year, and stratified by sex. RESULTS: A total of 7,131 RP cases were identified among 810,739 workers. Among men, higher risks were observed for truck drivers (HR = 1.23, 95% CI = 1.08-1.41), driver-salesmen (HR = 2.54, 95% CI = 1.21-5.34), those in mining and quarrying-related cutting, handling, and loading (HR = 2.57, 95% CI = 1.29-5.15), and construction trades laboring and elemental work (HR = 1.70, 95% CI = 1.24-2.34). Among women, higher risks were observed for those working in waitressing and related (HR = 1.70, 95% CI = 1.22-2.38), food and beverage preparation (HR = 1.34, 95% CI = 1.02-1.76), and electrical equipment fabricating and assembling (HR 1.96, 95% CI = 1.08-3.55). CONCLUSION: Study findings show elevated risks of RP among various occupations, with notable differences between men and women. These differences may be attributable to variations in potential exposures and susceptibility to RP. Findings underscore the need for large cohort studies to examine RP across various occupational groups and both sexes. CI - © 2025 The Author(s). American Journal of Industrial Medicine published by Wiley Periodicals LLC. FAU - Yeo, Ryann E AU - Yeo RE AUID- ORCID: 0009-0005-4280-9881 AD - Occupational Cancer Research Centre, Ontario Health, Toronto, Ontario, Canada. FAU - Eros, Fanni R AU - Eros FR AD - Occupational Cancer Research Centre, Ontario Health, Toronto, Ontario, Canada. FAU - Demers, Paul A AU - Demers PA AD - Occupational Cancer Research Centre, Ontario Health, Toronto, Ontario, Canada. AD - Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada. FAU - Sritharan, Jeavana AU - Sritharan J AD - Occupational Cancer Research Centre, Ontario Health, Toronto, Ontario, Canada. AD - Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada. LA - eng GR - This work was supported by the Ontario Ministry of Labour, Immigration, Training and Skills Development (14-R-029). The Occupational Cancer Research Centre is supported by the Ontario Ministry of Labour, Immigration, Training and Skills Development and the Ontario Health agency./ PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20250109 PL - United States TA - Am J Ind Med JT - American journal of industrial medicine JID - 8101110 SB - IM MH - Humans MH - *Raynaud Disease/epidemiology/etiology MH - Male MH - Female MH - Adult MH - Ontario/epidemiology MH - Middle Aged MH - *Occupational Exposure/adverse effects/statistics & numerical data MH - *Occupational Diseases/epidemiology/etiology MH - Proportional Hazards Models MH - Risk Factors MH - *Occupations/statistics & numerical data MH - Sex Factors MH - Workers' Compensation/statistics & numerical data MH - Cohort Studies PMC - PMC11898170 OTO - NOTNLM OT - Raynaud's phenomenon OT - occupation OT - sex differences OT - surveillance OT - vibration white finger COIS- The authors declare no conflicts of interest. EDAT- 2025/01/09 17:01 MHDA- 2025/03/12 12:31 PMCR- 2025/03/12 CRDT- 2025/01/09 07:53 PHST- 2024/12/11 00:00 [revised] PHST- 2024/09/04 00:00 [received] PHST- 2024/12/18 00:00 [accepted] PHST- 2025/03/12 12:31 [medline] PHST- 2025/01/09 17:01 [pubmed] PHST- 2025/01/09 07:53 [entrez] PHST- 2025/03/12 00:00 [pmc-release] AID - AJIM23700 [pii] AID - 10.1002/ajim.23700 [doi] PST - ppublish SO - Am J Ind Med. 2025 Apr;68(4):344-357. doi: 10.1002/ajim.23700. Epub 2025 Jan 9. PMID- 24112969 OWN - NLM STAT- MEDLINE DCOM- 20160422 LR - 20250711 IS - 1752-8526 (Electronic) VI - 2013 DP - 2013 Oct 10 TI - Raynaud's phenomenon (primary). PG - 1119 LID - 1119 [pii] AB - INTRODUCTION: Raynaud's phenomenon is an episodic, reversible vasospasm of the peripheral arteries (usually digital). It causes pallor, followed by cyanosis and/or redness, often with pain and, at times, paraesthesia. On rare occasions, it can lead to ulceration of the fingers and toes (and, in some cases, of the ears or nose). This review focuses on primary (idiopathic) Raynaud's phenomenon, occurring in the absence of an underlying disease. The prevalence of primary Raynaud's phenomenon varies by sex, country, and exposure to workplace vibration. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical question: What are the effects of drug treatments for primary Raynaud's phenomenon? We searched: Medline, Embase, The Cochrane Library, and other important databases up to August 2013 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found 9 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review we present information relating to the effectiveness and safety of the following interventions: amlodipine, diltiazem, nicardipine, and nifedipine. FAU - Pope, Janet AU - Pope J AD - St Joseph's Health Care Rheumatology Centre, London, Ontario, Canada. LA - eng PT - Journal Article PT - Systematic Review DEP - 20131010 PL - England TA - BMJ Clin Evid JT - BMJ clinical evidence JID - 101294314 RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Administration, Oral MH - Humans MH - *Nifedipine/therapeutic use MH - Prevalence MH - *Raynaud Disease/drug therapy MH - Ulcer MH - Vibration PMC - PMC3794700 EDAT- 2013/10/12 06:00 MHDA- 2016/04/23 06:00 PMCR- 2015/10/10 CRDT- 2013/10/12 06:00 PHST- 2013/10/12 06:00 [entrez] PHST- 2013/10/12 06:00 [pubmed] PHST- 2016/04/23 06:00 [medline] PHST- 2015/10/10 00:00 [pmc-release] AID - 1119 [pii] PST - epublish SO - BMJ Clin Evid. 2013 Oct 10;2013:1119. PMID- 30238433 OWN - NLM STAT- MEDLINE DCOM- 20181231 LR - 20210323 IS - 1179-1918 (Electronic) IS - 1173-2563 (Linking) VI - 38 IP - 11 DP - 2018 Nov TI - Riociguat for the Treatment of Raynaud's Phenomenon: A Single-Dose, Double-Blind, Randomized, Placebo-Controlled Cross-Over Pilot Study (DIGIT). PG - 1061-1069 LID - 10.1007/s40261-018-0698-1 [doi] AB - BACKGROUND AND OBJECTIVE: Raynaud's phenomenon (RP) is characterized by transient digital ischemia and is commonly associated with connective tissue disease. Treatment remains unsatisfactory. Here we evaluate the efficacy, safety, and pharmacokinetics of a single dose of the soluble guanylate cyclase stimulator riociguat in RP. METHODS: DIGIT was a double-blind, randomized, placebo-controlled pilot study. Patients with primary or secondary RP were randomized to a single oral dose of riociguat 2 mg or placebo in a cross-over design (7 ± 3 days). Efficacy was assessed as placebo-corrected change in digital blood flow 2 h post-dose at room temperature (RT) or following cold exposure (CE), measured by laser-speckle contrast analysis. Patients were regarded as responders if placebo-corrected digital blood flow increased by ≥ 10% from baseline at RT or after CE. RESULTS: Of 20 eligible patients, 17 (85%) were female and mean [standard deviation (SD)] age was 52 (13.8) years. Placebo-corrected changes in digital blood flow were + 46% [90% confidence interval (CI) - 6 to + 98] at RT and - 9% (90% CI - 63 to + 44) after CE, with high inter-individual variability. Eight patients (40%) were responders at RT, and 12 (60%) after CE. Riociguat increased mean (SD) digital blood flow in responders at RT by + 136% (114) and in responders following CE by + 39% (53). Riociguat was well tolerated, with few adverse events. CONCLUSION: In this pilot study, single-dose riociguat was well tolerated in patients with RP and resulted in improved digital blood flow in some patient subsets, with high inter-individual variability. Long-term evaluation is warranted. FAU - Huntgeburth, Michael AU - Huntgeburth M AD - Clinic III for Internal Medicine, Department of Cardiology, Heart Center, University Hospital of Cologne, Kerpener Str. 62, 50937, Cologne, Germany. FAU - Kießling, Johannes AU - Kießling J AD - Clinic III for Internal Medicine, Department of Cardiology, Heart Center, University Hospital of Cologne, Kerpener Str. 62, 50937, Cologne, Germany. FAU - Weimann, Gerrit AU - Weimann G AD - Bayer AG, Berlin, Germany. FAU - Wilberg, Verena AU - Wilberg V AD - ClinStat GmbH, Cologne, Germany. FAU - Saleh, Soundos AU - Saleh S AD - Bayer AG, Berlin, Germany. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology, University of Cologne, Cologne, Germany. FAU - Rosenkranz, Stephan AU - Rosenkranz S AD - Clinic III for Internal Medicine, Department of Cardiology, Heart Center, University Hospital of Cologne, Kerpener Str. 62, 50937, Cologne, Germany. stephan.rosenkranz@uk-koeln.de. AD - Heart Center, Cologne Cardiovascular Research Center (CCRC), Cologne, Germany. stephan.rosenkranz@uk-koeln.de. LA - eng PT - Journal Article PT - Randomized Controlled Trial PL - New Zealand TA - Clin Drug Investig JT - Clinical drug investigation JID - 9504817 RN - 0 (Enzyme Activators) RN - 0 (Pyrazoles) RN - 0 (Pyrimidines) RN - RU3FE2Y4XI (riociguat) SB - IM MH - Administration, Oral MH - Adult MH - Aged MH - Blood Flow Velocity/*drug effects/physiology MH - Cross-Over Studies MH - Double-Blind Method MH - Enzyme Activators/*administration & dosage MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Pilot Projects MH - Pyrazoles/*administration & dosage MH - Pyrimidines/*administration & dosage MH - Raynaud Disease/diagnosis/*drug therapy/physiopathology MH - Treatment Outcome EDAT- 2018/09/22 06:00 MHDA- 2019/01/01 06:00 CRDT- 2018/09/22 06:00 PHST- 2018/09/22 06:00 [pubmed] PHST- 2019/01/01 06:00 [medline] PHST- 2018/09/22 06:00 [entrez] AID - 10.1007/s40261-018-0698-1 [pii] AID - 10.1007/s40261-018-0698-1 [doi] PST - ppublish SO - Clin Drug Investig. 2018 Nov;38(11):1061-1069. doi: 10.1007/s40261-018-0698-1. PMID- 26590619 OWN - NLM STAT- MEDLINE DCOM- 20160419 LR - 20151123 IS - 0300-8495 (Print) IS - 0300-8495 (Linking) VI - 44 IP - 11 DP - 2015 TI - Nailfold dermatoscopy in general practice. PG - 809-12 AB - BACKGROUND: Nailfold capillary examination can assist in distinguishing between primary Raynaud's phenomenon and secondary Raynaud's that is associated with a connective tissue disease. Dermatoscopy is a reliable technique in the evaluation of nailfold capillaries and assists in the diagnosis of connective tissue diseases such as scleroderma. OBJECTIVE: This article provides an overview of the usefulness of nailfold capillary dermatoscopy in rheumatic and non-rheumatic diseases, and includes the MDAD (morphology, diameter, architecture and density) approach to nailfold dermatoscopy. DISCUSSION: Dermatoscopes are useful devices in examining nailfold capillaries. Many general practitioners are skilled in dermatoscopy and are well placed to examine nailfold capillaries. The MDAD approach to nailfold dermatoscopy considers capillary morphology, diameter, architecture and density. In Raynaud's phenomenon, nailfold dermatoscopy assists in the diagnosis of an underlying connective tissue disease. FAU - Rennie, Darryn AU - Rennie D AD - MBBS, FRACGP, DipACSCM, General Practitioner, Dalyellup Family Medical Centre, and Clinical Senior Lecturer School of Primary, Aboriginal and Rural Health Care with the University of Western Australia, Dalyellup, WA. LA - eng PT - Journal Article PT - Review PL - Australia TA - Aust Fam Physician JT - Australian family physician JID - 0326701 SB - IM MH - Capillaries/pathology MH - Connective Tissue Diseases/*diagnosis MH - Dermoscopy/*methods MH - General Practice/*methods MH - Humans MH - Nails/blood supply/*pathology MH - Raynaud Disease/*diagnosis EDAT- 2015/11/23 06:00 MHDA- 2016/04/20 06:00 CRDT- 2015/11/23 06:00 PHST- 2015/11/23 06:00 [entrez] PHST- 2015/11/23 06:00 [pubmed] PHST- 2016/04/20 06:00 [medline] PST - ppublish SO - Aust Fam Physician. 2015;44(11):809-12. PMID- 37691224 OWN - NLM STAT- MEDLINE DCOM- 20240418 LR - 20240418 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 20 IP - 2 DP - 2024 TI - The Value of Nailfold Capillaroscopy in the Classification and Differential Diagnosis of Raynaud's Phenomenon in Rheumatology. PG - 108-114 LID - 10.2174/1573397119666230905151903 [doi] AB - Among instrumental techniques, nailfold capillaroscopy plays a leading role in the assessment of Raynaud's phenomenon (RP) patients because it is the only method that provides opportunities for morphological assessment of capillaroscopic findings in the nailfold area, with proven diagnostic and prognostic significance in rheumatology. The discussion about updating the classification of RP in rheumatology is interesting given the current understanding of capillaroscopic findings in rheumatic diseases and improvements in immunological diagnostics. The presence of dilation of the "true" capillary diameters in primary RP could be observed. There are some cases of primary RP where the capillaroscopic pattern is completely normal and there are no dilated capillaries present, which could be related to the duration and severity of the symptoms. It is possible that longer duration and greater severity are associated with the appearance of capillary dilations, but more research is needed to confirm it. Rarely, pathological capillaroscpic features of microangiopathy could be observed in RP patients in whom clinical, laboratory and immunological findings are compatible with the diagnosis "primary RP". These cases should be defined as "suspected secondary RP" and require closer follow-up for the assessment of symptom evolution. Abnormal "scleroderma" type capillaroscopic pattern has been established as a new classification criterion for systemic sclerosis (SSc) in 2013. Similar changes ("scleroderma-like" pattern) could be observed in other rheumatic diseases, i.e., undifferentiated connective tissue disease (UCTD), systemic lupus erythematosus, dermatomyositis, rheumatoid arthritis, including without evidence of overlap with scleroderma. The appearance of such microvascular abnormalities at disease presentation is less well studied in diseases different from SSc. However, "scleroderma-like" microangiopathy has also been reported as an initial sign in some systemic rheumatic diseases, such as UCTD and systemic lupus erythematosus. Thus, interpretation of capillaroscopic findings is performed in overall context, including clinical findings and laboratory and immunological test results. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Department of Propaedeutics of Internal Diseases "Prof Dr. Anton Mitov", Faculty of Medicine, Medical University of Plovdiv, 4002 Plovdiv, Bulgaria. AD - Department of Rheumatology, MHAT "Sveti Mina", 4002 Plovdiv, Bulgaria. LA - eng PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Humans MH - Microscopic Angioscopy/methods MH - *Connective Tissue Diseases MH - *Rheumatology MH - Diagnosis, Differential MH - *Rheumatic Diseases/diagnosis MH - Capillaries/diagnostic imaging MH - *Lupus Erythematosus, Systemic/complications MH - *Scleroderma, Systemic/complications/diagnostic imaging MH - *Raynaud Disease/complications MH - *Scleroderma, Localized/pathology OTO - NOTNLM OT - Capillaroscopy OT - Raynaud’s phenomenon OT - capillary dilations OT - classification OT - microangiopathy. OT - undifferentiated connective tissue disease EDAT- 2023/09/11 06:42 MHDA- 2024/04/18 06:44 CRDT- 2023/09/11 00:43 PHST- 2023/06/02 00:00 [received] PHST- 2023/07/20 00:00 [revised] PHST- 2023/08/10 00:00 [accepted] PHST- 2024/04/18 06:44 [medline] PHST- 2023/09/11 06:42 [pubmed] PHST- 2023/09/11 00:43 [entrez] AID - CRR-EPUB-134290 [pii] AID - 10.2174/1573397119666230905151903 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2024;20(2):108-114. doi: 10.2174/1573397119666230905151903. PMID- 16527790 OWN - NLM STAT- MEDLINE DCOM- 20060829 LR - 20131121 IS - 0832-610X (Print) IS - 0832-610X (Linking) VI - 53 IP - 3 DP - 2006 Mar TI - Bilateral pain relief after unilateral thoracic percutaneous sympathectomy. PG - 258-62 AB - PURPOSE: To present a case of unexpected bilateral pain relief following unilateral thoracic percutaneous sympathectomy. CLINICAL FINDINGS: We present a case report where severe ischemic pain due to paraneoplastic Raynaud's syndrome with distal gangrene was successfully treated by means of percutaneous thoracic sympathectomy. A unilateral T2, T3 radiofrequency sympathectomy combined with small volume phenol injection resulted in unexpected bilateral pain relief. CONCLUSION: Our observations from this case report suggest a possible crossover of sympathetic innervation at the cervical and thoracic levels. Percutanenous thoracic radiofrequency sympathectomy is a feasible option for the treatment of refractory ischemic upper limb pain. FAU - Gofeld, Michael AU - Gofeld M AD - Department of Anesthesia, Sunnybrook & Women's College Health Sciences Centre, University of Toronto, Ontario M4N 3M5, Canada. mgofeld@rogers.com FAU - Faclier, Gil AU - Faclier G LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Can J Anaesth JT - Canadian journal of anaesthesia = Journal canadien d'anesthesie JID - 8701709 RN - 0 (Sclerosing Solutions) RN - 339NCG44TV (Phenol) SB - IM MH - Aged MH - Electrocoagulation/instrumentation/methods MH - Fingers/blood supply/innervation/physiopathology MH - Gangrene/complications MH - Humans MH - Male MH - Pain/physiopathology/*surgery MH - Pain Measurement MH - Paraneoplastic Syndromes/complications MH - Phenol/therapeutic use MH - Raynaud Disease/complications/*surgery MH - Sclerosing Solutions/therapeutic use MH - Sympathectomy/instrumentation/*methods MH - Thoracic Nerves/*surgery MH - Treatment Outcome EDAT- 2006/03/11 09:00 MHDA- 2006/08/30 09:00 CRDT- 2006/03/11 09:00 PHST- 2006/03/11 09:00 [pubmed] PHST- 2006/08/30 09:00 [medline] PHST- 2006/03/11 09:00 [entrez] AID - 53/3/258 [pii] AID - 10.1007/BF03022212 [doi] PST - ppublish SO - Can J Anaesth. 2006 Mar;53(3):258-62. doi: 10.1007/BF03022212. PMID- 36040673 OWN - NLM STAT- MEDLINE DCOM- 20221115 LR - 20240406 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 12 DP - 2022 Dec TI - Nailfold capillaroscopy: tips and challenges. PG - 3629-3640 LID - 10.1007/s10067-022-06354-1 [doi] AB - Although nailfold capillaroscopy (NFC) appears to have a bright future in clinical practice, the lack of familiarity with the technique and how to interpret its outcomes is major barriers which have made nailfold capillaroscopy an underutilized method in standard clinical practice. Traditional methods for assessment and measurement of capillary patterns, density, and blood flow are falling behind and face some challenges. In fact, there have been calls for improvement, hence the recent publication of the standardization of NFC by the EULAR Study Group on Microcirculation in Rheumatic Diseases. Nailfold capillaroscopy has the advantage of being a non-invasive technique that provides a window into the digital microcirculation. This paved the way for a rapidly growing interest in using capillaroscopy parameters as outcome measures in research. In standard clinical practice, whilst its main application is in the identification of an underlying systemic sclerosis spectrum disorder in patients presenting with Raynaud's phenomenon, its use has expanded to include other clinical features possibly suggestive of an underlying connective tissue disease. This article presents the challenges, provides tips, and highlights the exciting potential of nailfold capillaroscopy in standard practice. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - El Miedany, Yasser AU - El Miedany Y AUID- ORCID: 0000-0002-4543-692X AD - Canterbury Christ Church University, Institute of Medical Sciences, Canterbury, England, UK. yasserelmiedany@gmail.com. FAU - Ismail, Sherif AU - Ismail S AUID- ORCID: 0000-0002-4465-1089 AD - Rheumatology and Rehabilitation, Internal Medicine Department, National Research Center, Cairo, Egypt. FAU - Wadie, Mary AU - Wadie M AUID- ORCID: 0000-0002-7067-2115 AD - Internal Medicine Cairo University, Cairo, Egypt. FAU - Hassan, Mohammed AU - Hassan M AUID- ORCID: 0000-0001-8436-8598 AD - Rheumatology and Rehabilitation, Tanta University, Tanta, Egypt. LA - eng PT - Journal Article PT - Review DEP - 20220830 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - Microscopic Angioscopy/methods MH - Nails/diagnostic imaging/blood supply MH - *Raynaud Disease/diagnostic imaging MH - Capillaries/diagnostic imaging MH - *Connective Tissue Diseases/diagnostic imaging MH - *Scleroderma, Systemic/diagnostic imaging OTO - NOTNLM OT - Blood flow OT - Challenges OT - Connective tissue disease OT - Microangiopathy OT - Musculoskeletal diseases OT - Nailfold capillaroscopy (NFC) OT - Raynaud’s phenomenon OT - Standards OT - Systemic sclerosis OT - Tips EDAT- 2022/08/31 06:00 MHDA- 2022/11/16 06:00 CRDT- 2022/08/30 11:25 PHST- 2022/06/07 00:00 [received] PHST- 2022/08/23 00:00 [accepted] PHST- 2022/08/22 00:00 [revised] PHST- 2022/08/31 06:00 [pubmed] PHST- 2022/11/16 06:00 [medline] PHST- 2022/08/30 11:25 [entrez] AID - 10.1007/s10067-022-06354-1 [pii] AID - 10.1007/s10067-022-06354-1 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Dec;41(12):3629-3640. doi: 10.1007/s10067-022-06354-1. Epub 2022 Aug 30. PMID- 32147386 OWN - NLM STAT- MEDLINE DCOM- 20201113 LR - 20201113 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 34 IP - 1 DP - 2020 Feb TI - Scleroderma mimics - Clinical features and management. PG - 101489 LID - S1521-6942(20)30006-1 [pii] LID - 10.1016/j.berh.2020.101489 [doi] AB - Systemic sclerosis is a severe immune-mediated rheumatic disease by virtue of its clinical impact and mortality. There are a number of other sclerosing skin diseases that should be considered in the differential diagnosis and these are important because they may require specialist investigation and management. In addition, long-term follow up of the different conditions should reflect the risk of associated complications and anticipated duration of therapy. This article reviews the clinical features of potential mimics of scleroderma (systemic sclerosis) including localised forms of scleroderma (morphoea) and other conditions that lead to skin thickening and connective tissue fibrosis or scarring. CI - Copyright © 2020 Elsevier Ltd. All rights reserved. FAU - Orteu, Catherine H AU - Orteu CH AD - Department of Dermatology, Royal Free London NHS Foundation Trust, Pond Street, London NW3 2QG, UK. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK. Electronic address: c.denton@ucl.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20200305 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Diagnosis, Differential MH - Humans MH - *Raynaud Disease/diagnosis MH - *Rheumatic Diseases MH - *Scleroderma, Localized/diagnosis/therapy MH - *Scleroderma, Systemic/diagnosis/therapy OTO - NOTNLM OT - Fasciitis OT - Graft versus host disease OT - Morphoea OT - Nephrogenic systemic fibrosis OT - Raynaud's phenomenon OT - Scleroderma OT - Scleromyxoedema OT - Systemic sclerosis COIS- Declaration of Competing Interest None. EDAT- 2020/03/10 06:00 MHDA- 2020/11/18 06:00 CRDT- 2020/03/10 06:00 PHST- 2020/03/10 06:00 [pubmed] PHST- 2020/11/18 06:00 [medline] PHST- 2020/03/10 06:00 [entrez] AID - S1521-6942(20)30006-1 [pii] AID - 10.1016/j.berh.2020.101489 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2020 Feb;34(1):101489. doi: 10.1016/j.berh.2020.101489. Epub 2020 Mar 5. PMID- 24951147 OWN - NLM STAT- MEDLINE DCOM- 20150202 LR - 20140621 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 141 IP - 6-7 DP - 2014 Jun-Jul TI - [Improvement of idiopathic Raynaud's phenomenon following local infusion of botulinum toxin]. PG - 462-3 LID - S0151-9638(14)00117-3 [pii] LID - 10.1016/j.annder.2014.03.003 [doi] FAU - Roguedas, A-M AU - Roguedas AM AD - Service de dermatologie, CHU de Brest, 5, avenue Foch, 29609 Brest cedex, France. Electronic address: anne-marie.roguedas-contios@chu-brest.fr. FAU - Misery, L AU - Misery L AD - Service de dermatologie, CHU de Brest, 5, avenue Foch, 29609 Brest cedex, France. LA - fre PT - Case Reports PT - Letter TT - Rémission d'un phénomène de Raynaud idiopathique après injection de toxine botulique. DEP - 20140408 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Acetylcholine Release Inhibitors) RN - 0 (Antihypertensive Agents) RN - 0 (Calcium Channel Blockers) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - EC 3.4.24.69 (incobotulinumtoxinA) RN - Hyperhidrosis Palmaris Et Plantaris SB - IM MH - Acetylcholine Release Inhibitors/administration & dosage/pharmacology/*therapeutic use MH - Adult MH - Antihypertensive Agents/therapeutic use MH - Botulinum Toxins, Type A/administration & dosage/pharmacology/*therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Female MH - Humans MH - Hyperhidrosis/complications/drug therapy MH - Hypertension/complications/drug therapy MH - Injections, Subcutaneous MH - Raynaud Disease/complications/*drug therapy MH - Vasoconstriction/drug effects/physiology EDAT- 2014/06/22 06:00 MHDA- 2015/02/03 06:00 CRDT- 2014/06/22 06:00 PHST- 2013/09/16 00:00 [received] PHST- 2014/01/09 00:00 [revised] PHST- 2014/03/03 00:00 [accepted] PHST- 2014/06/22 06:00 [entrez] PHST- 2014/06/22 06:00 [pubmed] PHST- 2015/02/03 06:00 [medline] AID - S0151-9638(14)00117-3 [pii] AID - 10.1016/j.annder.2014.03.003 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2014 Jun-Jul;141(6-7):462-3. doi: 10.1016/j.annder.2014.03.003. Epub 2014 Apr 8. PMID- 19543731 OWN - NLM STAT- MEDLINE DCOM- 20100802 LR - 20221207 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 30 IP - 7 DP - 2010 May TI - Discoid lupus erythematosus in a patient with scleroderma and hepatitis C virus infection. PG - 969-71 LID - 10.1007/s00296-009-1009-1 [doi] AB - A 49-year-old Japanese woman presented with discoid lupus erythematosus (DLE) on the face. The presence of Raynaud's phenomenon, swollen fingers, a high anti-nuclear antibody titer, and the results of a biopsy revealed limited-type systemic sclerosis (lSSc). The association of SSc with DLE is rare, although some single case reports have been published in Japan. Our patient was positive for hepatitis C virus infection. Racial predisposition and immune imbalance are proposed to have played a role in the development of these lesions in our case. FAU - Yamamoto, Mami AU - Yamamoto M AD - Department of Dermatology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan. yamamoto-tki@umin.ac.jp FAU - Yamamoto, Toshiyuki AU - Yamamoto T FAU - Tsuboi, Ryoji AU - Tsuboi R LA - eng PT - Case Reports PT - Journal Article DEP - 20090619 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/analysis/blood MH - Asian People MH - Biopsy MH - Ear/pathology/physiopathology MH - Female MH - Hand/innervation/pathology/physiopathology MH - Hepatitis C/*complications/*immunology/physiopathology MH - Humans MH - Immune System/physiopathology MH - Japan MH - Lip/immunology/pathology/physiopathology MH - Lupus Erythematosus, Discoid/ethnology/*immunology/physiopathology MH - Male MH - Middle Aged MH - Raynaud Disease/complications/immunology/physiopathology MH - Scleroderma, Systemic/*complications/*immunology/physiopathology EDAT- 2009/06/23 09:00 MHDA- 2010/08/03 06:00 CRDT- 2009/06/23 09:00 PHST- 2009/02/28 00:00 [received] PHST- 2009/06/02 00:00 [accepted] PHST- 2009/06/23 09:00 [entrez] PHST- 2009/06/23 09:00 [pubmed] PHST- 2010/08/03 06:00 [medline] AID - 10.1007/s00296-009-1009-1 [doi] PST - ppublish SO - Rheumatol Int. 2010 May;30(7):969-71. doi: 10.1007/s00296-009-1009-1. Epub 2009 Jun 19. PMID- 24850874 OWN - NLM STAT- MEDLINE DCOM- 20150123 LR - 20140918 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 53 IP - 10 DP - 2014 Oct TI - Novel photoplethysmography cardiovascular assessments in patients with Raynaud's phenomenon and systemic sclerosis: a pilot study. PG - 1855-63 LID - 10.1093/rheumatology/keu196 [doi] AB - OBJECTIVE: Multisite photoplethysmography (PPG) cardiovascular assessments can evaluate endothelial, peripheral autonomic and arterial dysfunction. The aim of this pilot study was to investigate the potential clinical utility of the technology in assessing patients with SSc and primary RP (PRP). METHODS: Multisite PPG pulse measurements, a reference ankle brachial pressure index (ABPI) and a full clinical assessment were undertaken for three subject groups: SSc, PRP and controls. Endothelial and autonomic function and arterial disease measures were obtained using pulse wave analysis. RESULTS: Nineteen SSc, 19 PRP and 23 control subjects were assessed and compared. Endothelial function was significantly impaired in SSc (P < 0.02), but with no difference between controls and PRP. Receiver operating characteristic-based classification accuracy was 81% (sensitivity 90%, specificity 74%) for separating SSc from controls and 82% (sensitivity 84%, specificity 79%) for separating SSc from PRP. SSc patients with digital ulcers had significantly lower endothelial function compared with those without ulcers (P < 0.05). Autonomic dysfunction was suggested in both SSc and PRP and was most exaggerated in patients with diffuse SSc. All groups had overall normal ABPI and arterial stiffness timing measures. Bilateral timing differences at the toes, which represents peripheral occlusive arterial disease, did show increased asymmetry in SSc (P < 0.02). CONCLUSION: Multisite PPG pulse technology showed potential diagnostic ability. By using measures of endothelial function, it differentiated SSc from control and PRP subjects with an accuracy of at least 81%. Objective pulse-derived measures of autonomic function and arterial disease in SSc have also been reported in this pilot study. CI - © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - McKay, Neil D AU - McKay ND AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. FAU - Di Maria, Costanzo AU - Di Maria C AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. FAU - Hedley, Stephen AU - Hedley S AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. FAU - Murray, Alan AU - Murray A AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. FAU - Allen, John AU - Allen J AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne, Lothian Rheumatic Diseases Unit, Western General Hospital, Edinburgh, Department of Microvascular Diagnostics, Freeman Hospital and Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK. john.allen@nuth.nhs.uk. LA - eng PT - Journal Article DEP - 20140521 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Aged MH - Ankle Brachial Index MH - Endothelium, Vascular/physiopathology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Photoplethysmography/*methods MH - Pilot Projects MH - Pulse Wave Analysis MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/*physiopathology MH - Sensitivity and Specificity OTO - NOTNLM OT - Raynaud’s phenomenon OT - cardiovascular disease OT - photoplethysmography OT - scleroderma OT - systemic sclerosis EDAT- 2014/05/23 06:00 MHDA- 2015/01/24 06:00 CRDT- 2014/05/23 06:00 PHST- 2014/05/23 06:00 [entrez] PHST- 2014/05/23 06:00 [pubmed] PHST- 2015/01/24 06:00 [medline] AID - keu196 [pii] AID - 10.1093/rheumatology/keu196 [doi] PST - ppublish SO - Rheumatology (Oxford). 2014 Oct;53(10):1855-63. doi: 10.1093/rheumatology/keu196. Epub 2014 May 21. PMID- 18372361 OWN - NLM STAT- MEDLINE DCOM- 20080812 LR - 20110311 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 17 IP - 3 DP - 2008 Mar TI - Severe digital ischemia-a presenting symptom of malignancy-associated antiphospholipid syndrome. PG - 206-9 LID - 10.1177/0961203307086235 [doi] AB - The association of the antiphospholipid syndrome with malignancy has been extensively reported. Raynaud's phenomenon has also been reported to be associated with various malignancies. In this report, we describe two patients who presented with severe digital ischemia mimicking Raynaud's phenomenon. The patients were found to have antiphospholipid syndrome, and upon extensive evaluation, a diagnosis of a malignancy was made. This report highlights the importance of malignancy workup in patients with severe digital ischemia associated with antiphospholipid syndrome. FAU - Grossman, A AU - Grossman A AD - Department of Internal Medicine E, Rabin Medical Center, Beilinson Campus, Petach Tikva Israel. Affiliated to Sackler Medical School, Tel Aviv University, Tel Aviv, Israel. alon2206@012.net.il FAU - Gafter-Gvili, A AU - Gafter-Gvili A FAU - Green, H AU - Green H FAU - Ben Aharon, I AU - Ben Aharon I FAU - Stemmer, S M AU - Stemmer SM FAU - Molad, Y AU - Molad Y FAU - Krause, I AU - Krause I LA - eng PT - Case Reports PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Aged MH - Antiphospholipid Syndrome/*etiology MH - Humans MH - Male MH - Middle Aged MH - Neoplasms/*complications MH - Raynaud Disease/*etiology EDAT- 2008/03/29 09:00 MHDA- 2008/08/13 09:00 CRDT- 2008/03/29 09:00 PHST- 2008/03/29 09:00 [pubmed] PHST- 2008/08/13 09:00 [medline] PHST- 2008/03/29 09:00 [entrez] AID - 17/3/206 [pii] AID - 10.1177/0961203307086235 [doi] PST - ppublish SO - Lupus. 2008 Mar;17(3):206-9. doi: 10.1177/0961203307086235. PMID- 20568411 OWN - NLM STAT- MEDLINE DCOM- 20100806 LR - 20140127 IS - 1426-9686 (Print) IS - 1426-9686 (Linking) VI - 28 IP - 167 DP - 2010 May TI - [Management of patients with systemic sclerosis]. PG - 421-3 AB - This paper presents progress in management of systemic sclerosis, with respect of EULAR recommendation. In the first part we present efficacy of overall immunosupresive treatments and preliminary data of autologous stem cell transplantation based on randomized clinical trials. The second part concerns a treatment specific for target organs: Raynaud's phenomenon, alimentary tract involvement, interstitial lung disease, pulmonary arterial hypertension and scleroderma renal crisis. Despite intensive investigations disease modifying drug therapy is still unknown, but specific organ treatment increase survival and improve health related quality of life. FAU - Wiesik-Szewczyk, Ewa AU - Wiesik-Szewczyk E AD - Instytut Reumatologii, Klinika i Poliklinika Układowych Chorób Tkanki Łacznej. ewa.w.szewczyk@gmail.com FAU - Olesińska, Marzena AU - Olesińska M LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Postepowanie z chorymi na twardzine układowa. PL - Poland TA - Pol Merkur Lekarski JT - Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego JID - 9705469 RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Bone Marrow Transplantation MH - Cyclophosphamide/therapeutic use MH - Humans MH - Hypertension, Pulmonary/drug therapy/etiology MH - Immunosuppressive Agents/therapeutic use MH - Kidney Diseases/etiology/therapy MH - Lung Diseases, Interstitial/etiology/therapy MH - Methotrexate/therapeutic use MH - Prognosis MH - Quality of Life MH - Raynaud Disease/etiology/therapy MH - Scleroderma, Systemic/complications/*therapy MH - Stem Cell Transplantation MH - Treatment Outcome RF - 21 EDAT- 2010/06/24 06:00 MHDA- 2010/08/07 06:00 CRDT- 2010/06/24 06:00 PHST- 2010/06/24 06:00 [entrez] PHST- 2010/06/24 06:00 [pubmed] PHST- 2010/08/07 06:00 [medline] PST - ppublish SO - Pol Merkur Lekarski. 2010 May;28(167):421-3. PMID- 21401971 OWN - NLM STAT- MEDLINE DCOM- 20160422 LR - 20250626 IS - 1752-8526 (Electronic) VI - 2011 DP - 2011 Mar 14 TI - Raynaud's phenomenon (primary). LID - 1119 [pii] AB - INTRODUCTION: Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor, followed by cyanosis and redness with pain, and sometimes paraesthesia. On rare occasions it can lead to ulceration of the fingers and toes (and in some cases of the ears or nose). This review focuses on primary (idiopathic) Raynaud's phenomenon, occurring in the absence of an underlying disease. The prevalence of primary Raynaud's phenomenon varies by sex, country, and exposure to workplace vibration. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments for primary Raynaud's phenomenon? We searched: Medline, Embase, The Cochrane Library, and other important databases up to May 2010 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found 16 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review we present information relating to the effectiveness and safety of the following interventions: amlodipine, diltiazem, exercise, inositol nicotinate, keeping warm, moxisylyte (thymoxamine), naftidrofuryl oxalate, nicardipine, nifedipine, prazosin, and smoking cessation. FAU - Pope, Janet Elizabeth AU - Pope JE AD - St Joseph's Health Care Rheumatology Centre, London, Ontario, Canada. LA - eng PT - Journal Article PT - Systematic Review DEP - 20110314 PL - England TA - BMJ Clin Evid JT - BMJ clinical evidence JID - 101294314 RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Administration, Oral MH - Humans MH - *Nifedipine/therapeutic use MH - Prevalence MH - *Raynaud Disease/drug therapy MH - Ulcer MH - Vibration PMC - PMC3275138 EDAT- 2011/03/16 06:00 MHDA- 2016/04/23 06:00 PMCR- 2013/03/14 CRDT- 2011/03/16 06:00 PHST- 2011/03/16 06:00 [entrez] PHST- 2011/03/16 06:00 [pubmed] PHST- 2016/04/23 06:00 [medline] PHST- 2013/03/14 00:00 [pmc-release] AID - 1119 [pii] PST - epublish SO - BMJ Clin Evid. 2011 Mar 14;2011:1119. PMID- 33914202 OWN - NLM STAT- MEDLINE DCOM- 20210928 LR - 20220406 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 40 IP - 10 DP - 2021 Oct TI - Clinical features and risk factors of Raynaud's phenomenon in primary Sjögren's syndrome. PG - 4081-4087 LID - 10.1007/s10067-021-05749-w [doi] AB - OBJECTIVE: The aim at the current study was to investigate the clinical characteristics and risk factors of Raynaud's phenomenon (RP) in patients with primary Sjögren's syndrome (pSS). METHODS: Retrospective analysis of the medical records of 333 new-onset pSS patients was performed. Demographic, clinical, and serological data were compared between individuals with and without RP. Logistic regression analysis was used to identify risk factors. RESULTS: RP was present in 11.41% of the pSS patients. pSS-RP patients were younger (49.74±14.56 years vs. 54.46±13.20 years, p=0.04) and exhibited higher disease activity (11 [5.75-15] vs. 7 [4-12], p=0.03) than those without. The prevalence of lung involvement was significantly higher in pSS patients with RP (60.53% vs. 17.29%; p<0.001). A significantly higher proportion of patients with pSS-RP tested positive about antinuclear (ANA), anti-RNP, and anti-centromere antibodies (ACA) compared to those without (p=0.003, <0.001, and 0.01, respectively). Multivariate analysis identified lung involvement (odds ratio [OR]=8.81, 95% confidence interval [CI] 2.02-38.47; p=0.04), anti-RNP positive status (OR=79.41, 95% CI 12.57-501.78; p<0.0001), as well as ACA (OR=13.17, 95% CI 2.60-66.72; p=0.002) as prognostic factors for pSS-RP. CONCLUSION: The presence of RP defined a subset of pSS with a unique phenotype, manifesting as increased lung involvement and a higher frequency of anti-RNP antibodies and ACA, as well as greater disease activity. These results suggest that RP has clinical and prognostic value of pSS patients. Further prospective studies with a larger number of subjects are warranted to confirm our findings and assess the prognostic and treatment implications of RP in pSS patients. Key Points • Raynaud's phenomenon (RP) was present in 38 (11.41%) of 333 patients with primary Sjögren's syndrome (pSS), with patients with RP exhibiting a younger age and higher disease activity. • The presence of RP indicates a subset of pSS with a unique phenotype, with manifestations including increased lung involvement and a higher frequency of anti-RNP antibodies and anti-centromere antibodies. • Patients with pSS and RP need close follow-up and long-term observation (including assessment of microangiopathy), with specific attention paid to the possible development of clinical features of systemic sclerosis. CI - © 2021. The Author(s). FAU - Lin, Wei AU - Lin W AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Xin, Zhifei AU - Xin Z AD - Department of Thoracic Surgery, Hebei General Hospital, Shijiazhuang, 050051, China. FAU - Ning, Xiaoran AU - Ning X AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. HBGHFSMYK@163.com. FAU - Li, Yang AU - Li Y AD - Department of Oncology, Hebei General Hospital, Shijiazhuang, 050051, China. FAU - Ren, Xiuying AU - Ren X AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Su, Yashuang AU - Su Y AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Liu, Meilu AU - Liu M AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Guo, Shaoying AU - Guo S AD - Department of Graduate School, Hebei Medical University, Shijiazhuang, 050017, China. FAU - Yang, Liu AU - Yang L AD - Department of Graduate School, Hebei Medical University, Shijiazhuang, 050017, China. FAU - Liu, Yixuan AU - Liu Y AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. FAU - Zhang, Fengxiao AU - Zhang F AD - Department of Rheumatology and Immunology, Hebei General Hospital, No. 348 Heping West Road, Shijiazhuang, 050051, Hebei, China. zfx9911@163.com. FAU - Zhang, Wen AU - Zhang W AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, 100730, China. LA - eng GR - No. 81801630/the National Nature Science Foundation of China/ GR - No. H2018307047/the National Nature Science Foundation of Hebei Province/ PT - Journal Article DEP - 20210429 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM CIN - Clin Rheumatol. 2022 Apr;41(4):1267-1268. doi: 10.1007/s10067-021-06043-5. PMID: 34984505 MH - Humans MH - Prospective Studies MH - *Raynaud Disease/complications/epidemiology MH - Retrospective Studies MH - Risk Factors MH - *Sjogren's Syndrome/complications/epidemiology PMC - PMC8463379 OTO - NOTNLM OT - Interstitial lung disease OT - Primary Sjögren’s syndrome OT - Raynaud’s phenomenon OT - Risk factors EDAT- 2021/04/30 06:00 MHDA- 2021/09/29 06:00 PMCR- 2021/04/29 CRDT- 2021/04/29 12:34 PHST- 2021/02/01 00:00 [received] PHST- 2021/04/21 00:00 [accepted] PHST- 2021/04/15 00:00 [revised] PHST- 2021/04/30 06:00 [pubmed] PHST- 2021/09/29 06:00 [medline] PHST- 2021/04/29 12:34 [entrez] PHST- 2021/04/29 00:00 [pmc-release] AID - 10.1007/s10067-021-05749-w [pii] AID - 5749 [pii] AID - 10.1007/s10067-021-05749-w [doi] PST - ppublish SO - Clin Rheumatol. 2021 Oct;40(10):4081-4087. doi: 10.1007/s10067-021-05749-w. Epub 2021 Apr 29. PMID- 32141306 OWN - NLM STAT- MEDLINE DCOM- 20201109 LR - 20220414 IS - 1477-0873 (Electronic) IS - 0269-2155 (Linking) VI - 34 IP - 5 DP - 2020 May TI - The complementary effects of galvanic current electrical stimulation associated with conservative treatment to increase vasodilation in patients with Raynaud's phenomenon: a randomized trial. PG - 595-606 LID - 10.1177/0269215520907652 [doi] AB - OBJECTIVE: To analyze the effectiveness of an electrotherapy intervention with galvanic current on symptoms associated with Raynaud's phenomenon. DESIGN: Single-blind randomized controlled trial, parallel design (1:1 ratio) and intention-to-treat analysis. SETTING: Virgen de las Nieves Hospital, Granada, Spain. SUBJECTS: Thirty-four participants with Raynaud's phenomenon, with a mean (SD) age of 43.43 (17.62) years. INTERVENTIONS: The patients were randomly assigned to a control group with conservative treatment (anti-inflammatory, vasodilatory and analgesic drugs) or an intervention group that received conservative treatment and vasodilatory electrical stimulation during seven weeks, three times/week for a total of 20 sessions. MAIN MEASURES: The primary outcome was the number of attacks. Secondary outcomes were pain, peripheral blow flow, oxygen saturation, upper limb disability, central sensitization, pain catastrophizing and temperature recovery. All outcomes were assessed at baseline, posttreatment and at two months of follow-up. RESULTS: The galvanic current electrotherapy group showed significantly greater improvements in the number of attacks (mean difference = 26.3, 95% confidence interval (CI) = 14.4 to 38.3), pre-cold stress pain (95% CI = 0.6 to 2.4), radial artery blood flow (95% CI = -7.8 ⩾ x ⩽ 1.3), ulnar artery blood flow (95% CI = -8.63 to 0.60), oxygen saturation (95% CI = -1.7 ⩾ x ⩽ -0.29), upper limb disability (95% CI = 1.1 to 22.3), central sensitization (95% CI = 6.7 to 18.2) and temperature recovery (95% CI = -5.7 ⩾ x ⩽ -0.32) than the conservative treatment group. CONCLUSION: This study suggests that a complementary treatment with galvanic current in combination to conservative approach is superior to conservative applied as isolate, in reducing the clinical manifestations and disability in Raynaud's phenomenon. FAU - Tapia-Haro, Rosa María AU - Tapia-Haro RM AUID- ORCID: 0000-0002-8151-2723 AD - Instituto de Investigación Biosanitaria ibs.GRANADA and Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), Granada, Spain. FAU - García-Ríos, Mª Carmen AU - García-Ríos MC AD - Instituto de Investigación Biosanitaria ibs.GRANADA and Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), Granada, Spain. FAU - Toledano-Moreno, Sonia AU - Toledano-Moreno S AD - Instituto de Investigación Biosanitaria ibs.GRANADA and Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), Granada, Spain. FAU - Casas-Barragán, Antonio AU - Casas-Barragán A AD - Instituto de Investigación Biosanitaria ibs.GRANADA and Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), Granada, Spain. FAU - Castro-Sánchez, Adelaida Mª AU - Castro-Sánchez AM AD - Department of Nursing, Physical Therapy and Medicine, University of Almeria, Almeria, Spain. FAU - Aguilar-Ferrándiz, María Encarnación AU - Aguilar-Ferrándiz ME AD - Instituto de Investigación Biosanitaria ibs.GRANADA and Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), Granada, Spain. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20200306 PL - England TA - Clin Rehabil JT - Clinical rehabilitation JID - 8802181 SB - IM MH - Adult MH - *Conservative Treatment MH - *Electric Stimulation Therapy MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/physiopathology/*therapy MH - Single-Blind Method MH - Spain MH - Treatment Outcome MH - Vasodilation OTO - NOTNLM OT - Raynaud disease OT - electric stimulation therapy OT - randomized controlled trial EDAT- 2020/03/07 06:00 MHDA- 2020/11/11 06:00 CRDT- 2020/03/07 06:00 PHST- 2020/03/07 06:00 [pubmed] PHST- 2020/11/11 06:00 [medline] PHST- 2020/03/07 06:00 [entrez] AID - 10.1177/0269215520907652 [doi] PST - ppublish SO - Clin Rehabil. 2020 May;34(5):595-606. doi: 10.1177/0269215520907652. Epub 2020 Mar 6. PMID- 26693634 OWN - NLM STAT- MEDLINE DCOM- 20161012 LR - 20230215 IS - 1952-4013 (Electronic) IS - 1167-1122 (Linking) VI - 25 IP - 5 DP - 2015 Sep-Oct TI - Digital ulcers in systemic sclerosis: role of flow-mediated dilatation and capillaroscopy as risk assessment tools. PG - 444-51 LID - 10.1684/ejd.2015.2605 [doi] AB - AIM: The aim of this study was to evaluate macrovascular endothelial dysfunction and microvascular damage as clinical markers of peripheral microangiopathy in patients with Raynaud's phenomenon (RP). PATIENTS AND METHODS: Seventy-seven secondary RP with systemic sclerosis, 32 primary RP and 34 healthy controls were included in our study. Secondary RP patients were divided into two subgroups: 39 with digital ulcers (DU) and 38 without digital ulcers (non-DU). RESULTS: Patients with DU had significantly lower flow-mediated dilatation values (5.34 ± 7.49%) compared to non-DU patients (16.21 ± 11.31%), primary RP (17.96 ± 12.78%) and controls (20.17 ± 8.86%), p<0.001, favouring macrovascular endothelium dysfunction. Regarding microvascular damage, the DU group had a predominately capillaroscopic late pattern (71.1%) whereas non-DU patients had an active pattern (56.4%). The microangiopathy evolution score was significantly higher in the DU group compared to the non-DU group (4.79 ± 1.82 vs. 1.79 ± 1.56, p<0.001). Flow-mediated dilation was significantly lower in late pattern (6.13 ± 7.09%) compared to active (12.58 ± 10.66%) and early patterns (17.72 ± 14.90%), p = 0.016 and p = 0.044 respectively. CONCLUSIONS: Low flow-mediated dilatation and microvascular damage in capillaroscopy are early clinical markers of DU risk in RP patients. FAU - Silva, Ivone AU - Silva I AD - Angiology, Vascular Surgery Service, Clinical Immunology Unit. FAU - Loureiro, Tiago AU - Loureiro T AD - Angiology and Vascular Surgery Service. FAU - Teixeira, Andreia AU - Teixeira A AD - Health Information and Decision Sciences Department, Universidade do Porto. FAU - Almeida, Isabel AU - Almeida I AD - Clinical Immunology Unit, Centro Hospitalar do Porto, Praça da Revista o Tripeiro, n° 42, hab 23, 4415-789 Porto, Portugal. FAU - Mansilha, Armando AU - Mansilha A AD - Unit of Angiology and Vascular Surgery; Faculty of Medicine of University of Porto. FAU - Vasconcelos, Carlos AU - Vasconcelos C AD - Clinical Immunology Unit, Centro Hospitalar do Porto, Praça da Revista o Tripeiro, n° 42, hab 23, 4415-789 Porto, Portugal. FAU - Almeida, Rui AU - Almeida R AD - Angiology and Vascular Surgery Service. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 SB - IM MH - Adult MH - Aged MH - Blood Flow Velocity MH - Case-Control Studies MH - Comorbidity MH - Female MH - Fingers MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/diagnosis/*epidemiology MH - Reference Values MH - Risk Assessment MH - Role MH - Scleroderma, Systemic/diagnosis/*epidemiology MH - Severity of Illness Index MH - Skin/*blood supply MH - Skin Ulcer/drug therapy/*physiopathology MH - Ultrasonography, Interventional MH - Vasodilation/physiology OTO - NOTNLM OT - Raynaud's phenomenon OT - capillaroscopy OT - digital ulcer OT - endothelial dysfunction OT - flow-mediated dilatation OT - systemic sclerosis EDAT- 2015/12/24 06:00 MHDA- 2016/10/13 06:00 CRDT- 2015/12/24 06:00 PHST- 2015/12/24 06:00 [entrez] PHST- 2015/12/24 06:00 [pubmed] PHST- 2016/10/13 06:00 [medline] AID - ejd.2015.2605 [pii] AID - 10.1684/ejd.2015.2605 [doi] PST - ppublish SO - Eur J Dermatol. 2015 Sep-Oct;25(5):444-51. doi: 10.1684/ejd.2015.2605. PMID- 25557659 OWN - NLM STAT- MEDLINE DCOM- 20151216 LR - 20220408 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 82 IP - 3 DP - 2015 May TI - Systemic sclerosis: Recent insights. PG - 148-53 LID - S1297-319X(14)00257-7 [pii] LID - 10.1016/j.jbspin.2014.10.010 [doi] AB - Systemic sclerosis is an orphan connective tissue disease characterized by alterations of the microvasculature, disturbances of the immune system and massive deposition of collagen and other matrix substances in the skin and internal organs. A major achievement of the recent years has been the validation of new classification criteria, allowing earlier diagnosis and earlier treatment of systemic sclerosis, before irreversible fibrosis and organ damage appeared ("window of opportunity"). Raynaud's phenomenon is usually the first sign of the disease and is considered as the main sentinel sign for the identification of very early systemic sclerosis. Systemic sclerosis is clinically heterogeneous and disease course remains unpredictable. Its prognosis depends on cardiopulmonary involvement and recent studies aim to identify serum or genetic biomarkers predictive of severe organ involvement. Moreover, the prospective follow-up of large cohorts has provided and will offer critical material to identify strong prognostic factors. Whereas the outcomes of vascular manifestations of the disease has been recently improved due to targeted therapy, recent data have highlighted that mortality has not changed over the past 40 years. This reflects the absence of efficacy of current available drugs to counteract the fibrotic process. Nevertheless, several targeted immunity therapies, commonly with proven efficacy in other immune diseases, are about to be investigated in systemic sclerosis. Indeed, promising results in small and open studies have been reported. This article deals with recent insights into classification criteria, pathogenesis, organ involvements, outcome and current and possible future therapeutic options in systemic sclerosis. CI - Copyright © 2014 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Elhai, Muriel AU - Elhai M AD - Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France. FAU - Avouac, Jérôme AU - Avouac J AD - Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France. FAU - Kahan, André AU - Kahan A AD - Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France. Electronic address: yannick.allanore@cch.aphp.fr. LA - eng PT - Journal Article PT - Review DEP - 20141231 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Antirheumatic Agents) SB - IM MH - Antirheumatic Agents/therapeutic use MH - Early Diagnosis MH - Fibrosis MH - Humans MH - Prognosis MH - Raynaud Disease/etiology MH - *Scleroderma, Systemic/classification/complications/physiopathology/therapy OTO - NOTNLM OT - Fibrosis OT - Pathogenesis OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Treatment EDAT- 2015/01/06 06:00 MHDA- 2015/12/19 06:00 CRDT- 2015/01/06 06:00 PHST- 2014/07/31 00:00 [received] PHST- 2014/10/29 00:00 [accepted] PHST- 2015/01/06 06:00 [entrez] PHST- 2015/01/06 06:00 [pubmed] PHST- 2015/12/19 06:00 [medline] AID - S1297-319X(14)00257-7 [pii] AID - 10.1016/j.jbspin.2014.10.010 [doi] PST - ppublish SO - Joint Bone Spine. 2015 May;82(3):148-53. doi: 10.1016/j.jbspin.2014.10.010. Epub 2014 Dec 31. PMID- 30414374 OWN - NLM STAT- MEDLINE DCOM- 20190508 LR - 20190508 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 43 IP - 3 DP - 2018 Jul-Sep TI - Iloprost infusion through elastomeric pump for the outpatient treatment of severe Raynaud's phenomenon and digital ulcers - a single centre experience. PG - 237-238 AB - Raynaud's phenomenon (RP) and digital ulcers (DU) are the main clinical features of vasculopathy that occurs in several systemic rheumatic diseases. Intravenous iloprost is recommended for the treatment of severe RP and DU in patients with systemic sclerosis and portable devices for iloprost infusion have been recently designed, allowing outpatient treatment. This new alternative for drug administration not only avoids absenteeism, with the patient having the opportunity to continue his own family and work life, but also reduces the costs associated with hospitalization. We describe our protocol and report our experience with 12 patients, for a total of 25 infusions, who have received domiciliary iloprost through elastomeric pump. Patients could easily manage the device and the treatment outcomes were promising, with all patients having DU healing, without any special safety concerns. FAU - Duarte, Ana Catarina AU - Duarte AC AD - Hospital Garcia de Orta. FAU - Barbosa, Lurdes AU - Barbosa L AD - Hospital Garcia de Orta. FAU - Santos, Maria José AU - Santos MJ AD - Hospital Garcia de Orta. FAU - Cordeiro, Ana AU - Cordeiro A AD - Hospital Garcia de Orta. LA - eng PT - Letter TT - Iloprost infusion through elastomeric pump for the outpatient treatment of severe Raynaud’s phenomenon and digital ulcers – a single centre experience. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 RN - 0 (Elastomers) RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Ambulatory Care MH - Elastomers MH - Equipment Design MH - Female MH - Fingers MH - Humans MH - Iloprost/*administration & dosage MH - Infusion Pumps MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy MH - Severity of Illness Index MH - Skin Ulcer/*drug therapy MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage EDAT- 2018/11/11 06:00 MHDA- 2019/05/09 06:00 CRDT- 2018/11/11 06:00 PHST- 2018/11/11 06:00 [entrez] PHST- 2018/11/11 06:00 [pubmed] PHST- 2019/05/09 06:00 [medline] AID - CE180207 [pii] PST - ppublish SO - Acta Reumatol Port. 2018 Jul-Sep;43(3):237-238. PMID- 18414458 OWN - NLM STAT- MEDLINE DCOM- 20080801 LR - 20131121 IS - 1745-8390 (Electronic) IS - 1745-8382 (Linking) VI - 4 IP - 6 DP - 2008 Jun TI - A case of undifferentiated connective tissue disease: is it a distinct clinical entity? PG - 328-32 LID - 10.1038/ncprheum0799 [doi] AB - BACKGROUND: In November 2001, a 24-year-old woman with thrombocytopenia and Raynaud's phenomenon presented to our clinic. Her physical examination was unremarkable except for bruising on her legs and arms. INVESTIGATIONS: Laboratory assays detected the presence of antinuclear and anti-ribonucleoprotein antibodies. Tests for antibodies to double-stranded DNA and for antiphospholipid (lupus anticoagulant and anticardiolipin), anticentromere, anti-Scl-70, and antiplatelet antibodies were negative, as was a Coombs test. An echocardiogram, chest X-ray, and abdominal scan showed no abnormalities. Nailfold digital capillaroscopy revealed minor capillary changes not specific for scleroderma. DIAGNOSIS: Undifferentiated connective tissue disease. MANAGEMENT: The patient was successfully treated initially with high doses of corticosteroids and azathioprine, followed by rapid dose tapering. Therapy was continued for 2 years and then stopped. Over the next 4 years the patient's disease history was unremarkable, apart from mild Raynaud's phenomenon of the hands and the presence antinuclear and anti-ribonucleoprotein antibodies. The diagnosis of undifferentiated connective tissue disease was confirmed at her most-recent assessment, in October 2007. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy. marta.mosca@int.med.unipi.it FAU - Tani, Chiara AU - Tani C FAU - Bombardieri, Stefano AU - Bombardieri S LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20080415 PL - United States TA - Nat Clin Pract Rheumatol JT - Nature clinical practice. Rheumatology JID - 101261802 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antimalarials) RN - 0 (Antirheumatic Agents) RN - MRK240IY2L (Azathioprine) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Antimalarials/therapeutic use MH - Antirheumatic Agents/therapeutic use MH - Azathioprine/therapeutic use MH - Diagnosis, Differential MH - Female MH - Humans MH - Mixed Connective Tissue Disease/complications/*diagnosis/drug therapy MH - Pregnancy MH - Pregnancy Complications/diagnosis/drug therapy MH - Raynaud Disease/complications/*diagnosis/immunology MH - Remission Induction MH - Thrombocytopenia/*complications/drug therapy/immunology RF - 21 EDAT- 2008/04/17 09:00 MHDA- 2008/08/02 09:00 CRDT- 2008/04/17 09:00 PHST- 2007/11/12 00:00 [received] PHST- 2008/03/06 00:00 [accepted] PHST- 2008/04/17 09:00 [pubmed] PHST- 2008/08/02 09:00 [medline] PHST- 2008/04/17 09:00 [entrez] AID - ncprheum0799 [pii] AID - 10.1038/ncprheum0799 [doi] PST - ppublish SO - Nat Clin Pract Rheumatol. 2008 Jun;4(6):328-32. doi: 10.1038/ncprheum0799. Epub 2008 Apr 15. PMID- 41874407 OWN - NLM STAT- MEDLINE DCOM- 20260428 LR - 20260429 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 10 IP - 1 DP - 2026 Jan 6 TI - Neurofascialvascular training in systemic sclerosis: Clinical outcomes, proposed rationale, and home-programme feasibility. LID - rxag030 [pii] LID - 10.1093/mrcr/rxag030 [doi] AB - BACKGROUND: Secondary Raynaud's phenomenon in limited cutaneous systemic sclerosis is driven by structural microvascular disease and is frequently accompanied by hand oedema, stiffness, and functional disability. Evidence for pragmatic physiotherapy approaches addressing both vascular symptoms and soft-tissue involvement remains limited. CASE: A 66-year-old man with limited cutaneous systemic sclerosis (diagnosed in 2009) and long-standing triphasic Raynaud's symptoms reported cold-induced digital pain, recurrent fissures with prior ulceration, puffy hands, reduced finger range of motion, and impaired fine motor function despite stable therapy, including intravenous prostacyclin every 2 weeks. Outcomes at baseline (November 2024), post-intervention (April 2025), and follow-up (December 2025) included the Raynaud's Condition Score, QuickDASH, ScleroID, COMPASS-31, and HAMIS. INTERVENTION: The patient completed a structured neurofascialvascular training programme comprising 24 supervised 30-minute sessions (November 2024 to April 2025) with home practice on non-clinic days, followed by a home-only programme (30 minutes/day) through follow-up; adherence was monitored with an exercise log. OUTCOMES: Symptom burden and hand function improved from baseline to post-intervention and were largely maintained at follow-up, with shorter typical Raynaud's episodes and better hand mobility and disability scores. About 5% of prescribed home sessions were missed. Infrared thermography and clinical photographs were used descriptively to show acute pre-post session thermal changes and longitudinal reductions in puffiness and colour changes; follow-up images were obtained in December (cold season). CONCLUSION: This case suggests that a neurofascialvascular training programme was feasible and was associated with sustained improvement in Raynaud-related burden and hand function, warranting prospective controlled evaluation. CI - © Japan College of Rheumatology 2026. Published by Oxford University Press. FAU - Bertacchini, Paolo AU - Bertacchini P AUID- ORCID: 0009-0005-1577-7140 AD - Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 SB - IM MH - Humans MH - Male MH - Aged MH - *Scleroderma, Systemic/complications/therapy/rehabilitation MH - *Raynaud Disease/etiology/therapy/rehabilitation/physiopathology MH - Feasibility Studies MH - Treatment Outcome MH - *Exercise Therapy/methods OTO - NOTNLM OT - Autonomic nervous system OT - case reports OT - fascia OT - microcirculation OT - scleroderma, systemic EDAT- 2026/03/24 12:37 MHDA- 2026/04/28 17:38 CRDT- 2026/03/24 11:03 PHST- 2026/01/21 00:00 [received] PHST- 2026/02/28 00:00 [revised] PHST- 2026/03/12 00:00 [accepted] PHST- 2026/04/28 17:38 [medline] PHST- 2026/03/24 12:37 [pubmed] PHST- 2026/03/24 11:03 [entrez] AID - 8539693 [pii] AID - 10.1093/mrcr/rxag030 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2026 Jan 6;10(1):rxag030. doi: 10.1093/mrcr/rxag030. PMID- 19037602 OWN - NLM STAT- MEDLINE DCOM- 20090819 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 4 DP - 2009 Feb TI - New lines in therapy of Raynaud's phenomenon. PG - 355-63 LID - 10.1007/s00296-008-0792-4 [doi] AB - Current knowledge about the pathogenesis of Raynaud's phenomenon (RP) results in novel approaches for therapy. Vasospasm without endothelial damage is thought to be the main cause for primary RP. The pathogenesis of secondary forms of RP is supposed to be initiated primary by endothelial damage. The aim of the review is to present main groups of medications as well as non-pharmacological regimen, that are used for the treatment of RP. The necessity of immediate assessment and treatment in severe forms of the disease with digital ulcers is highlighted. The mild forms of primary RP can be controlled by non-pharmacologic approaches. If the effect is insufficient, medications of first choice are calcium channel blockers. In the severe forms of the disorder, intravenous infusion of prostacyclin as well as endothelin-1 receptor antagonists and specific inhibitors of phosphodiesterase-5 are the treatment of choice. Treatment in the future may include selective blockers of alpha-2c adrenergic receptors, inhibitors of protein tyrosine kinase and Rho-kinase, as well as calcitonin gene-related peptide. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria. sevdalina_n@abv.bg FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Journal Article PT - Review DEP - 20081127 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Endothelin A Receptor Antagonists) RN - 0 (Endothelin-1) RN - 0 (Receptor, Endothelin A) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - *Endothelin A Receptor Antagonists MH - Endothelin-1/*therapeutic use MH - Epoprostenol/*therapeutic use MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Receptor, Endothelin A/*therapeutic use MH - Treatment Outcome RF - 81 EDAT- 2008/11/28 09:00 MHDA- 2009/08/20 09:00 CRDT- 2008/11/28 09:00 PHST- 2008/07/06 00:00 [received] PHST- 2008/09/07 00:00 [accepted] PHST- 2008/11/28 09:00 [pubmed] PHST- 2009/08/20 09:00 [medline] PHST- 2008/11/28 09:00 [entrez] AID - 10.1007/s00296-008-0792-4 [doi] PST - ppublish SO - Rheumatol Int. 2009 Feb;29(4):355-63. doi: 10.1007/s00296-008-0792-4. Epub 2008 Nov 27. PMID- 22994661 OWN - NLM STAT- MEDLINE DCOM- 20130301 LR - 20220311 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 51 IP - 10 DP - 2012 Oct TI - Caring for new mothers: diagnosis, management and treatment of nipple dermatitis in breastfeeding mothers. PG - 1149-61 LID - 10.1111/j.1365-4632.2011.05445.x [doi] AB - Breastfeeding is thought to be the most optimal form of infant nutrition. Nursing mothers are generally advised to continue breastfeeding until the infant is two years of age or beyond. Unfortunately, however, a majority of nursing mothers will discontinue breastfeeding much earlier than recommended. The most common reason for early discontinuation of breastfeeding is nipple pain. It is, therefore, essential that dermatologists know how to appropriately diagnose and effectively treat nipple pain associated with nipple dermatitis among nursing mothers. This review article provides a detailed discussion on the clinical features and management of various causes of nipple dermatitis during lactation, including problems with infant latch-on, congenital oral anomalies, plugged lactiferous ducts, atopic dermatitis, irritant contact dermatitis, allergic contact dermatitis, yeast infections, bacterial infections, herpes simplex virus, and Raynaud's phenomenon of the nipple. CI - © 2012 The International Society of Dermatology. FAU - Heller, Misha M AU - Heller MM AD - Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. FAU - Fullerton-Stone, Honor AU - Fullerton-Stone H FAU - Murase, Jenny E AU - Murase JE LA - eng PT - Journal Article PT - Review PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 SB - IM MH - Breast Feeding/*adverse effects MH - Dermatitis, Atopic/complications/*diagnosis/microbiology MH - Dermatitis, Contact/complications/*diagnosis/microbiology MH - Dermatomycoses/complications/diagnosis MH - Female MH - Herpes Simplex/complications/diagnosis MH - Humans MH - Infant, Newborn MH - Mammary Glands, Human/physiopathology MH - *Mothers MH - *Nipples MH - Pain/etiology MH - Psoriasis/diagnosis MH - Raynaud Disease/complications/diagnosis/microbiology MH - Skin Diseases, Bacterial/complications/diagnosis MH - Sucking Behavior/*physiology EDAT- 2012/09/22 06:00 MHDA- 2013/03/02 06:00 CRDT- 2012/09/22 06:00 PHST- 2012/09/22 06:00 [entrez] PHST- 2012/09/22 06:00 [pubmed] PHST- 2013/03/02 06:00 [medline] AID - 10.1111/j.1365-4632.2011.05445.x [doi] PST - ppublish SO - Int J Dermatol. 2012 Oct;51(10):1149-61. doi: 10.1111/j.1365-4632.2011.05445.x. PMID- 26210126 OWN - NLM STAT- MEDLINE DCOM- 20160509 LR - 20220309 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 41 IP - 3 DP - 2015 Aug TI - Management of Systemic Sclerosis-Related Skin Disease: A Review of Existing and Experimental Therapeutic Approaches. PG - 399-417 LID - S0889-857X(15)00025-3 [pii] LID - 10.1016/j.rdc.2015.04.004 [doi] AB - The skin is the most common organ system involved in patients with systemic sclerosis (SSc). Nearly all patients experience cutaneous symptoms, including sclerosis, Raynaud's phenomenon, digital ulcers, telangiectasias, and calcinosis. In addition to posing functional challenges, cutaneous symptoms are often a major cause of pain, psychological distress, and body image dissatisfaction. The present article reviews the main features of SSc-related cutaneous manifestations and highlights an evidence-based treatment approach for treating each manifestation. This article also describes novel treatment approaches and opportunities for further research in managing this important clinical dimension of SSc. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Volkmann, Elizabeth R AU - Volkmann ER AD - Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, 1000 Veteran Avenue, Suite 32-59, Los Angeles, CA 90095, USA. Electronic address: Evolkmann@mednet.ucla.edu. FAU - Furst, Daniel E AU - Furst DE AD - Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, 1000 Veteran Avenue, Suite 32-59, Los Angeles, CA 90095, USA. LA - eng PT - Journal Article PT - Review DEP - 20150520 PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 RN - 8N3DW7272P (Cyclophosphamide) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Animals MH - Cyclophosphamide/therapeutic use MH - Diagnosis, Differential MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - Methotrexate/therapeutic use MH - Raynaud Disease/*therapy MH - Scleroderma, Systemic/diagnosis/pathology/*therapy MH - Treatment Failure OTO - NOTNLM OT - Cyclophosphamide (CYC) OT - Diffuse cutaneous systemic sclerosis (DcSSc) OT - Digital ulcers (DU) OT - Hematopoietic stem cell transplantation (HSCT) OT - Limited cutaneous systemic sclerosis (LcSSc) OT - Methotrexate (MTX) OT - Modified Rodnan skin score (mRSS) OT - Raynaud’s phenomenon (RP) EDAT- 2015/07/27 06:00 MHDA- 2016/05/10 06:00 CRDT- 2015/07/27 06:00 PHST- 2015/07/27 06:00 [entrez] PHST- 2015/07/27 06:00 [pubmed] PHST- 2016/05/10 06:00 [medline] AID - S0889-857X(15)00025-3 [pii] AID - 10.1016/j.rdc.2015.04.004 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2015 Aug;41(3):399-417. doi: 10.1016/j.rdc.2015.04.004. Epub 2015 May 20. PMID- 30684190 OWN - NLM STAT- MEDLINE DCOM- 20190617 LR - 20200225 IS - 1573-742X (Electronic) IS - 0929-5305 (Print) IS - 0929-5305 (Linking) VI - 47 IP - 2 DP - 2019 Feb TI - Unfavourably altered plasma clot properties in patients with primary Raynaud's phenomenon: association with venous thromboembolism. PG - 248-254 LID - 10.1007/s11239-019-01805-0 [doi] AB - Associations of Raynaud's phenomenon (RP) with venous thromboembolism (VTE) are unclear. We investigated the occurrence of RP together with prothrombotic state markers and fibrin clot properties in VTE patients. In this prospective cohort study we enrolled 360 patients free of known autoimmune disease. D-dimer, von Willebrand factor (vWF), plasma clot permeability (K(s)), clot lysis time (CLT) along with fibrinolysis activators and inhibitors were determined at least 3 months since the VTE event. The presence/absence of RP was diagnosed at least 6 months before VTE. Primary RP occurred in 57 subjects (17%) with a 3.6-fold higher prevalence among women. Patients with RP had 11% higher fibrinogen, 16% higher vWF, 5% lower K(s), and 10% longer CLT (all p < 0.05). Females with RP (21%) had 6.6% lower K(s), 11.2% longer CLT, and 18.5% higher vWF (all p < 0.05) compared with men. CLT was predicted by PAI-1 and vWF levels. Regression analysis showed that RP was a predictor of prolonged CLT in the whole patient group (OR 3.46, 95% CI 1.92-6.24) and in women following VTE (OR 2.75, 95% CI 1.31-5.78). Primary RP patients tend to form denser plasma fibrin clots displaying impaired lysability and increased endothelial damage. RP might be a novel risk factor for VTE, especially in women. FAU - Żuk, Joanna AU - Żuk J AD - Second Department of Internal Medicine, Jagiellonian University Medical College, Krakow, Poland. FAU - Snarska-Drygalska, Agnieszka AU - Snarska-Drygalska A AD - Specialist Dermatological Clinic, Krakow, Poland. FAU - Malinowski, Krzysztof Piotr AU - Malinowski KP AD - Faculty of Health Science, Institute of Public Health, Jagiellonian University Medical College, Krakow, Poland. FAU - Papuga-Szela, Elżbieta AU - Papuga-Szela E AD - John Paul II Hospital, Krakow, Poland. FAU - Natorska, Joanna AU - Natorska J AUID- ORCID: 0000-0003-3176-8007 AD - John Paul II Hospital, Krakow, Poland. AD - Institute of Cardiology, Jagiellonian University Medical College, 80 Pradnicka St., 31-202, Krakow, Poland. FAU - Undas, Anetta AU - Undas A AD - John Paul II Hospital, Krakow, Poland. mmundas@cyf-kr.edu.pl. AD - Institute of Cardiology, Jagiellonian University Medical College, 80 Pradnicka St., 31-202, Krakow, Poland. mmundas@cyf-kr.edu.pl. AD - Faculty of Medicine and Health Sciences, Jan Kochanowski University, Kielce, Poland. mmundas@cyf-kr.edu.pl. LA - eng GR - UMO-2013/09/B/NZ5/00254/Narodowe Centrum Nauki/ PT - Journal Article PL - Netherlands TA - J Thromb Thrombolysis JT - Journal of thrombosis and thrombolysis JID - 9502018 RN - 0 (Biomarkers) RN - 0 (Fibrin Fibrinogen Degradation Products) RN - 0 (fibrin fragment D) RN - 0 (von Willebrand Factor) RN - 9001-31-4 (Fibrin) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Biomarkers/blood MH - Cross-Sectional Studies MH - Female MH - Fibrin/analysis MH - Fibrin Fibrinogen Degradation Products/analysis MH - *Fibrinolysis MH - Humans MH - Male MH - Middle Aged MH - Poland/epidemiology MH - Predictive Value of Tests MH - Prevalence MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/*blood/diagnosis/epidemiology MH - Risk Factors MH - Sex Factors MH - Venous Thromboembolism/*blood/diagnosis/epidemiology MH - Young Adult MH - von Willebrand Factor/analysis PMC - PMC6394442 OTO - NOTNLM OT - Fibrin OT - Fibrinolysis OT - Raynaud phenomenon OT - Venous thromboembolism COIS- CONFLICT OF INTEREST: All the authors declare that they have no conflict of interest. ETHICAL APPROVAL: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. INFORMED CONSENT: Informed consent was obtained from all individual participants included in the study. EDAT- 2019/01/27 06:00 MHDA- 2019/06/18 06:00 PMCR- 2019/01/25 CRDT- 2019/01/27 06:00 PHST- 2019/01/27 06:00 [pubmed] PHST- 2019/06/18 06:00 [medline] PHST- 2019/01/27 06:00 [entrez] PHST- 2019/01/25 00:00 [pmc-release] AID - 10.1007/s11239-019-01805-0 [pii] AID - 1805 [pii] AID - 10.1007/s11239-019-01805-0 [doi] PST - ppublish SO - J Thromb Thrombolysis. 2019 Feb;47(2):248-254. doi: 10.1007/s11239-019-01805-0. PMID- 21327676 OWN - NLM STAT- MEDLINE DCOM- 20110726 LR - 20211020 IS - 1613-7671 (Electronic) IS - 0043-5325 (Linking) VI - 123 IP - 3-4 DP - 2011 Feb TI - Auricular electroacupuncture reduces frequency and severity of Raynaud attacks. PG - 112-6 LID - 10.1007/S00508-011-1531-5 [doi] AB - BACKGROUND: Acupuncture has been shown to influence skin perfusion and the subjective cold perception threshold. Therefore, we hypothesized that auricular electroacupuncture (EA) might reduce symptoms in primary Raynaud's phenomenon (PRP). METHODS: Twenty-six patients with PRP received 6 cycles of auricular EA. After 3, 6 and 24 weeks attack frequency and severity were reevaluated using standardized questionnaires and a visual analogue scale (VAS). Skin temperature was assessed by infrared thermography and laser Doppler perfusion imaging was used to determine skin perfusion. RESULTS: Compared to baseline we found a significant reduction of attack frequency after 3 (p = 0.001) and 6 weeks (p < 0.001) of auricular EA. This improvement sustained following cessation of EA, after 24 weeks (p < 0.001). Furthermore, attack associated pain was reduced after 3 (p = 0.003), 6 (p = 0.003) and 24 weeks (p = 0.001) of treatment, while skin temperature and skin perfusion did not change significantly throughout the study period. CONCLUSIONS: Auricular EA reduces symptoms by means of frequency and severity of attacks in PRP but has no influence on skin perfusion and skin temperature. FAU - Schlager, Oliver AU - Schlager O AD - Division of Angiology, Department of Internal Medicine II, Vienna Medical University, Vienna General Hospital, Vienna, Austria. FAU - Gschwandtner, Michael E AU - Gschwandtner ME FAU - Mlekusch, Irene AU - Mlekusch I FAU - Herberg, Karin AU - Herberg K FAU - Frohner, Tanja AU - Frohner T FAU - Schillinger, Martin AU - Schillinger M FAU - Koppensteiner, Renate AU - Koppensteiner R FAU - Mlekusch, Wolfgang AU - Mlekusch W LA - eng PT - Journal Article DEP - 20110217 PL - Austria TA - Wien Klin Wochenschr JT - Wiener klinische Wochenschrift JID - 21620870R SB - IM MH - Acupuncture, Ear/*statistics & numerical data MH - Adult MH - Austria/epidemiology MH - Electroacupuncture/*statistics & numerical data MH - Female MH - Humans MH - Incidence MH - Male MH - Raynaud Disease/diagnosis/*epidemiology/*prevention & control MH - Risk Assessment MH - Risk Factors MH - Treatment Outcome EDAT- 2011/02/18 06:00 MHDA- 2011/07/27 06:00 CRDT- 2011/02/18 06:00 PHST- 2010/12/04 00:00 [received] PHST- 2011/01/14 00:00 [accepted] PHST- 2011/02/18 06:00 [entrez] PHST- 2011/02/18 06:00 [pubmed] PHST- 2011/07/27 06:00 [medline] AID - 10.1007/S00508-011-1531-5 [doi] PST - ppublish SO - Wien Klin Wochenschr. 2011 Feb;123(3-4):112-6. doi: 10.1007/S00508-011-1531-5. Epub 2011 Feb 17. PMID- 34510957 OWN - NLM STAT- MEDLINE DCOM- 20210914 LR - 20210917 IS - 1473-2300 (Electronic) IS - 0300-0605 (Print) IS - 0300-0605 (Linking) VI - 49 IP - 9 DP - 2021 Sep TI - Cerebral infarction caused by systemic sclerosis: a case report. PG - 3000605211044045 LID - 10.1177/03000605211044045 [doi] LID - 03000605211044045 AB - Systemic sclerosis, also known as scleroderma, is a rare multisystem autoimmune disease characterized by vascular lesions caused by collagen deposition in the skin and viscera and damage to the endothelium. Endothelial injury and microvascular occlusion result in Raynaud's phenomenon, finger ischemia, pulmonary hypertension, and scleroderma renal crisis. Scleroderma itself is a rare disease with an incidence ranging from 0.1 to 14 per 100,000 people in the general population. Cerebral involvement is not considered a common manifestation of systemic sclerosis, although studies have shown that the brain can be involved. Therefore, to deepen the understanding of this disease, we herein report a case of cerebral infarction associated with systemic sclerosis. FAU - Wang, Qingqing AU - Wang Q AUID- ORCID: 0000-0001-7298-1811 AD - The Affiliated Fuyang Hospital of Bengbu Medical College, Fuyang City, Anhui Province, China. AD - Department of Neurology, Fuyang People's Hospital, Fuyang City, Anhui Province, China. FAU - Zhang, Mengen AU - Zhang M AD - Department of Neurology, Fuyang People's Hospital, Fuyang City, Anhui Province, China. FAU - Zhai, Mingfeng AU - Zhai M AD - Department of Neurology, Fuyang People's Hospital, Fuyang City, Anhui Province, China. FAU - Li, Zongyou AU - Li Z AD - The Affiliated Fuyang Hospital of Bengbu Medical College, Fuyang City, Anhui Province, China. AD - Department of Neurology, Fuyang People's Hospital, Fuyang City, Anhui Province, China. LA - eng PT - Case Reports PT - Journal Article PL - England TA - J Int Med Res JT - The Journal of international medical research JID - 0346411 SB - IM MH - Cerebral Infarction/diagnostic imaging/etiology MH - Humans MH - *Hypertension, Pulmonary MH - *Raynaud Disease MH - *Scleroderma, Systemic/complications MH - Skin PMC - PMC8442488 OTO - NOTNLM OT - Cerebral infarction OT - Raynaud’s phenomenon OT - anti-nuclear antibody OT - case report OT - computed tomography OT - magnetic resonance imaging OT - systemic sclerosis COIS- Declaration of competing interests: The authors declare that there are no conflicts of interest. EDAT- 2021/09/14 06:00 MHDA- 2021/09/15 06:00 PMCR- 2021/09/12 CRDT- 2021/09/13 05:34 PHST- 2021/09/13 05:34 [entrez] PHST- 2021/09/14 06:00 [pubmed] PHST- 2021/09/15 06:00 [medline] PHST- 2021/09/12 00:00 [pmc-release] AID - 10.1177_03000605211044045 [pii] AID - 10.1177/03000605211044045 [doi] PST - ppublish SO - J Int Med Res. 2021 Sep;49(9):3000605211044045. doi: 10.1177/03000605211044045. PMID- 17359558 OWN - NLM STAT- MEDLINE DCOM- 20070921 LR - 20131121 IS - 1748-5460 (Electronic) IS - 0022-2151 (Linking) VI - 121 IP - 6 DP - 2007 Jun TI - Iloprost-induced sudden hearing loss. PG - 609-10 AB - We report a patient who developed sudden, bilateral, sensorineural hearing loss during therapeutic use of iloprost for Raynaud's phenomenon. The sudden hearing loss was attributed to iloprost use and completely reversed in eight days with conservative therapy. Iloprost may be a potentially ototoxic drug, causing sudden hearing loss. FAU - Dursun, E AU - Dursun E AD - Department of Otorhinolaryngology, GATA Haydarpasa Training Hospital, Istanbul, Turkey. drengindursun@yahoo.com FAU - Dogru, S AU - Dogru S FAU - Cincik, H AU - Cincik H FAU - Cekin, E AU - Cekin E FAU - Gungor, A AU - Gungor A FAU - Poyrazoglu, E AU - Poyrazoglu E LA - eng PT - Case Reports PT - Journal Article DEP - 20070315 PL - England TA - J Laryngol Otol JT - The Journal of laryngology and otology JID - 8706896 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Hearing Loss, Bilateral/*chemically induced MH - Hearing Tests MH - Humans MH - Iloprost/*adverse effects MH - Male MH - Raynaud Disease/drug therapy MH - Tinnitus/chemically induced MH - Vasodilator Agents/*adverse effects EDAT- 2007/03/16 09:00 MHDA- 2007/09/22 09:00 CRDT- 2007/03/16 09:00 PHST- 2006/12/12 00:00 [accepted] PHST- 2007/03/16 09:00 [pubmed] PHST- 2007/09/22 09:00 [medline] PHST- 2007/03/16 09:00 [entrez] AID - S0022215107006858 [pii] AID - 10.1017/S0022215107006858 [doi] PST - ppublish SO - J Laryngol Otol. 2007 Jun;121(6):609-10. doi: 10.1017/S0022215107006858. Epub 2007 Mar 15. PMID- 41197064 OWN - NLM STAT- MEDLINE DCOM- 20260213 LR - 20260213 IS - 2379-3961 (Electronic) IS - 1098-1861 (Linking) VI - 124 IP - 4 DP - 2025 TI - Unilateral, Episodic, Transient Blanching of Nipple With Pain in a Male: A Case Report. PG - 398-401 AB - INTRODUCTION: Causes of nipple pain include trauma, vasospasm, malignancy, Paget's disease, inflammation secondary to blockage of the ducts, infection, and medications. Raynaud's phenomenon (RP) of the nipple is reported more often in women and typically presents bilaterally. CASE PRESENTATION: A 63-year-old man presented with episodic, stabbing pain and blanching of the left nipple, worsening over 3 years. Symptoms began insidiously during military service. Examination revealed no abnormalities, but blanching was observed during a painful episode. Laboratory and imaging studies were unremarkable. Conservative measures and pharmacologic agents, including nitroglycerin ointment and amlodipine, provided minimal relief. Intercostal nerve blocks and cervical sympathetic blocks did not provide lasting relief. Ultrasound-guided paravertebral sympathetic block with local anesthetic provided temporary relief lasting week. Botulinum toxin injection around the areola resulted in sustained pain relief lasting more than month. DISCUSSION: The patient's presentation is consistent with primary RP of the nipple, a rare and underrecognized condition. While it typically affects distal extremities, it can involve other areas, including the nipple. Diagnosis is clinical, often requiring documentation during episodes. Management is challenging and largely anecdotal. Botulinum toxin has shown promise in RP, though evidence remains mixed. CONCLUSIONS: This case highlights a rare presentation of unilateral, vasospastic nipple pain resembling primary RP, with partial response to botulinum toxin injection. CI - Copyright© Board of Regents of the University of Wisconsin System and The Medical College of Wisconsin, Inc. FAU - Rodriguez, Marisol AU - Rodriguez M AD - Department of Anesthesiology, Medical College of Wisconsin, Milwaukee, Wisconsin. FAU - Shankar, Hariharan AU - Shankar H AUID- ORCID: 0000-0002-1567-3887 AD - Department of Anesthesiology, Medical College of Wisconsin, Milwaukee, Wisconsin, hshankar@mcw.edu. AD - Clement Zablocki VA Medical Center, Milwaukee, Wisconsin. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - WMJ JT - WMJ : official publication of the State Medical Society of Wisconsin JID - 9716054 RN - 0 (Sympatholytics) RN - 0 (Calcium Channel Blockers) SB - IM MH - Humans MH - Male MH - Middle Aged MH - *Nipples MH - *Pain/drug therapy/etiology MH - *Raynaud Disease/complications/diagnosis/drug therapy MH - Nerve Block MH - Sympatholytics/therapeutic use MH - Calcium Channel Blockers/therapeutic use EDAT- 2025/11/06 18:27 MHDA- 2025/11/06 18:28 CRDT- 2025/11/06 14:52 PHST- 2025/11/06 18:28 [medline] PHST- 2025/11/06 18:27 [pubmed] PHST- 2025/11/06 14:52 [entrez] PST - ppublish SO - WMJ. 2025;124(4):398-401. PMID- 20359139 OWN - NLM STAT- MEDLINE DCOM- 20100503 LR - 20100402 IS - 0025-7818 (Print) IS - 0025-7818 (Linking) VI - 100 IP - 6 DP - 2009 Nov-Dec TI - [Hand-arm vibration syndrome in a nurse carrying out gypsum cutting operations]. PG - 471-5 AB - BACKGROUND: A professional nurse, employed mainly on gypsum cutting operations, developed a hand-arm vibration syndrome with Raynaud's phenomenon, neurosensitive disorders and impairment of the bone and joints apparatus of the hand and arm. METHODS: The nurse underwent diagnostic investigations (cold test, X-ray of the upper limbs, blood tests); also the vibration levels transmitted from instrument were measured and the exposure times were established. RESULTS AND CONCLUSIONS: Clinical investigations showed the presence of a hand-arm vibration syndrome with secondary Raynaud's phenomenon and environmental surveys revealed very high vibration levels, such as could be associated with the disease with a causal relationship. In the literature no reports exist of the vibration syndrome being associated with health care workers in orthopaedic departments. The case described in this study occurred because of peculiar organisational factors that most likely have never occurred in other hospitals or orthopaedic departments. FAU - Speziale, Martina AU - Speziale M AD - Azienda Sanitaria 10 di Firenze, U.F. TAV & G.O., Firenze. martina.speziale@asf.toscana.it FAU - Picchiotti, E AU - Picchiotti E LA - ita PT - Case Reports PT - English Abstract PT - Journal Article TT - Sindrome da vibrazioni mano-braccio in un infermiere addetto all'attività di gipsotomia. PL - Italy TA - Med Lav JT - La Medicina del lavoro JID - 0401176 SB - IM MH - Arm Injuries/diagnosis/*etiology MH - *Casts, Surgical MH - Hand Injuries/diagnosis/*etiology MH - Humans MH - Hypesthesia/diagnosis/*etiology MH - Male MH - Middle Aged MH - Occupational Diseases/*etiology MH - *Orthopedic Nursing/instrumentation/organization & administration MH - Paresthesia/diagnosis/*etiology MH - Raynaud Disease/diagnosis/*etiology MH - Vibration/*adverse effects EDAT- 2010/04/03 06:00 MHDA- 2010/05/04 06:00 CRDT- 2010/04/03 06:00 PHST- 2010/04/03 06:00 [entrez] PHST- 2010/04/03 06:00 [pubmed] PHST- 2010/05/04 06:00 [medline] PST - ppublish SO - Med Lav. 2009 Nov-Dec;100(6):471-5. PMID- 16564187 OWN - NLM STAT- MEDLINE DCOM- 20060928 LR - 20220410 IS - 1078-5884 (Print) IS - 1078-5884 (Linking) VI - 32 IP - 2 DP - 2006 Aug TI - Thoracoscopic sympathectomy for Raynaud's phenomenon--a long term follow-up study. PG - 198-202 AB - OBJECTIVES: To assess the long term results of thoracoscopic sympathectomy for Raynaud's phenomenon. DESIGN, MATERIALS AND METHODS: A retrospective study of 34 consecutive patients who were treated for Raynaud's phenomenon by thoracoscopic sympathectomy from 1996 to 2005. Eight patients presented with ulcerations of the digits and 10 had severe ischaemia without ulcerations. The hospital records were retrieved and questionnaires were mailed to the patients for follow-up. RESULTS: The questionnaire was answered by 91% of patients after a median follow-up time of 40 months. An immediate effect was seen in 83% of the patients but symptoms recurred in 60% during the follow-up period. Compensatory sweating occurred in 63 and 30% reported gustatory sweating. Thirteen patients (43%) regretted having the operation. CONCLUSION: The majority of patients with Raynaud's phenomenon have an excellent immediate effect from thoracoscopic sympathectomy and one third achieve a long lasting effect. Side effects are frequent. We now only use thoracoscopic sympathectomy in severe cases of Raynaud's phenomenon. FAU - Thune, T H AU - Thune TH AD - Department of Cardiothoracic and Vascular Surgery, Odense University Hospital, Odense, Denmark. tom@thune.nu FAU - Ladegaard, L AU - Ladegaard L FAU - Licht, P B AU - Licht PB LA - eng PT - Journal Article DEP - 20060327 PL - England TA - Eur J Vasc Endovasc Surg JT - European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery JID - 9512728 SB - IM CIN - Eur J Vasc Endovasc Surg. 2007 Jan;33(1):133. doi: 10.1016/j.ejvs.2006.09.020. PMID: 17097319 MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Patient Satisfaction MH - Raynaud Disease/*surgery MH - Recurrence MH - Retrospective Studies MH - Surveys and Questionnaires MH - Sweating MH - Sympathectomy/adverse effects/*methods MH - *Thoracoscopy EDAT- 2006/03/28 09:00 MHDA- 2006/09/29 09:00 CRDT- 2006/03/28 09:00 PHST- 2005/11/15 00:00 [received] PHST- 2006/01/21 00:00 [accepted] PHST- 2006/03/28 09:00 [pubmed] PHST- 2006/09/29 09:00 [medline] PHST- 2006/03/28 09:00 [entrez] AID - S1078-5884(06)00090-6 [pii] AID - 10.1016/j.ejvs.2006.01.017 [doi] PST - ppublish SO - Eur J Vasc Endovasc Surg. 2006 Aug;32(2):198-202. doi: 10.1016/j.ejvs.2006.01.017. Epub 2006 Mar 27. PMID- 26116156 OWN - NLM STAT- MEDLINE DCOM- 20160121 LR - 20260128 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 54 IP - 11 DP - 2015 Nov TI - Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis. PG - 2015-24 LID - 10.1093/rheumatology/kev201 [doi] AB - OBJECTIVE: Digital vasculopathy (comprising RP, digital ulceration and critical digital ischaemia) is responsible for much of the pain and disability experienced by patients with SSc. However, there is a limited evidence base to guide clinicians in the management of SSc-related digital vasculopathy. Our aim was to produce recommendations that would be helpful for clinicians, especially for those managing patients outside specialist centres. METHODS: The UK Scleroderma Study Group set up several working groups to develop a number of consensus best practice pathways for the management of SSc-specific complications, including digital vasculopathy. RESULTS: This overview presents the background and best practice consensus pathways for SSc-related RP, digital ulceration and critical ischaemia. Examples of drug therapies, including doses, are suggested in order to inform prescribing practice. CONCLUSION: A number of treatment algorithms are provided that are intended to provide the clinician with accessible reference tools for use in daily management. CI - © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre, Manchester, michael.hughes-6@postgrad.manchester.ac.uk. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Anderson, Marina E AU - Anderson ME AD - Institute of Ageing and Chronic Disease, Faculty of Health & Life Sciences, University of Liverpool. FAU - Hall, Frances AU - Hall F AD - Department of Rheumatology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. FAU - Moinzadeh, Pia AU - Moinzadeh P AD - Department for Dermatology and Venereology, University of Cologne, Cologne, Germany. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne and. FAU - Baildam, Eileen AU - Baildam E AD - Department of Paediatric Rheumatology, Alder Hey Children's Foundation NHS Trust, Liverpool, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre, Manchester. LA - eng PT - Consensus Statement PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20150626 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Nat Rev Rheumatol. 2015 Oct;11(10):569-71. doi: 10.1038/nrrheum.2015.111. PMID: 26260763 MH - Algorithms MH - Disease Management MH - Fingers/*blood supply/pathology MH - Humans MH - Ischemia/etiology/therapy MH - *Practice Patterns, Physicians' MH - Raynaud Disease/etiology/therapy MH - Scleroderma, Systemic/*complications MH - Ulcer/etiology/therapy MH - United Kingdom MH - Vascular Diseases/*etiology/*therapy OTO - NOTNLM OT - Raynaud’s phenomenon OT - critical digital ischaemia OT - digital ulcer OT - digital vasculopathy OT - scleroderma OT - systemic sclerosis EDAT- 2015/06/28 06:00 MHDA- 2016/01/23 06:00 CRDT- 2015/06/28 06:00 PHST- 2014/10/22 00:00 [received] PHST- 2015/06/28 06:00 [entrez] PHST- 2015/06/28 06:00 [pubmed] PHST- 2016/01/23 06:00 [medline] AID - kev201 [pii] AID - 10.1093/rheumatology/kev201 [doi] PST - ppublish SO - Rheumatology (Oxford). 2015 Nov;54(11):2015-24. doi: 10.1093/rheumatology/kev201. Epub 2015 Jun 26. PMID- 40890234 OWN - NLM STAT- MEDLINE DCOM- 20250901 LR - 20251019 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 15 IP - 1 DP - 2025 Sep 1 TI - A comprehensive study on drug-related Raynaud's phenomenon based on the FDA adverse event reporting system. PG - 32059 LID - 10.1038/s41598-025-17182-z [doi] LID - 32059 AB - Raynaud's phenomenon (RP) is often an overlooked adverse event, mainly secondary RP, where drug induction or exacerbation is a controllable and preventable factor. This study aimed to systematically evaluate the association between drugs and RP using the FDA adverse event reporting system (FAERS) database. Utilizing disproportionality analysis, we quantified the risk of RP-associated drugs based on large-scale FAERS case data. Drugs were categorized by ATC classification, and descriptive analyses were conducted to assess population characteristics and reporting patterns. Secondary analyses and Weibull shape parameter testing were performed to verify the reliability of the results and the temporal patterns of adverse event onset. A total of 562 drugs and 4303 cases were linked to RP. Significant signals were identified across 8 ATC categories, predominantly nervous system agents (21 drugs, n = 632), cardiovascular drugs (20 drugs, n = 403), and immunomodulators (14 drugs, n = 185). Pharmacologically, CGRP inhibitors, triptans, non-selective β-blockers, interferons, and antineoplastic/immunomodulatory agents demonstrated high mechanistic relevance to RP. Indications such as rheumatoid arthritis (256 cases, 5.95%), narcolepsy (149 cases, 3.46%), and attention-deficit hyperactivity disorder (117 cases, 2.72%) were frequently associated with RP. Cases were concentrated in individuals aged 25-59, with a female predominance (ratio 3.13:1). This study systematically reports drug-RP associations. Clinicians should consider RP risk when prescribing CGRP inhibitors, triptans, non-selective β-blockers, interferons, or antineoplastic/immunomodulatory agents. Middle-aged and elderly females, particularly those with rheumatoid arthritis, warrant heightened vigilance for drug-induced RP. CI - © 2025. The Author(s). FAU - Zheng, Pingping AU - Zheng P AD - First School of Clinical Medicine, Fujian University of Traditional Chinese Medicine, Fuzhou, China. FAU - Zheng, Xiulian AU - Zheng X AD - College of Rehabilitation Medicine, Fujian University of Traditional Chinese Medicine, Fuzhou, 350122, China. FAU - Chen, Qun AU - Chen Q AD - The School of Adult Education, Fujian University of Traditional Chinese Medicine, Fuzhou, China. FAU - Wang, Dongmei AU - Wang D AD - College of Rehabilitation Medicine, Fujian University of Traditional Chinese Medicine, Fuzhou, 350122, China. FAU - Huang, Longzhuan AU - Huang L AD - Key Specialty of Clinical Pharmacy, The First Affiliated Hospital of Guangdong Pharmaceutical University, Guangzhou, China. FAU - Lin, Jianping AU - Lin J AD - School of Health, Fujian Medical University, Fuzhou, 350122, China. ljp1985@fjmu.edu.cn. FAU - Chen, Shaoqing AU - Chen S AD - College of Rehabilitation Medicine, Fujian University of Traditional Chinese Medicine, Fuzhou, 350122, China. chensq@fjtcm.edu.cn. LA - eng GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ GR - NO. X2021003/the Special Financial Grant Project of Fujian University of Traditional Chinese Medicine/ PT - Journal Article DEP - 20250901 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Humans MH - *Raynaud Disease/chemically induced/epidemiology MH - United States/epidemiology MH - Female MH - *Adverse Drug Reaction Reporting Systems/statistics & numerical data MH - United States Food and Drug Administration MH - Male MH - Middle Aged MH - Adult MH - Aged MH - *Drug-Related Side Effects and Adverse Reactions/epidemiology MH - Child MH - Adolescent MH - Young Adult MH - Databases, Factual MH - Child, Preschool PMC - PMC12402058 OTO - NOTNLM OT - Disproportionality analysis OT - Drug safety OT - Drugs OT - FAERS database OT - Raynaud’s phenomenon COIS- Declarations. Competing interests: The authors declare no competing interests. EDAT- 2025/09/02 00:44 MHDA- 2025/09/03 02:18 PMCR- 2025/09/01 CRDT- 2025/09/01 23:25 PHST- 2025/05/08 00:00 [received] PHST- 2025/08/21 00:00 [accepted] PHST- 2025/09/03 02:18 [medline] PHST- 2025/09/02 00:44 [pubmed] PHST- 2025/09/01 23:25 [entrez] PHST- 2025/09/01 00:00 [pmc-release] AID - 10.1038/s41598-025-17182-z [pii] AID - 17182 [pii] AID - 10.1038/s41598-025-17182-z [doi] PST - epublish SO - Sci Rep. 2025 Sep 1;15(1):32059. doi: 10.1038/s41598-025-17182-z. PMID- 28940121 OWN - NLM STAT- MEDLINE DCOM- 20180827 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 3 DP - 2018 Mar TI - Anti-CENP-B and anti-TOPO-1-containing sera from systemic sclerosis-related diseases with Raynaud's phenomenon induce vascular endothelial cell senescence not via classical p53-p21 pathway. PG - 749-756 LID - 10.1007/s10067-017-3845-9 [doi] AB - Raynaud's phenomenon (RP) is the earliest and most common clinical manifestation in patients with systemic sclerosis (SSc) and its related diseases containing anti-TOPO-1 and/or anti-CENP-B autoantibodies in the sera. However, the cause-effect relationship between the two autoantibodies and RP remains elucidation. Sera containing anti-CENP-B and anti-TOPO-1 autoantibodies were obtained from SSc-related diseases manifesting RP. The polyclonal auto-antibodies were purified from pooled sera by affinity chromatography. Mouse monoclonal anti-CENP-B and anti-TOPO-1 were purchased. Calf pulmonary arterial endothelial cells (CPAE) were incubated with 40% patient sera, purified polyclonal antibodies or mouse monoclonal antibodies for 1-6 days. The vascular endothelial biomarkers von Willebrand factor (vWF), thrombomodulin (CD141) and 6-keto-prostaglandin F1α (6-keto-PGF1α), cell viability marker ATP, and cell necrosis/lysis marker LDH in the culture supernatants were measured by ELISA. The cell senescence biomarker β-galactosidase and telomere content in the cells were stained by the respective kit. The classical p53-p21 senescence pathway was detected by Western blot. We found that 40% anti-CENP-B or anti-TOPO-1-containing sera without heat-inactivation and mouse monoclonal antibodies suppressed 6-keto-PGF1α production, increased β-galactosidase, and decreased relative telomere content. The cell senescence effects were proved not via p53-p21 pathway. The pathognomonic anti-CENP-B and anti-TOPO-1 autoantibodies in SSc-related diseases accelerate vascular endothelial cell senescence and functional impairment inducing RP. The real signaling pathway for autoantibody-induced cell senescence remains exploration. FAU - Shen, Chieh-Yu AU - Shen CY AD - Institue of Clinical Medicine, National Taiwan University College of Medicine, Taipei, 100, Taiwan. AD - Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, #7 Chung-Shan South Road, Taipei, 100, Taiwan. FAU - Li, Ko-Jen AU - Li KJ AD - Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, #7 Chung-Shan South Road, Taipei, 100, Taiwan. FAU - Lai, Pei-Hsuan AU - Lai PH AD - Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, #7 Chung-Shan South Road, Taipei, 100, Taiwan. FAU - Yu, Chia-Li AU - Yu CL AD - Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, #7 Chung-Shan South Road, Taipei, 100, Taiwan. FAU - Hsieh, Song-Chou AU - Hsieh SC AD - Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, #7 Chung-Shan South Road, Taipei, 100, Taiwan. hsiehsc@ntu.edu.tw. LA - eng PT - Journal Article DEP - 20170923 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Centromere Protein B) RN - 0 (Thrombomodulin) RN - 0 (von Willebrand Factor) RN - 58962-34-8 (6-Ketoprostaglandin F1 alpha) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - 6-Ketoprostaglandin F1 alpha/metabolism MH - Animals MH - Autoantibodies/*blood MH - Cattle MH - Cell Line MH - Cellular Senescence/*immunology MH - Centromere Protein B/*immunology MH - DNA Topoisomerases, Type I/*immunology MH - Endothelial Cells/immunology/metabolism MH - Endothelium, Vascular/immunology/metabolism MH - Humans MH - Raynaud Disease/blood/*immunology MH - Scleroderma, Systemic/blood/*immunology MH - Signal Transduction/*immunology MH - Thrombomodulin/metabolism MH - von Willebrand Factor/metabolism OTO - NOTNLM OT - Anti-CENP-B OT - Anti-TOPO-1 OT - Cell senescence OT - Raynaud’s phenomenon OT - Telomere content OT - p53-p21 pathway OT - β-galactosidase EDAT- 2017/09/25 06:00 MHDA- 2018/08/28 06:00 CRDT- 2017/09/24 06:00 PHST- 2017/04/02 00:00 [received] PHST- 2017/09/13 00:00 [accepted] PHST- 2017/08/28 00:00 [revised] PHST- 2017/09/25 06:00 [pubmed] PHST- 2018/08/28 06:00 [medline] PHST- 2017/09/24 06:00 [entrez] AID - 10.1007/s10067-017-3845-9 [pii] AID - 10.1007/s10067-017-3845-9 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Mar;37(3):749-756. doi: 10.1007/s10067-017-3845-9. Epub 2017 Sep 23. PMID- 29390130 OWN - NLM STAT- MEDLINE DCOM- 20180507 LR - 20181202 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 57 IP - 5 DP - 2018 May 1 TI - Comment on: Reproducibility of the scleroderma pattern assessed by wide-field capillaroscopy in subjects suffering from Raynaud's phenomenon: reply. PG - 941-942 LID - 10.1093/rheumatology/kex483 [doi] FAU - Constans, Joël AU - Constans J AD - Service de médecine Vasculaire, Hôpital St André, Bordeaux. FAU - Blaise, Sophie AU - Blaise S AD - Service de médecine Vasculaire, CHU Grenoble-Alpes, La Tronche. FAU - Lazareth, Isabelle AU - Lazareth I AD - Service de médecine Vasculaire, Hôpital Saint-Joseph, Paris. FAU - Le Hello, Claire AU - Le Hello C AD - Service de médecine Vasculaire, CHU de St Etienne, Hôpital Nord, Saint-Etienne. FAU - Pistorius, Marc-Antoine AU - Pistorius MA AD - Service de médecine Vasculaire, Hôtel Dieu, Nantes. FAU - Imbert, Bernard AU - Imbert B AD - Service de médecine Vasculaire, CHU Grenoble-Alpes, La Tronche. FAU - Mangin, Marion AU - Mangin M AD - Service de médecine Vasculaire, Hôpital St André, Bordeaux. FAU - Sintes, Pierre AU - Sintes P AD - Médecine Vasculaire, Saint-Cloud. FAU - Senet, Patricia AU - Senet P AD - Service de dermatologie, Médecine Vasculaire et Allergologie, Hôpital Tenon. FAU - Decamps-Le Chevoir, Joelle AU - Decamps-Le Chevoir J AD - Service de médecine interne, Hôpital de la Pitié, Paris, France. FAU - Tribout, Laurent AU - Tribout L AD - Service de dermatologie, Médecine Vasculaire et Allergologie, Hôpital Tenon. FAU - Carpentier, Patrick AU - Carpentier P AD - Service de médecine Vasculaire, CHU Grenoble-Alpes, La Tronche. FAU - Boulon, Carine AU - Boulon C AD - Service de médecine Vasculaire, Hôpital St André, Bordeaux. LA - eng PT - Comment PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2017 Oct 1;56(10):1780-1783. doi: 10.1093/rheumatology/kex282. PMID: 28957564 CON - Rheumatology (Oxford). 2018 May 1;57(5):940-941. doi: 10.1093/rheumatology/kex482. PMID: 29390117 MH - Humans MH - *Microscopic Angioscopy MH - Nails MH - *Raynaud Disease MH - Reproducibility of Results MH - Scleroderma, Localized MH - Scleroderma, Systemic EDAT- 2018/02/02 06:00 MHDA- 2018/05/08 06:00 CRDT- 2018/02/02 06:00 PHST- 2017/10/18 00:00 [received] PHST- 2017/11/08 00:00 [accepted] PHST- 2018/02/02 06:00 [pubmed] PHST- 2018/05/08 06:00 [medline] PHST- 2018/02/02 06:00 [entrez] AID - 4829712 [pii] AID - 10.1093/rheumatology/kex483 [doi] PST - ppublish SO - Rheumatology (Oxford). 2018 May 1;57(5):941-942. doi: 10.1093/rheumatology/kex483. PMID- 25262916 OWN - NLM STAT- MEDLINE DCOM- 20150825 LR - 20260518 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 97 DP - 2015 Jan TI - Choroidal impairment and macular thinning in patients with systemic sclerosis: the acute study. PG - 31-6 LID - S0026-2862(14)00128-9 [pii] LID - 10.1016/j.mvr.2014.08.008 [doi] AB - Raynaud's phenomenon (RP) is a reversible vasospastic response of the extremities to cold or emotion, and can be the first manifestation or may be present before the development of an overt systemic sclerosis (SSc). The disturbance of the balance between vasodilation and vasoconstriction is not limited to the peripheral microcirculation of the skin, but it is also observed in other organs, such as the choroidal plexus of the eye. We aimed to examine the choroidal thickness (CT), the macular thickness and ganglion cell complex (GCC) average in thirty consecutive patients, without visual symptoms, classified as primary RP (pRP), RP secondary to suspected SSc, and overt SSc. All the patients underwent rheumatologic and ophthalmologic examination, capillaroscopy, test for anti-nuclear antibodies, anti-dsDNA, and anti-extractable nuclear antigens. Ophthalmologic examination included: best corrected visual acuity; slit lamp biomicroscopy; intraocular pressure measurements, fundus examination, and Spectral Domain-Optical Coherence Tomography (SD-OCT) with enhanced depth imaging scan system. Twenty-seven healthy subjects similar for gender and age were analyzed. In pRP, CT was significantly thinner than controls in the outer nasal and temporal regions. In secondary RP, the inner and outer nasal areas were significantly thinner than controls. In SSc, the central, inner inferior, inner nasal, inner superior, outer temporal, outer inferior, and outer nasal regions were significantly thinner than controls. A decreasing trend of central foveal thickness was noted. All the patients had GCC average significantly lower than controls. A thinning of choroidal and macular thickness, as well as of GCC was observed in patients with pRP and becomes more severe and extensive in RP secondary to suspected SSc and overt SSc. To our knowledge, this is the first study to analyze the choroidal features using SD-OCT in RP. These data may be clinically useful in suggesting an early involvement of ocular microcirculation with significant reduction of choroidal perfusion. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, Istituto Gaetano Pini, Department of Clinical Sciences & Community Health, University of Milano, Piazza cardinal Ferrari 1, Milano, Italy. Electronic address: francesca.ingegnoli@unimi.it. FAU - Gualtierotti, Roberta AU - Gualtierotti R AD - Division of Rheumatology, Istituto Gaetano Pini, Department of Clinical Sciences & Community Health, University of Milano, Piazza cardinal Ferrari 1, Milano, Italy. Electronic address: roberta.gualtierotti@unimi.it. FAU - Pierro, Luisa AU - Pierro L AD - Department of Ophthalmology, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: pierro.luisa@hsr.it. FAU - Del Turco, Claudia AU - Del Turco C AD - Department of Ophthalmology, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: c.delturco@studenti.unisr.it. FAU - Miserocchi, Elisabetta AU - Miserocchi E AD - Ocular immunology and Uveitis Service, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: miserocchi.elisabetta@hsr.it. FAU - Schioppo, Tommaso AU - Schioppo T AD - Division of Rheumatology, Istituto Gaetano Pini, Department of Clinical Sciences & Community Health, University of Milano, Piazza cardinal Ferrari 1, Milano, Italy. Electronic address: invidendus@gmail.com. FAU - Meroni, Pier Luigi AU - Meroni PL AD - Division of Rheumatology, Istituto Gaetano Pini, Department of Clinical Sciences & Community Health, University of Milano, Piazza cardinal Ferrari 1, Milano, Italy. Electronic address: pierluigi.meroni@unimi.it. CN - ACUTE study group CN - ACUTE study group LA - eng PT - Comparative Study PT - Journal Article DEP - 20140926 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Biomarkers) SB - IM MH - Biomarkers/blood MH - Case-Control Studies MH - Choroid/*pathology/physiopathology MH - Choroid Diseases/diagnosis/*etiology/physiopathology MH - Female MH - Humans MH - Intraocular Pressure MH - Macula Lutea/*pathology/physiopathology MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/*etiology/physiopathology MH - Retinal Diseases/diagnosis/*etiology/physiopathology MH - Retinal Ganglion Cells/pathology MH - Scleroderma, Systemic/*complications/diagnosis/physiopathology MH - Severity of Illness Index MH - Slit Lamp MH - Tomography, Optical Coherence MH - Visual Acuity OTO - NOTNLM OT - Choroid OT - Connective tissue disease OT - Eye OT - Macula OT - Microcirculation OT - Raynaud's phenomenon OT - SD-OCT OT - Screening OT - Systemic sclerosis FIR - Gagliardi, Marco IR - Gagliardi M IRAD- Department of Ophthalmology, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: marco.gagliardi@studenti.unisr.it. FIR - Modorati, Giulio IR - Modorati G IRAD- Ocular immunology and Uveitis Service, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: modorati.giulio@hsr.it. FIR - Querques, Giuseppe IR - Querques G IRAD- Department of Ophthalmology, San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, Milano, Italy. Electronic address: giuseppe.querques@hsr.it. EDAT- 2014/09/30 06:00 MHDA- 2015/08/26 06:00 CRDT- 2014/09/30 06:00 PHST- 2014/04/28 00:00 [received] PHST- 2014/08/23 00:00 [revised] PHST- 2014/08/23 00:00 [accepted] PHST- 2014/09/30 06:00 [entrez] PHST- 2014/09/30 06:00 [pubmed] PHST- 2015/08/26 06:00 [medline] AID - S0026-2862(14)00128-9 [pii] AID - 10.1016/j.mvr.2014.08.008 [doi] PST - ppublish SO - Microvasc Res. 2015 Jan;97:31-6. doi: 10.1016/j.mvr.2014.08.008. Epub 2014 Sep 26. PMID- 39034217 OWN - NLM STAT- MEDLINE DCOM- 20240926 LR - 20250430 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 38 IP - 3 DP - 2024 Sep TI - Capillaroscopy in the daily clinic of the pediatric rheumatologist. PG - 101978 LID - S1521-6942(24)00049-4 [pii] LID - 10.1016/j.berh.2024.101978 [doi] AB - In the last decade, nailfold capillaroscopy is finding its way to the daily clinic of (pediatric) rheumatologist. This review will provide the necessary knowledge for the clinician performing this easy and non-invasive examination in children. In the first part, background information on type of capillaroscopy device and standardized (internationally validated) interpretations for the different capillary variables compared to healthy pediatric controls will be provided. The second part focusses on capillary changes that are observed in Raynaud's phenomenon with follow-up recommendations. This part will also cover capillaroscopy findings in juvenile systemic sclerosis, childhood-onset systemic lupus erythematosus, juvenile dermatomyositis and -mixed connective tissue disease, as well as correlations with disease severity. Lastly, a research agenda shows the current gaps we have in knowledge in this niche of nailfold capillaroscopy in pediatric connective tissue diseases. CI - Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Schonenberg-Meinema, D AU - Schonenberg-Meinema D AD - Department of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, the Netherlands. Electronic address: d.schonenberg@amsterdamumc.nl. FAU - Cutolo, M AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Polyclinic San Martino Hospital, Genova, Italy. FAU - Smith, V AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Belgium; Faculty of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. LA - eng PT - Journal Article PT - Review DEP - 20240720 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Child MH - *Raynaud Disease/diagnosis/physiopathology/diagnostic imaging MH - *Dermatomyositis/diagnostic imaging/diagnosis/physiopathology MH - *Lupus Erythematosus, Systemic/diagnostic imaging MH - *Scleroderma, Systemic/diagnostic imaging/physiopathology MH - Capillaries/diagnostic imaging MH - Rheumatology/methods MH - Rheumatologists MH - Nails/blood supply/diagnostic imaging MH - Mixed Connective Tissue Disease/diagnostic imaging MH - Scleroderma, Localized OTO - NOTNLM OT - Capillaroscopy OT - Childhood-onset systemic lupus erythematosus OT - JDM OT - Juvenile systemic sclerosis OT - Raynaud COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/07/22 00:41 MHDA- 2024/09/27 00:42 CRDT- 2024/07/21 21:57 PHST- 2024/03/25 00:00 [received] PHST- 2024/05/02 00:00 [revised] PHST- 2024/07/17 00:00 [accepted] PHST- 2024/09/27 00:42 [medline] PHST- 2024/07/22 00:41 [pubmed] PHST- 2024/07/21 21:57 [entrez] AID - S1521-6942(24)00049-4 [pii] AID - 10.1016/j.berh.2024.101978 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2024 Sep;38(3):101978. doi: 10.1016/j.berh.2024.101978. Epub 2024 Jul 20. PMID- 41812319 OWN - NLM STAT- MEDLINE DCOM- 20260522 LR - 20260522 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 78 DP - 2026 Jun TI - Dermatoscopy limitation and the critical role of capillaroscopy in the evaluation of systemic sclerosis and Raynaud's phenomenon among African American Veterans. PG - 152961 LID - S0049-0172(26)00050-8 [pii] LID - 10.1016/j.semarthrit.2026.152961 [doi] AB - INTRODUCTION: Nailfold capillaroscopy (NFC) is a crucial tool for assessing diagnostic microvascular abnormalities in patients with Raynaud's phenomenon (RP). While dermatoscopy is a widely accepted, cost-effective screening method for systemic sclerosis (SSc), its utility depends on optimal visibility for accurate interpretation. The objective of this study was to determine if skin pigmentation affects the interpretability of nailfold evaluation. METHODS: In an Institutional Review Board-approved study at the TVHS Hospital, patients undergoing evaluation for CTD-related RP were enrolled. Images were captured using two devices: a 200X dermatoscope (smartphone focus) and a 250X Smart G-Scope (USB automatic focus). Two capillaroscopy experts independently classified each image for clarity and the overall pattern (scleroderma or non-scleroderma). Skin pigmentation was assessed using the Fitzpatrick classification (I-VI). RESULTS: A total of 38 United States (US) Veterans with RP were assessed; 28% (n = 11) were self-identified as African American. Dermatoscopy analysis yielded blurry images in 63.6% (7/11) of patients with Fitzpatrick skin tones IV, V and VI, compared to 37% (10/31) in skin tones I, II, and III. The USB-capillaroscope improved interpretability across the entire cohort, especially in skin tones IV, V and VI. Notably, a Scleroderma (SD)-pattern was detected by the USB-capillaroscope in 4 of the 5 studies that had been uninterpretable with the dermatoscope. CONCLUSIONS: In conclusion, dermatoscopy resulted in a high rate of uninterpretable examinations in patients with RP, particularly among African American Veterans. The USB-capillaroscope device significantly improved interpretability, underscoring that relying solely on dermatoscopy as a screening tool may lead to underdiagnosis of SSc. CI - Copyright © 2026 The Author(s). Published by Elsevier Inc. All rights reserved. FAU - Maldonado, Genessis AU - Maldonado G AD - Department of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA. Electronic address: genessis.c.maldonado@vumc.org. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academinc Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address: mcutolo@unige.it. FAU - Frech, Tracy AU - Frech T AD - Department of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville Veteran Affairs, Nashville, TN, USA. Electronic address: tracy.frech@vumc.org. FAU - Radic, Mislav AU - Radic M AD - Department of Internal Medicine, Division of Rheumatology, Allergology and Clinical Immunology, Center of Excellence for Systemic Sclerosis in Croatia, University Hospital of Split, Split, 21000, Croatia; Internal Medicine Department, School of Medicine, University of Split, Split, 21000, Croatia. Electronic address: mislavradic@gmail.com. FAU - Snow, Marcus AU - Snow M AD - Department of Internal Medicine, Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE, USA. Electronic address: msnow@unmc.edu. FAU - Smith, Vanessa AU - Smith V AD - Ghent University Hospital, Department of Rheumatology, Guent University, Department of Internal Medicine and Unit for Molecular Immunology and Inflammation, VIB Research Center (IRC), Guent, Belgium. Electronic address: vanessa.smith@ugent.be. LA - eng PT - Journal Article DEP - 20260305 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Microscopic Angioscopy MH - *Scleroderma, Systemic/diagnosis/diagnostic imaging MH - *Raynaud Disease/diagnosis/diagnostic imaging MH - Black or African American MH - Male MH - Middle Aged MH - Female MH - *Dermoscopy MH - Veterans MH - Aged MH - Nails/blood supply MH - Adult MH - Skin Pigmentation MH - United States MH - White OTO - NOTNLM OT - African American OT - Dermatoscopy OT - Nailfold capillaroscopy OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Veterans COIS- Declaration of competing interest Co-author TF is supported by VA Merit Award I01CX002111, but no known competing financial interests or personal relationship that could have appeared to influence the work reported in this paper. Co-author VS is a Senior Clinical Investigator of the Research Foundation – Flanders (Belgium) (FWO) grant number 1802,920 N and 1802,925 N. The other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/03/12 01:09 MHDA- 2026/05/23 05:38 CRDT- 2026/03/11 19:04 PHST- 2025/11/16 00:00 [received] PHST- 2026/02/06 00:00 [revised] PHST- 2026/02/25 00:00 [accepted] PHST- 2026/05/23 05:38 [medline] PHST- 2026/03/12 01:09 [pubmed] PHST- 2026/03/11 19:04 [entrez] AID - S0049-0172(26)00050-8 [pii] AID - 10.1016/j.semarthrit.2026.152961 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2026 Jun;78:152961. doi: 10.1016/j.semarthrit.2026.152961. Epub 2026 Mar 5. PMID- 21704799 OWN - NLM STAT- MEDLINE DCOM- 20110829 LR - 20250626 IS - 1532-821X (Electronic) IS - 0003-9993 (Linking) VI - 92 IP - 7 DP - 2011 Jul TI - Effectiveness of interventions for secondary Raynaud's phenomenon: a systematic review. PG - 1166-80 LID - 10.1016/j.apmr.2011.01.022 [doi] AB - OBJECTIVES: To present an evidence-based overview of the effectiveness of (non)surgical symptomatic interventions to treat secondary Raynaud's phenomenon (RP). DATA SOURCES: The Cochrane Library, PubMed, Embase, PEDro, and CINAHL were searched for relevant systematic reviews and randomized controlled trials (RCTs). STUDY SELECTION: Two reviewers independently applied the inclusion criteria to select potential studies. DATA EXTRACTION: Two reviewers independently extracted data and assessed the methodologic quality. DATA SYNTHESIS: If pooling of data was not possible, a best-evidence synthesis was used to summarize the results. Of the 5 reviews and 19 RCTs included, 1 RCT studied acupuncture and another RCT reported on percutaneous radiofrequency thoracic sympathectomy. All others concentrated on the effectiveness of drugs (oral or intravenous [IV]). It appeared that calcium channel blockers significantly reduce the frequency and severity of Raynaud attacks, and are therefore effective in the treatment of secondary RP. Iloprost (oral and IV) was also found to be effective. Limited evidence was found for atorvastatin. For other traditional and more recently discovered interventions, no clear favorable effects were found. CONCLUSIONS: This review shows that there is clear evidence in favor of calcium channel blockers and iloprost (oral and IV) to treat secondary RP. For all other interventions, only limited, conflicting, or no evidence was found. More high-quality, well-designed RCTs are needed in this field, especially for new interventions based on recent knowledge about the pathophysiology of secondary RP. CI - Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved. FAU - Huisstede, Bionka M AU - Huisstede BM AD - Department of General Practice, Erasmus MC - University Medical Center Rotterdam, the Netherlands. b.huisstede@erasmusmc.nl FAU - Hoogvliet, Peter AU - Hoogvliet P FAU - Paulis, Winifred D AU - Paulis WD FAU - van Middelkoop, Marienke AU - van Middelkoop M FAU - Hausman, Michael AU - Hausman M FAU - Coert, J Henk AU - Coert JH FAU - Koes, Bart W AU - Koes BW LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review PL - United States TA - Arch Phys Med Rehabil JT - Archives of physical medicine and rehabilitation JID - 2985158R RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Acupuncture Therapy MH - Calcium Channel Blockers/therapeutic use MH - Humans MH - Iloprost/therapeutic use MH - Radiofrequency Therapy MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/drug therapy/*therapy MH - Vasodilator Agents/therapeutic use EDAT- 2011/06/28 06:00 MHDA- 2011/08/30 06:00 CRDT- 2011/06/28 06:00 PHST- 2010/12/02 00:00 [received] PHST- 2011/01/31 00:00 [revised] PHST- 2011/01/31 00:00 [accepted] PHST- 2011/06/28 06:00 [entrez] PHST- 2011/06/28 06:00 [pubmed] PHST- 2011/08/30 06:00 [medline] AID - S0003-9993(11)00121-3 [pii] AID - 10.1016/j.apmr.2011.01.022 [doi] PST - ppublish SO - Arch Phys Med Rehabil. 2011 Jul;92(7):1166-80. doi: 10.1016/j.apmr.2011.01.022. PMID- 33422171 OWN - NLM STAT- MEDLINE DCOM- 20211008 LR - 20211008 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 28 IP - 3 DP - 2020 Dec TI - What is the Best Way to Treat Patients with Raynaud's Phenomenon and a Tendency towards Hypotension? PG - 166-170 AB - Episodes of excessive vasospasm are common in patients with Raynaud's phenomenon (RP). Pharmacological treatment may often result in side-effects such as hypotension, leading to discontinuation of treatment. Review of therapeutic interventions with regard to tendency towards hypotension was done in medical databases including PubMed, Scopus, and Medline to summarize the current state of the knowledge. Despite the episodes of blood pressure drops caused by hypotension, calcium channel blockers (CCB) have been widely used in RP as first-line treatment medication. The use of other CCB apart from nifedipine is controversial due to the variety of results in clinical trials. A clinical study comparing the efficacy and tolerability of losartan with nifedipine revealed a significant reduction in RP severity, frequency of episodes, and reported adverse effects. Application of oral sildenafil 100 mg/d as an add-on therapy increased microvascular blood flow in secondary RP, while being well-tolerated and with no withdrawal from the study. Topical vasodilators may be applied as an adjuvant therapy for patients with RP. Clinical studies approved 10% nifedipine cream and 10% nitroglycerine gel as an efficient RP therapy with side-effects comparable with placebo usage. Non-pharmacological interventions, such as cold avoidance, stress management, and smoking cessation are recommended in reducing episodes of RP. Calcium channel blockers, with a particular emphasis on nifedipine, in combination with non-pharmacological management seem to be the optimal way to treat the patients with a tendency to hypotension. FAU - Drobek, Hanna Helena AU - Drobek HH AD - Hanna H. Drobek. MD, Medical University of Silesia in Katowice, Katowice, Poland; hdrobek@op.pl. FAU - Porwolik, Mateusz AU - Porwolik M FAU - Pietrauszka, Kornelia AU - Pietrauszka K FAU - Miziołek, Bartosz AU - Miziołek B FAU - Bergler-Czop, Beata AU - Bergler-Czop B FAU - Brzezińska-Wcisło, Ligia AU - Brzezińska-Wcisło L LA - eng PT - Journal Article PT - Review PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 RN - 0 (Antihypertensive Agents) RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - G59M7S0WS3 (Nitroglycerin) RN - I9ZF7L6G2L (Nifedipine) RN - JMS50MPO89 (Losartan) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Humans MH - Hypotension/chemically induced/*etiology MH - Losartan/therapeutic use MH - Nifedipine/therapeutic use MH - Nitroglycerin/therapeutic use MH - Raynaud Disease/*complications/*therapy MH - Sildenafil Citrate/therapeutic use MH - Smoking Cessation MH - Stress, Psychological/prevention & control MH - Vasodilator Agents/therapeutic use EDAT- 2021/01/11 06:00 MHDA- 2021/10/09 06:00 CRDT- 2021/01/10 20:25 PHST- 2021/01/10 20:25 [entrez] PHST- 2021/01/11 06:00 [pubmed] PHST- 2021/10/09 06:00 [medline] PST - ppublish SO - Acta Dermatovenerol Croat. 2020 Dec;28(3):166-170. PMID- 18571695 OWN - NLM STAT- MEDLINE DCOM- 20100202 LR - 20091125 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 39 IP - 3 DP - 2009 Dec TI - Interferon-gamma is increased in patients with primary Sjogren's syndrome and Raynaud's phenomenon. PG - 197-202 LID - 10.1016/j.semarthrit.2008.04.002 [doi] AB - OBJECTIVES: To determine the prevalence of Raynaud's phenomenon (RP) in patients with primary Sjogren's syndrome (pSS) and to identify clinical and immunological characteristics associated with this manifestation. Since increased interferon-gamma (INF-gamma) has been associated with RP, we also compared the INF-gamma production in pSS patients with or without RP. METHODS: RP was diagnosed if pSS patients presented with characteristic sequence of skin color changes of the digits. In uncertain cases noninvasive vascular tests were performed by ultrasound examination. The secretion of INF-gamma by peripheral blood mononuclear cells was assessed by enzyme-linked immunospot analysis. Further, we examined the expression of different lymphocyte activation markers (CD25, CD45RO, CD69) on CD4+ T-cells by flow cytometric analysis. RESULTS: Thirty-six of 108 patients with pSS had RP. In these patients we found a significantly increased number of INF-gamma-secreting peripheral blood mononuclear cells compared with patients without RP or to healthy controls. Further, in patients with RP a significantly increased percentage of CD25-positive T-helper cells was detectable. In addition we found an association of leukopenia, thyroiditis, and lower C3 levels with RP in pSS patients. CONCLUSIONS: These results suggest a pathogenic role of INF-gamma in pSS patients with RP. Whether the RP is immune-mediated or whether INF-gamma directly causes vasospasm still remains to be elucidated. FAU - Willeke, Peter AU - Willeke P AD - Department of Medicine B, Muenster University Hospital, Muenster, Germany. willeke.peter@uni-muenster.de FAU - Schlüter, Bernhard AU - Schlüter B FAU - Schotte, Heiko AU - Schotte H FAU - Domschke, Wolfram AU - Domschke W FAU - Gaubitz, Markus AU - Gaubitz M FAU - Becker, Heidemarie AU - Becker H LA - eng PT - Journal Article DEP - 20080624 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Complement C3) RN - 0 (HLA-DR3 Antigen) RN - 0 (HLA-DR4 Antigen) RN - 0 (Interleukin-2 Receptor alpha Subunit) RN - 82115-62-6 (Interferon-gamma) SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Complement C3/metabolism MH - Female MH - HLA-DR3 Antigen/blood MH - HLA-DR4 Antigen/blood MH - Humans MH - Interferon-gamma/*blood MH - Interleukin-2 Receptor alpha Subunit/blood MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/*blood/*epidemiology MH - Risk Factors MH - Sjogren's Syndrome/*blood/*complications MH - T-Lymphocytes, Helper-Inducer/immunology EDAT- 2008/06/24 09:00 MHDA- 2010/02/03 06:00 CRDT- 2008/06/24 09:00 PHST- 2007/12/21 00:00 [received] PHST- 2008/03/21 00:00 [revised] PHST- 2008/04/05 00:00 [accepted] PHST- 2008/06/24 09:00 [pubmed] PHST- 2010/02/03 06:00 [medline] PHST- 2008/06/24 09:00 [entrez] AID - S0049-0172(08)00079-6 [pii] AID - 10.1016/j.semarthrit.2008.04.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2009 Dec;39(3):197-202. doi: 10.1016/j.semarthrit.2008.04.002. Epub 2008 Jun 24. PMID- 19445785 OWN - NLM STAT- MEDLINE DCOM- 20160422 LR - 20250623 IS - 1752-8526 (Electronic) VI - 2008 DP - 2008 Dec 16 TI - Raynaud's phenomenon (primary). LID - 1119 [pii] AB - INTRODUCTION: Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia. On rare occasions it can lead to ulceration of the fingers and toes (and in some cases of the ears or nose). This review focuses on primary (idiopathic) Raynaud's phenomenon occurring in the absence of an underlying disease. The prevalence of primary Raynaud's phenomenon varies by sex, country, and exposure to workplace vibration. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments for primary Raynaud's phenomenon? We searched: Medline, Embase, The Cochrane Library, and other important databases up to May 2008 (Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found 15 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review we present information relating to the effectiveness and safety of the following interventions: amlodipine, diltiazem, exercise, inositol nicotinate, keeping warm, moxisylyte (thymoxamine), naftidrofuryl oxalate, nicardipine, nifedipine, prazosin, and smoking cessation. FAU - Pope, Janet Elizabeth AU - Pope JE AD - St Joseph's Health Care Rheumatology Centre, London, Ontario, Canada. LA - eng PT - Journal Article PT - Systematic Review DEP - 20081216 PL - England TA - BMJ Clin Evid JT - BMJ clinical evidence JID - 101294314 RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Administration, Oral MH - Humans MH - *Nifedipine/therapeutic use MH - Prevalence MH - *Raynaud Disease/drug therapy MH - Ulcer MH - Vibration PMC - PMC2907991 EDAT- 2008/01/01 00:00 MHDA- 2016/04/23 06:00 PMCR- 2010/12/16 CRDT- 2009/05/19 09:00 PHST- 2009/05/19 09:00 [entrez] PHST- 2008/01/01 00:00 [pubmed] PHST- 2016/04/23 06:00 [medline] PHST- 2010/12/16 00:00 [pmc-release] AID - 1119 [pii] PST - epublish SO - BMJ Clin Evid. 2008 Dec 16;2008:1119. PMID- 32423910 OWN - NLM STAT- MEDLINE DCOM- 20210205 LR - 20221207 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 13 IP - 5 DP - 2020 May 17 TI - Acral vascular syndrome during an immune checkpoint inhibitor. LID - 10.1136/bcr-2019-233463 [doi] LID - e233463 AB - Immune checkpoint inhibitors, including antiprogrammed death cell protein 1 (anti-PD-1) and anti cytotoxic T-lymphocyte-associated protein 4 (anti-CTLA-4), have been associated with a range of autoimmune-related side effects since their introduction in cancer treatment. Small vessel digital necrosis, referred to as the acral vascular syndrome, is a rare but serious complication that can result in loss of digits. Here we present a case report of acral vascular syndrome and review possible aetiologies. A 45- year-old woman with invasive ductal carcinoma of the left breast presented to the emergency department during neoadjuvant treatment with carboplatin, docetaxel and pembrolizumab with complaints of severe pain in her right third digit. She had physical findings consistent with ischaemic necrosis and gangrene of the distal phalanx. Angiography demonstrated Raynaud's phenomenon in the distal portion of the digits. Laboratory testing showed a weakly positive RNA polymerase III antibody level. Her case resulted in surgical amputation of her affected digit after partial resolution of symptoms with prednisone, vasodilators and antibiotics. CI - © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ. FAU - O'Connor, Patrick AU - O'Connor P AUID- ORCID: 0000-0002-7462-3230 AD - University of Kansas School of Medicine, Kansas City, Kansas, USA poconnor2@kumc.edu. FAU - Bhadbhade, Pooja AU - Bhadbhade P AD - Department of Allergy, Clinical Immunology, and Rheumatology, University of Kansas Medical Center, Kansas City, Kansas, USA. FAU - Khan, Qamar AU - Khan Q AD - Department of Oncology, University of Kansas Medical Center, Kansas City, Kansas, USA. FAU - Williamson, Stephen AU - Williamson S AD - Department of Medical Oncology, University of Kansas Medical Center, Kansas City, Kansas, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200517 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Immune Checkpoint Inhibitors) SB - IM EIN - BMJ Case Rep. 2021 Feb 18;14(2):e233463corr1. doi: 10.1136/bcr-2019-233463corr1. PMID: 33602782 MH - Black or African American MH - Diagnosis, Differential MH - Female MH - Fingers/blood supply/pathology MH - Gangrene/chemically induced/*diagnostic imaging/*pathology/surgery MH - Humans MH - Immune Checkpoint Inhibitors/*adverse effects MH - Middle Aged MH - Raynaud Disease/chemically induced/*diagnostic imaging/*pathology/surgery MH - Thrombosis MH - Vasculitis PMC - PMC7239533 OTO - NOTNLM OT - oncology OT - unwanted effects / adverse reactions OT - vasculitis COIS- Competing interests: None declared. EDAT- 2020/05/20 06:00 MHDA- 2021/02/07 06:00 PMCR- 2022/05/17 CRDT- 2020/05/20 06:00 PHST- 2020/05/20 06:00 [entrez] PHST- 2020/05/20 06:00 [pubmed] PHST- 2021/02/07 06:00 [medline] PHST- 2022/05/17 00:00 [pmc-release] AID - 13/5/e233463 [pii] AID - bcr-2019-233463 [pii] AID - 10.1136/bcr-2019-233463 [doi] PST - epublish SO - BMJ Case Rep. 2020 May 17;13(5):e233463. doi: 10.1136/bcr-2019-233463. PMID- 40188811 OWN - NLM STAT- MEDLINE DCOM- 20251201 LR - 20251203 IS - 1423-002X (Electronic) IS - 0378-7346 (Print) IS - 0378-7346 (Linking) VI - 90 IP - 6 DP - 2025 TI - Endometriosis, Raynaud's Syndrome, and Migraine: A Retrospective Study of 12 Million Women. PG - 632-640 LID - 10.1159/000545204 [doi] AB -

Objective: Our study focused on evaluating a possible relationship between endometriosis, Raynaud's syndrome, or migraine among women. DESIGN: This was a cross-sectional population-based study. Participants/Materials; Setting; Methods: We used 12,684,067 hospitalized patient records in the Healthcare Cost and Utilization Project (HCUP) database between 2007 and 2014. We calculated the prevalence of endometriosis, Raynaud's syndrome, and migraine. We also evaluated the possible role of depression, anxiety, and autoimmune diseases to eliminate confounding factors. Unadjusted and adjusted multivariate logistic regressions were applied to evaluate the relationship between variables. RESULTS: Unadjusted analysis revealed a strong association between endometriosis and Raynaud's syndrome and migraine (OR = 2.491; 95% CI: 1.677-3.699). After adjusting for sociodemographic characteristics as well as depression and anxiety, the association remained significant (OR = 1.779; 95% CI: 1.166-2.716). Among younger patients aged 18-35 with endometriosis, the associations were stronger with Raynaud's syndrome (adjusted OR = 1.61, 95% CI = 1.20-2.16) and migraine (adjusted OR = 2.59, 95% CI = 2.47-2.72). LIMITATIONS: The HCUP database is cross-sectional in nature, and hence, we could not establish the temporal relationship between endometriosis, Raynaud's syndrome, and migraine. Also, the severity of endometriosis and the treatment received by the patients were not included in the dataset, and it prevented us from investigating the role of potential confounding factors. CONCLUSION: Our study suggests an association between endometriosis, Raynaud's syndrome, and migraine. It is possible that these conditions share a similar mechanism possibly vascular reaction and endothelial dysfunction related to chronic inflammation.

. CI - © 2025 The Author(s). Published by S. Karger AG, Basel. FAU - Suarthana, Eva AU - Suarthana E FAU - Nassiri Kigloo, Hormoz AU - Nassiri Kigloo H AD - Research Institute, McGill University Health Centre, Montreal, Québec, Canada. FAU - Suarthana, Eva AU - Suarthana E AD - Research Institute, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Obstetrics and Gynecology, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Obstetrics and Gynecology, McGill University, Montreal, Québec, Canada. FAU - Montreuil, Tina C AU - Montreuil TC AD - Research Institute, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Psychiatry, McGill University, Montreal, Québec, Canada. FAU - Jamal, Mohammad AU - Jamal M AD - Department of Obstetrics and Gynecology, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Obstetrics and Gynecology, McGill University, Montreal, Québec, Canada. FAU - Tulandi, Togas AU - Tulandi T AD - Research Institute, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Obstetrics and Gynecology, McGill University Health Centre, Montreal, Québec, Canada. AD - Department of Obstetrics and Gynecology, McGill University, Montreal, Québec, Canada. LA - eng PT - Journal Article DEP - 20250404 PL - Switzerland TA - Gynecol Obstet Invest JT - Gynecologic and obstetric investigation JID - 7900587 SB - IM MH - Humans MH - Female MH - *Raynaud Disease/epidemiology MH - *Migraine Disorders/epidemiology MH - Adult MH - Cross-Sectional Studies MH - *Endometriosis/epidemiology MH - Retrospective Studies MH - Young Adult MH - Middle Aged MH - Prevalence MH - Adolescent PMC - PMC12112891 OTO - NOTNLM OT - Autoimmune disease OT - Chronic disease OT - Mental health OT - Population-based study COIS- The authors report no conflict of interest. EDAT- 2025/04/07 02:02 MHDA- 2025/12/01 18:51 PMCR- 2025/04/04 CRDT- 2025/04/06 18:23 PHST- 2024/08/29 00:00 [received] PHST- 2025/03/06 00:00 [accepted] PHST- 2025/12/01 18:51 [medline] PHST- 2025/04/07 02:02 [pubmed] PHST- 2025/04/06 18:23 [entrez] PHST- 2025/04/04 00:00 [pmc-release] AID - 000545204 [pii] AID - 545204 [pii] AID - 10.1159/000545204 [doi] PST - ppublish SO - Gynecol Obstet Invest. 2025;90(6):632-640. doi: 10.1159/000545204. Epub 2025 Apr 4. PMID- 35835622 OWN - NLM STAT- MEDLINE DCOM- 20221006 LR - 20221006 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 43 IP - 10 DP - 2022 Oct TI - [Paroxysmal vascular acrosyndromes: Practical approach to diagnosis and management]. PG - 596-602 LID - S0248-8663(22)00083-2 [pii] LID - 10.1016/j.revmed.2022.03.338 [doi] AB - Paroxysmal vascular acrosyndromes are related to a peripheral vasomotor disorder and presented as paroxysmal color changes of the fingers. They include primary Raynaud's phenomenon (RP), which is the most common, secondary RP and erythermalgia. They are to be distinguished from non-paroxysmal acrosyndromes such as acrocyanosis and chilblains, which are very frequent and often associated with RP, digital ischemia and necrosis, spontaneous digital hematoma and acrocholosis. The challenge of a consultation for a vascular acrosyndrome is to make positive diagnosis through history and clinical examination, and to specify its nature, to prescribe complementary exams. In any patient consulting for RP, assessment includes at least an antinuclear antibody test and capillaroscopy. For erythermalgia, a blood count and even a search for JAK2 mutation are required. A thryoid-stimulating hormon assay, a test for antinuclear antibodies, and a search for small fiber neuropathy are also performed. The treatment of RP is essentially documented for secondary RP where calcium channel blockers are indicated in first line, and iloprost in severe cases. The treatment of primitive erythermalgia is based on sodium channel blockers such as mexiletine or lidocaine infusions, and on drugs effective on neuropathic pain, such as gabapentin or amitryptiline, in case of erythermalgia associated with small fiber neuropathy. The treatment of erythermalgia associated with myeloproliferative syndromes is based on etiological treatment and aspirin. CI - Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Senet, P AU - Senet P AD - Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address: patricia.senet@aphp.fr. LA - fre PT - Journal Article TT - Acrosyndromes vasculaires paroxystiques : démarche pratique de diagnostic et de prise en charge. DEP - 20220711 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Antibodies, Antinuclear) RN - 0 (Calcium Channel Blockers) RN - 0 (Sodium Channel Blockers) RN - 1U511HHV4Z (Mexiletine) RN - 6CW7F3G59X (Gabapentin) RN - 98PI200987 (Lidocaine) RN - JED5K35YGL (Iloprost) RN - R16CO5Y76E (Aspirin) SB - IM MH - Antibodies, Antinuclear MH - Aspirin MH - Calcium Channel Blockers/therapeutic use MH - *Erythromelalgia MH - Gabapentin MH - Humans MH - Iloprost MH - Lidocaine MH - Mexiletine MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Small Fiber Neuropathy MH - Sodium Channel Blockers/therapeutic use OTO - NOTNLM OT - Acrosyndrome OT - Capillaroscopie OT - Erythermalgia OT - Nailfold capillaroscopy OT - Phénomène de Raynaud OT - Raynaud phenomenon OT - Érythermalgie EDAT- 2022/07/15 06:00 MHDA- 2022/10/07 06:00 CRDT- 2022/07/14 22:03 PHST- 2022/02/07 00:00 [received] PHST- 2022/03/20 00:00 [accepted] PHST- 2022/07/15 06:00 [pubmed] PHST- 2022/10/07 06:00 [medline] PHST- 2022/07/14 22:03 [entrez] AID - S0248-8663(22)00083-2 [pii] AID - 10.1016/j.revmed.2022.03.338 [doi] PST - ppublish SO - Rev Med Interne. 2022 Oct;43(10):596-602. doi: 10.1016/j.revmed.2022.03.338. Epub 2022 Jul 11. PMID- 16972070 OWN - NLM STAT- MEDLINE DCOM- 20070228 LR - 20181113 IS - 0020-9554 (Print) IS - 0020-9554 (Linking) VI - 47 IP - 10 DP - 2006 Oct TI - [Systemic sclerosis]. PG - 1051-61; quiz 1062 AB - Systemic sclerosis (SSc; syn. systemic scleroderma) is a rare autoimmune disorder with characteristic cutaneous manifestations. Prevalence in women is fivefold higher than in men. The course of the disease is slowly progressive with a variable degree of internal organ involvement due to fibrosis and obliteration of small vessels. The diffuse form shows more frequent and severe organ manifestations compared to the limited form. Increased mortality is particularly related to a cardiopulmonary involvement leading to a 5-year survival of around 75%. Treatment indications are dependent on the severity of the disease. In the acute state, immunosuppressive agents are needed in case of significant organ involvement. Vasodilative drugs are often used for the symptomatic treatment of Raynaud's phenomenon. FAU - Kleinert, S AU - Kleinert S AD - Schwerpunkt Rheumatologie und Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Klinikstrasse 6-8, 97070, Würzburg, Germany. Kleinert_S@klinik.uni-wuerzburg.de FAU - Tony, H P AU - Tony HP FAU - Kneitz, C AU - Kneitz C LA - ger PT - English Abstract PT - Journal Article TT - Systemische Sklerose. PL - Germany TA - Internist (Berl) JT - Der Internist JID - 0264620 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Autoantibodies/blood MH - Centromere/immunology MH - Cross-Sectional Studies MH - DNA Topoisomerases, Type I/immunology MH - Diagnosis, Differential MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Raynaud Disease/diagnosis/drug therapy MH - Scleroderma, Systemic/*diagnosis/drug therapy/mortality MH - Sex Factors MH - Survival Analysis MH - Vasodilator Agents/therapeutic use EDAT- 2006/09/15 09:00 MHDA- 2007/03/01 09:00 CRDT- 2006/09/15 09:00 PHST- 2006/09/15 09:00 [pubmed] PHST- 2007/03/01 09:00 [medline] PHST- 2006/09/15 09:00 [entrez] AID - 10.1007/s00108-006-1702-z [doi] PST - ppublish SO - Internist (Berl). 2006 Oct;47(10):1051-61; quiz 1062. doi: 10.1007/s00108-006-1702-z. PMID- 29981761 OWN - NLM STAT- MEDLINE DCOM- 20190409 LR - 20190409 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 122 DP - 2019 Mar TI - Evidence of macro- and micro-angiopathy in scleroderma: An integrated approach combining 22-MHz power Doppler ultrasonography and video-capillaroscopy. PG - 125-130 LID - S0026-2862(18)30063-3 [pii] LID - 10.1016/j.mvr.2018.07.001 [doi] AB - OBJECTIVE: We aimed to study in SSc patients macrovascular involvement by using power Doppler ultrasound (PDUS) and microvascular one by PDUS and nailfold video-capillaroscopy (NVC) and to examine the association between history of digital ulcers (HDU) and imaging (PDUS and NVC) parameters. METHODS: NVC and PDUS were systematically performed in 106 consecutive SSc patients at the 3rd and 4th finger of the dominant hand after exclusion of ulnar artery occlusion (UAO). 22 MHz PDUS measurements included nailbed and fingertip qualitatively graded, and resistivity index (RI) of ulnar and radial proper digital arteries. Capillary number/mm was calculated by NVC on the same digits examined by PDUS. RESULTS: Vascularization at fingertip and nailbed showed a good correlation between them and to capillary number. RI, representative of macrovascular involvement, did not correlate to microvascular involvement as assessed by PDUS and NVC. RI and capillary number at NVC showed significant correlation to HDU while fingertip and nailbed vascularization as assessed by PDUS did not. As such, PDUS and NVC provide different and potentially complementary information on SSc-related peripheral macro- and micro-vascular involvement. CONCLUSION: Macro- and micro-vascular involvement in SSc patients are different processes and are not present at the same time in every patient. Thus, both these aspects should be carefully evaluated in SSc patients. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Schioppo, Tommaso AU - Schioppo T AD - Division of Rheumatology, ASST Pini-CTO, Milano, Italy. FAU - Orenti, Annalisa AU - Orenti A AD - Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy; Lab of Medical Statistics, Epidemiology and Biometry GA Maccacaro, Milano, Italy. FAU - Boracchi, Patrizia AU - Boracchi P AD - Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy; Lab of Medical Statistics, Epidemiology and Biometry GA Maccacaro, Milano, Italy. FAU - De Lucia, Orazio AU - De Lucia O AD - Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy. FAU - Murgo, Antonella AU - Murgo A AD - Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Pini-CTO, Milano, Italy; Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy. Electronic address: francesca.ingegnoli@unimi.it. LA - eng PT - Journal Article PT - Observational Study DEP - 20180706 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Capillaries/*diagnostic imaging/physiopathology MH - Female MH - Humans MH - Male MH - *Microcirculation MH - *Microscopic Angioscopy MH - Middle Aged MH - Multimodal Imaging MH - Nails/*blood supply MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - Radial Artery/*diagnostic imaging/physiopathology MH - Raynaud Disease/*diagnostic imaging/etiology/physiopathology MH - Regional Blood Flow MH - Scleroderma, Systemic/complications/*diagnostic imaging/physiopathology MH - Skin Ulcer/*diagnostic imaging/etiology/physiopathology MH - Ulnar Artery/*diagnostic imaging/physiopathology MH - *Ultrasonography, Doppler OTO - NOTNLM OT - Capillaroscopy OT - Iloprost OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Ultrasound EDAT- 2018/07/10 06:00 MHDA- 2019/04/10 06:00 CRDT- 2018/07/09 06:00 PHST- 2018/04/06 00:00 [received] PHST- 2018/07/03 00:00 [revised] PHST- 2018/07/04 00:00 [accepted] PHST- 2018/07/10 06:00 [pubmed] PHST- 2019/04/10 06:00 [medline] PHST- 2018/07/09 06:00 [entrez] AID - S0026-2862(18)30063-3 [pii] AID - 10.1016/j.mvr.2018.07.001 [doi] PST - ppublish SO - Microvasc Res. 2019 Mar;122:125-130. doi: 10.1016/j.mvr.2018.07.001. Epub 2018 Jul 6. PMID- 25752312 OWN - NLM STAT- MEDLINE DCOM- 20151002 LR - 20220408 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 54 IP - 8 DP - 2015 Aug TI - Prediction and impact of attacks of Raynaud's phenomenon, as judged by patient perception. PG - 1443-7 LID - 10.1093/rheumatology/kev002 [doi] AB - OBJECTIVE: To evaluate whether patients can predict attacks of RP (if so, this would have implications for developing new treatments) and to evaluate the impact of RP attacks on quality of life (QoL). METHODS: Individuals with RP were invited through international patient associations to participate in an online survey. RESULTS: Responses from 443 subjects with self-reported RP from 15 countries were evaluable. The mean age of subjects was 41 years (91% female). Fifty-eight per cent of subjects reported they could predict at least 51% of RP attacks, and 57% could predict attack severity either fairly well or better [with 43% predicting severity only poorly (30%) or very poorly (13%)]. Sixty-four per cent of subjects reported a poor or very poor current ability to prevent/control RP attacks. One hundred and eighty-two subjects (41%) reported current or previous use of medications for RP: 82% reported at least one currently used medication being tolerated, but only 16% reported at least one current medication being effective. Most subjects (78%) reported making at least one life adjustment due to RP, with more in subjects with secondary RP compared with primary RP (87% vs 71%, P = 0.001). Current QoL with RP was impaired [mean = 6/10 (10 best imaginable)] and secondary RP subjects reported a greater absolute improvement when asked to imagine their QoL without RP (2.3 vs 3.3 P = 0.0002). CONCLUSION: Subjects' ability to predict RP attacks is limited. Treatments were generally considered tolerable but seldom fully effective. Our results confirm an unmet need for new treatments. RP significantly impacts on QoL in all subjects. CI - © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK, michael.hughes-6@postgrad.manchester.ac.uk. FAU - Snapir, Amir AU - Snapir A AD - Orion Corporation Orion Pharma, Research and Development, Turku, Finland. FAU - Wilkinson, Jack AU - Wilkinson J AD - Research and Development, Salford Royal NHS Foundation Trust, Salford, UK and. FAU - Snapir, Daniel AU - Snapir D AD - Orion Corporation Orion Pharma, Research and Development, Turku, Finland. FAU - Wigley, Fredrick M AU - Wigley FM AD - Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20150309 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Data Collection MH - Europe MH - Female MH - Forecasting MH - Humans MH - International Cooperation MH - Male MH - Middle Aged MH - Patient Participation/*psychology MH - Quality of Life/psychology MH - Raynaud Disease/*diagnosis/*psychology MH - *Self Report MH - Severity of Illness Index MH - Young Adult OTO - NOTNLM OT - Raynaud’s phenomenon OT - impact OT - predict OT - quality of life OT - severity OT - systemic sclerosis EDAT- 2015/03/11 06:00 MHDA- 2015/10/03 06:00 CRDT- 2015/03/11 06:00 PHST- 2014/07/09 00:00 [received] PHST- 2015/03/11 06:00 [entrez] PHST- 2015/03/11 06:00 [pubmed] PHST- 2015/10/03 06:00 [medline] AID - kev002 [pii] AID - 10.1093/rheumatology/kev002 [doi] PST - ppublish SO - Rheumatology (Oxford). 2015 Aug;54(8):1443-7. doi: 10.1093/rheumatology/kev002. Epub 2015 Mar 9. PMID- 33386120 OWN - NLM STAT- MEDLINE DCOM- 20210423 LR - 20210423 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 361 IP - 4 DP - 2021 Apr TI - A 65-year-old Woman With Persistent Dyspnea, Arthritis, and Raynaud's Phenomenon. PG - 526-533 LID - S0002-9629(20)30500-0 [pii] LID - 10.1016/j.amjms.2020.11.012 [doi] AB - Antisynthetase syndrome (AS) is a rare disease that affects patients with inflammatory myopathies such as polymyositis (PM) and dermatomyositis (DM). In patients with AS, up to 95% of patients develop antisynthetase syndrome-associated interstitial lung disease (AS-ILD). Although AS-ILD commonly occurs in patients with a well-established diagnosis of AS, it can be the first or only manifestation of an occult AS. The frequency of interstitial lung disease (ILD), myopathy, and skin involvement are often dependent on the type of myositis-specific antibodies present. AS-ILD patients who are positive for both anti-Jo-1 and anti-SSA/RO-52 autoantibodies often present with a severe degree of lung restriction on pulmonary function tests and radiologic imaging with an inadequate response toward immunosuppressive therapies. We describe a 65-year-old woman who presents with chronic dyspnea. She was initially diagnosed with corticosteroid-resistant cryptogenic organizing pneumonia based on the radiological findings on her CT chest. Her symptoms did not improve, and she suffered from intolerable corticosteroid-related side effects. Reviews of systems were positive for arthritis and Raynaud's phenomenon. She was found to have elevated inflammatory markers and autoantibodies such as anti-Jo-1, anti-RO-52, and anti-SSA. A diagnosis of AS-ILD resistant to corticosteroid therapy was made. Her lung function improved with combination therapy of mycophenolate and rituximab. Our case highlights that a detailed history and physical exam, compatible radiologic imaging, and autoantibodies are essential for the diagnosis of AS-ILD. CI - Copyright © 2020 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved. FAU - Chong, Woon H AU - Chong WH AD - Department of Pulmonary and Critical Care Medicine, Albany Medical Center, New York. Electronic address: chongw@amc.edu. FAU - Saha, Biplab K AU - Saha BK AD - Department of Pulmonary and Critical Care, Ozarks Medical Center, West Plains, Missouri. FAU - Beegle, Scott AU - Beegle S AD - Department of Pulmonary and Critical Care Medicine, Albany Medical Center, New York. LA - eng PT - Case Reports PT - Journal Article DEP - 20201122 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) RN - HU9DX48N0T (Mycophenolic Acid) RN - Antisynthetase syndrome SB - IM MH - Aged MH - Antirheumatic Agents/therapeutic use MH - Arthritis/*drug therapy/etiology MH - Drug Therapy, Combination MH - Dyspnea/*drug therapy/etiology MH - Female MH - Humans MH - Lung Diseases, Interstitial/complications/*diagnosis/drug therapy MH - Mycophenolic Acid/therapeutic use MH - Myositis/complications/*diagnosis/drug therapy MH - Raynaud Disease/*drug therapy/etiology MH - Rituximab/therapeutic use OTO - NOTNLM OT - Antisynthetase syndrome OT - Antisynthetase syndrome-associated with interstitial lung disease OT - Connective tissue disease-associated with interstitial lung disease OT - interstitial lung disease EDAT- 2021/01/03 06:00 MHDA- 2021/04/24 06:00 CRDT- 2021/01/02 05:14 PHST- 2020/06/01 00:00 [received] PHST- 2020/10/02 00:00 [revised] PHST- 2020/11/18 00:00 [accepted] PHST- 2021/01/03 06:00 [pubmed] PHST- 2021/04/24 06:00 [medline] PHST- 2021/01/02 05:14 [entrez] AID - S0002-9629(20)30500-0 [pii] AID - 10.1016/j.amjms.2020.11.012 [doi] PST - ppublish SO - Am J Med Sci. 2021 Apr;361(4):526-533. doi: 10.1016/j.amjms.2020.11.012. Epub 2020 Nov 22. PMID- 27155203 OWN - NLM STAT- MEDLINE DCOM- 20180105 LR - 20180105 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 55 IP - 7 DP - 2016 Jul TI - Management of Raynaud's digital ulcer with interdigital Botulinum toxin A injection. PG - 1216 LID - 10.1093/rheumatology/kew225 [doi] FAU - Navarro, Erika P AU - Navarro EP AD - Internal Medicine Department. FAU - Cañas, Carlos A AU - Cañas CA AD - Rheumatology Department, CES University, Fundación Valle del Lili, Cali, Colombia. FAU - Tobón, Gabriel J AU - Tobón GJ AD - Rheumatology Department, CES University, Fundación Valle del Lili, Cali, Colombia gtobon1@yahoo.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20160506 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Botulinum Toxins, Type A/administration & dosage/*therapeutic use MH - Female MH - *Fingers MH - Humans MH - Injections, Intradermal MH - Neuromuscular Agents/administration & dosage/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Skin Ulcer/*drug therapy EDAT- 2016/05/08 06:00 MHDA- 2018/01/06 06:00 CRDT- 2016/05/08 06:00 PHST- 2016/05/08 06:00 [entrez] PHST- 2016/05/08 06:00 [pubmed] PHST- 2018/01/06 06:00 [medline] AID - kew225 [pii] AID - 10.1093/rheumatology/kew225 [doi] PST - ppublish SO - Rheumatology (Oxford). 2016 Jul;55(7):1216. doi: 10.1093/rheumatology/kew225. Epub 2016 May 6. PMID- 23952193 OWN - NLM STAT- MEDLINE DCOM- 20140319 LR - 20151119 IS - 1557-8992 (Electronic) IS - 1044-5463 (Linking) VI - 23 IP - 6 DP - 2013 Aug TI - Dose-dependent Raynaud's phenomenon developing from use of atomoxetine in a girl. PG - 428-30 LID - 10.1089/cap.2012.0131 [doi] FAU - Gökçen, Cem AU - Gökçen C FAU - Kutuk, Meryem Ozlem AU - Kutuk MO FAU - Coşkun, Şeyma AU - Coşkun Ş LA - eng PT - Case Reports PT - Letter PL - United States TA - J Child Adolesc Psychopharmacol JT - Journal of child and adolescent psychopharmacology JID - 9105358 RN - 0 (Adrenergic Uptake Inhibitors) RN - 0 (Propylamines) RN - 57WVB6I2W0 (Atomoxetine Hydrochloride) SB - IM MH - Adrenergic Uptake Inhibitors/administration & dosage/*adverse effects/therapeutic use MH - Atomoxetine Hydrochloride MH - Attention Deficit Disorder with Hyperactivity/drug therapy MH - Child MH - Dose-Response Relationship, Drug MH - Female MH - Humans MH - Propylamines/administration & dosage/*adverse effects/therapeutic use MH - Raynaud Disease/*chemically induced EDAT- 2013/08/21 06:00 MHDA- 2014/03/22 06:00 CRDT- 2013/08/20 06:00 PHST- 2013/08/20 06:00 [entrez] PHST- 2013/08/21 06:00 [pubmed] PHST- 2014/03/22 06:00 [medline] AID - 10.1089/cap.2012.0131 [doi] PST - ppublish SO - J Child Adolesc Psychopharmacol. 2013 Aug;23(6):428-30. doi: 10.1089/cap.2012.0131. PMID- 19797310 OWN - NLM STAT- MEDLINE DCOM- 20101105 LR - 20131121 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 1 DP - 2010 Jan TI - A retrospective clinical analysis of pharmacological modalities used for symptomatic relief of Raynaud's phenomenon in children treated in a UK paediatric rheumatology centre. PG - 193-4 LID - 10.1093/rheumatology/kep309 [doi] FAU - Gargh, Kapil AU - Gargh K FAU - Baildam, Eileen M AU - Baildam EM FAU - Cleary, Gavin A AU - Cleary GA FAU - Beresford, Michael W AU - Beresford MW FAU - McCann, Liza J AU - McCann LJ LA - eng PT - Letter DEP - 20091001 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Calcium Channel Blockers) RN - 0 (Delayed-Action Preparations) RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Administration, Cutaneous MH - Adolescent MH - Calcium Channel Blockers/administration & dosage MH - Child MH - Child, Preschool MH - Delayed-Action Preparations MH - Female MH - Humans MH - Infant MH - Male MH - Nitroglycerin/administration & dosage MH - Raynaud Disease/*drug therapy MH - Retrospective Studies MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage EDAT- 2009/10/03 06:00 MHDA- 2010/11/06 06:00 CRDT- 2009/10/03 06:00 PHST- 2009/10/03 06:00 [entrez] PHST- 2009/10/03 06:00 [pubmed] PHST- 2010/11/06 06:00 [medline] AID - kep309 [pii] AID - 10.1093/rheumatology/kep309 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Jan;49(1):193-4. doi: 10.1093/rheumatology/kep309. Epub 2009 Oct 1. PMID- 36197673 OWN - NLM STAT- MEDLINE DCOM- 20221007 LR - 20221224 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 8 IP - 2 DP - 2022 Sep TI - Understanding the value of non-specific abnormal capillary dilations in presence of Raynaud's phenomenon: a detailed capillaroscopic analysis. LID - 10.1136/rmdopen-2022-002449 [doi] LID - e002449 AB - BACKGROUND: Nailfold videocapillaroscopy (NVC) non-specific abnormalities may be present in subjects with isolated Raynaud's phenomenon (RP) before the potential transition to systemic sclerosis (SSc) specific microvascular alterations ('scleroderma pattern'). This study aims to investigate NVC non-specific abnormalities, notably capillary dilations, in RP patients, as possible forerunners of the 'scleroderma pattern'. METHODS: A 10-year retrospective NVC-based investigation evaluated 55 RP patients sorted into 3 sex-matched and age-matched groups according to clinical evolution: 18 later developing SSc (cases), 19 later developing other connective tissue disease and 18 maintaining primary RP at long-term follow-up (controls). All patients had a basal NVC showing non-specific abnormalities, namely non-specific >30 µm dilated capillaries (30-50 μm diameter). Sequential NVCs were longitudinally evaluated using current standardised approach. Statistical analysis assessed the risk for developing a 'scleroderma pattern'. RESULTS: Significantly larger capillary diameters were observed in cases versus controls both at basal NVC and during follow-up NVC (p=<0.05 to <0.001). Interestingly, controls showed stable NVC non-specific abnormalities over the study follow-up. The number of >30 µm dilated capillaries/mm at basal NVC was the strongest single predictor of 'scleroderma pattern' evolution with 24% increased risk per each dilated capillary (OR 1.24, 95% CI 1.17,1.32). Additionally, a tree-based analysis suggested the efferent (venous) diameter of the most dilated capillary on basal NVCas a variable of interest to identify patients maintaining primary RP. CONCLUSION: This is the first study to describe an NVC 'prescleroderma signature' to potentially identify RP patients later developing a 'scleroderma pattern'. CI - © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Pacini, Greta AU - Pacini G AUID- ORCID: 0000-0003-3678-4452 AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Pogna, Andrea AU - Pogna A AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Pendolino, Monica AU - Pendolino M AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Carmisciano, Luca AU - Carmisciano L AD - Biostatistics Unit, Department of Health Sciences, University of Genoa, Genova, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Schenone, Carlotta AU - Schenone C AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Department of Rheumatology, University Hospital Ghent, Gent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine and Specialties, IRCCS Ospedale Policlinico San Martino, Genova, Italy mcutolo@unige.it. LA - eng PT - Journal Article PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM EIN - RMD Open. 2022 Dec;8(2):e002449corr1. doi: 10.1136/rmdopen-2022-002449corr1. PMID: 36564102 MH - Capillaries MH - Dilatation MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/diagnosis/etiology MH - Retrospective Studies MH - *Scleroderma, Systemic/diagnosis PMC - PMC9462093 OTO - NOTNLM OT - Connective Tissue Diseases OT - Immune System Diseases OT - Scleroderma, Systemic COIS- Competing interests: None declared. EDAT- 2022/10/06 06:00 MHDA- 2022/10/12 06:00 PMCR- 2022/09/08 CRDT- 2022/10/05 11:33 PHST- 2022/05/02 00:00 [received] PHST- 2022/08/22 00:00 [accepted] PHST- 2022/10/05 11:33 [entrez] PHST- 2022/10/06 06:00 [pubmed] PHST- 2022/10/12 06:00 [medline] PHST- 2022/09/08 00:00 [pmc-release] AID - rmdopen-2022-002449 [pii] AID - 10.1136/rmdopen-2022-002449 [doi] PST - ppublish SO - RMD Open. 2022 Sep;8(2):e002449. doi: 10.1136/rmdopen-2022-002449. PMID- 23292819 OWN - NLM STAT- MEDLINE DCOM- 20130531 LR - 20220408 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 15 IP - 1 DP - 2013 Jan TI - Management of Raynaud's phenomenon and digital ischemia. PG - 303 LID - 10.1007/s11926-012-0303-1 [doi] AB - This review focuses on new findings and developments relevant to the clinician caring for patients with primary and secondary [especially systemic sclerosis (SSc)-related] Raynaud phenomenon (RP). In the last 18 months, several clinical trials and observational studies of RP and of SSc-related digital ulceration have been published, reflecting increased awareness of disease burden and increased interest by pharmaceutical companies: new insights into pathophysiology are driving new approaches to treatment. Key developments are the increased use of phosphodiesterase type V inhibitors in severe RP, and of bosentan (an endothelin-1 receptor antagonist) for prevention of recurrent SSc-related digital ulcers. Other treatments being researched include topical glyceryl trinitrate (applied locally to the digits), botulinum toxin (for severe digital ischemia/ulceration), and several other drugs including oral prostanoids. Increased availability and interest in nailfold capillaroscopy, by facilitating early diagnosis of SSc, should pave the way for studies of early intervention and vascular protection. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, Institute of Inflammation and Repair, University of Manchester, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, University of Manchester, Salford M6 8HD, UK. ariane.herrick@manchester.ac.uk LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - 0 (Phosphodiesterase Inhibitors) SB - IM MH - Fingers/*blood supply MH - Humans MH - Ischemia/*drug therapy/etiology MH - Phosphodiesterase Inhibitors/therapeutic use MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/complications/diagnosis MH - Skin Ulcer/etiology/prevention & control EDAT- 2013/01/08 06:00 MHDA- 2013/06/01 06:00 CRDT- 2013/01/08 06:00 PHST- 2013/01/08 06:00 [entrez] PHST- 2013/01/08 06:00 [pubmed] PHST- 2013/06/01 06:00 [medline] AID - 10.1007/s11926-012-0303-1 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2013 Jan;15(1):303. doi: 10.1007/s11926-012-0303-1. PMID- 27258205 OWN - NLM STAT- MEDLINE DCOM- 20170426 LR - 20181202 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 65 IP - 1 DP - 2017 TI - Altered microrheological parameters in Raynaud's phenomenon. PG - 23-29 LID - 10.3233/CH-162069 [doi] AB - Raynaud's phenomenon is an episodic, painful attack of the acral parts caused by local diminished blood supply. The aim of our study was to examine hemorheological parameters, cold agglutinins, cryoglobulins and their relationship in patients suffering from Raynaud's phenomenon.Blood was taken from 74 patients (mean age: 48 years, female/male: 56/18). Cold agglutinins and cryoglobulins were determined. Hemorheological parameters were also measured such as hematocrit, plasma and whole blood viscosity, red blood cell aggregation and deformability. Results were compared to a group of 58 healthy controls (mean age: 31.5 years, female/male: 24/34).Cold agglutinins were positive in 70%, cryoglobulins in 43% of patients. When compared to healthy controls, increased red blood cell aggregation (64.54  ±  8.93 vs. 61.11  ±  7.05) and decreased red blood cell deformability (0.669  ±  0.002 vs. 0.681  ±  0.001) was observed in Raynaud's patients (p < 0.05), but there were no differences in hematocrit (43.27% ± 3.85 vs. 44.10% ± 3.70), plasma (1.27 mPas ± 0.08 vs. 1.24 mPas ± 0.09) and whole blood viscosity (4.12 mPas ± 0.52 vs. 4.26 mPas ± 0.46). No differences were found between the hemorheological profile of cold agglutinin/cryoglobulin positive and negative patients. Also primary and secondary Raynaud's patients had similar rheological profile.Erythrocyte aggregation and deformability seems to be unfavorable in Raynaud's patients that can play a role in the disturbance of the microcirculation. FAU - Papp, Judit AU - Papp J AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Sandor, Barbara AU - Sandor B AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Toth, Andras AU - Toth A AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Biro, Katalin AU - Biro K AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Rabai, Miklos AU - Rabai M AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Botor, David AU - Botor D AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Kovacs, David AU - Kovacs D AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Csernus, Zita AU - Csernus Z AD - Hungarian National Blood Transfusion Service, Regional Centre Pecs, Pecs, Hungary. FAU - Toth, Kalman AU - Toth K AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. FAU - Kesmarky, Gabor AU - Kesmarky G AD - 1st Department of Medicine, University of Pecs, School of Medicine, Pecs, Hungary. LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 0 (Cryoglobulins) RN - 0 (cold agglutinins) SB - IM MH - Cryoglobulins MH - Erythrocyte Aggregation MH - Erythrocyte Deformability MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*blood MH - *Rheology OTO - NOTNLM OT - Raynaud’s phenomenon OT - cold agglutinin OT - cryoglobulin OT - erythrocyte deformability OT - erythrocyte aggregation OT - hemorheology OT - viscosity EDAT- 2016/06/04 06:00 MHDA- 2017/04/27 06:00 CRDT- 2016/06/04 06:00 PHST- 2016/06/04 06:00 [pubmed] PHST- 2017/04/27 06:00 [medline] PHST- 2016/06/04 06:00 [entrez] AID - CH2069 [pii] AID - 10.3233/CH-162069 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2017;65(1):23-29. doi: 10.3233/CH-162069. PMID- 20858141 OWN - NLM STAT- MEDLINE DCOM- 20101221 LR - 20131121 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 39 IP - 6 DP - 2010 Nov TI - Pheochromocytoma in a patient with a preliminary diagnosis of Raynaud's phenomenon. PG - 523-4 LID - 10.3109/03009742.2010.487052 [doi] FAU - Keles, Z AU - Keles Z FAU - Onur, O AU - Onur O FAU - Carlioglu, A AU - Carlioglu A FAU - Sarifakioglu, E AU - Sarifakioglu E LA - eng PT - Case Reports PT - Letter DEP - 20100921 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antihypertensive Agents) RN - 0 (Platelet Aggregation Inhibitors) RN - X4W3ENH1CV (Norepinephrine) RN - YKH834O4BH (Epinephrine) SB - IM MH - Adrenal Gland Neoplasms/*diagnosis/surgery MH - Adult MH - Antihypertensive Agents/therapeutic use MH - Cardiomyopathy, Dilated/complications/diagnosis/drug therapy MH - Diagnosis, Differential MH - Epinephrine/blood MH - Heart Failure/drug therapy/etiology MH - Humans MH - Male MH - Norepinephrine/blood MH - Pheochromocytoma/*diagnosis/surgery MH - Platelet Aggregation Inhibitors/therapeutic use MH - Raynaud Disease/*diagnosis MH - Treatment Outcome EDAT- 2010/09/23 06:00 MHDA- 2010/12/22 06:00 CRDT- 2010/09/23 06:00 PHST- 2010/09/23 06:00 [entrez] PHST- 2010/09/23 06:00 [pubmed] PHST- 2010/12/22 06:00 [medline] AID - 10.3109/03009742.2010.487052 [doi] PST - ppublish SO - Scand J Rheumatol. 2010 Nov;39(6):523-4. doi: 10.3109/03009742.2010.487052. Epub 2010 Sep 21. PMID- 20303994 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20100601 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 80 IP - 1 DP - 2010 Jul TI - Impaired digital vascular function mimicking Raynaud's phenomenon as a complication of argon laser therapy in tuberous sclerosis. PG - 2 LID - 10.1016/j.mvr.2010.03.006 [doi] FAU - Pauling, J D AU - Pauling JD FAU - Shipley, J A AU - Shipley JA FAU - McHugh, N J AU - McHugh NJ LA - eng PT - Case Reports PT - Letter DEP - 20100318 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Cold Temperature MH - Diagnosis, Differential MH - Fibroma/etiology/*surgery MH - Fingers/*blood supply/physiopathology/radiation effects MH - Humans MH - Laser Therapy/*adverse effects MH - Lasers, Gas/therapeutic use MH - Male MH - Microvessels/physiopathology/radiation effects MH - Middle Aged MH - Pain/etiology MH - Pallor/etiology MH - Raynaud Disease/*diagnosis MH - Tuberous Sclerosis/complications/*surgery MH - Vascular Diseases/*diagnosis/*etiology/physiopathology EDAT- 2010/03/23 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/03/23 06:00 PHST- 2010/02/18 00:00 [received] PHST- 2010/03/11 00:00 [revised] PHST- 2010/03/12 00:00 [accepted] PHST- 2010/03/23 06:00 [entrez] PHST- 2010/03/23 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - S0026-2862(10)00051-8 [pii] AID - 10.1016/j.mvr.2010.03.006 [doi] PST - ppublish SO - Microvasc Res. 2010 Jul;80(1):2. doi: 10.1016/j.mvr.2010.03.006. Epub 2010 Mar 18. PMID- 16804699 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20260128 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 4 DP - 2006 Jul TI - [Evidence-based therapy of Raynaud's syndrome]. PG - 285-9 AB - Raynaud's syndrome has a prevalence of 3-5% in the general population. Despite its high frequency, the majority of available therapies have not been validated in randomized controlled trials. Effective therapies with a high level of evidence include the calcium channel blocker nifedipine. As analyzed by meta-analyses, nifedipine showed improvement of the peripheral circulation, as well as reduction of both the intensity and frequency of attacks in patients with primary and secondary Raynaud's syndrome as compared to placebo. Similar results in a metaanalysis were obtained for intravenous infusions of iloprost in patients with secondary Raynaud's phenomenon associated with systemic sclerosis. In addition, intravenous infusions of iloprost improved healing of fingertip ulcers in patients with systemic sclerosis. Therapies with significant effects in single randomized controlled trials include angiotensin II-receptor type 1 antagonists (losartan), the calcium channel blockers felodipine und amlodipine, serotonin-reuptake-inhibitors (fluoxetine) und phosphodiesterase-V-inhibitors (sildenafil, vardenafil). However, the results for these promising substances have to be confirmed in long-term trials with larger patient numbers. FAU - Distler, M AU - Distler M AD - Abt. Dermatologie und Venerologie, Georg-August-Universität Göttingen. FAU - Distler, J AU - Distler J FAU - Ciurea, A AU - Ciurea A FAU - Kyburz, D AU - Kyburz D FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Reich, K AU - Reich K FAU - Distler, O AU - Distler O LA - ger PT - Journal Article PT - Review TT - Evidenzbasierte Therapie des Raynaud-Syndroms. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - EC 3.1.4.35 (3',5'-Cyclic-GMP Phosphodiesterases) RN - 0 (Angiotensin II Type 1 Receptor Blockers) RN - Q326023R30 (Bosentan) RN - 0 (Calcium Channel Blockers) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) RN - 0 (Endothelin Receptor Antagonists) RN - DCR9Z582X0 (Epoprostenol) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - EC 3.1.4.35 (PDE5A protein, human) SB - IM MH - 3',5'-Cyclic-GMP Phosphodiesterases/antagonists & inhibitors MH - Angiotensin II Type 1 Receptor Blockers/therapeutic use MH - Bosentan MH - Calcium Channel Blockers/therapeutic use MH - Cyclic Nucleotide Phosphodiesterases, Type 5 MH - Endothelin Receptor Antagonists MH - Epoprostenol/analogs & derivatives/therapeutic use MH - *Evidence-Based Medicine MH - Humans MH - Phosphodiesterase Inhibitors/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/diagnosis/*drug therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Sulfonamides/therapeutic use MH - Vasodilator Agents/*therapeutic use RF - 38 EDAT- 2006/06/29 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/06/29 09:00 PHST- 2006/06/29 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/06/29 09:00 [entrez] AID - 10.1007/s00393-006-0068-x [doi] PST - ppublish SO - Z Rheumatol. 2006 Jul;65(4):285-9. doi: 10.1007/s00393-006-0068-x. PMID- 22079910 OWN - NLM STAT- MEDLINE DCOM- 20120917 LR - 20180302 IS - 1559-0488 (Electronic) IS - 1559-047X (Linking) VI - 33 IP - 3 DP - 2012 May-Jun TI - Management of severe rheumatological disease in the burn center. PG - e153-9 LID - 10.1097/BCR.0b013e318233fad6 [doi] AB - In recent years, Burn Center has evolved to become a "wound intensive care unit" treating disease processes other than those due to thermal injury. Recent data have shown that more than 16% of admissions to Burn Centers are for nonburn injuries, particularly severe dermatologic diseases. The role of the Burn Center has been expanded to include treatment of patients with severe cutaneous manifestations of rheumatologic diseases. This approach has not been described before. All collagen vascular disease admissions to the Burn Center from 2005 to 2010 have been reviewed. There were 16 admissions where intensive wound management was a major component of the disease management. Disease processes included systemic lupus erythematosus, progressive systemic sclerosis, Raynaud's phenomenon, antiphospholipid syndrome, and dermatomyositis, among others. The authors describe five of these cases in detail. Comanagement of these patients by the Rheumatology and Burn services led to outstanding, successful outcomes. Collagen vascular diseases represent another area where the Burn Center may be the appropriate site for therapy. FAU - Reddy, Preetham AU - Reddy P AD - Section of Rheumatology, Regions Hospital, St. Paul, Minnesota 55101, USA. FAU - Ahrenholz, David H AU - Ahrenholz DH FAU - Mohr, William J 3rd AU - Mohr WJ 3rd FAU - Gertner, Elie AU - Gertner E LA - eng PT - Journal Article PL - England TA - J Burn Care Res JT - Journal of burn care & research : official publication of the American Burn Association JID - 101262774 SB - IM MH - Academic Medical Centers MH - Adult MH - Aged MH - Antiphospholipid Syndrome/complications/diagnosis/therapy MH - Burn Units MH - Burns/*complications/diagnosis/*therapy MH - Combined Modality Therapy MH - Disease Management MH - Female MH - Follow-Up Studies MH - Humans MH - Injury Severity Score MH - Lupus Erythematosus, Systemic/complications/diagnosis/therapy MH - Male MH - Middle Aged MH - Raynaud Disease/complications/diagnosis/therapy MH - Retrospective Studies MH - Rheumatic Diseases/*complications/diagnosis/*therapy MH - Risk Assessment MH - Sampling Studies MH - Scleroderma, Systemic/complications/diagnosis/therapy MH - Severity of Illness Index MH - Sjogren's Syndrome/complications/diagnosis/therapy MH - Treatment Outcome MH - Young Adult EDAT- 2011/11/15 06:00 MHDA- 2012/09/18 06:00 CRDT- 2011/11/15 06:00 PHST- 2011/11/15 06:00 [entrez] PHST- 2011/11/15 06:00 [pubmed] PHST- 2012/09/18 06:00 [medline] AID - 10.1097/BCR.0b013e318233fad6 [doi] PST - ppublish SO - J Burn Care Res. 2012 May-Jun;33(3):e153-9. doi: 10.1097/BCR.0b013e318233fad6. PMID- 30654995 OWN - NLM STAT- MEDLINE DCOM- 20200302 LR - 20200302 IS - 1879-1360 (Electronic) IS - 0022-3999 (Linking) VI - 116 DP - 2019 Jan TI - Comparison of mental and physical health between patients with primary and secondary Raynaud's phenomenon Category: Article. PG - 6-9 LID - S0022-3999(18)30689-5 [pii] LID - 10.1016/j.jpsychores.2018.11.001 [doi] AB - OBJECTIVE: To compare anxiety, depression, physical health and quality of life in patients with primary Raynaud's phenomenon (PRP) and patients with secondary Raynaud's phenomenon (SRP). METHOD: Adult patients with Raynaud's phenomenon (RP) were assessed for the severity of anxiety and depressive symptoms, physical health and quality of life by using the General Anxiety Disorder Scale (GAD-7), the Beck Depression Inventory (BDI), the 12-item Short Form Health Survey: Physical Component Scale (PCS-12), and the Raynaud Specific Quality of Life Questionnaire (RQLQ), respectively. Patients with PRP and SRP were recruited for comparison at a single clinical center in Debrecen, Hungary from September to December 2017. RESULTS: In total, 60 primary and 41 secondary patients with RP were studied. Gender distribution, family status, employment status and smoking were similar in the two groups. Significantly more patients with SRP had anxiety and depressive symptoms than patients with PRP. Patients with SRP had significantly higher GAD-7 and BDI; and lower overall PCS-12 and RQLQ scores than patients with PRP. CONCLUSION: Anxiety and depression is more common in patients with SRP than in patients with PRP. Patients with SRP have a lower physical health condition and RP specific quality of life than patients with PRP. Anxiety, depression and quality of life impairments should be taken into account when managing all patients with RP. Further study is needed to assess whether appropriately designed interventions have the potential to reduce the mental and physical health burdens of RP on quality of life (QOL), especially in patients with SRP. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Fábián, Balázs AU - Fábián B AD - Department of Behavioural Sciences, Faculty of Public Health, University of Debrecen, Debrecen, Hungary. Electronic address: fabian.balazs@sph.unideb.hu. FAU - Fábián, Anna Klaudia AU - Fábián AK AD - Department of Behavioural Sciences, Faculty of Public Health, University of Debrecen, Debrecen, Hungary. FAU - Bugán, Antal AU - Bugán A AD - Department of Behavioural Sciences, Faculty of Public Health, University of Debrecen, Debrecen, Hungary. FAU - Csiki, Zoltán AU - Csiki Z AD - Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. LA - eng PT - Journal Article DEP - 20181105 PL - England TA - J Psychosom Res JT - Journal of psychosomatic research JID - 0376333 SB - IM MH - Female MH - Humans MH - Male MH - Mental Health/*trends MH - Middle Aged MH - *Physical Functional Performance MH - Quality of Life/*psychology MH - Raynaud Disease/*psychology OTO - NOTNLM OT - Anxiety OT - Depression OT - Quality of life OT - Raynaud's phenomenon EDAT- 2019/01/19 06:00 MHDA- 2020/03/03 06:00 CRDT- 2019/01/19 06:00 PHST- 2018/07/18 00:00 [received] PHST- 2018/11/03 00:00 [revised] PHST- 2018/11/04 00:00 [accepted] PHST- 2019/01/19 06:00 [entrez] PHST- 2019/01/19 06:00 [pubmed] PHST- 2020/03/03 06:00 [medline] AID - S0022-3999(18)30689-5 [pii] AID - 10.1016/j.jpsychores.2018.11.001 [doi] PST - ppublish SO - J Psychosom Res. 2019 Jan;116:6-9. doi: 10.1016/j.jpsychores.2018.11.001. Epub 2018 Nov 5. PMID- 22966961 OWN - NLM STAT- MEDLINE DCOM- 20140624 LR - 20220311 IS - 1369-1635 (Electronic) IS - 0953-7104 (Linking) VI - 24 IP - 7 DP - 2013 TI - The contribution of platelets to the pathogenesis of Raynaud's phenomenon and systemic sclerosis. PG - 503-15 LID - 10.3109/09537104.2012.719090 [doi] AB - Raynaud's phenomenon (RP) describes the excessive vascular response of the digital vessels in response to cold exposure and emotional stress. It is typically the earliest manifestation of systemic sclerosis (SSc), a multisystem disease of unknown aetiology characterised by vasculopathy, inflammation and fibrosis. The biological actions of platelets are known to extend beyond primary haemostasis with a growing appreciation of their contribution to vascular function, inflammation and wound repair. This has led to a considerable body of work evaluating associations between platelet function analysis and RP/SSc. This review provides a conceptual framework upon which the potential contribution of platelets to vascular dysfunction, autoimmunity and tissue remodelling in RP and SSc is considered. We describe the existing evidence to support excessive platelet activation in RP and SSc, ranging from the early studies of platelet aggregability and circulating platelet-derived mediators, to the important findings of the recent work that has begun to explore the potential direct pathogenic role of platelets in established murine models of SSc. We shall describe and critically appraise the findings of previous therapeutic studies evaluating the use of anti-platelet agents in RP and SSc, along with their implications for future therapeutic intervention in these conditions. FAU - Pauling, J D AU - Pauling JD AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases , Bath , UK. FAU - O'Donnell, V B AU - O'Donnell VB FAU - Mchugh, N J AU - Mchugh NJ LA - eng GR - British Heart Foundation/United Kingdom GR - Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20120911 PL - England TA - Platelets JT - Platelets JID - 9208117 SB - IM MH - Animals MH - Blood Platelets/*pathology MH - Humans MH - Raynaud Disease/*blood MH - Scleroderma, Systemic/*blood EDAT- 2012/09/13 06:00 MHDA- 2014/06/25 06:00 CRDT- 2012/09/13 06:00 PHST- 2012/09/13 06:00 [entrez] PHST- 2012/09/13 06:00 [pubmed] PHST- 2014/06/25 06:00 [medline] AID - 10.3109/09537104.2012.719090 [doi] PST - ppublish SO - Platelets. 2013;24(7):503-15. doi: 10.3109/09537104.2012.719090. Epub 2012 Sep 11. PMID- 36356481 OWN - NLM STAT- MEDLINE DCOM- 20221122 LR - 20221129 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 57 DP - 2022 Dec TI - Harnessing the therapeutic potential therapeutic efficacy of α-1 adrenergic blockers for Raynaud's phenomenon and digital ischaemia secondary to central nervous system stimulants used for attention deficit hyperactivity disorder. PG - 152114 LID - S0049-0172(22)00165-2 [pii] LID - 10.1016/j.semarthrit.2022.152114 [doi] FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK; Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. Electronic address: Michael.hughes-6@postgrad.manchester.ac.uk. FAU - Umair, Hafiz M AU - Umair HM AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Sandler, Robert D AU - Sandler RD AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Alunno, Alessia AU - Alunno A AD - Internal Medicine and Nephrology Unit, Department of Life, Health & Environmental Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC, Florence, Italy; Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy. LA - eng PT - Comment PT - Letter DEP - 20221021 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Central Nervous System Stimulants) RN - 0 (Adrenergic Antagonists) SB - IM CON - Semin Arthritis Rheum. 2022 Dec;57:152115. doi: 10.1016/j.semarthrit.2022.152115. PMID: 36343456 MH - Humans MH - *Central Nervous System Stimulants MH - *Attention Deficit Disorder with Hyperactivity/complications/drug therapy MH - *Raynaud Disease/complications/drug therapy MH - Adrenergic Antagonists MH - Ischemia/complications/drug therapy COIS- Declaration of Competing Interest None by any of the authors. EDAT- 2022/11/11 06:00 MHDA- 2022/11/23 06:00 CRDT- 2022/11/10 18:26 PHST- 2022/10/03 00:00 [received] PHST- 2022/10/20 00:00 [accepted] PHST- 2022/11/11 06:00 [pubmed] PHST- 2022/11/23 06:00 [medline] PHST- 2022/11/10 18:26 [entrez] AID - S0049-0172(22)00165-2 [pii] AID - 10.1016/j.semarthrit.2022.152114 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Dec;57:152114. doi: 10.1016/j.semarthrit.2022.152114. Epub 2022 Oct 21. PMID- 17805483 OWN - NLM STAT- MEDLINE DCOM- 20080415 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 3 DP - 2008 Mar TI - Lupus nephritis and Raynaud's phenomenon are significant risk factors for vascular thrombosis in SLE patients with positive antiphospholipid antibodies. PG - 345-51 AB - This study is aimed to determine the predictors of nongravid vascular thrombosis in systemic lupus erythematosus (SLE) patients with positive antiphospholipid antibodies (SLE-aPL). A cohort of 67 SLE-aPL patients who had at least one positive test for lupus anticoagulant (LA), anticardiolipin (aCL), or anti-beta2glycoprotein-1(B2) was examined. Main outcome was the presence of vascular thrombosis. Association between thrombosis and risk factors was examined by contingency table. The odds ratio (OR) of significant predictors was determined by logistic regression. Three percent of patients were LA(+), 6% were aCL(+), 31% were B2(+), 3% were aCL(+)LA(+), 35.8% were aCL(+)B2(+), 7.5% were LA(+)B2(+), and 13.4% were positive for all tests. As for clinical manifestations, 79% had lymphopenia, 76% had lupus nephritis (LN), 41.8% had autoimmune hemolytic anemia, 34.3% had thrombocytopenia, 20.9% had abortion, and 19.4% had Raynaud's phenomenon (RP). Thrombosis occurred in 26 patients. The prevalence of thrombosis for SLE-aPL was 38.8%. Thrombosis was observed more frequently in patients with LA(+) (12 of 18) than the others (14 of 49; p = 0.01). Two-by-two table showed that oral contraceptive and LN were significantly associated with increased risk of thrombosis, while lymphopenia and antimalarials were significantly associated with decreased risk of thrombosis. Multivariate analysis confirmed that LN and RP were associated with increased risk of thrombosis (OR = 6.2 and 3.2; p = 0.005 and 0.008), while lymphopenia and antimalarials were associated with decreased risk of thrombosis (OR = 0.86 and 0.18; p = 0.02 and 0.034). LA is the strongest test to determine the risk of thrombosis in SLE-aPL. The presence of LN and RP strongly predicts thrombosis, while lymphopenia and antimalarials are protective. These findings help to identify patients who may benefit from prophylactic therapy. FAU - Choojitarom, Kittiwan AU - Choojitarom K AD - Division of Allergy-Immunology-Rheumatology, Department of Medicine, Ramathibodi Hospital, 270 Rama6 Road, Bangkok, 10400, Thailand. FAU - Verasertniyom, Orawan AU - Verasertniyom O FAU - Totemchokchyakarn, Kitti AU - Totemchokchyakarn K FAU - Nantiruj, Kanokrat AU - Nantiruj K FAU - Sumethkul, Vasant AU - Sumethkul V FAU - Janwityanujit, Suchela AU - Janwityanujit S LA - eng PT - Journal Article DEP - 20070902 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Contraceptives, Oral) RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Adult MH - Cohort Studies MH - Contraceptives, Oral/adverse effects MH - Female MH - Humans MH - Lupus Coagulation Inhibitor/*blood MH - Lupus Nephritis/blood/*complications MH - Male MH - Odds Ratio MH - Raynaud Disease/blood/*complications MH - Risk Factors MH - Thrombosis/*complications/immunology EDAT- 2007/09/07 09:00 MHDA- 2008/04/16 09:00 CRDT- 2007/09/07 09:00 PHST- 2007/06/05 00:00 [received] PHST- 2007/08/12 00:00 [accepted] PHST- 2007/07/24 00:00 [revised] PHST- 2007/09/07 09:00 [pubmed] PHST- 2008/04/16 09:00 [medline] PHST- 2007/09/07 09:00 [entrez] AID - 10.1007/s10067-007-0721-z [doi] PST - ppublish SO - Clin Rheumatol. 2008 Mar;27(3):345-51. doi: 10.1007/s10067-007-0721-z. Epub 2007 Sep 2. PMID- 17250538 OWN - NLM STAT- MEDLINE DCOM- 20070327 LR - 20131121 IS - 0909-752X (Print) IS - 0909-752X (Linking) VI - 13 IP - 1 DP - 2007 Feb TI - In vivo study of skin mechanical properties in Raynaud's phenomenon. PG - 91-4 AB - BACKGROUND/AIM: Raynaud's phenomenon (RP) is usually the first symptom in patients with systemic sclerosis (SS) and may precede skin changes by several months or years. Non-invasive measurements of skin elasticity are very sensitive and appropriate for objective and quantitative evaluation of sclerodermatous skin. The aim of this study was to investigate and compare the mechanical properties of the skin in patients with primary, secondary, and suspected secondary RP. MATERIALS/METHODS: A total of 63 patients were studied. They were classified as having scleroderma-type nailfold capillary abnormalities--17 with indurative phase of scleroderma (group 1), nine with edematous phase of scleroderma (group 2), 18 with suspected secondary RP (group 3) and as having RP-type nailfold capillary abnormalities, 19 with primary RP (group 4). Thirty-nine sex- and age-matched healthy individuals with normal nailfold capillaroscopy pattern were also studied as controls. Mechanical properties of the skin were evaluated using a non-invasive suction device (Cutometer) equipped with a 2 mm probe. Measurements were performed over five anatomic regions: cheeks, volar forearms, wrists, hands, and proximal phalanx of the fingers. The skin mechanical parameters analyzed were distensibility (Uf), elasticity (Ua/Uf) and viscoelasticity (Uv/Ue). RESULTS: Most demonstrative changes were observed over volar forearms. Patients included in groups 1-3 were characterized by significantly lower Uf and higher Uv/Ue compared with patient group 4 and controls. Patient groups 1 and 2 showed significantly lower Ua/Uf, as well. There were no significant differences in skin mechanical parameters between patient group 4 and control group. CONCLUSION: Mechanical properties of the skin in patients with suspected secondary RP significantly differ from these in patients with primary RP and resemble those in patients with edematous phase of scleroderma. Our findings suggest that the non-invasive measurements of skin elasticity could be helpful in identifying patients with RP at risk of developing SS. FAU - Dobrev, Hristo AU - Dobrev H AD - Department of Dermatology and Venereology, Medical University, Plovdiv, Bulgaria. hristo_dobrev@hotmail.com LA - eng PT - Journal Article PL - England TA - Skin Res Technol JT - Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) JID - 9504453 SB - IM MH - Biomechanical Phenomena/methods MH - Elasticity MH - Female MH - Hardness MH - Hardness Tests MH - Humans MH - Male MH - Raynaud Disease/complications/*physiopathology MH - Scleroderma, Systemic/complications/*physiopathology MH - Skin/*physiopathology MH - Stress, Mechanical MH - Viscosity EDAT- 2007/01/26 09:00 MHDA- 2007/03/28 09:00 CRDT- 2007/01/26 09:00 PHST- 2007/01/26 09:00 [pubmed] PHST- 2007/03/28 09:00 [medline] PHST- 2007/01/26 09:00 [entrez] AID - SRT197 [pii] AID - 10.1111/j.1600-0846.2007.00197.x [doi] PST - ppublish SO - Skin Res Technol. 2007 Feb;13(1):91-4. doi: 10.1111/j.1600-0846.2007.00197.x. PMID- 29292666 OWN - NLM STAT- MEDLINE DCOM- 20190429 LR - 20220409 IS - 1473-4877 (Electronic) IS - 0300-7995 (Linking) VI - 34 IP - 3 DP - 2018 Mar TI - Raynaud's phenomenon and nailfold capillaroscopic findings in anorexia nervosa. PG - 547-550 LID - 10.1080/03007995.2017.1417828 [doi] AB - BACKGROUND: Peripheral vascular abnormalities caused by a dysregulation between peripheral vasoconstriction and vasodilatation, clinically appearing with Raynaud's phenomenon, have been described in anorexia nervosa but specific characteristics of microcirculation in anorexic patients have not yet been studied. METHODS: We performed nailfold videocapillaroscopy to assess microcirculatory alteration in anorexic patients and found three different patterns: normal, aspecific and early scleroderma pattern. We also evaluated several laboratory and clinical parameters to better assess our capillaroscopic findings. RESULTS: None of the clinical parameters examined correlated with specific capillaroscopic findings. An increased risk of autoimmune diseases in eating disorder patients has been described. Our results evidenced an association between early scleroderma capillaroscopic pattern and Raynaud's phenomenon that occurs in anorexia nervosa patients, whereas no significant association was found between all three capillaroscopic patterns and the presence of autoantibodies, as well as ESR and CRP values. CONCLUSIONS: Our study reveals that patients with AN suffering from RP exhibit NVC findings typical of connective tissue diseases. FAU - De Martinis, Massimo AU - De Martinis M AUID- ORCID: 0000-0003-4253-1312 AD - a Department of Life, Health, & Environmental Sciences , University of L'Aquila , Italy. AD - b Allergology and Clinical Immunology Unit, AUSL 04, Teramo , Italy. FAU - Sirufo, Maria Maddalena AU - Sirufo MM AD - a Department of Life, Health, & Environmental Sciences , University of L'Aquila , Italy. AD - b Allergology and Clinical Immunology Unit, AUSL 04, Teramo , Italy. FAU - Ginaldi, Lia AU - Ginaldi L AD - a Department of Life, Health, & Environmental Sciences , University of L'Aquila , Italy. AD - b Allergology and Clinical Immunology Unit, AUSL 04, Teramo , Italy. LA - eng PT - Journal Article DEP - 20180115 PL - England TA - Curr Med Res Opin JT - Current medical research and opinion JID - 0351014 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Anorexia Nervosa/*epidemiology MH - Autoantibodies/immunology MH - Autoimmune Diseases/epidemiology MH - Connective Tissue Diseases/epidemiology MH - Female MH - Humans MH - Male MH - Microcirculation MH - Microscopic Angioscopy/*methods MH - Peripheral Vascular Diseases/epidemiology MH - Raynaud Disease/*epidemiology MH - Young Adult OTO - NOTNLM OT - Anorexia nervosa OT - Raynaud phenomenon OT - capillaroscopy OT - microcirculation OT - translational medicine EDAT- 2018/01/03 06:00 MHDA- 2019/04/30 06:00 CRDT- 2018/01/03 06:00 PHST- 2018/01/03 06:00 [pubmed] PHST- 2019/04/30 06:00 [medline] PHST- 2018/01/03 06:00 [entrez] AID - 10.1080/03007995.2017.1417828 [doi] PST - ppublish SO - Curr Med Res Opin. 2018 Mar;34(3):547-550. doi: 10.1080/03007995.2017.1417828. Epub 2018 Jan 15. PMID- 19107084 OWN - NLM STAT- MEDLINE DCOM- 20090209 LR - 20081224 IS - 0303-464X (Print) IS - 0303-464X (Linking) VI - 33 IP - 4 DP - 2008 Oct-Dec TI - [Nailfold capillaroscopy in children and adolescents with rheumatic diseases]. PG - 395-400 AB - Nailfold capillaroscopy is a simple, noninvasive and inexpensive method which allows a functional and morphological study of the capillary network through direct visualization of the distal row of periungueal capillaries of the fingers. This method has been used as a diagnostic auxiliary in diseases such as scleroderma, dermatomyositis, systemic lupus erythematosus and mixed connective tissue disease. It has also been used to differentiate between active and non active diseases, especially dermatomyositis, and to distinguish between primary and secondary Raynaud's phenomenon. Most reports of nailfold capillaroscopy are qualitative and semi-quantitative. Manuscripts describing quantitative methods (video-capillaroscopy) are scarce, particularly in childhood. The authors did a literature review based on Medline, Lilacs and Pubmed data using the keywords: nailfold capillaroscopy, colagenosis, Raynaud, children and adolescents. FAU - Petry, Daniela G AU - Petry DG AD - Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. FAU - Terreri, Maria T AU - Terreri MT FAU - Len, Cláudio A AU - Len CA FAU - Hilário, Maria O AU - Hilário MO LA - por PT - English Abstract PT - Journal Article PT - Review TT - Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adolescent MH - Child MH - Connective Tissue Diseases/*diagnosis MH - Dermatomyositis/diagnosis MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/diagnosis RF - 45 EDAT- 2008/12/25 09:00 MHDA- 2009/02/10 09:00 CRDT- 2008/12/25 09:00 PHST- 2008/12/25 09:00 [entrez] PHST- 2008/12/25 09:00 [pubmed] PHST- 2009/02/10 09:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2008 Oct-Dec;33(4):395-400. PMID- 27526772 OWN - NLM STAT- MEDLINE DCOM- 20171116 LR - 20250530 IS - 1471-2474 (Electronic) IS - 1471-2474 (Linking) VI - 17 IP - 1 DP - 2016 Aug 15 TI - Absence of Scleroderma pattern at nail fold capillaroscopy valuable in the exclusion of Scleroderma in unselected patients with Raynaud's Phenomenon. PG - 342 LID - 10.1186/s12891-016-1206-5 [doi] LID - 342 AB - BACKGROUND: To report the predictive value of nail-fold capillaroscopy (NFC) patterns of vasculopathy for systemic sclerosis (Scleroderma; SSc) in an unselected cohort of patients with Raynaud's phenomenon (RP). METHODS: Patients referred to a tertiary SSc clinic with RP were evaluated by light/video-NFC. Clinical diagnosis, details and serology were recorded. Primary RP was defined as RP with no features of connective tissue disease (CTD)/antibody. NFC patterns were determined: normal, non-specific, 'early', 'active' or 'late' SSc patterns. Fulfilment of the VEDOSS or 2013 ACR/EULAR criteria for SSc was determined following NFC assessment. RESULTS: Three hundred forty-seven patients were referred: mean (SD) age 47 (15.2) years. On clinical review, 54 (16 %) did not have RP, 69 (20 %) had primary RP, 52 (15 %) had SSc and 172 (50 %) had secondary RP. NFC SSc pattern was detected in 80 (23 %) patients; 37/52 with SSc, 30/172 with secondary RP, 9/69 with primary RP and 4/54 with no RP. For identifying patients who met either the VEDOSS or 2013 ACR/EULAR criteria for SSc, detection of a SSc NFC pattern had a sensitivity of 71 %, specificity 95 %, positive predictive value 84 % and negative predictive value 90 %. CONCLUSIONS: The absence of SSc NFC pattern in patients with RP or suspected CTD is very valuable in the exclusion of SSc. FAU - Bissell, Lesley-Anne AU - Bissell LA AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2nd Floor, Chapel Allerton Hospital, Chapeltown Road, Leeds, LS7 4SA, UK. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Abignano, Giuseppina AU - Abignano G AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2nd Floor, Chapel Allerton Hospital, Chapeltown Road, Leeds, LS7 4SA, UK. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Emery, Paul AU - Emery P AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2nd Floor, Chapel Allerton Hospital, Chapeltown Road, Leeds, LS7 4SA, UK. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2nd Floor, Chapel Allerton Hospital, Chapeltown Road, Leeds, LS7 4SA, UK. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Buch, Maya H AU - Buch MH AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2nd Floor, Chapel Allerton Hospital, Chapeltown Road, Leeds, LS7 4SA, UK. m.buch@leeds.ac.uk. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. m.buch@leeds.ac.uk. LA - eng GR - CDF-2014-07-051/DH_/Department of Health/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160815 PL - England TA - BMC Musculoskelet Disord JT - BMC musculoskeletal disorders JID - 100968565 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*diagnostic imaging MH - Patient Selection MH - Predictive Value of Tests MH - Raynaud Disease/*diagnostic imaging MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnostic imaging MH - Sensitivity and Specificity PMC - PMC4986250 OTO - NOTNLM OT - Nail-fold capillaroscopy OT - Raynaud’s Phenomenon OT - Systemic sclerosis EDAT- 2016/08/17 06:00 MHDA- 2017/11/29 06:00 PMCR- 2016/08/15 CRDT- 2016/08/17 06:00 PHST- 2016/05/18 00:00 [received] PHST- 2016/08/05 00:00 [accepted] PHST- 2016/08/17 06:00 [entrez] PHST- 2016/08/17 06:00 [pubmed] PHST- 2017/11/29 06:00 [medline] PHST- 2016/08/15 00:00 [pmc-release] AID - 10.1186/s12891-016-1206-5 [pii] AID - 1206 [pii] AID - 10.1186/s12891-016-1206-5 [doi] PST - epublish SO - BMC Musculoskelet Disord. 2016 Aug 15;17(1):342. doi: 10.1186/s12891-016-1206-5. PMID- 19594214 OWN - NLM STAT- MEDLINE DCOM- 20090925 LR - 20151119 IS - 1080-6032 (Print) IS - 1080-6032 (Linking) VI - 20 IP - 2 DP - 2009 Summer TI - Can people with Raynaud's phenomenon travel to high altitude? PG - 129-38 LID - 10.1580/08-WEME-OR-260R1.1 [doi] AB - OBJECTIVE: To determine whether high altitude travel adversely affects mountain enthusiasts with Raynaud's phenomenon. METHODS: Volunteers with Raynaud's phenomenon were recruited using announcements disseminated by organizations dedicated to climbing or wilderness travel and Internet discussion boards dedicated to mountain activities to complete an online, anonymous survey. Survey questions addressed demographic variables, aspects of their Raynaud's phenomenon, and features of their mountain activities. Respondents compared experiences with Raynaud's phenomenon between high (>2440 m; 8000 feet) and low elevations and rated agreement with statements concerning their disease and the effects of high altitude. RESULTS: One hundred forty-two people, 98% of whom had primary Raynaud's phenomenon, completed the questionnaire. Respondents spent 5 to 7 days per month at elevations above 2440 m and engaged in 5.4 +/- 2.0 different activities. Eighty-nine percent of respondents engaged in winter sports and only 22% reported changing their mountain activities because of Raynaud's phenomenon. Respondents reported a variety of tactics to prevent and treat Raynaud's attacks, but only 12% used prophylactic medications. Fifteen percent of respondents reported an episode of frostbite following a Raynaud's phenomenon attack at high altitude. There was considerable heterogeneity in participants' perceptions of the frequency, duration, and severity of attacks at high altitude compared to their home elevation. CONCLUSIONS: Motivated individuals with primary Raynaud's phenomenon, employing various prevention and treatment strategies, can engage in different activities, including winter sports, at altitudes above 2440 m. Frostbite may be common in this population at high altitude, and care must be taken to prevent its occurrence. FAU - Luks, Andrew M AU - Luks AM AD - Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA 98104, USA. aluks@u.washington.edu FAU - Grissom, Colin K AU - Grissom CK FAU - Jean, Dominique AU - Jean D FAU - Swenson, Erik R AU - Swenson ER LA - eng PT - Journal Article PL - United States TA - Wilderness Environ Med JT - Wilderness & environmental medicine JID - 9505185 RN - 0 (Vasodilator Agents) SB - IM MH - Adult MH - *Altitude MH - Extremities/*blood supply MH - Female MH - Frostbite/*epidemiology/prevention & control MH - Humans MH - Male MH - *Mountaineering MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow MH - Seasons MH - Surveys and Questionnaires MH - Travel MH - Vasodilator Agents/therapeutic use EDAT- 2009/07/15 09:00 MHDA- 2009/09/26 06:00 CRDT- 2009/07/15 09:00 PHST- 2009/07/15 09:00 [entrez] PHST- 2009/07/15 09:00 [pubmed] PHST- 2009/09/26 06:00 [medline] AID - 1080-6032-20-2-129 [pii] AID - 10.1580/08-WEME-OR-260R1.1 [doi] PST - ppublish SO - Wilderness Environ Med. 2009 Summer;20(2):129-38. doi: 10.1580/08-WEME-OR-260R1.1. PMID- 37419757 OWN - NLM STAT- MEDLINE DCOM- 20231216 LR - 20250721 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 37 IP - 1 DP - 2023 Mar TI - Nailfold capillaroscopy. PG - 101849 LID - S1521-6942(23)00035-9 [pii] LID - 10.1016/j.berh.2023.101849 [doi] AB - Nailfold capillaroscopy is a safe and well-established method for the assessment of structural alterations of the microcirculation. It is a crucial tool in the investigation and monitoring of patients presenting with Raynaud's phenomenon. Detection of the characteristic "scleroderma pattern" on capillaroscopy may indicate an underlying rheumatic disease, particularly systemic sclerosis (SSc). Herein, we highlight the practical aspects of videocapillaroscopy, including image acquisition and analysis, with mention of dermoscopy. Special emphasis is placed on standardized use of terminology to describe capillary characteristics. Systematic evaluation of images in discerning the normal from the abnormal using the validated European Alliance of Associations for Rheumatology (EULAR) Study Group consensus reporting framework is paramount. In addition to the relevance of capillaroscopy in the (very) early diagnosis of SSc, its emerging predictive value (especially capillary loss) for new organ involvement and disease progression is underscored. We further provide capillaroscopic findings in selected other rheumatic diseases. CI - Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. Electronic address: Vanessa.Smith@ugent.be. FAU - Ickinger, Claudia AU - Ickinger C AD - Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. FAU - Hysa, Elvis AU - Hysa E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy - IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Snow, Marcus AU - Snow M AD - Department of Internal Medicine, Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE, USA. FAU - Frech, Tracy AU - Frech T AD - Internal Medicine, Vanderbilt University Medical Center and Tennessee Valley Healthcare System Nashville, TN, USA. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy - IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy - IRCCS San Martino Polyclinic, Genoa, Italy. LA - eng GR - I01 CX001183/CX/CSRD VA/United States GR - I01 CX002111/CX/CSRD VA/United States PT - Journal Article PT - Review DEP - 20230706 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Humans MH - Microscopic Angioscopy/methods MH - Capillaries/diagnostic imaging MH - *Rheumatic Diseases/diagnostic imaging MH - *Scleroderma, Systemic/diagnostic imaging MH - *Rheumatology MH - *Raynaud Disease/diagnostic imaging MH - Nails/diagnostic imaging/blood supply OTO - NOTNLM OT - Connective tissue diseases OT - EULAR study group on microcirculation in rheumatic diseases OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - Scleroderma pattern OT - Systemic sclerosis EDAT- 2023/07/08 10:41 MHDA- 2023/12/17 09:43 CRDT- 2023/07/07 22:00 PHST- 2023/05/25 00:00 [received] PHST- 2023/06/19 00:00 [accepted] PHST- 2023/12/17 09:43 [medline] PHST- 2023/07/08 10:41 [pubmed] PHST- 2023/07/07 22:00 [entrez] AID - S1521-6942(23)00035-9 [pii] AID - 10.1016/j.berh.2023.101849 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2023 Mar;37(1):101849. doi: 10.1016/j.berh.2023.101849. Epub 2023 Jul 6. PMID- 26259425 OWN - NLM STAT- MEDLINE DCOM- 20150908 LR - 20150811 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 62 IP - 12 DP - 2014 Dec TI - Systemic Sclerosis Sine Scleroderma. PG - 54-6 AB - Systemic sclerosis sine scleroderma is a rare form of limited systemic sclerosis. These patients are without skin involvement, but do not differ in its clinical or laboratory features and prognosis from classical systemic sclerosis. In the absence of cutaneous signs/symptoms, its diagnosis is delayed leading to significant morbidity and mortality. We report a case of sixty year old female who presented to us with dyspnoea on exertion and Raynaud's phenomenon. She was investigated and was found to have this disorder with pulmonary artery hypertension. FAU - Manoria, Piyush AU - Manoria P FAU - Joshi, Prakash AU - Joshi P FAU - Sharma, Padmnabh AU - Sharma P FAU - Jha, R K AU - Jha RK LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Dyspnea/etiology MH - Female MH - Humans MH - Hypertension, Pulmonary/etiology MH - Middle Aged MH - Raynaud Disease/etiology MH - Scleroderma, Limited/complications/diagnosis/*pathology EDAT- 2015/08/12 06:00 MHDA- 2015/09/09 06:00 CRDT- 2015/08/12 06:00 PHST- 2015/08/12 06:00 [entrez] PHST- 2015/08/12 06:00 [pubmed] PHST- 2015/09/09 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2014 Dec;62(12):54-6. PMID- 26222712 OWN - NLM STAT- MEDLINE DCOM- 20160519 LR - 20150812 IS - 1557-8992 (Electronic) IS - 1044-5463 (Linking) VI - 25 IP - 6 DP - 2015 Aug TI - OROS-Methylphenidate-Induced Raynaud's Phenomenon: A Dose-Related Side Effect. PG - 521-2 LID - 10.1089/cap.2015.0033 [doi] FAU - Bayram, Özlem AU - Bayram Ö AD - Department of Child and Adolescent Psychiatry, Meram Faculty of Medicine, Necmettin Erbakan University , Meram, Konya, Turkey . FAU - Hergüner, Sabri AU - Hergüner S AD - Department of Child and Adolescent Psychiatry, Meram Faculty of Medicine, Necmettin Erbakan University , Meram, Konya, Turkey . LA - eng PT - Case Reports PT - Journal Article DEP - 20150729 PL - United States TA - J Child Adolesc Psychopharmacol JT - Journal of child and adolescent psychopharmacology JID - 9105358 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) SB - IM MH - Adolescent MH - Attention Deficit Disorder with Hyperactivity/drug therapy MH - Central Nervous System Stimulants/administration & dosage/*adverse effects MH - Dose-Response Relationship, Drug MH - Drug Delivery Systems MH - Female MH - Humans MH - Methylphenidate/administration & dosage/*adverse effects MH - Raynaud Disease/*chemically induced EDAT- 2015/07/30 06:00 MHDA- 2016/05/20 06:00 CRDT- 2015/07/30 06:00 PHST- 2015/07/30 06:00 [entrez] PHST- 2015/07/30 06:00 [pubmed] PHST- 2016/05/20 06:00 [medline] AID - 10.1089/cap.2015.0033 [doi] PST - ppublish SO - J Child Adolesc Psychopharmacol. 2015 Aug;25(6):521-2. doi: 10.1089/cap.2015.0033. Epub 2015 Jul 29. PMID- 24664962 OWN - NLM STAT- MEDLINE DCOM- 20140919 LR - 20211021 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 66 IP - 8 DP - 2014 Aug TI - Raynaud's phenomenon, inflammatory arthritis, and weight loss: pay attention to the man behind the curtain. PG - 1263-8 LID - 10.1002/acr.22331 [doi] FAU - Stojan, George AU - Stojan G AD - Harvard Medical School, Boston, Massachusetts, and Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Illei, Peter B AU - Illei PB FAU - Yung, Rex C AU - Yung RC FAU - Gelber, Allan C AU - Gelber AC LA - eng GR - T32 AR048522/AR/NIAMS NIH HHS/United States GR - T32-AR048522/AR/NIAMS NIH HHS/United States PT - Clinical Conference PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM CIN - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453. doi: 10.1002/acr.22489. PMID: 25303107 CIN - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453-4. doi: 10.1002/acr.22491. PMID: 25303330 MH - Arthritis, Rheumatoid/*etiology MH - Carcinoma, Non-Small-Cell Lung/complications/*diagnosis MH - Female MH - Humans MH - Lung Neoplasms/complications/*diagnosis MH - Middle Aged MH - Raynaud Disease/*etiology MH - *Weight Loss PMC - PMC4153596 MID - NIHMS613199 COIS- Drs.Stojan, Illei, Yung and Gelber have no conflicts of interest to disclose. EDAT- 2014/03/26 06:00 MHDA- 2014/09/23 06:00 PMCR- 2015/08/01 CRDT- 2014/03/26 06:00 PHST- 2013/10/08 00:00 [received] PHST- 2014/03/18 00:00 [accepted] PHST- 2014/03/26 06:00 [entrez] PHST- 2014/03/26 06:00 [pubmed] PHST- 2014/09/23 06:00 [medline] PHST- 2015/08/01 00:00 [pmc-release] AID - 10.1002/acr.22331 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2014 Aug;66(8):1263-8. doi: 10.1002/acr.22331. PMID- 19041079 OWN - NLM STAT- MEDLINE DCOM- 20090212 LR - 20081201 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 22 IP - 6 DP - 2008 Dec TI - Capillaroscopy. PG - 1093-108 LID - 10.1016/j.berh.2008.09.001 [doi] AB - Capillaroscopy is the most reliable way to distinguish between primary and secondary Raynaud's phenomenon (RP) through identification of an early pattern of systemic sclerosis (SSc). The presence of giant capillaries and microhaemorrhages on nailfold videocapillaroscopy (NVC) is sufficient to identify the scleroderma pattern (early), and an increase in these features and the addition of loss of capillaries (active pattern) is followed by neo-angiogenesis, fibrosis and 'desertification' (late pattern). The sensitivity of the American College of Rheumatology's classification criteria for SSc increases from 67% to 99% with the addition of these specific NVC abnormalities. Based on the appearance of the scleroderma pattern on NVC, almost 15% of patients shift from primary to secondary RP over a mean follow-up period of 29.4+/-10 months. Follow-up by NVC (every 6 months) is suggested for RP patients. A scoring system for NVC changes is available, and scores change significantly during follow-up of SSc patients. Several other NVC patterns have also been identified, such as in dermatomyosistis, systemic lupus eythaematosus, mixed connective tissue disease and antiphospholipid syndrome. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Clinical Academic Unit of Rheumatology, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. mcutolo@unige.it FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Secchi, Maria Elena AU - Secchi ME FAU - Sulli, Alberto AU - Sulli A LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Capillaries/*pathology MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/diagnosis/physiopathology MH - Scleroderma, Systemic/*diagnosis/physiopathology RF - 44 EDAT- 2008/12/02 09:00 MHDA- 2009/02/13 09:00 CRDT- 2008/12/02 09:00 PHST- 2008/12/02 09:00 [pubmed] PHST- 2009/02/13 09:00 [medline] PHST- 2008/12/02 09:00 [entrez] AID - S1521-6942(08)00098-3 [pii] AID - 10.1016/j.berh.2008.09.001 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2008 Dec;22(6):1093-108. doi: 10.1016/j.berh.2008.09.001. PMID- 30277867 OWN - NLM STAT- MEDLINE DCOM- 20190129 LR - 20231012 IS - 0392-856X (Print) IS - 1593-098X (Electronic) IS - 0392-856X (Linking) VI - 36 Suppl 113 IP - 4 DP - 2018 Jul-Aug TI - Implications of endothelial shear stress on systemic sclerosis vasculopathy and treatment. PG - 175-182 LID - 10.55563/clinexprheumatol/7ulgbj [doi] AB - There are no Federal Drug Administration approved drugs for the treatment of systemic sclerosis vascular digital ulcers (DU) in the United States, which are thought to be an end-stage result of prolonged ischaemia due to severe, prolonged Raynaud's phenomenon. Most therapeutics for vasodilation used in SSc work different pathways to target the smooth muscle to induce vessel relaxation. Longitudinal studies of vascular function allow insight into the effects of medications used for Raynaud's phenomenon in the SSc patient population. In this review, we discuss vascular tone, the function of the endothelium in SSc, and provide the rationale for longitudinal studies of vascular function and therapeutics that target the endothelial shear stress in addition to vasodilation for treatment and prevention of DU. This review provides the rationale for vasodilatory medication use for treatment of SSc-related DU and justifies access to non-FDA approved medications for this indication. FAU - Frech, Tracy M AU - Frech TM AD - University of Utah, Department of Internal Medicine; University of Utah Hospitals and Clinics; and VAMC Salt Lake City, GRECC, Salt Lake City, UT, USA. tracy.frech@hsc.utah.edu. FAU - Machin, Daniel R AU - Machin DR AD - University of Utah, Department of Internal Medicine, Salt Lake City, UT, USA. FAU - Murtaugh, Maureen A AU - Murtaugh MA AD - University of Utah, Department of Internal Medicine, Salt Lake City, UT, USA. FAU - Stoddard, Gregory J AU - Stoddard GJ AD - University of Utah, Department of Internal Medicine, Salt Lake City, UT, USA. FAU - Bloom, Samuel I AU - Bloom SI AD - University of Utah, Department of Nutrition and Integrative Physiology, University of Utah, Salt Lake City, UT, USA. FAU - Phibbs, Jessica V AU - Phibbs JV AD - University of Utah Hospitals and Clinics, Salt Lake City, UT, USA. FAU - Donato, Anthony J AU - Donato AJ AD - University of Utah, Department of Internal Medicine; University of Utah, Department of Exercise and Sport Science; University of Utah, Dept. of Nutrition and Integrative Physiology, University of Utah; and VAMC Salt Lake City, GRECC, Salt Lake City, USA. LA - eng GR - I01 CX001183/CX/CSRD VA/United States GR - K02 AG045339/AG/NIA NIH HHS/United States GR - K23 AR067889/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Review DEP - 20180920 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vasodilator Agents) SB - IM MH - Animals MH - Endothelium, Vascular/drug effects/*physiopathology MH - Hand/*blood supply MH - Humans MH - *Microcirculation/drug effects MH - Raynaud Disease/diagnosis/drug therapy/etiology/*physiopathology MH - Scleroderma, Systemic/complications/diagnosis/drug therapy/*physiopathology MH - Skin Ulcer/diagnosis/drug therapy/etiology/*physiopathology MH - Stress, Mechanical MH - Treatment Outcome MH - *Vasodilation/drug effects MH - Vasodilator Agents/therapeutic use PMC - PMC6542469 MID - NIHMS996636 COIS- Competing interests: none declared. EDAT- 2018/10/03 06:00 MHDA- 2019/01/30 06:00 PMCR- 2019/09/20 CRDT- 2018/10/03 06:00 PHST- 2018/05/17 00:00 [received] PHST- 2018/07/30 00:00 [accepted] PHST- 2018/10/03 06:00 [entrez] PHST- 2018/10/03 06:00 [pubmed] PHST- 2019/01/30 06:00 [medline] PHST- 2019/09/20 00:00 [pmc-release] AID - 12999 [pii] AID - 10.55563/clinexprheumatol/7ulgbj [doi] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jul-Aug;36 Suppl 113(4):175-182. doi: 10.55563/clinexprheumatol/7ulgbj. Epub 2018 Sep 20. PMID- 40183325 OWN - NLM STAT- MEDLINE DCOM- 20250804 LR - 20250806 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 8 DP - 2025 Aug TI - Primary versus systemic sclerosis-associated Raynaud's phenomenon: relationship with clinical and environmental factors. PG - 1380-1385 LID - 10.55563/clinexprheumatol/m7qpn3 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) can be induced by stress and environmental factors, occurring as a primary disease (pRP) or associated with connective tissue disease. RP is seen in more than 95% of patients with systemic sclerosis (SSc) and may precede its diagnosis by several years. Accordingly, there is a clear need to identify those patients with RP who will eventually develop connective tissue disease, including SSc. The aim of this case-control study was to assess the association of SSc-RP versus pRP with respect to environmental factors, lifestyle habits, and clinical setting. METHODS: A questionnaire was used to collect current data from 180 patients with SSc-RP and 103 with pRP. Statistical analyses were performed to identify possible risk factors for SSc-RP. RESULTS: SSc-RP was found to be inversely associated with living in urban area (OR=0.37; p<0.001), computer use (OR=0.38, p<0.001), contraceptive use (OR=0.32; p=0.017), habitual alcohol use (OR=0.35; p=0.029), and hepatitis B virus vaccine (OR=0.09; p=0.011),while it was directly associated to cold sensitivity (OR=3.48; p=0.001), lower quality of life (OR=2.69; p<0.001), finger pain (OR=3.03; p<0.001) and autoimmune hypothyroidism (OR=3.62; p=0.007). All associations were supported by either multivariate and/or multivariable analyses. CONCLUSIONS: This study revealed differences in lifestyle and preventive health behaviours between SSc-RP and pRP, and also suggests that patients with pRP and autoimmune hypothyroidism should be strictly monitored for any clinical changes that may indicate SSc onset. Further investigations are needed to prospectively evaluate autoimmune hypothyroidism as a predisposing condition for SSc-RP. FAU - Favoino, Elvira AU - Favoino E AD - Laboratory of Cellular and Molecular Immunology, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. elvira.favoino@uniba.it. FAU - Prete, Marcella AU - Prete M AD - Internal Medicine Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Rheumatology Section, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Sisto, Adriana AU - Sisto A AD - Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. FAU - Corrado, Ada AU - Corrado A AD - Rheumatology Unit, Department of Medical and Surgery Sciences, University of Foggia, Italy. FAU - Leone, Patrizia AU - Leone P AD - Internal Medicine Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. FAU - Lisco, Giuseppe AU - Lisco G AD - Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. FAU - Vomero, Marta AU - Vomero M AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome; and Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Biancalana, Edoardo AU - Biancalana E AD - Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Chiara, Emanuele AU - Chiara E AD - Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Emmi, Giacomo AU - Emmi G AD - Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Racanelli, Vito AU - Racanelli V AD - Centre for Medical Sciences, University of Trento and Internal Medicine Division, Santa Chiara Hospital, Provincial Health Care Agency (APSS), Trento, Italy. FAU - Marcoccia, Antonella AU - Marcoccia A AD - Department of Medical Area, Vascular Disease and Immunology Unit, Sandro Pertini Hospital, Rome, Italy. FAU - Grembiale, Rosa Daniela AU - Grembiale RD AD - Rheumatology Research Unit, Department of Health Sciences, University of Catanzaro Magna Graecia, Catanzaro, Italy. FAU - Cantatore, Francesco Paolo AU - Cantatore FP AD - Rheumatology Unit, Department of Medical and Surgery Sciences, University of Foggia, Italy. FAU - Navarini, Luca AU - Navarini L AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome; and Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, Italy. FAU - Ciccia, Francesco AU - Ciccia F AD - Rheumatology Section, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome; and Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Perosa, Federico AU - Perosa F AD - Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. federico.perosa@uniba.it. LA - eng PT - Comparative Study PT - Journal Article DEP - 20250401 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Case-Control Studies MH - Life Style MH - Logistic Models MH - Multivariate Analysis MH - Odds Ratio MH - Quality of Life MH - *Raynaud Disease/etiology/diagnosis/epidemiology MH - Risk Factors MH - *Scleroderma, Systemic/diagnosis/epidemiology/complications MH - Surveys and Questionnaires EDAT- 2025/04/04 12:30 MHDA- 2025/08/04 12:27 CRDT- 2025/04/04 07:03 PHST- 2024/07/25 00:00 [received] PHST- 2024/11/15 00:00 [accepted] PHST- 2025/08/04 12:27 [medline] PHST- 2025/04/04 12:30 [pubmed] PHST- 2025/04/04 07:03 [entrez] AID - 21465 [pii] AID - 10.55563/clinexprheumatol/m7qpn3 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Aug;43(8):1380-1385. doi: 10.55563/clinexprheumatol/m7qpn3. Epub 2025 Apr 1. PMID- 18847172 OWN - NLM STAT- MEDLINE DCOM- 20081104 LR - 20081013 IS - 1565-1088 (Print) VI - 10 IP - 8-9 DP - 2008 Aug-Sep TI - Diagnostic workup for mixed connective tissue disease in childhood. PG - 650-2 AB - Raynaud's phenomenon, fatigue and pain (myalgia and arthralgia) are important presenting symptoms of pediatric-onset mixed connective tissue disease. The difficulty is that many adolescent girls complain of pain along with fatigue without evidence for serious disease. However, in patients with Raynaud's phenomenon one should search for evidence of connective tissue diseases. Capillaroscopy could be helpful since capillary changes of the SD-type significantly correlate with future development of scleroderma spectrum disorders. Symptoms of MCTD change in most patients during the disease course: in general the inflammatory features that are also seen in systemic lupus erythematosus and juvenile dermatomyositis have the tendency to disappear over years, but Raynaud's phenomenon is persistent and scleroderma symptoms become progressively prominent. Long-lasting remission occurs only in a minority of patients, while the majority has mild disease activity. Mortality in children with MCTD is lower than in adults. Since a change of symptoms is in the nature of the disease, a thorough and frequent evaluation of children with (probable) MCTD is important to detect organ involvement, which should be treated at an early (pre-symptomatic) stage. We present a diagnostic workup scheme for children and adolescents with propable MCTD. FAU - Swart, Joost F AU - Swart JF AD - VU University Medical Center, Amsterdam, The Netherlands. swart@vumc.nl FAU - Wulffraat, Nico M AU - Wulffraat NM LA - eng PT - Journal Article PT - Review PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 SB - IM MH - Adolescent MH - Child MH - Fatigue/complications MH - Humans MH - Mixed Connective Tissue Disease/complications/*diagnosis MH - Pain/complications MH - Raynaud Disease/complications RF - 18 EDAT- 2008/10/14 09:00 MHDA- 2008/11/05 09:00 CRDT- 2008/10/14 09:00 PHST- 2008/10/14 09:00 [pubmed] PHST- 2008/11/05 09:00 [medline] PHST- 2008/10/14 09:00 [entrez] PST - ppublish SO - Isr Med Assoc J. 2008 Aug-Sep;10(8-9):650-2. PMID- 18246378 OWN - NLM STAT- MEDLINE DCOM- 20081031 LR - 20211020 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 6 DP - 2008 Jun TI - The diagnostic accuracy of power Doppler ultrasonography for differentiating secondary from primary Raynaud's phenomenon in undifferentiated connective tissue disease. PG - 783-6 LID - 10.1007/s10067-008-0851-y [doi] AB - This study was conducted to evaluate the diagnostic accuracy of power Doppler ultrasonography (PDU) for differentiating secondary from primary Raynaud's phenomenon (RP), and also compared PDU with nailfold capillaroscopy (NFC) for the assessment of microvascularity in undifferentiated connective tissue disease (UCTD) patients with RP. Microvascularity in the nailfold and finger tip was evaluated using PDU with cold challenge, and the findings of PDU were classified according to the qualitative grading system before and after cold challenge. NFC was performed at the same day in all persons. The results of PDU were compared with the clinical, laboratory data, and the findings of NFC. The 14 UCTD patients with RP were included in our study. Seven patients were suspected of secondary RP in NFC examination, thus NFC yielded a correct classification into secondary RP in 50% of the UCTD patients. The PDU finding of pattern II, which is regarded as the finding of secondary RP, was observed in 12 UCTD patients with RP. Thus, PDU yielded a correct classification into secondary RP in 86% of UCTD patients. In conclusion, we confirmed that PDU has a higher correct classification rate than NFC for the diagnosing of secondary RP in UCTD patients. Our results suggest that PDU has better accuracy than NFC in differentiating secondary from primary RP, and PDU is more useful in assessing microvascular abnormalities in UCTD patients with RP. FAU - Kim, Sang-Hyon AU - Kim SH AD - Department of Internal Medicine, Chosun University College of Medicine, Gwangju, Republic of Korea. FAU - Kim, Hyun-Ok AU - Kim HO FAU - Jeong, Yong-Geun AU - Jeong YG FAU - Lee, Sang Yong AU - Lee SY FAU - Yoo, Wan-Hee AU - Yoo WH FAU - Choi, Tae Hyun AU - Choi TH FAU - Lee, Sang-Il AU - Lee SI LA - eng PT - Clinical Trial PT - Journal Article PT - Validation Study DEP - 20080202 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Capillaries/diagnostic imaging MH - Connective Tissue Diseases/*diagnostic imaging MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nails/blood supply/diagnostic imaging MH - Raynaud Disease/*diagnostic imaging MH - Reproducibility of Results MH - Ultrasonography, Doppler/*methods/*standards EDAT- 2008/02/05 09:00 MHDA- 2008/11/01 09:00 CRDT- 2008/02/05 09:00 PHST- 2008/01/06 00:00 [received] PHST- 2008/01/15 00:00 [accepted] PHST- 2008/02/05 09:00 [pubmed] PHST- 2008/11/01 09:00 [medline] PHST- 2008/02/05 09:00 [entrez] AID - 10.1007/s10067-008-0851-y [doi] PST - ppublish SO - Clin Rheumatol. 2008 Jun;27(6):783-6. doi: 10.1007/s10067-008-0851-y. Epub 2008 Feb 2. PMID- 19547979 OWN - NLM STAT- MEDLINE DCOM- 20091028 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 11 DP - 2009 Sep TI - The role of capillaroscopy in differentiation of primary and secondary Raynaud's phenomenon in rheumatic diseases: a review of the literature and two case reports. PG - 1263-71 LID - 10.1007/s00296-009-1019-z [doi] AB - The purpose of this study is to study and systematize the current knowledge about the role of capillaroscopy in differentiation of primary and secondary Raynaud's phenomenon (RP) in rheumatic diseases. This method is a review of the literature. Capillaroscopy is of crucial value for diagnosis and differentiation of primary and secondary RP in rheumatic diseases. The appearance of abnormal capillaroscopic pattern inherits high positive predictive value for the development of systemic rheumatic disease. The most specific pattern is found in systemic sclerosis (SSc), so called "scleroderma pattern", which is characterized by the presence of dilated capillaries, hemorrhages, avascular areas and neoangiogeneis. It is found in more than 90% of patients with overt SSc. Similar changes are found in patients with dermatomyositis, mixed connective tissue disease, undifferentiated connective tissue disease and they are called "scleroderma-like pattern". Absence of abnormal capillaroscopic findings can be regarded as a diagnostic criterion for primary RP. Inclusion of pathologic capillaroscopic pattern may increase the sensitivity of ACR classification criteria for SSc. In conclusion, capillaroscopy is of crucial importance for the differentiation of primary and secondary RP in rheumatic diseases, and also in differentiation between different forms of connective tissue diseases as well as for their early diagnosis. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Medical University, Clinic of Rheumatology, Plovdiv, Bulgaria. sevdalina_n@abv.bg FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20090623 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Connective Tissue Diseases/diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/complications MH - Male MH - Microcirculation MH - Microscopic Angioscopy/instrumentation/*methods MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - Rheumatic Diseases/*complications MH - Scleroderma, Systemic/complications MH - Sjogren's Syndrome/complications MH - Skin/blood supply RF - 80 EDAT- 2009/06/24 09:00 MHDA- 2009/10/29 06:00 CRDT- 2009/06/24 09:00 PHST- 2008/08/23 00:00 [received] PHST- 2009/06/05 00:00 [accepted] PHST- 2009/06/24 09:00 [entrez] PHST- 2009/06/24 09:00 [pubmed] PHST- 2009/10/29 06:00 [medline] AID - 10.1007/s00296-009-1019-z [doi] PST - ppublish SO - Rheumatol Int. 2009 Sep;29(11):1263-71. doi: 10.1007/s00296-009-1019-z. Epub 2009 Jun 23. PMID- 41972282 OWN - NLM STAT- MEDLINE DCOM- 20260413 LR - 20260413 IS - 1464-5157 (Electronic) IS - 0265-6736 (Linking) VI - 43 IP - 1 DP - 2026 Dec TI - Effects of locally applied water-filtered infrared a irradiation adjunctive to iloprost and carbon dioxide hand baths in patients with systemic sclerosis and severe Raynaud's phenomenon - a randomized controlled trial. PG - 2653012 LID - 10.1080/02656736.2026.2653012 [doi] AB - BACKGROUND: Almost all patients with systemic sclerosis (SSc) experience Raynaud's phenomenon (RP) with risk of digital ulcers (DU). In severe RP, intravenous iloprost is standard, yet additive hyperthermic therapy may enhance vasodilation and microcirculation. However, whether more intensive or alternative hyperthermic modalities offer benefits beyond commonly used CO(2) hand baths remains unclear. METHODS: In this randomized, controlled trial, all patients received a baseline therapy of iloprost plus CO(2) hand baths. The intervention group (IG) received additional water-filtered infrared-A (wIRA; 2 × 30 min/day, 8 days), while the control group (CG) received baseline therapy alone. The primary endpoint was the difference in RP-associated pain (VAS 0-100 mm) after treatment. Secondary outcomes included changes in RP duration, frequency, and intensity, Health Assessment Questionnaire (HAQ), serum interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). RESULTS: Of 46 randomized patients, 38 (IG = 19, CG = 19) completed the study. Pain decreased from 70.4 ± 27.8 to 56.7 ± 21.4 mm with wIRA and from 73.9 ± 27.5 to 65.3 ± 26.8 mm in controls. Multivariable ANCOVA showed a mean difference of -14.7, 95% CI [-28.8;-0.7], p = 0.041 in favor of wIRA. RP duration improved more in the wIRA group (day 23: β = -3.8, 95% CI [-7.4;-0.2], p = 0.041), while RP frequency, intensity, HAQ, IL-6, and VEGF showed no significant differences (all p > 0.05). No adverse events occurred. CONCLUSIONS: Adjunctive wIRA during iloprost therapy was safe and yielded modest, short-term improvements in pain and RP duration. These exploratory effects warrant confirmation in larger controlled trials. FAU - Schulz, Nils AU - Schulz N AUID- ORCID: 0009-0000-0378-3766 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Boettger, Priyanka AU - Boettger P AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. AD - Department of Cardiology, Justus Liebig University Giessen, Giessen, Germany. FAU - Bär, Joachim AU - Bär J AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Wilhelmi, Tim AU - Wilhelmi T AUID- ORCID: 0009-0008-2400-2840 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - van Wijnen, Pascal AU - van Wijnen P AUID- ORCID: 0009-0007-2629-1837 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AUID- ORCID: 0009-0000-8832-8112 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Lange, Uwe AU - Lange U AUID- ORCID: 0000-0001-7904-0619 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Klemm, Philipp AU - Klemm P AUID- ORCID: 0000-0001-7911-4235 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Justus Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20260413 PL - England TA - Int J Hyperthermia JT - International journal of hyperthermia : the official journal of European Society for Hyperthermic Oncology, North American Hyperthermia Group JID - 8508395 RN - JED5K35YGL (Iloprost) RN - 142M471B3J (Carbon Dioxide) SB - IM MH - Humans MH - *Iloprost/therapeutic use/pharmacology MH - Female MH - *Raynaud Disease/therapy/drug therapy MH - *Scleroderma, Systemic/therapy/drug therapy MH - Male MH - Middle Aged MH - *Carbon Dioxide/therapeutic use/pharmacology MH - *Infrared Rays/therapeutic use MH - Aged MH - Adult MH - Hand MH - Baths OTO - NOTNLM OT - Circulation OT - Raynaud’s phenomenon OT - hyperthermia OT - systemic sclerosis OT - water-filtered infrared A irradiation EDAT- 2026/04/13 12:37 MHDA- 2026/04/13 12:38 CRDT- 2026/04/13 06:49 PHST- 2026/04/13 12:38 [medline] PHST- 2026/04/13 12:37 [pubmed] PHST- 2026/04/13 06:49 [entrez] AID - 10.1080/02656736.2026.2653012 [doi] PST - ppublish SO - Int J Hyperthermia. 2026 Dec;43(1):2653012. doi: 10.1080/02656736.2026.2653012. Epub 2026 Apr 13. PMID- 41186316 OWN - NLM STAT- MEDLINE DCOM- 20260305 LR - 20260311 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 55 IP - 2 DP - 2026 Mar TI - Signalling profile of circulating leucocytes of subjects with Raynaud's phenomenon in relation to systemic sclerosis: a pilot study. PG - 115-122 LID - 10.1080/03009742.2025.2562676 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) is a common symptom and may be an early sign of systemic sclerosis (SSc). To identify biomarkers distinguishing whether RP is associated with definitive SSc, we used phosphospecific flow cytometry to measure phosphorylated (p) signalling molecules [signal transducers and activators of transcription (pSTAT3, pSTAT6, pSTAT4), pSmad2/3, nuclear factor-κB (pNF-κB)] in peripheral blood leucocytes of RP patients undergoing nailfold videocapillaroscopy. METHOD: Leucocyte subsets (CD14(+) monocytes, CD4(+) and CD8(+) T cells, CD19(+) B cells) were identified by surface markers, and phosphorylation was measured after cytokine or lipopolysaccharide stimulation. Medical records were reviewed 9-10 years later, comparing RP subjects who developed SSc (SSc(+), n = 8) with those who did not (SSc(-), n = 17) and healthy controls (HCs, n = 8). RESULTS: SSc(+) patients had significantly higher constitutive pSmad2/3 levels in CD4(+) T cells than SSc(-) or HCs (p = 0.005 and p = 0.034). SSc(+) and SSc(-) had higher stimulated pSTAT3(pY705) levels in CD4(+) T cells than HCs (p = 0.001 and p = 0.026). SSc(+) had higher stimulated pSTAT6 levels in CD4(+) T cells compared with HCs (p = 0.017) and in CD19(+) B cells compared with SSc(-) and HCs (p = 0.006 and p < 0.001). SSc(+) had higher stimulated pSTAT4 levels in CD4(+) T cells compared with SSc(-) and HCs (p = 0.004 and p = 0.037) and in CD8(+) T cells compared with SSc(-) (p = 0.007). No significant differences were found in pNF-κB and pSTAT3(pS727) levels. CONCLUSION: The results give insights into the pathogenesis of SSc. Smad2/3, STAT3(pY705), STAT6, and STAT4 pathways may serve as novel SSc biomarkers. FAU - Parmanne, P AU - Parmanne P AUID- ORCID: 0000-0002-4691-2107 AD - Department of Rheumatology, Inflammation Centre, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. FAU - Kuuliala, A AU - Kuuliala A AUID- ORCID: 0000-0002-3190-207X AD - Bacteriology and Immunology, Medicum, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. FAU - Häme, A AU - Häme A AD - Department of Rheumatology, Inflammation Centre, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. FAU - Luosujärvi, R AU - Luosujärvi R AD - Department of Rheumatology, Inflammation Centre, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. FAU - Leirisalo-Repo, M AU - Leirisalo-Repo M AUID- ORCID: 0000-0002-1568-9045 AD - Department of Rheumatology, Inflammation Centre, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. FAU - Kuuliala, K AU - Kuuliala K AUID- ORCID: 0000-0002-3586-5712 AD - Bacteriology and Immunology, Medicum, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20251104 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (STAT3 Transcription Factor) RN - 0 (Biomarkers) RN - 0 (STAT3 protein, human) RN - 0 (STAT6 Transcription Factor) RN - 0 (Smad3 Protein) RN - 0 (Smad2 Protein) RN - 0 (STAT4 Transcription Factor) RN - 0 (NF-kappa B) RN - 0 (STAT6 protein, human) RN - 0 (SMAD3 protein, human) RN - 0 (STAT4 protein, human) SB - IM MH - Humans MH - *Raynaud Disease/blood/metabolism/etiology MH - *Scleroderma, Systemic/complications/blood/metabolism MH - Female MH - Pilot Projects MH - Male MH - Middle Aged MH - Adult MH - STAT3 Transcription Factor/metabolism MH - Signal Transduction MH - *Leukocytes/metabolism MH - Biomarkers/blood/metabolism MH - STAT6 Transcription Factor/metabolism MH - Smad3 Protein/metabolism MH - Smad2 Protein/metabolism MH - STAT4 Transcription Factor/metabolism MH - NF-kappa B/metabolism MH - Aged MH - Phosphorylation MH - Flow Cytometry MH - CD4-Positive T-Lymphocytes/metabolism MH - CD8-Positive T-Lymphocytes/metabolism MH - Case-Control Studies EDAT- 2025/11/04 12:27 MHDA- 2026/03/06 08:58 CRDT- 2025/11/04 08:23 PHST- 2026/03/06 08:58 [medline] PHST- 2025/11/04 12:27 [pubmed] PHST- 2025/11/04 08:23 [entrez] AID - 10.1080/03009742.2025.2562676 [doi] PST - ppublish SO - Scand J Rheumatol. 2026 Mar;55(2):115-122. doi: 10.1080/03009742.2025.2562676. Epub 2025 Nov 4. PMID- 20646358 OWN - NLM STAT- MEDLINE DCOM- 20100824 LR - 20170214 IS - 0394-6320 (Print) IS - 0394-6320 (Linking) VI - 23 IP - 2 DP - 2010 Apr-Jun TI - Functional infrared imaging of paroxysmal ischemic events in patients with Raynaud's phenomenon. PG - 627-32 AB - The use of thermal infrared (IR) imaging together with the study of the thermal recovery from a controlled cold challenge has been proposed in the diagnosis and follow-up of therapeutic response of Raynaud's Phenomenon (RP) and Systemic Sclerosis (SSc). The controlled cold challenge test usually performed during IR investigations may induce a RP in patients with the latter condition. In our Institution we routinely perform capillaroscopy and thermal IR to follow-up SSc patients. In this paper, we describe the thermal recovery patterns shown by two SSc patients (a 40 year-old male with diffuse variant of SSc and a 71 year-old female with a limited variant of SSc) who presented ischemic and paroxysmal RP attack while recovering from the routine controlled cold challenge test. During RP attack, the cutaneous temperature of some fingers continued to decrease for some minutes even after the cessation of the cold stress. To the best of our knowledge, to date, no literature report has documented the thermal behaviour of SSc patients' fingers which occasionally present ischemic and paroxysmal response. Triggering of ischemic RP attack appears to not rely only on morphological and structural finger impairment, but also upon other aspects, like the emotional attitude of the subject and the possible discomfort experienced with the proceeding of the functional cold stress test. FAU - Grossi, G AU - Grossi G FAU - Mariotti, A AU - Mariotti A FAU - Di Donato, L AU - Di Donato L FAU - Amerio, P AU - Amerio P FAU - Tulli, A AU - Tulli A FAU - Romani, G L AU - Romani GL FAU - Merla, A AU - Merla A LA - eng PT - Letter PL - England TA - Int J Immunopathol Pharmacol JT - International journal of immunopathology and pharmacology JID - 8911335 SB - IM MH - Adult MH - Aged MH - Cold Temperature MH - Female MH - Fingers/blood supply MH - Humans MH - *Infrared Rays MH - Ischemia/*diagnosis MH - Male MH - Raynaud Disease/*physiopathology MH - Scleroderma, Systemic/*physiopathology MH - Skin Temperature MH - Vasoconstriction EDAT- 2010/07/22 06:00 MHDA- 2010/08/25 06:00 CRDT- 2010/07/22 06:00 PHST- 2010/07/22 06:00 [entrez] PHST- 2010/07/22 06:00 [pubmed] PHST- 2010/08/25 06:00 [medline] AID - 25 [pii] AID - 10.1177/039463201002300225 [doi] PST - ppublish SO - Int J Immunopathol Pharmacol. 2010 Apr-Jun;23(2):627-32. doi: 10.1177/039463201002300225. PMID- 20194444 OWN - NLM STAT- MEDLINE DCOM- 20100622 LR - 20100402 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 4 DP - 2010 Apr TI - Thumb involvement in Raynaud's phenomenon as an indicator of underlying connective tissue disease. PG - 783-6 LID - 10.3899/jrheum.091117 [doi] AB - OBJECTIVE: To conduct a retrospective study to assess whether the degree of thumb involvement differs between primary Raynaud's phenomenon (PRP) and secondary Raynaud's phenomenon (SRP). METHODS: Thermography images from all patients attending Salford Royal Hospital and referred for thermography for assessment of RP between 2004 and 2006 were retrospectively reviewed. A distal dorsal difference (DDD) of -1 degrees C or less between the fingertips and dorsum of the hand (fingers cooler) at 23 degrees C was considered clinically relevant. The worse score (the lower score, i.e., the more negative value) from each pair of digits was considered for analysis. RESULTS: One hundred seventy patients fulfilled the study criteria. DDD at 23 degrees C for the thumbs were significantly higher (digital tips warmer) compared with other digits (p < 0.001) in both PRP and SRP. All digits were significantly warmer in PRP compared to SRP with the exception of the thumbs. The proportion of patients with clinically relevant involvement of thumbs was significantly higher in SRP compared to PRP (p = 0.003) and this difference was more pronounced in the thumbs compared with other digits. CONCLUSION: Although the median temperature gradient along the thumb was not significantly different between SRP and PRP, the thumb is more likely to be involved in SRP than in PRP. Thumb involvement is one of a number of clinical indicators that should alert the clinician to the possibility of an underlying connective tissue disease/disorder. FAU - Chikura, Batsi AU - Chikura B AD - Department of Rheumatology, Lincoln County Hospital, Lincoln, UK. batsi@doctors.org.uk FAU - Moore, Tonia AU - Moore T FAU - Manning, Joanne AU - Manning J FAU - Vail, Andy AU - Vail A FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Journal Article DEP - 20100301 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Analysis of Variance MH - Connective Tissue Diseases/*diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - Retrospective Studies MH - Thermography MH - *Thumb EDAT- 2010/03/03 06:00 MHDA- 2010/06/23 06:00 CRDT- 2010/03/03 06:00 PHST- 2010/03/03 06:00 [entrez] PHST- 2010/03/03 06:00 [pubmed] PHST- 2010/06/23 06:00 [medline] AID - jrheum.091117 [pii] AID - 10.3899/jrheum.091117 [doi] PST - ppublish SO - J Rheumatol. 2010 Apr;37(4):783-6. doi: 10.3899/jrheum.091117. Epub 2010 Mar 1. PMID- 38711393 OWN - NLM STAT- MEDLINE DCOM- 20240806 LR - 20240806 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 20 IP - 9 DP - 2024 Sep TI - Can we define difficult-to-treat systemic sclerosis? PG - 1065-1081 LID - 10.1080/1744666X.2024.2352450 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a chronic autoimmune rheumatic disease characterized by microvascular alterations, immunopathology, and widespread fibrosis involving various organs. It is considered difficult to treat due to several reasons: complex pathogenesis, heterogeneity, late diagnosis, limited treatment options for certain organ manifestations, lack of personalized medicine. AREAS COVERED: This review presents the heterogeneity, survival and organ manifestations with their risk factors of systemic sclerosis and their current treatment options, while drawing attention to difficult-to-treat forms of the disease, based on literature indexed in PubMed. EXPERT OPINION: Despite recent advances in the management of SSc over the last decades, the disease presents significant morbidity and mortality. Although available treatment protocols brought significant advancements in terms of survival in SSc-associated interstitial lung disease and pulmonary arterial hypertension, less success has been achieved in the treatment of Raynaud's phenomenon and digital ulcers and the results are modest in case of heart, gastrointestinal, and renal manifestations. There are patients who do not respond to treatment and deteriorate even with adequate therapy. They can be considered difficult-to treat (D2T) cases. We have created a possible score system based on the individual organ manifestations and highlighted treatment options for the D2T SSc category. FAU - Szűcs, Gabriella AU - Szűcs G AD - Department of Rheumatology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. FAU - Szekanecz, Zoltán AU - Szekanecz Z AD - Department of Rheumatology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. FAU - Szamosi, Szilvia AU - Szamosi S AD - Department of Rheumatology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary. LA - eng PT - Journal Article PT - Review DEP - 20240514 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - *Scleroderma, Systemic/therapy/diagnosis MH - Humans MH - Lung Diseases, Interstitial/therapy/etiology/diagnosis MH - Raynaud Disease/therapy/diagnosis MH - Precision Medicine MH - Risk Factors OTO - NOTNLM OT - Autoimmune diseases OT - connective tissue diseases OT - difficult-to-treat OT - systemic sclerosis OT - treatment EDAT- 2024/05/07 06:42 MHDA- 2024/08/06 06:43 CRDT- 2024/05/07 03:13 PHST- 2024/08/06 06:43 [medline] PHST- 2024/05/07 06:42 [pubmed] PHST- 2024/05/07 03:13 [entrez] AID - 10.1080/1744666X.2024.2352450 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2024 Sep;20(9):1065-1081. doi: 10.1080/1744666X.2024.2352450. Epub 2024 May 14. PMID- 40958138 OWN - NLM STAT- MEDLINE DCOM- 20251016 LR - 20260522 IS - 1542-2011 (Electronic) IS - 1526-9523 (Linking) VI - 70 IP - 5 DP - 2025 Sep-Oct TI - Raynaud's Phenomenon of the Nipple: A Case Report of Postpartum Recurrence and Treatment. PG - 807-811 LID - 10.1111/jmwh.70012 [doi] AB - Raynaud phenomenon is a well-known condition that is characterized by episodic vasoconstriction of the extremities leading to pain and discoloration. It is more common among women than men and often results from exposure to cold or stress. Raynaud phenomenon can also affect the nipple during breastfeeding, causing severe pain and distress for the lactating individual and newborn, leading to premature cessation of breastfeeding. Raynaud phenomenon of the nipple is often confused with other breastfeeding pain causes, which can result in treatment oversights. The etiology of Raynaud phenomenon of the nipple is complex and thought to be caused by an interplay of hormones and stress in the peripartum period. Literature on this condition is limited, mostly consisting of case reports, and there are very little data about its recurrence in subsequent peripartum periods. Treatment options are similar to those for Raynaud phenomenon and, if initiated in a timely fashion, can allow breastfeeding to continue uninterrupted. This clinical rounds article presents a case report of Raynaud phenomenon of the nipple after a first birth and a reoccurrence during a subsequent (second) postpartum period. Discussion of the pathophysiology, clinical presentation, diagnostic tips, and appropriate treatment options are included. CI - © 2025 by the American College of Nurse‐Midwives. FAU - Trebbin, Jennifer AU - Trebbin J AUID- ORCID: 0009-0002-4274-9389 AD - Cambridge Health Alliance, Cambridge, Massachusetts. AD - Harvard School of Public Health, Boston, Massachusetts. FAU - Singh, Tara A AU - Singh TA AD - Cambridge Health Alliance, Cambridge, Massachusetts. AD - Harvard Medical School, Boston, Massachusetts. LA - eng PT - Clinical Conference PT - Journal Article DEP - 20250916 PL - United States TA - J Midwifery Womens Health JT - Journal of midwifery & women's health JID - 100909407 SB - IM MH - Adult MH - Female MH - Humans MH - Infant, Newborn MH - *Breast Diseases/therapy/diagnosis MH - *Breast Feeding/adverse effects MH - *Nipples/blood supply MH - *Postpartum Period MH - *Raynaud Disease/therapy/diagnosis MH - Recurrence OTO - NOTNLM OT - Raynaud phenomenon of the nipple OT - breast/chest health OT - breastfeeding cessation OT - lactation OT - nipple vasospasm OT - painful breastfeeding OT - postpartum care OT - recurrence OT - treatment EDAT- 2025/09/17 00:30 MHDA- 2025/10/16 12:31 CRDT- 2025/09/16 23:53 PHST- 2025/10/16 12:31 [medline] PHST- 2025/09/17 00:30 [pubmed] PHST- 2025/09/16 23:53 [entrez] AID - 10.1111/jmwh.70012 [doi] PST - ppublish SO - J Midwifery Womens Health. 2025 Sep-Oct;70(5):807-811. doi: 10.1111/jmwh.70012. Epub 2025 Sep 16. PMID- 16977343 OWN - NLM STAT- MEDLINE DCOM- 20061211 LR - 20260128 IS - 1434-5161 (Print) IS - 1434-5161 (Linking) VI - 51 IP - 10 DP - 2006 TI - Glutathione S-transferase M1 and GST T1 genetic polymorphisms and Raynaud's phenomenon in French vinyl chloride monomer-exposed workers. PG - 879-886 LID - 10.1007/s10038-006-0038-9 [doi] AB - Occupational vinyl chloride monomer (VCM) exposure can induce Raynaud's phenomenon (RP). However, not all VCM workers developed RP, which suggests an underlying genetic susceptibility. Genetic polymorphisms of glutathione S-transferases (GSTs), involved in VCM metabolism, have been shown to influence certain VCM-related health effects. We have conducted a case-control study of 58 subjects with RP along with 247 subjects without RP, from a population of 305 French workers exposed or formerly exposed to VCM, to assess any association between GST M1 and GST T1 gene polymorphisms, either separately or in combination, and the presence of RP. None of the GST M1 or GST T1 genotypes were significantly associated with the presence of RP among studied VCM workers. A combination of positive genotypes for both GST M1 and GST T1 was significantly associated with RP presence, compared to the other combinations of genotypes (OR=2.1, 95% CI=1.1-3.8). OR adjusted for age, smoking status, alcohol consumption and history of treated hypertension did not reach significance (OR=2.0, 95% CI=0.9-5.2). None of the GST M1 and GST T1 genotypes seem to contribute separately to the presence of RP, suggesting that they are not, when taken alone, a major determinant of interindividual variability for VCM-induced PR. However, the combination of both positive GST M1 and GST T1 genotypes appears to contribute slightly to susceptibility to RP in VCM-exposed subjects. Nevertheless, our study-the first to examine the role of a genetic component in the occurrence of RP secondary to occupational exposure to a chemical-corroborates the previous considerations that interaction between the genetic constitution and environmental factors is of importance in determining the health-adverse effects of VCM exposure. FAU - Fontana, Luc AU - Fontana L AD - Institut de Médecine du Travail, Faculté de Médecine, UFR Médecine, Univ Clermont 1, 28 place Henri Dunant, 63001, Clermont-Ferrand, France. Luc.FONTANA@u-clermont1.fr. AD - Service de Médecine du travail et de pathologie professionnelle, CHU Clermont-Ferrand, 28 place Henri Dunant, 63001, Clermont-Ferrand Cedex, France. Luc.FONTANA@u-clermont1.fr. FAU - Marion, Marie-Jeanne AU - Marion MJ AD - INSERM U271, 151 cours Albert Thomas, 69424, Lyon Cedex 03, France. FAU - Ughetto, Sylvie AU - Ughetto S AD - Service d'épidémiologie, économie de santé et prévention, CHU Clermont-Ferrand, Hôtel Dieu, boulevard Léon Malfreyt, 63000, Clermont-Ferrand, France. FAU - Catilina, Pierre AU - Catilina P AD - Faculté de Médecine, Institut de Médecine du Travail, Université d'Auvergne, 28 place Henri Dunant, 63001, Clermont-Ferrand Cedex, France. LA - eng PT - Journal Article DEP - 20060915 PL - England TA - J Hum Genet JT - Journal of human genetics JID - 9808008 RN - EC 2.5.1.18 (Glutathione Transferase) RN - WD06X94M2D (Vinyl Chloride) RN - EC 2.5.1.- (glutathione S-transferase T1) SB - IM MH - Aged MH - Case-Control Studies MH - France MH - *Genetic Predisposition to Disease MH - Genotype MH - Glutathione Transferase/*genetics MH - Humans MH - Male MH - Middle Aged MH - *Occupational Exposure MH - *Polymorphism, Genetic MH - Raynaud Disease/chemically induced/*genetics MH - Vinyl Chloride/*toxicity MH - White People EDAT- 2006/09/16 09:00 MHDA- 2006/12/12 09:00 CRDT- 2006/09/16 09:00 PHST- 2006/04/07 00:00 [received] PHST- 2006/07/09 00:00 [accepted] PHST- 2006/09/16 09:00 [pubmed] PHST- 2006/12/12 09:00 [medline] PHST- 2006/09/16 09:00 [entrez] AID - 10.1007/s10038-006-0038-9 [pii] AID - 10.1007/s10038-006-0038-9 [doi] PST - ppublish SO - J Hum Genet. 2006;51(10):879-886. doi: 10.1007/s10038-006-0038-9. Epub 2006 Sep 15. PMID- 41261736 OWN - NLM STAT- MEDLINE DCOM- 20260407 LR - 20260409 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 78 IP - 4 DP - 2026 Apr TI - Drug-Induced Raynaud's Phenomenon and Underlying Mechanism: A Disproportionality Analysis From the World Health Organization Pharmacovigilance Database. PG - 985-992 LID - 10.1002/art.43442 [doi] AB - OBJECTIVES: The aim of this study is to generate hypotheses about unknown drugs associated with the onset or worsening of Raynaud's phenomenon (RP) and to explore their potential pathophysiologic mechanisms through a mixed disproportionality/clustering analysis from the World Health Organization (WHO) pharmacovigilance database. METHODS: Using the WHO pharmacovigilance database, we identified cases using the Medical Dictionary for Regulatory Activities Preferred Term "Raynaud's phenomenon," and we excluded all Individual Case Safety Reports (ICSRs) associated with at least one drug used in RP treatment. To estimate signals of disproportionate reporting (SDR), we calculated information component (IC) values (IC(LB) >0 deemed significant). We performed several sensitivity analyses to assess the robustness of the results. We evaluated and prioritized the plausibility of signals according to expert review of cases characteristics, robustness of results, and pharmacological hypotheses. Lastly, to explore pathophysiologic mechanisms, we used drug target extraction from DrugBank and a clustering method to identify similar patterns of adverse events reporting. RESULTS: We included 4,430 ICSRs of RP in our analysis. We found 124 significant SDRs in the primary analysis, of which 52 SDRs were consistent across all sensitivity analyses, and 16 were considered probable after signal evaluation and prioritization, including amphetamine-like, antimigraine drugs, antineoplastics drugs, and dopaminergic agonists. Most of the targets involved were 5-HT1A receptors, sodium-dependent noradrenaline transporters, and beta-1 and beta-2 adrenergic receptors. Cluster analyses yielded inconsistent results according to the method used. CONCLUSION: This study allowed us to identify robust safety signals (such as solriamfetol, tyrosine kinase inhibitors, and calcitonin gene-related peptide inhibitors) for drugs associated with RP and potential implicated pathophysiologic mechanisms. CI - © 2025 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Hlavaty, Alex AU - Hlavaty A AUID- ORCID: 0009-0002-8483-5023 AD - Pharmacovigilance Unit, Grenoble Alpes University Hospital, University Grenoble Alpes, F-38000, Grenoble, France. AD - University Grenoble Alpes, INSERM U1300, HP2, Grenoble, France. FAU - Dari, Loubna AU - Dari L AD - Department of Vascular Medicine, Bordeaux University Hospital, Bordeaux, France. AD - Bordeaux Population Health, INSERM U1219, Bordeaux, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Pharmacovigilance Unit, Grenoble Alpes University Hospital, University Grenoble Alpes, F-38000, Grenoble, France. AD - University Grenoble Alpes, INSERM U1300, HP2, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AUID- ORCID: 0000-0003-4475-1626 AD - University Grenoble Alpes, INSERM U1300, HP2, Grenoble, France. AD - University Grenoble Alpes, INSERM CIC1406, Grenoble Alpes University Hospital, Grenoble, France. FAU - Khouri, Charles AU - Khouri C AUID- ORCID: 0000-0002-8427-8573 AD - Pharmacovigilance Unit, Grenoble Alpes University Hospital, University Grenoble Alpes, F-38000, Grenoble, France. AD - University Grenoble Alpes, INSERM U1300, HP2, Grenoble, France. AD - University Grenoble Alpes, INSERM CIC1406, Grenoble Alpes University Hospital, Grenoble, France. LA - eng PT - Journal Article DEP - 20260203 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 SB - IM MH - Humans MH - *Raynaud Disease/chemically induced/physiopathology MH - *Pharmacovigilance MH - Databases, Factual MH - World Health Organization MH - Female MH - Male MH - Middle Aged MH - Adult MH - Aged MH - Cluster Analysis PMC - PMC13054453 EDAT- 2025/11/20 06:26 MHDA- 2026/04/07 06:31 PMCR- 2026/04/07 CRDT- 2025/11/20 01:13 PHST- 2025/10/13 00:00 [revised] PHST- 2025/07/24 00:00 [received] PHST- 2025/10/20 00:00 [accepted] PHST- 2026/04/07 06:31 [medline] PHST- 2025/11/20 06:26 [pubmed] PHST- 2025/11/20 01:13 [entrez] PHST- 2026/04/07 00:00 [pmc-release] AID - ART43442 [pii] AID - 10.1002/art.43442 [doi] PST - ppublish SO - Arthritis Rheumatol. 2026 Apr;78(4):985-992. doi: 10.1002/art.43442. Epub 2026 Feb 3. PMID- 25112484 OWN - NLM STAT- MEDLINE DCOM- 20150828 LR - 20141224 IS - 1607-842X (Electronic) IS - 0891-6934 (Linking) VI - 48 IP - 1 DP - 2015 Feb TI - The association between vibration and vascular injury in rheumatic diseases: a review of the literature. PG - 61-8 LID - 10.3109/08916934.2014.947477 [doi] AB - Vascular manifestations can be seen early in the pathogenesis of inflammatory rheumatic diseases. Animal experiments, laboratory and clinical findings indicated that acute or long-term vibration exposure can induce vascular abnormalities. Recent years, in addition to Raynaud's phenomenon (RP), vibration as a risk factor for other rheumatic diseases has also received corresponding considered. This review is concentrated upon the role of vibration in the disease of systemic sclerosis (SSc). In this review, we are going to discuss the main mechanisms which are thought to be important in pathophysiology of vascular injury under the three broad headings of "vascular", "neural" and "intravascular". Aspects on the vibration and vascular inflammation are briefly discussed. And the epidemiological studies related to vibration studies in SSc and other rheumatic diseases are taken into account. FAU - Wang, Yu-Jie AU - Wang YJ AD - Department of Epidemiology and Biostatistics, School of Public Health, Anhui Medical University , Hefei , China . FAU - Huang, Xiao-Lei AU - Huang XL FAU - Yan, Jun-Wei AU - Yan JW FAU - Wan, Ya-Nan AU - Wan YN FAU - Wang, Bing-Xiang AU - Wang BX FAU - Tao, Jin-Hui AU - Tao JH FAU - Chen, Bing AU - Chen B FAU - Li, Bao-Zhu AU - Li BZ FAU - Yang, Guo-Jun AU - Yang GJ FAU - Wang, Jing AU - Wang J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20140812 PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Angiogenic Proteins) RN - 0 (Blood Coagulation Factors) SB - IM MH - Angiogenic Proteins/metabolism MH - Arthritis, Rheumatoid/etiology/metabolism/*pathology MH - Blood Coagulation Factors/metabolism MH - Case-Control Studies MH - Humans MH - Microvessels/injuries/metabolism/pathology MH - Occupational Diseases/etiology/metabolism/*pathology MH - Raynaud Disease/etiology/metabolism/*pathology MH - Scleroderma, Systemic/etiology/metabolism/*pathology MH - Vascular System Injuries/etiology/metabolism/*pathology MH - Vibration/*adverse effects OTO - NOTNLM OT - Epidemiology OT - inflammation OT - rheumatic diseases OT - vasculopathy OT - vibration EDAT- 2014/08/13 06:00 MHDA- 2015/09/01 06:00 CRDT- 2014/08/13 06:00 PHST- 2014/08/13 06:00 [entrez] PHST- 2014/08/13 06:00 [pubmed] PHST- 2015/09/01 06:00 [medline] AID - 10.3109/08916934.2014.947477 [doi] PST - ppublish SO - Autoimmunity. 2015 Feb;48(1):61-8. doi: 10.3109/08916934.2014.947477. Epub 2014 Aug 12. PMID- 36550549 OWN - NLM STAT- MEDLINE DCOM- 20221226 LR - 20221226 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Dec 22 TI - Severe Raynaud's phenomenon from ethosuximide raised concern over possible onset of systemic vasculitis: a case report. PG - 120 LID - 10.1186/s12969-022-00782-8 [doi] LID - 120 AB - BACKGROUND: Ethosuximide and other anti-epileptic drugs have been reported to cause idiosyncratic reactions such as lupus-like syndromes, with elevated antinuclear antibody (ANA) levels. Herein, we present a case of a girl who developed a very severe Raynaud's phenomenon reaction and anti-Scl-70 antibodies related to treatment with ethosuximide, due to juvenile absence epilepsy (JAE). CASE PRESENTATION: A 12-year-old girl was diagnosed with JAE and treatment with ethosuximide was initiated. Two and a half months later her fingers, digits II-V bilaterally, began to ache and were discolored, alternatingly white, blue, or normal-colored. Two weeks later, her fingers were bluish-black, aching severely, almost continuously. The family sought medical advice. Ethosuximide was halted and due to the severe symptoms, treatment with both prednisolone and intravenous iloprost was commenced. Laboratory tests revealed high ANA levels with anti-Scl-70 pattern and confirmed anti-Scl-70 antibodies. After a few weeks, she started to improve and the symptoms slowly decreased over five months. Anti-Scl-70 was still detectable four months after onset of symptoms, though she was much improved. After eleven months, repeated ANA analyses were completely negative. CONCLUSION: Although extremely rare, it is important to recognize that severe Raynaud's phenomenon, threatening peripheral digital circulation, may occur as an idiosyncratic reaction to ethosuximide, raising concern over possible onset of vasculitis. CI - © 2022. The Author(s). FAU - Berntson, Lillemor AU - Berntson L AUID- ORCID: 0000-0003-3962-0453 AD - Department of Women's and Children's Health, Uppsala University, SE-75185, Uppsala, Sweden. lillemor.berntson@kbh.uu.se. FAU - Liminga, Gunnar AU - Liminga G AD - Department of Women's and Children's Health, Uppsala University, SE-75185, Uppsala, Sweden. LA - eng GR - Gillbergska stiftelsen/Gillbergska stiftelsen/ GR - Uppsala Universitet/Uppsala Universitet/ PT - Case Reports PT - Journal Article DEP - 20221222 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - 5SEH9X1D1D (Ethosuximide) SB - IM MH - Female MH - Humans MH - Child MH - Ethosuximide/adverse effects MH - *Raynaud Disease/chemically induced MH - *Connective Tissue Diseases MH - Fingers MH - *Systemic Vasculitis MH - Pain PMC - PMC9783411 OTO - NOTNLM OT - Absence epilepsy OT - Adverse effect OT - Anti-epileptic drugs OT - Anti-scl-70 OT - Drug-induced OT - Ethosuximide OT - Raynaud’s syndrome OT - Vasculitis COIS- The authors declare that they have no competing interests. EDAT- 2022/12/23 06:00 MHDA- 2022/12/27 06:00 PMCR- 2022/12/22 CRDT- 2022/12/22 23:55 PHST- 2022/10/17 00:00 [received] PHST- 2022/12/15 00:00 [accepted] PHST- 2022/12/22 23:55 [entrez] PHST- 2022/12/23 06:00 [pubmed] PHST- 2022/12/27 06:00 [medline] PHST- 2022/12/22 00:00 [pmc-release] AID - 10.1186/s12969-022-00782-8 [pii] AID - 782 [pii] AID - 10.1186/s12969-022-00782-8 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Dec 22;20(1):120. doi: 10.1186/s12969-022-00782-8. PMID- 33063580 OWN - NLM STAT- MEDLINE DCOM- 20210827 LR - 20210827 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 50 IP - 2 DP - 2021 Mar TI - Low body weight and involuntary weight loss are associated with Raynaud's phenomenon in both men and women. PG - 153-160 LID - 10.1080/03009742.2020.1780310 [doi] AB - Objectives: Low body weight is an easily assessable cause of Raynaud's phenomenon (RP), and is frequently overlooked by clinicians. We aim to investigate the association of low body weight (body mass index < 18.5 kg/m(2)), involuntary weight loss, and nutritional restrictions with the presence of RP.Method: Participants from the Lifelines Cohort completed a validated self-administered connective tissue disease questionnaire. Subjects who reported cold-sensitive fingers and biphasic or triphasic colour changes were considered to suffer from RP. Patient characteristics, anthropometric measurements, and nutritional habits were collected. Statistical analyses was stratified for gender.Results: Altogether, 93 935 participants completed the questionnaire. The prevalence of RP was 4.2% [95% confidence interval (CI) 4.1-4.4%], and was three-fold higher in women than in men (5.7% vs 2.1%, p < 0.001). Subjects with RP had a significantly lower daily caloric intake than those without RP. Multivariate analysis, correcting for creatinine level, daily caloric intake, and other known aetiological factors associated with RP, revealed that low body weight [men: odds ratio (OR) 5.55 (95% CI 2.82-10.93); women: 3.14 (2.40-4.10)] and involuntary weight loss [men: OR 1.32 (1.17-1.48); women: 1.31 (1.20-1.44)] were significantly associated with the presence of RP. Low-fat diet was also associated with RP in women [OR 1.27 (1.15-1.44)].Conclusion: Low body weight and prior involuntary weight loss are associated with an increased risk of RP in both men and women. This study emphasizes that low body weight and weight loss are easily overlooked risk factors for RP, and should be assessed and monitored in subjects with RP. FAU - Abdulle, A E AU - Abdulle AE AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Arends, S AU - Arends S AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - van Goor, H AU - van Goor H AD - Department of Pathology and Medical Biology, Section Pathology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Brouwer, E AU - Brouwer E AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - van Roon, A M AU - van Roon AM AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Westra, J AU - Westra J AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Herrick, A L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - de Leeuw, K AU - de Leeuw K AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Mulder, D J AU - Mulder DJ AD - Department of Internal Medicine, Division Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. LA - eng PT - Journal Article DEP - 20201016 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Body Mass Index MH - Body Weight/*physiology MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/epidemiology/*physiopathology MH - Surveys and Questionnaires MH - Weight Loss/*physiology EDAT- 2020/10/17 06:00 MHDA- 2021/08/28 06:00 CRDT- 2020/10/16 08:38 PHST- 2020/10/17 06:00 [pubmed] PHST- 2021/08/28 06:00 [medline] PHST- 2020/10/16 08:38 [entrez] AID - 10.1080/03009742.2020.1780310 [doi] PST - ppublish SO - Scand J Rheumatol. 2021 Mar;50(2):153-160. doi: 10.1080/03009742.2020.1780310. Epub 2020 Oct 16. PMID- 31790328 OWN - NLM STAT- MEDLINE DCOM- 20210624 LR - 20210624 IS - 2688-1535 (Electronic) IS - 2688-1527 (Linking) VI - 16 IP - 10 DP - 2020 Oct TI - Raynaud's Phenomenon From PD-1 Immune Checkpoint Inhibition. PG - 701-702 LID - 10.1200/JOP.19.00333 [doi] FAU - Kim, Haesoo AU - Kim H AUID- ORCID: 0000-0002-7821-264X AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Jones, Adrian J AU - Jones AJ AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Labadzhyan, Artak AU - Labadzhyan A AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Placencio-Hickok, Veronica R AU - Placencio-Hickok VR AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Wallace, Daniel J AU - Wallace DJ AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Gong, Jun AU - Gong J AUID- ORCID: 0000-0001-8713-1406 AD - Cedars-Sinai Medical Center, Los Angeles, CA. FAU - Hendifar, Andrew E AU - Hendifar AE AD - Cedars-Sinai Medical Center, Los Angeles, CA. LA - eng PT - Journal Article DEP - 20191202 PL - United States TA - JCO Oncol Pract JT - JCO oncology practice JID - 101758685 RN - 0 (Immune Checkpoint Inhibitors) RN - 0 (Programmed Cell Death 1 Receptor) SB - IM MH - Humans MH - *Immune Checkpoint Inhibitors MH - Programmed Cell Death 1 Receptor MH - *Raynaud Disease EDAT- 2019/12/04 06:00 MHDA- 2021/06/25 06:00 CRDT- 2019/12/03 06:00 PHST- 2019/12/04 06:00 [pubmed] PHST- 2021/06/25 06:00 [medline] PHST- 2019/12/03 06:00 [entrez] AID - 10.1200/JOP.19.00333 [doi] PST - ppublish SO - JCO Oncol Pract. 2020 Oct;16(10):701-702. doi: 10.1200/JOP.19.00333. Epub 2019 Dec 2. PMID- 30711504 OWN - NLM STAT- MEDLINE DCOM- 20190730 LR - 20220409 IS - 1615-5947 (Electronic) IS - 0890-5096 (Linking) VI - 57 DP - 2019 May TI - Ulnar Artery Thrombosis Presented with Unilateral Raynaud's Phenomenon Findings after Long-Term Intensive Handicraft Activity: Hypothenar Hammer Syndrome. PG - 275.e13-275.e15 LID - S0890-5096(19)30056-1 [pii] LID - 10.1016/j.avsg.2018.10.034 [doi] AB - BACKGROUND: Hypothenar hammer syndrome (HHS) is an uncommon vascular syndrome of upper extremity. HHS should be considered in patients who are presented with digital ischemia. Distal ulnar artery compression at the level of Guyon's canal with trauma results in thrombus or aneurysm. It may be observed after repetitive chronic trauma or acute blunt trauma to hypothenar eminence. Middle-aged male laborers, smokers, and dominant hands are affected frequently. Hand pain, discoloration or ulceration of digits, cold intolerance, hypothenar pulsatile mass, hypothenar weakness, and numbness are significant clinical findings. CASE CHARACTERISTICS: In this report, we presented a 37-year-old woman complaining with intermittent hand pain, paleness, and cyanosis at third, fourth, and fifth fingers of the right hand. She had no blunt trauma to her hand but intense amount of needle lace with her hands. Doppler ultrasonography revealed ulnar arterial thrombus at right Guyon's canal level. CONCLUSIONS: She was diagnosed as HHS secondary to intense needlework. A calcium channel blocker and low-dose aspirin were prescribed to her, and avoidance of hand traumas was suggested. These interventions relieved digital ischemia symptoms on her hand. CI - Copyright © 2019 Elsevier Inc. All rights reserved. FAU - Mülkoğlu, Cevriye AU - Mülkoğlu C AD - Department of Physical Therapy and Rehabilitation of University of Health Sciences, Ankara Health Application and Research Center, Ankara, Turkey. Electronic address: drckaraca@hotmail.com. FAU - Genç, Hakan AU - Genç H AD - Department of Physical Therapy and Rehabilitation of University of Health Sciences, Ankara Health Application and Research Center, Ankara, Turkey. LA - eng PT - Case Reports PT - Journal Article DEP - 20190131 PL - Netherlands TA - Ann Vasc Surg JT - Annals of vascular surgery JID - 8703941 RN - 0 (Calcium Channel Blockers) RN - 0 (Fibrinolytic Agents) MH - Adult MH - Arterial Occlusive Diseases/diagnostic imaging/drug therapy/*etiology/physiopathology MH - Calcium Channel Blockers/therapeutic use MH - Female MH - Fibrinolytic Agents/therapeutic use MH - *Hobbies MH - Humans MH - Ischemia/diagnostic imaging/drug therapy/*etiology/physiopathology MH - Raynaud Disease/diagnostic imaging/drug therapy/*etiology/physiopathology MH - Regional Blood Flow MH - Syndrome MH - Thrombosis/diagnostic imaging/drug therapy/*etiology/physiopathology MH - Time Factors MH - Treatment Outcome MH - *Ulnar Artery/diagnostic imaging/drug effects/physiopathology MH - Ultrasonography, Doppler, Color EDAT- 2019/02/04 06:00 MHDA- 2019/07/31 06:00 CRDT- 2019/02/04 06:00 PHST- 2018/05/18 00:00 [received] PHST- 2018/10/24 00:00 [accepted] PHST- 2019/02/04 06:00 [pubmed] PHST- 2019/07/31 06:00 [medline] PHST- 2019/02/04 06:00 [entrez] AID - S0890-5096(19)30056-1 [pii] AID - 10.1016/j.avsg.2018.10.034 [doi] PST - ppublish SO - Ann Vasc Surg. 2019 May;57:275.e13-275.e15. doi: 10.1016/j.avsg.2018.10.034. Epub 2019 Jan 31. PMID- 27443761 OWN - NLM STAT- MEDLINE DCOM- 20170518 LR - 20170518 IS - 1896-494X (Electronic) IS - 1232-1087 (Linking) VI - 29 IP - 4 DP - 2016 TI - Postocclusive reactive hyperemia in hand-arm vibration syndrome. PG - 659-66 LID - 59326 [pii] LID - 10.13075/ijomeh.1896.00765 [doi] AB - OBJECTIVES: To assess laser Doppler-recorded postocclusive reactive hyperemic responses in vibration-induced Raynaud's phenomenon and compare it with primary and secondary to sclerodermy Raynaud's phenomenon. MATERIAL AND METHODS: Thirty patients with vibration-induced Raynaud's phenomenon and 30 healthy controls and patients with primary and secondary to sclerodermy Raynaud's phenomenon were investigated. Fingerpulp skin blood flow was monitored by laser Doppler flowmetry during postocclusive reactive hyperemia test. RESULTS: Lower initial perfusion values were established in all the patients with Raynaud's phenomenon compared to the healthy controls (p < 0.0001). The postocclusive reactive hyperemic peak was lower in all the Raynaud's phenomenon groups compared to the controls (p < 0.0001). The postocclusive and basal perfusions were lower in the secondary Raynaud's phenomenon groups compared to the control and the primary Raynaud's phenomenon groups (p < 0.0001). The velocities to postocclusive hyperemic peak were lower in all the Raynaud's phenomenon patients (p < 0.0001), so were in the vibration-induced (p < 0.002) and the sclerodermy Raynaud's phenomenon (p < 0.004) groups in relation to the primary Raynaud's phenomenon group. The perfusion values and the velocities were significantly influenced by the initial superficial skin temperatures and perfusions, while the velocities were dependent also on gender, and the hyperemic peak on age. CONCLUSIONS: Postocclusive reactive hyperemia is abnormal in all Raynaud's phenomenon patients. Laser Doppler-recorded reactive hyperemia test contributes to diagnosing Raynaud's phenomenon and has proved to be valuable for group analysis. The applied method is not sensitive enough to discriminate adequately the type of Raynaud's phenomenon among individual cases. CI - This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license. FAU - Stoyneva, Zlatka AU - Stoyneva Z AD - Medical University of Sofia, Sofia, Bulgaria (University Hospital St. Ivan Rilsky, Clinic of Occupational Diseases). zlatka_stoyneva@yahoo.com. LA - eng PT - Comparative Study PT - Journal Article PL - Poland TA - Int J Occup Med Environ Health JT - International journal of occupational medicine and environmental health JID - 9437093 SB - IM MH - Adult MH - Female MH - Fingers/blood supply MH - Hand-Arm Vibration Syndrome/*physiopathology MH - Humans MH - Hyperemia/*physiopathology MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Occupational Diseases/physiopathology MH - Raynaud Disease/*physiopathology MH - Scleroderma, Systemic/physiopathology MH - Skin Temperature OTO - NOTNLM OT - Raynaud’s phenomenon OT - hand-arm vibration syndrome OT - laser Doppler flowmetry OT - microcirculation OT - reactive hyperemia test OT - sclerodermy EDAT- 2016/07/23 06:00 MHDA- 2017/05/19 06:00 CRDT- 2016/07/23 06:00 PHST- 2016/07/23 06:00 [entrez] PHST- 2016/07/23 06:00 [pubmed] PHST- 2017/05/19 06:00 [medline] AID - 59326 [pii] AID - 10.13075/ijomeh.1896.00765 [doi] PST - ppublish SO - Int J Occup Med Environ Health. 2016;29(4):659-66. doi: 10.13075/ijomeh.1896.00765. PMID- 28595537 OWN - NLM STAT- MEDLINE DCOM- 20180925 LR - 20180925 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 14 IP - 1 DP - 2018 Apr 20 TI - Capillaroscopic Findings in Primary Fibromyalgia. PG - 36-41 LID - 10.2174/1573397113666170607160854 [doi] AB - INTRODUCTION: Although Raynaud's Phenomenon (RP) is observed in a significant proportion of patients with primary fibromyalgia, the available data on capillaroscopic findings in primary fibromyalgia are scarce. OBJECTIVE: The purpose of the study was to assess the capillaroscopic pattern in patients with primary fibromyalgia. PATIENTS AND METHODS: 26 patients with primary fibromyalgia (25 women and 1 man) were included in the study. Mean age was 55±10 years. As control groups were examined 31 patients with primary RP and 35 healthy volunteers. Capillaroscopic examination was performed with a videocapillaroscope Videocap 3.0 (DS Medica), magnification 200x with analysis of the main capillaroscopic parameters as follows: capillary distribution, shape, mean capillary diameters and length, mean capillary density, visibility of the subpapillary plexus; presence of avascular areas, microhaemorrhages and neoangiogenic capillaries. RESULTS: Symptoms of RP were observed in 65% (17/26) of the cases with primary fibromyalgia. At capillaroscopic examination, the most frequent finding in patients with primary fibromyalgia was the presence of capillary dilation in 85% (22/26) of the patients - both in cases with and without RP. However, the mean arterial and venous capillary diameters were significantly higher in the subgroup of fibromyalgia patients with clinical symptoms of RP. Of note, microvascular abnormalities characteristic of connective tissue diseases could not be observed in primary fibromyalgia patients. Analogous changes - presence of dilated capillaries - were found in 96.6% (29/30) of patients with primary RP. CONCLUSION: In our study, the most frequent capillaroscopic finding in patients with primary fibromyalgia was the presence of dilated capillary loops analogous to primary RP. Capillaroscopic signs suggestive of connective tissue disease could not be found in primary fibromyalgia patients. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Faculty of Medicine, Department of Propaedeutics of Internal Diseases, Medical University, Plovdiv, Bulgaria. FAU - Muller-Ladner, Ulf AU - Muller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen Bad Nauheim, Germany. LA - eng PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Adult MH - Female MH - Fibromyalgia/*complications/*diagnostic imaging/pathology MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease/*complications OTO - NOTNLM OT - Nailfold capillaroscopy OT - connective tissue OT - dilated capillaries OT - patients OT - primary Raynaud’s phenomenon OT - primary fibromyalgia. EDAT- 2017/06/10 06:00 MHDA- 2018/09/27 06:00 CRDT- 2017/06/10 06:00 PHST- 2017/06/10 06:00 [pubmed] PHST- 2018/09/27 06:00 [medline] PHST- 2017/06/10 06:00 [entrez] AID - CRR-EPUB-83944 [pii] AID - 10.2174/1573397113666170607160854 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2018 Apr 20;14(1):36-41. doi: 10.2174/1573397113666170607160854. PMID- 24352340 OWN - NLM STAT- MEDLINE DCOM- 20140507 LR - 20260128 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 53 IP - 4 DP - 2014 Apr TI - Head-to-head comparison of udenafil vs amlodipine in the treatment of secondary Raynaud's phenomenon: a double-blind, randomized, cross-over study. PG - 658-64 LID - 10.1093/rheumatology/ket417 [doi] AB - OBJECTIVE: RP is a reversible vasoconstriction of digital arteries that causes pain and skin discoloration. This study compared the efficacy of the new phosphodiesterase type 5 inhibitor udenafil with that of the calcium channel blocker amlodipine in the treatment of secondary RP. METHODS: A total of 29 patients with secondary RP associated with connective tissue diseases were enrolled in this double-blind, randomized, cross-over study. The patients were randomized to receive udenafil 100 mg/day or amlodipine 10 mg/day for 4 weeks. After a washout period they were crossed over to the other drug for another 4 weeks. The primary outcome was RP frequency before and after treatment. The secondary outcomes were RP condition scores, RP duration, number of digital ulcers, HAQ, physician global assessment and digital artery flow before and after treatment. RESULTS: Amlodipine and udenafil both decreased the rate of RP attack significantly. The drugs did not differ in terms of RP frequency or any of the secondary outcomes except for digital blood flow; udenafil improved it significantly better than amlodipine (P = 0.021). Udenafil was well tolerated without serious adverse effects. CONCLUSION: Udenafil and amlodipine have comparable efficacy in improving RP attacks. In addition, udenafil improves the blood flow in digital arteries compared with amlodipine. TRIAL REGISTRATION: www.clinicaltrials.gov, protocol number NCT01280266. FAU - Lee, Eun Young AU - Lee EY AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea. leb7616@snu.ac.kr. FAU - Park, Jin Kyun AU - Park JK FAU - Lee, Whal AU - Lee W FAU - Kim, Yeo Koon AU - Kim YK FAU - Park, Claire Su-Yeon AU - Park CS FAU - Giles, Jon T AU - Giles JT FAU - Park, Jun Won AU - Park JW FAU - Shin, Kichul AU - Shin K FAU - Lee, Jeong Seok AU - Lee JS FAU - Song, Yeong Wook AU - Song YW FAU - Lee, Eun Bong AU - Lee EB LA - eng SI - ClinicalTrials.gov/NCT01280266 PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20131217 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 1J444QC288 (Amlodipine) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Pyrimidines) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - L5IB4XLY36 (udenafil) RN - 0 (Benzenesulfonamides) SB - IM MH - Adult MH - Amlodipine/*therapeutic use MH - Connective Tissue Diseases/complications MH - Cross-Over Studies MH - Double-Blind Method MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Pyrimidines/*therapeutic use MH - Raynaud Disease/*drug therapy/etiology MH - Sulfonamides/*therapeutic use MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use MH - Benzenesulfonamides OTO - NOTNLM OT - Raynaud’s phenomenon OT - amlodipine OT - digital blood flow OT - udenafil OT - vasodilator EDAT- 2013/12/20 06:00 MHDA- 2014/05/08 06:00 CRDT- 2013/12/20 06:00 PHST- 2013/12/20 06:00 [entrez] PHST- 2013/12/20 06:00 [pubmed] PHST- 2014/05/08 06:00 [medline] AID - ket417 [pii] AID - 10.1093/rheumatology/ket417 [doi] PST - ppublish SO - Rheumatology (Oxford). 2014 Apr;53(4):658-64. doi: 10.1093/rheumatology/ket417. Epub 2013 Dec 17. PMID- 17349450 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20071203 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 120 IP - 3 DP - 2007 Mar TI - Smoking, alcohol consumption, and Raynaud's phenomenon in middle age. PG - 264-71 AB - BACKGROUND: Data suggest Raynaud's phenomenon shares risk factors with cardiovascular disease. Studies of smoking, alcohol consumption, and Raynaud's have produced conflicting results and were limited by small sample size and failure to adjust for confounders. Our objective was to determine whether smoking and alcohol are independently associated with Raynaud's in a large, community-based cohort. METHODS: By using a validated survey to classify Raynaud's in the Framingham Heart Study Offspring Cohort, we performed sex-specific analyses of Raynaud's status by smoking and alcohol consumption in 1840 women and 1602 men. Multivariable logistic regression analyses were used to examine the relationship of Raynaud's to smoking and alcohol consumption. RESULTS: Current smoking was not associated with Raynaud's in women but was associated with increased risk in men (adjusted odds ratio [OR] 2.59, 95% confidence interval [CI], 1.11-6.04). Heavy alcohol consumption in women was associated with increased risk of Raynaud's (adjusted OR 1.69, 95% CI, 1.02-2.82), whereas moderate alcohol consumption in men was associated with reduced risk (adjusted OR 0.51, 95% CI, 0.29-0.89). In both genders, red wine consumption was associated with a reduced risk of Raynaud's (adjusted OR 0.59, 95% CI, 0.36-0.96 in women and adjusted OR 0.30, 95% CI, 0.15-0.62 in men). CONCLUSIONS: Our data suggest that middle-aged women and men may have distinct physiologic mechanisms underlying their Raynaud's, and thus sex-specific therapeutic approaches may be appropriate. Our data also support the possibility that moderate red wine consumption may protect against Raynaud's. FAU - Suter, Lisa G AU - Suter LG AD - VA Connecticut Healthcare System, West Haven, Conn, USA. lisa.suter@yale.edu FAU - Murabito, Joanne M AU - Murabito JM FAU - Felson, David T AU - Felson DT FAU - Fraenkel, Liana AU - Fraenkel L LA - eng GR - AR47785/AR/NIAMS NIH HHS/United States GR - K23 AR048826-01/AR/NIAMS NIH HHS/United States GR - N01-HC-25195/HC/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 SB - IM MH - Age Factors MH - Alcohol Drinking/*epidemiology MH - Cardiovascular Diseases/epidemiology/prevention & control MH - Cohort Studies MH - Female MH - Follow-Up Studies MH - Health Surveys MH - Humans MH - Incidence MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Raynaud Disease/diagnosis/*epidemiology MH - Severity of Illness Index MH - Sex Factors MH - Smoking/*epidemiology MH - United States/epidemiology EDAT- 2007/03/14 09:00 MHDA- 2007/03/31 09:00 CRDT- 2007/03/14 09:00 PHST- 2006/01/15 00:00 [received] PHST- 2006/05/24 00:00 [revised] PHST- 2006/06/01 00:00 [accepted] PHST- 2007/03/14 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2007/03/14 09:00 [entrez] AID - S0002-9343(06)00686-3 [pii] AID - 10.1016/j.amjmed.2006.06.007 [doi] PST - ppublish SO - Am J Med. 2007 Mar;120(3):264-71. doi: 10.1016/j.amjmed.2006.06.007. PMID- 26471183 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20181113 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 36 IP - 2 DP - 2016 Feb TI - Intermediate-term follow-up of chronically ill patients with digital ischemia treated with peripheral digital sympathectomy. PG - 301-7 AB - Digital ischemia is commonly found in patients with scleroderma and has been shown to respond to peripheral digital sympathectomy. While favorable long- and intermediate-term results have been documented in the literature, minimal objective data are available and the mechanism of surgical sympathectomy has not been entirely elucidated. Patients with digital ischemia secondary to Raynaud's phenomenon that had undergone peripheral sympathectomy surgery between 2001 and 2009 were identified and contacted for participation. Radial artery Doppler ultrasound studies were performed and compared to those done at the time of their sympathectomy. Of 11 patients treated over a 9-year period, only two patients were available for detailed follow-up analysis. Four patients were deceased, and two were lost to follow-up. Four of the five remaining patients reported excellent use of the hand and no significant episodes of digital ischemia. Of the two patients studied, functional results were favorable and pain was markedly improved despite worsening of the digital flow resistance over time. We conclude that peripheral digital sympathectomy may provide favorable long-term results in patients with digital ischemia from autoimmune causes, although this intervention should be considered in the early stages once ischemic symptoms manifest. Interestingly, Doppler data did not appear to correlate with functional status and symptom severity in these two patients. Further research, particularly prospective studies, is warranted to guide clinical decisions in this patient population. FAU - Soberón, José R Jr AU - Soberón JR Jr FAU - Greengrass, Roy A AU - Greengrass RA FAU - Davis, William E AU - Davis WE FAU - Murray, Peter M AU - Murray PM FAU - Feinglass, Neil AU - Feinglass N LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Chronic Disease MH - Female MH - Fingers/*blood supply/*innervation MH - Follow-Up Studies MH - Humans MH - Ischemia/diagnosis/etiology/physiopathology/*surgery MH - Middle Aged MH - Radial Artery/physiopathology MH - Raynaud Disease/*complications/diagnosis/physiopathology MH - Recovery of Function MH - Regional Blood Flow MH - Sympathectomy/*methods MH - *Sympathectomy, Chemical MH - Time Factors MH - Treatment Outcome MH - Ultrasonography, Doppler EDAT- 2015/10/17 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/10/17 06:00 PHST- 2015/08/17 00:00 [received] PHST- 2015/10/07 00:00 [accepted] PHST- 2015/10/17 06:00 [entrez] PHST- 2015/10/17 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1007/s00296-015-3383-1 [pii] AID - 10.1007/s00296-015-3383-1 [doi] PST - ppublish SO - Rheumatol Int. 2016 Feb;36(2):301-7. doi: 10.1007/s00296-015-3383-1. PMID- 16841869 OWN - NLM STAT- MEDLINE DCOM- 20061109 LR - 20071115 IS - 1232-1966 (Print) IS - 1232-1966 (Linking) VI - 13 IP - 1 DP - 2006 TI - Influence on operator's health of hand-transmitted vibrations from handles of a single-axle tractor. PG - 33-8 AB - The operators of the single-axle tractors are especially exposed to hand-arm transmitted vibrations. These vibrations can cause the complex of vascular, neurological and musculoskeletal disorders, collectively named hand-arm vibration syndrome. Among these, the most common disorder is vibration-induced white finger (Raynaud's phenomenon). The vibration levels were measured in three tractor's working conditions, namely idling, transportation and soil tillage. The vibration level on the handles was measured and analysed and the frequency spectra for the chosen working conditions were obtained. The frequency-weighted acceleration, given in m/s2, was calculated and the obtained values are graphically presented. The measured vibration levels are then discussed with regard to the operator's daily exposure limits recommended by the ISO 5349. The vibration levels were much higher in the x and y directions than the z-direction in all working conditions. The vibration total values in idling, transportation and soil tillage were 3.37, 8.37 and 9.62 m/s2, respectively. Results showed that the 10% of workers are exposed to a risk of vibration-induced white finger disorder of the hands after relatively short periods (3-4 years), if the tractor is used 8 hour per day in soil tillage and transportation at full load. Considering the criteria of the ISO 5349, the daily working time with the single-axle tractor should be limited in order to protect the operator and work schedules should be arranged to include vibration-free periods. FAU - Goglia, Vlado AU - Goglia V AD - Faculty of Forestry, Svetosimunska 25, 10000 Zagreb, Croatia. goglia@hrast.sumfak.hr FAU - Gospodaric, Zlatko AU - Gospodaric Z FAU - Filipovic, Dubravko AU - Filipovic D FAU - Djukic, Igor AU - Djukic I LA - eng PT - Journal Article PL - Poland TA - Ann Agric Environ Med JT - Annals of agricultural and environmental medicine : AAEM JID - 9500166 SB - IM MH - Acceleration/adverse effects MH - Agriculture MH - Croatia MH - Cumulative Trauma Disorders/epidemiology/*etiology/prevention & control MH - Guidelines as Topic MH - Humans MH - *Occupational Exposure MH - *Occupational Health MH - Off-Road Motor Vehicles MH - Prevalence MH - Raynaud Disease/epidemiology/*etiology/prevention & control MH - Time Factors MH - Vibration/*adverse effects EDAT- 2006/07/18 09:00 MHDA- 2006/11/11 09:00 CRDT- 2006/07/18 09:00 PHST- 2006/07/18 09:00 [pubmed] PHST- 2006/11/11 09:00 [medline] PHST- 2006/07/18 09:00 [entrez] AID - 13033 [pii] PST - ppublish SO - Ann Agric Environ Med. 2006;13(1):33-8. PMID- 18651053 OWN - NLM STAT- MEDLINE DCOM- 20081103 LR - 20191111 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 60 IP - 2 DP - 2008 Apr-Jun TI - [Role of videocapillaroscopy in early detection of transition from primary to secondary Raynaud's phenomenon in systemic sclerosis]. PG - 102-7 AB - Patients initially diagnosed as having primary Raynaud's phenomenon (PRP) may shift to secondary (SRP) during the follow-up. Nailfold videocapillaroscopy (NVC) is a tool that allows to distinguish between PRP and SRP through the identification of the "early" scleroderma-pattern of microangiopathy. The aim of this study was to evaluate the transition from PRP to SRP in an Italian cohort of patients during their follow-up. 129 patients with PRP were identified and followed-up for 2721 months. The diagnosis of PRP was achieved as suggested by LeRoy. The NVC diagnosis of scleroderma-pattern was based on the presence of specific "early" capillary abnormalities (i.e. giant capillaries, microhaemorrhages, and/or slight reduction of capillary density). Based on the identification of the "early" scleroderma-pattern by NVC, 14% of patients changed from PRP to SRP during the follow-up. Interestingly, 4.6% of these patients showed at baseline a fully normal NVC pattern (transition from normal to scleroderma NVC pattern in 3427 months), and 10% showed slight and not-specific nailfold capillary abnormalities (i.e. dystrophic capillaries and/or enlarged capillaries) at baseline (transition to scleroderma NVC pattern in 2515 months). Following a careful NVC analysis, we showed the progression from PRP to SRP in 14% of the analyzed patients. We suggest the capillaroscopic analysis twice a year in presence of PRP, in order to early detect the transition to SRP in patients showing at the beginning a normal pattern or not-specific nailfold capillary abnormalities, as assessed by NVC. FAU - Secchi, M E AU - Secchi ME AD - U.O.C Clinica Reumatologica, Dipartimento di Medicina Interna, Università degli Studi di Genova, Azienda Ospedaliera Universitaria San Martino, Genoa, Italy. FAU - Sulli, A AU - Sulli A FAU - Grollero, M AU - Grollero M FAU - Pizzorni, C AU - Pizzorni C FAU - Parodi, M AU - Parodi M FAU - Paolino, S AU - Paolino S FAU - Seriolo, B AU - Seriolo B FAU - Cutolo, M AU - Cutolo M LA - ita PT - English Abstract PT - Journal Article TT - Ruolo della videocapillaroscopia periungueale nella identificazione precoce della progressione del fenomeno di Raynaud da primario a secondario nella sclerosi sistemica. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Aged MH - Disease Progression MH - Early Diagnosis MH - Humans MH - *Microscopic Angioscopy/methods MH - Raynaud Disease/*etiology MH - Scleroderma, Systemic/*complications MH - *Video Recording EDAT- 2008/07/25 09:00 MHDA- 2008/11/04 09:00 CRDT- 2008/07/25 09:00 PHST- 2008/07/25 09:00 [pubmed] PHST- 2008/11/04 09:00 [medline] PHST- 2008/07/25 09:00 [entrez] AID - 10.4081/reumatismo.2008.102 [doi] PST - ppublish SO - Reumatismo. 2008 Apr-Jun;60(2):102-7. doi: 10.4081/reumatismo.2008.102. PMID- 42127451 OWN - NLM STAT- MEDLINE DCOM- 20260530 LR - 20260530 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 117 DP - 2026 Jun TI - A proof of concept case series of botulinum toxin a treatment for ischemic feet due to Raynaud's phenomenon. PG - 222-225 LID - S1748-6815(26)00245-7 [pii] LID - 10.1016/j.bjps.2026.04.028 [doi] AB - Botulinum Toxin A (BTX-A) has shown benefit in treating Raynaud's phenomenon (RP) of the hands, but its use in the feet remains largely unreported. We present a retrospective case series of seven patients undergoing 11 BTX-A treatment sessions for refractory ischemic RP of the feet. Injections (100-150 units per foot) were administered under ultrasound guidance. At six weeks, 81.8% of treatment episodes demonstrated resolution of ulcers and/or gangrene. Pain significantly improved (p=0.013), with the proportion of pain-free cases increasing from 9.1% to 63.6%. One transient adverse event was observed. These findings suggest that BTX-A may represent a safe and promising treatment option for ischemic RP of the feet, warranting further investigation in prospective studies. CI - Copyright © 2026 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Stadelmeier, L F AU - Stadelmeier LF AD - Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. Electronic address: adowlats@bidmc.harvard.edu. FAU - Eeuwen, J E AU - Eeuwen JE AD - Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. FAU - Engmann, T F AU - Engmann TF AD - Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. FAU - Hilbig-Vlatten, L AU - Hilbig-Vlatten L AD - Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. FAU - Carroll, B J AU - Carroll BJ AD - Department of Cardiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. FAU - Nashel, J AU - Nashel J AD - Department of Rheumatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. FAU - Dowlatshahi, A S AU - Dowlatshahi AS AD - Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States. LA - eng PT - Journal Article DEP - 20260505 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Neuromuscular Agents) SB - IM MH - Humans MH - *Botulinum Toxins, Type A/therapeutic use/administration & dosage MH - *Raynaud Disease/complications MH - Female MH - *Ischemia/drug therapy/etiology MH - Retrospective Studies MH - Middle Aged MH - Male MH - *Foot/blood supply MH - *Neuromuscular Agents/therapeutic use/administration & dosage MH - Aged MH - Treatment Outcome MH - Adult OTO - NOTNLM OT - Botulinum Toxin A OT - Ischemic Feet OT - Raynaud’s Phenomenon OT - Vasospasm OT - Wound Healing COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/05/13 18:30 MHDA- 2026/05/31 05:33 CRDT- 2026/05/13 18:00 PHST- 2026/01/20 00:00 [received] PHST- 2026/04/20 00:00 [revised] PHST- 2026/04/29 00:00 [accepted] PHST- 2026/05/31 05:33 [medline] PHST- 2026/05/13 18:30 [pubmed] PHST- 2026/05/13 18:00 [entrez] AID - S1748-6815(26)00245-7 [pii] AID - 10.1016/j.bjps.2026.04.028 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2026 Jun;117:222-225. doi: 10.1016/j.bjps.2026.04.028. Epub 2026 May 5. PMID- 36822366 OWN - NLM STAT- MEDLINE DCOM- 20230613 LR - 20230614 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 148 DP - 2023 Jul TI - Comparison of different forms of Raynaud's phenomenon by LASCA proximal-distal gradient perfusion. PG - 104509 LID - S0026-2862(23)00035-3 [pii] LID - 10.1016/j.mvr.2023.104509 [doi] AB - OBJECTIVE: To evaluate finger proximal-distal gradient (PDG) perfusion in subjects with primary Raynaud's phenomenon (PRP), then making comparisons with systemic sclerosis (SSc) patients and healthy controls (HC). METHODS: Consecutive adult PRP subjects were enrolled, along with an equal number of SSc and HC. Peripheral blood perfusion of the hands was assessed by laser speckle contrast analysis (LASCA). PDG was then calculated applying a generalizable formula independent of both intra- and inter-personal factors. Non-specific anti-nuclear autoantibody (ANA) isolated positivity was assessed. RESULTS: Fifty PRP patients (88 % female, mean age 45 ± 17.9 years) were enrolled, along with 50 SSc patients and 50 HC. After adjusting mean PDG results for age and sex, no significant differences emerged between PRP and SSc (1.80 ± 0.43 vs 1.76 ± 0.53; p = 0.294). Conversely, PRP values were significantly reduced when compared to HC (2.72 ± 0.37; p < 0.001). Among PRP subjects, no significant differences were found regarding isolated ANA positivity (1.86 ± 0.44 vs 1.74 ± 0.44; p = 0.42). CONCLUSION: PRP and SSc seems to share the same basal PDG perfusion impairment assessed by LASCA. Isolated ANA positivity, in the absence of clinical and capillaroscopic suspicion for secondary causes, should not be considered an exclusion criterion for PRP classification. CI - Copyright © 2023 Elsevier Inc. All rights reserved. FAU - Di Battista, Marco AU - Di Battista M AD - Rheumatology Unit, University of Pisa, Pisa, Italy; Department of Medical Biotechnologies, University of Siena, Siena, Italy. Electronic address: dibattista.marco91@gmail.com. FAU - Morganti, Riccardo AU - Morganti R AD - Section of Statistics, University of Pisa, Pisa, Italy. FAU - Da Rio, Mattia AU - Da Rio M AD - Rheumatology Unit, University of Pisa, Pisa, Italy. FAU - De Mattia, Giammarco AU - De Mattia G AD - Rheumatology Unit, University of Pisa, Pisa, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Rheumatology Unit, University of Pisa, Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, University of Pisa, Pisa, Italy. LA - eng PT - Journal Article DEP - 20230221 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Humans MH - Female MH - Middle Aged MH - Male MH - Skin MH - Regional Blood Flow MH - *Scleroderma, Systemic/diagnosis MH - Perfusion MH - *Raynaud Disease/diagnosis MH - Lasers OTO - NOTNLM OT - Laser speckle contrast analysis OT - Microangiopathy OT - Peripheral perfusion OT - Raynaud's phenomenon OT - Systemic sclerosis COIS- Declaration of competing interest There are no conflicts of interest to declare. EDAT- 2023/02/24 06:00 MHDA- 2023/06/13 06:42 CRDT- 2023/02/23 19:29 PHST- 2023/01/17 00:00 [received] PHST- 2023/02/12 00:00 [revised] PHST- 2023/02/17 00:00 [accepted] PHST- 2023/06/13 06:42 [medline] PHST- 2023/02/24 06:00 [pubmed] PHST- 2023/02/23 19:29 [entrez] AID - S0026-2862(23)00035-3 [pii] AID - 10.1016/j.mvr.2023.104509 [doi] PST - ppublish SO - Microvasc Res. 2023 Jul;148:104509. doi: 10.1016/j.mvr.2023.104509. Epub 2023 Feb 21. PMID- 27722973 OWN - NLM STAT- MEDLINE DCOM- 20180329 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 36 IP - 7 DP - 2017 Jul TI - Primary Raynaud's phenomenon and nailfold videocapillaroscopy: age-related changes in capillary morphology. PG - 1637-1642 LID - 10.1007/s10067-016-3442-3 [doi] AB - This study aimed to detect by nailfold videocapillaroscopy (NVC) the presence of age-related capillary morphological patterns in a large cohort of subjects affected by primary Raynaud's phenomenon (PRP). NVC was performed in 877 patients affected by PRP, divided into three age groups: <35, 35-55 and >55 years. The following qualitative parameters were assessed and compared in the three groups of patients: apical dilations, irregular (non-homogeneous) dilations, venous branch dilations, microhaemorrhages, tortuosities and subpapillary venous plexus visibility. Patients with either irregular dilations or venous branch dilations were found significantly younger than those without (p < 0.0001). The presence of either irregular or venous branch dilations seems to exclude the presence of apical dilations. Patients with microhaemorrhages were found significantly younger than those without (p = 0.05), and 81 % of patients without microhaemorrhages did not show irregular and venous branch dilations. The subpapillary venous plexus seems more visible in subjects with age < 35, as well as in those with age > 55 years (p < 0.0001). A statistically significant negative correlation was found between presence of apical and irregular dilations (p < 0.0001), apical dilations and venous branch dilations (p = 0.02), apical dilations and tortuosities (p = 0.0005), microhaemorrhages and tortuosities (p < 0.0001) and venous branch dilations and tortuosities (p = 0.02). Finally, a statistically significant positive correlation was found between irregular and venous branch dilations (p < 0.0001), irregular dilations and microhaemorrhages (p < 0.0001) and venous branch dilations and microhaemorrhages (p < 0.0001). In conclusion, our study detected different age-related morphological capillary changes mainly in younger patients with PRP, as well as statistically significant correlations between the presence of different capillary variables. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. carmen.pizzorni@unige.it. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Department of Internal Medicine, Ghent University Hospital, Ghent University, Ghent, Belgium. FAU - Ruaro, Barbara AU - Ruaro B AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. FAU - Trombetta, Amelia Chiara AU - Trombetta AC AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratories and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino, University of Genova, Viale Benedetto XV, no. 6, 16132, Genoa, Italy. LA - eng PT - Journal Article DEP - 20161008 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Age Factors MH - Capillaries/*pathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*pathology OTO - NOTNLM OT - Aging OT - Capillary morphology OT - Connective tissue diseases OT - Nailfold videocapillaroscopy OT - Primary Raynaud’s phenomenon EDAT- 2016/10/11 06:00 MHDA- 2018/03/30 06:00 CRDT- 2016/10/11 06:00 PHST- 2016/07/12 00:00 [received] PHST- 2016/09/29 00:00 [accepted] PHST- 2016/09/26 00:00 [revised] PHST- 2016/10/11 06:00 [pubmed] PHST- 2018/03/30 06:00 [medline] PHST- 2016/10/11 06:00 [entrez] AID - 10.1007/s10067-016-3442-3 [pii] AID - 10.1007/s10067-016-3442-3 [doi] PST - ppublish SO - Clin Rheumatol. 2017 Jul;36(7):1637-1642. doi: 10.1007/s10067-016-3442-3. Epub 2016 Oct 8. PMID- 16410532 OWN - NLM STAT- MEDLINE DCOM- 20060223 LR - 20250214 IS - 0003-4967 (Print) IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 65 IP - 2 DP - 2006 Feb TI - Colour duplex sonography of finger arteries in vasculitis and in systemic sclerosis. PG - 265-7 AB - CASE REPORTS: Three patients-two with Wegener's granulomatosis and one with an overlap syndrome of rheumatoid vasculitis, systemic lupus erythematosus, and antiphospholipid syndrome-are described. All patients experienced a sudden onset of Raynaud's phenomenon or acrocyanosis when they had a flare of their disease. DISCUSSION: Ultrasonography (US) showed dark (hypoechoic) arteries without colour signals, resembling the US pattern of embolism. In contrast, US in patients with systemic sclerosis is entirely different, delineating a smaller artery lumen, reduced pulsation, and thickened, slightly hyperechoic artery walls. FAU - Schmidt, W A AU - Schmidt WA AD - Medical Centre for Rheumotology Berlin-Buch, Karower Str 11, 13125, Berlin, Germany. w.schmidt@immanuel.de FAU - Wernicke, D AU - Wernicke D FAU - Kiefer, E AU - Kiefer E FAU - Gromnica-Ihle, E AU - Gromnica-Ihle E LA - eng PT - Case Reports PT - Comparative Study PT - Journal Article PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Acute Disease MH - Adult MH - Antiphospholipid Syndrome/diagnostic imaging/physiopathology MH - Arterial Occlusive Diseases/diagnostic imaging/physiopathology MH - Arteries/*diagnostic imaging MH - Collateral Circulation MH - Female MH - Fingers/*blood supply MH - Granulomatosis with Polyangiitis/diagnostic imaging/physiopathology MH - Hemorrhage/diagnostic imaging/physiopathology MH - Humans MH - Male MH - Nail Diseases/diagnostic imaging/physiopathology MH - Raynaud Disease/diagnostic imaging/physiopathology MH - Scleroderma, Systemic/*diagnostic imaging/physiopathology MH - *Ultrasonography, Doppler, Color MH - *Ultrasonography, Doppler, Duplex MH - Vasculitis/*diagnostic imaging/physiopathology PMC - PMC1798001 COIS- Conflict of interest: None. EDAT- 2006/01/18 09:00 MHDA- 2006/02/24 09:00 PMCR- 2009/02/01 CRDT- 2006/01/18 09:00 PHST- 2006/01/18 09:00 [pubmed] PHST- 2006/02/24 09:00 [medline] PHST- 2006/01/18 09:00 [entrez] PHST- 2009/02/01 00:00 [pmc-release] AID - S0003-4967(24)20306-X [pii] AID - ar39149 [pii] AID - 10.1136/ard.2005.039149 [doi] PST - ppublish SO - Ann Rheum Dis. 2006 Feb;65(2):265-7. doi: 10.1136/ard.2005.039149. PMID- 18077495 OWN - NLM STAT- MEDLINE DCOM- 20080110 LR - 20151119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 1 DP - 2008 Jan TI - Cold stimulus fingertip lacticemy test--an effective method to monitor acute therapeutic intervention on primary Raynaud's phenomenon and systemic sclerosis. PG - 80-3 AB - OBJECTIVES: The recently developed cold stimulus fingertip lacticemy test (CS-FTL) provides biochemical assessment of peripheral perfusion in patients with Raynaud's phenomenon (RP). We evaluated how the CS-FTL test can assess the acute effect of nifedipine in microvascular dynamics on primary RP and RP secondary to SSc. METHODS: A double-blinded controlled trial with crossover design was performed in 20 primary RP and 20 SSc patients. Patients received one single sublingual placebo or 10 mg nifedipine capsule, with crossover after a 15-day washout period. FTL was determined in resting conditions (pre-CS-FTL) and 10 min after CS (post-CS-FTL), before and 1 h after drug administration. Percent variation in post- vs pre-CS-FTL was expressed as deltaCS-FTL. RESULTS: Before intervention, CS induced FTL decrease in primary RP (deltaCS-FTL = -21.3 +/- 13.0%) and FTL increase in SSc patients (deltaCS-FTL = +24.5 +/- 21.2%). Placebo had no effect on pre-CS-FTL, post-CS-FTL and deltaCS-FTL in primary RP and SSc. Nifedipine induced a significant decrease in pre-CS-FTL (1.94 +/- 0.45 vs 1.57 +/- 0.41 mg/dl; P = 0.005) and post-CS-FTL (1.53 +/- 0.35 vs 1.32 +/- 0.37 mg/dl; P = 0.004) in primary RP and a significant decrease in post-CS-FTL (3.18 +/- 1.43 vs 2.56 +/- 1.30 mg/dl; P = 0.028) and deltaCS-FTL (+15.9 +/- 24.7% vs -12.9 +/- 16.6%; P = 0.001) in SSc. CONCLUSIONS: The CS-FTL test was able to demonstrate and quantify a dual effect of nifedipine on the biochemical dimension of peripheral perfusion in primary RP and in SSc patients in which there was a significant improvement in tissue perfusion in resting conditions and after exposure to a CS. The CS-FTL test will enrich the armamentarium for investigation and clinical evaluation of conditions associated with RP. FAU - Fontenelle, S M A AU - Fontenelle SM AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, SP 04023-062, Brazil. FAU - Kayser, C AU - Kayser C FAU - Pucinelli, M L C AU - Pucinelli ML FAU - Andrade, L E C AU - Andrade LE LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) RN - 33X04XA5AT (Lactic Acid) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Administration, Sublingual MH - Adult MH - *Cold Temperature MH - Cross-Over Studies MH - Double-Blind Method MH - Drug Monitoring/*methods MH - Female MH - Fingers/blood supply/*pathology MH - Humans MH - Lactic Acid/blood MH - Male MH - Microcirculation/drug effects/pathology/physiopathology MH - Middle Aged MH - Nifedipine/*therapeutic use MH - Raynaud Disease/blood/*diagnosis/drug therapy/etiology MH - Scleroderma, Diffuse/blood/complications/diagnosis/drug therapy MH - Scleroderma, Localized/blood/complications/diagnosis/drug therapy MH - Scleroderma, Systemic/blood/complications/*diagnosis/drug therapy MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use EDAT- 2007/12/14 09:00 MHDA- 2008/01/11 09:00 CRDT- 2007/12/14 09:00 PHST- 2007/12/14 09:00 [pubmed] PHST- 2008/01/11 09:00 [medline] PHST- 2007/12/14 09:00 [entrez] AID - 47/1/80 [pii] AID - 10.1093/rheumatology/kem300 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Jan;47(1):80-3. doi: 10.1093/rheumatology/kem300. PMID- 17968938 OWN - NLM STAT- MEDLINE DCOM- 20071219 LR - 20250529 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 11 DP - 2007 Nov TI - Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. PG - 3837-46 AB - OBJECTIVE: To evaluate the efficacy and tolerability of prolonged administration of quinapril, a long-acting angiotensin-converting enzyme inhibitor, in the management of the peripheral vascular manifestations of limited cutaneous systemic sclerosis (lcSSc) and in the prevention of the progression of visceral organ involvement in the disease. METHODS: This was a multicenter, randomized, double-blind, placebo-controlled study evaluating quinapril 80 mg/day, or the maximum tolerated dosage, in 210 patients with lcSSc or with Raynaud's phenomenon (RP) and the presence of SSc-specific antinuclear antibodies. Treatment was for 2-3 years. The primary outcome measure was the number of new ischemic ulcers appearing on the hands; secondary measures were the frequency and severity of RP attacks, skin score, treatments for ischemia, health status (measured by the Short Form 36 instrument), measures of kidney and lung function, and echocardiographic estimates of pulmonary artery pressure. An intent-to-treat analysis was used. RESULTS: Quinapril did not affect the occurrence of digital ulcers or the frequency or severity of RP episodes. It did not alter the treatments that were prescribed for either infected ulcers or severe RP symptoms. There was no apparent effect on the estimated tricuspid gradient. Health status was not affected by quinapril, and one-half of the patients who believed they had benefited from the trial treatment were in the placebo arm. Quinapril was not tolerated by one-fifth of the patients, with dry cough being the most frequent side effect. CONCLUSION: Administration of quinapril for up to 3 years had no demonstrable effects on the occurrence of upper limb digital ulcers or on other vascular manifestations of lcSSc in this patient population. FAU - Gliddon, A E AU - Gliddon AE AD - University of Wales, Bangor, UK. FAU - Doré, C J AU - Doré CJ FAU - Black, C M AU - Black CM FAU - McHugh, N AU - McHugh N FAU - Moots, R AU - Moots R FAU - Denton, C P AU - Denton CP FAU - Herrick, A AU - Herrick A FAU - Barnes, T AU - Barnes T FAU - Camilleri, J AU - Camilleri J FAU - Chakravarty, K AU - Chakravarty K FAU - Emery, P AU - Emery P FAU - Griffiths, B AU - Griffiths B FAU - Hopkinson, N D AU - Hopkinson ND FAU - Hickling, P AU - Hickling P FAU - Lanyon, P AU - Lanyon P FAU - Laversuch, C AU - Laversuch C FAU - Lawson, T AU - Lawson T FAU - Mallya, R AU - Mallya R FAU - Nisar, M AU - Nisar M FAU - Rhys-Dillon, C AU - Rhys-Dillon C FAU - Sheeran, T AU - Sheeran T FAU - Maddison, P J AU - Maddison PJ LA - eng SI - ISRCTN/ISRCTN57984794 GR - G0600404/MRC_/Medical Research Council/United Kingdom GR - MC_U122886349/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Placebos) RN - 0 (Tetrahydroisoquinolines) RN - RJ84Y44811 (Quinapril) SB - IM MH - Adult MH - Aged MH - Angiotensin-Converting Enzyme Inhibitors/*administration & dosage/adverse effects MH - Disease Progression MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Placebos MH - Quinapril MH - Raynaud Disease/*drug therapy/immunology/prevention & control MH - Scleroderma, Limited/*drug therapy/immunology MH - Tetrahydroisoquinolines/*administration & dosage/adverse effects MH - Treatment Outcome EDAT- 2007/10/31 09:00 MHDA- 2007/12/20 09:00 CRDT- 2007/10/31 09:00 PHST- 2007/10/31 09:00 [pubmed] PHST- 2007/12/20 09:00 [medline] PHST- 2007/10/31 09:00 [entrez] AID - 10.1002/art.22965 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Nov;56(11):3837-46. doi: 10.1002/art.22965. PMID- 21901350 OWN - NLM STAT- MEDLINE DCOM- 20130220 LR - 20220311 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 10 DP - 2012 Oct TI - Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow-up study of 3,029 patients with primary Raynaud's phenomenon. PG - 3039-45 AB - To assess the prognostic value of scleroderma pattern of nailfold capillary changes for the development of connective tissue diseases (CTD) in subjects with primary Raynaud's phenomenon (RP). The study included 3,029 consecutive patients with primary RP who had been followed at 6-month intervals during the mean of 4.8 years. The pathological features of nailfold capillaroscopy were recorded in all patients who had neither clinical nor serological signs of a CTD. In patients who developed CTD, capillary changes obtained 6 months prior to diagnosis were analyzed. A possible relationship between capillary changes and the presence of associated CTD was assessed. At the end of follow-up, 1,660 (54,8%) patients have still the primary RP, 246 (8,1%) had suspected secondary RP, and 1,123 (37,1%) patients developed CTD (363 undifferentiated CTD, 263 systemic sclerosis, 143 systemic lupus erythematosus, 106 rheumatoid arthritis, 102 Sjögren's syndrome, 61 overlap syndrome, 30 vasculitides, 24 mixed CTD, 19 polymyositis, 7 dermatomyositis, and 5 primary antiphospholipid syndrome). Scleroderma pattern were significantly associated with the development of systemic sclerosis [P = .00001, sensitivity 94%, specificity 92%, positive predictive value 52%, negative predictive value 99%, and odds ratio 163 (95% CI, 97,9-271,5)], as well as dermatomyositis (P = .0004), overlap syndrome with signs of systemic sclerosis (P = .0001), and mixed connective tissue disease (P = .007). Capillary microscopy is effective method for differentiation between primary and secondary RP and useful tool for the prediction of scleroderma spectrum disorders in RP patients. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AD - Institute of Rheumatology, Belgrade, Serbia. dolijan@eunet.rs FAU - Damjanov, Nemanja S AU - Damjanov NS FAU - Stojanovic, Roksanda M AU - Stojanovic RM FAU - Vujasinovic Stupar, Nada Z AU - Vujasinovic Stupar NZ FAU - Stanisavljevic, Dejana M AU - Stanisavljevic DM LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110908 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Capillaries/*pathology MH - Chi-Square Distribution MH - Child MH - Connective Tissue Diseases/*etiology/pathology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Odds Ratio MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/*complications/pathology MH - Risk Assessment MH - Risk Factors MH - Scleroderma, Systemic/*etiology/pathology MH - Sensitivity and Specificity MH - Time Factors MH - Young Adult EDAT- 2011/09/09 06:00 MHDA- 2013/02/21 06:00 CRDT- 2011/09/09 06:00 PHST- 2011/03/29 00:00 [received] PHST- 2011/08/22 00:00 [accepted] PHST- 2011/09/09 06:00 [entrez] PHST- 2011/09/09 06:00 [pubmed] PHST- 2013/02/21 06:00 [medline] AID - 10.1007/s00296-011-2109-2 [doi] PST - ppublish SO - Rheumatol Int. 2012 Oct;32(10):3039-45. doi: 10.1007/s00296-011-2109-2. Epub 2011 Sep 8. PMID- 36385255 OWN - NLM STAT- MEDLINE DCOM- 20230130 LR - 20230415 IS - 1600-0846 (Electronic) IS - 0909-752X (Print) IS - 0909-752X (Linking) VI - 29 IP - 1 DP - 2023 Jan TI - Role of short courses on nailfold capillaroscopy in obtaining abilities for the identification of microvasculopathy in patients with Raynaud's phenomenon. PG - e13223 LID - 10.1111/srt.13223 [doi] LID - e13223 AB - INTRODUCTION: An early distinction between "normal" and "abnormal" capillaroscopic pattern during the first visit to a dermatologist has a crucial significance for a diagnostic management of Raynaud's phenomenon (RP). There exists a question about the level of expertise sufficient to evaluate the microcirculation. AIM: To evaluate the utility of short courses on NFC among dermatologists and medical students in obtaining sufficient abilities for the identification of microvasculopathy in patients with RP using videocapillaroscope and handheld dermoscope. METHODS: Both groups participated in 1-h course on NFC. Before the course, participants were asked to classify 20 videocapillaroscopic and 10 dermoscopic capillaroscopic pictures into "normal" or "abnormal" pattern. Each picture was displayed on a separate slide MS PowerPoint for 10 s. The evaluation was repeated soon after the course. RESULTS: A total of 36 dermatologists and 49 medical students were enrolled. The rate of properly classified dermoscopic and videodermoscopic pictures increased after the course in both groups, but students improved the accuracy of classification on dermoscopic pictures to the greater extent than dermatologists. The rate of correctly recognized pictures with "abnormal" pattern was significantly greater than ones with "normal" pattern at the baseline and after the course, independently of imagining tool. CONCLUSIONS: Short courses on NFC may improve the classification of capillaroscopic images, even in medical staff with no previous experience in NFC. The recognition of capillaroscopic abnormalities seems to be easier than obtaining the confidence that evaluated picture has "normal pattern." CI - © 2022 The Authors. Skin Research and Technology published by John Wiley & Sons Ltd. FAU - Miziołek, Bartosz AU - Miziołek B AUID- ORCID: 0000-0001-8388-4419 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Pieczyrak, Robert AU - Pieczyrak R AUID- ORCID: 0000-0003-1882-0210 AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Polak, Karina AU - Polak K AUID- ORCID: 0000-0001-7785-5427 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Frątczak, Aleksandra AU - Frątczak A AUID- ORCID: 0000-0001-9047-7483 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Jedlecka, Aleksandra AU - Jedlecka A AD - Students' Scientific Association at the Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Grosicka, Anida AU - Grosicka A AUID- ORCID: 0000-0002-1056-5625 AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Bergler-Czop, Beata AU - Bergler-Czop B AUID- ORCID: 0000-0003-3788-5984 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. LA - eng PT - Journal Article DEP - 20221116 PL - England TA - Skin Res Technol JT - Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) JID - 9504453 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Nails/diagnostic imaging MH - Capillaries MH - *Raynaud Disease/diagnostic imaging MH - Microcirculation PMC - PMC9838766 OTO - NOTNLM OT - Raynaud's phenomenon OT - dermoscopy OT - microvasculopathy OT - nailfold capillaroscopy EDAT- 2022/11/18 06:00 MHDA- 2023/01/31 06:00 PMCR- 2022/11/16 CRDT- 2022/11/17 10:45 PHST- 2022/09/22 00:00 [received] PHST- 2022/10/11 00:00 [accepted] PHST- 2022/11/18 06:00 [pubmed] PHST- 2023/01/31 06:00 [medline] PHST- 2022/11/17 10:45 [entrez] PHST- 2022/11/16 00:00 [pmc-release] AID - SRT13223 [pii] AID - 10.1111/srt.13223 [doi] PST - ppublish SO - Skin Res Technol. 2023 Jan;29(1):e13223. doi: 10.1111/srt.13223. Epub 2022 Nov 16. PMID- 27339587 OWN - NLM STAT- MEDLINE DCOM- 20180820 LR - 20181202 IS - 0030-9982 (Print) IS - 0030-9982 (Linking) VI - 66 IP - 6 DP - 2016 Jun TI - Raynaud's phenomenon and bilateral olecranon bursitis co-existing in a patient with chronic hepatitis B and D treated with pegylated interferon. PG - 768-70 AB - Pegylated interferon remains the first line treatment for patients with hepatitis D virus and more than one year therapy may be necessary. Interferon a has the most extensive clinical application and is used for the treatment of chronic hepatitis B and D virus as well as HCV infections. The attachment of polyethylene glycol to interferon increases its half-life. Treatment with peg interferon is associated with many troublesome and occasionally with serious or even life-threatening side effects. In this case report, we have described a patient with chronic hepatitis B and D, who developed Raynaud's phenomenon, ischaemic digital necrosis and bilateral olecranon bursitis during Pegylated interferon therapy. The patient underwent a very extensive workup in order to determine the underlying cause of his digital ischaemia and olecranon bursitis, which was finally determined to be secondary to the use of Pegylated interferon. FAU - Arain, Shafique Rehman AU - Arain SR AD - Department of Rheumatology, Liaquat National Hospital and Medical College, Karachi, Pakistan. FAU - Umer, Tahira Perveen AU - Umer TP AD - Department of Rheumatology, Liaquat National Hospital and Medical College, Karachi, Pakistan. LA - eng PT - Case Reports PT - Journal Article PL - Pakistan TA - J Pak Med Assoc JT - JPMA. The Journal of the Pakistan Medical Association JID - 7501162 RN - 0 (Antiviral Agents) RN - 0 (Interferon-alpha) RN - 0 (Recombinant Proteins) RN - 49717AWG6K (Ribavirin) SB - IM MH - Antiviral Agents/*therapeutic use MH - Bursitis/*complications MH - Hepatitis B, Chronic/*complications/drug therapy MH - Hepatitis C, Chronic MH - Hepatitis D/*complications MH - Humans MH - Interferon-alpha/therapeutic use MH - Olecranon Process MH - Raynaud Disease/*complications MH - Recombinant Proteins MH - Ribavirin OTO - NOTNLM OT - Pegylated Interferon; digital ischaemia; necrosis: Raynaud's phenomenon; bursitis. EDAT- 2016/06/25 06:00 MHDA- 2018/08/21 06:00 CRDT- 2016/06/25 06:00 PHST- 2016/06/25 06:00 [entrez] PHST- 2016/06/25 06:00 [pubmed] PHST- 2018/08/21 06:00 [medline] AID - 7802 [pii] PST - ppublish SO - J Pak Med Assoc. 2016 Jun;66(6):768-70. PMID- 34057304 OWN - NLM STAT- MEDLINE DCOM- 20220113 LR - 20220113 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 73 IP - 12 DP - 2021 Dec TI - Factors Influencing Patient Decision-Making Concerning Treatment Escalation in Raynaud's Phenomenon Secondary to Systemic Sclerosis. PG - 1845-1852 LID - 10.1002/acr.24710 [doi] AB - OBJECTIVE: To explore patient priorities and ranking of factors influencing patient decision-making concerning treatment escalation in the management of Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). METHODS: Patients with SSc were invited to participate in an online survey disseminated through patient-led organizations and social media platforms. RESULTS: Responses from 747 individuals with self-reported SSc-RP were evaluable with broad international representation. The mean ± SD age (54.7 ± 12.1 years), clinical phenotype, and disease subsets distribution (limited cutaneous SSc [402 of 747, 53.8%], diffuse cutaneous SSc [260 of 747, 34.8%], and overlap disease [85 of 747, 11.4%]) were consistent with expected demographic information. Around one-half (56.3%) of patients reported that their SSc-RP symptoms were adequately controlled. The 5 highest ranked factors (of 13) that would prompt treatment escalation for SSc-RP were as follows: 1) inability to use the fingers properly; 2) emergence of new digital ulcer on ≥1 fingers; 3) worsening pain or discomfort from RP; 4) more severe attacks; and 5) if it may help with internal problems. Despite symptoms not being adequately controlled, 47.1% were concerned about potential treatment side effects and were more likely to accept mild (~20-40%) versus severe (2%) side effects. Patients were open to different management strategies for uncontrolled RP that included adding new treatment in combination with existing treatment (52.8%), drug substitution (40.9%), increasing the current dose (28.8%), or focusing on nonpharmacologic approaches (29.7%). CONCLUSION: We have identified the relative importance of different factors influencing patient preferences for treatment decision-making regarding SSc-RP. Side-effect profiles influence acceptability of drug treatments, and many patients report a preference for nonpharmacologic management of SSc-RP. CI - © 2021, American College of Rheumatology. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Huang, Suiyuan AU - Huang S AD - University of Michigan, Ann Arbor. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals) and University of Bath, Bath, UK. FAU - Sabbagh, Maya AU - Sabbagh M AD - University of Michigan, Ann Arbor. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. LA - eng PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Decision Making MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Patient Acceptance of Health Care MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Systemic/*complications MH - Surveys and Questionnaires MH - Young Adult EDAT- 2021/06/01 06:00 MHDA- 2022/01/14 06:00 CRDT- 2021/05/31 09:24 PHST- 2021/02/24 00:00 [received] PHST- 2021/05/18 00:00 [accepted] PHST- 2021/06/01 06:00 [pubmed] PHST- 2022/01/14 06:00 [medline] PHST- 2021/05/31 09:24 [entrez] AID - 10.1002/acr.24710 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2021 Dec;73(12):1845-1852. doi: 10.1002/acr.24710. PMID- 20167641 OWN - NLM STAT- MEDLINE DCOM- 20110804 LR - 20191210 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 60 IP - 3 DP - 2010 May TI - Infrared thermometry in the diagnosis of hand-arm vibration syndrome. PG - 225-30 LID - 10.1093/occmed/kqq004 [doi] AB - BACKGROUND: Clinicians need an accurate diagnostic test for hand-arm vibration syndrome (HAVS). AIMS: To validate a simple thermometric method to diagnose HAVS-related Raynaud's phenomenon. METHODS: Fifteen workers with photographically confirmed HAVS-related Raynaud's phenomenon were compared to controls without Raynaud's phenomenon and an occupational history of hand-arm vibration exposure. Digit temperatures were measured using an infrared thermometer before and after immersion in 5 degrees C water for 1 min. RESULTS: The HAVS patients differed significantly from the controls in terms of baseline temperature, rewarming time and rate. The fingertip-base temperature gradient was more commonly positive among the controls. CONCLUSIONS: The test method evaluated in this study is simple, cheap and accurate. If the pre-test probability is at least 35%, the best test variable to confirm the diagnosis of HAVS-related Raynaud's phenomenon is the time to rewarm to baseline of the first three fingertips providing the interval is > or =8-9 min. FAU - Youakim, S AU - Youakim S AD - Department of Medicine, University of British Columbia, Vancouver, Canada. syouakim@telusplanet.net LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Validation Study DEP - 20100218 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Adult MH - Case-Control Studies MH - *Cold Temperature MH - Fingers/blood supply MH - Hand-Arm Vibration Syndrome/*diagnosis/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*diagnosis/physiopathology MH - Raynaud Disease/*diagnosis/physiopathology MH - Sensitivity and Specificity MH - Skin Temperature/physiology MH - Thermography/instrumentation/*methods MH - Time Factors EDAT- 2010/02/20 06:00 MHDA- 2011/08/05 06:00 CRDT- 2010/02/20 06:00 PHST- 2010/02/20 06:00 [entrez] PHST- 2010/02/20 06:00 [pubmed] PHST- 2011/08/05 06:00 [medline] AID - kqq004 [pii] AID - 10.1093/occmed/kqq004 [doi] PST - ppublish SO - Occup Med (Lond). 2010 May;60(3):225-30. doi: 10.1093/occmed/kqq004. Epub 2010 Feb 18. PMID- 37202251 OWN - NLM STAT- MEDLINE DCOM- 20230710 LR - 20250130 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 61 DP - 2023 Aug TI - Domain reporting in systemic sclerosis-related Raynaud's phenomenon: An OMERACT scoping review. PG - 152208 LID - S0049-0172(23)00050-1 [pii] LID - 10.1016/j.semarthrit.2023.152208 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) is a cardinal feature of SSc and is associated with significant disease-related morbidity that impacts on quality of life. The assessment of SSc-RP is challenging. The aim of this scoping review was to evaluate the outcome domains studied and outcome measures used in clinical studies of SSc-RP. METHODS: Embase, MEDLINE, and the Cochrane Central Register of Controlled Trials were used to identify randomized control trials (RCTs), quasi-randomized studies, case-control studies, prospective and retrospective cohort studies, case series, and cross-sectional studies of adult participants with SSc-associated RP, written in English. A minimum of 25 participants for studies of imaging modalities and 40 participants for questionnaire-based studies was required for inclusion. Basic laboratory and genetic studies were excluded. No limitations were imposed based on intervention, comparator, or study setting. Study characteristics and primary and secondary target domains in each study were recorded. RESULTS: 58 studies (24 randomized clinical trials) were included in the final analysis. The commonest domains captured were severity of attacks (n=35), frequency of attacks (n=28), and duration of attacks (n=19). Objective assessments of digital perfusion were also commonly used in studies of SSc-RP. CONCLUSION: The outcome domains and the associated outcomes used to assess the impact of SSc-RP in research studies are broad and have varied across studies. The results of this study will inform the OMERACT Vascular Disease in Systemic Sclerosis Working Group to establish a core set of disease domains encompassing the impact of RP in SSc. CI - Copyright © 2023. Published by Elsevier Inc. FAU - Maltez, Nancy AU - Maltez N AD - Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada. Electronic address: nmaltez@toh.ca. FAU - Hughes, Michael AU - Hughes M AD - Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK; Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Brown, Edith AU - Brown E AD - Manchester, United Kingdom. FAU - Hickey, Virginia AU - Hickey V AD - Adelaide, Australia. FAU - Shea, Beverley AU - Shea B AD - Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Proudman, Susanna AU - Proudman S AD - Discipline of Medicine, University of Adelaide and Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia. FAU - Merkel, Peter A AU - Merkel PA AD - Division of Rheumatology, Department of Medicine, Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA. FAU - Pauling, John D AU - Pauling JD AD - North Bristol NHS Trust, Bristol, UK; Bristol Medical School, University of Bristol, Bristol, UK. LA - eng PT - Journal Article PT - Scoping Review DEP - 20230427 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Humans MH - *Scleroderma, Systemic/complications MH - Surveys and Questionnaires MH - *Raynaud Disease/complications MH - Cross-Sectional Studies MH - Case-Control Studies OTO - NOTNLM OT - Clinical trials OT - OMERACT OT - Outcomes OT - Raynaud's phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of Competing Interest Dr Hughes reports speaking fees from Actelion Pharmaceuticals, Eli Lilly, and Pfizer, outside of the submitted work. Research funding from Janssen. Member of a Data and Safety Monitoring Board for Certa Therapeutics. Dr. Pauling reports grants and personal fees from Janssen, outside the submitted work; Dr Pauling reports personal and consultancy fees from Boehringer Ingelheim, Sojournix Pharma, Astra Zeneca, IsoMab and Permeatus, Inc. Dr Proudman reports receiving funds for the following activities: advisory board: Boehringer-Ingelheim, Janssen, Gossamer. Research Support: Janssen. Dr. Merkel reports receiving funds for the following activities: Consulting: AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, CSL Behring, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Jannsen, Kiniksa, Kyverna, Magenta, Novartis, Pfizer, Sparrow, Takeda, Talaris. Research Support: AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Sanofi. Royalties: UpToDate. Dr Herrick reports research funding from Gesynta Pharma, consultancies with Arena, Boehringer-Ingelheim, Camurus, CSL Behring, Gesynta Pharma and speaker's fees from Janssen. EDAT- 2023/05/19 01:04 MHDA- 2023/07/10 06:42 CRDT- 2023/05/18 21:59 PHST- 2023/01/19 00:00 [received] PHST- 2023/04/17 00:00 [revised] PHST- 2023/04/19 00:00 [accepted] PHST- 2023/07/10 06:42 [medline] PHST- 2023/05/19 01:04 [pubmed] PHST- 2023/05/18 21:59 [entrez] AID - S0049-0172(23)00050-1 [pii] AID - 10.1016/j.semarthrit.2023.152208 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2023 Aug;61:152208. doi: 10.1016/j.semarthrit.2023.152208. Epub 2023 Apr 27. PMID- 35583625 OWN - NLM STAT- MEDLINE DCOM- 20220922 LR - 20220922 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 10 DP - 2022 Oct TI - The clinical relevance of Raynaud's phenomenon symptom characteristics in systemic sclerosis. PG - 3049-3054 LID - 10.1007/s10067-022-06206-y [doi] AB - Raynaud's phenomenon (RP) is a cardinal feature of systemic sclerosis (SSc) and manifests with pain, digital colour change, sensory symptoms, and impaired function. SSc-RP is exacerbated by cold exposure (RP 'attacks') but many patients report persistent symptoms of background digital ischaemia. The aim of our study was to examine the significance of RP with digital colour change with or without symptoms, and persistent colour change in between attacks. Patients with SSc responses were obtained from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP). We enquired about symptoms associated with Raynaud's attacks, and persistent symptoms in between attacks. Data were analysed as descriptive statistics with appropriate parametric/non-parametric testing. Relevant PASRAP survey question data from 747 evaluable SSc patients from across three continents were analysed. Isolated colour change was rare (29/484, 6%). Digital ulcers were more common in SSc-RP associated with other sensory symptoms (42.1% vs. 24.1%, P=0.057) and more readily treated with phosphodiesterase-type 5 inhibitors (22.5% vs. 10.3%%, P=0.124). Over one-third of patients (n=92/239, 38%) reported persistent colour change in between Raynaud's attacks. Patients with persistent colour change were more likely to have pulmonary arterial hypertension (15.2% vs. 7.5%, P=0.057) and be treated with calcium channel blockers (54.3% vs. 39.0%, P=0.021). SSc-RP with colour change and other symptoms and/or or persistent decolourisation in between attacks were more likely to have vascular complications of SSc and be treated with vascular therapies. Future research should explore the judicious use of vascular therapies as a potential form of disease modification in SSc. Key Points • Isolated colour change without other symptoms is rare in SSc patients. • SSc patients often identify persistent symptoms in between attacks of RP. • SSc-RP with colour change and other symptoms, or persistent decolourisation, may have greater disease severity and be treated with vascular therapies. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Department of Rheumatology, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, UK. Michael.hughes-6@postgrad.manchester.ac.uk. AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK. Michael.hughes-6@postgrad.manchester.ac.uk. FAU - Huang, Suiyuan AU - Huang S AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. AD - Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, USA. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Sabbagh, Maya AU - Sabbagh M AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. LA - eng GR - K24 AR AR063120/Foundation for the National Institutes of Health/ PT - Journal Article DEP - 20220518 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Calcium Channel Blockers) RN - EC 3.1.4.- (Phosphoric Diester Hydrolases) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Humans MH - Phosphoric Diester Hydrolases/therapeutic use MH - *Raynaud Disease/etiology MH - *Scleroderma, Systemic MH - *Skin Ulcer/etiology OTO - NOTNLM OT - Colour change OT - Management OT - Raynaud’s phenomenon OT - Symptoms OT - Systemic sclerosis OT - Treatment EDAT- 2022/05/19 06:00 MHDA- 2022/09/23 06:00 CRDT- 2022/05/18 11:36 PHST- 2022/04/11 00:00 [received] PHST- 2022/05/08 00:00 [accepted] PHST- 2022/05/06 00:00 [revised] PHST- 2022/05/19 06:00 [pubmed] PHST- 2022/09/23 06:00 [medline] PHST- 2022/05/18 11:36 [entrez] AID - 10.1007/s10067-022-06206-y [pii] AID - 10.1007/s10067-022-06206-y [doi] PST - ppublish SO - Clin Rheumatol. 2022 Oct;41(10):3049-3054. doi: 10.1007/s10067-022-06206-y. Epub 2022 May 18. PMID- 16769658 OWN - NLM STAT- MEDLINE DCOM- 20060802 LR - 20060613 IS - 0891-6934 (Print) IS - 0891-6934 (Linking) VI - 39 IP - 3 DP - 2006 May TI - Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies. PG - 243-7 AB - The idiopathic inflammatory myopathies are a heterogeneous group of diseases that can involve various systems. Antibodies directed against aminoacyl-tRNA synthetases, such as anti-Jo-1 antibodies, are strongly associated with a syndrome which consists of myositis, interstitial lung disease (ILD), arthritis and Raynaud's phenomenon. Forty-one patients with various forms of idiopathic inflammatory myopathies were assessed: 14 patients with anti-Jo-1 antibodies and 27 patients without anti-Jo-1 antibodies as a control group. We retrospectively analysed clinical symptoms, treatment and outcome in both groups. Patients with anti-Jo-1 antibodies more often had ILD (64.2 vs. 11.1%), arthritis (64.2 vs. 18.1%) and Raynaud's phenomenon (38 vs. 0%). Patients without the anti-Jo-1 antibody presented worse muscle strength and more frequently myalgia (37 vs. 21%), cutaneous rash (18.5 vs. 7%), heliotrope rash (29% vs. 7%) and periungueal changes (22 vs. 0%) than the anti-Jo-1-positive patients. Outcome was good in both groups. Improvement was achieved in the 14 (100%) Jo-1 positive patients, and in 25 (92.5%) controls. Two (7.5%) patients from control group achieved remission. FAU - Mielnik, Pawel AU - Mielnik P AD - Institute of Rheumatology, Department of Connective Tissue Diseases, Warsaw, Poland. mielnik_p@ir.ids.pl FAU - Wiesik-Szewczyk, Ewa AU - Wiesik-Szewczyk E FAU - Olesinska, Marzena AU - Olesinska M FAU - Chwalinska-Sadowska, Hanna AU - Chwalinska-Sadowska H FAU - Zabek, Jakub AU - Zabek J LA - eng PT - Journal Article PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) SB - IM MH - Antibodies, Antinuclear/*blood MH - Arthritis/diagnosis/physiopathology MH - Female MH - Humans MH - Lung Diseases, Interstitial/diagnosis/physiopathology MH - Male MH - Myositis/*diagnosis/immunology/physiopathology MH - Prognosis MH - Raynaud Disease/diagnosis/physiopathology MH - Retrospective Studies EDAT- 2006/06/14 09:00 MHDA- 2006/08/03 09:00 CRDT- 2006/06/14 09:00 PHST- 2006/06/14 09:00 [pubmed] PHST- 2006/08/03 09:00 [medline] PHST- 2006/06/14 09:00 [entrez] AID - G724L45043281266 [pii] AID - 10.1080/08916930600623767 [doi] PST - ppublish SO - Autoimmunity. 2006 May;39(3):243-7. doi: 10.1080/08916930600623767. PMID- 38306796 OWN - NLM STAT- MEDLINE DCOM- 20240721 LR - 20240721 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 3 IP - Apr-Jun DP - 2024 Jul 1 TI - Systematic literature review to inform the Portuguese recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases. PG - 128-144 LID - 10.63032/YHBL8967 [doi] AB - OBJECTIVE: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients. METHODS: A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD. Eligible study designs included randomized controlled trials (RCTs), controlled clinical trials, and their extensions for assessing efficacy and safety of interventions. Observational studies with a comparator were included for evaluating the efficacy and safety of non-pharmacological interventions and safety of pharmacological interventions. The risk of bias of each study was assessed using standard tools. RESULTS: Out of 71 publications meeting the inclusion criteria, 59 evaluated pharmacological and 12 non-pharmacological interventions. We found moderate quality evidence supporting the efficacy of calcium channel blockers, phosphodiesterase-5 inhibitors, and intravenous prostacyclin analogues in reducing RP frequency, severity, and duration. Intravenous iloprost had a small to moderate effect size in improving DU healing. Phosphodiesterase-5 inhibitors were effective in reducing total DU count, new DU occurrence, and enhancing DU healing. Bosentan effectively prevented new DU in SSc patients. No new safety concerns were associated with these treatments. The studies on non-pharmacological interventions were, in general, of low quality, and had a small sample size. Warming measures decreased frequency and duration of RP attacks; laser therapy improved RP-related outcomes; local oxygen-ozone therapy improved RP outcomes as an add-on therapy; bone marrow mononuclear cell implantation improved DU-associated pain; periarterial sympathectomy and vascular bypass reduced DU number and finger amputation risk. CONCLUSION: The available evidence supports the efficacy and safety of pharmacological interventions, namely nifedipine, sildenafil, iloprost, and bosentan in treating RP and DU in patients with SSc and other CTD. Scarce and low-quality evidence does support the use of some non-pharmacological interventions but with only a modest effect size. This SLR underscores the limited availability of high-quality evidence for determining the optimal treatment. FAU - Costa, Emanuel AU - Costa E AD - Hospital de Braga, Braga, Portugal. FAU - Cunha-Santos, Filipe AU - Cunha-Santos F AD - Unidade Local de Saúde da Guarda, Guarda, Portugal. FAU - Dourado, Eduardo AU - Dourado E AD - Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. FAU - Oliveira, Daniela AU - Oliveira D AD - Centro Hospitalar Universitário de São João, Porto, Portugal. FAU - Falzon, Louise AU - Falzon L AD - University of Sheffield, Sheffield, United Kingdom. FAU - Romão, Vasco AU - Romão V AD - Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Duarte, Ana Catarina AU - Duarte AC AD - Hospital Garcia de Orta, Almada, Portugal. FAU - Cordeiro, Ana AU - Cordeiro A AD - Hospital Garcia de Orta, Almada, Portugal. FAU - Santiago, Tânia AU - Santiago T AD - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Sepriano, Alexandre AU - Sepriano A AD - Universidade Nova de Lisboa, Lisbon, Portugal. LA - eng PT - Journal Article PT - Systematic Review TT - Systematic literature review to inform the Portuguese recommendations for the management of Raynaud’s phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases. DEP - 20240701 PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 RN - 0 (Calcium Channel Blockers) RN - JED5K35YGL (Iloprost) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - *Raynaud Disease/therapy/etiology/drug therapy MH - *Scleroderma, Systemic/complications/therapy MH - *Connective Tissue Diseases/complications/therapy MH - *Skin Ulcer/therapy/etiology/drug therapy MH - Portugal/epidemiology MH - Calcium Channel Blockers/therapeutic use MH - Fingers/blood supply MH - Practice Guidelines as Topic MH - Iloprost/therapeutic use MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2024/02/03 00:42 MHDA- 2024/07/21 17:42 CRDT- 2024/02/02 18:05 PHST- 2024/02/02 00:00 [aheadofprint] PHST- 2024/07/21 17:42 [medline] PHST- 2024/02/03 00:42 [pubmed] PHST- 2024/02/02 18:05 [entrez] AID - AR230341 [pii] AID - 10.63032/YHBL8967 [doi] PST - epublish SO - ARP Rheumatol. 2024 Jul 1;3(Apr-Jun):128-144. doi: 10.63032/YHBL8967. PMID- 18320192 OWN - NLM STAT- MEDLINE DCOM- 20081219 LR - 20211020 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 28 IP - 10 DP - 2008 Aug TI - Management of cutaneous vascular complications in systemic scleroderma: experience from the German network. PG - 1023-9 LID - 10.1007/s00296-008-0556-1 [doi] AB - Major cutaneous vascular complications of systemic sclerosis (SSc) are secondary Raynaud's phenomenon (RP) and digital ulcers. Even though SSc is a rare disease, timely and appropriate management of its vascular complications is mandatory for reducing the rate of major impairment. It should therefore be aware to physicians from different specialties. We evaluated the diagnostic and therapeutic approach toward secondary RP and ulceration in SSc patients at 28 German clinical centers at the time of initiation of the German network for Systemic Scleroderma (DNSS). We retrieved data via questionnaires and from the DNSS patient registry. Management of RP and ulcerations in SSc were heterogeneous at initiation of the network, reflecting a sometimes insufficient use of the diagnostic and therapeutic possibilities. As such, (1) calcium channel blockers were the first line therapy in most centers; but often in insufficient dosages, and (2) only 21.2% of patients with acral ulceration had received prostacyclins when recruited into the network. The sometimes insufficient care of vascular complications of SSc in Germany revealed the need for their standardized management, e.g. within a network for SSc and for consensus on a diagnostic or therapeutic algorithm. FAU - Herrgott, Ilka AU - Herrgott I AD - Department of Dermatology, University Hospital Münster, Münster, Germany. FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Hunzelmann, Nicolas AU - Hunzelmann N FAU - Sunderkötter, Cord AU - Sunderkötter C LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20080305 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Calcium Channel Blockers) RN - 0 (Prostaglandins I) RN - 0 (Vasodilator Agents) SB - IM MH - Calcium Channel Blockers/therapeutic use MH - Germany MH - *Health Care Surveys MH - Humans MH - Professional Practice MH - Prostaglandins I/therapeutic use MH - Raynaud Disease/diagnosis/*drug therapy/*etiology MH - Registries MH - Scleroderma, Systemic/*complications/diagnosis/*drug therapy MH - Skin/*blood supply MH - Skin Ulcer/diagnosis/drug therapy/etiology MH - Surveys and Questionnaires MH - Vasodilator Agents/therapeutic use EDAT- 2008/03/06 09:00 MHDA- 2008/12/20 09:00 CRDT- 2008/03/06 09:00 PHST- 2007/12/13 00:00 [received] PHST- 2008/02/18 00:00 [accepted] PHST- 2008/03/06 09:00 [pubmed] PHST- 2008/12/20 09:00 [medline] PHST- 2008/03/06 09:00 [entrez] AID - 10.1007/s00296-008-0556-1 [doi] PST - ppublish SO - Rheumatol Int. 2008 Aug;28(10):1023-9. doi: 10.1007/s00296-008-0556-1. Epub 2008 Mar 5. PMID- 36920010 OWN - NLM STAT- MEDLINE DCOM- 20230316 LR - 20230316 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 19 IP - 818 DP - 2023 Mar 15 TI - [What's new in the management of systemic sclerosis]. PG - 521-525 LID - 10.53738/REVMED.2023.19.818.521 [doi] AB - This review of the literature highlights the results of the recent randomized controlled trials about the management of systemic sclerosis (SSc) and its complications. The latest randomized studies have failed to demonstrate the utility against placebo of the injections of botulinum toxin A to achieve a better control of Raynaud's phenomenon and the efficacy of the adipose-derived cell transplantation for the treatment of hand dysfunction. Rituximab allows a significant improvement of cutaneous induration. The injections of mesenchymal stromal cells are well tolerated and should encourage future randomized trials to evaluate their efficacy. Finally, nintedanib and tocilizumab allow a reduction in the rate of decline of lung function, as well as a possible stabilization with tocilizumab. FAU - Khelifa, Mona AU - Khelifa M AD - Service de rhumatologie, Département de médecine, Hôpitaux universitaires de Genève, 1211 Genève 14. FAU - Guemara, Romain AU - Guemara R AUID- ORCID: 0000-0001-7776-4533 AD - Service de rhumatologie, Département de médecine, Hôpitaux universitaires de Genève, 1211 Genève 14. FAU - Iudici, Michele AU - Iudici M AUID- ORCID: 0000-0001-5871-8806 AD - Service de rhumatologie, Département de médecine, Hôpitaux universitaires de Genève, 1211 Genève 14. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Nouveautés dans la prise en charge de la sclérodermie systémique. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Humans MH - *Botulinum Toxins, Type A/therapeutic use MH - *Scleroderma, Systemic/therapy/complications MH - Hand MH - *Raynaud Disease/drug therapy/etiology MH - Injections/adverse effects COIS- Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article. EDAT- 2023/03/16 06:00 MHDA- 2023/03/17 06:00 CRDT- 2023/03/15 08:43 PHST- 2023/03/15 08:43 [entrez] PHST- 2023/03/16 06:00 [pubmed] PHST- 2023/03/17 06:00 [medline] AID - RMS0818-006 [pii] AID - 10.53738/REVMED.2023.19.818.521 [doi] PST - ppublish SO - Rev Med Suisse. 2023 Mar 15;19(818):521-525. doi: 10.53738/REVMED.2023.19.818.521. PMID- 25751473 OWN - NLM STAT- MEDLINE DCOM- 20150526 LR - 20150310 IS - 0028-3886 (Print) IS - 0028-3886 (Linking) VI - 63 IP - 1 DP - 2015 Jan-Feb TI - An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries. PG - 72-6 LID - 10.4103/0028-3886.152642 [doi] AB - Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy. FAU - Patil, Anil Kumar B AU - Patil AK FAU - Prabhakar, A T AU - Prabhakar AT FAU - Sivadasan, Ajith AU - Sivadasan A FAU - Alexander, Mathew AU - Alexander M AD - Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India. FAU - Chacko, Geeta AU - Chacko G LA - eng PT - Case Reports PT - Journal Article PL - India TA - Neurol India JT - Neurology India JID - 0042005 RN - 0 (Immunoglobulins) RN - 8N3DW7272P (Cyclophosphamide) RN - EC 2.7.3.2 (Creatine Kinase) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Capillaries/*pathology MH - Creatine Kinase/blood MH - Cyclophosphamide/therapeutic use MH - Dermatomyositis/*diagnosis/therapy MH - Female MH - Humans MH - Immunoglobulins/therapeutic use MH - Inflammation/diagnosis/therapy MH - Methylprednisolone/therapeutic use MH - Muscle, Skeletal/blood supply/pathology MH - Necrosis/diagnosis/therapy MH - Peripheral Nerves/*pathology MH - Raynaud Disease/diagnosis/therapy EDAT- 2015/03/10 06:00 MHDA- 2015/05/27 06:00 CRDT- 2015/03/10 06:00 PHST- 2015/03/10 06:00 [entrez] PHST- 2015/03/10 06:00 [pubmed] PHST- 2015/05/27 06:00 [medline] AID - ni_2015_63_1_72_152642 [pii] AID - 10.4103/0028-3886.152642 [doi] PST - ppublish SO - Neurol India. 2015 Jan-Feb;63(1):72-6. doi: 10.4103/0028-3886.152642. PMID- 18035518 OWN - NLM STAT- MEDLINE DCOM- 20080603 LR - 20161209 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 37 IP - 5 Pt 2 DP - 2008 May TI - [Iloprost for the treatment of systemic sclerosis]. PG - 831-9 AB - An imbalance between prostacyclin (PGI2) and thromboxane A2 is observed in patients with scleroderma. Iloprost is a stable analogue of PGI2 with a plasma half-life of 20-30 min. Intravenous iloprost is effective in the treatment of Raynaud's phenomenon related to scleroderma, decreasing the frequency and severity of attacks. It also appears useful for the treatment of digital ulcers. Inhaled iloprost is an effective treatment for NYHA class III pulmonary arterial hypertension, either idiopathic primary or associated with a particular condition, such as scleroderma. Intravenous iloprost improves kidney vasospasm in patients with scleroderma. The possible benefits of sequential intravenous iloprost on the natural course of scleroderma require further investigation. FAU - Hachulla, Eric AU - Hachulla E AD - Centre de référence des maladies auto-immunes et maladies systémiques rares sclérodermie systémique, Service de médecine interne, Hôpital Claude Huriez, CHU, Université de Lille 2, F-59037 Lille Cedex, France. ehachulla@chru-lille.fr FAU - Launay, David AU - Launay D FAU - Hatron, Pierre-Yves AU - Hatron PY LA - fre PT - English Abstract PT - Journal Article PT - Review TT - L'iloprost dans le traitement de la sclérodermie systémique. DEP - 20071126 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Humans MH - Hypertension/drug therapy/etiology MH - Iloprost/*therapeutic use MH - Raynaud Disease/drug therapy/etiology MH - Renal Circulation/drug effects MH - Scleroderma, Systemic/complications/*drug therapy MH - Vasodilator Agents/*therapeutic use RF - 44 EDAT- 2007/11/24 09:00 MHDA- 2008/06/05 09:00 CRDT- 2007/11/24 09:00 PHST- 2007/02/24 00:00 [received] PHST- 2007/05/31 00:00 [revised] PHST- 2007/06/01 00:00 [accepted] PHST- 2007/11/24 09:00 [pubmed] PHST- 2008/06/05 09:00 [medline] PHST- 2007/11/24 09:00 [entrez] AID - S0755-4982(07)00552-0 [pii] AID - 10.1016/j.lpm.2007.06.016 [doi] PST - ppublish SO - Presse Med. 2008 May;37(5 Pt 2):831-9. doi: 10.1016/j.lpm.2007.06.016. Epub 2007 Nov 26. PMID- 31734401 OWN - NLM STAT- MEDLINE DCOM- 20200219 LR - 20200219 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 19 IP - 1 DP - 2020 Jan TI - Systemic sclerosis induced by CNS stimulants for ADHD: A case series and review of the literature. PG - 102439 LID - S1568-9972(19)30250-2 [pii] LID - 10.1016/j.autrev.2019.102439 [doi] AB - INTRODUCTION: Methylphenidate (Ritalin) is a CNS stimulant, and is a common treatment for children and adults with ADHD. It has been associated with Raynaud's phenomenon (RP) but not with Systemic Sclerosis (SSc). We report a case series of patients pointing out the connection between Methylphenidate and SSc. CASES: We identified three patients in a single Rheumatology clinic in Israel, who developed SSc following treatment with CNS stimulants for ADHD. All three cases had Raynaud's phenomenon, skin changes, pathological capillaroscopy and positive ANA. Symptoms appeared and worsened over months following the use of methylphenidate and subsided after its cessation. CONCLUSION: This is the first report in the literature of a causative relation between methylphenidate and the development of SSc, a serious, life-threatening condition. Patients treated with CNS stimulants should be followed closely for side-effects such as RP and skin changes. CI - Copyright © 2019. Published by Elsevier B.V. FAU - Meridor, Katya AU - Meridor K AD - Department of Internal Medicine, Meir Medical Center, Kfar Saba, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel-Aviv, Israel. FAU - Levy, Yair AU - Levy Y AD - Department of Internal Medicine, Meir Medical Center, Kfar Saba, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel-Aviv, Israel. Electronic address: LEVY.YAIR@clalit.org.il. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20191114 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Central Nervous System Stimulants) SB - IM MH - Adult MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Central Nervous System Stimulants/*adverse effects/therapeutic use MH - Female MH - Humans MH - Israel MH - Raynaud Disease/chemically induced MH - Scleroderma, Systemic/*chemically induced MH - Young Adult OTO - NOTNLM OT - ADHD OT - CNS stimulants OT - Methylphenidate OT - Raynaud's phenomenon OT - Systemic sclerosis EDAT- 2019/11/18 06:00 MHDA- 2020/02/20 06:00 CRDT- 2019/11/18 06:00 PHST- 2019/07/08 00:00 [received] PHST- 2019/07/12 00:00 [accepted] PHST- 2019/11/18 06:00 [pubmed] PHST- 2020/02/20 06:00 [medline] PHST- 2019/11/18 06:00 [entrez] AID - S1568-9972(19)30250-2 [pii] AID - 10.1016/j.autrev.2019.102439 [doi] PST - ppublish SO - Autoimmun Rev. 2020 Jan;19(1):102439. doi: 10.1016/j.autrev.2019.102439. Epub 2019 Nov 14. PMID- 27318627 OWN - NLM STAT- MEDLINE DCOM- 20170901 LR - 20190512 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 46 IP - 2 DP - 2016 Oct TI - Differential diagnosis of critical digital ischemia in systemic sclerosis: Report of five cases and review of the literature. PG - 209-216 LID - S0049-0172(16)30043-9 [pii] LID - 10.1016/j.semarthrit.2016.05.001 [doi] AB - OBJECTIVES: Critical digital ischemia is a rare, but serious complication of systemic sclerosis (SSc) and is not always due solely to the non-inflammatory angiopathy that characterizes the SSc disease process. Our objective was to illustrate the range of presentations and causes of critical digital ischemia in patients with SSc in order to highlight how optimal management is dependent upon establishing the correct diagnosis. METHODS: Five cases exemplifying differential diagnoses were identified and their case notes reviewed in order to extract clinically relevant data and images. A review of the literature was performed in PubMed in English. RESULTS: Causes of critical digital ischemia included typical micro-angiopathic changes and proximal (large vessel) disease. One case highlighted the difficulty of ascertaining whether an inflammatory cause is also present in SSc/SLE overlap syndrome. Two cases demonstrated embolic causes (thromboembolism due to atrial fibrillation and septic emboli). CONCLUSIONS: Critical digital ischemia in patients with SSc requires thorough investigation in order to avoid missing additional potentially modifiable causes including large vessel disease, inflammation, embolism, infection, and paraneoplastic syndromes. A firm evidence base for current medical and surgical interventions is lacking, highlighting the need for further research into the optimum management of this rare, but painful, debilitating, and limb-threatening complication of SSc. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Sharp, Charlotte A AU - Sharp CA AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK. Electronic address: Charlotte.a.sharp@gmail.com. FAU - Akram, Qasim AU - Akram Q AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Stott Lane, Manchester, M6 8HD, UK. FAU - Muir, Lindsay AU - Muir L AD - Department of Orthopedic Surgery, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Stott Lane, Manchester, M6 8HD, UK; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, 29 Grafton Street, Manchester, M13 9WU, UK. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20160512 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Aged MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*diagnosis/etiology MH - Middle Aged MH - Raynaud Disease/*diagnosis/etiology MH - Scleroderma, Systemic/*complications MH - Toes/*blood supply OTO - NOTNLM OT - Critical digital ischemia OT - Digital ulcer OT - Digital vasculopathy OT - Embolism OT - Raynaud’s phenomenon OT - Scleroderma OT - Systemic sclerosis OT - Vasculitis EDAT- 2016/06/20 06:00 MHDA- 2017/09/02 06:00 CRDT- 2016/06/20 06:00 PHST- 2016/02/02 00:00 [received] PHST- 2016/04/11 00:00 [revised] PHST- 2016/05/09 00:00 [accepted] PHST- 2016/06/20 06:00 [entrez] PHST- 2016/06/20 06:00 [pubmed] PHST- 2017/09/02 06:00 [medline] AID - S0049-0172(16)30043-9 [pii] AID - 10.1016/j.semarthrit.2016.05.001 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Oct;46(2):209-216. doi: 10.1016/j.semarthrit.2016.05.001. Epub 2016 May 12. PMID- 30986311 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1526-4637 (Electronic) IS - 1526-2375 (Linking) VI - 21 IP - 5 DP - 2020 May 1 TI - Pain Intensity, Pressure Pain Hypersensitivity, Central Sensitization, and Pain Catastrophizing Related to Vascular Alterations in Raynaud's Phenomenon: A Preliminary Case-Control Study. PG - 891-901 LID - 10.1093/pm/pnz089 [doi] AB - OBJECTIVE: To evaluate pain intensity, widespread pressure pain, central sensitization (CS), and catastrophizing between subjects with primary and secondary Raynaud's phenomenon (RP) and healthy controls and to compare the relationships between vascular impairment and pain perception. METHODS: A preliminary case-control study was performed with a total sample of 57 participants (37 with RP). Sociodemographic data, clinical/vascular data, and pain variables (pain intensity, pressure pain sensitivity, pain magnitude and threshold, CS, and catastrophizing) were registered. Results were analyzed by analysis of covariance and Pearson correlation. RESULTS: Participants with RP had a lower basal temperature (more vasoconstriction) in their hands (P ≤ 0.012), higher pain intensity (P ≤ 0.001), higher electrical pain magnitude (P < 0.001), and lower pressure pain (P ≤ 0.05) and electrical pain (P < 0.001) thresholds in comparison with healthy controls. Secondary RP participants showed a significantly higher level of CS compared with controls and primary RP participants (P = 0.001). Catastrophizing was higher in the primary and secondary RP (P ≤ 0.001) groups than in controls. No correlations were observed between severity of vasoconstriction and pain variables. CONCLUSIONS: RP participants showed bilateral hypersensitivity to pressure pain. However, the severity of vascular alterations seems not to be related to central pain experiences. Additional mechanisms such as catastrophizing may influence pain in RP; nevertheless, central sensitization only appears to be involved in the secondary form of RP. CI - © 2019 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. FAU - Tapia-Haro, Rosa María AU - Tapia-Haro RM AD - Department of Physical Therapy, Faculty of Health Science. FAU - Guisado-Barrilao, Rafael AU - Guisado-Barrilao R AD - Department of Nursing. FAU - García-Ríos, María Del Carmen AU - García-Ríos MDC AD - Department of Physical Therapy, University of Granada, Granada, Spain. FAU - Raya-Álvarez, Enrique AU - Raya-Álvarez E AD - Department of Physical Therapy, Faculty of Health Science. AD - Rheumatology Division, University Hospital "San Cecilio," Granada, Spain. FAU - Pérez-Mármol, José Manuel AU - Pérez-Mármol JM AD - Instituto de Investigación Biosanitaria Granada, Department of Physical Therapy, Faculty of Health Science, University of Granada, Granada, Spain. FAU - Aguilar-Ferrándiz, María Encarnación AU - Aguilar-Ferrándiz ME AD - Instituto de Investigación Biosanitaria Granada, Department of Physical Therapy, Faculty of Health Science, University of Granada, Granada, Spain. LA - eng PT - Journal Article PL - England TA - Pain Med JT - Pain medicine (Malden, Mass.) JID - 100894201 SB - IM MH - Case-Control Studies MH - *Catastrophization MH - Central Nervous System Sensitization MH - Humans MH - *Raynaud Disease MH - Vasoconstriction OTO - NOTNLM OT - Catastrophization OT - Central Nervous System Sensitization OT - Pain OT - Peripheral Vascular Diseases OT - Raynaud Disease EDAT- 2019/04/16 06:00 MHDA- 2021/05/15 06:00 CRDT- 2019/04/16 06:00 PHST- 2019/04/16 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2019/04/16 06:00 [entrez] AID - 5464929 [pii] AID - 10.1093/pm/pnz089 [doi] PST - ppublish SO - Pain Med. 2020 May 1;21(5):891-901. doi: 10.1093/pm/pnz089. PMID- 36919392 OWN - NLM STAT- MEDLINE DCOM- 20230316 LR - 20230316 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 30 IP - 4 DP - 2022 Dec TI - Very Early Diagnosis of Systemic Sclerosis in Clinical Practice - Case Report and Review of the Literature. PG - 251-255 AB - Systemic sclerosis (SSc) is a systemic autoimmune disease characterised by generalized microangiopathy and fibrosis of skin and internal organs. The 2013 American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) criteria have contributed considerably to classifying patients with SSc in earlier stages, but they still lack sensitivity for a very early stage of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been proposed by EULAR Scleroderma Trial and Research group (EUSTAR) which include three red flags: Raynaud's phenomenon, puffy fingers and antinuclear antibody positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capillaroscopy. We report a case of a 54-year-old female patient with 6-week history of puffy fingers, Raynaud phenomenon and positive antinuclear antibodies. Further workup revealed early pathologic capillary pattern by nailfold capillaroscopy and positive anticentromere antibodies. Screening for internal organ involvement detected no heart, lung, or upper gastrointestinal tract involvement. The patient was started on pentoxifylline with further follow-up. The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earliest possible stage, so that subclinical internal organ involvement could be detected and appropriate treatment started at a potentially reversible stage. FAU - Marković, Ivan AU - Marković I FAU - Posavec, Anja Ljilja AU - Posavec AL FAU - Morović-Vergles, Jadranka AU - Morović-Vergles J FAU - Mitrović, Joško AU - Mitrović J AD - Assist. Prof. Joško Mitrović, MD, PhD Division of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, School of Medicine University of Zagreb, Dubrava University Hospital, Zagreb, Croatia Avenija Gojka Šuška 6 Zagreb, Croatia josko.mitrovic@kbd.hr. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 SB - IM MH - Female MH - Humans MH - Middle Aged MH - *Scleroderma, Systemic/diagnosis MH - *Rheumatology MH - Early Diagnosis MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Localized EDAT- 2023/03/16 06:00 MHDA- 2023/03/17 06:00 CRDT- 2023/03/15 03:52 PHST- 2023/03/15 03:52 [entrez] PHST- 2023/03/16 06:00 [pubmed] PHST- 2023/03/17 06:00 [medline] PST - ppublish SO - Acta Dermatovenerol Croat. 2022 Dec;30(4):251-255. PMID- 33823574 OWN - NLM STAT- MEDLINE DCOM- 20220804 LR - 20250728 IS - 2724-5772 (Electronic) IS - 2724-5683 (Linking) VI - 70 IP - 4 DP - 2022 Aug TI - Capillaroscopy: a useful tool in the early diagnosis of connective tissue disease and non-scleroderma spectrum disorders. PG - 476-483 LID - 10.23736/S2724-5683.21.05513-7 [doi] AB - BACKGROUND: Detection of early capillaroscopic alterations in the preclinical phase may prove useful in patients with non-scleroderma connective tissue disease (CTD). We aimed to verify whether certain capillaroscopic alterations, alone or in combination, might be predictive of CTD. METHODS: We retrospectively collected data on patients with Raynaud's phenomenon who underwent capillaroscopy conducted by highly expert examiners with a degree in vascular medicine at our institutions. Included subjects were divided in two groups: those developing rheumatic disease during follow-up, and those without subsequent diagnosis of CTD. Notably, we excluded subjects who presented with an evident scleroderma pattern or rheumatic disease during their initial examination. RESULTS: We included a total of 76 patients, 60 of whom developed CTD during follow-up, which spanned in this group 23±7 months, and 16 who did not develop CTD during follow-up, which spanned 23±9 months. The following features were significantly associated with Raynaud's phenomenon: 1) angiotectonic disorder (P<0.001); 2) nonhomogeneous loop morphology (P<0.001); 3) avascular areas (P<0.001); 4) pseudo-avascular areas (P<0.001), and, albeit to a lesser degree; and 5) ectasias (P<0.050). Notably, the initial capillaroscopic pattern did not undergo any changes in subsequent tests. CONCLUSIONS: Although certain pathological characteristics of the capillaroscopic pattern are non-specific and not diagnostic if considered individually, they can be significantly suggestive for latent CTD when found in combination. At the very least, they warrant an in-depth diagnostic analysis and a lengthy follow-up. FAU - DI Pino, Luigi AU - DI Pino L AD - Section of Angiology, Department of Surgery and Medical-Surgical Specialties, University of Catania, Catania, Italy. FAU - Bilancini, Salvino AU - Bilancini S AD - J.F. Merlen Center for Vascular Diseases Studies, Frosinone, Italy - silviasilvietta@libero.it. FAU - Peruzzi, Mariangela AU - Peruzzi M AD - Department of Medical-Surgical Sciences and Biotechnologies, Sapienza University, Latina, Italy. AD - Mediterranea Cardiocentro, Naples, Italy. FAU - Lucchi, Massimo AU - Lucchi M AD - J.F. Merlen Center for Vascular Diseases Studies, Frosinone, Italy. LA - eng PT - Journal Article DEP - 20210407 PL - Italy TA - Minerva Cardiol Angiol JT - Minerva cardiology and angiology JID - 101776555 SB - IM MH - *Connective Tissue Diseases/complications/diagnosis/pathology MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/complications/diagnosis/pathology MH - Retrospective Studies MH - *Rheumatic Diseases/complications/diagnosis MH - *Scleroderma, Localized/complications/diagnosis EDAT- 2021/04/08 06:00 MHDA- 2022/08/05 06:00 CRDT- 2021/04/07 04:47 PHST- 2021/04/08 06:00 [pubmed] PHST- 2022/08/05 06:00 [medline] PHST- 2021/04/07 04:47 [entrez] AID - S2724-5683.21.05513-7 [pii] AID - 10.23736/S2724-5683.21.05513-7 [doi] PST - ppublish SO - Minerva Cardiol Angiol. 2022 Aug;70(4):476-483. doi: 10.23736/S2724-5683.21.05513-7. Epub 2021 Apr 7. PMID- 31482318 OWN - NLM STAT- MEDLINE DCOM- 20200406 LR - 20210110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 12 DP - 2019 Dec TI - Optimisation of botulinum toxin type a treatment for the management of Raynaud's phenomenon using a dorsal approach: a prospective case series. PG - 3669-3676 LID - 10.1007/s10067-019-04762-4 [doi] AB - INTRODUCTION: Raynaud's phenomenon (RP) is a common condition and causes pain, paraesthesia, ulceration and gangrene. Botulinum toxin A (Btx-A) is effective when injected via a digital palmar approach, in the treatment of severe RP. However, hand weakness resulting from lumbrical malfunction is a recognized complication. This study aimed to determine the effect of Btx-A injected via a dorsal approach. METHOD: Forty patients received 100 units of Btx-A, injected across both hands via a dorsal approach. Each patient had a baseline, 6- and 12-week hand assessment and thermographic image (FLIR E60bx) performed for the study. RESULTS: Eighty-eight percent of patients reported an improvement in symptoms including reduction in pain, improved colour change with reduced swelling and edema at 6 weeks. Of these patients, 80% reported an improvement in cold intolerance with a reduction in the frequency and severity of Raynaud's attacks. There was a significant improvement in both the DASH score (p = 0.001), Kapandji score (p = 0.001) and hand strength (p < 0.05). No patients reported weakness. Improvements in hand function and symptoms of RP were still evident at 12 weeks. CONCLUSIONS: Btx-A injected via a dorsal approach improves symptoms and reduces the number of RP. We have shown an effective non-surgical approach technique to treat RP.Key Points• Raynaud's phenomenon is a common vasospastic disorder of the digital vessels, which can cause severe pain, restrictions to hand function and ulceration.• Dorsal botulinum toxin type A injections can improve the symptoms of secondary Raynaud's phenomenon and hand function for approximately 3 months. FAU - Dhaliwal, Kiran AU - Dhaliwal K AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. AD - UCL Centre for Nanotechnology and Regenerative Medicine, London, UK. AD - Division of Surgery & Interventional Science, University College London, London, UK. AD - Royal Free Hospital, London, UK. AD - Plastic & Reconstructive Surgery, Royal Free Hospital, London, UK. FAU - Griffin, Michelle F AU - Griffin MF AUID- ORCID: 0000-0002-2027-547X AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. 12michellegriffin@gmail.com. AD - UCL Centre for Nanotechnology and Regenerative Medicine, London, UK. 12michellegriffin@gmail.com. AD - Division of Surgery & Interventional Science, University College London, London, UK. 12michellegriffin@gmail.com. AD - Royal Free Hospital, London, UK. 12michellegriffin@gmail.com. AD - Plastic & Reconstructive Surgery, Royal Free Hospital, London, UK. 12michellegriffin@gmail.com. FAU - Salinas, Sebastian AU - Salinas S AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. AD - UCL Centre for Nanotechnology and Regenerative Medicine, London, UK. AD - Division of Surgery & Interventional Science, University College London, London, UK. AD - Royal Free Hospital, London, UK. AD - Plastic & Reconstructive Surgery, Royal Free Hospital, London, UK. FAU - Howell, Kevin AU - Howell K AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. AD - Royal Free Hospital, London, UK. AD - Center for Rheumatology, UCL Division of Medicine and Royal Free London NHS Foundation Trust Hospital, London, UK. FAU - Denton, Christopher P AU - Denton CP AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. AD - Royal Free Hospital, London, UK. AD - Center for Rheumatology, UCL Division of Medicine and Royal Free London NHS Foundation Trust Hospital, London, UK. FAU - Butler, Peter E M AU - Butler PEM AD - Charles Wolfson Center for Reconstructive Surgery, Royal Free Hospital, London, UK. AD - UCL Centre for Nanotechnology and Regenerative Medicine, London, UK. AD - Division of Surgery & Interventional Science, University College London, London, UK. AD - Royal Free Hospital, London, UK. AD - Plastic & Reconstructive Surgery, Royal Free Hospital, London, UK. LA - eng PT - Journal Article DEP - 20190903 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*administration & dosage MH - Female MH - Humans MH - Middle Aged MH - Neuromuscular Agents/*administration & dosage MH - Prospective Studies MH - Raynaud Disease/diagnostic imaging/*drug therapy MH - Thermography OTO - NOTNLM OT - Botox OT - Botulinum toxin OT - Raynauds phenomenon OT - Systemic sclerosis OT - Vasospasm EDAT- 2019/09/05 06:00 MHDA- 2020/04/09 06:00 CRDT- 2019/09/05 06:00 PHST- 2019/04/15 00:00 [received] PHST- 2019/08/22 00:00 [accepted] PHST- 2019/08/09 00:00 [revised] PHST- 2019/09/05 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2019/09/05 06:00 [entrez] AID - 10.1007/s10067-019-04762-4 [pii] AID - 10.1007/s10067-019-04762-4 [doi] PST - ppublish SO - Clin Rheumatol. 2019 Dec;38(12):3669-3676. doi: 10.1007/s10067-019-04762-4. Epub 2019 Sep 3. PMID- 32920325 OWN - NLM STAT- MEDLINE DCOM- 20210929 LR - 20250522 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 50 IP - 5 DP - 2020 Oct TI - Performance of laser-derived imaging for assessing digital perfusion in clinical trials of systemic sclerosis-related digital vasculopathy: A systematic literature review. PG - 1114-1130 LID - S0049-0172(20)30197-9 [pii] LID - 10.1016/j.semarthrit.2020.06.018 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) and digital ulcers (DU) are important features of digital vasculopathy in systemic sclerosis (SSc). Laser Doppler flowmetry (LDF), Laser Doppler Imaging (LDI) and Laser Speckle Contrast Imaging (LSCI) can non-invasively quantify digital perfusion and may be useful outcome measures for SSc-RP and/or SSc-DU clinical trials. We undertook a systematic literature review to evaluate the performance of laser-derived imaging as outcome measures in clinical trials of SSc-related digital vasculopathy. METHODS: Standardized searches (EMBASE and MEDLINE) identified trials that incorporated laser-derived imaging of digital vasculopathy in adult subjects with SSc. Data was extracted (by >2 reviewers) on study design, laser endpoints, and reported outcomes. Study quality was assessed using validated instruments (PROSPERO 2019:CRD42019142409). RESULTS: Of 126 identified articles, full data extraction was undertaken from 29 studies. Fifteen randomized and 14 non-randomized trials (total of 689 SSc patients with mean 23.8/study) have evaluated a broad range of oral, intravenous and topical interventions for SSc-RP (n = 11), digital perfusion alone (n = 15) and SSc-DU (n = 3). The studies were published between 1987 and 2019 (17/29 since 2010) and incorporated LDF (11/29), LDI (15/29) and LSCI (4/29, including one with LDF); with LSCI and LDI more commonly incorporated in recent trials. Most studies (16/29, 55%) reported improvement in digital perfusion following intervention, often concordant with patient- and clinician-derived outcomes. CONCLUSIONS: Establishing laser-derived methods as a surrogate for SSc-related digital vasculopathy will greatly support drug development. Full-field perfusion of the digits (with/without provocation testing) is a promising clinical trial outcome measure for trials of SSc-related digital vasculopathy. CI - Copyright © 2020 Elsevier Inc. All rights reserved. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK; Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. Electronic address: jdp32@bath.ac.uk. FAU - Hackett, Natalia AU - Hackett N AD - University of Bristol Medical School, Bristol, UK. FAU - Guida, Andrea AU - Guida A AD - University of Pisa, Pisa, Italy. FAU - Merkel, Peter A AU - Merkel PA AD - University of Pennsylvania, Philadelphia, PA, USA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20200706 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Fingers/diagnostic imaging MH - Humans MH - Lasers MH - Perfusion MH - *Raynaud Disease/diagnostic imaging/etiology MH - *Scleroderma, Systemic/complications/diagnostic imaging OTO - NOTNLM OT - Clinical trials OT - Laser doppler OT - Laser speckle contrast imaging OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Vasculopathy EDAT- 2020/09/14 06:00 MHDA- 2021/09/30 06:00 CRDT- 2020/09/13 20:33 PHST- 2020/03/18 00:00 [received] PHST- 2020/06/10 00:00 [revised] PHST- 2020/06/30 00:00 [accepted] PHST- 2020/09/14 06:00 [pubmed] PHST- 2021/09/30 06:00 [medline] PHST- 2020/09/13 20:33 [entrez] AID - S0049-0172(20)30197-9 [pii] AID - 10.1016/j.semarthrit.2020.06.018 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2020 Oct;50(5):1114-1130. doi: 10.1016/j.semarthrit.2020.06.018. Epub 2020 Jul 6. PMID- 31637927 OWN - NLM STAT- MEDLINE DCOM- 20200731 LR - 20200731 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 49 IP - 2 DP - 2020 Mar TI - Assessing recovery after cold challenge and thumb involvement can help to rule out systemic sclerosis in patients presenting with Raynaud's phenomenon. PG - 137-140 LID - 10.1080/03009742.2019.1643911 [doi] AB - Objective: Our aim was to study whether recovery from a Raynaud's attack and involvement of the thumb are differentiators for systemic sclerosis (SSc) in patients with Raynaud's phenomenon (RP).Method: A stepwise cooling and recovery procedure was performed, provoking an RP attack, in patients with primary Raynaud's phenomenon (PRP, n = 68) and SSc (n = 18). During the procedure, the perfusion of all five fingers during cooling and recovery was assessed by photoelectric plethysmography.Results: In SSc patients, perfusion after 10 min in one or more fingers was more frequently not restored than in PRP patients (p = 0.001), with a negative predictive value of 98%. The thumb was more frequently involved in SSc patients (p = 0.036), with a negative predictive value of 95%. Positive predictive values were low.Conclusions: In patients with RP, when there is restoration of perfusion in all fingers after 10 min or when the thumb is spared, the presence of an underlying SSc is very unlikely. Although these results need to be validated in a clinical setting in a larger prospective study, these signs can help physicians to select additional testing for SSc in RP patients. FAU - van Roon, A M AU - van Roon AM AD - Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - van Roon, A M AU - van Roon AM AD - Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Stel, A J AU - Stel AJ AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Bootsma, H AU - Bootsma H AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Smit, A J AU - Smit AJ AD - Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Mulder, D J AU - Mulder DJ AD - Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. LA - eng PT - Journal Article DEP - 20191022 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Aged MH - Cold Temperature MH - Female MH - Humans MH - Male MH - Middle Aged MH - Perfusion MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis MH - Thumb/*blood supply EDAT- 2019/10/23 06:00 MHDA- 2020/08/01 06:00 CRDT- 2019/10/23 06:00 PHST- 2019/10/23 06:00 [pubmed] PHST- 2020/08/01 06:00 [medline] PHST- 2019/10/23 06:00 [entrez] AID - 10.1080/03009742.2019.1643911 [doi] PST - ppublish SO - Scand J Rheumatol. 2020 Mar;49(2):137-140. doi: 10.1080/03009742.2019.1643911. Epub 2019 Oct 22. PMID- 29931173 OWN - NLM STAT- MEDLINE DCOM- 20200323 LR - 20200323 IS - 1559-0488 (Electronic) IS - 1559-047X (Linking) VI - 40 IP - 1 DP - 2019 Jan 1 TI - Sildenafil Therapy in Patients With Digital Burns and Raynaud's Syndrome. PG - 136-139 LID - 10.1093/jbcr/iry004 [doi] AB - The Raynaud's phenomenon (RP) is characterized by an exaggerated vascular response to cold temperature or emotional stress causing temporary ischemia. It is more prevalent in the digits of the hands and feet, and when occurring in conjunction with a rheumatological condition, it is also termed Raynaud's syndrome, or secondary RP. Healing following a burn requires appropriate tissue perfusion to promote primary restoration of the skin, prevent further burn progression, and to promote skin graft take in wounds requiring autologous split skin grafting. The addition of vascular compromise caused by RP to a burn wound is therefore hypothesized to impair burn wound healing and worsen burn wound progression. The authors describe a 51-year-old female with digital burns on a background of scleroderma and Raynaud's syndrome successfully treated with oral sildenafil therapy and autologous split skin grafting. The case report further highlights the potential role for sildenafil therapy in wound healing and patients requiring autologous skin grafting or local skin flaps. In future cases, we plan to involve rheumatology services early in the course of the injury aiming to improve outcomes. FAU - Cordova, Leonardo Zandavalli AU - Cordova LZ AD - Tasmanian Burns Unit, Royal Hobart Hospital, Hobart, Tasmania, Australia. FAU - Schrale, Rebecca AU - Schrale R AD - Tasmanian Burns Unit, Royal Hobart Hospital, Hobart, Tasmania, Australia. FAU - Castley, Andrew AU - Castley A AD - Tasmanian Burns Unit, Royal Hobart Hospital, Hobart, Tasmania, Australia. LA - eng PT - Case Reports PT - Journal Article PL - England TA - J Burn Care Res JT - Journal of burn care & research : official publication of the American Burn Association JID - 101262774 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Burns/*therapy MH - Combined Modality Therapy MH - Female MH - Hand Injuries/*therapy MH - Humans MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate/*therapeutic use MH - Skin Transplantation MH - Surgical Flaps EDAT- 2018/06/23 06:00 MHDA- 2020/03/24 06:00 CRDT- 2018/06/23 06:00 PHST- 2018/06/23 06:00 [pubmed] PHST- 2020/03/24 06:00 [medline] PHST- 2018/06/23 06:00 [entrez] AID - 4843986 [pii] AID - 10.1093/jbcr/iry004 [doi] PST - ppublish SO - J Burn Care Res. 2019 Jan 1;40(1):136-139. doi: 10.1093/jbcr/iry004. PMID- 29706243 OWN - NLM STAT- MEDLINE DCOM- 20190423 LR - 20260304 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 48 IP - 4 DP - 2019 Feb TI - Practical suggestions on intravenous iloprost in Raynaud's phenomenon and digital ulcer secondary to systemic sclerosis: Systematic literature review and expert consensus. PG - 686-693 LID - S0049-0172(18)30013-1 [pii] LID - 10.1016/j.semarthrit.2018.03.019 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud's phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3-5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials. METHODS: A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise. RESULTS: Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0ng/kg/min according to patient tolerability with a frequency depending on the indication. CONCLUSIONS: Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy; Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milano, Italy. Electronic address: francesca.ingegnoli@unimi.it. FAU - Schioppo, Tommaso AU - Schioppo T AD - Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University, Cochin Hospital, Rheumatology A department, INSERM U1016, Paris, France. FAU - Caporali, Roberto AU - Caporali R AD - Division of Rheumatology, University of Pavia, IRCCS S. Matteo Foundation, Pavia, Italy. FAU - Colaci, Michele AU - Colaci M AD - Rheumatology Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Switzerland. FAU - Furst, Daniel E AU - Furst DE AD - Los Angeles, USA UCLA (emeritus); University of Washington, Seattle Wash; University of Florence, Florence, Italy. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology, University of Cologne, Cologne, Germany. FAU - Iannone, Florenzo AU - Iannone F AD - DETO-Section of Rheumathology, Bari, Italy. FAU - Khanna, Dinesh AU - Khanna D AD - Ann Arbor, University of Michigan, Ann Arbor, Michigan. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence & Department of Geriatric Medicine, Division of Rheumatology AOUC, Florence, Italy. LA - eng PT - Consensus Statement PT - Journal Article PT - Systematic Review DEP - 20180404 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Fingers MH - Humans MH - Iloprost/administration & dosage/*therapeutic use MH - Injections, Intravenous MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology MH - Vasodilator Agents/administration & dosage/*therapeutic use OTO - NOTNLM OT - Digital ulcer OT - Iloprost OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2018/05/01 06:00 MHDA- 2019/04/24 06:00 CRDT- 2018/05/01 06:00 PHST- 2018/01/13 00:00 [received] PHST- 2018/03/27 00:00 [revised] PHST- 2018/03/29 00:00 [accepted] PHST- 2018/05/01 06:00 [pubmed] PHST- 2019/04/24 06:00 [medline] PHST- 2018/05/01 06:00 [entrez] AID - S0049-0172(18)30013-1 [pii] AID - 10.1016/j.semarthrit.2018.03.019 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2019 Feb;48(4):686-693. doi: 10.1016/j.semarthrit.2018.03.019. Epub 2018 Apr 4. PMID- 17670849 OWN - NLM STAT- MEDLINE DCOM- 20071023 LR - 20161124 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 16 IP - 7 DP - 2007 TI - Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus. PG - 505-8 AB - In patients with Systemic lupus erythematosus (SLE), Raynaud phenomenon (RP) is frequently present and associated with pulmonary hypertension (PHT). Elevated pulmonary artery systolic pressure (PASP) is an indicator of PHT and can be estimated noninvasively. We attempt to explore the significance of RP in SLE and to correlate it with clinical and serological parameters of the disease. The study population consisted of 34 patients (age, sex and disease duration matched) who fulfilled the revised SLE criteria of the American College of Rheumatology, and were categorized into two groups: Group 1 had patients having SLE and RP (2 males/15 females, mean age 45 +/- 18 years) and group 2 had patients with SLE but without RP (3 males/14 females, mean age 40 +/- 14 years. Detailed cardiac ultrasound was performed including measurement of PASP, while clinical and serological features of both groups were collected and correlated. Significant differences were shown in the presence of arterial hypertension (P < 0.05), arthralgias (P < 0.005), arthritis (P < 0.05), myalgias (P < 0.05), alopecia (P < 0.05) and PASP (P < 0.0001). No difference was observed among the cardiac ultrasound indices and the ejection fraction between the two groups. PASP was significantly correlated with RP, while no correlation was observed regarding the disease duration. In patients with SLE, the presence of RP was associated with elevation in PASP. Further investigation is needed to clarify the significance of this relation. FAU - Kasparian, A AU - Kasparian A AD - Department of Cardiology, Laikon General Hospital, Univ. of Athens, School of Medicine, Athens, Greece. FAU - Floros, A AU - Floros A FAU - Gialafos, E AU - Gialafos E FAU - Kanakis, M AU - Kanakis M FAU - Tassiopoulos, S AU - Tassiopoulos S FAU - Kafasi, N AU - Kafasi N FAU - Vaiopoulos, G AU - Vaiopoulos G LA - eng PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM CIN - Lupus. 2008 Apr;17(4):355. doi: 10.1177/0961203307088290. PMID: 18413419 MH - Adult MH - Echocardiography MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/diagnostic imaging/*etiology/physiopathology MH - Lupus Erythematosus, Systemic/complications/*physiopathology MH - Male MH - Middle Aged MH - Prognosis MH - Pulmonary Wedge Pressure/*physiology MH - Raynaud Disease/complications/diagnostic imaging/*physiopathology MH - Severity of Illness Index MH - Systole MH - Time Factors EDAT- 2007/08/03 09:00 MHDA- 2007/10/24 09:00 CRDT- 2007/08/03 09:00 PHST- 2007/08/03 09:00 [pubmed] PHST- 2007/10/24 09:00 [medline] PHST- 2007/08/03 09:00 [entrez] AID - 16/7/505 [pii] AID - 10.1177/0961203307080629 [doi] PST - ppublish SO - Lupus. 2007;16(7):505-8. doi: 10.1177/0961203307080629. PMID- 20448801 OWN - NLM STAT- MEDLINE DCOM- 20110127 LR - 20260128 IS - 1178-2048 (Electronic) IS - 1176-6344 (Print) IS - 1176-6344 (Linking) VI - 6 DP - 2010 Mar 24 TI - Advances in the treatment of Raynaud's phenomenon. PG - 167-77 AB - Raynaud's phenomenon is a common condition characterized by vasospasm of the digital arteries and resulting cyanosis and redness. It often does not require pharmacologic management, but in some cases symptoms are severe and pharmacologic management is necessary. Calcium channel blockers are often used first-line, but in some patients are ineffective. Patients with severe symptoms or intolerance to available therapies have prompted exploration of alternative therapies, including endothelin antagonists, phosphodiesterase-5 inhibitors, antioxidants, newer vasodilators, statins, and botulinum toxin. These newer therapies provide the focus for this review. FAU - Levien, Terri L AU - Levien TL AD - College of Pharmacy, Washington State University Spokane, WA, USA. levient@wsu.edu LA - eng PT - Journal Article PT - Review DEP - 20100324 PL - New Zealand TA - Vasc Health Risk Manag JT - Vascular health and risk management JID - 101273479 RN - 0 (Antioxidants) RN - Q326023R30 (Bosentan) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Calcium Channel Blockers) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - JED5K35YGL (Iloprost) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Serotonin Antagonists) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) SB - IM MH - Antioxidants/therapeutic use MH - Bosentan MH - Botulinum Toxins, Type A/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Endothelin Receptor Antagonists MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use MH - Iloprost/therapeutic use MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Raynaud Disease/*drug therapy MH - Serotonin Antagonists/therapeutic use MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Sulfonamides/therapeutic use MH - Vasodilator Agents/therapeutic use PMC - PMC2860448 OTO - NOTNLM OT - Raynaud OT - bosentan OT - iloprost OT - phosphodiesterase-5 inhibitors EDAT- 2010/05/08 06:00 MHDA- 2011/01/29 06:00 PMCR- 2010/03/24 CRDT- 2010/05/08 06:00 PHST- 2010/03/15 00:00 [received] PHST- 2010/05/08 06:00 [entrez] PHST- 2010/05/08 06:00 [pubmed] PHST- 2011/01/29 06:00 [medline] PHST- 2010/03/24 00:00 [pmc-release] AID - vhrm-6-167 [pii] AID - 10.2147/vhrm.s4551 [doi] PST - epublish SO - Vasc Health Risk Manag. 2010 Mar 24;6:167-77. doi: 10.2147/vhrm.s4551. PMID- 31810547 OWN - NLM STAT- MEDLINE DCOM- 20200217 LR - 20200217 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 33 IP - 4 DP - 2019 Aug TI - Very early systemic sclerosis. PG - 101428 LID - S1521-6942(19)30097-X [pii] LID - 10.1016/j.berh.2019.101428 [doi] AB - The early diagnosis of systemic sclerosis (SSc) can be very difficult, when most of the typical signs and symptoms are absent. For this reason, the approach to SSc has changed during the last decades because the importance of an early diagnosis and treatment has been widely understood. "Very early SSc" is identified as a condition characterized by Raynaud's phenomenon, puffy fingers, disease-specific autoantibodies, and microvascular alterations at capillaroscopy. However, reliable biomarkers able to predict the disease evolution are missing, and decision whether to treat or not to treat in the earliest phase of the disease remains a dilemma. Presently, the only feasible clinical strategy in very early SSc remains a tight follow-up program to detect in "real time" the onset of internal organ involvement, which may thus allow an aggressive therapeutic agenda. CI - Copyright © 2019. Published by Elsevier Ltd. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Dept. of Experimental and Clinical Medicine, University of Florence, Italy. Electronic address: s.bellandorandone@gmail.com. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Dept. of Experimental and Clinical Medicine, University of Florence, Italy. Electronic address: marco.matuccicerinic@unifi.it. LA - eng PT - Journal Article PT - Review DEP - 20190903 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy MH - Raynaud Disease/etiology MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy OTO - NOTNLM OT - Classification criteria OT - Diagnosis OT - Very early systemic sclerosis EDAT- 2019/12/08 06:00 MHDA- 2020/02/18 06:00 CRDT- 2019/12/08 06:00 PHST- 2019/12/08 06:00 [entrez] PHST- 2019/12/08 06:00 [pubmed] PHST- 2020/02/18 06:00 [medline] AID - S1521-6942(19)30097-X [pii] AID - 10.1016/j.berh.2019.101428 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2019 Aug;33(4):101428. doi: 10.1016/j.berh.2019.101428. Epub 2019 Sep 3. PMID- 22707610 OWN - NLM STAT- MEDLINE DCOM- 20130116 LR - 20221207 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 39 IP - 8 DP - 2012 Aug TI - Digital amputation in systemic sclerosis: prevalence and clinical associations. A retrospective longitudinal study. PG - 1648-53 LID - 10.3899/jrheum.111506 [doi] AB - OBJECTIVE: To evaluate the prevalence of digital necrosis requiring surgical amputation in a single-center group of patients with systemic sclerosis (SSc) and to compare the characteristics of patients with and those without this severe complication. METHODS: We reviewed the medical records of 188 patients with SSc [162 women, 26 men, mean age 59.2 yrs, mean disease duration 8.0 yrs, mean time from onset of Raynaud's phenomenon (RP) 11.7 yrs, median followup duration 92 mo] enrolled in the Rheumatology Unit since 2004. Demographic and clinical features were collected, as well as the presence of the typical risk factors for atherosclerosis. RESULTS: Nine patients (4.8%) underwent partial or total surgical digital amputation because of necrotic process; all these patients except 1 had a long history of multiple and persisting digital ulcers. All 9 patients had concomitant large-vessel involvement. Comparison of cases with and without digital amputation showed that this complication was associated with older age, long history of RP, long disease duration, presence of anticentromere antibody, and coexistence of peripheral artery disease and hypercholesterolemia. Discussion. We noted that 4.8% of patients with SSc underwent digital amputation. Our retrospective analysis suggests that peripheral artery disease is strongly associated with digital amputation. The preventive strategy for digital ulcers and amputation associated with SSc should include an extensive diagnostic and preventive investigation for peripheral atherosclerosis. FAU - Caramaschi, Paola AU - Caramaschi P AD - Unità di Reumatologia, Policlinico G.B. Rossi, Piazzale Scuro, 37134 Verona, Italy.. paola.caramaschi@ospedaleuniverona.it. FAU - Biasi, Domenico AU - Biasi D FAU - Caimmi, Cristian AU - Caimmi C FAU - Barausse, Giovanni AU - Barausse G FAU - Sabbagh, Dania AU - Sabbagh D FAU - Tinazzi, Ilaria AU - Tinazzi I FAU - LA Verde, Valentina AU - LA Verde V FAU - Tonetta, Sara AU - Tonetta S FAU - Adami, Silvano AU - Adami S LA - eng PT - Journal Article DEP - 20120615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Amputation, Surgical/*statistics & numerical data MH - Female MH - Fingers/blood supply/pathology/*surgery MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Necrosis MH - Prevalence MH - Raynaud Disease/etiology/pathology/*surgery MH - Retrospective Studies MH - Scleroderma, Systemic/complications/pathology/*surgery MH - Skin Ulcer/etiology/pathology/*surgery EDAT- 2012/06/19 06:00 MHDA- 2013/01/17 06:00 CRDT- 2012/06/19 06:00 PHST- 2012/06/19 06:00 [entrez] PHST- 2012/06/19 06:00 [pubmed] PHST- 2013/01/17 06:00 [medline] AID - jrheum.111506 [pii] AID - 10.3899/jrheum.111506 [doi] PST - ppublish SO - J Rheumatol. 2012 Aug;39(8):1648-53. doi: 10.3899/jrheum.111506. Epub 2012 Jun 15. PMID- 17345017 OWN - NLM STAT- MEDLINE DCOM- 20070716 LR - 20070308 IS - 0723-5003 (Print) IS - 0723-5003 (Linking) VI - 102 IP - 3 DP - 2007 Mar 15 TI - [Therapeutic management of acral manifestations of systemic sclerosis]. PG - 209-18 AB - Acral manifestations of systemic sclerosis include Raynaud's phenomenon, calcinosis cutis, and sclerodactyly. In the later stages of the disease, contractures of the skin and joints as well as obliterative vasculopathy leading to digital ulcers and necrotic lesions may occur. Patients with acral manifestations of systemic sclerosis are ideally treated by a team that includes a rheumatologist, dermatologist, hand surgeon, physiotherapist, and, eventually, a psychologist. Calcium channel antagonists, alpha(1)-adrenergic blockade with prazosin, and prostacyclin analogs were proven to be effective in the treatment of scleroderma-related Raynaud's phenomenon. Losartan, an angiotensin II receptor inhibitor, and fluoxetine, a selective serotonin reuptake inhibitor, have been beneficial for systemic sclerosis-associated Raynaud's phenomenon in pilot studies. Parenteral prostacyclin analogs, e. g., iloprost, can be recommended as first-line treatment of ischemic digital ulcers. When prostacyclin analogs fail, the phosphodiesterase type 5 inhibitor sildenafil can be tried to improve ulcer healing. Bosentan, an endothelin receptor antagonist, may prevent new digital ulcers. At present, there are no medical agents agreed to be generally effective in the reduction of calcinotic deposits or cutaneous fibrosis, although some drugs have been identified as potentially beneficial. Surgical treatment of acral manifestations consists of excision or curettage of symptomatic calcific deposits, digital sympathectomy, arterial reconstruction, and amputation in rare cases. Flexion contractures of the proximal interphalangeal joints, with secondary hyperextension of the metacarpophalangeal joints, can be treated by arthrodesis of the proximal interphalangeal joints and resection arthroplasty or prostheses at the metacarpophalangeal joints to improve hand function. FAU - Meyer, Martin F AU - Meyer MF AD - Medizinische Klinik I, Zentrum für Innere Medizin, Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil GmbH, Ruhr-Universität Bochum, Bochum, Deutschland. Martin.Meyer-2@rub.de FAU - Daigeler, Adrien AU - Daigeler A FAU - Lehnhardt, Marcus AU - Lehnhardt M FAU - Steinau, Hans-Ulrich AU - Steinau HU FAU - Klein, Harald H AU - Klein HH LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Therapeutisches Management akraler Manifestationen der systemischen Sklerose. PL - Germany TA - Med Klin (Munich) JT - Medizinische Klinik (Munich, Germany : 1983) JID - 8303501 SB - IM MH - Calcinosis/diagnosis/*therapy MH - Combined Modality Therapy MH - Humans MH - Prognosis MH - Raynaud Disease/diagnosis/*therapy MH - Scleroderma, Systemic/diagnosis/*therapy MH - Skin Diseases/diagnosis/*therapy RF - 128 EDAT- 2007/03/09 09:00 MHDA- 2007/07/17 09:00 CRDT- 2007/03/09 09:00 PHST- 2006/10/02 00:00 [received] PHST- 2007/01/22 00:00 [revised] PHST- 2007/03/09 09:00 [pubmed] PHST- 2007/07/17 09:00 [medline] PHST- 2007/03/09 09:00 [entrez] AID - 10.1007/s00063-007-1025-4 [doi] PST - ppublish SO - Med Klin (Munich). 2007 Mar 15;102(3):209-18. doi: 10.1007/s00063-007-1025-4. PMID- 24491823 OWN - NLM STAT- MEDLINE DCOM- 20141002 LR - 20260120 IS - 1095-9157 (Electronic) IS - 0896-8411 (Print) IS - 0896-8411 (Linking) VI - 48-49 DP - 2014 Feb-Mar TI - International consensus criteria for the diagnosis of Raynaud's phenomenon. PG - 60-5 LID - S0896-8411(14)00023-7 [pii] LID - 10.1016/j.jaut.2014.01.020 [doi] AB - Vasoconstriction accompanied by changes in skin color is a normal physiologic response to cold. The distinction between this normal physiology and Raynaud's phenomenon (RP) has yet to be well characterized. In anticipation of the 9th International Congress on Autoimmunity, a panel of 12 RP experts from 9 different institutes and four different countries were assembled for a Delphi exercise to establish new diagnostic criteria for RP. Relevant investigators with highly cited manuscripts in Raynaud's-related research were identified using the Web of Science and invited to participate. Surveys at each stage were administered to participants via the on-line SurveyMonkey software tool. The participants evaluated the level of appropriateness of statements using a scale of 1 (extremely inappropriate) through 9 (extremely appropriate). In the second stage, panel participants were asked to rank rewritten items from the first round that were scored as "uncertain" for the diagnosis of RP, items with significant disagreement (Disagreement Index > 1), and new items suggested by the panel. Results were analyzed using the Interpercentile Range Adjusted for Symmetry (IPRAS) method. A 3-Step Approach to diagnose RP was then developed using items the panelists "agreed" were "appropriate" diagnostic criteria. In the final stage, the panel was presented with the newly developed diagnostic criteria and asked to rate them against previous models. Following the first two iterations of the Delphi exercise, the panel of 12 experts agreed that 36 of the items were "appropriate", 12 items had "uncertain" appropriateness, and 13 items were "inappropriate" to use in the diagnostic criteria of RP. Using an expert committee, we developed a 3-Step Approach for the diagnosis of RP and 5 additional criteria for the diagnosis of primary RP. The committee came to an agreement that the proposed criteria were "appropriate and accurate" for use by physicians to diagnose patients with RP. CI - Published by Elsevier Ltd. FAU - Maverakis, Emanual AU - Maverakis E AD - Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA; Department of Dermatology, Veterans Affairs Northern California Health Care System, Sacramento, CA 95655, USA. Electronic address: emaverakis@ucdavis.edu. FAU - Patel, Forum AU - Patel F AD - Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA. FAU - Kronenberg, Daniel G AU - Kronenberg DG AD - Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA. FAU - Chung, Lorinda AU - Chung L AD - Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA; Department of Dermatology, Stanford University, Redwood City, CA 94305, USA. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Paris Descartes University, Paris, France. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Rheumatology, University of Florence, Florence, Italy. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Hummers, Laura K AU - Hummers LK AD - Department of Medicine/Rheumatology, Johns Hopkins University, Baltimore, MD 21287, USA. FAU - Duong, Chris AU - Duong C AD - Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA. FAU - Kahaleh, Bashar AU - Kahaleh B AD - Department of Internal Medicine, Division of Rheumatology, University of Toledo, Toledo, OH 43614, USA. FAU - Macgregor, Alexander AU - Macgregor A AD - Department of Rheumatology, University of East Anglia, Norwich, Norfolk, United Kingdom. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Medicine & Rheumatology, University of Florence, Florence, Italy. FAU - Wollheim, Frank A AU - Wollheim FA AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Mayes, Maureen D AU - Mayes MD AD - Department of Internal Medicine, Division of Rheumatology and Clinical Immunogenetics, University of Texas-Houston, Houston, TX 77030, USA. FAU - Gershwin, M Eric AU - Gershwin ME AD - Department of Internal Medicine, Division of Rheumatology, University of California, Davis, CA 95616, USA. LA - eng GR - DP2 OD008752/OD/NIH HHS/United States GR - HHMI/Howard Hughes Medical Institute/United States GR - NIH DP2OD008752/OD/NIH HHS/United States PT - Consensus Statement PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20140201 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 SB - IM MH - Arthritis, Rheumatoid/diagnosis MH - Autoimmune Diseases/diagnosis MH - Data Collection MH - Dermatomyositis/diagnosis MH - Diagnosis, Differential MH - International Cooperation MH - Lupus Erythematosus, Systemic/diagnosis MH - Mixed Connective Tissue Disease/diagnosis MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/diagnosis MH - Sjogren's Syndrome/diagnosis PMC - PMC4018202 MID - NIHMS564554 OTO - NOTNLM OT - Diagnostic criteria OT - Primary Raynaud's OT - Raynaud's phenomenon OT - Secondary Raynaud's COIS- Conflict of interest disclosures: None Reported EDAT- 2014/02/05 06:00 MHDA- 2014/10/03 06:00 PMCR- 2015/02/01 CRDT- 2014/02/05 06:00 PHST- 2013/10/07 00:00 [received] PHST- 2013/11/13 00:00 [accepted] PHST- 2014/02/05 06:00 [entrez] PHST- 2014/02/05 06:00 [pubmed] PHST- 2014/10/03 06:00 [medline] PHST- 2015/02/01 00:00 [pmc-release] AID - S0896-8411(14)00023-7 [pii] AID - 10.1016/j.jaut.2014.01.020 [doi] PST - ppublish SO - J Autoimmun. 2014 Feb-Mar;48-49:60-5. doi: 10.1016/j.jaut.2014.01.020. Epub 2014 Feb 1. PMID- 23681135 OWN - NLM STAT- MEDLINE DCOM- 20130920 LR - 20130517 IS - 0026-4725 (Print) IS - 0026-4725 (Linking) VI - 61 IP - 3 DP - 2013 Jun TI - Mesoglycan treatment in Raynaud phenomenon: a case series. PG - 323-31 AB - AIM AND METHODS: Raynaud's phenomenon (RP) is a common clinical disorder without cure or gold standard therapy. Mesoglycan is an well-balanced extract of glycosaminoglycans active on endothelial layers at microcirculatory level. Herein we investigated for the first time the efficacy and tolerability of mesoglycan on vasospastic attacks and videocapillaroscopy patterns in 25 consecutive patients with primary or secondary RP. RESULTS AND CONCLUSION: During 12 months of add-on therapy, mesoglycan obtained a remarkable and significant reduction in the frequency of Raynaud attacks and an improvement of the capillaroscopy abnormalities in most patients, without important adverse effects, revealing a good convenience in the management if this condition. FAU - Di Biase, A AU - Di Biase A AD - Ambulatory of Vascular Diagnostic, Geriatric Medicine Villa Verde, Taranto, Italy. a.dibiase@email.it LA - eng PT - Clinical Trial PT - Journal Article PL - Italy TA - Minerva Cardioangiol JT - Minerva cardioangiologica JID - 0400725 RN - 0 (Glycosaminoglycans) RN - 0 (Vasodilator Agents) RN - 80455-95-4 (mesoglycan) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Female MH - Fingers/blood supply MH - Follow-Up Studies MH - Glycosaminoglycans/*therapeutic use MH - Humans MH - Male MH - Microcirculation/drug effects MH - Microscopic Angioscopy MH - Middle Aged MH - Outpatients MH - Raynaud Disease/*drug therapy MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use EDAT- 2013/05/18 06:00 MHDA- 2013/09/21 06:00 CRDT- 2013/05/18 06:00 PHST- 2013/05/18 06:00 [entrez] PHST- 2013/05/18 06:00 [pubmed] PHST- 2013/09/21 06:00 [medline] AID - R05Y2013N03A0323 [pii] PST - ppublish SO - Minerva Cardioangiol. 2013 Jun;61(3):323-31. PMID- 39793863 OWN - NLM STAT- MEDLINE DCOM- 20250523 LR - 20260525 IS - 1873-1740 (Electronic) IS - 0033-0620 (Linking) VI - 89 DP - 2025 Mar-Apr TI - Cigarette smoke extract induces p38-mediated expression and ROS/rho-mediated translocation of alpha 2C adrenoceptor in human microvascular smooth muscle cells. PG - 119-126 LID - S0033-0620(25)00002-7 [pii] LID - 10.1016/j.pcad.2025.01.002 [doi] AB - Raynaud's phenomenon (RP) is a vascular disease characterized by exaggerated vasoconstriction in response to stressors, mainly cold and emotional stress. This vasoconstriction is mediated solely by alpha 2C-adrenoceptors (α(2C)-AR) expressed in vascular smooth muscle cells of dermal arterioles. Several factors, among which is cigarette smoking, are associated with aggravated symptoms of and increased risk for RP. Evidence shows that cigarette smoking induces the production of reactive oxygen species (ROS), which is a major driver of RP pathogenesis. However, the exact mechanism by which smoking contributes to RP or α(2C)-AR remains unclear. Here, we show that cigarette smoke extract (CSE) upregulates the expression of α(2C)-AR in a concentration- and time-dependent manner in VSMCs extracted from human dermal arterioles. This increase is associated with the activation of p38 MAPK, as pretreatment with SB-202190, a p38 specific inhibitor, attenuated CSE-induced α(2C)-AR expression. Furthermore, our results show that CSE induces ROS production followed by increased RhoA activation. We also show that CSE induces translocation of vascular α(2C)-AR to the plasma membrane, and that this mobilization is attenuated by inhibiting ROS via N-acetylcysteine or apocynin. Similarly, inhibition of Rho kinase via H- 11522 abolished CSE-induced α(2C)-AR translocation. Collectively, these results indicate that CSE activates two different signaling pathways to induce the expression and the translocation of α(2C)-AR. While CSE activates a p38-dependent mechanism to increase α(2C)-AR expression, it initiates the receptor's spatial and functional rescue via a ROS/RhoA signaling pathway. These results provide mechanistic insight into the effect of cigarette smoking on RP, and further reinforce that smoking avoidance/cessation is critical to manage this disease, especially in the absence of a definitive drug for RP. CI - Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Fardoun, Manal M AU - Fardoun MM AD - Faculty of Medicine, American University of Beirut, Beirut, P.O. Box 11-0236, Lebanon. FAU - Matar, Ayah AU - Matar A AD - Mayo Clinic, MN, Rochester, USA. FAU - Khachab, Maha AU - Khachab M AD - Department of Biomedical Sciences, Faculty of Medicine and Medical Sciences, University of Balamand Beirut, Lebanon. FAU - Dakroub, Ali H AU - Dakroub AH AD - Blavatnik Family Research Institute, Departments of Cardiology and Population Health Science and Policy, Icahn School of Medicine at Mount Sinai, New York, NY, USA. FAU - Eid, Ali H AU - Eid AH AD - Department of Basic Medical Sciences, College of Medicine, QU Health, Qatar University, P.O. Box 2713, Doha, Qatar. Electronic address: ali.eid@qu.edu.qa. LA - eng PT - Journal Article DEP - 20250109 PL - United States TA - Prog Cardiovasc Dis JT - Progress in cardiovascular diseases JID - 0376442 RN - 0 (Reactive Oxygen Species) RN - EC 2.7.11.24 (p38 Mitogen-Activated Protein Kinases) RN - 0 (Receptors, Adrenergic, alpha-2) RN - 0 (Smoke) RN - EC 3.6.5.2 (rhoA GTP-Binding Protein) RN - 124671-05-2 (RHOA protein, human) RN - EC 2.7.11.1 (rho-Associated Kinases) SB - IM MH - Humans MH - *Reactive Oxygen Species/metabolism MH - *p38 Mitogen-Activated Protein Kinases/metabolism/antagonists & inhibitors MH - *Muscle, Smooth, Vascular/enzymology/drug effects/pathology MH - *Myocytes, Smooth Muscle/drug effects/enzymology/pathology MH - Cells, Cultured MH - *Receptors, Adrenergic, alpha-2/metabolism/drug effects MH - Signal Transduction MH - *Raynaud Disease/enzymology/etiology/physiopathology MH - *Smoke/adverse effects MH - *rhoA GTP-Binding Protein/metabolism MH - Protein Transport MH - *Cigarette Smoking/adverse effects MH - Arterioles/enzymology/drug effects MH - *rho-Associated Kinases/metabolism OTO - NOTNLM OT - Cigarette smoke extract OT - Raynaud's phenomenon OT - Reactive oxygen species OT - α(2C)-adrenoceptor COIS- Declaration of competing interest None. EDAT- 2025/01/11 13:57 MHDA- 2025/05/24 00:45 CRDT- 2025/01/10 19:17 PHST- 2025/01/07 00:00 [received] PHST- 2025/01/07 00:00 [accepted] PHST- 2025/05/24 00:45 [medline] PHST- 2025/01/11 13:57 [pubmed] PHST- 2025/01/10 19:17 [entrez] AID - S0033-0620(25)00002-7 [pii] AID - 10.1016/j.pcad.2025.01.002 [doi] PST - ppublish SO - Prog Cardiovasc Dis. 2025 Mar-Apr;89:119-126. doi: 10.1016/j.pcad.2025.01.002. Epub 2025 Jan 9. PMID- 22286653 OWN - NLM STAT- MEDLINE DCOM- 20120529 LR - 20131121 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 52 IP - 1 DP - 2012 Jan-Feb TI - Elevated serum homocysteine levels in paediatric patients with primary Raynaud's phenomenon. PG - 128-30 LID - S0482-50042012000100014 [pii] AB - INTRODUCTION: Raynaud's phenomenon (RP) is a paroxysmal and recurrent acral ischemia resulting from an abnormal arterial vasospastic response to cold or emotional stress. Homocysteine, a sulphured amino acid, has been linked to cardiovascular and neurodegenerative diseases, diabetes, thrombosis, and bone fragility. Homocysteine has been also linked to the pathogenesis of RP, as increased serum homocysteine (S-homocysteine) levels were observed in patients with RP. OBJECTIVE: As all publications concerning S-homocysteine in RP involved only adult patients, our aim was to evaluate S-homocysteine in children and adolescents with RP. METHODS: Nineteen patients (two boys and 17 girls; mean age 16.1 ± 2.2 SD) with primary RP were enrolled. The controls were 51 children and adolescents (21 boys and 30 girls; mean age 15.1 ± 1.8 SD). RESULTS: The S-homocysteine level was significantly higher in the RP group in comparison with controls (11.2 ± 2.4 vs. 8.0 ± 2.0 µmol/L; P = 0.00001). S-homocysteine levels in RP were not age-dependent. CONCLUSION: Paediatric patients with RP have increased S-homocysteine levels, suggesting that homocysteine plays an important role in the development of vascular dysfunction, even at an early age. FAU - Kutilek, Stepan AU - Kutilek S AD - Departamento de Pediatria, Hospital Pardubice, Czech Republic. stepan.kutilek@nemocnice-pardubice.cz FAU - Nemec, Vladimir AU - Nemec V FAU - Bockayova, Eva AU - Bockayova E LA - eng LA - por PT - Journal Article PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0LVT1QZ0BA (Homocysteine) SB - IM MH - Adolescent MH - Child MH - Female MH - Homocysteine/*blood MH - Humans MH - Male MH - Raynaud Disease/*blood MH - Young Adult EDAT- 2012/01/31 06:00 MHDA- 2012/05/30 06:00 CRDT- 2012/01/31 06:00 PHST- 2011/03/15 00:00 [received] PHST- 2011/11/02 00:00 [accepted] PHST- 2012/01/31 06:00 [entrez] PHST- 2012/01/31 06:00 [pubmed] PHST- 2012/05/30 06:00 [medline] AID - S0482-50042012000100014 [pii] PST - ppublish SO - Rev Bras Reumatol. 2012 Jan-Feb;52(1):128-30. PMID- 24515450 OWN - NLM STAT- MEDLINE DCOM- 20150206 LR - 20151119 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 66 IP - 10 DP - 2014 Oct TI - Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity. PG - 1520-7 LID - 10.1002/acr.22304 [doi] AB - OBJECTIVE: To investigate whether patients affected by 1 of the 3 subsets of early systemic sclerosis (SSc; scleroderma), i.e., subset I, Raynaud's phenomenon with SSc marker autoantibodies and typical capillaroscopic findings; subset II, autoantibody positive only; and subset III, capillaroscopy positive only and not satisfying the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SSc at admission, differ from each other in the time to satisfy the criteria. METHODS: Early SSc patients subdivided into the 3 subsets indicated above consecutively admitted to a rheumatology/angiology center were monitored for 12-102 months (median 36 months). Patients were reevaluated twice yearly to assess whether and when each patient satisfied the new ACR/EULAR classification criteria for SSc. Patients with undifferentiated connective tissue disease (UCTD) served as the comparator group. RESULTS: During followup, 11 (52.3%) of 21 subset I, 10 (66.6%) of 15 subset II, 0 of 24 subset III, and 0 of 44 UCTD patients satisfied the criteria (P = 0.0001). The difference was significant between early SSc and UCTD patients (P = 0.0001) and, within the group of early SSc patients, between each of the 2 autoantibody-positive subsets (subsets I and II) and the capillaroscopic-positive/autoantibody-negative subset (subset I versus III: P = 0.0001; subset II versus III: P = 0.0009). There was no difference between the 2 autoantibody-positive subsets (P = 0.454). In addition to marker autoantibody positivity, preclinical lung or heart involvement was associated with an increased risk to satisfy the criteria during followup. CONCLUSION: Our data demonstrated faster progression of SSc in autoantibody-positive patients, particularly in those with preclinical internal organ involvement at baseline, than in autoantibody-negative patients. CI - Copyright © 2014 by the American College of Rheumatology. FAU - Valentini, Gabriele AU - Valentini G AD - Second University of Naples, Naples, Italy. FAU - Marcoccia, Antonella AU - Marcoccia A FAU - Cuomo, Giovanna AU - Cuomo G FAU - Vettori, Serena AU - Vettori S FAU - Iudici, Michele AU - Iudici M FAU - Bondanini, Francesco AU - Bondanini F FAU - Santoriello, Carlo AU - Santoriello C FAU - Ciani, Aldo AU - Ciani A FAU - Cozzolino, Domenico AU - Cozzolino D FAU - De Matteis, Giovanni Maria AU - De Matteis GM FAU - Cappabianca, Salvatore AU - Cappabianca S FAU - Vitelli, Filiberto AU - Vitelli F FAU - Spanò, Alberto AU - Spanò A LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Case-Control Studies MH - Disease Progression MH - Early Diagnosis MH - Female MH - Humans MH - Italy MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/blood/*diagnosis/immunology/pathology MH - Scleroderma, Systemic/blood/*diagnosis/immunology/pathology MH - Time Factors MH - Young Adult EDAT- 2014/02/12 06:00 MHDA- 2015/02/07 06:00 CRDT- 2014/02/12 06:00 PHST- 2013/10/19 00:00 [received] PHST- 2014/02/04 00:00 [accepted] PHST- 2014/02/12 06:00 [entrez] PHST- 2014/02/12 06:00 [pubmed] PHST- 2015/02/07 06:00 [medline] AID - 10.1002/acr.22304 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2014 Oct;66(10):1520-7. doi: 10.1002/acr.22304. PMID- 35737067 OWN - NLM STAT- MEDLINE DCOM- 20220627 LR - 20220728 IS - 2072-6651 (Electronic) IS - 2072-6651 (Linking) VI - 14 IP - 6 DP - 2022 Jun 14 TI - Botulinum Toxin Type A for the Treatment of Skin Ulcers: A Review Article. LID - 10.3390/toxins14060406 [doi] LID - 406 AB - The normal biological wound healing process consists of three precisely and highly programmed phases that require optimal conditions including internal and external factors. Any negative factors that disrupt the sequence or time frame of the healing mechanism can result in a non-healing wound or chronic ulcers. Botulinum neurotoxin A (BoNT-A) which is generally known as anti-contraction of muscles has been reported as a successful treatment in various types of chronic ulcers. The aim of this study is to review the outcome of treatment with BoNT-A for chronic skin ulcers. The results demonstrated some positive effects of BoNT-A on chronic ulcers. Ischemic ulcers secondary to Raynaud's phenomenon seem to be the most promising type of ulcers that have benefited from BoNT-A. The rationale behind using BoNT-A to fasten the wound healing process is also discussed. Further clinical trial studies should be conducted to affirm the efficacy of wound healing using BoNT-A administration. FAU - Winayanuwattikun, Waranaree AU - Winayanuwattikun W AD - Division of Dermatology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand. FAU - Vachiramon, Vasanop AU - Vachiramon V AUID- ORCID: 0000-0002-7328-939X AD - Division of Dermatology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand. LA - eng PT - Journal Article PT - Review DEP - 20220614 PL - Switzerland TA - Toxins (Basel) JT - Toxins JID - 101530765 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/pharmacology/therapeutic use MH - Humans MH - *Raynaud Disease/drug therapy MH - *Skin Ulcer/drug therapy MH - Ulcer/drug therapy MH - Wound Healing PMC - PMC9230442 OTO - NOTNLM OT - BoNT-A OT - Raynaud’s phenomenon OT - ischemia OT - neuromodulators OT - neurotoxin OT - wound OT - wound healing process COIS- The authors have stated explicitly that there are no conflicts of interest in connection with this article. EDAT- 2022/06/24 06:00 MHDA- 2022/06/28 06:00 PMCR- 2022/06/14 CRDT- 2022/06/23 11:14 PHST- 2022/05/20 00:00 [received] PHST- 2022/06/09 00:00 [revised] PHST- 2022/06/11 00:00 [accepted] PHST- 2022/06/23 11:14 [entrez] PHST- 2022/06/24 06:00 [pubmed] PHST- 2022/06/28 06:00 [medline] PHST- 2022/06/14 00:00 [pmc-release] AID - toxins14060406 [pii] AID - toxins-14-00406 [pii] AID - 10.3390/toxins14060406 [doi] PST - epublish SO - Toxins (Basel). 2022 Jun 14;14(6):406. doi: 10.3390/toxins14060406. PMID- 28975961 OWN - NLM STAT- MEDLINE DCOM- 20180725 LR - 20250623 IS - 1424-3997 (Electronic) IS - 0036-7672 (Linking) VI - 147 DP - 2017 TI - Outcomes, rates and predictors of transition of isolated Raynaud's phenomenon: a systematic review and meta-analysis. PG - w14506 LID - 10.4414/smw.2017.14506 [doi] AB - QUESTIONS: Published studies lack clear indicators of risk and predictors of transition from Raynaud's phenomenon (Rp) to connective tissue diseases (CTDs). Therefore, we aimed to study the outcomes, rates and predictors of transition to CTDs in patients with Rp. METHODS: A sensitive search was developed in Medline and Embase. Observational studies reporting incidence and risk factors of transition from Rp to a CTD were analysed by two independent reviewers. The main outcome was the rate of transition to a CTD; the secondary outcome was the evaluation of predictors. RESULTS: Of 856 articles captured, 7 selected studies met the inclusion criteria. A total of 4051 patients with primary Rp (pRp) and 1220 transitions to overt CTDs were recorded. The mean incidence rate of transition from pRp to a CTD was 2.65/100 person-years (standard error [SE] 1.2, 95% confidence interval [CI] 0.44-5.73). A total of 657 patients with suspected secondary Rp (ssRp) had antinuclear antibodies (ANAs) and/or capillary abnormalities; 188 transitions to CTDs were recorded, the mean incidence rate of transition from ssRp to CTD was 11.01/100 person-years (SE 4.0, 95% CI 0.11-22.12), and 135 transitions to systemic sclerosis (SSc), giving a mean incidence rate of transition from ssRp to SSc of 5.7/100 person-years (SE 2.19, 95% CI 1.02-13.19). With respect to patients with pRp, having ANAs without capillary abnormalities was associated with a risk for developing a CTD (pooled relative risks [RR] 7.63, 95% CI 2.87-20.29), whereas capillary abnormalities without ANAs resulted in a weaker risk of CTD transition (RR 5.53, 95% CI 1.45-21.06). The coexistence of ANAs and abnormal capillaroscopy significantly increased the risk of transition to CTD (RR 16.96, 95% CI 6.61-43.55). CONCLUSIONS: A low incidence rate of transition from pRp to overt CTD was found. In spite of a possible study selection bias, ssRp appears to have a strong risk of transition to a CTD when there is concomitant presence of ANAs and abnormal capillaroscopy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Pini, Dept. of Clinical Sciences and Community Health, Università degli Studi di Milano, Italy. FAU - Ughi, Nicola AU - Ughi N AD - Division of Rheumatology, ASST Pini, Dept. of Clinical Sciences and Community Health, Università degli Studi di Milano, Italy. FAU - Crotti, Chiara AU - Crotti C AD - Division of Rheumatology, ASST Pini, Dept. of Clinical Sciences and Community Health, Università degli Studi di Milano, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Tani, Chiara AU - Tani C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20170930 PL - Switzerland TA - Swiss Med Wkly JT - Swiss medical weekly JID - 100970884 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/analysis MH - Connective Tissue Diseases/*etiology MH - Humans MH - Raynaud Disease/*complications/*immunology MH - Risk Factors EDAT- 2017/10/05 06:00 MHDA- 2018/07/26 06:00 CRDT- 2017/10/05 06:00 PHST- 2017/10/05 06:00 [entrez] PHST- 2017/10/05 06:00 [pubmed] PHST- 2018/07/26 06:00 [medline] AID - smw-14506 [pii] AID - 10.4414/smw.2017.14506 [doi] PST - epublish SO - Swiss Med Wkly. 2017 Sep 30;147:w14506. doi: 10.4414/smw.2017.14506. eCollection 2017. PMID- 26004614 OWN - NLM STAT- MEDLINE DCOM- 20161109 LR - 20181202 IS - 2284-0729 (Electronic) IS - 1128-3602 (Linking) VI - 19 IP - 9 DP - 2015 TI - Local sympathetic stimulation not only have local effects in patients with Raynaud's phenomenon. PG - 1711-5 LID - 8896 [pii] AB - OBJECTIVE: Many other organs and system can be affected in the course of Primary Raynaud's Phenomenon (RP). Simultaneously increased vasospasm in the pulmonary vascular bed may likely affect the pulmonary function. Therefore, we investigated the effect of Raynaud's phenomenon on the respiratory functions in this study. PATIENTS AND METHODS: Between March 2014 and December 2014, 30 patients with the diagnosis of PRP more than two years and 32 age-sex matched healthy controls were enrolled into this study. Cold stimulation test (CST) was performed. Pulmonary function test were performed following 30 minutes after CST and spirometric measurements were calculated. RESULTS: There were no statistically significant differences between two groups regarding their demographic and clinical data. Mean duration of symptoms from onset to present was 3.01 ± 1.05 years. Patients with Primary RP had significantly lower FVC and higher FEV1/FVC values compared to the control groups (p = 0.015 and p=0.045, respectively). CONCLUSIONS: We found that statistically significant decrease of FVC values in patients with Primary RP compared to the healthy controls could be a impaired innervation of pulmonary system and a predictor of pulmonary vasospasm and/or pulmonary Raynaud's phenomenon, which may develop in future periods. FAU - Karabacak, K AU - Karabacak K AD - Department of Cardiovascular Surgery, Gulhane Military Academy of Medicine, Ankara, Turkey. kubilaykarabacak@yahoo.com. FAU - Dogan, D AU - Dogan D FAU - Celik, M AU - Celik M FAU - Kaya, E AU - Kaya E FAU - Kadan, M AU - Kadan M FAU - Isik, H AU - Isik H FAU - Ocal, N AU - Ocal N FAU - Doganci, S AU - Doganci S FAU - Yildirim, V AU - Yildirim V FAU - Demirkilic, U AU - Demirkilic U LA - eng PT - Journal Article PL - Italy TA - Eur Rev Med Pharmacol Sci JT - European review for medical and pharmacological sciences JID - 9717360 SB - IM MH - Case-Control Studies MH - Cold Temperature MH - Female MH - Humans MH - Male MH - Prospective Studies MH - *Pulmonary Circulation MH - Raynaud Disease/*physiopathology MH - Spirometry MH - Young Adult EDAT- 2015/05/26 06:00 MHDA- 2016/11/10 06:00 CRDT- 2015/05/26 06:00 PHST- 2015/05/26 06:00 [entrez] PHST- 2015/05/26 06:00 [pubmed] PHST- 2016/11/10 06:00 [medline] AID - 8896 [pii] PST - ppublish SO - Eur Rev Med Pharmacol Sci. 2015;19(9):1711-5. PMID- 26460379 OWN - NLM STAT- MEDLINE DCOM- 20161214 LR - 20260127 IS - 1880-8026 (Electronic) IS - 0019-8366 (Print) IS - 0019-8366 (Linking) VI - 53 IP - 6 DP - 2015 TI - Human response to vibration stress in Japanese workers: lessons from our 35-year studies A narrative review. PG - 522-32 LID - 10.2486/indhealth.2015-0040 [doi] AB - The occupational uses with vibratory tools or vehicles provoked health disorders of users. We reviewed narratively our articles of 35 yr studies and their related literatures, and considered the pathophysiology of the hand-arm vibration disorders. Concerning the risk factors of health impairments in workers with vibratory tools, there are two conflicting schools of the researchers: The peripheral school emphasizes that vibration only makes predominant impairments on hands and arms, showing typically Raynaud's phenomenon in the fingers. In the systemic school, the health disorders are produced by combination with vibration, noise and working environment, namely vibratory work itself, leading to diversified symptoms and signs in relation to systemic impairments. Our 35 yr studies have evidently supported the systemic school, including disorders of the central and autonomic nervous systems. The genesis is vibratory work itself, including vibration, noise, cold working environment, ergonomic and biodynamic conditions, and emotional stress in work. Because the health disorders yield in the whole body, the following measures would contribute to the prevention of health impairments: the attenuation of vibration and noise generated form vibratory machines and the regulations on operating tool hours. In conclusion, this occupational disease results from systemic impairments due to long-term occupational work with vibratory tools. FAU - Matoba, Tsunetaka AU - Matoba T AD - Kurume University School of Medicine, Japan. LA - eng PT - Journal Article PT - Review DEP - 20151010 PL - Japan TA - Ind Health JT - Industrial health JID - 2985065R SB - IM MH - Animals MH - Autonomic Nervous System/*physiopathology MH - Cold Temperature/adverse effects MH - Ergonomics MH - Hand-Arm Vibration Syndrome/diagnosis/*etiology/*physiopathology/therapy MH - Humans MH - Japan MH - Noise, Occupational/adverse effects MH - Occupational Health MH - Raynaud Disease/etiology/physiopathology MH - Risk Factors MH - Stress, Psychological/psychology MH - Vibration/*adverse effects PMC - PMC4667043 EDAT- 2015/10/16 06:00 MHDA- 2016/12/15 06:00 PMCR- 2015/11/01 CRDT- 2015/10/14 06:00 PHST- 2015/10/14 06:00 [entrez] PHST- 2015/10/16 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] PHST- 2015/11/01 00:00 [pmc-release] AID - 2015-0040 [pii] AID - 10.2486/indhealth.2015-0040 [doi] PST - ppublish SO - Ind Health. 2015;53(6):522-32. doi: 10.2486/indhealth.2015-0040. Epub 2015 Oct 10. PMID- 22132698 OWN - NLM STAT- MEDLINE DCOM- 20120522 LR - 20131121 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 39 IP - 2 DP - 2012 Feb TI - Three siblings with systemic lupus erythematosus. PG - 164-7 LID - 10.1111/j.1346-8138.2011.01333.x [doi] AB - We present the cases of three siblings with systemic lupus erythematosus (SLE). The diagnosis was made when the sisters were of age 21, 25 and 28 years. They shared some clinical features, including typical facial rash, photosensitivity and Raynaud's phenomenon, and tested positive for antinuclear antibodies. However, their symptoms and clinical courses varied. Human leukocyte antigen (HLA) typing revealed that DR4 and A2 were present in all three sisters, while HLA type A11, B35 and B54 were each found in two of the three sisters. The two elder sisters developed lupus glomerulonephritis 8 and 11 years after the onset of SLE. It is suggested that there are genes responsible for the onset of the disease and also unknown regulatory genes other than HLA result in different phenotypes. CI - © 2011 Japanese Dermatological Association. FAU - Shu, En AU - Shu E AD - Department of Dermatology, Gifu University Graduate School of Medicine Department of Dermatology, Kizawa Memorial Hospital, Gifu, Japan. gif142@gifu-u.ac.jp FAU - Ichiki, Yoshiro AU - Ichiki Y FAU - Moriya, Chie AU - Moriya C FAU - Iwata, Hiroaki AU - Iwata H FAU - Kitajima, Yasuo AU - Kitajima Y FAU - Seishima, Mariko AU - Seishima M LA - eng PT - Case Reports PT - Journal Article DEP - 20111202 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Antibodies, Antinuclear) RN - 0 (Dermatologic Agents) RN - 0 (HLA Antigens) RN - 9PHQ9Y1OLM (Prednisolone) RN - MRK240IY2L (Azathioprine) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood/immunology MH - Azathioprine/therapeutic use MH - Dermatologic Agents/therapeutic use MH - Exanthema/diagnosis MH - Female MH - HLA Antigens/analysis/genetics/immunology MH - Humans MH - Lupus Erythematosus, Systemic/*diagnosis/drug therapy/genetics MH - Photosensitivity Disorders/diagnosis/immunology MH - Prednisolone/therapeutic use MH - Raynaud Disease/diagnosis/immunology MH - Siblings MH - Treatment Outcome MH - Young Adult EDAT- 2011/12/03 06:00 MHDA- 2012/05/23 06:00 CRDT- 2011/12/03 06:00 PHST- 2011/12/03 06:00 [entrez] PHST- 2011/12/03 06:00 [pubmed] PHST- 2012/05/23 06:00 [medline] AID - 10.1111/j.1346-8138.2011.01333.x [doi] PST - ppublish SO - J Dermatol. 2012 Feb;39(2):164-7. doi: 10.1111/j.1346-8138.2011.01333.x. Epub 2011 Dec 2. PMID- 20186674 OWN - NLM STAT- MEDLINE DCOM- 20100430 LR - 20210409 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 39 IP - 1 DP - 2010 Feb TI - Functional vascular diseases: Raynaud's syndrome, acrocyanosis and erythromelalgia. PG - 33-41 LID - 10.1024/0301-1526/a000003 [doi] AB - Raynauds syndrome, acrocyanosis and erythromelalgia are functional vascular diseases that differ with respect to prevalence, clinical picture, therapy, prognosis, and impairment of quality of life. Raynauds syndrome occurs in 5 to 20 % of the population in Europe, is observed four times more often in women than in men and appears first at the age of 40 (3 to 80), on the average. Raynauds attacks are characterized by a paroxysmal white-blue-red or just white and blue discoloration of the fingers and toes; the attacks are induced by cold or stress, usually, cease after no more than some minutes (average 23 min.), but can also persist for hours. A distinction must be made between primary (aetiology unknown), secondary (aetiology known) and suspected secondary Raynauds syndromes (causal underlying disease suspected). There are several different therapy options, but not all of them have been substantiated by evidence. Acrocyanosis is rarer than Raynauds syndrome, and contrary to the latter, is characterized by nonparoxysmal, in most cases persistent, painless bluish-red symmetrical discolorations of the hands, feet and knees. It is more frequent in women than in men and becomes manifest before the 25th year of age, on the average (15th to 70th year of age). A distinction is made between primary acrocyanosis without detectable underlying disease and secondary acrocyanosis with a specific underlying disease. No effective therapy for primary acrocyanosis is known, but secondary forms can sometimes be treated. Patients with primary and secondary erythromelalgia, a very rare condition, sustain paroxysmal burning pain with marked reddening of the legs, feet and less often the hands. The attacks are triggered by warmth. Women are affected more often than men. The age of first manifestation is 40 to 55 years, but the first attacks may just as well occur during childhood. There are different therapeutic approaches with occasional success, but no general recommendations. FAU - Heidrich, H AU - Heidrich H AD - Prof.Heidrich@web.de LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Child, Preschool MH - Erythromelalgia/*diagnosis/epidemiology MH - Female MH - Heart Defects, Congenital/*epidemiology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/epidemiology MH - Sex Ratio MH - Vascular Diseases/*classification RF - 53 EDAT- 2010/02/27 06:00 MHDA- 2010/05/01 06:00 CRDT- 2010/02/27 06:00 PHST- 2010/02/27 06:00 [entrez] PHST- 2010/02/27 06:00 [pubmed] PHST- 2010/05/01 06:00 [medline] AID - 10.1024/0301-1526/a000003 [doi] PST - ppublish SO - Vasa. 2010 Feb;39(1):33-41. doi: 10.1024/0301-1526/a000003. PMID- 18709692 OWN - NLM STAT- MEDLINE DCOM- 20081211 LR - 20220408 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 9 DP - 2008 Sep TI - Statins: potentially useful in therapy of systemic sclerosis-related Raynaud's phenomenon and digital ulcers. PG - 1801-8 AB - OBJECTIVE: Systemic sclerosis (SSc) is characterized by fibrosis and widespread vascular pathology. Raynaud's phenomenon (RP) and digital ulceration are prominent manifestations of vascular involvement in SSc. Digital ulcers (DU) remain a serious complication, and effective therapy remains elusive. Statins display pleiotropic effects on endothelial function that may potentially retard vascular injury. We evaluated the potential efficacy of statin therapy in endothelial dysfunction and in management of RP and DU. METHODS: Eighty-four SSc patients who fulfilled the American College of Rheumatology criteria for classification of SSc with secondary RP despite ongoing vasodilator therapy were randomized into 2 groups; the first group (n = 56) received 40 mg/day atorvastatin for 4 months; the second group (n = 28) received placebo. Seventy-five healthy volunteers served as controls. Assessment of RP and DU was performed monthly. The primary outcome measure was the number of DU. Secondary endpoints included the modified Scleroderma Health Assessment Questionnaire Disability Index (SHAQ-DI), safety, and tolerability. Measurement of functional status in relation to RP included the SHAQ-DI, visual analog scale (VAS) for RP, DU and pain scales, and VAS for physician's global assessment for health. Endothelial damage markers were also assessed. Endothelium-dependent flow-mediated dilatation was measured by high-resolution echo-Doppler ultrasonography. RESULTS: The overall number of DU was significantly reduced in the statin group. Among patients in the statin group a mean of 1.6 new ulcers developed per patient compared to 2.5 new ulcers per patient in the placebo group. There was a statistically significant improvement in SHAQ-DI score in patients receiving statin versus those on placebo. VAS for RP, DU severity, and pain scales and the VAS for physician global assessment improved significantly in the statin group compared to the placebo group. Endothelial markers of activation showed statistically significant improvement from baseline values in the statin versus the placebo group. CONCLUSION: Our results showed that statins retarded vascular injury and improved patient function. The findings suggest that statins may aid in treating RP and DU in SSc patients. Given the safety and relative low cost of statins and good patient tolerability to this class of drugs, statins may be of clinical benefit in SSc patients. FAU - Abou-Raya, Anna AU - Abou-Raya A AD - Department of Rheumatology, University of Alexandria, Alexandria, Egypt. annaaraya@yahoo.com FAU - Abou-Raya, Suzan AU - Abou-Raya S FAU - Helmii, Madihah AU - Helmii M LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20080815 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Heptanoic Acids) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Pyrroles) RN - A0JWA85V8F (Atorvastatin) SB - IM CIN - Nat Clin Pract Rheumatol. 2009 Feb;5(2):70-1. doi: 10.1038/ncprheum0993. PMID: 19182811 MH - Atorvastatin MH - Double-Blind Method MH - Female MH - Fingers MH - Health Status MH - Heptanoic Acids/*therapeutic use MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/*therapeutic use MH - Male MH - Middle Aged MH - Pain/drug therapy MH - Pain Measurement MH - Pyrroles/*therapeutic use MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/complications/*drug therapy/physiopathology MH - Severity of Illness Index MH - Skin Ulcer/*drug therapy/etiology/physiopathology MH - Surveys and Questionnaires MH - Treatment Outcome EDAT- 2008/08/19 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/08/19 09:00 PHST- 2008/08/19 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/08/19 09:00 [entrez] AID - 08/13/0815 [pii] PST - ppublish SO - J Rheumatol. 2008 Sep;35(9):1801-8. Epub 2008 Aug 15. PMID- 17550096 OWN - NLM STAT- MEDLINE DCOM- 20070830 LR - 20071203 IS - 0738-0658 (Print) IS - 0738-0658 (Linking) VI - 25 IP - 4 DP - 2006 Dec TI - Clinical manifestations and vascular events in patients with lupus erythematosus anticardiolipin antibodies and raynaud's phenomenon. PG - 307-13 AB - BACKGROUND: Raynaud's phenomenon (RP) and anticardiolipin antibodies (ACL) are two common clinical manifestations in patients with systemic lupus erythematosus (SLE). RP may lead to digital or limb loss. ACL are associated to thrombotic episodes. It is not yet clear if there is an association between RP and the presence of ACL in patients with SLE. OBJECTIVES: To study if the presence of both RP and ACL in patients with SLE may be associated with certain clinical manifestations or thrombotic events compared to SLE patients without RP or ACL. METHODS: SLE patients from two lupus clinics were recruited. The patients were divided into 4 groups. Patients with RP and positive ACL (RP+ ACL+), patients with RP but negative ACL (RP+ ACL-), patients with negative RP and positive ACL (RP- ACL+), and patients that were negative for RP and ACL (RP- ACL-) used as the control group. Demographic data, diagnostic criteria, clinical manifestations, history of arterial thrombosis, venous thrombosis and abortions were recorded. A physical examination was done. Anticardiolipin antibodies IgG and IgM were done in the rheumatology laboratory at the University of Puerto Rico School of Medicine. Descriptive statistics as well as analysis of variances (ANOVA), and polytomous logistic regression were used. RESULTS: 236 patients with SLE were studied. There was a tendency toward an increase in arterial thrombosis (p-value = 0.094) and venous thrombosis (p-value = 0.067) in the group that were positive for RP and ACL (RP+ ACL+). Although it was not statistical significant, when polytomous logistic regression was used, both arterial and venous thrombosis had an increase in relative risk 3.21 for arterial and 3.11 for venous thrombosis. Abortions were not increased in any of the four groups. Clinical manifestations from SLE did not differ among the four groups. CONCLUSIONS: Patients with both RP and ACL seem to be at an increase risk for both arterial and venous thrombotic events; these patients may benefit from an antiplatelet medication to prevent these events to occur. FAU - Rodriguez, Vanessa E AU - Rodriguez VE AD - Rheumatology Section, Department of Internal Medicine, University of Puerto Rico School of Medicine, Sun Juan 00936-5067. varodriguez@rcm.upr.edu FAU - Gonzalez-Pares, Esther N AU - Gonzalez-Pares EN FAU - Rivera, Cynthia AU - Rivera C LA - eng GR - 1P20 RR 11126/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - Puerto Rico TA - P R Health Sci J JT - Puerto Rico health sciences journal JID - 8303541 RN - 0 (Antibodies, Anticardiolipin) SB - IM MH - Adult MH - Antibodies, Anticardiolipin/*blood MH - Cross-Sectional Studies MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*blood/*complications MH - Male MH - Raynaud Disease/*blood/*complications MH - Thrombosis/*blood/*etiology EDAT- 2007/06/07 09:00 MHDA- 2007/08/31 09:00 CRDT- 2007/06/07 09:00 PHST- 2007/06/07 09:00 [pubmed] PHST- 2007/08/31 09:00 [medline] PHST- 2007/06/07 09:00 [entrez] PST - ppublish SO - P R Health Sci J. 2006 Dec;25(4):307-13. PMID- 30289161 OWN - NLM STAT- MEDLINE DCOM- 20191028 LR - 20191028 IS - 1600-0412 (Electronic) IS - 0001-6349 (Linking) VI - 98 IP - 2 DP - 2019 Feb TI - Long-term neurotoxicity and Raynaud's phenomenon in patients treated with cisplatin-based chemotherapy for malignant ovarian germ cell tumor. PG - 240-249 LID - 10.1111/aogs.13477 [doi] AB - INTRODUCTION: The aim was to evaluate "overall neuropathy", defined as peripheral paresthesia and Raynaud's phenomenon, in long-term survivors of malignant ovarian germ cell tumors (MOGCTs) treated with cisplatin-based chemotherapy (CBCT). MATERIAL AND METHODS: Ninety-three MOGCT survivors recorded in Norway in 1980-2009 (median follow up: 15 years) were included in this analysis. Forty-nine received CBCT (CBCT group) and 44 received other or no chemotherapy (non-CBCT group). Applying the scale for chemotherapy-induced neurotoxicity, the prevalence of overall neuropathy (ie score >1 on a 0-3 scale) was compared between the two groups. Forty women from the CBCT group also underwent neurophysiological and neurological examinations; results from the neurological examination were graded according to the National Cancer Institute-Common Terminology Criteria for Adverse Effects version 4 (NCI-CTCAE scale v4). These women were then categorized into subgroups of low (≤3 cycles of CBCT, n = 23) and high CBCT (≥4 cycles of CBCT, n = 17). RESULTS: Twenty-eight (57%) women from the CBCT group reported overall neuropathy, compared with 20 (45%) in the non-CBCT group (P = .06). Of the 40 MOGCT survivors in the CBCT group who underwent neurophysiological and neurological examinations, 14 (35%) showed NCI-CTCAE grade ≥1 signs or symptoms of peripheral neuropathy. Pathological findings of NCI-CTCAE grade 2 or 3 signs or symptoms were found in four survivors (10%) from the high CBCT subgroup, all of whom also showed objective signs of neuropathy. CONCLUSIONS: Though about half of our MOGCT survivors reported overall neuropathy after CBCT, more severe pathological neurological/neurophysiological findings that impacted daily living were recorded in only 10% of them. Our observations of a similar prevalence of self-reported overall neuropathy in the CBCT and non-CBCT group, combined with limited objective neurological findings, warrant further study to increase the understanding of the specific pathophysiological pathways of long-term CBCT-induced neuropathy. CI - © 2018 Nordic Federation of Societies of Obstetrics and Gynecology. FAU - Solheim, Olesya AU - Solheim O AUID- ORCID: 0000-0003-1936-3453 AD - Department of Gynecologic Oncology, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway. AD - National Resource Center for Late Effects after Cancer Treatment, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway. FAU - Skalleberg, Jacob AU - Skalleberg J AD - Department of Otolaryngology, Head and Neck Surgery, Oslo University Hospital Rikshospitalet, Oslo, Norway. FAU - Warncke, Torhild AU - Warncke T AD - Department of Neurology, Oslo University Hospital Rikshospitalet, Oslo, Norway. FAU - Ørstavik, Kristin AU - Ørstavik K AD - Department of Neurology, Oslo University Hospital Rikshospitalet, Oslo, Norway. FAU - Tropé, Claes AU - Tropé C AD - Department of Gynecologic Oncology, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway. AD - Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. FAU - Fosså, Sophie D AU - Fosså SD AD - National Resource Center for Late Effects after Cancer Treatment, Oslo University Hospital, Norwegian Radium Hospital, Oslo, Norway. AD - Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. LA - eng GR - 6736/South-Eastern Norway Regional Health Authority/International PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20181122 PL - United States TA - Acta Obstet Gynecol Scand JT - Acta obstetricia et gynecologica Scandinavica JID - 0370343 RN - 0 (Antineoplastic Agents) RN - Q20Q21Q62J (Cisplatin) RN - Ovarian Germ Cell Cancer SB - IM MH - Adult MH - Antineoplastic Agents/administration & dosage/adverse effects MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/adverse effects MH - Cancer Survivors MH - *Cisplatin/administration & dosage/adverse effects MH - Female MH - Humans MH - Long Term Adverse Effects/diagnosis/epidemiology MH - Middle Aged MH - Neoplasms, Germ Cell and Embryonal/*drug therapy/epidemiology/pathology MH - Norway/epidemiology MH - Ovarian Neoplasms/*drug therapy/epidemiology/pathology MH - *Paresthesia/chemically induced/diagnosis/epidemiology MH - *Peripheral Nervous System Diseases/chemically induced/diagnosis/epidemiology MH - Prevalence MH - *Raynaud Disease/chemically induced/diagnosis/epidemiology OTO - NOTNLM OT - Raynaud's phenomenon OT - cisplatin-based chemotherapy OT - malignant ovarian germ cell tumors OT - neuropathy OT - peripheral paresthesia EDAT- 2018/10/06 06:00 MHDA- 2019/10/29 06:00 CRDT- 2018/10/06 06:00 PHST- 2018/02/15 00:00 [received] PHST- 2018/09/28 00:00 [accepted] PHST- 2018/10/06 06:00 [pubmed] PHST- 2019/10/29 06:00 [medline] PHST- 2018/10/06 06:00 [entrez] AID - 10.1111/aogs.13477 [doi] PST - ppublish SO - Acta Obstet Gynecol Scand. 2019 Feb;98(2):240-249. doi: 10.1111/aogs.13477. Epub 2018 Nov 22. PMID- 32184178 OWN - NLM STAT- MEDLINE DCOM- 20210115 LR - 20210115 IS - 1873-3476 (Electronic) IS - 0378-5173 (Linking) VI - 580 DP - 2020 Apr 30 TI - Ultrasound-mediated topical delivery of econazole nitrate with potential for treating Raynaud's phenomenon. PG - 119229 LID - S0378-5173(20)30213-1 [pii] LID - 10.1016/j.ijpharm.2020.119229 [doi] AB - The study aims to assess the ultrasound-assisted econazole nitrate (EN) permeation from topically applied formulations with potential for treating Raynaud's phenomenon. Optimization of ultrasound parameters such as the distance of the horn, application time and amplitude were performed. In vitro percutaneous absorption studies were performed using econazole formulations (F2_HPMC dispersion, F4_Lipoderm® Activemax™ Cream) across the ultrasound-treated porcine skin and were compared with the control group (skin samples without ultrasound). Histology and ATR-FTIR studies were performed on treated skin samples. A constant frequency (20 kHz) ultrasound application with 40% amplitude, 0.5 cm distance between ultrasound horn and the skin surface for 2 min was optimized. The permeation of EN was found to be higher from ultrasound-treated skin samples than the control group. Drug permeation from F2_HPMC dispersion was found to be higher as compared to the other formulations and the marketed EN cream. Histological evaluation confirmed that F2_HPMC dispersion showed no signs of toxicity. ATR-FTIR studies revealed a slight increase in the CH(2) stretching vibrations (~2920 cm(-1) and 2850 cm(-1)) in ultrasound-treated skin samples as compared with the control. In conclusion, the ultrasound-assisted transdermal delivery of F2_HPMC dispersion could be further studied as a new therapy for Raynaud's phenomenon. CI - Copyright © 2020 Elsevier B.V. All rights reserved. FAU - Daftardar, Saloni AU - Daftardar S AD - College of Pharmacy and Pharmaceutical Sciences, The University of Toledo Health Science Campus, The University of Toledo, Toledo, OH 43614, USA. FAU - Bahl, Dherya AU - Bahl D AD - College of Pharmacy and Pharmaceutical Sciences, The University of Toledo Health Science Campus, The University of Toledo, Toledo, OH 43614, USA. FAU - Boddu, Sai H S AU - Boddu SHS AD - College of Pharmacy and Pharmaceutical Sciences, The University of Toledo Health Science Campus, The University of Toledo, Toledo, OH 43614, USA; Department of Pharmaceutical Sciences, College of Pharmacy and Health Sciences, Ajman University, Ajman, United Arab Emirates. Electronic address: s.boddu@ajman.ac.ae. FAU - Altorok, Nezam AU - Altorok N AD - Department of Medicine, Division of Rheumatology, University of Toledo, Toledo, USA. FAU - Kahaleh, Bashar AU - Kahaleh B AD - Department of Medicine, Division of Rheumatology, University of Toledo, Toledo, USA. LA - eng PT - Journal Article DEP - 20200314 PL - Netherlands TA - Int J Pharm JT - International journal of pharmaceutics JID - 7804127 RN - 0 (Antifungal Agents) RN - 6Z1Y2V4A7M (Econazole) SB - IM MH - Administration, Cutaneous MH - Animals MH - Antifungal Agents/administration & dosage MH - Chemistry, Pharmaceutical/methods MH - Econazole/*administration & dosage MH - Permeability MH - Raynaud Disease/*drug therapy MH - Skin/metabolism MH - Skin Absorption/physiology MH - Swine OTO - NOTNLM OT - Cold exposure OT - Econazole nitrate OT - Flux OT - HPMC OT - Lag-time OT - Permeation OT - Raynaud’s phenomenon OT - TRPM-8 OT - Topical OT - Ultrasound COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2020/03/19 06:00 MHDA- 2021/01/16 06:00 CRDT- 2020/03/19 06:00 PHST- 2020/01/08 00:00 [received] PHST- 2020/03/10 00:00 [revised] PHST- 2020/03/13 00:00 [accepted] PHST- 2020/03/19 06:00 [pubmed] PHST- 2021/01/16 06:00 [medline] PHST- 2020/03/19 06:00 [entrez] AID - S0378-5173(20)30213-1 [pii] AID - 10.1016/j.ijpharm.2020.119229 [doi] PST - ppublish SO - Int J Pharm. 2020 Apr 30;580:119229. doi: 10.1016/j.ijpharm.2020.119229. Epub 2020 Mar 14. PMID- 22761213 OWN - NLM STAT- MEDLINE DCOM- 20140204 LR - 20240325 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2012 DP - 2012 Jul 3 TI - Paraneoplastic Raynaud's phenomenon manifesting before the diagnosis of lung cancer. LID - 10.1136/bcr.03.2012.5985 [doi] LID - bcr0320125985 AB - This description pertains to a previously healthy gentleman aged 54 years who developed symptoms coherent with Raynaud's phenomenon. The patient never had any prior episodes of peripheral cyanosis. The patient's first presentation was in summer and the paroxysms of peripheral cyanosis were not associated with any specific aggravating factor. The paroxysms went on to become more severe and painful across a span of 6 months, when he also developed non-radiating pain in the right lateral chest-wall, which would aggravate after episodes of cough. A chest roentgenogram then demonstrated the presence of a mass lesion in the right lung and a fine-needle-aspiration cytology confirmed malignancy- an adenocarcinoma. There was a dramatic relief in pain and a reduction in the intensity and duration of paroxysms of peripheral cyanosis within 2-weeks of initiation of chemotherapy for lung cancer. FAU - Madabhavi, Irappa AU - Madabhavi I AD - Department of Internal Medicine, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India. FAU - Revannasiddaiah, Swaroop AU - Revannasiddaiah S FAU - Rastogi, Madhup AU - Rastogi M FAU - Gupta, Manoj Kumar AU - Gupta MK LA - eng PT - Case Reports PT - Journal Article DEP - 20120703 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0W860991D6 (Deoxycytidine) RN - Q20Q21Q62J (Cisplatin) RN - 0 (Gemcitabine) SB - IM MH - Adenocarcinoma/*complications/*diagnosis/drug therapy MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Cisplatin/administration & dosage MH - Deoxycytidine/administration & dosage/analogs & derivatives MH - Humans MH - Lung Neoplasms/*complications/*diagnosis/drug therapy MH - Male MH - Middle Aged MH - Paraneoplastic Syndromes/*etiology MH - Raynaud Disease/*etiology MH - Gemcitabine PMC - PMC3391388 COIS- Competing interests: None. EDAT- 2012/07/05 06:00 MHDA- 2014/02/05 06:00 PMCR- 2014/07/27 CRDT- 2012/07/05 06:00 PHST- 2012/07/05 06:00 [entrez] PHST- 2012/07/05 06:00 [pubmed] PHST- 2014/02/05 06:00 [medline] PHST- 2014/07/27 00:00 [pmc-release] AID - bcr.03.2012.5985 [pii] AID - 10.1136/bcr.03.2012.5985 [doi] PST - epublish SO - BMJ Case Rep. 2012 Jul 3;2012:bcr0320125985. doi: 10.1136/bcr.03.2012.5985. PMID- 19132148 OWN - NLM STAT- MEDLINE DCOM- 20090521 LR - 20191111 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 60 IP - 4 DP - 2008 Oct-Dec TI - [Nailfold capillaroscopic report: qualitative and quantitative methods]. PG - 249-53 AB - Nailfold capillaroscopy (NVC) is a simple and non-invasive method used for the assessment of patients with Raynaud's phenomenon (RP) and in the differential diagnosis of various connective tissue diseases. The scleroderma pattern abnormalities (giant capillaries, haemorrages and/or avascular areas) have a positive predictive value for the development of scleroderma spectrum disorders. Thus, an analytical approach to nailfold capillaroscopy can be useful in quantitatively and reproducibly recording various parameters. We developed a new method to assess patients with RP that is capable of predicting the 5-year transition from isolated RP to RP secondary to scleroderma spectrum disorders. This model is a weighted combination of different capillaroscopic parameters (giant capillaries, microhaemorrages, number of capillaries) that allows physicians to stratify RP patients easily using a relatively simple diagram to deduce prognosis. FAU - Lubatti, C AU - Lubatti C AD - Dipartimento e Cattedra di Reumatologia, Istituto Gaetano Pini, 20122 Milano, Italia. chiara.lubatti@virgilio.it FAU - Ingegnoli, F AU - Ingegnoli F FAU - Gualtierotti, R AU - Gualtierotti R FAU - Boracchi, P AU - Boracchi P FAU - Zahalkova, L AU - Zahalkova L FAU - Zeni, S AU - Zeni S FAU - Soldi, A AU - Soldi A FAU - Fantini, F AU - Fantini F LA - ita PT - English Abstract PT - Journal Article TT - La refertazione dell'esame capillaroscopico: metodi di valutazione qualitativi e quantitativi. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Humans MH - *Microscopic Angioscopy MH - Nails/*pathology MH - Raynaud Disease/*pathology EDAT- 2009/01/10 09:00 MHDA- 2009/05/22 09:00 CRDT- 2009/01/10 09:00 PHST- 2009/01/10 09:00 [entrez] PHST- 2009/01/10 09:00 [pubmed] PHST- 2009/05/22 09:00 [medline] AID - 10.4081/reumatismo.2008.249 [doi] PST - ppublish SO - Reumatismo. 2008 Oct-Dec;60(4):249-53. doi: 10.4081/reumatismo.2008.249. PMID- 36088751 OWN - NLM STAT- MEDLINE DCOM- 20221122 LR - 20250729 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 57 DP - 2022 Dec TI - Placebo response in Raynaud's Phenomenon clinical trials: The prominent role of regression towards the mean: Placebo response in Raynaud's Phenomenon. PG - 152087 LID - S0049-0172(22)00138-X [pii] LID - 10.1016/j.semarthrit.2022.152087 [doi] AB - BACKGROUND: Substantial placebo response has been observed in trials assessing treatments in Raynaud's Phenomenon (RP), which makes any treatment effect difficult to detect. However, whether this response is due to a real placebo effect or to other nonspecific effects, such as regression towards the mean (RTM), has not been explored. Our objectives were to explore and quantify placebo response in RP, and to evaluate the magnitude of RTM contribution. METHODS: We combined trial-level and individual-level data from a series of n-of-1 trials and a network meta-analysis, respectively. Main outcomes were the daily frequency and the mean duration of RP attacks, as well as the Raynaud's Condition Score (RCS). We estimated the placebo response by the mean difference between the placebo period (or arm) and the baseline. RTM was estimated by the relationship between placebo response and baseline, and with Galton squeeze plots. Finally, we simulated the effect of the threshold used for inclusion in clinical trials on RTM. FINDINGS: We observed a large and significant placebo response from both individual and trial data for RCS [-1.20 (-1.63, -0.77) and -0.65 (-0.89, -0.41)] and the daily frequency of RP [-0.61 (-0.85, -0.37) and -0.75 (-0.95, -0.54)]. Outcome at baseline was significantly associated with placebo response, suggesting the presence of RTM. The latter was confirmed on individual data, through Galton squeeze plots. INTERPRETATION: Placebo response is large in RP trials, and likely due to regression towards the mean rather than 'true' placebo effect. This should be carefully considered when designing future trials. FUNDING: This work has been partially supported by MIAI @ Grenoble Alpes (ANR-19-P3IA-0003). CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Roustit, Matthieu AU - Roustit M AD - Univ. Grenoble Alpes, Inserm U1300, 38000 Grenoble, France; Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. Electronic address: MRoustit@chu-grenoble.fr. FAU - Jullien, Ariane AU - Jullien A AD - Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. FAU - Jambon-Barbara, Clément AU - Jambon-Barbara C AD - Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. FAU - Goudon, Hugo AU - Goudon H AD - Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. FAU - Blaise, Sophie AU - Blaise S AD - Univ. Grenoble Alpes, Inserm U1300, 38000 Grenoble, France; Department of Vascular Medicine, CHU Grenoble Alpes, 38000 Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Univ. Grenoble Alpes, Inserm U1300, 38000 Grenoble, France; Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. FAU - Khouri, Charles AU - Khouri C AD - Univ. Grenoble Alpes, Inserm U1300, 38000 Grenoble, France; Univ. Grenoble Alpes, Inserm CIC 1406, CHU Grenoble Alpes, 38000 Grenoble, France. LA - eng SI - Dryad/10.5061/dryad.c670tq2 PT - Journal Article PT - Network Meta-Analysis PT - Research Support, Non-U.S. Gov't DEP - 20220827 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Raynaud Disease/drug therapy MH - Placebo Effect OTO - NOTNLM OT - Placebo response OT - Raynaud's phenomenon OT - Regression towards the mean COIS- Declaration of Competing Interest The authors declare no competing interest for this work. EDAT- 2022/09/12 06:00 MHDA- 2022/11/23 06:00 CRDT- 2022/09/11 18:11 PHST- 2022/04/08 00:00 [received] PHST- 2022/08/11 00:00 [revised] PHST- 2022/08/19 00:00 [accepted] PHST- 2022/09/12 06:00 [pubmed] PHST- 2022/11/23 06:00 [medline] PHST- 2022/09/11 18:11 [entrez] AID - S0049-0172(22)00138-X [pii] AID - 10.1016/j.semarthrit.2022.152087 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Dec;57:152087. doi: 10.1016/j.semarthrit.2022.152087. Epub 2022 Aug 27. PMID- 29513932 OWN - NLM STAT- MEDLINE DCOM- 20190703 LR - 20220410 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 70 IP - 6 DP - 2018 Jun TI - Patient Perceptions of the Raynaud's Condition Score Diary Provide Insight Into Its Performance in Clinical Trials of Raynaud's Phenomenon: Comment on the Article by Denton et al. PG - 973-974 LID - 10.1002/art.40481 [doi] FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases at Royal United Hospitals and University of Bath, Bath, UK. FAU - Saketkoo, Lesley A AU - Saketkoo LA AD - Tulane University School of Medicine, Scleroderma and Sarcoidosis Patient Care and Research Center and University Medical Center Comprehensive, Pulmonary Hypertension Center, New Orleans, LA. FAU - Domsic, Robyn T AU - Domsic RT AD - University of Pittsburgh Medical Center, Pittsburgh, PA. LA - eng PT - Comment PT - Letter DEP - 20180429 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Acetamides) RN - 0 (Pyrazines) RN - 5EXC0E384L (selexipag) SB - IM CON - Arthritis Rheumatol. 2017 Dec;69(12):2370-2379. doi: 10.1002/art.40242. PMID: 29193819 CIN - Arthritis Rheumatol. 2018 Jun;70(6):974. doi: 10.1002/art.40480. PMID: 29513930 MH - Acetamides MH - Adult MH - Humans MH - Pyrazines MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2018/03/08 06:00 MHDA- 2019/07/04 06:00 CRDT- 2018/03/08 06:00 PHST- 2018/03/08 06:00 [pubmed] PHST- 2019/07/04 06:00 [medline] PHST- 2018/03/08 06:00 [entrez] AID - 10.1002/art.40481 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Jun;70(6):973-974. doi: 10.1002/art.40481. Epub 2018 Apr 29. PMID- 17828538 OWN - NLM STAT- MEDLINE DCOM- 20080717 LR - 20181113 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 28 IP - 4 DP - 2008 Feb TI - Clinical features of Thai male lupus: an age-matched controlled study. PG - 339-44 AB - To study the prevalence, and clinical and laboratory manifestations of male lupus, and compare these findings with their age-matched female lupus in Thai patients. The medical records of patients with diagnosed Systemic lupus erythematosus (SLE) were reviewed. The clinical and laboratory manifestations were determined. There were 37 males in 508 patients with SLE (7.3%). There was no difference in mean +/- SD age and disease duration between male patients and their 74 female age-matched controls. When compared with females, male lupus patients had a significantly lower prevalence of alopecia (13.6 vs. 44.6%, P = 0.001), arthralgia (2.7 vs. 17.6%, P = 0.032), Raynaud's phenomenon (0.0 vs. 12.2%, P = 0.027), and psychosis (0.0 vs. 13.5%, P = 0.029), but they had a significantly higher prevalence of thrombocytopenia (32.4 vs. 12.2%, P = 0.019) and renal insufficiency (40.5 vs. 16.4%, P = 0.006). Our study showed several existing sex-related differences in the clinical manifestations in Thai SLE patients. FAU - Mongkoltanatus, Jitima AU - Mongkoltanatus J AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. FAU - Wangkaew, Suparaporn AU - Wangkaew S FAU - Kasitanon, Nuntana AU - Kasitanon N FAU - Louthrenoo, Worawit AU - Louthrenoo W LA - eng PT - Journal Article DEP - 20070906 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Alopecia/ethnology/etiology MH - Arthralgia/ethnology/etiology MH - Case-Control Studies MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/*ethnology MH - Male MH - Middle Aged MH - Prevalence MH - Psychotic Disorders/ethnology/etiology MH - Raynaud Disease/ethnology/etiology MH - Renal Insufficiency/ethnology/etiology MH - Retrospective Studies MH - Sex Factors MH - Thailand/epidemiology MH - Thrombocytopenia/ethnology/etiology EDAT- 2007/09/11 09:00 MHDA- 2008/07/18 09:00 CRDT- 2007/09/11 09:00 PHST- 2007/04/22 00:00 [received] PHST- 2007/07/30 00:00 [accepted] PHST- 2007/09/11 09:00 [pubmed] PHST- 2008/07/18 09:00 [medline] PHST- 2007/09/11 09:00 [entrez] AID - 10.1007/s00296-007-0442-2 [doi] PST - ppublish SO - Rheumatol Int. 2008 Feb;28(4):339-44. doi: 10.1007/s00296-007-0442-2. Epub 2007 Sep 6. PMID- 23219768 OWN - NLM STAT- MEDLINE DCOM- 20140121 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 12 IP - 8 DP - 2013 Jun TI - The role of nail-videocapillaroscopy in early diagnosis of scleroderma. PG - 821-5 LID - S1568-9972(12)00285-6 [pii] LID - 10.1016/j.autrev.2012.11.006 [doi] AB - Raynaud's phenomenon (RP) is a clinical sign of precocious abnormal microcirculation and can be considered a major risk factor for the development of connective tissue disease, especially systemic sclerosis (SSc). Nailfold videocapillaroscopy is the most valuable tool for the early diagnosis of SSc and related disorders. It allows classification of capillary abnormalities. Scoring capillaroscopic alterations, which change significantly during patient follow-up, should be systematically used in order to monitor microangiopathy. The effectiveness of the nailfold videocapillaroscopy in allowing an early diagnosis of SSc and monitoring the progression of the disease, and its predictive value of clinical complications make it a powerful tool for clinical evaluation and research. CI - Copyright © 2012. Published by Elsevier B.V. FAU - Rossi, Daniela AU - Rossi D AD - Department of Rare, Immunologic, Hematologic Diseases and Transfusion Medicine, Research Center of Immunopathology and Rare Diseases (CMID), Giovanni Bosco Hospital and University of Turin, Italy. daniela.rossi@unito.it FAU - Russo, Alessandra AU - Russo A FAU - Manna, Erika AU - Manna E FAU - Binello, Giovanni AU - Binello G FAU - Baldovino, Simone AU - Baldovino S FAU - Sciascia, Savino AU - Sciascia S FAU - Roccatello, Dario AU - Roccatello D LA - eng PT - Journal Article PT - Review DEP - 20121204 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Connective Tissue Diseases/*diagnosis MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/blood supply MH - Raynaud Disease/diagnosis MH - Scleroderma, Systemic/diagnosis/pathology/physiopathology EDAT- 2012/12/12 06:00 MHDA- 2014/01/22 06:00 CRDT- 2012/12/11 06:00 PHST- 2012/12/11 06:00 [entrez] PHST- 2012/12/12 06:00 [pubmed] PHST- 2014/01/22 06:00 [medline] AID - S1568-9972(12)00285-6 [pii] AID - 10.1016/j.autrev.2012.11.006 [doi] PST - ppublish SO - Autoimmun Rev. 2013 Jun;12(8):821-5. doi: 10.1016/j.autrev.2012.11.006. Epub 2012 Dec 4. PMID- 29787366 OWN - NLM STAT- MEDLINE DCOM- 20190129 LR - 20190129 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 Suppl 113 IP - 4 DP - 2018 Jul-Aug TI - Parasympathetic activity increases with digital microvascular damage and vascular endothelial growth factor in systemic sclerosis. PG - 24-27 AB - OBJECTIVES: The imbalance between angiogenic and angiostatic factors with derangement of the microvasculature are hallmarks of systemic sclerosis (SSc). Raynaud's phenomenon in SSc probably is due to the impaired neuroendothelial control mechanisms between vasoconstriction and vasodilatation. The aim of this study is to evaluate autonomic nervous system function using heart rate variability (HRV) analysis and to correlate with vascular endothelial growth factor (VEGF). METHODS: Twenty-seven SSc patients were enrolled. HRV was measured and markers of global sympathetic and parasympathetic system, respectively standard deviation of normal-to-normal RR intervals (SDNN) and square root of the mean of the sum of the squares of differences between adjacent NN intervals (RMSSD) were evaluated. Serum VEGF levels and nailfold videocapillaroscopy (NVC) were performed. RESULTS: A linear positive correlation was observed between RMSSD and VEGF (p<0.01, r=0.55), and RMSSD and disease duration (p< 0.01, r=0.54). The RMSSD median value was significantly increased (p< 0.05) with NVC damage progression. The RMSSD median value was significantly (p<0.05) higher in SSc patients with digital ulcers (DUs) than in SSc patients without DUs [44 (39.4-60.2) vs 24.6 (23-37.1)]. CONCLUSIONS: In our study parasympathetic modulation increases in relation to VEGF. When microcirculation is modified with capillaroscopic pattern progression and DUs, autonomic system seems to stimulate vasodilatation trough parasympathetic system. We can conclude that parasympathetic activity increases with digital microvascular damage and promotes VEGF release. FAU - Gigante, Antonietta AU - Gigante A AD - Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy. antonietta.gigante@uniroma1.it. FAU - Margiotta, Domenico AU - Margiotta D AD - Department of Immuno-Rheumatology Unit, Campus Bio-Medico, University of Rome, Italy. FAU - Navarini, Luca AU - Navarini L AD - Department of Immuno-Rheumatology Unit, Campus Bio-Medico, University of Rome, Italy. FAU - Liberatori, Marta AU - Liberatori M AD - Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy. FAU - Barbano, Biagio AU - Barbano B AD - Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy. FAU - Tubani, Luigi AU - Tubani L AD - Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy. FAU - Afeltra, Antonella AU - Afeltra A AD - Department of Immuno-Rheumatology Unit, Campus Bio-Medico, University of Rome, Italy. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Scleroderma Unit, Sapienza University of Rome, Italy. LA - eng PT - Journal Article DEP - 20180511 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (VEGFA protein, human) RN - 0 (Vascular Endothelial Growth Factor A) SB - IM MH - Adult MH - Disease Progression MH - Female MH - Fingers/*blood supply MH - Heart/*innervation MH - Heart Rate MH - Hemodynamics MH - Humans MH - Male MH - Microvessels/*physiopathology MH - Middle Aged MH - Parasympathetic Nervous System/*physiopathology MH - Raynaud Disease/blood/diagnosis/*etiology/physiopathology MH - Regional Blood Flow MH - Scleroderma, Systemic/blood/*complications/diagnosis/physiopathology MH - Skin Ulcer/blood/diagnosis/*etiology/physiopathology MH - Vascular Endothelial Growth Factor A/*blood EDAT- 2018/05/23 06:00 MHDA- 2019/01/30 06:00 CRDT- 2018/05/23 06:00 PHST- 2017/08/09 00:00 [received] PHST- 2017/11/27 00:00 [accepted] PHST- 2018/05/23 06:00 [pubmed] PHST- 2019/01/30 06:00 [medline] PHST- 2018/05/23 06:00 [entrez] AID - 12157 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jul-Aug;36 Suppl 113(4):24-27. Epub 2018 May 11. PMID- 40478262 OWN - NLM STAT- MEDLINE DCOM- 20250721 LR - 20250721 IS - 1432-1041 (Electronic) IS - 0031-6970 (Linking) VI - 81 IP - 8 DP - 2025 Aug TI - Global burden of vaccine-associated Raynaud's phenomenon, 1968-2024: A comprehensive analysis of the pharmacovigilance database. PG - 1197-1206 LID - 10.1007/s00228-025-03854-2 [doi] AB - PURPOSE: Reports of Raynaud's phenomenon following vaccination have been steadily increasing; however, research on vaccine-associated Raynaud's phenomenon remains limited. This study aims to provide a comprehensive analysis of the association between Raynaud's phenomenon and various vaccines. METHOD: This study used data from international pharmacovigilance, which contains over 35 million adverse event cases from more than 140 countries. Two established pharmacovigilance indicators, the information component (IC) and reporting odds ratio (ROR), were employed in the analysis with 95% confidence interval (CI). The IC was derived using a Bayesian methodology to compare the reporting and non-reporting groups, while the ROR, a frequentist measure of association, was calculated using contingency tables based on the number of adverse events. RESULTS: The signal with Raynaud's phenomenon was highest for papillomavirus vaccines (ROR: 11.49 [95% CI, 9.66-13.67]; IC: 3.45 [IC(0.25), 3.16]), followed in order by typhoid (5.86 [2.93-11.72]), hepatitis B (5.63 [4.25-7.45]; 2.42 [1.95]), COVID-19 mRNA (5.00 [4.70-5.31]; 2.00 [1.91]), and hepatitis A vaccines (4.35 [2.87-6.62]; 2.02 [1.30]). The signal was higher in females (ROR: 3.74 [95% CI, 3.54-3.95]; IC: 1.67 [IC(0.25), 1.59]) compared to males (3.44 [3.12-3.78]; 1.57 [1.43]), and it increased monotonically with age (0-11 years: IC [IC(0.25)] 0.03 [-0.56]; 12-17 years: 1.54 [1.25]; 18-44 years: 1.64 [1.52]; 45-64 years: 2.00 [1.87]; ≥ 65 years: 2.12 [1.91]). CONCLUSION: This study suggests the potential signal association between various vaccines and Raynaud's phenomenon. Although our study does not imply causality, we propose the need to strengthen post-vaccination monitoring and establish support policies to address such adverse events. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Jeong, Jinyoung AU - Jeong J AD - Department of Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. FAU - Kim, Hyunjee AU - Kim H AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Department of Precision Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. FAU - Jo, Hyesu AU - Jo H AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Department of Regulatory Science, Kyung Hee University, Seoul, South Korea. FAU - Park, Jaeyu AU - Park J AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Department of Precision Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. FAU - Cho, Jaehyeong AU - Cho J AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Department of Medicine, CHA University School of Medicine, Seongnam, South Korea. FAU - Lee, Hayeon AU - Lee H AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. AD - Department of Biomedical Engineering, Kyung Hee University, Yongin, South Korea. AD - Department of Electronics and Information Convergence Engineering, Kyung Hee University, Yongin, South Korea. FAU - Cho, Hanseul AU - Cho H AD - Department of Epidemiology, Harvard T.H. Chan School of Public Health, Boston, MA, USA. FAU - Rahmati, Masoud AU - Rahmati M AD - CEReSS-Health Service Research and Quality of Life Center, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France. masoud2_rahmati@yahoo.com. AD - Department of Physical Education and Sport Sciences, Faculty of Literature and Human Sciences, Lorestan University, Khoramabad, Iran. masoud2_rahmati@yahoo.com. AD - Department of Physical Education and Sport Sciences, Faculty of Literature and Humanities, Vali-E-Asr University of Rafsanjan, Rafsanjan, Iran. masoud2_rahmati@yahoo.com. AD - Department of Sport Sciences, Faculty of Literature and Human Sciences, Lorestan University, Khorramabad, A81, FCM5+637, Lorestan Province, Iran. masoud2_rahmati@yahoo.com. FAU - Woo, Ho Geol AU - Woo HG AD - Department of Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. nr85plasma@naver.com. AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. nr85plasma@naver.com. AD - Department of Neurology, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, 23 Kyungheedae-Ro, Dongdaemun-Gu, Seoul, 02447, Republic of Korea. nr85plasma@naver.com. FAU - Yon, Dong Keon AU - Yon DK AD - Department of Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. yonkkang@gmail.com. AD - Center for Digital Health, Medical Science Research Institute, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, Seoul, South Korea. yonkkang@gmail.com. AD - Department of Precision Medicine, Kyung Hee University College of Medicine, Seoul, South Korea. yonkkang@gmail.com. AD - Department of Regulatory Science, Kyung Hee University, Seoul, South Korea. yonkkang@gmail.com. AD - Department of Pediatrics, Kyung Hee University Medical Center, Kyung Hee University College of Medicine, 23 Kyungheedae-Ro, Dongdaemun-Gu, Seoul, 02447, South Korea. yonkkang@gmail.com. LA - eng GR - MSIT; RS-2023-00239251/Ministry of Science and ICT/ GR - IITP-2024-RS-2024-00438239/Institute for Information & Communications Technology Planning & Evaluation/ PT - Journal Article DEP - 20250606 PL - Germany TA - Eur J Clin Pharmacol JT - European journal of clinical pharmacology JID - 1256165 RN - 0 (Vaccines) SB - IM MH - Humans MH - *Pharmacovigilance MH - *Raynaud Disease/epidemiology/chemically induced MH - Male MH - Female MH - Databases, Factual MH - *Vaccines/adverse effects MH - Adult MH - Middle Aged MH - Young Adult MH - Aged MH - Adolescent MH - Child MH - Adverse Drug Reaction Reporting Systems MH - Child, Preschool MH - Infant OTO - NOTNLM OT - Global OT - Raynaud’s phenomenon OT - Vaccines COIS- Declarations. Ethical approval: This study utilized the data with the approval of Kyung Hee University. Furthermore, as this study is based on population-level data, ethical approval and informed consent were not required. Conflict of interest: The authors declare no competing interests. EDAT- 2025/06/06 12:34 MHDA- 2025/07/21 12:40 CRDT- 2025/06/06 11:03 PHST- 2025/02/12 00:00 [received] PHST- 2025/05/21 00:00 [accepted] PHST- 2025/07/21 12:40 [medline] PHST- 2025/06/06 12:34 [pubmed] PHST- 2025/06/06 11:03 [entrez] AID - 10.1007/s00228-025-03854-2 [pii] AID - 10.1007/s00228-025-03854-2 [doi] PST - ppublish SO - Eur J Clin Pharmacol. 2025 Aug;81(8):1197-1206. doi: 10.1007/s00228-025-03854-2. Epub 2025 Jun 6. PMID- 33018282 OWN - NLM STAT- MEDLINE DCOM- 20201019 LR - 20201019 IS - 2694-0604 (Electronic) IS - 2375-7477 (Linking) VI - 2020 DP - 2020 Jul TI - Thermography for the detection of Secondary Raynaud's Phenomenon by means of the Distal-Dorsal Distance. PG - 1528-1531 LID - 10.1109/EMBC44109.2020.9175870 [doi] AB - Raynaud's phenomenon (RP) is a disease characterized by a transient ischemic process, in an exaggerated vascular response to cold or emotional stress. Thermography is a resource applied to support diagnosis of changes in the circulatory system. The aim of the study was to use the DistalDorsal Thermography Difference (DDD) in thermographic images to assess thermal behavior in individuals with secondary RP. The research was carried out in the period between 2018 and 2019. The sample means of the Distal-consisted of 44 individuals in a control group (Control) and 44 individuals in a pathological group (RP2). The participants, after acclimatization, were submitted to the cold stress protocol. The protocol consisted of immersing hands in a container of water at a temperature of 15°C for 60 seconds. The acquisition of thermographic images was performed at the pre-test moment and at the 1st, 3rd, 5th, 7th, 10th and 15th minute. At each time, the DDD values (of all fingers - minimum, maximum and sum) between the groups were analyzed. For statistical analysis, the independent t test and Cohen's d test were used. Regarding the results, there was a difference in relation to the rate of temperature recovery between the groups. The first group showed a rate of reheating just after the first minute subsequent to the cold stress test, while the RP2 group was unable to recover the temperature over 15 minutes. DDD, regardless of the selected criterion, proved to be a valid index for verifying the temperature gradient in the study with individuals identified with secondary RP. FAU - R, Viana J AU - R VJ FAU - D, Campos AU - D C FAU - L, Ulbricht AU - L U FAU - Y, Sato G AU - Y SG FAU - L, Ripka W AU - L RW LA - eng PT - Journal Article PL - United States TA - Annu Int Conf IEEE Eng Med Biol Soc JT - Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference JID - 101763872 SB - IM MH - Cold Temperature MH - Fingers/blood supply MH - Hand MH - Humans MH - Ischemia MH - *Raynaud Disease/diagnosis MH - *Thermography EDAT- 2020/10/07 06:00 MHDA- 2020/10/21 06:00 CRDT- 2020/10/06 01:03 PHST- 2020/10/06 01:03 [entrez] PHST- 2020/10/07 06:00 [pubmed] PHST- 2020/10/21 06:00 [medline] AID - 10.1109/EMBC44109.2020.9175870 [doi] PST - ppublish SO - Annu Int Conf IEEE Eng Med Biol Soc. 2020 Jul;2020:1528-1531. doi: 10.1109/EMBC44109.2020.9175870. PMID- 21134617 OWN - NLM STAT- MEDLINE DCOM- 20110408 LR - 20220330 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 35 IP - 12 DP - 2010 Dec TI - Botulinum toxin type A in the treatment of Raynaud's phenomenon. PG - 2085-92 LID - 10.1016/j.jhsa.2010.09.019 [doi] AB - PURPOSE: Raynaud's phenomenon is a vasospastic disorder of the palmar and digital vessels of the hand and feet that can lead to ischemic ulcers, pain, and loss of function. This study is a review of patients I have injected with botulinum toxin type A for patients with Raynaud's phenomenon. METHODS: Raynaud's patients were injected with 50 to 100 units of onabotulinumtoxinA to improve perfusion of the digits. An institutional review board-approved retrospective review was undertaken to analyze outcomes. Laser Doppler scans were performed before and after injection to quantitatively measure perfusion. RESULTS: A total of 14 men and 19 women with Raynaud's phenomenon were injected with onabotulinumtoxinA. All but 5 patients experienced improved vascularity and relief of pain. Laser Doppler scans illustrated notable improvement in perfusion. Five patients had repeat injections for recurrent pain. CONCLUSIONS: Botulinum toxin appears to improve perfusion of the hand after direct injection around the neurovascular bundles. Further investigations are warranted to identify the exact mode of action in relieving vasospasm and alleviating pain. CI - Copyright © 2010. Published by Elsevier Inc. FAU - Neumeister, Michael W AU - Neumeister MW AD - Division of Plastic Surgery, Southern Illinois University School of Medicine, Springfield, IL 62794, USA. mneumeister@siumed.edu LA - eng PT - Journal Article PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Neuromuscular Agents) RN - 0 (Synaptosomal-Associated Protein 25) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/pharmacology/*therapeutic use MH - Contraindications MH - Female MH - Hand/blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Neuromuscular Agents/pharmacology/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Retreatment MH - Synaptosomal-Associated Protein 25/physiology MH - Treatment Outcome EDAT- 2010/12/08 06:00 MHDA- 2011/04/09 06:00 CRDT- 2010/12/08 06:00 PHST- 2010/03/03 00:00 [received] PHST- 2010/09/16 00:00 [accepted] PHST- 2010/12/08 06:00 [entrez] PHST- 2010/12/08 06:00 [pubmed] PHST- 2011/04/09 06:00 [medline] AID - S0363-5023(10)01118-4 [pii] AID - 10.1016/j.jhsa.2010.09.019 [doi] PST - ppublish SO - J Hand Surg Am. 2010 Dec;35(12):2085-92. doi: 10.1016/j.jhsa.2010.09.019. PMID- 35608095 OWN - NLM STAT- MEDLINE DCOM- 20231114 LR - 20250801 IS - 1759-9873 (Electronic) IS - 0964-5284 (Print) IS - 0964-5284 (Linking) VI - 41 IP - 2 DP - 2023 Apr TI - The use of acupuncture in patients with Raynaud's syndrome: a systematic review and meta-analysis of randomized controlled trials. PG - 63-72 LID - 10.1177/09645284221076504 [doi] AB - OBJECTIVE: To assess the effectiveness of acupuncture for the treatment of Raynaud's syndrome by conducting a systematic review and meta-analysis of randomized controlled trials (RCTs). METHODS: Studies were identified from English and Chinese databases from their inception to September 2020. The outcomes of interest were remission incidence, number of daily attacks, incidence of positive cold stimulation tests and incidence of cold provocation tests. We conducted meta-analysis and network meta-analysis using meta and gemtc. RESULTS: Six trials (n = 272 participants) were included in the meta-analysis. Pairwise meta-analyses show that acupuncture was associated with increased remission incidence (risk ratio (RR) = 1.21, 95% confidence interval (CI) = 1.10 to 1.34), decreased daily number of attacks (weighted mean difference (WMD) = -0.57, 95% CI = -1.14 to -0.01), and increased incidence of positive cold stimulation tests (RR = 1.64, 95% CI = 1.27 to 2.11). There was not enough evidence to associate acupuncture with decreased incidence of positive cold provocation tests. The network meta-analyses did not demonstrate significant results for the effectiveness of any acupuncture treatments (electroacupuncture or manual acupuncture ± moxibustion), compared with controls, in terms of remission incidence or daily number of attacks, possibly due to small sample sizes and a lack of statistical power. CONCLUSION: The use of acupuncture may be effective for the treatment of Raynaud's syndrome in terms of increasing remission incidence, decreasing daily number of attacks and increasing incidences of positive cold stimulation tests. However, our findings should be interpreted with caution due to small sample sizes, very low quality of evidence and high risk of bias. Future large-scale RCTs are warranted. FAU - Zhou, Fangwen AU - Zhou F AD - Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada. FAU - Huang, Emma AU - Huang E AD - Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada. FAU - Zheng, Elena AU - Zheng E AD - Faculty of Applied Health Sciences, University of Waterloo, Waterloo, ON, Canada. FAU - Deng, Jiawen AU - Deng J AUID- ORCID: 0000-0002-8274-6468 AD - Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada. LA - eng PT - Journal Article PT - Network Meta-Analysis PT - Systematic Review DEP - 20220524 PL - England TA - Acupunct Med JT - Acupuncture in medicine : journal of the British Medical Acupuncture Society JID - 9304117 SB - IM MH - Humans MH - *Acupuncture Therapy/methods MH - Randomized Controlled Trials as Topic MH - *Raynaud Disease/therapy MH - Treatment Outcome PMC - PMC10115941 OTO - NOTNLM OT - Raynaud’s phenomenon OT - Raynaud’s syndrome OT - acupuncture OT - meta-analysis OT - network meta-analysis COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2022/05/25 06:00 MHDA- 2023/04/20 06:41 PMCR- 2023/04/20 CRDT- 2022/05/24 06:12 PHST- 2023/04/20 06:41 [medline] PHST- 2022/05/25 06:00 [pubmed] PHST- 2022/05/24 06:12 [entrez] PHST- 2023/04/20 00:00 [pmc-release] AID - 10.1177_09645284221076504 [pii] AID - 10.1177/09645284221076504 [doi] PST - ppublish SO - Acupunct Med. 2023 Apr;41(2):63-72. doi: 10.1177/09645284221076504. Epub 2022 May 24. PMID- 24461384 OWN - NLM STAT- MEDLINE DCOM- 20141002 LR - 20161125 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 48-49 DP - 2014 Feb-Mar TI - Diagnostic criteria of systemic sclerosis. PG - 38-41 LID - S0896-8411(14)00015-8 [pii] LID - 10.1016/j.jaut.2013.11.004 [doi] AB - Systemic sclerosis (SSc) is a multisystem disease characterized by vascular abnormalities, immune system activation manifested by SSc-specific autoantibodies and disturbances in fibroblast function. The clinical manifestations are highly heterogeneous and commonly include skin thickening, Raynaud's phenomenon, digital ulcers, gastroesophageal reflux disease, interstitial lung disease and cardiac diastolic dysfunction. The diagnosis of SSc in a patient with typical end-organ disease is relatively straight-forward, but is unsatisfactory because it implies that the diagnosis is delayed until irreversible tissue damage is present. Diagnostic criteria are generally designed to facilitate the clinical process and to allow early institution of therapy to relieve symptoms and possibly prevent irreversible damage. Several attempts at defining diagnostic criteria for SSc have been made in the past. Raynaud's phenomenon, SSc-specific autoantibodies and nailfold capillary abnormalities are among the most promising items likely to be retained in a final set of diagnostic criteria. The EULAR Scleroderma Trial and Research group (EUSTAR) is currently in the process of prospectively validating a set of diagnostic criteria for the very early diagnosis of SSc and results are expected in 2015. CI - Copyright © 2014 Elsevier Ltd. All rights reserved. FAU - Hudson, Marie AU - Hudson M AD - Department of Medicine, McGill University, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, H3T 1E3 Quebec, Canada; Division of Rheumatology, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, H3T 1E3 Quebec, Canada; Lady Davis Institute, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, Quebec H3T 1E3, Canada. Electronic address: marie.hudson@mcgill.ca. FAU - Fritzler, Marvin J AU - Fritzler MJ AD - Faculty of Medicine, University of Calgary, 3330 Hospital Drive, Calgary, T2N 4N1 Alberta, Canada. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20140122 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (anticentromere antibody) RN - EC 2.7.7.6 (RNA Polymerase III) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 5.99.1.2 (TOP1 protein, human) SB - IM MH - Antibodies, Antinuclear/*biosynthesis/blood MH - Autoantibodies/*biosynthesis/blood MH - DNA Topoisomerases, Type I/biosynthesis/blood/*immunology MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy MH - Prospective Studies MH - RNA Polymerase III/adverse effects/biosynthesis/immunology MH - Randomized Controlled Trials as Topic/*methods MH - Raynaud Disease/*diagnosis/enzymology/*immunology MH - Scleroderma, Systemic/*diagnosis/enzymology/immunology MH - Telangiectasis/diagnosis/enzymology/immunology MH - Validation Studies as Topic OTO - NOTNLM OT - Diagnostic criteria OT - Systemic sclerosis EDAT- 2014/01/28 06:00 MHDA- 2014/10/03 06:00 CRDT- 2014/01/28 06:00 PHST- 2013/10/07 00:00 [received] PHST- 2013/11/13 00:00 [accepted] PHST- 2014/01/28 06:00 [entrez] PHST- 2014/01/28 06:00 [pubmed] PHST- 2014/10/03 06:00 [medline] AID - S0896-8411(14)00015-8 [pii] AID - 10.1016/j.jaut.2013.11.004 [doi] PST - ppublish SO - J Autoimmun. 2014 Feb-Mar;48-49:38-41. doi: 10.1016/j.jaut.2013.11.004. Epub 2014 Jan 22. PMID- 38625644 OWN - NLM STAT- MEDLINE DCOM- 20240522 LR - 20240722 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 43 IP - 6 DP - 2024 Jun TI - Rare combo: moyamoya and lupus in men. PG - 2139-2143 LID - 10.1007/s10067-024-06960-1 [doi] AB - Moyamoya syndrome (MMS) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries. Cerebral angiography images are used for detection through measurement. Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause multisystemic involvement. The coexistence of SLE and MMS has been rarely reported in the literature. A 46-year-old male patient with malar rash, Raynaud phenomenon presented to the hospital with a complaint of weakness in the left lower extremity, which began 3 days before the date of the visit. In the diffusion magnetic resonance imaging, multiple diffusion restrictions were observed in the right frontal region. The patient underwent MR angiography, revealing stenosis in the terminal and supraclinoid segments of the right internal carotid artery, which made us consider moyamoya disease. This patient, with a malar rash and Raynaud's, a positive antibody profile, was diagnosed as a male with SLE accompanied by MMS. CI - © 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Gökten, Dilara Bulut AU - Gökten DB AUID- ORCID: 0000-0002-9226-7532 AD - Department of Rheumatology, Tekirdag Namik Kemal University, Tekirdag, Turkey. dilarabulutgokten@gmail.com. FAU - Gökten, Murat AU - Gökten M AUID- ORCID: 0000-0002-5659-4763 AD - Department of Neurosurgery, Tekirdag City Hospital, Tekirdag, Turkey. FAU - Deniz, Çiğdem AU - Deniz Ç AUID- ORCID: 0000-0003-1325-4328 AD - Department of Neurology, Tekirdag Namik Kemal University, Tekirdag, Turkey. FAU - Mercan, Rıdvan AU - Mercan R AUID- ORCID: 0000-0003-1537-2192 AD - Department of Rheumatology, Tekirdag Namik Kemal University, Tekirdag, Turkey. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20240416 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - Male MH - *Moyamoya Disease/diagnostic imaging/complications MH - Middle Aged MH - *Lupus Erythematosus, Systemic/complications/diagnostic imaging/diagnosis MH - *Magnetic Resonance Angiography MH - Raynaud Disease/complications/diagnosis MH - Cerebral Angiography MH - Carotid Artery, Internal/diagnostic imaging MH - Diffusion Magnetic Resonance Imaging OTO - NOTNLM OT - Lupus OT - Male lupus OT - Moyamoya OT - SLE EDAT- 2024/04/16 12:42 MHDA- 2024/05/23 00:42 CRDT- 2024/04/16 11:17 PHST- 2024/02/22 00:00 [received] PHST- 2024/04/02 00:00 [accepted] PHST- 2024/03/30 00:00 [revised] PHST- 2024/05/23 00:42 [medline] PHST- 2024/04/16 12:42 [pubmed] PHST- 2024/04/16 11:17 [entrez] AID - 10.1007/s10067-024-06960-1 [pii] AID - 10.1007/s10067-024-06960-1 [doi] PST - ppublish SO - Clin Rheumatol. 2024 Jun;43(6):2139-2143. doi: 10.1007/s10067-024-06960-1. Epub 2024 Apr 16. PMID- 41609954 OWN - NLM STAT- MEDLINE DCOM- 20260607 LR - 20260607 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 45 IP - 3 DP - 2026 Mar TI - An epidemiological study estimating the burden of cancer risk in patients with Raynaud's phenomenon. PG - 1651-1656 LID - 10.1007/s10067-026-07961-y [doi] AB - INTRODUCTION/OBJECTIVE: Raynaud's phenomenon (RP) is a common vasospastic condition that may develop secondary to cancer and/or in association with systemic autoimmune rheumatic diseases (SARDs). We aimed to estimate the risk of cancer in RP without known SARDs, phenotypically akin to 'primary' RP. METHODS: A cohort study using data from North American electronic healthcare organization records. RP was defined using ≥ 2 ICD (I73.0) codes, excluding SARDs. Comparators had ≥ 2 irritable bowel syndrome ICD (K58) codes: selected with similar epidemiology to RP, and without any known excess in cancer risk. Cohorts were stratified by age (< 45 and ≥ 45 years). Our primary outcome was any cancer event. Secondary outcomes were rates of specific cancers: head and neck, digestive, thorax, skin, breast, haematological, male/female genital. Risk of each outcome was compared using 1:1 propensity score-matched Cox proportional hazard models. RESULTS: Among 34,582 (< 45 years) and 68,836 (≥ 45 years) matched pairs, the hazard ratio (HR) of any cancer was higher in RP: < 45 [1.11 (1.03, 1.19)] and ≥ 45 [1.08 (1.05, 1.11)]. Cancer-specific risks were calculated. RP was associated in both age groups with increased risk of thorax (HR 2.077 & 1.433), skin (HR 1.202 &1.213), and haematological (HR 1.647 & 1.338) cancers. RP was associated with decreased risk of digestive cancer (HR 0.686 & 0.894). CONCLUSION: RP was associated with an increased risk of cancer, independent of age. We also describe varying cancer-specific risks. Future research is warranted to confirm and explore these novel observations, including potentially shared pathobiological mechanisms. Key Points • RP was associated with an overall increased risk of all cancers in younger (<45years) and older (≥45 years) individuals. • There was an increased risk of certain (e.g., thorax, skin, and haematological) cancers with RP. • A reduced risk of cancer was also noted for certain cancers, especially GI-related. • Future research should confirm and explore these observations, including the pathobiology underpinning potential cancer risk in RP. CI - © 2026. The Author(s). FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biological Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Stopford Building, Oxford Road, Manchester, UK. Michael.hughes-6@manchester.ac.uk. AD - Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK. Michael.hughes-6@manchester.ac.uk. AD - NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. Michael.hughes-6@manchester.ac.uk. FAU - Jude, Edward AU - Jude E AUID- ORCID: 0000-0002-3186-4122 AD - Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton under Lyne, UK. AD - University of Manchester, Manchester, UK. AD - Manchester Metropolitan University, Manchester, UK. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - Department of Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Alam, Uazman AU - Alam U AUID- ORCID: 0000-0002-3190-1122 AD - Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK. AD - Department of Medicine, University Hospital Aintree, Liverpool University NHS Foundation Trust, Liverpool, UK. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, Pulmonology Unit, Trieste, Italy. FAU - Zhao, Sizheng Steven AU - Zhao SS AUID- ORCID: 0000-0002-3558-7353 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biological Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Stopford Building, Oxford Road, Manchester, UK. AD - NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. LA - eng PT - Journal Article DEP - 20260129 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - *Raynaud Disease/epidemiology/complications MH - Middle Aged MH - Female MH - *Neoplasms/epidemiology MH - Male MH - Adult MH - Risk Factors MH - Aged MH - Proportional Hazards Models MH - Cohort Studies PMC - PMC12923405 OTO - NOTNLM OT - Autoimmunity OT - Cancer OT - Malignancy OT - Raynaud’s phenomenon OT - Rheumatology OT - Systemic sclerosis COIS- Declarations. Competing interests: MH reports Research Funding and Speaker Fees from Janssen; Conference Support from UCB; and Consultancy Fees from Boehringer Ingelheim and Novartis (none of which are relevant to this manuscript). UA has no direct COI. However, UA declares that he has received honoraria from Viatris, Grünenthal, Eli Lilly, Procter & Gamble, and for educational meetings and has received investigator-led funding from Procter & Gamble. UA has received sponsorship to educational meetings from Daiichi Sankyo and Sanofi. None of the other authors report any relevant disclosures to the manuscript. EDAT- 2026/01/29 19:11 MHDA- 2026/02/20 23:42 PMCR- 2026/01/29 CRDT- 2026/01/29 12:44 PHST- 2025/12/23 00:00 [received] PHST- 2026/01/19 00:00 [accepted] PHST- 2026/01/17 00:00 [revised] PHST- 2026/02/20 23:42 [medline] PHST- 2026/01/29 19:11 [pubmed] PHST- 2026/01/29 12:44 [entrez] PHST- 2026/01/29 00:00 [pmc-release] AID - 10.1007/s10067-026-07961-y [pii] AID - 7961 [pii] AID - 10.1007/s10067-026-07961-y [doi] PST - ppublish SO - Clin Rheumatol. 2026 Mar;45(3):1651-1656. doi: 10.1007/s10067-026-07961-y. Epub 2026 Jan 29. PMID- 19363679 OWN - NLM STAT- MEDLINE DCOM- 20090924 LR - 20220318 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 9 DP - 2009 Jul TI - Ulnar artery vasculopathy in systemic sclerosis. PG - 1081-6 LID - 10.1007/s00296-009-0906-7 [doi] AB - Although digital ulceration frequently occurs in patients with systemic sclerosis, there have been few reports on macrovascular involvement. Macrovascular disease in systemic sclerosis has recently been described. We retrospectively reviewed the medical records and brachial angiographic findings of 19 systemic sclerosis patients, who exhibited Raynaud's phenomenon and digital ulceration. We found that ulnar artery involvement is frequent in systemic sclerosis, although the precise mechanism is not known. There was no significant difference in risk factors of macrovascular disease between ulnar artery-involved patients and not-involved subjects. Thirteen patients underwent surgical intervention; five of the 13 patients had vascular graft performed due to ulnar artery involvement. We suggest that angiographic screening and early surgical intervention such as revascularization should be considered in patients with systemic sclerosis who manifest a severe form of Raynaud's phenomenon and/or digital ulceration and especially in patients with diffuse sclerosis. FAU - Park, Jeong Ha AU - Park JH AD - Department of Rheumatology, The Hospital for Rheumatic Diseases, Hanyang University, Seoul, Republic of Korea. FAU - Sung, Yoon-Kyoung AU - Sung YK FAU - Bae, Sang-Cheol AU - Bae SC FAU - Song, Soon-Young AU - Song SY FAU - Seo, Heong Seok AU - Seo HS FAU - Jun, Jae-Bum AU - Jun JB LA - eng PT - Journal Article PT - Review DEP - 20090412 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Angiography/adverse effects MH - Brachial Artery/surgery MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*etiology MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*complications MH - Ulnar Artery/*pathology MH - Vascular Surgical Procedures/adverse effects RF - 13 EDAT- 2009/04/14 09:00 MHDA- 2009/09/25 06:00 CRDT- 2009/04/14 09:00 PHST- 2008/07/02 00:00 [received] PHST- 2009/03/25 00:00 [accepted] PHST- 2009/04/14 09:00 [entrez] PHST- 2009/04/14 09:00 [pubmed] PHST- 2009/09/25 06:00 [medline] AID - 10.1007/s00296-009-0906-7 [doi] PST - ppublish SO - Rheumatol Int. 2009 Jul;29(9):1081-6. doi: 10.1007/s00296-009-0906-7. Epub 2009 Apr 12. PMID- 17016765 OWN - NLM STAT- MEDLINE DCOM- 20061212 LR - 20091119 IS - 1090-0586 (Print) IS - 1090-0586 (Linking) VI - 31 IP - 3 DP - 2006 Sep TI - Thermal biofeedback for primary Raynaud's phenomenon: a review of the literature. PG - 203-16 AB - The clinical presentation of primary Raynaud's phenomenon (RP) derives from various pathogenic triggers. The use of thermal biofeedback (TBF) may be of benefit in reducing the severity and frequency of attacks. This article summarizes the relevant research regarding the pathophysiology of primary RP and mechanism of TBF for RP. Systematic reviews of the efficacy of TBF for RP and treatment guidelines for clinicians are provided. The panel concludes that the level of evidence for TBF efficacy is categorized as Level IV: efficacious. The rationale, based on three randomized controlled trials conducted in independent laboratories, demonstrated "superiority or equivalence" of treatments that include TBF. However, randomly controlled trials (RCT) with positive clinical outcomes tended to be small. A large RCT with negative results did not effectively teach handwarming skills. Procedures for reviewing and rating of the levels of evidence of efficacy of studies was based on the Template for Developing Guidelines for the Evaluation of the Clinical Efficacy of Psychophysiological Interventions developed by the joint task force of the AAPB and the Society for Neuronal Regulation (SNR). FAU - Karavidas, Maria Katsamanis AU - Karavidas MK AD - University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, Piscataway, New Jersey, USA. karavima@umdnj.edu FAU - Tsai, Pei-Shan AU - Tsai PS FAU - Yucha, Carolyn AU - Yucha C FAU - McGrady, Angele AU - McGrady A FAU - Lehrer, Paul M AU - Lehrer PM LA - eng PT - Journal Article PT - Review PL - Germany TA - Appl Psychophysiol Biofeedback JT - Applied psychophysiology and biofeedback JID - 9712383 SB - IM MH - *Biofeedback, Psychology MH - Clinical Trials as Topic MH - Guidelines as Topic MH - Humans MH - Raynaud Disease/*therapy RF - 34 EDAT- 2006/10/04 09:00 MHDA- 2006/12/13 09:00 CRDT- 2006/10/04 09:00 PHST- 2006/10/04 09:00 [pubmed] PHST- 2006/12/13 09:00 [medline] PHST- 2006/10/04 09:00 [entrez] AID - 10.1007/s10484-006-9018-2 [doi] PST - ppublish SO - Appl Psychophysiol Biofeedback. 2006 Sep;31(3):203-16. doi: 10.1007/s10484-006-9018-2. PMID- 16639488 OWN - NLM STAT- MEDLINE DCOM- 20060731 LR - 20061115 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 58 IP - 1 DP - 2006 Jan-Mar TI - [Migraine in SLE: role of antiphospholipid antibodies and Raynaud's phenomenon]. PG - 50-8 AB - OBJECTIVES: To determine the role of antiphospholipid antibodies (aPL) and of Raynaud's phenomenon (RP) in the development of migraine in patients with systemic lupus erythematosus (SLE). METHODS: 50 unselected SLE patients and 20 rheumatoid arthritis (RA) controls underwent an interview to define the presence of migraine according to the guidelines of the International Headache Society (1988). Serological tests for aPL were performed in all patients. SLE patients were divided according to positivity for RP and/or aPL into 4 subsets: R-/aPL-, R-/aPL+, R+/aPL- and R+/aPL+. Data were analysed using Fisher's exact test, Chi-square test and U Mann-Whitney test. RESULTS: SLE and RA patients were similar for demographic and clinical features; aPL positivity was found in a greater proportion of SLE patients versus RA controls (68% vs 25%, p=0.0036). 31 of the 50 lupic patients (62%) and 7 of the 20 RA controls (35%) suffered from migraine (OR=3, CI:1-8.9). Among SLE and RA patients, migraine was associated with aPL positivity (p=0.027 and p=0.019). Analysing the combined effect of aPL and RP on migraine, in R+/aPL+ patients we detected an higher frequency of migraine (85.7%) with respect to the patients negative for these two features (27%, p=0.0051, OR=16, CI:2.2-118) and to the patients positive only for aPL (65%, p=0.0031, OR=6.2, CI:1.2-32). CONCLUSIONS: Migraine in SLE and RA associates with aPL positivity. The simultaneous presence of RP increases by 2,5 times the probability of having migraine, suggesting that cerebral vasospasm might be more common in patients with peripheral vasospasm, given the presence of aPL. FAU - Annese, Virginia AU - Annese V AD - Cattedra di Reumatologia, Università Cattolica di Roma FAU - Tomietto, Paola AU - Tomietto P FAU - Venturini, Paolo AU - Venturini P FAU - D'Agostini, Serena AU - D'Agostini S FAU - Ferraccioli, Gianfranco AU - Ferraccioli G LA - ita PT - Comparative Study PT - English Abstract PT - Journal Article TT - Emicrania nel LES: ruolo del fenomeno di Raynaud e degli anticorpi antifospolipidi. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/blood/*immunology MH - Antiphospholipid Syndrome/blood/complications/immunology MH - Arthritis, Rheumatoid/blood/complications/immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/blood/*complications/immunology MH - Male MH - Middle Aged MH - Migraine Disorders/*etiology/immunology/physiopathology MH - Pain Measurement MH - Raynaud Disease/*etiology/immunology MH - Socioeconomic Factors MH - Vasospasm, Intracranial/*etiology/immunology/physiopathology EDAT- 2006/04/28 09:00 MHDA- 2006/08/01 09:00 CRDT- 2006/04/28 09:00 PHST- 2006/04/28 09:00 [pubmed] PHST- 2006/08/01 09:00 [medline] PHST- 2006/04/28 09:00 [entrez] PST - ppublish SO - Reumatismo. 2006 Jan-Mar;58(1):50-8. PMID- 39178618 OWN - NLM STAT- MEDLINE DCOM- 20240921 LR - 20250919 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 151 IP - 3 DP - 2024 Sep TI - Nailfold capillaroscopy in acrocyanosis among patients with associated Raynaud's phenomenon. PG - 103309 LID - S0151-9638(24)00065-6 [pii] LID - 10.1016/j.annder.2024.103309 [doi] FAU - Guelimi, R AU - Guelimi R AD - Department of Dermatology, Vascular Medicine and Allergology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, 4 rue de la Chine, F-75020 Paris, France. Electronic address: robin.guelimi@gmail.com. FAU - Monfort, J-B AU - Monfort JB AD - Department of Dermatology, Vascular Medicine and Allergology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, 4 rue de la Chine, F-75020 Paris, France. FAU - Chaby, G AU - Chaby G AD - Dermatology of Department, Amiens-Picardie University Hospital Center, F-80000 Amiens, France. FAU - Lok, C AU - Lok C AD - Dermatology of Department, Amiens-Picardie University Hospital Center, F-80000 Amiens, France. FAU - Lazareth, I AU - Lazareth I AD - Department of Vascular Medicine, Institut de la cicatrisation Jean-Paul-Belmondo, Paris Saint-Joseph Hospital, F-75014 Paris, France. FAU - Maillard, H AU - Maillard H AD - Department of Dermatology, Le Mans Hospital Centre, F-72037 Le Mans, France. FAU - Beneton, N AU - Beneton N AD - Department of Dermatology, Le Mans Hospital Centre, F-72037 Le Mans, France. FAU - Kottler, D AU - Kottler D AD - Department of Dermatology, Caen Hospital Centre, F-14033 Caen, France. FAU - Blaise, S AU - Blaise S AD - Department of Vascular Medicine, Grenoble Alpes University Hospital, F-38000 Grenoble, France. FAU - Imbert, B AU - Imbert B AD - Department of Vascular Medicine, Grenoble Alpes University Hospital, F-38000 Grenoble, France. FAU - Journet, J AU - Journet J AD - Department of Dermatology, William Morey Hospital, F-71100 Chalon-sur-Saône, France. FAU - Goujon, E AU - Goujon E AD - Department of Dermatology, William Morey Hospital, F-71100 Chalon-sur-Saône, France. FAU - Jacquin, A AU - Jacquin A AD - Department of Dermatology and Vascular Medicine, Victor-Dupouy Hospital, F-95100 Argenteuil, France. FAU - Tella, E AU - Tella E AD - Department of Dermatology and Vascular Medicine, Victor-Dupouy Hospital, F-95100 Argenteuil, France. FAU - Vicaut, E AU - Vicaut E AD - Clinical Research Unit, Fernand Vidal Hospital F-75010 Paris, France. FAU - Klejtman, T AU - Klejtman T AD - Department of Vascular Medicine, Institut de la cicatrisation Jean-Paul-Belmondo, Paris Saint-Joseph Hospital, F-75014 Paris, France. FAU - Senet, P AU - Senet P AD - Department of Dermatology, Vascular Medicine and Allergology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, 4 rue de la Chine, F-75020 Paris, France. CN - Angiodermatology Group of the French Society of Dermatology LA - eng PT - Journal Article DEP - 20240822 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis MH - *Microscopic Angioscopy MH - Female MH - *Cyanosis/etiology MH - *Nails/blood supply/pathology MH - Male MH - Middle Aged MH - Adult EDAT- 2024/08/24 16:43 MHDA- 2024/09/22 04:20 CRDT- 2024/08/23 18:05 PHST- 2023/10/26 00:00 [received] PHST- 2024/05/03 00:00 [revised] PHST- 2024/07/11 00:00 [accepted] PHST- 2024/09/22 04:20 [medline] PHST- 2024/08/24 16:43 [pubmed] PHST- 2024/08/23 18:05 [entrez] AID - S0151-9638(24)00065-6 [pii] AID - 10.1016/j.annder.2024.103309 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2024 Sep;151(3):103309. doi: 10.1016/j.annder.2024.103309. Epub 2024 Aug 22. PMID- 34833493 OWN - NLM STAT- MEDLINE DCOM- 20211130 LR - 20211130 IS - 1648-9144 (Electronic) IS - 1010-660X (Print) IS - 1010-660X (Linking) VI - 57 IP - 11 DP - 2021 Nov 20 TI - Clinical and Histopathological Features of Scleroderma-like Disorders: An Update. LID - 10.3390/medicina57111275 [doi] LID - 1275 AB - Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical-pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud's phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis with systemic sclerosis. When scleroderma can be excluded, on the basis of the main body sites, clinical evolution, any associated pathological conditions and specific histological features, it is possible to make a correct diagnosis. FAU - Foti, Rosario AU - Foti R AD - Division of Reumathology, A.O.U. "Policlinico-San Marco", 95123 Catania, Italy. FAU - De Pasquale, Rocco AU - De Pasquale R AD - U.O. Dermatologia, Ospedale San Marco, 95123 Catania, Italy. FAU - Dal Bosco, Ylenia AU - Dal Bosco Y AD - Division of Reumathology, A.O.U. "Policlinico-San Marco", 95123 Catania, Italy. FAU - Visalli, Elisa AU - Visalli E AD - Division of Reumathology, A.O.U. "Policlinico-San Marco", 95123 Catania, Italy. FAU - Amato, Giorgio AU - Amato G AD - Division of Reumathology, A.O.U. "Policlinico-San Marco", 95123 Catania, Italy. FAU - Gangemi, Pietro AU - Gangemi P AD - U.O. Anatomia Patologica, Ospedale San Marco, 95123 Catania, Italy. FAU - Foti, Riccardo AU - Foti R AD - U.O. Dermatologia, Ospedale San Marco, 95123 Catania, Italy. FAU - Ramondetta, Alice AU - Ramondetta A AD - U.O. Dermatologia, Ospedale San Marco, 95123 Catania, Italy. LA - eng PT - Journal Article PT - Review DEP - 20211120 PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear MH - Humans MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Localized/diagnosis MH - *Scleroderma, Systemic/diagnosis MH - Skin PMC - PMC8625286 OTO - NOTNLM OT - cutaneous sclerosis OT - scleredema OT - scleroderma-like OT - sclerodermiform mucinosis COIS- The authors declare no conflict of interest. EDAT- 2021/11/28 06:00 MHDA- 2021/12/01 06:00 PMCR- 2021/11/20 CRDT- 2021/11/27 01:21 PHST- 2021/10/14 00:00 [received] PHST- 2021/11/10 00:00 [revised] PHST- 2021/11/18 00:00 [accepted] PHST- 2021/11/27 01:21 [entrez] PHST- 2021/11/28 06:00 [pubmed] PHST- 2021/12/01 06:00 [medline] PHST- 2021/11/20 00:00 [pmc-release] AID - medicina57111275 [pii] AID - medicina-57-01275 [pii] AID - 10.3390/medicina57111275 [doi] PST - epublish SO - Medicina (Kaunas). 2021 Nov 20;57(11):1275. doi: 10.3390/medicina57111275. PMID- 18560461 OWN - NLM STAT- MEDLINE DCOM- 20100601 LR - 20171116 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 40 IP - 3 DP - 2008 Jun 18 TI - [Experience in treatment of Raynaud's syndrome by chemical sympathectomy: 97 cases]. PG - 310-3 AB - OBJECTIVE: To summarize the curative effect in treatment of Raynaud's syndrome with chemical thoracic sympathectomy (CTS) guided by X-ray since 2001. METHODS: From March 2001 to August 2007, 97 patients with Raynaud's syndrome (186 limbs) were treated by CTS. Guided by X-ray, a needle was punctured through the back skin to the second thoracic sympathetic ganglion beside the thoracic vertebrae and 2 mL of 5% (v/v) phenol was injected. RESULTS: The first CTS treatment produced a good effect on 146 limbs with an effective rate of 78.5% (146/186). The same treatment was performed on the limbs with no effect 1-2 days after the first treatment and produced good effect on 13 limbs with an effective rate of 32.5% (13/40). The total effective rate of the patients who were hospitalized for the first time was 85.5% (159/186). Of the 97 patients, 78 patients (80.4%) were followed up for 47 months on an average with an effective rate of 69.5%. And the rate of complications was 11.4% for pheumothorax, and 3.9% for hydrothorax. Hyperalgesia on axillary region and anterior chest wall; Horner syndrome and sinus bradycardia were rare. CONCLUSION: CTS is minimally invasive and effective in treatment of Raynaud's syndrome. FAU - Han, Jin Tao AU - Han JT AD - Department of Vascular Surgery, Peking University Third Hospital, Beijing, China. bmucystal@163.com FAU - Zhao, Jun AU - Zhao J FAU - Peng, Yong Guang AU - Peng YG LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 339NCG44TV (Phenol) SB - IM MH - Adult MH - Aged MH - Female MH - Follow-Up Studies MH - Humans MH - Hydrothorax/etiology MH - Male MH - Middle Aged MH - Phenol/administration & dosage MH - Pneumothorax/etiology MH - Raynaud Disease/*therapy MH - *Sympathectomy, Chemical/adverse effects EDAT- 2008/06/19 09:00 MHDA- 2010/06/02 06:00 CRDT- 2008/06/19 09:00 PHST- 2008/06/19 09:00 [pubmed] PHST- 2010/06/02 06:00 [medline] PHST- 2008/06/19 09:00 [entrez] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2008 Jun 18;40(3):310-3. PMID- 29668199 OWN - NLM STAT- MEDLINE DCOM- 20180516 LR - 20180516 IS - 0033-2240 (Print) IS - 0033-2240 (Linking) VI - 73 IP - 6 DP - 2016 TI - [Assessment of ECG and holter parameters among patients with systemic sclerosis]. PG - 364-7 AB - INTRODUCTION: The aim of our study was to assess electrocardiographic abnormalities on the standard ECG and Holter monitoring in patients with systemic sclerosis. MATERIALS AND METHODS: 70 patients with systemic sclerosis and 20 healthy individuals were included in the study. Mean disease duration was 100,8 ± 95,79 months. The following electrocardiographic parameters were assessed: driving rhythm, P wave, PQ interval, atrio-ventricular and intraventricular conduction disturbances, ST-T complex abnormalities, mean heart rate, presence of conduction disturbances, supraventricular and ventricular arrhythmias. We evaluated if there could be any relationship between the Raynaud’s phenomenon or disease duration and electrocardiographic abnormalities. RESULTS: We found that patients with systemic sclerosis had higher burden of supraventricular and ventricular arrhythmias than individuals in the control group. The longer duration of the Raynaud’s phenomenon or systemic sclerosis the higher incidence of these arrhythmias could be found. Patients with supraventricular arrhythmias were more often in NYHA class I/II heart failure whilst patients with ventricular arrhythmias were in NYHA class II. CONCLUSIONS: Patients with the longstanding Raynaud’s phenomenon and systemic sclerosis are at a high risk of developing cardiac arrhythmias. It seems that a vigilant electrocardiographic monitoring could increase patients safety. LA - pol PT - Journal Article PL - Poland TA - Przegl Lek JT - Przeglad lekarski JID - 19840720R SB - IM MH - Arrhythmias, Cardiac/diagnosis/*epidemiology/etiology MH - Electrocardiography, Ambulatory MH - Female MH - Humans MH - Male MH - Prevalence MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications EDAT- 2016/01/01 00:00 MHDA- 2016/01/01 00:01 CRDT- 2018/04/19 06:00 PHST- 2018/04/19 06:00 [entrez] PHST- 2016/01/01 00:00 [pubmed] PHST- 2016/01/01 00:01 [medline] PST - ppublish SO - Przegl Lek. 2016;73(6):364-7. PMID- 31507123 OWN - NLM STAT- MEDLINE DCOM- 20190917 LR - 20190917 IS - 1565-1088 (Print) VI - 21 IP - 7 DP - 2019 Jul TI - The Vessels Contribute to Fibrosis in Systemic Sclerosis. PG - 471-474 AB - Microvascular damage, clinically expressed by Raynaud's phenomenon, is generally the first symptom of the disease and the injured vascular cells, both endothelial and perivascular, may transdifferentiate to myofibroblasts, thus leading to collagen deposition in the tissue and consequent fibrosis. Systemic sclerosis (SSc, scleroderma) is complex disease characterized by autoimmunity, vasculopathy, and fibrosis. It has been shown that microvascular damage may be the first symptom of SSc. Injured endothelial cells and pericytes may transdifferentiate into myofibroblasts, the cells responsible for fibrosis and collagen deposition in the tissue. Based on these factors, the process of myofibroblast generation may link two pivotal events of SSc: microvascular damage and fibrosis. Understanding the development, differentiation, and function of myofibroblasts is therefore crucial to individuate early pathogenetic events and develop new therapeutic target for SSc, a condition in which no disease-modifying agents are available. The aim of this review was to discuss the possible origins of myofibroblasts in SSc, highlighting the process of endothelial mesenchymal transition and pericytes to myofibroblast transition and to show how these events may contribute to pathogenesis of the disease. FAU - Di Benedetto, Paola AU - Di Benedetto P AD - Unit of Clinical Pathology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Unit of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Unit of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Cipriani, Paola AU - Cipriani P AD - Unit of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Unit of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy. LA - eng PT - Journal Article PT - Review PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 SB - IM MH - Cell Differentiation/physiology MH - Endothelial Cells/cytology MH - Epithelial-Mesenchymal Transition/physiology MH - Fibrosis/pathology MH - Humans MH - Myofibroblasts/*cytology MH - Pericytes/cytology MH - Raynaud Disease/*physiopathology MH - Scleroderma, Systemic/*physiopathology EDAT- 2019/09/12 06:00 MHDA- 2019/09/19 06:00 CRDT- 2019/09/12 06:00 PHST- 2019/09/12 06:00 [entrez] PHST- 2019/09/12 06:00 [pubmed] PHST- 2019/09/19 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2019 Jul;21(7):471-474. PMID- 19524178 OWN - NLM STAT- MEDLINE DCOM- 20090901 LR - 20161125 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 20 IP - 4 DP - 2009 Jul TI - Systolic and diastolic function in patients with systemic sclerosis. PG - 378-82 LID - 10.1016/j.ejim.2008.10.011 [doi] AB - BACKGROUND: Clinical and epidemiological findings indicate that symptomatic heart disease in patients with systemic sclerosis (SSc) predicts poor prognosis, but cardiac involvement may occur years before clinical manifestation. The aim of this study was to evaluate the cardiac function in patients with SSc and to correlate the echocardiographic parameters with others that quantify the diseases' severity. METHODS: Twenty consecutive patients with SSc were investigated with transthoracic echocardiography (TTE). Two dimensional, pulsed Doppler and pulsed tissue Doppler imaging (TDI) techniques were used, in all the patients, to assess the systolic and diastolic function for left ventricle (LV). Correlations were made between echocardiographic measurements and some clinical and serological features of the patients. RESULTS: None of the patients had any clinical signs of cardiac involvement, nor ECG or TTE systolic function impairment; there are significant differences between systemic sclerosis patients and control group for peak A velocity (0.75+/-0.22 vs 0.57+/-0.32, P=0.05), E/A ratio (1.14+/-0.22 vs 1.48+/-0.26, P=0.01), E/Ea ratio (8.25+/-1.57 vs 7+/-2.2, P=0.05), which account for filling impairment of LV. There are also significant correlations between some other parameters, like the mean duration of Raynaud's phenomenon and E/Ea ratio (r=0.48, P<0.05). CONCLUSIONS: The analysis of SSc heart disease, mainly at a preclinical level, is important in all the cases as an asymptomatic patient may have diastolic dysfunction which can be treated and should be closely observed. FAU - Poanta, Laura AU - Poanta L AD - University of Medicine and Pharmacy Iuliu Hatieganu, 2nd Medical Department, Cluj Napoca, Romania. laurapoanta@yahoo.com FAU - Dadu, Razvan AU - Dadu R FAU - Tiboc, Cristina AU - Tiboc C FAU - Rednic, Simona AU - Rednic S FAU - Dumitrascu, Dan AU - Dumitrascu D LA - eng PT - Journal Article DEP - 20081129 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 SB - IM MH - Adult MH - Diastole MH - Echocardiography MH - Echocardiography, Doppler MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/diagnostic imaging/etiology/physiopathology MH - Scleroderma, Systemic/*complications/*physiopathology MH - Systole MH - *Ventricular Dysfunction, Left/diagnostic imaging/etiology/physiopathology MH - *Ventricular Function, Left EDAT- 2009/06/16 09:00 MHDA- 2009/09/02 06:00 CRDT- 2009/06/16 09:00 PHST- 2008/06/12 00:00 [received] PHST- 2008/09/22 00:00 [revised] PHST- 2008/10/24 00:00 [accepted] PHST- 2009/06/16 09:00 [entrez] PHST- 2009/06/16 09:00 [pubmed] PHST- 2009/09/02 06:00 [medline] AID - S0953-6205(08)00297-5 [pii] AID - 10.1016/j.ejim.2008.10.011 [doi] PST - ppublish SO - Eur J Intern Med. 2009 Jul;20(4):378-82. doi: 10.1016/j.ejim.2008.10.011. Epub 2008 Nov 29. PMID- 30047430 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 36 IP - 4 DP - 2018 Jul-Aug TI - Systemic sclerosis: Current concepts of skin and systemic manifestations. PG - 459-474 LID - S0738-081X(18)30063-4 [pii] LID - 10.1016/j.clindermatol.2018.04.004 [doi] AB - Systemic sclerosis is an uncommon autoimmune connective tissue disease with multiorgan system involvement and significant associated morbidity and mortality. Cutaneous signs and clinical manifestations are of particular importance, as they may be recognized before systemic manifestations, allowing earlier risk stratification into the limited and diffuse cutaneous subtypes, as well as earlier initiation of treatment. Important cutaneous manifestations include Raynaud's phenomenon, digital ulcers, cutaneous sclerosis, calcinosis cutis, telangiectasias, pruritus, and dyspigmentation. Despite investigation of a wide variety of treatments, no FDA-approved pharmacologic therapies exist for systemic sclerosis, and data from high-quality studies are limited. In the following review, we will discuss skin-directed therapies. Although there is evidence to support specific treatments for Raynaud's phenomenon, digital ulcers, and cutaneous sclerosis, there are limited rigorous studies evaluating the treatment of other cutaneous signs and clinical manifestations. Additional randomized-controlled trials and large observational studies are necessary to develop future evidence-based treatment options. CI - Published by Elsevier Inc. FAU - Pearson, David R AU - Pearson DR AD - Corporal Michael J. Crescenz VAMC, Philadelphia, Pennsylvania, USA; Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. Electronic address: david.pearson@uphs.upenn.edu. FAU - Werth, Victoria P AU - Werth VP AD - Corporal Michael J. Crescenz VAMC, Philadelphia, Pennsylvania, USA; Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. FAU - Pappas-Taffer, Lisa AU - Pappas-Taffer L AD - Corporal Michael J. Crescenz VAMC, Philadelphia, Pennsylvania, USA; Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. LA - eng PT - Journal Article PT - Review DEP - 20180412 PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Calcinosis/etiology/therapy MH - Fingers MH - Humans MH - Pigmentation Disorders/drug therapy/etiology MH - Pruritus/drug therapy/etiology MH - Raynaud Disease/etiology/therapy MH - Scleroderma, Localized/etiology/*therapy MH - Scleroderma, Systemic/classification/*complications/*therapy MH - Skin Ulcer/etiology/therapy MH - Telangiectasis/etiology/therapy MH - Terminology as Topic EDAT- 2018/07/27 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/07/27 06:00 PHST- 2018/07/27 06:00 [entrez] PHST- 2018/07/27 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] AID - S0738-081X(18)30063-4 [pii] AID - 10.1016/j.clindermatol.2018.04.004 [doi] PST - ppublish SO - Clin Dermatol. 2018 Jul-Aug;36(4):459-474. doi: 10.1016/j.clindermatol.2018.04.004. Epub 2018 Apr 12. PMID- 20568410 OWN - NLM STAT- MEDLINE DCOM- 20100806 LR - 20100623 IS - 1426-9686 (Print) IS - 1426-9686 (Linking) VI - 28 IP - 167 DP - 2010 May TI - [Contemporaneous clinical picture of systemic sclerosis]. PG - 416-20 AB - Systemic sclerosis is a multi-system disease characterized by skin fibrosis and visceral involvement. The course of disease is unpredictable, sometimes stable for years sometimes rapidly progressive, leading to death during months. Prognosis is limited by internal organ involvement. Nowadays leading cause of death is interstitial lung fibrosis and pulmonary arterial hypertension. In this paper we present two subsets of systemic sclerosis: limited and diffuse and newly proposed classification for early systemic sclerosis, including objective documentation of Raynaud's phenomenon and systemic sclerosis-type naifold capillary pattern or presence of selective auto-antibodies (anti-centromer or anti-Scl 70). We describe clinical symptoms, internal organ involvement and tools to its detection, with use of HRCT, Doppler echocardiography, spirometric gas transfer (DLCO). We present current approach to staging the disease according to parameters proposed by Medsger et al. involving general symptoms, Raynaud's phenomenon severity, Rodnan skin score, musculoskeletal symptoms, lung, heart and kidney involvement. Factors related to scleroderma renal crisis are presented, to provide clinical evaluation of patients at risk of this complication. FAU - Wiesik-Szewczyk, Ewa AU - Wiesik-Szewczyk E AD - Instytut Reumatologii w Warszawie, Klinika i Poliklinika Układowych Chorób Tkanki Łacznej. ewa.w.szewczyk@gmail.com FAU - Olesińska, Marzena AU - Olesińska M LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Współczesny obraz kliniczny twardziny układowej. PL - Poland TA - Pol Merkur Lekarski JT - Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego JID - 9705469 SB - IM MH - Cause of Death MH - Diagnosis, Differential MH - Disease Progression MH - Echocardiography, Doppler MH - Humans MH - Prognosis MH - Raynaud Disease/diagnosis MH - Scleroderma, Diffuse/*diagnosis MH - Scleroderma, Limited/*diagnosis MH - Tomography, X-Ray Computed RF - 20 EDAT- 2010/06/24 06:00 MHDA- 2010/08/07 06:00 CRDT- 2010/06/24 06:00 PHST- 2010/06/24 06:00 [entrez] PHST- 2010/06/24 06:00 [pubmed] PHST- 2010/08/07 06:00 [medline] PST - ppublish SO - Pol Merkur Lekarski. 2010 May;28(167):416-20. PMID- 35135560 OWN - NLM STAT- MEDLINE DCOM- 20220222 LR - 20240822 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Feb 8 TI - Capillary microscopy is a potential screening method for connective tissue disease in children with Raynaud's phenomenon. PG - 11 LID - 10.1186/s12969-022-00671-0 [doi] LID - 11 AB - BACKGROUND: Nailfold capillary microscopy (NCM) is a cornerstone in the diagnosis of Systemic Sclerosis (SSc) in adulthood. Although Raynaud's phenomenon (RP) is very common in childhood, studies on diagnostic methods to differentiate between primary RP (PRP) and secondary RP (SRP) at a young age are scarce. The aim of this study was to determine the value of NCM in differentiating between PRP and SRP in children and adolescents with RP. METHODS: In this nested case-control study, 83 patients diagnosed with RP and having underwent NCM in childhood were retrospectively included. Based on whether they were diagnosed with a connective tissue disease (CTD) during follow-up, patients were classified as PRP or SRP. NCM was performed by a vascular technician. PRP and SRP patients were compared on demographics, NCM and serology. Variables associated with SRP were included in a multivariate logistic regression model. Predictive values were calculated for NCM, ANA positivity and the combination of NCM and ANA positivity. RESULTS: At the time of the NCM, the mean age of the RP patients was 15.4 ± 2.3 years. Of these patients, 78.3% were classified as PRP and 21.7% as SRP at mean follow-up of 6.4 ± 3.20 years. CTDs were miscellaneous, with only one patient having developed SSc. Of the NCM parameters, only capillary loss was associated with SRP (p = 0.01). In a multivariate logistic regression model including ANA, capillary loss was not a predictor of SRP. In a model without ANAs, capillary loss was an independent predictor (OR = 3.98, CI 95% 1.22-12.99). Capillary loss had a sensitivity of 44.4% and a specificity of 84.4% for SRP. ANA combined with capillary loss had a sensitivity of 66.7% and a specificity of 85.7%. CONCLUSION: Whereas RP in adulthood is most strongly associated with SSc, children with RP seem to be at risk for developing other CTDs with less apparent NCM abnormalities. Of all NCM findings, only capillary loss was predictive for SRP. NCM did not add to the predictive value of ANA screening. However, with a specificity of 84.4% and being non-invasive, NCM shows potential as a screening method for SRP. More research with a larger study population is required before drawing conclusions. CI - © 2022. The Author(s). FAU - Farenhorst, Claudette A AU - Farenhorst CA AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. FAU - Roon, Anniek M AU - Roon AM AD - Deptartment of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands. a.m.van.roon01@umcg.nl. FAU - Gessel, Anne I AU - Gessel AI AD - Deptartment of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands. FAU - Stel, Alja J AU - Stel AJ AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. AD - Deptartment of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands. FAU - Bootsma, Hendrika AU - Bootsma H AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. FAU - Armbrust, Wineke AU - Armbrust W AD - Department of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. FAU - Mulder, Douwe J AU - Mulder DJ AD - Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. AD - Deptartment of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands. LA - eng PT - Journal Article DEP - 20220208 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM MH - Adolescent MH - Capillaries/*diagnostic imaging MH - Case-Control Studies MH - Child MH - Connective Tissue Diseases/*diagnosis MH - Female MH - Humans MH - Male MH - Microscopy/*methods MH - Nails/*blood supply MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis MH - Retrospective Studies PMC - PMC8822798 OTO - NOTNLM OT - Connective tissue disease OT - Nailfold capillary microscopy OT - Raynaud’s phenomenon COIS- The authors declare that they have no competing interests. EDAT- 2022/02/10 06:00 MHDA- 2022/02/23 06:00 PMCR- 2022/02/08 CRDT- 2022/02/09 05:39 PHST- 2021/08/30 00:00 [received] PHST- 2022/01/27 00:00 [accepted] PHST- 2022/02/09 05:39 [entrez] PHST- 2022/02/10 06:00 [pubmed] PHST- 2022/02/23 06:00 [medline] PHST- 2022/02/08 00:00 [pmc-release] AID - 10.1186/s12969-022-00671-0 [pii] AID - 671 [pii] AID - 10.1186/s12969-022-00671-0 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Feb 8;20(1):11. doi: 10.1186/s12969-022-00671-0. PMID- 37980267 OWN - NLM STAT- MEDLINE DCOM- 20240214 LR - 20240214 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 45 IP - 2 DP - 2024 Feb TI - [Update on targeted biopharmaceuticals in systemic sclerosis!]. PG - 109-113 LID - S0248-8663(23)01246-8 [pii] LID - 10.1016/j.revmed.2023.11.002 [doi] AB - Systemic sclerosis (SSc) is a rare connective tissue disease characterized by inflammation, fibrosis, and autoimmunity. Despite few clinical trials when compared to other autoimmune diseases, SSc has benefited from renewed interest over the past ten years and a large number of clinical trials have been performed or are underway. We present here the results of the trials published in the last 5 years in ScS according to the chosen endpoint criteria and describe the trials in progress or expected in the years to come. CI - Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Chaigne, B AU - Chaigne B AD - Service de médecine interne, centre de référence maladies autoimmunes systémiques Rares d'Ile de France, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; AP-HP-CUP, Université Paris Cité, Hôpital Cochin, 75014 Paris, France. Electronic address: benjamin.chaigne@aphp.fr. FAU - Mouthon, L AU - Mouthon L AD - Service de médecine interne, centre de référence maladies autoimmunes systémiques Rares d'Ile de France, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; AP-HP-CUP, Université Paris Cité, Hôpital Cochin, 75014 Paris, France. LA - fre PT - English Abstract PT - Journal Article TT - Biothérapies ciblées : nouveautés dans la sclérodermie systémique ! DEP - 20231118 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Biological Products) SB - IM MH - Humans MH - *Biological Products/therapeutic use MH - *Scleroderma, Systemic/drug therapy MH - *Connective Tissue Diseases MH - *Raynaud Disease OTO - NOTNLM OT - Fibrose OT - Fibrosis OT - Interstitial lung disease OT - Pneumopathie interstitielle diffuse OT - Raynaud OT - Raynaud's phenomenon OT - Sclérodermie systémique OT - Systemic sclerosis OT - Traitement EDAT- 2023/11/19 09:42 MHDA- 2024/02/11 16:43 CRDT- 2023/11/18 22:01 PHST- 2023/09/08 00:00 [received] PHST- 2023/10/24 00:00 [revised] PHST- 2023/11/04 00:00 [accepted] PHST- 2024/02/11 16:43 [medline] PHST- 2023/11/19 09:42 [pubmed] PHST- 2023/11/18 22:01 [entrez] AID - S0248-8663(23)01246-8 [pii] AID - 10.1016/j.revmed.2023.11.002 [doi] PST - ppublish SO - Rev Med Interne. 2024 Feb;45(2):109-113. doi: 10.1016/j.revmed.2023.11.002. Epub 2023 Nov 18. PMID- 16905623 OWN - NLM STAT- MEDLINE DCOM- 20070824 LR - 20161124 IS - 0962-7480 (Print) IS - 0962-7480 (Linking) VI - 56 IP - 7 DP - 2006 Oct TI - Thenar hammer syndrome: a case report. PG - 507-9 AB - BACKGROUND: Raynaud's phenomenon occurs among automobile mechanics secondary to long-term use of vibrating hand-held tools. It can also occur from traumatic injury to the upper extremity. AIM: This report describes a case of single digit Raynaud's phenomenon in an automobile mechanic due to focal arterial impact trauma. CASE REPORT: A 38-year-old right-handed transmission mechanic complained of paraesthesia and blanching of the right index finger on exposure to cold and eventually developed a transient necrotic ulcer at the tip of the digit. He had a long history of occupational exposure to vibrating hand-held power tools. Evaluation for common causes of Raymond's phenomenon was negative. The diagnosis of hand-arm vibration syndrome (HAVS) was rejected because of the rapidity of progression and severity of the symptoms restricted only to the index finger without corresponding symptoms of the other digits of the right hand as would be expected. Angiography revealed an obstructive lesion of the distal right radial artery at the wrist and he was diagnosed with thenar hammer syndrome. This uncommon condition was due to focal injury of the distal radial artery caused by repeated slamming of transmission parts on a work table. CONCLUSIONS: Not all cases of Raynaud's phenomenon in workers using vibrating hand-held tools are due to HAVS. Alternative aetiologies should be considered especially if symptoms are asymmetrical and unilateral. FAU - Youakim, Sami AU - Youakim S AD - WorkSafeBC, Workers' Compensation Board of British Columbia, Canada. syouakim@telusplanet.net LA - eng PT - Case Reports PT - Journal Article DEP - 20060811 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Adult MH - *Automobiles MH - Finger Injuries/diagnostic imaging/etiology MH - Humans MH - Male MH - Occupational Diseases/diagnostic imaging/*etiology MH - Radial Artery/diagnostic imaging MH - Radiography MH - Raynaud Disease/diagnostic imaging/*etiology MH - Vibration/adverse effects MH - Wounds, Nonpenetrating/diagnostic imaging/etiology MH - Wrist Injuries/diagnostic imaging/etiology EDAT- 2006/08/15 09:00 MHDA- 2007/08/25 09:00 CRDT- 2006/08/15 09:00 PHST- 2006/08/15 09:00 [pubmed] PHST- 2007/08/25 09:00 [medline] PHST- 2006/08/15 09:00 [entrez] AID - kql085 [pii] AID - 10.1093/occmed/kql085 [doi] PST - ppublish SO - Occup Med (Lond). 2006 Oct;56(7):507-9. doi: 10.1093/occmed/kql085. Epub 2006 Aug 11. PMID- 32173825 OWN - NLM STAT- MEDLINE DCOM- 20200917 LR - 20200917 IS - 2724-7031 (Electronic) IS - 0041-4131 (Linking) VI - 97 IP - 11 DP - 2019 Nov TI - Antinuclear antibodies in interstitial lung disease: Prevalence and clinical significance. PG - 1240-1245 AB - INTRODUCTION: The diagnosis of interstitial lung disease (ILD) requires elimination of underlying connective tissue disease. Consequently, antinuclear antibodies (ANA) are routinely screened in patients with idiopathic interstitial pneumonia. However the clinical usefulness of this practice is not well clear. AIM: In this study, we evaluated the frequency of ANA in ILD's patients and investigated the clinical significance of the ANA's presence in these patients. METHODS: We conducted a retrospective study of hospitalized patients diagnosed ILD at pulmonary department and for which ANA was performed in the immunology laboratory of our institution. Demographic features, clinical symptoms, biological and radiologic findings and CTD-ILD diagnoses were compared between patients with positive ANA versus negative ANA. RESULTS: We enrolled 73 patients. The ANA's prevalence was 32%. There were no significant differences in demographics, pulmonary function test values and radiologic findings between patients with and without ANA. Patients with positive ANA had more cutaneous manifestations (p꞊0.011) and Raynaud's phenomenon (p꞊0.029). The diagnosis of connective tissue disease was made in 42% of patients with positive ANA versus 8% with negative ANA (p꞊ 0.001). ANA's titer higher than 1/320 was predictive of CTD diagnosis (OR꞊14.4) (p<0.001). CONCLUSIONS: The research of ANA in PID's patients is an important tool of CTD diagnosis specially in those with suggestive symptoms of autoimmune disease. FAU - Ghrairi, Najla AU - Ghrairi N FAU - Aouadi, Samira AU - Aouadi S FAU - Elhechmi, Youssef Zied AU - Elhechmi YZ FAU - Ben Saad, Soumaya AU - Ben Saad S FAU - Ben Ali, Imen AU - Ben Ali I FAU - Yalaoui, Sadok AU - Yalaoui S LA - eng PT - Journal Article PL - Tunisia TA - Tunis Med JT - La Tunisie medicale JID - 0413766 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*blood MH - Autoimmune Diseases/blood/complications/diagnosis/*epidemiology MH - Case-Control Studies MH - Connective Tissue Diseases/blood/diagnosis/epidemiology MH - Female MH - Hospitalization/statistics & numerical data MH - Humans MH - Idiopathic Pulmonary Fibrosis/blood/diagnosis/epidemiology MH - Lung Diseases, Interstitial/blood/*diagnosis/*epidemiology/therapy MH - Male MH - Prevalence MH - Prognosis MH - Raynaud Disease/blood/diagnosis/epidemiology MH - Respiratory Function Tests MH - Retrospective Studies MH - Seroepidemiologic Studies MH - Tomography, X-Ray Computed EDAT- 2020/03/17 06:00 MHDA- 2020/09/18 06:00 CRDT- 2020/03/17 06:00 PHST- 2020/03/17 06:00 [entrez] PHST- 2020/03/17 06:00 [pubmed] PHST- 2020/09/18 06:00 [medline] AID - /article-medicale-tunisie.php?article=3620 [pii] PST - ppublish SO - Tunis Med. 2019 Nov;97(11):1240-1245. PMID- 29558039 OWN - NLM STAT- MEDLINE DCOM- 20190701 LR - 20220330 IS - 1899-5276 (Print) IS - 1899-5276 (Linking) VI - 27 IP - 11 DP - 2018 Nov TI - Analysis of peripheral nerve and autonomic nervous system function and the stage of microangiopathy in patients with secondary Raynaud's phenomenon in the course of connective tissue diseases. PG - 1587-1592 LID - 10.17219/acem/75618 [doi] AB - BACKGROUND: The pathogenesis of secondary Raynaud's phenomenon (SRP) associated with connective tissue diseases (CTD) is not entirely understood. Nervous system dysfunction and microangiopathy are considered to be causes of this pathology. OBJECTIVES: Peripheral and autonomic nervous system function, the stage of microangiopathy, and the relationships between these in patients with SRP were analyzed. MATERIAL AND METHODS: In the study, 20 patients with CTD-related SRP and 30 healthy controls were subject to capillaroscopy, standard conduction velocity tests and conduction velocity distribution (CVD) tests in ulnar and peroneal nerves, heart rate variability (HRV), and sympathetic skin response (SSR) tests. RESULTS: There were no significant differences in the standard motor and sensory conduction velocity tests, or in CVD tests in the ulnar and peroneal nerves in SRP patients compared with the controls. The patients with SRP had a significantly lower SSR amplitude and longer latency in hands and feet. The patients with CTD-related SRP had a significantly lower mean HRV with higher low frequency (LF) values in the spectral analysis and expiration/inspiration ratio (E/I) during deep breathing. There was no correlation between the stage of microangiopathy and neurophysiological test results. CONCLUSIONS: Correct standard conduction velocity and CVD testing in patients with SPR suggest that vasomotor disturbances may occur in CTD regardless of peripheral neuropathy. The lack of relationship between SSR and microangiopathy could confirm that these 2 processes occur independently in patients with CTD-related SRP. Autonomic nervous system impairment together with normal peripheral nerve function suggest the central origin of CTD-related SRP. FAU - Gosk-Bierska, Izabela AU - Gosk-Bierska I AD - Department of Angiology, Hypertension and Diabetology, Wroclaw Medical University, Poland. FAU - Misterska-Skóra, Maria AU - Misterska-Skóra M AD - Department and Clinic of Rheumatology and Internal Medicine, Wroclaw Medical University, Poland. FAU - Wasilewska, Marta AU - Wasilewska M AD - Department of Angiology, Hypertension and Diabetology, Wroclaw Medical University, Poland. FAU - Bilińska, Małgorzata AU - Bilińska M AD - Department and Clinic of Neurology, Wroclaw Medical University, Poland. FAU - Gosk, Jerzy AU - Gosk J AD - Clinical Department of Traumatology and Hand Surgery, Wroclaw Medical University, Poland. FAU - Adamiec, Rajmund AU - Adamiec R AD - Department of Angiology, Hypertension and Diabetology, Wroclaw Medical University, Poland. FAU - Koszewicz, Magdalena AU - Koszewicz M AD - Department and Clinic of Neurology, Wroclaw Medical University, Poland. LA - eng PT - Journal Article PL - Poland TA - Adv Clin Exp Med JT - Advances in clinical and experimental medicine : official organ Wroclaw Medical University JID - 101138582 SB - IM MH - Autonomic Nervous System/*physiology MH - Case-Control Studies MH - Connective Tissue Diseases/*physiopathology MH - Humans MH - Neural Conduction MH - Peripheral Nerves/*physiology MH - Peroneal Nerve MH - Raynaud Disease/*physiopathology OTO - NOTNLM OT - autonomic nervous system OT - connective tissue diseases OT - microangiopathy OT - peripheral nerves OT - secondary Raynaud’s syndrome EDAT- 2018/03/21 06:00 MHDA- 2019/07/02 06:00 CRDT- 2018/03/21 06:00 PHST- 2018/03/21 06:00 [pubmed] PHST- 2019/07/02 06:00 [medline] PHST- 2018/03/21 06:00 [entrez] AID - 10.17219/acem/75618 [doi] PST - ppublish SO - Adv Clin Exp Med. 2018 Nov;27(11):1587-1592. doi: 10.17219/acem/75618. PMID- 16763543 OWN - NLM STAT- MEDLINE DCOM- 20061114 LR - 20151119 IS - 0392-9590 (Print) IS - 0392-9590 (Linking) VI - 25 IP - 2 DP - 2006 Jun TI - The role of endothelin-1 and selected cytokines in the pathogenesis of Raynaud's phenomenon associated with systemic connective tissue diseases. PG - 221-7 AB - AIM: In the pathogenesis of Raynaud's phenomenon humoral and immunoinflammatory agents are involved. The aim of this study was the assessment of the level of endothelin-1 (ET-1), tumor necrosis factor (TNF-alpha), interleukin-6 (IL-6), soluble IL-6 receptor (IL-6sR), von Willebrand's factor (vWF) and platelet factor 4 (PF-4) in patients with Raynaud's phenomenon associated with systemic connective tissue diseases. METHODS: The examined group consisted of 32 patients (24 women and 8 men) with Raynaud's phenomenon associated with selected connective tissue diseases, aged 28-50 years. A control group consisted of 13 healthy volunteers. Immediately after a cold provocation test venous blood was taken in order to assess serum concentrations of: TNF-alpha, IL-6, IL-6sR, vWF, PF-4, antinuclear antibodies (ANA), antineutrophil antibodies (c-ANCA). RESULTS: In the group of patients with Raynaud's phenomenon mean serum concentration of ET-1, TNF-alpha, PF-4, and vWF was significantly greater than in the healthy group. In contrast, serum IL-6 and IL-6sR concentrations did not differ significantly between the diseased and healthy groups. In a subgroup of Raynaud's phenomenon patients showing particularly high concentration of serum ET-1 (twice as much as mean control concentration), the increase in IL-6, IL-6sR, vWF and c-ANCA concentration exhibited statistical significance in comparison with patients with lower serum ET-1 concentration. The vWF concentration exhibited positive correlation with time interval between the occurrence of clinical symptoms and serum ANA antibodies concentration. The increase in ET-1 synthesis in Raynaud's phenomenon patients is dependent on the increase in IL-6 level and c-ANCA antibodies level. CONCLUSIONS: The patients with Raynaud's phenomenon show an increase in ET-1 and TNF-alpha concentrations. An enhanced ET-1 synthesis is dependent on the augmentation of serum c-ANCA antibodies and IL-6 concentrations. FAU - Rychlik-Golema, W AU - Rychlik-Golema W AD - Department and Clinic of Angiology, Hypertension and Diabetology, Medical University of Wroclaw, Wroclaw, Poland. FAU - Mastej, K AU - Mastej K FAU - Adamiec, R AU - Adamiec R LA - eng PT - Comparative Study PT - Journal Article PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Cytokines) RN - 0 (Endothelin-1) RN - 0 (Interleukin-6) RN - 0 (Receptors, Interleukin-6) RN - 0 (Tumor Necrosis Factor-alpha) RN - 0 (von Willebrand Factor) RN - 37270-94-3 (Platelet Factor 4) SB - IM CIN - Int Angiol. 2006 Dec;25(4):436. PMID: 17164754 MH - Adult MH - Antibodies, Antineutrophil Cytoplasmic/blood MH - Antibodies, Antinuclear/blood MH - Biomarkers/blood MH - Connective Tissue Diseases/blood/*complications MH - Cytokines/*metabolism MH - Disease Progression MH - Endothelin-1/*metabolism MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Follow-Up Studies MH - Humans MH - Interleukin-6/blood MH - Male MH - Middle Aged MH - Platelet Factor 4/metabolism MH - Raynaud Disease/blood/*etiology MH - Receptors, Interleukin-6/blood MH - Severity of Illness Index MH - Tumor Necrosis Factor-alpha/metabolism MH - von Willebrand Factor/metabolism EDAT- 2006/06/10 09:00 MHDA- 2006/11/15 09:00 CRDT- 2006/06/10 09:00 PHST- 2006/06/10 09:00 [pubmed] PHST- 2006/11/15 09:00 [medline] PHST- 2006/06/10 09:00 [entrez] PST - ppublish SO - Int Angiol. 2006 Jun;25(2):221-7. PMID- 19779765 OWN - NLM STAT- MEDLINE DCOM- 20100219 LR - 20161020 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 19 IP - 6 DP - 2009 TI - The specificity of capillaroscopic pattern in connective autoimmune diseases. A comparison with microvascular changes in diseases of social importance: arterial hypertension and diabetes mellitus. PG - 600-5 LID - 10.1007/s10165-009-0221-x [doi] AB - Capillaroscopy is a method with substantial value for diagnosis and differentiation of primary and secondary Raynaud's phenomenon in rheumatic diseases. The most specific finding is in systemic sclerosis--the so-called "scleroderma pattern." which is characterized by the presence of dilated capillaries, hemorrhages, avascular areas, and neoangiogenesis. Similar changes are found in patients with dermatomyositis, overlap syndromes, and others and are termed "scleroderma-like pattern." For the development of these patterns, the most specific finding in the early phase is appearance of dilated capillaries. Capillaroscopic changes in connective autoimmune diseases are specific and differ significantly from those of that can be found in other diseases. Diseases of social importance such as diabetes mellitus and arterial hypertension often present as comorbidity in patients with rheumatic diseases. In diabetes mellitus, the capillaroscopic examination does not show dilated capillaries until the advanced stages of the disease. In the late stages of connective tissue disease, a loss of capillaries is typical. In addition, in diabetes mellitus, the diabetic stiff-hand syndrome and sclerodactyly are common complications, which have to be differentiated from similar signs in rheumatic diseases, and capillaroscopic examination appears to be useful in these situations. In arterial hypertension, a reduced capillary density in different body regions has been observed in patients with established disease as well as in preclinical stages. Analogous phenomenon of reduction in the nail-fold area has also been observed in a group of patients with essential hypertension, none of whom previously received hypertensive drugs. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Clinic of Rheumatology, Medical University, 4002 Plovdiv, Bulgaria. sevdalina_n@abv.bg FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Autoimmune Diseases/complications/*diagnosis MH - Capillaries/*pathology MH - Connective Tissue Diseases/complications/*diagnosis MH - Diabetes Mellitus/*diagnosis MH - Humans MH - Hypertension/*diagnosis MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/diagnosis/etiology MH - Rheumatic Diseases/complications/diagnosis RF - 52 EDAT- 2009/09/26 06:00 MHDA- 2010/02/20 06:00 CRDT- 2009/09/26 06:00 PHST- 2009/02/24 00:00 [received] PHST- 2009/07/29 00:00 [accepted] PHST- 2009/09/26 06:00 [entrez] PHST- 2009/09/26 06:00 [pubmed] PHST- 2010/02/20 06:00 [medline] AID - 10.1007/s10165-009-0221-x [doi] PST - ppublish SO - Mod Rheumatol. 2009;19(6):600-5. doi: 10.1007/s10165-009-0221-x. PMID- 21338324 OWN - NLM STAT- MEDLINE DCOM- 20111013 LR - 20110822 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 40 IP - 4 DP - 2011 TI - The combination of laser Doppler perfusion imaging and photoplethysmography is useful in the characterization of scleroderma and primary Raynaud's phenomenon. PG - 292-8 LID - 10.3109/03009742.2010.530293 [doi] AB - OBJECTIVE: To investigate simultaneously skin perfusion and digital artery pulsatility of hands in patients with primary Raynaud's phenomenon (PRP) and systemic sclerosis (SSc). METHODS: In 100 SSc patients, 92 PRP patients, and 80 healthy controls, perfusion and digital artery pulsatility of the hands were investigated by laser Doppler perfusion imaging (LDPI) and photoplethysmography (PPG), respectively. RESULTS: Both the mean value of mean perfusion and the mean value of sphygmic wave amplitude were lower (p < 0.0001) in the PRP group than in the SSc group, and also in the SSc group compared with the healthy controls. A positive correlation (r = 0.95) was found between the mean value of mean perfusion and the mean value of sphygmic wave amplitude. A homogeneous perfusion distribution pattern was present in 95% of the healthy controls, 93% of the PRP patients, and 4% of the SSc patients. PPG shows a homogeneous pattern in 95% of the healthy controls, 93% of the PRP patients, and 28% of the SSc patients. LDPI and PPG showed a positive concordance (p < 0.05) in homogeneous pattern evaluation. In the SSc patients, no correlation (r = 0.38) was observed between the mean value of sphygmic wave amplitude and the mean value of mean perfusion of each finger. CONCLUSION: LDPI and PPG can provide useful information in distinguishing patients with PRP and SSc, although nailfold videocapillaroscopy (NVC) is the best method for analysing microvascular damage in rheumatic diseases. FAU - Rosato, E AU - Rosato E AD - Department of Clinical Medicine, Clinical Immunology Unit - Scleroderma Centre, Sapienza University of Rome, Rome, Italy. FAU - Molinaro, I AU - Molinaro I FAU - Rossi, C AU - Rossi C FAU - Pisarri, S AU - Pisarri S FAU - Salsano, F AU - Salsano F LA - eng PT - Journal Article DEP - 20110222 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Blood Volume/physiology MH - Capillaries/physiology MH - Case-Control Studies MH - Female MH - Hand/blood supply MH - Humans MH - Male MH - Middle Aged MH - Nails/blood supply MH - Perfusion Imaging/*methods MH - Photoplethysmography/*methods MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/*physiopathology EDAT- 2011/02/23 06:00 MHDA- 2011/10/14 06:00 CRDT- 2011/02/23 06:00 PHST- 2011/02/23 06:00 [entrez] PHST- 2011/02/23 06:00 [pubmed] PHST- 2011/10/14 06:00 [medline] AID - 10.3109/03009742.2010.530293 [doi] PST - ppublish SO - Scand J Rheumatol. 2011;40(4):292-8. doi: 10.3109/03009742.2010.530293. Epub 2011 Feb 22. PMID- 29866252 OWN - NLM STAT- MEDLINE DCOM- 20190204 LR - 20221207 IS - 1681-7168 (Electronic) IS - 1022-386X (Linking) VI - 28 IP - 6 DP - 2018 Jun TI - A Rare Case of Kikuchi-Fujimoto Disease Associated with Systemic Lupus Erythematosus. PG - S143-S145 LID - 10.29271/jcpsp.2018.06.S143 [doi] AB - Kikuchi-Fujimoto Disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-remitting, immune-mediated rare disorder having unique histopathological characteristics which is commonly seen in young Asian females, but can occur in all ethnicities. There is a strong association between KFD and Systemic Lupus Erythematosus (SLE). We present a case of a young Pakistani boy who presented with cervical lymphadenopathy, fever, blackish discoloration of finger tips, and Raynaud's phenomenon. His lymph node biopsy was suggestive of KFD. The American Rheumatology Association diagnostic criteria were not met as no other features of SLE were present. His autoimmune workup including Anti-Nuclear Antibodies (ANA) and Anti-Double Stranded DNA (Anti-Ds DNA) antibodies were positive and supported the diagnosis of SLE. He improved clinically with steroid therapy and nifedipine with resolution of symptoms. FAU - Bari, Attia AU - Bari A AD - Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore. FAU - Zeeshan, Fatima AU - Zeeshan F AD - Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore. FAU - Hanif, Ghazala AU - Hanif G AD - Department of Histopathology, The Children's Hospital and the ICH, Lahore. FAU - Jabeen, Uzma AU - Jabeen U AD - Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore. FAU - Bano, Iqbal AU - Bano I AD - Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore. FAU - Rathore, Ahsan Waheed AU - Rathore AW AD - Department of Pediatric Medicine, The Children's Hospital and the ICH, Lahore. LA - eng PT - Case Reports PT - Journal Article PL - Pakistan TA - J Coll Physicians Surg Pak JT - Journal of the College of Physicians and Surgeons--Pakistan : JCPSP JID - 9606447 RN - 0 (Steroids) RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adolescent MH - Asian People MH - Fever/*etiology MH - Histiocytic Necrotizing Lymphadenitis/complications/*diagnosis/drug therapy MH - Humans MH - Lupus Erythematosus, Systemic/complications/drug therapy MH - Lymphadenopathy/*etiology MH - Male MH - Nifedipine/therapeutic use MH - Raynaud Disease/diagnosis/drug therapy MH - Sentinel Lymph Node Biopsy MH - Steroids/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2018/06/06 06:00 MHDA- 2019/02/05 06:00 CRDT- 2018/06/06 06:00 PHST- 2017/09/13 00:00 [received] PHST- 2018/01/02 00:00 [accepted] PHST- 2018/06/06 06:00 [entrez] PHST- 2018/06/06 06:00 [pubmed] PHST- 2019/02/05 06:00 [medline] AID - 040579197 [pii] AID - 10.29271/jcpsp.2018.06.S143 [doi] PST - ppublish SO - J Coll Physicians Surg Pak. 2018 Jun;28(6):S143-S145. doi: 10.29271/jcpsp.2018.06.S143. PMID- 38363440 OWN - NLM STAT- MEDLINE DCOM- 20240526 LR - 20240526 IS - 1863-4362 (Electronic) IS - 0021-1265 (Linking) VI - 193 IP - 3 DP - 2024 Jun TI - Interstitial lung disease and associated factors in patients with Sjögren's syndrome. PG - 1385-1389 LID - 10.1007/s11845-024-03629-1 [doi] AB - BACKGROUND: Interstitial lung disease (ILD) is a common pulmonary manifestation of Sjögren's syndrome (SjS) and associated with an increased risk of death. Early detection and treatment of ILDs and knowing the risk factors are very important for prognosis in rheumatic diseases. AIMS: This study was performed to determine ILD and associated factors in patients with SjS. METHODS: Four hundred three SjS patients were evaluated in this cross-sectional cohort study. Clinical, laboratory, serological, and imaging features were compared of patients with and without pulmonary involvement. Logistic regression analyses were used to identify risk factors for lung involvement and to identify independent risk factors. RESULTS: Thirty-five (8.7%) of SjS patients had ILD and 368 (91.3%) had no ILD. The presence of Raynaud's phenomenon was significantly more common in ILD. The geriatric age group over the age of 65 years (OR 8198; 95% CI 3788-17,742; p < 0.001), Raynaud's phenomenon (OR 17,852; 95% CI 6155-51,779; p < 0.001), and smoking (OR 3598; 95% CI 1495-8657; p = 0.003) were risk factors to be associated for ILD in the multivariable analysis. The most common abnormality was non-specific interstitial pneumonia in 20 patients (57.1%) and usual interstitial pneumonia in 15 (42.9%) patients. CONCLUSIONS: The distribution of male patients compared to female patients was higher in patients with lung involvement than in patients without lung involvement. This may be related to older age, higher smoking rate, and longer nicotine consumption in men. Age, smoking, and severity of lung involvement are more important than inflammation status and autoantibodies for prognosis. CI - © 2024. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland. FAU - Sargın, Gökhan AU - Sargın G AUID- ORCID: 0000-0002-3778-8351 AD - Department of Rheumatology, Medical Faculty, Aydin Adnan Menderes University, Aydın, Turkey. gokhan_sargin@hotmail.com. FAU - Baygin, Huseyin AU - Baygin H AD - Department of Rheumatology, Medical Faculty, Aydin Adnan Menderes University, Aydın, Turkey. FAU - Cildag, Songul AU - Cildag S AD - Department of Rheumatology, Medical Faculty, Aydin Adnan Menderes University, Aydın, Turkey. FAU - Senturk, Taskin AU - Senturk T AD - Department of Rheumatology, Medical Faculty, Aydin Adnan Menderes University, Aydın, Turkey. LA - eng PT - Journal Article DEP - 20240216 PL - Ireland TA - Ir J Med Sci JT - Irish journal of medical science JID - 7806864 SB - IM MH - Humans MH - *Sjogren's Syndrome/complications MH - *Lung Diseases, Interstitial/etiology/complications MH - Female MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - Risk Factors MH - Aged MH - *Raynaud Disease/etiology/epidemiology/complications MH - Smoking/adverse effects/epidemiology MH - Adult MH - Age Factors OTO - NOTNLM OT - Associated factors OT - Interstitial lung disease OT - Pulmonary involvement OT - Sjögren’s syndrome EDAT- 2024/02/16 12:47 MHDA- 2024/05/27 00:42 CRDT- 2024/02/16 11:11 PHST- 2023/11/06 00:00 [received] PHST- 2024/02/08 00:00 [accepted] PHST- 2024/05/27 00:42 [medline] PHST- 2024/02/16 12:47 [pubmed] PHST- 2024/02/16 11:11 [entrez] AID - 10.1007/s11845-024-03629-1 [pii] AID - 10.1007/s11845-024-03629-1 [doi] PST - ppublish SO - Ir J Med Sci. 2024 Jun;193(3):1385-1389. doi: 10.1007/s11845-024-03629-1. Epub 2024 Feb 16. PMID- 17497610 OWN - NLM STAT- MEDLINE DCOM- 20070709 LR - 20221207 IS - 0722-1819 (Print) IS - 0722-1819 (Linking) VI - 39 IP - 2 DP - 2007 Apr TI - [Manifestations of scleroderma at the hand--options for hand surgery in an interdisciplinary concept]. PG - 128-34 AB - Clinical manifestations of scleroderma at the hand include Raynaud's phenomenon, calcinosis cutis, sclerodactylia and teleangiectasia. With the progression of the disease, cutaneous and joint contractions, acro-osteolysis, necrosis of the finger tips, and even extensive digital ulceration are likely to occur. These painful and often rapidly advancing lesions cause loss of function and disfigurement and, untreated, often lead to mutilation of the affected hand. Only an interdisciplinary management including the hand surgeon, the rheumatologist, and the physiotherapist can guarantee optimal treatment. Drug therapy should be included as well as physical therapy. Both should be made use of before and accompanying surgical treatment. Surgical therapy consists of treatment of the infections, excision of calcinosis, arthrodesis, in particular of the proximal interphalangeal joints, and sympathectomy. Amputation remains a final option, whereas with timely and sufficient treatment, amputations can be avoided and an improvement of function and an alleviation of the symptoms can be achieved. Among the non-operative treatment options, behavioural training, calcium antagonists, prostacyclin derivatives, topical nitrates as well as plexus anesthesia and stellatum blocks have proved to be effective. Recent drug therapies include endothelin-receptor antagonists for the prevention of digital ulceration and phosphodiesterase-V antagonists in treatment of Raynaud's phenomenon and induction of ulcer healing. With reference to several cases seen at our institution, we propose an interdisciplinary treatment concept for acral manifestations of scleroderma. FAU - Daigeler, A AU - Daigeler A AD - Universitätsklinik für Plastische Chirurgie und Schwerbrandverletzte, Handchirurgiezentrum, Operatives Referenzzentrum für Gliedmassentumoren, Berufsgenossenschaftliche Kliniken Bergmannsheil, Bürkle-de-la-Camp Platz 1, 44789 Bochum. adrien.daigeler@rub.de FAU - Meyer, M AU - Meyer M FAU - Joneidi-Jafari, H AU - Joneidi-Jafari H FAU - Möcklinghoff, C AU - Möcklinghoff C FAU - Steinau, H-U AU - Steinau HU FAU - Lehnhardt, M AU - Lehnhardt M LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Handbeteiligungen im Rahmen der progressiven systemischen Sklerodermie--Möglichkeiten der Handchirurgie im interdisziplinären Konzept. PL - Germany TA - Handchir Mikrochir Plast Chir JT - Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V... JID - 8302815 SB - IM MH - Aged MH - Amputation, Surgical MH - CREST Syndrome/surgery/therapy MH - Calcinosis/surgery MH - Female MH - Fingers/surgery MH - Follow-Up Studies MH - Hand/*surgery MH - Hand Deformities, Acquired/*surgery MH - Humans MH - Middle Aged MH - Necrosis/surgery MH - Patient Care Team MH - Raynaud Disease/surgery/therapy MH - Scleroderma, Systemic/complications/*surgery/therapy MH - Sympathectomy MH - Time Factors EDAT- 2007/05/15 09:00 MHDA- 2007/07/10 09:00 CRDT- 2007/05/15 09:00 PHST- 2007/05/15 09:00 [pubmed] PHST- 2007/07/10 09:00 [medline] PHST- 2007/05/15 09:00 [entrez] AID - 10.1055/s-2006-924536 [doi] PST - ppublish SO - Handchir Mikrochir Plast Chir. 2007 Apr;39(2):128-34. doi: 10.1055/s-2006-924536. PMID- 17401513 OWN - NLM STAT- MEDLINE DCOM- 20071003 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 9 DP - 2007 Sep TI - Antioxidant status after iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis. PG - 1517-21 AB - Oxidative stress is involved in pathogenesis of Raynaud's phenomenon (RP), a hallmark of systemic sclerosis (SSc). Frequent episodes of ischemia-reperfusion may lead to release of free radicals and enhanced lipid peroxidation reflected by elevated levels of malondialdehyde (MDA). The failure of native antioxidants (Catalase [CAT], Superoxide dismutase [SOD], and Ceruloplasmin [CP]) might be crucial in endothelial cells damage in RP. Iloprost (IL) synthetic prostacyclin analogue is currently used in the treatment of SSc patients with RP. The objectives of this study were to compare the serum levels of MDA and CP, CAT and SOD activity in red blood cells hemolysate in SSc patients compared to healthy controls; and to study the effect of 5-days IL infusions on MDA and CP levels, and CAT and SOD activity in SSc patients with RP. Twelve SSc patients were treated with 50 mug IL for 5 days. Blood samples were taken before and after day 1st and after day 5th of IL infusions. Levels of CAT were measured according to the Aebi's method; SOD, according to the Misra and Fridovich method; MDA, according to Slater's method; and CP, according to Ravin's method. Activities of CAT (p < 0.001) and SOD (p < 0.04) were significantly reduced; levels of CP (p < 0.006) and MDA (p < 0.06) were raised in SSc compared to controls. IL infusions caused reduction in MDA (p < 0.0001) levels and enhanced production of SOD (p < 0.006) and CAT (p < 0.003). The levels of CP did not change (p = 0.48). Oxidant status in SSc patients with RP is impaired. Therapy with IL led to normalization of antioxidant activity. We suggest that CAT may be a sensitive and reliable laboratory marker of oxidative stress severity in RP. We found that IL, in addition to its vasoactive properties, has a potential to activate inner antioxidant system. Activation of inner antioxidant activity may explain long-term effect of IL instead of its very short half-life time. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B. Shine Department of Rheumatology, Rambam Health Care Campus, Haifa, Israel. a_balbir@rambam.health.gov.il FAU - Braun-Moscovici, Yolanda AU - Braun-Moscovici Y FAU - Livshitz, Vladimir AU - Livshitz V FAU - Schapira, Daniel AU - Schapira D FAU - Markovits, Doron AU - Markovits D FAU - Rozin, Alexander AU - Rozin A FAU - Boikaner, Tatiana AU - Boikaner T FAU - Nahir, A Menahem AU - Nahir AM LA - eng PT - Clinical Trial PT - Journal Article DEP - 20070331 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antioxidants) RN - 0 (Platelet Aggregation Inhibitors) RN - 4Y8F71G49Q (Malondialdehyde) RN - EC 1.11.1.6 (Catalase) RN - EC 1.15.1.1 (Superoxide Dismutase) RN - EC 1.16.3.1 (Ceruloplasmin) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Antioxidants MH - Catalase/blood/drug effects MH - Ceruloplasmin/analysis/drug effects MH - Female MH - Humans MH - Iloprost/*pharmacology MH - Malondialdehyde/blood/metabolism MH - Middle Aged MH - Oxidative Stress/*drug effects MH - Platelet Aggregation Inhibitors/*pharmacology MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications/drug therapy MH - Superoxide Dismutase/blood/drug effects EDAT- 2007/04/03 09:00 MHDA- 2007/10/04 09:00 CRDT- 2007/04/03 09:00 PHST- 2006/12/14 00:00 [received] PHST- 2007/03/19 00:00 [accepted] PHST- 2007/03/17 00:00 [revised] PHST- 2007/04/03 09:00 [pubmed] PHST- 2007/10/04 09:00 [medline] PHST- 2007/04/03 09:00 [entrez] AID - 10.1007/s10067-007-0613-2 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Sep;26(9):1517-21. doi: 10.1007/s10067-007-0613-2. Epub 2007 Mar 31. PMID- 19959303 OWN - NLM STAT- MEDLINE DCOM- 20100506 LR - 20100209 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 35 IP - 1 DP - 2010 Feb TI - [Etiological profile of digital necrosis of the upper limbs: analysis of 25 cases]. PG - 12-6 LID - 10.1016/j.jmv.2009.11.001 [doi] AB - AIM: To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases. PATIENTS AND METHODS: We retrospectively reviewed the medical records of patients treated for digital necrosis of the upper limb in four departments of internal medicine from January 1997 to December 2003. RESULTS: There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynaud's phenomenon was noted in 12 cases. Connective tissue diseases were the most common cause (nine cases), all of them were women. The second cause was atherosclerosis (five cases) and Buerger's disease (five cases). In the other cases, the following diagnoses were found: vasculitis (three cases) and neoplasm (two cases). No cause could be identified in one female smoker. CONCLUSION: Digital necrosis is a common symptom, revealing a vascular pathology. Its causes are diverse. In women, it first suggests a connective tissue disease whereas in men, a diffuse arteriopathy. The etiological diagnosis strategy should consider drug intake, anamnesis and Raynaud's phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis. CI - Copyright 2009 Elsevier Masson SAS. All rights reserved. FAU - Abdallah, M AU - Abdallah M AD - Service de médecine interne, CHU Mongi Slim, Sidi Daoud, 2046 La Marsa, Tunisie. meyaabdelkefi@yahoo.fr FAU - Hamzaoui, S AU - Hamzaoui S FAU - Larbi, T AU - Larbi T FAU - Bouslama, K AU - Bouslama K FAU - Harmel, A AU - Harmel A FAU - Ennafaa, M AU - Ennafaa M FAU - Bahloul, Z AU - Bahloul Z FAU - Rokbani, L AU - Rokbani L FAU - Othmani, S AU - Othmani S FAU - Ben Dridi, M AU - Ben Dridi M FAU - M'rad, S AU - M'rad S LA - fre PT - English Abstract PT - Journal Article TT - Profil étiologique des nécroses digitales des membres supérieurs : analyse de 25 observations. DEP - 20091202 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Arteriosclerosis/complications/epidemiology MH - Autoimmune Diseases/complications/epidemiology MH - Connective Tissue Diseases/complications/epidemiology MH - Female MH - Fingers/blood supply/*pathology MH - Humans MH - Ischemia/epidemiology/etiology/*pathology MH - Male MH - Middle Aged MH - Necrosis MH - Raynaud Disease/complications/epidemiology MH - Retrospective Studies MH - Smoking/adverse effects MH - Thromboangiitis Obliterans/complications/epidemiology MH - Tunisia/epidemiology EDAT- 2009/12/05 06:00 MHDA- 2010/05/07 06:00 CRDT- 2009/12/05 06:00 PHST- 2009/05/11 00:00 [received] PHST- 2009/10/21 00:00 [accepted] PHST- 2009/12/05 06:00 [entrez] PHST- 2009/12/05 06:00 [pubmed] PHST- 2010/05/07 06:00 [medline] AID - S0398-0499(09)00308-4 [pii] AID - 10.1016/j.jmv.2009.11.001 [doi] PST - ppublish SO - J Mal Vasc. 2010 Feb;35(1):12-6. doi: 10.1016/j.jmv.2009.11.001. Epub 2009 Dec 2. PMID- 17603692 OWN - NLM STAT- MEDLINE DCOM- 20071026 LR - 20191110 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 59 IP - 2 DP - 2007 Apr-Jun TI - [Endothelin-1 in systemic sclerosis]. PG - 129-34 AB - We evaluated endothelin-1 (ET-1) plasma levels in patients affected by primary Raynaud's phenomenon (PRP), as well as in patients with systemic sclerosis (SSc) and secondary Raynaud's phenomenon (SRP). Furthermore, ET-1 levels were investigated in SSc patients with different patterns of peripheral microvascular damage, as evaluated by nailfold videocapillaroscopy (NVC). METHODS: 23 PRP patients, 67 SSc patients according to ACR criteria, and 23 healthy subjects were enrolled. SSc microvascular involvement was classified in three different patterns (Early, Active, and Late) by NVC, as previously described. RESULTS: ET-1 was found significantly higher in both PRP and SRP, when compared with controls (median +/-IQR: 3.3+/-2.8, 2.7+/-2.2, 2.0+/-2.2, respectively) (p=0.05). No statistically significant difference of ET-1 levels was observed between PRP and SRP patients. ET-1 was found higher in patients with Late NVC pattern, when compared with both Active and Early NVC patterns (median+/-IQR: 3.4+/-2.5, 2.4+/-2.2, 2.5+/-2.1, respectively), but without statistical significance. Patients with Late NVC pattern showed significantly higher ET-1 plasma levels than controls (p=0.03). No correlation was found between ET-1 levels and disease duration in both groups, as well as between ET-1 levels and age of patients. CONCLUSIONS: These data support previous studies, reporting increased ET-1 plasma levels in both PRP and SRP patients. Interestingly, patients with the Late NVC pattern of microangiopathy showed higher ET-1 plasma levels than controls. The high levels of ET-1 detected in the Late NVC pattern of microangiopathy might be related to the larger fibrotic involvement typical of the advanced stages of disease. FAU - Secchi, M E AU - Secchi ME AD - U.O.C. Clinica Reumatologica, Dipartimento di Medicina Interna Università degli Studi di Genova, Genova, Italia. secchime@yahoo.it FAU - Sulli, A AU - Sulli A FAU - Pizzorni, C AU - Pizzorni C FAU - Cutolo, M AU - Cutolo M LA - ita PT - English Abstract PT - Journal Article TT - Endotelina-1 e sclerosi sistemica. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Endothelin-1) SB - IM MH - Endothelin-1/*blood MH - Humans MH - Middle Aged MH - Raynaud Disease/*blood/complications MH - Scleroderma, Systemic/*blood/complications EDAT- 2007/07/03 09:00 MHDA- 2007/10/30 09:00 CRDT- 2007/07/03 09:00 PHST- 2007/07/03 09:00 [pubmed] PHST- 2007/10/30 09:00 [medline] PHST- 2007/07/03 09:00 [entrez] AID - 10.4081/reumatismo.2007.129 [doi] PST - ppublish SO - Reumatismo. 2007 Apr-Jun;59(2):129-34. doi: 10.4081/reumatismo.2007.129. PMID- 26659258 OWN - NLM STAT- MEDLINE DCOM- 20161007 LR - 20181113 IS - 1179-1950 (Electronic) IS - 0012-6667 (Linking) VI - 76 IP - 2 DP - 2016 Feb TI - Diagnosis and Management of Systemic Sclerosis: A Practical Approach. PG - 203-13 LID - 10.1007/s40265-015-0491-x [doi] AB - Systemic sclerosis is a devastating multisystem rheumatologic condition that is characterized by autoimmunity, tissue fibrosis, obliterative vasculopathy and inflammation. Clinical presentation and course of the condition vary greatly, which complicates both diagnosis and corresponding treatment. In this regard, recent advances in disease understanding, both clinically and biochemically, have led to newer classification criteria for systemic sclerosis that are more inclusive than ever before. Still, significant disease modifying therapies do not yet exist for most patients. Therefore, organ-based management strategies are employed and research has been directed within this paradigm focusing on either the most debilitating symptoms, such as Raynaud's phenomenon, digital ulcers and cutaneous sclerosis, or life-threatening organ involvement such as interstitial lung disease and pulmonary arterial hypertension. The current trends in systemic sclerosis diagnosis, evidence-based treatment recommendations and potential future directions in systemic sclerosis treatment are discussed. FAU - Lee, Jason J AU - Lee JJ AD - Division of Rheumatology, Department of Medicine, Schulich School of Medicine, Western University, London, ON, Canada. FAU - Pope, Janet E AU - Pope JE AD - Division of Rheumatology, Department of Medicine, Schulich School of Medicine, Western University, London, ON, Canada. janet.pope@sjhc.london.on.ca. AD - Division of Rheumatology, St. Joseph's Health Care, 268 Grosvenor St., London, ON, N6A 4V2, Canada. janet.pope@sjhc.london.on.ca. LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Drugs JT - Drugs JID - 7600076 SB - IM MH - Disease Management MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Lung Diseases, Interstitial/drug therapy MH - Raynaud Disease/drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Skin Ulcer/drug therapy EDAT- 2015/12/15 06:00 MHDA- 2016/10/08 06:00 CRDT- 2015/12/15 06:00 PHST- 2015/12/15 06:00 [entrez] PHST- 2015/12/15 06:00 [pubmed] PHST- 2016/10/08 06:00 [medline] AID - 10.1007/s40265-015-0491-x [pii] AID - 10.1007/s40265-015-0491-x [doi] PST - ppublish SO - Drugs. 2016 Feb;76(2):203-13. doi: 10.1007/s40265-015-0491-x. PMID- 22304658 OWN - NLM STAT- MEDLINE DCOM- 20121029 LR - 20120704 IS - 1533-2500 (Electronic) IS - 1530-7085 (Linking) VI - 12 IP - 6 DP - 2012 Jul TI - Modulation of somatosensory profiles by spinal cord stimulation in primary Raynaud's syndrome. PG - 469-75 LID - 10.1111/j.1533-2500.2012.00531.x [doi] AB - BACKGROUND AND GOAL:  Spinal cord stimulation (SCS) is an effective antinociceptive treatment for various neuropathic pain syndromes. Apart from antinociceptive action, it may modulate overall somatosensory perception. This case report targets the question of whether SCS may alter quantitative sensory testing (QST) in a patient with primary Raynaud's syndrome. MATERIALS AND METHODS:  We report on a 44-year-old female patient with primary Raynaud's syndrome who had SCS via cervical and lumbar electrodes. QST was performed in a standardized manner assessing cold detection threshold (CDT) and warm detection threshold (WDT), cold pain threshold (CPT) and heat pain threshold (HPT), mechanical detection threshold (MDT) and mechanical pain threshold (MPT) thresholds, and vibration detection threshold (VDT) and pressure pain thresholds (PPT). We tested at the dorsum of the right/left hand of the patient with engaged and disengaged SCS. Test results were compared with a control group of 80 subjects. RESULTS:  Without SCS, the patient showed a sensory decrease in CDT, MDT, MPT, and VDT. SCS influenced the perception of cold, warm, and tactile detection thresholds, whereby CDT, WDT, and VDT were impaired and MDT was improved. CONCLUSION:  SCS significantly modulated the somatosensory profile in a patient with primary Raynaud's syndrome. These effects were pronounced in qualities involving Aβ, C, and A∂ nerve fibers. Further investigations may help to understand the mechanisms of action of SCS. CI - © 2012 The Authors. Pain Practice © 2012 World Institute of Pain. FAU - Münster, Tino AU - Münster T AD - Department of Anesthesiology, Friedrich-Alexander University Erlangen-Nuremberg, Erlangen, Germany. muenster@kfa.imed.uni-erlangen.de FAU - Tiebel, Nils AU - Tiebel N FAU - Seyer, Hendrikus AU - Seyer H FAU - Maihöfner, Christian AU - Maihöfner C LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120205 PL - United States TA - Pain Pract JT - Pain practice : the official journal of World Institute of Pain JID - 101130835 SB - IM MH - Adult MH - Cervical Vertebrae MH - Electric Stimulation Therapy/*methods MH - Female MH - Humans MH - Lumbar Vertebrae MH - Nociception MH - Raynaud Disease/*therapy MH - Sensory Thresholds MH - Somatosensory Disorders/*therapy MH - *Spinal Cord MH - *Thermosensing MH - Touch EDAT- 2012/02/07 06:00 MHDA- 2012/10/30 06:00 CRDT- 2012/02/07 06:00 PHST- 2012/02/07 06:00 [entrez] PHST- 2012/02/07 06:00 [pubmed] PHST- 2012/10/30 06:00 [medline] AID - 10.1111/j.1533-2500.2012.00531.x [doi] PST - ppublish SO - Pain Pract. 2012 Jul;12(6):469-75. doi: 10.1111/j.1533-2500.2012.00531.x. Epub 2012 Feb 5. PMID- 26231412 OWN - NLM STAT- MEDLINE DCOM- 20160516 LR - 20150803 IS - 1776-2588 (Electronic) IS - 0761-8425 (Linking) VI - 32 IP - 6 DP - 2015 Jun TI - [Pulmonary manifestations of antisynthetase syndrome]. PG - 618-28 LID - S0761-8425(14)00371-4 [pii] LID - 10.1016/j.rmr.2014.07.013 [doi] AB - Antisynthetase syndrome is an inflammatory myopathy frequently associated with pulmonary manifestations, especially interstitial lung diseases, and uncommonly pulmonary hypertension. In the context of a suggestive clinical and radiological picture, positive anti-RNA synthetase antibodies confirm the diagnosis. Anti-Jo1, anti-PL7, and anti-PL12 antibodies are the more commonly encountered. The presence of a number of extra-thoracic manifestations in association with pulmonary disease may suggest the diagnosis. These include: myalgia or muscular deficit, Raynaud's phenomenon, polyarthritis, fever, mechanics hands. Serum creatine kinase levels are usually increased. Electromyogram, muscular magnetic resonance imaging or muscle pathology are not mandatory to make the diagnosis. There is a high variability in symptoms and severity, between patients but also during the course of the disease in the same patient. The presence of an interstitial lung disease is a major prognostic factor and an indication for more intensive treatment, principally with systemic corticosteroids with or without immunosuppressive drugs. Improving respiratory physicians' knowledge of this disease, which is often revealed by its pulmonary manifestations, should help diagnosis, therapeutic management, and possibly prognosis. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Jouneau, S AU - Jouneau S AD - Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université de Rennes 1, Rennes, France. Electronic address: stephane.jouneau@chu-rennes.fr. FAU - Hervier, B AU - Hervier B AD - Service de médecine interne et immunologie clinique, groupe hospitalier Pitié-Salpêtrière, AP-HP-UPMC, Paris, France. FAU - Jutant, E-M AU - Jutant EM AD - Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France. FAU - Decaux, O AU - Decaux O AD - Service de médecine interne, hôpital sud, Rennes, France; UMR CNRS 6290 IGDR, université de Rennes 1, Rennes, France. FAU - Kambouchner, M AU - Kambouchner M AD - Service d'anatomie pathologique, hôpital Avicenne, AP-HP, Bobigny, France. FAU - Humbert, M AU - Humbert M AD - Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France. FAU - Delaval, P AU - Delaval P AD - Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université de Rennes 1, Rennes, France. FAU - Montani, D AU - Montani D AD - Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Les manifestations pulmonaires du syndrome des antisynthétases. DEP - 20141019 PL - France TA - Rev Mal Respir JT - Revue des maladies respiratoires JID - 8408032 RN - Antisynthetase syndrome SB - IM MH - Disease Progression MH - Humans MH - Hypertension, Pulmonary/diagnosis/epidemiology/*etiology MH - Lung Diseases, Interstitial/diagnosis/epidemiology/*etiology MH - Myositis/*complications/diagnosis/epidemiology MH - Prognosis MH - Radiography, Thoracic MH - Raynaud Disease/diagnosis/epidemiology/etiology OTO - NOTNLM OT - Anti-Jo1 OT - Hypertension pulmonaire OT - Infiltrative lung disease OT - Inflammatory myopathy OT - Myopathie inflammatoire OT - Myosite OT - Myositis OT - Pneumopathie interstitielle diffuse OT - Pulmonary hypertension EDAT- 2015/08/02 06:00 MHDA- 2016/05/18 06:00 CRDT- 2015/08/02 06:00 PHST- 2014/02/18 00:00 [received] PHST- 2014/07/31 00:00 [accepted] PHST- 2015/08/02 06:00 [entrez] PHST- 2015/08/02 06:00 [pubmed] PHST- 2016/05/18 06:00 [medline] AID - S0761-8425(14)00371-4 [pii] AID - 10.1016/j.rmr.2014.07.013 [doi] PST - ppublish SO - Rev Mal Respir. 2015 Jun;32(6):618-28. doi: 10.1016/j.rmr.2014.07.013. Epub 2014 Oct 19. PMID- 40436656 OWN - NLM STAT- MEDLINE DCOM- 20250901 LR - 20250901 IS - 1768-319X (Electronic) IS - 0294-1260 (Linking) VI - 70 IP - 5 DP - 2025 Sep TI - DIEP breast reconstruction in a patient with an unknown Raynaud's phenomenon: A reflection on microvascular disorders and free flaps. PG - 361-365 LID - S0294-1260(25)00038-X [pii] LID - 10.1016/j.anplas.2025.03.008 [doi] AB - Free flap reconstructions are the gold standard in autologous breast reconstruction. Although free flap reconstruction in a patient with Raynaud's phenomenon is not a contraindication, this phenomenon must be recognized by the surgeon and all the necessary measures taken. If the surgeon is aware of this particularity, he will be prepared to take the necessary measures in the immediate postoperative period. The presence of Raynaud's phenomenon can influence the immediate postoperative evolution of a free flap by making it difficult to monitor. We present a case of a 50-year-old woman with an unknown Raynaud's phenomenon who underwent breast reconstruction using a free DIEP flap. She was referred to our department where a one-stage reconstruction was performed using a free deep inferior epigastric artery perforator (DIEP) flap. The postoperative course of the flap progressed from pale to cyanotic to mottled, an evocative appearance for Raynaud's phenomenon. At the three-month follow-up evaluation, the aesthetic result was satisfactory, despite a worrying postoperative appearance. Good outcomes are possible by using appropriate methods in the postoperative period, even if the clinical evolution of the flap is atypical. By taking into account and specifically treating Raynaud's phenomenon, the flap was preserved with an usual result. CI - Copyright © 2025 Elsevier Masson SAS. All rights reserved. FAU - Gherle, B AU - Gherle B AD - Doctoral School of Biological and Biomedical Sciences, University of Oradea, 1 University Street, 410081 Oradea, Romania; Department of Plastic, Reconstructive and Aesthetic Surgery, Rangueil University Hospital, 1, avenue du Pr-Jean-Poulhes, 31400 Toulouse, France. Electronic address: gherlebogdandaniel@gmail.com. FAU - Gandolfi, S AU - Gandolfi S AD - Department of Plastic, Reconstructive and Aesthetic Surgery, Rangueil University Hospital, 1, avenue du Pr-Jean-Poulhes, 31400 Toulouse, France. FAU - Commenge, V AU - Commenge V AD - Department of Plastic, Reconstructive and Aesthetic Surgery, Rangueil University Hospital, 1, avenue du Pr-Jean-Poulhes, 31400 Toulouse, France. FAU - Mohan, A G AU - Mohan AG AD - Faculty of Medicine and Pharmacy, University of Oradea, 10 P-ta. 1 Decembrie Street, RO 410073 Oradea, Romania. FAU - Chaput, B AU - Chaput B AD - Department of Plastic, Reconstructive and Aesthetic Surgery, Rangueil University Hospital, 1, avenue du Pr-Jean-Poulhes, 31400 Toulouse, France. FAU - Meresse, T AU - Meresse T AD - Department of Plastic, Reconstructive and Aesthetic Surgery, Rangueil University Hospital, 1, avenue du Pr-Jean-Poulhes, 31400 Toulouse, France; Department of Plastic and Reconstructive Surgery, Institut Universitaire du Cancer Toulouse Oncopole, Toulouse, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20250527 PL - France TA - Ann Chir Plast Esthet JT - Annales de chirurgie plastique et esthetique JID - 8305839 SB - IM MH - Humans MH - Female MH - *Mammaplasty/methods MH - Middle Aged MH - *Raynaud Disease/complications MH - *Perforator Flap/blood supply MH - *Free Tissue Flaps/blood supply MH - *Epigastric Arteries/transplantation MH - Breast Neoplasms/surgery/complications OTO - NOTNLM OT - Autologous free flap breast reconstruction OT - Breast reconstruction OT - DIEP OT - Free flap in breast reconstruction OT - Lambeau libre en reconstruction mammaire OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Reconstruction mammaire OT - Reconstruction mammaire autologue par lambeau libre COIS- Disclosure of interest The authors declare that they have no competing interest. EDAT- 2025/05/29 00:27 MHDA- 2025/09/02 00:44 CRDT- 2025/05/28 21:56 PHST- 2025/01/29 00:00 [received] PHST- 2025/03/23 00:00 [revised] PHST- 2025/03/26 00:00 [accepted] PHST- 2025/09/02 00:44 [medline] PHST- 2025/05/29 00:27 [pubmed] PHST- 2025/05/28 21:56 [entrez] AID - S0294-1260(25)00038-X [pii] AID - 10.1016/j.anplas.2025.03.008 [doi] PST - ppublish SO - Ann Chir Plast Esthet. 2025 Sep;70(5):361-365. doi: 10.1016/j.anplas.2025.03.008. Epub 2025 May 27. PMID- 31573670 OWN - NLM STAT- MEDLINE DCOM- 20191202 LR - 20191202 IS - 1652-7518 (Electronic) IS - 0023-7205 (Linking) VI - 116 DP - 2019 Sep 26 TI - [Systemic sclerosis - a rare but important diagnosis in primary health care]. LID - FPL6 [pii] AB - Systemic sclerosis is an autoimmune systemic disease with an annual incidence in Sweden of only 20 cases per million and a standardised mortality rate of 3-4. Disease onset is usually preceded by a period with Raynaud's phenomenon, combined with structurally abnormal nailbed capillaries and accompanied by presence of scleroderma related autoantibodies. The presenting symptoms are skin thickness, puffy fingers, digital ulcers, dysphagia, joint stiffness and pain, and pruritus. Optimal management involves a number of specialists including allied health professionals. Early recognition, diagnosis and treatment are important. The dominating causes of death are cardiopulmonary. FAU - Andreasson, Kristofer AU - Andreasson K AD - Lunds Universitet - Institutionen för kliniska vetenskaper, Avdelning för reumatologi Lund, Sweden Department of Clinical Sciences Rheumatology Lund - Rheumatology Lund, Sweden. FAU - Lillpers, Kerstin AU - Lillpers K AD - Lunds Universitet - Institutionen för kliniska vetenskaper, Avdelning för reumatologi Lund, Sweden Lund University - Department of Clinical Sciences, Section of Rheumatology Lund, Sweden. FAU - Wollheim, Frank AU - Wollheim F AD - Lunds Universitet - Institutionen för kliniska vetenskaper, Avdelning för reumatologi Lund, Sweden Department of Clinical Sciences Rheumatology Lund - Rheumatology Lund, Sweden. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Lunds Universitet Institutionen for kliniska vetenskaper i Lund - Lund, Sweden Lunds Universitet Institutionen for kliniska vetenskaper i Lund - Lund, Sweden. LA - swe PT - Journal Article PT - Review TT - Systemisk skleros – en ovanlig men viktig diagnos i primärvården. DEP - 20190926 PL - Sweden TA - Lakartidningen JT - Lakartidningen JID - 0027707 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/immunology MH - Humans MH - Primary Health Care MH - Rare Diseases/complications/diagnosis/pathology/therapy MH - Raynaud Disease/etiology MH - Referral and Consultation MH - *Scleroderma, Systemic/complications/diagnosis/pathology/therapy EDAT- 2019/10/02 06:00 MHDA- 2019/12/04 06:00 CRDT- 2019/10/02 06:00 PHST- 2019/10/02 06:00 [entrez] PHST- 2019/10/02 06:00 [pubmed] PHST- 2019/12/04 06:00 [medline] AID - FPL6 [pii] PST - epublish SO - Lakartidningen. 2019 Sep 26;116:FPL6. PMID- 19330378 OWN - NLM STAT- MEDLINE DCOM- 20090708 LR - 20211020 IS - 1432-0991 (Electronic) IS - 0343-8651 (Linking) VI - 59 IP - 1 DP - 2009 Jul TI - Bacterial analysis of breast milk: a tool to differentiate Raynaud's phenomenon from infectious mastitis during lactation. PG - 59-64 LID - 10.1007/s00284-009-9393-z [doi] AB - Lactational Raynaud's syndrome may be misdiagnosed as infectious mastitis on the basis of the breast pain. The objective of this work was to elucidate if microbiological analysis of milk may contribute to the differentiation of both conditions. Ten lactating women clinically diagnosed by Spanish lactation consultants were included in the study. Of these, five suffered from mastitis and the remaining five suffered from Raynaud's syndrome. Breast milk samples were inoculated on diverse culture media. Seventy isolates were selected and identified by 16S rDNA PCR sequencing. Parallel, PCR-DGGE and quantitative real-time PCR were used to assess the presence of bacterial DNA in the samples. Neither bacteria nor yeasts could be detected in the milk samples provided by the women suffering from Raynaud's syndrome. In contrast, large numbers of bacteria were isolated from those with infectious lactational mastitis. Globally, the levels of bacterial DNA were significantly higher in the milk of mastitis-suffering women. Bacteriological analysis of milk can be an useful tool to facilitate the differential diagnosis between the infectious mastitis and Raynaud's syndrome during lactation. FAU - Delgado, Susana AU - Delgado S AD - Nutrición, Bromatología y Tecnología de los Alimentos, Universidad Complutense de Madrid, Madrid, Spain. sdelgado@vet.ucm.es FAU - Collado, M Carmen AU - Collado MC FAU - Fernández, Leonides AU - Fernández L FAU - Rodríguez, Juan M AU - Rodríguez JM LA - eng SI - GENBANK/FM992866 SI - GENBANK/FM992867 SI - GENBANK/FM992868 SI - GENBANK/FM992869 SI - GENBANK/FM992870 PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090328 PL - United States TA - Curr Microbiol JT - Current microbiology JID - 7808448 RN - 0 (DNA, Bacterial) RN - 0 (DNA, Ribosomal) RN - 0 (RNA, Ribosomal, 16S) SB - IM MH - Adult MH - Bacteria/classification/genetics/*isolation & purification MH - DNA, Bacterial/genetics MH - DNA, Ribosomal/genetics MH - Female MH - Humans MH - *Lactation MH - Mastitis/*microbiology MH - Milk, Human/*microbiology MH - Molecular Sequence Data MH - RNA, Ribosomal, 16S/genetics MH - Raynaud Disease/*microbiology MH - Young Adult EDAT- 2009/03/31 09:00 MHDA- 2009/07/09 09:00 CRDT- 2009/03/31 09:00 PHST- 2008/12/16 00:00 [received] PHST- 2009/02/25 00:00 [accepted] PHST- 2009/02/19 00:00 [revised] PHST- 2009/03/31 09:00 [entrez] PHST- 2009/03/31 09:00 [pubmed] PHST- 2009/07/09 09:00 [medline] AID - 10.1007/s00284-009-9393-z [doi] PST - ppublish SO - Curr Microbiol. 2009 Jul;59(1):59-64. doi: 10.1007/s00284-009-9393-z. Epub 2009 Mar 28. PMID- 32942034 OWN - NLM STAT- MEDLINE DCOM- 20210106 LR - 20210106 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 19 IP - 11 DP - 2020 Nov TI - Undifferentiated connective tissue disease at risk for systemic sclerosis: Which patients might be labeled prescleroderma? PG - 102659 LID - S1568-9972(20)30234-2 [pii] LID - 10.1016/j.autrev.2020.102659 [doi] AB - Undifferentiated Connective Tissue Disease at risk for Systemic Sclerosis (UCTD-risk-SSc), otherwise referred to as very early-early SSc (very early-early diagnosis of systemic sclerosis VEDOSS), is a condition characterized by Raynaud's phenomenon (RP) and either SSc serum marker autoantibodies or a capillaroscopic scleroderma pattern or both, but without satisfying classification criteria for SSc neither features consistent with SSc sine scleroderma. Approximately half the UCTD-risk-SSc patients develop definite SSc over 5-10 years of follow-up. Identifying patients who will undergo such evolution is an unmet need. Predicting at onset which patients with RP are going to develop SSc over time has long been a research objective and still is an unaccomplished task. The present review is devoted to the critical analysis of the nosographic boundaries of the condition and of items predictive of evolution including serological, capillaroscopic and circulating markers. A weighted score, based on serum antinuclear antibody titre, serum marker antibodies positivity and avascular areas has been developed and may identify in the meanwhile patients to be labeled prescleroderma i.e. those probably developing SSc over time. Future research should be directed to investigate unexplored features, validate and improve the performance of the score and highlight the involved pathways to be contrasted in order to identify a targeted therapy hampering the development of overt SSc. CI - Copyright © 2020 Elsevier B.V. All rights reserved. FAU - Valentini, Gabriele AU - Valentini G AD - Università degli Studi della Campania "Luigi Vanvitelli", Department of Precision Medicine, Section of Rheumatology, Italy. Electronic address: gabrielevalentini981@gmail.com. FAU - Pope, Janet E AU - Pope JE AD - Università degli Studi della Campania "Luigi Vanvitelli", Department of Precision Medicine, Section of Rheumatology, Italy; Schulich School of Medicine and Dentistry, University of Western Ontario, Division of Rheumatology, St. Joseph's Health Care, London, Ontario, Canada. LA - eng PT - Journal Article PT - Review DEP - 20200914 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Systemic/diagnosis/etiology MH - *Undifferentiated Connective Tissue Diseases/complications/diagnosis COIS- Declaration of Competing Interest GV has consulted for Abbvie, BMS, Boehringer, MSD, Pfizer, Sanophi, but the opinions expressed are solely his JEP consults for several pharmaceutical companies with potential treatment in scleroderma (Actelion, Bayer, BI, BMS, Celgene, Emerald Health Pharmaceuticals, EICOS Sciences, Janssen, Merck, Roche, Pfizer, Seattle Genetics) but the opinions expressed are solely hers. IRB was not needed for this manuscript. EDAT- 2020/09/18 06:00 MHDA- 2021/01/07 06:00 CRDT- 2020/09/17 20:13 PHST- 2020/04/06 00:00 [received] PHST- 2020/04/12 00:00 [accepted] PHST- 2020/09/18 06:00 [pubmed] PHST- 2021/01/07 06:00 [medline] PHST- 2020/09/17 20:13 [entrez] AID - S1568-9972(20)30234-2 [pii] AID - 10.1016/j.autrev.2020.102659 [doi] PST - ppublish SO - Autoimmun Rev. 2020 Nov;19(11):102659. doi: 10.1016/j.autrev.2020.102659. Epub 2020 Sep 14. PMID- 40999744 OWN - NLM STAT- MEDLINE DCOM- 20260122 LR - 20260125 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 78 IP - 1 DP - 2026 Jan TI - Rethinking Strategies for a Pharmaceutical Approach to Pain Related to Connective Tissue-Related Raynaud Phenomenon in the United States. PG - 106-112 LID - 10.1002/acr.25660 [doi] AB - OBJECTIVE: There are no US Food and Drug Administration-approved therapies for Raynaud phenomenon (RP) in the United States. Clinical trials have been challenged by study design. Important advances in RP patient-reported outcome measures and mechanistic quantification allow RP-related pain characterization. The rationale for this narrative review is current RP treatment guidelines that focus on vasodilation. METHODS: The question of why there are limitations to RP treatment in the United States is addressed through a comprehensive search strategy of published RP treatment guidelines up until September 1, 2025. Search databases included Medline (PubMed), Embase, and Scopus for the index terms "Raynaud's phenomenon treatment guidelines." If a society guideline was updated, only the most recent was included. Eligibility, data extraction, risk of bias, and quality assessment were subject to review by two independent reviewers, with a third reviewer resolving discrepancies. US-specific considerations of published guidelines are reviewed. RESULTS: A total of 118 published articles were identified by the search terms "Raynaud's phenomenon treatment guidelines," and 27 abstracts were reviewed. Four articles published as RP treatment recommendations or guidelines were reviewed for full content. Pain management for RP is not included in guideline-based care. CONCLUSION: There are advances in outcome measures for quantifying pain now available for RP clinical trials. Large US-based registries for systemic sclerosis using patient-reported outcomes can allow serial data collection on RP and RP-related digital lesions to provide real-world data on medication efficacy for pain relief. CI - © 2025 The Author(s). Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Vanderbilt University Medical Center and Tennessee Valley Healthcare System, Nashville, Tennessee. FAU - Frech, Charles G AU - Frech CG AD - Vanderbilt University, Nashville, Tennessee. FAU - Merryman, W David AU - Merryman WD AD - Vanderbilt University, Nashville, Tennessee. FAU - Sternlicht, Andrew AU - Sternlicht A AD - Tufts University School of Medicine, Boston, Massachusetts. FAU - Baba, Justin AU - Baba J AD - Vanderbilt University, Nashville, Tennessee. LA - eng GR - I01 CX002111/CX/CSRD VA/United States GR - 5I01CX002111-03/U.S. Department of Veterans Affairs/ PT - Journal Article PT - Review DEP - 20251212 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Humans MH - *Raynaud Disease/drug therapy/complications/diagnosis MH - United States MH - *Pain/drug therapy/etiology/diagnosis MH - Practice Guidelines as Topic MH - *Connective Tissue Diseases/complications/drug therapy/diagnosis PMC - PMC12826088 EDAT- 2025/09/26 06:31 MHDA- 2026/01/22 13:30 PMCR- 2026/01/22 CRDT- 2025/09/26 02:23 PHST- 2025/09/05 00:00 [revised] PHST- 2025/07/08 00:00 [received] PHST- 2025/09/16 00:00 [accepted] PHST- 2026/01/22 13:30 [medline] PHST- 2025/09/26 06:31 [pubmed] PHST- 2025/09/26 02:23 [entrez] PHST- 2026/01/22 00:00 [pmc-release] AID - ACR25660 [pii] AID - 10.1002/acr.25660 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2026 Jan;78(1):106-112. doi: 10.1002/acr.25660. Epub 2025 Dec 12. PMID- 16292469 OWN - NLM STAT- MEDLINE DCOM- 20061107 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 4 DP - 2006 Jul TI - Raynaud's phenomenon: prevalence in an Italian population sample. PG - 506-10 AB - To estimate the prevalence of Raynaud's phenomenon (RP) in an Italian population aged 18 years and above, a validated questionnaire was mailed to a random sample of 3,664 subjects. Sociodemographic data and data regarding the prevalence of RP symptoms were collected. Trained rheumatologists carried out a subsequent standardized clinical examination to confirm diagnosis of RP and to detect associated diseases. The subjects (2,155) completed the questionnaire, with 45 reporting RP [prevalence rate 2.1%, 95% confidence intervals (CI) 1.8-2.4]. The mean age [standard deviation (SD)] was 57.5 (12.7), and 40 (88.8%) were women. Prevalence was found higher in women (3.4%, 95% CI 2.8-4.1) than in men (0.5%, 95% CI 0.2-0.9) (p<0.0001). A connective tissue disease was diagnosed in six (0.28%) subjects, three of the participants (0.14%) were identified as having RP associated to an idiopathic carpal tunnel syndrome, and 36 (1.67%) were diagnosed as having primary RP. Our study has found a lower prevalence of RP than others have reported. There is thought to be an association with climate and ethnic factors. No relationship was found regarding factors that are thought to be among the most relevant triggering factors for RP, e.g., occupational exposure. Differences with previous studies may be related to the current characteristics of the population surveyed. FAU - De Angelis, Rossella AU - De Angelis R AD - Clinica Reumatologica, Ospedale "Augusto Murri", via dei Colli, 52, 60035, Jesi, (AN), Italy. rossella.deangelis@asl5.marche.it FAU - Salaffi, Fausto AU - Salaffi F FAU - Grassi, Walter AU - Grassi W LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20051115 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Aged MH - Demography MH - Female MH - Humans MH - Italy/epidemiology MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/*epidemiology MH - Risk Factors MH - *Surveys and Questionnaires EDAT- 2005/11/18 09:00 MHDA- 2006/11/09 09:00 CRDT- 2005/11/18 09:00 PHST- 2005/06/13 00:00 [received] PHST- 2005/07/06 00:00 [accepted] PHST- 2005/07/01 00:00 [revised] PHST- 2005/11/18 09:00 [pubmed] PHST- 2006/11/09 09:00 [medline] PHST- 2005/11/18 09:00 [entrez] AID - 10.1007/s10067-005-0077-1 [doi] PST - ppublish SO - Clin Rheumatol. 2006 Jul;25(4):506-10. doi: 10.1007/s10067-005-0077-1. Epub 2005 Nov 15. PMID- 31264893 OWN - NLM STAT- MEDLINE DCOM- 20200820 LR - 20200820 IS - 1557-8992 (Electronic) IS - 1044-5463 (Linking) VI - 29 IP - 8 DP - 2019 Oct TI - Raynaud's Phenomenon Related with Atomoxetine Treatment in a Child with Autism and Attention-Deficit/Hyperactivity Disorder. PG - 649-650 LID - 10.1089/cap.2019.0025 [doi] FAU - Gülle, Zeynep Nur AU - Gülle ZN AD - Department of Child and Adolescent Psychiatry, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. FAU - Karayagmurlu, Ali AU - Karayagmurlu A AD - Department of Child and Adolescent Psychiatry, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. FAU - Coskun, Murat AU - Coskun M AD - Department of Child and Adolescent Psychiatry, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. LA - eng PT - Letter DEP - 20190702 PL - United States TA - J Child Adolesc Psychopharmacol JT - Journal of child and adolescent psychopharmacology JID - 9105358 RN - 0 (Adrenergic Uptake Inhibitors) RN - 57WVB6I2W0 (Atomoxetine Hydrochloride) SB - IM MH - Adrenergic Uptake Inhibitors/*therapeutic use MH - Atomoxetine Hydrochloride/*therapeutic use MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Autistic Disorder/*drug therapy MH - Child MH - Drug-Related Side Effects and Adverse Reactions MH - Humans MH - Male MH - Problem Behavior MH - *Raynaud Disease EDAT- 2019/07/03 06:00 MHDA- 2020/08/21 06:00 CRDT- 2019/07/03 06:00 PHST- 2019/07/03 06:00 [pubmed] PHST- 2020/08/21 06:00 [medline] PHST- 2019/07/03 06:00 [entrez] AID - 10.1089/cap.2019.0025 [doi] PST - ppublish SO - J Child Adolesc Psychopharmacol. 2019 Oct;29(8):649-650. doi: 10.1089/cap.2019.0025. Epub 2019 Jul 2. PMID- 19585167 OWN - NLM STAT- MEDLINE DCOM- 20100119 LR - 20211020 IS - 1619-1560 (Electronic) IS - 0959-9851 (Linking) VI - 19 IP - 6 DP - 2009 Dec TI - Sympathetic skin response in primary Raynaud's phenomenon. PG - 355-62 LID - 10.1007/s10286-009-0021-6 [doi] AB - OBJECTIVES: The pathogenetic hypotheses of Raynaud's phenomenon include increased activation of sympathetic noradrenergic nerves controlling muscle tone of digit arteriolar walls. Because acral sympathetic fibres contain vasoactive adrenergic and cholinergic fibres for sweat glands, we tested cholinergic sympathetic fibre function in primary Raynaud's phenomenon (PRP) patients by sympathetic skin response (SSR). METHODS: Twenty-six consecutive patients (19 women, 7 men, mean age 37.8 years) with PRP were enroled prospectively. SSR was obtained by random electrical stimulation of the left ulnar nerve at the wrist recording from the palm (PSSR), third (M3SSR) and fifth fingers (U5SSR) on the right side. For each subject latency of shortest response, area of largest response and grand mean latencies and areas of 12 consecutive responses were calculated. The differences between patients and a control group (15 women, 6 men, mean age 38.9 years) were calculated. SSR habituation was also compared between patients and controls. RESULTS: PSSRs were recorded in all patients and no difference in any PSSR parameter was found between patients and controls. U5SSRs and M3SSRs were absent in two patients. Grand mean area and mean of largest M3SSRs and U5SSRs were significantly lower in patients than in controls. Grand mean latency and mean of shortest M3SSRs and U5SSRs were significantly slower in patients than in controls. M3 and U5SSRs habituated less in patients than in controls. INTERPRETATION: Dysregulation of cholinergic sympathetic fibres innervating the fingers was found in PRP. Abnormal peripheral mechanisms may be the cause. Since SSR habituation was also not normal, even central mechanisms may be implicated. FAU - Mondelli, Mauro AU - Mondelli M AD - EMG Service, Local Health Unit, Siena, Italy. m.mondelli@usl7.toscana.it FAU - de Stefano, Renato AU - de Stefano R FAU - Rossi, Stefania AU - Rossi S FAU - Aretini, Alessandro AU - Aretini A FAU - Romano, Clara AU - Romano C LA - eng PT - Journal Article DEP - 20090708 PL - Germany TA - Clin Auton Res JT - Clinical autonomic research : official journal of the Clinical Autonomic Research Society JID - 9106549 SB - IM MH - Adolescent MH - Adrenergic Fibers/*physiology MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Neural Conduction/*physiology MH - Prospective Studies MH - Raynaud Disease/*physiopathology MH - Skin/innervation/*physiopathology MH - Sympathetic Nervous System/*physiopathology MH - Young Adult EDAT- 2009/07/09 09:00 MHDA- 2010/01/20 06:00 CRDT- 2009/07/09 09:00 PHST- 2008/12/12 00:00 [received] PHST- 2009/06/11 00:00 [accepted] PHST- 2009/07/09 09:00 [entrez] PHST- 2009/07/09 09:00 [pubmed] PHST- 2010/01/20 06:00 [medline] AID - 10.1007/s10286-009-0021-6 [doi] PST - ppublish SO - Clin Auton Res. 2009 Dec;19(6):355-62. doi: 10.1007/s10286-009-0021-6. Epub 2009 Jul 8. PMID- 16647656 OWN - NLM STAT- MEDLINE DCOM- 20060630 LR - 20060501 IS - 1526-0046 (Print) IS - 1526-0046 (Linking) VI - 5 IP - 2 DP - 2006 TI - Rheumatic diseases that can be confused with work-related upper extremity disorders. PG - 397-405, ix AB - Rheumatic illnesses are a common cause for musculoskeletal complaints in the general population. All ages can be affected, including people in the prime of their working years. Secondary problems, such as entrapment neuropathies, enthesopathies, and Raynaud's syndrome, can be associated with various inflammatory arthritides. A detailed history and physical are the most important tools in screening for potential inflammatory disease in workers with upper extremity complaints. FAU - June, Lisa AU - June L AD - Rheumatology Associates, 4213 Maryknoll Lane, Louisville, KY 40207, USA. lisajunemd@hotmail.com LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Occup Environ Med JT - Clinics in occupational and environmental medicine JID - 100893239 SB - IM MH - Adult MH - Age Distribution MH - Aged MH - Arm Injuries/*diagnosis/epidemiology/therapy MH - Carpal Tunnel Syndrome/diagnosis MH - Causality MH - Cost of Illness MH - Cumulative Trauma Disorders/*diagnosis/epidemiology/therapy MH - Diagnosis, Differential MH - Fibromyalgia/diagnosis MH - Humans MH - Joint Instability/diagnosis MH - Mass Screening MH - Medical History Taking MH - Middle Aged MH - Occupational Diseases/*diagnosis/epidemiology/therapy MH - Occupational Health MH - Occupational Medicine MH - Physical Examination MH - Prevalence MH - Raynaud Disease/diagnosis MH - Rheumatic Diseases/*diagnosis/epidemiology/therapy MH - United States/epidemiology RF - 44 EDAT- 2006/05/02 09:00 MHDA- 2006/07/01 09:00 CRDT- 2006/05/02 09:00 PHST- 2006/05/02 09:00 [pubmed] PHST- 2006/07/01 09:00 [medline] PHST- 2006/05/02 09:00 [entrez] AID - S1526-0046(05)00024-5 [pii] AID - 10.1016/j.coem.2005.11.008 [doi] PST - ppublish SO - Clin Occup Environ Med. 2006;5(2):397-405, ix. doi: 10.1016/j.coem.2005.11.008. PMID- 32407896 OWN - NLM STAT- MEDLINE DCOM- 20201023 LR - 20201023 IS - 1879-3649 (Electronic) IS - 1537-1891 (Linking) VI - 131 DP - 2020 Aug TI - Estrogen increases expression of vascular alpha 2C adrenoceptor through the cAMP/Epac/JNK/AP-1 pathway and potentiates cold-induced vasoconstriction. PG - 106690 LID - S1537-1891(19)30287-3 [pii] LID - 10.1016/j.vph.2020.106690 [doi] AB - Cutaneous cold-induced vasoconstriction is a normal physiological reaction mediated by alpha 2C-adrenergic receptors (α(2C)-ARs) expressed in vascular smooth muscle cells (VSMCs). When this reaction is exaggerated, Raynaud's phenomenon (RP) ensues. RP is more prevalent in females compared to age-matched men. We previously established that 17-β estradiol (estrogen) upregulates α(2C)-ARs in human VSMCs via a cAMP/Epac/Rap pathway. We also showed that cAMP acts through JNK to increase α(2C)-AR expression. However, whether estrogen employs JNK to regulate α(2C)-AR is not investigated. Knowing that the α(2C)-AR promoter harbors an activator protein-1 (AP-1) binding site that can be potentially activated by JNK, we hypothesized that estrogen regulates α(2C-)AR expression through an Epac/JNK/AP-1 pathway. Our results show that estrogen (10(-10) M) activated JNK in human VSMCs extracted from cutaneous arterioles. Pretreatment with ESI09 (10 μM; an Epac inhibitor), abolished estrogen-induced JNK activation. In addition, pre-treatment with SP600125 (3 μM; a JNK specific inhibitor) abolished estrogen-induced expression of α(2C)-AR. Importantly, estrogen-induced activation of α(2C)-AR promoter was attenuated with SP600125. Moreover, transient transfection of VSMCs with an Epac dominant negative mutant (Epac-DN) abolished estrogen-induced activation of α(2C)-AR promoter. However, co-transfection of constitutively active JNK mutant overrode the inhibitory effect of Epac-DN on α(2C)-AR promoter. Moreover, estrogen caused a concentration-dependent increase in the activity of AP-1-driven reporter construct. Mutation of AP-1 site in the α(2C)-AR promoter abolished its activation by estrogen. This in vitro estrogen-increased α(2C)-AR expression was mirrored by an increase in the ex vivo functional responsiveness of arterioles. Indeed, estrogen potentiated α(2C)-AR-mediated cold-induced vasoconstriction, which was abolished by SP600125. Collectively, these results indicate that estrogen upregulates α(2C)-AR expression via an EPAC-mediated JNK/AP-1- dependent mechanism. These results provide an insight into the mechanism by which exaggerated cold-induced vasoconstriction occurs in estrogen-replete females and identify Epac and JNK as potential targets for the treatment of RP. CI - Copyright © 2020. Published by Elsevier Inc. FAU - Fardoun, Manal M AU - Fardoun MM AD - Department of Biology, American University of Beirut, Beirut, Lebanon. FAU - Issa, Khodr AU - Issa K AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - Maaliki, Dina AU - Maaliki D AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - Nasser, Suzanne A AU - Nasser SA AD - Department of Pharmacology and Therapeutics, Beirut Arab University, Beirut, Lebanon. FAU - Baydoun, Elias AU - Baydoun E AD - Department of Biology, American University of Beirut, Beirut, Lebanon. FAU - Eid, Ali H AU - Eid AH AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon; Department of Biomedical Sciences, Qatar University, Doha, Qatar. Electronic address: ae81@aub.edu.lb. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200511 PL - United States TA - Vascul Pharmacol JT - Vascular pharmacology JID - 101130615 RN - 0 (ADRA2C protein, human) RN - 0 (Guanine Nucleotide Exchange Factors) RN - 0 (RAPGEF3 protein, human) RN - 0 (Receptors, Adrenergic, alpha-2) RN - 0 (Transcription Factor AP-1) RN - 4TI98Z838E (Estradiol) RN - E0399OZS9N (Cyclic AMP) RN - EC 2.7.11.24 (JNK Mitogen-Activated Protein Kinases) SB - IM MH - Animals MH - Arterioles/drug effects/enzymology MH - Cells, Cultured MH - *Cold Temperature MH - Cyclic AMP/*metabolism MH - Estradiol/*pharmacology MH - Guanine Nucleotide Exchange Factors/genetics/*metabolism MH - Humans MH - JNK Mitogen-Activated Protein Kinases/*metabolism MH - Male MH - Mice, Inbred C57BL MH - Muscle, Smooth, Vascular/*drug effects/enzymology MH - Myocytes, Smooth Muscle/*drug effects/enzymology MH - Raynaud Disease/drug therapy/enzymology/physiopathology MH - Receptors, Adrenergic, alpha-2/genetics/*metabolism MH - Signal Transduction MH - Tail/*blood supply MH - Transcription Factor AP-1/genetics/*metabolism MH - Up-Regulation MH - Vasoconstriction/*drug effects OTO - NOTNLM OT - AP-1 OT - Estrogen OT - JNK OT - Raynaud's phenomenon OT - Vasoconstriction OT - α(2C)-adrenergic receptor COIS- Declaration of Competing Interest The authors declare that they have no conflicts of interest. EDAT- 2020/05/15 06:00 MHDA- 2020/10/24 06:00 CRDT- 2020/05/15 06:00 PHST- 2019/10/03 00:00 [received] PHST- 2020/05/09 00:00 [revised] PHST- 2020/05/10 00:00 [accepted] PHST- 2020/05/15 06:00 [pubmed] PHST- 2020/10/24 06:00 [medline] PHST- 2020/05/15 06:00 [entrez] AID - S1537-1891(19)30287-3 [pii] AID - 10.1016/j.vph.2020.106690 [doi] PST - ppublish SO - Vascul Pharmacol. 2020 Aug;131:106690. doi: 10.1016/j.vph.2020.106690. Epub 2020 May 11. PMID- 39103464 OWN - NLM STAT- MEDLINE DCOM- 20240805 LR - 20240825 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 14 IP - 1 DP - 2024 Aug 5 TI - Serum levels of biomarkers related to severity staging of Raynaud's phenomenon, neurosensory manifestations, and vibration exposure in patients with hand-arm vibration injury. PG - 18128 LID - 10.1038/s41598-024-68846-1 [doi] LID - 18128 AB - Our aim was to explore possible relationships between serum levels of biomarkers in patients with hand-arm vibration injury in relation to the severity of the vascular, i.e., Raynaud's phenomenon (RP), and neurosensory manifestations, the current exposure level, and the duration of exposure. This study was of case series design and involved 92 patients diagnosed with hand-arm vibration injury. Jonckheere's trend test was used to assess any association between serum levels of biomarkers and RP as well as neurosensory manifestations, graded by the International Consensus Criteria. Generalized linear models with adjustment for possible confounders were also used for associations between serum levels of biomarkers and; (1) severity of RP recorded as the extent of finger blanching calculated with Griffin score, (2) vibration perception thresholds, (3) magnitude of current exposure as [A(8); (m/s(2))] value, and (4) the duration of exposure in years. Serum levels of thrombomodulin, von Willebrand factor, calcitonin gene related peptide (CGRP), heat shock protein 27, and caspase-3 were positively associated with severity of RP. Serum levels of CGRP were positively associated with the neurosensory component. No associations with exposure were shown for these biomarkers. For Intercellular adhesion molecule 1 and monocyte chemoattractant protein 1, no associations were found with neither severity nor exposure. Levels of serum biomarkers associated with endothelial injury or dysfunction, inflammation, vasodilation, neuroprotection, and apoptosis were positively associated with the severity of hand-arm vibration injury. CI - © 2024. The Author(s). FAU - Tekavec, Eva AU - Tekavec E AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. eva.tekavec@med.lu.se. FAU - Nilsson, Tohr AU - Nilsson T AD - Division of Sustainable Health and Medicine, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Dahlin, Lars B AU - Dahlin LB AD - Department of Translational Medicine - Hand Surgery, Lund University, 221 00, Lund, Sweden. FAU - Huynh, Elizabeth AU - Huynh E AD - Department of Occupational and Environmental Medicine, Region Skåne, 223 63, Lund, Sweden. FAU - Nordander, Catarina AU - Nordander C AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. FAU - Riddar, Jakob AU - Riddar J AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. FAU - Kåredal, Monica AU - Kåredal M AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. AD - Department of Occupational and Environmental Medicine, Region Skåne, 223 63, Lund, Sweden. LA - eng GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 170124/AFA Insurance/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - 2022-01942/Swedish Research Council/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ GR - DIA2020-492/The Swedish Diabetes Foundation/ PT - Journal Article DEP - 20240805 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Biomarkers) RN - 0 (von Willebrand Factor) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Humans MH - *Raynaud Disease/blood/diagnosis MH - *Biomarkers/blood MH - Male MH - Female MH - Middle Aged MH - *Vibration/adverse effects MH - Adult MH - *Hand-Arm Vibration Syndrome/blood/diagnosis MH - Severity of Illness Index MH - von Willebrand Factor/metabolism/analysis MH - Calcitonin Gene-Related Peptide/blood MH - Aged PMC - PMC11300662 OTO - NOTNLM OT - Aβ fibers OT - Aδ fibers OT - C-fibers OT - Cold intolerance OT - Endothelial dysfunction OT - Grading of injury OT - Hand-arm vibration syndrome (HAVS) OT - Neuroprotection OT - Occupational OT - Serum biomarkers OT - Vibration exposure COIS- The authors declare no competing interests. EDAT- 2024/08/06 00:42 MHDA- 2024/08/06 00:43 PMCR- 2024/08/05 CRDT- 2024/08/05 23:22 PHST- 2024/03/12 00:00 [received] PHST- 2024/07/29 00:00 [accepted] PHST- 2024/08/06 00:43 [medline] PHST- 2024/08/06 00:42 [pubmed] PHST- 2024/08/05 23:22 [entrez] PHST- 2024/08/05 00:00 [pmc-release] AID - 10.1038/s41598-024-68846-1 [pii] AID - 68846 [pii] AID - 10.1038/s41598-024-68846-1 [doi] PST - epublish SO - Sci Rep. 2024 Aug 5;14(1):18128. doi: 10.1038/s41598-024-68846-1. PMID- 29569858 OWN - NLM STAT- MEDLINE DCOM- 20190722 LR - 20190901 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 70 IP - 9 DP - 2018 Sep TI - An Autoimmune Basis for Raynaud's Phenomenon: Murine Model and Human Disease. PG - 1489-1499 LID - 10.1002/art.40505 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) is common in anti-RNP-positive patients with rheumatic diseases but is not itself known to be caused by autoimmunity. The aim of this study was to assess autoantibodies that could mediate this process. METHODS: Antibodies derived from patient sera and from murine models of anti-RNP autoimmunity were screened for the ability to induce RP-like tissue ischemia and endothelial cell apoptosis in murine models and in vitro systems. RESULTS: RNP-positive sera from RP patients and murine sera from RNP-positive B cell adoptive transfer recipients induced RP-like tissue ischemia and endothelial cell apoptosis. Proteomic analysis identified cytokeratin 10 (K10) as a candidate autoantigen in RP. Monoclonal anti-K10 antibodies reproduced patterns of ischemic tissue loss and endothelial cell apoptosis; K10 knockout or depletion of anti-K10 activity in serum was protective. Cold exposure enhanced K10 expression and in vivo tissue loss. CONCLUSION: Anti-K10 antibodies are sufficient to mediate RP-like ischemia in murine models and are implicated in the pathogenesis of RP in patients with anti-RNP autoimmunity. CI - © 2018, American College of Rheumatology. FAU - Ascherman, D P AU - Ascherman DP AD - Miami VA Medical Center and University of Miami Miller School of Medicine, Miami, Florida. FAU - Zang, Y AU - Zang Y AD - University of Miami Miller School of Medicine, Miami, Florida. FAU - Fernandez, I AU - Fernandez I AD - University of Miami Miller School of Medicine, Miami, Florida. FAU - Clark, E S AU - Clark ES AD - University of Miami Miller School of Medicine, Miami, Florida. FAU - Khan, W N AU - Khan WN AD - University of Miami Miller School of Medicine, Miami, Florida. FAU - Martinez, L AU - Martinez L AD - Miami VA Medical Center, Miami, Florida. FAU - Greidinger, E L AU - Greidinger EL AD - Miami VA Medical Center and University of Miami Miller School of Medicine, Miami, Florida. LA - eng GR - I01 BX002047/BX/BLRD VA/United States GR - R01 AR043308/AR/NIAMS NIH HHS/United States GR - R01 AR071369/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20180725 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antibodies, Monoclonal) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 147785-83-9 (Keratin-10) SB - IM MH - Adoptive Transfer MH - Animals MH - Antibodies, Monoclonal/*immunology MH - Autoantibodies/*blood MH - Autoantigens/*blood MH - Autoimmunity/*immunology MH - Disease Models, Animal MH - Humans MH - Keratin-10/blood/immunology MH - Mice MH - Mice, Inbred C57BL MH - Proteomics MH - Raynaud Disease/*immunology PMC - PMC6115278 MID - NIHMS952863 COIS- The authors do not have any financial interests that could be perceived as being a conflict of interest. No patents pertaining to the results presented in the paper have been awarded or filed. EDAT- 2018/03/24 06:00 MHDA- 2019/07/23 06:00 PMCR- 2019/09/01 CRDT- 2018/03/24 06:00 PHST- 2017/01/20 00:00 [received] PHST- 2018/03/15 00:00 [accepted] PHST- 2018/03/24 06:00 [pubmed] PHST- 2019/07/23 06:00 [medline] PHST- 2018/03/24 06:00 [entrez] PHST- 2019/09/01 00:00 [pmc-release] AID - 10.1002/art.40505 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Sep;70(9):1489-1499. doi: 10.1002/art.40505. Epub 2018 Jul 25. PMID- 24192563 OWN - NLM STAT- MEDLINE DCOM- 20140923 LR - 20220408 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 65 IP - 4 DP - 2013 Oct 31 TI - Prospective capillaroscopy-based study on transition from primary to secondary Raynaud's phenomenon: preliminary results. PG - 186-91 LID - 10.4081/reumatismo.2013.186 [doi] AB - The objective of this prospective study was to investigate the transition from primary (PRP) to secondary (SRP) Raynaud's phenomenon (RP), in a large cohort of patients affected by isolated RP. A total of 2065 patients with RP were investigated by clinical interview, laboratory examinations, and nailfold videocapillaroscopy (NVC). Patients with negative NVC at first visit were yearly followed to monitor either the appearance of specific morphological alterations at NVC, or clinical manifestations of an underlying disease. Capillary abnormalities at NVC were scored, as well as the qualitative patterns of microangiopathy (Early, Active and Late). NVC was found negative at first visit in 1500 subjects; among them, 412 patients were evaluable and they were followed for a mean time of 5±4 years (range 2-13 years). Sixty-eight patients (16%) achieved a diagnosis of SRP during follow-up, showing normal or not specific capillary alterations at NVC 4% of patients (the diagnosis was undifferentiated connective tissue diseases), Early scleroderma-pattern 57%, Active scleroderma-pattern 7%, Late scleroderma-pattern 12%, and scleroderma-like pattern 18% of patients. The time of transition from normal/not specific capillary alterations to Early scleroderma-pattern was 4.4±3.8 years. Enlarged capillaries (diameter between 20 and 50 microns) and mild reduction of capillary density were found the more frequent markers at first NVC visit in patients who progressed to a scleroderma pattern (P=0.01). This study demonstrates in a large cohort, that almost 16% of patients initially diagnosed as affected by RP with negative NVC may transit to SRP during a mean follow-up of 4.4 years. PRP patients showing major notspecific alterations of nailfold capillaries at first NVC should be strictly monitored at least once a year since at higher risk of transition to SRP. FAU - Bernero, E AU - Bernero E AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa. elenabernero@gmail.com. FAU - Sulli, A AU - Sulli A FAU - Ferrari, G AU - Ferrari G FAU - Ravera, F AU - Ravera F FAU - Pizzorni, C AU - Pizzorni C FAU - Ruaro, B AU - Ruaro B FAU - Zampogna, G AU - Zampogna G FAU - Alessandri, E AU - Alessandri E FAU - Cutolo, M AU - Cutolo M LA - eng PT - Journal Article DEP - 20131031 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Disease Progression MH - Humans MH - *Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Prospective Studies MH - Raynaud Disease/*pathology EDAT- 2013/11/07 06:00 MHDA- 2014/09/24 06:00 CRDT- 2013/11/07 06:00 PHST- 2013/05/22 00:00 [received] PHST- 2013/10/09 00:00 [accepted] PHST- 2013/10/08 00:00 [revised] PHST- 2013/11/07 06:00 [entrez] PHST- 2013/11/07 06:00 [pubmed] PHST- 2014/09/24 06:00 [medline] AID - 10.4081/reumatismo.2013.186 [doi] PST - epublish SO - Reumatismo. 2013 Oct 31;65(4):186-91. doi: 10.4081/reumatismo.2013.186. PMID- 21239749 OWN - NLM STAT- MEDLINE DCOM- 20110720 LR - 20110404 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 38 IP - 4 DP - 2011 Apr TI - Plasma platelet-derived microparticles in patients with connective tissue diseases. PG - 680-4 LID - 10.3899/jrheum.100780 [doi] AB - OBJECTIVE: To clarify the role of platelet-derived microparticles (PDMP), which are small vesicles with thrombotic and immunological properties, in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis/polymyositis (PM/DM), and mixed connective tissue disease (MCTD). METHODS: Plasma levels of PDMP were measured by ELISA, and compared among patients with one of the 4 diseases. Association of PDMP levels with clinical characteristics and medication of the patients was also examined. RESULTS: PDMP levels were higher in patients with MCTD and SSc than in controls. Multiple linear regression analysis revealed that patients with Raynaud's phenomenon (RP) showed higher PDMP levels than those without. PDMP levels in individual patients did not fluctuate significantly over several months. CONCLUSION: PDMP level is associated with MCTD, SSc, and RP, and could be a novel marker for RP. FAU - Oyabu, Chinami AU - Oyabu C AD - Department of Clinical Pathology and Immunology, Kobe University Graduate School of Medicine, Kusunoki-cho, 650-0017, Kobe, Japan. FAU - Morinobu, Akio AU - Morinobu A FAU - Sugiyama, Daisuke AU - Sugiyama D FAU - Saegusa, Jun AU - Saegusa J FAU - Tanaka, Shino AU - Tanaka S FAU - Morinobu, Sahoko AU - Morinobu S FAU - Tsuji, Goh AU - Tsuji G FAU - Kasagi, Shimpei AU - Kasagi S FAU - Kawano, Seiji AU - Kawano S FAU - Kumagai, Shunichi AU - Kumagai S LA - eng PT - Journal Article DEP - 20110115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2011 Apr;38(4):590-2. doi: 10.3899/jrheum.101359. PMID: 21459954 MH - Adult MH - Blood Platelets/*chemistry MH - Cell-Derived Microparticles/*metabolism MH - Connective Tissue Diseases/*blood MH - Dermatomyositis/blood MH - Female MH - Humans MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/blood MH - Polymyositis/blood MH - Raynaud Disease/blood MH - Scleroderma, Systemic/blood EDAT- 2011/01/18 06:00 MHDA- 2011/07/21 06:00 CRDT- 2011/01/18 06:00 PHST- 2011/01/18 06:00 [entrez] PHST- 2011/01/18 06:00 [pubmed] PHST- 2011/07/21 06:00 [medline] AID - jrheum.100780 [pii] AID - 10.3899/jrheum.100780 [doi] PST - ppublish SO - J Rheumatol. 2011 Apr;38(4):680-4. doi: 10.3899/jrheum.100780. Epub 2011 Jan 15. PMID- 21136243 OWN - NLM STAT- MEDLINE DCOM- 20110516 LR - 20211020 IS - 1615-6722 (Electronic) IS - 0723-5003 (Linking) VI - 105 IP - 11 DP - 2010 Nov TI - [Treatment options of acral ulcers in MCTD]. PG - 837-40 LID - 10.1007/s00063-010-1143-2 [doi] AB - In patients with systemic sclerosis or mixed connective tissue disease (MCTD) acral ulcers are considered as frequent manifestations that may lead to amputation and loss of function, respectively, as a result of secondary Raynaud's phenomenon. Thus it is necessary to take advantage of all available medical and supportive measures. Adjuvant treatments such as hyperbaric oxygenation and regional sympathetic block represent interdisciplinary treatment options to improve oxygenation of critical ischemia and analgesia and should be subject of further investigation. FAU - Herink, Ann-Kristin AU - Herink AK AD - Universitätsklinik und Poliklinik für Innere Medizin II, Universitätsklinikum Halle (Saale), Halle (Saale), Germany. ann-kristin.herink@medizin.uni-halle.de FAU - Köthe, Lars AU - Köthe L FAU - Girndt, Matthias AU - Girndt M FAU - Keysser, Gernot AU - Keysser G LA - ger PT - Case Reports PT - Journal Article TT - Therapie akraler Nekrosen bei MCTD: Kombination von Iloprost, hyperbarer Oxygenation und regionaler Sympathikolyse - ein Fallbericht. DEP - 20101207 PL - Germany TA - Med Klin (Munich) JT - Medizinische Klinik (Munich, Germany : 1983) JID - 8303501 RN - 0 (Amides) RN - 0 (Vasodilator Agents) RN - 7IO5LYA57N (Ropivacaine) RN - 9PHQ9Y1OLM (Prednisolone) RN - I9ZF7L6G2L (Nifedipine) RN - JED5K35YGL (Iloprost) RN - MRK240IY2L (Azathioprine) SB - IM MH - Adult MH - *Amides MH - Analgesia, Patient-Controlled MH - Angiography, Digital Subtraction MH - *Autonomic Nerve Block MH - Azathioprine/therapeutic use MH - Combined Modality Therapy MH - Drug Therapy, Combination MH - Female MH - Fingers/*blood supply MH - Humans MH - *Hyperbaric Oxygenation MH - Iloprost/*therapeutic use MH - Infusions, Intravenous MH - Mixed Connective Tissue Disease/*therapy MH - Nifedipine/therapeutic use MH - Prednisolone/therapeutic use MH - Raynaud Disease/*therapy MH - Ropivacaine MH - Skin Ulcer/*therapy MH - Vasodilator Agents/*therapeutic use EDAT- 2010/12/08 06:00 MHDA- 2011/05/17 06:00 CRDT- 2010/12/08 06:00 PHST- 2010/08/28 00:00 [received] PHST- 2010/08/30 00:00 [accepted] PHST- 2010/12/08 06:00 [entrez] PHST- 2010/12/08 06:00 [pubmed] PHST- 2011/05/17 06:00 [medline] AID - 10.1007/s00063-010-1143-2 [doi] PST - ppublish SO - Med Klin (Munich). 2010 Nov;105(11):837-40. doi: 10.1007/s00063-010-1143-2. Epub 2010 Dec 7. PMID- 26168646 OWN - NLM STAT- MEDLINE DCOM- 20150812 LR - 20260128 IS - 0017-7768 (Print) IS - 0017-7768 (Linking) VI - 154 IP - 5 DP - 2015 May TI - [THE ADVERSE EFFECTS OF SMOKING ON THE HANDS]. PG - 327-9, 338 AB - Cigarette smoking is known to cause a multitude of harmful effects throughout the body. There are only a few accounts in the literature of these effects as related to the hands. This is a review of the literature, demonstrating the collected knowledge of decreased hand vascularity due to tobacco use and assessing the evidence connecting smoking and supposed resultant maladies, including Raynaud's phenomenon, hand-arm vibration syndrome, Buerger's disease, Dupuytren's contracture, carpal tunnel syndrome, effects on skin and fingernails, decreased skin and bone healing, complications of digit replantation and complex regional pain syndrome. Also presented is the possible increased risk of congenital hand malformations as related to maternal smoking. FAU - Dreyfuss, Daniel AU - Dreyfuss D FAU - Calif, Edward AU - Calif E FAU - Stahl, Shalom AU - Stahl S LA - heb PT - English Abstract PT - Journal Article PT - Review PL - Israel TA - Harefuah JT - Harefuah JID - 0034351 SB - IM MH - Carpal Tunnel Syndrome/etiology MH - Dupuytren Contracture/etiology MH - Hand/*blood supply/pathology/physiopathology MH - Hand Deformities, Congenital/etiology MH - Humans MH - Raynaud Disease/etiology MH - Regional Blood Flow/*drug effects MH - Risk Factors MH - *Smoking/adverse effects/physiopathology MH - Tobacco Use Disorder/complications/physiopathology MH - Nicotiana EDAT- 2015/07/15 06:00 MHDA- 2015/08/13 06:00 CRDT- 2015/07/15 06:00 PHST- 2015/07/15 06:00 [entrez] PHST- 2015/07/15 06:00 [pubmed] PHST- 2015/08/13 06:00 [medline] PST - ppublish SO - Harefuah. 2015 May;154(5):327-9, 338. PMID- 36853887 OWN - NLM STAT- MEDLINE DCOM- 20230911 LR - 20230916 IS - 1557-0681 (Electronic) IS - 1478-2189 (Linking) VI - 21 IP - 3 DP - 2023 Sep TI - 'It's just not the same… I am just a spectator': A qualitative study on changes in leisure participation experienced by people with scleroderma. PG - 733-740 LID - 10.1002/msc.1746 [doi] AB - BACKGROUND/OBJECTIVE: The purpose of this qualitative study was to provide insight into the changes and barriers to leisure experienced by people with scleroderma. METHOD: Twenty-five people with scleroderma consented to be interviewed via telephone using a semi-structured interview. The conversations were audio recorded and transcribed verbatim. Thorne's Interpretive Description informed the analysis process. RESULTS: Three themes emerged from the analysis: barriers to leisure participation (impact of Raynaud's Phenomenon symptoms, fatigue, changes in body structures and functions, unpredictability of daily symptoms and selection of leisure activities), decreases in leisure participation (less time outdoors, reduction in time spent in active leisure, more time spent in passive leisure) and experiences of losing a valued leisure activity (depressed mood, identity change, fear of loss and sense of isolation). CONCLUSIONS: The changes in leisure participation found in this study were reported to be due to the rate of disease progression and the severity of disease symptoms, particularly, Raynaud symptoms and fatigue. Health professionals should work with people with SSc to facilitate participation in valued leisure activities. CI - © 2023 John Wiley & Sons Ltd. FAU - Poole, Janet L AU - Poole JL AUID- ORCID: 0000-0003-3093-5707 AD - Occupational Therapy Graduate Program, Department of Pediatrics, University of New Mexico, Albuquerque, New Mexico, USA. FAU - Greaves, Jessica AU - Greaves J AD - Rehabilitation Services Department, University of New Mexico Hospital, Albuquerque, New Mexico, USA. FAU - Mendelson, Cyd AU - Mendelson C AD - Constellation Hospice, Philadelphia, Pennsylvania, USA. LA - eng PT - Journal Article DEP - 20230228 PL - England TA - Musculoskeletal Care JT - Musculoskeletal care JID - 101181344 SB - IM MH - Humans MH - Qualitative Research MH - Disease Progression MH - *Raynaud Disease/diagnosis MH - Fatigue MH - Leisure Activities MH - *Scleroderma, Systemic OTO - NOTNLM OT - leisure activities OT - qualitative research OT - scleroderma OT - systemic sclerosis EDAT- 2023/03/01 06:00 MHDA- 2023/09/11 06:43 CRDT- 2023/02/28 12:43 PHST- 2023/02/03 00:00 [revised] PHST- 2023/01/20 00:00 [received] PHST- 2023/02/11 00:00 [accepted] PHST- 2023/09/11 06:43 [medline] PHST- 2023/03/01 06:00 [pubmed] PHST- 2023/02/28 12:43 [entrez] AID - 10.1002/msc.1746 [doi] PST - ppublish SO - Musculoskeletal Care. 2023 Sep;21(3):733-740. doi: 10.1002/msc.1746. Epub 2023 Feb 28. PMID- 21360507 OWN - NLM STAT- MEDLINE DCOM- 20130124 LR - 20220410 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 63 IP - 3 DP - 2011 Mar TI - Modified-release sildenafil reduces Raynaud's phenomenon attack frequency in limited cutaneous systemic sclerosis. PG - 775-82 LID - 10.1002/art.30195 [doi] AB - OBJECTIVE: To examine the effect of sildenafil in patients with Raynaud's phenomenon (RP) secondary to limited cutaneous systemic sclerosis (lcSSc). METHODS: In this double-blind, placebo-controlled study, 57 patients with RP secondary to lcSSc were randomized to receive modified-release sildenafil 100 mg once daily for 3 days followed by modified-release sildenafil 200 mg once daily for 25 days or placebo. The primary assessment was the percentage change in the number of RP attacks per week in the per-protocol population. Secondary end points included Raynaud's Condition Score, duration of attacks, RP pain score, endothelial dysfunction assessed by a peripheral arterial tonometric (PAT) device, and serum biomarker levels. RESULTS: The mean percentage reduction from baseline to day 28 in attacks per week was greater for modified-release sildenafil than for placebo (-44.0% versus -18.1%, P = 0.034); the mean number of attacks per week improved from 25.0 at baseline to 19.3 after placebo treatment and from 30.5 to 18.7 after modified-release sildenafil treatment (P = 0.244). Decreases from baseline in Raynaud's Condition Score, duration of attacks, and RP pain score were not significantly different between groups. Mean values and changes from baseline in PAT responses and serum biomarker levels were similar between groups. The most frequent adverse events were headache and dyspepsia; the majority of adverse events were mild or moderate. CONCLUSION: Our findings indicate that modified-release sildenafil reduced attack frequency in patients with RP secondary to lcSSc and was well tolerated. Modified-release sildenafil may be a treatment option in this patient population. CI - Copyright © 2011 by the American College of Rheumatology. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester, Manchester Academic Health Science Centre, Salford Royal Hospital, Salford, UK. ariane.herrick@manchester.ac.uk FAU - van den Hoogen, Frank AU - van den Hoogen F FAU - Gabrielli, Armando AU - Gabrielli A FAU - Tamimi, Nihad AU - Tamimi N FAU - Reid, Carol AU - Reid C FAU - O'Connell, Damian AU - O'Connell D FAU - Vázquez-Abad, Maria-Dolores AU - Vázquez-Abad MD FAU - Denton, Christopher P AU - Denton CP LA - eng PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Biomarkers) RN - 0 (Delayed-Action Preparations) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Adult MH - Aged MH - Biomarkers/blood MH - Delayed-Action Preparations/administration & dosage/adverse effects MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Piperazines/*administration & dosage/adverse effects MH - Purines/administration & dosage/adverse effects MH - Raynaud Disease/*drug therapy/*etiology MH - Scleroderma, Limited/*complications MH - Secondary Prevention MH - Sildenafil Citrate MH - Sulfones/*administration & dosage/adverse effects MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage/adverse effects EDAT- 2011/03/02 06:00 MHDA- 2013/01/25 06:00 CRDT- 2011/03/02 06:00 PHST- 2011/03/02 06:00 [entrez] PHST- 2011/03/02 06:00 [pubmed] PHST- 2013/01/25 06:00 [medline] AID - 10.1002/art.30195 [doi] PST - ppublish SO - Arthritis Rheum. 2011 Mar;63(3):775-82. doi: 10.1002/art.30195. PMID- 30441211 OWN - NLM STAT- MEDLINE DCOM- 20191024 LR - 20200928 IS - 2694-0604 (Electronic) IS - 2375-7477 (Linking) VI - 2018 DP - 2018 Jul TI - Comparison between thermal recovery in women with Raynaud's Phenomenon and not diagnosed women using thermography. PG - 3886-3889 LID - 10.1109/EMBC.2018.8513367 [doi] AB - Thermography detects the infrared radiation emanated from bodies and transduces it in electrical analog signal. It has application as a complementary exam in several medical segments, including the reheating study to detect diseases like Raynaud's Phenomenon (RP). In this way, the aim of this study is to compare the heating behavior of the RP women and not diagnosed (ND) women for selection of diagnosis criteria. This retrospective study was undertaken in the city of Curitiba, Brazil. For the study, twenty-four volunteer women, with the age range of 30-70 years, were taken to participate of a survey, 12 of them have the clinical diagnostic of Secondary Raynauld's Phenomenon, and twelve women were not diagnosed. Volunteers answered an anamnesis and had the central body temperature measured. They were oriented to keep the hands free and to do not touch anything during 15 minutes for acclimatization. Then, the hands were immersed for 60 seconds in a container with water at 10 °C. New pictures were taken every five minutes during twenty minutes after the immersion. The heating curve of the right hand shows that RP women's hands are colder than ND women and are slower to reheat the temperature after cold stress. The ring (fourth finger) has a linear behavior in both hands. It was the coldest one and the slowest to reheat. Statistical difference was observed in critical times of reheating at 15 and 20 minutes after the cold stress. These results show that this finger could become a reference in studies to determine cutting points and to facilitate the clinical diagnosis of RP. FAU - Campos, M F AU - Campos MF FAU - Heimbecher, C T AU - Heimbecher CT FAU - Romaneli, E F R AU - Romaneli EFR FAU - Ripka, W L AU - Ripka WL FAU - Ulbricht, L AU - Ulbricht L FAU - Stadinik, A M AU - Stadinik AM LA - eng PT - Journal Article PL - United States TA - Annu Int Conf IEEE Eng Med Biol Soc JT - Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference JID - 101763872 SB - IM MH - Adult MH - Aged MH - Brazil MH - Cold Temperature MH - Female MH - Fingers MH - Humans MH - Middle Aged MH - *Raynaud Disease/diagnosis MH - Retrospective Studies MH - *Thermography EDAT- 2018/11/18 06:00 MHDA- 2019/10/28 06:00 CRDT- 2018/11/17 06:00 PHST- 2018/11/17 06:00 [entrez] PHST- 2018/11/18 06:00 [pubmed] PHST- 2019/10/28 06:00 [medline] AID - 10.1109/EMBC.2018.8513367 [doi] PST - ppublish SO - Annu Int Conf IEEE Eng Med Biol Soc. 2018 Jul;2018:3886-3889. doi: 10.1109/EMBC.2018.8513367. PMID- 19568080 OWN - NLM STAT- MEDLINE DCOM- 20100603 LR - 20220318 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 124 IP - 1 DP - 2009 Jul TI - Botox therapy for ischemic digits. PG - 191-201 LID - 10.1097/PRS.0b013e3181a80576 [doi] AB - BACKGROUND: Treating patients with Raynaud's phenomenon who have chronic pain and ulcerations is extremely challenging. Unrelenting pain can lead to dysfunction and disuse, rendering the patient debilitated and/or chronically depressed. Pharmacologic vasodilators and surgical sympathectomies offer variable benefits. Outcomes of symptomatic patients treated with botulinum toxin type A (Botox) injections for Raynaud's phenomenon are presented. METHODS: A retrospective study focused on patient outcomes was performed on 19 patients diagnosed with Raynaud's phenomenon. Patients suffered from chronic ischemic hand pain. All patients had vascular studies to rule out occlusive disease. Fifty to 100 units of Botox were injected into the palm around each involved neurovascular bundle. Preinjection and postinjection laser Doppler scanning was performed on most patients to measure blood flow. RESULTS: Sixteen of 19 patients (84 percent) reported pain reduction at rest. Thirteen patients reported immediate relief; three reported more gradual pain reduction over 1 to 2 months. Three patients had no or minimal pain relief. Tissue perfusion results demonstrated a marked change in blood flow (-48.15 percent to 425 percent) to the digits. All patients with chronic finger ulcers healed within 60 days. Most patients [n = 12 (63 percent)] remained pain-free (13 to 59 months) with a single-injection schedule. Four patients (21 percent) required repeated injections because of recurrent pain. CONCLUSIONS: Vascular function is abnormal in patients with Raynaud's phenomenon. Although its mechanism is unknown, Botox yielded a distinct improvement in perfusion and reduction in pain in patients failing conservative management. Continued research may lead to more specific and reliable treatment for Raynaud's patients. FAU - Neumeister, Michael W AU - Neumeister MW AD - Springfield, Ill. From the Plastic Surgery Institute, Southern Illinois University School of Medicine. FAU - Chambers, Christopher B AU - Chambers CB FAU - Herron, Margo S AU - Herron MS FAU - Webb, Kelli AU - Webb K FAU - Wietfeldt, Joel AU - Wietfeldt J FAU - Gillespie, Jessica N AU - Gillespie JN FAU - Bueno, Rueben A Jr AU - Bueno RA Jr FAU - Cooney, Carisa M AU - Cooney CM LA - eng PT - Journal Article PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*drug therapy/*etiology MH - Male MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*complications/*drug therapy MH - Retrospective Studies MH - Young Adult EDAT- 2009/07/02 09:00 MHDA- 2010/06/04 06:00 CRDT- 2009/07/02 09:00 PHST- 2009/07/02 09:00 [entrez] PHST- 2009/07/02 09:00 [pubmed] PHST- 2010/06/04 06:00 [medline] AID - 00006534-200907000-00025 [pii] AID - 10.1097/PRS.0b013e3181a80576 [doi] PST - ppublish SO - Plast Reconstr Surg. 2009 Jul;124(1):191-201. doi: 10.1097/PRS.0b013e3181a80576. PMID- 17120594 OWN - NLM STAT- MEDLINE DCOM- 20070212 LR - 20170214 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 15 IP - 10 DP - 2006 TI - Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus. PG - 671-4 AB - There are potential concerns regarding serotonin receptor agonists in SLE patients with migraine, particularly patients with concomitant Raynaud's syndrome. We estimated the prevalence of lupus-related headache and Raynaud's syndrome in the Montreal General Hospital SLE clinic cohort and evaluated the relationship between these two variables in multivariable logistic regression models, controlling for age, sex, race, SLE duration and the presence of lupus anticoagulant and antibodies to cardiolipin and beta2 glycoprotein I. We also assessed, through chart review in those individuals with both Raynaud's syndrome and migraine, a history of serotonin receptor agonist use, and any associated worsening vasospasm. Based on Systemic Lupus Activity Measure (SLAM) scores, the cumulative incidence of lupus-related headache in our sample (n = 391) was 46.1%; the prevalence of Raynaud's syndrome was 49.4%. The adjusted odds ratio (OR) for lupus-related headache and Raynaud's syndrome was 1.7 (95% CI 1.1, 2.5). In addition, there was a strong independent relationship between headache and anti-beta2 glycoprotein I antibodies (adjusted OR 5.6 [95% CI 1.8, 17.0]). The data from our chart review suggest that careful use of serotonin receptor agonists in patients with both Raynaud's syndrome and migraines may be undertaken, although caution would necessitate that these agents not be used in individuals with very severe Raynaud's (eg, digital ulcerations, and so on). FAU - Bernatsky, S AU - Bernatsky S AD - Division of Clinical Epidemiology, Montreal General Hospital, Montreal, PQ, Canada. FAU - Pineau, C A AU - Pineau CA FAU - Lee, J L AU - Lee JL FAU - Clarke, A E AU - Clarke AE LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Serotonin Receptor Agonists) SB - IM MH - Adult MH - Cohort Studies MH - Female MH - Headache/*epidemiology MH - Humans MH - Lupus Erythematosus, Systemic/*drug therapy/epidemiology/physiopathology MH - Male MH - Prevalence MH - Raynaud Disease/*epidemiology MH - Regression Analysis MH - Serotonin Receptor Agonists/*therapeutic use EDAT- 2006/11/24 09:00 MHDA- 2007/02/13 09:00 CRDT- 2006/11/24 09:00 PHST- 2006/11/24 09:00 [pubmed] PHST- 2007/02/13 09:00 [medline] PHST- 2006/11/24 09:00 [entrez] AID - 10.1177/0961203306069997 [doi] PST - ppublish SO - Lupus. 2006;15(10):671-4. doi: 10.1177/0961203306069997. PMID- 21461651 OWN - NLM STAT- MEDLINE DCOM- 20111129 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 30 IP - 8 DP - 2011 Aug TI - The association of Raynaud's syndrome with rheumatoid arthritis--a meta-analysis. PG - 1013-9 LID - 10.1007/s10067-011-1727-0 [doi] AB - Rheumatoid arthritis (RA) has traditionally been included among the diseases associated with Raynaud's syndrome (RS). The prevalence of RS in patients with RA is not well defined. The objective of this paper was to assess the prevalence of RS in patients with RA-a meta-analysis of published data was performed. The PubMed database of the National Library of Medicine and ISI Web of Knowledge was used for studies dealing with RS and RA. The studies provided sufficient data to estimate the prevalence of RS in patients with RA. A forest plot was determined by the revealed prevalences. Statistical analysis was based on methods for a random effects meta-analysis and a finite mixture model for proportions. Publication bias was investigated with the linear regression test (Egger's method). A meta-regression was conducted by the year of publication. Twenty-eight eligible studies, contributing data on 3,730 subjects, were included in this meta-analysis. For RA, a pooled prevalence of 12.3% and 95% CI = 0.093-0.157 were obtained. A mixture model analysis found five latent classes. Statistically and graphically, publication bias was present (p = 0.031). In the meta-regression, the estimated prevalence decreased within the observation period (1977-2010) from 11.2% to 9.4%. Despite some heterogeneity, there is a possible indication of an association for RS and patients with RA. FAU - Hartmann, Peter AU - Hartmann P AD - Department of Biometry and Medical Statistics, Campus Charité Mitte, Charité Universitätsmedizin Berlin, Ebene, Charitéplatz 1, Berlin, Germany. peter.hartmann@live.de FAU - Mohokum, Melvin AU - Mohokum M FAU - Schlattmann, Peter AU - Schlattmann P LA - eng PT - Journal Article PT - Meta-Analysis DEP - 20110402 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Arthritis, Rheumatoid/*epidemiology MH - Comorbidity MH - Databases, Factual MH - Female MH - Humans MH - Male MH - Prevalence MH - Raynaud Disease/*epidemiology MH - Sex Factors EDAT- 2011/04/05 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/04/05 06:00 PHST- 2011/01/25 00:00 [received] PHST- 2011/02/24 00:00 [accepted] PHST- 2011/04/05 06:00 [entrez] PHST- 2011/04/05 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 10.1007/s10067-011-1727-0 [doi] PST - ppublish SO - Clin Rheumatol. 2011 Aug;30(8):1013-9. doi: 10.1007/s10067-011-1727-0. Epub 2011 Apr 2. PMID- 25711876 OWN - NLM STAT- MEDLINE DCOM- 20160510 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 34 IP - 8 DP - 2015 Aug TI - Early systemic sclerosis-opportunities for treatment. PG - 1327-31 LID - 10.1007/s10067-015-2902-5 [doi] AB - Systemic sclerosis (SSc) is characterized by microvasculopathy (Raynaud's phenomenon and fibrointimal proliferation), presence of autoantibodies and collagen deposition in skin (scleroderma) and internal organs. Microvasculopathy, detected by nailfold capillaroscopy, and disease-specific autoantibodies (anti-topoisomerase I, anti-centromere, anti-RNA polymerase III antibodies) usually appear earlier, even years before scleroderma. At that stage of the disease, immune activation with T cells and B cells promote fibrosis. Diagnosis of SSc has been relied on scleroderma, and by this time, internal organs may have developed fibrosis, a lethal feature with no available treatment. The new EULAR/ACR 2013 criteria for the classification of SSc will help identify SSc patients before fibrosis of internal organs. The early diagnosis of SSc, before the development of fibrosis in internal organs, will allow the introduction of immunosuppressive medications in these patients in a controlled setting (randomized trials). It is anticipated that this approach will change the hitherto grim prognosis of SSc for the better. FAU - Sakkas, Lazaros I AU - Sakkas LI AD - Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Biopolis, 41 110, Larissa, Greece, lsakkas@med.uth.gr. FAU - Simopoulou, Theodora AU - Simopoulou T FAU - Katsiari, Christina AU - Katsiari C FAU - Bogdanos, Dimitrios AU - Bogdanos D FAU - Chikanza, Ian C AU - Chikanza IC LA - eng PT - Journal Article PT - Review DEP - 20150226 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) SB - IM MH - Autoantibodies MH - Early Diagnosis MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Microscopic Angioscopy MH - Prognosis MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis/drug therapy/immunology EDAT- 2015/02/26 06:00 MHDA- 2016/05/11 06:00 CRDT- 2015/02/26 06:00 PHST- 2015/02/09 00:00 [received] PHST- 2015/02/15 00:00 [accepted] PHST- 2015/02/26 06:00 [entrez] PHST- 2015/02/26 06:00 [pubmed] PHST- 2016/05/11 06:00 [medline] AID - 10.1007/s10067-015-2902-5 [doi] PST - ppublish SO - Clin Rheumatol. 2015 Aug;34(8):1327-31. doi: 10.1007/s10067-015-2902-5. Epub 2015 Feb 26. PMID- 26242228 OWN - NLM STAT- MEDLINE DCOM- 20160623 LR - 20160707 IS - 1179-1888 (Electronic) IS - 1175-0561 (Linking) VI - 16 IP - 5 DP - 2015 Oct TI - The Dermatological Manifestations of Postural Tachycardia Syndrome: A Review with Illustrated Cases. PG - 425-30 LID - 10.1007/s40257-015-0144-6 [doi] AB - Postural tachycardia syndrome (POTS) is a syndrome of excessive tachycardia with orthostatic challenge, and relief of such symptoms with recumbence. There are several proposed subtypes of the syndrome, each with unique pathophysiology. Numerous symptoms such as excessive tachycardia, lightheadedness, blurry vision, weakness, fatigue, palpitations, chest pain, and tremulousness are associated with orthostatic intolerance. Other co-morbid conditions associated with POTS are not clearly attributable to orthostatic intolerance. These include chronic headache, fibromyalgia, functional gastrointestinal or bladder disorders, cognitive impairment, and sleep disturbances. Dermatological manifestations of POTS are also common and wide ranging, from livedo reticularis to Raynaud's phenomenon, from cutaneous flushing to erythromelalgia. Here, we provide three illustrative cases of POTS with dermatological manifestations. We discuss the potential pathophysiology underlying such dermatological manifestations, and how such mechanisms could in turn help guide development of management. FAU - Huang, Hao AU - Huang H AD - Department of Neurology, Boston University Medical Campus, 72 East Concord St, A-302, Boston, MA, 02118, USA. hhuang@bu.edu. FAU - Hohler, Anna DePold AU - Hohler AD AD - Department of Neurology, Boston University Medical Campus, 72 East Concord St, A-302, Boston, MA, 02118, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - New Zealand TA - Am J Clin Dermatol JT - American journal of clinical dermatology JID - 100895290 SB - IM MH - Adult MH - Female MH - Flushing/*etiology MH - Humans MH - Hyperemia/*etiology MH - Livedo Reticularis/*etiology MH - Postural Orthostatic Tachycardia Syndrome/*complications MH - Raynaud Disease/*etiology MH - Young Adult EDAT- 2015/08/06 06:00 MHDA- 2016/06/24 06:00 CRDT- 2015/08/06 06:00 PHST- 2015/08/06 06:00 [entrez] PHST- 2015/08/06 06:00 [pubmed] PHST- 2016/06/24 06:00 [medline] AID - 10.1007/s40257-015-0144-6 [pii] AID - 10.1007/s40257-015-0144-6 [doi] PST - ppublish SO - Am J Clin Dermatol. 2015 Oct;16(5):425-30. doi: 10.1007/s40257-015-0144-6. PMID- 39142284 OWN - NLM STAT- MEDLINE DCOM- 20240912 LR - 20260211 IS - 2666-979X (Electronic) IS - 2666-979X (Linking) VI - 4 IP - 9 DP - 2024 Sep 11 TI - Genetic and functional analysis of Raynaud's syndrome implicates loci in vasculature and immunity. PG - 100630 LID - S2666-979X(24)00234-9 [pii] LID - 10.1016/j.xgen.2024.100630 [doi] LID - 100630 AB - Raynaud's syndrome is a dysautonomia where exposure to cold causes vasoconstriction and hypoxia, particularly in the extremities. We performed meta-analysis in four cohorts and discovered eight loci (ADRA2A, IRX1, NOS3, ACVR2A, TMEM51, PCDH10-DT, HLA, and RAB6C) where ADRA2A, ACVR2A, NOS3, TMEM51, and IRX1 co-localized with expression quantitative trait loci (eQTLs), particularly in distal arteries. CRISPR gene editing further showed that ADRA2A and NOS3 loci modified gene expression and in situ RNAscope clarified the specificity of ADRA2A in small vessels and IRX1 around small capillaries in the skin. A functional contraction assay in the cold showed lower contraction in ADRA2A-deficient and higher contraction in ADRA2A-overexpressing smooth muscle cells. Overall, our study highlights the power of genome-wide association testing with functional follow-up as a method to understand complex diseases. The results indicate temperature-dependent adrenergic signaling through ADRA2A, effects at the microvasculature by IRX1, endothelial signaling by NOS3, and immune mechanisms by the HLA locus in Raynaud's syndrome. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Tervi, Anniina AU - Tervi A AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. Electronic address: anniina.tervi@helsinki.fi. FAU - Ramste, Markus AU - Ramste M AD - Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Abner, Erik AU - Abner E AD - Institute of Genomics, University of Tartu, Tartu, Estonia. FAU - Cheng, Paul AU - Cheng P AD - Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Lane, Jacqueline M AU - Lane JM AD - Division of Sleep and Circadian Disorders, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA; Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA; Broad Institute of MIT and Harvard, Cambridge, MA, USA. FAU - Maher, Matthew AU - Maher M AD - Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA; Broad Institute of MIT and Harvard, Cambridge, MA, USA. FAU - Valliere, Jesse AU - Valliere J AD - Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA; Broad Institute of MIT and Harvard, Cambridge, MA, USA. FAU - Lammi, Vilma AU - Lammi V AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. FAU - Strausz, Satu AU - Strausz S AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. FAU - Riikonen, Juha AU - Riikonen J AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. FAU - Nguyen, Trieu AU - Nguyen T AD - Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Martyn, Gabriella E AU - Martyn GE AD - Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA; Basic Science and Engineering Initiative, Stanford Children's Health, Betty Irene Moore Children's Heart Center, Stanford, CA, USA. FAU - Sheth, Maya U AU - Sheth MU AD - Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA; Basic Science and Engineering Initiative, Stanford Children's Health, Betty Irene Moore Children's Heart Center, Stanford, CA, USA. FAU - Xia, Fan AU - Xia F AD - Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA; Basic Science and Engineering Initiative, Stanford Children's Health, Betty Irene Moore Children's Heart Center, Stanford, CA, USA. FAU - Docampo, Mauro Lago AU - Docampo ML AD - Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA; Stanford Children's Health Betty Irene Moore Children's Heart Center, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA. FAU - Gu, Wenduo AU - Gu W AD - Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. CN - FinnGen, Estonian Biobank research team FAU - Esko, Tõnu AU - Esko T AD - Institute of Genomics, University of Tartu, Tartu, Estonia. FAU - Saxena, Richa AU - Saxena R AD - Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA; Broad Institute of MIT and Harvard, Cambridge, MA, USA; Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. FAU - Pirinen, Matti AU - Pirinen M AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland; Department of Mathematics and Statistics, University of Helsinki, Helsinki, Finland; Public Health, Faculty of Medicine, University of Helsinki, Helsinki, Finland. FAU - Palotie, Aarno AU - Palotie A AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland; Stanley Center for Psychiatric Research, Broad Institute of MIT and Harvard, Cambridge, MA, USA; Analytic and Translational Genetics Unit, Massachusetts General Hospital, Boston, MA, USA; Program in Medical and Population Genetics, Broad Institute of MIT and Harvard, Cambridge, MA, USA. FAU - Ripatti, Samuli AU - Ripatti S AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland; Broad Institute of MIT and Harvard, Cambridge, MA, USA; Public Health, Faculty of Medicine, University of Helsinki, Helsinki, Finland. FAU - Sinnott-Armstrong, Nasa AU - Sinnott-Armstrong N AD - Herbold Computational Biology Program, Public Health Sciences Division, Fred Hutch, Seattle, WA, USA. FAU - Daly, Mark AU - Daly M AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland; Stanley Center for Psychiatric Research, Broad Institute of MIT and Harvard, Cambridge, MA, USA; Analytic and Translational Genetics Unit, Massachusetts General Hospital, Boston, MA, USA; Program in Medical and Population Genetics, Broad Institute of MIT and Harvard, Cambridge, MA, USA. FAU - Engreitz, Jesse M AU - Engreitz JM AD - Department of Genetics, Stanford University School of Medicine, Stanford, CA, USA; Basic Science and Engineering Initiative, Stanford Children's Health, Betty Irene Moore Children's Heart Center, Stanford, CA, USA; The Novo Nordisk Foundation Center for Genomic Mechanisms of Disease, Broad Institute of MIT and Harvard, Cambridge, MA, USA; Gene Regulation Observatory, Broad Institute of MIT and Harvard, Cambridge, MA, USA; Stanford Cardiovascular Institute, Stanford University, Stanford, CA, USA. FAU - Rabinovitch, Marlene AU - Rabinovitch M AD - Stanford Children's Health Betty Irene Moore Children's Heart Center, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA. FAU - Heckman, Caroline A AU - Heckman CA AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. FAU - Quertermous, Thomas AU - Quertermous T AD - Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Jones, Samuel E AU - Jones SE AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland. FAU - Ollila, Hanna M AU - Ollila HM AD - Institute for Molecular Medicine Finland, FIMM, Helsinki Institute of Life Science - HiLIFE, University of Helsinki, Helsinki, Finland; Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA; Broad Institute of MIT and Harvard, Cambridge, MA, USA; Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA. Electronic address: hanna.m.ollila@helsinki.fi. LA - eng GR - R01 HL138473/HL/NHLBI NIH HHS/United States GR - R00 HG009917/HG/NHGRI NIH HHS/United States GR - UM1 HG011972/HG/NHGRI NIH HHS/United States GR - R01 HL159176/HL/NHLBI NIH HHS/United States GR - R01 AI170850/AI/NIAID NIH HHS/United States GR - K99 HG009917/HG/NHGRI NIH HHS/United States PT - Journal Article DEP - 20240813 PL - United States TA - Cell Genom JT - Cell genomics JID - 9918284260106676 RN - EC 1.14.13.39 (Nitric Oxide Synthase Type III) SB - IM MH - *Raynaud Disease/genetics/immunology MH - Humans MH - *Quantitative Trait Loci MH - *Genome-Wide Association Study MH - Nitric Oxide Synthase Type III/genetics/metabolism MH - Female MH - Male PMC - PMC11480858 COIS- Declaration of interests J.M.E. is an inventor on patents and patent applications related to CRISPR screening technologies, has received materials from 10× Genomics unrelated to this study, and has received speaking honoraria from GSK plc. EDAT- 2024/08/15 00:42 MHDA- 2024/09/13 00:46 PMCR- 2024/08/13 CRDT- 2024/08/14 18:42 PHST- 2023/11/28 00:00 [received] PHST- 2024/04/25 00:00 [revised] PHST- 2024/07/14 00:00 [accepted] PHST- 2024/09/13 00:46 [medline] PHST- 2024/08/15 00:42 [pubmed] PHST- 2024/08/14 18:42 [entrez] PHST- 2024/08/13 00:00 [pmc-release] AID - S2666-979X(24)00234-9 [pii] AID - 100630 [pii] AID - 10.1016/j.xgen.2024.100630 [doi] PST - ppublish SO - Cell Genom. 2024 Sep 11;4(9):100630. doi: 10.1016/j.xgen.2024.100630. Epub 2024 Aug 13. PMID- 39652122 OWN - NLM STAT- MEDLINE DCOM- 20250427 LR - 20250518 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 1 DP - 2025 Jan TI - Barnidipine as a potential alternative treatment for Raynaud's phenomenon secondary to systemic sclerosis: a retrospective pilot study. PG - 299-304 LID - 10.1007/s10067-024-07238-2 [doi] AB - OBJECTIVES: To assess short-term barnidipine efficacy and tolerability on Systemic Sclerosis (SSc)-Raynaud's phenomenon (RP). METHODS: We retrospectively evaluated patients with SSc starting barnidipine 10 mg/day. Raynaud's Condition Score (RCS) and mean blood pressure (MBP) were assessed at baseline and 6-month follow-up. Discontinuation rates and adverse events (AEs) were compared with retrospectively evaluated patients who started MR-nifedipine 20 mg/day at our centre. RESULTS: Sixty-four patients (29 barnidipine, 35 MR-nifedipine) were evaluated. Most patients starting barnidipine had a previous CCB exposure (69%; 57.9% of these were withdrawn for AEs). During follow-up, RCS decreased significantly (6.6 ± 1.8 vs 4.4 ± 1.8, p < 0.001), while the MBP did not change significantly (86.4 ± 8.4 vs 85 ± 7.3 mmHg; p = 0.36). Nine patients (31%) discontinued barnidipine during the follow-up (AEs 6/9, inefficacy 3/9). Previous CCB failure did not predict barnidipine withdrawal (OR = 0.188 95%CI [0.02-1.8]; p = 0.147). No predictors of barnidipine withdrawal were found, and no clinical or demographic differences were found between patients withdrawing and continuing barnidipine. The 6-month retention rates of barnidipine and MR-nifedipine did not differ significantly (69 vs 80%; log-rank p = 0.23), and the two groups had a similar incidence of AEs leading to discontinuation (20.1 vs 17%; p = 0.48). CONCLUSIONS: Barnidipine could be an effective and well-tolerated therapeutic option to treat SSc-RP, even in patients previously failing other CCBs. Key Points • Calcium channel blockers (CCBs) currently represent the first-line treatment for Raynaud's phenomenon secondary to Systemic Sclerosis (SSc-RP). However, little data exists about using novel CCBs, such as barnidipine, in this condition. • This study retrospectively evaluated patients with SSc-RP treated with barnidipine to assess its short-term efficacy. To serve as a control group for tolerability, we retrospectively compared these patients with others starting modified release-nifedipine (MR-nifedipine). • In patients treated with barnidipine, we observed a significant decrease in Raynaud's condition score at the 6-month follow-up without substantial changes in mean blood pressure. • The proportion of adverse events and drug withdrawal rate between patients starting barnidipine and MR-nifedipine did not differ significantly, even in patients starting barnidipine after previously failing other CCBs. CI - © 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Bixio, Riccardo AU - Bixio R AUID- ORCID: 0000-0001-9335-3371 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. riccard.bixio@univr.it. FAU - Mastropaolo, Francesca AU - Mastropaolo F AUID- ORCID: 0000-0002-3225-2152 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Appoloni, Matteo AU - Appoloni M AUID- ORCID: 0009-0002-6967-7805 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Bertelle, Davide AU - Bertelle D AUID- ORCID: 0000-0002-5901-7742 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. AD - Rheumatology Section, Department of Medicine, Azienda Sanitaria Friuli Occidentale, Pordenone, Italy. FAU - Bertoldo, Eugenia AU - Bertoldo E AUID- ORCID: 0000-0001-5678-3161 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. AD - Internal Medicine Unit, Department of Medicine, Mater Salutis Hospital, Legnago, Italy. FAU - Morciano, Andrea AU - Morciano A AUID- ORCID: 0000-0002-8653-7858 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Di Donato, Stefano AU - Di Donato S AUID- ORCID: 0000-0003-1256-0761 AD - Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Adami, Giovanni AU - Adami G AUID- ORCID: 0000-0002-8915-0755 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Viapiana, Ombretta AU - Viapiana O AUID- ORCID: 0000-0003-1917-5655 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Rossini, Maurizio AU - Rossini M AUID- ORCID: 0000-0001-9692-2293 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. FAU - Luca, Idolazzi AU - Luca I AUID- ORCID: 0000-0002-7254-4686 AD - Rheumatology Unit, Department of Medicine, University of Verona, P.le L.A. Scuro 10, 37134, Verona, Italy. LA - eng PT - Journal Article DEP - 20241209 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - I9ZF7L6G2L (Nifedipine) RN - 0 (Calcium Channel Blockers) RN - 0 (Dihydropyridines) SB - IM MH - Humans MH - Retrospective Studies MH - Female MH - Male MH - Middle Aged MH - *Scleroderma, Systemic/complications MH - *Raynaud Disease/drug therapy/etiology MH - Pilot Projects MH - *Nifedipine/therapeutic use/analogs & derivatives/adverse effects MH - Aged MH - Adult MH - Blood Pressure/drug effects MH - *Calcium Channel Blockers/therapeutic use MH - Treatment Outcome MH - *Dihydropyridines/therapeutic use OTO - NOTNLM OT - Barnidipine OT - Nifedipine OT - Raynaud’s phenomenon OT - Systemic sclerosis COIS- Declarations. Transparency declaration: The lead author (the manuscript’s guarantor) affirms that the manuscript is an honest, accurate, and transparent account of the study being reported; that no important aspects of the study have been omitted; and that any discrepancies from the study as planned (and, if relevant, registered) have been explained. Ethical and consent statements: The studies involving human participants were reviewed and approved by our local Ethics Committee (Verona, Italy). The study was conducted within protocol 1483CESC, per the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Written informed consent was obtained from all individual participants included. The patients/participants provided their written informed consent to participate in this study. Conflict of interest: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare no conflicts of interest. EDAT- 2024/12/09 17:33 MHDA- 2025/01/13 12:36 CRDT- 2024/12/09 11:03 PHST- 2024/04/26 00:00 [received] PHST- 2024/11/12 00:00 [accepted] PHST- 2024/11/07 00:00 [revised] PHST- 2025/01/13 12:36 [medline] PHST- 2024/12/09 17:33 [pubmed] PHST- 2024/12/09 11:03 [entrez] AID - 10.1007/s10067-024-07238-2 [pii] AID - 10.1007/s10067-024-07238-2 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Jan;44(1):299-304. doi: 10.1007/s10067-024-07238-2. Epub 2024 Dec 9. PMID- 38956991 OWN - NLM STAT- MEDLINE DCOM- 20240703 LR - 20240705 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 3 IP - 2 DP - 2024 Apr-Jun TI - Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases. PG - 84-94 AB - OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs). METHODS: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined. RESULTS: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use. CONCLUSIONS: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions. FAU - Santiago, Tânia AU - Santiago T AD - Centro Hospitalar e Universitário de Coimbra. FAU - Duarte, Ana Catarina AU - Duarte AC AD - Unidade Local de Saúde de Almada-Seixal - Hospital Garcia de Orta, Almada, Portugal. FAU - Sepriano, Alexandre AU - Sepriano A AD - Hospital de Egas Moniz, Unidade Local de Saúde de Lisboa Ocidental, Lisboa, Portugal. FAU - Castro, Alice AU - Castro A AD - Unidade Local de Saúde Almada-Seixal - Hospital Garcia de Orta, Almada, Portugal. FAU - Rosa, Bruno AU - Rosa B AD - Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Resende, Catarina AU - Resende C AD - Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Oliveira, Daniela AU - Oliveira D AD - Unidade Local de Saúde de São João, Porto, Portugal. FAU - Dourado, Eduardo AU - Dourado E AD - Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal. FAU - Costa, Emanuel AU - Costa E AD - Unidade Local de Saúde de Braga, Braga, Portugal. FAU - Cunha-Santos, Filipe AU - Cunha-Santos F AD - Unidade Local de Saúde da Guarda - Hospital Sousa Martins, Guarda, Portugal. FAU - Terroso, Georgina AU - Terroso G AD - Unidade Local de Saúde de São João, Porto, Portugal. FAU - Boleto, Gonçalo AU - Boleto G AD - Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Silva, Ivone AU - Silva I AD - Centro Hospitalar Universitário do Porto. FAU - Barbosa, Lurdes AU - Barbosa L AD - Unidade Local de Saúde Almada-Seixal - Hospital Garcia de Orta, Almada, Portugal. FAU - Silva, Joana AU - Silva J AD - Unidade Local de Saúde de Braga, Braga, Portugal. FAU - Sousa Neves, Joana AU - Sousa Neves J AD - Unidade Local de Saúde de Braga, Braga, Portugal. FAU - Salvador, Maria João AU - Salvador MJ AD - Unidade Local de Saúde de Coimbra - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Gonçalves, Maria João AU - Gonçalves MJ AD - Hospital de Egas Moniz, Unidade Local de Saúde de Lisboa Ocidental, Lisboa, Portugal. FAU - Gomes Guerra, Miguel AU - Gomes Guerra M AD - Unidade Local de Saúde da Cova da Beira, Covilhã, Portugal. FAU - Miriam Ferreira, Raquel AU - Miriam Ferreira R AD - Unidade Local de Saúde de São João, Porto, Portugal. FAU - Duarte-Fernandes, Rúben AU - Duarte-Fernandes R AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom. FAU - Barreira, Sofia AU - Barreira S AD - Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Silvestre Teixeira, Vítor AU - Silvestre Teixeira V AD - Unidade Local de Saúde do Algarve. FAU - Tomás, Ana Lúcia AU - Tomás AL AD - Liga Portuguesa Contra as Doenças Reumáticas. FAU - Romão, Vasco AU - Romão V AD - Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Cordeiro, Ana AU - Cordeiro A AD - Unidade Local de Saúde Almada-Seixal - Hospital Garcia de Orta, Almada, Portugal. LA - eng PT - Journal Article PT - Practice Guideline PT - Systematic Review TT - Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases. PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 SB - IM MH - Humans MH - *Connective Tissue Diseases/complications/therapy MH - *Fingers/blood supply/pathology MH - Portugal MH - *Raynaud Disease/therapy/etiology MH - *Scleroderma, Systemic/complications/therapy MH - *Skin Ulcer/therapy/etiology EDAT- 2024/07/03 06:41 MHDA- 2024/07/03 06:42 CRDT- 2024/07/03 02:53 PHST- 2024/07/03 06:42 [medline] PHST- 2024/07/03 06:41 [pubmed] PHST- 2024/07/03 02:53 [entrez] AID - PC240111 [pii] PST - ppublish SO - ARP Rheumatol. 2024 Apr-Jun;3(2):84-94. PMID- 32424835 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 47 IP - 8 DP - 2020 Aug TI - Prevalence and clinical characteristics of earlobe crease in systemic sclerosis: Possible association with vascular dysfunction. PG - 870-875 LID - 10.1111/1346-8138.15400 [doi] AB - Patients with systemic sclerosis (SSc) develop various vasculopathy-induced vascular disorders such as Raynaud's phenomenon, abnormal nail-fold capillaries, persistent digital ischemia, digital ulcers (DU), and sometimes develop renal crisis and pulmonary artery hypertension (PAH), affecting prognosis. Earlobe crease (ELC), also known as Frank's sign, is a wrinkle extending from the tragus to the outer border of the earlobe and is generally recognized as the sign of cardiovascular events. However, no previous study analyzed the association between ELC and SSc. In this study, we examined the prevalence and clinical characteristics of ELC in SSc patients. We analyzed 145 Japanese SSc patients and found that the prevalence of ELC in SSc patients was 23.4% (43/145), similar to that previously reported in the general population without SSc. Using univariate analysis, we found that SSc patients with ELC were characterized by old age, high incidence of DU, ILD and PAH, and high complication of coronary artery diseases (CAD) compared with SSc patients without ELC. In multivariate analysis, ELC was significantly associated with old age and incidence of DU. PAH tended to correlate with ELC without statistical significance. These results suggest that ELC may be associated with vascular disorders in SSc patients. This is the first report concerning the prevalence and clinical characteristics of ELC in patients with SSc. ELC is very easy to detect in clinical practice and helps physicians to identify SSc patients at risk of developing vascular disorders such as DU and PAH. CI - © 2020 Japanese Dermatological Association. FAU - Sekiguchi, Akiko AU - Sekiguchi A AUID- ORCID: 0000-0002-7016-337X AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Inoue, Yuta AU - Inoue Y AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Yamazaki, Sahori AU - Yamazaki S AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uchiyama, Akihiko AU - Uchiyama A AUID- ORCID: 0000-0002-2169-2427 AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Ishikawa, Osamu AU - Ishikawa O AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Kuribayashi, Shiko AU - Kuribayashi S AD - Department of, Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uraoka, Toshio AU - Uraoka T AD - Department of, Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Hara, Kenichiro AU - Hara K AD - Department of, Respiratory Medicine, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Yamaguchi, Kouichi AU - Yamaguchi K AD - Department of, Respiratory Medicine, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Maeno, Toshitaka AU - Maeno T AD - Department of, Respiratory Medicine, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uchida, Mitsuo AU - Uchida M AD - Department of, Public Health, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Koyama, Hiroshi AU - Koyama H AD - Department of, Public Health, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Motegi, Sei-Ichiro AU - Motegi SI AUID- ORCID: 0000-0001-8286-0669 AD - Departments of, Department of, Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. LA - eng PT - Journal Article DEP - 20200518 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - *Ear Auricle MH - Humans MH - Prevalence MH - *Raynaud Disease/diagnosis/epidemiology/etiology MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology MH - *Skin Ulcer/diagnosis/epidemiology/etiology OTO - NOTNLM OT - digital ulcers OT - earlobe crease OT - pulmonary artery hypertension OT - systemic sclerosis OT - vascular dysfunction EDAT- 2020/05/20 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/05/20 06:00 PHST- 2020/03/01 00:00 [received] PHST- 2020/04/20 00:00 [accepted] PHST- 2020/05/20 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/05/20 06:00 [entrez] AID - 10.1111/1346-8138.15400 [doi] PST - ppublish SO - J Dermatol. 2020 Aug;47(8):870-875. doi: 10.1111/1346-8138.15400. Epub 2020 May 18. PMID- 17824306 OWN - NLM STAT- MEDLINE DCOM- 20080401 LR - 20220408 IS - 0001-5385 (Print) IS - 0001-5385 (Linking) VI - 62 IP - 4 DP - 2007 Aug TI - Combined systolic and diastolic heart failure as the first presentation of mixed connective tissue disease. PG - 421-3 AB - A 50-year-old woman presented with combined systolic and diastolic heart failure and Raynaud's phenomenon with painful necrotic lesions around several nail beds. Several clinical criteria and the specific biochemical findings lead to the diagnosis of mixed connective tissue disease (MCTD) which is an autoimmune connective tissue disease characterized by cutaneous and visceral fibrosis and widespread vascular pathology.After treatment with diuretics, angiotensin-converting enzyme inhibitors (ACE-inhibitor) and beta blockers the heart failure symptoms disappeared and the ejection fraction improved. The severe pain in the hands due to Raynaud's phenomenon diminished only after treatment with calcium channel blocker nifedipine (Adalat, 10 mg, PO, Bayer HealthCare, Germany) without affecting the cardiovascular status. Cardiac involvement in connective tissue disease is not uncommon, but to our knowledge we present here the first case of MCTD with a combined systolic and diastolic heart failure as primary manifestation. FAU - Schwagten, Bruno AU - Schwagten B AD - AZ Middelheim, Antwerpen, Belgium. Brunoschwagten@hotmail.com FAU - Verheye, Stefan AU - Verheye S FAU - Van den Heuvel, Paul AU - Van den Heuvel P LA - eng PT - Case Reports PT - Journal Article PL - England TA - Acta Cardiol JT - Acta cardiologica JID - 0370570 RN - 0 (Adrenergic beta-Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) RN - 0 (Diuretics) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adrenergic beta-Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Diuretics/therapeutic use MH - Female MH - Heart Failure, Diastolic/drug therapy/*etiology/physiopathology MH - Heart Failure, Systolic/drug therapy/*etiology/physiopathology MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications/drug therapy MH - Nifedipine/therapeutic use MH - Raynaud Disease/drug therapy/etiology MH - Stroke Volume/drug effects EDAT- 2007/09/11 09:00 MHDA- 2008/04/02 09:00 CRDT- 2007/09/11 09:00 PHST- 2007/09/11 09:00 [pubmed] PHST- 2008/04/02 09:00 [medline] PHST- 2007/09/11 09:00 [entrez] AID - 10.2143/AC.62.4.2022289 [doi] PST - ppublish SO - Acta Cardiol. 2007 Aug;62(4):421-3. doi: 10.2143/AC.62.4.2022289. PMID- 19319790 OWN - NLM STAT- MEDLINE DCOM- 20090619 LR - 20131121 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 134 Suppl Falldatenbank DP - 2009 TI - [Unusual reason for unilateral (corrected) Raynaud's phenomenon with intensification when the arms are elevated]. PG - F3 LID - 10.1055/s-0028-1082827 [doi] AB - Thoracic outlet syndrome (TOS) is a broad term for compression of the neurovascular structures in the area of the 1. rib and the clavicle. The cause can be either fibrous bands, cervical ribs, anomalous muscles or posttraumatic changes as well as tumors. Symptoms depend on the affected structure, in most cases (up to 97% of TOS patients) neurologic symptoms are present. In case of an arterial compression, for example due to a cervical rib like in our case, embolism of the arm and finger arteries can occur. For mild or moderate symptoms a conservative approach with physiotherapy can be helpful. For severe cases surgical resection of the compressing structure and the first rib is necessary. In our case, the cervical and first rib were excised after an initial lysis therapy. Furthermore, the aneurysm of the subclavian artery was excised. FAU - Henes, J AU - Henes J AD - Medizinische Universitätsklinik Tübingen, Abteilung für Onkologie, Hämatologie, Rheumatologie, Immunologie, Pulmonologie, Tübingen, Germany. FAU - Oberländer, Y AU - Oberländer Y FAU - Tepe, G AU - Tepe G FAU - Schneider, W AU - Schneider W FAU - Balletshofer, B AU - Balletshofer B LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Einseitiges Raynaud-Phänomen mit Verstärkung bei Elevation des Arms. DEP - 20090324 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Anticoagulants) RN - 0 (Enoxaparin) RN - 0 (Fibrinolytic Agents) RN - 0 (Platelet Aggregation Inhibitors) RN - EC 3.4.21.73 (Urokinase-Type Plasminogen Activator) RN - R16CO5Y76E (Aspirin) SB - IM EIN - Dtsch Med Wochenschr. 2009;134:E2 MH - Adult MH - Aneurysm/complications/surgery MH - Anticoagulants/administration & dosage MH - Aspirin/administration & dosage MH - Cervical Rib Syndrome/*complications/*diagnosis/surgery MH - Diagnosis, Differential MH - Enoxaparin/administration & dosage MH - Female MH - Fibrinolytic Agents/administration & dosage MH - Humans MH - Infusions, Intra-Arterial MH - Platelet Aggregation Inhibitors/administration & dosage MH - Raynaud Disease/*etiology/therapy MH - Ribs/abnormalities/surgery MH - Subclavian Artery MH - Thromboembolism/*etiology/therapy MH - Urokinase-Type Plasminogen Activator/administration & dosage EDAT- 2009/06/18 09:00 MHDA- 2009/06/20 09:00 CRDT- 2009/03/26 09:00 PHST- 2009/03/26 09:00 [entrez] PHST- 2009/06/18 09:00 [pubmed] PHST- 2009/06/20 09:00 [medline] AID - 10.1055/s-0028-1082827 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2009;134 Suppl Falldatenbank:F3. doi: 10.1055/s-0028-1082827. Epub 2009 Mar 24. PMID- 39404484 OWN - NLM STAT- MEDLINE DCOM- 20241015 LR - 20241015 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 10 DP - 2024 Oct TI - Nailfold videocapillaroscopy abnormalities and vascular manifestations in Behçet's syndrome. PG - 2065-2070 LID - 10.55563/clinexprheumatol/v5mz8d [doi] AB - OBJECTIVES: To evaluate microcirculation abnormalities and their clinical association in patients with BS, especially with vascular manifestations. METHODS: A cross-sectional study was carried out using nailfold videocapillaroscopy (NVC) to evaluate the microcirculation in patients with BS. RESULTS: A total of 65 patients were included in the study. Thirty-four (52.3%) were men, and 84.6% were European Caucasian. Vascular involvement was present in 24 (36.9%) patients. Qualitative abnormalities in NVC were observed in 47.7% of patients. The most frequent were tortuous and branched capillaries (21.5%), followed by microhaemorrhage (12.3%), enlarged capillary (7.7%) and giant capillary (3.1%). We found a significant relationship between the presence of tortuous and branched capillaries and previous superficial thrombophlebitis (ST) (p=0.025). The presence of ≥2 qualitative abnormalities in NVC was associated with vascular involvement (p=0.031), mainly with venous thrombotic events (p=0.024) and particularly with ST (p=0.003). No specific Cutolo's pattern was observed. No association was observed between NVC and Raynaud's phenomenon or ANA, although patients with positive ANA presented more frequent non-specific capillaroscopic abnormalities (p=0.003). CONCLUSIONS: The presence of ≥2 qualitative abnormalities in NVC is associated with Vasculo-Behçet. NVC might be a potential tool for early detection of patients at risk of vascular events. FAU - Mercadé-Torras, Joan Maria AU - Mercadé-Torras JM AD - Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Parc Taulí Universitari Hospital, Institut d'Investigació i Innovació Parc Taulí I3PT-CERCA, Universitat Autònoma de Barcelona, Sabadell, Spain. FAU - Guillén-Del-Castillo, Alfredo AU - Guillén-Del-Castillo A AD - Systemic Autoimmune Diseases Unit, Vall d'Hebron General Hospital, Universitat Autònoma de Barcelona, Spain. FAU - Buján, Segundo AU - Buján S AD - Systemic Autoimmune Diseases Unit, Vall d'Hebron General Hospital, Universitat Autònoma de Barcelona, Spain. FAU - Solans-Laque, Roser AU - Solans-Laque R AD - Systemic Autoimmune Diseases Unit, Vall d'Hebron General Hospital, Universitat Autònoma de Barcelona, Spain. rsolanslaq@gmail.com. LA - eng PT - Journal Article DEP - 20241004 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Male MH - *Behcet Syndrome/complications/physiopathology/diagnosis/diagnostic imaging MH - Female MH - *Microscopic Angioscopy MH - Cross-Sectional Studies MH - Adult MH - *Nails/blood supply MH - Middle Aged MH - *Capillaries/diagnostic imaging/pathology/physiopathology MH - *Microcirculation MH - Predictive Value of Tests MH - Young Adult MH - Raynaud Disease/physiopathology/etiology/diagnostic imaging EDAT- 2024/10/15 16:19 MHDA- 2024/10/15 16:20 CRDT- 2024/10/15 09:33 PHST- 2024/03/22 00:00 [received] PHST- 2024/07/18 00:00 [accepted] PHST- 2024/10/15 16:20 [medline] PHST- 2024/10/15 16:19 [pubmed] PHST- 2024/10/15 09:33 [entrez] AID - 21093 [pii] AID - 10.55563/clinexprheumatol/v5mz8d [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Oct;42(10):2065-2070. doi: 10.55563/clinexprheumatol/v5mz8d. Epub 2024 Oct 4. PMID- 21920896 OWN - NLM STAT- MEDLINE DCOM- 20120716 LR - 20161125 IS - 1399-3003 (Electronic) IS - 0903-1936 (Linking) VI - 39 IP - 3 DP - 2012 Mar TI - Significance of connective tissue disease features in idiopathic interstitial pneumonia. PG - 661-8 LID - 10.1183/09031936.00174910 [doi] AB - In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP. IIP patients undergoing surgical lung biopsy (1979-2005) were studied (nonspecific interstitial pneumonia (NSIP), n = 45; idiopathic pulmonary fibrosis, n = 56). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated. UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p<0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged <50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p = 0.02) independent of IIP severity. UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear. FAU - Corte, T J AU - Corte TJ AD - Dept of Respiratory Medicine, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College, Emmanuel Kaye Building, 1B Manresa Road, London SW3 6LP, UK. FAU - Copley, S J AU - Copley SJ FAU - Desai, S R AU - Desai SR FAU - Zappala, C J AU - Zappala CJ FAU - Hansell, D M AU - Hansell DM FAU - Nicholson, A G AU - Nicholson AG FAU - Colby, T V AU - Colby TV FAU - Renzoni, E AU - Renzoni E FAU - Maher, T M AU - Maher TM FAU - Wells, A U AU - Wells AU LA - eng PT - Journal Article DEP - 20110915 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Algorithms MH - Autoantibodies/blood MH - Biopsy MH - Connective Tissue Diseases/blood/diagnostic imaging/*mortality/pathology MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/diagnostic imaging/*mortality/pathology MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/diagnostic imaging/mortality/pathology MH - Retrospective Studies MH - Severity of Illness Index MH - Survival MH - Tomography, X-Ray Computed EDAT- 2011/09/17 06:00 MHDA- 2012/07/17 06:00 CRDT- 2011/09/17 06:00 PHST- 2011/09/17 06:00 [entrez] PHST- 2011/09/17 06:00 [pubmed] PHST- 2012/07/17 06:00 [medline] AID - 09031936.00174910 [pii] AID - 10.1183/09031936.00174910 [doi] PST - ppublish SO - Eur Respir J. 2012 Mar;39(3):661-8. doi: 10.1183/09031936.00174910. Epub 2011 Sep 15. PMID- 40325585 OWN - NLM STAT- MEDLINE DCOM- 20250506 LR - 20250507 IS - 1681-7168 (Electronic) IS - 1022-386X (Linking) VI - 35 IP - 5 DP - 2025 May TI - Frequency of Disease Subsets and Spectrum of Organ Involvement and Interstitial Lung Disease Patterns in Patients of Systemic Sclerosis in Pakistani Population. PG - 646-650 LID - 10.29271/jcpsp.2025.05.646 [doi] AB - OBJECTIVE: To assess the frequency of disease subsets and the spectrum of organ involvement, particularly focusing on interstitial lung disease (ILD) patterns among patients with systemic sclerosis (SSc). STUDY DESIGN: Observational study. Place and Duration of the Study: Institute of Rheumatic Diseases, Central Park Teaching Hospital, and its affiliated Arthritis Care Centre, Lahore, Pakistan, from July 2022 to December 2023. METHODOLOGY: Data were extracted from electronic records and gathered using a questionnaire. Patients were classified into limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc) subsets based on clinical criteria. Gender, age, duration of disease, clinical features, and ILD patterns were compared between the groups. RESULTS: The mean age of the 68 (88.3%) female patients was 37.9 ± 11.2 years. Younger patients presented with diffuse scleroderma (36 ± 11.3 years) compared to those with limited scleroderma (40.2 ± 10.7 years). The most common clinical feature was Raynaud's phenomenon (90.9%), followed by digital ulcers (41.6%). ILD was present in 68.8%, with non-specific interstitial pneumonia (NSIP) identified in 31.2%, usual interstitial pneumonia (UIP) in 19.5%, and fibrosing NSIP in 3.9%. CONCLUSION: Systemic sclerosis predominantly affects younger females, with a higher diffuse cutaneous subtype. Raynaud's phenomenon is the most common manifestation. ILD is a significant complication, with NSIP being the most common pattern observed. KEY WORDS: Systemic sclerosis, Rheumatic diseases, Limited cutaneous systematic sclerosis, Diffuse cutaneous systematic sclerosis. FAU - Arshad, Umbreen AU - Arshad U AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. FAU - Saeed, Muhammad Ahmed AU - Saeed MA AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. AD - Department of Rheumatology, National Hospital and Postgraduate Medical Centre, Lahore, Pakistan. AD - Department of Rheumatology, Arthritis Care Foundation, Lahore, Pakistan. FAU - Hameed, Muhammad Rafaqat AU - Hameed MR AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. AD - Department of Rheumatology, National Hospital and Postgraduate Medical Centre, Lahore, Pakistan. AD - Department of Rheumatology, Arthritis Care Foundation, Lahore, Pakistan. FAU - Aamer, Maryam AU - Aamer M AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. FAU - Din, Shamas U AU - Din SU AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. FAU - Qamar, Hafiz Yasir AU - Qamar HY AD - Department of Rheumatology, Central Park Teaching Hospital, Lahore, Pakistan. LA - eng PT - Journal Article PT - Observational Study PL - Pakistan TA - J Coll Physicians Surg Pak JT - Journal of the College of Physicians and Surgeons--Pakistan : JCPSP JID - 9606447 SB - IM MH - Humans MH - Female MH - Pakistan/epidemiology MH - *Lung Diseases, Interstitial/epidemiology/etiology/diagnosis MH - Male MH - Adult MH - Middle Aged MH - *Scleroderma, Systemic/complications/epidemiology MH - Raynaud Disease/epidemiology/etiology EDAT- 2025/05/06 06:30 MHDA- 2025/05/06 06:31 CRDT- 2025/05/06 00:40 PHST- 2024/03/20 00:00 [received] PHST- 2024/11/11 00:00 [accepted] PHST- 2025/05/06 06:31 [medline] PHST- 2025/05/06 06:30 [pubmed] PHST- 2025/05/06 00:40 [entrez] AID - 040579197 [pii] AID - 10.29271/jcpsp.2025.05.646 [doi] PST - ppublish SO - J Coll Physicians Surg Pak. 2025 May;35(5):646-650. doi: 10.29271/jcpsp.2025.05.646. PMID- 38466107 OWN - NLM STAT- MEDLINE DCOM- 20241024 LR - 20241024 IS - 1744-5078 (Electronic) IS - 0927-3948 (Linking) VI - 32 IP - 9 DP - 2024 Nov TI - Ocular Involvement in Systemic Sclerosis: Updated Review and New Insights on Microvascular Impairment. PG - 2209-2216 LID - 10.1080/09273948.2024.2308030 [doi] AB - Systemic sclerosis (SSc) is a chronic multisystemic disease characterized by immunological activation, diffuse vasculopathy, and generalized fibrosis exhibiting a variety of symptoms. A recognized precursor of SSc is Raynaud's phenomenon, which is part of the very early disease of systemic sclerosis (VEDOSS) in combination with nailfold videocapillaroscopy (NVC) impairment. The pathophysiology of ocular involvement, alterations in internal organs, and body integumentary system involvement in SSc patients are complicated and poorly understood, with multiple mechanisms presumptively working together. The most prevalent ocular symptoms of SSc are abnormalities of the eyelids and conjunctiva as well as dry eye syndrome, due to fibroblasts' dysfunction and inflammation of the ocular surface. In particular, lagophthalmos, blepharophimosis limitation of eyelid motion, eyelid telangiectasia, and rigidity or tightening of the lids may affect up to two-third of the patients. In addition, reduction in central corneal thickness, iris defects and higher rates of glaucoma were reported. In the first reports based on retinography or fluorescein angiography, about 50% of SSc patients showed signs of vascular disease: peripheral artery occlusion, thinning of retinal pigment epithelium and choroidal capillaries, ischemic areas surrounded by intraretinal extravasation and microaneurysms, and peripheral capillary non-perfusion. Successively, thanks to the advent of optical coherence tomography angiography (OCTA), several studies highlighted significant impairment of either the choriocapillaris and retinal vascular plexuses, also correlating with NVC involvement and skin disease, even in VEDOSS disease. Given the sensitivity of this technique, ocular micro-vasculopathy may act as a tool for early SSc identification and discriminate between disease stages. FAU - Carlà, Matteo Mario AU - Carlà MM AUID- ORCID: 0000-0003-2979-1638 AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. FAU - Gambini, Gloria AU - Gambini G AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. FAU - Caporossi, Tomaso AU - Caporossi T AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. AD - Vitreoretinal Surgery Unit, Fatebenefratelli Isola Tiberina Gemelli Isola Hospital, Rome, Italy. FAU - Giannuzzi, Federico AU - Giannuzzi F AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. FAU - Boselli, Francesco AU - Boselli F AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. FAU - Crincoli, Emanuele AU - Crincoli E AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. FAU - Ripa, Matteo AU - Ripa M AD - Department of Ophthalmology, William Harvey Hospital, East Kent Hospitals University NHS Foundation Trust, Willesborough, UK. FAU - Rizzo, Stanislao AU - Rizzo S AD - Ophthalmology Department, "Fondazione Policlinico Universitario A. Gemelli, IRCCS", Rome, Italy. AD - Ophthalmology Department, Catholic University "Sacro Cuore", Rome, Italy. LA - eng PT - Journal Article PT - Review DEP - 20240311 PL - England TA - Ocul Immunol Inflamm JT - Ocular immunology and inflammation JID - 9312169 SB - IM MH - Humans MH - *Scleroderma, Systemic/physiopathology/complications/diagnosis MH - Fluorescein Angiography/methods MH - Tomography, Optical Coherence/methods MH - Microvessels/physiopathology/pathology MH - Eye Diseases/etiology/diagnosis/physiopathology MH - Dry Eye Syndromes/physiopathology/diagnosis/etiology MH - Conjunctiva/blood supply/pathology MH - Eyelid Diseases/diagnosis/physiopathology/etiology MH - Microscopic Angioscopy/methods MH - Raynaud Disease/diagnosis/physiopathology/etiology OTO - NOTNLM OT - Dry eye disease OT - eyelid alterations OT - micro-vasculopathy OT - optical coherence tomography angiography OT - systemic sclerosis EDAT- 2024/03/11 12:43 MHDA- 2024/10/25 00:21 CRDT- 2024/03/11 10:03 PHST- 2024/10/25 00:21 [medline] PHST- 2024/03/11 12:43 [pubmed] PHST- 2024/03/11 10:03 [entrez] AID - 10.1080/09273948.2024.2308030 [doi] PST - ppublish SO - Ocul Immunol Inflamm. 2024 Nov;32(9):2209-2216. doi: 10.1080/09273948.2024.2308030. Epub 2024 Mar 11. PMID- 29745875 OWN - NLM STAT- MEDLINE DCOM- 20180920 LR - 20180920 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 IP - 4 DP - 2018 Jul-Aug TI - Interstitial lung disease in patients with mixed connective tissue disease: pilot study on predictors of lung involvement. PG - 648-651 AB - OBJECTIVES: Mixed connective tissue disease (MCTD) is an immune-mediated systemic disorder characterised by serum autoantibodies against U1-ribonucleoprotein and diverse multisystemic clinical manifestations. Approximately 50% of patients with MCTD develop a radiologic pattern of interstitial lung disease (ILD). Our single centre, cross-sectional study sought to identify clinical and serologic associations of ILD in patients with MCTD which may serve as predictors of lung disease and prognosis. METHODS: Patients who met validated criteria for diagnosis of MCTD were included in the study, and were further differentiated into study and control groups based on presence or absence of ILD. RESULTS: Multivariate logistic regression showed an association of two clinical variables: dysphagia with an R2 value of 0.33 (p-value <0.001) and Raynaud's phenomenon with R2 value of 0.28 (p-value <0.001). CONCLUSIONS: An association of dysphagia with the development of ILD in our study is in harmony with the existing literature. There are primarily case reports, suggesting an association of Raynaud's phenomenon with development of ILD in patients with undifferentiated CTD. To our knowledge, this is the first study highlighting the association of Raynaud's phenomenon with development of ILD in patients with MCTD. The mechanistic aspects of the association between Raynaud's phenomenon and ILD remain unexplored. The association of easily elicited historical and clinical features of MCTD with subtle, but worrisome, pulmonary pathology carries the promise of sensitising the unsuspecting clinician about the entity of ILD in MCTD. FAU - Narula, Neha AU - Narula N AD - Rheumatology Department, Veterans Administration Clinic, Jacksonville, FL, USA. docnehu@gmail.com. FAU - Narula, Tathagat AU - Narula T AD - Division of Lung transplant, Mayo Clinic, Jacksonville, FL, USA. FAU - Mira-Avendano, Isabel AU - Mira-Avendano I AD - Division of Pulmonary, Allergy and Sleep Medicine, Mayo Clinic, Jacksonville, FL, USA. FAU - Wang, Benjamin AU - Wang B AD - Division of Rheumatology, Mayo Clinic, Jacksonville, FL, USA. FAU - Abril, Andy AU - Abril A AD - Division of Rheumatology, Mayo Clinic, Jacksonville, FL, USA. LA - eng PT - Journal Article DEP - 20180508 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Female MH - Humans MH - Logistic Models MH - Lung Diseases, Interstitial/*etiology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications MH - Pilot Projects MH - Raynaud Disease/complications EDAT- 2018/05/11 06:00 MHDA- 2018/09/21 06:00 CRDT- 2018/05/11 06:00 PHST- 2017/11/22 00:00 [received] PHST- 2018/02/12 00:00 [accepted] PHST- 2018/05/11 06:00 [pubmed] PHST- 2018/09/21 06:00 [medline] PHST- 2018/05/11 06:00 [entrez] AID - 12452 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jul-Aug;36(4):648-651. Epub 2018 May 8. PMID- 23426043 OWN - NLM STAT- MEDLINE DCOM- 20131126 LR - 20250711 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 72 IP - 10 DP - 2013 Oct TI - Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials. PG - 1696-9 LID - 10.1136/annrheumdis-2012-202836 [doi] AB - INTRODUCTION: Recent controlled trials have assessed the efficacy of phosphodiesterase-5 (PDE-5) inhibitors in secondary Raynaud's phenomenon (RP). However, the conclusions are conflicting, and whether these drugs are effective remains unclear. The objective of this meta-analysis was to determine the efficacy of PDE-5 inhibitors on Raynaud's Condition Score (RCS) and frequency and duration of attacks. METHODS: A systematic review of articles was performed (sources included Medline, Embase, Web of Science, the Cochrane Central Register of Controlled Trials). Only double-blind, randomised controlled trials (RCTs) were included. Studies were selected independently by two authors using predefined data fields, including study quality indicators. RESULTS: Six RCTs were included (one with sildenafil, one with modified-release sildenafil, three with tadalafil and one with vardenafil). PDE-5 inhibitors significantly decreased mean RCS by -0.46 (-0.74 to -0.17) (p=0.002), the daily frequency of ischaemic attacks by -0.49 (-0.71 to -0.28) (p<0.0001), and daily duration of RP attacks by -14.62 (-20.25 to -9.00) min (p<0.0001). CONCLUSIONS: PDE-5 inhibitors appear to have significant but moderate efficacy in secondary RP. A further large RCT is needed. FAU - Roustit, Matthieu AU - Roustit M AD - Inserm U1042, Université Joseph Fourier, Grenoble, France. MRoustit@chu-grenoble.fr FAU - Blaise, Sophie AU - Blaise S FAU - Allanore, Yannick AU - Allanore Y FAU - Carpentier, Patrick H AU - Carpentier PH FAU - Caglayan, Evren AU - Caglayan E FAU - Cracowski, Jean-Luc AU - Cracowski JL LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20130220 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Carbolines) RN - 0 (Imidazoles) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Triazines) RN - 5O8R96XMH7 (Vardenafil Dihydrochloride) RN - 742SXX0ICT (Tadalafil) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Carbolines/therapeutic use MH - Humans MH - Imidazoles/therapeutic use MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Piperazines/therapeutic use MH - Purines/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate MH - Sulfones/therapeutic use MH - Tadalafil MH - Treatment Outcome MH - Triazines/therapeutic use MH - Vardenafil Dihydrochloride OTO - NOTNLM OT - Cardiovascular Disease OT - Outcomes research OT - Systemic Sclerosis EDAT- 2013/02/22 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/02/22 06:00 PHST- 2013/02/22 06:00 [entrez] PHST- 2013/02/22 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - S0003-4967(24)21351-0 [pii] AID - 10.1136/annrheumdis-2012-202836 [doi] PST - ppublish SO - Ann Rheum Dis. 2013 Oct;72(10):1696-9. doi: 10.1136/annrheumdis-2012-202836. Epub 2013 Feb 20. PMID- 29538754 OWN - NLM STAT- MEDLINE DCOM- 20191023 LR - 20200306 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 58 IP - 1 DP - 2019 Jan 1 TI - The patient experience of Raynaud's phenomenon in systemic sclerosis. PG - 18-26 LID - 10.1093/rheumatology/key026 [doi] AB - RP is the most common manifestation of SSc and a major cause of disease-related morbidity. This review provides a detailed appraisal of the patient experience of SSc-RP and potential implications for disease classification, patient-reported outcome instrument development and SSc-RP clinical trial design. The review explores the clinical features of SSc-RP, the severity and burden of SSc-RP symptoms and the impact of SSc-RP on function, work and social participation, body image dissatisfaction and health-related quality of life in SSc. Where management of SSc-RP is concerned, the review focuses on the 'patient experience' of interventions for SSc-RP, examining geographic variation in clinical practice and potential barriers to the adoption of treatment recommendations concerning best-practice management of SSc-RP. Knowledge gaps are highlighted that could form the focus of future research. A more thorough understanding of the patient experience could support the development of novel reported outcome instruments for assessing SSc-RP. FAU - Pauling, John D AU - Pauling JD AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. AD - Royal National Hospital for Rheumatic Diseases (part of Royal United Hospitals), Upper Borough Walls, Bath, UK. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - School of Medicine, University of Tulane Medical Center, New Orleans, LA, USA. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Pini, Department of Clinical Sciences and Community Health, Universita degli Studi di Milano, Milano, Italy. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, Department of Medicine, University of Michigan, Ann Arbor, MI, USA. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Body Image/psychology MH - Female MH - Humans MH - Male MH - Quality of Life MH - Raynaud Disease/etiology/*psychology MH - Scleroderma, Systemic/*complications MH - Social Participation/psychology PMC - PMC6293480 EDAT- 2018/03/15 06:00 MHDA- 2019/10/24 06:00 PMCR- 2020/01/01 CRDT- 2018/03/15 06:00 PHST- 2017/08/15 00:00 [received] PHST- 2018/03/15 06:00 [pubmed] PHST- 2019/10/24 06:00 [medline] PHST- 2018/03/15 06:00 [entrez] PHST- 2020/01/01 00:00 [pmc-release] AID - 4930650 [pii] AID - key026 [pii] AID - 10.1093/rheumatology/key026 [doi] PST - ppublish SO - Rheumatology (Oxford). 2019 Jan 1;58(1):18-26. doi: 10.1093/rheumatology/key026. PMID- 29916580 OWN - NLM STAT- MEDLINE DCOM- 20190704 LR - 20190704 IS - 1592-7830 (Print) IS - 1592-7830 (Linking) VI - 39 IP - 3 DP - 2017 Nov TI - [Guidelines for occupational exposures to mechanical vibration.]. PG - 162-165 AB - OBJECTIVES: In this study, the daily exposure action values (EAV) and the daily exposure limit values (ELV) for hand-transmitted vibration (HTV) and whole-body vibration (WBV), established by the EU Directive 2002/44/EC and the Italian Decree 81/2008, and expressed in terms of 8-hr frequency weighted r.m.s. acceleration magnitude, are discussed upon consideration of the findings of experimental and epidemiological studies. METHODS: There is some epidemiological support for the EAV A(8) of 2.5 ms-2 r.m.s. and the ELV A(8) of 5 ms-2 r.m.s. for HTV, at least for the vascular component (secondary Raynaud's phenomenon) of the hand-arm vibration syndrome. RESULTS: There is some experimental evidence for the EAV A(8) of 0.5 ms-2 r.m.s. for WBV, while there is neither biodynamic nor epidemiological validation for the ELV A(8) of 1.15 ms-2 r.m.s. for WBV, this latter lowered to 1 ms-2 r.m.s. in the Italian legislation. CI - Copyright© by Aracne Editrice, Roma, Italy. FAU - Bovenzi, Massimo AU - Bovenzi M AD - Bioengineering Department S. Maugeri Foundation, Telese Terme (BN), Italy. FAU - Mauro, Marcella AU - Mauro M AD - Bioengineering Department S. Maugeri Foundation, Telese Terme (BN), Italy. LA - ita PT - Journal Article TT - I valori guida nella valutazione del rischio da vibrazioni meccaniche. PL - Italy TA - G Ital Med Lav Ergon JT - Giornale italiano di medicina del lavoro ed ergonomia JID - 9712708 SB - IM MH - European Union MH - *Guidelines as Topic MH - Hand-Arm Vibration Syndrome/etiology/prevention & control MH - Humans MH - Italy MH - Occupational Diseases/etiology/*prevention & control MH - Occupational Exposure/*adverse effects MH - Raynaud Disease/etiology/prevention & control MH - Vibration/*adverse effects OTO - NOTNLM OT - biodynamics OT - epidemiology OT - exposure values OT - mechanical vibration COIS- The authors of this article have no conflict of interests to disclose. EDAT- 2018/06/20 06:00 MHDA- 2019/07/05 06:00 CRDT- 2018/06/20 06:00 PHST- 2016/12/14 00:00 [received] PHST- 2017/05/09 00:00 [accepted] PHST- 2018/06/20 06:00 [entrez] PHST- 2018/06/20 06:00 [pubmed] PHST- 2019/07/05 06:00 [medline] AID - 28/2016 [pii] PST - ppublish SO - G Ital Med Lav Ergon. 2017 Nov;39(3):162-165. PMID- 25854827 OWN - NLM STAT- MEDLINE DCOM- 20160721 LR - 20260128 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 24 IP - 11 DP - 2015 Oct TI - Development of mixed connective tissue disease and Sjögren's syndrome in a patient with trisomy X. PG - 1217-20 LID - 10.1177/0961203315580873 [doi] AB - Increased risk of developing systemic lupus erythematosus (SLE) has been reported in patients with Klinefelter syndrome. Here, we describe a 16-year-old Japanese patient with trisomy X (47,XXX) who developed mixed connective tissue disease (MCTD) and Sjögren's syndrome. She had polyarthritis, edematous fingers with Raynaud's phenomenon, sicca syndrome, interstitial lung disease, possible myositis, and was positive for anti-nuclear antibody, anti-nRNP antibody and rheumatoid factor. This is the first report in the literature of a case of MCTD with female polysomy X, which further supports the link between the presence of extra X chromosome(s) and the development of autoimmune diseases. CI - © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Fujimoto, M AU - Fujimoto M AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan Department of Diabetes, Metabolism and Endocrinology, Chiba University Hospital, Chiba, Japan. FAU - Ikeda, K AU - Ikeda K AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan K.Ikeda@faculty.chiba-u.jp. FAU - Nakamura, T AU - Nakamura T AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan. FAU - Iwamoto, T AU - Iwamoto T AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan. FAU - Furuta, S AU - Furuta S AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan. FAU - Nakajima, H AU - Nakajima H AD - Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20150408 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) RN - I031V2H011 (tocilizumab) RN - Triple X syndrome SB - IM MH - Adolescent MH - Antibodies, Antinuclear/analysis MH - Antibodies, Monoclonal, Humanized/administration & dosage MH - Chromosomes, Human, X MH - Female MH - Glucocorticoids/administration & dosage MH - Humans MH - Japan MH - Klinefelter Syndrome/genetics MH - Mixed Connective Tissue Disease/drug therapy/*genetics/immunology MH - Myositis/blood/pathology MH - Prednisolone/administration & dosage MH - Raynaud Disease/blood/pathology MH - Sex Chromosome Aberrations MH - Sex Chromosome Disorders of Sex Development/*complications MH - Sjogren's Syndrome/*genetics MH - Trisomy OTO - NOTNLM OT - Klinefelter’s syndrome OT - Mixed connective tissue disease OT - Sjögren’s syndrome OT - polysomy X OT - trisomy X EDAT- 2015/04/10 06:00 MHDA- 2016/07/22 06:00 CRDT- 2015/04/10 06:00 PHST- 2015/02/08 00:00 [received] PHST- 2015/03/17 00:00 [accepted] PHST- 2015/04/10 06:00 [entrez] PHST- 2015/04/10 06:00 [pubmed] PHST- 2016/07/22 06:00 [medline] AID - 0961203315580873 [pii] AID - 10.1177/0961203315580873 [doi] PST - ppublish SO - Lupus. 2015 Oct;24(11):1217-20. doi: 10.1177/0961203315580873. Epub 2015 Apr 8. PMID- 25333175 OWN - NLM STAT- MEDLINE DCOM- 20141113 LR - 20250529 VI - 17 IP - Pt 1 DP - 2014 TI - An automated system for detecting and measuring nailfold capillaries. PG - 658-65 AB - Nailfold capillaroscopy is an established qualitative technique in the assessment of patients displaying Raynaud's phenomenon. We describe a fully automated system for extracting quantitative biomarkers from capillaroscopy images, using a layered machine learning approach. On an unseen set of 455 images, the system detects and locates individual capillaries as well as human experts, and makes measurements of vessel morphology that reveal statistically significant differences between patients with (relatively benign) primary Raynaud's phenomenon, and those with potentially life-threatening systemic sclerosis. FAU - Berks, Michael AU - Berks M FAU - Tresadern, Phil AU - Tresadern P FAU - Dinsdale, Graham AU - Dinsdale G FAU - Murray, Andrea AU - Murray A FAU - Moore, Tonia AU - Moore T FAU - Herrick, Ariane AU - Herrick A FAU - Taylor, Chris AU - Taylor C LA - eng GR - 094342/WT_/Wellcome Trust/United Kingdom GR - 19465/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Germany TA - Med Image Comput Comput Assist Interv JT - Medical image computing and computer-assisted intervention : MICCAI ... International Conference on Medical Image Computing and Computer-Assisted Intervention JID - 101249582 SB - IM MH - Algorithms MH - Capillaries/*pathology MH - Humans MH - Image Enhancement/methods MH - Image Interpretation, Computer-Assisted/*methods MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply/*pathology MH - Pattern Recognition, Automated/*methods MH - Raynaud Disease/*pathology MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Subtraction Technique PMC - PMC4936512 MID - EMS69023 OID - NLM: EMS69023 EDAT- 2014/10/22 06:00 MHDA- 2014/11/14 06:00 PMCR- 2016/07/07 CRDT- 2014/10/22 06:00 PHST- 2014/10/22 06:00 [entrez] PHST- 2014/10/22 06:00 [pubmed] PHST- 2014/11/14 06:00 [medline] PHST- 2016/07/07 00:00 [pmc-release] AID - 10.1007/978-3-319-10404-1_82 [doi] PST - ppublish SO - Med Image Comput Comput Assist Interv. 2014;17(Pt 1):658-65. doi: 10.1007/978-3-319-10404-1_82. PMID- 26074390 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 42 IP - 12 DP - 2015 Dec TI - Case of H syndrome with massive skin involvement, retroperitoneal fibrosis and Raynaud's phenomenon with a novel mutation in the SLC29A3 gene. PG - 1169-71 LID - 10.1111/1346-8138.12973 [doi] AB - We describe a case of H syndrome with massive skin involvement, retroperitoneal fibrosis and Raynaud's phenomenon. A 48-year-old man with parents of a consanguineous marriage, first appeared with decreased urine output, skin sclerosis on his inner thighs and short stature (142 cm, 47 kg). The patient had suffered from hearing loss since the age of 1 year, and his secondary sexual characteristics had not developed. Computed tomography showed periaortic fibrosis, bilateral ureteral stenosis, hydronephrosis and sclerosis of the germinal cords. A biopsy from the retroperitoneal mass revealed remarkable fibrosis with chronic inflammatory cells. Biopsies from the skin lesion showed thick collagen bundles through the dermis and lymphohistiocytic infiltration with numerous plasma cells. Serum inflammatory markers, such as C-reactive protein, vascular endothelial factor, transforming growth factor-β and soluble interleukin-2 receptor, were elevated. Prednisolone was effective in treating skin lesions and in lowering serum inflammatory markers. After a long period of follow up, genomic DNA of the patient was obtained, and we identified a homozygous mutation in exon 5, c.625G>A, which caused transition of glycine to arginine, p.Gly208Arg, in the patient, but not in DNA samples from another 50 healthy individuals. This is the first case of H syndrome with Raynaud's phenomenon and retroperitoneal fibrosis, and the first Japanese case of H syndrome reported in the English published work with a novel mutation in the SLC29A3 gene. CI - © 2015 Japanese Dermatological Association. FAU - Fujita, Etsuko AU - Fujita E AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. FAU - Komine, Mayumi AU - Komine M AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. FAU - Tsuda, Hidetoshi AU - Tsuda H AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. FAU - Adachi, Akimasa AU - Adachi A AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. FAU - Murata, Satoru AU - Murata S AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. FAU - Kamata, Yasuyuki AU - Kamata Y AD - Department of Allergy & Rheumatology, Jichi Medical University, Shimotsuke, Japan. FAU - Minota, Seiji AU - Minota S AD - Department of Allergy & Rheumatology, Jichi Medical University, Shimotsuke, Japan. FAU - Ohtsuki, Mamitaro AU - Ohtsuki M AD - Department of Dermatology, Jichi Medical University, Shimotsuke, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20150613 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Nucleoside Transport Proteins) RN - 0 (SLC29A3 protein, human) RN - Histiocytosis with joint contractures and sensorineural deafness SB - IM MH - Contracture/*genetics/pathology MH - Hearing Loss, Sensorineural/*genetics/pathology MH - Histiocytosis/*genetics/pathology MH - Homozygote MH - Humans MH - Male MH - Middle Aged MH - Mutation, Missense MH - Nucleoside Transport Proteins/*genetics MH - Raynaud Disease/*genetics MH - Retroperitoneal Fibrosis/*genetics MH - Skin Diseases, Genetic/*genetics/pathology MH - Syndrome OTO - NOTNLM OT - H syndrome OT - Raynaud's phenomenon OT - SLC29A3 OT - multifocal fibrosis OT - retroperitoneal fibrosis EDAT- 2015/06/16 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/06/16 06:00 PHST- 2014/12/30 00:00 [received] PHST- 2015/04/30 00:00 [accepted] PHST- 2015/06/16 06:00 [entrez] PHST- 2015/06/16 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1111/1346-8138.12973 [doi] PST - ppublish SO - J Dermatol. 2015 Dec;42(12):1169-71. doi: 10.1111/1346-8138.12973. Epub 2015 Jun 13. PMID- 21175758 OWN - NLM STAT- MEDLINE DCOM- 20110520 LR - 20101223 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 38 IP - 1 DP - 2011 Jan TI - Dermoscopy findings of nail fold capillaries in connective tissue diseases. PG - 66-70 LID - 10.1111/j.1346-8138.2010.01092.x [doi] AB - Nail fold (video) capillaroscopy is a well-established technique to assess patients with Raynaud's phenomenon, in whom specific abnormalities of capillaries are predictive of underlying systemic sclerosis and its related diseases (scleroderma spectrum disorder). The typical abnormalities are also found in patients with dermatomyositis and those findings are useful for the assessment of vascular injury and the evaluation of therapeutic effect in patients with scleroderma spectrum disorder and dermatomyositis. Recently, it has been suggested that dermoscopy can replace the capillaroscopy in significant part for detection of nail fold capillary abnormalities. In this review, I summarized the established capillaroscopy findings in connective tissues diseases and tried to apply the findings of dermoscopy to the findings and classification of capillaroscopy. CI - © 2010 Japanese Dermatological Association. FAU - Hasegawa, Minoru AU - Hasegawa M AD - Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan. minoruha@derma.m.kanazawa-u.ac.jp LA - eng PT - Journal Article PT - Review PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - *Capillaries MH - Connective Tissue Diseases/blood/*diagnosis MH - Dermoscopy/*methods MH - Humans MH - Microscopic Angioscopy/methods MH - Nails/*blood supply MH - Raynaud Disease/diagnosis EDAT- 2010/12/24 06:00 MHDA- 2011/05/21 06:00 CRDT- 2010/12/24 06:00 PHST- 2010/12/24 06:00 [entrez] PHST- 2010/12/24 06:00 [pubmed] PHST- 2011/05/21 06:00 [medline] AID - 10.1111/j.1346-8138.2010.01092.x [doi] PST - ppublish SO - J Dermatol. 2011 Jan;38(1):66-70. doi: 10.1111/j.1346-8138.2010.01092.x. PMID- 23910615 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - A subset of systemic sclerosis but not of systemic lupus erythematosus is defined by isolated anti-Ku autoantibodies. PG - 118-21 AB - OBJECTIVES: We aimed to analyse the annual incidence of anti-Ku antibodies and to study their clinical associations in patients mainly affected by systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap diseases. METHODS: Anti-Ku were detected by counterimmunoelectrophoresis in a total of 203 sera during 14 years of anti-ENA detection. Anti-Ku+ sera belong to 46 patients, mostly affected by UCTD (12 cases), SSc spectrum diseases (13 cases), including SSc/PM, SSc/DM and SSc; and SLE spectrum diseases (9 cases), including SLE, SLE/APS, SLE/SS, SLE/PM. RESULTS: Anti-Ku antibodies represent 2% of all anti-ENA positive sera, and are found in 1.3-3% of anti-ENA positive sera per year. Anti-Ku+ SSc represents 2% of all SSc patients. All anti-Ku+ SSc spectrum diseases showed a limited cutaneous disease; myositis, dysphagia and isolated anti-Ku in more than 70% of cases. Interstitial lung disease (ILD) was found in 54% of SSc patients, frequently with mild functional impairment. When compared with other limited SSc cases, anti-Ku+ SSc showed a higher rate of male patients, arthritis, myositis and ILD. A lower rate of digital ulcers was found, although both groups showed the same rate of SSc pattern at nailfold capillaroscopy. Anti-Ku+ SLE patients (representing 1.5% of all SLE) showed cutaneous features, Raynaud's phenomenon, multiple autoantibodies, without major organ manifestations. Isolated anti-Ku+ patients significantly show a diagnosis within SSc spectrum diseases, with arthralgias, Raynaud's phenomenon, dysphagia, ILD, myositis and sclerodactyly. CONCLUSIONS: In SLE spectrum diseases, anti-Ku are found associated with other autoantibodies, without a peculiar clinical profile, except for Raynaud's phenomenon and severe alterations of capillary bed. In SSc anti-Ku are frequently associated with myositis and ILD, mostly found as isolated autoantibodies. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, piazzale Spedali Civili 1, 25100 Brescia, Italy. ilariacava@virgilio.it FAU - Fredi, Micaela AU - Fredi M FAU - Taraborelli, Mara AU - Taraborelli M FAU - Quinzanini, Marzia AU - Quinzanini M FAU - Tincani, Angela AU - Tincani A FAU - Franceschini, Franco AU - Franceschini F LA - eng PT - Journal Article DEP - 20130723 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) RN - EC 3.6.4.- (DNA Helicases) RN - EC 3.6.4.12 (XRCC5 protein, human) RN - EC 4.2.99.- (Ku Autoantigen) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood/*immunology MH - DNA Helicases/*immunology MH - Female MH - Humans MH - Ku Autoantigen MH - Lupus Erythematosus, Systemic/*epidemiology/*immunology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Myositis/epidemiology/immunology MH - Raynaud Disease/epidemiology/immunology MH - Risk Factors MH - Scleroderma, Systemic/*epidemiology/*immunology MH - Seroepidemiologic Studies MH - Young Adult EDAT- 2013/08/16 06:00 MHDA- 2013/09/11 06:00 CRDT- 2013/08/06 06:00 PHST- 2013/03/25 00:00 [received] PHST- 2013/06/21 00:00 [accepted] PHST- 2013/08/06 06:00 [entrez] PHST- 2013/08/16 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 7041 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):118-21. Epub 2013 Jul 23. PMID- 40757424 OWN - NLM STAT- MEDLINE DCOM- 20250804 LR - 20250804 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 8 DP - 2025 Aug TI - Hopelessness is associated to severity of both digital vasculopathy and lung disease in systemic sclerosis patients: a prospective one-year study. PG - 1508-1515 LID - 10.55563/clinexprheumatol/cp6fec [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune fibrosing disease with multi-organ involvement, significantly impacting quality of life. This study assessed the burden of hopelessness and its clinical and psychosocial correlates in SSc patients. METHODS: 76 SSc patients were followed prospectively over one year. Clinical assessments included Medsger Severity Score (MSS), disease activity (revised EUSTAR Activity Index), modified Rodnan skin score (mRSS), and digital ulcer (DU) presence and severity (DUCAS), Hand disability (HAMIS), Raynaud diary, and Raynaud's Condition Score (RCS). Psychosocial measures included the Beck Hopelessness Scale (BHS), 36-Item Short Form Survey (SF-36), Hospital Anxiety and Depression Scale (HADS), Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F), and Body Image Scale (BIS). Patients were stratified by BHS into mild (≤8) and moderate/severe (>8) hopelessness. RESULTS: SSc patients had significantly higher BHS scores than controls (p<0.001). Moderate/severe hopelessness was linked to more severe disease (MSS: BHS>8 = 6 [4-10] vs. ≤8 = 3 [2-5], p=0.008) and worse MSS lung scores over time (p<0.05). BHS>8 was also associated with poorer HAMIS, RCS, and ADL function. Multivariate analysis showed significant associations between hopelessness and MSS lung scores (Coeff = 0.490, CI [0.030-0.957], p=0.037), RCS (Coeff = 0.180, CI [0.029-0.329], p=0.019), and BIS (Coeff = 0.229, CI [0.165-0.292], p<0.001). In SSc patients with active DU, hopelessness correlated with DUCAS (Coeff =0.636, CI [0.033-1.239], p=0.039). CONCLUSIONS: Hopelessness is common in SSc and linked to lung severity and digital vasculopathy, highlighting the importance of targeting hand function and pulmonary disease to improve psycho-social wellbeing. FAU - Bearzi, Pietro AU - Bearzi P AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy; and Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland. FAU - Navarini, Luca AU - Navarini L AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome; and Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome, Italy. FAU - Capra Mattioli, Luigi AU - Capra Mattioli L AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy. FAU - Bindi, Gioele AU - Bindi G AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy. FAU - Minerba, Marco AU - Minerba M AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy; and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, UK. FAU - Salvolini, Chiara AU - Salvolini C AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy. FAU - Orlando, Antonio AU - Orlando A AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy. FAU - Lanaia, Giulia AU - Lanaia G AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome, Italy. FAU - Vizzini, Marta AU - Vizzini M AD - Department of Dynamic and Clinical Psychology and Health Studies, Sapienza University of Rome, Italy. FAU - Cocchiaro, Teresa AU - Cocchiaro T AD - UOC di Fisiopatologia della Riproduzione e Andrologia, Centro di Riferimento Regionale Oncofertilità, Banca degli Ovociti, Sandro Pertini Hospital, Rome, Italy. FAU - Vomero, Marta AU - Vomero M AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome, Italy. FAU - Berardicurti, Onorina AU - Berardicurti O AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome; and Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome, Italy. FAU - Currado, Damiano AU - Currado D AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome; and Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome, Italy. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. vasiliki.liakouli@unicampania.it. FAU - Marcoccia, Antonella AU - Marcoccia A AD - Centro di Riferimento Interdisciplinare, Interdipartimentale della Sclerodermia (CRIIS), Sandro Pertini Hospital, Rome, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome Campus Bio-Medico, Rome; and Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome, Italy. LA - eng PT - Journal Article DEP - 20250801 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - *Scleroderma, Systemic/psychology/diagnosis/complications/physiopathology MH - Female MH - Male MH - Prospective Studies MH - Middle Aged MH - Severity of Illness Index MH - Quality of Life MH - Adult MH - Aged MH - *Raynaud Disease/psychology/diagnosis/etiology/physiopathology MH - *Lung Diseases/psychology/diagnosis/etiology/physiopathology MH - Disability Evaluation MH - *Fingers/blood supply MH - *Hope MH - Time Factors MH - Depression/psychology EDAT- 2025/08/04 12:27 MHDA- 2025/08/04 12:28 CRDT- 2025/08/04 06:28 PHST- 2025/05/11 00:00 [received] PHST- 2025/07/21 00:00 [accepted] PHST- 2025/08/04 12:28 [medline] PHST- 2025/08/04 12:27 [pubmed] PHST- 2025/08/04 06:28 [entrez] AID - 22573 [pii] AID - 10.55563/clinexprheumatol/cp6fec [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Aug;43(8):1508-1515. doi: 10.55563/clinexprheumatol/cp6fec. Epub 2025 Aug 1. PMID- 22204922 OWN - NLM STAT- MEDLINE DCOM- 20120511 LR - 20161125 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 33 IP - 2 DP - 2012 Feb TI - [Antisynthetase syndrome: a retrospective study of 14 patients]. PG - 76-9 LID - 10.1016/j.revmed.2011.11.017 [doi] AB - PURPOSE: Antisynthetase syndrome is a rare entity characterized by myositis (dermatomyositis or polymyositis), interstitial lung disease, arthritis, Raynaud's phenomena and mechanic's hands skin manifestation, and the presence of autoantibodies against aminoacyl-transfer RNA synthetase. PATIENTS AND METHODS: Fourteen patients with antisynthetase syndrome followed-up between 1997 and 2009 were included. We studied retrospectively their clinical, radiological, and pathological findings. RESULTS: The sex ratio women/men was 2.5. Mean age at disease onset was 46 years. Arthritis (43%) and interstitial lung disease (38%) were the most frequent features at disease onset. Seven patients had myositis. Ten patients had anti-Jo1 autoantibodies, three had anti-PL7 and one anti-PL12. Corticosteroid therapy was given in all cases, immunosuppressive drugs in 12 cases, due to initial severity (n=8), disease relapse (n=3) or corticosteroid dependence (n=1). After a mean follow-up of 64 months, nine patients improved, four stabilized and one patient died after lung transplantation, required for pulmonary hypertension. CONCLUSION: The diffusion of immunologic assay will help us in the future to identify the specificity of this syndrome in order to improve care. CI - Copyright © 2011 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved. FAU - Dieval, C AU - Dieval C AD - Service de médecine interne et maladies tropicales, hôpital St-André, 1, rue Jean-Burguet, 33075 Bordeaux cedex, France. celine.dieval@chu-bordeaux.fr FAU - Ribeiro, E AU - Ribeiro E FAU - Mercié, P AU - Mercié P FAU - Blanco, P AU - Blanco P FAU - Duffau, P AU - Duffau P FAU - Longy-Boursier, M AU - Longy-Boursier M LA - fre PT - Journal Article TT - Le syndrome des antisynthétases : étude rétrospective à propos d'une série de 14 patients. DEP - 20111226 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Aged MH - Arthritis/diagnosis/immunology MH - Autoantibodies/blood MH - Biomarkers/blood MH - Drug Therapy, Combination MH - Female MH - Follow-Up Studies MH - Glucocorticoids/*therapeutic use MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/diagnosis/immunology MH - Male MH - Middle Aged MH - Myositis/*diagnosis/diagnostic imaging/drug therapy/enzymology/*immunology/pathology MH - Radiography MH - Raynaud Disease/diagnosis/immunology MH - Retrospective Studies MH - Sex Distribution MH - Treatment Outcome EDAT- 2011/12/30 06:00 MHDA- 2012/05/12 06:00 CRDT- 2011/12/30 06:00 PHST- 2011/06/03 00:00 [received] PHST- 2011/11/08 00:00 [revised] PHST- 2011/11/27 00:00 [accepted] PHST- 2011/12/30 06:00 [entrez] PHST- 2011/12/30 06:00 [pubmed] PHST- 2012/05/12 06:00 [medline] AID - S0248-8663(11)01222-7 [pii] AID - 10.1016/j.revmed.2011.11.017 [doi] PST - ppublish SO - Rev Med Interne. 2012 Feb;33(2):76-9. doi: 10.1016/j.revmed.2011.11.017. Epub 2011 Dec 26. PMID- 38291895 OWN - NLM STAT- MEDLINE DCOM- 20250130 LR - 20250530 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 2 DP - 2025 Feb 1 TI - A phase 2 trial investigating the efficacy and safety of the mPGES-1 inhibitor vipoglanstat in systemic sclerosis-related Raynaud's. PG - 704-713 LID - 10.1093/rheumatology/keae049 [doi] AB - OBJECTIVE: Our objective was to test the hypothesis, in a double-blind, placebo-controlled study that vipoglanstat, an inhibitor of microsomal prostaglandin E synthase-1 (mPGES-1), which decreases prostaglandin E2 (PGE2) and increases prostacyclin biosynthesis, improves RP. METHODS: Patients with SSc and ≥7 RP attacks during the last screening week prior to a baseline visit were randomized to 4 weeks treatment with vipoglanstat 120 mg or placebo. A daily electronic diary captured RP attacks (duration and pain) and Raynaud's Condition Score, with change in RP attacks/week as the primary end point. Cold challenge assessments were performed at baseline and end of treatment. Exploratory end points included patients' and physicians' global impression of change, Assessment of Scleroderma-associated Raynaud's Phenomenon questionnaire, mPGES-1 activity, and urinary excretion of arachidonic acid metabolites. RESULTS: Sixty-nine subjects received vipoglanstat (n = 33) or placebo (n = 36). The mean weekly number of RP attacks [baseline; vipoglanstat 14.4 (S.D. 6.7), placebo 18.2 (12.6)] decreased by 3.4 (95% CI -5.8; -1.0) and 4.2 (-6.5; -2.0) attacks per week (P = 0.628), respectively. All patient-reported outcomes improved, with no difference between the groups. The mean change in recovery of peripheral blood flow after the cold challenge did not differ between the study groups. Vipoglanstat fully inhibited mPGES-1, resulting in 57% reduction of PGE2 and 50% increase of prostacyclin metabolites in the urine. Vipoglanstat was safe and well tolerated. CONCLUSION: Although vipoglanstat was safe, and well tolerated in a dose achieving full inhibition of mPGES-1, it was ineffective in SSc-related RP. Further development and evaluation of vipoglanstat will therefore be in other diseases where mPGES-1 plays a pathogenetic role. TRIAL REGISTRATION: ClinicalTrials.gov, https://www.clinicaltrials.gov, NCT0474420. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Tornling, Göran AU - Tornling G AD - Respiratory Medicine Division, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. AD - Gesynta Pharma AB, Stockholm, Sweden. FAU - Edenius, Charlotte AU - Edenius C AD - Gesynta Pharma AB, Stockholm, Sweden. AD - Rheumatology Division, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. AD - Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. AD - Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Royal Free Hospital and University College London, London, UK. FAU - Olsson, Anna AU - Olsson A AD - Gesynta Pharma AB, Stockholm, Sweden. FAU - Kowalski, Jan AU - Kowalski J AD - JK Biostatistics AB, Stockholm, Sweden. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Centre for Musculoskeletal Research, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Anderson, Marina AU - Anderson M AD - Aintree University Hospital, Liverpool University Hospitals NHS Trust, Liverpool, UK. AD - Lancaster Medical School, Lancaster University, Lancaster, UK. FAU - Bhat, Smita AU - Bhat S AD - Ninewells Hospital and Medical School, Dundee, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatology & Musculoskeletal Medicine, and NIHR Leeds Biomedical Research Centre, University of Leeds, Leeds, UK. FAU - Hall, Frances AU - Hall F AD - Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. FAU - Korkosz, Mariusz AU - Korkosz M AD - Centrum Medyczne Pratia MCM Krakow, Krakow, Poland. AD - Jagiellonian University Medical College, Krakow, Poland. FAU - Krasowska, Dorota AU - Krasowska D AD - Department of Dermatology, Venereology and Pediatric Dermatology Medical University of Lublin, Poland. FAU - Olas, Jacek AU - Olas J AD - Małopolskie Centrum Kliniczne, Kraków, Poland. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. FAU - van Laar, Jacob M AU - van Laar JM AUID- ORCID: 0000-0001-5544-5785 AD - University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Wojteczek, Anna AU - Wojteczek A AD - Early Phase Clinical Trials Centre, Medical University of Gdańsk, Gdańsk, Poland. AD - Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng GR - Gesynta Pharma AB/ PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - EC 5.3.99.3 (Prostaglandin-E Synthases) SB - IM MH - Humans MH - Female MH - Male MH - *Raynaud Disease/drug therapy/etiology MH - Double-Blind Method MH - *Scleroderma, Systemic/complications/drug therapy MH - Middle Aged MH - Treatment Outcome MH - *Prostaglandin-E Synthases/antagonists & inhibitors MH - Adult MH - Aged PMC - PMC11781579 OTO - NOTNLM OT - Raynaud’s phenomenon OT - clinical trial OT - microsomal prostaglandin E synthase-1 OT - systemic sclerosis OT - vipoglanstat EDAT- 2024/01/31 06:43 MHDA- 2025/01/30 18:21 PMCR- 2024/01/30 CRDT- 2024/01/31 03:33 PHST- 2023/11/05 00:00 [received] PHST- 2024/01/11 00:00 [accepted] PHST- 2025/01/30 18:21 [medline] PHST- 2024/01/31 06:43 [pubmed] PHST- 2024/01/31 03:33 [entrez] PHST- 2024/01/30 00:00 [pmc-release] AID - 7593822 [pii] AID - keae049 [pii] AID - 10.1093/rheumatology/keae049 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Feb 1;64(2):704-713. doi: 10.1093/rheumatology/keae049. PMID- 39089171 OWN - NLM STAT- MEDLINE DCOM- 20240908 LR - 20250605 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 68 DP - 2024 Oct TI - Clinically relevant differences between primary Raynaud's phenomenon and secondary to connective tissue disease. PG - 152521 LID - S0049-0172(24)00161-6 [pii] LID - 10.1016/j.semarthrit.2024.152521 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) is a symptom complex associated with digital vascular compromise. Our aim was to examine for clinically relevant differences between primary RP (PRP) and secondary RP (SRP) to connective tissue disease. METHODS: We report cross-sectional results from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP), which aimed to explore the broad-ranging impact of RP. The survey was widely distributed online including via social medial. Participation was voluntary and responses were anonymous. RESULTS: 1229 respondents completed PASRAP with self-reported RP: PRP 218 (17.7 %) and SRP 1011 (82.3 %) of which 903 (92.9 %) Systemic Sclerosis. The mean (SD) age was significantly lower in respondents with PRP (41.7 [11.8] vs 54.2 [12.4] years, P<0.0001). During attacks, more subjects with SRP reported cyanotic colour changes (92.2 % vs 86.5 %, P=0.0089). Patients with PRP experienced more pain (72.1 % vs 55.9 %, P<0.0001), numbness (80.3 % vs 69.4 %, P=0.0016), stinging/throbbing (93.4 % vs 80.8 %, P<0.0001), and tingling (84.0 % vs 77.5 %, P=0.0345). Only half of respondents' symptoms were adequately controlled by their current medication(s), more commonly in SRP (55.2 % vs 45.2 %, P=0.0084). There were important differences in the triggers, number, and seasonal variation of RP attacks. CONCLUSION: There are clinically relevant differences between PRP and SRP concerning the multifaceted lived patient experience of RP. Neurosensory symptoms are more common in PRP. Patients with SRP are older and present with more colour changes, overrepresented by cyanosis, and with less complete resolution of symptoms between attacks. These data provide novel insights for future RP clinical trial design. CI - Copyright © 2024 Elsevier Inc. All rights reserved. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom. FAU - Huang, Suiyuan AU - Huang S AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA; Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, USA. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust Southmead Hospital, Bristol, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom. FAU - Sabbagh, Maya AU - Sabbagh M AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA. FAU - Hughes, Michael AU - Hughes M AD - Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK; Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: Michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Journal Article DEP - 20240718 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Raynaud Disease MH - Female MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - Adult MH - *Connective Tissue Diseases/complications MH - Aged MH - Surveys and Questionnaires MH - Scleroderma, Systemic/complications/physiopathology OTO - NOTNLM OT - Clinical trials OT - Connective tissue disease OT - Outcome measures OT - Primary raynaud's OT - Raynaud's phenomenon OT - Scleroderma OT - Secondary raynaud's OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Michael Hughes reports a relationship with Janssen Pharmaceuticals Inc, Certa Therapeutics that includes: board membership, funding grants, and speaking and lecture fees. John D Pauling reports a relationship with Boehringer Ingelheim, Astra Zenaca, Janssen, Sojournix Pharma, IsoMab and Permeatus Inc that includes: consulting or advisory and speaking and lecture fees. Francesco Del Galdo reports a relationship with Abbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab Therapeutics, Ergomed, GSK, Janssen, Mitsubishi-Tanabe that includes: consulting or advisory and funding grants. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/08/02 00:47 MHDA- 2024/09/09 00:50 CRDT- 2024/08/01 18:09 PHST- 2024/03/10 00:00 [received] PHST- 2024/06/26 00:00 [revised] PHST- 2024/07/08 00:00 [accepted] PHST- 2024/09/09 00:50 [medline] PHST- 2024/08/02 00:47 [pubmed] PHST- 2024/08/01 18:09 [entrez] AID - S0049-0172(24)00161-6 [pii] AID - 10.1016/j.semarthrit.2024.152521 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2024 Oct;68:152521. doi: 10.1016/j.semarthrit.2024.152521. Epub 2024 Jul 18. PMID- 19756834 OWN - NLM STAT- MEDLINE DCOM- 20100402 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 29 IP - 1 DP - 2010 Jan TI - Fifteen-year experience of pediatric-onset mixed connective tissue disease. PG - 53-8 LID - 10.1007/s10067-009-1276-y [doi] AB - The aim of this study was to investigate the initial clinical manifestations, laboratory data, complications, and outcomes of patients with pediatric-onset mixed connective tissue disease (MCTD) in Taiwan. We reviewed medical charts of patients younger than 18 years with a diagnosis of mixed connective tissue disease based on the criteria of Kasukawa (1) at the pediatric department of National Taiwan University Hospital from 1993 to 2008. A total of 12 patients were included. All of the patients were female. The mean age at disease onset was 10.7 years (range 6.5 to 14 years). The most common symptoms at disease onset were polyarthritis (7/12 patients) and Raynaud's phenomenon (7/12 patients). The clinical symptoms changed with time, and other symptoms encompassing the criteria for MCTD developed sequentially. Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively. In this retrospective study, sclerodermatous changes of internal organs were a poor prognostic factor in our population, and we emphasize that long-term follow-up is necessary, and appropriate treatment should be applied to improve the outcomes. FAU - Tsai, Yi-Ying AU - Tsai YY AD - Department of Pediatrics, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei, 10002, Taiwan, Republic of China. FAU - Yang, Yao-Hsu AU - Yang YH FAU - Yu, Hsin-Hui AU - Yu HH FAU - Wang, Li-Chieh AU - Wang LC FAU - Lee, Jyh-Hong AU - Lee JH FAU - Chiang, Bor-Luen AU - Chiang BL LA - eng PT - Journal Article DEP - 20090916 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Age of Onset MH - Arthritis/*complications/pathology MH - Child MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Mixed Connective Tissue Disease/complications/*pathology MH - Prognosis MH - Raynaud Disease/*complications/pathology MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications/pathology MH - Severity of Illness Index MH - Taiwan EDAT- 2009/09/17 06:00 MHDA- 2010/04/03 06:00 CRDT- 2009/09/17 06:00 PHST- 2009/06/11 00:00 [received] PHST- 2009/08/21 00:00 [accepted] PHST- 2009/08/19 00:00 [revised] PHST- 2009/09/17 06:00 [entrez] PHST- 2009/09/17 06:00 [pubmed] PHST- 2010/04/03 06:00 [medline] AID - 10.1007/s10067-009-1276-y [doi] PST - ppublish SO - Clin Rheumatol. 2010 Jan;29(1):53-8. doi: 10.1007/s10067-009-1276-y. Epub 2009 Sep 16. PMID- 30635856 OWN - NLM STAT- MEDLINE DCOM- 20200211 LR - 20200225 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 9 DP - 2019 Sep TI - Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands. PG - 2309-2318 LID - 10.1007/s10067-018-04422-z [doi] AB - OBJECTIVES: The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. METHODS: The clinical manifestations and capillaroscopy of 576 patients with scleroderma, dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, undifferentiated and mixed connective tissue diseases were evaluated and compared in patients with and without mechanic hands. RESULTS: A total of 576 patients were enrolled. Mechanic hands were observed in 17.2% of patients: 50% of mixed connective tissue disease, 35% of dermatomyositis, 15.4% of scleroderma, 14.9% of undifferentiated connective tissue disease, 14.3% of Sjogren's syndrome, and no patient with SLE. Among them, 80.8% had abnormal capillaroscopic findings. In dermatomyositis patients, Raynaud's phenomenon, anti-Jo-1 positivity, and some capillaroscopy findings were detected more frequently in patients with mechanic hand. In scleroderma, positive Scl70 and capillary loss were observed more frequently in patients without mechanic hands. CONCLUSIONS: Mechanic hands can be a presenting sign of some systemic connective tissue diseases. Probably, finding this sign on examination, especially together with Raynaud's phenomenon or abnormal capillaroscopy, can be helpful in the early diagnosis of the connective tissue diseases and can be used as a predictive and prognostic tool in future studies. FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AD - Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Fars, Iran. FAU - Habibi, Shaghayegh AU - Habibi S AUID- ORCID: 0000-0002-5209-9220 AD - Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Fars, Iran. shghyghhabibi@gmail.com. FAU - Habibi, Yasamin AU - Habibi Y AD - Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Fars, Iran. FAU - Nazarinia, Mohammadali AU - Nazarinia M AD - Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Fars, Iran. LA - eng GR - 95-01-01-11391/Shiraz University of Medical Sciences/ PT - Journal Article DEP - 20190112 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Connective Tissue Diseases/*diagnosis/diagnostic imaging MH - Cross-Sectional Studies MH - Female MH - Fingers/*diagnostic imaging MH - Humans MH - Keratosis/*diagnosis/diagnostic imaging MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply/diagnostic imaging MH - Raynaud Disease/diagnosis/diagnostic imaging OTO - NOTNLM OT - Connective tissue diseases OT - Dermatomyositis OT - Mechanic hands OT - Nailfold capillaroscopy OT - Scleroderma OT - Undifferentiated connective tissue disease EDAT- 2019/01/13 06:00 MHDA- 2020/02/12 06:00 CRDT- 2019/01/13 06:00 PHST- 2018/10/15 00:00 [received] PHST- 2018/12/27 00:00 [accepted] PHST- 2018/12/17 00:00 [revised] PHST- 2019/01/13 06:00 [pubmed] PHST- 2020/02/12 06:00 [medline] PHST- 2019/01/13 06:00 [entrez] AID - 10.1007/s10067-018-04422-z [pii] AID - 10.1007/s10067-018-04422-z [doi] PST - ppublish SO - Clin Rheumatol. 2019 Sep;38(9):2309-2318. doi: 10.1007/s10067-018-04422-z. Epub 2019 Jan 12. PMID- 17990152 OWN - NLM STAT- MEDLINE DCOM- 20080117 LR - 20221212 IS - 1424-7860 (Print) IS - 0036-7672 (Linking) VI - 137 IP - 41-42 DP - 2007 Oct 20 TI - Clinical diagnosis compared to classification criteria in in a cohort of 54 patients with systemic sclerosis and associated disorders. PG - 586-90 AB - OBJECTIVE: To compare clinical diagnosis with two validated classification criteria for systemic sclerosis (SSc) in a cohort of Swiss patients with SSc and associated disorders. METHODS: Charts of 54 patients with SSc and associated disorders were reviewed and compared with data obtained at a thorough clinical examination using a standardised protocol (Raynaud's phenomen [RP], skin involvement, nailfold capillary microscopy and determination of autoantibody pattern). RESULTS: According to patient records 6 patients had diffuse cutaneous SSc (dcSSc), 23 limited cutaneous SSc (lcSSc) and 20 were not classified. Two patients had mixed connective tissue disease (MCTD) and 3 overlap syndromes. At the time of clinical examination, 7 patients showed dcSSc (6 plus 1 patient originally classified as lcSSc), 26 lcSSc (20 plus 6 originally not classified) and 16 patients had severe RP which was arbitrarily classified as Raynaud's syndrome (RS). 15 of the latter 16 were antinuclear antibody positive and 7 exhibited pathological nailfold capillaries. On the basis of LeRoy and Medsger's criteria, 6 of these patients could be further classified as limited SSc (lSSc). Of 49 sera tested, 14 contained centromere antibodies at clinical examination, 16 Scl-70, 5 RNA-pol, 1 Ku, 12 antibodies with unknown specificity, and one serum was autoantibody negative. CONCLUSIONS: A substantial number of patients with minor cutaneous manifestations do not fulfil ACR classification criteria, though they have typical clinical signs of SSc. Characteristic features in these patients are presence of Raynaud's phenomenon, antinuclear antibodies and pathological changes in nailfold capillary microscopy. Application of the diagnostic criteria recently proposed by LeRoy and Medsger makes it possible to name many of these patients. The use of these criteria is recommended for clinical management. FAU - Ziswiler, Hans-Rudolf AU - Ziswiler HR AD - Department of Rheumatology, Clinical Immunology and Allergology, University Hospital, Bern, Switzerland. hansruedi.ziswiler@insel.ch FAU - Urech, Romana AU - Urech R FAU - Balmer, Judith AU - Balmer J FAU - Ostensen, Monika AU - Ostensen M FAU - Mierau, Rudolf AU - Mierau R FAU - Villiger, Peter M AU - Villiger PM LA - eng PT - Comparative Study PT - Journal Article PL - Switzerland TA - Swiss Med Wkly JT - Swiss medical weekly JID - 100970884 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear MH - Female MH - Health Status Indicators MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/diagnosis/physiopathology MH - Scleroderma, Systemic/classification/*diagnosis/physiopathology MH - Severity of Illness Index MH - Switzerland EDAT- 2007/11/09 09:00 MHDA- 2008/01/18 09:00 CRDT- 2007/11/09 09:00 PHST- 2007/11/09 09:00 [pubmed] PHST- 2008/01/18 09:00 [medline] PHST- 2007/11/09 09:00 [entrez] AID - smw-11900 [pii] AID - 10.4414/smw.2007.11900 [doi] PST - ppublish SO - Swiss Med Wkly. 2007 Oct 20;137(41-42):586-90. doi: 10.4414/smw.2007.11900. PMID- 35673285 OWN - NLM STAT- MEDLINE DCOM- 20220609 LR - 20220716 IS - 2075-0528 (Electronic) IS - 2075-051X (Print) IS - 2075-051X (Linking) VI - 22 IP - 2 DP - 2022 May TI - Is there any Sympathetic Skin Response Abnormality in Raynaud Phenomenon? PG - 274-279 LID - 10.18295/squmj.4.2021.066 [doi] AB - OBJECTIVES: This study aimed to evaluate sympathetic skin response (SSR) among patients with Raynaud phenomenon (RP). SSR is a technique for assessment of the damage of peripheral neuropathies and the disorders of the sympathetic system. METHODS: Between January 2015 and December 2018, approximately 20 patients with RP and 20 healthy subjects (control group) were recruited from patients referred to the outpatient clinics of Shiraz University of Medical Sciences, Shiraz, Iran. All participants were clinically examined and the SSR was evaluated using a standard protocol. SSR is abnormal when the latency is prolonged and/or the amplitude is reduced. RESULTS: The RP group consisted of 19 women (95%) and one male (5%); three patients (15%) had primary Raynaud's phenomenon (PRP) and 17 patients (85%) had secondary Raynaud's phenomenon. The control group consisted of 16 women (80%) and four males (20%). The mean age of the RP group and control subjects was 43.1 ± 9 and 36.7 ± 8.6 years, respectively. The SSR to the electrical stimulus was absent in three patients with PRP. The total median nerve mean latencies in the upper limb were 1.90 ± 0.57 and 1.19 ± 0.52 seconds for the RP group and control groups, respectively (P <0.001). These findings revealed significantly prolonged SSR latencies in the RP group, while the mean amplitude showed no significant differences in both groups (P = 0.756). CONCLUSION: Absence or prolonged latency of SSR was associated with the disorders of the unmyelinated axons in the sympathetic system. The findings of the present study suggested the disorders of unmyelinated axons in Raynaud's phenomenon. CI - © Copyright 2022, Sultan Qaboos University Medical Journal, All Rights Reserved. FAU - Emad, Mohammad R AU - Emad MR AD - Bone and Joint Diseases Research Center, Department of Physical Medicine and Rehabilitation, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Farpour, Hamid R AU - Farpour HR AD - Bone and Joint Diseases Research Center, Department of Physical Medicine and Rehabilitation, Shiraz University of Medical Sciences, Shiraz, Iran. AD - Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Ahmed, Faisal AU - Ahmed F AD - Urology Research Center, Al-Thora General Hospital, Department of Urology, Ibb University of Medical Science, Ibb, Yemen. FAU - Tayebi, Masoumeh AU - Tayebi M AD - Bone and Joint Diseases Research Center, Department of Physical Medicine and Rehabilitation, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Nazarinia, Mohammadali AU - Nazarinia M AD - Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. AD - Department of Rheumatology, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Askarpour, Mohammad R AU - Askarpour MR AD - Department of Urology, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Nikbakht, Hossein-Ali AU - Nikbakht HA AD - Department of Biostatics and Epidemiology, Babol University of Medical Science, Babol, Iran. LA - eng PT - Journal Article DEP - 20220526 PL - Oman TA - Sultan Qaboos Univ Med J JT - Sultan Qaboos University medical journal JID - 101519915 SB - IM MH - Female MH - Humans MH - Iran MH - Male MH - Median Nerve MH - *Peripheral Nervous System Diseases MH - *Raynaud Disease/diagnosis MH - Reaction Time/physiology PMC - PMC9155028 OTO - NOTNLM OT - Autonomic Nervous System OT - Electrodiagnosis OT - Iran OT - Nerve Conduction OT - Raynaud Disease OT - Sympathetic Fibers COIS- CONFLICT OF INTEREST The authors declare no conflicts of interest. EDAT- 2022/06/09 06:00 MHDA- 2022/06/10 06:00 PMCR- 2022/05/01 CRDT- 2022/06/08 01:54 PHST- 2020/09/07 00:00 [received] PHST- 2020/12/02 00:00 [revised] PHST- 2021/02/16 00:00 [revised] PHST- 2021/02/23 00:00 [accepted] PHST- 2022/06/08 01:54 [entrez] PHST- 2022/06/09 06:00 [pubmed] PHST- 2022/06/10 06:00 [medline] PHST- 2022/05/01 00:00 [pmc-release] AID - squmj2205-274-279 [pii] AID - 10.18295/squmj.4.2021.066 [doi] PST - ppublish SO - Sultan Qaboos Univ Med J. 2022 May;22(2):274-279. doi: 10.18295/squmj.4.2021.066. Epub 2022 May 26. PMID- 20302639 OWN - NLM STAT- MEDLINE DCOM- 20100923 LR - 20211020 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 12 IP - 2 DP - 2010 TI - Anticentromere antibody positive Sjögren's Syndrome: a retrospective descriptive analysis. PG - R47 LID - 10.1186/ar2958 [doi] AB - INTRODUCTION: A subgroup of patients with primary Sjögren's Syndrome (SS) and positive anticentromere antibodies (ACA) were recognized as having features intermediate between SS and systemic sclerosis (SSc). Our goal was to describe this group clinically and serologically and define its tendency to evolve to full blown SSc. METHODS: Among 535 patients with primary SS we identified 20 ACA positive (ACA+/SS). We compared them to 61 randomly selected ACA negative SS patients (ACA-/SS), 31 ACA positive SSc patients with sicca manifestations [SSc/(+) sicca] and 20 ACA positive SSc patients without sicca manifestations [SSc/(-) sicca]. RESULTS: Prevalence of ACA among SS patients was 3.7%. Cases and controls did not differ in sex ratio and age at disease onset. ACA+/SS patients had a lower prevalence of dry eyes, hypergammaglobulinaemia, anti-Ro and anti-La antibodies and a higher prevalence of Raynaud's phenomenon and dysphagia compared to ACA-/SS patients. They also had lower prevalence of telangiectasias, puffy fingers, sclerodactyly, Raynaud's phenomenon, digital ulcers and gastroesophageal reflux in comparison to both of the SSc subgroups and a lower prevalence of dyspnoea and lung fibrosis compared to the SSc/(+) sicca subgroup. Two patients originally having ACA+/SS evolved to full blown SSc. Four deaths occurred, all among SSc patients. Kaplan Meier analysis showed a significant difference between cases and controls in time from disease onset to development of gastroesophageal reflux, telangiectasias, digital ulcers, arthritis, puffy fingers, xerostomia, hypergammaglobulinaemia and dysphagia. CONCLUSIONS: ACA+/SS has a clinical phenotype intermediate between ACA-/SS and SSc and shows little tendency to evolve to SSc. FAU - Bournia, Vasiliki-Kalliopi K AU - Bournia VK AD - Department of Pathophysiology, Medical School, National and Kapodistrian University, 75 Mikras Asias Street, 11527, Athens, Greece. FAU - Diamanti, Konstantina D AU - Diamanti KD FAU - Vlachoyiannopoulos, Panayiotis G AU - Vlachoyiannopoulos PG FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100313 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Autoantibodies) SB - IM CIN - Arthritis Res Ther. 2010;12(5):406; author reply 407. doi: 10.1186/ar3153. PMID: 21067558 MH - Adult MH - Autoantibodies/*blood MH - Centromere/*immunology MH - Deglutition Disorders/complications/immunology MH - Female MH - Humans MH - Keratoconjunctivitis Sicca/blood/complications/immunology MH - Male MH - Middle Aged MH - Raynaud Disease/complications/immunology MH - Retrospective Studies MH - Scleroderma, Systemic/blood/complications/*immunology MH - Sjogren's Syndrome/blood/complications/*immunology PMC - PMC2888196 EDAT- 2010/03/23 06:00 MHDA- 2010/09/24 06:00 PMCR- 2010/03/13 CRDT- 2010/03/23 06:00 PHST- 2010/01/08 00:00 [received] PHST- 2010/02/23 00:00 [revised] PHST- 2010/03/13 00:00 [accepted] PHST- 2010/03/23 06:00 [entrez] PHST- 2010/03/23 06:00 [pubmed] PHST- 2010/09/24 06:00 [medline] PHST- 2010/03/13 00:00 [pmc-release] AID - ar2958 [pii] AID - 10.1186/ar2958 [doi] PST - ppublish SO - Arthritis Res Ther. 2010;12(2):R47. doi: 10.1186/ar2958. Epub 2010 Mar 13. PMID- 36426766 OWN - NLM STAT- MEDLINE DCOM- 20221214 LR - 20260513 IS - 1526-4610 (Electronic) IS - 0017-8748 (Linking) VI - 62 IP - 10 DP - 2022 Nov TI - Raynaud's phenomenon associated with calcitonin gene-related peptide receptor antagonists case report. PG - 1419-1423 LID - 10.1111/head.14417 [doi] AB - BACKGROUND: Calcitonin gene-related peptide (CGRP) is a potent vasodilator that regulates the cerebrovascular and peripheral circulation. A new class of migraine therapies decreases CGRP through various mechanisms. One unknown off-target effect is the impact decreasing CGRP will have on the peripheral circulation. The following cases report new onset Raynaud's phenomenon (RP) following the use of CGRP receptor antagonists (-gepants) for both the acute and preventive treatment of migraine. These cases describe the development of RP in two individuals after using each of the currently available gepant medications. To our knowledge these are the first cases reported of RP associated with the use of gepants. RP has previously been reported in association with monoclonal antibodies to the CGRP ligand and CGRP receptor indicated in the prevention of migraine. CASE PRESENTATION: One case involved oral CGRP receptor antagonists for acute treatment inducing RP. In this case, rimegepant and ubrogepant used separately for different migraine attacks each led to RP in the digits. The other case involved oral CGRP receptor antagonist, atogepant, used as a preventive treatment and induced RP in the digits. This patient had a prior history of areolar tissue RP while breastfeeding, but never in her fingers. In both cases, the offending medications were discontinued, and the patients reported no further episodes of RP. CONCLUSION: Two cases are reported of people with migraine with new onset digital RP while taking CGRP receptor antagonists (rimegepant, ubrogepant, atogepant) for acute and preventive treatment. CI - © 2022 American Headache Society. FAU - Bedrin, Kate AU - Bedrin K AUID- ORCID: 0000-0003-0715-9949 AD - Medstar Georgetown Headache Center, Department of Neurology, MedStar Georgetown University Hospital, Washington, District of Columbia, USA. FAU - Ailani, Jessica AU - Ailani J AD - Medstar Georgetown Headache Center, Department of Neurology, MedStar Georgetown University Hospital, Washington, District of Columbia, USA. FAU - Dougherty, Carrie AU - Dougherty C AD - Medstar Georgetown Headache Center, Department of Neurology, MedStar Georgetown University Hospital, Washington, District of Columbia, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20221125 PL - United States TA - Headache JT - Headache JID - 2985091R RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - 0 (Receptors, Calcitonin Gene-Related Peptide) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) RN - 7CRV8RR151 (atogepant) RN - 0 (Piperidines) RN - 0 (Pyridines) RN - 0 (Pyrroles) RN - 0 (Spiro Compounds) SB - IM MH - Humans MH - Female MH - Calcitonin Gene-Related Peptide Receptor Antagonists/pharmacology MH - Receptors, Calcitonin Gene-Related Peptide MH - Calcitonin Gene-Related Peptide MH - *Migraine Disorders/drug therapy MH - *Raynaud Disease/chemically induced MH - Piperidines MH - Pyridines MH - Pyrroles MH - Spiro Compounds OTO - NOTNLM OT - Raynaud's phenomenon OT - atogepant OT - calcitonin gene-related peptide receptor antagonist OT - case report OT - rimegepant OT - ubrogepant EDAT- 2022/11/26 06:00 MHDA- 2022/12/15 06:00 CRDT- 2022/11/25 06:33 PHST- 2022/09/09 00:00 [revised] PHST- 2022/05/05 00:00 [received] PHST- 2022/09/12 00:00 [accepted] PHST- 2022/11/26 06:00 [pubmed] PHST- 2022/12/15 06:00 [medline] PHST- 2022/11/25 06:33 [entrez] AID - 10.1111/head.14417 [doi] PST - ppublish SO - Headache. 2022 Nov;62(10):1419-1423. doi: 10.1111/head.14417. Epub 2022 Nov 25. PMID- 37758266 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20241119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 8 DP - 2024 Aug 1 TI - Digital artery reconstruction with interposition vein graft: impact on secondary Raynaud's phenomenon. PG - 2123-2127 LID - 10.1093/rheumatology/kead524 [doi] AB - OBJECTIVE: Patients with secondary RP show a wide range of symptoms depending on the condition of vascular structures. If the symptoms are localized to specific fingers and angiography reveals a discrete segment of occlusion of a proper digital artery, we perform proper digital artery reconstruction with an interposition vein graft. The objective of this study was to evaluate the results of the surgery in patients with chronic hand ischaemia. METHODS: A retrospective chart review was performed on patients who underwent proper digital artery reconstruction. Each digit that underwent grafting was analysed separately. The results of surgical intervention and recurrence based on patient symptoms were evaluated. Cox proportional hazards regression models were used to identify independent risk factors associated with recurrence, and the Kaplan-Meier method was used to predict the 5-year recurrence-free rate. RESULTS: A total of 79 digits from 57 patients were included in this study. The majority of patients demonstrated resolution of ischaemic pain and ulceration (97.5% and 95.3%, respectively). Recurrence occurred in 16 (20.3%) patients during the follow-up period. In two cases (2.5%) surgery had no effect. In the multivariate Cox regression analysis, smoking and concomitant periarterial sympathectomy were significant factors associated with recurrence. In the Kaplan-Meier analysis, the 5-year recurrence-free rate in the total study population was 69.3%. CONCLUSIONS: Digital artery reconstruction using an interposition vein graft is an effective procedure for improving ischaemic pain and ulceration in patients with RP. Smoking and concomitant periarterial sympathectomy were significantly associated with recurrence. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Park, Seong Oh AU - Park SO AUID- ORCID: 0000-0001-8990-0635 AD - Department of Plastic and Reconstructive Surgery, Hanyang University College of Medicine, Seoul, Korea. FAU - Wang, Hyung Woo AU - Wang HW AD - Department of Plastic and Reconstructive Surgery, Hanyang University College of Medicine, Seoul, Korea. FAU - Han, Youngseo AU - Han Y AD - Department of Medicine, Hanyang University College of Medicine, Seoul, Korea. FAU - Ahn, Hee Chang AU - Ahn HC AD - Department of Plastic and Reconstructive Surgery, CHA University Bundang Medical Center, Seongnam-si, Gyeonggi-do, Korea. LA - eng GR - 2021R1G1A1006221/NRF/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Female MH - Male MH - Middle Aged MH - Retrospective Studies MH - *Raynaud Disease/surgery/etiology MH - *Fingers/blood supply/surgery MH - Adult MH - Aged MH - Treatment Outcome MH - Recurrence MH - Veins/transplantation MH - Ischemia/surgery/etiology MH - Plastic Surgery Procedures/methods MH - Kaplan-Meier Estimate MH - Arteries/transplantation MH - Risk Factors MH - Proportional Hazards Models OTO - NOTNLM OT - Raynaud disease OT - amputation OT - arterial occlusive disease OT - ischemia OT - sympathectomy EDAT- 2023/09/28 00:42 MHDA- 2024/08/01 06:42 CRDT- 2023/09/27 20:13 PHST- 2023/07/14 00:00 [received] PHST- 2023/09/18 00:00 [accepted] PHST- 2024/08/01 06:42 [medline] PHST- 2023/09/28 00:42 [pubmed] PHST- 2023/09/27 20:13 [entrez] AID - 7284106 [pii] AID - 10.1093/rheumatology/kead524 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Aug 1;63(8):2123-2127. doi: 10.1093/rheumatology/kead524. PMID- 19248104 OWN - NLM STAT- MEDLINE DCOM- 20090427 LR - 20131121 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 60 IP - 3 DP - 2009 Mar TI - MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud's phenomenon: a randomized, controlled trial. PG - 870-7 LID - 10.1002/art.24351 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) affects 3-9% of the general population and >90% of patients with systemic sclerosis. Nitrates are often prescribed for the treatment of RP, but currently available formulations are limited by side effects, particularly headaches, dizziness, and skin irritation. The purpose of this study was to evaluate the tolerability and efficacy of a novel formulation of topical nitroglycerin, MQX-503, in the treatment of RP in an ambulatory setting. METHODS: We conducted a multicenter, randomized, placebo-controlled study with a 2-week single-blind run-in period to determine baseline severity, followed by a 4-week double-blind treatment phase. Two hundred nineteen adult patients with a clinical diagnosis of primary or secondary RP received 0.9% MQX-503 gel or matching placebo during the treatment period. Gel was applied immediately before or within 5 minutes of the beginning of an episode of RP (maximum of 4 applications daily). End points included the change in the mean Raynaud's Condition Score (RCS; scale 0-10), the frequency and duration of episodes, and subjective assessments at the target week (the week during the treatment phase that most closely matched the run-in period in terms of ambient temperature) compared with baseline. RESULTS: The mean (%) change in the RCS at the target week compared with baseline was significantly greater in the MQX-503 group (0.48 [14.3%]) than that in the placebo group (0.04 [1.3%]; P = 0.04). Changes in the frequency and duration of RP episodes and subjective assessments were not statistically different between the groups. MQX-503 had a side effect profile similar to that of placebo. CONCLUSION: MQX-503 is well tolerated and more effective than placebo for the treatment of RP. FAU - Chung, Lorinda AU - Chung L AD - Stanford University, Stanford, California, and VA Palo Alto Health Care System, Palo Alto, CA 94304, USA. shauwei@stanford.edu FAU - Shapiro, Lee AU - Shapiro L FAU - Fiorentino, David AU - Fiorentino D FAU - Baron, Murray AU - Baron M FAU - Shanahan, Joseph AU - Shanahan J FAU - Sule, Sangeeta AU - Sule S FAU - Hsu, Vivien AU - Hsu V FAU - Rothfield, Naomi AU - Rothfield N FAU - Steen, Virginia AU - Steen V FAU - Martin, Richard W AU - Martin RW FAU - Smith, Edwin AU - Smith E FAU - Mayes, Maureen AU - Mayes M FAU - Simms, Robert AU - Simms R FAU - Pope, Janet AU - Pope J FAU - Kahaleh, Bashar AU - Kahaleh B FAU - Csuka, M E AU - Csuka ME FAU - Gruber, Barry AU - Gruber B FAU - Collier, David AU - Collier D FAU - Sweiss, Nadera AU - Sweiss N FAU - Gilbert, Adam AU - Gilbert A FAU - Dechow, Frederick J AU - Dechow FJ FAU - Gregory, Jeffrey AU - Gregory J FAU - Wigley, Fredrick M AU - Wigley FM LA - eng PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM CIN - Nat Rev Rheumatol. 2009 May;5(5):246-7. doi: 10.1038/nrrheum.2009.64. PMID: 19412190 MH - Administration, Topical MH - Adult MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nitroglycerin/administration & dosage/adverse effects/*therapeutic use MH - Raynaud Disease/*drug therapy/pathology MH - Severity of Illness Index MH - Single-Blind Method MH - Vasodilator Agents/administration & dosage/adverse effects/*therapeutic use EDAT- 2009/02/28 09:00 MHDA- 2009/04/28 09:00 CRDT- 2009/02/28 09:00 PHST- 2009/02/28 09:00 [entrez] PHST- 2009/02/28 09:00 [pubmed] PHST- 2009/04/28 09:00 [medline] AID - 10.1002/art.24351 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Mar;60(3):870-7. doi: 10.1002/art.24351. PMID- 35131401 OWN - NLM STAT- MEDLINE DCOM- 20221019 LR - 20221206 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 87 IP - 5 DP - 2022 Nov TI - Systemic sclerosis in adults. Part II: management and therapeutics. PG - 957-978 LID - S0190-9622(22)00191-8 [pii] LID - 10.1016/j.jaad.2021.10.066 [doi] AB - The management of systemic sclerosis (SSc) is complex, evolving, and requires a multidisciplinary approach. At diagnosis and throughout the disease course, clinical assessment and monitoring of skin involvement is vital using the modified Rodnan Skin Score, patient-reported outcomes, and new global composite scores (such as the Combined Response Index for Systemic Sclerosis, which also considers lung function). Immunomodulation is the mainstay of skin fibrosis treatment, with mycophenolate mofetil considered first line. Meanwhile vasculopathy-related manifestations (Raynaud's phenomenon, digital ulcers) and calcinosis, require general measures combined with specific pharmacologic (calcium-channel blockers, phosphodiesterase type 5 inhibitors, and prostanoids), nonpharmacologic (digital sympathectomy and botulinum toxin injections), and often multifaceted, management approaches. Patients should be screened at the time of diagnosis specifically for systemic manifestations and then regularly thereafter, with appropriate treatment. Numerous targeted therapeutic options for SSc, including skin fibrosis, are emerging and include B-cell depletion, anti-interleukin 6, Janus kinase, and transforming growth factor β inhibition. This second article in the continuing medical education series discusses these key aspects of SSc assessment and treatment, with particular focus on skin involvement. It is vital that dermatologists play a key role in the multidisciplinary approach to SSc management. CI - Copyright © 2022. Published by Elsevier Inc. FAU - Jerjen, Rebekka AU - Jerjen R AD - Department of Dermatology, The Alfred Hospital, Melbourne, Australia. FAU - Nikpour, Mandana AU - Nikpour M AD - Department of Rheumatology, St Vincent's Hospital, Melbourne, Australia; Department of Medicine, The University of Melbourne, Melbourne, Australia. FAU - Krieg, Thomas AU - Krieg T AD - Department Dermatology and Translational Matrix Biology, CMMC and CECAD, Faculty of Medicine, University of Cologne, Cologne, Germany. FAU - Denton, Christopher P AU - Denton CP AD - Division of Medicine, Centre for Rheumatology and Connective Tissues Diseases, University College London, London, United Kingdom; Department of Rheumatology, Royal Free NHS Foundation Trust, London, United Kingdom. FAU - Saracino, Amanda M AU - Saracino AM AD - Department of Dermatology, The Alfred Hospital, Melbourne, Australia; Department of Medicine, Monash University, Melbourne, Australia. Electronic address: a.saracino@monash.edu.au. LA - eng PT - Journal Article PT - Review DEP - 20220204 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins) RN - 0 (Transforming Growth Factor beta) RN - EC 2.7.10.2 (Janus Kinases) RN - EC 3.4.24.69 (Botulinum Toxins) RN - HU9DX48N0T (Mycophenolic Acid) RN - SY7Q814VUP (Calcium) SB - IM MH - Adult MH - *Botulinum Toxins/therapeutic use MH - Calcium/therapeutic use MH - Fibrosis MH - Humans MH - Janus Kinases MH - Mycophenolic Acid/therapeutic use MH - Phosphodiesterase 5 Inhibitors MH - Prostaglandins/therapeutic use MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/diagnosis/therapy MH - *Skin Ulcer/drug therapy MH - Transforming Growth Factor beta OTO - NOTNLM OT - Raynaud's phenomenon OT - calcinocic cutis OT - digital ulcers OT - management OT - systemic sclerosis OT - treatment COIS- Conflicts of interest Dr Nikpour has received research grant support from Actelion, BMS, GSK, Janssen, and UCB and honoraria from Actelion, Boehringer Ingelheim, Janssen, Eli Lilly, Pfizer, and UCB. Dr Krieg has received speaking fees from Actelion. Dr Denton reports personal fees or research grants to his institution from GlaxoSmithKline, Galapagos, Boehringer Ingelheim, Roche, CSL Behring, Corbus, Horizon, and Arxx Therapeutics outside the submitted work. Dr Saracino has received speaking fees from UCB. Dr Jerjen has no conflict of interest to declare. EDAT- 2022/02/09 06:00 MHDA- 2022/10/20 06:00 CRDT- 2022/02/08 05:41 PHST- 2021/07/23 00:00 [received] PHST- 2021/10/14 00:00 [revised] PHST- 2021/10/26 00:00 [accepted] PHST- 2022/02/09 06:00 [pubmed] PHST- 2022/10/20 06:00 [medline] PHST- 2022/02/08 05:41 [entrez] AID - S0190-9622(22)00191-8 [pii] AID - 10.1016/j.jaad.2021.10.066 [doi] PST - ppublish SO - J Am Acad Dermatol. 2022 Nov;87(5):957-978. doi: 10.1016/j.jaad.2021.10.066. Epub 2022 Feb 4. PMID- 17631741 OWN - NLM STAT- MEDLINE DCOM- 20070830 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 25 IP - 3 DP - 2007 May-Jun TI - Angiotensin-converting enzyme gene polymorphism in Kuwaiti patients with systemic lupus erythematosus. PG - 437-42 AB - OBJECTIVE: To investigate the frequency of angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism genotypes in patients with systemic lupus erythematosus (SLE), and to study the correlation between I/D polymorphism of the ACE gene and the clinical manifestations of SLE, especially vascular involvement, lupus nephritis and disease severity. METHODS: The frequency of ACE gene I/D polymorphism genotypes was determined in 92 patients with SLE from Kuwait, and compared to that in 100 ethnically matched healthy controls using the polymerase chain reaction. RESULTS: The distribution of ACE I/D polymorphism and allele frequencies in SLE patients was not significantly different from controls. Further analyses of SLE patients showed that there was a significant association between DD genotype and Raynaud's phenomenon (p=0.008, odd ratio=5.4, 95% confidence interval: 1.6-18.6). However, there was no significant association between the ACE genotype and lupus nephritis or disease severity. CONCLUSION: No difference was found between the distribution of the ACE genotype in SLE patients and the general pop-ulation in Kuwait. However, the presence of the DD genotype may confer susceptibility to the development of vascular morbidity. FAU - Al-Awadhi, A M AU - Al-Awadhi AM AD - Department of Medicine, Faculty of Medicine, Health Sciences Center, Kuwait University, and Rheumatic Disease Unit, Al-Amiri Hospital, Kuwait. aalawadhi@hsc.edu.kw FAU - Haider, M Z AU - Haider MZ FAU - Sharma, P N AU - Sharma PN FAU - Hasan, E A AU - Hasan EA FAU - Botaiban, F AU - Botaiban F FAU - Al-Herz, A AU - Al-Herz A FAU - Nahar, I AU - Nahar I FAU - Al-Enezi, H AU - Al-Enezi H FAU - Al-Saeid, K AU - Al-Saeid K LA - eng PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - EC 3.4.15.1 (Peptidyl-Dipeptidase A) SB - IM MH - Adult MH - Case-Control Studies MH - Female MH - Gene Expression Regulation, Enzymologic MH - Gene Frequency MH - Genetic Predisposition to Disease/ethnology/genetics MH - Genotype MH - Humans MH - Kuwait MH - Lupus Erythematosus, Systemic/ethnology/*genetics MH - Lupus Nephritis/ethnology/genetics MH - Male MH - Middle Aged MH - Peptidyl-Dipeptidase A/*genetics MH - *Polymorphism, Genetic MH - Raynaud Disease/ethnology/genetics MH - Severity of Illness Index EDAT- 2007/07/17 09:00 MHDA- 2007/08/31 09:00 CRDT- 2007/07/17 09:00 PHST- 2007/07/17 09:00 [pubmed] PHST- 2007/08/31 09:00 [medline] PHST- 2007/07/17 09:00 [entrez] AID - 2072 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2007 May-Jun;25(3):437-42. PMID- 26242276 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20181113 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 33 IP - 4 Suppl 91 DP - 2015 Jul-Aug TI - Pilot study to determine whether transient receptor potential melastatin type 8 (TRPM8) antibodies are detected in scleroderma. PG - S123-6 AB - OBJECTIVES: A key mediator in cold-sensation is the protein transient receptor potential melastatin 8 (TRPM8), which is expressed on sensory nerves and cutaneous blood vessels. These receptors are activated by cold temperatures and play a key role in body thermoregulation. Cold sensitivity and Raynaud's phenomenon are frequent clinical features in scleroderma, and are thought to be secondary to a local defect in cutaneous thermoregulation. We investigated whether autoantibodies targeting TRPM8 were present in the sera of patients with scleroderma as evidence for a possible mechanism for an acquired immune mediated defect in thermoregulation. METHODS: Sera from 50 well-characterised scleroderma patients with Raynaud's phenomenon were studied. TRPM8 autoantibodies were assayed as follows: 1. immunoprecipitation with 35S-methionine-labelled TRPM8 generated by in vitro transcription and translation, 2. immunoblotting lysates made from cells transiently transfected with TRPM8 cDNA, 3. Immunoprecipitation of TRPM8 transfected lysates with detection by blotting and 4. flow cytometry. RESULTS: Fifty scleroderma patients with Raynaud's phenomenon (41 female, 39 Caucasian, 23 with limited scleroderma, and 20 with history of cancer) were studied. Four different methods to assay for TRPM8 antibodies were set up, optimised and validated using commercial antibodies. All 50 scleroderma patients' sera were assayed using each of the above methods, and all were negative for TRPM8 autoantibodies. CONCLUSIONS: Antibodies against TRPM8 are not found in scleroderma patient sera, suggesting that the abnormal cold sensitivity and associated abnormal vascular reactivity in scleroderma patients is not the result of an immune process targeting TRPM8. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. ashah32@jhmi.edu. FAU - Montagne, Janelle AU - Montagne J AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Oh, Sun-Young AU - Oh SY AD - Division of Allergy and Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. LA - eng GR - R01 AR044684/AR/NIAMS NIH HHS/United States GR - R56 AR062615-01A1/AR/NIAMS NIH HHS/United States GR - R56 AR062615/AR/NIAMS NIH HHS/United States GR - K23 AR061439/AR/NIAMS NIH HHS/United States GR - R01 AR-44684/AR/NIAMS NIH HHS/United States GR - P30-AR-053503/AR/NIAMS NIH HHS/United States GR - P30 AR053503/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150805 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (TRPM Cation Channels) RN - 0 (TRPM8 protein, human) SB - IM MH - Adult MH - Aged MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Body Temperature Regulation MH - Cross-Sectional Studies MH - Female MH - Humans MH - Middle Aged MH - Pilot Projects MH - Predictive Value of Tests MH - Raynaud Disease/blood/diagnosis/*immunology/physiopathology MH - Scleroderma, Diffuse/blood/diagnosis/*immunology/physiopathology MH - Scleroderma, Limited/blood/diagnosis/*immunology/physiopathology MH - Serologic Tests MH - TRPM Cation Channels/*immunology MH - Thermosensing PMC - PMC4567034 MID - NIHMS720590 COIS- The authors declare no conflict of interest. EDAT- 2015/08/06 06:00 MHDA- 2015/10/27 06:00 PMCR- 2015/09/11 CRDT- 2015/08/06 06:00 PHST- 2014/12/23 00:00 [received] PHST- 2015/06/23 00:00 [accepted] PHST- 2015/08/06 06:00 [entrez] PHST- 2015/08/06 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] PHST- 2015/09/11 00:00 [pmc-release] AID - 9002 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S123-6. Epub 2015 Aug 5. PMID- 27310203 OWN - NLM STAT- MEDLINE DCOM- 20170406 LR - 20181202 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 27 IP - 1 DP - 2017 Jan TI - Effect of treatment with iloprost with or without bosentan on nailfold videocapillaroscopic alterations in patients with systemic sclerosis. PG - 110-114 LID - 10.1080/14397595.2016.1192761 [doi] AB - INTRODUCTION: Vascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis; it is responsible for some important clinical manifestations of the disease such as Raynaud's phenomenon and digital ulcers (DU). Bosentan, a dual receptor endothelin antagonist, and iloprost, often in combination therapy, seems to be able to interfere with the scleroderma microangiopathy. OBJECTIVES: Aim of the study was to evaluate the effect of bosentan and iloprost on scleroderma microangiopathy, analyzed by means of capillaroscopic skin ulcer risk index (CSURI), in SSc patients treated for the prevention of DU. METHODS: Nailfold videocapillaroscopy (NVC) was performed in 95 SSc patients, treated with iloprost alone (group 1) or combination therapy with iloprost and bosentan (group 2), at baseline and after one year. In all patients CSURI was calculated according to the formula "diameter × number of megacapillaries/(total number of capillaries)(2)": in addition, total number of capillaries, giant capillaries, micro-hemorrhages, disorganization of the vascular array, and ramified capillaries were evaluated by means of a semiquantitative score. RESULTS: After 12 months, we observed a reduction of the number of giant capillaries in both groups, while an increase of ramified capillaries was recorded only in group 2. CSURI improved slightly in group 2 without statistical significance; on the contrary, in group 1 a significant worsening was recorded (p ≤ 0.001). CONCLUSIONS: Our study confirms the effectiveness of bosentan, in combination with iloprost, in SSc microangiopathy observed to NVC. Moreover, the observed findings further support the role of CSURI in the evaluation and monitoring of SSc microangiopathy. FAU - Cestelli, Valentina AU - Cestelli V AD - a Rheumatology Unit , University of Modena and Reggio Emilia , Italy. FAU - Manfredi, Andreina AU - Manfredi A AD - a Rheumatology Unit , University of Modena and Reggio Emilia , Italy. FAU - Sebastiani, Marco AU - Sebastiani M AD - a Rheumatology Unit , University of Modena and Reggio Emilia , Italy. FAU - Praino, Emanuela AU - Praino E AD - b Rheumatology Unit , University of Bari , Italy , and. FAU - Cannarile, Francesca AU - Cannarile F AD - c Rheumatology Unit , University of Perugia , Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - a Rheumatology Unit , University of Modena and Reggio Emilia , Italy. FAU - Ferri, Clodoveo AU - Ferri C AD - a Rheumatology Unit , University of Modena and Reggio Emilia , Italy. LA - eng PT - Journal Article DEP - 20160616 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - JED5K35YGL (Iloprost) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/pharmacology/*therapeutic use MH - Bosentan MH - Capillaries/diagnostic imaging/*drug effects MH - Drug Therapy, Combination MH - Female MH - Humans MH - Iloprost/pharmacology/*therapeutic use MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/diagnostic imaging/*drug therapy/etiology MH - Scleroderma, Systemic/complications/diagnostic imaging/*drug therapy MH - Sulfonamides/pharmacology/*therapeutic use MH - Treatment Outcome OTO - NOTNLM OT - Bosentan OT - CSURI OT - Iloprost OT - Microangiopathy OT - Systemic sclerosis OT - Videocapillaroscopy EDAT- 2016/06/17 06:00 MHDA- 2017/04/07 06:00 CRDT- 2016/06/17 06:00 PHST- 2016/06/17 06:00 [pubmed] PHST- 2017/04/07 06:00 [medline] PHST- 2016/06/17 06:00 [entrez] AID - 10.1080/14397595.2016.1192761 [doi] PST - ppublish SO - Mod Rheumatol. 2017 Jan;27(1):110-114. doi: 10.1080/14397595.2016.1192761. Epub 2016 Jun 16. PMID- 20591195 OWN - NLM STAT- MEDLINE DCOM- 20110203 LR - 20211020 IS - 1471-2474 (Electronic) IS - 1471-2474 (Linking) VI - 11 DP - 2010 Jul 1 TI - Anti-centromere antibody-seropositive Sjögren's syndrome differs from conventional subgroup in clinical and pathological study. PG - 140 LID - 10.1186/1471-2474-11-140 [doi] AB - BACKGROUND: To clarify the clinicopathological characteristics of primary Sjögren's syndrome (pSS) with anti-centromere antibody (ACA). METHODS: Characteristics of 14 patients of pSS with ACA were evaluated. All patients were anti-SS-A/Ro and SS-B/La antibodies negative (ACA+ group) without sclerodactyly. The prevalence of Raynaud's phenomenon (RP), titer of IgG and focus score (FS) in the minor salivary glands (MSGs) were determined. Quantification analysis of Azan Mallory staining was performed to detect collagenous fiber. Forty eight patients in whom ACA was absent were chosen as the conventional (ACA-) pSS group. RESULTS: Prevalence of ACA+ SS patients was 14 out of 129 (10.85%) pSS patients. RP was observed in 61.5% of the patients with ACA. The level of IgG in the ACA+ group was significantly lower than that of the ACA- group (p = 0.018). Statistical difference was also found in the FS of MSGs from the ACA+ group (1.4 +/- 1.0) as compared with the ACA- group (2.3 +/- 1.6) (p = 0.035). In contrast, the amount of fibrous tissue was much higher in the ACA+ group (65052.2 +/- 14520.6 microm(2) versus 26251.3 +/- 14249.8 microm(2)) (p = 1.3 x 10(-12)). CONCLUSIONS: Low cellular infiltration but with an increase in fibrous tissues may explain the clinical feature of a high prevalence of RP and normal IgG concentration in ACA+ pSS. FAU - Nakamura, Hideki AU - Nakamura H AD - Unit of Translational Medicine, Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Nagasaki, Japan. nakamura_hideki911@yahoo.co.jp FAU - Kawakami, Atsushi AU - Kawakami A FAU - Hayashi, Tomayoshi AU - Hayashi T FAU - Iwamoto, Naoki AU - Iwamoto N FAU - Okada, Akitomo AU - Okada A FAU - Tamai, Mami AU - Tamai M FAU - Yamasaki, Satoshi AU - Yamasaki S FAU - Ida, Hiroaki AU - Ida H FAU - Eguchi, Katsumi AU - Eguchi K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100701 PL - England TA - BMC Musculoskelet Disord JT - BMC musculoskeletal disorders JID - 100968565 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunoglobulin G) SB - IM MH - Aged MH - Autoantibodies/*biosynthesis/*blood MH - Biomarkers/blood MH - Centromere/*immunology/*pathology MH - Disease Progression MH - Female MH - Fibrosis/diagnosis/immunology/pathology MH - Humans MH - Immunoglobulin G/biosynthesis/blood MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/immunology/pathology MH - Salivary Glands/immunology/pathology MH - Severity of Illness Index MH - Sjogren's Syndrome/diagnosis/*immunology/*pathology PMC - PMC2902414 EDAT- 2010/07/02 06:00 MHDA- 2011/02/04 06:00 PMCR- 2010/07/01 CRDT- 2010/07/02 06:00 PHST- 2010/03/04 00:00 [received] PHST- 2010/07/01 00:00 [accepted] PHST- 2010/07/02 06:00 [entrez] PHST- 2010/07/02 06:00 [pubmed] PHST- 2011/02/04 06:00 [medline] PHST- 2010/07/01 00:00 [pmc-release] AID - 1471-2474-11-140 [pii] AID - 10.1186/1471-2474-11-140 [doi] PST - epublish SO - BMC Musculoskelet Disord. 2010 Jul 1;11:140. doi: 10.1186/1471-2474-11-140. PMID- 27091617 OWN - NLM STAT- MEDLINE DCOM- 20170113 LR - 20181202 IS - 0035-9351 (Print) IS - 0035-9351 (Linking) VI - 95 IP - 3 DP - 2016 Mar TI - [Lumbar sympathectomy literature review over the past 15 years]. PG - 101-6 AB - INTRODUCTION: Lumbar sympathectomy (LS) irreversibly damages a part of the sympathetic trunk and adjacent ganglia between L1 and L5, typically between L2 and L4. The first LS was performed in 1923. Initially, it used to be performed very often; however, with the progress of vascular and endovascular surgery its importance gradually continues to decline. The aim of the paper is to present literature review focusing on LS over the past 15 years. METHOD: Literature review of 113 academic articles found in academic journal databases. PATHOPHYSIOLOGY: Irreversible interruption of the efferent innervation leads to relative vasodilation of small vessels in lower extremities (α1-receptors blockade), and it reduces the volume of sweat due to inactivation of eccrine glands and nociception from lower limbs. INDICATION: Raynaud´s phenomenon, thromboangitis obliterans, non-revascularizable peripheral arterial disease (PAD) (Fontain grade III-IV), hyperhidrosis, persistent pain in lower extremities, chronic pain of amputation stump, frostbites, chilblains.Effect: The three largest studies showed a positive effect in 63.6-93.4% cases of PAD and in 97%100% cases of hyperhidrosis. The positive effect was defined as warmer lower extremities, increased blood flow, acceleration of chronic defects healing, sweating disappearance and pain reduction. CONCLUSION: Lumbar sympathectomy still remains a useful method in the treatment of above mentioned diseases if properly indicated. KEY WORDS: lumbar sympathectomy - Raynaud´s phenomenon - thromboangitis obliterans -peripheral arterial disease - hyperhidrosis. FAU - Pekař, M AU - Pekař M FAU - Mazur, M AU - Mazur M FAU - Pekařová, A AU - Pekařová A FAU - Kozák, J AU - Kozák J FAU - Foltys, A AU - Foltys A LA - cze PT - Journal Article PT - Review TT - Lumbálna sympatektómia prehľad svetovej literatúry za posledných 15 rokov. PL - Czech Republic TA - Rozhl Chir JT - Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnosti JID - 9815441 SB - IM MH - Chilblains/*surgery MH - Frostbite/*surgery MH - Humans MH - Hyperhidrosis/*surgery MH - Lower Extremity MH - Lumbosacral Plexus/*surgery MH - Peripheral Arterial Disease/*surgery MH - Phantom Limb/*surgery MH - Raynaud Disease/*surgery MH - *Sympathectomy MH - Thromboangiitis Obliterans/*surgery EDAT- 2016/04/20 06:00 MHDA- 2017/01/14 06:00 CRDT- 2016/04/20 06:00 PHST- 2016/04/20 06:00 [entrez] PHST- 2016/04/20 06:00 [pubmed] PHST- 2017/01/14 06:00 [medline] AID - 57935 [pii] PST - ppublish SO - Rozhl Chir. 2016 Mar;95(3):101-6. PMID- 23222375 OWN - NLM STAT- MEDLINE DCOM- 20140219 LR - 20220317 VI - 122 Suppl 1 DP - 2012 TI - Very early diagnosis of systemic sclerosis. PG - 18-23 AB - Systemic sclerosis (SSc) is an incurable chronic autoimmune disease associated with high morbidity and mortality. The current validated or proposed criteria are not appropriate to make a very early diagnosis of SSc. This implies that the diagnosis of SSc and, consequently, an appropriate therapy are delayed until the appearance of skin involvement and/or clinically detectable internal organ involvement when microvascular remodeling, tissue fibrosis, or atrophy are already irreversible. In a recent Delphi exercise, 4 signs/symptoms have been identified as necessary for the very early diagnosis of SSc: Raynaud's phenomenon (RP), puffy swollen digits turning into sclerodactily, antinuclear antibodies and specific SSc antibodies (anticentromere and antitopoisomerase‑I antibodies), and abnormal capillaroscopy with scleroderma pattern. Patients with very early SSc are the target of the recently launched the VEDOSS program, which has been designed to diagnose SSc very early and to examine whether this may change the disease prognosis. Although patients with RP, autoantibodies, and SSc capillaroscopic pattern could be easily followed up, there is still no agreement on the predictors that may allow us to identify patients who will develop an established disease. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Rheumatology, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M LA - eng PT - Journal Article PT - Review PL - Poland TA - Pol Arch Med Wewn JT - Polskie Archiwum Medycyny Wewnetrznej JID - 0401225 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - EC 5.99.1.- (DNA Topoisomerases) SB - IM MH - Antibodies, Antinuclear/analysis MH - Biomarkers/analysis MH - DNA Topoisomerases/analysis MH - *Early Diagnosis MH - Edema/etiology MH - Humans MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/complications/*diagnosis EDAT- 2012/12/19 06:00 MHDA- 2014/02/20 06:00 CRDT- 2012/12/11 06:00 PHST- 2012/12/11 06:00 [entrez] PHST- 2012/12/19 06:00 [pubmed] PHST- 2014/02/20 06:00 [medline] PST - ppublish SO - Pol Arch Med Wewn. 2012;122 Suppl 1:18-23. PMID- 21283970 OWN - NLM STAT- MEDLINE DCOM- 20110323 LR - 20141120 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 40 IP - 1 DP - 2011 Jan TI - Systemic sclerosis - a systematic overview: part 2 - immunosuppression, treatment of SSc-associated vasculopathy, and treatment of pulmonary arterial hypertension. PG - 20-30 LID - 10.1024/0301-1526/a000066 [doi] AB - Here we give an overview over treatment recommendations propagated by the European League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research Group, the German Network for Systemic Sclerosis, the European Respiratory Society, and the International Society of Heart and Lung Transplantation. As response to immunosuppressant (IS) therapy is usually weaker in systematic sclerosis (SSc) compared to other connective tissue disorders IS should be considered with caution. To prevent scleroderma renal crisis steroid doses should not exceed 15 mg/d. The definitive role of a number of new immunosuppressant drugs and the effects of autologous stem cell transplantation in systemic clerosis (SSc) have to be elucidated. Prostanoids, especially iloprost, are widely used as intravenous formulas for the treatment of severe Raynaud's phenomenon (RP) and digital ulcers (DU). Calcium antagonists are of limited therapeutic value. Bosentan, an oral endothelin receptor antagonists (ETRA), was shown to prevent new DU, but failed to heal existing DU, while the oral phopshodiesterase inhibitor (PDI) Sildenafil reduces the occurrence of RP and might be effective in ulcer healing. Combination therapies of PDI with ETRA are currently evaluated. Therapy of pulmonary arterial hypertension (PAH) is usually started as oral monotherapy, frequently using an ETRA. When this first-line therapy is not tolerated ETRA is substituted by PDI. If treatment goals are not reached with monotherapy combinationtherapy is started, for example by adding a PDI to an existing ETRA. In general, treatment of PAH in patients with connective tissue disease follows the same algorithms as in idiopathic PAH. FAU - Opitz, C AU - Opitz C AD - Klinik für Innere Medizin, Schwerpunkt Kardiologie, DRK Kliniken Berlin, Germany. p.klein-weigel@drk-kliniken-berlin.de FAU - Klein-Weigel, P F AU - Klein-Weigel PF FAU - Riemekasten, G AU - Riemekasten G LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 RN - 0 (Antihypertensive Agents) RN - 0 (Cardiovascular Agents) RN - 0 (Immunosuppressive Agents) SB - IM CIN - Vasa. 2011 Jan;40(1):3-4. doi: 10.1024/0301-1526/a000064. PMID: 21375075 MH - Antihypertensive Agents/therapeutic use MH - Cardiovascular Agents/*therapeutic use MH - Drug Therapy, Combination MH - Europe MH - Familial Primary Pulmonary Hypertension MH - Humans MH - Hypertension, Pulmonary/drug therapy/immunology/physiopathology MH - Immunosuppressive Agents/*therapeutic use MH - Practice Guidelines as Topic MH - Raynaud Disease/*drug therapy/immunology/physiopathology MH - Scleroderma, Systemic/*drug therapy/immunology/physiopathology MH - Societies, Medical MH - Stem Cell Transplantation MH - Treatment Outcome MH - Ulcer/drug therapy/immunology/physiopathology EDAT- 2011/02/02 06:00 MHDA- 2011/03/24 06:00 CRDT- 2011/02/02 06:00 PHST- 2011/02/02 06:00 [entrez] PHST- 2011/02/02 06:00 [pubmed] PHST- 2011/03/24 06:00 [medline] AID - 10.1024/0301-1526/a000066 [doi] PST - ppublish SO - Vasa. 2011 Jan;40(1):20-30. doi: 10.1024/0301-1526/a000066. PMID- 19097597 OWN - NLM STAT- MEDLINE DCOM- 20090206 LR - 20131121 IS - 2072-0939 (Print) IS - 2072-0939 (Linking) VI - 31 IP - 5 DP - 2008 Sep-Oct TI - Vascular response of Raynaud's phenomenon to nifedipine or herbal medication (duhuo-tisheng tang with danggui-sini tang): a preliminary study. PG - 492-502 AB - BACKGROUND: Raynaud's phenomenon (RP) is a common manifestation in connective tissue diseases. Calcium channel antagonists are most effective and frequently used for treating RP. This study compared the efficacy, digital vascular response, and tolerability between nifedipine and a combination of 2 Chinese herbal medications (duhuo-tisheng tang and danggui-sini tang) for treating RP. METHODS: This open-label non-randomized clinical trial included 47 connective tissue disease patients with RP. The herbal group and the nifedipine group included 26 and 21 patients, respectively. The duration of therapy was 4 weeks. Baseline and posttreatment laser Doppler blood flow imaging of both hands were performed at room temperature and after cold challenge. Nailfold capillary microscopy was performed at the baseline and after 4 weeks of therapy. Serum levels of soluble intercellular adhesion molecule-1 (sICAM-1), prostaglandin E2 (PGE2), nitrite (NO2), and nitrate (NO3), and plasma levels of endothelin-1 (ET-1) were also measured. Self-reported symptoms, using a visual analog scale (VAS) and a physician global assessment (PGA), were recorded at the baseline and after treatment. RESULTS: After 4 weeks of treatment, VAS scores improved (p = 0.0035) and the physician's global assessment of RP severity decreased in the nifedipine group (p = 0.0078) but not in the herbal group. Episodes of RP attacks decreased in the nifedipine group after treatment (p = 0.008). The nifedipine group had increased laser Doppler flow (116.3 +/- 70.7 AU) compared to the baseline (72.4 +/- 49.0 AU, p = 0.0008). Laser Doppler images improved at various time points after cold challenge in the nifedipine group after therapy. Laser Doppler flow in the herbal group did not significantly change with therapy. Capillary microscopy demonstrated no significant difference in enlargements, avascularity, or hemorrhagic spots between groups. Serum NO2 concentrations were higher in the nifedipine group than in the herbal group. Levels of sICAM-1, PGE2, NO3, and ET-1 after therapy were similar to those at the baseline in both groups. CONCLUSIONS: The digital vascular response in RP improved with nifedipine but was unchanged with a combination of the Chinese medicines Duhuo-Tisheng Tang and Danggui-Sini Tang. FAU - Wu, Yeong-Jian Jan AU - Wu YJ AD - Division of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Keelung, Chang Gung University College of Medicine, Taoyuan, Taiwan. FAU - Luo, Shue-Fen AU - Luo SF FAU - Yang, Sien-Hung AU - Yang SH FAU - Chen, Ji-Yih AU - Chen JY FAU - Yu, Kuang-Hui AU - Yu KH FAU - See, Lai-Chu AU - See LC LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China (Republic : 1949- ) TA - Chang Gung Med J JT - Chang Gung medical journal JID - 101088034 RN - 0 (Calcium Channel Blockers) RN - 0 (Drugs, Chinese Herbal) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Blood Vessels/*drug effects/physiopathology MH - Calcium Channel Blockers/*therapeutic use MH - Drugs, Chinese Herbal/*therapeutic use MH - Female MH - Humans MH - Male MH - Nifedipine/*therapeutic use MH - Raynaud Disease/*drug therapy/physiopathology EDAT- 2008/12/23 09:00 MHDA- 2009/02/07 09:00 CRDT- 2008/12/23 09:00 PHST- 2008/12/23 09:00 [entrez] PHST- 2008/12/23 09:00 [pubmed] PHST- 2009/02/07 09:00 [medline] AID - 3105/310510 [pii] PST - ppublish SO - Chang Gung Med J. 2008 Sep-Oct;31(5):492-502. PMID- 30128730 OWN - NLM STAT- MEDLINE DCOM- 20191230 LR - 20200309 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 39 IP - 2 DP - 2019 Feb TI - Raynaud's phenomenon in Northern Sweden: a population-based nested case-control study. PG - 265-275 LID - 10.1007/s00296-018-4133-y [doi] AB - The aim of this study was to determine the association between individual and external exposure factors, and the reporting of Raynaud's phenomenon, with or without concomitant cold sensitivity. In a population-based nested case-control study, cases with Raynaud's phenomenon (N = 578), and matched controls (N = 1156), were asked to respond to a questionnaire focusing on different risk factors. Univariate and multiple conditional logistic regression were performed. Analyses were stratified according to whether the cases reported cold sensitivity or not. In total, 1400 out of 1734 study subjects answered the questionnaire (response rate 80.7%). In the final multiple model, the factor with the strongest association to Raynaud's phenomenon, with and without cold sensitivity, was previous frostbite affecting the hands (OR 12.44; 95% CI 5.84-26.52 and OR 4.01; 95% CI 1.78-9.01, respectively). Upper extremity nerve injury was associated to reporting Raynaud's phenomenon and cold sensitivity (OR 2.23; 95% CI 1.29-3.85), but not Raynaud's phenomenon alone. Reporting any exposure to hand-arm vibration or cumulative cold exposure was significant in univariate analyses for cases with both Raynaud's phenomenon and cold sensitivity, but not in the multiple model. Raynaud's phenomenon is strongly associated to previous cold injury, with a larger effect size among those who also report cold sensitivity. The fact that only upper extremity nerve injury differed significantly between case groups in our multiple model offers additional support to the neural basis for cold sensitivity. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Occupational and Environmental Medicine, Umeå University, 901 87, Umeå, Sweden. albin.stjernbrandt@umu.se. FAU - Pettersson, Hans AU - Pettersson H AUID- ORCID: 0000-0001-7077-2389 AD - Occupational and Environmental Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Liljelind, Ingrid AU - Liljelind I AUID- ORCID: 0000-0002-5936-1172 AD - Occupational and Environmental Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AUID- ORCID: 0000-0003-2789-6321 AD - Occupational and Environmental Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Wahlström, Jens AU - Wahlström J AUID- ORCID: 0000-0002-2359-509X AD - Occupational and Environmental Medicine, Umeå University, 901 87, Umeå, Sweden. LA - eng GR - VLL-646641/Västerbotten Läns Landsting/International PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180820 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Cold Temperature MH - Female MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Raynaud Disease/*etiology MH - Risk Factors OTO - NOTNLM OT - Cold exposure OT - Epidemiology OT - Frostbite OT - Hand OT - Occupational exposure OT - Risk factors EDAT- 2018/08/22 06:00 MHDA- 2019/12/31 06:00 CRDT- 2018/08/22 06:00 PHST- 2018/07/10 00:00 [received] PHST- 2018/08/14 00:00 [accepted] PHST- 2018/08/22 06:00 [pubmed] PHST- 2019/12/31 06:00 [medline] PHST- 2018/08/22 06:00 [entrez] AID - 10.1007/s00296-018-4133-y [pii] AID - 10.1007/s00296-018-4133-y [doi] PST - ppublish SO - Rheumatol Int. 2019 Feb;39(2):265-275. doi: 10.1007/s00296-018-4133-y. Epub 2018 Aug 20. PMID- 27373498 OWN - NLM STAT- MEDLINE DCOM- 20171109 LR - 20191210 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 46 IP - 3 DP - 2016 Dec TI - Validation of the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis in patients from a capillaroscopy clinic. PG - 350-355 LID - S0049-0172(16)30059-2 [pii] LID - 10.1016/j.semarthrit.2016.05.007 [doi] AB - OBJECTIVE: To validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients from a capillaroscopy clinic. METHODS: All patients attended in a capillaroscopy clinic were included. Sociodemographic and SSc-related variables were collected. Using as gold standard for SSc the clinical judgement, the performance (sensitivity, specificity, predictive values, and likelihood ratios) of the 2013 ACR/EULAR criteria were analyzed. Receiver operating characteristic (ROC) curve and the area under the curve (AUC) were calculated for the global score and individual items, and the best cutoffs were obtained. RESULTS: We included 327 patients (84% women, mean age at capillaroscopy 48 years). Main reasons for capillaroscopy referral were Raynaud's phenomenon (39%) and SSc evaluation (27%). The most frequent final clinical diagnosis were SSc (32.4%) and primary Raynaud's phenomenon (25.7%). The 2013 ACR/EULAR SSc classification criteria were met by 116 patients (35.5%). Sensitivity and specificity of the new criteria were 98.1% and 94.6%, respectively, and positive and negative predictive values were 89.7% and 99.1%. The individual variables with the best sensitivity were Raynaud's phenomenon (99.1%) and abnormal nailfold capillaries (81.1%). All the individual variables, except Raynaud's phenomenon, puffy fingers and sclerodactily showed high specificity values, over 90%. The best cutoffs of the total score were ≥8, ≥9, and ≥10, and the AUC = 0.993. CONCLUSIONS: We validated the new ACR/EULAR classification criteria for SSc in unselected patients from a capillaroscopy clinic. Global score and individual items included in the new criteria show high diagnostic accuracy and discriminatory capacity. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Melchor, Sheila AU - Melchor S AD - Servicio de Reumatología, Hospital Universitario12 de Octubre, Avda de Córdoba s/n, 28041, Madrid, Spain. FAU - Joven, Beatriz E AU - Joven BE AD - Servicio de Reumatología, Hospital Universitario12 de Octubre, Avda de Córdoba s/n, 28041, Madrid, Spain. FAU - Andreu, Jose Luis AU - Andreu JL AD - Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain. FAU - Loza, Estibaliz AU - Loza E AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Garcia de Yebenes, M Jesus AU - Garcia de Yebenes MJ AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Carmona, Loreto AU - Carmona L AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario12 de Octubre, Avda de Córdoba s/n, 28041, Madrid, Spain. Electronic address: carreira@h12o.es. LA - eng PT - Journal Article PT - Validation Study DEP - 20160603 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Area Under Curve MH - Child MH - Cross-Sectional Studies MH - Europe MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - ROC Curve MH - Raynaud Disease/diagnosis/diagnostic imaging/etiology MH - Rheumatology MH - Scleroderma, Systemic/classification/complications/*diagnosis/diagnostic imaging MH - Sensitivity and Specificity MH - Societies, Medical MH - United States MH - Young Adult OTO - NOTNLM OT - Classification criteria OT - Systemic sclerosis OT - Validity EDAT- 2016/07/05 06:00 MHDA- 2017/11/10 06:00 CRDT- 2016/07/05 06:00 PHST- 2016/03/11 00:00 [received] PHST- 2016/04/18 00:00 [revised] PHST- 2016/05/27 00:00 [accepted] PHST- 2016/07/05 06:00 [pubmed] PHST- 2017/11/10 06:00 [medline] PHST- 2016/07/05 06:00 [entrez] AID - S0049-0172(16)30059-2 [pii] AID - 10.1016/j.semarthrit.2016.05.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Dec;46(3):350-355. doi: 10.1016/j.semarthrit.2016.05.007. Epub 2016 Jun 3. PMID- 25446164 OWN - NLM STAT- MEDLINE DCOM- 20150807 LR - 20250529 IS - 1879-3649 (Electronic) IS - 1537-1891 (Print) IS - 1537-1891 (Linking) VI - 63 IP - 3 DP - 2014 Dec TI - Reduced levels of S-nitrosothiols in plasma of patients with systemic sclerosis and Raynaud's phenomenon. PG - 178-81 LID - S1537-1891(14)00163-3 [pii] LID - 10.1016/j.vph.2014.09.003 [doi] AB - OBJECTIVE: S-Nitrosothiols (RSNOs) are bioactive forms of nitric oxide which are involved in cell signalling and redox regulation of vascular function. Circulating S-nitrosothiols are predominantly in the form of S-nitrosoalbumin. In this study plasma concentrations of S-nitrosothiols were measured in patients with systemic sclerosis (SSc) where NO metabolism is known to be abnormal. PATIENTS AND METHODS: Venous blood was collected from 16 patients with Raynaud's phenomenon (RP), 45 with systemic sclerosis (SSc) (34 patients had limited SSc (IcSSc) and 11 diffuse cutaneous disease (dcSSc)). Twenty six healthy subjects were used as controls. Plasma S-nitrosothiol concentrations were measured by chemiluminescence. The measurements were related to the extent of biological age, capillary/skin scores and disease duration. RESULTS: Plasma RSNO levels in patients with Raynaud's phenomenon (RP) and in those with SSc was significantly lower compared to the concentrations in control subjects. In SSc, plasma S-nitrosothiols were often below the level of detection (1nM). CONCLUSIONS: Low S-nitrosothiol concentrations were observed in the blood of patients with SSc and patients with RP indicating a profound disturbance of nitric oxide metabolism. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Kundu, Devi AU - Kundu D AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London NW3 2PF, UK. Electronic address: Devi.Kundu@ucl.ac.uk. FAU - Abraham, David AU - Abraham D AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London NW3 2PF, UK. FAU - Black, Carol M AU - Black CM AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London NW3 2PF, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London NW3 2PF, UK. FAU - Bruckdorfer, K Richard AU - Bruckdorfer KR AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London NW3 2PF, UK. LA - eng GR - 19427/VAC_/Versus Arthritis/United Kingdom GR - 144524/ARC_/Arthritis Research UK/United Kingdom GR - 14383/ARC_/Arthritis Research UK/United Kingdom GR - 19427/ARC_/Arthritis Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141015 PL - United States TA - Vascul Pharmacol JT - Vascular pharmacology JID - 101130615 RN - 0 (S-Nitrosothiols) RN - 31C4KY9ESH (Nitric Oxide) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nitric Oxide/blood MH - Raynaud Disease/*blood MH - S-Nitrosothiols/*blood MH - Scleroderma, Systemic/*blood MH - Young Adult PMC - PMC4265732 OTO - NOTNLM OT - Acetic acid (PubChem: ClD:176) OT - Copper sulphate (PubChem: ClD 24462) OT - Dithiothreitol (PubChem: ClD 19001) OT - Ethylenediaminetetracetic acid (Pub Chem ClD 6049) OT - N-ethylmaleimide (PubChem ClD:4362) OT - Nitric oxide (PubChem: ClD: 145068) OT - Potassium iodide (PubChem ClD: 4875) OT - S-nitrosothiols OT - Skin score OT - Sulfanilamide (PubChem: ClD 5333) OT - Systemic sclerosis EDAT- 2014/12/03 06:00 MHDA- 2015/08/08 06:00 PMCR- 2014/12/01 CRDT- 2014/12/03 06:00 PHST- 2014/01/14 00:00 [received] PHST- 2014/09/03 00:00 [revised] PHST- 2014/09/07 00:00 [accepted] PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/08/08 06:00 [medline] PHST- 2014/12/01 00:00 [pmc-release] AID - S1537-1891(14)00163-3 [pii] AID - 10.1016/j.vph.2014.09.003 [doi] PST - ppublish SO - Vascul Pharmacol. 2014 Dec;63(3):178-81. doi: 10.1016/j.vph.2014.09.003. Epub 2014 Oct 15. PMID- 24141348 OWN - NLM STAT- MEDLINE DCOM- 20140904 LR - 20131021 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 38 IP - 2 DP - 2013 Apr-Jun TI - Prospective nailfold capillaroscopy evaluation of Raynaud's phenomenon in children and adolescents. PG - 114-21 AB - OBJECTIVE: To evaluate prospectively the clinical features and nailfold capillaroscopy findings of a cohort of children and adolescents who presented Raynaud's phenomenon (RP) without criteria for autoimmune rheumatic diseases. METHODS: 40 children and adolescents with isolated RP were included. Evidence of systemic autoimmune rheumatic diseases (SARD) was ruled out by thorough clinical and laboratory examination. Concomitantly we also performed wide-field nailfold capillaroscopy evaluation using an optical microscope with magnifications of 10 and 16X. All patients were prospectively re-evaluated within a mean interval time between evaluations of 1.6 years. RESULTS: Thirty (75%) out of 40 patients were female with a mean age of 14.6 years and mean follow-up time of 4.2 years. The mean age of disease onset was 10.4 years and the mean time until diagnosis was 1.4 years. Fourteen out of 40 patients (35%) presented antinuclear antibodies (ANA). Five (12.5%) patients had altered nailfold capillaroscopy at first examination: four presented non-specific microangiopathy and one presented scleroderma pattern. At the re-evaluation three patients (7.5%) presented nailfold capillaroscopy alterations (two SD pattern and one non-specific microangiopathy). The two patients who showed scleroderma pattern at the nailfold capillaroscopy presented along the follow-up a diagnosis of mixed connective tissue disease and hypothyroidism, respectively. One 10 year-old girl with normal nailfold capillaroscopy and presence of autoantibodies (ANA 1/640, nuclear homogeneous pattern, anti-native DNA 1/80) was diagnosed with systemic lupus erythematosus after 1 year of initial evaluation. None of the other children presented diagnosis of SARD along the follow-up. CONCLUSIONS: Primary Raynaud´s phenomenon remained the diagnosis in most cases in this series of children and adolescents presenting with initial RP complaint. Nailfold capillaroscopy and determination of autoantibodies were useful ancillary tools in the investigation of possible evolution towards SARD. FAU - Piotto, Daniela G P AU - Piotto DG FAU - Hilário, Maria O E AU - Hilário MO FAU - Carvalho, Natalia S AU - Carvalho NS FAU - Len, Cláudio A AU - Len CA FAU - Andrade, Luis E C AU - Andrade LE FAU - Terreri, Maria T R A AU - Terreri MT LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adolescent MH - Child MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Prospective Studies MH - Raynaud Disease/*diagnosis EDAT- 2013/10/22 06:00 MHDA- 2014/09/05 06:00 CRDT- 2013/10/22 06:00 PHST- 2013/10/22 06:00 [entrez] PHST- 2013/10/22 06:00 [pubmed] PHST- 2014/09/05 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2013 Apr-Jun;38(2):114-21. PMID- 34465533 OWN - NLM STAT- MEDLINE DCOM- 20220404 LR - 20220405 IS - 1440-1592 (Electronic) IS - 1323-8930 (Linking) VI - 71 IP - 2 DP - 2022 Apr TI - Drug-induced scleroderma-like lesion. PG - 163-168 LID - S1323-8930(21)00093-9 [pii] LID - 10.1016/j.alit.2021.08.005 [doi] AB - Drug-induced scleroderma-like lesion is a condition in which administration of a drug induces skin sclerotic lesions similar to systemic sclerosis or morphea. The clinical manifestations of drug-induced scleroderma-like lesion can be divided into two types: scleroderma-like lesions and morphea-like plaques. A wide variety of drugs can cause drug-induced scleroderma-like lesion. Bleomycin, (L)-tryptophan, vinyl chloride, and phytonadione (vitamin K(1)) have been reported, but in recent years, cases due to chemotherapeutic agents, such as taxane-based agents, gemcitabine, and tegafur-uracil, and immune checkpoint inhibitors have increased. Drug-induced scleroderma-like lesion differs from systemic sclerosis in that it does not include Raynaud's phenomenon, nail-fold capillary abnormality, organ involvement, such as reflux esophagitis, interstitial pneumonia, renal crisis, or anti-nuclear Abs. On the other hand, there are reports of cases in which Raynaud's phenomenon, positive conversion of anti-nuclear Abs, and development of skin sclerosis from the fingers developed after initiation of the drug. Whether the skin sclerosis improves after discontinuation of the drug depends on the patient. In patients with severe skin sclerosis, functional impairment, such as flexion contracture of the fingers, may occur, and systemic therapy, such as steroids, may be necessary. When treating patients with skin sclerosis, it is important to keep in mind the possibility that the sclerotic lesion may be induced by a drug. CI - Copyright © 2021 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved. FAU - Hamaguchi, Yasuhito AU - Hamaguchi Y AD - Department of Molecular Pathology of Skin, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. Electronic address: yasuhito@med.kanazawa-u.ac.jp. LA - eng PT - Journal Article PT - Review DEP - 20210828 PL - England TA - Allergol Int JT - Allergology international : official journal of the Japanese Society of Allergology JID - 9616296 SB - IM MH - Capillaries/pathology MH - Humans MH - *Raynaud Disease/chemically induced/diagnosis MH - *Scleroderma, Localized/chemically induced/diagnosis/pathology MH - *Scleroderma, Systemic/diagnosis/pathology MH - *Vascular Malformations OTO - NOTNLM OT - Drug OT - Fibrosis OT - Morphea OT - Scleroderma OT - Skin sclerosis EDAT- 2021/09/02 06:00 MHDA- 2022/04/05 06:00 CRDT- 2021/09/01 05:53 PHST- 2021/07/11 00:00 [received] PHST- 2021/07/26 00:00 [accepted] PHST- 2021/09/02 06:00 [pubmed] PHST- 2022/04/05 06:00 [medline] PHST- 2021/09/01 05:53 [entrez] AID - S1323-8930(21)00093-9 [pii] AID - 10.1016/j.alit.2021.08.005 [doi] PST - ppublish SO - Allergol Int. 2022 Apr;71(2):163-168. doi: 10.1016/j.alit.2021.08.005. Epub 2021 Aug 28. PMID- 31177398 OWN - NLM STAT- MEDLINE DCOM- 20200501 LR - 20200501 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 10 DP - 2019 Oct TI - Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility. PG - 2925-2932 LID - 10.1007/s10067-019-04627-w [doi] AB - OBJECTIVES: Clinical recognition of vascular acrosyndromes is often challenging. The term Raynaud's phenomenon (RP) is commonly overused to describe any form of cold-related disorder. This study aims to formally evaluate peripheral vascular symptoms affecting the population, aged ≤ 40 years, and identify any correlations to joint hypermobility (JH). PATIENTS AND METHODS: Fifty patients (31 males, 19 females) with vasomotor symptoms enrolled in this five-year prospective observational study. Clinical examination by a rheumatologist and a vascular surgeon was performed along with cardiology, echocardiographic and Doppler evaluation. Patients underwent blood cell count, biochemistry, thyroid and selectively immunologic testing. Twenty-four (48%) of them performed nailfold capillaroscopy. The SPSS for Windows, v.17.0, Chicago, USA, was used for the statistical analyses. RESULTS: Twenty-eight patients (56%) presented with erythromelalgia (EM), 6 (12%) with acrocyanosis (AC) and 9 (18%) as a combination of the above disorder. RP diagnosed in five (10%) while two patients (4%) presented as a mix of EM-RP. There was no correlation with abnormal laboratory tests. Increased incidence of JH was found in EM and AC patients. Among those who were tested with nailfold capillaroscopy, 75% had abnormalities ranged from mild to autoimmune-like diseases. CONCLUSIONS: Erythromelalgia is the commonest functional vasculopathy in young population followed by acrocyanosis and a combination of these conditions. Joint hypermobility is markedly increased, indicating that dysautonomy may be considered the causative factor following a trigger event. Overall, RP was observed in 14% of patients. Clinical recognition of these disorders avoids unnecessary investigation. Key Points • Vascular acrosyndromes in young adults are commonly functional disorders resembling vascular algodystrophy induced by thermic stress. • Dysautonomy of joint hypermobility is the co-factor influencing the appearance of the vascular disorders. • Raynaud's phenomenon accounts to approximately 14% of vascular acrosyndromes presented in the young adult population. FAU - Vounotrypidis, Periklis AU - Vounotrypidis P AD - Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece. perivoun@email.com. FAU - Pyrpasopoulou, Athina AU - Pyrpasopoulou A AD - 2nd Propaedeutic Clinic, Department of Internal Medicine, "Ippokration" Hospital, Thessaloniki, Greece. FAU - Sakellariou, Grigorios T AU - Sakellariou GT AD - Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece. FAU - Zisopoulos, Dimitrios AU - Zisopoulos D AD - Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece. FAU - Kefala, Nikoleta AU - Kefala N AD - Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece. FAU - Oikonomou, Dimitrios I AU - Oikonomou DI AD - Department of Immunology and Microbiology, 424 General Military Hospital, Thessaloniki, Greece. FAU - Stefanis, Constantinos AU - Stefanis C AD - Cardiology Department, 424 General Military Hospital, Thessaloniki, Greece. FAU - Aslanidis, Spyros AU - Aslanidis S AD - 2nd Propaedeutic Clinic, Department of Internal Medicine, "Ippokration" Hospital, Thessaloniki, Greece. FAU - Bermperidis, Charalambos AU - Bermperidis C AD - Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece. FAU - Pappas, Periklis AU - Pappas P AD - Vascular Surgery Department, 424 General Military Hospital, Thessaloniki, Greece. LA - eng GR - A68/25-7-2011/Hellenic Rheumatology Society/ PT - Journal Article PT - Observational Study DEP - 20190608 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Cyanosis/complications MH - Erythromelalgia/complications MH - Female MH - Humans MH - Incidence MH - Joint Instability/*complications MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/*complications MH - Vascular Diseases/*complications MH - Young Adult OTO - NOTNLM OT - Acrocyanosis OT - Capillaroscopy OT - Dysautonomy OT - Erythromelalgia OT - Raynaud’s phenomenon EDAT- 2019/06/10 06:00 MHDA- 2020/05/02 06:00 CRDT- 2019/06/10 06:00 PHST- 2019/02/24 00:00 [received] PHST- 2019/05/28 00:00 [accepted] PHST- 2019/05/24 00:00 [revised] PHST- 2019/06/10 06:00 [pubmed] PHST- 2020/05/02 06:00 [medline] PHST- 2019/06/10 06:00 [entrez] AID - 10.1007/s10067-019-04627-w [pii] AID - 10.1007/s10067-019-04627-w [doi] PST - ppublish SO - Clin Rheumatol. 2019 Oct;38(10):2925-2932. doi: 10.1007/s10067-019-04627-w. Epub 2019 Jun 8. PMID- 22193226 OWN - NLM STAT- MEDLINE DCOM- 20130507 LR - 20220309 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 12 DP - 2012 Dec TI - Predictors of early mortality in systemic sclerosis: a case-control study comparing early versus late mortality in systemic sclerosis. PG - 3841-4 LID - 10.1007/s00296-011-2301-4 [doi] AB - To compare the characteristics of patients with systemic sclerosis who died within 2 years of diagnosis to those who died after 2 years of diagnosis. A retrospective chart review of all medical records of deceased systemic sclerosis (SSc) patients who had been followed at our institution from 1985 to 2007 was performed. We identified 87 deceased SSc patients within this period. From the 87 deceased individuals, 20 had died within 2 years after they were diagnosed, and 67 had died after 2 years of their diagnosis. Patients who died within 2 years of diagnosis were more likely to be anticentromere antibody negative when compared to the patients who died after 2 years (17/20 vs. 48/67, P = 0.03). The time from the first appearance of non-Raynaud's symptoms to diagnosis was significantly shorter in the group who died within 2 years than in the group who died after 2 years of diagnosis (11.8 ± 10.3 vs. 60.7 ± 64.9 months, P = 0.002). According to the Medsger severity score, there was more severe muscle (0.82 ± 1.13 vs. 1.8 ± 1.28, P = 0.0014) and heart (0.86 ± 1.37 vs. 2.1 ± 1.71, P = 0.0013) involvement at the initial evaluation in patients who died before 2 years of diagnosis when compared to the group of patients who died after 2 years of diagnosis. The time from the first symptoms to treatment initiation was significantly shorter in patients who died early (9.43 ± 6.3 vs. 38.3 ± 54.4 months, P = 0.05). The interval between treatment initiation and death was also significantly shorter (15.1 ± 9.48 vs. 60.7 ± 49.7 months, P = 0.001), reflecting greater severity of disease. Patients who died within the first 2 years of SSc diagnosis were typically anticentromere negative and had significant muscle and cardiac involvement. The time from the first appearance of non-Raynaud phenomenon symptoms to death was much shorter in the patients who died within 2 years of diagnosis, suggesting a very fulminant form of systemic sclerosis. FAU - Derk, Chris T AU - Derk CT AD - Division of Rheumatology, University of Pennsylvania, 8th Floor Penn Tower; One Convention Blvd, Philadelphia, PA 19104, USA. Chris.Derk@uphs.upenn.edu FAU - Huaman, Gonzalo AU - Huaman G FAU - Littlejohn, Jayne AU - Littlejohn J FAU - Otieno, Franklin AU - Otieno F FAU - Jimenez, Sergio AU - Jimenez S LA - eng PT - Journal Article DEP - 20111223 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - Fibrosis MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/*diagnosis/*mortality MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnosis/*mortality EDAT- 2011/12/24 06:00 MHDA- 2013/05/08 06:00 CRDT- 2011/12/24 06:00 PHST- 2011/08/27 00:00 [received] PHST- 2011/12/10 00:00 [accepted] PHST- 2011/12/24 06:00 [entrez] PHST- 2011/12/24 06:00 [pubmed] PHST- 2013/05/08 06:00 [medline] AID - 10.1007/s00296-011-2301-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Dec;32(12):3841-4. doi: 10.1007/s00296-011-2301-4. Epub 2011 Dec 23. PMID- 39323322 OWN - NLM STAT- MEDLINE DCOM- 20241123 LR - 20250227 IS - 1524-475X (Electronic) IS - 1067-1927 (Linking) VI - 32 IP - 6 DP - 2024 Nov-Dec TI - Digital ulcers associated with scleroderma: A major unmet medical need. PG - 949-959 LID - 10.1111/wrr.13224 [doi] AB - Scleroderma or systemic sclerosis (SSc)-associated digital ischaemic complications, such as digital ulcers (SSc-DUs), appear relatively early during the disease course and are a major burden with substantial deterioration of quality of life. Expert rheumatologist and wound specialists have defined a DU; however, international application of the definition is still disorganised. Appearance of SSc-DUs is secondary to the onset of Raynaud's phenomenon and as a consequence, recommended first-line of treatment mainly includes vasodilators; however, many DUs are refractory to this treatment. Despite important practical issues, such as a lack of well-characterised SSc-wound healing animal model, significant efforts are needed to mechanistically understand the pathogenesis of SSc-DUs for developing clinically targetable disease modifying therapies. CI - © 2024 The Wound Healing Society. FAU - Naik, Angha AU - Naik A AD - College of Dentistry, University of Saskatchewan, Saskatoon, Saskatchewan, Canada. FAU - Stratton, Richard J AU - Stratton RJ AD - Centre for Rheumatology and Connective Tissue Disease, University College London (Royal Free Campus), London, UK. FAU - Leask, Andrew AU - Leask A AUID- ORCID: 0000-0003-2832-283X AD - College of Dentistry, University of Saskatchewan, Saskatoon, Saskatchewan, Canada. LA - eng GR - MOP-183830/CIHR/Canada GR - MOP-183830/CIHR/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20240926 PL - United States TA - Wound Repair Regen JT - Wound repair and regeneration : official publication of the Wound Healing Society [and] the European Tissue Repair Society JID - 9310939 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/therapy MH - *Skin Ulcer/etiology/therapy MH - *Fingers/pathology MH - Wound Healing MH - Animals MH - Raynaud Disease/etiology MH - Quality of Life OTO - NOTNLM OT - chronic wound OT - connective tissue OT - rheumatology OT - scleroderma EDAT- 2024/09/26 06:57 MHDA- 2024/11/23 11:17 CRDT- 2024/09/26 03:23 PHST- 2024/09/09 00:00 [revised] PHST- 2024/07/08 00:00 [received] PHST- 2024/09/11 00:00 [accepted] PHST- 2024/11/23 11:17 [medline] PHST- 2024/09/26 06:57 [pubmed] PHST- 2024/09/26 03:23 [entrez] AID - 10.1111/wrr.13224 [doi] PST - ppublish SO - Wound Repair Regen. 2024 Nov-Dec;32(6):949-959. doi: 10.1111/wrr.13224. Epub 2024 Sep 26. PMID- 40462205 OWN - NLM STAT- MEDLINE DCOM- 20250604 LR - 20250609 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 27 IP - 1 DP - 2025 Jun 3 TI - Artificial intelligence-based Raynaud's quantification index (ARTIX): an objective mobile-based tool for patient-centered assessment of Raynaud's phenomenon. PG - 120 LID - 10.1186/s13075-025-03569-w [doi] LID - 120 AB - BACKGROUND: We aimed to develop an artificial intelligence algorithm able to assess Raynaud's phenomenon (RP) from mobile phone photography, ensuring as a patient-centered, image-based method for RP quantification. METHODS: ARTIX (artificial intelligence-based Raynaud's quantification index) score was developed as a multi-step process of segmentation, decomposition and filters application to mobile phone pictures of the hand. ARTIX was validated by the ability to assess finger response to standardised cold challenge in patients with primary and secondary RP and healthy controls (HC) and compared with thermography as a reference. RESULTS: Forty-five RP patients (91.1% female, mean age 52.2 years, 75.5% secondary RP) were enrolled, along with 22 HC comparable for age and gender. RP patients presented significantly lower ARTIX values than HC both at baseline (p < 0.001) and across all timepoints of the cold challenge (p < 0.01 for all), paralleling a similarly significant difference observed by thermography. ARTIX score was higher in males and in patients taking vasoactive drugs, whereas lower values were obtained in patients with late capillaroscopic pattern, diffuse cutaneous skin subset, or negative for anti-centromere antibodies. ARTIX showed also good ability to discriminate between RP and HC response to cold challenge. CONCLUSION: We developed and validated ARTIX, a novel machine learning-driven method for the objective quantification of RP. Real-life longitudinal studies in patients with RP will determine the value of ARTIX to complement patient self-assessment surrogate measures of RP activity and severity. CI - © 2025. The Author(s). FAU - Di Battista, Marco AU - Di Battista M AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - Rheumatology Unit, University of Pisa, Pisa, Italy. FAU - Colak, Seda AU - Colak S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - Department of Rheumatology, Gulhane Training and Research Hospital, Ankara, Turkey. FAU - Howard, Anna AU - Howard A AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Donadoni, Francesca AU - Donadoni F AD - Procedure Health Limited, London, UK. FAU - Owen-Smith, Chris AU - Owen-Smith C AD - Procedure Health Limited, London, UK. FAU - Rindone, Andrea AU - Rindone A AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - Department of Rheumatology and Medical Science, University of Milan, ASST Gaetano Pini- CTO Institute, Milan, Italy. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Hartley, Collette AU - Hartley C AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Bissell, Lesley-Anne AU - Bissell LA AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, LS7 4SA, UK. f.delgaldo@leeds.ac.uk. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. f.delgaldo@leeds.ac.uk. LA - eng GR - Translational Award (Grant Code 22413)/VAC_/Versus Arthritis/United Kingdom GR - NIHR203331/Leeds Biomedical Research Centre/ PT - Journal Article DEP - 20250603 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Humans MH - Female MH - *Raynaud Disease/diagnosis MH - Male MH - Middle Aged MH - *Artificial Intelligence MH - Adult MH - Aged MH - Thermography/methods MH - *Mobile Applications MH - Cell Phone MH - Photography/methods MH - Algorithms MH - Patient-Centered Care MH - Severity of Illness Index PMC - PMC12131432 OTO - NOTNLM OT - Artificial intelligence OT - Cold challenge OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Thermography COIS- Declarations. Ethics approval and consent to participate: This study was conducted as part of the observational study STRIKE, approved by North East - Newcastle & North Tyneside 2 Research Ethics Committee (IRAS ID: 178638; REC Reference: 15/NE/0211). All participants voluntarily agreed to participate providing written informed consent. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2025/06/04 00:29 MHDA- 2025/06/04 06:27 PMCR- 2025/06/03 CRDT- 2025/06/03 23:48 PHST- 2025/02/11 00:00 [received] PHST- 2025/05/04 00:00 [accepted] PHST- 2025/06/04 06:27 [medline] PHST- 2025/06/04 00:29 [pubmed] PHST- 2025/06/03 23:48 [entrez] PHST- 2025/06/03 00:00 [pmc-release] AID - 10.1186/s13075-025-03569-w [pii] AID - 3569 [pii] AID - 10.1186/s13075-025-03569-w [doi] PST - epublish SO - Arthritis Res Ther. 2025 Jun 3;27(1):120. doi: 10.1186/s13075-025-03569-w. PMID- 19367242 OWN - NLM STAT- MEDLINE DCOM- 20101012 LR - 20151119 IS - 0392-9590 (Print) IS - 0392-9590 (Linking) VI - 28 IP - 2 DP - 2009 Apr TI - Autonomic dysfunction in primary Raynaud's phenomenon. PG - 127-31 AB - AIM: The pathogenesis of Raynaud's phenomenon is still unclear. Neural and intravascular mechanisms are thought to be involved in the pathological process. The role of the autonomic nervous system is continually discussed, with particular attention to over-reactivity of the sympathetic part. The aim of this study was the clinical and electrophysiological analysis of autonomic nervous system function in patients with primary Raynaud's phenomenon. METHODS: Thirty four patients with primary Raynaud's phenomenon and 31 sex and age-matched controls were examined. Neurological examination, modified Low's Questionnaire, orthostatic and sustained handgrip tests, conduction velocity study in three nerves, sympathetic skin response (SSR), and heart rate variability (HRV) during deep breathing and at rest with the fast Fourier transform were performed. RESULTS: In the clinical examinations, 35.3% of the primary Raynaud's patients presented sensory neuropathy, but this was not confirmed in the standard conduction velocity tests. The modified Low's Questionnaire revealed dysautonomy in 82% of the patients. Autonomic regulation during the orthostatic and handgrip tests were within the normal limits. HRV at rest and the E/I ratio were significantly lower in the patient group than in the controls, while HRV spectrum analysis revealed the predominance of the low-frequency band in the patients. CONCLUSIONS: These results indicate the presence of sympathetic dysregulation and impairment of parasympathetic modulation of heart function in primary Raynaud's patients. The different cardiovascular and sudomotor functions are not affected to the same degree. These observations might support the theory of a central impairment of autonomic function in primary Raynaud's phenomenon. Peripheral nerve lesion as a coexisting cause of the observed dysautonomy remains uncertain. FAU - Koszewicz, M AU - Koszewicz M AD - Department of Neurology, Medical University of Wrocław, Wrocław, Poland. magda.koszewicz@onet.pl FAU - Gosk-Bierska, I AU - Gosk-Bierska I FAU - Bilińska, M AU - Bilińska M FAU - Podemski, R AU - Podemski R FAU - Budrewicz, S AU - Budrewicz S FAU - Adamiec, R AU - Adamiec R FAU - Słotwiński, K AU - Słotwiński K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Int Angiol JT - International angiology : a journal of the International Union of Angiology JID - 8402693 SB - IM MH - Adult MH - Autonomic Nervous System/*physiopathology MH - Blood Pressure MH - Case-Control Studies MH - Electric Stimulation MH - Fourier Analysis MH - Hand Strength MH - Heart/*innervation MH - Heart Rate MH - Humans MH - Middle Aged MH - Neural Conduction MH - Neurologic Examination MH - Poland MH - Raynaud Disease/*physiopathology MH - Reaction Time MH - Sensation MH - Skin/*innervation MH - Surveys and Questionnaires MH - Time Factors MH - Young Adult EDAT- 2009/04/16 09:00 MHDA- 2010/10/13 06:00 CRDT- 2009/04/16 09:00 PHST- 2009/04/16 09:00 [entrez] PHST- 2009/04/16 09:00 [pubmed] PHST- 2010/10/13 06:00 [medline] PST - ppublish SO - Int Angiol. 2009 Apr;28(2):127-31. PMID- 19183180 OWN - NLM STAT- MEDLINE DCOM- 20090713 LR - 20260518 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 160 IP - 4 DP - 2009 Apr TI - Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors. PG - 835-43 LID - 10.1111/j.1365-2133.2008.09004.x [doi] AB - BACKGROUND: Digital ulcers (DU) are a major complication in the course of systemic sclerosis (SSc). In recent years, efficacious, but expensive therapies (e.g. iloprost, sildenafil, bosentan) have been shown to improve healing or to reduce the recurrence of DU. For optimal management it would be useful to identify the risk factors for DU. Such statistical analyses have been rare because they require a high number of patients. OBJECTIVES: To identify potential risk factors for DU in patients with SSc. METHODS: We used the registry of the German Network for Systemic Scleroderma and evaluated the data of 1881 patients included by August 2007. We assessed potential risk factors for DU by comparing patients with (24.1%) and without active DU at time of entry (75.9%). RESULTS: Multivariate analysis revealed that male sex, presence of pulmonary arterial hypertension (PAH), involvement of the oesophagus, diffuse skin sclerosis (only when PAH was present), anti-Scl70 antibodies, young age at onset of Raynaud's phenomenon (RP), and elevated erythrocyte sedimentation rate (ESR) significantly impacted on the appearance of DU. Certain combinations increased the patients' probability of presenting with DU, with the highest probability (88%) for male patients with early onset of RP, ESR>30 mm h(-1), anti-Scl70 antibodies and PAH. Patients with DU developed RP, skin sclerosis and organ involvement approximately 2-3 years earlier than patients without DU. CONCLUSIONS: The results reveal possible risk factors for the occurrence of DU in SSc. As DU are prone to local complications, prophylactic vasoactive treatment for patients presenting with these factors may be justified. FAU - Sunderkötter, C AU - Sunderkötter C AD - Department of Dermatology, University of Münster, Von-Esmarch-Str. 58, 48149 Münster, Germany. cord.sunderkoetter@ukmuenster.de FAU - Herrgott, I AU - Herrgott I FAU - Brückner, C AU - Brückner C FAU - Moinzadeh, P AU - Moinzadeh P FAU - Pfeiffer, C AU - Pfeiffer C FAU - Gerss, J AU - Gerss J FAU - Hunzelmann, N AU - Hunzelmann N FAU - Böhm, M AU - Böhm M FAU - Krieg, T AU - Krieg T FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Genth, E AU - Genth E FAU - Schulze-Lohoff, E AU - Schulze-Lohoff E FAU - Meurer, M AU - Meurer M FAU - Melchers, I AU - Melchers I FAU - Riemekasten, G AU - Riemekasten G CN - DNSS Centers LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090113 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 SB - IM MH - Adult MH - Female MH - Fingers MH - Germany MH - Humans MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Raynaud Disease/*etiology/psychology/therapy MH - Risk Assessment MH - Scleroderma, Systemic/*etiology/psychology/therapy MH - Socioeconomic Factors MH - Ulcer/*complications/psychology/therapy FIR - Worm, M IR - Worm M FIR - Klaus, P IR - Klaus P FIR - Rubbert, A IR - Rubbert A FIR - Steinbrink, K IR - Steinbrink K FIR - Grundt, Boris IR - Grundt B FIR - Mierau, Rudolf IR - Mierau R FIR - Hein, R IR - Hein R FIR - Hinrichs, R IR - Hinrichs R FIR - Szeimies, R-M IR - Szeimies RM FIR - Karrer, S IR - Karrer S FIR - Muller, A M IR - Muller AM FIR - Meyering, R IR - Meyering R FIR - Seitz, C IR - Seitz C FIR - Schmidt, E IR - Schmidt E FIR - Lehmann, P IR - Lehmann P FIR - Foeldvari, I IR - Foeldvari I FIR - Reichenberger, F IR - Reichenberger F FIR - Gross, L IR - Gross L FIR - Kuhn, A IR - Kuhn A FIR - Haust, M IR - Haust M FIR - Reich, K IR - Reich K FIR - Becker, M IR - Becker M FIR - Saar, P IR - Saar P FIR - Schmeiser, T IR - Schmeiser T FIR - Fierlbeck, G IR - Fierlbeck G FIR - Kotter, I IR - Kotter I FIR - Lorenz, H-M IR - Lorenz HM FIR - Blank, N IR - Blank N FIR - Grafenstein, K IR - Grafenstein K FIR - Juche, A IR - Juche A FIR - Aberer, E IR - Aberer E FIR - Bali, G IR - Bali G FIR - Fiehn, C IR - Fiehn C FIR - Stadler, R IR - Stadler R FIR - Bartels, V IR - Bartels V FIR - Buslau, M IR - Buslau M FIR - Distler, J IR - Distler J FIR - Sticherling, M IR - Sticherling M EDAT- 2009/02/03 09:00 MHDA- 2009/07/14 09:00 CRDT- 2009/02/03 09:00 PHST- 2009/02/03 09:00 [entrez] PHST- 2009/02/03 09:00 [pubmed] PHST- 2009/07/14 09:00 [medline] AID - BJD9004 [pii] AID - 10.1111/j.1365-2133.2008.09004.x [doi] PST - ppublish SO - Br J Dermatol. 2009 Apr;160(4):835-43. doi: 10.1111/j.1365-2133.2008.09004.x. Epub 2009 Jan 13. PMID- 29473715 OWN - NLM STAT- MEDLINE DCOM- 20190711 LR - 20190901 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 70 IP - 9 DP - 2018 Sep TI - Multinational Qualitative Research Study Exploring the Patient Experience of Raynaud's Phenomenon in Systemic Sclerosis. PG - 1373-1384 LID - 10.1002/acr.23475 [doi] AB - OBJECTIVE: Raynaud's phenomenon (RP) is the most common manifestation of systemic sclerosis (SSc). RP is an episodic phenomenon, not easily assessed in the clinic, leading to reliance on self-report. A thorough understanding of the patient experience of SSc-RP is essential to ensuring that patient-reported outcome (PRO) instruments capture domains important to the target patient population. We report the findings of an international qualitative research study investigating the patient experience of SSc-RP. METHODS: Focus groups of SSc patients were conducted across 3 scleroderma centers in the US and UK, using a topic guide and a priori purposive sampling framework devised by qualitative researchers, SSc patients, and SSc experts. Focus groups were audio recorded, transcribed, anonymized, and analyzed using inductive thematic analysis. Focus groups were conducted until thematic saturation was achieved. RESULTS: Forty SSc patients participated in 6 focus groups conducted in Bath (UK), New Orleans (Louisiana), and Pittsburgh (Pennsylvania). Seven major themes were identified that encapsulate the patient experience of SSc-RP: physical symptoms, emotional impact, triggers and exacerbating factors, constant vigilance and self-management, impact on daily life, uncertainty, and adaptation. The interrelationship of the 7 constituent themes can be arranged within a conceptual map of SSc-RP. CONCLUSION: We have explored the patient experience of SSc-RP in a diverse and representative SSc cohort and identified a complex interplay of experiences that result in significant impact. Work to develop a novel PRO instrument for assessing the severity and impact of SSc-RP, comprising domains/items grounded in the patient experiences of SSc-RP identified in this study, is underway. CI - © 2017, American College of Rheumatology. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and University of Bath, Bath, UK. FAU - Domsic, Robyn T AU - Domsic RT AD - University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. FAU - Saketkoo, Lesley A AU - Saketkoo LA AD - Tulane University School of Medicine, New Orleans, Louisiana. FAU - Almeida, Celia AU - Almeida C AD - University of the West of England, Bristol, UK. FAU - Withey, Jane AU - Withey J AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. FAU - Jay, Hilary AU - Jay H AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - University of Utah and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City. FAU - Ingegnoli, Francesca AU - Ingegnoli F AUID- ORCID: 0000-0002-6727-1273 AD - University of Milano, Milano, Italy. FAU - Dures, Emma AU - Dures E AD - University of the West of England, Bristol, UK. FAU - Robson, Joanna AU - Robson J AD - University of the West of England, Bristol, UK. FAU - McHugh, Neil J AU - McHugh NJ AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and University of Bath, Bath, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester, Salford Royal Hospital NHS Foundation Trust, and Manchester Academic Health Science Centre, Manchester, UK. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - University of Florence, Florence, Italy. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan, Ann Arbor. FAU - Hewlett, Sarah AU - Hewlett S AD - University of the West of England, Bristol, UK. LA - eng GR - I01 CX001183/CX/CSRD VA/United States GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20180816 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Aged MH - Female MH - Focus Groups MH - Humans MH - Male MH - Middle Aged MH - Qualitative Research MH - Raynaud Disease/etiology/*psychology MH - Scleroderma, Systemic/*complications PMC - PMC5962378 MID - NIHMS921080 COIS- Conflicts of Interest: None of the authors report any conflicts of interest relevant to the content of this work EDAT- 2018/02/24 06:00 MHDA- 2019/07/12 06:00 PMCR- 2019/09/01 CRDT- 2018/02/24 06:00 PHST- 2017/08/18 00:00 [received] PHST- 2017/11/14 00:00 [accepted] PHST- 2018/02/24 06:00 [pubmed] PHST- 2019/07/12 06:00 [medline] PHST- 2018/02/24 06:00 [entrez] PHST- 2019/09/01 00:00 [pmc-release] AID - 10.1002/acr.23475 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2018 Sep;70(9):1373-1384. doi: 10.1002/acr.23475. Epub 2018 Aug 16. PMID- 16835313 OWN - NLM STAT- MEDLINE DCOM- 20070221 LR - 20151119 IS - 1060-0280 (Print) IS - 1060-0280 (Linking) VI - 40 IP - 7-8 DP - 2006 Jul-Aug TI - Phosphodiesterase inhibitors in Raynaud's phenomenon. PG - 1388-93 AB - OBJECTIVE: To evaluate the efficacy of the phosphodiesterase type 5 (PDE5) inhibitors in the treatment of Raynaud's phenomenon. DATA SOURCES: Searches of MEDLINE (1966-March 2006) and Web of Science (1980-March 2006) were conducted; search terms were sildenafil, tadalafil, vardenafil, phosphodiesterase, and Raynaud. Studies and case reports published in English were retrieved. Additional references were identified in bibliographic reviews. DATA SYNTHESIS: Several small studies and a number of case reports have described the use of PDE5 inhibitors in patients with either primary or secondary Raynaud's phenomenon. The data from the best designed study show a reduced attack frequency and duration, reduced Raynaud Condition Score, and increased capillary blood flow in patients with secondary Raynaud's phenomenon. CONCLUSIONS: Available evidence suggests that sildenafil may be associated with improved microcirculation, symptomatic relief, and ulcer healing in patients with secondary Raynaud's phenomenon. Limited information suggests similar effects with tadalafil and vardenafil. Improved blood flow and clinical improvements have also been observed in some patients with primary Raynaud's phenomenon treated with PDE5 inhibitors; however, studies have yielded conflicting results. FAU - Levien, Terri L AU - Levien TL AD - Pharmacotherapy Department, College of Pharmacy, Washington State University Spokane, 99210-1495, USA. Levient@wsu.edu LA - eng PT - Journal Article PT - Review DEP - 20060711 PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - 0 (Carbolines) RN - 0 (Imidazoles) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Triazines) RN - 5O8R96XMH7 (Vardenafil Dihydrochloride) RN - 742SXX0ICT (Tadalafil) RN - BW9B0ZE037 (Sildenafil Citrate) RN - EC 3.1.4.35 (3',5'-Cyclic-GMP Phosphodiesterases) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) RN - EC 3.1.4.35 (PDE5A protein, human) SB - IM MH - 3',5'-Cyclic-GMP Phosphodiesterases/antagonists & inhibitors MH - Carbolines/adverse effects/therapeutic use MH - Clinical Trials as Topic MH - Cyclic Nucleotide Phosphodiesterases, Type 5 MH - Humans MH - Imidazoles/adverse effects/therapeutic use MH - Phosphodiesterase Inhibitors/adverse effects/*therapeutic use MH - Piperazines/adverse effects/therapeutic use MH - Purines MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate MH - Sulfones/adverse effects/therapeutic use MH - Tadalafil MH - Triazines/adverse effects/therapeutic use MH - Vardenafil Dihydrochloride RF - 18 EDAT- 2006/07/13 09:00 MHDA- 2007/02/22 09:00 CRDT- 2006/07/13 09:00 PHST- 2006/07/13 09:00 [pubmed] PHST- 2007/02/22 09:00 [medline] PHST- 2006/07/13 09:00 [entrez] AID - aph.1H005 [pii] AID - 10.1345/aph.1H005 [doi] PST - ppublish SO - Ann Pharmacother. 2006 Jul-Aug;40(7-8):1388-93. doi: 10.1345/aph.1H005. Epub 2006 Jul 11. PMID- 33486597 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20220402 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 40 IP - 4 DP - 2021 Apr TI - How do patients define Raynaud's phenomenon? Differences between primary and secondary disease. PG - 1611-1616 LID - 10.1007/s10067-021-05598-7 [doi] AB - INTRODUCTION/OBJECTIVES: To examine how people define Raynaud's phenomenon (RP) based on their lived experiences and explore if differences exist depending on primary or secondary RP diagnosis. METHOD: An international survey was sent to people with RP through health systems, foundations, and social media. Qualitative coding of responses to an open text question regarding one's own definition of RP was performed and themes were identified. The prevalence of themes among the sample and then comparisons between themes among people who self-reported primary versus secondary diagnosis of RP were performed. RESULTS: There were 1345 respondents from 45 countries (mean age 51.5 years, 93% female) who defined RP in their own words; 17% reported primary RP and 83% reported secondary RP (69% of secondary RP was scleroderma-related, n = 927). Over half defined their RP by describing the body parts affected, color changes, pain, and triggers or situations in which an episode occurs. Patients with primary RP more frequently defined RP in terms of its impact on function/quality of life and pain compared to those with secondary RP (34.5% versus 25.3%, respectively, p=0.004; 54.0% versus 46.8%, p=0.05). Patients with secondary RP more frequently included specific body parts, color change, the management of attacks, and other digital vascular complications in their definition of RP. CONCLUSIONS: We have identified differences in how people with primary and secondary RP define RP, in terms of how they feel and function. Our findings have implications for the domains of outcome measures for assessing RP within different patient populations. Key Points • Pain is more often mentioned in primary RP and color change in secondary RP. • Over 25% of patients included reduced the quality of life as part of their RP definition. • The concept of "attack" is used to define RP by only 2% of patients. FAU - Murphy, Susan L AU - Murphy SL AUID- ORCID: 0000-0001-7924-0012 AD - Department of Physical Medicine and Rehabilitation, University of Michigan, 24 Frank Lloyd Wright Drive, Lobby M Suite 3100, Ann Arbor, MI, 48105, USA. sumurphy@umich.edu. AD - VA Ann Arbor Health Care System, Geriatric Research, Education, and Clinical Center (GRECC), 2215 Fuller Rd, Ann Arbor, MI, 48105, USA. sumurphy@umich.edu. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France. AD - Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France. AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Alore, Mary AU - Alore M AD - Peer Mentors Program, University of Michigan Scleroderma Program, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. FAU - Sabbagh, Maya AU - Sabbagh M AD - Peer Mentors Program, University of Michigan Scleroderma Program, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. LA - eng GR - CORECT Visiting Grant 2020/Rennes University Hospital/ GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - K24-AR-063129/AR/NIAMS NIH HHS/United States GR - R01 AR070470/AR/NIAMS NIH HHS/United States GR - AAP JCM2020/Hôpitaux Universitaire du Grand Ouest/ GR - R43 AR063129/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20210124 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM EIN - Clin Rheumatol. 2021 Apr;40(4):1617-1620. doi: 10.1007/s10067-021-05620-y. PMID: 33547561 MH - Female MH - Humans MH - Male MH - Middle Aged MH - Quality of Life MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Localized MH - *Scleroderma, Systemic MH - Surveys and Questionnaires PMC - PMC8080278 MID - NIHMS1689728 OTO - NOTNLM OT - Connective tissue diseases OT - Qualitative analysis OT - Raynaud’s phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- Declarations Ethics Approval: The study was approved with exempt status by the University of Michigan IRB (Study ID: HUM00175143; OHRP IRB Registration Number(s): IRB00000246). EDAT- 2021/01/25 06:00 MHDA- 2021/05/15 06:00 PMCR- 2022/04/01 CRDT- 2021/01/24 20:52 PHST- 2020/12/11 00:00 [received] PHST- 2021/01/17 00:00 [accepted] PHST- 2021/01/12 00:00 [revised] PHST- 2021/01/25 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2021/01/24 20:52 [entrez] PHST- 2022/04/01 00:00 [pmc-release] AID - 10.1007/s10067-021-05598-7 [pii] AID - 10.1007/s10067-021-05598-7 [doi] PST - ppublish SO - Clin Rheumatol. 2021 Apr;40(4):1611-1616. doi: 10.1007/s10067-021-05598-7. Epub 2021 Jan 24. PMID- 20040526 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20220410 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 3 DP - 2010 Mar TI - Effect of the dual endothelin receptor antagonist bosentan on Raynaud's phenomenon secondary to systemic sclerosis: a double-blind prospective, randomized, placebo-controlled pilot study. PG - 583-7 LID - 10.1093/rheumatology/kep413 [doi] AB - OBJECTIVE: To investigate the efficacy of the endothelin receptor antagonist, bosentan, in patients with RP secondary to SSc without pre-existing digital ulcers. METHODS: Single-centre, randomized, prospective, double-blinded comparison of bosentan and placebo. Patients received either 62.5 mg bosentan twice daily for 4 weeks, followed by 125 mg twice daily for 12 weeks or matching doses of placebo. RESULTS: Of the 17 patients enrolled, 16 completed the study and 1 withdrew from the study due to the reversible development of peripheral oedema. Compared with placebo, bosentan did not improve the frequency, duration, pain or severity of RP attacks. However, in contrast to placebo, bosentan significantly improved the functional scores. With respect to baseline, the scleroderma HAQ disability index changes were in favour of bosentan at Weeks 12 (P = 0.03) and 20 (P = 0.01), and the United Kingdom functional score changes at Weeks 8 (P = 0.038) and 16 (P = 0.039). CONCLUSIONS: Bosentan is not effective in SSc-related RP without pre-existing digital ulcers, but it might benefit functional impairment in those patients. TRIAL REGISTRATION: European Union Drug Regulating Authorities Clinical Trials, https://eudract.emea.europa.eu, EudraCT-Nr 2004-002686-21. FAU - Nguyen, Van Anh AU - Nguyen VA AD - Department of Dermatology, Innsbruck Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria. FAU - Eisendle, Klaus AU - Eisendle K FAU - Gruber, Ingrid AU - Gruber I FAU - Hugl, Beate AU - Hugl B FAU - Reider, Daniela AU - Reider D FAU - Reider, Norbert AU - Reider N LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20091229 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antihypertensive Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Drug Administration Schedule MH - *Endothelin Receptor Antagonists MH - Epidemiologic Methods MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications MH - Sulfonamides/*therapeutic use MH - Treatment Outcome EDAT- 2009/12/31 06:00 MHDA- 2010/12/14 06:00 CRDT- 2009/12/31 06:00 PHST- 2009/12/31 06:00 [entrez] PHST- 2009/12/31 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - kep413 [pii] AID - 10.1093/rheumatology/kep413 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Mar;49(3):583-7. doi: 10.1093/rheumatology/kep413. Epub 2009 Dec 29. PMID- 22075318 OWN - NLM STAT- MEDLINE DCOM- 20120309 LR - 20151119 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 22 IP - 6 DP - 2011 Dec TI - Impaired carbon monoxide diffusing capacity as a marker of limited systemic sclerosis. PG - e80-6 LID - 10.1016/j.ejim.2011.05.007 [doi] AB - BACKGROUND: As impairment of diffusing capacity for carbon monoxide (DLCO) likely reflects underlying pulmonary vasculopathy in limited systemic sclerosis (lSSc), we examined whether DLCO could help to distinguish secondary from idiopathic Raynaud's phenomenon (iRP). METHODS: We compared pulmonary function test (PFT) results in 145 lSSc patients and 24 age- and sex-matched iRP patients. RP duration at time of PFT was similar in the two groups. RESULTS: DLCO values were low (<80% of predicted) in 106 (73%) of the 145 lSSc patients, and in 69 (71%) of the 97 patients with early lSSc. Interstitial lung disease (ILD) was found in 10% of lSSc patients. DLCO was significantly lower in lSSc than in iRP (72±15% versus 89±9%, p<0.0001). When evaluated, alveolar capillary membrane conductance (Dm) was markedly lower in lSSc patients without ILD than in iRP patients (45±12% versus 71±2.5%, p=0.003), although capillary blood volume was not different. DLCO was low in 3 iRP patients (12.5%). The sensitivity and specificity of low DLCO values for early lSSc diagnosis in patients with Raynaud's phenomenon were 71% and 87.5%, respectively. Sensitivity was similar to that of anti-centromere-antibodies (75%) and nailfold capillary abnormalities (81%). A DLCO cutoff of <70% had a sensitivity and specificity of 41% and 100%, respectively. In multivariable analysis, age and low DLCO were the only independent predictors of death; the hazard ratio for DLCO ≤50% was 7.9 (95% CI 2.3-26, p=0.0007). CONCLUSION: Isolated DLCO impairment is significantly more frequent in patients with lSSc than in patients with idiopathic iRP. DLCO measurement could be a useful diagnostic tool for lSSc. CI - Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. FAU - Trad, Salim AU - Trad S AD - Service de Médecine Interne, CHU Ambroise Paré, 9 av Charles de Gaulle 92100 Boulogne, France. tradsalim@aol.com FAU - Huong, Du Le Thi AU - Huong du LT FAU - Frances, Camille AU - Frances C FAU - Wechsler, Bertrand AU - Wechsler B FAU - Cacoub, Patrice AU - Cacoub P FAU - Costedoat, Nathalie AU - Costedoat N FAU - Haroche, Julien AU - Haroche J FAU - Piette, Jean-Charles AU - Piette JC FAU - Hanslik, Thomas AU - Hanslik T FAU - Amoura, Zahir AU - Amoura Z LA - eng PT - Comparative Study PT - Journal Article DEP - 20110612 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Biomarkers) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Adult MH - Aged MH - Biomarkers/metabolism MH - Carbon Monoxide/*metabolism MH - Diagnosis, Differential MH - Diffusion MH - Female MH - Follow-Up Studies MH - Humans MH - Kaplan-Meier Estimate MH - Lung Diseases, Interstitial/*diagnosis/metabolism/mortality MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Predictive Value of Tests MH - Pulmonary Alveoli/blood supply/metabolism MH - Pulmonary Circulation/physiology MH - Raynaud Disease/*diagnosis/metabolism/mortality MH - Respiratory Function Tests/*methods MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnosis/metabolism/mortality MH - Sensitivity and Specificity MH - Young Adult EDAT- 2011/11/15 06:00 MHDA- 2012/03/10 06:00 CRDT- 2011/11/15 06:00 PHST- 2010/12/03 00:00 [received] PHST- 2011/04/19 00:00 [revised] PHST- 2011/05/13 00:00 [accepted] PHST- 2011/11/15 06:00 [entrez] PHST- 2011/11/15 06:00 [pubmed] PHST- 2012/03/10 06:00 [medline] AID - S0953-6205(11)00099-9 [pii] AID - 10.1016/j.ejim.2011.05.007 [doi] PST - ppublish SO - Eur J Intern Med. 2011 Dec;22(6):e80-6. doi: 10.1016/j.ejim.2011.05.007. Epub 2011 Jun 12. PMID- 25616446 OWN - NLM STAT- MEDLINE DCOM- 20160414 LR - 20220318 IS - 1590-3478 (Electronic) IS - 1590-1874 (Linking) VI - 36 IP - 7 DP - 2015 Jul TI - Treatment of Raynaud's phenomenon with botulinum toxin type A. PG - 1225-31 LID - 10.1007/s10072-015-2084-6 [doi] AB - Raynaud's phenomenon (RP), an episodic vasospasm of the peripheral arteries, is quite common in general population. The current therapies of RP are limited by efficacy, side effects, and polypharmacy concerns. Botulinum toxin type A (BTX-A) local injections have been reported for the treatment of RP, but the injection sites, concentration and dose of BTX-A were different from each other in previous trials. In addition, so far, there have been no reports concerning local injection of BTX-A in Asian RP patients. Ten patients with RP in China were included in this retrospective study. All the patients had intractable pain and were non-responsive to conservative and/or medical therapy. A patterned BTX-A injection was performed in RP patients, guided by ultrasonography. BTX-A was injected as 20 u/ml devoid of preservatives. Outcomes were measured by ultrasonography, surface temperature, visual analog scale (VAS) for clinical symptoms (pain, numbness, stiffness and swelling), and changes in ulcers or gangrene. Overall, a great improvement in artery flow velocity (P < 0.01), surface temperature (P < 0.01), ulcer and VAS for clinical symptoms, was observed after BTX-A local injection. Complications were very rarely found, and no patients complained of hand weakness and bruise. BTX-A patterned injection guided by ultrasonography might be a useful therapeutic tool in the management of intractable RP. FAU - Zhang, Xiaolong AU - Zhang X AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Xin-Cun Road 389, Shanghai, 200065, China. FAU - Hu, Yong AU - Hu Y FAU - Nie, Zhiyu AU - Nie Z FAU - Song, Ye AU - Song Y FAU - Pan, Yougui AU - Pan Y FAU - Liu, Ying AU - Liu Y FAU - Jin, Lingjing AU - Jin L LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150124 PL - Italy TA - Neurol Sci JT - Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology JID - 100959175 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Body Temperature/drug effects MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Functional Laterality MH - Hand/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Retrospective Studies MH - Ulcer/drug therapy/etiology MH - Ultrasonography MH - Visual Analog Scale EDAT- 2015/01/27 06:00 MHDA- 2016/04/15 06:00 CRDT- 2015/01/25 06:00 PHST- 2014/08/08 00:00 [received] PHST- 2015/01/16 00:00 [accepted] PHST- 2015/01/25 06:00 [entrez] PHST- 2015/01/27 06:00 [pubmed] PHST- 2016/04/15 06:00 [medline] AID - 10.1007/s10072-015-2084-6 [doi] PST - ppublish SO - Neurol Sci. 2015 Jul;36(7):1225-31. doi: 10.1007/s10072-015-2084-6. Epub 2015 Jan 24. PMID- 38325052 OWN - NLM STAT- MEDLINE DCOM- 20240322 LR - 20250605 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 65 DP - 2024 Apr TI - Patients' perspectives on systemic sclerosis-related Raynaud's phenomenon in the feet: A qualitative study from the OMERACT Foot and Ankle Working Group. PG - 152372 LID - S0049-0172(24)00013-1 [pii] LID - 10.1016/j.semarthrit.2024.152372 [doi] AB - OBJECTIVE: To explore, from patients' perspectives, the symptoms and impact of Raynaud's phenomenon (RP) on the feet of patients with systemic sclerosis (SSc-RP), and to identify which foot-related domains are important to patients. METHODS: Forty participants (34 women) with SSc-RP took part in one of six focus groups held in the United Kingdom or United States. Participants were purposively sampled to ensure diversity in disease type, duration, and ethnicity. The topic guide included questions on RP impact, self-management, and treatment expectations. Qualitative content analysis was employed to identify key concepts in the data relating to foot-specific symptoms and their impact. Themes were organized by corresponding domains of potential importance. RESULTS: Twenty-eight participants (70 %) reported experiencing RP in their feet. Five themes were identified corresponding to domains of potential importance: temperature changes, pain, cramping and stiffness, numbness, and color changes. These issues negatively affected participants' lives, impairing walking, driving, and socializing, and causing issues with footwear and hosiery. CONCLUSIONS: This large qualitative study exploring the experiences of patients with SSc-RP in the feet identified several key domains of high importance to patients. SSc-RP is common in the feet, presents in several patterns, and impacts multiple aspects of patients' lives. These findings indicate where future foot-specific interventions for RP could be targeted. Findings from this study improve understanding of what domains are important to patients with SSc-RP affecting the feet and will contribute to the development of a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Chapman, Lara S AU - Chapman LS AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Alcacer-Pitarch, Begonya AU - Alcacer-Pitarch B AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath, Bath, UK; University of Bath, UK. FAU - Flurey, Caroline A AU - Flurey CA AD - School of Social Sciences, College of Health, Science and Society, University of the West of England, Bristol, UK. FAU - Redmond, Anthony C AU - Redmond AC AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; National Institute for Health Research (NIHR) Leeds Biomedical Research Centre, Leeds, UK. FAU - Richards, Pamela AU - Richards P AD - OMERACT Patient Research Partner, Bristol, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Merkel, Peter A AU - Merkel PA AD - Division of Rheumatology, Department of Medicine, Division of Clinical Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, USA. FAU - Proudman, Susanna AU - Proudman S AD - Discipline of Medicine, University of Adelaide and Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia. FAU - Menz, Hylton B AU - Menz HB AD - School of Allied Health, Human Services and Sport, La Trobe University, Melbourne, Victoria, Australia. FAU - Helliwell, Philip S AU - Helliwell PS AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Hannan, Marian T AU - Hannan MT AD - Hinda and Arthur Marcus Institute for Aging Research, Hebrew SeniorLife, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Domsic, Robyn T AU - Domsic RT AD - University of Pittsburgh Medical Center, Pittsburgh, PA, USA. FAU - Saketkoo, Lesley A AU - Saketkoo LA AD - Tulane University School of Medicine, New Orleans, LA, USA. FAU - Shea, Beverley AU - Shea B AD - Clinical Epidemiology Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada. FAU - Siddle, Heidi J AU - Siddle HJ AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. Electronic address: H.Siddle@leeds.ac.uk. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20240202 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - Female MH - Ankle MH - *Scleroderma, Systemic/complications MH - Qualitative Research MH - Pain/complications MH - *Raynaud Disease/etiology OTO - NOTNLM OT - Domains OT - Foot OT - OMERACT OT - Qualitative OT - Raynaud's phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of competing interest The authors have no competing interests to declare. EDAT- 2024/02/08 00:42 MHDA- 2024/03/22 06:44 CRDT- 2024/02/07 18:07 PHST- 2023/10/16 00:00 [received] PHST- 2023/12/11 00:00 [revised] PHST- 2024/01/03 00:00 [accepted] PHST- 2024/03/22 06:44 [medline] PHST- 2024/02/08 00:42 [pubmed] PHST- 2024/02/07 18:07 [entrez] AID - S0049-0172(24)00013-1 [pii] AID - 10.1016/j.semarthrit.2024.152372 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2024 Apr;65:152372. doi: 10.1016/j.semarthrit.2024.152372. Epub 2024 Feb 2. PMID- 17021989 OWN - NLM STAT- MEDLINE DCOM- 20061109 LR - 20220309 IS - 0012-0472 (Print) IS - 0012-0472 (Linking) VI - 131 IP - 40 DP - 2006 Oct 6 TI - [A 56-year-old female patient with Raynaud's syndrome, increased liver enzymes and neuropsychiatric symptoms]. PG - 2213-6 AB - CASE HISTORY: A 56-year-old woman presented with increased liver enzymes (GPT, GOT), arthralgias, Raynaud's syndrome and disturbance of sleep and concentration. FINDINGS AND DIAGNOSIS: Serology and liver biopsy indicated chronic hepatitis C infection (HCV) and viral-induced liver cirrhosis with unremarkable liver synthesizing parameters. An HCV-triggered cryoglobinemia was excluded, but high elevated antinuclear antibodies (ANA) and anti-RNP autoantibodies, typical serological parameters of mixed tissue collagenous (Sharp}s disease), were detectable. Magnetic resonance spectroscopy (H-MRS) was performed to differentiate between cerebral vasculitis and mild hepatic encephalopathy. This detected abnormal pattern of cerebral metabolites (myo-inositol and choline), is specific for HE. TREATMENT AND COURSE: After onset of an antiviral therapy (terferon/ribavirin), low protein diet with supplementation of l-ornithine-l-aspartate the arthralgia and neuropsychiatric symptoms rapidly improved and HCV-RNA PCR became negative. Unfortunately, after cessation of antiviral treatment the patient had a relapse of HCV with a worsening of the arthralgia and the Raynaud symptoms (HCV-triggered Sharp}s disease). CONCLUSION: Even in patients with mildly abnormal liver function and liver cirrhosis it is important to consider (mild) hepatic encephalopathy if neuropsychiatric symptoms occur. FAU - Hass, H G AU - Hass HG AD - Abteilung Innere Medizin 3, Marienhospital, Stuttgart. FAU - Kaiser, S AU - Kaiser S LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - 56-jährige Patientin mit Raynaud-Symptomatik, erhöhten Leberenzymen und neuropsychiatrischen Symptomen. PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antiviral Agents) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Hepatitis C Antibodies) RN - 0 (RNA, Viral) RN - 0 (snRNP Core Proteins) RN - EC 2.6.1.1 (Aspartate Aminotransferases) RN - EC 2.6.1.2 (Alanine Transaminase) SB - IM MH - Alanine Transaminase/blood MH - Antibodies, Antinuclear/blood MH - Antiviral Agents/therapeutic use MH - Arthralgia/etiology MH - Aspartate Aminotransferases/blood MH - Attention MH - Autoantibodies/blood MH - Autoantigens/immunology MH - Brain/metabolism/pathology MH - Diagnosis, Differential MH - Female MH - Hepacivirus/genetics/immunology/isolation & purification MH - Hepatic Encephalopathy/complications/*diagnosis/virology MH - Hepatitis C Antibodies/blood MH - Hepatitis C, Chronic/complications/*diagnosis/drug therapy MH - Humans MH - Liver/*enzymology/pathology/virology MH - Liver Cirrhosis/pathology/virology MH - Magnetic Resonance Imaging MH - Middle Aged MH - Mixed Connective Tissue Disease/diagnosis/etiology MH - RNA, Viral/blood MH - Raynaud Disease/*etiology MH - Sleep Wake Disorders/etiology MH - snRNP Core Proteins EDAT- 2006/10/06 09:00 MHDA- 2006/11/11 09:00 CRDT- 2006/10/06 09:00 PHST- 2006/10/06 09:00 [pubmed] PHST- 2006/11/11 09:00 [medline] PHST- 2006/10/06 09:00 [entrez] AID - 10.1055/s-2006-951354 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2006 Oct 6;131(40):2213-6. doi: 10.1055/s-2006-951354. PMID- 25596148 OWN - NLM STAT- MEDLINE DCOM- 20160104 LR - 20150316 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 98 DP - 2015 Mar TI - Decreased numbers of endothelial progenitor cells in patients in the early stages of systemic sclerosis. PG - 82-7 LID - S0026-2862(15)00005-9 [pii] LID - 10.1016/j.mvr.2015.01.004 [doi] AB - INTRODUCTION: Microangiopathy and endothelial dysfunction are present in the early stages of systemic sclerosis (SSc). Defective vasculogenesis mediated by bone marrow-derived endothelial progenitor cells (EPCs) might be involved in the vascular abnormalities found in SSc. OBJECTIVES: To evaluate the circulating EPC levels and EPC subtypes via flow cytometry and early outgrowth colony-forming units (CFUs) in patients with SSc compared to healthy subjects. METHODS: Thirty-nine female SSc patients (30 in the early stages of SSc) and 44 age-matched healthy women were included. Peripheral blood EPCs were quantified using flow cytometry and by counting the early outgrowth CFUs. RESULTS: The EPCs quantified with flow cytometry and the CFU numbers were significantly lower in SSc patients than in control subjects (155.1 ± 95.1 vs. 241.3 ± 184.2 EPC/10(6) lymphomononuclear cells, p=0.011; 15.4 ± 8.6 vs. 23.5 ± 10.9 CFU, p<0.001; respectively), as well as in the group of patients in the early stages of SSc compared to the controls. Patients with digital ulcers had significantly higher CFU counts than those without ulcers (p=0.013). Among patients with the scleroderma pattern on nailfold capillaroscopy, patients with the late pattern had significantly lower EPC levels than those with the early and active patterns (p=0.046). There were no significant correlations of EPCs or CFU levels with RP duration. CONCLUSIONS: The present study revealed decreased EPCs in SSc patients, including those with early disease onset. These findings suggest that defective vasculogenesis occurs in the early phases of the disease. Therefore, EPCs might be an important therapeutic target for the prevention of vascular complications in SSc patients. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Andrigueti, Fernando V AU - Andrigueti FV AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil. FAU - Arismendi, Maria I AU - Arismendi MI AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil. FAU - Ebbing, Pâmela C C AU - Ebbing PC AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil. Electronic address: cristiane.kayser@unifesp.br. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150113 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Case-Control Studies MH - Endothelial Progenitor Cells/*cytology MH - Endothelium, Vascular/physiopathology MH - Female MH - Flow Cytometry MH - Humans MH - Leukocytes, Mononuclear/cytology MH - Microcirculation MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/blood/physiopathology MH - Scleroderma, Systemic/blood/*pathology MH - Stem Cells OTO - NOTNLM OT - Endothelial progenitor cells OT - Microcirculation OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Vasculogenesis EDAT- 2015/01/18 06:00 MHDA- 2016/01/05 06:00 CRDT- 2015/01/18 06:00 PHST- 2014/09/05 00:00 [received] PHST- 2014/12/12 00:00 [revised] PHST- 2015/01/05 00:00 [accepted] PHST- 2015/01/18 06:00 [entrez] PHST- 2015/01/18 06:00 [pubmed] PHST- 2016/01/05 06:00 [medline] AID - S0026-2862(15)00005-9 [pii] AID - 10.1016/j.mvr.2015.01.004 [doi] PST - ppublish SO - Microvasc Res. 2015 Mar;98:82-7. doi: 10.1016/j.mvr.2015.01.004. Epub 2015 Jan 13. PMID- 37114914 OWN - NLM STAT- MEDLINE DCOM- 20230525 LR - 20230527 IS - 1477-0393 (Electronic) IS - 0748-2337 (Print) IS - 0748-2337 (Linking) VI - 39 IP - 6 DP - 2023 Jun TI - Effects on blood parameters from hand-arm vibrations exposure. PG - 291-297 LID - 10.1177/07482337231173733 [doi] AB - Vibration exposure from handheld tools can affect the hands with neurological symptoms and vibration-induced Raynaud's phenomenon (VRP). The underlying pathophysiological mechanisms are not fully known, however, changes in the composition of blood parameters may contribute to VRP with an increase in blood viscosity and inflammatory response. The aim of this study was to examine the effect on blood parameters in capillary blood from fingers that had been exposed to a vibrating hand-held tool. This study involved nine healthy participants who had been exposed to vibration and an unexposed control group of six participants. Capillary blood samples were collected before and after vibration exposure for the exposed group, and repeated samples also from the control group. The exposed groups were exposed to vibration for a 15-min period or until they reached a 5.0 m/s(2) vibration dose. Analysis of blood status and differential counting of leucocytes was performed on the capillary blood samples. The results of the blood samples showed an increase in mean value for erythrocyte volume fraction (EVF), hemoglobin, red blood cell count, white blood cell count and neutrophils, as well as a decrease of mean cell volume, mean cell hemoglobin, and mean cell hemoglobin concentration. The increase of EVF and neutrophils was statistically significant for samples taken from the index finger but not the little finger. Even though the study was small it showed that an acute vibration exposure to the hands might increase EVF and neutrophilic granulocytes levels in the capillary blood taken from index fingers. FAU - Johansson, Niclas AU - Johansson N AD - Department of Laboratory Medicine, Faculty of Medicine and Health, Örebro University Hospital, Örebro, Sweden. RINGGOLD: 570872 FAU - Ragnebro, Oscar AU - Ragnebro O AD - Department of Occupational and Environmental Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Stjernbrandt, Albin AU - Stjernbrandt A AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. RINGGOLD: 59588 FAU - Graff, Pål AU - Graff P AUID- ORCID: 0000-0003-4928-617X AD - National Institute of Occupational Health, STAMI, Oslo, Norway. RINGGOLD: 70672 FAU - Bryngelsson, Ing-Liss AU - Bryngelsson IL AD - Department of Occupational and Environmental Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Vihlborg, Per AU - Vihlborg P AUID- ORCID: 0000-0002-4256-1880 AD - Department of Geriatrics, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 LA - eng PT - Journal Article DEP - 20230428 PL - England TA - Toxicol Ind Health JT - Toxicology and industrial health JID - 8602702 SB - IM MH - Humans MH - Vibration MH - Fingers/blood supply MH - Erythrocytes MH - *Raynaud Disease/diagnosis MH - Leukocytes MH - *Occupational Diseases PMC - PMC10210194 OTO - NOTNLM OT - Hand-arm vibration OT - Raynaud’s syndrome OT - blood viscosity OT - neutrophilic granulocytes OT - vibration exposure OT - vibration white fingers COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/04/28 12:42 MHDA- 2023/05/25 06:42 PMCR- 2023/05/25 CRDT- 2023/04/28 09:03 PHST- 2023/05/25 06:42 [medline] PHST- 2023/04/28 12:42 [pubmed] PHST- 2023/04/28 09:03 [entrez] PHST- 2023/05/25 00:00 [pmc-release] AID - 10.1177_07482337231173733 [pii] AID - 10.1177/07482337231173733 [doi] PST - ppublish SO - Toxicol Ind Health. 2023 Jun;39(6):291-297. doi: 10.1177/07482337231173733. Epub 2023 Apr 28. PMID- 36214062 OWN - NLM STAT- MEDLINE DCOM- 20230728 LR - 20230731 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 75 IP - 8 DP - 2023 Aug TI - Assessment of the Systemic Sclerosis-Associated Raynaud's Phenomenon Questionnaire: Item Bank and Short-Form Development. PG - 1725-1734 LID - 10.1002/acr.25038 [doi] AB - OBJECTIVE: To develop, refine, and score a novel patient-reported outcome instrument to assess the severity and impact of Raynaud's phenomenon (RP) in systemic sclerosis (SSc). METHODS: The Assessment of Systemic Sclerosis-Associated Raynaud's Phenomenon (ASRAP) questionnaire items were developed with patient insight partner support and grounded in the lived patient experience of SSc-RP. ASRAP items underwent formal qualitative assessment and linguistic testing. An international multicenter study was undertaken to field test the preliminary ASRAP questionnaire. RESULTS: A preliminary 37-item ASRAP questionnaire was supplemented with 2 additional items following expert review to enhance content coverage before undergoing formal linguistic testing to optimize readability. Patient cognitive debriefing interviews were undertaken to enhance comprehension, ambiguity, cognitive difficulty, relevance, and content coverage of both the ASRAP items and instructions. We enrolled 420 SSc patients from scleroderma centers in the UK and US over 2 consecutive winters. Factor analysis with item response theory was undertaken to remove redundant and poorly fitting items. The retained 27-item long-form ASRAP questionnaire was calibrated and scored using the graded response model. A fixed 10-item short-form ASRAP questionnaire was developed using computerized adaptive testing simulations. CONCLUSION: The ASRAP questionnaire has been developed with extensive SSc patient input, with items grounded in the lived experience of SSc-RP to ensure strong content validity, with a focus on how patients feel and function. An advanced psychometric approach with expert input has removed redundant and/or poorly fitting items without eroding content validity. Long- and short-form ASRAP questionnaires have been calibrated and scored to permit formal validation. CI - © 2022 American College of Rheumatology. FAU - Yu, Lan AU - Yu L AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Domsic, Robyn T AU - Domsic RT AUID- ORCID: 0000-0002-2765-0922 AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Saketkoo, Lesley-Ann AU - Saketkoo LA AD - University of Tulane Medical Center, New Orleans, Louisiana. FAU - Withey, Jane AU - Withey J AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust, Bath, UK. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Vanderbilt University, Nashville, Tennessee. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, and NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester, UK, and Salford Royal NHS Foundation Trust, Salford, UK. FAU - Hummers, Laura K AU - Hummers LK AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Royal Free Hospital, London, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust, Bath, UK, and North Bristol NHS Trust and University of Bristol, Bristol, UK. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20230130 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/diagnosis MH - Surveys and Questionnaires MH - *Scleroderma, Localized MH - *Raynaud Disease/diagnosis/etiology MH - Emotions EDAT- 2022/10/11 06:00 MHDA- 2023/07/28 06:43 CRDT- 2022/10/10 04:43 PHST- 2022/09/12 00:00 [revised] PHST- 2022/06/24 00:00 [received] PHST- 2022/10/04 00:00 [accepted] PHST- 2023/07/28 06:43 [medline] PHST- 2022/10/11 06:00 [pubmed] PHST- 2022/10/10 04:43 [entrez] AID - 10.1002/acr.25038 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2023 Aug;75(8):1725-1734. doi: 10.1002/acr.25038. Epub 2023 Jan 30. PMID- 33713089 OWN - NLM STAT- MEDLINE DCOM- 20210316 LR - 20220403 IS - 1426-9686 (Print) IS - 1426-9686 (Linking) VI - 49 IP - 289 DP - 2021 Feb 24 TI - Serum concentration of angiopoietin-like protein 4 in patients with systemic sclerosis. PG - 28-31 AB - Systemic sclerosis (SSc) is a connective tissue disease characterized by endothelial cell damage, perivascular inflammation and tissue hypoxia. Angiopoietin-like protein 4 (ANGPTL4) has been demonstrated to affect vascular permeability, inflammation and oxidative stress, thus may contribute to SSc pathogenesis. AIM: The aim of the study was to evaluate serum ANGPTL4 in systemic sclerosis and correlate it with disease subtype (localized and diffuse, lcSSc and dcSSc respectively), disease duration, skin fibrosis and internal organ involvement. MATERIALS AND METHODS: Twenty-two patients with systemic sclerosis (15 lcSSc, 7 dcSSc) and thirteen healthy controls were analyzed. Clinical and laboratory data were collected including modified Rodnan Skin Score (mRSS), Raynaud's phenomenon, disease duration, digital pitting scars, oesophageal involvement and interstitial lung disease. ANGPTL4 sera concentrations were measured by ELISA. RESULTS: Patients with systemic sclerosis had lower ANGPTL4 serum levers in comparison to healthy controls, however without statistical significance (160.15 ± 117.53 vs. 127.15 ± 83.58 ng/ml; p=0.64). No association between ANGPTL4 levels and disease subtype, disease duration, severity of skin involvement (mRSS) and Raynaud's phenomenon onset was found. CONCLUSIONS: This is the first study evaluating the serum concentration of ANGPTL4 in patients with systemic sclerosis. This study contributes to still undetermined role of ANGPTL4 in the development or progression of systemic sclerosis. Therefore the role of ANGPTL4 in hypoxia-related diseases such as systemic sclerosis needs further research. CI - © 2021 MEDPRESS. FAU - Żółkiewicz, Jakub AU - Żółkiewicz J AD - Department of Dermatology, Medical University of Warsaw, Poland. FAU - Stochmal, Anna AU - Stochmal A AD - Department of Dermatology, Medical University of Warsaw, Poland. FAU - Zaremba, Michał AU - Zaremba M AD - Department of Dermatology, Medical University of Warsaw, Poland. FAU - Hoffmann, Aleksandra AU - Hoffmann A AD - Department of Dermatology, Medical University of Warsaw, Poland. FAU - Czuwara, Joanna AU - Czuwara J AD - Department of Dermatology, Medical University of Warsaw, Poland. FAU - Rudnicka, Lidia AU - Rudnicka L AD - Department of Dermatology, Medical University of Warsaw, Poland. LA - eng PT - Journal Article PL - Poland TA - Pol Merkur Lekarski JT - Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego JID - 9705469 RN - 0 (Angiopoietin-Like Protein 4) SB - IM MH - Angiopoietin-Like Protein 4 MH - Humans MH - *Lung Diseases, Interstitial MH - *Raynaud Disease/etiology MH - *Scleroderma, Diffuse MH - *Scleroderma, Systemic MH - Skin OTO - NOTNLM OT - ANGPTL4 OT - biomarkers OT - hypoxia OT - systemic sclerosis EDAT- 2021/03/14 06:00 MHDA- 2021/03/17 06:00 CRDT- 2021/03/13 12:05 PHST- 2021/03/13 12:05 [entrez] PHST- 2021/03/14 06:00 [pubmed] PHST- 2021/03/17 06:00 [medline] AID - PML289-028 [pii] PST - ppublish SO - Pol Merkur Lekarski. 2021 Feb 24;49(289):28-31. PMID- 38701966 OWN - NLM STAT- MEDLINE DCOM- 20240615 LR - 20250324 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 154 DP - 2024 Jul TI - Relationship between nailfold videocapillaroscopic findings and cardiovascular risk factors. PG - 104693 LID - S0026-2862(24)00042-6 [pii] LID - 10.1016/j.mvr.2024.104693 [doi] AB - BACKGROUND: Nailfold Videocapillaroscopy (NVC) is a valuable tool in the differential diagnosis of Raynaud's phenomenon (RP), present in certain Rheumatic diseases (RD). Knowing that many people have cardiovascular risk factors (CVRF), the main objective was to demonstrate that CVRF and carotid plaques produce NVC alterations. METHODS: Cross-sectional unicentric study carried out from 2020 to 2023. Four groups were formed: subjects with RD and RP, participants with RD without RP, subjects with RP without RD and finally participants without RP or RD (study group). Each subject exhibiting CVRF presented only a single risk factor. The variables collected were: sociodemographic, CVRF (diabetes, tobacco, alcohol (ALC), obesity (OBE), dyslipidemia and arterial hypertension (AH)), diseases, RP, treatments, tortuosities and NVC alterations (ramified capillaries, enlarged capillaries, giant capillaries, haemorrhages and density loss) and carotid ultrasound (CU). RESULTS: 402 subjects were included (76 % women, mean age 51 ± 16 years), 67 % had CVRF, 50 % RP and 38 % RD. Tortuosities were present in 100 % of CVRF participants. A statistically significant association was found between the presence of CVRF and all the NVC alterations: ramified capillaries (OR = 95.6), enlarged capillaries (OR = 59.2), giant capillaries (OR = 8.32), haemorrhages (OR = 17.6) and density loss (OR = 14.4). In particular, an association was found between giant capillaries with AH (p = 0,008) and OBE (p 〈0,001), and haemorrhages and density loss with ALC and OBE (p < 0,001). On the other hand, 40 subjects presented CU plaques (9.9 %), associated with enlarged capillaries (OR = 8.08), haemorrhages (OR = 4.04) and ramified capillaries (OR = 3.01). The pathological intima-media thickness was also associated with haemorrhages (OR = 3.14). CONCLUSIONS: There is a clear association between CVRF and ultrasound atherosclerotic findings in carotid with NVC alterations. These findings are of special interest for a correct NVC interpretation and to avoid false positives in the diagnosis of primary and secondary RP. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Álvarez Andrés, Eva AU - Álvarez Andrés E AD - Autonomous University of Madrid, Severo Ochoa University Hospital, Rheumatology Department, ciudad universitaria cantoblanco, Avda de Orellana s/n, Madrid, Spain. Electronic address: evap.alvarez@estudiante.uam.es. FAU - de Miguel, Eugenio AU - de Miguel E AD - Autonomous University of Madrid, La Paz University Hospital, Rheumatology Department, ciudad universitaria cantoblanco, Paseo de la castellana, 261 Madrid, Spain. FAU - de Yébenes, María Jesús García AU - de Yébenes MJG AD - Department Institute of Musculoskeletal Health (InMusc), c/Mendez Álvaro, 20, Madrid, Spain. FAU - Carmona, Loreto AU - Carmona L AD - Department Institute of Musculoskeletal Health (InMusc), c/Mendez Álvaro, 20, Madrid, Spain. FAU - Miranda, Cristina Gómez AU - Miranda CG AD - Image Radiology Center Dr. Gomez Pereda, c/ Elvira de Hidalgo 6-8, Zaragoza, Spain. FAU - Ramos, Paz Collado AU - Ramos PC AD - Severo Ochoa University Hospital, Rheumatology Department, Avda de Orellana s/n, Madrid Spain. FAU - de la Peña Lefebvre, Paloma García AU - de la Peña Lefebvre PG AD - Faculty of Health Sciences of HM Hospitals, Camilo José Cela University, c/castillo de Alarcón, 49, Madrid, Spain. LA - eng PT - Journal Article DEP - 20240501 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Humans MH - Female MH - Cross-Sectional Studies MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Adult MH - *Heart Disease Risk Factors MH - *Predictive Value of Tests MH - Aged MH - *Capillaries/diagnostic imaging/pathology/physiopathology MH - *Nails/blood supply MH - *Raynaud Disease/diagnostic imaging/diagnosis/epidemiology/physiopathology MH - Risk Assessment MH - Plaque, Atherosclerotic MH - Carotid Artery Diseases/diagnostic imaging/epidemiology OTO - NOTNLM OT - Capillaroscopy OT - Cardiovascular risk factors OT - Carotid ultrasound OT - Nailfold Videocapillaroscopy COIS- Declaration of competing interest The authors declare that there is no conflict of interests regarding the publication of the paper. EDAT- 2024/05/04 11:50 MHDA- 2024/06/16 11:58 CRDT- 2024/05/03 19:20 PHST- 2024/02/28 00:00 [received] PHST- 2024/04/28 00:00 [revised] PHST- 2024/04/30 00:00 [accepted] PHST- 2024/06/16 11:58 [medline] PHST- 2024/05/04 11:50 [pubmed] PHST- 2024/05/03 19:20 [entrez] AID - S0026-2862(24)00042-6 [pii] AID - 10.1016/j.mvr.2024.104693 [doi] PST - ppublish SO - Microvasc Res. 2024 Jul;154:104693. doi: 10.1016/j.mvr.2024.104693. Epub 2024 May 1. PMID- 18576359 OWN - NLM STAT- MEDLINE DCOM- 20080808 LR - 20080715 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 58 IP - 7 DP - 2008 Jul TI - Prognostic model based on nailfold capillaroscopy for identifying Raynaud's phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination). PG - 2174-82 LID - 10.1002/art.23555 [doi] AB - OBJECTIVE: To construct a prognostic index based on nailfold capillaroscopic examinations that is capable of predicting the 5-year transition from isolated Raynaud's phenomenon (RP) to RP secondary to scleroderma spectrum disorders (SSDs). METHODS: The study involved 104 consecutive adult patients with a clinical history of isolated RP, and the index was externally validated in another cohort of 100 patients with the same characteristics. Both groups were followed up for 1-8 years. Six variables were examined because of their potential prognostic relevance (branching, enlarged and giant loops, capillary disorganization, microhemorrhages, and the number of capillaries). RESULTS: The only factors that played a significant prognostic role were the presence of giant loops (hazard ratio [HR] 2.64, P = 0.008) and microhemorrhages (HR 2.33, P = 0.01), and the number of capillaries (analyzed as a continuous variable). The adjusted prognostic role of these factors was evaluated by means of multivariate regression analysis, and the results were used to construct an algorithm-based prognostic index. The model was internally and externally validated. CONCLUSION: Our prognostic capillaroscopic index identifies RP patients in whom the risk of developing SSDs is high. This model is a weighted combination of different capillaroscopy parameters that allows physicians to stratify RP patients easily, using a relatively simple diagram to deduce the prognosis. Our results suggest that this index could be used in clinical practice, and its further inclusion in prospective studies will undoubtedly help in exploring its potential in predicting treatment response. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Rheumatology, Instituto Gaetano Pini, University of Milan, Milan, Italy. francesca.ingegnoli@unimi.it FAU - Boracchi, Patrizia AU - Boracchi P FAU - Gualtierotti, Roberta AU - Gualtierotti R FAU - Lubatti, Chiara AU - Lubatti C FAU - Meani, Laura AU - Meani L FAU - Zahalkova, Lenka AU - Zahalkova L FAU - Zeni, Silvana AU - Zeni S FAU - Fantini, Flavio AU - Fantini F LA - eng PT - Clinical Trial PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/*diagnosis MH - Risk Assessment MH - Risk Factors MH - Scleroderma, Systemic/*diagnosis MH - Severity of Illness Index EDAT- 2008/06/26 09:00 MHDA- 2008/08/09 09:00 CRDT- 2008/06/26 09:00 PHST- 2008/06/26 09:00 [pubmed] PHST- 2008/08/09 09:00 [medline] PHST- 2008/06/26 09:00 [entrez] AID - 10.1002/art.23555 [doi] PST - ppublish SO - Arthritis Rheum. 2008 Jul;58(7):2174-82. doi: 10.1002/art.23555. PMID- 40835242 OWN - NLM STAT- MEDLINE DCOM- 20250820 LR - 20250820 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 18 IP - 8 DP - 2025 Aug 19 TI - Three distinct presentations of systemic sclerosis in patients with previous silica dust exposure. LID - e264237 [pii] LID - 10.1136/bcr-2024-264237 [doi] AB - This case report encompasses three patients who had worked in the construction industry. All three patients had significant silica dust exposure and were subsequently diagnosed with systemic sclerosis (SSc). Despite variations in clinical presentation, including Raynaud's phenomenon, small bowel involvement and skin thickening, commonalities in occupational history and smoking status underscore the potential role of silica exposure as a trigger for autoimmune diseases. Erasmus syndrome is a rare condition characterised by the coexistence of SSc and silicosis and is often associated with prolonged occupational exposure to silica dust. It is important to note that these patients did not present with silicosis or interstitial lung disease, which has historically been associated with Erasmus syndrome. These cases highlight the importance of understanding SSc's potentially life-threatening aspects. It is associated with significant morbidity and mortality owing to multiorgan involvement. They also highlighted various clinical phenotypes of the disease, including serological tests and treatments. CI - © BMJ Publishing Group Limited 2025. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Landers, David AU - Landers D AUID- ORCID: 0009-0000-7762-3394 AD - Department of Rheumatology, Cork University Hospital, Cork, Ireland davidlanders17@gmail.com. FAU - Hehir, Davida AU - Hehir D AD - Department of Rheumatology, Cork University Hospital, Cork, Ireland. FAU - Murphy, Gráinne AU - Murphy G AD - Department of Rheumatology, Cork University Hospital, Cork, Ireland. LA - eng PT - Case Reports PT - Journal Article DEP - 20250819 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 7631-86-9 (Silicon Dioxide) RN - 0 (Dust) SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/etiology/complications MH - *Silicon Dioxide/adverse effects MH - *Occupational Exposure/adverse effects MH - Male MH - *Dust MH - *Silicosis/complications/diagnosis MH - Middle Aged MH - Female MH - Raynaud Disease/etiology MH - Construction Industry OTO - NOTNLM OT - Connective tissue disease OT - Dermatology OT - Immunology COIS- Competing interests: None declared. EDAT- 2025/08/21 00:30 MHDA- 2025/08/21 00:31 CRDT- 2025/08/20 20:43 PHST- 2025/08/21 00:31 [medline] PHST- 2025/08/21 00:30 [pubmed] PHST- 2025/08/20 20:43 [entrez] AID - 18/8/e264237 [pii] AID - 10.1136/bcr-2024-264237 [doi] PST - epublish SO - BMJ Case Rep. 2025 Aug 19;18(8):e264237. doi: 10.1136/bcr-2024-264237. PMID- 40461743 OWN - NLM STAT- MEDLINE DCOM- 20250708 LR - 20250708 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 7 DP - 2025 Jul TI - Clinical features of anti-RNP-positive primary Sjögren's syndrome. PG - 2911-2917 LID - 10.1007/s10067-025-07499-5 [doi] AB - OBJECTIVE: This study sought to explore the clinical features exhibited by individuals with anti-RNP antibodies who had primary Sjögren's syndrome (pSS). METHODS: Various clinical data of 410 pSS patients from our hospital between September 2018 and August 2023 were retrospectively analysed. Each individual fulfilled the 2016 American College of Rheumatology/European League Against Rheumatism (EULAR) classification criteria for pSS. Comparative analyses were conducted between pSS individuals with and without anti-RNP antibodies. Logistic regression analysis was utilized to ascertain relevant factors. RESULTS: Among the cohort, 38 (9.3%) of pSS patients were anti-RNP positive and 372 were anti-RNP negative. Anti-RNP-positive individuals had a higher median EULAR Sjögren's syndrome disease activity index at diagnosis (p = 0.001) and more frequent extraglandular manifestations (p < 0.001), including articular, pulmonary and muscular involvement (p = 0.001, p < 0.001 and p = 0.024, respectively) and Raynaud's phenomenon (p < 0.001). Additionally, patients possessing anti-RNP antibodies had a higher frequency of hypergammaglobulinemia and positive antinuclear and anti-Ro/SSA antibodies (p < 0.001, p = 0.006 and p = 0.042, respectively), but a lower frequency of anti-centromere positivity (p = 0.010). Multivariate analysis identified anti-RNP positivity as independent variable significantly associated with interstitial lung disease (OR = 2.60, 95% CI 1.22-5.53; p = 0.014) and Raynaud's phenomenon (OR = 6.26, 95% CI 3.01-13.01; p < 0.001) in pSS patients. CONCLUSION: The presence of anti-RNP antibodies may defined a specific subset of pSS patients characterized by distinct phenotypic attributes, especially in terms of higher prevalence of interstitial lung disease and Raynaud's phenomenon. Key Points • Anti-RNP antibodies were detected in 9.3% of patients with primary Sjögren's syndrome (pSS), which was related to increased systemic disease activity and more frequent extraglandular manifestations. • pSS patients carrying anti-RNP antibodies displayed higher prevalence of interstitial lung disease, Raynaud's phenomenon and hypergammaglobulinemia. • Anti-RNP positivity was identified as independent predictors of interstitial lung disease and Raynaud's phenomenon in pSS. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Cheng, Fang AU - Cheng F AUID- ORCID: 0000-0002-4980-0952 AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. chengfangsmmu@126.com. FAU - Wang, Yan-Ling AU - Wang YL AUID- ORCID: 0009-0004-3445-2184 AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Ai, Xiang-Yan AU - Ai XY AUID- ORCID: 0009-0006-8588-343X AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Liu, Yang AU - Liu Y AUID- ORCID: 0009-0006-7448-218X AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Zhu, Zhen-Hang AU - Zhu ZH AUID- ORCID: 0009-0009-0258-0460 AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Zhang, Ke-Ke AU - Zhang KK AUID- ORCID: 0000-0003-0927-6540 AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Zhao, Fu-Tao AU - Zhao FT AUID- ORCID: 0009-0005-3223-3401 AD - Department of Rheumatology and Immunology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. ftzhao@moisten.org. LA - eng GR - JYJC202408/Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine/ PT - Comparative Study PT - Journal Article DEP - 20250603 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Ribonucleoproteins) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Retrospective Studies MH - Humans MH - Male MH - Female MH - Adult MH - Middle Aged MH - Logistic Models MH - *Sjogren's Syndrome/blood/complications/immunology MH - *Ribonucleoproteins/immunology MH - *Antibodies, Antinuclear/blood/immunology MH - Raynaud Disease/immunology MH - Severity of Illness Index MH - Lung Diseases, Interstitial/immunology OTO - NOTNLM OT - Autoantibodies OT - Interstitial lung disease OT - Raynaud’s phenomenon OT - Sjögren’s syndrome OT - U1 snRNP COIS- Declarations. Ethics approval: The study was approved by the ethics committee of Ninth People’s Hospital, and written informed consent was waived as the retrospective nature of this study. Conflict of interest: The authors declare they have no conflicts of interest. EDAT- 2025/06/04 00:29 MHDA- 2025/07/07 12:28 CRDT- 2025/06/03 23:30 PHST- 2024/09/18 00:00 [received] PHST- 2025/05/15 00:00 [accepted] PHST- 2025/05/14 00:00 [revised] PHST- 2025/07/07 12:28 [medline] PHST- 2025/06/04 00:29 [pubmed] PHST- 2025/06/03 23:30 [entrez] AID - 10.1007/s10067-025-07499-5 [pii] AID - 10.1007/s10067-025-07499-5 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Jul;44(7):2911-2917. doi: 10.1007/s10067-025-07499-5. Epub 2025 Jun 3. PMID- 32744019 OWN - NLM STAT- MEDLINE DCOM- 20210824 LR - 20210824 IS - 2589-451X (Electronic) IS - 0255-2922 (Linking) VI - 40 IP - 4 DP - 2020 Aug TI - Effectiveness of Chinese herbal medicine for primary Raynaud's phenomenon: a systematic review and Meta-analysis of randomized controlled trials. PG - 509-517 LID - 10.19852/j.cnki.jtcm.2020.04.001 [doi] AB - OBJECTIVE: To evaluate the effectiveness of Chinese herbal medicine for primary Raynaud's phenomenon (PRP). METHODS: The Cochrane Central Register of Controlled Trials, PubMed, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, China Science and Technology Journal Database, and Wanfang Database were searched up to February 13, 2018. Randomized controlled trials (RCTs) on treatment of PRP with Chinese herbal medicine compared with placebo, blank control, lifestyle changes, or calcium antagonists were identified and reviewed. The quality of included trials was assessed using a risk of bias tool. RESULTS: Eight RCTs involving 674 participants were included. The methodological quality of the included trials was generally poor. Meta-analysis of two trials showed that Buyang Huanwu Tang plus Danggui Sini Tang produced greater improvement in global symptoms than nifedipine. One trial showed that Danggui Sini Tang and a self-composed Chinese herbal medicine decoction, respectively, produced greater improvement in global symptoms than nifedipine alone. In one trial, modified Danggui Sini Tang showed greater improvement in global symptoms and arterial peak systolic velocity compared with nifedipine. One trial showed that Jiejing Tongmi Tang produced greater improvement in global symptoms, plasma endothelin, and plasma nitric oxide than cinepazide maleate injection. However, Jiejing Tongmi Tang did not produce a significant difference in skin temperature and peripheral artery blood stream drawing after cold pressor testing compared with cinepazide maleate injection. None of the trials reported frequency of attacks, duration of attacks, participant preference scores, or adverse events. CONCLUSION: Chinese herbal medicine may have a positive effective on PRP. However, owing to weak methodology, the benefits of Chinese herbal medicine for PRP are inconclusive. More rigorously designed studies are needed to confirm these findings. FAU - Zhang, Jinchao AU - Zhang J AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. AD - Institute of Acupuncture and Moxibustion, China Academy of Chinese Medical Sciences, Beijing100700, China. FAU - Hu, Jing AU - Hu J AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. FAU - He, Xiujuan AU - He X AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. FAU - Meng, Yujiao AU - Meng Y AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. FAU - Chen, Guangkun AU - Chen G AD - Institute of Informationon Traditional Chinese Medicine, China Academy of Chinese Medical Sciences, Beijing 100700, China. FAU - Chen, Zhaoxia AU - Chen Z AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. FAU - Lü, Jingjing AU - Lü J AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. FAU - Li, Ping AU - Li P AD - Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital University of Medicine Sciences, Beijing 100010, China. LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review PL - China TA - J Tradit Chin Med JT - Journal of traditional Chinese medicine = Chung i tsa chih ying wen pan JID - 8211546 RN - 0 (Drugs, Chinese Herbal) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Drugs, Chinese Herbal/*therapeutic use MH - Female MH - Humans MH - Male MH - Middle Aged MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - Chinese herbal medicine OT - Meta-analysi OT - Primary Raynaud's phenomenon OT - Randomized controlled trial OT - Systematic review EDAT- 2020/08/04 06:00 MHDA- 2021/08/25 06:00 CRDT- 2020/08/04 06:00 PHST- 2020/08/04 06:00 [entrez] PHST- 2020/08/04 06:00 [pubmed] PHST- 2021/08/25 06:00 [medline] AID - 3045 [pii] AID - 10.19852/j.cnki.jtcm.2020.04.001 [doi] PST - ppublish SO - J Tradit Chin Med. 2020 Aug;40(4):509-517. doi: 10.19852/j.cnki.jtcm.2020.04.001. PMID- 29866668 OWN - NLM STAT- MEDLINE DCOM- 20181102 LR - 20200604 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2018 DP - 2018 Jun 4 TI - Case of primary Sjogren's syndrome preceded by dystonia. LID - bcr-2017-223468 [pii] LID - 10.1136/bcr-2017-223468 [doi] LID - bcr2017223468 AB - There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association. CI - © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. FAU - Ararat, Kerime AU - Ararat K AD - Department of Medicine, Neurology, University of Massachusetts Medical School, Worcester, Massachusetts, USA. FAU - Berrios, Idanis AU - Berrios I AD - Department of Neurology, University of Massachusetts School of Medicine, Worcester, Massachusetts, USA. FAU - Hannoun, Anas AU - Hannoun A AD - Department of Medicine, Neurology, University of Massachusetts Medical School, Worcester, Massachusetts, USA. FAU - Ionete, Carolina AU - Ionete C AD - Neurology, University of Massachusetts, Worcester, Massachusetts, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20180604 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) SB - IM MH - Adult MH - Antibodies, Antinuclear/immunology MH - Dystonia/*diagnosis/etiology/immunology MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Migraine Disorders/diagnosis/etiology/immunology MH - Neuralgia/diagnosis/etiology/immunology MH - Primary Dysautonomias/diagnosis/etiology/immunology MH - Raynaud Disease/diagnosis/etiology/immunology MH - Salivary Glands, Minor/pathology MH - Sensation Disorders/diagnosis/etiology/immunology MH - Sjogren's Syndrome/complications/*diagnosis/drug therapy/immunology MH - Syncope/diagnosis/etiology/immunology MH - Urinary Bladder, Neurogenic/diagnosis/etiology/immunology PMC - PMC5990091 OTO - NOTNLM OT - Sjogren’s syndrome OT - movement disorders (other than Parkinsons) OT - musculoskeletal and joint disorders COIS- Competing interests: CI: Biogen Idec and Genzyme. She has received compensations for consulting and advisory boards from Genzyme and TEVA. IB has received compensation for consulting in advisory board from Genzyme. EDAT- 2018/06/06 06:00 MHDA- 2018/11/06 06:00 PMCR- 2020/06/04 CRDT- 2018/06/06 06:00 PHST- 2018/06/06 06:00 [entrez] PHST- 2018/06/06 06:00 [pubmed] PHST- 2018/11/06 06:00 [medline] PHST- 2020/06/04 00:00 [pmc-release] AID - bcr-2017-223468 [pii] AID - 10.1136/bcr-2017-223468 [doi] PST - epublish SO - BMJ Case Rep. 2018 Jun 4;2018:bcr2017223468. doi: 10.1136/bcr-2017-223468. PMID- 16678012 OWN - NLM STAT- MEDLINE DCOM- 20060803 LR - 20131121 IS - 0891-5849 (Print) IS - 0891-5849 (Linking) VI - 40 IP - 10 DP - 2006 May 15 TI - Postocclusive reactive hyperemia inversely correlates with urinary 15-F2t-isoprostane levels in systemic sclerosis. PG - 1732-7 AB - Microvascular dysfunction and increased oxidative stress are major hallmarks of the systemic sclerosis disease process. The primary objective of this study was to test whether there is a link between peak postocclusive hyperemia and urinary levels of the F2-isoprostane 15-F2t-IsoP (8-iso-PGF2alpha) in patients suffering from systemic sclerosis. We enrolled 43 patients suffering from systemic sclerosis, 33 patients with primary Raynaud's phenomenon (RP), and 25 healthy volunteers. Microvascular function was assessed using the postocclusive hyperemia monitored by laser Doppler flowmetry. Endothelium-independent response was monitored after 0.4 mg sublingual nitroglycerin. Oxidative stress status was assessed by urinary levels of the F2-isoprostane 15-F2t-IsoP using GC-MS. The peak postocclusive vascular conductance was altered in subjects with systemic sclerosis and primary RP compared to controls (respectively 28 (7-48), 30 (13-48), and 39.9 (13-63) mV/mm Hg, p = 0.01). F2-isoprostanes were increased in the systemic sclerosis group compared to primary Raynaud's phenomenon and healthy controls (respectively 230 (155-387), 182 (101-284), and 207 (109-291) pg/mg, p = 0.006). In patients suffering from systemic sclerosis, there was a significant inverse correlation between F2-isoprostanes and postocclusive hyperemia, expressed as raw data (R = -0.45, p = 0.007) or as an increase over baseline (R = -0.28, p = 0.04). Conversely, no correlation was found with the nitroglycerin response. In conclusion, we provide evidence that there is an inverse correlation between postocclusive hyperemia and urinary F2-isoprostane levels in patients suffering from systemic sclerosis. Whether oxygen free radicals initiate the vascular dysfunction or whether there is an initial trigger that initiates both processes will need to be further clarified in future studies. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - INSERM ESPRI HP2 Laboratory, EA 3745, Grenoble Medical School, Grenoble, France. Jean-Luc.Cracowski@ujf-grenoble.fr FAU - Kom, Ghainsom D AU - Kom GD FAU - Salvat-Melis, Muriel AU - Salvat-Melis M FAU - Renversez, Jean-Charles AU - Renversez JC FAU - McCord, Gregg AU - McCord G FAU - Boignard, Aude AU - Boignard A FAU - Carpentier, Patrick H AU - Carpentier PH FAU - Schwedhelm, Edzard AU - Schwedhelm E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060207 PL - United States TA - Free Radic Biol Med JT - Free radical biology & medicine JID - 8709159 RN - 0 (Interleukin-1) RN - 0 (Interleukin-6) RN - 27415-26-5 (8-epi-prostaglandin F2alpha) RN - B7IN85G1HY (Dinoprost) SB - IM CIN - Free Radic Biol Med. 2006 May 15;40(10):1698-9. doi: 10.1016/j.freeradbiomed.2006.03.001. PMID: 16678008 MH - Dinoprost/*analogs & derivatives/urine MH - Humans MH - Hyperemia/*physiopathology MH - Interleukin-1/blood MH - Interleukin-6/blood MH - Laser-Doppler Flowmetry MH - Lipid Peroxidation/physiology MH - Microcirculation/*pathology MH - Oxidative Stress/physiology MH - Raynaud Disease/pathology/physiopathology/urine MH - Scleroderma, Systemic/pathology/*physiopathology/*urine MH - Skin/blood supply EDAT- 2006/05/09 09:00 MHDA- 2006/08/04 09:00 CRDT- 2006/05/09 09:00 PHST- 2005/11/04 00:00 [received] PHST- 2006/01/04 00:00 [revised] PHST- 2006/01/09 00:00 [accepted] PHST- 2006/05/09 09:00 [pubmed] PHST- 2006/08/04 09:00 [medline] PHST- 2006/05/09 09:00 [entrez] AID - S0891-5849(06)00041-4 [pii] AID - 10.1016/j.freeradbiomed.2006.01.014 [doi] PST - ppublish SO - Free Radic Biol Med. 2006 May 15;40(10):1732-7. doi: 10.1016/j.freeradbiomed.2006.01.014. Epub 2006 Feb 7. PMID- 34256627 OWN - NLM STAT- MEDLINE DCOM- 20220920 LR - 20220920 IS - 1708-539X (Electronic) IS - 1708-5381 (Linking) VI - 30 IP - 5 DP - 2022 Oct TI - Outcomes of periarterial sympathectomy in patients with digital ischemia. PG - 859-866 LID - 10.1177/17085381211032854 [doi] AB - INTRODUCTION: Digital ischemia with subsequent severe pain and tissue loss is often difficult to treat, with no obvious guidelines or strong evidence in the literature to support a specific treatment modality. Patients who fail medical treatment remain with very limited surgical options due to the difficulty of any intervention in this "no man's land" area of the hand, as described since 1918. Extended distal periarterial sympathectomy is reported as an effective treatment option since the eighties of last century. The procedure entails large incisions and major technical difficulties. In this study, we describe a less invasive approach with very promising results and equally high success rates. MATERIALS AND METHODS: This was a prospective study. All patients with severe digital ischemia manifesting with bluish discoloration, ulceration, and/or dry gangrene who failed medical treatment underwent distal periarterial sympathectomy for the radial and ulnar arteries, with added digital sympathectomy in very severe cases. Primary endpoints were ulcer healing and improvement in pain scores assessed by Visual Analog Scale pain scoring system. Secondary endpoints included complications and amputation rates. RESULTS: This study recruited 17 patients between January 2019 and January 2020. The mean follow-up was 14.6 months. The mean age was 33.71 (±SD 13.14) years. 41% were males. 59% suffered from vasculitis, 35% of patients had dry gangrene, and 71% had ulcers. Periarterial radial and ulnar sympathectomy was performed for all cases, with digital sympathectomy for 12 fingers. We had 50% complete ulcer healing within 1 month (p = 0.031), and 100% were completely healed at 6 months (p < 0.001). Pain scores showed significant reductions at 1 (p = 0.001) and 6 months (p < 0.001) of follow-up. CONCLUSION: Distal periarterial sympathectomy demonstrates high success rates in terms of pain relief and ulcer healing in severe digital ischemia. FAU - Elshabrawy, Ahmed A AU - Elshabrawy AA AUID- ORCID: 0000-0002-9876-7271 AD - Department of Vascular and Endovascular Surgery, Faculty of Medicine, 68780Mansoura University, Mansoura, Egypt. FAU - Elkassaby, Mohammed AU - Elkassaby M AUID- ORCID: 0000-0002-7263-426X AD - Department of Vascular and Endovascular Surgery, Faculty of Medicine, 68780Mansoura University, Mansoura, Egypt. AD - Department of Vascular and Endovascular Surgery, St James's University Hospital, Dublin, Ireland. FAU - Abdelgawad, Mohamed S AU - Abdelgawad MS AUID- ORCID: 0000-0002-5558-4133 AD - Department of Vascular and Endovascular Surgery, Faculty of Medicine, 68780Mansoura University, Mansoura, Egypt. FAU - Atif, Ehab AU - Atif E AD - Department of General Surgery, Faculty of Medicine, Mansoura University, Mansoura, Egypt. FAU - Megahed, Abdelsalam AU - Megahed A AD - Department of Vascular and Endovascular Surgery, Faculty of Medicine, 68780Mansoura University, Mansoura, Egypt. FAU - Regal, Samer AU - Regal S AD - Department of Vascular and Endovascular Surgery, Faculty of Medicine, 68780Mansoura University, Mansoura, Egypt. LA - eng PT - Journal Article DEP - 20210714 PL - England TA - Vascular JT - Vascular JID - 101196722 SB - IM MH - Adult MH - Female MH - Fingers/blood supply/surgery MH - Gangrene/complications/surgery MH - Humans MH - Ischemia/diagnostic imaging/surgery MH - Male MH - Pain MH - Prospective Studies MH - *Raynaud Disease/complications/surgery MH - Sympathectomy/adverse effects/methods MH - Ulcer/surgery MH - Ulnar Artery OTO - NOTNLM OT - digital ischemia OT - periarterial sympathectomy OT - raynaud’s EDAT- 2021/07/15 06:00 MHDA- 2022/09/21 06:00 CRDT- 2021/07/14 05:31 PHST- 2021/07/15 06:00 [pubmed] PHST- 2022/09/21 06:00 [medline] PHST- 2021/07/14 05:31 [entrez] AID - 10.1177/17085381211032854 [doi] PST - ppublish SO - Vascular. 2022 Oct;30(5):859-866. doi: 10.1177/17085381211032854. Epub 2021 Jul 14. PMID- 27873751 OWN - NLM STAT- MEDLINE DCOM- 20170301 LR - 20181113 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2016 DP - 2016 Nov 8 TI - Rheumatoid vasculitis: early presentation of rheumatoid arthritis. LID - 10.1136/bcr-2016-217557 [doi] LID - bcr2016217557 AB - Rheumatoid vasculitis is a rare and late complication of rheumatoid arthritis and may affect small-to-medium-sized vessels. Here, we report a case of a 49-year-old man who presented with amaurosis fugax in the left eye, symmetric polyarthritis, Raynaud's symptoms and paraesthesia in both lower extremities. The patient subsequently experienced right foot drop, nail fold infracts and gangrene of his right second toe. He was found to have a high titre of rheumatoid factor and treatment with rituximab and high dose of corticosteroids led to significant improvement of his symptoms. This is rare case describing the early onset of rheumatoid vasculitis in a patient with rheumatoid arthritis. CI - 2016 BMJ Publishing Group Ltd. FAU - Abdulqader, Yasir AU - Abdulqader Y AD - Department of Internal Medicine, Maricopa Integrated Health System, Phoenix, Arizona, USA. FAU - Al-Ani, Muhsen AU - Al-Ani M AD - Maricopa Medical Center, Phoenix, Arizona, USA. FAU - Parperis, Konstantinos AU - Parperis K AD - Department of Medicine, Maricopa Medical Center and University of Arizona College of Medicine, Phoenix, Phoenix, Arizona, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20161108 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Arthritis, Rheumatoid/*complications/*diagnosis/drug therapy MH - Humans MH - Male MH - Middle Aged MH - Musculoskeletal Pain/etiology MH - Raynaud Disease/etiology MH - Rheumatoid Vasculitis/*etiology PMC - PMC5129099 COIS- Conflicts of Interest: None declared. EDAT- 2016/11/23 06:00 MHDA- 2017/03/03 06:00 PMCR- 2018/11/08 CRDT- 2016/11/23 06:00 PHST- 2016/11/23 06:00 [entrez] PHST- 2016/11/23 06:00 [pubmed] PHST- 2017/03/03 06:00 [medline] PHST- 2018/11/08 00:00 [pmc-release] AID - bcr-2016-217557 [pii] AID - 10.1136/bcr-2016-217557 [doi] PST - epublish SO - BMJ Case Rep. 2016 Nov 8;2016:bcr2016217557. doi: 10.1136/bcr-2016-217557. PMID- 24110975 OWN - NLM STAT- MEDLINE DCOM- 20150817 LR - 20200928 IS - 2694-0604 (Electronic) IS - 2375-7477 (Linking) VI - 2013 DP - 2013 TI - Scleroderma capillary pattern identification using texture descriptors and ensemble classification. PG - 5473-6 LID - 10.1109/EMBC.2013.6610788 [doi] AB - Various connective tissue diseases lead to morphological alternations of blood capillaries. Consequently, observation of the capillaries at the finger nailfold - nailfold capillaroscopy (NC) - is a standard method for diagnosing diseases such as scleroderma or Raynaud's phenomenon. This is typically performed through manual inspection by an expert to lead to a determination of one of the established NC scleroderma patterns (early, active, and late). In this paper, we present an automated method of analysing nailfold capillaroscopy images and categorising them into NC patterns. For this purpose, we extract a carefully chosen set of texture features from the images and employ an ensemble classification approach to arrive at decisions for each captured finger which are then aggregated to form a diagnosis for the patient. Experimental results on a set of 60 NC images from 16 subjects demonstrate the accuracy and usefulness of our presented approach. FAU - Schaefer, Gerald AU - Schaefer G FAU - Krawczyk, Bartosz AU - Krawczyk B FAU - Doshi, Niraj P AU - Doshi NP FAU - Merla, Arcangelo AU - Merla A LA - eng PT - Evaluation Study PT - Journal Article PL - United States TA - Annu Int Conf IEEE Eng Med Biol Soc JT - Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference JID - 101763872 SB - IM MH - Capillaries/*pathology MH - Case-Control Studies MH - Connective Tissue Diseases/*pathology MH - Diagnosis, Computer-Assisted/methods MH - Humans MH - Image Processing, Computer-Assisted/methods MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Pattern Recognition, Automated/*methods MH - Raynaud Disease/pathology EDAT- 2013/10/11 06:00 MHDA- 2015/08/19 06:00 CRDT- 2013/10/11 06:00 PHST- 2013/10/11 06:00 [entrez] PHST- 2013/10/11 06:00 [pubmed] PHST- 2015/08/19 06:00 [medline] AID - 10.1109/EMBC.2013.6610788 [doi] PST - ppublish SO - Annu Int Conf IEEE Eng Med Biol Soc. 2013;2013:5473-6. doi: 10.1109/EMBC.2013.6610788. PMID- 21291581 OWN - NLM STAT- MEDLINE DCOM- 20110829 LR - 20181201 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 155 DP - 2011 TI - [A complication of coronary angiography in a female patient with systemic sclerosis]. PG - A2704 AB - Systemic sclerosis is an autoimmune connective tissue disorder characterized by microvascular obliterations of the skin, lungs, and heart. Pulmonary hypertension is a potentially life-threatening complication of systemic sclerosis and coronary angiography is indicated for diagnosing this complication. A 79-year-old woman, who suffered from systemic sclerosis and Raynaud's syndrome, presented with a cold, painful, ulcerated right hand. It appeared that arterial occlusion of the radial artery had occurred following coronary angiography. Symptoms initially worsened, but improved following treatment with bosentan. This complication could have been avoided by performing the coronary angiography via the femoral artery. This case study emphasises the importance of taking medical history and comorbidities into account when carrying out invasive diagnostic procedures. FAU - Dogan, Kemal AU - Dogan K AD - Gelre Ziekenhuizen, locatie Lukas, afdeling Heelkunde, Apeldoorn, The Netherlands. dogan_kemal@hotmail.com FAU - Bakx, Roel AU - Bakx R FAU - Klemm, Peter L AU - Klemm PL LA - dut PT - Case Reports PT - Journal Article TT - Complicatie van coronairangiografie bij een patiënte met sclerodermie. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Aged MH - Antihypertensive Agents/therapeutic use MH - Arterial Occlusive Diseases/drug therapy/*etiology MH - Bosentan MH - Coronary Angiography/*adverse effects MH - Female MH - Humans MH - Raynaud Disease/*complications MH - Scleroderma, Systemic/*complications MH - Sulfonamides/therapeutic use EDAT- 2011/02/05 06:00 MHDA- 2011/08/30 06:00 CRDT- 2011/02/05 06:00 PHST- 2011/02/05 06:00 [entrez] PHST- 2011/02/05 06:00 [pubmed] PHST- 2011/08/30 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2011;155:A2704. PMID- 41296301 OWN - NLM STAT- MEDLINE DCOM- 20251126 LR - 20251215 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 73 IP - 11S DP - 2025 Nov TI - Erasmus Syndrome: A Case Report and Review of Cases Published from India. PG - 36-39 LID - 10.59556/japi.73.1231 [doi] AB - Erasmus syndrome is a rare clinical syndrome characterized by the development of systemic sclerosis (SSc) following chronic exposure to silica; however, the presence of silicosis is not a prerequisite for this diagnosis. It is infrequently reported in the literature, and recognizing the association between occupational silica exposure and SSc is crucial for timely diagnosis and management in workplace settings. We report a case of Erasmus syndrome in a stone cutter in his late 30s, who presented with gangrene in both feet, Raynaud's phenomenon, without any evidence of other organ involvement. Anti-Scl-70 antibody was positive on the line immunoassay, supporting the diagnosis. The patient was started on a combination therapy including calcium channel blockers, phosphodiesterase 5 inhibitors, and wound care. At 6-month follow-up, he had no further disease progression with stabilization of gangrene. CI - © Journal of The Association of Physicians of India 2025. FAU - Alria, Ankit AU - Alria A AD - Physician, Department of Medicine, Sawai Man Singh Medical College, Jaipur, Rajasthan, India, Orcid: https://orcid.org/0009-0003-6975-3422. FAU - Singh, Aradhana AU - Singh A AD - Professor, Department of Clinical Immunology & Rheumatology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India. FAU - Maheshwari, Rashi AU - Maheshwari R AD - Assistant Professor, Department of Clinical Immunology & Rheumatology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India. FAU - Jain, Avinash AU - Jain A AD - Assistant Professor, Department of Clinical Immunology & Rheumatology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India, Orcid: https://orcid.org/0000-0003-3207-4509, Corresponding Author. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Humans MH - Male MH - Adult MH - India MH - *Scleroderma, Systemic/diagnosis/etiology MH - *Silicosis/complications/diagnosis MH - Syndrome MH - Gangrene/etiology MH - Raynaud Disease/etiology MH - *Occupational Exposure/adverse effects EDAT- 2025/11/26 13:12 MHDA- 2025/11/26 13:13 CRDT- 2025/11/26 11:23 PHST- 2025/11/26 13:13 [medline] PHST- 2025/11/26 13:12 [pubmed] PHST- 2025/11/26 11:23 [entrez] AID - 10.59556/japi.73.1231 [doi] PST - ppublish SO - J Assoc Physicians India. 2025 Nov;73(11S):36-39. doi: 10.59556/japi.73.1231. PMID- 39160005 OWN - NLM STAT- MEDLINE DCOM- 20240819 LR - 20260304 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 20 IP - 7 DP - 2024 Aug-Sep TI - Development of a core domain set for nailfold capillaroscopy reporting. PG - 345-352 LID - S2173-5743(24)00095-9 [pii] LID - 10.1016/j.reumae.2024.07.003 [doi] AB - BACKGROUND: The peripheral microangiopathy may be well evaluated and studied by nailfold capillaroscopy (NFC) which is a safe and non-invasive technique. NFC has been reported to have both diagnostic and prognostic values in patients presenting with Raynaud's phenomenon. OBJECTIVE: The overarching objective of this work was to make a consensus on what domains should be included in a capillaroscopy report and that it can be used in daily clinical practice and clinical research in the area of rheumatology. METHODS: A Delphi questionnaire was developed regarding capillaroscopy report from a literature review and expert consensus. The first Delphi round included 14 core areas, its 18 domains with 50 subdomains, derived from a systematic literature review. The level of evidence was determined for each core set using the Oxford Centre for Evidence-based Medicine (CEBM) system. Nine response categories have been set per each item ranging between 1 and 9. Round 2, aimed to reach preliminary consensus "in" or "out" for domains. It included all items that were rated "critical" by at least 80% of the participants as well as any new domains proposed in round 1. RESULTS: The participants to the first, and second round were 11 experts. Fourteen domains were discussed in the two rounds. At the end of the survey, the final report template of NFC in rheumatology reached a consensus. CONCLUSION: A nailfold capillaroscopy report template has been developed by this study, based on outcomes of a Delphi process, by international participants panel. All domains met the 80% voting threshold set in this work. The reporting template can be used for both clinical research as well as day to day practice to provide guidance and standardize the NFC reporting. CI - Copyright © 2024. Published by Elsevier España, S.L.U. FAU - El Miedany, Yasser AU - El Miedany Y AD - Canterbury Christ Church University, England, UK. FAU - Ismail, Sherif AU - Ismail S AD - National Research Center, Internal Medicine, Rheumatology and Rehabilitation, Egypt. FAU - Wadie, Mary AU - Wadie M AD - Cairo University, Internal Medicine, Egypt. FAU - Müller-Ladneru, Ulf AU - Müller-Ladneru U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University of Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Istituto di Clinica Medica, Università, L'Aquila Italy. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Unit of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila Italy. FAU - Hermann, Walter AU - Hermann W AD - Abteilung für Rheumatologie und Klinische Immunologie, Kerckhoff-Klinik GmbH, Benekestr. 2-8, 61231 Bad Nauheim, Germany. FAU - Fathy, Nihal AU - Fathy N AD - Rheumatology and Rehabilitation, Assiut University, Assiut, Egypt. FAU - El Gaafary, Maha AU - El Gaafary M AD - Community and Public Health, Ain Shams University, Cairo, Egypt. FAU - Fouad, Nermin A AU - Fouad NA AD - Rheumatology and Rehabilitation, Fayoum University, Fayoum, Egypt. FAU - Saber, Sally AU - Saber S AD - Rheumatology and Rehabilitation, Ain Shams University, Cairo, Egypt. FAU - Abu-Zaid, Mohamed Hassan AU - Abu-Zaid MH AD - Rheumatology and Rehabilitation, Tanta University, Tanta, Egypt. Electronic address: drmhassan113@yahoo.com. LA - eng PT - Consensus Statement PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - Delphi Technique MH - *Microscopic Angioscopy/standards MH - *Nails/blood supply/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging OTO - NOTNLM OT - Capilaroscopia ungueal OT - Connective tissue disease OT - Consenso OT - Consensus OT - Enfermedad del tejido conectivo OT - Estándar de informes OT - Fenómeno de Raynaud OT - Nailfold capillaroscopy OT - Plantilla OT - Raynaud's phenomenon OT - Reporting standard OT - Template EDAT- 2024/08/20 00:42 MHDA- 2024/08/20 00:43 CRDT- 2024/08/19 20:57 PHST- 2023/12/13 00:00 [received] PHST- 2024/04/04 00:00 [accepted] PHST- 2024/08/20 00:43 [medline] PHST- 2024/08/20 00:42 [pubmed] PHST- 2024/08/19 20:57 [entrez] AID - S2173-5743(24)00095-9 [pii] AID - 10.1016/j.reumae.2024.07.003 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2024 Aug-Sep;20(7):345-352. doi: 10.1016/j.reumae.2024.07.003. PMID- 23073231 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - Alteration of microcirculation is a hallmark of very early systemic sclerosis patients: a laser speckle contrast analysis. PG - 109-14 AB - OBJECTIVES: To investigate blood flow and microvascular reactivity by laser speckle perfusion imager (Perimed, Jarfalla) in consecutive patients affected by Raynaud's phenomenon at baseline and following dynamic stimulations. METHODS: Skin blood flow in the dorsum of the hand was measured at baseline and after cold test and post-occlusive hyperemia test in 56 consecutive subjects affected by Raynaud's phenomenon (RP), 20 primary (PRP) and 36 secondary to systemic sclerosis (SSc). Twenty healthy subjects (HS) were studied as controls. RESULTS: After cold test, SSc had a significant reduction of blood flow (-58%) as compared to HS (-19%) (p=0.01). Recovery time was significantly higher in SSc (58 minutes) as compared to HS (18 minutes) and PRP (19 minutes) (p=0.006 and 0.0016, respectively). Peak flow after ischaemic test was significantly reduced in SSc (+237%) as compared to PRP (+485%) (p=0.0068). Post-ischaemic hyperemic area under the curve (AUC) was blunted in SSc (79U/sec) compared to PRP (167 U/sec) (p=0.0126). Proximal distal gradient was noticed in 74% of HS, 45% of PRP and 36% of SSc (p=0.01). Homogeneous pattern of flux distribution was significantly different between HS (95%), PRP (80%), and SSc (16%) (p<0.0001). Among SSc patients, a significant difference in ischaemic challenge was shown between patients with early-SSc versus patients with definite-SSc. CONCLUSIONS: Our preliminary results indicate a clearcut alteration of the dynamic of microcirculation in SSc-RP as compared to PRP and HS. Among SSc patients, early-SSc is a separate entity as compared to established disease. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Department of Musculoskeletal and Skin Diseases, Rheumatology Unit, University of Pisa, Italy. a.dellarossa@ao-pisa.toscana.it FAU - Cazzato, Massimiliano AU - Cazzato M FAU - d'Ascanio, Anna AU - d'Ascanio A FAU - Tavoni, Antonio AU - Tavoni A FAU - Bencivelli, Walter AU - Bencivelli W FAU - Pepe, Pasquale AU - Pepe P FAU - Mosca, Marta AU - Mosca M FAU - Baldini, Chiara AU - Baldini C FAU - Rossi, Marco AU - Rossi M FAU - Bombardieri, Stefano AU - Bombardieri S LA - eng PT - Journal Article DEP - 20121022 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Cold Temperature MH - Diagnosis, Differential MH - Early Diagnosis MH - Female MH - Humans MH - Lasers MH - Male MH - Microcirculation/*physiology MH - Microscopic Angioscopy/instrumentation/*methods MH - Middle Aged MH - Raynaud Disease/*diagnosis/physiopathology MH - Scleroderma, Systemic/*diagnosis/physiopathology MH - Skin/blood supply MH - Young Adult EDAT- 2012/10/18 06:00 MHDA- 2013/09/11 06:00 CRDT- 2012/10/18 06:00 PHST- 2012/09/04 00:00 [received] PHST- 2012/10/09 00:00 [accepted] PHST- 2012/10/18 06:00 [entrez] PHST- 2012/10/18 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 6441 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):109-14. Epub 2012 Oct 22. PMID- 26357658 OWN - NLM STAT- MEDLINE DCOM- 20160609 LR - 20181113 IS - 2314-6141 (Electronic) IS - 2314-6133 (Print) VI - 2015 DP - 2015 TI - The Application of Fluorescence Optical Imaging in Systemic Sclerosis. PG - 658710 LID - 10.1155/2015/658710 [doi] LID - 658710 AB - OBJECTIVE: The aim of this study was to visualize soft tissue inflammation using FOI on patients with Systemic Sclerosis (SSc) characterized by SSc-related Raynaud's phenomenon and to detect the therapeutic response to treatment with iloprost or alprostadil. METHODS: Twenty-one patients with SSc and Raynaud's phenomenon and twenty-six healthy controls were prospectively included. The SSc patients were intravenously treated with iloprost or alprostadil over seven days. FOI was performed at baseline and after seven days using an intravenous application of indocyanine green (ICG). The hands were divided into nineteen segments per hand. All segments were quantitatively evaluated to determine changes in ICG. RESULTS: The sensitivity and specificity of FOI in the detection of ICG enhancement in patients with SSc were 95% versus 96%. At baseline, 31.5% hand segments showed ICG enhancement. After seven days of either iloprost or alprostadil therapy a significant reduction in the ICG was observed which ranged from 40.9% to 24.7%. CONCLUSION: The study demonstrates that the FOI technique is able to visualize soft-tissue inflammation with both high sensitivity and specificity. The anti-inflammatory therapeutic effects of iloprost were slightly stronger than alprostadil. FOI offers promising benefits in the diagnosis and therapy of patients with SSc-associated Raynaud's phenomenon. FAU - Pfeil, Alexander AU - Pfeil A AD - Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. FAU - Drummer, Karl F AU - Drummer KF AD - Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. FAU - Böttcher, Joachim AU - Böttcher J AD - Institute of Diagnostic and Interventional Radiology, SRH Wald-Klinikum Gera, Straße des Friedens 122, 07548 Gera, Germany. FAU - Jung, Christian AU - Jung C AD - Department of Internal Medicine I, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. FAU - Oelzner, Peter AU - Oelzner P AD - Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. FAU - Renz, Diane M AU - Renz DM AD - Department of Radiology, Charité-University Medicine Berlin, Charitéplatz 1, 10117 Berlin, Germany. FAU - Franz, Marcus AU - Franz M AD - Department of Internal Medicine I, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. FAU - Hansch, Andreas AU - Hansch A AD - Institute of Diagnostic and Interventional Radiology, Heinrich Braun Clinic Zwickau, Karl-Keil-Straße 35, 08060 Zwickau, Germany. FAU - Wolf, Gunter AU - Wolf G AD - Department of Internal Medicine III, Jena University Hospital, Friedrich Schiller University Jena, Erlanger Allee 101, 07747 Jena, Germany. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150818 PL - United States TA - Biomed Res Int JT - BioMed research international JID - 101600173 RN - 0 (Vasodilator Agents) RN - F5TD010360 (Alprostadil) RN - IX6J1063HV (Indocyanine Green) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Alprostadil/therapeutic use MH - Case-Control Studies MH - Female MH - Fluorescence MH - Humans MH - Iloprost/therapeutic use MH - Indocyanine Green/administration & dosage MH - Inflammation/drug therapy/pathology MH - Male MH - Optical Imaging/methods MH - Prospective Studies MH - Raynaud Disease/drug therapy/pathology MH - Scleroderma, Systemic/drug therapy/*pathology MH - Sensitivity and Specificity MH - Vasodilator Agents/therapeutic use MH - Young Adult PMC - PMC4555360 EDAT- 2015/09/12 06:00 MHDA- 2016/06/10 06:00 PMCR- 2015/08/18 CRDT- 2015/09/11 06:00 PHST- 2015/02/06 00:00 [received] PHST- 2015/04/03 00:00 [accepted] PHST- 2015/09/11 06:00 [entrez] PHST- 2015/09/12 06:00 [pubmed] PHST- 2016/06/10 06:00 [medline] PHST- 2015/08/18 00:00 [pmc-release] AID - 10.1155/2015/658710 [doi] PST - ppublish SO - Biomed Res Int. 2015;2015:658710. doi: 10.1155/2015/658710. Epub 2015 Aug 18. PMID- 22370242 OWN - NLM STAT- MEDLINE DCOM- 20120711 LR - 20220409 IS - 1361-6579 (Electronic) IS - 0967-3334 (Linking) VI - 33 IP - 3 DP - 2012 Mar TI - Infrared thermal imaging in medicine. PG - R33-46 LID - 10.1088/0967-3334/33/3/R33 [doi] AB - This review describes the features of modern infrared imaging technology and the standardization protocols for thermal imaging in medicine. The technique essentially uses naturally emitted infrared radiation from the skin surface. Recent studies have investigated the influence of equipment and the methods of image recording. The credibility and acceptance of thermal imaging in medicine is subject to critical use of the technology and proper understanding of thermal physiology. Finally, we review established and evolving medical applications for thermal imaging, including inflammatory diseases, complex regional pain syndrome and Raynaud's phenomenon. Recent interest in the potential applications for fever screening is described, and some other areas of medicine where some research papers have included thermal imaging as an assessment modality. In certain applications thermal imaging is shown to provide objective measurement of temperature changes that are clinically significant. FAU - Ring, E F J AU - Ring EF AD - Medical Imaging Research Unit, University of Glamorgan, Pontypridd, CF37 1DL, UK. efring@glam.ac.uk FAU - Ammer, K AU - Ammer K LA - eng PT - Journal Article PT - Review DEP - 20120228 PL - England TA - Physiol Meas JT - Physiological measurement JID - 9306921 SB - IM MH - Arthritis/diagnosis MH - Complex Regional Pain Syndromes/diagnosis MH - Diagnostic Imaging/instrumentation/methods/standards MH - Fever/diagnosis MH - Humans MH - Inflammation/diagnosis MH - Infrared Rays MH - Medicine/*instrumentation MH - Raynaud Disease/diagnosis MH - Skin Temperature MH - Thermography/instrumentation/*methods/standards EDAT- 2012/03/01 06:00 MHDA- 2012/07/12 06:00 CRDT- 2012/02/29 06:00 PHST- 2012/02/29 06:00 [entrez] PHST- 2012/03/01 06:00 [pubmed] PHST- 2012/07/12 06:00 [medline] AID - 10.1088/0967-3334/33/3/R33 [doi] PST - ppublish SO - Physiol Meas. 2012 Mar;33(3):R33-46. doi: 10.1088/0967-3334/33/3/R33. Epub 2012 Feb 28. PMID- 30101908 OWN - NLM STAT- MEDLINE DCOM- 20190222 LR - 20240416 IS - 1349-7235 (Electronic) IS - 0918-2918 (Print) IS - 0918-2918 (Linking) VI - 57 IP - 23 DP - 2018 Dec 1 TI - Anticentromere Antibody-positive Scleroderma Renal Crisis Requiring Dialysis. PG - 3479-3483 LID - 10.2169/internalmedicine.0980-18 [doi] AB - A 70-year-old man with prior Raynaud's phenomena developed hypertension and renal insufficiency. Raynaud's phenomena, finger skin thickening, interstitial lung disease, and positive anticentromere antibody findings indicated systemic sclerosis (SSc). Based on the presence of SSc, severe hypertension with rapidly progressive renal failure, and proliferative and obliterative arteriolar vasculopathy, scleroderma renal crisis (SRC) was diagnosed. Despite good blood pressure control with antihypertensive drugs, hemodialysis was initiated and could not be withdrawn owing to unimproved renal dysfunction. Although SRC in anticentromere antibody-positive limited cutaneous SSc is extremely rare, some patients may develop SRC, and their renal prognosis may be poor. FAU - Ubukata, Masamitsu AU - Ubukata M AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. AD - Department IV, Internal Medicine, Tokyo Women's Medical University, Japan. FAU - Mitsuhashi, Atsushi AU - Mitsuhashi A AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. FAU - Nishizawa, Yuki AU - Nishizawa Y AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. FAU - Fujii, Teruhiro AU - Fujii T AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. AD - Department IV, Internal Medicine, Tokyo Women's Medical University, Japan. FAU - Hara, Masaki AU - Hara M AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. AD - Department IV, Internal Medicine, Tokyo Women's Medical University, Japan. FAU - Ohta, Akihito AU - Ohta A AD - Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan. FAU - Nitta, Kosaku AU - Nitta K AD - Department IV, Internal Medicine, Tokyo Women's Medical University, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20180810 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antihypertensive Agents) RN - 0 (anticentromere antibody) SB - IM MH - Acute Kidney Injury/*etiology/*therapy MH - Aged MH - Antibodies, Antinuclear/*blood MH - Antihypertensive Agents/therapeutic use MH - Humans MH - Hypertension, Malignant/drug therapy/etiology MH - Lung Diseases, Interstitial/etiology MH - Male MH - Prognosis MH - Raynaud Disease/complications MH - *Renal Dialysis MH - Scleroderma, Localized/*complications/diagnosis/*immunology PMC - PMC6306537 OTO - NOTNLM OT - anticentromere antibody OT - scleroderma renal crisis OT - systemic sclerosis EDAT- 2018/08/14 06:00 MHDA- 2019/02/23 06:00 PMCR- 2018/12/01 CRDT- 2018/08/14 06:00 PHST- 2018/08/14 06:00 [pubmed] PHST- 2019/02/23 06:00 [medline] PHST- 2018/08/14 06:00 [entrez] PHST- 2018/12/01 00:00 [pmc-release] AID - 10.2169/internalmedicine.0980-18 [doi] PST - ppublish SO - Intern Med. 2018 Dec 1;57(23):3479-3483. doi: 10.2169/internalmedicine.0980-18. Epub 2018 Aug 10. PMID- 24990822 OWN - NLM STAT- MEDLINE DCOM- 20150421 LR - 20220330 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 94 DP - 2014 Jul TI - Abnormal amplitude and kinetics of digital postocclusive reactive hyperemia in systemic sclerosis. PG - 90-5 LID - S0026-2862(14)00084-3 [pii] LID - 10.1016/j.mvr.2014.05.007 [doi] AB - OBJECTIVES: Postocclusive reactive hyperemia is mediated by two major mediators: sensory nerves and endothelium-derived hyperpolarizing factors. We hypothesized that the skin microvascular response to 5 min ischemia would differ depending upon the hand location in patients with systemic sclerosis (SSc), primary Raynaud's phenomenon (PRP) and healthy controls. METHODS: Fifteen patients with SSc, 15 sex- and age-matched patients with PRP and healthy controls were enrolled. Their right hands were subjected to 5 min ischemia followed by a postocclusive hyperemia test, with local microcirculation monitoring by laser speckle contrast imaging on the dorsal face of the hand. RESULTS: Postocclusive reactive hyperemia was abnormal in terms of peak and area under the curve (AUC) on all fingers except the thumb in patients with SSc and PRP compared with controls. In contrast, the kinetics of the response was longer only in SSc patients, with mean (SD) time to peak on the index, middle and ring finger were respectively 72 (58), 73 (51) and 67 (47) s for SSc; 40 (20), 40 (20) and 36 (19) s for PRP; and 34 (30), 34 (30) and 29 (24) s for controls (P=0.009 for interaction). CONCLUSIONS: We observed decreased distal digital microvascular perfusion following 5 min of ischemia in patients presenting with PRP or SSc, while the kinetics was prolonged only in SSc. A dynamic assessment of digital skin blood flow using laser speckle contrast imaging following 5 min ischemia could be used as a tool to assess microvascular abnormalities in patients with Raynaud's phenomenon secondary to SSc. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Gaillard-Bigot, F AU - Gaillard-Bigot F AD - Univ. Grenoble Alpes, HP2, 38000, France; Clinical Pharmacology Unit, Inserm CIC003, Grenoble University Hospital, 38043, France. FAU - Roustit, M AU - Roustit M AD - Univ. Grenoble Alpes, HP2, 38000, France; Clinical Pharmacology Unit, Inserm CIC003, Grenoble University Hospital, 38043, France. FAU - Blaise, S AU - Blaise S AD - Univ. Grenoble Alpes, HP2, 38000, France; Vascular Medicine Department, Grenoble University Hospital, 38043, France. FAU - Gabin, M AU - Gabin M AD - Univ. Grenoble Alpes, HP2, 38000, France. FAU - Cracowski, C AU - Cracowski C AD - Univ. Grenoble Alpes, HP2, 38000, France; Clinical Pharmacology Unit, Inserm CIC003, Grenoble University Hospital, 38043, France. FAU - Seinturier, C AU - Seinturier C AD - Vascular Medicine Department, Grenoble University Hospital, 38043, France. FAU - Imbert, B AU - Imbert B AD - Vascular Medicine Department, Grenoble University Hospital, 38043, France. FAU - Carpentier, P AU - Carpentier P AD - Vascular Medicine Department, Grenoble University Hospital, 38043, France. FAU - Cracowski, J L AU - Cracowski JL AD - Univ. Grenoble Alpes, HP2, 38000, France; Clinical Pharmacology Unit, Inserm CIC003, Grenoble University Hospital, 38043, France. Electronic address: Jean-Luc.Cracowski@ujf-grenoble.fr. LA - eng SI - ClinicalTrials.gov/NCT01743612 PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140602 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Aged MH - Area Under Curve MH - Blood Pressure MH - Case-Control Studies MH - Endothelium, Vascular/*pathology MH - Female MH - Fingers/blood supply MH - Hand/blood supply MH - Humans MH - Hyperemia/*physiopathology MH - Ischemia MH - Kinetics MH - Laser-Doppler Flowmetry MH - Microcirculation/physiology MH - Middle Aged MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Research Design MH - Scleroderma, Systemic/*pathology/*physiopathology MH - Skin/blood supply MH - Time Factors OTO - NOTNLM OT - Endothelium OT - Laser speckle contrast imaging OT - Microcirculation OT - Microcirculation imaging OT - Post-occlusive reactive hyperemia OT - Primary Raynaud's Phenomenon OT - Raynaud's phenomenon OT - Skin OT - Systemic sclerosis OT - Vasodilatation EDAT- 2014/07/06 06:00 MHDA- 2015/04/22 06:00 CRDT- 2014/07/04 06:00 PHST- 2014/03/20 00:00 [received] PHST- 2014/05/23 00:00 [revised] PHST- 2014/05/26 00:00 [accepted] PHST- 2014/07/04 06:00 [entrez] PHST- 2014/07/06 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] AID - S0026-2862(14)00084-3 [pii] AID - 10.1016/j.mvr.2014.05.007 [doi] PST - ppublish SO - Microvasc Res. 2014 Jul;94:90-5. doi: 10.1016/j.mvr.2014.05.007. Epub 2014 Jun 2. PMID- 18597404 OWN - NLM STAT- MEDLINE DCOM- 20090114 LR - 20211020 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 8 DP - 2008 Aug TI - Discrepancy between simultaneous digital skin microvascular and brachial artery macrovascular post-occlusive hyperemia in systemic sclerosis. PG - 1576-83 AB - OBJECTIVE: Vascular impairment, a main feature of the pathogenesis of systemic sclerosis (SSc), involves both the macro- and the microvasculature. We compared and correlated simultaneously measured skin microvascular and brachial artery macrovascular post-occlusive hyperemia in 3 groups: patients with SSc, patients with primary Raynaud's phenomenon (RP), and healthy volunteers. METHODS: Thirty-three healthy volunteers, 36 patients with primary RP, and 42 patients with SSc were enrolled. For each subject, brachial artery flow-mediated dilation (FMD) and cutaneous post-occlusive reactive hyperemia (PORH) were simultaneously recorded after 5-minute occlusion of the brachial artery. Local thermal hyperemia, nitroglycerin-mediated dilation (NMD), intima-media thickness (IMT), and pulse wave velocity (PWV) were also assessed. RESULTS: Digital cutaneous peak PORH was altered in patients with primary RP and SSc compared to healthy controls, whereas FMD was not significantly different among all groups. We observed a correlation between digital peak cutaneous vascular conductance and brachial FMD in healthy controls (r = 0.49; p = 0.004), but not in patients with primary RP or SSc. Thermal hyperemia was altered only in patients with SSc. Brachial NMD, IMT, and PWV were not different among all groups. CONCLUSION: We observed a loss of the correlation between brachial FMD and digital cutaneous PORH in patients with SSc and primary RP. Microvascular function is impaired in SSc, whereas brachial artery endothelial function is normal. FAU - Roustit, Matthieu AU - Roustit M AD - Clinical Research Center, INSERM CIC03, Grenoble University Hospital, Grenoble, France. FAU - Simmons, Grant H AU - Simmons GH FAU - Baguet, Jean-Philippe AU - Baguet JP FAU - Carpentier, Patrick AU - Carpentier P FAU - Cracowski, Jean-Luc AU - Cracowski JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Brachial Artery/*physiology MH - Case-Control Studies MH - Female MH - Humans MH - Hyperemia/complications/*diagnosis/*physiopathology MH - Male MH - Microvessels/*physiopathology MH - Middle Aged MH - Raynaud Disease/complications/physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/complications/*physiopathology MH - Skin/*blood supply PMC - PMC3291814 MID - HALMS361994 OID - NLM: HALMS361994 EDAT- 2008/07/04 09:00 MHDA- 2009/01/15 09:00 PMCR- 2012/03/02 CRDT- 2008/07/04 09:00 PHST- 2008/07/04 09:00 [pubmed] PHST- 2009/01/15 09:00 [medline] PHST- 2008/07/04 09:00 [entrez] PHST- 2012/03/02 00:00 [pmc-release] AID - 08/13/0622 [pii] PST - ppublish SO - J Rheumatol. 2008 Aug;35(8):1576-83. Epub 2008 Jun 15. PMID- 18078620 OWN - NLM STAT- MEDLINE DCOM- 20080410 LR - 20191210 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 25 IP - 5 DP - 2007 Sep-Oct TI - Disease-modifying effects of long-term cyclic iloprost therapy in systemic sclerosis. A retrospective analysis and comparison with a control group. PG - 722-7 AB - OBJECTIVE: To evaluate the role of iloprost, a derivative of prostacyclin, as a possible disease-modifying agent for systemic sclerosis (SSc). METHODS: Fifty-six consecutive SSc patients treated for a median period of 4 years with cyclic infusions of iloprost for severe Raynaud's phenomenon and ischemic ulcers were compared with 56 control patients matched for age, sex, disease subset and duration. Control patients were also similar to the iloprost group with regard to autoantibody status, the presence of major disease-related organ manifestations at baseline, and the use of other treatments. The evolution of lung function test results, the frequency of major disease-specific complications and the survival of the cohorts were the objects of this analysis. RESULTS: No significant difference was observed between the two groups with regard to changes in lung function tests over time, or the number of patients who presented with the onset of active interstitial lung disease, pulmonary arterial hypertension or scleroderma renal crisis. Survival did not differ between the two groups. CONCLUSION: The evolution of lung function test results, the frequency of major disease-specific complications, and survival did not differ significantly between SSc patients treated with cyclic iloprost and a group of patients matched for sex, age, and disease subset and duration. However, no cases of severe pulmonary arterial hypertension were observed in the patients treated with iloprost, suggesting that studies focusing on the possible preventive action of iloprost on the progression of SSc- associated mild pulmonary arterial hypertension would be warranted. FAU - Airò, P AU - Airò P AD - Rheumatology and Clinical Immunology Clinic, Spedali Civili, Brescia, Italy. airo@bresciareumatologia.it FAU - Rossi, M AU - Rossi M FAU - Scarsi, M AU - Scarsi M FAU - Danieli, E AU - Danieli E FAU - Grottolo, A AU - Grottolo A FAU - Zambruni, A AU - Zambruni A LA - eng PT - Comparative Study PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antirheumatic Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Antirheumatic Agents/*therapeutic use MH - Dose-Response Relationship, Drug MH - Female MH - Humans MH - Hypertension, Pulmonary/physiopathology/prevention & control MH - Iloprost/*therapeutic use MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Outcome Assessment, Health Care MH - Raynaud Disease/drug therapy/physiopathology MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/*drug therapy/physiopathology MH - Survival Rate MH - Ulcer/drug therapy/physiopathology EDAT- 2007/12/15 09:00 MHDA- 2008/04/11 09:00 CRDT- 2007/12/15 09:00 PHST- 2007/12/15 09:00 [pubmed] PHST- 2008/04/11 09:00 [medline] PHST- 2007/12/15 09:00 [entrez] AID - 2183 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2007 Sep-Oct;25(5):722-7. PMID- 25790900 OWN - NLM STAT- MEDLINE DCOM- 20160303 LR - 20150515 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 40 IP - 3 DP - 2015 May TI - [Unilateral isolated Raynaud's phenomenon leading to diagnosis of multifocal giant-cell arteritis complicated with renal ischemia]. PG - 200-5 LID - S0398-0499(15)00077-3 [pii] LID - 10.1016/j.jmv.2015.02.003 [doi] AB - We report a case of a 76-year-old woman with isolated unilateral Raynaud phenomenon revealing giant-cell arteritis with diffuse arterial lesions and bilateral renal artery stenosis. Doppler ultrasonography showed bilateral stenosis of the subclavian and axillary arteries. Angio-CT PET enlightened diffuse arterial lesions, mainly involving the aorta and the brachial and femoral arteries as well as bilateral renal ostial stenosis with right kidney ischemia. Diagnosis of giant-cell arteritis was made on the temporal artery biopsy. Corticosteroid therapy led to rapid clinical and radiological improvement. Clinical manifestations of giant-cell arteritis may be atypical. Diffuse arterial disease may exist in the absence of cephalic symptoms or significant inflammatory biological features. Ostial renal artery stenosis may induce potentially threatening renal ischemia. CI - Copyright © 2015 Elsevier Masson SAS. All rights reserved. FAU - Goupil de Bouillé, J AU - Goupil de Bouillé J AD - Service de médecine interne, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Lecouffe-Desprets, M AU - Lecouffe-Desprets M AD - Service de médecine interne, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Bigot, A AU - Bigot A AD - Service de médecine interne, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Halimi, J-M AU - Halimi JM AD - EA-4245, service de néphrologie-immunologie clinique, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Courtehoux, M AU - Courtehoux M AD - Service de médecine nucléaire, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Guilmot, J-L AU - Guilmot JL AD - Service de médecine interne, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. FAU - Breteau, C AU - Breteau C AD - Cabinet d'angiologie, 17, place de la Tranchée, 37100 Tours, France. FAU - Diot, E AU - Diot E AD - Service de médecine interne, CHU de Tours, université François-Rabelais, 2, boulevard Tonnellé, 37044 Tours cedex 9, France. Electronic address: ediot@med.univ-tours.fr. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Phénomène de Raynaud unilatéral isolé révélateur d'une artérite à cellules géantes multifocale (maladie de Horton) compliquée d'ischémie rénale. DEP - 20150316 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Aged MH - Female MH - Giant Cell Arteritis/*complications/*diagnosis MH - Humans MH - Ischemia/*complications MH - Kidney/*blood supply MH - Raynaud Disease/*etiology OTO - NOTNLM OT - Aortite OT - Aortitis OT - Artérite à cellules géantes OT - Giant-cell arteritis OT - Phénomène de Raynaud OT - Raynaud phenomenon OT - Renal artery stenosis OT - Sténose des artères rénales EDAT- 2015/03/21 06:00 MHDA- 2016/03/05 06:00 CRDT- 2015/03/21 06:00 PHST- 2014/10/01 00:00 [received] PHST- 2015/01/09 00:00 [accepted] PHST- 2015/03/21 06:00 [entrez] PHST- 2015/03/21 06:00 [pubmed] PHST- 2016/03/05 06:00 [medline] AID - S0398-0499(15)00077-3 [pii] AID - 10.1016/j.jmv.2015.02.003 [doi] PST - ppublish SO - J Mal Vasc. 2015 May;40(3):200-5. doi: 10.1016/j.jmv.2015.02.003. Epub 2015 Mar 16. PMID- 17133611 OWN - NLM STAT- MEDLINE DCOM- 20070206 LR - 20220408 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 54 IP - 12 DP - 2006 Dec TI - Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. PG - 3971-8 AB - OBJECTIVE: To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group of children and describe the clinical evolution of the disease and compare it with the adult form. METHODS: Data on 153 patients with juvenile SSc collected from 55 pediatric rheumatology centers in Europe, Asia, and South and North America were analyzed. Demographic, clinical, and immunologic characteristics of children with juvenile SSc at the onset, at diagnosis, and during the disease course were evaluated. RESULTS: Raynaud's phenomenon was the most frequent symptom, followed by skin induration in approximately 75% of patients. Musculoskeletal symptoms were present in one-third of patients, and the most frequently involved internal organs were respiratory and gastrointestinal, while involvement of renal, cerebral, and cardiovascular systems was extremely rare. Antinuclear antibodies were present in the sera of 81% of patients. Anti-topoisomerase I (Scl-70) and anticentromere antibodies were found to be positive in 34% and 7.1% of patients, respectively. Involvement of the respiratory, gastrointestinal, and cardiovascular systems was more frequent and occurred earlier in patients who died than in those who survived. Compared with the adult form, juvenile SSc appears to be less severe, with the involvement of fewer internal organs, particularly at the time of diagnosis, and has a less characterized immunologic profile. CONCLUSION: This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome. FAU - Martini, Giorgia AU - Martini G AD - University of Padua, Padua, Italy. FAU - Foeldvari, Ivan AU - Foeldvari I FAU - Russo, Ricardo AU - Russo R FAU - Cuttica, Ruben AU - Cuttica R FAU - Eberhard, Anne AU - Eberhard A FAU - Ravelli, Angelo AU - Ravelli A FAU - Lehman, Thomas J A AU - Lehman TJ FAU - de Oliveira, Sheila Knupp Feitosa AU - de Oliveira SK FAU - Susic, Gordana AU - Susic G FAU - Lyskina, Galina AU - Lyskina G FAU - Nemcova, Dana AU - Nemcova D FAU - Sundel, Robert AU - Sundel R FAU - Falcini, Fernanda AU - Falcini F FAU - Girschick, Herman AU - Girschick H FAU - Lotito, Ana Paula AU - Lotito AP FAU - Buoncompagni, Antonella AU - Buoncompagni A FAU - Sztajnbok, Flavio AU - Sztajnbok F FAU - Al-Mayouf, Sulaiman M AU - Al-Mayouf SM FAU - Orbàn, Ilonka AU - Orbàn I FAU - Ferri, Clodoveo AU - Ferri C FAU - Athreya, Balu H AU - Athreya BH FAU - Woo, Patricia AU - Woo P FAU - Zulian, Francesco AU - Zulian F CN - Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Autoantibodies/blood MH - Autoimmune Diseases/etiology/immunology/physiopathology MH - Child MH - Child, Preschool MH - *Databases, Factual MH - Female MH - Humans MH - Infant MH - *International Cooperation MH - Male MH - Middle Aged MH - Musculoskeletal Diseases/etiology/immunology/physiopathology MH - Raynaud Disease/etiology/immunology/physiopathology MH - *Scleroderma, Systemic/complications/immunology/mortality/physiopathology MH - Survival Rate EDAT- 2006/11/30 09:00 MHDA- 2007/02/07 09:00 CRDT- 2006/11/30 09:00 PHST- 2006/11/30 09:00 [pubmed] PHST- 2007/02/07 09:00 [medline] PHST- 2006/11/30 09:00 [entrez] AID - 10.1002/art.22207 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Dec;54(12):3971-8. doi: 10.1002/art.22207. PMID- 18565257 OWN - NLM STAT- MEDLINE DCOM- 20080728 LR - 20220410 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 2 DP - 2008 Mar-Apr TI - Cardiovascular risk and prostanoids in systemic sclerosis. PG - 333-6 AB - OBJECTIVE: Systemic sclerosis (SSc) is characterized by Raynaud's phenomenon and frequent cutaneous ulcers. In patients resistant to oral treatments, i.v. prostanoids are usefully employed. Some anecdotal reports underlined the potential risk to develop cardiovascular ischemic complications in prostanoid-treated SSc patients. METHODS: Fifty SSc patients (group 1: 44 female and 6 male, mean age 60.4 +/- 13.8SD) undergoing long-term prostanoid therapy (iloprost or alprostadil) and 42 control patients (group 2), treated with only oral drugs, were retrospectively evaluated for the cardiovascular risk and incidence of ischemic events. RESULTS: Ischemic cardiovascular complications, i.e., myocardial infarction or stroke, were recorded in a significantly higher number of patients undergoing prostanoid treatment compared to controls (group 1: 7/50, 14% vs. group 2: 1/42, 2.4%; p=0.041). Interestingly, these events were significantly more frequent in the subgroup of patients with high cardiovascular risk (group 1: 6/10, 60% vs. group 2: 1/19, 5.2%; p=0.0026). CONCLUSION: The present study suggests a possible role of prostanoid treatment in the pathogenesis of ischemic cardiovascular complications in SSc patients non-responders to oral vasodilators and high cardiovascular risk. Since prostanoids represent the first choice treatment of the most severe scleroderma ischemic cutaneous lesions, cardiovascular risk should be carefully evaluated in all patients before therapy. FAU - Colaci, M AU - Colaci M AD - Chair and Rheumatology Unit, University of Modena e Reggio Emilia, Medical School; Policlinico di Modena, Modena, Italy. FAU - Sebastiani, M AU - Sebastiani M FAU - Giuggioli, D AU - Giuggioli D FAU - Manfredi, A AU - Manfredi A FAU - Rossi, R AU - Rossi R FAU - Modena, M G AU - Modena MG FAU - Ferri, C AU - Ferri C LA - eng PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vasodilator Agents) RN - F5TD010360 (Alprostadil) RN - JED5K35YGL (Iloprost) SB - IM MH - Aged MH - Alprostadil/*adverse effects MH - Female MH - Humans MH - Iloprost/*adverse effects MH - Incidence MH - Male MH - Middle Aged MH - Myocardial Infarction/*epidemiology MH - Raynaud Disease/drug therapy/epidemiology MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*drug therapy/*epidemiology MH - Stroke/*epidemiology MH - Vasodilator Agents/*adverse effects EDAT- 2008/06/21 09:00 MHDA- 2008/07/29 09:00 CRDT- 2008/06/21 09:00 PHST- 2008/06/21 09:00 [pubmed] PHST- 2008/07/29 09:00 [medline] PHST- 2008/06/21 09:00 [entrez] AID - 2305 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 Mar-Apr;26(2):333-6. PMID- 24268960 OWN - NLM STAT- MEDLINE DCOM- 20140226 LR - 20250103 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 42 IP - 12 DP - 2013 Dec TI - [Hand involvement in systemic sclerosis]. PG - 1616-26 LID - S0755-4982(13)00773-2 [pii] LID - 10.1016/j.lpm.2013.10.003 [doi] AB - Hand involvement is very common in patients with systemic sclerosis and represents an important tool for the early diagnosis of the disease. Vascular involvement of the hands is at the forefront of systemic sclerosis, with Raynaud's phenomenon, which may be complicated by digital ulcers, digital necrosis causing pain, infection and significant disability. Joint inflammation can also be disabling and causes the occurrence of contractures and deformities resulting in a marked hand disability. Skin sclerosis involving fingers and hands leads to the occurrence of pain and functional impairment. Hand involvement contributes to 75 % of global disability in patients with systemic sclerosis. Only a comprehensive care, taking into account the vascular, skin and joint manifestations, including physical therapy if needed can improve hand function in patients with systemic sclerosis. CI - Copyright © 2013 Elsevier Masson SAS. All rights reserved. FAU - Mouthon, Luc AU - Mouthon L AD - Assistance publique-Hôpitaux de Paris, hôpital Cochin, université Paris Descartes, centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, service de médecine interne, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France. Electronic address: luc.mouthon@cch.aphp.fr. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - L'atteinte de la main dans la sclérodermie systémique. DEP - 20131121 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 SB - IM MH - Disability Evaluation MH - Persons with Disabilities MH - Fingers/blood supply MH - Hand/blood supply/*physiopathology MH - Hand Dermatoses/etiology/therapy MH - Humans MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications/diagnosis/*physiopathology/therapy MH - Skin Ulcer/diagnosis/etiology EDAT- 2013/11/26 06:00 MHDA- 2014/02/27 06:00 CRDT- 2013/11/26 06:00 PHST- 2013/10/21 00:00 [received] PHST- 2013/10/22 00:00 [accepted] PHST- 2013/11/26 06:00 [entrez] PHST- 2013/11/26 06:00 [pubmed] PHST- 2014/02/27 06:00 [medline] AID - S0755-4982(13)00773-2 [pii] AID - 10.1016/j.lpm.2013.10.003 [doi] PST - ppublish SO - Presse Med. 2013 Dec;42(12):1616-26. doi: 10.1016/j.lpm.2013.10.003. Epub 2013 Nov 21. PMID- 31848701 OWN - NLM STAT- MEDLINE DCOM- 20201109 LR - 20201109 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 79 IP - 7 DP - 2020 Sep TI - [Influence of time to diagnosis of patients with systemic sclerosis on lung function and comorbidities: a preclinical and clinical analysis]. PG - 718-724 LID - 10.1007/s00393-019-00738-6 [doi] AB - BACKGROUND: Scleroderma or systemic sclerosis (SSc) is a rare autoimmune rheumatic connective tissue disease. The clinical picture is manifold and symptoms can vary greatly between different patients. All manifestations are possible ranging from isolated skin involvement up to systemic disease with multiple organ manifestations. Due to this inhomogeneous clinical picture, it often takes years until the correct diagnosis is made and adequate treatment is started. METHODS: Patients with the main or secondary diagnosis of systemic sclerosis (M34) between 2002 and 2017 were retrospectively recorded from the patient databases of the ACURA clinic for acute rheumatology in Bad Kreuznach and the data were evaluated. Of special interest were pulmonary parameters over the course of time. Furthermore, standardized questionnaires were distributed to general practitioners in Rhineland-Palatinate via the Association of Statutory Health Insurance Physicians as well as to patients admitted to the hospital (2016-2017). RESULTS: A total of 135 patients could be evaluated. For women the median age of onset was 52 years (interquartile range, IQR 44-64 years) and for men the median age of onset was 49 years (IQR 38-54 years). Lung involvement was detected in 54% of the cases. Including the individual time to diagnosis, there was a significant worsening of the diffusing capacity for carbon monoxide (73% vs. 56%, p = 0.046) between earlier (<4 months) and later (4-18 months) diagnoses, which also persisted in the follow-up (74% vs. 53%) despite adequate treatment. CONCLUSION: A rapid diagnosis within 3 months of the onset of Raynaud's phenomenon seems to play a key role in the preservation of lung function. FAU - Janto, A AU - Janto A AD - Abteilung Urologie, Siloah St. Trudpert Klinikum, Pforzheim, Deutschland. FAU - Triantafyllias, K AU - Triantafyllias K AD - ACURA Rheumakliniken Rheinland-Pfalz GmbH, Kaiser-Wilhelm-Str. 9-11, 55543, Bad Kreuznach, Deutschland. ktriantafyllias@gmail.com. FAU - Schwarting, A AU - Schwarting A AD - ACURA Rheumakliniken Rheinland-Pfalz GmbH, Kaiser-Wilhelm-Str. 9-11, 55543, Bad Kreuznach, Deutschland. AD - Schwerpunkt Rheumatologie und klinische Immunologie, Universitätsmedizin der Johannes-Gutenberg-Universität, Mainz, Deutschland. LA - ger PT - Journal Article TT - Einfluss der Zeitdauer bis zur Diagnose einer systemischen Sklerose auf Lungenfunktion und Komorbiditäten: eine präklinische und klinische Analyse. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Adult MH - Comorbidity MH - *Connective Tissue Diseases MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Raynaud Disease/diagnosis/epidemiology MH - Retrospective Studies MH - *Scleroderma, Systemic/diagnosis/epidemiology OTO - NOTNLM OT - ADAPTHERA OT - Comorbidities OT - Patient care OT - Scleroderma OT - Time to diagnosis EDAT- 2019/12/19 06:00 MHDA- 2020/11/11 06:00 CRDT- 2019/12/19 06:00 PHST- 2019/12/19 06:00 [pubmed] PHST- 2020/11/11 06:00 [medline] PHST- 2019/12/19 06:00 [entrez] AID - 10.1007/s00393-019-00738-6 [pii] AID - 10.1007/s00393-019-00738-6 [doi] PST - ppublish SO - Z Rheumatol. 2020 Sep;79(7):718-724. doi: 10.1007/s00393-019-00738-6. PMID- 20483576 OWN - NLM STAT- MEDLINE DCOM- 20110224 LR - 20100906 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 104 IP - 10 DP - 2010 Oct TI - Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD). PG - 1527-34 LID - 10.1016/j.rmed.2010.04.022 [doi] AB - BACKGROUND: Although idiopathic nonspecific interstitial pneumonia (NSIP) was initially identified as a provisional diagnosis, the 2008 American Thoracic Society Project concluded that idiopathic NSIP is a distinct form of idiopathic interstitial pneumonia. However, an association between idiopathic NSIP and autoimmune diseases still attracts interest. In this context, a recent study proposed an intriguing concept that idiopathic NSIP is the pulmonary manifestation of undifferentiated connective tissue disease (UCTD). However, this has not been confirmed in a large number of patients with idiopathic NSIP. The present study was conducted to investigate the proportion and characteristics of patients with idiopathic NSIP who meet the criteria for UCTD. METHODS: We reviewed 47 consecutive patients with idiopathic NSIP and examined whether they met prespecified criteria for UCTD. Furthermore, we compared the clinical characteristics between patients fulfilling the UCTD criteria (UCTD-NSIP) and those not meeting them (Non-UCTD-NSIP). RESULTS: Of 47 patients with idiopathic NSIP, 22 (47%) met the UCTD criteria. Common symptoms associated with connective tissue diseases (CTDs) were skin change (50%) and Raynaud's phenomenon (41%) in UCTD-NSIP. UCTD-NSIP showed a female predominance and significantly higher percentages of lymphocytes with a lower CD4/CD8 ratio in bronchoalveolar lavage than Non-UCTD-NSIP. Interestingly, UCTD-NSIP had a significantly better survival than Non-UCTD-NSIP. CONCLUSIONS: Idiopathic NSIP included subjects who fulfilled the UCTD criteria, and these subjects had different clinical characteristics with significantly better outcome than those who did not meet the criteria. These data suggest that a part, but not all, of patients with idiopathic NSIP show CTD-like features with a distinct prognosis. FAU - Suda, Takafumi AU - Suda T AD - 2nd Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan. suda@hama-med.ac.jp FAU - Kono, Masato AU - Kono M FAU - Nakamura, Yutaro AU - Nakamura Y FAU - Enomoto, Noriyuki AU - Enomoto N FAU - Kaida, Yusuke AU - Kaida Y FAU - Fujisawa, Tomoyuki AU - Fujisawa T FAU - Imokawa, Shiro AU - Imokawa S FAU - Yasuda, Kazumasa AU - Yasuda K FAU - Hashizume, Hideo AU - Hashizume H FAU - Yokomura, Koushi AU - Yokomura K FAU - Toyoshima, Mikio AU - Toyoshima M FAU - Koshimizu, Naoki AU - Koshimizu N FAU - Suganuma, Hideki AU - Suganuma H FAU - Shirai, Toshihiro AU - Shirai T FAU - Hashimoto, Dai AU - Hashimoto D FAU - Inui, Naoki AU - Inui N FAU - Colby, Thomas V AU - Colby TV FAU - Chida, Kingo AU - Chida K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100518 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 SB - IM MH - Adult MH - Aged MH - Connective Tissue Diseases/complications/*diagnosis/mortality MH - Diagnosis, Differential MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/complications/*diagnosis/mortality MH - Male MH - Middle Aged MH - Prevalence MH - Prognosis MH - Raynaud Disease/*diagnosis/mortality MH - Young Adult EDAT- 2010/05/21 06:00 MHDA- 2011/02/25 06:00 CRDT- 2010/05/21 06:00 PHST- 2010/02/23 00:00 [received] PHST- 2010/04/15 00:00 [revised] PHST- 2010/04/25 00:00 [accepted] PHST- 2010/05/21 06:00 [entrez] PHST- 2010/05/21 06:00 [pubmed] PHST- 2011/02/25 06:00 [medline] AID - S0954-6111(10)00202-7 [pii] AID - 10.1016/j.rmed.2010.04.022 [doi] PST - ppublish SO - Respir Med. 2010 Oct;104(10):1527-34. doi: 10.1016/j.rmed.2010.04.022. Epub 2010 May 18. PMID- 35640131 OWN - NLM STAT- MEDLINE DCOM- 20221227 LR - 20240909 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 62 IP - 1 DP - 2022 Dec 23 TI - Nailfold capillaroscopy: a survey of current UK practice and 'next steps' to increase uptake among rheumatologists. PG - 335-340 LID - 10.1093/rheumatology/keac320 [doi] AB - OBJECTIVES: To identify barriers to the use of nailfold capillaroscopy as a diagnostic tool for patients presenting with Raynaud's phenomenon in UK rheumatology centres and to obtain rheumatologists' views on a proposed internet-based standardized system for clinical reporting of nailfold capillaroscopy images. METHODS: An online survey was developed using expert opinion from clinicians, scientists and health service researchers. The survey was piloted and sent to UK-based rheumatologists using established electronic mailing lists between October 2020 and March 2021. Survey data were analysed using descriptive statistics. RESULTS: A total of 104 rheumatologists representing rheumatology centres across the UK responded to the survey. Wide variations in terms of workloads and practices were described. Thirty-four (33%) respondents reported using nailfold capillaroscopy only at their own centre, 33 (32%) referred to other centres, 9 (9%) did both and 28 (27%) did not use capillaroscopy at all. Of the 43 respondents using capillaroscopy on site, 25 (58%) used either a dermatoscope or universal serial bus microscope and 9 (21%) used videocapillaroscopy. Among the 61 respondents not undertaking capillaroscopy on site, barriers included lack of equipment (85%), lack of experience in acquiring images (69%) and lack of expertise in interpreting images (67%). Sixty-six respondents (63%) expressed interest in an internet-based, standardized automated system for reporting images. CONCLUSION: Most UK rheumatologists currently do not perform nailfold capillaroscopy on site. An internet-based nailfold capillaroscopy system for use with low-cost microscopes as well as with videocapillaroscopy could help increase uptake of capillaroscopy and thereby facilitate early diagnosis of SSc across the UK. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Eden, Martin AU - Eden M AUID- ORCID: 0000-0002-1542-2527 AD - Manchester Centre for Health Economics. FAU - Wilkinson, Sarah AU - Wilkinson S AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester. FAU - Murray, Andrea AU - Murray A AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre. FAU - Bharathi, Praveen Gurunath AU - Bharathi PG AD - Division of Informatics, Imaging and Data Sciences. FAU - Vail, Andy AU - Vail A AD - Centre for Biostatistics, The University of Manchester, Manchester, UK. FAU - Taylor, Chris J AU - Taylor CJ AD - Division of Informatics, Imaging and Data Sciences. FAU - Payne, Katherine AU - Payne K AD - Manchester Centre for Health Economics. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre. LA - eng GR - II-LB-1117-20006/DH_/Department of Health/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2023 Oct 3;62(10):e303-e304. doi: 10.1093/rheumatology/kead386. PMID: 37527027 CIN - Rheumatology (Oxford). 2023 Oct 3;62(10):e305. doi: 10.1093/rheumatology/kead385. PMID: 37527028 MH - Humans MH - Microscopic Angioscopy/methods MH - Rheumatologists MH - *Raynaud Disease/diagnosis MH - Surveys and Questionnaires MH - United Kingdom MH - Nails/diagnostic imaging MH - Capillaries MH - *Scleroderma, Systemic PMC - PMC9788809 OTO - NOTNLM OT - Raynaud’s phenomenon OT - SSc OT - nailfold capillaroscopy OT - service evaluation EDAT- 2022/06/01 06:00 MHDA- 2022/12/28 06:00 PMCR- 2022/05/28 CRDT- 2022/05/31 16:33 PHST- 2022/03/17 00:00 [received] PHST- 2022/05/20 00:00 [revised] PHST- 2022/06/01 06:00 [pubmed] PHST- 2022/12/28 06:00 [medline] PHST- 2022/05/31 16:33 [entrez] PHST- 2022/05/28 00:00 [pmc-release] AID - 6594483 [pii] AID - keac320 [pii] AID - 10.1093/rheumatology/keac320 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Dec 23;62(1):335-340. doi: 10.1093/rheumatology/keac320. PMID- 29846161 OWN - NLM STAT- MEDLINE DCOM- 20181126 LR - 20260127 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 Suppl 112 IP - 3 DP - 2018 May-Jun TI - Prevalence and spectrum of symptomatic pulmonary involvement in primary Sjögren's syndrome. PG - 94-101 AB - OBJECTIVES: The present cross-sectional study aimed to estimate the prevalence of chronic respiratory symptoms in primary Sjögren's syndrome (pSS) and define the clinical, functional and imaging characteristics of symptomatic pulmonary disease in pSS. METHODS: Four hundred and fourteen consecutive pSS patients were interviewed for the presence of chronic respiratory complaints (cough and/or dyspnea). Symptomatic pSS patients without respiratory or other comorbidities underwent further investigation with clinical evaluation and assessment with pulmonary functional testing (PFTs) and chest high resolution CT (hrCT) on inspiratory and expiratory phase. Comparison of clinical and laboratory features between symptomatic and asymptomatic pSS patients was also performed. RESULTS: Prevalence of chronic respiratory symptoms in pSS was estimated at 21.5% (89/414). Symptoms were attributed to underlying comorbidities in approximately one third of cases (30/89). Thirty nine of the remaining 59 patients were finally assessed with PFTs and hrCT. Small airway disease was diagnosed in 20 individuals with an obstructive pattern in PFTs and/or compatible radiological signs. Seven patients were diagnosed with interstitial lung disease, while in the remaining 12 pSS patients, with normal PFTs and hrCT, symptoms were attributed to xerotrachea. Raynaud's phenomenon occurred more frequently in symptomatic than asymptomatic patients (p=0.024). CONCLUSIONS: Approximately one fifth of a large cohort of pSS patients presented chronic respiratory symptoms. Small airway disease was the most commonly recognized pulmonary disorder among symptomatic pSS patients, followed by xerotrachea and interstitial lung disease. FAU - Kampolis, Christos F AU - Kampolis CF AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece. chkamp77@gmail.com. FAU - Fragkioudaki, Sofia AU - Fragkioudaki S AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Mavragani, Clio P AU - Mavragani CP AD - Department of Pathophysiology, and Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Zormpala, Alexandra AU - Zormpala A AD - Department of Radiology, "Laiko" General Hospital, Athens, Greece. FAU - Samakovli, Anastasia AU - Samakovli A AD - "Evgenidion Clinic Agia Trias", Respiratory Department, Athens, Greece. FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece. LA - eng PT - Journal Article DEP - 20180529 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Aged MH - Chronic Disease MH - Comorbidity MH - Cough/diagnostic imaging/*epidemiology/immunology/physiopathology MH - Cross-Sectional Studies MH - Dyspnea/diagnostic imaging/*epidemiology/immunology/physiopathology MH - Female MH - Greece/epidemiology MH - Humans MH - *Lung/diagnostic imaging/immunology/physiopathology MH - Lung Diseases, Interstitial/diagnostic imaging/*epidemiology/immunology/physiopathology MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/epidemiology/immunology MH - Respiratory Function Tests MH - Risk Factors MH - Sjogren's Syndrome/diagnosis/*epidemiology/immunology MH - Tomography, X-Ray Computed EDAT- 2018/05/31 06:00 MHDA- 2018/11/27 06:00 CRDT- 2018/05/31 06:00 PHST- 2018/01/03 00:00 [received] PHST- 2018/03/26 00:00 [accepted] PHST- 2018/05/31 06:00 [pubmed] PHST- 2018/11/27 06:00 [medline] PHST- 2018/05/31 06:00 [entrez] AID - 12577 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 May-Jun;36 Suppl 112(3):94-101. Epub 2018 May 29. PMID- 38717538 OWN - NLM STAT- MEDLINE DCOM- 20240614 LR - 20241204 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 44 IP - 7 DP - 2024 Jul TI - Insights into systemic lupus erythematosus: a retrospective observational study of clinical features, autoantibodies, and gender-related differences. PG - 1255-1263 LID - 10.1007/s00296-024-05592-7 [doi] AB - This study aims to analyze the clinical and immunologic features of SLE in Jordan, while also investigating the impact of age and gender on disease presentation. The study included 275 patients diagnosed with SLE. Data were collected through meticulous patient interviews and thorough examination of patient hospital records. The cohort exhibited a mean age of 36.8 ± 12.9 years, with an average disease duration of 7.0 ± 7.8 years. The mean age at diagnosis was 29.9 ± 12.1 years, and the female to male ratio was 7.8:1. The most frequently observed symptoms were arthralgia (90.2%), fatigue (80.7%), hematologic manifestations (62%), photosensitivity (60.7%), Raynaud's phenomenon (53.5%), and malar rash (50.9%). The frequencies of various autoantibodies were as follows: ANA (96.7%), anti-dsDNA (39.6%), anti-SSA/Ro (32.8%), anti-Sm (21.8%), anti-U1-RNP (20.6%), and anti-SSB/La (15.5%). Male patients tended to receive a diagnosis at a younger age and exhibited a higher likelihood of experiencing severe manifestations compared to females. Additionally, juvenile onset patients demonstrated an increased likelihood of fever, photosensitivity, myositis, and anti-dsDNA autoantibodies, while adult onset patients were more predisposed to having anti-Ro, anti-La, and RF autoantibodies. This study reveals that the most prevalent manifestations of SLE in the Jordanian cohort encompassed arthralgia, fatigue, and hematologic manifestations. The prevalence of alopecia and Raynaud's phenomenon exceeded that observed in other published cohorts, while arthritis and discoid rash were less frequently encountered. The study highlights that males are more susceptible to developing severe manifestations of SLE compared to females. CI - © 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Adwan, Marwan H AU - Adwan MH AUID- ORCID: 0000-0003-4160-0371 AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. FAU - Qasem, Ula AU - Qasem U AUID- ORCID: 0000-0001-6063-2323 AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. FAU - Atawnah, Saed Y AU - Atawnah SY AUID- ORCID: 0000-0002-2769-137X AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. FAU - Itmeizeh, Muath AU - Itmeizeh M AUID- ORCID: 0000-0002-8513-7967 AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. FAU - Hanbali, Rifaat AU - Hanbali R AUID- ORCID: 0000-0003-2440-5574 AD - Department of Rheumatology, AlBashir Hospital, Amman, Jordan. FAU - Alsoofi, Najla Ali AU - Alsoofi NA AUID- ORCID: 0000-0002-1171-3992 AD - Department of Rheumatology, AlBashir Hospital, Amman, Jordan. FAU - Jbara, Mohammed Abu AU - Jbara MA AUID- ORCID: 0000-0002-3116-0109 AD - Department of Rheumatology, AlBashir Hospital, Amman, Jordan. FAU - AbuHelal, Ayman AU - AbuHelal A AUID- ORCID: 0009-0008-2294-2695 AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. FAU - Alnaimat, Fatima AU - Alnaimat F AUID- ORCID: 0000-0002-5574-2939 AD - Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan. f.naimat@ju.edu.jo. LA - eng PT - Journal Article PT - Observational Study DEP - 20240508 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Male MH - Female MH - Adult MH - *Lupus Erythematosus, Systemic/immunology/epidemiology/complications MH - Middle Aged MH - Retrospective Studies MH - Young Adult MH - Sex Factors MH - Jordan/epidemiology MH - *Autoantibodies/blood MH - Adolescent MH - Raynaud Disease/immunology/epidemiology/etiology MH - Arthralgia/epidemiology/immunology/etiology MH - Antibodies, Antinuclear/blood/immunology MH - Fatigue/epidemiology/etiology MH - Age Factors OTO - NOTNLM OT - Age OT - Arab countries OT - Gender OT - Jordan OT - Systemic Lupus Erythematosus EDAT- 2024/05/08 12:44 MHDA- 2024/06/14 12:44 CRDT- 2024/05/08 11:06 PHST- 2024/01/20 00:00 [received] PHST- 2024/04/06 00:00 [accepted] PHST- 2024/06/14 12:44 [medline] PHST- 2024/05/08 12:44 [pubmed] PHST- 2024/05/08 11:06 [entrez] AID - 10.1007/s00296-024-05592-7 [pii] AID - 10.1007/s00296-024-05592-7 [doi] PST - ppublish SO - Rheumatol Int. 2024 Jul;44(7):1255-1263. doi: 10.1007/s00296-024-05592-7. Epub 2024 May 8. PMID- 16870099 OWN - NLM STAT- MEDLINE DCOM- 20061114 LR - 20220410 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 24 IP - 3 DP - 2006 May-Jun TI - Extra-articular manifestations of rheumatoid arthritis: results of a university hospital of 526 patients in Turkey. PG - 305-8 AB - OBJECTIVE: Presence of extra-articular manifestations (EAM) in rheumatoid arthritis (RA) is associated with more severe disease and increased mortality. Prevalence of EAM may vary in different geographic areas and in different ethnic populations. In this study we investigated the frequency of EAM in 526 RA patients from a single university hospital in Turkey. METHODS: The hospital records of patients who had been diagnosed as RA in Hacettepe University Department of Rheumatology between the years 1988 and 2003 were retrospectively evaluated. There were 73 males and 453 females, and mean age of the patients was 48.0 +/- 12.3 years. The mean follow-up period was 4.8 +/- 4.1 years. Three hundred and fifty-nine patients were rheumatoid factor (RF) positive (68.3%). RESULTS: The overall frequency of EAM was 38.4% (202 patients). The most common EAM was rheumatoid nodules (18.1%). Sicca symptoms, pulmonary findings, Raynaud's phenomenon, livedo reticularis, carpal tunnel syndrome, vasculitis, amyloidosis, and Felty syndrome were present in 11.4%, 4.8%, 3%, 4.8%, 2.8%, 1.3%, 1.1%, and 0.3% of the patients, respectively. Overall EAM and rheumatoid nodules were significantly more common in RF positive patients than RF negative patients. The frequency of rheumatoid nodules was significantly higher in males than in females. CONCLUSION: The prevalence of EAM in Turkey is higher than East Asia and Africa, and lower than UK and North America. Excluding secondary Sjögren's syndrome, our results are similar to other Mediterranean populations like Italy. FAU - Calgüneri, M AU - Calgüneri M AD - Hacettepe University Department of Rheumatology, Ankara, Turkey. FAU - Ureten, K AU - Ureten K FAU - Akif Oztürk, M AU - Akif Oztürk M FAU - Onat, A M AU - Onat AM FAU - Ertenli, I AU - Ertenli I FAU - Kiraz, S AU - Kiraz S FAU - Akdogan, A AU - Akdogan A LA - eng PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Arthritis, Rheumatoid/*complications/pathology MH - Comorbidity MH - Female MH - Hospitals, University MH - Humans MH - Lung Diseases/*complications/pathology MH - Male MH - Middle Aged MH - Raynaud Disease/*complications/pathology MH - Retrospective Studies MH - Rheumatoid Nodule/*etiology/pathology MH - Sjogren's Syndrome/*complications/pathology MH - Skin Diseases, Vascular/*complications/pathology MH - Turkey/epidemiology EDAT- 2006/07/28 09:00 MHDA- 2006/11/15 09:00 CRDT- 2006/07/28 09:00 PHST- 2006/07/28 09:00 [pubmed] PHST- 2006/11/15 09:00 [medline] PHST- 2006/07/28 09:00 [entrez] AID - 1848 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2006 May-Jun;24(3):305-8. PMID- 22246858 OWN - NLM STAT- MEDLINE DCOM- 20120703 LR - 20220410 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 64 IP - 6 DP - 2012 Jun TI - Risk factors for Raynaud's phenomenon in the workforce. PG - 898-904 LID - 10.1002/acr.21615 [doi] AB - OBJECTIVE: To assess the prevalence of and risk factors for Raynaud's phenomenon (RP) in a French working population characterized by various levels of exposure to work-related constraints. METHODS: The study population comprised 3,710 workers (2,161 men and 1,549 women) who were followed up by 83 occupational physicians and were representative of the region's workforce. RP, as diagnosed by a questionnaire and a standardized interview, was defined as the occurrence of at least occasional attacks of finger blanching triggered by exposure to environmental cold during the previous 12 months. Personal factors and work exposure were assessed by self-administered questionnaires. The associations between RP and personal and occupational factors were analyzed using logistic regression modeling. RESULTS: A total of 87 cases of RP (56 women and 31 men) were diagnosed. The population-based annual prevalence rates of RP were 3.6% (95% confidence interval [95% CI] 2.7-4.5%) for women and 1.4% (95% CI 0.9-1.9%) for men. Women had a higher risk of RP (odds ratio [OR] 2.1 [95% CI 1.3-3.4]) and the risk decreased continuously with body mass index (OR for 1-kg/m(2) increment 0.87 [95% CI 0.81-0.94]). The risk of RP increased consistently but moderately with age after 35 years (ORs ranging from 2.0 [95% CI 1.1-3.8] to 2.9 [95% CI 1.6-5.2]). Among the work-related factors studied, RP was associated with an exposure to a cold environment or objects (OR 2.2 [95% CI 1.0-4.6]), a high repetitiveness of a task (OR 1.7 [95% CI 1.0-2.7]), a high psychological demand at work (OR 1.7 [95% CI 1.0-2.7]), and low support from supervisors (OR 2.4 [95% CI 1.5-3.8]). CONCLUSION: Personal and work-related factors were associated with RP, with a clear difference between the sexes. Work-related psychosocial stressors played a significant role independently of biomechanical and environmental exposure. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Roquelaure, Yves AU - Roquelaure Y AD - L'Université Nantes Angers Le Mans, Université d'Angers, Centre Hospitalier Universitaire d'Angers, Laboratoire d'Ergonomie et d'épidémiologie en santé au travail, Angers, France. yvroquelaure@chu-angers.fr FAU - Ha, Catherine AU - Ha C FAU - Le Manac'h, Audrey Petit AU - Le Manac'h AP FAU - Bodin, Julie AU - Bodin J FAU - Bodere, Anaïs AU - Bodere A FAU - Bosseau, Christian AU - Bosseau C FAU - Descatha, Alexis AU - Descatha A FAU - Leclerc, Annette AU - Leclerc A FAU - Goldberg, Marcel AU - Goldberg M FAU - Imbernon, Ellen AU - Imbernon E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120113 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Age Factors MH - Cold Temperature/*adverse effects MH - Cross-Sectional Studies MH - Cumulative Trauma Disorders/complications MH - Female MH - France MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Occupational Health MH - Prevalence MH - Psychology MH - Raynaud Disease/*epidemiology/*etiology MH - Retrospective Studies MH - Risk Factors MH - Surveys and Questionnaires EDAT- 2012/01/17 06:00 MHDA- 2012/07/04 06:00 CRDT- 2012/01/17 06:00 PHST- 2012/01/17 06:00 [entrez] PHST- 2012/01/17 06:00 [pubmed] PHST- 2012/07/04 06:00 [medline] AID - 10.1002/acr.21615 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 Jun;64(6):898-904. doi: 10.1002/acr.21615. Epub 2012 Jan 13. PMID- 29193819 OWN - NLM STAT- MEDLINE DCOM- 20171211 LR - 20220410 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 69 IP - 12 DP - 2017 Dec TI - Efficacy and Safety of Selexipag in Adults With Raynaud's Phenomenon Secondary to Systemic Sclerosis: A Randomized, Placebo-Controlled, Phase II Study. PG - 2370-2379 LID - 10.1002/art.40242 [doi] AB - OBJECTIVE: To determine the effect of selexipag, an oral, selective IP prostacyclin receptor agonist, on the frequency of attacks of Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc). METHODS: Patients with SSc-related RP were randomized 1:1 to placebo (n = 38) or selexipag (n = 36) in individualized doses (maximum of 1,600 μg twice daily) during a 3-week titration period. The primary end point was the weekly average number of RP attacks during the study maintenance period, analyzed using a Bayesian approach with a negative binomial model adjusted for baseline number of RP attacks. Other outcome measures included Raynaud's Condition Score (RCS), RP attack duration, and treatment-emergent adverse events (AEs). RESULTS: Baseline characteristics were comparable between treatment groups. For 83.3% of patients, the individualized maintenance dosage of selexipag was ≤800 μg twice daily. No significant difference was observed between placebo and selexipag in weekly average number of electronic diary (eDiary)-recorded RP attacks during the maintenance period (14.2 attacks during the maintenance period and 21.5 attacks during the baseline week in the placebo group [n = 32] versus 18.0 attacks during the maintenance period and 22.4 attacks during the baseline week in the selexipag group [n = 27]; adjusted mean treatment difference of 3.4 in favor of placebo). No significant treatment effect was observed on RCS or RP attack duration. In the double-blind period, 86.8% of placebo-treated patients and 100% of selexipag-treated patients reported ≥1 AE; 55.3% and 91.7%, respectively, reported ≥1 prostacyclin-associated AE. CONCLUSION: Treatment with selexipag did not reduce the number of RP attacks compared with placebo. The safety profile of selexipag was similar to that previously reported. This study provides important information about the feasibility of eDiary reporting of RP attacks in clinical trials. CI - © 2017, American College of Rheumatology. FAU - Denton, Christopher P AU - Denton CP AD - Royal Free Hospital, London, UK. FAU - Hachulla, Éric AU - Hachulla É AD - National Referral Centre for Rare Systemic Auto-immune Diseases, Department of Internal Medicine, Hôpital Huriez, University of Lille, Lille, France. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Charité University of Medicine, Berlin, Germany. FAU - Schwarting, Andreas AU - Schwarting A AD - Johannes Gutenberg University, Mainz, and ACURA Rheumatology Centre RLP, Bad Kreuznach, Germany. FAU - Frenoux, Jean-Marie AU - Frenoux JM AD - Actelion Pharmaceuticals Ltd., Allschwil, Switzerland. FAU - Frey, Aline AU - Frey A AD - Actelion Pharmaceuticals Ltd., Allschwil, Switzerland. FAU - Le Brun, Franck-Olivier AU - Le Brun FO AD - Actelion Pharmaceuticals Ltd., Allschwil, Switzerland. FAU - Herrick, Ariane L AU - Herrick AL AD - The University of Manchester, Salford Royal NHS Foundation Trust and NIHR Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. CN - Raynaud Study Investigators LA - eng SI - ClinicalTrials.gov/NCT02260557 PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Acetamides) RN - 0 (Antihypertensive Agents) RN - 0 (Pyrazines) RN - 5EXC0E384L (selexipag) SB - IM CIN - Arthritis Rheumatol. 2017 Dec;69(12):2256-2258. doi: 10.1002/art.40307. PMID: 28859256 CIN - Arthritis Rheumatol. 2018 Jun;70(6):974. doi: 10.1002/art.40480. PMID: 29513930 CIN - Arthritis Rheumatol. 2018 Jun;70(6):973-974. doi: 10.1002/art.40481. PMID: 29513932 MH - Acetamides/*administration & dosage MH - Adult MH - Antihypertensive Agents/*administration & dosage MH - Bayes Theorem MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pyrazines/*administration & dosage MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications MH - Severity of Illness Index MH - Treatment Outcome PMC - PMC6099416 EDAT- 2017/12/02 06:00 MHDA- 2017/12/12 06:00 PMCR- 2018/08/20 CRDT- 2017/12/02 06:00 PHST- 2017/04/21 00:00 [received] PHST- 2017/08/22 00:00 [accepted] PHST- 2017/12/02 06:00 [entrez] PHST- 2017/12/02 06:00 [pubmed] PHST- 2017/12/12 06:00 [medline] PHST- 2018/08/20 00:00 [pmc-release] AID - ART40242 [pii] AID - 10.1002/art.40242 [doi] PST - ppublish SO - Arthritis Rheumatol. 2017 Dec;69(12):2370-2379. doi: 10.1002/art.40242. PMID- 19762389 OWN - NLM STAT- MEDLINE DCOM- 20100105 LR - 20101118 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 18 IP - 12 DP - 2009 Oct TI - Prevalence, risk factors and outcome of digital gangrene in 2684 lupus patients. PG - 1112-8 LID - 10.1177/0961203309106643 [doi] AB - The aim of the study is to assess the clinical characteristics, risk factors and outcome of patients with systemic lupus erythematosus (SLE) complicated with digital gangrene. In all, 2684 consecutive SLE inpatients admitted to Peking Union Medical College Hospital from December 1997 to August 2007 were studied. Demographic data, clinical features, laboratory findings as well as therapeutic regimens were systematically reviewed and a database was established. Cases with digital gangrene were identified and followed up. 1) Eighteen patients with SLE were complicated with digital gangrene, the average age at event was 33.1 +/- 11.8 years and the average disease duration was 99.1 +/- 60.1 months. 2) Patients with SLE, with long disease duration (> or =4 years), Raynaud's phenomenon and elevated serum C-reactive protein (CRP) were more likely to develop digital gangrene, P = 0.006, 0.001, and 0.031, respectively, OR = 1.03 (95% CI 1.01, 1.04), 35.76 (95% CI 4.67, 273.83) and 9.93 (95% CI 1.23, 80.30), respectively. 3) Fifteen gangrene patients started prednisone > or =1 mg/kg/d, and 18 were treated with cyclophosphamide, although 8 cases failed and ultimately received digital amputation. Prompt corticosteroid treatment (prednisone > or = mg/kg/d started within 3 weeks) decreased the hazard of amputation, P = 0.073, HR = 0.13 (95% CI 0.01, 1.21). Long disease duration, Raynaud's phenomenon and elevated serum CRP were independent predictive factors for SLE to develop digital gangrene. Early and aggressive corticosteroid treatment prevented gangrene from progression and improved prognosis. FAU - Liu, A AU - Liu A AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Zhang, W AU - Zhang W FAU - Tian, X AU - Tian X FAU - Zhang, X AU - Zhang X FAU - Zhang, F AU - Zhang F FAU - Zeng, X AU - Zeng X LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adult MH - Female MH - Fingers/*pathology MH - Gangrene/diagnosis/*epidemiology/*etiology MH - Humans MH - Kaplan-Meier Estimate MH - Lupus Erythematosus, Systemic/*complications/drug therapy/*pathology MH - Male MH - Prognosis MH - Raynaud Disease/complications/pathology MH - Risk Factors MH - Toes/*pathology MH - Treatment Outcome MH - Young Adult EDAT- 2009/09/19 06:00 MHDA- 2010/01/06 06:00 CRDT- 2009/09/19 06:00 PHST- 2009/09/19 06:00 [entrez] PHST- 2009/09/19 06:00 [pubmed] PHST- 2010/01/06 06:00 [medline] AID - 18/12/1112 [pii] AID - 10.1177/0961203309106643 [doi] PST - ppublish SO - Lupus. 2009 Oct;18(12):1112-8. doi: 10.1177/0961203309106643. PMID- 39467409 OWN - NLM STAT- MEDLINE DCOM- 20241213 LR - 20250903 IS - 1873-2518 (Electronic) IS - 0264-410X (Linking) VI - 42 IP - 26 DP - 2024 Dec 2 TI - Exploring risk factors for Raynaud's phenomenon post COVID-19 vaccination. PG - 126470 LID - S0264-410X(24)01152-6 [pii] LID - 10.1016/j.vaccine.2024.126470 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) has recently been observed in recipients of the COVID-19 vaccine. It is unclear whether RP is directly caused by the COVID-19 vaccine. This study aims to investigate the potential causation between RP and COVID-19 vaccination. METHODS: In this study, we searched PubMed, EMBASE, and Web of Science from January 1, 2020, to March 19, 2024. We included the articles with clinical related findings, specifically case reports and case series. Conference abstracts, editorial publications, preprint, and those not specifically related to COVID-19 vaccination are excluded. The refined selection process aimed to ensure a focused and clinically relevant analysis of the association between RP and COVID-19 vaccination. RESULTS: A total of six articles were ultimately included in this study, comprising five case reports and one case series involving 24 patients with RP after vaccination. Baseline characteristics of the studies showed the RP post COVID-19 vaccination frequently occurred with females compared to males (70.83 vs. 29.17 %). Of the patients with RP post COVID-19 vaccination, 87.5 % (21/24) had either a history or possible predisposing factors of RP. Among the patients with detailed information of vaccination (n = 20), the number of vaccine doses was not related to RP development (45 % (1st) vs. 30 % (2nd) vs. 25 % 3rd dose). For types of vaccine, 75 % of RP were found to have received the administration of mRNA vaccine (15/20). CONCLUSION: The risk of bias was increased due to the uncontrolled study designs and small sample size, making it impossible to attribute causation between RP and COVID-19 vaccination. These few cases may have occurred independently of vaccination. However, physicians should still remain vigilant for RP following COVID-19 vaccination, particularly as the number of vaccinated individuals continues to rise. CI - Copyright © 2024. Published by Elsevier Ltd. FAU - Ho, Tzu-Chuan AU - Ho TC AD - Department of Medical Imaging and Radiological Sciences, Kaohsiung Medical University, Kaohsiung 807, Taiwan. FAU - Chuang, Shih-Chang AU - Chuang SC AD - Division of General and Digestive Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807, Taiwan; School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan. FAU - Hung, Kuo-Chen AU - Hung KC AD - Division of General and Digestive Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807, Taiwan. FAU - Chang, Chin-Chuan AU - Chang CC AD - Department of Nuclear Medicine, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan; School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan. FAU - Chuang, Kuo-Pin AU - Chuang KP AD - Graduate Institute of Animal Vaccine Technology, College of Veterinary Medicine, National Pingtung University of Science and Technology, Pingtung 912, Taiwan. FAU - Yuan, Cheng-Hui AU - Yuan CH AD - Mass Spectrometry Laboratory, Department of Chemistry, National University of Singapore, Singapore 119077, Singapore. FAU - Yang, Ming-Hui AU - Yang MH AD - Division of General and Digestive Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807, Taiwan. Electronic address: 1120439@kmuh.org.tw. FAU - Tyan, Yu-Chang AU - Tyan YC AD - Department of Medical Imaging and Radiological Sciences, Kaohsiung Medical University, Kaohsiung 807, Taiwan; Graduate Institute of Animal Vaccine Technology, College of Veterinary Medicine, National Pingtung University of Science and Technology, Pingtung 912, Taiwan; Department of Medical Research, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan; Center for Tropical Medicine and Infectious Disease Research, Kaohsiung Medical University, Kaohsiung 807, Taiwan; Center for Cancer Research, Kaohsiung Medical University, Kaohsiung 807, Taiwan. Electronic address: yctyan@kmu.edu.tw. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20241029 PL - Netherlands TA - Vaccine JT - Vaccine JID - 8406899 RN - 0 (COVID-19 Vaccines) SB - IM MH - Female MH - Humans MH - Male MH - Middle Aged MH - *COVID-19/prevention & control MH - *COVID-19 Vaccines/adverse effects/administration & dosage MH - *Raynaud Disease/etiology MH - Risk Factors MH - *Vaccination/adverse effects OTO - NOTNLM OT - Autoimmune disorder OT - COVID-19 OT - Raynaud's phenomenon OT - mRNA vaccination COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/10/29 00:22 MHDA- 2024/12/13 11:32 CRDT- 2024/10/28 19:05 PHST- 2024/05/20 00:00 [received] PHST- 2024/10/12 00:00 [revised] PHST- 2024/10/18 00:00 [accepted] PHST- 2024/12/13 11:32 [medline] PHST- 2024/10/29 00:22 [pubmed] PHST- 2024/10/28 19:05 [entrez] AID - S0264-410X(24)01152-6 [pii] AID - 10.1016/j.vaccine.2024.126470 [doi] PST - ppublish SO - Vaccine. 2024 Dec 2;42(26):126470. doi: 10.1016/j.vaccine.2024.126470. Epub 2024 Oct 29. PMID- 34655948 OWN - NLM STAT- MEDLINE DCOM- 20220318 LR - 20250130 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 51 IP - 6 DP - 2021 Dec TI - Association between central nervous system stimulants used to treat attention deficit hyperactivity disorder (ADHD) and Raynaud's phenomenon: A scoping review. PG - 1200-1204 LID - S0049-0172(21)00181-5 [pii] LID - 10.1016/j.semarthrit.2021.09.002 [doi] AB - The association between central nervous (CNS) stimulants used to treat attention deficit hyperactivity disorder (ADHD) and Raynaud's phenomenon (RP) has received little attention to date. Our aim was to map the existing literature on aetiopathogenesis, clinical presentation and management of peripheral vasculopathy, with a focus on RP, secondary to drug therapy for ADHD. We searched the PubMed® database (01/11/1951 to 01/08/2020) and included articles written in English, which focussed on CNS stimulants used to treat ADHD and RP. The search identified 150 articles 9 of which were eligible for inclusion (70 patients). The majority of studies (n = 6) related to children or adolescents; however, adult cases were also identified. Peripheral vascular manifestations included attacks of RP (new and worsening) and cold sensitivity (acrocyanosis and perniosis). Irreversible ischaemic complications including digital autoamputation and lower limb critical digital ischaemia have also been reported. The implicated causative CNS stimulants were Methylphenidate (n = 5), Dextroamphetamine (n = 4), Atomoxetine (n = 2), and Lisdexamphetamine (n = 2). Complete resolution of RP symptoms was observed in half (n = 5) of studies upon drug cessation. Other therapeutic strategies have included dose reduction and switching to an alternative drug therapy. A potential autoimmune association has also been postulated including drug-induced autoimmunity and new cases of systemic sclerosis which have been potentially attributed to treatment. Future research is required to understand the association between CNS stimulant drug therapies for ADHD and peripheral vascular manifestations, including RP. CI - Copyright © 2021 Elsevier Inc. All rights reserved. FAU - Umair, Hafiz M AU - Umair HM AD - Department of Rheumatology, Royal Hallamshire Hospitals, Sheffield Teaching Hospital NHS Foundation Trust, Sheffield, UK. FAU - Sandler, Robert D AU - Sandler RD AD - Department of Rheumatology, Royal Hallamshire Hospitals, Sheffield Teaching Hospital NHS Foundation Trust, Sheffield, UK. FAU - Alunno, Alessia AU - Alunno A AD - Internal Medicine and Nephrology Unit, Department of Life, Health & Environmental Sciences, University of L'Aquila, L'Aquila, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy; Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Tameside Hospital, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, UK; The University of Manchester, Manchester, UK. Electronic address: Michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Journal Article PT - Scoping Review DEP - 20210916 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) RN - 57WVB6I2W0 (Atomoxetine Hydrochloride) SB - IM CIN - Semin Arthritis Rheum. 2022 Dec;57:152115. doi: 10.1016/j.semarthrit.2022.152115. PMID: 36343456 MH - Adolescent MH - Adult MH - Atomoxetine Hydrochloride/therapeutic use MH - *Attention Deficit Disorder with Hyperactivity/drug therapy MH - *Central Nervous System Stimulants/adverse effects MH - Child MH - Humans MH - *Methylphenidate/therapeutic use MH - *Raynaud Disease/drug therapy OTO - NOTNLM OT - Attention deficit hyperactivity disorder OT - Central nervous system stimulants OT - Peripheral ischaemia OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Vasculopathy COIS- Declaration of Competing Interest None. EDAT- 2021/10/17 06:00 MHDA- 2022/03/19 06:00 CRDT- 2021/10/16 20:21 PHST- 2021/07/27 00:00 [received] PHST- 2021/08/27 00:00 [revised] PHST- 2021/09/13 00:00 [accepted] PHST- 2021/10/17 06:00 [pubmed] PHST- 2022/03/19 06:00 [medline] PHST- 2021/10/16 20:21 [entrez] AID - S0049-0172(21)00181-5 [pii] AID - 10.1016/j.semarthrit.2021.09.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2021 Dec;51(6):1200-1204. doi: 10.1016/j.semarthrit.2021.09.002. Epub 2021 Sep 16. PMID- 19258141 OWN - NLM STAT- MEDLINE DCOM- 20090618 LR - 20221207 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 34 IP - 3 DP - 2009 Mar TI - Botulinum toxin type A: a treatment option for digital ischemia in patients with Raynaud's phenomenon. PG - 446-52 LID - 10.1016/j.jhsa.2008.11.026 [doi] AB - PURPOSE: Raynaud's phenomenon is an exaggerated vasospastic response that causes pallor and cyanosis. In the hand, it results in pain, disability, and the need for amputation. Current accepted medical and surgical treatments are not uniformly successful and have their inherent morbidities. Reports in the literature describe the use of botulinum toxin type A (BTX-A) for the treatment of vasospastic ischemia of the digits. We report the results of the treatment of recalcitrant digital ischemia with BTX-A in our institution. METHODS: We performed a retrospective chart review between January 2003 and February 2007. All patients presented with a diagnosis of Raynaud's phenomenon with worsening pain, discoloration, or nonhealing wound of the hand. Patients received BTX-A injections into the perineurovascular tissue of the wrist or the distal palm, or along the digit. Outcomes measured included pain rating, digit color and appearance, transcutaneous oxygen saturation, and healing of chronic ulcers. RESULTS: Twenty-six patients were treated, with a total of 55 treatment encounters. Patients were observed for an average of 18 months. Statistically significant improvements were noted for pain score and digit transcutaneous oxygen saturation measurements after treatment (p < .05). We found smokers and women were more likely to have improved coloration and appearance after injections. Complications included localized injection-related pain and transient intrinsic muscle weakness. CONCLUSIONS: Botulinum toxin type A significantly improves pain and improves healing in Raynaud's patients with few complications. BTX-A was found to be a safe and useful treatment option for vasospastic digital ischemia. FAU - Fregene, Alero AU - Fregene A AD - Division of Plastic Surgery, Henry Ford Hospital, Detroit, MI, USA. FAU - Ditmars, Donald AU - Ditmars D FAU - Siddiqui, Aamir AU - Siddiqui A LA - eng PT - Journal Article PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - S88TT14065 (Oxygen) SB - IM MH - Adult MH - Aged MH - Amputation, Surgical MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Injections MH - Ischemia/*drug therapy MH - Male MH - Middle Aged MH - Muscle Weakness/chemically induced MH - Neuromuscular Agents/*therapeutic use MH - Oxygen/blood MH - Pain Measurement MH - Pigmentation MH - Raynaud Disease/*drug therapy MH - Retrospective Studies MH - Skin Ulcer/drug therapy/etiology MH - Wound Healing EDAT- 2009/03/05 09:00 MHDA- 2009/06/19 09:00 CRDT- 2009/03/05 09:00 PHST- 2008/08/12 00:00 [received] PHST- 2008/11/19 00:00 [revised] PHST- 2008/11/24 00:00 [accepted] PHST- 2009/03/05 09:00 [entrez] PHST- 2009/03/05 09:00 [pubmed] PHST- 2009/06/19 09:00 [medline] AID - S0363-5023(08)01044-7 [pii] AID - 10.1016/j.jhsa.2008.11.026 [doi] PST - ppublish SO - J Hand Surg Am. 2009 Mar;34(3):446-52. doi: 10.1016/j.jhsa.2008.11.026. PMID- 20810512 OWN - NLM STAT- MEDLINE DCOM- 20110429 LR - 20220410 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 12 DP - 2010 Dec TI - Bosentan improves skin perfusion of hands in patients with systemic sclerosis with pulmonary arterial hypertension. PG - 2531-9 LID - 10.3899/jrheum.100358 [doi] AB - OBJECTIVE: Our aim was to investigate effects of bosentan on hand perfusion in patients with systemic sclerosis (SSc) with pulmonary arterial hypertension (PAH), using laser Doppler perfusion imaging (LDPI). METHODS: We enrolled 30 SSc patients with PAH, 30 SSc patients without PAH, and 30 healthy controls. In SSc patients and healthy controls at baseline, skin blood flow of the dorsum of the hands was determined with a Lisca laser Doppler perfusion imager. The dorsal surface of the hands was divided into 3 regions of interest (ROI). ROI 1 included 3 fingers of the hand from the second to the fourth distally to the proximal interphalangeal finger joint. ROI 2 included the area between the proximal interphalangeal and the metacarpophalangeal joint. ROI 3 included only the dorsal surface of the hand without the fingers. LDPI was repeated in SSc patients and controls after 4, 8, and 16 weeks of treatment. In SSc patients, nailfold videocapillaroscopy and Raynaud Condition Score (RCS) were performed at baseline and at 4, 8, and 16 weeks. RESULTS: SSc patients with PAH enrolled in the study received treatment with bosentan as standard care for PAH. In these patients with PAH, after 8 and 16 weeks of treatment, bosentan improved minimum, mean, and maximum perfusion and the perfusion proximal-distal gradient. Bosentan seems to be most effective in patients with the early and active capillaroscopic pattern than in patients with the late pattern. Bosentan improved skin blood flow principally in the ROI 1 compared to the ROI 2 and ROI 3. Bosentan restored the perfusion proximal-distal gradient in 57% of SSc patients with the early capillaroscopic pattern. No significant differences from baseline were observed in the RCS in SSc patients with PAH. CONCLUSION: Bosentan improved skin perfusion in SSc patients with PAH, although it did not ameliorate symptoms of Raynaud's phenomenon. Skin blood perfusion increased in SSc patients with PAH, particularly in the skin region distal to the proximal interphalangeal joint, and in patients with the early/active capillaroscopic pattern. Double-blind randomized clinical trials are needed to evaluate the effects of bosentan on skin perfusion of SSc patients without PAH and with active digital ulcers. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy. FAU - Molinaro, Ilenia AU - Molinaro I FAU - Borghese, Federica AU - Borghese F FAU - Rossi, Carmelina AU - Rossi C FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Clinical Trial PT - Journal Article DEP - 20100901 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - *Antihypertensive Agents/pharmacology/therapeutic use MH - Bosentan MH - Female MH - Humans MH - Hypertension, Pulmonary/complications/*drug therapy/physiopathology MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Raynaud Disease/drug therapy/etiology/physiopathology MH - Regional Blood Flow MH - Scleroderma, Systemic/*drug therapy/physiopathology MH - Skin/*blood supply/*drug effects MH - *Sulfonamides/pharmacology/therapeutic use EDAT- 2010/09/03 06:00 MHDA- 2011/04/30 06:00 CRDT- 2010/09/03 06:00 PHST- 2010/09/03 06:00 [entrez] PHST- 2010/09/03 06:00 [pubmed] PHST- 2011/04/30 06:00 [medline] AID - jrheum.100358 [pii] AID - 10.3899/jrheum.100358 [doi] PST - ppublish SO - J Rheumatol. 2010 Dec;37(12):2531-9. doi: 10.3899/jrheum.100358. Epub 2010 Sep 1. PMID- 17159717 OWN - NLM STAT- MEDLINE DCOM- 20070119 LR - 20191026 IS - 0755-4982 (Print) IS - 0755-4982 (Linking) VI - 35 IP - 12 Pt 2 DP - 2006 Dec TI - [Classification criteria of scleroderma]. PG - 1916-22 AB - Classification criteria of scleroderma aim to delineate the different clinical forms of the disease, which are associated with different prognoses. The basic classification between localized and systemic scleroderma remains pertinent. Among the former, the problems are essentially esthetic or sometimes functional; but the second may yield many other problems which are pertinent to function but also to life, owing to the involvement of body organs, including lungs, heart, digestive tract, kidneys and locomotor system. Overall, the quality of life of scleroderma patients is often poor. The patients are subclassified according to the skin involvement. Those patients with systemic scleroderma involving the trunk are classified as "Cutaneous diffuse systemic scleroderma"; the association of Raynaud's phenomenon, capillaroscopic abnormalities and specific autoantibodies defines "limited systemic scleroderma"; among the latter patients, those with distal skin involvement are classified "Cutaneous limited systemic sclerosis". The old term "CREST" tends to be abandoned due to its lack of specificity. FAU - Cabane, Jean AU - Cabane J AD - Service de Médecine Interne, Hôpital Saint-Antoine, Paris. jean.cabane@sat.ap-hop-paris.fr CN - Groupe Français de Recherche sur la Sclérodermie LA - fre PT - Comparative Study PT - English Abstract PT - Journal Article PT - Review TT - Critères de classification des sclérodermies. PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Biomarkers) SB - IM MH - Biomarkers MH - Humans MH - Prognosis MH - Quality of Life MH - Raynaud Disease/diagnosis MH - Scleroderma, Diffuse/classification/diagnosis/immunology MH - Scleroderma, Limited/classification/diagnosis/immunology MH - Scleroderma, Systemic/*classification/diagnosis/immunology MH - Time Factors RF - 16 EDAT- 2006/12/13 09:00 MHDA- 2007/01/20 09:00 CRDT- 2006/12/13 09:00 PHST- 2006/12/13 09:00 [pubmed] PHST- 2007/01/20 09:00 [medline] PHST- 2006/12/13 09:00 [entrez] AID - S0755-4982(06)74925-9 [pii] AID - 10.1016/s0755-4982(06)74925-9 [doi] PST - ppublish SO - Presse Med. 2006 Dec;35(12 Pt 2):1916-22. doi: 10.1016/s0755-4982(06)74925-9. PMID- 18552072 OWN - NLM STAT- MEDLINE DCOM- 20080709 LR - 20080616 IS - 0342-9601 (Print) IS - 0342-9601 (Linking) VI - 31 IP - 5 DP - 2008 May TI - [Systemic sclerosis]. PG - 162-70; quiz 171-2 AB - Systemic sclerosis (SSc) is a severe fibrotic multiorgan connective tissue disease. Vascular abnormalities such as fingertip ulcers and Raynaud's syndrome as well as involvement of organs including the lungs, heart, kidney and the gastrointestinal tract are prominent features of the disease. There are currently no disease modifying drugs available that can modify the course of the disease. In this review we will discuss medications that have been found to be effective in improving specific organ involvement due to SSc. For the treatment of gastroesophageal reflux disease (GERD), proton pump inhibitors are effective agents. In the setting of clinically significant gastrointestinal dysmotility, metoclopramide, erythromycin and octreotide may be beneficial. Small bowel bacterial overgrowth should be treated with oral antibiotics. Angiotensin converting enzyme inhibitors are the first-line agents for acute renal crisis. A variety of treatment options are available for Raynaud's phenomenon and include calcium channel blockers, iloprost (i. v.), losartan, fluoxetine and sildenafil. Fingertip ulcers can be prevented by using the endothelin receptor antagonist bosentan. The therapeutic options for treatment of pulmonary hypertension associated with SSc include bosentan, sildenafil and various prostacyclin analogs (eg, epoprostenol, treprostinil, iloprost). Sitaxentan, ambrisentan and new phosphodiesterase-5 inhibitors could be new options for therapy as well. Therapeutic options for interstitial lung fibrosis include cyclophosphamide, however, clinical effects are mild to moderate. Methotrexate has been used to treat skin fibrosis and can be beneficial when arthritis is present. FAU - Tamborrini, Giorgio AU - Tamborrini G AD - Rheumaklinik, Universitätsspital Zürich, Zürich, Schweiz. FAU - Distler, Meike AU - Distler M FAU - Distler, Oliver AU - Distler O LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Systemische Sklerose/Sklerodermie. PL - Germany TA - Med Monatsschr Pharm JT - Medizinische Monatsschrift fur Pharmazeuten JID - 7802665 RN - 0 (Immunosuppressive Agents) SB - IM MH - Evidence-Based Medicine MH - Fibrosis/drug therapy/etiology MH - Gastroesophageal Reflux/drug therapy/etiology MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Kidney Diseases/complications MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications/pathology/*therapy RF - 30 EDAT- 2008/06/17 09:00 MHDA- 2008/07/10 09:00 CRDT- 2008/06/17 09:00 PHST- 2008/06/17 09:00 [pubmed] PHST- 2008/07/10 09:00 [medline] PHST- 2008/06/17 09:00 [entrez] PST - ppublish SO - Med Monatsschr Pharm. 2008 May;31(5):162-70; quiz 171-2. PMID- 41487110 OWN - NLM STAT- MEDLINE DCOM- 20260317 LR - 20260317 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 44 IP - 3 DP - 2026 Mar TI - Nailfold videocapillaroscopy in the assessment of juvenile connective tissue diseases. PG - 582-588 LID - 10.55563/clinexprheumatol/6bz7ok [doi] AB - OBJECTIVES: To evaluate nailfold videocapillaroscopy (NVC) findings in paediatric patients with primary Raynaud's phenomenon (pRP) and connective tissue diseases (CTDs). We aimed to assess potential associations between NVC features and clinical/organ involvement in juvenile CTDs. METHODS: NVC was performed in children with pRP and in those with CTDs - including juvenile systemic sclerosis (JSSc), dermatomyositis (JDM), and systemic lupus erythematosus (JSLE) - regardless of RP status, all of whom were followed at our Paediatric Rheumatology Unit. For each patient, 32 images were acquired, and microvascular alterations were analysed and classified as either non-specific or scleroderma patterns (early, active, and late) by two independent observers. A semiquantitative rating scale was adopted to score six capillary abnormalities. RESULTS: A total of 1600 NVC images from 50 subjects (30 females; mean age 16.4±4.0 years) were evaluated. Scleroderma pattern was significantly more frequent in JSSc vs. pRP (p<0.001) and JDM (p<0.005). Differences in capillaroscopic alterations were observed only between pRP and JSSc for reduced capillary density (p<0.001) and presence of giants (p=0.01). Scleroderma pattern was associated with skin fibrosis (21/25 vs. 0/9; p<0.001) and gastrointestinal involvement (17/25 vs. 1/9; p=0.006), with a trend towards significance for digital ulcers (8/25 vs. 0/9; p=0.07). A significantly higher avascular score was found in patients with interstitial lung disease (ILD) than in those without (0.69±0.51 vs. 0.44±0.38; p=0.048). Indeed, patients with severe reduction of capillary density (≤4 capillaries/millimetre) were more likely to have ILD (5/10 vs. 4/13; p=0.02). CONCLUSIONS: NVC is a valuable tool for differentiating pRP from early juvenile CTDs and may help risk stratification for organ involvement, particularly ILD. FAU - Binda, Marco AU - Binda M AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Italy. FAU - Moccaldi, Beatrice AU - Moccaldi B AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Italy. FAU - Tirelli, Francesca AU - Tirelli F AD - Rheumatology Unit, Department of Woman and Child Health, Padova University Hospital, Italy. FAU - Sanetti, Virginia AU - Sanetti V AD - Rheumatology Unit, Department of Woman and Child Health, Padova University Hospital, Italy. FAU - Cuberli, Anna AU - Cuberli A AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Italy. FAU - Benini, Andrea AU - Benini A AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Italy. FAU - Meneghel, Alessandra AU - Meneghel A AD - Rheumatology Unit, Department of Woman and Child Health, Padova University Hospital, Italy. FAU - Ramonda, Roberta AU - Ramonda R AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Italy. FAU - Zulian, Francesco AU - Zulian F AD - Rheumatology Unit, Department of Woman and Child Health, Padova University Hospital, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital; and Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Italy. elisabetta.zanatta@unipd.it. LA - eng PT - Journal Article DEP - 20251204 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - Juvenile systemic scleroderma SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Female MH - Male MH - Adolescent MH - *Raynaud Disease/diagnostic imaging/physiopathology/diagnosis MH - Child MH - *Capillaries/diagnostic imaging/pathology/physiopathology MH - *Nails/blood supply MH - *Scleroderma, Systemic/diagnostic imaging/physiopathology MH - Predictive Value of Tests MH - *Dermatomyositis/diagnostic imaging MH - *Connective Tissue Diseases/diagnosis MH - *Lupus Erythematosus, Systemic/diagnostic imaging/physiopathology EDAT- 2026/01/05 06:47 MHDA- 2026/03/17 14:24 CRDT- 2026/01/05 05:13 PHST- 2025/08/26 00:00 [received] PHST- 2025/10/13 00:00 [accepted] PHST- 2026/03/17 14:24 [medline] PHST- 2026/01/05 06:47 [pubmed] PHST- 2026/01/05 05:13 [entrez] AID - 22981 [pii] AID - 10.55563/clinexprheumatol/6bz7ok [doi] PST - ppublish SO - Clin Exp Rheumatol. 2026 Mar;44(3):582-588. doi: 10.55563/clinexprheumatol/6bz7ok. Epub 2025 Dec 4. PMID- 40170646 OWN - NLM STAT- MEDLINE DCOM- 20250515 LR - 20250515 IS - 1565-1088 (Print) VI - 27 IP - 2 DP - 2025 Feb TI - Low Serum IgG4 in Systemic Sclerosis: Lessons from an Israeli Cohort. PG - 92-97 AB - BACKGROUND: Immunoglobulin 4 (IgG4) is the least abundant immunoglobulin in the sera of healthy individuals; however, its levels can vary in different diseases such as IgG4-related disease (high) or Sjögren's syndrome (low). While previous studies have suggested the importance of IgG4 in autoimmune diseases, the clinical and biological significance of high or low levels remains unclear. OBJECTIVES: To investigate the association between IgG4 antibody levels and systemic sclerosis (SSc), as well as the clinical features of the disease. METHODS: We measured IgG4 levels in the sera of 74 SSc patients from the years 2000 to 2019 and compared them to IgG4 levels in 80 healthy donors from the Israeli national blood bank. We performed correlation analyses between IgG4 levels and various factors, including age, sex, disease subtype, disease duration, organs involved, and medications taken by the patients. RESULTS: Our findings revealed significantly lower IgG4 levels in SSc patients compared to healthy participants. SSc patients receiving steroid treatment exhibited prominently lower IgG4 levels. In addition, SSc patients with Raynaud's phenomenon tended to have lower IgG4 levels compared to those without Raynaud's phenomenon. CONCLUSIONS: Our study demonstrates that IgG4 levels are lower in SSc patients. Further research is needed to elucidate whether this observation contributes to the etiology of the disease or if it represents a common manifestation among other autoimmune diseases. FAU - Ben Ari, Anat AU - Ben Ari A AD - Department of Surgery, Meir Medical Center, Kfar Saba, Israel. FAU - Rabinowicz, Noa AU - Rabinowicz N AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel. FAU - Paran, Haim AU - Paran H AD - Department of Surgery, Meir Medical Center, Kfar Saba, Israel, School of Medicine, Faculty of Medical and Health Sciences, Tel Aviv University, Tel Aviv, Israel. FAU - Carmi, Or AU - Carmi O AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel. FAU - Levy, Yair AU - Levy Y AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel, School of Medicine, Faculty of Medical and Health Sciences, Tel Aviv University, Tel Aviv, Israel. LA - eng PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - 0 (Immunoglobulin G) SB - IM MH - Humans MH - *Scleroderma, Systemic/immunology/blood/drug therapy/physiopathology MH - Male MH - Female MH - *Immunoglobulin G/blood MH - Israel/epidemiology MH - Middle Aged MH - Adult MH - Aged MH - *Raynaud Disease/immunology MH - Cohort Studies MH - Case-Control Studies EDAT- 2025/04/02 06:26 MHDA- 2025/04/02 06:27 CRDT- 2025/04/02 04:13 PHST- 2025/04/02 06:27 [medline] PHST- 2025/04/02 06:26 [pubmed] PHST- 2025/04/02 04:13 [entrez] PST - ppublish SO - Isr Med Assoc J. 2025 Feb;27(2):92-97. PMID- 19350343 OWN - NLM STAT- MEDLINE DCOM- 20090806 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 28 IP - 7 DP - 2009 Jul TI - No effects of bosentan on microvasculature in patients with limited cutaneous systemic sclerosis. PG - 825-33 LID - 10.1007/s10067-009-1157-4 [doi] AB - The endothelium-derived vasoconstrictor molecule endothelin-1 (ET-1) has been suggested to play a role in the pathogenesis of Raynaud's phenomenon (RP) and systemic sclerosis (SSc). We studied the effect of bosentan on microvascular structure and function in patients with RP secondary to limited cutaneous SSc in a mechanistic pilot study. In this single center, open study, 15 patients with limited cutaneous SSc were treated with bosentan for 16 weeks with a follow-up period of 4 weeks. Changes in microvascular structure and function were studied with assessment of vasodilatory microvascular responses using laser Doppler fluxmetry combined with iontophoresis, capillary permeability using fluorescence videomicroscopy, nailfold capillary microscopy, and serological markers of endothelial activation. No significant changes were seen in vasodilator responses to acetylcholine and sodium nitroprusside following bosentan treatment. No effect was noted on capillary permeability during treatment. The number of nailfold capillaries remained unchanged. The endothelial activation marker vascular cell adhesion molecule did not change during treatment, but levels of thrombomodulin significantly decreased after 12 weeks of treatment. Bosentan did not induce significant changes in vasodilator responses, capillary permeability, and capillary density during treatment, so no evidence was obtained for structural improvement of microvascular structure and function in this short-time mechanistic pilot study in patients with lcSSc. FAU - Hettema, Martha E AU - Hettema ME AD - Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, P.O. Box 30001, 9700 RB, Groningen, The Netherlands. M.Hettema@ziekenhuis-mst.nl FAU - Zhang, Dan AU - Zhang D FAU - Stienstra, Ymkje AU - Stienstra Y FAU - Smit, Andries J AU - Smit AJ FAU - Bootsma, Hendrika AU - Bootsma H FAU - Kallenberg, Cees G M AU - Kallenberg CG LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090407 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antihypertensive Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Drug Therapy, Combination MH - Endothelium, Vascular/drug effects/pathology/physiopathology MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation/*drug effects/physiology MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/blood supply MH - Outpatients MH - Pilot Projects MH - Raynaud Disease/complications/*drug therapy/pathology MH - Regional Blood Flow/*drug effects/physiology MH - Scleroderma, Limited/complications/*drug therapy/pathology MH - Sulfonamides/*therapeutic use MH - Treatment Outcome MH - Vasodilation/drug effects/physiology PMC - PMC2686804 EDAT- 2009/04/08 09:00 MHDA- 2009/08/07 09:00 PMCR- 2009/05/27 CRDT- 2009/04/08 09:00 PHST- 2008/10/07 00:00 [received] PHST- 2009/03/05 00:00 [accepted] PHST- 2008/12/11 00:00 [revised] PHST- 2009/04/08 09:00 [entrez] PHST- 2009/04/08 09:00 [pubmed] PHST- 2009/08/07 09:00 [medline] PHST- 2009/05/27 00:00 [pmc-release] AID - 1157 [pii] AID - 10.1007/s10067-009-1157-4 [doi] PST - ppublish SO - Clin Rheumatol. 2009 Jul;28(7):825-33. doi: 10.1007/s10067-009-1157-4. Epub 2009 Apr 7. PMID- 22679320 OWN - NLM STAT- MEDLINE DCOM- 20130524 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2011 DP - 2011 Sep 28 TI - Primary Sjogren's syndrome with tuberculous arthritis of left knee. LID - 10.1136/bcr.07.2011.4488 [doi] LID - bcr0720114488 AB - A 56-year-old woman presented with recurrent pain and swelling of left knee, Raynaud's phenomenon and dry mouth. She was initially diagnosed with primary Sjögren's syndrome and was put on prednisone, which substantially relieved her complaints. But 8 months later, pain and swelling of left knee recurred with spiking fever, chills and shortness of breath. Escalation of prednisone did not improve the pain and swelling of left knee. CT of chest revealed pulmonary interstitial changes with coexisting infection. MRI of left knee was highly consistent with tuberculous arthritis, which was further confirmed by positive blood culture of Mycobacterium tuberculosis and acid-fast stain of synovial fluid. FAU - Zhang, Ting AU - Zhang T AD - Rheumatology Department, Peking Union Medical College Hospital, Beijing, China. FAU - Cong, Jia AU - Cong J FAU - Xu, Dong AU - Xu D FAU - Leng, Xiaomei AU - Leng X FAU - Zhang, Fengchun AU - Zhang F LA - eng PT - Case Reports PT - Journal Article DEP - 20110928 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antitubercular Agents) RN - 0 (Glucocorticoids) RN - VB0R961HZT (Prednisone) SB - IM MH - Antitubercular Agents/therapeutic use MH - Diagnosis, Differential MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - *Knee Joint MH - Magnetic Resonance Imaging MH - Middle Aged MH - Prednisone/therapeutic use MH - Raynaud Disease/*diagnosis MH - Sjogren's Syndrome/*diagnosis/drug therapy MH - Tomography, X-Ray Computed MH - Tuberculosis, Osteoarticular/*diagnosis/drug therapy PMC - PMC3185876 COIS- Competing interests None. EDAT- 2011/01/01 00:00 MHDA- 2013/05/28 06:00 PMCR- 2013/09/30 CRDT- 2012/06/09 06:00 PHST- 2012/06/09 06:00 [entrez] PHST- 2011/01/01 00:00 [pubmed] PHST- 2013/05/28 06:00 [medline] PHST- 2013/09/30 00:00 [pmc-release] AID - bcr.07.2011.4488 [pii] AID - 10.1136/bcr.07.2011.4488 [doi] PST - epublish SO - BMJ Case Rep. 2011 Sep 28;2011:bcr0720114488. doi: 10.1136/bcr.07.2011.4488. PMID- 20144625 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20100601 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 80 IP - 1 DP - 2010 Jul TI - Correlation of infrared thermography and skin perfusion in Raynaud patients and in healthy controls. PG - 54-7 LID - 10.1016/j.mvr.2010.01.010 [doi] AB - BACKGROUND: We aimed to investigate the correlation of infrared thermography (IT) with laser Doppler perfusion imager (LDPI) among patients with primary Raynaud's phenomenon and healthy controls. METHODS: Forty-seven individuals were included: we examined 25 patients with primary Raynaud's phenomenon and 22 age and gender matched healthy controls. IT of the volar surface of the subjects' left hands was performed to record skin temperature while skin perfusion of the same area was determined using LDPI. All measurements were obtained at room temperature (baseline measurements) and following standardized cold provocation. RESULTS: Good correlation of baseline measurements was found between IT and LDPI in primary Raynaud patients and healthy controls (r=0.868, p<0.0001 vs. r=0.790, p<0.0001). Following cold challenge, correlation was weaker in both groups (r=0.742 vs. r=0.766, p<0.0001). Correlation after cold provocation was statistically significant among patients with primary Raynaud's phenomenon in contrast to controls (Chi Quadrat, p=0.023 vs. p=0.306). CONCLUSION: A significant correlation was found between IT and LDPI in primary Raynaud patients and in healthy controls (r=0.868 and r=0.742, both p<0.0001). Following cold provocation, correlation decreases in both groups. Thus, at room temperature IT might substitute for skin perfusion measured by LDPI. CI - Copyright 2010 Elsevier Inc. All rights reserved. FAU - Schlager, Oliver AU - Schlager O AD - Division of Angiology, Department of Internal Medicine II, Medical University Vienna, Vienna General Hospital, Vienna, Austria. FAU - Gschwandtner, Michael E AU - Gschwandtner ME FAU - Herberg, Karin AU - Herberg K FAU - Frohner, Tanja AU - Frohner T FAU - Schillinger, Martin AU - Schillinger M FAU - Koppensteiner, Renate AU - Koppensteiner R FAU - Mlekusch, Wolfgang AU - Mlekusch W LA - eng PT - Journal Article DEP - 20100206 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Female MH - Fingers/physiology/physiopathology MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/*physiology MH - Skin/*blood supply MH - Skin Temperature/*physiology MH - *Thermography EDAT- 2010/02/11 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/02/11 06:00 PHST- 2009/11/11 00:00 [received] PHST- 2010/01/01 00:00 [revised] PHST- 2010/01/28 00:00 [accepted] PHST- 2010/02/11 06:00 [entrez] PHST- 2010/02/11 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - S0026-2862(10)00011-7 [pii] AID - 10.1016/j.mvr.2010.01.010 [doi] PST - ppublish SO - Microvasc Res. 2010 Jul;80(1):54-7. doi: 10.1016/j.mvr.2010.01.010. Epub 2010 Feb 6. PMID- 22972592 OWN - NLM STAT- MEDLINE DCOM- 20130521 LR - 20220410 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 65 IP - 4 DP - 2013 Apr TI - Evaluation of test characteristics for outcome measures used in Raynaud's phenomenon clinical trials. PG - 630-6 LID - 10.1002/acr.21858 [doi] AB - OBJECTIVE: Randomized controlled trials (RCTs) in Raynaud's phenomenon (RP) have shown conflicting efficacy data. Also, there is no consensus on the outcome measures that should be used. Our objectives were to assess the reliability of individual core set measures used in 3 RCTs, evaluate the placebo response for individual core set measures, and determine if a composite of individual core set measures will decrease the placebo response, which may improve our ability to see treatment effects in future trials. METHODS: We analyzed core set measures from 249 patients in the placebo-treated groups from 3 RCTs. Core set measures analyzed included the Raynaud's Condition Score (RCS); patient and physician assessment of RP; pain, numbness, and tingling during an RP attack; average number of attacks/day; and duration of attacks. Intraclass correlation coefficients (ICCs) were calculated during the run-in period to the RCTs. RESULTS: ICCs of ≥0.70 were observed for the RCS, attack symptoms, and average attacks/day. A high placebo response rate was observed for all individual core measures except the duration of attacks. For the RCS, the placebo response ranged from 56% with ≥10% improvement to 19.5% with ≥60% improvement. In contrast, placebo response rates of 10-20% were observed when several core set measures were combined to develop a composite score. CONCLUSION: Outcome measures used in RCTs of RP are associated with marked variability. A combination of outcome measures is associated with low placebo responses. Future studies are needed to assess if a composite score will be able to differentiate placebo from an effective agent. CI - Copyright © 2013 by the American College of Rheumatology. FAU - Gladue, Heather AU - Gladue H AD - University of Michigan Scleroderma Program, Ann Arbor, MI 48109, USA. FAU - Maranian, Paul AU - Maranian P FAU - Paulus, Harold E AU - Paulus HE FAU - Khanna, Dinesh AU - Khanna D LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - K24AR063120/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Vasodilator Agents) SB - IM MH - Dose-Response Relationship, Drug MH - Female MH - Humans MH - Male MH - Middle Aged MH - Outcome Assessment, Health Care/*methods MH - Raynaud Disease/*drug therapy MH - Reproducibility of Results MH - Retrospective Studies MH - Vasodilator Agents/*administration & dosage PMC - PMC3529989 MID - NIHMS405863 EDAT- 2012/09/14 06:00 MHDA- 2013/05/23 06:00 PMCR- 2014/04/01 CRDT- 2012/09/14 06:00 PHST- 2012/04/30 00:00 [received] PHST- 2012/09/05 00:00 [accepted] PHST- 2012/09/14 06:00 [entrez] PHST- 2012/09/14 06:00 [pubmed] PHST- 2013/05/23 06:00 [medline] PHST- 2014/04/01 00:00 [pmc-release] AID - 10.1002/acr.21858 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2013 Apr;65(4):630-6. doi: 10.1002/acr.21858. PMID- 20100796 OWN - NLM STAT- MEDLINE DCOM- 20110311 LR - 20220408 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 4 DP - 2010 Apr TI - Improving outcome prediction of systemic sclerosis from isolated Raynaud's phenomenon: role of autoantibodies and nail-fold capillaroscopy. PG - 797-805 LID - 10.1093/rheumatology/kep447 [doi] AB - OBJECTIVE: A simple weighted prognostic algorithm, based on capillaroscopy and autoantibodies, is developed to classify patients at different risk of transition from isolated RP to SSc within 5 years from the screening visit. METHODS: Two hundred and eighty-eight of 768 patients with isolated RP who underwent capillaroscopy were recruited. The prognostic contributions of capillaroscopic findings (giant loops, haemorrhages and the number of capillaries) and SSc-associated autoantibodies (ACAs, anti-topo I and ANAs) were assessed in a semi-parametric regression models suitable for competing risks. A prognostic index was built by a bagging technique. A structured tree approach was used to extract simple classificatory rules that can be directly interpreted. RESULTS: Thirty-four transitions from isolated RP to SSc and 42 to other CTDs were observed. All of the chosen variables had a substantial prognostic impact. A complex non-linear prognostic pattern was observed for capillaries, with the risk of developing SSc increasing as the number of loops decreased. The presence of ANAs had a strong impact on prognosis [hazard ratio (HR) = 9.70], which was increased by the presence of ACA (HR = 3.94; P < 0.001). A weighted prognostic classification for the development of SSc was constructed using capillary number, giant loops and ANAs. The prognostic discrimination was satisfactory (Harrell's C-index = 0.86). CONCLUSION: Our prognostic model is based on easy-to-obtain features (i.e. the number of capillaries, giant loops and ANAs) and could be used to facilitate clinical decision making in the screening phase, and may also have important implications for stratifying patients into risk groups for future clinical assessment. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Rheumatology, Istituto Gaetano Pini, Milano, Italy. francesca.ingegnoli@unimi.it FAU - Boracchi, Patrizia AU - Boracchi P FAU - Gualtierotti, Roberta AU - Gualtierotti R FAU - Biganzoli, Elia M AU - Biganzoli EM FAU - Zeni, Silvana AU - Zeni S FAU - Lubatti, Chiara AU - Lubatti C FAU - Fantini, Flavio AU - Fantini F LA - eng PT - Journal Article DEP - 20100125 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Autoantibodies/*blood/metabolism MH - Cohort Studies MH - Disease Progression MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Models, Biological MH - *Nails MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/diagnosis/*physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/diagnosis/*physiopathology MH - Severity of Illness Index MH - Skin/blood supply EDAT- 2010/01/27 06:00 MHDA- 2011/03/12 06:00 CRDT- 2010/01/27 06:00 PHST- 2010/01/27 06:00 [entrez] PHST- 2010/01/27 06:00 [pubmed] PHST- 2011/03/12 06:00 [medline] AID - kep447 [pii] AID - 10.1093/rheumatology/kep447 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Apr;49(4):797-805. doi: 10.1093/rheumatology/kep447. Epub 2010 Jan 25. PMID- 18196175 OWN - NLM STAT- MEDLINE DCOM- 20080325 LR - 20131121 IS - 1533-3159 (Print) IS - 1533-3159 (Linking) VI - 11 IP - 1 DP - 2008 Jan TI - Successful treatment of digital ulcers in a scleroderma patient with continuous bilateral thoracic sympathetic block. PG - 91-6 AB - BACKGROUND: Raynaud's phenomenon (RP) associated with connective tissue disease (secondary RP) may be difficult to manage with conservative therapy. A combination of sympathetically mediated vasospasm and vaso-occlusion has been implicated as the etiology of digital ischemic phenomenon. Thoracic sympathetic outflow blocking has been performed with various techniques. However, there have been some limitations in all treatment options. OBJECTIVE: We report on a patient with medically refractory digital ulceration and gangrene caused by scleroderma who was successfully treated with a continuous infusion of mepivacaine into the thoracic sympathetic ganglions as a means to improve finger circulation. CASE REPORT: We are reporting on a 32-year-old female patient suffering from a medically intractable gangrenous ulcer in the right third finger and the left second and third fingers, accompanied by aching pain (VAS, visual analogue scale, 5 - 6/10) and numbness in both forearms. She underwent continuous infusion of mepivacaine through the thoracic sympathetic catheter placed in T2 vertebral segment for 13 days on the right and for 11 days on the left and cervical epidural infusion of mepivcaine with fentanyl for 10 days after the medical treatment failed. Her finger temperature increased 2 degrees C - 5 degrees C during the thoracic sympathetic block with continuous infusion of mepivacine. Her finger wounds healed completely with 13 days of the continuous thoracic sympathetic block without any complications. CONCLUSIONS: Continuous infusion of mepivacaine into the thoracic sympathetic ganglionic space led to the healing of the medically refractory gangrenous ulcer of the fingers in the patient with scleroderma. FAU - Han, Kyung Ream AU - Han KR AD - Pain Clinic, Department of Anesthesiology and Pain Medicine, Ajou University Hospital, Suwon, Korea. FAU - Kim, Chan AU - Kim C FAU - Park, Eun Jung AU - Park EJ LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pain Physician JT - Pain physician JID - 100954394 RN - 0 (Sympatholytics) RN - B6E06QE59J (Mepivacaine) SB - IM MH - Adult MH - *Anesthesia, Conduction MH - Anesthesia, Epidural MH - Female MH - Fingers/blood supply/pathology MH - Ganglia, Sympathetic/drug effects/physiopathology MH - Gangrene/complications/therapy MH - Humans MH - Mepivacaine/*administration & dosage MH - Pain/complications MH - *Pain Management MH - Raynaud Disease/*complications/therapy MH - Scleroderma, Systemic/*complications/therapy MH - Skin Ulcer/*complications/therapy MH - Sympatholytics/*administration & dosage MH - Thoracic Vertebrae EDAT- 2008/01/16 09:00 MHDA- 2008/03/26 09:00 CRDT- 2008/01/16 09:00 PHST- 2008/01/16 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2008/01/16 09:00 [entrez] PST - ppublish SO - Pain Physician. 2008 Jan;11(1):91-6. PMID- 27727416 OWN - NLM STAT- MEDLINE DCOM- 20170529 LR - 20171011 IS - 1652-7518 (Electronic) IS - 0023-7205 (Linking) VI - 113 DP - 2016 Oct 7 TI - [Hypothenar hammer syndrome is rare - or simply an unusually overlooked condition]. LID - DYCP [pii] AB - Hypothenar hammer syndrome is a possibly underdiagnosed but treatable cause of Raynaud's phenomenon and hand ischemia elicited by thombosis or aneurysm secondary to acute or chronic blunt trauma to the ulnar artery at the level of Guyon's canal. This paper provides a summary of the condition with some emphasis on prophylactic and therapeutic aspects. FAU - Seldén, Anders AU - Seldén A AD - Centre for Occupational and Environmental Medicine - Unit of Occupational Health Stockholm, Sweden Centre for Occupational and Environmental Medicine - Unit of Occupational Health Stockholm, Sweden. FAU - Hermiz, Fatin AU - Hermiz F AD - Örebro University Hospital - Dept of Physiology Örebro, Sweden Örebro University Hospital - Dept of Physiology Örebro, Sweden. FAU - Östlund, Bengt AU - Östlund B AD - Nyköping Hospital - Dept of Orthopedics Nyköping, Sweden Nyköping Hospital - Dept of Orthopedics Nyköping, Sweden. LA - swe PT - Journal Article PT - Review TT - Hammarsjuka är ovanligt – eller bara ett ovanligt förbisett tillstånd - Effektiv behandling finns – skärpt differentialdiagnostik är motiverad. DEP - 20161007 PL - Sweden TA - Lakartidningen JT - Lakartidningen JID - 0027707 SB - IM MH - Aneurysm/etiology MH - Arterial Occlusive Diseases/complications/*diagnosis/diagnostic imaging/therapy MH - Critical Pathways MH - Diagnosis, Differential MH - Hand/anatomy & histology/blood supply MH - Hand Injuries/complications/*diagnosis/diagnostic imaging/therapy MH - Humans MH - Ischemia/etiology MH - Raynaud Disease/etiology MH - Ulnar Artery/diagnostic imaging/*injuries MH - Ultrasonography EDAT- 2016/10/12 06:00 MHDA- 2017/05/30 06:00 CRDT- 2016/10/12 06:00 PHST- 2016/10/12 06:00 [entrez] PHST- 2016/10/12 06:00 [pubmed] PHST- 2017/05/30 06:00 [medline] AID - DYCP [pii] PST - epublish SO - Lakartidningen. 2016 Oct 7;113:DYCP. PMID- 27796521 OWN - NLM STAT- MEDLINE DCOM- 20170815 LR - 20181113 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 37 IP - 2 DP - 2017 Feb TI - Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. PG - 245-249 LID - 10.1007/s00296-016-3582-4 [doi] AB - Intravenous iloprost is a first-line option for the treatment of scleroderma-related digital vasculopathy, and some studies have suggested its favourable role on disease progression. The aim of our study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive patients chronically treated with intravenous iloprost. Our retrospective study enrolled 68 scleroderma patients (68 F, 54.4 ± 12.3 years) treated with iloprost for 7.1 ± 2.9 years, with a schedule of 5-6 consecutive daily infusions per month (6 h/day, 0.5-2.0 ng/kg/min). In all patients, modified Rodnan skin score (4.7 ± 5.3 vs. 3.7 ± 5.3, p < 0.0001), systolic pulmonary arterial pressure (sPAP) (30.9 ± 6.4 vs. 24.0 ± 3.2 mmHg, p < 0.0001), tricuspid annular plane systolic excursion (22.1 ± 2.4 vs. 23.8 ± 3.5 mm, p = 0.0001), pro-brain natriuretic peptide (97.2 ± 69.3 vs. 65.8 ± 31.7 pg/ml, p = 0.0005) showed statistically significant improvement from baseline. In the subgroup of patients with baseline sPAP ≥36 mmHg (n = 17), a significant sPAP reduction was observed (from 39.5 ± 3.8 to 25.1 ± 4.5 mmHg, p < 0.0001) after 7.6 ± 2.5 years of follow-up. The number of patients with digital ulcers (DUs) at follow-up was reduced from baseline (42.6 vs. 11.8%, p < 0.001), and none of the free-DU patients at baseline presented DUs at follow-up. An intensive and chronic regimen of IV iloprost administration seems to stabilize and potentially improve the long-term development of disease in SSc patients, as suggested by stabilization or significant improvement of cardiopulmonary parameters and vasculopathy. FAU - Foti, Rosario AU - Foti R AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. rosfoti@tiscali.it. FAU - Visalli, Elisa AU - Visalli E AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Amato, Giorgio AU - Amato G AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Benenati, Alessia AU - Benenati A AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Converso, Giovanni AU - Converso G AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Farina, Alberto AU - Farina A AD - Medical Affairs Department, Italfarmaco S.p.A., Milan, Italy. FAU - Bellofiore, Salvatore AU - Bellofiore S AD - Division of General Thoracic Surgery, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Mulè, Massimiliano AU - Mulè M AD - Division of Cardiology, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. FAU - Di Gangi, Marcella AU - Di Gangi M AD - Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. LA - eng PT - Journal Article DEP - 20161028 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Disease Progression MH - Female MH - Humans MH - Iloprost/administration & dosage/*therapeutic use MH - Infusions, Intravenous MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy MH - Skin Ulcer/*drug therapy/etiology MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage/*therapeutic use PMC - PMC5258785 OTO - NOTNLM OT - Digital ulcers OT - Iloprost OT - Raynaud’s phenomenon OT - Scleroderma OT - Systolic pulmonary arterial pressure COIS- Alberto Farina is an employee of Italfarmaco S.p.A., and the other authors report no conflict of interest. Ethical approval All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent Informed consent was obtained from all individual participants included in the study. EDAT- 2016/11/01 06:00 MHDA- 2017/08/16 06:00 PMCR- 2016/10/28 CRDT- 2016/11/01 06:00 PHST- 2016/07/22 00:00 [received] PHST- 2016/10/19 00:00 [accepted] PHST- 2016/11/01 06:00 [pubmed] PHST- 2017/08/16 06:00 [medline] PHST- 2016/11/01 06:00 [entrez] PHST- 2016/10/28 00:00 [pmc-release] AID - 10.1007/s00296-016-3582-4 [pii] AID - 3582 [pii] AID - 10.1007/s00296-016-3582-4 [doi] PST - ppublish SO - Rheumatol Int. 2017 Feb;37(2):245-249. doi: 10.1007/s00296-016-3582-4. Epub 2016 Oct 28. PMID- 35936062 OWN - NLM STAT- MEDLINE DCOM- 20220809 LR - 20250728 IS - 1742-1241 (Electronic) IS - 1368-5031 (Print) IS - 1368-5031 (Linking) VI - 2022 DP - 2022 TI - Clinical Study on Systemic Lupus Erythematosus Complicated with Knee Bone Infarction. PG - 7025811 LID - 10.1155/2022/7025811 [doi] LID - 7025811 AB - OBJECTIVE: The present study aims to (1) analyze the clinical characteristics and related influencing factors of knee bone infarction in systemic lupus erythematosus (SLE) and (2) improve the understanding of SLE complicated with knee bone infarction. METHODS: The data of patients with SLE complicated with knee bone infarction were retrospectively analysed; patients with SLE during the same period who matched in age, gender, and disease duration were selected as control subjects, with a 1 : 1 ratio with the SLE group. The clinical data were collected to analyze the risk factors for SLE complicated with knee bone infarction. RESULTS: In a total of 36 (6.4%) of 563 patients aged 19-33 (25.8 ± 4.8) years who had SLE during the same period, the disease was complicated with knee bone infarction. The diagnosis of knee bone infarction was made at an SLE duration of 7-65 (26.2 ± 15.7) months. During the SLE course, knee bone infarction occurred within 1 year in 6 cases (16.7%), within 1-5 years in 28 cases (77.8%), and in >5 years in 2 cases (5.6%). Raynaud's phenomenon incidence and anti-nRNP antibody positivity were significantly higher in the knee bone infarction group than in the control group (P < 0.01 and P < 0.05, respectively). The cumulative glucocorticoid dose at 1, 3, and 6 months was significantly higher in the knee bone infarction group than in the control group (P < 0.05). SLE complicated with knee necrosis had a statistically significant rank correlation with Raynaud's phenomenon (r = 0.445, P < 0.001), anti-nRNP antibody (r = 0.309, P=0.008), and renal injury (r = 0.252, P=0.032). The multivariate analysis of SLE complicated with knee bone infarction showed that Raynaud's phenomenon was an independent influencing factor for the complicated knee bone infarction in SLE patients (OR = 4.938, P=0.004), and the probability of SLE complicated with knee bone infarction in Raynaud's phenomenon positive patients was 4.938 times that of Raynaud's phenomenon negative patients. CONCLUSIONS: The risk of knee bone infarction was relatively high in patients with SLE within a 5-year disease course and in young patients. The risk factors were Raynaud's phenomenon, anti-nRNP antibody positivity, and early high-dose glucocorticoid therapy. CI - Copyright © 2022 Gui-Qi Zhu et al. FAU - Zhu, Gui-Qi AU - Zhu GQ AD - Department of Rheumatology, Zaozhuang Municipal Hospital, Zaozhuang 277000, China. FAU - Qiu, Hong-Xia AU - Qiu HX AD - Department of Rheumatology, Xi'an Fifth Hospital, Xi'an 710000, China. FAU - Ma, Xin-Mei AU - Ma XM AD - Department of Rheumatology, Zaozhuang Municipal Hospital, Zaozhuang 277000, China. FAU - Liu, Mei-Xia AU - Liu MX AUID- ORCID: 0000-0001-6832-0404 AD - Department of Physiotherapy, Zaozhuang Municipal Hospital, Zaozhuang 277000, China. LA - eng PT - Journal Article DEP - 20220718 PL - United States TA - Int J Clin Pract JT - International journal of clinical practice JID - 9712381 RN - 0 (Glucocorticoids) SB - IM MH - Glucocorticoids/therapeutic use MH - Humans MH - Infarction/complications MH - *Lupus Erythematosus, Systemic/complications/drug therapy MH - *Raynaud Disease/complications/epidemiology MH - Retrospective Studies PMC - PMC9314162 COIS- The authors declare that they have no conflicts of interest. EDAT- 2022/08/09 06:00 MHDA- 2022/08/10 06:00 PMCR- 2022/07/18 CRDT- 2022/08/08 03:43 PHST- 2022/04/25 00:00 [received] PHST- 2022/06/09 00:00 [revised] PHST- 2022/06/11 00:00 [accepted] PHST- 2022/08/08 03:43 [entrez] PHST- 2022/08/09 06:00 [pubmed] PHST- 2022/08/10 06:00 [medline] PHST- 2022/07/18 00:00 [pmc-release] AID - 10.1155/2022/7025811 [doi] PST - epublish SO - Int J Clin Pract. 2022 Jul 18;2022:7025811. doi: 10.1155/2022/7025811. eCollection 2022. PMID- 16410528 OWN - NLM STAT- MEDLINE DCOM- 20060223 LR - 20250214 IS - 0003-4967 (Print) IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 65 IP - 2 DP - 2006 Feb TI - Clinical characteristics of patients with myositis and autoantibodies to different fragments of the Mi-2 beta antigen. PG - 242-5 AB - OBJECTIVES: To assess the clinical implications of autoantibodies directed against different parts of the Mi-2 beta autoantigen in patients with myositis. METHODS: A systematic assessment of the clinical, laboratory, and histological characteristics of 48 anti-Mi-2 positive patients from six European centres was made. Anti-Mi-2 autoantibodies were determined with an ELISA using four overlapping fragments spanning the entire amino acid sequence of the autoantigen. Data were compared with results for a large group of anti-Mi-2 negative patients with myositis published previously. RESULTS: Anti-Mi-2 autoantibodies were found in dermatomyositis, polymyositis, and inclusion body myositis. In general, myositis with anti-Mi-2 autoantibodies was characterised by relatively mild disease, sometimes accompanied by extra-muscular symptoms, including arthralgia, arthritis, Raynaud's phenomenon, and interstitial lung disease. Cardiac disease was not seen, and treatment response was fair. No differences were found between patients with autoantibodies to different fragments of the Mi-2 beta antigen, except for a potentially increased risk of cancer in patients with antibodies directed to the N-terminal fragment of the autoantigen. CONCLUSIONS: Anti-Mi-2 autoantibodies are not a marker of a specific subtype of myositis. No significant differences between patients with autoantibodies to different fragments of the Mi-2 beta autoantigen are found, with the possible exception of an increased risk of cancer in patients with antibodies to the N-terminal fragment. FAU - Hengstman, G J D AU - Hengstman GJ AD - Neuromuscular Centre Nijmegen, Department of Neurology, University Medical Centre Nijmegen, PO Box 9101, Internal Code 935, 6500 HB Nijmegen, The Netherlands. g.hengstman@neuro.umcn.nl. FAU - Vree Egberts, W T M AU - Vree Egberts WT FAU - Seelig, H P AU - Seelig HP FAU - Lundberg, I E AU - Lundberg IE FAU - Moutsopoulos, H M AU - Moutsopoulos HM FAU - Doria, A AU - Doria A FAU - Mosca, M AU - Mosca M FAU - Vencovsky, J AU - Vencovsky J FAU - van Venrooij, W J AU - van Venrooij WJ FAU - van Engelen, B G M AU - van Engelen BG LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (CHD4 protein, human) RN - 0 (Peptide Fragments) RN - EC 3.5.1.98 (Mi-2 Nucleosome Remodeling and Deacetylase Complex) RN - EC 3.6.1.- (Adenosine Triphosphatases) RN - EC 3.6.4.- (DNA Helicases) SB - IM MH - Adenosine Triphosphatases/*immunology MH - Autoantibodies/*blood MH - Autoantigens/*immunology MH - Chi-Square Distribution MH - DNA Helicases/*immunology MH - Enzyme-Linked Immunosorbent Assay MH - Europe MH - Female MH - Humans MH - Male MH - Mi-2 Nucleosome Remodeling and Deacetylase Complex MH - Muscular Atrophy/complications/immunology MH - Myositis/complications/*immunology MH - Neoplasms/etiology MH - Peptide Fragments/immunology MH - Raynaud Disease/complications/immunology MH - Risk Assessment MH - Statistics, Nonparametric PMC - PMC1798024 COIS- Conflict: None of the authors have a conflict of interest to declare. EDAT- 2006/01/18 09:00 MHDA- 2006/02/24 09:00 PMCR- 2009/02/01 CRDT- 2006/01/18 09:00 PHST- 2006/01/18 09:00 [pubmed] PHST- 2006/02/24 09:00 [medline] PHST- 2006/01/18 09:00 [entrez] PHST- 2009/02/01 00:00 [pmc-release] AID - S0003-4967(24)20300-9 [pii] AID - ar40717 [pii] AID - 10.1136/ard.2005.040717 [doi] PST - ppublish SO - Ann Rheum Dis. 2006 Feb;65(2):242-5. doi: 10.1136/ard.2005.040717. PMID- 21633912 OWN - NLM STAT- MEDLINE DCOM- 20120605 LR - 20161020 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 22 IP - 1 DP - 2012 Feb TI - Efficacy of low-dose imatinib mesylate for cutaneous involvement in systemic sclerosis: a preliminary report of three cases. PG - 94-9 LID - 10.1007/s10165-011-0472-1 [doi] AB - In this pilot study, the effect of low-dose imatinib mesylate (100 mg/day) on cutaneous involvement in patients with systemic sclerosis (SSc) was analyzed. Three patients with SSc were treated with 100 mg/day of imatinib mesylate for 6 months because of pulmonary arterial hypertension refractory to conventional treatments, including beraprost, bosentan, sildenafil, and epoprostenol. Changes in cutaneous involvement were evaluated at 1, 3, and 6 months. During the treatment, the total skin score gradually improved in all of the patients. Contracture of phalanges was attenuated in two patients, one of whom also experienced the partial restoration of large-joint mobility. Nailfold bleeding, initially seen in two patients, was gradually attenuated and had completely disappeared at 6 months. In all patients, Raynaud's phenomenon was attenuated at around 3 months and had completely disappeared at 6 months. Although transient renal dysfunction was observed in one patient, none of the patients experienced common adverse effects of imatinib, such as edema, nausea, rash, and musculoskeletal pain. These clinical data indicate the tolerability and efficacy of low-dose imatinib in SSc, especially against cutaneous vascular involvement, including Raynaud's phenomenon and nailfold bleeding. FAU - Tamaki, Zenshiro AU - Tamaki Z AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. FAU - Asano, Yoshihide AU - Asano Y FAU - Hatano, Masaru AU - Hatano M FAU - Yao, Atsushi AU - Yao A FAU - Kawashima, Tomohiko AU - Kawashima T FAU - Tomita, Manabu AU - Tomita M FAU - Kinugawa, Koichiro AU - Kinugawa K FAU - Nagai, Ryozo AU - Nagai R FAU - Sato, Shinichi AU - Sato S LA - eng PT - Case Reports PT - Journal Article DEP - 20110603 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Benzamides) RN - 0 (Piperazines) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Pyrimidines) RN - 8A1O1M485B (Imatinib Mesylate) SB - IM MH - Aged MH - Benzamides MH - Contracture/drug therapy/etiology/physiopathology MH - Dose-Response Relationship, Drug MH - Female MH - Hemorrhage/drug therapy/etiology/physiopathology MH - Humans MH - Imatinib Mesylate MH - Male MH - Middle Aged MH - Nail Diseases/drug therapy/etiology/physiopathology MH - Nails/blood supply/pathology MH - Piperazines/*therapeutic use MH - Protein Kinase Inhibitors/*therapeutic use MH - Pyrimidines/*therapeutic use MH - Range of Motion, Articular MH - Raynaud Disease/drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy/physiopathology MH - Skin/blood supply/*drug effects/physiopathology MH - Treatment Outcome EDAT- 2011/06/03 06:00 MHDA- 2012/06/06 06:00 CRDT- 2011/06/03 06:00 PHST- 2011/02/21 00:00 [received] PHST- 2011/05/10 00:00 [accepted] PHST- 2011/06/03 06:00 [entrez] PHST- 2011/06/03 06:00 [pubmed] PHST- 2012/06/06 06:00 [medline] AID - 10.1007/s10165-011-0472-1 [doi] PST - ppublish SO - Mod Rheumatol. 2012 Feb;22(1):94-9. doi: 10.1007/s10165-011-0472-1. Epub 2011 Jun 3. PMID- 26088182 OWN - NLM STAT- MEDLINE DCOM- 20170208 LR - 20181202 IS - 1591-9528 (Electronic) IS - 1591-8890 (Linking) VI - 16 IP - 3 DP - 2016 Aug TI - Efficacy of cilostazol for the treatment of Raynaud's phenomenon in systemic sclerosis patients. PG - 407-12 LID - 10.1007/s10238-015-0370-5 [doi] AB - Cilostazol is a selective inhibitor of phosphodiesterase-III with antiplatelet, antithrombotic and vasodilating properties. The aim of our study was to evaluate the effect of the drug on vasculopathy and Raynaud's phenomenon (RP), in a series of patients with systemic sclerosis (SSc), before and after cilostazol treatment. Twenty-one consecutive SSc patients with moderate or severe RP were enrolled in an open-label study. Cilostazol was administered at the dose of 100 mg twice a day, for 12 months. Evaluations included: daily RP attack diary documenting the frequency and duration of RP episodes, Health Assessment Questionnaire-Disability Index, scleroderma visual analogue scales (VAS), flow-mediated dilation and immunological status, including endothelin 1 and interleukin 6 plasma levels. Thirteen patients completed the study. RP duration and daily number episodes recorded over a 3-week period significantly decreased after cilostazol treatment (p = 0.0049 and p = 0.0067, respectively). VAS score indicated a significant amelioration of the patients' perception of RP (p = 0.0117), and both baseline and post-ischemic brachial artery diameters were significantly increased after cilostazol treatment, as compared with basal values (p = 0.0119 and p = 0.0076, respectively). None of the patients developed digital ulcers during the study. A significant clinical improvement of RP was recorded in SSc patients undergoing cilostazol treatment. Study results indicate a potential role of cilostazol as oral maintenance therapy in SSc patients with RP. FAU - Negrini, Simone AU - Negrini S AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. negrini@unige.it. AD - Centre of Excellence for Biomedical Research, University of Genoa, 16132, Genoa, Italy. negrini@unige.it. FAU - Spanò, Francesca AU - Spanò F AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. FAU - Penza, Elena AU - Penza E AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. FAU - Rollando, Daniela AU - Rollando D AD - Unit of Cardiovascular Diseases, University of Genoa, 16132, Genoa, Italy. FAU - Indiveri, Francesco AU - Indiveri F AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. FAU - Filaci, Gilberto AU - Filaci G AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. AD - Centre of Excellence for Biomedical Research, University of Genoa, 16132, Genoa, Italy. FAU - Puppo, Francesco AU - Puppo F AD - Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, 16132, Genoa, Italy. LA - eng PT - Journal Article DEP - 20150619 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Tetrazoles) RN - 0 (Vasodilator Agents) RN - N7Z035406B (Cilostazol) SB - IM MH - Adult MH - Aged MH - Cilostazol MH - Controlled Before-After Studies MH - Humans MH - Middle Aged MH - Raynaud Disease/*therapy MH - Scleroderma, Systemic/*complications MH - Surveys and Questionnaires MH - Tetrazoles/*administration & dosage MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage OTO - NOTNLM OT - Cilostazol OT - Raynaud phenomenon OT - Scleroderma OT - Systemic sclerosis EDAT- 2015/06/20 06:00 MHDA- 2017/02/09 06:00 CRDT- 2015/06/20 06:00 PHST- 2015/01/27 00:00 [received] PHST- 2015/06/08 00:00 [accepted] PHST- 2015/06/20 06:00 [entrez] PHST- 2015/06/20 06:00 [pubmed] PHST- 2017/02/09 06:00 [medline] AID - 10.1007/s10238-015-0370-5 [pii] AID - 10.1007/s10238-015-0370-5 [doi] PST - ppublish SO - Clin Exp Med. 2016 Aug;16(3):407-12. doi: 10.1007/s10238-015-0370-5. Epub 2015 Jun 19. PMID- 22833239 OWN - NLM STAT- MEDLINE DCOM- 20131030 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 4 DP - 2013 Apr TI - Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis-related pulmonary artery hypertension and cutaneous vascular complications. PG - 1047-52 LID - 10.1007/s00296-012-2466-5 [doi] AB - Pulmonary artery hypertension (PAH) remains the leading cause of morbidity and mortality in systemic sclerosis, while Raynaud's phenomenon and digital ulcers significantly add to the morbidity in systemic sclerosis (SSc). This study was undertaken to evaluate the role of sildenafil in PAH, Raynaud's phenomenon, and digital ulcers in systemic sclerosis patients. A prospective, open-label, uncontrolled pilot study was done at a tertiary care centre in India to study the safety and efficacy of oral sildenafil in PAH, Raynaud's phenomenon, digital infarcts, and ulcers in SSc. Seventeen patients fulfilling ACR classification criteria for scleroderma and having PAH were recruited. Six-minute walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and 2D ECHO were performed in all the study subjects at baseline and at 3 months post-treatment. All patients were treated with oral sildenafil 25 mg three times a day for a period of 3 months. The pre- and post-treatment values of mean pulmonary artery pressure (PAP), 6-min walk test, WHO class of dyspnoea, and severity of Raynaud's phenomenon were compared to look for any significant change. Sixteen patients who completed 3-month follow-up had shown statistically significant improvement in 6-min walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and mPAP. Also, there was no occurrence of new digital infarcts or ulcers, and existing ulcers showed signs of healing. Sildenafil is highly efficacious cheaper and safe alternative to other available therapies for SSc-associated PAH, Raynaud's phenomenon, and digital infarcts/ulcers. FAU - Kumar, Uma AU - Kumar U AD - Clinical Immunology and Rheumatology Division, Department of Medicine, All India Institute of Medical Sciences, New Delhi 110029, India. umaakumar@yahoo.co.in FAU - Sankalp, Gokhale AU - Sankalp G FAU - Sreenivas, V AU - Sreenivas V FAU - Kaur, Satbir AU - Kaur S FAU - Misra, Durgaprasanna AU - Misra D LA - eng PT - Clinical Trial PT - Journal Article DEP - 20120726 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM EIN - Rheumatol Int. 2013 Apr;33(4):1053. Gokhle, Sankalp S [corrected to Sankalp, Gokhale] MH - Adult MH - Arterial Pressure/drug effects/physiology MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy/etiology/physiopathology MH - Male MH - Middle Aged MH - Pilot Projects MH - Piperazines/administration & dosage/adverse effects/*therapeutic use MH - Prospective Studies MH - Pulmonary Artery/physiopathology MH - Purines/administration & dosage/adverse effects/therapeutic use MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Systemic/*complications/physiopathology MH - Sildenafil Citrate MH - Skin/blood supply/physiopathology MH - Skin Ulcer/*drug therapy/etiology/physiopathology MH - Sulfones/administration & dosage/adverse effects/*therapeutic use MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage/adverse effects/*therapeutic use EDAT- 2012/07/27 06:00 MHDA- 2013/10/31 06:00 CRDT- 2012/07/27 06:00 PHST- 2012/01/21 00:00 [received] PHST- 2012/07/07 00:00 [accepted] PHST- 2012/07/27 06:00 [entrez] PHST- 2012/07/27 06:00 [pubmed] PHST- 2013/10/31 06:00 [medline] AID - 10.1007/s00296-012-2466-5 [doi] PST - ppublish SO - Rheumatol Int. 2013 Apr;33(4):1047-52. doi: 10.1007/s00296-012-2466-5. Epub 2012 Jul 26. PMID- 24176941 OWN - NLM STAT- MEDLINE DCOM- 20141028 LR - 20220331 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 25 IP - 2 DP - 2014 Feb TI - Immunological profiles determine neurological involvement in Sjögren's syndrome. PG - 177-81 LID - S0953-6205(13)00979-5 [pii] LID - 10.1016/j.ejim.2013.10.005 [doi] AB - BACKGROUND: Up to 68% of patients with primary Sjögren's syndrome (pSS) undergo neurological complications, and evidence for distinct immunological subgroups is emerging. We sought to determine systemic and immunological profiles associated with neurological manifestations. METHODS: 420 patients fulfilling the 2002 American-European pSS criteria were retrospectively analyzed. Neurological manifestations were diagnosed through clinical, biological, electrophysiological, and imaging findings. Biographical, clinical, and laboratory data were compared. RESULTS: Within 93 (22%) patients with neurological manifestations, peripheral and central nervous systems were involved in 66% and 44%, respectively. Raynaud's phenomenon, cutaneous vasculitis, renal involvement, and cryoglobulinemia were associated with sensorimotor neuropathy and mononeuritis multiplex (p<0.05). Conversely, pure sensory neuropathy occurred without extraglandular manifestation, and without anti-Ro/SSA antibodies (p<0.05). All neurological manifestations were associated with increased use of corticosteroids and immunosuppressive drugs (p<0.05). CONCLUSIONS: In pSS, patients with sensorimotor neuropathies and pure sensory neuropathies have distinct extraglandular and immunological profiles. CI - © 2013. FAU - Jamilloux, Yvan AU - Jamilloux Y AD - Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France; EA 3842 - Department of Immunology, Limoges University, Limoges F-87042, France. Electronic address: yvanjamilloux@hotmail.com. FAU - Magy, Laurent AU - Magy L AD - Department of Neurology, Limoges University Hospital, Limoges F-87042, France. FAU - Hurtevent, Jean-François AU - Hurtevent JF AD - Department of Neurology, Lille University Hospital, Lille F-59037, France. FAU - Gondran, Guillaume AU - Gondran G AD - Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France. FAU - de Seze, Jérôme AU - de Seze J AD - Department of Neurology, Strasbourg University Hospital, Strasbourg F-67091, France. FAU - Launay, David AU - Launay D AD - Department of Internal Medicine, Lille University Hospital, Lille F-59037, France. FAU - Ly, Kim H AU - Ly KH AD - Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France. FAU - Lambert, Marc AU - Lambert M AD - Department of Internal Medicine, Lille University Hospital, Lille F-59037, France. FAU - Hachulla, Eric AU - Hachulla E AD - Department of Internal Medicine, Lille University Hospital, Lille F-59037, France. FAU - Hatron, Pierre-Yves AU - Hatron PY AD - Department of Internal Medicine, Lille University Hospital, Lille F-59037, France. FAU - Vidal, Elisabeth AU - Vidal E AD - Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France. FAU - Fauchais, Anne-Laure AU - Fauchais AL AD - Department of Internal Medicine, Limoges University Hospital, Limoges F-87042, France; EA 3842 - Department of Immunology, Limoges University, Limoges F-87042, France. LA - eng PT - Journal Article DEP - 20131029 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Antibodies, Antinuclear) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/*immunology MH - Central Nervous System Diseases/etiology/*immunology MH - Cohort Studies MH - Cryoglobulinemia MH - Female MH - Humans MH - Kidney Diseases/etiology/immunology MH - Male MH - Middle Aged MH - Mononeuropathies/etiology/immunology MH - Motor Neuron Disease/etiology/immunology MH - Peripheral Nervous System Diseases/etiology/*immunology MH - Polyradiculopathy/etiology/immunology MH - Proportional Hazards Models MH - Raynaud Disease/etiology/immunology MH - Retrospective Studies MH - Rheumatoid Factor/*immunology MH - Sjogren's Syndrome/complications/*immunology/physiopathology MH - Somatosensory Disorders/etiology/immunology MH - Vasculitis/etiology/immunology OTO - NOTNLM OT - Anti-SSA antibodies OT - Neurological manifestations OT - Neuronopathy OT - Primary Sjögren's syndrome EDAT- 2013/11/02 06:00 MHDA- 2014/10/29 06:00 CRDT- 2013/11/02 06:00 PHST- 2013/06/06 00:00 [received] PHST- 2013/09/06 00:00 [revised] PHST- 2013/10/13 00:00 [accepted] PHST- 2013/11/02 06:00 [entrez] PHST- 2013/11/02 06:00 [pubmed] PHST- 2014/10/29 06:00 [medline] AID - S0953-6205(13)00979-5 [pii] AID - 10.1016/j.ejim.2013.10.005 [doi] PST - ppublish SO - Eur J Intern Med. 2014 Feb;25(2):177-81. doi: 10.1016/j.ejim.2013.10.005. Epub 2013 Oct 29. PMID- 33664035 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 14 IP - 3 DP - 2021 Mar 4 TI - Novel case of a scleroderma-mimicking syndrome associated with gastrointestinal stromal tumour. LID - 10.1136/bcr-2020-240211 [doi] LID - e240211 AB - We report a case of a 54-year-old man who developed an atypical systemic syndrome involving Raynaud's phenomenon, pulmonary fibrosis and skin thickening. These features were initially suggestive of newly diagnosed scleroderma. However, he displayed atypical clinical features of same, antinuclear antibody was negative and symptoms were refractory to various immunosuppressive therapies. CT imaging revealed a gastric mass, which later proved to be a gastrointestinal stromal tumour (GIST). Resection of the GIST leads to minimal symptomatic improvement. Surveillance imaging 1 year later revealed metastatic deposits. He was subsequently initiated on imatinib therapy, which led to a rapid improvement in fibrotic changes within weeks. While there have been previous descriptions of paraneoplastic fibrotic disorders, this is the first description of a scleroderma mimic in the setting of a GIST. It highlights an important potential overlap in the pathogenesis of these disease processes and the potential efficacy of tyrosine kinase inhibitors for scleroderma-like fibrotic disorders. CI - © BMJ Publishing Group Limited 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Butt, Zaran Ahmad AU - Butt ZA AD - Department of Rheumatology, Beaumont Hospital, Dublin, Leinster, Ireland svcsm4butt.zaran@gmail.com. FAU - Ng, Wan Lin AU - Ng WL AD - Department of Rheumatology, Beaumont Hospital, Dublin, Leinster, Ireland. FAU - Osman, Kamal AU - Osman K AD - Department of Rheumatology, Beaumont Hospital, Dublin, Leinster, Ireland. FAU - Howard, Donough AU - Howard D AD - Department of Rheumatology, Beaumont Hospital, Dublin, Leinster, Ireland. LA - eng PT - Case Reports PT - Journal Article DEP - 20210304 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 8A1O1M485B (Imatinib Mesylate) SB - IM MH - *Gastrointestinal Stromal Tumors/complications/diagnosis MH - Humans MH - Imatinib Mesylate/therapeutic use MH - Male MH - Middle Aged MH - *Raynaud Disease/etiology MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/complications/diagnosis MH - *Stomach Neoplasms PMC - PMC7934738 OTO - NOTNLM OT - biological agents OT - connective tissue disease OT - drugs: musculoskeletal and joint diseases OT - gastric cancer OT - musculoskeletal syndromes COIS- Competing interests: None declared. EDAT- 2021/03/06 06:00 MHDA- 2021/05/15 06:00 PMCR- 2021/03/04 CRDT- 2021/03/05 05:56 PHST- 2021/03/05 05:56 [entrez] PHST- 2021/03/06 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2021/03/04 00:00 [pmc-release] AID - 14/3/e240211 [pii] AID - bcr-2020-240211 [pii] AID - 10.1136/bcr-2020-240211 [doi] PST - epublish SO - BMJ Case Rep. 2021 Mar 4;14(3):e240211. doi: 10.1136/bcr-2020-240211. PMID- 34694689 OWN - NLM STAT- MEDLINE DCOM- 20220314 LR - 20220314 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 24 IP - 12 DP - 2021 Dec TI - Asymptomatic myocardial dysfunction was revealed by feature tracking cardiac magnetic resonance imaging in patients with primary Sjögren's syndrome. PG - 1482-1490 LID - 10.1111/1756-185X.14227 [doi] AB - AIM: To evaluate subclinical left ventricular (LV) regional dysfunction in patients with primary Sjögren's syndrome (pSS) using feature tracking cardiac magnetic resonance (FT-CMR) imaging and to identify pSS characteristics independently associated with LV regional dysfunction. METHOD: Fifty patients with pSS and 20 controls without cardiovascular disease underwent non-contrast CMR imaging. Labial gland biopsy was performed in 42 patients (84%). Disease activity was assessed using the European League Against Rheumatism Sjögren's syndrome disease activity index (ESSDAI). LV global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) were measured using FT-CMR. RESULTS: No significant differences in cardiovascular risk factors were found between the pSS group and controls. The pSS group had significantly lower GLS (P = .015) and GCS (P = .008) than the control group. Multiple linear regression analysis indicated that GCS was significantly associated with Raynaud's phenomenon (P = .015), focus score ≥2 (P = .032), and total ESSDAI score ≥8 (P = .029). CONCLUSION: FT-CMR can reveal subclinical LV regional dysfunction in patients with pSS without cardiovascular disease. Furthermore, patients with pSS and Raynaud's phenomenon, a focus score ≥2, or an ESSDAI score ≥8 were considered to be at high risk for myocardial dysfunction. CI - © 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Yokoe, Isamu AU - Yokoe I AUID- ORCID: 0000-0002-2676-5357 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Kobayashi, Hitomi AU - Kobayashi H AUID- ORCID: 0000-0002-0282-7074 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Nishiwaki, Atsuma AU - Nishiwaki A AUID- ORCID: 0000-0003-2517-7235 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Nagasawa, Yosuke AU - Nagasawa Y AUID- ORCID: 0000-0003-3416-1649 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Kitamura, Noboru AU - Kitamura N AUID- ORCID: 0000-0002-8454-988X AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Haraoka, Masaki AU - Haraoka M AUID- ORCID: 0000-0002-5843-2645 AD - Department of Medical Information and Communication Technology Research, Graduate School of Medicine, St. Marianna University School of Medicine, Kawasaki, Japan. FAU - Kobayashi, Yasuyuki AU - Kobayashi Y AUID- ORCID: 0000-0001-6058-8166 AD - Department of Medical Information and Communication Technology Research, Graduate School of Medicine, St. Marianna University School of Medicine, Kawasaki, Japan. FAU - Takei, Masami AU - Takei M AUID- ORCID: 0000-0002-5791-3359 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. FAU - Nakamura, Hideki AU - Nakamura H AUID- ORCID: 0000-0002-5837-6348 AD - Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan. LA - eng PT - Journal Article DEP - 20211025 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Asymptomatic Diseases MH - Cardiac Imaging Techniques/methods MH - Case-Control Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - Magnetic Resonance Imaging MH - Middle Aged MH - Raynaud Disease/complications MH - Sjogren's Syndrome/blood/complications/*physiopathology MH - Ventricular Dysfunction, Left/*diagnosis/etiology OTO - NOTNLM OT - European League Against Rheumatism Sjӧgren's syndrome disease activity index OT - Raynaud's phenomenon OT - feature tracking cardiac magnetic resonance imaging OT - focus score OT - global longitudinal strain OT - left ventricular regional function OT - primary Sjögren's syndrome EDAT- 2021/10/26 06:00 MHDA- 2022/03/15 06:00 CRDT- 2021/10/25 12:36 PHST- 2021/09/26 00:00 [revised] PHST- 2021/07/10 00:00 [received] PHST- 2021/10/03 00:00 [accepted] PHST- 2021/10/26 06:00 [pubmed] PHST- 2022/03/15 06:00 [medline] PHST- 2021/10/25 12:36 [entrez] AID - 10.1111/1756-185X.14227 [doi] PST - ppublish SO - Int J Rheum Dis. 2021 Dec;24(12):1482-1490. doi: 10.1111/1756-185X.14227. Epub 2021 Oct 25. PMID- 41053948 OWN - NLM STAT- MEDLINE DCOM- 20251007 LR - 20260112 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 28 IP - 10 DP - 2025 Oct TI - A Case Report of Atypical Presentation of Systemic Lupus Erythematosus in a Young Child. PG - e70426 LID - 10.1111/1756-185x.70426 [doi] AB - Systemic lupus erythematosus is a chronic multisystem autoimmune disorder with varied etiology and clinical presentation. It is unusual during infancy. We describe a 2-year-old boy who presented with progressive mucocutaneous lesions with arthralgia and limb pain. He had hepatomegaly, Raynaud's phenomenon, brisk deep tendon reflexes, and bilateral upper and lower limb weakness. On evaluation, he was positive for ANA and SSA only without any complement activation and was subsequently diagnosed with mixed features of both subacute and chronic cutaneous lupus erythematosus. Infantile onset lupus is rare and should be suspected in children with mucocutaneous and musculoskeletal involvement. CI - © 2025 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Agarwal, Saurabh AU - Agarwal S AUID- ORCID: 0009-0004-8185-2076 AD - Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur, India. FAU - Prabhakaran, Kalyana AU - Prabhakaran K AD - Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur, India. FAU - Nalwa, Aasma AU - Nalwa A AD - Department of Pathology, All India Institute of Medical Sciences, Jodhpur, India. FAU - Goyal, Jagdish Prasad AU - Goyal JP AD - Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur, India. FAU - Kumar, Prawin AU - Kumar P AUID- ORCID: 0000-0002-2365-8097 AD - Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur, India. LA - eng PT - Case Reports PT - Letter PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Immunosuppressive Agents) RN - 0 (Biomarkers) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Male MH - Child, Preschool MH - Arthralgia/etiology MH - Treatment Outcome MH - *Lupus Erythematosus, Systemic/diagnosis/immunology/drug therapy MH - Raynaud Disease/etiology MH - Immunosuppressive Agents/therapeutic use MH - Biomarkers/blood MH - Antibodies, Antinuclear/blood MH - Hepatomegaly/etiology EDAT- 2025/10/07 06:29 MHDA- 2025/10/07 06:30 CRDT- 2025/10/07 00:20 PHST- 2025/08/13 00:00 [revised] PHST- 2025/02/26 00:00 [received] PHST- 2025/09/11 00:00 [accepted] PHST- 2025/10/07 06:30 [medline] PHST- 2025/10/07 06:29 [pubmed] PHST- 2025/10/07 00:20 [entrez] AID - 10.1111/1756-185x.70426 [doi] PST - ppublish SO - Int J Rheum Dis. 2025 Oct;28(10):e70426. doi: 10.1111/1756-185x.70426. PMID- 22543529 OWN - NLM STAT- MEDLINE DCOM- 20130425 LR - 20211021 IS - 1432-1076 (Electronic) IS - 0340-6199 (Linking) VI - 171 IP - 11 DP - 2012 Nov TI - A 12-year-old girl with absent radial pulse: arterial thoracic outlet syndrome with subclavian artery aneurysm and thrombosis of the brachial artery. PG - 1707-9 LID - 10.1007/s00431-012-1748-y [doi] AB - Brachial arterial occlusion is rare in children and adolescents. Once a traumatic cause is excluded, the differential diagnosis consists of a variety of rare conditions. We report the case of a 12-year-old girl whose presenting symptoms--an absent radial pulse and Raynaud's phenomenon of the right hand--could be easily mistaken for a vasculitis. She was found to have arterial thoracic outlet syndrome with right subclavian artery compression and aneurysm formation caused by an anomalous first rib and consecutive thromboembolic occlusion of the brachial artery. The diagnosis and differential diagnosis of this condition are reviewed. FAU - Schroeder, S AU - Schroeder S AD - Rheumatology, University Children's Hospital, Steinwiesstr. 75, 8032 Zurich, Switzerland. silke.schroeder@kispi.uzh.ch FAU - Cannizzaro, E AU - Cannizzaro E FAU - Kellenberger, C J AU - Kellenberger CJ FAU - Saurenmann, R K AU - Saurenmann RK LA - eng PT - Case Reports PT - Journal Article DEP - 20120428 PL - Germany TA - Eur J Pediatr JT - European journal of pediatrics JID - 7603873 SB - IM MH - Aneurysm/diagnostic imaging/*etiology MH - *Brachial Artery/diagnostic imaging MH - Child MH - Female MH - Humans MH - Pulse MH - Radiography MH - Raynaud Disease/etiology MH - Ribs/abnormalities MH - *Subclavian Artery/diagnostic imaging MH - Thoracic Outlet Syndrome/complications/*diagnosis MH - Thrombosis/diagnostic imaging/*etiology EDAT- 2012/05/01 06:00 MHDA- 2013/04/26 06:00 CRDT- 2012/05/01 06:00 PHST- 2012/03/06 00:00 [received] PHST- 2012/04/12 00:00 [accepted] PHST- 2012/05/01 06:00 [entrez] PHST- 2012/05/01 06:00 [pubmed] PHST- 2013/04/26 06:00 [medline] AID - 10.1007/s00431-012-1748-y [doi] PST - ppublish SO - Eur J Pediatr. 2012 Nov;171(11):1707-9. doi: 10.1007/s00431-012-1748-y. Epub 2012 Apr 28. PMID- 21320318 OWN - NLM STAT- MEDLINE DCOM- 20110620 LR - 20211020 IS - 1471-2474 (Electronic) IS - 1471-2474 (Linking) VI - 12 DP - 2011 Feb 14 TI - A case report of vibration-induced hand comorbidities in a postwoman. PG - 47 LID - 10.1186/1471-2474-12-47 [doi] AB - BACKGROUND: Prolonged exposure to hand-transmitted vibration is associated with an increased occurrence of symptoms and signs of disorders in the vascular, neurological and osteoarticular systems of the upper limbs. However, the available epidemiological evidence is derived from studies on high vibration levels caused by vibratory tools, whereas little is known about possible upper limb disorders caused by chronic exposure to low vibration levels emitted by fixed sources. CASE PRESENTATION: We present the case of a postwoman who delivered mail for 15 years using a low-powered motorcycle. The woman was in good health until 2002, when she was diagnosed with bilateral Raynaud's phenomenon. In March 2003 a bilateral carpal tunnel syndrome was electromyographically diagnosed; surgical treatment was ineffective. Further examinations in 2005 highlighted the presence of chronic tendonitis (right middle finger flexor). RISK ASSESSMENT: From 1987, for 15 years, our patient rode her motorcycle for 4 h/day, carrying a load of 20-30 kg. For about a quarter of the time she drove over country roads. Using the information collected about the tasks carried out every day by the postwoman and some measurements performed on both handles of the motorcycle, as well as on both iron parts of the handlebars, we reconstructed the woman's previous exposure to hand-arm vibration. 8-hour energy-equivalent frequency weighted acceleration was about 2.4 m/s². The lifetime dose was 1.5 × 10⁹(m²/s⁴)hd. CONCLUSIONS: The particular set of comorbidities presented by our patient suggests a common pathophysiological basis for all the diseases. Considering the level of exposure to vibrations and the lack of specific knowledge on the effects of vibration in women, we hypothesize an association between the work exposure and the onset of the diseases. FAU - Mattioli, Stefano AU - Mattioli S AD - Section of Occupational Medicine, Department of Internal Medicine, Geriatrics and Nephrology, University of Bologna, Bologna, Italy. FAU - Graziosi, Francesca AU - Graziosi F FAU - Bonfiglioli, Roberta AU - Bonfiglioli R FAU - Barbieri, Giuseppe AU - Barbieri G FAU - Bernardelli, Sandra AU - Bernardelli S FAU - Acquafresca, Luciano AU - Acquafresca L FAU - Violante, Francesco S AU - Violante FS FAU - Farioli, Andrea AU - Farioli A FAU - Hagberg, Mats AU - Hagberg M LA - eng PT - Case Reports PT - Journal Article DEP - 20110214 PL - England TA - BMC Musculoskelet Disord JT - BMC musculoskeletal disorders JID - 100968565 SB - IM MH - Carpal Tunnel Syndrome/diagnosis/*epidemiology MH - Comorbidity MH - Electromyography MH - Female MH - Hand-Arm Vibration Syndrome/diagnosis/*epidemiology MH - Humans MH - Middle Aged MH - Motorcycles MH - Occupational Diseases/diagnosis/epidemiology MH - *Postal Service MH - Raynaud Disease/diagnosis/*epidemiology MH - Risk Assessment MH - Tendinopathy/diagnosis/*epidemiology MH - Vibration/adverse effects PMC - PMC3045397 EDAT- 2011/02/16 06:00 MHDA- 2011/06/21 06:00 PMCR- 2011/02/14 CRDT- 2011/02/16 06:00 PHST- 2010/06/18 00:00 [received] PHST- 2011/02/14 00:00 [accepted] PHST- 2011/02/16 06:00 [entrez] PHST- 2011/02/16 06:00 [pubmed] PHST- 2011/06/21 06:00 [medline] PHST- 2011/02/14 00:00 [pmc-release] AID - 1471-2474-12-47 [pii] AID - 10.1186/1471-2474-12-47 [doi] PST - epublish SO - BMC Musculoskelet Disord. 2011 Feb 14;12:47. doi: 10.1186/1471-2474-12-47. PMID- 29605552 OWN - NLM STAT- MEDLINE DCOM- 20190128 LR - 20250530 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 118 DP - 2018 Jul TI - Automated structure and flow measurement - a promising tool in nailfold capillaroscopy. PG - 173-177 LID - S0026-2862(17)30266-2 [pii] LID - 10.1016/j.mvr.2018.03.016 [doi] AB - OBJECTIVES: Despite increasing interest in nailfold capillaroscopy, objective measures of capillary structure and blood flow have been little studied. We aimed to test the hypothesis that structural measurements, capillary flow, and a combined measure have the predictive power to separate patients with systemic sclerosis (SSc) from those with primary Raynaud's phenomenon (PRP) and healthy controls (HC). METHODS: 50 patients with SSc, 12 with PRP, and 50 HC were imaged using a novel capillaroscopy system that generates high-quality nailfold images and provides fully-automated measurements of capillary structure and blood flow (capillary density, mean width, maximum width, shape score, derangement and mean flow velocity). Population statistics summarise the differences between the three groups. Areas under ROC curves (A(Z)) were used to measure classification accuracy when assigning individuals to SSc and HC/PRP groups. RESULTS: Statistically significant differences in group means were found between patients with SSc and both HC and patients with PRP, for all measurements, e.g. mean width (μm) ± SE: 15.0 ± 0.71, 12.7 ± 0.74 and 11.8 ± 0.23 for SSc, PRP and HC respectively. Combining the five structural measurements gave better classification (A(Z) = 0.919 ± 0.026) than the best single measurement (mean width, A(Z) = 0.874 ± 0.043), whilst adding flow further improved classification (A(Z) = 0.930 ± 0.024). CONCLUSIONS: Structural and blood flow measurements are both able to distinguish patients with SSc from those with PRP/HC. Importantly, these hold promise as clinical trial outcome measures for treatments aimed at improving finger blood flow or microvascular remodelling. CI - Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Berks, Michael AU - Berks M AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, The University of Manchester, Manchester, UK. FAU - Dinsdale, Graham AU - Dinsdale G AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Murray, Andrea AU - Murray A AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Moore, Tonia AU - Moore T AD - Salford Royal NHS Foundation Trust, Salford, UK. FAU - Manning, Joanne AU - Manning J AD - Salford Royal NHS Foundation Trust, Salford, UK. FAU - Taylor, Chris AU - Taylor C AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, The University of Manchester, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: ariane.herrick@manchester.ac.uk. LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom GR - 09342/Z/10/Z/WT_/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180329 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Automation MH - Blood Flow Velocity MH - Capillaries/*pathology/*physiopathology MH - Case-Control Studies MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - *Microcirculation MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Predictive Value of Tests MH - Raynaud Disease/*diagnosis/pathology/physiopathology MH - Regional Blood Flow MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis/pathology/physiopathology MH - Young Adult PMC - PMC5956308 OTO - NOTNLM OT - Microcirculation OT - Nailfold capillaroscopy OT - Red blood cell velocity OT - Systemic sclerosis EDAT- 2018/04/02 06:00 MHDA- 2019/01/29 06:00 PMCR- 2018/07/01 CRDT- 2018/04/02 06:00 PHST- 2017/12/07 00:00 [received] PHST- 2018/03/08 00:00 [revised] PHST- 2018/03/28 00:00 [accepted] PHST- 2018/04/02 06:00 [pubmed] PHST- 2019/01/29 06:00 [medline] PHST- 2018/04/02 06:00 [entrez] PHST- 2018/07/01 00:00 [pmc-release] AID - S0026-2862(17)30266-2 [pii] AID - 10.1016/j.mvr.2018.03.016 [doi] PST - ppublish SO - Microvasc Res. 2018 Jul;118:173-177. doi: 10.1016/j.mvr.2018.03.016. Epub 2018 Mar 29. PMID- 16767559 OWN - NLM STAT- MEDLINE DCOM- 20061031 LR - 20161020 IS - 1439-7595 (Print) IS - 1439-7595 (Linking) VI - 16 IP - 3 DP - 2006 TI - A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review. PG - 183-7 AB - We encountered a 62-year-old woman who had systemic sclerosis (SSc) complicated by idiopathic portal hypertension (IPH). She had a 10-year history of scleroderma and Raynaud's phenomenon. She also had pancytopenia, splenomegaly, and esophageal varices. Treatment with prednisolone and endoscopic variceal ligation resulted in improvement of her symptoms. According to our literature review, the prognosis of patients with SSc complicated by IPH is relatively poor. However, the factors that predict outcome of these patients have not been elucidated. FAU - Takagi, Kenji AU - Takagi K AD - Division of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan. kjtakagi@med.toho-u.ac.jp FAU - Nishio, Shinichiro AU - Nishio S FAU - Akimoto, Kimiko AU - Akimoto K FAU - Yoshino, Takumi AU - Yoshino T FAU - Kawai, Shinichi AU - Kawai S LA - eng PT - Case Reports PT - Journal Article PT - Review PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Ascites/etiology/therapy MH - Esophageal and Gastric Varices/etiology/surgery MH - Female MH - Glucocorticoids/*administration & dosage MH - Humans MH - Hypertension, Portal/*complications/*etiology MH - Ligation MH - Middle Aged MH - Pleural Effusion/etiology/therapy MH - Prednisolone/*administration & dosage MH - Prognosis MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications RF - 19 EDAT- 2006/06/13 09:00 MHDA- 2006/11/01 09:00 CRDT- 2006/06/13 09:00 PHST- 2006/02/06 00:00 [received] PHST- 2006/03/28 00:00 [accepted] PHST- 2006/06/13 09:00 [pubmed] PHST- 2006/11/01 09:00 [medline] PHST- 2006/06/13 09:00 [entrez] AID - 10.1007/s10165-006-0479-1 [doi] PST - ppublish SO - Mod Rheumatol. 2006;16(3):183-7. doi: 10.1007/s10165-006-0479-1. PMID- 25330753 OWN - NLM STAT- MEDLINE DCOM- 20150330 LR - 20220317 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 67 IP - 1 DP - 2015 Jan TI - Relationship of age and body mass index to skin temperature and skin perfusion in primary Raynaud's phenomenon. PG - 238-42 LID - 10.1002/art.38923 [doi] AB - OBJECTIVE: To assess the relationship of age and body mass index (BMI) to skin temperature and perfusion in patients with primary Raynaud's phenomenon (RP) compared with controls. METHODS: Patients with RP as well as age- and sex-matched controls underwent external cold provocation by exposure to 20 °C water for 1 minute. Before and after cold provocation, skin temperature and skin perfusion were measured. RESULTS: Twenty-six patients with RP (20 women and 6 men; median age 41.9 years) and 22 controls (17 women and 5 men; median age 42.9 years) were studied. In RP patients, cold exposure led to a median change in skin temperature of -7% (interquartile range [IQR] -13.1, -4.1) and to a median change in skin perfusion of -26.4% (IQR -36.2, 2.9). In controls, skin temperature changed by -15.7% (IQR -18.3, -11.6) and skin perfusion by -33% (IQR -53.3, -1.1) upon cold exposure. In patients with RP, age and BMI were related to skin temperature (for age, r = 0.683, P < 0.0001; for BMI r = 0.657, P < 0.0001) and skin perfusion (for age, r = 0.595, P = 0.002; for BMI, r = 0.653, P < 0.0001), while no association was found in controls. The cold-induced decrease in skin temperature was inversely related to age (r = -0.518, P = 0.003) and BMI (r = -0.662, P < 0.0001) in patients with RP; correlations were not observed in controls. The cold-induced change in skin perfusion was not related to age or BMI in either group. CONCLUSION: The cold-induced decrease in skin temperature is related to age and BMI in patients with RP but not in controls. Further studies are needed to clarify the pathophysiology of digital ischemia in primary RP. CI - Copyright © 2015 by the American College of Rheumatology. FAU - Giurgea, Georgiana-Aura AU - Giurgea GA AD - Medical University of Vienna, Vienna, Austria. FAU - Mlekusch, Wolfgang AU - Mlekusch W FAU - Charwat-Resl, Silvia AU - Charwat-Resl S FAU - Mueller, Markus AU - Mueller M FAU - Hammer, Alexandra AU - Hammer A FAU - Gschwandtner, Michael E AU - Gschwandtner ME FAU - Koppensteiner, Renate AU - Koppensteiner R FAU - Schlager, Oliver AU - Schlager O LA - eng PT - Journal Article PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 SB - IM MH - Adult MH - Aging/*physiology MH - Blood Circulation/physiology MH - *Body Mass Index MH - Case-Control Studies MH - Cold Temperature MH - Female MH - Fingers/blood supply/physiology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*physiopathology MH - Skin/*blood supply/physiopathology MH - Skin Temperature/*physiology EDAT- 2014/10/22 06:00 MHDA- 2015/03/31 06:00 CRDT- 2014/10/22 06:00 PHST- 2014/05/20 00:00 [received] PHST- 2014/10/14 00:00 [accepted] PHST- 2014/10/22 06:00 [entrez] PHST- 2014/10/22 06:00 [pubmed] PHST- 2015/03/31 06:00 [medline] AID - 10.1002/art.38923 [doi] PST - ppublish SO - Arthritis Rheumatol. 2015 Jan;67(1):238-42. doi: 10.1002/art.38923. PMID- 22773149 OWN - NLM STAT- MEDLINE DCOM- 20130117 LR - 20161125 IS - 1473-5628 (Electronic) IS - 0143-3636 (Linking) VI - 33 IP - 10 DP - 2012 Oct TI - Comparison of the diagnostic performances of two protocols of hand perfusion scintigraphy for Raynaud's phenomenon. PG - 1032-8 LID - 10.1097/MNM.0b013e3283567492 [doi] AB - OBJECTIVE: The aim of this study was to compare the diagnostic performances of two hand perfusion scintigraphy protocols for diagnosing Raynaud's phenomenon (RP). METHODS: We enrolled 130 patients who underwent hand perfusion scintigraphy for suspected RP and 40 normal controls. Of these, 66 patients (group A) and 25 normal controls underwent 99mTc-methylene diphosphonate hand perfusion scintigraphy without one-hand chilling, and the finger-to-palm ratio (FPR) was calculated. The remaining 64 patients (group B) and 15 normal controls underwent 99mTc-methylene diphosphonate hand perfusion scintigraphy with one-hand chilling, and three parameters (the chilled-to-ambient hand ratios of the first peak height, initial slope, and blood pool uptake) were calculated. RESULTS: Forty-eight and 47 patients were clinically diagnosed with RP in groups A and B, respectively. In group A, patients with RP had significantly lower FPRs compared with those without RP, and the receiver operating characteristic curve analysis showed that an FPR of 0.51 was the best cutoff value for diagnosing RP, with a sensitivity of 63% and a specificity of 83%. In group B, the three aforementioned parameters differed significantly (lower or higher) between patients with and without RP. The receiver operating characteristic curve analysis provided highly sensitive and specific results for all three parameters. The initial slope ratio showed the highest sensitivity of 87% and a specificity of 88% when using cutoff values of 0.78 and 1.25. CONCLUSION: Although both protocols for hand perfusion scintigraphy showed high specificity for diagnosing RP, the protocol with one-hand chilling showed higher diagnostic ability compared with that without one-hand chilling. FAU - Lee, Jeong Won AU - Lee JW AD - Department of Nuclear Medicine, Jeju National University School of Medicine, Jeju National University Hospital, Jeju, Korea. FAU - Jeong, Woo Seong AU - Jeong WS FAU - Lee, Sang Mee AU - Lee SM FAU - Kim, Jinseok AU - Kim J LA - eng PT - Clinical Trial PT - Comparative Study PT - Journal Article PL - England TA - Nucl Med Commun JT - Nuclear medicine communications JID - 8201017 RN - X89XV46R07 (Technetium Tc 99m Medronate) SB - IM MH - Adult MH - Case-Control Studies MH - Cold Temperature MH - Gated Blood-Pool Imaging/*methods MH - Hand/blood supply/*diagnostic imaging MH - Humans MH - Middle Aged MH - Perfusion Imaging/*methods MH - ROC Curve MH - Raynaud Disease/*diagnostic imaging MH - Technetium Tc 99m Medronate EDAT- 2012/07/10 06:00 MHDA- 2013/01/18 06:00 CRDT- 2012/07/10 06:00 PHST- 2012/07/10 06:00 [entrez] PHST- 2012/07/10 06:00 [pubmed] PHST- 2013/01/18 06:00 [medline] AID - 10.1097/MNM.0b013e3283567492 [doi] PST - ppublish SO - Nucl Med Commun. 2012 Oct;33(10):1032-8. doi: 10.1097/MNM.0b013e3283567492. PMID- 25379977 OWN - NLM STAT- MEDLINE DCOM- 20150310 LR - 20151119 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 44 IP - 1 DP - 2015 TI - Microvascular abnormalities in patients with early systemic sclerosis: less severe morphological changes than in patients with definite disease. PG - 48-55 LID - 10.3109/03009742.2014.926566 [doi] AB - OBJECTIVES: To evaluate the morphological and functional abnormalities of the microcirculation associated with markers of vascular injury in patients with early systemic sclerosis (SSc). METHOD: Forty-six patients with early SSc were compared with 80 patients with definite SSc, 40 patients with primary Raynaud's phenomenon (PRP), and 45 healthy subjects. Widefield nailfold capillaroscopy (NFC) (10-25 × magnification), videocapillaroscopy (200 × magnification), and laser Doppler imaging (LDI) assessment were performed in all participants. The number of capillaries/mm, enlarged, giant and ramified capillaries, microhaemorrhages, and the avascular score were determined by widefield NFC and videocapillaroscopy. Fingertip blood flow (FBF) was measured using LDI before and after cold stimulus (CS). Serum endothelin-1 (ET-1), von Willebrand factor (vWF), and transforming growth factor beta-1 (TGF-β1) were measured by enzyme-linked immunosorbent assay (ELISA). RESULTS: Upon both widefield NFC and videocapillaroscopy, patients with early SSc showed significantly higher numbers of capillaries/mm, lower enlarged and giant capillaries, and a lower avascular score than definite SSc patients (p < 0.001). They also had more enlarged capillaries, microhaemorrhages and a higher avascular score compared to PRP and controls (p < 0.001). FBF before and after CS were significantly higher in controls than in PRP, early SSc, and definite SSc patients (p < 0.001), with no difference between early and definite SSc. Serum levels of ET-1, vWF, and TGF-β1 were similar between early and definite SSc patients. CONCLUSIONS: Early SSc patients showed functional changes and vascular injury marker levels similar to patients with established disease. Nonetheless, the morphological changes were less severe in early SSc, thus providing an opportunity for further prevention of vasculopathy progression. FAU - Camargo, C Z AU - Camargo CZ AD - Rheumatology Division, Federal University of São Paulo , São Paulo , Brazil. FAU - Sekiyama, J Y AU - Sekiyama JY FAU - Arismendi, M I AU - Arismendi MI FAU - Kayser, C AU - Kayser C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141107 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Endothelin-1) RN - 0 (Transforming Growth Factor beta1) RN - 0 (von Willebrand Factor) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Biomarkers/metabolism MH - Cross-Sectional Studies MH - Endothelin-1/metabolism MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fingers/blood supply MH - Humans MH - Male MH - *Microcirculation MH - Microscopic Angioscopy/*methods MH - Microvessels/immunology/*pathology MH - Middle Aged MH - Raynaud Disease/immunology/pathology MH - Scleroderma, Systemic/immunology/*pathology MH - *Severity of Illness Index MH - Transforming Growth Factor beta1/metabolism MH - von Willebrand Factor/metabolism EDAT- 2014/11/08 06:00 MHDA- 2015/03/11 06:00 CRDT- 2014/11/08 06:00 PHST- 2014/11/08 06:00 [entrez] PHST- 2014/11/08 06:00 [pubmed] PHST- 2015/03/11 06:00 [medline] AID - 10.3109/03009742.2014.926566 [doi] PST - ppublish SO - Scand J Rheumatol. 2015;44(1):48-55. doi: 10.3109/03009742.2014.926566. Epub 2014 Nov 7. PMID- 23718566 OWN - NLM STAT- MEDLINE DCOM- 20141114 LR - 20211021 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 15 IP - 3 DP - 2013 TI - Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers. PG - R63 AB - INTRODUCTION: Early systemic sclerosis (SSc) is characterized by Raynaud's phenomenon together with scleroderma marker autoantibodies and/or a scleroderma pattern at capillaroscopy and no other distinctive feature of SSc. Patients presenting with marker autoantibodies plus a capillaroscopic scleroderma pattern seem to evolve into definite SSc more frequently than patients with either feature. Whether early SSc patients with only marker autoantibodies or capillaroscopic positivity differ in any aspect at presentation is unclear. METHODS: Seventy-one consecutive early SSc patients were investigated for preclinical cardiopulmonary alterations. Out of these, 44 patients and 25 controls affected by osteoarthritis or primary fibromyalgia syndrome were also investigated for serum markers of fibroblast (carboxyterminal propeptide of collagen I), endothelial (soluble E-selectin) and T-cell (soluble IL-2 receptor alpha) activation. RESULTS: Thirty-two of the 71 patients (45.1%) had both a marker autoantibody and a capillaroscopic scleroderma pattern (subset 1), 16 patients (22.5%) had only a marker autoantibody (subset 2), and 23 patients (32.4%) had only a capillaroscopic scleroderma pattern (subset 3). Patients with marker autoantibodies (n = 48, 67.6%) had a higher prevalence of impaired diffusing lung capacity for carbon monoxide (P = 0.0217) and increased serum levels of carboxyterminal propeptide of collagen I (P = 0.0037), regardless of capillaroscopic alterations. Patients with a capillaroscopic scleroderma pattern (n = 55, 77.5%) had a higher prevalence of puffy fingers (P = 0.0001) and increased serum levels of soluble E-selectin (P = 0.0003) regardless of marker autoantibodies. CONCLUSION: These results suggest that the autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Longitudinal studies are warranted to investigate whether these subsets undergo a different disease course over time. FAU - Valentini, Gabriele AU - Valentini G FAU - Marcoccia, Antonella AU - Marcoccia A FAU - Cuomo, Giovanna AU - Cuomo G FAU - Vettori, Serena AU - Vettori S FAU - Iudici, Michele AU - Iudici M FAU - Bondanini, Francesco AU - Bondanini F FAU - Santoriello, Carlo AU - Santoriello C FAU - Ciani, Aldo AU - Ciani A FAU - Cozzolino, Domenico AU - Cozzolino D FAU - De Matteis, Giovanni Maria AU - De Matteis GM FAU - Cappabianca, Salvatore AU - Cappabianca S FAU - Vitelli, Filiberto AU - Vitelli F FAU - Spanò, Alberto AU - Spanò A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Arthritis/epidemiology/etiology MH - Autoantibodies/*blood MH - Autoantigens/immunology MH - Biomarkers/*blood MH - Disease Progression MH - Female MH - Humans MH - Lung Diseases/epidemiology/etiology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prevalence MH - Raynaud Disease/epidemiology/etiology MH - Scleroderma, Systemic/*immunology/*pathology MH - Young Adult PMC - PMC4060381 EDAT- 2013/05/31 06:00 MHDA- 2014/11/15 06:00 PMCR- 2013/05/29 CRDT- 2013/05/31 06:00 PHST- 2012/11/06 00:00 [received] PHST- 2013/02/20 00:00 [revised] PHST- 2013/05/29 00:00 [accepted] PHST- 2013/05/31 06:00 [entrez] PHST- 2013/05/31 06:00 [pubmed] PHST- 2014/11/15 06:00 [medline] PHST- 2013/05/29 00:00 [pmc-release] AID - ar4236 [pii] AID - 10.1186/ar4236 [doi] PST - ppublish SO - Arthritis Res Ther. 2013;15(3):R63. doi: 10.1186/ar4236. PMID- 23557780 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - A Delphi exercise and cluster analysis to aid in the development of potential classification criteria for systemic sclerosis using SSc experts and databases. PG - 24-30 AB - OBJECTIVES: Since the 1980 ACR classification criteria for systemic sclerosis (SSc) do not identify 20% with SSc, revised criteria are necessary. METHODS: Suggested new criteria from the literature were sent in random order to 96 SSc experts. A 3-round Delphi Consensus eliminated criteria. Then cluster analysis reduced items. The Canadian Scleroderma Research Group (CSRG) database was used to determine the prevalence of each item. RESULTS: Seventy-one of 96 (71%) completed all 3 rounds; 47 items were expanded to 76 in round 2. Thirty items had at least 50% consensus and 18 had >75% agreement to include (a priori cut point). Clustering occurred for 4 categories: proximal to MCP skin involvement, vascular abnormalities, autoantibodies and tissue damage. Proximal to MCPs skin involvement identified 80% of patients. Adding one item from each of the other 3 categories or 1 or more items from 2 of 3 remaining categories increased the proportion of patients classified to 94% in CSRG patients. Categories included (1) Vascular (dilated capillaries, telangiectasia, Raynaud's phenomenon [RP]), (2) Autoantibodies (anticentromere [ACA] or antitopoisomeraseI [Topo1]) and (3) Fibrosis/damage (esophogeal dysmotility dysphagia, sclerodactyly, digital ulcers). In the CSRG, 98% were identified if using proximal skin involvement; or sclerodactyly plus one of: RP, ACA or Topo1. CONCLUSIONS: This is a first step toward developing new SSc classification criteria. A Delphi exercise alone cannot suffice for item reduction. Also, validation prospectively in SSc patients and diseases that mimic SSc is needed in order to calculate sensitivity and specificity of future criteria. FAU - Coulter, Corrine AU - Coulter C AD - School of Medicine and Dentistry, Western University, London, ON, Canada. corrine.coulter@gmail.com FAU - Baron, Murray AU - Baron M FAU - Pope, Janet E AU - Pope JE LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130402 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Cluster Analysis MH - Consensus MH - Databases, Factual MH - *Delphi Technique MH - Diagnosis-Related Groups/*standards MH - Esophageal Motility Disorders/classification/diagnosis MH - Humans MH - Raynaud Disease/classification/diagnosis MH - Rheumatology/*standards MH - Scleroderma, Systemic/*classification/*diagnosis MH - Sensitivity and Specificity MH - Skin Ulcer/classification/diagnosis MH - Telangiectasis/classification/diagnosis EDAT- 2013/04/06 06:00 MHDA- 2013/09/11 06:00 CRDT- 2013/04/06 06:00 PHST- 2012/07/29 00:00 [received] PHST- 2012/10/25 00:00 [accepted] PHST- 2013/04/06 06:00 [entrez] PHST- 2013/04/06 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 6335 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):24-30. Epub 2013 Apr 2. PMID- 22275160 OWN - NLM STAT- MEDLINE DCOM- 20120703 LR - 20250529 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 64 IP - 6 DP - 2012 Jun TI - Efficacy of Rho kinase inhibitor fasudil in secondary Raynaud's phenomenon. PG - 925-9 LID - 10.1002/acr.21622 [doi] AB - OBJECTIVE: The RhoA/Rho kinase pathway plays a pivotal role in cold-induced vasoconstriction, vascular smooth muscle cells function, and vascular homeostasis. This study evaluates the efficacy of fasudil, a RhoA/Rho kinase inhibitor, to reverse cold-induced vasospasm in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc; scleroderma). METHODS: This is a single-center, double-blind, placebo-controlled, randomized, 3-period crossover study of oral fasudil (40 mg or 80 mg) or placebo administered 2 hours before a standardized cold challenge. The fall in skin temperature after the cold challenge and time to recover 50% and 70% of prechallenge digital skin temperature were used as primary outcomes. Digital blood flow assessed by laser Doppler, time to minimum skin temperature, and rate of skin cooling were also measured. RESULTS: A total of 17 patients with SSc and RP completed the study. After the cold challenge, skin temperatures and the average time (minutes) to recover 50% (7.9 minutes for placebo, 7.5 minutes for fasudil 40 mg, and 8.2 minutes for fasudil 80 mg; P = 0.791) and 70% (18.2 minutes for placebo, 15.0 minutes for fasudil 40 mg, and 17.1 minutes for fasudil 80 mg; P = 0.654) of prechallenge skin temperature were not significantly different across the 3 groups. The digital blood flow measurements were higher in fasudil-treated groups than placebo, but differences were not significant (P = 0.693). CONCLUSION: Fasudil administered at a single oral dose of 40 mg or 80 mg was not associated with significant benefit in terms of the skin temperature recovery time and the digital blood flow after the cold challenge. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Fava, Andrea AU - Fava A AD - Division of Rheumatology, JohnsHopkins University School of Medicine, 5200 Eastern Avenue, Baltimore, MD 21224, USA. FAU - Wung, Peter K AU - Wung PK FAU - Wigley, Fredrick M AU - Wigley FM FAU - Hummers, Laura K AU - Hummers LK FAU - Daya, Natalie R AU - Daya NR FAU - Ghazarian, Sharon R AU - Ghazarian SR FAU - Boin, Francesco AU - Boin F LA - eng GR - K23 AR055667/AR/NIAMS NIH HHS/United States GR - AR-055667/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20120124 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 84477-87-2 (1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine) RN - EC 2.7.11.1 (rho-Associated Kinases) RN - Q0CH43PGXS (fasudil) SB - IM MH - 1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine/administration & dosage/*analogs & derivatives/pharmacology/therapeutic use MH - Administration, Oral MH - Adult MH - Aged MH - Cross-Over Studies MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/*etiology MH - Regional Blood Flow/drug effects MH - Scleroderma, Systemic/*complications MH - Skin/blood supply MH - Skin Temperature/drug effects MH - Treatment Outcome MH - rho-Associated Kinases/*antagonists & inhibitors PMC - PMC3343172 MID - NIHMS351521 EDAT- 2012/01/26 06:00 MHDA- 2012/07/04 06:00 PMCR- 2013/06/01 CRDT- 2012/01/26 06:00 PHST- 2012/01/26 06:00 [entrez] PHST- 2012/01/26 06:00 [pubmed] PHST- 2012/07/04 06:00 [medline] PHST- 2013/06/01 00:00 [pmc-release] AID - 10.1002/acr.21622 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 Jun;64(6):925-9. doi: 10.1002/acr.21622. Epub 2012 Jan 24. PMID- 28401298 OWN - NLM STAT- MEDLINE DCOM- 20180919 LR - 20260127 IS - 1432-1246 (Electronic) IS - 0340-0131 (Print) IS - 0340-0131 (Linking) VI - 90 IP - 7 DP - 2017 Oct TI - Neurovascular hand symptoms in relation to cold exposure in northern Sweden: a population-based study. PG - 587-595 LID - 10.1007/s00420-017-1221-3 [doi] AB - PURPOSE: To describe the self-reported ambient cold exposure in northern Sweden and to relate the level of cumulative cold exposure to the occurrence of sensory and vascular hand symptoms. We hypothesize that cold exposure is positively related to reporting such symptoms. METHODS: A questionnaire about cold exposure and related symptoms was sent out to 35,144 subjects aged 18-70 years and living in northern Sweden. RESULTS: A total of 12,627 out of 35,144 subjects returned the questionnaire (response rate 35.9%). Subjects living in the rural alpine areas reported more extensive cold exposure both during work and leisure time compared to the urbanized coastal regions. Frostbite in the hands was present in 11.4% of men and 7.1% of women, cold sensitivity was present in 9.7 and 14.4%, and Raynaud's phenomenon was present in 11.0% of men and 14.0% of women. There was a positive association between cumulative cold exposure and neurovascular hand symptoms. CONCLUSION: The present study demonstrates that the cold environment in northern Sweden might be an underestimated health risk. Our hypothesis that cold exposure is positively related to reporting of neurovascular hand symptoms was supported by our findings. In addition, such symptoms were common not only in conjunction with an overt cold injury. Our results warrant further study on pathophysiological mechanisms and suggest the need for confirmatory prevalence studies to support national public health planning. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. albin.stjernbrandt@umu.se. AD - Occupational and Environmental Medicine, University Hospital of Umeå, 901 85, Umeå, Sweden. albin.stjernbrandt@umu.se. FAU - Björ, Bodil AU - Björ B AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. AD - Occupational and Environmental Medicine, University Hospital of Umeå, 901 85, Umeå, Sweden. FAU - Andersson, Martin AU - Andersson M AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. AD - Occupational and Environmental Medicine, University Hospital of Umeå, 901 85, Umeå, Sweden. FAU - Burström, Lage AU - Burström L AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Liljelind, Ingrid AU - Liljelind I AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. AD - Occupational and Environmental Medicine, University Hospital of Umeå, 901 85, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Lundström, Ronnie AU - Lundström R AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. AD - Department of Radiation Sciences, Umeå University, 901 87, Umeå, Sweden. FAU - Wahlström, Jens AU - Wahlström J AD - Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. AD - Occupational and Environmental Medicine, University Hospital of Umeå, 901 85, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20170411 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Aged MH - Arctic Regions/epidemiology MH - Cold Climate/*adverse effects MH - Cold Injury/*epidemiology MH - Comorbidity MH - Environmental Exposure/*adverse effects MH - Female MH - Frostbite/epidemiology MH - *Hand MH - Health Status MH - Humans MH - *Leisure Activities MH - Male MH - Middle Aged MH - Occupational Exposure/adverse effects MH - Raynaud Disease/epidemiology MH - Residence Characteristics/statistics & numerical data MH - Sex Distribution MH - Smoking/epidemiology MH - Sweden/epidemiology MH - Young Adult PMC - PMC5583276 OTO - NOTNLM OT - Cold exposure OT - Cold sensitivity OT - Frostbite OT - Hand OT - Raynaud’s phenomenon OT - Sweden COIS- CONFLICT OF INTEREST: The authors declare that they have no conflict of interest. INFORMED CONSENT: Informed consent was obtained from all individual participants included in the study. EDAT- 2017/04/13 06:00 MHDA- 2018/09/20 06:00 PMCR- 2017/04/11 CRDT- 2017/04/13 06:00 PHST- 2016/06/14 00:00 [received] PHST- 2017/04/06 00:00 [accepted] PHST- 2017/04/13 06:00 [pubmed] PHST- 2018/09/20 06:00 [medline] PHST- 2017/04/13 06:00 [entrez] PHST- 2017/04/11 00:00 [pmc-release] AID - 10.1007/s00420-017-1221-3 [pii] AID - 1221 [pii] AID - 10.1007/s00420-017-1221-3 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2017 Oct;90(7):587-595. doi: 10.1007/s00420-017-1221-3. Epub 2017 Apr 11. PMID- 19445801 OWN - NLM STAT- MEDLINE DCOM- 20160422 LR - 20250623 IS - 1752-8526 (Electronic) VI - 2008 DP - 2008 Sep 26 TI - Raynaud's phenomenon (secondary). LID - 1125 [pii] AB - INTRODUCTION: Raynaud's phenomenon is episodic vasospasm of the peripheral vessels, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia, and, rarely, ulceration of the fingers and toes. It presents as episodic colour changes of the digits, usually in response to cold exposure or stress. The classic triphasic colour change is white (ischaemia), then blue (deoxygenation), then red (reperfusion). Raynaud's phenomenon can be primary (idiopathic) or secondary to several different conditions and causes. This review deals with secondary Raynaud's phenomenon. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of self-help measures for secondary Raynaud's phenomenon? What are the effects of drug treatments for secondary Raynaud's phenomenon? We searched: Medline, Embase, The Cochrane Library, and other important databases up to May 2007 (BMJ Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found 25 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review we present information relating to the effectiveness and safety of the following interventions: alpha-blockers; angiotensin-converting enzyme (ACE) inhibitors; angiotensin II receptor antagonists; antithrombotics/inhibitors of platelet aggregation; biofeedback; calcium channel blockers; endothelin-1 receptor anatagonists; glyceryl trinitrate (transdermal); hand exercises; inositol nicotinate; moxisylyte; nafitidrofuryl oxylate; phosphodiesterase inhibitors; prostaglandins (oral, intravenous); relaxation therapy; serotonin reuptake inhibitors SRIs; smoking cessation; and warming hands and feet. FAU - Herrick, Ariane AU - Herrick A AD - Rheumatic Diseases Centre, University of Manchester, Salford, UK. LA - eng PT - Journal Article PT - Systematic Review DEP - 20080926 PL - England TA - BMJ Clin Evid JT - BMJ clinical evidence JID - 101294314 RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Receptor, Endothelin A) SB - IM MH - Administration, Oral MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Endothelin Receptor Antagonists/therapeutic use MH - Humans MH - Phosphodiesterase Inhibitors/therapeutic use MH - *Raynaud Disease/drug therapy MH - *Receptor, Endothelin A PMC - PMC2907943 EDAT- 2008/01/01 00:00 MHDA- 2016/04/23 06:00 PMCR- 2010/09/26 CRDT- 2009/05/19 09:00 PHST- 2009/05/19 09:00 [entrez] PHST- 2008/01/01 00:00 [pubmed] PHST- 2016/04/23 06:00 [medline] PHST- 2010/09/26 00:00 [pmc-release] AID - 1125 [pii] PST - epublish SO - BMJ Clin Evid. 2008 Sep 26;2008:1125. PMID- 28426903 OWN - NLM STAT- MEDLINE DCOM- 20170821 LR - 20220410 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 69 IP - 8 DP - 2017 Aug TI - The Therapeutic Efficacy of Botulinum Toxin in Treating Scleroderma-Associated Raynaud's Phenomenon: A Randomized, Double-Blind, Placebo-Controlled Clinical Trial. PG - 1661-1669 LID - 10.1002/art.40123 [doi] AB - OBJECTIVE: To assess the therapeutic efficacy of local injections of botulinum toxin type A (Btx-A) in improving blood flow to the hands of patients with Raynaud's phenomenon (RP) secondary to scleroderma. METHODS: In this randomized, double-blind, placebo-controlled clinical trial, patients with scleroderma-associated RP received Btx-A (50 units in 2.5 ml sterile saline) in one randomly selected hand and sterile saline (2.5 ml) in the opposite hand. Follow-up at 1 and 4 months postinjection included laser Doppler imaging of hands, patient-reported outcomes, and physical examination. We compared outcomes using paired t-tests and population-average generalized models with generalized estimating equations. RESULTS: Of 40 patients enrolled, 25 had limited scleroderma and 15 had diffuse scleroderma. From baseline to 1-month follow-up, there was a greater reduction in average blood flow in Btx-A-treated hands compared to placebo-treated hands. The model estimated that this difference was statistically significant (average difference -30.08 flux units [95% confidence interval -56.19, -3.98], P for interaction = 0.024). This difference was mainly influenced by patients with longstanding RP and diffuse scleroderma. Change in blood flow at 4-month follow-up was not significantly different between groups. Clinical measures (QuickDASH, McCabe Cold Sensitivity Score, pain on a visual analog scale, and Raynaud's Condition Score) improved slightly for Btx-A-treated hands. CONCLUSION: Our laboratory-based laser Doppler imaging flow data do not support using Btx-A to treat RP in all scleroderma patients. The secondary clinical outcomes suggest some positive effect, but its clinical meaningfulness is questionable. The role of Btx-A in treating RP should be further studied with more homogeneous patient populations and in unique clinical situations such as acute digital ischemia. CI - © 2017, American College of Rheumatology. FAU - Bello, Ricardo J AU - Bello RJ AD - Johns Hopkins University, Baltimore, Maryland. FAU - Cooney, Carisa M AU - Cooney CM AD - Johns Hopkins University, Baltimore, Maryland. FAU - Melamed, Eitan AU - Melamed E AD - Johns Hopkins University, Baltimore, Maryland. FAU - Follmar, Keith AU - Follmar K AD - Johns Hopkins University, Baltimore, Maryland. FAU - Yenokyan, Gayane AU - Yenokyan G AD - Johns Hopkins University, Baltimore, Maryland. FAU - Leatherman, Gwendolyn AU - Leatherman G AD - Johns Hopkins University, Baltimore, Maryland. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University, Baltimore, Maryland. FAU - Wigley, Fredrick M AU - Wigley FM AD - Johns Hopkins University, Baltimore, Maryland. FAU - Hummers, Laura K AU - Hummers LK AD - Johns Hopkins University, Baltimore, Maryland. FAU - Lifchez, Scott D AU - Lifchez SD AD - Johns Hopkins University, Baltimore, Maryland. LA - eng SI - ClinicalTrials.gov/NCT02165111 SI - ClinicalTrials.gov/NCT02165111 GR - UL1 TR001079/TR/NCATS NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20170626 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*therapeutic use MH - Double-Blind Method MH - Female MH - Hand/blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Pain Measurement MH - Patient Reported Outcome Measures MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Diffuse/complications/drug therapy MH - Scleroderma, Limited/complications/drug therapy MH - Treatment Outcome MH - Young Adult PMC - PMC5529251 MID - NIHMS866973 EDAT- 2017/04/21 06:00 MHDA- 2017/08/22 06:00 PMCR- 2018/08/01 CRDT- 2017/04/21 06:00 PHST- 2016/11/11 00:00 [received] PHST- 2017/04/06 00:00 [accepted] PHST- 2017/04/21 06:00 [pubmed] PHST- 2017/08/22 06:00 [medline] PHST- 2017/04/21 06:00 [entrez] PHST- 2018/08/01 00:00 [pmc-release] AID - 10.1002/art.40123 [doi] PST - ppublish SO - Arthritis Rheumatol. 2017 Aug;69(8):1661-1669. doi: 10.1002/art.40123. Epub 2017 Jun 26. PMID- 18095014 OWN - NLM STAT- MEDLINE DCOM- 20080822 LR - 20211020 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 7 DP - 2008 Jul TI - Manganese superoxide dismutase Ala-9Val mitochondrial targeting sequence polymorphism in systemic lupus erythematosus in Poland. PG - 827-31 AB - Systemic lupus erythematosus (SLE) is a chronic and progressive autoimmune disease in which reactive oxygen species contribute to pathogenesis. We analysed the distribution of manganese superoxide dismutase (MnSOD2) 47C>T (Ala-9Val) functional polymorphic variants within the mitochondrial targeting sequence in SLE patients (n = 102) and controls (n = 199). We did not find significant differences in the distribution of MnSOD2 47C>T polymorphic variants in SLE patients and controls. However, we found that MnSOD2 Val/Val genotype (recessive model) showed a significant association with Raynaud's phenomenon, odds ratio (OR) = 12.000 [95% confidence interval (CI) = 2.315-62.193], p = 0.0015. We also found that the MnSOD2 Val/Val genotype contributes to immunologic manifestations, OR = 2.957 (95% CI = 1.207-7.243), p = 0.0222, and anti-dsDNA antibody presence OR = 3.365 (95% CI = 1.364-8.304), p = 0.0107, in patients. Our observations indicate that MnSOD2 Val/Val variant can be linked to some clinical manifestations in patients with SLE. FAU - Sobkowiak, Adam AU - Sobkowiak A AD - Department of Biochemistry and Molecular Biology, Poznan University of Medical Sciences, 6 Swiecickiego St., 60-781 Poznań, Poland. FAU - Lianeri, Margarita AU - Lianeri M FAU - Wudarski, Mariusz AU - Wudarski M FAU - Lacki, Jan K AU - Lacki JK FAU - Jagodziński, Paweł P AU - Jagodziński PP LA - eng PT - Journal Article DEP - 20071219 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - EC 1.15.1.1 (Superoxide Dismutase) SB - IM MH - Adult MH - Autoantibodies/*genetics/metabolism MH - Case-Control Studies MH - Female MH - Genetic Predisposition to Disease/genetics MH - Humans MH - Lupus Erythematosus, Systemic/*genetics MH - Middle Aged MH - Poland MH - Polymorphism, Single Nucleotide/*genetics MH - Raynaud Disease/*genetics MH - Superoxide Dismutase/*genetics EDAT- 2007/12/21 09:00 MHDA- 2008/08/23 09:00 CRDT- 2007/12/21 09:00 PHST- 2007/08/26 00:00 [received] PHST- 2007/11/09 00:00 [accepted] PHST- 2007/11/01 00:00 [revised] PHST- 2007/12/21 09:00 [pubmed] PHST- 2008/08/23 09:00 [medline] PHST- 2007/12/21 09:00 [entrez] AID - 10.1007/s10067-007-0796-6 [doi] PST - ppublish SO - Clin Rheumatol. 2008 Jul;27(7):827-31. doi: 10.1007/s10067-007-0796-6. Epub 2007 Dec 19. PMID- 26946215 OWN - NLM STAT- MEDLINE DCOM- 20170703 LR - 20260127 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 68 IP - 12 DP - 2016 Dec TI - Epidemiology of Mixed Connective Tissue Disease, 1985-2014: A Population-Based Study. PG - 1843-1848 LID - 10.1002/acr.22872 [doi] AB - OBJECTIVE: To characterize the epidemiology of mixed connective tissue disease (MCTD) from 1983 to 2014. METHODS: An inception cohort of patients with incident MCTD in 1985-2014 in Olmsted County, Minnesota was identified based on comprehensive individual medical record review. Diagnosis of MCTD required fulfillment of at least 1 of the 4 widely accepted diagnostic criteria without fulfillment of classification criteria for other connective tissue diseases. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality. RESULTS: A total of 50 incident cases of MCTD were identified (mean age 48.1 years and 84% were female). The annual incidence of MCTD was 1.9 per 100,000 population. Raynaud's phenomenon was the most common initial symptoms (50%), followed by arthralgia (30%) and swollen hands (16%). The diagnosis was frequently delayed with the median time from first symptom to fulfillment of criteria of 3.6 years. At fulfillment of criteria, arthralgia was the most prevalent manifestation (86%), followed by Raynaud's phenomenon (80%), swollen hands (64%), leukopenia/lymphopenia (44%), and heartburn (38%). Evolution to other connective tissue occurred infrequently with a 10-year rate of evolution of 8.5% and 6.3% for systemic lupus erythematosus and systemic sclerosis, respectively. The overall mortality was not different from the general population with a standardized mortality ratio of 1.1 (95% confidence interval 0.4-2.6). CONCLUSION: This study was the first population-based study of MCTD to provide a complete picture of epidemiology and clinical characteristics of MCTD. MCTD occurred in about 2 persons per 100,000 per year. Evolution to other connective diseases occurred infrequently and the mortality was not affected. CI - © 2016, American College of Rheumatology. FAU - Ungprasert, Patompong AU - Ungprasert P AD - Mayo Clinic, Rochester, Minnesota. FAU - Crowson, Cynthia S AU - Crowson CS AD - Mayo Clinic, Rochester, Minnesota. FAU - Chowdhary, Vaidehi R AU - Chowdhary VR AD - Mayo Clinic, Rochester, Minnesota. FAU - Ernste, Floranne C AU - Ernste FC AD - Mayo Clinic, Rochester, Minnesota. FAU - Moder, Kevin G AU - Moder KG AD - Mayo Clinic, Rochester, Minnesota. FAU - Matteson, Eric L AU - Matteson EL AD - Mayo Clinic, Rochester, Minnesota. LA - eng GR - R01 AG034676/AG/NIA NIH HHS/United States GR - R01 AR030582/AR/NIAMS NIH HHS/United States GR - UL1 TR000135/TR/NCATS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20161001 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Arthralgia/epidemiology/etiology MH - Cohort Studies MH - Edema/epidemiology/etiology MH - Female MH - Hand MH - Heartburn/epidemiology/etiology MH - Humans MH - Incidence MH - Leukopenia/epidemiology/etiology MH - Male MH - Middle Aged MH - Minnesota/epidemiology MH - Mixed Connective Tissue Disease/complications/*epidemiology MH - Raynaud Disease/epidemiology/etiology MH - Time Factors PMC - PMC5426802 MID - NIHMS854207 COIS- Conflict of interest: All authors have disclosed no conflict of interest. EDAT- 2016/03/08 06:00 MHDA- 2017/07/04 06:00 PMCR- 2017/12/01 CRDT- 2016/03/07 06:00 PHST- 2015/12/24 00:00 [received] PHST- 2016/02/05 00:00 [revised] PHST- 2016/02/23 00:00 [accepted] PHST- 2016/03/08 06:00 [pubmed] PHST- 2017/07/04 06:00 [medline] PHST- 2016/03/07 06:00 [entrez] PHST- 2017/12/01 00:00 [pmc-release] AID - 10.1002/acr.22872 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2016 Dec;68(12):1843-1848. doi: 10.1002/acr.22872. Epub 2016 Oct 1. PMID- 36753288 OWN - NLM STAT- MEDLINE DCOM- 20230314 LR - 20231129 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 48 IP - 4 DP - 2023 Apr TI - State of the art review: The management of chronic vascular disorders in the hand and upper limb. PG - 295-302 LID - 10.1177/17531934221145498 [doi] AB - This review article summarizes the basic principles of vascular anatomy, physiology, diagnostic work-up and treatment for patients with nontraumatic upper extremity vascular disorders. Vascular disorders can be considered vasospastic or occlusive. The most commonly encountered vasospastic condition is Raynaud's Phenomenon secondary to scleroderma. While historically this has been managed medically with vasodilators, more advanced cases can benefit from surgical treatment to improve blood flow and minimize tissue loss, with compelling evidence that earlier surgical intervention can modify disease process and should be considered. Occlusive disease can present as aneurysm or thrombosis and often requires surgical treatment with resection of the occluded segment with or without vascular reconstruction. In advanced atherosclerotic disease or end stage ischemia, arterialization of the venous system can be considered to avoid more proximal amputations. FAU - Titan, Ashley L AU - Titan AL AUID- ORCID: 0000-0002-5461-9832 AD - Department of Surgery, Division of Plastic Surgery, Stanford University Hospital, Stanford, CA, USA. FAU - Chang, James AU - Chang J AD - Department of Surgery, Division of Plastic Surgery, Stanford University Hospital, Stanford, CA, USA. FAU - Megerle, Kai AU - Megerle K AD - Centre for Hand Surgery, Microsurgery and Plastic Surgery, Schön Clinic, Munich, Germany. FAU - Murray, Peter AU - Murray P AD - Department of Orthopaedic Surgery, Mayo Clinic, Jacksonville Florida, USA. FAU - Hammert, Warren AU - Hammert W AUID- ORCID: 0000-0001-7767-4124 AD - Department of Orthopaedic Surgery, Duke University, Durham, North Carolina, USA. LA - eng PT - Journal Article PT - Review DEP - 20230208 PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 SB - IM CIN - J Hand Surg Eur Vol. 2023 Dec;48(11):1246-1248. doi: 10.1177/17531934231185094. PMID: 37401124 MH - Humans MH - *Hand MH - *Raynaud Disease MH - Ischemia/surgery OTO - NOTNLM OT - Vascular disorders OT - occlusive disease OT - vasospastic disease OT - vessel reconstruction EDAT- 2023/02/09 06:00 MHDA- 2023/03/15 06:00 CRDT- 2023/02/08 11:42 PHST- 2023/02/09 06:00 [pubmed] PHST- 2023/03/15 06:00 [medline] PHST- 2023/02/08 11:42 [entrez] AID - 10.1177/17531934221145498 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2023 Apr;48(4):295-302. doi: 10.1177/17531934221145498. Epub 2023 Feb 8. PMID- 37828025 OWN - NLM STAT- MEDLINE DCOM- 20231102 LR - 20250530 IS - 2041-1723 (Electronic) IS - 2041-1723 (Linking) VI - 14 IP - 1 DP - 2023 Oct 12 TI - ADRA2A and IRX1 are putative risk genes for Raynaud's phenomenon. PG - 6156 LID - 10.1038/s41467-023-41876-5 [doi] LID - 6156 AB - Raynaud's phenomenon (RP) is a common vasospastic disorder that causes severe pain and ulcers, but despite its high reported heritability, no causal genes have been robustly identified. We conducted a genome-wide association study including 5,147 RP cases and 439,294 controls, based on diagnoses from electronic health records, and identified three unreported genomic regions associated with the risk of RP (p < 5 × 10(-8)). We prioritized ADRA2A (rs7090046, odds ratio (OR) per allele: 1.26; 95%-CI: 1.20-1.31; p < 9.6 × 10(-27)) and IRX1 (rs12653958, OR: 1.17; 95%-CI: 1.12-1.22, p < 4.8 × 10(-13)) as candidate causal genes through integration of gene expression in disease relevant tissues. We further identified a likely causal detrimental effect of low fasting glucose levels on RP risk (r(G) = -0.21; p-value = 2.3 × 10(-3)), and systematically highlighted drug repurposing opportunities, like the antidepressant mirtazapine. Our results provide the first robust evidence for a strong genetic contribution to RP and highlight a so far underrated role of α(2A)-adrenoreceptor signalling, encoded at ADRA2A, as a possible mechanism for hypersensitivity to catecholamine-induced vasospasms. CI - © 2023. Springer Nature Limited. FAU - Hartmann, Sylvia AU - Hartmann S AD - Computational Medicine, Berlin Institute of Health at Charité-Universitätsmedizin Berlin, Berlin, Germany. FAU - Yasmeen, Summaira AU - Yasmeen S AUID- ORCID: 0000-0001-5470-2081 AD - Computational Medicine, Berlin Institute of Health at Charité-Universitätsmedizin Berlin, Berlin, Germany. FAU - Jacobs, Benjamin M AU - Jacobs BM AUID- ORCID: 0000-0002-6023-6010 AD - Preventive Neurology Unit, Wolfson Institute of Population Health, Queen Mary University of London, London, UK. FAU - Denaxas, Spiros AU - Denaxas S AD - Institute of Health Informatics, University College London, London, UK. AD - Health Data Research UK, London, UK. AD - British Heart Foundation Data Science Centre, London, UK. AD - National Institute of Health Research University College London Hospitals Biomedical Research Centre, London, UK. FAU - Pirmohamed, Munir AU - Pirmohamed M AUID- ORCID: 0000-0002-7534-7266 AD - Department of Pharmacology and Therapeutics, The Wolfson Centre for Personalised Medicine, University Liverpool, Liverpool, UK. FAU - Gamazon, Eric R AU - Gamazon ER AUID- ORCID: 0000-0003-4204-8734 AD - Division of Genetic Medicine and Vanderbilt Genetics Institute, Vanderbilt University Medical Center, Nashville, TN, 37232, USA. FAU - Caulfield, Mark J AU - Caulfield MJ AUID- ORCID: 0000-0001-9295-3594 AD - William Harvey Research Institute, Queen Mary University of London, London, UK. CN - Genes & Health Research Team FAU - Hemingway, Harry AU - Hemingway H AUID- ORCID: 0000-0003-2279-0624 AD - Institute of Health Informatics, University College London, London, UK. AD - Health Data Research UK, London, UK. AD - National Institute of Health Research University College London Hospitals Biomedical Research Centre, London, UK. FAU - Pietzner, Maik AU - Pietzner M AUID- ORCID: 0000-0003-3437-9963 AD - Computational Medicine, Berlin Institute of Health at Charité-Universitätsmedizin Berlin, Berlin, Germany. maik.pietzner@bih-charite.de. AD - MRC Epidemiology Unit, University of Cambridge, Cambridge, UK. maik.pietzner@bih-charite.de. AD - Precision Healthcare University Research Institute, Queen Mary University of London, London, UK. maik.pietzner@bih-charite.de. FAU - Langenberg, Claudia AU - Langenberg C AUID- ORCID: 0000-0002-5017-7344 AD - Computational Medicine, Berlin Institute of Health at Charité-Universitätsmedizin Berlin, Berlin, Germany. claudia.langenberg@bih-charite.de. AD - MRC Epidemiology Unit, University of Cambridge, Cambridge, UK. claudia.langenberg@bih-charite.de. AD - Precision Healthcare University Research Institute, Queen Mary University of London, London, UK. claudia.langenberg@bih-charite.de. LA - eng GR - WT102627/WT_/Wellcome Trust/United Kingdom GR - NIHR202349/DH_/Department of Health/United Kingdom GR - MR/L006758/1/MRC_/Medical Research Council/United Kingdom GR - MC_PC_20030/MRC_/Medical Research Council/United Kingdom GR - WT210561/WT_/Wellcome Trust/United Kingdom GR - MR/V033867/1/MRC_/Medical Research Council/United Kingdom GR - M009017/MRC_/Medical Research Council/United Kingdom GR - MR/X009777/1/MRC_/Medical Research Council/United Kingdom GR - MC_PC_20051/MRC_/Medical Research Council/United Kingdom GR - 210561/Z/18/Z/WT_/Wellcome Trust/United Kingdom GR - MR/V028766/1/MRC_/Medical Research Council/United Kingdom GR - MC_UU_00006/1/MRC_/Medical Research Council/United Kingdom GR - MC_PC_20059/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231012 PL - England TA - Nat Commun JT - Nature communications JID - 101528555 RN - 0 (IRX1 protein, human) RN - 0 (Transcription Factors) RN - 0 (Homeodomain Proteins) RN - 0 (ADRA2A protein, human) RN - 0 (Receptors, Adrenergic, alpha-2) SB - IM MH - Humans MH - *Genome-Wide Association Study MH - Ulcer MH - *Raynaud Disease/genetics/complications MH - Pain/complications MH - Transcription Factors/genetics MH - Homeodomain Proteins MH - Receptors, Adrenergic, alpha-2/genetics PMC - PMC10570309 COIS- Mu. P. has received partnership funding for the following: MRC Clinical Pharmacology Training Scheme (co-funded by MRC and Roche, UCB, Eli Lilly, and Novartis); and a Ph.D. studentship jointly funded by EPSRC and Astra Zeneca. Mu. P. also has unrestricted educational grant support for the UK Pharmacogenetics and Stratified Medicine Network from Bristol-Myers Squibb. Mu. P. has developed an HLA genotyping panel with MC Diagnostics, but does not benefit financially from this. He is part of the IMI Consortium ARDAT (www.ardat.org). None of the funding outlined above is related to the current paper. None of the other authors have competing interests. EDAT- 2023/10/13 00:43 MHDA- 2023/11/02 12:42 PMCR- 2023/10/12 CRDT- 2023/10/12 23:17 PHST- 2023/01/06 00:00 [received] PHST- 2023/09/21 00:00 [accepted] PHST- 2023/11/02 12:42 [medline] PHST- 2023/10/13 00:43 [pubmed] PHST- 2023/10/12 23:17 [entrez] PHST- 2023/10/12 00:00 [pmc-release] AID - 10.1038/s41467-023-41876-5 [pii] AID - 41876 [pii] AID - 10.1038/s41467-023-41876-5 [doi] PST - epublish SO - Nat Commun. 2023 Oct 12;14(1):6156. doi: 10.1038/s41467-023-41876-5. PMID- 27839740 OWN - NLM STAT- MEDLINE DCOM- 20180226 LR - 20250623 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 46 IP - 5 DP - 2017 Apr TI - Systematic review of systemic sclerosis-specific instruments for the EULAR Outcome Measures Library: An evolutional database model of validated patient-reported outcomes. PG - 609-614 LID - S0049-0172(16)30343-2 [pii] LID - 10.1016/j.semarthrit.2016.10.002 [doi] AB - OBJECTIVES: The EULAR Outcome Measures Library (OML) is a freely available database of validated patient-reported outcomes (PROs). The aim of this study was to provide a comprehensive review of validated PROs specifically developed for systemic sclerosis (SSc) to feed the EULAR OML. METHODS: A sensitive search was developed in Medline and Embase to identify all validation studies, cohort studies, reviews, or meta-analyses in which the objective were the development or validation of specific PROs evaluating organ involvement, disease activity or damage in SSc. A reviewer screened title and abstracts, selected the studies, and collected data concerning validation using ad hoc forms based on the COSMIN checklist. RESULTS: From 13,140 articles captured, 74 met the predefined criteria. After excluding two instruments as they were unavailable in English the selected 23 studies provided information on seven SSc-specific PROs on different SSc domains: burden of illness (symptom burden index), functional status (Scleroderma Assessment Questionnaire), functional ability (scleroderma Functional Score), Raynaud's phenomenon (Raynaud's condition score), mouth involvement (Mouth Handicap in SSc), gastro-intestinal involvement (University of California Los Angeles-Scleroderma Clinical Trial Consortium Gastro-Intestinal tract 2.0), and skin involvement (skin self-assessment). Each of them is partially validated and has different psychometric requirements. CONCLUSIONS: Seven SSc-specific PROs have a minimum validation and were included in the EULAR OML. Further development in the area of disease-specific PROs in SSc is warranted. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Clinical Sciences and Community Health, Università degli Studi di Milano, P.zza Cardinal Ferrari 1, 20122 Milano, Italy. Electronic address: francesca.ingegnoli@unimi.it. FAU - Carmona, Loreto AU - Carmona L AD - Instituto de Salud Musculoesquelética, InMusc, Madrid, Spain. FAU - Castrejon, Isabel AU - Castrejon I AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milano, Italy. LA - eng PT - Journal Article PT - Systematic Review DEP - 20161013 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM EIN - Semin Arthritis Rheum. 2017 Dec;47(3):e13. doi: 10.1016/j.semarthrit.2017.06.002. PMID: 28676176 MH - Cross-Sectional Studies MH - Databases, Factual MH - Humans MH - Longitudinal Studies MH - Meta-Analysis as Topic MH - *Patient Reported Outcome Measures MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/physiopathology MH - Review Literature as Topic MH - Scleroderma, Systemic/*physiopathology MH - Self Report MH - Severity of Illness Index MH - Validation Studies as Topic OTO - NOTNLM OT - Functional status OT - Mouth Handicap in Systemic Sclerosis OT - Patient-reported outcomes OT - Questionnaire OT - Raynaud’s condition score OT - Scleroderma OT - Scleroderma Assessment Questionnaire OT - Scleroderma Functional Score OT - Self-assessment OT - Skin self-assessment OT - Symptom burden index OT - Systemic sclerosis OT - University of California Los Angeles-Scleroderma Clinical Trial Consortium Gastro-Intestinal tract 2.0 EDAT- 2016/11/15 06:00 MHDA- 2018/02/27 06:00 CRDT- 2016/11/15 06:00 PHST- 2016/05/23 00:00 [received] PHST- 2016/09/30 00:00 [revised] PHST- 2016/10/07 00:00 [accepted] PHST- 2016/11/15 06:00 [pubmed] PHST- 2018/02/27 06:00 [medline] PHST- 2016/11/15 06:00 [entrez] AID - S0049-0172(16)30343-2 [pii] AID - 10.1016/j.semarthrit.2016.10.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2017 Apr;46(5):609-614. doi: 10.1016/j.semarthrit.2016.10.002. Epub 2016 Oct 13. PMID- 19590932 OWN - NLM STAT- MEDLINE DCOM- 20100126 LR - 20181201 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 19 IP - 5 DP - 2009 TI - Long-term beneficial effects of statins on vascular manifestations in patients with systemic sclerosis. PG - 530-5 LID - 10.1007/s10165-009-0199-4 [doi] AB - We conducted a 24-month, open-label trial to evaluate the long-term effects of statins on vascular symptoms in patients with systemic sclerosis (SSc). Ten patients received 10 mg/day atorvastatin, but two dropped out to treat other organ involvement. Raynaud's phenomenon, global measures of health, and psychological scales were assessed in addition to circulating angiogenic factors and endothelial activation/injury markers in eight patients at 0 (pretreatment), 1, 3, 12, and 24 months of treatment. Circulating endothelial progenitor cells (EPCs) were serially quantified by cell sorting and three-color flow cytometry. There were no adverse events. Raynaud's phenomenon improved during atorvastatin treatment, with significant reductions in the Raynaud's Condition Score (P = 0.01) and the patient assessment by visual analog scale (P = 0.0003). SSc-associated upregulation of angiogenic factors and vascular endothelial activation/injury markers were reduced (P < 0.01 for all comparisons). Improvement in these parameters was best at 12 and 24 months of treatment. The EPC number was increased at 1 month of treatment (P < 0.01), but soon dropped below baseline. This pilot study suggests that statins may be beneficial in treating vascular manifestations of SSc, through their pleiotropic effects. However, this treatment did not correct the defect in EPC recruitment. FAU - Kuwana, Masataka AU - Kuwana M AD - Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan. kuwanam@sc.itc.keio.ac.jp FAU - Okazaki, Yuka AU - Okazaki Y FAU - Kaburaki, Junichi AU - Kaburaki J LA - eng PT - Journal Article DEP - 20090710 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Anticholesteremic Agents) RN - 0 (E-Selectin) RN - 0 (Heptanoic Acids) RN - 0 (Pyrroles) RN - 0 (Vascular Cell Adhesion Molecule-1) RN - 0 (Vascular Endothelial Growth Factor A) RN - 103107-01-3 (Fibroblast Growth Factor 2) RN - A0JWA85V8F (Atorvastatin) SB - IM MH - Aged MH - Analysis of Variance MH - Anticholesteremic Agents/therapeutic use MH - Atorvastatin MH - Cell Count MH - Cell Separation MH - Disease Progression MH - E-Selectin/blood MH - Endothelial Cells/cytology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fibroblast Growth Factor 2/blood MH - Flow Cytometry MH - Fluorescent Antibody Technique MH - Health Status MH - Heptanoic Acids/*therapeutic use MH - Humans MH - Immunoprecipitation MH - Middle Aged MH - Prospective Studies MH - Pyrroles/*therapeutic use MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy MH - Severity of Illness Index MH - Stem Cells/cytology MH - Treatment Outcome MH - Vascular Cell Adhesion Molecule-1/blood MH - Vascular Endothelial Growth Factor A/blood EDAT- 2009/07/11 09:00 MHDA- 2010/01/27 06:00 CRDT- 2009/07/11 09:00 PHST- 2009/05/18 00:00 [received] PHST- 2009/06/09 00:00 [accepted] PHST- 2009/07/11 09:00 [entrez] PHST- 2009/07/11 09:00 [pubmed] PHST- 2010/01/27 06:00 [medline] AID - 10.1007/s10165-009-0199-4 [doi] PST - ppublish SO - Mod Rheumatol. 2009;19(5):530-5. doi: 10.1007/s10165-009-0199-4. Epub 2009 Jul 10. PMID- 27228628 OWN - NLM STAT- MEDLINE DCOM- 20160616 LR - 20160527 IS - 1565-1088 (Print) VI - 18 IP - 3-4 DP - 2016 Mar-Apr TI - A New Way of Thinking about Systemic Sclerosis: The Opportunity for a Very Early Diagnosis. PG - 141-3 AB - Systemic sclerosis (SSc) is a heterogeneous chronic autoimmune disease that it is extremely difficult to diagnose in the early phase, resulting in a critical delay in therapy which is often begun when internal organ involvement is already irreversible. The ACR or LeRoy criteria have a low sensitivity for the early phases; these criteria were replaced by the ACR/EULAR 2013 criteria which improved the disease classification. Therefore, the SSc diagnosis may be delayed for several years after the onset of Raynaud's phenomenon (RP) and even after the onset of the first non-RP symptom. RP, antinuclear antibodies (ANA) positivity, and puffy fingers were recently indicated as "red flags" (by the VEDOSS project)--that is, the main elements for suspicion of SSc in the very early phase of the disease. Confirming the diagnosis requires further tests, particularly nailfold videocapillaroscopy and evaluation of specific disease antibodies (anti-centromere and anti-topoisomerase I). In this way, the VEDOSS project identified patients in the very early phase of disease enabling a "window of opportunity" whereby the physician can act with effective drugs to block or at least slow the progression of the disease. The principal challenge in the fight against SSc is to detect valid predictors of disease evolution in order to treat patients in the early stage of disease. While waiting to find valid predictors, a close follow-up of the patients with the VEDOSS red flags is essential, as is a close collaboration between rheumatologists and general practitioners in order to identify all potential SSc patients as soon as possible. FAU - Guiducci, Serena AU - Guiducci S FAU - Bellando-Randone, Silvia AU - Bellando-Randone S FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M LA - eng PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Antibodies, Antinuclear/*immunology MH - DNA Topoisomerases, Type I/immunology MH - Early Diagnosis MH - Early Medical Intervention MH - Humans MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/*immunology MH - *Scleroderma, Systemic/diagnosis/immunology EDAT- 2016/05/28 06:00 MHDA- 2016/06/17 06:00 CRDT- 2016/05/28 06:00 PHST- 2016/05/28 06:00 [entrez] PHST- 2016/05/28 06:00 [pubmed] PHST- 2016/06/17 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2016 Mar-Apr;18(3-4):141-3. PMID- 19147617 OWN - NLM STAT- MEDLINE DCOM- 20090527 LR - 20260128 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 68 IP - 5 DP - 2009 May TI - EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). PG - 620-8 LID - 10.1136/ard.2008.096677 [doi] AB - PURPOSE: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. METHODS: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. RESULTS: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. CONCLUSIONS: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc. FAU - Kowal-Bielecka, O AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Landewé, R AU - Landewé R FAU - Avouac, J AU - Avouac J FAU - Chwiesko, S AU - Chwiesko S FAU - Miniati, I AU - Miniati I FAU - Czirjak, L AU - Czirjak L FAU - Clements, P AU - Clements P FAU - Denton, C AU - Denton C FAU - Farge, D AU - Farge D FAU - Fligelstone, K AU - Fligelstone K FAU - Földvari, I AU - Földvari I FAU - Furst, D E AU - Furst DE FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Seibold, J AU - Seibold J FAU - Silver, R M AU - Silver RM FAU - Takehara, K AU - Takehara K FAU - Toth, B Garay AU - Toth BG FAU - Tyndall, A AU - Tyndall A FAU - Valentini, G AU - Valentini G FAU - van den Hoogen, F AU - van den Hoogen F FAU - Wigley, F AU - Wigley F FAU - Zulian, F AU - Zulian F FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M CN - EUSTAR Co-Authors LA - eng PT - Consensus Statement PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090115 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Evidence-Based Medicine/methods MH - Gastrointestinal Diseases/drug therapy/etiology MH - Humans MH - Hypertension, Pulmonary/drug therapy/etiology MH - Kidney Diseases/drug therapy/etiology MH - Lung Diseases, Interstitial/drug therapy/etiology MH - Raynaud Disease/drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy MH - Treatment Outcome RF - 85 EDAT- 2009/01/17 09:00 MHDA- 2009/05/28 09:00 CRDT- 2009/01/17 09:00 PHST- 2009/01/17 09:00 [entrez] PHST- 2009/01/17 09:00 [pubmed] PHST- 2009/05/28 09:00 [medline] AID - S0003-4967(24)42277-2 [pii] AID - 10.1136/ard.2008.096677 [doi] PST - ppublish SO - Ann Rheum Dis. 2009 May;68(5):620-8. doi: 10.1136/ard.2008.096677. Epub 2009 Jan 15. PMID- 17234652 OWN - NLM STAT- MEDLINE DCOM- 20070619 LR - 20250214 IS - 0003-4967 (Print) IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 66 IP - 6 DP - 2007 Jun TI - Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. PG - 754-63 AB - BACKGROUND: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. AIMS AND METHODS: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. RESULTS: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs lcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. CONCLUSION: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally. FAU - Walker, U A AU - Walker UA AD - Department of Rheumatology, Basle University, Felix Platter Spital, Burgfelderstrasse 101, Basel 4012, Switzerland. ulrich.walker@fps-basel.ch FAU - Tyndall, A AU - Tyndall A FAU - Czirják, L AU - Czirják L FAU - Denton, C AU - Denton C FAU - Farge-Bancel, D AU - Farge-Bancel D FAU - Kowal-Bielecka, O AU - Kowal-Bielecka O FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Bocelli-Tyndall, C AU - Bocelli-Tyndall C FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20070118 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) RN - 0 (Nuclear Proteins) RN - 0 (Scl 70 antigen, human) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adult MH - Age Distribution MH - Age of Onset MH - Aged MH - Autoantibodies/blood MH - Cross-Sectional Studies MH - DNA Topoisomerases, Type I MH - Databases, Factual MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nuclear Proteins/immunology MH - Raynaud Disease/etiology/immunology MH - Risk Assessment MH - Scleroderma, Diffuse/complications/immunology MH - Scleroderma, Limited/complications/immunology MH - Scleroderma, Systemic/*complications/immunology MH - Sex Factors PMC - PMC1954657 COIS- Competing interests: None declared. EDAT- 2007/01/20 09:00 MHDA- 2007/06/20 09:00 PMCR- 2010/06/01 CRDT- 2007/01/20 09:00 PHST- 2007/01/20 09:00 [pubmed] PHST- 2007/06/20 09:00 [medline] PHST- 2007/01/20 09:00 [entrez] PHST- 2010/06/01 00:00 [pmc-release] AID - S0003-4967(24)19259-X [pii] AID - ar62901 [pii] AID - 10.1136/ard.2006.062901 [doi] PST - ppublish SO - Ann Rheum Dis. 2007 Jun;66(6):754-63. doi: 10.1136/ard.2006.062901. Epub 2007 Jan 18. PMID- 33736541 OWN - NLM STAT- MEDLINE DCOM- 20211006 LR - 20211006 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 30 IP - 5 DP - 2021 Apr TI - Identifying additional risk factors for arterial and venous thrombosis among pediatric antiphospholipid antibodies carriers. PG - 828-832 LID - 10.1177/09612033211002256 [doi] AB - BACKGROUND: Antiphospholipid antibodies (aPL) have been extensively reported in children, but investigations into thrombotic risks associated with aPL positivity in pediatric patients is scarce. Positive aPL are not uncommon in pediatric connective tissue diseases (CTD), but identification and management of these patients is challenging due to lack of validated criteria and a paucity of data. In this study, we identify potential additional risk factors for thrombosis in a unique cohort of pediatric aPL positive carriers. METHODS: Retrospective chart review was performed on 491 pediatric patients with CTD seen in our institution from 2001 to 2019. Patients without persistently moderate to high titer aPL at least 12 weeks apart were excluded. Univariate analysis was performed to evaluate correlation between different risk factors and thrombotic events. RESULTS: Seventy-one aPL positive children with underlying CTD are included in this cohort. The majority (87%) are female and of Hispanic ethnicity (56%). Mean age of the cohort at the diagnosis of connective tissue disease is 12.7 (SD 2.6) years, and mean age of first positive aPL is 13.3 (SD 2.5) years. Average length of follow-up is 4.3 (SD 2.5) years. Four (5.6%) patients experienced arterial thrombosis, and 11 (15.5%) had venous thrombosis. Fifty-seven (80.3%) patients did not have any thromboembolic events. Among traditional risk factors and signs of endothelial injury, only Raynaud's phenomena demonstrated significant association with arterial thrombosis (OR = 8.4, 95%CI 1.13-111, P = 0.039), and hypertension or anti-hypertensive use demonstrated significant association with venous thrombosis (OR = 8.387, 95%CI 1.2 - 94, P = 0.02). CONCLUSION: Data from our cohort suggest that Raynaud's phenomenon is a potential predictor of arterial thrombosis while the presence of hypertension or anti-hypertensive medication use is a potential predictor of venous thrombosis in aPL positive pediatric carriers. Further studies investigating pediatric aPL profiles and risk factors for development of thrombosis are needed to help guide clinicians in caring for these challenging patients. FAU - Sloan, Elizabeth AU - Sloan E AD - Division of Pediatric Rheumatology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, USA. AD - Division of Pediatric Rheumatology, Department of Pediatrics, Texas Scottish Rite Hospital for Children, Dallas, USA. AD - Division of Pediatric Rheumatology, Department of Pediatrics, Children's Medical Center, Dallas, USA. FAU - Wright, Tracey AU - Wright T AD - Division of Pediatric Rheumatology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, USA. AD - Division of Pediatric Rheumatology, Department of Pediatrics, Texas Scottish Rite Hospital for Children, Dallas, USA. AD - Division of Pediatric Rheumatology, Department of Pediatrics, Children's Medical Center, Dallas, USA. FAU - Zuo, Yu AU - Zuo Y AUID- ORCID: 0000-0001-6721-7755 AD - Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, USA. LA - eng PT - Journal Article DEP - 20210318 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Antihypertensive Agents) RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Adolescent MH - Antibodies, Antiphospholipid/*blood MH - Antihypertensive Agents/adverse effects/therapeutic use MH - Antiphospholipid Syndrome/complications MH - Arteries/pathology MH - Carrier State/blood MH - Child MH - Connective Tissue Diseases/complications/diagnosis/*immunology/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension/complications/diagnosis/drug therapy/epidemiology MH - Lupus Coagulation Inhibitor/blood MH - Lupus Erythematosus, Systemic/complications MH - Male MH - Raynaud Disease/complications/diagnosis/epidemiology MH - Retrospective Studies MH - Risk Factors MH - Thrombosis/diagnosis/epidemiology/*immunology MH - Venous Thrombosis/epidemiology/*immunology OTO - NOTNLM OT - Thrombosis OT - antiphospholipid syndrome OT - systemic lupus erythematosus EDAT- 2021/03/20 06:00 MHDA- 2021/10/07 06:00 CRDT- 2021/03/19 05:44 PHST- 2021/03/20 06:00 [pubmed] PHST- 2021/10/07 06:00 [medline] PHST- 2021/03/19 05:44 [entrez] AID - 10.1177/09612033211002256 [doi] PST - ppublish SO - Lupus. 2021 Apr;30(5):828-832. doi: 10.1177/09612033211002256. Epub 2021 Mar 18. PMID- 24804712 OWN - NLM STAT- MEDLINE DCOM- 20140730 LR - 20151119 IS - 1463-1741 (Print) IS - 1463-1741 (Linking) VI - 16 IP - 69 DP - 2014 Mar-Apr TI - Raynaud's phenomenon among men and women with noise-induced hearing loss in relation to vibration exposure. PG - 89-94 LID - 10.4103/1463-1741.132087 [doi] AB - Raynaud's phenomenon is characterized by constriction in blood supply to the fingers causing finger blanching, of white fingers (WF) and is triggered by cold. Earlier studies found that workers using vibrating hand-held tools and who had vibration-induced white fingers (VWF) had an increased risk for hearing loss compared with workers without VWF. This study examined the occurrence of Raynaud's phenomenon among men and women with noise-induced hearing loss in relation to vibration exposure. All 342 participants had a confirmed noise-induced hearing loss medico legally accepted as work-related by AFA Insurance. Each subject answered a questionnaire concerning their health status and the kinds of exposures they had at the time when their hearing loss was first discovered. The questionnaire covered types of exposures, discomforts in the hands or fingers, diseases and medications affecting the blood circulation, the use of alcohol and tobacco and for women, the use of hormones and whether they had been pregnant. The participation rate was 41% (n = 133) with 38% (n = 94) for men and 50% (n = 39) for women. 84 men and 36 women specified if they had Raynaud's phenomenon and also if they had used hand-held vibrating machines. Nearly 41% of them had used hand-held vibrating machines and 18% had used vibrating machines at least 2 h each workday. There were 23 men/6 women with Raynaud's phenomenon. 37% reported WF among those participants who were exposed to hand-arm vibration (HAV) and 15% among those not exposed to HAV. Among the participants with hearing loss with daily use of vibrating hand-held tools more than twice as many reports WF compared with participants that did not use vibrating hand-held tools. This could be interpreted as Raynaud's phenomenon could be associated with an increased risk for noise-induced hearing loss. However, the low participation rate limits the generalization of the results from this study. FAU - Pettersson, Hans AU - Pettersson H AD - Umeå University, Department of Public Health and Clinical Medicine, Occupational and Environmental Medicine, Umeå, Sweden. FAU - Burström, Lage AU - Burström L FAU - Nilsson, Tohr AU - Nilsson T LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - India TA - Noise Health JT - Noise & health JID - 9815620 SB - IM MH - Adult MH - Female MH - Hand-Arm Vibration Syndrome/*epidemiology MH - Hearing Loss, Noise-Induced/*epidemiology MH - Humans MH - Male MH - Middle Aged MH - Noise, Occupational/*statistics & numerical data MH - Occupational Diseases/*epidemiology MH - Occupational Exposure/adverse effects/statistics & numerical data MH - Raynaud Disease/*epidemiology MH - Surveys and Questionnaires MH - Sweden/epidemiology MH - Vibration/*adverse effects EDAT- 2014/05/09 06:00 MHDA- 2014/07/31 06:00 CRDT- 2014/05/09 06:00 PHST- 2014/05/09 06:00 [entrez] PHST- 2014/05/09 06:00 [pubmed] PHST- 2014/07/31 06:00 [medline] AID - NoiseHealth_2014_16_69_89_132087 [pii] AID - 10.4103/1463-1741.132087 [doi] PST - ppublish SO - Noise Health. 2014 Mar-Apr;16(69):89-94. doi: 10.4103/1463-1741.132087. PMID- 21229358 OWN - NLM STAT- MEDLINE DCOM- 20120725 LR - 20220321 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 4 DP - 2012 Apr TI - The prevalences of some rheumatic diseases in western Turkey: Havsa study. PG - 895-908 LID - 10.1007/s00296-010-1699-4 [doi] AB - To study the prevalence major rheumatic diseases in western Turkey. This survey was conducted in Havsa which have a total population of 18,771. Physicians and interns visited every household, interviewed face to face a questionnaire about the symptoms of rheumatic disorders. The individuals replied positively to any question were examined at the nearest health center. Those have no objective findings related to any rheumatic diseases were excluded. People could not be clinically diagnosed were asked to come to the hospital for further evaluation. A total 17,835 of 18,771 residents participated. We estimated the prevalence of Behçet's Disease (BD) as 0.019%; ankylosing spondylitis: 0.120%; rheumatoid arthritis: 0.321%; knee osteoarthritis (OA): 5.351%; hand OA: 1.110%; hand and knee OA: 1.958%; total OA: 8.420%; primary Raynaud's: 1.192%; psoriasis: 0.424 %; psoriatic arthritis: 0.050%; rheumatic fever: 0.318%; rheumatic heart disease: 0.200%; inflammatory bowel disease: 0.023%; lupus: 0.059%; gout: 0.018%; systemic sclerosis: 0.022%; juvenile rheumatoid arthritis: 0.032%; temporal arteritis: 0.020%, and familial Mediterranean fever (FMF) as 0.006%. Figures were adjusted for age-sex of the general Turkish population. The prevalence's of BD and FMF are considerably lower in Havsa as compared to other regions in Turkey. FAU - Cakır, Necati AU - Cakır N AD - Division of Rheumatology, Medical Faculty, Trakya University, Edirne, Turkey. opamuk@bidmc.harvard.edu FAU - Pamuk, Ömer Nuri AU - Pamuk ÖN FAU - Derviş, Emine AU - Derviş E FAU - Imeryüz, Neşe AU - Imeryüz N FAU - Uslu, Haşim AU - Uslu H FAU - Benian, Ömer AU - Benian Ö FAU - Elelçi, Edip AU - Elelçi E FAU - Erdem, Genco AU - Erdem G FAU - Sarvan, Fatma Oğuz AU - Sarvan FO FAU - Senocak, Mustafa AU - Senocak M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110113 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Arthritis/*epidemiology MH - Autoimmune Diseases/*epidemiology MH - Behcet Syndrome/*epidemiology MH - Child MH - Familial Mediterranean Fever/*epidemiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Psoriasis/*epidemiology MH - Raynaud Disease/*epidemiology MH - Rheumatic Diseases/*epidemiology MH - Turkey/epidemiology EDAT- 2011/01/14 06:00 MHDA- 2012/07/26 06:00 CRDT- 2011/01/14 06:00 PHST- 2010/07/16 00:00 [received] PHST- 2010/11/21 00:00 [accepted] PHST- 2011/01/14 06:00 [entrez] PHST- 2011/01/14 06:00 [pubmed] PHST- 2012/07/26 06:00 [medline] AID - 10.1007/s00296-010-1699-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Apr;32(4):895-908. doi: 10.1007/s00296-010-1699-4. Epub 2011 Jan 13. PMID- 30322339 OWN - NLM STAT- MEDLINE DCOM- 20190502 LR - 20190502 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 48 IP - 3 DP - 2019 May TI - Vascular trauma of the hand - a systematic review. PG - 205-215 LID - 10.1024/0301-1526/a000743 [doi] AB - Hypothenar or thenar hammer syndrome (HHS) and hand-arm vibration syndrome (HAVS) are diseases caused by acute or chronic trauma to the upper extremities. Since both diseases are generally related to occupation and are recognised as occupational diseases in most countries, vascular physicians need to be able to distinguish between the two entities and differentiate them from other diagnoses. A total of 867 articles were identified as part of an Internet search on PubMed and in non-listed occupational journals. For the analysis we included 119 entries on HHS as well as 101 papers on HAVS. A professional history and a job analysis were key components when surveying the patient's medical history. The Doppler-Allen test, duplex sonography and optical acral pulse oscillometry were suitable for finding an objective basis for the clinical tests. In the case of HHS, digital subtraction angiography was used to confirm the diagnosis and plan treatment. Radiological tomographic techniques provided very limited information distal to the wrist. The vascular component of HAVS proved to be strongly dependent on temperature and had to be differentiated from the various other causes of secondary Raynaud's phenomenon. The disease was medicated with anticoagulants and vasoactive substances. If these were not effective, a bypass was performed in addition to various endovascular interventions, especially in the case of HHS. Despite the relatively large number of people exposed, trauma-induced circulatory disorders of the hands can be observed in a comparatively small number of cases. For the diagnosis of HHS, the morphological detection of vascular lesions through imaging is essential since the disorder can be accompanied by critical limb ischaemia, which may require bypass surgery. In the case of HAVS, vascular and sensoneurological pathologies must be objectified through provocation tests. The main therapeutic approach to HAVS is preventing exposure. FAU - Wahl, Uwe AU - Wahl U AD - 1 BG Hospital Bergmannstrost Halle, Department of Internal Medicine, Halle, Germany. FAU - Kaden, Ingmar AU - Kaden I AD - 2 BG Hospital Bergmannstrost Halle, Department of Diagnostic Imaging and Interventional Radiology, Halle, Germany. FAU - Köhler, Andreas AU - Köhler A AD - 3 Practice for Cardiology and Vascular Diseases, Halle, Germany. FAU - Hirsch, Tobias AU - Hirsch T AD - 4 Practice for Internal Medicine and Vascular Diseases, Vein Competence Centre, Halle, Germany. LA - eng PT - Journal Article PT - Systematic Review DEP - 20181016 PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - Hand MH - Humans MH - Occupational Diseases MH - Raynaud Disease MH - *Vascular System Injuries MH - Vibration OTO - NOTNLM OT - Raynaud’s phenomenon OT - hand-arm vibration syndrome OT - hypothenar hammer syndrome OT - occupational disease OT - vascular trauma OT - vibration-induced white finger EDAT- 2018/10/17 06:00 MHDA- 2019/05/03 06:00 CRDT- 2018/10/17 06:00 PHST- 2018/10/17 06:00 [pubmed] PHST- 2019/05/03 06:00 [medline] PHST- 2018/10/17 06:00 [entrez] AID - 10.1024/0301-1526/a000743 [doi] PST - ppublish SO - Vasa. 2019 May;48(3):205-215. doi: 10.1024/0301-1526/a000743. Epub 2018 Oct 16. PMID- 40757426 OWN - NLM STAT- MEDLINE DCOM- 20250804 LR - 20250804 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 8 DP - 2025 Aug TI - Evaluation of different algorithms to identify the scleroderma pattern in nailfold videocapillaroscopy. PG - 1499-1507 LID - 10.55563/clinexprheumatol/0igmnr [doi] AB - OBJECTIVES: Although the role of nailfold videocapillaroscopy (NVC) in the investigation of Raynaud's phenomenon (RP) and systemic sclerosis (SSc) is well established, there is significant heterogeneity in the parameters used to identify the scleroderma pattern. Recently, different algorithms have been proposed for the identification of the scleroderma pattern associated with SSc. This study aimed to explore the accuracy of different capillaroscopic parameters and algorithms (the Fast Track algorithm and the CAPI-score) for identifying the scleroderma pattern in individuals with and without RP and autoimmune rheumatic diseases. METHODS: A total of 258 NVCs were analysed. The accuracy and area under the curve (AUC) of qualitative and quantitative NVC parameters were analysed to discriminate between scleroderma and non-scleroderma patterns. RESULTS: The scleroderma pattern was identified in 101 (39.15%) NVCs. A density of ≤8 capillaries/mm was defined as the optimal cut-off point (AUC 0.911, 95% CI 0.871-0.950), yielding the highest accuracy (87.94%) for identifying the SD pattern versus normal and nonspecific microangiopathy. Cut-off values of ≤3 or ≤6 capillaries/mm resulted in lower sensitivity despite high specificity. The presence of giant capillaries demonstrated high specificity (98.09%) and an accuracy of 85.66%. The accuracy improved when the presence of giant capillaries and ≤8 capillaries/mm or ≤7 capillaries/mm were combined (accuracies of 91.08% and 86.82%, respectively). CONCLUSIONS: The combination of two capillaroscopy parameters (giant capillaries and capillary density) inspired by the Fast Track and CAPI-score, was highly accurate for defining the scleroderma pattern in our cohort. FAU - Shinzato, Andressa AU - Shinzato A AD - Division of Rheumatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, SP, Brazil. FAU - Sekiyama, Juliana Y AU - Sekiyama JY AD - Maringá Regional University Hospital, Universidade Estadual de Maringá, Maringá, PR, Brazil. FAU - Kayser, Cristiane AU - Kayser C AD - Division of Rheumatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, SP, Brazil. cristiane.kayser@unifesp.br. LA - eng PT - Comparative Study PT - Evaluation Study PT - Journal Article DEP - 20250801 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - *Algorithms MH - *Scleroderma, Systemic/diagnosis/diagnostic imaging/physiopathology MH - Female MH - *Nails/blood supply MH - Middle Aged MH - Male MH - *Capillaries/pathology/diagnostic imaging MH - Adult MH - *Raynaud Disease/diagnosis/diagnostic imaging MH - Predictive Value of Tests MH - Aged MH - Reproducibility of Results EDAT- 2025/08/04 12:27 MHDA- 2025/08/04 12:28 CRDT- 2025/08/04 06:28 PHST- 2025/05/12 00:00 [received] PHST- 2025/07/21 00:00 [accepted] PHST- 2025/08/04 12:28 [medline] PHST- 2025/08/04 12:27 [pubmed] PHST- 2025/08/04 06:28 [entrez] AID - 22578 [pii] AID - 10.55563/clinexprheumatol/0igmnr [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Aug;43(8):1499-1507. doi: 10.55563/clinexprheumatol/0igmnr. Epub 2025 Aug 1. PMID- 33129579 OWN - NLM STAT- MEDLINE DCOM- 20211124 LR - 20211124 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 42 IP - 2 DP - 2021 Feb TI - [Monocentric study on pharmaceuticals taken by patients to treat systemic sclerosis]. PG - 86-92 LID - S0248-8663(20)30363-5 [pii] LID - 10.1016/j.revmed.2020.08.014 [doi] AB - INTRODUCTION: Pharmaceutical prescription in systemic sclerosis is guided by national and international recommendations. This study's primary objective was to describe and analyze these prescriptions among patients of our cohort. We also aimed to assess drug compliance among our patients. METHODS: This is a monocentric observational study on two cohorts of patients with systemic sclerosis; a primary cohort comprising ambulatory patients, who were prospectively included, with exhaustive prescription's data collection; and a secondary cohort included patients asked to fill in a self-questionnaire on treatment compliance. RESULTS: The main cohort included 157 patients, including 31 cases of diffuse systemic sclerosis. A vasodilator drug for Raynaud's phenomenon was prescribed in 75 patients (47.9%) and a specific treatment for pulmonary arterial hypertension in 10 patients (6.4%). Immuno-modulators/immunosuppressants was prescribed in 62 patients (39.5%), who received prednisone (n=37, 23.6%), mycophenolate mofetil (n=14, 8.9%), hydroxychloroquine (n=12, 7.6%) and colchicine (n=22, 14%). Treatment for "gastro-intestinal tract involvement" was prescribed for 106 patients (67.5%) and treatment of a scleroderma renal crisis with an angiotensin-converting enzyme inhibitor for 6 patients (3.8%). Among the 42 patients in the secondary cohort, 21.4% reported a good compliance, mostly older patients (P=0.045) or those who had not experienced adverse events (P=0.009). CONCLUSION: This study provides original real-life data illustrating the heterogeneity of prescription habits in systemic sclerosis. As previously reported, treatment compliance was insufficient. CI - Copyright © 2020 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Renaud, A AU - Renaud A AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. Electronic address: arthur.renaud49@gmail.com. FAU - Durant, C AU - Durant C AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. FAU - Achille, A AU - Achille A AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. FAU - Artifoni, M AU - Artifoni M AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. FAU - Espitia, O AU - Espitia O AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. FAU - Agard, C AU - Agard C AD - Service de médecine interne, centre hospitalo-universitaire, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France. LA - fre PT - Journal Article PT - Observational Study TT - Étude monocentrique sur les médicaments pris par les patients pour le traitement de la sclérodermie systémique. DEP - 20201029 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Pharmaceutical Preparations) SB - IM MH - Angiotensin-Converting Enzyme Inhibitors MH - Humans MH - *Pharmaceutical Preparations MH - *Raynaud Disease/drug therapy/epidemiology MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/drug therapy/epidemiology OTO - NOTNLM OT - Compliance OT - Observance OT - Sclérodermie systémique OT - Systemic sclerosis OT - Traitement OT - Treatment EDAT- 2020/11/02 06:00 MHDA- 2021/11/25 06:00 CRDT- 2020/11/01 20:27 PHST- 2020/03/15 00:00 [received] PHST- 2020/07/14 00:00 [revised] PHST- 2020/08/03 00:00 [accepted] PHST- 2020/11/02 06:00 [pubmed] PHST- 2021/11/25 06:00 [medline] PHST- 2020/11/01 20:27 [entrez] AID - S0248-8663(20)30363-5 [pii] AID - 10.1016/j.revmed.2020.08.014 [doi] PST - ppublish SO - Rev Med Interne. 2021 Feb;42(2):86-92. doi: 10.1016/j.revmed.2020.08.014. Epub 2020 Oct 29. PMID- 22753661 OWN - NLM STAT- MEDLINE DCOM- 20130116 LR - 20171116 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 39 IP - 8 DP - 2012 Aug TI - Noninvasive measurement of skin autofluorescence is increased in patients with systemic sclerosis: an indicator of increased advanced glycation endproducts? PG - 1654-8 LID - 10.3899/jrheum.111359 [doi] AB - OBJECTIVE: Skin autofluorescence noninvasively assesses expression of advanced glycation endproducts and therefore potentially the presence of oxidative stress that is implicated in the pathogenesis of systemic sclerosis (SSc). We investigated whether autofluorescence was increased in patients with SSc, primary Raynaud's phenomenon (RP), and morphea as compared to healthy controls. METHODS: Measurements of autofluorescence were made at 5 upper limb sites in 16 healthy controls, 16 patients with diffuse cutaneous SSc (dcSSc), 15 with limited cutaneous SSc (lcSSc), 15 with primary RP, and 13 with morphea. For patients with morphea, additional measurements were made at the affected and an adjacent unaffected site. RESULTS: Autofluorescence was significantly increased in patients with dcSSc but not lcSSc as compared to controls at the proximal phalanx [dcSSc median 0.15, interquartile range (IQR) 0.10-0.24, vs control 0.10, IQR 0.07-0.13; p = 0.014], dorsum of the hand (dcSSc 0.17, IQR 0.11-0.36, vs control 0.12, IQR 0.09-0.17; p = 0.031), the wrist (dcSSc 0.22, IQR 0.13-0.33, vs control 0.13, IQR 0.09-0.18; p = 0.005), and forearm (dcSSc 0.19, IQR 0.12-0.47, vs control 0.14, IQR 0.10-0.16; p = 0.022). There was a trend for autofluorescence to be increased in patients with lcSSc and at morphea sites, compared to noninvolved skin. CONCLUSION: Autofluorescence is increased in patients with dcSSc compared to primary RP and to healthy controls. This suggests increased oxidative stress and the potential for autofluorescence as a biomarker. FAU - Murray, Andrea K AU - Murray AK AD - Musculoskeletal Research Group, School of Translational Medicine, Salford Royal NHS Foundation, Manchester M6 8HD, UK. Andrea.murray@manchester.ac.uk. FAU - Moore, Tonia L AU - Moore TL FAU - Manning, Joanne B AU - Manning JB FAU - Griffiths, Christopher E M AU - Griffiths CE FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Journal Article DEP - 20120701 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Glycation End Products, Advanced) SB - IM CIN - J Rheumatol. 2013 Feb;40(2):206. doi: 10.3899/jrheum.121060. PMID: 23378499 CIN - J Rheumatol. 2013 Feb;40(2):206. doi: 10.3899/jrheum.121275. PMID: 23487862 MH - Adult MH - Aged MH - Female MH - Glycation End Products, Advanced/*metabolism MH - Humans MH - Male MH - Middle Aged MH - Optical Imaging MH - Oxidative Stress MH - Raynaud Disease/metabolism/pathology MH - Scleroderma, Localized/metabolism/pathology MH - Scleroderma, Systemic/*metabolism/pathology MH - Skin/*metabolism/pathology EDAT- 2012/07/04 06:00 MHDA- 2013/01/17 06:00 CRDT- 2012/07/04 06:00 PHST- 2012/07/04 06:00 [entrez] PHST- 2012/07/04 06:00 [pubmed] PHST- 2013/01/17 06:00 [medline] AID - jrheum.111359 [pii] AID - 10.3899/jrheum.111359 [doi] PST - ppublish SO - J Rheumatol. 2012 Aug;39(8):1654-8. doi: 10.3899/jrheum.111359. Epub 2012 Jul 1. PMID- 20837499 OWN - NLM STAT- MEDLINE DCOM- 20110919 LR - 20220410 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 12 DP - 2010 Dec TI - Efficacy of tadalafil in secondary Raynaud's phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. PG - 2420-8 LID - 10.1093/rheumatology/keq291 [doi] AB - OBJECTIVE: To evaluate the efficacy of tadalafil as add-on therapy in secondary RP resistant to vasodilators. METHODS: Patients with scleroderma and MCTD having four or more RP attacks per week despite being on vasodilators were randomized to receive either placebo or tadalafil (20 mg) on alternate days as add-on therapy to their current vasodilators for 6 weeks. After a 7-day washout, patients were crossed over to the other arm. Primary endpoints were improvement in the daily frequency and duration of RP episodes and RP condition score (RCS). Secondary outcome measures were healing of existing and appearance of new digital ulcers (DUs) and improvement in scleroderma-specific HAQ (SHAQ), quality of life (QoL), flow-mediated dilatation (FMD), patient and physician global assessment. RESULTS: Twenty-four of 25 recruited patients completed the study. All the patients were receiving calcium channel blockers and in addition 18 were receiving other vasodilators. During tadalafil therapy significant improvement in mean daily frequency, mean daily duration of RP and mean daily RCS were observed as compared with baseline and placebo. All the 24 digital lesions healed during tadalafil therapy as compared with 3/13 during the placebo treatment (P<0.0001). One new DU was reported during tadalafil therapy vs 13 during placebo therapy (P=0.0005). QoL, SHAQ, FMD, patient and physician global assessment significantly improved while on tadalafil. No serious adverse event was observed. CONCLUSION: Tadalafil as add-on therapy improves symptoms of RP, heals and prevents new DUs and improves QoL in patients with resistant secondary RP. TRIAL REGISTRATION: Clinicaltrials.gov, http://clinicaltrials.gov/, identifier: NCT00626665. FAU - Shenoy, Padmanabha D AU - Shenoy PD AD - Department of Immunology, SGPGIMS, Lucknow 226014, Uttar Pradesh, India. vikasagr@yahoo.com. FAU - Kumar, Sudeep AU - Kumar S FAU - Jha, Lalan K AU - Jha LK FAU - Choudhary, Sunil K AU - Choudhary SK FAU - Singh, Uttam AU - Singh U FAU - Misra, Ramnath AU - Misra R FAU - Agarwal, Vikas AU - Agarwal V LA - eng SI - ClinicalTrials.gov/NCT00626665 PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20100912 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Carbolines) RN - 0 (Vasodilator Agents) RN - 742SXX0ICT (Tadalafil) SB - IM MH - Adult MH - Carbolines/*therapeutic use MH - Cross-Over Studies MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/*complications/drug therapy MH - Tadalafil MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use MH - Young Adult EDAT- 2010/09/15 06:00 MHDA- 2011/09/20 06:00 CRDT- 2010/09/15 06:00 PHST- 2010/09/15 06:00 [entrez] PHST- 2010/09/15 06:00 [pubmed] PHST- 2011/09/20 06:00 [medline] AID - keq291 [pii] AID - 10.1093/rheumatology/keq291 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Dec;49(12):2420-8. doi: 10.1093/rheumatology/keq291. Epub 2010 Sep 12. PMID- 20501526 OWN - NLM STAT- MEDLINE DCOM- 20101206 LR - 20110412 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 19 IP - 10 DP - 2010 Sep TI - Cutaneous manifestations and serological findings in 260 patients with systemic lupus erythematosus. PG - 1187-94 LID - 10.1177/0961203310367656 [doi] AB - The prevalence and prognostic value of cutaneous manifestations in patients with systemic lupus erythematosus (SLE) is not clear due to a lack of distinct criteria. Our aim was to investigate the prevalence of cutaneous manifestations in SLE patients according to strict dermatological classification, compare the results with other studies and to assess differences in serological markers between patients with and without cutaneous lupus erythematosus (CLE). Secondary aims were to investigate the validity of the criteria 'malar rash' and 'photosensitivity' for SLE diagnosis. We included 260 consecutive SLE patients, and 164 with skin complaints were examined by a dermatologist. CLE was found in 23% of the 260 SLE patients. There was agreement on the presence of malar rash in only 60% of patients seen by both rheumatologists and dermatologists. A history of polymorphous light eruption (PLE) was found in 42% of patients. Raynaud's phenomenon was significantly more common in patients with CLE. In addition, four malignant melanomas were found. Based on our findings, we suggest that the American College of Rheumatology (ACR) criteria for SLE diagnosis include histopathologically confirmed CLE as one criterion, and that the criteria photosensitivity and malar rash should be re-defined. Regular examination by a dermatologist is called for in SLE patients. FAU - Grönhagen, C M AU - Grönhagen CM AD - Karolinska Institutet, Department of Clinical Sciences, Danderyd Hospital, Division of Dermatology, Danderyd, Sweden. carina.gronhagen@ds.se FAU - Gunnarsson, I AU - Gunnarsson I FAU - Svenungsson, E AU - Svenungsson E FAU - Nyberg, F AU - Nyberg F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100525 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM EIN - Lupus. 2011;20(3):336 MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Lupus Erythematosus, Cutaneous/diagnosis/epidemiology/*physiopathology MH - Lupus Erythematosus, Discoid/diagnosis/epidemiology/*physiopathology MH - Lupus Erythematosus, Systemic/diagnosis/epidemiology/*physiopathology MH - Male MH - Melanoma/diagnosis/pathology MH - Middle Aged MH - Photosensitivity Disorders/diagnosis/etiology MH - Prevalence MH - Prognosis MH - Raynaud Disease/diagnosis/etiology MH - Skin/pathology MH - Young Adult EDAT- 2010/05/27 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/05/27 06:00 PHST- 2010/05/27 06:00 [entrez] PHST- 2010/05/27 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 0961203310367656 [pii] AID - 10.1177/0961203310367656 [doi] PST - ppublish SO - Lupus. 2010 Sep;19(10):1187-94. doi: 10.1177/0961203310367656. Epub 2010 May 25. PMID- 19433434 OWN - NLM STAT- MEDLINE DCOM- 20090921 LR - 20090616 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 48 IP - 7 DP - 2009 Jul TI - The efficacy of complementary and alternative medicine in the treatment of Raynaud's phenomenon: a literature review and meta-analysis. PG - 791-5 LID - 10.1093/rheumatology/kep039 [doi] AB - OBJECTIVE: Conventional treatment for RP is limited due to side effects, and complementary and alternative medicines (CAM) are widely used by the population. Our objective was to find an effective and well-tolerated CAM for the treatment of RP. METHODS: Using MEDLINE, EMBASE and AMED, 20 randomized controlled trials (RCTs) were found and divided into nine treatment subcategories: acupuncture (n = 2 trials), anti-oxidants (n = 2), biofeedback (n = 5), essential fatty acids (n = 3), Ginkgo biloba (n = 1), L-arginine (n = 2), laser (n = 3), glucosaminoglycans (n = 1) and therapeutic gloves (n = 1). Trials in each subcategory were meta-analysed together. RESULTS: Several categories did not have enough trials to do a meta-analysis and most trials were negative, of poor quality and done prior to 1990. Biofeedback was negative for a change in frequency, duration and severity of RP attacks, and actually favoured control (sham biofeedback; P < 0.02). The therapeutic glove favoured active treatment (P < 0.00001). Laser resulted in one less RP attack on average over 2 weeks vs sham [weighted mean difference (WMD) 1.18; 95% CI 1.06, 1.29], and a change in severity of attacks (WMD 1.98; 95% CI 1.57, 2.39; P < 0.05). No significant differences were found in the nutritional supplements that were studied. CONCLUSIONS: There is a need for well-designed trials of CAM in RP. The literature is inconclusive except that biofeedback does not work for RP, therapeutic gloves may improve RP (but results may not be generalizable due to single trial site and no intent-to-treat analysis) and laser may be effective but the improvement may not be clinically relevant. FAU - Malenfant, Deanne AU - Malenfant D AD - Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada. FAU - Catton, Michelle AU - Catton M FAU - Pope, Janet E AU - Pope JE LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20090511 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - *Complementary Therapies MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/drug therapy/*therapy MH - Treatment Failure RF - 27 EDAT- 2009/05/13 09:00 MHDA- 2009/09/22 06:00 CRDT- 2009/05/13 09:00 PHST- 2009/05/13 09:00 [entrez] PHST- 2009/05/13 09:00 [pubmed] PHST- 2009/09/22 06:00 [medline] AID - kep039 [pii] AID - 10.1093/rheumatology/kep039 [doi] PST - ppublish SO - Rheumatology (Oxford). 2009 Jul;48(7):791-5. doi: 10.1093/rheumatology/kep039. Epub 2009 May 11. PMID- 19037604 OWN - NLM STAT- MEDLINE DCOM- 20090902 LR - 20260128 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 7 DP - 2009 May TI - Effects of bosentan on the skin lesions: an observational study from a single center in Japan. PG - 769-75 LID - 10.1007/s00296-008-0789-z [doi] AB - Effects of a dual endothelin receptor antagonist, bosentan on peripheral circulatioin and skin lesions as well as pulmonary arterial hypertension (PAH) were investigated in Japanese patients with connective tissue diseases (CTD). Fifteen patients with PAH associated with CTD [systemic sclerosis (SSc) 13, mixed connective tissue disease (MCTD) 2] were treated with bosentan for 40-96 weeks, and changes of exercise capacity (6-min walk distance and Borg's dyspnea scale), cardio-pulmonary hemodynamics (right ventricular pressure, specific activity scale and cardiac index), Raynaud's phenomenon, digital ulcers and dermal sclerosis were observed. Bosentan improved exercise capacity, had a positive effect on hemodynamic parameters, and was well tolerated as previously reported. After a median 8 weeks of treatment, 13 out of 15 patients had improved Raynaud's phenomenon. Digital ulcers also improved after a median 12 weeks' treatment in all of 8 patients. Modified Rodnan total skin score decreased from 21.0 +/- 5.9 to 11.5 +/- 3.9 in diffuse cutaneous SSc and from 17.0 +/- 6.5 to 9.5 +/- 4.5 in limited cutaneous SSc after 24 months' treatment, reaching significance after 6 months in both groups. These data suggest that bosentan is effective for both PAH and peripheral vascular diseases in Japanese patients with CTD. The pathological background to the improvement in dermal sclerosis observed in this study should be further investigated. FAU - Funauchi, Masanori AU - Funauchi M AD - Department of Nephrology and Rheumatology, Kinki University School of Medicine, 377-2 Ohnohigashi, Osaka-Sayama, Osaka, 589-8511, Japan. mn-funa@med.kindai.ac.jp FAU - Kishimoto, K AU - Kishimoto K FAU - Shimazu, H AU - Shimazu H FAU - Nagare, Y AU - Nagare Y FAU - Hino, S AU - Hino S FAU - Yano, T AU - Yano T FAU - Kinoshita, K AU - Kinoshita K LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20081127 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*administration & dosage/adverse effects MH - Arteries/drug effects/immunology/physiopathology MH - Bosentan MH - Connective Tissue Diseases/complications/*drug therapy/physiopathology MH - Exercise Tolerance/drug effects/physiology MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy/immunology/physiopathology MH - Japan MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/complications/drug therapy/physiopathology MH - Prospective Studies MH - Pulmonary Artery/drug effects/immunology/physiopathology MH - Raynaud Disease/drug therapy/immunology/physiopathology MH - Scleroderma, Systemic/complications/drug therapy/physiopathology MH - Skin Diseases/*drug therapy/immunology/physiopathology MH - Sulfonamides/*administration & dosage/adverse effects MH - Ulcer/drug therapy/immunology/physiopathology MH - Vasculitis/*drug therapy/immunology/physiopathology EDAT- 2008/11/28 09:00 MHDA- 2009/09/03 06:00 CRDT- 2008/11/28 09:00 PHST- 2008/06/11 00:00 [received] PHST- 2008/09/03 00:00 [accepted] PHST- 2008/11/28 09:00 [pubmed] PHST- 2009/09/03 06:00 [medline] PHST- 2008/11/28 09:00 [entrez] AID - 10.1007/s00296-008-0789-z [doi] PST - ppublish SO - Rheumatol Int. 2009 May;29(7):769-75. doi: 10.1007/s00296-008-0789-z. Epub 2008 Nov 27. PMID- 24615537 OWN - NLM STAT- MEDLINE DCOM- 20150624 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 33 IP - 11 DP - 2014 Nov TI - Association between endothelial function and microvascular changes in patients with secondary Raynaud's phenomenon. PG - 1627-33 LID - 10.1007/s10067-014-2553-y [doi] AB - Nailfold capillaroscopy (NC) represents the method to analyze microvascular abnormalities in autoimmune rheumatic diseases, but the pathophysiological link between the microvascular derangement which is seen in NC and endothelial function is yet to be discovered. We investigated the association between endothelial function and microvascular derangement in patients with Raynaud's phenomenon (RP). Postmenopausal women (n = 37) with secondary RP and age-matched healthy controls (n = 25) were evaluated with NC. Microvascular alterations were assessed by microangiopathy evolution score. Endothelial function was examined by brachial artery flow-mediated dilatation (reactive FMD, endothelium-dependent) and response to 40 μg of sublingual nitroglycerine (NTG-induced dilatation, endothelium-independent). There was significant capillary loop dilatation (apical width; 14.1 ± 5.6 vs. 10.4 ± 1.7 μm, p = 0.001 and total width; 40.6 ± 15.1 vs. 31.6 ± 4.6 μm, p = 0.002) and lengthening (316.0 ± 78.5 vs. 270.4 ± 34.7 μm, p = 0.004) in secondary RP compared to controls. Additionally, giant capillaries, loss of capillaries, hemorrhage, and background pallor were much more prevalent in secondary RP as compared to controls (all p's < 0.05). Although there were no significant differences in NTG-induced dilatation between secondary RP and controls (16.1 ± 5.9 vs. 19.6 ± 9.0 %, p = 0.091), significant decreases in the reactive FMD value (6.1 ± 3.5 vs. 9.0 ± 2.2 %, p = 0.001) were noted. Both FMD and NTG-induced dilatation showed a significant inverse association with microangiopathy evolution score (r = -0.355, p = 0.005 and r = -0.285, p = 0.028). Significantly impaired endothelial function was found in secondary RP, and microvascular derangement was associated with endothelial dysfunction. FAU - Le, Ji Hyun AU - Le JH AD - Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center, Busan, Republic of Korea. FAU - Cho, Kyoung Im AU - Cho KI LA - eng PT - Journal Article DEP - 20140311 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Brachial Artery/*physiopathology MH - Capillaries/*physiopathology MH - Endothelium, Vascular/*physiopathology MH - Female MH - Humans MH - Microscopic Angioscopy MH - Middle Aged MH - Postmenopause/physiology MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Vasodilation/*physiology EDAT- 2014/03/13 06:00 MHDA- 2015/06/25 06:00 CRDT- 2014/03/12 06:00 PHST- 2013/12/29 00:00 [received] PHST- 2014/02/21 00:00 [accepted] PHST- 2014/02/17 00:00 [revised] PHST- 2014/03/12 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2015/06/25 06:00 [medline] AID - 10.1007/s10067-014-2553-y [doi] PST - ppublish SO - Clin Rheumatol. 2014 Nov;33(11):1627-33. doi: 10.1007/s10067-014-2553-y. Epub 2014 Mar 11. PMID- 39498828 OWN - NLM STAT- MEDLINE DCOM- 20250123 LR - 20250530 IS - 1365-2249 (Electronic) IS - 0009-9104 (Print) IS - 0009-9104 (Linking) VI - 219 IP - 1 DP - 2025 Jan 21 TI - The B-cells paradigm in systemic sclerosis: an update on pathophysiology and B-cell-targeted therapies. LID - 10.1093/cei/uxae098 [doi] LID - uxae098 AB - Systemic sclerosis (SSc) is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting in the production of autoantibodies. Abnormalities of the immune system compromise the normal function of blood vessels leading to a vasculopathy manifested by Raynaud's phenomenon, an early sign of SSc . As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis. Several SSc-specific autoantibodies are currently known and are associated with specific clinical manifestations and prognosis. Although the pathogenetic role of these autoantibodies is still unclear, their production by B cells and plasma cells suggests the importance of these cells in the development of SSc. This review narratively examines B-cell dysfunctions and their role in the pathogenesis of SSc and discusses B-cell-targeted therapies currently used or potentially useful for the management of end-organ complications. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology. FAU - Scaletti, Cristina AU - Scaletti C AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. FAU - Pratesi, Sara AU - Pratesi S AUID- ORCID: 0000-0003-3550-1959 AD - Flow Cytometry Diagnostic Center and Immunotherapy, University Hospital Careggi, Florence, Italy. FAU - Bellando Randone, Silvia AU - Bellando Randone S AD - Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Florence, and Scleroderma Unit, University Hospital Careggi, Florence, Italy. FAU - Di Pietro, Linda AU - Di Pietro L AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AUID- ORCID: 0000-0001-6806-3794 AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy. FAU - Annunziato, Francesco AU - Annunziato F AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Flow Cytometry Diagnostic Center and Immunotherapy, University Hospital Careggi, Florence, Italy. FAU - Matucci Cerinic, Marco AU - Matucci Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy. AD - Vita Salute San Raffaele University, Milan, Italy. LA - eng PT - Journal Article PT - Review PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Scleroderma, Systemic/immunology/therapy/pathology MH - *B-Lymphocytes/immunology MH - Autoantibodies/immunology MH - Animals MH - Raynaud Disease/immunology PMC - PMC11754866 OTO - NOTNLM OT - B cells OT - autoantibodies OT - autoimmunity OT - chimeric antigen receptor T cell COIS- None declared. EDAT- 2024/11/05 17:01 MHDA- 2025/01/23 12:35 PMCR- 2024/11/05 CRDT- 2024/11/05 06:54 PHST- 2024/07/30 00:00 [received] PHST- 2024/10/05 00:00 [revised] PHST- 2024/11/04 00:00 [accepted] PHST- 2025/01/23 12:35 [medline] PHST- 2024/11/05 17:01 [pubmed] PHST- 2024/11/05 06:54 [entrez] PHST- 2024/11/05 00:00 [pmc-release] AID - 7876127 [pii] AID - uxae098 [pii] AID - 10.1093/cei/uxae098 [doi] PST - ppublish SO - Clin Exp Immunol. 2025 Jan 21;219(1):uxae098. doi: 10.1093/cei/uxae098. PMID- 19851110 OWN - NLM STAT- MEDLINE DCOM- 20100216 LR - 20250529 IS - 1531-6963 (Electronic) IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 22 IP - 1 DP - 2010 Jan TI - Rheumatic manifestations of skin disease. PG - 78-84 LID - 10.1097/BOR.0b013e328333b9e2 [doi] AB - PURPOSE OF REVIEW: There is an increasing interest in improving the understanding of pathophysiology, outcome measures, and therapies of rheumatic skin disease. Increasingly, studies are using the skin as a primary endpoint for evaluating therapies. This will review the current state of the art for the most common rheumatic skin diseases. RECENT FINDINGS: A number of medications, including biologics such as tumor necrosis factor alpha and interferon, have been associated with onset of cutaneous lupus. The cutaneous lupus erythematosus area and severity index has been further validated and utilized in a number of studies. Smoking continues to be associated both with presence and refractoriness of cutaneous lupus erythematosus to therapy. There are several tools now available for evaluating the skin disease of dermatomyositis, but there is a need for new effective therapies. Measurement of skin disease in scleroderma continues to be a challenge, and there is a need for more effective therapies. Several studies show efficacy of intravenous iloprost for severe Raynaud's and skin ulcers, and of bosentan for digital ulcers. SUMMARY: The present review covers new outcome measures, treatments, and unusual manifestations of cutaneous lupus, dermatomyositis, scleroderma, and rheumatoid arthritis. There have been a number of new studies related to validation of disease activity measures, as well as their use in evaluation of new therapies for these conditions. Validated outcome measures are required to perform meaningful studies, and will facilitate organized epidemiologic, quality of life, and therapeutic studies. FAU - Clarke, Jennie T AU - Clarke JT AD - Department of Dermatology, Penn State Hershey Medical Center, Hershey, USA. FAU - Werth, Victoria P AU - Werth VP LA - eng GR - K24 AR002207/AR/NIAMS NIH HHS/United States GR - K24-AR 02207/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Cytokines) RN - 0 (Immunosuppressive Agents) SB - IM MH - Cytokines/adverse effects MH - Dermatomyositis/chemically induced/immunology/physiopathology MH - Humans MH - Immunosuppressive Agents/pharmacology/therapeutic use MH - Lupus Erythematosus, Cutaneous/chemically induced/immunology/physiopathology MH - Raynaud Disease/drug therapy/immunology MH - Rheumatic Diseases/*complications/*immunology/physiopathology MH - Scleroderma, Systemic/chemically induced/immunology/physiopathology MH - Skin Diseases/drug therapy/*immunology/physiopathology PMC - PMC3081507 MID - NIHMS272679 EDAT- 2009/10/24 06:00 MHDA- 2010/02/17 06:00 PMCR- 2011/04/23 CRDT- 2009/10/24 06:00 PHST- 2009/10/24 06:00 [entrez] PHST- 2009/10/24 06:00 [pubmed] PHST- 2010/02/17 06:00 [medline] PHST- 2011/04/23 00:00 [pmc-release] AID - 10.1097/BOR.0b013e328333b9e2 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2010 Jan;22(1):78-84. doi: 10.1097/BOR.0b013e328333b9e2. PMID- 34132168 OWN - NLM STAT- MEDLINE DCOM- 20220207 LR - 20220207 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 50 IP - 6 DP - 2021 Nov TI - Temperature response to cold challenge and mobile phone thermography as outcome measures for systemic sclerosis-related Raynaud's phenomenon. PG - 479-484 LID - 10.1080/03009742.2021.1907926 [doi] AB - Objectives: Objective outcome measures of systemic sclerosis (SSc)-related Raynaud's phenomenon (RP) are badly needed. Our objectives were to validate the thermographic response to a standard hand cold challenge as an outcome measure by assessing sensitivity to change, and to explore mobile phone thermography as a feasible, ambulatory tool.Method: Twelve patients with an SSc-spectrum disorder admitted for intravenous iloprost infusions underwent a standard cold challenge before and after one infusion. Thermographic measurements included area under the rewarming curve (AUC) and maximum rewarming temperature (MAX). Before and during another infusion, each patient underwent monitoring of finger skin temperature by two methods: continuous thermocouple recording (standard method) and mobile phone thermography.Results: All cold challenge summary measures, including AUC and MAX, increased after iloprost (most not significantly). However, when the response curves were modelled after averaging across fingers (linear mixed models, three versions), significant change was detected. For example, with Model 1 (no interaction between period and time), temperature was on average 1.67ºC [95% confidence interval (CI) 1.49-1.85, p < 0.001] higher post-iloprost. Mobile phone and thermocouple temperature measurements showed a strong estimated latent correlation (0.88, 95% CI 0.81-0.92). The estimated increases/hour were 0.25ºC (95% CI 0.05-0.45) for the thermocouple and 0.36ºC (95% CI 0.13-0.60) for mobile phone thermography.Conclusion: Our pilot study suggests that the thermographic response to a cold challenge is sensitive to change and mobile phone thermography could bring feasibility to thermographic parameters as outcome measures in later-phase, large-scale, community-based clinical trials of RP. FAU - Herrick, A L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Heal, C AU - Heal C AD - Centre for Biostatistics, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK. FAU - Wilkinson, J AU - Wilkinson J AD - Centre for Biostatistics, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK. FAU - Dinsdale, G AU - Dinsdale G AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Manning, J AU - Manning J AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Gunnarsson, K AU - Gunnarsson K AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Jakobsson, P-J AU - Jakobsson PJ AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Murray, A AU - Murray A AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Journal Article DEP - 20210616 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - JED5K35YGL (Iloprost) SB - IM MH - Cell Phone MH - Cold Temperature MH - Humans MH - Iloprost MH - Outcome Assessment, Health Care MH - Pilot Projects MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Systemic/complications/therapy MH - *Thermography EDAT- 2021/06/17 06:00 MHDA- 2022/02/08 06:00 CRDT- 2021/06/16 08:51 PHST- 2021/06/17 06:00 [pubmed] PHST- 2022/02/08 06:00 [medline] PHST- 2021/06/16 08:51 [entrez] AID - 10.1080/03009742.2021.1907926 [doi] PST - ppublish SO - Scand J Rheumatol. 2021 Nov;50(6):479-484. doi: 10.1080/03009742.2021.1907926. Epub 2021 Jun 16. PMID- 28869415 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20171221 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 Suppl 106 IP - 4 DP - 2017 Sep-Oct TI - A novel iloprost administration method with portable syringe pump for the treatment of acral ulcers and Raynaud's phenomenon in systemic sclerosis patients. A pilot study (ILOPORTA). PG - 173-178 AB - OBJECTIVES: The objective of the study is to evaluate the feasibility, safety and efficacy of home infusion of iloprost with the new portable syringe pump Infonde®, for the treatment of scleroderma-related Raynaud's phenomenon and digital ulcers. METHODS: 12 scleroderma patients were treated with iloprost at home, using the pump, with infusion cycles of 2 days per month (24 hours a day), for 6 months. RESULTS: The home treatment proved feasible since ten patients (83%) completed the entire infusion cycle, thus satisfying the feasibility target imposed by the protocol (75%). Side effects related to the device or venous access occurred in 3 out of 65 total 48-hour infusions (4.6%). They mostly consisted in phlebitis. No adverse events related to the device management were reported. Among the ten patients who completed the infusions, three showed a reduction in the number of ulcers, three maintained the same number, and four had no ulcers throughout the observation period. Patient's perception of their quality of life and wellness during home infusions, expressed with the Visual Analogue Scale (VAS) improved from 79/100 at the first infusion to 91/100 at the end of the study. All patients expressed a positive global judgment regarding this innovative method of iloprost infusion. CONCLUSIONS: The infusion of iloprost at home with Infonde® is feasible, safe and effective. Moreover, this approach presents potential advantages from the economic and organisational point of view. Because of the pilot design of our study, these results need to be confirmed in larger randomised trials. FAU - Fraticelli, Paolo AU - Fraticelli P AD - Clinica Medica, Department of Internal Medicine, Ospedali Riuniti, Università Politecnica delle Marche, Ancona, Italy. paolo.fraticelli@ospedaliriuniti.marche.it. FAU - Martino, Giuseppe Pio AU - Martino GP AD - Clinica Medica, Department of Internal Medicine, Ospedali Riuniti, Università Politecnica delle Marche, Ancona, Italy. FAU - Murri, Marta AU - Murri M AD - Clinica Medica, Department of Internal Medicine, Ospedali Riuniti, Università Politecnica delle Marche, Ancona, Italy. FAU - Mattioli, Massimo AU - Mattioli M AD - Clinica Medica, Department of Internal Medicine, Ospedali Riuniti, Università Politecnica delle Marche, Ancona, Italy. FAU - Gabrielli, Armando AU - Gabrielli A AD - Clinica Medica, Department of Internal Medicine, Ospedali Riuniti University Hospital, Ancona; and Department of Clinical and Molecular Sciences, Section of Clinical Medicine, Università Politecnica delle Marche, School of Medicine, Ancona, Italy. LA - eng PT - Journal Article DEP - 20170831 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Iloprost/*administration & dosage/adverse effects MH - Male MH - Middle Aged MH - Pilot Projects MH - Quality of Life MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*complications/drug therapy MH - Skin Ulcer/*drug therapy MH - Syringes EDAT- 2017/09/05 06:00 MHDA- 2017/12/22 06:00 CRDT- 2017/09/05 06:00 PHST- 2017/03/07 00:00 [received] PHST- 2017/06/20 00:00 [accepted] PHST- 2017/09/05 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/09/05 06:00 [entrez] AID - 11698 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):173-178. Epub 2017 Aug 31. PMID- 22901779 OWN - NLM STAT- MEDLINE DCOM- 20150622 LR - 20231104 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 14 IP - 4 DP - 2012 Aug 17 TI - Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement. PG - R188 LID - 10.1186/ar4019 [doi] AB - INTRODUCTION: We investigated early systemic sclerosis (SSc) (that is, Raynaud's phenomenon with SSc marker autoantibodies and/or typical capillaroscopic findings and no manifestations other than puffy fingers or arthritis) versus undifferentiated connective tissue disease (UCTD) to identify predictors of short-term disease evolution. METHODS: Thirty-nine early SSc and 37 UCTD patients were investigated. At baseline, all patients underwent clinical evaluation, B-mode echocardiography, lung function tests and esophageal manometry to detect preclinical alterations of internal organs, and were re-assessed every year. Twenty-one early SSc and 24 UCTD patients, and 25 controls were also investigated for serum endothelial, T-cell and fibroblast activation markers. RESULTS: At baseline, 48.7% of early SSc and 37.8% of UCTD patients had at least one preclinical functional alteration (P > 0.05). Ninety-two percent of early SSc patients developed manifestations consistent with definite SSc (that is, skin sclerosis, digital ulcers/scars, two or more teleangectasias, clinically visible nailfold capillaries, cutaneous calcinosis, X-ray bibasilar lung fibrosis, X-ray esophageal dysmotility, ECG signs of myocardial fibrosis and laboratory signs of renal crisis) within five years versus 17.1% of UCTD patients (X² = 12.26; P = 0.0005). Avascular areas (HR = 4.39 95% CI 1.18 to 16.3; P = 0.02), increased levels of soluble IL-2 receptor alpha (HR = 4.39; 95% CI 1.03 to 18.6; P = 0.03), and of procollagen III aminopropeptide predicted disease evolution (HR = 4.55; 95% CI 1.18 to 17; P = 0.04). CONCLUSION: Most early SSc but only a few UCTD patients progress to definite SSc within a short-term follow-up. Measurement of circulating markers of T-cell and fibroblast activation might serve to identify early SSc patients who are more likely to develop features of definite SSc. FAU - Valentini, Gabriele AU - Valentini G FAU - Vettori, Serena AU - Vettori S FAU - Cuomo, Giovanna AU - Cuomo G FAU - Iudici, Michele AU - Iudici M FAU - D'Abrosca, Virginia AU - D'Abrosca V FAU - Capocotta, Domenico AU - Capocotta D FAU - Del Genio, Gianmattia AU - Del Genio G FAU - Santoriello, Carlo AU - Santoriello C FAU - Cozzolino, Domenico AU - Cozzolino D LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120817 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Biomarkers/blood MH - *Disease Progression MH - Early Diagnosis MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Mixed Connective Tissue Disease/blood/*diagnostic imaging MH - Predictive Value of Tests MH - Radiography MH - Raynaud Disease/blood/*diagnostic imaging MH - Scleroderma, Systemic/blood/*diagnostic imaging MH - Young Adult PMC - PMC3580584 EDAT- 2012/08/21 06:00 MHDA- 2015/06/24 06:00 PMCR- 2012/08/17 CRDT- 2012/08/21 06:00 PHST- 2012/04/05 00:00 [received] PHST- 2012/08/17 00:00 [accepted] PHST- 2012/08/21 06:00 [entrez] PHST- 2012/08/21 06:00 [pubmed] PHST- 2015/06/24 06:00 [medline] PHST- 2012/08/17 00:00 [pmc-release] AID - ar4019 [pii] AID - 10.1186/ar4019 [doi] PST - epublish SO - Arthritis Res Ther. 2012 Aug 17;14(4):R188. doi: 10.1186/ar4019. PMID- 20043182 OWN - NLM STAT- MEDLINE DCOM- 20100413 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 29 IP - 3 DP - 2010 Mar TI - Capillary dimension measured by computer-based digitalized image correlated with plasma endothelin-1 levels in patients with systemic sclerosis. PG - 247-54 LID - 10.1007/s10067-009-1288-7 [doi] AB - Endothelial and vascular damage are main leading disability in systemic sclerosis (SSc). Raynaud's phenomenon is the early symptom that presents vascular damage. Nailfold capillaroscopy (NFC) is an easily accessible diagnostic tool in secondary Raynaud's phenomenon. Considering the endothelial damage, clinical manifestations, and plasma cytokines was compared with traditionally used NFC parameter for, which to observe the number of capillaries, deletions in 3 mm, apical limb width and the capillary width itself. We hypothesize that a computer-based NFC system can generate a new powerful parameter which predicts the capillary dimension. We investigated the relationship among the plasma endothelin-1 (ET-1), clinical manifestations and quantitative analysis of computerized NFC, and to assess the optimal method in SSc. The level of ET-1 in 60 SSc patients, 30 healthy, and 23 disease controls were measured by enzyme-linked immunosorbent assay (ELISA) kit. We present a significant difference in all parameters of NFC between SSc patients and control groups. ET-1 level was increased in patients with SSc. In SSc group, capillary dimension and loss of capillaries were strongly associated with digital ulceration (p < 0.01) and pulmonary hypertension (p < 0.05). Capillary dimension and ET-1 level was in correlation with skin-hardening grade, and was higher in SSc patients with pulmonary hypertension or digital ulcer. Capillary dimension showed strong correlation with the endothelin-1 in SSc, healthy and disease control groups. (Rs = 0.31/p < 0.05, Rs = 0.82/p < 0.001, Rs = 0.83/p < 0.001). The results suggest that computer-based microscopic analysis of NFC is a useful method that potentially provides information on organ involvement and plasma ET-1. Capillary dimension maybe a powerful parameter possibly applicable in outpatient clinic for assessing SSc patients. FAU - Kim, Hyun-Sook AU - Kim HS AD - Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Chosun University of Korea, Gwangju, Korea. healthyra@chosun.ac.kr FAU - Park, Mi-Kyung AU - Park MK FAU - Kim, Ho-Youn AU - Kim HY FAU - Park, Sung-Hwan AU - Park SH LA - eng PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20091231 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Biomarkers) RN - 0 (Endothelin-1) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Biomarkers/blood MH - Capillaries/*pathology MH - Endothelin-1/*blood MH - Female MH - Humans MH - Image Processing, Computer-Assisted/*methods MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/blood supply MH - Predictive Value of Tests MH - Raynaud Disease/metabolism/pathology MH - Scleroderma, Systemic/*metabolism/*pathology MH - Young Adult EDAT- 2010/01/01 06:00 MHDA- 2010/04/14 06:00 CRDT- 2010/01/01 06:00 PHST- 2008/11/22 00:00 [received] PHST- 2009/09/16 00:00 [accepted] PHST- 2009/09/13 00:00 [revised] PHST- 2010/01/01 06:00 [entrez] PHST- 2010/01/01 06:00 [pubmed] PHST- 2010/04/14 06:00 [medline] AID - 10.1007/s10067-009-1288-7 [doi] PST - ppublish SO - Clin Rheumatol. 2010 Mar;29(3):247-54. doi: 10.1007/s10067-009-1288-7. Epub 2009 Dec 31. PMID- 28645243 OWN - NLM STAT- MEDLINE DCOM- 20190410 LR - 20230928 IS - 1558-9455 (Electronic) IS - 1558-9447 (Print) IS - 1558-9447 (Linking) VI - 13 IP - 4 DP - 2018 Jul TI - Extended Periarterial Sympathectomy: Evaluation of Long-term Outcomes. PG - 395-402 LID - 10.1177/1558944717715119 [doi] AB - BACKGROUND: Periarterial sympathectomy is a proposed surgical treatment for patients with refractory Raynaud syndrome; however, there is debate regarding the indications and extent of dissection. Due to the segmental arterial sympathetic innervation, we favor an extended sympathectomy in concert with vein graft reconstruction of occluded vessels when necessary. The purpose of this study is to examine outcomes of extended periarterial sympathectomy in our patients. METHODS: A retrospective chart review was performed on 46 patients who underwent 58 periarterial sympathectomies (12 bilateral) since 1981. The data collected include demographics, comorbidities, previous therapy, operative details, and surgical outcomes. In addition, we contacted available patients for a phone survey. RESULTS: Of 58 cases, 68.9% were female, 29.3% were current smokers, and 58.6% had known connective tissue disease. Thirty-three vein graft reconstructions were performed with a long-term patency of 77.4%. Sustained improvement of ischemic pain was reported in 94.8% of cases, and 78% of patients with ulcers completely healed. For the most symptomatic fingertip, mean Semmes-Weinstein monofilament measurements improved from 4.15 preoperatively to 3.29 postoperatively ( P ≤ .05). Mean follow-up was 3.97 years. Of 10 patients contacted by telephone, all reported a decrease in frequency and severity of Raynaud attacks, while 9 reported a long-term decrease in pain an average of 11.6 years after surgery. CONCLUSIONS: Extended periarterial sympathectomy is an effective and safe procedure for patients with refractory Raynaud syndrome. Our data demonstrate long-term improvement in ischemic pain and sensibility, along with a high rate of ulcer healing and patient satisfaction. FAU - Pace, Collier S AU - Pace CS AD - 1 Virginia Commonwealth University, Richmond, USA. FAU - Merritt, Wyndell H AU - Merritt WH AD - 1 Virginia Commonwealth University, Richmond, USA. LA - eng PT - Journal Article DEP - 20170623 PL - United States TA - Hand (N Y) JT - Hand (New York, N.Y.) JID - 101264149 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Arterial Occlusive Diseases/diagnostic imaging/surgery MH - Female MH - Fingers/blood supply/innervation/*surgery MH - Follow-Up Studies MH - Humans MH - Magnetic Resonance Angiography MH - Male MH - Middle Aged MH - Pain/surgery MH - Radial Artery/surgery MH - Raynaud Disease/*surgery MH - Reoperation/statistics & numerical data MH - Retrospective Studies MH - Sympathectomy/*methods MH - Ulnar Artery/surgery MH - Vascular Patency MH - Veins/transplantation PMC - PMC6081779 OTO - NOTNLM OT - Raynaud’s OT - adventectomy OT - hand ischemia OT - periarterial sympathectomy COIS- Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2017/06/25 06:00 MHDA- 2019/04/11 06:00 PMCR- 2019/07/01 CRDT- 2017/06/25 06:00 PHST- 2017/06/25 06:00 [pubmed] PHST- 2019/04/11 06:00 [medline] PHST- 2017/06/25 06:00 [entrez] PHST- 2019/07/01 00:00 [pmc-release] AID - 10.1177_1558944717715119 [pii] AID - 10.1177/1558944717715119 [doi] PST - ppublish SO - Hand (N Y). 2018 Jul;13(4):395-402. doi: 10.1177/1558944717715119. Epub 2017 Jun 23. PMID- 27162291 OWN - NLM STAT- MEDLINE DCOM- 20160616 LR - 20161126 IS - 0043-5147 (Print) IS - 0043-5147 (Linking) VI - 69 IP - 1 DP - 2016 TI - [Autoimmune diseases with the presence of anti-ku antibodies - analysis of three cases]. PG - 24-6 AB - a-Ku are rare antibodies, which are reported in course of connective tissue diseases. Their prevalence ranges from 0 to 10% , 2%, on average. The main symptoms associated with the presence of a-Ku antibodies include: myositis, arthritis, Raynaud`s phenomenon and skin lesions. The above features are often defined as autoimmune clinical syndrome associated with a-Ku antibodies. In recent years, three cases with the presence of a-Ku antibodies were observed at the Department of Rheumatology and Connective Tissue Diseases. Case 1, a 77-year-old man, with the diagnosis of mixed connective tissue disease according to Raynaud`s phenomenon, myositis, arthritis and presence of a-ribonucleoprotein antibodies. Moreover, secondary Sjögren syndrome (SS) and myasthenia gravis were diagnosed. Case 2, a 56-year-old woman with longstanding history of Raynaud`s phenomenon, sclerodactyly, myositis and arthritis. Based on clinical manifestations and additional tests, systemic sclerosis and myositis were diagnosed. Case 3, a 46-year-old woman with SS diagnosis, long-standing history of Raynaud`s phenomenon, arthralgia and polyneuropathy. Moreover, HCV infection with the presence of cryoglobulin was confirmed. The presence of a-Ku antibodies in high titers was found in all cases. The clinical conditions improved after steroid and immunosuppressive therapy. In conclusion, clinical syndromes with the presence of a-Ku antibodies are associated with a wide range of non-specific symptoms, regarding muscle, joint and skin involvement, in particular. The conditions are more often diagnosed in the elderly; in the majority of cases, they are characterized by mild courses, good response to steroid therapy and good prognosis. FAU - Wielosz, Ewa AU - Wielosz E AD - Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin. FAU - Majdan, Maria AU - Majdan M AD - Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin. FAU - Jeleniewicz, Radosław AU - Jeleniewicz R AD - Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin. FAU - Mazurek, Marcin AU - Mazurek M AD - Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny, Lublin. LA - pol PT - Case Reports PT - Journal Article TT - Choroby autoimmunologiczne z obecnośćia przeciwciał anty-ku na podstawie analizy trzech przypadków klinicznych. PL - Poland TA - Wiad Lek JT - Wiadomosci lekarskie (Warsaw, Poland : 1960) JID - 9705467 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (DNA-Binding Proteins) RN - EC 3.6.4.12 (Xrcc6 protein, human) RN - EC 4.2.99.- (Ku Autoantigen) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood/immunology MH - Antigens, Nuclear/*immunology MH - Arthralgia/etiology MH - Autoimmune Diseases/*diagnosis/*immunology MH - DNA-Binding Proteins/*immunology MH - Female MH - Humans MH - Ku Autoantigen MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/*diagnosis/*immunology MH - Raynaud Disease/etiology EDAT- 2016/05/11 06:00 MHDA- 2016/06/17 06:00 CRDT- 2016/05/11 06:00 PHST- 2016/05/11 06:00 [entrez] PHST- 2016/05/11 06:00 [pubmed] PHST- 2016/06/17 06:00 [medline] PST - ppublish SO - Wiad Lek. 2016;69(1):24-6. PMID- 28578493 OWN - NLM STAT- MEDLINE DCOM- 20180719 LR - 20260127 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 36 IP - 9 DP - 2017 Sep TI - Nailfold videocapillaroscopy results in patients with rheumatoid arthritis. PG - 1969-1974 LID - 10.1007/s10067-017-3696-4 [doi] AB - We aimed to analyse the nailfold capillaryscopy findings morphologically and examine their relationship with disease activity and demographic characteristics in patients with rheumatoid arthritis. In accordance with the 2010 ACR/EULAR classification criteria, 201 patients diagnosed with Romatoiad artrit (RA) and 50 healthy controls were included. We analysed capillaroscopic abnormalities such asmegacapillaries, haemorrhages, ramifications and avascular areas in patients affected with rheumatoid arthritis. The findings in our study are as follows: in 45.77% of the RA patients, there were nonspecific capillaryscopy findings. When compared to control group, the incidence of tortuosity, dilated capillary and bushy capillary was higher in RA patients (p values, respectively, 0.110, 0.330, 0.440 and 0.516). In RA patients with Raynaud's phenomenon, the incidence of nonspecific capillaryscopy findings was higher. While there is a weak relationship between tortuosity and the duration of disease, no significant relation was detected between capillaryscopy findings and parameters such as RF, anti-CCP positivity and disease activity score (DAS28). When compared to controls, we have detected that RA patients have more nonspecific capillaryscopic findings. We could not find a relationship between nonspecific capillaryscopic findings and RA'a clinical findings and laboratory parameters. There is a need for a long-term wider-scale follow-up study to investigate whether there is a capillaryscopic pattern that can be correlated with RA's clinical findings. FAU - Sag, Sinem AU - Sag S AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. drsinemyamac@yahoo.com. FAU - Sag, Mustafa Serdar AU - Sag MS AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. FAU - Tekeoglu, Ibrahim AU - Tekeoglu I AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. FAU - Kamanli, Ayhan AU - Kamanli A AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. FAU - Nas, Kemal AU - Nas K AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. FAU - Aydın, Yıldıray AU - Aydın Y AD - Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Sakarya University Medical Faculty, Sakarya, Turkey. LA - eng PT - Journal Article DEP - 20170604 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Arthritis, Rheumatoid/*diagnostic imaging MH - Capillaries/*diagnostic imaging MH - Case-Control Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/complications MH - Severity of Illness Index MH - Turkey OTO - NOTNLM OT - DAS28 OT - Nailfold capillaroscopy OT - capillaryscopic pattern EDAT- 2017/06/05 06:00 MHDA- 2018/07/20 06:00 CRDT- 2017/06/05 06:00 PHST- 2017/05/10 00:00 [received] PHST- 2017/05/19 00:00 [accepted] PHST- 2017/05/17 00:00 [revised] PHST- 2017/06/05 06:00 [pubmed] PHST- 2018/07/20 06:00 [medline] PHST- 2017/06/05 06:00 [entrez] AID - 10.1007/s10067-017-3696-4 [pii] AID - 10.1007/s10067-017-3696-4 [doi] PST - ppublish SO - Clin Rheumatol. 2017 Sep;36(9):1969-1974. doi: 10.1007/s10067-017-3696-4. Epub 2017 Jun 4. PMID- 16733438 OWN - NLM STAT- MEDLINE DCOM- 20060623 LR - 20191110 IS - 0398-0499 (Print) IS - 0398-0499 (Linking) VI - 31 IP - 2 DP - 2006 May TI - [Sub acute ischemia of a lower limb in a patient with juvenile peripheral arterial disease and arterial cocaine toxicity]. PG - 76-8 AB - A 35-year-old woman was hospitalized for subacute ischemia of the left leg following an intermittent claudication for some weeks. She also presented paleness and coldness of both hands. The radial pulses could not be palpated. Smoking was the only cardiovascular risk factor. Duplex ultrasonography and angiography revealed a left popliteal thrombus combined with low diameter leg arteries and in the upper limbs stenosis of the left radial artery and thrombosis of the right radial artery. Search for a metabolic, embolic or thrombophilic etiology was negative. More minute history taking revealed use of cannabis and recent nasal administration of cocaine. Her condition improved with heparin therapy except for the upper limbs with ischemia of the hands and disabling Raynaud's phenomenon. This report highlights the combined arterial toxicity of drugs often used together by drug addicts. The association of cannabis use and tobacco smoking is not rare in patients with Buerger-like juvenile arteriopathy and cocaine may provoke peripheral vascular disease by embolism or in situ thrombosis. Interrogation of a patient presenting with Buerger-like peripheral arterial disease should insist on detecting use of drugs in association with tobacco smoking. FAU - Seinturier, C AU - Seinturier C AD - Service de Médecine Vasculaire, CHU Michallon, Grenoble. cseinturier@chu-grenoble.fr FAU - Pichot, O AU - Pichot O FAU - Blaise, S AU - Blaise S FAU - Imbert, B AU - Imbert B FAU - Carpentier, P AU - Carpentier P LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Ischémie subaiguë d'un membre inférieur et toxicité artérielle périphérique de la cocaïne chez une patiente présentant une artériopathie juvénile. PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 RN - 9005-49-6 (Heparin) RN - I5Y540LHVR (Cocaine) SB - IM MH - Adult MH - Cannabis/adverse effects MH - Cocaine/*toxicity MH - Constriction, Pathologic MH - Female MH - Hand/blood supply MH - Heparin/therapeutic use MH - Humans MH - *Ischemia/chemically induced MH - Leg/*blood supply MH - Popliteal Artery MH - Radial Artery MH - Raynaud Disease/complications MH - Smoking/adverse effects MH - Thrombosis/chemically induced/diagnosis/drug therapy MH - Vascular Diseases/*chemically induced/drug therapy EDAT- 2006/05/31 09:00 MHDA- 2006/06/24 09:00 CRDT- 2006/05/31 09:00 PHST- 2006/05/31 09:00 [pubmed] PHST- 2006/06/24 09:00 [medline] PHST- 2006/05/31 09:00 [entrez] AID - MDOI-JMV-05-2006-31-2-0398-0499-101019-200517834 [pii] AID - 10.1016/s0398-0499(06)76522-2 [doi] PST - ppublish SO - J Mal Vasc. 2006 May;31(2):76-8. doi: 10.1016/s0398-0499(06)76522-2. PMID- 24294139 OWN - NLM STAT- MEDLINE DCOM- 20140624 LR - 20260128 IS - 1537-744X (Electronic) IS - 2356-6140 (Print) IS - 1537-744X (Linking) VI - 2013 DP - 2013 TI - Common and specific associations of anti-SSA/Ro60 and anti-Ro52/TRIM21 antibodies in systemic lupus erythematosus. PG - 832789 LID - 10.1155/2013/832789 [doi] LID - 832789 AB - Little information exists about the association of anti-SSA/Ro60 and anti-Ro52/TRIM21 with systemic lupus erytematosus (SLE) features. In this work, we analysed the associations of both anti-Ro reactivities with clinical and immunological manifestations in 141 SLE patients. Photosensitivity and xerophtalmia/xerostomia were found to be positively associated with both anti-SSA/Ro60 (P = 0.024 and P = 0.019, resp.) and anti-Ro52/TRIM21 (P = 0.026 and P = 0.022, resp.). In contrast, a negative association was detected regarding anti-phospholipid antibodies, anti-SSA/Ro60 having a stronger effect (P = 0.014) than anti-Ro52/TRIM21. Anti-SSA/Ro60 showed a specific positive association with hypocomplementemia (P = 0.041), mainly with low C4 levels (P = 0.008), whereas anti-Ro52/TRIM21 was found to be positively associated with Raynaud's phenomenon (P = 0.026) and cytopenia (P = 0.048) and negatively associated with anti-dsDNA (P = 0.013). Lymphocytes are involved in the relationship between anti-Ro52/TRIM21 and cytopenia since positive patients showed lower cell levels than negative patients (P = 0.036). In conclusion, anti-SSA/Ro60 and anti-Ro52/TRIM21 showed both common and specific associations in SLE. These data thus increase evidence of the different associations of the two anti-Ro specificities even in a particular disease. FAU - Menéndez, Aurora AU - Menéndez A AD - Department of Immunology, Hospital Universitario Central de Asturias, Celestino Villamil S/N, 33006 Oviedo, Spain. FAU - Gómez, Jesús AU - Gómez J FAU - Caminal-Montero, Luis AU - Caminal-Montero L FAU - Díaz-López, José Bernardino AU - Díaz-López JB FAU - Cabezas-Rodríguez, Iván AU - Cabezas-Rodríguez I FAU - Mozo, Lourdes AU - Mozo L LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131030 PL - United States TA - ScientificWorldJournal JT - TheScientificWorldJournal JID - 101131163 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Complement C3) RN - 0 (Complement C4) RN - 0 (Lupus Coagulation Inhibitor) RN - 0 (Ribonucleoproteins) RN - 0 (SS-A Antigen) RN - 0 (SS-A antibodies) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood/*immunology MH - Antibodies, Antiphospholipid/blood MH - Complement C3/deficiency MH - Complement C4/deficiency MH - Female MH - Humans MH - Lupus Coagulation Inhibitor/analysis MH - Lupus Erythematosus, Systemic/blood/complications/*immunology MH - Lymphopenia/etiology/immunology MH - Male MH - Oral Ulcer/etiology/immunology MH - Phenotype MH - Photosensitivity Disorders/etiology/immunology MH - Raynaud Disease/etiology/immunology MH - Ribonucleoproteins/*immunology MH - Xerophthalmia/etiology/immunology MH - Xerostomia/etiology/immunology MH - Young Adult MH - SS-A Antigen PMC - PMC3833022 EDAT- 2013/12/03 06:00 MHDA- 2014/06/25 06:00 PMCR- 2013/10/30 CRDT- 2013/12/03 06:00 PHST- 2013/08/01 00:00 [received] PHST- 2013/09/16 00:00 [accepted] PHST- 2013/12/03 06:00 [entrez] PHST- 2013/12/03 06:00 [pubmed] PHST- 2014/06/25 06:00 [medline] PHST- 2013/10/30 00:00 [pmc-release] AID - 10.1155/2013/832789 [doi] PST - epublish SO - ScientificWorldJournal. 2013 Oct 30;2013:832789. doi: 10.1155/2013/832789. eCollection 2013. PMID- 37450876 OWN - NLM STAT- MEDLINE DCOM- 20230717 LR - 20250910 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 91 IP - 1 DP - 2023 Jul 1 TI - The Use of Botulinum Toxin in Raynaud Phenomenon: A Comprehensive Literature Review. PG - 159-186 LID - 10.1097/SAP.0000000000003603 [doi] AB - BACKGROUND: Raynaud phenomenon (RP) is a vasospastic condition of the digits that can be primary or secondary to systemic disease. Symptoms are triggered by cold or stress and can cause pain and skin color changes. The chronic ischemia may lead to necrosis, ulceration, and amputation. There are no Food and Drug Administration-approved treatments and cases refractory to pharmacologic and surgical treatments are difficult to control. Local botulinum toxin injections have been increasingly used in the treatment of Raynaud disease and have shown promising results. AIM OF THE STUDY: To examine the outcomes, techniques, and complications of botulinum toxin use for the treatment of Raynaud disease. METHODS: The PubMed database was queried using "botulinum toxin" AND "Raynaud phenomenon" as title key words. Preferred reporting items for systematic reviews and meta-analysis criteria were used. Additional articles were selected while reviewing the references of the articles from PubMed. No time restrictions were followed. Articles of all languages were included. Articles were analyzed for study type, demographics, diagnosis/inclusion criteria, treatment methods, outcome measures, length of follow-up, results, and complications. A positive outcome was defined as subjective improvement in symptoms and/or improvement in the outcome measures. A poor outcome was defined as harm done to the patient by the injection that would not have occurred otherwise. RESULTS: Forty-two clinical studies describing the use of botulinum toxin for Raynaud's phenomenon were found. A total of 425 patients with primary or secondary Raynaud's were treated, with ages ranging from 14 to 91 years. There were 342 women and 81 men, with a female-to-male ratio of 38:9. Outcomes were positive in 96.2% of patients. There were 14.2% of the studies that reported 3.5% of all patients showing no subjective improvement. A single study reported a poor outcome for 1 patient. There were 40.5% of the studies that reported complications, affecting 20.2% of all patients. The most frequently reported complication was transient hand weakness, affecting 44.2% of patients with complications and 8.9% of total patients. Weakness resolved in hours to months after injection. Pain at the injection site lasting minutes to days was reported in 40.7% of patients with complications, and 8.2% of total patients. CONCLUSIONS: Botulinum toxin treatment for RP is effective. Complications are minor and self-limiting. CI - Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved. FAU - Lawson, Olivia AU - Lawson O AD - University of Tennessee Health Science Center, College of Medicine, Memphis, TN FAU - Sisti, Andrea AU - Sisti A AD - Division of General Surgery, Department of Surgery, University of Texas Medical Branch, Galveston, TX AD - Shriners Hospital for Children, Galveston, TX FAU - Konofaos, Petros AU - Konofaos P AD - Division of Plastic Surgery, Department of Surgery, University of Texas Medical Branch, Galveston, TX LA - eng PT - Journal Article PT - Review PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Neuromuscular Agents) SB - IM MH - Humans MH - Male MH - Female MH - *Botulinum Toxins, Type A/therapeutic use MH - *Neuromuscular Agents/therapeutic use MH - Injections/adverse effects MH - Pain MH - *Raynaud Disease/etiology COIS- Conflicts of interest and sources of funding: None declared. EDAT- 2023/07/14 19:07 MHDA- 2023/07/17 06:42 CRDT- 2023/07/14 16:33 PHST- 2023/07/17 06:42 [medline] PHST- 2023/07/14 19:07 [pubmed] PHST- 2023/07/14 16:33 [entrez] AID - 00000637-202307000-00028 [pii] AID - 10.1097/SAP.0000000000003603 [doi] PST - ppublish SO - Ann Plast Surg. 2023 Jul 1;91(1):159-186. doi: 10.1097/SAP.0000000000003603. PMID- 19048465 OWN - NLM STAT- MEDLINE DCOM- 20090416 LR - 20160526 IS - 0743-684X (Print) IS - 0743-684X (Linking) VI - 25 IP - 2 DP - 2009 Feb TI - Periarterial sympathectomy of the foot for the treatment of necrotizing Raynaud's phenomena. PG - 133-7 LID - 10.1055/s-0028-1103511 [doi] AB - Patients with critical peripheral vascular disease and nonhealing toe ulcers secondary to collagen vascular disease often require toe amputation when nonsurgical measures fail to control their symptoms. The aim of this study was to evaluate the effectiveness of periarterial sympathectomy (PAS) of the foot/ankle in patients with unreconstructable vaso-occlusive disease and nonhealing digit ulcers unresponsive to nonsurgical measures. Five patients (seven feet and nine toe ulcers) were treated with PAS of their involved foot and followed for a minimal of 3 years (3 to 7 years). The technique included PAS of the dorsalis pedis, posterior tibial and anterior tibial arteries. Eight toe ulcers healed uneventfully within 3 months after surgery. Only one patient developed a new ulcer 5 years after PAS. The mean visual analog pain scale improved from 5.7 to 1 (P<0.03). The mean Wake Forest University (WFU) scale improved from 1.8 to 0.5 (P=0.07). There was no change in the WFU numbness score or the McCabe Cold sensitivity scale. The results suggest that foot/ankle PAS is a beneficial salvage treatment option for patients with non-reconstructable, vaso-occlusive disease that ameliorates foot symptoms, facilitates healing of toe ulcerations, and reduces the incidence of toe amputations. FAU - Li, Zhongyu AU - Li Z AD - Department of Orthopaedic Surgery, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157-1070, USA. zli@wfubmc.edu FAU - Smith, Beth Paterson AU - Smith BP FAU - Holden, Martha AU - Holden M FAU - Koman, L Andrew AU - Koman LA LA - eng PT - Journal Article DEP - 20081201 PL - United States TA - J Reconstr Microsurg JT - Journal of reconstructive microsurgery JID - 8502670 SB - IM MH - Adolescent MH - Adult MH - Analysis of Variance MH - Female MH - Foot/blood supply/innervation MH - Foot Ulcer/*surgery MH - Humans MH - Male MH - Middle Aged MH - Necrosis MH - Quality of Life MH - Raynaud Disease/*surgery MH - Sympathectomy/*methods MH - Treatment Outcome EDAT- 2008/12/03 09:00 MHDA- 2009/04/17 09:00 CRDT- 2008/12/03 09:00 PHST- 2008/12/03 09:00 [pubmed] PHST- 2009/04/17 09:00 [medline] PHST- 2008/12/03 09:00 [entrez] AID - 10.1055/s-0028-1103511 [doi] PST - ppublish SO - J Reconstr Microsurg. 2009 Feb;25(2):133-7. doi: 10.1055/s-0028-1103511. Epub 2008 Dec 1. PMID- 19255934 OWN - NLM STAT- MEDLINE DCOM- 20100302 LR - 20151119 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 38 IP - 4 DP - 2009 TI - Enhanced interleukin-10 production by dendritic cells upon stimulation with Toll-like receptor 4 agonists in systemic sclerosis that is possibly implicated in CCL18 secretion. PG - 282-90 LID - 10.1080/03009740802572467 [doi] AB - BACKGROUND: It has been suggested that the T-cell attracting and profibrotic chemokine CCL18 might play a role in the pathogenesis of systemic sclerosis (SSc). However, it is unclear what underlies the higher CCL18 levels in SSc. The aim of our study was to determine whether Toll-like receptor (TLR)-mediated stimulation of monocytes and dendritic cells (DCs) contributes to the higher levels of CCL18 in SSc. METHODS: CCL18 levels were measured in 40 patients with SSc, primary Raynaud's phenomenon (RP) and healthy controls. The presence of TLR4 agonists in the circulation of SSc patients was investigated using TLR4 transgenic Chinese hamster ovary (CHO) cells. CCL18 and interleukin (IL)-10 secretion by monocytes/macrophages and monocyte-derived DCs (moDCs) was measured in the supernatant. The indirect effect of lipopolysaccharide (LPS)-stimulated moDCs on CCL18 secretion by monocytes/macrophages was investigated using a transwell system. RESULTS: CCL18 levels were significantly elevated in SSc patients compared to patients with RP and healthy controls. SSc sera strongly induced CD25 expression on CHO cells genetically modified to express TLR4 but not on those expressing CD14 only. By contrast, serum from systemic lupus erythematosus (SLE) patients or healthy individuals did not have an effect. Neither monocytes/macrophages nor moDCs from SSc patients secreted higher levels of CCL18 compared to healthy controls. However, moDCs matured with the TLR4 ligand LPS from patients with SSc did secrete significantly higher amounts of IL-10 compared to those from healthy counterparts, which were IL-10 dependent. CONCLUSIONS: Our results suggest that elevated CCL18 levels in SSc are not caused by an intrinsically enhanced CCL18 secretion by monocytes/macrophages but are, at least partly, orchestrated by an enhanced IL-10 secretion by TLR4-stimulated DCs. These observations suggest a role for TLR4 ligands and DCs in the pathogenesis of SSc, a topic that warrants further investigation. FAU - van Lieshout, A W T AU - van Lieshout AW AD - Department of Rheumatology, Radboud University Nijmegen Medical Centre, the Netherlands. FAU - Vonk, M C AU - Vonk MC FAU - Bredie, S J H AU - Bredie SJ FAU - Joosten, L B A AU - Joosten LB FAU - Netea, M G AU - Netea MG FAU - van Riel, P L C M AU - van Riel PL FAU - Lafyatis, R AU - Lafyatis R FAU - van den Hoogen, F H J AU - van den Hoogen FH FAU - Radstake, T R D J AU - Radstake TR LA - eng PT - Comparative Study PT - Journal Article PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Biomarkers) RN - 0 (CCL18 protein, human) RN - 0 (Chemokines, CC) RN - 0 (Cytokines) RN - 0 (Toll-Like Receptor 4) RN - 130068-27-8 (Interleukin-10) SB - IM MH - Animals MH - Biomarkers/blood MH - Case-Control Studies MH - Cells, Cultured MH - Chemokines, CC/genetics/*metabolism MH - Cricetinae MH - Cytokines/metabolism MH - Dendritic Cells/drug effects/*immunology MH - Female MH - Humans MH - Interleukin-10/*immunology/metabolism MH - Male MH - Monocytes/drug effects/immunology MH - Probability MH - Raynaud Disease/blood/*immunology/physiopathology MH - Reference Values MH - Scleroderma, Systemic/blood/*immunology/physiopathology MH - Sensitivity and Specificity MH - Statistics, Nonparametric MH - Toll-Like Receptor 4/agonists EDAT- 2009/03/04 09:00 MHDA- 2010/03/03 06:00 CRDT- 2009/03/04 09:00 PHST- 2009/03/04 09:00 [entrez] PHST- 2009/03/04 09:00 [pubmed] PHST- 2010/03/03 06:00 [medline] AID - 909181712 [pii] AID - 10.1080/03009740802572467 [doi] PST - ppublish SO - Scand J Rheumatol. 2009;38(4):282-90. doi: 10.1080/03009740802572467. PMID- 36746531 OWN - NLM STAT- MEDLINE DCOM- 20230209 LR - 20240912 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 1 DP - 2023 Feb TI - Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies. LID - 10.1136/rmdopen-2022-002827 [doi] LID - e002827 AB - INTRODUCTION: Early diagnosis of systemic sclerosis (SSc) is important to start therapeutic interventions timely. Important risk factors for progression to SSc are the SSc-specific autoantibodies, of whom anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (ATA) are the most frequent. ATA is associated with a severe disease course. A more detailed characterisation of the ATA-response in SSc might increase insights in preclinical disease stages and improve prognostication. To address this we identified all patients with suspected very early ATA-positive SSc, defined as all patients who are ATA-positive not fulfilling American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria, in the Leiden Combined Care in Systemic Sclerosis (CCISS)-cohort and found very low numbers. METHODS: This triggered us to search the literature on the ATA prevalence in patients with suspected very early SSc and contribution of the SSc-specific autoantibodies to progression from suspected very early to definite SSc. To increase insights on the ATA-response in suspected very early SSc, we then evaluated the association between the ATA-response and time between onset of Raynaud's phenomenon (RP) and first non-RP symptom, as a proxy for progressing to definite SSc, in all patients with ATA-positive SSc from the Leiden CCISS-cohort. RESULTS: In short, included studies show that prevalence of ATA is much lower in suspected very early SSc than in populations fulfilling ACR/EULAR 2013 criteria. After 1-15 years of follow-up, only 52% of the patients with suspected very early SSc progress to definite SSc. ATA-IgG levels tend to be higher in patients with ATA-positive SSc with more rapid disease progression. CONCLUSION: Although a role of ATA in disease progression is suggested, more studies on the ATA response in suspected very early SSc are warranted. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Liem, Sophie I E AU - Liem SIE AUID- ORCID: 0000-0003-0328-7062 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands S.I.E.Liem@lumc.nl. FAU - Neppelenbroek, Sam AU - Neppelenbroek S AUID- ORCID: 0000-0002-9582-2837 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Fehres, Cynthia M AU - Fehres CM AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Wevers, Brigitte A AU - Wevers BA AD - Department of Clinical Chemistry and Laboratory Medicine, Leiden University Medical Center, Leiden, The Netherlands. FAU - Toes, René E M AU - Toes REM AUID- ORCID: 0000-0002-9618-6414 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Allaart, Cornelia F AU - Allaart CF AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Huizinga, Tom W J AU - Huizinga TWJ AUID- ORCID: 0000-0001-7033-7520 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Scherer, Hans Ulrich AU - Scherer HU AUID- ORCID: 0000-0002-5700-5617 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - De Vries-Bouwstra, Jeska K AU - De Vries-Bouwstra JK AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. LA - eng PT - Journal Article PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Autoantibodies) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Humans MH - *Autoantibodies MH - Disease Progression MH - DNA Topoisomerases, Type I MH - *Raynaud Disease/diagnosis/epidemiology MH - *Scleroderma, Systemic/complications MH - United States PMC - PMC9906376 OTO - NOTNLM OT - Autoantibodies OT - Autoimmunity OT - Scleroderma, Systemic COIS- Competing interests: None declared. EDAT- 2023/02/07 06:00 MHDA- 2023/02/09 06:00 PMCR- 2023/02/06 CRDT- 2023/02/06 20:52 PHST- 2022/11/01 00:00 [received] PHST- 2023/01/11 00:00 [accepted] PHST- 2023/02/06 20:52 [entrez] PHST- 2023/02/07 06:00 [pubmed] PHST- 2023/02/09 06:00 [medline] PHST- 2023/02/06 00:00 [pmc-release] AID - rmdopen-2022-002827 [pii] AID - 10.1136/rmdopen-2022-002827 [doi] PST - ppublish SO - RMD Open. 2023 Feb;9(1):e002827. doi: 10.1136/rmdopen-2022-002827. PMID- 31735790 OWN - NLM STAT- MEDLINE DCOM- 20200914 LR - 20200914 IS - 1349-7235 (Electronic) IS - 0918-2918 (Print) IS - 0918-2918 (Linking) VI - 59 IP - 5 DP - 2020 Mar 1 TI - Drop Head Syndrome as a Rare Complication in Mixed Connective Tissue Disease. PG - 729-732 LID - 10.2169/internalmedicine.3626-19 [doi] AB - A 54-year-old woman developed drop head syndrome (DHS), Raynaud's phenomenon and creatine kinase (CK) elevation. She did not meet the international classification criteria of dermatomyositis/polymyositis, as we observed no muscle weakness, grasping pain or electromyography abnormality in her limbs, and anti-aminoacyl tRNA synthetase (ARS) antibody was negative. Cervical magnetic resonance imaging and a muscle biopsy of the trapezius muscle revealed myositis findings as the only clinical observations in muscle. These findings, along with her anti-U1-ribonucleoprotein (RNP) antibody positivity and leukopenia, resulted in a diagnosis of mixed connective tissue disease (MCTD). Prednisolone treatment significantly improved her myositis. To our knowledge, this is the first report of DHS as the only muscle complication of MCTD. FAU - Akagi, Midori AU - Akagi M AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Umeda, Masataka AU - Umeda M AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. AD - Medical Education Development Center, Nagasaki University Hospital, Japan. FAU - Hashisako, Mikiko AU - Hashisako M AD - Department of Anatomic Pathology, Graduate School of Medicine Sciences, Kyushu University, Japan. FAU - Hara, Kazusato AU - Hara K AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Tsuji, Sousuke AU - Tsuji S AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Endo, Yushiro AU - Endo Y AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Takatani, Ayuko AU - Takatani A AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Shimizu, Toshimasa AU - Shimizu T AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Fukui, Shoichi AU - Fukui S AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Koga, Tomohiro AU - Koga T AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Kawashiri, Shin-Ya AU - Kawashiri SY AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Iwamoto, Naoki AU - Iwamoto N AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Igawa, Takashi AU - Igawa T AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Ichinose, Kunihiro AU - Ichinose K AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Tamai, Mami AU - Tamai M AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Nakamura, Hideki AU - Nakamura H AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. FAU - Origuchi, Tomoki AU - Origuchi T AD - Department of Physical Therapy Sciences, Graduate School of Biomedical Sciences, Nagasaki University, Japan. FAU - Niino, Daisuke AU - Niino D AD - Nagasaki Educational and Diagnostic Center of Pathology, Nagasaki University Hospital, Japan. FAU - Kawakami, Atsushi AU - Kawakami A AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20191118 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) RN - EC 2.7.3.2 (Creatine Kinase) SB - IM MH - Antibodies, Antinuclear/blood MH - Creatine Kinase/blood MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications/drug therapy MH - Muscle Weakness/*etiology MH - Myositis/drug therapy MH - Neck Muscles/*pathology MH - Prednisolone/therapeutic use MH - Raynaud Disease/complications PMC - PMC7086316 OTO - NOTNLM OT - drop head syndrome OT - mixed connective tissue disease OT - myositis OT - neck extensor muscles COIS- The authors state that they have no Conflict of Interest (COI). EDAT- 2019/11/19 06:00 MHDA- 2020/09/15 06:00 PMCR- 2020/03/01 CRDT- 2019/11/19 06:00 PHST- 2019/11/19 06:00 [pubmed] PHST- 2020/09/15 06:00 [medline] PHST- 2019/11/19 06:00 [entrez] PHST- 2020/03/01 00:00 [pmc-release] AID - 10.2169/internalmedicine.3626-19 [doi] PST - ppublish SO - Intern Med. 2020 Mar 1;59(5):729-732. doi: 10.2169/internalmedicine.3626-19. Epub 2019 Nov 18. PMID- 32067722 OWN - NLM STAT- MEDLINE DCOM- 20210427 LR - 20210427 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 50 IP - 3 DP - 2020 Jun TI - Prevalence and clinical significance of extra-articular manifestations at diagnosis in the ESPOIR cohort with recent-onset arthritis. PG - 409-413 LID - S0049-0172(20)30004-4 [pii] LID - 10.1016/j.semarthrit.2020.01.004 [doi] AB - OBJECTIVES: The aim of this study was to determine the prevalence and clinical significance of extra-articular manifestations (EAMs) at inclusion into a cohort of patients with recent-onset arthritis consistent with rheumatoid arthritis (RA). METHODS: The ESPOIR cohort included patients aged 18 to 70 years who had a definitive or probable diagnosis of RA. Symptoms consistent with EAMs were collected at baseline. We divided the patients into two groups, with vs. without baseline EAMs. We looked for associations linking the presence of EAMs at baseline to patient and disease characteristics at baseline and 5 years later, as well as to diagnostic certainty after 2 years. The analyses were adjusted for multiple comparisons using the Benjamini-Hochberg procedure to control the false discovery rate. RESULTS: Of 798 patients, 330 (41.4%) had at least one symptom consistent with EAM at baseline, with the most common being sicca syndrome (28.4%) and Raynaud's phenomenon (17.3%). The EAM+ group had a higher mean baseline DAS-28 value (5.3 ± 1.3 versus 5.0 ± 1.3; corrected p value = 0.005) compared to the EAM- group. The final diagnosis did not differ between the two groups. After 5 years, the EAM+ group had significantly higher values for the tender joint count (3.9 ± 6.4 versus 1.8 ± 3.3, corrected p value = 0.005) and swollen joint count (1.3 ± 2.8 versus 1.1 ± 2.3, corrected p value =0.0005) compared to the EAM- group. CONCLUSION: EAMs, particularly sicca syndrome and Raynaud's phenomenon, are very common in patients with early arthritis consistent with RA. In this population, several parameters reflecting disease activity were higher among patients with EAMs, at baseline and after 5 years. CI - Copyright © 2020 Elsevier Inc. All rights reserved. FAU - Guellec, Dewi AU - Guellec D AD - Service de rhumatologie, CHU Brest, INSERM CIC 1412, Brest, France. FAU - Cozien, Servane AU - Cozien S AD - Servane Cozien, Service de rhumatologie, CHU Brest, Brest, France. FAU - Ruyssen-Witrand, Adeline AU - Ruyssen-Witrand A AD - Adeline Ruyssen-Witrand, Centre de rhumatologie, Hôpital Pierre-Paul-Riquet, INSERM UMR 1027, Université Paul-Sabatier Toulouse III, Toulouse, France. FAU - Dieudé, Philippe AU - Dieudé P AD - Philippe Dieudé, Service de Rhumatologie, Hôpital Bichat, APHP, Université Paris Diderot, 46 rue Henri Huchard, 75018 Paris, France. FAU - Saraux, Alain AU - Saraux A AD - Service de rhumatologie, CHU Brest, INSERM 1227, Université de Bretagne Occidentale, LabEx IGO, Brest, France. Electronic address: alain.saraux@chu-brest.fr. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20200127 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Arthritis, Rheumatoid/*epidemiology MH - Case-Control Studies MH - Female MH - France/epidemiology MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Prevalence MH - Prospective Studies MH - Raynaud Disease/*epidemiology/etiology MH - Rheumatoid Nodule/epidemiology/etiology MH - Risk Factors MH - Sex Distribution MH - Sjogren's Syndrome/*epidemiology/etiology OTO - NOTNLM OT - Cohort study OT - Extra-articular manifestations OT - Rheumatoid arthritis COIS- Declaration of Competing Interest None. EDAT- 2020/02/19 06:00 MHDA- 2021/04/28 06:00 CRDT- 2020/02/19 06:00 PHST- 2019/10/10 00:00 [received] PHST- 2020/01/13 00:00 [revised] PHST- 2020/01/21 00:00 [accepted] PHST- 2020/02/19 06:00 [pubmed] PHST- 2021/04/28 06:00 [medline] PHST- 2020/02/19 06:00 [entrez] AID - S0049-0172(20)30004-4 [pii] AID - 10.1016/j.semarthrit.2020.01.004 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2020 Jun;50(3):409-413. doi: 10.1016/j.semarthrit.2020.01.004. Epub 2020 Jan 27. PMID- 20521412 OWN - NLM STAT- MEDLINE DCOM- 20100812 LR - 20180109 IS - 1049-510X (Print) IS - 1049-510X (Linking) VI - 20 IP - 1 Suppl 1 DP - 2010 Winter TI - Clinical and sociodemographic features of Puerto Ricans with systemic sclerosis. PG - S1-185-9 AB - INTRODUCTION: Systemic sclerosis is an autoimmune disease of unknown etiology characterized by fibrotic changes in the skin, blood vessels, and various internal organs. The disease has a wide spectrum of presentation and a variable clinical course that includes limited skin involvement to life-threatening disease. Clinical manifestations and disease severity differs among ethnic groups. The objective of this study is to describe the clinical and sociodemographic features of patients with well-characterized systemic sclerosis from Puerto Rico. METHODS: A structured questionnaire was completed for each patient to gather information about demographic factors, clinical manifestations, laboratory findings, diagnostic studies, and pharmacologic treatments. RESULTS: Of the 24 patients with systemic sclerosis, 96% were females, 83% had Raynaud's phenomenon, 67% had gastrointestinal involvement, 63% had skin hypopigmentation, 50% had digital pitting scars, 46% had arterial hypertension, 11% had pulmonary hypertension, and 4.8% had renal involvement. The overall median modified Rodnan skin score was 24.5 (inter-quartile range 16.0-31.3). Pulmonary function tests resulted in abnormal in 60% of 14 patients, of which 57% had restrictive lung disease (FVC < 70%) and 42.9% had decreased diffusion capacity. Serologically, 66.7% were positive for antinuclear antibody and 62.5% were positive for anti-centromere. CONCLUSIONS: In this study, the predominant clinical features of Puerto Ricans with systemic sclerosis were gastrointestinal involvement, Raynaud's phenomenon, digital pitting scars, and lung disease. Patients had a moderate severity of skin disease. The presence of renal involvement and pulmonary hypertension were low in our group. No significant differences were found between systemic sclerosis disease subsets. FAU - Ríos, Grissel AU - Ríos G AD - Division of Rheumatology Office 824; MCS University of Puerto Rico, P.O. Box 365067, San Juan. grissel.rios@upr.edu FAU - Mayor, Angel M AU - Mayor AM LA - eng GR - 1P20-RR11126/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Ethn Dis JT - Ethnicity & disease JID - 9109034 SB - IM MH - Adult MH - Cross-Sectional Studies MH - Female MH - Humans MH - Lung Diseases/etiology MH - Male MH - Middle Aged MH - Puerto Rico/epidemiology MH - Raynaud Disease/etiology MH - Respiratory Function Tests MH - Scleroderma, Systemic/complications/*diagnosis/*ethnology/physiopathology EDAT- 2010/06/04 06:00 MHDA- 2010/08/13 06:00 CRDT- 2010/06/04 06:00 PHST- 2010/06/04 06:00 [entrez] PHST- 2010/06/04 06:00 [pubmed] PHST- 2010/08/13 06:00 [medline] PST - ppublish SO - Ethn Dis. 2010 Winter;20(1 Suppl 1):S1-185-9. PMID- 17067982 OWN - NLM STAT- MEDLINE DCOM- 20061130 LR - 20061027 IS - 0003-3197 (Print) IS - 0003-3197 (Linking) VI - 57 IP - 5 DP - 2006 Oct-Nov TI - Nailfold videocapillaroscopy in primary Sjögren's syndrome. PG - 593-9 AB - Nailfold videocapillaroscopy was performed in 2 groups of subjects: 14 healthy volunteers (C) and 15 patients with primary Sjögren's syndrome (PSS). This was a controlled clinical trial, matched by age and sex. The aims of this study were to evaluate (1) functional capillary density (number of capillaries with flowing red blood cells per mm(2), FCD); (2) capillary red blood cell velocity at rest (RBV), maximum capillary red blood cell velocity (RBV(max)) after 1 minute ischemia, and the time to reach it (TRBV(max)), taking into account the presence or absence of Raynaud's phenomenon (RP) in the analysis; (3) nailfold capillary morphology; and (4) afferent (AFD), apical (APD), and efferent (EFD) capillary diameters. The mean values obtained for controls versus patients, respectively, were (mean +/- SD): FCD (per mm(2)) 8.0 +/-1.6 and 10.1 +/-3.6; RBV (mm/s) 0.9 +/-0.4 and 0.7 +/-0.2; RBV(max) (mm/s) 1.7 +/-0.9 and 1.3 +/-0.3; TRBV(max) (s) 4.5 +/-0.8 and 5.8 +/-1.6 (p=0.02); and TRBV(max) (s) in patients with RP=6.7 +/-1.6 and without RP=5.6 +/-1.6 (p=0.52). The correlation between RBV and RBV(max) for each group, using the Pearson's coefficient, was significant only for the control group (p=0.007), estimated correlation coefficient = 0.68. Controls and patients showed, in the majority of fields examined, normal morphologic patterns of capillaries. The capillary diameters were AFD (mum) 10.8 +/-1.5 and 11.3 +/-1.8; APD (mum) 16.3 +/-2.4 and 16.8 +/-2.9; and EFD (mum) 12.3 +/-1.4 and 13.7 +/-1.9. These results indicate that these patients have longer time to reach RBV(max), suggesting an impairment of the reactive hyperemia response, which could correlate with clinical features of the disease, ie, abnormal macrovascular and microvascular reactivity. FAU - Aguiar, Thula AU - Aguiar T AD - Laboratory for Research in Microcirculation, State University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil. FAU - Furtado, Eliane AU - Furtado E FAU - Dorigo, David AU - Dorigo D FAU - Bottino, Daniel AU - Bottino D FAU - Bouskela, Eliete AU - Bouskela E LA - eng PT - Journal Article PL - United States TA - Angiology JT - Angiology JID - 0203706 SB - IM MH - Adult MH - Blood Flow Velocity MH - Capillaries/pathology MH - Female MH - Humans MH - Male MH - Microcirculation MH - Microscopy, Video MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/complications/physiopathology MH - Sjogren's Syndrome/complications/*physiopathology EDAT- 2006/10/28 09:00 MHDA- 2006/12/09 09:00 CRDT- 2006/10/28 09:00 PHST- 2006/10/28 09:00 [pubmed] PHST- 2006/12/09 09:00 [medline] PHST- 2006/10/28 09:00 [entrez] AID - 57/5/593 [pii] AID - 10.1177/0003319706293127 [doi] PST - ppublish SO - Angiology. 2006 Oct-Nov;57(5):593-9. doi: 10.1177/0003319706293127. PMID- 16572286 OWN - NLM STAT- MEDLINE DCOM- 20070731 LR - 20220716 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 6 DP - 2007 Jun TI - Ascending aortic aneurysm in a man with scleroderma. PG - 1027-8 AB - Macrovascular involvement in scleroderma has received relatively little attention. We hereby describe a 5.6-cm ascending aortic aneurysm in a 56-year-old man presenting with increased dyspnea, diagnosed with antibody-negative, rapidly progressive diffuse cutaneous scleroderma. The patient had sclerosis and induration of all extremities, face, thorax and abdomen. Other features included Raynaud's phenomenon, arthralgia, dysphagia, dyspnea attributed to pulmonary fibrosis, pericardial effusion, and cardiomyopathy. To the best of our knowledge, there is only one other report to date in the English language of a thoracic aortic aneurysm associated with scleroderma. FAU - Attaran, Robert R AU - Attaran RR AD - The University of Arizona Affiliated Hospitals, 1501 N Campbell Avenue, Tucson, AZ 85724, USA. rattaran@email.arizona.edu FAU - Guarraia, David AU - Guarraia D LA - eng PT - Case Reports PT - Journal Article DEP - 20060330 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Aortic Aneurysm/*complications/diagnosis MH - Dyspnea/etiology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease MH - Scleroderma, Diffuse/*complications/*diagnosis EDAT- 2006/03/31 09:00 MHDA- 2007/08/01 09:00 CRDT- 2006/03/31 09:00 PHST- 2006/02/12 00:00 [received] PHST- 2006/02/22 00:00 [accepted] PHST- 2006/02/15 00:00 [revised] PHST- 2006/03/31 09:00 [pubmed] PHST- 2007/08/01 09:00 [medline] PHST- 2006/03/31 09:00 [entrez] AID - 10.1007/s10067-006-0267-5 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Jun;26(6):1027-8. doi: 10.1007/s10067-006-0267-5. Epub 2006 Mar 30. PMID- 20889363 OWN - NLM STAT- MEDLINE DCOM- 20110707 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 78 IP - 2 DP - 2011 Mar TI - Bilateral thenar hammer syndrome. PG - 212-4 LID - 10.1016/j.jbspin.2010.08.011 [doi] AB - BACKGROUND: Thenar hammer syndrome is a very rare condition that mimics rheumatic diseases such as carpal syndrome tunnel, Raynaud's phenomenon, and hand synovitis. OBJECTIVE: To describe the sonographic presentation of thenar hammer syndrome in a typical patient. METHODS: Grey-scale sonography and colour Doppler imaging of the hands with an iU22 scanner (Philips) were performed. RESULTS: In B mode, the lesion was seen as a large, rounded, heterogeneous area combining hypoechoic and echogenic components. Pseudoaneurysm was diagnosed based on presence of a cystic saccular formation arising directly from the adjacent artery and exhibiting an irregular thick wall with turbulent blood flow in the lumen. Colour Doppler showed blood flow in part of the lumen, the rest of which was filled with a thrombus seen as echogenic tissue. CONCLUSION: Sonography can help to diagnose thenar hammer syndrome. Angiography may be unnecessary in patients with normal colour Doppler findings. CI - Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Jousse-Joulin, Sandrine AU - Jousse-Joulin S AD - Rheumatology Unit, Immunology Department, Brest Teaching Hospitals, BP 814, 29609 Brest cedex, France. FAU - Plat, Emmanuel AU - Plat E FAU - Guias, Bruno AU - Guias B FAU - D'agostino, Maria Antonietta AU - D'agostino MA FAU - Bressollette, Luc AU - Bressollette L FAU - Saraux, Alain AU - Saraux A LA - eng PT - Case Reports PT - Journal Article PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Adult MH - Hand MH - Humans MH - Hypesthesia/*diagnostic imaging MH - Male MH - Occupational Diseases/diagnostic imaging MH - Paresthesia/*diagnostic imaging MH - Radial Artery/*injuries/physiopathology/surgery MH - Raynaud Disease/*diagnostic imaging MH - Regional Blood Flow/physiology MH - Syndrome MH - Treatment Outcome MH - Tunica Intima/diagnostic imaging/injuries MH - Ultrasonography, Doppler, Color EDAT- 2010/10/05 06:00 MHDA- 2011/07/08 06:00 CRDT- 2010/10/05 06:00 PHST- 2010/04/19 00:00 [received] PHST- 2010/08/23 00:00 [accepted] PHST- 2010/10/05 06:00 [entrez] PHST- 2010/10/05 06:00 [pubmed] PHST- 2011/07/08 06:00 [medline] AID - S1297-319X(10)00225-3 [pii] AID - 10.1016/j.jbspin.2010.08.011 [doi] PST - ppublish SO - Joint Bone Spine. 2011 Mar;78(2):212-4. doi: 10.1016/j.jbspin.2010.08.011. PMID- 29212687 OWN - NLM STAT- MEDLINE DCOM- 20171213 LR - 20171213 IS - 2049-4408 (Electronic) IS - 2049-4394 (Linking) VI - 99-B IP - 12 DP - 2017 Dec TI - Comparative study on the effectiveness of a corticosteroid injection for carpal tunnel syndrome in patients with and without Raynaud's phenomenon. PG - 1637-1642 LID - 10.1302/0301-620X.99B12.BJJ-2017-0371.R2 [doi] AB - AIMS: The aim of this study was to compare the efficacy of a corticosteroid injection for the treatment of carpal tunnel syndrome (CTS) in patients with and without Raynaud's phenomenon. PATIENTS AND METHODS: In a prospective study, 139 patients with CTS were treated with a corticosteroid injection (10 mg triamcinolone acetonide); 34 had Raynaud's phenomenon and 105 did not (control group). Grip strength, perception of touch with a Semmes-Weinstein monofilament and the Boston Carpal Tunnel Questionnaires (BCTQ) were assessed at baseline and at six, 12 and 24 weeks after the injection. The Cold Intolerance Severity Score (CISS) questionnaire was also assessed at baseline and 24 weeks after the injection. RESULTS: The two groups had similar baseline BCTQ scores, but the scores in the Raynaud's phenomenon group were significantly higher than those in the control group at 12 and 24 weeks after the injection. Throughout the 24-week follow-up, there were no significant differences in the mean grip strength between the groups, whereas the mean Semmes-Weinstein monofilament sensory index for the control group was significantly higher than that of the Raynaud's phenomenon group. The mean CISSs were not significantly different between the groups at baseline and at 24 weeks. After 24 weeks, 11 patients (32%) in the Raynaud's phenomenon group and 16 (15%) in the control group required carpal tunnel decompression (p = 0.028). Multivariable analysis indicated that concurrent Raynaud's phenomenon (odds ratio (OR) 2.6) and severe electrophysiological grade (OR 2.1) were independently associated with a failure of treatment after a corticosteroid injection. CONCLUSION: Although considerable improvements in symptoms will probably occur in patients with Raynaud's phenomenon who have CTS, they have higher risk of poor functional outcomes and failure of treatment than those without Raynaud's phenomenon. Cite this article: Bone Joint J 2017;99-B:1637-42. CI - ©2017 The British Editorial Society of Bone & Joint Surgery. FAU - Roh, Y H AU - Roh YH AD - Ewha Womans University, College of Medicine, 1071 Anyangcheon-ro, Yangcheon-gu, Seoul 07985, South Korea. FAU - Noh, J H AU - Noh JH AD - Kangwon National University Hospital, 156 Baengnyeong-ro, Chuncheon-si, Gangwon-do 24289, South Korea. FAU - Gong, H S AU - Gong HS AD - Seoul National University Bundang Hospital, 173 Gumi-ro, Bundang-gu, Sungnam 13620, South Korea. FAU - Baek, G H AU - Baek GH AD - Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, South Korea. LA - eng PT - Comparative Study PT - Journal Article PT - Retracted Publication PL - England TA - Bone Joint J JT - The bone & joint journal JID - 101599229 RN - 0 (Glucocorticoids) RN - F446C597KA (Triamcinolone Acetonide) SB - IM ECI - Bone Joint J. 2020 Aug;102-B(8):1107. doi: 10.1302/0301-620X.102B8.BJJ-2020-00009. PMID: 32731830 RIN - Bone Joint J. 2020 Oct;102-B(10):1428. doi: 10.1302/0301-620X.102B10.BJJ-2020-00012. PMID: 32993331 MH - Adult MH - Carpal Tunnel Syndrome/complications/*drug therapy MH - Female MH - Glucocorticoids/*administration & dosage MH - Humans MH - Injections MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/*etiology MH - Treatment Outcome MH - Triamcinolone Acetonide/*administration & dosage OTO - NOTNLM OT - Carpal tunnel syndrome OT - Corticosteroid injection OT - Prognosis OT - Raynaud phenomenon EDAT- 2017/12/08 06:00 MHDA- 2017/12/14 06:00 CRDT- 2017/12/08 06:00 PHST- 2017/03/23 00:00 [received] PHST- 2017/08/03 00:00 [accepted] PHST- 2017/12/08 06:00 [entrez] PHST- 2017/12/08 06:00 [pubmed] PHST- 2017/12/14 06:00 [medline] AID - 99-B/12/1637 [pii] AID - 10.1302/0301-620X.99B12.BJJ-2017-0371.R2 [doi] PST - ppublish SO - Bone Joint J. 2017 Dec;99-B(12):1637-1642. doi: 10.1302/0301-620X.99B12.BJJ-2017-0371.R2. PMID- 38743488 OWN - NLM STAT- MEDLINE DCOM- 20240629 LR - 20250129 IS - 1477-0393 (Electronic) IS - 0748-2337 (Print) IS - 0748-2337 (Linking) VI - 40 IP - 8 DP - 2024 Aug TI - Blood biomarkers for occupational hand-arm vibration exposure. PG - 432-440 LID - 10.1177/07482337241253996 [doi] AB - Hand-arm vibration is a common occupational exposure that causes neurological impairment, myalgia, and vibration-induced Raynaud's phenomena or vibration white fingers (VWF). The pathological mechanism is largely unknown, though several mechanisms have been proposed, involving both immunological vascular damage and defective neural responses. The aim of this study was to test whether the substances interleukin-33 (IL-33), macrophage-derived chemokine (MDC), interleukin-10 (IL-10), endothelin-1 (ET-1), C-C motif chemokine ligand 20 (CCL20), calcitonin, and thromboxane (TXA(2)) changed before and after occupational hand-arm vibration exposure. 38 full-time shift workers exposed to hand-arm vibration were recruited. All the participants underwent medical examinations regarding symptoms of Raynaud's phenomena. In 29 of the participants, the concentration of IL-33, MDC, IL-10, ET-1, CCL20, calcitonin, and TXA(2) was measured before and after a workday. There was a significant increase in ET-1 and calcitonin concentration and a decrease in the CCL20 concentration after the work shift in all participants. In the group suffering from VWF, but not in the non-VWF group, MDC was statistically significantly lower before the work shift (p = .023). The VWF group also showed a significant increase in MDC after the work shift. Exposure to occupational hand-arm vibration is associated with changes in ET-1, calcitonin, and MDC concentration in subjects suffering from vibration white fingers, suggesting a role of these biomarkers in the pathophysiology of this condition. FAU - Vihlborg, Per AU - Vihlborg P AUID- ORCID: 0000-0002-4256-1880 AD - Department of Geriatrics, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Lundberg, Oscar AU - Lundberg O AD - Department of Occupational and Environmental Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Pettersson-Pablo, Paul AU - Pettersson-Pablo P AD - Department of Laboratory Medicine, Faculty of Medicine and Health, Örebro University Hospital, Örebro, Sweden. RINGGOLD: 59566 FAU - Johansson, Niclas AU - Johansson N AD - Department of Occupational and Environmental Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Bryngelsson, Ing-Liss AU - Bryngelsson IL AD - Department of Occupational and Environmental Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 AD - Department of Ophthalmology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. RINGGOLD: 596174 FAU - Stjernbrandt, Albin AU - Stjernbrandt A AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. RINGGOLD: 59588 FAU - Graff, Pål AU - Graff P AD - Department of Chemical Work Environment, National Institute of Occupational Health (STAMI), Oslo, Norway. RINGGOLD: 70672 LA - eng PT - Journal Article DEP - 20240514 PL - England TA - Toxicol Ind Health JT - Toxicology and industrial health JID - 8602702 RN - 0 (Biomarkers) RN - 0 (Endothelin-1) RN - 0 (Interleukin-33) RN - 130068-27-8 (Interleukin-10) RN - 57576-52-0 (Thromboxane A2) SB - IM MH - Humans MH - *Occupational Exposure/adverse effects MH - *Biomarkers/blood MH - Male MH - Adult MH - *Hand-Arm Vibration Syndrome/blood/diagnosis MH - *Vibration/adverse effects MH - Middle Aged MH - Endothelin-1/blood MH - Female MH - Interleukin-33/blood MH - Interleukin-10/blood MH - Raynaud Disease/blood/etiology MH - Thromboxane A2/blood PMC - PMC11755968 OTO - NOTNLM OT - Hand-arm vibration OT - Raynauds syndrome OT - vibration exposure OT - vibration white fingers COIS- Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2024/05/15 05:47 MHDA- 2024/06/29 15:42 PMCR- 2025/01/23 CRDT- 2024/05/14 12:03 PHST- 2024/06/29 15:42 [medline] PHST- 2024/05/15 05:47 [pubmed] PHST- 2024/05/14 12:03 [entrez] PHST- 2025/01/23 00:00 [pmc-release] AID - 10.1177_07482337241253996 [pii] AID - 10.1177/07482337241253996 [doi] PST - ppublish SO - Toxicol Ind Health. 2024 Aug;40(8):432-440. doi: 10.1177/07482337241253996. Epub 2024 May 14. PMID- 25824427 OWN - NLM STAT- MEDLINE DCOM- 20160524 LR - 20250711 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 35 IP - 9 DP - 2015 Sep TI - Efficacy of Raynaud's phenomenon and digital ulcer pharmacological treatment in systemic sclerosis patients: a systematic literature review. PG - 1447-59 LID - 10.1007/s00296-015-3241-1 [doi] AB - To evaluate the efficacy of current treatments for the Raynaud phenomenon (RP) in patients with systemic sclerosis (SSc), a systematic literature search was performed using Medline, EMBASE, and Cochrane Central Register of Controlled Trials (from 1961 to October 2011). We included meta-analyses, systematic reviews, clinical trials, and high-quality cohort studies published in English or Spanish. Patient populations had to include adults diagnosed with limited cutaneous or diffuse SSc who had associated RP and/or digital ulcers under pharmacological treatment. Efficacy of treatments was evaluated based on: number of RP episodes, RP severity, episode-free time, ulcer improvement/healing, and appearance of new ulcers. We used the Jadad scale of methodological quality to evaluate the quality of randomized clinical trials, and the 2009 Oxford Centre for Evidence-Based Medicine classification for other studies. Of a total of 1617 studies identified, only 27 fulfilled inclusion criteria. Drugs received the following grade recommendations: Grade A for nifedipine, nicardipine, quinapril, IV iloprost, bosentan, tadalafil, and MQx-503; Grade B for beraprost, cicaprost, DMSO, cyclofenil, and atorvastatin; and Grade C for misoprostol, prazosin, OPC-2826, enalapril, sildenafil, antioxidant, and stanazolol. Calcium channel blockers, prostanoids, tadalafil, and bosentan received the highest recommendation level for their effectiveness. However, most systematic reviews reviewed just a handful of studies with small sample sizes and short follow-ups. Our review shows that the existing evidence on the efficacy of RP treatment in SSc patients is inconclusive which calls for further research, especially in the form of prospective studies of high quality with long-term follow-ups. FAU - García de la Peña Lefebvre, Paloma AU - García de la Peña Lefebvre P AD - Servicio de Reumatología, Hospitales Universitarios Grupo Madrid (Madrid, Montepríncipe, Sanchinarro y Torrelodones), c/Oña nº10, 28050, Madrid, Spain, reumatologia.hms@hmhospitales.com. FAU - Nishishinya, María Betina AU - Nishishinya MB FAU - Pereda, Claudia Alejandra AU - Pereda CA FAU - Loza, Estíbaliz AU - Loza E FAU - Sifuentes Giraldo, Walter Alberto AU - Sifuentes Giraldo WA FAU - Román Ivorra, José Andrés AU - Román Ivorra JA FAU - Carreira, Patricia AU - Carreira P FAU - Rúa-Figueroa, Iñigo AU - Rúa-Figueroa I FAU - Pego-Reigosa, Jose María AU - Pego-Reigosa JM FAU - Muñoz-Fernández, Santiago AU - Muñoz-Fernández S LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20150401 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - Raynaud Disease/complications/*drug therapy MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/complications/*drug therapy MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use EDAT- 2015/04/01 06:00 MHDA- 2016/05/25 06:00 CRDT- 2015/04/01 06:00 PHST- 2014/09/22 00:00 [received] PHST- 2015/03/02 00:00 [accepted] PHST- 2015/04/01 06:00 [entrez] PHST- 2015/04/01 06:00 [pubmed] PHST- 2016/05/25 06:00 [medline] AID - 10.1007/s00296-015-3241-1 [doi] PST - ppublish SO - Rheumatol Int. 2015 Sep;35(9):1447-59. doi: 10.1007/s00296-015-3241-1. Epub 2015 Apr 1. PMID- 24038322 OWN - NLM STAT- MEDLINE DCOM- 20140522 LR - 20131008 IS - 1097-0274 (Electronic) IS - 0271-3586 (Linking) VI - 56 IP - 11 DP - 2013 Nov TI - Hypothenar hammer syndrome: a multicenter case-control study. PG - 1352-8 LID - 10.1002/ajim.22237 [doi] AB - BACKGROUND: The rarely diagnosed hypothenar hammer syndrome (HHS) is due to vascular damage to the distal part of the ulnar artery probably caused by acute or repetitive blunt trauma to the hypothenar region. To date, mainly case reports have been published, while epidemiological data are almost absent. AIM: To identify potential risk factors for HHS. METHOD: An interview-based multicenter case-control study of 71 patients with HHS and 105 matched controls was conducted with standardized questions regarding disease specific variables, occupation, exposure of the hands to different types of trauma in occupational and leisure context. Medical data were verified from individual medical records. RESULTS: Multivariable logistic regression analysis revealed that using the hand as a hammer on a daily basis (adjusted odds ratio [aOR] 17.04, 95% CI 5.51-52.67) daily pressure to the palm of the hand (aOR 4.96, 95% CI 1.39-17.71), and daily exposure to vibrating tools (aOR 3.41, 95% CI 1.03-11.31) were significant risk factors for HHS. CONCLUSIONS: This investigation represents one of the largest groups of patients with HHS described so far. Work-related repeated blunt trauma to the palm of the hand significantly increases the risk of HHS. CI - © 2013 Wiley Periodicals, Inc. FAU - Scharnbacher, Jutta AU - Scharnbacher J AD - Institute of Occupational, Social and Environmental Medicine, University Medical Center of the Johannes Gutenberg University of Mainz, Germany. FAU - Claus, Matthias AU - Claus M FAU - Reichert, Jörg AU - Reichert J FAU - Röhrl, Tobias AU - Röhrl T FAU - Hoffmann, Ulrich AU - Hoffmann U FAU - Ulm, Kurt AU - Ulm K FAU - Letzel, Stephan AU - Letzel S FAU - Nowak, Dennis AU - Nowak D LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20130822 PL - United States TA - Am J Ind Med JT - American journal of industrial medicine JID - 8101110 SB - IM MH - Adult MH - Aged MH - Arterial Occlusive Diseases/complications/*epidemiology MH - Case-Control Studies MH - Cumulative Trauma Disorders/complications/*epidemiology MH - Hand Injuries/complications/*epidemiology MH - Humans MH - Ischemia/etiology MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Occupational Injuries/complications/*epidemiology MH - Odds Ratio MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Risk Factors MH - Syndrome MH - Ulnar Artery/*injuries MH - Vibration/adverse effects OTO - NOTNLM OT - Raynaud's phenomenon OT - etiology OT - hand trauma OT - hypothenar hammer syndrome OT - occupational OT - risk factors EDAT- 2013/09/17 06:00 MHDA- 2014/05/23 06:00 CRDT- 2013/09/17 06:00 PHST- 2013/07/18 00:00 [accepted] PHST- 2013/09/17 06:00 [entrez] PHST- 2013/09/17 06:00 [pubmed] PHST- 2014/05/23 06:00 [medline] AID - 10.1002/ajim.22237 [doi] PST - ppublish SO - Am J Ind Med. 2013 Nov;56(11):1352-8. doi: 10.1002/ajim.22237. Epub 2013 Aug 22. PMID- 24963499 OWN - NLM STAT- MEDLINE DCOM- 20141203 LR - 20220317 IS - 2314-7156 (Electronic) IS - 2314-8861 (Print) IS - 2314-7156 (Linking) VI - 2014 DP - 2014 TI - Clinical course, prognosis, and cause of death in primary Sjögren's syndrome. PG - 647507 LID - 10.1155/2014/647507 [doi] LID - 647507 AB - The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4±6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk. FAU - Horvath, Ildiko Fanny AU - Horvath IF AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, Hungary. FAU - Szanto, Antonia AU - Szanto A AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, Hungary. FAU - Papp, Gabor AU - Papp G AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, Hungary. FAU - Zeher, Margit AU - Zeher M AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, Hungary. LA - eng PT - Journal Article DEP - 20140520 PL - Egypt TA - J Immunol Res JT - Journal of immunology research JID - 101627166 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Cause of Death MH - Child MH - Child, Preschool MH - Female MH - Follow-Up Studies MH - Humans MH - Infant MH - Infant, Newborn MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/*pathology MH - Retrospective Studies MH - Risk Factors MH - Sjogren's Syndrome/immunology/*mortality/*pathology MH - Survival Analysis MH - Thyroiditis/*pathology MH - Vasculitis/*pathology PMC - PMC4054879 EDAT- 2014/06/26 06:00 MHDA- 2014/12/15 06:00 PMCR- 2014/05/20 CRDT- 2014/06/26 06:00 PHST- 2014/02/25 00:00 [received] PHST- 2014/04/24 00:00 [revised] PHST- 2014/04/25 00:00 [accepted] PHST- 2014/06/26 06:00 [entrez] PHST- 2014/06/26 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] PHST- 2014/05/20 00:00 [pmc-release] AID - 10.1155/2014/647507 [doi] PST - ppublish SO - J Immunol Res. 2014;2014:647507. doi: 10.1155/2014/647507. Epub 2014 May 20. PMID- 21664914 OWN - NLM STAT- MEDLINE DCOM- 20120104 LR - 20110802 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 82 IP - 2 DP - 2011 Sep TI - Sample entropy of laser Doppler flowmetry signals increases in patients with systemic sclerosis. PG - 152-5 LID - 10.1016/j.mvr.2011.05.007 [doi] AB - Associated to reactivity tests, laser Doppler flowmetry (LDF) emphasizes abnormal skin microvascular function in diseases affecting digits, such as Raynaud's phenomenon (RP) and systemic sclerosis (SSc). However, baseline perfusion value does not discriminate between disease states. We study if LDF sample entropy (SampEn) allows distinguishing healthy subjects, RP and SSc patients. LDF measurements were performed on finger pad and forearm of 108 subjects (27 controls, 28 RP patients, 53 SSc patients), before and after local thermal hyperemia. We also assessed the reproducibility of SampEn [expressed as within-subject coefficients of variation (CV) and intra-class correlation coefficients (ICC)]. Baseline SampEn is significantly increased in patients with SSc compared to RP and controls on finger pad [0.49 (0.19), 0.38 (0.14) and 0.36 (0.15), respectively; P<0.002], but not on forearm. However, local thermal hyperemia increased SampEn at all sites and for all groups. Finally, reproducibility of SampEn computed on two baseline segments was acceptable (CV=26%, ICC=0.63). SampEn of skin blood flow at rest is increased on finger pad of patients with SSc but not on forearm. This is consistent with the pathophysiology of the disease, which predominantly affects digital microcirculation in most patients. SampEn of LDF signal could be a reproducible tool to predict digital microvascular impairment. CI - Copyright © 2011 Elsevier Inc. All rights reserved. FAU - Figueiras, E AU - Figueiras E AD - Instrumentation Center (GEI-CI), Physics Department, Faculty of Sciences and Technology of Coimbra University, Rua Larga, P-3004-516 Coimbra, Portugal. editefigueiras@gmail.com FAU - Roustit, M AU - Roustit M FAU - Semedo, S AU - Semedo S FAU - Ferreira, L F Requicha AU - Ferreira LF FAU - Crascowski, J L AU - Crascowski JL FAU - Humeau, A AU - Humeau A LA - eng PT - Journal Article DEP - 20110612 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Entropy MH - Female MH - Humans MH - Laser-Doppler Flowmetry/*methods MH - Male MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/pathology MH - Regional Blood Flow MH - Reproducibility of Results MH - Scleroderma, Systemic/*blood/*pathology MH - Signal Processing, Computer-Assisted MH - Skin/blood supply/pathology EDAT- 2011/06/15 06:00 MHDA- 2012/01/05 06:00 CRDT- 2011/06/14 06:00 PHST- 2011/03/17 00:00 [received] PHST- 2011/05/10 00:00 [revised] PHST- 2011/05/24 00:00 [accepted] PHST- 2011/06/14 06:00 [entrez] PHST- 2011/06/15 06:00 [pubmed] PHST- 2012/01/05 06:00 [medline] AID - S0026-2862(11)00100-2 [pii] AID - 10.1016/j.mvr.2011.05.007 [doi] PST - ppublish SO - Microvasc Res. 2011 Sep;82(2):152-5. doi: 10.1016/j.mvr.2011.05.007. Epub 2011 Jun 12. PMID- 22590983 OWN - NLM STAT- MEDLINE DCOM- 20130711 LR - 20191210 IS - 1442-2050 (Electronic) IS - 1120-8694 (Linking) VI - 26 IP - 2 DP - 2013 Feb-Mar TI - Symptoms and esophageal motility based on phenotypic findings of scleroderma. PG - 197-203 LID - 10.1111/j.1442-2050.2012.01349.x [doi] AB - Scleroderma esophagus is characterized by ineffective peristalsis and reduced esophageal sphincter pressure. Esophageal disease in scleroderma can precede cutaneous manifestations and has been associated with Raynaud's phenomenon (RP) and pulmonary fibrosis (PF). The objective of the study is to evaluate the impact of cutaneous findings, RP, and PF on demographics, symptoms, and esophageal motility in patients with scleroderma. Scleroderma patients with esophageal involvement were included after review of esophageal manometries and charts over a 6-year period. High-resolution esophageal manometry was performed. Patients completed a symptom questionnaire. The study enrolled 28 patients (22 females; mean age 50.3 ± 12.8 years) with scleroderma esophagus. Patients without skin involvement (n= 12) reported more severe heartburn (P= 0.02), while those with cutaneous findings (n= 16) had more frequent dysphagia with solids (P= 0.02). Patients with RP (n= 22) had lower amplitude of distal esophageal contractions (P= 0.01) than those without RP (n= 6). Patients with PF (n= 11) reported more severe coughing and wheezing (both P= 0.03) than those without lung disease (n= 17). This study highlights subgroups of patients with scleroderma esophagus according to phenotypic findings of dermatologic changes, RP, and PF. Heartburn and dysphagia are important symptoms that may be associated with different stages of disease progression based on skin changes in scleroderma. RP was associated with greater esophageal dysmotility. Coughing and wheezing were more severe in patients with PF. CI - © 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus. FAU - Tang, D M AU - Tang DM AD - Section of Gastroenterology, Department of Medicine, Temple University School of Medicine, Philadelphia, Pennsylvania 19140, USA. FAU - Pathikonda, M AU - Pathikonda M FAU - Harrison, M AU - Harrison M FAU - Fisher, R S AU - Fisher RS FAU - Friedenberg, F K AU - Friedenberg FK FAU - Parkman, H P AU - Parkman HP LA - eng PT - Evaluation Study PT - Journal Article DEP - 20120516 PL - United States TA - Dis Esophagus JT - Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus JID - 8809160 SB - IM MH - Adult MH - Disease Progression MH - Esophageal Motility Disorders/diagnosis/*etiology MH - Female MH - Heartburn/etiology MH - Humans MH - Male MH - Manometry MH - Middle Aged MH - *Phenotype MH - Pulmonary Fibrosis/*etiology MH - Raynaud Disease/*etiology MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*diagnosis MH - Severity of Illness Index MH - Surveys and Questionnaires EDAT- 2012/05/18 06:00 MHDA- 2013/07/13 06:00 CRDT- 2012/05/18 06:00 PHST- 2012/05/18 06:00 [entrez] PHST- 2012/05/18 06:00 [pubmed] PHST- 2013/07/13 06:00 [medline] AID - 10.1111/j.1442-2050.2012.01349.x [doi] PST - ppublish SO - Dis Esophagus. 2013 Feb-Mar;26(2):197-203. doi: 10.1111/j.1442-2050.2012.01349.x. Epub 2012 May 16. PMID- 37481713 OWN - NLM STAT- MEDLINE DCOM- 20240502 LR - 20260602 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 5 DP - 2024 May 2 TI - Construct validity and reliability of the Assessment of Systemic Sclerosis-Associated Raynaud's Phenomenon (ASRAP) questionnaire. PG - 1281-1290 LID - 10.1093/rheumatology/kead371 [doi] AB - OBJECTIVES: Assessment of construct validity and reliability of a novel patient-reported outcome (PRO) instrument for assessing the severity and impact of RP in SSc. METHODS: An international multicentre study validation study of the 27-item Assessment of Systemic Sclerosis-Associated Raynaud's Phenomenon (ASRAP) and 10-item short-form (ASRAP-SF) questionnaires. The relationship between ASRAP questionnaires and demographics, clinical phenotype and legacy instruments for assessing SSc-RP severity, disability and pain was assessed. Repeatability was evaluated at 1 week. Anchor-based statements of health status facilitated assessment of ASRAP thresholds of meaning. RESULTS: A total of 420 SSc subjects were enrolled. There was good correlation between ASRAP (and ASRAP-SF) with RP visual analogue scale (VAS) and Scleroderma Health Assessment Questionnaire RP VAS (rho range 0.648-0.727, P < 0.001). Correlation with diary-based assessment of SSc-RP attack frequency and duration was lower (rho range 0.258-0.504, P < 0.001). ASRAP questionnaires had good correlation with instruments for assessing disability, hand function, pain and global health assessment (rho range 0.427-0.575, P < 0.001). Significantly higher ASRAP scores were identified in smokers, patients with active digital ulceration (DU), previous history of DU and calcinosis (P < 0.05 for all comparisons). There was excellent repeatability at 1 week among patients with stable SSc-RP symptoms (intra-class coefficients of 0.891 and 0.848, P < 0.001). Patient-acceptable symptom state thresholds for ASRAP and ASRAP-SF were 45.34 and 45.77, respectively. A preliminary Minimally Important Clinical Difference threshold of 4.17 (95% CI 0.53, 7.81, P = 0.029) was estimated. CONCLUSION: ASRAP and ASRAP-SF questionnaires are valid and reliable novel PRO instruments for assessing the severity and impact of SSc-RP. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. AD - Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. FAU - Yu, Lan AU - Yu L AD - Department of Medicine, University of Pittsburgh, PA, USA. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Vanderbilt University, Nashville, TN, USA. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Hummers, Laura K AU - Hummers LK AD - Department of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Shah, Ami A AU - Shah AA AD - Department of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - Department of Rheumatology, University of Tulane Medical Center, New Orleans, LA, USA. FAU - Withey, Jane AU - Withey J AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan Scleroderma Program, Ann Arbor, MI, USA. AD - Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. FAU - Domsic, Robyn T AU - Domsic RT AUID- ORCID: 0000-0002-2765-0922 AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA. LA - eng GR - I01 CX002111/CX/CSRD VA/United States GR - K24 AR080217/AR/NIAMS NIH HHS/United States GR - W81XWH-18–1-0602/US Department of Defence/ PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Validation Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - *Scleroderma, Systemic/complications/diagnosis MH - Female MH - Male MH - Middle Aged MH - Reproducibility of Results MH - Surveys and Questionnaires/standards MH - Adult MH - *Severity of Illness Index MH - Disability Evaluation MH - Patient Reported Outcome Measures MH - Aged MH - Pain Measurement/methods OTO - NOTNLM OT - RP OT - SSc OT - clinical trial OT - patient-reported outcome instrument OT - validation EDAT- 2023/07/23 13:08 MHDA- 2024/05/02 18:47 CRDT- 2023/07/23 06:46 PHST- 2023/01/27 00:00 [received] PHST- 2023/06/27 00:00 [accepted] PHST- 2024/05/02 18:47 [medline] PHST- 2023/07/23 13:08 [pubmed] PHST- 2023/07/23 06:46 [entrez] AID - 7229563 [pii] AID - 10.1093/rheumatology/kead371 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 May 2;63(5):1281-1290. doi: 10.1093/rheumatology/kead371. PMID- 35020814 OWN - NLM STAT- MEDLINE DCOM- 20220908 LR - 20250728 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 9 DP - 2022 Aug 30 TI - Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort. PG - 3686-3692 LID - 10.1093/rheumatology/keac006 [doi] AB - OBJECTIVE: This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc. METHODS: This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated. RESULTS: Among 217 patients, 153 (70.5%) were classified as SSc, including 65 (30%) in the first investigation; 69.3% of the SSc patients met VEDOSS criteria compared with 6.3% of patients who did not progress to SSc. The combinations most associated with progression to SSc were RP + puffy fingers (PF) + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), with an odds ratio (OR) of 19.52 (95% CI 4.48, 85.06; P < 0.001) and RP + PF + positive ANA (VEDOSS level 1; 'red flags') (OR 15.45; P < 0.001), while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR 0.03; P < 0.001) and RP + anticentromere + SD-NFC (OR 0.06; P = 0.006) were associated with non-progression to SSc. CONCLUSION: Among patients with RP with at least one manifestation of SSc, without skin involvement, combinations of VEDOSS characteristics were the strongest predictors of progression to SSc at a median follow-up of 4 years. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Siqueira, Valdirene S AU - Siqueira VS AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Helbingen, Mariely F S AU - Helbingen MFS AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Medeiros-Ribeiro, Ana Cristina AU - Medeiros-Ribeiro AC AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Carriço da Silva, Henrique AU - Carriço da Silva H AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Miossi, Renata AU - Miossi R AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Luppino-Assad, Ana Paula AU - Luppino-Assad AP AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Sampaio-Barros, Percival D AU - Sampaio-Barros PD AUID- ORCID: 0000-0001-9843-6686 AD - Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Cross-Sectional Studies MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/complications MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Raynaud’s phenomenon OT - antinuclear antibodies OT - nailfold capillaroscopy OT - puffy fingers OT - systemic sclerosis EDAT- 2022/01/13 06:00 MHDA- 2022/09/09 06:00 CRDT- 2022/01/12 17:30 PHST- 2021/09/11 00:00 [received] PHST- 2021/12/08 00:00 [revised] PHST- 2022/01/13 06:00 [pubmed] PHST- 2022/09/09 06:00 [medline] PHST- 2022/01/12 17:30 [entrez] AID - 6502299 [pii] AID - 10.1093/rheumatology/keac006 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Aug 30;61(9):3686-3692. doi: 10.1093/rheumatology/keac006. PMID- 22883334 OWN - NLM STAT- MEDLINE DCOM- 20130425 LR - 20140226 IS - 0578-1426 (Print) IS - 0578-1426 (Linking) VI - 51 IP - 5 DP - 2012 May TI - [The diagnostic significance of nailfold video-capillaroscopy in systemic sclerosis]. PG - 362-5 AB - OBJECTIVE: To observe nailfold capillary changes in a cohort of connective tissue disease (CTD) with Raynaud's phenomenon (RP) and to explore the diagnostic value of nailfold video-capillaroscopy (NVC) in systemic sclerosis (SSc). METHODS: Sixty CTD patients with RP divided into SSc group (n = 36) and non-SSc group (n = 24) were referred to an experienced operator for NVC. RESULTS: The patients had decreased capillary loops in SSc group with the capillary diameter more enlarged in SSc group than non-SSc group. The number of patients in SSc group with giant capillaries was 14, while 3 in non-SSc group. There were 23 patients with haemorrhages in SSc group and 9 in non-SSc group. The number of patients with severe effusion was 15 in SSc group, while 2 in non-SSc group. By using the ROC curves, indexes with AUC at least 0.7 of the input capillary diameter, the output capillary diameter, the middle capillary diameter, blood color and effusion for the diagnostic cutoff points were 18.5 µm, 24.5 µm, 19.5µm, deep red and severe effusion. With at least 2 out of the top 3 indexes, the diagnostic sensitivity and specificity of SSc were higher. CONCLUSIONS: CTD Patients with RP of SSc have less capillary loops, more enlarged capillaries, more giant capillaries, more severe effusion and more haemorrhages than non-SSc patients. The characteristics of nailfold capillary changes in SSc patients with RP can be helpful for the diagnosis and the differential diagnosis of SSc. FAU - Li, Lin-Guang AU - Li LG AD - Department of Rheumatology, Chinese PLA General Hospital, Beijing 100853, China. FAU - Zhang, Jiang-Lin AU - Zhang JL FAU - Liu, Xiu-Hua AU - Liu XH FAU - Huang, Feng AU - Huang F LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Nei Ke Za Zhi JT - Zhonghua nei ke za zhi JID - 16210490R SB - IM MH - Adult MH - Capillaries MH - Female MH - Humans MH - Male MH - Microcirculation MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis MH - Young Adult EDAT- 2012/08/14 06:00 MHDA- 2013/04/26 06:00 CRDT- 2012/08/14 06:00 PHST- 2012/08/14 06:00 [entrez] PHST- 2012/08/14 06:00 [pubmed] PHST- 2013/04/26 06:00 [medline] PST - ppublish SO - Zhonghua Nei Ke Za Zhi. 2012 May;51(5):362-5. PMID- 23414913 OWN - NLM STAT- MEDLINE DCOM- 20130813 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 3 DP - 2013 May-Jun TI - Chronic chilblains: the clinical presentation and disease course in a large paediatric series. PG - 463-8 AB - OBJECTIVES: Children often present during winter with painful, red-purple swollen fingers and/or toes, usually misdiagnosed as Raynaud's phenomenon. Pernio, or chronic chilblains, is a localised inflammatory lesion of the skin resulting from an abnormal response to cold. The aim of this study was to better characterise the clinical presentation of chronic chilblains in children. METHODS: This is a single-centre retrospective study of patients referred to our paediatric rheumatology clinic with cold, purple, and painful hands. Patients were identified from the paediatric rheumatology clinic database, at the Safra Children Hospital, Israel. Data of the clinical presentation, physical findings, laboratory investigations and the course of the disease were extracted from the patients' charts and analysed. RESULTS: A total of 33 patients (27 females, sex ratio 4.5:1) were identified. Patients age at presentation was 13.5±2.1, and disease duration was 2.0±1.0 winters. Patients presented with prolonged capillary refill time (100%) and abnormal modified Allen test (75.6%). Fingers swelling was the most common finding (81.8%), followed by proximal interphalangeal joint (PIPs) swelling (63.6%), skin ulceration (54.5%), and dry, irritated skin (45.5%). Nailfold capillary microscopy was normal in all patients. The only abnormal laboratory test was the test for anti-nuclear factor (ANA) in 25%. CONCLUSIONS: We report a large series of children with a unique symptomatology consisting in chronic chilblains. FAU - Padeh, Shai AU - Padeh S AD - Department of Pediatrics A, Edmond & Lily Safra Children's Hospital, Tel Hashomer, Israel. shay.pade@sheba.health.gov.il FAU - Gerstein, Maya AU - Gerstein M FAU - Greenberger, Shoshana AU - Greenberger S FAU - Berkun, Yackov AU - Berkun Y LA - eng PT - Journal Article DEP - 20130215 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Antibodies, Antinuclear/*immunology MH - Arthritis/diagnosis/etiology MH - Chilblains/complications/*diagnosis/immunology MH - Child MH - Chronic Disease MH - Cohort Studies MH - Diagnosis, Differential MH - Disease Progression MH - Edema/diagnosis/etiology MH - Female MH - Finger Joint/pathology MH - Humans MH - Male MH - Physical Examination MH - Raynaud Disease/*diagnosis MH - Retrospective Studies MH - Skin Ulcer/diagnosis/etiology EDAT- 2013/02/19 06:00 MHDA- 2013/08/14 06:00 CRDT- 2013/02/19 06:00 PHST- 2012/05/30 00:00 [received] PHST- 2012/09/24 00:00 [accepted] PHST- 2013/02/19 06:00 [entrez] PHST- 2013/02/19 06:00 [pubmed] PHST- 2013/08/14 06:00 [medline] AID - 6176 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 May-Jun;31(3):463-8. Epub 2013 Feb 15. PMID- 29983545 OWN - NLM STAT- MEDLINE DCOM- 20181031 LR - 20220318 IS - 1177-8881 (Electronic) IS - 1177-8881 (Linking) VI - 12 DP - 2018 TI - The mechanism of botulinum A on Raynaud syndrome. PG - 1905-1915 LID - 10.2147/DDDT.S161113 [doi] AB - BACKGROUND: Botulinum neurotoxin type A (BoNT/A) is emerging as a treatment modality for Raynaud's phenomenon (RP). However, the mechanism of the role of BoNT/A in antagonizing the constriction of arteriola in RP remains unclear. MATERIALS AND METHODS: We tested the constriction of arteriole diameter and the distribution of adrenergic receptors on the rat cremaster modle. Moreover, we measured the secretion of norepinephrine (NE), protein level changes and related receptors on cultured rat superior cervical ganglia neurons(SCGNs), a model of sympathetic neuron. RESULTS: Based on our results, the inhibition of arteriole vasoconstriction was increased with increasing doses of BoNT/A. BoNT/A, prazosin, and BQ123 treatment can result in significant inhibition of arteriole vasoconstriction with the same electrical stimulation. The inhibition effect of prazosin was equivalent to BoNT/A, while BQ123 has a synergistic effect with BoNT/A. After treating SCGNs using BoNT/A for 30 min, the decrease in fluorescence intensity of FM1-43 slowed down which was correlated with the doses of BoNT/A. Furthermore, release of NE in the supernatant was significantly decreased as measured by enzyme-linked immunosorbent assay, 24 h after a high dose of BoNT/A (25 µ/mL). Cleaved-SNAP-25 was detected by Western blotting 24 h following BoNT/A (50 µ/mL) treatment. Moreover, receptor SV2C, GM1, and FGFR3 were detected on sympathetic neurons, similarly to cholinergic neurons. CONCLUSION: Our study showed that BoNT/A could significantly inhibit electrical stimulation-induced arteriole vasoconstriction through the sympathetic pathway. The mechanism was similar to the cholinergic one, in which the vesicle release of sympathetic neurons could be inhibited by cleavage of SNAP-25. The end result was blocked vesicle fusion with the presynaptic membrane after BoNT/A treatment, inhibiting the release of the NE. FAU - Zhou, Yanwen AU - Zhou Y AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Liu, Ying AU - Liu Y AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Hao, Yunhua AU - Hao Y AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Feng, Ya AU - Feng Y AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Pan, Lizhen AU - Pan L AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Liu, Wuchao AU - Liu W AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Li, Bing AU - Li B AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Xiao, Libin AU - Xiao L AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Jin, Lingjing AU - Jin L AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. FAU - Nie, Zhiyu AU - Nie Z AD - Department of Neurology, Shanghai Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China. LA - eng PT - Journal Article DEP - 20180626 PL - New Zealand TA - Drug Des Devel Ther JT - Drug design, development and therapy JID - 101475745 RN - 0 (Receptors, Adrenergic, alpha-1) RN - 0 (Snap25 protein, rat) RN - 0 (Synaptosomal-Associated Protein 25) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - X4W3ENH1CV (Norepinephrine) SB - IM MH - Animals MH - Arterioles/drug effects/physiology MH - Botulinum Toxins, Type A/pharmacology/*therapeutic use MH - Dose-Response Relationship, Drug MH - Male MH - Norepinephrine/metabolism MH - Rats MH - Rats, Sprague-Dawley MH - Raynaud Disease/*drug therapy MH - Receptors, Adrenergic, alpha-1/analysis MH - Synaptosomal-Associated Protein 25/physiology PMC - PMC6027706 OTO - NOTNLM OT - FGFR3 OT - GM1 OT - Raynaud’s phenomenon OT - SNAP-25 OT - SV2C OT - arteriole diameter constrict rate OT - botulinum neurotoxin type A OT - sympathetic neuron OT - vesicle cycle OT - α-adrenoceptor COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2018/07/10 06:00 MHDA- 2018/11/01 06:00 PMCR- 2018/06/26 CRDT- 2018/07/10 06:00 PHST- 2018/07/10 06:00 [entrez] PHST- 2018/07/10 06:00 [pubmed] PHST- 2018/11/01 06:00 [medline] PHST- 2018/06/26 00:00 [pmc-release] AID - dddt-12-1905 [pii] AID - 10.2147/DDDT.S161113 [doi] PST - epublish SO - Drug Des Devel Ther. 2018 Jun 26;12:1905-1915. doi: 10.2147/DDDT.S161113. eCollection 2018. PMID- 30234574 OWN - NLM STAT- MEDLINE DCOM- 20190109 LR - 20220331 IS - 1538-8654 (Electronic) IS - 1527-7941 (Linking) VI - 31 IP - 10 DP - 2018 Oct TI - Advances in Upper Extremity Scleroderma Wound Care. PG - 446-455 LID - 10.1097/01.ASW.0000544615.18501.fd [doi] AB - GENERAL PURPOSE: To provide information about the pathophysiology, diagnosis, and treatment options for systemic sclerosis. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant should be better able to:1. Describe the pathophysiology, signs, symptoms, and diagnosis of systemic sclerosis.2. Outline the evidence-based medical and surgical management of systemic sclerosis. ABSTRACT: OBJECTIVE:: To perform a targeted review of systemic sclerosis, including epidemiology, pathophysiology, diagnosis, signs and symptoms, and medical and surgical management of upper extremity manifestations. DATA SOURCES AND STUDY SELECTION: An electronic literature review was conducted using PubMed for all publication dates through October 2017. Searches were performed using combinations of terms including "systemic sclerosis," "scleroderma," "management," "upper extremity," "hypercalcinosis," "Raynaud's phenomenon," "sympathectomy," and "digital ulcers." Only full-length articles written in English that discussed the management of upper extremity scleroderma were used. DATA EXTRACTION AND SYNTHESIS: The epidemiology, pathophysiology, diagnosis, upper extremity manifestations, and medical and surgical management of systemic sclerosis were reviewed. The case described in this article reports the utility of microsurgical interventions in the treatment of medically refractory upper extremity systemic sclerosis. CONCLUSIONS: Systemic sclerosis is a rare rheumatologic disease that greatly impacts quality of life. Medical management is the mainstay of treatment, propelling an improvement in the dismal 10-year cumulative survival rate from 54% in the 1970s to 66% in the 1990s. However, the pathophysiology of this disease is still poorly understood, and when medical management fails and the disease inevitably progresses, surgical approaches are critical. FAU - Cohen, Joshua M AU - Cohen JM AD - Joshua M. Cohen, MD • Resident Physician • New York University School of Medicine • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York Rachel A. Sibley, MD • Resident Physician • New York University School of Medicine • Department of Internal Medicine • NYU Langone Health • New York, New York Ernest S. Chiu, MD, FACS • Associate Professor • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York Sheel Sharma, MD • Clinical Associate Professor • Hansjörg Wyss Department of Plastic Surgery • NYU Langone Health • New York, New York. FAU - Sibley, Rachel A AU - Sibley RA FAU - Chiu, Ernest S AU - Chiu ES FAU - Sharma, Sheel AU - Sharma S LA - eng PT - Journal Article PT - Review PL - United States TA - Adv Skin Wound Care JT - Advances in skin & wound care JID - 100911021 RN - 0 (Dermatologic Agents) MH - Combined Modality Therapy/methods MH - Dermatologic Agents/therapeutic use MH - Dermatologic Surgical Procedures/methods MH - Female MH - Humans MH - Male MH - Prognosis MH - Raynaud Disease/diagnosis/*therapy MH - Scleroderma, Localized/*diagnosis/*therapy MH - Severity of Illness Index MH - Skin Ulcer/physiopathology/*therapy MH - Upper Extremity EDAT- 2018/09/21 06:00 MHDA- 2019/01/10 06:00 CRDT- 2018/09/21 06:00 PHST- 2018/09/21 06:00 [entrez] PHST- 2018/09/21 06:00 [pubmed] PHST- 2019/01/10 06:00 [medline] AID - 00129334-201810000-00005 [pii] AID - 10.1097/01.ASW.0000544615.18501.fd [doi] PST - ppublish SO - Adv Skin Wound Care. 2018 Oct;31(10):446-455. doi: 10.1097/01.ASW.0000544615.18501.fd. PMID- 24760110 OWN - NLM STAT- MEDLINE DCOM- 20151117 LR - 20211021 IS - 1573-2592 (Electronic) IS - 0271-9142 (Linking) VI - 34 IP - 6 DP - 2014 Aug TI - Early systemic sclerosis: serum profiling of factors involved in endothelial, T-cell, and fibroblast interplay is marked by elevated interleukin-33 levels. PG - 663-8 LID - 10.1007/s10875-014-0037-0 [doi] AB - PURPOSE: To assess the serum profile of factors involved in endothelial, T-cell, and fibroblast interplay in patients with Raynaud's phenomenon (RP) associated with nailfold vodeocapillaroscopy (NVC) scleroderma findings and/or systemic sclerosis (SSc) marker autoantibodies, recently labeled as early SSc patients. METHODS: Serum levels of soluble intercellular adhesion molecule-1 (sICAM-1), soluble vascular adhesion molecule-1 (sVCAM-1), CCL2, CXCL8, IL-13, IL-33, and transforming growth factor-β (TGF-β) were measured in 24 early SSc patients, 48 definite SSc patients, and 24 osteoarthritis/fibromyalgia controls by multiplex suspension immunoassay. All SSc patients were investigated for the presence/absence of preclinical and clinical organ involvement, SSc marker autoantibodies, and NVC abnormalities. RESULTS: Serum sICAM-1, CCL2, CXCL8, and IL-13 were increased in all SSc patients as compared to controls, and paralleled the severity of the disease subset (early SSc < limited cutaneous SSc < diffuse cutaneous SSc; p < 0.0001). Surprisingly, IL-33 was significantly higher in early SSc patients as compared to both controls (p < 0.01) and definite SSc patients (p < 0.05). In early SSc there were no differences in the investigated markers according to the functional and serological features assessed. CONCLUSIONS: Our study suggests that an endothelial, T-cell and fibroblast activation can be present in patients with early SSc and it is associated with a distinct profile of circulating factors involved in the cross-talk of these cells. The marked increase of IL-33 in early SSc patients suggests new routes of investigation of cell-cell dynamics in target tissues predating overt disease manifestations, thus opening to new therapeutic approaches. FAU - Vettori, Serena AU - Vettori S AD - Rheumatology Unit, Second University of Naples, Via S. Pansini 5, 80131, Naples, Italy, serenavettori@libero.it. FAU - Cuomo, Giovanna AU - Cuomo G FAU - Iudici, Michele AU - Iudici M FAU - D'Abrosca, Virginia AU - D'Abrosca V FAU - Giacco, Veronica AU - Giacco V FAU - Barra, Giusi AU - Barra G FAU - De Palma, Raffaele AU - De Palma R FAU - Valentini, Gabriele AU - Valentini G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140424 PL - Netherlands TA - J Clin Immunol JT - Journal of clinical immunology JID - 8102137 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Chemokine CCL2) RN - 0 (IL33 protein, human) RN - 0 (Interleukin-13) RN - 0 (Interleukin-33) RN - 0 (Interleukin-8) RN - 0 (Interleukins) RN - 126547-89-5 (Intercellular Adhesion Molecule-1) SB - IM MH - Adult MH - Animals MH - Antibodies, Antinuclear/metabolism MH - Biomarkers/*metabolism MH - Capillaries/pathology MH - Cell Communication MH - Cells, Cultured MH - Chemokine CCL2/blood MH - Disease Progression MH - Endothelial Cells/*immunology MH - Female MH - Fibroblasts/*immunology MH - Humans MH - Intercellular Adhesion Molecule-1/blood MH - Interleukin-13/blood MH - Interleukin-33 MH - Interleukin-8/blood MH - Interleukins/*metabolism MH - Male MH - Mice MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*diagnosis/immunology MH - Scleroderma, Systemic/*diagnosis/immunology MH - T-Lymphocytes/*immunology EDAT- 2014/04/25 06:00 MHDA- 2015/11/18 06:00 CRDT- 2014/04/25 06:00 PHST- 2013/12/17 00:00 [received] PHST- 2014/04/02 00:00 [accepted] PHST- 2014/04/25 06:00 [entrez] PHST- 2014/04/25 06:00 [pubmed] PHST- 2015/11/18 06:00 [medline] AID - 10.1007/s10875-014-0037-0 [doi] PST - ppublish SO - J Clin Immunol. 2014 Aug;34(6):663-8. doi: 10.1007/s10875-014-0037-0. Epub 2014 Apr 24. PMID- 19886888 OWN - NLM STAT- MEDLINE DCOM- 20101006 LR - 20230215 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 162 IP - 4 DP - 2010 Apr TI - Serum levels of soluble vascular endothelial growth factor receptor-2 in patients with systemic sclerosis. PG - 751-8 LID - 10.1111/j.1365-2133.2009.09567.x [doi] AB - BACKGROUND: Although vascular endothelial growth factor (VEGF)-A/VEGF receptor 2 (KDR) signalling may play a major role in the microangiopathy of systemic sclerosis (SSc), serum levels of soluble KDR (sKDR) in this disease have not yet been determined. OBJECTIVES: To evaluate the possibility that serum sKDR levels can be a specific disease marker of SSc. METHODS: Serum sKDR levels of 42 patients with SSc, 10 patients with Raynaud's phenomenon (RP) and 22 healthy controls were measured with specific enzyme-linked immunosorbent assays. Quantitative real-time polymerase chain reaction (PCR) was performed to determine KDR mRNA levels. RESULTS: In females, the serum sKDR levels were significantly higher in patients with SSc, especially limited cutaneous SSc, than in patients with RP or healthy controls. Quantitative real-time PCR with RNA from skin sections revealed that KDR mRNA levels were also increased in the skin of patients with SSc with elevated serum sKDR levels. A significantly lower prevalence of pulmonary fibrosis, higher percentage vital capacity, and a higher incidence of telangiectasia were seen in female patients with SSc with elevated serum sKDR levels than those with normal levels. CONCLUSIONS: These results suggest that the skin can be one of the sources of elevated serum sKDR levels, and that serum sKDR levels are useful for diagnosis and may be a marker of microangiopathy in patients with SSc, especially females. The VEGF-A/KDR signalling system may be involved in the pathogenesis of the disease. FAU - Jinnin, M AU - Jinnin M AD - Department of Dermatology and Plastic Surgery, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan. mjin@kumamoto-u.ac.jp FAU - Makino, T AU - Makino T FAU - Kajihara, I AU - Kajihara I FAU - Honda, N AU - Honda N FAU - Makino, K AU - Makino K FAU - Ogata, A AU - Ogata A FAU - Ihn, H AU - Ihn H LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20091103 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Biomarkers) RN - 0 (RNA, Messenger) RN - EC 2.7.10.1 (Vascular Endothelial Growth Factor Receptor-2) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Biomarkers/blood MH - Case-Control Studies MH - Child MH - Collagen Diseases/*blood/pathology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Male MH - Middle Aged MH - Polymerase Chain Reaction MH - RNA, Messenger/metabolism MH - Raynaud Disease/*blood/pathology MH - Scleroderma, Systemic/*blood/pathology MH - Severity of Illness Index MH - Sex Factors MH - Statistics as Topic MH - Vascular Endothelial Growth Factor Receptor-2/*blood MH - Young Adult EDAT- 2009/11/06 06:00 MHDA- 2010/10/07 06:00 CRDT- 2009/11/06 06:00 PHST- 2009/11/06 06:00 [entrez] PHST- 2009/11/06 06:00 [pubmed] PHST- 2010/10/07 06:00 [medline] AID - BJD9567 [pii] AID - 10.1111/j.1365-2133.2009.09567.x [doi] PST - ppublish SO - Br J Dermatol. 2010 Apr;162(4):751-8. doi: 10.1111/j.1365-2133.2009.09567.x. Epub 2009 Nov 3. PMID- 34260723 OWN - NLM STAT- MEDLINE DCOM- 20220413 LR - 20260120 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 61 IP - 4 DP - 2022 Apr 11 TI - Evidence and consensus-based recommendations for non-pharmacological treatment of fatigue, hand function loss, Raynaud's phenomenon and digital ulcers in patients with systemic sclerosis. PG - 1476-1486 LID - 10.1093/rheumatology/keab537 [doi] AB - OBJECTIVE: SSc is a complex CTD affecting mental and physical health. Fatigue, hand function loss, and RP are the most prevalent disease-specific symptoms of systemic sclerosis. This study aimed to develop consensus and evidence-based recommendations for non-pharmacological treatment of these symptoms. METHODS: A multidisciplinary task force was installed comprising 20 Dutch experts. After agreeing on the method for formulating the recommendations, clinically relevant questions about patient education and treatments were inventoried. During a face-to-face task force meeting, draft recommendations were generated through a systematically structured discussion, following the nominal group technique. To support the recommendations, an extensive literature search was conducted in MEDLINE and six other databases until September 2020, and 20 key systematic reviews, randomized controlled trials, and published recommendations were selected. Moreover, 13 Dutch medical specialists were consulted on non-pharmacological advice regarding RP and digital ulcers. For each recommendation, the level of evidence and the level of agreement was determined. RESULTS: Forty-one evidence and consensus-based recommendations were developed, and 34, concerning treatments and patient education of fatigue, hand function loss, and RP/digital ulcers-related problems, were approved by the task force. CONCLUSIONS: These 34 recommendations provide guidance on non-pharmacological treatment of three of the most frequently described symptoms in patients with systemic sclerosis. The proposed recommendations can guide referrals to health professionals, inform the content of non-pharmacological interventions, and can be used in the development of national and international postgraduate educational offerings. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Stöcker, Juliane K AU - Stöcker JK AUID- ORCID: 0000-0002-3852-1157 AD - Department of Research, Sint Maartenskliniek. AD - Musculoskeletal Rehabilitation Research Group, HAN University of Applied Sciences. AD - Department of Rheumatic Diseases, Radboud University Medical Center, Nijmegen. FAU - Schouffoer, Anne A AU - Schouffoer AA AD - Department of Rheumatology, Leiden University Medical Center, Leiden. AD - Department of Rheumatology, Haga Teaching Hospital, The Hague. FAU - Spierings, Julia AU - Spierings J AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. AD - Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College London, London, UK. FAU - Schriemer, Marisca R AU - Schriemer MR AD - Department of Research, Sint Maartenskliniek. AD - National Association for People with Lupus, Systemic Sclerosis, Antiphospholipid Syndrome, and Mixed Connective Tissue Disease, Utrecht. FAU - Potjewijd, Judith AU - Potjewijd J AD - Department of Clinical Immunology, Maastricht University Medical Center, Maastricht. FAU - de Pundert, Lian AU - de Pundert L AD - Department of Physical Therapy, Haga Teaching Hospital, The Hague. AD - Department of Rehabilitation, Physical Therapy Science and Sport, UMC Utrecht Brain Center, University Medical Center Utrecht, Utrecht. FAU - van den Hoogen, Frank H J AU - van den Hoogen FHJ AD - Department of Research, Sint Maartenskliniek. AD - Department of Rheumatic Diseases, Radboud University Medical Center, Nijmegen. FAU - Nijhuis-van der Sanden, Maria W G AU - Nijhuis-van der Sanden MWG AD - Radboud Institute for Health Sciences, IQ Healthcare, Radboud University Medical Center. FAU - Staal, J Bart AU - Staal JB AD - Musculoskeletal Rehabilitation Research Group, HAN University of Applied Sciences. AD - Radboud Institute for Health Sciences, IQ Healthcare, Radboud University Medical Center. FAU - Satink, Ton AU - Satink T AD - Research Group Neuro Rehabilitation, HAN University of Applied Sciences, Nijmegen. AD - European Masters of Science in Occupational Therapy, Amsterdam, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatic Diseases, Radboud University Medical Center, Nijmegen. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AUID- ORCID: 0000-0002-4352-2824 AD - Department of Research, Sint Maartenskliniek. AD - Department of Rheumatic Diseases, Radboud University Medical Center, Nijmegen. CN - Arthritis Research and Collaboration Hub study group LA - eng PT - Consensus Statement PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Fatigue/etiology/therapy MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy MH - *Scleroderma, Systemic/drug therapy/therapy MH - *Skin Ulcer/drug therapy/therapy MH - Ulcer PMC - PMC8996778 OTO - NOTNLM OT - RP OT - SSc OT - digital ulcers OT - fatigue OT - hand function loss EDAT- 2021/07/15 06:00 MHDA- 2022/04/14 06:00 PMCR- 2021/07/14 CRDT- 2021/07/14 17:37 PHST- 2021/04/15 00:00 [received] PHST- 2021/06/25 00:00 [revised] PHST- 2021/07/15 06:00 [pubmed] PHST- 2022/04/14 06:00 [medline] PHST- 2021/07/14 17:37 [entrez] PHST- 2021/07/14 00:00 [pmc-release] AID - 6321467 [pii] AID - keab537 [pii] AID - 10.1093/rheumatology/keab537 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Apr 11;61(4):1476-1486. doi: 10.1093/rheumatology/keab537. PMID- 38251814 OWN - NLM STAT- MEDLINE DCOM- 20240925 LR - 20241023 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 38 IP - 10 DP - 2024 Oct TI - Nailfold dermoscopy predicts the absence of a capillaroscopy sclerodermic pattern: The multicentre, prospective VASCUL-R trial. PG - 1982-1987 LID - 10.1111/jdv.19803 [doi] AB - BACKGROUND: Nailfold capillaroscopy is recommended to diagnose primary or secondary Raynaud's phenomenon (RP). Capillaroscopy is normal in primary RP, which is the most frequent. Screening for RP capillary anomalies with nailfold dermoscopy has been promising. OBJECTIVE: To determine whether normal nailfold dermoscopy-based on the absence of five criteria that define a sclerodermic pattern-is able to predict normal capillaroscopy with good positive-predictive value (PPV). METHODS: Prospective, 2-phase (monocentre and multicentre) study on patients at first consultation for RP undergoing nailfold video capillaroscopy (NVC) and nailfold dermoscopy by two different 'blinded' trained observers, respectively, a vascular specialist and a dermatologist, not familiar with capillaroscopy. The five criteria noted were as follows: disorganization, megacapillaries, low capillary density, avascular areas and haemorrhages. RESULTS: Based on 105 patients, the dermoscopy PPV for a normal NVC was 100% (p = 0.015), with 37.9% sensitivity, when no criterion was observed. Excluding haemorrhages, the PPV remained 100% (p < 0.0001), with sensitivity rising to 73.7% and 100% specificity. CONCLUSIONS: Normal nailfold dermoscopy with the absence of four easy-to-observe criteria predicts normal NVC with an excellent PPV. CI - © 2024 European Academy of Dermatology and Venereology. FAU - Monfort, Jean-Benoît AU - Monfort JB AUID- ORCID: 0000-0002-4792-2622 AD - Service de Dermatologie et Médecine Vasculaire, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Sorbonne Université, Paris, France. FAU - Klejtman, Tiffany AU - Klejtman T AUID- ORCID: 0000-0003-1645-9281 AD - Service de Médecine Vasculaire, Institut de la Cicatrisation Jean-Paul-Belmondo, Groupe Hospitalier Paris Saint-Joseph, Paris, France. FAU - Lazareth, Isabelle AU - Lazareth I AD - Service de Médecine Vasculaire, Institut de la Cicatrisation Jean-Paul-Belmondo, Groupe Hospitalier Paris Saint-Joseph, Paris, France. FAU - Kottler, Diane AU - Kottler D AD - Service de Dermatologie, Centre Hospitalier Universitaire de Caen, Caen, France. FAU - Blaise, Sophie AU - Blaise S AD - Department of Vascular Medicine, Grenoble Alpes University Hospital, Grenoble, France. FAU - Imbert, Bernard AU - Imbert B AD - Department of Vascular Medicine, Grenoble Alpes University Hospital, Grenoble, France. FAU - Chaby, Guillaume AU - Chaby G AD - Service de Dermatologie, Centre Hospitalier Amiens, Amiens, France. FAU - Lok, Catherine AU - Lok C AD - Service de Dermatologie, Centre Hospitalier Amiens, Amiens, France. FAU - Maillard, Hervé AU - Maillard H AD - Service de Dermatologie, Centre Hospitalier Le Mans, Le Mans, France. FAU - Beneton, Nathalie AU - Beneton N AD - Service de Dermatologie, Centre Hospitalier Le Mans, Le Mans, France. FAU - Journet-Tollhupp, Julie AU - Journet-Tollhupp J AD - Service de Dermatologie, Centre Hospitalier Chalon-sur-Saône, Chalon-sur-Saône, France. FAU - Goujon, Elisa AU - Goujon E AD - Service de Dermatologie, Centre Hospitalier Chalon-sur-Saône, Chalon-sur-Saône, France. FAU - Jacquin, Aurélien AU - Jacquin A AD - Service de Dermatologie et Médecine Vasculaire, Centre Hospitalier Victor-Dupouy, Argenteuil, France. FAU - Tella, Emilie AU - Tella E AD - Service de Dermatologie et Médecine Vasculaire, Centre Hospitalier Victor-Dupouy, Argenteuil, France. FAU - Mboup, Bassirou AU - Mboup B AD - Unité de Recherche Clinique, Hôpital Fernand-Widal, Paris, France. FAU - Vicaut, Eric AU - Vicaut E AD - Unité de Recherche Clinique, Hôpital Fernand-Widal, Paris, France. FAU - Senet, Patricia AU - Senet P AD - Service de Dermatologie et Médecine Vasculaire, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Sorbonne Université, Paris, France. CN - Groupe d'Angio‐Dermatologie de la Société Française de Dermatologie LA - eng GR - Société Française de Dermatologie et de Pathologie Sexuellement Transmissible/ PT - Journal Article PT - Multicenter Study DEP - 20240122 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 SB - IM MH - Humans MH - Prospective Studies MH - *Microscopic Angioscopy/methods MH - Female MH - Male MH - Middle Aged MH - *Dermoscopy/methods MH - *Nails/blood supply/diagnostic imaging MH - Adult MH - Raynaud Disease/diagnosis/diagnostic imaging MH - Aged MH - Predictive Value of Tests MH - Capillaries/diagnostic imaging/pathology EDAT- 2024/01/22 12:42 MHDA- 2024/09/25 16:17 CRDT- 2024/01/22 08:20 PHST- 2023/03/15 00:00 [received] PHST- 2023/11/28 00:00 [accepted] PHST- 2024/09/25 16:17 [medline] PHST- 2024/01/22 12:42 [pubmed] PHST- 2024/01/22 08:20 [entrez] AID - 10.1111/jdv.19803 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2024 Oct;38(10):1982-1987. doi: 10.1111/jdv.19803. Epub 2024 Jan 22. PMID- 20420843 OWN - NLM STAT- MEDLINE DCOM- 20101116 LR - 20161125 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 80 IP - 2 DP - 2010 Sep TI - Digital laser doppler flowmetry may discriminate "limited" from "diffuse" systemic sclerosis. PG - 221-6 LID - 10.1016/j.mvr.2010.04.006 [doi] AB - OBJECTIVES: To investigate skin blood flux and microvascular functional changes by laser Doppler flowmetry (LD) in patients with systemic sclerosis (SSc) at baseline and following dynamic stimulations. METHODS: Skin blood flux of the dorsal hands was recorded by LD at baseline and after the cold test and the post-occlusive hyperemia test in 59 SSc patients (49 limited cutaneous, 10 diffuse cutaneous). Twenty-five patients with primary Raynaud's phenomenon (PRP), and 31 healthy donors (HD) were studied as controls. RESULTS: After the cold test, SSc patients had a significantly higher reduction of the blood flux (-38.4%+/-28) than PRP (-21.1%+/-37) and HD (-22.1%+/-23) subjects (p<0.05). Within the SSc group, the cold test flux was significantly reduced in limited-SSc (-399%+/-28, p<0.05), but not in diffuse-SSc (-31.2%+/-29), whereas, the time needed to recover the basal flux after the occlusive/ischemic test was significantly longer in diffuse-SSc (18.8s+/-21)than in limited-SSc (4.5s+/-4, p<0.01) or HD (2.2s+/-2, p<0.01) or PRP (0.4s+/-0.7, p<0.01). CONCLUSIONS: These data clearly indicate an impairment of vascular tone regulatory mechanisms in SSc and suggest that a peculiar pathogenic mechanism may be involved in different SSc subset. Nevertheless, it has clear that PRP and SSc-associated RP have a distinct pattern at LD evaluation, and monitoring patients with PRP could be helpful to understand whether a change in the LD pattern might predict the development of SSc. CI - Copyright 2010 Elsevier Inc. All rights reserved. FAU - Grattagliano, Vito AU - Grattagliano V AD - DiMIMP, Rheumatology Unit, Clinica delle Malattie Infettive, University of Bari, Italy. FAU - Iannone, Florenzo AU - Iannone F FAU - Praino, Emanuela AU - Praino E FAU - De Zio, Alessandra AU - De Zio A FAU - Riccardi, Maria Teresa AU - Riccardi MT FAU - Carrozzo, Norma AU - Carrozzo N FAU - Covelli, Michele AU - Covelli M FAU - Maggi, Paolo AU - Maggi P FAU - Lapadula, Giovanni AU - Lapadula G LA - eng PT - Journal Article DEP - 20100424 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - *Blood Flow Velocity MH - Capillaries/diagnostic imaging/pathology/physiopathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Laser-Doppler Flowmetry/*methods MH - Male MH - Microcirculation MH - Middle Aged MH - Nails/blood supply MH - Raynaud Disease/diagnostic imaging/pathology MH - *Regional Blood Flow MH - Scleroderma, Diffuse/diagnostic imaging/*pathology/physiopathology MH - Scleroderma, Limited/diagnostic imaging/*pathology/physiopathology MH - Skin/*blood supply/pathology MH - Ultrasonography EDAT- 2010/04/28 06:00 MHDA- 2010/11/17 06:00 CRDT- 2010/04/28 06:00 PHST- 2010/01/28 00:00 [received] PHST- 2010/04/11 00:00 [revised] PHST- 2010/04/14 00:00 [accepted] PHST- 2010/04/28 06:00 [entrez] PHST- 2010/04/28 06:00 [pubmed] PHST- 2010/11/17 06:00 [medline] AID - S0026-2862(10)00086-5 [pii] AID - 10.1016/j.mvr.2010.04.006 [doi] PST - ppublish SO - Microvasc Res. 2010 Sep;80(2):221-6. doi: 10.1016/j.mvr.2010.04.006. Epub 2010 Apr 24. PMID- 32921028 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20260127 IS - 1120-9763 (Print) IS - 1120-9763 (Linking) VI - 44 IP - 4 DP - 2020 Jul-Aug TI - The burden of air pollution and temperature on Raynaud's phenomenon secondary to systemic sclerosis. PG - 218-227 LID - 10.19191/EP20.4.P228.052 [doi] AB - OBJECTIVES: to evaluate the effect of air pollution (ozone - O3 and particulate matter <=10 μm and <=2.5 μm - PM10 and PM2.5) on the severity of Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). DESIGN: cross-sectional, observational, and single centre study. SETTING AND PARTICIPANTS: all consecutive SSc patients residing in Lombardy (Northern Italy) were enrolled. PM10, PM2.5, and O3 concentrations were calculated for each patient at municipality resolution in the week before the evaluation. Similar considerations were made for meteorological variables (temperature and humidity). MAIN OUTCOME MEASURES: patients were asked to assess RP severity during the week before the evaluation according to a visual analogue scale (VAS). Ordinal logistic regression models were fitted to evaluate the short-term effect of temperature and air pollution with respect to RP. A univariate linear regression model was created to consider the association between temperature and pollutants. RESULTS: in this study, 87 SSc patients were enrolled. Temperature was confirmed to strongly influence RP severity. PM10 and PM.5 were found to significantly worsen RP severity for the first four days before the evaluation, including the day of the visit, and as mean up to six days before the evaluation. O3 seemed to exert a protective effect on RP severity that was significant for the first four days before the evaluation, including the day of the visit, and as mean up to seven days before the evaluation. CONCLUSIONS: since the overwhelming effect of temperature on RP, final conclusions about the exact contribution of pollutants on RP severity cannot be drawn because of the strong inter-correlation between air pollution and temperature. FAU - Schioppo, Tommaso AU - Schioppo T AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). FAU - De Lucia, Orazio AU - De Lucia O AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). FAU - Murgo, Antonella AU - Murgo A AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). FAU - Caporali, Roberto AU - Caporali R AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy). FAU - Orenti, Annalisa AU - Orenti A AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy). AD - Laboratory of Medical Statistics, Epidemiology and Biometry GA Maccacaro, Milan, (Italy). FAU - Boracchi, Patrizia AU - Boracchi P AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy). AD - Laboratory of Medical Statistics, Epidemiology and Biometry GA Maccacaro, Milan, (Italy). FAU - Iodice, Simona AU - Iodice S AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy). AD - Laboratory of Epidemiology, Epigenetics e Toxicology (EPIGET), Università degli Studi di Milano, Milan (Italy). FAU - Ubiali, Tania AU - Ubiali T AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). FAU - Bollati, Valentina AU - Bollati V AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy). AD - Laboratory of Epidemiology, Epigenetics e Toxicology (EPIGET), Università degli Studi di Milano, Milan (Italy). FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Clinical Rheumatology, Azienda Socio Sanitaria Territoriale Pini-CTO, Milan (Italy). AD - Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan (Italy); francesca.ingegnoli@unimi.it. LA - eng PT - Journal Article TT - Il contributo dell'inquinamento atmosferico e della temperatura sul fenomeno di Raynaud secondario a sclerosi sistemica. PL - Italy TA - Epidemiol Prev JT - Epidemiologia e prevenzione JID - 8902507 RN - 0 (Air Pollutants) RN - 0 (Particulate Matter) SB - IM MH - *Air Pollutants/analysis/toxicity MH - Air Pollution/analysis/*statistics & numerical data MH - Cities MH - Cross-Sectional Studies MH - Humans MH - Italy/epidemiology MH - Particulate Matter/adverse effects/analysis MH - Raynaud Disease/*epidemiology MH - *Scleroderma, Systemic/complications/epidemiology MH - *Temperature EDAT- 2020/09/15 06:00 MHDA- 2021/06/24 06:00 CRDT- 2020/09/14 04:26 PHST- 2020/09/14 04:26 [entrez] PHST- 2020/09/15 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] AID - 10.19191/EP20.4.P228.052 [doi] PST - ppublish SO - Epidemiol Prev. 2020 Jul-Aug;44(4):218-227. doi: 10.19191/EP20.4.P228.052. PMID- 29698501 OWN - NLM STAT- MEDLINE DCOM- 20180806 LR - 20210109 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 13 IP - 4 DP - 2018 TI - Association of Raynaud's phenomenon with a polymorphism in the NOS1 gene. PG - e0196279 LID - 10.1371/journal.pone.0196279 [doi] LID - e0196279 AB - BACKGROUND: Raynaud's phenomenon (RP) describes the phenomenon of recurrent vasospasm of digital arteries, associated with skin colour changes: pallor, cyanosis and erythema. Twin studies have indicated a genetic predisposition for RP; however, the precise aetiology of RP remains unknown. It is thought that genetic variation in temperature-responsive or vasospastic genes might underlie RP so performed a candidate gene study in a large, population based sample. We assessed the association between RP and single nucleotide polymorphisms (SNPs) in the TRPA1, TRPM8, CALCA, CALCB and NOS1 genes. METHODS: Analysis included a total of 4276 individuals from the TwinsUK database. RP status had been determined using validated, self-administered questionnaires and was diagnosed in 640 individuals (17.6%). 66 tag SNPs across the candidate genes were tested for association with RP status using a linear regression model, accounting for covariates. Adjustment was made for multiple testing. RegulomeDB and GTEx databases were used to assess possible functional effects of the polymorphisms. RESULTS: Nominally significant associations between RP and four SNPs in NOS1 and one in CALCB were identified. After permutation testing, rs527590 SNP in NOS1 passed the significance threshold. RegulomeDB scores indicated an unlikely functional effect of this variant, while the survey of the GTEx database found the SNP and several variants in linkage disequilibrium to be cis-eQTLs in skin. CONCLUSION: Results indicate that RP is associated with variation in gene NOS1. This finding may be related to the observation that the significant SNP in NOS1 is known to exhibit functional influence on the gene expression. FAU - Munir, Sabrina AU - Munir S AUID- ORCID: 0000-0002-4669-869X AD - Department of Twin Research and Genetic Epidemiology, King's College London, London, United Kingdom. FAU - Freidin, Maxim B AU - Freidin MB AD - Department of Twin Research and Genetic Epidemiology, King's College London, London, United Kingdom. FAU - Brain, Susan AU - Brain S AD - Section of Vascular Biology & Inflammation, BHF Centre for Cardiovascular Excellence, School of Cardiovascular Medicine and Sciences, King's College London, London, United Kingdom. FAU - Williams, Frances M K AU - Williams FMK AD - Department of Twin Research and Genetic Epidemiology, King's College London, London, United Kingdom. LA - eng GR - WT_/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Twin Study DEP - 20180426 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - 0 (CALCA protein, human) RN - 0 (CALCB protein, human) RN - 0 (TRPA1 Cation Channel) RN - 0 (TRPA1 protein, human) RN - 0 (TRPM Cation Channels) RN - 0 (TRPM8 protein, human) RN - EC 1.14.13.39 (NOS1 protein, human) RN - EC 1.14.13.39 (Nitric Oxide Synthase Type I) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Calcitonin Gene-Related Peptide/genetics MH - Diseases in Twins MH - Female MH - *Genetic Predisposition to Disease MH - Genetic Variation MH - Humans MH - Male MH - Middle Aged MH - Nitric Oxide Synthase Type I/*genetics MH - *Polymorphism, Single Nucleotide MH - Raynaud Disease/*genetics MH - Skin/pathology MH - TRPA1 Cation Channel/genetics MH - TRPM Cation Channels/genetics MH - Temperature MH - Young Adult PMC - PMC5919461 COIS- Competing Interests: The authors have declared that no competing interests exist. EDAT- 2018/04/27 06:00 MHDA- 2018/08/07 06:00 PMCR- 2018/04/26 CRDT- 2018/04/27 06:00 PHST- 2018/01/10 00:00 [received] PHST- 2018/04/10 00:00 [accepted] PHST- 2018/04/27 06:00 [entrez] PHST- 2018/04/27 06:00 [pubmed] PHST- 2018/08/07 06:00 [medline] PHST- 2018/04/26 00:00 [pmc-release] AID - PONE-D-18-00916 [pii] AID - 10.1371/journal.pone.0196279 [doi] PST - epublish SO - PLoS One. 2018 Apr 26;13(4):e0196279. doi: 10.1371/journal.pone.0196279. eCollection 2018. PMID- 24803229 OWN - NLM STAT- MEDLINE DCOM- 20150727 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 33 IP - 12 DP - 2014 Dec TI - Takayasu's arteritis in Arabs. PG - 1777-83 LID - 10.1007/s10067-014-2633-z [doi] AB - The objective of this study was to describe epidemiological and clinical features of Takayasu's arteritis (TA) among Arab populations and to compare it to other populations. We conducted a systematic review of reports about TA from Arab countries published in English and French until 2013. All published papers were reviewed including original research and case reports. There were 197 patients (176 females) reported in 28 publications that comprised 8 original research publications (with a total of 163 patients) and 20 case reports (reporting 34 patients). These patients were from countries with a total population of approximately 80 million (Tunisia, Morocco, Jordan, Lebanon, Kuwait, Saudi Arabia, and Bahrain). The female to male ratio was 7:1. Mean age at disease onset was 28 years, and the mean delay in diagnosis was 3.5 years. Clinical manifestations are constitutional symptoms in 44 %, limb claudication in 64 %, Raynaud's in 6 %, erythema nodosum in 3.6 %, visual disturbances in 30 %, carotidynia in 7 %, neurologic manifestations in 56 %, and hypertension in 34.5 % of patients. Involvement of the aortic arch and its branches were observed in about 80 % of patients. The overall mortality was very low over a period of 5.4 years of follow-up, and the course of the disease was quite stable in about 50 % of patients. The demographical and clinical findings of TA in Arabs are similar to what has been reported from different parts of the world. A relatively long delay in diagnosis may be in part due to low awareness of a relatively rare disease. FAU - Mustafa, Khader N AU - Mustafa KN AD - Division of Rheumatology, Department of Internal Medicine, Jordan University Hospital, The University of Jordan, Queen Rania St, PO Box 13046, Amman, 11942, Jordan, kmustafa@ju.edu.jo. LA - eng PT - Journal Article DEP - 20140507 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Arabs MH - Erythema Nodosum/diagnosis MH - Female MH - Humans MH - Hypertension/diagnosis MH - Intermittent Claudication/diagnosis MH - Male MH - Middle East MH - Nervous System Diseases/diagnosis MH - Raynaud Disease/diagnosis MH - Takayasu Arteritis/*epidemiology/*ethnology MH - Treatment Outcome MH - Young Adult EDAT- 2014/05/08 06:00 MHDA- 2015/07/28 06:00 CRDT- 2014/05/08 06:00 PHST- 2013/06/28 00:00 [received] PHST- 2014/04/15 00:00 [accepted] PHST- 2014/01/23 00:00 [revised] PHST- 2014/05/08 06:00 [entrez] PHST- 2014/05/08 06:00 [pubmed] PHST- 2015/07/28 06:00 [medline] AID - 10.1007/s10067-014-2633-z [doi] PST - ppublish SO - Clin Rheumatol. 2014 Dec;33(12):1777-83. doi: 10.1007/s10067-014-2633-z. Epub 2014 May 7. PMID- 25172934 OWN - NLM STAT- MEDLINE DCOM- 20150326 LR - 20150122 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 54 IP - 2 DP - 2015 Feb TI - Proteomic identification of heterogeneous nuclear ribonucleoprotein K as a novel cold-associated autoantigen in patients with secondary Raynaud's phenomenon. PG - 349-58 LID - 10.1093/rheumatology/keu325 [doi] AB - OBJECTIVE: The aim of this study was to identify cold-associated autoantibodies in patients with RP secondary to CTDs. METHODS: Indirect immunofluorescence staining was performed on non-permeabilized cold-stimulated normal human dermal microvascular endothelial cells (dHMVECs), using patients' sera. Cold-induced alterations in cell surface proteomes were analysed by isobaric tag for relative and absolute quantitation (iTRAQ) analysis. Serological proteome analysis (SERPA) was applied to screen cold-associated autoantigens. The prevalence of the candidate autoantibody was determined by ELISA in 290 patients with RP secondary to CTDs (SSc, SLE or MCTD), 10 patients with primary RP and 27 healthy controls. RESULTS: Enhanced cell surface immunoreactivity was detected in cold-stimulated dHMVECs when incubated with sera from patients with secondary RP. By iTRAQ analysis, many proteins, including heterogeneous nuclear ribonucleoprotein K (hnRNP-K), were found to be increased on the cell surface of dHMVECs after cold stimulation. By the SERPA approach, hnRNP-K was identified as a candidate autoantigen in patients with secondary RP. Cold-induced translocation of hnRNP-K to the cell surface was confirmed by immunoblotting and flow cytometry. By ELISA analysis, patients with secondary RP show a significantly higher prevalence of anti-hnRNP-K autoantibody (30.0%, 61/203) than patients without RP (9.2%, 8/87, P = 0.0001), patients with primary RP (0%, 0/10, P = 0.0314) or healthy controls (0%, 0/27, P = 0.0001). CONCLUSION: By comprehensive proteomics, we identified hnRNP-K as a novel cold-associated autoantigen in patients with secondary RP. Anti-hnRNP-K autoantibody may potentially serve as a biomarker for RP secondary to various CTDs. CI - © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Yang, Lingli AU - Yang L AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Fujimoto, Minoru AU - Fujimoto M AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Murota, Hiroyuki AU - Murota H AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Serada, Satoshi AU - Serada S AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Honda, Hiromi AU - Honda H AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Yamada, Kohji AU - Yamada K AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Suzuki, Katsuya AU - Suzuki K AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Nishikawa, Ayumi AU - Nishikawa A AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Hosono, Yuji AU - Hosono Y AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Yoneda, Yoshihiro AU - Yoneda Y AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Takehara, Kazuhiko AU - Takehara K AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Imura, Yoshitaka AU - Imura Y AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Mimori, Tsuneyo AU - Mimori T AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Takeuchi, Tsutomu AU - Takeuchi T AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Katayama, Ichiro AU - Katayama I AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. FAU - Naka, Tetsuji AU - Naka T AD - Department of Dermatology, Osaka University Graduate School of Medicine, Laboratory of Immune Signal, National Institute of Biomedical Innovation, Department of Dermatology, Kanazawa University, Kanazawa, Biomolecular Dynamics Group, Graduate School of Frontier Biosciences, Osaka University, Osaka, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto and National Institute of Biomedical Innovation, Osaka, Japan. tnaka@nibio.go.jp. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140828 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Heterogeneous-Nuclear Ribonucleoprotein K) SB - IM MH - Autoantibodies/metabolism MH - Autoantigens/*metabolism MH - Autoimmunity/physiology MH - Case-Control Studies MH - Cells, Cultured MH - *Cold Temperature MH - Endothelial Cells/metabolism MH - Female MH - Heterogeneous-Nuclear Ribonucleoprotein K/*metabolism MH - Humans MH - Male MH - Middle Aged MH - Proteomics/methods MH - Raynaud Disease/*immunology OTO - NOTNLM OT - Raynaud’s phenomenon OT - autoantibody OT - heterogeneous nuclear RNP-K OT - proteomics EDAT- 2014/08/31 06:00 MHDA- 2015/03/27 06:00 CRDT- 2014/08/31 06:00 PHST- 2014/08/31 06:00 [entrez] PHST- 2014/08/31 06:00 [pubmed] PHST- 2015/03/27 06:00 [medline] AID - keu325 [pii] AID - 10.1093/rheumatology/keu325 [doi] PST - ppublish SO - Rheumatology (Oxford). 2015 Feb;54(2):349-58. doi: 10.1093/rheumatology/keu325. Epub 2014 Aug 28. PMID- 19644893 OWN - NLM STAT- MEDLINE DCOM- 20090910 LR - 20191210 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 61 IP - 8 DP - 2009 Aug 15 TI - Noninvasive imaging techniques in the assessment of scleroderma spectrum disorders. PG - 1103-11 LID - 10.1002/art.24645 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) affects both microvascular structure and function. Laser Doppler imaging (LDI) and thermal imaging can be used to measure cutaneous blood vessel function. Nailfold capillaroscopy (NC) measures capillary morphology. The aim of this study was to investigate the relationship between capillary morphology and blood flow, and to determine which combination of techniques allows the best discrimination between patients with SSc, primary Raynaud's phenomenon (RP), and healthy controls. METHODS: NC was performed in 16 patients with SSc, 14 patients with primary RP, and 16 healthy controls. In addition, participants underwent cold stimulus with cold water. Hands were imaged to monitor rewarming and reperfusion. Nailfold morphologic features were measured and baseline images and rewarming curves were analyzed. RESULTS: Significant differences were found between groups (analysis of variance) for capillary morphologic features and rewarming curve characteristics. A correlation (P < 0.001) was found between LDI and thermal imaging at baseline (0.667) and maximum (0.729) blood flow and skin temperature, and for the areas under the rewarming curves (0.684). Receiver operating characteristic curves indicated that NC, thermal imaging, and LDI allowed 89%, 74%, and 72%, respectively, of SSc patient data to be correctly classified versus primary RP patients and controls. CONCLUSION: NC, LDI, and thermal imaging each independently provide good discrimination between patients with SSc and those with primary RP and healthy controls (NC being the most suitable technique for classifying patient groups). However, a combination of all 3 techniques improves classification. LDI and thermal imaging give equivalent information on dynamic changes in the cutaneous microcirculation; however, these only weakly correspond to capillary morphology. FAU - Murray, Andrea K AU - Murray AK AD - School of Translational Medicine, University of Manchester, Salford Royal NHS Foundation Trust, Salford, UK. andrea.murray@manchester.ac.uk FAU - Moore, Tonia L AU - Moore TL FAU - Manning, Joanne B AU - Manning JB FAU - Taylor, Christopher AU - Taylor C FAU - Griffiths, Christopher E M AU - Griffiths CE FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Comparative Study PT - Evaluation Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adult MH - Aged MH - Blood Flow Velocity MH - Cold Temperature MH - Female MH - Humans MH - Infrared Rays/therapeutic use MH - Laser-Doppler Flowmetry MH - *Lasers MH - Male MH - Microcirculation/physiology MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/physiopathology MH - Reference Values MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis/physiopathology MH - Skin Temperature MH - Thermography/*methods EDAT- 2009/08/01 09:00 MHDA- 2009/09/11 06:00 CRDT- 2009/08/01 09:00 PHST- 2009/08/01 09:00 [entrez] PHST- 2009/08/01 09:00 [pubmed] PHST- 2009/09/11 06:00 [medline] AID - 10.1002/art.24645 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Aug 15;61(8):1103-11. doi: 10.1002/art.24645. PMID- 18166220 OWN - NLM STAT- MEDLINE DCOM- 20090611 LR - 20220311 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 38 IP - 4 DP - 2009 Feb TI - Feasibility of different capillaroscopic measures for identifying nailfold microvascular alterations. PG - 289-95 LID - 10.1016/j.semarthrit.2007.10.008 [doi] AB - OBJECTIVE: To ascertain the most reliable and relevant capillaroscopic measurements of nailfold videocapillaroscopy (NVC) by analyzing their inter- and intraobserver agreement and predictive value. METHODS: We studied 217 subjects (110 with Raynaud's phenomenon under ongoing evaluation, and 107 with connective tissue diseases) by evaluating the number of capillaries, intercapillary distances, avascular areas, capillary disorganization, capillary loop length, capillary width, percentage of minor abnormalities (tortuous, crossed, or enlarged capillaries), and major abnormalities (giant, bushy, meandering, or branching capillaries), microhemorrhage, skin transparency, and subpapillary plexus visibility. Every finger of both hands was examined. All of the measurements were made by 2 observers under blinded conditions. RESULTS: A total of 877 nailfold images were analyzed. The number of capillaries/mm, interpeak distance, and avascular areas were poorly discriminant, with no statistical differences between their areas under the receiver operating characteristic curve; their reproducibility and repeatability were good, except for the intercapillary distance. Minor abnormalities were observed in 75% of the cases and major abnormalities in 34%; the inter- and intraobserver agreement concerning the major abnormalities was almost perfect. There was very good inter- and intraobserver agreement regarding the analysis of capillary disorganization and hemorrhages. CONCLUSIONS: This study shows that NVC can be useful in quantitatively and reproducibly recording various parameters. We suggest that combining the parameters showing the greatest reliability and prognostic value may be the best means of analyzing NVC images. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Rheumatology, Istituto Gaetano Pini, University of Milan, Milan, Italy. francesca.ingegnoli@unimi.it FAU - Gualtierotti, Roberta AU - Gualtierotti R FAU - Lubatti, Chiara AU - Lubatti C FAU - Zahalkova, Lenka AU - Zahalkova L FAU - Meani, Laura AU - Meani L FAU - Boracchi, Patrizia AU - Boracchi P FAU - Zeni, Silvana AU - Zeni S FAU - Fantini, Flavio AU - Fantini F LA - eng PT - Journal Article DEP - 20071231 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Child MH - Connective Tissue Diseases/*diagnosis MH - Feasibility Studies MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Microvessels/pathology MH - Middle Aged MH - Nails/*blood supply MH - Observer Variation MH - Prognosis MH - Raynaud Disease/*diagnosis MH - Reproducibility of Results MH - Vascular Diseases/*pathology EDAT- 2008/01/02 09:00 MHDA- 2009/06/12 09:00 CRDT- 2008/01/02 09:00 PHST- 2007/07/22 00:00 [received] PHST- 2007/10/16 00:00 [revised] PHST- 2007/10/29 00:00 [accepted] PHST- 2008/01/02 09:00 [pubmed] PHST- 2009/06/12 09:00 [medline] PHST- 2008/01/02 09:00 [entrez] AID - S0049-0172(07)00175-8 [pii] AID - 10.1016/j.semarthrit.2007.10.008 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2009 Feb;38(4):289-95. doi: 10.1016/j.semarthrit.2007.10.008. Epub 2007 Dec 31. PMID- 40410857 OWN - NLM STAT- MEDLINE DCOM- 20250524 LR - 20260127 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 23 IP - 1 DP - 2025 May 23 TI - Nailfold videocapillaroscopy in patients with deficiency of adenosine deaminase 2 (DADA2): a case-control study. PG - 58 LID - 10.1186/s12969-025-01104-4 [doi] LID - 58 AB - BACKGROUND: Deficiency of adenosine deaminase 2 (DADA2) is a rare monogenic autoinflammatory disease mainly characterized by the presence of systemic inflammation and vascular manifestations such as vasculitis and early-onset stroke. Raynaud's phenomenon (RP) can occur in up to 22% of DADA2 patients. The aim of this work was to investigate the microvascular status of DADA2 patients by the mean of nailfold videocapillaroscopy (NVC) comparing them with adequate healthy controls (HC) and primary RP patients. FINDINGS: NVC data of 9 DADA2 patients (mean age 18 ± 6 y) followed at the Children Gaslini Institute were retrospectively retrieved and compared to age and sex cross matched 11 HCs and 7 with primary RP patients. The NVC parameters were classified according to the EULAR SG Fast Track Algorithm and distinguished between scleroderma-pattern (giant capillaries and/or loss of capillaries combined with abnormally shaped capillaries) and non-scleroderma patterns (non-specific NVC alterations). In all DADA2 patients, NVC showed the presence of non-specific alterations (capillaries with dilations in 100% of cases, abnormal shapes in 23% and microhaemorrhages in 11% of patients). The capillary density was normal and no scleroderma pattern was found. Between DADA2, RP patients and HC, no significant differences in the rate of each microvascular finding were detected (p values NS). CONCLUSIONS: This is the first report on NVC in DADA2 patients. Only non-specific abnormalities were found, characterized mainly by capillaries' dilations, but in the absence of giant capillaries. However, larger studies are needed to definitively disclose the microvascular status in DADA2 disease. CI - © 2025. The Author(s). FAU - Bica, Pietro Francesco AU - Bica PF AD - Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy. FAU - Matucci-Cerinic, Caterina AU - Matucci-Cerinic C AUID- ORCID: 0000-0003-3710-0569 AD - Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy. c.matuccicerinic@gmail.com. AD - Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, Genoa, 16147, Italy. c.matuccicerinic@gmail.com. FAU - Hysa, Elvis AU - Hysa E AD - Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy. FAU - Cere, Andrea AU - Cere A AD - Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy. FAU - Rosina, Silvia AU - Rosina S AD - Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, Genoa, 16147, Italy. FAU - Volpi, Stefano AU - Volpi S AD - Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy. AD - Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, Genoa, 16147, Italy. FAU - Caorsi, Roberta AU - Caorsi R AD - Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy. AD - Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, Genoa, 16147, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS San Martino Polyclinic, Genova, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS San Martino Polyclinic, Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - Department of Rheumatology, University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, Flemish Institute for Biotechnology, Inflammation Research Center, Ghent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS San Martino Polyclinic, Genova, Italy. FAU - Gattorno, Marco AU - Gattorno M AD - Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, Genoa, 16147, Italy. LA - eng PT - Journal Article DEP - 20250523 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - EC 3.5.4.4 (Adenosine Deaminase) RN - EC 3.5.4.4 (ADA2 protein, human) RN - 0 (Intercellular Signaling Peptides and Proteins) RN - deficiency of adenosine deaminase 2 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Female MH - Male MH - Adolescent MH - Case-Control Studies MH - *Adenosine Deaminase/deficiency MH - Child MH - Retrospective Studies MH - *Raynaud Disease/diagnosis/etiology MH - *Capillaries/pathology/diagnostic imaging MH - *Nails/blood supply MH - Young Adult MH - Intercellular Signaling Peptides and Proteins MH - Hereditary Autoinflammatory Diseases PMC - PMC12100850 OTO - NOTNLM OT - DADA2 OT - Nailfold capillaroscopy OT - Rare diseases OT - Vasculitis COIS- Declarations. Ethics approval and consent to participate: The local Ethical Committee approval was obtained for this study (392REG2017). Consent for publication: The included patients have given their consent to publication. Competing interests: None declared. EDAT- 2025/05/24 00:46 MHDA- 2025/05/25 00:47 PMCR- 2025/05/23 CRDT- 2025/05/23 23:46 PHST- 2024/10/05 00:00 [received] PHST- 2025/04/27 00:00 [accepted] PHST- 2025/05/25 00:47 [medline] PHST- 2025/05/24 00:46 [pubmed] PHST- 2025/05/23 23:46 [entrez] PHST- 2025/05/23 00:00 [pmc-release] AID - 10.1186/s12969-025-01104-4 [pii] AID - 1104 [pii] AID - 10.1186/s12969-025-01104-4 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2025 May 23;23(1):58. doi: 10.1186/s12969-025-01104-4. PMID- 17937467 OWN - NLM STAT- MEDLINE DCOM- 20080201 LR - 20071106 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 34 IP - 11 DP - 2007 Nov TI - Elevated plasma adrenomedullin and vascular manifestations in patients with systemic sclerosis. PG - 2224-9 AB - OBJECTIVE: Adrenomedullin (ADM), a vasodilating peptide that possesses antiinflammatory properties, may have a regulatory role in the vascular manifestations of scleroderma (systemic sclerosis, SSc). We examined associations between ADM concentrations and vascular manifestations in a cohort of patients with SSc. METHODS: Patients were examined for manifestations of severe Raynaud's phenomenon (RP), defined as digital resorption, previous iloprost infusion, and sympathectomy. Doppler echocardiography and lung function tests were performed to detect elevation in pulmonary arterial pressure (PAP; > 35 mm Hg) and interstitial lung disease (ILD). Plasma ADM was measured by radioimmunoassay. RESULTS: Plasma ADM was measured in 62 SSc patients and 21 healthy controls. Elevated PAP was found in 15 (24.2%) SSc patients (mean PAP 46.5 +/- 11.2 mm Hg, range 37-74). ADM was not found to be related to age, sex, disease duration, or clinical subset. ADM level was significantly higher (median 13.9 pmol/l) in SSc patients with elevated PAP compared to those with lower PAP (median 7.2 pmol/l) (p = 0.01) and controls (median 7.9 pmol/l) (p = 0.04). ADM level was not different among patients who had elevated PAP with (n = 10) and without concomitant ILD (n = 5) (p = 0.21). SSc patients with severe RP (38.7%; median ADM 11.9 pmol/l) were found not to have different ADM levels compared to controls (p = 0.75). Patients who had both severe RP and elevated PAP were found to have significantly higher ADM levels (median 22.3 pmol/l) than patients who had neither manifestation (median 8.0 pmol/l) (p = 0.006) and those with severe RP alone (median 4.2 pmol/l) (p = 0.006). CONCLUSION: Elevated ADM was found in SSc patients with increased PAP regardless of concomitant ILD. FAU - Mok, Mo Yin AU - Mok MY AD - Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong. mymok@netvigator.com FAU - Cheung, Bernard Man Yung AU - Cheung BM FAU - Lo, Yi AU - Lo Y FAU - Leung, Raymond Y H AU - Leung RY FAU - Wong, Woon Sing AU - Wong WS FAU - Lau, Chak Sing AU - Lau CS LA - eng PT - Journal Article DEP - 20071015 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (ADM protein, human) RN - 148498-78-6 (Adrenomedullin) SB - IM MH - Adrenomedullin/*blood MH - Adult MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Female MH - Humans MH - Hypertension, Pulmonary/blood/etiology MH - Lung Diseases, Interstitial/blood/etiology MH - Male MH - Middle Aged MH - Raynaud Disease/blood/etiology MH - Scleroderma, Systemic/*blood/complications/*physiopathology EDAT- 2007/10/17 09:00 MHDA- 2008/02/02 09:00 CRDT- 2007/10/17 09:00 PHST- 2007/10/17 09:00 [pubmed] PHST- 2008/02/02 09:00 [medline] PHST- 2007/10/17 09:00 [entrez] AID - 07/13/1017 [pii] PST - ppublish SO - J Rheumatol. 2007 Nov;34(11):2224-9. Epub 2007 Oct 15. PMID- 31343948 OWN - NLM STAT- MEDLINE DCOM- 20201009 LR - 20201101 IS - 1522-1601 (Electronic) IS - 8750-7587 (Print) IS - 0161-7567 (Linking) VI - 127 IP - 5 DP - 2019 Nov 1 TI - "Beet" the cold: beetroot juice supplementation improves peripheral blood flow, endothelial function, and anti-inflammatory status in individuals with Raynaud's phenomenon. PG - 1478-1490 LID - 10.1152/japplphysiol.00292.2019 [doi] AB - Raynaud's phenomenon (RP) is characterized by recurrent transient peripheral vasospasm and lower nitric oxide (NO) bioavailability in the cold. We investigated the effect of nitrate-rich beetroot juice (BJ) supplementation on 1) NO-mediated vasodilation, 2) cutaneous vascular conductance (CVC) and skin temperature (T(sk)) following local cooling, and 3) systemic anti-inflammatory status. Following baseline testing, 23 individuals with RP attended four times, in a double-blind, randomized crossover design, following acute and chronic (14 days) BJ and nitrate-depleted beetroot juice (NDBJ) supplementation. Peripheral T(sk) and CVC were measured during and after mild hand and foot cooling, and during transdermal delivery of acetylcholine and sodium nitroprusside. Markers of anti-inflammatory status were also measured. Plasma nitrite concentration ([nitrite]) was increased in the BJ conditions (P < 0.001). Compared with the baseline visit, thumb CVC was greater following chronic-BJ (Δ2.0 flux/mmHg, P = 0.02) and chronic-NDBJ (Δ1.45 flux/mmHg, P = 0.01) supplementation; however, no changes in T(sk) were observed (P > 0.05). Plasma [interleukin-10] was greater, pan endothelin and systolic and diastolic blood pressure (BP) were reduced, and forearm endothelial function was improved, by both BJ and NDBJ supplementation (P < 0.05). Acute and chronic BJ and NDBJ supplementation improved anti-inflammatory status, endothelial function and blood pressure (BP). CVC following cooling increased post chronic-BJ and chronic-NDBJ supplementation, but no effect on T(sk) was observed. The key findings are that beetroot supplementation improves thumb blood flow, improves endothelial function and anti-inflammatory status, and reduces BP in people with Raynaud's.NEW & NOTEWORTHY This is the first study to examine the effect of dietary nitrate supplementation in individuals with Raynaud's phenomenon. The principal novel findings from this study were that both beetroot juice and nitrate-depleted beetroot juice 1) increased blood flow in the thumb following a cold challenge; 2) enhanced endothelium-dependent and -independent vasodilation in the forearm; 3) reduced systolic and diastolic blood pressure, and pan-endothelin concentration; and 4) improved inflammatory status in comparison to baseline. FAU - Shepherd, Anthony I AU - Shepherd AI AUID- ORCID: 0000-0001-6392-7944 AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Costello, Joseph T AU - Costello JT AUID- ORCID: 0000-0001-9510-7932 AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Bailey, Stephen J AU - Bailey SJ AD - National Centre for Sport and Exercise Medicine, School of Sport, Exercise and Health Sciences, Loughborough University, Epinal Way, Loughborough, United Kingdom. FAU - Bishop, Nicolette AU - Bishop N AD - National Centre for Sport and Exercise Medicine, School of Sport, Exercise and Health Sciences, Loughborough University, Epinal Way, Loughborough, United Kingdom. AD - University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester, United Kingdom. FAU - Wadley, Alex J AU - Wadley AJ AD - National Centre for Sport and Exercise Medicine, School of Sport, Exercise and Health Sciences, Loughborough University, Epinal Way, Loughborough, United Kingdom. AD - University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester, United Kingdom. FAU - Young-Min, Steven AU - Young-Min S AD - Rheumatology Department, Portsmouth Hospitals NHS Trust, Portsmouth, United Kingdom. FAU - Gilchrist, Mark AU - Gilchrist M AUID- ORCID: 0000-0002-0284-6048 AD - University of Exeter Medical School and NIHR Exeter Clinical Research Facility, Royal Devon and Exeter Hospital, Exeter, Devon, United Kingdom. FAU - Mayes, Harry AU - Mayes H AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - White, Danny AU - White D AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Gorczynski, Paul AU - Gorczynski P AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Saynor, Zoe L AU - Saynor ZL AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Massey, Heather AU - Massey H AUID- ORCID: 0000-0002-7542-513X AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. FAU - Eglin, Clare M AU - Eglin CM AD - School of Sport, Health and Exercise Science, University of Portsmouth, Portsmouth, United Kingdom. LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20190725 PL - United States TA - J Appl Physiol (1985) JT - Journal of applied physiology (Bethesda, Md. : 1985) JID - 8502536 RN - 0 (Anti-Inflammatory Agents) SB - IM MH - Aged MH - Anti-Inflammatory Agents/*administration & dosage MH - *Beta vulgaris MH - Blood Flow Velocity/drug effects/*physiology MH - Cross-Over Studies MH - Dietary Supplements MH - Double-Blind Method MH - Endothelium, Vascular/drug effects/*physiology MH - Female MH - *Fruit and Vegetable Juices MH - Humans MH - Male MH - Microvessels/drug effects/physiology MH - Middle Aged MH - Raynaud Disease/*diet therapy/physiopathology MH - Regional Blood Flow/drug effects/*physiology PMC - PMC6879832 OTO - NOTNLM OT - antioxidants OT - blood flow OT - nitrate COIS- M. Gilchrist received funding from James White Drinks Ltd for development of the placebo. No conflicts of interest, financial or otherwise, are declared by any of the other authors. EDAT- 2019/07/26 06:00 MHDA- 2020/10/10 06:00 PMCR- 2020/11/01 CRDT- 2019/07/26 06:00 PHST- 2019/07/26 06:00 [pubmed] PHST- 2020/10/10 06:00 [medline] PHST- 2019/07/26 06:00 [entrez] PHST- 2020/11/01 00:00 [pmc-release] AID - JAPPL-00292-2019 [pii] AID - 10.1152/japplphysiol.00292.2019 [doi] PST - ppublish SO - J Appl Physiol (1985). 2019 Nov 1;127(5):1478-1490. doi: 10.1152/japplphysiol.00292.2019. Epub 2019 Jul 25. PMID- 27552783 OWN - NLM STAT- MEDLINE DCOM- 20170106 LR - 20200103 IS - 0204-8043 (Print) IS - 0204-8043 (Linking) VI - 58 IP - 2 DP - 2016 Apr-Jun TI - The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology. PG - 77-88 LID - /j/folmed.2016.58.issue-2/folmed-2016-0011/folmed-2016-0011.xml [pii] LID - 10.1515/folmed-2016-0011 [doi] AB - Micro- and macrovascular pathology is a frequent finding in a number of common rheumatic diseases. Secondary Raynaud's phenomenon (RP) is among the most common symptoms in systemic sclerosis and several other systemic autoimmune diseases including a broad differential diagnosis. It should be also differential from other peripheral vascular syndromes such as embolism, thrombosis, etc., some of which lead to clinical manifestation of the blue toe syndrome. The current review discusses the instrumental methods for vascular assessments. Nailfold capillaroscopy is the only method among the imaging techniques that can be used for morphological assessment of the nutritive capillaries in the nailfold area. Laser-Doppler flowmetry and laser-Doppler imaging are methods for functional assessment of microcirculation, while thermography and plethysmography reflect both blood flow in peripheral arteries and microcirculation. Doppler ultrasound and angiography visualize peripheral arteries. The choice of the appropriate instrumental method is guided by the clinical presentation. The main role of capillaroscopy is to provide differential diagnosis between primary and secondary RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been recently defined as diagnostic. The appearance of abnormal capillaroscopic pattern inherits high positive predictive value for the development of a connective tissue disease that is higher than the predictive value of antinuclear antibodies. In cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, unilateral or lower limb involvement, Doppler ultrasound and angiography are indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms. FAU - Lambova, Sevdalina N AU - Lambova SN AD - Department of Propedeutics of Internal Medicine, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria LA - eng PT - Journal Article PT - Review PL - Bulgaria TA - Folia Med (Plovdiv) JT - Folia medica JID - 2984761R SB - IM MH - Angiography MH - Antiphospholipid Syndrome/*diagnostic imaging MH - Blue Toe Syndrome/diagnostic imaging MH - Connective Tissue Diseases/diagnostic imaging MH - Humans MH - Laser-Doppler Flowmetry MH - Microscopic Angioscopy MH - Peripheral Arterial Disease/*diagnostic imaging MH - Peripheral Vascular Diseases/*diagnostic imaging MH - Plethysmography MH - Raynaud Disease/diagnostic imaging MH - Rheumatology MH - Scleroderma, Systemic/*diagnostic imaging MH - Thermography MH - Ultrasonography, Doppler MH - Vasculitis/*diagnostic imaging EDAT- 2016/08/24 06:00 MHDA- 2017/01/07 06:00 CRDT- 2016/08/24 06:00 PHST- 2016/03/09 00:00 [received] PHST- 2016/05/04 00:00 [accepted] PHST- 2016/08/24 06:00 [entrez] PHST- 2016/08/24 06:00 [pubmed] PHST- 2017/01/07 06:00 [medline] AID - /j/folmed.2016.58.issue-2/folmed-2016-0011/folmed-2016-0011.xml [pii] AID - 10.1515/folmed-2016-0011 [doi] PST - ppublish SO - Folia Med (Plovdiv). 2016 Apr-Jun;58(2):77-88. doi: 10.1515/folmed-2016-0011. PMID- 35888663 OWN - NLM STAT- MEDLINE DCOM- 20220728 LR - 20240821 IS - 1648-9144 (Electronic) IS - 1010-660X (Print) IS - 1010-660X (Linking) VI - 58 IP - 7 DP - 2022 Jul 17 TI - Association of Clinical Manifestations of Systemic Lupus Erythematosus and Complementary Therapy Use in Taiwanese Female Patients: A Cross-Sectional Study. LID - 10.3390/medicina58070944 [doi] LID - 944 AB - Background and Objectives: Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects predominantly women in the childbearing years. Patients may seek complementary therapies to manage their health and to reduce symptoms. However, to our knowledge, no studies have explored the association between clinical manifestations of SLE and complementary therapies. Therefore, this study aimed to investigate the association of complementary therapies with common clinical manifestations in Taiwanese female patients with SLE. Materials and Methods: A cross-sectional study was conducted at a regional teaching hospital in southern Taiwan. Outpatients from the rheumatology clinic who met the inclusion criteria were consecutively recruited. Demographic data, clinical manifestations of SLE, and types of complementary therapy use were determined using paper-based questionnaire. Multiple logistic regression analyses were conducted to investigate the use of complementary therapies associated with clinical manifestations of SLE. Results: Of the 317 female patients with SLE, 60.9% were 40 years or older. The five SLE clinical manifestations with the highest prevalence were Raynaud’s phenomenon (61.2%), photosensitivity (50.2%), Sjögren’s syndrome (28.4%), arthralgia and arthritis (22.1%), and renal involvement (14.5%). Multiple logistic regression analyses revealed that Raynaud’s phenomenon was significantly associated with fitness walking or strolling (adjusted odds ratio [aOR] 1.77; p = 0.027) and fish oil supplements (aOR 3.55, p < 0.001). Photosensitivity was significantly and inversely associated with the use of probiotics (aOR 0.49; p = 0.019). Renal involvement was significantly associated with the use of probiotics (aOR 2.43; p = 0.026) and visit to the Chinese medicine department in a hospital (aOR 3.14, p = 0.026). Conclusions: We found that different clinical manifestations of SLE were associated with the use of different complementary therapies. Health care providers should have up-to-date knowledge of common complementary therapies and be ready to provide evidence-based advice to patients with SLE. FAU - Lu, Ming-Chi AU - Lu MC AUID- ORCID: 0000-0001-9051-0351 AD - Division of Allergy, Immunology and Rheumatology, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin, Chiayi 622401, Taiwan. AD - School of Medicine, Tzu Chi University, Hualien City 97004, Taiwan. FAU - Hsu, Chia-Wen AU - Hsu CW AD - Department of Medical Research, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin, Chiayi 622401, Taiwan. FAU - Lo, Hui-Chin AU - Lo HC AD - Department of Medical Research, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin, Chiayi 622401, Taiwan. FAU - Chang, Hsiu-Hua AU - Chang HH AD - Department of Medical Research, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin, Chiayi 622401, Taiwan. FAU - Koo, Malcolm AU - Koo M AUID- ORCID: 0000-0002-9242-9167 AD - Graduate Institute of Long-Term Care, Tzu Chi University of Science and Technology, Hualien City 970302, Taiwan. AD - Dalla Lana School of Public Health, University of Toronto, Toronto, ON M5T 3M7, Canada. LA - eng GR - TCMF-A 108-05/Buddhist Tzu Chi Medical Foundation/ GR - No: DTCRD109-I-21, DTCRD109-I-23/Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation/ PT - Journal Article DEP - 20220717 PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 SB - IM MH - *Complementary Therapies MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/complications/epidemiology/therapy MH - *Raynaud Disease/complications MH - *Sjogren's Syndrome/complications PMC - PMC9317495 OTO - NOTNLM OT - Raynaud’s phenomenon OT - clinical manifestations OT - complementary medicine OT - probiotics OT - systemic lupus erythematosus COIS- The authors declare no conflict of interest. EDAT- 2022/07/28 06:00 MHDA- 2022/07/29 06:00 PMCR- 2022/07/17 CRDT- 2022/07/27 01:31 PHST- 2022/06/07 00:00 [received] PHST- 2022/07/05 00:00 [revised] PHST- 2022/07/13 00:00 [accepted] PHST- 2022/07/27 01:31 [entrez] PHST- 2022/07/28 06:00 [pubmed] PHST- 2022/07/29 06:00 [medline] PHST- 2022/07/17 00:00 [pmc-release] AID - medicina58070944 [pii] AID - medicina-58-00944 [pii] AID - 10.3390/medicina58070944 [doi] PST - epublish SO - Medicina (Kaunas). 2022 Jul 17;58(7):944. doi: 10.3390/medicina58070944. PMID- 29993255 OWN - NLM STAT- MEDLINE DCOM- 20200518 LR - 20210109 IS - 1557-7708 (Electronic) IS - 1075-5535 (Print) IS - 1075-5535 (Linking) VI - 24 IP - 12 DP - 2018 Dec TI - Herbal Medicines for Cold Hypersensitivity in the Hands and Feet: A Systematic Review and Meta-Analysis. PG - 1150-1158 LID - 10.1089/acm.2018.0009 [doi] AB - Objectives: Cold hypersensitivity in the hands and feet (CHHF) and Raynaud's phenomenon (RP) are prevalent among Asian populations, especially among women, who exhibit a higher rate of cold hypersensitivity that may be associated with gynecological problems. In several countries, herbal medicine has effectively treated cold hypersensitivity symptoms. This systematic review and meta-analysis of the literature was undertaken to evaluate the efficacy of herbal medicine for the treatment of CHHF in adults. Design: Through March 31, 2018, comprehensive databases were searched, including MEDLINE, EMBASE, Cochrane Library, Chinese Academic Journal, and Japanese National Institute of Informatics, to identify relevant studies and extract data. Outcome measures: Primary: total effective rate (TER); secondary: skin temperature, peripheral blood flow, adverse events. Results: Fourteen randomized controlled trials (n = 974) were included. Thirteen studies with dichotomous values showed a significant reduction in CHHF and RP (risk ratio 0.31, 0.24-0.40) when comparing herbal medicine with/without Western medicine, and no treatment or Western medicine alone. Reductions in CHHF and RP were also observed between herbal medicine plus Western medicine and Western medicine alone (risk ratio 0.45, 0.24-0.86), as well as between herbal medicine and Western medicine alone (risk ratio 0.30, 0.21-0.41). In the only study using a placebo arm, herbal medicine was found to be superior to placebo in increasing skin temperature and peripheral blood flow. Six participants exhibited minor adverse drug reactions. Herbal medicine showed a superior TER, especially when combined with Western medicine, to Western medicine alone or placebo. However, there was a high risk of bias within all studies. Conclusion: Although herbal medicine shows potential to be a safe and effective treatment for CHHF and RP, the high risk of bias in all studies prevents definitive conclusions; thus, higher quality studies must be performed. FAU - Yu, Jun-Sang AU - Yu JS AD - Department of Sasang Constitutional Medicine, College of Korean Medicine, Sangji University, Wonju, Republic of Korea. FAU - Lee, Dongnyung AU - Lee D AD - Department of Gynecology Medicine, College of Korean Medicine, Semyung University, Chungju, Republic of Korea. FAU - Hyun, Daesung AU - Hyun D AD - Department of Preventive Medicine, The Graduate School of Yonsei University, Wonju, Republic of Korea. AD - Department of Biostatistics and Computing, The Graduate School of Yonsei University, Wonju, Republic of Korea. FAU - Chang, Sei-Jin AU - Chang SJ AD - Department of Preventive Medicine, Wonju College of Medicine, Yonsei University, Wonju, Republic of Korea. AD - Institute of Environmental & Occupational Medicine, Wonju College of Medicine, Yonsei University, Wonju, Republic of Korea. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20180711 PL - United States TA - J Altern Complement Med JT - Journal of alternative and complementary medicine (New York, N.Y.) JID - 9508124 RN - 0 (Drugs, Chinese Herbal) RN - Cold Hypersensitivity SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Cryopyrin-Associated Periodic Syndromes/*drug therapy MH - Drugs, Chinese Herbal/*therapeutic use MH - Female MH - Foot/*physiopathology MH - Hand/*physiopathology MH - Herbal Medicine/*methods MH - Humans MH - Male MH - Middle Aged MH - Phytotherapy/*methods MH - Plants, Medicinal/chemistry MH - Raynaud Disease/*drug therapy MH - Treatment Outcome PMC - PMC6308292 OTO - NOTNLM OT - Raynaud's phenomenon OT - cold hypersensitivity OT - coldness OT - herbal medicine OT - meta-analysis OT - systematic review COIS- No competing financial interests exist. EDAT- 2018/07/12 06:00 MHDA- 2020/05/19 06:00 PMCR- 2018/12/14 CRDT- 2018/07/12 06:00 PHST- 2018/07/12 06:00 [pubmed] PHST- 2020/05/19 06:00 [medline] PHST- 2018/07/12 06:00 [entrez] PHST- 2018/12/14 00:00 [pmc-release] AID - 10.1089/acm.2018.0009 [pii] AID - 10.1089/acm.2018.0009 [doi] PST - ppublish SO - J Altern Complement Med. 2018 Dec;24(12):1150-1158. doi: 10.1089/acm.2018.0009. Epub 2018 Jul 11. PMID- 35835174 OWN - NLM STAT- MEDLINE DCOM- 20220913 LR - 20250728 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 144 DP - 2022 Nov TI - Sudden winter iloprost withdrawal in scleroderma patients during COVID-19 pandemic. PG - 104404 LID - S0026-2862(22)00094-2 [pii] LID - 10.1016/j.mvr.2022.104404 [doi] AB - INTRODUCTION: Intravenous iloprost is currently recommended in the treatment of Raynaud's phenomenon (RP) refractory to oral therapy and of digital ulcers (DUs) related to systemic sclerosis (SSc). In real-life practice there is a huge heterogeneity about the Iloprost regimens used. METHODS: A survey was carried out on SSc patients that interrupted Iloprost infusion to compare acral vascular symptoms just before Iloprost withdrawal and just after the missed infusion. Severity, and frequency of RP, new DUs onset or aggravation of those pre-existing were reported. Last available capillaroscopic images were also evaluated. RESULTS: The analysis includes 50 patients. After iloprost withdrawal, 11 patients reported a RP worsening because of enhanced intensity (p = 0.007). Only 8 patients of them also complained of an increased frequency (p = 0.07). None of the patients experienced digital ulcers for the first-time during quarantine. Among the 27 patients with a history of digital ulcers, 9 reported worsening and 7 recurrence of DUs. Overall, 17 patients (34.0 %) complained of a worsening of SSc vascular acral manifestations, namely RP or DUs. Reduced capillary density was associated with RP worsening, in particular, each unit increase of capillary density corresponds to an average 44 % decrease in the odds of RP worsening (OR 0.56, CI 95 % 0.36-0.97, p = 0.037). As for RP worsening, the aggravation of DU was associated with a lower capillary density. CONCLUSIONS: Low capillary density can predict a worsening of both RP and DUs in controlled quarantine conditions within a month after iloprost discontinuation in SSc patients. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - De Lorenzis, Enrico AU - De Lorenzis E AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - Natalello, Gerlando AU - Natalello G AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - Verardi, Lucrezia AU - Verardi L AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - Cerasuolo, Pier Giacomo AU - Cerasuolo PG AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - Gigante, Laura AU - Gigante L AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - D'Agostino, Maria Antonietta AU - D'Agostino MA AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Rome, Italy. Electronic address: silvialaura.bosello@policlinicogemelli.it. LA - eng PT - Journal Article DEP - 20220711 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - JED5K35YGL (Iloprost) SB - IM MH - *COVID-19 MH - Humans MH - Iloprost/adverse effects MH - Pandemics MH - *Raynaud Disease/diagnosis/drug therapy MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy MH - *Skin Ulcer/diagnosis/drug therapy MH - Ulcer/complications PMC - PMC9271454 OTO - NOTNLM OT - COVID-19 OT - Iloprost OT - Nailfold capillaroscopy OT - Systemic sclerosis COIS- Declaration of competing interest There are no competing interests for any author. All the authors gave substantial contributions to the conception or design of the work, acquisition, analysis or interpretation of data, drafting the work or revising it critically for important intellectual content and final approval of the version published. EDAT- 2022/07/15 06:00 MHDA- 2022/09/14 06:00 PMCR- 2022/07/11 CRDT- 2022/07/14 19:32 PHST- 2022/03/02 00:00 [received] PHST- 2022/06/27 00:00 [revised] PHST- 2022/07/05 00:00 [accepted] PHST- 2022/07/15 06:00 [pubmed] PHST- 2022/09/14 06:00 [medline] PHST- 2022/07/14 19:32 [entrez] PHST- 2022/07/11 00:00 [pmc-release] AID - S0026-2862(22)00094-2 [pii] AID - 104404 [pii] AID - 10.1016/j.mvr.2022.104404 [doi] PST - ppublish SO - Microvasc Res. 2022 Nov;144:104404. doi: 10.1016/j.mvr.2022.104404. Epub 2022 Jul 11. PMID- 28147261 OWN - NLM STAT- MEDLINE DCOM- 20170426 LR - 20220408 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 16 IP - 3 DP - 2017 Mar TI - Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: Results from a multicenter, international and retrospective study. PG - 253-257 LID - S1568-9972(17)30020-4 [pii] LID - 10.1016/j.autrev.2017.01.008 [doi] AB - OBJECTIVE: Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. METHODS: Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. RESULTS: 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002). CONCLUSION: Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings. CI - Copyright © 2017 Elsevier B.V. All rights reserved. FAU - Bartoloni, Elena AU - Bartoloni E AD - Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. Electronic address: elena.bartolonibocci@unipg.it. FAU - Gonzalez-Gay, Miguel A AU - Gonzalez-Gay MA AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - Scirè, Carlo AU - Scirè C AD - Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy. FAU - Castaneda, Santos AU - Castaneda S AD - Rheumatology Department, Hospital Universitario de la Princesa, IIS-IP, Madrid, Spain. FAU - Gerli, Roberto AU - Gerli R AD - Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. FAU - Lopez-Longo, Francisco Javier AU - Lopez-Longo FJ AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Martinez-Barrio, Julia AU - Martinez-Barrio J AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Govoni, Marcello AU - Govoni M AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Furini, Federica AU - Furini F AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Pina, Trinitario AU - Pina T AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - Iannone, Florenzo AU - Iannone F AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. FAU - Giannini, Margherita AU - Giannini M AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. FAU - Nuño, Laura AU - Nuño L AD - Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. FAU - Quartuccio, Luca AU - Quartuccio L AD - Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, Udine, Italy. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. FAU - Alunno, Alessia AU - Alunno A AD - Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. FAU - Specker, Christopher AU - Specker C AD - Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - Triantafyllias, Konstantinos AU - Triantafyllias K AD - ACURA Rheumatology Center, Bad Kreuznach, Germany. FAU - Balduzzi, Silvia AU - Balduzzi S AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - Sifuentes-Giraldo, Walter Alberto AU - Sifuentes-Giraldo WA AD - Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. FAU - Paolazzi, Giuseppe AU - Paolazzi G AD - Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. FAU - Bravi, Elena AU - Bravi E AD - Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. FAU - Schwarting, Andreas AU - Schwarting A AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. FAU - Pellerito, Raffaele AU - Pellerito R AD - Division of Rheumatology, Mauriziano Hospital, Turin, Italy. FAU - Russo, Alessandra AU - Russo A AD - Division of Rheumatology, Mauriziano Hospital, Turin, Italy. FAU - Selmi, Carlo AU - Selmi C AD - Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy. FAU - Saketkoo, Lesley-Ann AU - Saketkoo LA AD - Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center New Orleans, New Orleans, LA, USA. FAU - Fusaro, Enrico AU - Fusaro E AD - Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. FAU - Parisi, Simone AU - Parisi S AD - Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. FAU - Pipitone, Nicolò AU - Pipitone N AD - Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera ASMN, Reggio Emilia, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Neri, Rossella AU - Neri R AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. LA - eng PT - Journal Article PT - Multicenter Study PT - Review DEP - 20170129 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - EC 6.- (Ligases) SB - IM MH - Autoantibodies MH - Female MH - Follow-Up Studies MH - Humans MH - Ligases/*antagonists & inhibitors MH - Male MH - Middle Aged MH - Raynaud Disease/*metabolism MH - Retrospective Studies MH - Syndrome OTO - NOTNLM OT - Anti-synthetase syndrome OT - Interstitial lung disease OT - Myositis OT - Prognosis OT - Raynaud's phenomenon OT - mechanic's hand EDAT- 2017/02/02 06:00 MHDA- 2017/04/27 06:00 CRDT- 2017/02/02 06:00 PHST- 2016/11/01 00:00 [received] PHST- 2016/11/16 00:00 [accepted] PHST- 2017/02/02 06:00 [pubmed] PHST- 2017/04/27 06:00 [medline] PHST- 2017/02/02 06:00 [entrez] AID - S1568-9972(17)30020-4 [pii] AID - 10.1016/j.autrev.2017.01.008 [doi] PST - ppublish SO - Autoimmun Rev. 2017 Mar;16(3):253-257. doi: 10.1016/j.autrev.2017.01.008. Epub 2017 Jan 29. PMID- 40543246 OWN - NLM STAT- MEDLINE DCOM- 20250918 LR - 20260602 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 74 DP - 2025 Oct TI - Comparing Raynaud's phenomenon attack measurement tools: paper or smartphone application? PG - 152765 LID - S0049-0172(25)00136-2 [pii] LID - 10.1016/j.semarthrit.2025.152765 [doi] AB - OBJECTIVE: To compare two methods of recording Raynaud's Phenomenon (RP) attack frequency, duration and severity: the traditional Raynaud Condition Score (RCS) paper diary and a new smartphone application. METHODS: We conducted a multicenter study of patients with stable SSc-RP. Participants were randomized to document their RP attacks in the RCS paper diary or smartphone application for one week, at which point they were again randomized to either continue the original recording method or cross over to the other recording method for an additional week. Participants who crossed over completed a questionnaire about their experience with each method. We compared patient preference, and RP documentation by recording method. RESULTS: Fifty-five patients with stable SSc-RP were included. The 24 participants who used both modalities were significantly more likely to report "liking" the smartphone application than the paper diary (92% vs. 58%, p=0.04). There was also a non-significant difference in real-time documentation with the smartphone than the paper diary (71% vs. 38%, p=0.06). Participants reported significantly more attacks with the paper diary than with the smartphone application, however, the severity and average attack length were not significantly different. CONCLUSION: This study supports the use of a smartphone application to document RP attack frequency, duration and severity. Not only did patients prefer the smartphone application to the paper diary, but they were also more likely to record RP attacks in real-time with the smartphone application, reducing the risk of recall bias. Future clinical trials should consider using a smartphone-based application to capture RP attacks. CI - Copyright © 2025. Published by Elsevier Inc. FAU - Wallwork, Rachel S AU - Wallwork RS AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Hu, Haomin AU - Hu H AD - School of Informatics, University of Pittsburgh. Pittsburgh, PA, USA. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Hummers, Laura AU - Hummers L AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath, Combe Park, Bath, UK; Department of Life Sciences, University of Bath, Bath, UK. FAU - Flower, Victoria AU - Flower V AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath, Combe Park, Bath, UK; Department of Life Sciences, University of Bath, Bath, UK. FAU - Parmanto, Bambang AU - Parmanto B AD - School of Informatics, University of Pittsburgh. Pittsburgh, PA, USA. FAU - Saptono, Andi AU - Saptono A AD - School of Informatics, University of Pittsburgh. Pittsburgh, PA, USA. FAU - Domsic, Robyn T AU - Domsic RT AD - Department of Medicine/Rheumatology, University of Pittsburgh School of Medicine. Pittsburgh, PA, USA. Electronic address: rtd4@pitt.edu. LA - eng GR - K24 AR080217/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial DEP - 20250606 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis MH - *Smartphone MH - Male MH - Female MH - Middle Aged MH - *Mobile Applications MH - Severity of Illness Index MH - Adult MH - Aged MH - Surveys and Questionnaires MH - Patient Preference MH - Paper OTO - NOTNLM OT - Outcome measure OT - Raynaud phenomenon OT - Smart phone application or app (journal preference for term) OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Robyn Domsic reports financial support was provided by Department of Defense. Ami Shah reports financial support was provided by National Institute of Arthritis and Musculoskeletal and Skin Diseases. Ami Shah reports financial support was provided by Donald B. and Dorothy L. Stabler Foundation. Rachel Wallwork reports financial support was provided by Jerome L Greene Foundation. Rachel Wallwork reports financial support was provided by Rheumatology Research Foundation. Robyn Domsic reports a relationship with Aisa pharmaceuticals that includes: consulting or advisory. Robyn Domsic reports a relationship with AstraZeneca that includes: consulting or advisory. Rachel Wallwork reports a relationship with Amgen Inc that includes: consulting or advisory. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/06/22 06:11 MHDA- 2025/09/19 00:36 CRDT- 2025/06/21 18:05 PHST- 2025/01/08 00:00 [received] PHST- 2025/05/21 00:00 [revised] PHST- 2025/05/27 00:00 [accepted] PHST- 2025/09/19 00:36 [medline] PHST- 2025/06/22 06:11 [pubmed] PHST- 2025/06/21 18:05 [entrez] AID - S0049-0172(25)00136-2 [pii] AID - 10.1016/j.semarthrit.2025.152765 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2025 Oct;74:152765. doi: 10.1016/j.semarthrit.2025.152765. Epub 2025 Jun 6. PMID- 19332631 OWN - NLM STAT- MEDLINE DCOM- 20090619 LR - 20230502 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 5 DP - 2009 May TI - Correlation of biomarkers of endothelium dysfunction and matrix remodeling in patients with systemic sclerosis. PG - 984-8 LID - 10.3899/jrheum.080924 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) is a multisystem disease characterized by microvascular dysfunction and excessive fibrosis. However, the relationship between these 2 features remains unclear. Endothelial dysfunction can be assessed by quantifying plasma asymmetric dimethylarginine (ADMA), an endogenous inhibitor of endothelial nitric oxide synthase. Matrix remodeling can be assessed by quantifying serum tissue inhibitor of matrix metalloproteinases-1 (TIMP-1). Both biomarkers are elevated in patients with SSc. Our objective was to test whether plasma ADMA is correlated with serum TIMP-1. METHODS: We enrolled 91 subjects, 39 patients with SSc, 28 patients with primary Raynaud's phenomenon (RP), and 24 healthy volunteers. Plasma ADMA concentrations were measured by liquid chromatography-tandem mass spectrometry. Serum TIMP-1 concentrations were determined by ELISA. RESULTS: Mean ADMA concentrations were higher in patients with SSc (0.68 microM +/- 0.12) than in patients with primary RP or healthy volunteers (respectively, 0.56 microM +/- 0.14 and 0.62 microM +/- 0.12; p = 0.002). Median serum TIMP-1 concentrations were increased in patients with SSc compared to primary RP and healthy volunteers [12 (9-15), 11 (8-13), and 10 (7-13) nM, respectively; p = 0.05]. In the SSc group, we observed a statistically significant correlation between plasma ADMA and serum TIMP-1 (r = 0.34, p = 0.035). CONCLUSION: These data are consistent with our hypothesis of an association of endothelial dysfunction and matrix remodeling in scleroderma spectrum disorders. FAU - Blaise, Sophie AU - Blaise S AD - Vascular MedicineDepartment, INSERM Clinical Research Center, Grenoble University Hospital, Grenoble, France. FAU - Maas, Renke AU - Maas R FAU - Trocme, Candice AU - Trocme C FAU - Kom, Ghainsom D AU - Kom GD FAU - Roustit, Matthieu AU - Roustit M FAU - Carpentier, Patrick H AU - Carpentier PH FAU - Cracowski, Jean-Luc AU - Cracowski JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090330 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Biomarkers) RN - 0 (Enzyme Inhibitors) RN - 0 (Tissue Inhibitor of Metalloproteinase-1) RN - 63CV1GEK3Y (N,N-dimethylarginine) RN - 94ZLA3W45F (Arginine) RN - EC 1.14.13.39 (Nitric Oxide Synthase) SB - IM MH - Arginine/*analogs & derivatives/metabolism MH - Biomarkers/metabolism MH - Endothelium, Vascular/*metabolism/physiopathology MH - Enzyme Inhibitors/*metabolism MH - Extracellular Matrix/*metabolism MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nitric Oxide Synthase/antagonists & inhibitors MH - Raynaud Disease/metabolism/physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/*metabolism/physiopathology MH - Skin/blood supply/metabolism/physiopathology MH - Tissue Inhibitor of Metalloproteinase-1/*metabolism EDAT- 2009/04/01 09:00 MHDA- 2009/06/20 09:00 CRDT- 2009/04/01 09:00 PHST- 2009/04/01 09:00 [entrez] PHST- 2009/04/01 09:00 [pubmed] PHST- 2009/06/20 09:00 [medline] AID - jrheum.080924 [pii] AID - 10.3899/jrheum.080924 [doi] PST - ppublish SO - J Rheumatol. 2009 May;36(5):984-8. doi: 10.3899/jrheum.080924. Epub 2009 Mar 30. PMID- 22289955 OWN - NLM STAT- MEDLINE DCOM- 20120515 LR - 20191112 IS - 2233-6869 (Electronic) IS - 1598-9992 (Linking) VI - 59 IP - 1 DP - 2012 Jan TI - Liver cirrhosis due to autoimmune hepatitis combined with systemic sclerosis. PG - 48-52 AB - Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management. FAU - You, Byung Chul AU - You BC AD - Department of Internal Medicine, Soon Chun Hyang University College of Medicine, Seoul, Korea. FAU - Jeong, Soung Won AU - Jeong SW FAU - Jang, Jae Young AU - Jang JY FAU - Goo, So Mi AU - Goo SM FAU - Kim, Sang Gyune AU - Kim SG FAU - Kim, Young Seok AU - Kim YS FAU - Jeon, Chan Hong AU - Jeon CH FAU - Jeen, Yoon Mi AU - Jeen YM LA - eng PT - Case Reports PT - Journal Article PL - Korea (South) TA - Korean J Gastroenterol JT - The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi JID - 101189416 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Anti-Inflammatory Agents/therapeutic use MH - Antibodies, Antinuclear/blood MH - Esophageal and Gastric Varices MH - Female MH - Gastrointestinal Hemorrhage MH - Hepatitis, Autoimmune/complications/*diagnosis/drug therapy MH - Humans MH - Liver Cirrhosis/*diagnosis/etiology/pathology MH - Middle Aged MH - Prednisolone/therapeutic use MH - Raynaud Disease/diagnosis MH - Scleroderma, Systemic/complications/*diagnosis MH - Skin/pathology EDAT- 2012/02/01 06:00 MHDA- 2012/05/16 06:00 CRDT- 2012/02/01 06:00 PHST- 2012/02/01 06:00 [entrez] PHST- 2012/02/01 06:00 [pubmed] PHST- 2012/05/16 06:00 [medline] AID - 201201188 [pii] AID - 10.4166/kjg.2012.59.1.48 [doi] PST - ppublish SO - Korean J Gastroenterol. 2012 Jan;59(1):48-52. doi: 10.4166/kjg.2012.59.1.48. PMID- 32371783 OWN - NLM STAT- MEDLINE DCOM- 20210416 LR - 20210416 IS - 1524-4725 (Electronic) IS - 1076-0512 (Linking) VI - 47 IP - 1 DP - 2021 Jan 1 TI - Botulinum Toxin A Treatment for Primary and Secondary Raynaud's Phenomenon in Teenagers. PG - 61-64 LID - 10.1097/DSS.0000000000002397 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) is a clinical syndrome characterized by recurrent episodes of digital vasospasm triggered by exposure to physical and chemical agents or emotional stress. Although many pharmacologic treatments have been tested, there is still no cure or gold standard therapy. Botulinum toxin treatment has been proved to reduce pain and increase arterial blood flow in treated hands of adult patients with RP. OBJECTIVE: The aim of this study is to evaluate the efficacy of botulinum toxin A on younger than 18-year-old patients with primary and secondary RP. MATERIALS AND METHODS: A single-center prospective study was performed, including 8 patients aged 14 to 17 years who were clinically diagnosed with primary or secondary RP. BTX was injected into each hand without sedation or anesthetic blockade. The primary outcome was pain reduction after BTX injection. Pain intensity was evaluated at baseline and in the first follow-up. Secondary outcomes included variations in the number and severity of RP episodes after the BTX injection. RESULTS: All patients stated significant pain reduction and decreased cold sensitivity, except one patient who did not feel any changes. No patients reported any loss of strength on thumb-index finger. CONCLUSION: BTX injection is a simple, noninvasive, and cost-effective treatment alternative, offering an important nonsurgical therapeutic option for the pediatric population. It could also help optimize the dose of other treatments used in these patients. The most commonly observed positive effect is a reduction in the pain associated with RP attacks. Further studies are needed to confirm these results. CI - Copyright © 2020 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved. FAU - Quintana Castanedo, Lucía AU - Quintana Castanedo L AD - All authors are affiliated with the Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - Feito Rodríguez, Marta AU - Feito Rodríguez M FAU - Nieto Rodríguez, Daniel AU - Nieto Rodríguez D FAU - Maseda Pedrero, Rocío AU - Maseda Pedrero R FAU - Chiloeches Fernández, Clara AU - Chiloeches Fernández C FAU - de Lucas Laguna, Raúl AU - de Lucas Laguna R LA - eng PT - Journal Article PL - United States TA - Dermatol Surg JT - Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] JID - 9504371 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adolescent MH - Botulinum Toxins, Type A/administration & dosage/*therapeutic use MH - Female MH - Humans MH - Injections MH - Male MH - Neuromuscular Agents/administration & dosage/*therapeutic use MH - Pain Management MH - Pain Measurement MH - Prospective Studies MH - Raynaud Disease/*drug therapy EDAT- 2020/05/07 06:00 MHDA- 2021/04/17 06:00 CRDT- 2020/05/07 06:00 PHST- 2020/05/07 06:00 [pubmed] PHST- 2021/04/17 06:00 [medline] PHST- 2020/05/07 06:00 [entrez] AID - 00042728-202101000-00017 [pii] AID - 10.1097/DSS.0000000000002397 [doi] PST - ppublish SO - Dermatol Surg. 2021 Jan 1;47(1):61-64. doi: 10.1097/DSS.0000000000002397. PMID- 23934522 OWN - NLM STAT- MEDLINE DCOM- 20140930 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 12 DP - 2013 Dec TI - The value of pattern capillary changes and antibodies to predict the development of systemic sclerosis in patients with primary Raynaud's phenomenon. PG - 2967-73 LID - 10.1007/s00296-013-2844-7 [doi] AB - The aim of this study is to assess the prognostic value of major provisional criteria for the development of systemic sclerosis (SSc) in primary Raynaud's phenomenon (RP) patients. We retrospectively studied the chart of 497 patients with primary RP in whom anticentromere (ACA) and antitopoisomerase I (ATA) antibodies tests and a capillary reading were available. Sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratios (LHR+), negative likelihood ratios (LHR-), odds ratio (OR), and area under the receiver operating characteristics curve (AUC) of those criteria were assessed to predict the development of SSc. During the average follow-up of 2.3 ± 1.9 years, 159 (32 %) patients evolved to SSc, 245 (49.3 %) evolved to other connective tissue diseases, and 93 (18.7 %) patients did not progress. The SSc pattern predicted SSc satisfactorily (LHR+ 4.12, LHR- 0.07, OR 63, AUC 0.819; P < 0.001). ACA were not significantly associated with the development of SSc (LHR+ 1.19, LHR- 0.9, OR 1.32, AUC 0.538, P = 0.156). ATA were significantly associated with the development of SSc (LHR+ 9.32, LHR- 0.67, OR 15.13, AUC 0.777; P < 0.001). Both SSc pattern and ACA or ATA were significantly associated with the development of SSc (LHR+ 2.98, LHR- 0.70, OR 4.2, AUC 0.674; P < 0.001 vs. LHR+ 16, LHR- 0.68, OR 24, AUC 0.819; P < 0.001, respectively). SSc pattern or ATA as independent risk factors, as well as following two parameters together (SSc pattern and ATA or SSc pattern and ACA) were good predictors for the development of SSc. FAU - Pavlov-Dolijanovic, Slavica R AU - Pavlov-Dolijanovic SR AD - Institute of Rheumatology Belgrade, Resavska 69, 11000, Belgrade, Serbia, dolijan@eunet.rs. FAU - Damjanov, Nemanja S AU - Damjanov NS FAU - Vujasinovic Stupar, Nada Z AU - Vujasinovic Stupar NZ FAU - Baltic, Snezana AU - Baltic S FAU - Babic, Dragan D AU - Babic DD LA - eng PT - Comparative Study PT - Journal Article DEP - 20130811 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies) RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adult MH - Antibodies/*blood MH - Antibodies, Antinuclear/*blood MH - Capillaries/*pathology MH - DNA Topoisomerases, Type I/*immunology MH - Humans MH - Microscopic Angioscopy MH - Middle Aged MH - Odds Ratio MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/*complications MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*diagnosis/*etiology/immunology MH - Sensitivity and Specificity EDAT- 2013/08/13 06:00 MHDA- 2014/10/01 06:00 CRDT- 2013/08/13 06:00 PHST- 2012/10/19 00:00 [received] PHST- 2013/07/30 00:00 [accepted] PHST- 2013/08/13 06:00 [entrez] PHST- 2013/08/13 06:00 [pubmed] PHST- 2014/10/01 06:00 [medline] AID - 10.1007/s00296-013-2844-7 [doi] PST - ppublish SO - Rheumatol Int. 2013 Dec;33(12):2967-73. doi: 10.1007/s00296-013-2844-7. Epub 2013 Aug 11. PMID- 18240233 OWN - NLM STAT- MEDLINE DCOM- 20080407 LR - 20131121 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 58 IP - 2 DP - 2008 Feb TI - Association between treatment with central nervous system stimulants and Raynaud's syndrome in children: a retrospective case-control study of rheumatology patients. PG - 563-6 LID - 10.1002/art.23301 [doi] AB - OBJECTIVE: To investigate whether central nervous system (CNS) stimulants used for the treatment of attention deficit hyperactivity disorder (ADHD) are associated with the development of Raynaud's syndrome (RS). METHODS: A case-control design was used for this study. All patients seen in a pediatric rheumatology practice during a 5-year period who had signs and symptoms of RS and met diagnostic criteria for RS as determined by pulse volume recording were studied as cases. Controls were randomly selected patients at the same clinic who did not have signs or symptoms of RS. They were matched to the cases for age, sex, and time of presentation. We tested for associations between various CNS medications and presence of RS. We also evaluated differences in laboratory test results between RS cases and matched controls. RESULTS: Sixty-four patients were enrolled in the study (32 cases with RS [23 female, 9 male] and 32 control patients). McNemar's test showed a significant association between the presence of RS and past or current use of ADHD stimulants (methylphenidate and dextroamphetamine) (chi(2) = 5.00, P = 0.01). The use of other CNS medications was not found to be significantly associated with RS (chi(2) = 1.33, P = 0.25 by McNemar's test). C-reactive protein levels and erythrocyte sedimentation rates were significantly higher in controls than in cases. CONCLUSION: The results of this study indicate that there is a significant association between development of RS and therapy with CNS stimulants used for the treatment of ADHD. Although this was a small study, these findings provide preliminary evidence of an adverse effect of CNS stimulant medications in patients seen in rheumatology practice. FAU - Goldman, William AU - Goldman W AD - Akron Children's Hospital and Medical Center, Akron, Ohio 44308, USA. wgoldman@chmca.org FAU - Seltzer, Ryan AU - Seltzer R FAU - Reuman, Peter AU - Reuman P LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) RN - TZ47U051FI (Dextroamphetamine) SB - IM MH - Adolescent MH - Adult MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Case-Control Studies MH - Central Nervous System Stimulants/*adverse effects MH - Child MH - Dextroamphetamine/*adverse effects MH - Female MH - Humans MH - Male MH - Methylphenidate/*adverse effects MH - Raynaud Disease/*chemically induced MH - Retrospective Studies MH - Rheumatic Diseases/chemically induced EDAT- 2008/02/02 09:00 MHDA- 2008/04/09 09:00 CRDT- 2008/02/02 09:00 PHST- 2008/02/02 09:00 [pubmed] PHST- 2008/04/09 09:00 [medline] PHST- 2008/02/02 09:00 [entrez] AID - 10.1002/art.23301 [doi] PST - ppublish SO - Arthritis Rheum. 2008 Feb;58(2):563-6. doi: 10.1002/art.23301. PMID- 39586592 OWN - NLM STAT- MEDLINE DCOM- 20250108 LR - 20250109 IS - 1442-2050 (Electronic) IS - 1120-8694 (Linking) VI - 38 IP - 1 DP - 2025 Jan 7 TI - High prevalence of esophageal motility disorders in patients with rheumatologic diseases. LID - doae108 [pii] LID - 10.1093/dote/doae108 [doi] AB - While it is commonly known that patients with rheumatologic diseases can have esophageal dysfunction, this association is insufficiently understood. The aim is to determine the prevalence and characteristics of esophageal motility disorders in patients with rheumatic diseases. This is a single-center retrospective study of adults with rheumatologic disease who underwent high-resolution esophageal manometry (HREM). Those with and without a motility disorder (defined per Chicago classification CCv3.0 criteria, given the timing of the prior studies) were compared and multivariable logistic regression was used to determine odds of motility disorder by rheumatic disease. Of 289 patients, the mean age was 60.5 ± 13.8 years. Rheumatic diseases included Raynaud's (42%), rheumatoid arthritis (RA) (39%), Sjogren's (21%), systemic lupus erythematous (19%), systemic sclerosis (17%), and mixed connective tissue disease (13%). On HREM, 58% had an esophageal motility disorder: achalasia (5%), EGJ outflow obstruction (20%), jackhammer (8%), diffuse esophageal spasm (1%), ineffective esophageal motility (28%), and fragmented peristalsis (2%). Of note, 50% of the sample with a normal barium swallow had an esophageal dysmotility disorder on HREM. Those with psoriatic arthritis were less likely to have esophageal dysmotility (73% vs. 27%; P = 0.04). There was decreased odds of esophageal hypocontractility in those with RA (OR [95%CI]: 0.27 [0.12-0.58]) and increased odds (OR [95%CI]: 3.13 [1.16-8.41]) of esophageal hypocontractility among those with scleroderma. Esophageal motor disorders were found in more than half of patients with rheumatologic diseases who underwent HREM. HREM should be considered in patients with rheumatic conditions presenting with esophageal symptoms. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. FAU - Reddy, Sumana AU - Reddy S AD - Division of Gastroenterology and Hepatology, University of Texas Southwestern Medical Center, Dallas, TX, USA. FAU - Patel, Beyla AU - Patel B AD - Department of Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, USA. FAU - Dellon, Evan S AU - Dellon ES AD - Center for Esophageal Diseases and Swallowing, Division of Gastroenterology and Hepatology, Center for Gastrointestinal Biology and Disease, Chapel Hill, NC, USA. FAU - Eluri, Swathi AU - Eluri S AD - Center for Esophageal Diseases and Swallowing, Division of Gastroenterology and Hepatology, Center for Gastrointestinal Biology and Disease, Chapel Hill, NC, USA. AD - Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL, USA. LA - eng PT - Journal Article PL - United States TA - Dis Esophagus JT - Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus JID - 8809160 SB - IM MH - Humans MH - *Esophageal Motility Disorders/epidemiology/physiopathology/etiology/complications MH - Middle Aged MH - Female MH - Male MH - Retrospective Studies MH - *Manometry MH - *Rheumatic Diseases/complications/epidemiology/physiopathology MH - Prevalence MH - Aged MH - Scleroderma, Systemic/complications/epidemiology/physiopathology MH - Logistic Models MH - Lupus Erythematosus, Systemic/complications/physiopathology/epidemiology MH - Raynaud Disease/epidemiology/etiology MH - Arthritis, Rheumatoid/complications/physiopathology/epidemiology MH - Sjogren's Syndrome/complications/epidemiology/physiopathology MH - Adult MH - Mixed Connective Tissue Disease/complications/epidemiology MH - Esophagus/physiopathology OTO - NOTNLM OT - esophageal motor disorders OT - esophagus OT - manometry EDAT- 2024/11/26 00:16 MHDA- 2025/01/09 00:22 CRDT- 2024/11/25 20:22 PHST- 2024/07/19 00:00 [received] PHST- 2024/10/25 00:00 [revised] PHST- 2024/11/15 00:00 [accepted] PHST- 2025/01/09 00:22 [medline] PHST- 2024/11/26 00:16 [pubmed] PHST- 2024/11/25 20:22 [entrez] AID - 7908445 [pii] AID - 10.1093/dote/doae108 [doi] PST - ppublish SO - Dis Esophagus. 2025 Jan 7;38(1):doae108. doi: 10.1093/dote/doae108. PMID- 24489014 OWN - NLM STAT- MEDLINE DCOM- 20140617 LR - 20250529 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 53 IP - 5 DP - 2014 May TI - A double-blind, randomized, placebo-controlled crossover trial of the α2C-adrenoceptor antagonist ORM-12741 for prevention of cold-induced vasospasm in patients with systemic sclerosis. PG - 948-52 LID - 10.1093/rheumatology/ket421 [doi] AB - OBJECTIVES: Our primary purpose was to evaluate the efficacy of the high-potency α2C-adrenoceptor antagonist ORM-12741 in the attenuation of a cold-induced reduction in finger blood flow and temperature in patients with RP secondary to SSc. Secondary objectives were to assess safety and tolerability. METHODS: This was a phase IIa, randomized, double-blind, crossover, single-dose, placebo-controlled, single-centre study. Patients attended five times: initial screening, treatment visits 1-3 (each at least 1 week apart) and 1-2 weeks after the last treatment. At each treatment visit, each subject received a single oral dose of 30 mg or 100 mg of ORM-12741 or placebo. Thirty minutes later the subject underwent a cold challenge. Blood flow to the fingers was assessed by three methods [temperature by probe, laser Doppler imaging (LDI) and infrared thermography] performed before, during and after the cold challenge. RESULTS: Twelve patients (10 female, mean age 58 years) were included. The area under the rewarming curve (LDI) of the right index finger (arbitrary flux units × time) was lower for both 30 mg (P = 0.043) and 100 mg (P = 0.025) of ORM-12741 compared with placebo, indicating delayed reperfusion. The time to 70% temperature recovery (middle finger probe) was longer with active than placebo treatment: mean (s.d.) values for placebo, 30 mg of ORM-12741 and 100 mg of ORM-12741 were 21.4 min (12.4), 25.7 min (12.2) and 26.9 min (13.9), respectively. Overall ORM-12741 was well tolerated. CONCLUSION: ORM-12741 did not expedite recovery from a cold challenge in the fingers of patients with SSc. TRIAL REGISTRATION: https://www.clinicaltrialsregister.eu/; no. 2010-024005-13. FAU - Herrick, Ariane L AU - Herrick AL AD - Clinical Sciences Building, Salford Royal NHS Foundation Trust, Salford M6 8HD, UK. ariane.herrick@manchester.ac.uk. FAU - Murray, Andrea K AU - Murray AK FAU - Ruck, Angela AU - Ruck A FAU - Rouru, Juha AU - Rouru J FAU - Moore, Tonia L AU - Moore TL FAU - Whiteside, John AU - Whiteside J FAU - Hakulinen, Pasi AU - Hakulinen P FAU - Wigley, Fredrick AU - Wigley F FAU - Snapir, Amir AU - Snapir A LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom PT - Clinical Trial, Phase II PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20140131 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Adrenergic alpha-2 Receptor Antagonists) RN - 0 (Benzofurans) RN - 0 (ORM-12741) RN - 0 (Quinolizidines) RN - 0 (Receptors, Adrenergic, alpha-2) RN - X4W3ENH1CV (Norepinephrine) RN - YKH834O4BH (Epinephrine) SB - IM MH - Adrenergic alpha-2 Receptor Antagonists/adverse effects/*pharmacology/*therapeutic use MH - Adult MH - Aged MH - Benzofurans/adverse effects/pharmacology/therapeutic use MH - Body Temperature/drug effects/physiology MH - Cold Temperature/*adverse effects MH - Cross-Over Studies MH - Dose-Response Relationship, Drug MH - Double-Blind Method MH - Epinephrine/blood MH - Female MH - Fingers/blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Norepinephrine/blood MH - Quinolizidines/adverse effects/pharmacology/therapeutic use MH - Raynaud Disease/*etiology/physiopathology/*prevention & control MH - Receptors, Adrenergic, alpha-2/*drug effects MH - Regional Blood Flow/drug effects/physiology MH - Scleroderma, Systemic/*complications MH - Thermography MH - Treatment Outcome OTO - NOTNLM OT - Raynaud’s phenomenon OT - randomized controlled trial OT - systemic sclerosis OT - α2C-adrenoceptor antagonism EDAT- 2014/02/04 06:00 MHDA- 2014/06/18 06:00 CRDT- 2014/02/04 06:00 PHST- 2014/02/04 06:00 [entrez] PHST- 2014/02/04 06:00 [pubmed] PHST- 2014/06/18 06:00 [medline] AID - ket421 [pii] AID - 10.1093/rheumatology/ket421 [doi] PST - ppublish SO - Rheumatology (Oxford). 2014 May;53(5):948-52. doi: 10.1093/rheumatology/ket421. Epub 2014 Jan 31. PMID- 23940211 OWN - NLM STAT- MEDLINE DCOM- 20141230 LR - 20260518 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 73 IP - 12 DP - 2014 Dec TI - Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. PG - 2087-93 LID - 10.1136/annrheumdis-2013-203716 [doi] AB - OBJECTIVES: The EULAR (European League Against Rheumatism) Scleroderma Trials and Research Group (EUSTAR) has identified preliminary criteria for very early diagnosis of systemic sclerosis (SSc). Our aim was to assess the prevalence of each proposed diagnostic item in a large observational patient cohort with Raynaud's phenomenon (RP). METHODS: Baseline data of 469 RP patients enrolled into the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) cohort are presented. RESULTS: 68% of all RP patients were antinuclear antibody (ANA) positive. ANA+ RP patients more frequently had previous or current puffy fingers (PuFi) (38.5% and 23.3%, p<0.01) and an SSc pattern on nailfold capillaroscopy (NC) (53.6% and 13.4%, p<0.001) than ANA- patients. Telangiectasia, current digital ulcers and digital pitting scars were also commoner in ANA+ RP patients. 38% of ANA+ patients presented with all three features, which should raise suspicion of very early SSc (ANA+RP+PuFi constitutes a 'red flag'). These patients more frequently exhibited an NC SSc pattern, sclerodactyly and telangiectases compared to ANA+ patients without PuFi. Almost 90% of patients with 'red flags' had anti-centromere or anti-topoisomerase I antibodies and/or an NC SSc pattern, and fulfilled the EUSTAR criteria for very early SSc. Previous or current PuFi were present in 23.3% of ANA- RP patients, eight of whom also had an NC SSc pattern. CONCLUSIONS: In addition to well-characterised predictive factors, PuFi is an important sign raising suspicion for underlying very early SSc in patients with RP. The relevance of PuFi in ANA- RP patients should be clarified. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. FAU - Minier, Tünde AU - Minier T AD - Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Lepri, Gemma AU - Lepri G AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Czirják, László AU - Czirják L AD - Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Walker, Ulrich A AU - Walker UA AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. FAU - Fransen, Jaap AU - Fransen J AD - Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Université Paris Descartes, Paris, France. FAU - Denton, Christopher AU - Denton C AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, UK. FAU - Cutolo, Maurizio AU - Cutolo M AD - Department of Internal Medicine, University of Genova, Research Laboratory and Academic Clinical Unit of Rheumatology, Viale Benedetto, Italy. FAU - Tyndall, Alan AU - Tyndall A AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Giessen, Germany. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. CN - EUSTAR co-workers CN - EUSTAR co-workers LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20130812 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/immunology MH - Cohort Studies MH - Early Diagnosis MH - Female MH - Fingers/blood supply/*pathology MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/complications/*diagnosis/immunology MH - Scleroderma, Systemic/complications/*diagnosis/immunology MH - Skin Ulcer/complications/*diagnosis/immunology MH - Telangiectasis/complications/*diagnosis/immunology OTO - NOTNLM OT - Autoantibodies OT - Disease Activity OT - Outcomes research OT - Pulmonary Fibrosis OT - Systemic Sclerosis FIR - Airò, Paolo IR - Airò P FIR - Zingarelli, Stefania IR - Zingarelli S FIR - Ananieva, Lidia IR - Ananieva L FIR - Desinova, Oxana IR - Desinova O FIR - Ancuta, Codrina Mihaela IR - Ancuta CM FIR - Belibou, Codruta Iulia IR - Belibou CI FIR - Avouac, Jerome IR - Avouac J FIR - Becvar, Radim IR - Becvar R FIR - Skacelova, Simona IR - Skacelova S FIR - Beretta, Lorenzo IR - Beretta L FIR - Vigone, Barbara IR - Vigone B FIR - Caramaschi, Paola IR - Caramaschi P FIR - Sabbagh, Dania IR - Sabbagh D FIR - Carpentier, Patrick IR - Carpentier P FIR - Damjanov, Nemanja IR - Damjanov N FIR - Simic-Pasalic, Katarina IR - Simic-Pasalic K FIR - Distler, Jorg Hw IR - Distler JH FIR - Farge-Bancel, Dominique IR - Farge-Bancel D FIR - Hadj-Khelifa, Sondess IR - Hadj-Khelifa S FIR - Foti, Rosario IR - Foti R FIR - Di Gangi, Marcella IR - Di Gangi M FIR - de la Pena Lefebvre, Paloma Garcia IR - de la Pena Lefebvre PG FIR - Hachulla, Eric IR - Hachulla E FIR - Salvador, Maria Joao IR - Salvador MJ FIR - Kayser, Cristiane IR - Kayser C FIR - Camargo, Cintia Zumstein IR - Camargo CZ FIR - Kumánovics, Gábor IR - Kumánovics G FIR - Li, Mengtao IR - Li M FIR - Xu, Dong IR - Xu D FIR - Marasini, Bianca IR - Marasini B FIR - Belloli, Laura IR - Belloli L FIR - Maurer, Britta IR - Maurer B FIR - Mayer, Miroslav IR - Mayer M FIR - Mihai, Carina IR - Mihai C FIR - Gherghe, Ana Maria IR - Gherghe AM FIR - Riccieri, Valeria IR - Riccieri V FIR - Stefanantoni, Katia IR - Stefanantoni K FIR - Salsano, Felice IR - Salsano F FIR - Rosato, Edoardo IR - Rosato E FIR - Senecal, Jean-Luc IR - Senecal JL FIR - Koenig, Martial IR - Koenig M FIR - Senet, Patricia IR - Senet P FIR - Frances, Camille IR - Frances C FIR - Sipek, Alenka IR - Sipek A FIR - Stankovic, Aleksandra IR - Stankovic A FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - Smith, Vanessa IR - Smith V FIR - Tarner, Ingo H IR - Tarner IH FIR - Wiland, Piotr IR - Wiland P EDAT- 2013/08/14 06:00 MHDA- 2014/12/31 06:00 CRDT- 2013/08/14 06:00 PHST- 2013/08/14 06:00 [entrez] PHST- 2013/08/14 06:00 [pubmed] PHST- 2014/12/31 06:00 [medline] AID - S0003-4967(24)09783-8 [pii] AID - 10.1136/annrheumdis-2013-203716 [doi] PST - ppublish SO - Ann Rheum Dis. 2014 Dec;73(12):2087-93. doi: 10.1136/annrheumdis-2013-203716. Epub 2013 Aug 12. PMID- 33461416 OWN - NLM STAT- MEDLINE DCOM- 20211004 LR - 20211204 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 30 IP - 4 DP - 2021 Apr TI - Myositis in systemic lupus erythematosus. PG - 615-619 LID - 10.1177/0961203320988587 [doi] AB - OBJECTIVES: Myositis is an infrequent feature of SLE and may often be overlooked. We aimed to estimate the incidence of myositis in SLE, and to determine demographic and clinical factors associated with it. METHODS: Within our lupus cohort, we identified potential myositis cases using the SLICC Damage Index for muscle atrophy or weakness, the SLEDAI-2K item for myositis, and annually measured serum creatinine kinase. Cases were confirmed through chart review. We performed descriptive analyses of prevalent myositis cases as of January 2000. From that point onward, we studies patients without myositis to determine risk of incident myositis, using cohort analyses adjusted for demographic variables (age, sex, race/ethnicity). RESULTS: As of January 2000, there were 5 prevalent myositis cases in our SLE cohort. Among 560 SLE patients with a study visit from January 2000 onward, with no history of myositis at baseline, 5 new cases (4 females, 1 male) were identified over an average follow-up of 8.5 years (incidence 1.05 cases per 1000 person-years). There was a higher proportion of Caucasians in the non-myositis group versus myositis group, with a trend for fewer females in the myositis cases. Arthritis, Raynaud's phenomenon, and anti-Smith antibodies were common pre-existing features, occurring in all incident myositis cases. In Cox regression analyses adjusting for age, race/ethnicity and sex, non-Caucasian patients had a markedly increased risk of developing myositis. CONCLUSION: We found a low incidence of myositis in our SLE cohort. A cluster of variables, particularly non-Caucasian race/ethnicity, arthritis, Raynaud's phenomenon, and anti-Smith antibodies were associated with risk of developing myositis in SLE. These variables may aid clinicians in identifying SLE patients at highest risk for this important complication. FAU - Cotton, Thaisa AU - Cotton T AD - Department of Medicine, McGill University, Montreal, Canada. FAU - Niaki, Omid Zahedi AU - Niaki OZ AD - Division of Rheumatology, McGill University Health Centre, Montreal, Canada. FAU - Zheng, Boyang AU - Zheng B AD - Department of Medicine, McGill University, Montreal, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Canada. FAU - Pineau, Christian A AU - Pineau CA AD - Department of Medicine, McGill University, Montreal, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Canada. FAU - Fritzler, Marvin AU - Fritzler M AUID- ORCID: 0000-0003-1652-6608 AD - Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Canada. FAU - Vinet, Evelyne AU - Vinet E AUID- ORCID: 0000-0001-7727-5879 AD - Department of Medicine, McGill University, Montreal, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Canada. FAU - Clarke, Ann E AU - Clarke AE AD - Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Canada. FAU - Bernatsky, Sasha AU - Bernatsky S AUID- ORCID: 0000-0002-9515-2802 AD - Department of Medicine, McGill University, Montreal, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Canada. LA - eng PT - Journal Article DEP - 20210118 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (anti-small nuclear ribonucleoproteins autoantibodies) RN - EC 2.7.3.2 (Creatine Kinase) SB - IM MH - Adult MH - Antibodies, Antinuclear/immunology MH - Arthritis/diagnosis/epidemiology MH - Atrophy/pathology MH - Cohort Studies MH - Creatine Kinase/blood MH - Female MH - Follow-Up Studies MH - Humans MH - Incidence MH - Lupus Erythematosus, Systemic/*complications/diagnosis/epidemiology MH - Male MH - Middle Aged MH - Muscle Weakness/physiopathology MH - Myositis/epidemiology/*ethnology/*etiology/*pathology MH - Prospective Studies MH - Raynaud Disease/diagnosis/epidemiology MH - Regression Analysis MH - Severity of Illness Index OTO - NOTNLM OT - Systemic lupus erythematosus OT - cohort OT - myositis EDAT- 2021/01/20 06:00 MHDA- 2021/10/05 06:00 CRDT- 2021/01/19 05:36 PHST- 2021/01/20 06:00 [pubmed] PHST- 2021/10/05 06:00 [medline] PHST- 2021/01/19 05:36 [entrez] AID - 10.1177/0961203320988587 [doi] PST - ppublish SO - Lupus. 2021 Apr;30(4):615-619. doi: 10.1177/0961203320988587. Epub 2021 Jan 18. PMID- 28894946 OWN - NLM STAT- MEDLINE DCOM- 20180614 LR - 20260518 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 37 IP - 11 DP - 2017 Nov TI - An international SUrvey on non-iNvaSive tecHniques to assess the mIcrocirculation in patients with RayNaud's phEnomenon (SUNSHINE survey). PG - 1879-1890 LID - 10.1007/s00296-017-3808-0 [doi] AB - To canvas opinion concerning the role of non-invasive techniques in the assessment of patients with Raynaud's phenomenon (Rp) in clinical and research settings: four nailfold capillaroscopy methods [videocapillaroscopy (NVC), dermoscopy, stereomicroscopy, digital USB microscopy], four laser Doppler methods (laser Doppler flowmetry, imaging, anemometry/velocimetry, laser speckle contrast analysis), thermographic imaging, and upper limb arterial Doppler ultrasound. Emails with a link to the survey were sent to physicians from the European Scleroderma Trials and Research group (EUSTAR), the EULAR Study Group on Microcirculation in Rheumatic Diseases (SG_MC/RD) and members of the pediatric rheumatology Email board. The main descriptive analysis related to physicians looking after adult patients, with some analysis also of opinions from paediatric rheumatologists. 106 'adult physicians' responded (a response rate of 25.8%), of whom 68.9% were European, and 81.1% practising for more than 10 years. Nineteen paediatricians responded. The most widely available technique was NVC (72.7%). Nailfold capillaroscopy was most frequently performed by the physician him/herself, using different types of equipment relating to availability. Most rheumatologists reported high levels of appropriateness for NVC in both clinical and research settings for global assessment and differential diagnosis of Rp. Other techniques were less used. Of all the different techniques, nailfold capillaroscopy was the one most used in both clinical and research settings by adult physicians, the majority of whom use NVC in their everyday practice. The low proportion of clinicians using other techniques suggests that these are currently mainly research tools, available only in specialist centres. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Gaetano Pini, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, P.zza Cardinal Ferrari 1, 20122, Milan, Italy. francesca.ingegnoli@unimi.it. FAU - Ughi, Nicola AU - Ughi N AD - Division of Rheumatology, ASST Gaetano Pini, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, P.zza Cardinal Ferrari 1, 20122, Milan, Italy. FAU - Dinsdale, Graham AU - Dinsdale G AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Orenti, Annalisa AU - Orenti A AD - Laboratory of Medical Statistics, Epidemiology and Biometry G.A. Maccacaro, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy. FAU - Boracchi, Patrizia AU - Boracchi P AD - Laboratory of Medical Statistics, Epidemiology and Biometry G.A. Maccacaro, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University and Cochin Hospital, AP-HP, Paris, France. FAU - Foeldvari, Ivan AU - Foeldvari I AD - Hamburger Zentrum für Kinder- und Jugend Rheumatologie, Klinikum Eilbek, Hamburg, Germany. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Ghent University, Ghent, Belgium. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. CN - EULAR Study Group on Microcirculation in RheumaticDiseases LA - eng PT - Journal Article DEP - 20170911 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Diagnosis, Differential MH - Humans MH - *Microcirculation MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/*physiopathology MH - Surveys and Questionnaires OTO - NOTNLM OT - Anemometry/velocimetry OT - Connective tissue disease OT - Dermoscopy OT - Laser Doppler flowmetry OT - Laser Doppler imaging OT - Laser speckle contrast analysis OT - Nailfold capillaroscopy OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Thermographic imaging OT - Upper limb arterial Doppler ultrasound OT - Videocapillaroscopy FIR - Hij, Adrian IR - Hij A FIR - Sulli, Alberto IR - Sulli A FIR - Nitsche, Alejandro IR - Nitsche A FIR - Vacca, Alessandra IR - Vacca A FIR - Balbir-Gurman, Alexandra IR - Balbir-Gurman A FIR - Abdessemed, Amina IR - Abdessemed A FIR - Vargas, Angelica IR - Vargas A FIR - Valenzuela, Antonia IR - Valenzuela A FIR - Makol, Ashima IR - Makol A FIR - Baranauskaite, Asta IR - Baranauskaite A FIR - Derfalvi, Beata IR - Derfalvi B FIR - Serrano Benavente, Belén IR - Serrano Benavente B FIR - Sozeri, Betul IR - Sozeri B FIR - Bica, Blanca E IR - Bica BE FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - Mihai, Carina IR - Mihai C FIR - Chizzolini, Carlo IR - Chizzolini C FIR - Abud Mendoza, Carlos IR - Abud Mendoza C FIR - de la Puente, Carlos IR - de la Puente C FIR - von Muhlen, Carlos IR - von Muhlen C FIR - Bertolazzi, Chiara IR - Bertolazzi C FIR - Pain, Clare IR - Pain C FIR - Ickinger, Claudia IR - Ickinger C FIR - Ancuta, Codrina IR - Ancuta C FIR - Sunderkotter, Cord IR - Sunderkotter C FIR - Kayser, Cristiane IR - Kayser C FIR - De Araujo, Daniel B IR - De Araujo DB FIR - Launay, David IR - Launay D FIR - Khanna, Dinesh IR - Khanna D FIR - Krasowska, Dorota IR - Krasowska D FIR - Veale, Douglas IR - Veale D FIR - Kaliterna, Dušanka M IR - Kaliterna DM FIR - Rosato, Edoardo IR - Rosato E FIR - de Langhe, Ellen IR - de Langhe E FIR - Hachulla, Eric IR - Hachulla E FIR - Naredo, Esperanza IR - Naredo E FIR - Loyo, Esthela IR - Loyo E FIR - Alvarez Hernández, Everardo IR - Alvarez Hernández E FIR - Sztajnbok, Flavio IR - Sztajnbok F FIR - Boin, Francesco IR - Boin F FIR - Longo, Francisco J IR - Longo FJ FIR - van den Hoogen, Frank IR - van den Hoogen F FIR - Hernandez Molina, Gabriela IR - Hernandez Molina G FIR - Riemekasten, Gabriela IR - Riemekasten G FIR - Szucs, Gabriella IR - Szucs G FIR - Moroncini, Gianluca IR - Moroncini G FIR - Fragoso Loyo, Hilda IR - Fragoso Loyo H FIR - Dobrev, Hristo IR - Dobrev H FIR - Janta, Iustina IR - Janta I FIR - Cracowski, Jean-Luc IR - Cracowski JL FIR - Pauling, John IR - Pauling J FIR - Akikusa, Jonathan IR - Akikusa J FIR - Sotoca Fernàndez, Jorge IR - Sotoca Fernàndez J FIR - Khan Ajaz, Kariem IR - Khan Ajaz K FIR - Solanki, Kamal IR - Solanki K FIR - Wierzba, Karol IR - Wierzba K FIR - Romanowska Próchnicka, Katarzyna IR - Romanowska Próchnicka K FIR - Rouster Stevens, Kelly IR - Rouster Stevens K FIR - Belloli, Laura IR - Belloli L FIR - Lewandowski, Laura IR - Lewandowski L FIR - Santos, Lelita IR - Santos L FIR - Saketkoo, Lesley A IR - Saketkoo LA FIR - Ananyeva, Lidia IR - Ananyeva L FIR - Beretta, Lorenzo IR - Beretta L FIR - Michalska Jakubus, Małgorzata IR - Michalska Jakubus M FIR - Audisio, Marcelo J IR - Audisio MJ FIR - Milchert, Marcin IR - Milchert M FIR - Molina, Maria J IR - Molina MJ FIR - Moraes, Fontes Maria F IR - Moraes FMF FIR - Terreri, Maria T IR - Terreri MT FIR - Puszczewicz, Mariusz IR - Puszczewicz M FIR - Barešić, Marko IR - Barešić M FIR - Hufnagel, Markus IR - Hufnagel M FIR - Mamani, Marta N IR - Mamani MN FIR - Gutierrez, Marwin IR - Gutierrez M FIR - Curran, Megan IR - Curran M FIR - Hughes, Michael IR - Hughes M FIR - Becker, Mike IR - Becker M FIR - Inanç, Murat IR - Inanç M FIR - Petraitis, Mykolas IR - Petraitis M FIR - Juan Carlos, Nieto-Gonzàlez IR - Juan Carlos NG FIR - Fathi, Nihal IR - Fathi N FIR - Aktay Ayaz, Nuray IR - Aktay Ayaz N FIR - Distler, Oliver IR - Distler O FIR - Sander, Oliver IR - Sander O FIR - Ömer, Pamuk N IR - Ömer PN FIR - García de la Peña Lefebvre, Paloma IR - García de la Peña Lefebvre P FIR - Caramaschi, Paola IR - Caramaschi P FIR - Hasler, Paul IR - Hasler P FIR - Ostojic, Predrag IR - Ostojic P FIR - Bečvář, Radim IR - Bečvář R FIR - Rodríguez, Reyna S Tatiana IR - Rodríguez RST FIR - Lima, Rodrigo IR - Lima R FIR - Hesselstrand, Roger IR - Hesselstrand R FIR - Cimaz, Rolando IR - Cimaz R FIR - Irace, Rosaria IR - Irace R FIR - Petty, Ross IR - Petty R FIR - de Angelis, Rossella IR - de Angelis R FIR - Dobrota, Rucsandra IR - Dobrota R FIR - Payne-Poff, Sarah IR - Payne-Poff S FIR - Kubo, Satoshi IR - Kubo S FIR - Guiducci, Serena IR - Guiducci S FIR - Popa, Serghei IR - Popa S FIR - Lambova, Sevdalina IR - Lambova S FIR - Stebbings, Simon IR - Stebbings S FIR - Rednic, Simona IR - Rednic S FIR - Yavuz, Sule IR - Yavuz S FIR - Benseler, Susa IR - Benseler S FIR - Shevtsova, Tatzana IR - Shevtsova T FIR - Daikeler, Thomas IR - Daikeler T FIR - Schmeiser, Tim IR - Schmeiser T FIR - Frech, Tracy IR - Frech T FIR - Minier, Tünde IR - Minier T FIR - Müller Ladner, Ulf IR - Müller Ladner U FIR - Walker, Ulrich IR - Walker U FIR - Riccieri, Valeria IR - Riccieri V FIR - Vilela, Verônica IR - Vilela V FIR - Hermann, Walter IR - Hermann W FIR - Braun-Moscovici, Yolanda IR - Braun-Moscovici Y FIR - Uziel, Yosef IR - Uziel Y FIR - Thierry, Zenone IR - Thierry Z EDAT- 2017/09/13 06:00 MHDA- 2018/06/15 06:00 CRDT- 2017/09/13 06:00 PHST- 2017/04/03 00:00 [received] PHST- 2017/08/28 00:00 [accepted] PHST- 2017/09/13 06:00 [pubmed] PHST- 2018/06/15 06:00 [medline] PHST- 2017/09/13 06:00 [entrez] AID - 10.1007/s00296-017-3808-0 [pii] AID - 10.1007/s00296-017-3808-0 [doi] PST - ppublish SO - Rheumatol Int. 2017 Nov;37(11):1879-1890. doi: 10.1007/s00296-017-3808-0. Epub 2017 Sep 11. PMID- 21781293 OWN - NLM STAT- MEDLINE DCOM- 20120329 LR - 20211020 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 13 IP - 4 DP - 2011 Jul 22 TI - Atypical clinical presentation of a subset of patients with anti-RNA polymerase III--non-scleroderma cases associated with dominant RNA polymerase I reactivity and nucleolar staining. PG - R119 LID - 10.1186/ar3422 [doi] AB - INTRODUCTION: Anti-RNA polymerase III (RNAP III) antibodies are highly specific markers of scleroderma (systemic sclerosis, SSc) and associated with a rapidly progressing subset of SSc. The clinical presentation of anti-RNAP III positive patients, onset of Raynaud's phenomenon (RP) and SSc in unselected patients in a rheumatology clinic were evaluated. METHODS: Autoantibodies in sera from 1,966 unselected patients (including 434 systemic lupus erythematosus (SLE), 119 SSc, 85 polymyositis/dermatomyositis (PM/DM)) in a rheumatology clinic were screened by radioimmunoprecipitation. Anti-RNAP III positive sera were also tested by immunofluorescence antinuclear antibodies and anti-RNAP III ELISA. Medical records of anti-RNAP III positive patients were reviewed. RESULTS: Among 21 anti-RNAP III positive patients, 16 met the American College of Rheumatology (ACR) SSc criteria at the initial visit but 5 did not; diagnoses were vasculitis, early polyarthritis, renal failure with RP, interstitial lung disease, and Sjögren's syndrome. The first two patients developed rapidly progressive diffuse SSc. An additional case presented with diffuse scleroderma without RP and RP developed two years later. Anti-RNAP III antibodies in these 6 cases of atypical clinical presentation were compared with those in 15 cases of typical (SSc with RP) cases. Anti-RNAP III levels by ELISA were lower in the former group (P = 0.04 by Mann-Whitney test) and 3 of 6 were negative versus only 1 of 15 negative in the latter (P < 0.05 by Fisher's exact test). Three cases of non-SSc anti-RNAP III positive patients had predominant reactivity with RNAP I with weak RNAP III reactivity and had a strong nucleolar staining. Three anti-RNAP III patients, who did not have RP at the initial visit, developed RP months later. Scleroderma developed prior to RP in 5 out of 16 (31%) in the anti-RNAP III group, but this was rare in patients with other autoantibodies. The interval between the onset of RP to scleroderma was short in anti-RNAP III positive patients. CONCLUSIONS: Anti-RNAP III antibodies are highly specific for SSc; however, a subset of anti-RNAP III positive patients do not present as typical SSc. The interval between RP and scleroderma in this group is short, and 31% of patients developed scleroderma prior to RP in this group. Anti-RNAP III positive patients may not present as typical SSc and detecting anti-RNAP III may have predictive value. FAU - Ceribelli, Angela AU - Ceribelli A AD - Department of Oral Biology, University of Florida, 1395 Center Drive, Gainesville, FL 32610-0424, USA. FAU - Krzyszczak, Malgorzata E AU - Krzyszczak ME FAU - Li, Yi AU - Li Y FAU - Ross, Steven J AU - Ross SJ FAU - Chan, Jason Y F AU - Chan JY FAU - Chan, Edward K L AU - Chan EK FAU - Burlingame, Rufus W AU - Burlingame RW FAU - Webb, Tyler T AU - Webb TT FAU - Bubb, Michael R AU - Bubb MR FAU - Sobel, Eric S AU - Sobel ES FAU - Reeves, Westley H AU - Reeves WH FAU - Satoh, Minoru AU - Satoh M LA - eng GR - M01R00082/PHS HHS/United States GR - R01-AR40391/AR/NIAMS NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20110722 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - EC 2.7.7.6 (RNA Polymerase I) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Adult MH - Aged MH - Autoantibodies/*blood/immunology MH - Autoantigens/immunology MH - Cell Nucleolus/immunology/metabolism MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fluorescent Antibody Technique MH - Humans MH - Middle Aged MH - RNA Polymerase I/*immunology MH - RNA Polymerase III/*immunology MH - Radioimmunoprecipitation Assay MH - Raynaud Disease/diagnosis/*immunology/metabolism MH - Scleroderma, Systemic/diagnosis/*immunology/metabolism PMC - PMC3239357 EDAT- 2011/07/26 06:00 MHDA- 2012/03/30 06:00 PMCR- 2011/07/22 CRDT- 2011/07/26 06:00 PHST- 2011/03/03 00:00 [received] PHST- 2011/05/24 00:00 [revised] PHST- 2011/07/22 00:00 [accepted] PHST- 2011/07/26 06:00 [entrez] PHST- 2011/07/26 06:00 [pubmed] PHST- 2012/03/30 06:00 [medline] PHST- 2011/07/22 00:00 [pmc-release] AID - ar3422 [pii] AID - 10.1186/ar3422 [doi] PST - epublish SO - Arthritis Res Ther. 2011 Jul 22;13(4):R119. doi: 10.1186/ar3422. PMID- 36066501 OWN - NLM STAT- MEDLINE DCOM- 20230309 LR - 20260518 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 75 IP - 3 DP - 2023 Mar TI - Efficacy and Safety of Botulinum Toxin in Adults with Raynaud's Phenomenon Secondary to Systemic Sclerosis: A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study. PG - 459-467 LID - 10.1002/art.42342 [doi] AB - OBJECTIVE: To determine whether a single session of botulinum toxin type A (BTA) injections into both hands more effectively decreases the frequency of systemic sclerosis-associated Raynaud's phenomenon (SSc-RP) episodes than placebo. METHODS: This multicenter, randomized, double-blind, placebo-controlled, parallel-group phase III trial in patients with SSc-RP assessed the effect of 50-unit BTA or placebo injections into the palms of both hands around each neurovascular bundle during 1 session in winter. The primary end point was the between-group difference in the median change in the number of RP episodes from baseline (day 0) to 4 weeks postinjection. Values between the groups were compared with the Wilcoxon rank-sum test. RESULTS: The intent-to-treat analysis included 46 BTA-treated patients and 44 placebo recipients. At 4 weeks after assigned treatment injections, the median number of daily RP episodes decreased comparably in the BTA and placebo groups (median change -1 episode/day [interquartile range (IQR) -1.5, 0 episodes/day] and -1 episode/day [IQR -2.5, 0 episodes/day], respectively) (P = 0.77 versus placebo). Moreover, change in Raynaud's Condition Score, quality of life assessed by Health Assessment Questionnaire disability index, and hand function assessed by shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH) and Cochin Hand Function Scale from baseline to follow-up weeks 4, 12, and 24 did not differ significantly between groups. The BTA group experienced transient hand muscle weakness significantly more frequently (P = 0.003). CONCLUSION: Neither the primary nor secondary end points were reached, and our results do not support any beneficial effect of palmar BTA injections to treat SSc-RP. CI - © 2022 American College of Rheumatology. FAU - Senet, Patricia AU - Senet P AUID- ORCID: 0000-0002-9253-8419 AD - Assistance Publique-Hôpitaux de Paris, and Service de Dermatologie, Centre Hospitalier Universitaire Tenon, Groupe Hospitalier Sorbonne Université, Paris, France. FAU - Maillard, Hervé AU - Maillard H AD - Service de Dermatologie, Centre Hospitalier Le Mans, Le Mans, France. FAU - Diot, Elisabeth AU - Diot E AD - Service de Médecine Interne et Immunologie Clinique, Centre Hospitalier Universitaire Bretonneau-Tours, Tours, France. FAU - Lazareth, Isabelle AU - Lazareth I AD - Service de Médecine Vasculaire, Hôpital Saint-Joseph, Paris, France. FAU - Blaise, Sophie AU - Blaise S AUID- ORCID: 0000-0001-7238-112X AD - Université Grenoble Alpes, Inserm, HP2, Grenoble, France. FAU - Arnault, Jean-Philippe AU - Arnault JP AD - Service de Dermatologie, Centre Hospitalier Universitaire Amiens-Picardie, Amiens, France. FAU - Pistorius, Marc-Antoine AU - Pistorius MA AD - Service de Médecine Interne, Centre Hospitalier Universitaire, Nantes, France. FAU - Boulon, Carine AU - Boulon C AD - Service de Médecine Vasculaire, Centre Hospitalier Universitaire St-André, Bordeaux, France. FAU - Cogrel, Olivier AU - Cogrel O AD - Unité de Dermatologie Interventionnelle, Service de Dermatologie, Centre Hospitalier Universitaire, Bordeaux, France. FAU - Warzocha, Ursula AU - Warzocha U AD - Assistance Publique-Hôpitaux de Paris, and Service de Médecine Interne, Centre Hospitalier Universitaire Avicenne, Bobigny, France. FAU - Rivière, Sébastien AU - Rivière S AD - Assistance Publique-Hôpitaux de Paris, and Service de Médecine Interne, Centre Hospitalier Universitaire Saint-Antoine, Groupe Hospitalier Sorbonne Université, Paris, France. FAU - Malloizel-Delaunay, Julie AU - Malloizel-Delaunay J AD - Service de Médecine Vasculaire, Centre Hospitalier Universitaire, Toulouse, France. FAU - Servettaz, Amélie AU - Servettaz A AD - Service de Médecine Interne, Maladies Infectieuses, Immunologie Clinique, Hôpital Robert-Debré, Centre Hospitalier Universitaire, Reims, France. FAU - Sassolas, Bruno AU - Sassolas B AD - Département de Médecine Interne et Pneumologie, Hôpital de la Cavale Blanche, Centre Hospitalier Régional Universitaire, Brest, France. FAU - Viguier, Manuelle AU - Viguier M AD - Service de Dermatologie-Vénéréologie, Hôpital Robert-Debré, Université Reims Champagne Ardenne, IRMAIC, EA7509, Reims, France. FAU - Monfort, Jean-Benoit AU - Monfort JB AD - Assistance Publique-Hôpitaux de Paris, and Service de Dermatologie, Centre Hospitalier Universitaire Tenon, Groupe Hospitalier Sorbonne Université, Paris, France. FAU - Janique, Solène AU - Janique S AD - Assistance Publique-Hôpitaux de Paris, Unité de Recherche Clinique, Centre Hospitalier Universitaire Fernand-Widal, Paris, France. FAU - Vicaut, Eric AU - Vicaut E AD - Assistance Publique-Hôpitaux de Paris, Unité de Recherche Clinique, Centre Hospitalier Universitaire Fernand-Widal, Paris, France. CN - BRASS collaborators LA - eng SI - ClinicalTrials.gov/NCT03717961 PT - Clinical Trial, Phase III PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20221214 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM CIN - Arthritis Rheumatol. 2023 Mar;75(3):486. doi: 10.1002/art.42379. PMID: 36209513 MH - Humans MH - Adult MH - Quality of Life MH - *Scleroderma, Systemic/complications/drug therapy MH - *Botulinum Toxins, Type A/therapeutic use MH - Hand MH - *Raynaud Disease/drug therapy/etiology FIR - Senet, Patricia IR - Senet P FIR - Monfort, Jean-Benoit IR - Monfort JB FIR - De Risi, Tullia IR - De Risi T FIR - Boulon, Carine IR - Boulon C FIR - Constans, Joel IR - Constans J FIR - Seneschal, Julien IR - Seneschal J FIR - Le Moal, Cyril IR - Le Moal C FIR - Blaise, Sophie IR - Blaise S FIR - Seinturier, Christophe IR - Seinturier C FIR - Imbert, Bernard IR - Imbert B FIR - Lazareth, Isabelle IR - Lazareth I FIR - Gautier, Valentine IR - Gautier V FIR - Michon-Pasturel, Ulriche IR - Michon-Pasturel U FIR - Diot, Elisabeth IR - Diot E FIR - Ferreira-Maldent, Nicole IR - Ferreira-Maldent N FIR - Pistorius, Marc-Antoine IR - Pistorius MA FIR - Espitia, Olivier IR - Espitia O FIR - Durant, Cécile IR - Durant C FIR - Lok, Catherine IR - Lok C FIR - Chaby, Guillaume IR - Chaby G FIR - Arnault, Jean-Philippe IR - Arnault JP FIR - Rivière, Sébastien IR - Rivière S FIR - Maillard, Hervé IR - Maillard H FIR - Beneton, Nathalie IR - Beneton N FIR - Malloizel, Julie IR - Malloizel J FIR - Lapebie, François-Xavier IR - Lapebie FX FIR - Viguier, Manuelle IR - Viguier M FIR - Servettaz, Amélie IR - Servettaz A FIR - Loget, Jeffrey IR - Loget J FIR - Robert-Debré, Hôpital IR - Robert-Debré H FIR - Zumelzu, Coralie IR - Zumelzu C FIR - Warzocha, Ursula IR - Warzocha U FIR - Larroche, Claire IR - Larroche C FIR - Caux, Frédéric IR - Caux F FIR - Empana-Barat, Florence IR - Empana-Barat F FIR - Perrot, Elodie IR - Perrot E FIR - Jouis, Véronique IR - Jouis V FIR - Janique, Solène IR - Janique S FIR - Mboup, Bassirou IR - Mboup B FIR - Guimfack, Aurélie IR - Guimfack A EDAT- 2022/09/07 06:00 MHDA- 2023/03/10 06:00 CRDT- 2022/09/06 10:52 PHST- 2022/08/10 00:00 [revised] PHST- 2022/04/07 00:00 [received] PHST- 2022/08/31 00:00 [accepted] PHST- 2022/09/07 06:00 [pubmed] PHST- 2023/03/10 06:00 [medline] PHST- 2022/09/06 10:52 [entrez] AID - 10.1002/art.42342 [doi] PST - ppublish SO - Arthritis Rheumatol. 2023 Mar;75(3):459-467. doi: 10.1002/art.42342. Epub 2022 Dec 14. PMID- 40875130 OWN - NLM STAT- MEDLINE DCOM- 20251118 LR - 20251123 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 44 IP - 10 DP - 2025 Oct TI - A retrospective natural history study in adult and juvenile patients with incident dermatomyositis and polymyositis using real world data. PG - 4237-4247 LID - 10.1007/s10067-025-07614-6 [doi] AB - OBJECTIVE: This retrospective natural history study used real-world data to describe baseline demographics, comorbidities, clinical characteristics, and treatments, and assess incidence rates (IRs) of extra-muscular outcomes in patients with incident dermatomyositis (DM), polymyositis (PM), juvenile DM (JDM), and juvenile PM (JPM). METHODS: De-identified clinical data were collected from the US Optum® electronic health records with supplemental claims (01 January 2016 to 31 March 2021). Total 9,009 patients were included (DM: 4,275; PM: 4,559; JDM: 128; JPM: 47). IRs of 13 outcomes were estimated in patients and an equal number of sex- and age-matched controls (MCs) without DM/PM. RESULTS: Mean age at index was 54.5 (DM), 57.3 (PM), 14.3 (JDM), and 15.1 years (JPM). Most common comorbidities were hypertension in DM (50.3%) and PM (63.9%) cohorts, dysphagia in JDM (15.6%) and liver disease in JPM (23.4%) cohorts. Most common clinical characteristics were Raynaud's phenomenon in DM (8.6%), PM (7.9%), and JDM (11.7%) cohorts, and arthritis in JPM (10.6%) cohort. Systemic steroids were the most frequent medication (DM: 70.3%; PM: 68.3%; JDM: 73.4%; JPM: 59.6%). IRs (per 100 person years) of outcomes in all cohorts were higher in patients versus their MCs. In DM and PM cohorts, highest IRs were observed for gastroesophageal reflux disease (DM:10.3; PM:12.8). In JDM cohort, dysphagia (4.3) had highest IR. In JPM cohort, cardiac dysrhythmia (3.5) had highest IR. CONCLUSION: This study addresses existing gaps in understanding the descriptive epidemiology of DM and PM in the US, particularly the IRs of extra-muscular disease manifestations and malignancy events. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Barnes, David M AU - Barnes DM AUID- ORCID: 0000-0002-7329-1254 AD - Pfizer Inc, MS 66HB-13N251/261, 66 Hudson Boulevard East, New York, NY, 10001, USA. david.barnes@pfizer.com. FAU - Graham, Daniela AU - Graham D AD - Pfizer Inc, Groton, CT, USA. FAU - Borlenghi, Cecilia E AU - Borlenghi CE AD - Pfizer Inc, Buenos Aires, Argentina. FAU - Edwards, Thomas AU - Edwards T AD - Panalgo Inc, Boston, MA, USA. FAU - Schachterle, Stephen E AU - Schachterle SE AD - Pfizer Inc, MS 66HB-13N251/261, 66 Hudson Boulevard East, New York, NY, 10001, USA. FAU - Sile, Helen AU - Sile H AD - Pfizer Inc, MS 66HB-13N251/261, 66 Hudson Boulevard East, New York, NY, 10001, USA. LA - eng PT - Journal Article PT - Observational Study DEP - 20250828 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Steroids) RN - 0 (Immunosuppressive Agents) SB - IM EIN - Clin Rheumatol. 2026 Jan;45(1):623. doi: 10.1007/s10067-025-07800-6. PMID: 41264141 MH - Retrospective Studies MH - *Dermatomyositis/complications/drug therapy/epidemiology MH - *Polymyositis/complications/drug therapy/epidemiology MH - Incidence MH - Raynaud Disease/epidemiology/etiology MH - Arthritis/epidemiology/etiology MH - Case-Control Studies MH - Gastroesophageal Reflux/epidemiology/etiology MH - Deglutition Disorders/epidemiology/etiology MH - Arrhythmias, Cardiac/epidemiology/etiology MH - Steroids/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Humans MH - Male MH - Female MH - Adolescent MH - Middle Aged MH - United States/epidemiology MH - Comorbidity PMC - PMC12634748 OTO - NOTNLM OT - Dermatomyositis (DM) OT - Incidence rates OT - Juvenile dermatomyositis (JDM) OT - Juvenile polymyositis (JPM) OT - Natural history OT - Polymyositis (PM) COIS- Declarations. Conflicts of interest: David M. Barnes, Daniela Graham, and Stephen E. Schachterle are employees of Pfizer and hold Pfizer stocks. Cecilia E. Borlenghi and Helen Sile are stockholders and former employees of Pfizer. Thomas Edwards has no conflicts of interest or financial disclosures to declare. EDAT- 2025/08/28 20:45 MHDA- 2025/10/13 18:29 PMCR- 2025/08/28 CRDT- 2025/08/28 11:20 PHST- 2025/03/03 00:00 [received] PHST- 2025/07/25 00:00 [accepted] PHST- 2025/07/21 00:00 [revised] PHST- 2025/10/13 18:29 [medline] PHST- 2025/08/28 20:45 [pubmed] PHST- 2025/08/28 11:20 [entrez] PHST- 2025/08/28 00:00 [pmc-release] AID - 10.1007/s10067-025-07614-6 [pii] AID - 7614 [pii] AID - 10.1007/s10067-025-07614-6 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Oct;44(10):4237-4247. doi: 10.1007/s10067-025-07614-6. Epub 2025 Aug 28. PMID- 23221323 OWN - NLM STAT- MEDLINE DCOM- 20130717 LR - 20130218 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 52 IP - 3 DP - 2013 Mar TI - Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. PG - 560-7 LID - 10.1093/rheumatology/kes315 [doi] AB - OBJECTIVE: To assess patients with SSc who present without circulating ANAs or RP. METHODS: Five thousand three hundred and ninety patients who fulfilled the ACR criteria for SSc and were enrolled in the EULAR Scleroderma Trials and Research (EUSTAR) database were screened for the absence of both RP and circulating ANA. To differentiate SSc from its mimics, additional information was gathered using a standardized questionnaire. RESULTS: Five thousand three hundred and seventy-eight (99.8%) of the 5390 SSc patients in the EUSTAR database had either detectable ANA or a history of RP. Twelve (0.2%) patients lacked both circulating ANA and RP. Details of the medical history could be obtained for seven patients. Three cases were compatible with ANA-negative and RP-negative SSc and were not typical of any known SSc mimic. Four patients had a malignancy: two had breast cancer, one had multiple myeloma with possible scleromyxoedema and one had bladder carcinoma. There was no temporal relationship between the onset of skin fibrosis and that of the tumour. Although no patient with confirmed nephrogenic systemic fibrosis was identified among the cases of ANA-negative and RP-negative SSc, the presentation of one patient could be compatible with that of nephrogenic systemic fibrosis other than for the absence of chronic kidney disease or of known prior gadolinium exposure. CONCLUSION: We have identified a very small subgroup of SSc patients who lack both circulating ANA and RP, none of whom fulfils the diagnostic criteria for any known SSc mimic. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients. FAU - Schneeberger, Daniel AU - Schneeberger D AD - Department of Rheumatology, Basel University, Felix Platter Spital, Basel, Switzerland. FAU - Tyndall, Alan AU - Tyndall A FAU - Kay, Jonathan AU - Kay J FAU - Søndergaard, Klaus H AU - Søndergaard KH FAU - Carreira, Patricia E AU - Carreira PE FAU - Morgiel, Ewa AU - Morgiel E FAU - Deuschle, Katrin AU - Deuschle K FAU - Derk, Chris T AU - Derk CT FAU - Widuchowska, Malgorzata AU - Widuchowska M FAU - Walker, Ulrich A AU - Walker UA LA - eng PT - Journal Article PT - Multicenter Study DEP - 20121205 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/*analysis MH - Databases, Factual MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/*immunology MH - Scleroderma, Diffuse/diagnosis/*immunology EDAT- 2012/12/12 06:00 MHDA- 2013/07/19 06:00 CRDT- 2012/12/11 06:00 PHST- 2012/12/11 06:00 [entrez] PHST- 2012/12/12 06:00 [pubmed] PHST- 2013/07/19 06:00 [medline] AID - kes315 [pii] AID - 10.1093/rheumatology/kes315 [doi] PST - ppublish SO - Rheumatology (Oxford). 2013 Mar;52(3):560-7. doi: 10.1093/rheumatology/kes315. Epub 2012 Dec 5. PMID- 18974625 OWN - NLM STAT- MEDLINE DCOM- 20090316 LR - 20220410 IS - 1349-7413 (Electronic) IS - 0911-4300 (Linking) VI - 31 IP - 5 DP - 2008 Oct TI - [Clinical features and laboratory findings in children with both anti-dsDNA and anti-U1-RNP antibody]. PG - 405-14 AB - Mixed connective tissue disease (MCTD) includes clinical features of systemic lupus erythematosus (SLE), dermatomyositis/polymyositis (DM/PM), and systemic sclerosis (SSc) occurring in conjunction with a high anti-U1-RNP antibody titer. Childhood MCTD rarely manifests the symptoms and signs of DM/PM and SSc, and mostly does those of SLE. Thus, the diagnosis of childhood MCTD is inevitably based on the two major findings, Raynaud's phenomenon and a high titer of anti-U1-RNP antibody. However, in clinical setting there exist patients who have both anti-dsDNA antibody, a SLE disease-marker, and anti-U1-RNP antibody, a MCTD disease-marker, and thus it is hard to differentiate MCTD patients from SLE. Eighty children were enrolled in this study, and divided into 3 groups ; group A, those who are positive for anti-dsDNA antibody/negative for anti-U1-RNP antibody (48 cases, 60.0%), group B : those who are positive for both anti-dsDNA and anti-U1-RNP antibody (22 cases, 27.5%), group C; those who are negative for anti-dsDNA antibody/positive for anti-U1-RNP antibody (10 cases, 12.5%), and each of the clinical characteristics among these 3 groups was mutually examined. The results indicated that the frequency of hypocomplementemia in group B was close to group A rather than group C, and the frequencies of both hyper-gamma-globulinemia and Raynaud's phenomenon were very close to group C, but not to group A. On the contrary, the findings which seemed to be specific to MCTD, high titers of speckled type anti-nuclear antibody and rheumatoid factor, located at the middle between group A and group C. Thus, children in group B essentially carried characteristic symptoms and signs of both SLE and MCTD, and it will be difficult to differentiate these two diseases at the onset of the disease. Taken together, children with high titers of both anti-dsDNA antibody and anti-U1-RNP antibody as well as clinical symptoms and signs such as hyper-gamma-globulinemia, Raynaud's phenomenon, membranous nephritis, positive speckled type anti-nuclear antibody and rheumatoid factor should be followed and treated as children with MCTD along with SLE. FAU - Miyamae, Takako AU - Miyamae T AD - Department of Pediatrics, Yokohama City University. FAU - Ito, Shuichi AU - Ito S FAU - Machida, Hiroyuki AU - Machida H FAU - Ozawa, Remi AU - Ozawa R FAU - Higuchi, Rumiko AU - Higuchi R FAU - Nakajima, Shoko AU - Nakajima S FAU - Imagawa, Tomoyuki AU - Imagawa T FAU - Nakamura, Tomoko AU - Nakamura T FAU - Mori, Masaaki AU - Mori M FAU - Aihara, Yuko AU - Aihara Y FAU - Ohshige, Kenji AU - Ohshige K FAU - Yokota, Shumpei AU - Yokota S LA - jpn PT - English Abstract PT - Journal Article PL - Japan TA - Nihon Rinsho Meneki Gakkai Kaishi JT - Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology JID - 9505992 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 9007-49-2 (DNA) SB - IM MH - Antibodies, Antinuclear/*analysis MH - Autoantibodies/*analysis MH - Child MH - DNA/*immunology MH - Humans MH - Lupus Erythematosus, Systemic/*immunology MH - Mixed Connective Tissue Disease/*diagnosis/immunology MH - Raynaud Disease/*immunology MH - Ribonucleoprotein, U1 Small Nuclear/*immunology EDAT- 2008/11/01 09:00 MHDA- 2009/03/17 09:00 CRDT- 2008/11/01 09:00 PHST- 2008/11/01 09:00 [pubmed] PHST- 2009/03/17 09:00 [medline] PHST- 2008/11/01 09:00 [entrez] AID - JST.JSTAGE/jsci/31.405 [pii] AID - 10.2177/jsci.31.405 [doi] PST - ppublish SO - Nihon Rinsho Meneki Gakkai Kaishi. 2008 Oct;31(5):405-14. doi: 10.2177/jsci.31.405. PMID- 23910609 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - Clinical characteristics of systemic sclerosis patients with digital ulcers in China. PG - 46-9 AB - OBJECTIVES: To investigate the clinical characteristics of SSc patients with DUs in China. METHODS: The data of 267 consecutive SSc patients based on the EUSTAR DATABASE from Peking Union Medical College Hospital from February 2009 to March 2012 were prospectively collected. The patients with DUs were compared to those without DUs. RESULTS: Seventy-nine patients (29.6%) had DUs out of 267 SSc patients analysed. There were significant differences between patients with and without DU based on sex (female/male: 65/14 vs. 174/14), age of onset of SSc (32.3±11.7 vs. 40.4±12.6 y), age of onset of Raynaud's phenomenon (31.8±12.3 vs. 38.7±12.2) (p<0.05). In addition, there was a higher rate of diffuse SSc, gastrointestinal involvement, (especially esophageal involvement), and pericardial effusion, higher mRodnan score, and more anti-scl70 antibody positivity in patients with DU (p<0.05). A multivariate analysis identified anti-Scl70 antibody positivity, gastrointestinal involvement and a younger age at disease onset as three risk factors for developing DUs in SSc patients. CONCLUSIONS: The occurrence of DUs in Chinese SSc patients is frequent. It is possible that SSc patients with DUs were influenced by the disease earlier in life, which should be detected early for effective intervention. FAU - Xu, Dong AU - Xu D AD - Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, China. xudong74@hotmail.com FAU - Li, Meng-Tao AU - Li MT FAU - Hou, Yong AU - Hou Y FAU - Wang, Qian AU - Wang Q FAU - Hu, Chao-Jun AU - Hu CJ FAU - Song, Ning AU - Song N FAU - Zhao, Jiu-Liang AU - Zhao JL FAU - Zeng, Xiao-Feng AU - Zeng XF FAU - Zhang, Feng-Chun AU - Zhang FC LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130722 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - China/epidemiology MH - Esophageal Diseases/epidemiology MH - Female MH - Fingers/*pathology MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/epidemiology MH - Scleroderma, Systemic/*epidemiology/*pathology/therapy MH - Skin Ulcer/*epidemiology/*pathology/therapy MH - Young Adult EDAT- 2013/08/16 06:00 MHDA- 2013/09/11 06:00 CRDT- 2013/08/06 06:00 PHST- 2013/01/03 00:00 [received] PHST- 2013/03/15 00:00 [accepted] PHST- 2013/08/06 06:00 [entrez] PHST- 2013/08/16 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 6759 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):46-9. Epub 2013 Jul 22. PMID- 17728365 OWN - NLM STAT- MEDLINE DCOM- 20071220 LR - 20220311 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 16 IP - 9 DP - 2007 TI - Peripheral vascular disease in systemic lupus erythematosus. PG - 720-3 AB - With increasing longevity of lupus patients, peripheral vascular disease (PVD) has become an important cause of morbidity. With no systematic study of PVD in systemic lupus erythematosus (SLE), this study was undertaken to define the frequency and spectrum of PVD in SLE and factors affecting such an occurrence. All medium-sized peripheral arteries of bilateral upper and lower extremities were studied in 50 SLE patients using Doppler ultrasonography. PVD was defined clinically as one or more of intermittent claudication, absent/unequal pulses, gangrene or ischemic ulcers and sub-clinically as asymptomatic patients with Doppler abnormalities, with > or =50% reduction in diameter considered hemodynamically significant. Mean (SD) age of the patients was 31.6 (10.1) years. Forty-one percent were hypertensive. Dyslipidemia was found in 62%. Fifteen (30%) had Raynaud's phenomenon. Fourteen (28%) patients had PVD, of whom three had positive markers for antiphospholipid antibody (aPL) and six were asymptomatic. Ischemic ulcers were seen in eight (16%), gangrene in three (6%), femoral artery plaques in two (4%), stenosis in four (8%) and intermittent claudication in none. Dyslipidemia was found to independently affect occurrence of PVD (OR = 5.37, [95% CI 1.05-27.5], P = 0.05). The causes of PVD overlap significantly and further studies are needed to ascertain the relative contribution of each. FAU - Bhatt, S P AU - Bhatt SP AD - Department of Medicine, All India Institute of Medical Sciences, New Delhi, India. FAU - Handa, R AU - Handa R FAU - Gulati, G S AU - Gulati GS FAU - Sharma, S AU - Sharma S FAU - Pandey, R M AU - Pandey RM FAU - Aggarwal, P AU - Aggarwal P FAU - Ramakrishnan, L AU - Ramakrishnan L FAU - Shankar, S AU - Shankar S LA - eng PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Adolescent MH - Adult MH - Antibodies, Antiphospholipid/metabolism MH - Cross-Sectional Studies MH - Dyslipidemias/complications MH - Female MH - Humans MH - Hypertension/complications MH - Lupus Erythematosus, Systemic/*complications/physiopathology MH - Male MH - Middle Aged MH - Peripheral Vascular Diseases/epidemiology/*etiology/*physiopathology MH - Prospective Studies MH - Raynaud Disease/complications MH - Ultrasonography, Doppler EDAT- 2007/08/31 09:00 MHDA- 2007/12/21 09:00 CRDT- 2007/08/31 09:00 PHST- 2007/08/31 09:00 [pubmed] PHST- 2007/12/21 09:00 [medline] PHST- 2007/08/31 09:00 [entrez] AID - 16/9/720 [pii] AID - 10.1177/0961203307081123 [doi] PST - ppublish SO - Lupus. 2007;16(9):720-3. doi: 10.1177/0961203307081123. PMID- 20506251 OWN - NLM STAT- MEDLINE DCOM- 20100729 LR - 20250529 IS - 1529-0131 (Electronic) IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 62 IP - 7 DP - 2010 Jul TI - Heritability of vasculopathy, autoimmune disease, and fibrosis in systemic sclerosis: a population-based study. PG - 2109-16 LID - 10.1002/art.27469 [doi] AB - OBJECTIVE: To investigate the familiality of systemic sclerosis (SSc) in relation to Raynaud's phenomenon (RP) (a marker of vasculopathy), other autoimmune inflammatory disease, and fibrotic interstitial lung disease (ILD). METHODS: A genealogic resource, the Utah Population Database (UPDB), was used to test heritability of RP, other autoimmune disease, and ILD. Diseases were defined by International Classification of Diseases, Ninth Revision codes and identified from statewide discharge data, the University of Utah Health Science Center Enterprise Data Warehouse, and death certificates and were linked to the UPDB for analysis. Familial standardized incidence ratio (FSIR), relative risks (RRs) to first-, second-, third-, and fourth-degree relatives for SSc, RP, other autoimmune disease, and ILD (with 95% confidence intervals [95% CIs]), and population attributable risk (PAR) were calculated. RESULTS: A software kinship analysis tool was used to analyze 1,037 unique SSc patients. Fifty SSc families had significant FSIRs, ranging from 2.07 to 17.60. The adjusted PAR was approximately 8%. The RRs were significant for other autoimmune disease in the first-degree relatives (2.49 [95% CI 1.99-3.41], P = 2.42 x 10(-15)) and second-degree relatives (1.48 [95% CI 1.34-2.39], P = 0.002), for RP in first-degree relatives (6.38 [95% CI 3.44-11.83], P = 4.04 x 10(-9)) and second-degree relatives (2.39 [95% CI 1.21-4.74], P = 0.012), and for ILD in first-degree relatives (1.53 [95% CI 1.04-2.26], P = 0.03), third-degree relatives (1.47 [95% CI 1.18-1.82], P = 0.0004), and fourth-degree relatives (1.2 [95% CI 1.06-1.35], P = 0.004). CONCLUSION: These data suggest that SSc pedigrees include more RP, autoimmune inflammatory disease, and ILD than would be expected by chance. In SSc pedigrees, genetic predisposition to vasculopathy is the most frequent risk among first-degree relatives. FAU - Frech, Tracy AU - Frech T AD - University of Utah, Division of Rheumatology, Department of Medicine, Salt Lake City, UT 84132, USA. tracy.frech@hsc.utah.edu FAU - Khanna, Dinesh AU - Khanna D FAU - Markewitz, Boaz AU - Markewitz B FAU - Mineau, Geraldine AU - Mineau G FAU - Pimentel, Richard AU - Pimentel R FAU - Sawitzke, Allen AU - Sawitzke A LA - eng GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - K23-AR-053858-03/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Autoimmune Diseases/epidemiology/*genetics MH - Cause of Death MH - Comorbidity MH - *Family Health MH - Female MH - *Genetic Predisposition to Disease MH - Humans MH - Lung Diseases, Interstitial/epidemiology/*genetics MH - Male MH - Pedigree MH - Raynaud Disease/epidemiology/*genetics MH - Scleroderma, Systemic/complications/*genetics MH - Utah/epidemiology PMC - PMC2956122 MID - NIHMS211096 EDAT- 2010/05/28 06:00 MHDA- 2010/07/30 06:00 PMCR- 2011/07/01 CRDT- 2010/05/28 06:00 PHST- 2010/05/28 06:00 [entrez] PHST- 2010/05/28 06:00 [pubmed] PHST- 2010/07/30 06:00 [medline] PHST- 2011/07/01 00:00 [pmc-release] AID - 10.1002/art.27469 [doi] PST - ppublish SO - Arthritis Rheum. 2010 Jul;62(7):2109-16. doi: 10.1002/art.27469. PMID- 31687398 OWN - NLM STAT- MEDLINE DCOM- 20200324 LR - 20200324 IS - 2314-6141 (Electronic) IS - 2314-6133 (Print) VI - 2019 DP - 2019 TI - Th1- and Th17-Related Cytokines in Venous and Arterial Blood of Sclerodermic Patients with and without Digital Ulcers. PG - 7908793 LID - 10.1155/2019/7908793 [doi] LID - 7908793 AB - The earliest clinical manifestation of SSc is usually Raynaud's phenomenon, a small-arteries vasospasm driven by vascular tone dysregulation and microcirculatory abnormalities, resulting in digital ulcers (DU) in up to 50% of patients. Many cytokines as well as growth factors have been shown to play a role in promoting vascular smooth muscle cell proliferation and fibroblast activation, leading to ischemic damage as well as skin fibrosis. We aim to investigate a possible difference in venous and arterial blood levels of many cytokines (Th1- and Th17-related), GM-CSF, and endothelin-1 (ET1) in patients with and without DU. In the same patients, the correlations between capillary damage, evaluated by nailfold videocapillaroscopy (NVC), extension of skin fibrosis, calculated by modified Rodnan skin score (mRSS), and cytokines, ET-1, and GM-CSF levels were also measured. Patients with DU showed venous levels of IL-1β (p=0.024), IL-6 (p=0.012), IL-22(p=0.006), and TGF-β (p=0.046) significantly higher compared to arterial levels and arterial levels of GM-CSF and TNF-alpha significantly higher compared to venous levels (p < 0.001). NVC abnormalities were correlated with arterial TNFa and venous IL22, IL23, and IL17 levels and negatively correlated with venous ET-1 levels, whereas mRSS showed a negative correlation with IL-21(ρ = -0.427, p=0.050). The increased Th17-cytokine levels in venous compared to arterial blood of patients with DU suggest local cytokine production on ulcer site. The higher TNFa and GM-CSF levels in arterial blood of DU patients support the attempt to mitigate the hypoxic damage, and the correlation between Th17-cytokines, mRSS, NVC, and ET1 agrees with the potent profibrotic stimulus at the onset of the disease, which decreases as the SSc progresses. CI - Copyright © 2019 S. Nicola et al. FAU - Nicola, S AU - Nicola S AUID- ORCID: 0000-0002-9955-194X AD - Università degli Studi di Torino, Dipartimento di Scienze Mediche-AO Ordine Mauriziano Umberto I, Torino, Italy. FAU - Fornero, M AU - Fornero M AD - Università degli Studi di Torino, Dipartimento di Scienze Mediche-AO Ordine Mauriziano Umberto I, Torino, Italy. FAU - Fusaro, E AU - Fusaro E AD - SC Reumatologia, AOU Città della Salute e della Scienza, Torino, Italy. FAU - Peroni, C AU - Peroni C AD - SC Reumatologia, AOU Città della Salute e della Scienza, Torino, Italy. FAU - Priora, M AU - Priora M AD - SC Reumatologia, AOU Città della Salute e della Scienza, Torino, Italy. FAU - Rolla, G AU - Rolla G AD - Università degli Studi di Torino, Dipartimento di Scienze Mediche-AO Ordine Mauriziano Umberto I, Torino, Italy. FAU - Bucca, C AU - Bucca C AUID- ORCID: 0000-0002-9941-9236 AD - Università degli Studi di Torino, Dipartimento di Scienze Mediche-AO Ordine Mauriziano Umberto I, Torino, Italy. FAU - Brussino, L AU - Brussino L AUID- ORCID: 0000-0001-7249-7616 AD - Università degli Studi di Torino, Dipartimento di Scienze Mediche-AO Ordine Mauriziano Umberto I, Torino, Italy. LA - eng PT - Journal Article DEP - 20191007 PL - United States TA - Biomed Res Int JT - BioMed research international JID - 101600173 RN - 0 (Cytokines) RN - 0 (Endothelin-1) RN - 0 (Tumor Necrosis Factor-alpha) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Arteries/*metabolism MH - Capillaries/metabolism MH - Cytokines/*blood/metabolism MH - Endothelin-1/metabolism MH - Female MH - Fibrosis/metabolism MH - Humans MH - Male MH - Microcirculation/physiology MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Raynaud Disease/*blood/metabolism MH - Skin/metabolism MH - Skin Ulcer/*blood/metabolism MH - Th1 Cells/*metabolism MH - Th17 Cells/*metabolism MH - Tumor Necrosis Factor-alpha/metabolism MH - Veins/*metabolism PMC - PMC6800960 COIS- Nicola S., Fornero M., Fusaro E., Peroni C., Priora M., Rolla G., Bucca C. and Brussino L. declare that they have no conflicts of interest. EDAT- 2019/11/07 06:00 MHDA- 2020/03/25 06:00 PMCR- 2019/10/07 CRDT- 2019/11/06 06:00 PHST- 2019/06/21 00:00 [received] PHST- 2019/07/10 00:00 [revised] PHST- 2019/08/30 00:00 [accepted] PHST- 2019/11/06 06:00 [entrez] PHST- 2019/11/07 06:00 [pubmed] PHST- 2020/03/25 06:00 [medline] PHST- 2019/10/07 00:00 [pmc-release] AID - 10.1155/2019/7908793 [doi] PST - epublish SO - Biomed Res Int. 2019 Oct 7;2019:7908793. doi: 10.1155/2019/7908793. eCollection 2019. PMID- 38782468 OWN - NLM STAT- MEDLINE DCOM- 20240711 LR - 20240711 IS - 1399-3003 (Electronic) IS - 0903-1936 (Linking) VI - 64 IP - 1 DP - 2024 Jul TI - Non-arterial line cardiac output calculation misclassifies exercise pulmonary hypertension and increases risk of data loss particularly in black, scleroderma and Raynaud's patients during invasive exercise testing. LID - 2302232 [pii] LID - 10.1183/13993003.02232-2023 [doi] AB - BACKGROUND: The direct Fick principle is the standard for calculating cardiac output (CO) to detect CO-dependent conditions like exercise pulmonary hypertension (ePH). Fick CO(arterial) incorporates arterial haemoglobin (Hb(a)) and oxygen saturation (S (aO(2)) ) with oxygen consumption from exercise testing, while Fick CO(non-arterial) substitutes mixed venous haemoglobin (Hb(mv)) and peripheral oxygen saturation (S (pO(2)) ) in the absence of an arterial line. The decision to employ an arterial catheter for exercise testing varies, and discrepancies in oxygen saturation and haemoglobin between arterial and non-arterial methods may lead to differences in Fick CO, potentially affecting ePH classification. METHODS: We performed a retrospective analysis of 296 consecutive invasive cardiopulmonary exercise testing (iCPET) studies comparing oxygen saturation from pulse oximetry (S (pO(2)) ) and radial arterial (S (aO(2)) ), Hb(a) and Hb(mv), and CO calculated with arterial (CO(arterial)) and non-arterial (CO(non-arterial)) values. We assessed the risk of misclassification of pre- and post-capillary ePH and data loss due to inaccurate S (pO(2)) . RESULTS: When considering all stages from rest to peak exercise, Hb(a) and Hb(mv) demonstrated high correlation, while S (pO(2)) and S (aO(2)) as well as CO(arterial) and CO(non-arterial) demonstrated low correlation. Data loss was significantly higher across all stages of exercise for S (pO(2)) (n=346/1926 (18%)) compared to S (aO(2)) (n=17/1923 (0.88%)). We found that pre- and post-capillary ePH were misclassified as CO(non-arterial) data (n=7/41 (17.1%) and n=2/23 (8.7%), respectively). Patients with scleroderma and/or Raynaud's (n=11/33 (33.3%)) and black patients (n=6/19 (31.6%)) had more S (pO(2)) data loss. CONCLUSION: Reliance upon S (pO(2)) during invasive exercise testing results in the misclassification of pre- and post-capillary ePH, and unmeasurable S (pO(2)) for black, scleroderma and Raynaud's patients can preclude accurate exercise calculations, thus limiting the diagnostic and prognostic value of invasive exercise testing without an arterial line. CI - Copyright ©The authors 2024. For reproduction rights and permissions contact permissions@ersnet.org. FAU - Campedelli, Luiz AU - Campedelli L AD - Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA. FAU - Nouraie, S Mehdi AU - Nouraie SM AUID- ORCID: 0000-0001-7465-0581 AD - Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA. AD - Centre for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung, Blood, and Vascular Medicine Institute, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA. FAU - Risbano, Michael G AU - Risbano MG AUID- ORCID: 0000-0003-3334-7046 AD - Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA risbanomg@upmc.edu. AD - Centre for Pulmonary Vascular Biology and Medicine, Pittsburgh Heart, Lung, Blood, and Vascular Medicine Institute, University of Pittsburgh School of Medicine and UPMC, Pittsburgh, PA, USA. LA - eng PT - Journal Article DEP - 20240711 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 RN - 0 (Hemoglobins) SB - IM CIN - Eur Respir J. 2024 Jul 11;64(1):2400783. doi: 10.1183/13993003.00783-2024. PMID: 38991723 MH - Humans MH - Retrospective Studies MH - Female MH - Male MH - Middle Aged MH - *Cardiac Output MH - *Exercise Test MH - *Hypertension, Pulmonary/physiopathology MH - *Raynaud Disease/diagnosis MH - *Exercise MH - Adult MH - *Oximetry MH - Scleroderma, Systemic/physiopathology MH - Aged MH - Oxygen Saturation MH - Oxygen Consumption MH - Hemoglobins/analysis/metabolism COIS- Conflict of interest: M.G. Risbano reports grants from the Shadyside Hospital Foundation, royalties from Springer, and advisory board participation from Gilead and Liquidia. L. Campedelli and S.M. Nouraie report grants from the Shadyside Hospital Foundation. EDAT- 2024/05/24 00:42 MHDA- 2024/07/12 00:41 CRDT- 2024/05/23 20:43 PHST- 2023/12/13 00:00 [received] PHST- 2024/05/04 00:00 [accepted] PHST- 2024/07/12 00:41 [medline] PHST- 2024/05/24 00:42 [pubmed] PHST- 2024/05/23 20:43 [entrez] AID - 13993003.02232-2023 [pii] AID - 10.1183/13993003.02232-2023 [doi] PST - epublish SO - Eur Respir J. 2024 Jul 11;64(1):2302232. doi: 10.1183/13993003.02232-2023. Print 2024 Jul. PMID- 34262566 OWN - NLM STAT- MEDLINE DCOM- 20210730 LR - 20221207 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 12 DP - 2021 TI - Case Report: Systemic Sclerosis After Covid-19 Infection. PG - 686699 LID - 10.3389/fimmu.2021.686699 [doi] LID - 686699 AB - The coronavirus disease (COVID-19) is a respiratory tract infection caused by the new virus SARS-CoV-2. The acute phase of the infection may in certain individuals be followed by another longer phase of disease (long COVID) of unknown etiology probably associated in certain cases with autoimmune activation. It has been shown that COVID-19 can trigger autoantibody production and in genetically predisposed patients may cause the onset or exacerbation of autoimmune diseases. We are reporting a case of mild COVID-19 infection complicated by autoantibody production and cutaneous and gastrointestinal symptoms and subsequently diagnosed with systemic sclerosis (SSc). A 47-year-old man with no history of any autoimmune diseases and in good health became sick together with his family on the 12th of November with mild symptoms: tiredness, fever, cough, and sore throat. Oropharyngeal swab for SARS-CoV-2 tested positive. He was isolated at home and did not require hospitalization. Three weeks later he presented with clinical manifestation compatible with suspicion of SSc. He briefly presented with skin rush, periorbital edema and conjunctivitis, vomiting, dysphagia, burning sensation in the skin, above all in the fingertips and around the mouth, puffy fingers, Raynaud's phenomenon, pain at the fingertip of the middle finger where a depressed area was noticed without a clear ulceration. ANA showed a strongly positive nucleolar pattern. Anti-PM/Scl 75 and PM/Scl 100 resulted positive. High-resolution computed tomography (HCRT) showed early stage of interstitial lung disease (ILD). The patient was diagnosed with SSc based on the persistence of autoantibodies and the clinical and radiological pictures according to the ACR/EULAR classification (scores: puffy finger, 2; ILD, 2; Raynaud's phenomenon, 3; SSc related antibodies, 3; total 10). There are several cases described in the medical literature of possible new onset of SLE after COVID-19 infection. This is the first case that describes a possible new onset of SSc. Conclusion: SARS-CoV-2 may trigger systemic sclerosis. CI - Copyright © 2021 Fineschi. FAU - Fineschi, Serena AU - Fineschi S AD - Department of Public Health and Caring Sciences, Unit of General Practice, Uppsala University, Uppsala, Sweden. AD - Östhammar Health Care Centre, Östhammar, Sweden. LA - eng PT - Case Reports PT - Journal Article DEP - 20210628 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - 0 (Calcium Channel Blockers) RN - 0 (Proton Pump Inhibitors) SB - IM MH - Autoantibodies/immunology MH - Autoimmune Diseases/*etiology MH - *Autoimmunity MH - COVID-19/*complications/immunology/virology MH - Calcium Channel Blockers/therapeutic use MH - Follow-Up Studies MH - Humans MH - Lung Diseases, Interstitial/diagnostic imaging/etiology MH - Male MH - Middle Aged MH - Proton Pump Inhibitors/therapeutic use MH - Raynaud Disease/diagnosis/etiology MH - SARS-CoV-2/*genetics MH - Scleroderma, Systemic/drug therapy/*etiology MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Post-Acute COVID-19 Syndrome PMC - PMC8273695 OTO - NOTNLM OT - COVID-19 OT - autoimmunity OT - long COVID OT - scleroderma OT - systemic sclerosis COIS- The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/07/16 06:00 MHDA- 2021/07/31 06:00 PMCR- 2021/06/28 CRDT- 2021/07/15 06:19 PHST- 2021/03/27 00:00 [received] PHST- 2021/06/07 00:00 [accepted] PHST- 2021/07/15 06:19 [entrez] PHST- 2021/07/16 06:00 [pubmed] PHST- 2021/07/31 06:00 [medline] PHST- 2021/06/28 00:00 [pmc-release] AID - 10.3389/fimmu.2021.686699 [doi] PST - epublish SO - Front Immunol. 2021 Jun 28;12:686699. doi: 10.3389/fimmu.2021.686699. eCollection 2021. PMID- 40108705 OWN - NLM STAT- MEDLINE DCOM- 20250320 LR - 20250529 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 27 IP - 1 DP - 2025 Mar 19 TI - Efficacy of adipose stromal cells-enriched high-density fat graft combined with BTX-A for Raynaud's phenomenon: a prospective cohort study. PG - 56 LID - 10.1186/s13075-025-03533-8 [doi] LID - 56 AB - BACKGROUND: Conventional treatments for Raynaud's phenomenon (RP) often show limited effectiveness due to their inability to address both vascular and inflammatory aspects. This study evaluates the combination of high-density fat grafting (HDFG) with botulinum toxin A (BTX-A) for treating RP. METHODS: Eleven patients with 20 affected hands diagnosed with RP were recruited and randomly assigned to receive either HDFG combined with BTX-A (intervention group, n = 11) or HDFG alone (control group, n = 9). Efficacy was assessed using Visual Analog Scale (VAS) pain scores and McCabe Cold Sensitivity Scores, along with finger ulcer healing time and infrared thermal imaging to evaluate blood perfusion improvements. RESULTS: The HDFG-BTX group showed significant improvements in hand symptoms. VAS pain scores decreased from a pre-treatment mean of 5.33 to 0.84 post-treatment (mean reduction of 4.49, p = 0.018), indicating effective pain relief. McCabe scores improved from 272.73 to 75.00 (mean reduction of 197.73, p = 0.001), demonstrating reduced cold sensitivity. Ulcer healing time was shorter in the HDFG-BTX group (14.25 days) compared to HDFG alone (25.6 days, p < 0.001), highlighting faster recovery. Infrared imaging indicated significant enhancements in blood perfusion. CONCLUSION: HDFG combined with BTX-A is a reliable and beneficial intervention for RP, leading to high patient satisfaction. CI - © 2025. The Author(s). FAU - Deng, Chengliang AU - Deng C AD - Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. FAU - Liu, Xin AU - Liu X AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. FAU - Wei, Miaomiao AU - Wei M AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. FAU - Wu, Bihua AU - Wu B AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. FAU - Zhang, Tianhua AU - Zhang T AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. FAU - Xiao, Shune AU - Xiao S AD - Department of Burns and Plastic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China. AD - The Collaborative Innovation Center of Tissue Damage Repair and Regeneration Medicine, Zunyi Medical University, Zunyi, China. FAU - Min, Peiru AU - Min P AD - Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. aru_ren@msn.com. FAU - Zhang, Yixin AU - Zhang Y AD - Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. zhangyixin2288@163.com. LA - eng GR - ZK2021-011/The key projects of science and technology plan of guizhou province/ GR - 2020-39/the Collaborative Innovation Center of Chinese Ministry of Education/ GR - rc220211202/the Program for Future Famous Clinical Doctors of Zunyi Medical University/ PT - Journal Article PT - Randomized Controlled Trial DEP - 20250319 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Neuromuscular Agents) SB - IM EIN - Arthritis Res Ther. 2025 Apr 22;27(1):92. doi: 10.1186/s13075-025-03556-1. PMID: 40264237 MH - Humans MH - *Raynaud Disease/therapy MH - Female MH - Male MH - Middle Aged MH - Adult MH - Prospective Studies MH - *Adipose Tissue/transplantation MH - *Botulinum Toxins, Type A/therapeutic use/administration & dosage MH - Treatment Outcome MH - Combined Modality Therapy MH - Cohort Studies MH - Pain Measurement MH - Stromal Cells/transplantation MH - *Neuromuscular Agents/therapeutic use PMC - PMC11921646 OTO - NOTNLM OT - Adipose-derived stromal cells OT - Botulinum toxins type A OT - Fat grafting OT - Raynaud’s phenomenon COIS- Declarations. Ethics approval and consent to participate: The study was conducted in compliance with all relevant national regulations and institutional policies and was approved by the Clinical Research Ethics Committee of the Affiliated Hospital of Zunyi Medical University (No.20210202). Furthermore, informed consent forms were signed by all participants to ensure their voluntary participation in the study. Consent for publication: Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient. Competing interests: The authors declare no competing interests. EDAT- 2025/03/20 06:24 MHDA- 2025/03/20 06:25 PMCR- 2025/03/19 CRDT- 2025/03/20 00:51 PHST- 2024/08/21 00:00 [received] PHST- 2025/03/11 00:00 [accepted] PHST- 2025/03/20 06:25 [medline] PHST- 2025/03/20 06:24 [pubmed] PHST- 2025/03/20 00:51 [entrez] PHST- 2025/03/19 00:00 [pmc-release] AID - 10.1186/s13075-025-03533-8 [pii] AID - 3533 [pii] AID - 10.1186/s13075-025-03533-8 [doi] PST - epublish SO - Arthritis Res Ther. 2025 Mar 19;27(1):56. doi: 10.1186/s13075-025-03533-8. PMID- 22052658 OWN - NLM STAT- MEDLINE DCOM- 20120719 LR - 20240318 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 64 IP - 3 DP - 2012 Mar TI - Validation of potential classification criteria for systemic sclerosis. PG - 358-67 LID - 10.1002/acr.20684 [doi] AB - OBJECTIVE: Classification criteria for systemic sclerosis (SSc; scleroderma) are being updated jointly by the American College of Rheumatology and European League Against Rheumatism. Potential items for classification were reduced to 23 using Delphi and nominal group techniques. We evaluated the face, discriminant, and construct validity of the items to be further studied as potential criteria. METHODS: Face validity was evaluated using the frequency of items in patients sampled from the Canadian Scleroderma Research Group, 1000 Faces of Lupus, and the Pittsburgh, Toronto, Madrid, and Berlin connective tissue disease (CTD) databases. Patients with SSc (n = 783) were compared to 1,071 patients with diseases similar to SSc (mimickers): systemic lupus erythematosus (n = 499), myositis (n = 171), Sjögren's syndrome (n = 95), Raynaud's phenomenon (RP; n = 228), mixed CTD (n = 29), and idiopathic pulmonary arterial hypertension (PAH; n = 49). Discriminant validity was evaluated using odds ratios (ORs). For construct validity, empirical ranking was compared to expert ranking. RESULTS: Compared to mimickers, patients with SSc were more likely to have skin thickening (OR 427); telangiectasias (OR 91); anti-RNA polymerase III antibody (OR 75); puffy fingers (OR 35); finger flexion contractures (OR 29); tendon/bursal friction rubs (OR 27); anti-topoisomerase I antibody (OR 25); RP (OR 24); fingertip ulcers/pitting scars (OR 19); anticentromere antibody (OR 14); abnormal nailfold capillaries (OR 10); gastroesophageal reflux disease symptoms (OR 8); antinuclear antibody, calcinosis, dysphagia, and esophageal dilation (all OR 6); interstitial lung disease/pulmonary fibrosis (OR 5); and anti-PM-Scl antibody (OR 2). Reduced carbon monoxide diffusing capacity, PAH, and reduced forced vital capacity had ORs of <2. Renal crisis and digital pulp loss/acroosteolysis did not occur in SSc mimickers (OR not estimated). Empirical and expert ranking were correlated (Spearman's ρ = 0.53, P = 0.01). CONCLUSION: The candidate items have good face, discriminant, and construct validity. Further item reduction will be evaluated in prospective SSc and mimicker cases. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Johnson, Sindhu R AU - Johnson SR AD - Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, Ontario, Canada. Sindhu.Johnson@uhn.on.ca FAU - Fransen, Jaap AU - Fransen J FAU - Khanna, Dinesh AU - Khanna D FAU - Baron, Murray AU - Baron M FAU - van den Hoogen, Frank AU - van den Hoogen F FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Peschken, Christine A AU - Peschken CA FAU - Carreira, Patricia E AU - Carreira PE FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Tyndall, Alan AU - Tyndall A FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M FAU - Pope, Janet E AU - Pope JE LA - eng GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - CAPMC/CIHR/Canada GR - K23-AR053858-05/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Autoantibodies) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adult MH - Autoantibodies/immunology MH - Cohort Studies MH - DNA Topoisomerases, Type I/immunology MH - Humans MH - Raynaud Disease/classification/diagnosis/immunology MH - Reproducibility of Results MH - Scleroderma, Systemic/*classification/diagnosis/immunology MH - Sjogren's Syndrome/classification/diagnosis/immunology PMC - PMC3376721 MID - NIHMS380995 COIS- DISCLOSURES Sindhu R. Johnson has no financial or other conflicts of interest in relation to this manuscript. Jaap Fransen has no financial or other conflicts of interest in relation to this manuscript. Dinesh Khanna has no financial or other conflicts of interest in relation to this manuscript. Murray Baron has no financial or other conflicts of interest in relation to this manuscript. Frank van den Hoogen has no financial or other conflicts of interest in relation to this manuscript. Thomas A. Medsger Jr. has no financial or other conflicts of interest in relation to this manuscript. Christine A. Peschken has no financial or other conflicts of interest in relation to this manuscript. Patricia E. Carreira has no financial or other conflicts of interest in relation to this manuscript. Gabriela Riemekasten has no financial or other conflicts of interest in relation to this manuscript. Alan Tyndall has no financial or other conflicts of interest in relation to this manuscript. Marco Matucci-Cerinic has no financial or other conflicts of interest in relation to this manuscript. Janet E. Pope has no financial or other conflicts of interest in relation to this manuscript. EDAT- 2011/11/05 06:00 MHDA- 2012/07/20 06:00 PMCR- 2013/03/01 CRDT- 2011/11/05 06:00 PHST- 2011/11/05 06:00 [entrez] PHST- 2011/11/05 06:00 [pubmed] PHST- 2012/07/20 06:00 [medline] PHST- 2013/03/01 00:00 [pmc-release] AID - 10.1002/acr.20684 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 Mar;64(3):358-67. doi: 10.1002/acr.20684. PMID- 36207966 OWN - NLM STAT- MEDLINE DCOM- 20221011 LR - 20221011 IS - 0578-1426 (Print) IS - 0578-1426 (Linking) VI - 61 IP - 10 DP - 2022 Oct 1 TI - [Standardized diagnosis and treatment of undifferentiated connective tissue disease and mixed connective tissue disease]. PG - 1119-1127 LID - 10.3760/cma.j.cn112138-20220104-00009 [doi] AB - Undifferentiated connective tissue disease (CTD) usually refers to patients who are presented with certain symptoms and signs related to CTD, and positive serological evidence of autoimmune diseases but don't fulfill any of the classification criteria for a certain CTD. Mixed CTD refers to patients who are presented with one or more clinical manifestations such as hand swelling, synovitis, myositis, Raynaud's phenomenon, and acrosclerosis. Patients with mixed CTD always have high-titer anti-nuclear antibodies (ANA) of speckled pattern and high-titer anti-U(1) ribonuclear protein (RNP) antibody in serum, while with negative anti-Sm antibody. The update of diagnosis and treatment of undifferentiated CTD and mixed CTD lags behind other established CTD. There is a lack of evidence from randomized controlled trials or guidelines/recommendations on the treatment of undifferentiated CTD or mixed CTD. At present, the conventional therapy is mainly adopted according to the specific clinical manifestations of the disease. The standardized diagnosis and treatment of undifferentiated CTD and mixed CTD were drafted by the Chinese Rheumatology Association based on the previous guidelines and the progress of available evidence, so as to improve the management of these patients in China. FAU - Mo, Y Q AU - Mo YQ AD - Department of Rheumatology, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou 510120, China. FAU - Yan, Q AU - Yan Q AD - Department of Rheumatology and Immunology, Fujian Provincial Hospital, Shengli Clinical Medical College of Fujian Medical University, Fuzhou 350001, China. FAU - Ye, S AU - Ye S AD - Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200001, China. FAU - Dai, L AU - Dai L AD - Department of Rheumatology, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou 510120, China. FAU - Zhao, Yan AU - Zhao Y AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. LA - chi PT - Journal Article PL - China TA - Zhonghua Nei Ke Za Zhi JT - Zhonghua nei ke za zhi JID - 16210490R RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear MH - *Connective Tissue Diseases/diagnosis/therapy MH - Humans MH - *Mixed Connective Tissue Disease/diagnosis/therapy MH - *Raynaud Disease/diagnosis MH - *Undifferentiated Connective Tissue Diseases/diagnosis EDAT- 2022/10/09 06:00 MHDA- 2022/10/12 06:00 CRDT- 2022/10/08 02:28 PHST- 2022/10/08 02:28 [entrez] PHST- 2022/10/09 06:00 [pubmed] PHST- 2022/10/12 06:00 [medline] AID - 10.3760/cma.j.cn112138-20220104-00009 [doi] PST - ppublish SO - Zhonghua Nei Ke Za Zhi. 2022 Oct 1;61(10):1119-1127. doi: 10.3760/cma.j.cn112138-20220104-00009. PMID- 29237099 OWN - NLM STAT- MEDLINE DCOM- 20180126 LR - 20250623 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 12 IP - 12 DP - 2017 Dec 13 TI - Calcium channel blockers for primary and secondary Raynaud's phenomenon. PG - CD000467 LID - 10.1002/14651858.CD000467.pub2 [doi] LID - CD000467 AB - BACKGROUND: Raynaud's phenomenon is a vasospastic disease characterized by digital pallor, cyanosis, and extremity pain. Primary Raynaud's phenomenon is not associated with underlying disease, but secondary Raynaud's phenomenon is associated with connective tissue disorders such as systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. Calcium channel blockers promote vasodilation and are commonly used when drug treatment for Raynaud's phenomenon is required. OBJECTIVES: To assess the benefits and harms of calcium channel blockers (CCBs) versus placebo for treatment of individuals with Raynaud's phenomenon with respect to Raynaud's type (primary vs secondary) and type and dose of CCBs. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (May 19, 2017), MEDLINE (1946 to May 19, 2017), Embase (1947 to May 19, 2017), clinicaltrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Portal. We applied no language restrictions. We also searched bibliographies of retrieved articles and contacted key experts for additional and unpublished data. SELECTION CRITERIA: All randomized controlled trials (RCTs) comparing calcium channel blockers versus placebo. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed search results and risk of bias and extracted trial data. We used the GRADE approach to assess the quality of evidence. MAIN RESULTS: This review contains 38 RCTs (33 cross-over RCTs) with an average duration of 7.4 weeks and 982 participants; however, not all trials reported all outcomes of interest. Nine of the identified trials studied patients with primary Raynaud's phenomenon (N = 365), five studied patients with secondary Raynaud's phenomenon (N = 63), and the rest examined a mixture of patients with primary and secondary Raynaud's phenomenon (N = 554). The most frequently encountered risk of bias types were incomplete outcome data and poor reporting of randomization and allocation methods.When researchers considered both primary and secondary Raynaud's phenomenon, evidence of moderate quality (downgraded for inconsistency) from 23 trials with 528 participants indicates that calcium channel blockers (CCBs) were superior to placebo in reducing the frequency of attacks. CCBs reduced the average number of attacks per week by six ( weighted mean difference (WMD) -6.13, 95% confidence interval (CI) -6.60 to - 5.67; I² = 98%) compared with 13.7 attacks per week with placebo. When review authors excluded Kahan 1985C, a trial showing a very large reduction in the frequency of attacks, data showed that CCBs reduced attack frequency by 2.93 per week (95% CI -3.44 to -2.43; I² = 77%).Low-quality evidence (downgraded for imprecision and inconsistency) from six trials with 69 participants suggests that the average duration of attacks did not differ in a statistically significant or clinically meaningful way between CCBs and placebo (WMD -1.67 minutes, 95% CI -3.29 to 0); this is equivalent to a -9% difference (95% CI -18% to 0%).Moderate-quality evidence (downgraded for inconsistency) based on 16 trials and 415 participants showed that CCBs reduced attack severity by 0.62 cm (95% CI -0.72 to - 0.51) on a 10-cm visual analogue scale (lower scores indicate less severity); this was equivalent to absolute and relative percent reductions of 6% (95% CI -11% to -8%) and 9% (95% CI -11% to -8%), respectively, which may not be clinically meaningful.Improvement in Raynaud's pain (low-quality evidence; downgraded for imprecision and inconsistency) and in disability as measured by a patient global assessment (moderate-quality evidence; downgraded for imprecision) favored CCBs (pain: WMD -1.47 cm, 95% CI -2.21 to -0.74; patient global: WMD -0.37 cm, 95% CI -0.73 to 0, when assessed on a 0 to 10 cm visual analogue scale, with lower scores indicating less pain and less disability). However, these effect estimates were likely underpowered, as they were based on limited numbers of participants, respectively, 62 and 92. For pain assessment, absolute and relative percent improvements were 15% (95% -22% to -7%) and 47% (95% CI -71% to -24%), respectively. For patient global assessment, absolute and relative percent improvements were 4% (95% CI -7% to 0%) and 9% (95% CI -19% to 0%), respectively.Subgroup analyses by Raynaud's type, CCB class, and CCB dose suggest that dihydropyridine CCBs in higher doses may be more effective for primary Raynaud's than for secondary Raynaud's, and CCBs likely have a greater effect in primary than in secondary Raynaud's. However, differences were small and were not found for all outcomes. Dihydropyridine CCBs were studied as they are the subgroup of CCBs that are not cardioselective and are traditionally used in RP treatment whereas other CCBs such as verapamil are not routinely used and diltiazem is not used as first line subtype of CCBs. Most trial data pertained to nifedipine.Withdrawals from studies due to adverse effects were inconclusive owing to a wide CI (risk ratio [RR] 1.30, 95% CI 0.51 to 3.33) from two parallel studies with 63 participants (low-quality evidence downgraded owing to imprecision and a high attrition rate); absolute and relative percent differences in withdrawals were 6% (95% CI -14% to 26%) and 30% (95% CI -49% to 233%), respectively. In cross-over trials, although a meta-analysis was not performed, withdrawals were more common with CCBs than with placebo. The most common side effects were headache, dizziness, nausea, palpitations, and ankle edema. However, in all trials, no serious adverse events (death or hospitalization) were reported. AUTHORS' CONCLUSIONS: Randomized controlled trials with evidence of low to moderate quality showed that CCBs (especially the dihydropyridine class) may be useful in reducing the frequency, duration, severity of attacks, pain and disability associated with Raynaud's phenomenon. Higher doses may be more effective than lower doses and these CCBs may be more effective in primary RP. Although there were more withdrawals due to adverse events in the treatment groups, no serious adverse events were reported. FAU - Rirash, Fadumo AU - Rirash F AD - Department of Medicine, University of Western Ontario, 268 Grosvenor Street, London, ON, Canada, N6A 4V2. FAU - Tingey, Paul C AU - Tingey PC FAU - Harding, Sarah E AU - Harding SE FAU - Maxwell, Lara J AU - Maxwell LJ FAU - Tanjong Ghogomu, Elizabeth AU - Tanjong Ghogomu E FAU - Wells, George A AU - Wells GA FAU - Tugwell, Peter AU - Tugwell P FAU - Pope, Janet AU - Pope J LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20171213 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Calcium Channel Blockers) RN - 0 (Dihydropyridines) RN - 0 (Placebos) RN - 7M8K3P6I89 (1,4-dihydropyridine) RN - I9ZF7L6G2L (Nifedipine) SB - IM UOF - doi: 10.1002/14651858.CD000467 MH - Calcium Channel Blockers/administration & dosage/*therapeutic use MH - Dihydropyridines/administration & dosage/therapeutic use MH - Humans MH - Nifedipine/administration & dosage/therapeutic use MH - Pain Management MH - Placebos/therapeutic use MH - Publication Bias MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy/etiology MH - Severity of Illness Index PMC - PMC6486273 COIS- JP: has consulted for Actelion, Mediquest, Pfizer, and United Therapeutics in the area of Raynaud's phenomenon and/or digital ulcers. PTu: grants/honoraria from Bristol Myers and UCB. FR: none known. PTi: none known. SH: none known. LM: none known. EG: none known. GW: none known. EDAT- 2017/12/14 06:00 MHDA- 2018/01/27 06:00 PMCR- 2018/12/13 CRDT- 2017/12/14 06:00 PHST- 2017/12/14 06:00 [pubmed] PHST- 2018/01/27 06:00 [medline] PHST- 2017/12/14 06:00 [entrez] PHST- 2018/12/13 00:00 [pmc-release] AID - CD000467.pub2 [pii] AID - 10.1002/14651858.CD000467.pub2 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2017 Dec 13;12(12):CD000467. doi: 10.1002/14651858.CD000467.pub2. PMID- 24564981 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - The association of antiphospholipid antibodies with cardiopulmonary manifestations of systemic sclerosis. PG - S-133-7 AB - OBJECTIVES: To determine the prevalence and correlates of antiphospholipid antibodies (APLA) in systemic sclerosis (SSc). METHODS: Nine hundred and forty SSc patients were tested for APLA using an ELISA assay at recruitment. Clinical manifestations were defined as present, if ever present from SSc diagnosis. Logistic regression analysis was used to determine the associations of APLA. RESULTS: One or more types of APLA were present in 226 (24.0%) patients. Anticardiolipin (ACA) IgG (ACA-IgG) antibodies were associated with right heart catheter-diagnosed pulmonary arterial hypertension (PAH), with higher titres corresponding with a higher likelihood of PAH (moderate titre (20-39 U/ml) ACA-IgG odds ratio [OR] 1.70, 95% CI: 1.01-2.93, p=0.047; high titre (>40 U/ml) ACA-IgG OR 4.60, 95% CI:1.02-20.8, p=0.047). Both ACA-IgM (OR 2.04, 95% CI: 1.4-3.0, p<0.0001) and ACA-IgG (OR 1.84, 95% CI: 1.2-2.8, p=0.005) were associated with interstitial lung disease (ILD). Increasing ACA-IgM and IgG titres were associated with increased likelihood of ILD. ACA-IgG was a marker of coexistent pulmonary hypertension and ILD (ILD-PH) (OR 2.10, 95% CI: 1.1-4.2, p=0.036). We also found an association between ACA-IgG and digital ulcers (OR 1.76, 95% CI: 1.16-2.67, p=0.008) and ACA-IgM and Raynaud's phenomenon (OR 2.39, 95% CI: 1.08-5.27, p=0.031). There was no association between APLA and SSc disease subtype, peak skin score, presence of other autoantibodies, mortality or other disease manifestations. CONCLUSIONS: The association of APLA with PAH, ILD, ILD-PH, Raynaud's phenomenon and digital ulcers suggests that endothelial abnormalities and small vessel thrombosis may be important in the pathogenesis of these disease features. FAU - Morrisroe, K B AU - Morrisroe KB AD - Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia. kbmorrisroe@gmail.com. FAU - Stevens, W AU - Stevens W FAU - Nandurkar, H AU - Nandurkar H FAU - Prior, D AU - Prior D FAU - Thakkar, V AU - Thakkar V FAU - Roddy, J AU - Roddy J FAU - Zochling, J AU - Zochling J FAU - Sahhar, J AU - Sahhar J FAU - Tymms, K AU - Tymms K FAU - Sturgess, A AU - Sturgess A FAU - Major, G AU - Major G FAU - Kermeen, F AU - Kermeen F FAU - Hill, C AU - Hill C FAU - Walker, J AU - Walker J FAU - Nash, P AU - Nash P FAU - Gabbay, E AU - Gabbay E FAU - Youssef, P AU - Youssef P FAU - Proudman, S M AU - Proudman SM FAU - Nikpour, M AU - Nikpour M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140224 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) SB - IM MH - Aged MH - Antibodies, Anticardiolipin/*immunology MH - Antibodies, Antiphospholipid/immunology MH - Cohort Studies MH - Female MH - Hand Dermatoses/etiology/immunology MH - Heart Diseases/etiology/*immunology MH - Humans MH - Hypertension, Pulmonary/etiology/*immunology MH - Immunoglobulin G/immunology MH - Immunoglobulin M/immunology MH - Logistic Models MH - Lung Diseases, Interstitial/etiology/*immunology MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/etiology/immunology MH - Scleroderma, Systemic/complications/*immunology MH - Skin Ulcer/etiology/immunology EDAT- 2014/02/26 06:00 MHDA- 2015/10/31 06:00 CRDT- 2014/02/26 06:00 PHST- 2013/07/30 00:00 [received] PHST- 2013/11/15 00:00 [accepted] PHST- 2014/02/26 06:00 [entrez] PHST- 2014/02/26 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 7423 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-133-7. Epub 2014 Feb 24. PMID- 20824423 OWN - NLM STAT- MEDLINE DCOM- 20110315 LR - 20211020 IS - 1432-2102 (Electronic) IS - 0033-832X (Linking) VI - 50 IP - 10 DP - 2010 Oct TI - [Angiographic diagnosis of acral circulatory disorders of the upper extremities]. PG - 879-86 LID - 10.1007/s00117-010-2005-x [doi] AB - Acral ischemic lesions rarely affect the upper extremities. While in the lower limbs atherosclerosis is responsible for the majority of lesions, vasculitis and autoimmune diseases play an important role in the pathogenesis of ischemic lesions of the upper limbs. A considerable number of acral circulatory disorders present with Raynaud's phenomenon and often without associated necrosis. Raynaud's phenomenon is mainly idiopathic but may also be secondary to underlying conditions, such as autoimmune diseases and vasculitis. Because of its high spatial resolution and the often discrete morphological findings digital subtraction angiography (DSA) is still an important diagnostic method in the radiological evaluation of acral circulatory disorders of the hand. Angiographic features of vasculitis are not strictly pathognomonic but certain morphologic DSA findings are very typical and may allow for a radiologic diagnosis. For instance, atherosclerosis results in irregular contours of vessel walls in DSA in contrast to autoimmune diseases and vasculitis, which are usually characterized by smooth vessel walls and optional vasospasm, the latter being especially typical for thromboangiitis obliterans and scleroderma. In thromboangiitis obliterans occlusions of the distal hand arteries, corkscrew collateral vessels and subsequent development of fine collateral networks are typical findings. Abrupt or filiform occlusions of distal finger arteries with sparse collateralization and symmetric affection of both hands are suggestive of scleroderma. Disseminated segmental ectasis and stenosis as well as microaneurysms (63% of all patients) are very common in patients with panarteriitis nodosa. FAU - Deák, Z AU - Deák Z AD - Institut für Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universität München, Campus Innenstadt, Pettenkoferstr. 8a, 80336, München, Deutschland. zsuzsanna.deak@med.uni-muenchen.de FAU - Treitl, M AU - Treitl M FAU - Reiser, M F AU - Reiser MF FAU - Degenhart, C AU - Degenhart C LA - ger PT - Journal Article PT - Review TT - Angiographische Diagnostik akraler Durchblutungsstörungen an der oberen Extremität. PL - Germany TA - Radiologe JT - Der Radiologe JID - 0401257 SB - IM MH - *Angiography MH - *Angiography, Digital Subtraction MH - Arm/*blood supply MH - Arthritis, Rheumatoid/diagnostic imaging MH - Fingers/blood supply MH - Humans MH - Ischemia/*diagnostic imaging MH - Lupus Erythematosus, Systemic/diagnostic imaging MH - Polyarteritis Nodosa/diagnostic imaging MH - Raynaud Disease/diagnostic imaging MH - Thromboangiitis Obliterans/diagnostic imaging MH - Vasculitis/*diagnostic imaging EDAT- 2010/09/09 06:00 MHDA- 2011/03/16 06:00 CRDT- 2010/09/09 06:00 PHST- 2010/09/09 06:00 [entrez] PHST- 2010/09/09 06:00 [pubmed] PHST- 2011/03/16 06:00 [medline] AID - 10.1007/s00117-010-2005-x [doi] PST - ppublish SO - Radiologe. 2010 Oct;50(10):879-86. doi: 10.1007/s00117-010-2005-x. PMID- 18208429 OWN - NLM STAT- MEDLINE DCOM- 20090723 LR - 20090615 IS - 1600-0838 (Electronic) IS - 0905-7188 (Linking) VI - 18 IP - 5 DP - 2008 Oct TI - The Enduro motorcyclist's wrist and other overuse injuries in competitive Enduro motorcyclists: a prospective study. PG - 582-90 LID - 10.1111/j.1600-0838.2007.00701.x [doi] AB - According to public opinion, Enduro motorcycling is a dangerous sport. Little is known about overuse injuries in connection with this sport. The study consisted of two phases. Phase 1 was planned as a prospective feasibility study for phase 2. The latter was initiated as a prospective investigation, comprising a random sample of the participants of phase 1. Enduro motorcyclists were interviewed with a prepared questionnaire. Clinical examinations were performed immediately before, and after, a major Enduro race, including pain assessment in the hand/wrist and the forearm using the visual analog scale. The occurrence of Raynaud's syndrome carpal tunnel syndrome (CTS), and loss of grip strength in the forearm muscles were recorded in detail. Phase 1 showed that the predominant overused anatomical regions were the hand/wrist and forearms. Nearly 50% of all the riders complained of pain or paresthetic sensations. A significant increase in pain was registered after the race. After the second run 32.28% of all riders had CTS in the left hand and 43.31% in the right hand. More than one-half of the athletes complained of overuse injuries. Transient CTS is an overuse injury closely related to sports. FAU - Sabeti-Aschraf, M AU - Sabeti-Aschraf M AD - Department for Orthopaedics and Orthopaedic Surgery AKH-Wien, Vienna Medical School, Vienna, Austria. manuel.sabeti-aschraf@meduniwien.ac.at FAU - Serek, M AU - Serek M FAU - Pachtner, T AU - Pachtner T FAU - Auner, K AU - Auner K FAU - Machinek, M AU - Machinek M FAU - Geisler, M AU - Geisler M FAU - Goll, A AU - Goll A LA - eng PT - Journal Article DEP - 20080114 PL - Denmark TA - Scand J Med Sci Sports JT - Scandinavian journal of medicine & science in sports JID - 9111504 SB - IM MH - Adult MH - Athletic Injuries/*epidemiology MH - Austria/epidemiology MH - Carpal Tunnel Syndrome/*epidemiology MH - Competitive Behavior MH - Cumulative Trauma Disorders/*epidemiology MH - Forearm Injuries/epidemiology MH - Hand Strength MH - Humans MH - Male MH - *Motorcycles/statistics & numerical data MH - Pain Measurement MH - Prospective Studies MH - Raynaud Disease/*epidemiology MH - Young Adult EDAT- 2008/01/23 09:00 MHDA- 2009/07/25 09:00 CRDT- 2008/01/23 09:00 PHST- 2008/01/23 09:00 [pubmed] PHST- 2009/07/25 09:00 [medline] PHST- 2008/01/23 09:00 [entrez] AID - SMS701 [pii] AID - 10.1111/j.1600-0838.2007.00701.x [doi] PST - ppublish SO - Scand J Med Sci Sports. 2008 Oct;18(5):582-90. doi: 10.1111/j.1600-0838.2007.00701.x. Epub 2008 Jan 14. PMID- 17057632 OWN - NLM STAT- MEDLINE DCOM- 20070504 LR - 20220409 IS - 0761-8425 (Print) IS - 0761-8425 (Linking) VI - 23 IP - 4 Suppl DP - 2006 Sep TI - [Pulmonary arterial hypertension in connective tissue disease]. PG - 13S61-72 AB - INTRODUCTION: Pulmonary arterial hypertension (PAH) is one of the most severe complications of connective tissue diseases. PAH is defined by mean arterial pulmonary pressure > 25 mmHg at rest (or 30 mmHg during exercise) during right heart catheterisation. STATE OF THE ART: About 10% of cases of PAH are related to connective tissue diseases. PAH may be observed in any of the connective tissue diseases; it is mostly encountered in systemic sclerosis (prevalence estimated to 8%), and is more frequent in the limited form of the disease with anticentromeres antibodies, in the presence of Raynaud's phenomenon, of antinuclear antibodies, decreased CO diffusion capacity, and during the perimenopausal period. Histopathological lesions are similar to that of idiopathic PAH (with plexogenic lesions being more rare); mutations have not been described. Venous thromboembolic disease (especially in lupus) and hypoxemia related to pulmonary fibrosis should be investigated. Screening for PAH is recommended in systemic sclerosis (by echocardiography every year and in case of dyspnea). Few clinical trials have been conducted specifically in PAH related to connective tissue diseases, therefore the treatment approach is similar to that of idiopathic PAH, with a poorer prognosis, including treatment with bosentan, epoprostenol and other prostacyclin analogs, and sildenafil. Immunosuppressive therapy may occasionally improve PAH in patients with systemic erythematosus lupus or mixed connective tissue disease. PERSPECTIVES: Respective indications of drugs and treatment associations need to be precised. CONCLUSION: The availability of efficacious treatments of PAH justifies its screening and early diagnosis in connective tissue diseases. FAU - Cottin, V AU - Cottin V AD - Service de Pneumologie, Centre de Référence des Maladies Orphelines Pulmonaires, Hôpital Louis Pradel, Hospices Civils de Lyon, Université de Lyon, UMR 754 INRA-ENVL-UCBL-IFR128, Lyon, France. vincent.cottin@chu-lyon.fr LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Hypertension artérielle pulmonaire associée aux connectivites. PL - France TA - Rev Mal Respir JT - Revue des maladies respiratoires JID - 8408032 RN - 0 (Antihypertensive Agents) RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Autoantibodies/analysis MH - Connective Tissue Diseases/*complications/immunology MH - Humans MH - Hypertension, Pulmonary/drug therapy/*etiology/immunology MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/complications MH - Mixed Connective Tissue Disease/complications MH - Prognosis MH - Pulmonary Diffusing Capacity/physiology MH - Pulmonary Fibrosis/complications MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications MH - Thromboembolism/complications RF - 88 EDAT- 2006/10/24 09:00 MHDA- 2007/05/05 09:00 CRDT- 2006/10/24 09:00 PHST- 2006/10/24 09:00 [pubmed] PHST- 2007/05/05 09:00 [medline] PHST- 2006/10/24 09:00 [entrez] AID - MDOI-RMR-09-2006-23-SUP4-0761-8425-101019-20064119 [pii] PST - ppublish SO - Rev Mal Respir. 2006 Sep;23(4 Suppl):13S61-72. PMID- 16636936 OWN - NLM STAT- MEDLINE DCOM- 20070426 LR - 20211020 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 3 DP - 2007 Mar TI - The natural course of progressive systemic sclerosis patients with interstitial lung involvement. PG - 349-54 AB - OBJECTIVE: The aim of the study is to assess the natural course of systemic sclerosis (SSc) in patients with interstitial lung involvement and to evaluate the effects of treatment. MATERIALS AND METHODS: Sixty SSc patients with interstitial lung involvement were included in the study and history and retrospective data records of the patients were reviewed. RESULTS: It was observed that 47 patients (78.3%) had Raynaud's phenomenon, 7 patients (11.7%) had skin involvement, 5 patients (8.3%) had joint involvement, and 1 patient (1.7%) had gastrointestinal system involvement as the first manifestation of the disease. Lung involvement had developed on an average of 113 +/- 106 months after the first manifestation of the disease and was apparent within the first year in 10 patients (16.7%), between 1 and 2 years in 3 patients (5%), between 2 and 3 years in 2 patients (3.3%), between 3 and 4 years in 5 patients (8.3%), between 4 and 10 years in 21 patients (35%), and after 10 years in 19 patients (31.7%). When the symptoms of lung involvement appeared, 37 patients (61.7%) were not receiving treatment while 23 (38.3%) were using an immunosuppressive agent. The time interval of lung involvement for the treated patients was 131 +/- 95 months while it was 101 +/- 112 months in untreated patients (p>0.05). CONCLUSION: In SSc patients with interstitial pulmonary involvement, the disease frequently starts with Raynaud's phenomenon and pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease. The first sign of the disease, the probability of interstitial pulmonary involvement, is highest during the first 15 years after. Although this probability decreases after 15 years, in up to 10% of the patients, interstitial pulmonary involvement can still occur even up to 40 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years. FAU - Benan, Musellim AU - Benan M AD - Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. FAU - Hande, Ikitimur AU - Hande I FAU - Gul, Ongen AU - Gul O LA - eng PT - Journal Article DEP - 20060425 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Aged MH - Disease Progression MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/*etiology MH - Male MH - Middle Aged MH - Raynaud Disease/*immunology MH - Retrospective Studies MH - Scleroderma, Diffuse/*complications/drug therapy EDAT- 2006/04/26 09:00 MHDA- 2007/04/27 09:00 CRDT- 2006/04/26 09:00 PHST- 2006/02/03 00:00 [received] PHST- 2006/04/03 00:00 [accepted] PHST- 2006/03/26 00:00 [revised] PHST- 2006/04/26 09:00 [pubmed] PHST- 2007/04/27 09:00 [medline] PHST- 2006/04/26 09:00 [entrez] AID - 10.1007/s10067-006-0302-6 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Mar;26(3):349-54. doi: 10.1007/s10067-006-0302-6. Epub 2006 Apr 25. PMID- 36548367 OWN - NLM STAT- MEDLINE DCOM- 20221226 LR - 20230113 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 17 IP - 12 DP - 2022 TI - Aptamer proteomics of serum exosomes from patients with Primary Raynaud's and patients with Raynaud's at risk of evolving into Systemic Sclerosis. PG - e0279461 LID - 10.1371/journal.pone.0279461 [doi] LID - e0279461 AB - BACKGROUND: A major unmet need for Systemic Sclerosis (SSc) clinical management is the lack of biomarkers for the early diagnosis of patients with Raynaud's Phenomenon at high risk of evolving into SSc. OBJECTIVE: To identify proteins contained within serum exosomes employing an aptamer proteomic analysis that may serve to reveal patients with Raynaud's Phenomenon at risk of developing SSc. METHODS: Exosomes were isolated from serum samples from patients with Primary Raynaud's Phenomenon and from patients with Raynaud's Phenomenon harbouring serum antinuclear antibodies (ANA) who may be at high risk of evolving into SSc. The expression of 1,305 proteins was quantified using SOMAscan aptamer proteomics, and associations of the differentially elevated or reduced proteins with the clinical subsets of Raynaud's Phenomenon were assessed. RESULTS: Twenty one differentially elevated and one differentially reduced (absolute fold change >|1.3|) proteins were identified. Principal component analysis using these 22 most differentially expressed proteins resulted in excellent separation of the two Raynaud's Phenomenon clinical subsets. Remarkably, the most differentially elevated proteins are involved in enhanced inflammatory responses, immune cell activation and cell migration, and abnormal vascular functions. CONCLUSION: Aptamer proteomic analysis of circulating exosomes identified differentially elevated or reduced proteins between Raynaud's Phenomenon at high risk of evolving into SSc and Primary Raynaud's Phenomenon patients. Some of these proteins are involved in relevant biological pathways that may play a role in SSc pathogenesis including enhanced inflammatory responses, immune cell activation, and endothelial cell and vascular abnormalities. CI - Copyright: © 2022 Piera-Velazquez et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. FAU - Piera-Velazquez, Sonsoles AU - Piera-Velazquez S AD - Jefferson Institute of Molecular Medicine, Scleroderma Center of Thomas Jefferson University, Philadelphia, Pennsylvania, United States of America. FAU - Dillon, Simon T AU - Dillon ST AD - Division of Interdisciplinary Medicine and Biotechnology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Genomics, Proteomics, Bioinformatics and Systems Biology Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Harvard Medical School, Boston, Massachusetts, United States of America. FAU - Gu, Xuesong AU - Gu X AD - Division of Interdisciplinary Medicine and Biotechnology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Genomics, Proteomics, Bioinformatics and Systems Biology Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Harvard Medical School, Boston, Massachusetts, United States of America. FAU - Libermann, Towia A AU - Libermann TA AD - Division of Interdisciplinary Medicine and Biotechnology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Genomics, Proteomics, Bioinformatics and Systems Biology Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States of America. AD - Harvard Medical School, Boston, Massachusetts, United States of America. FAU - Jimenez, Sergio A AU - Jimenez SA AUID- ORCID: 0000-0001-5213-1203 AD - Jefferson Institute of Molecular Medicine, Scleroderma Center of Thomas Jefferson University, Philadelphia, Pennsylvania, United States of America. LA - eng PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20221222 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Exosomes MH - Proteomics MH - *Scleroderma, Systemic/complications MH - Biomarkers MH - *Raynaud Disease PMC - PMC9779033 COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. This does not alter our adherence to PLOS ONE policies on sharing data and materials. EDAT- 2022/12/23 06:00 MHDA- 2022/12/27 06:00 PMCR- 2022/12/22 CRDT- 2022/12/22 13:55 PHST- 2022/07/19 00:00 [received] PHST- 2022/12/07 00:00 [accepted] PHST- 2022/12/22 13:55 [entrez] PHST- 2022/12/23 06:00 [pubmed] PHST- 2022/12/27 06:00 [medline] PHST- 2022/12/22 00:00 [pmc-release] AID - PONE-D-22-20405 [pii] AID - 10.1371/journal.pone.0279461 [doi] PST - epublish SO - PLoS One. 2022 Dec 22;17(12):e0279461. doi: 10.1371/journal.pone.0279461. eCollection 2022. PMID- 20379818 OWN - NLM STAT- MEDLINE DCOM- 20130220 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 10 DP - 2012 Oct TI - Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome. PG - 3265-8 AB - The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud's phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma. FAU - Leite, Pollyanna d'Ávila AU - Leite Pd AD - Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3º anda, sala 3190, São Paulo 01246-903, Brazil. FAU - de Carvalho, Jozélio Freire AU - de Carvalho JF LA - eng PT - Case Reports PT - Journal Article DEP - 20100409 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Lupus Coagulation Inhibitor) RN - 0 (anticentromere antibody) SB - IM MH - Antibodies, Anticardiolipin/blood MH - Antibodies, Antinuclear/blood MH - Antiphospholipid Syndrome/blood/*complications/diagnosis/immunology/therapy MH - Biomarkers/blood MH - Capillaries/*pathology MH - Female MH - Humans MH - Lupus Coagulation Inhibitor/blood MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/etiology MH - Retinal Vein Occlusion/etiology MH - Scleroderma, Systemic/blood/diagnosis/*etiology/immunology/therapy EDAT- 2010/04/10 06:00 MHDA- 2013/02/21 06:00 CRDT- 2010/04/10 06:00 PHST- 2010/01/05 00:00 [received] PHST- 2010/03/27 00:00 [accepted] PHST- 2010/04/10 06:00 [entrez] PHST- 2010/04/10 06:00 [pubmed] PHST- 2013/02/21 06:00 [medline] AID - 10.1007/s00296-010-1482-6 [doi] PST - ppublish SO - Rheumatol Int. 2012 Oct;32(10):3265-8. doi: 10.1007/s00296-010-1482-6. Epub 2010 Apr 9. PMID- 16705051 OWN - NLM STAT- MEDLINE DCOM- 20070220 LR - 20151119 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 IP - 12 DP - 2006 Dec TI - Digital thermal hyperaemia impairment does not relate to skin fibrosis or macrovascular disease in systemic sclerosis. PG - 1490-6 AB - OBJECTIVES: Thermal hyperaemia is impaired in patients with systemic sclerosis (SSc). The objective of these studies was to determine whether this was consecutive to skin fibrosis, microangiopathy or macroangiopathy. METHODS: Using laser Doppler flowmetry, we first compared the thermal hyperaemia on the third left finger pad and on the left forearm in 21 patients with non-diffuse systemic sclerosis (SSc), in comparison with primary Raynaud's phenomenon and healthy volunteers. Second, we tested whether the altered thermal hyperaemia correlated to the digital pressure index at baseline, and following the thermal challenge. RESULTS: In the first study, thermal hyperaemia of the finger pad was impaired in terms of both amplitude and kinetics, but not on the forearm in patients with SSc. In the seven SSc patients without cutaneous fibrosis, the response was similarly altered in terms of amplitude and kinetics. In the second study, we observed a weak correlation between the digital systolic blood pressure index. However, in the 15 SSc patients tested at 44 degrees C, the median digital systolic blood pressure index was 1.04 (0.84-1.24) at baseline vs 1.08 (0.87-1.29) at 44 degrees C (NS), while seven of them had an abnormal response in terms of kinetic. Furthermore, only one patient showed a clear-cut decrease in digital systolic blood pressure at 44 degrees C. CONCLUSION: In patients with SSc, digital thermal hyperaemia is impaired, but does not relate to the skin fibrosis or to an associated macroangiopathy in most cases. Further studies are required to determine whether its impairment reflects a functional or structural microvascular damage. FAU - Salvat-Melis, M AU - Salvat-Melis M AD - Inserm ESPRI HP2 Laboratory, Grenoble Medical School, Grenoble University Hospital, Grenoble, France. FAU - Carpentier, P H AU - Carpentier PH FAU - Minson, C T AU - Minson CT FAU - Boignard, A AU - Boignard A FAU - McCord, G R AU - McCord GR FAU - Paris, A AU - Paris A FAU - Moreau-Gaudry, A AU - Moreau-Gaudry A FAU - Cracowski, J-L AU - Cracowski JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060516 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Blood Pressure MH - Female MH - Fibrosis/complications MH - Fingers/*blood supply MH - Forearm/blood supply MH - Hot Temperature MH - Humans MH - Hyperemia/*etiology/physiopathology MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/physiopathology MH - Scleroderma, Systemic/*complications/physiopathology MH - Skin/*pathology MH - Vasodilation EDAT- 2006/05/18 09:00 MHDA- 2007/02/21 09:00 CRDT- 2006/05/18 09:00 PHST- 2006/05/18 09:00 [pubmed] PHST- 2007/02/21 09:00 [medline] PHST- 2006/05/18 09:00 [entrez] AID - kel116 [pii] AID - 10.1093/rheumatology/kel116 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Dec;45(12):1490-6. doi: 10.1093/rheumatology/kel116. Epub 2006 May 16. PMID- 35780227 OWN - NLM STAT- MEDLINE DCOM- 20221018 LR - 20221018 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 11 DP - 2022 Nov TI - Clinical characteristics and outcomes of digital gangrene in connective tissue disorders: a longitudinal single-centre experience from Jodhpur, India. PG - 3543-3549 LID - 10.1007/s10067-022-06265-1 [doi] AB - Epidemiology, clinical presentation, and outcomes for digital gangrene in connective tissue disorders (CTD) remain underreported from tropical countries like India. In this series, we aimed to explore the clinical profile and outcomes of patients who presented with digital gangrene and a diagnosis of CTD. Hospital-based longitudinal observational study. Patients with digital gangrene and underlying diagnosis of CTD presenting to our tertiary-care centre in Jodhpur, India between1(st) January 2018 and 31(st) June 2021 were included. Clinical outcomes including mortality, limb outcomes, functional status and other systemic involvement were assessed. Of the 312 patients registered in the rheumatology clinic during this period, 22 (7%) patients were found to satisfy the inclusion criteria. Mean age was 46 years and 90% were females. The most common underlying diagnosis was Mixed connective tissue disorder (MCTD). Digital gangrene was the presenting symptom in 13 (60%) patients. Half of the patients received only corticosteroids as immunosuppression. Two died due to systemic complications. Complete resolution occurred in 17 (85%), autoamputation in 3, and infection requiring surgical drainage in one patient. All surviving patients reported good functional limb outcome on 6 months follow-up. MCTD is an important cause of digital gangrene in rheumatology practice. In patients presenting with digital gangrene, an active search for an underlying CTD is imperative, as this could result in timely initiation of appropriate limb-saving therapy. Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in CTD. Key Points • Mixed connective tissue disorder is an important cause of digital gangrene in rheumatology practice in western India. • In patients presenting with digital gangrene, an active search for an underlying connective tissue disorder is imperative, as this could result in timely initiation of appropriate therapy and can prove limb saving. • Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in connective tissue disorders. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Vijayan, Neeraja AU - Vijayan N AD - Department of Internal Medicine, All India Institute of Medical Sciences, Basni Industrial Estate, Jodhpur, 342005, India. FAU - Vijayvergia, Parag AU - Vijayvergia P AD - Department of Internal Medicine, All India Institute of Medical Sciences, Basni Industrial Estate, Jodhpur, 342005, India. FAU - Bohra, Gopal Krishna AU - Bohra GK AD - Department of Internal Medicine, All India Institute of Medical Sciences, Basni Industrial Estate, Jodhpur, 342005, India. FAU - Garg, Mahendra Kumar AU - Garg MK AD - Department of Internal Medicine, All India Institute of Medical Sciences, Basni Industrial Estate, Jodhpur, 342005, India. FAU - Gopalakrishnan, Maya AU - Gopalakrishnan M AUID- ORCID: 0000-0002-0661-9854 AD - Department of Internal Medicine, All India Institute of Medical Sciences, Basni Industrial Estate, Jodhpur, 342005, India. maya.gopalakrishnan@gmail.com. LA - eng PT - Journal Article PT - Observational Study DEP - 20220703 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Connective Tissue MH - *Connective Tissue Diseases/complications/diagnosis MH - Female MH - Gangrene/complications MH - Humans MH - India MH - Male MH - Middle Aged MH - *Mixed Connective Tissue Disease/complications MH - *Raynaud Disease/etiology OTO - NOTNLM OT - Critical-limb ischemia OT - Mixed connective tissue disorder OT - Raynaud’s phenomenon OT - Systemic lupus erythematosus OT - U1RNP EDAT- 2022/07/03 06:00 MHDA- 2022/10/19 06:00 CRDT- 2022/07/02 23:26 PHST- 2021/10/18 00:00 [received] PHST- 2022/06/24 00:00 [accepted] PHST- 2022/06/16 00:00 [revised] PHST- 2022/07/03 06:00 [pubmed] PHST- 2022/10/19 06:00 [medline] PHST- 2022/07/02 23:26 [entrez] AID - 10.1007/s10067-022-06265-1 [pii] AID - 10.1007/s10067-022-06265-1 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Nov;41(11):3543-3549. doi: 10.1007/s10067-022-06265-1. Epub 2022 Jul 3. PMID- 36214919 OWN - NLM STAT- MEDLINE DCOM- 20230110 LR - 20230111 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 42 IP - 1 DP - 2023 Jan TI - Assessing scleroderma patterns with superb microvascular imaging: is it possible? New prospects for ultrasound. PG - 301-306 LID - 10.1007/s10067-022-06405-7 [doi] AB - Nailfold videocapillaroscopy is the gold standard for the early differentiation of primary and secondary Raynaud's phenomenon. Advances in high-frequency ultrasound with superb microvascular imaging show significant potential for exploring structural changes that were previously inaccessible. Ultrasound makes it possible to assess not only the superficial layers of the skin but also structural microvascular abnormalities in the deep layers of the nail fold. There is potential for identifying a 'scleroderma pattern', which presents with the loss of continuous vascular arches above and below the nail plate in transverse and longitudinal scans of the nail folds. The 'active' pattern presents with the loss of the junctions between vascular signals, which is not seen in the 'early' pattern. Severely reduced vascularity with avascular areas in both of the nail fold zones is seen in a 'late' pattern. The quality of the evaluation is highly dependent on how experienced the sonographer is. This is the first detailed description of every pattern assessed through superb microvascular imaging, including high-quality images for a better understanding of the technique. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Jasionyte, Gabija AU - Jasionyte G AUID- ORCID: 0000-0001-8436-9275 AD - Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 01513, Vilnius, Lithuania. gabija.jasionyte@gmail.com. FAU - Seskute, Goda AU - Seskute G AUID- ORCID: 0000-0003-4830-1497 AD - Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 01513, Vilnius, Lithuania. FAU - Rugiene, Rita AU - Rugiene R AUID- ORCID: 0000-0002-2670-647X AD - Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 01513, Vilnius, Lithuania. AD - Department of Experimental, Preventive and Clinical Medicine, State Research Institute Centre for Innovative Medicine, 08406, Vilnius, Lithuania. FAU - Butrimiene, Irena AU - Butrimiene I AUID- ORCID: 0000-0002-3795-7741 AD - Clinic of Rheumatology, Orthopaedics Traumatology, and Reconstructive Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 01513, Vilnius, Lithuania. LA - eng PT - Journal Article PT - Review DEP - 20221010 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - Nails/diagnostic imaging/blood supply MH - Skin/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging MH - Ultrasonography, Doppler MH - Capillaries MH - *Scleroderma, Localized MH - Microscopic Angioscopy/methods MH - *Scleroderma, Systemic/diagnostic imaging OTO - NOTNLM OT - Nail fold, Scleroderma pattern OT - Superb microvascular imaging OT - Ultrasound OT - Videocapillaroscopy EDAT- 2022/10/11 06:00 MHDA- 2023/01/11 06:00 CRDT- 2022/10/10 11:17 PHST- 2022/07/15 00:00 [received] PHST- 2022/09/27 00:00 [accepted] PHST- 2022/09/26 00:00 [revised] PHST- 2022/10/11 06:00 [pubmed] PHST- 2023/01/11 06:00 [medline] PHST- 2022/10/10 11:17 [entrez] AID - 10.1007/s10067-022-06405-7 [pii] AID - 10.1007/s10067-022-06405-7 [doi] PST - ppublish SO - Clin Rheumatol. 2023 Jan;42(1):301-306. doi: 10.1007/s10067-022-06405-7. Epub 2022 Oct 10. PMID- 41283914 OWN - NLM STAT- MEDLINE DCOM- 20251124 LR - 20251210 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 45 IP - 12 DP - 2025 Nov 24 TI - Factors associated with diagnostic delay and specialty consultation patterns in systemic sclerosis: A cross-sectional study. PG - 278 LID - 10.1007/s00296-025-06034-8 [doi] AB - Early diagnosis of Systemic Sclerosis (SSc) is challenging due to nonspecific symptoms and clinical overlap with other autoimmune diseases. To investigate factors associated with diagnostic delays in SSc, focusing on the number and specialties of physicians consulted before diagnosis. This cross-sectional study included SSc patients registered at a university rheumatology clinic. Demographics, clinical features, and physical examination findings were recorded. The number and specialties of physicians consulted before diagnosis were obtained through structured face-to-face interviews. Of 240 screened patients, 135 were included (120 female, 88.8%; 55 with diffuse SSc, 40.7%; mean age 52.1 ± 11.5 years). The median time to diagnosis was 36 months (IQR 12-96) from Raynaud's phenomenon (RP) onset and 11(IQR 0-35) months from the first non-RP symptom. Patients with limited SSc had a significantly longer RP-to-diagnosis interval than those with diffuse SSc (44 vs. 20 months, p = 0.024). The median number of physicians consulted before diagnosis was 3 (range 1-9). Higher educational level (IRR = 0.97, p = 0.036) and referral by a familiar healthcare professional (IRR = 0.78, p = 0.035) were independently associated with fewer pre-diagnostic consultations. Among 119 patients who recalled their first physician, 42 (35.3%) initially consulted an internist, 28 (23.5%) a family physician, 11 (9.2%) a dermatologist, and 10 (8.4%) a cardiovascular surgeon; the remainder visited other specialists. Only 8 patients (6.7%) received an SSc diagnosis at their first consultation. SSc diagnosis is often delayed, requiring multiple consultations. Greater physician awareness and timely rheumatology referrals are essential. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Erez, Yesim AU - Erez Y AUID- ORCID: 0000-0002-2669-2880 AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. erezyesim@gmail.com. FAU - Kenar Artın, Gökce AU - Kenar Artın G AUID- ORCID: 0000-0002-0485-1369 AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. FAU - Yarkan Tugsal, Handan AU - Yarkan Tugsal H AUID- ORCID: 0000-0003-0633-790X AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. FAU - Sen, Gercek AU - Sen G AUID- ORCID: 0000-0001-8347-0873 AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. FAU - Onen, Fatoş AU - Onen F AUID- ORCID: 0000-0002-6341-2622 AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. FAU - Birlik, Merih AU - Birlik M AUID- ORCID: 0000-0001-5118-9307 AD - Division of Rheumatology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Dokuz Eylul Universitesi Sağlık Yerleşkesi, Balçova, 35340, İzmir, Turkey. LA - eng PT - Journal Article DEP - 20251124 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Humans MH - Female MH - Cross-Sectional Studies MH - Male MH - Middle Aged MH - *Delayed Diagnosis MH - *Referral and Consultation/statistics & numerical data MH - Adult MH - Aged MH - *Scleroderma, Systemic/diagnosis MH - Time Factors MH - Raynaud Disease/diagnosis/etiology MH - Rheumatology MH - *Scleroderma, Diffuse/diagnosis OTO - NOTNLM OT - Delayed Diagnosis OT - Physicians OT - Referral and Consultation OT - Scleroderma, Systemic COIS- Declarations. Conflict of interest: The authors have declared no conflicts of interest. Ethical approval: The study was initiated following the approval of the Dokuz Eylul University School of Medicine. EDAT- 2025/11/24 12:39 MHDA- 2025/11/24 18:39 CRDT- 2025/11/24 11:03 PHST- 2025/08/09 00:00 [received] PHST- 2025/11/06 00:00 [accepted] PHST- 2025/11/24 18:39 [medline] PHST- 2025/11/24 12:39 [pubmed] PHST- 2025/11/24 11:03 [entrez] AID - 10.1007/s00296-025-06034-8 [pii] AID - 10.1007/s00296-025-06034-8 [doi] PST - epublish SO - Rheumatol Int. 2025 Nov 24;45(12):278. doi: 10.1007/s00296-025-06034-8. PMID- 20696074 OWN - NLM STAT- MEDLINE DCOM- 20110119 LR - 20211020 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 12 IP - 4 DP - 2010 TI - Comparison of laser Doppler imaging, fingertip lacticemy test, and nailfold capillaroscopy for assessment of digital microcirculation in systemic sclerosis. PG - R157 LID - 10.1186/ar3112 [doi] AB - INTRODUCTION: Laser Doppler imaging (LDI) is a relatively new method for assessing the functional aspect of superficial skin blood flow in systemic sclerosis (SSc) and Raynaud's phenomenon. The present study investigated the dynamic behavior of digital skin microvascular blood flow before and after cold stimulus (CS) in SSc patients and in healthy controls by means of a comprehensive approach of the functional (LDI), morphological (nailfold capillaroscopy (NFC)), and biochemical (fingertip lacticemy (FTL)) microcirculation components. METHODS: Forty-four SSc patients and 40 healthy controls were included. After acclimatization, all subjects underwent NFC followed by LDI and FTL measurement. NFC was performed with a stereomicroscope under 10× to 20× magnification in the 10 digits of the hands. Skin blood flow of the dorsum of four fingertips (excluding the thumb) of the left hand was measured using LDI at baseline and for 30 minutes after CS. The mean finger blood flow (FBF) of the four fingertips was expressed as arbitrary perfusion units. FTL was determined on the fourth left finger before (pre-CS-FTL) and 10 minutes after CS. RESULTS: LDI showed significantly lower mean baseline FBF in SSc patients as compared with controls (296.9 ± 208.8 vs. 503.6 ± 146.4 perfusion units; P < 0.001) and also at all time points after CS (P < 0.001). There was a significant decrease in mean FBF after CS as compared with baseline in SSc patients and in controls, followed by recovery of the blood flow 27 minutes after CS in healthy controls, but not in SSc patients. FBF tended to be lower in patients with digital scars and previous ulceration/amputation (P = 0.06). There was no correlation between mean baseline FBF and NFC parameters. Interestingly, there was a negative correlation between FTL and FBF measured by LDI in basal conditions and 10 minutes after CS in SSc patients. CONCLUSIONS: LDI showed lower digital blood flow in SSc patients when compared with healthy controls and correlated well with FTL both at baseline and after CS, allowing objective measurement of blood perfusion in SSc patients. The lack of correlation between functional and morphological microvascular abnormalities, measured by LDI and NFC, suggests they are complementary tools for evaluation of independent microangiopathy aspects in SSc patients. FAU - Correa, Marcelo Ju AU - Correa MJ AD - Department of Medicine, Universidade Federal de São Paulo, São Paulo 04023-062, Brazil. marcelo.reumato@yahoo.com.br FAU - Andrade, Luis Ec AU - Andrade LE FAU - Kayser, Cristiane AU - Kayser C LA - eng PT - Comparative Study PT - Controlled Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100810 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM CIN - Nat Rev Rheumatol. 2010 Oct;6(10):555. doi: 10.1038/nrrheum.2010.146. PMID: 20925149 CIN - Arthritis Res Ther. 2011 Jan 24;13(1):301; author reply 302. doi: 10.1186/ar3201. PMID: 21345273 MH - Adult MH - Cold Temperature MH - Female MH - Fingers/blood supply MH - Humans MH - Laser-Doppler Flowmetry/*methods MH - Male MH - Microcirculation/*physiology MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/blood supply MH - Raynaud Disease/diagnostic imaging/physiopathology MH - Scleroderma, Systemic/*diagnostic imaging/*physiopathology MH - Ultrasonography PMC - PMC2945058 EDAT- 2010/08/11 06:00 MHDA- 2011/01/20 06:00 PMCR- 2010/08/10 CRDT- 2010/08/11 06:00 PHST- 2010/02/18 00:00 [received] PHST- 2010/07/02 00:00 [revised] PHST- 2010/08/10 00:00 [accepted] PHST- 2010/08/11 06:00 [entrez] PHST- 2010/08/11 06:00 [pubmed] PHST- 2011/01/20 06:00 [medline] PHST- 2010/08/10 00:00 [pmc-release] AID - ar3112 [pii] AID - 10.1186/ar3112 [doi] PST - ppublish SO - Arthritis Res Ther. 2010;12(4):R157. doi: 10.1186/ar3112. Epub 2010 Aug 10. PMID- 33001586 OWN - NLM STAT- MEDLINE DCOM- 20210322 LR - 20260127 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 73 IP - 3 DP - 2021 Mar TI - Dissecting the Cellular Mechanism of Prostacyclin Analog Iloprost in Reversing Vascular Dysfunction in Scleroderma. PG - 520-529 LID - 10.1002/art.41536 [doi] AB - OBJECTIVE: Intravenous iloprost improves Raynaud's phenomenon (RP) and promotes healing of digital ulcers in systemic sclerosis (SSc; scleroderma). Despite a short half-life, its clinical efficacy lasts weeks. Endothelial adherens junctions, which are formed by VE-cadherin clustering between endothelial cells (ECs), regulate endothelial properties including barrier function, endothelial-to-mesenchymal transition (EndoMT), and angiogenesis. We undertook this study to investigate the hypothesis that junctional disruption contributes to vascular dysfunction in SSc, and that the protective effect of iloprost is mediated by strengthening of those junctions. METHODS: Dermal ECs from SSc patients and healthy controls were isolated. The effect of iloprost on ECs was examined using immunofluorescence, permeability assays, Matrigel tube formation, and quantitative polymerase chain reaction. RESULTS: Adherens junctions in SSc were disrupted compared to normal ECs, as indicated by reduced levels of VE-cadherin and increased permeability in SSc ECs (P < 0.05). Iloprost increased VE-cadherin clustering at junctions and restored junctional levels of VE-cadherin in SSc ECs (mean ± SD 37.3 ± 4.3 fluorescence units) compared to normal ECs (mean ± SD 29.7 ± 3.4 fluorescence units; P < 0.05), after 2 hours of iloprost incubation. In addition, iloprost reduced permeability of monolayers, increased tubulogenesis, and blocked EndoMT in both normal and SSc ECs (n ≥ 3; P < 0.05). The effects in normal ECs were inhibited by a function-blocking antibody that prevents junctional clustering of VE-cadherin. CONCLUSION: Our data suggest that the long-lasting effects of iloprost reflect its ability to stabilize adherens junctions, resulting in increased tubulogenesis and barrier function and reduced EndoMT. These findings provide a mechanistic basis for the use of iloprost in treating SSc patients with RP and digital ulcers. CI - © 2020, American College of Rheumatology. FAU - Tsou, Pei-Suen AU - Tsou PS AUID- ORCID: 0000-0002-7149-9115 AD - University of Michigan, Ann Arbor. FAU - Palisoc, Pamela J AU - Palisoc PJ AD - University of Michigan, Ann Arbor. FAU - Flavahan, Nicholas A AU - Flavahan NA AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - R01 AR070470/AR/NIAMS NIH HHS/United States GR - R01 HD078639/HD/NICHD NIH HHS/United States GR - UL1 TR002240/TR/NCATS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20210208 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antigens, CD) RN - 0 (Cadherins) RN - JED5K35YGL (Iloprost) RN - 0 (Vasodilator Agents) RN - 0 (Cadherin 5) SB - IM MH - Adherens Junctions/*drug effects/metabolism MH - Antigens, CD/*drug effects/metabolism MH - Cadherins/*drug effects/metabolism MH - Capillary Permeability/drug effects MH - Case-Control Studies MH - Cells, Cultured MH - Endothelial Cells/*drug effects/metabolism MH - Epithelial-Mesenchymal Transition/drug effects MH - Female MH - Humans MH - Iloprost/*pharmacology/therapeutic use MH - Male MH - Middle Aged MH - Neovascularization, Physiologic/drug effects MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Scleroderma, Diffuse/complications/*drug therapy/physiopathology MH - Vasodilator Agents/*pharmacology/therapeutic use MH - Cadherin 5 PMC - PMC7914149 MID - NIHMS1634712 COIS- Competing interests: Dr. Khanna is the Chief Medical Officer and stock holder in Eicos Sciences, Inc, which is currently conducting Phase 3 clinical trial for intravenous iloprost in the treatment of digital ischemic episodes due to systemic sclerosis. EDAT- 2020/10/02 06:00 MHDA- 2021/03/23 06:00 PMCR- 2022/03/01 CRDT- 2020/10/01 12:19 PHST- 2020/04/22 00:00 [received] PHST- 2020/09/22 00:00 [accepted] PHST- 2020/10/02 06:00 [pubmed] PHST- 2021/03/23 06:00 [medline] PHST- 2020/10/01 12:19 [entrez] PHST- 2022/03/01 00:00 [pmc-release] AID - 10.1002/art.41536 [doi] PST - ppublish SO - Arthritis Rheumatol. 2021 Mar;73(3):520-529. doi: 10.1002/art.41536. Epub 2021 Feb 8. PMID- 20410154 OWN - NLM STAT- MEDLINE DCOM- 20100909 LR - 20110311 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 19 IP - 7 DP - 2010 Jun TI - Clinical analysis of systemic lupus erythematosus with gastrointestinal manifestations. PG - 866-9 LID - 10.1177/0961203310365883 [doi] AB - Our objective was to analyse the clinical characteristics of systemic lupus erythematosus (SLE) patients with gastrointestinal manifestations. Medical charts of 177 hospitalized SLE patients were systematically reviewed, including demographic data, clinical features, laboratory findings, and treatments, as well as outcomes. Thirty-nine cases (22.0%) had SLE-related gastrointestinal manifestations, and in 12 cases (30.8%), gastrointestinal manifestations occurred as the initial symptoms. Twenty-five cases (64.1%) had abdominal pain, 22 cases (56.4%) had nausea and vomiting, 12 cases (30.8%) had diarrhea, and gastrointestinal hemorrhage occurred in three cases (7.7%). Protein losing enteropathy and intestinal pseudo-obstruction were the most common identifiable gastrointestinal complications, though other reasons such as superior mesenteric venous thrombosis, pancreatitis, peritonitis, and liver impairment could also occur in SLE. The incidences of Raynaud's phenomenon and pyeloureterectasis were significantly higher in patients with gastrointestinal complications than those without (p < 0.05). Multivariable analysis indicated Raynaud's phenomenon, decreased C3, CH50, and anti-neutrophil cytoplasmic antibody positivity were independent predictors of gastrointestinal involvements (p < 0.05). Gastrointestinal complications are common, diverse, and could be the initial and major manifestations of lupus. SLE patients who had Raynaud's phenomenon, hypocomplementemia and positive anti-neutrophil cytoplasmic antibody were at increasing risk of developing gastrointestinal complication. FAU - Xu, D AU - Xu D AD - Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China. FAU - Yang, H AU - Yang H FAU - Lai, C-C AU - Lai CC FAU - Li, P AU - Li P FAU - Zhang, X AU - Zhang X FAU - Yang, X-O AU - Yang XO FAU - Zhang, F-C AU - Zhang FC FAU - Qian, J-M AU - Qian JM LA - eng PT - Journal Article DEP - 20100421 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 9007-36-7 (Complement System Proteins) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antineutrophil Cytoplasmic/metabolism MH - Child MH - Complement System Proteins/metabolism MH - Female MH - Gastrointestinal Diseases/diagnosis/*etiology/physiopathology MH - Humans MH - Lupus Erythematosus, Systemic/*complications/diagnosis MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Raynaud Disease/*complications MH - Retrospective Studies MH - Risk Factors MH - Young Adult EDAT- 2010/04/23 06:00 MHDA- 2010/09/10 06:00 CRDT- 2010/04/23 06:00 PHST- 2010/04/23 06:00 [entrez] PHST- 2010/04/23 06:00 [pubmed] PHST- 2010/09/10 06:00 [medline] AID - 0961203310365883 [pii] AID - 10.1177/0961203310365883 [doi] PST - ppublish SO - Lupus. 2010 Jun;19(7):866-9. doi: 10.1177/0961203310365883. Epub 2010 Apr 21. PMID- 24004754 OWN - NLM STAT- MEDLINE DCOM- 20150413 LR - 20250529 IS - 1875-9270 (Electronic) IS - 1051-9815 (Print) IS - 1051-9815 (Linking) VI - 47 IP - 1 DP - 2014 TI - Vibration induced white-feet: overview and field study of vibration exposure and reported symptoms in workers. PG - 101-10 LID - 10.3233/WOR-131692 [doi] AB - BACKGROUND: Workers who stand on platforms or equipment that vibrate are exposed to foot-transmitted vibration (FTV). Exposure to FTV can lead to vibration white feet/toes resulting in blanching of the toes, and tingling and numbness in the feet and toes. OBJECTIVES: The objectives are 1) to review the current state of knowledge of the health risks associated with foot-transmitted vibration (FTV), and 2) to identify the characteristics of FTV and discuss the associated risk of vibration-induced injury. PARTICIPANTS: Workers who operated locomotives (n=3), bolting platforms (n=10), jumbo drills (n=7), raise drilling platforms (n=4), and crushers (n=3), participated. METHODS: A tri-axial accelerometer was used to measure FTV in accordance with ISO 2631-1 guidelines. Frequency-weighted root-mean-square acceleration and the dominant frequency are reported. Participants were also asked to report pain/ache/discomfort in the hands and/or feet. RESULTS: Reports of pain/discomfort/ache were highest in raise platform workers and jumbo drill operators who were exposed to FTV in the 40 Hz and 28 Hz range respectively. Reports of discomfort/ache/pain were lowest in the locomotive and crusher operators who were exposed to FTV below 10 Hz. These findings are consistent with animal studies that have shown vascular and neural damage in exposed appendages occurs at frequencies above 40 Hz. CONCLUSIONS: Operators exposed to FTV at 40 Hz appear to be at greater risk of experiencing vibration induced injury. Future research is required to document the characteristics of FTV and epidemiological evidence is required to link exposure with injury. FAU - Eger, Tammy AU - Eger T AD - School of Human Kinetics, Laurentian University, Sudbury, ON, Canada Centre for Research in Occupational Safety and Health, Laurentian University, Sudbury, ON, Canada. FAU - Thompson, Aaron AU - Thompson A AD - Department of Medicine, Division of Occupational Medicine, University of Toronto, Toronto, ON, Canada Department of Occupational and Environmental Health, St. Michael's Hospital, Toronto, ON, Canada. FAU - Leduc, Mallorie AU - Leduc M AD - School of Human Kinetics, Laurentian University, Sudbury, ON, Canada. FAU - Krajnak, Kristine AU - Krajnak K AD - Engineering and Controls Technology Branch, National Institute for Occupational Safety and Health, Morgantown, WV, USA. FAU - Goggins, Katie AU - Goggins K AD - School of Human Kinetics, Laurentian University, Sudbury, ON, Canada. FAU - Godwin, Alison AU - Godwin A AD - School of Human Kinetics, Laurentian University, Sudbury, ON, Canada Centre for Research in Occupational Safety and Health, Laurentian University, Sudbury, ON, Canada. FAU - House, Ron AU - House R AD - Department of Medicine, Division of Occupational Medicine, University of Toronto, Toronto, ON, Canada Department of Occupational and Environmental Health, St. Michael's Hospital, Toronto, ON, Canada. LA - eng GR - CC999999/ImCDC/Intramural CDC HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Work JT - Work (Reading, Mass.) JID - 9204382 SB - IM MH - Accelerometry MH - Adult MH - Foot Diseases/*etiology MH - Humans MH - Middle Aged MH - *Mining MH - Occupational Diseases/*etiology MH - Occupational Exposure/*adverse effects MH - Pain/etiology MH - Raynaud Disease/*etiology MH - Vibration/*adverse effects PMC - PMC4597303 MID - NIHMS723146 OID - NLM: HHSPA723146 OTO - NOTNLM OT - Occupational vibration OT - Raynaud's OT - standing OT - vibration white-toes OT - white-foot EDAT- 2013/09/06 06:00 MHDA- 2015/04/14 06:00 PMCR- 2015/10/08 CRDT- 2013/09/06 06:00 PHST- 2013/09/06 06:00 [entrez] PHST- 2013/09/06 06:00 [pubmed] PHST- 2015/04/14 06:00 [medline] PHST- 2015/10/08 00:00 [pmc-release] AID - C57324813764W588 [pii] AID - 10.3233/WOR-131692 [doi] PST - ppublish SO - Work. 2014;47(1):101-10. doi: 10.3233/WOR-131692. PMID- 41965070 OWN - NLM STAT- MEDLINE DCOM- 20260513 LR - 20260515 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 65 IP - 5 DP - 2026 May 5 TI - Novel pleiotropic loci link vascular and immune pathways in systemic sclerosis and primary Raynaud's phenomenon. LID - 10.1093/rheumatology/keag164 [doi] LID - keag164 AB - OBJECTIVE: Raynaud's phenomenon (RP) is the first noticeable symptom of systemic sclerosis (SSc), appearing even years before other signs. Vascular and immune pathways, hallmarks in SSc pathogenesis, are implicated in primary RP. Hence, we aimed to define the shared genetic architecture between these conditions to explore pathogenic mechanisms and whether pleiotropic loci could help identify primary RP patients at increased genetic risk of developing SSc. METHODS: We analysed genome-wide association study (GWAS) summary statistics for primary RP (4 986 cases and 850 981 controls) and SSc (10 654 cases and 18 043 controls). We performed a cross-trait meta-analysis (∼8.6M single nucleotide polymorphisms) to identify variants associated with both diseases. Functional annotation was used to prioritise potential causal genes. We also constructed a polygenic risk score (PRS) to assess clinical implications. RESULTS: Beyond the well-known HLA associations, we identified five additional pleiotropic loci, including MEOX2 as a novel association for both traits with opposing effects, ADRA2A representing a novel genetic factor for SSc, and IL12A, NFKB1 and TNIP1 as novel loci for primary RP. Functional annotation highlighted vascular and inflammatory pathways. Finally, the PRS model shows modest discrimination (area under the curve = 0.57). However, it allows the identification of primary RP individuals at high genetic risk of developing SSc (75th percentile, relative risk =1.29 [95% CI 1.02, 1.62]; P-value= 3.58 × 10-2). CONCLUSION: This study reveals a genetic link between primary RP and SSc, identifying five novel pleiotropic loci and supporting the potential of genetic profiling for early risk assessment and personalised monitoring strategies. CI - © The Author(s) 2026. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Rangel-Peláez, Carlos AU - Rangel-Peláez C AUID- ORCID: 0000-0001-7697-1696 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Rodriguez-Martin, Inmaculada AU - Rodriguez-Martin I AUID- ORCID: 0000-0001-5413-1581 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Rosa-Baez, Carlos AU - Rosa-Baez C AUID- ORCID: 0000-0002-1257-3338 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Kerick, Martin AU - Kerick M AUID- ORCID: 0000-0002-6298-4514 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Guillén-Del-Castillo, Alfredo AU - Guillén-Del-Castillo A AUID- ORCID: 0000-0003-0626-507X AD - Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, Barcelona, Spain. FAU - Simeón-Aznar, Carmen P AU - Simeón-Aznar CP AUID- ORCID: 0000-0003-3390-9029 AD - Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, Barcelona, Spain. FAU - Callejas, José L AU - Callejas JL AUID- ORCID: 0000-0002-1653-0087 AD - Systemic Autoimmune Disease Unit, Hospital Clínico San Cecilio, Instituto de Investigación Biosanitaria Ibs.GRANADA, Granada, Spain. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Proudman, Susanna M AU - Proudman SM AUID- ORCID: 0000-0002-3046-9884 AD - Rheumatology Unit, Royal Adelaide Hospital and the University of Adelaide, Adelaide, South Australia, Australia. FAU - Nikpour, Mandana AU - Nikpour M AUID- ORCID: 0000-0002-6585-5611 AD - Department of Medicine, University of Melbourne, Fitzroy, Victoria, Australia. AD - Faculty of Medicine and Health, The University of Sydney School of Public Health, Sydney, New South Wales, Australia. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology and Venereology, University Hospital Cologne, Cologne, Germany. FAU - Moroncini, Gianluca AU - Moroncini G AUID- ORCID: 0000-0002-0380-0105 AD - Department of Clinical and Molecular Science, Marche Polytechnic University, Ancona, Italy. AD - Department of Internal Medicine, Marche University Hospital, Ancona, Italy. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AUID- ORCID: 0000-0002-5624-1415 AD - The Department of Rheumatology, Leiden University Medical Center, Leiden, the Netherlands. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Allanore, Yannick AU - Allanore Y AUID- ORCID: 0000-0002-6149-0002 AD - Department of Rheumatology, Université Paris Cité UFR de Médecine, Paris, France. FAU - Alarcón-Riquelme, Marta E AU - Alarcón-Riquelme ME AUID- ORCID: 0000-0002-7632-4154 AD - Centre for Genomics and Oncological Research, Pfizer, University of Granada/Andalusian Regional Government, Spain, Granada. FAU - Beretta, Lorenzo AU - Beretta L AUID- ORCID: 0000-0002-6529-6258 AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Mayes, Maureen D AU - Mayes MD AUID- ORCID: 0000-0001-5070-2535 AD - UT Health Houston, Division of Rheumatology, McGovern Medical School, Houston, TX, USA. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Royal Free Hospital, UCL, London, UK. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - UT Health Houston, Division of Rheumatology, McGovern Medical School, Houston, TX, USA. FAU - Martín, Javier AU - Martín J AUID- ORCID: 0000-0002-2202-0622 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Acosta-Herrera, Marialbert AU - Acosta-Herrera M AUID- ORCID: 0000-0002-9868-6535 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. FAU - Ortiz-Fernández, Lourdes AU - Ortiz-Fernández L AUID- ORCID: 0000-0002-0247-4280 AD - Institute of Parasitology and Biomedicine López-Neyra, CSIC, Granada, Spain. CN - and the International SSc Group, the PRECISESADS Clinical Consortium, and the Australian Scleroderma Interest Group (ASIG)# LA - eng PT - Journal Article PT - Meta-Analysis PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Scleroderma, Systemic/genetics/immunology MH - *Raynaud Disease/genetics/immunology MH - Genome-Wide Association Study MH - Genetic Predisposition to Disease MH - Polymorphism, Single Nucleotide MH - *Genetic Pleiotropy MH - Genetic Loci PMC - PMC13171172 OTO - NOTNLM OT - MEOX2 OT - GWAS OT - Raynaud’s phenomenon OT - cross-trait OT - polygenic risk score OT - systemic sclerosis FIR - Carreira, P IR - Carreira P FIR - Castellvi, I IR - Castellvi I FIR - Ortego-Centeno, N IR - Ortego-Centeno N FIR - García Portales, R IR - García Portales R FIR - Fernández-Nebro, A IR - Fernández-Nebro A FIR - García-Hernández, F J IR - García-Hernández FJ FIR - Aguirre, M A IR - Aguirre MA FIR - Fernández-Gutiérrez, B IR - Fernández-Gutiérrez B FIR - Rodríguez-Rodríguez, L IR - Rodríguez-Rodríguez L FIR - García de la Peña, P IR - García de la Peña P FIR - Vicente, E IR - Vicente E FIR - Andreu, J L IR - Andreu JL FIR - Fernández de Castro, M IR - Fernández de Castro M FIR - López-Longo, F J IR - López-Longo FJ FIR - Fonollosa, V IR - Fonollosa V FIR - Guillén, A IR - Guillén A FIR - Espinosa, G IR - Espinosa G FIR - Tolosa, C IR - Tolosa C FIR - Pros, A IR - Pros A FIR - Beltrán, E IR - Beltrán E FIR - Carballeira, M Rodríguez IR - Carballeira MR FIR - Narváez, F J IR - Narváez FJ FIR - Rivas, M Rubio IR - Rivas MR FIR - Ortiz-Santamaría, V IR - Ortiz-Santamaría V FIR - Madroñero, A B IR - Madroñero AB FIR - González-Gay, M A IR - González-Gay MA FIR - Díaz, B IR - Díaz B FIR - Trapiella, L IR - Trapiella L FIR - Egurbide, M V IR - Egurbide MV FIR - Fanlo-Mateo, P IR - Fanlo-Mateo P FIR - Saez-Comet, L IR - Saez-Comet L FIR - Díaz, F IR - Díaz F FIR - Roman-Ivorra, J A IR - Roman-Ivorra JA FIR - Sancho, J J Alegre IR - Sancho JJA FIR - Freire, M IR - Freire M FIR - Garcia, F J Blanco IR - Garcia FJB FIR - Oreiro, N IR - Oreiro N FIR - Witte, T IR - Witte T FIR - Kreuter, A IR - Kreuter A FIR - Riemekasten, G IR - Riemekasten G FIR - Airo, P IR - Airo P FIR - Magro, C IR - Magro C FIR - Voskuyl, A E IR - Voskuyl AE FIR - Vonk, M C IR - Vonk MC FIR - Hesselstrand, R IR - Hesselstrand R FIR - Nordin, A IR - Nordin A FIR - Lunardi, C IR - Lunardi C FIR - Moroncini, G IR - Moroncini G FIR - Gabrielli, A IR - Gabrielli A FIR - Hoffmann-Vold, A IR - Hoffmann-Vold A FIR - Distler, J H W IR - Distler JHW FIR - Padyukov, L IR - Padyukov L FIR - Koeleman, B P C IR - Koeleman BPC FIR - Radstake, T R D J IR - Radstake TRDJ FIR - Orozco, G IR - Orozco G FIR - Barton, A IR - Barton A FIR - Fonseca, C IR - Fonseca C FIR - Vigone, Barbara IR - Vigone B FIR - Pers, Jacques-Olivier IR - Pers JO FIR - Saraux, Alain IR - Saraux A FIR - Devauchelle-Pensec, Valérie IR - Devauchelle-Pensec V FIR - Cornec, Divi IR - Cornec D FIR - Jousse-Joulin, Sandrine IR - Jousse-Joulin S FIR - Lauwerys, Bernard IR - Lauwerys B FIR - Ducreux, Julie IR - Ducreux J FIR - Maudoux, Anne-Lise IR - Maudoux AL FIR - Vasconcelos, Carlos IR - Vasconcelos C FIR - Tavares, Ana IR - Tavares A FIR - Neves, Esmeralda IR - Neves E FIR - Faria, Raquel IR - Faria R FIR - Brandão, Mariana IR - Brandão M FIR - Campar, Ana IR - Campar A FIR - Marinho, António IR - Marinho A FIR - Farinha, Fátima IR - Farinha F FIR - Mantecón, Miguel Angel Gonzalez-Gay IR - Mantecón MAG FIR - Alonso, Ricardo Blanco IR - Alonso RB FIR - Martínez, Alfonso Corrales IR - Martínez AC FIR - Cervera, Ricard IR - Cervera R FIR - Rodríguez-Pintó, Ignasi IR - Rodríguez-Pintó I FIR - Espinosa, Gerard IR - Espinosa G FIR - Lories, Rik IR - Lories R FIR - Langhe, Ellen De IR - Langhe E FIR - Hunzelmann, Nicolas IR - Hunzelmann N FIR - Belz, Doreen IR - Belz D FIR - Witte, Torsten IR - Witte T FIR - Baerlecken, Niklas IR - Baerlecken N FIR - Stummvoll, Georg IR - Stummvoll G FIR - Zauner, Michael IR - Zauner M FIR - Lehner, Michaela IR - Lehner M FIR - Collantes, Eduardo IR - Collantes E FIR - Ortega-Castro, Rafaela IR - Ortega-Castro R FIR - Aguirre-Zamorano, M Angeles IR - Aguirre-Zamorano MA FIR - Escudero-Contreras, Alejandro IR - Escudero-Contreras A FIR - Castro-Villegas, M Carmen IR - Castro-Villegas MC FIR - Ortego, Norberto IR - Ortego N FIR - Roldán, María Concepción Fernández IR - Roldán MCF FIR - Raya, Enrique IR - Raya E FIR - Moleón, Inmaculada Jiménez IR - Moleón IJ FIR - de Ramon, Enrique IR - de Ramon E FIR - Quintero, Isabel Díaz IR - Quintero ID FIR - Meroni, Pier Luigi IR - Meroni PL FIR - Gerosa, Maria IR - Gerosa M FIR - Schioppo, Tommaso IR - Schioppo T FIR - Artusi, Carolina IR - Artusi C FIR - Chizzolini, Carlo IR - Chizzolini C FIR - Zuber, Aleksandra IR - Zuber A FIR - Wynar, Donatienne IR - Wynar D FIR - Kovács, Laszló IR - Kovács L FIR - Balog, Attila IR - Balog A FIR - Deák, Magdolna IR - Deák M FIR - Bocskai, Márta IR - Bocskai M FIR - Dulic, Sonja IR - Dulic S FIR - Kádár, Gabriella IR - Kádár G FIR - Hiepe, Falk IR - Hiepe F FIR - Gerl, Velia IR - Gerl V FIR - Thiel, Silvia IR - Thiel S FIR - Maresca, Manuel Rodriguez IR - Maresca MR FIR - López-Berrio, Antonio IR - López-Berrio A FIR - Aguilar-Quesada, Rocío IR - Aguilar-Quesada R FIR - Navarro-Linares, Héctor IR - Navarro-Linares H FIR - Ferdowsi, N IR - Ferdowsi N FIR - Hansen, D IR - Hansen D FIR - Host, L V IR - Host LV FIR - Major, G IR - Major G FIR - Ngian, G-S IR - Ngian GS FIR - Quinlivan, A IR - Quinlivan A FIR - Ross, L IR - Ross L FIR - Sahhar, J IR - Sahhar J FIR - Stevens, W IR - Stevens W FIR - Tabesh, M IR - Tabesh M FIR - Walker, J IR - Walker J EDAT- 2026/04/12 04:35 MHDA- 2026/05/14 06:16 PMCR- 2026/04/08 CRDT- 2026/04/11 14:33 PHST- 2025/10/27 00:00 [received] PHST- 2026/03/10 00:00 [accepted] PHST- 2026/05/14 06:16 [medline] PHST- 2026/04/12 04:35 [pubmed] PHST- 2026/04/11 14:33 [entrez] PHST- 2026/04/08 00:00 [pmc-release] AID - 8644332 [pii] AID - keag164 [pii] AID - 10.1093/rheumatology/keag164 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 May 5;65(5):keag164. doi: 10.1093/rheumatology/keag164. PMID- 39874231 OWN - NLM STAT- MEDLINE DCOM- 20250128 LR - 20260127 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 84 IP - 1 DP - 2025 Jan TI - EULAR recommendations for the treatment of systemic sclerosis: 2023 update. PG - 29-40 LID - S0003-4967(24)00584-3 [pii] LID - 10.1136/ard-2024-226430 [doi] AB - OBJECTIVES: To update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for treatment of systemic sclerosis (SSc), incorporating new evidence and therapies. METHODS: An international task force was convened in line with EULAR standard operating procedures. A nominal group technique exercise was performed in two rounds to define questions underpinning a subsequent systematic literature review. The evidence derived was discussed and overarching principles, recommendations and future research agenda were iteratively developed with voting rounds. RESULTS: The task force agreed on 22 recommendations covering 8 clinical/organ domains including Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease (ILD), gastrointestinal manifestations and arthritis. Most new recommendations are related to skin fibrosis and ILD. These included novel recommendations for the use of mycophenolate mofetil, nintedanib, rituximab and tocilizumab for the treatment of these crucial disease manifestations. The recommendations also included first-line and second-line interventions, providing increased utility for rheumatology practitioners. Important additions to the future research agenda included consideration of novel interventions for the management of vascular, musculoskeletal and gastrointestinal manifestations and calcinosis, as well as for the local management of digital ulcers. CONCLUSION: These updated recommendations include the first set of synthetic and biological targeted therapies recommended for key fibrotic manifestations of SSc as well as first-line combination treatment for newly diagnosed pulmonary artery hypertension and prioritise a new research agenda for the coming years. CI - Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. Electronic address: f.delgaldo@leeds.ac.uk. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France. FAU - Conaghan, Philip G AU - Conaghan PG AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Bertoldo, Eugenia AU - Bertoldo E AD - Department of Medicine, Rheumatology Unit, Universita degli Studi di Verona, Verona, Italy. FAU - Čolić, Jelena AU - Čolić J AD - Rheumatology, University of Belgrade Faculty of Medicine, Beograd, Serbia. FAU - Santiago, Tânia AU - Santiago T AD - Rheumatology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal. FAU - Suliman, Yossra A AU - Suliman YA AD - Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assiut University, Assiut, Egypt; Rheumatology division, Ain Alkhaleej Hospital, Alain, Abu-Dhabi, UAE. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), and Inflammation, fibrosis and ageing initiative (INFLAGE), IRCCS San Raffaele Hospital, Milano, Italy. FAU - Gabrielli, Armando AU - Gabrielli A AD - Scienze Cliniche e Molecolari, Università Politecnica delle Marche Facoltà di Medicina e Chirurgia, Ancona, Italy. FAU - Distler, Oliver AU - Distler O AD - University Hospital Zürich Center of Experimental Rheumatology, Zurich, Switzerland. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AD - Oslo University Hospital, Oslo, Norway. FAU - Castellví, Ivan AU - Castellví I AD - Rheumatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. Electronic address: https://twitter.com/IvanCastellvi. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B. Shine Department of Rheumatology, Department of Internal Medicine B, Rambam Health Care Campus, Haifa, Israel. FAU - Vonk, Madelon AU - Vonk M AD - Department of Rheumatic diseases, Radboud Universiteit, Nijmegen, Netherlands. FAU - Ananyeva, Lidia AU - Ananyeva L AD - Institute of Rheumatology, Russian Academy of Medical Sciences, Moskva, Russian Federation. FAU - Rednic, Simona AU - Rednic S AD - Clinica Reumatologie, UMF Iuliu Haţieganu Cluj-Napoca, Cluj-Napoca, Romania. FAU - Tarasova, Anna AU - Tarasova A AD - Nasonova Research Institute of Rheumatology of RAMS, Moskva, Moskva, Russian Federation. FAU - Ostojic, Pedrag AU - Ostojic P AD - Institute of Rheumatology, University of Belgrade Faculty of Medicine, Belgrade, Serbia. FAU - Boyadzhieva, Vladimira AU - Boyadzhieva V AD - Department of Rheumatology, Medical University-Sofia, Sofia, Bulgaria. FAU - El Aoufy, Khadija AU - El Aoufy K AD - Department of Clinical and Experimental Medicine, University of Florence Faculty of Medicine and Surgery, Firenze, Italy. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK; Federation of European Scleroderma Associations, Milan, Italy. FAU - Galetti, Ilaria AU - Galetti I AD - Federation of European Scleroderma Associations, Milan, Italy. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Royal Free Hospital, UCL, London, UK. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Mueller-Ladner, Ulf AU - Mueller-Ladner U AD - Rheumatology and Clinical Immunology, University of Giessen, Giessen, Germany. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Université Paris Cité UFR de Médecine, Paris, France. LA - eng PT - Journal Article PT - Practice Guideline DEP - 20250102 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - G6HRD2P839 (nintedanib) RN - 4F4X42SYQ6 (Rituximab) RN - I031V2H011 (tocilizumab) RN - 0 (Indoles) RN - 0 (Antibodies, Monoclonal, Humanized) SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/therapy/drug therapy MH - Raynaud Disease/etiology/drug therapy MH - Rheumatology MH - Lung Diseases, Interstitial/etiology/drug therapy MH - Skin Ulcer/etiology MH - Rituximab/therapeutic use MH - Pulmonary Arterial Hypertension/etiology/drug therapy MH - Indoles MH - Antibodies, Monoclonal, Humanized OTO - NOTNLM OT - Connective Tissue Diseases OT - Pulmonary Arterial Hypertension OT - Pulmonary Fibrosis OT - Scleroderma OT - Systemic OT - Therapeutics EDAT- 2025/01/28 18:21 MHDA- 2025/01/28 18:22 CRDT- 2025/01/28 13:24 PHST- 2024/07/16 00:00 [received] PHST- 2024/09/20 00:00 [accepted] PHST- 2025/01/28 18:22 [medline] PHST- 2025/01/28 18:21 [pubmed] PHST- 2025/01/28 13:24 [entrez] AID - S0003-4967(24)00584-3 [pii] AID - 10.1136/ard-2024-226430 [doi] PST - ppublish SO - Ann Rheum Dis. 2025 Jan;84(1):29-40. doi: 10.1136/ard-2024-226430. Epub 2025 Jan 2. PMID- 24951144 OWN - NLM STAT- MEDLINE DCOM- 20150202 LR - 20140621 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 141 IP - 6-7 DP - 2014 Jun-Jul TI - [Clinical and capillaroscopic regression of CD30 anaplastic lymphoma associated with limited cutaneous systemic sclerosis following autologous bone marrow transplantation]. PG - 446-51 LID - S0151-9638(14)00271-3 [pii] LID - 10.1016/j.annder.2014.04.116 [doi] AB - BACKGROUND: In rare cases, tumors are associated with secondary Raynaud's phenomenon in systemic sclerosis (SSc). We report the case of a patient presenting cutaneous limited SSc associated with CD30 anaplastic lymphoma with cutaneous and lymph node involvement in whom the capillaroscopic scleroderma pattern regressed completely after autologous bone marrow transplantation, with complete remission of the lymphoma. CASE REPORT: A 37-year-old man presented bilateral Raynaud's phenomenon associated with digital ulcers contracted one year earlier but subsequently neglected. Right axillary lymph nodes and regional cutaneous tumors were present, leading to the diagnosis of CD30+ anaplastic lymphoma with cutaneous and lymph node involvement. Chemotherapy containing cyclophosphamide achieved only partial remission of the lymphoma. Clinical examination showed bilateral Raynaud's phenomenon, sclerodactyly, a right axillary subcutaneous nodule and a pathological Allen's test for the right hand. Antinuclear antibodies were positive without any other immunological abnormalities, and capillaroscopy showed an SSc pattern with numerous megacapillaries. Digital blood pressure was reduced in the right index and the left middle fingers, in which ulcers of the pulp were observed. Bone marrow transplantation was performed, resulting in complete remission of the lymphoma and disappearance of the sclerodactyly, with no recurrence of the pulp ulcers and complete normalization of capillaroscopic appearance and digital pressure. DISCUSSION: This case raises the question of authentic SSc and neoplasia and highlights the importance of capillaroscopy in the follow-up of SSc. The complete regression of SSc and of capillaroscopic abnormalities could be explained by the paraneoplastic nature of SSc or by the direct action of the chemotherapy and bone marrow transplantation. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Bellis, R AU - Bellis R AD - Service de dermatologie-allergologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address: bellis.reza@neuf.fr. FAU - Francès, C AU - Francès C AD - Service de dermatologie-allergologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France; Université Paris VI, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Barète, S AU - Barète S AD - Service de dermatologie-allergologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Senet, P AU - Senet P AD - Service de dermatologie-allergologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Régression clinique et capillaroscopique après autogreffe de moelle d'une sclérodermie systémique cutanée limitée associée à un lymphome anaplasique CD30. DEP - 20140602 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Antibodies, Antinuclear) RN - 11056-06-7 (Bleomycin) RN - 80168379AG (Doxorubicin) RN - 8N3DW7272P (Cyclophosphamide) RN - RSA8KO39WH (Vindesine) RN - VB0R961HZT (Prednisone) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Antineoplastic Combined Chemotherapy Protocols/*therapeutic use MH - Bleomycin/administration & dosage MH - *Bone Marrow Transplantation MH - Combined Modality Therapy MH - Cyclophosphamide/administration & dosage MH - Doxorubicin/administration & dosage MH - Fingers/blood supply MH - Humans MH - Lymphoma, Large-Cell, Anaplastic/*complications/therapy MH - Male MH - Methotrexate/administration & dosage MH - Microscopic Angioscopy MH - Paraneoplastic Syndromes/*etiology/immunology/therapy MH - Prednisone/administration & dosage MH - Raynaud Disease/*etiology MH - Remission Induction MH - Scleroderma, Limited/*etiology/immunology/therapy MH - Skin Ulcer/etiology MH - Transplantation, Autologous MH - Vindesine/administration & dosage OTO - NOTNLM OT - Capillaroscopie OT - Capillaroscopy OT - Lymphome non hodgkinien OT - Non-Hodgkin's lymphoma OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Sclérodermie systémique OT - Systemic sclerosis EDAT- 2014/06/22 06:00 MHDA- 2015/02/03 06:00 CRDT- 2014/06/22 06:00 PHST- 2013/12/26 00:00 [received] PHST- 2014/03/18 00:00 [revised] PHST- 2014/04/02 00:00 [accepted] PHST- 2014/06/22 06:00 [entrez] PHST- 2014/06/22 06:00 [pubmed] PHST- 2015/02/03 06:00 [medline] AID - S0151-9638(14)00271-3 [pii] AID - 10.1016/j.annder.2014.04.116 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2014 Jun-Jul;141(6-7):446-51. doi: 10.1016/j.annder.2014.04.116. Epub 2014 Jun 2. PMID- 35685580 OWN - NLM STAT- MEDLINE DCOM- 20220613 LR - 20220716 IS - 1742-1241 (Electronic) IS - 1368-5031 (Print) IS - 1368-5031 (Linking) VI - 2022 DP - 2022 TI - The Association of Flow-Mediated Dilatation and Blood Parameters in Primary Raynaud's Phenomenon. PG - 9347946 LID - 10.1155/2022/9347946 [doi] LID - 9347946 AB - INTRODUCTION: Raynaud's phenomenon (RP) is a multifactorial disorder. If any underlying disease cannot be determined to be responsible for RP, then it is considered to be the primary RP (pRP). We aimed to investigate the differences between laboratory markers and impaired endothelial function in pRP. MATERIALS AND METHODS: Forty-two pRP patients and 30 healthy individuals were included as the study and control groups, respectively. The endothelial function was evaluated with flow-mediated dilatation (FMD) of the brachial artery. The blood samples were obtained from both groups, and white blood cell (WBC), hemoglobin, platelets, mean platelet volume (MPV), creatinine, alanine aminotransferase (ALT), aspartate aminotransferase (AST), D-dimer, fibrinogen, albumin, fibrinogen-to-albumin ratio (FAR), neutrophil-to-lymphocyte ratio (NLR), D-dimer-to-albumin ratio (DDAR), and monocyte chemoattractant protein-1 (MCP-1) parameters were studied. The blood parameters and FMD values obtained were compared between groups. RESULTS: The groups were similar in regard to age, gender, and smoking history (p < 0.05). There was no difference between the two groups in regard to hemoglobin, platelet, MPV, creatinine, ALT, D-dimer, albumin, FAR, NLR, and DDAR levels (p < 0.05). AST levels were slightly higher in the pRP group (p=0.027). Markedly increased WBC, fibrinogen, MPV, and MCP-1 values were detected in the pRP group (p=0.001), as well as higher abnormal FMD responses (p=0.001). There was a direct correlation between abnormal FMD response and serum MCP-1 values in patients with pRP (R: 0.308, R (2): 0.095, p: 0.044). CONCLUSION: It seems to be that MCP-1 levels are higher in patients with pRP, and increased values of MCP-1 levels seem to be related to impaired endothelial functions. CI - Copyright © 2022 Süheyla Uzun and İlker Kaya. FAU - Uzun, Süheyla AU - Uzun S AUID- ORCID: 0000-0003-3570-5361 AD - Department of Internal Medicine, Gaziosmanpasa University, Tokat, Turkey. FAU - Kaya, İlker AU - Kaya İ AUID- ORCID: 0000-0001-6222-2133 AD - Department of Cardiovascular Surgery, Tokat State Hospital, Tokat, Turkey. LA - eng PT - Journal Article DEP - 20220131 PL - United States TA - Int J Clin Pract JT - International journal of clinical practice JID - 9712381 RN - 0 (Albumins) RN - 9001-32-5 (Fibrinogen) RN - AYI8EX34EU (Creatinine) SB - IM MH - Albumins MH - *Brachial Artery MH - Creatinine MH - Dilatation MH - Fibrinogen MH - Humans MH - *Raynaud Disease PMC - PMC9159131 COIS- The authors declare that they have no conflicts of interest. EDAT- 2022/06/11 06:00 MHDA- 2022/06/14 06:00 PMCR- 2022/01/31 CRDT- 2022/06/10 02:32 PHST- 2021/10/07 00:00 [received] PHST- 2021/11/30 00:00 [accepted] PHST- 2022/06/10 02:32 [entrez] PHST- 2022/06/11 06:00 [pubmed] PHST- 2022/06/14 06:00 [medline] PHST- 2022/01/31 00:00 [pmc-release] AID - 10.1155/2022/9347946 [doi] PST - epublish SO - Int J Clin Pract. 2022 Jan 31;2022:9347946. doi: 10.1155/2022/9347946. eCollection 2022. PMID- 24871260 OWN - NLM STAT- MEDLINE DCOM- 20150116 LR - 20240503 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 11 IP - 6 DP - 2014 May 27 TI - Effects of partially ionised medical oxygen, especially with O2•-, in vibration white finger patients. PG - 5698-707 LID - 10.3390/ijerph110605698 [doi] AB - A major symptom of hand-arm vibration syndrome is a secondary Raynaud's phenomenon-vibration white finger (VWF)-which results from a vasospasm of the digital arteries caused by work with vibration devices leading to occupational disease. Pharmacotherapy of VWF is often ineffective or has adverse effects. The aim of this work was to verify the influence of inhalation of partially ionized oxygen (O2•-) on peripheral blood vessels in the hands of patients with VWF. Ninety one (91)patients with VWF underwent four-finger adsorption plethysmography, and the pulse wave amplitude was recorded expressed in numeric parameters-called the native record. Next, a cold water test was conducted following with second plethysmography. The patients were divided in to the three groups. First and second inhaled 20-min of ionized oxygen O2•- or oxygen O2 respectively. Thirth group was control without treatment. All three groups a follow-up third plethysmography-the post-therapy record. Changes in the pulse wave amplitudes were evaluated. Inpatients group inhaling O2•- a modest increase of pulse wave amplitude was observed compared to the native record; patients inhaling medical oxygen O2 and the control showed a undesirable decline of pulse wave amplitude in VWF fingers. Strong vasodilatation were more frequent in the group inhaling O2•- compare to O2 (p < 0.05). Peripheral vasodilatation achieved by inhalation of O2•- could be used for VWF treatment without undesirable side effect in hospital as well as at home environment. FAU - Perečinský, Slavomír AU - Perečinský S AD - Department of Occupational Medicine and Clinical Toxicology, Faculty of Medicine, Pavol Jozef Safarik University, 04190 Kosice, Slovak Republic. slavomir.perecinsky@upjs.sk. FAU - Murínová, Lenka AU - Murínová L AD - Department of Occupational Medicine and Clinical Toxicology, Faculty of Medicine, Pavol Jozef Safarik University, 04190 Kosice, Slovak Republic. murinovalenka@yahoo.com. FAU - Engler, Ivan AU - Engler I AD - Department of Human Physiology, Faculty of Medicine, Pavol Jozef Safarik University, 04001 Kosice, Slovak Republic. engler@io2th.com. FAU - Donič, Viliam AU - Donič V AD - Department of Human Physiology, Faculty of Medicine, Pavol Jozef Safarik University, 04001 Kosice, Slovak Republic. viliam.donic@upjs.sk. FAU - Murín, Pavol AU - Murín P AD - Cardiology Clinic, Faculty of Medicine, Pavol Jozef Safarik University, 04001 Košice, Slovak Republic. murinpavol@yahoo.com. FAU - Varga, Marek AU - Varga M AD - Department of Occupational Medicine and Clinical Toxicology, Faculty of Medicine, Pavol Jozef Safarik University, 04190 Kosice, Slovak Republic. marek.varga@upjs.sk. FAU - Legáth, Lubomír AU - Legáth L AD - Department of Occupational Medicine and Clinical Toxicology, Faculty of Medicine, Pavol Jozef Safarik University, 04190 Kosice, Slovak Republic. lubomir.legath@upjs.sk. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140527 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 SB - IM MH - Adult MH - *Air Ionization MH - Female MH - Hand-Arm Vibration Syndrome/*therapy MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases MH - Oxygen Inhalation Therapy/*methods MH - Raynaud Disease/therapy MH - Vibration/*adverse effects PMC - PMC4078543 EDAT- 2014/05/30 06:00 MHDA- 2015/01/17 06:00 PMCR- 2014/06/01 CRDT- 2014/05/30 06:00 PHST- 2014/03/12 00:00 [received] PHST- 2014/05/16 00:00 [revised] PHST- 2014/05/16 00:00 [accepted] PHST- 2014/05/30 06:00 [entrez] PHST- 2014/05/30 06:00 [pubmed] PHST- 2015/01/17 06:00 [medline] PHST- 2014/06/01 00:00 [pmc-release] AID - ijerph110605698 [pii] AID - ijerph-11-05698 [pii] AID - 10.3390/ijerph110605698 [doi] PST - epublish SO - Int J Environ Res Public Health. 2014 May 27;11(6):5698-707. doi: 10.3390/ijerph110605698. PMID- 22415927 OWN - NLM STAT- MEDLINE DCOM- 20121126 LR - 20131121 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 21 IP - 8 DP - 2012 Jul TI - Microcirculation as determined by iontophoresis in SLE-patients and controls. PG - 815-20 LID - 10.1177/0961203312439117 [doi] AB - BACKGROUND: The risk of cardiovascular disease (CVD), microangiopathy and prevalence of atherosclerotic plaques are increased in Systemic Lupus Erythematosus (SLE). As systemic endothelial dysfunction is one of the earliest signs of these vascular outcomes in the general population we assessed skin microvascular endothelial function in SLE patients. METHODS: Endothelial function in skin was tested with local application of acetylcholine (inducing endothelium-dependent vasodilatation) and any concomitant increase in skin perfusion was measured with Laser Doppler Fluxmetry (LDF) in 84 SLE-patients (83% women, mean age 47 years) and 81 age and sex matched controls. Common carotid intima-media thickness (cIMT) and plaque occurrence were also determined using B-mode ultrasound. RESULTS: There were no significant differences in skin microvascular endothelial function between SLE-patients and controls. In the SLE group, endothelial function did not vary in relation to skin manifestations, Raynaud's phenomenon, nephritis or plaque occurrence. In SLE patients with CVD, however, endothelial function was impaired. CONCLUSION: Skin microvascular endothelial function is associated with CVD but not with early signs of atherosclerosis in SLE-patients. The endothelial function is not different in SLE-patients as compared to controls. FAU - Anania, C AU - Anania C AD - Department of Medicine, at the Karolinska Institute, Stockholm, Sweden. FAU - Norman, M AU - Norman M FAU - Heimburger, M AU - Heimburger M FAU - Gustafsson, T AU - Gustafsson T FAU - Jogestrand, T AU - Jogestrand T FAU - Hafström, I AU - Hafström I FAU - Frostegård, J AU - Frostegård J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120313 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Vasodilator Agents) RN - N9YNS0M02X (Acetylcholine) SB - IM MH - Acetylcholine/administration & dosage MH - Adult MH - Atherosclerosis/complications/*physiopathology MH - Carotid Intima-Media Thickness MH - Carotid Stenosis/complications/physiopathology MH - Endothelium, Vascular/*physiopathology MH - Female MH - Humans MH - Iontophoresis MH - Lasers MH - Lupus Erythematosus, Systemic/complications/*physiopathology MH - Male MH - *Microcirculation MH - Middle Aged MH - Nephritis/physiopathology MH - Raynaud Disease/physiopathology MH - Skin/*blood supply MH - Statistics, Nonparametric MH - Vasodilator Agents/administration & dosage EDAT- 2012/03/15 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/03/15 06:00 PHST- 2012/03/15 06:00 [entrez] PHST- 2012/03/15 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - 0961203312439117 [pii] AID - 10.1177/0961203312439117 [doi] PST - ppublish SO - Lupus. 2012 Jul;21(8):815-20. doi: 10.1177/0961203312439117. Epub 2012 Mar 13. PMID- 34463711 OWN - NLM STAT- MEDLINE DCOM- 20220509 LR - 20220711 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 5 DP - 2022 May 5 TI - Causes of Raynaud's phenomenon and the predictive laboratory and capillaroscopy features for the evolution to a definite connective tissue disease. PG - 1975-1985 LID - 10.1093/rheumatology/keab668 [doi] AB - OBJECTIVE: In patients with RP, capillaroscopy is useful for discriminating primary from secondary causes. There are certain capillaroscopy and lab values as predictive factors leading to a known CTD. We conducted the present study to evaluate the causes of RP in our area and followed the studied subjects to find prognostic factors indicating a definite CTD or remaining a UCTD. METHODS: In this retrospective cohort study we included all adult patients with RP who were referred for capillaroscopy from 2010 to 2019. All the patients with primary and secondary RP with follow-up were evaluated for demography, laboratory results and capillaroscopy to find the risk factors of their progression to a CTD. RESULTS: A total of 760 of 776 patients were included, with 679 being female (89.3%) and 81 (10.7%) male. There were 660 subjects (90.8%) with secondary RP [mostly UCTD (48.2%) and then SSc (16.4%)] and 67 (9.2%) with primary RP; 109 patients were followed up and 42 (42%) of those with secondary RP developed a definite CTD. The scleroderma pattern and some capillary changes on capillaroscopy and/or positive ANA had statistically significant differences for CTD transition. CONCLUSION: We had a small number of patients with primary RP. The most prevalent causes of secondary RP in our patients were UCTD and SSc. Some capillaroscopy and laboratory results alone or in combination could be used as a predictive marker for the transition of patients with UCTD to CTD. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AD - Department of Internal Medicine, Division of Rheumatology. FAU - Ajri, Mehrnoush AU - Ajri M AUID- ORCID: 0000-0001-9066-3155 AD - Department of Internal Medicine. FAU - Hamidi, Sahand AU - Hamidi S AD - Department of Cardiology, Shiraz University of Medical Sciences, Shiraz, Iran. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2022 Jul 6;61(7):e188-e189. doi: 10.1093/rheumatology/keab884. PMID: 34864903 CIN - Rheumatology (Oxford). 2022 Jul 6;61(7):e190-e191. doi: 10.1093/rheumatology/keab885. PMID: 34864926 MH - Adult MH - Capillaries/diagnostic imaging MH - *Connective Tissue Diseases/complications/diagnostic imaging MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - *Raynaud Disease/complications MH - Retrospective Studies OTO - NOTNLM OT - RP OT - UCTD OT - capillaroscopy EDAT- 2021/09/01 06:00 MHDA- 2022/05/10 06:00 CRDT- 2021/08/31 13:11 PHST- 2021/04/13 00:00 [received] PHST- 2021/08/18 00:00 [accepted] PHST- 2021/09/01 06:00 [pubmed] PHST- 2022/05/10 06:00 [medline] PHST- 2021/08/31 13:11 [entrez] AID - 6360480 [pii] AID - 10.1093/rheumatology/keab668 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 May 5;61(5):1975-1985. doi: 10.1093/rheumatology/keab668. PMID- 19373465 OWN - NLM STAT- MEDLINE DCOM- 20091104 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 10 DP - 2009 Aug TI - Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital. PG - 1243-5 LID - 10.1007/s00296-009-0938-z [doi] AB - We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement. FAU - Teh, C L AU - Teh CL AD - Unit of Rheumatology, Department of Medicine, Sarawak General Hospital, Jalan Hospital, 93450, Kuching, Sarawak, Malaysia. tehchenglay@yahoo.com FAU - Kuan, Y C AU - Kuan YC FAU - Wong, J S AU - Wong JS LA - eng PT - Journal Article DEP - 20090417 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) RN - 0 (Calcium Channel Blockers) SB - IM MH - Age of Onset MH - Autoantibodies MH - Calcium Channel Blockers MH - Cross-Sectional Studies MH - Female MH - *Hospitals, Public MH - Humans MH - Hypertension, Pulmonary/*diagnosis MH - Malaysia MH - Male MH - Middle Aged MH - Pulmonary Fibrosis/*diagnosis MH - Raynaud Disease/*diagnosis MH - Scleroderma, Diffuse/diagnosis MH - Scleroderma, Limited/diagnosis MH - Scleroderma, Systemic/*diagnosis/pathology MH - Skin Ulcer/*diagnosis EDAT- 2009/04/18 09:00 MHDA- 2009/11/05 06:00 CRDT- 2009/04/18 09:00 PHST- 2008/12/22 00:00 [received] PHST- 2009/03/28 00:00 [accepted] PHST- 2009/04/18 09:00 [entrez] PHST- 2009/04/18 09:00 [pubmed] PHST- 2009/11/05 06:00 [medline] AID - 10.1007/s00296-009-0938-z [doi] PST - ppublish SO - Rheumatol Int. 2009 Aug;29(10):1243-5. doi: 10.1007/s00296-009-0938-z. Epub 2009 Apr 17. PMID- 41101787 OWN - NLM STAT- MEDLINE DCOM- 20260313 LR - 20260501 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 11 IP - 4 DP - 2025 Oct 15 TI - Autoantibodies directed against the angiotensin II type 1 receptor and the endothelin-1 type A receptor in patients with systemic sclerosis. LID - 10.1136/rmdopen-2025-005787 [doi] LID - e005787 AB - OBJECTIVES: To evaluate the clinical applicability of autoantibodies (AAbs) measured by ELISA against the angiotensin II type 1 receptor (AT(1)R) and endothelin-1 type A receptor (ET(A)R) in systemic sclerosis (SSc) patients. METHODS: Serum samples from n=279 SSc patients from the Leiden Systemic Sclerosis cohort, n=42 patients with primary Raynaud's phenomenon, n=24 patients with rheumatoid arthritis and n=20 healthy controls were tested for anti-AT(1)R- and anti-ET(A)R AAbs. Levels were compared between groups with Mann-Whitney U tests or Kruskal-Wallis tests. Risk ratios and Kaplan-Meier analyses were used to determine associations between AAbs and disease manifestations or all-cause mortality. Analyses were repeated in an independent cohort with n=310 SSc patients from the Radboud University Medical Center. RESULTS: AAbs against AT(1)R and ET(A)R could be detected by ELISA in the sera of all groups tested. Levels were slightly higher in the SSc group compared with the pooled non-SSc group (p=0.043). No associations could be found between anti-AT(1)R AAbs or anti-ET(A)R AAbs and disease manifestations or all-cause mortality. In the Radboud cohort, patients with diffuse cutaneous SSc (p=0.001) and interstitial lung disease (p=0.007) had higher median anti-ET(A)R AAb levels. Patients who died during follow-up had lower levels of anti-AT(1)R- (p=0.005) and anti-ET(A)R AAbs (p=0.020). CONCLUSIONS: We confirm positive ELISAs for anti-AT(1)R AAbs and anti-ET(A)R AAbs in the sera of several patient groups and healthy controls. Previously described associations with disease manifestations and all-cause mortality could not be confirmed in our cohorts. Based on the current study, the determination of these AAbs is of limited predictive value in clinical practice. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - van der Wouden, Katherine E AU - van der Wouden KE AUID- ORCID: 0009-0001-8238-1649 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. AD - Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Center, Amsterdam, Netherlands. FAU - Ahmed, Saad AU - Ahmed S AUID- ORCID: 0000-0002-3098-0172 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - van Oostveen, Wieke M AU - van Oostveen WM AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Hoekstra, Eva M AU - Hoekstra EM AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Liem, Sophie I E AU - Liem SIE AUID- ORCID: 0000-0003-0328-7062 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Huizinga, Tom W J AU - Huizinga TWJ AUID- ORCID: 0000-0001-7033-7520 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Toes, René E M AU - Toes REM AUID- ORCID: 0000-0002-9618-6414 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Voskuyl, Alexandre E AU - Voskuyl AE AD - Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Center, Amsterdam, Netherlands. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany. FAU - Fehres, Cynthia M AU - Fehres CM AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Vonk, Madelon AU - Vonk M AUID- ORCID: 0000-0002-2266-9907 AD - Department of Rheumatic Diseases, Radboud University Medical Center, Nijmegen, Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AUID- ORCID: 0000-0002-5624-1415 AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands j.k.de_Vries-Bouwstra@lumc.nl. LA - eng PT - Journal Article DEP - 20251015 PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Receptor, Endothelin A) RN - 0 (Receptor, Angiotensin, Type 1) RN - 0 (Autoantibodies) SB - IM MH - *Scleroderma, Systemic/blood/immunology/mortality MH - Enzyme-Linked Immunosorbent Assay MH - *Receptor, Endothelin A/immunology MH - *Receptor, Angiotensin, Type 1/immunology MH - *Autoantibodies/blood/immunology MH - Case-Control Studies MH - Cause of Death MH - Raynaud Disease/blood/immunology/mortality MH - Arthritis, Rheumatoid/blood/immunology/mortality MH - Healthy Volunteers MH - *Lung Diseases, Interstitial/blood/immunology/mortality MH - Humans MH - Male MH - Female MH - Adult MH - Middle Aged MH - Aged PMC - PMC12530366 OTO - NOTNLM OT - Autoantibodies OT - Autoimmune Diseases OT - Biomarkers OT - Systemic Sclerosis COIS- Competing interests: SA: J&J-Innovative Medicine. MV: Speaker fees from Janssen Pharmaceutical Companies of Johnson & Johnson, MSD, Novartis and Roche, and consulting fees from Boehringer-Ingelheim and Janssen Pharmaceutical Companies of Johnson & Johnson. JKdV-B: AbbVie, Janssen, Boehringer-Ingelheim, Janssen-Cilag, Galapagos and Roche. EDAT- 2025/10/17 00:31 MHDA- 2025/10/17 00:32 PMCR- 2025/10/15 CRDT- 2025/10/16 20:52 PHST- 2025/04/11 00:00 [received] PHST- 2025/08/12 00:00 [accepted] PHST- 2025/10/17 00:32 [medline] PHST- 2025/10/17 00:31 [pubmed] PHST- 2025/10/16 20:52 [entrez] PHST- 2025/10/15 00:00 [pmc-release] AID - rmdopen-2025-005787 [pii] AID - 10.1136/rmdopen-2025-005787 [doi] PST - epublish SO - RMD Open. 2025 Oct 15;11(4):e005787. doi: 10.1136/rmdopen-2025-005787. PMID- 25842153 OWN - NLM STAT- MEDLINE DCOM- 20160419 LR - 20150508 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 99 DP - 2015 May TI - Prevalence and evolution of scleroderma pattern at nailfold videocapillaroscopy in systemic sclerosis patients: Clinical and prognostic implications. PG - 92-5 LID - S0026-2862(15)00032-1 [pii] LID - 10.1016/j.mvr.2015.03.005 [doi] AB - BACKGROUND: Microvascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis occurring early in the course of the disease. Microangiopathy is responsible of important clinical manifestations, such as Raynaud's phenomenon, digital ulceration, and pulmonary arterial hypertension. Typical microvascular alterations, called scleroderma pattern, are detectable at nailfold capillaroscopy in a significant percentage of SSc patients; however its prevalence is highly variable in published studies. AIM: The aims of this study are to evaluate the prevalence and the evolution of scleroderma pattern in SSc patients and analyze their demographic, clinical and prognostic characteristics according to capillaroscopic features. METHODS: Two hundred and seventy-five SSc patients, underwent at least two nailfold videocapillaroscopy during follow-up, were retrospectively enrolled. RESULTS: A scleroderma pattern was observed in 80% of patients at baseline and 87.1% during follow-up, and it was significantly associated to digital ulcers, interstitial lung disease, reduction of diffusion lung of carbon monoxide <75%, teleangectasias and melanodermia, while sicca syndrome and arthralgias were associated to normal/nonspecific pattern. Digital ulcers, teleangectasias, sicca syndrome, and arthralgias remained independently associated with scleroderma pattern on multivariate analysis. In conclusion, the main clinical manifestation correlated with scleroderma pattern is the occurrence of digital ulcers, and their appearance is strictly correlated with the variation of capillaroscopic feature during the time. Further studies should confirm the association between SSc pattern and lung fibrosis. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Ghizzoni, Cecilia AU - Ghizzoni C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. FAU - Sebastiani, Marco AU - Sebastiani M AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. Electronic address: marco.sebastiani@unimore.it. FAU - Manfredi, Andreina AU - Manfredi A AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. FAU - Campomori, Federica AU - Campomori F AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. FAU - Colaci, Michele AU - Colaci M AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. FAU - Ferri, Clodoveo AU - Ferri C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Italy. LA - eng PT - Journal Article DEP - 20150402 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Autoantibodies) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Adult MH - Aged MH - Autoantibodies/chemistry MH - Capillaries/pathology MH - Carbon Monoxide/chemistry MH - Diffusion MH - Female MH - Fingers/*pathology MH - Follow-Up Studies MH - Humans MH - Lung Diseases/pathology MH - Lung Diseases, Interstitial/*diagnosis/epidemiology/physiopathology MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply MH - Prevalence MH - Prognosis MH - Raynaud Disease/complications MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnosis/epidemiology/physiopathology MH - Skin Ulcer/pathology MH - Ulcer/physiopathology OTO - NOTNLM OT - Digital ulcers OT - Interstitial lung disease OT - Nailfold videocapillaroscopy OT - Scleroderma OT - Systemic sclerosis EDAT- 2015/04/07 06:00 MHDA- 2016/04/20 06:00 CRDT- 2015/04/06 06:00 PHST- 2015/01/01 00:00 [received] PHST- 2015/03/21 00:00 [revised] PHST- 2015/03/25 00:00 [accepted] PHST- 2015/04/06 06:00 [entrez] PHST- 2015/04/07 06:00 [pubmed] PHST- 2016/04/20 06:00 [medline] AID - S0026-2862(15)00032-1 [pii] AID - 10.1016/j.mvr.2015.03.005 [doi] PST - ppublish SO - Microvasc Res. 2015 May;99:92-5. doi: 10.1016/j.mvr.2015.03.005. Epub 2015 Apr 2. PMID- 18425964 OWN - NLM STAT- MEDLINE DCOM- 20080613 LR - 20250623 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) IP - 2 DP - 2008 Apr 16 TI - Oral vasodilators for primary Raynaud's phenomenon. PG - CD006687 LID - 10.1002/14651858.CD006687.pub2 [doi] AB - BACKGROUND: Many different drugs have been suggested for the symptomatic treatment of primary Raynaud's phenomenon. Apart from calcium channel blockers, which are considered the drugs of choice, the evidence of the effects of alternative pharmacological treatments is limited. OBJECTIVES: To assess the effects of various drugs with vasodilator actions on primary Raynaud's phenomenon. SEARCH STRATEGY: The Cochrane Peripheral Vascular Diseases Group searched their Specialised Register (last searched 24 July 2007), and the Cochrane Central Register of Controlled Trials (CENTRAL) (last searched Issue 3, 2007). In addition, we searched MEDLINE (January 1966 to July 2007), EMBASE (1980 to July 2007) and reference lists of relevant articles. We contacted pharmaceutical companies. There were no language restrictions. SELECTION CRITERIA: Randomised controlled trials evaluating the effects of oral formulations of any drug with vasodilator effects on subjective symptoms in primary Raynaud's phenomenon. Treatment with, or comparison with, calcium channel blockers was not assessed in this review. DATA COLLECTION AND ANALYSIS: Both authors assessed the trials for inclusion and their quality. One author (BV) extracted the data MS checked the results. Data extraction included adverse events. we contacted trial authors for missing data. MAIN RESULTS: Eight studies involving 290 participants were included. Two trials examined the effects of captopril, the rest were single trials on single drugs. All comparisons were with placebo. The methodological quality of most trials was poor. Enalapril was associated with a small increase in the frequency of attacks per week (difference in means 0.8; 95% CI 0.43 to 1.17). The difference between the intervention groups on a subjective improvement score was non-significant. There was a significant effect of buflomedil on the frequency of attacks per week (weighted mean difference (WMD) -8.8; 95% CI -17.55 to -0.09), but there was no evidence of effect on the severity score. The proportion with fewer attacks was significantly higher on moxisylyte than on placebo (relative risk (RR) 4.33; 95% CI 1.36 to 13.81). For captopril, beraprost, dazoxiben and ketanserin there was no evidence of an effect on the frequency, severity or duration of attacks. Beraprost and moxisylyte gave significantly more adverse effects than placebo. AUTHORS' CONCLUSIONS: Poor methodological quality, small sample sizes and the limited data available resulted in low precision of the statistical results and limited value of the overall results . The overall results show that there is no evidence for an effect of vasodilator drugs on primary Raynaud's phenomenon. FAU - Vinjar, B AU - Vinjar B AD - Møre and Romsdal County, Department of Health and Social Services, Fylkeshusa, Molde, Norway, 6407. bergljot.vinjar@nesset.post.no FAU - Stewart, M AU - Stewart M LA - eng GR - CZB/4/609/CSO_/Chief Scientist Office/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20080416 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Vasodilator Agents) SB - IM UIN - Cochrane Database Syst Rev. 2012 Jul 11;(7):CD006687. doi: 10.1002/14651858.CD006687.pub3. PMID: 22786498 MH - Administration, Oral MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Vasodilator Agents/*administration & dosage RF - 104 EDAT- 2008/04/22 09:00 MHDA- 2008/06/14 09:00 CRDT- 2008/04/22 09:00 PHST- 2008/04/22 09:00 [pubmed] PHST- 2008/06/14 09:00 [medline] PHST- 2008/04/22 09:00 [entrez] AID - 10.1002/14651858.CD006687.pub2 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2008 Apr 16;(2):CD006687. doi: 10.1002/14651858.CD006687.pub2. PMID- 31331136 OWN - NLM STAT- MEDLINE DCOM- 20190814 LR - 20220409 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 66 IP - 6 DP - 2018 Jun TI - Systemic Sclerosis: A Clinical Analysis of Foot Findings in 52 Cases. PG - 49-51 AB - INTRODUCTION: The diagnosis of systemic sclerosis is mostly clinical and is identified by the various cutaneous changes. The cutaneous changes in systemic sclerosis are already well established and supported by a large number of studies and textbooks. Very few literatures describe foot findings in patients with systemic sclerosis. This study was aimed at identifying the various changes in plantar aspect of foot of patients with systemic sclerosis. METHODS: All patients diagnosed with systemic sclerosis were recruited. Apart from clinical examination, X - ray of both feet was done in all the patients. RESULTS: A total of 52 patients were recruited in the study period. Most of the patients (86.54%) were females and the mean age at presentation was 34.19 years. A history of Raynaud's phenomenon was present in 47(90.38%) patients. The foot findings were digital pitted scars in 44(84.61%) patients, macular hyperpigmentation in 40(76.92%) patients and corns in weight bearing areas were found in 40 (76.92%) cases. CONCLUSIONS: In our study corn was a common finding in the foot of patients of systemic sclerosis which has not been routinely documented. CI - © Journal of the Association of Physicians of India 2011. FAU - Patro, Nibedita AU - Patro N AD - Associate Professor, Hi-Tech Medical College & Hospital, Bhubaneshwar, Orissa; Corresponding Author. FAU - Panda, Maitreyee AU - Panda M AD - Associate Professor, IMS & SUM Hospital, Bhubaneshwar, Orissa. FAU - Jena, Subhransu Sekhar AU - Jena SS AD - Assistant Professor, Hi-Tech Medical College & Hospital, Bhubaneshwar, Orissa. LA - eng PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 MH - Adult MH - Female MH - Foot MH - Humans MH - *Raynaud Disease MH - *Scleroderma, Systemic MH - Skin EDAT- 2019/07/25 06:00 MHDA- 2019/08/15 06:00 CRDT- 2019/07/24 06:00 PHST- 2019/07/24 06:00 [entrez] PHST- 2019/07/25 06:00 [pubmed] PHST- 2019/08/15 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2018 Jun;66(6):49-51. PMID- 18238768 OWN - NLM STAT- MEDLINE DCOM- 20080221 LR - 20190101 IS - 1535-2900 (Electronic) IS - 1079-2082 (Linking) VI - 65 IP - 4 DP - 2008 Feb 15 TI - Treatment of complications associated with systemic sclerosis. PG - 315-21 LID - 10.2146/ajhp070024 [doi] AB - PURPOSE: Current and emerging drug therapy options for patients suffering from the complications of systemic sclerosis are presented. SUMMARY: Systemic sclerosis is a devastating and rare, chronic, autoimmune disease and is characterized by various disease complications due to skin thickening, vascular damage, and inflammation affecting numerous organs. There are two major subtypes of systemic sclerosis: limited cutaneous scleroderma and diffuse cutaneous scleroderma. Patients suffer from Raynaud's phenomenon, skin changes, musculoskeletal changes, gastrointestinal complications, pulmonary complications, scleroderma renal crisis, and dryness of the eyes and mouth. Currently, there is no cure for systemic sclerosis, but research is focusing on decreasing the progression and symptoms of this disease. Raynaud's phenomenon is the temporary vasoconstriction of the small vessels of the fingers, toes, tip of the nose, and earlobes. Skin thickening is the cardinal symptom of systemic sclerosis, with as many as 50% of patients developing digital ulcers. Care of these ulcers is crucial in the prevention of osteomyelitis and other infections. Malabsorption syndrome may also occur in patients, many of whom will eventually require parenteral nutrition to maintain their caloric needs. Pulmonary interstitial fibrosis and pulmonary arterial hypertension are additional serious complications of systemic sclerosis. The use of prostacyclin analogues, phosphodiesterase inhibitors, calcium-channel blockers, cyclophosphamide, bosentan, and other agents has been investigated in patients suffering from the complications of systemic sclerosis. CONCLUSION: Systemic sclerosis is characterized by various circulatory, dermatological, gastrointestinal, musculoskeletal, pulmonary, and renal complications. Although there is no cure for systemic sclerosis, management of its associated complications can help improve patients' quality of life. FAU - Moore, Stacie C AU - Moore SC AD - Robert Wood Johnson University Hospital (RWJUH), New Brunswick, NJ, USA. FAU - Desantis, Evelyn R Hermes AU - Desantis ER LA - eng PT - Journal Article PT - Review PL - England TA - Am J Health Syst Pharm JT - American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists JID - 9503023 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Antihypertensive Agents) RN - 0 (Calcium Channel Blockers) RN - 0 (Immunosuppressive Agents) RN - 0 (Phosphodiesterase Inhibitors) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use MH - Antihypertensive Agents/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Epoprostenol/analogs & derivatives/therapeutic use MH - Female MH - Gastrointestinal Diseases/drug therapy MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Joint Diseases/drug therapy MH - Male MH - Muscle Weakness/drug therapy MH - Phosphodiesterase Inhibitors/therapeutic use MH - Pulmonary Fibrosis/drug therapy MH - Quality of Life MH - Raynaud Disease/drug therapy MH - Scleroderma, Systemic/*drug therapy/pathology MH - Sjogren's Syndrome/drug therapy MH - Skin Ulcer/drug therapy RF - 51 EDAT- 2008/02/02 09:00 MHDA- 2008/02/22 09:00 CRDT- 2008/02/02 09:00 PHST- 2008/02/02 09:00 [pubmed] PHST- 2008/02/22 09:00 [medline] PHST- 2008/02/02 09:00 [entrez] AID - 65/4/315 [pii] AID - 10.2146/ajhp070024 [doi] PST - ppublish SO - Am J Health Syst Pharm. 2008 Feb 15;65(4):315-21. doi: 10.2146/ajhp070024. PMID- 21572005 OWN - NLM STAT- MEDLINE DCOM- 20110930 LR - 20200930 IS - 1522-1539 (Electronic) IS - 0363-6135 (Linking) VI - 301 IP - 2 DP - 2011 Aug TI - Impaired transient vasodilation and increased vasoconstriction to digital local cooling in primary Raynaud's phenomenon. PG - H324-30 LID - 10.1152/ajpheart.00246.2011 [doi] AB - Raynaud's phenomenon (RP) is defined as episodic ischemia of the extremities in response to cold. Although the structure of skin capillaries is normal in primary RP, some data suggest impairment of microvascular function. We aimed at testing whether digital skin blood flow was lower in RP than in controls while cooling locally. We further evaluated the contribution of sensory nerves in the response. We recruited 21 patients with primary RP and 20 healthy volunteers matched on age and gender. After a 10-min baseline at 33°C, skin temperature was cooled at 15 or 24°C during 30 min on the forearm and the finger while monitoring perfusion with a custom-design laser Doppler flowmetry probe. Perfusion was also assessed after topical anesthesia. Blood flow was expressed as cutaneous vascular conductance (CVC). Data were subsequently expressed as area above the curve (AAC(0-30)) of the percentage decrease from baseline CVC (%BL). CVC on the dorsum of the finger was lower in RP patients compared with controls at 15°C (AAC(0-30) were 106,237.2 and 69,544.3%BL·s, respectively; P = 0.02) and at 24°C (AAC(0-30) were 86,915 and 57,598%BL·s, respectively; P = 0.04) whereas we observed no significant difference on the finger pad and the forearm. Topical anesthesia increased CVC in patients with RP (P = 0.05), whereas it did not affect reactivity in controls (P = 0.86). Our study shows exaggerated skin microvascular vasoconstriction to local cooling on the dorsum of the finger in primary RP compared with controls. Part of this abnormal response in primary RP depends on sensitive nerves. FAU - Roustit, Matthieu AU - Roustit M AD - Clinical Pharmacology Unit, Inserm CIC03, Grenoble University Hospital, France. MRoustit@chu-grenoble.fr FAU - Blaise, Sophie AU - Blaise S FAU - Millet, Claire AU - Millet C FAU - Cracowski, Jean-Luc AU - Cracowski JL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110513 PL - United States TA - Am J Physiol Heart Circ Physiol JT - American journal of physiology. Heart and circulatory physiology JID - 100901228 RN - 0 (Anesthetics, Combined) RN - 0 (Anesthetics, Local) RN - 0 (Lidocaine, Prilocaine Drug Combination) RN - 0 (Ointments) RN - 046O35D44R (Prilocaine) RN - 98PI200987 (Lidocaine) SB - IM MH - Administration, Cutaneous MH - Adult MH - Analysis of Variance MH - Anesthetics, Combined/administration & dosage MH - Anesthetics, Local/administration & dosage MH - Blood Flow Velocity MH - Case-Control Studies MH - *Cold Temperature MH - Female MH - Fingers/*blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Lidocaine/administration & dosage MH - Lidocaine, Prilocaine Drug Combination MH - Male MH - *Microcirculation/drug effects MH - Microvessels/drug effects/innervation/*physiopathology MH - Middle Aged MH - Ointments MH - Prilocaine/administration & dosage MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow MH - Sensory Receptor Cells/drug effects MH - *Skin Temperature MH - Thermosensing MH - Time Factors MH - *Vasoconstriction/drug effects MH - *Vasodilation/drug effects EDAT- 2011/05/17 06:00 MHDA- 2011/10/01 06:00 CRDT- 2011/05/17 06:00 PHST- 2011/05/17 06:00 [entrez] PHST- 2011/05/17 06:00 [pubmed] PHST- 2011/10/01 06:00 [medline] AID - ajpheart.00246.2011 [pii] AID - 10.1152/ajpheart.00246.2011 [doi] PST - ppublish SO - Am J Physiol Heart Circ Physiol. 2011 Aug;301(2):H324-30. doi: 10.1152/ajpheart.00246.2011. Epub 2011 May 13. PMID- 25293362 OWN - NLM STAT- MEDLINE DCOM- 20170103 LR - 20260128 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 19 IP - 3 DP - 2016 Mar TI - Clinical differences between Thai systemic sclerosis patients with positive versus negative anti-topoisomerase I. PG - 312-20 LID - 10.1111/1756-185X.12492 [doi] AB - BACKGROUND: Anti-topoisomerase I antibody (ATA) carries an increased risk of systemic sclerosis (SSc) internal organ involvement. There have been no published comparisons of the clinical characteristics of patients positive and negative for ATA in Thailand, where the positive rate for ATA is higher than among Caucasians. OBJECTIVE: To define the clinical differences between SSc, positive versus negative, for ATA. METHODS: A retrospective cohort study was performed among SSc patients over 18 at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006-December 2013. SSc-overlap syndrome was excluded. RESULTS: Two hundred and ninety-four SSc patients were included (female : male 2.5 : 1). The majority (68.6%) were the diffuse cutaneous SSc subset (dcSSc). ATA was positive in 252 patients (85.7%), among whom 71.7% had dcSSc and 28.2% limited cutaneous SSc (lcSSc). Using a multivariate analysis, hand deformity had a significantly positive association with ATA (odds ratio [OR] 7.01; 95% CI 1.02-48.69), whereas being anti-centromere (ACA) positive had a negative association (OR 0.17; 95% CI 0.03-0.92). After doing a subgroup analysis of the SSc subset, the median duration of disease at time of pulmonary fibrosis detection among ATA positive dcSSc was significantly shorter than the ATA negative group (1.05 vs. 6.77 years, P = 0.01). Raynaud's phenomenon (RP) at onset was significantly more frequent in lcSSc sufferers who were ATA negative than those who were ATA positive (90.5% vs. 56.9%, P = 0.005). CONCLUSIONS: A high prevalence of ATA positivity was found among Thai SSc patients and this was associated with a high frequency of hand deformity, ACA negativity, a short duration of pulmonary fibrosis in dcSSc and a lower frequency of RP in lcSSc. CI - © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Suwannachat, Prangsuporn AU - Suwannachat P AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Netwijitpan, Sittichai AU - Netwijitpan S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Nanagara, Ratanavadee AU - Nanagara R AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. CN - Scleroderma Research Group LA - eng PT - Comparative Study PT - Journal Article DEP - 20141008 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 5.99.1.2 (TOP1 protein, human) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Chi-Square Distribution MH - DNA Topoisomerases, Type I/*immunology MH - Female MH - Hand Deformities, Acquired/epidemiology/immunology MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Predictive Value of Tests MH - Prevalence MH - Pulmonary Fibrosis/epidemiology/immunology MH - Raynaud Disease/epidemiology/immunology MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Diffuse/blood/diagnosis/epidemiology/*immunology MH - Scleroderma, Limited/blood/diagnosis/epidemiology/*immunology MH - Serologic Tests MH - Thailand/epidemiology MH - Young Adult OTO - NOTNLM OT - anti-Scl70 OT - anti-topoisomerase I OT - autoimmune disease OT - scleroderma OT - systemic sclerosis EDAT- 2014/10/09 06:00 MHDA- 2017/01/04 06:00 CRDT- 2014/10/09 06:00 PHST- 2014/10/09 06:00 [entrez] PHST- 2014/10/09 06:00 [pubmed] PHST- 2017/01/04 06:00 [medline] AID - 10.1111/1756-185X.12492 [doi] PST - ppublish SO - Int J Rheum Dis. 2016 Mar;19(3):312-20. doi: 10.1111/1756-185X.12492. Epub 2014 Oct 8. PMID- 41328832 OWN - NLM STAT- MEDLINE DCOM- 20260401 LR - 20260401 IS - 0300-5283 (Print) IS - 0300-5283 (Linking) VI - 80 IP - 6 DP - 2025 Nov TI - Cross-sectional study on association of autoantibodies and organ involvement in systemic sclerosis patients. PG - 748-752 AB - INTRODUCTION: Systemic sclerosis (SSc) is a connective tissue disease characterised by inflammation, fibrosis, and vascular abnormalities affecting multiple organs, including the skin, lungs, heart and kidneys. Between 75% and 95% of SSc patients have positive SSc-associated autoantibodies. The 2013 European League Against Rheumatism/American College of Rheumatology classification criteria for SSc includes autoantibodies as an important domain, highlighting their importance when clinical manifestations are subtle. However, the association of autoantibodies and specific clinical manifestations vary across different geographical regions and ethnicities, warranting further study across diverse populations. Hence, the objective of this study is to evaluate the association between autoantibodies and organ involvement in SSc patients at a tertiary centre in Malaysia. MATERIALS AND METHODS: This cross-sectional study included 48 SSc patients who received follow-up care at a tertiary centre in Malaysia from July 2013 to June 2023 (a ten-year period). Demographic, clinical, laboratory and radiological information were extracted from patient records. RESULTS: A total of 48 patients were enrolled in our study. Forty-five (93.8%) patients were female and 3 (6.2%) patients were male. Regarding ethnicity, 26 (54.2%) patients were Malay, 17 (35.4%) patients were Chinese and 5 (10.4%) patients were Indian. Mean age at diagnosis was 52.96 years (SD ± 13.99). Thirty-nine (81.2%) patients had limited subtype and 9 (18.8%) patients had diffuse subtype. The most common clinical manifestations were sclerodactyly (97.9%) and Raynaud's phenomenon (79.2%). The most commonly found autoantibodies were anti-Ro-60 (37.5%) and anti-Scl- 70 (33.3%) while anti-Jo-1 (2.1%) was the least detected. Antinuclear antibody (ANA) was detected in 87.5% of our cohort. Anti-Scl-70 was significantly associated with interstitial lung disease (ILD) and ILD progression. Anti- Centromere was significantly associated with telangiectasia and gastroesophageal reflux disease (GERD). Meanwhile, anti-La was associated with synovitis and anti- Ribonucleoprotein (RNP) was associated with microstomia. Twenty-nine (60.4%) patients had evidence of ILD and 11 (22.9%) patients had progressive ILD. Additionally, pulmonary hypertension of varying severity was observed in 14 (29.2%) patients. CONCLUSION: This study supports the well-established association of anti-Scl-70 with ILD and ILD progression. Other unique associations observed in this study could be due to the distinct ethnic and genetic background in the Malaysian population. To gain a more comprehensive understanding of these unique autoantibody-clinical manifestation patterns in Malaysian SSc patients, larger multicentre studies are recommended. FAU - Cheok, L H AU - Cheok LH AD - Hospital Tuanku Jaafar Seremban, Department of Internal Medicine, Rheumatology Unit, Ministry of Health, Malaysia. hock977@hotmail.com. FAU - Goh, W C AU - Goh WC AD - Hospital Tuanku Jaafar Seremban, Department of Internal Medicine, Rheumatology Unit, Ministry of Health, Malaysia. FAU - Chandran, M AU - Chandran M AD - Hospital Tuanku Jaafar Seremban, Department of Internal Medicine, Rheumatology Unit, Ministry of Health, Malaysia. FAU - Nadiah, M N AU - Nadiah MN AD - Hospital Tuanku Jaafar Seremban, Department of Internal Medicine, Rheumatology Unit, Ministry of Health, Malaysia. FAU - Asmah, M AU - Asmah M AD - Hospital Tuanku Jaafar Seremban, Department of Internal Medicine, Rheumatology Unit, Ministry of Health, Malaysia. LA - eng PT - Journal Article PL - Malaysia TA - Med J Malaysia JT - The Medical journal of Malaysia JID - 0361547 RN - 0 (Autoantibodies) RN - 0 (RO60 protein, human) RN - 0 (SS-A Antigen) RN - 0 (Scl 70 antigen, human) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - 0 (Nuclear Proteins) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) SB - IM MH - Cross-Sectional Studies MH - *Autoantibodies/blood/immunology MH - *Scleroderma, Systemic/blood/ethnology/immunology MH - Malaysia MH - Humans MH - Male MH - Female MH - Adult MH - Middle Aged MH - Aged MH - *Lung Diseases, Interstitial/blood/ethnology/immunology MH - *Scleroderma, Localized/blood/ethnology/immunology MH - *Raynaud Disease/blood/ethnology/immunology MH - SS-A Antigen/immunology MH - DNA Topoisomerases, Type I/immunology MH - Nuclear Proteins/immunology MH - Histidine-tRNA Ligase MH - *Hypertension, Pulmonary/blood/ethnology/immunology EDAT- 2025/12/02 12:56 MHDA- 2025/12/02 12:57 CRDT- 2025/12/02 08:04 PHST- 2025/12/02 12:57 [medline] PHST- 2025/12/02 12:56 [pubmed] PHST- 2025/12/02 08:04 [entrez] PST - ppublish SO - Med J Malaysia. 2025 Nov;80(6):748-752. PMID- 27039996 OWN - NLM STAT- MEDLINE DCOM- 20160608 LR - 20160404 IS - 0030-6002 (Print) IS - 0030-6002 (Linking) VI - 157 IP - 15 DP - 2016 Apr 10 TI - [The shades of anti-Jo1 positive antisynthetase syndrome in a Hungarian cohort]. PG - 575-83 LID - 10.1556/650.2016.30400 [doi] AB - INTRODUCTION: In idiopathic inflammatory myopathies, the presence of anti-Jo-1 antibody defines a distinct clinical phenotype (myositis, arthritis, interstitial lung disease, Raynaud's phenomenon fever, mechanic's hands), called antisynthetase syndrome. AIM: To determine the demographic data as well as clinical, laboratory and terapeutical features of anti-Jo1 positive patients, followed by the department of the authors. METHOD: The medical records of 49 consecutive anti-Jo1 patients were reviewed. RESULTS: Demographic and clinical results were very similar to those published by other centers. Significant correlation was found between the anti-Jo-1 titer and the creatine kinase and C-reactive protein levels. Distinct laboratory results measured at the time of diagnosis of the disease (C-reactive protein, antigen A associated with Sjogren's syndrome, positive rheumatoid factor), and the presence of certain clinical symptoms (fever, vasculitic skin) may indicate a worse prognosis within the antisyntetase positive patient group. CONCLUSION: In the cases above more agressive immunosuppressive therapy may be required. FAU - Szabó, Katalin AU - Szabó K AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. FAU - Nagy-Vincze, Melinda AU - Nagy-Vincze M AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. FAU - Bodoki, Levente AU - Bodoki L AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. FAU - Hodosi, Katalin AU - Hodosi K AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. FAU - Dankó, Katalin AU - Dankó K AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. FAU - Griger, Zoltán AU - Griger Z AD - Klinikai Immunológiai Tanszék, Debreceni Egyetem, Klinikai Központ Debrecen, Móricz Zs. út 22., 4032. LA - hun PT - English Abstract PT - Journal Article TT - Az anti-Jo-1-pozitív antiszintetáz szindróma jellegzetességei gondozott betegeink alapján. PL - Hungary TA - Orv Hetil JT - Orvosi hetilap JID - 0376412 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antinuclear) RN - 0 (Antirheumatic Agents) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) RN - 4F4X42SYQ6 (Rituximab) RN - 9007-41-4 (C-Reactive Protein) RN - EC 2.7.3.2 (Creatine Kinase) RN - Antisynthetase syndrome SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Aged MH - Antibodies, Antinuclear/*immunology MH - Antirheumatic Agents/therapeutic use MH - Arthritis/immunology MH - Autoantibodies/*blood MH - C-Reactive Protein/metabolism MH - Creatine Kinase/blood MH - Dermatomyositis/immunology MH - Female MH - Fever/immunology MH - Humans MH - Hungary MH - Immunoglobulins, Intravenous/therapeutic use MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/immunology MH - Male MH - Medical Records MH - Middle Aged MH - Myositis/drug therapy/*immunology/pathology/physiopathology MH - Polymyositis/immunology MH - Raynaud Disease/immunology MH - Retrospective Studies MH - Rituximab/therapeutic use MH - Severity of Illness Index MH - Sjogren's Syndrome/immunology MH - Vasculitis/immunology OTO - NOTNLM OT - anti-Jo-1 OT - anti-Jo1 OT - antisynthetase syndrome OT - antiszintetáz-szindróma OT - dermatomyositis OT - polymyositis EDAT- 2016/04/05 06:00 MHDA- 2016/06/09 06:00 CRDT- 2016/04/05 06:00 PHST- 2016/04/05 06:00 [entrez] PHST- 2016/04/05 06:00 [pubmed] PHST- 2016/06/09 06:00 [medline] AID - 10.1556/650.2016.30400 [doi] PST - ppublish SO - Orv Hetil. 2016 Apr 10;157(15):575-83. doi: 10.1556/650.2016.30400. PMID- 20028729 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20191210 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 3 DP - 2010 Mar TI - Reliability of dermoscopy in the assessment of patients with Raynaud's phenomenon. PG - 542-7 LID - 10.1093/rheumatology/kep408 [doi] AB - OBJECTIVE: Few rheumatologists have access to wide field or video capillaroscopy (abnormal capillaries being highly predictive of CTD), and a key question is whether they should, therefore, purchase a dermatoscope. The aim of this study was to estimate the inter- and intra-observer variability of dermoscopy (magnification 10x) among rheumatologists with little or no experience of the technique. Good reliability is a necessary prerequisite for a test to be a valid clinical or research tool. METHODS: Dermoscopy was performed in all 10 nail folds from 16 subjects with a range of capillary normality/abnormality. The 160 nail fold images thus acquired were made into two PowerPoint presentations, each of 80 images with 16 duplicate slides. Each participating rheumatologist graded one of the sets of 96 images, grading scale (0-3): normal, mildly abnormal/'suspicious', definitely abnormal, grossly abnormal capillaries or 'unclassifiable' when capillaries could not be adequately identified. Data from both presentations were pooled for analysis. RESULTS: Twenty-eight rheumatologists participated in the study. For the decision as to whether an image could be classified or not, the inter- and intra-observer kappa-coefficients were 0.59 (95% CI 0.51, 0.67) and 0.63 (95% CI 0.45, 0.74), respectively. Conditional on being able to classify, the intra-class correlation coefficient for inter- and intra-observer reliability was 0.72 (95% CI 0.66, 0.77) and 0.85 (95% CI 0.82, 0.92), respectively. CONCLUSIONS: Inter- and intra-observer reliability were good, suggesting that with little training, dermoscopy is likely to be a useful technique to identify capillary distortions/underlying CTD. FAU - Moore, Tonia L AU - Moore TL AD - Salford Royal NHS Foundation Trust, Salford M6 8HD, UK. FAU - Roberts, Christopher AU - Roberts C FAU - Murray, Andrea K AU - Murray AK FAU - Helbling, Ingrid AU - Helbling I FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Evaluation Study PT - Journal Article DEP - 20091222 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Capillaries/pathology MH - Clinical Competence MH - Dermoscopy/methods/*standards MH - Epidemiologic Methods MH - Humans MH - Nails/blood supply MH - Observer Variation MH - Raynaud Disease/*diagnosis EDAT- 2009/12/24 06:00 MHDA- 2010/12/14 06:00 CRDT- 2009/12/24 06:00 PHST- 2009/12/24 06:00 [entrez] PHST- 2009/12/24 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - kep408 [pii] AID - 10.1093/rheumatology/kep408 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Mar;49(3):542-7. doi: 10.1093/rheumatology/kep408. Epub 2009 Dec 22. PMID- 17571265 OWN - NLM STAT- MEDLINE DCOM- 20090729 LR - 20220331 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 28 IP - 1 DP - 2007 Nov TI - Neurological manifestations of connective tissue diseases mimicking multiple sclerosis. PG - 15-20 AB - The objective of the study was to analyze retrospectively the clinical, laboratory and imaging findings of multiple sclerosis (MS), such as the manifestations in a cohort of 132 patients referred to the neurology in and outpatient clinic. The proposed clinical and laboratory diagnostic criteria for MS and connective tissue disorders were systematically assessed in 132 consecutive patients. Cerebrospinal fluid serology and brain or spinal cord MRI were studied in all cases. In patients suspected for connective tissue disorder, schirmer test, rose bengal staining and biopsy of minor salivary glands were performed. A total of 115 (87%) patients were diagnosed to have definite MS, while 17 (13%) were diagnosed to have connective tissue disorder. Positive neurological and MRI findings were observed in both groups. The majority of patients with connective tissue disorder demonstrated extra-neurological manifestations like Raynaud's phenomenon, arthritis, livedo reticularis, purpura and presence of multiple autoantibodies in their sera. All patients with MS should be screened systematically for connective tissue disorder. In the absence of pathognomonic clinical and laboratory findings, the diagnosis of MS is a diagnosis of exclusion. FAU - Pelidou, Sigliti-Henrietta AU - Pelidou SH AD - Department of Neurology, University of Ioannina School of Medicine, University Campus, Ioannina 45110, Greece. FAU - Giannopoulos, Sotiris AU - Giannopoulos S FAU - Tzavidi, Sotiria AU - Tzavidi S FAU - Tsifetaki, Niki AU - Tsifetaki N FAU - Kitsos, Georgios AU - Kitsos G FAU - Stefanou, Dimitrios AU - Stefanou D FAU - Kostadima, Vassiliki AU - Kostadima V FAU - Drosos, Alexandros A AU - Drosos AA FAU - Kyritsis, Athanassios P AU - Kyritsis AP LA - eng PT - Journal Article DEP - 20070615 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Arthritis/pathology MH - Autoantibodies/blood MH - Brain/diagnostic imaging/*pathology MH - Cohort Studies MH - Connective Tissue Diseases/*diagnosis/pathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Livedo Reticularis/pathology MH - Magnetic Resonance Imaging/methods MH - Male MH - Middle Aged MH - Multiple Sclerosis/cerebrospinal fluid/*diagnosis/pathology MH - Radiography MH - Raynaud Disease/pathology MH - Serologic Tests MH - Spinal Cord/diagnostic imaging/*pathology MH - Young Adult EDAT- 2007/06/16 09:00 MHDA- 2009/07/30 09:00 CRDT- 2007/06/16 09:00 PHST- 2007/01/30 00:00 [received] PHST- 2007/05/22 00:00 [accepted] PHST- 2007/06/16 09:00 [pubmed] PHST- 2009/07/30 09:00 [medline] PHST- 2007/06/16 09:00 [entrez] AID - 10.1007/s00296-007-0384-8 [doi] PST - ppublish SO - Rheumatol Int. 2007 Nov;28(1):15-20. doi: 10.1007/s00296-007-0384-8. Epub 2007 Jun 15. PMID- 25750566 OWN - NLM STAT- MEDLINE DCOM- 20160329 LR - 20220408 IS - 2005-6648 (Electronic) IS - 1226-3303 (Print) IS - 1226-3303 (Linking) VI - 30 IP - 2 DP - 2015 Mar TI - Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. PG - 232-41 LID - 10.3904/kjim.2015.30.2.232 [doi] AB - BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 ± 1.245 vs. 1.00 ± 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients. FAU - Min, Hong Ki AU - Min HK AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Lee, Jae Ho AU - Lee JH AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Jung, Seung Min AU - Jung SM AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Lee, Jennifer AU - Lee J AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Kang, Kwi Young AU - Kang KY AD - Division of Rheumatology, Department of Internal Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea. FAU - Kwok, Seung-Ki AU - Kwok SK AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Ju, Ji Hyeon AU - Ju JH AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Park, Kyung-Su AU - Park KS AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. FAU - Park, Sung-Hwan AU - Park SH AD - Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. LA - eng PT - Journal Article DEP - 20150227 PL - Korea (South) TA - Korean J Intern Med JT - The Korean journal of internal medicine JID - 8712418 SB - IM MH - Adolescent MH - Adult MH - Cardiomegaly/diagnosis/epidemiology MH - Chi-Square Distribution MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/*mortality MH - Kaplan-Meier Estimate MH - Lung Diseases, Interstitial/diagnosis/mortality MH - Lupus Erythematosus, Systemic/diagnosis/*mortality MH - Lupus Nephritis/diagnosis/mortality MH - Male MH - Multivariate Analysis MH - Prognosis MH - Proportional Hazards Models MH - Raynaud Disease/diagnosis/epidemiology MH - Republic of Korea MH - Retrospective Studies MH - Risk Factors MH - Young Adult PMC - PMC4351331 OTO - NOTNLM OT - Hypertension, pulmonary OT - Lupus erythematosus, systemic OT - Mortality OT - Survival rate COIS- No potential conflict of interest relevant to this article was reported. EDAT- 2015/03/10 06:00 MHDA- 2016/03/30 06:00 PMCR- 2015/03/01 CRDT- 2015/03/10 06:00 PHST- 2014/03/01 00:00 [received] PHST- 2014/03/26 00:00 [revised] PHST- 2014/04/01 00:00 [accepted] PHST- 2015/03/10 06:00 [entrez] PHST- 2015/03/10 06:00 [pubmed] PHST- 2016/03/30 06:00 [medline] PHST- 2015/03/01 00:00 [pmc-release] AID - 10.3904/kjim.2015.30.2.232 [doi] PST - ppublish SO - Korean J Intern Med. 2015 Mar;30(2):232-41. doi: 10.3904/kjim.2015.30.2.232. Epub 2015 Feb 27. PMID- 27421219 OWN - NLM STAT- MEDLINE DCOM- 20170707 LR - 20220419 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 30 IP - 1 DP - 2016 Feb TI - Mixed connective tissue disease. PG - 95-111 LID - S1521-6942(16)00014-0 [pii] LID - 10.1016/j.berh.2016.03.002 [doi] AB - The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3.8 per 100,000 and the mean annual incidence to be 2.1 per million per year, supporting the notion that MCTD is the least common CTD. Little is known about the etiology of MCTD, but recent genetic studies have confirmed that MCTD is a strongly HLA (​human leukocyte antigen)-linked disease, as the HLA profiles of MCTD differ distinctly from the corresponding profiles of ethnically matched healthy controls and other CTDs. In the first section of this review, we provide an update on the clinical, immunological, and genetic features of MCTD and discuss the relationship between MCTD and the other CTDs. Then we proceed to discuss the recent advances in therapy and our current understanding of prognosis and prognostic factors, especially those that are associated with the more serious pulmonary and cardiovascular complications of the disease. In the final section, we discuss some of the key, unresolved questions related to anti-RNP-associated diseases and indicate how these questions may be approached in future studies. CI - Copyright © 2016. Published by Elsevier Ltd. FAU - Gunnarsson, Ragnar AU - Gunnarsson R AD - Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway. Electronic address: ragunnar@gmail.com. FAU - Hetlevik, Siri Opsahl AU - Hetlevik SO AD - Institute of Clinical Medicine, University of Oslo, Oslo, Norway. FAU - Lilleby, Vibke AU - Lilleby V AD - Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway. FAU - Molberg, Øyvind AU - Molberg Ø AD - Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway. LA - eng PT - Journal Article PT - Review DEP - 20160412 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Antibodies, Antinuclear) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM MH - Antibodies, Antinuclear/*immunology MH - Humans MH - Hypertension, Pulmonary/diagnosis MH - Mixed Connective Tissue Disease/*diagnosis MH - Myositis/diagnosis MH - Prognosis MH - Raynaud Disease/diagnosis MH - Ribonucleoprotein, U1 Small Nuclear/*immunology OTO - NOTNLM OT - Dermatomyositis OT - Genetics OT - Interstitial pulmonary fibrosis OT - Mixed connective tissue disease OT - Myositis OT - Polymyositis OT - Pulmonary hypertension OT - Systemic lupus erythematosus OT - Systemic sclerosis OT - U1 small nuclear ribonucleoprotein EDAT- 2016/07/17 06:00 MHDA- 2017/07/08 06:00 CRDT- 2016/07/17 06:00 PHST- 2016/07/17 06:00 [entrez] PHST- 2016/07/17 06:00 [pubmed] PHST- 2017/07/08 06:00 [medline] AID - S1521-6942(16)00014-0 [pii] AID - 10.1016/j.berh.2016.03.002 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2016 Feb;30(1):95-111. doi: 10.1016/j.berh.2016.03.002. Epub 2016 Apr 12. PMID- 17659501 OWN - NLM STAT- MEDLINE DCOM- 20071212 LR - 20101118 IS - 0393-5590 (Print) IS - 0393-5590 (Linking) VI - 24 IP - 4 DP - 2007 Jul-Aug TI - [Renal involvement in systemic sclerosis]. PG - 295-310 AB - Systemic sclerosis is an autoimmune disease characterized by fibrosis of the skin and internal organs. Raynaud's phenomenon generally precedes other disease manifestations. The distribution of skin lesions and the internal organ involvement are the basis for the classification into limited and diffuse forms of the disease. Clinically evident renal disease is observed in 10-40% of patients. The most common renal presentation is renal crisis, characterized by acute onset of renal failure and severe hypertension; some patients remain normotensive, showing microangiopathic hemolytic anemia. Renal complications due to penicillamine may occur in some patients. Finally, ANCA-associated glomerulonephritis is a rare complication of the disorder. In spite of treatment with ACE inhibitors, 20-50% of patients with renal crisis progress to end-stage renal disease. In the absence of a specific therapy, there is accumulating evidence supporting the effectiveness of prostacyclin derivatives, antifibrotic and immunosuppressive drugs. The evidence is strong that the ACE inhibitors that are used in renal crisis are disease modifying. In our series including 193 patients with systemic sclerosis, renal involvement was observed in 19 patients; 11 presented renal crisis (hypertensive in 8; normotensive in 3); 5 had chronic nephropathy; 2 developed penicillamine-induced nephrotic syndrome, and 1 ANCA-associated glomerulonephritis. Renal disease occurs in a minority of patients with systemic sclerosis, and may have a variable clinicopathological picture. As renal involvement is associated with a worse prognosis, careful monitoring of blood pressure, urine chemistry and renal function is required, particularly in patients with diffuse skin disease. FAU - Guerini, S AU - Guerini S AD - Cattedra e Divisione di Nefrologia, Università e Spedali Civili, Brescia. FAU - Cavazzana, I AU - Cavazzana I FAU - Venturelli, C AU - Venturelli C FAU - Rozzi, M AU - Rozzi M FAU - Turina, S AU - Turina S FAU - Sottini, L AU - Sottini L FAU - Tardanico, R AU - Tardanico R FAU - Franceschini, F AU - Franceschini F FAU - Scolari, F AU - Scolari F LA - ita PT - English Abstract PT - Journal Article PT - Review TT - Sclerosi sistemica e coinvolgimento renale. PL - Italy TA - G Ital Nefrol JT - Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia JID - 9426434 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Autoantibodies) SB - IM MH - Acute Kidney Injury/diagnosis/epidemiology/*immunology/pathology/physiopathology/therapy MH - Anemia, Hemolytic/etiology MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Autoantibodies/blood MH - Diagnosis, Differential MH - Humans MH - Hypertension/etiology MH - Italy/epidemiology MH - Kidney/*pathology/*physiopathology MH - Kidney Failure, Chronic/etiology MH - Microcirculation MH - Prognosis MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/immunology/pathology/physiopathology/therapy RF - 108 EDAT- 2007/07/31 09:00 MHDA- 2007/12/13 09:00 CRDT- 2007/07/31 09:00 PHST- 2007/07/31 09:00 [pubmed] PHST- 2007/12/13 09:00 [medline] PHST- 2007/07/31 09:00 [entrez] PST - ppublish SO - G Ital Nefrol. 2007 Jul-Aug;24(4):295-310. PMID- 39585725 OWN - NLM STAT- MEDLINE DCOM- 20250701 LR - 20260525 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - 7 DP - 2025 Jul 1 TI - Pulmonary involvement in systemic sclerosis: can sex play a role? PG - 4320-4324 LID - 10.1093/rheumatology/keae639 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a rare and complex autoimmune disease with significant complications. During the past few years, research interest has focused on the differences between female and male patients. However, there is a lack of data regarding the role of sex in the presence of small airway disease (SAD). Therefore, we aimed to investigate the impact of sex on pulmonary involvement in a cohort of SSc patients, focusing primarily on small airways. METHODS: For this retrospective study, consecutive patients with a diagnosis of SSc that visited our department up to January 2024 were recruited. Demographic data, disease manifestations, serological profile and internal organ involvement were compared between the two groups. RESULTS: In total, 393 patients (female-to-male ratio 6:1) were included. Median time to diagnosis after the onset of Raynaud's was significantly longer for female patients. Electrocardiogram abnormalities were more common in male patients, while telangiectasias, calcinosis and arthralgias were more common in female individuals. Moreover, male SSc patients exhibited a higher prevalence of ILD and shorter time until the diagnosis of ILD. However, female individuals demonstrated a lower maximal-mid expiratory flow rate (MMEF) and higher ratio of residual volume to total lung capacity. Interestingly, sex remained an independent predictor of MMEF, in the multivariate analysis. CONCLUSION: Our results showed that, although ILD is more common in male SSc patients, SAD was more prevalent among female individuals. Importantly, female sex remained an independent predictor of SAD. This study further supports the presence of sex-related differences in SSc, with important implications in disease course and management. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Koletsos, Nikolaos AU - Koletsos N AUID- ORCID: 0000-0001-6976-2802 AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. FAU - Kaltsonoudis, Evripidis AU - Kaltsonoudis E AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. FAU - Trentzidis, Konstantinos AU - Trentzidis K AD - Radiology Department, Theageneio Anticancer Hospital, Thessaloniki, Greece. FAU - Pelechas, Elftherios AU - Pelechas E AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. FAU - Gerolymatou, Nafsika AU - Gerolymatou N AUID- ORCID: 0000-0001-5332-9318 AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. FAU - Memi, Tereza AU - Memi T AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. FAU - Voulgari, Paraskevi V AU - Voulgari PV AUID- ORCID: 0000-0002-5193-2284 AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Male MH - Female MH - *Scleroderma, Systemic/complications/physiopathology/epidemiology MH - Retrospective Studies MH - Middle Aged MH - Sex Factors MH - Adult MH - Aged MH - *Lung Diseases, Interstitial/epidemiology/etiology MH - Raynaud Disease/etiology OTO - NOTNLM OT - interstitial lung disease OT - sex differences OT - small airway disease OT - systemic sclerosis EDAT- 2024/11/25 12:32 MHDA- 2025/07/02 00:27 CRDT- 2024/11/25 11:43 PHST- 2024/08/15 00:00 [received] PHST- 2024/11/15 00:00 [accepted] PHST- 2025/07/02 00:27 [medline] PHST- 2024/11/25 12:32 [pubmed] PHST- 2024/11/25 11:43 [entrez] AID - 7908402 [pii] AID - 10.1093/rheumatology/keae639 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Jul 1;64(7):4320-4324. doi: 10.1093/rheumatology/keae639. PMID- 40156631 OWN - NLM STAT- MEDLINE DCOM- 20250515 LR - 20251205 IS - 1591-9528 (Electronic) IS - 1591-8890 (Print) IS - 1591-8890 (Linking) VI - 25 IP - 1 DP - 2025 Mar 29 TI - Undifferentiated connective tissue disease: the diagnoses critically revised-experience of a single center. PG - 100 LID - 10.1007/s10238-025-01614-1 [doi] LID - 100 AB - Although anti-nuclear antibodies (ANA) are considered the main entry criteria for a diagnosis of undifferentiated connective tissue disease (UCTD), many patients show different rate of ANA positivity and questionable diagnoses. Aim of the study was to revise the UCTD diagnoses and analyse the main predictors of evolution in a monocentric cohort. We retrospectively revised the diagnoses of 331 ANA positive patients, with at least one year of follow-up, classified as UCTD from 2009 and 2017. The diagnosis of UCTD was confirmed in 180 cases (54.4%). The evolution occurred in 18% of cases, after a follow-up of 6.9 (SD: 4.4) years. Raynaud's phenomenon (RP) (OR: 2.39), puffy hands (OR: 6.3), anti-ENA (OR: 2.34), anti-Topoisomerase I antibodies (OR: 4.93), rheumatoid factor (RF) (OR: 2.86) were associated with evolution. Evolution in Systemic Lupus Erythematosus (SLE) occurred in 5 patients (2.78%) and associated with the addition of new autoantibodies, compared with other evolutions (p: 0.034; OR: 12; 95CI: 1.4-103.4). Evolution in Systemic Sclerosis and pSS was found in 14 (7.8%) and 8 cases (4.4%), respectively. Puffy hands and RF positivity as the predictors of SSc and pSS evolution, respectively. A confirmed diagnosis of UCTD, according with the available criteria, was assessed in about a half patients. The occurrence of puffy hands since the onset defines a patient with a potential evolution into SSc, while the addition of new specific autoantibodies represents a typical "fingerprint" of patients developing SLE. Trial registration: Studio ANACTD np 1318. CI - © 2025. The Author(s). FAU - Cavazzana, Ilaria AU - Cavazzana I AUID- ORCID: 0000-0002-2757-7120 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. ilaria.cavazzana@unibs.it. FAU - Semeraro, Paolo AU - Semeraro P AUID- ORCID: 0000-0002-7053-3086 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. FAU - Tomasi, Cesare AU - Tomasi C AUID- ORCID: 0000-0002-3699-8744 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. FAU - Kessler, Elena Sofia AU - Kessler ES AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, University of Brescia, Brescia, Italy. FAU - Piantoni, Silvia AU - Piantoni S AUID- ORCID: 0000-0003-0913-0197 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. FAU - Caproli, Alessia AU - Caproli A AUID- ORCID: 0000-0002-8933-7683 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, University of Brescia, Brescia, Italy. FAU - Fredi, Micaela AU - Fredi M AUID- ORCID: 0000-0002-6511-4936 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. FAU - Franceschini, Franco AU - Franceschini F AUID- ORCID: 0000-0003-3678-6124 AD - Rheumatology and Clinical Immunology Unit and Chair, Department of Experimental and Clinical Science, ERN-Reconnect Centre, ASST Spedali Civili, University of Brescia, Brescia, Italy. LA - eng PT - Journal Article DEP - 20250329 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Antibodies, Antinuclear) RN - 9009-79-4 (Rheumatoid Factor) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Male MH - Female MH - Retrospective Studies MH - Middle Aged MH - Adult MH - *Antibodies, Antinuclear/blood MH - *Undifferentiated Connective Tissue Diseases/diagnosis/immunology MH - Aged MH - Rheumatoid Factor/blood MH - Young Adult MH - Lupus Erythematosus, Systemic/diagnosis MH - Raynaud Disease MH - Autoantibodies/blood MH - *Connective Tissue Diseases/diagnosis PMC - PMC11954846 OTO - NOTNLM OT - Anti-nuclear antibodies OT - Raynaud’s phenomenon OT - Systemic lupus erythematosus OT - Systemic sclerosis OT - Undifferentiated connective tissue disease COIS- Declarations. Conflict of interest: The authors have no relevant financial or non-financial interests to disclose. Ethical approval: This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Local Ethics Committee (Trial registration: Studio ANACTD np 1318). Consent to participate: Informed consent was obtained from all individual participants included in the study. EDAT- 2025/03/30 23:01 MHDA- 2025/03/30 23:02 PMCR- 2025/03/29 CRDT- 2025/03/29 12:04 PHST- 2024/12/30 00:00 [received] PHST- 2025/02/24 00:00 [accepted] PHST- 2025/03/30 23:02 [medline] PHST- 2025/03/30 23:01 [pubmed] PHST- 2025/03/29 12:04 [entrez] PHST- 2025/03/29 00:00 [pmc-release] AID - 10.1007/s10238-025-01614-1 [pii] AID - 1614 [pii] AID - 10.1007/s10238-025-01614-1 [doi] PST - epublish SO - Clin Exp Med. 2025 Mar 29;25(1):100. doi: 10.1007/s10238-025-01614-1. PMID- 16882594 OWN - NLM STAT- MEDLINE DCOM- 20061027 LR - 20060802 IS - 0300-9742 (Print) IS - 0300-9742 (Linking) VI - 35 IP - 4 DP - 2006 Jul-Aug TI - Random number generation evaluation in patients with systemic lupus erythematosus indicates a heterogeneous nature of central nervous system vulnerability. PG - 295-9 AB - OBJECTIVE: To evaluate the vulnerability of the central nervous system (CNS) in patients with systemic lupus erythematosus (SLE). METHODS: Forty-eight patients with SLE, 58 with schizophrenia in remission and 39 healthy controls were enrolled in the study. Patients vocally generated 100 numbers in a random fashion, using numbers 0 to 9, and were evaluated with seriality scores. Patients with SLE were subgrouped according to differences in the presence of Raynaud's phenomenon, anti-phospholipid antibody, lupus activity, and a history of neuropsychiatric (NP) lupus, and these patients were also evaluated by comparison with their counterparts. RESULTS: In general, patients with SLE showed lower seriality scores than patients with schizophrenia, and higher seriality scores than normal controls. The scores of the patients with a history of NP lupus matched those with schizophrenia, and the scores of never having NP lupus matched those of the healthy controls. CONCLUSIONS: CNS vulnerability may be prolonged in patients who have a history of NP lupus even when they appear to be in normal NP status. The damage in random number generation (RNG) observed in patients with a history of NP lupus seemed equal to that found in those with schizophrenia, whereas those patients never having NP lupus appeared to be equal to the controls. The current study suggests a heterogeneous nature of SLE and prolonged damage, especially in CNS vulnerability, when evaluating with RNG. FAU - Matsukawa, Y AU - Matsukawa Y AD - Division of Haematology and Rheumatology, Department of Medicine, Nihon University of Medicine, Oyaguchi-Kamimachi, Itabashi, 173-8610, Tokyo, Japan. m-2000@mbk.ocn.ne.jp FAU - Nagashima, M AU - Nagashima M FAU - Kamei, S AU - Kamei S FAU - Tanabe, E AU - Tanabe E FAU - Takahashi, S AU - Takahashi S FAU - Kojima, T AU - Kojima T FAU - Taira, M AU - Taira M FAU - Morita, K AU - Morita K FAU - Matsuura, M AU - Matsuura M FAU - Sawada, S AU - Sawada S LA - eng PT - Journal Article PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Antiphospholipid Syndrome/physiopathology MH - Central Nervous System/*physiopathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*physiopathology MH - Lupus Vasculitis, Central Nervous System/physiopathology MH - Male MH - Middle Aged MH - Raynaud Disease/physiopathology MH - Schizophrenia/physiopathology EDAT- 2006/08/03 09:00 MHDA- 2006/10/28 09:00 CRDT- 2006/08/03 09:00 PHST- 2006/08/03 09:00 [pubmed] PHST- 2006/10/28 09:00 [medline] PHST- 2006/08/03 09:00 [entrez] AID - R0G1548132442682 [pii] AID - 10.1080/03009740600556027 [doi] PST - ppublish SO - Scand J Rheumatol. 2006 Jul-Aug;35(4):295-9. doi: 10.1080/03009740600556027. PMID- 29947047 OWN - NLM STAT- MEDLINE DCOM- 20190624 LR - 20190624 IS - 1600-0625 (Electronic) IS - 0906-6705 (Linking) VI - 27 IP - 9 DP - 2018 Sep TI - CXCL13 produced by macrophages due to Fli1 deficiency may contribute to the development of tissue fibrosis, vasculopathy and immune activation in systemic sclerosis. PG - 1030-1037 LID - 10.1111/exd.13724 [doi] AB - CXCL13, a chemokine for B cells, follicular T cells, T helper 17 cells, and regulatory T cells, is reported to contribute to the development of systemic sclerosis (SSc), reflecting aberrant activation of immune system. To better understand the role of CXCL13 in SSc, we investigated the influence of Fli1 deficiency, a potential predisposing factor of this disease, on CXCL13 expression and assessed the clinical correlation of serum CXCL13 levels by multivariate regression analysis. Haploinsufficient loss of Fli1 remarkably induced CXCL13 expression in murine peritoneal macrophages, while gene silencing of FLI1 did not affect the expression of CXCL13 in human dermal fibroblasts and human dermal microvascular endothelial cells. Serum CXCL13 levels were elevated in SSc patients compared with healthy controls and correlated positively with skin score and negatively with pulmonary function test results. SSc patients with elevated serum CXCL13 levels had longer disease duration, diffuse cutaneous involvement, interstitial lung disease (ILD), heart involvement, pulmonary arterial hypertension, Raynaud's phenomenon, pitting scars, digital ulcers, telangiectasia, and high serum IgG levels more frequently than the other patients. In particular, serum CXCL13 levels were associated with ILD and digital ulcers by multivariate regression analysis. Taken together, these results indicate that CXCL13 expression is upregulated by Fli1 deficiency in macrophages, potentially contributing to the development of tissue fibrosis, vasculopathy and immune activation in SSc, especially ILD and digital ulcers. CI - © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Taniguchi, Takashi AU - Taniguchi T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miyagawa, Takuya AU - Miyagawa T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Toyama, Satoshi AU - Toyama S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yamashita, Takashi AU - Yamashita T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Nakamura, Kouki AU - Nakamura K AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Saigusa, Ryosuke AU - Saigusa R AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Ichimura, Yohei AU - Ichimura Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Takahashi, Takehiro AU - Takahashi T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Toyama, Tetsuo AU - Toyama T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yoshizaki, Ayumi AU - Yoshizaki A AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Sato, Shinichi AU - Sato S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Asano, Yoshihide AU - Asano Y AUID- ORCID: 0000-0001-5560-9778 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180729 PL - Denmark TA - Exp Dermatol JT - Experimental dermatology JID - 9301549 RN - 0 (CXCL13 protein, human) RN - 0 (Chemokine CXCL13) RN - 0 (Cxcl13 protein, mouse) RN - 0 (FLI1 protein, human) RN - 0 (Fli1 protein, mouse) RN - 0 (Lipopolysaccharides) RN - 0 (Proto-Oncogene Protein c-fli-1) RN - 0 (RNA, Messenger) SB - IM MH - Aged MH - Animals MH - Cells, Cultured MH - Chemokine CXCL13/*blood/genetics MH - Endothelial Cells MH - Female MH - Fibroblasts MH - Fibrosis MH - Fingers MH - Gene Expression/drug effects/genetics MH - Gene Silencing MH - Humans MH - Lipopolysaccharides/pharmacology MH - Lung/*pathology MH - Lung Diseases, Interstitial/*blood/etiology/physiopathology MH - Macrophages/metabolism MH - Male MH - Mice MH - Middle Aged MH - Proto-Oncogene Protein c-fli-1/*deficiency/genetics/metabolism MH - RNA, Messenger/metabolism MH - Raynaud Disease/blood/etiology MH - Respiratory Function Tests MH - Scleroderma, Systemic/*blood/complications/immunology MH - Skin/*pathology MH - Skin Ulcer/*blood/etiology OTO - NOTNLM OT - CXCL13 OT - Fli1 OT - fibrosis OT - macrophages OT - systemic sclerosis OT - vasculopathy EDAT- 2018/06/28 06:00 MHDA- 2019/06/25 06:00 CRDT- 2018/06/28 06:00 PHST- 2018/02/08 00:00 [received] PHST- 2018/06/13 00:00 [revised] PHST- 2018/06/22 00:00 [accepted] PHST- 2018/06/28 06:00 [pubmed] PHST- 2019/06/25 06:00 [medline] PHST- 2018/06/28 06:00 [entrez] AID - 10.1111/exd.13724 [doi] PST - ppublish SO - Exp Dermatol. 2018 Sep;27(9):1030-1037. doi: 10.1111/exd.13724. Epub 2018 Jul 29. PMID- 21081523 OWN - NLM STAT- MEDLINE DCOM- 20110415 LR - 20260128 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 70 IP - 3 DP - 2011 Mar TI - Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. PG - 476-81 LID - 10.1136/ard.2010.136929 [doi] AB - OBJECTIVE: To identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc). METHODS: A list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores. RESULTS: Physicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily); vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting. CONCLUSION: The three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort. FAU - Avouac, J AU - Avouac J AD - Rheumatology A department, Cochin Hospital, 27 rue du Faubourg Saint Jacques, 75014 Paris, France. javouac@me.com FAU - Fransen, J AU - Fransen J FAU - Walker, U A AU - Walker UA FAU - Riccieri, V AU - Riccieri V FAU - Smith, V AU - Smith V FAU - Muller, C AU - Muller C FAU - Miniati, I AU - Miniati I FAU - Tarner, I H AU - Tarner IH FAU - Randone, S Bellando AU - Randone SB FAU - Cutolo, M AU - Cutolo M FAU - Allanore, Y AU - Allanore Y FAU - Distler, O AU - Distler O FAU - Valentini, G AU - Valentini G FAU - Czirjak, L AU - Czirjak L FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Furst, D E AU - Furst DE FAU - Tyndall, A AU - Tyndall A FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M CN - EUSTAR Group LA - eng PT - Consensus Statement PT - Journal Article DEP - 20101115 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/blood MH - Delphi Technique MH - Diagnosis, Differential MH - Early Diagnosis MH - Edema/etiology MH - Fingers MH - Humans MH - Microscopic Angioscopy MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/complications/*diagnosis/immunology MH - Skin Diseases/etiology EDAT- 2010/11/18 06:00 MHDA- 2011/04/19 06:00 CRDT- 2010/11/18 06:00 PHST- 2010/11/18 06:00 [entrez] PHST- 2010/11/18 06:00 [pubmed] PHST- 2011/04/19 06:00 [medline] AID - S0003-4967(24)14648-1 [pii] AID - 10.1136/ard.2010.136929 [doi] PST - ppublish SO - Ann Rheum Dis. 2011 Mar;70(3):476-81. doi: 10.1136/ard.2010.136929. Epub 2010 Nov 15. PMID- 17329053 OWN - NLM STAT- MEDLINE DCOM- 20070912 LR - 20070604 IS - 0398-0499 (Print) IS - 0398-0499 (Linking) VI - 32 IP - 2 DP - 2007 Apr TI - [Fourier transform spectral analysis of cutaneous blood flux in systemic sclerosis]. PG - 83-9 AB - OBJECTIVES: Endothelial dysfunction is an early event and a critical step in the pathogenesis of systemic sclerosis. Accurate and sensitive tests are needed to correctly assess the degree of microvascular endothelial dysfunction. Spectral analysis of skin blood flow contains a characteristic low frequency reported to be associated with endothelial function in healthy subjects. We hypothesized that the relative amplitude of the oscillation recorded for this low frequency spectrum (0.008 to 0.021 Hz) would be less pronounced in patients with systemic sclerosis than in healthy subjects and in patients with primary Raynaud's phenomenon. PATIENTS AND METHOD: Twenty-one patients with systemic sclerosis, twenty patients with primary Raynaud phenomenon and eleven healthy subjects were enrolled. Skin perfusion was recorded at rest for 30 minutes using laser Doppler flowmetry on the pad of the left third left. Fourier transform spectral analysis was applied to obtain the mean amplitude of the cutaneous blood perfusion signal of the total spectrum from 0.008 to 1.6 Hz and the mean amplitude of each characteristic frequency in the laser Doppler flowmeter blood flow oscillations. RESULTS: The relative amplitudes of each characteristic frequency in the laser Doppler flowmeter blood flow oscillations were not statistically different in the three groups, particularly for frequency spectrum from 0.008 Hz to 0.021 Hz. CONCLUSION: Fourier transform spectral analysis of baseline cutaneous blood flow does not provide significant information. Further studies are required, perhaps using wavelet spectral analysis or stimulated conditions. FAU - Salvat-Melis, M AU - Salvat-Melis M AD - Laboratoire HP2 EA 3745, Inserm ERI 017, université de Grenoble 1 EA 3745, CHU de Grenoble, 38043 Grenoble cedex 09, France. FAU - Carpentier, P-H AU - Carpentier PH FAU - Moreau-Gaudry, A AU - Moreau-Gaudry A FAU - Boignard, A AU - Boignard A FAU - Paris, A AU - Paris A FAU - Cracowski, J-L AU - Cracowski JL LA - fre PT - English Abstract PT - Journal Article TT - Analyse spectrale par transformée de Fourier du flux sanguin cutané dans la sclérodermie systémique. PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - *Blood Flow Velocity MH - Endothelium, Vascular/physiopathology MH - Female MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Oscillometry MH - Pulmonary Fibrosis/epidemiology MH - Raynaud Disease/epidemiology MH - Scleroderma, Systemic/diagnosis/*physiopathology MH - Skin/*blood supply EDAT- 2007/03/03 09:00 MHDA- 2007/09/13 09:00 CRDT- 2007/03/03 09:00 PHST- 2006/10/19 00:00 [received] PHST- 2006/12/13 00:00 [accepted] PHST- 2007/03/03 09:00 [pubmed] PHST- 2007/09/13 09:00 [medline] PHST- 2007/03/03 09:00 [entrez] AID - S0398-0499(07)00109-6 [pii] AID - 10.1016/j.jmv.2006.12.005 [doi] PST - ppublish SO - J Mal Vasc. 2007 Apr;32(2):83-9. doi: 10.1016/j.jmv.2006.12.005. PMID- 33159775 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 0030-9982 (Print) IS - 0030-9982 (Linking) VI - 70 IP - 10 DP - 2020 Oct TI - A rare case of gangrenous foot in juvenile systemic lupus erythematosus. PG - 1869-1873 LID - 10.47391/JPMA.642 [doi] AB - This report describes a rare case of Juvenile Systemic Lupus Erythematosus (JSLE). A young 13-year-old girl presented to the Civil Hospital Karachi on February 15, 2019 with gangrene as the only manifestation of this autoimmune disease. JSLE has several clinical manifestations such as butterfly rash, fever, joint pain, cardiac problems like pericardial infusion and neuropsychiatric disorders. However, in this case gangrene was the only presenting symptom; only laboratory investigations - anti-SSA and anti-ribosomal P protein - were suggestive of JSLE, while anti dsDNA, considered to be the most sensitive and reliable diagnostic tool for Systemic Lupus Erythematosus (SLE), was negative. Raynaud's phenomenon and gangrene have been described as rare symptoms, with gangrene occurring in only a small percentage of SLE patients. Moreover, the patient had received a blood transfusion a few months ago in Hyderabad which was suspected to be the cause of the transmission of infection which lead to polyclonal activation of lymphocytes. FAU - Yasmin, Farah AU - Yasmin F AD - 2nd Year Medical Student, Dow Medical College, Karachi Pakistan. FAU - Jawaid, Haris AU - Jawaid H AD - 2nd Year Medical Student, Dow Medical College, Karachi Pakistan. FAU - Batra, Simran AU - Batra S AD - 3rd Year Medical Student, Dow Medical College, Karachi Pakistan. FAU - Vighio, Ashfaque Ahmed AU - Vighio AA AD - Civil Hospital, Karachi, Pakistan. FAU - Shaikh, Majid Ahmed AU - Shaikh MA AD - Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan. LA - eng PT - Case Reports PT - Journal Article PL - Pakistan TA - J Pak Med Assoc JT - JPMA. The Journal of the Pakistan Medical Association JID - 7501162 RN - 9007-49-2 (DNA) SB - IM MH - Adolescent MH - *Autoimmune Diseases MH - DNA MH - Female MH - Gangrene/etiology MH - Humans MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - *Raynaud Disease OTO - NOTNLM OT - Juvenile Systemic Lupus Erythematosus, Gangrene, Auto-immune, Vasculitis. EDAT- 2020/11/08 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/11/07 17:05 PHST- 2020/11/07 17:05 [entrez] PHST- 2020/11/08 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] AID - 10178 [pii] AID - 10.47391/JPMA.642 [doi] PST - ppublish SO - J Pak Med Assoc. 2020 Oct;70(10):1869-1873. doi: 10.47391/JPMA.642. PMID- 21533912 OWN - NLM STAT- MEDLINE DCOM- 20121207 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 6 DP - 2012 Jun TI - Juvenile diffuse systemic sclerosis/systemic lupus erythematosus overlap syndrome--a case report. PG - 1809-11 LID - 10.1007/s00296-011-1937-4 [doi] AB - We report a rare case of diffuse systemic sclerosis (SSc) evolving into diffuse SSc/systemic lupus erythematosus (SLE) overlap syndrome. A 15-year-old boy was diagnosed as diffuse SSc with initial presentations of Raynaud's phenomenon and skin tightening. He underwent Chinese herbal treatment and clinical symptoms deteriorated in the following 3 years. On admission to our ward, serositis with pleural effusion, severe pulmonary fibrosis with moderate pulmonary hypertension, swallowing difficulty, and polyarthritis were observed. Autoantibody profiles revealed concurrence of anti-double-stranded DNA, anti-Smith, anti-topoisomerase I, and anti-ribonucleoprotein antibodies. The patient fulfills the criteria for both diffuse SSc and SLE. After drainage for pleural effusion accompanied by oral prednisolone and sildenafil, there were improvement of respiratory distress, swallowing difficulty, and pulmonary hypertension. In conclusion, connective tissue diseases may overlap with each other during the disease course. Serial follow-up for clinical symptoms as well as serological changes is recommended. FAU - Lin, Heng-Kuei AU - Lin HK AD - Division of Hematology and Oncology, Department of Pediatrics, Taichung Veterans General Hospital, No. 160, Sec. 3, Chung-Kang Rd., Taichung, 40705, Taiwan. linhengkuei@gmail.com FAU - Wang, Jiaan-Der AU - Wang JD FAU - Fu, Lin-Shien AU - Fu LS LA - eng PT - Case Reports PT - Journal Article DEP - 20110501 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antihypertensive Agents) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Drugs, Chinese Herbal) RN - 0 (Glucocorticoids) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 9PHQ9Y1OLM (Prednisolone) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Adolescent MH - Antihypertensive Agents/therapeutic use MH - Arthritis/etiology MH - Autoantibodies/blood MH - Biomarkers/blood MH - Deglutition Disorders/etiology MH - Disease Progression MH - Drainage MH - Drugs, Chinese Herbal/therapeutic use MH - Glucocorticoids/therapeutic use MH - Humans MH - Hypertension, Pulmonary/etiology MH - Lupus Erythematosus, Systemic/*complications/diagnosis/immunology/therapy MH - Male MH - Piperazines/therapeutic use MH - Pleural Effusion/etiology MH - Prednisolone/therapeutic use MH - Pulmonary Fibrosis/etiology MH - Purines/therapeutic use MH - Raynaud Disease/etiology MH - Scleroderma, Diffuse/*complications/diagnosis/immunology/therapy MH - Serositis/etiology MH - Sildenafil Citrate MH - Sulfones/therapeutic use MH - Treatment Outcome EDAT- 2011/05/03 06:00 MHDA- 2012/12/12 06:00 CRDT- 2011/05/03 06:00 PHST- 2010/12/09 00:00 [received] PHST- 2011/03/13 00:00 [accepted] PHST- 2011/05/03 06:00 [entrez] PHST- 2011/05/03 06:00 [pubmed] PHST- 2012/12/12 06:00 [medline] AID - 10.1007/s00296-011-1937-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Jun;32(6):1809-11. doi: 10.1007/s00296-011-1937-4. Epub 2011 May 1. PMID- 35835173 OWN - NLM STAT- MEDLINE DCOM- 20220803 LR - 20250728 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 143 DP - 2022 Sep TI - Nailfold video capillaroscopy as a useful diagnostic tool in systemic vasculitis. PG - 104406 LID - S0026-2862(22)00096-6 [pii] LID - 10.1016/j.mvr.2022.104406 [doi] AB - BACKGROUND: Nailfold video capillaroscopy (NVC) enables us a direct view of the microvasculature. Only several capillaroscopy studies in adult patients with vasculitis have been reported. AIM: To characterize NVC changes in vasculitis. METHODS: Vasculitis patients and healthy controls were evaluated by NVC. NVC changes associated with vasculitis were assessed retrospectively in a cohort of 100 patients with Raynaud's phenomenon (RP). RESULTS: 17 patients with active vasculitis and 8 patients with vasculitis in remission were compared to 25 age and sex-matched healthy controls. Active vasculitis patients demonstrated higher rates of neoangiogenesis and capillary loss in comparison to other groups. Two novel NVC abnormalities were observed in patients with vasculitis: "Rolling" (slow capillary flow) and "peri-capillary stippling" (PCS), small deposits that may represent capillary leak. PCS was observed exclusively in 5 of 17 patients with active vasculitis. Retrospectively, we were able to detect PCS also in 14 % of 100 patients that were evaluated for RP, of whom 64 % were diagnosed with scleroderma or a related disorder. CONCLUSIONS: Patients with active vasculitis demonstrate frequent capillary abnormalities. Although these abnormalities are non-specific, we suggest that their combination may aid the diagnosis of vasculitis. Future studies are needed to validate our findings. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Keret, Shiri AU - Keret S AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Mazzawi, Jan AU - Mazzawi J AD - Internal Medicine A, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Slobodin, Gleb AU - Slobodin G AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Rimar, Ori AU - Rimar O AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Rosner, Itzhak AU - Rosner I AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Rozenbaum, Michael AU - Rozenbaum M AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Kaly, Lisa AU - Kaly L AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Boulman, Nina AU - Boulman N AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Awisat, Abid AU - Awisat A AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Shouval, Aniela AU - Shouval A AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Clinical Rheumatology, Istituto Gaetano Pini, Dept of Clinical Sciences & Community Health, Università degli Studi di Milano, Milano, Italy. FAU - Rimar, Doron AU - Rimar D AD - Rheumatology Unit, Bnai-Zion Medical Center, Faculty of Medicine, Technion, Haifa, Israel. Electronic address: doronrimar@gmail.com. LA - eng PT - Journal Article DEP - 20220711 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Capillaries MH - Humans MH - Microscopic Angioscopy MH - Nails/blood supply MH - *Raynaud Disease/diagnosis MH - Retrospective Studies MH - *Scleroderma, Systemic MH - *Systemic Vasculitis MH - *Vasculitis OTO - NOTNLM OT - ANCA OT - Nailfold video capillaroscopy OT - Peri-capillary stippling OT - Polyarteritis nodosa OT - Rolling OT - Systemic sclerosis OT - Takayasu OT - Vasculitis EDAT- 2022/07/15 06:00 MHDA- 2022/08/04 06:00 CRDT- 2022/07/14 19:32 PHST- 2022/03/26 00:00 [received] PHST- 2022/06/10 00:00 [revised] PHST- 2022/07/06 00:00 [accepted] PHST- 2022/07/15 06:00 [pubmed] PHST- 2022/08/04 06:00 [medline] PHST- 2022/07/14 19:32 [entrez] AID - S0026-2862(22)00096-6 [pii] AID - 10.1016/j.mvr.2022.104406 [doi] PST - ppublish SO - Microvasc Res. 2022 Sep;143:104406. doi: 10.1016/j.mvr.2022.104406. Epub 2022 Jul 11. PMID- 24720644 OWN - NLM STAT- MEDLINE DCOM- 20150608 LR - 20191112 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 10 IP - 1 DP - 2014 TI - The concept of early systemic sclerosis following 2013 ACR\EULAR criteria for the classification of systemic sclerosis. PG - 38-44 AB - For many years observational studies and clinical trials on systemic sclerosis (SSc) have been carried out only on patients who met the 1980 American College of Rheumatology-ACR preliminary classification criteria. However, this lead to the exclusion from all those studies of subset patients, particularly those with a limited cutaneous SSc-sine scleroderma subset, because, despite a diagnosis of SSc based on Raynaud's phenomenon (RP) associated with digital pitting scars/ulcers, or by sclerodactyly and telangiectasia and/or typical esophagopathy and/or interstitial fibrosis detected by High Resolution Computed Tomography of the lungs, they did not satisfy the classification criteria. In that setting, LeRoy and Medsger proposed to label as affected by limited SSc (lSSc) or early SSc, cases presenting with RP associated with an SSc-type nailfold capillary pattern and/or SSc-selective autoantibodies. In 2008, Koenig et al. validated these criteria and proposed to name early SSc, or pre-scleroderma, patients with RP and either a scleroderma marker autoantibody or typical capillaroscopy abnormalities or both, who had been clearly shown to have high probabilities but not the certainty to develop definite SSc during a 20-year follow-up. This definition has been recently challenged by the development of the new ACR/EULAR criteria for the classification of SSc. At present, to be labeled as affected by early SSc, or pre-scleroderma, a patient should suffer from RP associated with either scleroderma marker autoantibodies or typical capillaroscopy findings and should not meet the 2013 ACR/EULAR criteria for the classification of SSc nor should he/she meet the criteria for SSc sine scleroderma. FAU - Valentini, Gabriele AU - Valentini G FAU - Marcoccia, Antonella AU - Marcoccia A FAU - Cuomo, Giovanna AU - Cuomo G FAU - Iudici, Michele AU - Iudici M FAU - Vettori, Serena AU - Vettori S AD - Rheumatology Unit, Second University of Napoli, Via S. Pansini 5, 80131 Napoli, Italy. gabriele.valentini@unina2.it. LA - eng PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/metabolism MH - Humans MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*classification/complications/*diagnosis MH - Time Factors EDAT- 2014/04/12 06:00 MHDA- 2015/06/09 06:00 CRDT- 2014/04/12 06:00 PHST- 2014/01/01 00:00 [received] PHST- 2014/04/20 00:00 [revised] PHST- 2014/05/18 00:00 [accepted] PHST- 2014/04/12 06:00 [entrez] PHST- 2014/04/12 06:00 [pubmed] PHST- 2015/06/09 06:00 [medline] AID - CRR-EPUB-59956 [pii] AID - 10.2174/1573397110666140404001756 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2014;10(1):38-44. doi: 10.2174/1573397110666140404001756. PMID- 33526731 OWN - NLM STAT- MEDLINE DCOM- 20210203 LR - 20220531 IS - 1512-0112 (Print) IS - 1512-0112 (Linking) IP - 309 DP - 2020 Dec TI - COMORBID CONDITION - DIABETES MELLITUS WITH CO-EXISTENT RAYNAUD'S SYNDROME IN PATIENTS WITH RHEUMATOID ARTHRITIS. PG - 59-64 AB - The most common comorbidities in patients with rheumatic diseases include cardiovascular diseases (CVD), liver and biliary tract infection, lung diseases, amyloidosis, fractures of different localizations, malignant neoplasms, metabolic disorders and diabetes mellitus (DM). The aim of study was to investigate the clinical course of DM and rheumatoid arthritis (RS) in patients with RA using laboratory and instrumental research methods. There were examined 85 patients with RA who were treated in the rheumatology department of Ivano-Frankivsk Central City Clinical Hospital. The patients' age ranged from 40 to 70 years. Endothelial dysfunction (ED) signs were observed in 76 (89.4%) patients. ED was diagnosed in all patients with RA, co-existent RS and DM. In the patients with RA and those with RA and co-existent RS, impaired EDVBA was detected. In the patients of Group II, the indicator of EDVBA (6.5±0.2%) was significantly lower as compared to the patients of Group I (8.8±0.3%) (р<0.05). The levels of both CRP and TNF-α, serving as non-specific inflammatory markers, were significantly higher (29.37±3.56 mg/l, p<0.01) in the patients with RS as compared to the patients with RA only (23.89±1.77 mg/l). A detailed study of the pathophysiological and immunological features of the clinical course of secondary RS will allow us to optimize its treatment schemes in patients with RA, reduce clinical and laboratory manifestation of RA and improve quality of life in such patients, especially those with a comorbidity. FAU - Hotiur, O AU - Hotiur O AD - Ivano-Frankivsk National Medical University, Ukraine. FAU - Boichuk, V AU - Boichuk V AD - Ivano-Frankivsk National Medical University, Ukraine. FAU - Skoropad, K AU - Skoropad K AD - Ivano-Frankivsk National Medical University, Ukraine. FAU - Vandzhura, Y AU - Vandzhura Y AD - Ivano-Frankivsk National Medical University, Ukraine. FAU - Bacur, M AU - Bacur M AD - Ivano-Frankivsk National Medical University, Ukraine. LA - eng PT - Journal Article PL - Georgia (Republic) TA - Georgian Med News JT - Georgian medical news JID - 101218222 SB - IM MH - Adult MH - Aged MH - *Arthritis, Rheumatoid/complications/epidemiology MH - *Cardiovascular Diseases MH - Comorbidity MH - *Diabetes Mellitus/epidemiology MH - Humans MH - Middle Aged MH - Quality of Life MH - *Raynaud Disease/complications EDAT- 2021/02/03 06:00 MHDA- 2021/02/04 06:00 CRDT- 2021/02/02 06:03 PHST- 2021/02/02 06:03 [entrez] PHST- 2021/02/03 06:00 [pubmed] PHST- 2021/02/04 06:00 [medline] PST - ppublish SO - Georgian Med News. 2020 Dec;(309):59-64. PMID- 21943540 OWN - NLM STAT- MEDLINE DCOM- 20120127 LR - 20161125 IS - 1776-2588 (Electronic) IS - 0761-8425 (Linking) VI - 28 IP - 7 DP - 2011 Sep TI - [Erasmus' syndrome with pseudo-tumour masses]. PG - 924-7 LID - 10.1016/j.rmr.2011.06.003 [doi] AB - INTRODUCTION: Erasmus' syndrome involves the association of systemic scleroderma (SS) and exposure to silica. Silicosis may precede the SS but the latter may be the presentation, in which case a history of exposure to silica should be sought as part of the diagnosis. CASE REPORT: A 46-year-old man with history of pulmonary tuberculosis presented with dyspnoea and dysphagea. Clinical examination revealed thickening of the facial skin with a pointed nose, erythema and telangiectasia, Raynaud's syndrome and sclerodactyly. A thoracic CT scan revealed bilateral, fibrotic, pseudo-tumoural masses. Antinuclear antibodies, anti-topoisomerase 1 and antihistone were positive. CONCLUSION: The clinical presentation of Erasmus' syndrome associating systemic scleroderma and pulmonary pseudo-tumours may pose a problem of differential diagnosis from lung cancer. This condition requires regular clinical and radiological monitoring, particularly as both scleroderma and silicosis increase the risk of lung cancer. CI - Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved. FAU - Chaouch, N AU - Chaouch N AD - Service de pneumologie, pavillon 2, hôpital A.-Mami, 2080 Ariana, Tunisie. besnawel@yahoo.fr FAU - Mjid, M AU - Mjid M FAU - Zarrouk, M AU - Zarrouk M FAU - Rouhou, S C AU - Rouhou SC FAU - Ammous, I AU - Ammous I FAU - Hantous, S AU - Hantous S FAU - Racil, H AU - Racil H FAU - Chabbou, A AU - Chabbou A LA - fre PT - Case Reports PT - Journal Article PT - Review TT - Un syndrome d'Erasmus avec des masses pseudo-tumorales. DEP - 20110723 PL - France TA - Rev Mal Respir JT - Revue des maladies respiratoires JID - 8408032 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantigens) RN - 0 (Histones) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 5.99.1.2 (TOP1 protein, human) SB - IM MH - Airway Obstruction/etiology MH - Antibodies, Antinuclear/blood/immunology MH - Autoantigens/immunology MH - DNA Topoisomerases, Type I/immunology MH - Deglutition Disorders/etiology MH - Diagnosis, Differential MH - Dyspnea/etiology MH - Fibrosis MH - Histones/immunology MH - Humans MH - Lung/diagnostic imaging/*pathology MH - Lung Neoplasms/diagnosis MH - Male MH - Middle Aged MH - Radiography MH - Raynaud Disease/etiology MH - Risk Factors MH - Scleroderma, Systemic/*complications/diagnosis/epidemiology/pathology MH - Silicotuberculosis/*complications/diagnosis/epidemiology/pathology MH - Smoking/adverse effects MH - Syndrome EDAT- 2011/09/29 06:00 MHDA- 2012/01/28 06:00 CRDT- 2011/09/28 06:00 PHST- 2010/02/23 00:00 [received] PHST- 2011/02/09 00:00 [accepted] PHST- 2011/09/28 06:00 [entrez] PHST- 2011/09/29 06:00 [pubmed] PHST- 2012/01/28 06:00 [medline] AID - S0761-8425(11)00271-3 [pii] AID - 10.1016/j.rmr.2011.06.003 [doi] PST - ppublish SO - Rev Mal Respir. 2011 Sep;28(7):924-7. doi: 10.1016/j.rmr.2011.06.003. Epub 2011 Jul 23. PMID- 34080745 OWN - NLM STAT- MEDLINE DCOM- 20211130 LR - 20211130 IS - 1600-0846 (Electronic) IS - 0909-752X (Linking) VI - 27 IP - 6 DP - 2021 Nov TI - Application of a novel finger temperature device in the assessment of subjects with Raynaud's phenomenon. PG - 1110-1115 LID - 10.1111/srt.13070 [doi] AB - INTRODUCTION: Finger skin thermometry is one of the most commonly used methods for evaluating the response of the digital vessels to cold stimulation. The aim of this study was to evaluate the applicability of a novel finger skin temperature device for performing cold-stimulation test (CST) in subjects with primary and secondary Raynaud's phenomenon (RP). METHODOLOGY: A total of 155 consecutive subjects were studied. They were divided into three groups: 73 patients with primary RP (8 males, 65 females, mean age 38.5 ± 16.2), 42 patients with secondary RP (4 males, 38 females, mean age 49.6 ± 13.1, connected with lupus erythematosus and systemic scleroderma), and 40 healthy controls (5 males, 35 females, mean age 38.8 ± 16.6). Standardized CST consisting of exposure of both hands to water with a temperature of 10℃ for 5 minutes was performed. Changes in skin temperature of both wrists and 2-5 fingers were measured using a novel finger temperature device (Courage & Khazaka). Measurements were made before and 5, 10, 15, 20, 25, and 30 minutes after cold stimulation. The time of recovery for baseline temperature of all fingers below 15 minutes was considered normal. RESULTS: The CST was normal in 6 (8.2%) of the patients with primary RP, in 7 (16.6%) of the patients with secondary RP, and in 28 (70%) of the healthy control subjects. The time of complete recovery of baseline temperature with respect to the first finger and for all 2-5 fingers in the three groups was as follows: 24.8 and 28.5 minutes (primary RP), 21.7 and 26.8 minutes (secondary RP), and 11.1 and 15.2 minutes (healthy subjects). Furthermore, the microcirculation was seriously disturbed (rewarming time >31 minutes of all 2-5 digits of both hands) in 54.1% (n = 79), 34.5% (n = 29) and 5% (n = 4) in the same study groups. DISCUSSION: Our results suggested that skin microcirculation is more disturbed in patients with primary RP than in patients with secondary RP. In support of this unexpected finding were the results reported by Ruaro B. et al (2019). They investigated the blood perfusion (BP) by laser speckle contrast analysis (LASCA) at different skin areas of hands and found that it was significantly lower in primary RP than in secondary RP related to systemic sclerosis. CONCLUSION: The new finger temperature device used could be considered useful for performing cold-stimulation test in patients with Raynaud's phenomenon. CI - © 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Aleksiev, Teodor AU - Aleksiev T AD - Department of Dermatology and Venereology, Medical Faculty, Medical University of Plovdiv, Plovdiv, Bulgaria. FAU - Ivanova, Zlatina AU - Ivanova Z AD - Department of Dermatology and Venereology, Medical Faculty, Medical University of Plovdiv, Plovdiv, Bulgaria. FAU - Dobrev, Hristo AU - Dobrev H AUID- ORCID: 0000-0002-5513-3789 AD - Department of Dermatology and Venereology, Medical Faculty, Medical University of Plovdiv, Plovdiv, Bulgaria. FAU - Atanasov, Nikolay AU - Atanasov N AD - Department of Health Management and Health Economics, Faculty of Public Health, Medical University of Plovdiv, Plovdiv, Bulgaria. LA - eng PT - Journal Article DEP - 20210603 PL - England TA - Skin Res Technol JT - Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) JID - 9504453 SB - IM MH - Adult MH - Cold Temperature MH - Female MH - Fingers MH - Humans MH - Male MH - Middle Aged MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Systemic MH - Skin Temperature MH - Temperature MH - Young Adult OTO - NOTNLM OT - cold-water provocation test OT - skin temperature EDAT- 2021/06/04 06:00 MHDA- 2021/12/01 06:00 CRDT- 2021/06/03 08:52 PHST- 2021/05/06 00:00 [revised] PHST- 2021/04/18 00:00 [received] PHST- 2021/05/14 00:00 [accepted] PHST- 2021/06/04 06:00 [pubmed] PHST- 2021/12/01 06:00 [medline] PHST- 2021/06/03 08:52 [entrez] AID - 10.1111/srt.13070 [doi] PST - ppublish SO - Skin Res Technol. 2021 Nov;27(6):1110-1115. doi: 10.1111/srt.13070. Epub 2021 Jun 3. PMID- 22786498 OWN - NLM STAT- MEDLINE DCOM- 20120921 LR - 20250626 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 2012 IP - 7 DP - 2012 Jul 11 TI - Oral vasodilators for primary Raynaud's phenomenon. PG - CD006687 LID - 10.1002/14651858.CD006687.pub3 [doi] LID - CD006687 AB - BACKGROUND: Many different drugs have been suggested for the symptomatic treatment of primary Raynaud's phenomenon. Apart from calcium channel blockers, which are considered the drugs of choice, the evidence of the effects of alternative pharmacological treatments is limited. This is an update of a review first published in 2008. OBJECTIVES: To assess the effects of various drugs with vasodilator actions on primary Raynaud's phenomenon. SEARCH METHODS: For this update the Cochrane Peripheral Vascular Diseases Group Trials Search Co-ordinator searched the Specialised Register (last searched 14 May 2012), CENTRAL (Issue 4, 2012) and clinical trials databases. We contacted one pharmaceutical company and one trial author for additional information. In addition, the reference lists of relevant studies were searched for additional citations. There were no language restrictions. SELECTION CRITERIA: Randomised controlled trials evaluating the effects of oral formulations of any drug with vasodilator effects on subjective symptoms in primary Raynaud's phenomenon. Treatment with, or comparison with, calcium channel blockers was not assessed in this review. DATA COLLECTION AND ANALYSIS: Two members of the review team independently assessed the trials for inclusion and their quality and extracted the data. Data extraction included adverse events. We contacted trial authors for missing data. MAIN RESULTS: Eight studies involving 290 participants were included. Two trials examined the effects of captopril, the rest were single trials on single drugs. All comparisons were with placebo. The methodological quality of most trials was poor.Enalapril was associated with a small increase in the frequency of attacks per week (difference in means 0.8; 95% CI 0.43 to 1.17). The difference between the intervention groups on a subjective improvement score was non-significant. There was a significant effect of buflomedil on the frequency of attacks per week (weighted mean difference (WMD) -8.8; 95% CI -17.55 to -0.09), but there was no evidence of effect on the severity score. The proportion with fewer attacks was significantly higher on moxisylyte than on placebo (relative risk (RR) 4.33; 95% CI 1.36 to 13.81). For captopril, beraprost, dazoxiben and ketanserin there was no evidence of an effect on the frequency, severity or duration of attacks. Beraprost and moxisylyte gave significantly more adverse effects than placebo. AUTHORS' CONCLUSIONS: Poor methodological quality, small sample sizes and the limited data available resulted in low precision of the statistical results and limited value of the overall results .The overall results show that there is no evidence for an effect of vasodilator drugs on primary Raynaud's phenomenon. FAU - Stewart, Marlene AU - Stewart M AD - Centre for Population Health Sciences, University of Edinburgh, Edinburgh, UK. Marlene.Stewart@ed.ac.uk. FAU - Morling, Joanne R AU - Morling JR LA - eng GR - CZB/4/788/CSO_/Chief Scientist Office/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20120711 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Vasodilator Agents) SB - IM UOF - Cochrane Database Syst Rev. 2008 Apr 16;(2):CD006687. doi: 10.1002/14651858.CD006687.pub2. PMID: 18425964 UIN - Cochrane Database Syst Rev. 2021 May 17;5:CD006687. doi: 10.1002/14651858.CD006687.pub4. PMID: 33998674 MH - Administration, Oral MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Vasodilator Agents/*administration & dosage PMC - PMC6718219 COIS- None known EDAT- 2012/07/13 06:00 MHDA- 2012/09/22 06:00 PMCR- 2013/07/11 CRDT- 2012/07/13 06:00 PHST- 2012/07/13 06:00 [entrez] PHST- 2012/07/13 06:00 [pubmed] PHST- 2012/09/22 06:00 [medline] PHST- 2013/07/11 00:00 [pmc-release] AID - CD006687.pub3 [pii] AID - 10.1002/14651858.CD006687.pub3 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2012 Jul 11;2012(7):CD006687. doi: 10.1002/14651858.CD006687.pub3. PMID- 26876191 OWN - NLM STAT- MEDLINE DCOM- 20170707 LR - 20170707 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 67 IP - 3 DP - 2015 Dec 30 TI - Erasmus syndrome in a marble worker. PG - 116-22 LID - 10.4081/reumatismo.2015.826 [doi] AB - Erasmus syndrome is defined as the association of silica exposure and subsequent development of systemic sclerosis. The limited number of cases reported in the literature mainly involves miners and only sporadically other professionals. We describe a case of Erasmus syndrome in a marble worker. A 68 year old man came to our observation complaining pelvic and scapular girdle pain, evening fever, intense weakness and emaciation for about 1 month. He also reported to have had Raynaud's phenomenon in his hands for the last 13 years. Also, his occupational history revealed a chronic exposure to silica dust. The patient presented pain in his shoulders and hips, moderate skin thickening and sclerosis in his hands and fingers extending proximally to his wrists. The diagnosis of systemic sclerosis was determined according to his clinical and medical history, the positivity of anti-Scl 70 antibodies, the nailfold capillaroscopy suggestive of an active scleroderma pattern and the detection of a mild restrictive pulmonary syndrome. The evaluation of the organbased complications excluded a gastroenterological and cardiovascular involvement, while the chest computed tomography (CT) detected multiple small nodules with a mantle distribution and enlarged lymph nodes with no signs of interstitial lung disease and fibrosis. Additional tests (positron emission tomography-CT, flexible bronchoscopy and broncho-alveolar lavage) excluded infectious diseases and cancer. However, given the pulmonary involvement, we performed a histological examination of the parenchyma and lymph nodes, which revealed a picture of pneumoconiosis. In the end, the occupational history and the findings from the diagnostic procedures led to the diagnosis of pulmonary silicosis. The precise definition of the pulmonary involvement was essential to the therapeutic approach to this patient. FAU - Bello, S AU - Bello S AD - Rheumatology Unit, Policlinico Hospital, University of Bari, Bari. s.bello1@virgilio.it. FAU - Rinaldi, A AU - Rinaldi A FAU - Trabucco, S AU - Trabucco S FAU - Serafino, L AU - Serafino L FAU - Bonali, C AU - Bonali C FAU - Lapadula, G AU - Lapadula G LA - eng PT - Case Reports PT - Journal Article DEP - 20151230 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Antibodies, Anti-Idiotypic) RN - 0 (Biomarkers) RN - H0G9379FGK (Calcium Carbonate) SB - IM MH - Aged MH - Antibodies, Anti-Idiotypic/blood MH - Biomarkers/blood MH - Calcium Carbonate/*adverse effects MH - Fever/etiology MH - Humans MH - Male MH - Mining MH - Occupational Exposure/*adverse effects MH - Pelvic Pain/etiology MH - Pneumoconiosis/diagnosis/etiology MH - Raynaud Disease/complications MH - Scleroderma, Systemic/blood/complications/*diagnosis MH - Silicosis/complications/*diagnosis/*etiology MH - Syndrome EDAT- 2016/02/16 06:00 MHDA- 2017/07/08 06:00 CRDT- 2016/02/16 06:00 PHST- 2015/05/02 00:00 [received] PHST- 2015/10/13 00:00 [accepted] PHST- 2015/10/13 00:00 [revised] PHST- 2016/02/16 06:00 [entrez] PHST- 2016/02/16 06:00 [pubmed] PHST- 2017/07/08 06:00 [medline] AID - 10.4081/reumatismo.2015.826 [doi] PST - epublish SO - Reumatismo. 2015 Dec 30;67(3):116-22. doi: 10.4081/reumatismo.2015.826. PMID- 17013439 OWN - NLM STAT- MEDLINE DCOM- 20061212 LR - 20191110 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 58 IP - 3 DP - 2006 Jul-Sep TI - [Determinants of depression in 111 Italian patients with systemic sclerosis]. PG - 219-25 AB - BACKGROUND: A high prevalence of depressive symptoms has been described in systemic sclerosis (SSc), but no clear association with organ involvement or objective indices of disease severity has been depicted. To date, no effort has been made to determine the prevalence of depressive symptoms in Italian patients with SSc or to clarify their cause. METHODS: One-hundred-eleven SSc patients were asked to fill in the Beck Depression Inventory (BDI) questionnaire, the scleroderma Health Assessment Questionnaire (sHAQ) and two additional questions assessing the patient's familiar support and the social consequences of the patient's change in physical appearnace. RESULTS: Thirty-seven subjects (33.4%) presented mild to severe depressive symptoms (BDI >/=17). On univariate analysis the diffuse cutaneous form of the disease (p=0.019), higher pulmonary systolic pressures on echocardiogram (p=0.016), lower FVC percentage of predicted values (p=0.022), higher sHAQ values (p<0.001) or higher VAS values for pain (p=0.007), lung involvement (p=0.02), Raynaud's phenomenon severity (p=0.002), ulcers severity (p=0.006) or disease severity (p<0.001), were associated with the presence of pathologic depressive symptoms. On multivariate analysis only the VAS for disease severity relevant to BDI scores (p=0.016). Social behaviour changes due to SSc-related physical involvement were reported in 14 patients (38%) with depressive symptoms (p=0,006) and were more likely to be observed in younger patients (p=0.001) with a more severe Raynauds's phenomenon (p=0.013). CONCLUSIONS: Mild to severe depressive symptoms are common in SSc patients especially in those with a worse perception of disease severity, these patients should be carefully monitored and a psychological assistance counselled whenever necessary. FAU - Beretta, L AU - Beretta L AD - Centro di Riferimento per le Malattie Autoimmuni Sistemiche, Fondazione IRCCS Policlinico-Mangiagalli-Regina Elena, Milano, Italia. lorberimm@hotmail.com FAU - Astori, S AU - Astori S FAU - Ferrario, E AU - Ferrario E FAU - Caronni, M AU - Caronni M FAU - Raimondi, M AU - Raimondi M FAU - Scorza, R AU - Scorza R LA - ita PT - Comparative Study PT - English Abstract PT - Journal Article TT - Determinanti della depressione in 111 pazienti italiani con sclerosi sistemica. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Chi-Square Distribution MH - Depression/diagnosis/epidemiology/*etiology MH - Female MH - Humans MH - Italy/epidemiology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Personality Inventory MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/diagnosis/epidemiology/physiopathology/*psychology MH - Severity of Illness Index MH - Surveys and Questionnaires MH - Vital Capacity EDAT- 2006/10/03 09:00 MHDA- 2006/12/13 09:00 CRDT- 2006/10/03 09:00 PHST- 2006/10/03 09:00 [pubmed] PHST- 2006/12/13 09:00 [medline] PHST- 2006/10/03 09:00 [entrez] AID - 10.4081/reumatismo.2006.219 [doi] PST - ppublish SO - Reumatismo. 2006 Jul-Sep;58(3):219-25. doi: 10.4081/reumatismo.2006.219. PMID- 26812367 OWN - NLM STAT- MEDLINE DCOM- 20170123 LR - 20170123 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 45 IP - 4 DP - 2016 Jul TI - Calcinosis preferentially affects the thumb compared to other fingers in patients with systemic sclerosis. PG - 317-20 LID - 10.3109/03009742.2015.1127412 [doi] AB - OBJECTIVES: Although Raynaud's phenomenon often spares the thumb, clinical experience suggests conversely that, in patients with systemic sclerosis (SSc), the thumb is frequently affected by calcinosis (as is demonstrated on plain radiographs). Our aim was to investigate the hypothesis that, in patients with SSc, thumbs are more commonly affected by calcinosis than other digits. METHOD: Sixty-eight hand radiographs with at least one area of calcinosis were identified. Each digit on both hands of each patient was assigned a severity score on a scale from 0 to 3 (0 = no calcinosis, 3 = most severe). The scoring was completed twice, including and excluding the metacarpals. RESULTS: Right hands were found to be associated with slightly higher scores than left hands [estimate 0.14, 95% confidence interval (CI) 0.03-0.26]. Scores were lower for other fingers compared to thumbs. There was strong evidence (p < 0.0001) of a trend of decreasing severity moving from the thumb to the little finger. There was no evidence that the pattern of severity across the fingers was different on left and right hands (p = 0.77). The results were similar whether or not metacarpals were included. CONCLUSIONS: The thumb is affected by calcinosis more than other digits, followed by the index finger. This observation provides insight into the pathogenesis of SSc-related calcinosis, which may relate more to repetitive trauma than to ischaemia. FAU - Gauhar, R AU - Gauhar R AD - a Centre for Musculoskeletal Research , Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre , Manchester , UK. FAU - Wilkinson, J AU - Wilkinson J AD - b Centre for Biostatistics, Institute of Population Health , University of Manchester, Manchester Academic Health Science Centre , Manchester , UK. FAU - Harris, J AU - Harris J AD - c Department of Radiology , Salford Royal NHS Foundation Trust , Salford , UK. FAU - Manning, J AU - Manning J AD - d Rheumatology Directorate , Salford Royal NHS Foundation Trust , Salford , UK. FAU - Herrick, A L AU - Herrick AL AD - a Centre for Musculoskeletal Research , Salford Royal NHS Foundation Trust, University of Manchester, Manchester Academic Health Science Centre , Manchester , UK. AD - e NIHR Manchester Musculoskeletal Biomedical Research Unit , Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK. LA - eng PT - Journal Article DEP - 20160126 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Aged MH - Calcinosis/*diagnostic imaging/etiology/physiopathology MH - Female MH - Finger Injuries/physiopathology MH - Fingers/blood supply/diagnostic imaging MH - Humans MH - Ischemia/physiopathology MH - Logistic Models MH - Male MH - Middle Aged MH - Radiography MH - Raynaud Disease/physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*diagnostic imaging/physiopathology MH - Thumb/blood supply/*diagnostic imaging/injuries EDAT- 2016/01/27 06:00 MHDA- 2017/01/24 06:00 CRDT- 2016/01/27 06:00 PHST- 2016/01/27 06:00 [entrez] PHST- 2016/01/27 06:00 [pubmed] PHST- 2017/01/24 06:00 [medline] AID - 10.3109/03009742.2015.1127412 [doi] PST - ppublish SO - Scand J Rheumatol. 2016 Jul;45(4):317-20. doi: 10.3109/03009742.2015.1127412. Epub 2016 Jan 26. PMID- 25326572 OWN - NLM STAT- MEDLINE DCOM- 20150713 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2014 DP - 2014 Oct 17 TI - Limited systemic sclerosis initially presenting with mesenteric panniculitis. LID - 10.1136/bcr-2014-206961 [doi] LID - bcr2014206961 AB - Mesenteric panniculitis pertains to a group of uncommon disorders named sclerosing mesenteritis that present with different levels of inflammation and fibrosis of the small bowel mesentery. It is associated with abdominal surgeries, trauma, malignancies, infections and connective tissue diseases. To the best of our knowledge, no cases of sclerosing mesenteritis have been reported in patients with systemic sclerosis. We present a case of a 61-year-old woman who had incidental CT findings of mesenteric panniculitis. Diagnosis was confirmed by biopsy that showed fat necrosis. On further review she had a 1-year history of Raynaud's phenomenon. Physical examination showed sclerodactyly. She had elevated anticentromere antibodies and skin biopsy was consistent with scleroderma. She was diagnosed with limited systemic sclerosis and was treated with D-penicillamine. After 6 years of follow-up, the mesenteric panniculitis and systemic sclerosis both remained stable. This case highlights the importance of considering rheumatic diseases in the differential diagnosis of sclerosing mesenteritis. CI - 2014 BMJ Publishing Group Ltd. FAU - Arroyo-Ávila, Mariangelí AU - Arroyo-Ávila M AD - Division of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico. FAU - Vilá, Luis M AU - Vilá LM AD - Division of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141017 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antirheumatic Agents) RN - GNN1DV99GX (Penicillamine) SB - IM MH - Antirheumatic Agents/therapeutic use MH - Biopsy MH - Diagnosis, Differential MH - Fat Necrosis/complications MH - Female MH - Follow-Up Studies MH - Humans MH - Mesentery/*diagnostic imaging/pathology MH - Middle Aged MH - Panniculitis, Peritoneal/complications/*diagnostic imaging/drug therapy MH - Penicillamine/therapeutic use MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications/*diagnostic imaging/drug therapy MH - Tomography, X-Ray Computed/methods PMC - PMC4202032 EDAT- 2014/10/19 06:00 MHDA- 2015/07/15 06:00 PMCR- 2016/10/17 CRDT- 2014/10/19 06:00 PHST- 2014/10/19 06:00 [entrez] PHST- 2014/10/19 06:00 [pubmed] PHST- 2015/07/15 06:00 [medline] PHST- 2016/10/17 00:00 [pmc-release] AID - bcr-2014-206961 [pii] AID - 10.1136/bcr-2014-206961 [doi] PST - epublish SO - BMJ Case Rep. 2014 Oct 17;2014:bcr2014206961. doi: 10.1136/bcr-2014-206961. PMID- 36291537 OWN - NLM STAT- MEDLINE DCOM- 20221028 LR - 20221222 IS - 2218-273X (Electronic) IS - 2218-273X (Linking) VI - 12 IP - 10 DP - 2022 Sep 20 TI - Anti-Phosphatidylethanolamine and Anti-Phosphatidylserine Antibodies-Association with Renal Involvement, Atherosclerosis, Cardiovascular Manifestations, Raynaud Phenomenon and Disease Activity in Polish Patients with Systemic Lupus Erythematosus. LID - 10.3390/biom12101328 [doi] LID - 1328 AB - OBJECTIVE: To evaluate the association between anti-phosphatidylethanolamine (aPE) and anti-phosphatidylserine (aPS) antibodies and cardiovascular risk, organ involvement and disease activity in systemic lupus erythematosus (SLE) patients. METHODS: We studied 93 SLE patients and 30 controls. We analyzed levels of anti-phospholipid antibodies, including aPS and aPE, the profiles of antinuclear, anti-neutrophil cytoplasmic (ANCA) and anti-endothelial antibodies, carotid intima-media thickness (cITM) and atherosclerotic plaque presence, ankle-brachial and high resistance indices, atherosclerotic risk factors, organ manifestations and treatment. RESULTS: Levels of aPS and aPE were significantly higher in SLE patients in comparison with the controls (p = 0.038 and p = 0.044, respectively). aPS was associated with the risk of Raynaud's phenomenon (p = 0.021) development. aPE increased the risk of renal involvement (p = 0.049), cerebral stroke (p = 0.050), high vlues of cIMT (p = 0.041) development as well as occurrence of selected serological markers associated with activity of the disease such as anti-double stranded DNA (p = 0.021). The long duration of regular smoking (p = 0.021) and the high number of cigarettes/day (p = 0.015) were significantly associated with the risk of aPE occurrence. CONCLUSIONS: Patients with aPS and aPE are at risk of vascular involvement. Especially the presence of aPE may significantly increase the risk of thrombotic complications development in SLE patients without classical serological markers of APS. Finally, aPE might be used as a marker of disease activity and risk of renal injury development in this patient group. The classical atherosclerotic markers including lipid indices play an important role in complex analysis of cardiovascular risk in lupus patients and enable to identify patients at the highest risk and implement effective preventive, diagnostic and therapeutic procedures. FAU - Fischer, Katarzyna AU - Fischer K AD - Individual Laboratory for Rheumatologic Diagnostics, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. FAU - Przepiera-Będzak, Hanna AU - Przepiera-Będzak H AUID- ORCID: 0000-0001-6727-2594 AD - Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. FAU - Brzosko, Iwona AU - Brzosko I AD - Individual Laboratory for Rheumatologic Diagnostics, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. FAU - Sawicki, Marcin AU - Sawicki M AD - Department of Imaging Diagnostics and Interventional Radiology, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. FAU - Walecka, Anna AU - Walecka A AD - Department of Imaging Diagnostics and Interventional Radiology, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. FAU - Brzosko, Marek AU - Brzosko M AD - Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, Poland. LA - eng PT - Journal Article DEP - 20220920 PL - Switzerland TA - Biomolecules JT - Biomolecules JID - 101596414 RN - 0 (Phosphatidylserines) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Biomarkers) RN - 9007-49-2 (DNA) SB - IM MH - Humans MH - Animals MH - *Antiphospholipid Syndrome/complications/diagnosis MH - Phosphatidylserines MH - Carotid Intima-Media Thickness MH - Smoking MH - Antibodies, Antineutrophil Cytoplasmic MH - Poland MH - *Lupus Erythematosus, Systemic/complications MH - *Atherosclerosis/complications MH - Biomarkers MH - *Raynaud Disease/complications MH - *Hominidae MH - DNA PMC - PMC9599205 OTO - NOTNLM OT - anti-phosphatidylethanolamine antibodies OT - anti-phosphatidylserine antibodies OT - antiphospholipid syndrome OT - cardiovascular risk OT - renal involvement OT - smoking status OT - systemic lupus erythematosus COIS- The authors declare no conflict of interest. EDAT- 2022/10/28 06:00 MHDA- 2022/10/29 06:00 PMCR- 2022/09/20 CRDT- 2022/10/27 01:12 PHST- 2022/07/19 00:00 [received] PHST- 2022/09/12 00:00 [revised] PHST- 2022/09/14 00:00 [accepted] PHST- 2022/10/27 01:12 [entrez] PHST- 2022/10/28 06:00 [pubmed] PHST- 2022/10/29 06:00 [medline] PHST- 2022/09/20 00:00 [pmc-release] AID - biom12101328 [pii] AID - biomolecules-12-01328 [pii] AID - 10.3390/biom12101328 [doi] PST - epublish SO - Biomolecules. 2022 Sep 20;12(10):1328. doi: 10.3390/biom12101328. PMID- 33661406 OWN - NLM STAT- MEDLINE DCOM- 20210818 LR - 20210818 IS - 2589-0409 (Electronic) IS - 1110-0362 (Linking) VI - 33 IP - 1 DP - 2021 Mar 4 TI - Acral vascular syndrome Lennert type T cell lymphoma-a case report. PG - 8 LID - 10.1186/s43046-021-00063-7 [doi] AB - BACKGROUND: Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and erythromelalgia but can be rarely seen in the malignant condition. Patients may present pain, permanent digital blanching or cyanosis, and desquamation or ulceration of the fingers. Acral vascular syndrome is rarely associated with lymphoid neoplasm and is associated with smoking, autoimmune connective tissue diseases, and vasculitis. Here, we describe a 79-year-old female who was diagnosed with vitiligo and peripheral T cell lymphoma Lennert type stage 4 with anemia of chronic disease with digital acral vascular syndrome. CASE PRESENTATION: A 79-year-old female with vitiligo presented with gangrene of the distal extremities associated with pain and intermittent fever for 2 months. On evaluation, she was found to have anemia of chronic disease and generalized lymphadenopathy and diagnosed as peripheral T cell lymphoma Lennert type with bone marrow involvement and digital acral vascular syndrome. CONCLUSION: Acral vascular syndrome can be a presentation of lymphoma; if intervened earlier, the patient can be saved from the amputation of fingers or affected limb. Though it is a rare presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevents functional dependence on caregivers. In our patient, gangrene of other fingers was prevented even though it is an aggressive variant of T cell lymphoma. FAU - Klanidhi, Kamal Bandhu AU - Klanidhi KB AD - Department of Geriatric Medicine, All India Institute of Medical Sciences (AIIMS), Room no. 3095A, New Delhi, 110029, India. FAU - Monian, S Ashwin AU - Monian SA AD - Department of Geriatric Medicine, All India Institute of Medical Sciences (AIIMS), Room no. 3095A, New Delhi, 110029, India. FAU - Chakrawarty, Avinash AU - Chakrawarty A AD - Department of Geriatric Medicine, All India Institute of Medical Sciences (AIIMS), Room no. 3095A, New Delhi, 110029, India. chakrawartyavinash@gmail.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20210304 PL - England TA - J Egypt Natl Canc Inst JT - Journal of the Egyptian National Cancer Institute JID - 9424566 SB - IM MH - Aged MH - Female MH - Fingers MH - Gangrene/diagnosis/etiology MH - Humans MH - *Lymphoma, Non-Hodgkin MH - *Lymphoma, T-Cell MH - *Raynaud Disease OTO - NOTNLM OT - Acral vascular syndrome OT - Peripheral T cell lymphoma OT - Vitiligo EDAT- 2021/03/05 06:00 MHDA- 2021/08/19 06:00 CRDT- 2021/03/04 12:14 PHST- 2020/10/07 00:00 [received] PHST- 2021/02/18 00:00 [accepted] PHST- 2021/03/04 12:14 [entrez] PHST- 2021/03/05 06:00 [pubmed] PHST- 2021/08/19 06:00 [medline] AID - 10.1186/s43046-021-00063-7 [pii] AID - 10.1186/s43046-021-00063-7 [doi] PST - epublish SO - J Egypt Natl Canc Inst. 2021 Mar 4;33(1):8. doi: 10.1186/s43046-021-00063-7. PMID- 36580065 OWN - NLM STAT- MEDLINE DCOM- 20230102 LR - 20230103 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 74 IP - 3 DP - 2022 Dec 29 TI - Nailfold capillaroscopy in the rheumatological current clinical practice in Italy: results of a national survey. LID - 10.4081/reumatismo.2022.1508 [doi] AB - This cross-sectional online study was designed by the study group on Capillaroscopy and Microcirculation in Rheumatic Diseases (CAP) of the Italian Society of Rheumatology (SIR) to provide an overview of the management of nailfold capillaroscopy in Italian rheumatology centers. Therefore, SIR distributed the survey to its members in July 2021, and each center's physician with the most expertise in capillaroscopy completed the questionnaire. The survey was completed by 102 centers, with at least one representative from each Italian region. Ninety-three centers perform capillaroscopy, and 52 (56) conduct more than 200 investigations annually. Seventy-eight (84%) of respondents have more than five years of experience with the technique, and 75 centers (80.6%) have received certification from specific national or international training courses. In 85 centers, a videocapillaroscope with 200x magnification is employed (91.4%). The average waiting period for the examination is 2.4 months, and less than 3 months in 64 of the locations (68.8%). The study demonstrates that capillaroscopy is an integral part of both the diagnostic phase of Raynaud's phenomenon and the monitoring of autoimmune connective tissue diseases (CTDs). However, the reporting methods and timing of patient followup are heterogeneous. FAU - Ingegnoli, F AU - Ingegnoli F AD - Clinical Rheumatology Unit, ASST Gaetano Pini-CTO, Department of Clinical and Community Sciences, University of Milano, Milano. francesca.ingegnoli@unimi.it. FAU - Cornalba, M AU - Cornalba M AD - Clinical Rheumatology Unit, ASST Gaetano Pini-CTO, Department of Clinical and Community Sciences, University of Milano, Milano. martinacornalba2@gmail.com. FAU - De Angelis, R AU - De Angelis R AD - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Jesi (AN). r.deangelis@staff.univpm.it. FAU - Guiducci, S AU - Guiducci S AD - Department of Clinical and Molecular Sciences, Division of Rheumatology, University of Firenze. serena.guiducci@unifi.it. FAU - Giuggioli, D AU - Giuggioli D AD - Rheumatology Unit, Hospital of Modena, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena. dilia.giuggioli@unimore.it. FAU - Pizzorni, C AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCSS Polyclinic San Martino, Genoa. 164950@unige.it. FAU - Riccieri, V AU - Riccieri V AD - Department of Clinical, Internal, Anesthesiologic and Cardiovascular Sciences, La Sapienza University, Roma. valeria.riccieri@uniroma1.it. FAU - Sebastiani, M AU - Sebastiani M AD - Rheumatology Unit, Hospital of Modena, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena. marco.sebastiani@unimore.it. FAU - Sulli, A AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCSS Polyclinic San Martino, Genoa. albertosulli@unige.it. FAU - Cutolo, M AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCSS Polyclinic San Martino, Genoa. mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20221229 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Humans MH - *Rheumatology MH - Microscopic Angioscopy/methods MH - Cross-Sectional Studies MH - *Rheumatic Diseases/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging MH - Italy MH - Nails/diagnostic imaging EDAT- 2022/12/30 06:00 MHDA- 2023/01/03 06:00 CRDT- 2022/12/29 10:39 PHST- 2022/07/03 00:00 [received] PHST- 2022/09/27 00:00 [accepted] PHST- 2022/12/29 10:39 [entrez] PHST- 2022/12/30 06:00 [pubmed] PHST- 2023/01/03 06:00 [medline] AID - 10.4081/reumatismo.2022.1508 [doi] PST - epublish SO - Reumatismo. 2022 Dec 29;74(3). doi: 10.4081/reumatismo.2022.1508. PMID- 20015282 OWN - NLM STAT- MEDLINE DCOM- 20101028 LR - 20220409 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 35 IP - 7 DP - 2010 Oct TI - Cutaneous lupus erythematosus: clinical and immunological study of 308 patients stratified by gender. PG - 729-35 LID - 10.1111/j.1365-2230.2009.03764.x [doi] AB - BACKGROUND: Numerous studies involving systemic lupus erythematosus (SLE) have attempted to identify gender differences in patients with lupus erythematosus (LE). However, few reports on cutaneous lupus erythematous (CLE) have identified gender differences. AIM: To analyse and compare the prevalence and characteristics of the main clinical and immunological features of male and female patients with CLE. METHODS: The medical records of 103 (33.4%) male and 205 (66.6%) female patients with CLE who were treated as inpatients or outpatients between January 1985 and December 2000 were retrospectively studied. All patients were reviewed in detail stratified by a predefined protocol. RESULTS: Female patients had a higher prevalence of Raynaud's phenomenon (P < 0.01), chilblain lupus (P = 0.005), arthralgias (P = 0.001) and SLE (P < 0.01). Female patients were also more likely to have an increased erythrocyte sedimentation rate (P < 0.005), higher levels of antinuclear antibodies (P < 0.001) and decreased levels of C3 (P < 0.001), C4 (P < 0.01) and CH50 (P < 0.01). There was a higher prevalence of clinical and laboratory abnormalities in female patients who had both SLE and CLE than in male patients with both conditions. Conclusions.  In our series, differences in the expression of CLE existed between male and female patients with respect to the type of lesions, systemic features, and immunological findings. CI - © 2009 The Author(s). Journal compilation © 2009 British Association of Dermatologists. FAU - Vera-Recabarren, M A AU - Vera-Recabarren MA AD - Department of Dermatology, Hospital Clinic, University of Barcelona, Catalonia, Spain. mavera@aedv.es FAU - García-Carrasco, M AU - García-Carrasco M FAU - Ramos-Casals, M AU - Ramos-Casals M FAU - Herrero, C AU - Herrero C LA - eng PT - Journal Article PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 RN - 0 (Antibodies, Antinuclear) RN - 9007-36-7 (Complement System Proteins) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/blood MH - Arthralgia/etiology MH - Blood Sedimentation MH - Chilblains/etiology MH - Child MH - Child, Preschool MH - Complement System Proteins/analysis MH - Female MH - Humans MH - Infant MH - Lupus Erythematosus, Cutaneous/*complications/immunology MH - Lupus Erythematosus, Systemic/complications MH - Male MH - Middle Aged MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Sex Factors MH - Young Adult EDAT- 2009/12/18 06:00 MHDA- 2010/10/29 06:00 CRDT- 2009/12/18 06:00 PHST- 2009/12/18 06:00 [entrez] PHST- 2009/12/18 06:00 [pubmed] PHST- 2010/10/29 06:00 [medline] AID - CED3764 [pii] AID - 10.1111/j.1365-2230.2009.03764.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2010 Oct;35(7):729-35. doi: 10.1111/j.1365-2230.2009.03764.x. PMID- 34682587 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20211101 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 18 IP - 20 DP - 2021 Oct 15 TI - Systemic Sclerosis: Elevated Levels of Leukotrienes in Saliva and Plasma Are Associated with Vascular Manifestations and Nailfold Capillaroscopic Abnormalities. LID - 10.3390/ijerph182010841 [doi] LID - 10841 AB - The role of leukotrienes (LTs) in the pathogenesis of systemic sclerosis (SSc) needs clarification. We analyzed the association of salivary (sa) and plasma (p) levels (pg/mL) of cysteinyl-leukotrienes (CysLT) and LTB4 with SSc vascular manifestations and nailfold capillaroscopy (NFC) in a cross-sectional study. Patients and healthy controls were evaluated for vascular manifestations and NFC. LTs were compared between groups as follows: SSc with or SSc without vascular features and controls, and by NFC parameters. Twenty SSc patients and 16 volunteers were recruited; Raynaud's phenomenon (RP) history (SSc: saCysLT 99.4 ± 21.8 vs. controls: 23.05 ± 23.7, p = 0.01), RP at examination (SSc: saCysLT 129.3 ± 24.6 vs. controls: 23.05 ± 22.46, p = 0.01; pCysLT SSc: 87.5 ± 11.2 vs. controls: 32.37 ± 10.75, p = 0.002), capillary loss (saCysLT 138.6 ± 26.7 vs. 23.05 ± 21.6, p = 0.0007; saLTB4 3380.9 ± 426.6 vs. 1216.33 ± 346.1, p = 0.0005), "late" scleroderma pattern vs. controls (saCysLT 205.6 ± 32 vs. 23 ± 19.6, p = 0.0002; saLTB4 4564.9 ± 503.6 vs. 1216.3 ± 308.3; p < 0.0001) were all significant. Late patterns had higher levels (saCysLT, p = 0.002; LTB4 p = 0.0006) compared to active and early patterns (LTB4, p = 0.0006), and giant capillaries (p = 0.01) showed higher levels of LTs. Levels of pCysLT were higher in patients with RP at examination vs. patients without RP; saCysLT and LTB4 were higher in SSc group with vs. without capillary loss. LTs could be involved in the pathophysiology of vascular abnormalities. Further research is required to determine if blocking LTs could be a therapeutic target for SSc vascular manifestations. FAU - Mandujano, Angélica AU - Mandujano A AUID- ORCID: 0000-0001-8006-7683 AD - Departamento de Atención a la Salud, Universidad Autónoma Metropolitana-Xochimilco, Mexico City 04960, Mexico. FAU - Méndez-Ramírez, Ignacio AU - Méndez-Ramírez I AD - Instituto de Investigaciones en Matemáticas Aplicadas y en Sistemas, Universidad Nacional Autónoma de México, Mexico City 04510, Mexico. FAU - Silveira-Torre, Luis Humberto AU - Silveira-Torre LH AD - Departamento de Reumatología, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico. LA - eng PT - Journal Article DEP - 20211015 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 RN - 0 (Leukotrienes) SB - IM MH - Cross-Sectional Studies MH - Humans MH - Leukotrienes MH - Microscopic Angioscopy MH - Nails MH - *Raynaud Disease MH - Saliva MH - *Scleroderma, Systemic PMC - PMC8536043 OTO - NOTNLM OT - Raynaud’s phenomenon OT - cysteinyl-leukotriene OT - leukotrienes OT - nailfold capillaroscopy OT - saliva OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2021/10/24 06:00 MHDA- 2021/11/03 06:00 PMCR- 2021/10/15 CRDT- 2021/10/23 01:12 PHST- 2021/08/25 00:00 [received] PHST- 2021/10/07 00:00 [revised] PHST- 2021/10/13 00:00 [accepted] PHST- 2021/10/23 01:12 [entrez] PHST- 2021/10/24 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] PHST- 2021/10/15 00:00 [pmc-release] AID - ijerph182010841 [pii] AID - ijerph-18-10841 [pii] AID - 10.3390/ijerph182010841 [doi] PST - epublish SO - Int J Environ Res Public Health. 2021 Oct 15;18(20):10841. doi: 10.3390/ijerph182010841. PMID- 22247347 OWN - NLM STAT- MEDLINE DCOM- 20120726 LR - 20260518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 39 IP - 3 DP - 2012 Mar TI - Agreement with guidelines from a large database for management of systemic sclerosis: results from the Canadian Scleroderma Research Group. PG - 524-31 LID - 10.3899/jrheum.110121 [doi] AB - OBJECTIVE: We determined congruence with published guidelines from the European League Against Rheumatism (EULAR)/EULAR Scleroderma Trials and Research group, for systemic sclerosis (SSc) investigations and treatment practices within the Canadian Scleroderma Research Group (CSRG). METHODS: Investigations and medication use for SSc complications were obtained from records of patients with SSc in the CSRG to determine adherence to guidelines for patients enrolled before and after the guidelines were published. RESULTS: The CSRG database of 1253 patients had 992 patients with SSc enrolled before publication of the guidelines and 261 after. For pulmonary arterial hypertension (PAH) treatment, there were no differences in use before and after the guidelines, yet annual echocardiograms for PAH screening were done in 95% of patients enrolled before the guidelines and in only 86% of those enrolled after (p <0.0001), and fewer followup echocardiograms were done 1 year later in the latter group (88% vs 59%). No differences were found for the frequency of PAH-specific treatment; 60% had ever used calcium channel blockers for Raynaud's phenomenon, with no differences in the groups before and after the guidelines. But the use of phosphodiesterase type 5 inhibitors (which does not have guidelines) was increased in the after-guidelines group. Proton pump inhibitors were used in > 80% with gastroesophageal reflux disease before and after the guidelines. One-quarter with gastrointestinal symptoms were taking prokinetic drugs. For those with past SSc renal crisis, use of angiotensin-converting enzyme inhibitors was not different before and after the guidelines. For early diffuse SSc < 2 years, ever-use of methotrexate was similar (one-quarter of each group); and for symptomatic interstitial lung disease, 19% had ever used cyclophosphamide before the guidelines and 9% after (p = nonsignificant). CSRG practices were generally comparable to recently published guidelines; however, use of iloprost and bosentan was low for digital ulcers because these drugs are not approved for use in Canada. CONCLUSION: There did not seem to be an increase in adherence to recommendations once the guidelines were published. For many guidelines, 25% to 40% of patients who would qualify received the recommended treatment. FAU - Pope, Janet AU - Pope J AD - Department of Medicine, University of Western Ontario, London, Ontario, Canada. janet.pope@sjhc.london.on.ca FAU - Harding, Sarah AU - Harding S FAU - Khimdas, Sarit AU - Khimdas S FAU - Bonner, Ashley AU - Bonner A CN - Canadian Scleroderma Research Group FAU - Baron, Murray AU - Baron M LA - eng PT - Comparative Study PT - Journal Article DEP - 20120115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Canada MH - *Databases, Factual MH - *Disease Management MH - Europe MH - Female MH - Gastrointestinal Diseases/therapy MH - Guideline Adherence MH - Humans MH - Hypertension, Pulmonary/therapy MH - Male MH - Middle Aged MH - *Practice Guidelines as Topic MH - Raynaud Disease/therapy MH - Retrospective Studies MH - Scleroderma, Systemic/*therapy FIR - Hudson, M IR - Hudson M FIR - Markland, J IR - Markland J FIR - Docherty, P IR - Docherty P FIR - Fritzler, M IR - Fritzler M FIR - Jones, N IR - Jones N FIR - Kaminska, E IR - Kaminska E FIR - Khalidi, N IR - Khalidi N FIR - Ligier, S IR - Ligier S FIR - Masetto, A IR - Masetto A FIR - Mathieu, J-P IR - Mathieu JP FIR - Robinson, D IR - Robinson D FIR - Smith, D IR - Smith D FIR - Sutton, E IR - Sutton E FIR - Abu-Hakim, M IR - Abu-Hakim M FIR - LeClercq, S IR - LeClercq S EDAT- 2012/01/17 06:00 MHDA- 2012/07/27 06:00 CRDT- 2012/01/17 06:00 PHST- 2012/01/17 06:00 [entrez] PHST- 2012/01/17 06:00 [pubmed] PHST- 2012/07/27 06:00 [medline] AID - jrheum.110121 [pii] AID - 10.3899/jrheum.110121 [doi] PST - ppublish SO - J Rheumatol. 2012 Mar;39(3):524-31. doi: 10.3899/jrheum.110121. Epub 2012 Jan 15. PMID- 26639033 OWN - NLM STAT- MEDLINE DCOM- 20170816 LR - 20180210 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 45 IP - 6 DP - 2016 Jun TI - A review of the effects of statins in systemic sclerosis. PG - 698-705 LID - S0049-0172(15)00274-7 [pii] LID - 10.1016/j.semarthrit.2015.10.013 [doi] AB - OBJECTIVES: We performed a literature review assessing possible benefits of statins in systemic sclerosis (SSc). METHODS: PubMed, Embase, Cochrane Databases, and Medline were searched. Full-text English publications were identified in which the effects of statins in SSc were examined. Letters, review articles, and studies on morphea were excluded. RESULTS: In all, 18 of 404 studies were relevant. In vitro, statins decreased transcription and translation of IL-6 and collagen, with reversal via mevalonate. Animal studies demonstrated reduced production of Ras (a protein superfamily of GTPases), Rho (part of the Ras superfamily), and extracellular signal-regulated kinases (ERK), less fibrosis and myofibroblast transdifferentiation, and improved macrovasculature. In human studies, IL-6, an inflammatory cytokine, was reduced. Usually endothelial progenitor cell concentrations increased, and flow-mediated dilatation improved. Raynaud's phenomenon, digital ulcers, and physician global assessments improved in the majority of studies of statin treatment in SSc. None of the 256 patients receiving statins experienced transaminitis or myopathy. CONCLUSIONS: Not all findings were consistent. However, in general, in vitro, animal, and human studies demonstrated benefit in SSc pathophysiology, likely mediated through inhibition of lipid intermediate synthesis. Clinical improvement in SSc circulatory complications was observed. Statins seemed safe and well tolerated in SSc. Larger longer-term multi-site randomized trials are needed to further determine the role of statins as adjunctive treatment of this complex, heterogeneous connective tissue disease. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Ladak, Karim AU - Ladak K AD - De Groote School of Medicine, McMaster University, Hamilton, Ontario, Canada. FAU - Pope, Janet E AU - Pope JE AD - Schulich School of Medicine, Western University, London, Ontario, Canada; St. Joseph's Health Care, 268 Grosvenor St., London, Ontario, Canada N6A 4V2. Electronic address: janet.pope@sjhc.london.on.ca. LA - eng PT - Journal Article PT - Review DEP - 20151102 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Interleukin-6) RN - 9007-34-5 (Collagen) RN - EC 2.7.11.24 (Extracellular Signal-Regulated MAP Kinases) RN - EC 3.6.5.2 (ras Proteins) SB - IM MH - Animals MH - Blood Vessels/drug effects MH - Cell Transdifferentiation/drug effects MH - Collagen/drug effects/metabolism MH - Endothelial Progenitor Cells/cytology/drug effects MH - Extracellular Signal-Regulated MAP Kinases/drug effects/metabolism MH - Fibrosis MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/pharmacology/*therapeutic use MH - In Vitro Techniques MH - Interleukin-6/metabolism MH - Myofibroblasts/drug effects MH - Raynaud Disease/etiology/physiopathology MH - Scleroderma, Systemic/complications/*drug therapy/metabolism/physiopathology MH - Skin Ulcer/etiology/physiopathology MH - Vasodilation/drug effects MH - ras Proteins/drug effects/metabolism OTO - NOTNLM OT - Animal models OT - Cytokines OT - Digital ulcers OT - Endothelial progenitor cells OT - Fibroblasts OT - Hydroxy-methyl-glutaryl-CoA inhibitors OT - Pleiotropic OT - Review OT - Statins OT - Systemic sclerosis (scleroderma) OT - Trials EDAT- 2015/12/08 06:00 MHDA- 2017/08/17 06:00 CRDT- 2015/12/08 06:00 PHST- 2015/06/28 00:00 [received] PHST- 2015/10/10 00:00 [revised] PHST- 2015/10/23 00:00 [accepted] PHST- 2015/12/08 06:00 [entrez] PHST- 2015/12/08 06:00 [pubmed] PHST- 2017/08/17 06:00 [medline] AID - S0049-0172(15)00274-7 [pii] AID - 10.1016/j.semarthrit.2015.10.013 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Jun;45(6):698-705. doi: 10.1016/j.semarthrit.2015.10.013. Epub 2015 Nov 2. PMID- 35947536 OWN - NLM STAT- MEDLINE DCOM- 20220812 LR - 20250728 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 23 DP - 2022 Aug 10 TI - Erasmus Syndrome: A Case Report and Literature Review. PG - e937061 LID - 10.12659/AJCR.937061 [doi] AB - BACKGROUND Erasmus syndrome is a rare disease entity characterized by the development of systemic sclerosis (SSc) in a background of silica exposure or silicosis. CASE REPORT We report the case of a 40-year-old Filipino man who previously worked in a silica grind mill for 10 years and eventually developed Erasmus syndrome. The patient initially presented with chronic back pain in 2018 associated with findings of pulmonary tuberculosis on chest X-ray, with notable improvement after 6 months of anti-tuberculosis treatment. However, his back pain recurred in 2021; this time with arthralgia, Raynaud's phenomenon, thickening of both hands, skin hypopigmentation on the chest, back, and forehead, and exertional dyspnea. Physical examination revealed salt-and-pepper dermopathy and skin tightening over the back, chest, and extremities. Mobility of his hands was limited, associated with sclerodactyly and digital pitting. Antinuclear antibody-immunofluorescence and anti-scleroderma-70 antibodies were strongly positive, confirming the diagnosis of SSc. Chest computed tomography illustrated multiple subcentimeter nodules and enlarged mediastinal lymph nodes with eggshell calcifications, consistent with silicosis. Spirometry with body plethysmography was normal but diffusing capacity for carbon monoxide was severely reduced. Histopathology of the skin showed markedly thickened collagen bundles in the dermis. CONCLUSIONS Chronic silica exposure is a risk factor for the development of silicosis. The clinical course of patients with silicosis may be complicated by SSc. Maintaining a high index of suspicion is key to the diagnosis of Erasmus syndrome. The present report emphasizes the importance of preventive measures and surveillance among those with occupational exposure to silica. To our knowledge, this is the first documented case of Erasmus syndrome in the Philippines. FAU - Lomanta, Jan Michael Jesse AU - Lomanta JMJ AD - Division of Pulmonary Medicine, Department of Medicine, Philippine General Hospital, Manila, Philippines. FAU - Atienza, Mary Antonette AU - Atienza MA AD - Department of Dermatology, Philippine General Hospital , Manila, Philippines. FAU - Gonzales, Juan Raphael M AU - Gonzales JRM AD - Division of Rheumatology, Department of Medicine, Philippine General Hospital, Manila, Philippines. FAU - Amante, Eric Jason Bautista AU - Amante EJB AD - Division of Rheumatology, Department of Medicine, Philippine General Hospital, Manila, Philippines. FAU - Urquiza, Sheen C AU - Urquiza SC AD - Department of Radiology, Adela Serra Ty Memorial Medical Center, Tandag City, Surigao del Sur, Philippines. FAU - Lucero-Orillaza, Hanna AU - Lucero-Orillaza H AD - Department of Dermatology, Philippine General Hospital, Manila, Philippines. FAU - Santiaguel, Joel Marquez AU - Santiaguel JM AD - Division of Pulmonary Medicine, Department of Medicine, Philippine General Hospital, Manila, Philippines. AD - Department of Medicine, Quirino Memorial Medical Center, Quezon City, Philippines. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20220810 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 RN - 0 (Antibodies, Antinuclear) RN - 0 (anti-scl-70 autoantibodies) RN - 7631-86-9 (Silicon Dioxide) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - Humans MH - *Raynaud Disease/complications MH - *Scleroderma, Systemic/diagnosis MH - Silicon Dioxide MH - *Silicosis/complications/diagnosis MH - Syndrome PMC - PMC9377720 COIS- Conflict of interest: None declared EDAT- 2022/08/11 06:00 MHDA- 2022/08/13 06:00 PMCR- 2022/08/10 CRDT- 2022/08/10 13:22 PHST- 2022/08/10 13:22 [entrez] PHST- 2022/08/11 06:00 [pubmed] PHST- 2022/08/13 06:00 [medline] PHST- 2022/08/10 00:00 [pmc-release] AID - 937061 [pii] AID - 10.12659/AJCR.937061 [doi] PST - epublish SO - Am J Case Rep. 2022 Aug 10;23:e937061. doi: 10.12659/AJCR.937061. PMID- 28564618 OWN - NLM STAT- MEDLINE DCOM- 20180103 LR - 20260127 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 16 IP - 8 DP - 2017 Aug TI - Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort. PG - 796-802 LID - S1568-9972(17)30137-4 [pii] LID - 10.1016/j.autrev.2017.05.013 [doi] AB - OBJECTIVES: According to the existence of subclinical organ involvement pre-scleroderma should be divided into two subsets: very early and early disease. Pre-scleroderma patients included in the Spanish Scleroderma Registry (RESCLE) Cohort were reclassified into subsets. Differences were evaluated and the risk of progression to definite systemic sclerosis was estimated. METHODS: The characteristics of very early and early SSc patients were compared. A logistic regression model was used to determine the risk factors of progression. RESULTS: 1632 patients were included, 36 (2.2%) in the very early subset and 111 (6.8%) in the early subset. There were no differences in sex, age at disease onset, duration of Raynaud's phenomenon, antinuclear antibodies or capillaroscopic findings. Three (8.3%) very early SSc patients evolved to definite SSc, 2 (5.6%) of them meeting the ACR/EULAR 2013 criteria, unlike 31 (28%) early SSc patients, 20 (24%) of them meeting the criteria (p=0.034). Digestive involvement was an independent risk factor of progression (OR 17; 95% CI, 6.1-47.2). CONCLUSIONS: The classification of early forms of scleroderma identifies patients with different prognostic risk of progression. The evolution to definite SSc is more frequent in early than in very early SSc patients. Digestive involvement is a risk factor of progression. An active assessment of organ damage in preclinical stages allows a correct classification and risk stratification, with implications for monitoring and treatment. CI - Copyright © 2017 Elsevier B.V. All rights reserved. FAU - Trapiella-Martínez, Luis AU - Trapiella-Martínez L AD - Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain. Electronic address: luistrapiella@yahoo.es. FAU - Díaz-López, José Bernardino AU - Díaz-López JB AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Caminal-Montero, Luis AU - Caminal-Montero L AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Tolosa-Vilella, Carles AU - Tolosa-Vilella C AD - Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain. FAU - Guillén-Del Castillo, Alfredo AU - Guillén-Del Castillo A AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Colunga-Argüelles, Dolores AU - Colunga-Argüelles D AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Rubio-Rivas, Manuel AU - Rubio-Rivas M AD - Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain. FAU - Iniesta-Arandia, Nerea AU - Iniesta-Arandia N AD - Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Clínic, Barcelona, Spain. FAU - Castillo-Palma, María Jesús AU - Castillo-Palma MJ AD - Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain. FAU - Sáez-Comet, Luis AU - Sáez-Comet L AD - Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain. FAU - Egurbide-Arberas, María Victoria AU - Egurbide-Arberas MV AD - Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Systemic Autoimmune Diseases Unit, Hospital Campus de la Salud, Complejo Universitario de Granada, Spain. FAU - Freire, Mayka AU - Freire M AD - Thrombosis and Vasculitis Unit, Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Spain. FAU - Vargas-Hitos, Jose Antonio AU - Vargas-Hitos JA AD - Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain. FAU - Ríos-Blanco, Juan José AU - Ríos-Blanco JJ AD - Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain. FAU - Todolí-Parra, Jose Antonio AU - Todolí-Parra JA AD - Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain. FAU - Rodríguez-Carballeira, Mónica AU - Rodríguez-Carballeira M AD - Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain. FAU - Marín-Ballvé, Adela AU - Marín-Ballvé A AD - Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Chamorro-Fernández, Antonio Javier AU - Chamorro-Fernández AJ AD - Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Spain. FAU - Pla-Salas, Xavier AU - Pla-Salas X AD - Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain. FAU - Madroñero-Vuelta, Ana Belén AU - Madroñero-Vuelta AB AD - Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain. FAU - Ruiz-Muñóz, Manuel AU - Ruiz-Muñóz M AD - Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain. FAU - Fonollosa-Pla, Vicent AU - Fonollosa-Pla V AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Simeón-Aznar, Carmen Pilar AU - Simeón-Aznar CP AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. CN - RESCLE Investigators, on Behalf of the Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) LA - eng PT - Journal Article DEP - 20170528 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/immunology MH - Cohort Studies MH - Disease Progression MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prognosis MH - Raynaud Disease/epidemiology/immunology MH - Registries MH - Risk Factors MH - Scleroderma, Systemic/*diagnosis/epidemiology/immunology MH - Spain/epidemiology MH - Young Adult OTO - NOTNLM OT - Early systemic sclerosis OT - Pre-scleroderma OT - Very early systemic sclerosis EDAT- 2017/06/01 06:00 MHDA- 2018/01/04 06:00 CRDT- 2017/06/01 06:00 PHST- 2017/05/06 00:00 [received] PHST- 2017/05/12 00:00 [accepted] PHST- 2017/06/01 06:00 [pubmed] PHST- 2018/01/04 06:00 [medline] PHST- 2017/06/01 06:00 [entrez] AID - S1568-9972(17)30137-4 [pii] AID - 10.1016/j.autrev.2017.05.013 [doi] PST - ppublish SO - Autoimmun Rev. 2017 Aug;16(8):796-802. doi: 10.1016/j.autrev.2017.05.013. Epub 2017 May 28. PMID- 40854723 OWN - NLM STAT- MEDLINE DCOM- 20250826 LR - 20250915 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 18 IP - 8 DP - 2025 Aug 25 TI - Paraneoplastic acral vascular syndrome in a patient with non-small cell lung adenocarcinoma with KRAS G12A oncogenic mutation. LID - e265443 [pii] LID - 10.1136/bcr-2025-265443 [doi] AB - Paraneoplastic acral vascular syndrome (PAVS) is a rare condition characterised by signs of digital ischaemia, including Raynaud's phenomenon, acrocyanosis and digital gangrene, in the context of an underlying neoplastic process. Prompt recognition of this syndrome, as well as accurate identification and classification of the associated neoplastic condition, are crucial.This report describes the case of a woman diagnosed with PAVS secondary to stage IV non-small cell lung adenocarcinoma harbouring a Kirsten Rat Sarcoma viral homolog oncogene (KRAS) G12A mutation (p.Gly12Ala). CI - © BMJ Publishing Group Limited 2025. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Arroyo Bravo, Miriam AU - Arroyo Bravo M AUID- ORCID: 0009-0004-4819-2344 AD - Internal Medicine, Parc Taulí Hospital Universitari, Sabadell, Spain. AD - Institut d'Investigació i Innovació Parc Taulí (I3PT-CERCA), Universitat Autònoma de Barcelona, Sabadell, Spain. FAU - Díaz-Martín, Estefanía AU - Díaz-Martín E AUID- ORCID: 0009-0007-0079-8752 AD - Internal Medicine, Parc Taulí Hospital Universitari, Sabadell, Spain. AD - Institut d'Investigació i Innovació Parc Taulí (I3PT-CERCA), Universitat Autònoma de Barcelona, Sabadell, Spain. FAU - Sierra-Boada, Marina AU - Sierra-Boada M AUID- ORCID: 0009-0006-6953-8505 AD - Institut d'Investigació i Innovació Parc Taulí (I3PT-CERCA), Universitat Autònoma de Barcelona, Sabadell, Spain msierra@tauli.cat. AD - Medical Oncology Department, Parc Taulí Hospital Universitari, Sabadell, Barcelona, Spain. FAU - Villacé Gallego, Pablo AU - Villacé Gallego P AD - Internal Medicine, Parc Taulí Hospital Universitari, Sabadell, Spain. AD - Institut d'Investigació i Innovació Parc Taulí (I3PT-CERCA), Universitat Autònoma de Barcelona, Sabadell, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20250825 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - EC 3.6.5.2 (Proto-Oncogene Proteins p21(ras)) RN - 0 (KRAS protein, human) SB - IM MH - Humans MH - Female MH - *Paraneoplastic Syndromes/genetics/diagnosis/etiology MH - *Lung Neoplasms/genetics/complications/pathology MH - *Proto-Oncogene Proteins p21(ras)/genetics MH - *Fingers/blood supply MH - Mutation MH - *Carcinoma, Non-Small-Cell Lung/genetics/complications/pathology MH - Adenocarcinoma of Lung/genetics MH - Raynaud Disease/etiology MH - Middle Aged OTO - NOTNLM OT - Cancer intervention OT - Lung cancer (oncology) OT - Thrombosis COIS- Competing interests: None declared. EDAT- 2025/08/26 00:45 MHDA- 2025/08/27 00:44 CRDT- 2025/08/25 21:23 PHST- 2025/08/27 00:44 [medline] PHST- 2025/08/26 00:45 [pubmed] PHST- 2025/08/25 21:23 [entrez] AID - 18/8/e265443 [pii] AID - 10.1136/bcr-2025-265443 [doi] PST - epublish SO - BMJ Case Rep. 2025 Aug 25;18(8):e265443. doi: 10.1136/bcr-2025-265443. PMID- 37421381 OWN - NLM STAT- MEDLINE DCOM- 20240603 LR - 20250716 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 6 DP - 2024 May 3 TI - Assessment of digital perfusion as a surrogate outcome in Raynaud's phenomenon clinical trials. PG - 1502-1506 LID - 10.1093/rheumatology/kead337 [doi] AB - OBJECTIVES: Measurement of digital perfusion, sometimes coupled with a cold challenge, has been widely used as an objective outcome in trials evaluating drug therapies in RP, in addition to patient-reported outcomes or to establish the proof-of-concept in preliminary studies. However, whether digital perfusion is a valid surrogate for clinical outcomes in RP trials has never been explored. The principal aim of this study was to evaluate the potential surrogacy of digital perfusion, by combining individual-level and trial-level data. METHODS: We used individual data from a series of n-of-1 trials, and trial data from a network meta-analysis. We estimated individual-level surrogacy through coefficients of determination between digital perfusion and clinical outcomes (R2ind). We further calculated the coefficients of determination between treatment effect on the clinical outcomes and on digital perfusion, at the individual level (R2TEind) and at the trial level (R2trial), using non-weighted linear regression, with their 95% CI calculated through bootstrapping. RESULTS: Results from 33 patients and 24 trials were included in the final analysis. At the individual level, there was no correlation between digital perfusion and clinical outcomes at rest and in response to various cooling tests (the highest R2ind was 0.03 [-0.07, 0.09]), and R2TEind was also very low 0.07 (0, 0.29). At the trial level, the highest value of R2trial was 0.1 (0, 0.477). CONCLUSIONS: Digital perfusion, at rest or in response to a cold challenge, and whatever the method used, does not fulfil the criteria of a valid surrogate for existing patient-reported outcomes in RP trials. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Guigui, Alicia AU - Guigui A AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. FAU - Liaigre, Léa AU - Liaigre L AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. FAU - Manceau, Marc AU - Manceau M AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. FAU - Gaget, Olivier AU - Gaget O AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Pharmacovigilance Unit, Univ. Grenoble Alpes, CHU Grenoble Alpes, Grenoble, France. FAU - Blaise, Sophie AU - Blaise S AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Department of Vascular Medicine, Univ. Grenoble Alpes, CHU Grenoble Alpes, Grenoble, France. FAU - Khouri, Charles AU - Khouri C AUID- ORCID: 0000-0002-8427-8573 AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. AD - Pharmacovigilance Unit, Univ. Grenoble Alpes, CHU Grenoble Alpes, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AUID- ORCID: 0000-0003-4475-1626 AD - Univ. Grenoble Alpes, Inserm, CHU Grenoble Alpes, HP2, Grenoble, France. AD - Univ. Grenoble Alpes, Inserm CIC1406, CHU Grenoble Alpes, Grenoble, France. LA - eng GR - ANR-19-P3IA-0003/MIAI @ Grenoble Alpes/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Clinical Trials as Topic MH - *Fingers/blood supply MH - *Raynaud Disease/drug therapy MH - Treatment Outcome OTO - NOTNLM OT - RP OT - SSc OT - skin perfusion OT - surrogacy EDAT- 2023/07/08 19:41 MHDA- 2024/06/04 00:45 CRDT- 2023/07/08 09:43 PHST- 2023/04/17 00:00 [received] PHST- 2023/06/27 00:00 [accepted] PHST- 2024/06/04 00:45 [medline] PHST- 2023/07/08 19:41 [pubmed] PHST- 2023/07/08 09:43 [entrez] AID - 7221532 [pii] AID - 10.1093/rheumatology/kead337 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 May 3;63(6):1502-1506. doi: 10.1093/rheumatology/kead337. PMID- 36624179 OWN - NLM STAT- MEDLINE DCOM- 20230417 LR - 20260127 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 42 IP - 5 DP - 2023 May TI - Does the use of nail cosmetics interfere with the reporting of nailfold capillaroscopy? PG - 1307-1313 LID - 10.1007/s10067-023-06503-0 [doi] AB - Capillaroscopy is a non-invasive tool evaluating the nail-fold capillaries, especially in approach to Raynaud's phenomenon (RP) and scleroderma (SSc) spectrum of diseases. It was recommended that the patients should not use any cosmetic procedure involving the nailfold to avoid misinterpretations. Therefore, we aimed to find the problems of using or recent removal of the nail polishes/artificial nails/henna before doing capillaroscopy. During 10 years, we looked for all capillaroscopy reports and nail fold images of patients who referred for capillaroscopy and had used or recently removed nail polish/artificial nail/henna in order to find the presence of any artifacts or misinterpretations in reports. Sixty-three patients were identified that had used or removed the nail cosmetic during 10 days before capillaroscopy. In patients who used nail polish, removed their nail polish, used artificial nails, or removed the artificial nails, and those using henna, 16.2%, 36.4%, 3.8%, 0%, and 1.4% of nail folds showed some stains in the upper part of the nail fold area, respectively, that had no interference with the report due to their distinct color. However, few areas were covered with polish stains in patients who removed the polish recently. The presence of nail cosmetics including nail polishes, henna, and artificial nails at the time of capillaroscopy does not induce a significant misinterpretation in capillaroscopy. However, some colored stains which were mostly distinguishable from hemorrhages could be seen. The removal of them 10 days before the time of capillaroscopy did not decrease the cosmetic artifacts. Key Points • The presence of nail cosmetics including nail polishes, henna, and artificial nails at the time of capillaroscopy does not induce a significant misinterpretation in capillaroscopy. • The removal of nail polish 10 days before the time of capillaroscopy did not decrease the procedure artifacts. • The removal of nail polish 10 days before capillaroscopy increased the area of artifacts and coverage of the study background. CI - © 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AUID- ORCID: 0000-0003-0522-7186 AD - Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Iran. shenavande@sums.ac.ir. LA - eng PT - Journal Article DEP - 20230110 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Cosmetics) RN - TLH4A6LV1W (lawsone) RN - 0 (Naphthoquinones) SB - IM MH - Humans MH - Nails/diagnostic imaging/blood supply MH - Microscopic Angioscopy MH - *Raynaud Disease/diagnostic imaging MH - *Cosmetics MH - Capillaries/diagnostic imaging MH - Naphthoquinones PMC - PMC9838451 OTO - NOTNLM OT - Artificial nail OT - Capillaroscopy OT - Henna OT - Nail polish OT - Nailfold EDAT- 2023/01/10 06:00 MHDA- 2023/04/17 06:41 PMCR- 2023/01/13 CRDT- 2023/01/09 23:20 PHST- 2022/11/24 00:00 [received] PHST- 2022/12/30 00:00 [accepted] PHST- 2022/12/23 00:00 [revised] PHST- 2023/04/17 06:41 [medline] PHST- 2023/01/10 06:00 [pubmed] PHST- 2023/01/09 23:20 [entrez] PHST- 2023/01/13 00:00 [pmc-release] AID - 10.1007/s10067-023-06503-0 [pii] AID - 6503 [pii] AID - 10.1007/s10067-023-06503-0 [doi] PST - ppublish SO - Clin Rheumatol. 2023 May;42(5):1307-1313. doi: 10.1007/s10067-023-06503-0. Epub 2023 Jan 10. PMID- 27751625 OWN - NLM STAT- MEDLINE DCOM- 20170614 LR - 20210103 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 41 IP - 6 DP - 2016 Dec TI - Paraneoplastic systemic sclerosis: About 3 cases and review of literature. PG - 365-370 LID - S0398-0499(16)30065-8 [pii] LID - 10.1016/j.jmv.2016.07.001 [doi] AB - OBJECTIVE: Association between cancer and systemic sclerosis (SSc) has been described. However, paraneoplastic SSc is not well known. The aim of this article is to describe cases of paraneoplastic systemic sclerosis and to compare them to other cases of the literature, to find characteristics that can make suspect a paraneoplastic mechanism when SSc is diagnosed. METHODS: We retrospectively analyzed patients, in our department who, over the last 15 years, presented with Raynaud's phenomenon with a diagnosis of SSc (including cancer during the period of SSc). Treatment of cancer had to be concomitted with an improvement of sclerosis and/or negativation of antinuclear antibodies. Review analysis of other cases was made with Pubmed. RESULTS: Three patients responded to the criteria. Two of them had an ovarian cancer with peritoneal carcinomatosis and a concomitted SSc. One had a colon cancer with an ovarian metastasis diagnosed 1 year before SSc. None of them had anti-ENA antibody. One had a normal capillaroscopy. After treatment of cancer, all of them had an improvement of sclerosis and a negativation of antinuclear antibodies. CONCLUSION: We believe that physicians should be aware of paraneoplastic SSc when presentation of SSc is atypical: no anti-ENA (anti-SCl70 or anti-centromere) or normal capillaroscopy, in particular gynecologic cancer in women. CI - Copyright © 2016 Elsevier Masson SAS. All rights reserved. FAU - Monfort, J-B AU - Monfort JB AD - Service de médecine vasculaire, groupe hospitalier Paris Saint-Joseph, 185, rue Raymond-Losserand, 75014 Paris, France. Electronic address: jb.monfort@wanadoo.fr. FAU - Lazareth, I AU - Lazareth I AD - Service de médecine vasculaire, groupe hospitalier Paris Saint-Joseph, 185, rue Raymond-Losserand, 75014 Paris, France. FAU - Priollet, P AU - Priollet P AD - Service de médecine vasculaire, groupe hospitalier Paris Saint-Joseph, 185, rue Raymond-Losserand, 75014 Paris, France. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20161015 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adenocarcinoma/complications/therapy MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/blood MH - Carcinoma/complications/drug therapy/surgery MH - Colonic Neoplasms/complications/therapy MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Ovarian Neoplasms/complications/drug therapy/surgery MH - Paraneoplastic Syndromes/*complications/diagnosis MH - Peritoneal Neoplasms/complications/drug therapy/surgery MH - Raynaud Disease/complications MH - Remission Induction MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/diagnosis OTO - NOTNLM OT - Cancer OT - Paraneoplastic OT - Paranéoplasique OT - Sclérodermie systémique OT - Systemic sclerosis EDAT- 2016/10/19 06:00 MHDA- 2017/06/15 06:00 CRDT- 2016/10/19 06:00 PHST- 2016/04/27 00:00 [received] PHST- 2016/07/01 00:00 [accepted] PHST- 2016/10/19 06:00 [pubmed] PHST- 2017/06/15 06:00 [medline] PHST- 2016/10/19 06:00 [entrez] AID - S0398-0499(16)30065-8 [pii] AID - 10.1016/j.jmv.2016.07.001 [doi] PST - ppublish SO - J Mal Vasc. 2016 Dec;41(6):365-370. doi: 10.1016/j.jmv.2016.07.001. Epub 2016 Oct 15. PMID- 28704466 OWN - NLM STAT- MEDLINE DCOM- 20170926 LR - 20260130 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 12 IP - 7 DP - 2017 TI - Hand-arm vibration and the risk of vascular and neurological diseases-A systematic review and meta-analysis. PG - e0180795 LID - 10.1371/journal.pone.0180795 [doi] LID - e0180795 AB - BACKGROUND: Increased occurrence of Raynaud's phenomenon, neurosensory injury and carpal tunnel syndrome has been reported for more than 100 years in association with work with vibrating machines. The current risk prediction modelling (ISO-5349) for "Raynaud's phenomenon" is based on a few studies published 70 to 40 years ago. There are no corresponding risk prediction models for neurosensory injury or carpal tunnel syndrome, nor any systematic reviews comprising a statistical synthesis (meta-analysis) of the evidence. OBJECTIVES: Our aim was to provide a systematic review of the literature on the association between Raynaud's phenomenon, neurosensory injuries and carpal tunnel syndrome and hand-arm vibration (HAV) exposure. Moreover the aim was to estimate the magnitude of such an association using meta-analysis. METHODS: This systematic review covers the scientific literature up to January 2016. The databases used for the literature search were PubMed and Science Direct. We found a total of 4,335 abstracts, which were read and whose validity was assessed according to pre-established criteria. 294 articles were examined in their entirety to determine whether each article met the inclusion criteria. The possible risk of bias was assessed for each article. 52 articles finally met the pre-established criteria for inclusion in the systematic review. RESULTS: The results show that workers who are exposed to HAV have an increased risk of vascular and neurological diseases compared to non-vibration exposed groups. The crude estimate of the risk increase is approximately 4-5 fold. The estimated effect size (odds ratio) is 6.9 for the studies of Raynaud's phenomenon when including only the studies judged to have a low risk of bias. The corresponding risk of neurosensory injury is 7.4 and the equivalent of carpal tunnel syndrome is 2.9. CONCLUSION: At equal exposures, neurosensory injury occurs with a 3-time factor shorter latency than Raynaud's phenomenon. Which is why preventive measures should address this vibration health hazard with greater attention. FAU - Nilsson, Tohr AU - Nilsson T AUID- ORCID: 0000-0003-2789-6321 AD - Occupational and Environmental Medicine, Department of Public Health & Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Wahlström, Jens AU - Wahlström J AD - Occupational and Environmental Medicine, Department of Public Health & Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Burström, Lage AU - Burström L AD - Occupational and Environmental Medicine, Department of Public Health & Clinical Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20170713 PL - United States TA - PLoS One JT - PloS one JID - 101285081 SB - IM MH - Carpal Tunnel Syndrome/*epidemiology MH - Hand-Arm Vibration Syndrome/*epidemiology MH - Humans MH - Nervous System Diseases/epidemiology MH - Raynaud Disease/*epidemiology MH - Risk Assessment PMC - PMC5509149 COIS- Competing Interests: The study was supported by Swedish AFA Insurance, The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript, this does not alter our adherence to the PLOS ONE policies on sharing data and material. EDAT- 2017/07/14 06:00 MHDA- 2017/09/28 06:00 PMCR- 2017/07/13 CRDT- 2017/07/14 06:00 PHST- 2016/10/04 00:00 [received] PHST- 2017/06/21 00:00 [accepted] PHST- 2017/07/14 06:00 [entrez] PHST- 2017/07/14 06:00 [pubmed] PHST- 2017/09/28 06:00 [medline] PHST- 2017/07/13 00:00 [pmc-release] AID - PONE-D-16-39416 [pii] AID - 10.1371/journal.pone.0180795 [doi] PST - epublish SO - PLoS One. 2017 Jul 13;12(7):e0180795. doi: 10.1371/journal.pone.0180795. eCollection 2017. PMID- 24970785 OWN - NLM STAT- MEDLINE DCOM- 20160202 LR - 20141206 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 39 IP - 6 DP - 2014 Dec TI - [False traumatic aneurysm of the ulnar artery in a teenager]. PG - 426-9 LID - S0398-0499(14)00096-1 [pii] LID - 10.1016/j.jmv.2014.04.013 [doi] AB - Most aneurysms of hand arteries are traumatic. It is a generally rare unrecognized pathology. Complications are serious (embolism and thromboses of interdigital arteries). Two main causes can be recalled: acute trauma, with development of a false aneurysm; repeated microtrauma (hand hammer syndrome), with occurrence of an arterial dysplasic aneurysm. The diagnosis is based on the presence of a pulsatile mass, with finger dysesthesia, unilateral Raynaud's phenomenon. It is confirmed by duplex Doppler. Arteriography is necessary but can be replaced by an angio-MR. We report a case of false traumatic aneurysm of the ulnar artery in a teenager. This case illustrates this rare condition and opens discussion on therapeutic options. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Nour, M AU - Nour M AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. Electronic address: nour.merouane@yahoo.fr. FAU - Talha, H AU - Talha H AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - El Idrissi, R AU - El Idrissi R AD - Service de chirurgie vasculaire D, CHU Avicenne, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Lahraoui, Y AU - Lahraoui Y AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Ouazzani, L AU - Ouazzani L AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Oubejja, H AU - Oubejja H AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Erraji, M AU - Erraji M AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Zerhouni, H AU - Zerhouni H AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. FAU - Ettayebi, F AU - Ettayebi F AD - Service des urgences chirurgicales pédiatriques, hôpital des enfants, boulevard Ibn Rochd, 10100 Souissi, Rabat, Maroc; Université Mohamed V - Souissi, Rabat, Maroc. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Faux anévrysme traumatique de l'artère ulnaire chez un adolescent. DEP - 20140623 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Adolescent MH - Aneurysm, False/*diagnosis/etiology/pathology MH - Angiography MH - Fingers MH - Hand/blood supply MH - Hand Injuries/complications MH - Humans MH - Magnetic Resonance Angiography MH - Male MH - Paresthesia MH - Raynaud Disease MH - Ulnar Artery/*injuries/pathology MH - Ultrasonography, Doppler, Duplex OTO - NOTNLM OT - Adolescent OT - Anévrysme post-traumatique OT - Artère ulnaire OT - Post-traumatic aneurysm OT - Teenager OT - Ulnar artery EDAT- 2014/06/28 06:00 MHDA- 2016/02/03 06:00 CRDT- 2014/06/28 06:00 PHST- 2013/07/25 00:00 [received] PHST- 2014/04/30 00:00 [accepted] PHST- 2014/06/28 06:00 [entrez] PHST- 2014/06/28 06:00 [pubmed] PHST- 2016/02/03 06:00 [medline] AID - S0398-0499(14)00096-1 [pii] AID - 10.1016/j.jmv.2014.04.013 [doi] PST - ppublish SO - J Mal Vasc. 2014 Dec;39(6):426-9. doi: 10.1016/j.jmv.2014.04.013. Epub 2014 Jun 23. PMID- 26631100 OWN - NLM STAT- MEDLINE DCOM- 20161007 LR - 20220317 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 35 IP - 1 DP - 2016 Jan TI - Combination therapy with Bosentan and Sildenafil improves Raynaud's phenomenon and fosters the recovery of microvascular involvement in systemic sclerosis. PG - 127-32 LID - 10.1007/s10067-015-3119-3 [doi] AB - The aim of this study was to evaluate in systemic sclerosis (SSc) retrospectively the effect of Bosentan and Sildenafil and their combination on Raynaud's phenomenon (RP), function, and capillaroscopic patterns. One hundred and twenty-three SSc patients (mean age ± sd, 57.69 ± 14.07 years) were retrospectively evaluated and divided into two groups according to American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification score: group 1 score < 10, group 2 score > 10. Each group was divided into three subgroups according to treatment: Bosentan, Sildenafil, and Bosentan + Sildenafil. Nailfold videocapillaroscopy (NVC), Scleroderma Health Assessment Questionnaire (SHAQ) and Raynaud Condition Score (RCS) were performed at baseline and after 3 and 6 months. In Bosentan (29 patients: 12, group 1; 17, group 2), NVC changed significantly in both groups, after 3 and 6 months (p = 0.00439, group 1; p = 0.00035, group 2). In group 1, the "active" and the "late" patterns reduced, and the "aspecific" increased. In group 2, there was a reduction of late patterns, a worsening of SHAQ (p < 0.005) and an improvement of RCS (p = 0.00014). In Sildenafil (63 patients: 35, group 1; 28, group 2), after 3 months, NVC patterns changed significantly in both groups(p = 0.042 group 1, p = 0.00089 group 2). In group 1, the late and early patterns increased, and the aspecific decreased. In group 2, a significant change of NVC pattern was observed also after 6 months (p = 0.00089): the late pattern increased while the active one reduced. After 6 months, SHAQ was significantly reduced in group 1 (p = 0.00027) and in group 2 (p = 0.0043). RCS improved in both groups (p = 0.0042, group 1; p = 0.0016, group 2). Combination therapy (Bosentan + Sildenafil) (31 patients: 14, group 1; 17, group 2) induced significant changes on NVC only in group 1 after 3 (p = 0.00256) and 6 months (p = 0.000349) with a reduction of the late and active patterns and an increase of the early pattern. In both groups, after 6 months, SHAQ (p < 0.05, group 1; p = 0.00049, group 2) and RCS significantly reduced (group 1, p = 0.00024; group 2, p = 0.0021). Patients treated with Bosentan + Sildenafil show a significant improvement of RCS and NVC. This combination therapy may exert a vascular activity achieving an amelioration of the structure of microvasculature in SSc. FAU - Bellando-Randone, S AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. s.bellandorandone@gmail.com. FAU - Lepri, G AU - Lepri G AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. lepri.gemma@gmail.com. FAU - Bruni, C AU - Bruni C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Blagojevic, J AU - Blagojevic J AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Radicati, A AU - Radicati A AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Cometi, L AU - Cometi L AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - De Paulis, A AU - De Paulis A AD - Department of Translational Medical Sciences, Centre for Basic and Clinical Immunology Research (CISI), University of Naples Federico II, Naples, Italy. FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Guiducci, S AU - Guiducci S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. LA - eng PT - Journal Article DEP - 20151203 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Bosentan MH - Capillaries/physiopathology MH - Drug Therapy, Combination MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Microvessels/*drug effects MH - Middle Aged MH - Nails/blood supply MH - Raynaud Disease/*drug therapy MH - Retrospective Studies MH - Scleroderma, Systemic/*drug therapy MH - Sildenafil Citrate/*administration & dosage MH - Sulfonamides/*administration & dosage MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage OTO - NOTNLM OT - Bosentan OT - Microvasculature OT - Sildenafil OT - Systemic sclerosis EDAT- 2015/12/04 06:00 MHDA- 2016/10/08 06:00 CRDT- 2015/12/04 06:00 PHST- 2015/08/20 00:00 [received] PHST- 2015/11/10 00:00 [accepted] PHST- 2015/10/26 00:00 [revised] PHST- 2015/12/04 06:00 [entrez] PHST- 2015/12/04 06:00 [pubmed] PHST- 2016/10/08 06:00 [medline] AID - 10.1007/s10067-015-3119-3 [pii] AID - 10.1007/s10067-015-3119-3 [doi] PST - ppublish SO - Clin Rheumatol. 2016 Jan;35(1):127-32. doi: 10.1007/s10067-015-3119-3. Epub 2015 Dec 3. PMID- 41191395 OWN - NLM STAT- MEDLINE DCOM- 20260224 LR - 20260527 IS - 1878-6847 (Electronic) IS - 0924-6479 (Linking) VI - 37 IP - 1 DP - 2026 Feb TI - Nifedipine and mycophenolate mofetil-induced gingival hyperplasia in a patient with systemic sclerosis: Role of concomitant therapy and pharmacovigilance aspects. PG - 144-147 LID - 10.1177/09246479251389217 [doi] AB - BackgroundDrug-induced gingival overgrowth (DIGO) is a common adverse effect of anticonvulsants, calcium channel blockers (CCBs), and immunosuppressants like cyclosporine, with nifedipine being the most frequently implicated CCB. In contrast, mycophenolate mofetil (MMF)-induced DIGO is rare due to its antifibrotic properties.Case reportWe report a case of a 26-year-old female with systemic sclerosis (SSc), receiving nifedipine and MMF for Raynaud's phenomenon and interstitial lung disease, who presented with grade I gingival hyperplasia confined to interdental papillae. Despite good oral hygiene, gingival inflammation, microstomia, and plaque accumulation contributed to DIGO. Nifedipine was substituted with tadalafil, and MMF was replaced with nintedanib due to worsening interstitial lung disease. The pathogenesis of DIGO involves TGF-β-mediated fibroblast proliferation and extracellular matrix deposition, mechanisms also implicated in SSc-related fibrosis. Genetic polymorphisms in TGF-β and fibroblast regulatory genes further increase susceptibility. Effective management includes drug substitution, meticulous oral hygiene, and surgical excision for persistent cases.ConclusionThis report highlights the need for vigilant pharmacovigilance in SSc patients, emphasizing early detection, genetic risk assessment, and preventive dental care to mitigate DIGO progression. Personalized risk stratification based on genetic profiling may enhance DIGO prevention and therapeutic strategies in high-risk individuals. FAU - Gowda, Shreya K AU - Gowda SK AD - Department of Dermatology and Venereology, All India Institute of Medical Sciences, Bhubaneshwar, India. FAU - Priyadarshan, Bevan AU - Priyadarshan B AD - Department of Dermatology and Venereology, All India Institute of Medical Sciences, Bhubaneshwar, India. FAU - Behera, Biswanath AU - Behera B AD - Department of Dermatology and Venereology, All India Institute of Medical Sciences, Bhubaneshwar, India. FAU - Thakur, Vishal AU - Thakur V AD - Department of Dermatology and Venereology, All India Institute of Medical Sciences, Bhubaneshwar, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20251105 PL - United States TA - Int J Risk Saf Med JT - The International journal of risk & safety in medicine JID - 9100907 RN - HU9DX48N0T (Mycophenolic Acid) RN - I9ZF7L6G2L (Nifedipine) RN - 0 (Immunosuppressive Agents) RN - 0 (Calcium Channel Blockers) RN - G6HRD2P839 (nintedanib) RN - 0 (Indoles) SB - IM MH - Humans MH - Female MH - *Scleroderma, Systemic/drug therapy/complications MH - Adult MH - *Gingival Hyperplasia/chemically induced MH - *Mycophenolic Acid/adverse effects/therapeutic use/administration & dosage MH - *Nifedipine/adverse effects/therapeutic use/administration & dosage MH - *Immunosuppressive Agents/adverse effects/therapeutic use MH - Pharmacovigilance MH - *Calcium Channel Blockers/adverse effects MH - Raynaud Disease/drug therapy MH - Lung Diseases, Interstitial/drug therapy MH - Indoles OTO - NOTNLM OT - calcium channel blockers OT - drug-induced gingival overgrowth OT - gum hyperplasia OT - nifedipine OT - systemic sclerosis COIS- Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/11/05 12:33 MHDA- 2026/02/24 06:32 CRDT- 2025/11/05 11:43 PHST- 2026/02/24 06:32 [medline] PHST- 2025/11/05 12:33 [pubmed] PHST- 2025/11/05 11:43 [entrez] AID - 10.1177/09246479251389217 [doi] PST - ppublish SO - Int J Risk Saf Med. 2026 Feb;37(1):144-147. doi: 10.1177/09246479251389217. Epub 2025 Nov 5. PMID- 38724227 OWN - NLM STAT- MEDLINE DCOM- 20250422 LR - 20260501 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 3 DP - 2025 Mar 1 TI - Arthritis in patients with very early systemic sclerosis: a comprehensive clinical and prognostic analysis. PG - 1243-1250 LID - 10.1093/rheumatology/keae247 [doi] AB - OBJECTIVE: Arthritis is associated with a worse prognosis in established SSc. However, knowledge about its relevance in very early SSc (veSSc) is scarce. We aimed to assess the prevalence and phenotype of arthritis, as well as its prognostic impact, in patients with veSSc. METHODS: We analysed patients with veSSc, defined as presence of Raynaud's phenomenon (RP) and/or at least one of: puffy fingers, antinuclear antibodies (ANA), abnormal capillaroscopy, not fulfilling the ACR/EULAR classification criteria for SSc at baseline. We investigated associations between arthritis and clinical parameters, followed by a longitudinal analysis to investigate arthritis as a potential predictor of progression towards established SSc. RESULTS: We included 159 patients, of whom 108 had at least one follow-up visit. SSc-related arthritis occurred in 22/159 (13.8%) patients at baseline. Arthritis was mostly seronegative, symmetrical, oligo- or polyarticular, non-erosive, and rarely associated with elevation of inflammatory markers. More than half of the patients needed treatment with DMARDs. Anti-centromere antibodies (ACA) were negatively associated with arthritis (odds ratio 0.707, 95% CI 0.513-0.973, P = 0.033). Overall, 43/108 (39.8%) patients with follow-up progressed to established SSc during the observation time. Arthritis was not a significant predictor for progression to established SSc in a multivariable Cox regression. CONCLUSION: In this first comprehensive analysis, we found a similar prevalence of arthritis in veSSc as seen in established SSc. Moreover, the use of DMARDs indirectly suggests a relevant disease burden. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Muraru, Sinziana AU - Muraru S AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Mihai, Carina AU - Mihai C AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Elhai, Muriel AU - Elhai M AUID- ORCID: 0000-0001-8627-5758 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Becker, Mike AU - Becker M AUID- ORCID: 0000-0001-9102-3088 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Jordan, Suzana AU - Jordan S AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Garaiman, Alexandru AU - Garaiman A AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Petelytska, Liubov AU - Petelytska L AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Dobrota, Rucsandra AU - Dobrota R AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. LA - eng GR - Iten-Kohaut Foundation/ GR - AstraZeneca/ GR - Pfizer/ GR - Iten-Kohaut foundation/ GR - Otsuka/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antirheumatic Agents) SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology MH - Female MH - Male MH - Prognosis MH - Middle Aged MH - Disease Progression MH - Antibodies, Antinuclear/blood/immunology MH - *Arthritis/epidemiology/etiology/drug therapy/diagnosis MH - Raynaud Disease/etiology MH - Adult MH - Aged MH - Longitudinal Studies MH - Prevalence MH - Antirheumatic Agents/therapeutic use MH - Microscopic Angioscopy PMC - PMC11879340 OTO - NOTNLM OT - arthritis OT - joint involvement OT - very early systemic sclerosis EDAT- 2024/05/10 00:43 MHDA- 2025/03/05 04:53 PMCR- 2024/05/09 CRDT- 2024/05/09 21:33 PHST- 2023/10/16 00:00 [received] PHST- 2024/04/18 00:00 [accepted] PHST- 2025/03/05 04:53 [medline] PHST- 2024/05/10 00:43 [pubmed] PHST- 2024/05/09 21:33 [entrez] PHST- 2024/05/09 00:00 [pmc-release] AID - 7667878 [pii] AID - keae247 [pii] AID - 10.1093/rheumatology/keae247 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Mar 1;64(3):1243-1250. doi: 10.1093/rheumatology/keae247. PMID- 20843907 OWN - NLM STAT- MEDLINE DCOM- 20110211 LR - 20260518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 11 DP - 2010 Nov TI - Discordance between patient and physician assessments of disease severity in systemic sclerosis. PG - 2307-12 LID - 10.3899/jrheum.100354 [doi] AB - OBJECTIVE: To describe the magnitude and correlates of discordance between patient and physician assessments of disease severity in patients with systemic sclerosis (SSc). METHODS: Subjects were patients enrolled in the Canadian Scleroderma Research Group Registry. The outcomes of interest were patient and physician global assessments of disease severity (scales ranging from 0-10). Predictors of disease severity represented the spectrum of disease in SSc (skin involvement, severity of Raynaud's phenomenon, shortness of breath, gastrointestinal symptoms and pain, number of fingertip ulcers, tender and swollen joints, creatinine, and fatigue). The results of the analysis were validated in an independent sample of patients with SSc from the United States. RESULTS: Patients perceived greater disease severity than physicians (mean difference 0.78 ± 2.65). The agreement between patient and physician assessments of disease severity was, at best, modest (intraclass correlation 0.3774; weighted κ 0.3771). Although both patients and physicians were influenced by skin scores, breathlessness, and pain, the relative importance of these predictors differed. Patients were also influenced by other subjective symptoms, while physicians were also influenced by disease duration and creatinine. The predictors explained 56% of the deviance in the patient global assessments and 29% in the physician assessments. These findings were confirmed in the US dataset. CONCLUSION: Patients and physicians rate SSc disease severity differently in magnitude and are influenced by different factors. Patient-assessed and physician-assessed measures of severity should be considered as complementary and used together in future studies of SSc. FAU - Hudson, Marie AU - Hudson M AD - Jewish General Hospital, Room A-725, 3755 Cote Ste Catherine Road, Montreal, Quebec H3T 1E2, Canada. marie.hudson@mcgill.ca FAU - Impens, Ann AU - Impens A FAU - Baron, Murray AU - Baron M FAU - Seibold, James R AU - Seibold JR FAU - Thombs, Brett D AU - Thombs BD FAU - Walker, Jennifer G AU - Walker JG CN - Canadian Scleroderma Research Group FAU - Steele, Russell AU - Steele R LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100915 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Canada MH - Humans MH - *Pain MH - Pain Measurement MH - Physicians MH - *Raynaud Disease MH - Registries MH - Regression Analysis MH - *Scleroderma, Systemic MH - *Severity of Illness Index MH - Surveys and Questionnaires MH - United States FIR - Baron, M IR - Baron M FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Khalidi, N IR - Khalidi N FIR - Docherty, P IR - Docherty P FIR - Kaminska, E IR - Kaminska E FIR - Masetto, A IR - Masetto A FIR - Smith, D IR - Smith D FIR - Sutton, E IR - Sutton E FIR - Mathieu, J-P IR - Mathieu JP FIR - Hudson, M IR - Hudson M FIR - Ligier, S IR - Ligier S FIR - Grodzicky, T IR - Grodzicky T FIR - Mittoo, S IR - Mittoo S FIR - Fritzler, M IR - Fritzler M EDAT- 2010/09/17 06:00 MHDA- 2011/02/12 06:00 CRDT- 2010/09/17 06:00 PHST- 2010/09/17 06:00 [entrez] PHST- 2010/09/17 06:00 [pubmed] PHST- 2011/02/12 06:00 [medline] AID - jrheum.100354 [pii] AID - 10.3899/jrheum.100354 [doi] PST - ppublish SO - J Rheumatol. 2010 Nov;37(11):2307-12. doi: 10.3899/jrheum.100354. Epub 2010 Sep 15. PMID- 33909342 OWN - NLM STAT- MEDLINE DCOM- 20211207 LR - 20211214 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 24 IP - 6 DP - 2021 Jun TI - Characteristics of Japanese patients with systemic sclerosis complicated with calcinosis. PG - 803-808 LID - 10.1111/1756-185X.14121 [doi] AB - AIM: Calcinosis is often observed in systemic sclerosis (SSc), but its pathogenesis remains unclear. The aim of the present study was to explore the association of clinical features with calcinosis in patients with SSc. METHODS: A retrospective cohort study was performed analyzing 416 SSc patients from our SSc database. We examined the clinical features with relation to calcinosis and SSc. RESULTS: Calcinosis was observed in 24.0% of patients with SSc. The group with calcinosis comprised more female patients (P < 0.05) and diffuse cutaneous types (P < 0.001) than the group without calcinosis. Complications of Raynaud's phenomenon (P < 0.05), nail fold bleeding (NFB) (P < 0.001), peripheral bone resorption (P < 0.001), myositis (P < 0.001), and pulmonary hypertension (P < 0.05) were more frequently observed in patients with calcinosis compared with those without calcinosis. The group with calcinosis had a higher modified Rodnan total skin-thickness score (mRSS) than the group without calcinosis (P < 0.001). The factors that affected calcinosis in multivariable analysis were peripheral bone resorption (partial correlation coefficient 0.46, 34%), anti-Scl-70 antibody (partial correlation coefficient 0.29, 20%), diffuse type (partial correlation coefficient 0.34, 16%) and NFB (partial correlation coefficient 0.23, 11.2%). CONCLUSIONS: Calcinosis in SSc is associated with Raynaud's phenomenon, NFB, and pulmonary hypertension, so peripheral circulatory insufficiency seems to be one of the causes of calcinosis. Furthermore, as it is related to mRSS and the diffuse cutaneous type, common factors related to skin fibrosis are considered to be involved. CI - © 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Takagi, Kae AU - Takagi K AD - Department of Medicine, Tokyo Women's Medical University Medical Center East, Tokyo, Japan. AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Kawamoto, Manabu AU - Kawamoto M AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Higuchi, Tomoaki AU - Higuchi T AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Tochimoto, Akiko AU - Tochimoto A AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Hirose, Hikaru AU - Hirose H AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Harigai, Masayoshi AU - Harigai M AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. FAU - Kawaguchi, Yasushi AU - Kawaguchi Y AUID- ORCID: 0000-0003-2025-1344 AD - Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan. LA - eng PT - Journal Article DEP - 20210428 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Adult MH - Aged MH - Calcinosis/*complications/epidemiology MH - Female MH - Humans MH - Hypertension, Pulmonary/epidemiology MH - Japan/epidemiology MH - Male MH - Middle Aged MH - Raynaud Disease/epidemiology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/epidemiology MH - Skin/pathology OTO - NOTNLM OT - autoantibody OT - calcinosis OT - skin ulcer OT - systemic sclerosis EDAT- 2021/04/29 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/04/28 12:33 PHST- 2021/03/24 00:00 [revised] PHST- 2021/04/01 00:00 [accepted] PHST- 2021/04/29 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/04/28 12:33 [entrez] AID - 10.1111/1756-185X.14121 [doi] PST - ppublish SO - Int J Rheum Dis. 2021 Jun;24(6):803-808. doi: 10.1111/1756-185X.14121. Epub 2021 Apr 28. PMID- 22864236 OWN - NLM STAT- MEDLINE DCOM- 20130408 LR - 20220408 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 21 IP - 13 DP - 2012 Nov TI - Distinct phenotypes in mixed connective tissue disease: subgroups and survival. PG - 1412-22 LID - 10.1177/0961203312456751 [doi] AB - The aim of the present study was to assess the autoantibody profile, dominant clinical symptoms and cluster characteristics of different mixed connective tissue disease (MCTD phenotypes. Two-hundred-and-one patients with MCTD were followed-up longitudinally. Five clinical parameters, Raynaud's phenomenon, pulmonary artery hypertension (PAH), myositis, interstitial lung disease (ILD), erosive arthritis and five auto-antibodies besides anti-U1RNP, antiendothelial cell antibodies (AECA), anti-CCP, anti-cardiolipin (anti-CL), anti-SSA/SSB and IgM rheumatoid factor (RF) were selected for cluster analysis. The mean age of patients was 52.9 ± 12.4 years and the mean follow-up of the disease was 12.5 ± 7.2 years. Patients were classified into three cluster groups. Cluster 1 with 77 patients, cluster 2 with 79 patients and cluster 3 with 45 patients. In cluster 1 the prevalence of PAH (55.8%; p < 0.001), Raynaud's phenomenon (92.2%; p < 0.001) and livedo reticularis (24.6%, p < 0.001) was significantly greater than in cluster 2 and 3. In cluster 2, the incidence of ILD (98.7%; p < 0.001), myositis (77.2%; p < 0.001), and esophageal dysmotility (89.8%; p < 0.001) was significantly greater than that in cluster 1 and 3. In cluster 3, anti-CCP antibodies were present in 31 of 45 patients (68.8%) with erosions. Anti-CCP antibodies were present in 37 of 42 patients (88.0%) with erosions. PAH, angina, venous thrombosis was observed in cluster 1 and pulmonary fibrosis in cluster 2, musculosceletal damage, gastrointestinal symptoms and osteoporotic fractures were most frequent in cluster 3. Cumulative survival assessment indicated cluster 1 patients having the worst prognosis. Cluster analysis is valuable to differentiate among various subsets of MCTD and useful prognostic factor regarding the disease course. FAU - Szodoray, P AU - Szodoray P AD - Institute of Immunology, Oslo University Hospital, Oslo, Norway. szodoray@gmail.com FAU - Hajas, A AU - Hajas A FAU - Kardos, L AU - Kardos L FAU - Dezso, B AU - Dezso B FAU - Soos, G AU - Soos G FAU - Zold, E AU - Zold E FAU - Vegh, J AU - Vegh J FAU - Csipo, I AU - Csipo I FAU - Nakken, B AU - Nakken B FAU - Zeher, M AU - Zeher M FAU - Szegedi, G AU - Szegedi G FAU - Bodolay, E AU - Bodolay E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120803 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Aged MH - Analysis of Variance MH - Arthritis/epidemiology MH - Autoantibodies/blood MH - Biomarkers/blood MH - Chi-Square Distribution MH - Cluster Analysis MH - Disease Progression MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hungary/epidemiology MH - Hypertension, Pulmonary/epidemiology MH - Incidence MH - Longitudinal Studies MH - Lung Diseases, Interstitial/epidemiology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/classification/diagnosis/*epidemiology/immunology/mortality MH - Myositis/epidemiology MH - Phenotype MH - Prevalence MH - Prognosis MH - Raynaud Disease/epidemiology MH - Survival Analysis MH - Time Factors EDAT- 2012/08/07 06:00 MHDA- 2013/04/09 06:00 CRDT- 2012/08/07 06:00 PHST- 2012/08/07 06:00 [entrez] PHST- 2012/08/07 06:00 [pubmed] PHST- 2013/04/09 06:00 [medline] AID - 0961203312456751 [pii] AID - 10.1177/0961203312456751 [doi] PST - ppublish SO - Lupus. 2012 Nov;21(13):1412-22. doi: 10.1177/0961203312456751. Epub 2012 Aug 3. PMID- 40440131 OWN - NLM STAT- MEDLINE DCOM- 20251003 LR - 20251119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 10 DP - 2025 Oct 1 TI - An exploratory trial of a single dose of CAM2043 (treprostinil subcutaneous depot) in SSc-related RP. PG - 5498-5503 LID - 10.1093/rheumatology/keaf291 [doi] AB - OBJECTIVES: Our aim was to explore the effect of a single subcutaneous dose of CAM2043, a novel extended-release subcutaneous formulation of treprostinil, on finger temperature in patients with SSc-related RP. METHODS: This was an exploratory, open-label, single-dose Phase 2 trial. Ten female patients (median age 54.0 years) attended on six occasions: screening, baseline (day 1), days 2, 3, 8 and 15. On day 1, patients received a single subcutaneous injection of 2.5 mg CAM2043. A standard cold challenge test of the hands was performed pre-dose and at 3, 6, 24, 72, 168 and 336 h post-dose, with temperature response over the subsequent 15 min measured by infrared thermography. The primary end point was the mean change in area under the temperature-time curve (AUCtherm) for rewarming (eight fingers) at 6 h vs baseline. RESULTS: AUCtherm increased 6 h post-dose (mean increase 192.7°C×sec [95% CI: -727.1, 1112.6]) and was significantly greater at 24 h than at baseline (mean increase 1175.8°C×sec [95% CI: 127.3, 2224.3]), returning to baseline values by day 15. Maximum temperature after rewarming increased significantly from baseline at 24 h, by 1.4°C (95% CI: 0.1, 2.7). Mean (S.D.) Raynaud's Condition Score (3.7 [1.3] units at baseline) improved significantly post-dosing, including day 8 (mean reduction 1.6 units [95% CI: -2.68, -0.52]). Adverse events were reported by all 10 patients: all reported erythema and pain at the injection site. CONCLUSION: The positive outcome indicates that CAM2043 could be further investigated in clinical trials of RP. TRIAL REGISTRATION: www.clinicaltrialsregister.eu/, EudraCT number 2019-002444-24. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Rheumatology Department, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Rheumatology Department, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - Rheumatology Department, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Manning, Joanne AU - Manning J AD - Rheumatology Department, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Chukwu, Chukwuma AU - Chukwu C AD - Clinical Research Facility, Northern Care Alliance NHS Foundation Trust, Salford, UK. FAU - Alvarez Fernandez, Marcia AU - Alvarez Fernandez M AD - Clinical Development, Camurus AB, Lund, Sweden. FAU - Axling, Ulrika AU - Axling U AD - Clinical Development, Camurus AB, Lund, Sweden. FAU - Seibold, James R AU - Seibold JR AD - Scleroderma Research Consultants LLC, Aiken, SC, USA. FAU - Tiberg, Fredrik AU - Tiberg F AUID- ORCID: 0000-0003-0080-4490 AD - Clinical Development, Camurus AB, Lund, Sweden. LA - eng GR - Camurus AB/ GR - NIHR/ GR - NIHR203308/Manchester Biomedical Research Centre/ GR - Department of Health and Social Care/ PT - Clinical Trial, Phase II PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - RUM6K67ESG (treprostinil) RN - DCR9Z582X0 (Epoprostenol) RN - 0 (Delayed-Action Preparations) RN - 0 (Antihypertensive Agents) SB - IM MH - Humans MH - Female MH - Middle Aged MH - Injections, Subcutaneous MH - Adult MH - *Epoprostenol/analogs & derivatives/administration & dosage/adverse effects/therapeutic use MH - Treatment Outcome MH - Aged MH - Delayed-Action Preparations MH - *Scleroderma, Systemic/complications/drug therapy MH - Fingers MH - *Antihypertensive Agents/administration & dosage MH - *Raynaud Disease/drug therapy/etiology MH - Skin Temperature/drug effects PMC - PMC12494207 OTO - NOTNLM OT - FluidCrystal® OT - RP OT - SSc OT - clinical trial OT - extended release OT - treprostinil EDAT- 2025/05/29 19:56 MHDA- 2025/10/03 18:28 PMCR- 2025/05/29 CRDT- 2025/05/29 12:32 PHST- 2024/12/19 00:00 [received] PHST- 2025/05/08 00:00 [accepted] PHST- 2025/10/03 18:28 [medline] PHST- 2025/05/29 19:56 [pubmed] PHST- 2025/05/29 12:32 [entrez] PHST- 2025/05/29 00:00 [pmc-release] AID - 8152875 [pii] AID - keaf291 [pii] AID - 10.1093/rheumatology/keaf291 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Oct 1;64(10):5498-5503. doi: 10.1093/rheumatology/keaf291. PMID- 22211766 OWN - NLM STAT- MEDLINE DCOM- 20140923 LR - 20140116 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 27 IP - 3 DP - 2013 Mar TI - Clinical significance of serum retinol binding protein-4 levels in patients with systemic sclerosis. PG - 337-44 LID - 10.1111/j.1468-3083.2011.04413.x [doi] AB - BACKGROUND: Retinol binding protein-4 (RBP-4) is a member of adipocytokines, which is potentially associated with fibrosis, vasodilation, and angiogenesis in addition to insulin resistance. OBJECTIVE: To investigate the clinical significance of serum RBP4 levels in patients with systemic sclerosis (SSc), which is a systemic autoimmune disease characterized by fibrosis and vasculopathy. METHODS: Serum RBP4 levels were determined by enzyme-linked immunosorbent assay in 62 SSc patients and 19 healthy controls. RESULTS: Similar to patients with chronic kidney disease, serum RBP4 levels inversely correlated with estimated glomerular filtration rate in SSc patients with renal dysfunction. Therefore, analyses were carried out by excluding SSc patients with estimated glomerular filtration rate <60 mL/min/1.73 m(2) . Serum RBP4 levels were significantly lower in diffuse cutaneous SSc (dcSSc) than in control subjects [median (25-75 percentile); 25.8 μg/mL (19.6-47.0) vs. 43.1 μg/mL (31.7-53.4), P < 0.05], while there was no significant difference between limited cutaneous SSc (lcSSc) [28.0 μg/mL (25.4-43.3)] and control subjects. In both of dcSSc and lcSSc, patients with Raynaud's phenomenon had RBP4 levels significantly lower than those without. Furthermore, serum RBP4 levels inversely correlated with pulmonary function test results in dcSSc and with right ventricular systolic pressure in lcSSc. CONCLUSION  Decreased RBP4 levels are associated with the prevalence of Raynaud's phenomenon in dcSSc and lcSSc, with the severity of interstitial lung disease in dcSSc, and with the degree of pulmonary vascular involvement in lcSSc, suggesting the possible contribution of RBP4 to the pathological events in this disorder. CI - © 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology. FAU - Toyama, T AU - Toyama T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Asano, Y AU - Asano Y FAU - Takahashi, T AU - Takahashi T FAU - Aozasa, N AU - Aozasa N FAU - Akamata, K AU - Akamata K FAU - Noda, S AU - Noda S FAU - Taniguchi, T AU - Taniguchi T FAU - Ichimura, Y AU - Ichimura Y FAU - Sumida, H AU - Sumida H FAU - Tamaki, Z AU - Tamaki Z FAU - Masui, Y AU - Masui Y FAU - Tada, Y AU - Tada Y FAU - Sugaya, M AU - Sugaya M FAU - Sato, S AU - Sato S FAU - Kadono, T AU - Kadono T LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20111226 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 RN - 0 (RBP4 protein, human) RN - 0 (Retinol-Binding Proteins, Plasma) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Cyclophosphamide/therapeutic use MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Glomerular Filtration Rate MH - Humans MH - Lung Diseases, Interstitial/blood MH - Male MH - Middle Aged MH - Raynaud Disease/blood MH - Retinol-Binding Proteins, Plasma/*metabolism MH - Scleroderma, Systemic/*blood/drug therapy/physiopathology EDAT- 2012/01/04 06:00 MHDA- 2014/09/24 06:00 CRDT- 2012/01/04 06:00 PHST- 2012/01/04 06:00 [entrez] PHST- 2012/01/04 06:00 [pubmed] PHST- 2014/09/24 06:00 [medline] AID - 10.1111/j.1468-3083.2011.04413.x [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2013 Mar;27(3):337-44. doi: 10.1111/j.1468-3083.2011.04413.x. Epub 2011 Dec 26. PMID- 38987832 OWN - NLM STAT- MEDLINE DCOM- 20240711 LR - 20260304 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 64 IP - 1 DP - 2024 Jul 10 TI - 2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis. PG - 52 LID - 10.1186/s42358-024-00392-w [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc. METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed. RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found. CONCLUSION: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice. CI - © 2024. The Author(s). FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo-UNIFESP, Rua dos Otonis 863, 2º andar, Vila Clementino, São Paulo, SP, 04025-002, Brazil. cristiane.kayser@unifesp.br. FAU - de Oliveira Delgado, Sandra Maximiano AU - de Oliveira Delgado SM AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo-UNIFESP, Rua dos Otonis 863, 2º andar, Vila Clementino, São Paulo, SP, 04025-002, Brazil. AD - Rheumatology Division, Hospital Sírio Libanês, Brasília, DF, Brazil. FAU - Zimmermann, Adriana Fontes AU - Zimmermann AF AD - Rheumatology Division, Professor Polydoro Ernani de São Tiago University Hospital, Universidade Federal de Santa Catarina-UFSC, Florianópolis, SC, Brazil. FAU - Horimoto, Alex Magno Coelho AU - Horimoto AMC AD - Rheumatology Division, Hospital Regional do Mato Grosso do Sul, Faculdade de Medicina, Universidade Federal de Mato Grosso do Sul-UFMS, Campo Grande, MS, Brazil. FAU - Del Rio, Ana Paula Toledo AU - Del Rio APT AD - Rheumatology Division, Universidade Estadual de Campinas-UNICAMP, Campinas, SP, Brazil. FAU - de Souza Müller, Carolina AU - de Souza Müller C AD - Rheumatology Division, Hospital de Clínicas, Universidade Federal do Paraná-UFPR, Curitiba, PR, Brazil. FAU - Camargo, Cintia Zumstein AU - Camargo CZ AD - Internal Medicine Departament, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista-UNESP, Botucatu, SP, Brazil. FAU - Lupo, Cristiano Michelini AU - Lupo CM AD - Rheumatology Division, Faculdade de Medicina de São José do Rio Preto-FAMERP, São José do Rio Preto, SP, Brazil. FAU - de Moraes, Daniela Aparecida AU - de Moraes DA AD - Medicine School, Centro Universitário Barão de Mauá-CBM, Ribeirão Preto, SP, Brazil. FAU - Do Rosário E Souza, Eduardo José AU - Do Rosário E Souza EJ AD - Rheumatology Division, Hospital de Santa Casa de Belo Horizonte, Belo Horizonte, MG, Brazil. FAU - Santos, Flávia Patrícia Sena Teixeira AU - Santos FPST AD - Rheumatology Division, Universidade Federal de Minas Gerais-UFMG, Belo Horizonte, MG, Brazil. FAU - Sekiyama, Juliana Yuri AU - Sekiyama JY AD - Internal Medicine Departament, Universidade Estadual de Maringá-UEM, Maringá, PR, Brazil. FAU - Lonzetti, Lilian Scussel AU - Lonzetti LS AD - Rheumatology Division, Universidade Federal de Ciências da Saúde de Porto Alegre-UFCSPA, Porto Alegre, RS, Brazil. FAU - de Oliveira Martins, Lucas Victória AU - de Oliveira Martins LV AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo-UNIFESP, Rua dos Otonis 863, 2º andar, Vila Clementino, São Paulo, SP, 04025-002, Brazil. FAU - Bezerra, Mailze Campos AU - Bezerra MC AD - Rheumatology Division, Universidade Federal do Ceará-UFC, Fortaleza, CE, Brazil. FAU - Bredemeier, Markus AU - Bredemeier M AD - Rheumatology Division, Hospital Nossa Senhora da Conceição, Porto Alegre, RS, Brazil. FAU - Oliveira, Maria Carolina AU - Oliveira MC AD - Clinical Immunology Division, Ribeirão Preto Medical School, Universidade de São Paulo-USP, Ribeirão Preto, SP, Brazil. FAU - da Fonseca Salgado, Maria Cecília AU - da Fonseca Salgado MC AD - Rheumatology Division, Universidade Federal do Estado do Rio de Janeiro-UNIRIO, Rio de Janeiro, RJ, Brazil. FAU - Miossi, Renata AU - Miossi R AD - Rheumatology Division, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo-USP, São Paulo, SP, Brazil. FAU - de Araújo Fontenele, Sheila Márcia AU - de Araújo Fontenele SM AD - Rheumatology Division, Universidade Estadual do Ceará-UECE, Fortaleza, CE, Brazil. FAU - Hax, Vanessa AU - Hax V AD - Rheumatology Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul-UFRGS, Porto Alegre, RS, Brazil. FAU - Dantas, Andrea Tavares AU - Dantas AT AD - Rheumatology Division, Universidade Federal de Pernambuco-UFPE, Recife, PE, Brazil. FAU - Sampaio-Barros, Percival Degrava AU - Sampaio-Barros PD AD - Rheumatology Division, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo-USP, São Paulo, SP, Brazil. LA - eng PT - Journal Article PT - Practice Guideline PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20240710 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 4F4X42SYQ6 (Rituximab) RN - I031V2H011 (tocilizumab) RN - 0 (Antirheumatic Agents) SB - IM MH - *Scleroderma, Systemic/complications/drug therapy MH - Humans MH - Brazil MH - *Rheumatology/standards MH - *Raynaud Disease/drug therapy MH - Societies, Medical MH - Lung Diseases, Interstitial/drug therapy MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Rituximab/therapeutic use MH - Randomized Controlled Trials as Topic MH - Skin Ulcer/etiology MH - Antirheumatic Agents/therapeutic use OTO - NOTNLM OT - Digital ulcer OT - Disease management OT - Guidelines OT - Interstitial lung disease OT - Raynaud’s phenomenon OT - Scleroderma OT - Systemic sclerosis OT - Treatment EDAT- 2024/07/11 00:42 MHDA- 2024/07/11 06:42 CRDT- 2024/07/10 23:49 PHST- 2023/09/30 00:00 [received] PHST- 2024/06/25 00:00 [accepted] PHST- 2024/07/11 06:42 [medline] PHST- 2024/07/11 00:42 [pubmed] PHST- 2024/07/10 23:49 [entrez] AID - 10.1186/s42358-024-00392-w [pii] AID - 10.1186/s42358-024-00392-w [doi] PST - epublish SO - Adv Rheumatol. 2024 Jul 10;64(1):52. doi: 10.1186/s42358-024-00392-w. PMID- 33423071 OWN - NLM STAT- MEDLINE DCOM- 20211115 LR - 20211115 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 41 IP - 3 DP - 2021 Mar TI - Characteristics and outcomes of overlap myositis: a comparative multigroup cohort study in adults from the MyoCite cohort. PG - 551-563 LID - 10.1007/s00296-020-04779-y [doi] AB - Overlap myositis (OM), an important subset of idiopathic inflammatory myopathies (IIM), is being increasingly recognized with wider myositis-specific autoantibody (MSA) testing. We studied the differences in clinical characteristics and long-term outcomes of OM with Dermatomyositis (DM), Polymyositis (PM), anti-synthetase syndrome (ASSD), and Cancer-associated IIM (CAM). Data from the MyoCite registry (Dec2017-May2020), a prospective dataset of IIM was extracted for the clinical profile, and MSAs, immunosuppressants received, disease activity (relapses and incomplete response), and treatment-related (drugs ADRs and infections) adverse events (DRAE and TRAE) were collected and analyzed between groups. Of 214 adults (58-OM,89-DM,27-ASSD,33-PM,7-CAM), OM had a greater female preponderance (13.5:1). Raynaud's and sclerodactyly were the prime distinguishing features of OM. OM could be distinguished from PM by frequent arthritis (OR-3.2) and infrequent dysphagia (OR-0.17); DM with greater nephritis (OR-20), infrequent dysphagia (OR-0.24) and rashes (OR-0.02); and ASSD by infrequent ILD (OR-0.07), and mechanic's hand (OR-0.05). 50% fulfilled the classification criteria for ASSD in the absence of MSA testing. ANA was positive more often (PM/DM: OR-6.7) and anti-Ro52 (OR-4.5) frequent in OM. Baseline serum creatinine and acute phase reactants were higher. OM received lower glucocorticoids (0 mg/kg, p < 0.001). Overall, 90% and 84% of OM at 12 and 24 months, respectively, achieved remission, with similar DRAE and TRAE as other IIM subsets. OM can be misdiagnosed as ASSD in the absence of MSA testing. Raynaud's, sclerodactyly, and a positive ANA may identify OM and prevent overtreatment. FAU - Naveen, R AU - Naveen R AUID- ORCID: 0000-0003-2014-3925 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Rathore, Upendra AU - Rathore U AUID- ORCID: 0000-0003-1658-8155 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Agarwal, Vikas AU - Agarwal V AUID- ORCID: 0000-0002-4508-1233 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Gupta, Latika AU - Gupta L AUID- ORCID: 0000-0003-2753-2990 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. drlatikagupta@gmail.com. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20210110 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Cohort Studies MH - Databases, Factual MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - India MH - Male MH - Middle Aged MH - Myositis/*complications/drug therapy/immunology/physiopathology MH - Raynaud Disease/*etiology MH - Scleroderma, Systemic/*etiology MH - Syndrome OTO - NOTNLM OT - Autoimmune diseases OT - Cohort studies OT - Connective tissue diseases OT - Myositis OT - Treatment outcome EDAT- 2021/01/11 06:00 MHDA- 2021/11/16 06:00 CRDT- 2021/01/10 20:49 PHST- 2020/11/26 00:00 [received] PHST- 2020/12/24 00:00 [accepted] PHST- 2021/01/11 06:00 [pubmed] PHST- 2021/11/16 06:00 [medline] PHST- 2021/01/10 20:49 [entrez] AID - 10.1007/s00296-020-04779-y [pii] AID - 10.1007/s00296-020-04779-y [doi] PST - ppublish SO - Rheumatol Int. 2021 Mar;41(3):551-563. doi: 10.1007/s00296-020-04779-y. Epub 2021 Jan 10. PMID- 27996340 OWN - NLM STAT- MEDLINE DCOM- 20170822 LR - 20260127 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 46 IP - 4 DP - 2017 Jul TI - Geographic variation as a risk factor for digital ulcers in systemic sclerosis patients: a multicentre registry. PG - 288-295 LID - 10.1080/03009742.2016.1233994 [doi] AB - OBJECTIVE: To evaluate the influence of geographic variation on the risk of digital ulcer (DU) development in systemic sclerosis (SSc) patients. METHODS: This cross-sectional, multicentre study evaluated patients with SSc from centres located in different geographic regions of Brazil (subtropical and tropical climate zones). Demographic and clinical data were collected. RESULTS: The study included 141 patients with SSc (26 from the subtropical and 115 from the tropical zone). In total, 43 DUs were observed in 23 (16%) of the patients. By a simple logistic regression model, the presence of DUs was associated with a higher modified Rodnan skin score, previous necrosis or amputation of the extremities, flexion contracture of the fingers, active smoking, higher avascular score on capillaroscopy, higher severity of Raynaud's phenomenon, a higher Health Assessment Questionnaire Disability Index (HAQ-DI) score, a higher visual analogue scale score for Raynaud's phenomenon and overall disease, and the subtropical climate zone. Using multiple logistic regression, the presence of DUs was significantly associated with patients living in the subtropical climate zone [odds ratio (OR) = 5.4, p = 0.002], necrosis or amputation (OR = 5.2, p = 0.011), and a higher HAQ-DI score (OR = 2.6, p = 0.021). CONCLUSION: In this multicentre study in a continental country with different climates, patients with SSc living in a subtropical climate region had a 5.4 times higher risk of developing DUs than patients living in a warmer region (tropical climate), suggesting a more severe course of peripheral vasculopathy among patients living in geographic regions with relatively cold weather. FAU - Souza, Ejr AU - Souza E AD - a Internal Medicine Service , Santa Casa Hospital , Belo Horizonte , Brazil. FAU - Muller, C S AU - Muller CS AD - b Rheumatology Division, Clinics Hospital , Federal University of Paraná , Curitiba , Brazil. FAU - Horimoto, Amc AU - Horimoto A AD - c Rheumatology Division , Federal University of Mato Grosso do Sul , Campo Grande , Brazil. FAU - Rezende, R A AU - Rezende RA AD - a Internal Medicine Service , Santa Casa Hospital , Belo Horizonte , Brazil. FAU - Guimarães, I AU - Guimarães I AD - b Rheumatology Division, Clinics Hospital , Federal University of Paraná , Curitiba , Brazil. FAU - Mariz, H A AU - Mariz HA AD - d Clinics Hospital , Federal University of Pernambuco , Recife , Brazil. FAU - Dantas, A T AU - Dantas AT AD - d Clinics Hospital , Federal University of Pernambuco , Recife , Brazil. FAU - Da Costa, I P AU - Da Costa IP AD - c Rheumatology Division , Federal University of Mato Grosso do Sul , Campo Grande , Brazil. FAU - Del-Rio, Apt AU - Del-Rio A AD - e School of Medical Sciences , State University of Campinas , Campinas , Brazil. FAU - Sekiyama, J AU - Sekiyama J AD - e School of Medical Sciences , State University of Campinas , Campinas , Brazil. FAU - Kahwage, C B AU - Kahwage CB AD - f Rheumatology Division, Paulista School of Medicine , Federal University of São Paulo , São Paulo , Brazil. FAU - Kayser, C AU - Kayser C AD - f Rheumatology Division, Paulista School of Medicine , Federal University of São Paulo , São Paulo , Brazil. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20161220 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Brazil MH - Contracture/epidemiology MH - Cross-Sectional Studies MH - Female MH - *Fingers/blood supply MH - Geography MH - Humans MH - Logistic Models MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Odds Ratio MH - Raynaud Disease/epidemiology MH - *Registries MH - Risk Factors MH - Scleroderma, Systemic/*epidemiology MH - Severity of Illness Index MH - Skin Ulcer/*epidemiology MH - Smoking/epidemiology EDAT- 2016/12/21 06:00 MHDA- 2017/08/23 06:00 CRDT- 2016/12/21 06:00 PHST- 2016/12/21 06:00 [pubmed] PHST- 2017/08/23 06:00 [medline] PHST- 2016/12/21 06:00 [entrez] AID - 10.1080/03009742.2016.1233994 [doi] PST - ppublish SO - Scand J Rheumatol. 2017 Jul;46(4):288-295. doi: 10.1080/03009742.2016.1233994. Epub 2016 Dec 20. PMID- 39923051 OWN - NLM STAT- MEDLINE DCOM- 20250208 LR - 20250512 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 23 IP - 1 DP - 2025 Feb 8 TI - An updated overview of Juvenile systemic sclerosis in a French cohort. PG - 13 LID - 10.1186/s12969-024-01043-6 [doi] LID - 13 AB - BACKGROUND: Systemic sclerosis encompasses a range of disorders characterized by vascular and connective tissue abnormalities. Although rare in pediatrics, juvenile systemic sclerosis (jSSc) is a severe and life-threatening condition that significantly impacts children's development. This study aimed to provide an overview of JSSc in France over the past decade. METHODS: Patients with disease onset before the age of 16 were included following a request for observations sent via email to member practitioners of the SOFREMIP (French pediatric Rheumatology society). RESULTS: Our study included 18 patients from 8 different French centers. While our cohort exhibited a balanced distribution between limited and diffuse subsets of the disease, we observed a higher prevalence of the diffuse subset in children above the age of 10. Skin induration was the most reported symptom, while Raynaud's phenomenon was present in 61% of the children at initial clinical evaluation. All children tested positive for antinuclear antibodies, with anti-Scl70 being the most common specificity, even among children with limited cutaneous subsets. Interestingly, we found a high sensitivity of the ACR / EULAR criteria for diagnosing jSSc in our cohort with 83% of patients meeting these criteria, except for 3 children who presented with overlap syndromes. Despite the frequent use of corticosteroids at the onset, no deaths or renal crises were reported. Three patients received treatment with biological agents, specifically Rituximab and Tocilizumab. CONCLUSION: JSSc is a rare but severe disease requiring rapid, specialized, and multidisciplinary care. Further studies are needed to validate proper diagnosis criteria including overlap syndromes and evaluate the use of biotherapies in children. CI - © 2024. The Author(s). FAU - Jacquel, Léa AU - Jacquel L AUID- ORCID: 0000-0002-5255-369X AD - Department of Clinical Immunology and Internal Medicine, University Hospital of Strasbourg, Strasbourg, France. l.jacquel2@chru-nancy.fr. AD - Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France. l.jacquel2@chru-nancy.fr. FAU - Bechara, Rouba AU - Bechara R AD - Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France. FAU - Terzic, Joëlle AU - Terzic J AD - Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France. FAU - Rameau, Anne-Cécile AU - Rameau AC AD - Department of Pediatrics, Groupe Hospitalier de la région de Mulhouse et Sud Alsace, Mulhouse, France. FAU - Chatelus, Emmanuel AU - Chatelus E AD - Department of Rheumatology, University Hospital of Strasbourg, Strasbourg, France. FAU - Rossi-Semerano, Linda AU - Rossi-Semerano L AD - Department of Paediatric Rheumatology, CEREMAIA, Hôpital de Bicêtre, APHP, Le Kremlin Bicêtre, France. FAU - Kone-Paut, Isabelle AU - Kone-Paut I AD - Department of Paediatric Rheumatology, CEREMAIA, Hôpital de Bicêtre, APHP, Le Kremlin Bicêtre, France. FAU - Meinzer, Ulrich AU - Meinzer U AD - Department of General Pediatrics, Hôpital Robert Debré, Paris, France. FAU - Lemelle, Irène AU - Lemelle I AD - Department of Pediatrics, Nancy University Hospital, Nancy, France. FAU - Rebelle, Charlotte AU - Rebelle C AD - Department of Pediatrics, Hôpital Marseille Saint Joseph, Marseille, France. FAU - Urbina, Diego AU - Urbina D AD - Department of Pediatrics, University Hospital of Marseille, Marseille, France. FAU - Pillet, Pascal AU - Pillet P AD - Department of Pediatrics, Pellegrin Hôpital des enfants, University Hospital of Bordeaux, Bordeaux, France. FAU - Choquet, Pauline AU - Choquet P AD - Department of Pediatrics, Hospital of Annecy Genevois, Annecy, France. FAU - El Maamari, Jad AU - El Maamari J AD - Division of Pediatric Hematology / Oncology, BC Children's Hospital, Vancouver, Canada. FAU - Zaloszyc, Ariane AU - Zaloszyc A AD - Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France. CN - SOFREMIP LA - eng PT - Journal Article PT - Multicenter Study DEP - 20250208 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Monoclonal, Humanized) RN - Juvenile systemic scleroderma SB - IM MH - Humans MH - France/epidemiology MH - *Scleroderma, Systemic/epidemiology/diagnosis/drug therapy MH - Child MH - Male MH - Female MH - Adolescent MH - Child, Preschool MH - Cohort Studies MH - Antibodies, Antinuclear/blood MH - Raynaud Disease/etiology MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Prevalence PMC - PMC11807294 OTO - NOTNLM OT - Autoimmune disease OT - Biological therapy OT - Juvenile systemic sclerosis OT - Scleroderma OT - Systemic COIS- Declarations. Ethics approval and consent to participate: This study was approved by the University Hospital of Strasbourg ethics committee (CE-2022-13). Consent for publication: Not applicable. Competing interests: The authors declare that they have no competing interests. EDAT- 2025/02/09 06:20 MHDA- 2025/02/09 06:21 PMCR- 2025/02/08 CRDT- 2025/02/08 23:25 PHST- 2024/01/10 00:00 [received] PHST- 2024/12/01 00:00 [accepted] PHST- 2025/02/09 06:21 [medline] PHST- 2025/02/09 06:20 [pubmed] PHST- 2025/02/08 23:25 [entrez] PHST- 2025/02/08 00:00 [pmc-release] AID - 10.1186/s12969-024-01043-6 [pii] AID - 1043 [pii] AID - 10.1186/s12969-024-01043-6 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2025 Feb 8;23(1):13. doi: 10.1186/s12969-024-01043-6. PMID- 35285493 OWN - NLM STAT- MEDLINE DCOM- 20221201 LR - 20230109 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 12 DP - 2022 Nov 28 TI - Nailfold microvascular abnormalities are associated with a higher prevalence of pulmonary arterial hypertension in patients with MCTD. PG - 4875-4884 LID - 10.1093/rheumatology/keac165 [doi] AB - OBJECTIVE: MCTD manifests with microvasculopathy and overlapping clinical features of SLE, SSc and idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the clinical significance of microvasculopathy in patients with MCTD using nailfold videocapillaroscopy (NVC). METHODS: Fifty patients with newly diagnosed and untreated MCTD were enrolled in this multicentre, prospective and observational study. Clinical features and NVC findings were assessed at baseline and after 1 year post-intervention, along with disease controls [SLE (n = 40), SSc (n = 70) and IIM (n = 50)]. RESULTS: All MCTD patients presented Raynaud's phenomenon and were positive for anti-U1 RNP antibodies, and 22.0% (11/50) had pulmonary arterial hypertension (PAH). The prevalence of NVC scleroderma patterns in MCTD was 38.0%, which was lower than SSc (88.6%) but higher than SLE (10.0%). In addition, when we divided MCTD patients into two groups by presence or absence of NVC scleroderma patterns, we found a higher prevalence of PAH in patients with NVC scleroderma patterns. Namely, NVC scleroderma patterns were observed in all MCTD patients with PAH, and in 21.0% of those without PAH. After intensive immunosuppressive therapy, NVC scleroderma patterns disappeared in half of the MCTD patients but were not changed in SSc patients. CONCLUSIONS: MCTD differed from SLE, SSc and IIM in terms of the prevalence and responsiveness of NVC scleroderma patterns to immunosuppressive therapy. Detection of nailfold microvascular abnormalities in MCTD could contribute to predicting PAH and help us to understand further aspects of the pathogenesis of MCTD. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Todoroki, Yasuyuki AU - Todoroki Y AUID- ORCID: 0000-0001-9693-9263 AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Kubo, Satoshi AU - Kubo S AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Nakano, Kazuhisa AU - Nakano K AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Miyazaki, Yusuke AU - Miyazaki Y AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Ueno, Masanobu AU - Ueno M AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Satoh-Kanda, Yurie AU - Satoh-Kanda Y AD - Department of Internal Medicine, Kitakyushu General Hospital, Kitakyushu, Japan. FAU - Kanda, Ryuichiro AU - Kanda R AUID- ORCID: 0000-0001-8247-6595 AD - Department of Internal Medicine, Kitakyushu General Hospital, Kitakyushu, Japan. FAU - Miyagawa, Ippei AU - Miyagawa I AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Hanami, Kentaro AU - Hanami K AD - Wakamatsu Hospital of the University of Occupational and Environmental Health, Kitakyushu. FAU - Nakatsuka, Keisuke AU - Nakatsuka K AD - Department of Internal Medicine, Fukuoka Yutaka Central Hospital, Fukuoka. FAU - Saito, Kazuyoshi AU - Saito K AD - Department of Internal Medicine, Tobata General Hospital, Kitakyushu, Japan. FAU - Nakayamada, Shingo AU - Nakayamada S AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. FAU - Tanaka, Yoshiya AU - Tanaka Y AUID- ORCID: 0000-0002-0807-7139 AD - First Department of Internal Medicine, University of Occupational and Environmental Health, Japan. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2022 Nov 28;61(12):e381-e382. doi: 10.1093/rheumatology/keac291. PMID: 35552624 MH - Humans MH - *Pulmonary Arterial Hypertension MH - *Mixed Connective Tissue Disease MH - Prospective Studies MH - Prevalence MH - Microscopic Angioscopy MH - Familial Primary Pulmonary Hypertension MH - *Raynaud Disease/epidemiology MH - *Myositis/epidemiology MH - *Lupus Erythematosus, Systemic MH - *Scleroderma, Systemic/complications/epidemiology OTO - NOTNLM OT - MCTD OT - RP OT - SSc OT - nailfold videocapillaroscopy OT - pulmonary arterial hypertension EDAT- 2022/03/15 06:00 MHDA- 2022/12/02 06:00 CRDT- 2022/03/14 08:42 PHST- 2021/12/10 00:00 [received] PHST- 2022/03/04 00:00 [accepted] PHST- 2022/03/15 06:00 [pubmed] PHST- 2022/12/02 06:00 [medline] PHST- 2022/03/14 08:42 [entrez] AID - 6548159 [pii] AID - 10.1093/rheumatology/keac165 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Nov 28;61(12):4875-4884. doi: 10.1093/rheumatology/keac165. PMID- 26292963 OWN - NLM STAT- MEDLINE DCOM- 20160520 LR - 20201209 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 17 IP - 1 DP - 2015 Aug 21 TI - Plexin-D1/Semaphorin 3E pathway may contribute to dysregulation of vascular tone control and defective angiogenesis in systemic sclerosis. PG - 221 LID - 10.1186/s13075-015-0749-4 [doi] LID - 221 AB - INTRODUCTION: The vascular and nervous systems have several anatomic and molecular mechanism similarities. Emerging evidence suggests that proteins involved in transmitting axonal guidance cues, including members of class III semaphorin (Sema3) family, play a critical role in blood vessel guidance during physiological and pathological vascular development. Sema3E is a natural antiangiogenic molecule that causes filopodial retraction in endothelial cells, inhibiting cell adhesion by disrupting integrin-mediated adhesive structures. The aim of the present study was to investigate whether in systemic sclerosis (SSc) Plexin-D1/Sema3E axis could be involved in the dysregulation of vascular tone control and angiogenesis. METHODS: Sema3E levels were measured by quantitative colorimetric sandwich ELISA in serum samples from 48 SSc patients, 45 subjects with primary Raynaud's phenomenon (pRP) and 48 age-matched and sex-matched healthy controls. Immunofluorescence staining on skin sections from 14 SSc patients and 12 healthy subjects was performed to evaluate Sema3E and Plexin-D1 expression. Western blotting was used to assess Plexin-D1/Sema3E axis in human SSc and healthy dermal microvascular endothelial cells (SSc-MVECs and H-MVECs, respectively) at basal condition and after stimulation with recombinant human vascular endothelial growth factor (VEGF), SSc and healthy sera. Capillary morphogenesis on Matrigel was performed on H-MVECs treated with healthy, pRP or SSc sera in the presence of Sema3E and Plexin-D1 soluble peptides. RESULTS: Serum Sema3E levels were significantly higher both in pRP subjects and SSc patients than in controls. In SSc, Sema3E levels were significantly increased in patients with early nailfold videocapillaroscopy (NVC) pattern compared to active/late patterns and pRP, and in patients without digital ulcers versus those with ulcers. In SSc skin, Sema3E expression was strongly increased in the microvascular endothelium. Cultured SSc-MVECs showed higher levels of phosphorylated Plexin-D1 and Sema3E expression than H-MVECs, and stimulation with SSc sera increased phosphorylated Plexin-D1 and Sema3E in H-MVECs. The addition of Sema3E-binding Plexin-D1 soluble peptide significantly attenuated the antiangiogenic effect of SSc sera on H-MVECs. CONCLUSIONS: Our findings suggest that Plexin-D1/Sema3E axis is triggered in SSc endothelium and may have a role in the dysregulation of angiogenesis and vascular tone control by inducing neuro-vascular mechanism alterations clinically evident in particular in the early disease phases. FAU - Mazzotta, Celestina AU - Mazzotta C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. celestina.mazzotta@unifi.it. FAU - Romano, Eloisa AU - Romano E AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. eloisaromano@libero.it. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. cosimobruni85@gmail.com. FAU - Manetti, Mirko AU - Manetti M AD - Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Largo Brambilla 3, I-50134, Florence, Italy. mirkomanetti@yahoo.it. FAU - Lepri, Gemma AU - Lepri G AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. lepri.gemma@gmail.com. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. s.bellandorandone@gmail.com. FAU - Blagojevic, Jelena AU - Blagojevic J AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. jelena308@hotmail.com. FAU - Ibba-Manneschi, Lidia AU - Ibba-Manneschi L AD - Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Largo Brambilla 3, I-50134, Florence, Italy. lidia.ibba@unifi.it. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. cerinic@unifi.it. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Viale Pieraccini 18, I-50139, Florence, Italy. s.guiducci@hotmail.com. LA - eng PT - Journal Article DEP - 20150821 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Cell Adhesion Molecules, Neuronal) RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (Membrane Glycoproteins) RN - 0 (PLXND1 protein, human) RN - 0 (SEMA3E protein, human) RN - 0 (Semaphorins) SB - IM MH - Adult MH - Aged MH - Blood Vessels/*metabolism/physiopathology MH - Blotting, Western MH - Cell Adhesion Molecules, Neuronal/*metabolism MH - Cells, Cultured MH - Endothelial Cells/metabolism MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Intracellular Signaling Peptides and Proteins MH - Male MH - Membrane Glycoproteins MH - Microscopy, Fluorescence MH - Middle Aged MH - Neovascularization, Pathologic/*metabolism/physiopathology MH - Phosphorylation MH - Raynaud Disease/metabolism/physiopathology MH - Scleroderma, Systemic/*metabolism/physiopathology MH - Semaphorins/blood/*metabolism MH - *Signal Transduction MH - Skin/blood supply/metabolism/pathology PMC - PMC4546224 EDAT- 2015/08/22 06:00 MHDA- 2016/05/21 06:00 PMCR- 2015/08/21 CRDT- 2015/08/22 06:00 PHST- 2015/02/23 00:00 [received] PHST- 2015/08/10 00:00 [accepted] PHST- 2015/08/22 06:00 [entrez] PHST- 2015/08/22 06:00 [pubmed] PHST- 2016/05/21 06:00 [medline] PHST- 2015/08/21 00:00 [pmc-release] AID - 10.1186/s13075-015-0749-4 [pii] AID - 749 [pii] AID - 10.1186/s13075-015-0749-4 [doi] PST - epublish SO - Arthritis Res Ther. 2015 Aug 21;17(1):221. doi: 10.1186/s13075-015-0749-4. PMID- 33436082 OWN - NLM STAT- MEDLINE DCOM- 20210621 LR - 20210621 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 23 IP - 1 DP - 2021 Jan 12 TI - Photoacoustic and high-frequency ultrasound imaging of systemic sclerosis patients. PG - 22 LID - 10.1186/s13075-020-02400-y [doi] LID - 22 AB - INTRODUCTION: Systemic sclerosis starts with an early phase characterized by Raynaud's phenomenon, puffy fingers/hands, autoantibodies, and a scleroderma nailfold microscopic pattern. Alterations in the nailfold microscopic pattern are not evident in all early SSc patients. Photoacoustics (PA) and high-frequency ultrasound (HFUS) could fulfill this need. The former can measure oxygen saturation while the latter can measure skin thickening. We hypothesize that photoacoustics and high-frequency ultrasound can distinguish (early) SSc patients from individuals with primary Raynaud's phenomenon (PRP) by measuring oxygenation of the fingertip and skin thickening. METHODS: We compared measurements of oxygenation and skin thickness of the third finger between (early) SSc patients and PRP individuals and healthy controls. The spearman rank correlation was used to analyze an association between capillary density and oxygen saturation of the fingers. RESULTS: Thirty-one adult subjects participated in this study: twelve patients with SSc, 5 patients with early SSc, 5 volunteers with PR, and 9 healthy controls. We found a significant difference in oxygen saturation between (early) SSc patients (80.8% ± 8.1 and 77.9% ± 10.5) and individuals with PRP (93.9% ± 1.1). Measurements of skin thickening showed a significant difference in (early) SSc patients compared to individuals with PRP (0.48 ± 0.06 mm and 0.51 ± 0.16 mm vs. 0.27 ± 0.01 mm). There was no significant difference between healthy and PRP individuals in oxygenation or skin thickening. CONCLUSION: Photoacoustic and high-frequency ultrasound could help to distinguish between (early) SSc, PRP, and healthy individuals in both oxygenation and skin thickening. FAU - Daoudi, Khalid AU - Daoudi K AD - Medical UltraSound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. FAU - Kersten, Brigit E AU - Kersten BE AD - Department of Rheumatic Diseaes Radboud University Medical Center, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. B.Kersten@radboudumc.nl. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AD - Medical UltraSound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. AD - Department of Rheumatollogy, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. FAU - van den Hoogen, Frank H J AU - van den Hoogen FHJ AD - Medical UltraSound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. AD - Department of Rheumatollogy, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AUID- ORCID: 0000-0002-2266-9907 AD - Medical UltraSound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. Madelon.Vonk@radboudumc.nl. FAU - de Korte, Chris L AU - de Korte CL AD - Department of Rheumatic Diseaes Radboud University Medical Center, Sint Maartenskliniek Post 766, PO box 9101, 6500 HB, Nijmegen, The Netherlands. LA - eng PT - Journal Article DEP - 20210112 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Adult MH - Capillaries MH - Fingers MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/diagnostic imaging MH - *Scleroderma, Systemic/diagnostic imaging MH - Ultrasonography PMC - PMC7802269 OTO - NOTNLM OT - Early diagnosis OT - High-frequency ultrasound OT - Photoacoustic OT - Skin thickening OT - Systemic sclerosis COIS- The authors declare that they have no competing interests. EDAT- 2021/01/14 06:00 MHDA- 2021/06/22 06:00 PMCR- 2021/01/12 CRDT- 2021/01/13 05:44 PHST- 2020/10/14 00:00 [received] PHST- 2020/12/14 00:00 [accepted] PHST- 2021/01/13 05:44 [entrez] PHST- 2021/01/14 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2021/01/12 00:00 [pmc-release] AID - 10.1186/s13075-020-02400-y [pii] AID - 2400 [pii] AID - 10.1186/s13075-020-02400-y [doi] PST - epublish SO - Arthritis Res Ther. 2021 Jan 12;23(1):22. doi: 10.1186/s13075-020-02400-y. PMID- 18991099 OWN - NLM STAT- MEDLINE DCOM- 20090127 LR - 20181201 IS - 1532-4311 (Electronic) IS - 0882-0139 (Linking) VI - 37 IP - 8 DP - 2008 TI - Endothelin-1 does not change the function of monocyte-derived dendritic cells grown from patients with systemic sclerosis. PG - 841-8 LID - 10.1080/08820130802438016 [doi] AB - Systemic sclerosis (SSc) is characterized by both vasculopathy and autoimmunity. The interplay between these pathogenetic links requires further exploration. The aim was to assess the interrelationship of endothelin-1 (ET-1), a vasoconstrictor peptide, whose levels are usually elevated in the plasma of the patients with SSc and the function of monocyte-derived dendritic cells (MDDCs), which serve as organizers of the immune response. MDDCs were grown from 5 patients with SSc and severe Raynaud's phenomenon and 5 healthy volunteers. The cells were further stimulated by synthetic ET-1, lipopolysaccharide (LPS) or both. The production of endogenous ET-1, TNFalpha and IL-12 was assessed by RT-PCR and/or ELISA. The plasma levels of ET-1 were significantly higher in patients with SSc compared to healthy controls (p = 0.0005). The production of ET-1 by MDDCs was negligible in all examined conditions, while the release of TNFalpha and IL-12 was stimulated by LPS but not by ET-1. The in vitro concentration of the exogenous ET-1, where added, was comparable to the plasma levels of ET-1 in patients with SSc. High plasma levels of ET-1 are characteristic for the patients with SSc and severe Raynaud's phenomenon. An in vitro model with concentrations of ET-1 comparable to those in the plasma of SSc patients has been elaborated. The examined function of MDDCs from SSc patients and healthy volunteers did not differ under these conditions and was not dependent on the presence of ET-1. FAU - Slobodin, Gleb AU - Slobodin G AD - Department of Internal Medicine, Bnai Zion Medical Center and Ruth & Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. gslobodin@yahoo.com FAU - Pavlotzky, Elsa AU - Pavlotzky E FAU - Panov, Julia AU - Panov J FAU - Rosner, Itzhak AU - Rosner I FAU - Kessel, Aharon AU - Kessel A FAU - Toubi, Elias AU - Toubi E LA - eng PT - Journal Article PL - England TA - Immunol Invest JT - Immunological investigations JID - 8504629 RN - 0 (Endothelin-1) RN - 0 (Peptides) RN - 0 (Tumor Necrosis Factor-alpha) RN - 187348-17-0 (Interleukin-12) SB - IM MH - Adult MH - Dendritic Cells/*immunology/*metabolism/pathology MH - Endothelin-1/chemistry/*immunology/*metabolism MH - Female MH - Humans MH - Interleukin-12/metabolism MH - Male MH - Middle Aged MH - Monocytes/*pathology MH - Peptides/chemistry/*metabolism MH - Raynaud Disease/immunology MH - Scleroderma, Systemic/*immunology MH - Tumor Necrosis Factor-alpha/metabolism EDAT- 2008/11/11 09:00 MHDA- 2009/01/28 09:00 CRDT- 2008/11/11 09:00 PHST- 2008/11/11 09:00 [pubmed] PHST- 2009/01/28 09:00 [medline] PHST- 2008/11/11 09:00 [entrez] AID - 905235381 [pii] AID - 10.1080/08820130802438016 [doi] PST - ppublish SO - Immunol Invest. 2008;37(8):841-8. doi: 10.1080/08820130802438016. PMID- 23812620 OWN - NLM STAT- MEDLINE DCOM- 20140609 LR - 20260128 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 32 IP - 11 DP - 2013 Nov TI - Decreased interleukin-20 level in patients with systemic sclerosis: are they related with angiogenesis? PG - 1599-603 LID - 10.1007/s10067-013-2317-0 [doi] AB - In this study, we aimed to evaluate the relation between angiogenesis indicators and T helper 17 cytokine group in patients with systemic sclerosis (SSc) which is a disease characterized by impaired angiogenesis and autoimmune response. In our study, patients with SSc are compared with patients with primary Raynaud's phenomenon (RP) and healthy controls. Forty SSc patients, 18 primary RP cases, and 20 healthy controls were included in our study. The demographic and clinical features of patients with SSc were recorded. The serum levels of vascular endothelial growth factor (VEGF), vascular endothelial (VE)-cadherin, interleukin (IL)-20, IL-22, and IL-23 were assessed. In the SSc group, IL-20 level was significantly lower than in both primary RP group and controls (p values <0.001). VE-cadherin level in SSc was significantly higher than in primary RP (p = 0.016). The IL-22 and IL-23 and VEGF levels of SSc, primary RP, and control groups were similar (p values >0.05). In SSc patients, IL-23 correlated negatively with VEGF (r = -0.36, p = 0.025) and positively with VE-cadherin (r = 0.55, p < 0.001). IL-20 levels in SSc patients correlated with disease duration (r = 0.32, p = 0.044). SSc patients with limited involvement had significantly higher VE-cadherin levels than SSc patients with diffuse involvement (p = 0.044). We observed that IL-20 which is an IL-10 group angiogenesis indicator was observed to be suppressed in SSc, suggesting abnormal angiogenesis. FAU - Aydoğdu, Erkan AU - Aydoğdu E AD - Department of Rheumatology, Trakya University Medical Faculty, Edirne, Turkey. FAU - Pamuk, Ömer Nuri AU - Pamuk ÖN FAU - Dönmez, Salim AU - Dönmez S FAU - Pamuk, Gülsüm Emel AU - Pamuk GE LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130629 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antigens, CD) RN - 0 (Cadherins) RN - 0 (Interleukin-23) RN - 0 (Interleukins) RN - 0 (Vascular Endothelial Growth Factor A) RN - 0 (Interleukin-22) RN - 0 (Cadherin 5) RN - U91R7IMG8U (interleukin 20) SB - IM MH - Adult MH - Aged MH - Antigens, CD/blood MH - Cadherins/blood MH - Female MH - Humans MH - Interleukin-23/blood MH - Interleukins/*blood MH - Male MH - Middle Aged MH - Neovascularization, Pathologic/*blood MH - Raynaud Disease/*blood MH - Scleroderma, Systemic/*blood MH - Vascular Endothelial Growth Factor A/blood MH - Interleukin-22 MH - Cadherin 5 EDAT- 2013/07/03 06:00 MHDA- 2014/06/10 06:00 CRDT- 2013/07/02 06:00 PHST- 2012/11/25 00:00 [received] PHST- 2013/06/09 00:00 [accepted] PHST- 2013/02/17 00:00 [revised] PHST- 2013/07/02 06:00 [entrez] PHST- 2013/07/03 06:00 [pubmed] PHST- 2014/06/10 06:00 [medline] AID - 10.1007/s10067-013-2317-0 [doi] PST - ppublish SO - Clin Rheumatol. 2013 Nov;32(11):1599-603. doi: 10.1007/s10067-013-2317-0. Epub 2013 Jun 29. PMID- 30798274 OWN - NLM STAT- MEDLINE DCOM- 20190605 LR - 20210222 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 12 IP - 2 DP - 2019 Feb 22 TI - Rare presentation of granulomatosis with polyangiitis. LID - 10.1136/bcr-2018-227218 [doi] LID - e227218 AB - A 62-year-old man with no pertinent medical history presented with lower extremity weakness and worsening distal fingertips and toe cyanosis/gangrene. In the outpatient setting, he was initially being treated for Raynaud's phenomenon with a calcium channel blocker. On presentation, the patient had elevated inflammatory markers and white blood cell count. Serum vasculitis panel (proteinase-3 antibody) supported the diagnosis of granulomatosis with polyangiitis. His hospital course was complicated by ischaemic stroke and a diagnosis of mononeuritis multiplex in his lower extremities. After initiating therapy with intravenous steroid and rituximab, his symptoms overall improved including cyanotic fingertips/toes. His inflammatory markers and leucocytosis also improved. Outpatient follow-up consisted of further rituximab infusions and unrelated umbilical hernia incarceration which required surgery. He was found incidentally to have subsegmental pulmonary emboli which most likely occurred during the initial presentation prior to his diagnosis. The patient moved out of state and was lost to follow-up. CI - © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Hines, Adam AU - Hines A AD - Department of Internal Medicine, New York-Presbyterian/Queens, Flushing, New York, USA. FAU - Bello, Vered AU - Bello V AD - Department of Internal Medicine, New York-Presbyterian/Queens, Flushing, New York, USA. FAU - Iftikhar, Asma AU - Iftikhar A AD - Department of Pulmonary and Critical Care, New York-Presbyterian/Queens, Flushing, New York, USA. FAU - Zein, Hadi AU - Zein H AD - Department of Internal Medicine, New York-Presbyterian/Queens, Flushing, New York, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20190222 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Diagnosis, Differential MH - Fingers/*pathology MH - Gangrene/drug therapy/etiology/*pathology MH - Granulomatosis with Polyangiitis/complications/*diagnosis/drug therapy/physiopathology MH - Humans MH - Immunologic Factors/*therapeutic use MH - Male MH - Methylprednisolone/*therapeutic use MH - Middle Aged MH - Raynaud Disease MH - Rituximab/*therapeutic use MH - Stroke/*etiology MH - Toes/*pathology MH - Treatment Outcome PMC - PMC6441313 OTO - NOTNLM OT - rheumatology OT - vasculitis COIS- Competing interests: None declared. EDAT- 2019/02/25 06:00 MHDA- 2019/06/06 06:00 PMCR- 2021/02/22 CRDT- 2019/02/25 06:00 PHST- 2019/02/25 06:00 [entrez] PHST- 2019/02/25 06:00 [pubmed] PHST- 2019/06/06 06:00 [medline] PHST- 2021/02/22 00:00 [pmc-release] AID - 12/2/e227218 [pii] AID - bcr-2018-227218 [pii] AID - 10.1136/bcr-2018-227218 [doi] PST - epublish SO - BMJ Case Rep. 2019 Feb 22;12(2):e227218. doi: 10.1136/bcr-2018-227218. PMID- 28807722 OWN - NLM STAT- MEDLINE DCOM- 20180517 LR - 20181202 IS - 1873-4111 (Electronic) IS - 0378-5122 (Linking) VI - 107 DP - 2018 Jan TI - Coronary microvascular dysfunction is not associated with a history of reproductive risk factors in women with angina pectoris-An iPOWER substudy. PG - 110-115 LID - S0378-5122(17)30585-6 [pii] LID - 10.1016/j.maturitas.2017.07.004 [doi] AB - BACKGROUND: Reproductive risk factors such as preeclampsia and recurrent miscarriages have been associated with adverse cardiovascular (CV) events. Underlying coronary microvascular dysfunction (CMD) may be a common denominator. PURPOSE: We investigated whether a history of reproductive risk factors was associated with CMD in women with angina pectoris and no obstructive coronary artery disease (CAD). METHODS: Participants from the iPOWER study, including women with angina pectoris and no obstructive CAD (<50% stenosis), were invited to complete an electronic survey regarding reproductive risk factors: recurrent miscarriages, gestational diabetes, preeclampsia, rhesus immunity, polycystic ovary syndrome and menopausal status as well as migraine and Raynaud phenomenon. CMD was assessed by transthoracic Doppler echocardiography with measurement of coronary flow velocity reserve (CFVR) during high-dose dipyridamole infusion, and analyzed in three categories with cut-off points at 2.0 and 2.5. Associations between CFVR and a history of reproductive risk factors were examined by age-adjusted trend test. RESULTS: The questionnaire was completed by 613 women (73% of those invited), of whom 550 had a successful CFVR measurement. There was no significant difference in baseline characteristics between participants and non-participants. Median (interquartile range (IQR)) age was 62.8 (54.8; 68.7) years, median (IQR) BMI 26.2 (23.2; 29.8) kg/m(2), and 81.5% were postmenopausal. We did not find any significant associations between any of the reproductive risk factors, Raynaud's phenomenon or migraine and CFVR. CONCLUSION: The lack of association between coronary microvascular function and a history of reproductive risk factors, migraine and Raynaud's phenomenon suggests that a common vascular pathophysiological mechanism underlying these conditions is unlikely. CI - Copyright © 2017 Elsevier B.V. All rights reserved. FAU - Suhrs, Hannah Elena AU - Suhrs HE AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. Electronic address: Hannah.elena.suhrs@regionh.dk. FAU - Kristensen, Anna Meta AU - Kristensen AM AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. FAU - Rask, Anna Bay AU - Rask AB AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. FAU - Michelsen, Marie Mide AU - Michelsen MM AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. FAU - Frestad, Daria AU - Frestad D AD - Department of Cardiology, Hvidovre Hospital, Kettegård Alle 30, 2650 Hvidovre, Denmark. FAU - Mygind, Naja Dam AU - Mygind ND AD - Department of Cardiology, Rigshospitalet, Blegdamsvej 9, 2100 København Ø, Denmark. FAU - Bové, Kira AU - Bové K AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. FAU - Prescott, Eva AU - Prescott E AD - Department of Cardiology, Bispebjerg University Hospital, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark. LA - eng PT - Journal Article DEP - 20170812 PL - Ireland TA - Maturitas JT - Maturitas JID - 7807333 SB - IM MH - Abortion, Habitual/epidemiology MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Angina Pectoris/*epidemiology MH - *Coronary Circulation MH - Coronary Vessels/physiology MH - Diabetes, Gestational/epidemiology MH - Echocardiography, Doppler MH - Female MH - Humans MH - *Microcirculation MH - Middle Aged MH - Migraine Disorders/epidemiology MH - Polycystic Ovary Syndrome/epidemiology MH - Pre-Eclampsia/epidemiology MH - Pregnancy MH - Raynaud Disease/epidemiology MH - Risk Factors MH - Young Adult OTO - NOTNLM OT - Angina OT - Coronary flow velocity reserve OT - Coronary microvascular dysfunction OT - Gestational diabetes mellitus OT - Menopause OT - Microvascular angina OT - Polycystic ovary syndrome OT - Preeclampsia EDAT- 2017/08/16 06:00 MHDA- 2018/05/18 06:00 CRDT- 2017/08/16 06:00 PHST- 2017/05/08 00:00 [received] PHST- 2017/06/28 00:00 [revised] PHST- 2017/07/12 00:00 [accepted] PHST- 2017/08/16 06:00 [pubmed] PHST- 2018/05/18 06:00 [medline] PHST- 2017/08/16 06:00 [entrez] AID - S0378-5122(17)30585-6 [pii] AID - 10.1016/j.maturitas.2017.07.004 [doi] PST - ppublish SO - Maturitas. 2018 Jan;107:110-115. doi: 10.1016/j.maturitas.2017.07.004. Epub 2017 Aug 12. PMID- 27706206 OWN - NLM STAT- MEDLINE DCOM- 20170615 LR - 20220331 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 11 IP - 10 DP - 2016 TI - Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study. PG - e0163894 LID - 10.1371/journal.pone.0163894 [doi] LID - e0163894 AB - OBJECTIVE: Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. METHODS: In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. RESULTS: Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide<80% predicted (65%), DU (34%), cardiac involvement (32%), FVC<80% predicted (31%), increased PAPsys>40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. CONCLUSION: In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to 'widen' the still very narrow 'window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately. FAU - Jaeger, Veronika K AU - Jaeger VK AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. FAU - Wirz, Elina G AU - Wirz EG AUID- ORCID: 0000-0002-7201-2188 AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. AD - Department of Dermatology, University Hospital Basel, Basel, Switzerland. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology A, Cochin Hospital, Paris Descartes University, Paris, France. FAU - Rossbach, Philipp AU - Rossbach P AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology, University Clinic Schleswig-Holstein, Lübeck, Germany. FAU - Hachulla, Eric AU - Hachulla E AD - Service de Médecine Interne, Hôpital Huriez, Université de Lille, Lille, France. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Airò, Paolo AU - Airò P AD - UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Balbir Gurman, Alexandra AU - Balbir Gurman A AD - B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Institute of Technology, Haifa, Israel. FAU - Tikly, Mohammed AU - Tikly M AUID- ORCID: 0000-0001-7850-3538 AD - Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa. FAU - Vettori, Serena AU - Vettori S AD - Rheumatology Department, Second University of Naples, Naples, Italy. FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Justus-Liebig University Giessen, Bad Nauheim, Germany. FAU - Distler, Jörg H W AU - Distler JH AD - Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. FAU - Li, Mangtao AU - Li M AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China. FAU - Walker, Ulrich A AU - Walker UA AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. CN - EUSTAR co-authors LA - eng PT - Journal Article PT - Multicenter Study DEP - 20161005 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Autoantibodies MH - Female MH - Gastrointestinal Diseases/*epidemiology MH - Heart Diseases/*epidemiology MH - Humans MH - Incidence MH - Kaplan-Meier Estimate MH - Kidney Diseases/*epidemiology MH - Longitudinal Studies MH - Lung Diseases/*epidemiology/physiopathology MH - Male MH - Middle Aged MH - Raynaud Disease/*complications/physiopathology MH - Regression Analysis MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications/physiopathology MH - Skin Diseases/*epidemiology PMC - PMC5051961 COIS- The authors have declared that no competing interests exist. EDAT- 2016/10/06 06:00 MHDA- 2017/06/16 06:00 PMCR- 2016/10/05 CRDT- 2016/10/06 06:00 PHST- 2016/07/07 00:00 [received] PHST- 2016/09/18 00:00 [accepted] PHST- 2016/10/06 06:00 [entrez] PHST- 2016/10/06 06:00 [pubmed] PHST- 2017/06/16 06:00 [medline] PHST- 2016/10/05 00:00 [pmc-release] AID - PONE-D-16-24817 [pii] AID - 10.1371/journal.pone.0163894 [doi] PST - epublish SO - PLoS One. 2016 Oct 5;11(10):e0163894. doi: 10.1371/journal.pone.0163894. eCollection 2016. PMID- 38302542 OWN - NLM STAT- MEDLINE DCOM- 20240205 LR - 20240206 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 14 IP - 1 DP - 2024 Feb 1 TI - Serum biomarkers in patients with hand-arm vibration injury and in controls. PG - 2719 LID - 10.1038/s41598-024-52782-1 [doi] LID - 2719 AB - Hand-arm vibration injury is a well-known occupational disorder that affects many workers globally. The diagnosis is based mainly on quantitative psychophysical tests and medical history. Typical manifestations of hand-arm vibration injury entail episodes of finger blanching, Raynaud's phenomenon (RP) and sensorineural symptoms from affected nerve fibres and mechanoreceptors in the skin. Differences in serum levels of 17 different biomarkers between 92 patients with hand-arm vibration injury and 51 controls were analysed. Patients with hand-arm vibration injury entailing RP and sensorineural manifestations showed elevated levels of biomarkers associated with endothelial injury or dysfunction, inflammation, vaso- or neuroprotective compensatory, or apoptotic mechanisms: intercellular adhesion molecule-1 (ICAM-1), monocyte chemoattractant protein-1 (MCP-1); thrombomodulin (TM), heat shock protein 27 (HSP27); von Willebrand factor, calcitonin gene-related peptide (CGRP) and caspase-3. This study adds important knowledge on pathophysiological mechanisms that can contribute to the implementation of a more objective method for diagnosis of hand-arm vibration injury. CI - © 2024. The Author(s). FAU - Tekavec, Eva AU - Tekavec E AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. eva.tekavec@med.lu.se. FAU - Nilsson, Tohr AU - Nilsson T AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, SE-901 87, Umeå, Sweden. FAU - Dahlin, Lars B AU - Dahlin LB AD - Department of Translational Medicine-Hand Surgery, Lund University, 221 00, Lund, Sweden. FAU - Huynh, Elizabeth AU - Huynh E AD - Occupational and Environmental Medicine, Region Skåne, 223 63, Lund, Sweden. FAU - Axmon, Anna AU - Axmon A AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. FAU - Nordander, Catarina AU - Nordander C AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. FAU - Riddar, Jakob AU - Riddar J AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. FAU - Kåredal, Monica AU - Kåredal M AD - Division of Occupational and Environmental Medicine, Department of Laboratory Medicine, Lund University, 221 00, Lund, Sweden. AD - Occupational and Environmental Medicine, Region Skåne, 223 63, Lund, Sweden. LA - eng PT - Journal Article DEP - 20240201 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Biomarkers) SB - IM MH - Humans MH - Vibration MH - Hand MH - Fingers/innervation MH - *Occupational Diseases MH - *Hand Injuries MH - *Arm Injuries MH - *Raynaud Disease MH - Biomarkers PMC - PMC10834969 COIS- The authors declare no competing interests. EDAT- 2024/02/02 00:42 MHDA- 2024/02/05 06:42 PMCR- 2024/02/01 CRDT- 2024/02/01 23:40 PHST- 2023/10/24 00:00 [received] PHST- 2024/01/23 00:00 [accepted] PHST- 2024/02/05 06:42 [medline] PHST- 2024/02/02 00:42 [pubmed] PHST- 2024/02/01 23:40 [entrez] PHST- 2024/02/01 00:00 [pmc-release] AID - 10.1038/s41598-024-52782-1 [pii] AID - 52782 [pii] AID - 10.1038/s41598-024-52782-1 [doi] PST - epublish SO - Sci Rep. 2024 Feb 1;14(1):2719. doi: 10.1038/s41598-024-52782-1. PMID- 33470159 OWN - NLM STAT- MEDLINE DCOM- 20211004 LR - 20211004 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 30 IP - 4 DP - 2021 Apr TI - Ultrasound characterization of the nail bed in patients with systemic lupus erythematosus. PG - 608-614 LID - 10.1177/0961203320988609 [doi] AB - OBJECTIVE: To characterize the ultrasound findings of the nail plate and nail bed in systemic lupus erythematosus (SLE) and its association with nail dystrophy. METHODS: Thirty-two SLE patients, 36 patients with osteoarthritis (OA) and 20 healthy individuals were studied. High-frequency linear ultrasound was performed in nails of the second to fifth fingers in all participants. Disease activity (SLEDAI-2K index), accrued organ damage (SLICC/ACR index), autoantibody profile, and Raynaud's phenomenon were also assessed in SLE patients. RESULTS: Nail bed thickness in SLE patients was higher than in healthy individuals (1.25 ± 0.31 mm vs 1.17 ± 0.29 mm; P = 0.01) but lower than in OA (1.39 ± 0.37 mm; P < 0.001), while nail plate thickness was similar among groups. Nail dystrophy was found more frequently in SLE and OA than in healthy individuals. SLE patients with nail dystrophy were older than their counterparts with no dystrophy (39.4 ± 10.4 years vs 27.8 ± 5.6 years; P = 0.004), although nail dystrophy showed no association with SLICC/ACR, SLEDAI-2K, nail bed vascularity, or autoantibodies. CONCLUSIONS: Nail bed in SLE patients is thicker than in healthy individuals but thinner than in OA patients. Nail dystrophy in SLE is associated with advanced age, but not with accrued organ damage, disease activity, Raynaud's phenomenon, or DIP synovitis assessed by ultrasound. FAU - Ferrusquia-Toriz, Diana AU - Ferrusquia-Toriz D AUID- ORCID: 0000-0002-7586-3770 AD - Hospital Ángeles del Pedregal, Mexico City, Mexico. FAU - Hernández-Díaz, Cristina AU - Hernández-Díaz C AD - Musculoskeletal Ultrasound Laboratory, Instituto Nacional de Rehabilitación Luis Guillermo Ibarra Ibarra, Mexico City, Mexico. FAU - Amezcua-Guerra, Luis M AU - Amezcua-Guerra LM AUID- ORCID: 0000-0002-6258-5732 AD - Department of Immunology, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico. FAU - Ventura-Ríos, Lucio AU - Ventura-Ríos L AD - Musculoskeletal Ultrasound Laboratory, Instituto Nacional de Rehabilitación Luis Guillermo Ibarra Ibarra, Mexico City, Mexico. FAU - Higuera-Ortiz, Violeta AU - Higuera-Ortiz V AD - Department of Rheumatology, The American British Cowdray Hospital, Mexico City, Mexico. AD - Department of Rheumatology, General Hospital No. 8, Instituto Mexicano del Seguro Social, Mexico City, Mexico. FAU - Lozada-Navarro, Ana C AU - Lozada-Navarro AC AD - Internal Medicine, Hospital Faro del Mayab/Christus Muguerza, Mérida Yucatán, Mexico. FAU - Silveira, Luis H AU - Silveira LH AD - Department of Rheumatology, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico. LA - eng PT - Comparative Study PT - Journal Article PT - Observational Study DEP - 20210120 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Age Factors MH - Autoantibodies/immunology MH - Female MH - Healthy Volunteers/statistics & numerical data MH - Humans MH - Lupus Erythematosus, Systemic/*complications/diagnosis/epidemiology/pathology MH - Male MH - Middle Aged MH - Multiple Organ Failure/complications/epidemiology MH - Nail Diseases/*etiology/pathology MH - Nails/*diagnostic imaging/pathology MH - Osteoarthritis/epidemiology/pathology MH - Raynaud Disease/complications MH - Severity of Illness Index MH - Ultrasonography/*methods OTO - NOTNLM OT - Nail dystrophy OT - systemic lupus erythematosus OT - ultrasound EDAT- 2021/01/21 06:00 MHDA- 2021/10/05 06:00 CRDT- 2021/01/20 08:40 PHST- 2021/01/21 06:00 [pubmed] PHST- 2021/10/05 06:00 [medline] PHST- 2021/01/20 08:40 [entrez] AID - 10.1177/0961203320988609 [doi] PST - ppublish SO - Lupus. 2021 Apr;30(4):608-614. doi: 10.1177/0961203320988609. Epub 2021 Jan 20. PMID- 20676648 OWN - NLM STAT- MEDLINE DCOM- 20120702 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 1 DP - 2012 Jan TI - Factors and comorbidities associated with central nervous system involvement in systemic lupus erythematosus: a retrospective cross-sectional case-control study from a single center. PG - 129-35 LID - 10.1007/s00296-010-1565-4 [doi] AB - To evaluate, by a retrospective cross-sectional case-control study from a single center, the distribution of a number of factors and comorbidities potentially related to central nervous system involvement in SLE Italian patients, a number of "generic" (i.e. not strictly SLE related) and "specific" (i.e. SLE related) risk factors were checked and their distribution analyzed in SLE patients with (NPSLE) and without (SLE) neuropsychiatric (NP) involvement. One hundred and fifty-three SLE patients with NP involvement observed from 1999 to 2008 and 247 SLE patients without NP manifestations, matched for sex, age and disease duration were included in the study. A neuropsychiatric (NP) event represented the heralding symptom of the disease in 40.5% of NPSLE. Headache, cerebrovascular events, mood disorders and seizures were the most frequent NP manifestations. NPSLE patients had a major cumulative number of the investigated factors than controls without NP involvement. Antiphospholipid antibodies (aPL), lupus anticoagulant (LA), Antiphospholipid antibodies syndrome (APS), Raynaud's phenomenon, smoke, assumption of contraceptives and higher cumulative dose of glucocorticosteroids (GC) were significantly more commonly observed among NPSLE. APS and systemic arterial hypertension were more frequently detected among patients with focal NP manifestations, especially cerebrovascular events. aPL, LA, APS, Raynaud's phenomenon, smoke, contraceptives intake and higher cumulative dose of GC did prove more frequently detected in NPSLE patients than in controls. In particular, overall, arterial hypertension should be regarded as a potential independent "risk factor" for focal involvement, especially for cerebrovascular events. FAU - Padovan, Melissa AU - Padovan M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Ferrara and Sant' Anna Hospital, Azienda Ospedaliero-Universitaria, C.so Giovecca 203, 44100 Ferrara, Italy. melipadovan@yahoo.it FAU - Castellino, Gabriella AU - Castellino G FAU - Bortoluzzi, Alessandra AU - Bortoluzzi A FAU - Caniatti, Luisa AU - Caniatti L FAU - Trotta, Francesco AU - Trotta F FAU - Govoni, Marcello AU - Govoni M LA - eng PT - Journal Article DEP - 20100731 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Adult MH - Aged MH - Antibodies, Antiphospholipid/blood MH - Antiphospholipid Syndrome/epidemiology MH - Case-Control Studies MH - Central Nervous System/*physiopathology MH - Cerebrovascular Disorders/*epidemiology MH - Comorbidity MH - Cross-Sectional Studies MH - Female MH - Humans MH - Lupus Coagulation Inhibitor/blood MH - Lupus Erythematosus, Systemic/*epidemiology/immunology/physiopathology MH - Lupus Vasculitis, Central Nervous System/*epidemiology/immunology/physiopathology MH - Male MH - Middle Aged MH - Mood Disorders/*epidemiology MH - Prevalence MH - Raynaud Disease/epidemiology MH - Retrospective Studies MH - Risk Factors MH - Seizures/*epidemiology EDAT- 2010/08/03 06:00 MHDA- 2012/07/03 06:00 CRDT- 2010/08/03 06:00 PHST- 2010/03/20 00:00 [received] PHST- 2010/07/11 00:00 [accepted] PHST- 2010/08/03 06:00 [entrez] PHST- 2010/08/03 06:00 [pubmed] PHST- 2012/07/03 06:00 [medline] AID - 10.1007/s00296-010-1565-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Jan;32(1):129-35. doi: 10.1007/s00296-010-1565-4. Epub 2010 Jul 31. PMID- 31858338 OWN - NLM STAT- MEDLINE DCOM- 20210122 LR - 20240607 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 39 IP - 4 DP - 2020 Apr TI - Vascular biomarkers and digital ulcerations in systemic sclerosis: results from a randomized controlled trial of oral treprostinil (DISTOL-1). PG - 1199-1205 LID - 10.1007/s10067-019-04863-0 [doi] AB - INTRODUCTION: Although there is abundant evidence of vascular perturbation from studies of peripheral blood in systemic sclerosis (SSc), there are few data about the ability to use biomarkers of vascular injury and growth factors to predict vascular outcomes and response to therapy. We sought to explore the association between candidate vascular biomarkers and digital ulcerations (DU) in a clinical trial context. METHODS: We examined 19 circulating vascular, angiogenic, and inflammatory biomarkers in 124 patients with scleroderma and DU who participated in a randomized controlled trial of oral treprostinil diolamine (ClinicalTrials.gov identifier NCT00775463). Correlation, regression, and random forest analyses were conducted to assess biomarker relationships in response to drug treatment. RESULTS: Over the 20-week trial, 82 (66%) patients had their cardinal ulcer completely heal, 54 (44%) developed new ulcers, and 72 (58%) had complete healing of all ulcers; mean change in ulcer burden comparing week 20 with baseline was - 0.36 ± 1.70. Nineteen biomarkers were analyzed for their association and ability to predict clinical DU outcomes. After adjusting for multiple comparisons, no individual biomarker (baseline level, week 20 level, or change over time) was significantly associated with any of the clinical outcomes, suggesting that traditional vascular, angiogenic or inflammatory drivers are not predictive of ulcer fate. CONCLUSIONS: The lack of strong response to any of the vascular, angiogenic, or inflammatory markers suggest that these pathways are not primary drivers in the development of DU clinical outcomes in a SSc population with prevalent DU. KEY POINTS: • Currently we lack robust biomarkers to predict vascular outcomes or response to therapy in scleroderma patients with Raynaud's phenomenon.• Longitudinal assessment of vascular biomarkers in a clinical trial setting provides a unique opportunity to define biomarkers that predict vascular outcomes.• In a randomized controlled trial of oral treprostinil diolamine for treatment of scleroderma-associated digital ulcers, biomarkers involved in several vascular, inflammatory, and angiogenic pathways did not predict short-term clinical response to therapy or digital ulcer outcomes.• Further study of these and other biomarkers should be considered in Raynaud's clinical trials in scleroderma patients without prevalent digital ulcers. FAU - Mecoli, Christopher A AU - Mecoli CA AUID- ORCID: 0000-0002-1139-2009 AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Perin, Jamie AU - Perin J AD - Johns Hopkins Bloomberg School of Public Health, 615 N. Wolfe Street, Room E3650, Baltimore, MD, 21205, USA. FAU - Van Eyk, Jennifer E AU - Van Eyk JE AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. AD - Cedars-Sinai Medical Center, 127 S. San Vicente Blvd., Advanced Health Sciences Pavilion, 9th Floor, Los Angeles, CA, 90048, USA. FAU - Zhu, Jie AU - Zhu J AD - Cedars-Sinai Medical Center, 127 S. San Vicente Blvd., Advanced Health Sciences Pavilion, 9th Floor, Los Angeles, CA, 90048, USA. FAU - Fu, Qin AU - Fu Q AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. AD - Cedars-Sinai Medical Center, 127 S. San Vicente Blvd., Advanced Health Sciences Pavilion, 9th Floor, Los Angeles, CA, 90048, USA. FAU - Allmon, Andrew G AU - Allmon AG AD - United Therapeutics, 1040 Spring Street, Silver Spring, MD, 20910, USA. FAU - Rao, Youlan AU - Rao Y AD - United Therapeutics, 1040 Spring Street, Silver Spring, MD, 20910, USA. FAU - Zeger, Scott AU - Zeger S AD - Johns Hopkins Bloomberg School of Public Health, 615 N. Wolfe Street, Room E3650, Baltimore, MD, 21205, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. Ami.Shah@jhmi.edu. LA - eng SI - ClinicalTrials.gov/NCT00775463 GR - K23 AR061439/AR/NIAMS NIH HHS/United States GR - R01 AR073208/AR/NIAMS NIH HHS/United States GR - R01 HL111362/HL/NHLBI NIH HHS/United States GR - 1R01 AR073208-01/AR/NIAMS NIH HHS/United States PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial DEP - 20191219 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Biomarkers) RN - DCR9Z582X0 (Epoprostenol) RN - RUM6K67ESG (treprostinil) SB - IM MH - Administration, Oral MH - Adult MH - Biomarkers MH - Epoprostenol/*analogs & derivatives/therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Regression Analysis MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology PMC - PMC8211019 MID - NIHMS1710482 OTO - NOTNLM OT - Biomarkers OT - Digital OT - Ischemic OT - Scleroderma OT - Ulcers COIS- Competing Interests: Two authors (AGA and YR) are employees of United Therapeutics as denoted above. The authors otherwise declare that they have no competing interests. EDAT- 2019/12/21 06:00 MHDA- 2021/01/23 06:00 PMCR- 2021/06/17 CRDT- 2019/12/21 06:00 PHST- 2019/05/09 00:00 [received] PHST- 2019/11/20 00:00 [accepted] PHST- 2019/11/01 00:00 [revised] PHST- 2019/12/21 06:00 [pubmed] PHST- 2021/01/23 06:00 [medline] PHST- 2019/12/21 06:00 [entrez] PHST- 2021/06/17 00:00 [pmc-release] AID - 10.1007/s10067-019-04863-0 [pii] AID - 10.1007/s10067-019-04863-0 [doi] PST - ppublish SO - Clin Rheumatol. 2020 Apr;39(4):1199-1205. doi: 10.1007/s10067-019-04863-0. Epub 2019 Dec 19. PMID- 41117362 OWN - NLM STAT- MEDLINE DCOM- 20260120 LR - 20260122 IS - 1365-2362 (Electronic) IS - 0014-2972 (Print) IS - 0014-2972 (Linking) VI - 56 IP - 1 DP - 2026 Jan TI - A matter arising: When should inflammatory and autoimmune rheumatic diseases be considered 'early'? PG - e70136 LID - 10.1111/eci.70136 [doi] LID - e70136 AB - BACKGROUND: Early diagnosis is pivotal for guiding the intensity of clinical monitoring, optimizing therapeutic strategies and preventing organ damage in inflammatory and autoimmune rheumatic diseases (IARDs). This review summarizes current evidence on early diagnostic and therapeutic approaches of some IARDs, including rheumatoid arthritis (RA), systemic sclerosis (SSc) and detection of large-vessel vasculitis (LVV) in polymyalgia rheumatica (PMR), representing distinct pathophysiological mechanisms of joint synovitis, tissue fibrosis and vasculitis, respectively. METHODS: A comprehensive narrative literature review was conducted focusing on early recognition strategies, searching PubMed and Scopus databases with emphasis on studies from the past 5 years and recent EULAR/ACR conference abstracts (2023-2025). RESULTS: In RA, clinically suspect arthralgia with seropositivity for rheumatoid factor and anti-citrullinated peptide antibodies significantly increases progression risk to definite RA. Musculoskeletal ultrasound detects subclinical synovitis in 44%-51% of high-risk individuals, while MRI identifies bone marrow edema predicting erosive progression. Abatacept significantly reduces RA development in seropositive individuals at high risk of RA. In SSc, Raynaud's phenomenon combined with SSc-specific autoantibodies and abnormal nailfold capillaroscopy predicts progression to definite disease, with 79.5% developing SSc within 4.6 years. LeRoy's criteria, validated by Koenig, enables early identification, though evidence for disease-modifying interventions in preclinical stages remains limited. For PMR, imaging reveals subclinical LVV in 16%-23% of patients without cranial symptoms. Subclinical LVV associates with higher relapse rates in retrospective studies, though optimal management approaches require prospective validation. CONCLUSIONS: Advances in early IARD recognition through refined clinical criteria, enhanced biomarkers and imaging enable risk stratification and personalized management. While intervention strategies show promise, particularly in RA, optimal patient selection and treatment protocols require further research. CI - © 2025 The Author(s). European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation. FAU - Hysa, Elvis AU - Hysa E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - Department of Experimental Medicine (DIMES), University of Genova, Genoa, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - IRCSS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - IRCSS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - IRCSS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - IRCSS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. FAU - Campitiello, Rosanna AU - Campitiello R AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - Department of Experimental Medicine (DIMES), University of Genova, Genoa, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine (DiMI), University of Genova, Genoa, Italy. AD - IRCSS Ospedale Policlinico San Martino, Genoa, Italy. LA - eng PT - Journal Article PT - Review DEP - 20251021 PL - England TA - Eur J Clin Invest JT - European journal of clinical investigation JID - 0245331 RN - 0 (Anti-Citrullinated Protein Antibodies) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Humans MH - Early Diagnosis MH - *Scleroderma, Systemic/diagnosis MH - *Arthritis, Rheumatoid/diagnosis/drug therapy MH - Synovitis/diagnosis/diagnostic imaging MH - *Rheumatic Diseases/diagnosis MH - *Polymyalgia Rheumatica/diagnosis MH - Vasculitis/diagnosis MH - Raynaud Disease/diagnosis MH - Disease Progression MH - *Autoimmune Diseases/diagnosis MH - Magnetic Resonance Imaging MH - Microscopic Angioscopy MH - Ultrasonography MH - Anti-Citrullinated Protein Antibodies MH - Rheumatoid Factor PMC - PMC12817248 OTO - NOTNLM OT - autoimmune rheumatic diseases OT - biomarkers OT - early diagnosis OT - imaging OT - inflammation COIS- The authors declare no competing interests. EDAT- 2025/10/21 12:35 MHDA- 2026/01/20 14:03 PMCR- 2026/01/20 CRDT- 2025/10/21 08:13 PHST- 2025/07/24 00:00 [received] PHST- 2025/09/23 00:00 [accepted] PHST- 2026/01/20 14:03 [medline] PHST- 2025/10/21 12:35 [pubmed] PHST- 2025/10/21 08:13 [entrez] PHST- 2026/01/20 00:00 [pmc-release] AID - ECI70136 [pii] AID - 10.1111/eci.70136 [doi] PST - ppublish SO - Eur J Clin Invest. 2026 Jan;56(1):e70136. doi: 10.1111/eci.70136. Epub 2025 Oct 21. PMID- 18626457 OWN - NLM STAT- MEDLINE DCOM- 20081205 LR - 20161020 IS - 1022-4742 (Print) IS - 1022-4742 (Linking) VI - 17 IP - 2 DP - 2008 Jul TI - Unusual presentation of progressive systemic sclerosis. PG - 192-6 AB - A female 38 years old, housewife, presented to the Department of Dermatology and Venereology, Mymensingh Medical College Hospital (MMCH) on 08.04.07 with the complaints of i) pain and reduced movement of hand, knee, shoulder and neck joints for 1 year and 9 months ii) tightness of skin over face, neck, limbs and trunk for 1 year and 6 months iii) patchy depigmentation over same areas for 1 year and 3 months iv) deformity of hands with flexion contractures for 6 months and v) dysphagia to solid food for 3 months. She had no complaints of Raynaud's phenomenon. On general examination, she was ill looking, anemic and nutritionally poor. Examination of integumentary system showed smooth, shiny, thick, hard and hidebound skin with pigmentary alteration of 'salt and pepper' appearance over fingers, hands, limbs, face, neck and trunk. Hands appear claw like but more on the right side than the left and there were no other obvious changes suggestive of digital ischaemia (atrophy, ulceration, scarring, gangrene etc). Face has got suggestive features of scleroderma. Examination of the respiratory system showed restriction of chest movement and reduced expansibility of chest wall. No other abnormality was found on examination of other systems. Laboratory investigations showed histopathology typical of scleroderma. X-ray of hands and feet showed suggestive changes, lung function test-showed restrictive lung disease. Barium swallow x-ray of esophagus in supine position showed mild dilation of lower oesophagus. But serology was non-reactive (negative ANA, Negative RA test, Negative VDRL). So, she was diagnosed as a case of progressive systemic sclerosis (PSS) with some atypicality. FAU - Chowdhury, S U AU - Chowdhury SU AD - Department of Dermatology and Venereology, Mymensingh Medical College & Hospital, Mymensingh, Bangladesh. FAU - Miah, M A AU - Miah MA FAU - Mahmud, M I AU - Mahmud MI FAU - Talukder, S I AU - Talukder SI FAU - Islam, M N AU - Islam MN FAU - Islam, N AU - Islam N LA - eng PT - Case Reports PT - Journal Article PL - Bangladesh TA - Mymensingh Med J JT - Mymensingh medical journal : MMJ JID - 9601799 SB - IM MH - Adult MH - Disease Progression MH - Female MH - Humans MH - Pain/complications/*diagnosis/physiopathology MH - Raynaud Disease MH - Scleroderma, Diffuse/complications/*diagnosis/physiopathology EDAT- 2008/07/16 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] PST - ppublish SO - Mymensingh Med J. 2008 Jul;17(2):192-6. PMID- 28993902 OWN - NLM STAT- MEDLINE DCOM- 20180914 LR - 20181113 IS - 1591-9528 (Electronic) IS - 1591-8890 (Linking) VI - 18 IP - 2 DP - 2018 May TI - Cardiac involvement in undifferentiated connective tissue disease at risk for systemic sclerosis (otherwise referred to as very early-early systemic sclerosis): a TDI study. PG - 237-243 LID - 10.1007/s10238-017-0477-y [doi] AB - Undifferentiated connective tissue disease at risk for systemic sclerosis (UCTD-risk-SSc), otherwise referred to as very early-early SSc, is a condition characterized by Raynaud's phenomenon with serum SSc marker autoantibodies and/or typical capillaroscopic findings and unsatisfying classification criteria for the disease. The aim of the present study was to assess the prevalence of right (RV) or left ventricular (LV) systolic and/or diastolic dysfunction by standard echocardiography and tissue Doppler imaging (TDI). Thirty patients with UCTD-risk-SSc (28 female, mean age 47 ± 13 years, range 21-70) and 30 age- and sex-matched controls underwent cardiac assessment by standard echocardiography and TDI. UCTD-risk-SSc patients and controls did not show any difference at standard echocardiography. Despite results falling within the respective normal ranges, TDI pointed out a mild impairment of LV and RV diastolic (E (m) 15 ± 4 vs. 19 ± 5, p = 0.0004; E/E (m) 6.1 ± 1.7 vs. 4.8 ± 1.2, p = 0.001; E (t) 14 ± 3 vs. 16 ± 2, p = 0.02; E (t)/A (t) 0.9 ± 0.4 vs. 1.3 ± 0.3, p = 0.002; E/E (t) 3.5 ± 1.2 vs. 4.2 ± 0.9, p = 0.02) and systolic function (S (m) 13 ± 3 vs. 15 ± 2 cm/s, p < 0.0003; S (t) 14 ± 2 vs. 16 ± 3 cm/s, p < 0.0001) and increased estimated pulmonary artery wedge pressure (9 ± 2 vs. 8 ± 1, p = 0.001) in UCTD-risk-SSc patients as compared to controls. Notably, a statistically significant difference also emerged in the prevalence of TDI detected E'/A'(t), (71% of UCTD-risk-SSc patients vs. 19% of controls; p < 0.0001). Our study shows that UCTD-risk-SSc patients show a previously unrecognized, mild biventricular systolic and diastolic dysfunction as compared to controls. The pathophysiologic meaning as well the predictive value of developing overt SSc await to be elucidated. FAU - D'Alto, Michele AU - D'Alto M AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Riccardi, Antonella AU - Riccardi A AD - Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Argiento, Paola AU - Argiento P AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Di Stefano, Ilaria AU - Di Stefano I AD - Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Romeo, Emanuele AU - Romeo E AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Iacono, Agostino Mattera AU - Iacono AM AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - D'Andrea, Antonello AU - D'Andrea A AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Fasano, Serena AU - Fasano S AD - Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Sanduzzi, Alessandro AU - Sanduzzi A AD - Department of Clinical Medicine and Surgery, Medical School, University "Federico II", Naples, Italy. FAU - Bocchino, Marialuisa AU - Bocchino M AD - Department of Clinical Medicine and Surgery, Medical School, University "Federico II", Naples, Italy. FAU - Docimo, Ludovico AU - Docimo L AD - Division of General and Bariatric Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Tolone, Salvatore AU - Tolone S AD - Division of General and Bariatric Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Russo, Maria Giovanna AU - Russo MG AD - Department of Cardiology, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Valentini, Gabriele AU - Valentini G AUID- ORCID: 0000-0002-7852-9137 AD - Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. gabriele.valentini@unicampania.it. LA - eng PT - Journal Article DEP - 20171009 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Echocardiography, Doppler MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*complications/physiopathology MH - Scleroderma, Systemic/*complications/physiopathology MH - Undifferentiated Connective Tissue Diseases/*complications/physiopathology MH - Ventricular Dysfunction/*diagnostic imaging/physiopathology MH - Young Adult OTO - NOTNLM OT - Cardiac involvement in SSc OT - Heart in UCTD-risk-SSc OT - Undifferentiated connective tissue disease at risk for systemic sclerosis OT - Very early–early systemic sclerosis EDAT- 2017/10/11 06:00 MHDA- 2018/09/15 06:00 CRDT- 2017/10/11 06:00 PHST- 2017/08/02 00:00 [received] PHST- 2017/09/27 00:00 [accepted] PHST- 2017/10/11 06:00 [pubmed] PHST- 2018/09/15 06:00 [medline] PHST- 2017/10/11 06:00 [entrez] AID - 10.1007/s10238-017-0477-y [pii] AID - 10.1007/s10238-017-0477-y [doi] PST - ppublish SO - Clin Exp Med. 2018 May;18(2):237-243. doi: 10.1007/s10238-017-0477-y. Epub 2017 Oct 9. PMID- 34323684 OWN - NLM STAT- MEDLINE DCOM- 20210802 LR - 20220407 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 39 Suppl 131 IP - 4 DP - 2021 Jul-Aug TI - Evaluation of endothelial dysfunction and clinical events in patients with early-stage vasculopathy in limited systemic sclerosis. PG - 57-65 LID - 10.55563/clinexprheumatol/243mpp [doi] AB - OBJECTIVES: Limited cutaneous systemic sclerosis (lcSSc) is characterised by vasculopathy contributing to vascular apoptosis, structural and functional changes. The aim of this study was to investigate parameters of endothelial dysfunction and their association to clinical events in lcSSc patients with early-stage vasculopathy. METHODS: Patients with lcSSc and early-stage vasculopathy defined as absent pre-existing pulmonary arterial hypertension (PAH), digital ulcers, and symptomatic cardiovascular diseases were recruited together with age-, race- and sex-matched controls with primary Raynaud's phenomenon. All subjects underwent measurements of flow-mediated (FMD) and nitroglycerine-mediated dilation (NMD), pulse-wave analysis, and biochemical analysis, including arginine, homoarginine, citrulline, ornithine, asymmetric dimethylarginine (ADMA), symmetric dimethylarginine (SDMA), and endothelial microparticles (EMP). Clinical events, including EUSTAR index, sicca symptoms, microvascular, skin, renal, gastrointestinal, and pulmonary involvement, were recorded by medical history, physical examination, laboratory parameters, disease-specific questionnaire, electrocardiogram, diagnostic imaging and spirometry. RESULTS: 38 patients with lcSSc and 38 controls were included after screening for eligibility. There was no difference in FMD (p=0.775), NMD (p=0.303), aortic pulse-wave velocity (p=0.662) or in augmentation index (p=0.600) between patients with lcSSc and controls. Higher values of ADMA (p=0.030), SDMA (p=0.025) and borderline significantly higher values for CD31+/CD42b- EMP (p=0.062) were observed in lcSSc patients, also with positive correlations between those parameters. ADMA, SDMA and CD31+/CD42b- were correlated with subclinical PAH, nephropathy and capillary changes. CONCLUSIONS: Selected parameters of endothelial dysfunction contribute to clinical events in lcSSc patients with early-stage vasculopathy and endothelial dysfunction seems to be primarily present in microvasculature, while its impact on macrovascular changes in lcSSc is still indistinct. FAU - Jud, Philipp AU - Jud P AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Austria. philipp.jud@medunigraz.at. FAU - Meinitzer, Andreas AU - Meinitzer A AD - Clinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University of Graz, Austria. FAU - Strohmaier, Heimo AU - Strohmaier H AD - Department Centre of Medical Research, Medical University of Graz, Austria. FAU - Schwantzer, Gerold AU - Schwantzer G AD - Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Austria. FAU - Foris, Vasile AU - Foris V AD - Division of Pulmonology, Department of Internal Medicine, Ludwig Boltzmann Institute for Lung Vascular Research and Medical University of Graz, Austria. FAU - Kovacs, Gabor AU - Kovacs G AD - Division of Pulmonology, Department of Internal Medicine, Ludwig Boltzmann Institute for Lung Vascular Research and Medical University of Graz, Austria. FAU - Avian, Alexander AU - Avian A AD - Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Austria. FAU - Odler, Balazs AU - Odler B AD - Division of Nephrology, Department of Internal Medicine, Medical University of Graz, Austria. FAU - Moazedi-Fürst, Florentine AU - Moazedi-Fürst F AD - Division of Rheumatology, Department of Internal Medicine, Medical University of Graz, Austria. FAU - Brodmann, Marianne AU - Brodmann M AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Austria. FAU - Hafner, Franz AU - Hafner F AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Austria. LA - eng PT - Journal Article DEP - 20210728 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 94ZLA3W45F (Arginine) SB - IM MH - Arginine MH - *Cardiovascular Diseases MH - Humans MH - Pulse Wave Analysis MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Limited MH - *Scleroderma, Systemic/complications/diagnosis MH - *Vascular Diseases EDAT- 2021/07/30 06:00 MHDA- 2021/08/03 06:00 CRDT- 2021/07/29 12:20 PHST- 2021/04/04 00:00 [received] PHST- 2021/06/25 00:00 [accepted] PHST- 2021/07/29 12:20 [entrez] PHST- 2021/07/30 06:00 [pubmed] PHST- 2021/08/03 06:00 [medline] AID - 17290 [pii] AID - 10.55563/clinexprheumatol/243mpp [doi] PST - ppublish SO - Clin Exp Rheumatol. 2021 Jul-Aug;39 Suppl 131(4):57-65. doi: 10.55563/clinexprheumatol/243mpp. Epub 2021 Jul 28. PMID- 25372793 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20260128 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - Frequency and impact of disease symptoms experienced by patients with systemic sclerosis from five European countries. PG - S-88-93 AB - OBJECTIVES: Knowledge about the nature and impact of symptoms faced by patients with systemic sclerosis (SSc) is needed to identify targets for research and treatment. The aim of this study was to assess and compare the frequency and impact on everyday activities of SSc symptoms among patients from five European countries. METHODS: European patients with SSc were invited through announcements by patient associations to complete an online survey. The survey included items assessing the frequency of 40 SSc symptoms and the impact on daily activities, if present. Chi-square tests were utilised to assess the differences in frequency and impact of symptoms across countries. RESULTS: In total, 537 patients were included from France (n=111), the Netherlands (n=229), Spain (n=61), Switzerland (n=50), and the United Kingdom (n=86). Symptoms experienced by ≥ 70% of patients in all countries were fatigue, Raynaud's phenomenon, joint pain, and muscle pain. Twenty symptoms were experienced by ≥ 50% of patients in all countries. Thirty symptoms had an impact on daily activities in ≥ 50% of patients who reported that the symptom was present in all countries. There were significant differences among countries in the prevalence of 17 out of 40 symptoms. Furthermore, in 24 out of 40 symptoms significant differences in the proportion of patients reporting impact of a specific symptom on everyday activities were observed. CONCLUSIONS: European patients with SSc experience a broad range of symptoms that have an impact on everyday activities. International research initiatives should target common SSc symptoms cooperatively. Further research is needed to better understand the differences in SSc symptoms among countries. FAU - Willems, L M AU - Willems LM AD - Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands. li.willems@maartenskliniek.nl. FAU - Kwakkenbos, L AU - Kwakkenbos L FAU - Leite, C C AU - Leite CC FAU - Thombs, B D AU - Thombs BD FAU - van den Hoogen, F H J AU - van den Hoogen FH FAU - Maia, A C AU - Maia AC FAU - Vliet Vlieland, T P M AU - Vliet Vlieland TP FAU - van den Ende, C H M AU - van den Ende CH LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141103 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - *Activities of Daily Living MH - Aged MH - Arthralgia/etiology MH - Cost of Illness MH - Fatigue/etiology MH - Female MH - France MH - Humans MH - Male MH - Middle Aged MH - *Mobility Limitation MH - Myalgia/etiology MH - Netherlands MH - Raynaud Disease/etiology MH - Scleroderma, Diffuse/complications/*physiopathology MH - Scleroderma, Limited/complications/*physiopathology MH - Scleroderma, Systemic/complications/physiopathology MH - Severity of Illness Index MH - Spain MH - Surveys and Questionnaires MH - Switzerland MH - United Kingdom EDAT- 2014/11/06 06:00 MHDA- 2015/10/31 06:00 CRDT- 2014/11/06 06:00 PHST- 2014/03/28 00:00 [received] PHST- 2014/05/26 00:00 [accepted] PHST- 2014/11/06 06:00 [entrez] PHST- 2014/11/06 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 8128 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-88-93. Epub 2014 Nov 3. PMID- 32865167 OWN - NLM STAT- MEDLINE DCOM- 20200917 LR - 20200917 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 38 Suppl 125 IP - 3 DP - 2020 May-Jun TI - Consensus-based evaluation of dermatoscopy versus nailfold videocapillaroscopy in Raynaud's phenomenon linking USA and Europe: a European League against Rheumatism study group on microcirculation in rheumatic diseases project. PG - 132-136 AB - OBJECTIVES: Nailfold videocapillaroscopy (NVC) is the current gold standard for detection and quantification of capillary abnormalities in Raynaud's phenomenon (RP). The objective of this study is to evaluate the role of dermatoscopy as a further screening tool in RP. METHODS: Nailfold capillaries of RP patients were examined by a hand-held non-contact polarised dermatoscope connected to the digital camera (D1) and connected to an iPad (D2). Both dermatoscopic images were marked with an arrowhead. NVC examination was evaluated at the arrowhead. Single blinded reader performed all examinations. NVC was graded as per standard of European League against Rheumatism (EULAR) study group on microcirculation in rheumatic diseases. Consensus evaluation of dermatoscopy characteristics/grade was determined and each dermatoscopic image was given a final impression of 'normal', 'non-specific' or 'scleroderma' pattern. The final interpretation by both techniques was compared after completion of the blinded reading. RESULTS: Classification of 100 consecutive dermatoscopic images resulted in 37 (wide view) 'non-interpretable', 2 'normal', 48 'non-specific' and 13 'scleroderma' pattern with D1; 23 'non-interpretable', 4 'normal', 52 'non-specific' and 21 'scleroderma' pattern by the experts with D2; 0 non-interpretable, 4 normal, 13 non-specific and 83 'scleroderma' pattern with NVC. CONCLUSIONS: Overall, 50% of dermatoscopic images were classified as non-specific and 30% were classified as non-interpretable in RP patients. However, all images classified by dermatoscopy as "normal" or as overt "scleroderma" pattern were confirmed by concomitant NVC analysis. These findings demonstrate tenuous promise for dermatoscopy as a tool for the initial screening of nailfold capillaries in RP. Further regular work up with NVC is needed to further clarify non-interpretable and non-specific findings possibly related to non-scleroderma patterns. FAU - Radic, Mislav AU - Radic M AD - Division of Rheumatology and Clinical Immunology, Centre of excellence for Systemic Sclerosis in Croatia, University Hospital Split, Split, Croatia; University of Split School of Medicine, Split, Croatia. mislavradic@gmail.com. FAU - Snow, Marcus AU - Snow M AD - Division of Rheumatology, Department of Internal Medicine, University of Nebraska Medical Centre, Omaha, NE, USA. FAU - Frech, Tracy M AU - Frech TM AD - Division of Rheumatology, Department of Internal Medicine,University of Utah and Salt Lake Veterans Affair Medical Centre, City, UT, USA. FAU - Saketkoo, Lesley A AU - Saketkoo LA AD - Tulane University School of Medicine, New Orleans Scleroderma & Sarcoidosis Patient Care & Research Centre, UMC Comprehensive Pulmonary Hypertension Centre, New Orleans, LA, USA. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Postgraduate School of Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Polyclinic Hospital San Martino, Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, and Department of Internal Medicine, Ghent University, Belgium. LA - eng PT - Journal Article DEP - 20200826 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Capillaries MH - Consensus MH - Dermoscopy MH - Europe MH - Humans MH - Microcirculation MH - Microscopic Angioscopy MH - Nails MH - *Raynaud Disease MH - *Rheumatic Diseases MH - *Scleroderma, Systemic EDAT- 2020/09/01 06:00 MHDA- 2020/09/18 06:00 CRDT- 2020/09/01 06:00 PHST- 2019/12/11 00:00 [received] PHST- 2020/06/08 00:00 [accepted] PHST- 2020/09/01 06:00 [entrez] PHST- 2020/09/01 06:00 [pubmed] PHST- 2020/09/18 06:00 [medline] AID - 15089 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2020 May-Jun;38 Suppl 125(3):132-136. Epub 2020 Aug 26. PMID- 20012052 OWN - NLM STAT- MEDLINE DCOM- 20110520 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 31 IP - 2 DP - 2011 Feb TI - Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome. PG - 183-9 LID - 10.1007/s00296-009-1255-2 [doi] AB - Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is associated with an unfavorable prognosis. We investigated the characteristics of SLE patients with PH. The patients with a pulmonary artery systolic pressure more than 30 mmHg at rest on echocardiogram were diagnosed with PH. Echocardiography was done only in patients with clinical or radiological evidence suggesting PH. Right heart catheterization was not performed. We identified 10 SLE patients with PH between 1980 and 2000. We compared their clinical and laboratory parameters with those of 97 consecutive SLE patients without PH. Nine of the ten patients with PH were females. The mean age at the time of SLE onset was 25.2 ± 6.9 years; the mean duration of follow-up was 93.4 ± 52.8 months, and the interval between the onset of SLE and PH diagnosis was 9.0 ± 4.6 (5-21) years. Antiphospholipid antibody positivity was significantly higher in the PH group (80 vs. 36%; p < 0.05), but there was no significant difference between two groups in regard to secondary antiphospholipid syndrome. The frequency of Raynaud's phenomenon was higher in PH group (60 vs. 27%; p < 0.05). Renal involvement (80 vs. 43%; p < 0.05), neuropsychiatric involvement (40 vs. 7.2%; p < 0.005) and serositis (70 vs. 14.4%; p < 0.001) were significantly more frequent in the PH group. The mean damage score in patients with and without PH were 4.0 ± 2.4 and 0.4 ± 1.0, respectively (p < 0.001). Four patients with PH died during the follow-up. This study reveals that the presence of PH defines a subgroup of patients with a severe disease and increased mortality. Antiphospholipid antibodies and Raynaud's phenomenon may contribute to the pathogenesis of PH. We recommend that all patients with SLE, especially those positive for antiphospholipid antibodies and/or with signs of Raynaud's phenomenon should be regularly evaluated for the development of PH. FAU - Cefle, Ayse AU - Cefle A AD - Division of Rheumatology, Medical Faculty, Kocaeli University, Kocaeli, Turkey. acefle@hotmail.com FAU - Inanc, Murat AU - Inanc M FAU - Sayarlioglu, Mehmet AU - Sayarlioglu M FAU - Kamali, Sevil AU - Kamali S FAU - Gul, Ahmet AU - Gul A FAU - Ocal, Lale AU - Ocal L FAU - Aral, Orhan AU - Aral O FAU - Konice, Meral AU - Konice M LA - eng PT - Journal Article DEP - 20091211 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/*immunology MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/diagnostic imaging/etiology/*immunology/mortality MH - Kidney Diseases/epidemiology MH - Lupus Erythematosus, Systemic/complications/diagnostic imaging/*immunology/mortality MH - Male MH - Mental Disorders/epidemiology MH - Prognosis MH - Raynaud Disease/epidemiology MH - Retrospective Studies MH - Serositis/epidemiology MH - Severity of Illness Index MH - Treatment Outcome MH - Ultrasonography MH - Young Adult EDAT- 2009/12/17 06:00 MHDA- 2011/05/21 06:00 CRDT- 2009/12/17 06:00 PHST- 2009/07/16 00:00 [received] PHST- 2009/11/28 00:00 [accepted] PHST- 2009/12/17 06:00 [entrez] PHST- 2009/12/17 06:00 [pubmed] PHST- 2011/05/21 06:00 [medline] AID - 10.1007/s00296-009-1255-2 [doi] PST - ppublish SO - Rheumatol Int. 2011 Feb;31(2):183-9. doi: 10.1007/s00296-009-1255-2. Epub 2009 Dec 11. PMID- 21244767 OWN - NLM STAT- MEDLINE DCOM- 20110324 LR - 20220410 IS - 0394-6320 (Print) IS - 0394-6320 (Linking) VI - 23 IP - 4 DP - 2010 Oct-Dec TI - Bosentan treatment for Raynauds phenomenon and skin fibrosis in patients with Systemic Sclerosis and pulmonary arterial hypertension: an open-label, observational, retrospective study. PG - 1185-94 AB - Raynaud?s phenomenon (RP) and cutaneous fibrosis are the distinctive manifestations of scleroderma, in which Endothelin-1 plays a fundamental pathogenetic role. Bosentan, an Endothelin-1 receptor antagonist used for the treatment of pulmonary arterial hypertension, retards the beginning of new sclerodermic digital ulcers (DU). This open-label, observational, retrospective study verified the effect of Bosentan on RP and skin fibrosis in sclerodermic outpatients affected by pulmonary arterial hypertension without DU. Fourteen subjects (13 women, 1 man; mean age 60 ± 7.5 years; ten with limited and four with diffuse scleroderma) were observed at baseline (T0) and after four (T1), twelve (T2), twenty-four (T3) and forty-eight (T4) weeks during treatment with Bosentan. They were evaluated for daily quantity and duration of RP attacks and skin thickness (using modified Rodnan total skin score, MRSS). Videocapillaroscopic evaluation was performed at T0 and T4. Bosentan decreased significantly the number and duration of RP attacks, beginning at T2 (p<0.05). Videocapillaroscopy showed significant improvement of microcirculatory patterns at T4 (p<0.05). MRSS decreased throughout the study, reaching the statistical significance at T3 and T4 (p<0.01) in the whole cohort. The present data suggest that Bosentan is effective in stabilizing the microcirculation involvement and in improving skin fibrosis irrespective of scleroderma patterns. FAU - Giordano, N AU - Giordano N AD - Department of Internal Medicine, Endocrine-Metabolic Sciences and Biochemistry, University of Siena, Italy. giordanon@unisi.it FAU - Puccetti, L AU - Puccetti L FAU - Papakostas, P AU - Papakostas P FAU - Di Pietra, N AU - Di Pietra N FAU - Bruni, F AU - Bruni F FAU - Pasqui, A L AU - Pasqui AL FAU - Acampa, M AU - Acampa M FAU - Bocchi, V AU - Bocchi V FAU - Donati, V AU - Donati V FAU - Voglino, M AU - Voglino M FAU - Fioravanti, A AU - Fioravanti A FAU - Montella, A AU - Montella A FAU - Auteri, A AU - Auteri A FAU - Nuti, R AU - Nuti R FAU - Pastorelli, M AU - Pastorelli M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Int J Immunopathol Pharmacol JT - International journal of immunopathology and pharmacology JID - 8911335 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Aged MH - Bosentan MH - *Endothelin Receptor Antagonists MH - Familial Primary Pulmonary Hypertension MH - Female MH - Fibrosis MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy MH - Retrospective Studies MH - Scleroderma, Systemic/*drug therapy MH - Skin/*pathology MH - Sulfonamides/*therapeutic use EDAT- 2011/01/20 06:00 MHDA- 2011/03/25 06:00 CRDT- 2011/01/20 06:00 PHST- 2011/01/20 06:00 [entrez] PHST- 2011/01/20 06:00 [pubmed] PHST- 2011/03/25 06:00 [medline] AID - 22 [pii] AID - 10.1177/039463201002300422 [doi] PST - ppublish SO - Int J Immunopathol Pharmacol. 2010 Oct-Dec;23(4):1185-94. doi: 10.1177/039463201002300422. PMID- 29457381 OWN - NLM STAT- MEDLINE DCOM- 20190708 LR - 20250530 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 70 IP - 6 DP - 2018 Jun TI - A Multicenter Study of the Validity and Reliability of Responses to Hand Cold Challenge as Measured by Laser Speckle Contrast Imaging and Thermography: Outcome Measures for Systemic Sclerosis-Related Raynaud's Phenomenon. PG - 903-911 LID - 10.1002/art.40457 [doi] AB - OBJECTIVE: Reliable and objective outcome measures to facilitate clinical trials of novel treatments for systemic sclerosis (SSc)-related Raynaud's phenomenon (RP) are badly needed. Laser speckle contrast imaging (LSCI) and thermography are noninvasive measures of perfusion that have shown excellent potential. This multicenter study was undertaken to determine the reliability and validity of a hand cold challenge protocol using LSCI, standard thermography, and low-cost cell phone/mobile phone thermography (henceforth referred to as mobile thermography) in patients with SSc-related RP. METHODS: Patients with RP secondary to SSc were recruited from 6 UK tertiary care centers. The patients underwent cold challenge on 2 consecutive days. Changes in cutaneous blood flow/skin temperature at each visit were imaged simultaneously using LSCI, standard thermography, and mobile thermography. Measurements included area under the curve (AUC) for reperfusion/rewarming and maximum blood flow rate/skin temperature after rewarming (MAX). Test-retest reliability was assessed using intraclass correlation coefficients (ICCs). Estimated latent correlations (estimated from multilevel models, taking values between -1 and 1; denoted as rho values) were used to assess the convergent validity of LSCI and thermography. RESULTS: In total, 159 patients (77% with limited cutaneous SSc) were recruited (84% female, median age 63.3 years). LSCI and standard thermography both had substantial reliability, with ICCs for the reperfusion/rewarming AUC of 0.67 (95% confidence interval [95% CI] 0.54, 0.76) and 0.68 (95% CI 0.58, 0.80), respectively, and ICCs for the MAX of 0.64 (95% CI 0.52, 0.75) and 0.72 (95% CI 0.64, 0.81), respectively. Very high latent correlations were present for the AUCs of LSCI and thermography (ρ = 0.94; 95% CI 0.87, 1.00) and for the AUCs of standard and mobile thermography (ρ = 0.98; 95% CI 0.94, 1.00). CONCLUSION: This is the first multicenter study to examine the reliability and validity of cold challenge using LSCI and thermography in patients with SSc-related RP. LSCI and thermography both demonstrated good potential as outcome measures. LSCI, standard thermography, and mobile thermography had very high convergent validity. CI - © 2018 The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology. FAU - Wilkinson, Jack D AU - Wilkinson JD AD - University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Leggett, Sarah A AU - Leggett SA AUID- ORCID: 0000-0002-8992-0801 AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Marjanovic, Elizabeth J AU - Marjanovic EJ AUID- ORCID: 0000-0003-4229-031X AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Moore, Tonia L AU - Moore TL AUID- ORCID: 0000-0002-8353-976X AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Allen, John AU - Allen J AUID- ORCID: 0000-0002-7263-0533 AD - Freeman Hospital and Newcastle University, Newcastle upon Tyne, UK. FAU - Anderson, Marina E AU - Anderson ME AUID- ORCID: 0000-0001-9073-7505 AD - University of Liverpool, Liverpool, UK. FAU - Britton, Jason AU - Britton J AUID- ORCID: 0000-0002-3145-9274 AD - Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Buch, Maya H AU - Buch MH AD - NIHR Leeds Musculoskeletal Biomedical Research Unit and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - NIHR Leeds Musculoskeletal Biomedical Research Unit and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - University College London Medical School, London, UK. FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Griffiths, Bridgett AU - Griffiths B AD - Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK. FAU - Hall, Frances AU - Hall F AD - Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. FAU - Howell, Kevin AU - Howell K AUID- ORCID: 0000-0003-1810-9867 AD - University College London Medical School, London, UK. FAU - MacDonald, Audrey AU - MacDonald A AD - Freeman Hospital and Newcastle University, Newcastle upon Tyne, UK. FAU - McHugh, Neil J AU - McHugh NJ AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust, Bath, UK. FAU - Manning, Joanne B AU - Manning JB AUID- ORCID: 0000-0002-4959-8387 AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust and University of Bath, Bath, UK. FAU - Roberts, Christopher AU - Roberts C AD - University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Shipley, Jacqueline A AU - Shipley JA AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals NHS Foundation Trust, Bath, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - University of Manchester and NIHR Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Murray, Andrea K AU - Murray AK AUID- ORCID: 0000-0001-9244-2882 AD - University of Manchester, Manchester, UK, and Salford Royal Foundation, NHS Trust, Manchester Academic Health Science Centre, Salford, UK. LA - eng GR - 20656/VAC_/Versus Arthritis/United Kingdom GR - CDF-2014-07-051/DH_/Department of Health/United Kingdom GR - 20656/ARC_/Arthritis Research UK/United Kingdom GR - 20380/ARC_/Arthritis Research UK/United Kingdom PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Validation Study DEP - 20180423 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Contrast Media) SB - IM MH - Aged MH - Area Under Curve MH - Cold Temperature MH - Contrast Media MH - Diagnostic Techniques, Cardiovascular/*statistics & numerical data MH - Feasibility Studies MH - Female MH - Fingers/blood supply/diagnostic imaging MH - Hand/blood supply/diagnostic imaging MH - Humans MH - Lasers MH - Male MH - Middle Aged MH - Observer Variation MH - Outcome Assessment, Health Care/*methods MH - Raynaud Disease/*diagnostic imaging/etiology MH - Regional Blood Flow MH - Reproducibility of Results MH - Scleroderma, Systemic/*complications MH - Skin Temperature MH - Statistics, Nonparametric MH - Thermography/methods/*statistics & numerical data PMC - PMC6001804 EDAT- 2018/02/20 06:00 MHDA- 2019/07/10 06:00 PMCR- 2018/06/14 CRDT- 2018/02/20 06:00 PHST- 2017/08/04 00:00 [received] PHST- 2018/02/13 00:00 [accepted] PHST- 2018/02/20 06:00 [pubmed] PHST- 2019/07/10 06:00 [medline] PHST- 2018/02/20 06:00 [entrez] PHST- 2018/06/14 00:00 [pmc-release] AID - ART40457 [pii] AID - 10.1002/art.40457 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Jun;70(6):903-911. doi: 10.1002/art.40457. Epub 2018 Apr 23. PMID- 39216493 OWN - NLM STAT- MEDLINE DCOM- 20240925 LR - 20260518 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 6 IP - 10 DP - 2024 Oct TI - The association of outdoor temperature and self-reported Raynaud's phenomenon severity among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network Cohort study. PG - e684-e692 LID - S2665-9913(24)00189-9 [pii] LID - 10.1016/S2665-9913(24)00189-9 [doi] AB - BACKGROUND: Raynaud's phenomenon is the earliest and most common systemic sclerosis manifestation. Episodes can be triggered by cold exposure and ambient temperature changes. Small studies have found that Raynaud's phenomenon outcomes were associated with season. We aimed to map the degree that differences in ambient temperature are associated with Raynaud's phenomenon outcomes across the temperature spectrum. METHODS: People with Raynaud's phenomenon secondary to systemic sclerosis in the Scleroderma Patient-centered Intervention Network Cohort completed past-week Raynaud's phenomenon severity assessments (0-10 numerical rating scale) at enrolment and longitudinally at 3-month intervals. Mean daily temperature and feels like temperature, which incorporates wind chill and humidity, for the week before each assessment were extracted for each participant from a weather site close to the participant's recruiting centre via the Iowa Environmental Mesonet. We used linear mixed models with basis splines to flexibly model non-linear changes in Raynaud's phenomenon severity across the temperature spectrum. People with lived experience of systemic sclerosis contributed to the study design and interpretation. FINDINGS: Between April 15, 2014 and Aug 1, 2023, we included data on 20 233 Raynaud's phenomenon severity assessments from 2243 participants. 1964 (88%) of 2243 participants were women, 279 (12%) were men, and 1813 (82%) were White. Mean age was 54·8 (SD 12·7) years. The maximum predicted Raynaud's phenomenon severity score was 6·8 points (95% CI 5·6-8·1), which occurred at -25°C. Severity scores decreased minimally from -15°C to 5°C (0·05-0·21 points per 5°C difference), then decreased in larger steps between 5°C and 25°C (0·37-0·54 points per 5°C difference). The minimum predicted score was at 25°C (2·6 points [95% CI 2·5-2·7]). Scores increased at temperatures above 25°C to 3·5 points (3·0-4·1) at 35°C and 5·6 points (4·5-6·8) at 40°C. Results were similar for feels like temperature. INTERPRETATION: Raynaud's phenomenon severity is worst at very cold temperatures but also increases with very warm temperatures, presumably due to air conditioning. Clinical management and Raynaud's phenomenon intervention trial designs should consider temperature patterns. FUNDING: Scleroderma Society of Ontario, Scleroderma Canada, Sclérodermie Québec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, the Canadian Institutes of Health Research, the Arthritis Society, the Lady Davis Institute for Medical Research of the Jewish General Hospital, the Jewish General Hospital Foundation, and McGill University. CI - Copyright © 2024 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Virgili-Gervais, Gabrielle AU - Virgili-Gervais G AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Matthews, Bianca AU - Matthews B AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Nassar, Elsa-Lynn AU - Nassar EL AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada; Department of Psychiatry, McGill University, Montreal, QC, Canada. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, Netherlands; Department of IQ Health, Radboud University Medical Center, Nijmegen, Netherlands; Centre for Mindfulness, Department of Psychiatry, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK; Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. FAU - Bartlett, Susan J AU - Bartlett SJ AD - Department of Medicine, McGill University, Montreal, QC, Canada; Research Institute, McGill University Health Centre, Montreal, QC, Canada; Arthritis Research Canada, Vancouver, BC, Canada. FAU - Gietzen, Amy AU - Gietzen A AD - National Scleroderma Foundation, Tri-State Chapter, Buffalo, NY, USA. FAU - Gottesman, Karen AU - Gottesman K AD - National Scleroderma Foundation, Los Angeles, CA, USA. FAU - Guillot, Geneviève AU - Guillot G AD - Sclérodermie Québec, Longueuil, QC, Canada. FAU - Hudson, Marie AU - Hudson M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada; Department of Medicine, McGill University, Montreal, QC, Canada. FAU - Hummers, Laura K AU - Hummers LK AD - Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA. FAU - Lawrie-Jones, Amanda AU - Lawrie-Jones A AD - Scleroderma Australia, Melbourne, VIC, Australia; Scleroderma Victoria, Melbourne, VIC, Australia. FAU - Malcarne, Vanessa L AU - Malcarne VL AD - Department of Psychology, San Diego State University, San Diego, CA, USA; San Diego State University-University of California, San Diego Joint Doctoral Program in Clinical Psychology, San Diego, CA, USA. FAU - Mayes, Maureen D AU - Mayes MD AD - University of Texas McGovern School of Medicine, Houston, TX, USA. FAU - Richard, Michelle AU - Richard M AD - Scleroderma Atlantic, Halifax, NS, Canada. FAU - Sauvé, Maureen AU - Sauvé M AD - Scleroderma Society of Ontario, Hamilton, ON, Canada; Scleroderma Canada, Hamilton, ON, Canada. FAU - Wojeck, Robyn K AU - Wojeck RK AD - University of Rhode Island, Kingston, RI, USA. FAU - Mouthon, Luc AU - Mouthon L AD - Service de Médecine Interne, Centre de Référence Maladies Autoimmunes et Autoinflammatoires Systémiques Rares d'Ile de France, de l'Est et de l'Ouest, Hôpital Cochin, Paris, France; Assistance Publique Hôpitaux de Paris-Centre, Université Paris Cité, Paris, France. FAU - Benedetti, Andrea AU - Benedetti A AD - Department of Medicine, McGill University, Montreal, QC, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada; Research Institute, McGill University Health Centre, Montreal, QC, Canada; Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, QC, Canada. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada; Department of Psychiatry, McGill University, Montreal, QC, Canada; Department of Medicine, McGill University, Montreal, QC, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada; Biomedical Ethics Unit, McGill University, Montreal, QC, Canada; Department of Psychology, McGill University, Montreal, QC, Canada. Electronic address: brett.thombs@mcgill.ca. CN - Scleroderma Patient-centered Intervention Network investigators LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20240828 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 SB - IM CIN - Lancet Rheumatol. 2024 Oct;6(10):e655-e657. doi: 10.1016/S2665-9913(24)00243-1. PMID: 39216492 MH - Humans MH - *Raynaud Disease/epidemiology/etiology MH - *Scleroderma, Systemic/complications MH - Female MH - Male MH - Middle Aged MH - *Severity of Illness Index MH - *Self Report MH - Adult MH - Temperature MH - Aged MH - Cohort Studies MH - Seasons COIS- Declaration of interests JDP has received speaker honoraria or consultancy fees from Astra Zeneca, Boehringer Ingelheim, IsoMab, Janssen, Permeatus, and Sojournix Pharma. MDM has received research grants or contracts from Prometheus Biosciences, Mitsubishi Tanabe, Boehringer Ingelheim, EICOS, Corbus, and Horizon Pharma; consulting fees from Cabaletta Pharma; an honorarium from GSK Pharma; and was a member of a data safety monitoring board or advisory board with Mitsubishi Tanabe, Boehringer Ingelheim, and EICOS. All other authors declare no competing interests. FIR - Fortuné, Catherine IR - Fortuné C FIR - Adams, Claire E IR - Adams CE FIR - Henry, Richard S IR - Henry RS FIR - El-Baalbaki, Ghassan IR - El-Baalbaki G FIR - Fligelstone, Kim IR - Fligelstone K FIR - Frech, Tracy IR - Frech T FIR - Harel, Daphna IR - Harel D FIR - Hinchcliff, Monique IR - Hinchcliff M FIR - Johnson, Sindhu R IR - Johnson SR FIR - Larche, Maggie IR - Larche M FIR - Leite, Catarina IR - Leite C FIR - Nguyen, Christelle IR - Nguyen C FIR - Nielsen, Karen IR - Nielsen K FIR - Pope, Janet IR - Pope J FIR - Rannou, François IR - Rannou F FIR - Rodriguez-Reyna, Tatiana Sofía IR - Rodriguez-Reyna TS FIR - Shouffoer, Anne A IR - Shouffoer AA FIR - Suarez-Almazor, Maria E IR - Suarez-Almazor ME FIR - Agard, Christian IR - Agard C FIR - Alric, Laurent IR - Alric L FIR - André, Marc IR - André M FIR - Beaslay, Floryan IR - Beaslay F FIR - Bernstein, Elana J IR - Bernstein EJ FIR - Berthier, Sabine IR - Berthier S FIR - Bissonnette, Lyne IR - Bissonnette L FIR - Blaise, Sophie IR - Blaise S FIR - Bories, Eva IR - Bories E FIR - Bruns, Alessandra IR - Bruns A FIR - Cacciatore, Carlotta IR - Cacciatore C FIR - Carreira, Patricia IR - Carreira P FIR - Casadevall, Marion IR - Casadevall M FIR - Chaigne, Benjamin IR - Chaigne B FIR - Chung, Lorinda IR - Chung L FIR - Crichi, Benjamin IR - Crichi B FIR - Deltombe, Thylbert IR - Deltombe T FIR - Denton, Christopher IR - Denton C FIR - Desroche, Tannvir IR - Desroche T FIR - Domsic, Robyn IR - Domsic R FIR - Dunne, James V IR - Dunne JV FIR - Dunogue, Bertrand IR - Dunogue B FIR - Fare, Regina IR - Fare R FIR - Farge-Bancel, Dominique IR - Farge-Bancel D FIR - Fortin, Paul R IR - Fortin PR FIR - Gauzère, Loraine IR - Gauzère L FIR - Gerber, Anne IR - Gerber A FIR - Gordon, Jessica IR - Gordon J FIR - Granel-Rey, Brigitte IR - Granel-Rey B FIR - Guffroy, Aurélien IR - Guffroy A FIR - Gyger, Geneviève IR - Gyger G FIR - Hachulla, Erica IR - Hachulla E FIR - Hoa, Sabrina IR - Hoa S FIR - Hughes, Michael IR - Hughes M FIR - Ikic, Alena IR - Ikic A FIR - Khalidi, Nader IR - Khalidi N FIR - Lakin, Kimberly IR - Lakin K FIR - Lambert, Marc IR - Lambert M FIR - Launay, David IR - Launay D FIR - Lee, Yvonne C IR - Lee YC FIR - Legendre, Paul IR - Legendre P FIR - Maillard, Hélène IR - Maillard H FIR - Maltez, Nancy IR - Maltez N FIR - Manning, Joanne IR - Manning J FIR - Marie, Isabelle IR - Marie I FIR - Martin Lopez, Maria IR - Martin Lopez M FIR - Martin, Thierry IR - Martin T FIR - Masetto, Ariel IR - Masetto A FIR - Mekinian, Arsène IR - Mekinian A FIR - Melchor Díaz, Sheila IR - Melchor Díaz S FIR - Mourguet, Morgane IR - Mourguet M FIR - Nikpour, Mandana IR - Nikpour M FIR - Olgane, Louis IR - Olgane L FIR - Poindron, Vincent IR - Poindron V FIR - Proudman, Susanna IR - Proudman S FIR - Pugnet, Grégory IR - Pugnet G FIR - Raffray, Loïc IR - Raffray L FIR - Régent, Alexis IR - Régent A FIR - Renou, Frederic IR - Renou F FIR - Rivière, Sébastien IR - Rivière S FIR - Robinson, David IR - Robinson D FIR - Rodríguez Almazar, Esther IR - Rodríguez Almazar E FIR - Roux, Sophie IR - Roux S FIR - Smets, Perrine IR - Smets P FIR - Sobanski, Vincent IR - Sobanski V FIR - Spiera, Robert IR - Spiera R FIR - Steen, Virginia IR - Steen V FIR - Sutton, Evelyn IR - Sutton E FIR - Thorne, Carter IR - Thorne C FIR - Vagner, Damien IR - Vagner D FIR - Varga, John IR - Varga J FIR - Wilcox, Pearce IR - Wilcox P FIR - Cañedo Ayala, Mara IR - Cañedo Ayala M FIR - Cook, Vanessa IR - Cook V FIR - Dal Santo, Cassidy IR - Dal Santo C FIR - Dal Santo, Tiffany IR - Dal Santo T FIR - D'Onofrio, Monica IR - D'Onofrio M FIR - Hu, Sophie IR - Hu S FIR - Neyer, Marieke Alexandra IR - Neyer MA FIR - Provencher, Sabrina IR - Provencher S EDAT- 2024/09/01 16:17 MHDA- 2024/09/26 04:18 CRDT- 2024/08/31 18:53 PHST- 2024/03/25 00:00 [received] PHST- 2024/06/20 00:00 [revised] PHST- 2024/06/26 00:00 [accepted] PHST- 2024/09/26 04:18 [medline] PHST- 2024/09/01 16:17 [pubmed] PHST- 2024/08/31 18:53 [entrez] AID - S2665-9913(24)00189-9 [pii] AID - 10.1016/S2665-9913(24)00189-9 [doi] PST - ppublish SO - Lancet Rheumatol. 2024 Oct;6(10):e684-e692. doi: 10.1016/S2665-9913(24)00189-9. Epub 2024 Aug 28. PMID- 31927087 OWN - NLM STAT- MEDLINE DCOM- 20200319 LR - 20200319 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 19 IP - 3 DP - 2020 Mar TI - Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud's phenomenon and systemic sclerosis. PG - 102458 LID - S1568-9972(20)30001-X [pii] LID - 10.1016/j.autrev.2020.102458 [doi] AB - Capillaroscopy is a non-invasive and safe tool which allows the evaluation of the morphology of the microcirculation. Since its recent incorporation in the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis together with its assessed role to monitor disease progression, capillaroscopy became a 'mainstream' investigation for rheumatologists. Given its increasing use by a variety of physicians internationally both in daily practice to differentiate primary from secondary Raynaud's phenomenon, as well as in research context to predict disease progression and monitor treatment effects, standardisation in capillaroscopic image acquisition and analysis seems paramount. To step forward to this need, experts in the field of capillaroscopy/microcirculation provide in this very consensus paper their view on image acquisition and analysis, different capillaroscopic techniques, normal and abnormal capillaroscopic characteristics and their meaning, scoring systems and reliability of image acquisition and interpretation. CI - Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. Electronic address: vanessa.smith@ugent.be. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester, UK; NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: ariane.herrick@manchester.ac.uk. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; Division of Rheumatology, ASST G. Pini, Milan, Italy. Electronic address: francesca.ingegnoli@unimi.it. FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia. Electronic address: nemanjadamjanov@yahoo.com. FAU - De Angelis, Rossella AU - De Angelis R AD - Department of Clinical and Molecular Sciences, Rheumatology Unit, Carlo Urbani Hospital, Polytechnic University of Marche, Jesi, Ancona, Italy. Electronic address: rossella.deangelis@sanita.marche.it. FAU - Denton, Christopher P AU - Denton CP AD - Department of Rheumatology, University College London, Royal Free Hospital, London, UK. Electronic address: c.denton@medsch.ucl.ac.uk. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. Electronic address: oliver.distler@usz.ch. FAU - Espejo, Karinna AU - Espejo K AD - Universidad Peruana De Ciencias Aplicadas (UPC), Centro de Excelencia en Reumatología (CER-PERU), Lima, Peru. Electronic address: krev76@gmail.com. FAU - Foeldvari, Ivan AU - Foeldvari I AD - Centre for Paediatric and Adolescent Rheumatology, Hamburg, Germany. Electronic address: foeldvari@t-online.de. FAU - Frech, Tracy AU - Frech T AD - Department of Rheumatology, University of Utah, Salt Lake City, Utah, USA. Electronic address: tracy.frech@hsc.utah.edu. FAU - Garro, Boris AU - Garro B AD - Universidad Peruana De Ciencias Aplicadas (UPC), Centro de Excelencia en Reumatología (CER-PERU), Lima, Peru. Electronic address: bgarrob1@gmail.com. FAU - Gutierrez, Marwin AU - Gutierrez M AD - Division of Musculoskeletal and Rheumatic Disorders, Instituto Nacional de Rehabilitación, Mexico City, Mexico. Electronic address: dr.gmarwin@gmail.com. FAU - Gyger, Genevieve AU - Gyger G AD - Division of Rheumatology, Jewish General Hospital, Department of Medicine, McGill University, Montreal, Quebec, Canada. Electronic address: gengyger@hotmail.com. FAU - Hachulla, Eric AU - Hachulla E AD - Univ. Lille, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Lille, France. Electronic address: ehachulla2@yahoo.fr. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Skåne University Hospital, Lund University, Lund, Sweden. Electronic address: roger.hesselstrand@med.lu.se. FAU - Iagnocco, Annamaria AU - Iagnocco A AD - Academic Rheumatology Center, Department of Clinical and Biological Science, University of Turin, Turin, Italy. Electronic address: annamaria.iagnocco1@gmail.com. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. Electronic address: cristiane.kayser@unifesp.br. FAU - Melsens, Karin AU - Melsens K AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. Electronic address: karin.melsens@ugent.be. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University of Giessen, Campus Kerckhoff, Bad Nauheim, Germany. Electronic address: u.mueller-ladner@kerckhoff-klinik.de. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address: sabrina.paolino@unige.it. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address: carmen.pizzorni@unige.it. FAU - Radic, Mislav AU - Radic M AD - Division of Rheumatology and Clinical Immunology, University Hospital Split, Split, Croatia. Electronic address: mislavradic@gmail.com. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Rome, Italy. Electronic address: valeria.riccieri@uniroma1.it. FAU - Snow, Marcus AU - Snow M AD - Department of Internal Medicine, Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE, USA. Electronic address: msnow@unmc.edu. FAU - Stevens, Wendy AU - Stevens W AD - Department of Rheumatology, St Vincent's Hospital, Melbourne, Victoria, Australia. Electronic address: wendy.stevens@bigpond.com. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address: albertosulli@unige.it. FAU - van Laar, Jacob M AU - van Laar JM AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: j.m.vanlaar@umcutrecht.nl. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. Electronic address: madelon.vonk@radboudumc.nl. FAU - Vanhaecke, Amber AU - Vanhaecke A AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. Electronic address: amber.vanhaecke@ugent.be. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. Electronic address: mcutolo@unige.it. CN - EULAR Study Group on Microcirculation in Rheumatic Diseases and the Scleroderma Clinical Trials Consortium Group on Capillaroscopy LA - eng PT - Journal Article PT - Review DEP - 20200110 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Humans MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis EDAT- 2020/01/14 06:00 MHDA- 2020/03/20 06:00 CRDT- 2020/01/14 06:00 PHST- 2019/09/12 00:00 [received] PHST- 2019/09/16 00:00 [accepted] PHST- 2020/01/14 06:00 [pubmed] PHST- 2020/03/20 06:00 [medline] PHST- 2020/01/14 06:00 [entrez] AID - S1568-9972(20)30001-X [pii] AID - 10.1016/j.autrev.2020.102458 [doi] PST - ppublish SO - Autoimmun Rev. 2020 Mar;19(3):102458. doi: 10.1016/j.autrev.2020.102458. Epub 2020 Jan 10. PMID- 38518985 OWN - NLM STAT- MEDLINE DCOM- 20240504 LR - 20241210 IS - 2210-741X (Electronic) IS - 2210-7401 (Linking) VI - 48 IP - 5 DP - 2024 May TI - Extrahepatic conditions of primary biliary cholangitis: A systematic review and meta-analysis of prevalence and risk. PG - 102321 LID - S2210-7401(24)00042-1 [pii] LID - 10.1016/j.clinre.2024.102321 [doi] AB - BACKGROUND AND AIM: Many studies reported the prevalence of extrahepatic conditions (EHC) of primary biliary cholangitis (PBC), but the great heterogeneity existed across different studies. Therefore, we conducted the systematic review and meta-analyses to determine EHC prevalence and association with PBC. METHODS: We searched PUBMED and included observational, cross-sectional and case-controlled studies. A random or fixed effects model was used to estimate the pooled prevalence and odd ratio (OR) as appropriate. RESULTS: Of 5370 identified publications, 129 publications with 133 studies met the inclusion criteria. Sjögren's syndrome had the highest prevalence (21.4 % vs. 3 % in non-PBC individuals), followed by Raynaud's syndrome (12.3 % vs. 1 %), rheumatoid arthritis-like arthritis (5 % vs. 3 %), systemic sclerosis (3.7 % vs. 0 %) and systemic lupus erythematosus (2 % vs. 0 %). The prevalence of overall thyroid diseases (11.3 %), autoimmune thyroid diseases (9.9 %), osteoporosis (21.1 %), celiac disease (1 %) and chronic bronchitis (4.6 %) was also increased among PBC patients. CONCLUSION: This is the first exhaustive study on the old theme about EHC of PBC. Given increased prevalence of many EHCs in PBC patients, promptly recognizing these EHCs are of great importance for timely and precise diagnosis of PBC. CI - Copyright © 2024 Elsevier Masson SAS. All rights reserved. FAU - Liang, Yan AU - Liang Y AD - Department of Laboratory Medicine, Changzheng Hospital, Naval Medical University, Shanghai, PR China. Electronic address: liangyan0829@163.com. FAU - Li, Jie AU - Li J AD - Department of Laboratory Medicine, Huangyan Hospital of Wenzhou Medical University, Taizhou First People's Hospital, Taizhou, Zhejiang, PR China. FAU - Zhang, Zhiyu AU - Zhang Z AD - Health Management Center, Changzheng Hospital, Naval Medical University, Shanghai, PR China. FAU - Jiang, Tingwang AU - Jiang T AD - Key Laboratory, Department of Science and Technology, The Second People's Hospital of Changshu, The Affiliated Changshu Hospital of Xuzhou Medical University, Changshu, Jiangsu, PR China. FAU - Yang, Zaixing AU - Yang Z AD - Department of Laboratory Medicine, Huangyan Hospital of Wenzhou Medical University, Taizhou First People's Hospital, Taizhou, Zhejiang, PR China. Electronic address: yangzaixingdiyi@163.com. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20240320 PL - France TA - Clin Res Hepatol Gastroenterol JT - Clinics and research in hepatology and gastroenterology JID - 101553659 SB - IM MH - Humans MH - Prevalence MH - *Liver Cirrhosis, Biliary/epidemiology/complications MH - *Sjogren's Syndrome/epidemiology/complications MH - *Scleroderma, Systemic/epidemiology/complications MH - Raynaud Disease/epidemiology MH - Arthritis, Rheumatoid/epidemiology/complications MH - Celiac Disease/epidemiology/complications MH - Osteoporosis/epidemiology MH - Lupus Erythematosus, Systemic/complications/epidemiology MH - Thyroid Diseases/epidemiology/complications MH - Autoimmune Diseases/epidemiology/complications OTO - NOTNLM OT - Extrahepatic conditions OT - Prevalence OT - Primary biliary cholangitis COIS- Declaration of competing interest We declare that all the authors of this manuscript entitled Extrahepatic conditions of primary biliary cholangitis: a systematic review and meta-analysis of prevalence and risk have no financial and personal relationships with other people or organizations that can inappropriately influence our work or may be considered as potential competing interests in this manuscript. EDAT- 2024/03/23 05:42 MHDA- 2024/05/05 07:47 CRDT- 2024/03/22 20:28 PHST- 2024/01/16 00:00 [received] PHST- 2024/02/16 00:00 [revised] PHST- 2024/03/16 00:00 [accepted] PHST- 2024/05/05 07:47 [medline] PHST- 2024/03/23 05:42 [pubmed] PHST- 2024/03/22 20:28 [entrez] AID - S2210-7401(24)00042-1 [pii] AID - 10.1016/j.clinre.2024.102321 [doi] PST - ppublish SO - Clin Res Hepatol Gastroenterol. 2024 May;48(5):102321. doi: 10.1016/j.clinre.2024.102321. Epub 2024 Mar 20. PMID- 24482037 OWN - NLM STAT- MEDLINE DCOM- 20140701 LR - 20250728 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) IP - 1 DP - 2014 Jan 30 TI - Calcium channel blockers for primary Raynaud's phenomenon. PG - CD002069 LID - 10.1002/14651858.CD002069.pub4 [doi] AB - BACKGROUND: Calcium channel blockers are the most commonly prescribed drugs for people with primary Raynaud's phenomenon. Primary Raynaud's phenomenon is a common condition characterised by an exaggerated vasospastic response to cold or emotion: classically the digits (fingers and toes) turn white, then blue, then red. OBJECTIVES: To assess the effects of different calcium channel blockers for primary Raynaud's phenomenon as determined by attack rates, severity scores, participant-preference scores and physiological measurements. SEARCH METHODS: The Cochrane Peripheral Vascular Diseases Group Trials Search Co-ordinator (TSC) searched the Specialised Register (last searched February 2013) and the Cochrane Central Register of Controlled Trials (CENTRAL) (2013, Issue 1). In addition the TSC searched clinical trials databases. SELECTION CRITERIA: Randomised controlled trials evaluating the effects of oral calcium channel blockers for the treatment of primary Raynaud's phenomenon. DATA COLLECTION AND ANALYSIS: Three review authors independently assessed the trials for inclusion and their quality, and extracted the data. Data extraction included adverse events. We contacted trial authors for missing data. MAIN RESULTS: We included seven randomised trials with 296 participants. Although overall all the trials were classed as being at low or unclear risk of bias, the sample size of the included trials was small and there was unclear reporting of outcomes. Four trials examined nifedipine and the remainder nicardipine. Comparisons were with placebo in six trials and with both dazoxiben and placebo in one trial (only the nifedipine versus placebo data were used within this review). Treatment with oral calcium channel blockers was minimally effective in primary Raynaud's phenomenon at decreasing the frequency of attacks (standardised mean difference of 0.23; 95% confidence interval (CI) 0.08 to 0.38, P = 0.003). This translates to 1.72 (95% CI 0.60 to 2.84) fewer attacks per week on calcium channel blockers compared to placebo. One trial provided details on duration of attacks reporting no statistically significant difference between the nicardipine and placebo groups (no P value reported). Only two trials provided any detail of statistical comparisons of (unvalidated) severity scores between treatment groups: one of these trials (60 participants) reported a mean severity score of 1.55 on placebo and 1.36 on nicardipine, difference 0.2 (95% CI of difference 0 to 0.4, no P value reported) and the other trial (three participants only with primary Raynaud's phenomenon) reported a median severity score of 2 on both nicardipine and placebo treatment (P > 0.999). Participant-preference scores were included in four trials, but in only two were results specific to participants with primary Raynaud's phenomenon, and scoring systems differed between trials: scores differed between treatments in only one trial, in which 33% of participants on placebo and 73% on nifedipine reported improvement in symptoms (P < 0.001). Physiological measurements were included as outcome measures in five trials (different methodologies were used in each): in none of these trials were any statistically significant between-treatment group differences found. Treatment with calcium channel blockers appeared to be associated with a number of adverse reactions, including headaches, flushing and oedema (swelling). AUTHORS' CONCLUSIONS: The randomised controlled trials included in this review provide moderate-quality evidence that oral calcium channel blockers are minimally effective in the treatment of primary Raynaud's phenomenon as measured by the frequency of attacks. However, the results of this review were limited by small sample sizes in the included studies and by variable data quality, particularly with regard to outcome measures. FAU - Ennis, Holly AU - Ennis H AD - Centre for Musculoskeletal Research, Institute of Inflammation and Repair, University of Manchester, Manchester Academic Health Science Centre, Stopford Building, Oxford Road, Manchester, UK, M13 9PT. FAU - Anderson, Marina E AU - Anderson ME FAU - Wilkinson, Jack AU - Wilkinson J FAU - Herrick, Ariane L AU - Herrick AL LA - eng GR - CZB/4/788/CSO_/Chief Scientist Office/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20140130 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Calcium Channel Blockers) RN - CZ5312222S (Nicardipine) RN - I9ZF7L6G2L (Nifedipine) SB - IM UIN - Cochrane Database Syst Rev. 2016 Feb 25;2:CD002069. doi: 10.1002/14651858.CD002069.pub5. PMID: 26914257 MH - Calcium Channel Blockers/*therapeutic use MH - Humans MH - Nicardipine/therapeutic use MH - Nifedipine/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy EDAT- 2014/02/01 06:00 MHDA- 2014/07/02 06:00 CRDT- 2014/02/01 06:00 PHST- 2014/02/01 06:00 [entrez] PHST- 2014/02/01 06:00 [pubmed] PHST- 2014/07/02 06:00 [medline] AID - 10.1002/14651858.CD002069.pub4 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2014 Jan 30;(1):CD002069. doi: 10.1002/14651858.CD002069.pub4. PMID- 36604358 OWN - NLM STAT- MEDLINE DCOM- 20230417 LR - 20230417 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 42 IP - 5 DP - 2023 May TI - Diagnostic accuracy and predictive value of autoantibody profiles in patients with systemic sclerosis: a single-center study. PG - 1297-1306 LID - 10.1007/s10067-022-06487-3 [doi] AB - OBJECTIVE: To determine diagnostic accuracy and evaluate the predictive value of autoantibody profiles in patients with systemic sclerosis (SSc). METHODS: A total of 140 patients with SSc (125 female, mean age 54.2 ± 14.2 years) were analyzed by a multiplex line immunoassay (Euroimmun) for autoantibodies against 12 SSc-related antigens. Associations between the presence of the autoantibodies and demographic clinical manifestations of patients with SSc were investigated. RESULTS: The sensitivity and specificity of this assay were as follows: 32.9% and 99.4% for anti-Scl-70, 29.3% and 88.9% for anti-CENP A, 28.6% and 87.8% for anti-CENP B, 7.1% and 97.8% for anti-RP11, 5.7% and 100% for anti-RP155, 2.9% and 99.4% for anti-NOR 90, 2.9% and 98.9% for anti-Th/To, 1.4% and 96.7% for anti-PM-Scl-100, 5.0% and 98.3% for anti-PM-Scl-75, and 2.9% and 97.2% for anti-Ku, respectively. Anti-Scl-70 was significantly associated with sine scleroderma (P = 0.003), digital ulcers (P = 0.047), and Raynaud's phenomenon as the first clinical manifestation of onset (P = 0.017). SSc-ILD was more common in patients with anti-Scl-70 (P = 0.029) and less frequent in patients with anti-CENP A (P < 0.001) and anti-CENP B (P < 0.001). There was a significant association between PAH with anti-CENP A (P = 0.008) and anti-CENP B (P = 0.025). Renal involvement was significantly related to anti-NOR90 (P = 0.026) and anti-Th/To (P = 0.026). CONCLUSIONS: This study confirmed the important role of autoantibodies in accurately diagnosing SSc. The autoimmune profile of patients with SSc was related to specific disease manifestations. Key Points • Autoantibody profiles were useful for diagnosing SSc and predicting clinical features of patients. CI - © 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Zhang, Xiaoying AU - Zhang X AUID- ORCID: 0000-0002-8130-1889 AD - Department of Rheumatology and Immunology, Peking University People's Hospital, No.11 Xizhimen South St., Beijing, 100044, China. FAU - Zhang, Huijuan AU - Zhang H AD - Department of Rheumatology, She Xian Hospital, Handan, Hebei Province, China. FAU - Zhao, Jing AU - Zhao J AD - Department of Rheumatology and Immunology, Peking University People's Hospital, No.11 Xizhimen South St., Beijing, 100044, China. FAU - Li, Yun AU - Li Y AD - Department of Rheumatology and Immunology, Peking University People's Hospital, No.11 Xizhimen South St., Beijing, 100044, China. FAU - Wang, Hongyan AU - Wang H AD - Department of Rheumatology and Immunology, Peking University People's Hospital, No.11 Xizhimen South St., Beijing, 100044, China. FAU - Li, Chun AU - Li C AD - Department of Rheumatology and Immunology, Peking University People's Hospital, No.11 Xizhimen South St., Beijing, 100044, China. fiona_leechun@163.com. LA - eng GR - 81871281/National Natural Science Foundation of China/ GR - Z-2018-40-2101/China International Medical Foundation/ GR - 7192211/Beijing Natural Science Foundation/ PT - Journal Article DEP - 20230106 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - digital ulcers SB - IM MH - Adult MH - Female MH - *Scleroderma, Systemic/complications MH - Skin Ulcer MH - *Raynaud Disease/complications MH - Aged MH - Immunoassay MH - Middle Aged MH - Humans MH - Autoantibodies OTO - NOTNLM OT - Autoantibody OT - Diagnosis OT - Predictive value OT - Systemic sclerosis EDAT- 2023/01/06 06:00 MHDA- 2023/04/17 06:41 CRDT- 2023/01/05 23:15 PHST- 2022/07/26 00:00 [received] PHST- 2022/12/12 00:00 [accepted] PHST- 2022/12/10 00:00 [revised] PHST- 2023/04/17 06:41 [medline] PHST- 2023/01/06 06:00 [pubmed] PHST- 2023/01/05 23:15 [entrez] AID - 10.1007/s10067-022-06487-3 [pii] AID - 10.1007/s10067-022-06487-3 [doi] PST - ppublish SO - Clin Rheumatol. 2023 May;42(5):1297-1306. doi: 10.1007/s10067-022-06487-3. Epub 2023 Jan 6. PMID- 25808833 OWN - NLM STAT- MEDLINE DCOM- 20150916 LR - 20160511 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 67 IP - 7 DP - 2015 Jul TI - Epitope Specificity Determines Pathogenicity and Detectability of Anti-Platelet-Derived Growth Factor Receptor α Autoantibodies in Systemic Sclerosis. PG - 1891-903 LID - 10.1002/art.39125 [doi] AB - OBJECTIVE: To identify the epitopes recognized by autoantibodies targeting platelet-derived growth factor receptor α (PDGFRα) in systemic sclerosis (SSc) and develop novel assays for detection of serum anti-PDGFRα autoantibodies. METHODS: Epstein-Barr virus-immortalized B cells from 1 patient with SSc (designated PAM) were screened for expression of IgG binding to PDGFRα and induction of reactive oxygen species in fibroblasts. The variable regions of anti-PDGFRα IgG were cloned into an IgG expression vector to generate distinct recombinant human monoclonal autoantibodies (mAb), which were characterized by binding and functional assays. The epitopes of anti-PDGFRα recombinant human mAb were defined by molecular docking, surface plasmon resonance binding assays, screening of a conformational peptide library spanning the PDGFRα extracellular domains, and expression analyses of alanine-scanned PDGFRα mutants. Direct or competitive enzyme-linked immunosorbent assays were established to detect all serum anti-PDGFRα autoantibodies or, selectively, the agonistic ones. RESULTS: Three types of anti-PDGFRα recombinant human mAb, with the same VH but distinct VL chains, were generated. Nonagonistic VH PAM-Vκ 13B8 recognized 1 linear epitope, whereas agonistic VH PAM-Vλ 16F4 and VH PAM-Vκ 16F4 recognized 2 distinct conformational epitopes. Serum anti-PDGFRα antibodies were detected in 66 of 70 patients with SSc, 63 of 130 healthy controls, 11 of 26 patients with primary Raynaud's phenomenon (RP), and 13 of 29 patients with systemic lupus erythematosus (SLE). Serum VH PAM-Vκ 16F4-like antibodies were found in 24 of 34 patients with SSc, but not in healthy controls, patients with primary RP, or patients with SLE. Peptides composing the VH PAM-Vκ 16F4 epitope inhibited PDGFRα signaling triggered by serum IgG from SSc patients. CONCLUSION: Agonistic anti-PDGFRα autoantibodies are enriched in SSc sera and recognize specific conformational epitopes that can be used to discriminate agonistic from nonagonistic antibodies and block PDGFRα signaling in patients with SSc. CI - © 2015, American College of Rheumatology. FAU - Moroncini, Gianluca AU - Moroncini G AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Grieco, Antonella AU - Grieco A AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Nacci, Giulia AU - Nacci G AD - Università di Torino, Turin, Italy. FAU - Paolini, Chiara AU - Paolini C AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Tonnini, Cecilia AU - Tonnini C AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Pozniak, Katarzyna N AU - Pozniak KN AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Cuccioloni, Massimiliano AU - Cuccioloni M AD - Università di Camerino, Camerino, Italy. FAU - Mozzicafreddo, Matteo AU - Mozzicafreddo M AD - Università di Camerino, Camerino, Italy. FAU - Svegliati, Silvia AU - Svegliati S AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. FAU - Angeletti, Mauro AU - Angeletti M AD - Università di Camerino, Camerino, Italy. FAU - Kazlauskas, Andrius AU - Kazlauskas A AD - Schepens Eye Research Institute and Harvard Medical School, Boston, Massachusetts. FAU - Avvedimento, Enrico V AU - Avvedimento EV AD - Università Federico II, Naples, Italy. FAU - Funaro, Ada AU - Funaro A AD - Università di Torino, Turin, Italy. FAU - Gabrielli, Armando AU - Gabrielli A AD - Università Politecnica delle Marche and Ospedali Riuniti Ancona, Ancona, Italy. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) RN - 0 (Epitopes) RN - 0 (Reactive Oxygen Species) RN - 9007-34-5 (Collagen) RN - EC 2.7.10.1 (Receptor, Platelet-Derived Growth Factor alpha) SB - IM MH - Amino Acid Sequence MH - Antibody Specificity/*immunology MH - Autoantibodies/blood/chemistry/*immunology MH - Case-Control Studies MH - Collagen/metabolism MH - Epitope Mapping MH - Epitopes/chemistry/*immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/blood/immunology MH - Middle Aged MH - Molecular Sequence Data MH - Protein Conformation MH - Raynaud Disease/blood/immunology MH - Reactive Oxygen Species/metabolism MH - Receptor, Platelet-Derived Growth Factor alpha/chemistry/*immunology MH - Scleroderma, Systemic/blood/*immunology EDAT- 2015/03/27 06:00 MHDA- 2015/09/17 06:00 CRDT- 2015/03/27 06:00 PHST- 2014/07/17 00:00 [received] PHST- 2015/03/17 00:00 [accepted] PHST- 2015/03/27 06:00 [entrez] PHST- 2015/03/27 06:00 [pubmed] PHST- 2015/09/17 06:00 [medline] AID - 10.1002/art.39125 [doi] PST - ppublish SO - Arthritis Rheumatol. 2015 Jul;67(7):1891-903. doi: 10.1002/art.39125. PMID- 26142066 OWN - NLM STAT- MEDLINE DCOM- 20160815 LR - 20181113 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 49 IP - 2 DP - 2015 Oct TI - Endothelial Dysfunction and Nailfold Videocapillaroscopy Pattern as Predictors of Digital Ulcers in Systemic Sclerosis: a Cohort Study and Review of the Literature. PG - 240-52 LID - 10.1007/s12016-015-8500-0 [doi] AB - Raynaud's phenomenon and digital ulcers (DUs) are frequent among systemic sclerosis (SSc) patients. Our aim was to investigate the diagnostic and predictive value for DU of endothelial dysfunction biomarkers (flow-mediated dilatation (FMD), serum levels of endothelin-1 (ET-1), and ADMA), angiogenic/angiostatic biomarkers (vascular endothelial growth factor (VEGF), endoglin, and endostatin), and nailfold videocapillaroscopy (NVC). We compared our results with a literature review. In a cohort study of 77 SSc patients, we followed two groups of patients: (i) naïve DU patients (39) and (ii) active DU at baseline (38 patients) for 3 years. Telangiectasia (p < 0.001) and diffuse disease subset (p = 0.001) were significantly more frequent in patients with active DU at enrolment. Additionally, NVC late scleroderma pattern (AUC 0.846, 95%CI 0.760-0.932), lower values of FMD (AUC 0.754, 95%CI 0.643-0.864), increased serum levels of ET-1 (AUC 0.758, 95%CI 0.649-0.866), ADMA (AUC 0.634, 95%CI 0.511-0.757), and endoglin as well as low VEGF serum levels (AUC 0.705, 95%CI 0.579-0.830) were significantly associated to new DU events in the 3-year follow-up. Cox regression analysis showed that FMD > 9.41 % (HR 0.37, 95%CI 0.14-0.99); ET-1 >11.85 pmol/L (HR 3.81, 95%CI 1.41-10.26) and late NVC pattern (HR 2.29, 95%CI 0.97-5.38) were independent predictors of DU recurrence. When estimating the probability of occurrence of first DU in naïve DU patients, only late NVC pattern (HR 12.66, 95%CI 2.06-77.89) was an independent predictor factor. In conclusion, late scleroderma patterns in NVC are the best independent predictors of SSc patients who are at risk of developing DU. Endothelial dysfunction assessed by FMD and ET-1 was also found to be an independent predictor of DU recurrence in a 3-year follow-up. FAU - Silva, Ivone AU - Silva I AD - Angiology and Vascular Surgery Service and Clinical Immunology Unit, Centro Hospitalar do Porto, Praça da Revista o Tripeiro, n° 42, hab 23, 4150-789, Porto, Portugal. heitor.ivone@gmail.com. AD - Clinical Immunology Unit, Porto, Portugal. heitor.ivone@gmail.com. FAU - Teixeira, Andreia AU - Teixeira A AD - CINTESIS - Center for Research in Health Technologies and Information Systems Porto, Porto, Portugal. FAU - Oliveira, José AU - Oliveira J AD - Clinical Pathology Department, Centro Hospitalar do Porto (CHP), Porto, Portugal. FAU - Almeida, Isabel AU - Almeida I AD - Clinical Immunology Unit, Porto, Portugal. FAU - Almeida, Rui AU - Almeida R AD - Angiology and Vascular Surgery Service and Clinical Immunology Unit, Centro Hospitalar do Porto, Praça da Revista o Tripeiro, n° 42, hab 23, 4150-789, Porto, Portugal. FAU - Águas, Artur AU - Águas A AD - Abel Salazar Biomedical Health Institute, Porto, Portugal. AD - Multidisciplinary Unit for Biomedical Investigation (UMIB), University of Porto, Porto, Portugal. FAU - Vasconcelos, Carlos AU - Vasconcelos C AD - Clinical Immunology Unit, Porto, Portugal. AD - Abel Salazar Biomedical Health Institute, Porto, Portugal. AD - Multidisciplinary Unit for Biomedical Investigation (UMIB), University of Porto, Porto, Portugal. LA - eng PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Antigens, CD) RN - 0 (Biomarkers) RN - 0 (ENG protein, human) RN - 0 (Endoglin) RN - 0 (Endothelin-1) RN - 0 (Receptors, Cell Surface) RN - 0 (Vascular Endothelial Growth Factor A) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antigens, CD/blood MH - Biomarkers/blood MH - Cohort Studies MH - Endoglin MH - Endothelin-1/*blood MH - Endothelium/metabolism/pathology MH - Female MH - Fingers/blood supply/*pathology MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/*diagnosis/etiology MH - Receptors, Cell Surface/blood MH - Scleroderma, Systemic/complications/*diagnosis MH - Ulcer/*diagnosis/etiology MH - Vascular Endothelial Growth Factor A/blood MH - Young Adult OTO - NOTNLM OT - Capillaroscopy OT - Digital ulcer OT - Endothelin-1 OT - Flow-mediated dilatation OT - VEGF EDAT- 2015/07/05 06:00 MHDA- 2016/08/16 06:00 CRDT- 2015/07/05 06:00 PHST- 2015/07/05 06:00 [entrez] PHST- 2015/07/05 06:00 [pubmed] PHST- 2016/08/16 06:00 [medline] AID - 10.1007/s12016-015-8500-0 [pii] AID - 10.1007/s12016-015-8500-0 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2015 Oct;49(2):240-52. doi: 10.1007/s12016-015-8500-0. PMID- 41108352 OWN - NLM STAT- MEDLINE DCOM- 20251112 LR - 20251205 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 12 DP - 2025 Dec TI - Concurrency of systemic sclerosis and sarcoidosis: a case-based review. PG - 5129-5133 LID - 10.1007/s10067-025-07756-7 [doi] AB - Systemic sclerosis (SSc) and sarcoidosis are two rare, multisystem autoimmune diseases with distinct pathophysiologic mechanisms and clinical features. Their coexistence is uncommon and remains an ongoing research subject to determine its significance and underlying mechanisms. Herein, we report the case of a 58-year-old woman with Raynaud's phenomenon, sclerodactyly, and anti-Scl-70 positive, who was initially diagnosed with SSc. Twelve months into treatment, she developed weight loss, fatigue, and progressive respiratory impairment. Imaging revealed mediastinal and mesenteric lymphadenopathy along with fibroatelectatic lung changes. Endobronchial ultrasound-guided biopsy and PET-CT scan suggested sarcoidosis, which was histologically confirmed by the presence of noncaseating granulomas and multinucleated giant cells. The patient was diagnosed with an overlap syndrome of SSc and sarcoidosis and treated accordingly. Clinical improvement and radiologic regression of lymphadenopathy were observed 2 months post-treatment. This is one of the few published cases of concurrent SSc and scleroderma. A review was also performed on the known cases, emphasizing possible pathophysiologic mechanisms, clinical and laboratory findings that can contribute to an early and accurate diagnosis. According to the known evidence, 68.9% of reported cases involve female patients, and in approximately half of them, SSc was diagnosed first. In particular, limited SSc appears to be nearly twice as prevalent as the diffuse form. Our literature review underscores the rarity of this overlap and highlights recurring patterns that may aid in clinical recognition. However, the limited number of reported cases highlights the need for further studies to better define the epidemiology, pathogenesis, and optimal management of this uncommon association. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Chatzistamati, Anastasia AU - Chatzistamati A AUID- ORCID: 0009-0007-3943-7380 AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. anastasiachatzistamati@gmail.com. FAU - Melissaropoulos, Konstantinos AU - Melissaropoulos K AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. FAU - Mparis, Neoklis AU - Mparis N AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. FAU - Bounia, Konstantina AU - Bounia K AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. FAU - Giannopoulos, Georgios AU - Giannopoulos G AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. FAU - Gkanetsou, Dimitra AU - Gkanetsou D AD - Department of Pathology, University Hospital of Patras, Patras, Greece. FAU - Bravou, Vasiliki AU - Bravou V AD - Department of Pathology, University Hospital of Patras, Patras, Greece. FAU - Sampsonas, Fotios AU - Sampsonas F AD - Department of Pulmonology, University Hospital of Patras, Patras, Greece. FAU - Vlachanastasi, Chrysavgi AU - Vlachanastasi C AD - Department of Rheumatology, Agios Andreas General Hospital, Patras, Greece. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20251018 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Female MH - Humans MH - Middle Aged MH - Lymphadenopathy/diagnostic imaging MH - Positron Emission Tomography Computed Tomography MH - Raynaud Disease/complications MH - *Sarcoidosis/complications/diagnosis/therapy MH - *Scleroderma, Systemic/complications/diagnosis/therapy OTO - NOTNLM OT - Concurrency OT - Lymphadenopathy OT - Sarcoidosis OT - Systemic sclerosis COIS- Compliance with ethical standards. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Disclosure: None. EDAT- 2025/10/18 12:30 MHDA- 2025/11/13 00:30 CRDT- 2025/10/18 11:03 PHST- 2025/09/03 00:00 [received] PHST- 2025/10/11 00:00 [accepted] PHST- 2025/10/09 00:00 [revised] PHST- 2025/11/13 00:30 [medline] PHST- 2025/10/18 12:30 [pubmed] PHST- 2025/10/18 11:03 [entrez] AID - 10.1007/s10067-025-07756-7 [pii] AID - 10.1007/s10067-025-07756-7 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Dec;44(12):5129-5133. doi: 10.1007/s10067-025-07756-7. Epub 2025 Oct 18. PMID- 37531032 OWN - NLM STAT- MEDLINE DCOM- 20230830 LR - 20260127 IS - 1534-3081 (Electronic) IS - 1534-3081 (Linking) VI - 27 IP - 9 DP - 2023 Sep TI - A Brief Review of Gepants. PG - 479-488 LID - 10.1007/s11916-023-01142-1 [doi] AB - PURPOSE OF REVIEW: Gepants are small molecules that antagonize calcitonin gene-related peptide (CGRP) receptors. Due to their favorable side effect profile and versatility in treating headaches acutely and preventively, gepants are preferred over triptans. We will cover the indications for the four FDA-approved gepants in adults: rimegepant, atogepant, ubrogepant, and zavegepant. This review will illustrate how gepants will continue to revolutionize the acute and preventive treatment of headaches. RECENT FINDINGS: Gepants are now available in oral tablet, dissolving tablet, and intra-nasal spray formulations. Recent studies have shown promising utility in treating the pre-headache or prodromal phase. They have favorable tolerability, no evidence for association with medication overuse, and remain a safer alternative in those who have cerebrovascular risk factors. Additional research is needed to explore occurrence of Raynaud's phenomenon in participants treated with gepants, as it has been associated with CGRP monoclonal antibodies, but are not extensively studied in gepants. Gepants are expected to play a significant role in the next generation of migraine treatments. CI - © 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. FAU - Li, Diana AU - Li D AUID- ORCID: 0009-0001-2168-0100 AD - Dartmouth Headache Center, Neurology Department, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH, 03756, USA. diana.z.li.md@gmail.com. FAU - Abreu, Jessica AU - Abreu J AD - Dartmouth Headache Center, Neurology Department, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH, 03756, USA. FAU - Tepper, Stewart J AU - Tepper SJ AD - Dartmouth Headache Center, Neurology Department, Dartmouth-Hitchcock Medical Center, 1 Medical Center Drive, Lebanon, NH, 03756, USA. LA - eng PT - Journal Article PT - Review DEP - 20230802 PL - United States TA - Curr Pain Headache Rep JT - Current pain and headache reports JID - 100970666 RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - 0 (Pyridines) RN - 0 (Pyrroles) RN - 1383NM3Q0H (rimegepant sulfate) RN - 0 (Piperidines) RN - 7CRV8RR151 (atogepant) RN - 0 (Spiro Compounds) RN - AD0O8X2QJR (ubrogepant) SB - IM MH - *Calcitonin Gene-Related Peptide Receptor Antagonists/administration & dosage/adverse effects/therapeutic use MH - Humans MH - *Pyridines/administration & dosage/adverse effects/therapeutic use MH - *Pyrroles/administration & dosage/adverse effects/therapeutic use MH - *Migraine Disorders/drug therapy MH - Raynaud Disease/chemically induced MH - Piperidines MH - Spiro Compounds OTO - NOTNLM OT - Atogepant OT - CGRP OT - Gepant OT - Rimegepant OT - Ubrogepant OT - Zavegepant EDAT- 2023/08/02 13:09 MHDA- 2023/08/29 12:42 CRDT- 2023/08/02 11:12 PHST- 2023/06/20 00:00 [accepted] PHST- 2023/08/29 12:42 [medline] PHST- 2023/08/02 13:09 [pubmed] PHST- 2023/08/02 11:12 [entrez] AID - 10.1007/s11916-023-01142-1 [pii] AID - 10.1007/s11916-023-01142-1 [doi] PST - ppublish SO - Curr Pain Headache Rep. 2023 Sep;27(9):479-488. doi: 10.1007/s11916-023-01142-1. Epub 2023 Aug 2. PMID- 27494156 OWN - NLM STAT- MEDLINE DCOM- 20170620 LR - 20210409 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 IP - 1 DP - 2017 Jan-Feb TI - Clinical features and management of erythromelalgia: long term follow-up of 46 cases. PG - 80-84 AB - OBJECTIVES: To review our clinical experience of this rare condition and describe the clinical features and response to therapy in a cohort of patients with erythromelalgia (EM), a rare condition, characterised by paroxysmal hyperthermia of the extremities with erythema, pain and intense burning. METHODS: A review was made of the electronic and paper medical records of patients with the diagnosis of EM, with a telephone interview to verify and complete clinical information relating treatment and outcome. RESULTS: 46 patients (41 females) were included in this study. Mean age was 57 years and mean duration of symptoms was 16 years. Raynaud's phenomenon was present in 36 patients (80%) and 4 patients (9%) had systemic sclerosis. Smoking (current or previous) was identified as a possible risk factor in 26 cases and exposure to chronic vibration in 3 cases. Overall, the effect on quality of life was mild in 15% of cases, moderate in 30% and severe in 48%. The most common symptoms were burning (96%), heat (93%), pain (87%), and redness (83%). Symptoms affected the lower limbs in 98% of cases, upper limbs in 76%, face in 20% and trunk in 11%. Triggers included heat (85%), exercise (78%) and time of day (76%). Various medications were tried, showing poor effect in most cases. Intravenous iloprost was given to 27 patients, with benefit in 17 patients (63%). CONCLUSIONS: Erythromelalgia is a rare chronic debilitating condition. Exercise, heat and night time are common triggers. Current medical therapies are seldom effective and further research is sorely needed. FAU - Parker, Louise K AU - Parker LK AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Ponte, Cristina AU - Ponte C AD - Department of Rheumatology, Hospital de Santa Maria, Lisbon; and Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Lisbon Academic Medical Centre, Lisbon, Portugal. FAU - Howell, Kevin J AU - Howell KJ AD - Microvascular Diagnostics, Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. c.denton@ucl.ac.uk. FAU - Schreiber, Benjamin E AU - Schreiber BE AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. LA - eng PT - Journal Article DEP - 20160802 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Platelet Aggregation Inhibitors) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Erythromelalgia/complications/*diagnosis/*drug therapy MH - Female MH - Follow-Up Studies MH - Humans MH - Iloprost/*therapeutic use MH - Male MH - Middle Aged MH - Platelet Aggregation Inhibitors/*therapeutic use MH - Quality of Life MH - Raynaud Disease/complications MH - Risk Factors MH - Smoking MH - Symptom Assessment MH - Treatment Outcome EDAT- 2016/08/06 06:00 MHDA- 2017/06/21 06:00 CRDT- 2016/08/06 06:00 PHST- 2016/03/03 00:00 [received] PHST- 2016/07/04 00:00 [accepted] PHST- 2016/08/06 06:00 [pubmed] PHST- 2017/06/21 06:00 [medline] PHST- 2016/08/06 06:00 [entrez] AID - 10493 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Jan-Feb;35(1):80-84. Epub 2016 Aug 2. PMID- 17494087 OWN - NLM STAT- MEDLINE DCOM- 20070917 LR - 20151119 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 7 DP - 2007 Jul TI - Maximum blood flow and microvascular regulatory responses in systemic sclerosis. PG - 1079-82 AB - Objectives. To investigate microvascular function using laser Doppler imaging (LDI) following response to hyperaemia, neurovascular reflex and iontophoresis in systemic sclerosis (SSc) in comparison with primary Raynaud's phenomenon (PRP) and age-matched controls. A secondary aim was to evaluate if SSc patients with a higher Medsger vascular score have lower endothelial responses. Methods. Twenty patients with SSc, 10 PRP and 17 controls were studied. Patients with SSc were scored using the vascular component of the Medsger severity scale. A baseline LDI scan was performed on the dorsal aspect of both hands. Digital responses were quantified following maximum hyperaemic response (MHR), contralateral vasoconstrictor response (CLVc) and iontophoresis with acetylcholine (Ach)-endothelial dependent and sodium nitroprusside (SNP)-endothelial independent. Mean blood flow was quantified over a standard region of interest. Results. MHR was lower in SSc patients compared with controls (P < 0.001). A similar trend was seen when comparing SSc with PRP although this did not reach significance (P = 0.07). CLVc and Ach/MHR were lower in SSc vs PRP (P < 0.05) and controls (P < 0.001). No difference was observed in MHR, CLVc and Ach/MHR between PRP and controls. Overall, SNP/MHR was similar in all the three groups. SSc patients with a higher Medsger vascular score had lower endothelial-dependent (P < 0.01) and independent (P < 0.05) responses. Conclusion. SSc patients have abnormal microvascular regulatory responses compared with PRP and controls. This study also suggests that the degree of endothelial dysfunction may be related to the degree of peripheral vascular involvement. FAU - Gunawardena, H AU - Gunawardena H AD - Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, BA1 1RL, UK. FAU - Harris, N D AU - Harris ND FAU - Carmichael, C AU - Carmichael C FAU - McHugh, N J AU - McHugh NJ LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070509 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) RN - 169D1260KM (Nitroprusside) RN - N9YNS0M02X (Acetylcholine) SB - IM MH - Acetylcholine MH - Adult MH - Case-Control Studies MH - Endothelium, Vascular/*physiopathology MH - Fingers/blood supply MH - Hand/blood supply MH - Humans MH - Hyperemia MH - Iontophoresis MH - Laser-Doppler Flowmetry MH - Microcirculation/*physiopathology MH - Middle Aged MH - Nitroprusside MH - Raynaud Disease/physiopathology MH - Regional Blood Flow/drug effects MH - Scleroderma, Systemic/*physiopathology MH - Statistics, Nonparametric MH - Vasodilator Agents EDAT- 2007/05/12 09:00 MHDA- 2007/09/18 09:00 CRDT- 2007/05/12 09:00 PHST- 2007/05/12 09:00 [pubmed] PHST- 2007/09/18 09:00 [medline] PHST- 2007/05/12 09:00 [entrez] AID - kem085 [pii] AID - 10.1093/rheumatology/kem085 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Jul;46(7):1079-82. doi: 10.1093/rheumatology/kem085. Epub 2007 May 9. PMID- 25542902 OWN - NLM STAT- MEDLINE DCOM- 20160325 LR - 20220409 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 24 IP - 8 DP - 2015 Jul TI - Hand function and performance of daily activities in systemic lupus erythematosus: a clinical study. PG - 827-34 LID - 10.1177/0961203314565690 [doi] AB - This clinical study was performed to investigate hand problems in individuals with systemic lupus erythematosus (SLE) in comparison with healthy controls, and to explore problems in the performance of daily activities related to these hand problems, in order to objectify findings from a previous mail survey. We also investigated whether a simple hand test could detect hand problems in SLE. All individuals, 71 with SLE and 71 healthy controls, were examined for manifestations in body structures and body functions of the hands with a study-specific protocol. The simple hand test was performed by all the individuals and the arthritis impact measurement scale (AIMS 2) questionnaire was completed by the SLE individuals. In the SLE group, 58% had some kind of difficulty in the simple hand test, compared with 8% in the control group. Fifty percent of the SLE individuals experienced problems in performing daily activities due to hand deficits. Pain in the hands, reduced strength and dexterity, Raynaud's phenomenon and trigger finger were the most prominent body functions affecting the performance of daily activities. Deficits in hand function are common in SLE and affect the performance of daily activities. The simple hand test may be a useful tool in detecting hand problems. CI - © The Author(s) 2014. FAU - Malcus Johnsson, P AU - Malcus Johnsson P AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden pia.malcusjohnsson@skane.se. FAU - Sandqvist, G AU - Sandqvist G AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden. FAU - Nilsson, J-Å AU - Nilsson JÅ AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden. FAU - Bengtsson, A A AU - Bengtsson AA AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden. FAU - Sturfelt, G AU - Sturfelt G AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden. FAU - Nived, O AU - Nived O AD - Lund University, Department of Clinical Sciences, Rheumatology, Lund, Sweden. LA - eng PT - Clinical Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141226 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - *Activities of Daily Living MH - Adult MH - Case-Control Studies MH - Female MH - Hand/*physiopathology MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Middle Aged MH - Pain MH - Raynaud Disease MH - Surveys and Questionnaires OTO - NOTNLM OT - SLE OT - daily activities OT - hand function EDAT- 2014/12/30 06:00 MHDA- 2016/03/26 06:00 CRDT- 2014/12/28 06:00 PHST- 2014/06/04 00:00 [received] PHST- 2014/12/03 00:00 [accepted] PHST- 2014/12/28 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2016/03/26 06:00 [medline] AID - 0961203314565690 [pii] AID - 10.1177/0961203314565690 [doi] PST - ppublish SO - Lupus. 2015 Jul;24(8):827-34. doi: 10.1177/0961203314565690. Epub 2014 Dec 26. PMID- 27731900 OWN - NLM STAT- MEDLINE DCOM- 20180326 LR - 20220331 IS - 1468-2494 (Electronic) IS - 0142-5463 (Linking) VI - 39 IP - 3 DP - 2017 Jun TI - Relationships between transepidermal water loss, cutaneous microcirculatory function and autonomic nervous activity. PG - 275-283 LID - 10.1111/ics.12373 [doi] AB - OBJECTIVE: Several studies have shown that a deterioration of skin properties, an impaired cutaneous microcirculatory function and an imbalance of autonomic nervous activity are observed in smokers and in patients with diabetes mellitus or Raynaud's phenomenon. These observations suggest that skin properties are associated with cutaneous microcirculatory function and autonomic nervous activity in pathological conditions. However, there is no published evidence to support the concept that these two functions have any relationship with skin properties even in healthy subjects. To investigate the hypothesis that these properties are related, we conducted a survey of healthy adult subjects to investigate the relationships between cutaneous microcirculatory function and autonomic nervous activity and skin properties. METHODS: The hydration of the stratum corneum and transepidermal water loss (TEWL) were investigated as skin properties, and the responsiveness of skin blood flow (SkBF) to local warming was examined as an index of cutaneous microcirculatory function in 19 healthy adult male subjects. Electrocardiograms were monitored for 24 h and heart rate variability was analysed considering low-frequency power (LF: 0.04-0.15 Hz), high-frequency power (HF: 0.15-0.40 Hz) and a ratio of low- to high-frequency power (LF/HF) as indices of autonomic nervous activity; HF is an index of parasympathetic activity, whereas LF/HF is an index of sympathovagal balance. The relationships between those indices were then analysed. RESULTS: A moderate negative correlation was found between TEWL and the relative maximum rate of increases in the responsiveness of SkBF on local warming. A moderate positive and a moderate negative correlation were observed between TEWL and LF/HF or HF, respectively. Moreover, a moderate negative and a moderate positive correlation were shown between the responsiveness of SkBF and LF/HF or HF, respectively. The hydration of the stratum corneum showed no correlations with any indices of microcirculation or autonomic nervous activity. CONCLUSION: These results indicate that skin barrier function, cutaneous microcirculatory function and autonomic nervous activity are mutually associated in healthy adults. CI - © 2016 Society of Cosmetic Scientists and the Société Française de Cosmétologie. FAU - Nomura, T AU - Nomura T AD - Biological Science Research, Kao Corporation, 2606 Akabane, Ichikai-machi, Haga, Tochigi, 321-3497 Japan. FAU - Yoshida-Amano, Y AU - Yoshida-Amano Y AD - Health Beauty Products, Kao Corporation, 2-1-3 Bunka, Sumida-ku, Tokyo, 131-8501 Japan. FAU - Yoshida, K AU - Yoshida K AD - Health Beauty Products, Kao Corporation, 2-1-3 Bunka, Sumida-ku, Tokyo, 131-8501 Japan. FAU - Fujii, A AU - Fujii A AD - Biological Science Research, Kao Corporation, 2606 Akabane, Ichikai-machi, Haga, Tochigi, 321-3497 Japan. FAU - Tanahashi, M AU - Tanahashi M AD - Health Beauty Products, Kao Corporation, 2-1-3 Bunka, Sumida-ku, Tokyo, 131-8501 Japan. FAU - Sugiyama, Y AU - Sugiyama Y AD - Health Beauty Products, Kao Corporation, 2-1-3 Bunka, Sumida-ku, Tokyo, 131-8501 Japan. FAU - Iwata, K AU - Iwata K AD - Lifestyle Research Center, Kao Corporation, 2-1-3 Bunka, Sumida-ku, Tokyo, 131-8501 Japan. FAU - Murase, T AU - Murase T AD - Biological Science Research, Kao Corporation, 2606 Akabane, Ichikai-machi, Haga, Tochigi, 321-3497 Japan. LA - eng PT - Journal Article DEP - 20161102 PL - England TA - Int J Cosmet Sci JT - International journal of cosmetic science JID - 8007161 SB - IM MH - Adult MH - Autonomic Nervous System/*physiopathology MH - *Body Water MH - Diabetes Mellitus/physiopathology MH - Humans MH - Male MH - *Microcirculation MH - Middle Aged MH - Raynaud Disease/physiopathology MH - Skin/*blood supply MH - Young Adult OTO - NOTNLM OT - autonomic nervous activity OT - local warming OT - skin barrier OT - skin blood flow EDAT- 2016/11/03 06:00 MHDA- 2018/03/27 06:00 CRDT- 2016/10/13 06:00 PHST- 2016/08/09 00:00 [received] PHST- 2016/10/08 00:00 [accepted] PHST- 2016/11/03 06:00 [pubmed] PHST- 2018/03/27 06:00 [medline] PHST- 2016/10/13 06:00 [entrez] AID - 10.1111/ics.12373 [doi] PST - ppublish SO - Int J Cosmet Sci. 2017 Jun;39(3):275-283. doi: 10.1111/ics.12373. Epub 2016 Nov 2. PMID- 16437362 OWN - NLM STAT- MEDLINE DCOM- 20070109 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 6 DP - 2006 Nov TI - Prevalence and classification of headache in patients with systemic lupus erythematosus. PG - 850-3 AB - Studies on the prevalence of headache in systemic lupus erythematosus (SLE) have shown that it varies from 32 to 78%. The purpose of our study was to determine the prevalence and characteristics of headache in SLE compared with patients with different types of diffuse connective tissue diseases (DCTD) and its relationship with clinical and laboratory manifestations of SLE. We studied patients with SLE (SLE group) and patients with DCTD (control group). All patients were made to answer questionnaire to assess the presence of headache, characterized by at least five episodes of headache during the last year, which was classified according to the International Headache Society criteria. A total of 207 patients were studied, 115 in SLE group and 92 in the control group. The 1-year prevalence of headache was 75.7% in SLE group and 66% in the control group. When the groups were analyzed, 66.1% met the diagnostic criteria for migraine in the SLE group compared with 52.2% in the control group (p=0.04) and 13.9% for tension-type headache in SLE group compared with 16.3% in the control group. The former was the only variable that reached statistical significance comparing the two groups. Both headache and migraine were associated with Raynaud's phenomenon in SLE patients (odds ratio of 2.80, 95% confidence interval: 1.11-7.05, p=0.02 and odds ratio of 2.34, 95% confidence interval: 1.04-5.23, p=0.03, respectively). These results suggest that headache is a common manifestation in SLE and in other DCTD and we cannot exclude the possibility that it may be related to the emotional stress induced by such clinical situations. FAU - Lessa, Bruno AU - Lessa B AD - Núcleo de Reumatologia da Bahia, Escola Bahiana de Medicina e Saúde Pública, Hospital Santa Izabel, Praça Almeida Couto, 500, Nazaré, Salvador, Bahia, CEP 40.000-000, Brazil. FAU - Santana, Alex AU - Santana A FAU - Lima, Isabella AU - Lima I FAU - Almeida, José Martônio AU - Almeida JM FAU - Santiago, Mittermayer AU - Santiago M LA - eng PT - Journal Article DEP - 20060126 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Connective Tissue Diseases/complications MH - Female MH - Headache/*classification/*epidemiology/etiology MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Male MH - Middle Aged MH - Migraine Disorders/epidemiology MH - Prevalence MH - Raynaud Disease/complications MH - Surveys and Questionnaires MH - Tension-Type Headache/epidemiology EDAT- 2006/01/27 09:00 MHDA- 2007/01/11 09:00 CRDT- 2006/01/27 09:00 PHST- 2005/08/08 00:00 [received] PHST- 2005/12/06 00:00 [accepted] PHST- 2005/12/04 00:00 [revised] PHST- 2006/01/27 09:00 [pubmed] PHST- 2007/01/11 09:00 [medline] PHST- 2006/01/27 09:00 [entrez] AID - 10.1007/s10067-005-0186-x [doi] PST - ppublish SO - Clin Rheumatol. 2006 Nov;25(6):850-3. doi: 10.1007/s10067-005-0186-x. Epub 2006 Jan 26. PMID- 39453291 OWN - NLM STAT- MEDLINE DCOM- 20241206 LR - 20241219 IS - 1525-1403 (Electronic) IS - 1094-7159 (Linking) VI - 27 IP - 8 DP - 2024 Dec TI - A Prospective Single-center Pilot Study on the Use of Closed-loop Spinal Cord Stimulation to Treat Chronic Pain Associated With Raynaud's Phenomenon. PG - 1457-1469 LID - S1094-7159(24)00668-8 [pii] LID - 10.1016/j.neurom.2024.08.005 [doi] AB - OBJECTIVES: Raynaud's phenomenon (RP) is a vascular disorder characterized by episodic peripheral artery vasospasms, resulting in paleness, cyanosis, and/or erythema. There are few reports, mostly case reports, on the benefits of spinal cord stimulation (SCS) for the treatment of RP. However, there is a lack of objective evidence on SCS-induced modulation of the sympathetic system (eg, vasodilation) in this condition. We hypothesize that evoked compound action potential-controlled closed-loop SCS may relieve pain, reduce the severity and frequency of Raynaud attacks, and improve peripheral blood flow. MATERIALS AND METHODS: This prospective, observational, single-center pilot study aimed to evaluate the effectiveness of SCS in treating primary and secondary RP. Patient outcomes such as pain, Raynaud severity/condition score, Cochin Hand Function Scale, Scleroderma Health Assessment Questionnaire RP visual analog scale, EQ-5D-5L, Patient Global Impression of Change, blood flow assessments, and neurophysiological measurements were collected at baseline, trial end, one month, three months, and six months. RESULTS: Ten patients were successfully enrolled in the study and underwent epidural electrode placement for SCS. SCS resulted in a significant improvement in the severity of RP attacks (severity difference from baseline at trial end: -1.8, 95% CI, -3.1 to -0.5; p = 0.01; at one month: -2.1; 95% CI, -3.4 to -0.8; p = 0.004; at three months: -2.9; 95% CI, -4.2 to -1.6; p = 0.0002) and Raynaud condition score (difference from baseline at trial end: -2.1; 95% CI, -3.3 to -0.9; p = 0.002; at one month: -2.2; 95% CI, -3.4 to -1.0; p = 0.002; at three months: -3.3; 95% CI, -4.6 to -2.1; p = 0.00002; at six months: -4.1; 95% CI, -5.4 to -2.8; p = 0.0000008), and an objective reduction in peripheral occlusion and ulceration. While one of the combined primary end points was successfully achieved in terms of severity at the three-month follow-up, it is worth noting that the primary end point related to frequency improvement was not met during the same time frame. CONCLUSIONS: This pilot study offers evidence linking SCS with the activation of large, myelinated fibers within the dorsal column in patients with RP. This activation is associated with improvement in the number of patient-related outcomes and enhanced peripheral circulation. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Maciaczyk, Jarek AU - Maciaczyk J AD - Department of Stereotactic and Functional Neurosurgery, University Hospital Bonn, Bonn, Germany; Department of Surgical Sciences, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand. Electronic address: jaroslaw.maciaczyk@ukbonn.de. FAU - Bara, Gregor AU - Bara G AD - Department of Stereotactic and Functional Neurosurgery, University Hospital Bonn, Bonn, Germany. FAU - Basilaia, Bela AU - Basilaia B AD - Department of Stereotactic and Functional Neurosurgery, University Hospital Bonn, Bonn, Germany. FAU - Abuassi, Mahmoud AU - Abuassi M AD - Department of Stereotactic and Functional Neurosurgery, University Hospital Bonn, Bonn, Germany. FAU - Dietz, Birte E AU - Dietz BE AD - Research, Saluda Medical, Harrogate, United Kingdom. FAU - Mugan, Dave AU - Mugan D AD - Research, Saluda Medical, Harrogate, United Kingdom. FAU - Mayr, Andreas AU - Mayr A AD - Institute for Medical Biometry, Informatics and Epidemiology, University Hospital Bonn, Bonn, Germany. FAU - Staerk, Christian AU - Staerk C AD - Institute for Medical Biometry, Informatics and Epidemiology, University Hospital Bonn, Bonn, Germany. FAU - Karakostas, Pantelis AU - Karakostas P AD - Departments of Rheumatology and Clinical Immunology, and Internal Medicine III, University Hospital Bonn, Bonn, Germany. FAU - Schäfer, Valentin S AU - Schäfer VS AD - Departments of Rheumatology and Clinical Immunology, and Internal Medicine III, University Hospital Bonn, Bonn, Germany. LA - eng PT - Journal Article PT - Observational Study DEP - 20241022 PL - United States TA - Neuromodulation JT - Neuromodulation : journal of the International Neuromodulation Society JID - 9804159 SB - IM MH - Humans MH - Pilot Projects MH - *Raynaud Disease/therapy MH - Female MH - *Spinal Cord Stimulation/methods MH - Middle Aged MH - Male MH - Prospective Studies MH - Adult MH - *Chronic Pain/therapy MH - Treatment Outcome MH - Aged MH - Pain Measurement/methods OTO - NOTNLM OT - Evoked compound action potential OT - Raynaud phenomenon OT - spinal cord stimulation OT - ultrasonography COIS- Conflict of Interest Jarek Maciaczyk receives speaker fees from Boston Scientific, Saluda Medical, and Nevro; is a member of advisory boards of Boston Scientific, Saluda Medical, and Nevro; and has received research grants from Mainstay and Saluda Medical. Valentin S. Schäfer receives speaker fees from AbbVie, Boehringer Ingelheim, Bristol Myers Squibb (BMS), Chugai, Celgene, Hexal, Janssen, Eli Lilly, Medac, Novartis, Onkowissen, Pfizer, Roche, Royal College of Physicians, Rheumaakademie, Sanofi, Takeda, and UCB; is an advisory board member for AbbVie, Amgen, BMS, Boehringer Ingelheim, Celgene, Chugai, Gilead, Hexal, Eli Lilly, Medac, Novartis, Pfizer, Roche, and Sanofi; and receives research support from Boehringer Ingelheim, Celgene, Hexal, Universität Bonn, Eli Lilly, Novartis, Roche, and Alexion. Birte E. Dietz and Dave Mugan are employed by Saluda Medical. The remaining authors reported no conflict of interest. EDAT- 2024/10/25 12:24 MHDA- 2024/12/07 14:47 CRDT- 2024/10/25 10:32 PHST- 2024/05/03 00:00 [received] PHST- 2024/07/30 00:00 [revised] PHST- 2024/08/13 00:00 [accepted] PHST- 2024/12/07 14:47 [medline] PHST- 2024/10/25 12:24 [pubmed] PHST- 2024/10/25 10:32 [entrez] AID - S1094-7159(24)00668-8 [pii] AID - 10.1016/j.neurom.2024.08.005 [doi] PST - ppublish SO - Neuromodulation. 2024 Dec;27(8):1457-1469. doi: 10.1016/j.neurom.2024.08.005. Epub 2024 Oct 22. PMID- 16724674 OWN - NLM STAT- MEDLINE DCOM- 20060713 LR - 20191210 IS - 0258-851X (Print) IS - 0258-851X (Linking) VI - 20 IP - 3 DP - 2006 May-Jun TI - Iloprost enhances portal flow velocity and volume in patients with systemic sclerosis. PG - 377-80 AB - BACKGROUND: Iloprost, a prostacyclin analog, reduces hepatic microcirculatory damage after ischemia-reperfusion injury in animal liver models. The objective of this study was to evaluate whether the portal flow velocity changes after Iloprost infusion in patients with systemic sclerosis and Raynaud's phenomenon, who usually have increased risk of microvascular thrombosis and transient liver disturbances. PATIENTS AND METHODS: Fifteen patients (3 males and 12 females, median age 58 years, range 47-66 years), with systemic sclerosis and Raynaud's phenomenon, were exclusively treated with an infusion of Iloprost (2 ng/kg/min, 6 h/day) for 5 days. In each subject, the portal flow velocity (PV, cm/sec) and portal flow volume (PFV, mL/min) were obtained by using portal color Doppler ultrasonography equipment. RESULTS: Iloprost administration significantly (p<0.001) increased both the PV (23.6+/-3.4 cmlsec vs. 29.1+/-3.9 cm/sec) and PFV (1748.8+/-310. 7 mL/min vs. 2254.9+/-404.1 mL/min) values. CONCLUSION: Hepatic perfusion significantly improved after Iloprost administration, suggesting that such treatment might be useful in preventing vascular complications in patients with systemic sclerosis. Iloprost improves the portal hemodynamics, favoring local microvascular patency, and its effectiveness may be safely monitored by using portal color Doppler ultrasonography. FAU - Zardi, Enrico Maria AU - Zardi EM AD - Interdisciplinary Center for Biomedical Research (CIR), Laboratory of Internal Medicine and Hepatology and Campus Bio-Medico University, School of Medicine, 00155 Roma, Italy. FAU - Picardi, Antonio AU - Picardi A FAU - Ambrosino, Giovanni AU - Ambrosino G FAU - Fazio, Vito Michele AU - Fazio VM FAU - Dobrina, Aldo AU - Dobrina A FAU - Frego, Mauro AU - Frego M FAU - Afeltra, Antonella AU - Afeltra A FAU - Lumachi, Franco AU - Lumachi F LA - eng PT - Comparative Study PT - Evaluation Study PT - Journal Article PL - Greece TA - In Vivo JT - In vivo (Athens, Greece) JID - 8806809 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Aged MH - Blood Flow Velocity/drug effects MH - Evaluation Studies as Topic MH - Female MH - Humans MH - Iloprost/*pharmacology/therapeutic use MH - Liver Circulation/drug effects MH - Male MH - Middle Aged MH - Portal System/diagnostic imaging/*drug effects MH - Raynaud Disease/*complications MH - Regional Blood Flow/drug effects MH - Scleroderma, Systemic/blood/*drug therapy/*physiopathology MH - Ultrasonography, Doppler, Color MH - Vasodilator Agents/*pharmacology/therapeutic use EDAT- 2006/05/27 09:00 MHDA- 2006/07/14 09:00 CRDT- 2006/05/27 09:00 PHST- 2006/05/27 09:00 [pubmed] PHST- 2006/07/14 09:00 [medline] PHST- 2006/05/27 09:00 [entrez] PST - ppublish SO - In Vivo. 2006 May-Jun;20(3):377-80. PMID- 35894657 OWN - NLM STAT- MEDLINE DCOM- 20230203 LR - 20260127 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 62 IP - 2 DP - 2023 Feb 1 TI - A phase 2 trial investigating the effects of the angiotensin II type 2 receptor agonist C21 in systemic sclerosis-related Raynaud's. PG - 824-828 LID - 10.1093/rheumatology/keac426 [doi] AB - OBJECTIVE: Our main aim was to investigate the effect of a single oral dose of C21, a selective angiotensin II type 2 receptor agonist, on cold-induced vasoconstriction in SSc-related RP. METHODS: This was a phase IIa, randomized, double-blind, cross-over, single-dose, placebo-controlled, single-centre study. Twelve female patients with SSc (median age 58.5 years, median duration of RP 19.0 years) attended on four occasions: screening, treatment visits 1 and 2 (separated by 3-7 days) and follow-up. At the first treatment visit, patients were randomized to receive either a single oral dose of C21 (200 mg) or placebo, then the opposite treatment on the second visit. Forty min after each treatment, each patient underwent a standard hand cold challenge. The primary end point was the area under the curve (AUC) for rewarming for each finger (eight fingers) over 15 min. Secondary end points included the maximum finger temperature after rewarming (MAX). Statistical analyses were performed by multiplicative ANCOVA models. RESULTS: For all eight fingers combined, mean AUC for rewarming was higher after treatment with C21 than after placebo (geometric mean 20 046°C*s vs 19 558°C*s), but not significantly (P = 0.380) and MAX (at 15 min) was also higher (geometric mean 23.5°C vs 22.5°C; P = 0.036). C21 was well tolerated. CONCLUSION: Despite the small trial size, a signal emerged suggesting that even in patients with established SSc, C21 may confer benefit for RP and deserves further investigation. TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT04388176. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, The University of Manchester, Northern Care Alliance NHS Foundation Trust. AD - NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Batta, Rohit AU - Batta R AD - Vicore Pharma AB, Gothenburg. FAU - Overbeck, Kamilla AU - Overbeck K AD - Vicore Pharma AB, Gothenburg. FAU - Raud, Johan AU - Raud J AD - Vicore Pharma AB, Gothenburg. AD - Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden. FAU - Manning, Joanne AU - Manning J AD - Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre. FAU - Tornling, Göran AU - Tornling G AD - Respiratory Medicine Division, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. LA - eng SI - ClinicalTrials.gov/NCT04388176 PT - Clinical Trial, Phase II PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Receptor, Angiotensin, Type 2) RN - RC2V4W0EYC (compound 21) RN - 0 (Imidazoles) RN - 0 (Sulfonamides) RN - 0 (Thiophenes) SB - IM MH - Humans MH - Female MH - Middle Aged MH - Receptor, Angiotensin, Type 2/therapeutic use MH - *Scleroderma, Systemic/complications/drug therapy/diagnosis MH - Fingers MH - Body Temperature MH - *Raynaud Disease/etiology/complications MH - Imidazoles MH - Sulfonamides MH - Thiophenes PMC - PMC9891408 OTO - NOTNLM OT - RP OT - SSc OT - angiotensin II type 2 receptor agonist OT - randomized controlled trial OT - thermography EDAT- 2022/07/28 06:00 MHDA- 2023/02/04 06:00 PMCR- 2022/07/27 CRDT- 2022/07/27 09:22 PHST- 2022/05/10 00:00 [received] PHST- 2022/07/19 00:00 [accepted] PHST- 2022/07/28 06:00 [pubmed] PHST- 2023/02/04 06:00 [medline] PHST- 2022/07/27 09:22 [entrez] PHST- 2022/07/27 00:00 [pmc-release] AID - 6650618 [pii] AID - keac426 [pii] AID - 10.1093/rheumatology/keac426 [doi] PST - ppublish SO - Rheumatology (Oxford). 2023 Feb 1;62(2):824-828. doi: 10.1093/rheumatology/keac426. PMID- 17301734 OWN - NLM STAT- MEDLINE DCOM- 20070425 LR - 20151119 IS - 0009-9236 (Print) IS - 0009-9236 (Linking) VI - 81 IP - 4 DP - 2007 Apr TI - The effects of tadalafil on cold-induced vasoconstriction in patients with Raynaud's phenomenon. PG - 503-9 AB - Raynaud's phenomenon (RP) is a disorder characterized by episodic periods of vasoconstriction typically provoked by exposure to cold. Phosphodiesterase 5 (PDE5) inhibitors may improve digital blood flow and clinical symptoms in patients with RP, but the mechanisms are unknown. We examined the hypothesis that a PDE5 inhibitor, tadalafil, attenuates cold-induced vasoconstriction. Additionally, we examined whether tadalafil reduced vascular dysfunction following ischemia, thus altering the response to repeated cooling. We conducted a double-blind, placebo-controlled crossover study in 20 subjects with RP on two separate study days, when subjects received either placebo or tadalafil (10 mg). Digital blood flow (flux) was measured by laser Doppler flowmetry at rest and during two graduated local heat and cold exposure cycles. Temperature-response curves were evaluated by E(max) (maximal flux during heating), E(min) (minimal flux during cooling), and ET(50) and ET(90) (the local temperature at which flux decreased by 50% and 90% of E(max)-E(min), respectively). Tadalafil did not increase baseline flux (81.0+/-73.0 vs 91.3+/-114.0 arbitrary unit (AU), P=0.57), E(max) (280.0+/-107.6 vs 279.5+/-119.8 AU, P=0.94), ET(50) (25.4+/-4.4 vs 26.6+/-5.7 degrees C, P=0.62), or ET(90) (21.2+/-3.9 vs 21.8+/-5.0 degrees C, P=0.78), (cycle 1 values presented). There were no differences between cycles on either study day. In conclusion, in patients with RP, single-dose tadalafil does not increase digital blood flow at baseline or in response to heating, nor does it attenuate cold-induced vasoconstriction. Furthermore, it does not precondition the endothelium to resist a second cooling challenge. The clinical benefit in patients with RP treated with PDE5 inhibitors probably involves mechanisms other than acute inhibition of cold-induced vasoconstriction. FAU - Friedman, E A AU - Friedman EA AD - 1Division of Clinical Pharmacology, Department of Medicine,Vanderbilt University School of Medicine, Nashville, Tennessee, USA. FAU - Harris, P A AU - Harris PA FAU - Wood, A J J AU - Wood AJ FAU - Stein, C M AU - Stein CM FAU - Kurnik, D AU - Kurnik D LA - eng GR - GM-31304/GM/NIGMS NIH HHS/United States GR - HL-04012/HL/NHLBI NIH HHS/United States GR - M01 RR-00095/RR/NCRR NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20070214 PL - United States TA - Clin Pharmacol Ther JT - Clinical pharmacology and therapeutics JID - 0372741 RN - 0 (Carbolines) RN - 0 (Phosphodiesterase Inhibitors) RN - 742SXX0ICT (Tadalafil) RN - EC 3.1.4.35 (3',5'-Cyclic-GMP Phosphodiesterases) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) RN - EC 3.1.4.35 (PDE5A protein, human) SB - IM MH - 3',5'-Cyclic-GMP Phosphodiesterases/metabolism MH - Adult MH - Carbolines/adverse effects/*pharmacology MH - Cold Temperature MH - Cross-Over Studies MH - Cyclic Nucleotide Phosphodiesterases, Type 5 MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Phosphodiesterase Inhibitors/adverse effects/*pharmacology MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/drug effects/physiology MH - Skin/blood supply/drug effects MH - Skin Temperature/drug effects/physiology MH - Tadalafil MH - Vasoconstriction/*drug effects EDAT- 2007/02/16 09:00 MHDA- 2007/04/26 09:00 CRDT- 2007/02/16 09:00 PHST- 2007/02/16 09:00 [pubmed] PHST- 2007/04/26 09:00 [medline] PHST- 2007/02/16 09:00 [entrez] AID - 6100103 [pii] AID - 10.1038/sj.clpt.6100103 [doi] PST - ppublish SO - Clin Pharmacol Ther. 2007 Apr;81(4):503-9. doi: 10.1038/sj.clpt.6100103. Epub 2007 Feb 14. PMID- 29781586 OWN - NLM STAT- MEDLINE DCOM- 20190722 LR - 20260127 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 70 IP - 11 DP - 2018 Nov TI - Treatment Algorithms for Systemic Sclerosis According to Experts. PG - 1820-1828 LID - 10.1002/art.40560 [doi] AB - OBJECTIVE: There is a lack of agreement regarding treatment for many aspects of systemic sclerosis (SSc). We undertook this study to generate SSc treatment algorithms endorsed by a high percentage of SSc experts. METHODS: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (n = 170) were asked whether they agreed with SSc algorithms from 2012. Two consensus rounds refined agreement; 62, 54, and 48 experts (36%, 32%, and 28%, respectively) completed the first, second, and third surveys, respectively. RESULTS: For treatment of scleroderma renal crisis, 81% of experts agreed (first-, second-, and third-line treatments were angiotensin-converting enzyme inhibitors, then adding calcium-channel blockers [CCBs], then adding angiotensin receptor blockers [ARBs], respectively). For pulmonary arterial hypertension (PAH), 81% of experts agreed (for mild PAH, treatments were phosphodiesterase 5 [PDE5] inhibitors, then endothelin receptor antagonists plus PDE5 inhibitors, then prostanoids, respectively; for severe PAH, prostanoids were first-line treatment). For mild Raynaud's phenomenon (RP), 79% of experts agreed (treatments were CCBs, then adding PDE5 inhibitors, then ARBs or switching to another CCB, respectively; after the third line of treatment, mild RP was deemed severe). For severe RP, the first- through fourth-line treatments were CCBs, then adding PDE5 inhibitors or prostanoids, then adding PDE5 inhibitors (if not added as second-line treatment) or prostanoids (if not added as second-line treatment), then switching to another CCB, respectively. For active treatment of digital ulcers, 66% of experts agreed (first- and second-line treatments were CCBs and PDE5 inhibitors, respectively). For interstitial lung disease, 69% of experts agreed (for induction therapy, treatments were mycophenolate mofetil [MMF], intravenous cyclophosphamide [IV CYC], and rituximab, respectively; for maintenance, first-line treatment was MMF). For skin involvement, 71% of experts agreed (for a modified Rodnan skin thickness score [MRSS] of 24, first- and second-line treatments were methotrexate [MTX] and MMF, respectively; for an MRSS of 32, first- through fourth-line treatments were MMF, MTX, IV CYC, and hematopoietic stem cell transplantation, respectively). For inflammatory arthritis, 79% of experts agreed (first- through fourth-line treatments were MTX, low-dose glucocorticoids, hydroxychloroquine, and rituximab or tocilizumab, respectively). Algorithms for cardiac and gastrointestinal involvement had ≥75% agreement. CONCLUSION: Total agreement for SSc algorithms was considerable. These algorithms may guide treatment. CI - © 2018, American College of Rheumatology. FAU - Fernández-Codina, Andreu AU - Fernández-Codina A AD - University of Western Ontario, London, Ontario, Canada, and Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Walker, Kyle M AU - Walker KM AD - The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada. FAU - Pope, Janet E AU - Pope JE AD - University of Western Ontario, London, Ontario, Canada. CN - Scleroderma Algorithm Group LA - eng PT - Consensus Statement PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180917 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Antirheumatic Agents) RN - 0 (Calcium Channels) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins) RN - 4F4X42SYQ6 (Rituximab) RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 8N3DW7272P (Cyclophosphamide) RN - HU9DX48N0T (Mycophenolic Acid) RN - I031V2H011 (tocilizumab) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - *Algorithms MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Antirheumatic Agents/therapeutic use MH - Arthritis/*drug therapy/etiology MH - Calcium Channels/therapeutic use MH - Cyclophosphamide/therapeutic use MH - Endothelin Receptor Antagonists/therapeutic use MH - Glucocorticoids/therapeutic use MH - Humans MH - Hydroxychloroquine MH - Hypertension, Pulmonary/*drug therapy/etiology MH - Immunosuppressive Agents/therapeutic use MH - Kidney Diseases/*drug therapy/etiology MH - Lung Diseases, Interstitial/*drug therapy/etiology MH - Methotrexate/therapeutic use MH - Mycophenolic Acid/therapeutic use MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Prostaglandins/therapeutic use MH - Raynaud Disease/*drug therapy/etiology MH - Rituximab/therapeutic use MH - Scleroderma, Systemic/complications/*drug therapy EDAT- 2018/05/22 06:00 MHDA- 2019/07/23 06:00 CRDT- 2018/05/22 06:00 PHST- 2018/01/26 00:00 [received] PHST- 2018/05/08 00:00 [accepted] PHST- 2018/05/22 06:00 [pubmed] PHST- 2019/07/23 06:00 [medline] PHST- 2018/05/22 06:00 [entrez] AID - 10.1002/art.40560 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Nov;70(11):1820-1828. doi: 10.1002/art.40560. Epub 2018 Sep 17. PMID- 26914257 OWN - NLM STAT- MEDLINE DCOM- 20160620 LR - 20250626 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 2 IP - 2 DP - 2016 Feb 25 TI - Calcium channel blockers for primary Raynaud's phenomenon. PG - CD002069 LID - 10.1002/14651858.CD002069.pub5 [doi] LID - CD002069 AB - BACKGROUND: Calcium channel blockers are the most commonly prescribed drugs for people with primary Raynaud's phenomenon. Primary Raynaud's phenomenon is a common condition characterised by an exaggerated vasospastic response to cold or emotion: classically the digits (fingers and toes) turn white, then blue, then red. This is an update of the review first published in 2014. OBJECTIVES: To assess the effects of different calcium channel blockers for primary Raynaud's phenomenon as determined by attack rates, severity scores, participant-preference scores and physiological measurements. SEARCH METHODS: For this update the Cochrane Vascular Trial Search Co-ordinator searched the Specialised Register (last searched January 2016) and the Cochrane Register of Studies (CENTRAL) (2015, Issue 12). In addition the TSC searched clinical trials databases. SELECTION CRITERIA: Randomised controlled trials evaluating the effects of oral calcium channel blockers for the treatment of primary Raynaud's phenomenon. DATA COLLECTION AND ANALYSIS: Three review authors independently assessed the trials for inclusion and their quality, and extracted the data. Data extraction included adverse events. We contacted trial authors for missing data. MAIN RESULTS: We included seven randomised trials with 296 participants. Four trials examined nifedipine and the remainder nicardipine. Comparisons were with placebo in six trials and with both dazoxiben and placebo in one trial (only the nifedipine versus placebo data were used within this review). Treatment with oral calcium channel blockers was minimally effective in primary Raynaud's phenomenon at decreasing the frequency of attacks (standardised mean difference of 0.23; 95% confidence interval (CI) 0.08 to 0.38, P = 0.003). This translates to 1.72 (95% CI 0.60 to 2.84) fewer attacks per week on calcium channel blockers compared to placebo. One trial provided details on duration of attacks reporting no statistically significant difference between the nicardipine and placebo groups (no P value reported). Only two trials provided any detail of statistical comparisons of (unvalidated) severity scores between treatment groups: one of these trials (60 participants) reported a mean severity score of 1.55 on placebo and 1.36 on nicardipine, difference 0.2 (95% CI of difference 0 to 0.4, no P value reported) and the other trial (three participants only with primary Raynaud's phenomenon) reported a median severity score of 2 on both nicardipine and placebo treatment (P > 0.999) suggesting little effect on severity. Participant-preference scores were included in four trials, but in only two were results specific to participants with primary Raynaud's phenomenon, and scoring systems differed between trials: scores differed between treatments in only one trial, in which 33% of participants on placebo and 73% on nifedipine reported improvement in symptoms (P < 0.001). Physiological measurements were included as outcome measures in five trials (different methodologies were used in each): none of these trials found any statistically significant between-treatment group differences. Treatment with calcium channel blockers appeared to be associated with a number of adverse reactions, including headaches, flushing and oedema (swelling). Overall, the trials were classed as being at low or unclear risk of bias; and the quality of the evidence presented was moderate for number of attacks, very low for duration of attacks, high for severity scores and low for patient preference scores. AUTHORS' CONCLUSIONS: The randomised controlled trials included in this review provide moderate quality evidence that oral calcium channel blockers are minimally effective in the treatment of primary Raynaud's phenomenon as measured by the frequency of attacks and high-quality evidence that they have little effect on severity. We are unable to comment on duration of attacks or on patient preference due to the very low and low quality of evidence as a result of small sample sizes in the included studies and the variable data quality of outcome measures. FAU - Ennis, Holly AU - Ennis H AD - Edinburgh Clinical Trials Unit, University of Edinburgh, OPD Building Level 2, Western General Hospital, Edinburgh, UK, EH4 2XU. FAU - Hughes, Michael AU - Hughes M FAU - Anderson, Marina E AU - Anderson ME FAU - Wilkinson, Jack AU - Wilkinson J FAU - Herrick, Ariane L AU - Herrick AL LA - eng GR - ETM/442/CSO_/Chief Scientist Office/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20160225 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Calcium Channel Blockers) RN - CZ5312222S (Nicardipine) RN - I9ZF7L6G2L (Nifedipine) SB - IM UOF - Cochrane Database Syst Rev. 2014 Jan 30;(1):CD002069. doi: 10.1002/14651858.CD002069.pub4. PMID: 24482037 MH - Calcium Channel Blockers/*therapeutic use MH - Humans MH - Nicardipine/therapeutic use MH - Nifedipine/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy PMC - PMC7065590 COIS- HE: declares that she is currently employed by the University of Edinburgh with no known conflicts.
 MH: none known.
 MA: has received honoraria and financial support to attend educational national and international meetings from Actelion Pharmaceuticals and Bristol‐Meyers Squibb. These included Coronary artery hypertension meeting and Consensus best practice for digital ulcer management meeting presentations (Actelion Pharmaceuticals) and national presentations (Bristol‐Meyers Squibb). MA received support to attend Advisory board and consensus best practice for digital ulcer management meeting organised and supported by Actelion Pharmaceutical. MA's institution receives National Digital Ulcer Registry participation payments (supported by Actelion Pharmaceuticals).
 AH: declares that she has undertaken consultancy work for Actelion and Apricus (Data Safety Monitoring Board). AH's institution has received research grant funding from Actelion and she has spoken at meetings sponsored by Actelion. AH has been a PI on a study sponsored by Orion (this study was on systemic sclerosis‐related Raynaud's phenomenon), and is a PI on studies sponsored by Actelion (these studies are on systemic sclerosis‐related Raynaud's phenomenon).
 JW: none known. EDAT- 2016/02/26 06:00 MHDA- 2016/06/21 06:00 PMCR- 2017/02/25 CRDT- 2016/02/26 06:00 PHST- 2016/02/26 06:00 [entrez] PHST- 2016/02/26 06:00 [pubmed] PHST- 2016/06/21 06:00 [medline] PHST- 2017/02/25 00:00 [pmc-release] AID - CD002069.pub5 [pii] AID - 10.1002/14651858.CD002069.pub5 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2016 Feb 25;2(2):CD002069. doi: 10.1002/14651858.CD002069.pub5. PMID- 21243388 OWN - NLM STAT- MEDLINE DCOM- 20110922 LR - 20260128 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 30 IP - 6 DP - 2011 Jun TI - Demographic, clinical, and serological features of Turkish patients with rheumatoid arthritis: evaluation of 165 patients. PG - 843-7 LID - 10.1007/s10067-011-1678-5 [doi] AB - The study was designed to describe demographic, clinical, serological, and radiological characteristics of patients with rheumatoid arthritis (RA) followed-up by a single institution. One hundred sixty-five patients, diagnosed as RA using ACR classification criteria, and followed-up in the rheumatology clinic between December 2005 and January 2010, were enrolled in the study. Of the patients, 125 were female, and 40 were male. Mean age of the patients was 52.5 years, and mean duration of the disease was 10.5 years. The most frequently involved joints were the wrist (95.2%), MCP (90.9%), and the PIP (92.6%). The knee and hip joint involvement rates were 44.8% and 23.6%, respectively. Patients (50.9%) were detected to have tenosynovitis. Involvement of the elbow joint was shown in 10.9% of the patients. The most common extra-articular manifestations were sicca symptom (40.6%) and carpal tunnel syndrome (35.7%), followed by pulmonary involvement (6.6%), vasculitis (3.6%), and Raynaud's phenomenon (1.2%). Rheumatoid nodules were detected in six patients (3.6%). One patient had Felty syndrome, and another patient had secondary amyloidosis. Patients (90.3%) had positive rheumatoid factor (RF), and 124 patients had positive anti-CCP antibody (75.2%). A more severe clinical course and a higher incidence of erosion, tenosynovitis, and deformities were detected in patients with anti-CCP antibody and positive RF (p = 0.03, p = 0.04, p = 0.01, p = 0.04, respectively). The wrist was the most frequently involved joint in our patients, and the most frequently seen extra-articular manifestation was sicca symptom. Presence of RF and anti-CCP antibody was associated with more severe disease including erosive and destructive arthropathy. Extra-articular involvement and presence of accompanying diseases increase the mortality. FAU - Kobak, Senol AU - Kobak S AD - Department of Rheumatology, Manisa Hospital, Manisa Devlet Hastanesi, Manisa, Turkey. senolkobak@yahoo.com LA - eng PT - Journal Article DEP - 20110119 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Anti-Idiotypic) RN - 0 (Peptides, Cyclic) RN - 0 (cyclic citrullinated peptide) RN - 9007-41-4 (C-Reactive Protein) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Antibodies, Anti-Idiotypic/*blood MH - Arthritis, Rheumatoid/*blood/diagnostic imaging/*epidemiology MH - Blood Sedimentation MH - C-Reactive Protein/metabolism MH - Carpal Tunnel Syndrome/epidemiology MH - Comorbidity MH - Female MH - Follow-Up Studies MH - Humans MH - Lung Diseases/epidemiology MH - Male MH - Middle Aged MH - Peptides, Cyclic/*immunology MH - Radiography MH - Raynaud Disease/epidemiology MH - Retrospective Studies MH - Rheumatoid Factor/*blood MH - Sjogren's Syndrome/*epidemiology MH - Tenosynovitis/*epidemiology MH - Turkey/epidemiology MH - Vasculitis/epidemiology EDAT- 2011/01/19 06:00 MHDA- 2011/09/23 06:00 CRDT- 2011/01/19 06:00 PHST- 2010/05/20 00:00 [received] PHST- 2011/01/02 00:00 [accepted] PHST- 2010/12/07 00:00 [revised] PHST- 2011/01/19 06:00 [entrez] PHST- 2011/01/19 06:00 [pubmed] PHST- 2011/09/23 06:00 [medline] AID - 10.1007/s10067-011-1678-5 [doi] PST - ppublish SO - Clin Rheumatol. 2011 Jun;30(6):843-7. doi: 10.1007/s10067-011-1678-5. Epub 2011 Jan 19. PMID- 41124832 OWN - NLM STAT- MEDLINE DCOM- 20251213 LR - 20251213 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 75 DP - 2025 Dec TI - Rheumatic manifestations of alkaptonuria: clinical, genetic and capillaroscopic characterisation of a referral centre cohort reveals new capillaroscopic marker of the disease. PG - 152856 LID - S0049-0172(25)00227-6 [pii] LID - 10.1016/j.semarthrit.2025.152856 [doi] AB - OBJECTIVES: Alkaptonuria is a rare genetic disorder caused by the build-up of homogentisic acid, leading to its deposition in connective tissue. Previous studies have documented histopathological evidence of extracellular matrix and vessel wall deposits and neoangiogenesis in patients with alkaptonuria, as well as the possible role of dermoscopy in characterising macroscopically visible skin deposits. This proof-of-concept study aimed to describe the capillaroscopic changes in patients with alkaptonuria. METHODS: In this cross-sectional single-centre study, alkaptonuria patients were evaluated phenotypically, genetically, and with capillaroscopy. Capillaroscopic findings were compared to three control groups: healthy controls, patients with primary Raynaud's phenomenon and patients with systemic sclerosis. RESULTS: Ten alkaptonuria patients were included (60 % female, mean age 54.4 ± 12.2 years). All patients had skin and/or sclerae pigmentation and the majority (90 %) had musculoskeletal manifestations. One patient had a normal capillaroscopy. Blue-blackish deposits suggestive of homogentisic acid accumulation were observed in six patients, the majority of which were not visible to the naked eye. None of the patients from the three control groups had this capillaroscopic finding. Nine patients with alkaptonuria presented atypical, non-specific capillaroscopic changes. Notably, we observed a significantly higher prevalence of abnormally shaped capillaries compared both to healthy controls (70.0 % vs 20.0 %, p = 0.034) and to patients with primary Raynaud's phenomenon (70.0 % vs 20.0 %, p = 0.034), and of dilated capillaries compared to patients with primary Raynaud's phenomenon (50.0 % vs 13.3 %, p = 0.045). CONCLUSIONS: This study is the first to document capillaroscopic changes in alkaptonuria patients, supporting the hypothesis of microcirculation damage in this disease and raising the question of a possible role of capillaroscopy as a diagnostic, prognostic, and disease monitoring tool in this condition. CI - Copyright © 2025 Elsevier Inc. All rights reserved. FAU - Pereira da Costa, Roberto AU - Pereira da Costa R AD - Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. Electronic address: robertopc25@hotmail.com. FAU - Costa, Filipa AU - Costa F AD - Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Fonseca, João Eurico AU - Fonseca JE AD - Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Khmelinskii, Nikita AU - Khmelinskii N AD - Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Oliveira, Anabela AU - Oliveira A AD - Internal Medicine Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Inherited Metabolic Disease CAML Reference Center, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Dourado, Eduardo AU - Dourado E AD - Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Rheumatology Department, Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal; Aveiro Rheumatology Research Centre, Egas Moniz Health Alliance, Aveiro, Portugal. LA - eng PT - Journal Article DEP - 20251015 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Alkaptonuria/genetics/complications MH - Female MH - *Microscopic Angioscopy MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - Adult MH - Aged MH - Raynaud Disease MH - *Rheumatic Diseases/etiology MH - Capillaries/pathology OTO - NOTNLM OT - Alkaptonuria OT - Capillaroscopy OT - Metabolic disorder OT - Microcirculation OT - Rheumatology COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/10/23 00:26 MHDA- 2025/12/14 00:39 CRDT- 2025/10/22 18:05 PHST- 2025/07/23 00:00 [received] PHST- 2025/10/11 00:00 [revised] PHST- 2025/10/13 00:00 [accepted] PHST- 2025/12/14 00:39 [medline] PHST- 2025/10/23 00:26 [pubmed] PHST- 2025/10/22 18:05 [entrez] AID - S0049-0172(25)00227-6 [pii] AID - 10.1016/j.semarthrit.2025.152856 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2025 Dec;75:152856. doi: 10.1016/j.semarthrit.2025.152856. Epub 2025 Oct 15. PMID- 33025880 OWN - NLM STAT- MEDLINE DCOM- 20201027 LR - 20201027 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 38 Suppl 126 IP - 4 DP - 2020 Jul-Aug TI - Nailfold capillaroscopy in Sjögren's syndrome: a systematic literature review and standardised interpretation. PG - 150-157 AB - OBJECTIVES: To identify the role of nailfold capillaroscopy (NC) in Sjögren's syndrome (SS). METHODS: The literature was systematically reviewed in three databases. All published original studies which assess patients with SS by NC were revised. A quality assessment was applied to all studies based on population description, presence of a control group, presence of instrumental specifications and/or standardly applied NC methodology, presence of clear descriptions of capillaroscopic characteristics and based on the used statistical analysis. The capillaroscopic findings per study were described in a EULAR consented standardised way. Significant associations of capillaroscopic characteristics in SS patients with clinical and laboratory variables were summarised. RESULTS: The search resulted in 869 hits. Based on title and abstract screening 29 original studies were identified and of these, 14 full texts described an assessment by NC in SS. Seven studies were retained after performing a critical quality assessment. One study compared NC in SS with healthy controls and attested a lower capillary density in SS. Concerning clinical associations, capillary density was associated with Raynaud's phenomenon in two studies and with interstitial lung disease or systemic manifestations in one study each. No association between serologic features (anti-nuclear antibodies, anti-SSA, anti-SSB and anti-RF) and NC characteristics were found. CONCLUSIONS: A small number of studies have investigated the role of NC in SS. More studies, including prospective follow up studies with standard NC evaluation in SS are needed. FAU - Melsens, Karin AU - Melsens K AD - Department of Rheumatology, Ghent University Hospital, and Department of Internal Medicine, Ghent University, Belgium. karin.melsens@ugent.be. FAU - Leone, Maria C AU - Leone MC AD - Department of Rheumatology, Ghent University Hospital, Belgium, and Rheumatology Unit, Department of Internal Medicine, University of Perugia, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy. FAU - Elewaut, Dirk AU - Elewaut D AD - Department of Rheumatology, Ghent University Hospital, and Department of Internal Medicine, Ghent University, Belgium. FAU - Gerli, Roberto AU - Gerli R AD - Rheumatology Unit, Department of Internal Medicine, University of Perugia, Italy. FAU - Vanhaecke, Amber AU - Vanhaecke A AD - Department of Rheumatology, Ghent University Hospital, and Department of Internal Medicine, Ghent University, Belgium. FAU - Peene, Isabelle AU - Peene I AD - Department of Rheumatology, Ghent University Hospital, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University, and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium. LA - eng PT - Journal Article PT - Systematic Review DEP - 20200921 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Microscopic Angioscopy MH - Nails/diagnostic imaging MH - Prospective Studies MH - *Raynaud Disease MH - *Sjogren's Syndrome/diagnostic imaging EDAT- 2020/10/08 06:00 MHDA- 2020/10/28 06:00 CRDT- 2020/10/07 08:40 PHST- 2020/05/20 00:00 [received] PHST- 2020/07/13 00:00 [accepted] PHST- 2020/10/08 06:00 [pubmed] PHST- 2020/10/28 06:00 [medline] PHST- 2020/10/07 08:40 [entrez] AID - 15850 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2020 Jul-Aug;38 Suppl 126(4):150-157. Epub 2020 Sep 21. PMID- 24130266 OWN - NLM STAT- MEDLINE DCOM- 20150219 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 74 IP - 1 DP - 2015 Jan TI - Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS): report from a single VEDOSS/EUSTAR centre. PG - 124-8 LID - 10.1136/annrheumdis-2013-203889 [doi] AB - BACKGROUND: The oesophagus is the first gastrointestinal (GI) tract involved in systemic sclerosis (SSc), followed by the anorectum. OBJECTIVE: Evaluation of oesophageal and anorectal involvement and their correlations in patients with very early diagnosis of SSc (VEDOSS). PATIENTS AND METHODS: 59 patients with VEDOSS, evaluated with oesophageal and anorectal manometry and investigated with lung function tests and chest HRCT. Demographic data, oesophageal and anorectal symptoms, Raynaud's phenomenon, autoantibodies, videocapillaroscopy patterns, puffy fingers and digital ulcers were recorded for all patients. RESULTS: In 4 patients oesophageal manometry and in 17 patients anorectal manometry was not performed because of scarce tolerance. Oesophageal peristalsis was absent in 14 patients; its pressure and speed were significantly lower in 41 patients (p<0.001 and p=0.005, respectively). The maximum pressure and mean pressure (Pmax and Pm) of lower oesophageal sphincter were significantly lower (p=0.012 and p=0.024, respectively). Patients with a diffusing capacity of the lung for carbon monoxide<80% presented a hypotonic lower oesophageal sphincter (p=0.008) and an abnormal peristalsis (p<0.001); patients with a diffusing capacity of the lung for carbon monoxide>80% showed only an abnormal peristalsis (<0.001). The anal resting pressure (ARP) at 4.3 cm and 2 cm from anal edge and the anal canal Pm were significantly decreased (p<0.001 and p=0.010, respectively). The maximum voluntary contraction was significantly abnormal in its Pmax and Pm (p=0.017 and p=0.005) and in its duration (p=0.001). In patients with a positive HRCT, the ARP and the canal Pmax and Pm were significantly lower; patients with negative HRCT presented only an abnormal ARP. CONCLUSIONS: In patients with VEDOSS, oesophageal and anorectal disorders are frequently detected, showing that very early SSc is characterised by GI involvement. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. FAU - Lepri, Gemma AU - Lepri G AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Giani, Iacopo AU - Giani I AD - General Surgery, USL-8 Arezzo, Arezzo, Italy. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Blagojevic, Jelena AU - Blagojevic J AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Carnesecchi, Giulia AU - Carnesecchi G AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Radicati, Alessandra AU - Radicati A AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. FAU - Pucciani, Filippo AU - Pucciani F AD - Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. FAU - Marco, Matucci-Cerinic AU - Marco MC AD - Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. LA - eng PT - Journal Article DEP - 20131015 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Adult MH - Anal Canal/physiopathology MH - Anus Diseases/*diagnosis/etiology/physiopathology MH - Early Diagnosis MH - Esophageal Diseases/*diagnosis/etiology/physiopathology MH - Esophageal Sphincter, Lower/physiopathology MH - Female MH - Humans MH - Lung/*diagnostic imaging/physiopathology MH - Lung Diseases/*diagnosis/etiology/physiopathology MH - Male MH - Manometry MH - Microscopic Angioscopy MH - Middle Aged MH - Radiography MH - Raynaud Disease/etiology MH - Rectal Diseases/diagnosis/etiology/physiopathology MH - Respiratory Function Tests MH - Scleroderma, Systemic/complications/*diagnosis/physiopathology OTO - NOTNLM OT - Autoantibodies OT - Disease Activity OT - Pulmonary Fibrosis OT - Systemic Sclerosis EDAT- 2013/10/17 06:00 MHDA- 2015/02/20 06:00 CRDT- 2013/10/17 06:00 PHST- 2013/10/17 06:00 [entrez] PHST- 2013/10/17 06:00 [pubmed] PHST- 2015/02/20 06:00 [medline] AID - S0003-4967(24)09737-1 [pii] AID - 10.1136/annrheumdis-2013-203889 [doi] PST - ppublish SO - Ann Rheum Dis. 2015 Jan;74(1):124-8. doi: 10.1136/annrheumdis-2013-203889. Epub 2013 Oct 15. PMID- 38190091 OWN - NLM STAT- MEDLINE DCOM- 20240202 LR - 20240223 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 43 IP - 2 DP - 2024 Feb TI - Microvascular status in juvenile Sjögren's disease: the first nailfold videocapillaroscopy investigation. PG - 733-741 LID - 10.1007/s10067-023-06857-5 [doi] AB - INTRODUCTION: Juvenile Sjögren's disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud's phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. METHODS: Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. RESULTS: Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer's test). RP was present in 8% of jSjD patients. CONCLUSIONS: The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients. CI - © 2024. The Author(s). FAU - Lercara, Adriano AU - Lercara A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Malattia, Clara AU - Malattia C AD - Clinica Pediatrica E Reumatologia, IRCCS Istituto Giannina Gaslini, Genova, Italy. AD - Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal Infantile Sciences (DINOGMI), University of Genova, Genova, Italy. FAU - Hysa, Elvis AU - Hysa E AUID- ORCID: 0000-0002-6970-0983 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Gattorno, Marco AU - Gattorno M AD - Clinica Pediatrica E Reumatologia, IRCCS Istituto Giannina Gaslini, Genova, Italy. AD - Autoinflammatory Diseases and Immunodeficiencies Center, IRCCS Istituto Giannina Gaslini, Genova, Italy. FAU - Cere, Andrea AU - Cere A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Lavarello, Claudio AU - Lavarello C AD - Clinica Pediatrica E Reumatologia, IRCCS Istituto Giannina Gaslini, Genova, Italy. FAU - Vojinovic, Tamara AU - Vojinovic T AUID- ORCID: 0000-0003-1720-1151 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AUID- ORCID: 0000-0002-4732-0306 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Paolino, Sabrina AU - Paolino S AUID- ORCID: 0000-0001-9269-5089 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Sulli, Alberto AU - Sulli A AUID- ORCID: 0000-0003-3674-4880 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University Hospital, University of Ghent, Ghent, Belgium. AD - Department of Rheumatology, Ghent University Hospital, University of Ghent, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, Flemish Institute for Biotechnology, Inflammation Research Center, Ghent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 6, 16132, Genova, Italy. mcutolo@unige.it. AD - IRCCS Ospedale Policlinico San Martino, Genova, Italy. mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20240108 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Male MH - Adult MH - Female MH - Humans MH - Child MH - Adolescent MH - Young Adult MH - Microscopic Angioscopy/methods MH - Nails/blood supply MH - Capillaries/diagnostic imaging/pathology MH - *Autoimmune Diseases/pathology MH - *Sjogren's Syndrome/diagnostic imaging/pathology MH - *Raynaud Disease/pathology MH - *Scleroderma, Systemic/pathology PMC - PMC10834566 OTO - NOTNLM OT - Connective tissue diseases OT - Juvenile Sjögren’s disease OT - Microcirculation OT - Microvascular damage OT - Nailfold videocapillaroscopy OT - Pediatric rheumatology EDAT- 2024/01/08 12:43 MHDA- 2024/02/02 06:43 PMCR- 2024/01/08 CRDT- 2024/01/08 11:26 PHST- 2023/10/12 00:00 [received] PHST- 2023/12/21 00:00 [accepted] PHST- 2023/12/18 00:00 [revised] PHST- 2024/02/02 06:43 [medline] PHST- 2024/01/08 12:43 [pubmed] PHST- 2024/01/08 11:26 [entrez] PHST- 2024/01/08 00:00 [pmc-release] AID - 10.1007/s10067-023-06857-5 [pii] AID - 6857 [pii] AID - 10.1007/s10067-023-06857-5 [doi] PST - ppublish SO - Clin Rheumatol. 2024 Feb;43(2):733-741. doi: 10.1007/s10067-023-06857-5. Epub 2024 Jan 8. PMID- 22426123 OWN - NLM STAT- MEDLINE DCOM- 20120809 LR - 20220311 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 83 IP - 3 DP - 2012 May TI - Capillaroscopic pattern in inflammatory arthritis. PG - 318-22 LID - 10.1016/j.mvr.2012.03.002 [doi] AB - BACKGROUND: There are limited data about the role of nailfold capillaroscopy in inflammatory arthritis. OBJECTIVES: To study the role of capillaroscopy in inflammatory arthritis-rheumatoid arthritis (RA), psoriatic arthritis (PsA) and early arthritis. METHODS: Patients from the following groups were included in the study: 62 patients with RA; 34 patients with PsA with involvement of the joints of the hands; 9 women with early arthritis. Nailfold capillaroscopy was performed with videocapillaroscope. RESULTS: Raynaud's phenomenon (RP) was found in 30.6% (19/62) of RA patients, in 32.4% (11/34) of PsA patients and 44.4%, (4/9) of cases with early arthritis. The most frequent found capillaroscopic changes in RA patients were presence of elongated capillaries in 58% of cases (36/62) and prominent subpapillary plexus in 69% (43/62). Dilated capillaries were found in 78.9% (15/19) of patients with secondary RP and in 62.8% (27/43) of those without RP. "Scleroderma-like" capillaroscopic pattern was observed with low frequency in RA patients (14.5%/9/62). "Scleroderma-like" capillaroscopic pattern was also found in 11.1% (1/9) in the group of patients with early arthritis. The low frequency of the last type of capillaroscopic pattern in RA requires patients with such changes to be observed during regular follow-up for the development of systemic rheumatic disease different from inflammatory arthritis. In patients with PsA capillaries with specific morphology (tight terminal convolutions) were found in 58.8% (20/34) of cases. CONCLUSIONS: Results from the present study confirm the necessity for inclusion of the nailfold capillaroscopy in the diagnostic algorithm in patients with inflammatory arthritis. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Medical University, Plovdiv, Department for Propedeutics in Internal Medicine, Bulgaria. sevdalina_n@abv.bg FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120309 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - Arthritis/*physiopathology MH - Arthritis, Psoriatic/*physiopathology MH - Arthritis, Rheumatoid/*physiopathology MH - Capillaries/*physiopathology MH - Case-Control Studies MH - Female MH - Humans MH - Inflammation MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease PMC - PMC3332153 EDAT- 2012/03/20 06:00 MHDA- 2012/08/10 06:00 PMCR- 2012/05/01 CRDT- 2012/03/20 06:00 PHST- 2012/01/24 00:00 [received] PHST- 2012/03/01 00:00 [accepted] PHST- 2012/03/20 06:00 [entrez] PHST- 2012/03/20 06:00 [pubmed] PHST- 2012/08/10 06:00 [medline] PHST- 2012/05/01 00:00 [pmc-release] AID - S0026-2862(12)00055-6 [pii] AID - YMVRE3210 [pii] AID - 10.1016/j.mvr.2012.03.002 [doi] PST - ppublish SO - Microvasc Res. 2012 May;83(3):318-22. doi: 10.1016/j.mvr.2012.03.002. Epub 2012 Mar 9. PMID- 36040491 OWN - NLM STAT- MEDLINE DCOM- 20221011 LR - 20221014 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 42 IP - 12 DP - 2022 Dec TI - Microvascular capillaroscopic abnormalities and occurrence of antinuclear autoantibodies in patients with sarcoidosis. PG - 2199-2210 LID - 10.1007/s00296-022-05190-5 [doi] AB - We described nailfold videocapillaroscopy (NVC) findings and estimated the prevalence of serum anti-nuclear (ANA) and extractable nuclear antigen autoantibodies (ENA) in a cohort of sarcoidosis patients, comparing them with adequate healthy controls (HCs) and with primary Raynaud's phenomenon patients (PRPs). NVC findings were also correlated with the occurrence of autoantibodies, current treatment, laboratory parameters, variables of lung function and whole-body imaging data. Twenty-six patients with sarcoidosis were assessed through NVC, laboratory parameters, pulmonary function tests, chest-X ray and 18- fluorodeoxyglucose positron emission tomography/computed tomography. The NVC parameters and ANA/ENA dosage were recorded also in 30 PRPs and 30 HCs. Sarcoidosis patients showed a higher rate of capillary dilations and nonspecific abnormalities and a lower mean capillary absolute number than PRPs and HCs (p < 0.01 for all comparisons). The prevalence of ANA positivity was higher in patients with sarcoidosis compared with PRPs and HCs (p < 0.02 for both), whereas ENA positivity was detected in one sarcoidosis patient (Ro52). Among sarcoidosis patients, the mean capillary absolute number negatively correlated with the C-reactive protein concentrations and was positively associated with the forced vital capacity percentage. Instead, a negative correlation was detected between serum ACE levels and the presence of capillary dilations (all p < 0.05). Our findings suggest a microvascular involvement in sarcoidosis whose investigation by NVC might be useful for the follow-up of patients displaying RP. Autoantibody positivity in sarcoidosis might suggest autoimmune implications in the disease or the production of autoantibodies reactive to tissue damage. CI - © 2022. The Author(s). FAU - Cattelan, Francesco AU - Cattelan F AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Hysa, Elvis AU - Hysa E AUID- ORCID: 0000-0002-6970-0983 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AUID- ORCID: 0000-0002-4732-0306 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AUID- ORCID: 0000-0001-5125-0200 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Bica, Pietro Francesco AU - Bica PF AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Grosso, Marco AU - Grosso M AD - Pneumology Unit, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Barisione, Emanuela AU - Barisione E AUID- ORCID: 0000-0001-5365-134X AD - Pneumology Unit, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Paolino, Sabrina AU - Paolino S AUID- ORCID: 0000-0001-9269-5089 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Carmisciano, Luca AU - Carmisciano L AUID- ORCID: 0000-0001-6263-3864 AD - Department of Health Sciences, University of Genova, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AUID- ORCID: 0000-0003-3674-4880 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCSS San Martino Polyclinic, Viale Benedetto XV, 6, 16132, Genoa, Italy. mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20220830 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Antigens, Nuclear MH - Autoantibodies MH - C-Reactive Protein MH - Capillaries MH - Humans MH - Microscopic Angioscopy/methods MH - Nails/blood supply MH - *Raynaud Disease/epidemiology MH - *Sarcoidosis/diagnostic imaging MH - *Scleroderma, Systemic/diagnosis PMC - PMC9548476 OTO - NOTNLM OT - Antinuclear antibodies OT - Microcirculation OT - Microscopic angioscopy OT - Sarcoidosis COIS- All the co-authors take full responsibility for the accuracy and integrity of all aspects of the work. The authors declare no conflict of interest. EDAT- 2022/08/31 06:00 MHDA- 2022/10/12 06:00 PMCR- 2022/08/30 CRDT- 2022/08/30 11:15 PHST- 2022/06/27 00:00 [received] PHST- 2022/08/13 00:00 [accepted] PHST- 2022/08/31 06:00 [pubmed] PHST- 2022/10/12 06:00 [medline] PHST- 2022/08/30 11:15 [entrez] PHST- 2022/08/30 00:00 [pmc-release] AID - 10.1007/s00296-022-05190-5 [pii] AID - 5190 [pii] AID - 10.1007/s00296-022-05190-5 [doi] PST - ppublish SO - Rheumatol Int. 2022 Dec;42(12):2199-2210. doi: 10.1007/s00296-022-05190-5. Epub 2022 Aug 30. PMID- 32194200 OWN - NLM STAT- MEDLINE DCOM- 20200424 LR - 20210326 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 19 IP - 5 DP - 2020 May TI - Spanish scleroderma risk score (RESCLESCORE) to predict 15-year all-cause mortality in scleroderma patients at the time of diagnosis based on the RESCLE cohort: Derivation and internal validation. PG - 102507 LID - S1568-9972(20)30059-8 [pii] LID - 10.1016/j.autrev.2020.102507 [doi] AB - A few scores predicting the short-term risk of mortality in Systemic sclerosis (SSc) have been reported to date. Our study aimed to create a predictive 15-year all-cause mortality score at the time of the diagnosis of SSc. The study was based on the Spanish Scleroderma Registry (RESCLE). The cohort was split up in derivation (DC) and validation cohort (VC). A multivariate analysis to detect variables related to all-cause mortality within the first 15 years from SSc diagnosis was performed, assigning points to the rounded beta values to create the score (RESCLESCORE). 1935 SSc patients were included. The variables in the final model were as follows: age at diagnosis (+2 points > 65 years-old), male gender (+1 point), lcSSc subset (-1 point), mode of onset other than Raynaud's (+1 point), cancer (+1 point) and visceral involvement, such as ILD (+1 point), PAH (+1 point), heart (+1 point) and renal involvement (+2 points). Autoantibodies did not achieve statistical significance in the multivariate analysis. The 3 categories of risk to predict 15-year all-cause mortality at the time of diagnosis were as follows: low risk (5% vs. 7%, p = .189), intermediate risk (26.5% vs. 25.5%, p = .911) and high risk (47.8% vs. 59%, p = .316). The AUC was 0.799 (DC) vs. 0.778 (VC) (p = .530). In conclusion, the RESCLESCORE demonstrated an excellent ability to categorize SSc patients at the time of diagnosis in separate 15-year all-cause mortality risk strata at the time of diagnosis. CI - Copyright © 2020 Elsevier B.V. All rights reserved. FAU - Rubio-Rivas, Manuel AU - Rubio-Rivas M AD - Autoimmune Diseases Unit, Department of Internal Medicine, Bellvitge University Hospital-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. Electronic address: mrubio@bellvitgehospital.cat. FAU - Corbella, Xavier AU - Corbella X AD - Autoimmune Diseases Unit, Department of Internal Medicine, Bellvitge University Hospital-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain; Group of Evaluation of Health Determinants and Health Policies, Hestia Chair in Integrated Health and Social Care, Faculty of Medicine and Health Sciences, Universitat Internacional de Catalunya, Barcelona, Spain. FAU - Guillén-Del-Castillo, Alfredo AU - Guillén-Del-Castillo A AD - Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Tolosa Vilella, Carles AU - Tolosa Vilella C AD - Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain. FAU - Colunga Argüelles, Dolores AU - Colunga Argüelles D AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Argibay, Ana AU - Argibay A AD - Unit of Systemic Autoimmune Diseases and Thrombosis, Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Vigo, Pontevedra, Spain. FAU - Vargas Hitos, José Antonio AU - Vargas Hitos JA AD - Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain. FAU - Todolí Parra, José Antonio AU - Todolí Parra JA AD - Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain. FAU - González-Echávarri, Cristina AU - González-Echávarri C AD - Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Barakaldo, Spain. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Inst Invest Biosanitaria Ibs Granada, Department of Internal Medicine, Unit of Systemic Autoimmune Diseases, Department of Medicine, Facultad de Medicina, Hospital Universitario San Cecilio, Granada, Spain. FAU - Trapiella Martínez, Luis AU - Trapiella Martínez L AD - Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain. FAU - Rodríguez Carballeira, Mónica AU - Rodríguez Carballeira M AD - Department of Internal Medicine, Hospital Universitario Mútua Terrassa, Terrassa, Barcelona, Spain. FAU - Marín Ballvé, Adela AU - Marín Ballvé A AD - Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Pla Salas, Xavier AU - Pla Salas X AD - Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Consorci Hospitalari de Vic, Vic, Barcelona, Spain. FAU - Perales Fraile, Isabel AU - Perales Fraile I AD - Department of Internal Medicine, Hospital Universitario Rey Juan Carlos, Móstoles, Madrid, Spain. FAU - Chamorro, Antonio-J AU - Chamorro AJ AD - Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain. FAU - Madroñero Vuelta, Ana Belén AU - Madroñero Vuelta AB AD - Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain. FAU - Freire, Mayka AU - Freire M AD - Department of Internal Medicine, Hospital Clínico Universitario de Santiago, Santiago de Compostela, A Coruña, Spain. FAU - Ruiz Muñoz, Manuel AU - Ruiz Muñoz M AD - Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Alcorcón, Madrid, Spain. FAU - González García, Andrés AU - González García A AD - Department of Internal Medicine, Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Pons Martín Del Campo, Isaac AU - Pons Martín Del Campo I AD - Department of Internal Medicine, Xarxa Assistencial Universitària de Manresa, Manresa, Barcelona, Spain. FAU - Sánchez García, María Esther AU - Sánchez García ME AD - Department of Internal Medicine, Hospital Universitario Virgen de Valme, Sevilla, Spain. FAU - Bernal Bello, David AU - Bernal Bello D AD - Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Fuenlabrada, Madrid, Spain. FAU - Espinosa, Gerard AU - Espinosa G AD - Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain. FAU - García Hernández, Francisco José AU - García Hernández FJ AD - Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain. FAU - Sáez Comet, Luis AU - Sáez Comet L AD - Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain. FAU - Ríos Blanco, Juan José AU - Ríos Blanco JJ AD - Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain. FAU - Fernández de la Puebla Giménez, Rafael Ángel AU - Fernández de la Puebla Giménez RÁ AD - Department of Internal Medicine, Hospital Universitario Reina Sofía, Córdoba, Spain. FAU - Sánchez Trigo, Sabela AU - Sánchez Trigo S AD - Department of Internal Medicine, Complejo Hospitalario Universitario de Ferrol, Ferrol, A Coruña, Spain. FAU - Fonollosa Pla, Vicent AU - Fonollosa Pla V AD - Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Simeón Aznar, Carmen Pilar AU - Simeón Aznar CP AD - Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. CN - RESCLE investigators, Autoimmune Diseases Study Group (GEAS) LA - eng PT - Journal Article DEP - 20200317 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM EIN - Autoimmun Rev. 2021 May;20(5):102793. doi: 10.1016/j.autrev.2021.102793. PMID: 33766615 MH - Aged MH - *Cause of Death MH - Cohort Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/diagnosis/mortality MH - Registries MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis/*mortality MH - Spain/epidemiology OTO - NOTNLM OT - Mortality OT - Prognosis OT - Score OT - Systemic sclerosis COIS- Declaration of Competing Interest The authors declare no conflicts of interest. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. EDAT- 2020/03/21 06:00 MHDA- 2020/04/25 06:00 CRDT- 2020/03/21 06:00 PHST- 2019/12/12 00:00 [received] PHST- 2019/12/17 00:00 [accepted] PHST- 2020/03/21 06:00 [pubmed] PHST- 2020/04/25 06:00 [medline] PHST- 2020/03/21 06:00 [entrez] AID - S1568-9972(20)30059-8 [pii] AID - 10.1016/j.autrev.2020.102507 [doi] PST - ppublish SO - Autoimmun Rev. 2020 May;19(5):102507. doi: 10.1016/j.autrev.2020.102507. Epub 2020 Mar 17. PMID- 19361698 OWN - NLM STAT- MEDLINE DCOM- 20090729 LR - 20090413 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 136 IP - 4 DP - 2009 Apr TI - [Treatment of connective tissue disorder-related acral syndromes using UVA-1 phototherapy. An open study of 11 cases]. PG - 323-9 LID - 10.1016/j.annder.2008.12.022 [doi] AB - BACKGROUND: UVA-1 phototherapy is used to treat various connective tissues disorders including systemic lupus erythematosus (SLE) and systemic sclerosis (SS). We conducted an open study to investigate the efficacy of this therapy on connective tissue disorder-related acrosyndrome. MATERIAL AND METHODS: Eleven patients with Raynaud's phenomenon refractory to the standard therapy (six SLE and five SS) were treated with UVA-1 in an open study. Whole-body phototherapy was given in seven cases but was restricted to the hands in four cases. The results were evaluated by comparing status before and after treatment using semi-quantitative tools in terms of daily frequency and intensity of spastic phenomena, pain, overall patient satisfaction, improvement of cutaneous lesions, trophic disorders and distal cutaneous flexibility for patients with ScS. RESULTS: After treatment, Raynaud's phenomenon improved in terms of both frequency and severity in 9/11 patients (82%: 4/6 SS and 5/5 SLE). Likewise, pain decreased in 8/11 cases (73%: 3/6 SS and 5/5 SLE). 7/12 patients felt their condition had improved (64%: 3/6 SS and 4/5 SLE). Cutaneous lesions improved in 5/11 patients (45%: 2/6 SS and 3/5 SLE), especially for lupus-related lesions including chilblains and in idiopathic chilblains that totally subsided within one month. Digital ulcers improved in all cases, with complete healing in 3/4 patients (75%). In SS, cutaneous flexibility significantly improved in 2/6 cases (33%). No major adverse effects were seen in patients treated with hand-only phototherapy but a slight and spontaneously reversible cutaneous rash, reminiscent of lupus lesions, occurred in one female patient receiving whole-body phototherapy. DISCUSSION: This study is the first to provide a precise evaluation of the efficiency of UVA-1 phototherapy on connective tissue disorder-related acrosyndrome. This therapeutic effect is not necessarily restricted to the laboratory effects of UVA-1 since the favourable impact of infrared radiation and a placebo effect cannot be ruled out. Although its methodological limitations are obvious, our study also confirms dare data in the recent literature data by demonstrating significant improvement in cutaneous lesions, trophic ulcers and Raynaud's phenomenon in patients presenting connective tissue disorders, including SS, without any major adverse effects. CONCLUSION: Although these preliminary results remain to be confirmed by large-scale, randomized studies, UVA-1 phototherapy clearly offers a new and valuable therapeutic option in connective tissue disorders associated with acral manifestations and/or lesions, including SLE and SS. FAU - Comte, C AU - Comte C AD - Service de dermatologie, université Montpellier 1, hôpital Saint-Eloi, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France. FAU - Bessis, D AU - Bessis D FAU - Picot, E AU - Picot E FAU - Peyron, J-L AU - Peyron JL FAU - Guillot, B AU - Guillot B FAU - Dereure, O AU - Dereure O LA - fre PT - English Abstract PT - Journal Article TT - Traitement des acrosyndromes des connectivites par photothérapie UVA-1. Etude ouverte de 11 cas. DEP - 20090317 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Connective Tissue Diseases/*radiotherapy MH - Fingers MH - Hand MH - Humans MH - Phototherapy/*methods MH - Radiotherapy Dosage MH - Raynaud Disease/*radiotherapy MH - Scleroderma, Diffuse/pathology/radiotherapy MH - Toes MH - Treatment Outcome MH - Ultraviolet Therapy/*methods EDAT- 2009/04/14 09:00 MHDA- 2009/07/30 09:00 CRDT- 2009/04/14 09:00 PHST- 2008/03/31 00:00 [received] PHST- 2008/12/22 00:00 [accepted] PHST- 2009/04/14 09:00 [entrez] PHST- 2009/04/14 09:00 [pubmed] PHST- 2009/07/30 09:00 [medline] AID - S0151-9638(09)00050-7 [pii] AID - 10.1016/j.annder.2008.12.022 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2009 Apr;136(4):323-9. doi: 10.1016/j.annder.2008.12.022. Epub 2009 Mar 17. PMID- 36403140 OWN - NLM STAT- MEDLINE DCOM- 20221122 LR - 20230106 IS - 2242-3982 (Electronic) IS - 1239-9736 (Print) IS - 1239-9736 (Linking) VI - 81 IP - 1 DP - 2022 Dec TI - A prospective study on local cold injuries in northern Sweden. PG - 2149381 LID - 10.1080/22423982.2022.2149381 [doi] LID - 2149381 AB - The study aimed to determine the prevalence and incidence proportion of local cold injuries in northern Sweden, and identify associated factors. It was based on prospective data from surveys in 2015 and 2021 sent to a population-based sample in northern Sweden. Multiple binary logistic regression was performed. The study included 5,017 subjects (response rate 44.4%). The prevalence of cold injuries in the hands was 11.4%, feet 12.6%, and face 19.9%, while the incidence proportion was 1.0%, 1.0%, and 0.9%, respectively. Male gender was associated with incident cold injuries in the hands (OR 1.69; 95% CI 1.31-1.28), feet (OR 1.34; 95% CI 1.04-1.73), and face (OR 1.53; 95% CI 1.15-2.03); mental stress with cold injuries in the hands (OR 1.55; 95% CI 1.16-2.05) and feet (OR 1.39; 95% CI 1.04-1.88); previous stroke with cold injuries in the hands (OR 2.64; 95% CI 1.09-6.40) and face (OR 3.09; 95% CI 1.26-7.56); and Raynaud's phenomenon with cold injuries in the hands (OR 2.48; 95% CI 1.80-3.41) and feet (OR 2.07; 95% CI 1.50-2.87). We conclude that male gender, mental stress, previous stroke, and Raynaud's phenomenon increased the probability of contracting local cold injuries. FAU - Moen, Karolina AU - Moen K AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87 Umeå, Sweden. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87 Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Int J Circumpolar Health JT - International journal of circumpolar health JID - 9713056 SB - IM MH - Humans MH - Male MH - Prospective Studies MH - Sweden/epidemiology MH - *Raynaud Disease MH - *Cold Injury/epidemiology MH - *Stroke PMC - PMC9683046 OTO - NOTNLM OT - Cold exposure OT - Sweden OT - cold injury COIS- No potential conflict of interest was reported by the author(s). EDAT- 2022/11/21 06:00 MHDA- 2022/11/23 06:00 PMCR- 2022/11/20 CRDT- 2022/11/20 13:58 PHST- 2022/11/20 13:58 [entrez] PHST- 2022/11/21 06:00 [pubmed] PHST- 2022/11/23 06:00 [medline] PHST- 2022/11/20 00:00 [pmc-release] AID - 2149381 [pii] AID - 10.1080/22423982.2022.2149381 [doi] PST - ppublish SO - Int J Circumpolar Health. 2022 Dec;81(1):2149381. doi: 10.1080/22423982.2022.2149381. PMID- 31025932 OWN - NLM STAT- MEDLINE DCOM- 20191021 LR - 20191022 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 37 Suppl 119 IP - 4 DP - 2019 Jul-Aug TI - A new avenue in the pathogenesis of systemic sclerosis: the molecular interface between the endothelial and the nervous systems. PG - 133-140 AB - Systemic sclerosis (SSc) is a connective tissue disorder characterised by immune dysregulation, endothelial cell dysfunction followed by defective vascular repair and neovascularization and progressive tissue fibrosis of the skin and internal organs, whose pathophysiology remains to be fully elucidated. Perturbed neuroendothelial control mechanisms comprising either endothelial cell or peripheral nerve fiber impairment are supposed to play an important role in the onset of Raynaud's phenomenon and development of microvascular abnormalities which are the earliest events and key features of SSc. Such pathogenic neuroendothelial mechanisms may trigger both the early endothelial cell damage and the subsequent loss of peripheral microvascular integrity characterised by the lack of compensatory angiogenesis. Of note, the vascular and nervous systems have several anatomical similarities that extend to molecular level, and the molecular mechanisms of nerve regulation are shared by the vascular system. In this context, increasing evidence demonstrated that endothelial cells express receptors for axon guidance molecules, including Ephrin family receptor tyrosine kinases, Neuropilins, Plexins, Robos, and UNC5B that are able to respond to their soluble neuroendothelial trophic ligands, such as Semaphorins and Slits, to guide the sprouting of endothelial tip cells. Here, we first provide a historical view of neuroendothelial control mechanism alterations in the pathogenesis of SSc, and then discuss the emerging role of a class of molecules sharing neurogenic and angiogenic properties, such as members of Semaphorin/Plexin/Neuropilin and Slit/Roundabout families, in SSc-related peripheral microvasculopathy. FAU - Romano, Eloisa AU - Romano E AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy. eloisaromano@libero.it. FAU - Rosa, Irene AU - Rosa I AD - Division of Rheumatology, Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC) and Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Fioretto, Bianca Saveria AU - Fioretto BS AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy. FAU - Manetti, Mirko AU - Manetti M AD - Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Scleroderma Unit, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20190411 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Endothelial Cells MH - Humans MH - *Neovascularization, Pathologic MH - Nervous System MH - Peripheral Vascular Diseases/*physiopathology MH - *Raynaud Disease MH - *Scleroderma, Systemic/physiopathology EDAT- 2019/04/27 06:00 MHDA- 2019/10/23 06:00 CRDT- 2019/04/27 06:00 PHST- 2018/12/20 00:00 [received] PHST- 2019/03/11 00:00 [accepted] PHST- 2019/04/27 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] PHST- 2019/04/27 06:00 [entrez] AID - 13777 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2019 Jul-Aug;37 Suppl 119(4):133-140. Epub 2019 Apr 11. PMID- 31667644 OWN - NLM STAT- MEDLINE DCOM- 20201106 LR - 20240112 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 39 IP - 1 DP - 2020 Jan TI - Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma: outcomes from a multicenter US-based systemic sclerosis registry. PG - 93-102 LID - 10.1007/s10067-019-04792-y [doi] AB - The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5 years of first non-Raynaud's symptom. The data collection methodology incorporates successful models from other SSc registries. The cohort is designed to provide linked bio-specimen and clinical outcomes data on a longitudinal cohort of SSc patients for validation of hypothesis-driven research and to provide a platform for studying patient-reported outcomes in scleroderma. The CONQUER registry was developed using the guidelines of the International Society for Biological Repositories, and was an iterative process between physicians with an expertise in SSc, patient stakeholders, and information technology experts. Enrollment commenced in June 2018. During the first 6 months of the CONQUER Scleroderma study, 151 SSc patients with less than 5 years of disease duration (from first non-Raynaud's symptom) have been recruited. The mean age is 51 ± 14 years, 83% are female, and 60% of patients have diffuse disease. Survey completion rates are above 88% for all patient-reported outcome surveys. Bio-specimen collection rates are over 97%, and disease severity score completion rates are over 98%. Pulmonary function test data is available on 91% of patients, and echocardiography is available 80%. The CONQUER scleroderma study provides a unique and growing resource for studying scleroderma in a longitudinal, US-based population. KEY POINTS : • The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5 years of first non-Raynaud's symptom. • The CONQUER scleroderma study provides a unique and growing resource for studying scleroderma in a longitudinal, US-based population. • CONQUER is innovative in its design in that it is focused on prospective collection of paired clinical and patient outcome data with bio-specimens. FAU - Shanmugam, Victoria K AU - Shanmugam VK AUID- ORCID: 0000-0002-5882-4884 AD - Division of Rheumatology, The George Washington University School of Medicine and Health Sciences, 2300 M Street, NW, Room 307, Washington, DC, 20007, USA. vshanmugam@mfa.gwu.edu. FAU - Frech, Tracy M AU - Frech TM AD - Division of Rheumatology, Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Salt Lake City, UT, USA. FAU - Steen, Virginia D AU - Steen VD AD - Division of Rheumatology, Georgetown University Medical Center, Washington, DC, USA. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Bernstein, Elana J AU - Bernstein EJ AD - Division of Rheumatology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, USA. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. FAU - Gordon, Jessica K AU - Gordon JK AD - Division of Rheumatology, Hospital for Special Surgery, New York, NY, USA. FAU - Castelino, Flavia V AU - Castelino FV AD - Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine and Palo Alto Veterans Affairs Health Care System, Palo Alto, CA, USA. FAU - Hant, Faye N AU - Hant FN AD - Division of Rheumatology and Immunology, Medical University of South Carolina, Charleson, SC, USA. FAU - Startup, Emily AU - Startup E AD - Department of Pediatrics, University of Utah, Salt Lake City, UT, USA. FAU - VanBuren, John M AU - VanBuren JM AD - Department of Pediatrics, University of Utah, Salt Lake City, UT, USA. FAU - Evnin, Luke B AU - Evnin LB AD - Scleroderma Research Foundation, San Francisco, CA, USA. FAU - Assassi, Shervin AU - Assassi S AD - Division of Rheumatology, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, TX, USA. LA - eng GR - K24AR063120/AR/NIAMS NIH HHS/United States GR - R01 AR073208/AR/NIAMS NIH HHS/United States GR - I01 CX001183/CX/CSRD VA/United States GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - R01AR073208/AR/NIAMS NIH HHS/United States GR - K23 AR075112/AR/NIAMS NIH HHS/United States GR - K23AR067889/AR/NIAMS NIH HHS/United States GR - K23 AR067889/AR/NIAMS NIH HHS/United States GR - K23AR075112/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study DEP - 20191030 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Databases, Factual MH - Female MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - *Patient Reported Outcome Measures MH - Raynaud Disease/physiopathology MH - *Registries MH - Respiratory Function Tests MH - Scleroderma, Systemic/*physiopathology MH - Self Report MH - Severity of Illness Index MH - United States MH - Young Adult PMC - PMC7280746 MID - NIHMS1583772 OTO - NOTNLM OT - Bio-specimens OT - Cohort study OT - Patient-reported outcomes OT - Prospective study OT - Registry OT - Systemic sclerosis EDAT- 2019/11/02 06:00 MHDA- 2020/11/11 06:00 PMCR- 2020/09/01 CRDT- 2019/11/01 06:00 PHST- 2019/08/17 00:00 [received] PHST- 2019/09/21 00:00 [accepted] PHST- 2019/09/15 00:00 [revised] PHST- 2019/11/02 06:00 [pubmed] PHST- 2020/11/11 06:00 [medline] PHST- 2019/11/01 06:00 [entrez] PHST- 2020/09/01 00:00 [pmc-release] AID - 10.1007/s10067-019-04792-y [pii] AID - 10.1007/s10067-019-04792-y [doi] PST - ppublish SO - Clin Rheumatol. 2020 Jan;39(1):93-102. doi: 10.1007/s10067-019-04792-y. Epub 2019 Oct 30. PMID- 40087926 OWN - NLM STAT- MEDLINE DCOM- 20250513 LR - 20250513 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 73 IP - 3 DP - 2025 Mar TI - Spectrum of Clinical Manifestations in Patients of Scleroderma and Correlation between Cutaneous and Pulmonary Involvement. PG - 17-20 LID - 10.59556/japi.73.0855 [doi] AB - PURPOSE: Our research aimed at characterizing the range of signs and symptoms observed in scleroderma patients at a tertiary care hospital and investigating potential correlations between pulmonary and cutaneous involvement. MATERIALS AND METHODS: We obtained informed consent from scleroderma patients for conducting a comprehensive clinical assessment, including detailed medical histories and physical examinations. Standard diagnostic investigations like complete blood counts, erythrocyte sedimentation rate, renal function, liver function, electrocardiograms (ECG), high-resolution computed tomography (HRCT) scans, and pulmonary function tests were performed. The modified Rodnan skin score (mRSS) was used to assess skin involvement. RESULTS: About 74 (71.2%) patients had diffuse cutaneous systemic scleroderma (dcSSc), while 30 (28.8%) patients had limited cutaneous scleroderma (lcSSc). 96 (92.3%) patients had Raynaud's phenomenon. About 68 (65.4%) patients had mild mRSS, while 26 (25%) and 10 (9.6%) patients had moderate and severe mRSS, respectively. Skin tightening (88.4%) and sclerodactyly (88.4%) were the most common cutaneous manifestations, followed by digital ulcers and pits (57.7%), diffuse edema of hands and feet (38.4%), salt-and-pepper skin (38.4%), calcinosis (30.8%), telangiectasia (25%), and contractures (19.2%). Pulmonary manifestations showed interstitial lung disease (ILD) in 62 (59.6%) patients and pulmonary hypertension (PH) in 14 (13.5%) patients. 8 (7.7%) patients had ILD with PH. 48 (46.2%) and 18 (17.3%) patients with dcSSc had mild and moderate mRSS, respectively, while 8 (7.7%) patients had severe mRSS. About 20 (19.2%) and 8 (7.7%) patients with lcSSc had mild and moderate mRSS, respectively, while 2 (1.9%) patients had severe mRSS. There was no significant correlation of mRSS and subtypes of scleroderma patients. The mean mRSS score in ILD was low in comparison to the mRSS score in patients with PH (25.7 ± 8.90 vs 28.9 ± 7.62). There was a significant correlation of mRSS and pulmonary involvement as indicated by the Student's t-test (p < 0.05). CONCLUSION: In systemic sclerosis (SSc) patients, the emergence of severe systemic complications such as pulmonary arterial hypertension and ILD can manifest regardless of disease duration or subtype. Symptoms might not consistently align with disease advancement. Therefore, a thorough evaluation that incorporates symptoms and specialized diagnostic tests, such as pulmonary function tests, echocardiography (ECHO), and HRCT, is crucial for early identification, timely treatment, and better prognosis. The mRSS serves as a valuable clinical instrument for monitoring scleroderma progression. CI - © Journal of The Association of Physicians of India 2025. FAU - Oak, Jyotsna AU - Oak J AD - Head of the Department, General Medicine, and Consultant in Rheumatology, Department of Rheumatology, Mumbai, Maharashtra, India, Corresponding Author. FAU - Unavane, Ojas AU - Unavane O AD - DNB Resident, Department of General Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India. FAU - Mathur, Rupali AU - Mathur R AD - DNB Resident, Department of General Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India. LA - eng PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Humans MH - Female MH - Male MH - Adult MH - Middle Aged MH - *Scleroderma, Systemic/complications/diagnosis MH - Aged MH - Raynaud Disease/etiology MH - *Lung Diseases/etiology MH - Young Adult MH - *Scleroderma, Diffuse/complications MH - Lung Diseases, Interstitial/etiology MH - Adolescent MH - Respiratory Function Tests EDAT- 2025/03/16 11:14 MHDA- 2025/03/16 11:15 CRDT- 2025/03/15 03:55 PHST- 2025/03/16 11:15 [medline] PHST- 2025/03/16 11:14 [pubmed] PHST- 2025/03/15 03:55 [entrez] AID - 10.59556/japi.73.0855 [doi] PST - ppublish SO - J Assoc Physicians India. 2025 Mar;73(3):17-20. doi: 10.59556/japi.73.0855. PMID- 40178987 OWN - NLM STAT- MEDLINE DCOM- 20250701 LR - 20260525 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - 7 DP - 2025 Jul 1 TI - Risk factors of digital gangrene secondary to systemic lupus erythematosus flare: real-world data from 2014 to 2022. PG - 4245-4256 LID - 10.1093/rheumatology/keaf123 [doi] AB - OBJECTIVES: To seek risk factors of gangrene secondary to systemic lupus erythematosus (SLE). METHODS: We reviewed 31 SLE patients who developed gangrene, following them up for over 2 years. A control group of 93 lupus patients without gangrene matched for SLEDAI-2000 scores was also included. Additionally, 93 age- and sex-matched healthy volunteers were recruited as a healthy control group. RESULTS: Thirty-one out of 3021 (1.03%) SLE patients with gangrene were recorded. The average age at event was 32.23 ± 18.77 years and the average disease duration was 80.23 ± 260.54 months. Gangrene, as the first manifestation of new onset of SLE, was found in 16 patients. Logistic regression analysis indicated that digital ulceration (OR = 8.56, 95% CI: 2.7 4-26.72), Raynaud's phenomenon (OR = 13.45, 95% CI: 2.4 0-75.40), thrombotic microangiopathy (TMA) (OR = 12.24, 95% CI: 1.0 6-141.90), elevated serum C-reactive protein (CRP) (OR = 1.01, 95% CI: 1.0 0-1.03) and declined natural killer (NK) cells (OR = 0.96, 95% CI: 0.9 4-0.99) (P all <0.011) significantly influenced the occurrence of gangrene. During two-year follow-up, nearly half of the gangrene patients (48.4%) experienced amputation. Subgroup analyses revealed that infections secondary to gangrene significantly increased the risk of amputation (P = 0.046). Regarding the follow-up, only three patients experienced a second or subsequent episode of gangrene. CONCLUSION: Our study establishes a close connection between declined NK cells and the pathogenesis of gangrene. The presence of digital ulceration and/or Raynaud's phenomenon and TMA, along with elevated serum CRP levels, increases the likelihood of gangrene, potentially serving as gangrene predictors in SLE. Infections secondary to gangrene further elevate the risk of amputation. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Liu, Yang AU - Liu Y AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Li, Qian AU - Li Q AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Qiao, Pengyan AU - Qiao P AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Liu, Ying AU - Liu Y AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Gao, Wenqin AU - Gao W AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Yang, Yanli AU - Yang Y AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Liu, Sumiao AU - Liu S AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. FAU - Xu, Ke AU - Xu K AD - Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Third Hospital of Shanxi Medical University, Tongji Shanxi Hospital, Taiyuan, China. LA - eng GR - 81871292/National Natural Science Foundation/ GR - Key Research and Development/ GR - 201803D31136/Projects of Shanxi Province/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Humans MH - *Gangrene/etiology/epidemiology MH - *Lupus Erythematosus, Systemic/complications MH - Female MH - Male MH - Adult MH - Risk Factors MH - *Fingers/pathology MH - Middle Aged MH - Raynaud Disease/complications MH - Retrospective Studies MH - Young Adult MH - Case-Control Studies MH - C-Reactive Protein/analysis/metabolism MH - Toes OTO - NOTNLM OT - NK cell OT - SLEDAI-2K scores OT - TMA OT - gangrene OT - systemic lupus erythematosus (SLE) EDAT- 2025/04/03 18:25 MHDA- 2025/07/02 00:27 CRDT- 2025/04/03 12:43 PHST- 2024/10/02 00:00 [received] PHST- 2025/02/16 00:00 [accepted] PHST- 2025/07/02 00:27 [medline] PHST- 2025/04/03 18:25 [pubmed] PHST- 2025/04/03 12:43 [entrez] AID - 8105567 [pii] AID - 10.1093/rheumatology/keaf123 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Jul 1;64(7):4245-4256. doi: 10.1093/rheumatology/keaf123. PMID- 19528045 OWN - NLM STAT- MEDLINE DCOM- 20100225 LR - 20220408 IS - 1470-7926 (Electronic) IS - 1351-0711 (Linking) VI - 67 IP - 1 DP - 2010 Jan TI - A prospective cohort study of exposure-response relationship for vibration-induced white finger. PG - 38-46 LID - 10.1136/oem.2009.046128 [doi] AB - OBJECTIVES: To investigate prospectively the relation between vibration-induced white finger (VWF) and measures of cumulative (lifetime) exposure to hand-transmitted vibration (HTV). METHODS: Two hundred and forty-nine HTV workers and 138 control men of the same companies participated in a 3-year follow-up study. The diagnosis of VWF (Raynaud's phenomenon in the controls) was based on the medical history, the administration of colour charts and the results of a cold test. Tool vibration magnitudes were expressed as root-mean-square (r.m.s.) acceleration, frequency-weighted according to international standard ISO 5349-1 and also unweighted over the frequency range 6.3-1250 Hz. From the vibration magnitudes and exposure durations, alternative measures of cumulative vibration dose were calculated for each HTV worker, according to the expression: dose = Sigmaa(i)(m)t(i), where a(i) is the acceleration magnitude on tool i, t(i) is the lifetime exposure duration (hours) for tool i, and m = 0, 1, 2 or 4. RESULTS: The incidence of VWF varied from 5 to 6% in the HTV workers versus 0 to 1.5% for Raynaud's phenomenon in the controls. After adjusting for potential confounders, measures of cumulative vibration dose derived from total operating hours and high powers of unweighted acceleration (ie, , with m>1) gave better predictions of the occurrence of VWF than dose measures calculated from frequency-weighted acceleration (ie, ). These findings were observed in the entire sample of HTV workers, in those with no VWF at the initial investigation, and in those with normal cold test results at baseline. CONCLUSIONS: This prospective cohort study suggests that measures of cumulative vibration doses constructed from unweighted r.m.s. acceleration perform better for the prediction of VWF than dose measures calculated according to the recommendations of current standards. These findings should contribute to the improvement of the ISO frequency weighting for evaluating the severity of hand-transmitted vibration. FAU - Bovenzi, M AU - Bovenzi M AD - Unità Clinica Operativa di Medicina del Lavoro, Dipartimento di Scienze di Medicina Pubblica, Azienda Ospedaliero-Universitaria "Ospedali Riuniti di Trieste", Università di Trieste, Centro Tumori, Via della Pietà 19, I-34129 Trieste, Italy. bovenzi@units.it LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20090614 PL - England TA - Occup Environ Med JT - Occupational and environmental medicine JID - 9422759 SB - IM MH - Adult MH - Cold Temperature MH - Epidemiologic Methods MH - Fingers/*blood supply MH - Forestry MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*epidemiology MH - Occupational Exposure/*adverse effects/statistics & numerical data MH - Raynaud Disease/*epidemiology MH - Time Factors MH - Vibration/*adverse effects EDAT- 2009/06/17 09:00 MHDA- 2010/02/26 06:00 CRDT- 2009/06/17 09:00 PHST- 2009/06/17 09:00 [entrez] PHST- 2009/06/17 09:00 [pubmed] PHST- 2010/02/26 06:00 [medline] AID - oem.2009.046128 [pii] AID - 10.1136/oem.2009.046128 [doi] PST - ppublish SO - Occup Environ Med. 2010 Jan;67(1):38-46. doi: 10.1136/oem.2009.046128. Epub 2009 Jun 14. PMID- 16399843 OWN - NLM STAT- MEDLINE DCOM- 20060804 LR - 20161128 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 IP - 6 DP - 2006 Jun TI - Abnormal nitric oxide metabolism in systemic sclerosis: increased levels of nitrated proteins and asymmetric dimethylarginine. PG - 676-84 AB - OBJECTIVES: Endothelial dysfunction is a primary event in systemic sclerosis; however, the aetiology of events and the role of nitric oxide (NO) is still unclear. The aim of the present study is to investigate whether there are abnormalities in NO metabolism in plasma from patients with primary Raynaud's phenomenon (RP) and in the pathogenesis of systemic sclerosis (SSc): limited SSc (lSSc) and diffuse (dSSc). We also wanted to investigate the effect of factors within patients' SSc serum on NO metabolism in human microvascular endothelial cells (HMECs). METHODS: Plasma (n=89) or serum (n=80) was assayed for total nitrate and nitrite (NOx), nitration of proteins and the NO inhibitor asymmetric dimethylarginine (ADMA). HMECs were treated with patients' SSc serum and assayed for indicators of NO metabolism. RESULTS: Plasma NOx was elevated in patients with RP or lSSc (P<0.002), but not in patients with dSSc, compared with controls. Nitrated proteins in plasma, however, were found to be very high in dSSc patients (P<0.03), compared with RP, lSSc or controls. Patients with dSSc also showed increased levels of serum ADMA (P<0.05). The high level of nitrated proteins in dSSc was strongly associated with the severity and duration of dSSc disease. Skin biopsy sections from dSSc patients also showed enhanced nitrotyrosine staining compared with controls. In HMECs, pre-incubation with SSc serum impaired the activity of nitric oxide synthase (NOS) but not the expression of inducible or endothelial NOS. SSc serum also induced a reduction in intracellular cGMP synthesis, and NOx production in the cell culture medium, but was not associated with increased cell cytotoxicity. CONCLUSIONS: NO formation is increased in patients with primary RP or lSSc, but nitration of proteins and elevated ADMA is a particular feature of dSSc and may reflect abnormal NO regulation and/or contribute to endothelial dysfunction in SSc. FAU - Dooley, A AU - Dooley A AD - Department of Biochemistry and Molecular Biology, Royal Free Campus, University College Medical School, London NW3 2PF, UK. FAU - Gao, B AU - Gao B FAU - Bradley, N AU - Bradley N FAU - Abraham, D J AU - Abraham DJ FAU - Black, C M AU - Black CM FAU - Jacobs, M AU - Jacobs M FAU - Bruckdorfer, K R AU - Bruckdorfer KR LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060106 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Enzyme Inhibitors) RN - 0 (Nitrates) RN - 0 (Nitrites) RN - 31C4KY9ESH (Nitric Oxide) RN - 63CV1GEK3Y (N,N-dimethylarginine) RN - 94ZLA3W45F (Arginine) RN - EC 1.14.13.39 (Nitric Oxide Synthase) SB - IM MH - Adult MH - Arginine/*analogs & derivatives/blood MH - Cells, Cultured MH - Endothelium, Vascular/metabolism MH - Enzyme Inhibitors/blood MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nitrates/blood MH - Nitric Oxide/*blood/metabolism MH - Nitric Oxide Synthase/antagonists & inhibitors/metabolism MH - Nitrites/blood MH - Raynaud Disease/blood MH - Scleroderma, Diffuse/blood MH - Scleroderma, Limited/blood MH - Scleroderma, Systemic/*blood MH - Severity of Illness Index EDAT- 2006/01/10 09:00 MHDA- 2006/08/05 09:00 CRDT- 2006/01/10 09:00 PHST- 2006/01/10 09:00 [pubmed] PHST- 2006/08/05 09:00 [medline] PHST- 2006/01/10 09:00 [entrez] AID - kei276 [pii] AID - 10.1093/rheumatology/kei276 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Jun;45(6):676-84. doi: 10.1093/rheumatology/kei276. Epub 2006 Jan 6. PMID- 29566759 OWN - NLM STAT- MEDLINE DCOM- 20180717 LR - 20181114 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 16 IP - 1 DP - 2018 Mar 23 TI - Impairment of microcirculation and vascular responsiveness in adolescents with primary Raynaud phenomenon. PG - 20 LID - 10.1186/s12969-018-0237-x [doi] LID - 20 AB - BACKGROUND: Raynaud's phenomenon (RP) is a functional vascular disease, presenting with recurrent episodes of ischemia of extremities in response to cold and emotional stress. Investigating cutaneous microcirculation is an important tool in understanding the complex neuro-immuno-vascular interactions in its pathophysiological mechanisms. Since there is no available data on vascular responsiveness in RP in the paediatric population, we investigated skin perfusion and heat-induced hyperaemia in comparison with clinical severity and laboratory parameters of the disease. METHODS: Fifty two adolescents (27 patients with primary RP and 25 age-matched healthy controls) were investigated in the study. Patients were divided into two groups according to the symptoms existing within the previous 2 months. Following baseline microcirculation measurement with Laser Doppler flowmetry (Periflux 5000 system), all subjects underwent local heating test at 42 °C and 44 °C. Besides routine laboratory parameters, immune-serological tests and the vasoactive sensory neuropeptides somatostatin and pituitary adenylate-cyclase activating polypeptide (PACAP) were measured. RESULTS: Baseline perfusion measured in perfusion units (PU) at 32 °C was significantly lower in symptomatic RP patients (97.6 ± 22.4 PU) compared with both healthy volunteers (248.3 ± 23.5 PU, p < 0.001) and RP patients without symptoms (187.4 ± 24.9 PU, p < 0.05). After local heating to 42 °C maximum blood flow was significantly reduced in primary RP participants with current symptoms (358.6 ± 43.9 PU, p < 0.001), but not in asymptomatic ones (482.3 ± 28.7 PU, p > 0.05) when compared with healthy subjects (555.9 ± 28.2 PU). Both the area under the response curve and the latency to reach the maximum flow were significantly increased in both RP groups (symptomatic 164.6 ± 7.4 s, p < 0.001, asymptomatic 236.4 ± 17.4 s, p < 0.001) when compared with the control group (101.9 ± 4.7 s). The heat-induced percentage increase from baseline to maximal blood flow was significantly greater in symptomatic RP adolescents in comparison with healthy ones. Laboratory parameters and neuropeptide plasma levels were not altered in any groups. CONCLUSION: To our knowledge this is the first study in paediatric population to show altered heat-induced cutaneous hyperaemia responses in relation with the clinical severity and symptomatology. FAU - Mosdósi, Bernadett AU - Mosdósi B AD - Clinical Center, Department of Pediatrics, University of Pécs, József Attila u. 7, Pécs, H-7623, Hungary. mosdosi.bernadett@pte.hu. FAU - Bölcskei, Kata AU - Bölcskei K AD - János Szentágothai Research Centre & Centre for Neuroscience, University of Pécs, Ifjúság útja 20, Pécs, H-7624, Hungary. AD - Medical School, Department of Pharmacology and Pharmacotherapy, University of Pécs, Szigeti út 12, Pécs, H-7624, Hungary. FAU - Helyes, Zsuzsanna AU - Helyes Z AD - János Szentágothai Research Centre & Centre for Neuroscience, University of Pécs, Ifjúság útja 20, Pécs, H-7624, Hungary. AD - Medical School, Department of Pharmacology and Pharmacotherapy, University of Pécs, Szigeti út 12, Pécs, H-7624, Hungary. LA - eng GR - GINOP-2.3.2.-15-2016-00048/Gazdaságfejlesztési és Innovációs Operatív Program/ GR - EFOP-3.6.1-16-2016-00004/Emberi Erőforrás Fejlesztési Operatív Program/ PT - Journal Article DEP - 20180323 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM MH - Adolescent MH - Area Under Curve MH - Female MH - Humans MH - Hyperemia/physiopathology MH - Laser-Doppler Flowmetry/methods MH - Male MH - Microcirculation/*physiology MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Skin/*blood supply/physiopathology MH - Young Adult PMC - PMC5865297 OTO - NOTNLM OT - Adolescents OT - Laser Doppler OT - Local hyperaemia OT - Microcirculation OT - Primary Raynaud phenomenon COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: The study was approved by The Regional Research Ethics Committee of the Medical Center, University of Pécs (5501/2015). Written informed consents were obtained from the adolescents and their parents for the participation. CONSENT FOR PUBLICATION: Written informed consents were obtained from the patients’ parents for publication of this study. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2018/03/24 06:00 MHDA- 2018/07/18 06:00 PMCR- 2018/03/23 CRDT- 2018/03/24 06:00 PHST- 2018/03/07 00:00 [received] PHST- 2018/03/12 00:00 [accepted] PHST- 2018/03/24 06:00 [entrez] PHST- 2018/03/24 06:00 [pubmed] PHST- 2018/07/18 06:00 [medline] PHST- 2018/03/23 00:00 [pmc-release] AID - 10.1186/s12969-018-0237-x [pii] AID - 237 [pii] AID - 10.1186/s12969-018-0237-x [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2018 Mar 23;16(1):20. doi: 10.1186/s12969-018-0237-x. PMID- 37140670 OWN - NLM STAT- MEDLINE DCOM- 20230804 LR - 20230804 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 41 IP - 8 DP - 2023 Aug TI - External clinical validation of automated software to identify structural abnormalities and microhaemorrhages in nailfold videocapillaroscopy images. PG - 1605-1611 LID - 10.55563/clinexprheumatol/m6obl3 [doi] AB - OBJECTIVES: Automated systems to analyse nailfold videocapillaroscopy (NVC) images are needed to promptly and comprehensively characterise patients with systemic sclerosis (SSc) or Raynaud's phenomenon (RP). We previously developed, and validated in-house, a deep convolutional neural network-based algorithm to classify NVC-captured images according to the presence/absence of structural abnormalities and/or microhaemorrhages. We present its external clinical validation. METHODS: A total of 1,164 NVC images of RP patients were annotated by 5 trained capillaroscopists according to the following categories: normal capillary; dilation; giant capillary; abnormal shape; tortuosity; microhaemorrhage. The images were also presented to the algorithm. Matches and discrepancies between algorithm predictions and those annotations obtained by consensus of ≥3 or ≥4 interobservers were analysed. RESULTS: Consensus among ≥3 capillaroscopists was achieved in 86.9% of images, 75.8% of which were correctly predicted by the algorithm. Consensus among ≥4 experts occurred in 52.0% of cases, in which 87.1% of the algorithm's results matched with those of the expert panel. The algorithm's positive predictive value was >80% for microhaemorrhages and unaltered, giant or abnormal capillaries. Sensitivity was >75% for dilations and tortuosities. Negative predictive value and specificity were >89% for all categories. CONCLUSIONS: This external clinical validation suggests that this algorithm is useful to assist in the diagnosis and follow-up of SSc or RP patients in a timely manner. It may also be helpful in the management of patients with any pathology presenting with microvascular changes, as the algorithm has been designed to also be useful for research aiming at extending the usage of nailfold capillaroscopy to more conditions. FAU - Gracia Tello, Borja C AU - Gracia Tello BC AD - Unit of Autoimmune Diseases, Lozano Blesa University Hospital, Zaragoza, and Instituto de Investigación Sanitaria Aragón (ISSA), Aragón, Spain. bcgracia@salud.aragon.es. FAU - Ramos Ibañez, Eduardo AU - Ramos Ibañez E AD - Software Engineer, Computer Science Graduate, University of Zaragoza, Spain. FAU - Saez Comet, Luis AU - Saez Comet L AD - Autoimmune Department, Hospital Universitario Miguel Servet, Zaragoza, and Sociedad Española de Medicina Interna, Grupo de Enfermedades Autoinmunes Sistémicas (GEAS), Spain. FAU - Guillén Del Castillo, Alfredo AU - Guillén Del Castillo A AD - Unit of Autoimmune Diseases, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Simeón Aznar, Carmen Pilar AU - Simeón Aznar CP AD - Unit of Autoimmune Diseases, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AD - Unit of Autoimmune Diseases, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Espinosa, Gerard AU - Espinosa G AD - Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain. FAU - Lledó, Gema AU - Lledó G AD - Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain. FAU - Freire Dapena, Mayka AU - Freire Dapena M AD - Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Spain. FAU - Martinez Robles, Elena AU - Martinez Robles E AD - Unit of Autoimmune Diseases, Hospital Universitario La Paz, Madrid, Spain. FAU - Ríos, Juan José AU - Ríos JJ AD - Unit of Autoimmune Diseases, Hospital Universitario La Paz, Madrid, Spain. FAU - Todolí Parra, José Antonio AU - Todolí Parra JA AD - Unit of Autoimmune Diseases, Hospital Universitario y Politécnico La Fe, Valencia, Spain. FAU - Marí Alfonso, Begoña AU - Marí Alfonso B AD - Internal Medicine, Hospital Parc Tauli, Barcelona, Spain. FAU - Ortego Centeno, Norberto AU - Ortego Centeno N AD - Unit of Autoimmune Diseases, Hospital Clínico San Cecilio, Granada, Spain. FAU - Marín Ballvé, Adela AU - Marín Ballvé A AD - Unit of Autoimmune Diseases, Lozano Blesa University Hospital, Zaragoza, and Instituto de Investigación Sanitaria Aragón (ISSA), Aragón, Spain. FAU - Callejas Rubio, José Luis AU - Callejas Rubio JL AD - Unit of Autoimmune Diseases, Hospital Clínico San Cecilio, Granada, Spain. FAU - Fonollosa Plá, Vicent AU - Fonollosa Plá V AD - Unit of Autoimmune Diseases, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Fanlo, Patricia AU - Fanlo P AD - Unit of Autoimmune Diseases, Hospital Virgen del Camino, Pamplona, and Sociedad Española Multidisciplinar de Enfermedades Autoinmunes Sistémicas (SEMAIS), Spain. LA - eng PT - Journal Article DEP - 20230504 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Microscopic Angioscopy/methods MH - Nails/blood supply MH - *Scleroderma, Systemic/diagnostic imaging/pathology MH - *Raynaud Disease/diagnostic imaging MH - Software MH - Capillaries/diagnostic imaging/pathology EDAT- 2023/05/04 12:41 MHDA- 2023/08/04 06:43 CRDT- 2023/05/04 11:07 PHST- 2022/12/01 00:00 [received] PHST- 2023/03/02 00:00 [accepted] PHST- 2023/08/04 06:43 [medline] PHST- 2023/05/04 12:41 [pubmed] PHST- 2023/05/04 11:07 [entrez] AID - 19447 [pii] AID - 10.55563/clinexprheumatol/m6obl3 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2023 Aug;41(8):1605-1611. doi: 10.55563/clinexprheumatol/m6obl3. Epub 2023 May 4. PMID- 41106412 OWN - NLM STAT- MEDLINE DCOM- 20251119 LR - 20251119 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 7 IP - 12 DP - 2025 Dec TI - Exploiting a unified vascular framework to predict organ-specific complications and accomplish disease modification in systemic sclerosis. PG - e895-e906 LID - S2665-9913(25)00251-6 [pii] LID - 10.1016/S2665-9913(25)00251-6 [doi] AB - Immune-mediated vascular endothelial injury is considered one of the earliest pathological features in systemic sclerosis and is thought to occur simultaneously within a broad range of organs, although typically clinically manifesting only as Raynaud's phenomenon in the early stages. Overt vascular systemic sclerosis manifestations include Raynaud's phenomenon, abnormal nailfold capillary morphology, digital ulcers, pulmonary arterial hypertension, cardiovascular disease (primary and coronary), telangiectasia, renal crisis, and gastric antral vascular ectasia. Tissue ischaemia might also contribute to aberrant tissue remodelling, resulting in calcinosis and fibrosis. Recognition of the substantial inter-relationship between these vascular complications is growing; examples of vascular treatment interventions targeting digital vasculopathy having off-target vascular benefits in other organs have been reported. In general, treatment of life-threatening vascular complications, such as pulmonary arterial hypertension, is not commenced until classifiable organ disease has occurred; however, the identification of robust prognostic biomarkers might allow such complications to be averted with preventative disease modification. In this Personal View, we describe the inter-relationship between vascular features of systemic sclerosis. We consider how these features might be exploited to establish a unified vascular conceptual framework that can inform the development of both predictive composite indices to guide preventative intervention, and a unified vascular composite endpoint model that can effectively capture clinically meaningful disease modification in future clinical trials of vasoactive treatments in systemic sclerosis. CI - Copyright © 2025 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust Southmead Hospital, Bristol, UK; School of Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Cochin Hospital, INSERM U1016, Université Paris Cité, Paris, France. FAU - Buch, Maya H AU - Buch MH AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester, UK; NIHR Manchester Biomedical Research Centre, Manchester, UK. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory for Experimental Rheumatology, Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; IRCCS San Martino Polyclinic Hospital, Genoa, Italy. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. FAU - Denton, Christopher P AU - Denton CP AD - Division of Medicine, University College London, London, UK. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK; ImmunoConcEpT, CNRS UMR 5164, University of Bordeaux, Bordeaux, France. FAU - Domsic, Robyn T AU - Domsic RT AD - School of Medicine, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Frech, Tracy AU - Frech T AD - Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA; Veterans Affairs Tennessee Valley Healthcare System, Nashville, TN, USA. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester, UK. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy; Inflammation, fibrosis and ageing initiative, IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita Salute San Raffaele University, Milan, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Center for Inflammation Research, Ghent, Belgium. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - ImmunoConcEpT, CNRS UMR 5164, University of Bordeaux, Bordeaux, France; Rheumatology Department, CHU Bordeaux Hospital, Bordeaux, France. FAU - Hughes, Michael AU - Hughes M AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester, UK; Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK. Electronic address: michael.hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20251014 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications MH - Raynaud Disease/etiology MH - *Vascular Diseases/etiology MH - Telangiectasis/etiology MH - Cardiovascular Diseases/etiology MH - Gastric Antral Vascular Ectasia/etiology MH - Pulmonary Arterial Hypertension/etiology COIS- Declaration of interests JDP has received speaker honoraria or consultancy fees from Johnson & Johnson, AstraZeneca, Permeatus, IsomAb, Boehringer Ingelheim, and Sojournix, and support for attending meetings from Boehringer Ingelheim, Johnson & Johnson, and CSL Vifor. YA received research grants from Alpine Immune Sciences, Corvus Pharma, Merck Serono, and Medsenic, and honorarium from AbbVie, Argenx, AstraZeneca, Boehringer Ingelheim, Medsenic, and Topadur Pharma. MHB received honoraria or consultancy fees and travel support for the field of systemic sclerosis from Boehringer Ingelheim and Johnson & Johnson. MC received support from the European Alliance of Associations for Rheumatology and the American College of Rheumatology for full participation in annual meetings; research grants from Boehringer Ingelheim, Amgen, Bristol Myers Squibb, and AbbVie; and honoraria for lectures from Alfasigma. FDG reports research grants from AbbVie, AstraZeneca, Boehringer Ingelheim, Calluna Pharma, Merck, and Mitusbishi-Tanabe; speaker or consultancy fees from AbbVie, AstraZeneca, Argenx, Bristol Myers Squibb, Boehringer Ingelheim, Calluna, DeepCure, Ergomed, GSK, Johnson & Johnson, Merck, Mitsubishi-Tanabe, Novartis, and Ventus Therapeutics; and participation on a data safety monitoring board or advisory board for Milteny Biotec. CPD reports speaker or consultancy fees from Boehringer Ingelheim, Johnson & Johnson, GSK, Novartis, ARXX, Roche, AbbVie, and Merck, and research grants from AbbVie, GSK, and Servier. RTD reports consulting fees from Aisa Pharma and AstraZeneca, and participation on a data safety monitoring board or advisory board for AstraZeneca. ALH has received consultancy fees from AbbVie, Arena Pharmaceuticals, Boehringer Ingelheim, Galderma, Johnson & Johnson, and Zura Bio, and speaker fees from Johnson & Johnson. MM-C reports grants from Boehringer Ingelheim and Argenx, and honoraria from Boehringer Ingelheim and MSD. VS is supported by an unrestricted educational chair on systemic sclerosis from Johnson & Johnson-Cilag; received financial research support from the Belgian Fund for Scientific Research in Rheumatic Diseases and Boehringer Ingelheim; consultancy fees from Boehringer Ingelheim, Johnson & Johnson-Cilag, Argenx, WebMD Global; speaker fees from Boehringer Ingelheim, Johnson & Johnson-Cilag, and BKC Moving Media Makers; and participated on advisory boards for Johnson & Johnson-Cilag and GSK. M-ET reports consulting fees from AstraZeneca, AbbVie, Novartis, and UCB; honoraria for lectures from Celltrion, UCB, and Johnson & Johnson; and support for attending meetings from Johnson & Johnson and UCB. MH reports research funding and speaker fees from Johnson & Johnson; conference support from UCB; and consultancy fees from Boehringer Ingelheim and Novartis (none of which are relevant to this manuscript). All other authors declare no competing interests. EDAT- 2025/10/18 00:29 MHDA- 2025/11/20 00:29 CRDT- 2025/10/17 18:53 PHST- 2025/02/26 00:00 [received] PHST- 2025/07/24 00:00 [revised] PHST- 2025/08/18 00:00 [accepted] PHST- 2025/11/20 00:29 [medline] PHST- 2025/10/18 00:29 [pubmed] PHST- 2025/10/17 18:53 [entrez] AID - S2665-9913(25)00251-6 [pii] AID - 10.1016/S2665-9913(25)00251-6 [doi] PST - ppublish SO - Lancet Rheumatol. 2025 Dec;7(12):e895-e906. doi: 10.1016/S2665-9913(25)00251-6. Epub 2025 Oct 14. PMID- 35441683 OWN - NLM STAT- MEDLINE DCOM- 20230207 LR - 20260127 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 62 IP - SI DP - 2023 Feb 6 TI - The effect of silver fibre gloves on Raynaud's phenomenon in patients with systemic sclerosis: a double-blind randomized crossover trial. PG - SI74-SI81 LID - 10.1093/rheumatology/keac243 [doi] AB - OBJECTIVES: Silver fibre gloves transport heat from the palm to the fingers, possibly reducing the burden of RP in SSc patients. We aim to evaluate the clinical efficiency of this intervention. METHODS: A multicentre, double-blind, randomized trial was performed, accounting for interindividual differences and external factors using a crossover design. Patients were randomized in two groups: group 1 wore 8% silver fibre gloves in period 1 and normal gloves in period 2 and group 2 vice versa. Each period lasted 6 weeks. The primary outcome was the Raynaud Condition Score (RCS) over time (minimal clinical important difference 1.4), assessed three times per week using an online questionnaire. Secondary outcomes included vascular complications and Scleroderma-Health Assessment Questionnaire (SHAQ). Outcomes were evaluated before unblinding using linear mixed models. RESULTS: A total of 85 SSc patients were included, with 76 completing the study. The mean RCS during 2 weeks before the study (i.e. without gloves) was 6.4 (s.d. 1.6). Both with silver fibre gloves and normal gloves the mean RCS decreased to 3.9 (s.d. 2.3) with a similar course over time. There was no difference in mean RCS over time between the type of gloves [β = 0.067 (95% CI -0.006, 0.19)]. Of secondary outcomes, total SHAQ [β = 0.036 (95% CI 0.026, 0.046)] was slightly higher with silver fibre gloves, which is clinically irrelevant. Three patients developed new digital ulcers with normal gloves vs one patient with silver fibre gloves [odds ratio 3.2 (95% CI 0.32, 31.1)]. CONCLUSIONS: Wearing gloves in SSc patients clearly decreases the RP burden. Our results do not support the hypothesis that increased heat transport of 8% silver fibre gloves is associated with less disease burden as measured in this study by the RCS compared with normal gloves. CLINICAL TRIAL REGISTRATION NUMBER: Netherlands Trial register (https://www.trialregister.nl/) NL7904. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Liem, Sophie I E AU - Liem SIE AD - Department of Rheumatology, Leiden University Medical Center, Leiden. FAU - Hoekstra, Eva M AU - Hoekstra EM AD - Department of Rheumatology, Leiden University Medical Center, Leiden. FAU - Bonte-Mineur, Femke AU - Bonte-Mineur F AD - Department of Rheumatology and Clinical Immunology, Maasstad Ziekenhuis, Rotterdam. FAU - Magro Checa, César AU - Magro Checa C AD - Department of Rheumatology, Zuyderland Medical Center, Heerlen. FAU - Schouffoer, Anne AU - Schouffoer A AD - Department of Rheumatology, Haga Ziekenhuis, The Hague, The Netherlands. FAU - Allaart, Cornelia F AU - Allaart CF AD - Department of Rheumatology, Leiden University Medical Center, Leiden. FAU - Huizinga, Tom W J AU - Huizinga TWJ AD - Department of Rheumatology, Leiden University Medical Center, Leiden. FAU - Bergstra, Sytske Anne AU - Bergstra SA AUID- ORCID: 0000-0002-7136-5248 AD - Department of Rheumatology, Leiden University Medical Center, Leiden. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Department of Rheumatology, Leiden University Medical Center, Leiden. LA - eng SI - NTR/NL7904 PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 3M4G523W1G (Silver) RN - digital ulcers SB - IM MH - Humans MH - Cross-Over Studies MH - Silver MH - *Scleroderma, Systemic/complications MH - *Scleroderma, Localized/complications MH - *Raynaud Disease/complications MH - Skin Ulcer PMC - PMC9910564 OTO - NOTNLM OT - RP OT - SSc OT - crossover trial OT - scleroderma OT - silver fibre gloves EDAT- 2022/04/21 06:00 MHDA- 2023/02/08 06:00 PMCR- 2022/04/20 CRDT- 2022/04/20 08:42 PHST- 2022/01/13 00:00 [received] PHST- 2022/03/30 00:00 [revised] PHST- 2022/04/21 06:00 [pubmed] PHST- 2023/02/08 06:00 [medline] PHST- 2022/04/20 08:42 [entrez] PHST- 2022/04/20 00:00 [pmc-release] AID - 6571140 [pii] AID - keac243 [pii] AID - 10.1093/rheumatology/keac243 [doi] PST - ppublish SO - Rheumatology (Oxford). 2023 Feb 6;62(SI):SI74-SI81. doi: 10.1093/rheumatology/keac243. PMID- 22660809 OWN - NLM STAT- MEDLINE DCOM- 20121126 LR - 20230215 IS - 1499-2752 (Electronic) IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 39 IP - 7 DP - 2012 Jul TI - Vascular leak is a central feature in the pathogenesis of systemic sclerosis. PG - 1385-91 LID - 10.3899/jrheum.111380 [doi] AB - OBJECTIVE: The main histopathological focus of systemic sclerosis (SSc) has concentrated on fibrotic changes. We investigated the microvasculature alterations in the skin of patients with SSc at various stages of disease duration with whole-field digital microscopy. METHODS: Twenty consecutive patients with SSc, 1 with Raynaud's phenomenon (RP) without SSc, and 4 healthy controls underwent punch biopsy on the medial forearm. Eighteen patients were included in the primary analysis. Two with recent-onset diffuse cutaneous disease, 1 repeat SSc biopsy, and 1 patient with RP without SSc were also evaluated. All specimens were processed with histochemical stains and immunohistochemistry. We analyzed microvasculature abnormalities in an objective and systematic manner taking advantage of recent advances in whole-field digital microscopy. This analysis was coupled with ultrastructural evaluation performed with transmission electron microscopy (TEM). RESULTS: Whole-field digital microscopy and TEM of SSc skin biopsies revealed that endothelial abnormalities are a universal feature regardless of clinical features and/or duration of disease. These features were not seen in any healthy control specimens or in the single RP patient samples. Whole-field digital microscopy identified increased interstitial edema (31.0% ± 9.6% vs 17.6% ± 3.3% in controls; p = 0.009) and fibrosis (75.6% ± 5.7% vs 66.1% ± 9.8% in controls; p = 0.02) in all patients with SSc. Lower CD34 staining was seen in SSc compared to healthy controls (0.32% ± 0.22% vs 1.31% ± 0.34%; p < 0.0001) and within the SSc population with interstitial lung disease (0.55% ± 0.22% vs 0.15% ± 0.16%; p = 0.01). Perivascular and interstitial infiltrate of mast cells was present in all SSc specimens. CONCLUSION: Whole-field digital microscopy offers a means of rapidly carrying out objective, fully quantitative, and reproducible measurements of microscopic features of SSc microvascular change. The universal morphologically abnormal endothelial cells and interstitial edema in all patients with SSc biopsied suggests that SSc may be intrinsically a disease of the endothelium characterized by vascular leak. FAU - Frech, Tracy M AU - Frech TM AD - Division of Rheumatology, Department of Internal Medicine, University of Utah, 4B200 SOM, 30 N 1900 E, Salt Lake City, Utah 84132, USA.. tracy.frech@hsc.utah.edu FAU - Revelo, Monica P AU - Revelo MP FAU - Drakos, Stavros G AU - Drakos SG FAU - Murtaugh, Maureen A AU - Murtaugh MA FAU - Markewitz, Boaz A AU - Markewitz BA FAU - Sawitzke, Allen D AU - Sawitzke AD FAU - Li, Dean Y AU - Li DY LA - eng GR - R01 HL077671/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20120601 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antigens, CD34) SB - IM MH - Adult MH - Aged MH - Antigens, CD34/analysis MH - Capillary Leak Syndrome/*pathology MH - Edema/pathology MH - Endothelium, Vascular/*pathology/ultrastructure MH - Female MH - Humans MH - Lung Diseases, Interstitial/pathology MH - Male MH - Mast Cells MH - Microscopy MH - Microvessels MH - Middle Aged MH - Raynaud Disease/pathology MH - Scleroderma, Systemic/*pathology MH - Severity of Illness Index MH - Skin/*blood supply/pathology PMC - PMC3640272 MID - NIHMS442676 EDAT- 2012/06/05 06:00 MHDA- 2012/12/10 06:00 PMCR- 2013/05/01 CRDT- 2012/06/05 06:00 PHST- 2012/06/05 06:00 [entrez] PHST- 2012/06/05 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] PHST- 2013/05/01 00:00 [pmc-release] AID - jrheum.111380 [pii] AID - 10.3899/jrheum.111380 [doi] PST - ppublish SO - J Rheumatol. 2012 Jul;39(7):1385-91. doi: 10.3899/jrheum.111380. Epub 2012 Jun 1. PMID- 19370186 OWN - NLM STAT- MEDLINE DCOM- 20090529 LR - 20191111 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 61 IP - 1 DP - 2009 Jan-Mar TI - [Nailfold capillaroscopy and blood flow laser-doppler analysis of the microvascular damage in systemic sclerosis: preliminary results]. PG - 34-40 AB - OBJECTIVES: Systemic sclerosis (SSc) is characterized by altered microvascular structure and function. Nailfold videocapillaroscopy (NVC) is the tool to evaluate capillary morphological structure and laser-Doppler Blood flowmetry (LDF) can be used to estimate cutaneous blood flow of microvessels. The aim of this study was to investigate possible relationships between capillary morphology and blood flow in SSc. METHODS: Twenty-seven SSc patients and 12 healthy subjects were enrolled. SSc microvascular involvement, as evaluated by NVC, was classified in three different patterns ("Early", "Active", "Late"). LDF analysis was performed at the II, III, IV, V hand fingers in both hands and both at cutaneous temperature and at 36 degrees C. Statistical evaluation was carried out by non-parametric procedures. RESULTS: Blood flow was found significantly lower in SSc patients when compared with healthy subjects (p<0.05). The heating of the probe to 36 degrees C induced a significant increase in peripheral blood flow in all subjects compared to baseline (p <0.05), however, the amount of variation was significantly lower in patients with SSc, compared with healthy controls (p <0.05). The SSc patients with NVC "Late" pattern, showed lower values of peripheral blood flow than patients with NVC "Active" or "Early" patterns (p<0.05). Moreover, a negative correlation between the tissue perfusion score and the progression of the SSc microangiopathy was observed, as well as between the tissue perfusion and the duration of the Raynaud's phenomenon (p <0.03). CONCLUSIONS: LDF can be employed to evaluate blood perfusion in the microvascular circulation in SSc patients. The blood flow changes observed with the LDF seem to correlate with the severity of microvascular damage in SSc as detected by NVC. FAU - Secchi, M E AU - Secchi ME AD - Laboratorio di Ricerca e U.O.C. di Reumatologia, Dipartimento di Medicina Interna, Università degli Studi di Genova, Italia. secchime@yahoo.it FAU - Sulli, A AU - Sulli A FAU - Pizzorni, C AU - Pizzorni C FAU - Cutolo, M AU - Cutolo M LA - ita PT - Comparative Study PT - English Abstract PT - Journal Article TT - Studio della microangiopatia sclerodermia mediante valutazione dinamica con laser-Doppler e morfologica con videocapillaroscopia periungueale: risultati preliminari. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Aged MH - Data Interpretation, Statistical MH - Humans MH - Iloprost/therapeutic use MH - *Laser-Doppler Flowmetry MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/blood supply MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis/drug therapy/physiopathology MH - Vasodilator Agents/therapeutic use MH - Video Recording EDAT- 2009/04/17 09:00 MHDA- 2009/05/30 09:00 CRDT- 2009/04/17 09:00 PHST- 2009/04/17 09:00 [entrez] PHST- 2009/04/17 09:00 [pubmed] PHST- 2009/05/30 09:00 [medline] AID - 10.4081/reumatismo.2009.34 [doi] PST - ppublish SO - Reumatismo. 2009 Jan-Mar;61(1):34-40. doi: 10.4081/reumatismo.2009.34. PMID- 39841617 OWN - NLM STAT- MEDLINE DCOM- 20250502 LR - 20250523 IS - 1365-2060 (Electronic) IS - 0785-3890 (Print) IS - 0785-3890 (Linking) VI - 57 IP - 1 DP - 2025 Dec TI - Clinical course and potential associated factors of progressive calcinosis cutis in early systemic sclerosis: a cohort study. PG - 2455535 LID - 10.1080/07853890.2025.2455535 [doi] LID - 2455535 AB - BACKGROUND: Calcinosis cutis of hands can progress and impair hand function in systemic sclerosis (SSc). Understanding the natural disease and comprehensive management is crucial. OBJECTIVE: To examine clinical course and identify risk factors associated with progressive calcinosis cutis in early SSc. METHODS: Dual time-point hand radiography was performed at initial and after diagnosis at median interval (range 2.9 ± 0.4 years) in 53 recruited patients with early SSc. Progressive calcinosis cutis defined as the worsening of severity according to simple soring scoring system (no, mild, moderate, severe) comparing to previous hand radiography. Odds ratio (OR) and their 95%CI were used to evaluate associated factors and calcinosis cutis progression. RESULTS: A total of 35 cases (155 per 100 person-year), showed progressive calcinosis cutis with the incidence of 22.6 per 100-person-years (95%CI 16.2-31.4). The most common area of progressive calcinosis cutis was at right distal phalanx, 12 of 35 (22.6%). Although statistically not significant by logistic regression analysis, elderly patients, Raynaud's phenomenon, ischemic ulcer, telangiectasia, and salt-pepper tended to be more frequent in progressive calcinosis cutis than those who had no progression. Around one-quarter of those who had no calcinosis cutis experienced worsening across more than one level of severity. CONCLUSION: Progression of calcinosis cutis in early SSc increased over time, particularly within 3 years after the first evaluation. Elderly patients and those with vasculopathy were found more frequently. Further study with a larger cohort is needed to support these findings. FAU - Kanjanajarurat, Vassana AU - Kanjanajarurat V AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Chowchuen, Prathana AU - Chowchuen P AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Thammaroj, Punthip AU - Thammaroj P AUID- ORCID: 0000-0001-5532-0165 AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. LA - eng PT - Journal Article DEP - 20250122 PL - England TA - Ann Med JT - Annals of medicine JID - 8906388 SB - IM MH - Humans MH - *Calcinosis/etiology/diagnostic imaging/epidemiology/pathology MH - Female MH - Male MH - *Scleroderma, Systemic/complications/diagnostic imaging MH - Middle Aged MH - Disease Progression MH - Aged MH - Risk Factors MH - Cohort Studies MH - Adult MH - Severity of Illness Index MH - Radiography MH - *Skin Diseases/etiology/diagnostic imaging MH - Incidence MH - Hand/diagnostic imaging MH - Raynaud Disease MH - Calcinosis Cutis PMC - PMC11755737 OTO - NOTNLM OT - Calcinosis cutis OT - cohort OT - hand radiograph OT - scleroderma OT - systemic sclerosis COIS- No potential conflict of interest was reported by the author(s). EDAT- 2025/01/22 18:25 MHDA- 2025/01/22 18:26 PMCR- 2025/01/22 CRDT- 2025/01/22 13:04 PHST- 2025/01/22 18:26 [medline] PHST- 2025/01/22 18:25 [pubmed] PHST- 2025/01/22 13:04 [entrez] PHST- 2025/01/22 00:00 [pmc-release] AID - 2455535 [pii] AID - 10.1080/07853890.2025.2455535 [doi] PST - ppublish SO - Ann Med. 2025 Dec;57(1):2455535. doi: 10.1080/07853890.2025.2455535. Epub 2025 Jan 22. PMID- 32515028 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20220531 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 47 IP - 9 DP - 2020 Sep TI - Decreased serum cathepsin S levels in patients with systemic sclerosis-associated interstitial lung disease. PG - 1027-1032 LID - 10.1111/1346-8138.15458 [doi] AB - Cathepsin S (CTSS) is a lysosomal proteolytic enzyme regulating intracellular and extracellular biological activities, including immunity/inflammation and remodeling of vasculature and extracellular matrix, which are the three cardinal pathological events associated with systemic sclerosis (SSc). To elucidate the potential role of CTSS in the development of SSc, we investigated the clinical correlation of serum CTSS levels. Because serum CTSS levels were inversely correlated with estimated glomerular filtration rate (eGFR) in SSc patients with renal dysfunction (eGFR, <60 min/mL per 1.73 m(2) ), SSc patients with normal renal function (eGFR, ≥60 min/mL per 1.73 m(2) ) were analyzed. Serum CTSS levels were significantly decreased in diffuse cutaneous SSc patients compared with limited cutaneous SSc patients and healthy controls. Among vascular and fibrotic clinical manifestations, Raynaud's phenomenon and interstitial lung disease (ILD) were relevant to a significant decrease in serum CTSS levels. Importantly, serum CTSS levels negatively correlated with serum levels of Krebs von den Lungen-6 and surfactant protein D in total SSc patients, while not correlating with modified Rodnan total skin thickness score and the percentage of predicted diffusion lung capacity for carbon monoxide and showing a positive trend with the percentage of predicted vital capacity. These results suggest a potential contribution of decreased CTSS expression to the development of ILD in patients with SSc. CI - © 2020 Japanese Dermatological Association. FAU - Toyama, Satoshi AU - Toyama S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yamashita, Takashi AU - Yamashita T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Saigusa, Ryosuke AU - Saigusa R AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miura, Shunsuke AU - Miura S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Nakamura, Kouki AU - Nakamura K AUID- ORCID: 0000-0002-2296-6004 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Hirabayashi, Megumi AU - Hirabayashi M AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miyagawa, Takuya AU - Miyagawa T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Fukui, Yuki AU - Fukui Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Omatsu, Jun AU - Omatsu J AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yoshizaki, Ayumi AU - Yoshizaki A AUID- ORCID: 0000-0002-8194-9140 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Sato, Shinichi AU - Sato S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Asano, Yoshihide AU - Asano Y AUID- ORCID: 0000-0001-5560-9778 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. LA - eng GR - The Ministry of Health, Labour, and Welfare of Japan/ PT - Journal Article DEP - 20200608 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Biomarkers) RN - EC 3.4.- (Cathepsins) RN - EC 3.4.22.27 (cathepsin S) SB - IM MH - Biomarkers MH - Cathepsins MH - Humans MH - Lung MH - *Lung Diseases, Interstitial/diagnosis/etiology MH - *Raynaud Disease MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - cathepsin S OT - fibrosis OT - lung OT - systemic sclerosis OT - vasculopathy EDAT- 2020/06/10 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/06/10 06:00 PHST- 2020/03/02 00:00 [received] PHST- 2020/05/11 00:00 [accepted] PHST- 2020/06/10 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/06/10 06:00 [entrez] AID - 10.1111/1346-8138.15458 [doi] PST - ppublish SO - J Dermatol. 2020 Sep;47(9):1027-1032. doi: 10.1111/1346-8138.15458. Epub 2020 Jun 8. PMID- 18578962 OWN - NLM STAT- MEDLINE DCOM- 20081210 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 3 DP - 2008 May-Jun TI - Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. PG - 414-20 AB - OBJECTIVE: To investigate the association between serum levels and clinical signs of lung fibrosis in patients with systemic sclerosis and inflammatory myopathies. METHODS: ELISA tests for a mucin-like glycoprotein KL-6, von Willebrandt factor (vWF), soluble E-selectin (sES) and surfactant protein D (SP-D) were performed in sera of 104 patients with systemic sclerosis, 31 patients with poly/dermatomyositis) and 24 patients with Raynaud's phenomenon as controls. The clinical and laboratory data were evaluated by a simple standard protocol including chest x-ray, lung function tests, echocardiography and, in selected cases, high resolution computer tomography (HRCT). Clinically significant pulmonary fibrosis (PF) defined as a simultaneous presence of radiological sign of pulmonary fibrosis and restrictive impairment. Severe PF was established if HRCT scans showed diffuse interstitial lung disease with low diffusing capacity. End stage PF was determined as severe PF with very low diffusing capacity. RESULTS: Patients with pulmonary fibrosis on chest x-ray showed significantly elevated serum levels of KL-6, SP-D and vWF. Inverse correlation was found between serum levels of KL-6/SP-D and lung function parameters, such as DLCO% and FVC. With regard to HRCT findings, patients with elevated serum level of KL-6 showed significantly more frequently ground glass opacity, diffuse and honeycombing fibrosis than patients with normal level of KL-6. The sensitivity of KL-6 for PF in SSc is increased with the severity of PF (PF on chest x-ray < severe PF < end stage of PF). Lung fibrosis occurred more frequently in patients with simultaneously elevated KL-6 and sES compared to cases with a single positivity of either KL-6 or sES. CONCLUSION: KL-6, SP-D, vWF and ES are good surrogate factors of pulmonary fibrosis but can not replace conventional diagnostic procedures. However, these markers are suitable for the assessment of progression and severity of pulmonary fibrosis in systemic autoimmune disorders once the diagnosis is established. FAU - Kumánovics, G AU - Kumánovics G AD - Department of Immunology and Rheumatology, Medical Faculty, University of Pécs, Pécs, Hungary. FAU - Minier, T AU - Minier T FAU - Radics, J AU - Radics J FAU - Pálinkás, L AU - Pálinkás L FAU - Berki, T AU - Berki T FAU - Czirják, L AU - Czirják L LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Biomarkers) RN - 0 (E-Selectin) RN - 0 (MUC1 protein, human) RN - 0 (Mucin-1) RN - 0 (Pulmonary Surfactant-Associated Protein D) RN - 0 (von Willebrand Factor) SB - IM MH - Adult MH - Aged MH - Biomarkers/blood MH - Case-Control Studies MH - Dermatomyositis/*blood/complications MH - Disease Progression MH - E-Selectin/*blood MH - Female MH - Humans MH - Male MH - Middle Aged MH - Mucin-1/*blood MH - Pulmonary Fibrosis/*blood/complications/diagnosis MH - Pulmonary Surfactant-Associated Protein D/*blood MH - Radiography, Thoracic MH - Raynaud Disease/blood MH - Respiratory Function Tests MH - Scleroderma, Systemic/*blood/complications MH - Sensitivity and Specificity MH - Severity of Illness Index MH - von Willebrand Factor/*metabolism EDAT- 2008/06/27 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/06/27 09:00 PHST- 2008/06/27 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/06/27 09:00 [entrez] AID - 2327 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 May-Jun;26(3):414-20. PMID- 28712039 OWN - NLM STAT- MEDLINE DCOM- 20180702 LR - 20181113 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 53 IP - 3 DP - 2017 Dec TI - Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. PG - 306-336 LID - 10.1007/s12016-017-8625-4 [doi] AB - Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management. FAU - Ferreli, Caterina AU - Ferreli C AUID- ORCID: 0000-0002-8223-6791 AD - Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy. ferreli@unica.it. FAU - Gasparini, Giulia AU - Gasparini G AD - Section of Dermatology, Department of Health Sciences, DISSAL, IRCSS-AOU S. Martino-IST, University of Genoa, Genoa, Italy. FAU - Parodi, Aurora AU - Parodi A AD - Section of Dermatology, Department of Health Sciences, DISSAL, IRCSS-AOU S. Martino-IST, University of Genoa, Genoa, Italy. FAU - Cozzani, Emanuele AU - Cozzani E AD - Section of Dermatology, Department of Health Sciences, DISSAL, IRCSS-AOU S. Martino-IST, University of Genoa, Genoa, Italy. FAU - Rongioletti, Franco AU - Rongioletti F AD - Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy. FAU - Atzori, Laura AU - Atzori L AD - Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy. LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Diabetes Complications/*diagnosis/immunology MH - Diagnosis, Differential MH - Graft vs Host Disease/*diagnosis/immunology MH - Humans MH - Porphyria Cutanea Tarda/*diagnosis/immunology MH - Raynaud Disease MH - Scleroderma, Localized/*diagnosis MH - Scleroderma, Systemic/*diagnosis/immunology MH - Skin/*pathology OTO - NOTNLM OT - Cutaneous sclerosis OT - Morphea OT - Scleroderma OT - Scleroderma-like disorders OT - Systemic sclerosis EDAT- 2017/07/18 06:00 MHDA- 2018/07/03 06:00 CRDT- 2017/07/17 06:00 PHST- 2017/07/18 06:00 [pubmed] PHST- 2018/07/03 06:00 [medline] PHST- 2017/07/17 06:00 [entrez] AID - 10.1007/s12016-017-8625-4 [pii] AID - 10.1007/s12016-017-8625-4 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID- 34076720 OWN - NLM STAT- MEDLINE DCOM- 20220719 LR - 20220721 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 42 IP - 8 DP - 2022 Aug TI - Nailfold capillaroscopy changes associated with anti-RNP antibodies in systemic lupus erythematosus. PG - 1355-1361 LID - 10.1007/s00296-021-04894-4 [doi] AB - Anti-U1RNP antibody is associated with distinct organ involvement in patients with systemic lupus erythematosus (SLE). Nailfold capillaroscopy (NFC) allows non-invasive assessment of microvascular abnormalities in several connective tissue diseases. The objective of this study is to determine the association of anti-U1RNP antibody with microvascular changes by NFC in RNP-positive SLE patients in comparison with RNP-negative SLE patients (negative disease controls) and mixed connective tissue disease (MCTD) cases (positive disease controls). NFC examination was performed in consecutive patients with SLE with or without anti-U1RNP positivity. MCTD patients were recruited as disease controls. Abnormalities noted in the three groups were compared using non-parametric tests. Ordinal logistic or linear regression was used wherever applicable. 81 patients were studied, of whom 28 were diagnosed as RNP-positive SLE (age 30.0 ± 10.37; 26 females), 26 were RNP-negative SLE (age 29.42 ± 9.20; 25 females) and 27 had MCTD (age36.5 ± 9.70; 25 females). RNP-positive SLE patients had more frequent giant capillaries, enlarged capillaries and ramified capillaries as compared to RNP-negative SLE (p = 0.05, < 0.01 and 0.03, respectively). The capillary density was lower in patients with MCTD as compared with patients with RNP-positive SLE (5.11 ± 1.69/mm vs 7.25 ± 1.38/ mm, p < 0.01) and RNP-negative SLE (8.92 ± 1.13/mm, p < 0.01). The reduction in capillary density was less severe in patients with RNP-negative SLE as compared with RNP-positive SLE (OR = 0.1058 [95% CI = 0.02-0.546], p < 0.01) which was independent of the presence of Raynaud's phenomenon, interstitial lung disease and disease duration. Presence of anti-U1RNP antibody is associated with notable patterns of microvascular abnormalities in SLE. These NFC abnormalities are noted more profoundly in patients with MCTD and are less marked in RNP-negative SLE patients. CI - © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Chebbi, Pramod Prahlad AU - Chebbi PP AD - Departments of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India. AD - Department of Rheumatology, SDM College of Medical Sciences and Hospital, SDM University, Dharwad, Karnataka, India. FAU - Goel, Ruchika AU - Goel R AD - Departments of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India. Ruchika.goel@cmcvellore.ac.in. FAU - Ramya, J AU - Ramya J AD - Departments of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India. AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College, Bengaluru, Karnataka, India. FAU - Gowri, M AU - Gowri M AD - Departments of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India. FAU - Herrick, ArianeL AU - Herrick A AD - Division of Musculoskeletal and Dermatological Sciences, Manchester Academic Health Science Centre, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester, M13 9PT, UK. FAU - Danda, Debashish AU - Danda D AD - Departments of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India. LA - eng PT - Journal Article DEP - 20210602 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Capillaries MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/diagnosis MH - Microscopic Angioscopy MH - *Mixed Connective Tissue Disease/diagnosis MH - *Raynaud Disease MH - Young Adult OTO - NOTNLM OT - MCTD OT - Nailfold capillaroscopy OT - RNP antibody OT - SLE OT - Videocapillaroscopy EDAT- 2021/06/03 06:00 MHDA- 2022/07/20 06:00 CRDT- 2021/06/02 12:22 PHST- 2021/02/25 00:00 [received] PHST- 2021/05/15 00:00 [accepted] PHST- 2021/06/03 06:00 [pubmed] PHST- 2022/07/20 06:00 [medline] PHST- 2021/06/02 12:22 [entrez] AID - 10.1007/s00296-021-04894-4 [pii] AID - 10.1007/s00296-021-04894-4 [doi] PST - ppublish SO - Rheumatol Int. 2022 Aug;42(8):1355-1361. doi: 10.1007/s00296-021-04894-4. Epub 2021 Jun 2. PMID- 18050188 OWN - NLM STAT- MEDLINE DCOM- 20080207 LR - 20220318 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 57 IP - 8 DP - 2007 Dec 15 TI - General and specific factors associated with severity of cognitive impairment in systemic lupus erythematosus. PG - 1461-72 AB - OBJECTIVE: To determine factors affecting the severity of cognitive impairment in systemic lupus erythematosus (SLE) and to analyze its anatomic location. METHODS: Fifteen cognitive functions grouped into 8 domains were evaluated in 52 patients with SLE and 20 with rheumatoid arthritis. Patients were classified according to severity of impairment as normal, mild, or moderate/severe. Multivariate analysis was performed to identify the main factors affecting severity of cognitive deficits. The most likely anatomic site of damage according to neuropsychological performance was compared with the lesion's location on magnetic resonance imaging (MRI). RESULTS: In SLE patients, a stepwise regression analysis showed that the number of impaired functions (dependent variable) was associated with antiphospholipid antibody positivity (aPL+; P = 0.04), the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI; P = 0.001), hypertension (P = 0.032), and was inversely related to educational level (P = 0.021). Including MRI, the number of impaired functions was associated with severity of MRI (P < 0.001), the SDI (P = 0.013), and the presence of Raynaud's phenomenon (P = 0.04). The contemporary presence of aPL+ and Raynaud's phenomenon resulted in a higher probability to develop moderate/severe cognitive deficits (P = 0.015). Two logistic multiple regression analyses identified hypertension (P < 0.05), the SDI (P < 0.01), and moderate/severe MRI findings as main predictors of moderate/severe impairment (dependent variable). The damage site hypothesized through neuropsychological testing corresponded with MRI findings in 71.7% of SLE patients K = 0.42, P = 0.005). CONCLUSION: Hypertension, aPL+, accumulated damage, and MRI lesions are the main factors affecting severity of cognitive impairment in SLE. The hypothesized sites of central nervous system involvement according to neuropsychological testing correlated with MRI findings in most patients. FAU - Tomietto, P AU - Tomietto P AD - University of Udine, Udine, Italy. FAU - Annese, V AU - Annese V FAU - D'agostini, S AU - D'agostini S FAU - Venturini, P AU - Venturini P FAU - La Torre, G AU - La Torre G FAU - De Vita, S AU - De Vita S FAU - Ferraccioli, G F AU - Ferraccioli GF LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/adverse effects/blood MH - Arthritis, Rheumatoid/complications/physiopathology/psychology MH - Case-Control Studies MH - Central Nervous System/pathology MH - Cognition Disorders/*etiology/physiopathology/*psychology MH - Female MH - Humans MH - Hypertension/complications MH - Lupus Erythematosus, Systemic/*complications/physiopathology/*psychology MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Neuropsychological Tests MH - Psychiatric Status Rating Scales MH - Raynaud Disease/complications MH - Risk Factors MH - *Severity of Illness Index EDAT- 2007/12/01 09:00 MHDA- 2008/02/08 09:00 CRDT- 2007/12/01 09:00 PHST- 2007/12/01 09:00 [pubmed] PHST- 2008/02/08 09:00 [medline] PHST- 2007/12/01 09:00 [entrez] AID - 10.1002/art.23098 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Dec 15;57(8):1461-72. doi: 10.1002/art.23098. PMID- 31969216 OWN - NLM STAT- MEDLINE DCOM- 20200917 LR - 20200917 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 38 Suppl 125 IP - 3 DP - 2020 May-Jun TI - Differential performance of nailfold video capillaroscopic parameters in the diagnosis and prognosis of systemic sclerosis. PG - 29-39 AB - OBJECTIVES: The contribution of nailfold video capillaroscopy (NVC) in identifying patients with Raynaud's phenomenon (RP) at risk for systemic sclerosis (SSc) is well established. Herein we comparatively assess the performance of different capillaroscopic parameters in diagnosing SSc among patients with RP and evaluate the prognostic capacity of NVC in SSc. METHODS: At baseline we clinically and capillaroscopically evaluated 242 consecutive patients referred to our department for NVC (138 with SSc); 175 were reevaluated after 3.38±1.47 years. Sixty-two healthy volunteers served as controls. Capillaroscopy pattern (normal/early/active/late) was qualitatively defined. Capillary loss, dilated, giant or ramified capillaries and micro-haemorrhages were scored semi-quantitatively. RESULTS: Capillary loss score had the highest diagnostic accuracy at discriminating patients with an SSc-spectrum disorder from patients with RP of different etiology and controls, as defined by ROC curve analysis [AUC (95% CI)=0.905 (0.869-0.942)], followed by dilatation score [0.863 (0.818-0.907)] and giant score [0.835 (0.787-0.884)]. By contrast, micro-haemorrhages [0.720 (0.662-0.779)] and ramifications scores [0.604 (0.539-0.670)] performed worse. Multivariate analysis in 94 SSc patients indicated that active (OR=3.305, p=0.043) and late (OR=6.900, p=0.023) baseline capillaroscopy pattern predicted occurrence of a combined adverse disease outcome [forced vital capacity (FVC) deterioration>10% and/or DLCO deterioration>15% and/or mRSS deterioration>3.5 and/or first occurrence of digital ulcers and/or death)] at 3 year follow-up. CONCLUSIONS: Dilatation score performs best of all semi-quantitative NVC parameters in diagnosing SSc. In addition, our study confirms earlier reports that worse capillaroscopy pattern at baseline correlates with higher likelihood for adverse prognosis. FAU - Bournia, Vasiliki Kalliopi AU - Bournia VK AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Kottas, Konstantinos AU - Kottas K AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Panopoulos, Stylianos AU - Panopoulos S AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Konstantonis, George AU - Konstantonis G AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Iliopoulos, Alexios AU - Iliopoulos A AD - Department of Rheumatology, Veterans Administration Hospital (NIMTS), Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Tektonidou, Maria G AU - Tektonidou MG AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Sfikakis, Petros P AU - Sfikakis PP AD - First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. FAU - Vlachoyiannopoulos, Panayiotis G AU - Vlachoyiannopoulos PG AD - Department of Pathophysiology and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. pvlah@med.uoa.gr. LA - eng PT - Journal Article DEP - 20200114 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Capillaries MH - Humans MH - Microscopic Angioscopy MH - Nails MH - Prognosis MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2020/01/24 06:00 MHDA- 2020/09/18 06:00 CRDT- 2020/01/24 06:00 PHST- 2019/02/27 00:00 [received] PHST- 2019/05/06 00:00 [accepted] PHST- 2020/01/24 06:00 [pubmed] PHST- 2020/09/18 06:00 [medline] PHST- 2020/01/24 06:00 [entrez] AID - 14046 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2020 May-Jun;38 Suppl 125(3):29-39. Epub 2020 Jan 14. PMID- 37332054 OWN - NLM STAT- MEDLINE DCOM- 20231130 LR - 20260120 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 75 IP - 12 DP - 2023 Dec TI - Proposed Response Parameters for Twelve-Month Drug Trial in Juvenile Systemic Sclerosis: Results of the Hamburg International Consensus Meetings. PG - 2453-2462 LID - 10.1002/acr.25171 [doi] AB - OBJECTIVE: Juvenile systemic sclerosis (SSc) is an orphan disease, associated with high morbidity and mortality. New treatment strategies are much needed, but clearly defining appropriate outcomes is necessary if successful therapies are to be developed. Our objective here was to propose such outcomes. METHODS: This proposal is the result of 4 face-to-face consensus meetings with a 27-member multidisciplinary team of pediatric rheumatologists, adult rheumatologists, dermatologists, pediatric cardiologists, pulmonologists, gastroenterologists, a statistician, and patients. Throughout the process, we reviewed the existing adult data in this field, the more limited pediatric literature for juvenile SSc outcomes, and data from 2 juvenile SSc patient cohorts to assist in making informed, data-driven decisions. The use of items for each domain as an outcome measure in an open label 12-month clinical trial of juvenile SSc was voted and agreed upon using a nominal group technique. RESULTS: After voting, the domains agreed on were global disease activity, skin, Raynaud's phenomenon, digital ulcers, musculoskeletal, cardiac, pulmonary, renal, and gastrointestinal involvement, and quality of life. Fourteen outcome measures had 100% agreement, 1 item had 91% agreement, and 1 item had 86% agreement. The domains of biomarkers and growth/development were moved to the research agenda. CONCLUSION: We reached consensus on multiple domains and items that should be assessed in an open label, 12-month clinical juvenile SSc trial as well as a research agenda for future development. CI - © 2023 American College of Rheumatology. FAU - Foeldvari, Ivan AU - Foeldvari I AUID- ORCID: 0000-0003-0659-5298 AD - Schön Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Torok, Kathryn S AU - Torok KS AUID- ORCID: 0000-0002-1662-143X AD - University of Pittsburgh and Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Anton, Jordi AU - Anton J AUID- ORCID: 0000-0002-8792-4219 AD - Hospital Sant Joan de Déu and Universitat de Barcelona, Barcelona, Spain. FAU - Blakley, Michael AU - Blakley M AD - Indiana University School of Medicine and Riley Hospital for Children at IU Health, Indianapolis. FAU - Constantin, Tamás AU - Constantin T AD - Semmelweis University, Budapest, Hungary. FAU - Curran, Megan AU - Curran M AD - University of Colorado School of Medicine and Children's Hospital Colorado, Aurora. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - University of Genoa and IRCCS San Martino Polyclinic Hospital, Genoa, Italy. FAU - Denton, Christopher AU - Denton C AUID- ORCID: 0000-0003-3975-8938 AD - Royal Free London NHS Foundation Trust, London, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma & Raynaud's United Kingdom, London, UK. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - University of Milan, ASST G. Pini, Milan, Italy. FAU - Li, Suzanne C AU - Li SC AD - Hackensack University Medical Center, Hackensack, New Jersey. FAU - Němcová, Dana AU - Němcová D AD - Charles University, Prague, Czech Republic. FAU - Orteu, Catherine AU - Orteu C AD - Royal Free London, London, UK. FAU - Pilkington, Clarissa AU - Pilkington C AD - Great Ormond Street Hospital, London, UK. FAU - Smith, Vanessa AU - Smith V AD - Ghent University, Ghent University Hospital, VIB Inflammation Research Center, and ERN ReCONNET, Ghent, Belgium. FAU - Stevens, Anne AU - Stevens A AD - Children's Hospital Research Institute and University of Washington, Seattle, and Janssen Pharmaceutical Companies of Johnson & Johnson, Spring House, Pennsylvania. FAU - Klotsche, Jens AU - Klotsche J AUID- ORCID: 0000-0002-2954-5755 AD - German Rheumatism Research Center, Berlin, Germany. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. FAU - Costa-Reis, Patrícia AU - Costa-Reis P AD - Hospital de Santa Maria, Faculdade de Medicina, and Universidade de Lisboa, Lisbon, Portugal. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - University of Leeds and Leeds Teaching Hospital Trust, Leeds, UK. FAU - Hinrichs, Bernd AU - Hinrichs B AD - Asklepios Klinik Nord-Heidberg, Hamburg, Germany. FAU - Kasapcopur, Ozgur AU - Kasapcopur O AUID- ORCID: 0000-0002-1125-7720 AD - Cerrahpasa Medical School and Istanbul University-Cerrahpasa, Istanbul, Turkey. FAU - Pain, Clare AU - Pain C AD - Alder Hey Children's Foundation NHS Trust, Liverpool, UK. FAU - Ruperto, Nicolino AU - Ruperto N AUID- ORCID: 0000-0001-8407-7782 AD - IRCCSG Istituto G. Gaslini, Genoa, Italy. FAU - Zheng, Alison AU - Zheng A AD - Chinese Organization for Scleroderma, Chengdu City, Sichuan Province, China. FAU - Furst, Daniel E AU - Furst DE AUID- ORCID: 0000-0001-7857-2373 AD - University of California, Los Angeles, University of Washington, Seattle, and University of Florence, Florence, Italy. LA - eng PT - Consensus Statement PT - Journal Article PT - Review DEP - 20230912 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - digital ulcers RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Adult MH - Quality of Life MH - Child MH - Humans MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/diagnosis/drug therapy/complications MH - Skin Ulcer MH - Scleroderma, Localized EDAT- 2023/06/19 00:42 MHDA- 2023/11/30 06:43 CRDT- 2023/06/18 23:37 PHST- 2023/04/03 00:00 [revised] PHST- 2022/11/24 00:00 [received] PHST- 2023/06/08 00:00 [accepted] PHST- 2023/11/30 06:43 [medline] PHST- 2023/06/19 00:42 [pubmed] PHST- 2023/06/18 23:37 [entrez] AID - 10.1002/acr.25171 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2023 Dec;75(12):2453-2462. doi: 10.1002/acr.25171. Epub 2023 Sep 12. PMID- 25736140 OWN - NLM STAT- MEDLINE DCOM- 20160531 LR - 20181113 IS - 1477-0962 (Electronic) IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 24 IP - 10 DP - 2015 Sep TI - Diagnosis and risk stratification in patients with anti-RNP autoimmunity. PG - 1057-66 LID - 10.1177/0961203315575586 [doi] AB - INTRODUCTION: Anti-RNP autoantibodies occur either in mixed connective tissue disease (MCTD) (with a frequently favorable prognosis), or in systemic lupus erythematosus (SLE) cases with aggressive major organ disease. It is uncertain how to assess for the risk of severe disease in anti-RNP + patients. METHODS: Following institutional review board-approved protocols, clinical data and blood were collected from patients with known or suspected anti-RNP autoimmunity and normal controls in a cohort study. Samples were screened for parameters of immune activation. Groups were compared based on clinical diagnoses, disease classification criteria, disease activity and specific end-organ clinical manifestations. RESULTS: Ninety-seven per cent of patients satisfying Alarcon-Segovia MCTD criteria also met Systemic Lupus International Collaborating Clinic (SLICC) SLE criteria, while 47% of the anti-RNP + SLE patients also met MCTD criteria. Among SLICC SLE patients, MCTD criteria were associated with reduced rates of renal disease (odds ratio (OR) 4.3, 95% confidence interval (CI) 1.3-14.0), increased rates of Raynaud's phenomenon (OR 3.5, 95% CI 1.3-9.5) and increased serum B-cell maturation antigen, transmembrane activator and CAML interactor and TNFα levels. Circulating immune markers and markers of type I interferon activation were not effective at distinguishing clinical subgroups. CONCLUSIONS: Among anti-RNP patients, the question of MCTD versus SLE is not either/or: most MCTD patients also have lupus. MCTD classification criteria (but not a broad set of immune markers) distinguish a subset of SLE patients at reduced risk for renal disease. CI - © The Author(s) 2015. FAU - Carpintero, M F AU - Carpintero MF AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA. FAU - Martinez, L AU - Martinez L AD - Miami VA Medical Center, Miami, USA. FAU - Fernandez, I AU - Fernandez I AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA. FAU - Romero, A C Garza AU - Romero AC AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA. FAU - Mejia, C AU - Mejia C AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA. FAU - Zang, Y J AU - Zang YJ AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA. FAU - Hoffman, R W AU - Hoffman RW AD - Eli Lilly and Company, Indianapolis, USA. FAU - Greidinger, E L AU - Greidinger EL AD - Division of Rheumatology, University of Miami Miller School of Medicine, Miami, USA Miami VA Medical Center, Miami, USA egreidinger@med.miami.edu. LA - eng GR - I01 BX002047/BX/BLRD VA/United States GR - R01 AR043308/AR/NIAMS NIH HHS/United States GR - AR48805/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20150302 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Ribonucleoproteins) RN - 63231-63-0 (RNA) SB - IM MH - Adult MH - Antibodies, Antinuclear/immunology MH - Autoantibodies/*blood/metabolism MH - Biomarkers/blood MH - Case-Control Studies MH - Cohort Studies MH - Female MH - Gene Expression MH - Humans MH - Lupus Erythematosus, Systemic/blood/diagnosis/immunology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/blood/*diagnosis/immunology MH - RNA/blood/genetics MH - Raynaud Disease/immunology MH - Ribonucleoproteins/*antagonists & inhibitors/immunology MH - Risk Assessment/methods PMC - PMC4529793 MID - NIHMS663271 OTO - NOTNLM OT - Systemic lupus erythematosus OT - classification criteria OT - inflammation OT - mixed connective tissue disease EDAT- 2015/03/05 06:00 MHDA- 2016/06/01 06:00 PMCR- 2016/09/01 CRDT- 2015/03/05 06:00 PHST- 2014/07/14 00:00 [received] PHST- 2015/02/05 00:00 [accepted] PHST- 2015/03/05 06:00 [entrez] PHST- 2015/03/05 06:00 [pubmed] PHST- 2016/06/01 06:00 [medline] PHST- 2016/09/01 00:00 [pmc-release] AID - 0961203315575586 [pii] AID - 10.1177/0961203315575586 [doi] PST - ppublish SO - Lupus. 2015 Sep;24(10):1057-66. doi: 10.1177/0961203315575586. Epub 2015 Mar 2. PMID- 39689020 OWN - NLM STAT- MEDLINE DCOM- 20250506 LR - 20260514 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - 5 DP - 2025 May 1 TI - The role of nailfold video-capillaroscopy in the assessment of dermatomyositis. PG - 2987-2994 LID - 10.1093/rheumatology/keae677 [doi] AB - OBJECTIVES: The clinical manifestations of dermatomyositis (DM) are diverse, nailfold video-capillaroscopy (NVC) can reflect microangiopathy, a process believed to contribute significantly to the clinical manifestations of DM. We aimed to explore the distinctive alterations and implications of nailfold capillary for evaluating disease progression in individuals with DM. METHODS: We gathered clinical data from 76 DM patients who underwent NVC in the Affiliated Hospital of Nantong University between September 2017 and September 2022. Additionally, we recruited 26 anti-synthase antibody syndrome (ASS) patients and 33 systemic sclerosis (SSc) patients as controls. Utilizing an unsupervised machine learning method (hierarchical clustering analysis) to categorized patients based on NVC results and compared clinical characteristics and survival outcomes. The follow-up period ended in December 2022. RESULTS: Anomalous NVC patterns were detected in 73.7% of the 76 DM patients and manifested as diminished capillary density and abnormal capillary morphology. Patients displaying abnormal NVC findings exhibited a significantly higher prevalence of Raynaud's phenomenon and a greater likelihood of being managed with triple combination therapy. Compared with SSc patients, milder NVC changes were observed in patients with DM, nonetheless, NVC abnormalities were more prominent in DM patients when contrasted with individuals with ASS. Furthermore, the patients were classified into two different clusters according to NVC data. Patients in cluster 1 were more likely to develop interstitial lung disease (ILD). Survival outcomes did not differ significantly between the two clusters. CONCLUSION: DM patients can experience varying degrees of aberrant NVC patterns, which can impact ILD risk and warrant clinical vigilance. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Xu, Hui AU - Xu H AD - Department of Rheumatology, Affiliated Hospital of Nantong University, Nantong, China. FAU - Qian, Jie AU - Qian J AUID- ORCID: 0000-0001-6461-3082 AD - Department of Rheumatology, Affiliated Hospital of Nantong University, Nantong, China. LA - eng GR - Z-2018-40/China International Medical Foundation/ GR - MSZ18108/Nantong Science and Technology Bureau/ GR - 2019JZ001/Special Clinical Basic Research Key Project of Nantong University/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Dermatomyositis/diagnostic imaging/diagnosis/physiopathology/complications MH - *Microscopic Angioscopy/methods MH - Male MH - Female MH - Middle Aged MH - Adult MH - Capillaries/diagnostic imaging MH - *Nails/blood supply MH - Scleroderma, Systemic/diagnostic imaging MH - Disease Progression MH - Raynaud Disease MH - Aged OTO - NOTNLM OT - cluster analysis OT - dermatomyositis OT - interstitial lung disease OT - nailfold video-capillaroscopy EDAT- 2024/12/17 18:24 MHDA- 2025/05/06 06:30 CRDT- 2024/12/17 13:13 PHST- 2024/11/22 00:00 [accepted] PHST- 2024/06/18 00:00 [received] PHST- 2025/05/06 06:30 [medline] PHST- 2024/12/17 18:24 [pubmed] PHST- 2024/12/17 13:13 [entrez] AID - 7926894 [pii] AID - 10.1093/rheumatology/keae677 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 May 1;64(5):2987-2994. doi: 10.1093/rheumatology/keae677. PMID- 27174391 OWN - NLM STAT- MEDLINE DCOM- 20170705 LR - 20170705 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 41 IP - 4 DP - 2016 Jul TI - [Macrovascular complications of systemic sclerosis: Prospective descriptive Doppler ultrasound study in 20 patients]. PG - 253-9 LID - S0398-0499(16)30011-7 [pii] LID - 10.1016/j.jmv.2016.04.001 [doi] AB - PURPOSE: Systemic sclerosis is characterized by cutaneous sclerosis, vascular disease and immunological dysfunction. The prevalence of macrovascular disease remains controversial. PATIENTS AND METHODS: This was a descriptive prospective single-center study conducted in the vascular medicine department of the University Hospital of Bordeaux from July 1 to September 1, 2015. All inpatients followed for systemic scleroderma were included. Each patient had a Doppler ultrasound of the supra-aortic, upper and lower limb, and digital arteries as well as the aorta. The main objective of the study was to describe the presence and location of arterial lesions in patients with systemic sclerosis. RESULTS: Of the twenty patients included, there were 13 women and 7 men; mean age was 58±16years. Patients exhibited wall thickening (n=16, 80%), calcified plaques (n=10, 50%), hemodynamically significant stenoses (n=3, 15%) and arterial occlusions (n=12, 60%). Ankle brachial pressure index (ABPI) was 0.98±0.16 on the right and 0.99±0.21 on the left. Two patients had ABPI<0.8. The mean brachial systolic blood pressure was 113±14mmHg. Arterial mapping (860 arterial sites) found wall thickening (n=93 arteries, 10%), calcified plaques (n=47, 5%), hemodynamically significant stenoses (n=7) and occlusions (n=22). Arterial occlusions were located in the ulnar arteries (n=2), the digital arteries (n=18), the posterior tibial artery (n=1) and the dorsalis pedis artery (n=1). CONCLUSION: The data of our study correlate with macrovascular disease described in the literature. This finding raises two questions: how does this concept integrate with the severity of Raynaud's phenomenon and the risk of digital ulcers and changes in patients' capillaroscopic landscape during follow-up? What is the cause of these vascular anomalies, some of which are very different from what is observed in atherosclerosis? CI - Copyright © 2016 Elsevier Masson SAS. All rights reserved. FAU - Doutrelon, C AU - Doutrelon C AD - Service de médecine vasculaire, groupe hospitalier Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux cedex, France. Electronic address: caro0313@gmail.com. FAU - Skopinski, S AU - Skopinski S AD - Service de médecine vasculaire, groupe hospitalier Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux cedex, France. FAU - Boulon, C AU - Boulon C AD - Service de médecine vasculaire, groupe hospitalier Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux cedex, France. FAU - Constans, J AU - Constans J AD - Service de médecine vasculaire, groupe hospitalier Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux cedex, France. LA - fre PT - Journal Article TT - Atteinte macrovasculaire de la sclérodermie systémique : étude descriptive prospective de 20 patients en échodoppler. DEP - 20160509 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Adult MH - Aged MH - Ankle Brachial Index MH - Autoimmune Diseases MH - Blood Pressure MH - Female MH - Fingers/blood supply MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease MH - Risk Factors MH - Scleroderma, Systemic/*complications MH - Skin Ulcer MH - Ulnar Artery MH - Ultrasonography, Doppler MH - Vascular Diseases/*diagnostic imaging/etiology OTO - NOTNLM OT - Lésions macrovasculaires OT - Macrovascular damage OT - Sclérodermie systémique OT - Systemic sclerosis EDAT- 2016/05/14 06:00 MHDA- 2017/07/06 06:00 CRDT- 2016/05/14 06:00 PHST- 2015/11/02 00:00 [received] PHST- 2016/04/02 00:00 [accepted] PHST- 2016/05/14 06:00 [entrez] PHST- 2016/05/14 06:00 [pubmed] PHST- 2017/07/06 06:00 [medline] AID - S0398-0499(16)30011-7 [pii] AID - 10.1016/j.jmv.2016.04.001 [doi] PST - ppublish SO - J Mal Vasc. 2016 Jul;41(4):253-9. doi: 10.1016/j.jmv.2016.04.001. Epub 2016 May 9. PMID- 30277860 OWN - NLM STAT- MEDLINE DCOM- 20190129 LR - 20260518 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 Suppl 113 IP - 4 DP - 2018 Jul-Aug TI - Gender differences in early systemic sclerosis patients: a report from the EULAR scleroderma trials and research group (EUSTAR) database. PG - 68-75 AB - OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets. METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. A group of patients with less than 3 years from the first SSc symptom, including Raynaud's phenomenon, was also analysed. SSc related variables, including antibodies, SSc subsets, disease activity and organ involvement were included. Descriptive and bivariate analyses were performed. RESULTS: A total of 1,027 patients were included, 90% Caucasian, 80% women, and 40% with diffuse cutaneous disease. In early stages of SSc, men showed more frequently than women active disease, diffuse cutaneous subset, anti-Scl-70 antibodies, elevated acute phase reactants, muscular and pulmonary involvement. Differences between men and women were confirmed in the limited, but not in the diffuse SSc subset. The results were similar when 650 patients with less than three years from the first SSc symptom, including Raynaud's phenomenon, were analysed. CONCLUSIONS: In early stages of SSc, men present signs and symptoms of more severe disease. In the limited disease subset, men might appear with clinical features and organ involvement similar to those of the diffuse subgroup. In clinical practice, the identification of such differences might help to select the appropriate management for each particular patient. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Spain. carreira@h12o.es. FAU - Carmona, Loreto AU - Carmona L AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Joven, Beatriz E AU - Joven BE AD - Servicio de Reumatología, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Spain. FAU - Loza, Estíbaliz AU - Loza E AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Andreu, Jose Luis AU - Andreu JL AD - Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology, University of Lübeck, Germany. FAU - Vettori, Serena AU - Vettori S AD - U.O.C. di Reumatologia, Dipartimento di Internistica Clinica e Sperimentale "F.Magrassi-A-Lanzara", Seconda Università degli Studi di Napoli, Italy. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B. Shine Rheumatology Unit, Rambam Health Care Campus and Rappaport Faculty of Medicine-Technion, Haifa, Israel. FAU - Airò, Paolo AU - Airò P AD - U.O. Reumatologia e Immunologia Clinica. Spedali Civili, Brescia, Italy. FAU - Walker, Ulrich A AU - Walker UA AD - Rheumatologische Universitätsklinik, Felix Platter Spital, Basel, Switzerland. FAU - Damjanov, Nemanja AU - Damjanov N AD - University of Belgrade School of Medicine, Institute of Rheumatology Belgrade, Serbia. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Florence, Italy. FAU - Ananieva, Lidia P AU - Ananieva LP AD - Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia. FAU - Rednic, Simona AU - Rednic S AD - Clinica Reumatologie, University of Medicine & Pharmacy 'Iuliu Hatieganu', Cluj-Napoca, Romania. FAU - Czirják, László AU - Czirják L AD - Department of Immunology and Rheumatology, Faculty of Medicine, University of Pécs, Hungary. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zürich, Switzerland. FAU - Farge, Dominique AU - Farge D AD - Department of Internal Medicine, Hopital Saint-Louis, Paris, France. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University Hospital, Sweden. FAU - Corrado, Ada AU - Corrado A AD - U.O. Reumatologia-Università degli Studi di Foggia, Ospedale 'Col. D'Avanzo', Foggia, Italy. FAU - Caramaschi, Paola AU - Caramaschi P AD - Rheumatology Unit, AOUI, Verona, Italy. FAU - Tikly, Mohammed AU - Tikly M AD - Rheumatology Unit, Department of Medicine Chris Hani Bardgwanath, Hospital and University of the Witwatersrand, Johannesburg, South Africa. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A Department, Cochin Hospital, APHP, Paris Descartes University, Paris, France. CN - the EUSTAR co-authors LA - eng PT - Comparative Study PT - Journal Article DEP - 20180929 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Acute-Phase Proteins) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Nuclear Proteins) RN - 0 (Scl 70 antigen, human) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Acute-Phase Proteins/analysis MH - Autoantibodies/blood MH - Biomarkers/blood MH - Cross-Sectional Studies MH - DNA Topoisomerases, Type I MH - Databases, Factual MH - Disease Progression MH - Female MH - *Health Status Disparities MH - Humans MH - Lung Diseases/diagnosis/etiology MH - Male MH - Nuclear Proteins/immunology MH - Prognosis MH - Raynaud Disease/diagnosis/etiology MH - Risk Factors MH - Scleroderma, Diffuse/blood/complications/*diagnosis/immunology MH - Scleroderma, Limited/blood/complications/*diagnosis/immunology MH - Severity of Illness Index MH - Sex Factors FIR - Valentini, Gabriele IR - Valentini G IRAD- Università di Napoli, Italy. FIR - Hanes, Jörg IR - Hanes J IRAD- Medizinische Universitätsklinik Abt. II (Oncologie, Hämatologie, Rheumatologie Immunologie, Pulmonologie) Tübingen, Germany. FIR - Gabrielli, Armando IR - Gabrielli A IRAD- Istituto di Clinica Medica Generale, Ematologia ed Immunologia Clinica, Università Politecnica delle Marche, Ancona, Italy. FIR - Lapadula, Giovanni IR - Lapadula G IRAD- Rheumatology Unit-DiMIMP, School of Medicine, University of Bari, Italy. FIR - Heitmann, Stefan IR - Heitmann S IRAD- Department of Rheumatology, Marienhospital Stuttgart, Germany. FIR - Valesini, Guido IR - Valesini G IRAD- Divisione di Reumatologia, Università di Roma La Sapienza, Roma, Italy. FIR - von Mühlen, Carlos Alberto IR - von Mühlen CA IRAD- RheumaCllnic, Porto Alegre, Brazil. FIR - Kucharz, Eugene J IR - Kucharz EJ IRAD- Department of Internal Medicine and Rheumatology, Medical University of Silesia, Katowice, Poland. FIR - Cozzi, Franco IR - Cozzi F IRAD- Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, Italy. FIR - Rozman, Blaz IR - Rozman B IRAD- Division of Internal Medicine, Department of Rheumatology, University Medical Center Ljubljana, Slovenia. FIR - Pellerito, Raffaele IR - Pellerito R IRAD- Centro di Reumatologia, Ospedale Mauriziano, Torino, Italy. FIR - Müller-Ladner, Ulf IR - Müller-Ladner U IRAD- Rheumatologie und Klinische Immunologie, Kerckhoff-Klinik Bad Nauheim, Germany. FIR - Montecucco, Carlomaurizio IR - Montecucco C IRAD- Unità Operativa di Reumatologia, IRCCS Policlinico San Matteo, Pavia, Italy. FIR - Smith, Vanessa IR - Smith V IRAD- Department of Rheumatology, University of Gent, Belgium. FIR - Jimenez, Sergio IR - Jimenez S IRAD- Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA. FIR - Martinovic, Duska IR - Martinovic D IRAD- Department of Internal Clinic, Clinical Hospital of Split Spinciceva, Split, Croatia. FIR - Novak, Sudan IR - Novak S IRAD- Department of Rheumatology and Clinical Immunology, Medicine KBC, Rijeka, Croatia. FIR - Burkhardt, Harald IR - Burkhardt H IRAD- Rheumatologische Ambulanz, Medizinische Klinik III, Klinikum der Johann Wolfgang Goethe Universität, Frankfurt am Main, Germany. FIR - Mihai, Carmen M IR - Mihai CM IRAD- Department of Internal Medicine and Rheumatology Clinic, Ion Cantacuzino Clinical Hospital, Bucharest, Romania. FIR - Pozzi, Maria Rosa IR - Pozzi MR IRAD- Dipartimento di Medicina, Ospedale San Gerardo, Monza, Italy. FIR - Vacca, Alessandra IR - Vacca A IRAD- II Chair of Rheumatology, University of Cagliari, Policlinico Universitario, Monserrato, Cagliari, Italy. FIR - Radominski, Sebastião Cezar IR - Radominski SC IRAD- Hospital de Clinicas da Universidade Federal do Paraná, Curitiba-Paraná, Brazil. FIR - Chizzolini, Carlo IR - Chizzolini C IRAD- Immunology and Allergy, University Hospital, Geneva, Switzerland. FIR - Krasowska, Dorota IR - Krasowska D IRAD- Department of Dermatology, Medical University of Lublin, Poland. FIR - Mouthon, Luc IR - Mouthon L IRAD- Department of Internal Medicine, Hôpital Cochin, Paris, France. FIR - Westhovens, Rene IR - Westhovens R IRAD- Department of Rheumatology, Catholic University of Leuven, Belgium. FIR - Mallia, Carmen IR - Mallia C IRAD- Stella Maris, Balzan, Malta. FIR - Wiland, Piotr IR - Wiland P IRAD- Department of Rheumatology and Internal Diseases, Wroclaw University of Medicine, Wroclaw, Poland. FIR - Kummel-Lorenz, Brigitte IR - Kummel-Lorenz B IRAD- Endokrinologikum Frankfurt, Germany. FIR - Vlachoyiannopoulos, P IR - Vlachoyiannopoulos P IRAD- Department of Pathophysiology, Medical School, National University of Athens, Greece. FIR - Hachulla, Eric IR - Hachulla E IRAD- Department of Internal Medicine, Hôpital Claude Huriez, Lille, France. FIR - Üprus, Maria IR - Üprus M IRAD- Department of Rheumatology, East-Tallin Central Hospital, Tallin, Estonia. FIR - Sulli, Alberto IR - Sulli A IRAD- Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Italy. FIR - Stork, Jiri IR - Stork J IRAD- Department of Dermatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic. FIR - Denton, Christopher P IR - Denton CP IRAD- Centre for Rheumatology, Royal Free and University College, London Medical School, London, UK. FIR - Ortiz, Vera IR - Ortiz V IRAD- Rheumatology Granollers General Hospital, Barcelona, Spain. FIR - Stamenkovic, Bojana IR - Stamenkovic B IRAD- Institute for Prevention, Treatment and Rehabilitation, Rheumatic and Cardiovascular Disease, Niska Banja, Serbia and Montenegro. FIR - de la Puente, Carlos IR - de la Puente C IRAD- Servicio de Reumatología, Hospital Ramon y Cajal, Madrid, Spain. FIR - Meroni, Pierluigi IR - Meroni P IRAD- Reumatologia, Università di Milano, Istituto Ortopedico G. Pini, Milano, Italy. FIR - Popa, Sergei IR - Popa S IRAD- Department of Rheumatology, Republican Clinical Hospital, Chisinau, Republic of Moldova. FIR - Solanki, Kamal IR - Solanki K IRAD- Rheumatology Unit, Waikato University Hospital, Hamilton City, New Zealand. FIR - Becvar, Radim IR - Becvar R IRAD- Institute of Rheumatology, 1st Medical School, Charles University, Prague, Czech Republic. FIR - Seidel, Matthias IR - Seidel M IRAD- Department of Rheumatology, Medizinische Universitäts-Poliklinik, Bonn, Germany. FIR - Pereira da Silva, José Antonio IR - Pereira da Silva JA IRAD- Department of Rheumatology, Hospitais da Universidade, Coimbra, Portugal. FIR - Selmi, Carlo Francesco IR - Selmi CF IRAD- Division of Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, BIOMETRA Department, University of Milan, Italy. FIR - Nielsen, Henrik IR - Nielsen H IRAD- Department of Rheumatology, University Hospital of Gentofte, Hellerup, Denmark. FIR - Aringer, Martin IR - Aringer M IRAD- Division of Rheumatology, Department of Medicine III, University Medical Center Carl Gustav Carus, Technical University of Dresden, Germany. FIR - Anic, Branimir IR - Anic B IRAD- Division of Clinical Immunology and Rheumatology, Department of Medicine, University Hospital Centre Zagreb, Croatia. FIR - Yavuz, Sule IR - Yavuz S IRAD- Department of Rheumatology, University of Marmara, Istanbul, Turkey. EDAT- 2018/10/03 06:00 MHDA- 2019/01/30 06:00 CRDT- 2018/10/03 06:00 PHST- 2017/04/21 00:00 [received] PHST- 2018/03/09 00:00 [accepted] PHST- 2018/10/03 06:00 [entrez] PHST- 2018/10/03 06:00 [pubmed] PHST- 2019/01/30 06:00 [medline] AID - 11860 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jul-Aug;36 Suppl 113(4):68-75. Epub 2018 Sep 29. PMID- 34471968 OWN - NLM STAT- MEDLINE DCOM- 20220105 LR - 20240823 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 41 IP - 1 DP - 2022 Jan TI - A 4-week comparison of capillaroscopy changes, healing effect, and cost-effectiveness of botulinum toxin-A vs prostaglandin analog infusion in refractory digital ulcers in systemic sclerosis. PG - 95-104 LID - 10.1007/s10067-021-05900-7 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a systemic multi-organ disease. Raynaud's phenomenon (RP) and digital ulcers (DUs) in SSc patients can be resistant to usual treatments. We studied the clinical benefits, capillaroscopy changes, and cost-effectiveness of local injection of botulinum toxin-A (BTX-A) and intravenous prostaglandin analogs (iloprost/alprostadil) in patients with SSc with resistant DUs. METHOD: In a clinical trial study, we evaluated 26 patients fulfilling the ACR/EULAR SSc criteria with resistant DUs. Visual analog scale of pain and RP, skin color and type of ulcers, and capillaroscopy were assessed before and 1 month after treatment. In the first group, 20 units of BTX-A was injected at the base of each involved fingers by a dermatologist. In the second group, 20 µg iloprost or 60 µg alprostadil was infused daily. The cost of these treatments was compared. RESULT: In 26 patients (43 fingers), there were 16 patients (22 fingers) in the BTX-A and 10 patients (21 fingers) in the prostaglandin group. In 95.5% of the BTX-A and 90.5% of the prostaglandin group, the ulcers were healed. In both groups, a significant decrease in pain was seen (p < 0.0001). Capillaroscopy patterns in both groups were not changed although the microhemorrhages disappeared significantly (p value: BTX-A: 0.03 and prostaglandin: 0.002). The cost was significantly lower in the BTX-A injection group (p < 0.0001). CONCLUSION: Both BTX-A and prostaglandins helped in the healing and pain control of DUs. In capillaroscopy, microhemorrhages were significantly decreased in both groups. In the BTX-A group, the cost was significantly lower as an outpatient treatment and was more time-saving. KEY MESSAGES: • BTX-A and prostaglandin analogs both contributed to the healing of digital tip ulcers and improving the pain • In capillaroscopy, microhemorrhages were significantly decreased or disappeared after both treatments • There was no significant side effect in both groups • Comparing both groups, in the BTX-A group, the cost was significantly lower when performed on an outpatient treatment and more time-saving. CI - © 2021. International League of Associations for Rheumatology (ILAR). FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AUID- ORCID: 0000-0003-0522-7186 AD - Department of Internal Medicine, Division of Rheumatology, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. shenavande@sums.ac.ir. FAU - Sepaskhah, Mozhdeh AU - Sepaskhah M AD - Molecular Dermatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Dehghani, Sanaz AU - Dehghani S AD - Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Nazarinia, MohammadAli AU - Nazarinia M AD - Department of Internal Medicine, Division of Rheumatology, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. LA - eng GR - 1396-01-01-15726/Shiraz University of Medical Sciences/ PT - Clinical Trial PT - Journal Article DEP - 20210901 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Prostaglandins) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - JED5K35YGL (Iloprost) SB - IM CIN - Int J Rheum Dis. 2023 Feb;26(2):187-189. doi: 10.1111/1756-185X.14487. PMID: 36703271 MH - *Botulinum Toxins, Type A/therapeutic use MH - Cost-Benefit Analysis MH - Fingers/diagnostic imaging MH - Humans MH - Iloprost MH - Microscopic Angioscopy MH - Prostaglandins MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/complications/diagnostic imaging/drug therapy MH - *Skin Ulcer/drug therapy/etiology MH - Ulcer PMC - PMC8409478 OTO - NOTNLM OT - Botulinum toxin OT - Capillaroscopy OT - Digital ulcer OT - Prostaglandin analogs OT - Systemic sclerosis EDAT- 2021/09/03 06:00 MHDA- 2022/01/06 06:00 PMCR- 2021/09/01 CRDT- 2021/09/02 07:03 PHST- 2021/04/23 00:00 [received] PHST- 2021/08/28 00:00 [accepted] PHST- 2021/08/25 00:00 [revised] PHST- 2021/09/03 06:00 [pubmed] PHST- 2022/01/06 06:00 [medline] PHST- 2021/09/02 07:03 [entrez] PHST- 2021/09/01 00:00 [pmc-release] AID - 10.1007/s10067-021-05900-7 [pii] AID - 5900 [pii] AID - 10.1007/s10067-021-05900-7 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Jan;41(1):95-104. doi: 10.1007/s10067-021-05900-7. Epub 2021 Sep 1. PMID- 29214548 OWN - NLM STAT- MEDLINE DCOM- 20180907 LR - 20220408 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 4 DP - 2018 Apr TI - First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study. PG - 999-1009 LID - 10.1007/s10067-017-3936-7 [doi] AB - The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis. FAU - Rubio-Rivas, Manuel AU - Rubio-Rivas M AD - Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. mrubio@bellvitgehospital.cat. FAU - Corbella, Xavier AU - Corbella X AD - Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. AD - Faculty of Medicine and Health Sciences, Universitat Internacional de Catalunya, Barcelona, Spain. FAU - Pestaña-Fernández, Melany AU - Pestaña-Fernández M AD - Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain. FAU - Tolosa-Vilella, Carles AU - Tolosa-Vilella C AD - Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain. FAU - Guillen-Del Castillo, Alfredo AU - Guillen-Del Castillo A AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Colunga-Argüelles, Dolores AU - Colunga-Argüelles D AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Trapiella-Martínez, Luis AU - Trapiella-Martínez L AD - Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain. FAU - Iniesta-Arandia, Nerea AU - Iniesta-Arandia N AD - Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Clínic, Barcelona, Spain. FAU - Castillo-Palma, María Jesús AU - Castillo-Palma MJ AD - Collagenosis and Pulmonary Hypertension Unit, Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain. FAU - Sáez-Comet, Luis AU - Sáez-Comet L AD - Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain. FAU - Egurbide-Arberas, María Victoria AU - Egurbide-Arberas MV AD - Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Department of Internal Medicine, Hospital Universitario San Cecilio, Granada, Spain. FAU - Freire, Mayka AU - Freire M AD - Thrombosis and Vasculitis Unit, Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Vigo, Spain. FAU - Vargas-Hitos, Jose Antonio AU - Vargas-Hitos JA AD - Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain. FAU - Ríos-Blanco, Juan José AU - Ríos-Blanco JJ AD - Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain. FAU - Todolí-Parra, Jose Antonio AU - Todolí-Parra JA AD - Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain. FAU - Rodríguez-Carballeira, Mónica AU - Rodríguez-Carballeira M AD - Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain. FAU - Marín-Ballvé, Adela AU - Marín-Ballvé A AD - Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Segovia-Alonso, Pablo AU - Segovia-Alonso P AD - Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain. FAU - Pla-Salas, Xavier AU - Pla-Salas X AD - Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain. FAU - Madroñero-Vuelta, Ana Belén AU - Madroñero-Vuelta AB AD - Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain. FAU - Ruiz-Muñoz, Manuel AU - Ruiz-Muñoz M AD - Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain. FAU - Fonollosa-Pla, Vicent AU - Fonollosa-Pla V AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. FAU - Simeón-Aznar, Carmen Pilar AU - Simeón-Aznar CP AD - Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. CN - RESCLE investigators, Autoimmune Diseases Study Group (GEAS) FAU - Callejas Moraga, E AU - Callejas Moraga E FAU - Calvo, E AU - Calvo E FAU - Carbonell, C AU - Carbonell C FAU - Castillo, M J AU - Castillo MJ FAU - Chamorro, A J AU - Chamorro AJ FAU - Colunga, D AU - Colunga D FAU - Corbella, X AU - Corbella X FAU - Egurbide, M V AU - Egurbide MV FAU - Espinosa, G AU - Espinosa G FAU - Fonollosa, V AU - Fonollosa V FAU - Freire, M AU - Freire M FAU - García Hernández, F J AU - García Hernández FJ FAU - González León, R AU - González León R FAU - Guillén Del Castillo, A AU - Guillén Del Castillo A FAU - Iniesta, N AU - Iniesta N FAU - Lorenzo, R AU - Lorenzo R FAU - Madroñero, A B AU - Madroñero AB FAU - Marí, B AU - Marí B FAU - Marín, A AU - Marín A FAU - Ortego-Centeno, N AU - Ortego-Centeno N FAU - Pérez Conesa, M AU - Pérez Conesa M FAU - Pestaña, M AU - Pestaña M FAU - Pla, X AU - Pla X FAU - Ríos Blanco, J J AU - Ríos Blanco JJ FAU - Rodríguez Carballeira, M AU - Rodríguez Carballeira M FAU - Rubio Rivas, M AU - Rubio Rivas M FAU - Ruiz Muñoz, M AU - Ruiz Muñoz M FAU - Sáez Comet, L AU - Sáez Comet L FAU - Segovia, P AU - Segovia P FAU - Simeón, C P AU - Simeón CP FAU - Soto, A AU - Soto A FAU - Tarí, E AU - Tarí E FAU - Todolí, J A AU - Todolí JA FAU - Tolosa, C AU - Tolosa C FAU - Trapiella, L AU - Trapiella L FAU - Vargas Hitos, J A AU - Vargas Hitos JA FAU - Verdejo, G AU - Verdejo G LA - eng PT - Journal Article DEP - 20171207 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM EIN - Clin Rheumatol. 2018 Aug;37(8):2303-2304. doi: 10.1007/s10067-018-4179-y. PMID: 29931516 MH - Adult MH - Aged MH - Arthralgia/*etiology MH - Female MH - Humans MH - Hypertension, Pulmonary/*etiology MH - Lung Diseases, Interstitial/*etiology MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/*etiology MH - Registries MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*diagnosis/physiopathology MH - Severity of Illness Index MH - Symptom Assessment OTO - NOTNLM OT - Onset OT - Prognosis OT - Raynaud OT - Scleroderma OT - Systemic sclerosis EDAT- 2017/12/08 06:00 MHDA- 2018/09/08 06:00 CRDT- 2017/12/08 06:00 PHST- 2017/07/14 00:00 [received] PHST- 2017/11/27 00:00 [accepted] PHST- 2017/11/07 00:00 [revised] PHST- 2017/12/08 06:00 [pubmed] PHST- 2018/09/08 06:00 [medline] PHST- 2017/12/08 06:00 [entrez] AID - 10.1007/s10067-017-3936-7 [pii] AID - 10.1007/s10067-017-3936-7 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Apr;37(4):999-1009. doi: 10.1007/s10067-017-3936-7. Epub 2017 Dec 7. PMID- 26246178 OWN - NLM STAT- MEDLINE DCOM- 20160314 LR - 20260127 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 67 IP - 12 DP - 2015 Dec TI - Interpretation of an Extended Autoantibody Profile in a Well-Characterized Australian Systemic Sclerosis (Scleroderma) Cohort Using Principal Components Analysis. PG - 3234-44 LID - 10.1002/art.39316 [doi] AB - OBJECTIVE: To determine the relationships between systemic sclerosis (SSc)-related autoantibodies, as well as their clinical associations, in a well-characterized Australian patient cohort. METHODS: Serum from 505 Australian SSc patients were analyzed with a commercial line immunoassay (EuroLine; Euroimmun) for autoantibodies to centromere proteins CENP-A and CENP-B, RNA polymerase III (RNAP III; epitopes 11 and 155), the 90-kd nucleolar protein NOR-90, fibrillarin, Th/To, PM/Scl-75, PM/Scl-100, Ku, topoisomerase I (topo I), tripartite motif-containing protein 21/Ro 52, and platelet-derived growth factor receptor. Patient subgroups were identified by hierarchical clustering of the first 2 dimensions of a principal components analysis of quantitative autoantibody scores. Results were compared with detailed clinical data. RESULTS: A total of 449 of the 505 patients were positive for at least 1 autoantibody by immunoblotting. Heatmap visualization of autoantibody scores, along with principal components analysis clustering, demonstrated strong, mutually exclusive relationships between CENP, RNAP III, and topo I. Five patient clusters were identified: CENP, RNAP III strong, RNAP III weak, topo I, and other. Clinical features associated with CENP, RNAP III, and topo I were consistent with previously published reports concerning limited cutaneous and diffuse cutaneous SSc. A novel finding was the statistical separation of RNAP III into 2 clusters. Patients in the RNAP III strong cluster had an increased risk of gastric antral vascular ectasia, but a lower risk of esophageal dysmotility. Patients in the other cluster were more likely to be male and to have a history of smoking and a history of malignancy, but were less likely to have telangiectasia, Raynaud's phenomenon, and joint contractures. CONCLUSION: Five major autoantibody clusters with specific clinical and serologic associations were identified in Australian SSc patients. Subclassification and disease stratification using autoantibodies may have clinical utility, particularly in early disease. CI - © 2015, American College of Rheumatology. FAU - Patterson, K A AU - Patterson KA AD - Flinders University, Bedford Park, South Australia, and Commonwealth Scientific and Industrial Research Organization (CSIRO), Adelaide, South Australia, Australia. FAU - Roberts-Thomson, P J AU - Roberts-Thomson PJ AD - Flinders University and Flinders Medical Centre, Bedford Park, South Australia, and SA Pathology, Adelaide, South Australia, Australia. FAU - Lester, S AU - Lester S AD - Queen Elizabeth Hospital, Woodville, South Australia, Australia. FAU - Tan, J A AU - Tan JA AD - Flinders Medical Centre, Bedford Park, South Australia, Australia. FAU - Hakendorf, P AU - Hakendorf P AD - Flinders Medical Centre, Bedford Park, South Australia, Australia. FAU - Rischmueller, M AU - Rischmueller M AD - University of Adelaide, Adelaide, South Australia, and Queen Elizabeth Hospital, Woodville, South Australia, Australia. FAU - Zochling, J AU - Zochling J AD - Menzies Institute for Medical Research, Hobart, Tasmania, Australia. FAU - Sahhar, J AU - Sahhar J AD - Monash Health and Monash University, Melbourne, Victoria, Australia. FAU - Nash, P AU - Nash P AD - University of Queensland, Brisbane, Queensland, Australia. FAU - Roddy, J AU - Roddy J AD - Royal Perth Hospital, Perth, Western Australia, Australia. FAU - Hill, C AU - Hill C AD - University of Adelaide, Adelaide, South Australia, and Queen Elizabeth Hospital, Woodville, South Australia, Australia. FAU - Nikpour, M AU - Nikpour M AD - University of Melbourne and St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Stevens, W AU - Stevens W AD - St. Vincent's Hospital, Melbourne, Victoria, Australia. FAU - Proudman, S M AU - Proudman SM AD - University of Adelaide and Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - Walker, J G AU - Walker JG AD - Flinders University and Flinders Medical Centre, Bedford Park, South Australia, and Repatriation General Hospital, Daw Park, South Australia, Australia. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Centromere Protein A) RN - 0 (Centromere Protein B) RN - 0 (Chromosomal Proteins, Non-Histone) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - 0 (DNA-Binding Proteins) RN - EC 3.1.- (Exoribonucleases) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - EC 4.2.99.- (Ku Autoantigen) RN - 0 (Pol1 Transcription Initiation Complex Proteins) RN - EC 2.7.7.6 (RNA Polymerase III) RN - 0 (RNA-Binding Proteins) RN - EC 2.7.10.1 (Receptors, Platelet-Derived Growth Factor) RN - 0 (Ribonucleoproteins) RN - 0 (SS-A Antigen) RN - 0 (CENPA protein, human) RN - 0 (CENPB protein, human) RN - 0 (EXOSC9 protein, human) RN - 0 (fibrillarin) RN - 0 (transcription factor UBF) RN - EC 3.1.13.- (EXOSC10 protein, human) RN - EC 3.6.4.12 (Xrcc6 protein, human) SB - IM CIN - Arthritis Rheumatol. 2015 Dec;67(12):3101-3. doi: 10.1002/art.39310. PMID: 26245737 MH - Aged MH - Antigens, Nuclear/immunology MH - Australia MH - Autoantibodies/*immunology MH - Autoantigens/immunology MH - Centromere Protein A MH - Centromere Protein B/immunology MH - Chromosomal Proteins, Non-Histone/immunology MH - Cohort Studies MH - Contracture/etiology/immunology MH - DNA Topoisomerases, Type I/immunology MH - DNA-Binding Proteins/immunology MH - Esophageal Motility Disorders/etiology/immunology MH - Exoribonucleases/immunology MH - Exosome Multienzyme Ribonuclease Complex/immunology MH - Female MH - Gastric Antral Vascular Ectasia/etiology/immunology MH - Humans MH - Immunoblotting MH - Ku Autoantigen MH - Male MH - Middle Aged MH - Neoplasms/epidemiology MH - Pol1 Transcription Initiation Complex Proteins/immunology MH - Principal Component Analysis MH - RNA Polymerase III/immunology MH - RNA-Binding Proteins/immunology MH - Raynaud Disease/etiology/immunology MH - Receptors, Platelet-Derived Growth Factor/immunology MH - Ribonucleoproteins/immunology MH - Scleroderma, Systemic/complications/epidemiology/*immunology MH - Sex Factors MH - Smoking/epidemiology MH - Telangiectasis/etiology/immunology MH - SS-A Antigen EDAT- 2015/08/08 06:00 MHDA- 2016/03/15 06:00 CRDT- 2015/08/07 06:00 PHST- 2015/02/05 00:00 [received] PHST- 2015/07/30 00:00 [accepted] PHST- 2015/08/07 06:00 [entrez] PHST- 2015/08/08 06:00 [pubmed] PHST- 2016/03/15 06:00 [medline] AID - 10.1002/art.39316 [doi] PST - ppublish SO - Arthritis Rheumatol. 2015 Dec;67(12):3234-44. doi: 10.1002/art.39316. PMID- 17237932 OWN - NLM STAT- MEDLINE DCOM- 20071108 LR - 20161124 IS - 0936-8051 (Print) IS - 0936-8051 (Linking) VI - 127 IP - 5 DP - 2007 Jul TI - Macro- and microcirculatory assessment of cold sensitivity after traumatic finger amputation and microsurgical replantation. PG - 355-60 AB - INTRODUCTION: Finger replantations after traumatic amputation are associated with good prognosis and acceptable functional results. However, cold sensitivity is a common and sometimes disabling sequelae after digital replantation. The exact causes of cold intolerance are still unclear; neural as well as vascular mechanisms have been discussed. We examined the macro- and microvascular performance of replanted fingers using high-resolution color-coded sonography for the assessment of skin vessel density of the fingertips as well as nailfold capillary microscopy and laser Doppler anemometry. Subsequently, we correlated these findings with the presence of cold sensitivity of the replanted digits. PATIENTS AND METHODS: Thirty-seven patients (mean age 45 years; range 19-72) with 40 traumatic finger amputations and microsurgical replantations were studied. The mean time interval between amputation and examination was 57.7 months (range 13-95). Macro- and microvascular examination consisted of electronic oscillograms of both arms, photoplethysmograms of all fingers before and after cold test, duplex ultrasound of the finger arteries, high-resolution color-coded sonography of the fingertips and nailfold capillary microscopy with laser Doppler anemometry. RESULTS: Cold sensitivity was present in 33 (83%) of the 40 replanted fingers. Peripheral arterial disease of the upper extremity could be excluded as all oscillograms showed normal findings. A vasospastic reaction after cold test was documented in 74% (30 of 38) of the replanted fingers, compared to 24% (9 of 38) of the contralateral uninjured fingers. Raynaud's phenomenon was restricted to replanted fingers and occurred in 10 of 40 patients (25%). Compared with the contralateral fingertips, reduced skin vessel density was found in 27 of 36 (75%) replants. Nailfold capillary microscopy revealed uncharacteristic morphologic patterns. The capillary flow velocity was 0.28 +/- 0.12 mm/s in the replanted fingers and 0.48 +/- 0.23 mm/s in their unaffected counterparts (P < 0.001). Correlating these findings with the presence of cold intolerance, reduced skin vessel density in the fingertips was significantly different between cold-sensitive replants and those without cold sensitivity (P = 0.05). Reduced skin vessel density was not related to the extent of reconstruction of nerves (P = n.s.), arteries (P = n.s.) and veins (P = n.s.). CONCLUSIONS: Our results do not confirm hypotheses that cold sensitivity after finger replantations is caused by macrovascular problems nor do they support assumptions of a primary capillary microcirculatory failure. Our findings of reduced vessel density point towards diminished thermoregulatory capacities in the fingertips of cold-sensitive replanted digits. FAU - Klein-Weigel, Peter AU - Klein-Weigel P AD - Department of Angiology, DRK-Kliniken Berlin, Mark Brandenburg, Drontheinmer Str. 38-40, 13559 Berlin, Germany. p.klein-weigel@drk-kliniken-markbrandenburg.de FAU - Pavelka, Michaela AU - Pavelka M FAU - Dabernig, Jörg AU - Dabernig J FAU - Rein, Patrik AU - Rein P FAU - Kronenberg, Florian AU - Kronenberg F FAU - Fraedrich, Gustav AU - Fraedrich G FAU - Piza-Katzer, Hildegunde AU - Piza-Katzer H LA - eng PT - Journal Article DEP - 20070120 PL - Germany TA - Arch Orthop Trauma Surg JT - Archives of orthopaedic and trauma surgery JID - 9011043 SB - IM MH - Adult MH - Aged MH - Amputation, Traumatic MH - Blood Flow Velocity MH - *Cold Temperature MH - Female MH - Finger Injuries/*complications/*surgery MH - Fingers/*blood supply/diagnostic imaging/surgery MH - Follow-Up Studies MH - Humans MH - Male MH - Microcirculation MH - Microscopy MH - Microsurgery MH - Middle Aged MH - Photoplethysmography MH - Raynaud Disease/etiology MH - *Replantation MH - Sensation Disorders/*etiology MH - Ultrasonography, Doppler, Duplex EDAT- 2007/01/24 09:00 MHDA- 2007/11/09 09:00 CRDT- 2007/01/24 09:00 PHST- 2006/06/11 00:00 [received] PHST- 2007/01/24 09:00 [pubmed] PHST- 2007/11/09 09:00 [medline] PHST- 2007/01/24 09:00 [entrez] AID - 10.1007/s00402-007-0287-x [doi] PST - ppublish SO - Arch Orthop Trauma Surg. 2007 Jul;127(5):355-60. doi: 10.1007/s00402-007-0287-x. Epub 2007 Jan 20. PMID- 37578960 OWN - NLM STAT- MEDLINE DCOM- 20230817 LR - 20230817 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 18 IP - 8 DP - 2023 TI - Adipose derived stromal vascular fraction and fat graft for treating the hands of patients with systemic sclerosis. A randomized clinical trial. PG - e0289594 LID - 10.1371/journal.pone.0289594 [doi] LID - e0289594 AB - BACKGROUND: Systemic Sclerosis in the hand is characteristically evidenced by Raynaud's phenomenon, fibrosis of the skin, tendons, ligaments, and joints as well as digital ulcers with prolonged healing. Current medical treatment does not always cure these complications. Local adipose-derived stromal vascular fraction administration into the hands has been proposed as an emerging treatment due to its regenerative properties. The objective of this randomized controlled clinical trial was to evaluate the safety and clinical effects of fat micrografts plus adipose derived-stromal vascular fraction administration into the hands of patients with systemic sclerosis. METHODS: This was an open-label, monocentric, randomized controlled study. Twenty patients diagnosed with systemic sclerosis were assigned to the experimental or control group. Fat micrografts plus the adipose derived-stromal vascular fraction were injected into the right hand of experimental group patients. The control group continued to receive only medical treatment. Demographic, serologic data and disease severity were recorded. Digital oximetry, pain, Raynaud phenomenon, digital ulcers number, mobility, thumb opposition, vascular density of the nail bed, skin affection of the hand, serologic antibodies, hand function, and quality of life scores were evaluated in both groups. RESULTS: The results of the intervention were analyzed with the Wilcoxon rank test, and the differences between the control and experimental groups at 0 days and 168 days were analyzed with the Mann-Whitney U test. Adverse events were not observed in both groups. At the end of the study, statistically significant improvements were observed in pain levels (p<0.05) and number of digital ulcers (p<0.01) in the experimental vs control group. CONCLUSION: The injection of adipose derived-stromal vascular fraction plus fat micrografts is a reproducible, and safe technique. Pain and digital ulcers in the hands of patients with systemic sclerosis can be treated with this technique plus conventional medical treatment. CI - Copyright: © 2023 Iglesias et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. FAU - Iglesias, Martin AU - Iglesias M AUID- ORCID: 0000-0002-3613-8090 AD - Plastic Surgery Service at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Torre-Villalvazo, Iván AU - Torre-Villalvazo I AD - Nutrition Physiology Department at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Butrón-Gandarillas, Patricia AU - Butrón-Gandarillas P AD - Plastic Surgery Service at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Rodríguez-Reyna, Tatiana S AU - Rodríguez-Reyna TS AD - Rheumatology Department at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Torre-Anaya, Erik A AU - Torre-Anaya EA AD - Nutrition Physiology Department at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Guevara-Cruz, Martha AU - Guevara-Cruz M AUID- ORCID: 0000-0002-8996-3481 AD - Nutrition Physiology Department at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Flores-Cháirez, Miguel A AU - Flores-Cháirez MA AD - Fellow-clerk in plastic surgery, Universidad Autonoma de Coahuila, Saltillo, Coahuila, Mexico. FAU - López-Contreras, Diana B AU - López-Contreras DB AD - Fellow-clerk in plastic surgery, Universidad Autonoma de Coahuila, Saltillo, Coahuila, Mexico. FAU - López-Sánchez, Joana Y AU - López-Sánchez JY AD - Fellow-clerk in plastic surgery, Universidad Autonoma de Coahuila, Saltillo, Coahuila, Mexico. FAU - Ruiz-Betanzos, Ángel J AU - Ruiz-Betanzos ÁJ AD - Fellow-clerk in plastic surgery, Instituto Nacional de Ciencias Médicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Méndez López, Ana L AU - Méndez López AL AD - Fellow-clerk in plastic surgery, Universidad Autonoma de Coahuila, Saltillo, Coahuila, Mexico. FAU - Rubio-Gutierrez, Carolina AU - Rubio-Gutierrez C AD - Fellow-clerk in plastic surgery, Universidad Autonoma de Coahuila, Saltillo, Coahuila, Mexico. FAU - Téllez-Pallares, Fernando AU - Téllez-Pallares F AD - Fellow-clerk in plastic surgery, Instituto Nacional de Ciencias Médicas y Nutricion Salvador Zubiran, Mexico City, Mexico. FAU - Nario-Chaidez, Fabian AU - Nario-Chaidez F AD - Mesenchymal Stem cell Therapy Department at CBCells Biotechnology, Zapopan, Mexico. LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20230814 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - digital ulcers SB - IM MH - Stromal Vascular Fraction MH - Quality of Life MH - *Raynaud Disease/therapy MH - *Scleroderma, Systemic/therapy/complications MH - Adipose Tissue MH - Skin Ulcer MH - Humans MH - Treatment Outcome PMC - PMC10424873 COIS- The authors have declared that no competing interests exist. EDAT- 2023/08/14 18:42 MHDA- 2023/08/17 06:43 PMCR- 2023/08/14 CRDT- 2023/08/14 13:33 PHST- 2023/01/26 00:00 [received] PHST- 2023/07/21 00:00 [accepted] PHST- 2023/08/17 06:43 [medline] PHST- 2023/08/14 18:42 [pubmed] PHST- 2023/08/14 13:33 [entrez] PHST- 2023/08/14 00:00 [pmc-release] AID - PONE-D-23-00573 [pii] AID - 10.1371/journal.pone.0289594 [doi] PST - epublish SO - PLoS One. 2023 Aug 14;18(8):e0289594. doi: 10.1371/journal.pone.0289594. eCollection 2023. PMID- 35220464 OWN - NLM STAT- MEDLINE DCOM- 20220614 LR - 20250623 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 41 IP - 7 DP - 2022 Jul TI - Inflammatory myopathies overlapping with systemic sclerosis: a systematic review. PG - 1951-1963 LID - 10.1007/s10067-022-06115-0 [doi] AB - We performed a systematic review of the clinical manifestations and complementary exams of patients with myopathies and systemic sclerosis overlap syndrome (MyoSScOS). Systematic review from January 1976 to November 2021 according PRISMA protocol on three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appears in the title; written in English, Portuguese, or Spanish; and addresses MyoSScOS. Brief communications, reviews, studies that addressed myopathies in children, congress proceedings, monographs, and dissertations were excluded. Thirty-five articles were selected. MyoSScOS seems to be more common in women. It also commonly affects the esophagus and joints with symmetrical and bilateral muscle involvement, Raynaud's phenomenon, and impairment of forced vital capacity. Concerning SSc, the most common subtype was the diffuse form. Cardiovascular and pulmonary complications are an important cause of death. Anti-centromere, anti-PM/Scl, anti-Scl70, anti-RNA polymerase III, anti-Ku, and anti-RNP were more correlated with this entity, and muscle biopsies may present a more aggressive pattern. Electroneuromyography patterns are quite similar to those found in inflammatory myopathies. The absence of studies with robust methodologies and the large number of case reports and series make more robust statistical analyses such as meta-analyses unfeasible. The characterization of MyoSScOS is important for the formulation of therapeutic measures and specific treatments aiming at better quality of life and prognosis. Greater and better theoretical contributions are necessary to better characterize it. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Júnior, Jucier Gonçalves AU - Júnior JG AUID- ORCID: 0000-0001-5077-7959 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil. FAU - Mugii, Naoki AU - Mugii N AUID- ORCID: 0000-0003-3102-5676 AD - Department of Rehabilitation, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan. FAU - Inaoka, Pleiades Tiharu AU - Inaoka PT AUID- ORCID: 0000-0002-7973-0705 AD - Division of Rehabilitation Science, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Sampaio-Barros, Percival Degrava AU - Sampaio-Barros PD AUID- ORCID: 0000-0001-9843-6686 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AUID- ORCID: 0000-0002-3682-4517 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil. samuel.shinjo@usp.br. LA - eng GR - 2014/09079-1/Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)/ GR - 303379/2018-9/Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)/ GR - Faculdade de Medicina da USP - SP/ PT - Journal Article PT - Systematic Review DEP - 20220227 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Child MH - *Connective Tissue Diseases/complications MH - Female MH - Humans MH - *Myositis/complications MH - Quality of Life MH - *Raynaud Disease/complications MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Inflammatory myopathies OT - Myositis OT - Systematic review OT - Systemic sclerosis EDAT- 2022/02/28 06:00 MHDA- 2022/06/15 06:00 CRDT- 2022/02/27 20:38 PHST- 2021/11/10 00:00 [received] PHST- 2022/02/21 00:00 [accepted] PHST- 2022/02/20 00:00 [revised] PHST- 2022/02/28 06:00 [pubmed] PHST- 2022/06/15 06:00 [medline] PHST- 2022/02/27 20:38 [entrez] AID - 10.1007/s10067-022-06115-0 [pii] AID - 10.1007/s10067-022-06115-0 [doi] PST - ppublish SO - Clin Rheumatol. 2022 Jul;41(7):1951-1963. doi: 10.1007/s10067-022-06115-0. Epub 2022 Feb 27. PMID- 26714268 OWN - NLM STAT- MEDLINE DCOM- 20170707 LR - 20181113 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 68 IP - 5 DP - 2016 May TI - Anti-Interferon-Inducible Protein 16 Antibodies Associate With Digital Gangrene in Patients With Scleroderma. PG - 1262-71 LID - 10.1002/art.39558 [doi] AB - OBJECTIVE: To examine the association between anti-interferon-inducible protein 16 (anti-IFI-16) antibodies and clinical features of scleroderma. METHODS: Sera from a discovery sample of 94 patients with scleroderma and 47 healthy controls were assayed for anti-IFI-16 antibodies by enzyme-linked immunosorbent assay, and associations were examined using regression analyses. Since anti-IFI-16 autoantibodies were found to be strongly associated with digital gangrene in the discovery sample, a subsequent case-control study (with subjects matched 1:1 on disease duration) was designed for further exploration. Cases were patients with scleroderma and digital gangrene, while controls were patients with scleroderma and Raynaud's phenomenon alone (n = 39 matched pairs). Nonparametric, unadjusted matched pairs analysis as well as univariate and multivariable conditional logistic regression analyses were performed. RESULTS: In the discovery sample, anti-IFI-16 antibodies were more prevalent in patients with scleroderma than in healthy controls (18% versus 2%; P = 0.01). Patients with anti-IFI-16 antibodies, compared to anti-IFI-16 antibody-negative patients, were more likely to have limited scleroderma (77% versus 46%; P = 0.03), a longer disease duration (median 15.2 years [interquartile range 10.6-18.3] versus 6.0 years [interquartile range 3.4-13.8]; P < 0.01), digital gangrene (24% versus 4%; P = 0.02), and a low diffusing capacity for carbon monoxide (DLco) (P < 0.01). In the case-control study, 35 (45%) of 78 patients were anti-IFI-16 antibody positive. Anti-IFI-16 antibody levels were significantly higher in cases with digital gangrene than in matched controls (P = 0.02). In analyses adjusted for age, cutaneous scleroderma subtype, smoking, and DLco, high anti-IFI-16 antibody levels were associated with the presence of digital gangrene (adjusted odds ratio 2.3, 95% confidence interval 1.0-5.6, P = 0.05). The odds of having digital gangrene increased with higher anti-IFI-16 antibody titers, in a dose-dependent manner. CONCLUSION: Anti-IFI-16 antibodies are associated with digital gangrene in patients with scleroderma. Longitudinal prospective studies exploring anti-IFI-16 antibodies as a disease biomarker, and biologic studies investigating the pathogenicity of these antibodies, are warranted. CI - © 2016, American College of Rheumatology. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Vaidya, Dhananjay AU - Vaidya D AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Wigley, Fredrick M AU - Wigley FM AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Rosen, Antony AU - Rosen A AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. LA - eng GR - R56 AR062615/AR/NIAMS NIH HHS/United States GR - K23 AR061439/AR/NIAMS NIH HHS/United States GR - R01 DE012354/DE/NIDCR NIH HHS/United States GR - R37 DE012354/DE/NIDCR NIH HHS/United States GR - P30 AR053503/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) RN - 0 (Nuclear Proteins) RN - 0 (Phosphoproteins) RN - 148998-64-5 (IFI16 protein, human) SB - IM MH - Adult MH - Aged MH - Autoantibodies MH - Case-Control Studies MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fingers/*pathology MH - Gangrene/immunology MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Nuclear Proteins/*immunology MH - Phosphoproteins/*immunology MH - Raynaud Disease/*immunology MH - Retrospective Studies MH - Scleroderma, Systemic/*immunology PMC - PMC4848112 MID - NIHMS779548 COIS- Conflicts of interest: DV declares a conflict of interest as he is a consultant for Consumable Science, Inc. None of the other authors has received any financial support or other benefits from commercial sources for the work reported in this manuscript, nor do any of the other authors have any financial interests which could create a potential conflict of interest, or the appearance thereof. EDAT- 2015/12/30 06:00 MHDA- 2017/07/08 06:00 PMCR- 2017/05/01 CRDT- 2015/12/30 06:00 PHST- 2015/05/28 00:00 [received] PHST- 2015/12/15 00:00 [accepted] PHST- 2015/12/30 06:00 [entrez] PHST- 2015/12/30 06:00 [pubmed] PHST- 2017/07/08 06:00 [medline] PHST- 2017/05/01 00:00 [pmc-release] AID - 10.1002/art.39558 [doi] PST - ppublish SO - Arthritis Rheumatol. 2016 May;68(5):1262-71. doi: 10.1002/art.39558. PMID- 24092682 OWN - NLM STAT- MEDLINE DCOM- 20131126 LR - 20260128 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 72 IP - 11 DP - 2013 Nov TI - 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. PG - 1747-55 LID - 10.1136/annrheumdis-2013-204424 [doi] AB - OBJECTIVE: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. METHODS: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by (1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and (2) validating against the combined view of a group of experts on a set of cases with or without SSc. RESULTS: It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, seven additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. CONCLUSIONS: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease. FAU - van den Hoogen, Frank AU - van den Hoogen F AD - St. Maartenskliniek and Radboud University Nijmegen Medical Centre, , Nijmegen, The Netherlands. FAU - Khanna, Dinesh AU - Khanna D FAU - Fransen, Jaap AU - Fransen J FAU - Johnson, Sindhu R AU - Johnson SR FAU - Baron, Murray AU - Baron M FAU - Tyndall, Alan AU - Tyndall A FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M FAU - Naden, Raymond P AU - Naden RP FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Carreira, Patricia E AU - Carreira PE FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Clements, Philip J AU - Clements PJ FAU - Denton, Christopher P AU - Denton CP FAU - Distler, Oliver AU - Distler O FAU - Allanore, Yannick AU - Allanore Y FAU - Furst, Daniel E AU - Furst DE FAU - Gabrielli, Armando AU - Gabrielli A FAU - Mayes, Maureen D AU - Mayes MD FAU - van Laar, Jacob M AU - van Laar JM FAU - Seibold, James R AU - Seibold JR FAU - Czirjak, Laszlo AU - Czirjak L FAU - Steen, Virginia D AU - Steen VD FAU - Inanc, Murat AU - Inanc M FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Valentini, Gabriele AU - Valentini G FAU - Veale, Douglas J AU - Veale DJ FAU - Vonk, Madelon C AU - Vonk MC FAU - Walker, Ulrich A AU - Walker UA FAU - Chung, Lorinda AU - Chung L FAU - Collier, David H AU - Collier DH FAU - Ellen Csuka, Mary AU - Ellen Csuka M FAU - Fessler, Barri J AU - Fessler BJ FAU - Guiducci, Serena AU - Guiducci S FAU - Herrick, Ariane AU - Herrick A FAU - Hsu, Vivien M AU - Hsu VM FAU - Jimenez, Sergio AU - Jimenez S FAU - Kahaleh, Bashar AU - Kahaleh B FAU - Merkel, Peter A AU - Merkel PA FAU - Sierakowski, Stanislav AU - Sierakowski S FAU - Silver, Richard M AU - Silver RM FAU - Simms, Robert W AU - Simms RW FAU - Varga, John AU - Varga J FAU - Pope, Janet E AU - Pope JE LA - eng GR - K24-AR-063120/AR/NIAMS NIH HHS/United States GR - Canadian Institutes of Health Research/Canada PT - Consensus Statement PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Europe MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hypertension, Pulmonary/etiology MH - Lung Diseases, Interstitial/etiology MH - Male MH - Middle Aged MH - Raynaud Disease/etiology MH - Reproducibility of Results MH - Scleroderma, Limited/classification/complications/diagnosis MH - Scleroderma, Systemic/*classification/complications/diagnosis MH - Sensitivity and Specificity MH - Telangiectasis/etiology MH - United States OTO - NOTNLM OT - Disease Activity OT - Systemic Sclerosis OT - Treatment EDAT- 2013/10/05 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/10/05 06:00 PHST- 2013/10/05 06:00 [entrez] PHST- 2013/10/05 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - S0003-4967(24)21306-6 [pii] AID - 10.1136/annrheumdis-2013-204424 [doi] PST - ppublish SO - Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424. PMID- 30885245 OWN - NLM STAT- MEDLINE DCOM- 20200406 LR - 20260127 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 21 IP - 1 DP - 2019 Mar 18 TI - Role of lectin pathway complement proteins and genetic variants in organ damage and disease severity of systemic sclerosis: a cross-sectional study. PG - 76 LID - 10.1186/s13075-019-1859-1 [doi] LID - 76 AB - BACKGROUND: The role of the complement system in the pathogenesis of systemic sclerosis (SSc) is controversial. This study investigated the role of the lectin pathway of complement as a mediator of ischemia/reperfusion injury in SSc. METHODS: This is a prospective observational cross-sectional study of 211 SSc patients and 29 patients with Raynaud's phenomenon in undifferentiated connective tissue disease (UCTD) at risk of developing SSc from two outpatient clinics. Serum levels of lectin pathway proteins (FCN-2, FCN-3, MBL, and MASP-2) and eight MBL2 and FCN2 single-nucleotide polymorphisms (SNP) were analyzed by sandwich-type immunoassays and genotyping and examined for their association with disease manifestations. RESULTS: Lectin pathway protein levels and SNPs were similar between SSc and UCTD patients. FCN-2 levels were however higher in SSc patients with present evidence of digital ulcers (mean 1.4 vs. 1.0 μg/mL, p = 0.05), pitting scars (mean 1.3 vs. 1.0 μg/mL, p = 0.01), and puffy fingers (mean 1.2 vs. 1.0 μg/mL, p = 0.04). Similarly, higher FCN-2 levels were observed in SSc patients with Scl-70 autoantibodies (mean 1.5 vs. 1.0 μg/mL, p = 0.001), interstitial lung disease (mean 1.2 vs. 0.9 μg/mL, p = 0.02), and a forced vital capacity (FVC) below 80% (mean 1.4 vs. 1.0 μg/mL, p = 0.02). In line, variant alleles in the FCN-2 SNP at position + 6359 were associated with a significantly reduced FVC and diffusion capacity. Furthermore, patients with SSc renal crisis harbored higher MBL levels (mean 2.7 vs. 1.5 μg/mL, p = 0.04). No other lectin pathway protein levels or polymorphisms were associated with disease manifestations, low complement C3 and/or C4 levels, or inflammatory markers. CONCLUSIONS: This study does not support a relevant role for several lectin pathway complement proteins in the pathogenesis of SSc. Higher FCN-2 levels were however associated with Scl-70 autoantibody positivity, interstitial lung involvement, and digital vasculopathy. Elevated MBL levels were associated with renal crisis. FAU - Osthoff, Michael AU - Osthoff M AUID- ORCID: 0000-0001-5439-957X AD - Department of Internal Medicine, University Hospital Basel, 4031, Basel, Switzerland. Michael.osthoff@usb.ch. AD - Department of Biomedicine, University Basel, 4031, Basel, Switzerland. Michael.osthoff@usb.ch. FAU - Jaeger, Veronika K AU - Jaeger VK AD - Department of Rheumatology, University Hospital Basel, 4031, Basel, Switzerland. FAU - Heijnen, Ingmar A F M AU - Heijnen IAFM AD - Division of Medical Immunology, Laboratory Medicine, University Hospital Basel, 4031, Basel, Switzerland. FAU - Trendelenburg, Marten AU - Trendelenburg M AD - Department of Internal Medicine, University Hospital Basel, 4031, Basel, Switzerland. AD - Department of Biomedicine, University Basel, 4031, Basel, Switzerland. FAU - Jordan, Suzana AU - Jordan S AD - Department of Rheumatology, University Hospital Zurich, 8091, Zurich, Switzerland. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, 8091, Zurich, Switzerland. FAU - Walker, Ulrich A AU - Walker UA AD - Department of Rheumatology, University Hospital Basel, 4031, Basel, Switzerland. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20190318 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Biomarkers) RN - 9007-36-7 (Complement System Proteins) RN - 0 (Lectins) RN - 0 (Ficolins) SB - IM MH - Adult MH - Aged MH - Biomarkers/blood MH - Complement Pathway, Mannose-Binding Lectin/*genetics MH - Complement System Proteins/*genetics/metabolism MH - Connective Tissue Diseases/complications MH - Cross-Sectional Studies MH - Humans MH - Lectins/blood/genetics/metabolism MH - Male MH - Middle Aged MH - *Polymorphism, Single Nucleotide MH - Prospective Studies MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications/*genetics/metabolism MH - Severity of Illness Index MH - Ficolins PMC - PMC6423822 OTO - NOTNLM OT - Complement system OT - Ficolin-2 OT - Innate immunity OT - Mannose-binding lectin OT - Systemic sclerosis COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Approved by the Ethics Committee at participating Basel and Zurich University EUSTAR centers, patients were required to provide informed written consent. CONSENT FOR PUBLICATION: Not applicable. COMPETING INTERESTS: OD has/had consultancy relationship and/or has received research funding from Actelion, AnaMar, Bayer, Boehringer Ingelheim, ChemomAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Novartis, Pfizer, Sanofi, Serodapharm, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of systemic sclerosis licensed. MO, IH, SJ, VJ, and UAW declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/03/20 06:00 MHDA- 2020/04/09 06:00 PMCR- 2019/03/18 CRDT- 2019/03/20 06:00 PHST- 2018/11/28 00:00 [received] PHST- 2019/03/05 00:00 [accepted] PHST- 2019/03/20 06:00 [entrez] PHST- 2019/03/20 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2019/03/18 00:00 [pmc-release] AID - 10.1186/s13075-019-1859-1 [pii] AID - 1859 [pii] AID - 10.1186/s13075-019-1859-1 [doi] PST - epublish SO - Arthritis Res Ther. 2019 Mar 18;21(1):76. doi: 10.1186/s13075-019-1859-1. PMID- 30392161 OWN - NLM STAT- MEDLINE DCOM- 20190607 LR - 20200225 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 3 DP - 2019 Mar TI - Long-term pulmonary outcomes and mortality in idiopathic inflammatory myopathies associated with interstitial lung disease. PG - 803-815 LID - 10.1007/s10067-018-4353-2 [doi] AB - OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). RESULTS: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. CONCLUSIONS: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD. FAU - Cobo-Ibáñez, Tatiana AU - Cobo-Ibáñez T AD - Rheumatology Department, Hospital Universitario Infanta Sofía, Universidad Europea, Paseo de Europa 34, 28702, Madrid, San Sebastián de los Reyes, Spain. mtcoboiba@yahoo.es. FAU - López-Longo, Francisco-Javier AU - López-Longo FJ AD - Rheumatology Department, Hospital General Universitario Gregorio Marañón, Dr. Esquerdo 46, 28007, Madrid, Spain. FAU - Joven, Beatriz AU - Joven B AD - Rheumatology Department, Hospital Universitario 12 de Octubre, Avda. Córdoba s/n, 28041, Madrid, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Rheumatology Department, Hospital Universitario 12 de Octubre, Avda. Córdoba s/n, 28041, Madrid, Spain. FAU - Muñoz-Fernández, Santiago AU - Muñoz-Fernández S AD - Rheumatology Department, Hospital Universitario Infanta Sofía, Universidad Europea, Paseo de Europa 34, 28702, Madrid, San Sebastián de los Reyes, Spain. FAU - Maldonado-Romero, Valentina AU - Maldonado-Romero V AD - Rheumatology Department, Hospital Universitario Ramón y Cajal, Ctra. de Colmenar Viejo Km. 9,100, 28034, Madrid, Spain. FAU - Larena-Grijalba, Carmen AU - Larena-Grijalba C AD - Rheumatology Department, Hospital Universitario Ramón y Cajal, Ctra. de Colmenar Viejo Km. 9,100, 28034, Madrid, Spain. FAU - Cubas, Irene Llorente AU - Cubas IL AD - Rheumatology Department, Hospital Universitario de la Princesa, Diego de León 62, 28006, Madrid, Spain. FAU - Muriel, Eva Tomero AU - Muriel ET AD - Rheumatology Department, Hospital Universitario de la Princesa, Diego de León 62, 28006, Madrid, Spain. FAU - Mateos, Carmen Barbadillo AU - Mateos CB AD - Rheumatology Department, Hospital Universitario Puerta de Hierro Majadahonda, Manuel de Falla 1, 28220, Madrid, Majadahonda, Spain. FAU - de la Peña Lefebvre, Paloma García AU - de la Peña Lefebvre PG AD - Rheumatology Department, Hospital Universitario HM Sanchinarro, Oña 10, 28050, Madrid, Spain. FAU - Gomez-Gomez, Alejandro AU - Gomez-Gomez A AD - Rheumatology Department, Hospital Universitario HM Sanchinarro, Oña 10, 28050, Madrid, Spain. FAU - Nogal, Laura Barrio AU - Nogal LB AD - Rheumatology Department, Hospital Universitario Príncipe de Asturias, Alcalá-Meco s/n, 28805, Madrid, Alcalá de Henares, Spain. FAU - Pérez, Ana AU - Pérez A AD - Rheumatology Department, Hospital Universitario Príncipe de Asturias, Alcalá-Meco s/n, 28805, Madrid, Alcalá de Henares, Spain. FAU - Almodovar, Raquel AU - Almodovar R AD - Rheumatology Department, Hospital Universitario Fundación Alcorcon, Budapest s/n, 28922, Madrid, Spain. FAU - Lojo, Leticia AU - Lojo L AD - Rheumatology Department, Hospital Universitario Infanta Leonor, Gran Vía del Este 80, 28031, Madrid, Spain. FAU - Ruiz-Gutiérrez, Lucía AU - Ruiz-Gutiérrez L AD - Rheumatology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo 65, 28009, Madrid, Spain. FAU - López-Robledillo, Juan Carlos AU - López-Robledillo JC AD - Rheumatology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo 65, 28009, Madrid, Spain. FAU - García de Yébenes, María Jesús AU - García de Yébenes MJ AD - Instituto de Salud Musculoesquelética (InMusc), Conde de la Cimera 6, Oficina C, 28040, Madrid, Spain. FAU - Nuño-Nuño, Laura AU - Nuño-Nuño L AD - Rheumatology Department, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain. LA - eng PT - Journal Article DEP - 20181103 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) RN - EC 4.1.2.13 (Fructose-Bisphosphate Aldolase) RN - Antisynthetase syndrome SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/immunology MH - Delayed Diagnosis/statistics & numerical data MH - Dermatomyositis/epidemiology/immunology/physiopathology MH - Disease Progression MH - Erythema/epidemiology MH - Female MH - Fructose-Bisphosphate Aldolase/metabolism MH - Humans MH - Infections/epidemiology MH - Kaplan-Meier Estimate MH - Longitudinal Studies MH - Lung Diseases, Interstitial/epidemiology/*physiopathology/therapy MH - Male MH - Middle Aged MH - *Mortality MH - Multivariate Analysis MH - Myositis/epidemiology/immunology/*physiopathology MH - Oxygen Inhalation Therapy/*statistics & numerical data MH - Polymyositis/epidemiology/immunology/physiopathology MH - Prognosis MH - Proportional Hazards Models MH - Protective Factors MH - Pulmonary Diffusing Capacity MH - Raynaud Disease/epidemiology MH - Respiratory Function Tests MH - Retrospective Studies MH - Risk Factors MH - Sex Factors MH - Spain/epidemiology MH - Vital Capacity MH - Young Adult OTO - NOTNLM OT - Interstitial lung disease OT - Myositis OT - Oxygen therapy OT - Prognosis OT - Respiratory function tests OT - Survival EDAT- 2018/11/06 06:00 MHDA- 2019/06/08 06:00 CRDT- 2018/11/05 06:00 PHST- 2018/06/26 00:00 [received] PHST- 2018/10/26 00:00 [accepted] PHST- 2018/09/30 00:00 [revised] PHST- 2018/11/06 06:00 [pubmed] PHST- 2019/06/08 06:00 [medline] PHST- 2018/11/05 06:00 [entrez] AID - 10.1007/s10067-018-4353-2 [pii] AID - 10.1007/s10067-018-4353-2 [doi] PST - ppublish SO - Clin Rheumatol. 2019 Mar;38(3):803-815. doi: 10.1007/s10067-018-4353-2. Epub 2018 Nov 3. PMID- 27987518 OWN - NLM STAT- MEDLINE DCOM- 20170802 LR - 20181202 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 48 IP - 6 DP - 2016 Dec 18 TI - [Clinical characteristics of 4 cases of scleritis associated with systemic lupus erythematosus]. PG - 1081-1085 AB - Episcleritis and scleritis are relatively rare ocular diseases, which are commonly associated with rheumatic diseases including systemic lupus erythematosus (SLE). To investigate clinical and laboratory features of SLE-associated episcleritis and scleritis, we now report 4 cases of inpatients who were diagnosed with episcleritis or scleritis secondary to SLE from September 2005 to July 2016 in the Department of Rheumatology and Immunology in Peking University People's Hospital. Demographic, clinical and laboratory characteristics were summarized together with the treatment regimen and the prognosis; the literature was reviewed. There were 3 female and 1 male patients. The average age was (49.0±23.8) years and the mean duration of SLE at the onset of episcleritis or scleritis was (2.1±1.4) years. In addition to the eye involvement, the patients had mucocutaneous manifestations, serositis, lupus nephritis and interstitial pneumonia simultaneously; in the past, 1 patient experienced arthritis, 2 presented Raynaud's phenomenon, and 2 had hematologic involvement. All the patients had antinuclear antibody (ANA) of high titer. The anti double-stranded DNA (ds-DNA) antibody titers were increased in 2 patients. Three patients had positive anti-nucleosome antibody (ANuA) while the other 1 patient did not test it. The complement levels were decreased in 3 patients. The systemic lupus erythematosus disease activity index (SLEDAI) scores were more than 4 points in all the patients (ranging from 7-16), suggesting active disease. Ocular symptoms included pain, redness of the eye and tears. Ophthalmic examinations revealed 3 cases of episcleritis and 1 case of scleritis. Among the 4 patients, 2 patients experienced ocular complications including decrease in vision and uveitis. All the patients were treated with systemic corticosteroids combined with hydroxycloroquine; 3 patients were treated with immunosuppressants (cyclophosphamide in 2 patients and leflunomide in 1 patient). All of the 4 patients received topical steroid and 1 patient received periocular injection of triamcinolone acetonide; 1 patient received topical nonsteroidal anti-inflammatory drug (NSAID).No recurrence of episcleritis or scleritis was observed during the follow-ups. As a conclusion, scleritis and episcleritis, although uncommon, may occur in patients with autoimmune rheumatic diseases including SLE. The occurrence of episcleritis and scleritis may suggest active disease of SLE. Ocular complications need to be aware of in the patients. Prompt diagnosis and treatment was associated with good visual outcomes in the follow-ups. FAU - Wang, L AU - Wang L AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Yang, Y AU - Yang Y AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Jia, Y AU - Jia Y AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Miao, H AU - Miao H AD - Department of Ophtalmology,Peking University People's Hospital, Beijing 100044, China. FAU - Zhou, Y S AU - Zhou YS AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhang, X Y AU - Zhang XY AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Antibodies, Antinuclear) RN - 0 (Isoxazoles) RN - 8N3DW7272P (Cyclophosphamide) RN - F446C597KA (Triamcinolone Acetonide) RN - G162GK9U4W (Leflunomide) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Aged MH - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use MH - Antibodies, Antinuclear/blood MH - Arthritis/complications MH - Cyclophosphamide/therapeutic use MH - Disease Progression MH - Female MH - Hematologic Diseases/complications MH - Humans MH - Isoxazoles/therapeutic use MH - Leflunomide MH - Lung Diseases, Interstitial/complications MH - Lupus Erythematosus, Systemic/*complications/*drug therapy MH - Lupus Nephritis/complications MH - Male MH - Middle Aged MH - Pain MH - Prognosis MH - Raynaud Disease/complications MH - Recurrence MH - Scleritis/*complications/*drug therapy MH - Serositis/complications MH - Treatment Outcome MH - Triamcinolone Acetonide/therapeutic use MH - Uveitis/etiology MH - Vision Disorders/etiology EDAT- 2016/12/18 06:00 MHDA- 2017/08/03 06:00 CRDT- 2016/12/18 06:00 PHST- 2016/12/18 06:00 [entrez] PHST- 2016/12/18 06:00 [pubmed] PHST- 2017/08/03 06:00 [medline] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2016 Dec 18;48(6):1081-1085. PMID- 40082520 OWN - NLM STAT- MEDLINE DCOM- 20250513 LR - 20250513 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 15 IP - 1 DP - 2025 Mar 13 TI - Alterations in nailfold videocapillaroscopy among patients with connective tissue diseases combined with pulmonary arterial hypertension: A cross-sectional study. PG - 8647 LID - 10.1038/s41598-025-92093-7 [doi] LID - 8647 AB - This study examines the correlation between nailfold videocapillaroscopy (NVC) abnormalities and pulmonary hypertension (PH) in connective tissue disease (CTD) patients, evaluating its diagnostic and predictive value for microcirculation alterations. A cross-sectional study included 351 CTD patients and 30 non-CTD healthy people, with NVC assessments conducted qualitatively, semi-quantitatively, and quantitatively by two independent physicians. Clinical and laboratory data were analyzed, comparing CTD patients with pulmonary arterial hypertension (CTD-PAH) and those without (CTD-non-PAH). Among the patients, 16.5% (n = 58) had pulmonary hypertension. CTD-PAH patients showed higher nailfold videocapillaroscopy scores (5.73 ± 3.54 vs. 4.30 ± 2.98, P = 0.001) and larger capillary diameters (17.06 ± 8.22 vs. 14.41 ± 9.25, P = 0.044) compared to CTD-non-PAH patients. Factors significantly influencing the nailfold videocapillaroscopy score included Raynaud's phenomenon, pulmonary hypertension, and the presence of anti-Scl-70 antibody. The ROC analysis yielded an AUC of 0.621 nailfold videocapillaroscopy score for predicting PAH. Additionally, pulmonary artery systolic pressure in CTD-PAH patients was positively correlated with both nailfold videocapillaroscopy score (R = 0.618, B = 3.26, P < 0.001) and capillary diameter (R = 0.541, B = 1.23, P < 0.001). Nailfold videocapillaroscopy abnormalities, such as higher scores and increased capillary diameters, are associated with pulmonary hypertension in patients with connective tissue diseases (CTD). This method demonstrates potential diagnostic and predictive value for detecting microcirculation alterations in these patients. CI - © 2025. The Author(s). FAU - Tang, Zhicheng AU - Tang Z AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Yang, Fan AU - Yang F AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Wu, Haolin AU - Wu H AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing University of Chinese Medicine, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Zhao, Ying AU - Zhao Y AD - Nantong Maternal and Child Health Care Hospital, 399 Century Avenue, Nantong, 226001, Jiangsu, China. FAU - Shen, Jingyi AU - Shen J AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Hong, Huiming AU - Hong H AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing University of Chinese Medicine, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Yin, Fanzhang AU - Yin F AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing University of Chinese Medicine, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Ma, Xiaolei AU - Ma X AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Geng, Linyu AU - Geng L AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Xu, Xue AU - Xu X AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. iamxuxue@163.com. FAU - Wei, Yu AU - Wei Y AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. amy09060301@163.com. FAU - Zhang, Huayong AU - Zhang H AD - Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. huayong.zhang@nju.edu.cn. LA - eng GR - 81802126/National Natural Science Foundation of China/ PT - Journal Article DEP - 20250313 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Humans MH - Male MH - Female MH - Cross-Sectional Studies MH - *Connective Tissue Diseases/complications/diagnostic imaging MH - Middle Aged MH - *Microscopic Angioscopy/methods MH - Adult MH - *Pulmonary Arterial Hypertension/complications/diagnosis MH - *Nails/blood supply MH - Capillaries/pathology MH - Aged MH - *Hypertension, Pulmonary/complications MH - Microcirculation MH - Raynaud Disease PMC - PMC11906740 OTO - NOTNLM OT - Connective tissue disease OT - Microcirculation OT - Nailfold videocapillaroscopy OT - Pulmonary hypertension COIS- Declarations. Competing interests: The authors declare no competing interests. Ethical statement: The Ethics Committee of the Nanjing Drum Tower Hospital approved the study(No. 2024–160-01), and informed consent was obtained from all participants. EDAT- 2025/03/14 11:23 MHDA- 2025/03/14 11:24 PMCR- 2025/03/13 CRDT- 2025/03/14 00:34 PHST- 2024/07/23 00:00 [received] PHST- 2025/02/25 00:00 [accepted] PHST- 2025/03/14 11:24 [medline] PHST- 2025/03/14 11:23 [pubmed] PHST- 2025/03/14 00:34 [entrez] PHST- 2025/03/13 00:00 [pmc-release] AID - 10.1038/s41598-025-92093-7 [pii] AID - 92093 [pii] AID - 10.1038/s41598-025-92093-7 [doi] PST - epublish SO - Sci Rep. 2025 Mar 13;15(1):8647. doi: 10.1038/s41598-025-92093-7. PMID- 35810370 OWN - NLM STAT- MEDLINE DCOM- 20220712 LR - 20220712 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 1 IP - 2 DP - 2022 Apr-Jun TI - The safety and persistence of intravenous iloprost in systemic sclerosis. PG - 122-128 AB - INTRODUCTION: Vasculopathy is a crucial feature of systemic sclerosis (SSc). It occurs in almost every patient with SSc, with Raynaud's phenomenon (RP) and digital ulcers (DU) having a great impact on the quality of patients' lives. Intravenous (IV) iloprost, a synthetic analogue of prostacyclin, is broadly used to treat RP and DU secondary to SSc. Currently, there is no standard protocol defined for the iloprost treatment of SSc-associated RP and DU, and, consequently, the management of this treatment is largely based on each centre's experience. OBJECTIVE: The objective of this study is to evaluate the safety profile of a particular scheme of IV iloprost used in our centre as the standard treatment of SSc-related vascular complications. METHODS: We retrospectively evaluated the clinical records of SSc patients, classified according to the 2013 European Alliance of Associations for Rheumatology (EULAR) criteria (31) with SSc-related DU and/or severe RP not responsive to CCB, receiving or who have received IV iloprost infusions from January 1st 2011 to March 31st 2021 Results: Within this time frame, 60 patients (n=44 for DU; n=16 for severe RP) were treated with a monthly 10-hour IV iloprost perfusion with a dosing regimen adapted to individual tolerance. Forty-nine of these 60 patients (81.7%) were on iloprost for more than one year. Within 12 months of therapy, 40 patients have healed the DUs (90.9%), with only 4 patients maintaining active DUs. A significant clinical improvement in RP at 12 months was observed in 87.5% (n=14/16) of SSc patients with severe RP. Eleven AE implying treatment dose/frequency adjustments or suspension were recorded (18.3% of patients): severe headache (n=5), hypotension (n=3), tachycardia (n=1), flushing (n=1) and generalised erythroderma (n=1). In all patients, the perfusion rate was reduced in the following treatment sessions with good tolerance, with the exception of the patient with the generalised erythroderma reaction, who suspended the perfusion and was later switched to bosentan. After a mean follow-up time of 6.9 (+/-) 4.0 years of treatment (range 0.06-22), 24 patients (40%) stopped the therapy, 14 (58.3%) of whom due to clinical improvement. The overall 5-, and 10-year survival rates of IV iloprost were 68.2% and 55.6%, respectively. CONCLUSION: SSc patients who received this flexible IV iloprost regimen achieved clinical improvement, reflected in the high persistence rate of the drug, with a good tolerability profile. In addition, most side effects were mild and easily managed. FAU - Martins, Patrícia AU - Martins P AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Centro Académico de Medicina de Lisboa. FAU - Dourado, Eduardo AU - Dourado E AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Centro Académico de Medicina de Lisboa. FAU - Fonseca, João Eurico AU - Fonseca JE AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Centro Académico de Medicina de Lisboa. FAU - Romão, Vasco AU - Romão V AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Centro Académico de Medicina de Lisboa. FAU - Resende, Catarina AU - Resende C AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte. LA - eng PT - Journal Article TT - The safety and persistence of intravenous iloprost in systemic sclerosis. PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 RN - JED5K35YGL (Iloprost) SB - IM MH - *Dermatitis, Exfoliative/chemically induced MH - Humans MH - Iloprost/adverse effects MH - *Raynaud Disease/drug therapy MH - Retrospective Studies MH - *Scleroderma, Systemic/complications MH - *Skin Ulcer/complications EDAT- 2022/07/11 06:00 MHDA- 2022/07/14 06:00 CRDT- 2022/07/10 07:32 PHST- 2022/07/10 07:32 [entrez] PHST- 2022/07/11 06:00 [pubmed] PHST- 2022/07/14 06:00 [medline] AID - PC220019 [pii] PST - ppublish SO - ARP Rheumatol. 2022 Apr-Jun;1(2):122-128. PMID- 35753143 OWN - NLM STAT- MEDLINE DCOM- 20220908 LR - 20250728 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 56 DP - 2022 Oct TI - False positive anti-Topoisomerase I (Scl-70) antibody results in clinical practice: A case series from a scleroderma referral center. PG - 152052 LID - S0049-0172(22)00103-2 [pii] LID - 10.1016/j.semarthrit.2022.152052 [doi] AB - PURPOSE: To determine if some patients who tested positive for anti-Scl-70 antibody in clinical practice, but did not have classifiable systemic sclerosis, were negative for anti-Scl-70 antibody by the more specific immunodiffusion method of testing. METHODS: Patients evaluated by a rheumatologist at a Scleroderma referral center who had tested positive for anti-Scl-70 antibody prior to referral, but did not have classifiable SSc based on clinical criteria, were invited to undergo testing for anti-Scl-70 antibody by immunodiffusion. Patient demographics and clinical features were recorded at the time of their evaluation, and diagnostic testing results were reviewed using the medical records. RESULTS: 52 patients were enrolled over an 8-year period, with 48 (92.3%) testing negative and 4 (7.7%) testing positive for anti-Scl-70 antibody by immunodiffusion. Of the 48 patients who tested negative, 18 (37.5%) tested negative for ANA by indirect immunofluorescence, 33 (68.8%) did not have Raynaud's phenomenon, and 43 (89.6%) had ≤1 clinical criteria items based on the 2013 ACR/EULAR SSc classification criteria. Nevertheless, 21 (43.8%) patients who were negative for anti-Scl-70 antibody by immunodiffusion had undergone a chest CT and 14 (29.2%) had undergone an echocardiogram. A total of 23 patients had at least one follow up clinic visit. 3 out of 4 patients who were positive for anti-Scl-70 antibody by immunodiffusion, but none of the 19 patients who tested negative by immunodiffusion, developed sufficient criteria during follow up to be classified as SSc. CONCLUSION: Assays for anti-Scl-70 antibody in commercial laboratories that are commonly utilized in clinical practice can produce false positive results. These results can lead to angst for patients, as well as unnecessary referrals and diagnostic evaluations. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Lam, Brian H AU - Lam BH AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Assassi, Shervin AU - Assassi S AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Charles, Julio AU - Charles J AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Taherian, Rana AU - Taherian R AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Lyons, Marka A AU - Lyons MA AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Jandali, Bochra AU - Jandali B AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Mayes, Maureen D AU - Mayes MD AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. FAU - Skaug, Brian AU - Skaug B AD - Division of Rheumatology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St., MSB 5.266, Houston, TX 77030, United States. Electronic address: brian.a.skaug@uth.tmc.edu. LA - eng GR - R01 AR073284/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20220617 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (anti-scl-70 autoantibodies) SB - IM CIN - Semin Arthritis Rheum. 2022 Dec;57:152085. doi: 10.1016/j.semarthrit.2022.152085. PMID: 36057189 MH - Antibodies, Antinuclear MH - Autoantibodies MH - Humans MH - *Raynaud Disease/diagnosis MH - Referral and Consultation MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/diagnosis PMC - PMC10061056 MID - NIHMS1882841 OTO - NOTNLM OT - ANA OT - Immunodiffusion OT - Scl-70 antibody OT - Scleroderma OT - Systemic sclerosis OT - Topoisomerase I antibody COIS- Declaration of Competing Interest SA has received grant support from the NIH, Department of Defense, Scleroderma Research Foundation, Momenta, Boehringer Ingelheim, and Janssen, consulting fees from Boehringer Ingelheim, Corbus, Novartis, CSL Behring, Abbvie, and AstraZeneca, payments or honoraria from Integrity CE and the North Carolina Rheumatology Association, and serves as an unpaid volunteer in leadership roles in the Scleroderma Clinical Trials Consortium and the Scleroderma Foundation Medical Advisory Board. MDM has received personal fees from Actelion Pharma, support to UTHSCH from Mitsubishi-Tanabe, Boehringer Ingelheim, EICOS, Corbus, and Horizon as a clinical trials investigator, royalties from Oxford University Press, Springer, and BMJ Publishing, consulting fees from Mitsubishi-Tanabe, Boehringer Ingelheim, and Eicos, and payment for lectures from Medtelligence. BHL, JC, RT, MAL, BJ, and BS have no competing interests to declare. EDAT- 2022/06/27 06:00 MHDA- 2022/09/09 06:00 PMCR- 2023/10/01 CRDT- 2022/06/26 18:08 PHST- 2022/03/25 00:00 [received] PHST- 2022/05/31 00:00 [revised] PHST- 2022/06/14 00:00 [accepted] PHST- 2022/06/27 06:00 [pubmed] PHST- 2022/09/09 06:00 [medline] PHST- 2022/06/26 18:08 [entrez] PHST- 2023/10/01 00:00 [pmc-release] AID - S0049-0172(22)00103-2 [pii] AID - 10.1016/j.semarthrit.2022.152052 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Oct;56:152052. doi: 10.1016/j.semarthrit.2022.152052. Epub 2022 Jun 17. PMID- 30277865 OWN - NLM STAT- MEDLINE DCOM- 20190129 LR - 20260518 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 Suppl 113 IP - 4 DP - 2018 Jul-Aug TI - The association of sociodemographic and disease variables with hand function: a Scleroderma Patient-centered Intervention Network cohort study. PG - 88-94 AB - OBJECTIVES: Impaired hand function in systemic sclerosis (SSc) is a primary cause of disability and contributes diminished health-related quality of life. The objective of the present study was to evaluate sociodemographic, lifestyle, and disease-related factors independently associated with hand function in SSc. METHODS: Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort who completed baseline study questionnaires between March 2014 and September 2017 were included. Hand function was measured using the Cochin Hand Function Scale (CHFS). Multiple linear regression analysis was used to identify independent correlates of impaired hand function. RESULTS: Among 1193 participants (88% female), the mean CHFS score was 13.3 (SD=16.1). Female sex (standardised regression coefficient, beta (β)=.05), current smoking (β=.07), higher BMI (β=.06), diffuse SSc (β=0.14), more severe Raynaud's scores (β=.23), more severe finger ulcer scores (β=.23), moderate (β=0.19) or severe small joint contractures (β=.20), rheumatoid arthritis (β=0.07), and idiopathic inflammatory myositis (β=0.06) were significantly associated with higher CHFS scores (more impaired hand function). Consumption of 1-7 alcoholic drinks per week (β=-0.07) was associated with lower CHFS scores (less impaired hand function) compared to no drinking. CONCLUSIONS: Multiple factors are associated with hand function in SSc. The presence of moderate or severe small joint contractures, the presence of digital ulcers, and severity of Raynaud's phenomenon had the largest associations. Effective interventions are needed to improve the management of hand function in patients with SSc. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Behavioural Science Institute, Clinical Psychology, Radboud University, Nijmegen, the Netherlands; Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal; Department of Psychiatry, McGill University, Montreal, Canada. FAU - Sanchez, Tatiana A AU - Sanchez TA AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada. FAU - Turner, Kimberly A AU - Turner KA AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada. FAU - Mouthon, Luc AU - Mouthon L AD - Université Paris Descartes, Assistance Publique-Hopitaux de Paris; Service de Medicine Interne, Centre de Reference Maladies Systémiques Autoimmunes Rares, vascularites nécrosantes et sclérodermie systémique, Hopital Cochin, Paris, France. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada. FAU - Hudson, Marie AU - Hudson M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal; Department of Medicine, McGill University, Montreal, Canada. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AD - Department of Rheumatology, Sint Maartenskliniek, the Netherlands. FAU - Schouffoer, Anne A AU - Schouffoer AA AD - Leiden University Medical Center, Leiden; Haga Teaching Hospital, The Hague, the Netherlands. FAU - Welling, Joep J K C AU - Welling JJKC AD - NVLE Dutch patient organisation for systemic autoimmune diseases, Utrecht, the Netherlands; and Federation of European Scleroderma Associations, Brussels, Belgium. FAU - Sauvé, Maureen AU - Sauvé M AD - Scleroderma Society of Ontario, Hamilton; and Scleroderma Society of Canada, Ottawa, Canada. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Inst. Med. Res., Jewish General Hosp., Montreal; Dept.of Psychiatry, Dept.of Medicine, Dept.of Epidemiology, Biostatistics and Occupational Health, Dept.of Educational & Counselling Psychology, Dept.of Psychology , McGill Univ., Montreal, Canada. CN - the SPIN Investigators LA - eng PT - Journal Article PT - Multicenter Study DEP - 20180929 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Canada/epidemiology MH - Contracture/epidemiology/physiopathology MH - Disability Evaluation MH - Europe/epidemiology MH - Female MH - Hand/*physiopathology MH - Humans MH - *Life Style MH - Male MH - Middle Aged MH - Prognosis MH - Raynaud Disease/epidemiology/physiopathology MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/*epidemiology/*physiopathology MH - Severity of Illness Index MH - Skin Ulcer/epidemiology/physiopathology MH - United States/epidemiology FIR - Murray, Baron IR - Murray B IRAD- McGill University, Montreal, Quebec, Canada. FIR - Bartlett, Susan J IR - Bartlett SJ IRAD- McGill University, Montreal, Quebec, Canada. FIR - Furst, Daniel E IR - Furst DE IRAD- Division of Rheumatology, Geffen School of Medicine at the University of California, Los Angeles, CA, USA. FIR - Gottesman, Karen IR - Gottesman K IRAD- Scleroderma Foundation, Los Angeles, CA, USA, and McGill University, Montreal, Quebec, Canada. FIR - van den Hoogen, Frank IR - van den Hoogen F IRAD- Radboud University Medical Center and Sint Maartenskliniek, Nijmegen, the Netherlands FIR - Malcarne, Vanessa IR - Malcarne V IRAD- San Diego State University, San Diego, CA, USA. FIR - Mayes, Maureen D IR - Mayes MD IRAD- University of Texas McGovern School of Medicine, Houston, Texas, USA. FIR - Nielson, Warren R IR - Nielson WR IRAD- St. Joseph’s Health Care, London, Ontario, Canada. FIR - Riggs, Robert IR - Riggs R IRAD- Scleroderma Foundation, Danvers, Massachusetts, USA. FIR - Wigley, Frederick IR - Wigley F IRAD- Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FIR - Assassi, Shervin IR - Assassi S IRAD- University of Texas McGovern School of Medicine, Houston, Texas, USA. FIR - Boutron, Isabelle IR - Boutron I IRAD- Université Paris Descartes, and Assistance Publique Hôpitaux de Paris, France. FIR - Costa Maia, Angela IR - Costa Maia A IRAD- University of Minho, Braga, Portugal. FIR - El-Baalbaki, Ghassan IR - El-Baalbaki G IRAD- Université du Québec à Montréal, Quebec, Canada. FIR - Ells, Carolyn IR - Ells C IRAD- McGill University, Montreal, Quebec, Canada. FIR - Fligelstone, Kim IR - Fligelstone K IRAD- Scleroderma Society, London, UK. FIR - Fortune, Catherine IR - Fortune C IRAD- Scleroderma Society of Ontario, Hamilton, Ontario, Canada. FIR - Frech, Tracy IR - Frech T IRAD- University of Utah, Salt Lake City, Utah, USA. FIR - Godard, Dominique IR - Godard D IRAD- Association des Sclérodermiques de France, Sorel-Moussel, France. FIR - Harel, Daphna IR - Harel D IRAD- New York University, New York, USA. FIR - Impens, Ann IR - Impens A IRAD- Midwestern University, Downers Grove, Illinois, USA. FIR - Jang, Beona IR - Jang B IRAD- McGill University, Montreal, Quebec, Canada FIR - Johnson, Sindhu R IR - Johnson SR IRAD- Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, and University of Toronto, Ontario, Canada. FIR - Tyrell Kennedy, Ann IR - Tyrell Kennedy A IRAD- Federation of European Scleroderma Associations, Dublin, Ireland. FIR - Körner, Annett IR - Körner A IRAD- McGill University, Montreal, Quebec, Canada FIR - Larche, Maggie IR - Larche M IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Leite, Catarina IR - Leite C IRAD- University of Minho, Braga, Portugal. FIR - Marra, Carlo IR - Marra C IRAD- Memorial University, St. John’s, Newfoundland, Canada. FIR - Nielsen, Karen IR - Nielsen K IRAD- Scleroderma Society of Ontario, Hamilton, Ontario, Canada. FIR - Pope, Janet IR - Pope J IRAD- University of Western Ontario, London, Ontario, Canada. FIR - Portales, Alexandra IR - Portales A IRAD- Asociación Española de Esclerodermia, Madrid, Spain. FIR - Rodriguez Reyna, Tatiana Sofia IR - Rodriguez Reyna TS IRAD- Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. FIR - Steele, Russell J IR - Steele RJ IRAD- Jewish General Hospital and McGill University, Montreal, Quebec, Canada. FIR - Suarez-Almazor, Maria E IR - Suarez-Almazor ME IRAD- University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FIR - Wong-Rieger, Durhane IR - Wong-Rieger D IRAD- Canadian Organization for Rare Disorders, Toronto, Ontario, Canada FIR - Agard, Christian IR - Agard C IRAD- Centre Hospitalier Universitaire, Hôtel-Dieu de Nantes, France. FIR - Albert, Alexandra IR - Albert A IRAD- Université Laval, Quebec, Canada. FIR - André, Marc IR - André M IRAD- Centre Hospitalier Universitaire Gabriel-Montpied, Clermont-Ferrand, France. FIR - Arsenault, Guylaine IR - Arsenault G IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Benmostefa, Nouria IR - Benmostefa N IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Benzidia, Ilham IR - Benzidia I IRAD- Assistance Publique Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Berthier, Sabine IR - Berthier S IRAD- Centre Hospitalier Universitaire Dijon Bourgogne, Dijon, France. FIR - Bissonnette, Lyne IR - Bissonnette L IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Boire, Gilles IR - Boire G IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Bruns, Alessandra IR - Bruns A IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Carreira, Patricia IR - Carreira P IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Casadevall, Marion IR - Casadevall M IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Chaigne, Benjamin IR - Chaigne B IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Chung, Lorinda IR - Chung L IRAD- Stanford University, California, USA. FIR - Cohen, Pascal IR - Cohen P IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Dagenais, Pierre IR - Dagenais P IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Denton, Christopher IR - Denton C IRAD- Royal Free London Hospital, London, UK. FIR - Domsic, Robyn IR - Domsic R IRAD- University of Pittsburgh, Pennsylvania, USA. FIR - Dunne, James V IR - Dunne JV IRAD- St. Paul's Hospital and University of British Columbia, Vancouver, BC, Canada. FIR - Fare, Regina IR - Fare R IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Farge-Bancel, Dominique IR - Farge-Bancel D IRAD- Assistance Publique Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Fortin, Paul R IR - Fortin PR IRAD- CHU de Québec, Université Laval, Quebec, Canada. FIR - Gill, Anna IR - Gill A IRAD- Royal Free London Hospital, London, UK. FIR - Gordon, Jessica IR - Gordon J IRAD- Hospital for Special Surgery, New York, USA. FIR - Granel-Rey, Brigitte IR - Granel-Rey B IRAD- Aix Marseille Université, and Assistance Publique Hôpitaux de Marseille, Hôpital Nord, Marseille, France. FIR - Grange, Claire IR - Grange C IRAD- Centre Hospitalier Lyon Sud, France. FIR - Gyger, Genevieve IR - Gyger G IRAD- Jewish General Hospital and McGill University, Montreal, Quebec, Canada. FIR - Hachulla, Eric IR - Hachulla E IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Hatron, Pierre-Yves IR - Hatron PY IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Herrick, Ariane L IR - Herrick AL IRAD- University of Manchester, Salford Royal NHS Foundation Trust, Manchester, UK. FIR - Hij, Adrian IR - Hij A IRAD- Assistance Publique Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Hinchcliff, Monique IR - Hinchcliff M IRAD- Northwestern University, Chicago, Illinois, USA. FIR - Ikic, Alena IR - Ikic A IRAD- Université Laval, Quebec, Canada. FIR - Jones, Niall IR - Jones N IRAD- University of Alberta, Edmonton, Alberta, Canada. FIR - Fernandes, Artur Jose de B IR - Fernandes AJB IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Kafaja, Suzanne IR - Kafaja S IRAD- University of California, Los Angeles, California, USA. FIR - Khalidi, Nader IR - Khalidi N IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Korman, Benjamin IR - Korman B IRAD- Northwestern University, Chicago, Illinois, USA. FIR - Lambert, Marc IR - Lambert M IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Launay, David IR - Launay D IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Liang, Patrick IR - Liang P IRAD- Université de Sherbrooke, Quebec, Canada. FIR - London, Jonathan IR - London J IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Luna, David IR - Luna D IRAD- Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. FIR - Manning, Joanne IR - Manning J IRAD- Salford Royal NHS Foundation Trust, Salford, UK. FIR - Martin, Maria IR - Martin M IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Martin, Thierry IR - Martin T IRAD- Les Hôpitaux Universitaires de Strasbourg, Nouvel Hôpital Civil, Strasbourg, France. FIR - Masetto, Ariel IR - Masetto A IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Maurier, François IR - Maurier F IRAD- Hôpitaux Privés de Metz, Hôpital Belle-Isle, Metz, France. FIR - Mekinian, Arsene IR - Mekinian A IRAD- Assistance Publique Hôpitaux de Paris, Hôpital St-Antoine, Paris, France. FIR - Melchor, Sheila IR - Melchor S IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Paule, Romain IR - Paule R IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Régent, Alexis IR - Régent A IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Rivière, Sébastien IR - Rivière S IRAD- Assistance Publique Hôpitaux de Paris, Hôpital St-Antoine, Paris, France. FIR - Robinson, David IR - Robinson D IRAD- University of Manitoba, Winnipeg, Manitoba, Canada. FIR - Rodriguez, Esther IR - Rodriguez E IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Roux, Sophie IR - Roux S IRAD- Université de Sherbrooke, Quebec, Canada. FIR - Smets, Perrine IR - Smets P IRAD- Centre Hospitalier Universitaire Gabriel-Montpied, Clermont-Ferrand, France. FIR - Smith, Doug IR - Smith D IRAD- University of Ottawa, Ontario, Canada. FIR - Sobanski, Vincent IR - Sobanski V IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Spiera, Robert IR - Spiera R IRAD- Hospital for Special Surgery, New York, USA. FIR - Steen, Virginia IR - Steen V IRAD- Georgetown University, Washington, DC, USA. FIR - Sutton, Evelyn IR - Sutton E IRAD- Dalhousie University, Halifax, Nova Scotia, Canada. FIR - Terrier, Benjamin IR - Terrier B IRAD- Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Thorne, Carter IR - Thorne C IRAD- Southlake Regional Health Centre, Newmarket, Ontario, Canada. FIR - Varga, John IR - Varga J IRAD- Northwestern University, Chicago, Illinois, USA. FIR - Wilcox, Pearce IR - Wilcox P IRAD- St. Paul's Hospital and University of British Columbia, Vancouver, BC, Canada. FIR - Cumin, Julie IR - Cumin J IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Fox, Rina S IR - Fox RS IRAD- San Diego State University and University of California, San Diego, California, USA. FIR - Gholizadeh, Shadi IR - Gholizadeh S IRAD- San Diego State University and University of California, San Diego, California, USA. FIR - Jewett, Lisa R IR - Jewett LR IRAD- Jewish General Hospital and McGill University, Montreal, Quebec, Canada. FIR - Levis, Brooke IR - Levis B IRAD- Jewish General Hospital and McGill University, Montreal, Quebec, Canada. FIR - Mills, Sarah D IR - Mills SD IRAD- San Diego State University and University of California, San Diego, California, USA. FIR - Pepin, Mia R IR - Pepin MR IRAD- Jewish General Hospital, Montreal, Quebec, Canada. EDAT- 2018/10/03 06:00 MHDA- 2019/01/30 06:00 CRDT- 2018/10/03 06:00 PHST- 2018/04/05 00:00 [received] PHST- 2018/07/02 00:00 [accepted] PHST- 2018/10/03 06:00 [entrez] PHST- 2018/10/03 06:00 [pubmed] PHST- 2019/01/30 06:00 [medline] AID - 12865 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jul-Aug;36 Suppl 113(4):88-94. Epub 2018 Sep 29. PMID- 34858387 OWN - NLM STAT- MEDLINE DCOM- 20220107 LR - 20240819 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 12 DP - 2021 TI - Systemic Sclerosis in Zimbabwe: Autoantibody Biomarkers, Clinical, and Laboratory Correlates. PG - 679531 LID - 10.3389/fimmu.2021.679531 [doi] LID - 679531 AB - INTRODUCTION: Systemic sclerosis (SScl) is an autoimmune disease whose prevalence is rarely reported in Africa. Autoantibodies are the biomarkers of the condition, precede overt disease and determine disease phenotypes. SSc specific autoantibodies also vary between racial groupings. Objective: To investigate the clinical and laboratory characteristics of Zimbabwean patients who were reactive SSc specific autoantibodies. MATERIALS AND METHOD: 240 patients, 173 of them female with SSc specific autoantibodies were included. Autoantibodies were detected by indirect immunofluorescence microscopy and immunoblotting using a panel of 13 SScl (Euroimmun Ag., Germany). Demographic, clinical and laboratory parameters relevant to the monitoring of SScl were captured. These included pulmonary function tests, hematology, clinical chemistry, serology and thyroid function tests. Allergy skin prick tests (SPT) to inhalant and food allergen sources were conducted when indicated. RESULTS: All the 240 patients (median age was 36 years) expressed SSc specific autoantibodies. 86% were Black, 11% White and 3% Asian and a fifth (20%) were younger than 16 years. Eleven (4.6%) fulfilled the ACR/EULAR classification of SSc. Clinically they had limited cutaneous (n=6), diffuse cutaneous (n=3) and SScl/inflammatory myopathy overlap (n=2). The most frequently detected antibodies anti-RNA polymerase III (RNAP) 55%, anti-Th/To (28%) anti-RNAP 11 (22%), anti-CENPB (18%) and anti-Scl-70/ATA (13%). Racial variations in the expression of these antibodies were apparent between Black, White and Asian patients. The majority (95%), who did not fulfil the ARA/EULAR criteria were symptomatic. Raynaud's Phenomenon was documented in 24%. Respiratory symptoms included coughing, dyspnea and wheezing. There was a restrictive ventilatory defect with increased FEV1/FVC ratio. Pruritus, urticaria and skin depigmentation were the main cutaneous features while constipation, bloating, Gastroesophageal reflux disease (GERD) and abdominal pain dominated GI symptoms. Mean blood pressure readings while normal varied with biomarkers. Haematology and biochemistry parameters were within normal reference ranges. CONCLUSION: The expression of SSc specific autoantibodies is common and associated with known SSc symptoms. The types and frequency of autoantibodies varied with racial groupings. A fifth of the patients were children below the age of 16 years. CI - Copyright © 2021 Sibanda, Dube, Chakawa, Mduluza and Mutapi. FAU - Sibanda, Elopy N AU - Sibanda EN AD - Department of Pathology, Faculty of Medicine, National University of Science and Technology, Bulawayo, Zimbabwe. AD - Asthma Allergy and Immunology Clinic, Harare, Zimbabwe. AD - Department of Pathophysiology and Allergy Research, Medical University of Vienna, Vienna, Austria. FAU - Dube, Yvonne AU - Dube Y AD - Laboratory Section, Asthma Allergy and Immunology Clinic, Harare, Zimbabwe. FAU - Chakawa, Mazvita AU - Chakawa M AD - Department of Biochemistry, University of Zimbabwe, Harare, Zimbabwe. FAU - Mduluza, Takafira AU - Mduluza T AD - Department of Biochemistry, University of Zimbabwe, Harare, Zimbabwe. FAU - Mutapi, Francisca AU - Mutapi F AD - Institute of Immunology and Infection Research, School of Biological Sciences, University of Edinburgh, Edinburgh, United Kingdom. LA - eng GR - 16/136/33/DH_/Department of Health/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211109 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - *Age Factors MH - Autoantibodies/metabolism MH - Autoimmune Diseases/epidemiology/*immunology MH - Biomarkers/metabolism MH - Child MH - Child, Preschool MH - Humans MH - Racial Groups MH - Raynaud Disease MH - Scleroderma, Systemic/epidemiology/*immunology MH - Seroepidemiologic Studies MH - Young Adult MH - Zimbabwe/epidemiology PMC - PMC8631108 OTO - NOTNLM OT - Zimbabwe OT - autoantibodies OT - clinical OT - cutaneous OT - laboratory OT - respiratory OT - systemic sclerosis COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/12/04 06:00 MHDA- 2022/01/08 06:00 PMCR- 2021/01/01 CRDT- 2021/12/03 07:02 PHST- 2021/03/12 00:00 [received] PHST- 2021/09/30 00:00 [accepted] PHST- 2021/12/03 07:02 [entrez] PHST- 2021/12/04 06:00 [pubmed] PHST- 2022/01/08 06:00 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2021.679531 [doi] PST - epublish SO - Front Immunol. 2021 Nov 9;12:679531. doi: 10.3389/fimmu.2021.679531. eCollection 2021. PMID- 34823828 OWN - NLM STAT- MEDLINE DCOM- 20211210 LR - 20211214 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 17 IP - 10 DP - 2021 Dec TI - Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas. PG - 601-606 LID - S2173-5743(21)00197-0 [pii] LID - 10.1016/j.reumae.2020.06.015 [doi] AB - OBJECTIVE: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. METHODS: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. RESULTS: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077). CONCLUSIONS: Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement. CI - Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Rivas-Vargas, Daniel AU - Rivas-Vargas D AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Caracas, Venezuela. Electronic address: rivasvargas4@gmail.com. FAU - Snih, Soham Al AU - Snih SA AD - Division of Rehabilitation Sciences/School of Health Professions, Division of Geriatrics/Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, United States. FAU - Rodríguez, Martín A AU - Rodríguez MA AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Caracas, Venezuela; Sealy Center on Aging, University of Texas Medical Branch, Galveston, TX, United States. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Adult MH - Aged MH - Female MH - Hispanic or Latino MH - Humans MH - *Lung Diseases, Interstitial/epidemiology MH - Middle Aged MH - *Raynaud Disease MH - *Scleroderma, Systemic/epidemiology MH - Tertiary Care Centers OTO - NOTNLM OT - Demografía OT - Demography OT - Epidemiology OT - Epidemiología OT - Esclerosis sistémica OT - Hispanic OT - Hispanos OT - Systemic sclerosis EDAT- 2021/11/27 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/11/26 05:44 PHST- 2019/09/12 00:00 [received] PHST- 2020/06/24 00:00 [accepted] PHST- 2021/11/26 05:44 [entrez] PHST- 2021/11/27 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] AID - S2173-5743(21)00197-0 [pii] AID - 10.1016/j.reumae.2020.06.015 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2021 Dec;17(10):601-606. doi: 10.1016/j.reumae.2020.06.015. PMID- 32736594 OWN - NLM STAT- MEDLINE DCOM- 20210906 LR - 20221207 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 60 IP - 1 DP - 2020 Jul 31 TI - Anti-annexin V autoantibodies and vascular abnormalities in systemic sclerosis: a longitudinal study. PG - 38 LID - 10.1186/s42358-020-00140-w [doi] AB - BACKGROUND: Annexins are a group of conserved proteins which exert several regulatory functions on various cellular activities. Increased frequency and levels of antibodies against annexin V have already been observed in several autoimmune diseases including systemic sclerosis (SSc), but their role as a vascular biomarker is unknown. The aim of this study was to determine the serum levels and the dynamical behavior of anti-annexin V antibodies over a 24 months follow-up in patients with SSc. METHODS: In this bicentric cross-sectional study, 70 patients with SSc were consecutively selected from March 2016 to April 2017. Demographic and clinical features, including the presence of active DUs, were collected. Serum anti-annexin V IgG and IgM antibodies were measured at baseline and after 6, 12 and 24 months of follow-up. Videocapillaroscopy was performed in all patients. RESULTS: Among the 70 SSc patients included anti-annexin V IgG was found in 11 patients (15.7%) (range of 15.88-39.48 U/mL) and anti-annexin V IgM in 10 patients (14.3%) (range of 14.16-22.69 U/mL) at baseline. During follow-up, the number of patients who were positive for anti-annexin V IgG and IgM remained stable over 24 months. Among the patients with positive anti-annexin V IgG at baseline the frequency of patients with necrosis or amputation of extremities, forced vital capacity less than 70% and pulmonary arterial hypertension (PAH) was significantly higher than in patients with negative anti-annexin V IgG antibodies. Patients with anti-annexin V IgG had also a higher Raynaud's Condition Score and a higher Health Assessment Questionnaire Disability Index (HAQ-DI) than patients without these antibodies at baseline. Patients with positive anti-annexin V IgM at baseline presented a higher frequency of PAH, compared to those with negative anti-annexin V IgM at baseline. CONCLUSIONS: Anti-annexin V antibodies are stable and do not change their positivity during a 24 month follow-up in SSc patients. Anti-annexin V IgG was associated with more severe interstitial lung involvement and digital microangiopathy, and patients with anti-annexin V IgG or IgM had a higher occurrence of PAH indicating an association of these biomarker with more severe disease. FAU - Horimoto, Alex Magno Coelho AU - Horimoto AMC AUID- ORCID: 0000-0002-5137-7719 AD - Rheumatology Division, Universidade Federal do Mato Grosso do Sul, Av. Senador Filinto Muller s/n°, Campo Grande, Mato Grosso do Sul, 79080-190, Brazil. clinicaactivite@gmail.com. FAU - de Jesus, Laize Guerreiro AU - de Jesus LG AD - Rheumatology Division, Universidade Federal do Mato Grosso do Sul, Av. Senador Filinto Muller s/n°, Campo Grande, Mato Grosso do Sul, 79080-190, Brazil. FAU - de Souza, Albert Schiaveto AU - de Souza AS AD - Institute of Biosciences, Universidade Federal do Mato Grosso do Sul, Campo Grande, Mato Grosso do Sul, Brazil. FAU - Rodrigues, Silvia Helena AU - Rodrigues SH AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20200731 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 RN - 0 (Annexin A5) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) SB - IM MH - Amputation, Surgical/statistics & numerical data MH - Annexin A5/*immunology MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Cross-Sectional Studies MH - Female MH - Follow-Up Studies MH - Humans MH - Immunoglobulin G/*blood MH - Immunoglobulin M/*blood MH - Longitudinal Studies MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/epidemiology MH - Scleroderma, Systemic/*immunology MH - Skin Ulcer/epidemiology MH - Time Factors OTO - NOTNLM OT - Anti-annexin V OT - Biomarkers OT - Digital ulcers OT - Systemic sclerosis OT - Vasculopathy EDAT- 2020/08/02 06:00 MHDA- 2021/09/07 06:00 CRDT- 2020/08/02 06:00 PHST- 2020/05/11 00:00 [received] PHST- 2020/07/20 00:00 [accepted] PHST- 2020/08/02 06:00 [entrez] PHST- 2020/08/02 06:00 [pubmed] PHST- 2021/09/07 06:00 [medline] AID - 10.1186/s42358-020-00140-w [pii] AID - 10.1186/s42358-020-00140-w [doi] PST - epublish SO - Adv Rheumatol. 2020 Jul 31;60(1):38. doi: 10.1186/s42358-020-00140-w. PMID- 30657102 OWN - NLM STAT- MEDLINE DCOM- 20200330 LR - 20200330 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 59 IP - 1 DP - 2019 Jan 6 TI - Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients. PG - 1 LID - 10.1186/s42358-018-0045-5 [doi] AB - BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients. METHODS: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients. RESULTS: Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011). CONCLUSION: In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication. FAU - Donnarumma, Juliana Fernandes Sarmento AU - Donnarumma JFS AD - Rheumatology Division, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. FAU - Ferreira, Eloara Vieira Machado AU - Ferreira EVM AD - Division of Pneumology, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. FAU - Ota-Arakaki, Jaquelina AU - Ota-Arakaki J AD - Division of Pneumology, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. FAU - Kayser, Cristiane AU - Kayser C AUID- ORCID: 0000-0003-0543-5305 AD - Rheumatology Division, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil. criskayser@terra.com.br. AD - Disciplina de Reumatologia da Universidade Federal de São Paulo, Rua Botucatu 740, 3 ° andar, São Paulo, SP, 04023-062, Brazil. criskayser@terra.com.br. LA - eng PT - Journal Article DEP - 20190106 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 SB - IM MH - Adult MH - Cardiac Catheterization/methods MH - Case-Control Studies MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/drug therapy MH - Male MH - *Microscopic Angioscopy MH - Pregnancy MH - Pregnancy Outcome MH - Pulmonary Arterial Hypertension/diagnosis/drug therapy/*etiology MH - Raynaud Disease MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Localized MH - Scleroderma, Systemic/*complications/diagnostic imaging OTO - NOTNLM OT - Nailfold capillaroscopy OT - Pulmonary arterial hypertension OT - Risk factors OT - Systemic lupus erythematosus EDAT- 2019/01/19 06:00 MHDA- 2020/03/31 06:00 CRDT- 2019/01/19 06:00 PHST- 2018/11/20 00:00 [received] PHST- 2018/12/20 00:00 [accepted] PHST- 2019/01/19 06:00 [entrez] PHST- 2019/01/19 06:00 [pubmed] PHST- 2020/03/31 06:00 [medline] AID - 10.1186/s42358-018-0045-5 [pii] AID - 10.1186/s42358-018-0045-5 [doi] PST - epublish SO - Adv Rheumatol. 2019 Jan 6;59(1):1. doi: 10.1186/s42358-018-0045-5. PMID- 34916687 OWN - NLM STAT- MEDLINE DCOM- 20211220 LR - 20220105 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 53 IP - 6 DP - 2021 Dec 18 TI - [Clinical and immunological characteristics of 88 cases of overlap myositis]. PG - 1088-1093 AB - OBJECTIVE: To investigate the clinical and immunological characteristics of overlap myositis (OM) patients. METHODS: The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People's Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron' s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic' s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud' s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival. RESULTS: A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5, 48.0) months vs. 4.0(2.0, 12.0) months, P < 0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud's phenomenon (14.8% vs. 1.8%, P < 0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P < 0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P < 0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients. CONCLUSION: Pulmonary hypertension and myocardial involvement were frequently observed in OM. FAU - Xiao, Y S AU - Xiao YS AD - Department of Pathology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhu, F Y AU - Zhu FY AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Luo, L AU - Luo L AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Xing, X Y AU - Xing XY AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Y H AU - Li YH AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhang, X W AU - Zhang XW AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Shen, D H AU - Shen DH AD - Department of Pathology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - *Dermatomyositis/epidemiology MH - Female MH - Humans MH - *Myositis/epidemiology MH - *Raynaud Disease MH - Retrospective Studies PMC - PMC8695150 OTO - NOTNLM OT - Autoantibodies OT - Idiopathic inflammatory myopathy OT - Overlap myositis OT - Pulmonary hypertension EDAT- 2021/12/18 06:00 MHDA- 2021/12/21 06:00 PMCR- 2021/12/18 CRDT- 2021/12/17 06:49 PHST- 2021/12/17 06:49 [entrez] PHST- 2021/12/18 06:00 [pubmed] PHST- 2021/12/21 06:00 [medline] PHST- 2021/12/18 00:00 [pmc-release] AID - bjdxxbyxb-53-6-1088 [pii] AID - 10.19723/j.issn.1671-167X.2021.06.014 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Dec 18;53(6):1088-1093. doi: 10.19723/j.issn.1671-167X.2021.06.014. PMID- 23176853 OWN - NLM STAT- MEDLINE DCOM- 20130726 LR - 20121126 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 139 IP - 11 Suppl DP - 2012 Oct TI - [Item 327--Raynaud phenomenon]. PG - A223-6 LID - S0151-9638(12)00490-5 [pii] LID - 10.1016/j.annder.2012.06.027 [doi] CN - CEDEF LA - fre PT - Journal Article TT - Item 327 - Phénomène de Raynaud. DEP - 20120919 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Adult MH - Antibodies, Antinuclear/blood MH - Autoimmune Diseases/complications MH - Chilblains/diagnosis MH - Cold Temperature/adverse effects MH - Cyanosis/diagnosis MH - Diagnosis, Differential MH - Disease Management MH - Emotions MH - Erythromelalgia/diagnosis MH - Female MH - Fingers/blood supply MH - Humans MH - Humidity/adverse effects MH - Ischemia/diagnosis MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/blood/chemically induced/*diagnosis/etiology/psychology MH - Scleroderma, Systemic/complications/diagnosis MH - Smoking/adverse effects MH - Ultrasonography, Doppler MH - Vascular Diseases/complications EDAT- 2012/12/05 06:00 MHDA- 2013/07/28 06:00 CRDT- 2012/11/27 06:00 PHST- 2012/11/27 06:00 [entrez] PHST- 2012/12/05 06:00 [pubmed] PHST- 2013/07/28 06:00 [medline] AID - S0151-9638(12)00490-5 [pii] AID - 10.1016/j.annder.2012.06.027 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2012 Oct;139(11 Suppl):A223-6. doi: 10.1016/j.annder.2012.06.027. Epub 2012 Sep 19. PMID- 38810160 OWN - NLM STAT- MEDLINE DCOM- 20240529 LR - 20260520 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 153 IP - 6 DP - 2024 Jun 1 TI - Discussion: Neurectomy of the Nerve of Henle Associated with Periarterial Sympathectomy for Management of Intractable Raynaud Phenomenon. PG - 1345-1346 LID - 10.1097/PRS.0000000000011066 [doi] FAU - Merritt, Wyndell H AU - Merritt WH AD - From Virginia Commonwealth University. LA - eng PT - Journal Article DEP - 20240520 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM MH - Humans MH - *Sympathectomy/methods MH - *Raynaud Disease/surgery/diagnosis/therapy EDAT- 2024/05/29 18:43 MHDA- 2024/05/29 18:44 CRDT- 2024/05/29 15:43 PHST- 2024/05/29 18:44 [medline] PHST- 2024/05/29 18:43 [pubmed] PHST- 2024/05/29 15:43 [entrez] AID - 00006534-202406000-00028 [pii] AID - 10.1097/PRS.0000000000011066 [doi] PST - ppublish SO - Plast Reconstr Surg. 2024 Jun 1;153(6):1345-1346. doi: 10.1097/PRS.0000000000011066. Epub 2024 May 20. PMID- 29624870 OWN - NLM STAT- MEDLINE DCOM- 20181015 LR - 20260127 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 21 IP - 5 DP - 2018 May TI - Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome. PG - 1068-1075 LID - 10.1111/1756-185X.13290 [doi] AB - AIM: To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS. METHODS: This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were analyzed by univariate binary logistic regression to identify possible risk factors for PAH. RESULTS: Twenty-nine patients with RHC-confirmed pSS-PAH were included (mean age at onset, 38.4 ± 8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestation of pSS in 12 patients (41.4%), and shortness of breath was the most common symptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg, mean cardiac index was 2.3 ± 0.8 L/min/m(2) , and mean pulmonary vascular resistance was 13.0 ± 6.0 Wood units in this group. Treatments included immunosuppressive agents (93.1%) and PAH-targeted therapies (86.2%). We identified four independent risk factors for PAH in pSS: Raynaud's phenomenon (odds ratio [OR] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/mL (OR = 6.691, P = 0.001), hepatic injury (OR = 3.284, P = 0.008) and pericardial effusion (OR = 3.279, P = 0.016). CONCLUSIONS: PAH can be the first manifestation of pSS. The pSS patients with Raynaud's phenomenon, high-titer rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH. CI - © 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Yan, Shumin AU - Yan S AUID- ORCID: 0000-0003-4945-7602 AD - Department of Rheumatology, Beijing Jishuitan Hospital, Beijing, China. AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Li, Mengtao AU - Li M AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Wang, Hui AU - Wang H AD - Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Yang, Xiaoxi AU - Yang X AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Zhao, Jiuliang AU - Zhao J AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Wang, Qian AU - Wang Q AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Liu, Yongtai AU - Liu Y AD - Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Lai, Jinzhi AU - Lai J AD - Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Tian, Zhuang AU - Tian Z AD - Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Song, Hui AU - Song H AD - Department of Rheumatology, Beijing Jishuitan Hospital, Beijing, China. FAU - Zhao, Yan AU - Zhao Y AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Zeng, Xiaofeng AU - Zeng X AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. LA - eng PT - Journal Article DEP - 20180406 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Antihypertensive Agents) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Adult MH - Antihypertensive Agents/therapeutic use MH - *Arterial Pressure/drug effects MH - Biomarkers/blood MH - Cardiac Catheterization MH - Case-Control Studies MH - Chi-Square Distribution MH - China/epidemiology MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/drug therapy/*epidemiology/physiopathology MH - Immunosuppressive Agents/therapeutic use MH - Liver Diseases/epidemiology MH - Logistic Models MH - Male MH - Middle Aged MH - Odds Ratio MH - Pericardial Effusion/epidemiology MH - Pulmonary Artery/drug effects/*physiopathology MH - Raynaud Disease/epidemiology MH - Rheumatoid Factor/blood MH - Risk Factors MH - Sjogren's Syndrome/blood/diagnosis/drug therapy/*epidemiology MH - Young Adult OTO - NOTNLM OT - clinical characteristics OT - primary Sjögren's syndrome OT - pulmonary arterial hypertension OT - risk factors EDAT- 2018/04/07 06:00 MHDA- 2018/10/16 06:00 CRDT- 2018/04/07 06:00 PHST- 2018/04/07 06:00 [pubmed] PHST- 2018/10/16 06:00 [medline] PHST- 2018/04/07 06:00 [entrez] AID - 10.1111/1756-185X.13290 [doi] PST - ppublish SO - Int J Rheum Dis. 2018 May;21(5):1068-1075. doi: 10.1111/1756-185X.13290. Epub 2018 Apr 6. PMID- 32301427 OWN - NLM STAT- MEDLINE DCOM- 20200917 LR - 20200917 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 38 Suppl 125 IP - 3 DP - 2020 May-Jun TI - Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of the scleroderma spectrum. PG - 40-47 AB - OBJECTIVES: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). METHODS: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. RESULTS: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features. CONCLUSIONS: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors. FAU - Ferri, Clodoveo AU - Ferri C AD - University of Modena and Reggio Emilia, Italy. clferri@unimore.it. FAU - Giuggioli, Dilia AU - Giuggioli D AD - University of Modena and Reggio Emilia, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - University of Florence, Italy. FAU - Lumetti, Federica AU - Lumetti F AD - University of Modena and Reggio Emilia, Italy. FAU - Bajocchi, Gianluigi AU - Bajocchi G AD - Arcispedale S. Maria Nuova, Reggio Emilia, Italy. FAU - Magnani, Luca AU - Magnani L AD - Arcispedale S. Maria Nuova, Reggio Emilia, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Policlinico San Matteo, Pavia, Italy. FAU - Ariani, Alarico AU - Ariani A AD - AOU Parma, Italy. FAU - Girelli, Francesco AU - Girelli F AD - Ospedale GB Morgagni, Forlì, Italy. FAU - Riccieri, Valeria AU - Riccieri V AD - Università di Roma La Sapienza, Italy. FAU - Pellegrino, Greta AU - Pellegrino G AD - Università di Roma La Sapienza, Italy. FAU - Bosello, Silvia AU - Bosello S AD - Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, UOC di Reumatologia, Roma, Italy. FAU - Foti, Rosario AU - Foti R AD - AOU Policlinico Vittorio Emanuele, Catania, Italy. FAU - Visalli, Elisa AU - Visalli E AD - AOU Policlinico Vittorio Emanuele, Catania, Italy. FAU - Amato, Giorgio AU - Amato G AD - AOU Policlinico Vittorio Emanuele, Catania, Italy. FAU - Benenati, Alessia AU - Benenati A AD - AOU Policlinico Vittorio Emanuele, Catania, Italy. FAU - Cuomo, Giovanna AU - Cuomo G AD - Università degli Studi della Campania Luigi Vanvitelli, Napoli, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - UO Reumatologia, DETO, Università di Bari, Italy. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AD - UO Reumatologia, DETO, Università di Bari, Italy. FAU - De Angelis, Rossella AU - De Angelis R AD - Clinica Reumatologica, Università Politecnica delle Marche, Ancona, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Università degli Studi di Milano, Italy. FAU - Talotta, Rossella AU - Talotta R AD - Ospedale L. Sacco, Milano, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Ospedale S. Raffaele, Milano, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - Ospedale S. Raffaele, Milano, Italy. FAU - De Luca, Giacomo AU - De Luca G AD - Ospedale S. Raffaele, Milano, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Università di Firenze, Italy. FAU - Spinella, Amelia AU - Spinella A AD - University of Modena and Reggio Emilia, Italy. FAU - Murdaca, Giuseppe AU - Murdaca G AD - Ospedale Policlinico S. Martino, Università di Genova, Italy. FAU - Romeo, Nicoletta AU - Romeo N AD - ASO S. Croce e Carle, Cuneo, Italy. FAU - De Santis, Maria AU - De Santis M AD - Istituto Clinico Humanitas, Rozzano, Milano, Italy. FAU - Generali, Elena AU - Generali E AD - Istituto Clinico Humanitas, Rozzano, Milano, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - AOU Santa Chiara, Università di Pisa, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - AOU Santa Chiara, Università di Pisa, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Spedali Civili di Brescia, Italy. FAU - Dall'Ara, Francesca AU - Dall'Ara F AD - Spedali Civili di Brescia, and General Medicine Unit, Ospedale Maggiore di Lodi, Italy. FAU - Lazzaroni, Maria G AU - Lazzaroni MG AD - Spedali Civili and University of Brescia, Italy. FAU - Cozzi, Franco AU - Cozzi F AD - Università degli Studi di Padova, Italy. FAU - Doria, Andrea AU - Doria A AD - Università degli Studi di Padova, Italy. FAU - Pigatto, Erika AU - Pigatto E AD - Università degli Studi di Padova, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Università degli Studi di Padova, Italy. FAU - Ciano, Giovanni AU - Ciano G AD - ASL Avellino, Italy. FAU - Beretta, Lorenzo AU - Beretta L AD - Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - AOR San Carlo di Potenza, Italy. FAU - D'Angelo, Salvatore AU - D'Angelo S AD - AOR San Carlo di Potenza, Italy. FAU - Mennillo, Gianna AU - Mennillo G AD - AOR San Carlo di Potenza, Italy. FAU - Bagnato, Gianluca AU - Bagnato G AD - Università degli Studi di Messina, Italy. FAU - Calabrese, Francesca AU - Calabrese F AD - SSD Reumatologia, Reggio Calabria, Italy. FAU - Caminiti, Maurizio AU - Caminiti M AD - SSD Reumatologia, Reggio Calabria, Italy. FAU - Pagano Mariano, Giuseppa AU - Pagano Mariano G AD - SSD Reumatologia, Reggio Calabria, Italy. FAU - Battaglia, Elisabetta AU - Battaglia E AD - AO ARNAS Garibaldi, Catania, Italy. FAU - Lubrano, Ennio AU - Lubrano E AD - Università del Molise, Campobasso, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Policlinico San Matteo, Pavia, Italy. FAU - Iuliano, Annamaria AU - Iuliano A AD - AO San Camillo Forlanini, Roma, Italy. FAU - Furini, Federica AU - Furini F AD - Department of Medical Sciences, University of Ferrara, Italy. FAU - Zanetti, Anna AU - Zanetti A AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Carrara, Greta AU - Carrara G AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Rumi, Federica AU - Rumi F AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Scirè, Carlo Alberto AU - Scirè CA AD - Department of Medical Sciences, University of Ferrara, and Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - University of Florence, Italy. CN - Italian Society for Rheumatology (SIR) LA - eng PT - Journal Article DEP - 20200414 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Cohort Studies MH - Humans MH - Italy MH - Male MH - Microscopic Angioscopy MH - *Raynaud Disease MH - Registries MH - *Scleroderma, Systemic EDAT- 2020/04/18 06:00 MHDA- 2020/09/18 06:00 CRDT- 2020/04/18 06:00 PHST- 2019/09/13 00:00 [received] PHST- 2019/12/03 00:00 [accepted] PHST- 2020/04/18 06:00 [pubmed] PHST- 2020/09/18 06:00 [medline] PHST- 2020/04/18 06:00 [entrez] AID - 14762 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2020 May-Jun;38 Suppl 125(3):40-47. Epub 2020 Apr 14. PMID- 23158111 OWN - NLM STAT- MEDLINE DCOM- 20130805 LR - 20130128 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 38 IP - 2 DP - 2013 Feb TI - Raynaud phenomenon. PG - 375-7; quiz 378 LID - S0363-5023(12)01212-9 [pii] LID - 10.1016/j.jhsa.2012.08.035 [doi] FAU - Porter, Steven B AU - Porter SB AD - Departments of Anesthesiology and Orthopedic Surgery, Mayo Clinic, Jacksonville, FL 32224, USA. FAU - Murray, Peter M AU - Murray PM LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20121113 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 SB - IM MH - Adrenergic Fibers/physiology MH - Angiography MH - Cold Temperature/adverse effects MH - Diagnosis, Differential MH - Female MH - Fingers/blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Middle Aged MH - Raynaud Disease/*diagnosis/etiology/physiopathology/surgery MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/physiopathology MH - Sympathectomy EDAT- 2012/11/20 06:00 MHDA- 2013/08/06 06:00 CRDT- 2012/11/20 06:00 PHST- 2012/08/20 00:00 [received] PHST- 2012/08/25 00:00 [accepted] PHST- 2012/11/20 06:00 [entrez] PHST- 2012/11/20 06:00 [pubmed] PHST- 2013/08/06 06:00 [medline] AID - S0363-5023(12)01212-9 [pii] AID - 10.1016/j.jhsa.2012.08.035 [doi] PST - ppublish SO - J Hand Surg Am. 2013 Feb;38(2):375-7; quiz 378. doi: 10.1016/j.jhsa.2012.08.035. Epub 2012 Nov 13. PMID- 18984220 OWN - NLM STAT- MEDLINE DCOM- 20090304 LR - 20081105 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 135 IP - 11 Suppl DP - 2008 Nov TI - [Item 327: Raynaud disease]. PG - F218-21 LID - 10.1016/j.annder.2008.07.051 [doi] CN - CEDEF LA - fre PT - Journal Article TT - Item 327--Phénomène de Raynaud. PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Diagnosis, Differential MH - Humans MH - Raynaud Disease/*diagnosis/*etiology EDAT- 2008/12/17 09:00 MHDA- 2009/03/05 09:00 CRDT- 2008/12/17 09:00 PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/03/05 09:00 [medline] PHST- 2008/12/17 09:00 [entrez] AID - S0151-9638(08)00538-3 [pii] AID - 10.1016/j.annder.2008.07.051 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2008 Nov;135(11 Suppl):F218-21. doi: 10.1016/j.annder.2008.07.051. PMID- 26280234 OWN - NLM STAT- MEDLINE DCOM- 20171026 LR - 20171026 IS - 1532-0650 (Electronic) IS - 0002-838X (Linking) VI - 92 IP - 4 DP - 2015 Aug 15 TI - Discoloration in the Hands. PG - 295-6 FAU - Aguilar Shea, Antonio L AU - Aguilar Shea AL AD - Centro de Salud Puerta de Madrid, Atencion Primaria, Madrid, Spain. FAU - Gallardo-Mayo, Cristina AU - Gallardo-Mayo C AD - Hospital Infanta Leonor, Madrid, Spain. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 SB - IM MH - Adult MH - Arterial Occlusive Diseases/*diagnosis MH - Cold Temperature/adverse effects MH - Diagnosis, Differential MH - Disease Management MH - Female MH - Fingers MH - Frostbite/*diagnosis MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - *Lupus Nephritis/complications/diagnosis MH - *Raynaud Disease/diagnosis/etiology/physiopathology/therapy MH - Skin Pigmentation/*physiology MH - Toes EDAT- 2015/08/19 06:00 MHDA- 2015/08/19 06:01 CRDT- 2015/08/18 06:00 PHST- 2015/08/18 06:00 [entrez] PHST- 2015/08/19 06:00 [pubmed] PHST- 2015/08/19 06:01 [medline] AID - d12189 [pii] PST - ppublish SO - Am Fam Physician. 2015 Aug 15;92(4):295-6. PMID- 16604250 OWN - NLM STAT- MEDLINE DCOM- 20060818 LR - 20131121 IS - 1684-1182 (Print) IS - 1684-1182 (Linking) VI - 39 IP - 2 DP - 2006 Apr TI - Pulmonary arterial hypertension in autoimmune diseases: an analysis of 19 cases from a medical center in northern Taiwan. PG - 162-8 AB - BACKGROUND AND PURPOSE: Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan. METHODS: Nineteen patients with various autoimmune diseases diagnosed with PAH at Taipei Veterans General Hospital from 2002 to 2004 were enrolled; the underlying autoimmune diseases included systemic lupus erythematosus (n = 6), primary Sjögren's syndrome (n = 5), systemic sclerosis (n = 4), adult-onset Still's disease (n = 2), and mixed connective tissue disease (n = 2). The characteristic manifestations of underlying autoimmune diseases and the clinical features of PAH were analyzed. RESULTS: There were 16 female and 3 male patients. The median age at onset of PAH was 44 years and the mean right ventricular systolic pressure (RVSP) was 67.9 mm Hg. Patients without pneumonitis had a significantly higher RVSP value than those with pneumonitis (77.5 +/- 24.3 vs 54.8 +/- 18.4 mm Hg, p=0.041). Four out of 7 patients (57.1%) with RVSP >or=80 mm Hg and 1 out of 12 patients (8.3%) with RVSP <80 mm Hg died. In all of the 19 patients, the severity of RVSP was significantly correlated with serum uric acid (UA) level (r = 0.686, p=0.001). Among the PAH patients without pneumonitis, the severity of RVSP inversely correlated with the diffusion capacity of the lung for carbon monoxide (DLCO) [r = -0.856, p=0.003]. The characteristic manifestations of underlying autoimmune diseases included a high incidence of Raynaud's phenomenon (15/19, 78.9%), a high titer of antinuclear antibody (13/17, 76.5%), positive anti-ribonucleoprotein antibody (8/15, 53.3%), hypergammaglobulinemia (15/19, 78.9%), hyperuricemia (13/19, 68.4%), and less renal involvement. CONCLUSIONS: PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO. FAU - Chen, Chun-Hsiung AU - Chen CH AD - Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan. FAU - Chen, Horng-An AU - Chen HA FAU - Wang, Hong-Pin AU - Wang HP FAU - Liao, Hsien-Tzung AU - Liao HT FAU - Chou, Chung-Tei AU - Chou CT FAU - Huang, De-Feng AU - Huang DF LA - eng PT - Journal Article PL - England TA - J Microbiol Immunol Infect JT - Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi JID - 100956211 RN - 0 (Antibodies, Antinuclear) RN - 0 (deoxyribonucleoprotein antibodies) RN - 268B43MJ25 (Uric Acid) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/blood MH - Autoimmune Diseases/*complications MH - Female MH - Hospitals MH - Humans MH - Hypergammaglobulinemia MH - Hypertension, Pulmonary/*etiology/mortality/*physiopathology MH - Male MH - Middle Aged MH - Pneumonia MH - Raynaud Disease MH - Taiwan MH - Uric Acid/blood MH - Ventricular Pressure EDAT- 2006/04/11 09:00 MHDA- 2006/08/19 09:00 CRDT- 2006/04/11 09:00 PHST- 2006/04/11 09:00 [pubmed] PHST- 2006/08/19 09:00 [medline] PHST- 2006/04/11 09:00 [entrez] PST - ppublish SO - J Microbiol Immunol Infect. 2006 Apr;39(2):162-8. PMID- 28281457 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20220410 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 Suppl 106 IP - 4 DP - 2017 Sep-Oct TI - Evaluation of the effect of sildenafil on the microvascular blood flow in patients with systemic sclerosis: a randomised, double-blind, placebo-controlled study. PG - 151-158 AB - OBJECTIVES: To evaluate the effect of sildenafil as add-on therapy on the microvascular blood flow in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). METHODS: In this double-blind, placebo-controlled study, 41 patients with RP secondary to SSc were randomly assigned to receive oral sildenafil 100 mg/day (21 patients, mean age 47.2 years) or placebo (20 patients, mean age 41.6 years) for 8 weeks. Patients were evaluated at baseline, 8 weeks after treatment, and 2 weeks after the end of the treatment. The primary outcome measures were the mean changes in finger blood flow (FBF) measured using laser Doppler imaging before and after cold stimulus at 8 weeks of treatment. Secondary endpoints included frequency and duration of RP attacks, Visual Analog Scale (VAS) score for RP severity, Raynaud's condition score, and serum levels of VEGF and endothelial progenitor cells (EPCs). RESULTS: After 8 weeks of treatment, the sildenafil group presented a significantly higher mean percentage change from baseline in FBF before cold stimulus (p=0.026), and in FBF after cold stimulus (p=0.028) compared with the placebo group. There was a significant improvement in the duration of RP and in the percentage change from baseline to week 8 in the RP VAS score in sildenafil compared with placebo. There were no changes in EPCs and VEGF levels after treatment in either group. CONCLUSIONS: Sildenafil improved digital blood flow and RP symptoms in SSc patients after 8 weeks of treatment, and might be a good therapeutic option for secondary RP. FAU - Andrigueti, Fernando V AU - Andrigueti FV AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil. FAU - Ebbing, Pâmela C C AU - Ebbing PCC AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil. FAU - Arismendi, Maria I AU - Arismendi MI AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil. cristiane.kayser@unifesp.br. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20170309 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vascular Endothelial Growth Factor A) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Adult MH - Double-Blind Method MH - Endothelial Progenitor Cells/physiology MH - Fingers/blood supply MH - Humans MH - Microcirculation/drug effects MH - Middle Aged MH - Raynaud Disease MH - Scleroderma, Systemic/*drug therapy/physiopathology MH - Sildenafil Citrate/pharmacology/*therapeutic use MH - Vascular Endothelial Growth Factor A/blood EDAT- 2017/03/11 06:00 MHDA- 2017/12/22 06:00 CRDT- 2017/03/11 06:00 PHST- 2016/11/08 00:00 [received] PHST- 2017/02/06 00:00 [accepted] PHST- 2017/03/11 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/03/11 06:00 [entrez] AID - 11300 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):151-158. Epub 2017 Mar 9. PMID- 34402690 OWN - NLM STAT- MEDLINE DCOM- 20211223 LR - 20231102 IS - 1365-2060 (Electronic) IS - 0785-3890 (Print) IS - 0785-3890 (Linking) VI - 53 IP - 1 DP - 2021 Dec TI - Clinical profiles of SS-ILD compared with SS-NILD in a Chinese population: a retrospective analysis of 735 patients. PG - 1340-1348 LID - 10.1080/07853890.2021.1965205 [doi] AB - BACKGROUND: Interstitial lung disease (ILD) is a serious complication in patients with Sjögren's syndrome (SS). Most studies on primary SS (pSS) with ILD are limited in sample size, and studies on secondary SS (sSS) with ILD are rare. This study aimed to elucidate both primary and secondary SS-associated ILD (SS-ILD) based on a large cohort. METHODS: The medical records of hospitalized patients diagnosed with SS at the Second Xiangya Hospital of Central South University from January 2010 to May 2020 were retrospectively reviewed. Clinical manifestations, medical history, biological results and imaging data were collected. RESULTS: Of the 735 SS patients enrolled in this study, 563 (76.6%) were diagnosed with pSS, 172 (23.4%) were diagnosed with sSS. Additionally, 316 (43.0%) were diagnosed with SS-ILD. No significant difference was found between the pSS and sSS groups concerning the incidence of ILD (p = .718). Factors associated with SS-ILD were older age (p < .001), male sex (p = .032), female sex at menopause (p = .002), Raynaud's phenomenon (p < .001), low levels of albumin (p = .010) and respiratory symptoms (p < .001). The SS-ILD group showed higher counts of platelets (p < .001). The three most frequent high-resolution CT (HRCT) findings of SS-ILD were irregular linear opacities (42.7%), grid shadows (30.7%) and pleural thickening (28.5%). NSIP (56.3%) was the most frequent HRCT pattern. Compared with pSS patients with ILD (pSS-ILD) patients, sSS patients with ILD (sSS-ILD) patients had a higher incidence of proteinuria (p < .001) and hypercreatinaemia (p = .013), a higher level of erythrocyte sedimentation rate (ESR) (p = .003), low levels of complement 3 (C3) (p = .013), lymphocytes (p = .009) and leukocytes (p = .024), and worse DLCO (%Pred) (p = .035). CONCLUSIONS: ILD is a common pulmonary involvement in both pSS patients and sSS patients. Older age, male sex, female sex at menopause, Raynaud's phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. NSIP is important HRCT feature of SS-ILD. sSS-ILD patients showed worse laboratory results and pulmonary function.KEY MESSAGEOlder age, male sex, female sex at menopause, Raynaud's phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD.SS-ILD patients show higher counts of platelets and less purpura.sSS-ILD patients have worse laboratory results and pulmonary function. FAU - Guo, Ting AU - Guo T AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Long, Yaomei AU - Long Y AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Shen, Qinxue AU - Shen Q AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Guo, Wei AU - Guo W AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Duan, Wang AU - Duan W AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Ouyang, Xiaoli AU - Ouyang X AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. FAU - Peng, Hong AU - Peng H AD - Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital of Central-South University, Changsha, China. AD - Research Unit of Respiratory Disease, Central-South University, Changsha, China. AD - The Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha, China. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Ann Med JT - Annals of medicine JID - 8906388 RN - 0 (Serum Albumin) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - China/epidemiology MH - Female MH - Humans MH - Incidence MH - Lung Diseases, Interstitial/diagnostic imaging/*ethnology MH - Male MH - Middle Aged MH - Raynaud Disease MH - Retrospective Studies MH - Serum Albumin MH - Sjogren's Syndrome/diagnosis/*ethnology MH - Young Adult PMC - PMC8382016 OTO - NOTNLM OT - Interstitial lung disease OT - primary Sjögren’s syndrome OT - pulmonary fibrosis OT - secondary Sjögren’s syndrome COIS- The authors report no declarations of interest. EDAT- 2021/08/18 06:00 MHDA- 2021/12/24 06:00 PMCR- 2021/08/17 CRDT- 2021/08/17 12:14 PHST- 2021/08/17 12:14 [entrez] PHST- 2021/08/18 06:00 [pubmed] PHST- 2021/12/24 06:00 [medline] PHST- 2021/08/17 00:00 [pmc-release] AID - 1965205 [pii] AID - 10.1080/07853890.2021.1965205 [doi] PST - ppublish SO - Ann Med. 2021 Dec;53(1):1340-1348. doi: 10.1080/07853890.2021.1965205. PMID- 24646581 OWN - NLM STAT- MEDLINE DCOM- 20140506 LR - 20140320 IS - 1538-8689 (Electronic) IS - 0360-4039 (Linking) VI - 44 IP - 4 DP - 2014 Apr TI - Getting in touch with Raynaud phenomenon. PG - 57-9 LID - 10.1097/01.NURSE.0000444727.60395.f8 [doi] FAU - Vacca, Vincent M Jr AU - Vacca VM Jr AD - Vincent M. Vacca, Jr., is a clinical nurse educator in the neuroscience ICU at Brigham and Women's Hospital in Boston, Mass. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Nursing JT - Nursing JID - 7600137 MH - Adult MH - Body Temperature/physiology MH - Female MH - Humans MH - Raynaud Disease/diagnosis/*nursing/physiopathology/therapy MH - Risk Factors MH - Skin Pigmentation/physiology EDAT- 2014/03/22 06:00 MHDA- 2014/05/07 06:00 CRDT- 2014/03/21 06:00 PHST- 2014/03/21 06:00 [entrez] PHST- 2014/03/22 06:00 [pubmed] PHST- 2014/05/07 06:00 [medline] AID - 00152193-201404000-00014 [pii] AID - 10.1097/01.NURSE.0000444727.60395.f8 [doi] PST - ppublish SO - Nursing. 2014 Apr;44(4):57-9. doi: 10.1097/01.NURSE.0000444727.60395.f8. PMID- 34686269 OWN - NLM STAT- MEDLINE DCOM- 20211026 LR - 20211026 IS - 1558-2264 (Electronic) IS - 0733-8651 (Linking) VI - 39 IP - 4 DP - 2021 Nov TI - Raynaud Phenomenon and Other Vasospastic Disorders. PG - 583-599 LID - S0733-8651(21)00060-6 [pii] LID - 10.1016/j.ccl.2021.06.010 [doi] AB - Vasospastic disorders are prevalent in the general population and can affect individuals of any age. Primary (or idiopathic) vasospastic disorders often have a benign course; treatment focuses on the control of symptoms. Secondary vasospastic disorders occur owing to an underlying condition and have an increased risk of complications, including tissue loss and digital ulcerations; treatment should focus on the underlying condition. In this review, we discuss the pathophysiology, clinical presentation, diagnosis, and management of vasospastic disorders, including Raynaud syndrome, acrocyanosis, livedo reticularis, and pernio. CI - Copyright © 2021 Elsevier Inc. All rights reserved. FAU - Casanegra, Ana I AU - Casanegra AI AD - Vascular Medicine Division, Cardiovascular Department, Gonda Vascular Center, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA. Electronic address: Casanegra.ana@mayo.edu. FAU - Shepherd, Roger F AU - Shepherd RF AD - Vascular Medicine Division, Cardiovascular Department, Gonda Vascular Center, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA. LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Cardiol Clin JT - Cardiology clinics JID - 8300331 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/epidemiology/therapy OTO - NOTNLM OT - Acrocyanosis OT - Livedo reticularis OT - Pernio OT - Primary Raynaud phenomenon OT - Raynaud syndrome OT - Secondary Raynaud phenomenon OT - Vasospastic disorders COIS- Disclosure The authors have nothing to disclose. EDAT- 2021/10/24 06:00 MHDA- 2021/10/27 06:00 CRDT- 2021/10/23 05:28 PHST- 2021/10/23 05:28 [entrez] PHST- 2021/10/24 06:00 [pubmed] PHST- 2021/10/27 06:00 [medline] AID - S0733-8651(21)00060-6 [pii] AID - 10.1016/j.ccl.2021.06.010 [doi] PST - ppublish SO - Cardiol Clin. 2021 Nov;39(4):583-599. doi: 10.1016/j.ccl.2021.06.010. PMID- 27114484 OWN - NLM STAT- MEDLINE DCOM- 20170403 LR - 20220331 IS - 1488-2329 (Electronic) IS - 0820-3946 (Print) IS - 0820-3946 (Linking) VI - 188 IP - 15 DP - 2016 Oct 18 TI - Raynaud phenomenon causing lingual pallor and dysarthria. PG - E396 LID - 10.1503/cmaj.150547 [doi] FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, Ohio chattes@ccf.org. LA - eng PT - Case Reports PT - Journal Article DEP - 20160425 PL - Canada TA - CMAJ JT - CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne JID - 9711805 SB - IM MH - Adult MH - Dysarthria/etiology/*physiopathology MH - Female MH - Humans MH - Pallor/etiology/*physiopathology MH - Raynaud Disease/complications/diagnosis/*physiopathology MH - Tongue/blood supply MH - Tongue Diseases/etiology/*physiopathology PMC - PMC5056893 EDAT- 2016/10/19 06:00 MHDA- 2017/04/04 06:00 PMCR- 2016/10/18 CRDT- 2016/04/27 06:00 PHST- 2016/10/19 06:00 [pubmed] PHST- 2017/04/04 06:00 [medline] PHST- 2016/04/27 06:00 [entrez] PHST- 2016/10/18 00:00 [pmc-release] AID - cmaj.150547 [pii] AID - 188e396 [pii] AID - 10.1503/cmaj.150547 [doi] PST - ppublish SO - CMAJ. 2016 Oct 18;188(15):E396. doi: 10.1503/cmaj.150547. Epub 2016 Apr 25. PMID- 19757751 OWN - NLM STAT- MEDLINE DCOM- 20090925 LR - 20090917 IS - 0028-2162 (Print) IS - 0028-2162 (Linking) VI - 153 IP - 22 DP - 2009 May 30 TI - [Raynauds phenomenon. Diagnosis and treatment]. PG - 1074-81 FAU - Hofstee, Herman M A AU - Hofstee HM AD - VU Medisch Centrum, Afd. Interne Geneeskunde, Amsterdam. hma.hofstee@vumc.nl FAU - Voskuyl, Alexandre E AU - Voskuyl AE FAU - Serné, Erik H AU - Serné EH FAU - Smulders, Yvo M AU - Smulders YM LA - dut PT - Journal Article PT - Review TT - Het fenomeen van Raynaud. Diagnostiek en behandeling. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Antibodies, Antinuclear) RN - 0 (Calcium Channel Blockers) SB - IM MH - Antibodies, Antinuclear/*blood MH - Calcium Channel Blockers/*therapeutic use MH - Humans MH - Microscopic Angioscopy MH - Prognosis MH - Raynaud Disease/*diagnosis/*drug therapy/immunology RF - 35 EDAT- 2009/09/18 06:00 MHDA- 2009/09/26 06:00 CRDT- 2009/09/18 06:00 PHST- 2009/09/18 06:00 [entrez] PHST- 2009/09/18 06:00 [pubmed] PHST- 2009/09/26 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2009 May 30;153(22):1074-81. PMID- 16529366 OWN - NLM STAT- MEDLINE DCOM- 20060517 LR - 20190907 IS - 1438-3276 (Print) IS - 1438-3276 (Linking) VI - 148 IP - 7 DP - 2006 Feb 16 TI - [Finger first white, then blue, finally red. How to recognize and treat Raynaud disease]. PG - 55-6 FAU - Ahmadi-Simab, K AU - Ahmadi-Simab K AD - Poliklinik für Rheumatologie, Universitätsklinikum Schleswig-Holstein, Lübeck. ahmadi@rheuma-zentrum.de FAU - Hellmich, B AU - Hellmich B FAU - Gross, W L AU - Gross WL LA - ger PT - Case Reports PT - News TT - Finger erst weiss, dann blau, schliesslich rot. Wie Sie das Raynaud-Syndrom erkennen und behandeln. PL - Germany TA - MMW Fortschr Med JT - MMW Fortschritte der Medizin JID - 100893959 SB - IM MH - Adult MH - Cold Temperature/adverse effects MH - Cyanosis/*etiology MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*etiology MH - Raynaud Disease/*diagnosis/etiology MH - *Skin Pigmentation EDAT- 2006/03/15 09:00 MHDA- 2006/05/18 09:00 CRDT- 2006/03/15 09:00 PHST- 2006/03/15 09:00 [pubmed] PHST- 2006/05/18 09:00 [medline] PHST- 2006/03/15 09:00 [entrez] AID - 10.1007/BF03364565 [doi] PST - ppublish SO - MMW Fortschr Med. 2006 Feb 16;148(7):55-6. doi: 10.1007/BF03364565. PMID- 26083293 OWN - NLM STAT- MEDLINE DCOM- 20160104 LR - 20150618 IS - 1538-8689 (Electronic) IS - 0360-4039 (Linking) VI - 45 IP - 7 DP - 2015 Jul TI - Understanding Raynaud phenomenon. PG - 43-5 LID - 10.1097/01.NURSE.0000461849.16529.30 [doi] FAU - Simmons, Susan AU - Simmons S AD - Susan Simmons is a family nurse practitioner at College Park Family Care Center in Overland Park, Kan. She is also a member of the Nursing2015 editorial board. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Nursing JT - Nursing JID - 7600137 MH - Adolescent MH - Female MH - Gymnastics/injuries MH - Humans MH - Nursing Diagnosis MH - Raynaud Disease/genetics/*nursing/*physiopathology MH - Severity of Illness Index MH - Wounds and Injuries/prevention & control EDAT- 2015/06/18 06:00 MHDA- 2016/01/05 06:00 CRDT- 2015/06/18 06:00 PHST- 2015/06/18 06:00 [entrez] PHST- 2015/06/18 06:00 [pubmed] PHST- 2016/01/05 06:00 [medline] AID - 00152193-201507000-00013 [pii] AID - 10.1097/01.NURSE.0000461849.16529.30 [doi] PST - ppublish SO - Nursing. 2015 Jul;45(7):43-5. doi: 10.1097/01.NURSE.0000461849.16529.30. PMID- 17938361 OWN - NLM STAT- MEDLINE DCOM- 20071113 LR - 20080317 IS - 0003-987X (Print) IS - 0003-987X (Linking) VI - 143 IP - 10 DP - 2007 Oct TI - Paraneoplastic Raynaud phenomenon with digital necrosis associated with hyperhomocysteinemia and antiphospholipid antibodies. PG - 1342-3 FAU - Carducci, Massimo AU - Carducci M FAU - Calcaterra, Roberta AU - Calcaterra R FAU - Rinaldi, Massimo AU - Rinaldi M FAU - Mussi, Anna AU - Mussi A FAU - Viola, Giuditta AU - Viola G FAU - Venturo, Irene AU - Venturo I FAU - Perracchio, Letizia AU - Perracchio L FAU - Franco, Gennaro AU - Franco G FAU - Lopez, Massimo AU - Lopez M FAU - Morrone, Aldo AU - Morrone A LA - eng PT - Case Reports PT - Letter PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Antibodies, Antiphospholipid/*blood MH - Fingers MH - Humans MH - Hyperhomocysteinemia/*complications MH - Male MH - Middle Aged MH - Necrosis MH - Paraneoplastic Syndromes/complications/*immunology/*pathology MH - Raynaud Disease/complications/*immunology/*pathology MH - Skin/*pathology MH - Toes EDAT- 2007/10/17 09:00 MHDA- 2007/11/14 09:00 CRDT- 2007/10/17 09:00 PHST- 2007/10/17 09:00 [pubmed] PHST- 2007/11/14 09:00 [medline] PHST- 2007/10/17 09:00 [entrez] AID - 143/10/1342 [pii] AID - 10.1001/archderm.143.10.1342 [doi] PST - ppublish SO - Arch Dermatol. 2007 Oct;143(10):1342-3. doi: 10.1001/archderm.143.10.1342. PMID- 19757752 OWN - NLM STAT- MEDLINE DCOM- 20090925 LR - 20131121 IS - 0028-2162 (Print) IS - 0028-2162 (Linking) VI - 153 IP - 22 DP - 2009 May 30 TI - [Diagnostic image. A breastfeeding woman with a painful, blue nipple]. PG - 1083 FAU - Velstra, Berit AU - Velstra B AD - Spaarne Ziekenhuis, afd. Heelkunde, Hoofddorp. beritvelstra@hotmail.com FAU - Bijlsma, Taco S AU - Bijlsma TS LA - dut PT - Case Reports PT - Journal Article TT - Een borstvoedende vrouw met een pijnlijke, blauwe tepel. Syndroom van Raynaud van de tepel. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Calcium Channel Blockers) RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Feeding MH - Calcium Channel Blockers/therapeutic use MH - Female MH - Humans MH - Nifedipine/therapeutic use MH - Nipples/*blood supply/pathology MH - Raynaud Disease/*complications/diagnosis/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2009/09/18 06:00 MHDA- 2009/09/26 06:00 CRDT- 2009/09/18 06:00 PHST- 2009/09/18 06:00 [entrez] PHST- 2009/09/18 06:00 [pubmed] PHST- 2009/09/26 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2009 May 30;153(22):1083. PMID- 30979757 OWN - NLM STAT- MEDLINE DCOM- 20200608 LR - 20200608 IS - 1715-5258 (Electronic) IS - 0008-350X (Print) IS - 0008-350X (Linking) VI - 65 IP - 4 DP - 2019 Apr TI - Raynaud phenomenon in children. PG - 264-265 AB - Question I have several patients, mostly girls, who are living with Raynaud phenomenon. Does this condition appear in children, and what should be the course of action?Answer Raynaud phenomenon, described in the 1860s, can present in children and even in the first decade of life. While most children will have primary Raynaud phenomenon, with no serious adverse consequences, in others it might be a sign of a pending systemic disease. Those children with a positive reaction to antinuclear antibody, specific autoantibodies associated with connective tissue disease, or nail fold capillary changes require referral to a pediatric rheumatologist and close follow-up. CI - Copyright© the College of Family Physicians of Canada. FAU - Goldman, Ran D AU - Goldman RD LA - eng PT - Journal Article PT - Review PL - Canada TA - Can Fam Physician JT - Canadian family physician Medecin de famille canadien JID - 0120300 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Antibodies, Antinuclear/blood MH - Autoimmune Diseases/complications MH - Child MH - Child, Preschool MH - Female MH - Fingers/physiopathology MH - Humans MH - Infant MH - Male MH - Raynaud Disease/complications/*diagnosis/physiopathology MH - Referral and Consultation PMC - PMC6467660 EDAT- 2019/04/14 06:00 MHDA- 2020/06/09 06:00 PMCR- 2019/04/01 CRDT- 2019/04/14 06:00 PHST- 2019/04/14 06:00 [entrez] PHST- 2019/04/14 06:00 [pubmed] PHST- 2020/06/09 06:00 [medline] PHST- 2019/04/01 00:00 [pmc-release] AID - 65/4/264 [pii] AID - 264 [pii] PST - ppublish SO - Can Fam Physician. 2019 Apr;65(4):264-265. PMID- 27433994 OWN - NLM STAT- MEDLINE DCOM- 20170526 LR - 20170526 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 22 IP - 6 DP - 2016 Sep TI - Raynaud Phenomenon With Severe Ulcers Associated With Cryoglobulins in an HIV+ but HCV Negative Patient. PG - 335-7 LID - 10.1097/RHU.0000000000000431 [doi] FAU - Santarelli, Ignacio M AU - Santarelli IM AD - Department of Medicine, Hospital de Clínicas "José de San Martín", University of Buenos Aires, Buenos Aires, Argentina, santarelli.ignacio@gmail.com Department of Medicine, Hospital de Clínicas "José de San Martín", University of Buenos Aires, Buenos Aires, Argentina. FAU - Manzella, Diego J AU - Manzella DJ FAU - Costa, Diego AU - Costa D FAU - Pisarevsky, Ana A AU - Pisarevsky AA FAU - Melero, Marcelo J AU - Melero MJ LA - eng PT - Case Reports PT - Letter PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Anti-HIV Agents) RN - 0 (Anticoagulants) RN - 0 (Vasodilator Agents) SB - IM MH - Adult MH - Anti-HIV Agents/*administration & dosage MH - Anticoagulants/*administration & dosage MH - Antiretroviral Therapy, Highly Active/methods MH - CD4 Lymphocyte Count/methods MH - *Cryoglobulinemia/blood/complications/diagnosis/therapy MH - *HIV Infections/blood/complications/diagnosis/drug therapy MH - Humans MH - Male MH - Plasmapheresis/*methods MH - *Raynaud Disease/diagnosis/etiology/physiopathology/therapy MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage EDAT- 2016/07/20 06:00 MHDA- 2017/05/27 06:00 CRDT- 2016/07/20 06:00 PHST- 2016/07/20 06:00 [entrez] PHST- 2016/07/20 06:00 [pubmed] PHST- 2017/05/27 06:00 [medline] AID - 10.1097/RHU.0000000000000431 [doi] PST - ppublish SO - J Clin Rheumatol. 2016 Sep;22(6):335-7. doi: 10.1097/RHU.0000000000000431. PMID- 25022088 OWN - NLM STAT- MEDLINE DCOM- 20140904 LR - 20190907 IS - 1438-3276 (Print) IS - 1438-3276 (Linking) VI - 156 IP - 11 DP - 2014 Jun 12 TI - [Writer's cramp amd Raynaud phenomenon. How do they go together?]. PG - 32 FAU - Limmroth, Volker AU - Limmroth V LA - ger PT - News TT - Schreibkrampf und Raynaud-Phänomen. Wie wirken die zusammen? PL - Germany TA - MMW Fortschr Med JT - MMW Fortschritte der Medizin JID - 100893959 SB - IM MH - Adult MH - Autonomic Nervous System/physiopathology MH - Dystonic Disorders/*complications/physiopathology MH - Extrapyramidal Tracts/physiopathology MH - Humans MH - Male MH - Peripheral Nerves/physiopathology MH - Raynaud Disease/*complications/physiopathology EDAT- 2014/07/16 06:00 MHDA- 2014/09/05 06:00 CRDT- 2014/07/16 06:00 PHST- 2014/07/16 06:00 [entrez] PHST- 2014/07/16 06:00 [pubmed] PHST- 2014/09/05 06:00 [medline] AID - 10.1007/s15006-014-3154-z [doi] PST - ppublish SO - MMW Fortschr Med. 2014 Jun 12;156(11):32. doi: 10.1007/s15006-014-3154-z. PMID- 35073635 OWN - NLM STAT- MEDLINE DCOM- 20220223 LR - 20230930 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 8S DP - 2021 Dec 1 TI - Kimura Disease Presenting With New Raynaud Phenomenon: A Common Symptom Caused by an Uncommon Disease. PG - S622-S624 LID - 10.1097/RHU.0000000000001704 [doi] FAU - Ahrari, Aida AU - Ahrari A AD - From the Department of Medicine, University of Toronto, Toronto, Ontario. FAU - Ahrari, Azin AU - Ahrari A AD - Division of Rheumatology. FAU - Chen, Luke Y C AU - Chen LYC AD - Division of Hematology. FAU - Wolber, Robert AU - Wolber R AD - Division of Anatomical Pathology. FAU - Basseri, Hamed AU - Basseri H AD - Department of Radiology, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Dehghan, Natasha AU - Dehghan N AD - Division of Rheumatology. FAU - Hemmati, Iman AU - Hemmati I AD - Division of Rheumatology. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Humans MH - *Kimura Disease MH - *Raynaud Disease/diagnosis/etiology COIS- The authors declare no conflict of interest. EDAT- 2022/01/26 06:00 MHDA- 2022/02/24 06:00 CRDT- 2022/01/25 01:01 PHST- 2022/01/25 01:01 [entrez] PHST- 2022/01/26 06:00 [pubmed] PHST- 2022/02/24 06:00 [medline] AID - 00124743-202112003-00100 [pii] AID - 10.1097/RHU.0000000000001704 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Dec 1;27(8S):S622-S624. doi: 10.1097/RHU.0000000000001704. PMID- 23347192 OWN - NLM STAT- MEDLINE DCOM- 20140219 LR - 20130514 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 43 IP - 5 DP - 2013 May TI - Treatment of Raynaud phenomenon in systemic sclerosis. PG - 476-83 LID - 10.1111/imj.12082 [doi] AB - Systemic sclerosis is a connective tissue disease characterised by microvascular injury and excessive fibrosis of the skin and internal organs. Most patients with this condition experience Raynaud phenomenon, usually as the earliest manifestation of disease. In addition to pain and functional impairment, Raynaud phenomenon can produce tissue ischaemia resulting in digital ulceration and gangrene. Current treatments have been only moderately successful in reducing the frequency and severity of Raynaud phenomenon in patients with systemic sclerosis. This review will address treatments available for Raynaud phenomenon in systemic sclerosis. CI - © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians. FAU - Sinnathurai, P AU - Sinnathurai P AD - Department of Rheumatology, Royal North Shore Hospital, Sydney, New South Wales, Australia. FAU - Schrieber, L AU - Schrieber L LA - eng PT - Journal Article PT - Review PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - 0 (Calcium Channel Blockers) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) SB - IM MH - Animals MH - Calcium Channel Blockers/administration & dosage MH - Disease Management MH - Drug Therapy, Combination MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/administration & dosage MH - Phosphodiesterase Inhibitors/administration & dosage MH - Raynaud Disease/diagnosis/*epidemiology/*therapy MH - Scleroderma, Systemic/diagnosis/*epidemiology/*therapy MH - Treatment Outcome EDAT- 2013/01/26 06:00 MHDA- 2014/02/20 06:00 CRDT- 2013/01/26 06:00 PHST- 2012/09/03 00:00 [received] PHST- 2012/12/26 00:00 [accepted] PHST- 2013/01/26 06:00 [entrez] PHST- 2013/01/26 06:00 [pubmed] PHST- 2014/02/20 06:00 [medline] AID - 10.1111/imj.12082 [doi] PST - ppublish SO - Intern Med J. 2013 May;43(5):476-83. doi: 10.1111/imj.12082. PMID- 37498616 OWN - NLM STAT- MEDLINE DCOM- 20230825 LR - 20230825 IS - 1538-3598 (Electronic) IS - 0098-7484 (Linking) VI - 330 IP - 7 DP - 2023 Aug 15 TI - Patient With Pulmonary Symptoms, Dysphagia, and Raynaud Disease. PG - 658-659 LID - 10.1001/jama.2023.13766 [doi] FAU - Ghias, Aisha AU - Ghias A AD - Department of Internal Medicine, NYU Langone Health, Mineola, New York. FAU - Chawla, Shalinee AU - Chawla S AD - Division of Pulmonary, Critical Care, and Sleep Medicine, NYU Langone Health, Mineola, New York. FAU - Agarwala, Priya AU - Agarwala P AD - NYU Langone Health, Mineola, New York. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - JAMA JT - JAMA JID - 7501160 SB - IM MH - Humans MH - Cough/etiology MH - *Deglutition Disorders/etiology MH - Lung MH - *Raynaud Disease/diagnosis/etiology MH - *Lung Diseases/etiology OAB - A nonsmoking patient with gastroesophageal reflux disease and Raynaud disease had 4 weeks of dysphagia and a 4.54-kg weight loss over 3 months, cough productive of yellow sputum, and dyspnea on exertion. White blood cell count and creatine kinase and aldolase levels were elevated; antinuclear antibody assay findings were positive; and chest CT showed bibasilar pulmonary consolidations and ground glass opacities. What is the diagnosis and what would you do next? OABL- eng EDAT- 2023/07/27 13:10 MHDA- 2023/08/16 06:43 CRDT- 2023/07/27 11:34 PHST- 2023/08/16 06:43 [medline] PHST- 2023/07/27 13:10 [pubmed] PHST- 2023/07/27 11:34 [entrez] AID - 2807957 [pii] AID - 10.1001/jama.2023.13766 [doi] PST - ppublish SO - JAMA. 2023 Aug 15;330(7):658-659. doi: 10.1001/jama.2023.13766. PMID- 29093119 OWN - NLM STAT- MEDLINE DCOM- 20180716 LR - 20180716 IS - 1526-3347 (Electronic) IS - 0191-9601 (Linking) VI - 38 IP - 11 DP - 2017 Nov TI - Hand and Foot Color Change: Diagnosis and Management. PG - 511-519 LID - 10.1542/pir.2016-0234 [doi] FAU - Fleck, Dustin E AU - Fleck DE AD - Division of Pediatric Rheumatology, Department of Pediatrics, University of Michigan, Ann Arbor, MI. FAU - Hoeltzel, Mark F AU - Hoeltzel MF AD - Division of Pediatric Rheumatology, Department of Pediatrics, University of Michigan, Ann Arbor, MI. LA - eng PT - Journal Article PT - Review PL - United States TA - Pediatr Rev JT - Pediatrics in review JID - 8103046 SB - IM MH - Child MH - *Color MH - *Cyanosis/diagnosis/etiology/therapy MH - Diagnosis, Differential MH - *Erythromelalgia/diagnosis/etiology/therapy MH - *Foot Diseases/diagnosis/etiology/therapy MH - *Hand MH - Humans MH - *Raynaud Disease/diagnosis/etiology/therapy EDAT- 2017/11/03 06:00 MHDA- 2018/07/17 06:00 CRDT- 2017/11/03 06:00 PHST- 2017/11/03 06:00 [entrez] PHST- 2017/11/03 06:00 [pubmed] PHST- 2018/07/17 06:00 [medline] AID - 38/11/511 [pii] AID - 10.1542/pir.2016-0234 [doi] PST - ppublish SO - Pediatr Rev. 2017 Nov;38(11):511-519. doi: 10.1542/pir.2016-0234. PMID- 19248132 OWN - NLM STAT- MEDLINE DCOM- 20090526 LR - 20090309 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 61 IP - 3 DP - 2009 Mar 15 TI - A growing need for capillaroscopy in rheumatology. PG - 405-10 LID - 10.1002/art.24274 [doi] FAU - De Angelis, Rossella AU - De Angelis R AD - Polytechnic University of the Marche, Ancona, Italy. rossella.deangelis@sanita.marche.it FAU - Grassi, Walter AU - Grassi W FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article PT - Review PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Diagnosis, Differential MH - Forecasting MH - Humans MH - *Microscopic Angioscopy/trends MH - Microscopy, Video MH - Raynaud Disease/*diagnosis/physiopathology/therapy MH - Scleroderma, Systemic/*diagnosis/physiopathology/therapy RF - 54 EDAT- 2009/02/28 09:00 MHDA- 2009/05/27 09:00 CRDT- 2009/02/28 09:00 PHST- 2009/02/28 09:00 [entrez] PHST- 2009/02/28 09:00 [pubmed] PHST- 2009/05/27 09:00 [medline] AID - 10.1002/art.24274 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Mar 15;61(3):405-10. doi: 10.1002/art.24274. PMID- 17576963 OWN - NLM STAT- MEDLINE DCOM- 20070726 LR - 20080317 IS - 0003-987X (Print) IS - 0003-987X (Linking) VI - 143 IP - 6 DP - 2007 Jun TI - Hypereosinophilic syndrome with peripheral circulatory insufficiency and cutaneous microthrombi. PG - 812-3 FAU - Hamada, Takahiro AU - Hamada T FAU - Kimura, Yoko AU - Kimura Y FAU - Hayashi, Shinsuke AU - Hayashi S FAU - Nakama, Takekuni AU - Nakama T FAU - Hashimoto, Takashi AU - Hashimoto T LA - eng PT - Case Reports PT - Letter PT - Research Support, Non-U.S. Gov't PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 SB - IM MH - Adult MH - Diagnosis, Differential MH - Humans MH - Hypereosinophilic Syndrome/complications/*diagnosis/pathology MH - Male MH - Raynaud Disease/complications/*diagnosis/pathology MH - Thrombosis/complications/*diagnosis/pathology EDAT- 2007/06/20 09:00 MHDA- 2007/07/27 09:00 CRDT- 2007/06/20 09:00 PHST- 2007/06/20 09:00 [pubmed] PHST- 2007/07/27 09:00 [medline] PHST- 2007/06/20 09:00 [entrez] AID - 143/6/812 [pii] AID - 10.1001/archderm.143.6.812 [doi] PST - ppublish SO - Arch Dermatol. 2007 Jun;143(6):812-3. doi: 10.1001/archderm.143.6.812. PMID- 22669698 OWN - NLM STAT- MEDLINE DCOM- 20120810 LR - 20120606 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 137 IP - 24 DP - 2012 Jun TI - [74-year-old woman with white fingers]. PG - 1301-2 LID - 10.1055/s-0031-1299044 [doi] FAU - Michalski, T AU - Michalski T AD - Universitätsklinikum Salzburg, Österreich. t.michalski@salk.at LA - ger PT - Case Reports PT - Journal Article TT - 74-jährige Patientin mit livider Verfärbung der Finger. DEP - 20120605 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Electrolytes) SB - IM MH - Aged MH - Diagnosis, Differential MH - Electrolytes/therapeutic use MH - Enteritis/*complications MH - Female MH - Fingers/*pathology MH - Humans MH - Raynaud Disease/*diagnosis/*etiology/therapy MH - Renal Insufficiency/complications MH - Treatment Outcome EDAT- 2012/06/07 06:00 MHDA- 2012/08/11 06:00 CRDT- 2012/06/07 06:00 PHST- 2012/06/07 06:00 [entrez] PHST- 2012/06/07 06:00 [pubmed] PHST- 2012/08/11 06:00 [medline] AID - 10.1055/s-0031-1299044 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2012 Jun;137(24):1301-2. doi: 10.1055/s-0031-1299044. Epub 2012 Jun 5. PMID- 31977646 OWN - NLM STAT- MEDLINE DCOM- 20220225 LR - 20230930 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 8S DP - 2021 Dec 1 TI - Development of Raynaud Phenomenon Following Use of Medical Cannabis. PG - S430-S431 LID - 10.1097/RHU.0000000000001312 [doi] FAU - Shimol, Jennifer Ben AU - Shimol JB AD - Edith Wolfson Medical Center, Holon, Israel Jennifer.benshimol@gmail.com. FAU - Sheinberg, Oded AU - Sheinberg O LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Medical Marijuana) SB - IM MH - Humans MH - *Medical Marijuana/adverse effects MH - *Raynaud Disease/chemically induced/diagnosis COIS- The authors declare no conflict of interest. EDAT- 2020/01/25 06:00 MHDA- 2022/02/26 06:00 CRDT- 2020/01/25 06:00 PHST- 2020/01/25 06:00 [pubmed] PHST- 2022/02/26 06:00 [medline] PHST- 2020/01/25 06:00 [entrez] AID - 00124743-202112003-00037 [pii] AID - 10.1097/RHU.0000000000001312 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Dec 1;27(8S):S430-S431. doi: 10.1097/RHU.0000000000001312. PMID- 22384763 OWN - NLM STAT- MEDLINE DCOM- 20120405 LR - 20190923 IS - 1547-1896 (Print) IS - 0893-7400 (Linking) VI - 25 IP - 1 DP - 2012 Jan TI - Raynaud phenomenon. PG - 73 FAU - Bunde, Cynthia AU - Bunde C AD - Department of Physician Assistant Studies, Idaho State University, Pocatello, USA. FAU - Howlett, Bernadette AU - Howlett B FAU - Ogami, Nicholas AU - Ogami N FAU - Hall, Rebecca AU - Hall R LA - eng PT - Case Reports PT - Journal Article PL - United States TA - JAAPA JT - JAAPA : official journal of the American Academy of Physician Assistants JID - 9513102 MH - Adult MH - Breast Diseases/*diagnosis/therapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Raynaud Disease/*diagnosis/therapy EDAT- 2012/03/06 06:00 MHDA- 2012/04/06 06:00 CRDT- 2012/03/06 06:00 PHST- 2012/03/06 06:00 [entrez] PHST- 2012/03/06 06:00 [pubmed] PHST- 2012/04/06 06:00 [medline] AID - 10.1097/01720610-201201000-00018 [doi] PST - ppublish SO - JAAPA. 2012 Jan;25(1):73. doi: 10.1097/01720610-201201000-00018. PMID- 22615460 OWN - NLM STAT- MEDLINE DCOM- 20120925 LR - 20260518 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 71 IP - 8 DP - 2012 Aug TI - Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. PG - 1355-60 LID - 10.1136/annrheumdis-2011-200742 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a rare disease requiring multicentre collaboration to reveal comprehensive details of disease-related causes for morbidity and mortality. METHODS: The European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) group initiated a database to prospectively gather key data of patients with SSc using a minimal essential dataset that was reorganised in 2008 introducing new items. Baseline visit data of patients who were registered between 2004 and 2011 were analysed using descriptive statistics. RESULTS: In June 2011, 7655 patients (2838 with diffuse cutaneous (dc) and 4481 with limited cutaneous (lc) SSc who fulfilled the American College of Rheumatology diagnostic criteria had been registered in 174 centres, mainly European. The most prominent hallmarks of disease were Raynaud's phenomenon (96.3%), antinuclear antibodies (93.4%) and a typical capillaroscopic pattern (90.9%). Scleroderma was more common on fingers and hands than on any other part of the skin. Proton pump inhibitors (65.2%), calcium channel blockers (52.7%), and corticosteroids (45.3%) were most often prescribed. Among the immunosuppressant agents, cyclophosphamide was used more often in dcSSc than in lcSSc. CONCLUSIONS: The EUSTAR database provides an abundance of information on the true clinical face of SSc that will be helpful in improving the classification of SSc and its subsets and for developing more specific therapeutic recommendations. FAU - Meier, Florian M P AU - Meier FM AD - Department of Internal Medicine and Rheumatology, Justus-Liebig-University Gießen Kerckhoff-Klinik, Bad Nauheim, Germany. FAU - Frommer, Klaus W AU - Frommer KW FAU - Dinser, Robert AU - Dinser R FAU - Walker, Ulrich A AU - Walker UA FAU - Czirjak, Laszlo AU - Czirjak L FAU - Denton, Christopher P AU - Denton CP FAU - Allanore, Yannick AU - Allanore Y FAU - Distler, Oliver AU - Distler O FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Valentini, Gabriele AU - Valentini G FAU - Müller-Ladner, Ulf AU - Müller-Ladner U CN - EUSTAR Co-authors LA - eng GR - 18475/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20120521 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Antibodies, Antinuclear) RN - 0 (Calcium Channel Blockers) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Proton Pump Inhibitors) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Antibodies, Antinuclear/blood MH - Calcium Channel Blockers/therapeutic use MH - Cohort Studies MH - Cyclophosphamide/therapeutic use MH - *Databases, Factual MH - Europe MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Prospective Studies MH - Proton Pump Inhibitors/therapeutic use MH - Raynaud Disease/complications/diagnosis MH - Scleroderma, Diffuse/*diagnosis/drug therapy/physiopathology MH - Scleroderma, Limited/*diagnosis/drug therapy/physiopathology FIR - Matucci-Cerinic, Marco IR - Matucci-Cerinic M FIR - Guiducci, Serena IR - Guiducci S FIR - Tyndall, Alan IR - Tyndall A FIR - Lapadula, Giovanni IR - Lapadula G FIR - Iannone, Florenzo IR - Iannone F FIR - Becvar, Radim IR - Becvar R FIR - Sierakowsky, Stanislaw IR - Sierakowsky S FIR - Kowal-Bielecka, Otylia IR - Kowal-Bielecka O FIR - Cutolo, Maurizio IR - Cutolo M FIR - Sulli, Alberto IR - Sulli A FIR - Rednic, Simona IR - Rednic S FIR - Nicoara, Ileana IR - Nicoara I FIR - Pamfil, Cristina IR - Pamfil C FIR - Kahan, André IR - Kahan A FIR - Avouac, Jerome IR - Avouac J FIR - Vlachoyiannopoulos, P IR - Vlachoyiannopoulos P FIR - Montecucco, C IR - Montecucco C FIR - Caporali, Roberto IR - Caporali R FIR - Štork, Jirí IR - Štork J FIR - Inanc, Murat IR - Inanc M FIR - Carreira, Patricia E IR - Carreira PE FIR - Novak, Srdan IR - Novak S FIR - Varju, Cecilia IR - Varju C FIR - Chizzolini, Carlo IR - Chizzolini C FIR - Kucharz, Eugene J IR - Kucharz EJ FIR - Kotulska, Anna IR - Kotulska A FIR - Kopec-Medrek, Magdalena IR - Kopec-Medrek M FIR - Widuchowska, Malgorzata IR - Widuchowska M FIR - Cozzi, Franco IR - Cozzi F FIR - Rozman, Blaz IR - Rozman B FIR - Mallia, Carmel IR - Mallia C FIR - Coleiro, Bernard IR - Coleiro B FIR - Gabrielli, Armando IR - Gabrielli A FIR - Farge-Bancel, Dominique IR - Farge-Bancel D FIR - Marjanovic, Zora IR - Marjanovic Z FIR - Durant, Cecile IR - Durant C FIR - Airò, Paolo IR - Airò P FIR - Wollheim, Frank IR - Wollheim F FIR - Hesselstrand, Roger IR - Hesselstrand R FIR - Scheja, Agneta IR - Scheja A FIR - Martinovic, Duska IR - Martinovic D FIR - Radic, Mislav IR - Radic M FIR - Gurmann, Alexandra Balbir IR - Gurmann AB FIR - Braun-Moscovici, Yolanda IR - Braun-Moscovici Y FIR - Trotta, F IR - Trotta F FIR - La Corte, R IR - La Corte R FIR - Lo Monaco, Andrea IR - Lo Monaco A FIR - Hunzelmann, Nicolas IR - Hunzelmann N FIR - Krieg, Thomas IR - Krieg T FIR - Pellerito, Raffaele IR - Pellerito R FIR - Bambara, Lisa Maria IR - Bambara LM FIR - Caramaschi, Paola IR - Caramaschi P FIR - Pieropan, Sara IR - Pieropan S FIR - Morovic-Vergles, Jadranka IR - Morovic-Vergles J FIR - Black, Carol IR - Black C FIR - Damjanov, Nemanja IR - Damjanov N FIR - Kötter, Ina IR - Kötter I FIR - Ortiz Santamaria, Vera IR - Ortiz Santamaria V FIR - Heitmann, Stefan IR - Heitmann S FIR - Krasowska, Dorota IR - Krasowska D FIR - Seidel, Matthias IR - Seidel M FIR - Hasler, Paul IR - Hasler P FIR - Burkhardt, Harald IR - Burkhardt H FIR - Himsel, Andrea IR - Himsel A FIR - Bajocchi, Gianluigi IR - Bajocchi G FIR - Da Silva, José Antonio Pereira IR - Da Silva JA FIR - Henriques, Maria João Salvador IR - Henriques MJ FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - Stankovic, Aleksandra IR - Stankovic A FIR - Marasini, Bianca IR - Marasini B FIR - Belloli, Laura IR - Belloli L FIR - Tikly, Mohammed IR - Tikly M FIR - Nasanov, E L IR - Nasanov EL FIR - Denisov, Lev N IR - Denisov LN FIR - Ananieva, Lidia P IR - Ananieva LP FIR - Guseva, N G IR - Guseva NG FIR - Nevskaja, Tatiana IR - Nevskaja T FIR - Tarner, Ingo H IR - Tarner IH FIR - Scorza, Raffaella IR - Scorza R FIR - Indiveri, Francesco IR - Indiveri F FIR - Engelhart, Merete IR - Engelhart M FIR - Strauss, Gitte IR - Strauss G FIR - Nielsen, Henrik IR - Nielsen H FIR - Damgaard, Kirsten IR - Damgaard K FIR - Szekanecz, Zoltan IR - Szekanecz Z FIR - Szücs, Gabriella IR - Szücs G FIR - Szamosi, Szilvia IR - Szamosi S FIR - de la Puente, Carlos IR - de la Puente C FIR - García de la Pena Lefebvre, Paloma IR - García de la Pena Lefebvre P FIR - Midtvedt, Øyvind IR - Midtvedt Ø FIR - Garen, Torhild IR - Garen T FIR - Hachulla, Eric IR - Hachulla E FIR - Launay, David IR - Launay D FIR - Valesini, Guido IR - Valesini G FIR - Riccieri, Valeria IR - Riccieri V FIR - Ionescu, Ruxandra Maria IR - Ionescu RM FIR - Opris, Daniela IR - Opris D FIR - Wigley, Fredrick M IR - Wigley FM FIR - Stoica, Viktor IR - Stoica V FIR - Mihai, Carmen M IR - Mihai CM FIR - Cornateanu, Roxana S IR - Cornateanu RS FIR - Udrea, Gabriela IR - Udrea G FIR - Ciurtin, Coziana IR - Ciurtin C FIR - Ionitescu, Razvan IR - Ionitescu R FIR - Bojinca, Mihai IR - Bojinca M FIR - Gherghe, Ana M IR - Gherghe AM FIR - Sunderkötter, Cord IR - Sunderkötter C FIR - Kuhn, Annegret IR - Kuhn A FIR - Herrgott, Ilka IR - Herrgott I FIR - Saritas, Senay IR - Saritas S FIR - Sandorfi, Nora IR - Sandorfi N FIR - Schett, Georg IR - Schett G FIR - Distler, Jörg IR - Distler J FIR - Beyer, Christian IR - Beyer C FIR - Meroni, Pierluigi IR - Meroni P FIR - Zeni, Silvana IR - Zeni S FIR - Guillevin, Loïc IR - Guillevin L FIR - Mouthon, Luc IR - Mouthon L FIR - De Keyser, Filip IR - De Keyser F FIR - Smith, Vanessa IR - Smith V FIR - Cantatore, Francesco P IR - Cantatore FP FIR - Corrado, Ada IR - Corrado A FIR - Ullman, Susanne IR - Ullman S FIR - Iversen, Line IR - Iversen L FIR - von Mühlen, Carlos Alberto IR - von Mühlen CA FIR - Bohn, Jussara M IR - Bohn JM FIR - Lonzetti, Lilian Scussel IR - Lonzetti LS FIR - Pozzi, Maria Rosa IR - Pozzi MR FIR - Eyerich, Kilian IR - Eyerich K FIR - Hein, Rüdiger IR - Hein R FIR - Knott, Elisabeth IR - Knott E FIR - Szechinski, Jacek IR - Szechinski J FIR - Wiland, Piotr IR - Wiland P FIR - Szmyrka-Kaczmarek, Magdalena IR - Szmyrka-Kaczmarek M FIR - Sokolik, Renata IR - Sokolik R FIR - Morgiel, Ewa IR - Morgiel E FIR - Houssiau, Frédéric A IR - Houssiau FA FIR - Vanthuyne, Marie IR - Vanthuyne M FIR - Román-Ivorra, Jose A IR - Román-Ivorra JA FIR - Alegre-Sancho, Juan Jose IR - Alegre-Sancho JJ FIR - Krummel-Lorenz, Brigitte IR - Krummel-Lorenz B FIR - Aringer, Martin IR - Aringer M FIR - Meurer, Michael IR - Meurer M FIR - Westhovens, Rene IR - Westhovens R FIR - De Langhe, Ellen IR - De Langhe E FIR - Anic, Branimir IR - Anic B FIR - Baresic, Marko IR - Baresic M FIR - Mayer, Miroslav IR - Mayer M FIR - Üprus, Maria IR - Üprus M FIR - Otsa, Kati IR - Otsa K FIR - Yavuz, Sule IR - Yavuz S FIR - Granel, Brigitte IR - Granel B FIR - Radominski, Sebastião Cezar IR - Radominski SC FIR - Müller, Carolina de Souza IR - Müller Cde S FIR - Azevedo, Valderílio Feijó IR - Azevedo VF FIR - Jimenez, Sergio IR - Jimenez S FIR - Busquets, Joanna IR - Busquets J FIR - Popa, Sergei IR - Popa S FIR - Agachi, Svetlana IR - Agachi S FIR - Groppa, Liliana IR - Groppa L FIR - Chiaburu, Lealea IR - Chiaburu L FIR - Russu, Eugen IR - Russu E FIR - Zenone, Thierry IR - Zenone T FIR - Pileckyte, Margarita IR - Pileckyte M FIR - Stebbings, Simon IR - Stebbings S FIR - Highton, John IR - Highton J FIR - Mathieu, Alessandro IR - Mathieu A FIR - Vacca, Alessandra IR - Vacca A FIR - Sampaio-Barros, Percival D IR - Sampaio-Barros PD FIR - Yoshinari, Natalino H IR - Yoshinari NH FIR - Marangoni, Roberta G IR - Marangoni RG FIR - Martin, Patrícia IR - Martin P FIR - Fuocco, Luiza IR - Fuocco L FIR - Stamp, Lisa IR - Stamp L FIR - Chapman, Peter IR - Chapman P FIR - O'Donnell, John IR - O'Donnell J FIR - Solanki, Kamal IR - Solanki K FIR - Doube, Alan IR - Doube A FIR - Schollum, Joanne Stephanie IR - Schollum JS FIR - Veale, Douglas IR - Veale D FIR - O'Rourke, Marie IR - O'Rourke M FIR - Loyo, Esthela IR - Loyo E FIR - Then, Jossiell IR - Then J FIR - Toloza, Sergio IR - Toloza S FIR - Li, Mengtao IR - Li M FIR - Mohamed, Walid Ahmed Abdel Atty IR - Mohamed WA FIR - Mohamed, Wessam Harby IR - Mohamed WH FIR - Olas, Jacek IR - Olas J FIR - Salsano, Felice IR - Salsano F FIR - Pisarri, Simonetta IR - Pisarri S FIR - Rosato, Edoardo IR - Rosato E FIR - Oksel, Fahrettin IR - Oksel F FIR - Yargucu, Figen IR - Yargucu F FIR - Tanaseanu, Cristina-Mihaela IR - Tanaseanu CM FIR - Popescu, Monica IR - Popescu M FIR - Dumitrascu, Alina IR - Dumitrascu A FIR - Tiglea, Isabela IR - Tiglea I FIR - Foti, Rosario IR - Foti R FIR - Chirieac, Rodica IR - Chirieac R FIR - Ancuta, Codrina IR - Ancuta C FIR - Furst, Daniel E IR - Furst DE FIR - Villiger, Peter IR - Villiger P FIR - Adler, Sabine IR - Adler S FIR - van Laar, Jacob IR - van Laar J FIR - Wiesik-Szewczyk, Ewa IR - Wiesik-Szewczyk E FIR - Olesinska, Marzena IR - Olesinska M FIR - Kayser, Cristiane IR - Kayser C FIR - C, Andrade Luis Eduardo IR - C AL FIR - Fathi, Nihal IR - Fathi N FIR - García de la Peña Lefebvre, Paloma IR - García de la Peña Lefebvre P FIR - Rodriguez Rubio, Silvia IR - Rodriguez Rubio S FIR - Valero Exposito, Marta IR - Valero Exposito M FIR - Carpentier, Patrick IR - Carpentier P FIR - Imbert, Bernard IR - Imbert B FIR - Francès, Camille IR - Francès C FIR - Senet, Patricia IR - Senet P FIR - Sibilia, Jean IR - Sibilia J FIR - Chatelus, Emmanuel IR - Chatelus E FIR - Gottenberg, Jacques Eric IR - Gottenberg JE FIR - Chifflot, Hélène IR - Chifflot H FIR - Litinsky, Ira IR - Litinsky I FIR - Senécal, Jean Luc IR - Senécal JL FIR - Koenig, Martial IR - Koenig M FIR - Joyal, France IR - Joyal F FIR - Tamara, Grodzicky IR - Tamara G FIR - Emery, Paul IR - Emery P FIR - Buch, Maya IR - Buch M FIR - Del Galdo, Francesco IR - Del Galdo F FIR - Bissel, Lesley Anne IR - Bissel LA FIR - Venalis, Algirdas IR - Venalis A FIR - Butrimiene, Irena IR - Butrimiene I FIR - Venalis, Paulius IR - Venalis P FIR - Rugiene, Rita IR - Rugiene R FIR - Karpec, Diana IR - Karpec D FIR - Saketkoo, Lesley Ann IR - Saketkoo LA FIR - Espinoza, Luis R IR - Espinoza LR FIR - Kerzberg, Eduardo IR - Kerzberg E FIR - Montoya, Fabiana IR - Montoya F FIR - Cosentino, Vanesa IR - Cosentino V FIR - Bianchi, Washington IR - Bianchi W FIR - Carneiro, Sueli IR - Carneiro S FIR - Maretti, Giselle Baptista IR - Maretti GB FIR - Bianchi, Dante Valdetaro IR - Bianchi DV FIR - Castellví, Ivan IR - Castellví I FIR - Vettori, Serena IR - Vettori S FIR - Cuomo, Giovanna IR - Cuomo G FIR - Deuschler, Katrin IR - Deuschler K FIR - Becker, Mike IR - Becker M EDAT- 2012/05/23 06:00 MHDA- 2012/09/26 06:00 CRDT- 2012/05/23 06:00 PHST- 2012/05/23 06:00 [entrez] PHST- 2012/05/23 06:00 [pubmed] PHST- 2012/09/26 06:00 [medline] AID - S0003-4967(24)18737-7 [pii] AID - 10.1136/annrheumdis-2011-200742 [doi] PST - ppublish SO - Ann Rheum Dis. 2012 Aug;71(8):1355-60. doi: 10.1136/annrheumdis-2011-200742. Epub 2012 May 21. PMID- 23288940 OWN - NLM STAT- MEDLINE DCOM- 20131122 LR - 20130104 IS - 1939-2869 (Electronic) IS - 0891-1150 (Linking) VI - 80 IP - 1 DP - 2013 Jan TI - Q: When do Raynaud symptoms merit a workup for autoimmune rheumatic disease? PG - 22-5 LID - 10.3949/ccjm.80a.12082 [doi] FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH 44195, USA. chattes@ccf.org LA - eng PT - Journal Article PL - United States TA - Cleve Clin J Med JT - Cleveland Clinic journal of medicine JID - 8703441 SB - IM MH - Algorithms MH - Autoimmune Diseases/complications/*diagnosis MH - Humans MH - Raynaud Disease/*diagnosis/etiology MH - Rheumatic Diseases/complications/*diagnosis EDAT- 2013/01/05 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/01/05 06:00 PHST- 2013/01/05 06:00 [entrez] PHST- 2013/01/05 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - 80/1/22 [pii] AID - 10.3949/ccjm.80a.12082 [doi] PST - ppublish SO - Cleve Clin J Med. 2013 Jan;80(1):22-5. doi: 10.3949/ccjm.80a.12082. PMID- 35512871 OWN - NLM STAT- MEDLINE DCOM- 20220509 LR - 20220623 IS - 1942-5546 (Electronic) IS - 0025-6196 (Linking) VI - 97 IP - 5 DP - 2022 May TI - 72-Year-Old Man With Puffy Fingers and Raynaud Phenomenon. PG - 1014-1019 LID - S0025-6196(21)00931-9 [pii] LID - 10.1016/j.mayocp.2021.10.029 [doi] FAU - Medina-Bielski, Sara AU - Medina-Bielski S AD - Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN. FAU - Mohan, Sneha AU - Mohan S AD - Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN. FAU - Makol, Ashima AU - Makol A AD - Advisor to residents and Consultant in Rheumatology, Mayo Clinic, Rochester, MN. Electronic address: makol.ashima@mayo.edu. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mayo Clin Proc JT - Mayo Clinic proceedings JID - 0405543 SB - IM MH - Fingers MH - Humans MH - Male MH - *Raynaud Disease/complications/diagnosis EDAT- 2022/05/06 06:00 MHDA- 2022/05/10 06:00 CRDT- 2022/05/05 21:03 PHST- 2021/04/30 00:00 [received] PHST- 2021/10/27 00:00 [revised] PHST- 2021/10/29 00:00 [accepted] PHST- 2022/05/05 21:03 [entrez] PHST- 2022/05/06 06:00 [pubmed] PHST- 2022/05/10 06:00 [medline] AID - S0025-6196(21)00931-9 [pii] AID - 10.1016/j.mayocp.2021.10.029 [doi] PST - ppublish SO - Mayo Clin Proc. 2022 May;97(5):1014-1019. doi: 10.1016/j.mayocp.2021.10.029. PMID- 33337814 OWN - NLM STAT- MEDLINE DCOM- 20220225 LR - 20230930 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 8S DP - 2021 Dec 1 TI - Hand Osteomyelitis in Patients With Secondary Raynaud Phenomenon. PG - S342-S345 LID - 10.1097/RHU.0000000000001621 [doi] FAU - Haque, Ashraful AU - Haque A FAU - Wyman, Matthew AU - Wyman M FAU - Dargan, Dallan AU - Dargan D FAU - Hughes, Michael AU - Hughes M FAU - Musson, Rachel AU - Musson R FAU - Caddick, Jennifer AU - Caddick J FAU - Giblin, Victoria AU - Giblin V LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Hand MH - Humans MH - *Osteomyelitis/complications/etiology MH - *Raynaud Disease/complications/etiology COIS- The authors declare no conflict of interest. EDAT- 2020/12/19 06:00 MHDA- 2022/02/26 06:00 CRDT- 2020/12/18 15:18 PHST- 2020/12/19 06:00 [pubmed] PHST- 2022/02/26 06:00 [medline] PHST- 2020/12/18 15:18 [entrez] AID - 00124743-202112003-00006 [pii] AID - 10.1097/RHU.0000000000001621 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Dec 1;27(8S):S342-S345. doi: 10.1097/RHU.0000000000001621. PMID- 20511986 OWN - NLM STAT- MEDLINE DCOM- 20100913 LR - 20100531 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 16 IP - 4 DP - 2010 Jun TI - Raynaud phenomenon of the tongue in a patient with scleroderma. PG - 201 LID - 10.1097/RHU.0b013e3181e08d65 [doi] FAU - Rosato, Edoardo AU - Rosato E AD - Clinical Immunology and Allergy Unit, Department of Clinical Medicine, Sapienza University of Rome, Viale dell'Università, Rome, Italy. edorosato@yahoo.com FAU - Salsano, Felice AU - Salsano F LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Female MH - Humans MH - Raynaud Disease/*complications/psychology MH - Scleroderma, Diffuse/*complications MH - Stress, Psychological/*complications MH - Tongue Diseases/*complications/psychology MH - Young Adult EDAT- 2010/06/01 06:00 MHDA- 2010/09/14 06:00 CRDT- 2010/06/01 06:00 PHST- 2010/06/01 06:00 [entrez] PHST- 2010/06/01 06:00 [pubmed] PHST- 2010/09/14 06:00 [medline] AID - 00124743-201006000-00019 [pii] AID - 10.1097/RHU.0b013e3181e08d65 [doi] PST - ppublish SO - J Clin Rheumatol. 2010 Jun;16(4):201. doi: 10.1097/RHU.0b013e3181e08d65. PMID- 28985172 OWN - NLM STAT- MEDLINE DCOM- 20180904 LR - 20180904 IS - 1939-2869 (Electronic) IS - 0891-1150 (Linking) VI - 84 IP - 10 DP - 2017 Oct TI - Treating Raynaud phenomenon: Beyond staying warm. PG - 797-804 LID - 10.3949/ccjm.84a.17025 [doi] AB - Raynaud phenomenon is an overactive vascular response to cold and emotional stress that results in cutaneous color changes and sensory symptoms in the digits. It can be idiopathic (primary) or secondary to another condition; the latter can be more severe and more apt to lead to ischemic complications such as digital ulceration and even loss of digits. If nonpharmacologic interventions prove inadequate, then vasodilator agents are used. CI - Copyright © 2017 Cleveland Clinic. FAU - Shapiro, Samantha C AU - Shapiro SC AD - Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine Baltimore, MD, USA. sshapi28@jhmi.edu. AD - Postdoctoral Fellow, Johns Hopkins Division of Rheumatology, Baltimore, MD, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. AD - Martha McCrory Professor of Medicine, Johns Hopkins Division of Rheumatology, Baltimore, MD, USA. LA - eng PT - Journal Article PT - Review PL - United States TA - Cleve Clin J Med JT - Cleveland Clinic journal of medicine JID - 8703441 RN - 0 (Calcium Channel Blockers) SB - IM CIN - Cleve Clin J Med. 2017 Oct;84(10):739-740. doi: 10.3949/ccjm.84b.10017. PMID: 28985168 MH - Calcium Channel Blockers/therapeutic use MH - Cold Temperature/adverse effects MH - Humans MH - Raynaud Disease/drug therapy/etiology/*therapy EDAT- 2017/10/07 06:00 MHDA- 2018/09/05 06:00 CRDT- 2017/10/07 06:00 PHST- 2017/10/07 06:00 [entrez] PHST- 2017/10/07 06:00 [pubmed] PHST- 2018/09/05 06:00 [medline] AID - 10.3949/ccjm.84a.17025 [doi] PST - ppublish SO - Cleve Clin J Med. 2017 Oct;84(10):797-804. doi: 10.3949/ccjm.84a.17025. PMID- 26289697 OWN - NLM STAT- MEDLINE DCOM- 20151112 LR - 20181113 IS - 1532-5415 (Electronic) IS - 0002-8614 (Print) IS - 0002-8614 (Linking) VI - 63 IP - 8 DP - 2015 Aug TI - Failure of Glucose Monitoring in an Individual with Pseudohypoglycemia. PG - 1706-8 LID - 10.1111/jgs.13572 [doi] FAU - Lee, Kimberley T AU - Lee KT AD - Internal Medicine Residency Program, School of Medicine, Johns Hopkins University, Baltimore, Maryland. FAU - Abadir, Peter M AU - Abadir PM AD - Division of Geriatric Medicine and Gerontology, School of Medicine, Johns Hopkins University, Baltimore, Maryland. LA - eng GR - P30 AG021334/AG/NIA NIH HHS/United States PT - Case Reports PT - Letter PL - United States TA - J Am Geriatr Soc JT - Journal of the American Geriatrics Society JID - 7503062 RN - 0 (Blood Glucose) SB - IM MH - Aged MH - Blood Glucose/*analysis MH - Blood Glucose Self-Monitoring/*methods MH - *Diagnostic Errors MH - Female MH - Fingers/*blood supply MH - Humans MH - Hypoglycemia/blood/complications/*diagnosis MH - Microcirculation/*physiology MH - Raynaud Disease/blood/*complications/physiopathology MH - Regional Blood Flow/physiology PMC - PMC5407681 MID - NIHMS785936 COIS- Conflict of Interest: None. EDAT- 2015/08/21 06:00 MHDA- 2015/11/13 06:00 PMCR- 2017/04/27 CRDT- 2015/08/21 06:00 PHST- 2015/08/21 06:00 [entrez] PHST- 2015/08/21 06:00 [pubmed] PHST- 2015/11/13 06:00 [medline] PHST- 2017/04/27 00:00 [pmc-release] AID - 10.1111/jgs.13572 [doi] PST - ppublish SO - J Am Geriatr Soc. 2015 Aug;63(8):1706-8. doi: 10.1111/jgs.13572. PMID- 18759283 OWN - NLM STAT- MEDLINE DCOM- 20100331 LR - 20161124 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 58 IP - 9 DP - 2008 Sep TI - Clinical image: Bone resorption of the third rib in systemic sclerosis. PG - 2873 LID - 10.1002/art.23754 [doi] FAU - Perez-Pampin, Eva AU - Perez-Pampin E AD - Hospital Clinico Universitario, Santiago de Compostela, Spain. FAU - Campos, Joaquin AU - Campos J FAU - de Alegria, Anxo Martinez AU - de Alegria AM FAU - Mera, Antonio AU - Mera A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Bone Resorption/complications/diagnostic imaging/*pathology MH - Female MH - Humans MH - Middle Aged MH - Radiography MH - Raynaud Disease/complications/diagnostic imaging/*pathology MH - Ribs/diagnostic imaging/*pathology MH - Scleroderma, Systemic/complications/diagnostic imaging/*pathology EDAT- 2008/09/02 09:00 MHDA- 2010/04/01 06:00 CRDT- 2008/09/02 09:00 PHST- 2008/09/02 09:00 [pubmed] PHST- 2010/04/01 06:00 [medline] PHST- 2008/09/02 09:00 [entrez] AID - 10.1002/art.23754 [doi] PST - ppublish SO - Arthritis Rheum. 2008 Sep;58(9):2873. doi: 10.1002/art.23754. PMID- 25042024 OWN - NLM STAT- MEDLINE DCOM- 20151117 LR - 20191112 VI - 124 IP - 9 DP - 2014 TI - Laser speckle contrast imaging of Raynaud phenomenon. PG - 483-4 LID - AOP_14_061 [pii] FAU - Hellmann, Marcin AU - Hellmann M FAU - Cracowski, Jean-Luc AU - Cracowski JL LA - eng PT - Journal Article DEP - 20140721 PL - Poland TA - Pol Arch Med Wewn JT - Polskie Archiwum Medycyny Wewnetrznej JID - 0401225 RN - 0 (Contrast Media) SB - IM MH - *Contrast Media MH - Diagnostic Imaging/*methods MH - Humans MH - *Lasers MH - Light MH - Radiography MH - Raynaud Disease/*diagnosis/diagnostic imaging MH - *Scattering, Radiation EDAT- 2014/07/22 06:00 MHDA- 2015/11/18 06:00 CRDT- 2014/07/22 06:00 PHST- 2014/07/22 06:00 [entrez] PHST- 2014/07/22 06:00 [pubmed] PHST- 2015/11/18 06:00 [medline] AID - AOP_14_061 [pii] AID - 10.20452/pamw.2411 [doi] PST - ppublish SO - Pol Arch Med Wewn. 2014;124(9):483-4. doi: 10.20452/pamw.2411. Epub 2014 Jul 21. PMID- 40607749 OWN - NLM STAT- MEDLINE DCOM- 20250905 LR - 20250907 IS - 1346-8138 (Electronic) IS - 0385-2407 (Print) IS - 0385-2407 (Linking) VI - 52 IP - 9 DP - 2025 Sep TI - The Prevalence and Severity of Pain in Patients With Systemic Sclerosis. PG - 1382-1394 LID - 10.1111/1346-8138.17843 [doi] AB - We investigated pain prevalence and severity in systemic sclerosis (SSc) and patient-physician perceptions. This was an internet-based survey that compared perceptions of pain type, location, severity, associated factors between patients with SSc and physicians, and pain treatment prescription patterns in Japan in March 2023. Data from 301 patients and 129 physicians revealed that 96.0% of patients experienced pain compared with 43.4% estimated by physicians. The median (interquartile range) Short-form McGill Pain Questionnaire (SF-MPQ-2) pain score was 47.0 (14.0-88.0). Continuous pain had the highest score (16.0 [3.0-27.0]), followed by neuropathic pain (14.0 [5.0-25.0]), intermittent pain (11.0 [1.0-25.0]), and affective descriptors (5.0 [1.0-14.0]). Pain at joints, fingertips, Raynaud's phenomenon (RP), and skin tightening were most prevalent across multiple pain types. Pain at fingertips and RP-affected locations were more common in limited cutaneous SSc (lcSSc) than in diffuse cutaneous SSc (dcSSc), and skin tightening was more common in dcSSc. Patients with dcSSc had significantly more severe pain than patients with lcSSc. Patients with nausea, insomnia, or diarrhea showed higher SF-MPQ-2 scores. Of the 129 physicians surveyed, 58.9% prescribed painkillers, 48.8% suggested self-care, 42.6% treated skin symptoms, and 16.3% referred patients to pain clinics for further management. Compared to the percentage of patients having pain in each location, physicians tend to be less aware of pain in the muscles, head, and abdomen. Most patients with SSc experience pain, which physicians tended to underestimate. Physicians' awareness of patients' experiences should be improved to provide adequate treatment for pain in SSc. Trial Registration: UMIN000050368. CI - © 2025 The Author(s). The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association. FAU - Motegi, Sei-Ichiro AU - Motegi SI AUID- ORCID: 0000-0001-8286-0669 AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uchida-Yamada, Mona AU - Uchida-Yamada M AUID- ORCID: 0000-0002-5781-9485 AD - Medical Affairs, Kyowa Kirin Co., Ltd., Tokyo, Japan. FAU - Shima, Yoshihito AU - Shima Y AUID- ORCID: 0000-0002-2523-2938 AD - Laboratory of Thermo-Therapeutics for Vascular Dysfunction, Osaka University Graduate School of Medicine, Suita, Japan. FAU - Shimada, Taku AU - Shimada T AD - Medical Affairs, Kyowa Kirin Co., Ltd., Tokyo, Japan. FAU - Ishii, Haruka AU - Ishii H AD - Medical Affairs, Kyowa Kirin Co., Ltd., Tokyo, Japan. FAU - Ohya, Yoshito AU - Ohya Y AD - Medical Affairs, Kyowa Kirin Co., Ltd., Tokyo, Japan. FAU - Kanai, Yasumasa AU - Kanai Y AUID- ORCID: 0009-0005-0300-4917 AD - Medical Affairs, Kyowa Kirin Co., Ltd., Tokyo, Japan. LA - eng GR - Kyowa Kirin Pharmaceutical Development/ PT - Journal Article DEP - 20250703 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - Humans MH - Male MH - Female MH - Prevalence MH - Middle Aged MH - Severity of Illness Index MH - *Scleroderma, Systemic/complications MH - Aged MH - Japan/epidemiology MH - Pain Measurement/statistics & numerical data MH - Surveys and Questionnaires/statistics & numerical data MH - Adult MH - *Pain/epidemiology/etiology/diagnosis/drug therapy MH - Pain Management/statistics & numerical data/methods MH - Raynaud Disease/etiology MH - *Neuralgia/epidemiology/etiology PMC - PMC12411825 OTO - NOTNLM OT - SF‐MPQ‐2 OT - gap analysis OT - physicians OT - questionnaire OT - systemic sclerosis COIS- Sei‐ichiro Motegi has received manuscript fees from Kyowa Kirin Co., Ltd.; grants or contracts from Taiho Pharmaceutical Co., Ltd., Sun Pharma Japan Ltd., Novartis Pharma K.K., Kaken Pharmaceutical Co., Ltd., Eli Lilly Japan K.K., and Maruho Co., Ltd.; and payment or honoraria from Janssen Pharmaceutical K.K., Eli Lilly Japan K.K., AbbVie G.K., Sanofi K.K., Daiichi Sankyo Co., Ltd., LEO Pharma K.K., Kyowa Kirin Co., Ltd., Nobelpharma Co., Ltd., Maruho, Co., Ltd., Torii Pharmaceutical Co., Ltd., Kaken Pharmaceutical Co., Ltd., Eisai Co., Ltd., Taiho Pharmaceutical Co., Ltd., Otsuka Pharmaceutical Co., Ltd., Bristol‐Myers Squibb K.K., and Sato Yakuhin Kogyo Co., Ltd. Yoshihito Shima has received manuscript fees from Kyowa Kirin Co., Ltd.; grants or contracts from Kobayashi Pharmaceutical Co., Ltd.; payment or honoraria from Asahi Kasei Pharma Corporation, Chugai Pharmaceutical Co., Ltd., and GlaxoSmithKline K.K.; and patents planned, issued, or pending from Kobayashi Pharmaceutical Co., Ltd. Mona Uchida‐Yamada, Taku Shimada, Haruka Ishii, Yoshito Ohya, and Yasumasa Kanai are employees of Kyowa Kirin Co., Ltd. Mona Uchida‐Yamada, Taku Shimada, Haruka Ishii, and Yoshito Ohya own stock in the company. EDAT- 2025/07/03 12:30 MHDA- 2025/09/05 06:35 PMCR- 2025/09/05 CRDT- 2025/07/03 08:32 PHST- 2025/05/27 00:00 [revised] PHST- 2025/02/06 00:00 [received] PHST- 2025/06/20 00:00 [accepted] PHST- 2025/09/05 06:35 [medline] PHST- 2025/07/03 12:30 [pubmed] PHST- 2025/07/03 08:32 [entrez] PHST- 2025/09/05 00:00 [pmc-release] AID - JDE17843 [pii] AID - 10.1111/1346-8138.17843 [doi] PST - ppublish SO - J Dermatol. 2025 Sep;52(9):1382-1394. doi: 10.1111/1346-8138.17843. Epub 2025 Jul 3. PMID- 21910706 OWN - NLM STAT- MEDLINE DCOM- 20120209 LR - 20111128 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 165 IP - 6 DP - 2011 Dec TI - Raynaud phenomenon, digital gangrene and hypergammaglobulinaemic purpura occurring in a patient with IgG4-related disease. PG - 1364-6 LID - 10.1111/j.1365-2133.2011.10609.x [doi] FAU - Ikawa, T AU - Ikawa T FAU - Kasuya, A AU - Kasuya A FAU - Hirakawa, S AU - Hirakawa S FAU - Tokura, Y AU - Tokura Y LA - eng PT - Case Reports PT - Letter PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Immunoglobulin G) SB - IM MH - Aged MH - Female MH - Fingers/*pathology MH - Gangrene/etiology/pathology MH - Humans MH - Hypergammaglobulinemia/complications/*pathology MH - Immunoglobulin G/*metabolism MH - Purpura/etiology/*pathology MH - Raynaud Disease/etiology/*pathology EDAT- 2011/09/14 06:00 MHDA- 2012/02/10 06:00 CRDT- 2011/09/14 06:00 PHST- 2011/09/14 06:00 [entrez] PHST- 2011/09/14 06:00 [pubmed] PHST- 2012/02/10 06:00 [medline] AID - 10.1111/j.1365-2133.2011.10609.x [doi] PST - ppublish SO - Br J Dermatol. 2011 Dec;165(6):1364-6. doi: 10.1111/j.1365-2133.2011.10609.x. PMID- 17938362 OWN - NLM STAT- MEDLINE DCOM- 20071113 LR - 20131121 IS - 0003-987X (Print) IS - 0003-987X (Linking) VI - 143 IP - 10 DP - 2007 Oct TI - Resolution of chronic pain and fingertip ulceration due to hand-arm vibration syndrome following combination pharmacotherapy. PG - 1343-4 FAU - Buell, Catherine AU - Buell C FAU - Tobinick, Edward AU - Tobinick E FAU - Lamp, Karen AU - Lamp K LA - eng PT - Case Reports PT - Letter PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) RN - SD6QCT3TSU (Pentoxifylline) SB - IM MH - Chronic Disease MH - Drug Therapy, Combination MH - Hand-Arm Vibration Syndrome/*complications MH - Humans MH - Male MH - Middle Aged MH - Nifedipine/therapeutic use MH - Pain/*etiology MH - Pentoxifylline/therapeutic use MH - Raynaud Disease/complications/*drug therapy/*etiology MH - Skin Ulcer/*etiology/pathology MH - Vasodilator Agents/*therapeutic use EDAT- 2007/10/17 09:00 MHDA- 2007/11/14 09:00 CRDT- 2007/10/17 09:00 PHST- 2007/10/17 09:00 [pubmed] PHST- 2007/11/14 09:00 [medline] PHST- 2007/10/17 09:00 [entrez] AID - 143/10/1343 [pii] AID - 10.1001/archderm.143.10.1343 [doi] PST - ppublish SO - Arch Dermatol. 2007 Oct;143(10):1343-4. doi: 10.1001/archderm.143.10.1343. PMID- 38418310 OWN - NLM STAT- MEDLINE DCOM- 20240930 LR - 20250331 IS - 1578-8989 (Electronic) IS - 0025-7753 (Linking) VI - 163 IP - 7 DP - 2024 Oct 18 TI - Raynaud phenomenon in a baseball player. PG - e79 LID - S0025-7753(24)00059-9 [pii] LID - 10.1016/j.medcli.2023.12.017 [doi] FAU - Yoshimoto, Kiyomi AU - Yoshimoto K AD - Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara 634-8522, Japan. Electronic address: kym@naramed-u.ac.jp. FAU - Kawashima, Hiromasa AU - Kawashima H AD - Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara 634-8522, Japan. FAU - Nakakita, Jun AU - Nakakita J AD - Department of General Medicine, Nara Medical University Hospital, Kashihara, Nara 634-8522, Japan. FAU - Nishio, Kenji AU - Nishio K AD - Department of General Medicine, Uda City Hospital, Uda, Nara 633-0298, Japan. LA - eng LA - spa PT - Case Reports PT - Letter DEP - 20240227 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - *Baseball/injuries MH - Male MH - Adult EDAT- 2024/02/29 00:43 MHDA- 2024/10/01 00:42 CRDT- 2024/02/28 21:54 PHST- 2023/11/26 00:00 [received] PHST- 2023/12/05 00:00 [revised] PHST- 2023/12/07 00:00 [accepted] PHST- 2024/10/01 00:42 [medline] PHST- 2024/02/29 00:43 [pubmed] PHST- 2024/02/28 21:54 [entrez] AID - S0025-7753(24)00059-9 [pii] AID - 10.1016/j.medcli.2023.12.017 [doi] PST - ppublish SO - Med Clin (Barc). 2024 Oct 18;163(7):e79. doi: 10.1016/j.medcli.2023.12.017. Epub 2024 Feb 27. PMID- 31135869 OWN - NLM STAT- MEDLINE DCOM- 20200520 LR - 20200520 IS - 1945-1997 (Electronic) IS - 0098-6151 (Linking) VI - 119 IP - 6 DP - 2019 Jun 1 TI - Primary Raynaud Disease. PG - 401 LID - 10.7556/jaoa.2019.072 [doi] FAU - Go, David AU - Go D FAU - Shubrook, Jay H AU - Shubrook JH LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Am Osteopath Assoc JT - The Journal of the American Osteopathic Association JID - 7503065 RN - 059QF0KO0R (Water) SB - IM MH - Cold Temperature MH - Female MH - Humans MH - Raynaud Disease/*diagnosis MH - Water MH - Young Adult EDAT- 2019/05/29 06:00 MHDA- 2020/05/21 06:00 CRDT- 2019/05/29 06:00 PHST- 2019/05/29 06:00 [entrez] PHST- 2019/05/29 06:00 [pubmed] PHST- 2020/05/21 06:00 [medline] AID - 2735175 [pii] AID - 10.7556/jaoa.2019.072 [doi] PST - ppublish SO - J Am Osteopath Assoc. 2019 Jun 1;119(6):401. doi: 10.7556/jaoa.2019.072. PMID- 37163538 OWN - NLM STAT- MEDLINE DCOM- 20230731 LR - 20230907 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 152 IP - 2 DP - 2023 Aug 1 TI - Reply: Scleroderma and Raynaud Phenomenon: The Cold Truth Regarding the Use of Operative Management. PG - 370e-371e LID - 10.1097/PRS.0000000000010424 [doi] FAU - Hakami, Lee M AU - Hakami LM AD - Department of Plastic Surgery, University of Virginia. FAU - Forster, Grace L AU - Forster GL AD - School of Medicine, University of Virginia. FAU - Jones, Marieke K AU - Jones MK AD - Department of Biomedical Science, Claude Moore Health Sciences Library, University of Virginia. FAU - DeGeorge, Brent R Jr AU - DeGeorge BR Jr AD - Department of Plastic Surgery, University of Virginia, Charlottesville, VA. LA - eng PT - Comment PT - Letter DEP - 20230510 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CON - Plast Reconstr Surg. 2023 Aug 1;152(2):369e-370e. doi: 10.1097/PRS.0000000000010423. PMID: 37163536 MH - Humans MH - *Scleroderma, Localized MH - Nails MH - *Raynaud Disease/etiology/surgery MH - Cold Temperature EDAT- 2023/05/10 18:41 MHDA- 2023/07/31 06:43 CRDT- 2023/05/10 13:43 PHST- 2023/07/31 06:43 [medline] PHST- 2023/05/10 18:41 [pubmed] PHST- 2023/05/10 13:43 [entrez] AID - 00006534-202308000-00051 [pii] AID - 10.1097/PRS.0000000000010424 [doi] PST - ppublish SO - Plast Reconstr Surg. 2023 Aug 1;152(2):370e-371e. doi: 10.1097/PRS.0000000000010424. Epub 2023 May 10. PMID- 37163536 OWN - NLM STAT- MEDLINE DCOM- 20230731 LR - 20230907 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 152 IP - 2 DP - 2023 Aug 1 TI - Scleroderma and Raynaud Phenomenon: The Cold Truth Regarding the Use of Operative Management. PG - 369e-370e LID - 10.1097/PRS.0000000000010423 [doi] FAU - Kuo, Shih-Chiang Edward AU - Kuo SE AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins University School of Medicine. FAU - Atayeva, Rena AU - Atayeva R AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins University School of Medicine. FAU - McGready, John AU - McGready J AD - Department of Biostatistics, Johns Hopkins Bloomberg School of Public Health. FAU - Cooney, Carisa M AU - Cooney CM AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. FAU - Lifchez, Scott D AU - Lifchez SD AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. LA - eng PT - Comment PT - Letter DEP - 20230510 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CON - Plast Reconstr Surg. 2022 Jul 1;150(1):105e-114e. doi: 10.1097/PRS.0000000000009187. PMID: 35544320 CIN - Plast Reconstr Surg. 2023 Aug 1;152(2):370e-371e. doi: 10.1097/PRS.0000000000010424. PMID: 37163538 MH - Humans MH - *Scleroderma, Localized MH - *Raynaud Disease/etiology/surgery MH - Cold Temperature EDAT- 2023/05/10 18:41 MHDA- 2023/07/31 06:43 CRDT- 2023/05/10 13:43 PHST- 2023/07/31 06:43 [medline] PHST- 2023/05/10 18:41 [pubmed] PHST- 2023/05/10 13:43 [entrez] AID - 00006534-202308000-00050 [pii] AID - 10.1097/PRS.0000000000010423 [doi] PST - ppublish SO - Plast Reconstr Surg. 2023 Aug 1;152(2):369e-370e. doi: 10.1097/PRS.0000000000010423. Epub 2023 May 10. PMID- 27834783 OWN - NLM STAT- MEDLINE DCOM- 20170602 LR - 20170602 IS - 1878-4429 (Electronic) IS - 1878-4429 (Linking) VI - 9 IP - 4 DP - 2016 TI - Neonatal Behçet's disease with Raynaud phenomenon. PG - 423-425 LID - 10.3233/NPM-1631 [doi] AB - We report a new clinical manifestation of neonatal Behcet's disease. A newborn from a mother with active Behcet's disease during pregnancy, who develops vasomotor phenomena (Raynaud) with oral ulcerations in the second day of life. Neonatal mucocutaneous lesions have been reported previously in few newborns of pregnant women with active Behçet disease. Although neonatal disease is a very infrequent situation, with a mostly transient and favorable course, clinicians should be aware of serious potential complications associated with this entity. FAU - Fernández-Fructuoso, J R AU - Fernández-Fructuoso JR FAU - Gil-Sánchez, S AU - Gil-Sánchez S FAU - Gómez-Santos, E AU - Gómez-Santos E FAU - Lloreda-García, J M AU - Lloreda-García JM FAU - Sevilla-Denia, S AU - Sevilla-Denia S FAU - Leante-Castellanos, J L AU - Leante-Castellanos JL LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Neonatal Perinatal Med JT - Journal of neonatal-perinatal medicine JID - 101468335 RN - 0 (Glucocorticoids) RN - 0 (Tubulin Modulators) RN - 9PHQ9Y1OLM (Prednisolone) RN - SML2Y3J35T (Colchicine) RN - VB0R961HZT (Prednisone) SB - IM MH - Behcet Syndrome/complications/drug therapy/*physiopathology MH - Colchicine/therapeutic use MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Infant, Newborn MH - Male MH - Oral Ulcer/drug therapy/etiology/*physiopathology MH - Prednisolone/therapeutic use MH - Prednisone/therapeutic use MH - Pregnancy MH - Pregnancy Complications, Cardiovascular/drug therapy/*physiopathology MH - Raynaud Disease/drug therapy/etiology/*physiopathology MH - Tubulin Modulators/therapeutic use MH - Young Adult OTO - NOTNLM OT - Behcet disease OT - Raynaud phenomenon OT - neonatal EDAT- 2016/11/12 06:00 MHDA- 2017/06/03 06:00 CRDT- 2016/11/12 06:00 PHST- 2016/11/12 06:00 [pubmed] PHST- 2017/06/03 06:00 [medline] PHST- 2016/11/12 06:00 [entrez] AID - NPM1631 [pii] AID - 10.3233/NPM-1631 [doi] PST - ppublish SO - J Neonatal Perinatal Med. 2016;9(4):423-425. doi: 10.3233/NPM-1631. PMID- 32580626 OWN - NLM STAT- MEDLINE DCOM- 20210604 LR - 20210604 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 29 IP - 9 DP - 2020 Aug TI - Podocytopathy as 'stand-alone' involvement in systemic lupus erythematosus: a case report. PG - 1148-1150 LID - 10.1177/0961203320935988 [doi] FAU - Tsuji, Kenji AU - Tsuji K AUID- ORCID: 0000-0001-5442-7008 AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Takatsu, Yoko AU - Takatsu Y AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Katayama, Yu AU - Katayama Y AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Fukushima, Kazuhiko AU - Fukushima K AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Kitamura, Shinji AU - Kitamura S AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Sugiyama, Hitoshi AU - Sugiyama H AD - Department of Human Resource Development of Dialysis Therapy for Kidney Disease, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. FAU - Wada, Jun AU - Wada J AD - Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. LA - eng PT - Case Reports PT - Letter DEP - 20200624 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adult MH - Female MH - Foot MH - Hand MH - Humans MH - Lupus Nephritis/complications/*diagnosis/drug therapy MH - Podocytes/ultrastructure MH - Prednisolone/therapeutic use MH - Raynaud Disease/complications/*diagnosis/drug therapy EDAT- 2020/06/26 06:00 MHDA- 2021/06/05 06:00 CRDT- 2020/06/26 06:00 PHST- 2020/06/26 06:00 [pubmed] PHST- 2021/06/05 06:00 [medline] PHST- 2020/06/26 06:00 [entrez] AID - 10.1177/0961203320935988 [doi] PST - ppublish SO - Lupus. 2020 Aug;29(9):1148-1150. doi: 10.1177/0961203320935988. Epub 2020 Jun 24. PMID- 21946463 OWN - NLM STAT- MEDLINE DCOM- 20120216 LR - 20131121 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 17 IP - 7 DP - 2011 Oct TI - Raynaud phenomenon of the nipple: a rare finding in rheumatology clinic. PG - 371-2 LID - 10.1097/RHU.0b013e31823207d1 [doi] AB - Many clinicians are familiar with the common presentation of Raynaud phenomenon affecting the hands and feet. Patients with Raynaud phenomenon, even in the absence of systemic disease, are frequently treated by rheumatologists. Raynaud phenomenon of the nipple is an important entity to recognize as a cause of severe nipple pain with breast-feeding and is perhaps underrecognized by patients and physicians. We describe a patient with Raynaud phenomenon of the nipple to improve identification of this clinical entity so that appropriate treatment may be instituted, thus allowing mothers to continue nursing. FAU - O'Sullivan, Shauna AU - O'Sullivan S AD - The Rheumatology Service, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. FAU - Keith, Michael P AU - Keith MP LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Diseases/*diagnosis/therapy MH - Breast Feeding MH - Cold Temperature MH - Color MH - Female MH - Humans MH - Mastodynia/diagnosis/therapy MH - Nifedipine/administration & dosage MH - *Nipples MH - Raynaud Disease/*diagnosis/therapy MH - Vasodilator Agents/administration & dosage EDAT- 2011/09/29 06:00 MHDA- 2012/02/18 06:00 CRDT- 2011/09/29 06:00 PHST- 2011/09/29 06:00 [entrez] PHST- 2011/09/29 06:00 [pubmed] PHST- 2012/02/18 06:00 [medline] AID - 10.1097/RHU.0b013e31823207d1 [doi] PST - ppublish SO - J Clin Rheumatol. 2011 Oct;17(7):371-2. doi: 10.1097/RHU.0b013e31823207d1. PMID- 21375075 OWN - NLM STAT- MEDLINE DCOM- 20110323 LR - 20141120 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 40 IP - 1 DP - 2011 Jan TI - Vascular disease in systemic sclerosis. PG - 3-4 FAU - Poredos, P AU - Poredos P LA - eng PT - Comment PT - Editorial PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 RN - 0 (Cardiovascular Agents) RN - 0 (Immunosuppressive Agents) SB - IM CON - Vasa. 2011 Jan;40(1):6-19. doi: 10.1024/0301-1526/a000065. PMID: 21283969 CON - Vasa. 2011 Jan;40(1):20-30. doi: 10.1024/0301-1526/a000066. PMID: 21283970 MH - Cardiovascular Agents/therapeutic use MH - Familial Primary Pulmonary Hypertension MH - Humans MH - Hypertension, Pulmonary/diagnosis/etiology/therapy MH - Immunosuppressive Agents/therapeutic use MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/*etiology/therapy MH - Scleroderma, Systemic/classification/*complications/diagnosis/therapy MH - Treatment Outcome MH - Ulcer/diagnosis/*etiology/therapy EDAT- 2011/03/08 06:00 MHDA- 2011/03/24 06:00 CRDT- 2011/03/08 06:00 PHST- 2011/03/08 06:00 [entrez] PHST- 2011/03/08 06:00 [pubmed] PHST- 2011/03/24 06:00 [medline] AID - 10.1024/0301-1526/a000064 [doi] PST - ppublish SO - Vasa. 2011 Jan;40(1):3-4. doi: 10.1024/0301-1526/a000064. PMID- 25455354 OWN - NLM STAT- MEDLINE DCOM- 20150806 LR - 20141203 IS - 1558-1969 (Electronic) IS - 0749-0712 (Linking) VI - 31 IP - 1 DP - 2015 Feb TI - The role of botulinum toxin in vasospastic disorders of the hand. PG - 23-37 LID - S0749-0712(14)00074-2 [pii] LID - 10.1016/j.hcl.2014.09.003 [doi] AB - Raynaud phenomenon may be a primary disorder or associated with a variety of other autoimmune processes. Raynaud phenomenon produces digital vasospasm, which can lead to ischemia and ulceration. The treatment of Raynaud phenomenon has been difficult because multiple medical treatments have not provided uniform resolution of symptoms. Many patients have turned to surgery and sympathectomies for the treatment of unrelenting vasospasm. Botulinum toxin has been shown to be an effective alternative to surgery, with a single treatment being capable of resolving pain and healing ulcer. This article reviews the use of botulinum toxin for the treatment of Raynaud phenomenon. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Neumeister, Michael W AU - Neumeister MW AD - Institute for Plastic Surgery, Southern Illinois University School of Medicine, 747 North Rutledge, 3rd Floor, PO Box 19653, Springfield, IL 62794, USA. Electronic address: mneumeister@siumed.edu. LA - eng PT - Journal Article PT - Review DEP - 20141125 PL - United States TA - Hand Clin JT - Hand clinics JID - 8510415 RN - 0 (Cardiovascular Agents) RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/*administration & dosage MH - Cardiovascular Agents/*administration & dosage MH - Hand/*blood supply/physiopathology MH - Humans MH - Ischemia/diagnosis/*drug therapy/physiopathology MH - Neuromuscular Agents/*administration & dosage MH - Raynaud Disease/diagnosis/*drug therapy/physiopathology MH - Vasoconstriction/drug effects OTO - NOTNLM OT - Botox OT - Botulinum toxin OT - Ischemia OT - Pain OT - Raynaud OT - Vasodilation OT - Vasospasm EDAT- 2014/12/03 06:00 MHDA- 2015/08/08 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/08/08 06:00 [medline] AID - S0749-0712(14)00074-2 [pii] AID - 10.1016/j.hcl.2014.09.003 [doi] PST - ppublish SO - Hand Clin. 2015 Feb;31(1):23-37. doi: 10.1016/j.hcl.2014.09.003. Epub 2014 Nov 25. PMID- 33974075 OWN - NLM STAT- MEDLINE DCOM- 20220309 LR - 20220309 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 2 DP - 2022 Feb 2 TI - At one's fingertips: can you nail the diagnosis? PG - 876 LID - 10.1093/rheumatology/keab430 [doi] FAU - Dourado, Eduardo AU - Dourado E AUID- ORCID: 0000-0003-2186-2833 AD - Rheumatology Department, Centro Hospitalar Universitário Lisboa Norte. AD - Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon. FAU - Valido, Ana AU - Valido A AD - Rheumatology Department, Unidade Local de Saúde do Litoral Alentejano, Santiago do Cacém, Portugal. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Aged MH - Female MH - Hand/pathology MH - Humans MH - Raynaud Disease/diagnosis/drug therapy/pathology MH - Scleroderma, Systemic/*diagnosis/drug therapy/pathology MH - Sildenafil Citrate/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2021/05/12 06:00 MHDA- 2022/03/11 06:00 CRDT- 2021/05/11 12:19 PHST- 2021/04/09 00:00 [received] PHST- 2021/04/22 00:00 [revised] PHST- 2021/05/11 00:00 [accepted] PHST- 2021/05/12 06:00 [pubmed] PHST- 2022/03/11 06:00 [medline] PHST- 2021/05/11 12:19 [entrez] AID - 6273681 [pii] AID - 10.1093/rheumatology/keab430 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Feb 2;61(2):876. doi: 10.1093/rheumatology/keab430. PMID- 30230582 OWN - NLM STAT- MEDLINE DCOM- 20190826 LR - 20190826 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 46 IP - 4 DP - 2019 Apr TI - Anti-PL-12 antibody-positive antisynthetase syndrome with recurrent digital ulcers. PG - e143-e145 LID - 10.1111/1346-8138.14661 [doi] FAU - Miyagawa, Fumi AU - Miyagawa F AUID- ORCID: 0000-0002-2030-6969 AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. FAU - Azukizawa, Hiroaki AU - Azukizawa H AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. FAU - Mimori, Tsuneyo AU - Mimori T AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Asada, Hideo AU - Asada H AD - Department of Dermatology, Nara Medical University School of Medicine, Nara, Japan. LA - eng PT - Case Reports PT - Letter DEP - 20180919 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome MH - Adult MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/*blood/immunology MH - Female MH - Humans MH - Myositis/blood/*complications/immunology MH - Raynaud Disease/blood/*immunology/pathology MH - Recurrence MH - Skin/immunology/pathology MH - Skin Ulcer/blood/*immunology/pathology EDAT- 2018/09/20 06:00 MHDA- 2019/08/27 06:00 CRDT- 2018/09/20 06:00 PHST- 2018/09/20 06:00 [pubmed] PHST- 2019/08/27 06:00 [medline] PHST- 2018/09/20 06:00 [entrez] AID - 10.1111/1346-8138.14661 [doi] PST - ppublish SO - J Dermatol. 2019 Apr;46(4):e143-e145. doi: 10.1111/1346-8138.14661. Epub 2018 Sep 19. PMID- 23748881 OWN - NLM STAT- MEDLINE DCOM- 20140317 LR - 20140113 IS - 1432-0584 (Electronic) IS - 0939-5555 (Linking) VI - 93 IP - 2 DP - 2014 Feb TI - Lymphoma-associated paraneoplastic digital ischemia. PG - 355-7 LID - 10.1007/s00277-013-1806-1 [doi] FAU - Woei-A-Jin, F J Sherida H AU - Woei-A-Jin FJ AD - Department of Hematology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands, f.j.s.h.woei-a-jin@lumc.nl. FAU - Tamsma, Jouke T AU - Tamsma JT FAU - Khoe, Laurence V AU - Khoe LV FAU - den Hartog, Wietske C E AU - den Hartog WC FAU - Gerritsen, Jan J AU - Gerritsen JJ FAU - Brand, Anneke AU - Brand A LA - eng PT - Case Reports PT - Letter DEP - 20130610 PL - Germany TA - Ann Hematol JT - Annals of hematology JID - 9107334 SB - IM MH - Female MH - *Fingers/blood supply/pathology MH - Humans MH - *Ischemia/complications/pathology/physiopathology MH - *Lymphoma/complications/pathology/physiopathology MH - Middle Aged MH - *Raynaud Disease/complications/pathology/physiopathology EDAT- 2013/06/12 06:00 MHDA- 2014/03/19 06:00 CRDT- 2013/06/11 06:00 PHST- 2013/04/29 00:00 [received] PHST- 2013/05/20 00:00 [accepted] PHST- 2013/06/11 06:00 [entrez] PHST- 2013/06/12 06:00 [pubmed] PHST- 2014/03/19 06:00 [medline] AID - 10.1007/s00277-013-1806-1 [doi] PST - ppublish SO - Ann Hematol. 2014 Feb;93(2):355-7. doi: 10.1007/s00277-013-1806-1. Epub 2013 Jun 10. PMID- 28633610 OWN - NLM STAT- MEDLINE DCOM- 20180918 LR - 20180918 IS - 1477-0377 (Electronic) IS - 1358-863X (Linking) VI - 22 IP - 5 DP - 2017 Oct TI - Popliteal artery entrapment syndrome presenting as Raynaud phenomenon. PG - 435-436 LID - 10.1177/1358863X17714065 [doi] FAU - Passias, Braden AU - Passias B AD - 1 Ohio University Heritage College of Osteopathic Medicine, Athens, OH, USA. FAU - Lyden, Sean AU - Lyden S AD - 2 Department of Vascular Surgery, Cleveland Clinic Foundation, Cleveland, OH, USA. FAU - Frederick, Amanda AU - Frederick A AD - 3 Department of Sonography, Riverside Methodist Hospital, Columbus, OH, USA. FAU - Jolly, Michael AU - Jolly M AD - 4 Department of Cardiovascular Medicine, Riverside Methodist Hospital, Columbus, OH, USA. FAU - Silver, Mitchell J AU - Silver MJ AD - 5 Center for Critical Limb Care, Riverside Methodist Hospital, Columbus, OH, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20170620 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Ankle Brachial Index MH - Arterial Occlusive Diseases/diagnosis/*etiology/physiopathology/surgery MH - Computed Tomography Angiography MH - Constriction, Pathologic MH - Female MH - Humans MH - *Muscle, Skeletal/diagnostic imaging/surgery MH - *Popliteal Artery/diagnostic imaging/physiopathology MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis/*etiology/physiopathology MH - Regional Blood Flow MH - Toes/*blood supply MH - Treatment Outcome MH - Vascular Patency MH - Walk Test MH - Young Adult EDAT- 2017/06/22 06:00 MHDA- 2018/09/19 06:00 CRDT- 2017/06/22 06:00 PHST- 2017/06/22 06:00 [pubmed] PHST- 2018/09/19 06:00 [medline] PHST- 2017/06/22 06:00 [entrez] AID - 10.1177/1358863X17714065 [doi] PST - ppublish SO - Vasc Med. 2017 Oct;22(5):435-436. doi: 10.1177/1358863X17714065. Epub 2017 Jun 20. PMID- 24152263 OWN - NLM STAT- MEDLINE DCOM- 20131031 LR - 20131024 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 369 IP - 17 DP - 2013 Oct 24 TI - Images in clinical medicine. Cold hands associated with scleroderma. PG - 1638 LID - 10.1056/NEJMicm1304702 [doi] FAU - Labrador, Salvador AU - Labrador S AD - Clinica Trasmiera, Cantabria, Spain salvadorlabrador@hotmail.com. FAU - Gonzalez-Gay, Miguel A AU - Gonzalez-Gay MA LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM MH - Female MH - Hand/pathology MH - Humans MH - Middle Aged MH - Raynaud Disease/etiology/*pathology MH - Scleroderma, Limited/*complications EDAT- 2013/10/25 06:00 MHDA- 2013/11/01 06:00 CRDT- 2013/10/25 06:00 PHST- 2013/10/25 06:00 [entrez] PHST- 2013/10/25 06:00 [pubmed] PHST- 2013/11/01 06:00 [medline] AID - 10.1056/NEJMicm1304702 [doi] PST - ppublish SO - N Engl J Med. 2013 Oct 24;369(17):1638. doi: 10.1056/NEJMicm1304702. PMID- 40671560 OWN - NLM STAT- MEDLINE DCOM- 20260129 LR - 20260130 IS - 1525-1470 (Electronic) IS - 0736-8046 (Linking) VI - 43 IP - 1 DP - 2026 Jan-Feb TI - Regional Infantile Hemangiomas Associated With Raynaud Phenomenon. PG - 113-115 LID - 10.1111/pde.16006 [doi] AB - Regional (segmental) infantile hemangioma (IH) can be associated with arterial anomalies, as seen in PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies) and LUMBAR (lower body infantile hemangiomas, urogenital anomalies and ulceration, myelopathy, bony deformities, anorectal malformations and arterial anomalies, and rectal anomalies) associations. We describe two patients with a regional IH on the hand associated with Raynaud phenomenon. We hypothesize that Raynaud phenomenon in the setting of a regional IH may be due to post-traumatic vasospastic response, arterial anomalies, cutaneous atrophy due to the IH, and/or changes in vasomotor regulation. CI - © 2025 Wiley Periodicals LLC. FAU - Bitar, Racquel A AU - Bitar RA AUID- ORCID: 0009-0003-4466-1863 AD - Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts, USA. AD - Department of Immunology, Dermatology Section, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Servattalab, Sarah E AU - Servattalab SE AUID- ORCID: 0000-0002-2999-303X AD - Department of Immunology, Dermatology Section, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Henderson, Lauren A AU - Henderson LA AD - Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Perron, Megan M AU - Perron MM AUID- ORCID: 0000-0003-4330-9692 AD - Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Taghinia, Amir H AU - Taghinia AH AD - Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts, USA. AD - Department of Plastic and Oral Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - Liang, Marilyn G AU - Liang MG AUID- ORCID: 0000-0002-2545-2754 AD - Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts, USA. AD - Department of Immunology, Dermatology Section, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250717 PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 SB - IM MH - Female MH - Humans MH - *Hemangioma/complications/pathology MH - *Raynaud Disease/complications/etiology MH - *Skin Neoplasms/complications/pathology MH - Child, Preschool MH - Adolescent OTO - NOTNLM OT - Raynaud disease OT - child OT - hemangioma OT - upper extremity OT - vascular neoplasms EDAT- 2025/07/17 06:27 MHDA- 2026/01/30 00:54 CRDT- 2025/07/17 04:03 PHST- 2025/05/22 00:00 [revised] PHST- 2025/02/06 00:00 [received] PHST- 2025/05/27 00:00 [accepted] PHST- 2026/01/30 00:54 [medline] PHST- 2025/07/17 06:27 [pubmed] PHST- 2025/07/17 04:03 [entrez] AID - 10.1111/pde.16006 [doi] PST - ppublish SO - Pediatr Dermatol. 2026 Jan-Feb;43(1):113-115. doi: 10.1111/pde.16006. Epub 2025 Jul 17. PMID- 31743267 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20230927 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 1 DP - 2021 Jan 1 TI - Raynaud Phenomenon in Antisynthetase Syndrome Treated With Epoprostenol. PG - e10-e11 LID - 10.1097/RHU.0000000000001193 [doi] FAU - Vick, Eric AU - Vick E AD - From the Departments of Internal Medicine. FAU - Sharma, Divya AU - Sharma D AD - Pathology. FAU - Elwing, Jean AU - Elwing J AD - Pulmonology and Critical Care Medicine, University of Cincinnati Medical Center, Cincinnati, OH. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - DCR9Z582X0 (Epoprostenol) RN - Antisynthetase syndrome SB - IM MH - Epoprostenol MH - Humans MH - *Myositis/diagnosis/drug therapy MH - *Raynaud Disease/diagnosis/drug therapy COIS- The authors have no financial disclosures. It should be noted that this represents an off-label use of epoprostenol. Currently, it is only Food and Drug Administration approved in the United States for the treatment of pulmonary artery hypertension, and not as a treatment for Raynaud phenomenon. The authors have no conflicts of interest related to this topic. EDAT- 2019/11/20 06:00 MHDA- 2021/06/24 06:00 CRDT- 2019/11/20 06:00 PHST- 2019/11/20 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] PHST- 2019/11/20 06:00 [entrez] AID - 00124743-202101000-00015 [pii] AID - 10.1097/RHU.0000000000001193 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Jan 1;27(1):e10-e11. doi: 10.1097/RHU.0000000000001193. PMID- 19852915 OWN - NLM STAT- MEDLINE DCOM- 20100126 LR - 20190917 IS - 0014-2565 (Print) IS - 0014-2565 (Linking) VI - 209 IP - 9 DP - 2009 Oct TI - [59 year-old man with progressive renal failure, orbital pseudotumor, pericardial effusion and Raynaud phenomenon]. PG - 444-6 FAU - Porteiro Sánchez, J AU - Porteiro Sánchez J AD - Centro Hospitalario de Vila Nova de Gaia-Espinho EPE, Vilanova de Gaia, Porto, Portugal. FAU - Brito Pereira de Lima, R J AU - Brito Pereira de Lima RJ FAU - Barbeito Barral, P AU - Barbeito Barral P FAU - Ferro dos Santos Alves, I AU - Ferro dos Santos Alves I LA - spa PT - Case Reports PT - Journal Article TT - Varón de 59 años con insufi ciencia renal progresiva, seudotumor orbitario, derrame pericárdico y fenómeno de Raynaud. PL - Spain TA - Rev Clin Esp JT - Revista clinica espanola JID - 8608576 SB - IM MH - Disease Progression MH - Fibrosis/complications/diagnosis MH - Humans MH - Male MH - Middle Aged MH - Orbital Pseudotumor/*complications/diagnosis MH - Pericardial Effusion/*complications/diagnosis MH - Raynaud Disease/*complications/diagnosis MH - Renal Insufficiency/*complications/diagnosis EDAT- 2009/10/27 06:00 MHDA- 2010/01/27 06:00 CRDT- 2009/10/27 06:00 PHST- 2009/10/27 06:00 [entrez] PHST- 2009/10/27 06:00 [pubmed] PHST- 2010/01/27 06:00 [medline] AID - S0014-2565(09)72518-4 [pii] AID - 10.1016/s0014-2565(09)72518-4 [doi] PST - ppublish SO - Rev Clin Esp. 2009 Oct;209(9):444-6. doi: 10.1016/s0014-2565(09)72518-4. PMID- 25455361 OWN - NLM STAT- MEDLINE DCOM- 20150806 LR - 20141203 IS - 1558-1969 (Electronic) IS - 0749-0712 (Linking) VI - 31 IP - 1 DP - 2015 Feb TI - Role and rationale for extended periarterial sympathectomy in the management of severe Raynaud syndrome: techniques and results. PG - 101-20 LID - S0749-0712(14)00082-1 [pii] LID - 10.1016/j.hcl.2014.09.011 [doi] AB - There is no consensus regarding etiology or best surgical technique for severe Raynaud syndrome in patients with connective tissue disease. Observations after 30 years' experience in more than 100 cases led to the conclusion that an extended periarterial sympathectomy (with or without vein-graft reconstruction) and adjunctive use of Botox topically will offer benefits that exceed palliation and reduce recurrent ulcerations. In this article the rationale for this approach is reviewed, techniques and results are outlined, and a hypothesis for the mechanism of Raynaud attacks is offered. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Merritt, Wyndell H AU - Merritt WH AD - Division of Plastic & Reconstructive Surgery, Virginia Commonwealth University, 830 East Main Street, Richmond, VA 23298, USA; Department of Plastic & Maxillofacial Surgery, University of Virginia, 1215 Lee Street, Charlottesville, VA 22903, USA. Electronic address: wyndell@hotmail.com. LA - eng PT - Journal Article PT - Review DEP - 20141125 PL - United States TA - Hand Clin JT - Hand clinics JID - 8510415 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/therapeutic use MH - Hand/*blood supply/physiopathology/surgery MH - Humans MH - Ischemia/diagnosis/physiopathology/*surgery/therapy MH - Neuromuscular Agents/therapeutic use MH - Radial Artery/*surgery MH - Raynaud Disease/diagnosis/*physiopathology/*surgery MH - *Sympathectomy MH - Ulnar Artery/*surgery MH - Vascular Grafting OTO - NOTNLM OT - Botulinum toxin OT - Evidence-based data OT - Extended periarterial sympathectomy OT - Raynaud syndrome EDAT- 2014/12/03 06:00 MHDA- 2015/08/08 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/08/08 06:00 [medline] AID - S0749-0712(14)00082-1 [pii] AID - 10.1016/j.hcl.2014.09.011 [doi] PST - ppublish SO - Hand Clin. 2015 Feb;31(1):101-20. doi: 10.1016/j.hcl.2014.09.011. Epub 2014 Nov 25. PMID- 20726685 OWN - NLM STAT- MEDLINE DCOM- 20101102 LR - 20101012 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 39 IP - 5 DP - 2010 TI - Capillaroscopy shows an active pattern of scleroderma in coeliac disease. PG - 438-9 LID - 10.3109/03009742.2010.489230 [doi] FAU - Thonhofer, R AU - Thonhofer R FAU - Trummer, M AU - Trummer M FAU - Siegel, C AU - Siegel C LA - eng PT - Case Reports PT - Letter PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Celiac Disease/*complications/diagnosis MH - Female MH - Humans MH - Microcirculation MH - Microscopic Angioscopy MH - Nails/blood supply MH - Raynaud Disease/diagnosis/etiology MH - Scleroderma, Systemic/diagnosis/*etiology EDAT- 2010/08/24 06:00 MHDA- 2010/11/03 06:00 CRDT- 2010/08/24 06:00 PHST- 2010/08/24 06:00 [entrez] PHST- 2010/08/24 06:00 [pubmed] PHST- 2010/11/03 06:00 [medline] AID - 10.3109/03009742.2010.489230 [doi] PST - ppublish SO - Scand J Rheumatol. 2010;39(5):438-9. doi: 10.3109/03009742.2010.489230. PMID- 29423873 OWN - NLM STAT- MEDLINE DCOM- 20190103 LR - 20190103 IS - 2240-2993 (Electronic) IS - 0300-9009 (Linking) VI - 118 IP - 1 DP - 2018 Mar TI - An unusual phenocopy of the HANAC syndrome without genetic involvement of COL4A1/COL4A2. PG - 135-136 LID - 10.1007/s13760-018-0890-3 [doi] FAU - Bougea, Anastasia AU - Bougea A AUID- ORCID: 0000-0003-3006-8711 AD - 1st Department of Neurology, Cognitive and Movement Disorders Clinic, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72 Vas. Sophias Ave, 11528, Athens, Greece. annita139@yahoo.gr. FAU - Kapaki, Elisabeth AU - Kapaki E AD - 1st Department of Neurology, Cognitive and Movement Disorders Clinic, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72 Vas. Sophias Ave, 11528, Athens, Greece. FAU - Constantinides, Vassilis AU - Constantinides V AD - 1st Department of Neurology, Cognitive and Movement Disorders Clinic, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72 Vas. Sophias Ave, 11528, Athens, Greece. FAU - Yapijakis, Christos AU - Yapijakis C AD - 1st Department of Neurology, Cognitive and Movement Disorders Clinic, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72 Vas. Sophias Ave, 11528, Athens, Greece. FAU - Paraskevas, George P AU - Paraskevas GP AD - 1st Department of Neurology, Cognitive and Movement Disorders Clinic, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72 Vas. Sophias Ave, 11528, Athens, Greece. LA - eng PT - Case Reports PT - Letter DEP - 20180208 PL - Italy TA - Acta Neurol Belg JT - Acta neurologica Belgica JID - 0247035 RN - 0 (COL4A1 protein, human) RN - 0 (COL4A2 protein, human) RN - 0 (Collagen Type IV) RN - 0 (NOTCH3 protein, human) RN - 0 (Receptor, Notch3) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Collagen Type IV/genetics MH - Humans MH - Male MH - Middle Aged MH - Muscle Cramp/*diagnosis/pathology/physiopathology MH - Pedigree MH - Raynaud Disease/*diagnosis/pathology/physiopathology MH - Receptor, Notch3/genetics EDAT- 2018/02/10 06:00 MHDA- 2019/01/04 06:00 CRDT- 2018/02/10 06:00 PHST- 2017/10/10 00:00 [received] PHST- 2018/01/29 00:00 [accepted] PHST- 2018/02/10 06:00 [pubmed] PHST- 2019/01/04 06:00 [medline] PHST- 2018/02/10 06:00 [entrez] AID - 10.1007/s13760-018-0890-3 [pii] AID - 10.1007/s13760-018-0890-3 [doi] PST - ppublish SO - Acta Neurol Belg. 2018 Mar;118(1):135-136. doi: 10.1007/s13760-018-0890-3. Epub 2018 Feb 8. PMID- 33752853 OWN - NLM STAT- MEDLINE DCOM- 20210707 LR - 20210707 IS - 2542-4513 (Electronic) IS - 2542-4513 (Linking) VI - 46 IP - 2 DP - 2021 Apr TI - Fingerstick artefactual hypoglycaemia: A clinical case report. PG - 97-99 LID - S2542-4513(21)00025-0 [pii] LID - 10.1016/j.jdmv.2021.02.001 [doi] FAU - Moreau, Y AU - Moreau Y AD - CHU De Limoges, 2, avenue Martin-Luther-King, 87000 Limoges, France. Electronic address: moreau.yse@gmail.com. FAU - Nassouri, S AU - Nassouri S AD - Hôpital d'instruction des armées Begin, 69, avenue de Paris, 94160 Saint-Mandé, France. FAU - Teissier, M P AU - Teissier MP AD - CHU De Limoges, 2, avenue Martin-Luther-King, 87000 Limoges, France. LA - eng PT - Case Reports PT - Letter DEP - 20210226 PL - France TA - J Med Vasc JT - Journal de medecine vasculaire JID - 101709200 RN - 0 (Biomarkers) RN - 0 (Blood Glucose) SB - IM MH - Artifacts MH - Biomarkers/blood MH - Blood Glucose/*metabolism MH - Diagnostic Errors MH - Female MH - Humans MH - Hypoglycemia/blood/*diagnosis MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/complications/diagnosis/*physiopathology MH - Scleroderma, Systemic/complications/diagnosis/*physiopathology MH - *Vasoconstriction OTO - NOTNLM OT - Artefactual hypoglycaemia OT - Pseudo-hypoglycaemia OT - Systemic sclerosis EDAT- 2021/03/24 06:00 MHDA- 2021/07/08 06:00 CRDT- 2021/03/23 06:03 PHST- 2020/12/02 00:00 [received] PHST- 2021/01/31 00:00 [accepted] PHST- 2021/03/23 06:03 [entrez] PHST- 2021/03/24 06:00 [pubmed] PHST- 2021/07/08 06:00 [medline] AID - S2542-4513(21)00025-0 [pii] AID - 10.1016/j.jdmv.2021.02.001 [doi] PST - ppublish SO - J Med Vasc. 2021 Apr;46(2):97-99. doi: 10.1016/j.jdmv.2021.02.001. Epub 2021 Feb 26. PMID- 32717000 OWN - NLM STAT- MEDLINE DCOM- 20210624 LR - 20210624 IS - 2326-6929 (Electronic) IS - 0011-4162 (Linking) VI - 105 IP - 6 DP - 2020 Jun TI - Raynaud phenomenon of the nipple successfully treated with nifedipine and gabapentin. PG - E22-E23 FAU - Gallego, Humberto AU - Gallego H AD - Sharp Rees-Stealy Medical Group, San Diego, California, USA. FAU - Aleshaki, Joseph S AU - Aleshaki JS AD - St. Joseph Mercy Hospital, Ann Arbor, Michigan, USA. LA - eng PT - Case Reports PT - Letter PL - United States TA - Cutis JT - Cutis JID - 0006440 RN - 6CW7F3G59X (Gabapentin) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Diseases/*diagnosis/drug therapy MH - Breast Feeding MH - Female MH - Gabapentin/*administration & dosage MH - Humans MH - Nifedipine/*administration & dosage MH - Nipples MH - Pain/etiology MH - Raynaud Disease/*diagnosis/drug therapy MH - Treatment Outcome EDAT- 2020/07/28 06:00 MHDA- 2021/06/25 06:00 CRDT- 2020/07/28 06:00 PHST- 2020/07/28 06:00 [entrez] PHST- 2020/07/28 06:00 [pubmed] PHST- 2021/06/25 06:00 [medline] PST - ppublish SO - Cutis. 2020 Jun;105(6):E22-E23. PMID- 23054928 OWN - NLM STAT- MEDLINE DCOM- 20130822 LR - 20211021 IS - 1525-1497 (Electronic) IS - 0884-8734 (Print) IS - 0884-8734 (Linking) VI - 28 IP - 3 DP - 2013 Mar TI - White and blue fingers: a red flag in sickle cell disease. PG - 477 LID - 10.1007/s11606-012-2238-7 [doi] FAU - Goodman, B Mitchell 3rd AU - Goodman BM 3rd AD - Department of Medicine, Eastern Virginia Medical School, Norfolk, VA 23507, USA. goodmabm@evms.edu FAU - Chen, Ian A AU - Chen IA LA - eng PT - Case Reports PT - Journal Article DEP - 20121005 PL - United States TA - J Gen Intern Med JT - Journal of general internal medicine JID - 8605834 SB - IM MH - Adult MH - Female MH - Fingers/pathology MH - Hemoglobin SC Disease/*complications MH - Humans MH - Raynaud Disease/diagnosis/*etiology PMC - PMC3579976 EDAT- 2012/10/12 06:00 MHDA- 2013/08/24 06:00 PMCR- 2014/03/01 CRDT- 2012/10/12 06:00 PHST- 2012/02/20 00:00 [received] PHST- 2012/09/12 00:00 [accepted] PHST- 2012/06/18 00:00 [revised] PHST- 2012/10/12 06:00 [entrez] PHST- 2012/10/12 06:00 [pubmed] PHST- 2013/08/24 06:00 [medline] PHST- 2014/03/01 00:00 [pmc-release] AID - 2238 [pii] AID - 10.1007/s11606-012-2238-7 [doi] PST - ppublish SO - J Gen Intern Med. 2013 Mar;28(3):477. doi: 10.1007/s11606-012-2238-7. Epub 2012 Oct 5. PMID- 29742540 OWN - NLM STAT- MEDLINE DCOM- 20201207 LR - 20210207 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 26 IP - 1 DP - 2020 Jan TI - Asymptomatic Rhabdomyolysis and Digital Necrosis-Clues for a Rheumatic Disease. PG - e4-e5 LID - 10.1097/RHU.0000000000000747 [doi] FAU - Eugénio, Gisela AU - Eugénio G AD - From the Rheumatology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Daniel, Alexandra AU - Daniel A FAU - Duarte, Cátia AU - Duarte C FAU - Inês, Luis AU - Inês L FAU - Malcata, Armando AU - Malcata A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antirheumatic Agents) RN - 0 (Autoantibodies) RN - 0 (Glucocorticoids) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Antirheumatic Agents/administration & dosage MH - Autoantibodies/blood MH - Diagnosis, Differential MH - Early Diagnosis MH - *Fingers/diagnostic imaging/pathology MH - Glucocorticoids MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Necrosis MH - Positron-Emission Tomography/*methods MH - *Raynaud Disease/complications/diagnosis/immunology MH - Rhabdomyolysis/diagnosis/etiology MH - Rheumatic Diseases/*diagnosis MH - Rituximab/administration & dosage EDAT- 2018/05/10 06:00 MHDA- 2020/12/15 06:00 CRDT- 2018/05/10 06:00 PHST- 2018/05/10 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2018/05/10 06:00 [entrez] AID - 00124743-202001000-00011 [pii] AID - 10.1097/RHU.0000000000000747 [doi] PST - ppublish SO - J Clin Rheumatol. 2020 Jan;26(1):e4-e5. doi: 10.1097/RHU.0000000000000747. PMID- 23324765 OWN - NLM STAT- MEDLINE DCOM- 20130311 LR - 20160524 IS - 2168-6084 (Electronic) IS - 2168-6068 (Linking) VI - 149 IP - 1 DP - 2013 Jan TI - Telangiectases of the distal lower extremities associated with paresthesia and Raynaud phenomenon. PG - 97-102 LID - 10.1001/archderm.149.1.97-b [doi] FAU - Word, Andrew P AU - Word AP AD - University of Texas Southwestern Medical Center, Dallas, TX, USA. FAU - Jackson, Michelle L AU - Jackson ML FAU - Costner, Melissa AU - Costner M FAU - Cockerell, Clay J AU - Cockerell CJ FAU - Sinkre, Prasanna A AU - Sinkre PA LA - eng PT - Case Reports PT - Journal Article PL - United States TA - JAMA Dermatol JT - JAMA dermatology JID - 101589530 RN - 0 (Collagen Type IV) SB - IM MH - Collagen Diseases/diagnosis/*pathology MH - Collagen Type IV/metabolism MH - Female MH - Humans MH - Lower Extremity MH - Paresthesia/diagnosis/pathology MH - Raynaud Disease/diagnosis/*pathology MH - Skin Diseases, Vascular/diagnosis/*pathology MH - Telangiectasis/diagnosis/*pathology MH - Young Adult EDAT- 2013/01/18 06:00 MHDA- 2013/03/12 06:00 CRDT- 2013/01/18 06:00 PHST- 2013/01/18 06:00 [entrez] PHST- 2013/01/18 06:00 [pubmed] PHST- 2013/03/12 06:00 [medline] AID - 1557772 [pii] AID - 10.1001/archderm.149.1.97-b [doi] PST - ppublish SO - JAMA Dermatol. 2013 Jan;149(1):97-102. doi: 10.1001/archderm.149.1.97-b. PMID- 23669128 OWN - NLM STAT- MEDLINE DCOM- 20140402 LR - 20130701 IS - 0736-4679 (Print) IS - 0736-4679 (Linking) VI - 45 IP - 1 DP - 2013 Jul TI - Woman with pale fingers. PG - e17-8 LID - S0736-4679(12)01598-3 [pii] LID - 10.1016/j.jemermed.2012.11.071 [doi] FAU - Robertson, Joshua AU - Robertson J AD - College of Medicine, University of South Florida, Tampa, Florida 33603, USA. FAU - Torano, Francisco AU - Torano F FAU - Peckler, Brad AU - Peckler B LA - eng PT - Case Reports PT - Journal Article DEP - 20130511 PL - United States TA - J Emerg Med JT - The Journal of emergency medicine JID - 8412174 SB - IM MH - Adult MH - Cold Temperature MH - Cyanosis/etiology MH - Female MH - Fingers/*physiopathology MH - Humans MH - Pallor/etiology MH - Paresthesia/etiology MH - Raynaud Disease/*complications/*diagnosis EDAT- 2013/05/15 06:00 MHDA- 2014/04/03 06:00 CRDT- 2013/05/15 06:00 PHST- 2012/03/19 00:00 [received] PHST- 2012/11/06 00:00 [accepted] PHST- 2013/05/15 06:00 [entrez] PHST- 2013/05/15 06:00 [pubmed] PHST- 2014/04/03 06:00 [medline] AID - S0736-4679(12)01598-3 [pii] AID - 10.1016/j.jemermed.2012.11.071 [doi] PST - ppublish SO - J Emerg Med. 2013 Jul;45(1):e17-8. doi: 10.1016/j.jemermed.2012.11.071. Epub 2013 May 11. PMID- 24211176 OWN - NLM STAT- MEDLINE DCOM- 20140922 LR - 20220410 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 39 IP - 1 DP - 2014 Jan TI - Raynaud disease. PG - 121-4 LID - S0363-5023(13)01185-4 [pii] LID - 10.1016/j.jhsa.2013.08.117 [doi] FAU - Butendieck, Ronald R AU - Butendieck RR AD - Departments of Rheumatology and Orthopedic Surgery, Mayo Clinic, Jacksonville, FL. FAU - Murray, Peter M AU - Murray PM AD - Departments of Rheumatology and Orthopedic Surgery, Mayo Clinic, Jacksonville, FL. Electronic address: murray.peter@mayo.edu. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20131106 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Calcium Channel Blockers) RN - 0 (Delayed-Action Preparations) RN - 0 (Enzyme Inhibitors) RN - 0 (Imidazoles) RN - 0 (Vasodilator Agents) RN - 09ZFC7974Q (dazoxiben) RN - I9ZF7L6G2L (Nifedipine) RN - PW8QYA7KI0 (Moxisylyte) SB - IM MH - Calcium Channel Blockers/*therapeutic use MH - Cold Temperature/adverse effects MH - Delayed-Action Preparations MH - Enzyme Inhibitors/therapeutic use MH - Female MH - Humans MH - Imidazoles/therapeutic use MH - Life Style MH - Moxisylyte/therapeutic use MH - Nifedipine/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*diagnosis/*drug therapy MH - Vasodilator Agents/*therapeutic use MH - Young Adult EDAT- 2013/11/12 06:00 MHDA- 2014/09/23 06:00 CRDT- 2013/11/12 06:00 PHST- 2013/05/31 00:00 [received] PHST- 2013/08/25 00:00 [accepted] PHST- 2013/11/12 06:00 [entrez] PHST- 2013/11/12 06:00 [pubmed] PHST- 2014/09/23 06:00 [medline] AID - S0363-5023(13)01185-4 [pii] AID - 10.1016/j.jhsa.2013.08.117 [doi] PST - ppublish SO - J Hand Surg Am. 2014 Jan;39(1):121-4. doi: 10.1016/j.jhsa.2013.08.117. Epub 2013 Nov 6. PMID- 33625134 OWN - NLM STAT- MEDLINE DCOM- 20210913 LR - 20210913 IS - 1524-4725 (Electronic) IS - 1076-0512 (Linking) VI - 47 IP - 5 DP - 2021 May 1 TI - Predicting Negative Outcomes of Cryolipolysis in Patients With and Without Raynaud Disease. PG - 675-677 LID - 10.1097/DSS.0000000000002925 [doi] AB - BACKGROUND: Cryolipolysis is an effective means of noninvasive fat reduction. There are numerous relative contraindications to cryolipolysis including scars, hernias, and cold-related disorders such as Raynaud disease, because cryolipolysis has a theoretical risk of exacerbating these conditions. OBJECTIVE: To examine predictors of negative outcomes of cryolipolysis, especially as it pertains to safety of cryolipolysis in patients with Raynaud disease. MATERIALS AND METHODS: A retrospective review of patients who received consultation for cryolipolysis was conducted and analyzed. RESULTS: Patients with Raynaud disease did not experience any exacerbations of their underlying condition after cryolipolysis. Side effects in all patients, regardless of medical history, were mild, temporary, and not associated with any predisposing factors. CONCLUSION: Cryolipolysis seems to be safe in patients with mild-moderate Raynaud disease. CI - Copyright © 2021 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved. FAU - Yanes, Daniel AU - Yanes D AD - All authors are affiliated with the Dermatology Laser and Cosmetic Center, Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Sawaya, Jennifer AU - Sawaya J FAU - Wanner, Molly AU - Wanner M FAU - Avram, Mathew AU - Avram M LA - eng PT - Journal Article PL - United States TA - Dermatol Surg JT - Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] JID - 9504371 SB - IM MH - Adipose Tissue MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Contraindications, Procedure MH - Cosmetic Techniques/*adverse effects MH - Cryotherapy/*adverse effects MH - Disease Progression MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*complications MH - Retrospective Studies MH - Treatment Failure MH - Young Adult EDAT- 2021/02/25 06:00 MHDA- 2021/09/14 06:00 CRDT- 2021/02/24 11:45 PHST- 2021/02/25 06:00 [pubmed] PHST- 2021/09/14 06:00 [medline] PHST- 2021/02/24 11:45 [entrez] AID - 00042728-202105000-00027 [pii] AID - 10.1097/DSS.0000000000002925 [doi] PST - ppublish SO - Dermatol Surg. 2021 May 1;47(5):675-677. doi: 10.1097/DSS.0000000000002925. PMID- 17644575 OWN - NLM STAT- MEDLINE DCOM- 20071019 LR - 20220225 IS - 0363-6135 (Print) IS - 0363-6135 (Linking) VI - 293 IP - 3 DP - 2007 Sep TI - Estrogen increases smooth muscle expression of alpha2C-adrenoceptors and cold-induced constriction of cutaneous arteries. PG - H1955-61 AB - Raynaud's phenomenon, which is characterized by intense cold-induced constriction of cutaneous arteries, is more common in women compared with men. Cold-induced constriction is mediated in part by enhanced activity of alpha(2C)-adrenoceptors (alpha(2C)-ARs) located on vascular smooth muscle cells (VSMs). Experiments were therefore performed to determine whether 17beta-estradiol regulates alpha(2C)-AR expression and function in cutaneous VSMs. 17beta-Estradiol (0.01-10 nmol/l) increased expression of the alpha(2C)-AR protein and the activity of the alpha(2C)-AR gene promoter in human cultured dermal VSMs, which was assessed following transient transfection of the cells with a promoter-reporter construct. The effect of 17beta-estradiol was associated with increased accumulation of cAMP and activation of the cAMP-responsive Rap2 GTP-binding protein. Transient transfection of VSMs with a dominant-negative mutant of Rap2 inhibited the 17beta-estradiol-induced activation of the alpha(2C)-AR gene promoter, whereas a constitutively active mutant of Rap2 increased alpha(2C)-AR promoter activity. The effects of 17beta-estradiol were inhibited by the estrogen receptor (ER) antagonist, ICI-182780 (1 micromol/l), and were mimicked by a cell-impermeable form of the hormone (estrogen:BSA) or by the selective ER-alpha receptor agonist 4,4',4'''-(4-propyl-[(1)H]-pyrazole-1,3,5-triyl)tris-phenol (PPT; 10 nmol/l) or the selective ER-beta receptor agonist 2,3-bis(4-hydroxyphenyl)-propionitrile (DPN; 10 nmol/l). Therefore, 17beta-estradiol increased expression of alpha(2C)-ARs by interacting with cell surface receptors to cause a cAMP/Rap2-dependent increase in alpha(2C)-AR transcription. In mouse tail arteries, 17beta-estradiol (10 nmol/l) increased alpha(2C)-AR expression and selectively increased the cold-induced amplification of alpha(2)-AR constriction, which is mediated by alpha(2C)-ARs. An estrogen-dependent increase in expression of cold-sensitive alpha(2C)-ARs may contribute to the increased activity of cold-induced vasoconstriction under estrogen-replete conditions. FAU - Eid, A H AU - Eid AH AD - Davis Heart and Lung Research Institute, The Ohio State University, Columbus, Ohio, USA. FAU - Maiti, K AU - Maiti K FAU - Mitra, S AU - Mitra S FAU - Chotani, M A AU - Chotani MA FAU - Flavahan, S AU - Flavahan S FAU - Bailey, S R AU - Bailey SR FAU - Thompson-Torgerson, C S AU - Thompson-Torgerson CS FAU - Flavahan, N A AU - Flavahan NA LA - eng GR - HL-080119/HL/NHLBI NIH HHS/United States GR - OH-008531/OH/NIOSH CDC HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20070720 PL - United States TA - Am J Physiol Heart Circ Physiol JT - American journal of physiology. Heart and circulatory physiology JID - 100901228 RN - 0 (ADRA2C protein, human) RN - 0 (Adra2c protein, mouse) RN - 0 (Estrogen Antagonists) RN - 0 (Receptors, Adrenergic, alpha-2) RN - 0 (Receptors, Estrogen) RN - 22X328QOC4 (Fulvestrant) RN - 31C4KY9ESH (Nitric Oxide) RN - 4TI98Z838E (Estradiol) RN - EC 3.6.1.- (RAP2A protein, human) RN - EC 3.6.5.2 (rap GTP-Binding Proteins) SB - IM MH - Arteries/*metabolism MH - Cells, Cultured MH - *Cold Temperature MH - Dermis/*blood supply MH - Estradiol/analogs & derivatives/*pharmacology MH - Estrogen Antagonists/pharmacology MH - Female MH - Fulvestrant MH - Humans MH - Male MH - Muscle, Smooth, Vascular/*metabolism MH - Nitric Oxide/metabolism MH - Raynaud Disease/metabolism MH - Receptors, Adrenergic, alpha-2/*metabolism MH - Receptors, Estrogen/antagonists & inhibitors/metabolism MH - Signal Transduction/physiology MH - Vasoconstriction/*physiology MH - rap GTP-Binding Proteins/metabolism EDAT- 2007/07/24 09:00 MHDA- 2007/10/20 09:00 CRDT- 2007/07/24 09:00 PHST- 2007/07/24 09:00 [pubmed] PHST- 2007/10/20 09:00 [medline] PHST- 2007/07/24 09:00 [entrez] AID - 00306.2007 [pii] AID - 10.1152/ajpheart.00306.2007 [doi] PST - ppublish SO - Am J Physiol Heart Circ Physiol. 2007 Sep;293(3):H1955-61. doi: 10.1152/ajpheart.00306.2007. Epub 2007 Jul 20. PMID- 18320700 OWN - NLM STAT- MEDLINE DCOM- 20080529 LR - 20191027 IS - 0212-7199 (Print) IS - 0212-7199 (Linking) VI - 24 IP - 12 DP - 2007 Dec TI - [Madelung disease and Raynaud]. PG - 615-6 FAU - Bugarín González, R AU - Bugarín González R FAU - Iglesias Caamaño, M AU - Iglesias Caamaño M FAU - del Río Martín, P AU - del Río Martín P FAU - García Rodríguez, R AU - García Rodríguez R FAU - Vidal Castiñeira, I AU - Vidal Castiñeira I FAU - Pérez Crespo, J M AU - Pérez Crespo JM LA - fre PT - Case Reports PT - Letter TT - Enfermedad de Madelung asociada a Raynaud. PL - Spain TA - An Med Interna JT - Anales de medicina interna (Madrid, Spain : 1984) JID - 9112183 SB - IM MH - Humans MH - Lipomatosis, Multiple Symmetrical/*complications MH - Male MH - Middle Aged MH - Raynaud Disease/*complications EDAT- 2008/03/07 09:00 MHDA- 2008/05/30 09:00 CRDT- 2008/03/07 09:00 PHST- 2008/03/07 09:00 [pubmed] PHST- 2008/05/30 09:00 [medline] PHST- 2008/03/07 09:00 [entrez] AID - 10.4321/s0212-71992007001200018 [doi] PST - ppublish SO - An Med Interna. 2007 Dec;24(12):615-6. doi: 10.4321/s0212-71992007001200018. PMID- 20364497 OWN - NLM STAT- MEDLINE DCOM- 20100423 LR - 20100406 IS - 1438-3276 (Print) IS - 1438-3276 (Linking) VI - 152 IP - 7 DP - 2010 Feb 18 TI - [Cold hurts the fingers. Warmth brings little improvement]. PG - 5 FAU - Rüthemann, Jens AU - Rüthemann J AD - Facharzt für Allgemeinmedizin, Am Kindergarten 2a, D-49843 Uelsen. LA - ger PT - Case Reports PT - Journal Article TT - Bei Kälte schmerzen die Finger. Wärme bringt kaum Besserung. PL - Germany TA - MMW Fortschr Med JT - MMW Fortschritte der Medizin JID - 100893959 RN - 0 (Adrenergic alpha-Antagonists) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Adult MH - Cold Temperature/*adverse effects MH - Diagnosis, Differential MH - Female MH - Humans MH - Raynaud Disease/*diagnosis/drug therapy EDAT- 2010/04/07 06:00 MHDA- 2010/04/24 06:00 CRDT- 2010/04/07 06:00 PHST- 2010/04/07 06:00 [entrez] PHST- 2010/04/07 06:00 [pubmed] PHST- 2010/04/24 06:00 [medline] PST - ppublish SO - MMW Fortschr Med. 2010 Feb 18;152(7):5. PMID- 38141212 OWN - NLM STAT- MEDLINE DCOM- 20240701 LR - 20241120 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 63 IP - 7 DP - 2024 Jul 1 TI - Boxer's shape, an unusual finding in nailfold capillaroscopy. PG - e198 LID - 10.1093/rheumatology/kead695 [doi] FAU - Corzo, Patricia AU - Corzo P AUID- ORCID: 0000-0002-0970-0631 AD - Rheumatology Department, Hospital Clinic, Barcelona, Spain; Members of CAPICAT-MAS: study group for autoimmune diseases and naildfold capillaroscopy of the Catalan Society of Rheumatology. FAU - Gómez-Puerta, Jose A AU - Gómez-Puerta JA AUID- ORCID: 0000-0001-8177-702X AD - Rheumatology Department, Hospital Clinic, Barcelona, Spain; Members of CAPICAT-MAS: study group for autoimmune diseases and naildfold capillaroscopy of the Catalan Society of Rheumatology. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Middle Aged MH - *Microscopic Angioscopy/methods MH - *Nails/blood supply/diagnostic imaging/pathology MH - Raynaud Disease/diagnosis/diagnostic imaging/pathology EDAT- 2023/12/23 22:53 MHDA- 2024/07/01 12:42 CRDT- 2023/12/23 15:43 PHST- 2024/07/01 12:42 [medline] PHST- 2023/12/23 22:53 [pubmed] PHST- 2023/12/23 15:43 [entrez] AID - 7492653 [pii] AID - 10.1093/rheumatology/kead695 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Jul 1;63(7):e198. doi: 10.1093/rheumatology/kead695. PMID- 32077812 OWN - NLM STAT- MEDLINE DCOM- 20200629 LR - 20200629 IS - 1938-9116 (Electronic) IS - 1538-5744 (Linking) VI - 54 IP - 4 DP - 2020 May TI - Ultrasonographic Evaluation of a Patient With a Successful Digital Sympathectomy in Raynaud Phenomenon: A Case Report. PG - 362-366 LID - 10.1177/1538574420907191 [doi] AB - Selective periarterial sympathectomy in Raynaud phenomenon (RP) has not been adequately studied as there was no reliable method to evaluate outcomes. However, dynamic Doppler ultrasonography may have clinical value in the management and follow-up of patients with RP; but few reports describe using the device to assess surgical outcomes. Here, we report a case of successful digital sympathectomy in a single digit and the postoperative evaluation using ultrasonography. A 23-year-old patient with secondary RP underwent surgery targeting both common digital artery (ulnar side) and the proper digital artery (radial side). The procedure yielded immediate pain relief and the improvement of recurrent fingertip ulceration. The 1-year postoperative assessment with dynamic Doppler ultrasonography using a hockey-stick probe was performed with a cold provocation test and revealed peak systolic velocity improvement comparable to the nontreated ulnar side but prominent fibrosis on the radial aspect. We anticipate that Doppler ultrasonography may be an effective tool for the postoperative assessment of patients who underwent digital sympathectomy for treatment of RP. FAU - Lee, Jae Min AU - Lee JM AUID- ORCID: 0000-0001-6173-0611 AD - Department of Plastic and Reconstructive Surgery, Korea University Anam Hospital, Seoul, Korea. FAU - Chung, Jae-Ho AU - Chung JH AUID- ORCID: 0000-0002-8351-2444 AD - Department of Plastic and Reconstructive Surgery, Korea University Anam Hospital, Seoul, Korea. FAU - Yoon, Eul-Sik AU - Yoon ES AD - Department of Plastic and Reconstructive Surgery, Korea University Anam Hospital, Seoul, Korea. FAU - Park, Seung-Ha AU - Park SH AD - Department of Plastic and Reconstructive Surgery, Korea University Anam Hospital, Seoul, Korea. FAU - Lee, Byung-Il AU - Lee BI AD - Department of Plastic and Reconstructive Surgery, Korea University Anam Hospital, Seoul, Korea. LA - eng PT - Case Reports PT - Journal Article DEP - 20200220 PL - United States TA - Vasc Endovascular Surg JT - Vascular and endovascular surgery JID - 101136421 SB - IM MH - Blood Flow Velocity MH - Female MH - Fingers/*blood supply MH - Humans MH - Predictive Value of Tests MH - Radial Artery/*diagnostic imaging/*innervation MH - Raynaud Disease/diagnostic imaging/physiopathology/*surgery MH - Regional Blood Flow MH - *Sympathectomy MH - Treatment Outcome MH - Ulnar Artery/*diagnostic imaging/*innervation MH - *Ultrasonography, Doppler MH - Vascular Patency MH - Young Adult OTO - NOTNLM OT - Raynaud disease OT - adventitial stripping OT - periarterial sympathectomy EDAT- 2020/02/23 06:00 MHDA- 2020/07/01 06:00 CRDT- 2020/02/21 06:00 PHST- 2020/02/23 06:00 [pubmed] PHST- 2020/07/01 06:00 [medline] PHST- 2020/02/21 06:00 [entrez] AID - 10.1177/1538574420907191 [doi] PST - ppublish SO - Vasc Endovascular Surg. 2020 May;54(4):362-366. doi: 10.1177/1538574420907191. Epub 2020 Feb 20. PMID- 38228855 OWN - NLM STAT- MEDLINE DCOM- 20240229 LR - 20241127 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 20 IP - 3 DP - 2024 Mar TI - Raynaud phenomenon: from GWAS to drug repurposing. PG - 139-140 LID - 10.1038/s41584-024-01076-x [doi] FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. ariane.herrick@manchester.ac.uk. FAU - Orozco, Gisela AU - Orozco G AUID- ORCID: 0000-0002-3479-0448 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. LA - eng GR - 207491/Z/17/Z/WT_/Wellcome Trust/United Kingdom PT - Journal Article PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - Humans MH - *Drug Repositioning MH - Genome-Wide Association Study MH - *Raynaud Disease/drug therapy/genetics EDAT- 2024/01/17 00:42 MHDA- 2024/02/29 06:42 CRDT- 2024/01/16 23:26 PHST- 2024/02/29 06:42 [medline] PHST- 2024/01/17 00:42 [pubmed] PHST- 2024/01/16 23:26 [entrez] AID - 10.1038/s41584-024-01076-x [pii] AID - 10.1038/s41584-024-01076-x [doi] PST - ppublish SO - Nat Rev Rheumatol. 2024 Mar;20(3):139-140. doi: 10.1038/s41584-024-01076-x. PMID- 31688838 OWN - NLM STAT- MEDLINE DCOM- 20210122 LR - 20210122 IS - 1897-9483 (Electronic) IS - 0032-3772 (Linking) VI - 130 IP - 1 DP - 2020 Jan 31 TI - Sulodexide improves capillary blood flow and the quality of life in patients with Raynaud syndrome: a pilot study. PG - 79-81 LID - 10.20452/pamw.15053 [doi] FAU - Madycki, Grzegorz AU - Madycki G AD - Department of Vascular Surgery and Angiology, Centre of Postgraduate Medical Education, Warsaw, Poland FAU - Obidzińska-Trościanko, Jolanta AU - Obidzińska-Trościanko J AD - Department of Vascular Surgery and Angiology, Centre of Postgraduate Medical Education, Warsaw, Poland FAU - Juszyński, Michał AU - Juszyński M AD - Department of Vascular Surgery and Angiology, Centre of Postgraduate Medical Education, Warsaw, Poland FAU - Zgliczyński, Wojciech AU - Zgliczyński W AD - Department of Endocrinology, Centre of Postgraduate Medical Education, Warsaw, Poland FAU - Glinicki, Piotr AU - Glinicki P AD - Department of Endocrinology, Centre of Postgraduate Medical Education, Warsaw, Poland. piotr.glinicki@bielanski.med.pl LA - eng PT - Journal Article DEP - 20191105 PL - Poland TA - Pol Arch Intern Med JT - Polish archives of internal medicine JID - 101700960 RN - 0 (Glycosaminoglycans) RN - 75HGV0062C (glucuronyl glucosamine glycan sulfate) SB - IM MH - Adult MH - Female MH - Glycosaminoglycans/*therapeutic use MH - *Hemodynamics MH - Humans MH - Male MH - Middle Aged MH - Pilot Projects MH - *Quality of Life MH - Raynaud Disease/*drug therapy/physiopathology MH - Treatment Outcome EDAT- 2019/11/07 06:00 MHDA- 2021/01/23 06:00 CRDT- 2019/11/06 06:00 PHST- 2019/11/07 06:00 [pubmed] PHST- 2021/01/23 06:00 [medline] PHST- 2019/11/06 06:00 [entrez] AID - 10.20452/pamw.15053 [doi] PST - ppublish SO - Pol Arch Intern Med. 2020 Jan 31;130(1):79-81. doi: 10.20452/pamw.15053. Epub 2019 Nov 5. PMID- 30260839 OWN - NLM STAT- MEDLINE DCOM- 20200323 LR - 20221207 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 82 IP - 1 DP - 2019 Jan TI - Botulinum Toxin Injection for Lower Face and Oral Cavity Raynaud Phenomenon After Mandibulectomy, Free Fibula Reconstruction, and Radiation Therapy. PG - 53-54 LID - 10.1097/SAP.0000000000001622 [doi] AB - Isolated lingual and lower face Raynaud phenomenon without primary Raynaud of the digits is a very rare condition associated with chemoradiation therapy (RT) in previous reports. The condition, which more commonly presents in patients with a history of Raynaud disease, is often self-limiting, but vasodilating agents and steroids have been suggested as possible treatment options. Spasmodic torticollis is a different, more common entity, also associated with history of RT or previous head and neck surgery. We present a rare case of a patient who developed Raynaud phenomenon of the lower face and tongue in the presence of spasmodic torticollis after mandibulectomy and free fibula reconstruction followed by RT to the oral cavity and neck. Possible causes, pathophysiologic mechanisms and treatment options are discussed. This is the first report of botulinum toxin treatment of isolated secondary Raynaud phenomenon of the lower face and tongue. FAU - Boukovalas, Stefanos AU - Boukovalas S FAU - Mays, Ashley C AU - Mays AC AD - Department of Plastic Surgery, MD Anderson Cancer Center, Houston, TX. FAU - Selber, Jesse C AU - Selber JC AD - Department of Plastic Surgery, MD Anderson Cancer Center, Houston, TX. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - Bone Transplantation/adverse effects/methods MH - Botulinum Toxins/*administration & dosage MH - Carcinoma, Squamous Cell/pathology/radiotherapy/surgery MH - Fibula/surgery MH - Follow-Up Studies MH - Humans MH - Male MH - Mandibular Neoplasms/pathology/radiotherapy/*surgery MH - Mandibular Osteotomy/*adverse effects/methods MH - Middle Aged MH - Neck Dissection/adverse effects/methods MH - Radiotherapy, Adjuvant/adverse effects/methods MH - Raynaud Disease/*drug therapy/etiology/physiopathology MH - Plastic Surgery Procedures/*adverse effects/methods MH - Risk Assessment MH - Torticollis/*drug therapy/etiology MH - Treatment Outcome EDAT- 2018/09/28 06:00 MHDA- 2020/03/24 06:00 CRDT- 2018/09/28 06:00 PHST- 2018/09/28 06:00 [pubmed] PHST- 2020/03/24 06:00 [medline] PHST- 2018/09/28 06:00 [entrez] AID - 10.1097/SAP.0000000000001622 [doi] PST - ppublish SO - Ann Plast Surg. 2019 Jan;82(1):53-54. doi: 10.1097/SAP.0000000000001622. PMID- 29454602 OWN - NLM STAT- MEDLINE DCOM- 20190620 LR - 20190620 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 145 Suppl 1 DP - 2018 Mar TI - [Not Available]. PG - S159-S164 LID - S0151-9638(18)30030-9 [pii] LID - 10.1016/j.annder.2018.01.029 [doi] CN - CEDEF LA - fre PT - Journal Article PT - Review TT - Item 237 – UE 8 Acrosyndromes. Phénomène de Raynaud, érythermalgie, acrocyanose, engelures, ischémie digitale. DEP - 20180214 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - *Chilblains/diagnosis/etiology MH - Cyanosis MH - Diagnosis, Differential MH - Embolism, Cholesterol/complications MH - *Erythromelalgia/diagnosis/drug therapy MH - Fingers/*blood supply MH - Humans MH - *Ischemia/diagnosis/etiology MH - *Raynaud Disease/diagnosis/etiology MH - Scleroderma, Systemic/complications MH - Thromboangiitis Obliterans/complications EDAT- 2018/02/20 06:00 MHDA- 2019/06/21 06:00 CRDT- 2018/02/19 06:00 PHST- 2018/02/20 06:00 [pubmed] PHST- 2019/06/21 06:00 [medline] PHST- 2018/02/19 06:00 [entrez] AID - S0151-9638(18)30030-9 [pii] AID - 10.1016/j.annder.2018.01.029 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2018 Mar;145 Suppl 1:S159-S164. doi: 10.1016/j.annder.2018.01.029. Epub 2018 Feb 14. PMID- 26644105 OWN - NLM STAT- MEDLINE DCOM- 20160325 LR - 20151208 IS - 0041-4131 (Print) IS - 0041-4131 (Linking) VI - 93 IP - 6 DP - 2015 Jun TI - [RACAND syndrome: distinct entity or initial presentation of systemic scleroderma]. PG - 392 FAU - Smiti Khanfir, Monia AU - Smiti Khanfir M FAU - Bellakhal, Syrine AU - Bellakhal S FAU - Said, Fatma AU - Said F FAU - Ben Salem, Thouraya AU - Ben Salem T FAU - Ben Ghorbel, Imed AU - Ben Ghorbel I FAU - Lamloum, Mounir AU - Lamloum M FAU - Hamzaoui, Amira AU - Hamzaoui A FAU - Houman, Mohamed Habib AU - Houman MH LA - fre PT - Case Reports PT - Letter TT - RACAND syndrome : Entité distincte de la sclérodermie systémique ou une forme inaugurale ? PL - Tunisia TA - Tunis Med JT - La Tunisie medicale JID - 0413766 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (anticentromere antibody) SB - IM MH - Administration, Intravenous MH - Adult MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - Diagnosis, Differential MH - Drug Therapy, Combination MH - Female MH - Fingers/*pathology MH - Glucocorticoids/administration & dosage/*therapeutic use MH - Humans MH - Immunosuppressive Agents/administration & dosage/*therapeutic use MH - Necrosis MH - Raynaud Disease/blood/*diagnosis/*drug therapy MH - Risk Factors MH - Scleroderma, Systemic/diagnosis MH - Severity of Illness Index MH - Syndrome MH - Time Factors MH - Treatment Outcome EDAT- 2015/12/09 06:00 MHDA- 2016/03/26 06:00 CRDT- 2015/12/09 06:00 PHST- 2015/12/09 06:00 [entrez] PHST- 2015/12/09 06:00 [pubmed] PHST- 2016/03/26 06:00 [medline] AID - /article-medicale-tunisie.php?article=2836 [pii] PST - ppublish SO - Tunis Med. 2015 Jun;93(6):392. PMID- 20538387 OWN - NLM STAT- MEDLINE DCOM- 20110531 LR - 20161018 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 32 IP - 2 DP - 2011 Feb TI - [Nail abnormality]. PG - 118-9 LID - 10.1016/j.revmed.2009.08.022 [doi] FAU - Benoit, J AU - Benoit J AD - Service de médecine interne, hôpital Saint-Antoine, Assistance publique-Hôpitaux de Paris, université Pierre-et-Marie-Curie, 75012 Paris cedex, France. FAU - Tolédano, C AU - Tolédano C FAU - Tiev, K-P AU - Tiev KP FAU - Gain, M AU - Gain M FAU - Josselin-Mahr, L AU - Josselin-Mahr L FAU - Cabane, J AU - Cabane J FAU - Kettaneh, A AU - Kettaneh A LA - fre PT - Case Reports PT - Journal Article TT - Des anomalies unguéales. DEP - 20100609 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Adult MH - Female MH - Humans MH - Nails, Malformed/*etiology MH - Raynaud Disease/*complications/diagnosis EDAT- 2010/06/12 06:00 MHDA- 2011/06/01 06:00 CRDT- 2010/06/12 06:00 PHST- 2009/08/08 00:00 [received] PHST- 2009/08/17 00:00 [accepted] PHST- 2010/06/12 06:00 [entrez] PHST- 2010/06/12 06:00 [pubmed] PHST- 2011/06/01 06:00 [medline] AID - S0248-8663(10)00739-3 [pii] AID - 10.1016/j.revmed.2009.08.022 [doi] PST - ppublish SO - Rev Med Interne. 2011 Feb;32(2):118-9. doi: 10.1016/j.revmed.2009.08.022. Epub 2010 Jun 9. PMID- 27941129 OWN - NLM STAT- MEDLINE DCOM- 20170821 LR - 20260518 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 76 IP - 8 DP - 2017 Aug TI - Update of EULAR recommendations for the treatment of systemic sclerosis. PG - 1327-1339 LID - 10.1136/annrheumdis-2016-209909 [doi] AB - The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Fransen, Jaap AU - Fransen J AD - Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Avouac, Jerome AU - Avouac J AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, Paris, France. FAU - Becker, Mike AU - Becker M AD - University Hospital Charité, Berlin, Germany. AD - University Hospital Zurich, Zurich, Switzerland. FAU - Kulak, Agnieszka AU - Kulak A AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, Paris, France. FAU - Distler, Oliver AU - Distler O AD - University Hospital Zurich, Zurich, Switzerland. FAU - Clements, Philip AU - Clements P AD - University of California at Los Angeles, Los Angeles, California, USA. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Clinical Division of Rheumatology, Department of Internal Medicine, University of Genova, IRCCS AOU San Martino, Genova, Italy. FAU - Czirjak, Laszlo AU - Czirjak L AD - Department of Rheumatology and Immunology, Medical Center, University of Pecs, Pecs, Hungary. FAU - Damjanov, Nemanja AU - Damjanov N AD - University of Belgrade, Belgrade, Serbia. FAU - Del Galdo, Francesco AU - Del Galdo F AD - University of Leeds, Leeds, UK. FAU - Denton, Christopher P AU - Denton CP AD - University College London, London, UK. FAU - Distler, Jörg H W AU - Distler JHW AD - University of Erlangen-Nuremberg, Erlangen, Germany. FAU - Foeldvari, Ivan AU - Foeldvari I AD - Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany. FAU - Figelstone, Kim AU - Figelstone K AD - FESCA, London, UK. FAU - Frerix, Marc AU - Frerix M AD - University of Giessen, Bad Nauheim, Germany. FAU - Furst, Daniel E AU - Furst DE AD - University of California at Los Angeles, Los Angeles, California, USA. FAU - Guiducci, Serena AU - Guiducci S AD - University of Florence, Florence, Italy. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - University of Cologne, Cologne, Germany. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan School of Medicine, Ann Arbor, Michigan, USA. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - University of Florence, Florence, Italy. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester, UK. FAU - van den Hoogen, Frank AU - van den Hoogen F AD - Radboud University Medical Center, Nijmegen, The Netherlands. FAU - van Laar, Jacob M AU - van Laar JM AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - University of Lübeck, Lübeck, Germany. FAU - Silver, Richard AU - Silver R AD - Medical University of South Carolina, Charleston, South Carolina, USA. FAU - Smith, Vanessa AU - Smith V AD - Ghent University Hospital, Ghent University, Ghent, Belgium. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratories and Clinical Division of Rheumatology, Department of Internal Medicine, University of Genova, IRCCS AOU San Martino, Genova, Italy. FAU - Tarner, Ingo AU - Tarner I AD - University of Giessen, Bad Nauheim, Germany. FAU - Tyndall, Alan AU - Tyndall A AD - Basel University, Basel, Switzerland. FAU - Welling, Joep AU - Welling J AD - FESCA Patient Research Partner, The Netherlands. FAU - Wigley, Frederic AU - Wigley F AD - Johns Hopkins University, Baltimore, Maryland, USA. FAU - Valentini, Gabriele AU - Valentini G AD - Second University of Naples, Naples, Italy. FAU - Walker, Ulrich A AU - Walker UA AD - Basel University, Basel, Switzerland. FAU - Zulian, Francesco AU - Zulian F AD - University of Padua, Padua, Italy. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - University of Giessen, Bad Nauheim, Germany. CN - EUSTAR Coauthors LA - eng PT - Consensus Statement PT - Journal Article PT - Practice Guideline PT - Systematic Review DEP - 20161109 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Endothelin Receptor Antagonists) RN - 01K63SUP8D (Fluoxetine) RN - 0 (Glucocorticoids) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins I) RN - 0 (Pyrazoles) RN - 0 (Pyrimidines) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - RU3FE2Y4XI (riociguat) SB - IM MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Delphi Technique MH - Endothelin Receptor Antagonists/therapeutic use MH - Europe MH - Fingers MH - Fluoxetine/therapeutic use MH - Gastrointestinal Diseases/etiology/*therapy MH - Glucocorticoids/therapeutic use MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - Hypertension, Pulmonary/etiology/*therapy MH - Kidney Diseases/etiology/*therapy MH - Lung Diseases/etiology/therapy MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Prostaglandins I/therapeutic use MH - Pyrazoles/therapeutic use MH - Pyrimidines/therapeutic use MH - Raynaud Disease/etiology/*therapy MH - Rheumatology MH - Scleroderma, Systemic/complications/*therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Ulcer/etiology/*therapy OTO - NOTNLM OT - Cyclophosphamide OT - Methotrexate OT - Systemic Sclerosis OT - Treatment COIS- Competing interests: OK-B: consultancies or speakers bureau: AbbVie, Actelion, Bayer, Inventiva, Pfizer, Roche; JA: grant/research support from BMS, Pfizer, Roche/Chugai, Sanofi-Aventis, Actelion; MB: consultant for Actelion; YA: consultant for: Bayer Pharmaceuticals, Actelion, Pfizer, Inventiva, Medac, Servier, Boehringer Ingelheim, Sanofi-Aventis, CSL Behring, Roche; OD: consultant for: 4D Science, Actelion, Active Biotec, Bayer-Schering, Biogen, Biovitrium, BMS, Boehringer Ingelheim Pharma, EpiPharm, Ergonex, GSK, Inventiva, Medac, Novartis, Pfizer, Pharmacyclics, Roche/Genentech, Sanofi/Genzyme, Serodapharm, Sinoxa and United BioSource Corporation; MC: Mundipharm, Actelion, BMS, Horizon, Pfizer, Biogen, Celltrion, Cellgene; LC: consultant for Actelion and Pfizer; CPD: consulting fees from Roche, Actelion, GSK, Bayer Pharmaceuticals; IF: constultant: Bayer, Roche/Chugai; MF: Actelion; DEF: grant/research support from AbbVie, Actelion, Amgen, BMS, NIH, Novartis, Pfizer, Roche/Genentech and consultancy with AbbVie, Actelion, Amgen, BMS, Cytori, Novartis, Pfizer, Roche/Genentech; NH: lecture fees from Actelion, Bayer, Roche; DK: consultancy with Actelion, BMS, Bayer, Covis, Cytori, Genentech/Roche, Gilead, Sanofi-Aventis and grant from NIH/NIAID, NIH/NIAMS, Bayer and BMS; ALH: consultant/speaker/research funding: Actelion, consultant: Apricus; JMvL: honoraria from MSD, BMS, Pfizer, Eli Lilly, Roche; GR: lecturers fees from Bayer, Pfizer, Novartis, Actelion, GSK, BMS and research grants from Actelion; RS: consultant for: Entelligence Program, supported by Actelion; inPractice Rheumatology, grant support: BMS, Bayer; AS: research grant from Actelion; IT: Actelion; UM-L: grant/research support from: EULAR grant, consultant for: Actelion, GSK, Bayer, Medac, Roche/Chugai. FIR - Daikeler, Thomas IR - Daikeler T FIR - Lanciano, Elisabetta IR - Lanciano E FIR - Bečvář, Radim IR - Bečvář R FIR - Tomcik, Michal IR - Tomcik M FIR - Gińdzieńska-Sieśkiewicz, Ewa IR - Gińdzieńska-Sieśkiewicz E FIR - Cuomo, Giovanna IR - Cuomo G FIR - Iudici, Michele IR - Iudici M FIR - Rednic, Simona IR - Rednic S FIR - Vlachoyiannopoulos, Panayiotis G IR - Vlachoyiannopoulos PG FIR - Caporali, Roberto IR - Caporali R FIR - Carreira, Patricia E IR - Carreira PE FIR - de Reumatología, Servicio IR - de Reumatología S FIR - Novak, Srdan IR - Novak S FIR - Minier, Tünde IR - Minier T FIR - Kucharz, Eugene J IR - Kucharz EJ FIR - Gabrielli, Armando IR - Gabrielli A FIR - Moroncini, Gianluca IR - Moroncini G FIR - Airo', Paolo IR - Airo' P FIR - Hesselstrand, Roger IR - Hesselstrand R FIR - Martinovic, Duska IR - Martinovic D FIR - Radić, Mislav IR - Radić M FIR - Marasovic-Krstulovic, Daniela IR - Marasovic-Krstulovic D FIR - Braun-Moscovici, Yolanda IR - Braun-Moscovici Y FIR - Balbir-Gurman, Alexandra IR - Balbir-Gurman A FIR - Monaco, Andrea Lo IR - Monaco AL FIR - Caramaschi, Paola IR - Caramaschi P FIR - Morović-Vergles, Jadranka IR - Morović-Vergles J FIR - Čulo, Melanie I IR - Čulo MI FIR - Henes, Jörg IR - Henes J FIR - Santamaria, Vera Ortiz IR - Santamaria VO FIR - Heitmann, Stefan IR - Heitmann S FIR - Krasowska, Dorota IR - Krasowska D FIR - Michalska-Jakubus, Małgorzata IR - Michalska-Jakubus M FIR - Seidel, Matthias F IR - Seidel MF FIR - Hasler, Paul IR - Hasler P FIR - Rheumatologie, Klinik Für IR - Rheumatologie KF FIR - Silva, José A Pereira Da IR - Silva JAPD FIR - Salvador, Maria J IR - Salvador MJ FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - Stankovic, Aleksandra IR - Stankovic A FIR - Tikly, Mohammed IR - Tikly M FIR - Ananieva, Lidia P IR - Ananieva LP FIR - Beretta, Lorenzo IR - Beretta L FIR - Szucs, Gabriella IR - Szucs G FIR - Szamosi, Szilvia IR - Szamosi S FIR - Bujidos, Carlos de la Puente IR - Bujidos CP FIR - de Reumatología, Servicio IR - de Reumatología S FIR - Midtvedt, Øyvind IR - Midtvedt Ø FIR - Hoffmann-Vold, Anna-Maria IR - Hoffmann-Vold AM FIR - Launay, David IR - Launay D FIR - Hachulla, Eric IR - Hachulla E FIR - Riccieri, Valeria IR - Riccieri V FIR - Ionescu, Ruxandra IR - Ionescu R FIR - Opris, Daniela IR - Opris D FIR - Mihai, Carina IR - Mihai C FIR - Herrgott, Ilka IR - Herrgott I FIR - Beyer, Christian IR - Beyer C FIR - Ingegnoli, Francesca IR - Ingegnoli F FIR - von Mühlen, Carlos Alberto IR - von Mühlen CA FIR - Alegre-Sancho, Juan José IR - Alegre-Sancho JJ FIR - Beltrán-Catalán, Emma IR - Beltrán-Catalán E FIR - Aringer, Martin IR - Aringer M FIR - Fantana, Julia IR - Fantana J FIR - Leuchten, Nicolai IR - Leuchten N FIR - Tausche, Anne-Kathrin IR - Tausche AK FIR - De Langhe, Ellen IR - De Langhe E FIR - Vanthuyne, Marie IR - Vanthuyne M FIR - Anic, Branimir IR - Anic B FIR - Barešić, Marko IR - Barešić M FIR - Mayer, Miroslav IR - Mayer M FIR - Üprus, Maria IR - Üprus M FIR - Otsa, Kati IR - Otsa K FIR - Yavuz, Sule IR - Yavuz S FIR - Granel, Brigitte IR - Granel B FIR - Azevedo, Valderilio F IR - Azevedo VF FIR - Muller, Carolina IR - Muller C FIR - Jimenez, Sergio A IR - Jimenez SA FIR - Popa, Serghei IR - Popa S FIR - Agachi, Svetlana IR - Agachi S FIR - Zenone, Thierry IR - Zenone T FIR - Stebbings, Simon IR - Stebbings S FIR - Dockerty, Joanne IR - Dockerty J FIR - Vacca, Alessandra IR - Vacca A FIR - Schollum, Joanna IR - Schollum J FIR - Veale, Douglas J IR - Veale DJ FIR - Toloza, Sergio IR - Toloza S FIR - Xu, Dong IR - Xu D FIR - Olas, Jacek IR - Olas J FIR - Rosato, Edoardo IR - Rosato E FIR - Foti, Rosario IR - Foti R FIR - Adler, Sabine IR - Adler S FIR - Dan, Diana IR - Dan D FIR - Wiesik-Szewczyk, Ewa IR - Wiesik-Szewczyk E FIR - Olesińska, Marzena IR - Olesińska M FIR - Kayser, Cristiane IR - Kayser C FIR - Fathi, Nihal IR - Fathi N FIR - Lefebvre, Paloma García de la Peña IR - Lefebvre PGP FIR - Imbert, Bernard IR - Imbert B EDAT- 2016/12/13 06:00 MHDA- 2017/08/22 06:00 CRDT- 2016/12/13 06:00 PHST- 2016/05/18 00:00 [received] PHST- 2016/08/22 00:00 [revised] PHST- 2016/10/09 00:00 [accepted] PHST- 2016/12/13 06:00 [pubmed] PHST- 2017/08/22 06:00 [medline] PHST- 2016/12/13 06:00 [entrez] AID - S0003-4967(24)19850-0 [pii] AID - 10.1136/annrheumdis-2016-209909 [doi] PST - ppublish SO - Ann Rheum Dis. 2017 Aug;76(8):1327-1339. doi: 10.1136/annrheumdis-2016-209909. Epub 2016 Nov 9. PMID- 19061610 OWN - NLM STAT- MEDLINE DCOM- 20090305 LR - 20081208 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 14 IP - 10 DP - 2008 Oct 15 TI - Inflammatory morphea in the context of Raynaud phenomenon. PG - 11 AB - A 37-year-old woman presented with a one-year history of asymptomatic, red-brown patches and plaques on the abdomen and extremities, in the context of Raynaud phenomenon and anti-centromere antibodies. Two biopsy specimens confirmed the diagnosis of inflammatory morphea. Even in the absence of initial symptoms to support systemic disease, patients presenting with morphea in the setting of Raynaud phenomenon or anti-centromere antibodies deserve close surveillance for the possibility of CREST syndrome and systemic sclerosis. FAU - Abbasi, Naheed AU - Abbasi N AD - Department of Dermatology, New York University, USA. FAU - Firoz, Bahar AU - Firoz B FAU - Bossenbroek, Nicole M AU - Bossenbroek NM FAU - Meehan, Shane A AU - Meehan SA FAU - Kamino, Hideko AU - Kamino H FAU - Franks, Andrew G Jr AU - Franks AG Jr LA - eng PT - Case Reports PT - Journal Article DEP - 20081015 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) SB - IM MH - Adult MH - Autoantibodies/blood/immunology MH - Autoantigens/immunology MH - CREST Syndrome/diagnosis MH - Centromere/immunology MH - Diagnosis, Differential MH - Female MH - Humans MH - Inflammation MH - Lyme Disease/diagnosis MH - Raynaud Disease/*complications/immunology MH - Scleroderma, Localized/complications/*diagnosis/immunology/pathology MH - Scleroderma, Systemic/diagnosis EDAT- 2008/12/09 09:00 MHDA- 2009/03/06 09:00 CRDT- 2008/12/09 09:00 PHST- 2008/12/09 09:00 [pubmed] PHST- 2009/03/06 09:00 [medline] PHST- 2008/12/09 09:00 [entrez] PST - epublish SO - Dermatol Online J. 2008 Oct 15;14(10):11. PMID- 16678008 OWN - NLM STAT- MEDLINE DCOM- 20060803 LR - 20131121 IS - 0891-5849 (Print) IS - 0891-5849 (Linking) VI - 40 IP - 10 DP - 2006 May 15 TI - Oxidative stress, microvascular dysfunction, and scleroderma: an association with potential therapeutic implications, a commentary on "Postocclusive reactive hyperemia inversely correlates with urinary 15-F2t-isoprostane levels in systemic sclerosis". PG - 1698-9 FAU - Chung, Cecilia P AU - Chung CP AD - Department of Medicine, Vanderbilt University School of Medicine, Rheumatology Division T-3219 MCN, 1161 21st Avenue, Nashville, TN, USA. c.chung@vanderbilt.edu FAU - Avalos, Ingrid AU - Avalos I FAU - Stein, C Michael AU - Stein CM LA - eng GR - HL04012/HL/NHLBI NIH HHS/United States GR - HL65082/HL/NHLBI NIH HHS/United States PT - Comment PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review DEP - 20060324 PL - United States TA - Free Radic Biol Med JT - Free radical biology & medicine JID - 8709159 RN - 27415-26-5 (8-epi-prostaglandin F2alpha) RN - B7IN85G1HY (Dinoprost) SB - IM CON - Free Radic Biol Med. 2006 May 15;40(10):1732-7. doi: 10.1016/j.freeradbiomed.2006.01.014. PMID: 16678012 MH - Animals MH - Dinoprost/*analogs & derivatives/urine MH - Humans MH - Hyperemia/*physiopathology MH - Microcirculation/*pathology MH - Oxidative Stress/*physiology MH - Raynaud Disease/pathology/physiopathology/urine MH - Scleroderma, Systemic/pathology/physiopathology/*urine MH - Skin/blood supply RF - 17 EDAT- 2006/05/09 09:00 MHDA- 2006/08/04 09:00 CRDT- 2006/05/09 09:00 PHST- 2006/05/09 09:00 [pubmed] PHST- 2006/08/04 09:00 [medline] PHST- 2006/05/09 09:00 [entrez] AID - S0891-5849(06)00160-2 [pii] AID - 10.1016/j.freeradbiomed.2006.03.001 [doi] PST - ppublish SO - Free Radic Biol Med. 2006 May 15;40(10):1698-9. doi: 10.1016/j.freeradbiomed.2006.03.001. Epub 2006 Mar 24. PMID- 25112102 OWN - NLM STAT- MEDLINE DCOM- 20140902 LR - 20140812 IS - 0255-2930 (Print) IS - 0255-2930 (Linking) VI - 34 IP - 6 DP - 2014 Jun TI - [Case of Raynaud disease]. PG - 598 FAU - Wang, Lin AU - Wang L LA - chi PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China TA - Zhongguo Zhen Jiu JT - Zhongguo zhen jiu = Chinese acupuncture & moxibustion JID - 8600658 SB - IM MH - Acupuncture Points MH - *Acupuncture Therapy MH - Female MH - Humans MH - Middle Aged MH - Raynaud Disease/*therapy EDAT- 2014/08/13 06:00 MHDA- 2014/09/03 06:00 CRDT- 2014/08/13 06:00 PHST- 2014/08/13 06:00 [entrez] PHST- 2014/08/13 06:00 [pubmed] PHST- 2014/09/03 06:00 [medline] PST - ppublish SO - Zhongguo Zhen Jiu. 2014 Jun;34(6):598. PMID- 23052723 OWN - NLM STAT- MEDLINE DCOM- 20140204 LR - 20131001 IS - 1940-1574 (Electronic) IS - 0003-3197 (Linking) VI - 64 IP - 8 DP - 2013 Nov TI - Three-grade classification of photoplethysmography for evaluating the effects of treatment in Raynaud phenomenon. PG - 609-13 LID - 10.1177/0003319712461930 [doi] AB - We assessed the utility of a 3-grade classification of photoplethysmography (PPG) with cold water provocation test in Raynaud phenomenon (RP). A total of 35 patients with RP and 38 healthy volunteers were recruited. In healthy volunteers, PPG was measured twice at intervals of 30 minutes for evaluating reproducibility of test. Percentage variation of the PPG amplitude in post- versus precold provocation was expressed on a 3-grade scale at 1, 3, and 5 minutes (reflection index: RI) in patients with RP. The improvement pattern of the PPG (IPPPG) was evaluated by summing the score differences from RI1 min to RI3 min and RI5 min. After a therapeutic period of 8 weeks with nifedipine, the decreases in posttherapeutic RI3 min and RI5 min were statistically significant (P < .05). Subgroup analysis of IPPPG showed statistical significance in patients with negative fluorescent antinuclear antibody (P < .05). The analysis of cold-provoked PPG waves demonstrated and quantified improvements in peripheral arterial circulation in RP. FAU - Yang, Shin-Seok AU - Yang SS AD - 1Department of Surgery, Division of Vascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. FAU - Park, Keun-Myoung AU - Park KM FAU - Kim, Young-Wook AU - Kim YW FAU - Kim, Dong-Ik AU - Kim DI LA - eng PT - Journal Article DEP - 20121010 PL - United States TA - Angiology JT - Angiology JID - 0203706 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear MH - Female MH - Humans MH - Male MH - Middle Aged MH - Monitoring, Physiologic MH - Photoplethysmography/*classification MH - Raynaud Disease/diagnosis/physiopathology/*therapy MH - Reproducibility of Results MH - Young Adult OTO - NOTNLM OT - Raynaud phenomenon OT - improvement pattern of PPG OT - photoplethysmography OT - reflex index EDAT- 2012/10/12 06:00 MHDA- 2014/02/05 06:00 CRDT- 2012/10/12 06:00 PHST- 2012/10/12 06:00 [entrez] PHST- 2012/10/12 06:00 [pubmed] PHST- 2014/02/05 06:00 [medline] AID - 0003319712461930 [pii] AID - 10.1177/0003319712461930 [doi] PST - ppublish SO - Angiology. 2013 Nov;64(8):609-13. doi: 10.1177/0003319712461930. Epub 2012 Oct 10. PMID- 16902805 OWN - NLM STAT- MEDLINE DCOM- 20070111 LR - 20181113 IS - 0017-8470 (Print) IS - 0017-8470 (Linking) VI - 57 IP - 9 DP - 2006 Sep TI - [Raynaud phenomenon in dermatology. Part 1: Pathophysiology and diagnostic approach]. PG - 819-28; quiz 829 AB - Raynaud phenomenon (RP) is characterized by recurrent spasms of small digital arterioles/arteries at fingers and toes, usually triggered by cold and emotional stress. Clinically a sudden pallor of individual digits is followed by reactive hyperemia, in severe cases also by cyanosis. One distinguishes between primary RP, i.e. RP without an underlying disease or drug intake, and secondary RP, which is causally related to an underlying disease or to intake of certain drugs (e.g. interferon, cisplatin). Primary RP is frequent (prevalence of about 13-20% in northern or central Europe), while secondary RP is rare, but the major presenting symptom for systemic sclerosis (SSc). Differential diagnosis includes cold-induced pallor, acute embolic events, paroxysmal hematoma of the finger or erythromelalgia. Vasoconstrictive mechanisms outweigh vasodilatory ones in endothelial cells and vascular smooth muscle. Although soluble mediators such as endothelin or certain prostaglandins have been exploited successfully for therapy, the extent of their involvement in the initial pathophysiology of RP is unclear. Secondary RP (associated with SSc) additionally features morphological alterations with compromise of the vessel lumen. As RP can result in severe discomfort and complications, timely diagnosis and treatment is essential. FAU - Sunderkötter, C AU - Sunderkötter C AD - Klinik und Poliklinik für Dermatologie, Universitätsklinikum Münster, Von-Esmarch-Strasse 58, 48129 Münster. cord.sunderkoetter@ukmuenster.de FAU - Riemekasten, G AU - Riemekasten G LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Raynaud-Phänomen in der Dermatologie. Teil 1: Pathophysiologie und Diagnostik. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 SB - IM MH - Humans MH - Practice Guidelines as Topic MH - Practice Patterns, Physicians' MH - Raynaud Disease/complications/*diagnosis/*physiopathology MH - Scleroderma, Systemic/complications/*diagnosis/*physiopathology RF - 33 EDAT- 2006/08/12 09:00 MHDA- 2007/01/12 09:00 CRDT- 2006/08/12 09:00 PHST- 2006/08/12 09:00 [pubmed] PHST- 2007/01/12 09:00 [medline] PHST- 2006/08/12 09:00 [entrez] AID - 10.1007/s00105-006-1192-x [doi] PST - ppublish SO - Hautarzt. 2006 Sep;57(9):819-28; quiz 829. doi: 10.1007/s00105-006-1192-x. PMID- 23419126 OWN - NLM STAT- MEDLINE DCOM- 20131119 LR - 20181202 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 11 IP - 4 DP - 2013 Apr TI - Influence of bosentan on fingertip rewarming in patients with systemic sclerosis. PG - 356-9 LID - 10.1111/ddg.12058 [doi] FAU - Autenrieth, Juliane AU - Autenrieth J FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Bert, Christoph AU - Bert C FAU - Worm, Margitta AU - Worm M LA - eng PT - Clinical Trial PT - Letter DEP - 20130219 PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antihypertensive Agents/administration & dosage MH - Bosentan MH - Female MH - Fingers/blood supply/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology/*physiopathology MH - Scleroderma, Systemic/complications/*drug therapy/*physiopathology MH - Skin/blood supply/drug effects/*physiopathology MH - Skin Temperature/*drug effects MH - Sulfonamides/*administration & dosage MH - Treatment Outcome EDAT- 2013/02/20 06:00 MHDA- 2013/11/20 06:00 CRDT- 2013/02/20 06:00 PHST- 2013/02/20 06:00 [entrez] PHST- 2013/02/20 06:00 [pubmed] PHST- 2013/11/20 06:00 [medline] AID - 10.1111/ddg.12058 [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2013 Apr;11(4):356-9. doi: 10.1111/ddg.12058. Epub 2013 Feb 19. PMID- 19412190 OWN - NLM STAT- MEDLINE DCOM- 20090716 LR - 20211020 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 5 IP - 5 DP - 2009 May TI - Therapy: A local approach to Raynaud phenomenon. PG - 246-7 LID - 10.1038/nrrheum.2009.64 [doi] AB - The current treatment options for Raynaud phenomenon are often ineffective and can have undesirable adverse effects. Topical, or transdermal, treatments that increase digital blood flow without systemic adverse effects are, therefore, an attractive option. A novel topical nitroglycerin formulation has shown promise in treating Raynaud attacks. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester and Salford Royal Hospital, Salford, Manchester, UK. ariane.herrick@manchester.ac.uk LA - eng PT - Comment PT - Journal Article PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - 0 (Emulsions) RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM CON - Arthritis Rheum. 2009 Mar;60(3):870-7. doi: 10.1002/art.24351. PMID: 19248104 MH - Administration, Topical MH - Emulsions MH - Fingers/blood supply MH - Hemodynamics/drug effects MH - Humans MH - Microcirculation/drug effects MH - Nitroglycerin/administration & dosage/*therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy/physiopathology MH - Regional Blood Flow/drug effects MH - Vasodilator Agents/administration & dosage/*therapeutic use EDAT- 2009/05/05 09:00 MHDA- 2009/07/17 09:00 CRDT- 2009/05/05 09:00 PHST- 2009/05/05 09:00 [entrez] PHST- 2009/05/05 09:00 [pubmed] PHST- 2009/07/17 09:00 [medline] AID - nrrheum.2009.64 [pii] AID - 10.1038/nrrheum.2009.64 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2009 May;5(5):246-7. doi: 10.1038/nrrheum.2009.64. PMID- 26733605 OWN - NLM STAT- MEDLINE DCOM- 20160622 LR - 20161107 IS - 1524-4539 (Electronic) IS - 0009-7322 (Linking) VI - 133 IP - 5 DP - 2016 Feb 2 TI - Relation of Nailfold Capillaries and Autoantibodies to Mortality in Patients With Raynaud Phenomenon. PG - 509-17 LID - 10.1161/CIRCULATIONAHA.115.017816 [doi] AB - BACKGROUND: In incipient Raynaud phenomenon, nailfold capillaroscopy and autoantibody tests are obtained to screen for an emerging connective tissue disease. Whether the presence of abnormal nailfold capillaries and autoantibodies are related to mortality in patients with incipient Raynaud phenomenon is not known. METHODS AND RESULTS: In 2958 consecutive patients (78% women, median age 45 years) with incipient Raynaud phenomenon without previously known connective tissue disease, nailfold capillaroscopy and laboratory tests for antinuclear antibodies (ANA) and ANA subsets were obtained at initial presentation. During a median follow-up period of 9.3 years, 227 women (9.9% of female patients) and 129 men (20% of male patients) with Raynaud phenomenon died. In comparison with a demographically matched standard population, survival was poorer in patients with Raynaud phenomenon (log-rank test P<0.0001). In patients with Raynaud phenomenon, mortality was higher in men than in women (P<0.0001, Cox proportional hazards model). In women, the presence of abnormal nailfold capillaries, ANA, and anti-Scl-70 antibodies were related to an increase in all-cause mortality. The conjoint presence of abnormal nailfold capillaries and autoantibodies was associated with the highest mortality rates. In men, abnormal nailfold capillaries, and ANA and ANA subsets, as well, were not related to survival. In both sexes, patients' age and serum creatinine were associated with mortality. CONCLUSIONS: In Raynaud phenomenon, male sex, age, and serum creatinine are related to mortality. Abnormal nailfold capillaries and autoantibodies are associated with an increase in all-cause mortality in female patients, but not in male patients with Raynaud phenomenon. CI - © 2016 American Heart Association, Inc. FAU - Mueller, Markus AU - Mueller M AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Gschwandtner, Michael E AU - Gschwandtner ME AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Gamper, Jutta AU - Gamper J AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Giurgea, Georgiana-Aura AU - Giurgea GA AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Charwat-Resl, Silvia AU - Charwat-Resl S AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Kiener, Hans P AU - Kiener HP AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Smolen, Josef S AU - Smolen JS AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Perkmann, Thomas AU - Perkmann T AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Koppensteiner, Renate AU - Koppensteiner R AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. FAU - Schlager, Oliver AU - Schlager O AD - From Division of Angiology, Department of Medicine II (M.M., M.E.G., G.-A.G., S.C.R., R.K., O.S.), Center of Medical Statistics, Informatics and Intelligent Systems (J.G.), Division of Rheumatology, Department of Medicine III (H.P.K., J.S.S.), and Department of Laboratory Medicine (T.P.), Medical University of Vienna, Austria. oliver.schlager@meduniwien.ac.at. LA - eng PT - Journal Article DEP - 20160105 PL - United States TA - Circulation JT - Circulation JID - 0147763 RN - 0 (Autoantibodies) SB - IM CIN - Circulation. 2016 May 31;133(22):e668. doi: 10.1161/CIRCULATIONAHA.116.021987. PMID: 27245654 CIN - Circulation. 2016 May 31;133(22):e669. doi: 10.1161/CIRCULATIONAHA.116.022853. PMID: 27245655 MH - Adult MH - Autoantibodies/*blood MH - *Capillaries/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy/methods/*mortality MH - Middle Aged MH - Mortality/trends MH - Prospective Studies MH - Raynaud Disease/*blood/diagnosis/*mortality OTO - NOTNLM OT - allergy and immunology OT - capillaries OT - epidemiology OT - microcirculation OT - prognosis EDAT- 2016/01/07 06:00 MHDA- 2016/06/23 06:00 CRDT- 2016/01/07 06:00 PHST- 2015/06/02 00:00 [received] PHST- 2015/12/21 00:00 [accepted] PHST- 2016/01/07 06:00 [entrez] PHST- 2016/01/07 06:00 [pubmed] PHST- 2016/06/23 06:00 [medline] AID - CIRCULATIONAHA.115.017816 [pii] AID - 10.1161/CIRCULATIONAHA.115.017816 [doi] PST - ppublish SO - Circulation. 2016 Feb 2;133(5):509-17. doi: 10.1161/CIRCULATIONAHA.115.017816. Epub 2016 Jan 5. PMID- 27829111 OWN - NLM STAT- MEDLINE DCOM- 20180629 LR - 20260127 IS - 2168-6084 (Electronic) IS - 2168-6068 (Linking) VI - 152 IP - 11 DP - 2016 Nov 1 TI - The Legacy of Maurice Raynaud. PG - 1253 LID - 10.1001/jamadermatol.2016.0010 [doi] FAU - Prabhu, Arpan V AU - Prabhu AV AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Oddis, Chester V AU - Oddis CV AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. LA - eng PT - Biography PT - Historical Article PT - Journal Article PL - United States TA - JAMA Dermatol JT - JAMA dermatology JID - 101589530 SB - IM MH - France MH - History, 19th Century MH - Raynaud Disease/*history PS - Raynaud M FPS - Raynaud, Maurice EDAT- 2016/11/10 06:00 MHDA- 2018/06/30 06:00 CRDT- 2016/11/10 06:00 PHST- 2016/11/10 06:00 [entrez] PHST- 2016/11/10 06:00 [pubmed] PHST- 2018/06/30 06:00 [medline] AID - 2579118 [pii] AID - 10.1001/jamadermatol.2016.0010 [doi] PST - ppublish SO - JAMA Dermatol. 2016 Nov 1;152(11):1253. doi: 10.1001/jamadermatol.2016.0010. PMID- 21467065 OWN - NLM STAT- MEDLINE DCOM- 20110607 LR - 20170519 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 139 IP - 4 DP - 2011 Apr TI - A 77-year-old woman with dyspnea and raynaud phenomenon. PG - 958-961 LID - S0012-3692(11)60201-8 [pii] LID - 10.1378/chest.10-2405 [doi] FAU - Wang, Janice AU - Wang J AD - Department of Pulmonary and Critical Care Medicine, North Shore/LIJ, New Hyde Park, NY. Electronic address: jwang@nshs.edu. FAU - Sadoughi, Ali AU - Sadoughi A AD - Department of Pulmonary and Critical Care Medicine, North Shore/LIJ, New Hyde Park, NY. FAU - Dedopoulos, Sophy AU - Dedopoulos S AD - Department of Pulmonary and Critical Care Medicine, North Shore/LIJ, New Hyde Park, NY. FAU - Talwar, Arunabh AU - Talwar A AD - Department of Pulmonary and Critical Care Medicine, North Shore/LIJ, New Hyde Park, NY. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 SB - IM MH - Aged MH - Diagnosis, Differential MH - Dyspnea/diagnosis/*etiology MH - Echocardiography MH - Female MH - Humans MH - Hypertension, Pulmonary/*complications/diagnosis MH - Raynaud Disease/*complications/diagnosis MH - Scleroderma, Systemic/*complications/diagnosis MH - Tomography, X-Ray Computed EDAT- 2011/04/07 06:00 MHDA- 2011/06/08 06:00 CRDT- 2011/04/07 06:00 PHST- 2011/04/07 06:00 [entrez] PHST- 2011/04/07 06:00 [pubmed] PHST- 2011/06/08 06:00 [medline] AID - S0012-3692(11)60201-8 [pii] AID - 10.1378/chest.10-2405 [doi] PST - ppublish SO - Chest. 2011 Apr;139(4):958-961. doi: 10.1378/chest.10-2405. PMID- 16964476 OWN - NLM STAT- MEDLINE DCOM- 20070117 LR - 20181113 IS - 0017-8470 (Print) IS - 0017-8470 (Linking) VI - 57 IP - 10 DP - 2006 Oct TI - [Raynaud phenomenon in dermatology : Part 2: therapy]. PG - 927-38; quiz 941 AB - While primary Raynaud phenomenon (RP) only rarely leads to complications, secondary RP when associated with systemic sclerosis (SSc) frequently results in necrosis, ulcers or even gangrene. Therefore timely therapeutic intervention is required. Management of RP includes physical therapy, avoidance of triggers, and a variety of medications, depending on the disease severity. On the basis of published studies and of our own experience we suggest: a) for primary and uncomplicated secondary RP, calcium channel blockers in adequate doses; b) for SSc-associated secondary RP with hypertension, cardial or renal involvement, drugs which interfere with angiotensin; c) in case of concomitant depression, exploitation of the vasodilatory effects of serotonin reuptake inhibitors, d) in presence of ulcers or marked trophic alterations, intravenous administration of iloprost, e) for recalcitrant cases and endangered digits, sildenafil, f) for prevention of severe relapses, the endothelin-receptor antagonist bosentan. The drugs mentioned in c-f are not approved for RP and used off-label. In patients with secondary SSc-associated RP, timely therapy can reduce the percentage of complications such as ulcers or amputations. In addition, timely treatment of RP possibly retards ensuing fibrotic processes. FAU - Sunderkötter, C AU - Sunderkötter C AD - Klinik und Poliklinik für Dermatologie, Universitätsklinik Münster, Von-Esmarch-Strasse 58, 48129, Münster, Deutschland. cord.sunderkoetter@ukmuenster.de FAU - Riemekasten, G AU - Riemekasten G LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Raynaud-Phänomen in der Dermatologie: Teil 2: Therapie. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 SB - IM MH - Humans MH - Practice Guidelines as Topic MH - Practice Patterns, Physicians' MH - Raynaud Disease/complications/*diagnosis/*therapy MH - Scleroderma, Systemic/complications/*diagnosis/*therapy RF - 64 EDAT- 2006/09/12 09:00 MHDA- 2007/01/18 09:00 CRDT- 2006/09/12 09:00 PHST- 2006/09/12 09:00 [pubmed] PHST- 2007/01/18 09:00 [medline] PHST- 2006/09/12 09:00 [entrez] AID - 10.1007/s00105-006-1218-4 [doi] PST - ppublish SO - Hautarzt. 2006 Oct;57(10):927-38; quiz 941. doi: 10.1007/s00105-006-1218-4. PMID- 19350771 OWN - NLM STAT- MEDLINE DCOM- 20090409 LR - 20090408 IS - 0023-7205 (Print) IS - 0023-7205 (Linking) VI - 106 IP - 7 DP - 2009 Feb 11-17 TI - [Vibration injuries]. PG - 439-42 FAU - Rehfisch, Pia AU - Rehfisch P AD - Akademiska sjukhuset, Uppsala. pia.rehfisch@medsci.uu.se FAU - Wålinder, Robert AU - Wålinder R LA - swe PT - Journal Article TT - Vibrationsskador. PL - Sweden TA - Lakartidningen JT - Lakartidningen JID - 0027707 SB - IM MH - Diagnosis, Differential MH - Humans MH - Low Back Pain/diagnosis/*etiology MH - Musculoskeletal Diseases/diagnosis/*etiology MH - Occupational Diseases/diagnosis/*etiology MH - Occupational Exposure/*adverse effects MH - Polyneuropathies/diagnosis/etiology MH - Prognosis MH - Raynaud Disease/diagnosis/*etiology MH - Vibration/*adverse effects EDAT- 2009/04/09 09:00 MHDA- 2009/04/10 09:00 CRDT- 2009/04/09 09:00 PHST- 2009/04/09 09:00 [entrez] PHST- 2009/04/09 09:00 [pubmed] PHST- 2009/04/10 09:00 [medline] PST - ppublish SO - Lakartidningen. 2009 Feb 11-17;106(7):439-42. PMID- 18211421 OWN - NLM STAT- MEDLINE DCOM- 20080327 LR - 20080123 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 22 IP - 2 DP - 2008 Feb TI - The hand of the wine grower: hypothenar hammer syndrome. PG - 238-9 LID - 10.1111/j.1468-3083.2007.02293.x [doi] FAU - Doutre, M S AU - Doutre MS FAU - Orlandini, V AU - Orlandini V FAU - Bijou, B AU - Bijou B FAU - Beylot-Barry, M AU - Beylot-Barry M LA - eng PT - Case Reports PT - Letter PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 SB - IM MH - Adult MH - Agricultural Workers' Diseases/*diagnosis/pathology MH - Female MH - Hand/*pathology MH - Hemorrhage MH - Humans MH - Nails/pathology MH - Necrosis MH - Occupational Diseases/diagnosis/pathology MH - Pain/*diagnosis/pathology MH - Raynaud Disease/*diagnosis/pathology MH - Skin Ulcer/*diagnosis/pathology MH - Syndrome MH - Thrombosis MH - Ulnar Artery/pathology MH - Wine EDAT- 2008/01/24 09:00 MHDA- 2008/03/28 09:00 CRDT- 2008/01/24 09:00 PHST- 2008/01/24 09:00 [pubmed] PHST- 2008/03/28 09:00 [medline] PHST- 2008/01/24 09:00 [entrez] AID - JDV2293 [pii] AID - 10.1111/j.1468-3083.2007.02293.x [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2008 Feb;22(2):238-9. doi: 10.1111/j.1468-3083.2007.02293.x. PMID- 17599734 OWN - NLM STAT- MEDLINE DCOM- 20070906 LR - 20131121 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 7 DP - 2007 Jul TI - Clinical images: iloprost-induced vascular remodeling. PG - 2243 FAU - Pyrpasopoulou, Athina AU - Pyrpasopoulou A AD - Hippokration General Hospital, Thessaloniki, Greece. FAU - Aslanidis, Spyros AU - Aslanidis S LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Capillaries/drug effects/*pathology MH - Female MH - Hemorrhage/pathology MH - Humans MH - Iloprost/*therapeutic use MH - Nails/blood supply MH - Neovascularization, Pathologic MH - Raynaud Disease/*drug therapy/pathology MH - Treatment Outcome MH - Vasodilator Agents/therapeutic use EDAT- 2007/06/30 09:00 MHDA- 2007/09/07 09:00 CRDT- 2007/06/30 09:00 PHST- 2007/06/30 09:00 [pubmed] PHST- 2007/09/07 09:00 [medline] PHST- 2007/06/30 09:00 [entrez] AID - 10.1002/art.22757 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Jul;56(7):2243. doi: 10.1002/art.22757. PMID- 23576170 OWN - NLM STAT- MEDLINE DCOM- 20131107 LR - 20211021 IS - 1432-1173 (Electronic) IS - 0017-8470 (Linking) VI - 64 IP - 4 DP - 2013 Apr TI - [Systemic scleroderma]. PG - 299-310; quiz 311-2 LID - 10.1007/s00105-013-2537-x [doi] AB - Systemic scleroderma is a chronic autoimmune disease affecting the skin, internal organs and the musculoskeletal system. The presence of Raynaud phenomenon, anti-nuclear antibodies and pathologic capillaroscopy are early signs of the disease. Limited cutaneous SSc, diffuse cutaneous SSc and SSc-overlap syndromes are the main clinical subtypes. Multidisciplinary care is mandatory. Follow-up examinations should be performed at least annually in order to recognize in a timely fashion treatable organ involvement such as pulmonary arterial hypertension. Besides symptomatic treatment of organ involvement, immunosuppressive therapy is indicated for a progressive inflammatory course. FAU - Hunzelmann, N AU - Hunzelmann N AD - Klinik und Poliklinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln. Nico.Hunzelmann@uni-koeln.de LA - ger PT - English Abstract PT - Journal Article TT - Systemische Sklerodermie. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 RN - 0 (Immunosuppressive Agents) SB - IM MH - Diagnosis, Differential MH - Humans MH - Hypertension, Pulmonary/*diagnosis/*drug therapy/etiology MH - Immunosuppressive Agents/*therapeutic use MH - Raynaud Disease/complications/*diagnosis/*drug therapy MH - Scleroderma, Systemic/complications/*diagnosis/*drug therapy EDAT- 2013/04/12 06:00 MHDA- 2013/11/08 06:00 CRDT- 2013/04/12 06:00 PHST- 2013/04/12 06:00 [entrez] PHST- 2013/04/12 06:00 [pubmed] PHST- 2013/11/08 06:00 [medline] AID - 10.1007/s00105-013-2537-x [doi] PST - ppublish SO - Hautarzt. 2013 Apr;64(4):299-310; quiz 311-2. doi: 10.1007/s00105-013-2537-x. PMID- 20703220 OWN - NLM STAT- MEDLINE DCOM- 20110118 LR - 20220408 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 6 IP - 10 DP - 2010 Oct TI - Assessing microvascular changes in systemic sclerosis diagnosis and management. PG - 578-87 LID - 10.1038/nrrheum.2010.104 [doi] AB - Microvascular damage and dysfunction represent the earliest morphological and functional markers of systemic sclerosis (SSc), a progressive connective tissue disease characterized by vascular abnormalities and diffuse fibrosis in the skin and internal organs. These early microvascular changes are clinically mirrored by Raynaud phenomenon, which can be primary (idiopathic) or secondary to several different conditions including SSc. Morphological and functional assessment of the cutaneous microvasculature have crucial implications for diagnosis, prognosis and therapy in SSc and secondary Raynaud phenomenon. Most importantly, imaging with nailfold videocapillaroscopy (NVC) enables the early differentiation between primary and secondary Raynaud phenomenon by identifying morphological patterns specific to various stages of SSc ('early', 'active' and 'late' patterns); the inclusion of these NVC patterns could increase the sensitivity of classification criteria for SSc. Findings on NVC are also markers of SSc severity and progression, as reduced capillary density has been associated with a high risk of developing digital skin ulcers and pulmonary arterial hypertension. Laser Doppler imaging and thermal imaging demonstrate the dysfunctional cutaneous blood flow in response to cold stimuli. Therapies targeting underlying vascular disease in SSc have been successfully designed to improve the symptoms of Raynaud phenomenon and to reduce ischemic injury to involved organs, and NVC patterns have been found to improve following targeted therapy; however, treatment of later fibrosis remains a challenge. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genova, 6-16132 Genova, Italy. mcutolo@unige.it FAU - Sulli, Alberto AU - Sulli A FAU - Smith, Vanessa AU - Smith V LA - eng PT - Journal Article PT - Review DEP - 20100810 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Laser-Doppler Flowmetry MH - Microscopic Angioscopy MH - Microvessels/*pathology MH - Nails/blood supply MH - Prognosis MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*diagnosis/drug therapy/physiopathology MH - Scleroderma, Systemic/*diagnosis/drug therapy/physiopathology MH - Skin/blood supply/*pathology MH - Thermography/methods MH - Vasodilator Agents/therapeutic use EDAT- 2010/08/13 06:00 MHDA- 2011/01/19 06:00 CRDT- 2010/08/13 06:00 PHST- 2010/08/13 06:00 [entrez] PHST- 2010/08/13 06:00 [pubmed] PHST- 2011/01/19 06:00 [medline] AID - nrrheum.2010.104 [pii] AID - 10.1038/nrrheum.2010.104 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2010 Oct;6(10):578-87. doi: 10.1038/nrrheum.2010.104. Epub 2010 Aug 10. PMID- 18627700 OWN - NLM STAT- MEDLINE DCOM- 20081216 LR - 20170214 IS - 1203-4754 (Print) IS - 1203-4754 (Linking) VI - 12 IP - 4 DP - 2008 Jul-Aug TI - Improvement in both Raynaud disease and hyperhidrosis in response to botulinum toxin type A treatment. PG - 189-93 AB - BACKGROUND: A patient with concurrent Raynaud disease presented for hyperhidrosis of the axillae and palms. After a positive response to botulinum toxin type A (BoNTA) for axillary hyperhidrosis, she returned requesting palmar treatment. OBJECTIVES: Our goal was to investigate the effect of BoNTA on Raynaud disease in concurrent hyperhidrosis with respect to color change, swelling, and digital pain. METHODS: The patient had treatment with 100 units of BoNTA to one hand at first, with the other being a negative control, followed by treatment of the second hand 1 week later. RESULTS: After the injection into the first palm, the patient demonstrated an 85% reduction in palmar hyperhidrosis and a significant improvement in her Raynaud symptoms. Specifically, the BoNTA-treated hand had reduced swelling, color change, and pain, whereas the untreated control hand remained affected. After the second hand was treated, it, too, demonstrated the same positive results. CONCLUSIONS: Our case report of concurrent Raynaud disease and palmar hyperhidrosis shows significant improvement in both conditions to BoNTA administration. The physiology is multifactorial and relates to BoNTA's effect on acetylcholine, noradrenaline, substance P, calcitonin gene-related peptide, and glutamate release from nerve terminals. These results present an encouraging novel treatment option in dermatology for patients with Raynaud disease. FAU - Kossintseva, Iràn AU - Kossintseva I AD - Division of Dermatology, University of Alberta, Edmonton, AB FAU - Barankin, Benjamin AU - Barankin B LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Cutan Med Surg JT - Journal of cutaneous medicine and surgery JID - 9614685 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Humans MH - Hyperhidrosis/*drug therapy MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*drug therapy EDAT- 2008/07/17 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/07/17 09:00 PHST- 2008/07/17 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/07/17 09:00 [entrez] AID - 10.2310/7750.2008.07044 [doi] PST - ppublish SO - J Cutan Med Surg. 2008 Jul-Aug;12(4):189-93. doi: 10.2310/7750.2008.07044. PMID- 19907728 OWN - NLM STAT- MEDLINE DCOM- 20101122 LR - 20210227 IS - 1936-7163 (Electronic) IS - 0033-6572 (Linking) VI - 41 IP - 1 DP - 2010 Jan TI - Intraoral telangiectasias associated with Raynaud disease: a report of two cases. PG - 17-20 AB - The finding of intraoral telangiectasias in two patients previously diagnosed with Raynaud disease is reported. Neither patient exhibited any other feature of CREST syndrome, namely, calcinosis cutis, esophageal dysfunction, or sclerodactyly. To the authors' knowledge, this is the first time intraoral telangiectasias has been reported in conjunction with Raynaud disease in the absence of any other features of CREST syndrome. FAU - Philipone, Elizabeth AU - Philipone E AD - Clinical Dentistry, Division of Oral and Maxillofactal Pathology, Columbia University College of Dental Medicine, New York, New York 10032, USA. FAU - Yoon, Angela J AU - Yoon AJ FAU - Zegarelli, David AU - Zegarelli D LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Quintessence Int JT - Quintessence international (Berlin, Germany : 1985) JID - 0342677 MH - Aged MH - Burning Mouth Syndrome/*etiology MH - CREST Syndrome/*pathology MH - Female MH - Humans MH - Middle Aged MH - Mouth Diseases/*etiology/pathology MH - Mouth Mucosa/blood supply MH - Palate, Hard/blood supply MH - Raynaud Disease/*complications MH - Telangiectasis/*complications/pathology MH - Tongue/blood supply/pathology EDAT- 2009/11/13 06:00 MHDA- 2010/12/14 06:00 CRDT- 2009/11/13 06:00 PHST- 2009/11/13 06:00 [entrez] PHST- 2009/11/13 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 840202 [pii] PST - ppublish SO - Quintessence Int. 2010 Jan;41(1):17-20. PMID- 22782008 OWN - NLM STAT- MEDLINE DCOM- 20121213 LR - 20220408 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 8 IP - 8 DP - 2012 Aug TI - The pathogenesis, diagnosis and treatment of Raynaud phenomenon. PG - 469-79 LID - 10.1038/nrrheum.2012.96 [doi] AB - The past 10 years have seen the publication of results from several multicentre clinical trials in primary and systemic sclerosis (SSc)-related Raynaud phenomenon. The publication of these studies has occurred as a result of new insights into the pathogenesis of Raynaud phenomenon, which are directing new treatment approaches, and increased international collaboration between clinicians and scientists. Although the pathogenesis of Raynaud phenomenon is complex, abnormalities of the blood vessel wall, of neural control mechanisms and of intravascular (circulating) factors are known to interact and contribute. Key players relevant in drug development include nitric oxide, endothelin-1, alpha adrenergic receptor activation, abnormal signal transduction in vascular smooth muscle, oxidative stress and platelet activation. The main advances in diagnosis have been a clearer understanding of autoantibodies and of abnormal nailfold capillary patterns as independent predictors of SSc, and widespread use and increased availability of capillaroscopy. The ultimate aim is to translate the advances made in the pathophysiology and early diagnosis into development of treatments to prevent and reverse digital vascular dysfunction and injury. This Review provides an update of the pathogenesis, diagnosis and treatment of Raynaud phenomenon. Current and future treatment approaches are discussed, and some key unanswered questions are highlighted. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester, Manchester Academic Health Science Centre, Salford Royal Hospital, Salford M6 8HD, UK. ariane.herrick@manchester.ac.uk LA - eng PT - Journal Article PT - Review DEP - 20120710 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - Blood Vessels/pathology MH - Capillaries/pathology MH - Connective Tissue Diseases/complications/diagnosis/therapy MH - Female MH - Humans MH - Male MH - Nails/blood supply MH - *Raynaud Disease/diagnosis/etiology/therapy MH - Sex Factors EDAT- 2012/07/12 06:00 MHDA- 2012/12/14 06:00 CRDT- 2012/07/12 06:00 PHST- 2012/07/12 06:00 [entrez] PHST- 2012/07/12 06:00 [pubmed] PHST- 2012/12/14 06:00 [medline] AID - nrrheum.2012.96 [pii] AID - 10.1038/nrrheum.2012.96 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2012 Aug;8(8):469-79. doi: 10.1038/nrrheum.2012.96. Epub 2012 Jul 10. PMID- 32568390 OWN - NLM STAT- MEDLINE DCOM- 20201016 LR - 20220812 IS - 1538-3598 (Electronic) IS - 0098-7484 (Print) IS - 0098-7484 (Linking) VI - 324 IP - 4 DP - 2020 Jul 28 TI - Digit Ulcerations in a Young Woman. PG - 385-386 LID - 10.1001/jama.2020.6084 [doi] FAU - Varghese, Bibin AU - Varghese B AD - Department of Medicine, The Johns Hopkins Hospital, Baltimore, Maryland. FAU - Chew, Erin AU - Chew E AD - Department of Medicine, The Johns Hopkins Hospital, Baltimore, Maryland. FAU - Konig, Maximilian F AU - Konig MF AD - Division of Rheumatology, Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland. LA - eng GR - T32 AR048522/AR/NIAMS NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - JAMA JT - JAMA JID - 7501160 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*complications/drug therapy MH - Nifedipine/therapeutic use MH - Photography MH - Raynaud Disease/diagnosis/drug therapy/*etiology MH - Scleroderma, Systemic/*complications/diagnosis MH - Skin Ulcer/diagnosis/drug therapy/*etiology MH - Vasodilator Agents/therapeutic use MH - Young Adult PMC - PMC9365669 MID - NIHMS1824312 COIS- Conflict of Interest Disclosures: None reported. EDAT- 2020/06/23 06:00 MHDA- 2020/10/21 06:00 PMCR- 2022/08/11 CRDT- 2020/06/23 06:00 PHST- 2020/06/23 06:00 [pubmed] PHST- 2020/10/21 06:00 [medline] PHST- 2020/06/23 06:00 [entrez] PHST- 2022/08/11 00:00 [pmc-release] AID - 2767679 [pii] AID - 10.1001/jama.2020.6084 [doi] PST - ppublish SO - JAMA. 2020 Jul 28;324(4):385-386. doi: 10.1001/jama.2020.6084. PMID- 33998674 OWN - NLM STAT- MEDLINE DCOM- 20210617 LR - 20260127 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 5 IP - 5 DP - 2021 May 17 TI - Vasodilators for primary Raynaud's phenomenon. PG - CD006687 LID - 10.1002/14651858.CD006687.pub4 [doi] LID - CD006687 AB - BACKGROUND: Numerous agents have been suggested for the symptomatic treatment of primary Raynaud's phenomenon. Apart from calcium channel blockers, which are considered to be the drugs of choice, evidence of the effects of alternative pharmacological treatments is limited. This is an update of a review first published in 2008. OBJECTIVES: To assess the effects of drugs with vasodilator effects on primary Raynaud's phenomenon as determined by frequency, severity, and duration of vasospastic attacks; quality of life; adverse events; and Raynauds Condition Score. SEARCH METHODS: The Cochrane Vascular Information Specialist searched the Cochrane Vascular Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL databases, and the World Health Organization International Clinical Trials Registry Platform and the ClinicalTrials.gov trial register to November 16, 2020. SELECTION CRITERIA: We included randomized controlled trials evaluating effects of oral, intravenous, and topical formulations of any drug with vasodilator effects on subjective symptoms, severity scores, and radiological outcomes in primary Raynaud's phenomenon. Treatment with calcium channel blockers was not assessed in this review, nor were these agents compared. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies for inclusion, assessed studies using the Cochrane "Risk of bias" tool, and extracted study data. Outcomes of interest included frequency, severity, and duration of attacks; quality of life (QoL); adverse events (AEs); and the Raynaud Condition Score (RCS). We assessed the certainty of the evidence using GRADE. MAIN RESULTS: We identified seven new studies for this update. In total, we included 15 studies involving 635 participants. These studies compared different vasodilators to placebo. Individual studies used different methods and measures to report different outcomes. Angiotensin-converting enzyme (ACE) inhibitors Combining data from three studies revealed a possible small increase in the frequency of attacks per week after treatment (captopril or enalapril) compared to placebo (mean difference [MD] 0.79, 95% confidence interval [CI] 0.43 to 1.17; low-certainty evidence). There was no evidence of a difference between groups in severity of attacks (MD -0.17, 95% CI -4.66 to 4.31; 34 participants, 2 studies; low-certainty evidence); duration of attacks (MD 0.54, 95% CI -2.42 to 1.34; 14 participants, 1 study; low-certainty evidence); or AEs (risk ratio [RR] 1.35, 95% CI 0.67 to 2.73; 46 participants, 3 studies; low-certainty evidence). QoL and RCS were not reported. Alpha blockers Two studies used alpha blockers (buflomedil or moxisylyte). We were unable to combine data due to the way results were presented. Buflomedil probably reduced the frequency of attacks compared to placebo (MD -8.82, 95% CI -11.04 to -6.60; 31 participants, 1 study; moderate-certainty evidence) and may improve severity scores (MD -0.41, 95% CI -0.62 to -0.30; moderate-certainty evidence). With moxisylyte, investigators reported fewer attacks (P < 0.02), less severe symptoms (P < 0.01), and shorter duration of attacks, but the clinical relevance of these results is unclear. No evidence of a difference in AEs between buflomedil and placebo groups was noted (RR 1.41, 95% CI 0.27 to 7.28; 31 participants, 1 study; moderate-certainty evidence). More AEs were observed in participants in the moxisylyte group than in the placebo group. Prostaglandin/prostacyclin analogues One study compared beraprost versus placebo. There was no evidence of benefit for frequency (MD 2.00, 95% CI -0.35 to 4.35; 118 participants, low-certainty evidence) or severity (MD -0.06, 95% CI -0.34 to 0.22; 118 participants, low-certainty evidence) of attacks. Overall, more AEs were noted in the beraprost group (RR 1.59, 95% CI 1.05 to 2.42; 125 participants; low-certainty evidence). This study did not report on duration of attacks, QoL, or RCS. Thromboxane synthase inhibitors One study compared a thromboxane synthase inhibitor (dazoxiben) versus placebo. There was no evidence of benefit for frequency of attacks (MD 0.8, 95% CI -1.81 to 3.41; 6 participants; very low-certainty evidence). Adverse events were not reported in subgroup analyses of participants with primary Raynaud's phenomenon, and the study did not report on duration of attacks, severity of symptoms, QoL, or RCS. Selective serotonin reuptake inhibitors One study compared ketanserin with placebo. There may be a slight reduction in the number of attacks per week with ketanserin compared to placebo (MD -14.0, 95% CI -27.72 to -0.28; 41 participants; very low-certainty evidence) and reduced severity score (MD -133.00, 95% CI -162.40 to -103.60; 41 participants; very low-certainty evidence). There was no evidence that ketanserin reduced the duration of attacks (MD -4.00, 95% CI -14.82 to 6.82; 41 participants; very low-certainty evidence), or that AEs were increased in either group (RR 1.54, 95% CI 0.89 to 2.65; 41 participants; very low-certainty evidence). This study did not report on QoL or RCS. Nitrate/nitrate derivatives Four studies compared topical treatments of nitroglycerin or glyceryl trinitrate versus placebo, each reporting on limited outcomes. Meta-analysis demonstrated no evidence of effect on frequency of attacks per week (MD -1.57, 95% CI -4.31 to 1.17; 86 participants, 2 studies; very low-certainty evidence). We were unable to pool any data for the remaining outcomes. Phosphodiesterase inhibitors Three studies compared phosphodiesterase inhibitors (vardenafil, cilostazol or PF-00489791) to an equivalent placebo. Results showed no evidence of a difference in frequency of attacks (standardized MD [SMD] -0.05, 95% CI -6.71 to 6.61; 111 participants, 2 studies; low-certainty evidence), severity of attacks (MD -0.03, 95% CI -1.04 to 0.97; 111 participants, 2 studies; very low-certainty evidence), duration of attacks (MD -1.60, 95% CI -7.51 to 4.31; 73 participants, 1 study; low-certainty evidence), or RCS (SMD -0.8, 95% CI -1.74 to 0.13; 79 participants, 2 studies; low-certainty evidence). Study authors reported that 35% of participants on cilostazol complained of headaches, which were not reported in the placebo group. PF-00489791 caused 34 of 54 participants to experience AEs versus 43 of 102 participants receiving placebo (RR 1.49). Headache was most common, affecting 14 participants (PF-00489791) versus nine participants (placebo). AUTHORS' CONCLUSIONS: The included studies investigated several different vasodilators (topical and oral) for treatment of primary Raynaud's phenomenon. Small sample sizes, limited data, and variability in outcome reporting yielded evidence of very low to moderate certainty. Evidence is insufficient to support the use of vasodilators and suggests that vasodilator use may even worsen disease. CI - Copyright © 2021 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. FAU - Su, Kevin Yc AU - Su KY AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. FAU - Sharma, Meghna AU - Sharma M AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. AD - School of Medicine, Bond University, Robina, Australia. FAU - Kim, Hyunjun Jonathan AU - Kim HJ AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. FAU - Kaganov, Elizabeth AU - Kaganov E AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. AD - School of Medicine, Bond University, Robina, Australia. FAU - Hughes, Ian AU - Hughes I AD - Office for Research Governance and Development, Gold Coast University Hospital, Southport, Australia. AD - School of Medicine, The University of Queensland, St Lucia, Australia. FAU - Abdeen, Mohamed Hashim AU - Abdeen MH AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. FAU - Ng, Jennifer Hwee Kwoon AU - Ng JHK AD - Department of Rheumatology, Gold Coast University Hospital, Southport, Australia. AD - School of Medicine, Griffith University, Gold Coast Campus, Southport, Australia. LA - eng SI - ClinicalTrials.gov/NCT01090492 GR - ETM/442/CSO_/Chief Scientist Office/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20210517 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Placebos) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - EC 5.3.99.5 (Thromboxane-A Synthase) RN - 0 (Vasodilator Agents) SB - IM UOF - Cochrane Database Syst Rev. 2012 Jul 11;(7):CD006687. doi: 10.1002/14651858.CD006687.pub3. PMID: 22786498 CIN - Int J Rheum Dis. 2022 Feb;25(2):232-235. doi: 10.1111/1756-185X.14243. PMID: 34845840 MH - Administration, Oral MH - Administration, Topical MH - Adrenergic alpha-Antagonists/administration & dosage MH - Angiotensin-Converting Enzyme Inhibitors/administration & dosage/adverse effects MH - Bias MH - Humans MH - Phosphodiesterase Inhibitors/administration & dosage MH - Placebos/therapeutic use MH - Quality of Life MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Thromboxane-A Synthase/antagonists & inhibitors MH - Vasodilator Agents/*administration & dosage PMC - PMC8127538 COIS- KYS: none known.
MS: none known.
HK: none known.
EK: none known.
IH: has declared affiliation to Gold Coast Health through guidelines for treatment of rheumatoid‐type diseases.
MHA: none known
JHKN: declares that her Institution received funds for clinical trials (Abbvie, Pfizer, MSD, Roche, and Paradigm). None of these trials are related to Raynaud's phenomenon. EDAT- 2021/05/18 06:00 MHDA- 2021/06/22 06:00 PMCR- 2022/05/17 CRDT- 2021/05/17 08:54 PHST- 2021/05/17 08:54 [entrez] PHST- 2021/05/18 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2022/05/17 00:00 [pmc-release] AID - CD006687.pub4 [pii] AID - 10.1002/14651858.CD006687.pub4 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2021 May 17;5(5):CD006687. doi: 10.1002/14651858.CD006687.pub4. PMID- 32356950 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 84 IP - 6S Suppl 5 DP - 2020 Jun TI - The Use of Ultrasound Guidance for the Treatment of Raynaud Disease of the Hand With Botulinum Toxin. PG - S386-S388 LID - 10.1097/SAP.0000000000002303 [doi] AB - BACKGROUND: This study sought to examine the feasibility and effectiveness of the use of ultrasound (US) as a clinical adjunct in the delivery of botulinum toxin for the treatment of Raynaud disease of the hand. METHODS: One cadaver hand was injected with a colored simulation material using the current traditional surface anatomy approach, whereas an additional cadaver hand was injected using US guidance. The hands were dissected and areas of distribution of the simulation material were evaluated. Five patients diagnosed with Raynaud disease were treated with botulinum toxin using US guidance and data using the Patient-Reported Outcomes Measurement Information System Upper Extremity were collected. RESULTS: The cadaver hand injected with a colored simulation material using US guidance demonstrated significantly reduced surface area of muscle infiltrated compared with the cadaver hand injected using the traditional surface anatomy approach (16 cm vs 37 cm, P < 0.001).All 5 patients treated with botulinum toxin using US guidance reported significant improvement in symptoms and function after injection (P < 0.05), where zero patients reported intrinsic hand weakness. CONCLUSIONS: The use of US significantly improves the accuracy of delivery of a surrogate dye in a cadaver model of Raynaud disease, whereas its use clinically results in at least as effective a treatment as the landmark approach, but with less botulinum toxin required to achieve the clinical effect. FAU - Lobb, David C AU - Lobb DC AD - From the Departments of Plastic and Maxillofacial Surgery. FAU - Pierce, Jennifer AU - Pierce J AD - Radiology, University of Virginia, Charlottesville, VA. FAU - Perry, Michael AU - Perry M AD - Radiology, University of Virginia, Charlottesville, VA. FAU - DeGeorge, Brent AU - DeGeorge B AD - From the Departments of Plastic and Maxillofacial Surgery. LA - eng PT - Journal Article PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/therapeutic use MH - Hand/diagnostic imaging MH - Humans MH - *Neuromuscular Agents MH - *Raynaud Disease/diagnostic imaging/drug therapy MH - Ultrasonography MH - Upper Extremity EDAT- 2020/05/02 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/05/02 06:00 PHST- 2020/05/02 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/05/02 06:00 [entrez] AID - 00000637-202006005-00008 [pii] AID - 10.1097/SAP.0000000000002303 [doi] PST - ppublish SO - Ann Plast Surg. 2020 Jun;84(6S Suppl 5):S386-S388. doi: 10.1097/SAP.0000000000002303. PMID- 26506940 OWN - NLM STAT- MEDLINE DCOM- 20160607 LR - 20160125 IS - 1097-6809 (Electronic) IS - 0741-5214 (Linking) VI - 63 IP - 2 DP - 2016 Feb TI - Digital periarterial sympathectomy in the management of post-traumatic Raynaud syndrome. PG - 459-65 LID - S0741-5214(15)01936-9 [pii] LID - 10.1016/j.jvs.2015.08.102 [doi] AB - OBJECTIVE: Among Basque handball players, the repeated impact of a ball on the palms of their hands hundreds of thousands of times throughout their sporting careers produces Raynaud syndrome. Treating this patient group is complex. Our objective was to assess the efficacy of digital periarterial sympathectomy in this patient group. METHODS: The study included all of the federated amateur and professional Basque handball patients who presented with Raynaud syndrome assessed in the vascular surgery service between January 2005 and December 2012. The postoperative assessment included a physical examination, basal photoplethysmography and photoplethysmography after heat hyperemia, and arteriography or magnetic resonance angiography. RESULTS: All 182 digital periarterial sympathectomies in the 114 fingers of 60 patients were in Porter functional class III or IV. All patients were discharged within the first 48 hours. Follow-up results, with a mean of 2 years ± 5 months, were 100%. All patients presented immediate pain remission, recovery of comfort, normal nail growth, rapid healing of all ulcers, distal anhidrosis, and return to active sport participation. The results remain steady in 58 patients (93.5%). Mean time until return to sports activity was 9.95 ± 1.61 weeks. CONCLUSIONS: Digital periarterial sympathectomy is a simple, relatively nonaggressive technique without adverse side effects and with excellent medium-term results. In patients with Raynaud syndrome refractory to medical treatment and with threat to the viability of one or several fingers, digital periarterial sympathectomy can be the first treatment option, especially in cases of arteritis associated with very severe spasms. CI - Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved. FAU - Letamendia, Ander AU - Letamendia A AD - Cátedra de Ingeniería Biomédica y Tecnologías Sanitarias, Universidad Católica San Antonio (UCAM), Murcia, Spain; Centro de Ingeniería Biomédica y Tecnologías Sanitarias, Madrid, Spain; Department of Vascular Surgery, Policlínica Gipuzkoa, San Sebastián, Spain. FAU - López-Román, Javier AU - López-Román J AD - Cátedra de Fisiología del Deporte, Universidad Católica San Antonio (UCAM), Murcia, Spain. FAU - Bustamante-Munguira, Juan AU - Bustamante-Munguira J AD - Department of Cardiovascular Surgery, Hospital Universitario de La Princesa, Madrid, Spain. Electronic address: jbustamantemunguira@gmail.com. FAU - Herreros, Jesús AU - Herreros J AD - Cátedra de Ingeniería Biomédica y Tecnologías Sanitarias, Universidad Católica San Antonio (UCAM), Murcia, Spain; Centro de Ingeniería Biomédica y Tecnologías Sanitarias, Madrid, Spain; Department of Cardiac Surgery, Hospital Nisa Pardo Aravaca, Madrid, Spain. LA - eng PT - Journal Article DEP - 20151023 PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Adult MH - Arteries/*innervation MH - Athletic Injuries/diagnosis/etiology/physiopathology/*surgery MH - Fingers/*blood supply MH - Follow-Up Studies MH - Hand Injuries/diagnosis/etiology/physiopathology/*surgery MH - Humans MH - Magnetic Resonance Angiography MH - Male MH - Photoplethysmography MH - Raynaud Disease/diagnosis/etiology/physiopathology/*surgery MH - Recovery of Function MH - Remission Induction MH - Retrospective Studies MH - Spain MH - Sympathectomy/adverse effects/*methods MH - Time Factors MH - Treatment Outcome MH - Wound Healing MH - Young Adult EDAT- 2015/10/29 06:00 MHDA- 2016/06/09 06:00 CRDT- 2015/10/29 06:00 PHST- 2015/06/16 00:00 [received] PHST- 2015/08/25 00:00 [accepted] PHST- 2015/10/29 06:00 [entrez] PHST- 2015/10/29 06:00 [pubmed] PHST- 2016/06/09 06:00 [medline] AID - S0741-5214(15)01936-9 [pii] AID - 10.1016/j.jvs.2015.08.102 [doi] PST - ppublish SO - J Vasc Surg. 2016 Feb;63(2):459-65. doi: 10.1016/j.jvs.2015.08.102. Epub 2015 Oct 23. PMID- 22261435 OWN - NLM STAT- MEDLINE DCOM- 20121119 LR - 20120914 IS - 1940-1574 (Electronic) IS - 0003-3197 (Linking) VI - 63 IP - 7 DP - 2012 Oct TI - The association of Raynaud syndrome with β-blockers: a meta-analysis. PG - 535-40 LID - 10.1177/0003319711432861 [doi] AB - Vasospastic disorders of the digital circulation such as the Raynaud syndrome (RS) are known side effects of treatment of β-adrenergic blockade. The prevalence of RS in patients during treatment with β-blockers is not well defined. The objective of this meta-analysis is to assess the prevalence of RS in patients receiving β-blockers. A literature search was performed for studies dealing with RS and patients receiving β-blockers. The studies provided sufficient data to estimate the prevalence of RS in patients receiving β-blockers. A total of 13 eligible studies, contributing data on 1012 individuals, were included in this meta-analysis. For RS in patients receiving β-blockers, a pooled prevalence of 14.7% (95% confidence interval 0.076-0.236) were obtained. Statistically publication bias was not present (P = .877). Despite some heterogeneity, there is a possible indication of an association between RS and patients receiving β-blockers. FAU - Mohokum, Melvin AU - Mohokum M AD - Department of Biometry and Clinical Epidemiology, Charité Universitätsmedizin Berlin, Berlin, Germany. melvin.mohokum@charite.de FAU - Hartmann, Peter AU - Hartmann P FAU - Schlattmann, Peter AU - Schlattmann P LA - eng PT - Journal Article PT - Meta-Analysis DEP - 20120118 PL - United States TA - Angiology JT - Angiology JID - 0203706 RN - 0 (Adrenergic beta-Antagonists) SB - IM MH - Adrenergic beta-Antagonists/*adverse effects/therapeutic use MH - Cross-Sectional Studies MH - Humans MH - Raynaud Disease/*chemically induced/diagnosis/*epidemiology EDAT- 2012/01/21 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/01/21 06:00 PHST- 2012/01/21 06:00 [entrez] PHST- 2012/01/21 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - 0003319711432861 [pii] AID - 10.1177/0003319711432861 [doi] PST - ppublish SO - Angiology. 2012 Oct;63(7):535-40. doi: 10.1177/0003319711432861. Epub 2012 Jan 18. PMID- 28242038 OWN - NLM STAT- MEDLINE DCOM- 20181024 LR - 20181024 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 38 IP - 4 DP - 2017 Apr TI - [Acrocyanosis: A common but poorly understood condition]. PG - 225-227 LID - S0248-8663(17)30032-2 [pii] LID - 10.1016/j.revmed.2017.01.009 [doi] FAU - Miranda, S AU - Miranda S AD - Normandie université, UNIROUEN, U1096, service de médecine interne, 76000 Rouen, France. FAU - Lévesque, H AU - Lévesque H AD - Normandie université, UNIROUEN, U1096, service de médecine interne, 76000 Rouen, France. Electronic address: herve.levesque@chu-rouen.fr. LA - fre PT - Editorial PT - Review TT - L’acrocyanose essentielle : un trouble vasomoteur fréquent pourtant méconnu. DEP - 20170224 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - *Cyanosis/diagnosis/etiology/therapy MH - Extremities/*blood supply/*pathology MH - Humans MH - Raynaud Disease/diagnosis/therapy MH - Syndrome OTO - NOTNLM OT - Acrocyanose OT - Acrocyanosis EDAT- 2017/03/01 06:00 MHDA- 2018/10/26 06:00 CRDT- 2017/03/01 06:00 PHST- 2017/01/13 00:00 [received] PHST- 2017/01/19 00:00 [accepted] PHST- 2017/03/01 06:00 [pubmed] PHST- 2018/10/26 06:00 [medline] PHST- 2017/03/01 06:00 [entrez] AID - S0248-8663(17)30032-2 [pii] AID - 10.1016/j.revmed.2017.01.009 [doi] PST - ppublish SO - Rev Med Interne. 2017 Apr;38(4):225-227. doi: 10.1016/j.revmed.2017.01.009. Epub 2017 Feb 24. PMID- 18078610 OWN - NLM STAT- MEDLINE DCOM- 20080410 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 25 IP - 5 DP - 2007 Sep-Oct TI - Nailfold capillaroscopy and classification criteria for systemic sclerosis. PG - 663-5 FAU - Cutolo, M AU - Cutolo M FAU - Matucci Cerinic, M AU - Matucci Cerinic M LA - eng PT - Comment PT - Editorial PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM CON - Clin Exp Rheumatol. 2007 Sep-Oct;25(5):754-7. PMID: 18078627 MH - Capillaries/pathology MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply/pathology MH - Raynaud Disease/diagnosis/pathology MH - Scleroderma, Systemic/*classification/*diagnosis/pathology MH - Sensitivity and Specificity MH - Societies, Medical MH - United States EDAT- 2007/12/15 09:00 MHDA- 2008/04/11 09:00 CRDT- 2007/12/15 09:00 PHST- 2007/12/15 09:00 [pubmed] PHST- 2008/04/11 09:00 [medline] PHST- 2007/12/15 09:00 [entrez] AID - 2173 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2007 Sep-Oct;25(5):663-5. PMID- 27799439 OWN - NLM STAT- MEDLINE DCOM- 20180306 LR - 20260127 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 26 IP - 7 DP - 2017 Jun TI - Systemic lupus erythematosus in Spanish males: a study of the Spanish Rheumatology Society Lupus Registry (RELESSER) cohort. PG - 698-706 LID - 10.1177/0961203316673728 [doi] AB - Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p < 0.001). Males were diagnosed earlier than females (p = 0.04) and had more cardiovascular comorbidities ( p < 0.001). Two hundred and thirty-six males (68%) with SLE required hospitalization in comparison with 1713 females (53%) ( p < 0.001). During follow-up, 208 patients died: 30 men (9.3%) and 178 women (5.9%) ( p = 0.02). As regards clinical manifestations, loss of weight ( p = 0.01), lymphadenopathies ( p = 0.02), and splenomegaly ( p = 0.02) were more common in male patients. Female patients were more likely to have inflammatory rash, alopecia, and arthritis ( p < 0.05). As for lung involvement, men with SLE had more pleural fibrosis ( p < 0.001) and pulmonary embolism ( p = 0.01). However, Raynaud's phenomenon was more common in women (35%) than in men (23.7%) ( p < 0.001); lupus nephritis was more common in men, being present in 155 (44.8%) of males versus 933 (29%) of females ( p < 0.001). Multivariate analysis showed that SLE patients with a high Charlson index (more than 3 points) and age > 50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women. FAU - Riveros Frutos, A AU - Riveros Frutos A AD - 1 Rheumatology Department, Germans Trias i Pujol University Hospital, Badalona, Spain. AD - 2 Medicine Department, UAB, Spain. FAU - Casas, I AU - Casas I AD - 3 Preventive Department, Germans Trias i Pujol University Hospital, Badalona, Spain. FAU - Rúa-Figueroa, I AU - Rúa-Figueroa I AD - 4 Rheumatology Department, Doctor Negrín University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain. FAU - López-Longo, F J AU - López-Longo FJ AD - 5 Rheumatology Department, Gregorio Marañón University Hospital, Madrid, Spain. FAU - Calvo-Alén, J AU - Calvo-Alén J AD - 6 Rheumatology Department, Sierrallana Hospital, Torrelavega, Spain. FAU - Galindo, M AU - Galindo M AD - 7 Rheumatology Department, Doce de Octubre University Hospital, Madrid, Spain. FAU - Fernández-Nebro, A AU - Fernández-Nebro A AD - 8 Rheumatology Department, Carlos Haya University Hospital, Málaga, Spain. FAU - Pego-Reigosa, J M AU - Pego-Reigosa JM AD - 9 Rheumatology Department, University Hospital Complex Instituto de Investigación Biomédica de Vigo (IBIV), Spain. FAU - Olivé Marqués, A AU - Olivé Marqués A AD - 1 Rheumatology Department, Germans Trias i Pujol University Hospital, Badalona, Spain. CN - RELESSER Group, part of the Spanish Society of Rheumatology Systemic Autoimmune Diseases Study Group (EASSER) LA - eng PT - Journal Article PT - Multicenter Study DEP - 20161031 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adolescent MH - Adult MH - Age Factors MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Cohort Studies MH - Cross-Sectional Studies MH - Female MH - Hospitalization/statistics & numerical data MH - Humans MH - Lupus Erythematosus, Systemic/complications/mortality/*physiopathology MH - Lupus Nephritis/*epidemiology MH - Male MH - Middle Aged MH - Raynaud Disease/*epidemiology MH - Registries MH - Retrospective Studies MH - Sex Factors MH - Spain MH - Young Adult OTO - NOTNLM OT - comorbidities OT - male patients OT - systemic lupus erythematosus EDAT- 2016/11/02 06:00 MHDA- 2018/03/07 06:00 CRDT- 2016/11/02 06:00 PHST- 2016/11/02 06:00 [pubmed] PHST- 2018/03/07 06:00 [medline] PHST- 2016/11/02 06:00 [entrez] AID - 0961203316673728 [pii] AID - 10.1177/0961203316673728 [doi] PST - ppublish SO - Lupus. 2017 Jun;26(7):698-706. doi: 10.1177/0961203316673728. Epub 2016 Oct 31. PMID- 31292429 OWN - NLM STAT- MEDLINE DCOM- 20200821 LR - 20200821 IS - 1897-9483 (Electronic) IS - 0032-3772 (Linking) VI - 129 IP - 9 DP - 2019 Sep 30 TI - Microvascular imaging of primary erythromelalgia. PG - 632-633 LID - 10.20452/pamw.14890 [doi] FAU - Hellmann, Marcin AU - Hellmann M AD - Department of Cardiac Diagnostics, Medical University of Gdańsk, Gdańsk, Poland. marcin.hellmann@gmail.com FAU - Imbert, Bernard AU - Imbert B AD - Department of Vascular Medicine, Grenoble University Hospital, Grenoble, France FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - INSERM HP2, University of Grenoble Alpes, Grenoble, France; Clinical Pharmacology Unit, INSERM Clinical Investigation Center, Grenoble University Hospital, Grenoble, France LA - eng PT - Case Reports PT - Journal Article DEP - 20190704 PL - Poland TA - Pol Arch Intern Med JT - Polish archives of internal medicine JID - 101700960 SB - IM MH - Cold Temperature MH - Diagnostic Imaging/methods MH - Erythromelalgia/*diagnostic imaging/physiopathology MH - Female MH - Hand/*physiopathology MH - Humans MH - Middle Aged MH - Raynaud Disease/*diagnostic imaging/physiopathology MH - Skin/blood supply EDAT- 2019/07/12 06:00 MHDA- 2020/08/22 06:00 CRDT- 2019/07/12 06:00 PHST- 2019/07/12 06:00 [pubmed] PHST- 2020/08/22 06:00 [medline] PHST- 2019/07/12 06:00 [entrez] AID - 10.20452/pamw.14890 [doi] PST - ppublish SO - Pol Arch Intern Med. 2019 Sep 30;129(9):632-633. doi: 10.20452/pamw.14890. Epub 2019 Jul 4. PMID- 27443032 OWN - NLM STAT- MEDLINE DCOM- 20160822 LR - 20260127 IS - 1462-2815 (Print) IS - 1462-2815 (Linking) VI - 89 IP - 6 DP - 2016 Jun TI - Last word with ... SUE FARRINGTON. PG - 48 FAU - Farrington, Sue AU - Farrington S LA - eng PT - Journal Article PL - England TA - Community Pract JT - Community practitioner : the journal of the Community Practitioners' & Health Visitors' Association JID - 9809060 MH - Charities/*organization & administration MH - Humans MH - Organizational Objectives MH - Raynaud Disease/epidemiology/*prevention & control MH - Scleroderma, Localized/epidemiology/*prevention & control MH - Scleroderma, Systemic/epidemiology/*prevention & control MH - United Kingdom/epidemiology EDAT- 2016/07/23 06:00 MHDA- 2016/08/23 06:00 CRDT- 2016/07/23 06:00 PHST- 2016/07/23 06:00 [entrez] PHST- 2016/07/23 06:00 [pubmed] PHST- 2016/08/23 06:00 [medline] PST - ppublish SO - Community Pract. 2016 Jun;89(6):48. PMID- 21623666 OWN - NLM STAT- MEDLINE DCOM- 20111013 LR - 20110822 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 40 IP - 4 DP - 2011 TI - Long-term outcome of lung transplantation in a patient with the anti-synthetase syndrome. PG - 327-8 LID - 10.3109/03009742.2011.566280 [doi] FAU - Sem, M AU - Sem M FAU - Lund, M B AU - Lund MB FAU - Molberg, O AU - Molberg O LA - eng PT - Case Reports PT - Letter DEP - 20110530 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antibodies) RN - EC 6.- (Ligases) SB - IM MH - Adult MH - Antibodies/*blood MH - Arthritis/immunology/*surgery MH - Female MH - Humans MH - Ligases/*immunology MH - Lung Diseases, Interstitial/immunology/*surgery MH - *Lung Transplantation MH - Raynaud Disease/immunology/*surgery MH - Syndrome MH - Time Factors MH - Treatment Outcome EDAT- 2011/06/01 06:00 MHDA- 2011/10/14 06:00 CRDT- 2011/06/01 06:00 PHST- 2011/06/01 06:00 [entrez] PHST- 2011/06/01 06:00 [pubmed] PHST- 2011/10/14 06:00 [medline] AID - 10.3109/03009742.2011.566280 [doi] PST - ppublish SO - Scand J Rheumatol. 2011;40(4):327-8. doi: 10.3109/03009742.2011.566280. Epub 2011 May 30. PMID- 27575610 OWN - NLM STAT- MEDLINE DCOM- 20170221 LR - 20220410 IS - 1546-3141 (Electronic) IS - 0361-803X (Linking) VI - 207 IP - 6 DP - 2016 Dec TI - Abnormal Bone Marrow Signal Intensity in the Phalanges of the Foot as a Manifestation of Raynaud Phenomenon: A Report of Six Patients. PG - 1252-1256 AB - OBJECTIVE: The objectives of our study were to describe the MRI findings of pedal phalangeal bone marrow edema in patients with Raynaud phenomenon (RP) and discuss the clinical implications of these MRI findings. CONCLUSION: There is a progressive distal-to-proximal pattern of pedal phalangeal bone marrow edema on MRI in patients with RP. This knowledge may allow early diagnosis and treatment of rheumatologic disorders that are potentially associated with RP. FAU - Smitaman, Edward AU - Smitaman E AD - 1 Department of Radiology, University of California San Diego Medical Center, 8899 University Center Ln, Ste 370, San Diego, CA 92122. FAU - Pereira, Bruno P G AU - Pereira BP AD - 1 Department of Radiology, University of California San Diego Medical Center, 8899 University Center Ln, Ste 370, San Diego, CA 92122. FAU - Huang, Brady K AU - Huang BK AD - 1 Department of Radiology, University of California San Diego Medical Center, 8899 University Center Ln, Ste 370, San Diego, CA 92122. FAU - Zakhary, Mina M AU - Zakhary MM AD - 2 Department of Radiology, Scripps Memorial Hospital, La Jolla, CA. FAU - Fliszar, Evelyne AU - Fliszar E AD - 1 Department of Radiology, University of California San Diego Medical Center, 8899 University Center Ln, Ste 370, San Diego, CA 92122. FAU - Resnick, Donald L AU - Resnick DL AD - 1 Department of Radiology, University of California San Diego Medical Center, 8899 University Center Ln, Ste 370, San Diego, CA 92122. LA - eng PT - Journal Article PT - Observational Study DEP - 20160830 PL - United States TA - AJR Am J Roentgenol JT - AJR. American journal of roentgenology JID - 7708173 SB - IM MH - Adolescent MH - Adult MH - Bone Marrow/*diagnostic imaging/pathology MH - Bone Marrow Diseases/*diagnostic imaging/etiology/pathology MH - Diagnosis, Differential MH - Edema/*diagnostic imaging/etiology/pathology MH - Female MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Male MH - Middle Aged MH - Raynaud Disease/complications/*diagnostic imaging/pathology MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Toe Phalanges/*diagnostic imaging/pathology OTO - NOTNLM OT - MRI OT - Raynaud phenomenon OT - abnormal bone marrow signal intensity OT - feet OT - phalanges EDAT- 2016/08/31 06:00 MHDA- 2017/02/22 06:00 CRDT- 2016/08/31 06:00 PHST- 2016/08/31 06:00 [pubmed] PHST- 2017/02/22 06:00 [medline] PHST- 2016/08/31 06:00 [entrez] AID - 10.2214/AJR.16.16366 [doi] PST - ppublish SO - AJR Am J Roentgenol. 2016 Dec;207(6):1252-1256. doi: 10.2214/AJR.16.16366. Epub 2016 Aug 30. PMID- 26210127 OWN - NLM STAT- MEDLINE DCOM- 20160509 LR - 20220318 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 41 IP - 3 DP - 2015 Aug TI - Management of Raynaud Phenomenon and Digital Ulcers in Scleroderma. PG - 419-38 LID - S0889-857X(15)00026-5 [pii] LID - 10.1016/j.rdc.2015.04.005 [doi] AB - Raynaud phenomenon (RP) and associated digital ischemia can be among the most vexing clinical problems for patients with systemic sclerosis (scleroderma). Understanding the treatment approach to RP and associated ischemia and how to prevent digital ulcers is important for clinicians caring for these patients. This article reviews the management of RP and digital ischemic ulcers. The magnitude of the problem and pathophysiology of RP are first discussed, with an emphasis on recent advances in understanding of the disease process. Options for the practical pharmacologic and nonpharmacologic interventions for RP and digital ischemic ulcers are detailed. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Cappelli, Laura AU - Cappelli L AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Suite 4100, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Suite 4100, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, USA. Electronic address: fwig@jhmi.edu. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20150523 PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 SB - IM MH - Adult MH - Female MH - *Fingers/blood supply MH - Humans MH - Raynaud Disease/etiology/*therapy MH - Scleroderma, Systemic/*complications MH - Ulcer/drug therapy/etiology/*therapy OTO - NOTNLM OT - Calcium channel blocker OT - Digital ischemia OT - Digital ulcer OT - Prostacyclin OT - Raynaud phenomenon OT - Scleroderma OT - Thermoregulatory vessels EDAT- 2015/07/27 06:00 MHDA- 2016/05/10 06:00 CRDT- 2015/07/27 06:00 PHST- 2015/07/27 06:00 [entrez] PHST- 2015/07/27 06:00 [pubmed] PHST- 2016/05/10 06:00 [medline] AID - S0889-857X(15)00026-5 [pii] AID - 10.1016/j.rdc.2015.04.005 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2015 Aug;41(3):419-38. doi: 10.1016/j.rdc.2015.04.005. Epub 2015 May 23. PMID- 36703271 OWN - NLM STAT- MEDLINE DCOM- 20230130 LR - 20230228 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 26 IP - 2 DP - 2023 Feb TI - Botulinum toxin injection for refractory Raynaud phenomenon and digital ulcers in systemic sclerosis. PG - 187-189 LID - 10.1111/1756-185X.14487 [doi] FAU - Shen, Yi-Hsuan AU - Shen YH AUID- ORCID: 0000-0002-1036-9800 AD - National Taiwan University Hospital, Taipei City, Taiwan. FAU - Lee, Yung-Heng AU - Lee YH AD - Department of Orthopedics, Cishan Hospital, Ministry of Health and Welfare, Kaohsiung, Taiwan. AD - Institute of Medical Science and Technology, National Sun Yat-sen University, Kaohsiung, Taiwan. AD - Department of Senior Services Industry Management, Minghsin University of Science and Technology, Hsinchu, Taiwan. AD - Department of Recreation and Sport Management, Shu-Te University, Kaohsiung, Taiwan. FAU - Leong, Pui-Ying AU - Leong PY AD - Division of Allergy, Immunology & Rheumatology, Department of Internal Medicine, Chung Shan Medical University Hospital, Taichung, Taiwan. AD - Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan. AD - School of Medicine, Chung Shan Medical University, Taichung, Taiwan. LA - eng PT - Comment PT - Editorial PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - EC 3.4.24.69 (Botulinum Toxins) RN - digital ulcers SB - IM CON - Clin Rheumatol. 2022 Jan;41(1):95-104. doi: 10.1007/s10067-021-05900-7. PMID: 34471968 MH - Humans MH - *Skin Ulcer/diagnosis/drug therapy/etiology MH - *Raynaud Disease/diagnosis/drug therapy MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy MH - *Botulinum Toxins OTO - NOTNLM OT - Raynaud phenomenon OT - botulinum toxin OT - digital ulcer OT - systemic sclerosis EDAT- 2023/01/28 06:00 MHDA- 2023/01/31 06:00 CRDT- 2023/01/27 00:12 PHST- 2022/09/20 00:00 [received] PHST- 2022/10/19 00:00 [accepted] PHST- 2023/01/27 00:12 [entrez] PHST- 2023/01/28 06:00 [pubmed] PHST- 2023/01/31 06:00 [medline] AID - 10.1111/1756-185X.14487 [doi] PST - ppublish SO - Int J Rheum Dis. 2023 Feb;26(2):187-189. doi: 10.1111/1756-185X.14487. PMID- 24286738 OWN - NLM STAT- MEDLINE DCOM- 20140812 LR - 20171116 IS - 1558-1969 (Electronic) IS - 0749-0712 (Linking) VI - 30 IP - 1 DP - 2014 Feb TI - Minimally invasive treatment of Raynaud phenomenon: the role of botulinum type A. PG - 17-24 LID - S0749-0712(13)00082-6 [pii] LID - 10.1016/j.hcl.2013.09.006 [doi] AB - Although the mechanism is unknown, Btx-A injection may be an effective, localized, nonsurgical treatment option without addictive properties or systemic side effects for the treatment of ischemic digits. Clinical research supports the safety and efficacy of injection of Btx-A for the treatment of Raynaud phenomenon. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Neumeister, Michael W AU - Neumeister MW AD - Department of Surgery - Institute for Plastic Surgery, SIU School of Medicine, 747 North Rutledge 3rd Floor, PO Box 19653, Springfield, IL 62794, USA. Electronic address: mneumeister@siumed.edu. FAU - Webb, Kelli Nicole Belangee AU - Webb KN FAU - Romanelli, Michael AU - Romanelli M LA - eng PT - Journal Article PT - Review PL - United States TA - Hand Clin JT - Hand clinics JID - 8510415 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/pharmacology/*therapeutic use MH - Contraindications MH - Humans MH - Injections, Intralesional MH - Neuromuscular Agents/pharmacology/*therapeutic use MH - Raynaud Disease/*drug therapy/physiopathology MH - Treatment Outcome MH - Vasoconstriction/drug effects/physiology OTO - NOTNLM OT - Botulinum type A OT - Minimally invasive OT - Raynaud phenomenon OT - Treatment EDAT- 2013/11/30 06:00 MHDA- 2014/08/13 06:00 CRDT- 2013/11/30 06:00 PHST- 2013/11/30 06:00 [entrez] PHST- 2013/11/30 06:00 [pubmed] PHST- 2014/08/13 06:00 [medline] AID - S0749-0712(13)00082-6 [pii] AID - 10.1016/j.hcl.2013.09.006 [doi] PST - ppublish SO - Hand Clin. 2014 Feb;30(1):17-24. doi: 10.1016/j.hcl.2013.09.006. PMID- 34785028 OWN - NLM STAT- MEDLINE DCOM- 20220318 LR - 20250530 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 52 DP - 2022 Feb TI - Symptom experience of limited cutaneous systemic sclerosis from the Patients' perspective: A qualitative study(✰,✰✰,★,★★). PG - 151926 LID - S0049-0172(21)00199-2 [pii] LID - 10.1016/j.semarthrit.2021.11.003 [doi] AB - OBJECTIVES: Limited cutaneous systemic sclerosis (lcSSc) is the largest subgroup of people with SSc, but little is known about symptom experience from the patients' perspective. This study aimed to comprehensively identify domains and symptoms reported as bothersome by patients with lcSSc and to analyze themes and sub-themes capturing symptom experience in this population. METHODS: Focus groups of patients with lcSSc were conducted using a structured guide. Patients were recruited based on an a priori purposive framework to include men and women with SSc. Focus groups were recorded, transcribed, anonymized, and analyzed using the RADaR technique (rigorous and accelerated data reduction) combined with iterative, deductive and inductive approaches. RESULTS: Four 2-hour Focus groups comprising participants with lcSSc were conducted (N = 26). Ninety-four symptoms were identified with 22 domains. Symptoms from the following domains were mentioned in all Focus groups: skin, musculoskeletal (MSK), cardiac, pulmonary, gastro-intestinal (GI) manifestations, fatigue, sleep, Raynaud's phenomenon (RP), pain, and digital ulcers. The three most cited domains in a pre-meeting survey were GI, RP and MSK. Seven themes defining symptom experience were identified: difficulty attributing symptoms to this complex systemic disease, influence of symptom temporality, impairment of emotional well-being, limitations in functioning (including several sub-themes of functioning), survival, symptoms management and uncertainty. CONCLUSION: This study comprehensively identified domains and symptoms considered bothersome from the perspective of patients with lcSSc and summarized patient experience of lcSSc-related symptoms. This study is the first step in the design of a future combined response index dedicated to lcSSc that could foster specific research on this subpopulation of patients. CI - Copyright © 2021 Elsevier Inc. All rights reserved. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, Rennes University Hospital, Rennes, France; Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France; Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA. FAU - Murphy, Susan L AU - Murphy SL AD - Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA; Department of Physical Medicine & Rehabilitation, University of Michigan, USA; VA Ann Arbor Healthcare System, Geriatric Research Education and Clinical Center, GRECC, Ann Arbor, MI USA. Electronic address: sumurphy@med.umich.edu. FAU - Chen, Yen T AU - Chen YT AD - Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA; Department of Physical Medicine & Rehabilitation, University of Michigan, USA. FAU - Vann, Nadia AU - Vann N AD - Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA. FAU - Galdo, Francesco Del AU - Galdo FD AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; Leeds Biomedical Research Centre, National Institute for Health Research, Leeds, UK. FAU - Cella, David AU - Cella D AD - Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, 625 Michigan Ave, 21st Floor, Chicago, IL, 60611, USA. FAU - Buch, Maya H AU - Buch MH AD - Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine & Health, University of Manchester, Manchester, UK.; NIHR Manchester Biomedical Research Centre, Manchester Academic Health Science Centre, Manchester University Foundation Trust, Manchester, UK. FAU - Khanna, Dinesh AU - Khanna D AD - Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA; Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA. LA - eng GR - 90ARCP0003/ACL/ACL HHS/United States GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20211107 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Female MH - Humans MH - Male MH - Qualitative Research MH - *Raynaud Disease MH - *Scleroderma, Limited MH - *Scleroderma, Systemic/diagnosis MH - *Skin Ulcer PMC - PMC9131352 MID - NIHMS1804925 OTO - NOTNLM OT - Combined response index OT - Domains OT - Limited cutaneous systemic sclerosis OT - Qualitative analysis OT - Scleroderma OT - Symptoms experience COIS- Declaration of Competing Interest Authors have nothing to declare. EDAT- 2021/11/18 06:00 MHDA- 2022/03/19 06:00 PMCR- 2022/05/25 CRDT- 2021/11/17 05:44 PHST- 2021/07/28 00:00 [received] PHST- 2021/10/30 00:00 [revised] PHST- 2021/11/04 00:00 [accepted] PHST- 2021/11/18 06:00 [pubmed] PHST- 2022/03/19 06:00 [medline] PHST- 2021/11/17 05:44 [entrez] PHST- 2022/05/25 00:00 [pmc-release] AID - S0049-0172(21)00199-2 [pii] AID - 10.1016/j.semarthrit.2021.11.003 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Feb;52:151926. doi: 10.1016/j.semarthrit.2021.11.003. Epub 2021 Nov 7. PMID- 22710940 OWN - NLM STAT- MEDLINE DCOM- 20121217 LR - 20220410 IS - 1538-3679 (Electronic) IS - 0003-9926 (Linking) VI - 172 IP - 15 DP - 2012 Aug 13 TI - Vardenafil for the treatment of raynaud phenomenon: a randomized, double-blind, placebo-controlled crossover study. PG - 1182-4 LID - 10.1001/archinternmed.2012.2271 [doi] FAU - Caglayan, Evren AU - Caglayan E FAU - Axmann, Sarah AU - Axmann S FAU - Hellmich, Martin AU - Hellmich M FAU - Moinzadeh, Pia AU - Moinzadeh P FAU - Rosenkranz, Stephan AU - Rosenkranz S LA - eng PT - Letter PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Arch Intern Med JT - Archives of internal medicine JID - 0372440 RN - 0 (Imidazoles) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Sulfones) RN - 0 (Triazines) RN - 5O8R96XMH7 (Vardenafil Dihydrochloride) SB - IM MH - Double-Blind Method MH - Drug Monitoring/methods MH - Female MH - *Fingers/blood supply/physiopathology MH - Half-Life MH - Humans MH - *Imidazoles/administration & dosage/adverse effects/pharmacokinetics MH - Laser-Doppler Flowmetry/methods MH - Male MH - Phosphodiesterase 5 Inhibitors/administration & dosage/adverse effects/pharmacokinetics MH - *Piperazines/administration & dosage/adverse effects/pharmacokinetics MH - *Raynaud Disease/diagnosis/drug therapy/physiopathology MH - Regional Blood Flow/*drug effects MH - Sulfones/administration & dosage/adverse effects/pharmacokinetics MH - Treatment Outcome MH - Triazines/administration & dosage/adverse effects/pharmacokinetics MH - Vardenafil Dihydrochloride EDAT- 2012/06/20 06:00 MHDA- 2012/12/18 06:00 CRDT- 2012/06/20 06:00 PHST- 2012/06/20 06:00 [entrez] PHST- 2012/06/20 06:00 [pubmed] PHST- 2012/12/18 06:00 [medline] AID - 1188038 [pii] AID - 10.1001/archinternmed.2012.2271 [doi] PST - ppublish SO - Arch Intern Med. 2012 Aug 13;172(15):1182-4. doi: 10.1001/archinternmed.2012.2271. PMID- 29338587 OWN - NLM STAT- MEDLINE DCOM- 20180925 LR - 20180925 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 27 IP - 5 DP - 2018 Apr TI - Antiphosphatidylserine/prothrombin (aPS/PT) antibodies are associated with Raynaud phenomenon and migraine in primary thrombotic antiphospholipid syndrome. PG - 812-819 LID - 10.1177/0961203317751644 [doi] AB - Objectives Antibodies to phosphatidylserine/prothrombin complex (aPS/PT) detectable in sera of some patients with antiphospholipid syndrome (APS) have been shown to correlate with thrombosis. However, associations of aPS/PT antibodies with APS related disorders remain unclear. Aim To evaluate whether there are any associations between aPS/PT antibodies and Raynaud phenomenon, migraine and/or valvular lesions in primary thrombotic APS (PAPS). Methods We enrolled 67 consecutive patients (56 women) with thrombotic PAPS (VTE in 80.6%), aged 46.2 ± 13.5 years. The exclusion criteria were: acute coronary syndromes or stroke within preceding 6 months, cancer, severe comorbidities and pregnancy. The IgG and IgM aPS/PT antibodies were determined by ELISA with the cut-off of 30 units. We recorded Raynaud phenomenon, migraine and valvular lesions. Results Positive IgM or/and IgG aPS/PT antibodies were observed in 29 patients (43.3%), with a higher prevalence of IgM antibodies ( n = 27, 40.3%) compared with IgG isotype ( n = 12, 17.9%, p = 0.014). aPS/PT antibodies were observed most commonly in patients with triple aPL ( n = 12, 85.7%) compared with those with double ( n = 5, 35.7%) or single aPL antibodies (n = 12, 30.8%, p = 0.03), with no association with demographics, the ANA titre, the type of thrombotic events or medications. Raynaud phenomenon, migraine and valvular lesions were observed in 15% ( n = 10), 30% ( n = 20) and 18% ( n = 12) of the patients, respectively. Raynaud phenomenon and migraine, but not valvular lesions, were markedly more frequent in PAPS patients presenting with positive aPS/PT antibodies ( n = 10, 34.5% vs. n = 0, 0%; p = 0.0001). Conclusions In PAPS patients aPS/PT antibodies are related to the occurrence of both Raynaud phenomenon and migraine. FAU - Kopytek, M AU - Kopytek M AD - 1 John Paul II Hospital, Kraków, Poland. FAU - Natorska, J AU - Natorska J AD - 1 John Paul II Hospital, Kraków, Poland. AD - 2 Institute of Cardiology, 49573 Jagiellonian University Medical College , Kraków, Poland. FAU - Undas, A AU - Undas A AD - 1 John Paul II Hospital, Kraków, Poland. AD - 2 Institute of Cardiology, 49573 Jagiellonian University Medical College , Kraków, Poland. LA - eng PT - Journal Article DEP - 20180116 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) RN - 0 (Phosphatidylserines) RN - 9001-26-7 (Prothrombin) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/*blood MH - Antiphospholipid Syndrome/blood/*complications/diagnosis/immunology MH - Cross-Sectional Studies MH - Female MH - Heart Valve Diseases/blood/etiology/immunology MH - Humans MH - Immunoglobulin G/*blood MH - Immunoglobulin M/*blood MH - Male MH - Middle Aged MH - Migraine Disorders/blood/diagnosis/*etiology/immunology MH - Phosphatidylserines/*immunology MH - Prothrombin/*immunology MH - Raynaud Disease/blood/diagnosis/*etiology/immunology MH - Risk Factors MH - Venous Thromboembolism/blood/diagnosis/*etiology/immunology OTO - NOTNLM OT - Primary antiphospholipid syndrome (PAPS) OT - Raynaud phenomenon OT - antiphosphatidylserine/prothrombin (aPS/PT) antibodies OT - antiphospholipid (aPL) antibodies OT - thrombosis EDAT- 2018/01/18 06:00 MHDA- 2018/09/27 06:00 CRDT- 2018/01/18 06:00 PHST- 2018/01/18 06:00 [pubmed] PHST- 2018/09/27 06:00 [medline] PHST- 2018/01/18 06:00 [entrez] AID - 10.1177/0961203317751644 [doi] PST - ppublish SO - Lupus. 2018 Apr;27(5):812-819. doi: 10.1177/0961203317751644. Epub 2018 Jan 16. PMID- 32674590 OWN - NLM STAT- MEDLINE DCOM- 20210326 LR - 20210326 IS - 1938-2707 (Electronic) IS - 0009-9228 (Linking) VI - 59 IP - 13 DP - 2020 Nov TI - Color Changes in the Fingers and Toes of an Infant. PG - 1205-1208 LID - 10.1177/0009922820941648 [doi] FAU - Murphey, Annie Coller AU - Murphey AC AUID- ORCID: 0000-0002-2655-4023 AD - University of Michigan, Ann Arbor, MI, USA. FAU - Morrison Ponce, Daphne AU - Morrison Ponce D AUID- ORCID: 0000-0002-6191-643X AD - Michigan Medicine, Ann Arbor, MI, USA. FAU - Hashikawa, Andrew N AU - Hashikawa AN AD - Michigan Medicine, Ann Arbor, MI, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200716 PL - United States TA - Clin Pediatr (Phila) JT - Clinical pediatrics JID - 0372606 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - R16CO5Y76E (Aspirin) SB - IM MH - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use MH - Aspirin/therapeutic use MH - Color MH - Diagnosis, Differential MH - Echocardiography MH - Exanthema MH - Fever MH - Fingers MH - Heart/diagnostic imaging/physiopathology MH - Heart Diseases/complications/diagnostic imaging/physiopathology MH - Humans MH - Infant MH - Magnetic Resonance Imaging MH - Male MH - Mucocutaneous Lymph Node Syndrome/*complications/*diagnosis/drug therapy MH - Raynaud Disease/*complications/*diagnosis/drug therapy MH - Toes EDAT- 2020/07/18 06:00 MHDA- 2021/03/27 06:00 CRDT- 2020/07/18 06:00 PHST- 2020/07/18 06:00 [pubmed] PHST- 2021/03/27 06:00 [medline] PHST- 2020/07/18 06:00 [entrez] AID - 10.1177/0009922820941648 [doi] PST - ppublish SO - Clin Pediatr (Phila). 2020 Nov;59(13):1205-1208. doi: 10.1177/0009922820941648. Epub 2020 Jul 16. PMID- 17014637 OWN - NLM STAT- MEDLINE DCOM- 20070130 LR - 20090303 IS - 0736-8046 (Print) IS - 0736-8046 (Linking) VI - 23 IP - 5 DP - 2006 Sep-Oct TI - The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary raynaud phenomenon: a follow-up study of 250 patients. PG - 437-42 AB - To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow-up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were diagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future development of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders. FAU - Pavlov-Dolijanović, Slavica AU - Pavlov-Dolijanović S AD - Institute of Rheumatology-Belgrade, Resavska, Belgrade, Serbia and Montenegro. dolijan@agrifaculty.bg.ac.yu FAU - Damjanov, Nemanja AU - Damjanov N FAU - Ostojić, Predrag AU - Ostojić P FAU - Susić, Gordana AU - Susić G FAU - Stojanović, Roksanda AU - Stojanović R FAU - Gacić, Dragica AU - Gacić D FAU - Grdinić, Aleksandra AU - Grdinić A LA - eng PT - Journal Article PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 SB - IM MH - Adolescent MH - Capillaries/physiopathology MH - Child MH - Connective Tissue Diseases/diagnosis/*etiology/physiopathology MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy MH - Nails/*blood supply MH - Predictive Value of Tests MH - Prognosis MH - Raynaud Disease/complications/*diagnosis/*physiopathology MH - Time Factors EDAT- 2006/10/04 09:00 MHDA- 2007/01/31 09:00 CRDT- 2006/10/04 09:00 PHST- 2006/10/04 09:00 [pubmed] PHST- 2007/01/31 09:00 [medline] PHST- 2006/10/04 09:00 [entrez] AID - PDE278 [pii] AID - 10.1111/j.1525-1470.2006.00278.x [doi] PST - ppublish SO - Pediatr Dermatol. 2006 Sep-Oct;23(5):437-42. doi: 10.1111/j.1525-1470.2006.00278.x. PMID- 32562363 OWN - NLM STAT- MEDLINE DCOM- 20210111 LR - 20210111 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 72 IP - 9 DP - 2020 Sep TI - Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma. PG - 1415-1426 LID - 10.1002/art.41406 [doi] AB - You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, finger swelling of several; months' duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest computed tomography shows evidence of ground-glass opacification in both lower lung fields. CI - © 2020, American College of Rheumatology. FAU - Simms, Robert W AU - Simms RW AUID- ORCID: 0000-0002-0101-1807 AD - Dartmouth College Geisel School of Medicine, Hanover, New Hampshire. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20200809 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Antihypertensive Agents) RN - 0 (Antirheumatic Agents) RN - 0 (Calcium Channel Blockers) RN - 0 (Complement C3) RN - 0 (Complement C4) RN - 0 (Complement Inactivating Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Indoles) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Vasodilator Agents) RN - 0 (anticentromere antibody) RN - 8N3DW7272P (Cyclophosphamide) RN - A3ULP0F556 (eculizumab) RN - EC 2.7.7.6 (RNA Polymerase III) RN - G6HRD2P839 (nintedanib) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Antibodies, Antinuclear/immunology MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Antihypertensive Agents/*therapeutic use MH - Antirheumatic Agents/*therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Complement C3/immunology MH - Complement C4/immunology MH - Complement Inactivating Agents/therapeutic use MH - Cyclophosphamide/therapeutic use MH - Endothelin Receptor Antagonists/therapeutic use MH - Humans MH - Hypertension/diagnosis/etiology/*therapy MH - Indoles/therapeutic use MH - Lung Diseases, Interstitial/diagnostic imaging/etiology/*therapy MH - Plasma Exchange MH - Protein Kinase Inhibitors/*therapeutic use MH - RNA Polymerase III/immunology MH - Raynaud Disease MH - Renal Insufficiency/etiology/immunology/pathology/*therapy MH - Scleroderma, Diffuse/complications/diagnosis/immunology/*therapy MH - Scleroderma, Limited/complications/diagnosis/immunology/*therapy MH - Stem Cell Transplantation MH - Tomography, X-Ray Computed MH - Transplantation, Autologous MH - Vasodilator Agents/therapeutic use EDAT- 2020/06/21 06:00 MHDA- 2021/01/12 06:00 CRDT- 2020/06/21 06:00 PHST- 2020/05/12 00:00 [received] PHST- 2020/06/12 00:00 [accepted] PHST- 2020/06/21 06:00 [pubmed] PHST- 2021/01/12 06:00 [medline] PHST- 2020/06/21 06:00 [entrez] AID - 10.1002/art.41406 [doi] PST - ppublish SO - Arthritis Rheumatol. 2020 Sep;72(9):1415-1426. doi: 10.1002/art.41406. Epub 2020 Aug 9. PMID- 24757247 OWN - NLM STAT- MEDLINE DCOM- 20150727 LR - 20181202 IS - 1935-5548 (Electronic) IS - 0149-5992 (Linking) VI - 37 IP - 5 DP - 2014 TI - Artifactual hypoglycemia: an old term for a new classification. PG - e85-6 LID - 10.2337/dc13-2891 [doi] FAU - Tarasova, Valentina D AU - Tarasova VD AD - Corresponding author: Valentina D. Tarasova, tarasova.md@gmail.com. FAU - Zena, Mohsen AU - Zena M FAU - Rendell, Marc AU - Rendell M LA - eng PT - Case Reports PT - Letter PL - United States TA - Diabetes Care JT - Diabetes care JID - 7805975 RN - 0 (Blood Glucose) SB - IM MH - Aged MH - Blood Flow Velocity/physiology MH - Blood Glucose/metabolism MH - Capillaries/chemistry/physiology MH - Female MH - Humans MH - Hypoglycemia/classification/*etiology/physiopathology MH - Raynaud Disease/*complications/physiopathology MH - Terminology as Topic EDAT- 2014/04/24 06:00 MHDA- 2015/07/28 06:00 CRDT- 2014/04/24 06:00 PHST- 2014/04/24 06:00 [entrez] PHST- 2014/04/24 06:00 [pubmed] PHST- 2015/07/28 06:00 [medline] AID - 37/5/e85 [pii] AID - 10.2337/dc13-2891 [doi] PST - ppublish SO - Diabetes Care. 2014;37(5):e85-6. doi: 10.2337/dc13-2891. PMID- 17265503 OWN - NLM STAT- MEDLINE DCOM- 20070309 LR - 20070222 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 2 DP - 2007 Feb TI - Clinical images: blue-purple ears. PG - 673 FAU - Martens, H A AU - Martens HA AD - University Medical Center Groningen, Groningen, The Netherlands. FAU - Bijl, M AU - Bijl M LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Aged MH - Biopsy MH - Bone Marrow/pathology MH - Cryoglobulinemia/*complications/etiology/pathology MH - Cyanosis/*diagnosis/*etiology/pathology MH - Diagnosis, Differential MH - Ear, External/*pathology MH - Female MH - Humans MH - Multiple Myeloma/*complications/diagnosis MH - Raynaud Disease/diagnosis/pathology EDAT- 2007/02/01 09:00 MHDA- 2007/03/10 09:00 CRDT- 2007/02/01 09:00 PHST- 2007/02/01 09:00 [pubmed] PHST- 2007/03/10 09:00 [medline] PHST- 2007/02/01 09:00 [entrez] AID - 10.1002/art.22365 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Feb;56(2):673. doi: 10.1002/art.22365. PMID- 30980719 OWN - NLM STAT- MEDLINE DCOM- 20200423 LR - 20200423 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 69 IP - 2 DP - 2019 Apr 13 TI - The gap between evidence on hand-arm vibration hazards and risk management. PG - 80-82 LID - 10.1093/occmed/kqy158 [doi] FAU - Nilsson, Tohr AU - Nilsson T AD - Occupational and Environmental Medicine, Department of Public Health & Clinical Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Editorial PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Arm/blood supply/innervation MH - Cumulative Trauma Disorders/*physiopathology MH - Energy Transfer/physiology MH - Hand/blood supply/innervation MH - Humans MH - Occupational Diseases/etiology/*physiopathology MH - Raynaud Disease/etiology/*physiopathology MH - Risk Management MH - Vibration/*adverse effects EDAT- 2019/04/14 06:00 MHDA- 2020/04/24 06:00 CRDT- 2019/04/14 06:00 PHST- 2019/04/14 06:00 [entrez] PHST- 2019/04/14 06:00 [pubmed] PHST- 2020/04/24 06:00 [medline] AID - 5455122 [pii] AID - 10.1093/occmed/kqy158 [doi] PST - ppublish SO - Occup Med (Lond). 2019 Apr 13;69(2):80-82. doi: 10.1093/occmed/kqy158. PMID- 16950937 OWN - NLM STAT- MEDLINE DCOM- 20061114 LR - 20190608 IS - 1526-3347 (Electronic) IS - 0191-9601 (Linking) VI - 27 IP - 9 DP - 2006 Sep TI - Systemic lupus erythematosus in children and adolescents. PG - 323-30 FAU - Gottlieb, Beth S AU - Gottlieb BS AD - Albert Einstein College of Medicine, NY, USA. FAU - Ilowite, Norman T AU - Ilowite NT LA - eng PT - Journal Article PT - Review PL - United States TA - Pediatr Rev JT - Pediatrics in review JID - 8103046 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Antibodies, Antinuclear/blood MH - Child MH - Child, Preschool MH - Humans MH - Incidence MH - Lupus Erythematosus, Systemic/diagnosis/epidemiology/immunology/*therapy MH - Raynaud Disease/diagnosis/immunology RF - 6 EDAT- 2006/09/05 09:00 MHDA- 2006/11/15 09:00 CRDT- 2006/09/05 09:00 PHST- 2006/09/05 09:00 [pubmed] PHST- 2006/11/15 09:00 [medline] PHST- 2006/09/05 09:00 [entrez] AID - 27/9/323 [pii] AID - 10.1542/pir.27-9-323 [doi] PST - ppublish SO - Pediatr Rev. 2006 Sep;27(9):323-30. doi: 10.1542/pir.27-9-323. PMID- 23666787 OWN - NLM STAT- MEDLINE DCOM- 20131017 LR - 20220330 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 65 IP - 8 DP - 2013 Aug TI - Review: evidence that systemic sclerosis is a vascular disease. PG - 1953-62 LID - 10.1002/art.37988 [doi] FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - University of Florence, Florence, Italy. FAU - Kahaleh, Bashar AU - Kahaleh B FAU - Wigley, Fredrick M AU - Wigley FM LA - eng PT - Journal Article PT - Review PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Fibrosis/complications/drug therapy/pathology MH - Humans MH - Molecular Targeted Therapy MH - Raynaud Disease/complications/pathology MH - Scleroderma, Systemic/complications/drug therapy/*pathology MH - Skin/blood supply MH - Vascular Diseases/complications/*pathology MH - Vasculitis/complications/pathology EDAT- 2013/05/15 06:00 MHDA- 2013/10/18 06:00 CRDT- 2013/05/14 06:00 PHST- 2012/10/08 00:00 [received] PHST- 2013/04/18 00:00 [accepted] PHST- 2013/05/14 06:00 [entrez] PHST- 2013/05/15 06:00 [pubmed] PHST- 2013/10/18 06:00 [medline] AID - 10.1002/art.37988 [doi] PST - ppublish SO - Arthritis Rheum. 2013 Aug;65(8):1953-62. doi: 10.1002/art.37988. PMID- 27749585 OWN - NLM STAT- MEDLINE DCOM- 20170209 LR - 20240326 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 95 IP - 40 DP - 2016 Oct TI - A case report of the beneficial effects of botulinum toxin type A on Raynaud phenomenon in a patient with lung cancer. PG - e5092 LID - 10.1097/MD.0000000000005092 [doi] LID - e5092 AB - OBJECTIVE: Raynaud phenomenon is a vasospastic disorder affecting the hands and feet, and the efficacies of traditional treatments, such as pharmacological therapies and sympathectomy, are not uniform. Patients with paraneoplastic Raynaud phenomenon do not benefit from the traditional treatments. The use of botulinum toxin type A (BTX-A) for Raynaud phenomenon has been reported for several years; however, there are few reports regarding botulinum toxin type A in the treatment of paraneoplastic Raynaud phenomenon. We describe a case report of the beneficial effects of botulinum toxin type A on Raynaud phenomenon in a patient with lung cancer. METHODS: A 63-year-old male complained of pain and discoloration of his fingers and indicated that oral nifedipine and low-dose aspirin were not effective. After approximately 8 months, he was diagnosed with lung cancer. Chemotherapy partially reduced the pain and discoloration of his fingers; however, no significant changes occurred in his fingers after the fourth cycle. We used BTX-A to treat this patient with paraneoplastic RP. A visual analogue scale (VAS) was used to assess the clinical response. RESULTS: After approximately 2 months, the patient reported relief from pain, stiffness, numbness, and cold sensation. Furthermore, no local or general adverse effects were exhibited by the patient. CONCLUSION: This study used botulinum toxin type A for a patient with paraneoplastic Raynaud phenomenon. Botulinum toxin type A significantly improved the patient's clinical symptoms without significant complications. These findings suggest that BTX-A may represent a good option for the treatment of paraneoplastic RP. FAU - Wang, Lu AU - Wang L AD - Department of Neurology, Tianjin Baodi Hospital, Tianjin Department of Neurology, The Second Hospital of Jilin University, Changchun, China. FAU - Lei, Qi-Song AU - Lei QS FAU - Liu, Yu-Ying AU - Liu YY FAU - Song, Guan-Jie AU - Song GJ FAU - Song, Chun-Ling AU - Song CL LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adenocarcinoma/*complications/diagnosis MH - Biopsy MH - Botulinum Toxins, Type A/*therapeutic use MH - Follow-Up Studies MH - Humans MH - Lung Neoplasms/*complications/diagnosis MH - Male MH - Middle Aged MH - Neuromuscular Agents/therapeutic use MH - Raynaud Disease/complications/diagnosis/*drug therapy MH - Skin/pathology MH - Tomography, X-Ray Computed PMC - PMC5059088 COIS- The authors have no conflicts of interest to disclose. EDAT- 2016/10/18 06:00 MHDA- 2017/02/10 06:00 PMCR- 2016/10/07 CRDT- 2016/10/18 06:00 PHST- 2016/10/18 06:00 [pubmed] PHST- 2017/02/10 06:00 [medline] PHST- 2016/10/18 06:00 [entrez] PHST- 2016/10/07 00:00 [pmc-release] AID - 00005792-201610040-00067 [pii] AID - 10.1097/MD.0000000000005092 [doi] PST - ppublish SO - Medicine (Baltimore). 2016 Oct;95(40):e5092. doi: 10.1097/MD.0000000000005092. PMID- 28329526 OWN - NLM STAT- MEDLINE DCOM- 20171017 LR - 20171017 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 23 IP - 3 DP - 2017 Mar 15 TI - Chronic granulomatous disease as a risk factor for cutaneous lupus in childhood. LID - 13030/qt2j6819c9 [pii] AB - Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies. FAU - Carvalho, Sandrina AU - Carvalho S AD - Department of Dermatology, Centro Hospitalar do Porto, Oporto, Portugal. carvalhosandrine@gmail.com. FAU - Machado, Susana AU - Machado S FAU - Sampaio, Rita AU - Sampaio R FAU - Guedes, Margarida AU - Guedes M FAU - Vasconcelos, Júlia AU - Vasconcelos J FAU - Semedo, Diogo AU - Semedo D FAU - Selores, Manuela AU - Selores M LA - eng PT - Case Reports PT - Journal Article DEP - 20170315 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Fungal) RN - 0 (anticentromere antibody) SB - IM MH - Antibodies, Antinuclear/immunology MH - Antibodies, Fungal/immunology MH - Cheilitis/complications/diagnosis/immunology MH - Child MH - Facial Dermatoses/complications/*diagnosis/immunology/pathology MH - Foot Dermatoses/complications/*diagnosis/immunology/pathology MH - Granulomatous Disease, Chronic/complications/*immunology MH - Humans MH - Lupus Erythematosus, Cutaneous/complications/*diagnosis/immunology/pathology MH - Male MH - Oral Ulcer/complications/diagnosis/immunology MH - Raynaud Disease/complications/diagnosis/immunology MH - Risk Factors MH - Saccharomyces cerevisiae/immunology EDAT- 2017/03/23 06:00 MHDA- 2017/10/19 06:00 CRDT- 2017/03/23 06:00 PHST- 2017/03/21 00:00 [received] PHST- 2017/03/21 00:00 [accepted] PHST- 2017/03/23 06:00 [entrez] PHST- 2017/03/23 06:00 [pubmed] PHST- 2017/10/19 06:00 [medline] AID - 13030/qt2j6819c9 [pii] PST - epublish SO - Dermatol Online J. 2017 Mar 15;23(3):13030/qt2j6819c9. PMID- 22508867 OWN - NLM STAT- MEDLINE DCOM- 20120604 LR - 20120417 IS - 1538-3652 (Electronic) IS - 0003-987X (Linking) VI - 148 IP - 4 DP - 2012 Apr TI - Botulinum toxin-A for the treatment of Raynaud syndrome. PG - 426-8 LID - 10.1001/archdermatol.2011.1144 [doi] FAU - Smith, Lauren AU - Smith L AD - The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 550 First Ave, New York, NY 10016, USA. FAU - Polsky, David AU - Polsky D FAU - Franks, Andrew G Jr AU - Franks AG Jr LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Arthritis, Rheumatoid/complications MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Fingers/blood supply/innervation MH - Humans MH - Lupus Erythematosus, Systemic/complications MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/complications/*drug therapy MH - Skin Ulcer/etiology EDAT- 2012/04/18 06:00 MHDA- 2012/06/05 06:00 CRDT- 2012/04/18 06:00 PHST- 2012/04/18 06:00 [entrez] PHST- 2012/04/18 06:00 [pubmed] PHST- 2012/06/05 06:00 [medline] AID - 148/4/426 [pii] AID - 10.1001/archdermatol.2011.1144 [doi] PST - ppublish SO - Arch Dermatol. 2012 Apr;148(4):426-8. doi: 10.1001/archdermatol.2011.1144. PMID- 30969221 OWN - NLM STAT- MEDLINE DCOM- 20200218 LR - 20210108 IS - 1873-233X (Electronic) IS - 0029-7844 (Linking) VI - 133 IP - 5 DP - 2019 May TI - Raynaud Phenomenon of the Nipple: An Under-Recognized Condition. PG - 975-977 LID - 10.1097/AOG.0000000000003219 [doi] AB - BACKGROUND: Obstetricians often feel ill-equipped to address the symptom of breast pain in pregnant and postpartum patients. CASES: In the first case, a 40-year-old woman in the second trimester of pregnancy reported nipple discoloration and severe pain. She was treated with nifedipine, and her symptoms decreased quickly and markedly. In the second case, a 32-year-old woman presented for a routine postpartum visit. She described breast pain and sporadic purple discoloration of the nipples, a finding confirmed on examination. Conservative measures of maintaining warmth were recommended. CONCLUSION: Raynaud phenomenon of the nipple is an underdiagnosed condition affecting women in both the prenatal and postpartum periods. A careful history and physical must be obtained in women presenting with breast pain, because diagnosis and treatment allows breastfeeding continuation and mitigation of symptoms. FAU - Jansen, Sierra AU - Jansen S AD - Department of Obstetrics and Gynecology, University of Wisconsin School of Medicine and Public Health, Meriter Hospital, and the Division of General Obstetrics and Gynecology, Department of Obstetrics and Gynecology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin. FAU - Sampene, Katherine AU - Sampene K LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Obstet Gynecol JT - Obstetrics and gynecology JID - 0401101 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Diseases/complications/*diagnosis/drug therapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Nifedipine/*therapeutic use MH - *Nipples MH - Pain/etiology MH - Pregnancy MH - *Prenatal Diagnosis MH - Puerperal Disorders/*diagnosis/drug therapy MH - Raynaud Disease/complications/*diagnosis/drug therapy MH - Vasodilator Agents/*therapeutic use EDAT- 2019/04/11 06:00 MHDA- 2020/02/19 06:00 CRDT- 2019/04/11 06:00 PHST- 2019/04/11 06:00 [pubmed] PHST- 2020/02/19 06:00 [medline] PHST- 2019/04/11 06:00 [entrez] AID - 00006250-201905000-00018 [pii] AID - 10.1097/AOG.0000000000003219 [doi] PST - ppublish SO - Obstet Gynecol. 2019 May;133(5):975-977. doi: 10.1097/AOG.0000000000003219. PMID- 33831463 OWN - NLM STAT- MEDLINE DCOM- 20211012 LR - 20240226 IS - 1872-7573 (Electronic) IS - 0378-8741 (Linking) VI - 275 DP - 2021 Jul 15 TI - Discovery of potential pharmacodynamic ingredients of Dang-Gui-Si-Ni decoction based on absorbed ingredients and molecular docking. PG - 114045 LID - S0378-8741(21)00272-5 [pii] LID - 10.1016/j.jep.2021.114045 [doi] AB - ETHNOPHARMACOLOGICAL RELEVANCE: The Dang-Gui-Si-Ni (DGSN) decoction as a classic prescription has been widely used for thousands of years in the clinical practice of traditional Chinese medicine (TCM). Especially in recent years, the potential efficacy of TCM for the treatment of Raynaud's syndrome has attracted great attention as there are still no specific remedies for this disease. However, the active constituents and underlying mechanisms responsible for the therapeutic benefits are not well understood, which makes it difficult to ensure quality control or to design research and drug development strategies. To identify the potential pharmacodynamic ingredients (PPIs) of TCM will help to achieve suitable process control procedures for industrial production and large-scale manufacturing. AIM OF THE STUDY: In the present study, we propose a multi-dimensional qualitative analysis method combining water-decoction spectra, in-vitro intestinal absorption spectra, in-vivo plasma spectra, and molecular docking of components to quickly identify the PPIs for the DGSN decoction of TCM. MATERIALS AND METHODS: Water-based decoctions of DGSN were prepared in accordance with the clinical use registered in ancient books. Ultra-high-performance liquid chromatography-quadrupole-time of flight mass spectrometry (UHPLC-Q/TOF-MS) coupled with computerized modelling activity screening was used to quickly identify the PPIs of the DGSN decoction. Bioactive compounds absorbed in vitro were identified using the everted intestinal sac model from rats and compounds absorbed in vivo were confirmed in portal vein blood samples obtained following oral administration in rats. Molecular docking validation experiments were adopted to predict the binding activity to coagulation factors I, II, VII, X, and IX. The active components were further confirmed by pharmacodynamics analysis. The anticoagulant activity of the DGSN decoction was verified using rat models. RESULTS: Thirty-one compounds were identified in the DGSN decoction. According to the in vivo experiments, 22 compounds that could be absorbed in vivo were detected by the everted intestinal sac model in rats. This model greatly reduces the scope of PPIs and is easy to perform. Ten compounds were detected in the portal vein blood in rats. The compounds detected in plasma provide stronger evidence supporting the PPIs. Molecular docking in vitro experiments indicated that 7 compounds exhibited better binding activity with coagulation factors I, II, VII, X, and IX. The animal experiments confirmed that the DGSN decoction could improve the microcirculation, providing indirect proof of anticoagulant activity suggested by the molecular docking studies. Finally, based on the multi-dimensional methods, 9 potential compounds present in the DGSN decoction were identified as PPIs (i.e., ferulic acid, paeoniflorin, albiflorin, chlorogenic acid, cryptochlorogenic acid, liquiritin, liquiritin apioside, cinnamaldehyde and glycyrrhizic acid). CONCLUSION: Overall, this study combined the water-decoction spectra, intestinal absorption spectra in vitro, plasma spectra in vivo, and molecular docking studies to establish a multi-dimensional qualitative analysis method of the DGSN decoction. Meanwhile, 9 compounds in DGSN decoction were identified as PPIs using this method, and are proposed for application as quality standards for complex TCM prescriptions. CI - Copyright © 2021 Elsevier B.V. All rights reserved. FAU - Li, Yun AU - Li Y AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China; School of Pharmaceutical Sciences, Shandong University of Traditional Chinese Medicine, Jinan, 250355, China. FAU - Liu, Shan-Shan AU - Liu SS AD - Beijing Center for Physical and Chemical Analysis, Beijing, 100089, China. FAU - Guo, Zhong-Yuan AU - Guo ZY AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China; College of Pharmacy, Henan University of Chinese Medicine, Henan, 450000, China. FAU - Yi, Hong AU - Yi H AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Li, Chun AU - Li C AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Chen, Liang-Mian AU - Chen LM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Gao, Hui-Min AU - Gao HM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Yan, Li-Hua AU - Yan LH AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Zhang, Wen-Wen AU - Zhang WW AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Feng, Xia-Xia AU - Feng XX AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Zhao, Jing-Yuan AU - Zhao JY AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Liu, Xiao-Qian AU - Liu XQ AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. Electronic address: lianyu1127@126.com. FAU - Wang, Zhi-Min AU - Wang ZM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. Electronic address: zmwang@icmm.ac.cn. LA - eng PT - Journal Article DEP - 20210405 PL - Ireland TA - J Ethnopharmacol JT - Journal of ethnopharmacology JID - 7903310 RN - 0 (Blood Coagulation Factors) RN - 0 (Drugs, Chinese Herbal) RN - 0 (Flavonoids) RN - 0 (Hydroxybenzoates) RN - 0 (Nucleosides) RN - 0 (Terpenes) RN - I3P9R8317T (phenolic acid) SB - IM MH - Administration, Oral MH - Animals MH - Blood Coagulation Factors/chemistry MH - Disease Models, Animal MH - Drugs, Chinese Herbal/administration & dosage/*chemistry/metabolism/*pharmacology MH - Flavonoids/analysis/chemistry MH - Hydroxybenzoates/analysis/chemistry MH - Intestinal Absorption MH - Male MH - Medicine, Chinese Traditional MH - Microcirculation/drug effects MH - Molecular Docking Simulation MH - Nucleosides/analysis/chemistry MH - Plasma/chemistry MH - Rats, Sprague-Dawley MH - Raynaud Disease/drug therapy MH - Terpenes/analysis/chemistry MH - Rats OTO - NOTNLM OT - Coagulation factor OT - Dang-Gui-Si-Ni (DGSN) decoction OT - Improving microcirculation OT - Molecular docking OT - Potential pharmacodynamic ingredients OT - UHPLC-Q/TOF-MS EDAT- 2021/04/09 06:00 MHDA- 2021/10/13 06:00 CRDT- 2021/04/08 20:17 PHST- 2020/06/07 00:00 [received] PHST- 2021/03/11 00:00 [revised] PHST- 2021/03/13 00:00 [accepted] PHST- 2021/04/09 06:00 [pubmed] PHST- 2021/10/13 06:00 [medline] PHST- 2021/04/08 20:17 [entrez] AID - S0378-8741(21)00272-5 [pii] AID - 10.1016/j.jep.2021.114045 [doi] PST - ppublish SO - J Ethnopharmacol. 2021 Jul 15;275:114045. doi: 10.1016/j.jep.2021.114045. Epub 2021 Apr 5. PMID- 30035365 OWN - NLM STAT- MEDLINE DCOM- 20191031 LR - 20191031 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 71 IP - 1 DP - 2019 Jan TI - Brief Report: How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria. PG - 91-98 LID - 10.1002/art.40674 [doi] AB - OBJECTIVE: Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. METHODS: Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. RESULTS: Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-β(2) -glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). CONCLUSION: In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria. CI - © 2018, American College of Rheumatology. FAU - Mosca, Marta AU - Mosca M AD - University of Pisa, Pisa, Italy. FAU - Costenbader, Karen H AU - Costenbader KH AD - Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts. FAU - Johnson, Sindhu R AU - Johnson SR AD - Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. FAU - Lorenzoni, Valentina AU - Lorenzoni V AD - Scuola Superiore Sant'Anna, Pisa, Italy. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AUID- ORCID: 0000-0003-2969-6649 AD - Azienda Ospedaliera San Camillo-Forlanini, Rome, Italy. FAU - Hoyer, Bimba F AU - Hoyer BF AD - Christian-Albrechts-University and University Hospital Schleswig-Holstein, Kiel, Germany. FAU - Navarra, Sandra AU - Navarra S AD - University of Santo Tomas, Manila, Philippines. FAU - Bonfa, Eloisa AU - Bonfa E AUID- ORCID: 0000-0002-0520-4681 AD - Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil. FAU - Ramsey-Goldman, Rosalind AU - Ramsey-Goldman R AD - Northwestern University Feinberg School of Medicine, Chicago, Illinois. FAU - Medina-Rosas, Jorge AU - Medina-Rosas J AD - Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. FAU - Piga, Matteo AU - Piga M AD - AOU University Clinic of Cagliari, Cagliari, Italy. FAU - Tani, Chiara AU - Tani C AD - University of Pisa, Pisa, Italy. FAU - Tedeschi, Sara K AU - Tedeschi SK AD - Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts. FAU - Dörner, Thomas AU - Dörner T AD - Charité University Medicine Berlin and Deutsches Rheuma-Forschungszentrum, Berlin, Germany. FAU - Aringer, Martin AU - Aringer M AD - University Medical Center and Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. FAU - Touma, Zahi AU - Touma Z AD - Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20181126 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (beta 2-Glycoprotein I) RN - 9007-36-7 (Complement System Proteins) RN - 9007-49-2 (DNA) SB - IM MH - Adult MH - Anemia, Hemolytic, Autoimmune/etiology MH - Antibodies, Antinuclear MH - Antiphospholipid Syndrome/diagnosis MH - Arthritis, Rheumatoid/diagnosis MH - Autoantibodies/immunology MH - Autoimmune Diseases/*diagnosis MH - Cohort Studies MH - Complement System Proteins/immunology MH - Coombs Test MH - DNA/immunology MH - Deglutition Disorders/etiology MH - Diagnosis, Differential MH - Female MH - Fever of Unknown Origin/etiology MH - Hepatitis, Autoimmune/diagnosis MH - Humans MH - Leukopenia/etiology MH - Lung Diseases, Interstitial/diagnosis MH - Lupus Erythematosus, Systemic/classification/complications/*diagnosis/immunology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/diagnosis MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/diagnosis MH - Sjogren's Syndrome/*diagnosis/etiology MH - Thyroiditis, Autoimmune/diagnosis MH - Undifferentiated Connective Tissue Diseases/diagnosis MH - Young Adult MH - beta 2-Glycoprotein I/immunology EDAT- 2018/07/24 06:00 MHDA- 2019/11/02 06:00 CRDT- 2018/07/24 06:00 PHST- 2018/02/01 00:00 [received] PHST- 2018/07/17 00:00 [accepted] PHST- 2018/07/24 06:00 [pubmed] PHST- 2019/11/02 06:00 [medline] PHST- 2018/07/24 06:00 [entrez] AID - 10.1002/art.40674 [doi] PST - ppublish SO - Arthritis Rheumatol. 2019 Jan;71(1):91-98. doi: 10.1002/art.40674. Epub 2018 Nov 26. PMID- 31389817 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20221005 IS - 1536-7355 (Electronic) IS - 1076-1608 (Print) IS - 1076-1608 (Linking) VI - 26 IP - 7 DP - 2020 Oct TI - Cyanosis With Dysautonomia Mimics Raynaud Disease. PG - e254-e255 LID - 10.1097/RHU.0000000000001107 [doi] FAU - Soloway, Alyxandra Morgan AU - Soloway AM AD - From the American University of the Caribbean, Coral Gables, FL. FAU - DePace, Nicholas L AU - DePace NL AD - Pennsylvania Hospital of the University of Pennsylvania Health System, Pennsylvania, PA. FAU - Colombo, Joseph AU - Colombo J AD - Parasympathetic and Sympathetic Nervous System Consultant, Franklin Cardiovascular Associates, Pa, Sewell. FAU - Soloway, Stephen AU - Soloway S AD - Stephen Soloway, Rheumatology, Vineland, NJ. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Cyanosis/diagnosis/etiology MH - Humans MH - *Primary Dysautonomias MH - *Raynaud Disease/diagnosis PMC - PMC7523574 COIS- The authors declare no conflict of interest. EDAT- 2019/08/08 06:00 MHDA- 2021/06/24 06:00 PMCR- 2020/09/29 CRDT- 2019/08/08 06:00 PHST- 2019/08/08 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] PHST- 2019/08/08 06:00 [entrez] PHST- 2020/09/29 00:00 [pmc-release] AID - 00124743-202010000-00028 [pii] AID - RHU50855 [pii] AID - 10.1097/RHU.0000000000001107 [doi] PST - ppublish SO - J Clin Rheumatol. 2020 Oct;26(7):e254-e255. doi: 10.1097/RHU.0000000000001107. PMID- 18982724 OWN - NLM STAT- MEDLINE DCOM- 20081124 LR - 20161018 IS - 0023-2130 (Print) IS - 0023-2130 (Linking) IP - 6 DP - 2008 Jun TI - [The treatment of neurospastic vascular diseases in Raynaud phenomenon]. PG - 36-9 AB - The efficacy of proposed methods for cardiovascular diseases with Raynaud phenomenon manifestations treatment was estimated. After application of the author method the patients extremities temperature had raised by 2 .50 degreesC. They had noted the pain and the extremities oedema elimination, trophic ulcers healing. According to Doppler investigation data the blood flow linear speed increase by 30 0% in comparison with pretreatment one was noted. The posttreatment complications were absent. Fair and good results were observed in 306 (82.3%) patients, in 21 (5.6%) patients the treatment was repeated in conditions of severe ischemia and the blood flow decompensation present. In 14 (3.8%) patients a high epidural blockade of sympathic nodes in portion of high thoracic and lower cervical vertebra according to the author method was performed. In far-remote period (in 3-10 years) the positive effect was prolonged in 298 (80.1%) patients. FAU - Buryĭ, V T AU - Buryĭ VT FAU - Buryĭ, V V AU - Buryĭ VV LA - ukr PT - Clinical Trial PT - English Abstract PT - Journal Article PL - Ukraine TA - Klin Khir JT - Klinichna khirurhiia JID - 9516872 RN - 0 (Vasodilator Agents) SB - IM MH - *Autonomic Nervous System/physiology MH - Cardiovascular Diseases/complications/physiopathology/*therapy MH - Electric Stimulation Therapy/*methods MH - Female MH - Foot/blood supply/innervation MH - Hand/blood supply/innervation MH - Humans MH - Male MH - *Radiation MH - Raynaud Disease/complications/physiopathology/*therapy MH - Regional Blood Flow/drug effects MH - Treatment Outcome MH - *Vasoconstriction/drug effects/physiology MH - Vasodilator Agents/administration & dosage/therapeutic use EDAT- 2008/11/06 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/11/06 09:00 PHST- 2008/11/06 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/11/06 09:00 [entrez] PST - ppublish SO - Klin Khir. 2008 Jun;(6):36-9. PMID- 31507130 OWN - NLM STAT- MEDLINE DCOM- 20191104 LR - 20191104 IS - 1565-1088 (Print) VI - 21 IP - 7 DP - 2019 Jul TI - Nailfold Video Capillaroscopy: Beyond Systemic Sclerosis. PG - 499-502 FAU - Rimar, Doron AU - Rimar D AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Rimar, Ori AU - Rimar O AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Rosner, Itzhak AU - Rosner I AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Rozenbaum, Michael AU - Rozenbaum M AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Kaly, Lisa AU - Kaly L AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Boulman, Nina AU - Boulman N AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. FAU - Slobodin, Gleb AU - Slobodin G AD - Rheumatology Unit, Bnai Zion Medical Center, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. LA - eng PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 SB - IM MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/pathology MH - Scleroderma, Systemic/*diagnosis/pathology MH - Vasculitis/diagnosis/pathology EDAT- 2019/09/12 06:00 MHDA- 2019/11/05 06:00 CRDT- 2019/09/12 06:00 PHST- 2019/09/12 06:00 [entrez] PHST- 2019/09/12 06:00 [pubmed] PHST- 2019/11/05 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2019 Jul;21(7):499-502. PMID- 17958022 OWN - NLM STAT- MEDLINE DCOM- 20071218 LR - 20071025 IS - 0035-3639 (Print) IS - 0035-3639 (Linking) VI - 28 IP - 4 DP - 2007 Sep TI - [When thinking to scleroderma?]. PG - 285-9 AB - Scleroderma encompasses an heterogeneous group of autoimmune disorders characterized by an hidebound thickened skin involvement. When the changes are limited to the skin, localized scleroderma is suspected. Although the latter is most often a benign disease, it may be exceptionally associated with involvement of multiple organs, mainly the neurological system. At the opposite, systemic sclerosis is a serious disorder associated with high morbidity and even mortality and defined by an extended skin sclerosis, multiple organ involvement and general symptoms. Raynaud phenomena is nearly always present at the beginning of the disease. Identifying initial manifestations of the disease (Raynaud phenomena, diffuse non pitting edema, symmetrical polyarthritis with tendon friction rubs, dysphagia associated with mucosal telangiectasia) may allow the clinician to rapidly transfer the patient to a specialized reference center in order to organize a multidisciplinary approach and to prompt optimum therapy. FAU - Cogan, E AU - Cogan E AD - Service de Médecine Interne, Hôpital Erasme, Bruxelles. LA - fre PT - English Abstract PT - Journal Article TT - Quand penser à une sclérodermie? PL - Belgium TA - Rev Med Brux JT - Revue medicale de Bruxelles JID - 8003474 SB - IM MH - Arthritis/etiology MH - Autoimmune Diseases/diagnosis MH - Diagnosis, Differential MH - Humans MH - Lung Diseases/etiology MH - Raynaud Disease/diagnosis/physiopathology MH - Scleroderma, Localized/diagnosis MH - Scleroderma, Systemic/classification/*diagnosis/mortality/physiopathology EDAT- 2007/10/26 09:00 MHDA- 2007/12/19 09:00 CRDT- 2007/10/26 09:00 PHST- 2007/10/26 09:00 [pubmed] PHST- 2007/12/19 09:00 [medline] PHST- 2007/10/26 09:00 [entrez] PST - ppublish SO - Rev Med Brux. 2007 Sep;28(4):285-9. PMID- 34364313 OWN - NLM STAT- MEDLINE DCOM- 20220214 LR - 20260513 IS - 1526-4610 (Electronic) IS - 0017-8748 (Linking) VI - 61 IP - 8 DP - 2021 Sep TI - The evolving understanding of risk with calcitonin gene-related peptide monoclonal antibodies based on real-world data: A focus on hypertension and Raynaud phenomenon. PG - 1274-1276 LID - 10.1111/head.14198 [doi] FAU - Breen, Ilana D AU - Breen ID AD - Mayo Clinic Alix School of Medicine, Scottsdale, AZ, USA. FAU - Mangold, Aaron R AU - Mangold AR AD - Dermatology, Mayo Clinic Arizona, Scottsdale, AZ, USA. FAU - VanderPluym, Juliana H AU - VanderPluym JH AD - Neurology, Mayo Clinic Arizona, Scottsdale, AZ, USA. LA - eng PT - Journal Article DEP - 20210807 PL - United States TA - Headache JT - Headache JID - 2985091R RN - 0 (Antibodies, Monoclonal) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Adult MH - Antibodies, Monoclonal/adverse effects MH - Calcitonin Gene-Related Peptide/immunology MH - Calcitonin Gene-Related Peptide Receptor Antagonists/*adverse effects MH - Comorbidity MH - Female MH - Humans MH - Hypertension/*chemically induced/epidemiology MH - Male MH - Middle Aged MH - Migraine Disorders/*drug therapy/epidemiology MH - Raynaud Disease/*chemically induced/epidemiology MH - Risk MH - *Symptom Flare Up OTO - NOTNLM OT - Raynaud phenomenon OT - adverse effects OT - calcitonin gene-related peptide OT - hypertension OT - monoclonal antibody OT - risk EDAT- 2021/08/08 06:00 MHDA- 2022/02/15 06:00 CRDT- 2021/08/07 20:30 PHST- 2021/06/14 00:00 [revised] PHST- 2021/05/17 00:00 [received] PHST- 2021/06/29 00:00 [accepted] PHST- 2021/08/08 06:00 [pubmed] PHST- 2022/02/15 06:00 [medline] PHST- 2021/08/07 20:30 [entrez] AID - 10.1111/head.14198 [doi] PST - ppublish SO - Headache. 2021 Sep;61(8):1274-1276. doi: 10.1111/head.14198. Epub 2021 Aug 7. PMID- 25536485 OWN - NLM STAT- MEDLINE DCOM- 20160112 LR - 20220410 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 11 IP - 3 DP - 2015 Mar TI - A vascular mechanistic approach to understanding Raynaud phenomenon. PG - 146-58 LID - 10.1038/nrrheum.2014.195 [doi] AB - During exposure to cold, our bodies attempt to maintain normal core temperature by restricting heat loss through cutaneous vasoconstriction, and by increasing heat production through shivering and nonshivering thermogenesis. In selected areas of human skin (including on the fingers and toes), the vascular system has specialized structural and functional features that enable it to contribute to thermoregulation. These features include arteriovenous anastomoses, which directly connect the arterial and venous systems and bypass the nutritional capillaries supplying blood to the skin tissue. Of note, Raynaud phenomenon predominantly affects the arterial territories supplying these specialized areas of skin. Indeed, Raynaud phenomenon can be considered a disorder of vascular thermoregulatory control. This Review presents an understanding of Raynaud phenomenon in the context of vascular and thermoregulatory control mechanisms, including the role of unique thermosensitive vascular structural and functional specialization, and describes the potential role of thermogenesis in this disorder. This new approach provides remarkable insight into the disease process and builds a framework to critically appraise the existing knowledge base. This paradigm also explains the deficiencies in some current therapeutic approaches, and highlights new areas of potential relevance to the pathogenesis and treatment of Raynaud phenomenon that should be expanded and explored. FAU - Flavahan, Nicholas A AU - Flavahan NA AD - Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Ross Building, R1159, 720 Rutland Avenue, Baltimore, MD 21205, USA. LA - eng PT - Journal Article PT - Review DEP - 20141223 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - Capillaries/physiology MH - Humans MH - Raynaud Disease/*physiopathology/therapy MH - Skin/*blood supply/physiopathology MH - Vasoconstriction/physiology EDAT- 2014/12/24 06:00 MHDA- 2016/01/13 06:00 CRDT- 2014/12/24 06:00 PHST- 2014/12/24 06:00 [entrez] PHST- 2014/12/24 06:00 [pubmed] PHST- 2016/01/13 06:00 [medline] AID - nrrheum.2014.195 [pii] AID - 10.1038/nrrheum.2014.195 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2015 Mar;11(3):146-58. doi: 10.1038/nrrheum.2014.195. Epub 2014 Dec 23. PMID- 31507129 OWN - NLM STAT- MEDLINE DCOM- 20191104 LR - 20191104 IS - 1565-1088 (Print) VI - 21 IP - 7 DP - 2019 Jul TI - Nailfold Capillaroscopy Patterns. PG - 497-498 FAU - Rossi, Daniela AU - Rossi D AD - Center of Research of Immunopathology and Rare Diseases (CMID) - Division of Nephrology and Dialysis (ERK-net member), St. Giovanni Bosco Hospital and University of Turin, Turin, Italy. FAU - Sciascia, Savino AU - Sciascia S AD - Center of Research of Immunopathology and Rare Diseases (CMID) - Division of Nephrology and Dialysis (ERK-net member), St. Giovanni Bosco Hospital and University of Turin, Turin, Italy. FAU - Roccatello, Dario AU - Roccatello D AD - Center of Research of Immunopathology and Rare Diseases (CMID) - Division of Nephrology and Dialysis (ERK-net member), St. Giovanni Bosco Hospital and University of Turin, Turin, Italy. LA - eng PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 SB - IM MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/pathology MH - Scleroderma, Systemic/*diagnosis/pathology EDAT- 2019/09/12 06:00 MHDA- 2019/11/05 06:00 CRDT- 2019/09/12 06:00 PHST- 2019/09/12 06:00 [entrez] PHST- 2019/09/12 06:00 [pubmed] PHST- 2019/11/05 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2019 Jul;21(7):497-498. PMID- 37888629 OWN - NLM STAT- MEDLINE DCOM- 20231030 LR - 20231112 IS - 2072-6651 (Electronic) IS - 2072-6651 (Linking) VI - 15 IP - 10 DP - 2023 Oct 4 TI - Cold Finger: Raynaud Phenomenon Following Snakebite Envenoming by Nikolsky's Viper (Vipera berus nikolskii). LID - 10.3390/toxins15100598 [doi] LID - 598 AB - A field biologist was bitten by a female Nikolsky's viper (Vipera berus nikolskii) in Kharkiv Oblast, Ukraine. Two months later, the patient began to experience cold-induced vasospasm of the affected digit diagnosed as acquired Raynaud phenomenon. The patient had more than 30 occurrences during the single winter following the bite, but the signs and symptoms of Raynaud phenomenon disappeared with the end of winter. This report describes the case and puts it into context with the literature on the topic of toxin-induced peripheral vasospastic disorders and their potential importance in snakebite envenoming. FAU - Zinenko, Oleksandr AU - Zinenko O AD - V. N. Karazin Kharkiv University, 61058 Kharkiv, Ukraine. AD - California Academy of Sciences, San Francisco, CA 94118, USA. FAU - Durkin, Daniela M AU - Durkin DM AD - California Academy of Sciences, San Francisco, CA 94118, USA. FAU - Carter, Rebecca W AU - Carter RW AUID- ORCID: 0000-0002-5977-7338 AD - Ophirex, Inc., Corte Madera, CA 94925, USA. FAU - Ritter, Brandi AU - Ritter B AD - Ophirex, Inc., Corte Madera, CA 94925, USA. FAU - Lewin, Matthew R AU - Lewin MR AUID- ORCID: 0000-0003-4480-9891 AD - California Academy of Sciences, San Francisco, CA 94118, USA. AD - Ophirex, Inc., Corte Madera, CA 94925, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20231004 PL - Switzerland TA - Toxins (Basel) JT - Toxins JID - 101530765 RN - 0 (Viper Venoms) RN - 0 (Antivenins) SB - IM MH - Animals MH - Humans MH - Female MH - *Snake Bites/complications MH - Viper Venoms/toxicity MH - *Viperidae MH - Upper Extremity MH - *Raynaud Disease/diagnosis/etiology MH - Antivenins PMC - PMC10610580 OTO - NOTNLM OT - Kharkiv OT - Nikolsky’s viper OT - Raynaud phenomenon OT - Ukraine OT - Vipera OT - chronic manifestations of snakebite OT - long-term disability OT - return to duty OT - snakebite OT - vasospasm COIS- The authors declare no conflict of interest. EDAT- 2023/10/27 12:43 MHDA- 2023/10/30 06:46 PMCR- 2023/10/04 CRDT- 2023/10/27 07:22 PHST- 2023/08/09 00:00 [received] PHST- 2023/09/13 00:00 [revised] PHST- 2023/09/14 00:00 [accepted] PHST- 2023/10/30 06:46 [medline] PHST- 2023/10/27 12:43 [pubmed] PHST- 2023/10/27 07:22 [entrez] PHST- 2023/10/04 00:00 [pmc-release] AID - toxins15100598 [pii] AID - toxins-15-00598 [pii] AID - 10.3390/toxins15100598 [doi] PST - epublish SO - Toxins (Basel). 2023 Oct 4;15(10):598. doi: 10.3390/toxins15100598. PMID- 22765239 OWN - NLM STAT- MEDLINE DCOM- 20121010 LR - 20220114 IS - 1365-2141 (Electronic) IS - 0007-1048 (Linking) VI - 158 IP - 4 DP - 2012 Aug TI - Raynaud-like phenomenon in two patients on nilotinib. PG - 431 LID - 10.1111/j.1365-2141.2012.09215.x [doi] FAU - Hazenberg, Carin L E AU - Hazenberg CL AD - Department of Internal Medicine, Medisch Spectrum Twente, Enschede, The Netherlands. FAU - Ossenkoppele, Gert J AU - Ossenkoppele GJ FAU - Smit, Willem M AU - Smit WM LA - eng PT - Case Reports PT - Journal Article DEP - 20120705 PL - England TA - Br J Haematol JT - British journal of haematology JID - 0372544 RN - 0 (Antineoplastic Agents) RN - 0 (Pyrimidines) RN - F41401512X (nilotinib) SB - IM MH - Adult MH - Antineoplastic Agents/*adverse effects/therapeutic use MH - Female MH - Humans MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy MH - Pyrimidines/*adverse effects/therapeutic use MH - Raynaud Disease/*chemically induced/pathology MH - Young Adult EDAT- 2012/07/07 06:00 MHDA- 2012/10/12 06:00 CRDT- 2012/07/07 06:00 PHST- 2012/07/07 06:00 [entrez] PHST- 2012/07/07 06:00 [pubmed] PHST- 2012/10/12 06:00 [medline] AID - 10.1111/j.1365-2141.2012.09215.x [doi] PST - ppublish SO - Br J Haematol. 2012 Aug;158(4):431. doi: 10.1111/j.1365-2141.2012.09215.x. Epub 2012 Jul 5. PMID- 24321162 OWN - NLM STAT- MEDLINE DCOM- 20141002 LR - 20151119 IS - 1872-6968 (Electronic) IS - 0303-8467 (Linking) VI - 115 Suppl 1 DP - 2013 Dec TI - Interferons beta have vasoconstrictive and procoagulant effects: a woman who developed livedo reticularis and Raynaud phenomenon in association with interferon beta treatment for multiple sclerosis. PG - S79-81 LID - S0303-8467(13)00381-8 [pii] LID - 10.1016/j.clineuro.2013.09.027 [doi] AB - A 31-year-old woman with MS developed livedo reticularis and secondary Raynaud phenomenon 2.5 years after introduction of interferon beta-1b. The symptoms disappeared after withdrawal of the drug. Livedo reticularis and Raynaud phenomenon as well as pulmonary arterial hypertension, venous sinus thrombosis, pulmonary embolism and renal thrombotic microangiopathy have all been described in association with interferon beta therapy. These complications strongly suggest that type I interferons have vasoconstrictive and procoagulant effects with potentially serious systemic complications. CI - Copyright © 2013 Elsevier B.V. All rights reserved. FAU - Rot, Uroš AU - Rot U AD - Department of Neurology, University Medical Centre, Zaloška cesta 2, 1525 Ljubljana, Slovenia. Electronic address: uros.rot@guest.arnes.si. FAU - Ledinek, Alenka Horvat AU - Ledinek AH LA - eng PT - Case Reports PT - Journal Article PL - Netherlands TA - Clin Neurol Neurosurg JT - Clinical neurology and neurosurgery JID - 7502039 RN - 145155-23-3 (Interferon beta-1b) RN - 77238-31-4 (Interferon-beta) SB - IM MH - Adult MH - Female MH - Humans MH - Interferon beta-1b MH - Interferon-beta/*adverse effects MH - Livedo Reticularis/*chemically induced/diagnosis/pathology MH - Multiple Sclerosis/*drug therapy MH - Raynaud Disease/*chemically induced/diagnosis MH - Treatment Outcome OTO - NOTNLM OT - Interferon beta OT - Livedo reticularis OT - Multiple sclerosis OT - Raynaud phenomenon OT - Side effects EDAT- 2014/01/01 06:00 MHDA- 2014/10/03 06:00 CRDT- 2013/12/11 06:00 PHST- 2013/12/11 06:00 [entrez] PHST- 2014/01/01 06:00 [pubmed] PHST- 2014/10/03 06:00 [medline] AID - S0303-8467(13)00381-8 [pii] AID - 10.1016/j.clineuro.2013.09.027 [doi] PST - ppublish SO - Clin Neurol Neurosurg. 2013 Dec;115 Suppl 1:S79-81. doi: 10.1016/j.clineuro.2013.09.027. PMID- 25447663 OWN - NLM STAT- MEDLINE DCOM- 20150519 LR - 20221207 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 53 IP - 23 DP - 2014 TI - A rare entity: RACAND syndrome. PG - 2749 FAU - Elqatni, Mohamed AU - Elqatni M AD - Department of Internal Medicine, Mohammed V Military Teaching Hospital, Mohammed V-Souissi University, Morocco. FAU - Mekouar, Fadwa AU - Mekouar F FAU - Amezyane, Taoufik AU - Amezyane T FAU - Ghafir, Driss AU - Ghafir D LA - eng PT - Case Reports PT - Journal Article DEP - 20141201 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (Platelet Aggregation Inhibitors) RN - 0 (anticentromere antibody) RN - 9PHQ9Y1OLM (Prednisolone) RN - JED5K35YGL (Iloprost) SB - IM MH - *Amputation, Surgical MH - Antibodies, Antinuclear/*metabolism MH - Female MH - Fingers/*pathology MH - Humans MH - Iloprost/therapeutic use MH - Middle Aged MH - Necrosis MH - Platelet Aggregation Inhibitors/therapeutic use MH - Prednisolone/therapeutic use MH - Raynaud Disease/*pathology/therapy MH - Severity of Illness Index MH - Syndrome MH - Toes/*pathology MH - Treatment Failure EDAT- 2014/12/03 06:00 MHDA- 2015/05/20 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/05/20 06:00 [medline] AID - DN/JST.JSTAGE/internalmedicine/53.2757 [pii] AID - 10.2169/internalmedicine.53.2757 [doi] PST - ppublish SO - Intern Med. 2014;53(23):2749. doi: 10.2169/internalmedicine.53.2757. Epub 2014 Dec 1. PMID- 17504680 OWN - NLM STAT- MEDLINE DCOM- 20071127 LR - 20190917 IS - 0014-2565 (Print) IS - 0014-2565 (Linking) VI - 207 IP - 5 DP - 2007 May TI - [Antisynthetase syndrome with good response to mycophenolate mofetil]. PG - 269-70 FAU - López de la Osa, A AU - López de la Osa A FAU - Sánchez Tapia, C AU - Sánchez Tapia C FAU - Arias Díaz, M AU - Arias Díaz M FAU - Terrancle de Juan, I AU - Terrancle de Juan I LA - spa PT - Case Reports PT - Letter TT - Síndrome antisintetasa con buena respuesta a micofenolato mofetilo. PL - Spain TA - Rev Clin Esp JT - Revista clinica espanola JID - 8608576 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) RN - HU9DX48N0T (Mycophenolic Acid) SB - IM EIN - Rev Clin Esp. 2007 Sep;207(8):415. Sanher Tapia, C [corrected to Sánchez Tapia, C]; Arias García, M [corrected to Arias Díaz, M] MH - Adult MH - Arthritis/*drug therapy/immunology MH - *Autoantibodies MH - Female MH - Histidine-tRNA Ligase/*immunology MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Mycophenolic Acid/*analogs & derivatives/therapeutic use MH - Polymyositis/*drug therapy/immunology MH - Raynaud Disease/*drug therapy/immunology MH - Remission Induction MH - Syndrome EDAT- 2007/05/17 09:00 MHDA- 2007/12/06 09:00 CRDT- 2007/05/17 09:00 PHST- 2007/05/17 09:00 [pubmed] PHST- 2007/12/06 09:00 [medline] PHST- 2007/05/17 09:00 [entrez] AID - 13102329 [pii] AID - 10.1157/13102329 [doi] PST - ppublish SO - Rev Clin Esp. 2007 May;207(5):269-70. doi: 10.1157/13102329. PMID- 28336160 OWN - NLM STAT- MEDLINE DCOM- 20171228 LR - 20260127 IS - 0040-5957 (Print) IS - 0040-5957 (Linking) VI - 72 IP - 5 DP - 2017 Oct TI - Unexpected drug-induced Raynaud phenomenon: Analysis from the French national pharmacovigilance database. PG - 547-554 LID - S0040-5957(17)30040-9 [pii] LID - 10.1016/j.therap.2017.01.008 [doi] AB - OBJECTIVE: To estimate the association between exposure to medicinal products and Raynaud phenomenon. METHODS: The study used the data of all adverse drug reactions notified to the French national pharmacovigilance database. All cases reported between 1st January 1995 and 10th December 2012 were selected. A case/non-case method was used to measure disproportionality of the association between drug exposure and Raynaud phenomenon. The cases concerned all observations involving Raynaud phenomenon. Non-cases comprised all other reports of adverse drug reactions over the same period. RESULTS/DISCUSSION: Among the 307,128 adverse drug reaction reports selected from the French national pharmacovigilance database, 175 involved Raynaud phenomenon, most of them affecting women (61.1%). The mean age was 50.1 years, and 8% had a past medical history of Raynaud phenomenon. According to the summaries of product characteristics, 42.3% of these cases were exposed to drugs known to induce Raynaud phenomenon. Unexpected Raynaud phenomenons (unlisted in the summaries of product characteristics) were associated with exposure to drugs for which Raynaud phenomenons are published (interferons, ribavirin, gemcitabine) or for which Raynaud phenomenons are not published (hepatitis B vaccine, isotretinoin, leflunomide, hydroxycarbamide, rofecoxib, telmisartan, zolmitriptan). CONCLUSION: The case/non-case method is usually used to generate signals. Further epidemiological studies are now necessary to confirm these findings. CI - Copyright © 2017 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved. FAU - Bouquet, Émilie AU - Bouquet É AD - Regional pharmacovigilance centre, Angers university hospital, 49933 Angers, France. Electronic address: bouquet.emilie@gmail.com. FAU - Urbanski, Geoffrey AU - Urbanski G AD - Department of internal and vascular medicine, Angers university hospital, 49933 Angers cedex 9, France. FAU - Lavigne, Christian AU - Lavigne C AD - Department of internal and vascular medicine, Angers university hospital, 49933 Angers cedex 9, France. FAU - Lainé-Cessac, Pascale AU - Lainé-Cessac P AD - Regional pharmacovigilance centre, Angers university hospital, 49933 Angers, France. LA - eng PT - Journal Article DEP - 20170222 PL - France TA - Therapie JT - Therapie JID - 0420544 SB - IM MH - Adverse Drug Reaction Reporting Systems MH - Databases, Factual MH - *Drug-Related Side Effects and Adverse Reactions MH - Female MH - France/epidemiology MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*chemically induced/*epidemiology OTO - NOTNLM OT - Drugs adverse effects OT - Effets indésirables médicamenteux OT - Pharmacovigilance OT - Phénomène de Raynaud OT - Raynaud phenomenon EDAT- 2017/03/25 06:00 MHDA- 2017/12/29 06:00 CRDT- 2017/03/25 06:00 PHST- 2016/06/11 00:00 [received] PHST- 2016/12/19 00:00 [revised] PHST- 2017/01/12 00:00 [accepted] PHST- 2017/03/25 06:00 [pubmed] PHST- 2017/12/29 06:00 [medline] PHST- 2017/03/25 06:00 [entrez] AID - S0040-5957(17)30040-9 [pii] AID - 10.1016/j.therap.2017.01.008 [doi] PST - ppublish SO - Therapie. 2017 Oct;72(5):547-554. doi: 10.1016/j.therap.2017.01.008. Epub 2017 Feb 22. PMID- 40878060 OWN - NLM STAT- MEDLINE DCOM- 20250829 LR - 20250904 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 26 DP - 2025 Aug 29 TI - Radial Periosteal Distraction as a Novel Intervention for Raynaud Syndrome with Gangrene: A Case Report. PG - e948422 LID - 10.12659/AJCR.948422 [doi] LID - e948422 AB - BACKGROUND Raynaud syndrome, also known as Raynaud phenomenon, is characterized by vasospasm of small blood vessels supplying the digits and is usually reversible but can rarely result in severe and irreversible ischemia. Periosteal distraction osteogenesis is a technique that creates a space between the periosteum and the bone surface to stimulate the formation of new bone. This report describes a 67-year-old woman with a 10-year history of Raynaud syndrome presenting with ischemia and gangrene of the right index and middle fingers managed with radial periosteal distraction osteogenesis. CASE REPORT Ten years earlier, the patient received a diagnosis of Raynaud syndrome and had been taking nifedipine sustained-release tablets orally for symptom control ever since. Two weeks before presentation, she successfully recovered from septic shock through the rescue of the Intensive Care Unit. One week before presentation, the patient experienced an aggravation of pain in both fingers. Her distal ends of the right index and middle fingers became darker. After performing the relevant examinations, she received a diagnosis of Raynaud syndrome (bilateral) and gangrene of the right finger. Following the exclusion of surgical contraindications, radial periosteal distraction was performed. The periosteum was continuously distracted after the operation. During follow-up, the patient's gangrene gradually progressively healed, and the pain in both hands markedly diminished. CONCLUSIONS This report supports recent studies that have shown the potential for periosteal distraction osteogenesis in the management of cases of severe and irreversible Raynaud disease of the digits. FAU - Zheng, Xiaoyu AU - Zheng X AD - School of Clinical Medicine, Chongqing Medical and Pharmaceutical College, Chongqing, China. FAU - Yang, Yang AU - Yang Y AD - Department of Orthopedics, The People's Hospital of Yubei District of Chongqing City, Chongqing, China. FAU - Jin, Hongliang AU - Jin H AD - Department of Orthopedics, The People's Hospital of Yubei District of Chongqing City, Chongqing, China. FAU - Chen, Yiguo AU - Chen Y AD - Department of Orthopedics, The People's Hospital of Yubei District of Chongqing City, Chongqing, China. FAU - Wang, Wei AU - Wang W AD - Department of Orthopedics, The People's Hospital of Yubei District of Chongqing City, Chongqing, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20250829 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - Humans MH - Female MH - *Raynaud Disease/complications/surgery MH - Aged MH - *Gangrene/surgery/etiology MH - *Fingers/pathology/surgery/blood supply MH - *Osteogenesis, Distraction/methods MH - *Periosteum/surgery PMC - PMC12404003 COIS- Conflict of interest: None declared EDAT- 2025/08/29 08:45 MHDA- 2025/09/03 02:19 PMCR- 2025/08/29 CRDT- 2025/08/29 01:22 PHST- 2025/09/03 02:19 [medline] PHST- 2025/08/29 08:45 [pubmed] PHST- 2025/08/29 01:22 [entrez] PHST- 2025/08/29 00:00 [pmc-release] AID - 948422 [pii] AID - 10.12659/AJCR.948422 [doi] PST - epublish SO - Am J Case Rep. 2025 Aug 29;26:e948422. doi: 10.12659/AJCR.948422. PMID- 17976138 OWN - NLM STAT- MEDLINE DCOM- 20071206 LR - 20071102 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 5 IP - 11 DP - 2007 Nov TI - Differential diagnosis of scleroderma and pseudoscleroderma. PG - 977-84 AB - The different forms of scleroderma and the pseudosclerodermas, which clinically partially imitate scleroderma, are rare. Due to the large variety and variability of the clinical course, particularly at the onset of disease, diagnosis may be difficult. For differential diagnosis, the presence of Raynaud phenomenon, antinuclear antibodies and the distribution of sclerosis play essential roles. Besides discussing the diseases that should be considered in the differential diagnosis, we present an algorithm which should facilitate the diagnosis and allow one to promptly initiate appropriate treatment. FAU - Fabri, Mario AU - Fabri M AD - Department of Dermatology, Venereology and Allergy, University of Cologne, Germany. FAU - Hunzelmann, Nicolas AU - Hunzelmann N LA - eng LA - ger PT - Journal Article PT - Review PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 SB - IM MH - *Algorithms MH - *Decision Support Systems, Clinical MH - Dermoscopy/*methods MH - Diagnosis, Differential MH - Humans MH - Raynaud Disease/classification/diagnosis MH - Scleroderma, Localized/*classification/*diagnosis MH - Scleroderma, Systemic/*classification/*diagnosis RF - 46 EDAT- 2007/11/03 09:00 MHDA- 2007/12/07 09:00 CRDT- 2007/11/03 09:00 PHST- 2007/11/03 09:00 [pubmed] PHST- 2007/12/07 09:00 [medline] PHST- 2007/11/03 09:00 [entrez] AID - DDG06311 [pii] AID - 10.1111/j.1610-0387.2007.06311.x [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2007 Nov;5(11):977-84. doi: 10.1111/j.1610-0387.2007.06311.x. PMID- 24796412 OWN - NLM STAT- MEDLINE DCOM- 20160930 LR - 20211021 IS - 0973-7693 (Electronic) IS - 0019-5456 (Linking) VI - 81 IP - 12 DP - 2014 Dec TI - Successfully treated fingertip necrosis in an infant with primary Raynaud phenomenon. PG - 1399-400 LID - 10.1007/s12098-014-1454-z [doi] FAU - Senel, Saliha AU - Senel S AD - Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital, Telsizler, 06080, Ankara, Turkey, drsaliha007@yahoo.com.tr. FAU - Zorlu, Pelin AU - Zorlu P FAU - Dogan, Burcin AU - Dogan B FAU - Acar, Mehtap AU - Acar M LA - eng PT - Case Reports PT - Letter DEP - 20140506 PL - India TA - Indian J Pediatr JT - Indian journal of pediatrics JID - 0417442 RN - 0 (Anticoagulants) RN - 0 (Enoxaparin) RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) RN - SD6QCT3TSU (Pentoxifylline) SB - IM MH - Anticoagulants/therapeutic use MH - Drug Therapy, Combination MH - Enoxaparin/therapeutic use MH - Fingers/*pathology MH - Humans MH - Infant MH - Male MH - Necrosis MH - Nifedipine/therapeutic use MH - Pentoxifylline/therapeutic use MH - Raynaud Disease/*complications/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2014/05/07 06:00 MHDA- 2016/10/01 06:00 CRDT- 2014/05/07 06:00 PHST- 2013/12/31 00:00 [received] PHST- 2014/04/07 00:00 [accepted] PHST- 2014/05/07 06:00 [entrez] PHST- 2014/05/07 06:00 [pubmed] PHST- 2016/10/01 06:00 [medline] AID - 10.1007/s12098-014-1454-z [doi] PST - ppublish SO - Indian J Pediatr. 2014 Dec;81(12):1399-400. doi: 10.1007/s12098-014-1454-z. Epub 2014 May 6. PMID- 22157263 OWN - NLM STAT- MEDLINE DCOM- 20120531 LR - 20181201 IS - 1097-6817 (Electronic) IS - 0194-5998 (Linking) VI - 146 IP - 4 DP - 2012 Apr TI - Dysphagia, oral telangiectasia, and Raynaud syndrome. PG - 676-7 LID - 10.1177/0194599811431060 [doi] FAU - Nisa, Lluís AU - Nisa L AD - Department of Otorhinolaryngology-Head & Neck Surgery, Hôpital de Sion-CHCVs-RSV, Avenue du Grand Champsec 80, 1950 Sion, Switzerland. llnisa@yahoo.ca FAU - Giger, Roland AU - Giger R LA - eng PT - Case Reports PT - Journal Article DEP - 20111207 PL - England TA - Otolaryngol Head Neck Surg JT - Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery JID - 8508176 RN - 0 (2-Pyridinylmethylsulfinylbenzimidazoles) RN - 0 (Anti-Ulcer Agents) RN - 0 (Immunosuppressive Agents) RN - D8TST4O562 (Pantoprazole) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - 2-Pyridinylmethylsulfinylbenzimidazoles/therapeutic use MH - Anti-Ulcer Agents/therapeutic use MH - CREST Syndrome/*diagnosis/drug therapy MH - Deglutition Disorders/diagnosis/drug therapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Methotrexate/therapeutic use MH - Middle Aged MH - Pantoprazole MH - Raynaud Disease/diagnosis/drug therapy MH - Telangiectasis/diagnosis/drug therapy EDAT- 2011/12/14 06:00 MHDA- 2012/06/01 06:00 CRDT- 2011/12/14 06:00 PHST- 2011/12/14 06:00 [entrez] PHST- 2011/12/14 06:00 [pubmed] PHST- 2012/06/01 06:00 [medline] AID - 0194599811431060 [pii] AID - 10.1177/0194599811431060 [doi] PST - ppublish SO - Otolaryngol Head Neck Surg. 2012 Apr;146(4):676-7. doi: 10.1177/0194599811431060. Epub 2011 Dec 7. PMID- 24047298 OWN - NLM STAT- MEDLINE DCOM- 20131113 LR - 20161125 IS - 1365-2044 (Electronic) IS - 0003-2409 (Linking) VI - 68 IP - 10 DP - 2013 Oct TI - Ultrasound-guided digital sympathectomy using botulinum toxin. PG - 1077 LID - 10.1111/anae.12416 [doi] FAU - Rajendram, R AU - Rajendram R AD - Royal Free Hospital, London, UK. rajkumarrajendram@doctors.org.uk. FAU - Hayward, A AU - Hayward A LA - eng PT - Letter PL - England TA - Anaesthesia JT - Anaesthesia JID - 0370524 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - Airway Management MH - Arteries/diagnostic imaging MH - Botulinum Toxins/administration & dosage/*therapeutic use MH - Hand/*blood supply/*diagnostic imaging MH - Humans MH - Nerve Block MH - Raynaud Disease/complications/therapy MH - Regional Blood Flow/physiology MH - Sympathectomy/*methods MH - Ultrasonography, Interventional/*methods EDAT- 2013/09/21 06:00 MHDA- 2013/11/14 06:00 CRDT- 2013/09/20 06:00 PHST- 2013/09/20 06:00 [entrez] PHST- 2013/09/21 06:00 [pubmed] PHST- 2013/11/14 06:00 [medline] AID - 10.1111/anae.12416 [doi] PST - ppublish SO - Anaesthesia. 2013 Oct;68(10):1077. doi: 10.1111/anae.12416. PMID- 21733945 OWN - NLM STAT- MEDLINE DCOM- 20120709 LR - 20120413 IS - 1940-1574 (Electronic) IS - 0003-3197 (Linking) VI - 63 IP - 4 DP - 2012 May TI - The association of Raynaud syndrome with thromboangiitis obliterans--a meta-analysis. PG - 315-9 LID - 10.1177/0003319711414868 [doi] AB - Thromboangiitis obliterans (TAO) has traditionally been included among the diseases associated with Raynaud syndrome (RS). The prevalence of RS in patients with TAO is not well defined. The objective of this meta-analysis is to assess the prevalence of RS in patients with TAO. A literature search was performed for the studies dealing with RS and TAO. The studies provided sufficient data to estimate the prevalence of RS in patients with TAO. A total of 8 eligible studies, contributing data on 851 patients, were included in this meta-analysis. For TAO, a pooled prevalence of 28.1% and 95% confidence interval ([CI] = 0.158, 0.423) were obtained. Statistically publication bias was not present (P = .232). Despite some heterogeneity, there is a possible indication of an association between RS and patients with TAO. FAU - Hartmann, Peter AU - Hartmann P AD - Department of Biometry and Clinical Epidemiology, Charité Universitätsmedizin Berlin, Berlin, Germany. peter.hartmann@live.de FAU - Mohokum, Melvin AU - Mohokum M FAU - Schlattmann, Peter AU - Schlattmann P LA - eng PT - Journal Article PT - Meta-Analysis DEP - 20110706 PL - United States TA - Angiology JT - Angiology JID - 0203706 SB - IM MH - Humans MH - Prevalence MH - Raynaud Disease/*complications/epidemiology MH - Thromboangiitis Obliterans/*complications EDAT- 2011/07/08 06:00 MHDA- 2012/07/10 06:00 CRDT- 2011/07/08 06:00 PHST- 2011/07/08 06:00 [entrez] PHST- 2011/07/08 06:00 [pubmed] PHST- 2012/07/10 06:00 [medline] AID - 0003319711414868 [pii] AID - 10.1177/0003319711414868 [doi] PST - ppublish SO - Angiology. 2012 May;63(4):315-9. doi: 10.1177/0003319711414868. Epub 2011 Jul 6. PMID- 28231987 OWN - NLM STAT- MEDLINE DCOM- 20171211 LR - 20171211 IS - 1097-6760 (Electronic) IS - 0196-0644 (Linking) VI - 69 IP - 3 DP - 2017 Mar TI - Elderly Woman With Painful Swollen Fingers. PG - 297-314 LID - S0196-0644(16)31100-3 [pii] LID - 10.1016/j.annemergmed.2016.09.027 [doi] FAU - Bickel, Scott T AU - Bickel ST AD - Chicago College of Osteopathic Medicine, Midwestern University, Downers Grove, IL. FAU - Hannafin, Blaine AU - Hannafin B AD - Department of Emergency Medicine, Kaiser Permanente South Sacramento Medical Center, Sacramento, CA. FAU - Lovecchio, Frank AU - Lovecchio F AD - Department of Emergency Medicine, Maricopa Medical Center, and Samaritan Regional Poison Control Center, Phoenix, AZ. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Ann Emerg Med JT - Annals of emergency medicine JID - 8002646 SB - IM MH - Aged MH - Diagnosis, Differential MH - Edema/diagnosis/*etiology MH - Female MH - Fingers/*blood supply/diagnostic imaging MH - Humans MH - Ischemia/*complications/diagnosis MH - Pain/diagnosis/*etiology MH - Raynaud Disease/*complications/diagnosis EDAT- 2017/02/25 06:00 MHDA- 2017/12/12 06:00 CRDT- 2017/02/25 06:00 PHST- 2016/09/03 00:00 [received] PHST- 2017/02/25 06:00 [entrez] PHST- 2017/02/25 06:00 [pubmed] PHST- 2017/12/12 06:00 [medline] AID - S0196-0644(16)31100-3 [pii] AID - 10.1016/j.annemergmed.2016.09.027 [doi] PST - ppublish SO - Ann Emerg Med. 2017 Mar;69(3):297-314. doi: 10.1016/j.annemergmed.2016.09.027. PMID- 23191999 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - Acute haemodynamic response to carbon dioxide hand immersion in patients with systemic sclerosis evaluated by Doppler ultrasonography. PG - 184-5 FAU - Müller-Eschner, Matthias AU - Müller-Eschner M FAU - Albrecht, Katinka AU - Albrecht K FAU - Tarner, Ingo H AU - Tarner IH FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Strunk, Johannes AU - Strunk J FAU - Lange, Uwe AU - Lange U LA - eng PT - Clinical Trial PT - Letter DEP - 20121129 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 142M471B3J (Carbon Dioxide) SB - IM MH - Aged MH - *Carbon Dioxide MH - Female MH - Hemodynamics/*physiology MH - Humans MH - Immersion MH - Male MH - Middle Aged MH - Pilot Projects MH - Raynaud Disease/*diagnostic imaging/physiopathology MH - Scleroderma, Systemic/*diagnostic imaging/physiopathology MH - Ultrasonography, Doppler/*methods EDAT- 2012/11/30 06:00 MHDA- 2013/09/11 06:00 CRDT- 2012/11/30 06:00 PHST- 2012/04/01 00:00 [received] PHST- 2012/07/05 00:00 [accepted] PHST- 2012/11/30 06:00 [entrez] PHST- 2012/11/30 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 6008 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):184-5. Epub 2012 Nov 29. PMID- 23415512 OWN - NLM STAT- MEDLINE DCOM- 20130618 LR - 20130429 IS - 1879-1913 (Electronic) IS - 0002-9149 (Linking) VI - 111 IP - 9 DP - 2013 May 1 TI - Prevalence of migraine and Raynaud phenomenon in women with apical ballooning syndrome (Takotsubo or stress cardiomyopathy). PG - 1284-8 LID - S0002-9149(13)00331-7 [pii] LID - 10.1016/j.amjcard.2013.01.269 [doi] AB - Apical ballooning syndrome (ABS), migraine, and Raynaud phenomenon are characterized by female predominance, identifiable triggers, and, likely, vascular dysfunction. Estrogen deficiency, such as in menopause, is considered to be a predisposing factor for ABS. We investigated the association of ABS with migraine, Raynaud phenomenon, and hormonal factors. We compared 25 consecutive residents (all women) of Olmsted County, Minnesota, presenting with ABS, to 2 age-matched control groups from the same community: 25 women presenting with ST-segment elevation myocardial infarction (STEMI), matched for the index ABS event date, and 50 women with neither diagnosis. The patients with ABS were more likely to have a migraine history (11 [44%] vs 4 [16%] STEMI controls, p = 0.031, and vs 6 [12%] population controls, p = 0.003), and "possible migraine" (including other headache syndromes suggestive of migraine; 15 [60%] vs 6 [24%] STEMI controls, p = 0.012; and vs 12 [24%] population controls, p = 0.003). Of the patients with ABS, 4 (16%) had Raynaud phenomenon compared to no STEMI controls and 1 (2%) population control (p = 0.038). No differences were present in parity, menopausal status, years since the onset of menopause, and frequency of oophorectomy. Current hormonal replacement therapy use was greater in those with ABS than in the population controls: 4 (16%) versus none (p = 0.002). In conclusion, the association of ABS with migraine and Raynaud phenomenon supports a role of vasomotor dysfunction in the pathogenesis of ABS. The absence of an association with reproductive characteristics and the finding that ABS occurred despite exogenous hormonal use in some patients suggests that estrogen deficiency per se is not the primary factor in the pathophysiology. CI - Copyright © 2013 Elsevier Inc. All rights reserved. FAU - Scantlebury, Dawn C AU - Scantlebury DC AD - Division of Cardiovascular Diseases, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA. FAU - Prasad, Abhiram AU - Prasad A FAU - Rabinstein, Alejandro A AU - Rabinstein AA FAU - Best, Patricia J M AU - Best PJ LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20130212 PL - United States TA - Am J Cardiol JT - The American journal of cardiology JID - 0207277 SB - IM MH - Coronary Angiography MH - Diagnosis, Differential MH - Electrocardiography MH - Female MH - Follow-Up Studies MH - Humans MH - Menopause MH - Migraine Disorders/diagnosis/*epidemiology/etiology MH - Minnesota/epidemiology MH - Prevalence MH - Raynaud Disease/diagnosis/*epidemiology/etiology MH - Retrospective Studies MH - Takotsubo Cardiomyopathy/*complications/diagnosis/epidemiology EDAT- 2013/02/19 06:00 MHDA- 2013/06/19 06:00 CRDT- 2013/02/19 06:00 PHST- 2012/12/06 00:00 [received] PHST- 2013/01/10 00:00 [revised] PHST- 2013/01/10 00:00 [accepted] PHST- 2013/02/19 06:00 [entrez] PHST- 2013/02/19 06:00 [pubmed] PHST- 2013/06/19 06:00 [medline] AID - S0002-9149(13)00331-7 [pii] AID - 10.1016/j.amjcard.2013.01.269 [doi] PST - ppublish SO - Am J Cardiol. 2013 May 1;111(9):1284-8. doi: 10.1016/j.amjcard.2013.01.269. Epub 2013 Feb 12. PMID- 17053515 OWN - NLM STAT- MEDLINE DCOM- 20070130 LR - 20061020 IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 18 IP - 6 DP - 2006 Nov TI - Bibliography. Current world literature. Raynaud phenomenon, scleroderma, overlap syndromes and other fibrosing syndromes. PG - 654-6 LA - eng PT - Bibliography PT - Journal Article PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Fibrosis MH - Humans MH - Raynaud Disease/*pathology MH - Scleroderma, Systemic/*pathology EDAT- 2006/10/21 09:00 MHDA- 2007/01/31 09:00 CRDT- 2006/10/21 09:00 PHST- 2006/10/21 09:00 [pubmed] PHST- 2007/01/31 09:00 [medline] PHST- 2006/10/21 09:00 [entrez] AID - 00002281-200611000-00016 [pii] AID - 10.1097/BOR.0b013e328010f1cd [doi] PST - ppublish SO - Curr Opin Rheumatol. 2006 Nov;18(6):654-6. doi: 10.1097/BOR.0b013e328010f1cd. PMID- 31522232 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20260518 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 39 IP - 11 DP - 2019 Nov TI - LAUDES Study: impact of digital ulcers on hand functional limitation, work productivity and daily activities, in systemic sclerosis patients. PG - 1875-1882 LID - 10.1007/s00296-019-04436-z [doi] AB - The objective of this study was to evaluate the impact of digital ulcers (DUs) in daily life of systemic sclerosis (SSc) Spanish patients. We developed a multicenter observational study to compare functional disability in SSc patients with active DUs vs. those without DUs. An additional correlation between perception of patients and physicians on disability due to DUs was performed. A total of 199 patients were enrolled, 70 (35%) with DUs. Patients with DUs were younger (48 vs. 58 years; p < 0.001) and had more frequently the diffuse subtype of SSc (45 vs. 24%; p = 0.004) than patients without DUs. Patients with DUs showed significantly higher scores in the Cochin Hand Function Scale overall (p < 0.002) and for each of its five dimensions. They also showed higher scores in the Systemic Sclerosis Health Assessment Questionnaire items related to hand function such as, dress and self-care (p < 0.013), eat (p < 0.013) and grip (p < 0.03), and higher Visual Analogic Scale scores for pain (p < 0.013), trouble related with Raynaud's Phenomenon (p < 0.001) and sense of severity (p < 0.004). Impact on daily activities was significantly higher in patients with DUs (p = 0.002), with a non-significant trend to experience higher impact on work productivity (p = 0.07). A high correlation was found between DUs patients and physicians opinion on the impact of DUs (daily life: Pearson R = 0.86; work productivity: Pearson R = 0.87). Study findings show an impaired hand function and increased disability for daily life activities and work productivity in SSc patients with DUs compared with patients without DUs in Spanish population. FAU - Castellví, Ivan AU - Castellví I AUID- ORCID: 0000-0002-5410-5807 AD - Unitat de Reumatologia, Hospital de la Santa Creu i Sant Pau, Carrer de Sant Quintí, 89, 08026, Barcelona, Spain. icastellvi@santpau.cat. FAU - Eguiluz, Saioa AU - Eguiluz S AD - Servicio de Medicina Interna, Hospital Universitario de Cruces, Barakaldo, Spain. FAU - Escudero-Contreras, Alejandro AU - Escudero-Contreras A AD - Unidad de Gestión Clínica de Reumatología, Hospital Universitario Reina Sofía, Córdoba, Spain. FAU - Ríos, Juan José AU - Ríos JJ AD - Servicio de Medicina Interna, Hospital Universitario La Paz, Madrid, Spain. FAU - Calvo-Alén, Jaime AU - Calvo-Alén J AD - Servicio de Reumatología, Hospital Universitario Araba, Vitoria, Spain. FAU - Callejas-Rubio, José Luis AU - Callejas-Rubio JL AD - Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario San Cecilio, Granada, Spain. FAU - De la Puente, Carlos AU - De la Puente C AD - Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Simeón, Carmen Pilar AU - Simeón CP AD - Unitat de Malalties Autoimmunes Sistèmicas, Servei de Medicina Interna, Hospital Universitari Vall d'Hebron, Barcelona, Spain. FAU - Narváez, Francisco Javier AU - Narváez FJ AD - Servei de Reumatologia, Hospital Universitari de Bellvitge, Barcelona, Spain. FAU - Espinosa, Gerard AU - Espinosa G AD - Servei de Medicina Interna, Hospital Clínic de Barcelona, Barcelona, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Rubio-Rivas, Manuel AU - Rubio-Rivas M AD - Unitat de Malalties Autoimmunes, Servei de Medicina Interna, Hospital Universitari de Bellvitge, Barcelona, Spain. FAU - Alegre, Juan José AU - Alegre JJ AD - Servei de Reumatologia, Hospital Universitari Dr. Peset, València, Spain. FAU - Guillén-Del-Castillo, Alfredo AU - Guillén-Del-Castillo A AD - Unitat de Malalties Autoimmunes Sistèmicas, Servei de Medicina Interna, Hospital Universitari Vall d'Hebron, Barcelona, Spain. FAU - Román-Ivorra, Jose Andrés AU - Román-Ivorra JA AD - Servei de Reumatologia, Hospital Universitari i Politècnic la Fe, Universitat Catòlica de València, València, Spain. FAU - Fonollosa, Vicent AU - Fonollosa V AD - Unitat de Malalties Autoimmunes Sistèmicas, Servei de Medicina Interna, Hospital Universitari Vall d'Hebron, Barcelona, Spain. CN - LAUDES Study Group LA - eng PT - Journal Article PT - Observational Study DEP - 20190914 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - *Activities of Daily Living MH - Adult MH - Aged MH - Aged, 80 and over MH - Cross-Sectional Studies MH - Disability Evaluation MH - *Efficiency MH - Female MH - Fingers/blood supply MH - Humans MH - Male MH - Middle Aged MH - Quality of Life MH - Raynaud Disease/*etiology MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*etiology MH - *Workplace MH - Young Adult OTO - NOTNLM OT - Disability evaluation OT - Hand OT - Human activities OT - Quality of life OT - Scleroderma and related disorders FIR - Aspe de la Iglesia, Bruno IR - Aspe de la Iglesia B FIR - Barbado Ajo, Julia IR - Barbado Ajo J FIR - Batista Perdomo, Daniel IR - Batista Perdomo D FIR - Beltrán Catalán, Emma IR - Beltrán Catalán E FIR - Belzunegui Otano, Joaquin IR - Belzunegui Otano J FIR - Blanco Rodríguez, Jorge Santiago IR - Blanco Rodríguez JS FIR - Caamaño Freire, Manuel IR - Caamaño Freire M FIR - Cantabrana Alútiz, Alberto IR - Cantabrana Alútiz A FIR - Carrio Montes, Isabel IR - Carrio Montes I FIR - Cia Lecumberri, Maite IR - Cia Lecumberri M FIR - Del Castillo Monsalvo, Rosa IR - Del Castillo Monsalvo R FIR - Diez Alvarez, Elvira IR - Diez Alvarez E FIR - Egües Dubuc, César Antonio IR - Egües Dubuc CA FIR - Egurbide Arberas, Maria Victoria IR - Egurbide Arberas MV FIR - Fanlo Mateo, Patricia IR - Fanlo Mateo P FIR - Freire Dapena, Maria Del Carmen IR - Freire Dapena MDC FIR - Freire González, Mercedes IR - Freire González M FIR - García Aparicio, Ángel IR - García Aparicio Á FIR - García Hernández, Francisco José IR - García Hernández FJ FIR - Gómez de Salazar, José IR - Gómez de Salazar J FIR - González Nieto, José Antonio IR - González Nieto JA FIR - Graña Gil, Jenaro IR - Graña Gil J FIR - Guillén-Del-Castillo, Alfredo IR - Guillén-Del-Castillo A FIR - Izquierdo Hidalgo, José IR - Izquierdo Hidalgo J FIR - Juan Mas, Toni IR - Juan Mas T FIR - Laiño Piñeiro, Maria Cruz IR - Laiño Piñeiro MC FIR - López Longo, Francisco Javier IR - López Longo FJ FIR - López Núñez, Lilian Maria IR - López Núñez LM FIR - Marín Ballvé, Adela IR - Marín Ballvé A FIR - Marras Fernandez-Cid, Carlos IR - Marras Fernandez-Cid C FIR - Miguelez, Roberto IR - Miguelez R FIR - Moran Rodriguez, Miguel Ángel IR - Moran Rodriguez MÁ FIR - Moreno Morales, Juan IR - Moreno Morales J FIR - Morlà, Rosa Maria IR - Morlà RM FIR - Ojeda Bruno, Soledad IR - Ojeda Bruno S FIR - Olivé Marqués, Alejandro IR - Olivé Marqués A FIR - Ornilla Laraundogoitia, Enrique IR - Ornilla Laraundogoitia E FIR - Ortego, Noberto IR - Ortego N FIR - Ortiz Santamaria, Vera IR - Ortiz Santamaria V FIR - Pérez Conesa, Mercedes Pilar IR - Pérez Conesa MP FIR - Pérez Sandoval, Trinidad IR - Pérez Sandoval T FIR - Pros Simon, Ana IR - Pros Simon A FIR - Rodríguez Lozano, Beatriz IR - Rodríguez Lozano B FIR - Rueda Cid, Amalia IR - Rueda Cid A FIR - Sáez Comet, Luis IR - Sáez Comet L FIR - Salvatierra Osorio, Juan IR - Salvatierra Osorio J FIR - Sanmartín López, Alejandro IR - Sanmartín López A FIR - Santos Soler, Gregorio IR - Santos Soler G FIR - Sarmiento Guevara, Mónica IR - Sarmiento Guevara M FIR - Todolí Parra, José Antonio IR - Todolí Parra JA FIR - Tornero Ramos, Carmelo IR - Tornero Ramos C FIR - Tovar Beltrán, Juan IR - Tovar Beltrán J FIR - Uceda Montañez, Julia Maria IR - Uceda Montañez JM FIR - Vicente Rabaneda, Esther IR - Vicente Rabaneda E FIR - Valveny, Neus IR - Valveny N FIR - Gallastegui, Nerea IR - Gallastegui N EDAT- 2019/09/16 06:00 MHDA- 2020/04/28 06:00 CRDT- 2019/09/16 06:00 PHST- 2019/07/08 00:00 [received] PHST- 2019/08/24 00:00 [accepted] PHST- 2019/09/16 06:00 [pubmed] PHST- 2020/04/28 06:00 [medline] PHST- 2019/09/16 06:00 [entrez] AID - 10.1007/s00296-019-04436-z [pii] AID - 10.1007/s00296-019-04436-z [doi] PST - ppublish SO - Rheumatol Int. 2019 Nov;39(11):1875-1882. doi: 10.1007/s00296-019-04436-z. Epub 2019 Sep 14. PMID- 31585746 OWN - NLM STAT- MEDLINE DCOM- 20210625 LR - 20210625 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 45 IP - 7 DP - 2020 Jul TI - Radial Artery Occlusion in a Patient With Lupus, Antiphospholipid Syndrome, and Raynaud Phenomenon: A Multimodal Approach. PG - 664.e1-664.e5 LID - S0363-5023(19)30169-8 [pii] LID - 10.1016/j.jhsa.2019.08.009 [doi] AB - Radial artery occlusion (RAO) is a known complication of transradial catheterization for cardiac procedures. The transradial approach has decreased bleeding complications compared with the transfemoral approach, but risks provoking hand ischemia. We present a case of a 29-year-old peripartum woman with a history of lupus, antiphospholipid syndrome, and Raynaud phenomenon who developed RAO with hand-threatening ischemia despite therapeutic anticoagulation. Given the patient's medical history, a multimodal approach was applied including thrombectomy, arterial bypass, venous arterialization, and onobotulinum toxin A sympathectomy. The patient's ischemia improved after the procedure, and she regained normal use of the hand. CI - Copyright © 2020 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved. FAU - Miller, Travis J AU - Miller TJ AD - The Buncke Clinic, San Francisco, CA; Department of Surgery, Division of Plastic and Reconstructive Surgery, Stanford University School of Medicine, Palo Alto, CA. Electronic address: travismi@stanford.edu. FAU - Lin, Walter C AU - Lin WC AD - The Buncke Clinic, San Francisco, CA. FAU - Safa, Bauback AU - Safa B AD - The Buncke Clinic, San Francisco, CA. LA - eng PT - Case Reports PT - Journal Article DEP - 20191002 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 SB - IM MH - Adult MH - *Antiphospholipid Syndrome/complications/therapy MH - *Arterial Occlusive Diseases/diagnostic imaging/etiology MH - Cardiac Catheterization MH - Female MH - Humans MH - Radial Artery/diagnostic imaging/surgery MH - *Raynaud Disease/etiology/therapy OTO - NOTNLM OT - Antiphospholipid syndrome OT - Raynaud phenomenon OT - lupus OT - radial artery occlusion OT - revascularization EDAT- 2019/10/06 06:00 MHDA- 2021/06/29 06:00 CRDT- 2019/10/06 06:00 PHST- 2019/01/30 00:00 [received] PHST- 2019/07/09 00:00 [revised] PHST- 2019/08/13 00:00 [accepted] PHST- 2019/10/06 06:00 [pubmed] PHST- 2021/06/29 06:00 [medline] PHST- 2019/10/06 06:00 [entrez] AID - S0363-5023(19)30169-8 [pii] AID - 10.1016/j.jhsa.2019.08.009 [doi] PST - ppublish SO - J Hand Surg Am. 2020 Jul;45(7):664.e1-664.e5. doi: 10.1016/j.jhsa.2019.08.009. Epub 2019 Oct 2. PMID- 37500206 OWN - NLM STAT- MEDLINE DCOM- 20230731 LR - 20230731 IS - 1535-7732 (Electronic) IS - 1051-0443 (Linking) VI - 34 IP - 8 DP - 2023 Aug TI - Cold-Induced Raynaud Phenomenon. PG - 1471 LID - S1051-0443(23)00289-0 [pii] LID - 10.1016/j.jvir.2023.04.007 [doi] FAU - Chorney, Michael Aaron AU - Chorney MA AD - Department of Radiology, University of Louisville School of Medicine, Louisville, Kentucky. Electronic address: Michael.Chorney@louisville.edu. LA - eng PT - Journal Article PL - United States TA - J Vasc Interv Radiol JT - Journal of vascular and interventional radiology : JVIR JID - 9203369 SB - IM MH - Humans MH - *Raynaud Disease/etiology MH - Cold Temperature EDAT- 2023/07/28 01:08 MHDA- 2023/07/31 06:43 CRDT- 2023/07/27 20:56 PHST- 2023/02/23 00:00 [received] PHST- 2023/04/11 00:00 [revised] PHST- 2023/04/13 00:00 [accepted] PHST- 2023/07/31 06:43 [medline] PHST- 2023/07/28 01:08 [pubmed] PHST- 2023/07/27 20:56 [entrez] AID - S1051-0443(23)00289-0 [pii] AID - 10.1016/j.jvir.2023.04.007 [doi] PST - ppublish SO - J Vasc Interv Radiol. 2023 Aug;34(8):1471. doi: 10.1016/j.jvir.2023.04.007. PMID- 21858485 OWN - NLM STAT- MEDLINE DCOM- 20120306 LR - 20260128 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 70 IP - 7 DP - 2011 Sep TI - [Maurice Raynaud (1834-1881) and the syndrome named after him]. PG - 620-4 LID - 10.1007/s00393-011-0849-8 [doi] AB - Approximately 150 years ago Maurice Raynaud described in his doctoral thesis a set of symptoms characterized by intermittent pallor and cyanosis of the extremities which in severe cases could lead to the development of gangrene. Because the symptoms could be triggered by spasms in small blood vessels the exclusion of an organic vascular disease is a prerequisite of the diagnosis. Raynaud had not yet recognized that this often observed syndrome could sometimes be advance or initial symptoms of a severe general disease. This is the reason why nowadays these are referred to as primary and secondary Raynaud syndromes. Simultaneously to his doctoral thesis Raynaud had submitted his PhD thesis with two noteworthy publications on the history of medicine. His postdoctoral thesis encompassed a comprehensive study of the"efferent process". Raynaud worked in various Paris clinics, occupied himself with many problems and was considered to be a good academic teacher. For political reasons he was neither promoted to professor nor did he receive a chair in the history of medicine. FAU - Kaiser, H AU - Kaiser H LA - ger PT - Biography PT - Historical Article PT - Journal Article PT - Portrait TT - Maurice Raynaud (1834-1881) und das nach ihm benannte Syndrom. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - France MH - History, 19th Century MH - Humans MH - Male MH - Manuscripts, Medical as Topic/*history MH - Raynaud Disease/*history PS - Raynaud M FPS - Raynaud, Maurice EDAT- 2011/08/23 06:00 MHDA- 2012/03/07 06:00 CRDT- 2011/08/23 06:00 PHST- 2011/08/23 06:00 [entrez] PHST- 2011/08/23 06:00 [pubmed] PHST- 2012/03/07 06:00 [medline] AID - 10.1007/s00393-011-0849-8 [doi] PST - ppublish SO - Z Rheumatol. 2011 Sep;70(7):620-4. doi: 10.1007/s00393-011-0849-8. PMID- 25770637 OWN - NLM STAT- MEDLINE DCOM- 20151110 LR - 20150316 IS - 1557-9808 (Electronic) IS - 1557-9808 (Linking) VI - 17 IP - 4 DP - 2014 Dec TI - Raynaud syndrome. PG - 241-6 LID - S1089-2516(14)00060-2 [pii] LID - 10.1053/j.tvir.2014.11.004 [doi] AB - Raynaud syndrome (RS) was first described by the French physician Maurice Raynaud in 1862 with the characteristic tricolor change featuring pallor (ischemic phase), cyanosis (deoxygenation phase), and erythema (reperfusion phase) induced by cold or stress. Although the underlying pathophysiological mechanism is unclear, alterations in activity of the peripheral adrenoceptor have been implicated, specifically an enhanced smooth muscle contraction due to overexpression or hyperactivity of postsynaptic alpha 2 receptors. There are 2 ways that RS can appear clinically; isolated, formerly referred as Raynaud disease or now primary RS and in association with other conditions, usually connective tissue disorders (eg, Sjögren syndrome, systemic lupus erythematosus, scleroderma, and rheumatoid arthritis), frequently called Raynaud phenomenon or secondary RS. The estimated prevalence in the general population is 3%-5%, with a higher prevalence in women than in men. The diagnosis is mainly clinical, based on patient descriptions of skin changes. Upper extremity pulse-volume recording is used to rule out proximal arterial obstruction. The differentiation between a vasospastic vs and obstructive mechanism is made using digital pressures and photoplethysmography, where an obstructive mechanism has decreased pressures and blunted waveforms. Cold challenge testing, such as ice water immersion with temperature recovery, is highly sensitive but lack specificity. Serologic screening (antinuclear antibody and rheumatoid factor) is advocated to rule out associated connective tissue disorders. Most patients with RS can be managed conservatively, with avoidance of cold exposure or hand warming. For those in whom conservative management is inadequate, a number of pharmacologic and surgical therapies have been used. Owing to lack of complete understanding of the underlying pathophysiology, targeted therapy has not been possible; rather, therapy has been focused on the use of general vasodilation strategies. In this review, the diagnosis, natural history, and current medical and invasive therapy are summarized. CI - Copyright © 2014. Published by Elsevier Inc. FAU - Valdovinos, Sergio Toledo AU - Valdovinos ST AD - Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR. FAU - Landry, Gregory J AU - Landry GJ AD - Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR. Electronic address: landryg@ohsu.edu. LA - eng PT - Journal Article PT - Review DEP - 20141113 PL - United States TA - Tech Vasc Interv Radiol JT - Techniques in vascular and interventional radiology JID - 9806675 SB - IM MH - Humans MH - Raynaud Disease/*diagnosis/*therapy OTO - NOTNLM OT - raynaud syndrome OT - upper extremity ischemia OT - vasospasm EDAT- 2015/03/17 06:00 MHDA- 2015/11/11 06:00 CRDT- 2015/03/16 06:00 PHST- 2015/03/16 06:00 [entrez] PHST- 2015/03/17 06:00 [pubmed] PHST- 2015/11/11 06:00 [medline] AID - S1089-2516(14)00060-2 [pii] AID - 10.1053/j.tvir.2014.11.004 [doi] PST - ppublish SO - Tech Vasc Interv Radiol. 2014 Dec;17(4):241-6. doi: 10.1053/j.tvir.2014.11.004. Epub 2014 Nov 13. PMID- 32727696 OWN - NLM STAT- MEDLINE DCOM- 20210222 LR - 20210222 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 41 IP - 10 DP - 2020 Oct TI - [Raynaud phenomenon with arterial thromboses and IgG4-related disease]. PG - 708-713 LID - S0248-8663(20)30192-2 [pii] LID - 10.1016/j.revmed.2020.05.008 [doi] AB - INTRODUCTION: This is a clinical case illustrating a diagnosis of an IgG4 related-disease (IgG4-RD) diagnosed in a vascular context. CASE REPORT: A 47-year-old man with no past medical history consulted for a recent and disabling Raynaud phenomenon without trophic disorder. Vascular examinations revealed multiple arterial thromboses with no abnormal finger and toe pressures. Secondly, weight loss and submandibular glands enlargement appeared, leading to the diagnosis of IgG4-RD without a link being able to be established with vascular involvement. This is the second observation of this association. A French translation of the new classification criteria for IgG4-RD published in 2019 by the American College of Rheumatology and European Ligue Against Rhumatism (ACR/EULAR) is offered with direct application to the clinical case. CONCLUSION: A Raynaud phenomenon with distal arterial thrombosis is rarely observed in the IgG4-RD. CI - Copyright © 2020. Published by Elsevier Masson SAS. FAU - Lenfant, T AU - Lenfant T AD - Service de Dermatologie et Allergologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. FAU - Moroch, J AU - Moroch J AD - Service d'Anatomopathologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. FAU - de Risi-Pugliese, T AU - de Risi-Pugliese T AD - Service de Dermatologie et Allergologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. FAU - Monfort, J-B AU - Monfort JB AD - Service de Dermatologie et Allergologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. FAU - Benjoar, M AU - Benjoar M AD - Centre d'Imagerie Manin Crimée, 92 bis Rue de Crimée, 75019 Paris, France. FAU - Barbaud, A AU - Barbaud A AD - Service de Dermatologie et Allergologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. FAU - Senet, P AU - Senet P AD - Service de Dermatologie et Allergologie, Hôpital Tenon, AP-HP, Sorbonne Université, 4 rue de la Chine, 75020 Paris, France. Electronic address: patricia.senet@aphp.fr. LA - fre PT - Case Reports PT - Journal Article TT - Phénomène de Raynaud avec thromboses artérielles au cours d'une maladie associée aux IgG4. DEP - 20200727 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - France MH - Humans MH - Immunoglobulin G4-Related Disease/complications/*diagnosis MH - Male MH - Middle Aged MH - Radial Artery/diagnostic imaging/pathology MH - Raynaud Disease/complications/*diagnosis MH - Salivary Gland Diseases/complications/diagnosis/pathology MH - Thrombosis/complications/*diagnosis/pathology MH - Tobacco Smoking/pathology MH - Ulnar Artery/diagnostic imaging/pathology OTO - NOTNLM OT - ACR OT - Acrosyndrome OT - Arterial thrombosis OT - IgG4 OT - Phénomène de Raynaud OT - Raynaud phenomenon OT - Thromboses artérielles EDAT- 2020/07/31 06:00 MHDA- 2021/02/23 06:00 CRDT- 2020/07/31 06:00 PHST- 2020/02/24 00:00 [received] PHST- 2020/04/29 00:00 [revised] PHST- 2020/05/21 00:00 [accepted] PHST- 2020/07/31 06:00 [pubmed] PHST- 2021/02/23 06:00 [medline] PHST- 2020/07/31 06:00 [entrez] AID - S0248-8663(20)30192-2 [pii] AID - 10.1016/j.revmed.2020.05.008 [doi] PST - ppublish SO - Rev Med Interne. 2020 Oct;41(10):708-713. doi: 10.1016/j.revmed.2020.05.008. Epub 2020 Jul 27. PMID- 21885525 OWN - NLM STAT- MEDLINE DCOM- 20120622 LR - 20131121 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 38 IP - 9 DP - 2011 Sep TI - Effects of iloprost on microvascular structure assessed by nailfold videocapillaroscopy: a pilot study. PG - 2079-80 LID - 10.3899/jrheum.110067 [doi] FAU - Shah, Preeti AU - Shah P FAU - Murray, Andrea K AU - Murray AK FAU - Moore, Tonia L AU - Moore TL FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Clinical Trial PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Capillaries/*drug effects MH - Female MH - Humans MH - Iloprost/*therapeutic use MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/blood supply MH - Pilot Projects MH - Raynaud Disease/drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy MH - Vasodilator Agents/*therapeutic use EDAT- 2011/09/03 06:00 MHDA- 2012/06/23 06:00 CRDT- 2011/09/03 06:00 PHST- 2011/09/03 06:00 [entrez] PHST- 2011/09/03 06:00 [pubmed] PHST- 2012/06/23 06:00 [medline] AID - 38/9/2079 [pii] AID - 10.3899/jrheum.110067 [doi] PST - ppublish SO - J Rheumatol. 2011 Sep;38(9):2079-80. doi: 10.3899/jrheum.110067. PMID- 21652164 OWN - NLM STAT- MEDLINE DCOM- 20110906 LR - 20121003 IS - 1097-6809 (Electronic) IS - 0741-5214 (Linking) VI - 54 IP - 1 DP - 2011 Jul TI - Thoracic sympathectomy for digital ischemia: a summary of evidence. PG - 273-7 LID - 10.1016/j.jvs.2011.01.069 [doi] AB - BACKGROUND: Thoracic sympathectomy is used in the management of a variety of upper limb disorders. We have analyzed the evidence for thoracic sympathectomy in the management of digital ischemia. METHODS: We reviewed the English literature between 1980 and 2010. Our analysis included reports with the clinical end points of relief, recurrence of symptoms or healing of ulcers, or both. Primary Raynaud disease (PRD) and secondary Raynaud phenomenon (SRP) were analyzed separately. RESULTS: An initial postoperative positive effect was reported in 92% of PRD patients and in 89% of SRP patients. Long-term beneficial effect was 58% for PRD and 89% for SRP. Ulcer healing or improvement was achieved in 95%. CONCLUSIONS: The available evidence suggests that thoracic sympathectomy has a role in the treatment of severe PRD and SRP, albeit with better results in SRP patients than in PRD patients. In case of digital ulceration, thoracic sympathectomy may maximize tissue preservation or prevent amputation. CI - Copyright © 2011 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved. FAU - Coveliers, Hans M E AU - Coveliers HM AD - Department of Surgery, VU University Medical Centre, Amsterdam, The Netherlands. h.coveliers@vumc.nl FAU - Hoexum, Frank AU - Hoexum F FAU - Nederhoed, Johanna H AU - Nederhoed JH FAU - Wisselink, Willem AU - Wisselink W FAU - Rauwerda, Jan A AU - Rauwerda JA LA - eng PT - Journal Article PT - Review DEP - 20110608 PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Evidence-Based Medicine MH - Fingers/*blood supply MH - Humans MH - Ischemia/pathology/*surgery MH - Raynaud Disease/pathology/*surgery MH - Skin Ulcer/pathology/*surgery MH - *Sympathectomy MH - Thoracic Nerves/*surgery MH - Time Factors MH - Treatment Outcome MH - Wound Healing EDAT- 2011/06/10 06:00 MHDA- 2011/09/07 06:00 CRDT- 2011/06/10 06:00 PHST- 2010/10/21 00:00 [received] PHST- 2011/01/19 00:00 [revised] PHST- 2011/01/25 00:00 [accepted] PHST- 2011/06/10 06:00 [entrez] PHST- 2011/06/10 06:00 [pubmed] PHST- 2011/09/07 06:00 [medline] AID - S0741-5214(11)00235-7 [pii] AID - 10.1016/j.jvs.2011.01.069 [doi] PST - ppublish SO - J Vasc Surg. 2011 Jul;54(1):273-7. doi: 10.1016/j.jvs.2011.01.069. Epub 2011 Jun 8. PMID- 29534983 OWN - NLM STAT- MEDLINE DCOM- 20181002 LR - 20181004 IS - 1873-1740 (Electronic) IS - 0033-0620 (Linking) VI - 60 IP - 6 DP - 2018 Mar-Apr TI - Cutaneous Manifestations of Chronic Vascular Disease. PG - 567-579 LID - S0033-0620(18)30054-9 [pii] LID - 10.1016/j.pcad.2018.03.004 [doi] AB - In the contemporary era of medical diagnosis via sophisticated radiographic imaging and/or comprehensive serological testing, a focused physical examination remains paramount in recognizing the cutaneous manifestations of chronic vascular disease. Recognition of the unique cutaneous signs of lymphatic and venous hypertension assists in the diagnosis as well as the staging and classification of both lymphedema and chronic venous insufficiency. Awareness of explicit dermatologic vasomotor manifestations aids not only in the identification of acrocyanosis, Raynaud phenomenon, pernio, and erythromelalgia but also mitigates confusion related to their clinical overlap. Although the clinical signs of peripheral artery disease are not necessarily specific or sensitive, a knowledge of suggestive dermatologic findings is helpful in recognition of severe limb ischemia. A brief review of the epidemiology, etiology, pathogenesis, and therapy of cutaneous related chronic vascular disease follows including an emphasis on characteristic clinical features supported by illustrative photographs. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Dean, Steven M AU - Dean SM AD - Division of Cardiovascular Medicine, Ohio State University Wexner Medical Center, Columbus, OH. Electronic address: steven.dean@osumc.edu. LA - eng PT - Journal Article PT - Review DEP - 20180310 PL - United States TA - Prog Cardiovasc Dis JT - Progress in cardiovascular diseases JID - 0376442 SB - IM MH - Chronic Disease MH - Diagnosis, Differential MH - Erythromelalgia/diagnosis/etiology MH - Female MH - Humans MH - Ischemia/diagnosis/etiology MH - Lymphedema/diagnosis/etiology MH - Male MH - Peripheral Vascular Diseases/*complications/diagnosis/*pathology MH - Raynaud Disease/diagnosis/etiology MH - Skin Diseases/*diagnosis/*etiology/pathology MH - Skin Ulcer/diagnosis/etiology OTO - NOTNLM OT - Acrocyanosis OT - Chronic Venous Insufficiency OT - Erythromelalgia OT - Lymphedema OT - Peripheral Artery Disease OT - Pernio OT - Raynaud phenomenon EDAT- 2018/03/15 06:00 MHDA- 2018/10/03 06:00 CRDT- 2018/03/15 06:00 PHST- 2018/03/08 00:00 [received] PHST- 2018/03/08 00:00 [accepted] PHST- 2018/03/15 06:00 [pubmed] PHST- 2018/10/03 06:00 [medline] PHST- 2018/03/15 06:00 [entrez] AID - S0033-0620(18)30054-9 [pii] AID - 10.1016/j.pcad.2018.03.004 [doi] PST - ppublish SO - Prog Cardiovasc Dis. 2018 Mar-Apr;60(6):567-579. doi: 10.1016/j.pcad.2018.03.004. Epub 2018 Mar 10. PMID- 28429663 OWN - NLM STAT- MEDLINE DCOM- 20180816 LR - 20180816 IS - 1477-0377 (Electronic) IS - 1358-863X (Linking) VI - 22 IP - 2 DP - 2017 Apr TI - Finding the groove: The groove sign in scleroderma and related disorders. PG - 161 LID - 10.1177/1358863X16686411 [doi] FAU - Gómez Gómez, Alejandro AU - Gómez Gómez A AD - 1 Rheumatology Department, Hospital Universitario Madrid Sanchinarro and Hospital Universitario Infanta Sofia, Madrid, Spain. FAU - Cecconi, Alberto AU - Cecconi A AD - 2 Cardiology Department, Hospital Universitario de La Princesa, Madrid, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20170321 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Diagnosis, Differential MH - Female MH - Forearm/*blood supply MH - Humans MH - Middle Aged MH - Raynaud Disease/diagnosis/*pathology MH - Scleroderma, Localized/diagnosis/*pathology MH - Scleroderma, Systemic/diagnosis/pathology MH - Skin/*blood supply/pathology EDAT- 2017/04/22 06:00 MHDA- 2018/08/17 06:00 CRDT- 2017/04/22 06:00 PHST- 2017/04/22 06:00 [entrez] PHST- 2017/04/22 06:00 [pubmed] PHST- 2018/08/17 06:00 [medline] AID - 10.1177/1358863X16686411 [doi] PST - ppublish SO - Vasc Med. 2017 Apr;22(2):161. doi: 10.1177/1358863X16686411. Epub 2017 Mar 21. PMID- 33599244 OWN - NLM STAT- MEDLINE DCOM- 20210818 LR - 20250530 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 60 IP - 6 DP - 2021 Jun 18 TI - A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies. PG - 2615-2628 LID - 10.1093/rheumatology/keab166 [doi] AB - OBJECTIVES: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening. METHODS: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review. RESULTS: Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers. CONCLUSION: Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Oldroyd, Alexander G S AU - Oldroyd AGS AUID- ORCID: 0000-0001-5701-6490 AD - National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. AD - Centre for Musculoskeletal Research, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Centre for Epidemiology Versus Arthritis, University of Manchester, Manchester, UK. AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Allard, Andrew B AU - Allard AB AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK. FAU - Callen, Jeffrey P AU - Callen JP AD - Division of Medicine, Department of Medicine, University of Louisville, Louisville, KY, USA. FAU - Chinoy, Hector AU - Chinoy H AUID- ORCID: 0000-0001-6492-1288 AD - National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. AD - Centre for Musculoskeletal Research, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Department of Medicine and Dermatology, Stanford University, Stanford, USA. AD - Palo Alto Health Care System, Palo Alto, USA. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Dermatology, School of Medicine, Stanford University, Stanford, CA, USA. FAU - George, Michael D AU - George MD AD - Division of Rheumatology, Philadelphia, PA, USA. AD - Division of Epidemiology, University of Pennsylvania, Philadelphia, PA, USA. FAU - Gordon, Patrick AU - Gordon P AD - Department of Rheumatology, King's College Hospital NHS Foundation Trust, London, UK. FAU - Kolstad, Kate AU - Kolstad K AD - Department of Dermatology, School of Medicine, Stanford University, Stanford, CA, USA. FAU - Kurtzman, Drew J B AU - Kurtzman DJB AD - Department of Dermatology, Wright State University, Dayton, OH, USA. FAU - Machado, Pedro M AU - Machado PM AUID- ORCID: 0000-0002-8411-7972 AD - Centre for Rheumatology and Department of Neuromuscular Diseases, University College London, London, UK. AD - National Institute for Health Research University College London Hospitals Biomedical Research Centre, University College London Hospitals NHS Trust, London, UK. AD - Department of Rheumatology, Northwick Park Hospital, London North West University Healthcare NHS Trust, London, UK. FAU - McHugh, Neil J AU - McHugh NJ AUID- ORCID: 0000-0003-2765-658X AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Postolova, Anna AU - Postolova A AD - Department of Immunology and Rheumatology, Stanford Health Care, Stanford, CA, USA. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AD - Systemic Autoimmune Unity, Vall D'Hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, Spain. FAU - Schmidt, Jens AU - Schmidt J AD - Department of Neurology, Neuromuscular Centre, Göttingen, Germany. FAU - Tansley, Sarah AU - Tansley S AUID- ORCID: 0000-0003-4633-9598 AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK. AD - Department of Neurology, Neuromuscular Centre, Göttingen, Germany. FAU - Vleugels, Ruth Ann AU - Vleugels RA AD - Harvard Medical School, Boston, MA, USA. AD - Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA. FAU - Werth, Victoria P AU - Werth VP AD - Department of Dermatology, University of Pennsylvania, Philadelphia, USA. AD - Division of Dermatology, Corporal Michael J. Crescenz VA Medical Center, Philadelphia, USA. FAU - Aggarwal, Rohit AU - Aggarwal R AUID- ORCID: 0000-0001-7531-8038 AD - Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA. LA - eng GR - 21993/VAC_/Versus Arthritis/United Kingdom GR - T32 AR050942/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Antinuclear) RN - 0 (DNA-Binding Proteins) RN - 0 (Jo-1 antibody) RN - 0 (TRIM33 protein, human) RN - 0 (Transcription Factors) RN - EC 1.1.1.27 (L-Lactate Dehydrogenase) RN - EC 2.7.3.2 (Creatine Kinase) RN - EC 3.6.1.- (Adenosine Triphosphatases) RN - EC 3.6.1.- (MORC3 protein, human) RN - Amyopathic dermatomyositis SB - IM EIN - Rheumatology (Oxford). 2021 Nov 3;60(11):5483. doi: 10.1093/rheumatology/keab616. PMID: 34689208 CIN - JAMA Dermatol. 2022 Mar 1;158(3):244-247. doi: 10.1001/jamadermatol.2021.5841. PMID: 35107570 MH - Adenosine Triphosphatases/immunology MH - Age Factors MH - Antibodies, Antinuclear/blood MH - Creatine Kinase/blood MH - DNA-Binding Proteins/immunology MH - Deglutition Disorders/complications MH - Dermatomyositis/complications/diagnosis/etiology MH - Female MH - *Guidelines as Topic MH - Humans MH - L-Lactate Dehydrogenase/blood MH - Lung Diseases, Interstitial/complications MH - Male MH - Myositis/blood/*complications MH - Neoplasms/*diagnosis/etiology MH - Publication Bias MH - Raynaud Disease/complications MH - Risk MH - Sex Factors MH - Skin Ulcer/complications MH - Tomography, X-Ray Computed MH - Transcription Factors/immunology PMC - PMC8213426 OTO - NOTNLM OT - CT scanning OT - autoantibodies OT - epidemiology OT - meta-analysis OT - muscle OT - myositis OT - neoplasia EDAT- 2021/02/19 06:00 MHDA- 2021/08/19 06:00 PMCR- 2021/02/18 CRDT- 2021/02/18 08:38 PHST- 2020/12/04 00:00 [received] PHST- 2021/01/26 00:00 [revised] PHST- 2021/02/11 00:00 [accepted] PHST- 2021/02/19 06:00 [pubmed] PHST- 2021/08/19 06:00 [medline] PHST- 2021/02/18 08:38 [entrez] PHST- 2021/02/18 00:00 [pmc-release] AID - 6143039 [pii] AID - keab166 [pii] AID - 10.1093/rheumatology/keab166 [doi] PST - ppublish SO - Rheumatology (Oxford). 2021 Jun 18;60(6):2615-2628. doi: 10.1093/rheumatology/keab166. PMID- 25997047 OWN - NLM STAT- MEDLINE DCOM- 20150727 LR - 20210109 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 94 IP - 20 DP - 2015 May TI - Prevalence of Raynaud phenomenon and nailfold capillaroscopic abnormalities in Fabry disease: a cross-sectional study. PG - e780 LID - 10.1097/MD.0000000000000780 [doi] LID - e780 AB - Fabry disease (FD) is a lysosomal disorder leading to progressive systemic involvement, including microvascular damage that leads to neurological and cardiovascular disorders. We hypothesize that the latter could be documented at an early stage by performing a microcirculation study with nailfold capillaroscopy and evaluation of Raynaud phenomenon.The objective was to measure the prevalence of Raynaud phenomenon and nailfold capillaroscopic abnormalities in FD.This cross-sectional study included a standardized questionnaire and a nailfold capillaroscopy that assessed previously reported patterns in FD (dystrophic and giant capillaries, avascular fields, irregular architecture, dilatation and density of capillaries, hemorrhage), and was conducted on 32 Fabry patients and 39 controls. Capillaroscopic photographs were reviewed by 2 independent blinded investigators.Twelve Fabry patients (38%) suffered from Raynaud phenomenon, 5 were males (ie, 50% of male Fabry patients), compared with 2 controls (13%) (P < 0.001), of whom none were males (P < 0.001). Raynaud phenomenon was concomitant or before the occurrence of pain in the extremities in 42% of Fabry patients.More ramified capillaries were significantly observed in Fabry patients (12/32, 38%) than in controls (5/39, 13%, P = 0.016).Secondary Raynaud phenomenon should lead to screening for FD, especially in men. By extension, in high-risk populations for FD, the presence of Raynaud phenomenon and ramified capillaries should be assessed. FAU - Deshayes, Samuel AU - Deshayes S AD - From the Departments of Internal Medicine (SD, BB) and Biostatistics (JJP), C.H.U. Côte de Nacre, Caen; University of Tours, Inserm Imagerie et Cerveau UMR U930, Tours (LA); Department of Internal Medicine and Infectious Diseases, C.H.U. Robert Debré, Reims, France (RJ); Department of Internal Medicine, Hôpital de la Croix Saint-Simon, Paris (OL); APMF, Vendresse (NT); Department of Internal Medicine, C.H.U. Michallon, Grenoble, France (BI). FAU - Auboire, Laurent AU - Auboire L FAU - Jaussaud, Roland AU - Jaussaud R FAU - Lidove, Olivier AU - Lidove O FAU - Parienti, Jean-Jacques AU - Parienti JJ FAU - Triclin, Nathalie AU - Triclin N FAU - Imbert, Bernard AU - Imbert B FAU - Bienvenu, Boris AU - Bienvenu B FAU - Aouba, Achille AU - Aouba A LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Capillaries/*pathology MH - Case-Control Studies MH - Cross-Sectional Studies MH - Fabry Disease/*complications/pathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Prevalence MH - Raynaud Disease/epidemiology/*etiology MH - Sex Factors PMC - PMC4602884 COIS- Disclosures: Samuel Deshayes has received reimbursement of congress fees from Sanofi Genzyme. Roland Jaussaud, Olivier Lidove, and Boris Bienvenu have received consulting fees and research grants from Sanofi Genzyme and Shire HGT. The other authors have no conflicts of interest to disclose. EDAT- 2015/05/23 06:00 MHDA- 2015/07/28 06:00 PMCR- 2015/05/22 CRDT- 2015/05/22 06:00 PHST- 2015/05/22 06:00 [entrez] PHST- 2015/05/23 06:00 [pubmed] PHST- 2015/07/28 06:00 [medline] PHST- 2015/05/22 00:00 [pmc-release] AID - 00005792-201505040-00013 [pii] AID - 10.1097/MD.0000000000000780 [doi] PST - ppublish SO - Medicine (Baltimore). 2015 May;94(20):e780. doi: 10.1097/MD.0000000000000780. PMID- 34826285 OWN - NLM STAT- MEDLINE DCOM- 20220124 LR - 20220124 IS - 2326-6929 (Electronic) IS - 0011-4162 (Linking) VI - 108 IP - 3 DP - 2021 Sep TI - Botulinum Toxin for the Treatment of Intractable Raynaud Phenomenon. PG - E11-E14 LID - 10.12788/cutis.0355 [doi] FAU - Shwe, Samantha AU - Shwe S AD - Department of Dermatology, University of California, Irvine. FAU - Sharma, Aditi A AU - Sharma AA AD - Department of Dermatology, University of California, Irvine. FAU - Chahal, Harvind S AU - Chahal HS AD - Department of Dermatology, University of California, Irvine. FAU - Doan, Linda T AU - Doan LT AD - Department of Dermatology, University of California, Irvine. FAU - Rojek, Nathan W AU - Rojek NW AD - Department of Dermatology, University of California, Irvine. LA - eng PT - Letter PL - United States TA - Cutis JT - Cutis JID - 0006440 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - *Botulinum Toxins MH - Humans MH - *Neuromuscular Agents MH - *Raynaud Disease/drug therapy EDAT- 2021/11/27 06:00 MHDA- 2022/01/27 06:00 CRDT- 2021/11/26 17:06 PHST- 2021/11/26 17:06 [entrez] PHST- 2021/11/27 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] AID - cutis.0355 [pii] AID - 10.12788/cutis.0355 [doi] PST - ppublish SO - Cutis. 2021 Sep;108(3):E11-E14. doi: 10.12788/cutis.0355. PMID- 24951141 OWN - NLM STAT- MEDLINE DCOM- 20150202 LR - 20140621 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 141 IP - 6-7 DP - 2014 Jun-Jul TI - [Nail-fold capillaroscopy in dermatology]. PG - 429-37 LID - S0151-9638(14)00275-0 [pii] LID - 10.1016/j.annder.2014.04.120 [doi] AB - Nail-fold capillaroscopy is a non-invasive tool to study the microcirculation and is increasingly being used in dermatology, angiology and rheumatology. More recently, the use of video-capillaroscopy has allowed computer storage of capillaroscopic images (video-capillaroscopy), enabling evaluation of changes in capillaroscopic abnormalities during the follow-up of patients with systemic sclerosis or mixed connective tissue disease. Qualitative and quantitative assessment of the nail-fold dermal capillaries and of their organization can readily distinguish between a normal capillaroscopic pattern in primary Raynaud phenomenon and a specific sclerodermic pattern in secondary Raynaud phenomenon carrying a very high risk of systemic sclerosis. Apart from its important role as a diagnostic tool for distinguishing between primary and secondary Raynaud phenomenon, capillaroscopy is now used to predict the risk of development of digital ulcers and of future visceral complications in patients with systemic sclerosis. Moreover, nail-fold capillaroscopy is essential for differential diagnosis between connective tissue diseases, for the etiologic diagnosis of digital necrosis and diffuse interstitial lung disease, and in sclerodermiform syndromes. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Senet, P AU - Senet P AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address: patricia.senet@tnn.aphp.fr. FAU - Fichel, F AU - Fichel F AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Baudot, N AU - Baudot N AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Gaitz, J-P AU - Gaitz JP AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Tribout, L AU - Tribout L AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. FAU - Frances, C AU - Frances C AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France; Université Paris-VI, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - La capillaroscopie péri-unguéale en dermatologie. DEP - 20140602 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Capillaries/ultrastructure MH - Cyanosis/diagnosis/pathology MH - Dermatology/*methods MH - Early Diagnosis MH - Fingers/*blood supply MH - Hemorrhage/diagnosis/pathology MH - Humans MH - Microcirculation MH - Microscopic Angioscopy/*methods MH - Nail Diseases/diagnosis/pathology MH - Nails MH - Odds Ratio MH - Raynaud Disease/diagnosis/pathology MH - Risk MH - Scleroderma, Systemic/*diagnosis/pathology MH - Skin Ulcer/diagnosis/pathology OTO - NOTNLM OT - Capillaroscopie OT - Connective tissue disease OT - Connectivites OT - Nail-fold capillaroscopy OT - Paysage sclérodermique OT - Phénomène de Raynaud OT - Raynaud phenomenon OT - Sclerodermic pattern OT - Sclérodermie OT - Systemic sclerosis EDAT- 2014/06/22 06:00 MHDA- 2015/02/03 06:00 CRDT- 2014/06/22 06:00 PHST- 2013/12/16 00:00 [received] PHST- 2014/03/11 00:00 [revised] PHST- 2014/04/02 00:00 [accepted] PHST- 2014/06/22 06:00 [entrez] PHST- 2014/06/22 06:00 [pubmed] PHST- 2015/02/03 06:00 [medline] AID - S0151-9638(14)00275-0 [pii] AID - 10.1016/j.annder.2014.04.120 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2014 Jun-Jul;141(6-7):429-37. doi: 10.1016/j.annder.2014.04.120. Epub 2014 Jun 2. PMID- 23682366 OWN - NLM STAT- MEDLINE DCOM- 20130528 LR - 20220311 IS - 2168-6084 (Electronic) IS - 2168-6068 (Linking) VI - 149 IP - 3 DP - 2013 Mar TI - Raynaud phenomenon of the nipple in breastfeeding mothers: an underdiagnosed cause of nipple pain. PG - 300-6 AB - OBJECTIVE: To elucidate the diagnostic criteria of Raynaud phenomenon of the nipple that will aid in recognizing and treating Raynaud phenomenon in breast feeding mothers with chronic deep nipple pain during lactation. DESIGN: Retrospective review of a patient database composed of 22 cases of breastfeeding mothers who fit the diagnostic criteria for Raynaud phenomenon of the nipple. SETTING: Menlo Dermatology Medical Group in Menlo Park, California, an academic-affiliated, private dermatologic referral center. PATIENTS: All patients diagnosed as having Raynaud phenomenon of the nipple evaluated from January 1, 2004,through December 31, 2010. MAIN OUTCOME MEASURES: The rate of failed treatment for Candida mastitis, the rate of improvement of symptoms with nifedipine use, and the overall rate of improvement of symptoms with appropriate therapy involving treatment of Raynaud phenomenon. RESULTS: Among the 22 patients with Raynaud phenomenon of the nipple, previous treatment for Candida mastitis with oral or topical antifungals was ineffective in 20(91%). Of the 12 patients who tolerated a trial of nifedipine,10 (83%) reported decreased or resolved nipple pain. All patients experienced marked improvement of symptoms with appropriate therapy involving treatment of Raynaud phenomenon. CONCLUSIONS: Most patients were treated with antifungals before presentation without resolution of nipple pain. Nifedipine appears to be an effective medication for the treatment of Raynaud phenomenon of the nipple. With appropriate management of Raynaud phenomenon,breastfeeding mothers demonstrated improvement of nipple pain. Raynaud phenomenon of the nipple should be considered in the differential diagnosis of nipple pain during lactation. FAU - Barrett, Meagan E AU - Barrett ME AD - University of Southern California, Keck School of Medicine, Los Angeles, CA, USA. FAU - Heller, Misha M AU - Heller MM FAU - Stone, Honor Fullerton AU - Stone HF FAU - Murase, Jenny E AU - Murase JE LA - eng PT - Journal Article PL - United States TA - JAMA Dermatol JT - JAMA dermatology JID - 101589530 RN - 0 (Antifungal Agents) SB - IM MH - Adult MH - Antifungal Agents/therapeutic use MH - Breast Diseases/*diagnosis/pathology MH - *Breast Feeding MH - Candidiasis/diagnosis/drug therapy MH - Databases, Factual MH - Female MH - Follow-Up Studies MH - Humans MH - Mastitis/diagnosis/drug therapy/microbiology MH - Middle Aged MH - Nipples MH - Pain/*etiology MH - Raynaud Disease/*diagnosis/pathology MH - Retrospective Studies MH - Treatment Failure EDAT- 2013/05/18 06:00 MHDA- 2013/05/29 06:00 CRDT- 2013/05/18 06:00 PHST- 2013/05/18 06:00 [entrez] PHST- 2013/05/18 06:00 [pubmed] PHST- 2013/05/29 06:00 [medline] AID - 10.1001/jamadermatol.2013.1560 [doi] PST - ppublish SO - JAMA Dermatol. 2013 Mar;149(3):300-6. doi: 10.1001/jamadermatol.2013.1560. PMID- 26195579 OWN - NLM STAT- MEDLINE DCOM- 20160201 LR - 20220331 IS - 1488-2329 (Electronic) IS - 0820-3946 (Print) IS - 0820-3946 (Linking) VI - 187 IP - 15 DP - 2015 Oct 20 TI - Lingual Raynaud phenomenon. PG - 1160 LID - 10.1503/cmaj.140784 [doi] FAU - Ghiam, Alireza Fotouhi AU - Ghiam AF AD - Department of Radiation Oncology (Ghiam, Cho), University of Toronto; Radiation Medicine Program (Ghiam, Cho), Princess Margaret Cancer Centre, University Health Network, Toronto, Ont. FAU - Cho, John AU - Cho J AD - Department of Radiation Oncology (Ghiam, Cho), University of Toronto; Radiation Medicine Program (Ghiam, Cho), Princess Margaret Cancer Centre, University Health Network, Toronto, Ont. john.cho@rmp.uhn.on.ca. LA - eng PT - Case Reports PT - Journal Article DEP - 20150720 PL - Canada TA - CMAJ JT - CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne JID - 9711805 SB - IM MH - Female MH - Humans MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - Tongue/blood supply/*pathology PMC - PMC4610842 EDAT- 2015/07/22 06:00 MHDA- 2016/02/02 06:00 PMCR- 2015/10/20 CRDT- 2015/07/22 06:00 PHST- 2015/07/22 06:00 [entrez] PHST- 2015/07/22 06:00 [pubmed] PHST- 2016/02/02 06:00 [medline] PHST- 2015/10/20 00:00 [pmc-release] AID - cmaj.140784 [pii] AID - 1871160 [pii] AID - 10.1503/cmaj.140784 [doi] PST - ppublish SO - CMAJ. 2015 Oct 20;187(15):1160. doi: 10.1503/cmaj.140784. Epub 2015 Jul 20. PMID- 31821859 OWN - NLM STAT- MEDLINE DCOM- 20210423 LR - 20210423 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 83 IP - 6 DP - 2020 Dec TI - Interdigital injection of botulinum toxin for patients with Raynaud phenomenon. PG - e399 LID - S0190-9622(19)33208-6 [pii] LID - 10.1016/j.jaad.2019.11.060 [doi] FAU - Quintana-Castanedo, Lucía AU - Quintana-Castanedo L AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. Electronic address: luciaquintana.e@gmail.com. FAU - Feito-Rodríguez, Marta AU - Feito-Rodríguez M AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - De Lucas-Laguna, Raúl AU - De Lucas-Laguna R AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. LA - eng PT - Journal Article PT - Video-Audio Media DEP - 20191207 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM CIN - J Am Acad Dermatol. 2020 Dec;83(6):e409-e410. doi: 10.1016/j.jaad.2020.02.085. PMID: 32244019 CIN - J Am Acad Dermatol. 2020 Dec;83(6):e411. doi: 10.1016/j.jaad.2020.03.066. PMID: 32244020 MH - Botulinum Toxins/*administration & dosage MH - Fingers MH - Humans MH - Injections/methods MH - Raynaud Disease/*drug therapy MH - Treatment Outcome OTO - NOTNLM OT - Raynaud phenomenon OT - botulinum toxin EDAT- 2019/12/11 06:00 MHDA- 2021/04/24 06:00 CRDT- 2019/12/11 06:00 PHST- 2019/11/11 00:00 [received] PHST- 2019/11/18 00:00 [accepted] PHST- 2019/12/11 06:00 [pubmed] PHST- 2021/04/24 06:00 [medline] PHST- 2019/12/11 06:00 [entrez] AID - S0190-9622(19)33208-6 [pii] AID - 10.1016/j.jaad.2019.11.060 [doi] PST - ppublish SO - J Am Acad Dermatol. 2020 Dec;83(6):e399. doi: 10.1016/j.jaad.2019.11.060. Epub 2019 Dec 7. PMID- 34952662 OWN - NLM STAT- MEDLINE DCOM- 20220217 LR - 20220217 IS - 1097-6833 (Electronic) IS - 0022-3476 (Linking) VI - 240 DP - 2022 Jan TI - 50 Years Ago in TheJournalofPediatrics: What's Behind Raynaud Phenomenon? PG - 248 LID - S0022-3476(21)01020-9 [pii] LID - 10.1016/j.jpeds.2021.10.027 [doi] FAU - Ashkenazi, Limor AU - Ashkenazi L AD - Pediatric Rheumatology Unit, Shaare Zedek Medical Center, Jerusalem, Israel. FAU - Hashkes, Philip J AU - Hashkes PJ AD - Pediatric Rheumatology Unit, Shaare Zedek Medical Center, Jerusalem, Israel. LA - eng PT - Journal Article PL - United States TA - J Pediatr JT - The Journal of pediatrics JID - 0375410 SB - IM MH - Diagnosis, Differential MH - Female MH - Humans MH - Infant MH - Mucocutaneous Lymph Node Syndrome/diagnosis MH - Raynaud Disease/*diagnosis EDAT- 2021/12/26 06:00 MHDA- 2022/02/19 06:00 CRDT- 2021/12/25 05:17 PHST- 2021/12/25 05:17 [entrez] PHST- 2021/12/26 06:00 [pubmed] PHST- 2022/02/19 06:00 [medline] AID - S0022-3476(21)01020-9 [pii] AID - 10.1016/j.jpeds.2021.10.027 [doi] PST - ppublish SO - J Pediatr. 2022 Jan;240:248. doi: 10.1016/j.jpeds.2021.10.027. PMID- 26501577 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20260127 IS - 1547-1896 (Print) IS - 0893-7400 (Linking) VI - 28 IP - 11 DP - 2015 Nov TI - Recognizing and treating Raynaud phenomenon. PG - 40-4 LID - 10.1097/01.JAA.0000471610.61784.da [doi] AB - Raynaud phenomenon is an episodic ischemia caused by cold, emotional stimuli, or rheumatologic disorders, and is more common in women than men. This article describes the clinical presentation of Raynaud phenomenon and how to diagnose and appropriately treat patients and prevent complications. Research on the use of different pharmacologic interventions for Raynaud phenomenon is ongoing and treatment options are expanding. FAU - Thoelen, Kristen AU - Thoelen K AD - Kristen Thoelen, Kimberly Maiers, and Kristy-Ann Bondi are graduates of the PA program at Pace University Lenox Hill Hospital in New York City. Jean Covino is an associate clinical professor at Pace University Lenox Hill Hospital. The authors have disclosed no potential conflicts of interest, financial or otherwise. FAU - Maiers, Kimberly AU - Maiers K FAU - Bondi, Kristy-Ann AU - Bondi KA FAU - Covino, Jean AU - Covino J LA - eng PT - Journal Article PT - Review PL - United States TA - JAAPA JT - JAAPA : official journal of the American Academy of Physician Assistants JID - 9513102 RN - 0 (Calcium Channel Blockers) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins, Synthetic) RN - 0 (Selective Serotonin Reuptake Inhibitors) MH - Blood Sedimentation MH - Calcium Channel Blockers/*therapeutic use MH - Humans MH - Life Style MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Physical Examination MH - Prostaglandins, Synthetic/*therapeutic use MH - *Protective Clothing MH - Raynaud Disease/diagnosis/*therapy MH - Selective Serotonin Reuptake Inhibitors/*therapeutic use EDAT- 2015/10/27 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/10/27 06:00 PHST- 2015/10/27 06:00 [entrez] PHST- 2015/10/27 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 01720610-201511000-00007 [pii] AID - 10.1097/01.JAA.0000471610.61784.da [doi] PST - ppublish SO - JAAPA. 2015 Nov;28(11):40-4. doi: 10.1097/01.JAA.0000471610.61784.da. PMID- 16432094 OWN - NLM STAT- MEDLINE DCOM- 20060223 LR - 20071115 IS - 0003-9926 (Print) IS - 0003-9926 (Linking) VI - 166 IP - 2 DP - 2006 Jan 23 TI - Phosphodiesterase type 5 inhibition is a novel therapeutic option in Raynaud disease. PG - 231-3 AB - BACKGROUND: Raynaud disease (RD) is a common disorder affecting 3% to 5% of the healthy population, and occurs in more than 90% of patients with connective tissue diseases. The therapeutic options remain limited, particularly in patients with secondary RD due to connective tissue disease. Theoretical considerations lead to the expectation that phosphodiesterase type 5 inhibitors may improve clinical symptoms and digital blood flow in patients with RD. METHODS: We conducted an open-label pilot study in 40 patients with RD, 33 (82%) of whom had secondary and 7 (18%) of whom had primary RD. Digital blood flow was measured by laser-Doppler flowmetry at room temperature and during the cold-exposure test before medical treatment, 1 hour after the initial intake, and after 2 weeks of continuous treatment (10 mg twice a day) with the novel phosphodiesterase type 5 inhibitor vardenafil. Clinical symptoms were recorded by a patient questionnaire and summarized as the Raynaud condition score. RESULTS: Laser-Doppler flowmetry revealed that vardenafil improved digital blood flow in 28 (70%) patients, whereas 12 (30%) did not respond. In individuals responding, digital blood flow significantly increased by a mean +/- SEM of 21.0% +/- 4.9% and 30.0% +/- 5.7% at 1 hour and 2 weeks of treatment at room temperature, respectively, and by 18.8% +/- 4.4% and 35.1% +/- 7.5% at 1 hour and 2 weeks during the cold-exposure test, respectively (P < .01 for all). Consistently, clinical symptoms improved in 27 (68%) of the 40 patients, and the Raynaud condition score declined from a mean +/- SEM of 5.05 +/- 0.38 to 3.54 +/- 0.31 (P < .001). CONCLUSION: Our data indicate that phosphodiesterase type 5 inhibition significantly improves peripheral blood flow and clinical symptoms in a large subset of patients with RD and, thus, may provide a novel therapeutic approach in such individuals. FAU - Caglayan, Evren AU - Caglayan E AD - Klinik III für Innere Medizin, Universität zu Köln, Köln, Germany. FAU - Huntgeburth, Michael AU - Huntgeburth M FAU - Karasch, Thomas AU - Karasch T FAU - Weihrauch, Julia AU - Weihrauch J FAU - Hunzelmann, Nicolas AU - Hunzelmann N FAU - Krieg, Thomas AU - Krieg T FAU - Erdmann, Erland AU - Erdmann E FAU - Rosenkranz, Stephan AU - Rosenkranz S LA - eng PT - Clinical Trial PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arch Intern Med JT - Archives of internal medicine JID - 0372440 RN - 0 (Phosphodiesterase Inhibitors) RN - EC 3.1.4.- (Phosphoric Diester Hydrolases) RN - EC 3.1.4.35 (3',5'-Cyclic-GMP Phosphodiesterases) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) RN - EC 3.1.4.35 (PDE5A protein, human) SB - IM MH - 3',5'-Cyclic-GMP Phosphodiesterases MH - Adult MH - Blood Flow Velocity MH - Cyclic Nucleotide Phosphodiesterases, Type 5 MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Female MH - Follow-Up Studies MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Middle Aged MH - Patient Satisfaction MH - Phosphodiesterase Inhibitors/*therapeutic use MH - Phosphoric Diester Hydrolases MH - Pilot Projects MH - Probability MH - Raynaud Disease/diagnosis/*drug therapy/*physiopathology MH - Risk Assessment MH - Severity of Illness Index MH - Treatment Outcome EDAT- 2006/01/25 09:00 MHDA- 2006/02/24 09:00 CRDT- 2006/01/25 09:00 PHST- 2006/01/25 09:00 [pubmed] PHST- 2006/02/24 09:00 [medline] PHST- 2006/01/25 09:00 [entrez] AID - 166/2/231 [pii] AID - 10.1001/archinte.166.2.231 [doi] PST - ppublish SO - Arch Intern Med. 2006 Jan 23;166(2):231-3. doi: 10.1001/archinte.166.2.231. PMID- 25644911 OWN - NLM STAT- MEDLINE DCOM- 20150421 LR - 20170522 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 147 IP - 2 DP - 2015 Feb TI - Therapeutic hypothermia after cardiac arrest in a patient with systemic sclerosis and Raynaud phenomenon. PG - e27-e30 LID - S0012-3692(15)30197-5 [pii] LID - 10.1378/chest.14-1184 [doi] AB - Therapeutic hypothermia favorably impacts neurologic outcomes in patients after cardiopulmonary arrest, although the appropriate target temperature is less clear. Its safety profile in patients with systemic sclerosis (SSc) and Raynaud phenomenon (RP), who may be at increased risk for ischemic complications, has not been addressed in the literature, to our knowledge. Digital lesions are commonly seen in patients with SSc, and cold-induced myocardial ischemia has also been reported. We describe a case of a man with SSc, RP, and digital ulcers who underwent therapeutic hypothermia after cardiopulmonary arrest. He regained full neurologic function, and except for digital necrosis, no hypothermia-associated adverse events were observed. Other risk factors for ischemia, such as cocaine use, may have contributed to the development of the digital necrosis. However, clinicians should be aware of the risk for ischemic complications in patients with SSc and RP when considering the appropriate target temperature after cardiopulmonary arrest. FAU - Bakal, Keren AU - Bakal K AD - Department of Medicine, NYU School of Medicine, US Department of Veterans Affairs. FAU - Danckers, Mauricio AU - Danckers M AD - Department of Medicine, NYU School of Medicine, US Department of Veterans Affairs. FAU - Denson, Joshua L AU - Denson JL AD - Department of Medicine, NYU School of Medicine, US Department of Veterans Affairs. FAU - Sauthoff, Harald AU - Sauthoff H AD - VA NY Harbor Healthcare System, US Department of Veterans Affairs, New York, NY; Department of Medicine, NYU School of Medicine, US Department of Veterans Affairs. Electronic address: Harald.sauthoff@nyumc.org. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 SB - IM MH - Comorbidity MH - Fingers/pathology MH - Heart Arrest/*epidemiology MH - Humans MH - *Hypothermia, Induced MH - Male MH - Middle Aged MH - Necrosis MH - Raynaud Disease/*epidemiology/*therapy MH - Scleroderma, Systemic/*epidemiology/*therapy MH - Skin Ulcer/therapy EDAT- 2015/02/04 06:00 MHDA- 2015/04/22 06:00 CRDT- 2015/02/04 06:00 PHST- 2015/02/04 06:00 [entrez] PHST- 2015/02/04 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] AID - S0012-3692(15)30197-5 [pii] AID - 10.1378/chest.14-1184 [doi] PST - ppublish SO - Chest. 2015 Feb;147(2):e27-e30. doi: 10.1378/chest.14-1184. PMID- 16936602 OWN - NLM STAT- MEDLINE DCOM- 20061012 LR - 20071115 IS - 0966-0461 (Print) IS - 0966-0461 (Linking) VI - 15 IP - 15 DP - 2006 Aug 10-Sep 13 TI - Autoimmune connective tissue disease: scleroderma. PG - 805-9 AB - Scleroderma is an umbrella term for a spectrum of rare and complex autoimmune connective tissue diseases, the cause and pathogenesis of which is only partially defined. Scleroderma can be divided into two main subgroups--systemic and localized--but the hallmark of both is skin fibrosis. As yet no drug has been found to be effective in reversing the disease process, however early intervention has been shown to give maximum benefit. Due to the chronic nature of the condition a multidisciplinary approach is essential and the nurse's input from an early stage is vital in supporting the patient to manage both their medical treatment and their activities of daily living. FAU - Wilson, Helen AU - Wilson H AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Vincent, Rachel AU - Vincent R LA - eng PT - Journal Article PT - Review PL - England TA - Br J Nurs JT - British journal of nursing (Mark Allen Publishing) JID - 9212059 MH - Activities of Daily Living MH - Adaptation, Psychological MH - *Autoimmune Diseases/diagnosis/etiology/therapy MH - Causality MH - Diagnosis, Differential MH - Early Diagnosis MH - Humans MH - Information Services MH - Internet MH - Nurse's Role/psychology MH - Nursing Assessment MH - Patient Care Team MH - Patient Education as Topic MH - Prognosis MH - Rare Diseases MH - Raynaud Disease/diagnosis/etiology/therapy MH - *Scleroderma, Localized/diagnosis/etiology/therapy MH - *Scleroderma, Systemic/diagnosis/etiology/therapy MH - Skin Care MH - Social Support RF - 15 EDAT- 2006/08/29 09:00 MHDA- 2006/10/13 09:00 CRDT- 2006/08/29 09:00 PHST- 2006/08/29 09:00 [pubmed] PHST- 2006/10/13 09:00 [medline] PHST- 2006/08/29 09:00 [entrez] AID - 10.12968/bjon.2006.15.15.21685 [doi] PST - ppublish SO - Br J Nurs. 2006 Aug 10-Sep 13;15(15):805-9. doi: 10.12968/bjon.2006.15.15.21685. PMID- 25539337 OWN - NLM STAT- MEDLINE DCOM- 20150309 LR - 20210106 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 135 IP - 1 DP - 2015 Jan TI - Fat grafting to the hand in patients with raynaud phenomenon: a novel therapeutic modality. PG - 229e-230e LID - 10.1097/PRS.0000000000000814 [doi] FAU - Pavlidis, Leonidas AU - Pavlidis L AD - Aristotle University of Thessaloniki, Thessaloniki, Greece. FAU - Sapountzis, Stamatis AU - Sapountzis S FAU - Spyropoulou, Georgia Alexandra AU - Spyropoulou GA FAU - Demiri, Efterpi AU - Demiri E LA - eng PT - Comment PT - Letter PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CON - Plast Reconstr Surg. 2014 May;133(5):1109-1118. doi: 10.1097/PRS.0000000000000104. PMID: 24445877 CIN - Plast Reconstr Surg. 2015 Jan;135(1):230e-231e. doi: 10.1097/PRS.0000000000000789. PMID: 25539338 MH - Adipose Tissue/*transplantation MH - Female MH - Hand/*blood supply/*surgery MH - Humans MH - Ischemia/*surgery MH - Male MH - Raynaud Disease/*surgery EDAT- 2014/12/30 06:00 MHDA- 2015/03/10 06:00 CRDT- 2014/12/25 06:00 PHST- 2014/12/25 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2015/03/10 06:00 [medline] AID - 00006534-201501000-00064 [pii] AID - 10.1097/PRS.0000000000000814 [doi] PST - ppublish SO - Plast Reconstr Surg. 2015 Jan;135(1):229e-230e. doi: 10.1097/PRS.0000000000000814. PMID- 20859229 OWN - NLM STAT- MEDLINE DCOM- 20110325 LR - 20101005 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 16 IP - 7 DP - 2010 Oct TI - Antisynthetase syndrome presenting as rheumatoid-like polyarthritis. PG - 307-12 LID - 10.1097/RHU.0b013e3181f3bf9c [doi] AB - BACKGROUND: The antisynthetase syndrome is a systemic inflammatory disease associated with anti-tRNA synthetase antibodies and consisting of the clinical features of inflammatory myopathy arthritis, interstitial lung disease (ILD), fever, Raynaud syndrome, and rash. It rarely presents with symmetric arthritis as the initial manifestation of the disease. OBJECTIVE: The aim of the study was to describe the clinical, laboratory, and radiographic characteristics of patients with antisynthetase syndrome who presented with symptoms of inflammatory arthritis, mimicking rheumatoid arthritis (RA) at the time of initial evaluation. METHODS: Six cases derived from a single university-based rheumatology clinic in Wisconsin are presented. The major clinical, laboratory, radiographic, and histopathologic data are described. RESULTS: All 6 patients demonstrated symmetric synovitis involving the hands. Five patients met the American College of Rheumatology classification criteria for RA. Three patients had nail-fold capillary abnormalities, and 4 patients were observed to have Raynaud phenomenon. Three patients demonstrated a cytoplasmic pattern when testing for antinuclear antibodies by immunofluorescent assay, and all had t-RNA synthetase antibodies. Two patients had positive rheumatoid factors, but none had strongly positive cyclic citrullinated peptide antibodies. None of the patients demonstrated radiographic erosions. All patients had evidence of ILD by imaging or pulmonary function testing. Prognosis was generally favorable, although disease severity and treatment varied considerably. CONCLUSION: In patients who present with features mimicking but atypical for RA, such as early ILD, nail-fold capillary abnormalities, Raynaud phenomenon, cytoplasmic antinuclear antibody pattern, negative cyclic citrullinated peptide antibody status, and nonerosive arthritis, the antisynthetase syndrome should be considered. FAU - Mumm, Gregory E AU - Mumm GE AD - University of Wisconsin Hospital and Clinics, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA. FAU - McKown, Kevin M AU - McKown KM FAU - Bell, Carolyn L AU - Bell CL LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Adult MH - Amino Acyl-tRNA Synthetases/*immunology MH - Antibodies/blood MH - Arthritis, Rheumatoid/*diagnosis/enzymology/etiology MH - Female MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/enzymology/etiology MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/enzymology/etiology MH - Syndrome EDAT- 2010/09/23 06:00 MHDA- 2011/03/26 06:00 CRDT- 2010/09/23 06:00 PHST- 2010/09/23 06:00 [entrez] PHST- 2010/09/23 06:00 [pubmed] PHST- 2011/03/26 06:00 [medline] AID - 10.1097/RHU.0b013e3181f3bf9c [doi] PST - ppublish SO - J Clin Rheumatol. 2010 Oct;16(7):307-12. doi: 10.1097/RHU.0b013e3181f3bf9c. PMID- 20542965 OWN - NLM STAT- MEDLINE DCOM- 20120521 LR - 20151119 IS - 1525-1489 (Electronic) IS - 0885-0666 (Linking) VI - 25 IP - 5 DP - 2010 Sep TI - Care of patients with scleroderma in the intensive care setting. PG - 247-58 LID - 10.1177/0885066610371181 [doi] AB - Scleroderma or systemic sclerosis (SSc) is a connective tissue disease (CTD) associated with fibrosing and vascular complications involving multiple organs. The care of these patients in the critical care setting is frequently challenging due to multiple complications and refractory organ involvement. However, awareness of specific organ involvement associated with scleroderma can allow many complications to be anticipated and effectively treated. Cardiac involvement can lead to arrhythmias and heart failure, whereas pulmonary involvement can be associated with pulmonary arterial hypertension, fibrosis, or both. Renal vascular disease and scleroderma renal crisis (SRC), once a uniformly fatal complication, is particularly important to recognize early, as it can be treated successfully. Gastrointestinal involvement can lead to bleeding, aspiration, obstruction, and malabsorption. Severe Raynaud may lead to digital ischemia and gangrene. Therapies must target involved organ system or organ systems. Corticosteroids, a mainstay for related CTDs, do not typically provide any benefit and may cause harm. Vasodilators can effectively treat vascular complications but must target the appropriate vascular bed. Proactive utilization of proton pump inhibitors, recognition of bleeding from gastrointestinal vascular ectasia, and nutritional support can considerably ameliorate gastrointestinal morbidities. Effective treatment of fibrotic complications remains elusive and is the current frontier for scleroderma therapeutics. FAU - Farber, Harrison W AU - Farber HW AD - Department of Medicine, Pulmonary Center, Boston University School of Medicine, Boston, MA, USA. FAU - Simms, Robert W AU - Simms RW FAU - Lafyatis, Robert AU - Lafyatis R LA - eng PT - Journal Article PT - Review DEP - 20100611 PL - United States TA - J Intensive Care Med JT - Journal of intensive care medicine JID - 8610344 SB - IM MH - *Critical Care MH - Gastrointestinal Diseases/etiology/therapy MH - Heart Diseases/etiology/therapy MH - Humans MH - Hypertension, Pulmonary/etiology/therapy MH - Lung Diseases/etiology/therapy MH - Pericardium MH - Raynaud Disease/complications/etiology/therapy MH - Renal Insufficiency/etiology/therapy MH - Scleroderma, Systemic/*complications/*therapy MH - Vascular Diseases/etiology/therapy EDAT- 2010/06/15 06:00 MHDA- 2012/05/23 06:00 CRDT- 2010/06/15 06:00 PHST- 2010/06/15 06:00 [entrez] PHST- 2010/06/15 06:00 [pubmed] PHST- 2012/05/23 06:00 [medline] AID - 0885066610371181 [pii] AID - 10.1177/0885066610371181 [doi] PST - ppublish SO - J Intensive Care Med. 2010 Sep;25(5):247-58. doi: 10.1177/0885066610371181. Epub 2010 Jun 11. PMID- 17937194 OWN - NLM STAT- MEDLINE DCOM- 20071105 LR - 20071115 IS - 1592-7830 (Print) IS - 1592-7830 (Linking) VI - 29 IP - 2 Suppl DP - 2007 TI - [Guidelines for prevention of disorders and pathologies caused by exposure to mechanical vibrations in work environment]. PG - 4-54 FAU - Bovenzi, Massimo AU - Bovenzi M AD - Medicina del Lavoro, Università di Trieste. FAU - Angotzi, Giuliano AU - Angotzi G FAU - Apostoli, Pietro AU - Apostoli P FAU - Negro, Corrado AU - Negro C FAU - Versini, Walter AU - Versini W LA - ita PT - Comparative Study PT - Journal Article TT - Linee guida per la prevenzione dei disturbi e delle patologie da esposizione a vibrazioni meccaniche negli ambienti di lavoro. PL - Italy TA - G Ital Med Lav Ergon JT - Giornale italiano di medicina del lavoro ed ergonomia JID - 9712708 SB - IM MH - European Union MH - Humans MH - Italy MH - Models, Theoretical MH - Occupational Diseases/diagnosis/etiology/*prevention & control/rehabilitation/therapy MH - Occupational Exposure/*adverse effects MH - Practice Guidelines as Topic MH - Raynaud Disease/diagnosis/etiology MH - Risk Assessment MH - Risk Factors MH - Time Factors MH - Vibration/*adverse effects EDAT- 2007/10/17 09:00 MHDA- 2007/11/06 09:00 CRDT- 2007/10/17 09:00 PHST- 2007/10/17 09:00 [pubmed] PHST- 2007/11/06 09:00 [medline] PHST- 2007/10/17 09:00 [entrez] PST - ppublish SO - G Ital Med Lav Ergon. 2007;29(2 Suppl):4-54. PMID- 29017842 OWN - NLM STAT- MEDLINE DCOM- 20181012 LR - 20181012 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 78 IP - 3 DP - 2018 Mar TI - The Frisbee maneuver: A novel method to abort acute attacks of the Raynaud phenomenon. PG - e61 LID - S0190-9622(17)32476-3 [pii] LID - 10.1016/j.jaad.2017.09.066 [doi] FAU - Curtiss, Paul AU - Curtiss P AD - Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Cobos, Gabriela AU - Cobos G AD - Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Lo Sicco, Kristen AU - Lo Sicco K AD - Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. FAU - Franks, Andrew G Jr AU - Franks AG Jr AD - Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. Electronic address: Andrew.Franks@nyumc.org. LA - eng PT - Journal Article PT - Video-Audio Media DEP - 20171007 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Acute Disease MH - Humans MH - *Movement MH - Raynaud Disease/*prevention & control MH - Upper Extremity EDAT- 2017/10/12 06:00 MHDA- 2018/10/13 06:00 CRDT- 2017/10/12 06:00 PHST- 2017/08/25 00:00 [received] PHST- 2017/09/22 00:00 [revised] PHST- 2017/09/29 00:00 [accepted] PHST- 2017/10/12 06:00 [pubmed] PHST- 2018/10/13 06:00 [medline] PHST- 2017/10/12 06:00 [entrez] AID - S0190-9622(17)32476-3 [pii] AID - 10.1016/j.jaad.2017.09.066 [doi] PST - ppublish SO - J Am Acad Dermatol. 2018 Mar;78(3):e61. doi: 10.1016/j.jaad.2017.09.066. Epub 2017 Oct 7. PMID- 31577265 OWN - NLM STAT- MEDLINE DCOM- 20200918 LR - 20200918 IS - 1897-9483 (Electronic) IS - 0032-3772 (Linking) VI - 129 IP - 11 DP - 2019 Nov 29 TI - Recent advances in the workup and management of Raynaud phenomenon. PG - 798-808 LID - 10.20452/pamw.15008 [doi] AB - Raynaud phenomenon (RP) is defined as recurrent, reversible episodes of vasospasm involving peripheral small vessels, typically in the fingers and toes. Primary (idiopathic) RP is common (it occurrs in about 5% of the general population) and is usually benign. Secondary RP accounts for 10% to 20% of all RP cases and may be associated with complications such as tissue loss, ulcers, and gangrene. Systemic sclerosis (SSc) or, more rarely, other connective tissue diseases are the main underlying conditions. A detailed clinical history and careful physical examination may be helpful in identifying the cause. Routine investigations include a full blood count, measurement of erythrocyte sedimentation rate, C‑reactive protein, antinuclear antibody levels, biochemical profile, thyroid function tests, protein electrophoresis, chest X‑ray, and nailfold capillaroscopy. Capillaroscopy can facilitate a very early diagnosis of SSc. Doppler ultrasound is recommended to evaluate the risk of pathologies in large to medium-sized arteries. Lifestyle modifications may be sufficient to control primary RP, but some patients, and most with secondary RP, require pharmacologic treatment. Several medications are proposed to manage RP and its complications, such as calcium channel blockers, phosphodiesterase type 5 inhibitors, intravenous prostanoids, and topical nitrates. However, scientific evidence for the use of these drugs is still weak to moderate. Despite the lack of efficacy of bosentan in RP treatment, this medication is approved for the secondary prevention of digital ulcers in patients with SSc. In conclusion, the management of RP still represents a challenge. Collaboration between healthcare professionals, patient organizations, and the society could encourage earlier medical assessment of people at risk of SSc. FAU - Lis-Święty, Anna AU - Lis-Święty A AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. alis-swiety@sum.edu.pl LA - eng PT - Journal Article PT - Review DEP - 20191002 PL - Poland TA - Pol Arch Intern Med JT - Polish archives of internal medicine JID - 101700960 SB - IM MH - Fingers/*blood supply MH - Humans MH - Raynaud Disease/*diagnosis/*physiopathology/*therapy EDAT- 2019/10/03 06:00 MHDA- 2020/09/20 06:00 CRDT- 2019/10/03 06:00 PHST- 2019/10/03 06:00 [pubmed] PHST- 2020/09/20 06:00 [medline] PHST- 2019/10/03 06:00 [entrez] AID - 10.20452/pamw.15008 [doi] PST - ppublish SO - Pol Arch Intern Med. 2019 Nov 29;129(11):798-808. doi: 10.20452/pamw.15008. Epub 2019 Oct 2. PMID- 34629385 OWN - NLM STAT- MEDLINE DCOM- 20220124 LR - 20230923 IS - 1538-8654 (Electronic) IS - 1527-7941 (Linking) VI - 35 IP - 2 DP - 2022 Feb 1 TI - From Raynaud Phenomenon to Systemic Sclerosis in COVID-19: A Case Report. PG - 123-124 LID - 10.1097/01.ASW.0000795240.63966.53 [doi] AB - In 2019, the novel SARS-CoV-2 infection emerged, causing the disease called COVID-19, which primarily affects the respiratory tract and lung at alveolar and interstitial levels. Systemic sclerosis (SSc) is an autoimmune connective disease characterized by vascular abnormalities and diffuse and progressive fibrosis of the skin and internal organs. Raynaud phenomenon (RP) occurs in virtually all patients affected by SSc and, in most cases, is an onset symptom of the disease; that is, RP may appear several years before overt illness. Although the exact pathophysiologic pathways leading to RP and SSc are still unknown, several infectious agents, especially viruses, have been suggested as possible triggering factors. Here, the authors describe the first case of RP secondary to SSc following SARS-CoV-2 infection. CI - Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved. FAU - Giuggioli, Dilia AU - Giuggioli D AD - In the Rheumatology Unit, University of Modena and Reggio Emilia Medical School, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Italy, Dilia Giuggioli, MD, PhD, is Associate Professor; Amelia Spinella, MD, PhD, is Medical Assistant; Marco de Pinto, MD, is Resident; Mascia Maria Teresa, MD, is Associate Professor; and Carlo Salvarani, MD, is Full Professor. The authors have disclosed no financial relationships related to this article. Submitted December 21, 2020; accepted in revised form March 15, 2021; published online ahead of print October 6, 2021. FAU - Spinella, Amelia AU - Spinella A FAU - de Pinto, Marco AU - de Pinto M FAU - Mascia, Maria Teresa AU - Mascia MT FAU - Salvarani, Carlo AU - Salvarani C LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Adv Skin Wound Care JT - Advances in skin & wound care JID - 100911021 MH - *COVID-19 MH - Humans MH - *Raynaud Disease/diagnosis/etiology MH - SARS-CoV-2 MH - *Scleroderma, Systemic/complications/diagnosis/therapy MH - Skin EDAT- 2021/10/12 06:00 MHDA- 2022/01/27 06:00 CRDT- 2021/10/11 05:42 PHST- 2021/10/12 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] PHST- 2021/10/11 05:42 [entrez] AID - 00129334-202202000-00012 [pii] AID - 10.1097/01.ASW.0000795240.63966.53 [doi] PST - ppublish SO - Adv Skin Wound Care. 2022 Feb 1;35(2):123-124. doi: 10.1097/01.ASW.0000795240.63966.53. PMID- 25077719 OWN - NLM STAT- MEDLINE DCOM- 20140924 LR - 20140801 IS - 1532-0650 (Electronic) IS - 0002-838X (Linking) VI - 90 IP - 3 DP - 2014 Aug 1 TI - Effectiveness of calcium channel blockers for Raynaud phenomenon. PG - 143-4 FAU - Seehusen, Dean A AU - Seehusen DA AD - Fort Belvoir Community Hospital, Fort Belvoir, VA, USA. FAU - Huang, Joseph AU - Huang J AD - Fort Belvoir Community Hospital, Fort Belvoir, VA, USA. LA - eng PT - Journal Article PT - Meta-Analysis PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 RN - 0 (Calcium Channel Blockers) SB - IM MH - Calcium Channel Blockers/adverse effects/*therapeutic use MH - Humans MH - Raynaud Disease/*drug therapy MH - Treatment Outcome EDAT- 2014/08/01 06:00 MHDA- 2014/09/25 06:00 CRDT- 2014/08/01 06:00 PHST- 2014/08/01 06:00 [entrez] PHST- 2014/08/01 06:00 [pubmed] PHST- 2014/09/25 06:00 [medline] AID - d11599 [pii] PST - ppublish SO - Am Fam Physician. 2014 Aug 1;90(3):143-4. PMID- 31576813 OWN - NLM STAT- MEDLINE DCOM- 20200302 LR - 20251101 IS - 1643-3750 (Electronic) IS - 1234-1010 (Print) IS - 1234-1010 (Linking) VI - 25 DP - 2019 Oct 2 TI - Computed Tomography (CT)-Guided Percutaneous Thoracic Sympathetic Chain Radiofrequency Thermocoagulation for Raynaud Disease. PG - 7391-7395 LID - 10.12659/MSM.917392 [doi] AB - BACKGROUND We introduce a minimally invasive technique for the treatment of Raynaud disease - CT-guided percutaneous thoracic sympathetic chain radiofrequency thermocoagulation. MATERIAL AND METHODS Under CT guidance, the radiofrequency needle was punctured from the upper edge of the costotransverse joint to the anterior superior edge of the 4th capitulum costae and the lateral parietal pleura. After sensorial (1.5 mA, 50 Hz) and motorial (1.5 mA, 2Hz) testing to determine that there was no nerve innervation zone with muscle numbness and twitches, radiofrequency coagulation was set at 95°C for 300 s. RESULTS A total of 17 patients were enrolled in the treatment group. All the patients underwent CT-guided percutaneous thoracic sympathetic chain puncture of the needles to the upper edge of the 4th capitulum costae on both sides. The perfusion index (PI) of the fingers began to rise 30 s after radiofrequency thermocoagulation, and the palm temperature (T) began to rise after 90 s. At the end of treatment, PI increased by an average of 4.6-fold, and the T average rose by 3.6°C. Postoperative cold-water stimulation testing could no longer induce Raynaud disease. Follow-up was conducted for 1 to 15 months. Two patients were found to have recurrence at 9 months and 13 months, respectively. CONCLUSIONS CT-guided percutaneous thoracic sympathetic nerve chain radiofrequency coagulation can effectively treat Raynaud disease. FAU - Huang, Hao AU - Huang H AD - Department of Anesthesiology and Pain Medicine, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China (mainland). FAU - Qiu, Weidong AU - Qiu W AD - Department of Anesthesiology and Pain Medicine, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China (mainland). FAU - Chen, Qunshan AU - Chen Q AD - Department of Anesthesiology and Pain Medicine, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China (mainland). FAU - Sun, Kai AU - Sun K AD - Department of Anesthesiology and Pain Medicine, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China (mainland). FAU - Huang, Bing AU - Huang B AD - Department of Pain Medicine, The First Hospital of Jiaxing, Jiaxing, Zhejiang, China (mainland). LA - eng PT - Journal Article DEP - 20191002 PL - United States TA - Med Sci Monit JT - Medical science monitor : international medical journal of experimental and clinical research JID - 9609063 SB - IM MH - Adult MH - China MH - Electrocoagulation/*methods MH - Female MH - Humans MH - Male MH - Middle Aged MH - Radio Waves MH - Radiofrequency Therapy/*methods MH - Raynaud Disease/*therapy MH - Tomography, X-Ray Computed MH - Treatment Outcome PMC - PMC12574465 EDAT- 2019/10/03 06:00 MHDA- 2020/03/03 06:00 PMCR- 2019/10/02 CRDT- 2019/10/03 06:00 PHST- 2019/10/03 06:00 [entrez] PHST- 2019/10/03 06:00 [pubmed] PHST- 2020/03/03 06:00 [medline] PHST- 2019/10/02 00:00 [pmc-release] AID - 917392 [pii] AID - 10.12659/MSM.917392 [doi] PST - epublish SO - Med Sci Monit. 2019 Oct 2;25:7391-7395. doi: 10.12659/MSM.917392. PMID- 40371880 OWN - NLM STAT- MEDLINE DCOM- 20250929 LR - 20250929 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 31 IP - 7 DP - 2025 Oct 1 TI - Management Practices for Raynaud Phenomenon in Patients With Systemic Sclerosis: A Real-World Data From Community-Based Practices in the United States. PG - 279-283 LID - 10.1097/RHU.0000000000002242 [doi] AB - OBJECTIVE: To describe Raynaud phenomenon (RP) management practices for systemic sclerosis (SSc) patients among US community-based rheumatologists. METHODS: We identified all adult SSc patients, diagnosed by a rheumatologist, from the FORWARD Databank between 1999 and 2023. We evaluated longitudinal RP medication use, from data collected by semiannual questionnaires. We evaluated factors associated with RP medication use with multivariable Andersen and Gill Cox proportional models. RESULTS: Of the 270 SSc patients, 61% received a medication for RP over the median (interquartile range) follow-up of 3.4 (1.3-7.8) years. Calcium-channel blockers were the most chosen overall (48%) and first-line (75%) medication, followed by renin-angiotensin system inhibitors (18% [23%]). The use of RP medications persistently (29%), combination regimens (20%), and advanced therapies (15%; phosphodiesterase-5 inhibitors [PDE5i], endothelin receptor antagonists, or prostaglandin analogs) throughout the follow-up was low. Whereas calcium-channel blocker use has declined, PDE5i use has increased since 2019. Factors associated with initiating medications for RP were hypertension (hazard ratio [HR], 1.57; 95% confidence interval [CI], 1.25-1.98), pulmonary disease (HR, 1.25; 95% CI, 1.04-1.52), immunomodulatory use (HR, 1.32; 95% CI, 1.04-1.68), higher annual income (HR, 1.33; 95% CI, 1.02-1.73), and having an insurance (HR, 2.37; 95% CI, 1.04-5.44). CONCLUSION: Overall use of RP medication was low with poor maintenance rates in less than one-third of the patients from this community sample. The pattern of RP medication use changed over time with increasing use of PDE5i use since 2019. Although socioeconomic factors had impact on RP medication initiation, there is also a need for education and guideline recommendations to assist community-based rheumatologists in RP management. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Ozen, Gulsen AU - Ozen G AUID- ORCID: 0000-0002-5423-393 AD - University of Iowa Health Care, Iowa City, IA. FAU - Pedro, Sofia AU - Pedro S AD - FORWARD, The National Databank for Rheumatic Diseases, Wichita, KS. FAU - Domsic, Robyn T AU - Domsic RT AD - University of Pittsburgh, Pittsburgh, PA. FAU - Michaud, Kaleb AU - Michaud K LA - eng PT - Journal Article DEP - 20250515 PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Calcium Channel Blockers) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) SB - IM MH - Humans MH - *Raynaud Disease/drug therapy/etiology/epidemiology/diagnosis MH - *Scleroderma, Systemic/complications/drug therapy/epidemiology MH - Female MH - Male MH - United States/epidemiology MH - Middle Aged MH - Calcium Channel Blockers/therapeutic use MH - Adult MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - *Practice Patterns, Physicians'/statistics & numerical data MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Aged MH - Disease Management COIS- The authors declare no conflict of interest. EDAT- 2025/05/15 12:37 MHDA- 2025/09/29 22:46 CRDT- 2025/05/15 07:43 PHST- 2025/09/29 22:46 [medline] PHST- 2025/05/15 12:37 [pubmed] PHST- 2025/05/15 07:43 [entrez] AID - 00124743-990000000-00346 [pii] AID - 10.1097/RHU.0000000000002242 [doi] PST - ppublish SO - J Clin Rheumatol. 2025 Oct 1;31(7):279-283. doi: 10.1097/RHU.0000000000002242. Epub 2025 May 15. PMID- 16762173 OWN - NLM STAT- MEDLINE DCOM- 20060629 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 24 IP - 2 DP - 2006 Mar-Apr TI - Possible role of iloprost (stable analog of PG12) in promoting neoangiogenesis in systemic sclerosis. PG - 220-1 FAU - Faggioli, P AU - Faggioli P FAU - Giani, L AU - Giani L FAU - Mazzone, A AU - Mazzone A LA - eng PT - Letter PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) RN - JED5K35YGL (Iloprost) RN - R16CO5Y76E (Aspirin) SB - IM MH - Aspirin/therapeutic use MH - Capillaries/drug effects/physiology MH - Drug Therapy, Combination MH - Female MH - Humans MH - Iloprost/*therapeutic use MH - Infusions, Intravenous MH - Microscopic Angioscopy MH - Microscopy, Video MH - Middle Aged MH - Nails/blood supply/drug effects/pathology MH - Neovascularization, Physiologic/*drug effects MH - Nifedipine/therapeutic use MH - Raynaud Disease/*drug therapy/etiology MH - Scleroderma, Systemic/complications/*drug therapy MH - Vasodilator Agents/*therapeutic use EDAT- 2006/06/10 09:00 MHDA- 2006/06/30 09:00 CRDT- 2006/06/10 09:00 PHST- 2006/06/10 09:00 [pubmed] PHST- 2006/06/30 09:00 [medline] PHST- 2006/06/10 09:00 [entrez] AID - 1810 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2006 Mar-Apr;24(2):220-1. PMID- 30047427 OWN - NLM STAT- MEDLINE DCOM- 20181220 LR - 20181220 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 36 IP - 4 DP - 2018 Jul-Aug TI - Rheumatologic dermatology. PG - 439-441 LID - S0738-081X(18)30119-6 [pii] LID - 10.1016/j.clindermatol.2018.05.002 [doi] FAU - Kurtzman, Drew AU - Kurtzman D AD - Division of Dermatology, St. Elizabeth Physicians, University of Louisville School of Medicine, 7370 Turfway Road, Suite 370, Florence, KY 41042. Electronic address: drewkurtzman@gmail.com. LA - eng PT - Editorial PT - Introductory Journal Article PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Connective Tissue Diseases/complications/*diagnosis/drug therapy MH - Humans MH - Raynaud Disease/complications/diagnosis MH - Rheumatic Diseases/complications/*diagnosis/drug therapy/immunology MH - Sjogren's Syndrome/complications/diagnosis MH - Skin Diseases/complications/*diagnosis/*drug therapy/etiology MH - Vasculitis/complications/diagnosis EDAT- 2018/07/27 06:00 MHDA- 2018/12/21 06:00 CRDT- 2018/07/27 06:00 PHST- 2018/07/27 06:00 [entrez] PHST- 2018/07/27 06:00 [pubmed] PHST- 2018/12/21 06:00 [medline] AID - S0738-081X(18)30119-6 [pii] AID - 10.1016/j.clindermatol.2018.05.002 [doi] PST - ppublish SO - Clin Dermatol. 2018 Jul-Aug;36(4):439-441. doi: 10.1016/j.clindermatol.2018.05.002. PMID- 29582070 OWN - NLM STAT- MEDLINE DCOM- 20190816 LR - 20200716 IS - 1539-3704 (Electronic) IS - 0003-4819 (Print) IS - 0003-4819 (Linking) VI - 169 IP - 2 DP - 2018 Jul 17 TI - Genomic Analysis to Avoid Misdiagnosis of Adults With Bilateral Renal Cysts. PG - 130-131 LID - 10.7326/L17-0644 [doi] FAU - Gulati, Ashima AU - Gulati A AD - Yale University School of Medicine, New Haven, Connecticut (A.G., S.S.). FAU - Bae, Kyongtae T AU - Bae KT AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania (K.T.B.). FAU - Somlo, Stefan AU - Somlo S AD - Yale University School of Medicine, New Haven, Connecticut (A.G., S.S.). FAU - Watnick, Terry AU - Watnick T AD - University of Maryland School of Medicine, Baltimore, Maryland (T.W.). LA - eng GR - P30 DK090868/DK/NIDDK NIH HHS/United States GR - P30 DK079310/DK/NIDDK NIH HHS/United States GR - S10 OD018521/OD/NIH HHS/United States GR - U54 HG006504/HG/NHGRI NIH HHS/United States GR - R01 DK100592/DK/NIDDK NIH HHS/United States GR - R01 DK095036/DK/NIDDK NIH HHS/United States PT - Case Reports PT - Letter PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20180327 PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps MH - Aged MH - Diagnosis, Differential MH - *Genetic Testing/methods MH - Humans MH - Kidney Diseases, Cystic/diagnosis/diagnostic imaging/*genetics MH - Magnetic Resonance Imaging MH - Male MH - Muscle Cramp/diagnosis/diagnostic imaging/genetics MH - Polycystic Kidney, Autosomal Dominant/diagnosis/diagnostic imaging/genetics MH - Raynaud Disease/diagnosis/diagnostic imaging/genetics PMC - PMC7196958 MID - NIHMS1579048 COIS- Conflict of interest: None EDAT- 2018/03/28 06:00 MHDA- 2019/08/17 06:00 PMCR- 2020/05/04 CRDT- 2018/03/28 06:00 PHST- 2018/03/28 06:00 [pubmed] PHST- 2019/08/17 06:00 [medline] PHST- 2018/03/28 06:00 [entrez] PHST- 2020/05/04 00:00 [pmc-release] AID - 2676949 [pii] AID - 10.7326/L17-0644 [doi] PST - ppublish SO - Ann Intern Med. 2018 Jul 17;169(2):130-131. doi: 10.7326/L17-0644. Epub 2018 Mar 27. PMID- 18474662 OWN - NLM STAT- MEDLINE DCOM- 20080620 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 67 IP - 6 DP - 2008 Jun TI - Tako-tsubo-like syndrome in systemic sclerosis: a sign of myocardial Raynaud phenomenon? PG - 898-9 LID - 10.1136/ard.2007.069500 [doi] FAU - Melchiorre, D AU - Melchiorre D FAU - Bernardo, P AU - Bernardo P FAU - Conforti, M L AU - Conforti ML FAU - Comunian, C AU - Comunian C FAU - Nacci, F AU - Nacci F FAU - Guiducci, S AU - Guiducci S FAU - Fiori, G AU - Fiori G FAU - Moggi-Pignone, A AU - Moggi-Pignone A FAU - Gensini, G F AU - Gensini GF FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M LA - eng PT - Case Reports PT - Letter PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Aged, 80 and over MH - Coronary Circulation MH - Female MH - Humans MH - Myocardial Ischemia/*complications MH - Raynaud Disease/*complications MH - Scleroderma, Systemic/*complications MH - Takotsubo Cardiomyopathy/*etiology EDAT- 2008/05/14 09:00 MHDA- 2008/06/21 09:00 CRDT- 2008/05/14 09:00 PHST- 2008/05/14 09:00 [pubmed] PHST- 2008/06/21 09:00 [medline] PHST- 2008/05/14 09:00 [entrez] AID - S0003-4967(24)43731-X [pii] AID - 10.1136/ard.2007.069500 [doi] PST - ppublish SO - Ann Rheum Dis. 2008 Jun;67(6):898-9. doi: 10.1136/ard.2007.069500. PMID- 35544320 OWN - NLM STAT- MEDLINE DCOM- 20220701 LR - 20230907 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 150 IP - 1 DP - 2022 Jul 1 TI - Scleroderma and Raynaud Phenomenon: The Cold Truth Regarding the Use of Operative Management. PG - 105e-114e LID - 10.1097/PRS.0000000000009187 [doi] AB - BACKGROUND: Raynaud phenomenon, with and without scleroderma, is a common vasospastic condition that manifests with extremity pain and skin discoloration. When conservative management fails, complications such as ischemia, ulceration, and gangrene may warrant surgical intervention. The purpose of this study was to determine the risk factors and use of surgical intervention in this population. METHODS: A national insurance claims-based database with patient records from the Centers for Medicare and Medicaid Services was used for data collection. Patients with first diagnoses of Raynaud phenomenon, scleroderma, or both between 2005 and 2014 were identified. Primary outcomes included the presence of upper extremity amputation or vascular procedure, and history of amputation within 5 years of a vascular procedure. Secondary outcomes included hospital admissions, upper extremity wounds, and amputation within 1 year of diagnosis. RESULTS: The Raynaud phenomenon, scleroderma, and Raynaud phenomenon with scleroderma cohorts consisted of 161,300, 117,564, and 25,096 patients, respectively. A diagnosis of both Raynaud phenomenon and scleroderma increased the odds of upper extremity amputation by 5.4-fold, vascular procedure by 4.8-fold, and amputation within 5 years of a vascular procedure by 1.5-fold. Patients with Raynaud phenomenon or scleroderma alone were 3.1 and 5.6 times less likely to undergo amputation within 5 years of a vascular procedure, respectively. CONCLUSIONS: Patients with both Raynaud phenomenon and scleroderma have higher likelihoods of having upper extremity amputations, vascular procedures, and amputations following vascular procedures compared to each diagnosis alone. Vascular procedures are rarely being performed. Further research is necessary to establish a standard of care and determine whether early and more frequent intervention with vascular procedures can decrease amputation rates in this patient population. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II. CI - Copyright © 2022 by the American Society of Plastic Surgeons. FAU - Hakami, Lee M AU - Hakami LM AD - From the Department of Plastic Surgery, the School of Medicine, and the Claude Moore Health Sciences Library, University of Virginia. FAU - Forster, Grace L AU - Forster GL AD - From the Department of Plastic Surgery, the School of Medicine, and the Claude Moore Health Sciences Library, University of Virginia. FAU - Jones, Marieke K AU - Jones MK AD - From the Department of Plastic Surgery, the School of Medicine, and the Claude Moore Health Sciences Library, University of Virginia. FAU - DeGeorge, Brent R Jr AU - DeGeorge BR Jr AD - From the Department of Plastic Surgery, the School of Medicine, and the Claude Moore Health Sciences Library, University of Virginia. LA - eng PT - Journal Article DEP - 20220510 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CIN - Plast Reconstr Surg. 2023 Aug 1;152(2):369e-370e. doi: 10.1097/PRS.0000000000010423. PMID: 37163536 MH - Aged MH - Amputation, Surgical/adverse effects MH - Humans MH - Ischemia/etiology MH - Medicare MH - *Raynaud Disease/complications/surgery MH - Retrospective Studies MH - Risk Factors MH - *Scleroderma, Localized MH - Treatment Outcome MH - United States EDAT- 2022/05/12 06:00 MHDA- 2022/07/02 06:00 CRDT- 2022/05/11 11:54 PHST- 2022/05/12 06:00 [pubmed] PHST- 2022/07/02 06:00 [medline] PHST- 2022/05/11 11:54 [entrez] AID - 00006534-202207000-00023 [pii] AID - 10.1097/PRS.0000000000009187 [doi] PST - ppublish SO - Plast Reconstr Surg. 2022 Jul 1;150(1):105e-114e. doi: 10.1097/PRS.0000000000009187. Epub 2022 May 10. PMID- 19920419 OWN - NLM STAT- MEDLINE DCOM- 20100127 LR - 20190109 IS - 1532-8651 (Electronic) IS - 1098-7339 (Linking) VI - 34 IP - 5 DP - 2009 Sep-Oct TI - Percutaneous upper thoracic radiofrequency sympathectomy in Raynaud phenomenon: a comparison of T2/T3 procedure versus T2 lesion with phenol application. PG - 425-9 LID - 10.1097/AAP.0b013e3181b48f9a [doi] AB - BACKGROUND AND OBJECTIVES: Percutaneous radiofrequency (RF) thoracic sympathectomy is an alternative method to surgical procedures for the treatment of acral ischemia in Raynaud phenomenon. The procedure is indicated if conservative therapy fails to provide sufficient relief. The aim of this study was to compare classic T2 and T3 RF thermolesioning with a less invasive procedure at the level of T2 only. METHODS: Fifty adult patients, American Society of Anesthesiologists (ASA) classification I to III, were randomly assigned to 1 of 2 groups. T2 and T3 thoracic RF thermolesion was performed in 1 group, whereas T2 thermolesion with local application of 0.5 mL of 6% phenol was delivered in the second group. Changes in cold perception, pain, and quality of life were assessed using a questionnaire. Blood circulation in the upper extremity was evaluated using infrared thermography. Patients were observed for a period of 3 months. RESULTS: A significant decrease in pain according to visual analog scale (P < 0.001), increase in peripheral temperature in the upper extremities (P < 0.001), and improvement in quality of life were observed in both groups of patients after the procedure. Susceptibility to cold-provoked vasospasm was not significantly affected in either group. There was no significant difference between the 2 groups in any parameter apart from the duration of the procedure. CONCLUSIONS: Thoracic RF upper sympathectomy is an effective method in the treatment of resistant forms of Raynaud phenomenon. A single-shot procedure at the level of T2 may be preferable because of the shorter procedure duration of this technique. FAU - Gabrhelik, Tomas AU - Gabrhelik T AD - Department of Anaesthesia and Intensive Care, University Hospital, Olomouc, Czech Republic. gabrhelikt@yahoo.co.uk FAU - Michalek, Pavel AU - Michalek P FAU - Adamus, Milan AU - Adamus M FAU - Berta, Emil AU - Berta E LA - eng PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Reg Anesth Pain Med JT - Regional anesthesia and pain medicine JID - 9804508 RN - 339NCG44TV (Phenol) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Combined Modality Therapy MH - *Electrocoagulation/adverse effects MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain/etiology/prevention & control MH - Pain Measurement MH - Pain Threshold MH - Patient Satisfaction MH - Phenol/*administration & dosage MH - Prospective Studies MH - Quality of Life MH - Raynaud Disease/complications/physiopathology/*surgery MH - Regional Blood Flow MH - Surveys and Questionnaires MH - Sympathectomy/adverse effects/*methods MH - Thermosensing MH - Thoracic Vertebrae/*innervation MH - Time Factors MH - Treatment Outcome MH - Young Adult EDAT- 2009/11/19 06:00 MHDA- 2010/01/28 06:00 CRDT- 2009/11/19 06:00 PHST- 2009/11/19 06:00 [entrez] PHST- 2009/11/19 06:00 [pubmed] PHST- 2010/01/28 06:00 [medline] AID - 00115550-200909000-00010 [pii] AID - 10.1097/AAP.0b013e3181b48f9a [doi] PST - ppublish SO - Reg Anesth Pain Med. 2009 Sep-Oct;34(5):425-9. doi: 10.1097/AAP.0b013e3181b48f9a. PMID- 25847811 OWN - NLM STAT- MEDLINE DCOM- 20150928 LR - 20181113 IS - 2325-4416 (Electronic) IS - 2325-4394 (Print) IS - 2325-4394 (Linking) VI - 21 DP - 2015 Apr 2 TI - How can follow-up of patients with Raynaud phenomenon be optimized? PG - 47-52 LID - 10.12659/MSMBR.893998 [doi] AB - BACKGROUND Raynaud phenomenon (RP) is common worldwide and presents diagnostic and therapeutic difficulties. We aimed to share our experience with optimizing of patient follow-up by using the cold-stimulation test (CST). MATERIAL AND METHODS Data of 81 patients admitted with RP symptomatology were collected. Demographic data and symptoms were recorded. A scale was used for determining the severity of disease at pre-treatment and post-treatment. CST was performed to all patients at pre-treatment and post-treatment for assessment of treatment efficiency in follow-up. Results were analyzed with the SPSS for Mac 20.0 program. RESULTS All the patients were male. Mean age was 22.3 ± 2.14 (19-29). Mean duration of symptoms from onset to present was 4.59 ± 2.85 years. There were statistically significant differences between pre-treatment and post-treatment hand temperatures measured by CST (p<0.001). However, there were no statistically significant differences between pre-treatment and post-treatment severity scores of patients (p=0.135). CONCLUSIONS To quantitatively determine the treatment efficacy, CST may be used instead of asking simple questions of patients. FAU - Kadan, Murat AU - Kadan M AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Erol, Gökhan AU - Erol G AD - Department of Cardiovascular Surgery, Maresal Cakmak Military Hospital, Erzurum, Turkey. FAU - Karabacak, Kubilay AU - Karabacak K AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Kaya, Erkan AU - Kaya E AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Arslan, Gökhan AU - Arslan G AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Doğancı, Suat AU - Doğancı S AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. FAU - Demirkılıç, Ufuk AU - Demirkılıç U AD - Department of Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey. LA - eng PT - Journal Article DEP - 20150402 PL - United States TA - Med Sci Monit Basic Res JT - Medical science monitor basic research JID - 101597444 SB - IM MH - Adult MH - Cold Temperature MH - Follow-Up Studies MH - Humans MH - Male MH - Physical Stimulation/methods MH - Raynaud Disease/*diagnosis/*therapy MH - Retrospective Studies MH - Rewarming/*methods MH - Statistics, Nonparametric MH - Time Factors MH - Treatment Outcome PMC - PMC4395022 EDAT- 2015/04/08 06:00 MHDA- 2015/09/29 06:00 PMCR- 2015/04/02 CRDT- 2015/04/08 06:00 PHST- 2015/04/08 06:00 [entrez] PHST- 2015/04/08 06:00 [pubmed] PHST- 2015/09/29 06:00 [medline] PHST- 2015/04/02 00:00 [pmc-release] AID - 893998 [pii] AID - 10.12659/MSMBR.893998 [doi] PST - epublish SO - Med Sci Monit Basic Res. 2015 Apr 2;21:47-52. doi: 10.12659/MSMBR.893998. PMID- 25052356 OWN - NLM STAT- MEDLINE DCOM- 20150413 LR - 20181202 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 158 DP - 2014 TI - [Systemic sclerosis: a multisystem disease]. PG - A7703 AB - Systemic sclerosis is a rare, systemic autoimmune disease, characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. The disease is associated with a significantly increased morbidity and mortality, and can be rapidly progressive. Interstitial lung disease, renal hypertensive crisis, cardiac involvement and pulmonary arterial hypertension are life-threatening complications. Early treatment with immunosuppressive drugs can prevent progression and decrease morbidity and mortality. FAU - Berrevoets, Marvin A H AU - Berrevoets MA AD - Radboudumc, afd. Reumatische ziekten, Nijmegen. FAU - Markhorst, Joekie AU - Markhorst J FAU - Meek, Inger L AU - Meek I FAU - van Ede, Annelies E AU - van Ede AE FAU - Vonk, Madelon C AU - Vonk MC LA - dut PT - Case Reports PT - Journal Article PT - Review TT - Systemische sclerose: multisysteemaandoening. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 SB - IM MH - Adult MH - Aged MH - Disease Progression MH - Female MH - Gastrointestinal Diseases/epidemiology/etiology MH - Humans MH - Hypertension/epidemiology/etiology MH - Joint Diseases/epidemiology/etiology MH - Male MH - Middle Aged MH - Muscle Weakness/epidemiology/etiology MH - Raynaud Disease/epidemiology/*etiology MH - Scleroderma, Systemic/*complications EDAT- 2014/07/24 06:00 MHDA- 2015/04/14 06:00 CRDT- 2014/07/24 06:00 PHST- 2014/07/24 06:00 [entrez] PHST- 2014/07/24 06:00 [pubmed] PHST- 2015/04/14 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2014;158:A7703. PMID- 42101599 OWN - NLM STAT- MEDLINE DCOM- 20260508 LR - 20260508 IS - 1532-0650 (Electronic) IS - 0002-838X (Linking) VI - 113 IP - 4 DP - 2026 Apr TI - Systemic Sclerosis: Evaluation and Treatment. PG - 349-357 AB - Systemic sclerosis is a rare autoimmune connective tissue disease characterized by progressive fibrosis of the skin and internal organs, vasculopathy, and the presence of specific autoantibodies. Despite its low prevalence, systemic sclerosis is associated with high morbidity. Early features often include Raynaud phenomenon, hand edema, and fatigue. Diagnosis requires a comprehensive approach, including clinical assessment, laboratory evaluation, imaging, and pulmonary function testing. The American College of Rheumatology and European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism) provide classification criteria and updated treatment recommendations. Management focuses on addressing eight disease domains: Raynaud phenomenon, digital ulcers, pulmonary artery hypertension, interstitial lung disease, renal crisis, gastrointestinal involvement, skin fibrosis, and musculoskeletal involvement. Vasodilator therapy is first-line treatment for Raynaud phenomenon, whereas phosphodiesterase-5 inhibitors and intravenous iloprost are used to treat digital ulcers. Combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists is first-line treatment for pulmonary artery hypertension. Mycophenolate mofetil is the preferred treatment for interstitial lung disease. FAU - Frazier, Winfred MD, MPH AU - Frazier W MD, MPH AD - Department of Family Medicine at the University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Goettel, Courtney MD, MS AU - Goettel C MD, MS AD - Williamsport Family Medicine Residency Program at the University of Pittsburgh Medical Center, Williamsport, Pennsylvania. FAU - Chaudhri, Parul DO AU - Chaudhri P DO AD - University of Toledo Family Medicine Residency Program, Toledo, Ohio. LA - eng PT - Journal Article PT - Review PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/therapy/drug therapy/physiopathology/complications MH - Raynaud Disease/etiology/drug therapy MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2026/05/08 12:37 MHDA- 2026/05/08 12:38 CRDT- 2026/05/08 11:10 PHST- 2026/05/08 12:38 [medline] PHST- 2026/05/08 12:37 [pubmed] PHST- 2026/05/08 11:10 [entrez] AID - 642e10d9-97b5-4a42-a889-10e730b7471d [pii] PST - ppublish SO - Am Fam Physician. 2026 Apr;113(4):349-357. PMID- 17019756 OWN - NLM STAT- MEDLINE DCOM- 20061027 LR - 20061003 IS - 0033-7021 (Print) IS - 0033-7021 (Linking) VI - 69 IP - 9 DP - 2006 Sep TI - What can anticonvulsants do for chronic pain? PG - 49 FAU - Nowlin, Anne AU - Nowlin A LA - eng PT - Journal Article PL - United States TA - RN JT - RN JID - 20010080R RN - 0 (Anticonvulsants) MH - Anticonvulsants/pharmacology/*therapeutic use MH - Chronic Disease MH - Humans MH - Neuralgia/*drug therapy/etiology MH - Raynaud Disease/*complications EDAT- 2006/10/05 09:00 MHDA- 2006/10/28 09:00 CRDT- 2006/10/05 09:00 PHST- 2006/10/05 09:00 [pubmed] PHST- 2006/10/28 09:00 [medline] PHST- 2006/10/05 09:00 [entrez] PST - ppublish SO - RN. 2006 Sep;69(9):49. PMID- 33930611 OWN - NLM STAT- MEDLINE DCOM- 20220401 LR - 20220401 IS - 2468-1210 (Electronic) IS - 2468-1210 (Linking) VI - 40 IP - 5 DP - 2021 Oct TI - Digital ischemia under WALANT phentolamine reversed for patient with Raynaud phenomenon. PG - 692-694 LID - S2468-1229(21)00118-3 [pii] LID - 10.1016/j.hansur.2021.04.006 [doi] FAU - Gueffier, X AU - Gueffier X AD - Artezieux Center, 40, Avenue des alpes, 38300 Bourgoin Jallieu, France. Electronic address: xavier.gueffier@icloud.com. FAU - Gueffier, E AU - Gueffier E AD - Université Picardie Jules Verne, 1-3 rue des louvels, 80000 Amiens, France. Electronic address: elisegueffier@icloud.com. FAU - Yalom, A AU - Yalom A AD - Oasis MD, 8901 Activity Road, San Diego, CA 92126, USA. Electronic address: ayalom@oasismd.com. LA - eng PT - Letter DEP - 20210427 PL - France TA - Hand Surg Rehabil JT - Hand surgery & rehabilitation JID - 101681801 RN - Z468598HBV (Phentolamine) SB - IM MH - Fingers MH - Humans MH - Ischemia/drug therapy MH - Phentolamine MH - *Raynaud Disease/drug therapy EDAT- 2021/05/01 06:00 MHDA- 2022/04/02 06:00 CRDT- 2021/04/30 20:23 PHST- 2020/11/27 00:00 [received] PHST- 2021/03/04 00:00 [revised] PHST- 2021/04/17 00:00 [accepted] PHST- 2021/05/01 06:00 [pubmed] PHST- 2022/04/02 06:00 [medline] PHST- 2021/04/30 20:23 [entrez] AID - S2468-1229(21)00118-3 [pii] AID - 10.1016/j.hansur.2021.04.006 [doi] PST - ppublish SO - Hand Surg Rehabil. 2021 Oct;40(5):692-694. doi: 10.1016/j.hansur.2021.04.006. Epub 2021 Apr 27. PMID- 25539338 OWN - NLM STAT- MEDLINE DCOM- 20150309 LR - 20210106 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 135 IP - 1 DP - 2015 Jan TI - Reply: fat grafting to the hand in patients with raynaud phenomenon: a novel therapeutic modality. PG - 230e-231e LID - 10.1097/PRS.0000000000000789 [doi] FAU - Bank, Jonathan AU - Bank J AD - Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago Medical Center, Chicago, Ill. FAU - Zachary, Lawrence S AU - Zachary LS LA - eng PT - Comment PT - Letter PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CON - Plast Reconstr Surg. 2014 May;133(5):1109-1118. doi: 10.1097/PRS.0000000000000104. PMID: 24445877 CON - Plast Reconstr Surg. 2015 Jan;135(1):229e-230e. doi: 10.1097/PRS.0000000000000814. PMID: 25539337 MH - Adipose Tissue/*transplantation MH - Female MH - Hand/*blood supply/*surgery MH - Humans MH - Ischemia/*surgery MH - Male MH - Raynaud Disease/*surgery EDAT- 2014/12/30 06:00 MHDA- 2015/03/10 06:00 CRDT- 2014/12/25 06:00 PHST- 2014/12/25 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2015/03/10 06:00 [medline] AID - 00006534-201501000-00065 [pii] AID - 10.1097/PRS.0000000000000789 [doi] PST - ppublish SO - Plast Reconstr Surg. 2015 Jan;135(1):230e-231e. doi: 10.1097/PRS.0000000000000789. PMID- 17532934 OWN - NLM STAT- MEDLINE DCOM- 20070627 LR - 20220410 IS - 1097-685X (Electronic) IS - 0022-5223 (Linking) VI - 133 IP - 6 DP - 2007 Jun TI - Long-term effects of thoracic sympathectomy on microcirculation in the hands of patients with primary Raynaud disease. PG - 1428-33 AB - OBJECTIVE: Videothoracoscopic sympathecomy is a widely used treatment modality in patients with severe Raynaud disease, but the reported late results are less than favorable. There have been no direct studies of the long-term effect of sympathectomy on microcirculation in the hands of these patients. METHODS: In 25 patients with Raynaud disease treated with videothoracoscopic Th2-Th4 sympathectomy, we performed basal laser-Doppler flowmetry and measured the maximal refilling time after 1-minute occlusion measurements preoperatively and at 1 week, 6 months, and 1, 2, 3, and 5 years after the sympathectomy. The results were compared with the same measurements obtained in the group of 50 healthy individuals. RESULTS: The patients' symptom severity was assessed by using the visual analogue scale. The basal capillary flow and the maximal refilling time improved after the sympathectomy to a level not different from that seen in the healthy population, and the effect was maintained during the 5-year follow-up period. The patients' symptom severity scores diminished to zero in the early postoperative period and increased to 28% of their initial value 5 years after the operation. CONCLUSIONS: The videothoracoscopic Th2-Th4 sympathectomy produces excellent and long-lasting improvement of microcirculation function in patients with Raynaud disease. The mild return of symptoms might be due to factors other than the capillary blood flow alterations. FAU - Maga, Paweł AU - Maga P AD - Department of Medicine, Jagiellonian University, Kraków, Poland. p.maga@chello.pl FAU - Kuzdzał, Jarosław AU - Kuzdzał J FAU - Nizankowski, Rafał AU - Nizankowski R FAU - Szczeklik, Andrzej AU - Szczeklik A FAU - Sładek, Krzysztof AU - Sładek K LA - eng PT - Journal Article PL - United States TA - J Thorac Cardiovasc Surg JT - The Journal of thoracic and cardiovascular surgery JID - 0376343 SB - IM MH - Adolescent MH - Adult MH - Analysis of Variance MH - Blood Flow Velocity MH - Case-Control Studies MH - Female MH - Hand/*blood supply/*innervation MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/*surgery MH - Sympathectomy/*methods MH - Thoracic Surgery, Video-Assisted MH - Thoracoscopy MH - Treatment Outcome EDAT- 2007/05/30 09:00 MHDA- 2007/06/28 09:00 CRDT- 2007/05/30 09:00 PHST- 2006/10/03 00:00 [received] PHST- 2006/12/04 00:00 [revised] PHST- 2006/12/12 00:00 [accepted] PHST- 2007/05/30 09:00 [pubmed] PHST- 2007/06/28 09:00 [medline] PHST- 2007/05/30 09:00 [entrez] AID - S0022-5223(07)00247-4 [pii] AID - 10.1016/j.jtcvs.2006.12.055 [doi] PST - ppublish SO - J Thorac Cardiovasc Surg. 2007 Jun;133(6):1428-33. doi: 10.1016/j.jtcvs.2006.12.055. PMID- 34288866 OWN - NLM STAT- MEDLINE DCOM- 20210924 LR - 20210924 IS - 2326-6929 (Electronic) IS - 0011-4162 (Linking) VI - 107 IP - 5 DP - 2021 May TI - Phacomatosis Pigmentokeratotica Associated With Raynaud Phenomenon, Segmental Nevi, Hyperhidrosis, and Scoliosis. PG - E24-E26 LID - 10.12788/cutis.0279 [doi] FAU - Ren, Faliang AU - Ren F AD - Drs. Ren and Tan are from the Department of Dermatology, Children's Hospital of Chongqing Medical University, China. Drs. Pruitt and Elston are from the Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston. FAU - Pruitt, Laura AU - Pruitt L AD - Drs. Ren and Tan are from the Department of Dermatology, Children's Hospital of Chongqing Medical University, China. Drs. Pruitt and Elston are from the Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston. FAU - Tan, Qi AU - Tan Q AD - Drs. Ren and Tan are from the Department of Dermatology, Children's Hospital of Chongqing Medical University, China. Drs. Pruitt and Elston are from the Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston. FAU - Elston, Dirk M AU - Elston DM AD - Drs. Ren and Tan are from the Department of Dermatology, Children's Hospital of Chongqing Medical University, China. Drs. Pruitt and Elston are from the Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston. LA - eng PT - Letter PL - United States TA - Cutis JT - Cutis JID - 0006440 RN - Phacomatosis pigmentokeratotica SB - IM MH - Humans MH - *Hyperhidrosis MH - *Nevus MH - Nevus, Pigmented MH - *Raynaud Disease/diagnosis MH - *Scoliosis/complications/diagnosis MH - *Skin Neoplasms/diagnosis EDAT- 2021/07/22 06:00 MHDA- 2021/09/25 06:00 CRDT- 2021/07/21 17:21 PHST- 2021/07/21 17:21 [entrez] PHST- 2021/07/22 06:00 [pubmed] PHST- 2021/09/25 06:00 [medline] AID - cutis.0279 [pii] AID - 10.12788/cutis.0279 [doi] PST - ppublish SO - Cutis. 2021 May;107(5):E24-E26. doi: 10.12788/cutis.0279. PMID- 27789028 OWN - NLM STAT- MEDLINE DCOM- 20190111 LR - 20190111 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 39 IP - 6 DP - 2018 Jun TI - [Abnormal nails]. PG - 443-444 LID - S0248-8663(16)30540-9 [pii] LID - 10.1016/j.revmed.2016.09.003 [doi] FAU - Nassarmadji, K AU - Nassarmadji K AD - Département de médecine interne, CHU de Rouen, 76031 Rouen cedex, France. FAU - Marie, I AU - Marie I AD - Département de médecine interne, CHU de Rouen, 76031 Rouen cedex, France. Electronic address: isabelle.marie@chu-rouen.fr. LA - fre PT - Case Reports PT - Journal Article TT - Des anomalies des ongles. DEP - 20161024 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Arthritis/drug therapy/etiology MH - Diagnosis, Differential MH - Female MH - Humans MH - Hydroxychloroquine/*adverse effects/therapeutic use MH - Middle Aged MH - Mixed Connective Tissue Disease/complications/*drug therapy MH - Nail Diseases/*chemically induced/diagnosis MH - Nails, Malformed/*chemically induced/*diagnosis/etiology MH - Raynaud Disease/drug therapy/etiology OTO - NOTNLM OT - Adverse effect OT - Antimalarial drugs OT - Antipaludéens de synthèse OT - Atteinte cutanée OT - Effet indésirable OT - Hydroxychloroquine OT - Melanonychia OT - Mélanonychie OT - Skin involvement EDAT- 2016/10/30 06:00 MHDA- 2019/01/12 06:00 CRDT- 2016/10/30 06:00 PHST- 2016/08/04 00:00 [received] PHST- 2016/09/16 00:00 [accepted] PHST- 2016/10/30 06:00 [pubmed] PHST- 2019/01/12 06:00 [medline] PHST- 2016/10/30 06:00 [entrez] AID - S0248-8663(16)30540-9 [pii] AID - 10.1016/j.revmed.2016.09.003 [doi] PST - ppublish SO - Rev Med Interne. 2018 Jun;39(6):443-444. doi: 10.1016/j.revmed.2016.09.003. Epub 2016 Oct 24. PMID- 17516368 OWN - NLM STAT- MEDLINE DCOM- 20070718 LR - 20091111 IS - 0023-2165 (Print) IS - 0023-2165 (Linking) VI - 224 IP - 5 DP - 2007 May TI - [Retrobulbar haemodynamics in normal and high tension glaucoma patients: the diagnostic importance of tinnitus, migraine and Raynaud-like symptoms]. PG - 396-400 AB - BACKGROUND: In the pathophysiology of open-angle glaucoma altered perfusion of the optic nerve head is of importance. Up to now these disturbances were presumed to be the chief cause of glaucomatous damage in patients with normal tension glaucoma showing other vascular disturbances like migraine or tinnitus. PATIENTS AND METHODS: Peak systolic velocity (PSV) and end-diastolic velocity (EDV) were measured and the resistive index (RI) was calculated by colour Doppler imaging (CDI) in the ophthalmic artery (OA), central retinal artery (CRA) and in the short and long posterior ciliary arteries (SPCA, LPCA) in 18 patients with normal tension glaucoma (NTG), in 18 patients with high tension glaucoma (HTG) and in 18 normal control subjects. RESULTS: In an upright sitting position both glaucoma groups showed statistically significant decreases in PSV and EDV in CRA and SPCA compared to the control subjects. HTG when compared to NTG and normal subjects showed statistically significant decreases of EDV and statistically significant increases of RI in LPCA. In addition, compared to normal subjects, HTG patients showed statistically significant increases of RI in both OA and SPCA. DISCUSSION: Both glaucoma groups showed decreased blood flow velocities in the small retrobulbar vessels in an upright sitting position. Normal tension glaucoma patients with symptoms of vasospasms compared to patients with high tension glaucoma showed only small differences in ocular haemodynamics. FAU - Wiermann, A AU - Wiermann A AD - Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Augenheilkunde, Hamburg. a.wiermann@uke.uni-hamburg.de FAU - Galambos, P AU - Galambos P FAU - Vafiadis, J AU - Vafiadis J FAU - Wagenfeld, L AU - Wagenfeld L FAU - Richard, G AU - Richard G FAU - Klemm, M AU - Klemm M FAU - Zeitz, O AU - Zeitz O LA - ger PT - English Abstract PT - Journal Article TT - Retrobulbäre Hämodynamik bei Hochdruck- und Normaldruckglaukompatienten: Diagnostische Bedeutung von Tinnitus, Migräne und raynaudartigen Beschwerden. PL - Germany TA - Klin Monbl Augenheilkd JT - Klinische Monatsblatter fur Augenheilkunde JID - 0014133 SB - IM MH - *Blood Flow Velocity MH - Female MH - Glaucoma/complications/*diagnosis/*physiopathology MH - Humans MH - Intraocular Pressure MH - Male MH - Middle Aged MH - Migraine Disorders/complications/diagnosis/*physiopathology MH - Optic Disk/blood supply/*physiopathology MH - Raynaud Disease/complications/diagnosis/*physiopathology MH - Reference Values MH - Tinnitus/complications/diagnosis/*physiopathology EDAT- 2007/05/23 09:00 MHDA- 2007/07/19 09:00 CRDT- 2007/05/23 09:00 PHST- 2007/05/23 09:00 [pubmed] PHST- 2007/07/19 09:00 [medline] PHST- 2007/05/23 09:00 [entrez] AID - 10.1055/s-2007-963118 [doi] PST - ppublish SO - Klin Monbl Augenheilkd. 2007 May;224(5):396-400. doi: 10.1055/s-2007-963118. PMID- 21432794 OWN - NLM STAT- MEDLINE DCOM- 20110909 LR - 20110324 IS - 1897-4279 (Electronic) IS - 0022-9032 (Linking) VI - 69 IP - 3 DP - 2011 TI - [Consequences of Raynaud phenomenon in systemic sclerosis]. PG - 250 FAU - Ciszewski, Andrzej AU - Ciszewski A AD - Samodzielna Pracownia Hemodynamiki, I Klinika Choroby Wieńcowej, Instytut Kardiologii, Warszawa. LA - pol PT - Comment PT - Journal Article TT - Następstwa sercowego zjawiska Raynaud w twardzinie układowej. PL - Poland TA - Kardiol Pol JT - Kardiologia polska JID - 0376352 SB - IM CON - Kardiol Pol. 2011;69(3):243-9. PMID: 21432793 MH - *Heart Diseases MH - Humans MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2011/03/25 06:00 MHDA- 2011/09/10 06:00 CRDT- 2011/03/25 06:00 PHST- 2011/03/25 06:00 [entrez] PHST- 2011/03/25 06:00 [pubmed] PHST- 2011/09/10 06:00 [medline] PST - ppublish SO - Kardiol Pol. 2011;69(3):250. PMID- 35925129 OWN - NLM STAT- MEDLINE DCOM- 20220808 LR - 20250728 IS - 2731-7099 (Electronic) IS - 2731-7080 (Linking) VI - 63 IP - 6 DP - 2022 Jun TI - [Functional vascular acrosyndromes]. PG - 591-600 LID - 10.1007/s00108-022-01340-w [doi] AB - Vascular acrosyndromes are characterized by sparse, uniform clinical manifestations and a variety of possible pathomechanisms. The present article focuses on the functional entities. Raynaud phenomenon is based on cold- or stress-induced vasospasms of acral arteries. It is defined by the color changes of the skin, in the typical case white-blue-red (tricolore). The long fingers are most commonly affected. The etiology is unknown, and the pathophysiology is poorly understood. A distinction is made between primary and a secondary Raynaud phenomenon. The most important underlying diseases include collagenosis, primarily systemic sclerosis, and malignancies; furthermore, medications and drugs may promote vasospasm. Treatment is aimed at preventing or breaking the vasospasm, but has been only partially effective in doing so. Acrocyanosis is a vasospastic dystonic acral disorder that results in permanent reddish-livid discoloration, especially of the hands and feet. Secondary forms occur in collagenosis, malignancies, and myelodysplastic syndromes. The etiology and pathophysiology are virtually unknown. Targeted pharmacological intervention is not possible. Unlike all other vascular acrosyndromes, erythromelalgia is characterized by hyperemia. The primary form is a genetic sodium channelopathy, while secondary forms include malignancies, connective tissue diseases, and myelodysplastic syndromes. The symptoms are often distressing and disabling. Therapy requires a multimodal approach that includes both nonpharmacological and pharmacological strategies. Close interdisciplinary collaboration is essential for the management of this disease. CI - © 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature. FAU - Klein-Weigel, Peter AU - Klein-Weigel P AD - Klinik für Angiologie, Klinikum Ernst von Bergmann, Charlottenstr. 72, 14467, Potsdam, Deutschland. peter.klein-weigel@klinikumevb.de. FAU - Ruttloff, Andreas AU - Ruttloff A AD - Klinik für Angiologie, Klinikum Ernst von Bergmann, Charlottenstr. 72, 14467, Potsdam, Deutschland. FAU - König, Dana AU - König D AD - Klinik für Angiologie, Klinikum Ernst von Bergmann, Charlottenstr. 72, 14467, Potsdam, Deutschland. FAU - Nielitz, Jessica AU - Nielitz J AD - Klinik für Angiologie, Klinikum Ernst von Bergmann, Charlottenstr. 72, 14467, Potsdam, Deutschland. FAU - Steindl, Julia AU - Steindl J AD - Klinik für Angiologie, Klinikum Ernst von Bergmann, Charlottenstr. 72, 14467, Potsdam, Deutschland. FAU - Sander, Oliver AU - Sander O AD - Poliklinik und Funktionsbereich für Rheumatologie & Hiller-Forschungszentrum für Rheumatologie, Universitätsklinikum Düsseldorf, Medizinische Fakultät, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Deutschland. FAU - Richter, Jutta G AU - Richter JG AD - Poliklinik und Funktionsbereich für Rheumatologie & Hiller-Forschungszentrum für Rheumatologie, Universitätsklinikum Düsseldorf, Medizinische Fakultät, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Deutschland. LA - ger PT - Journal Article PT - Review TT - Funktionelle akrale Durchblutungsstörungen. DEP - 20220516 PL - Germany TA - Inn Med (Heidelb) JT - Innere Medizin (Heidelberg, Germany) JID - 9918384885306676 SB - IM MH - Cyanosis/complications MH - *Erythromelalgia/complications MH - Humans MH - *Myelodysplastic Syndromes/complications MH - *Raynaud Disease/diagnosis MH - *Vascular Diseases/complications OTO - NOTNLM OT - Acral cyanosis OT - Circulation disorders, acral OT - Erythromelalgia OT - Raynaud disease OT - Vasospasm EDAT- 2022/08/05 06:00 MHDA- 2022/08/09 06:00 CRDT- 2022/08/04 11:13 PHST- 2022/04/25 00:00 [accepted] PHST- 2022/08/04 11:13 [entrez] PHST- 2022/08/05 06:00 [pubmed] PHST- 2022/08/09 06:00 [medline] AID - 10.1007/s00108-022-01340-w [pii] AID - 10.1007/s00108-022-01340-w [doi] PST - ppublish SO - Inn Med (Heidelb). 2022 Jun;63(6):591-600. doi: 10.1007/s00108-022-01340-w. Epub 2022 May 16. PMID- 23938099 OWN - NLM STAT- MEDLINE DCOM- 20140714 LR - 20130926 IS - 1578-2190 (Electronic) IS - 0001-7310 (Linking) VI - 104 IP - 8 DP - 2013 Oct TI - Smoker with Raynaud phenomenon and painful nodules on the legs. PG - 717-8 LID - S1578-2190(13)00181-9 [pii] LID - 10.1016/j.adengl.2013.03.007 [doi] FAU - Floristán, M U AU - Floristán MU AD - Unidad de Dermatología, Hospital Universitario Fundación Alcorcón, Alcorcón, Madrid, Spain. Electronic address: uxuafloristan@hotmail.com. FAU - Almodovar, R AU - Almodovar R FAU - Salamanca, F J AU - Salamanca FJ LA - eng PT - Case Reports PT - Journal Article DEP - 20130809 PL - Spain TA - Actas Dermosifiliogr JT - Actas dermo-sifiliograficas JID - 0373062 SB - IM MH - Biopsy MH - Erythema/*etiology/pathology MH - Humans MH - Ischemia/etiology MH - Leg/*blood supply MH - Raynaud Disease/*etiology MH - Skin Diseases/*etiology/pathology MH - Smoking/*adverse effects MH - Smoking Cessation MH - Thromboangiitis Obliterans/*diagnosis/etiology/pathology EDAT- 2013/08/14 06:00 MHDA- 2014/07/16 06:00 CRDT- 2013/08/14 06:00 PHST- 2013/01/02 00:00 [received] PHST- 2013/03/25 00:00 [accepted] PHST- 2013/08/14 06:00 [entrez] PHST- 2013/08/14 06:00 [pubmed] PHST- 2014/07/16 06:00 [medline] AID - S1578-2190(13)00181-9 [pii] AID - 10.1016/j.adengl.2013.03.007 [doi] PST - ppublish SO - Actas Dermosifiliogr. 2013 Oct;104(8):717-8. doi: 10.1016/j.adengl.2013.03.007. Epub 2013 Aug 9. PMID- 20811271 OWN - NLM STAT- MEDLINE DCOM- 20110106 LR - 20101201 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 340 IP - 6 DP - 2010 Dec TI - The different photoplethysmographic patterns can help to distinguish patients with primary and sclerodermic Raynaud phenomenon. PG - 457-61 LID - 10.1097/MAJ.0b013e3181eecfad [doi] AB - INTRODUCTION: The aim of this study is to investigate pulsatility of digital arteries of hands by means of photoplethysmography (PPG) in patients with primary Raynaud phenomenon (PRP) and systemic sclerosis (SSc) and to compare the results with those obtained in healthy controls. METHODS: One hundred five patients with SSc, 96 patients with PRP and 85 healthy controls were recruited in this study. Nailfold videocapillaroscopy and PPG were performed in healthy controls and patients. In patients with SSc, the capillaroscopic pattern was classified as early, active and late group pattern. A baseline PPG was recorded simultaneously in all 10 fingers of the hands. The photoplethysmographic curves were evaluated for morphology and amplitude of sphygmic wave. RESULTS: In healthy controls group, PPG shows the presence of photoplethysmographic homogeneous pattern and high mean value of sphygmic wave amplitude. In PRP group, PPG demonstrates homogeneous photoplethysmographic pattern and low mean value of sphygmic wave amplitude. Finally, in the SSc group, photoplethysmographic pattern is dyshomogeneous, and the mean value of sphygmic wave amplitude is intermediate between the other 2 groups. The PPG findings are different in the 3 capillaroscopic groups of patients with SSc and 2 subsets of disease. CONCLUSION: PPG represents a technique noninvasive to evaluate simultaneously in all 10 fingers of hands digital arteries pulsatility. PPG improves the evaluation of vascular damage in patients with primary and sclerodermic RP. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center, Sapienza University of Rome, Rome, Italy. FAU - Rossi, Carmelina AU - Rossi C FAU - Borghese, Federica AU - Borghese F FAU - Molinaro, Ilenia AU - Molinaro I FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Photoplethysmography/*methods MH - Raynaud Disease/*diagnosis/physiopathology MH - Scleroderma, Systemic/*diagnosis/physiopathology EDAT- 2010/09/03 06:00 MHDA- 2011/01/07 06:00 CRDT- 2010/09/03 06:00 PHST- 2010/09/03 06:00 [entrez] PHST- 2010/09/03 06:00 [pubmed] PHST- 2011/01/07 06:00 [medline] AID - S0002-9629(15)31424-5 [pii] AID - 10.1097/MAJ.0b013e3181eecfad [doi] PST - ppublish SO - Am J Med Sci. 2010 Dec;340(6):457-61. doi: 10.1097/MAJ.0b013e3181eecfad. PMID- 25974818 OWN - NLM STAT- MEDLINE DCOM- 20160104 LR - 20181202 IS - 1536-3732 (Electronic) IS - 1049-2275 (Linking) VI - 26 IP - 3 DP - 2015 May TI - Nasal septal perforation reconstruction with superior-based pedicled nasolabial island flap in a patient with the Raynaud phenomenon. PG - e209-10 LID - 10.1097/SCS.0000000000001432 [doi] AB - Perforation of the nasal septum may have multiple causes, and there are lots of options for reconstruction. We discuss a septal perforation case with Raynaud phenomenon. FAU - Keles, Musa Kemal AU - Keles MK AD - *Departments of Plastic, Reconstructive and Aesthetic Surgery †Anesthesia, Konya Numune Hospital ‡Department of ENT, Mevlana University, Konya, Turkey. FAU - Cepni, Hakan AU - Cepni H FAU - Cicekci, Faruk AU - Cicekci F FAU - Yenigün, Alper AU - Yenigün A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Craniofac Surg JT - The Journal of craniofacial surgery JID - 9010410 MH - Adult MH - Female MH - Humans MH - Nasal Septal Perforation/complications/*surgery MH - Raynaud Disease/*complications MH - Rhinoplasty/*methods MH - *Surgical Flaps EDAT- 2015/05/15 06:00 MHDA- 2016/01/05 06:00 CRDT- 2015/05/15 06:00 PHST- 2015/05/15 06:00 [entrez] PHST- 2015/05/15 06:00 [pubmed] PHST- 2016/01/05 06:00 [medline] AID - 00001665-201505000-00132 [pii] AID - 10.1097/SCS.0000000000001432 [doi] PST - ppublish SO - J Craniofac Surg. 2015 May;26(3):e209-10. doi: 10.1097/SCS.0000000000001432. PMID- 31453918 OWN - NLM STAT- MEDLINE DCOM- 20200717 LR - 20200717 IS - 1536-5166 (Electronic) IS - 1070-8022 (Linking) VI - 39 IP - 4 DP - 2019 Dec TI - Hereditary Angiopathy With Nephropathy, Aneurysm, and Muscle Cramps (HANAC) Syndrome Presenting to Neuro-Ophthalmology With Metamorphopsia. PG - 506-510 LID - 10.1097/WNO.0000000000000812 [doi] FAU - Jordan, Michael A AU - Jordan MA AD - Department of Ophthalmology and Visual Neurosciences (MAJ, MEP, RHJ, MSL, CMM), University of Minnesota School of Medicine, Minneapolis, Minnesota; Division of Genetics and Metabolism (MEP), Department of Pediatrics, University of Minnesota School of Medicine, Minneapolis, Minnesota; and Edina Retina Consultants, PA (RHJ), Edina, Minnesota. FAU - Pierpont, Mary Ella AU - Pierpont ME FAU - Johnston, Richard H AU - Johnston RH FAU - Lee, Michael S AU - Lee MS FAU - McClelland, Collin M AU - McClelland CM LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Neuroophthalmol JT - Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society JID - 9431308 RN - 0 (COL4A1 protein, human) RN - 0 (Collagen Type IV) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Collagen Type IV/genetics MH - Female MH - Humans MH - Middle Aged MH - Muscle Cramp/*diagnosis/genetics MH - Raynaud Disease/*diagnosis/genetics MH - Retinal Hemorrhage/diagnosis MH - Retinal Vessels/pathology MH - Tomography, Optical Coherence MH - Vision Disorders/*diagnosis EDAT- 2019/08/28 06:00 MHDA- 2020/07/18 06:00 CRDT- 2019/08/28 06:00 PHST- 2019/08/28 06:00 [pubmed] PHST- 2020/07/18 06:00 [medline] PHST- 2019/08/28 06:00 [entrez] AID - 10.1097/WNO.0000000000000812 [doi] PST - ppublish SO - J Neuroophthalmol. 2019 Dec;39(4):506-510. doi: 10.1097/WNO.0000000000000812. PMID- 18463040 OWN - NLM STAT- MEDLINE DCOM- 20080811 LR - 20210227 IS - 1934-2403 (Electronic) IS - 1530-891X (Linking) VI - 14 IP - 3 DP - 2008 Apr TI - Pseudohypoglycemia: a cause for unreliable finger-stick glucose measurements. PG - 337-9 AB - OBJECTIVE: To identify patients with an inaccurate diagnosis of hypoglycemia and discuss predisposing factors. METHODS: We describe our patient's clinical presentation, laboratory work-up, hospital course, and follow-up and review similar cases from the literature. RESULTS: A 27-year-old woman with Raynaud phenomenon was admitted because of symptomatic hypoglycemia. Physical examination showed tremulousness, sweating, and the classic Raynaud color changes of the hands during episodic symptoms. A 72-hour fast revealed finger-stick capillary glucose values ranging from 32 to 45 mg/dL on multiple occasions, while concurrent plasma glucose values were consistently 1.5 to 2 times higher. Capillary measurements of glucose performed in the arms and legs at room temperature and after warming of each extremity disclosed an increase in glucose levels from a range of 35 to 52 mg/dL at room temperature to a range of 82 to 100 mg/dL after warming, confirming a discordance between capillary and venous blood results. The diagnosis of pseudohypoglycemia was made. Pseudohypoglycemia has been reported in patients with Raynaud phenomenon, peripheral vascular disease, and shock and may result from increased glucose extraction by the tissues because of low capillary flow and increased glucose transit time. CONCLUSION: Pseudohypoglycemia should be suspected in the setting of impaired microcirculation and can be confirmed by readily available means. FAU - El Khoury, Marc AU - El Khoury M AD - Department of Internal Medicine, University of Cincinnati, College of Medicine, Cincinnati, Ohio 45208, USA. FAU - Yousuf, Farheen AU - Yousuf F FAU - Martin, Vincent AU - Martin V FAU - Cohen, Robert M AU - Cohen RM LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - Endocr Pract JT - Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists JID - 9607439 RN - 0 (Blood Glucose) SB - IM MH - Adult MH - Blood Glucose/metabolism MH - Blood Glucose Self-Monitoring/*instrumentation/methods MH - *Diagnostic Errors MH - Female MH - Fingers/*blood supply MH - Humans MH - Hypoglycemia/*blood/*diagnosis MH - Microcirculation/physiopathology MH - Raynaud Disease/blood/physiopathology MH - Regional Blood Flow/physiology RF - 6 EDAT- 2008/05/09 09:00 MHDA- 2008/08/12 09:00 CRDT- 2008/05/09 09:00 PHST- 2008/05/09 09:00 [pubmed] PHST- 2008/08/12 09:00 [medline] PHST- 2008/05/09 09:00 [entrez] AID - S1530-891X(20)43582-7 [pii] AID - 10.4158/EP.14.3.337 [doi] PST - ppublish SO - Endocr Pract. 2008 Apr;14(3):337-9. doi: 10.4158/EP.14.3.337. PMID- 26260163 OWN - NLM STAT- MEDLINE DCOM- 20160811 LR - 20181113 IS - 1533-3450 (Electronic) IS - 1046-6673 (Print) IS - 1046-6673 (Linking) VI - 27 IP - 4 DP - 2016 Apr TI - HANAC Syndrome Col4a1 Mutation Causes Neonate Glomerular Hyperpermeability and Adult Glomerulocystic Kidney Disease. PG - 1042-54 LID - 10.1681/ASN.2014121217 [doi] AB - Hereditary angiopathy, nephropathy, aneurysms, and muscle cramps (HANAC) syndrome is an autosomal dominant syndrome caused by mutations in COL4A1 that encodes the α1 chain of collagen IV, a major component of basement membranes. Patients present with cerebral small vessel disease, retinal tortuosity, muscle cramps, and kidney disease consisting of multiple renal cysts, chronic kidney failure, and sometimes hematuria. Mutations producing HANAC syndrome localize within the integrin binding site containing CB3[IV] fragment of the COL4A1 protein. To investigate the pathophysiology of HANAC syndrome, we generated mice harboring the Col4a1 p.Gly498Val mutation identified in a family with the syndrome. Col4a1 G498V mutation resulted in delayed glomerulogenesis and podocyte differentiation without reduction of nephron number, causing albuminuria and hematuria in newborns. The glomerular defects resolved within the first month, but glomerular cysts developed in 3-month-old mutant mice. Abnormal structure of Bowman's capsule was associated with metalloproteinase induction and activation of the glomerular parietal epithelial cells that abnormally expressed CD44,α-SMA, ILK, and DDR1. Inflammatory infiltrates were observed around glomeruli and arterioles. Homozygous Col4a1 G498V mutant mice additionally showed dysmorphic papillae and urinary concentration defects. These results reveal a developmental role for the α1α1α2 collagen IV molecule in the embryonic glomerular basement membrane, affecting podocyte differentiation. The observed association between molecular alteration of the collagenous network in Bowman's capsule of the mature kidney and activation of parietal epithelial cells, matrix remodeling, and inflammation may account for glomerular cyst development and CKD in patients with COL4A1-related disorders. CI - Copyright © 2016 by the American Society of Nephrology. FAU - Chen, Zhiyong AU - Chen Z AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; FAU - Migeon, Tiffany AU - Migeon T AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; Sorbonne University, Université Pierre et Marie Curie, Paris 06, UMR_S 1155, Paris, France; FAU - Verpont, Marie-Christine AU - Verpont MC AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; Sorbonne University, Université Pierre et Marie Curie, Paris 06, UMR_S 1155, Paris, France; FAU - Zaidan, Mohamad AU - Zaidan M AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; FAU - Sado, Yoshikazu AU - Sado Y AD - Division of Immunology, Shigei Medical Research Institute, Okayama, Japan; FAU - Kerjaschki, Dontscho AU - Kerjaschki D AD - Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria; and. FAU - Ronco, Pierre AU - Ronco P AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; Sorbonne University, Université Pierre et Marie Curie, Paris 06, UMR_S 1155, Paris, France; Assistance Publique-Hôpitaux de Paris, Department of Nephrology and Dialysis, Tenon Hospital, Paris, France. FAU - Plaisier, Emmanuelle AU - Plaisier E AD - Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche (UMR)S 1155, Paris, France; Sorbonne University, Université Pierre et Marie Curie, Paris 06, UMR_S 1155, Paris, France; Assistance Publique-Hôpitaux de Paris, Department of Nephrology and Dialysis, Tenon Hospital, Paris, France emmanuelle.plaisier@tnn.aphp.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150810 PL - United States TA - J Am Soc Nephrol JT - Journal of the American Society of Nephrology : JASN JID - 9013836 RN - 0 (Collagen Type IV) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Age Factors MH - Animals MH - Animals, Newborn MH - Collagen Type IV/*genetics MH - Kidney Diseases, Cystic/*etiology/metabolism MH - Kidney Glomerulus/metabolism MH - Mice MH - Muscle Cramp/*complications/*genetics/metabolism/physiopathology MH - *Mutation MH - Permeability MH - Raynaud Disease/*complications/*genetics/metabolism/physiopathology PMC - PMC4814176 OTO - NOTNLM OT - genetic renal disease OT - glomerular epithelial cells OT - kidney development OT - molecular biology EDAT- 2015/08/12 06:00 MHDA- 2016/08/12 06:00 PMCR- 2017/04/01 CRDT- 2015/08/12 06:00 PHST- 2014/12/12 00:00 [received] PHST- 2015/06/16 00:00 [accepted] PHST- 2015/08/12 06:00 [entrez] PHST- 2015/08/12 06:00 [pubmed] PHST- 2016/08/12 06:00 [medline] PHST- 2017/04/01 00:00 [pmc-release] AID - ASN.2014121217 [pii] AID - 2014121217 [pii] AID - 10.1681/ASN.2014121217 [doi] PST - ppublish SO - J Am Soc Nephrol. 2016 Apr;27(4):1042-54. doi: 10.1681/ASN.2014121217. Epub 2015 Aug 10. PMID- 20436070 OWN - NLM STAT- MEDLINE DCOM- 20100708 LR - 20220408 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 6 DP - 2010 Jun TI - Peripheral blood perfusion correlates with microvascular abnormalities in systemic sclerosis: a laser-Doppler and nailfold videocapillaroscopy study. PG - 1174-80 LID - 10.3899/jrheum.091356 [doi] AB - OBJECTIVE: To investigate possible correlations between fingertip blood perfusion (FBP) status, assessed by laser Doppler flowmetry (LDF), and morphological microvascular abnormalities, detected by nailfold videocapillaroscopy (NVC), in patients with systemic sclerosis (SSc). The effects on FBP of intravenous (IV) treatment with the prostacyclin analog iloprost were also investigated. METHODS: Thirty-four consecutive patients with SSc and 16 healthy subjects were evaluated. LDF was performed by analyzing blood perfusion at the fingertips in both hands. Patients with SSc were distributed into the appropriate NVC pattern of microangiopathy (early, active, and late). Iloprost was administered to inpatients with SSc by 24-hour IV infusion for 7 consecutive days (4 microg/h). RESULTS: FBP was significantly lower in patients with SSc (p < 0.05) compared to controls. Heating of the LDF probe at 36 degrees C induced a significant increase of FBP in all subjects (p < 0.001), but the slope of variation was significantly lower in patients with SSc compared to controls (p < 0.05). Patients with SSc showing the late NVC pattern of microangiopathy had significantly lower FBP than patients with the active and early NVC patterns (p < 0.05). A negative correlation was observed between FBP and NVC rating of the microvascular damage (p < 0.05). After iloprost treatment, a significant increase of FBP was observed in patients with SSc (p < 0.05). CONCLUSION: Patients with SSc show a decreased FBP partially reversible by local skin heating. The FBP correlated negatively with the extent of nailfold microvascular damage, and IV iloprost treatment increased the FBP. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, no. 6, 16132 Genova, Italy. mcutolo@unige.it FAU - Ferrone, Carmela AU - Ferrone C FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Soldano, Stefano AU - Soldano S FAU - Seriolo, Bruno AU - Seriolo B FAU - Sulli, Alberto AU - Sulli A LA - eng PT - Journal Article DEP - 20100501 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Capillaries/drug effects/pathology/physiology/physiopathology MH - Female MH - Humans MH - Hyperthermia, Induced MH - Iloprost/therapeutic use MH - Injections, Intravenous MH - Laser-Doppler Flowmetry/*methods MH - Microcirculation/drug effects/physiology MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply/drug effects MH - Raynaud Disease/complications/drug therapy/*pathology/physiopathology MH - Scleroderma, Systemic/complications/drug therapy/*pathology/physiopathology MH - Vasodilation/drug effects/physiology MH - Vasodilator Agents/therapeutic use EDAT- 2010/05/04 06:00 MHDA- 2010/07/09 06:00 CRDT- 2010/05/04 06:00 PHST- 2010/05/04 06:00 [entrez] PHST- 2010/05/04 06:00 [pubmed] PHST- 2010/07/09 06:00 [medline] AID - jrheum.091356 [pii] AID - 10.3899/jrheum.091356 [doi] PST - ppublish SO - J Rheumatol. 2010 Jun;37(6):1174-80. doi: 10.3899/jrheum.091356. Epub 2010 May 1. PMID- 18051256 OWN - NLM STAT- MEDLINE DCOM- 20080201 LR - 20071204 IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 19 IP - 6 DP - 2007 Nov TI - Bibliography. Current world literature. Raynaud phenomenon, scleroderma, overlap syndromes and other fibrosing syndromes. PG - 653-6 LA - eng PT - Bibliography PT - Journal Article PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - *Raynaud Disease MH - *Scleroderma, Systemic MH - Syndrome EDAT- 2007/12/07 09:00 MHDA- 2008/02/02 09:00 CRDT- 2007/12/07 09:00 PHST- 2007/12/07 09:00 [pubmed] PHST- 2008/02/02 09:00 [medline] PHST- 2007/12/07 09:00 [entrez] PST - ppublish SO - Curr Opin Rheumatol. 2007 Nov;19(6):653-6. PMID- 35773968 OWN - NLM STAT- MEDLINE DCOM- 20230816 LR - 20260217 IS - 2542-5641 (Electronic) IS - 0366-6999 (Print) IS - 0366-6999 (Linking) VI - 135 IP - 9 DP - 2022 May 5 TI - Botulinum toxin in the treatment of Raynaud phenomenon in patients with systemic sclerosis: a systemic review. PG - 1133-1134 LID - 10.1097/CM9.0000000000001903 [doi] FAU - Cai, Ruyi AU - Cai R AD - Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing 100191, China. FAU - Yi, Zixi AU - Yi Z FAU - Li, Ting AU - Li T FAU - Mu, Rong AU - Mu R LA - eng PT - Letter PT - Systematic Review DEP - 20220505 PL - China TA - Chin Med J (Engl) JT - Chinese medical journal JID - 7513795 RN - EC 3.4.24.69 (Botulinum Toxins) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Humans MH - *Botulinum Toxins MH - *Botulinum Toxins, Type A MH - Immunotherapy MH - *Raynaud Disease/drug therapy MH - *Scleroderma, Systemic/drug therapy PMC - PMC9276337 COIS- None. EDAT- 2022/07/02 06:00 MHDA- 2022/07/06 06:00 PMCR- 2022/05/05 CRDT- 2022/07/01 01:32 PHST- 2022/07/01 01:32 [entrez] PHST- 2022/07/02 06:00 [pubmed] PHST- 2022/07/06 06:00 [medline] PHST- 2022/05/05 00:00 [pmc-release] AID - 00029330-202205050-00026 [pii] AID - CMJ-2021-1641 [pii] AID - 10.1097/CM9.0000000000001903 [doi] PST - epublish SO - Chin Med J (Engl). 2022 May 5;135(9):1133-1134. doi: 10.1097/CM9.0000000000001903. PMID- 32244020 OWN - NLM STAT- MEDLINE DCOM- 20210414 LR - 20210414 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 83 IP - 6 DP - 2020 Dec TI - Reply to "Interdigital injection of botulinum toxin for patients with Raynaud phenomenon". PG - e411 LID - S0190-9622(20)30489-8 [pii] LID - 10.1016/j.jaad.2020.03.066 [doi] FAU - Quintana-Castanedo, Lucía AU - Quintana-Castanedo L AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. Electronic address: luciaquintana.e@gmail.com. FAU - Feito-Rodríguez, Marta AU - Feito-Rodríguez M AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. FAU - De Lucas-Laguna, Raúl AU - De Lucas-Laguna R AD - Department of Dermatology, La Paz University Hospital, Madrid, Spain. LA - eng PT - Comment PT - Letter DEP - 20200331 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM CON - J Am Acad Dermatol. 2020 Dec;83(6):e399. doi: 10.1016/j.jaad.2019.11.060. PMID: 31821859 CON - J Am Acad Dermatol. 2020 Dec;83(6):e409-e410. doi: 10.1016/j.jaad.2020.02.085. PMID: 32244019 MH - *Botulinum Toxins/therapeutic use MH - Humans MH - Injections MH - *Neuromuscular Agents/therapeutic use MH - *Raynaud Disease/drug therapy EDAT- 2020/04/04 06:00 MHDA- 2021/04/15 06:00 CRDT- 2020/04/04 06:00 PHST- 2020/03/21 00:00 [received] PHST- 2020/03/23 00:00 [accepted] PHST- 2020/04/04 06:00 [pubmed] PHST- 2021/04/15 06:00 [medline] PHST- 2020/04/04 06:00 [entrez] AID - S0190-9622(20)30489-8 [pii] AID - 10.1016/j.jaad.2020.03.066 [doi] PST - ppublish SO - J Am Acad Dermatol. 2020 Dec;83(6):e411. doi: 10.1016/j.jaad.2020.03.066. Epub 2020 Mar 31. PMID- 27219314 OWN - NLM STAT- MEDLINE DCOM- 20170602 LR - 20170602 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 22 IP - 4 DP - 2016 Jun TI - Nailfold Capillary Patterns in a Patient With Multicentric Reticulohistiocytosis and Raynaud Phenomenon. PG - 220-1 LID - 10.1097/RHU.0000000000000270 [doi] FAU - Corominas, Hèctor AU - Corominas H AD - From the *Servei de Reumatologia and †Servei de Dermatologia; and Hospital Moisès Broggi (CSI), Sant Joan Despí, Barcelona, Catalonia, Spain. FAU - Villareal, Johan AU - Villareal J FAU - Estrada, Paula AU - Estrada P FAU - Roig-Vilaseca, Daniel AU - Roig-Vilaseca D FAU - Torrente-Segarra, Vicenç AU - Torrente-Segarra V FAU - Reina, Delia AU - Reina D FAU - Cerdà-Gabaroi, Dacia AU - Cerdà-Gabaroi D FAU - García-Diaz, Silvia AU - García-Diaz S FAU - Vidal, David AU - Vidal D LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Aged MH - Biopsy MH - Capillaries/*pathology MH - Diagnosis, Differential MH - Female MH - Histiocytosis/*diagnosis MH - Humans MH - Nail Diseases/*diagnosis MH - Raynaud Disease/*diagnosis EDAT- 2016/05/25 06:00 MHDA- 2017/06/03 06:00 CRDT- 2016/05/25 06:00 PHST- 2016/05/25 06:00 [entrez] PHST- 2016/05/25 06:00 [pubmed] PHST- 2017/06/03 06:00 [medline] AID - 00124743-201606000-00015 [pii] AID - 10.1097/RHU.0000000000000270 [doi] PST - ppublish SO - J Clin Rheumatol. 2016 Jun;22(4):220-1. doi: 10.1097/RHU.0000000000000270. PMID- 31460971 OWN - NLM STAT- MEDLINE DCOM- 20200330 LR - 20200330 IS - 1547-1896 (Print) IS - 0893-7400 (Linking) VI - 32 IP - 9 DP - 2019 Sep TI - Hypothenar hammer syndrome: An uncommon cause of secondary syndrome and digital ischemia. PG - 33-35 LID - 10.1097/01.JAA.0000578972.17680.39 [doi] AB - Hypothenar hammer syndrome affects less than 1% of the population, but if the diagnosis is delayed, digital gangrene and critical ischemia can ensue. The condition is caused by injury to the ulnar artery at the level of the hook of hamate when the palm of the hand is repetitively used as a hammer. Injury includes segmental occlusion of the ulnar artery and aneurysmal formation with or without occlusion. Patients with hypothenar hammer syndrome often present with symptoms of secondary Raynaud syndrome; if Raynaud is unilateral, a vascular origin should be suspected and ruled out. Treatment options for hypothenar hammer syndrome include conservative treatment measures, fibrinolysis, or surgical resection and repair, and depend on the specific injury and timing of diagnosis. FAU - Finke-Fyffe, Sarah AU - Finke-Fyffe S AD - At the time this article was written, Sarah Finke-Fyffe practiced at North Shore Vascular Associates in Northfield, Ill. She now practices at Ohio State University's Wexner Medical Center in Columbus, Ohio. At North Shore Vascular Associates, Jeanne Regan is a registered vascular technologist and John Golan is president and a vascular surgeon. The authors have disclosed no potential conflicts of interest, financial or otherwise. FAU - Regan, Jeanne AU - Regan J FAU - Golan, John AU - Golan J LA - eng PT - Case Reports PT - Journal Article PL - United States TA - JAAPA JT - JAAPA : official journal of the American Academy of Physician Assistants JID - 9513102 MH - Adult MH - Aneurysm/complications/*diagnostic imaging/surgery MH - Humans MH - Magnetic Resonance Angiography MH - Male MH - Raynaud Disease/*diagnosis/etiology MH - Ulnar Artery/*diagnostic imaging/injuries/surgery MH - Ultrasonography EDAT- 2019/08/29 06:00 MHDA- 2020/03/31 06:00 CRDT- 2019/08/29 06:00 PHST- 2019/08/29 06:00 [entrez] PHST- 2019/08/29 06:00 [pubmed] PHST- 2020/03/31 06:00 [medline] AID - 01720610-201909000-00007 [pii] AID - 10.1097/01.JAA.0000578972.17680.39 [doi] PST - ppublish SO - JAAPA. 2019 Sep;32(9):33-35. doi: 10.1097/01.JAA.0000578972.17680.39. PMID- 34237010 OWN - NLM STAT- MEDLINE DCOM- 20210831 LR - 20210831 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 48 IP - 4 DP - 2021 Apr TI - Selexipag Therapy for Raynaud Phenomenon-induced Severe Digital Ischemia in Intravenous Epoprostenol Responders With Connective Tissue Disease. PG - 616-617 LID - 10.3899/jrheum.200716 [doi] FAU - Langleben, David AU - Langleben D AUID- ORCID: 0000-0002-6495-0094 AD - Center for Pulmonary Vascular Disease, Azrieli Heart Center, Division of Cardiology; david.langleben@mcgill.ca. FAU - Berkson, Laeora AU - Berkson L AD - Division of Rheumatology, Jewish General Hospital and McGill University. FAU - Chartrand, Sandra AU - Chartrand S AD - Division of Rheumatology, Hôpital Maisonneuve-Rosemont and Université de Montreal, Montreal, Quebec, Canada. LA - eng PT - Journal Article PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Acetamides) RN - 0 (Antihypertensive Agents) RN - 0 (Pyrazines) RN - 5EXC0E384L (selexipag) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Acetamides MH - Antihypertensive Agents/adverse effects MH - *Connective Tissue Diseases/drug therapy MH - Epoprostenol/therapeutic use MH - Humans MH - Ischemia/drug therapy MH - Pyrazines MH - *Raynaud Disease/drug therapy EDAT- 2021/07/09 06:00 MHDA- 2021/09/01 06:00 CRDT- 2021/07/08 17:25 PHST- 2021/07/08 17:25 [entrez] PHST- 2021/07/09 06:00 [pubmed] PHST- 2021/09/01 06:00 [medline] AID - 48/4/616 [pii] AID - 10.3899/jrheum.200716 [doi] PST - ppublish SO - J Rheumatol. 2021 Apr;48(4):616-617. doi: 10.3899/jrheum.200716. PMID- 17517216 OWN - NLM STAT- MEDLINE DCOM- 20071206 LR - 20190917 IS - 1695-4033 (Print) IS - 1695-4033 (Linking) VI - 66 IP - 5 DP - 2007 May TI - [Infant with symmetric acrocyanosis of both feet]. PG - 547-8 FAU - Cabezuelo Huerta, G AU - Cabezuelo Huerta G AD - Servicio de Pediatría, Hospital Universitario La Fe, Valencia, Spain. FAU - Vidal Micó, S AU - Vidal Micó S FAU - Abeledo Gómez, A AU - Abeledo Gómez A FAU - Frontera Izquierdo, P AU - Frontera Izquierdo P LA - spa PT - Case Reports PT - Journal Article TT - Lactante con acrocianosis simétrica de ambos pies. PL - Spain TA - An Pediatr (Barc) JT - Anales de pediatria (Barcelona, Spain : 2003) JID - 101162596 SB - IM MH - Female MH - Foot MH - Humans MH - Infant MH - Raynaud Disease/*diagnosis EDAT- 2007/05/23 09:00 MHDA- 2007/12/07 09:00 CRDT- 2007/05/23 09:00 PHST- 2007/05/23 09:00 [pubmed] PHST- 2007/12/07 09:00 [medline] PHST- 2007/05/23 09:00 [entrez] AID - 13102526 [pii] AID - 10.1157/13102526 [doi] PST - ppublish SO - An Pediatr (Barc). 2007 May;66(5):547-8. doi: 10.1157/13102526. PMID- 26181855 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20221207 IS - 1538-8654 (Electronic) IS - 1527-7941 (Linking) VI - 28 IP - 8 DP - 2015 Aug TI - Ulceration of the Digits: Autoamputation Cause and Consequence. PG - 342 LID - 10.1097/01.ASW.0000469335.06257.db [doi] FAU - Salcido, Richard AU - Salcido R AD - Richard "Sal" Salcido, MD, EdD, is the Editor-in-Chief of Advances in Skin & Wound Care and the Course Director for the Annual Clinical Symposium on Advances in Skin & Wound Care. He is the William Erdman Professor, Department of Physical Medicine and Rehabilitation; Senior Fellow, Institute on Aging; and Associate, Institute of Medicine and Bioengineering, at the University of Pennsylvania Health System, Philadelphia. LA - eng PT - Editorial PL - United States TA - Adv Skin Wound Care JT - Advances in skin & wound care JID - 100911021 MH - *Amputation, Surgical MH - Diabetic Angiopathies/*complications/therapy MH - Humans MH - Raynaud Disease/*complications/therapy MH - Thromboangiitis Obliterans/*complications/therapy EDAT- 2015/07/17 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/07/17 06:00 PHST- 2015/07/17 06:00 [entrez] PHST- 2015/07/17 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 00129334-201508000-00001 [pii] AID - 10.1097/01.ASW.0000469335.06257.db [doi] PST - ppublish SO - Adv Skin Wound Care. 2015 Aug;28(8):342. doi: 10.1097/01.ASW.0000469335.06257.db. PMID- 24377442 OWN - NLM STAT- MEDLINE DCOM- 20141205 LR - 20140218 IS - 1532-1940 (Electronic) IS - 0266-4356 (Linking) VI - 52 IP - 3 DP - 2014 Mar TI - Antisynthetase syndrome: two cases presenting orofacial manifestations. PG - 285-7 LID - S0266-4356(13)00527-5 [pii] LID - 10.1016/j.bjoms.2013.12.006 [doi] AB - Antisynthetase syndrome is a rare autoimmune disease that is characterised by inflammatory myositis, and interstitial lung disease or chronic arthropathy, or both. To the best of our knowledge, orofacial manifestations have not previously been reported. We present 2 patients with orofacial disease: one with aphthous-like oral ulceration and the other with hyposalivation. CI - Copyright © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Gormley, M AU - Gormley M AD - Edinburgh Dental Institute, Lauriston Building, Lauriston Place, Edinburgh, Scotland, UK. Electronic address: mark.gormley@nhslothian.scot.nhs.uk. FAU - Scully, C AU - Scully C AD - Edinburgh Dental Institute, Lauriston Building, Lauriston Place, Edinburgh, Scotland, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20131227 PL - Scotland TA - Br J Oral Maxillofac Surg JT - The British journal of oral & maxillofacial surgery JID - 8405235 RN - Antisynthetase syndrome SB - IM MH - Female MH - Herpes Labialis/complications MH - Humans MH - Middle Aged MH - Myositis/*complications MH - Raynaud Disease/complications MH - Stomatitis, Aphthous/*etiology MH - Xerostomia/*etiology OTO - NOTNLM OT - Antisynthetase syndrome mouth ulcers OT - Dry mouth OT - Raynaud phenomenon EDAT- 2014/01/01 06:00 MHDA- 2014/12/15 06:00 CRDT- 2014/01/01 06:00 PHST- 2013/05/12 00:00 [received] PHST- 2013/12/07 00:00 [accepted] PHST- 2014/01/01 06:00 [entrez] PHST- 2014/01/01 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] AID - S0266-4356(13)00527-5 [pii] AID - 10.1016/j.bjoms.2013.12.006 [doi] PST - ppublish SO - Br J Oral Maxillofac Surg. 2014 Mar;52(3):285-7. doi: 10.1016/j.bjoms.2013.12.006. Epub 2013 Dec 27. PMID- 35192594 OWN - NLM STAT- MEDLINE DCOM- 20220224 LR - 20230927 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 28 IP - 2 DP - 2022 Mar 1 TI - Clinical Significance of Raynaud Phenomenon in Systemic Lupus Erythematosus. PG - e488-e490 LID - 10.1097/RHU.0000000000001773 [doi] AB - OBJECTIVE: There are limited reports of the clinical significance of Raynaud phenomenon (RP) in systemic lupus erythematosus (SLE), with some suggesting RP is associated with less severe lupus. Since most prior studies were small and/or focused on a specific race/ethnic demographic, it is unclear if those results are generalizable. We evaluated whether RP was associated with demographic and clinical factors in a large multiethnic SLE cohort. METHODS: We studied Montreal General Hospital SLE cohort patients who are followed with standardized annual assessments. We included patients with at least 1 visit across 2011-2018 and assessed demographic and clinical variables (using the 1997 American College of Rheumatology criteria and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index) at their first visit. We present multivariate logistics regression analyses of cross-sectional associations between these variables and RP in SLE. RESULTS: Of 489 SLE patients, most were female (n = 445, 91%). Mean age at SLE diagnosis was 31.5 (standard deviation, 13.5) years, and 169 (34.6%) had RP. In our fully adjusted model, female sex (odds ratio [OR], 2.43; 95% confidence interval [CI], 1.07-6.03), White race/ethnicity (OR, 1.85; 95% CI, 1.10-3.17), neurological/neuropsychiatric manifestations (OR, 1.98; 95% CI, 1.10-3.56), and anti-RNP antibodies (OR, 3.03; 95% CI, 1.73-5.38) were positively associated with RP, whereas hemolytic anemia and cellular casts were negatively associated. CONCLUSIONS/DISCUSSION: Over one third of our large multiethnic North American SLE cohort had RP. This study confirmed associations between RP and a specific SLE phenotype. CI - Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved. FAU - Barbacki, Ariane AU - Barbacki A AD - From the McGill University Health Center. FAU - Rached-d'Astous, Nour AU - Rached-d'Astous N AD - From the McGill University Health Center. FAU - Pineau, Christian A AU - Pineau CA AD - From the McGill University Health Center. FAU - Vinet, Evelyne AU - Vinet E AD - From the McGill University Health Center. FAU - Grenier, Louis-Pierre AU - Grenier LP AD - From the McGill University Health Center. FAU - Kalache, Fares AU - Kalache F AD - From the McGill University Health Center. FAU - Fallavollita, Sabrina AU - Fallavollita S AD - Lady Davis Institute for Medical Research, Montreal, Quebec, Canada. FAU - Lukusa, Luck AU - Lukusa L AD - From the McGill University Health Center. FAU - Bernatsky, Sasha AU - Bernatsky S AD - From the McGill University Health Center. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Cohort Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Lupus Erythematosus, Discoid/complications MH - *Lupus Erythematosus, Systemic/complications/diagnosis/epidemiology MH - *Raynaud Disease/diagnosis/epidemiology/etiology COIS- The authors declare no conflict of interest. EDAT- 2022/02/23 06:00 MHDA- 2022/02/25 06:00 CRDT- 2022/02/22 17:12 PHST- 2022/02/23 06:00 [pubmed] PHST- 2022/02/25 06:00 [medline] PHST- 2022/02/22 17:12 [entrez] AID - 00124743-202203000-00038 [pii] AID - 10.1097/RHU.0000000000001773 [doi] PST - ppublish SO - J Clin Rheumatol. 2022 Mar 1;28(2):e488-e490. doi: 10.1097/RHU.0000000000001773. PMID- 18447200 OWN - NLM STAT- MEDLINE DCOM- 20080806 LR - 20091119 IS - 0032-6518 (Print) IS - 0032-6518 (Linking) VI - 252 IP - 1704 DP - 2008 Mar TI - Hand problems. PG - 39, 41-3 FAU - Stollery, Nigel AU - Stollery N AD - Leicester Royal Infirmary. LA - eng PT - Journal Article PT - Review PL - England TA - Practitioner JT - The Practitioner JID - 0404245 SB - IM MH - Acromegaly/*diagnosis MH - Dupuytren Contracture/*diagnosis MH - Ganglion Cysts/*diagnosis MH - Granuloma, Pyogenic/*diagnosis MH - Hand Dermatoses/*diagnosis MH - Humans MH - Photography MH - Raynaud Disease/*diagnosis RF - 9 EDAT- 2008/05/02 09:00 MHDA- 2008/08/07 09:00 CRDT- 2008/05/02 09:00 PHST- 2008/05/02 09:00 [pubmed] PHST- 2008/08/07 09:00 [medline] PHST- 2008/05/02 09:00 [entrez] PST - ppublish SO - Practitioner. 2008 Mar;252(1704):39, 41-3. PMID- 16924768 OWN - NLM STAT- MEDLINE DCOM- 20060823 LR - 20161020 IS - 1052-1577 (Print) IS - 1052-1577 (Linking) VI - 31 IP - 10 DP - 2006 Aug TI - Viagra may help with cold feet and hands. PG - 7 LA - eng PT - News PL - United States TA - Harv Health Lett JT - Harvard health letter JID - 9425764 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) MH - Humans MH - Piperazines/*therapeutic use MH - Purines MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate MH - Sulfones MH - Vasodilator Agents/*therapeutic use EDAT- 2006/08/24 09:00 MHDA- 2006/08/24 09:01 CRDT- 2006/08/24 09:00 PHST- 2006/08/24 09:00 [pubmed] PHST- 2006/08/24 09:01 [medline] PHST- 2006/08/24 09:00 [entrez] PST - ppublish SO - Harv Health Lett. 2006 Aug;31(10):7. PMID- 18953973 OWN - NLM STAT- MEDLINE DCOM- 20081118 LR - 20220409 IS - 0002-838X (Print) IS - 0002-838X (Linking) VI - 78 IP - 8 DP - 2008 Oct 15 TI - Systemic sclerosis/scleroderma: a treatable multisystem disease. PG - 961-8 AB - Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or middle-age woman with a history of Raynaud phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, and high-resolution computed tomography of the chest, establish the diagnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. Prognosis is determined by the degree of internal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. Medications (e.g., calcium channel blockers and angiotensin-II receptor blockers for Raynaud phenomenon, appropriate treatments for gastroesophageal reflux disease) and lifestyle modifications can help prevent complications, such as digital ulcers and Barrett esophagus. Endothelin-1 receptor blockers and phosphodiesterase-5 inhibitors improve pulmonary arterial hypertension. The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. Optimal patient care includes an integrated, multidisciplinary approach to promptly and effectively recognize, evaluate, and manage complications and limit end-organ dysfunction. FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Northwestern University, Feinberg School of Medicine, Chicago, Illinois 60611, USA. m-hinchcliff@northwestern.edu FAU - Varga, John AU - Varga J LA - eng PT - Journal Article PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 SB - IM MH - Arthralgia/etiology/therapy MH - Deglutition Disorders/etiology/therapy MH - Diagnosis, Differential MH - Dyspnea/etiology/therapy MH - Gastroesophageal Reflux/etiology/therapy MH - Heart Diseases/etiology/therapy MH - Humans MH - Kidney Diseases/etiology/therapy MH - Lung Diseases/etiology/therapy MH - Prognosis MH - Raynaud Disease/etiology/therapy MH - Scleroderma, Systemic/*complications/*diagnosis/epidemiology/therapy EDAT- 2008/10/29 09:00 MHDA- 2008/11/19 09:00 CRDT- 2008/10/29 09:00 PHST- 2008/10/29 09:00 [pubmed] PHST- 2008/11/19 09:00 [medline] PHST- 2008/10/29 09:00 [entrez] PST - ppublish SO - Am Fam Physician. 2008 Oct 15;78(8):961-8. PMID- 21513046 OWN - NLM STAT- MEDLINE DCOM- 20111213 LR - 20181201 IS - 0032-6518 (Print) IS - 0032-6518 (Linking) VI - 255 IP - 1738 DP - 2011 Mar TI - Photoguide: Ganglion or myxoid cyst? PG - 31; author reply 31 FAU - Guinan, Kevin AU - Guinan K LA - eng PT - Comment PT - Letter PL - England TA - Practitioner JT - The Practitioner JID - 0404245 SB - IM CON - Practitioner. 2010 Nov;254(1734):35-7. PMID: 21166298 MH - Chilblains/*pathology MH - Ganglion Cysts/*pathology MH - Granuloma, Pyogenic/*pathology MH - Humans MH - Paronychia/*pathology MH - Raynaud Disease/*pathology MH - Warts/*pathology EDAT- 2011/04/26 06:00 MHDA- 2011/12/14 06:00 CRDT- 2011/04/23 06:00 PHST- 2011/04/23 06:00 [entrez] PHST- 2011/04/26 06:00 [pubmed] PHST- 2011/12/14 06:00 [medline] PST - ppublish SO - Practitioner. 2011 Mar;255(1738):31; author reply 31. PMID- 19300288 OWN - NLM STAT- MEDLINE DCOM- 20090707 LR - 20131121 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 15 IP - 3 DP - 2009 Apr TI - Acrocyanosis from phenazopyridine-induced sulfhemoglobinemia mistaken for Raynaud phenomenon. PG - 127-9 LID - 10.1097/RHU.0b013e31819db6db [doi] AB - Rheumatologists are often asked to evaluate patients with Raynaud phenomenon. Occasionally, an alternate explanation is revealed such as acrocyanosis. Methemoglobinemia and sulfhemoglobinemia are rare causes of cyanosis that can be medication-induced. Both are known complications of therapy with phenazopyridine. We report an unusual case of a 45-year-old woman in whom sulfhemoglobinemia from chronic therapy with phenazopyridine was misdiagnosed as due to Raynaud phenomenon and limited scleroderma. This case illustrates the importance of taking into account medication-related adverse events when evaluating patients with Raynaud-like phenomenon. FAU - Kermani, Tanaz A AU - Kermani TA AD - Division of Rheumatology, Mayo Clinic, Rochester, MN 55905, USA. kermani.tanaz@mayo.edu FAU - Pislaru, Sorin V AU - Pislaru SV FAU - Osborn, Thomas G AU - Osborn TG LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Anesthetics, Local) RN - K2J09EMJ52 (Phenazopyridine) SB - IM MH - Adult MH - Anesthetics, Local/*adverse effects MH - Cyanosis/*etiology MH - Diagnosis, Differential MH - Dyspnea/etiology MH - Female MH - Humans MH - Phenazopyridine/*adverse effects MH - Polypharmacy MH - Raynaud Disease/*diagnosis MH - *Sulfhemoglobinemia/chemically induced/complications/diagnosis EDAT- 2009/03/21 09:00 MHDA- 2009/07/08 09:00 CRDT- 2009/03/21 09:00 PHST- 2009/03/21 09:00 [entrez] PHST- 2009/03/21 09:00 [pubmed] PHST- 2009/07/08 09:00 [medline] AID - 10.1097/RHU.0b013e31819db6db [doi] PST - ppublish SO - J Clin Rheumatol. 2009 Apr;15(3):127-9. doi: 10.1097/RHU.0b013e31819db6db. PMID- 16897099 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20181113 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 5 DP - 2006 Sep TI - [Systemic sclerosis--a challenge in rheumatology]. PG - 429-38; quiz 439-40 AB - The connective tissue disease systemic sclerosis (SSc) is still a challenge to every rheumatologist. SSc is characterized by progressing fibrosis of the skin and internal organs, abnormal activation of the immune system and distinct changes in microcirculation. Although it is a rare disease with a prevalence of about 20:100,000 one may come across it in daily practice. SSc is classified into limited and diffuse forms. Both entities usually involve internal organs. Life expectancy is limited and depends predominantly on the extent of the organs involved. Therefore, it is essential to diagnose SSc early and to identify and closely monitor the organs involved. FAU - Saar, P AU - Saar P AD - Abt. für Rheumatologie und klinische Immunologie, Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie der Justus-Liebig-Universität Giessen, Kerckhoff-Klinik, Bad Nauheim. p.saar@kerckhoff-klinik.de FAU - Müller-Ladner, U AU - Müller-Ladner U LA - ger PT - English Abstract PT - Journal Article TT - Die systemische Sklerose--Eine rheumatologische Herausforderung. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) RN - GNN1DV99GX (Penicillamine) SB - IM MH - Antibodies, Antinuclear/blood MH - Gastrointestinal Diseases/diagnosis/drug therapy/immunology MH - Humans MH - Hypertension, Pulmonary/diagnosis/drug therapy/immunology MH - Immunosuppressive Agents/administration & dosage/adverse effects MH - Kidney Failure, Chronic/diagnosis/drug therapy/immunology MH - Penicillamine/administration & dosage/adverse effects MH - Prognosis MH - Pulmonary Fibrosis/diagnosis/drug therapy/immunology MH - Raynaud Disease/diagnosis/drug therapy/immunology MH - Risk Factors MH - Scleroderma, Systemic/*diagnosis/drug therapy/immunology MH - Tomography, X-Ray Computed MH - Vasodilator Agents/administration & dosage/adverse effects EDAT- 2006/08/10 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/08/10 09:00 PHST- 2006/08/10 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/08/10 09:00 [entrez] AID - 10.1007/s00393-006-0076-x [doi] PST - ppublish SO - Z Rheumatol. 2006 Sep;65(5):429-38; quiz 439-40. doi: 10.1007/s00393-006-0076-x. PMID- 28347560 OWN - NLM STAT- MEDLINE DCOM- 20190425 LR - 20190425 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 144 IP - 5 DP - 2017 May TI - [Can dermoscopy be used in place of capillaroscopy?]. PG - 331-332 LID - S0151-9638(17)30109-6 [pii] LID - 10.1016/j.annder.2017.03.002 [doi] FAU - Senet, P AU - Senet P AD - Service de dermatologie, allergologie et médecine vasculaire, hôpital Tenon, AP-HP, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address: patricia.senet@aphp.fr. LA - fre PT - Editorial TT - Le dermatoscope peut-il remplacer le capillaroscope ? DEP - 20170324 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - *Dermoscopy MH - Humans MH - *Microscopic Angioscopy MH - Raynaud Disease/*pathology EDAT- 2017/03/30 06:00 MHDA- 2019/04/26 06:00 CRDT- 2017/03/29 06:00 PHST- 2017/03/30 06:00 [pubmed] PHST- 2019/04/26 06:00 [medline] PHST- 2017/03/29 06:00 [entrez] AID - S0151-9638(17)30109-6 [pii] AID - 10.1016/j.annder.2017.03.002 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2017 May;144(5):331-332. doi: 10.1016/j.annder.2017.03.002. Epub 2017 Mar 24. PMID- 22180282 OWN - NLM STAT- MEDLINE DCOM- 20120207 LR - 20260518 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 137 IP - 1-2 DP - 2012 Jan TI - [Management of digital ulcers in patients with systemic sclerosis]. PG - 34-40 LID - 10.1055/s-0031-1298798 [doi] FAU - Riemekasten, G AU - Riemekasten G AD - Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Centrum Innere Medizin und Dermatologie, Charité - Universitätsmedizin Berlin, Berlin, Germany. gabriela.riemekasten@charite.de FAU - Hoffmann, U AU - Hoffmann U FAU - Sunderkötter, C AU - Sunderkötter C FAU - Weiss, N AU - Weiss N FAU - Kuhn, A AU - Kuhn A CN - angiologisch-dermatologisch-rheumatologische DU-Expertenboard LA - ger PT - Journal Article PT - Review TT - Digitale Ulzera bei systemischer Sklerose: Diagnostik und Therapie. DEP - 20111216 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Antihypertensive Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) RN - Q326023R30 (Bosentan) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Bosentan MH - Endothelin Receptor Antagonists MH - Female MH - *Fingers/blood supply MH - Humans MH - Iloprost/therapeutic use MH - Ischemia/diagnosis/therapy MH - Male MH - Raynaud Disease/diagnosis/therapy MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/*therapy MH - Skin Ulcer/diagnosis/*therapy MH - Sulfonamides/therapeutic use MH - Vasodilator Agents/therapeutic use FIR - Ahmadi-Simab, Keihan IR - Ahmadi-Simab K FIR - Hellmich, Bernard IR - Hellmich B FIR - Bauersachs, Rupert M IR - Bauersachs RM FIR - Baumhäkel, Magnus IR - Baumhäkel M FIR - Beissert, Stefan IR - Beissert S FIR - Creutzig, Andreas IR - Creutzig A FIR - Distler, Jörg IR - Distler J FIR - Fierlbeck, Gerhard IR - Fierlbeck G FIR - Fischer-Betz, Rebecca IR - Fischer-Betz R FIR - Gaubitz, Markus IR - Gaubitz M FIR - Hein, Rüdiger IR - Hein R FIR - Hehrlein, Christoph IR - Hehrlein C FIR - Hiepe, Falk IR - Hiepe F FIR - Himsel, Andrea IR - Himsel A FIR - Hunzelmann, Nicolas IR - Hunzelmann N FIR - Kreuter, Alexander IR - Kreuter A FIR - Kuipers, Jens IR - Kuipers J FIR - Lawall, Holger IR - Lawall H FIR - Mengden, Thomas IR - Mengden T FIR - Messer, Gerald IR - Messer G FIR - Müller-Ladner, Ulf IR - Müller-Ladner U FIR - Norgauer, Johannes IR - Norgauer J FIR - Schaefer, Christian IR - Schaefer C FIR - Schröder, Frank IR - Schröder F FIR - Schulte, Karl-Ludwig IR - Schulte KL FIR - Seidel, Matthias IR - Seidel M FIR - Wenzel, Jörg IR - Wenzel J EDAT- 2011/12/20 06:00 MHDA- 2012/02/09 06:00 CRDT- 2011/12/20 06:00 PHST- 2011/12/20 06:00 [entrez] PHST- 2011/12/20 06:00 [pubmed] PHST- 2012/02/09 06:00 [medline] AID - 10.1055/s-0031-1298798 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2012 Jan;137(1-2):34-40. doi: 10.1055/s-0031-1298798. Epub 2011 Dec 16. PMID- 21166298 OWN - NLM STAT- MEDLINE DCOM- 20110317 LR - 20110422 IS - 0032-6518 (Print) IS - 0032-6518 (Linking) VI - 254 IP - 1734 DP - 2010 Nov TI - Fingers and toes. PG - 35-7 FAU - Stollery, Nigel AU - Stollery N AD - Leicester Royal Infirmary. LA - eng PT - Journal Article PL - England TA - Practitioner JT - The Practitioner JID - 0404245 SB - IM CIN - Practitioner. 2011 Mar;255(1738):31; author reply 31. PMID: 21513046 MH - Chilblains/*pathology MH - Fingers MH - Ganglion Cysts/*pathology MH - Granuloma, Pyogenic/*pathology MH - Humans MH - Medical Illustration MH - Paronychia/*pathology MH - Photography MH - Raynaud Disease/*pathology MH - Toes MH - Warts/*pathology EDAT- 2010/12/21 06:00 MHDA- 2011/03/18 06:00 CRDT- 2010/12/21 06:00 PHST- 2010/12/21 06:00 [entrez] PHST- 2010/12/21 06:00 [pubmed] PHST- 2011/03/18 06:00 [medline] PST - ppublish SO - Practitioner. 2010 Nov;254(1734):35-7. PMID- 30824643 OWN - NLM STAT- MEDLINE DCOM- 20200921 LR - 20260127 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 10 DP - 2019 Oct TI - Factors Influencing Raynaud Condition Score Diary Outcomes in Systemic Sclerosis. PG - 1326-1334 LID - 10.3899/jrheum.180818 [doi] AB - OBJECTIVE: Raynaud phenomenon (RP) in systemic sclerosis (SSc) could be influenced by clinical phenotype, environmental factors (e.g., season), and personal factors (e.g., coping strategies and ill-health perceptions). We studied the relative influence of a range of putative factors affecting patient-reported assessment of SSc-RP severity. METHODS: SSc patients were enrolled at UK and US sites. Participants completed the 2-week Raynaud Condition Score (RCS) diary alongside collection of patient demographics, clinical phenotype, the Coping Strategies Questionnaire, Pain Catastrophizing Scale, Scleroderma Health Assessment Questionnaire (SHAQ), and both patient/physician visual analog scale (VAS) assessments for RP, digital ulcer disease, and global disease. Environmental temperature data were obtained at each site. A second RCS diary was completed 6 months after enrollment. RESULTS: We enrolled 107 patients (baseline questionnaires returned by 94). There were significant associations between RCS diary variables and both catastrophizing and coping strategies. There were significant associations between RCS diary outcomes and both environmental temperature and season of enrollment. Age, disease duration, sex, disease subtype, smoking, and vasodilator use were not associated with RCS diary outcomes. The best-fitting multivariate model identified the patient RP VAS, SHAQ pain VAS, and SHAQ gastrointestinal VAS subscales as the strongest independent predictors of the RCS. CONCLUSION: Patient-reported assessment of SSc-RP severity is associated with a number of factors including pain, catastrophizing, and coping strategies. The effects of seasonal variation in environmental temperature on SSc-RP burden has implications for clinical trial design. Treatments targeting SSc-RP pain and the development of behavioral interventions enhancing coping strategies may reduce the burden of SSc-RP. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - From the Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); Department of Pharmacy and Pharmacology, University of Bath; Department of Mathematical Sciences, University of Bath, Bath, UK; University of Utah, and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City, Utah, USA. JohnPauling@nhs.net. AD - J.D. Pauling, BMedSci, BMBS, PhD, FRCP, Senior Lecturer, Consultant Rheumatologist, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath; E. Reilly, MBBCh, MRCP, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); T. Smith, BA, BSc, PhD, Department of Mathematical Sciences, University of Bath; T.M. Frech, MD, MS, University of Utah, and Salt Lake Regional Veterans Affairs Medical Center. JohnPauling@nhs.net. FAU - Reilly, Elizabeth AU - Reilly E AD - From the Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); Department of Pharmacy and Pharmacology, University of Bath; Department of Mathematical Sciences, University of Bath, Bath, UK; University of Utah, and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City, Utah, USA. AD - J.D. Pauling, BMedSci, BMBS, PhD, FRCP, Senior Lecturer, Consultant Rheumatologist, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath; E. Reilly, MBBCh, MRCP, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); T. Smith, BA, BSc, PhD, Department of Mathematical Sciences, University of Bath; T.M. Frech, MD, MS, University of Utah, and Salt Lake Regional Veterans Affairs Medical Center. FAU - Smith, Theresa AU - Smith T AD - From the Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); Department of Pharmacy and Pharmacology, University of Bath; Department of Mathematical Sciences, University of Bath, Bath, UK; University of Utah, and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City, Utah, USA. AD - J.D. Pauling, BMedSci, BMBS, PhD, FRCP, Senior Lecturer, Consultant Rheumatologist, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath; E. Reilly, MBBCh, MRCP, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); T. Smith, BA, BSc, PhD, Department of Mathematical Sciences, University of Bath; T.M. Frech, MD, MS, University of Utah, and Salt Lake Regional Veterans Affairs Medical Center. FAU - Frech, Tracy M AU - Frech TM AD - From the Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); Department of Pharmacy and Pharmacology, University of Bath; Department of Mathematical Sciences, University of Bath, Bath, UK; University of Utah, and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City, Utah, USA. AD - J.D. Pauling, BMedSci, BMBS, PhD, FRCP, Senior Lecturer, Consultant Rheumatologist, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath; E. Reilly, MBBCh, MRCP, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals); T. Smith, BA, BSc, PhD, Department of Mathematical Sciences, University of Bath; T.M. Frech, MD, MS, University of Utah, and Salt Lake Regional Veterans Affairs Medical Center. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190301 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Vasodilator Agents) SB - IM CIN - J Rheumatol. 2019 Nov;46(11):1544-1545. doi: 10.3899/jrheum.190565. PMID: 31308205 CIN - J Rheumatol. 2019 Nov;46(11):1543-1544. doi: 10.3899/jrheum.190463. PMID: 31308207 MH - *Adaptation, Psychological MH - Adult MH - Aged MH - Catastrophization/*epidemiology MH - Cities/epidemiology MH - Cohort Studies MH - Comorbidity MH - England/epidemiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Morbidity MH - Pain/*epidemiology MH - Raynaud Disease/drug therapy/*epidemiology MH - Scleroderma, Systemic/*epidemiology MH - Seasons MH - *Severity of Illness Index MH - Surveys and Questionnaires MH - Temperature MH - Utah/epidemiology MH - Vasodilator Agents/therapeutic use OTO - NOTNLM OT - CLINICAL TRIALS OT - OUTCOME MEASURES OT - PATIENT-REPORTED OUTCOMES OT - RAYNAUD PHENOMENON OT - SYSTEMIC SCLEROSIS OT - VALIDATION STUDIES EDAT- 2019/03/03 06:00 MHDA- 2020/09/22 06:00 CRDT- 2019/03/03 06:00 PHST- 2018/11/12 00:00 [accepted] PHST- 2019/03/03 06:00 [pubmed] PHST- 2020/09/22 06:00 [medline] PHST- 2019/03/03 06:00 [entrez] AID - jrheum.180818 [pii] AID - 10.3899/jrheum.180818 [doi] PST - ppublish SO - J Rheumatol. 2019 Oct;46(10):1326-1334. doi: 10.3899/jrheum.180818. Epub 2019 Mar 1. PMID- 32244019 OWN - NLM STAT- MEDLINE DCOM- 20210414 LR - 20210414 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 83 IP - 6 DP - 2020 Dec TI - Reply to: "Interdigital injection of botulinum toxin for patients with Raynaud phenomenon". PG - e409-e410 LID - S0190-9622(20)30488-6 [pii] LID - 10.1016/j.jaad.2020.02.085 [doi] FAU - Jaloux, Charlotte AU - Jaloux C AD - Department of Hand Surgery and Plastic and Reconstructive Surgery of the Limbs, La Timone University Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France. Electronic address: drcharlottejaloux@gmail.com. FAU - Kachouh, Najib AU - Kachouh N AD - Department of Hand Surgery and Plastic and Reconstructive Surgery of the Limbs, La Timone University Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France. FAU - Legré, Régis AU - Legré R AD - Department of Hand Surgery and Plastic and Reconstructive Surgery of the Limbs, La Timone University Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France. FAU - Amatore, Florent AU - Amatore F AD - Department of Dermatology, La Timone University Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France. FAU - Mayoly, Alice AU - Mayoly A AD - Department of Hand Surgery and Plastic and Reconstructive Surgery of the Limbs, La Timone University Hospital, Assistance Publique Hôpitaux de Marseille, Marseille, France. LA - eng PT - Comment PT - Letter DEP - 20200331 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM CON - J Am Acad Dermatol. 2020 Dec;83(6):e399. doi: 10.1016/j.jaad.2019.11.060. PMID: 31821859 CIN - J Am Acad Dermatol. 2020 Dec;83(6):e411. doi: 10.1016/j.jaad.2020.03.066. PMID: 32244020 MH - *Botulinum Toxins/therapeutic use MH - Humans MH - Injections MH - *Neuromuscular Agents/therapeutic use MH - *Raynaud Disease/drug therapy EDAT- 2020/04/04 06:00 MHDA- 2021/04/15 06:00 CRDT- 2020/04/04 06:00 PHST- 2020/02/13 00:00 [received] PHST- 2020/02/20 00:00 [revised] PHST- 2020/02/25 00:00 [accepted] PHST- 2020/04/04 06:00 [pubmed] PHST- 2021/04/15 06:00 [medline] PHST- 2020/04/04 06:00 [entrez] AID - S0190-9622(20)30488-6 [pii] AID - 10.1016/j.jaad.2020.02.085 [doi] PST - ppublish SO - J Am Acad Dermatol. 2020 Dec;83(6):e409-e410. doi: 10.1016/j.jaad.2020.02.085. Epub 2020 Mar 31. PMID- 27357717 OWN - NLM STAT- MEDLINE DCOM- 20170213 LR - 20260127 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 35 IP - 8 DP - 2016 Aug TI - Nailfold capillaroscopy assessment of microcirculation abnormalities and endothelial dysfunction in children with primary or secondary Raynaud syndrome. PG - 1993-2001 LID - 10.1007/s10067-016-3340-8 [doi] AB - Raynaud syndrome (RS) manifests as episodes of transient spasms of peripheral blood vessels, most often in response to cold. The reason of that symptom (primary RS (pRS)) usually cannot be found but may be accompanied by some autoimmune diseases (secondary RS (sRS)). In this study, we assessed microcapillary status and serum concentrations of chosen cytokines, adhesive molecules, and nitric oxide (NO) in patients with pRS and sRS in comparison with healthy children. Eighty-six patients with RS were enrolled into the study, including 52 with pRS and 34 with sRS. The control group consisted of 29 healthy children. A decrease in myorelaxative and anticoagulant abilities was observed, with simultaneous prevalence of vasopressor substances and procoagulative activity. Therefore, several important factors such as endothelin-1 (ET-1), E-selectin (E-sel), interleukin-18 (IL-18), and nitrogen oxide (NO) were also analyzed. Two types of capillaroscopy status were determined: normal and microangiopathic. There was a significant relationship between presence of microangiopathy and higher serum ET-1 (p = 0.018) and E-sel (p = 0.021) levels. Similarly, we have found a correlation between presence of ANA and higher ET-1 (p = 0.005), but not E-sel (p = 0.241). In patients with pRS, we found significant relationship between ANA and higher ET-1 (p = 0.008). No such relations were observed in sRS patients. Our data indicates that external factor-induced vasoconstrictive effects dominated in pRS, whereas in sRS in the course of connective tissue diseases, it was accompanied by coexistent vasodilation due to endothelial dysfunction. The latter phenomenon is at least partially dependent on insufficient NO release. FAU - Latuskiewicz-Potemska, Joanna AU - Latuskiewicz-Potemska J AD - Department of Pediatric Rheumatology, Medical University of Lodz, 36/50 Sporna St, 91-738, Lodz, Poland. FAU - Chmura-Skirlinska, Antonina AU - Chmura-Skirlinska A AD - Laboratory of EPR Spectroscopy, Jagiellonian Centre for Experimental Therapeutic, Jagiellonian University in Krakow, 14 Bobrzynskiego St, 30-348, Krakow, Poland. FAU - Gurbiel, Ryszard J AU - Gurbiel RJ AD - Laboratory of EPR Spectroscopy, Jagiellonian Centre for Experimental Therapeutic, Jagiellonian University in Krakow, 14 Bobrzynskiego St, 30-348, Krakow, Poland. AD - Department of Molecular Biophysics, Faculty of Biochemistry, Biophysics, and Biotechnology, Jagiellonian University in Krakow, 7 Gronostajowa St, 30-387, Krakow, Poland. FAU - Smolewska, Elzbieta AU - Smolewska E AD - Department of Pediatric Rheumatology, Medical University of Lodz, 36/50 Sporna St, 91-738, Lodz, Poland. e.smolewska@wp.pl. LA - eng PT - Journal Article DEP - 20160629 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (E-Selectin) RN - 0 (Endothelin-1) RN - 0 (Interleukin-18) RN - 0 (Nitrogen Oxides) SB - IM MH - Adolescent MH - Case-Control Studies MH - Child MH - Connective Tissue Diseases/*physiopathology MH - E-Selectin/blood MH - Endothelin-1/blood MH - Endothelium/*physiopathology MH - Female MH - Humans MH - Interleukin-18/blood MH - Male MH - *Microcirculation MH - Microscopic Angioscopy MH - Nails/*blood supply MH - Nitrogen Oxides/blood MH - Poland MH - Raynaud Disease/diagnosis/*physiopathology OTO - NOTNLM OT - Capillaroscopy OT - Connective tissue disease OT - Endothelium OT - Microcirculation OT - Raynaud syndrome EDAT- 2016/07/01 06:00 MHDA- 2017/02/14 06:00 CRDT- 2016/07/01 06:00 PHST- 2016/06/07 00:00 [received] PHST- 2016/06/21 00:00 [accepted] PHST- 2016/06/19 00:00 [revised] PHST- 2016/07/01 06:00 [entrez] PHST- 2016/07/01 06:00 [pubmed] PHST- 2017/02/14 06:00 [medline] AID - 10.1007/s10067-016-3340-8 [pii] AID - 10.1007/s10067-016-3340-8 [doi] PST - ppublish SO - Clin Rheumatol. 2016 Aug;35(8):1993-2001. doi: 10.1007/s10067-016-3340-8. Epub 2016 Jun 29. PMID- 30776810 OWN - NLM STAT- MEDLINE DCOM- 20191206 LR - 20191217 IS - 1539-3704 (Electronic) IS - 0003-4819 (Linking) VI - 170 IP - 4 DP - 2019 Feb 19 TI - In Raynaud phenomenon, on-demand sildenafil did not reduce disability or frequency or duration of attacks. PG - JC21 LID - 10.7326/ACPJ201902190-021 [doi] FAU - Mirza, Reza AU - Mirza R FAU - Guyatt, Gordon AU - Guyatt G LA - eng PT - Comment PT - Journal Article PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM CON - Ann Intern Med. 2018 Nov 20;169(10):694-703. doi: 10.7326/M18-0517. PMID: 30383134 MH - Humans MH - *Raynaud Disease MH - Sildenafil Citrate MH - Vasodilator Agents EDAT- 2019/02/19 06:00 MHDA- 2019/12/18 06:00 CRDT- 2019/02/19 06:00 PHST- 2019/02/19 06:00 [entrez] PHST- 2019/02/19 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] AID - 2725109 [pii] AID - 10.7326/ACPJ201902190-021 [doi] PST - ppublish SO - Ann Intern Med. 2019 Feb 19;170(4):JC21. doi: 10.7326/ACPJ201902190-021. PMID- 19379655 OWN - NLM STAT- MEDLINE DCOM- 20090624 LR - 20131121 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 15 IP - 3 DP - 2009 Mar 15 TI - Success of prednisone in the treatment of a patient with sarcoidosis and systemic sclerosis: Report of a case. PG - 11 AB - We report a case of a man with sarcoidosis and diffuse systemic sclerosis who showed improvement of both diseases after prednisone therapy and remains asymptomatic after three years of follow-up. The association of these two diseases is rare and it is controversial whether this is a chance association or if there is a relationship between the two autoimmune diseases. Despite the poor prognosis and response to therapy of the patients with diffuse systemic sclerosis, our patient had a marked improvement of the disease with prednisone. FAU - Borges da Costa, João AU - Borges da Costa J AD - Clínica Universitária de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal. joaobc77@hotmail.com FAU - Mayer-da-Silva, Augusto AU - Mayer-da-Silva A FAU - Soares de Almeida, Luís Miguel AU - Soares de Almeida LM FAU - Marques Gomes, Manuel AU - Marques Gomes M LA - eng PT - Case Reports PT - Journal Article DEP - 20090315 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Immunosuppressive Agents) RN - VB0R961HZT (Prednisone) SB - IM MH - Autoimmune Diseases/*drug therapy/pathology MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Male MH - Middle Aged MH - Prednisone/*therapeutic use MH - Prognosis MH - Raynaud Disease/drug therapy/etiology MH - Remission Induction MH - Sarcoidosis/complications/*drug therapy/pathology MH - Scleroderma, Systemic/complications/*drug therapy/pathology EDAT- 2009/04/22 09:00 MHDA- 2009/06/25 09:00 CRDT- 2009/04/22 09:00 PHST- 2009/04/22 09:00 [entrez] PHST- 2009/04/22 09:00 [pubmed] PHST- 2009/06/25 09:00 [medline] PST - epublish SO - Dermatol Online J. 2009 Mar 15;15(3):11. PMID- 21960037 OWN - NLM STAT- MEDLINE DCOM- 20120120 LR - 20110930 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 23 IP - 6 DP - 2011 Nov TI - Current world literature. PG - 620-5 LID - 10.1097/BOR.0b013e32834ccef6 [doi] LA - eng PT - Bibliography PT - Journal Article PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Animals MH - Disease Models, Animal MH - Heart Diseases/etiology MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - Hypertension, Pulmonary/etiology MH - Muscular Diseases/etiology MH - Neoplasms/complications MH - Raynaud Disease/therapy MH - *Scleroderma, Systemic/complications/therapy EDAT- 2011/10/01 06:00 MHDA- 2012/01/21 06:00 CRDT- 2011/10/01 06:00 PHST- 2011/10/01 06:00 [entrez] PHST- 2011/10/01 06:00 [pubmed] PHST- 2012/01/21 06:00 [medline] AID - 00002281-201111000-00017 [pii] AID - 10.1097/BOR.0b013e32834ccef6 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2011 Nov;23(6):620-5. doi: 10.1097/BOR.0b013e32834ccef6. PMID- 17497666 OWN - NLM STAT- MEDLINE DCOM- 20070629 LR - 20070517 IS - 8755-1039 (Print) IS - 1097-0339 (Linking) VI - 35 IP - 6 DP - 2007 Jun TI - Chaetomium fungal elements in a conventional vaginal smear. PG - 376-8 FAU - Hitchcock, Thomas AU - Hitchcock T FAU - Avant, Cameron AU - Avant C FAU - Colello, Cheryl AU - Colello C FAU - Hoda, Rana S AU - Hoda RS LA - eng PT - Case Reports PT - Letter PL - United States TA - Diagn Cytopathol JT - Diagnostic cytopathology JID - 8506895 SB - IM MH - Chaetomium/*isolation & purification MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Mycoses/*diagnosis MH - Raynaud Disease/microbiology MH - *Vaginal Smears EDAT- 2007/05/15 09:00 MHDA- 2007/06/30 09:00 CRDT- 2007/05/15 09:00 PHST- 2007/05/15 09:00 [pubmed] PHST- 2007/06/30 09:00 [medline] PHST- 2007/05/15 09:00 [entrez] AID - 10.1002/dc.20644 [doi] PST - ppublish SO - Diagn Cytopathol. 2007 Jun;35(6):376-8. doi: 10.1002/dc.20644. PMID- 30677800 OWN - NLM STAT- MEDLINE DCOM- 20190514 LR - 20190514 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 24 IP - 12 DP - 2018 Dec 15 TI - Botulinum toxin for treatment of Raynaud phenomenon in CREST syndrome. LID - 13030/qt9zr1318w [pii] AB - Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is a form of a rare, clinical subtype of systemic sclerosis, known as limited systemic sclerosis. Limited systemic sclerosis, including CREST syndrome, manifests as fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement. We present a case of a 75-year-old woman with a longstanding history of CREST syndrome complicated by a digital ulceration and persistent pain associated with recalcitrant Raynaud phenomenon. After failing a number of first-line pharmacologic therapies such as diltiazem, sildenafil, and topical nitropaste, the patient was started on a trial of botulinum toxin for the left second digit, with 10 unit injections into both webspaces for a total of 20 units. Following injection, the patient reported no further baseline pain in the affected finger and an over fifty-percent improvement in discomfort with manipulation of the digit at a follow-up time of one week. The ulceration started healing within the following three weeks. This result was maintained at a follow-up time of six weeks. FAU - Berk-Krauss, Juliana AU - Berk-Krauss J AD - The Ronald O. Perelman Department of Dermatology, New York University Langone Health, New York, New York Yale University School of Medicine, New Haven, Connecticut. jberkkrauss@gmail.com. FAU - Christman, Mitalee P AU - Christman MP FAU - Franks, Andrew AU - Franks A FAU - Sicco, Kristen Lo AU - Sicco KL FAU - Liebman, Tracey N AU - Liebman TN LA - eng PT - Case Reports PT - Journal Article DEP - 20181215 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Acetylcholine Release Inhibitors) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - EC 3.4.24.69 (Botulinum Toxins) RN - EE92BBP03H (Diltiazem) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Acetylcholine Release Inhibitors/*therapeutic use MH - Administration, Topical MH - Aged MH - Botulinum Toxins/*therapeutic use MH - CREST Syndrome/drug therapy MH - Diltiazem/therapeutic use MH - Female MH - Humans MH - Nitroglycerin/therapeutic use MH - Pain/etiology MH - Raynaud Disease/complications/*drug therapy MH - Sildenafil Citrate/therapeutic use MH - Treatment Failure MH - Ulcer/etiology MH - Vasodilator Agents/therapeutic use EDAT- 2019/01/27 06:00 MHDA- 2019/05/15 06:00 CRDT- 2019/01/25 06:00 PHST- 2019/01/21 00:00 [received] PHST- 2019/01/21 00:00 [accepted] PHST- 2019/01/25 06:00 [entrez] PHST- 2019/01/27 06:00 [pubmed] PHST- 2019/05/15 06:00 [medline] AID - 13030/qt9zr1318w [pii] PST - epublish SO - Dermatol Online J. 2018 Dec 15;24(12):13030/qt9zr1318w. PMID- 26935364 OWN - NLM STAT- MEDLINE DCOM- 20160829 LR - 20181202 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 55 IP - 5 DP - 2016 TI - Thermal Disparity between Fingers after Cold-water Immersion of Hands: A Useful Indicator of Disturbed Peripheral Circulation in Raynaud Phenomenon Patients. PG - 461-6 LID - 10.2169/internalmedicine.55.5218 [doi] AB - OBJECTIVE: To devise an effective method to assess the peripheral circulation using an infrared thermographic analysis. METHODS: Sequential measurements of the skin temperature before and after cold-water immersion of the hands were analyzed by a thermographic examination in healthy controls and patients diagnosed to have Raynaud phenomenon (RP). The skin temperatures of the dorsum of all fingernail folds and the metacarpophalangeal (MCP) joints were measured at baseline. Then the hands were immersed in 10°C water for 10 s, and the skin temperatures were measured at 0, 3, 5, 10, 15, 20 and 30 min after immersion. The mean temperature, recovery rate and disparity (coefficient of variation) of the nail fold temperatures were calculated. The distal-dorsal difference (DDD) was calculated by subtracting the mean MCP temperature from the mean nail fold temperature. Receiver operating characteristic (ROC) curves were generated to compare these parameters in terms of their capability to differentiate patients with RP. RESULTS: Thirty-one RP patients and 25 controls were included in the study. The baseline nail fold temperature was significantly lower in RP patients than in the controls. The RP patients had a lower recovery rate, lower DDD and higher disparity than the controls. The disparity and DDD were negatively correlated (r=-0.63, p<0.01), whereas the recovery rate and DDD were positively correlated (r=0.91, p<0.01). The ROC curve analysis revealed that the disparity in nail fold temperature effectively differentiated RP patients from controls (area under the curve: recovery rate 0.72; disparity 0.88; DDD 0.79). CONCLUSION: The temperature disparity between fingers is a useful thermographic parameter for evaluating disturbed peripheral circulation in patients with Raynaud phenomenon. FAU - Horikoshi, Masanobu AU - Horikoshi M AD - Department of Allergy and Rheumatic Diseases, Japanese Red Cross Medical Center, Japan. FAU - Inokuma, Shigeko AU - Inokuma S FAU - Kijima, Yasuo AU - Kijima Y FAU - Kobuna, Mika AU - Kobuna M FAU - Miura, Yoko AU - Miura Y FAU - Okada, Rika AU - Okada R FAU - Kobayashi, Shoko AU - Kobayashi S LA - eng PT - Journal Article DEP - 20160301 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 059QF0KO0R (Water) SB - IM MH - Adult MH - Blood Circulation MH - *Cold Temperature MH - Female MH - Fingers/*blood supply MH - Hand/blood supply MH - Humans MH - Middle Aged MH - Predictive Value of Tests MH - ROC Curve MH - Raynaud Disease/*diagnosis/physiopathology MH - Retrospective Studies MH - Skin Temperature MH - Thermography MH - Water EDAT- 2016/03/05 06:00 MHDA- 2016/08/30 06:00 CRDT- 2016/03/04 06:00 PHST- 2016/03/04 06:00 [entrez] PHST- 2016/03/05 06:00 [pubmed] PHST- 2016/08/30 06:00 [medline] AID - 10.2169/internalmedicine.55.5218 [doi] PST - ppublish SO - Intern Med. 2016;55(5):461-6. doi: 10.2169/internalmedicine.55.5218. Epub 2016 Mar 1. PMID- 22371406 OWN - NLM STAT- MEDLINE DCOM- 20130508 LR - 20181201 IS - 1732-2693 (Electronic) IS - 0032-5449 (Linking) VI - 66 DP - 2012 Jan 30 TI - Diabetic microangiopathy in capillaroscopic examination of juveniles with diabetes type 1. PG - 51-9 AB - INTRODUCTION: The aim of this work was a quantitative and qualitative assessment of a selected part of the microcirculation in children with diabetes type 1 using videocapillaroscopy technique. MATERIAL/METHODS: The authors tested a group consisting of 145 children (70 boys, 75 girls) diagnosed and treated for diabetes type 1 in the Diabetic Clinic of GCZD in Katowice for at least one year. The study included history, clinical examination (including dermatological examination) and videocapillaroscopy. Capillaroscopy, a non-invasive, painless and easily repeatable test, was performed using videocapillaroscopy with digital storage of the obtained images. All nailfolds were examined in all children using videocapillaroscopy, and the obtained images were assessed quantitatively and qualitatively for changes in capillary loops in the tested children according to the defined diagnostic procedure. RESULTS: The analysis of capillaroscopic images described selected quantitative and qualitative characteristics. The conducted analysis showed an increase in the number of capillaries and their elongation, the presence of megacapillaries and Raynaud loops, which were accompanied by an intensive red background, indicating possible neoangiogenesis. The increase in the number of capillaries, disturbances in distribution of capillaries and the presence of abnormal capillaries were correlated with the longer duration of diabetes. Raynaud loops were more frequently found in the cases of increased mean values of HbA1c. Higher values of HbA1c influenced the capillaroscopic images, mainly the number of vessels, including Raynaud loops. CONCLUSIONS: Videocapillaroscopy technique could be a useful tool to detect the early changes of microangiopathy in children with diabetes type 1. FAU - Kaminska-Winciorek, Grażyna AU - Kaminska-Winciorek G AD - Department of Dermatology, Silesian Medical University, Katowice. dermatolog.pl@gmail.com FAU - Deja, Grażyna AU - Deja G FAU - Polańska, Joanna AU - Polańska J FAU - Jarosz-Chobot, Przemysława AU - Jarosz-Chobot P LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120130 PL - Poland TA - Postepy Hig Med Dosw (Online) JT - Postepy higieny i medycyny doswiadczalnej (Online) JID - 101206517 SB - IM MH - Adolescent MH - Capillaries/*pathology/*physiopathology MH - Child MH - Child, Preschool MH - Diabetes Mellitus, Type 1/*pathology/*physiopathology MH - Diabetic Angiopathies/*pathology/*physiopathology MH - Female MH - Humans MH - Male MH - Microcirculation MH - Microscopic Angioscopy/methods MH - Microscopy, Video MH - Neovascularization, Pathologic/pathology/physiopathology MH - Raynaud Disease/pathology/physiopathology EDAT- 2012/03/01 06:00 MHDA- 2013/05/09 06:00 CRDT- 2012/02/29 06:00 PHST- 2012/02/29 06:00 [entrez] PHST- 2012/03/01 06:00 [pubmed] PHST- 2013/05/09 06:00 [medline] AID - 979395 [pii] PST - epublish SO - Postepy Hig Med Dosw (Online). 2012 Jan 30;66:51-9. PMID- 37978357 OWN - NLM STAT- MEDLINE DCOM- 20231127 LR - 20240112 IS - 1471-2431 (Electronic) IS - 1471-2431 (Linking) VI - 23 IP - 1 DP - 2023 Nov 17 TI - Kaposiform hemangioendothelioma presented with raynaud phenomenon: a case report. PG - 574 LID - 10.1186/s12887-023-04407-1 [doi] LID - 574 AB - BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm affecting infants or young children. KHE includes a spectrum of lesions, ranging from small and superficial tumors to large and invasive lesions with Kasabach-Merritt phenomenon (KMP). Currently, no published studies have reported a KHE presenting as thrombocytopenia and Raynaud phenomenon. CASE PRESENTATION: A 2-year-old boy with right hand swelling and thrombocytopenia was admitted to our hospital. His right hand turned swelling and red, even occasionally cyanotic. This condition became worse in response to cool environments and improved with warming, and platelet counts were between 50 ~ 80 × 10^9/L. Physical examination on admission revealed the swelling and frostbite-like rash of the right-hand fingers, and the skin temperature of the right hand was lower than the left. On day 3 of admission, chest CT results showed an irregular mass on the right side of the spine. The puncture biopsy demonstrated positive CD31, D2-40, and FLI1 immunohistochemical staining, but negative GLUT1 staining, confirming the diagnosis of KHE. Furthermore, endothelin-1 (ET1) expression levels significantly increased, and eNOS and A20 expression levels significantly decreased comparing with control patients. The patient received methylprednisolone and sirolimus treatments, and his condition gradually improved during the follow-up. CONCLUSIONS: We reported the first case of KHE presenting with thrombocytopenia and Raynaud phenomenon. The development of Raynaud phenomenon could be associated with increased ET-1 and reduced eNOS and A20 expressions. Careful differential diagnosis of hidden KHE should be considered in children with thrombocytopenia and Raynaud phenomenon. CI - © 2023. The Author(s). FAU - Liu, Lingke AU - Liu L AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. AD - Pediatrics, The Affiliated Hospital of Shaoxing University, Shaoxing, 312000, PR China. FAU - Gu, Weizhong AU - Gu W AD - Pathology, National Clinical Research Center for Child Health, Hangzhou, 310003, PR China. FAU - Teng, Liping AU - Teng L AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. FAU - Xu, Yiping AU - Xu Y AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. FAU - Zheng, Fei AU - Zheng F AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. FAU - Hu, Minfei AU - Hu M AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. FAU - Lu, Meiping AU - Lu M AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. FAU - Xu, Xuefeng AU - Xu X AD - Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, P.R. China. xuxuefeng@zju.edu.cn. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231117 PL - England TA - BMC Pediatr JT - BMC pediatrics JID - 100967804 RN - Kaposiform Hemangioendothelioma SB - IM MH - Infant MH - Child MH - Male MH - Humans MH - Child, Preschool MH - *Kasabach-Merritt Syndrome/complications/diagnosis/pathology MH - *Hemangioendothelioma/complications/diagnosis/pathology MH - *Sarcoma, Kaposi/complications/diagnosis/pathology MH - *Raynaud Disease/complications/diagnosis PMC - PMC10655467 OTO - NOTNLM OT - Case report OT - Children OT - Kaposiform hemangioendothelioma OT - Raynaud phenomenon OT - Thrombocytopenia COIS- The authors declare no conflict of interests. EDAT- 2023/11/18 11:45 MHDA- 2023/11/27 12:43 PMCR- 2023/11/17 CRDT- 2023/11/18 01:36 PHST- 2023/01/24 00:00 [received] PHST- 2023/11/06 00:00 [accepted] PHST- 2023/11/27 12:43 [medline] PHST- 2023/11/18 11:45 [pubmed] PHST- 2023/11/18 01:36 [entrez] PHST- 2023/11/17 00:00 [pmc-release] AID - 10.1186/s12887-023-04407-1 [pii] AID - 4407 [pii] AID - 10.1186/s12887-023-04407-1 [doi] PST - epublish SO - BMC Pediatr. 2023 Nov 17;23(1):574. doi: 10.1186/s12887-023-04407-1. PMID- 17674117 OWN - NLM STAT- MEDLINE DCOM- 20080114 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 11 DP - 2007 Nov TI - Chronic myositis with cardiomyopathy and respiratory failure associated with mild form of organ-specific autoimmune diseases. PG - 1917-9 AB - We report the four patients with chronic myositis characterized by a very slow progression with cardiomyopathy and frequently with marked respiratory muscle weakness associated with other organ-specific autoimmune diseases such as primary biliary cirrhosis. The histopathology of the muscle showed many degenerative and regenerative fibers, but inflammatory-cell infiltration were minimal. The patients showed favorable response to high-dose corticosteroid treatment. Because of these clinical features, these patients are sometimes misdiagnosed as muscular dystrophy and not treated properly. It is important to distinguish this type of treatable myositis. FAU - Tanaka, K AU - Tanaka K AD - Department of Neurology, Brain Research Institute, Niigata University, Niigata City, Niigata, 951-8585, Japan. keiko@bri.niigata-u.ac.jp FAU - Sato, A AU - Sato A FAU - Kasuga, K AU - Kasuga K FAU - Kanazawa, M AU - Kanazawa M FAU - Yanagawa, K AU - Yanagawa K FAU - Umeda, M AU - Umeda M FAU - Tada, M AU - Tada M FAU - Tanaka, M AU - Tanaka M FAU - Nishizawa, M AU - Nishizawa M LA - eng PT - Case Reports PT - Journal Article DEP - 20070803 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Autoimmune Diseases/*complications/diagnosis MH - Cardiomyopathies/*complications/diagnosis MH - Chronic Disease MH - Disease Progression MH - Fatal Outcome MH - Female MH - Humans MH - Middle Aged MH - Muscles/pathology MH - Myositis/*complications/diagnosis/mortality/*pathology MH - Raynaud Disease/complications/diagnosis MH - Remission Induction MH - Respiratory Insufficiency/*complications/diagnosis MH - Scleroderma, Systemic/complications/diagnosis MH - Treatment Outcome EDAT- 2007/08/04 09:00 MHDA- 2008/01/15 09:00 CRDT- 2007/08/04 09:00 PHST- 2007/06/15 00:00 [received] PHST- 2007/06/27 00:00 [accepted] PHST- 2007/06/25 00:00 [revised] PHST- 2007/08/04 09:00 [pubmed] PHST- 2008/01/15 09:00 [medline] PHST- 2007/08/04 09:00 [entrez] AID - 10.1007/s10067-007-0698-7 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Nov;26(11):1917-9. doi: 10.1007/s10067-007-0698-7. Epub 2007 Aug 3. PMID- 22571581 OWN - NLM STAT- MEDLINE DCOM- 20121030 LR - 20181201 IS - 1440-0960 (Electronic) IS - 0004-8380 (Linking) VI - 53 IP - 2 DP - 2012 May TI - Endothelin receptor antagonist bosentan improves the dermal sclerosis in a patient with systemic sclerosis. PG - e32-3 LID - 10.1111/j.1440-0960.2010.00719.x [doi] AB - We report a case of systemic sclerosis with interstitial pneumonia and severe pulmonary hypertension treated with bosentan, an endothelin receptor antagonist. Within a short period of administration of the bosentan the skin sclerosis improved. CI - © 2010 The Authors. Australasian Journal of Dermatology © 2010 The Australasian College of Dermatologists. FAU - Nishibu, Akiko AU - Nishibu A AD - Department of Dermatology, Fukushima Medical University, Fukushima, Japan. anishibu@kanazawa-med.ac.jp FAU - Sakai, Erika AU - Sakai E FAU - Oyama, Noritaka AU - Oyama N FAU - Yamamoto, Toshiyuki AU - Yamamoto T LA - eng PT - Case Reports PT - Journal Article DEP - 20101129 PL - Australia TA - Australas J Dermatol JT - The Australasian journal of dermatology JID - 0135232 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Anti-Inflammatory Agents/therapeutic use MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Humans MH - Hypertension, Pulmonary/complications/*drug therapy MH - Lung Diseases, Interstitial/complications/drug therapy MH - Male MH - Methylprednisolone/therapeutic use MH - Middle Aged MH - Raynaud Disease/complications/drug therapy MH - Scleroderma, Systemic/complications/*drug therapy MH - Sulfonamides/*therapeutic use EDAT- 2012/05/11 06:00 MHDA- 2012/10/31 06:00 CRDT- 2012/05/11 06:00 PHST- 2012/05/11 06:00 [entrez] PHST- 2012/05/11 06:00 [pubmed] PHST- 2012/10/31 06:00 [medline] AID - 10.1111/j.1440-0960.2010.00719.x [doi] PST - ppublish SO - Australas J Dermatol. 2012 May;53(2):e32-3. doi: 10.1111/j.1440-0960.2010.00719.x. Epub 2010 Nov 29. PMID- 24689095 OWN - NLM STAT- MEDLINE DCOM- 20150409 LR - 20140401 IS - 1842-1121 (Electronic) IS - 1841-8724 (Linking) VI - 23 IP - 1 DP - 2014 Mar TI - Nailfold capillaroscopy in primary biliary cirrhosis: a useful tool for the early diagnosis of scleroderma. PG - 39-43 AB - BACKGROUND & AIM: Primary biliary cirrhosis (PBC) is frequently associated with other autoimmune diseases, including systemic sclerosis (SSc). In the last years many efforts have been dedicated to the research of widely accepted criteria for the early diagnosis of SSc. Since studies on the prevalence of early SSc in PBC patients are lacking, our aim was to investigate its hitherto unknown prevalence in a large cohort of PBC patients. METHODS: We studied 80 PBC patients and 72 patients with other chronic liver diseases. Diagnostic workup included research into signs of connective tissue disease, determination of autoantibody profile, and examination of capillary abnormalities through nailfold videocapillaroscopy. RESULTS: Ten PBC patients (12.5%) satisfied diagnostic criteria for early SSc and 5 (6.3%) had definite SSc. None of the patients in the control group were diagnosed either with early or definite SSc. No differences were observed in terms of aminotransferases, alkaline phosphatase, and liver function tests between PBC patients with and without associated SSc. CONCLUSIONS: Early SSc is significantly frequent in PBC patients. The detection of early SSc in PBC patients may lead to a prompt treatment of its complications, preventing inabilities and preserving the chance of liver transplantation. FAU - Tovoli, Francesco AU - Tovoli F AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. francesco.tovoli@studio.unibo.it. FAU - Granito, Alessandro AU - Granito A AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. FAU - Giampaolo, Luca AU - Giampaolo L AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. FAU - Frisoni, Magda AU - Frisoni M AD - Department of Internal Medicine and Digestive Diseases, Azienda Ospedaliero-Universitaria S.Orsola-Malpighi, Bologna, Italy. FAU - Volta, Umberto AU - Volta U AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. FAU - Fusconi, Marco AU - Fusconi M AD - Department of Internal Medicine and Digestive Diseases, Azienda Ospedaliero-Universitaria S.Orsola-Malpighi, Bologna, Italy. FAU - Masi, Chiara AU - Masi C AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. FAU - Lenzi, Marco AU - Lenzi M AD - Department of Medical and Surgical Sciences, University of Bologna, Italy. LA - eng PT - Journal Article PL - Romania TA - J Gastrointestin Liver Dis JT - Journal of gastrointestinal and liver diseases : JGLD JID - 101272825 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoantibodies/blood MH - Autoimmune Diseases/epidemiology/immunology MH - Case-Control Studies MH - Early Diagnosis MH - Female MH - Humans MH - Italy/epidemiology MH - Liver Cirrhosis, Biliary/*complications/epidemiology/immunology MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/blood supply MH - Prevalence MH - Prospective Studies MH - Raynaud Disease/complications/epidemiology/immunology MH - Scleroderma, Systemic/complications/*diagnosis/epidemiology/immunology MH - Young Adult EDAT- 2014/04/02 06:00 MHDA- 2015/04/10 06:00 CRDT- 2014/04/02 06:00 PHST- 2014/04/02 06:00 [entrez] PHST- 2014/04/02 06:00 [pubmed] PHST- 2015/04/10 06:00 [medline] AID - 10 [pii] PST - ppublish SO - J Gastrointestin Liver Dis. 2014 Mar;23(1):39-43. PMID- 19011490 OWN - NLM STAT- MEDLINE DCOM- 20090226 LR - 20081117 IS - 1550-5073 (Electronic) IS - 0893-2190 (Linking) VI - 22 IP - 4 DP - 2008 Oct-Dec TI - Conquering common breast-feeding problems. PG - 267-74 LID - 10.1097/01.JPN.0000341356.45446.23 [doi] AB - Meeting mothers' personal breast-feeding goals depends on a number of factors, including the timely resolution of any problems she encounters. Nurses are often the first providers who interact with the mother during the perinatal period and are positioned to guide mothers through the prevention and solving of breast-feeding problems. Although many problems may be "common," failure to remedy conditions that cause pain, frustration, and anxiety can lead to premature weaning and avoidance of breast-feeding subsequent children. This article describes strategies and interventions to alleviate common problems that breast-feeding mothers frequently encounter. FAU - Walker, Marsha AU - Walker M AD - National Alliance for Breastfeeding Advocacy, Weston, MA 02493, USA. Marshalact@aol.com LA - eng PT - Journal Article PT - Review PL - United States TA - J Perinat Neonatal Nurs JT - The Journal of perinatal & neonatal nursing JID - 8801387 MH - Breast Feeding/*adverse effects/psychology MH - Causality MH - Humans MH - Mastitis/etiology/*prevention & control MH - *Mothers/education/psychology MH - Neonatal Nursing/methods MH - Nipples/blood supply/*injuries MH - *Nurse's Role MH - Nursing Assessment MH - Pain/etiology/*prevention & control MH - Patient Education as Topic MH - Postnatal Care/methods MH - Raynaud Disease/etiology/prevention & control MH - Skin Care/methods/nursing MH - Sucking Behavior RF - 65 EDAT- 2008/11/18 09:00 MHDA- 2009/02/27 09:00 CRDT- 2008/11/18 09:00 PHST- 2008/11/18 09:00 [pubmed] PHST- 2009/02/27 09:00 [medline] PHST- 2008/11/18 09:00 [entrez] AID - 00005237-200810000-00007 [pii] AID - 10.1097/01.JPN.0000341356.45446.23 [doi] PST - ppublish SO - J Perinat Neonatal Nurs. 2008 Oct-Dec;22(4):267-74. doi: 10.1097/01.JPN.0000341356.45446.23. PMID- 18520173 OWN - NLM STAT- MEDLINE DCOM- 20080730 LR - 20080603 IS - 0021-4884 (Print) IS - 0021-4884 (Linking) VI - 57 IP - 5 DP - 2008 May TI - [Skin manifestations of collagen diseases unfamiliar to internists and pediatricians]. PG - 524-8 FAU - Tsuchida, Tetsuya AU - Tsuchida T AD - Department of Dermatology, Saitama Medical University. LA - jpn PT - Journal Article PT - Review PL - Japan TA - Arerugi JT - Arerugi = [Allergy] JID - 0241212 SB - IM MH - Acute Disease MH - Chronic Disease MH - Collagen Diseases/*diagnosis/*pathology MH - Dermatomyositis/pathology MH - Diagnosis, Differential MH - Face/pathology MH - Hand/pathology MH - Humans MH - *Internal Medicine MH - *Pediatrics MH - Raynaud Disease/pathology MH - Scleroderma, Diffuse/pathology MH - Sjogren's Syndrome/pathology MH - Skin/*pathology RF - 1 EDAT- 2008/06/04 09:00 MHDA- 2008/07/31 09:00 CRDT- 2008/06/04 09:00 PHST- 2008/06/04 09:00 [pubmed] PHST- 2008/07/31 09:00 [medline] PHST- 2008/06/04 09:00 [entrez] AID - 057050524e [pii] PST - ppublish SO - Arerugi. 2008 May;57(5):524-8. PMID- 39415373 OWN - NLM STAT- MEDLINE DCOM- 20241017 LR - 20241114 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 50 IP - 4 DP - 2024 Nov TI - Vascular, Soft Tissue, and Musculoskeletal Imaging in Systemic Sclerosis. PG - 661-681 LID - S0889-857X(24)00056-5 [pii] LID - 10.1016/j.rdc.2024.07.006 [doi] AB - This review examines the role of various imaging techniques in assessing vascular and musculoskeletal manifestations in Systemic Sclerosis (SSc). Imaging modalities, such as thermography, capillaroscopy, ultrasound, optical coherence tomography, laser speckle contrast analysis, radiography, computed tomography, and MRI, offer valuable insights into SSc-related complications. Findings suggest that these techniques aid in diagnosing conditions like Raynaud phenomenon, digital ulcers, calcinosis, acro-osteolysis, and hand contractures. However, each modality has its advantages and limitations, necessitating a multimodal approach for comprehensive evaluation and accurate diagnosis of SSc-related manifestations. CI - Copyright © 2024 Elsevier Inc. All rights reserved. FAU - González, José Miguel AU - González JM AD - Department of Radiology, Pontificia Universidad Católica de Chile, Marcoleta 377, Santiago, Chile. FAU - Valenzuela, Antonia AU - Valenzuela A AD - Department of Clinical Immunology and Rheumatology, Pontificia Universidad Católica de Chile, Diagonal Paraguay 362, Piso 6, Of 629, Santiago, Chile. Electronic address: antonia.valenzuela@uc.cl. LA - eng PT - Journal Article PT - Review DEP - 20240916 PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnostic imaging/complications MH - Magnetic Resonance Imaging/methods MH - Musculoskeletal Diseases/diagnostic imaging/etiology MH - Raynaud Disease/diagnostic imaging/etiology MH - Tomography, X-Ray Computed/methods MH - Microscopic Angioscopy/methods MH - Tomography, Optical Coherence/methods MH - Thermography/methods MH - Vascular Diseases/diagnostic imaging/etiology MH - Ultrasonography/methods MH - Laser Speckle Contrast Imaging MH - Diagnostic Imaging/methods MH - Calcinosis/diagnostic imaging/etiology OTO - NOTNLM OT - Imaging OT - Musculoskeletal OT - Soft tissue OT - Systemic sclerosis OT - Vascular COIS- Disclosures The authors have nothing to disclose. EDAT- 2024/10/17 10:05 MHDA- 2024/10/17 10:06 CRDT- 2024/10/17 00:51 PHST- 2024/10/17 10:06 [medline] PHST- 2024/10/17 10:05 [pubmed] PHST- 2024/10/17 00:51 [entrez] AID - S0889-857X(24)00056-5 [pii] AID - 10.1016/j.rdc.2024.07.006 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2024 Nov;50(4):661-681. doi: 10.1016/j.rdc.2024.07.006. Epub 2024 Sep 16. PMID- 22030896 OWN - NLM STAT- MEDLINE DCOM- 20120702 LR - 20260128 IS - 1533-4023 (Electronic) IS - 0160-2446 (Linking) VI - 59 IP - 3 DP - 2012 Mar TI - No significant effect of ginkgo biloba special extract EGb 761 in the treatment of primary Raynaud phenomenon: a randomized controlled trial. PG - 215-21 LID - 10.1097/FJC.0b013e31823c0bed [doi] AB - BACKGROUND: Medicinal treatment of vasospastic Raynaud phenomenon is limited to primarily vasodilator medicines. OBJECTIVE: To explore the possible beneficial effects and tolerability of 120 mg two times a day of Ginkgo Biloba special extract EGb 761 in patients suffering from Raynaud disease (RD) (primary Raynaud phenomenon). METHODS: In a placebo-controlled, double-blind, pilot study, 41 patients with RD were randomized to either the active treatment group (EGb 761, n = 21) or placebo group for 10 weeks, after an initial 2-week run-in phase. The primary efficacy variables were self-reported changes of the frequency, duration, and severity of vasospastic attacks between the placebo-controlled run-in phase and the end of the study. RESULTS: Most of the patients were female, and both groups were perfectly matched with respect to demographic characteristics. The frequency of daily attacks reduced from 3.6 ± 2.3 to 2.4 ± 2.6 (-33%) in the EGb 761 group and from 2.9 ± 2.0 to 2.0 ± 1.8 (-31%) in the placebo group with no significant difference according to the ordinary least squares test (P = 0.3564). Furthermore, no significant differences were found with respect to the duration and severity of vasospastic attacks between the EGb 761 and placebo groups (P = 0.4392 and P = 0.7187, respectively). In all, 17 adverse events (AEs) were reported, 6 AEs from 5 patients in the EGb 761 group and 11 AEs from 8 patients in the placebo group. Serious AEs did not occur. CONCLUSION: EGb 761 treatment showed an excellent safety profile in patients with RD but could not demonstrate a statistically significant reduction in clinically relevant symptoms compared with placebo. FAU - Bredie, Sebastian J H AU - Bredie SJ AD - Department of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands. s.bredie@aig.umcn.nl FAU - Jong, Miek C AU - Jong MC LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - J Cardiovasc Pharmacol JT - Journal of cardiovascular pharmacology JID - 7902492 RN - 0 (Plant Extracts) RN - 19FUJ2C58T (Ginkgo biloba extract) RN - 0 (Ginkgo Extract) SB - IM MH - Adult MH - Double-Blind Method MH - Female MH - Ginkgo biloba MH - Humans MH - Male MH - Middle Aged MH - Pilot Projects MH - Plant Extracts/adverse effects/*pharmacology MH - Raynaud Disease/*drug therapy/physiopathology MH - Severity of Illness Index MH - Time Factors MH - Treatment Outcome MH - *Vasoconstriction MH - Ginkgo Extract EDAT- 2011/10/28 06:00 MHDA- 2012/07/03 06:00 CRDT- 2011/10/28 06:00 PHST- 2011/10/28 06:00 [entrez] PHST- 2011/10/28 06:00 [pubmed] PHST- 2012/07/03 06:00 [medline] AID - 10.1097/FJC.0b013e31823c0bed [doi] PST - ppublish SO - J Cardiovasc Pharmacol. 2012 Mar;59(3):215-21. doi: 10.1097/FJC.0b013e31823c0bed. PMID- 23724751 OWN - NLM STAT- MEDLINE DCOM- 20130725 LR - 20260128 IS - 0032-6518 (Print) IS - 0032-6518 (Linking) VI - 257 IP - 1760 DP - 2013 Apr TI - Blue brains (or peripheral stasis). 1913. PG - 37 FAU - Goodhart, James F AU - Goodhart JF LA - eng PT - Biography PT - Historical Article PT - Journal Article PT - Seminal Article PL - England TA - Practitioner JT - The Practitioner JID - 0404245 SB - IM MH - Arterial Pressure MH - History, 20th Century MH - Humans MH - Peripheral Vascular Diseases/*history MH - Raynaud Disease/history PS - Goodhart JF FPS - Goodhart, James F EDAT- 2013/06/04 06:00 MHDA- 2013/07/26 06:00 CRDT- 2013/06/04 06:00 PHST- 2013/06/04 06:00 [entrez] PHST- 2013/06/04 06:00 [pubmed] PHST- 2013/07/26 06:00 [medline] PST - ppublish SO - Practitioner. 2013 Apr;257(1760):37. PMID- 17478765 OWN - NLM STAT- MEDLINE DCOM- 20070524 LR - 20151119 IS - 1066-8969 (Print) IS - 1066-8969 (Linking) VI - 15 IP - 2 DP - 2007 Apr TI - Segmental arterial mediolysis with accompanying venous angiopathy: a clinical pathologic review, report of 3 new cases, and comments on the role of endothelin-1 in its pathogenesis. PG - 121-34 AB - The authors review 20 cases of segmental arterial mediolysis (SAM) including 3 newly reported cases. SAM developed in areas of vascular distention in 2 of the latter cases: 1 in utero in the heart of a recipient of a twin transfusion syndrome and the other in the jejunum secondary to partial venous obstruction. In the third case, it occurred in a patient with Raynaud disease. Characterizing SAM are injurious and reparative lesions that occur in the media and/or at the adventitial medial junction. Four distinctive alterations are recognized: (1) mediolysis, (2) a tearing separation of the outer media from adventitia, (3) arterial gaps, and (4) a florid reparative response that replaces zones of mediolysis and fills areas of medial adventitial separation. The repair can transform SAM into lesions indistinguishable from common types of fibromuscular dysplasia (FMD.) A venous angiopathy involving large and medium-sized veins accompanies SAM. It features medial muscle vacuolar change with lysis leading to apparent separation of residual muscle bundles. Immunostaining shows endothelin-1 (ET-1) decorating adventitial capillaries in SAM and neighboring arteries, in capillaries of adjoining tissues, and outlining smooth muscle cell membranes in adjacent veins including those of the venous angiopathy. The significance of these changes is uncertain. Vasospasm is believed to cause SAM, but ET-1 is not the direct pressor agent responsible for this condition. The reason(s) for synthesis and release of ET-1 in SAM are still hypothetical, but local perturbations in vascular tone may be an important factor. ET-1 may be indirectly play a role in SAM by cross-talking and potentiating the activities of other vasoconstrictors such as norepinephrine and by orchestrating its reparative phase. FAU - Slavin, Richard E AU - Slavin RE AD - Cascade Pathology Group, Legacy Portland Hospitals, Emanuel Hospital and Health Center, Portland, Oregon, USA. rslavin@lhs.org FAU - Inada, Kiyoshi AU - Inada K LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Int J Surg Pathol JT - International journal of surgical pathology JID - 9314927 RN - 0 (Biomarkers) RN - 0 (Endothelin-1) SB - IM MH - Adult MH - Arteries/metabolism/*pathology MH - Arteritis/complications/metabolism/*pathology MH - Biomarkers/metabolism MH - Coronary Vessels/metabolism/pathology MH - Dilatation, Pathologic/complications/metabolism/pathology MH - Endothelin-1/*metabolism MH - Female MH - Fetal Diseases/metabolism/pathology MH - Fetofetal Transfusion/metabolism/pathology MH - Humans MH - Jejunum/blood supply MH - Male MH - Middle Aged MH - Muscle, Smooth, Vascular/metabolism/pathology MH - Peripheral Vascular Diseases/complications/metabolism/pathology MH - Pregnancy MH - Raynaud Disease/complications/metabolism/pathology MH - Tunica Media/metabolism/*pathology MH - Veins/embryology/metabolism/*pathology EDAT- 2007/05/05 09:00 MHDA- 2007/05/26 09:00 CRDT- 2007/05/05 09:00 PHST- 2007/05/05 09:00 [pubmed] PHST- 2007/05/26 09:00 [medline] PHST- 2007/05/05 09:00 [entrez] AID - 15/2/121 [pii] AID - 10.1177/1066896906297684 [doi] PST - ppublish SO - Int J Surg Pathol. 2007 Apr;15(2):121-34. doi: 10.1177/1066896906297684. PMID- 38469635 OWN - NLM STAT- MEDLINE DCOM- 20240719 LR - 20240917 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 49 IP - 8 DP - 2024 Jul 19 TI - Nailfold capillary abnormalities are associated with increased severity of Raynaud phenomenon among patients with systemic sclerosis: a pilot study. PG - 912-913 LID - 10.1093/ced/llae079 [doi] FAU - Lau, Charles B AU - Lau CB AD - Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA. AD - Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA. FAU - Smith, Gideon P AU - Smith GP AD - Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA. LA - eng PT - Letter PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 SB - IM MH - Humans MH - *Raynaud Disease/complications MH - Pilot Projects MH - *Scleroderma, Systemic/complications/pathology MH - Female MH - *Nails/pathology/blood supply MH - Middle Aged MH - Male MH - *Capillaries/pathology/abnormalities MH - Severity of Illness Index MH - Adult MH - Aged COIS- Conflicts of interest The authors declare no conflicts of interest. EDAT- 2024/03/12 06:43 MHDA- 2024/07/19 12:43 CRDT- 2024/03/12 04:03 PHST- 2024/02/24 00:00 [received] PHST- 2024/03/14 00:00 [accepted] PHST- 2024/07/19 12:43 [medline] PHST- 2024/03/12 06:43 [pubmed] PHST- 2024/03/12 04:03 [entrez] AID - 7625614 [pii] AID - 10.1093/ced/llae079 [doi] PST - ppublish SO - Clin Exp Dermatol. 2024 Jul 19;49(8):912-913. doi: 10.1093/ced/llae079. PMID- 19177933 OWN - NLM STAT- MEDLINE DCOM- 20090202 LR - 20151119 IS - 0023-7205 (Print) IS - 0023-7205 (Linking) VI - 105 IP - 50 DP - 2008 Dec 10-16 TI - [Raynaud phenomenon as adverse effect of ADHD treatment]. PG - 3662-3 LA - swe PT - Journal Article TT - Raynauds fenomen som biverkan till ADHD-behandling. PL - Sweden TA - Lakartidningen JT - Lakartidningen JID - 0027707 RN - 0 (Adrenergic Uptake Inhibitors) RN - 0 (Central Nervous System Stimulants) RN - 0 (Dopamine Uptake Inhibitors) RN - 0 (Propylamines) RN - 207ZZ9QZ49 (Methylphenidate) RN - 57WVB6I2W0 (Atomoxetine Hydrochloride) SB - IM MH - Adrenergic Uptake Inhibitors/adverse effects MH - Atomoxetine Hydrochloride MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Central Nervous System Stimulants/*adverse effects MH - Dopamine Uptake Inhibitors/adverse effects MH - Humans MH - Methylphenidate/adverse effects MH - Propylamines/adverse effects MH - Raynaud Disease/*chemically induced EDAT- 2009/01/31 09:00 MHDA- 2009/02/03 09:00 CRDT- 2009/01/31 09:00 PHST- 2009/01/31 09:00 [entrez] PHST- 2009/01/31 09:00 [pubmed] PHST- 2009/02/03 09:00 [medline] PST - ppublish SO - Lakartidningen. 2008 Dec 10-16;105(50):3662-3. PMID- 37582056 OWN - NLM STAT- MEDLINE DCOM- 20230928 LR - 20250526 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 35 IP - 6 DP - 2023 Nov 1 TI - Raynaud phenomenon and microvasculopathy in systemic sclerosis: multi-modality imaging for diagnosis and evaluation. PG - 324-333 LID - 10.1097/BOR.0000000000000965 [doi] AB - PURPOSE OF REVIEW: To describe the clinical significance of and the diagnostic approach to Raynaud phenomenon (RP) in the peripheral extremities and the heart. RECENT FINDINGS: Nailfold capillaroscopy has recently been standardized in an expert consensus paper. Abnormal capillaroscopy in combination with specific autoantibody profiles and clinical signs are highly predictive of progression of RP to systemic sclerosis (SSc). Magnetic resonance imaging (MRI) can also perform tissue characterization of both the extremities and the heart. Microvascular wall abnormalities detected using nailfold capillaroscopy in patients with SSc may lead to deposition of erythrocyte-derived iron, due to microhemorrhages, which may predispose to fibrosis. MRI can assess the presence of iron using T2∗ measurements. SUMMARY: RP is a hallmark of the microvasculopathy in SSc and can affect both the peripheral extremities and the heart. Nailfold capillaroscopy is the current gold standard for the evaluation of the peripheral microvasculature. Other imaging modalities include thermography, laser Doppler-derived methods, 99m Tc-pertechnetate hand perfusion scintigraphy, power Doppler ultrasonography, dynamic optical coherence tomography, MRI, and photoacoustic imaging, but these are currently not widely used. Cardiac RP can be investigated with positron emission tomography or cardiovascular magnetic resonance, with the latter offering the additional possibility of tissue characterization and iron content quantification secondary to microhemorrhages. CI - Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved. FAU - Markousis-Mavrogenis, George AU - Markousis-Mavrogenis G AD - University Research Institute of Maternal and Child Health and Precision Medicine and UNESCO Chair in Adolescent Health Care, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital. FAU - Bournia, Vasiliki-Kalliopi AU - Bournia VK AD - Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School. FAU - Sfikakis, Petros P AU - Sfikakis PP AD - Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School. FAU - Mavrogeni, Sophie I AU - Mavrogeni SI AD - University Research Institute of Maternal and Child Health and Precision Medicine and UNESCO Chair in Adolescent Health Care, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital. AD - Onassis Cardiac Surgery Hospital, Athens, Greece. LA - eng PT - Journal Article PT - Review DEP - 20230813 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging/etiology MH - Ultrasonography MH - Heart MH - Multimodal Imaging MH - Microscopic Angioscopy/methods EDAT- 2023/08/15 18:41 MHDA- 2023/09/28 06:43 CRDT- 2023/08/15 13:03 PHST- 2023/09/28 06:43 [medline] PHST- 2023/08/15 18:41 [pubmed] PHST- 2023/08/15 13:03 [entrez] AID - 00002281-202311000-00006 [pii] AID - 10.1097/BOR.0000000000000965 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2023 Nov 1;35(6):324-333. doi: 10.1097/BOR.0000000000000965. Epub 2023 Aug 13. PMID- 29274125 OWN - NLM STAT- MEDLINE DCOM- 20181106 LR - 20181106 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 43 IP - 4 DP - 2018 Jun TI - Ischaemic ulcers on the toes secondary to Raynaud phenomenon in a patient with systemic sclerosis successfully treated with botulinum toxin. PG - 503-505 LID - 10.1111/ced.13333 [doi] FAU - Garrido-Ríos, A A AU - Garrido-Ríos AA AD - Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. FAU - González-Olivares, M AU - González-Olivares M AD - Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. FAU - Navarro-Vidal, B AU - Navarro-Vidal B AD - Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. FAU - Martínez-Morán, C AU - Martínez-Morán C AD - Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. FAU - Borbujo, J AU - Borbujo J AD - Department of Dermatology, Hospital Universitario de Fuenlabrada, Madrid, Spain. LA - eng PT - Journal Article DEP - 20171222 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Humans MH - Ischemia/*drug therapy/etiology MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*complications MH - Scleroderma, Systemic/*complications MH - Toes/*blood supply MH - Treatment Outcome MH - Ulcer/*drug therapy/etiology EDAT- 2017/12/24 06:00 MHDA- 2018/11/07 06:00 CRDT- 2017/12/24 06:00 PHST- 2017/05/10 00:00 [accepted] PHST- 2017/12/24 06:00 [pubmed] PHST- 2018/11/07 06:00 [medline] PHST- 2017/12/24 06:00 [entrez] AID - 10.1111/ced.13333 [doi] PST - ppublish SO - Clin Exp Dermatol. 2018 Jun;43(4):503-505. doi: 10.1111/ced.13333. Epub 2017 Dec 22. PMID- 19299112 OWN - NLM STAT- MEDLINE DCOM- 20090721 LR - 20090504 IS - 1873-569X (Electronic) IS - 0923-1811 (Linking) VI - 54 IP - 3 DP - 2009 Jun TI - Transcriptional activity of genes coding transforming growth factor beta-1 and its receptors in patients with systemic sclerosis and Raynaud phenomenon. PG - 216-8 LID - 10.1016/j.jdermsci.2009.01.008 [doi] FAU - Lis-Swiety, Anna AU - Lis-Swiety A FAU - Gola, Joanna AU - Gola J FAU - Mazurek, Urszula AU - Mazurek U FAU - Brzezińska-Wcisło, Ligia AU - Brzezińska-Wcisło L LA - eng PT - Letter DEP - 20090318 PL - Netherlands TA - J Dermatol Sci JT - Journal of dermatological science JID - 9011485 RN - 0 (Receptors, Transforming Growth Factor beta) RN - 0 (Transforming Growth Factor beta1) SB - IM MH - Adult MH - Gene Dosage MH - *Gene Expression Regulation MH - Humans MH - Middle Aged MH - Raynaud Disease/*genetics MH - Receptors, Transforming Growth Factor beta/*genetics MH - Scleroderma, Systemic/*genetics MH - Transcription, Genetic MH - Transforming Growth Factor beta1/*genetics EDAT- 2009/03/21 09:00 MHDA- 2009/07/22 09:00 CRDT- 2009/03/21 09:00 PHST- 2008/12/08 00:00 [received] PHST- 2009/01/26 00:00 [revised] PHST- 2009/01/29 00:00 [accepted] PHST- 2009/03/21 09:00 [entrez] PHST- 2009/03/21 09:00 [pubmed] PHST- 2009/07/22 09:00 [medline] AID - S0923-1811(09)00051-6 [pii] AID - 10.1016/j.jdermsci.2009.01.008 [doi] PST - ppublish SO - J Dermatol Sci. 2009 Jun;54(3):216-8. doi: 10.1016/j.jdermsci.2009.01.008. Epub 2009 Mar 18. PMID- 17849090 OWN - NLM STAT- MEDLINE DCOM- 20080122 LR - 20181113 IS - 0017-8470 (Print) IS - 0017-8470 (Linking) VI - 58 IP - 10 DP - 2007 Oct TI - [Therapy of systemic sclerosis]. PG - 851-7 AB - The therapy of systemic sclerosis (SSc) remains a challenge for dermatology, rheumatology, internal medicine, and other disciplines. Organ involvement, above all kidney and lungs, is a key therapeutic issue. The current developments in organ-specific therapy are the main topic of the article. Finally, possibilities of disease-modifying drugs and value of HSCT are discussed. FAU - Meurer, M AU - Meurer M AD - Dermatologie am Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany. Michael.Meurer@uniklinikum-dresden.de FAU - Rehberger, P AU - Rehberger P LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Therapie bei systemischer Sklerodermie. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Gastrointestinal Agents) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - JED5K35YGL (Iloprost) SB - IM MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Cardiovascular Diseases/diagnosis/therapy MH - Fibrosis MH - Fingers/blood supply MH - Gastrointestinal Agents/therapeutic use MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - Hypertension, Pulmonary/therapy MH - Iloprost/therapeutic use MH - Kidney Transplantation MH - PUVA Therapy MH - Piperazines/therapeutic use MH - Purines/therapeutic use MH - Raynaud Disease/diagnosis/therapy MH - Recurrence MH - Scleroderma, Systemic/diagnosis/*therapy MH - Sildenafil Citrate MH - Skin Ulcer/diagnosis/therapy MH - Sulfones/therapeutic use MH - Ultraviolet Therapy MH - Vasodilator Agents/therapeutic use RF - 64 EDAT- 2007/09/13 09:00 MHDA- 2008/01/23 09:00 CRDT- 2007/09/13 09:00 PHST- 2007/09/13 09:00 [pubmed] PHST- 2008/01/23 09:00 [medline] PHST- 2007/09/13 09:00 [entrez] AID - 10.1007/s00105-007-1404-z [doi] PST - ppublish SO - Hautarzt. 2007 Oct;58(10):851-7. doi: 10.1007/s00105-007-1404-z. PMID- 28418945 OWN - NLM STAT- MEDLINE DCOM- 20180521 LR - 20220317 IS - 1536-3686 (Electronic) IS - 1075-2765 (Linking) VI - 24 IP - 5 DP - 2017 Sep/Oct TI - Tendonitis and Tendon Rupture After Treatment With Rituximab: A Case Series. PG - e592-e595 LID - 10.1097/MJT.0000000000000591 [doi] AB - CLINICAL DATA: Rituximab is a mouse/human chimeric anti-CD20 IgG1 monoclonal antibody used to treat cancer and autoimmune conditions. Side effects of rituximab include fever, rash, cytopenia and hypotension, back pain, arthralgia, and myalgia. Here, we report on 3 patients who developed moderate to severe tendonitis after the second infusion of rituximab. THERAPEUTIC CHALLENGE: We report 3 patients who developed tendonitis after the second infusion of rituximab. These patients were undergoing treatment for connective tissue diseases. All 3 patients received 2 rituximab infusions, 2 weeks apart. The 3 cases developed clinical tendonitis that was confirmed by magnetic resonance imaging in 2 cases. INTERPRETATION: This is the first case series reporting new onset tendonitis in patients with connective tissue diseases after rituximab therapy. All 3 cases developed tendonitis 1 week after receiving the second dose of rituximab. Clinical features of tendonitis resolved 3-4 months in all cases. The underlying pathogenic mechanism by which rituximab causes tendonitis is not clear, but tendonitis and tendon rupture have been reported after using other medications such as quinolones. The tendon damage was progressive leading to tendon rupture in 1 patient, highlighting the importance of early recognition. It is plausible that there is a cause-effect relation between tendonitis and administration of rituximab in our 3 cases, since none of these cases had previous history of tendonitis; however, more data are needed to confirm this observation. FAU - Alqahtani, Ali AU - Alqahtani A AD - 1Department of Internal Medicine, University of Toledo Medical Center, Toledo, OH; and 2Division of Rheumatology and Immunology, Department of Internal Medicine, University of Toledo Medical Center, Toledo, OH. FAU - Sabha, Marwa AU - Sabha M FAU - Abdelfattah, Thaer AU - Abdelfattah T FAU - Srour, Khaled AU - Srour K FAU - Dhayihi, Turki AU - Dhayihi T FAU - Kahaleh, Bashar AU - Kahaleh B FAU - Altorok, Nezam AU - Altorok N LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Ther JT - American journal of therapeutics JID - 9441347 RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - Antisynthetase syndrome SB - IM MH - Achilles Tendon/diagnostic imaging/injuries MH - Adult MH - Arthritis, Rheumatoid/blood/*drug therapy/immunology MH - Female MH - Humans MH - Immunologic Factors/*adverse effects MH - Magnetic Resonance Imaging MH - Middle Aged MH - Myositis/blood/*drug therapy/immunology MH - Pain/etiology MH - Raynaud Disease/blood/*drug therapy/immunology MH - Rituximab/*adverse effects MH - Rupture/diagnostic imaging/etiology MH - Tendinopathy/*chemically induced/complications/diagnostic imaging MH - Tendon Injuries/diagnostic imaging/*etiology EDAT- 2017/04/19 06:00 MHDA- 2018/05/22 06:00 CRDT- 2017/04/19 06:00 PHST- 2017/04/19 06:00 [pubmed] PHST- 2018/05/22 06:00 [medline] PHST- 2017/04/19 06:00 [entrez] AID - 10.1097/MJT.0000000000000591 [doi] PST - ppublish SO - Am J Ther. 2017 Sep/Oct;24(5):e592-e595. doi: 10.1097/MJT.0000000000000591. PMID- 35777828 OWN - NLM STAT- MEDLINE DCOM- 20220830 LR - 20250728 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 28 IP - 6 DP - 2022 Sep 1 TI - Raynaud Phenomenon: Angiographic Appearances of Reversible Vasoconstriction. PG - e693-e694 LID - 10.1097/RHU.0000000000001880 [doi] FAU - Clements, Warren AU - Clements W AUID- ORCID: 0000-0003-1859-5850 FAU - Kuang, Ronny J D AU - Kuang RJD AD - Department of Radiology, Monash Health, Melbourne, Australia. LA - eng PT - Journal Article DEP - 20220630 PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Angiography MH - Humans MH - *Raynaud Disease MH - *Vasoconstriction COIS- The authors declare no conflict of interest. EDAT- 2022/07/02 06:00 MHDA- 2022/08/31 06:00 CRDT- 2022/07/01 20:52 PHST- 2022/07/02 06:00 [pubmed] PHST- 2022/08/31 06:00 [medline] PHST- 2022/07/01 20:52 [entrez] AID - 00124743-202209000-00011 [pii] AID - 10.1097/RHU.0000000000001880 [doi] PST - ppublish SO - J Clin Rheumatol. 2022 Sep 1;28(6):e693-e694. doi: 10.1097/RHU.0000000000001880. Epub 2022 Jun 30. PMID- 25303330 OWN - NLM STAT- MEDLINE DCOM- 20150422 LR - 20181202 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 67 IP - 3 DP - 2015 Mar TI - Reply: To PMID 24664962. PG - 453-4 LID - 10.1002/acr.22491 [doi] FAU - Stojan, George AU - Stojan G AD - Harvard Medical School, Boston, MA. FAU - Gelber, Allan C AU - Gelber AC LA - eng PT - Comment PT - Letter PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM CON - Arthritis Care Res (Hoboken). 2014 Aug;66(8):1263-8. doi: 10.1002/acr.22331. PMID: 24664962 CON - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453. doi: 10.1002/acr.22489. PMID: 25303107 MH - Arthritis, Rheumatoid/*etiology MH - Carcinoma, Non-Small-Cell Lung/*diagnosis MH - Female MH - Humans MH - Lung Neoplasms/*diagnosis MH - Raynaud Disease/*etiology MH - *Weight Loss EDAT- 2014/10/11 06:00 MHDA- 2015/04/23 06:00 CRDT- 2014/10/11 06:00 PHST- 2014/10/11 06:00 [entrez] PHST- 2014/10/11 06:00 [pubmed] PHST- 2015/04/23 06:00 [medline] AID - 10.1002/acr.22491 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453-4. doi: 10.1002/acr.22491. PMID- 22658741 OWN - NLM STAT- MEDLINE DCOM- 20121003 LR - 20220419 IS - 1474-547X (Electronic) IS - 0140-6736 (Linking) VI - 380 IP - 9845 DP - 2012 Sep 8 TI - Acro-osteolysis. PG - 916 LID - 10.1016/S0140-6736(12)60275-X [doi] FAU - Ferreira, Ivo R AU - Ferreira IR AD - Department of Radiology, Centro Hospitalar do Porto, Porto, Portugal. ivoferreira@ecsaude.uminho.pt FAU - Domingues, Vital S AU - Domingues VS LA - eng PT - Case Reports PT - Journal Article DEP - 20120601 PL - England TA - Lancet JT - Lancet (London, England) JID - 2985213R SB - IM MH - Acro-Osteolysis/*diagnostic imaging/etiology MH - Aged MH - Hand Bones/*diagnostic imaging MH - Humans MH - Male MH - Radiography MH - Raynaud Disease/*complications EDAT- 2012/06/05 06:00 MHDA- 2012/10/04 06:00 CRDT- 2012/06/05 06:00 PHST- 2012/06/05 06:00 [entrez] PHST- 2012/06/05 06:00 [pubmed] PHST- 2012/10/04 06:00 [medline] AID - S0140-6736(12)60275-X [pii] AID - 10.1016/S0140-6736(12)60275-X [doi] PST - ppublish SO - Lancet. 2012 Sep 8;380(9845):916. doi: 10.1016/S0140-6736(12)60275-X. Epub 2012 Jun 1. PMID- 16550713 OWN - NLM STAT- MEDLINE DCOM- 20060612 LR - 20081121 IS - 1661-8157 (Print) IS - 1661-8157 (Linking) VI - 95 IP - 10 DP - 2006 Mar 8 TI - [Blue discoloration of the fingers]. PG - 379-80 FAU - Geeser, A AU - Geeser A AD - Medizinische Poliklinik, Universitätsspital Basel. LA - ger PT - Case Reports PT - Journal Article TT - Blauverfärbung der Finger. PL - Switzerland TA - Praxis (Bern 1994) JT - Praxis JID - 101468093 SB - IM MH - Cold Temperature/*adverse effects MH - Cyanosis/*etiology MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply MH - Humans MH - Middle Aged MH - Paresthesia/etiology MH - Raynaud Disease/*diagnosis EDAT- 2006/03/23 09:00 MHDA- 2006/06/13 09:00 CRDT- 2006/03/23 09:00 PHST- 2006/03/23 09:00 [pubmed] PHST- 2006/06/13 09:00 [medline] PHST- 2006/03/23 09:00 [entrez] AID - 10.1024/0369-8394.95.10.379 [doi] PST - ppublish SO - Praxis (Bern 1994). 2006 Mar 8;95(10):379-80. doi: 10.1024/0369-8394.95.10.379. PMID- 19839886 OWN - NLM STAT- MEDLINE DCOM- 20100203 LR - 20191111 IS - 1744-5108 (Electronic) IS - 0167-6830 (Linking) VI - 28 IP - 4 DP - 2009 TI - Graves' disease associated with primary systemic sclerosis. PG - 262-3 AB - Graves' disease can be associated with other autoimmune disorders. Primary systemic sclerosis (PSS) is such a disease characterised by multi-organ fibrosis. Abnormal thyroid antibody titres and hypothyroidism as well as hyperthyroidism have been observed in PSS patients. Specific and idiopathic inflammatory disorders are an important differential diagnosis in Graves'orbitopathy (GO). Mycophenolate mofetil (MM) is an immunosuppressive drug which depletes guanosin nucleotides in proliferating B-and T-lymphocytes. We describe the effect of MM on the endocrine orbitopathy of a patient with PSS. FAU - Wimmersberger, Yves AU - Wimmersberger Y AD - Hôpital Cantonal Genève, HUG, Clinique d'Ophtalmologie, Switzerland. FAU - Zuercher, Doris AU - Zuercher D LA - eng PT - Case Reports PT - Journal Article PL - England TA - Orbit JT - Orbit (Amsterdam, Netherlands) JID - 8301221 RN - 0 (Glucocorticoids) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) RN - 0 (Sulfonamides) RN - 8N3DW7272P (Cyclophosphamide) RN - HU9DX48N0T (Mycophenolic Acid) RN - MRK240IY2L (Azathioprine) RN - Q326023R30 (Bosentan) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Azathioprine/therapeutic use MH - Bosentan MH - Cyclophosphamide/therapeutic use MH - Drug Therapy, Combination MH - Female MH - Glucocorticoids/therapeutic use MH - Graves Disease/*complications/radiotherapy MH - Humans MH - Hypertension, Pulmonary/complications/drug therapy MH - Immunoglobulins, Intravenous/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Methotrexate/therapeutic use MH - Middle Aged MH - Mycophenolic Acid/analogs & derivatives/therapeutic use MH - Proteinuria/complications MH - Raynaud Disease/complications/drug therapy MH - Scleroderma, Systemic/*complications/drug therapy MH - Sulfonamides/therapeutic use EDAT- 2009/10/21 06:00 MHDA- 2010/02/04 06:00 CRDT- 2009/10/21 06:00 PHST- 2009/10/21 06:00 [entrez] PHST- 2009/10/21 06:00 [pubmed] PHST- 2010/02/04 06:00 [medline] AID - 10.1080/01676830903104637 [pii] AID - 10.1080/01676830903104637 [doi] PST - ppublish SO - Orbit. 2009;28(4):262-3. doi: 10.1080/01676830903104637. PMID- 19761954 OWN - NLM STAT- MEDLINE DCOM- 20091207 LR - 20120202 IS - 1532-9283 (Electronic) IS - 1360-8592 (Linking) VI - 13 IP - 4 DP - 2009 Oct TI - Effects of the myofascial release in diffuse systemic sclerosis. PG - 320-7 LID - 10.1016/j.jbmt.2008.04.042 [doi] AB - OBJECTIVE: To improve breathing and functionality of the temporomandibular joint (TMJ) and hands, by increasing the range of motion (ROM), and to reduce the level of pain. METHOD: Twenty myofascial release (MR) sessions in 2002 with assessments (chest expansion, mouth opening, ROM of wrist and fingers). Between the 19th and the 20th session there was a break of 110 days. Every winter, 1-3 sessions have been made. RESULTS: Chest: expansion increased by 3.5 cm and pain was eliminated at the scar from a biopsy; TMJ: an 8mm increase in mouth opening with pain eliminated; hands and fingers: increase of ROM in all joints of fingers and wrists, of up to 100%, reduction in ulcerations and recovery of nail growth. CONCLUSION: The connective tissue affected by diffuse systemic sclerosis (dSSc) is subject to remodeling through MR, receding when the work is interrupted. Resuming the treatment on a regular basis increased the ROM in joints, reduced the effects of the Raynaud Phenomenon and the pain. FAU - Martin, Marilene Marfin AU - Martin MM AD - ABR-Associação Brasileira de Rolfing, ERA-European Rolfing Association, Rua Ambrosina de Macedo, 107, CEP 04013-030 São Paulo, Brazil. marilene-martin@hotmail.com LA - eng PT - Case Reports PT - Journal Article DEP - 20080617 PL - United States TA - J Bodyw Mov Ther JT - Journal of bodywork and movement therapies JID - 9700068 SB - IM CIN - J Bodyw Mov Ther. 2012 Jan;16(1):2-4; author reply 4-6. doi: 10.1016/j.jbmt.2011.09.001. PMID: 22196419 MH - Adult MH - Female MH - Finger Joint/physiology MH - Humans MH - Myofascial Pain Syndromes/etiology/physiopathology/*therapy MH - *Physical Therapy Modalities MH - Range of Motion, Articular MH - Raynaud Disease/physiopathology/therapy MH - Respiratory Mechanics MH - Scleroderma, Systemic/complications/physiopathology/*therapy MH - Temporomandibular Joint/physiology MH - Wrist Joint/physiology EDAT- 2009/09/19 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/19 06:00 PHST- 2008/03/20 00:00 [received] PHST- 2008/04/20 00:00 [revised] PHST- 2008/04/27 00:00 [accepted] PHST- 2009/09/19 06:00 [entrez] PHST- 2009/09/19 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - S1360-8592(08)00077-6 [pii] AID - 10.1016/j.jbmt.2008.04.042 [doi] PST - ppublish SO - J Bodyw Mov Ther. 2009 Oct;13(4):320-7. doi: 10.1016/j.jbmt.2008.04.042. Epub 2008 Jun 17. PMID- 17507897 OWN - NLM STAT- MEDLINE DCOM- 20081020 LR - 20191210 IS - 1643-3750 (Electronic) IS - 1234-1010 (Linking) VI - 13 Suppl 1 DP - 2007 May TI - Comparison of thermography and Doppler sonography in the evaluation of the cold immersion test in women with excessive vasospastic reaction. PG - 121-8 AB - BACKGROUND: Determining the predisposition to vasospastic reactions in a function test is important at the earliest possible stage of diagnosis. Thermography is an acknowledged procedure for monitoring function tests; however, its availability is limited. The aim of this study was to compare the progression of the cold immersion test in a thermal picture and an US-CD examination with the flow assessment of the small blood vessels of the hand. MATERIAL/METHODS: A group of 16 women declaring high cold tolerance of their hands was compared with a group of 24 women reporting hand complaints when exposed to the cold. The subjects underwent a test in which they immersed their right hand in ice-cold water (0 degrees C). Then images of the temperature distribution in the hand were recorded with a thermovisory camera. At the same time, spectral waveforms were registered in the proper palmar digital artery and in the radial artery at specified time intervals. RESULTS: Data analysis comprised temperature measurements at the level of the nail plate of the middle finger and at the wrist. There were differences between the groups at all the stages of the immersion test (efficient return of temperature to initial values in the reference group, delayed return in subjects with low tolerance to thermal stimuli). Flow resistance analysis using the Doppler method in the studied arteries revealed similar differences between the groups (swift flow normalization and efficient reperfusion in the reference group, higher flow resistance and low reperfusion in subjects with low tolerance to thermal stimuli). CONCLUSIONS: Changes in the parameters of hand warmth during the immersion test assessed by thermography and changes in the flow parameters observed by Doppler sonography demonstrated similar progression, which suggests the equivalence of both methods. Doppler sonography may thus serve as a method of monitoring function tests to establish a predisposition to vasospasm. FAU - Stefańczyk, Ludomir AU - Stefańczyk L AD - Department of Radiology and Diagnostic Imaging, Medical University of Łódź, Łódź, Poland. stefanczyk_l@wp.pl FAU - Woźniakowski, Bartłomiej AU - Woźniakowski B FAU - Pietrzak, Piotr AU - Pietrzak P FAU - Majos, Agata AU - Majos A FAU - Grzelak, Piotr AU - Grzelak P LA - eng PT - Evaluation Study PT - Journal Article PL - United States TA - Med Sci Monit JT - Medical science monitor : international medical journal of experimental and clinical research JID - 9609063 SB - IM MH - Adult MH - Body Temperature MH - *Cold Temperature MH - Female MH - Fingers/blood supply/diagnostic imaging MH - Hand/blood supply/diagnostic imaging MH - Humans MH - *Immersion MH - Middle Aged MH - *Raynaud Disease/diagnosis/diagnostic imaging/physiopathology MH - *Thermography MH - Thermosensing/physiology MH - *Ultrasonography, Doppler MH - Vasoconstriction/*physiology EDAT- 2007/05/18 09:00 MHDA- 2008/10/22 09:00 CRDT- 2007/05/18 09:00 PHST- 2007/02/05 00:00 [received] PHST- 2007/03/26 00:00 [accepted] PHST- 2007/05/18 09:00 [pubmed] PHST- 2008/10/22 09:00 [medline] PHST- 2007/05/18 09:00 [entrez] AID - 10146 [pii] PST - ppublish SO - Med Sci Monit. 2007 May;13 Suppl 1:121-8. PMID- 27684814 OWN - NLM STAT- MEDLINE DCOM- 20170215 LR - 20240523 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 95 IP - 39 DP - 2016 Sep TI - Undiagnosed connective tissue diseases: High prevalence in pulmonary arterial hypertension patients. PG - e4827 LID - 10.1097/MD.0000000000004827 [doi] LID - e4827 AB - Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory.To estimate frequency and prognosis of previously undiagnosed CTDs in a suspect idiopathic (i) PAH cohort.Consecutive patients with PAH confirmed by right heart catheterization referred at the Cardiology Division of our Hospital without a previous rheumatological assessment or the occurrence of other conditions explaining PAH were checked for CTD by a clinical, laboratory, and instrumental evaluation. Survival in each group has also been analyzed.In our study 17 of 49 patients were classified as CTD-PAH, corresponding to a prevalence (95% CI) of 34.7% (21.7-49.6%). ANA positivity had 94% (71.3-99.9%) sensitivity and 78.1% (60-90.7%) specificity for a diagnosis of CTD-PAH; Raynaud phenomenon (RP) showed 83.3% (51.6-97.9%) sensitivity and 100% (90.5-100%) specificity for the diagnosis of Systemic Sclerosis (SSc)-PAH. At diagnosis, SSc patients were older and had a lower creatinine clearance compared with iPAH and other CTD-PAH. After a median follow-up of 44 (2-132) months, 18 of 49 (36.7%) patients died: 31.2% in the iPAH group, 20% in the CTD-, and 58.3% in the SSc-PAH group. Mortality was significantly higher in SSc-PAH (HR 3.32, 1.11-9.95, P <0.05) versus iPAH.We show a high prevalence of undiagnosed CTDs in patients with iPAH without a previous rheumatological assessment. All patients with RP were diagnosed with SSc. Our data stress the importance of a rheumatological assessment in PAH, especially because of the unfavorable prognostic impact of an associated SSc. FAU - Cavagna, Lorenzo AU - Cavagna L AD - aDivision of Rheumatology bDepartment of Cardiology, University and IRCCS Foundation Policlinico S. Matteo, Pavia cEpidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Codullo, Veronica AU - Codullo V FAU - Ghio, Stefano AU - Ghio S FAU - Scirè, Carlo Alberto AU - Scirè CA FAU - Guzzafame, Eleonora AU - Guzzafame E FAU - Scelsi, Laura AU - Scelsi L FAU - Rossi, Silvia AU - Rossi S FAU - Montecucco, Carlomaurizio AU - Montecucco C FAU - Caporali, Roberto AU - Caporali R LA - eng PT - Evaluation Study PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Adult MH - Aged MH - Connective Tissue Diseases/complications/*diagnosis/epidemiology MH - Delayed Diagnosis/*statistics & numerical data MH - Female MH - Humans MH - Hypertension, Pulmonary/etiology/*mortality MH - Male MH - Middle Aged MH - Prevalence MH - Prognosis MH - Raynaud Disease/complications/*diagnosis/epidemiology MH - Scleroderma, Systemic/complications/*diagnosis/epidemiology MH - Sensitivity and Specificity PMC - PMC5265907 COIS- The authors have no conflicts of interest to disclose. EDAT- 2016/09/30 06:00 MHDA- 2017/02/16 06:00 PMCR- 2016/09/30 CRDT- 2016/09/30 06:00 PHST- 2016/09/30 06:00 [entrez] PHST- 2016/09/30 06:00 [pubmed] PHST- 2017/02/16 06:00 [medline] PHST- 2016/09/30 00:00 [pmc-release] AID - 00005792-201609270-00024 [pii] AID - 10.1097/MD.0000000000004827 [doi] PST - ppublish SO - Medicine (Baltimore). 2016 Sep;95(39):e4827. doi: 10.1097/MD.0000000000004827. PMID- 27744398 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20180209 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 44 IP - 1 DP - 2017 Jan TI - Prevalence of Systemic Sclerosis in Primary Biliary Cholangitis Using the New ACR/EULAR Classification Criteria. PG - 33-39 LID - 10.3899/jrheum.160243 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria. The secondary objective was to evaluate the frequency of individual clinical features. METHODS: One hundred consecutive patients with PBC without previously diagnosed SSc were recruited between 2005 and 2007 from a tertiary care gastroenterology clinic. All patients underwent a complete clinical examination, determination of SSc-specific antibodies, and a nailfold capillary microscopy. Fulfillment of the 3 different criteria sets was analyzed, along with individual disease features. RESULTS: Of 100 patients with PBC, 1% met the ACR 1980 criteria, 22% met the 2001 LeRoy and Medsger criteria for early SSc, and 17% the 2013 ACR/EULAR criteria. Raynaud phenomenon, SSc-related antibodies, and SSc capillaroscopic patterns were the most prevalent findings, with the highest sensitivities to help guide future screening. CONCLUSION: Our data show a high prevalence of SSc in patients with PBC with probable underestimation by previous studies using the original ACR criteria. Comorbid SSc should be actively searched for based on newly described criteria to improve detection and increase benefits of earlier treatment. FAU - Zheng, Boyang AU - Zheng B AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. boyangz123@hotmail.com. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. boyangz123@hotmail.com. FAU - Vincent, Catherine AU - Vincent C AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. FAU - Fritzler, Marvin J AU - Fritzler MJ AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. FAU - Senécal, Jean-Luc AU - Senécal JL AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. FAU - Koenig, Martial AU - Koenig M AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. FAU - Joyal, France AU - Joyal F AD - From the Department of Internal Medicine, University of Montreal Hospital Center (CHUM), Hôpital Notre-Dame, Montreal, Quebec; the Department of Hepatology, CHUM, Hôpital St. Luc, Montreal, Quebec; the Department of Rheumatology, CHUM, Hôpital Notre-Dame, Montreal, Quebec; and the Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada. AD - B. Zheng, MD, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; C. Vincent, MD, Department of Hepatology, CHUM, Hôpital St. Luc; M.J. Fritzler, MD, PhD, Faculty of Medicine, University of Calgary; J.L. Senécal, MD, Department of Rheumatology, CHUM, Hôpital Notre-Dame; M. Koenig, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame; F. Joyal, MD, MSc, Department of Internal Medicine, CHUM, Hôpital Notre-Dame. LA - eng PT - Journal Article DEP - 20161015 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Aged MH - Cholangitis/*epidemiology MH - Comorbidity MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prevalence MH - Raynaud Disease/diagnosis/*epidemiology MH - Scleroderma, Systemic/diagnosis/*epidemiology OTO - NOTNLM OT - AUTOIMMUNE DISEASE OT - PRIMARY BILIARY CHOLANGITIS OT - PRIMARY BILIARY CIRRHOSIS OT - SYSTEMIC SCLEROSIS EDAT- 2016/10/17 06:00 MHDA- 2017/12/22 06:00 CRDT- 2016/10/17 06:00 PHST- 2016/09/09 00:00 [accepted] PHST- 2016/10/17 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2016/10/17 06:00 [entrez] AID - jrheum.160243 [pii] AID - 10.3899/jrheum.160243 [doi] PST - ppublish SO - J Rheumatol. 2017 Jan;44(1):33-39. doi: 10.3899/jrheum.160243. Epub 2016 Oct 15. PMID- 18004178 OWN - NLM STAT- MEDLINE DCOM- 20071219 LR - 20210108 IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 86 IP - 6 DP - 2007 Nov TI - Long-term follow-up of hypothenar hammer syndrome: a series of 47 patients. PG - 334-343 LID - 10.1097/MD.0b013e31815c95d3 [doi] AB - Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. This prompted us to conduct the current retrospective study to 1) evaluate the prevalence of HHS in patients with Raynaud phenomenon and 2) assess the short-term and long-term outcome in patients with HHS. From 1990 to 2006, 4148 consecutive patients were referred to the Department of Internal Medicine at the University of Rouen medical center for evaluation of Raynaud phenomenon using nailfold capillaroscopy. HHS was diagnosed in 47 of these 4148 patients (1.13% of cases).Forty-three patients (91.5%) had occupational exposure to repetitive palmar trauma. The more common occupations were factory worker (21.3%), mason (12.8%), carpenter (10.6%), and metal worker (10.6%); the mean duration of occupational exposure to repetitive palmar trauma at HHS diagnosis was 21 years. One patient (2.1%) had recreational exposure (aikido training) to repetitive trauma of the palmar ulnar artery, and 3 other patients (6.4%) developed HHS related to a single direct injury to the hypothenar area. Clinical manifestations were more often unilateral (87.2%) involving the dominant hand (93%). HHS complications included digital ischemic symptoms (ischemia: n = 21, necrosis: n = 20) and irritation of the sensory branch of the ulnar nerve (n = 11). In HHS patients, angiography demonstrated occlusion of the ulnar artery in the area of the Guyon space (59.6%), aneurysm of the ulnar artery in the area of the Guyon space (40.4%), and embolic multiple occlusions of the digital arteries (57.4%). All patients were advised to change their occupational exposure. They were given vasodilators, including calcium channel blocker (n = 37) and buflomedil (n = 12); 36 patients (76.6%) also received oral platelet aggregation inhibitors. Twenty-one patients with digital ischemia/necrosis were further given hemodilution therapy to reduce the hematocrit level to 35%. In 3 patients with HHS-related digital necrosis who exhibited partial improvement with vasodilators, prostacyclin analog therapy (a 5-day regimen of intravenous prostacyclin analog) was instituted, resulting in complete healing of digital ulcer in these 3 patients. Other conservative treatment options included controlling risk factors (smoking cessation, low-lipid diet, therapy for arterial hypertension) and careful local wound care of fingers in the 20 patients with digital necrosis. Only 2 patients, exhibiting digital necrosis and multiple digital artery occlusions, with nonthrombotic ulnar artery aneurysm underwent reconstructive surgery, that is, resection of the aneurysm with end-to-end anastomosis of the ulnar artery. The median length of follow-up in patients with HHS was 15.9 months. Thirteen patients (27.7%) exhibited clinical recurrences of HHS; the median time of HHS recurrence onset was 11 months. Outcome of HHS relapse was favorable with conservative measures in all cases. Awareness of HHS is required to increase suspicion of the disorder so that further exposure to risk factors like repetitive hypothenar trauma can be avoided for these patients; this is of great importance for their overall prognosis. We found favorable outcomes in most patients after conservative measures were initiated; therefore we suggest that surgery may be undertaken in the subgroup of patients who exhibit partial improvement while receiving conservative therapy. Finally, because we observed recurrence of HHS in 27.7% of patients, we note that HHS patients require close follow-up, including both regular and systematic physical vascular examination. FAU - Marie, Isabelle AU - Marie I AD - From Department of Internal Medicine (IM, FH, NC, HL) and Department of Radiology (EP), Rouen University Hospital, Rouen, France. FAU - Hervé, Fabien AU - Hervé F FAU - Primard, Etienne AU - Primard E FAU - Cailleux, Nicole AU - Cailleux N FAU - Levesque, Hervé AU - Levesque H LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Adult MH - Aneurysm/etiology MH - *Arterial Occlusive Diseases/diagnosis/epidemiology/etiology/therapy MH - *Cumulative Trauma Disorders/diagnosis/epidemiology/etiology/therapy MH - Embolism/etiology MH - Female MH - France/epidemiology MH - *Hand Injuries/diagnosis/epidemiology/etiology/therapy MH - Humans MH - Ischemia/etiology MH - Male MH - Middle Aged MH - *Occupational Diseases/diagnosis/epidemiology/etiology/therapy MH - Occupations MH - Prevalence MH - *Raynaud Disease/diagnosis/epidemiology/etiology/therapy MH - Risk Factors MH - Ulnar Artery/*injuries EDAT- 2007/11/16 09:00 MHDA- 2007/12/20 09:00 CRDT- 2007/11/16 09:00 PHST- 2007/11/16 09:00 [pubmed] PHST- 2007/12/20 09:00 [medline] PHST- 2007/11/16 09:00 [entrez] AID - 00005792-200711000-00003 [pii] AID - 10.1097/MD.0b013e31815c95d3 [doi] PST - ppublish SO - Medicine (Baltimore). 2007 Nov;86(6):334-343. doi: 10.1097/MD.0b013e31815c95d3. PMID- 18946335 OWN - NLM STAT- MEDLINE DCOM- 20090115 LR - 20081023 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 20 IP - 6 DP - 2008 Nov TI - Current world literature. PG - 729-35 LID - 10.1097/BOR.0b013e328317a234 [doi] LA - eng PT - Bibliography PT - Journal Article PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Animals MH - Fibrosis MH - Humans MH - Muscular Diseases MH - Myositis MH - Raynaud Disease MH - *Rheumatic Diseases MH - Scleroderma, Systemic EDAT- 2008/10/24 09:00 MHDA- 2009/01/16 09:00 CRDT- 2008/10/24 09:00 PHST- 2008/10/24 09:00 [pubmed] PHST- 2009/01/16 09:00 [medline] PHST- 2008/10/24 09:00 [entrez] AID - 00002281-200811000-00016 [pii] AID - 10.1097/BOR.0b013e328317a234 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2008 Nov;20(6):729-35. doi: 10.1097/BOR.0b013e328317a234. PMID- 31228526 OWN - NLM STAT- MEDLINE DCOM- 20200206 LR - 20200206 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 81 IP - 4 DP - 2019 Oct TI - Reply: Additional clinical pearls for the management of Raynaud phenomenon of the nipple. PG - e113 LID - S0190-9622(19)30987-9 [pii] LID - 10.1016/j.jaad.2019.06.023 [doi] FAU - Waldman, Reid A AU - Waldman RA AD - Department of Dermatology, University of Connecticut, Farmington, Connecticut. FAU - Finch, Justin AU - Finch J AD - Department of Dermatology, University of Connecticut, Farmington, Connecticut. FAU - Grant-Kels, Jane M AU - Grant-Kels JM AD - Department of Dermatology, University of Connecticut, Farmington, Connecticut. FAU - Whitaker-Worth, Diane AU - Whitaker-Worth D AD - Department of Dermatology, University of Connecticut, Farmington, Connecticut. Electronic address: whitaker@uchc.edu. LA - eng PT - Comment PT - Letter DEP - 20190619 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM CON - J Am Acad Dermatol. 2019 Jun;80(6):1483-1494. doi: 10.1016/j.jaad.2018.08.067. PMID: 30452953 CON - J Am Acad Dermatol. 2019 Oct;81(4):e111-e112. doi: 10.1016/j.jaad.2019.06.021. PMID: 31228529 MH - Humans MH - Nipples MH - *Raynaud Disease MH - *Skin Diseases EDAT- 2019/06/23 06:00 MHDA- 2020/02/07 06:00 CRDT- 2019/06/23 06:00 PHST- 2019/06/11 00:00 [received] PHST- 2019/06/12 00:00 [accepted] PHST- 2019/06/23 06:00 [pubmed] PHST- 2020/02/07 06:00 [medline] PHST- 2019/06/23 06:00 [entrez] AID - S0190-9622(19)30987-9 [pii] AID - 10.1016/j.jaad.2019.06.023 [doi] PST - ppublish SO - J Am Acad Dermatol. 2019 Oct;81(4):e113. doi: 10.1016/j.jaad.2019.06.023. Epub 2019 Jun 19. PMID- 37003608 OWN - NLM STAT- MEDLINE DCOM- 20230803 LR - 20260127 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 50 IP - 8 DP - 2023 Aug TI - Preliminary Clinical and Laser Speckle Contrast Analysis Data on Selexipag Efficacy for the Treatment of Digital Vasculopathy in Systemic Sclerosis. PG - 1029-1031 LID - 10.3899/jrheum.221113 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) is burdened by Raynaud phenomenon (RP) and digital ulcers (DUs), and sometimes standard vasoactive therapies are ineffective or contraindicated. Selexipag is an oral selective IP prostacyclin receptor agonist approved for the treatment of SSc-related pulmonary arterial hypertension. We aimed to evaluate the clinical and instrumental efficacy of selexipag in SSc digital vasculopathy. METHODS: Patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies were administered selexipag. RP- and DU-related clinical outcomes were evaluated, and digital perfusion was assessed by laser speckle contrast analysis (LASCA), all at baseline and after 3 months. RESULTS: Selexipag was administered to 9 patients with SSc (66.6% female, mean age 52.3 [SD 16.6] yrs). One patient had to stop the drug because of adverse effects. After 3 months of selexipag administration, there was a significant reduction in RP daily episodes (P = 0.01) and RP mean duration (P = 0.04). The number of DUs decreased from 10 to 4 without reaching statistical significance. A significant improvement in mean perfusion of the fingers (P = 0.02) was observed with LASCA. CONCLUSION: Selexipag showed good potential for the treatment of SSc digital vasculopathy. Our results are certainly preliminary, yet quite encouraging. New trials for the evaluation of selexipag efficacy in SSc digital vasculopathy are needed. CI - Copyright © 2023 by the Journal of Rheumatology. FAU - Di Battista, Marco AU - Di Battista M AUID- ORCID: 0000-0002-4788-5729 AD - M. Di Battista, MD, Rheumatology Unit, University of Pisa, Pisa, and Department of Medical Biotechnologies, University of Siena, Siena; dibattista.marco91@gmail.com. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - A. Della Rossa, MD, PhD, M. Da Rio, MD, G. De Mattia, MD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa. FAU - Da Rio, Mattia AU - Da Rio M AD - A. Della Rossa, MD, PhD, M. Da Rio, MD, G. De Mattia, MD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa. FAU - De Mattia, Giammarco AU - De Mattia G AD - A. Della Rossa, MD, PhD, M. Da Rio, MD, G. De Mattia, MD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa. FAU - Morganti, Riccardo AU - Morganti R AD - R. Morganti, ScD, PhD, Section of Statistics, University of Pisa, Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AUID- ORCID: 0000-0001-5937-4574 AD - A. Della Rossa, MD, PhD, M. Da Rio, MD, G. De Mattia, MD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa. LA - eng PT - Journal Article DEP - 20230401 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 5EXC0E384L (selexipag) RN - 0 (Acetamides) RN - 0 (Pyrazines) RN - digital ulcers SB - IM MH - Humans MH - Female MH - Middle Aged MH - Male MH - *Vascular Diseases/complications MH - Fingers MH - *Scleroderma, Systemic/complications/drug therapy MH - *Raynaud Disease/drug therapy/etiology MH - Lasers MH - *Skin Ulcer/drug therapy/etiology MH - Acetamides MH - Pyrazines OTO - NOTNLM OT - Raynaud phenomenon OT - digital ulcers OT - laser speckle contrast analysis OT - selexipag OT - systemic sclerosis OT - vasculopathy EDAT- 2023/04/02 06:00 MHDA- 2023/08/03 06:43 CRDT- 2023/04/01 20:42 PHST- 2023/03/08 00:00 [accepted] PHST- 2023/08/03 06:43 [medline] PHST- 2023/04/02 06:00 [pubmed] PHST- 2023/04/01 20:42 [entrez] AID - jrheum.221113 [pii] AID - 10.3899/jrheum.221113 [doi] PST - ppublish SO - J Rheumatol. 2023 Aug;50(8):1029-1031. doi: 10.3899/jrheum.221113. Epub 2023 Apr 1. PMID- 26055062 OWN - NLM STAT- MEDLINE DCOM- 20160406 LR - 20180524 IS - 1878-0814 (Electronic) IS - 1877-1173 (Linking) VI - 132 DP - 2015 TI - Temperature-Sensitive Intracellular Traffic of α2C-Adrenergic Receptor. PG - 245-65 LID - S1877-1173(15)00037-X [pii] LID - 10.1016/bs.pmbts.2015.02.008 [doi] AB - α(2C)-Adrenergic receptor (α(2C)-AR) is the least characterized adrenergic receptor subtype and still very little is known about the intracellular traffic properties and pathophysiological roles of this receptor. α(2C)-AR has an atypical subcellular localization. At 37 °C, in the vascular smooth muscle cells and in fibroblasts, the receptor is poorly localized at the plasma membrane and accumulates inside the cell. Exposure to lower temperatures stimulates α(2C)-AR transport to the cell surface. This particular intracellular trafficking of α(2C)-AR is significant in the pathology of Raynaud phenomenon. In this brief review, I will present general information on the tissue distribution and cellular localization of α(2C)-AR. Also, I will discuss the mechanisms involved in the receptor transport by focusing on the trafficking motifs and on the molecular chaperones. CI - © 2015 Elsevier Inc. All rights reserved. FAU - Filipeanu, Catalin M AU - Filipeanu CM AD - Department of Pharmacology, College of Medicine, Howard University, Washington, District of Columbia, USA. Electronic address: catalin.filipeanu@howard.edu. LA - eng GR - P20-GM103514/GM/NIGMS NIH HHS/United States GR - R03AR064008/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20150329 PL - Netherlands TA - Prog Mol Biol Transl Sci JT - Progress in molecular biology and translational science JID - 101498165 RN - 0 (Carrier Proteins) RN - 0 (FLNC protein, human) RN - 0 (Filamins) RN - 0 (HSP90 Heat-Shock Proteins) RN - 0 (Molecular Chaperones) RN - 0 (Receptors, Adrenergic, alpha-2) RN - EC 3.6.4.- (ATPases Associated with Diverse Cellular Activities) RN - EC 3.6.4.- (DNA Helicases) RN - EC 3.6.4.12 (RUVBL1 protein, human) SB - IM MH - ATPases Associated with Diverse Cellular Activities MH - Amino Acid Sequence MH - Animals MH - Carrier Proteins/metabolism MH - Cell Line MH - Cell Membrane/metabolism MH - DNA Helicases/metabolism MH - Fibroblasts/metabolism MH - Filamins/metabolism MH - HEK293 Cells MH - HSP90 Heat-Shock Proteins/metabolism MH - Humans MH - Molecular Chaperones/metabolism MH - Molecular Sequence Data MH - Muscle, Smooth, Vascular/metabolism MH - Protein Structure, Tertiary MH - Protein Transport MH - Raynaud Disease/*metabolism/physiopathology MH - Receptors, Adrenergic, alpha-2/*metabolism MH - Temperature MH - Tissue Distribution OTO - NOTNLM OT - Intracellular traffic OT - Molecular chaperones OT - Raynaud phenomenon OT - Temperature-sensitive OT - α(2C)-Adrenergic receptor EDAT- 2015/06/10 06:00 MHDA- 2016/04/07 06:00 CRDT- 2015/06/10 06:00 PHST- 2015/06/10 06:00 [entrez] PHST- 2015/06/10 06:00 [pubmed] PHST- 2016/04/07 06:00 [medline] AID - S1877-1173(15)00037-X [pii] AID - 10.1016/bs.pmbts.2015.02.008 [doi] PST - ppublish SO - Prog Mol Biol Transl Sci. 2015;132:245-65. doi: 10.1016/bs.pmbts.2015.02.008. Epub 2015 Mar 29. PMID- 36633635 OWN - NLM STAT- MEDLINE DCOM- 20230116 LR - 20230322 IS - 2731-7013 (Electronic) IS - 2731-7005 (Linking) VI - 74 IP - 1 DP - 2023 Jan TI - [Nailfold capillaroscopy - an important diagnostic procedure also for dermatologists]. PG - 53-54 LID - 10.1007/s00105-022-05095-1 [doi] FAU - Sunderkötter, Cord AU - Sunderkötter C AD - Universitätsklinik und Poliklinik für Dermatologie und Venerologie, Universitätsklinikum Halle (Saale), Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube-Str. 40, 06120, Halle (Saale), Deutschland. cord.sunderkoetter@uk-halle.de. LA - ger PT - Comment PT - Editorial TT - Kapillarmikroskopie – eine wichtige Untersuchung auch in der Dermatologie. DEP - 20230112 PL - Germany TA - Dermatologie (Heidelb) JT - Dermatologie (Heidelberg, Germany) JID - 9918384885206676 SB - IM CON - Z Rheumatol. 2022 May;81(4):313-322. doi: 10.1007/s00393-022-01200-w. PMID: 35445832 MH - Humans MH - *Microscopic Angioscopy/methods MH - Dermatologists MH - Nails/diagnostic imaging MH - *Raynaud Disease/diagnosis EDAT- 2023/01/13 06:00 MHDA- 2023/01/17 06:00 CRDT- 2023/01/12 11:14 PHST- 2022/12/05 00:00 [accepted] PHST- 2023/01/12 11:14 [entrez] PHST- 2023/01/13 06:00 [pubmed] PHST- 2023/01/17 06:00 [medline] AID - 10.1007/s00105-022-05095-1 [pii] AID - 10.1007/s00105-022-05095-1 [doi] PST - ppublish SO - Dermatologie (Heidelb). 2023 Jan;74(1):53-54. doi: 10.1007/s00105-022-05095-1. Epub 2023 Jan 12. PMID- 17416178 OWN - NLM STAT- MEDLINE DCOM- 20070530 LR - 20221207 IS - 0945-1129 (Print) IS - 0945-1129 (Linking) VI - 60 IP - 3 DP - 2007 Mar TI - [Management of problem wounds--a case report: amputation could be avoided]. PG - 128-30 FAU - Loczenski, Barbara AU - Loczenski B AD - Barbara@Loczenski.de LA - ger PT - Case Reports PT - Journal Article TT - Versorgung von Problemwunden--eine Kasuistik: Amputation liess sich vermeiden. PL - Germany TA - Pflege Z JT - Pflege Zeitschrift JID - 9430463 RN - 0 (Colloids) MH - Adult MH - Amputation, Surgical/*nursing MH - Bandages MH - Chronic Disease MH - Colloids MH - Female MH - Humans MH - Ischemia/nursing MH - Limb Salvage/*nursing MH - Raynaud Disease/nursing MH - Scleroderma, Systemic/nursing MH - Skin Ulcer/*nursing MH - *Toes/blood supply EDAT- 2007/04/10 09:00 MHDA- 2007/05/31 09:00 CRDT- 2007/04/10 09:00 PHST- 2007/04/10 09:00 [pubmed] PHST- 2007/05/31 09:00 [medline] PHST- 2007/04/10 09:00 [entrez] PST - ppublish SO - Pflege Z. 2007 Mar;60(3):128-30. PMID- 22772318 OWN - NLM STAT- MEDLINE DCOM- 20121207 LR - 20181201 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 51 IP - 10 DP - 2012 Oct TI - Comment on: a multicentre study on the reliability of qualitative and quantitative nail-fold videocapillaroscopy assessment. PG - 1921-2; author reply 1922 FAU - Houtman, Pieternella M AU - Houtman PM LA - eng PT - Comment PT - Letter DEP - 20120706 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2012 Apr;51(4):749-55. doi: 10.1093/rheumatology/ker403. PMID: 22190689 MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/*pathology MH - Scleroderma, Systemic/*pathology EDAT- 2012/07/10 06:00 MHDA- 2012/12/12 06:00 CRDT- 2012/07/10 06:00 PHST- 2012/07/10 06:00 [entrez] PHST- 2012/07/10 06:00 [pubmed] PHST- 2012/12/12 06:00 [medline] AID - kes172 [pii] AID - 10.1093/rheumatology/kes172 [doi] PST - ppublish SO - Rheumatology (Oxford). 2012 Oct;51(10):1921-2; author reply 1922. doi: 10.1093/rheumatology/kes172. Epub 2012 Jul 6. PMID- 37011178 OWN - NLM STAT- MEDLINE DCOM- 20230724 LR - 20250802 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 29 IP - 5 DP - 2023 Aug 1 TI - Botulinum Toxins for the Treatment of Raynaud Phenomenon: A Systematic Review With Meta-analysis. PG - e92-e99 LID - 10.1097/RHU.0000000000001965 [doi] AB - OBJECTIVE: Botulinum toxin (Btx) therapy has emerged as a potential treatment for patients with Raynaud phenomenon (RP) in recent years. This study aimed to investigate the efficacy and safety of Btx treatment for RP. METHODS: Databases of PubMed, EMBASE, Web of Science, and the Cochrane Central Register of Controlled Trials were searched from their inception up to August 2022. Studies that reported Btx use for the treatment of RP were included. A meta-analysis was conducted for the Shortened version of the Disabilities of the Arm, Shoulder, and Hand (Quick DASH) score and visual analog scale pain score using a random-effects model. RESULTS: Thirteen full-text studies were included. The pooled standard mean changes for the visual analog scale pain score and QuickDASH score were -3.82 (95% confidence interval, -6.62 to -1.02) and 0.83 (95% confidence interval, -1.47 to -0.19), respectively. The 2 most common complications were injection site pain and intrinsic hand weakness. CONCLUSIONS: The effect of Btx treatment on RP is promising based on current evidence. Nevertheless, more studies and randomized clinical trials with larger sample sizes are needed to confirm the current results. CI - Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved. FAU - Zhou, Yannan AU - Zhou Y AD - From the West China School of Medicine. FAU - Yu, Yue AU - Yu Y AD - Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. FAU - Bi, Siwei AU - Bi S AD - Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. FAU - Cen, Ying AU - Cen Y AD - Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20230404 PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Neuromuscular Agents) SB - IM MH - Humans MH - *Botulinum Toxins, Type A/adverse effects MH - *Neuromuscular Agents/adverse effects MH - Pain MH - Hand MH - *Raynaud Disease/diagnosis/drug therapy COIS- The authors declare no conflict of interest. EDAT- 2023/04/04 06:00 MHDA- 2023/07/24 06:42 CRDT- 2023/04/03 15:03 PHST- 2023/07/24 06:42 [medline] PHST- 2023/04/04 06:00 [pubmed] PHST- 2023/04/03 15:03 [entrez] AID - 00124743-202308000-00012 [pii] AID - 10.1097/RHU.0000000000001965 [doi] PST - ppublish SO - J Clin Rheumatol. 2023 Aug 1;29(5):e92-e99. doi: 10.1097/RHU.0000000000001965. Epub 2023 Apr 4. PMID- 21049762 OWN - NLM STAT- MEDLINE DCOM- 20101123 LR - 20130912 IS - 0011-4162 (Print) IS - 0011-4162 (Linking) VI - 86 IP - 1 DP - 2010 Jul TI - Hypergammaglobulinemic purpura of Waldenström. PG - 23-4 AB - Hypergammaglobulinemic purpura of Waldenström is a rare syndrome that includes recurrent episodic purpura occurring mainly on the lower extremities and dorsum of the feet. The hallmark of this condition is polyclonal hypergammaglobulinemia primarily composed of IgG. Although the condition generally is benign, it may herald an underlying connective tissue disease or hematologic malignancy. We report a case of a 47-year-old woman with episodic purpura of 3 years' duration associated with Raynaud phenomenon. FAU - Frankel, Amylynne AU - Frankel A AD - Mount Sinai School of Medicine, New York, New York, USA. FAU - Ingraffea, Adam AU - Ingraffea A FAU - Massé, Maureen AU - Massé M FAU - Zhou, Hua AU - Zhou H LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Cutis JT - Cutis JID - 0006440 RN - 0 (Immunoglobulin G) SB - IM MH - Female MH - Follow-Up Studies MH - Humans MH - Immunoglobulin G/*blood MH - Middle Aged MH - Purpura, Hyperglobulinemic/diagnosis/etiology/*immunology MH - Raynaud Disease/*complications EDAT- 2010/11/06 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/11/06 06:00 PHST- 2010/11/06 06:00 [entrez] PHST- 2010/11/06 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] PST - ppublish SO - Cutis. 2010 Jul;86(1):23-4. PMID- 31308207 OWN - NLM STAT- MEDLINE DCOM- 20200512 LR - 20200518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 11 DP - 2019 Nov TI - Effect of Season on Internet Searches for Information on Raynaud Phenomenon. PG - 1543-1544 LID - 10.3899/jrheum.190463 [doi] FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - (Rheumatology), Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. Michael.Hughes-6@postgrad.manchester.ac.uk. LA - eng PT - Comment PT - Letter DEP - 20190715 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CON - J Rheumatol. 2019 Oct;46(10):1326-1334. doi: 10.3899/jrheum.180818. PMID: 30824643 CIN - J Rheumatol. 2019 Nov;46(11):1544-1545. doi: 10.3899/jrheum.190565. PMID: 31308205 MH - Humans MH - Internet MH - *Raynaud Disease MH - *Scleroderma, Systemic MH - Seasons EDAT- 2019/07/17 06:00 MHDA- 2020/05/19 06:00 CRDT- 2019/07/17 06:00 PHST- 2019/07/17 06:00 [pubmed] PHST- 2020/05/19 06:00 [medline] PHST- 2019/07/17 06:00 [entrez] AID - jrheum.190463 [pii] AID - 10.3899/jrheum.190463 [doi] PST - ppublish SO - J Rheumatol. 2019 Nov;46(11):1543-1544. doi: 10.3899/jrheum.190463. Epub 2019 Jul 15. PMID- 30197053 OWN - NLM STAT- MEDLINE DCOM- 20190509 LR - 20221207 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 145 IP - 12 DP - 2018 Dec TI - [Digital necrosis revealing cold agglutinin disease: Treatment with rituximab]. PG - 761-764 LID - S0151-9638(18)30500-3 [pii] LID - 10.1016/j.annder.2018.07.010 [doi] AB - BACKGROUND: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult. PATIENTS AND METHODS: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing. DISCUSSION: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period. CI - Copyright © 2018 Elsevier Masson SAS. All rights reserved. FAU - Klejtman, T AU - Klejtman T AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. Electronic address: tiffany.klejtman@gmail.com. FAU - Garel, B AU - Garel B AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. FAU - Senet, P AU - Senet P AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. FAU - Tribout, L AU - Tribout L AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. FAU - Bachmeyer, C AU - Bachmeyer C AD - Service de médecine interne, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France. FAU - Barbaud, A AU - Barbaud A AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. FAU - Monfort, J-B AU - Monfort JB AD - Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France. LA - fre PT - Case Reports PT - Journal Article TT - Nécroses digitales révélant une maladie des agglutinines froides : traitement par rituximab. DEP - 20180907 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Cryoglobulins) RN - 0 (Immunoglobulin kappa-Chains) RN - 0 (Immunosuppressive Agents) RN - 0 (cold agglutinins) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Amputation, Surgical MH - Anemia, Hemolytic, Autoimmune/complications/*diagnosis/drug therapy/surgery MH - Cold Temperature MH - Combined Modality Therapy MH - Computed Tomography Angiography MH - Cryoglobulins/analysis MH - Fingers/blood supply/diagnostic imaging/*pathology/surgery MH - Hand Deformities, Acquired/*etiology MH - Humans MH - Immunoglobulin kappa-Chains/blood MH - Immunosuppressive Agents/*therapeutic use MH - Ischemia/*etiology/surgery MH - Male MH - Middle Aged MH - Necrosis MH - Occupational Diseases/etiology MH - Raynaud Disease/diagnostic imaging/*etiology MH - Rituximab/*therapeutic use MH - Smoking/adverse effects OTO - NOTNLM OT - Acrosyndrome OT - Cold agglutinin disease OT - Digital necrosis OT - Maladie des agglutinines froides OT - Nécrose digitale OT - Raynaud OT - Rituximab EDAT- 2018/09/11 06:00 MHDA- 2019/05/10 06:00 CRDT- 2018/09/11 06:00 PHST- 2017/10/31 00:00 [received] PHST- 2017/12/11 00:00 [revised] PHST- 2018/07/20 00:00 [accepted] PHST- 2018/09/11 06:00 [pubmed] PHST- 2019/05/10 06:00 [medline] PHST- 2018/09/11 06:00 [entrez] AID - S0151-9638(18)30500-3 [pii] AID - 10.1016/j.annder.2018.07.010 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2018 Dec;145(12):761-764. doi: 10.1016/j.annder.2018.07.010. Epub 2018 Sep 7. PMID- 36242592 OWN - NLM STAT- MEDLINE DCOM- 20221018 LR - 20221209 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 1 IP - 3 DP - 2022 Jul-Sep TI - Nailfold capillaroscopy: from the early ages to the early diagnosis of systemic sclerosis. PG - 188-189 FAU - Araújo, Filipe C AU - Araújo FC AD - Rheumatology and Osteoporosis Unit, Hospital de Sant'Ana - SCML. FAU - Rocha, Margarida Lucas AU - Rocha ML AD - Rheumatology Department, Centro Hospitalar e Universitário do Algarve EPE. LA - eng PT - Editorial TT - Nailfold capillaroscopy: from the early ages to the early diagnosis of systemic sclerosis. PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 SB - IM MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease/diagnosis MH - *Scleroderma, Systemic/diagnosis EDAT- 2022/10/16 06:00 MHDA- 2022/10/19 06:00 CRDT- 2022/10/15 11:02 PHST- 2022/10/15 11:02 [entrez] PHST- 2022/10/16 06:00 [pubmed] PHST- 2022/10/19 06:00 [medline] AID - ED220248 [pii] PST - ppublish SO - ARP Rheumatol. 2022 Jul-Sep;1(3):188-189. PMID- 18603658 OWN - NLM STAT- MEDLINE DCOM- 20081014 LR - 20080825 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 9 DP - 2008 Sep TI - Pulmonary artery hypertension as the presenting feature of systemic sclerosis sine scleroderma. PG - 1431-2 LID - 10.1093/rheumatology/ken241 [doi] FAU - Pauling, J D AU - Pauling JD FAU - Gunawardena, H AU - Gunawardena H FAU - Coghlan, J G AU - Coghlan JG FAU - Easaw, J AU - Easaw J FAU - Suntharalingam, J AU - Suntharalingam J FAU - McHugh, N J AU - McHugh NJ LA - eng PT - Case Reports PT - Letter DEP - 20080628 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Female MH - Humans MH - Hypertension, Pulmonary/*etiology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications MH - Tomography, X-Ray Computed EDAT- 2008/07/08 09:00 MHDA- 2008/10/15 09:00 CRDT- 2008/07/08 09:00 PHST- 2008/07/08 09:00 [pubmed] PHST- 2008/10/15 09:00 [medline] PHST- 2008/07/08 09:00 [entrez] AID - ken241 [pii] AID - 10.1093/rheumatology/ken241 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Sep;47(9):1431-2. doi: 10.1093/rheumatology/ken241. Epub 2008 Jun 28. PMID- 17640308 OWN - NLM STAT- MEDLINE DCOM- 20080306 LR - 20191210 IS - 0007-0963 (Print) IS - 0007-0963 (Linking) VI - 157 IP - 4 DP - 2007 Oct TI - Assessment of abnormal blood flow and efficacy of treatment in patients with systemic sclerosis using a newly developed microwireless laser Doppler flowmeter and arm-raising test. PG - 690-7 AB - Background Patients with systemic sclerosis (SSc) frequently suffer from recalcitrant digital ulceration because of impaired cutaneous blood flow (CBF). A simple and accurate CBF measurement would be helpful to evaluate the disease status and efficacy of treatment in such patients. Objectives To examine the feasibility of a newly developed, micromachined integrated laser blood flowmeter (MILBF) for evaluation of abnormal CBF responses in patients with SSc. Methods CBF of finger pulp was measured in eight patients with SSc and in six healthy controls using MILBF. CBF in the steady state and the responses to the arm-raising test and cold provocation were assessed. The therapeutic efficacy of a single and an intensive prostaglandin E(1) (PGE(1)) infusion treatment was also evaluated in some of the SSc patients. Results The patients with SSc showed significantly lower steady-state CBF than controls. The rate of blood flow with cold provocation and the velocity of blood flow recovery after cold provocation (VR-CP) tended to be lower in patients with SSc. Augmentation of amplitude of the digital pulse wave by arm raising (AA-AR) was observed in controls, but not in patients with SSc. We also found that VR-CP and AA-AR may be good markers for evaluating the efficacy of vasodilatory treatment. It should be noted that the examined patients did not complain of any pain and/or distress during the arm-raising test, as opposed to during cold provocation. Conclusions CBF assessment using MILBF and an arm-raising test is accurate, noninvasive and well tolerated and thus the combination may be a better alternative method to evaluate abnormal CBF and efficacy of treatment in patients with SSc. FAU - Kido, M AU - Kido M AD - Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. macky@dermatol.med.kyushu-u.ac.jp FAU - Takeuchi, S AU - Takeuchi S FAU - Hayashida, S AU - Hayashida S FAU - Urabe, K AU - Urabe K FAU - Sawada, R AU - Sawada R FAU - Furue, M AU - Furue M LA - eng PT - Evaluation Study PT - Journal Article DEP - 20070719 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Vasodilator Agents) RN - F5TD010360 (Alprostadil) SB - IM MH - Aged MH - Alprostadil/therapeutic use MH - Cold Temperature MH - Equipment Design MH - Female MH - Fingers/*blood supply MH - Humans MH - Laser-Doppler Flowmetry/*instrumentation/methods MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis/drug therapy/etiology MH - Regional Blood Flow MH - Scleroderma, Systemic/drug therapy/*physiopathology MH - Skin/blood supply MH - Treatment Outcome MH - Vasodilator Agents/therapeutic use EDAT- 2007/07/21 09:00 MHDA- 2008/03/07 09:00 CRDT- 2007/07/21 09:00 PHST- 2007/07/21 09:00 [pubmed] PHST- 2008/03/07 09:00 [medline] PHST- 2007/07/21 09:00 [entrez] AID - BJD8093 [pii] AID - 10.1111/j.1365-2133.2007.08093.x [doi] PST - ppublish SO - Br J Dermatol. 2007 Oct;157(4):690-7. doi: 10.1111/j.1365-2133.2007.08093.x. Epub 2007 Jul 19. PMID- 23036295 OWN - NLM STAT- MEDLINE DCOM- 20130405 LR - 20221207 IS - 1558-0504 (Electronic) IS - 0094-1298 (Linking) VI - 39 IP - 4 DP - 2012 Oct TI - New concepts and technologies in reconstructive hand surgery. PG - 445-51 LID - S0094-1298(12)00097-1 [pii] LID - 10.1016/j.cps.2012.07.013 [doi] AB - Complex traumatic injuries and degenerative conditions of the hand continue to lead to significant impairment and disability. From technical innovations to regenerative concepts, this article presents the latest advances in the dynamic field of hand surgery in which worldwide efforts are made around the globe to repair, regenerate, or restore each composite tissue forming the hand. The systematic method by which finger replantation is performed, from bony fixation to skin closure, provides a platform for discussion of the newest innovations available to reconstructive hand surgeons. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Schmitt, Taliah AU - Schmitt T AD - Division of Plastic & Reconstructive Surgery, Department of Surgery, Stanford University Hospital & Clinics, 770 Welch Road, Suite 400, Palo Alto, CA 94304, USA. DrTSchmitt@gmail.com FAU - Talley, John AU - Talley J FAU - Chang, James AU - Chang J LA - eng PT - Journal Article PT - Review DEP - 20120830 PL - United States TA - Clin Plast Surg JT - Clinics in plastic surgery JID - 0424767 RN - 0 (Adhesives) RN - 0 (Cyanoacrylates) RN - 0 (Neuromuscular Agents) RN - 106392-12-5 (Poloxamer) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adhesives MH - Anastomosis, Surgical/instrumentation MH - Bone Transplantation MH - Botulinum Toxins, Type A/therapeutic use MH - Cicatrix/surgery MH - Cyanoacrylates MH - Guided Tissue Regeneration MH - Hand Bones/injuries/surgery MH - Hand Injuries/*surgery MH - Humans MH - Neuromuscular Agents/therapeutic use MH - Neurosurgical Procedures/instrumentation MH - Osteomyelitis/surgery MH - Peripheral Nerve Injuries/surgery MH - Poloxamer MH - Raynaud Disease/etiology/therapy MH - Plastic Surgery Procedures/*methods/*trends MH - Tendons/transplantation MH - Tissue Engineering EDAT- 2012/10/06 06:00 MHDA- 2013/04/06 06:00 CRDT- 2012/10/06 06:00 PHST- 2012/10/06 06:00 [entrez] PHST- 2012/10/06 06:00 [pubmed] PHST- 2013/04/06 06:00 [medline] AID - S0094-1298(12)00097-1 [pii] AID - 10.1016/j.cps.2012.07.013 [doi] PST - ppublish SO - Clin Plast Surg. 2012 Oct;39(4):445-51. doi: 10.1016/j.cps.2012.07.013. Epub 2012 Aug 30. PMID- 17018510 OWN - NLM STAT- MEDLINE DCOM- 20061024 LR - 20131121 IS - 0029-7844 (Print) IS - 0029-7844 (Linking) VI - 108 IP - 3 Pt 2 DP - 2006 Sep TI - Vasospasm of the nipple presenting as painful lactation. PG - 806-8 AB - BACKGROUND: Breast pain is a common complaint among lactating women. Vasospasm of the nipple should be considered in the differential diagnosis of breast pain, particularly when no other signs of infection or trauma are encountered. This report demonstrates a case of vasospasm successfully treated with nifedipine. CASE: A 26-year-old breastfeeding multipara presented with intermittent episodes of extreme pain associated with blanching of the nipple. The pain subsided upon return of normal color to the nipple. She was able to continue breastfeeding after successful treatment with nifedipine. CONCLUSION: Vasospasm of the nipple causes severe episodic breast pain and may lead to discontinuation of breastfeeding if not appropriately treated. This phenomenon is not well reported in the obstetric and gynecologic literature, although the obstetrician may be the first physician to evaluate a patient with symptoms. Patients with episodic nipple pain and pallor can be successfully treated with nifedipine. FAU - Page, Sarah M AU - Page SM AD - Department of Obstetrics, Wright-Patterson Medical Center and Wright State University, Dayton, OH 45433, usa. Sarah.page@wpafb.af.mil FAU - McKenna, David S AU - McKenna DS LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Obstet Gynecol JT - Obstetrics and gynecology JID - 0401101 RN - 0 (Vasodilator Agents) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Breast Feeding MH - Female MH - Humans MH - *Lactation MH - Nifedipine/therapeutic use MH - Nipples/*blood supply MH - *Pain MH - Raynaud Disease/diagnosis/drug therapy MH - Vascular Diseases/*diagnosis/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2006/10/05 09:00 MHDA- 2006/10/25 09:00 CRDT- 2006/10/05 09:00 PHST- 2006/10/05 09:00 [pubmed] PHST- 2006/10/25 09:00 [medline] PHST- 2006/10/05 09:00 [entrez] AID - 108/3/806 [pii] AID - 10.1097/01.AOG.0000214671.19023.68 [doi] PST - ppublish SO - Obstet Gynecol. 2006 Sep;108(3 Pt 2):806-8. doi: 10.1097/01.AOG.0000214671.19023.68. PMID- 41443361 OWN - NLM STAT- MEDLINE DCOM- 20260309 LR - 20260309 IS - 1879-0518 (Electronic) IS - 0010-7824 (Linking) VI - 156 DP - 2026 Apr TI - Novel systemic vasculo-cardiac syndrome following nuvaring initiation: Persistent tachycardia, raynaud-like episodes, and elevated D-dimer. PG - 111322 LID - S0010-7824(25)00513-X [pii] LID - 10.1016/j.contraception.2025.111322 [doi] AB - A 45-year-old woman developed persistent tachycardia, elevated D-dimer (899 ng/mL), Raynaud-like episodes, and a persistent non-pruritic erythematous rash one month after NuvaRing initiation. Complete symptom resolution occurred within ten days of discontinuation, except for persistent rash. This represents a novel systemic thromboinflammatory syndrome. CI - Copyright © 2026 Elsevier Inc. All rights reserved. FAU - Nigro, Angelo AU - Nigro A AD - Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. Electronic address: angelo.nigro@asmbasilicata.it. FAU - Gianciotta, Rossana AU - Gianciotta R AD - Specialist in Legal Medicine, Matera, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20251222 PL - United States TA - Contraception JT - Contraception JID - 0234361 RN - 0 (fibrin fragment D) RN - 0 (Fibrin Fibrinogen Degradation Products) SB - IM MH - Humans MH - Female MH - Middle Aged MH - *Fibrin Fibrinogen Degradation Products/metabolism/analysis MH - *Raynaud Disease/chemically induced MH - *Tachycardia/chemically induced MH - Syndrome MH - Exanthema/chemically induced OTO - NOTNLM OT - Contraception OT - D-dimer OT - NuvaRing OT - Tachycardia OT - Thromboinflammation EDAT- 2025/12/25 00:29 MHDA- 2026/03/09 07:42 CRDT- 2025/12/24 19:31 PHST- 2025/08/18 00:00 [received] PHST- 2025/12/10 00:00 [revised] PHST- 2025/12/10 00:00 [accepted] PHST- 2026/03/09 07:42 [medline] PHST- 2025/12/25 00:29 [pubmed] PHST- 2025/12/24 19:31 [entrez] AID - S0010-7824(25)00513-X [pii] AID - 10.1016/j.contraception.2025.111322 [doi] PST - ppublish SO - Contraception. 2026 Apr;156:111322. doi: 10.1016/j.contraception.2025.111322. Epub 2025 Dec 22. PMID- 39617410 OWN - NLM STAT- MEDLINE DCOM- 20250426 LR - 20250728 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 52 IP - 2 DP - 2025 Feb 1 TI - Association of the Apollo Wearable With Fatigue, Raynaud Phenomenon, and Quality of Life in Patients With Systemic Sclerosis: A Pilot Study. PG - 158-164 LID - 10.3899/jrheum.2024-0551 [doi] AB - OBJECTIVE: In patients with systemic sclerosis (SSc), fatigue is the highest-ranked symptom affecting quality of life (QOL), followed by Raynaud phenomenon (RP). We report results from a pilot study of the Apollo wearable device in patients with SSc. METHODS: Twenty-five adult participants with SSc, moderate fatigue, and RP were enrolled. Participants completed a 4-week intervention, during which they wore the Apollo device daily for a minimum of 15 minutes. The primary outcome was change on the Patient Reported Outcomes Measurement Information System Fatigue 13a (PROMIS Fatigue) at 4 weeks. RESULTS: After 4 weeks of using the Apollo wearable, participants reported less fatigue on the PROMIS Fatigue (P < 0.001) scale. The average daily number of RP attacks declined (P = 0.007), as did the Raynaud Condition Score (P = 0.007) after 4 weeks of use. Average device usage (2.87 hours/day) far exceeded the requested time, and no adverse events occurred. The PROMIS-29 subscores assessment demonstrated QOL improvement in physical function (P = 0.01), depression (P = 0.03), fatigue (P = 0.01), sleep disturbance (P = 0.002), and ability to participate in social roles and activities (P < 0.001). Significant improvements were also noted for depression (P = 0.004) and disability (P < 0.05) measures. CONCLUSION: Use of the Apollo wearable for 4 weeks was associated with improvement in fatigue and RP symptoms in patients with SSc, with improved QOL measures, lower depression scores, and improved disability measures. Future studies should further test the efficacy of the Apollo wearable in these domains and QOL of patients with SSc. (ClinicalTrials.gov: NCT04854850). CI - Copyright © 2025 by the Journal of Rheumatology. FAU - Hammaker, Krista AU - Hammaker K AUID- ORCID: 0000-0001-7234-2120 AD - K. Hammaker, BS, Northeast Ohio Medical University, Department of Medicine, Rootstown, Ohio. FAU - Hu, Haomin AU - Hu H AUID- ORCID: 0000-0001-9200-062X AD - H. Hu, MS, Department of Health Information Management, University of Pittsburgh, Pittsburgh, Philadelphia. FAU - Laffoon, Maureen AU - Laffoon M AD - M. Laffoon, BS, L.A. Freno, CRNP, R. Lafyatis, MD, R.T. Domsic, MD, MPH, Division of Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, Philadelphia. FAU - Freno, Leigh A AU - Freno LA AD - M. Laffoon, BS, L.A. Freno, CRNP, R. Lafyatis, MD, R.T. Domsic, MD, MPH, Division of Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, Philadelphia. FAU - Lafyatis, Robert AU - Lafyatis R AUID- ORCID: 0000-0002-9398-5034 AD - M. Laffoon, BS, L.A. Freno, CRNP, R. Lafyatis, MD, R.T. Domsic, MD, MPH, Division of Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, Philadelphia. FAU - Park, Yongseok AU - Park Y AD - Y. Park, PhD, Department of Biostatistics, University of Pittsburgh, Pittsburgh, Philadelphia, USA. FAU - Domsic, Robyn T AU - Domsic RT AD - M. Laffoon, BS, L.A. Freno, CRNP, R. Lafyatis, MD, R.T. Domsic, MD, MPH, Division of Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, Philadelphia; rtd4@pitt.edu. LA - eng SI - ClinicalTrials.gov/NCT04854850 PT - Clinical Trial PT - Journal Article DEP - 20250201 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Fatigue/etiology/therapy MH - Patient Reported Outcome Measures MH - Pilot Projects MH - *Quality of Life MH - *Raynaud Disease/etiology/therapy MH - *Scleroderma, Systemic/complications/therapy/physiopathology MH - *Wearable Electronic Devices OTO - NOTNLM OT - Raynaud phenomenon OT - fatigue OT - quality of life OT - systemic sclerosis OT - wearable EDAT- 2024/12/02 05:30 MHDA- 2025/02/02 05:10 CRDT- 2024/12/01 20:42 PHST- 2024/11/12 00:00 [accepted] PHST- 2025/02/02 05:10 [medline] PHST- 2024/12/02 05:30 [pubmed] PHST- 2024/12/01 20:42 [entrez] AID - jrheum.2024-0551 [pii] AID - 10.3899/jrheum.2024-0551 [doi] PST - epublish SO - J Rheumatol. 2025 Feb 1;52(2):158-164. doi: 10.3899/jrheum.2024-0551. PMID- 29635077 OWN - NLM STAT- MEDLINE DCOM- 20180612 LR - 20180612 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 17 IP - 6 DP - 2018 Jun TI - Systemic lupus erythematosus and systemic sclerosis: All roads lead to platelets. PG - 625-635 LID - S1568-9972(18)30091-0 [pii] LID - 10.1016/j.autrev.2018.01.012 [doi] AB - Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) are two phenotypically distincts inflammatory systemic diseases. However, SLE and SSc share pathogenic features such as interferon signature, loss of tolerance against self-nuclear antigens and increased tissue damage such as fibrosis. Recently, platelets have emerged as a major actor in immunity including auto-immune diseases. Both SLE and SSc are characterized by strong platelet system activation, which is likely to be both the witness and culprit in their pathogenesis. Platelet activation pathways are multiple and sometimes redundant. They include immune complexes, Toll-like receptors activation, antiphospholipid antibodies and ischemia-reperfusion associated with Raynaud phenomenon. Once activated, platelet promote immune dysregulation by priming interferon production by immune cells, providing CD40L supporting B lymphocyte functions and providing a source of autoantigens. Platelets are actively implicated in SLE and SSc end-organ damage such as cardiovascular and renal disease and in the promotion of tissue fibrosis. Finally, after understanding the main pathogenic implications of platelet activation in both diseases, we discuss potential therapeutics targeting platelets. CI - Copyright © 2018 Elsevier B.V. All rights reserved. FAU - Scherlinger, Marc AU - Scherlinger M AD - Service de Rhumatologie, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Guillotin, Vivien AU - Guillotin V AD - Service de médecine interne, FHU ACRONIM, Hôpital Saint André, Centre Hospitalier Universitaire, 1 rue Jean Burguet, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Service de Rhumatologie, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Contin-Bordes, Cécile AU - Contin-Bordes C AD - Laboratoire d'Immunologie et Immunogénétique, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Sisirak, Vanja AU - Sisirak V AD - Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Duffau, Pierre AU - Duffau P AD - Service de médecine interne, FHU ACRONIM, Hôpital Saint André, Centre Hospitalier Universitaire, 1 rue Jean Burguet, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Lazaro, Estibaliz AU - Lazaro E AD - Laboratoire d'Immunologie et Immunogénétique, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Richez, Christophe AU - Richez C AD - Service de Rhumatologie, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. FAU - Blanco, Patrick AU - Blanco P AD - Laboratoire d'Immunologie et Immunogénétique, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire, Place Amélie Raba Léon, 33076 Bordeaux, France; Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France; CNRS-UMR 5164, ImmunoConcept, Université de Bordeaux, 146 rue Léo Saignat, 33076 Bordeaux, France. Electronic address: patrick.blanco@chu-bordeaux.fr. LA - eng PT - Journal Article PT - Review DEP - 20180407 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Antigen-Antibody Complex) SB - IM MH - Antibodies, Antiphospholipid/blood MH - Antigen-Antibody Complex/blood MH - Blood Platelets/immunology/*physiology MH - Humans MH - Lupus Erythematosus, Systemic/blood/*etiology/immunology MH - Raynaud Disease/blood/immunology MH - Scleroderma, Systemic/blood/*etiology/immunology OTO - NOTNLM OT - Auto-immunity OT - Microparticles OT - Platelets OT - Systemic lupus erythematosus OT - Systemic sclerosis EDAT- 2018/04/11 06:00 MHDA- 2018/06/13 06:00 CRDT- 2018/04/11 06:00 PHST- 2018/01/13 00:00 [received] PHST- 2018/01/18 00:00 [accepted] PHST- 2018/04/11 06:00 [pubmed] PHST- 2018/06/13 06:00 [medline] PHST- 2018/04/11 06:00 [entrez] AID - S1568-9972(18)30091-0 [pii] AID - 10.1016/j.autrev.2018.01.012 [doi] PST - ppublish SO - Autoimmun Rev. 2018 Jun;17(6):625-635. doi: 10.1016/j.autrev.2018.01.012. Epub 2018 Apr 7. PMID- 25546651 OWN - NLM STAT- MEDLINE DCOM- 20150527 LR - 20220331 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 67 IP - 4 DP - 2015 Apr TI - Achenbach's syndrome (paroxysmal finger hematoma) with capillaroscopic evidence of microhemorrhages. PG - 1073 LID - 10.1002/art.39003 [doi] FAU - Frerix, Marc AU - Frerix M AD - Justus-Liebig University Giessen, Giessen, Germany and Kerckhoff Clinic Bad Nauheim, Bad Nauheim, Germany. FAU - Richter, Katrin AU - Richter K FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Hermann, Walter AU - Hermann W LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 SB - IM MH - Diagnosis, Differential MH - Female MH - Fingers/*blood supply/pathology MH - Hematoma/*pathology MH - Hemorrhage/*pathology MH - Humans MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*pathology MH - Syndrome EDAT- 2014/12/30 06:00 MHDA- 2015/05/28 06:00 CRDT- 2014/12/30 06:00 PHST- 2014/12/30 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2015/05/28 06:00 [medline] AID - 10.1002/art.39003 [doi] PST - ppublish SO - Arthritis Rheumatol. 2015 Apr;67(4):1073. doi: 10.1002/art.39003. PMID- 16601551 OWN - NLM STAT- MEDLINE DCOM- 20060912 LR - 20080410 IS - 1076-1608 (Print) IS - 1076-1608 (Linking) VI - 12 IP - 2 DP - 2006 Apr TI - RACAND syndrome as a paraneoplastic syndrome? PG - 104-5 FAU - El Mahou, S AU - El Mahou S FAU - Sailler, L AU - Sailler L FAU - Madaule, S AU - Madaule S FAU - Arlet, P AU - Arlet P LA - eng PT - Case Reports PT - Letter PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Autoantibodies/*blood MH - Carcinoma, Small Cell/complications/diagnosis MH - Centromere/*immunology MH - Fingers/*pathology MH - Humans MH - Lung Neoplasms/complications/diagnosis MH - Male MH - Necrosis MH - Paraneoplastic Syndromes/*complications MH - Raynaud Disease/*complications EDAT- 2006/04/08 09:00 MHDA- 2006/09/13 09:00 CRDT- 2006/04/08 09:00 PHST- 2006/04/08 09:00 [pubmed] PHST- 2006/09/13 09:00 [medline] PHST- 2006/04/08 09:00 [entrez] AID - 00124743-200604000-00016 [pii] AID - 10.1097/01.rhu.0000209611.07688.a2 [doi] PST - ppublish SO - J Clin Rheumatol. 2006 Apr;12(2):104-5. doi: 10.1097/01.rhu.0000209611.07688.a2. PMID- 27245654 OWN - NLM STAT- MEDLINE DCOM- 20161110 LR - 20181202 IS - 1524-4539 (Electronic) IS - 0009-7322 (Linking) VI - 133 IP - 22 DP - 2016 May 31 TI - Letter by Boulon and Constans Regarding Article, "Relation of Nailfold Capillaries and Autoantibodies to Mortality in Patients With Raynaud Phenomenon". PG - e668 LID - 10.1161/CIRCULATIONAHA.116.021987 [doi] FAU - Boulon, Carine AU - Boulon C AD - Médecine vasculaire Université de Bordeaux and Hôpital Saint-André Bordeaux, France. FAU - Constans, Joël AU - Constans J AD - Médecine vasculaire Université de Bordeaux and Hôpital Saint-André Bordeaux, France. LA - eng PT - Comment PT - Letter PL - United States TA - Circulation JT - Circulation JID - 0147763 RN - 0 (Autoantibodies) SB - IM CON - Circulation. 2016 Feb 2;133(5):509-17. doi: 10.1161/CIRCULATIONAHA.115.017816. PMID: 26733605 CIN - Circulation. 2016 May 31;133(22):e669. doi: 10.1161/CIRCULATIONAHA.116.022853. PMID: 27245655 MH - *Autoantibodies MH - *Capillaries MH - Connective Tissue Diseases MH - Humans MH - Raynaud Disease EDAT- 2016/06/02 06:00 MHDA- 2016/11/11 06:00 CRDT- 2016/06/02 06:00 PHST- 2016/06/02 06:00 [entrez] PHST- 2016/06/02 06:00 [pubmed] PHST- 2016/11/11 06:00 [medline] AID - CIRCULATIONAHA.116.021987 [pii] AID - 10.1161/CIRCULATIONAHA.116.021987 [doi] PST - ppublish SO - Circulation. 2016 May 31;133(22):e668. doi: 10.1161/CIRCULATIONAHA.116.021987. PMID- 21903346 OWN - NLM STAT- MEDLINE DCOM- 20120203 LR - 20220318 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 36 IP - 10 DP - 2011 Oct TI - Recent advances for the management of Raynaud phenomenon using botulinum neurotoxin A. PG - 1708-10 LID - 10.1016/j.jhsa.2011.07.011 [doi] FAU - Mannava, Sandeep AU - Mannava S AD - Wake Forest University School of Medicine, Department of Orthopaedic Surgery, Medical Center Boulevard, Winston-Salem, NC 27157-1070, USA. smannava@wakehealth.edu FAU - Plate, Johannes F AU - Plate JF FAU - Stone, Austin V AU - Stone AV FAU - Smith, Thomas L AU - Smith TL FAU - Smith, Beth P AU - Smith BP FAU - Koman, L Andrew AU - Koman LA FAU - Tuohy, Christopher J AU - Tuohy CJ LA - eng PT - Journal Article PT - Review DEP - 20110908 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/*administration & dosage/adverse effects MH - Humans MH - Injections MH - Neuromuscular Agents/*administration & dosage MH - Raynaud Disease/*therapy EDAT- 2011/09/10 06:00 MHDA- 2012/02/04 06:00 CRDT- 2011/09/10 06:00 PHST- 2011/06/09 00:00 [received] PHST- 2011/07/12 00:00 [revised] PHST- 2011/07/15 00:00 [accepted] PHST- 2011/09/10 06:00 [entrez] PHST- 2011/09/10 06:00 [pubmed] PHST- 2012/02/04 06:00 [medline] AID - S0363-5023(11)00887-2 [pii] AID - 10.1016/j.jhsa.2011.07.011 [doi] PST - ppublish SO - J Hand Surg Am. 2011 Oct;36(10):1708-10. doi: 10.1016/j.jhsa.2011.07.011. Epub 2011 Sep 8. PMID- 32948347 OWN - NLM STAT- MEDLINE DCOM- 20210625 LR - 20210625 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 46 IP - 1 DP - 2021 Jan TI - Botulinum Toxin for the Treatment of Intractable Raynaud Phenomenon. PG - 54-59 LID - S0363-5023(20)30406-8 [pii] LID - 10.1016/j.jhsa.2020.07.009 [doi] AB - Raynaud phenomenon (RP) is a condition causing vasospasm in the fingers and toes of patients that can have a significant negative impact on quality of life. This can lead to pain, ulceration, and possible loss of digits. Several pharmacological options are available for treatment. However, RP can often be refractory to traditional modalities, leaving surgery or injections as the next available options. This article provides a review and update on the use of botulinum toxin as an effective therapy for the treatment of RP refractory to medical management. CI - Copyright © 2021 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved. FAU - Gallegos, Jose E AU - Gallegos JE AD - College of Medicine, Medical University of South Carolina, Charleston, SC. FAU - Inglesby, Dani C AU - Inglesby DC AD - College of Medicine, Medical University of South Carolina, Charleston, SC. FAU - Young, Zachary T AU - Young ZT AD - College of Medicine, Medical University of South Carolina, Charleston, SC; Division of Plastic and Reconstructive Surgery, Medical University of South Carolina, Charleston, SC. FAU - Herrera, Fernando A AU - Herrera FA AD - College of Medicine, Medical University of South Carolina, Charleston, SC; Division of Plastic and Reconstructive Surgery, Medical University of South Carolina, Charleston, SC; Department of Surgery, Ralph H Johnson Veterans Affairs Medical Center, Charleston, SC. Electronic address: herreraf@musc.edu. LA - eng PT - Journal Article PT - Review DEP - 20200916 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/therapeutic use MH - Fingers MH - Humans MH - *Neuromuscular Agents/therapeutic use MH - Quality of Life MH - *Raynaud Disease/drug therapy OTO - NOTNLM OT - BOTOX OT - Dysport OT - Raynaud OT - botulinum toxin OT - vasospasm EDAT- 2020/09/20 06:00 MHDA- 2021/06/29 06:00 CRDT- 2020/09/19 05:27 PHST- 2020/04/07 00:00 [received] PHST- 2020/06/23 00:00 [revised] PHST- 2020/07/18 00:00 [accepted] PHST- 2020/09/20 06:00 [pubmed] PHST- 2021/06/29 06:00 [medline] PHST- 2020/09/19 05:27 [entrez] AID - S0363-5023(20)30406-8 [pii] AID - 10.1016/j.jhsa.2020.07.009 [doi] PST - ppublish SO - J Hand Surg Am. 2021 Jan;46(1):54-59. doi: 10.1016/j.jhsa.2020.07.009. Epub 2020 Sep 16. PMID- 24124825 OWN - NLM STAT- MEDLINE DCOM- 20131202 LR - 20220330 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 69 IP - 5 DP - 2013 Nov TI - A pilot study evaluating the efficacy of botulinum toxin A in the treatment of Raynaud phenomenon. PG - 834-835 LID - S0190-9622(13)00662-2 [pii] LID - 10.1016/j.jaad.2013.06.029 [doi] FAU - Jenkins, Sasha N AU - Jenkins SN AD - Department of Dermatology, Emory University, Atlanta, Georgia. Electronic address: sasha.nicole.jenkins@emory.edu. FAU - Neyman, Kimberly M AU - Neyman KM AD - Dermatology Associates of Colorado, Aurora, Colorado. FAU - Veledar, Emir AU - Veledar E AD - Department of Dermatology, Emory University, Atlanta, Georgia. FAU - Chen, Suephy C AU - Chen SC AD - Department of Dermatology, Emory University, Atlanta, Georgia. LA - eng PT - Letter PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*therapeutic use MH - Double-Blind Method MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pilot Projects MH - Raynaud Disease/*drug therapy EDAT- 2013/10/16 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/10/16 06:00 PHST- 2013/01/03 00:00 [received] PHST- 2013/06/12 00:00 [revised] PHST- 2013/06/15 00:00 [accepted] PHST- 2013/10/16 06:00 [entrez] PHST- 2013/10/16 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - S0190-9622(13)00662-2 [pii] AID - 10.1016/j.jaad.2013.06.029 [doi] PST - ppublish SO - J Am Acad Dermatol. 2013 Nov;69(5):834-835. doi: 10.1016/j.jaad.2013.06.029. PMID- 37384892 OWN - NLM STAT- MEDLINE DCOM- 20240529 LR - 20260520 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 153 IP - 6 DP - 2024 Jun 1 TI - Neurectomy of the Nerve of Henle Associated with Periarterial Sympathectomy for Management of Intractable Raynaud Phenomenon. PG - 1333-1344 LID - 10.1097/PRS.0000000000010902 [doi] AB - BACKGROUND: In periarterial sympathectomy for intractable Raynaud phenomenon, the extent of adventitectomy and postoperative outcomes and hand perfusion assessment tools remain debatable. The authors evaluated the outcome of neurectomy of the nerve of Henle combined with ulnar tunnel release and periarterial adventitectomy in the treatment of refractory Raynaud phenomenon using objective measurements and patient-reported outcomes. METHODS: Nineteen patients with 20 affected hands were prospectively enrolled and underwent the proposed procedures from 2015 to 2021. Relevant data, including Michigan Hand Outcomes Questionnaire and 36-Item Short Form health questionnaire scores, were documented for analysis during a 3-year follow-up. RESULTS: The average ingress value of the three measured fingers (index, long, and ring) on indocyanine green angiography increased after surgery ( P = 0.02). The median number of ulcers decreased ( P < 0.001), and the median digital skin temperature increased ( P < 0.001). Questionnaire scores showed improvement in physical aspects, such as overall hand function ( P ≤ 0.001), activities of daily living ( P = 0.001), work performance ( P = 0.02), pain ( P < 0.001), physical function ( P = 0.053), and general health ( P = 0.048), and mental aspects, such as patient satisfaction ( P < 0.001) and mental health ( P = 0.001). The average indocyanine green ingress value of the three measured fingers significantly correlated with the patient-reported outcomes, including overall hand function ( r = 0.46, P = 0.04), work performance ( r = 0.68, P = 0.001), physical function ( r = 0.51, P = 0.02), and patient satisfaction ( r = 0.35, P= 0.03). CONCLUSIONS: The proposed surgical procedures provided satisfactory outcomes, both subjectively and objectively, over a follow-up period of up to 3 years. Indocyanine green angiography may provide rapid and quantitative measurements for perioperative hand perfusion assessment. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV. CI - Copyright © 2023 by the American Society of Plastic Surgeons. FAU - Chen, Shih-Heng AU - Chen SH AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Lien, Po-Hao AU - Lien PH AD - Chang-Gung Memorial Hospital, Keelung Branch, Chang-Gung University and Medical College. FAU - Lee, Che-Hsiung AU - Lee CH AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Huang, Ren-Wen AU - Huang RW AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Hsu, Chung-Cheng AU - Hsu CC AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Lin, Cheng-Hung AU - Lin CH AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Lin, Yu-Te AU - Lin YT AD - From the Departments of Plastic and Reconstructive Surgery. FAU - Tsai, Chia-Hsuan AU - Tsai CH AD - Department of Plastic and Reconstructive Surgery. FAU - Tsai, Hsin-I AU - Tsai HI AD - Anesthesiology. FAU - Liu, Yuan-Chang AU - Liu YC AD - Medical Imaging and Intervention, Institute for Radiologic Research, Chang-Gung Memorial Hospital, Linkou Branch. LA - eng PT - Journal Article DEP - 20230627 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM MH - Humans MH - *Sympathectomy/methods MH - *Raynaud Disease/surgery/diagnosis MH - Female MH - Male MH - Adult MH - Middle Aged MH - Treatment Outcome MH - Prospective Studies MH - Follow-Up Studies MH - Patient Reported Outcome Measures MH - Young Adult EDAT- 2023/06/29 19:11 MHDA- 2024/05/29 18:43 CRDT- 2023/06/29 16:23 PHST- 2024/05/29 18:43 [medline] PHST- 2023/06/29 19:11 [pubmed] PHST- 2023/06/29 16:23 [entrez] AID - 00006534-202406000-00027 [pii] AID - 10.1097/PRS.0000000000010902 [doi] PST - ppublish SO - Plast Reconstr Surg. 2024 Jun 1;153(6):1333-1344. doi: 10.1097/PRS.0000000000010902. Epub 2023 Jun 27. PMID- 30270692 OWN - NLM STAT- MEDLINE DCOM- 20190605 LR - 20190605 IS - 1552-6941 (Electronic) IS - 1534-7346 (Linking) VI - 17 IP - 4 DP - 2018 Dec TI - A Rare Cause of Leg Ulcer: Calcinosis Cutis as a Part of CREST Syndrome. PG - 282-284 LID - 10.1177/1534734618799576 [doi] AB - Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient's other serious complaints such as Raynaud's phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease. FAU - Odyakmaz Demirsoy, Evren AU - Odyakmaz Demirsoy E AD - 1 Department of Dermatology, Kocaeli University School of Medicine, Kocaeli, Turkey. FAU - Çatal, Cansu AU - Çatal C AD - 1 Department of Dermatology, Kocaeli University School of Medicine, Kocaeli, Turkey. FAU - Yazıcı, Ayten AU - Yazıcı A AD - 2 Department of Rheumatology, Kocaeli University School of Medicine, Kocaeli, Turkey. FAU - Bayramgurler, Dilek AU - Bayramgurler D AD - 1 Department of Dermatology, Kocaeli University School of Medicine, Kocaeli, Turkey. LA - eng PT - Case Reports PT - Journal Article DEP - 20181001 PL - United States TA - Int J Low Extrem Wounds JT - The international journal of lower extremity wounds JID - 101128359 SB - IM MH - Aged, 80 and over MH - Biopsy, Needle MH - CREST Syndrome/*complications/diagnosis MH - Calcinosis/*complications/diagnosis MH - Combined Modality Therapy MH - Female MH - Humans MH - Hyperbaric Oxygenation/*methods MH - Immunohistochemistry MH - Leg Ulcer/*etiology/physiopathology/*therapy MH - Rare Diseases MH - Severity of Illness Index MH - Skin Transplantation/*methods MH - Treatment Outcome OTO - NOTNLM OT - CREST OT - calcinosis cutis OT - leg ulcer EDAT- 2018/10/03 06:00 MHDA- 2019/06/06 06:00 CRDT- 2018/10/02 06:00 PHST- 2018/10/03 06:00 [pubmed] PHST- 2019/06/06 06:00 [medline] PHST- 2018/10/02 06:00 [entrez] AID - 10.1177/1534734618799576 [doi] PST - ppublish SO - Int J Low Extrem Wounds. 2018 Dec;17(4):282-284. doi: 10.1177/1534734618799576. Epub 2018 Oct 1. PMID- 34556381 OWN - NLM STAT- MEDLINE DCOM- 20220215 LR - 20220215 IS - 1873-569X (Electronic) IS - 0923-1811 (Linking) VI - 104 IP - 1 DP - 2021 Oct TI - Contribution to the peripheral vasculopathy and endothelial cell dysfunction by CXCL4 in Systemic Sclerosis. PG - 63-73 LID - S0923-1811(21)00172-9 [pii] LID - 10.1016/j.jdermsci.2021.07.006 [doi] AB - BACKGROUND: CXCL4, a chemokine with anti-angiogenic property, is involved in systemic sclerosis (SSc) related pulmonary arterial hypertension (PAH). OBJECTIVE: To investigated the contribution of CXCL4 to SSc development by focusing on the correlation of circulatory CXCL4 levels with their peripheral vasculopathy, and the effect of CXCL4 on endothelial cell dysfunction and the potential signaling. METHODS: We measured the plasma CXCL4 levels in 58 patients with SSc, 10 patients with the very early diagnosis of SSc (VEDOSS), and 80 healthy controls (HCs). Then, CXCL4 concentrations were correlated with clinical features, especially the peripheral vasculopathy. These observations were further validated in an additional cohort. Moreover, we studied the anti-angiogenic effects of CXCL4 and the underlying downstream signaling in human umbilical vein endothelial cells (HUVECs) in vitro. RESULTS: Circulating CXCL4 levels were 103.62 % higher in patients with SSc and 201.51 % higher in patients with VEDOSS than matched HCs, which were confirmed in two independent cohorts. CXCL4 levels were associated with digital ulcers (DU) and nailfold videocapillaroscopy (NVC) abnormalities in SSc. The proliferation, migration, and tube formation of HUVECs were inhibited by CXCL4 or SSc derived plasma, which reversed by CXCL4 neutralizing antibody, but failed by CXCR3 inhibitor. CXCL4 downregulated the transcription factor Friend leukaemia integration factor-1 (Fli-1) via c-Abl signaling. Furthermore, CXCL4 blocked the transforming growth factor (TGF) -β or platelet-derived growth factor (PDGF) induced cell proliferation of HUVECs. CONCLUSIONS: CXCL4 may contribute to peripheral vasculopathy in SSc by downregulating Fli-1 via c-Abl signaling in endothelial cells and interfering angiogenesis. CI - Copyright © 2021. Published by Elsevier B.V. FAU - Jiang, Zhixing AU - Jiang Z AD - Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai, China. FAU - Chen, Chen AU - Chen C AD - Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai, China. FAU - Yang, Sen AU - Yang S AD - Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai, China. FAU - He, Hang AU - He H AD - Department of Pancreatic Surgery, Pancreatic Disease Institute, Fudan University, Shanghai, China. Electronic address: hhe10@fudan.edu.cn. FAU - Zhu, Xiaoxia AU - Zhu X AD - Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai, China. Electronic address: xxzhu@unirheuma.org. FAU - Liang, Minrui AU - Liang M AD - Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai, China. Electronic address: lmr_fudan@aliyun.com. LA - eng PT - Journal Article PT - Observational Study DEP - 20210714 PL - Netherlands TA - J Dermatol Sci JT - Journal of dermatological science JID - 9011485 RN - 0 (FLI1 protein, human) RN - 0 (PF4 protein, human) RN - 0 (Proto-Oncogene Protein c-fli-1) RN - 37270-94-3 (Platelet Factor 4) RN - EC 2.7.10.2 (Proto-Oncogene Proteins c-abl) SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Cell Movement MH - Cell Proliferation MH - Early Diagnosis MH - Endothelium, Vascular/immunology/metabolism/*pathology MH - Female MH - Foot Ulcer/blood/diagnosis/*immunology/pathology MH - Healthy Volunteers MH - Human Umbilical Vein Endothelial Cells MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Platelet Factor 4/blood/*metabolism MH - Proto-Oncogene Protein c-fli-1/metabolism MH - Proto-Oncogene Proteins c-abl/metabolism MH - Raynaud Disease/blood/diagnosis/*immunology/pathology MH - Scleroderma, Systemic/blood/*complications/immunology/pathology MH - Signal Transduction/immunology MH - Skin/blood supply/diagnostic imaging/immunology/pathology MH - THP-1 Cells MH - Young Adult OTO - NOTNLM OT - CXCL4 OT - Digital ulcers OT - Fli-1 OT - Systemic sclerosis (SSc) OT - VEDOSS COIS- Declaration of Competing Interest The data presented in this manuscript are original and have not been published or submitted elsewhere. All listed authors have approved the manuscript and agreed with the submission. The authors declare that they have no conflict of interest. EDAT- 2021/09/25 06:00 MHDA- 2022/02/16 06:00 CRDT- 2021/09/24 05:49 PHST- 2020/10/21 00:00 [received] PHST- 2021/06/11 00:00 [revised] PHST- 2021/07/10 00:00 [accepted] PHST- 2021/09/25 06:00 [pubmed] PHST- 2022/02/16 06:00 [medline] PHST- 2021/09/24 05:49 [entrez] AID - S0923-1811(21)00172-9 [pii] AID - 10.1016/j.jdermsci.2021.07.006 [doi] PST - ppublish SO - J Dermatol Sci. 2021 Oct;104(1):63-73. doi: 10.1016/j.jdermsci.2021.07.006. Epub 2021 Jul 14. PMID- 20035332 OWN - NLM STAT- MEDLINE DCOM- 20110815 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 31 IP - 5 DP - 2011 May TI - Primary Raynaud phenomenon and small-fiber neuropathy: is there a connection? A pilot neurophysiologic study. PG - 577-85 LID - 10.1007/s00296-009-1293-9 [doi] AB - The pathophysiologic factors of primary Raynaud phenomenon (RP) are unknown. Preliminary evidence from skin biopsy suggests small-fiber neuropathy (SFN) in primary RP. We aimed to quantitatively assess SFN in participants with primary RP. Consecutive patients with an a priori diagnosis of primary RP presenting to our outpatient rheumatology clinic over a 6-month period were invited to participate. Cases of secondary RP were excluded. All participants were required to have normal results on nailfold capillary microscopy. Assessment for SFN was accomplished with autonomic reflex screening, which includes quantitative sudomotor axonal reflex test (QSART), and cardiovagal and adrenergic function testing, thermoregulatory sweat test (TST), and quantitative sensory test (QST) for vibratory, cooling, and heat-pain sensory thresholds. Nine female participants with a median age of 38 years (range 21-46 years) and a median symptom duration of 9 years (range 5 months-31 years) were assessed. Three participants had abnormal results on QSART, indicating peripheral sudomotor autonomic dysfunction. Two participants had evidence of large-fiber involvement with heat-pain thresholds on QST. Heart rate and blood pressure responses to deep breathing, Valsalva maneuver, and 70° tilt were normal for all participants. Also, all participants had normal TST results. In total, three of the nine participants had evidence of SFN. The presence of SFN raises the possibility that a subset of patients with primary RP have an underlying, subclinical small-fiber dysfunction. These data open new avenues of research and therapeutics for this common condition. FAU - Manek, Nisha J AU - Manek NJ AD - Mayo Clinic, Rochester, MN 55905, USA. manek.nisha@mayo.edu FAU - Holmgren, Aaron R AU - Holmgren AR FAU - Sandroni, Paola AU - Sandroni P FAU - Osborn, Thomas G AU - Osborn TG FAU - Davis, Mark D P AU - Davis MD LA - eng PT - Journal Article DEP - 20091225 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM CIN - Rheumatol Int. 2011 Jul;31(7):977-8. doi: 10.1007/s00296-010-1491-5. PMID: 20390278 MH - Adult MH - Ambulatory Care Facilities MH - Autonomic Nervous System/pathology/*physiopathology MH - Baroreflex MH - Blood Pressure MH - Electromyography MH - Female MH - Heart Rate MH - Humans MH - Middle Aged MH - Minnesota MH - *Nerve Fibers/pathology MH - Neural Conduction MH - Neurologic Examination MH - Pain Threshold MH - Peripheral Nervous System Diseases/pathology/*physiopathology MH - Pilot Projects MH - Raynaud Disease/pathology/*physiopathology MH - Reflex, Abnormal MH - Sweating MH - Thermosensing MH - Tilt-Table Test MH - Valsalva Maneuver MH - Young Adult EDAT- 2009/12/26 06:00 MHDA- 2011/08/16 06:00 CRDT- 2009/12/26 06:00 PHST- 2009/09/25 00:00 [received] PHST- 2009/11/29 00:00 [accepted] PHST- 2009/12/26 06:00 [entrez] PHST- 2009/12/26 06:00 [pubmed] PHST- 2011/08/16 06:00 [medline] AID - 10.1007/s00296-009-1293-9 [doi] PST - ppublish SO - Rheumatol Int. 2011 May;31(5):577-85. doi: 10.1007/s00296-009-1293-9. Epub 2009 Dec 25. PMID- 34132891 OWN - NLM STAT- MEDLINE DCOM- 20220110 LR - 20220218 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 41 IP - 8 DP - 2021 Aug TI - The effect of vitamin D3 and thyroid hormones on the capillaroscopy-confirmed microangiopathy in pediatric patients with a suspicion of systemic connective tissue disease-a single-center experience with Raynaud phenomenon. PG - 1485-1493 LID - 10.1007/s00296-021-04919-y [doi] AB - Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. The study analyzed the nail-fold video-capillaroscopy (NVC) findings and laboratory results of 81 children between the ages 6 and 17 who were referred to pediatric rheumatologist with a suspicion of SCTD. Out of 52 patients presenting with RP at the time of evaluation, abnormalities in capillary microscopic imaging were found in 34. NVC findings were then compared to levels of specific biomarkers in serum. Vitamin D3 serum levels have been significantly decreased in patients with RP (23.4 ng/mL ± 8.76 vs. 30.0 ng/mL ± 12.66, P = 0.0148). There were positive significant correlations between levels of vitamin D3 and acute-phase reactants in serum, such as C-reactive protein (P = 0.0292). Furthermore, free thyroxine levels (fT4) in patients with both RP (P = 0.0126) and micro-angiopathy (P = 0.05496) persisted in the lower range of the normal limit (< 1.0 ng/dL). Regular oral supplementation of vitamin D3 should be always considered in children with RP if deficiency is found. Additionally, low fT4 level (< 1.0 ng/dL) should be considered as an indication to perform NVC in patients suspected of SCTD even when they do not present RP. FAU - Kapten, Katarzyna AU - Kapten K AUID- ORCID: 0000-0001-5676-7233 AD - Department of Pediatric Cardiology and Rheumatology, Central Teaching Hospital of Medical University of Lodz, Lodz, Poland. FAU - Orczyk, Krzysztof AU - Orczyk K AUID- ORCID: 0000-0001-7294-1676 AD - Department of Pediatric Cardiology and Rheumatology, Medical University of Lodz, Sporna 36/50, 91-738, Lodz, Poland. FAU - Smolewska, Elzbieta AU - Smolewska E AUID- ORCID: 0000-0002-8421-0448 AD - Department of Pediatric Cardiology and Rheumatology, Medical University of Lodz, Sporna 36/50, 91-738, Lodz, Poland. e.smolewska@wp.pl. LA - eng GR - 503/8-000-01/503-81-001-19-00/Uniwersytet Medyczny w Lodzi/ PT - Journal Article PT - Observational Study DEP - 20210616 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Biomarkers) RN - 1C6V77QF41 (Cholecalciferol) RN - Q51BO43MG4 (Thyroxine) SB - IM MH - Adolescent MH - Biomarkers/blood MH - Child MH - Cholecalciferol/blood/*deficiency MH - Connective Tissue Diseases/*blood/diagnosis MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Raynaud Disease/*blood/diagnosis MH - Retrospective Studies MH - Thyroxine/blood/*deficiency PMC - PMC8207495 OTO - NOTNLM OT - Capillaroscopy OT - Microangiopathy OT - Raynaud phenomenon OT - Thyroid hormones OT - Vitamin D3 OT - fT4 COIS- The Dino-Lite Capillaryscope 200 Pro was donated by AbbVie Pharmaceutical Research & Development. The authors declare no other competing interests. EDAT- 2021/06/17 06:00 MHDA- 2022/01/11 06:00 PMCR- 2021/06/16 CRDT- 2021/06/16 12:27 PHST- 2021/03/24 00:00 [received] PHST- 2021/06/09 00:00 [accepted] PHST- 2021/06/17 06:00 [pubmed] PHST- 2022/01/11 06:00 [medline] PHST- 2021/06/16 12:27 [entrez] PHST- 2021/06/16 00:00 [pmc-release] AID - 10.1007/s00296-021-04919-y [pii] AID - 4919 [pii] AID - 10.1007/s00296-021-04919-y [doi] PST - ppublish SO - Rheumatol Int. 2021 Aug;41(8):1485-1493. doi: 10.1007/s00296-021-04919-y. Epub 2021 Jun 16. PMID- 23032649 OWN - NLM STAT- MEDLINE DCOM- 20130228 LR - 20131121 IS - 1542-6270 (Electronic) IS - 1060-0280 (Linking) VI - 46 IP - 10 DP - 2012 Oct TI - Paradoxical reaction of raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis. PG - e28 LID - 10.1345/aph.1R093 [doi] AB - OBJECTIVE: To report a paradoxical reaction of Raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis with vascular involvement. CASE SUMMARY: In January 2006, a 40-year-old male was diagnosed with diffuse cutaneous systemic sclerosis with pulmonary, esophageal, cutaneous, and vascular involvement (Raynaud phenomenon, with digital ulcers on his hands). In December 2008, treatment with iloprost was started due to worsening disease. Nine cycles of iloprost were administered at a rate of 0.5-1 ng/kg/min (6 hours per day, for 5 days every 6-8 weeks); the patient tolerated this treatment well. However, on the fourth day of cycles 10 and 11, the patient developed paradoxical Raynaud phenomenon in the hand with perfusion when the infusion was increased to 1 ng/kg/min, requiring treatment to be stopped. Treatment was continued during cycles 12 and 13 at 0.5 ng/kg/min; the patient tolerated the treatment well, although paradoxical Raynaud phenomenon occurred when the rate of infusion was increased. DISCUSSION: Raynaud phenomenon is extremely common in patients with scleroderma, and often is severe. Iloprost has vasodilating, antiplatelet, cytoprotective, and immunomodulating properties, and has been found to be an efficacious alternative to nifedipine for the treatment of Raynaud phenomenon in patients with scleroderma. The Naranjo probability scale indicated that iloprost was the probable cause of the paradoxical Raynaud phenomenon in this patient. CONCLUSIONS: This case demonstrates a probable relationship between the rate of infusion of iloprost and the paradoxical reaction of Raynaud phenomenon. FAU - Barreira, Rebeca Iglesias AU - Barreira RI AD - Department of Pharmacy, Ferrol Health District, Spain. rebeca_ib@sefh.es FAU - García, Belén Bardán AU - García BB FAU - López, Mónica Granero AU - López MG FAU - Legazpi, Iria Rodríguez AU - Legazpi IR FAU - Díaz, Hortensia Álvarez AU - Díaz HÁ FAU - Penín, Isaura Rodríguez AU - Penín IR LA - eng PT - Case Reports PT - Journal Article DEP - 20121002 PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Humans MH - Iloprost/administration & dosage/*adverse effects MH - Male MH - Raynaud Disease/*chemically induced MH - Scleroderma, Diffuse/*drug therapy MH - Vasodilator Agents/administration & dosage/*adverse effects EDAT- 2012/10/04 06:00 MHDA- 2013/03/01 06:00 CRDT- 2012/10/04 06:00 PHST- 2012/10/04 06:00 [entrez] PHST- 2012/10/04 06:00 [pubmed] PHST- 2013/03/01 06:00 [medline] AID - aph.1R093 [pii] AID - 10.1345/aph.1R093 [doi] PST - ppublish SO - Ann Pharmacother. 2012 Oct;46(10):e28. doi: 10.1345/aph.1R093. Epub 2012 Oct 2. PMID- 20682742 OWN - NLM STAT- MEDLINE DCOM- 20110915 LR - 20151119 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 60 IP - 7 DP - 2010 Oct TI - Vibration-white foot: a case report. PG - 572-4 LID - 10.1093/occmed/kqq107 [doi] AB - BACKGROUND: Hand-arm vibration syndrome (HAVS) refers to the neurological, vascular and musculoskeletal problems that may arise due to exposure to segmental vibration to the hands. An analogous syndrome may occur in the lower extremities of workers exposed to foot-transmitted vibration. AIMS: This report describes the case of a worker with a history of foot-transmitted vibration exposure presenting with cold intolerance in the feet and blanching in the toes. Case report A 54-year-old miner presented with a chief complaint of blanching and pain in his toes. The worker had a history of foot-transmitted vibration exposure over his 18 year career as a miner, primarily from the operation of vehicle-mounted bolting machines. Cold provocation digital plethysmography showed cold-induced vasospastic disease in the feet, but not in the hands. CONCLUSIONS: This case illustrates a condition descriptively termed 'vibration-white foot': a disease analogous to HAVS arising after segmental vibration exposure to the feet. Further research is required to increase awareness of, and direct preventive efforts for, this potentially debilitating condition. FAU - Thompson, A M S AU - Thompson AM AD - Department of Occupational and Environmental Medicine, St. Michael's Hospital,Toronto, Ontario M5B 1WB, Canada. aaron.thompson@utoronto.ca FAU - House, R AU - House R FAU - Krajnak, K AU - Krajnak K FAU - Eger, T AU - Eger T LA - eng PT - Case Reports PT - Journal Article DEP - 20100803 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Cold Temperature/*adverse effects MH - Diagnosis, Differential MH - Foot Diseases/diagnosis/*etiology MH - Humans MH - Male MH - Middle Aged MH - *Mining MH - Occupational Exposure/*adverse effects MH - Plethysmography MH - Raynaud Disease/diagnosis/*etiology MH - Vibration/*adverse effects EDAT- 2010/08/05 06:00 MHDA- 2011/09/16 06:00 CRDT- 2010/08/05 06:00 PHST- 2010/08/05 06:00 [entrez] PHST- 2010/08/05 06:00 [pubmed] PHST- 2011/09/16 06:00 [medline] AID - kqq107 [pii] AID - 10.1093/occmed/kqq107 [doi] PST - ppublish SO - Occup Med (Lond). 2010 Oct;60(7):572-4. doi: 10.1093/occmed/kqq107. Epub 2010 Aug 3. PMID- 20309697 OWN - NLM STAT- MEDLINE DCOM- 20100803 LR - 20260128 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 69 IP - 3 DP - 2010 May TI - [Capillaroscopy. Procedure and nomenclature]. PG - 253-62 LID - 10.1007/s00393-010-0618-0 [doi] AB - Capillaroscopy has high diagnostic and prognostic value in autoimmune connective tissue diseases, in particular systemic sclerosis (SSc). Our working group has developed a consensus on nomenclature, technical equipment, procedure, and diagnostic interpretation of results. The following are required: binocular microscopes with at least 20-/50- and 160-/200-fold magnification and digital archiving. Documentation of defined findings is mandatory. The simultaneous occurrence of, e.g. caliber variations, ectasia, ramifications, elongation (length > 350 microm), torsion (at least two crossing segments per capillary loop), sludge, hemorrhage, and edema is of pathological significance. The isolated occurrence of bushy capillaries (multiple ramifications), thrombosis, giant capillary (capillary lumen > 50 microm), and avascular areas also indicates disease. The latter two findings are highly specific for SSc. Other findings are consistent with connective tissue diseases. These standardized definitions increase quality and comparability of nailfold capillaroscopy in Germany. FAU - Sander, O AU - Sander O AD - Klinik für Endokrinologie, Diabetologie und Rheumatologie, Heinrich-Heine-Universität Düsseldorf, Moorenstr. 5, 40225 Düsseldorf, Deutschland. Sander@rheumanet.org FAU - Sunderkötter, C AU - Sunderkötter C FAU - Kötter, I AU - Kötter I FAU - Wagner, I AU - Wagner I FAU - Becker, M AU - Becker M FAU - Herrgott, I AU - Herrgott I FAU - Schwarting, A AU - Schwarting A FAU - Ostendorf, B AU - Ostendorf B FAU - Iking-Konert, C AU - Iking-Konert C FAU - Genth, E AU - Genth E LA - ger PT - Consensus Statement PT - English Abstract PT - Journal Article TT - Kapillarmikroskopie. Durchführung und Nomenklatur. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Adolescent MH - Age Factors MH - Antiphospholipid Syndrome/classification/diagnosis MH - Capillaries/pathology MH - Child MH - Connective Tissue Diseases/classification/*diagnosis MH - Dermatomyositis/classification/diagnosis MH - Disease Progression MH - Documentation/methods/standards MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/pathology MH - Microscopic Angioscopy/instrumentation/methods/*standards MH - Prognosis MH - Raynaud Disease/classification/*diagnosis MH - Reference Standards MH - Reference Values MH - Scleroderma, Systemic/classification/*diagnosis MH - *Terminology as Topic RF - 36 EDAT- 2010/03/24 06:00 MHDA- 2010/08/04 06:00 CRDT- 2010/03/24 06:00 PHST- 2010/03/24 06:00 [entrez] PHST- 2010/03/24 06:00 [pubmed] PHST- 2010/08/04 06:00 [medline] AID - 10.1007/s00393-010-0618-0 [doi] PST - ppublish SO - Z Rheumatol. 2010 May;69(3):253-62. doi: 10.1007/s00393-010-0618-0. PMID- 19487218 OWN - NLM STAT- MEDLINE DCOM- 20090706 LR - 20220330 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 48 Suppl 3 DP - 2009 Jun TI - Digital ulcers: overt vascular disease in systemic sclerosis. PG - iii19-24 LID - 10.1093/rheumatology/kep105 [doi] AB - RP is an almost universal manifestation of SSc, with 95% of all patients being affected, and resulting in digital ulcers (DUs) in approximately 30% of the patients each year. DUs are a major clinical problem, being associated with substantial morbidity (reduced quality of life, pain, disability and disfigurement) that can escalate to gangrene and amputation. Ideally, the treatment of DUs would improve tissue integrity and viability, promote ulcer healing and reduce the formation of new ulcers. Treatments that have shown potential include calcium channel blockers, prostacyclin analogues and endothelin receptor antagonists. However, until recently, management was based on empirical experience. The recent approval (in Europe) of the dual endothelin receptor antagonist, bosentan, to reduce the number of new DUs in patients with SSc and ongoing DU disease, means that there is now an approved therapy--and new hope--for the treatment of DUs in these severely afflicted patients. FAU - Steen, V AU - Steen V AD - Department of Medicine, Georgetown University Medical Center, Washington, DC 20007, USA. steenv@georgetown.edu FAU - Denton, C P AU - Denton CP FAU - Pope, J E AU - Pope JE FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Bosentan MH - Databases, Factual MH - Endothelium, Vascular/physiopathology MH - Fingers/blood supply MH - Hand Dermatoses/*etiology/physiopathology MH - Humans MH - Raynaud Disease/*etiology/physiopathology MH - Scleroderma, Systemic/*complications/physiopathology/therapy MH - Skin Ulcer/*etiology/physiopathology MH - Sulfonamides/therapeutic use RF - 18 EDAT- 2009/06/12 09:00 MHDA- 2009/07/07 09:00 CRDT- 2009/06/03 09:00 PHST- 2009/06/03 09:00 [entrez] PHST- 2009/06/12 09:00 [pubmed] PHST- 2009/07/07 09:00 [medline] AID - kep105 [pii] AID - 10.1093/rheumatology/kep105 [doi] PST - ppublish SO - Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii19-24. doi: 10.1093/rheumatology/kep105. PMID- 22580491 OWN - NLM STAT- MEDLINE DCOM- 20121023 LR - 20121105 IS - 1537-8918 (Electronic) IS - 1537-890X (Linking) VI - 11 IP - 3 DP - 2012 May-Jun TI - Cold weather issues in sideline and event management. PG - 135-41 LID - 10.1249/JSR.0b013e3182578783 [doi] AB - Exercise in cold environments exerts a unique physiologic stress on the human body, which, under certain conditions, may result in a cold-related injury. Environmental factors are the most important risk factors for the development of hypothermia in athletes. Frostbite occurs as a result of direct cold injury to peripheral tissues. The biggest risk for frostbite is temperature. Trench foot is a result of repeated and constant immersion in cold water. Chilblains are local erythematous or cyanotic skin lesions that develop at ambient air temperatures of 32°F to 60°F after an exposure time of about 1 to 5 h. Cold urticaria is, essentially, an allergic reaction to a cold exposure and can be controlled with avoidance of the cold. There are a number of risk factors and conditions that predispose athletes to cold injury, but exercise in the cold can be done safely with proper education and planning. FAU - McMahon, J Andrew AU - McMahon JA AD - Maine Medical Center, Portland, Maine 04105, USA. FAU - Howe, Allyson AU - Howe A LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Sports Med Rep JT - Current sports medicine reports JID - 101134380 SB - IM MH - Body Temperature/*physiology MH - Cold Temperature/*adverse effects MH - Frostbite/etiology/therapy MH - Humans MH - Hypothermia/etiology/therapy MH - Immersion Foot/etiology/therapy MH - Raynaud Disease/etiology/therapy MH - Urticaria/etiology MH - Wounds and Injuries/*etiology/*prevention & control EDAT- 2012/05/15 06:00 MHDA- 2012/10/24 06:00 CRDT- 2012/05/15 06:00 PHST- 2012/05/15 06:00 [entrez] PHST- 2012/05/15 06:00 [pubmed] PHST- 2012/10/24 06:00 [medline] AID - 00149619-201205000-00009 [pii] AID - 10.1249/JSR.0b013e3182578783 [doi] PST - ppublish SO - Curr Sports Med Rep. 2012 May-Jun;11(3):135-41. doi: 10.1249/JSR.0b013e3182578783. PMID- 28933138 OWN - NLM STAT- MEDLINE DCOM- 20180710 LR - 20181202 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 69 IP - 3 DP - 2017 Sep 21 TI - Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis. PG - 134-136 LID - 10.4081/reumatismo.2017.986 [doi] AB - Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment. FAU - Ruaro, B AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa. barbara.ruaro@yahoo.it. FAU - Paolino, S AU - Paolino S FAU - Pizzorni, C AU - Pizzorni C FAU - Cutolo, M AU - Cutolo M FAU - Sulli, A AU - Sulli A LA - eng PT - Journal Article DEP - 20170921 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Sulfonamides) RN - 0 (para-Aminobenzoates) RN - 03JLX11PE9 (aminaftone) RN - 39J1LGJ30J (Tramadol) RN - JED5K35YGL (Iloprost) RN - Q326023R30 (Bosentan) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Bosentan MH - Female MH - Fingers/blood supply/*diagnostic imaging MH - Humans MH - Iloprost/therapeutic use MH - Lasers MH - Methotrexate/therapeutic use MH - Microcirculation MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*diagnostic imaging/etiology MH - Scattering, Radiation MH - Scleroderma, Systemic/complications/*diagnostic imaging/drug therapy MH - Skin Ulcer/*diagnostic imaging/etiology MH - Sulfonamides/therapeutic use MH - Tramadol/therapeutic use MH - Treatment Outcome MH - para-Aminobenzoates/therapeutic use EDAT- 2017/09/22 06:00 MHDA- 2018/07/11 06:00 CRDT- 2017/09/22 06:00 PHST- 2017/03/20 00:00 [received] PHST- 2017/07/04 00:00 [accepted] PHST- 2017/06/27 00:00 [revised] PHST- 2017/09/22 06:00 [entrez] PHST- 2017/09/22 06:00 [pubmed] PHST- 2018/07/11 06:00 [medline] AID - 10.4081/reumatismo.2017.986 [doi] PST - epublish SO - Reumatismo. 2017 Sep 21;69(3):134-136. doi: 10.4081/reumatismo.2017.986. PMID- 19837535 OWN - NLM STAT- MEDLINE DCOM- 20091221 LR - 20121003 IS - 1097-6809 (Electronic) IS - 0741-5214 (Linking) VI - 50 IP - 6 DP - 2009 Dec TI - Prevalence, risk factors, and clinical correlates of ulnar artery occlusion in the general population. PG - 1333-9 LID - 10.1016/j.jvs.2009.07.076 [doi] AB - BACKGROUND: Occlusion of the ulnar artery is found in a substantial proportion of elderly patients. The aim of this study was to estimate the prevalence of ulnar artery occlusion in a sample of the general population of France, look for its risk factors, and evaluate its clinical correlates. METHODS: This study was an offshoot of a cross-sectional epidemiologic study in the general population of four locations in France (Tarentaise, Grenoble, Nyons, and Toulon). In phase I, random samples of 2000 individuals per location aged >or=18 years old were interviewed by phone for screening of Raynaud phenomenon. In phase II, subsamples of individuals were invited to a medical interview and physical examination where the presence of Raynaud phenomenon and occupational risk factors were recorded and a bilateral clinical Allen test was performed for the detection of ulnar artery occlusion. Phase II comprised 688 women and 335 men. RESULTS: In 36 men and seven women, at least one occluded ulnar artery was found. The estimated prevalence was 9.6% in men and 1.0% in women (P < .001). The occluded artery was more often in the dominant hand of both men (8.1% vs 2.4%; P < .001) and women (0.9% vs 0.4%; P = .34). Ulnar artery occlusion was found more often in men aged >50 years (16.4%) than in younger men (1.4%; P < .001). Besides age, male sex, and dominant side, the only independent risk factor was an occupational exposure in men to repeated palmar trauma, with a significant quantitative relationship in the frequency of the impacts (P < .001) and the duration of the exposure (P < .001). Exposures to hand-held vibrating tools and cigarette smoking did not show a significant relationship in the multivariate analysis. Most individuals with ulnar artery occlusion did not have associated complaints; however, the diagnostic criteria for Raynaud phenomenon was validated in 13 of the 36 affected men. The association remained significant after adjusting for occupational exposure to vibrating tools. One individual reported a previous episode consistent with an attack of permanent digital ischemia. CONCLUSION: This study confirms a substantial prevalence of ulnar artery occlusions in the general population, mostly in middle-aged and elderly men, which appears to be principally related to an occupational exposure to repeated occupational palmar trauma. Although there is a significant association with Raynaud phenomenon, most often the consequences of this occlusion remain subclinical. FAU - Carpentier, Patrick H AU - Carpentier PH AD - Clinique Universitaire de Médecine Vasculaire, Pôle Pluridisciplinaire de Médecine, Centre Hospitalier Universitaire, Grenoble, France. patrick.carpentier@ujf-grenoble.fr FAU - Biro, Christine AU - Biro C FAU - Jiguet, Myriam AU - Jiguet M FAU - Maricq, Hildegard R AU - Maricq HR LA - eng GR - AR-31283/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20091017 PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Adult MH - Age Distribution MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Arterial Occlusive Diseases/complications/*epidemiology/etiology/physiopathology MH - Constriction, Pathologic MH - Cross-Sectional Studies MH - Female MH - France/epidemiology MH - Functional Laterality MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Occupational Diseases/complications/*epidemiology/etiology/physiopathology MH - Occupational Exposure MH - Odds Ratio MH - Population Surveillance MH - Prevalence MH - Raynaud Disease/*epidemiology/etiology/physiopathology MH - Regional Blood Flow MH - Risk Assessment MH - Risk Factors MH - Sex Distribution MH - Sex Factors MH - *Ulnar Artery/physiopathology MH - Vibration/adverse effects EDAT- 2009/10/20 06:00 MHDA- 2009/12/22 06:00 CRDT- 2009/10/20 06:00 PHST- 2007/07/24 00:00 [received] PHST- 2009/07/14 00:00 [revised] PHST- 2009/07/15 00:00 [accepted] PHST- 2009/10/20 06:00 [entrez] PHST- 2009/10/20 06:00 [pubmed] PHST- 2009/12/22 06:00 [medline] AID - S0741-5214(09)01484-0 [pii] AID - 10.1016/j.jvs.2009.07.076 [doi] PST - ppublish SO - J Vasc Surg. 2009 Dec;50(6):1333-9. doi: 10.1016/j.jvs.2009.07.076. Epub 2009 Oct 17. PMID- 21779969 OWN - NLM STAT- MEDLINE DCOM- 20120504 LR - 20220321 IS - 1432-1041 (Electronic) IS - 0031-6970 (Print) IS - 0031-6970 (Linking) VI - 68 IP - 1 DP - 2012 Jan TI - Dopamine agonists and ischemic complications in Parkinson's disease: a nested case-control study. PG - 83-8 LID - 10.1007/s00228-011-1084-6 [doi] AB - BACKGROUND: It has been suggested that ergoline dopamine agonists can cause ischemic complications. The effect of dopamine agonists in general on the prevalence of ischemic events in patients with Parkinson's disease (PD) has not been studied. OBJECTIVE: Our aim was to investigate the association between the use of dopamine agonists and hospitalization due to ischemic events in patients with PD. METHODS: We performed a nested case-control study using the PHARMO Institute for Drug Outcome Research database. All patients issued at least one prescription for levodopa after the age of 55 years between 1994 and 2006 were initially identified. Cases were patients who were hospitalized for the first time after November 1997 for an ischemic event and were matched to as many as four controls. Exposure to dopamine agonists during the year preceding the index date was identified. RESULTS: The study population consisted of 542 cases and 2,155 controls. The mean effect of dopamine agonist use 1 year prior to the index date on ischemic events requiring hospitalization is shown with 95% probability in the 0.95-1.49 range. Stratified results according to the type of dopamine agonist showed no risk differences between ergoline and nonergoline agonists. CONCLUSIONS: This study does not support an association between dopamine agonist use and an increased risk of ischemic events requiring hospitalization. FAU - Arbouw, Maurits E L AU - Arbouw ME AD - Department of Clinical Pharmacy, Division Laboratory and Pharmacy, University Medical Center Utrecht, PO Box 85500, 3508 GA, Utrecht, The Netherlands. FAU - Movig, Kris L L AU - Movig KL FAU - Guchelaar, Henk-Jan AU - Guchelaar HJ FAU - Neef, Cees AU - Neef C FAU - Egberts, Toine C G AU - Egberts TC LA - eng PT - Journal Article DEP - 20110722 PL - Germany TA - Eur J Clin Pharmacol JT - European journal of clinical pharmacology JID - 1256165 RN - 0 (Antiparkinson Agents) RN - 0 (Dopamine Agonists) RN - 0 (Ergolines) RN - 46627O600J (Levodopa) SB - IM MH - Aged MH - Aged, 80 and over MH - Antiparkinson Agents/*adverse effects/therapeutic use MH - Brain Ischemia/chemically induced/etiology/therapy MH - Case-Control Studies MH - Databases, Factual MH - Dopamine Agonists/*adverse effects/therapeutic use MH - Drug Prescriptions MH - Ergolines/adverse effects/therapeutic use MH - Female MH - Hospitalization MH - Humans MH - Ischemia/*chemically induced/etiology/therapy MH - Levodopa/therapeutic use MH - Male MH - Myocardial Ischemia/chemically induced/etiology/therapy MH - Netherlands/epidemiology MH - Parkinson Disease/*drug therapy/physiopathology MH - Practice Patterns, Physicians' MH - Prevalence MH - Raynaud Disease/chemically induced/etiology/therapy MH - Severity of Illness Index PMC - PMC3249169 EDAT- 2011/07/23 06:00 MHDA- 2012/05/05 06:00 PMCR- 2011/07/22 CRDT- 2011/07/23 06:00 PHST- 2011/02/17 00:00 [received] PHST- 2011/06/07 00:00 [accepted] PHST- 2011/07/23 06:00 [entrez] PHST- 2011/07/23 06:00 [pubmed] PHST- 2012/05/05 06:00 [medline] PHST- 2011/07/22 00:00 [pmc-release] AID - 1084 [pii] AID - 10.1007/s00228-011-1084-6 [doi] PST - ppublish SO - Eur J Clin Pharmacol. 2012 Jan;68(1):83-8. doi: 10.1007/s00228-011-1084-6. Epub 2011 Jul 22. PMID- 17717029 OWN - NLM STAT- MEDLINE DCOM- 20080310 LR - 20071030 IS - 0931-0509 (Print) IS - 0931-0509 (Linking) VI - 22 IP - 11 DP - 2007 Nov TI - Chronic urticaria and mesangial proliferative glomerulonephritis: a case report. PG - 3327-9 FAU - Merkle, Monika AU - Merkle M AD - Nephrologie, Klinikum Traunstein, D-83278 Traunstein, Germany. FAU - Weiss, Max AU - Weiss M FAU - Wörnle, Markus AU - Wörnle M LA - eng PT - Case Reports PT - Journal Article DEP - 20070823 PL - England TA - Nephrol Dial Transplant JT - Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association JID - 8706402 RN - 0 (Cardiolipins) RN - 0 (Complement C4) RN - 0 (Immunoglobulin G) SB - IM MH - Adult MH - Biopsy MH - Cardiolipins/immunology MH - Chronic Disease MH - Complement C4/analysis MH - Female MH - Glomerulonephritis, Membranoproliferative/*diagnosis/pathology MH - Humans MH - Immunoglobulin G/blood MH - Kidney/pathology MH - Raynaud Disease/complications MH - Urticaria/*etiology EDAT- 2007/08/25 09:00 MHDA- 2008/03/11 09:00 CRDT- 2007/08/25 09:00 PHST- 2007/08/25 09:00 [pubmed] PHST- 2008/03/11 09:00 [medline] PHST- 2007/08/25 09:00 [entrez] AID - gfm548 [pii] AID - 10.1093/ndt/gfm548 [doi] PST - ppublish SO - Nephrol Dial Transplant. 2007 Nov;22(11):3327-9. doi: 10.1093/ndt/gfm548. Epub 2007 Aug 23. PMID- 18077494 OWN - NLM STAT- MEDLINE DCOM- 20080110 LR - 20131121 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 1 DP - 2008 Jan TI - Vasodilator iontophoresis a possible new therapy for digital ischaemia in systemic sclerosis? PG - 76-9 AB - OBJECTIVE: This study investigated whether whole finger vasodilator iontophoresis increases digital blood flow in patients with systemic sclerosis (SSc): If so, this might indicate a novel approach to therapy. METHODS: Eight patients and 8 healthy controls underwent whole finger iontophoresis using a specially designed chamber. Treatment was with 0.5% sodium nitroprusside (NaNP) or 1% acetylcholine chloride (ACh), and the procedure then repeated with the other vasodilator (randomly assigned order). Three treatments were carried out for each chemical; 2 min treatments were carried out bilaterally at 200 microA, a third was then carried out for 5 min on one digit only (randomly assigned to left or right). Blood flow increases were monitored with laser Doppler imaging (LDI). Maximum perfusion increase from baseline (MAX) and the area under the time perfusion curve (AUC), normalized for baseline, were calculated. Data were compared with a three-way analysis of variance test. RESULTS: Perfusion increased in both patients and controls, but significantly more so in controls (P(MAX) = 0.001, P(AUC) = 0.005, respectively). Values were significantly higher for the 5 min treatment compared with the 2 min treatment (P(MAX) = 0.011 and P(AUC) = 0.008 for both groups). No significant differences were found between the use of NaNP and ACh. CONCLUSIONS: The increased perfusion with both ACh and NaNP in the patient group (albeit to a lesser degree than in the control group) indicates that this local approach to vasodilation is effective. Increasing iontophoresis time causes more sustained vasodilation. Further studies are indicated to investigate a possible therapeutic effect in patients with severe digital ischaemia. FAU - Murray, A K AU - Murray AK AD - Rheumatic Diseases Centre, Hope Hospital, Salford M6 8HD, UK. andrea.murray@manchester.ac.uk FAU - Moore, T L AU - Moore TL FAU - King, T A AU - King TA FAU - Herrick, A L AU - Herrick AL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Vasodilator Agents) RN - 169D1260KM (Nitroprusside) RN - N9YNS0M02X (Acetylcholine) SB - IM MH - Acetylcholine/therapeutic use MH - Adult MH - Aged MH - Female MH - Fingers/*blood supply MH - Humans MH - Iontophoresis/*methods MH - Ischemia/etiology/physiopathology/*therapy MH - Male MH - Microcirculation/drug effects/physiopathology MH - Middle Aged MH - Nitroprusside/therapeutic use MH - Raynaud Disease/etiology/physiopathology/*therapy MH - Scleroderma, Systemic/complications/physiopathology/*therapy MH - Vasodilation/physiology MH - Vasodilator Agents/*therapeutic use EDAT- 2007/12/14 09:00 MHDA- 2008/01/11 09:00 CRDT- 2007/12/14 09:00 PHST- 2007/12/14 09:00 [pubmed] PHST- 2008/01/11 09:00 [medline] PHST- 2007/12/14 09:00 [entrez] AID - 47/1/76 [pii] AID - 10.1093/rheumatology/kem314 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Jan;47(1):76-9. doi: 10.1093/rheumatology/kem314. PMID- 34997227 OWN - NLM STAT- MEDLINE DCOM- 20220322 LR - 20220322 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 18 IP - 2 DP - 2022 Feb TI - Evaluating early diagnostic criteria for SSc. PG - 62 LID - 10.1038/s41584-021-00745-5 [doi] FAU - Phillips, Robert AU - Phillips R AD - Nature Reviews Rheumatology, . nrrheum@nature.com. LA - eng PT - Journal Article PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - Humans MH - *Raynaud Disease MH - *Scleroderma, Systemic/diagnosis EDAT- 2022/01/09 06:00 MHDA- 2022/03/23 06:00 CRDT- 2022/01/08 06:04 PHST- 2022/01/09 06:00 [pubmed] PHST- 2022/03/23 06:00 [medline] PHST- 2022/01/08 06:04 [entrez] AID - 10.1038/s41584-021-00745-5 [pii] AID - 10.1038/s41584-021-00745-5 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2022 Feb;18(2):62. doi: 10.1038/s41584-021-00745-5. PMID- 20436078 OWN - NLM STAT- MEDLINE DCOM- 20100708 LR - 20230215 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 6 DP - 2010 Jun TI - Brachial artery endothelial-dependent flow-mediated dilation identifies early-stage endothelial dysfunction in systemic sclerosis and correlates with nailfold microvascular impairment. PG - 1168-73 LID - 10.3899/jrheum.091116 [doi] AB - OBJECTIVE: To assess possible correlations between endothelial-dependent flow-mediated dilation (FMD) of the brachial artery and nailfold videocapillaroscopy (NVC) in patients with systemic sclerosis (SSc). Evidence has shown that vascular impairment in SSc may be a sign of endothelial dysfunction involving both microvascular and macrovascular systems, although the pathological mechanisms of the dysfunction are poorly understood. METHODS: Forty-three consecutive patients (mean age 51 +/- 11 yrs) with SSc were studied. Thirty patients had limited cutaneous SSc, 13 had diffuse cutaneous SSc. Twenty-seven healthy subjects (mean age 48 +/- 8 yrs) were recruited as controls. Ultrasound assessment of FMD was performed on all subjects in order to evaluate macrovascular function. Patients were divided into 3 patterns of microvascular damage on the basis of NVC (early, active, and late), and the microangiopathy evolution score was calculated, as reported elsewhere. RESULTS: FMD was significantly reduced in patients with SSc compared to healthy subjects [median 8.0% (3.0%-9.0%) vs 15.0% (12.0%-16.0%), respectively; p < 0.0001]. Patients with an early pattern of microangiopathy showed reduced FMD values compared to controls (p = 0.0001). FMD was significantly reduced in patients with SSc who had the late NVC pattern of microangiopathy compared to active and early patterns (p = 0.003 and p = 0.001, respectively). FMD was inversely correlated with the microvascular damage rate in patients with SSc (p < 0.0001). CONCLUSION: We demonstrated the simultaneous presence of macrovascular and microvascular impairment in patients with SSc, which was already present in the early phase of the vascular disease. FAU - Rollando, Daniela AU - Rollando D AD - Academic Unit of Cardiovascular Diseases, Department of Internal Medicine, University of Genoa, Genoa, Italy. FAU - Bezante, Gian Paolo AU - Bezante GP FAU - Sulli, Alberto AU - Sulli A FAU - Balbi, Manrico AU - Balbi M FAU - Panico, Nicoletta AU - Panico N FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Negrini, Simone AU - Negrini S FAU - Brunelli, Claudio AU - Brunelli C FAU - Barsotti, Antonio AU - Barsotti A FAU - Cutolo, Maurizio AU - Cutolo M FAU - Indiveri, Francesco AU - Indiveri F FAU - Ghio, Massimo AU - Ghio M LA - eng PT - Journal Article DEP - 20100501 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Blood Flow Velocity MH - Brachial Artery/*pathology/physiopathology MH - Capillaries/pathology MH - Endothelium, Vascular/*pathology/physiopathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/etiology/*pathology/physiopathology MH - Scleroderma, Diffuse/complications/*diagnosis/physiopathology MH - Scleroderma, Limited/complications/*diagnosis/physiopathology EDAT- 2010/05/04 06:00 MHDA- 2010/07/09 06:00 CRDT- 2010/05/04 06:00 PHST- 2010/05/04 06:00 [entrez] PHST- 2010/05/04 06:00 [pubmed] PHST- 2010/07/09 06:00 [medline] AID - jrheum.091116 [pii] AID - 10.3899/jrheum.091116 [doi] PST - ppublish SO - J Rheumatol. 2010 Jun;37(6):1168-73. doi: 10.3899/jrheum.091116. Epub 2010 May 1. PMID- 32144624 OWN - NLM STAT- MEDLINE DCOM- 20210409 LR - 20210409 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 39 IP - 8 DP - 2020 Aug TI - Clinical features and outcomes of the patients with anti-glycyl tRNA synthetase syndrome. PG - 2417-2424 LID - 10.1007/s10067-020-04979-8 [doi] AB - OBJECTIVE: To analyze the clinical features and outcomes of the patients with anti-glycyl tRNA synthetase (anti-EJ) syndrome in a large Chinese cohort. METHODS: The medical records, imaging, and serologic data of patients with anti-EJ antibodies from the China-Japan Friendship Hospital database were retrospectively investigated. Anti-EJ antibodies were identified using immunoblot assay. Long-term follow-up was conducted. RESULTS: Anti-EJ antibodies were present in 46 (19.7%) of the 234 patients with antisynthetase syndrome (ASS), preceded by anti-Jo-1 and anti-PL-7 antibodies. The mean age of disease onset was 51.2 ± 15.9 years, and 69.6% of these patients were female. The most prevalent clinical feature was interstitial lung disease (ILD) (96.7%), which was also the most common initial manifestation, followed by fever (60.9%), mechanic's hands (56.5%), muscle involvement (50%), and Raynaud phenomenon (8.7%). Ten (21.7%) patients developed rapidly progressive ILD (RP-ILD). Organizing pneumonia (OP) on high-resolution computed tomography (HRCT) scans (OR = 37.5, p = 0.006) and a higher C-reactive protein-to-albumin ratio (CAR) (OR = 28.3, p = 0.01) were independent risk factors for RP-ILD. Muscular pathological features were heterogeneous. Concomitant infection was noted in 63.0% of the patients during the disease course. Hypoalbuminemia (OR = 0.759, p = 0.002) was an independent risk factor for concomitant infection. Patients responded well to glucocorticoid therapy. The 5- and 10-year survival rates of the patients with anti-EJ were 97.8% and 88%, respectively. CONCLUSION: Anti-EJ syndrome was found to be a relatively common ASS subtype, with a favorable outcome. A notable proportion of the patients experienced RP-ILD, which was prone to OP on HRCT and a higher CAR, and needed aggressive management. Key Points • ILD was the most common initial manifestation of anti-glycyl tRNA synthetase syndrome. • RP-ILD was notable in anti-EJ positive patients. • Anti-EJ positive patients possessed a favorable long-term prognosis, but easily relapsed. FAU - Zhang, Yinli AU - Zhang Y AUID- ORCID: 0000-0001-5596-4518 AD - Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China. FAU - Ge, Yongpeng AU - Ge Y AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. FAU - Yang, Hanbo AU - Yang H AUID- ORCID: 0000-0002-0665-4692 AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. FAU - Chen, He AU - Chen H AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. FAU - Tian, Xiaolan AU - Tian X AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. FAU - Huang, Zhenguo AU - Huang Z AD - Department of Radiology, China-Japan Friendship Hospital, Beijing, China. FAU - Liu, Shengyun AU - Liu S AD - Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China. FAU - Lu, Xin AU - Lu X AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. FAU - Wang, Guochun AU - Wang G AUID- ORCID: 0000-0002-4616-9376 AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. guochunwang@hotmail.com. LA - eng GR - 81571603/National Natural Science Foundation of China/ GR - Z181100001718063, Z171100001017208/Beijing Municipal Science and Technology Commission/ PT - Journal Article DEP - 20200306 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Glucocorticoids) RN - EC 6.1.1.14 (Glycine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Aged MH - Autoantibodies/*immunology MH - China MH - Disease Progression MH - Female MH - Glucocorticoids/therapeutic use MH - Glycine-tRNA Ligase/immunology MH - Humans MH - Lung Diseases, Interstitial/drug therapy/immunology/*physiopathology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Myositis/drug therapy/immunology/*physiopathology MH - Prognosis MH - Raynaud Disease/drug therapy/immunology/*physiopathology MH - Retrospective Studies MH - Survival Analysis MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Anti-EJ antibodies OT - Interstitial lung disease OT - Prognosis EDAT- 2020/03/08 06:00 MHDA- 2021/04/10 06:00 CRDT- 2020/03/08 06:00 PHST- 2019/09/25 00:00 [received] PHST- 2020/02/07 00:00 [accepted] PHST- 2020/01/13 00:00 [revised] PHST- 2020/03/08 06:00 [pubmed] PHST- 2021/04/10 06:00 [medline] PHST- 2020/03/08 06:00 [entrez] AID - 10.1007/s10067-020-04979-8 [pii] AID - 10.1007/s10067-020-04979-8 [doi] PST - ppublish SO - Clin Rheumatol. 2020 Aug;39(8):2417-2424. doi: 10.1007/s10067-020-04979-8. Epub 2020 Mar 6. PMID- 34237004 OWN - NLM STAT- MEDLINE DCOM- 20210831 LR - 20210831 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 48 IP - 3 DP - 2021 Mar TI - Raynaud Phenomenon With Lingual Involvement in a 6-year-old Female. PG - 465-466 LID - 10.3899/jrheum.200258 [doi] FAU - Hunter, Julia A AU - Hunter JA AD - Julia A. Hunter, MB BCh BAO; Deborah M. Levy, MD, MS, FRCPC; Roberta A. Berard, MD, MSc, FRCPC, Division of Pediatric Rheumatology, Western University, London, Ontario, Canada. FAU - Levy, Deborah M AU - Levy DM AUID- ORCID: 0000-0003-1023-9499 AD - Julia A. Hunter, MB BCh BAO; Deborah M. Levy, MD, MS, FRCPC; Roberta A. Berard, MD, MSc, FRCPC, Division of Pediatric Rheumatology, Western University, London, Ontario, Canada. FAU - Berard, Roberta A AU - Berard RA AUID- ORCID: 0000-0003-2780-0617 AD - Julia A. Hunter, MB BCh BAO; Deborah M. Levy, MD, MS, FRCPC; Roberta A. Berard, MD, MSc, FRCPC, Division of Pediatric Rheumatology, Western University, London, Ontario, Canada. Roberta.berard@lhsc.on.ca. LA - eng PT - Case Reports PT - Journal Article PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Child MH - Female MH - Humans MH - *Raynaud Disease MH - Tongue EDAT- 2021/07/09 06:00 MHDA- 2021/09/01 06:00 CRDT- 2021/07/08 17:23 PHST- 2021/07/08 17:23 [entrez] PHST- 2021/07/09 06:00 [pubmed] PHST- 2021/09/01 06:00 [medline] AID - 48/3/465 [pii] AID - 10.3899/jrheum.200258 [doi] PST - ppublish SO - J Rheumatol. 2021 Mar;48(3):465-466. doi: 10.3899/jrheum.200258. PMID- 19301062 OWN - NLM STAT- MEDLINE DCOM- 20090806 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 7 DP - 2009 Jul TI - A score of risk factors associated with ischemic digital ulcers in patients affected by systemic sclerosis treated with iloprost. PG - 807-13 LID - 10.1007/s10067-009-1155-6 [doi] AB - A single series of patients affected by systemic sclerosis (SSc) and cyclically treated with iloprost was reviewed in order to evaluate the incidence of digital ulcers (DUs) and to compare the characteristics between the patients with and without this painful and disabling vascular complication. The record charts of 85 SSc patients were revised. Ischemic DUs and scleroderma contracture ulcers were separately considered. Twenty-nine subjects developed ischemic DUs during the course of the disease; whereas, scleroderma contracture ulcers occurred in six subjects. Ischemic DUs were associated with younger age at scleroderma onset, a longer disease duration, a longer time delay from scleroderma diagnosis to iloprost therapy, a bigger skin involvement, the presence of joint contractures, a videocapillaroscopic late pattern, a history of smoking, and of corticosteroids therapy. After the exclusion of four subjects with concomitant peripheral arterial disease, a forward-stepwise logistic regression analysis showed that only four variables, i.e., age at scleroderma onset, delay in beginning iloprost therapy, history of smoking, and presence of joint contractures remained significantly associated with ischemic DUs. In a score reflecting the sum of these four risk factors, the prevalence of ischemic DUs increased progressively from the lowest to the highest value of the score. The predictivity of this model was evaluated by the receiver-operating characteristics curve, with an estimated area under the curve of 0.836 with 95% confidence interval from 0.736 to 0.937. All the patients with scleroderma contracture ulcers were characterized by both diffuse pattern of disease and positivity for anti-Scl70 antibody. In this retrospective study, scleroderma patients with ischemic DUs are characterized by early disease onset, delay in beginning iloprost therapy, smoking habit, and presence of joint contraction. A score reflecting the sum of these factors may be useful to predict the risk of developing ischemic DUs. FAU - Caramaschi, Paola AU - Caramaschi P AD - Dipartimento di Medicina Clinica e Sperimentale, Università di Verona, Policlinico G.B. Rossi, Piazzale Scuro, 37134, Verona, Italy. paola.caramaschi@azosp.vr.it FAU - Martinelli, Nicola AU - Martinelli N FAU - Volpe, Alessandro AU - Volpe A FAU - Pieropan, Sara AU - Pieropan S FAU - Tinazzi, Ilaria AU - Tinazzi I FAU - Patuzzo, Giuseppe AU - Patuzzo G FAU - Mahamid, Helal AU - Mahamid H FAU - Bambara, Lisa Maria AU - Bambara LM FAU - Biasi, Domenico AU - Biasi D LA - eng PT - Journal Article DEP - 20090320 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Female MH - Fingers/blood supply MH - Humans MH - Iloprost/*therapeutic use MH - Ischemia/drug therapy/*etiology/physiopathology MH - Male MH - Middle Aged MH - ROC Curve MH - Raynaud Disease/drug therapy/*etiology/physiopathology MH - Risk Factors MH - Scleroderma, Systemic/*complications/drug therapy/physiopathology MH - Skin/blood supply MH - Ulcer/drug therapy/*etiology/physiopathology MH - Vasodilator Agents/*therapeutic use EDAT- 2009/03/21 09:00 MHDA- 2009/08/07 09:00 CRDT- 2009/03/21 09:00 PHST- 2008/12/21 00:00 [received] PHST- 2009/02/25 00:00 [accepted] PHST- 2009/02/24 00:00 [revised] PHST- 2009/03/21 09:00 [entrez] PHST- 2009/03/21 09:00 [pubmed] PHST- 2009/08/07 09:00 [medline] AID - 10.1007/s10067-009-1155-6 [doi] PST - ppublish SO - Clin Rheumatol. 2009 Jul;28(7):807-13. doi: 10.1007/s10067-009-1155-6. Epub 2009 Mar 20. PMID- 25303107 OWN - NLM STAT- MEDLINE DCOM- 20150422 LR - 20181202 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 67 IP - 3 DP - 2015 Mar TI - Anti-RNA polymerase III autoantibody-positive scleroderma as a paraneoplastic syndrome: comment on the article by Stojan et al. PG - 453 LID - 10.1002/acr.22489 [doi] FAU - Bachmeyer, Claude AU - Bachmeyer C AD - Service de Médecine Interne, Hôpital Tenon, Assistance Publique Hôpitaux de Paris, Paris, France. FAU - Rein, Christopher AU - Rein C LA - eng PT - Comment PT - Letter PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM CON - Arthritis Care Res (Hoboken). 2014 Aug;66(8):1263-8. doi: 10.1002/acr.22331. PMID: 24664962 CIN - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453-4. doi: 10.1002/acr.22491. PMID: 25303330 MH - Arthritis, Rheumatoid/*etiology MH - Carcinoma, Non-Small-Cell Lung/*diagnosis MH - Female MH - Humans MH - Lung Neoplasms/*diagnosis MH - Raynaud Disease/*etiology MH - *Weight Loss EDAT- 2014/10/11 06:00 MHDA- 2015/04/23 06:00 CRDT- 2014/10/11 06:00 PHST- 2014/10/11 06:00 [entrez] PHST- 2014/10/11 06:00 [pubmed] PHST- 2015/04/23 06:00 [medline] AID - 10.1002/acr.22489 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2015 Mar;67(3):453. doi: 10.1002/acr.22489. PMID- 24751944 OWN - NLM STAT- MEDLINE DCOM- 20150421 LR - 20140725 IS - 1421-9832 (Electronic) IS - 1018-8665 (Linking) VI - 228 IP - 4 DP - 2014 TI - Systemic sclerosis with normal or nonspecific nailfold capillaroscopy. PG - 360-7 LID - 10.1159/000360159 [doi] AB - BACKGROUND: In systemic sclerosis (SSc), a specific nailfold videocapillaroscopy (NVC) pattern is observed in 90% of cases and seems to be associated with severity and progression of the disease. OBJECTIVE: To describe the characteristics of SSc patients with normal or nonspecific (normal/nonspecific) NVC. METHODS: In a retrospective cohort study, clinical features and visceral involvements of 25 SSc cases with normal/nonspecific NVC were compared to 63 SSc controls with the SSc-specific NVC pattern. RESULTS: Normal/nonspecific NVC versus SSc-specific NVC pattern was significantly associated with absence of skin sclerosis (32 vs. 6.3%, p = 0.004), absence of telangiectasia (47.8 vs. 17.3%, p = 0.006) and absence of sclerodactyly (60 vs. 25.4%, p = 0.002), and less frequent severe pulmonary involvement (26.3 vs. 58.2%, p = 0.017). CONCLUSION: Normal/nonspecific NVC in SSc patients appears to be associated with less severe skin involvement and less frequent severe pulmonary involvement. CI - © 2014 S. Karger AG, Basel. FAU - Fichel, Fanny AU - Fichel F AD - Department of Dermatology, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. FAU - Baudot, Nathalie AU - Baudot N FAU - Gaitz, Jean-Pierre AU - Gaitz JP FAU - Trad, Salim AU - Trad S FAU - Barbe, Coralie AU - Barbe C FAU - Francès, Camille AU - Francès C FAU - Senet, Patricia AU - Senet P LA - eng PT - Journal Article DEP - 20140415 PL - Switzerland TA - Dermatology JT - Dermatology (Basel, Switzerland) JID - 9203244 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/blood MH - Female MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Microscopy, Video MH - Microvessels/*pathology MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/immunology/*pathology MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/immunology/*pathology MH - Severity of Illness Index MH - Telangiectasis/pathology EDAT- 2014/04/23 06:00 MHDA- 2015/04/22 06:00 CRDT- 2014/04/23 06:00 PHST- 2013/11/18 00:00 [received] PHST- 2014/01/29 00:00 [accepted] PHST- 2014/04/23 06:00 [entrez] PHST- 2014/04/23 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] AID - 000360159 [pii] AID - 10.1159/000360159 [doi] PST - ppublish SO - Dermatology. 2014;228(4):360-7. doi: 10.1159/000360159. Epub 2014 Apr 15. PMID- 17543693 OWN - NLM STAT- MEDLINE DCOM- 20070713 LR - 20121003 IS - 0741-5214 (Print) IS - 0741-5214 (Linking) VI - 45 IP - 6 DP - 2007 Jun TI - Unusual etiology of upper extremity ischemia in a scleroderma patient: thoracic outlet syndrome with arterial embolization. PG - 1259-61 AB - Although the causes of digital ischemia and necrosis are diverse in women, the most common etiology is connective tissue disease. We describe a patient with scleroderma who presented with arm ischemia secondary to arterial embolization from thoracic outlet syndrome. Her sublavian artery was compressed by the anterior scalene muscle and a cervical rib, leading to a stenosis with poststenotic dilation of the artery. Within the aneurysmal formation was a thrombus, which was probably the source of the distal embolization. The patient underwent surgical resection of the cervical and first rib. The abnormal portion of the subclavian artery was resected and replaced with an interposition graft. FAU - Hugl, Beate AU - Hugl B AD - Section of Vascular Surgery, Department of Rheumatology, Mayo Clinic Jacksonville, Jacksonville, FL 32224, USA. beate.hugl@i-med.ac.at FAU - Oldenburg, W Andrew AU - Oldenburg WA FAU - Hakaim, Albert G AU - Hakaim AG FAU - Persellin, Scott T AU - Persellin ST LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Blood Vessel Prosthesis Implantation MH - Diagnosis, Differential MH - Embolism/*complications/etiology/pathology MH - Female MH - Humans MH - Ischemia/etiology/pathology MH - Middle Aged MH - Raynaud Disease/*etiology/pathology MH - Scleroderma, Diffuse/*complications MH - Subclavian Steal Syndrome/*complications/etiology/pathology/surgery MH - Thoracic Outlet Syndrome/*complications/diagnosis/etiology/pathology/surgery MH - Treatment Outcome MH - Upper Extremity/*blood supply EDAT- 2007/06/05 09:00 MHDA- 2007/07/14 09:00 CRDT- 2007/06/05 09:00 PHST- 2006/11/17 00:00 [received] PHST- 2007/01/11 00:00 [accepted] PHST- 2007/06/05 09:00 [pubmed] PHST- 2007/07/14 09:00 [medline] PHST- 2007/06/05 09:00 [entrez] AID - S0741-5214(07)00056-0 [pii] AID - 10.1016/j.jvs.2007.01.040 [doi] PST - ppublish SO - J Vasc Surg. 2007 Jun;45(6):1259-61. doi: 10.1016/j.jvs.2007.01.040. PMID- 21118406 OWN - NLM STAT- MEDLINE DCOM- 20130708 LR - 20151119 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 42 IP - 5 DP - 2012 May TI - Platelet activation in patients with the Raynaud phenomenon. PG - 531-5 LID - 10.1111/j.1445-5994.2010.02399.x [doi] AB - BACKGROUND: The Raynaud phenomenon (RP) is an exaggerated and reversible vasospasm of small arteries triggered by cold or emotional stress. Primary RP (PRP) term is used when the underlying condition is unknown. An altered regulation in vascular tone and/or release of soluble mediators from activated platelets plays a role in PRP through an increased oxidative stress. We assessed platelet activation and oxidative stress in patients with PRP by measuring platelet PAC-1, an index of glycoprotein (Gp) IIb/IIIa receptor activation, thromboxane A(2) (TXA(2)), an index of platelet activation and 8-epi-prostaglandin F(2α) (8-epi-PGF(2α)), a marker of endogenous in vivo peroxidation. METHODS: Eighteen asymptomatic patients with PRP (age 41.37 ± 16.94 years; 17 women, 1 man) and 18 healthy subjects (age of 35.11 ± 13.16 years; 16 women, 2 men) were studied. PAC-1 was analysed by flow cytometry while circulating TXB(2) , a stable metabolite of TXA(2) and 8-epi-PGF(2α) levels were assessed by ELISA kit. RESULTS: Our results show a significant platelet activation in PRP patients as indicated by increased PAC-1 expression (65.29 ± 15.24%; P < 0.001), TXB(2) (1477.83 ± 454.04 pg/mL; P= 0.003) and 8-epi-PGF(2α) circulating levels (42.50 ± 14.14 ng/mL; P < 0.001). An inverse correlation between the degree of PAC-1 expression and TXB(2) levels (r=-0.527; P= 0.02) was also found in PRP patients, suggesting that downregulation of GpIIb/IIIa receptor expression may occur during thrombocytopoiesis, as a consequence of the chronic exposure to increased TXB(2) concentration. CONCLUSIONS: Our study for the first time shows a marked activation of GpIIb/IIIa receptor in asymptomatic patients with PRP and supports antiplatelet therapy in PRP patients. CI - © 2010 The Authors. Internal Medicine Journal © 2010 Royal Australasian College of Physicians. FAU - Polidoro, L AU - Polidoro L AD - University of L'Aquila, Department of Internal Medicine and Public Health, S. Salvatore Hospital, L'Aquila, Italy. FAU - Barnabei, R AU - Barnabei R FAU - Giorgini, P AU - Giorgini P FAU - Petrazzi, L AU - Petrazzi L FAU - Ferri, C AU - Ferri C FAU - Properzi, G AU - Properzi G LA - eng PT - Journal Article PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - 0 (Biomarkers) RN - 0 (Platelet Glycoprotein GPIIb-IIIa Complex) SB - IM MH - Adult MH - Biomarkers/blood/metabolism MH - Cohort Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Platelet Activation/*physiology MH - Platelet Glycoprotein GPIIb-IIIa Complex/*metabolism MH - Raynaud Disease/*blood/diagnosis MH - Young Adult EDAT- 2010/12/02 06:00 MHDA- 2013/07/09 06:00 CRDT- 2010/12/02 06:00 PHST- 2010/12/02 06:00 [entrez] PHST- 2010/12/02 06:00 [pubmed] PHST- 2013/07/09 06:00 [medline] AID - 10.1111/j.1445-5994.2010.02399.x [doi] PST - ppublish SO - Intern Med J. 2012 May;42(5):531-5. doi: 10.1111/j.1445-5994.2010.02399.x. PMID- 17657684 OWN - NLM STAT- MEDLINE DCOM- 20070809 LR - 20070727 IS - 0300-9742 (Print) IS - 0300-9742 (Linking) VI - 36 IP - 3 DP - 2007 May-Jun TI - A patient with antisynthetase syndrome associated with deforming arthritis and periarticular calcinosis sine myositis. PG - 239-41 FAU - Oztürk, M A AU - Oztürk MA FAU - Unverdi, S AU - Unverdi S FAU - Goker, B AU - Goker B FAU - Haznedaroglu, S AU - Haznedaroglu S FAU - Tunç, L AU - Tunç L LA - eng PT - Case Reports PT - Letter PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - EC 6.- (Ligases) SB - IM MH - Antibodies, Antinuclear/*blood MH - Arthritis/*immunology MH - Calcinosis/*immunology MH - Female MH - Humans MH - Ligases/*immunology MH - Lung Diseases, Interstitial/*immunology MH - Middle Aged MH - Myositis/*immunology MH - Raynaud Disease/*immunology MH - Syndrome EDAT- 2007/07/28 09:00 MHDA- 2007/08/10 09:00 CRDT- 2007/07/28 09:00 PHST- 2007/07/28 09:00 [pubmed] PHST- 2007/08/10 09:00 [medline] PHST- 2007/07/28 09:00 [entrez] AID - 780896908 [pii] AID - 10.1080/03009740600902437 [doi] PST - ppublish SO - Scand J Rheumatol. 2007 May-Jun;36(3):239-41. doi: 10.1080/03009740600902437. PMID- 18172572 OWN - NLM STAT- MEDLINE DCOM- 20080617 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 4 DP - 2008 Apr TI - Anti-cyclic citrullinated peptide-2 (CCP2) autoantibodies and extra-articular manifestations in Greek patients with rheumatoid arthritis. PG - 511-3 LID - 10.1007/s10067-007-0800-1 [doi] AB - The objective of our study was to establish whether there is an association between rheumatoid arthritis with extra-articular manifestations (exRA) and anti-cyclic citrullinated peptide 2 (anti-CCP2) antibodies in Greeks. A retrospective study of 220 Greek patients with RA, 95 with exRA and 125 without extra-articular manifestations (cRA). Serum anti-CCP2 antibodies and IgM rheumatoid factor (RF) were measured. CCP2(+) were 65.3% of exRA and 58.4% of cRA patients. RF(+) were 69.5% of exRA and 60.0% of cRA patients. Among exRA patients, 37.9% had high serum anti-CCP2 antibody levels (>100 IU/ml) compared to 21.6% cRA patients (p = 0.008). Serositis and pulmonary fibrosis were found to be associated with high levels of anti-CCP2 antibodies (52.9 vs 26.6%, p = 0.02 and 63.6 vs 26.8%, p = 0.008, respectively). Serum RF levels were 265.0 +/- 52.0 IU/ml (mean +/- SEM) in exRA and 205.1 +/- 40.6 (mean +/- SEM) in cRA (NS). High serum RF levels (>268 IU/ml) were more likely to have sicca syndrome. In Greek patients with rheumatoid arthritis (RA), high serum anti-CCP2 antibodies are associated with serositis and pulmonary fibrosis. Therefore, anti-CCP2 antibodies have prognostic significance in patients with RA. FAU - Alexiou, Ioannis AU - Alexiou I AD - Department of Medicine/Rheumatology, University Hospital of Larisa, Larisa, Greece. FAU - Germenis, Anastasios AU - Germenis A FAU - Koutroumpas, Athanasios AU - Koutroumpas A FAU - Kontogianni, Anastasia AU - Kontogianni A FAU - Theodoridou, Katerina AU - Theodoridou K FAU - Sakkas, Lazaros I AU - Sakkas LI LA - eng PT - Journal Article DEP - 20080103 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Peptides, Cyclic) RN - 0 (cyclic citrullinated peptide) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Aged MH - Arthritis, Rheumatoid/*complications/ethnology/*immunology MH - Autoantibodies/*blood MH - Felty Syndrome/diagnosis/etiology/immunology MH - Greece/ethnology MH - Humans MH - Middle Aged MH - Peptides, Cyclic/*immunology MH - Prognosis MH - Pulmonary Fibrosis/diagnosis/*etiology/immunology MH - Raynaud Disease/diagnosis/etiology/immunology MH - Retrospective Studies MH - Rheumatoid Factor/immunology MH - Rheumatoid Nodule/diagnosis/etiology/immunology MH - Serositis/diagnosis/*etiology/immunology MH - Sjogren's Syndrome/diagnosis/etiology/immunology MH - Vasculitis/diagnosis/etiology/immunology EDAT- 2008/01/04 09:00 MHDA- 2008/06/18 09:00 CRDT- 2008/01/04 09:00 PHST- 2007/10/18 00:00 [received] PHST- 2007/11/09 00:00 [accepted] PHST- 2007/10/30 00:00 [revised] PHST- 2008/01/04 09:00 [pubmed] PHST- 2008/06/18 09:00 [medline] PHST- 2008/01/04 09:00 [entrez] AID - 10.1007/s10067-007-0800-1 [doi] PST - ppublish SO - Clin Rheumatol. 2008 Apr;27(4):511-3. doi: 10.1007/s10067-007-0800-1. Epub 2008 Jan 3. PMID- 17891412 OWN - NLM STAT- MEDLINE DCOM- 20090421 LR - 20220310 IS - 1432-1246 (Electronic) IS - 0340-0131 (Linking) VI - 81 IP - 5 DP - 2008 Apr TI - Use of color charts for the diagnosis of finger whiteness in vibration-exposed workers. PG - 633-8 AB - OBJECTIVES: To assess the usefulness of color charts for the diagnosis of finger whiteness in vibration-exposed workers. METHODS: A group of 146 forestry and stone workers exposed to hand-transmitted vibration (HTV) were examined twice over 1 year follow up period. The anamnestic diagnosis of finger whiteness was made on the basis of (a) a medical history alone, and (b) the administration color charts which showed changes in the skin color of fingers and hands. The cold response of digital arteries was assessed by measuring the change in finger systolic blood pressure (FSBP) after local cooling from 30 to 10 degrees C (FSBP%(10 degrees )). RESULTS: Assuming the administration of color charts as the gold standard, the sensitivity and specificity of the medical history alone to diagnose finger whiteness was 88.2 and 93.8%, respectively, at the initial cross-sectional study and 94.4 and 97.7% at the end of the follow-up. Random-intercept linear regression analysis of follow up data showed that after adjustment for several covariates, FSBP%(10 degrees )was significantly associated with finger whiteness assessed by either medical history alone (P < 0.005) or the color charts (P < 0.001). However, a statistical measure of overall fit of regression models (Bayesian Information Criterion) suggested that the color chart method performed better than medical history alone for the prediction of the cold response of digital arteries. CONCLUSION: The administration of color charts seems to reduce the proportion of false positive responses for finger whiteness in a population of vibration-exposed workers. The color chart method was a more significant predictor of digital arterial hyperresponsiveness to cold than medical history alone. These findings suggest that the use of color charts in clinical and epidemiological studies may be of help to assist in the diagnosis of finger whiteness in vibration-exposed workers. FAU - Negro, Corrado AU - Negro C AD - Department of Public Health Sciences, University of Trieste, Centro Tumori, Trieste, Italy. negro@units.it FAU - Rui, Francesca AU - Rui F FAU - D'Agostin, Flavia AU - D'Agostin F FAU - Bovenzi, Massimo AU - Bovenzi M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070922 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - Adult MH - Blood Pressure MH - Cold Temperature MH - Color MH - Fingers/*blood supply MH - Forestry MH - Hand-Arm Vibration Syndrome/*diagnosis/etiology/physiopathology MH - Humans MH - Raynaud Disease/*diagnosis/etiology/physiopathology MH - Vibration/*adverse effects EDAT- 2007/09/25 09:00 MHDA- 2009/04/22 09:00 CRDT- 2007/09/25 09:00 PHST- 2007/08/10 00:00 [received] PHST- 2007/09/05 00:00 [accepted] PHST- 2007/09/25 09:00 [pubmed] PHST- 2009/04/22 09:00 [medline] PHST- 2007/09/25 09:00 [entrez] AID - 10.1007/s00420-007-0248-2 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2008 Apr;81(5):633-8. doi: 10.1007/s00420-007-0248-2. Epub 2007 Sep 22. PMID- 20661065 OWN - NLM STAT- MEDLINE DCOM- 20101216 LR - 20151119 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 16 IP - 5 DP - 2010 Aug TI - Clues for previously undiagnosed connective tissue disease in patients with trigeminal neuralgia. PG - 205-8 LID - 10.1097/RHU.0b013e3181e928e6 [doi] AB - BACKGROUND: Connective tissue diseases (CTD) may be associated with idiopathic trigeminal neuralgia (TN). The prevalence and diagnostic implications of this association are, however, not well established. OBJECTIVES: The objective of this study was to evaluate, in TN patients, if rheumatologic clinical and laboratory findings could contribute to the early diagnosis of rheumatic diseases. METHODS: Forty-six consecutive TN patients, 67% female, mean disease duration 8.78 +/- 7.25 years, and 47 controls were initially interviewed using a standard questionnaire based on common signs/symptoms of systemic lupus erythematosus, Sjögren syndrome, mixed CTD, and systemic sclerosis. Autoantibodies were detected by standard techniques. Those with rheumatologic complaints or positive autoantibodies were referred to the Rheumatology Outpatient Clinic for a more detailed evaluation. Secondary causes of TN were excluded. RESULTS: The frequency of Raynaud phenomenon (P = 0.026) and ANA reactivity (P = 0.04) were significantly higher in TN patients compared with controls. Fourteen TN patients were ANA positive. Seven of them reported concomitant rheumatic complaints, and interestingly, diffuse CTD was diagnosed in 4 (57%) of these patients: 1 systemic lupus erythematosus; 2 Sjögren syndrome; and 1 undifferentiated disease with scleritis and positive parotid scintigraphy. In all cases, TN preceded by at least 10 months the rheumatologic signs/symptoms. Moreover, these 4 TN patients with CTD had a higher frequency of sicca symptoms (P = 0.001) and higher titers of ANA (>or=1:320) (P = 0.006) than the remaining 42 TN patients without CTD diagnoses. Sixteen patients had isolated laboratory or clinical abnormalities, and none of them had CTD diagnoses. CONCLUSIONS: The concomitant presence of sicca symptoms and high titer ANA are clues for the early investigation of rheumatic diseases in TN patients. FAU - Nascimento, Iana Souza AU - Nascimento IS AD - Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Hospital das Clínicas da, São Paulo, Brazil. FAU - Bonfá, Eloísa AU - Bonfá E FAU - de Carvalho, Jozélio Freire AU - de Carvalho JF FAU - Saad, Carla Gonçalves AU - Saad CG FAU - Vendramini, Margarete Borges Galhardo AU - Vendramini MB FAU - Teixeira, Manoel Jacobsen AU - Teixeira MJ FAU - Nóbrega, José Cláudio Marinho AU - Nóbrega JC FAU - Viana, Vilma Santos Trindade AU - Viana VS LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Aged MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - Case-Control Studies MH - Connective Tissue Diseases/diagnosis/*epidemiology/*immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/epidemiology/immunology MH - Male MH - Middle Aged MH - Raynaud Disease/diagnosis/epidemiology/immunology MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/epidemiology/immunology MH - Sjogren's Syndrome/diagnosis/epidemiology/immunology MH - Trigeminal Neuralgia/blood/*complications/immunology EDAT- 2010/07/28 06:00 MHDA- 2010/12/17 06:00 CRDT- 2010/07/28 06:00 PHST- 2010/07/28 06:00 [entrez] PHST- 2010/07/28 06:00 [pubmed] PHST- 2010/12/17 06:00 [medline] AID - 00124743-201008000-00001 [pii] AID - 10.1097/RHU.0b013e3181e928e6 [doi] PST - ppublish SO - J Clin Rheumatol. 2010 Aug;16(5):205-8. doi: 10.1097/RHU.0b013e3181e928e6. PMID- 17901975 OWN - NLM STAT- MEDLINE DCOM- 20090421 LR - 20211020 IS - 1432-1246 (Electronic) IS - 0340-0131 (Linking) VI - 81 IP - 5 DP - 2008 Apr TI - Measurement, evaluation, and assessment of peripheral neurological disorders caused by hand-transmitted vibration. PG - 559-73 AB - Regular exposure to hand-transmitted vibration can result in symptoms and signs of peripheral vascular, neurological and other disorders collectively known as the hand-arm vibration syndrome. The measurement of the effects of hand-transmitted vibration involves converting the evidence of disorder (symptoms and signs) into information that can be stored. Evaluation requires the use of scales on which to indicate the severity of the various symptoms and signs. Assessment involves a judgement of severity relative to a criterion, usually for a specific purpose (e.g. to decide on removal from work or compensation). The measurement and evaluation of symptoms and signs is necessary when monitoring patient health and when performing epidemiological studies for research. The assessment of the severity of the hand-arm vibration syndrome is currently performed with staging systems, but the criteria are not clear and not related to defined methods for measuring or evaluating the symptoms and signs. Recognizing that similar symptoms can occur without injury from occupational exposures to hand-transmitted vibration, this paper attempts to define significant peripheral neurological symptoms caused by hand-transmitted vibration (i.e. 'unusual symptoms') and how these symptoms and related signs may be measured. Scales for evaluating the symptoms (e.g. their extent) and the related signs (e.g. their probability relative to the probability of the sign being present in persons not exposed to vibration) are defined. A method of relating unusual symptoms to both the signs of disorder and the pattern of vibration exposure is illustrated. Assessments of severity will vary according to the reasons for assessing the health effects of vibration, and will depend on local practice and convenience, but a way of combining evaluations of symptoms and signs is demonstrated in a staging system. Although inherently complex, the methods may assist the collection of data required to improve understanding of the effects of hand-transmitted vibration and also support a more complete reporting of the condition in those adversely affected by hand-transmitted vibration. FAU - Griffin, Michael J AU - Griffin MJ AD - Human Factors Research Unit, Institute of Sound and Vibration Research, University of Southampton, Southampton, UK. M.J.Griffin@soton.ac.uk LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070928 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - *Cumulative Trauma Disorders/diagnosis/etiology/physiopathology MH - Disability Evaluation MH - Female MH - *Hand-Arm Vibration Syndrome/diagnosis/etiology/physiopathology MH - Health Status MH - Humans MH - Male MH - Raynaud Disease/diagnosis/etiology/physiopathology MH - Severity of Illness Index MH - Vibration/*adverse effects EDAT- 2007/09/29 09:00 MHDA- 2009/04/22 09:00 CRDT- 2007/09/29 09:00 PHST- 2007/08/08 00:00 [received] PHST- 2007/09/05 00:00 [accepted] PHST- 2007/09/29 09:00 [pubmed] PHST- 2009/04/22 09:00 [medline] PHST- 2007/09/29 09:00 [entrez] AID - 10.1007/s00420-007-0253-5 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2008 Apr;81(5):559-73. doi: 10.1007/s00420-007-0253-5. Epub 2007 Sep 28. PMID- 18018385 OWN - NLM STAT- MEDLINE DCOM- 20080212 LR - 20170306 VI - 117 IP - 8 DP - 2007 Aug TI - [Coexistence of scleroderma-like syndrome and idiopathic myelofibrosis in a 54-year-old female patient: case report]. PG - 370-3 AB - Systemic sclerosis (SSc) is characterized by immunological disturbances, vascular damage and overproduction of extracellular matrix by stimulated fibroblasts. It has been postulated that immunological reactions involved in the pathogenesis of SSc may promote the development of malignancies. Coexistence of this disease with neoplasmatic processes is relatively frequent. In our report we describe a case a 54-year-old woman with scleroderma-like syndrome, which has preceded the occurrence of idiopathic myelofibrosis by many years. Owing to multiple repeated diagnostic tests we managed to diagnose this disease at the early stage, which enabled effective therapy with remission of blood dyscrasia as well as inhibition of skin lesions and lung fibrosis. FAU - Domysławska, Izabela AU - Domysławska I AD - Klinika Reumatologii i Chorób Wewnetrznych, Akademia Medyczna, Białystok. izadom@amb.edu.pl FAU - Ciołkiewicz, Mariusz AU - Ciołkiewicz M FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O FAU - Lewszuk, Andrzej AU - Lewszuk A FAU - Klimiuk, Piotr Adrian AU - Klimiuk PA FAU - Sierakowski, Stanisław AU - Sierakowski S LA - pol PT - Case Reports PT - English Abstract PT - Journal Article TT - Współistnienie zespołu twardzinopodobnego i samoistnego włóknienia szpiku u 54-letniej pacjentki: opis przypadku. PL - Poland TA - Pol Arch Med Wewn JT - Polskie Archiwum Medycyny Wewnetrznej JID - 0401225 RN - 0 (Antineoplastic Agents) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - MRK240IY2L (Azathioprine) RN - VB0R961HZT (Prednisone) RN - X6Q56QN5QC (Hydroxyurea) SB - IM MH - Antineoplastic Agents/*therapeutic use MH - Azathioprine/therapeutic use MH - Female MH - Glucocorticoids/*therapeutic use MH - Humans MH - Hydroxyurea/therapeutic use MH - Immunosuppressive Agents/*therapeutic use MH - Middle Aged MH - Prednisone/therapeutic use MH - Primary Myelofibrosis/*complications/diagnosis/*drug therapy MH - Raynaud Disease/complications/diagnosis MH - Scleroderma, Systemic/*complications/diagnosis/*drug therapy MH - Treatment Outcome EDAT- 2007/11/21 09:00 MHDA- 2008/02/13 09:00 CRDT- 2007/11/21 09:00 PHST- 2007/11/21 09:00 [pubmed] PHST- 2008/02/13 09:00 [medline] PHST- 2007/11/21 09:00 [entrez] PST - ppublish SO - Pol Arch Med Wewn. 2007 Aug;117(8):370-3. PMID- 30554151 OWN - NLM STAT- MEDLINE DCOM- 20201015 LR - 20201015 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 9 DP - 2019 Sep TI - Abnormal Nailfold Capillaroscopy Is Common in Patients with Connective Tissue Disease and Associated with Abnormal Pulmonary Function Tests. PG - 1109-1116 LID - 10.3899/jrheum.180615 [doi] AB - OBJECTIVE: To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT). METHODS: NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Abnormal PFT was defined as forced vital or diffusion capacity < 70%. Patients were classified as primary RP (n = 245), or secondary: no definite diagnosis (n = 391), SSc (n = 40), primary Sjögren syndrome (pSS; n = 30), systemic lupus erythematosus (SLE; n = 30), mixed connective tissue disease (MCTD; n = 7), rheumatoid arthritis (RA; n = 15). RESULTS: An SSc pattern on NCM was frequently observed in most patients with a definite diagnosis: SSc (88%), pSS (33%), SLE (17%), MCTD (71%), and RA (13%). In patients without definite diagnosis, 17% had a normal NCM pattern, 35% nonspecific, and 48% SSc pattern. Abnormal PFT was more frequent in patients with an SSc pattern (35.9% vs 19.5%, p = 0.002), even when corrected for SSc diagnosis (p = 0.003). Absence of an SSc pattern had high negative predictive value (88%); positive predictive values were low. CONCLUSION: SSc pattern on NCM is common in patients with RP, and in those with connective tissue diseases other than SSc. It is associated with a higher prevalence of abnormal PFT, independent of the presence of an SSc diagnosis. Although these data need validation in a prospective setting, they underline the importance of NCM in RP and putative value to stratify the risk of pulmonary involvement in early stages of disease. FAU - van Roon, Anniek M AU - van Roon AM AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. a.m.van.roon01@umcg.nl. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. a.m.van.roon01@umcg.nl. FAU - Huisman, Cato C AU - Huisman CC AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. FAU - van Roon, Arie M AU - van Roon AM AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. a.m.van.roon01@umcg.nl. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. a.m.van.roon01@umcg.nl. FAU - Zhang, Dan AU - Zhang D AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. FAU - Stel, Alja J AU - Stel AJ AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. FAU - Smit, Andries J AU - Smit AJ AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. FAU - Bootsma, Hendrika AU - Bootsma H AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. FAU - Mulder, Douwe J AU - Mulder DJ AD - From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. AD - A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. LA - eng PT - Journal Article DEP - 20181215 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2019 Sep;46(9):1061-1063. doi: 10.3899/jrheum.181408. PMID: 31474612 CIN - J Rheumatol. 2020 Jun 1;47(6):942. doi: 10.3899/jrheum.200020. PMID: 32295850 CIN - J Rheumatol. 2020 Jun 1;47(6):943. doi: 10.3899/jrheum.200136. PMID: 32295851 MH - Adult MH - Connective Tissue Diseases/diagnostic imaging/*physiopathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/diagnostic imaging/*physiopathology MH - Respiratory Function Tests MH - Scleroderma, Systemic/diagnostic imaging/*physiopathology MH - Young Adult OTO - NOTNLM OT - CONNECTIVE TISSUE DISEASES OT - MICROSCOPIC ANGIOSCOPY OT - RAYNAUD PHENOMENON OT - RESPIRATORY FUNCTION TESTS OT - SYSTEMIC SCLEROSIS EDAT- 2018/12/17 06:00 MHDA- 2020/10/21 06:00 CRDT- 2018/12/17 06:00 PHST- 2018/09/11 00:00 [accepted] PHST- 2018/12/17 06:00 [pubmed] PHST- 2020/10/21 06:00 [medline] PHST- 2018/12/17 06:00 [entrez] AID - jrheum.180615 [pii] AID - 10.3899/jrheum.180615 [doi] PST - ppublish SO - J Rheumatol. 2019 Sep;46(9):1109-1116. doi: 10.3899/jrheum.180615. Epub 2018 Dec 15. PMID- 32931678 OWN - NLM STAT- MEDLINE DCOM- 20210104 LR - 20210806 IS - 1066-2936 (Print) IS - 1066-2936 (Linking) VI - 47 IP - 3 DP - 2020 Third Quarter TI - ARTERIAL INSUFFICIENCIES: Hyperbaric Oxygen Therapy for Selected Problem Wounds. PG - 491-530 LID - 10.22462/03.07.2020.11 [doi] AB - The use of hyperbaric oxygen (HBO2) for the treatment of selected problem wounds has focused almost entirely on the diabetic foot ulcer (DFU) in recent years. The prevalence of DFUs in today's patient population and the reimbursement available for the treatment of DFUs have given it priority status in discussions about problem wounds, but there are sound fundamental reasons why additional oxygen may have benefits in the treatment of non-DFU wounds. CI - Copyright© Undersea and Hyperbaric Medical Society. FAU - Huang, Enoch T AU - Huang ET AD - Hyperbaric Medicine / Wound Healing, Legacy Emanuel Medical Center, Portland, Oregon U.S. FAU - Savaser, Davut J AU - Savaser DJ AD - Hyperbaric Medicine / Wound Healing, Legacy Emanuel Medical Center, Portland, Oregon U.S. FAU - Heyboer Iii, Marvin AU - Heyboer Iii M AD - Department of Emergency Medicine, Division of Hyperbaric Medicine and Wound Care, SUNY Upstate Medical University, Syracuse, New York U.S. LA - eng PT - Journal Article PT - Review PL - United States TA - Undersea Hyperb Med JT - Undersea & hyperbaric medicine : journal of the Undersea and Hyperbaric Medical Society, Inc JID - 9312954 RN - S88TT14065 (Oxygen) SB - IM MH - Anemia, Sickle Cell/complications MH - Calciphylaxis/therapy MH - Diabetic Foot/physiopathology/therapy MH - Graft vs Host Disease/therapy MH - Humans MH - Hyperbaric Oxygenation/*methods MH - Limb Salvage MH - Meta-Analysis as Topic MH - Oxygen/*physiology MH - Partial Pressure MH - Patient Selection MH - Peripheral Arterial Disease/physiopathology/therapy MH - Practice Guidelines as Topic MH - Pyoderma Gangrenosum/physiopathology/therapy MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/physiopathology/therapy MH - Scleroderma, Systemic/physiopathology/therapy MH - Skin Ulcer/etiology/physiopathology/*therapy MH - Systematic Reviews as Topic MH - Treatment Outcome MH - Utilization Review MH - Wound Healing/*physiology MH - Wounds and Injuries/complications/*therapy OTO - NOTNLM OT - arterial insufficiencies OT - problem wounds COIS- The author of this paper declares no conflicts of interest exist with this submission. EDAT- 2020/09/16 06:00 MHDA- 2021/01/05 06:00 CRDT- 2020/09/15 17:15 PHST- 2020/09/15 17:15 [entrez] PHST- 2020/09/16 06:00 [pubmed] PHST- 2021/01/05 06:00 [medline] AID - 10.22462/03.07.2020.11 [doi] PST - ppublish SO - Undersea Hyperb Med. 2020 Third Quarter;47(3):491-530. doi: 10.22462/03.07.2020.11. PMID- 20717643 OWN - NLM STAT- MEDLINE DCOM- 20110315 LR - 20211020 IS - 1432-2102 (Electronic) IS - 0033-832X (Linking) VI - 50 IP - 10 DP - 2010 Oct TI - [Therapy of peripheral vessel stenosis and occlusion in patients with thromboangiitis obliterans]. PG - 887-93 LID - 10.1007/s00117-010-2003-z [doi] AB - Vasculitis consists of a group of diseases characterized by an inflammatory process of the vessel wall. There is a wide variation in symptoms and almost any organ or tissue can be affected. Thromboangiitis obliterans (TAO; also known as Buerger's disease) is a special form of vasculitis with recurring inflammation and thrombosis of small and medium size arteries and veins of the hands and feet. To date the etiology still remains unclear but there is a strong association with the use of tobacco products. Ulcerations and gangrene of the extremities are common complications often resulting in the need for amputation of the extremity involved. Treatment of TAO includes both surgical and non-surgical methods but there is still no agreement concerning the optimal treatment strategy. In this contribution the advantages and disadvantages of different treatment options will be addressed and representative cases will be discussed. FAU - Sandner, T A AU - Sandner TA AD - Institut für Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universität München, Campus Innenstadt, Pettenkoferstr. 8a, 80336, München, Deutschland. Torleif.Sandner@med.lmu.de FAU - Degenhart, C AU - Degenhart C FAU - Becker-Lienau, J AU - Becker-Lienau J FAU - Reiser, M F AU - Reiser MF FAU - Treitl, M AU - Treitl M LA - ger PT - Case Reports PT - Journal Article TT - Therapie peripherer Gefäßstenosen und -verschlüsse bei Thromboangiitis obliterans. PL - Germany TA - Radiologe JT - Der Radiologe JID - 0401257 RN - 0 (Prostaglandins) RN - 9005-49-6 (Heparin) SB - IM MH - Adult MH - Angiography MH - Angioplasty MH - Arterial Occlusive Diseases/diagnostic imaging/*therapy MH - Arteries/surgery MH - Combined Modality Therapy MH - Foot/blood supply MH - Hand/blood supply MH - Heparin/therapeutic use MH - Humans MH - Ischemia/diagnostic imaging/*therapy MH - Limb Salvage MH - Male MH - Microsurgery MH - Prostaglandins/therapeutic use MH - Raynaud Disease/diagnostic imaging/therapy MH - Smoking/adverse effects MH - Thromboangiitis Obliterans/diagnostic imaging/*therapy MH - Young Adult EDAT- 2010/08/19 06:00 MHDA- 2011/03/16 06:00 CRDT- 2010/08/19 06:00 PHST- 2010/08/19 06:00 [entrez] PHST- 2010/08/19 06:00 [pubmed] PHST- 2011/03/16 06:00 [medline] AID - 10.1007/s00117-010-2003-z [doi] PST - ppublish SO - Radiologe. 2010 Oct;50(10):887-93. doi: 10.1007/s00117-010-2003-z. PMID- 33199454 OWN - NLM STAT- MEDLINE DCOM- 20210524 LR - 20210524 IS - 1488-2329 (Electronic) IS - 0820-3946 (Print) IS - 0820-3946 (Linking) VI - 192 IP - 46 DP - 2020 Nov 16 TI - Paraneoplastic acral vascular syndrome. PG - E1470 LID - 10.1503/cmaj.200430 [doi] FAU - Jud, Philipp AU - Jud P AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria philipp.jud@medunigraz.at. FAU - Raggam, Reinhard B AU - Raggam RB AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Hafner, Franz AU - Hafner F AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. LA - eng PT - Case Reports PT - Journal Article PL - Canada TA - CMAJ JT - CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne JID - 9711805 SB - IM MH - Aged, 80 and over MH - *Carcinoma, Non-Small-Cell Lung MH - Diagnosis, Differential MH - Hand/blood supply MH - Humans MH - *Lung Neoplasms MH - Male MH - *Neoplasm Recurrence, Local MH - Paraneoplastic Syndromes/*diagnosis MH - Raynaud Disease/*diagnosis PMC - PMC7682999 COIS- Competing interests: None declared. EDAT- 2020/11/18 06:00 MHDA- 2021/05/25 06:00 PMCR- 2020/11/16 CRDT- 2020/11/17 06:13 PHST- 2020/11/17 06:13 [entrez] PHST- 2020/11/18 06:00 [pubmed] PHST- 2021/05/25 06:00 [medline] PHST- 2020/11/16 00:00 [pmc-release] AID - 192/46/E1470 [pii] AID - 192e1470 [pii] AID - 10.1503/cmaj.200430 [doi] PST - ppublish SO - CMAJ. 2020 Nov 16;192(46):E1470. doi: 10.1503/cmaj.200430. PMID- 25452188 OWN - NLM STAT- MEDLINE DCOM- 20150818 LR - 20141202 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 41 IP - 12 DP - 2014 Dec TI - Retinal vessels thrombosis as onset manifestation of systemic sclerosis: 3 clinical cases. PG - 2495-6 LID - 10.3899/jrheum.140624 [doi] FAU - Rotondo, Cinzia AU - Rotondo C AD - Associate Professor of Rheumatology, Rheumatology Unit, Interdisciplinary Department of Medicine, School of Medicine, University of Bari, Bari, Italy. FAU - Lopalco, Giuseppe AU - Lopalco G AD - Associate Professor of Rheumatology, Rheumatology Unit, Interdisciplinary Department of Medicine, School of Medicine, University of Bari, Bari, Italy. FAU - Lapadula, Giovanni AU - Lapadula G AD - Associate Professor of Rheumatology, Rheumatology Unit, Interdisciplinary Department of Medicine, School of Medicine, University of Bari, Bari, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Associate Professor of Rheumatology, Rheumatology Unit, Interdisciplinary Department of Medicine, School of Medicine, University of Bari, Bari, Italy. florenzo.iannone@uniba.it. LA - eng PT - Case Reports PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Female MH - Fluorescein Angiography MH - Humans MH - Middle Aged MH - Raynaud Disease/complications MH - Retinal Artery Occlusion/*diagnosis/*etiology MH - Retinal Vein Occlusion/*diagnosis/*etiology MH - Scleroderma, Systemic/*complications EDAT- 2014/12/03 06:00 MHDA- 2015/08/19 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/08/19 06:00 [medline] AID - 41/12/2495 [pii] AID - 10.3899/jrheum.140624 [doi] PST - ppublish SO - J Rheumatol. 2014 Dec;41(12):2495-6. doi: 10.3899/jrheum.140624. PMID- 16311072 OWN - NLM STAT- MEDLINE DCOM- 20060417 LR - 20071115 IS - 1521-6616 (Print) IS - 1521-6616 (Linking) VI - 119 IP - 1 DP - 2006 Apr TI - Strange symptoms in Sneddon's syndrome. PG - 13-5 FAU - Wohlrab, Johannes AU - Wohlrab J AD - Martin-Luther-Universität Halle-Wittenberg, Germany. FAU - Francès, Camille AU - Francès C FAU - Sullivan, Kathleen E AU - Sullivan KE LA - eng PT - Case Reports PT - Clinical Conference PT - Journal Article DEP - 20051128 PL - United States TA - Clin Immunol JT - Clinical immunology (Orlando, Fla.) JID - 100883537 SB - IM MH - Adult MH - Autoimmune Diseases/complications MH - Blood Coagulation Disorders/complications MH - Diagnosis, Differential MH - Fatigue/etiology MH - Female MH - Humans MH - Raynaud Disease/complications MH - Skin Diseases, Vascular/complications MH - Sneddon Syndrome/complications/*diagnosis MH - Stroke/complications EDAT- 2005/11/29 09:00 MHDA- 2006/04/18 09:00 CRDT- 2005/11/29 09:00 PHST- 2005/09/30 00:00 [received] PHST- 2005/10/10 00:00 [revised] PHST- 2005/10/11 00:00 [accepted] PHST- 2005/11/29 09:00 [pubmed] PHST- 2006/04/18 09:00 [medline] PHST- 2005/11/29 09:00 [entrez] AID - S1521-6616(05)00335-9 [pii] AID - 10.1016/j.clim.2005.10.001 [doi] PST - ppublish SO - Clin Immunol. 2006 Apr;119(1):13-5. doi: 10.1016/j.clim.2005.10.001. Epub 2005 Nov 28. PMID- 21807877 OWN - NLM STAT- MEDLINE DCOM- 20120201 LR - 20161020 IS - 1526-3347 (Electronic) IS - 0191-9601 (Linking) VI - 32 IP - 8 DP - 2011 Aug TI - Index of suspicion. PG - 353-8 LID - 10.1542/pir.32-8-353 [doi] FAU - Balma-Mena, Alexandra AU - Balma-Mena A AD - Hospital for Sick Children, Toronto, Ontario, Canada. FAU - Weinstein, Miriam AU - Weinstein M FAU - Rosenthal, Alana AU - Rosenthal A FAU - Henry, Pauline AU - Henry P FAU - Gahzarian, Danny AU - Gahzarian D FAU - Miller, Jillian AU - Miller J FAU - Maul, Erich AU - Maul E FAU - McGuirl, Jennifer AU - McGuirl J FAU - Moorthy, Lakshmi AU - Moorthy L LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pediatr Rev JT - Pediatrics in review JID - 8103046 SB - IM MH - Arthritis/etiology MH - Bronchiectasis/complications/*diagnosis MH - Child MH - Cough/etiology MH - Diagnosis, Differential MH - Exanthema/etiology MH - Female MH - Fingers/abnormalities MH - Hoarseness/etiology MH - Humans MH - Male MH - Mixed Connective Tissue Disease/complications/*diagnosis MH - Pityriasis Lichenoides/complications/*pathology MH - Pruritus/etiology MH - Raynaud Disease/etiology MH - Skin Ulcer/etiology EDAT- 2011/08/03 06:00 MHDA- 2012/02/02 06:00 CRDT- 2011/08/03 06:00 PHST- 2011/08/03 06:00 [entrez] PHST- 2011/08/03 06:00 [pubmed] PHST- 2012/02/02 06:00 [medline] AID - 32/8/353 [pii] AID - 10.1542/pir.32-8-353 [doi] PST - ppublish SO - Pediatr Rev. 2011 Aug;32(8):353-8. doi: 10.1542/pir.32-8-353. PMID- 23738531 OWN - NLM STAT- MEDLINE DCOM- 20140305 LR - 20130729 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 11 IP - 8 DP - 2013 Aug TI - Capillaroscopy. PG - 731-6 LID - 10.1111/ddg.12137 [doi] AB - Microscopy of the nailfold capillaries has found increasing use in dermatology, rheumatology and angiology particularly as an important tool to distinguish between primary and secondary Raynaud disease. The best evidence is available in systemic sclerosis where specific capillaroscopic patterns have a high positive predictive value for the development of the disease. Conversely, a regular capillary pattern rules out systemic sclerosis with high degree of probability. PRINCE (prognostic index for nailfold capillaroscopic examination) was developed to identify patients at high risk of developing systemic sclerosis. CSURI (capillaroscopic skin ulcer risk index) should predict the risk of developing digital ulcers in patients with systemic sclerosis with high specificity and sensitivity. As a consequence of recent results a pathologic capillary pattern was integrated by the EULAR Scleroderma Trials and Research Group (EUSTAR) in the diagnostic algorithm of the VEDOSS-Project (very early diagnosis of systemic sclerosis). Capillary patterns may correlate with visceral involvement and capillaroscopy thus has the potential as a screening tool to enable early diagnosis of organ involvement in systemic sclerosis. CI - © The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin. FAU - Jung, Peter AU - Jung P AD - Department of Dermatology, State Hospital St. Pölten, Austria. peter.jung@stpoelten.lknoe.at FAU - Trautinger, Franz AU - Trautinger F LA - eng LA - ger PT - Journal Article PT - Review DEP - 20130605 PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 SB - IM MH - Angiography/*methods MH - Diagnosis, Differential MH - Humans MH - Microscopic Angioscopy/*methods MH - Microscopy/*methods MH - Raynaud Disease/*pathology MH - Scleroderma, Diffuse/*pathology MH - Skin Ulcer/*pathology EDAT- 2013/06/07 06:00 MHDA- 2014/03/07 06:00 CRDT- 2013/06/07 06:00 PHST- 2012/12/21 00:00 [received] PHST- 2013/04/23 00:00 [accepted] PHST- 2013/06/07 06:00 [entrez] PHST- 2013/06/07 06:00 [pubmed] PHST- 2014/03/07 06:00 [medline] AID - 10.1111/ddg.12137 [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2013 Aug;11(8):731-6. doi: 10.1111/ddg.12137. Epub 2013 Jun 5. PMID- 26244328 OWN - NLM STAT- MEDLINE DCOM- 20150813 LR - 20161125 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 373 IP - 6 DP - 2015 Aug 6 TI - IMAGES IN CLINICAL MEDICINE. Band Acro-osteolysis. PG - e6 LID - 10.1056/NEJMicm1311597 [doi] FAU - Tavare, Aniket N AU - Tavare AN AD - Royal Free London NHS Foundation Trust, London, United Kingdom aniket.tavare@gmail.com. FAU - Marmery, Helen AU - Marmery H LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Acro-Osteolysis/complications/*diagnostic imaging MH - Antibodies, Antinuclear/blood MH - Autoimmune Diseases/complications MH - Cholangitis, Sclerosing/complications MH - Humans MH - Male MH - Radiography MH - Raynaud Disease/complications MH - Young Adult EDAT- 2015/08/06 06:00 MHDA- 2015/08/14 06:00 CRDT- 2015/08/06 06:00 PHST- 2015/08/06 06:00 [entrez] PHST- 2015/08/06 06:00 [pubmed] PHST- 2015/08/14 06:00 [medline] AID - 10.1056/NEJMicm1311597 [doi] PST - ppublish SO - N Engl J Med. 2015 Aug 6;373(6):e6. doi: 10.1056/NEJMicm1311597. PMID- 23647531 OWN - NLM STAT- MEDLINE DCOM- 20140514 LR - 20151119 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 169 IP - 3 DP - 2013 Sep TI - CXCL13 and B-cell activating factor as putative biomarkers in systemic sclerosis. PG - 723-5 LID - 10.1111/bjd.12411 [doi] FAU - Wutte, N AU - Wutte N AD - Department of Dermatology, Medical University of Graz, Auenbruggerplatz 8, 8036, Graz, Austria. FAU - Kovacs, G AU - Kovacs G FAU - Berghold, A AU - Berghold A FAU - Reiter, H AU - Reiter H FAU - Aberer, W AU - Aberer W FAU - Aberer, E AU - Aberer E LA - eng PT - Comparative Study PT - Letter PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (B-Cell Activating Factor) RN - 0 (Biomarkers) RN - 0 (CXCL13 protein, human) RN - 0 (Chemokine CXCL13) SB - IM MH - Adult MH - B-Cell Activating Factor/*metabolism MH - Biomarkers/metabolism MH - Chemokine CXCL13/*metabolism MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pilot Projects MH - Raynaud Disease/metabolism MH - Scleroderma, Systemic/*diagnosis EDAT- 2013/05/08 06:00 MHDA- 2014/05/16 06:00 CRDT- 2013/05/08 06:00 PHST- 2013/05/08 06:00 [entrez] PHST- 2013/05/08 06:00 [pubmed] PHST- 2014/05/16 06:00 [medline] AID - 10.1111/bjd.12411 [doi] PST - ppublish SO - Br J Dermatol. 2013 Sep;169(3):723-5. doi: 10.1111/bjd.12411. PMID- 41966122 OWN - NLM STAT- MEDLINE DCOM- 20260412 LR - 20260412 IS - 1565-1088 (Print) VI - 28 IP - 4 DP - 2026 Apr TI - Comparative Analysis of Clinical Features and Autoantibody Profiles in Systemic Sclerosis Patients Among Jewish and Arab Populations in Israel. PG - 237-242 AB - BACKGROUND: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of the skin and internal organs. Its expression can vary across ethnic groups. OBJECTIVES: To compare clinical and serological manifestations of SSc between Jewish and Arab patients in Israel. METHODS: We conducted a retrospective single-center study included 100 patients with SSc selected from our rheumatology clinic at Meir Medical Center, comprising 50 Jewish and 50 Arab patients with available complete clinical and laboratory data. Demographic characteristics, disease features, autoantibody profiles, organ involvement, and treatment patterns were collected. RESULTS: Most clinical, laboratory, and treatment variables did not differ significantly between Jewish and Arab patients. Significant difference was the higher prevalence of skin telangiectasia in Jewish patients (86%) compared to Arab patients (38%) (P < 0.001) as well as Raynaud phenomenon and pulmonary hypertension. Other manifestations, including organ involvement and autoantibody prevalence, were similar across the groups. CONCLUSIONS: This study reveals significant similarities in the clinical and serological expression of SSc between Jewish and Arab patients in Israel. The higher prevalence of telangiectasia in Jewish patients suggests a possible ethnic or environmental influence on vascular manifestations. Further research is needed to explore the potential genetic or environmental factors contributing to this difference and to assess if this impacts disease progression or treatment outcomes. FAU - Hagog Natour, Hitam AU - Hagog Natour H AD - Institute of Endocrinology, Diabetes and Metabolism, Meir Medical Center, Kfar Saba, Israel. FAU - Asaly, Abedalla AU - Asaly A AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel. FAU - Elgardt, Izabella AU - Elgardt I AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel. FAU - Natour, Amed AU - Natour A AD - Department of Otolaryngology-Head and Neck Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. FAU - Levy, Yair AU - Levy Y AD - Department of Internal Medicine E, Meir Medical Center, Kfar Saba, Israel, Gray Faculty of Medical and Health Sciences, Tel Aviv University, Tel Aviv, Israel. LA - eng PT - Comparative Study PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Scleroderma, Systemic/immunology/ethnology/physiopathology/blood MH - Israel/epidemiology MH - Female MH - Male MH - *Autoantibodies/blood/immunology MH - *Arabs/statistics & numerical data MH - *Jews/statistics & numerical data MH - Retrospective Studies MH - Middle Aged MH - Adult MH - Prevalence MH - Aged MH - Raynaud Disease/etiology/epidemiology MH - Hypertension, Pulmonary/etiology/epidemiology EDAT- 2026/04/12 12:32 MHDA- 2026/04/12 12:33 CRDT- 2026/04/12 09:02 PHST- 2026/04/12 12:33 [medline] PHST- 2026/04/12 12:32 [pubmed] PHST- 2026/04/12 09:02 [entrez] PST - ppublish SO - Isr Med Assoc J. 2026 Apr;28(4):237-242. PMID- 23137613 OWN - NLM STAT- MEDLINE DCOM- 20130314 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 30 IP - 6 Suppl 74 DP - 2012 Nov-Dec TI - Autoimmune rheumatic disease associated symptoms in fibromyalgia patients and their influence on anxiety, depression and somatisation: a comparative study. PG - 65-9 AB - OBJECTIVES: In this study we evaluated the frequency of autoimmune rheumatic disease associated major symptoms in fibromyalgia (FM) patients, and the association between their presence and anxiety, depression and somatisation. METHODS: Two hundred and thirty-two FM, 78 systemic lupus erythematosus (SLE) patients and 70 healthy controls were included. All subjects were questioned face-to-face for the presence of autoimmune rheumatic disease-associated symptoms and antinuclear antibody (ANA) was determined. All FM patients were questioned for the severity of pain and symptoms of FM by using a visual analogue scale. In addition, all subjects were interrogated for anxiety, depression, somatic symptoms and neuropathic pain by using different validated questionnaires. RESULTS: FM patients had significantly higher frequency of photosensitivity (27.6% vs. 11.4%) and Raynaud phenomenon (22% vs. 10%) when compared to controls (p-values, 0.005 and 0.026). FM patients had significantly lower frequencies of photosensitivity, oral ulcers, xerostomia, and xerophthalmia than SLE patients (all p-values <0.001). ANA positivity was 11.8% in FM patients and 7.1% in healthy controls. ANA-positive and negative FM patients had similar frequencies of autoimmune rheumatic disease symptoms. FM patients with photosensitivity had higher anxiety (p=0.002), somatic symptoms (p=0.015) and neuropathic pain (p=0.03) scores than others. FM patients with Raynaud had higher anxiety (p=0.004), depression (p=0.001), somatic symptom (p<0.001) and neuropathic pain scores than others. CONCLUSIONS: The presence of which findings in FM seems to be associated with anxiety, depression, and somatization rather than ANA positivity and disease severity. FAU - Dönmez, Salim AU - Dönmez S AD - Division of Rheumatology, Trakya University Medical Faculty, Edirne, Turkey. drsalimdonmez@hotmail.com FAU - Pamuk, Ömer Nuri AU - Pamuk ÖN FAU - Ümit, Elif Gülsüm AU - Ümit EG FAU - Top, Mehmet Şerif AU - Top MŞ LA - eng PT - Comparative Study PT - Journal Article DEP - 20121214 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Analysis of Variance MH - Antibodies, Antinuclear/blood MH - Anxiety/diagnosis/*epidemiology/psychology MH - Biomarkers/blood MH - Chi-Square Distribution MH - Depression/diagnosis/*epidemiology/psychology MH - Fibromyalgia/diagnosis/*epidemiology/psychology MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/*epidemiology/psychology MH - Middle Aged MH - Oral Ulcer/epidemiology/psychology MH - Pain/epidemiology/psychology MH - Pain Measurement MH - Photosensitivity Disorders/epidemiology/psychology MH - Predictive Value of Tests MH - Psychiatric Status Rating Scales MH - Raynaud Disease/epidemiology/psychology MH - Rheumatic Diseases/diagnosis/*epidemiology/psychology MH - Severity of Illness Index MH - Somatoform Disorders/diagnosis/*epidemiology/psychology MH - Surveys and Questionnaires MH - Turkey/epidemiology MH - Xerophthalmia/epidemiology/psychology MH - Xerostomia/epidemiology/psychology EDAT- 2012/11/10 06:00 MHDA- 2013/03/15 06:00 CRDT- 2012/11/10 06:00 PHST- 2012/02/08 00:00 [received] PHST- 2012/09/26 00:00 [accepted] PHST- 2012/11/10 06:00 [entrez] PHST- 2012/11/10 06:00 [pubmed] PHST- 2013/03/15 06:00 [medline] AID - 5841 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2012 Nov-Dec;30(6 Suppl 74):65-9. Epub 2012 Dec 14. PMID- 17526311 OWN - NLM STAT- MEDLINE DCOM- 20070621 LR - 20161021 IS - 1060-3441 (Print) IS - 1060-3441 (Linking) VI - 19 IP - 2 DP - 2007 Apr TI - Connective tissue disorders. PG - 200, 207 FAU - Babin, Lynn A AU - Babin LA AD - Cincinnati State Technical and Community College, OH, USA. LA - eng PT - Journal Article PL - United States TA - Dermatol Nurs JT - Dermatology nursing JID - 9011113 MH - *Connective Tissue Diseases/diagnosis/etiology/therapy MH - Curriculum MH - Dermatology/education MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis MH - Raynaud Disease/diagnosis MH - Scleroderma, Systemic/diagnosis MH - Specialties, Nursing/education EDAT- 2007/05/29 09:00 MHDA- 2007/06/22 09:00 CRDT- 2007/05/29 09:00 PHST- 2007/05/29 09:00 [pubmed] PHST- 2007/06/22 09:00 [medline] PHST- 2007/05/29 09:00 [entrez] PST - ppublish SO - Dermatol Nurs. 2007 Apr;19(2):200, 207. PMID- 20110057 OWN - NLM STAT- MEDLINE DCOM- 20100903 LR - 20260128 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 136 Suppl 7 DP - 2009 Dec TI - [What's new in internal medicine?]. PG - S417-25 LID - 10.1016/S0151-9638(09)73383-6 [doi] AB - Among diagnostic progress over the last three years in internal medicine, Antisynthetase Syndrome is now more easily recognised with the diffusion of laboratory tests for research of antibodies against tRNA synthetases (Anti JO1, anti PL7, Anti PL12). In two third of cases, these antibodies are found despite absence of antinuclear antibodies. Hence, we have to search them specifically in patients with polyarthritis associated with myositis, cutaneous manifestations (Raynaud phenomenom and "mechanic'hands") and interstitial lung disease. Discovery of asymptomatic mutation in the L ferritin coding sequence help us to better understand the "unexplained" hyperferritinemia. Initially described by japonese gastroenterologists, auto immune pancreatitis in fact a part of a systemic sclerosing disease with a biochemical hallmark: in crease of a subclass of immunoglobulins G (IgG4). A new pediatric disease due to a deficiency of the interleukin1 receptor antagonist (multifocal aseptic osteitis, periostitis, stomatitis, disseminated pustulosis) help us to better understand unexplained auto inflammatory diseases. The therapeutic progress is primarily due to an explosion of biological therapies, particularly four of them very useful for internists (in an off label use) : Interleukin 1 inhibitors (anakinra, Canakinumab) to treat some auto inflammatory diseases (cryopirin associated periodic syndromes and deficency of interleukin 1 receptor antagonist), monoclonal antibody against interleukin 5 (mepolizumab) to treat some hypereosinophilic syndromes and Churg and Strauss angiitis, interleukin 6 inhibitiors to treat multifocal Castleman's disease and adult Still disease, a monoclonal antibody against vascular endothelial growth factor (Bevacizumab) to treat hereditary hemorrhagic telangiectasia. CI - Copyright 2009 Elsevier Masson SAS. All rights reserved. FAU - Blétry, O AU - Blétry O AD - Service de Médecine Interne, Hôpital Foch, Suresnes, France. o.bletry@hopital-foch.org FAU - Sene, T AU - Sene T FAU - Kahn, J-E AU - Kahn JE FAU - Ackermann, F AU - Ackermann F FAU - Charles, P AU - Charles P FAU - Leport, J AU - Leport J FAU - Piette, A-M AU - Piette AM LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Quoi de neuf en médecine interne? PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - 0 (Antibodies, Monoclonal) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 9007-73-2 (Ferritins) RN - 0 (Interleukin 1 Receptor Antagonist Protein) RN - 0 (Interleukin-6 Inhibitors) SB - IM MH - Acute Generalized Exanthematous Pustulosis/diagnosis/immunology MH - Amino Acyl-tRNA Synthetases/immunology MH - Antibodies, Monoclonal/therapeutic use MH - Arthritis/diagnosis/immunology MH - Autoantibodies/immunology MH - Autoimmune Diseases/*diagnosis/drug therapy/genetics/*immunology MH - Biomarkers/metabolism MH - Drug Therapy, Combination MH - Ferritins/genetics MH - Humans MH - Interleukin 1 Receptor Antagonist Protein/therapeutic use MH - Internal Medicine/*trends MH - Iron Overload/diagnosis/genetics MH - Lung Diseases, Interstitial/diagnosis/immunology MH - Mutation MH - Myositis/diagnosis/immunology MH - Osteitis/diagnosis/immunology MH - Pancreatitis/diagnosis/immunology MH - Periostitis/diagnosis/immunology MH - Raynaud Disease/diagnosis/immunology MH - Stomatitis/diagnosis/immunology MH - Syndrome MH - Treatment Outcome MH - Interleukin-6 Inhibitors RF - 32 EDAT- 2010/01/30 06:00 MHDA- 2010/09/04 06:00 CRDT- 2010/01/30 06:00 PHST- 2010/01/30 06:00 [entrez] PHST- 2010/01/30 06:00 [pubmed] PHST- 2010/09/04 06:00 [medline] AID - S0151-9638(09)73383-6 [pii] AID - 10.1016/S0151-9638(09)73383-6 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2009 Dec;136 Suppl 7:S417-25. doi: 10.1016/S0151-9638(09)73383-6. PMID- 38879188 OWN - NLM STAT- MEDLINE DCOM- 20240902 LR - 20240902 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 51 IP - 9 DP - 2024 Sep 1 TI - Long-Term Data on Efficacy and Safety of Selexipag for Digital Systemic Sclerosis Vasculopathy. PG - 899-903 LID - 10.3899/jrheum.2024-0103 [doi] AB - OBJECTIVE: Raynaud phenomenon (RP) and digital ulcers (DUs) are the main signs of digital vasculopathy in systemic sclerosis (SSc). Selexipag is an oral prostacyclin agonist approved for SSc-related pulmonary arterial hypertension. Following our previous preliminary short-course report, we herein present long-term data on selexipag safety and efficacy in the treatment of SSc digital vasculopathy. METHODS: Selexipag was administered to patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies. Each subject was assessed at baseline and after 3, 6, and 12 months. Clinical outcomes related to RP and DUs were evaluated along with modified Rodnan skin score of the fingers. Digital perfusion was assessed by laser speckle contrast analysis (LASCA). Nailfold videocapillaroscopy (NVC) was also performed. RESULTS: Eight patients with SSc (63% female, mean age 50.1 years) received selexipag. After 12 months of treatment, RP was reported to significantly decrease in the number of daily episodes and mean duration (P < 0.001 and P = 0.01, respectively). All patients achieved a complete healing of their DUs (P = 0.03) within 6 months. A progressive reduction of fingers skin score was observed (P = 0.03). No structural changes of capillaries were noted on NVC. Conversely, LASCA revealed an important increase in total digital perfusion (P = 0.004) despite seasonal variability. The safety profile was consistent with that reported in the literature. CONCLUSION: We observed a sustained efficacy of selexipag on SSc digital vasculopathy during 1 year of administration. Our promising results encourage the design of a new randomized controlled trial to evaluate the effect of selexipag on SSc digital vasculopathy. CI - Copyright © 2024 by the Journal of Rheumatology. FAU - Di Battista, Marco AU - Di Battista M AUID- ORCID: 0000-0002-4788-5729 AD - M. Di Battista, MD, Rheumatology Unit, University of Pisa, Pisa, and Department of Medical Biotechnologies, University of Siena, Siena; dibattista.marco91@gmail.com. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - A. Della Rossa, MD, PhD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AUID- ORCID: 0000-0001-5937-4574 AD - A. Della Rossa, MD, PhD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa, Italy. LA - eng PT - Journal Article DEP - 20240901 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 5EXC0E384L (selexipag) RN - 0 (Acetamides) RN - 0 (Pyrazines) SB - IM MH - Humans MH - Female MH - *Scleroderma, Systemic/drug therapy/complications MH - Male MH - Middle Aged MH - *Raynaud Disease/drug therapy/etiology MH - *Fingers/blood supply MH - Treatment Outcome MH - *Acetamides/therapeutic use/adverse effects MH - Adult MH - *Pyrazines/therapeutic use/adverse effects MH - Aged MH - Skin Ulcer/etiology/drug therapy MH - Microscopic Angioscopy/methods OTO - NOTNLM OT - Raynaud phenomenon OT - digital ulcers OT - digital vasculopathy OT - selexipag OT - systemic sclerosis EDAT- 2024/06/16 11:58 MHDA- 2024/09/02 08:45 CRDT- 2024/06/15 20:42 PHST- 2024/06/04 00:00 [accepted] PHST- 2024/09/02 08:45 [medline] PHST- 2024/06/16 11:58 [pubmed] PHST- 2024/06/15 20:42 [entrez] AID - jrheum.2024-0103 [pii] AID - 10.3899/jrheum.2024-0103 [doi] PST - epublish SO - J Rheumatol. 2024 Sep 1;51(9):899-903. doi: 10.3899/jrheum.2024-0103. PMID- 23238003 OWN - NLM STAT- MEDLINE DCOM- 20140128 LR - 20220330 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 80 IP - 3 DP - 2013 May TI - Endocardial and myocardial involvement in systemic sclerosis--is there a relevant inflammatory component? PG - 320-3 LID - S1297-319X(12)00278-3 [pii] LID - 10.1016/j.jbspin.2012.10.009 [doi] AB - OBJECTIVES: Myocardial manifestations of systemic sclerosis are mainly due to fibrotic remodeling. We report on two cases, where an endocardial and myocardial inflammation may be a relevant component of cardiac disease. CASE SERIES: Case 1 presented with fulminant tricuspid failure in the absence of pulmonary hypertension and with newly developing systemic sclerosis. Myocardial biopsy and MRI supported endocardial and myocardial inflammation. Treatment with cyclophosphamide resulted in stabilization of cardiac function and normalization of cardiac enzymes. The patient died due to infectious complications. Case 2, also newly developed systemic sclerosis, presented with renal crisis and pulmonary alveolitis. Elevated cardiac troponin T persisted in the presence of cyclophosphamide treatment, subsequent MRI suggested myocardial inflammation. After stepping up treatment by addition of rituximab cardiac enzymes normalized and cardiac function stabilized. CONCLUSION: We hypothesize that low-grade endocardial and myocardial inflammation may be more relevant in systemic sclerosis than appreciated previously. CI - Copyright © 2012. Published by Elsevier SAS. FAU - Dinser, Robert AU - Dinser R AD - Department of Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Rheumatology and Clinical Immunology, Benekestr 2-8, 61231 Bad Nauheim, Germany. FAU - Frerix, Marc AU - Frerix M FAU - Meier, Florian M P AU - Meier FM FAU - Klingel, Karin AU - Klingel K FAU - Rolf, Andreas AU - Rolf A LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20121210 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Aged MH - Endocarditis/*etiology/pathology MH - Female MH - Fibrosis/etiology/pathology MH - Humans MH - Hypertension, Pulmonary/etiology/pathology MH - Magnetic Resonance Imaging MH - Middle Aged MH - Myocarditis/*etiology/pathology MH - Raynaud Disease/etiology/pathology MH - Scleroderma, Systemic/*complications/*immunology/pathology MH - Tricuspid Valve Insufficiency/*etiology/pathology EDAT- 2012/12/15 06:00 MHDA- 2014/01/29 06:00 CRDT- 2012/12/15 06:00 PHST- 2012/03/23 00:00 [received] PHST- 2012/10/02 00:00 [accepted] PHST- 2012/12/15 06:00 [entrez] PHST- 2012/12/15 06:00 [pubmed] PHST- 2014/01/29 06:00 [medline] AID - S1297-319X(12)00278-3 [pii] AID - 10.1016/j.jbspin.2012.10.009 [doi] PST - ppublish SO - Joint Bone Spine. 2013 May;80(3):320-3. doi: 10.1016/j.jbspin.2012.10.009. Epub 2012 Dec 10. PMID- 38019158 OWN - NLM STAT- MEDLINE DCOM- 20240329 LR - 20240329 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 3 DP - 2024 Mar TI - Raynaud phenomenon as an isolated manifestation of autoimmune/inflammatory syndrome induced by adjuvants (ASIA). PG - 768 LID - 10.55563/clinexprheumatol/qinbvd [doi] FAU - Silva, Augusto AU - Silva A AD - Rheumatology and Metabolic Bone Diseases Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Center and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET), RITA full member, Lisbon; and Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, Portugal. augusto.silva@chln.min-saude.pt. FAU - Vieira-Sousa, Elsa AU - Vieira-Sousa E AD - Rheumatology and Metabolic Bone Diseases Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Center and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET), RITA full member, Lisbon; and Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, Portugal. FAU - Cruz-Machado, Ana Rita AU - Cruz-Machado AR AD - Rheumatology and Metabolic Bone Diseases Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Center and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET), RITA full member, Lisbon; and Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina da Universidade de Lisboa, Portugal. LA - eng PT - Letter DEP - 20231129 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Adjuvants, Immunologic) SB - IM MH - Humans MH - Adjuvants, Immunologic/adverse effects MH - Syndrome MH - *Raynaud Disease/chemically induced MH - *Autoimmune Diseases/chemically induced/diagnosis EDAT- 2023/11/29 12:43 MHDA- 2024/03/29 06:45 CRDT- 2023/11/29 10:13 PHST- 2023/08/29 00:00 [received] PHST- 2023/10/11 00:00 [accepted] PHST- 2024/03/29 06:45 [medline] PHST- 2023/11/29 12:43 [pubmed] PHST- 2023/11/29 10:13 [entrez] AID - 20293 [pii] AID - 10.55563/clinexprheumatol/qinbvd [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Mar;42(3):768. doi: 10.55563/clinexprheumatol/qinbvd. Epub 2023 Nov 29. PMID- 32134199 OWN - NLM STAT- MEDLINE DCOM- 20201005 LR - 20210702 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 72 IP - 7 DP - 2020 Jul TI - Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis. PG - 1049-1058 LID - 10.1002/art.41246 [doi] AB - Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc. CI - © 2020, American College of Rheumatology. FAU - Nagaraja, Vivek AU - Nagaraja V AUID- ORCID: 0000-0002-4930-3200 AD - University of Michigan, Ann Arbor. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - University of Florence, Florence, Italy. FAU - Furst, Daniel E AU - Furst DE AD - University of California in Los Angeles, University of Washington, Seattle, and University of Florence, Florence, Italy. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Nippon Medical School, Tokyo, Japan. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University, INSERM U1016, Université Sorbonne Paris Cité, and Cochin Hospital, Paris, France. FAU - Denton, Christopher P AU - Denton CP AD - University College London and Royal Free Hospital, London, UK. FAU - Raghu, Ganesh AU - Raghu G AD - University of Washington, Seattle. FAU - Mclaughlin, Vallerie AU - Mclaughlin V AD - University of Michigan, Ann Arbor. FAU - Rao, Panduranga S AU - Rao PS AD - University of Michigan, Ann Arbor. FAU - Seibold, James R AU - Seibold JR AD - Scleroderma Research Consultants, LLC, Aiken, South Carolina. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, Bath, UK. FAU - Whitfield, Michael L AU - Whitfield ML AD - Geisel School of Medicine at Dartmouth, Hanover, New Hampshire. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - R01 AR070470/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20200518 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antihypertensive Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Immunosuppressive Agents) RN - 0 (Prostaglandins I) SB - IM MH - Acute Disease MH - Antihypertensive Agents/therapeutic use MH - Disease Progression MH - Endothelin Receptor Antagonists/therapeutic use MH - Fibrosis MH - Fingers MH - Heart Diseases/etiology/physiopathology/*therapy MH - Hematopoietic Stem Cell Transplantation MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Kidney Diseases/etiology/physiopathology/*therapy MH - Lung Diseases, Interstitial/etiology/physiopathology/*therapy MH - Myocardium/pathology MH - Outcome Assessment, Health Care MH - Prostaglandins I/therapeutic use MH - Pulmonary Arterial Hypertension/etiology/physiopathology/*therapy MH - Raynaud Disease/etiology/physiopathology/*therapy MH - Scleroderma, Systemic/complications/physiopathology/*therapy MH - Severity of Illness Index MH - Skin Ulcer/etiology/physiopathology/*therapy PMC - PMC7329619 MID - NIHMS1574853 EDAT- 2020/03/07 06:00 MHDA- 2020/10/06 06:00 PMCR- 2021/07/01 CRDT- 2020/03/06 06:00 PHST- 2019/04/12 00:00 [received] PHST- 2020/02/27 00:00 [accepted] PHST- 2020/03/07 06:00 [pubmed] PHST- 2020/10/06 06:00 [medline] PHST- 2020/03/06 06:00 [entrez] PHST- 2021/07/01 00:00 [pmc-release] AID - 10.1002/art.41246 [doi] PST - ppublish SO - Arthritis Rheumatol. 2020 Jul;72(7):1049-1058. doi: 10.1002/art.41246. Epub 2020 May 18. PMID- 20395645 OWN - NLM STAT- MEDLINE DCOM- 20100708 LR - 20221207 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 6 DP - 2010 Jun TI - Association study of serotonin transporter gene (SLC6A4) in systemic sclerosis in European Caucasian populations. PG - 1164-7 LID - 10.3899/jrheum.091156 [doi] AB - OBJECTIVE: Serotonin is a key contributing factor in pulmonary arterial hypertension (PAH) by inducing pulmonary arterial smooth muscle cell (PA-SMC) proliferation. This relates specifically to the internalization process in PA-SMC of the serotonin transporter (SLC6A4 or 5-HTT). A long (L)/short (S) (44 base pair insertion) functional polymorphism within the promoter of the transporter SLC6A4 gene has been reported to be associated with familial and idiopathic PAH. Our objective was to determine whether polymorphisms of SLC6A4 confer susceptibility to SSc and its vascular phenotype. METHODS: Three Tag single-nucleotide polymorphisms (SNP) (rs2066713, rs1042173, rs6354) chosen using Hapmap and linkage disequilibrium data were genotyped in a total cohort of 667 SSc patients (56 with PAH, 207 with digital ulcerations) and 447 controls. All individuals were of French Caucasian origin. L/S polymorphism genotyping was determined by polymerase chain reaction in a random subgroup of 364 SSc patients (34 with PAH, 138 with digital ulcerations) and 218 controls. RESULTS: Three polymorphisms (L/S, rs2066713, rs1042173) were in Hardy-Weinberg equilibrium in the control population, but rs6354 deviated. Allelic and genotypic frequencies for these 3 polymorphisms were similar in SSc patients and controls. Subphenotype analyses of subsets with PAH and digital ulceration did not detect any difference between SSc patients compared to controls. CONCLUSION: These results from a large cohort of European Caucasian SSc patients do not support the implication of SLC6A4 in the pathogenesis of SSc and its vascular subphenotypes. However, serotonin pathways remain good candidates to contribute to the vasculopathy of SSc. FAU - Wipff, Julien AU - Wipff J AD - Service de Rhumatologie A, Hôpital Cochin, 27 rue du Faubourg St Jacques, 75014 Paris, France. julienwipff@cch.aphp.fr FAU - Bonnet, Pierre AU - Bonnet P FAU - Ruiz, Barbara AU - Ruiz B FAU - Dieude, Philippe AU - Dieude P FAU - Avouac, Jerome AU - Avouac J FAU - Tiev, Kiet AU - Tiev K FAU - Hachulla, Eric AU - Hachulla E FAU - Cracowski, Jean-Luc AU - Cracowski JL FAU - Diot, Elizabeth AU - Diot E FAU - Sibilia, Jean AU - Sibilia J FAU - Mouthon, Luc AU - Mouthon L FAU - Meyer, Olivier AU - Meyer O FAU - Kahan, Andre AU - Kahan A FAU - Boileau, Catherine AU - Boileau C FAU - Allanore, Yannick AU - Allanore Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100415 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (SLC6A4 protein, human) RN - 0 (Serotonin Plasma Membrane Transport Proteins) SB - IM MH - Cohort Studies MH - Female MH - France/epidemiology MH - *Genetic Predisposition to Disease MH - Genotype MH - Humans MH - Hypertension, Pulmonary/epidemiology/etiology/*genetics MH - Linkage Disequilibrium MH - Male MH - Middle Aged MH - *Polymorphism, Single Nucleotide MH - Raynaud Disease/complications/epidemiology/*genetics MH - Scleroderma, Systemic/complications/epidemiology/*genetics MH - Serotonin Plasma Membrane Transport Proteins/*genetics MH - Skin Ulcer/epidemiology/etiology/genetics MH - White People EDAT- 2010/04/17 06:00 MHDA- 2010/07/09 06:00 CRDT- 2010/04/17 06:00 PHST- 2010/04/17 06:00 [entrez] PHST- 2010/04/17 06:00 [pubmed] PHST- 2010/07/09 06:00 [medline] AID - jrheum.091156 [pii] AID - 10.3899/jrheum.091156 [doi] PST - ppublish SO - J Rheumatol. 2010 Jun;37(6):1164-7. doi: 10.3899/jrheum.091156. Epub 2010 Apr 15. PMID- 26065099 OWN - NLM STAT- MEDLINE DCOM- 20150710 LR - 20150611 IS - 1070-910X (Print) IS - 1070-910X (Linking) VI - 22 IP - 3 DP - 2014 Nov TI - Hands don't work like they used to? Help is on the way. Hand pain and stiffness, often tolerated without medical attention, can be successfully treated. PG - 4-5 LA - eng PT - Journal Article PL - United States TA - Harv Womens Health Watch JT - Harvard women's health watch JID - 9423147 RN - 0 (Analgesics) MH - Aged MH - Analgesics/therapeutic use MH - Arthritis, Rheumatoid/*complications MH - Carpal Tunnel Syndrome/*complications MH - Female MH - Hand/*blood supply/physiopathology MH - Humans MH - Middle Aged MH - Pain/drug therapy/*etiology/*prevention & control MH - Raynaud Disease/*complications EDAT- 2015/06/13 06:00 MHDA- 2015/07/15 06:00 CRDT- 2015/06/13 06:00 PHST- 2015/06/13 06:00 [entrez] PHST- 2015/06/13 06:00 [pubmed] PHST- 2015/07/15 06:00 [medline] PST - ppublish SO - Harv Womens Health Watch. 2014 Nov;22(3):4-5. PMID- 19480752 OWN - NLM STAT- MEDLINE DCOM- 20090811 LR - 20170214 IS - 1203-4754 (Print) IS - 1203-4754 (Linking) VI - 13 Suppl 1 DP - 2009 May-Jun TI - The challenge of scleroderma ulcers. PG - S42-8 FAU - Ramien, Michele AU - Ramien M AD - Division of Dermatology, University of Toronto, Toronto, ON. FAU - Brassard, Alain AU - Brassard A LA - eng PT - Journal Article PT - Review PL - United States TA - J Cutan Med Surg JT - Journal of cutaneous medicine and surgery JID - 9614685 RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) SB - IM MH - Endothelium, Vascular/drug effects MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/pharmacology MH - Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/pharmacology MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*etiology/therapy RF - 77 EDAT- 2009/06/02 09:00 MHDA- 2009/08/12 09:00 CRDT- 2009/06/02 09:00 PHST- 2009/06/02 09:00 [entrez] PHST- 2009/06/02 09:00 [pubmed] PHST- 2009/08/12 09:00 [medline] AID - 10.2310/7750.2009.00010 [doi] PST - ppublish SO - J Cutan Med Surg. 2009 May-Jun;13 Suppl 1:S42-8. doi: 10.2310/7750.2009.00010. PMID- 32099191 OWN - NLM STAT- MEDLINE DCOM- 20200423 LR - 20210322 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 16 IP - 4 DP - 2020 Apr TI - Raynaud phenomenon and digital ulcers in systemic sclerosis. PG - 208-221 LID - 10.1038/s41584-020-0386-4 [doi] AB - Raynaud phenomenon is a symptom complex caused by impaired digital perfusion and can occur as a primary phenomenon or secondary to a wide range of underlying causes. Raynaud phenomenon occurs in virtually all patients with systemic sclerosis (SSc) and is often the earliest clinical manifestation to occur. Careful assessment is required in patients with Raynaud phenomenon to avoid missing secondary causes such as SSc. Digital ulcers are a painful and disabling visible manifestation of digital vascular injury in patients with SSc. Progress has been made in the classification and assessment of digital ulcers and in understanding ulcer pathogenesis, and there are a wide range of treatments available to both prevent and heal digital ulcers, some of which are also used in Raynaud phenomenon management. In this Review, the assessment of patients with Raynaud phenomenon is discussed, including 'red flags' that are suggestive of SSc. The pathogenesis, classification and assessment of SSc-associated digital ulcers are also covered, alongside an overview of management approaches for SSc-associated Raynaud phenomenon and digital ulcers. Finally, unmet needs are discussed and the concept of a unified vascular phenotype in which therapies that affect the vasculature to support disease modification strategies is introduced. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. Michael.hughes-6@postgrad.manchester.ac.uk. AD - Centre for Musculoskeletal Research, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK. Michael.hughes-6@postgrad.manchester.ac.uk. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Cochin Hospital, AP-HP, Paris Descartes University, Paris, France. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine and Palo Alto VA Health Care System, Palo Alto, CA, USA. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. AD - Royal National Hospital for Rheumatic Diseases (part of Royal United Hospitals Bath NHS Foundation Trust), Bath, UK. FAU - Denton, Christopher P AU - Denton CP AD - Department of Rheumatology, Royal Free Hospital, University College London, London, UK. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, University of Florence, Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20200225 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM EIN - Nat Rev Rheumatol. 2021 Apr;17(4):246. doi: 10.1038/s41584-021-00591-5. PMID: 33750917 MH - Fingers/*blood supply MH - Humans MH - Raynaud Disease/*complications MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/diagnosis/*etiology EDAT- 2020/02/27 06:00 MHDA- 2020/04/24 06:00 CRDT- 2020/02/27 06:00 PHST- 2020/01/29 00:00 [accepted] PHST- 2020/02/27 06:00 [pubmed] PHST- 2020/04/24 06:00 [medline] PHST- 2020/02/27 06:00 [entrez] AID - 10.1038/s41584-020-0386-4 [pii] AID - 10.1038/s41584-020-0386-4 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2020 Apr;16(4):208-221. doi: 10.1038/s41584-020-0386-4. Epub 2020 Feb 25. PMID- 19422150 OWN - HSR STAT- MEDLINE DCOM- 20090515 LR - 20141120 IS - 1167-7422 (Print) IS - 1167-7422 (Linking) VI - 17 IP - 98 DP - 2008 Dec TI - Bosentan: new indication. Scleroderma and finger ulcers: no tangible efficacy. PG - 238 AB - No tangible clinical efficacy, but potentially severe adverse effects and a high potential for drug-drug interactions. LA - eng PT - Journal Article PL - France TA - Prescrire Int JT - Prescrire international JID - 9439295 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) MH - Endothelin Receptor Antagonists MH - Europe MH - Fingers/pathology MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Sulfonamides/administration & dosage/adverse effects/*therapeutic use MH - Ulcer/*drug therapy EDAT- 2009/05/09 09:00 MHDA- 2009/05/16 09:00 CRDT- 2009/05/09 09:00 PHST- 2009/05/09 09:00 [entrez] PHST- 2009/05/09 09:00 [pubmed] PHST- 2009/05/16 09:00 [medline] PST - ppublish SO - Prescrire Int. 2008 Dec;17(98):238. PMID- 19265349 OWN - NLM STAT- MEDLINE DCOM- 20090619 LR - 20090306 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 15 IP - 2 DP - 2009 Mar TI - The diagnosis ... From the patient's point of view. PG - 71 LID - 10.1097/RHU.0b013e31819bd6ca [doi] AB - Systemic scleroderma takes a long time to declare itself. Processing the diagnosis also takes a long time. Physicians should choose their words carefully, even when speaking to medically educated patients. The scene in the doctor's office will get replayed many times before the facts sink in, and will ultimately be a factor in how the patient deals with the disease. FAU - Rietveld, Barbara H AU - Rietveld BH AD - brietveld@aol.com LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - *Attitude to Health MH - Disease Progression MH - Humans MH - *Physician-Patient Relations MH - Raynaud Disease/diagnosis MH - Scleroderma, Systemic/*diagnosis/*psychology EDAT- 2009/03/07 09:00 MHDA- 2009/06/20 09:00 CRDT- 2009/03/07 09:00 PHST- 2009/03/07 09:00 [entrez] PHST- 2009/03/07 09:00 [pubmed] PHST- 2009/06/20 09:00 [medline] AID - 00124743-200903000-00006 [pii] AID - 10.1097/RHU.0b013e31819bd6ca [doi] PST - ppublish SO - J Clin Rheumatol. 2009 Mar;15(2):71. doi: 10.1097/RHU.0b013e31819bd6ca. PMID- 33337816 OWN - NLM STAT- MEDLINE DCOM- 20210917 LR - 20230927 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 6S DP - 2021 Sep 1 TI - Videocapillaroscopic Findings in Patients With Systemic Lupus Erythematosus With or Without Jaccoud Arthropathy. PG - S198-S203 LID - 10.1097/RHU.0000000000001617 [doi] AB - BACKGROUND/OBJECTIVE: Systemic lupus erythematosus (SLE) is an autoimmune disease that can present changes in blood vessels, which can be evaluated by periungual nailfold videocapillaroscopy (VCP). This technique is important for the diagnosis of systemic sclerosis and to identify individuals with Raynaud phenomenon at higher risk of developing systemic sclerosis. This study aims to describe the videocapillaroscopic profile of a series of SLE patients and to investigate if the VCP pattern is different among those with Jaccoud arthropathy (JA) compared with those without. METHODS: Between September 2014 and March 2015, the patients in this study underwent VCP, clinical evaluation, and laboratory tests. The capillaroscopic patterns were defined as minor, major, and scleroderma (SD). The presence of capillaroscopic findings, such as elongated capillaries, tortuosity, ectasia, prominent venous plexus, neoangiogenesis, hemorrhage, and megacapillaries, were also observed. Associations were calculated using the χ2, Fisher exact, or Student t test. RESULTS: In a population of 113 females with SLE (67 without JA and 46 with JA), at least 1 alteration was observed in VCP in 89.40% of them, among which "nonspecific changes" were the most prevalent. Minor changes were seen in 39 (58.2%) and 26 (56.5%), major changes in 21 (31.3%) and 11 (23.9%), and SD pattern in 2 (3.0%) and 3 (6.5%), in the patients without and with JA, respectively (p > 0.05). CONCLUSIONS: The majority of patients with SLE demonstrated changes in the VCP examination, but this tool did not allow discrimination between those with or without JA. CI - Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved. FAU - Pimenta da Fonseca, Emanuela AU - Pimenta da Fonseca E AD - From the Bahia School of Medicine and Public Health. FAU - Lins, Carolina Freitas AU - Lins CF AD - From the Bahia School of Medicine and Public Health. FAU - de Sá Ribeiro, Daniel Lima AU - de Sá Ribeiro DL AD - From the Bahia School of Medicine and Public Health. FAU - Santos, Willer Gonçalves Dourado AU - Santos WGD AD - From the Bahia School of Medicine and Public Health. FAU - Rosa, Genevievi AU - Rosa G AD - From the Bahia School of Medicine and Public Health. FAU - Machicado, Viviane AU - Machicado V AD - From the Bahia School of Medicine and Public Health. FAU - Pedreira, Ana Luiza AU - Pedreira AL AD - From the Bahia School of Medicine and Public Health. FAU - Souza, Anna Paula Mota Duque AU - Souza APMD AD - From the Bahia School of Medicine and Public Health. FAU - Baleeiro, Carla AU - Baleeiro C AD - From the Bahia School of Medicine and Public Health. FAU - Dos Santos Ferreira, Luana Grazielle AU - Dos Santos Ferreira LG AD - From the Bahia School of Medicine and Public Health. FAU - de Oliveira, Isabela Silva AU - de Oliveira IS AD - Department of Clinical and Toxicological Analysis, Faculty of Pharmacy, Federal University of Bahia. FAU - da Silva, João Paulo Cotrim Gama AU - da Silva JPCG AD - Department of Clinical and Toxicological Analysis, Faculty of Pharmacy, Federal University of Bahia. FAU - Atta, Ajax Merces AU - Atta AM AD - Department of Clinical and Toxicological Analysis, Faculty of Pharmacy, Federal University of Bahia. FAU - Santiago, Mittermayer Barreto AU - Santiago MB LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Capillaries MH - Female MH - Humans MH - *Joint Diseases MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - Microscopic Angioscopy MH - Nails MH - *Raynaud Disease/diagnosis/epidemiology COIS- The authors declare no conflict of interest. EDAT- 2020/12/19 06:00 MHDA- 2021/09/18 06:00 CRDT- 2020/12/18 15:18 PHST- 2020/12/19 06:00 [pubmed] PHST- 2021/09/18 06:00 [medline] PHST- 2020/12/18 15:18 [entrez] AID - 00124743-202109002-00010 [pii] AID - 10.1097/RHU.0000000000001617 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Sep 1;27(6S):S198-S203. doi: 10.1097/RHU.0000000000001617. PMID- 29446745 OWN - NLM STAT- MEDLINE DCOM- 20181012 LR - 20181012 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 14 IP - 1 DP - 2018 Apr 20 TI - Editorial: Nailfold Capillaroscopy in Rheumatology. PG - 2-4 LID - 10.2174/1573397114666180215102621 [doi] FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Department of Propaedeutics of Internal Diseases Faculty of Medicine Medical University - Plovdiv, Bulgaria. FAU - Muller-Ladner, Ulf AU - Muller-Ladner U AD - Department of Rheumatology and Clinical Immunology Justus-Liebig University Giessen Kerckhoff Clinic, Bad Nauheim, Germany. LA - eng PT - Editorial PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Humans MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/*diagnosis MH - Rheumatology/*methods EDAT- 2018/02/16 06:00 MHDA- 2018/10/13 06:00 CRDT- 2018/02/16 06:00 PHST- 2018/02/16 06:00 [pubmed] PHST- 2018/10/13 06:00 [medline] PHST- 2018/02/16 06:00 [entrez] AID - CRR-EPUB-88595 [pii] AID - 10.2174/1573397114666180215102621 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2018 Apr 20;14(1):2-4. doi: 10.2174/1573397114666180215102621. PMID- 25125696 OWN - NLM STAT- MEDLINE DCOM- 20141208 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 73 IP - 11 DP - 2014 Nov TI - Evaluation of blood perfusion by laser speckle contrast analysis in different areas of hands and face in patients with systemic sclerosis. PG - 2059-61 LID - 10.1136/annrheumdis-2014-205528 [doi] FAU - Sulli, A AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy. FAU - Ruaro, B AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy. FAU - Cutolo, M AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy. LA - eng PT - Evaluation Study PT - Letter DEP - 20140814 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Aged MH - Face/*blood supply MH - Female MH - Hand/*blood supply MH - Humans MH - Lasers MH - Male MH - Middle Aged MH - Raynaud Disease/*diagnosis MH - Regional Blood Flow MH - Scleroderma, Systemic/*complications OTO - NOTNLM OT - Autoimmune Diseases OT - Epidemiology OT - Systemic Sclerosis EDAT- 2014/08/16 06:00 MHDA- 2014/12/15 06:00 CRDT- 2014/08/16 06:00 PHST- 2014/08/16 06:00 [entrez] PHST- 2014/08/16 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] AID - S0003-4967(24)09833-9 [pii] AID - 10.1136/annrheumdis-2014-205528 [doi] PST - ppublish SO - Ann Rheum Dis. 2014 Nov;73(11):2059-61. doi: 10.1136/annrheumdis-2014-205528. Epub 2014 Aug 14. PMID- 22438006 OWN - NLM STAT- MEDLINE DCOM- 20120508 LR - 20160512 IS - 1535-1386 (Electronic) IS - 0021-9355 (Linking) VI - 94 IP - 6 DP - 2012 Mar 21 TI - What's new in hand surgery. PG - 569-73 LID - 10.2106/JBJS.K.01566 [doi] FAU - Amadio, Peter C AU - Amadio PC AD - Department of Orthopedics, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. pamadio@mayo.edu LA - eng PT - Journal Article PL - United States TA - J Bone Joint Surg Am JT - The Journal of bone and joint surgery. American volume JID - 0014030 SB - IM MH - Bone Diseases MH - Cost-Benefit Analysis MH - Hand/blood supply/*surgery MH - Hand Injuries/diagnosis/rehabilitation/*surgery MH - Humans MH - Microsurgery MH - Musculoskeletal Diseases/*surgery MH - Raynaud Disease/drug therapy MH - Replantation MH - Tendon Injuries/rehabilitation/*surgery MH - Wrist Injuries/*surgery EDAT- 2012/03/23 06:00 MHDA- 2012/05/09 06:00 CRDT- 2012/03/23 06:00 PHST- 2012/03/23 06:00 [entrez] PHST- 2012/03/23 06:00 [pubmed] PHST- 2012/05/09 06:00 [medline] AID - 10.2106/JBJS.K.01566 [doi] PST - ppublish SO - J Bone Joint Surg Am. 2012 Mar 21;94(6):569-73. doi: 10.2106/JBJS.K.01566. PMID- 21879251 OWN - NLM STAT- MEDLINE DCOM- 20120305 LR - 20181201 IS - 1651-2057 (Electronic) IS - 0001-5555 (Linking) VI - 91 IP - 6 DP - 2011 Oct TI - Bosentan is effective against digital ulcerations and hyperkeratosis in systemic sclerosis. PG - 716-7 LID - 10.2340/00015555-1138 [doi] FAU - Kurgyis, Zsuzsanna AU - Kurgyis Z FAU - Varga, Rita AU - Varga R FAU - Sick, Isabell AU - Sick I FAU - Lang, Martin Ulrich AU - Lang MU FAU - Ruzicka, Thomas AU - Ruzicka T FAU - Sárdy, Miklós AU - Sárdy M LA - eng PT - Case Reports PT - Letter PL - Sweden TA - Acta Derm Venereol JT - Acta dermato-venereologica JID - 0370310 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Female MH - Hand Dermatoses/drug therapy MH - Humans MH - Keratosis/*drug therapy/etiology MH - Middle Aged MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology MH - Sulfonamides/*therapeutic use EDAT- 2011/09/01 06:00 MHDA- 2012/03/06 06:00 CRDT- 2011/09/01 06:00 PHST- 2011/09/01 06:00 [entrez] PHST- 2011/09/01 06:00 [pubmed] PHST- 2012/03/06 06:00 [medline] AID - 10.2340/00015555-1138 [doi] PST - ppublish SO - Acta Derm Venereol. 2011 Oct;91(6):716-7. doi: 10.2340/00015555-1138. PMID- 21700982 OWN - NLM STAT- MEDLINE DCOM- 20110920 LR - 20161125 IS - 1546-3141 (Electronic) IS - 0361-803X (Linking) VI - 197 IP - 1 DP - 2011 Jul TI - Dynamic Doppler evaluation of the hand arteries to distinguish between primary and secondary raynaud phenomenon. PG - W175-80 LID - 10.2214/AJR.10.5740 [doi] AB - OBJECTIVE: The objective of our study was to use Doppler sonography to detect the flow characteristics and parameters of the hand arteries that are needed to distinguish between primary Raynaud phenomenon (RP) and secondary RP. SUBJECTS AND METHODS: The diameter, resistive index (RI), and flow volume of the digital, ulnar, and radial arteries of patients with primary RP and those with secondary RP were measured at rest and after cold provocation. The flow starting time in the digital artery and the flow normalizing time of all three arteries were also recorded after cold provocation. RESULTS: At baseline and after cold provocation, the diameters of the radial and digital arteries and the flow volumes of the three arteries were less in patients with secondary RP than in primary RP patients. In primary RP and secondary RP, the flow normalizing times (mean ± SD) were 9.8 ± 3.88 and 25.88 ± 7.14 minutes, respectively, in the radial artery; 11.3 ± 7.43 and 32.15 ± 12.57 minutes in the ulnar artery; and 12.22 ± 6.82 and 32.67 ± 10.76 minutes in the digital artery. A flow normalizing time cutoff in the radial artery of 17 minutes yielded a sensitivity of 90% and specificity of 100%. A flow normalizing time cutoff in the ulnar artery of 23 minutes yielded a sensitivity and specificity of 71% and 100%, respectively. A flow normalizing time cutoff in the digital artery of 23 minutes yielded a sensitivity and specificity of 82.6% and 98%, respectively. The flow starting time of the digital artery was 3.80 ± 3.27 minutes in primary RP and 16.78 ± 9.97 minutes in secondary RP (p < 0.0001). The flow starting time cutoff of the digital artery was 7 minutes (sensitivity, 82.6%; specificity, 95.7%). CONCLUSION: The diameter of the radial and distal arteries; flow volume; and flow volume normalizing time of the digital, ulnar, and radial arteries' flow starting time in the digital artery may be helpful in distinguishing between primary RP and secondary RP with high sensitivity and specificity values. These parameters may also facilitate objective follow-up of treatment. The noninvasive nature of Doppler sonography is an additional advantage, and there is no need for extra hardware or software. FAU - Toprak, Ugur AU - Toprak U AD - Department of Radiology, Ankara Numune Training and Research Hospital, Turkiye. toprakugur@yahoo.com FAU - Hayretci, Merve AU - Hayretci M FAU - Erhuner, Zeynep AU - Erhuner Z FAU - Tascilar, Koray AU - Tascilar K FAU - Ates, Askin AU - Ates A FAU - Karaaslan, Yasar AU - Karaaslan Y FAU - Karademir, Mehmet Alp AU - Karademir MA LA - eng PT - Journal Article PL - United States TA - AJR Am J Roentgenol JT - AJR. American journal of roentgenology JID - 7708173 SB - IM MH - Adult MH - Arteries/*diagnostic imaging MH - Cold Temperature MH - Diagnosis, Differential MH - Female MH - Hand/*blood supply/*diagnostic imaging MH - Humans MH - Male MH - Raynaud Disease/*diagnostic imaging/etiology MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Ultrasonography, Doppler/*methods EDAT- 2011/06/28 06:00 MHDA- 2011/09/21 06:00 CRDT- 2011/06/25 06:00 PHST- 2011/06/25 06:00 [entrez] PHST- 2011/06/28 06:00 [pubmed] PHST- 2011/09/21 06:00 [medline] AID - 197/1/W175 [pii] AID - 10.2214/AJR.10.5740 [doi] PST - ppublish SO - AJR Am J Roentgenol. 2011 Jul;197(1):W175-80. doi: 10.2214/AJR.10.5740. PMID- 17098538 OWN - NLM STAT- MEDLINE DCOM- 20061130 LR - 20121003 IS - 0741-5214 (Print) IS - 0741-5214 (Linking) VI - 44 IP - 5 DP - 2006 Nov TI - Incidence and natural history of Raynaud phenomenon: A long-term follow-up (14 years) of a random sample from the general population. PG - 1023-8 AB - BACKGROUND: Because the natural history of primary Raynaud phenomenon (RP) is unclear, we undertook this long-term (14 years) follow-up of an epidemiologic study on RP to investigate the incidence, remittance rate, and transition rate toward systemic sclerosis and other scleroderma spectrum disorders in a population-based sample of subjects. METHODS: In 1988 and 1989, 296 subjects obtained from a random sample of the general population of the Alpine valley of Tarentaise (southeast France) completed a cross-sectional study on RP. Of these, 78 met the diagnostic criteria for RP (RP+). From April 2002 to March 2003, we were able to get follow-up information on 292 people (dropout rate, 1.4%). Eighteen subjects (6.1%) had died, and the remaining 274 were successfully contacted. They were first evaluated by a standardized phone interview regarding their cold sensitivity, digital color changes, and RP. If any significant medical changes related to RP and/or suggesting scleroderma were reported, these subjects were invited for a medical evaluation. RESULTS: Mortality was similar in RP+ and RP- subjects, and no death was due to an RP-related condition. Seven cases of new RP were diagnosed in the RP- group, which corresponds to an annual incidence rate of 0.25%. Among the 72 RP+ subjects and the 7 subjects with a new RP available for follow-up, none developed clinical features of scleroderma. A disappearance of RP attacks for 2 winters or more was reported by 24 RP+ subjects (33%). CONCLUSIONS: These results show that, in the general population, RP is most often a benign condition and may disappear in a substantial proportion of subjects. FAU - Carpentier, Patrick H AU - Carpentier PH AD - Centre de Recherche Universitaire, La Léchère, France. patrick.carpentier@ujf-grenoble.fr FAU - Satger, Bernadette AU - Satger B FAU - Poensin, Dominique AU - Poensin D FAU - Maricq, Hildegard R AU - Maricq HR LA - eng GR - AR-31283/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Cross-Sectional Studies MH - Female MH - Follow-Up Studies MH - France/epidemiology MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - *Population Surveillance MH - Prognosis MH - Raynaud Disease/*epidemiology/*etiology MH - Retrospective Studies MH - Survival Rate MH - Time Factors EDAT- 2006/11/14 09:00 MHDA- 2006/12/09 09:00 CRDT- 2006/11/14 09:00 PHST- 2005/07/19 00:00 [received] PHST- 2006/07/20 00:00 [accepted] PHST- 2006/11/14 09:00 [pubmed] PHST- 2006/12/09 09:00 [medline] PHST- 2006/11/14 09:00 [entrez] AID - S0741-5214(06)01361-9 [pii] AID - 10.1016/j.jvs.2006.07.037 [doi] PST - ppublish SO - J Vasc Surg. 2006 Nov;44(5):1023-8. doi: 10.1016/j.jvs.2006.07.037. PMID- 19433883 OWN - NLM STAT- MEDLINE DCOM- 20090707 LR - 20090512 IS - 1386-0291 (Print) IS - 1386-0291 (Linking) VI - 42 IP - 2 DP - 2009 TI - Nailfold capillaroscopy: Specific features in Fabry disease. PG - 99-106 LID - 10.3233/CH-2009-1158 [doi] AB - OBJECTIVE: Fabry disease is a rare X-linked disorder caused by deficiency of alpha-galactosidase A. The metabolic defect results in the progressive accumulation of globotriaosylceramide within vascular cells leading to renal, cardiac and cerebrovascular manifestations. The aim of this study was to evaluate nailfold capillaroscopy as a non-invasive diagnostic tool in Fabry disease and to characterize morphological and functional changes of the capillaries in vivo. METHODS: Twenty-five consecutive patients with Fabry disease (17 males) without enzyme-replacement therapy had been studied by fluorescence nailfold capillaroscopy. Macrocirculation of digital arteries was tested by digital pulse volume recording and patients had been asked about the presence of Raynaud phenomenon. RESULTS: Significant more bushy capillaries and clusters were present in Fabry patients (72%) compared to healthy controls (10%). No avascular fields had been seen, and in only one patient atypical architecture and in another one a giant capillary was present. Enhanced natrium-fluorescein diffusion into the pericapillary area has been observed in three male patients. Six patients (one female) reported Raynaud phenomenon of all fingers. CONCLUSIONS: In Fabry disease morphological and functional microangiopathy of nailfold capillaries is present. Furthermore, these new findings might explain, at least in part, the unusual high frequency of Raynaud phenomenon in Fabry patients, which has not been described so far. Our data suggest that capillaroscopy might be used as an additional non-invasive diagnostic tool for Fabry disease. FAU - Wasik, Jan S AU - Wasik JS AD - Division of Angiology, Department of Internal Medicine, University Hospital of Zurich, Zurich, Switzerland. FAU - Simon, Roger W AU - Simon RW FAU - Meier, Thomas AU - Meier T FAU - Steinmann, Beat AU - Steinmann B FAU - Amann-Vesti, Beatrice R AU - Amann-Vesti BR LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 SB - IM MH - Adult MH - Aged MH - Fabry Disease/complications/*diagnosis/pathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/blood supply/pathology MH - Raynaud Disease/etiology MH - Renal Insufficiency/etiology EDAT- 2009/05/13 09:00 MHDA- 2009/07/08 09:00 CRDT- 2009/05/13 09:00 PHST- 2009/05/13 09:00 [entrez] PHST- 2009/05/13 09:00 [pubmed] PHST- 2009/07/08 09:00 [medline] AID - 54M4158388006X46 [pii] AID - 10.3233/CH-2009-1158 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2009;42(2):99-106. doi: 10.3233/CH-2009-1158. PMID- 21790927 OWN - NLM STAT- MEDLINE DCOM- 20130708 LR - 20120523 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 42 IP - 5 DP - 2012 May TI - Use of biological disease-modifying anti-rheumatic drugs in patients with concurrent rheumatic disease and hepatitis B. PG - 523-31 LID - 10.1111/j.1445-5994.2011.02569.x [doi] AB - BACKGROUND: Biological disease-modifying anti-rheumatic drugs (bDMARDs) are effective in the management of inflammatory arthritides. Reactivation of hepatitis B virus (HBV) is a potential adverse outcome in patients treated with bDMARDs. There is currently no consensus on the approach to identifying and treating these patients with underlying HBV infection. AIM: The aims of this study were to assess the risk of HBV reactivation in patients treated with bDMARDs, and to determine whether HBV screening should be carried out in all patients prior to commencing bDMARDs. METHODS: A literature search was undertaken to identify all reports of patients with inflammatory arthritides and concurrent HBV infection being treated with bDMARDs. RESULTS: Forty-three patients with HBV infection were identified, of whom eight patients developed HBV reactivation after exposure to bDMARDs. Of the patients who experienced reactivation, two had unknown infection that surfaced during bDMARD therapy. Patients who experienced reactivation were promptly treated with antiviral therapy and saw clinical improvement. There are no long-term data on these patients. CONCLUSIONS: HBV reactivation may result in serious consequences, including death. Tuberculosis screening prior to bDMARD treatment is already standard practice, as is HBV screening for patients undergoing cancer chemotherapy. Implementing HBV screening for all patients prior to bDMARD treatment can identify patients with chronic HBV who may require antiviral therapy. CI - © 2011 The Authors. Internal Medicine Journal © 2011 Royal Australasian College of Physicians. FAU - King, L K AU - King LK AD - Concord Clinical School, Sydney Medical School, Sydney, New South Wales, Australia. FAU - Lee, A AU - Lee A FAU - Anandacoomarasamy, A AU - Anandacoomarasamy A LA - eng PT - Journal Article PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - 0 (Antirheumatic Agents) SB - IM MH - Adult MH - Antirheumatic Agents/*therapeutic use MH - Female MH - Hepatitis B/*drug therapy/*epidemiology MH - Humans MH - Male MH - Middle Aged MH - Platelet Activation/drug effects/physiology MH - Raynaud Disease/drug therapy/epidemiology MH - Rheumatic Diseases/*drug therapy/*epidemiology MH - Young Adult EDAT- 2011/07/28 06:00 MHDA- 2013/07/09 06:00 CRDT- 2011/07/28 06:00 PHST- 2011/07/28 06:00 [entrez] PHST- 2011/07/28 06:00 [pubmed] PHST- 2013/07/09 06:00 [medline] AID - 10.1111/j.1445-5994.2011.02569.x [doi] PST - ppublish SO - Intern Med J. 2012 May;42(5):523-31. doi: 10.1111/j.1445-5994.2011.02569.x. PMID- 22898439 OWN - NLM STAT- MEDLINE DCOM- 20130411 LR - 20211021 IS - 1471-230X (Electronic) IS - 1471-230X (Linking) VI - 12 DP - 2012 Aug 16 TI - Primary biliary cirrhosis in a genetically homogeneous population: disease associations and familial occurrence rates. PG - 110 AB - BACKGROUND: Primary biliary cirrhosis (PBC) is a disease with genetic and environmental pathogenetic background. Chemicals, infectious agents, hormone therapy, reproductive history and surgical interventions have been implicated in the induction of PBC. Familial PBC has been documented in first degree relatives (FDR). Most cohort studies are genetically heterogeneous. Our study aimed to determine eventual lifestyle or disease associations and familial occurrence rates in a genetically homogeneous and geographically defined population of PBC patients. METHODS: 111 consenting PBC patients, were compared with 115 FDR and 149 controls matched for age, sex, Cretan origin and residence. All participants completed a questionnaire regarding demographics, lifestyle, medical, surgical and reproductive history. Significant variables on the univariate analysis were analyzed by multivariate analysis using a forward step-wise logistic regression model. RESULTS: Dyslipidaemia was found in 69.4% of patients, 60% of FDR and 40.9% of controls (p < 0.0001 and p = 0.003 respectively), autoimmune diseases in 36.9% of patients, 30.4% of FDR and 13.4% of controls (p < 0.0001 and p = 0.011 respectively). Hashimoto's disease (p = 0.003), Raynaud syndrome (p = 0.023) and Sjögren syndrome (p = 0.044) were significantly associated with PBC. On multivariate analysis statistically significant associations were found with primary educational level (AOR 2.304, 95% CI 1.024-5.181), cholecystectomy (AOR 2.927, 95% CI 1.347-6.362) and the presence of at least another autoimmune disease (AOR 3.318, 95% CI 1.177-6.22). Cancer history was more frequent in patients than in controls (p = 0.033). Familial PBC was found to be 9.9%. CONCLUSIONS: Dyslipidaemia and autoimmune diseases were significantly increased not only in patients as expected but also in their FDR. An increased prevalence of malignancies was found in patients. Primary educational level, cholecystectomy and the presence of at least another autoimmune disease were found as putative risk factors for PBC. No association was found with smoking, urinary tract infection or reproductive history. The reported high familial occurrence of PBC could imply screening with AMA of FDR with at least another autoimmune disease. FAU - Mantaka, Aikaterini AU - Mantaka A AD - Department of Gastroenterology and Hepatology, University Hospital of Heraklion, P,O, BOX 1352, Heraklion, 71100, Crete, Greece. katmant@gmail.com FAU - Koulentaki, Mairi AU - Koulentaki M FAU - Chlouverakis, Gregory AU - Chlouverakis G FAU - Enele-Melono, Jean Marie AU - Enele-Melono JM FAU - Darivianaki, Aikaterini AU - Darivianaki A FAU - Tzardi, Maria AU - Tzardi M FAU - Kouroumalis, Elias A AU - Kouroumalis EA LA - eng PT - Journal Article DEP - 20120816 PL - England TA - BMC Gastroenterol JT - BMC gastroenterology JID - 100968547 SB - IM MH - Aged MH - Autoimmune Diseases/*epidemiology/*genetics MH - Case-Control Studies MH - Cholecystectomy MH - Dyslipidemias/*epidemiology/*genetics MH - Educational Status MH - *Family MH - Female MH - Greece MH - Hashimoto Disease/epidemiology/genetics MH - Humans MH - Life Style MH - Liver Cirrhosis, Biliary/*epidemiology/*genetics MH - Logistic Models MH - Male MH - Middle Aged MH - Prevalence MH - Raynaud Disease/epidemiology/genetics MH - Risk Factors MH - Sjogren's Syndrome/epidemiology/genetics PMC - PMC3444887 EDAT- 2012/08/18 06:00 MHDA- 2013/04/12 06:00 PMCR- 2012/08/16 CRDT- 2012/08/18 06:00 PHST- 2011/12/08 00:00 [received] PHST- 2012/07/16 00:00 [accepted] PHST- 2012/08/18 06:00 [entrez] PHST- 2012/08/18 06:00 [pubmed] PHST- 2013/04/12 06:00 [medline] PHST- 2012/08/16 00:00 [pmc-release] AID - 1471-230X-12-110 [pii] AID - 10.1186/1471-230X-12-110 [doi] PST - epublish SO - BMC Gastroenterol. 2012 Aug 16;12:110. doi: 10.1186/1471-230X-12-110. PMID- 24400539 OWN - NLM STAT- MEDLINE DCOM- 20140218 LR - 20190724 IS - 0021-5384 (Print) IS - 0021-5384 (Linking) VI - 102 IP - 10 DP - 2013 Oct 10 TI - [Serious organ damage and intractable clinical conditions in rheumatic and connective tissue disease--progress in pathophysiology and treatment. Topics: I. Damage to important organs whose early treatment makes a big difference; 8. Skin and mucosa]. PG - 2591-9 FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Kanazawa University Graduate School of Medical Science, Japan. LA - jpn PT - Journal Article PT - Review PL - Japan TA - Nihon Naika Gakkai Zasshi JT - Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine JID - 19130210R SB - IM MH - Collagen Diseases/*complications/pathology MH - Humans MH - Lupus Erythematosus, Systemic/complications/pathology MH - Mucous Membrane/*pathology MH - Raynaud Disease/etiology MH - Skin/*pathology MH - Skin Diseases/*etiology/*pathology EDAT- 2014/01/10 06:00 MHDA- 2014/02/19 06:00 CRDT- 2014/01/10 06:00 PHST- 2014/01/10 06:00 [entrez] PHST- 2014/01/10 06:00 [pubmed] PHST- 2014/02/19 06:00 [medline] AID - 10.2169/naika.102.2591 [doi] PST - ppublish SO - Nihon Naika Gakkai Zasshi. 2013 Oct 10;102(10):2591-9. doi: 10.2169/naika.102.2591. PMID- 27436003 OWN - NLM STAT- MEDLINE DCOM- 20170620 LR - 20220419 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 56 IP - 3 DP - 2017 Mar 1 TI - Mixed connective tissue disease-enigma variations? PG - 326-333 LID - 10.1093/rheumatology/kew265 [doi] AB - In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial. We undertook a literature review, focusing on the articles reporting new data about MCTD published in the last decade, to determine whether any new observations help to answer the conundrum of MCTD. After reviewing recent data, we question whether the term MCTD is appropriately retained, preferring to use the term undifferentiated autoimmune rheumatic disease. CI - © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Ciang, Natalia C O AU - Ciang NC AD - Division of Rheumatology, Department of Medicine, Queen Elizabeth Hospital, Hong Kong. FAU - Pereira, Nídia AU - Pereira N AD - Internal Medicine Department, Hospital Pedro Hispano, Matosinhos, Portugal. FAU - Isenberg, David A AU - Isenberg DA AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. LA - eng PT - Journal Article PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (RNA, Small Nuclear) RN - 0 (U1 small nuclear RNA) SB - IM MH - Arthritis/etiology/immunology MH - Autoantibodies/immunology MH - Esophageal Diseases/etiology/immunology MH - Glomerulonephritis, Membranous/etiology/immunology MH - Glucocorticoids/therapeutic use MH - Humans MH - Hypertension, Pulmonary/etiology/immunology MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/etiology/immunology MH - Microscopic Angioscopy MH - Mixed Connective Tissue Disease/*classification/complications/drug therapy/immunology MH - Myositis/etiology/immunology MH - Pericarditis/etiology/immunology MH - RNA, Small Nuclear/immunology MH - Raynaud Disease/etiology/immunology MH - Vasodilation OTO - NOTNLM OT - anti-U1snRNP OT - antigen modification OT - capillaroscopy OT - clinical features OT - epitope spreading OT - immunology OT - mixed connective tissue disease OT - pulmonary hypertension EDAT- 2016/07/21 06:00 MHDA- 2017/06/21 06:00 CRDT- 2016/07/21 06:00 PHST- 2015/10/15 00:00 [received] PHST- 2016/07/21 06:00 [pubmed] PHST- 2017/06/21 06:00 [medline] PHST- 2016/07/21 06:00 [entrez] AID - kew265 [pii] AID - 10.1093/rheumatology/kew265 [doi] PST - ppublish SO - Rheumatology (Oxford). 2017 Mar 1;56(3):326-333. doi: 10.1093/rheumatology/kew265. PMID- 16496080 OWN - NLM STAT- MEDLINE DCOM- 20070619 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 4 DP - 2007 Apr TI - Primary biliary cirrhosis (PBC)-CREST overlap syndrome with coexistence of Sjögren's syndrome and thyroid dysfunction. PG - 596-600 AB - Calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, a limited form of systemic sclerosis, is sometimes complicated by primary biliary cirrhosis (PBC). A 52- and 61-year-old Japanese woman with PBC-CREST overlap syndrome accompanied by Sjögren's syndrome, and Hashimoto's thyroiditis, and Graves' disease, respectively, are reported. They had suffered from Raynaud's phenomena, sclerodactyly, morning stiffness, arthralgia, and sicca symptoms during these several years. They exhibited an increased level of alkaline phosphatase, gamma-glutamyl transpeptidase, positive antibodies against mitochondria and centromere, and hyperglobulinemia without any cholestatic symptoms. Histological findings from liver biopsy specimens were consistent with those of PBC. Clinically, they were diagnosed as both asymptomatic PBC and incomplete CREST syndrome. Their human leukocyte antigen typing showed both DR4 and DR8 positive. The association of the four autoimmune conditions is clinically and etiologically important. Although a combination of these diseases is rare, it is of importance to keep in mind that various autoimmune diseases could occur simultaneously. Of critical importance is that an active diagnostic attitude towards them is admirable, and that early diagnosis and therapy are needed. FAU - Nakamura, Tadashi AU - Nakamura T AD - Section of Internal Medicine and Rheumatology, Kumamoto Center for Arthritis and Rheumatology, 1-15-7 Kuhonji, Kumamoto 862-0976, Japan. naktrkme@koh.marutakai.or.jp FAU - Higashi, Syu-ichi AU - Higashi S FAU - Tomoda, Kunihiko AU - Tomoda K FAU - Tsukano, Michishi AU - Tsukano M FAU - Sugi, Kazuhiro AU - Sugi K LA - eng PT - Case Reports PT - Journal Article DEP - 20060222 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - CREST Syndrome/complications/*immunology MH - Female MH - Graves Disease/complications/*immunology MH - Hashimoto Disease/complications/*immunology MH - Humans MH - Liver Cirrhosis, Biliary/complications/*immunology MH - Middle Aged MH - Sjogren's Syndrome/complications/*immunology MH - Syndrome EDAT- 2006/02/24 09:00 MHDA- 2007/06/20 09:00 CRDT- 2006/02/24 09:00 PHST- 2005/09/28 00:00 [received] PHST- 2005/12/04 00:00 [accepted] PHST- 2005/12/04 00:00 [revised] PHST- 2006/02/24 09:00 [pubmed] PHST- 2007/06/20 09:00 [medline] PHST- 2006/02/24 09:00 [entrez] AID - 10.1007/s10067-005-0178-x [doi] PST - ppublish SO - Clin Rheumatol. 2007 Apr;26(4):596-600. doi: 10.1007/s10067-005-0178-x. Epub 2006 Feb 22. PMID- 35199140 OWN - NLM STAT- MEDLINE DCOM- 20220602 LR - 20240829 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 61 IP - 6 DP - 2022 May 30 TI - Is there a role for nailfold videocapillaroscopy in interstitial lung disease? PG - 2217-2220 LID - 10.1093/rheumatology/keac102 [doi] FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Internal Medicine, Ghent University. AD - Department of Rheumatology, Ghent University Hospital. AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Du Four, Tessa AU - Du Four T AD - Department of Internal Medicine, Ghent University. AD - Department of Rheumatology, Ghent University Hospital. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy. LA - eng PT - Comment PT - Editorial PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2022 May 30;61(6):2221-2234. doi: 10.1093/rheumatology/keab772. PMID: 34668513 MH - Humans MH - *Lung Diseases, Interstitial/diagnostic imaging MH - Microscopic Angioscopy MH - Nails/diagnostic imaging MH - *Raynaud Disease/diagnosis PMC - PMC9157075 EDAT- 2022/02/25 06:00 MHDA- 2022/06/03 06:00 PMCR- 2022/02/23 CRDT- 2022/02/24 05:46 PHST- 2022/01/07 00:00 [received] PHST- 2022/01/28 00:00 [revised] PHST- 2022/02/01 00:00 [accepted] PHST- 2022/02/25 06:00 [pubmed] PHST- 2022/06/03 06:00 [medline] PHST- 2022/02/24 05:46 [entrez] PHST- 2022/02/23 00:00 [pmc-release] AID - 6535250 [pii] AID - keac102 [pii] AID - 10.1093/rheumatology/keac102 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 May 30;61(6):2217-2220. doi: 10.1093/rheumatology/keac102. PMID- 34586992 OWN - NLM STAT- MEDLINE DCOM- 20211125 LR - 20211125 IS - 2162-5239 (Electronic) IS - 0094-6354 (Linking) VI - 89 IP - 5 DP - 2021 Oct TI - Peripheral Nerve Blockade for Patients With Raynaud Phenomenon and Other Causes of Digital Ischemia: A Case Report and Practice Implications. PG - 391-395 AB - Raynaud phenomenon can be an idiopathic benign disease, or it can be associated with vascular insufficiency due to arterial disease caused by other processes. Medical management of Raynaud phenomenon can be difficult, and digital ischemia, gangrene, and ulcers may occur secondary to vascular insufficiency. This case report describes the anesthetic management of a patient with a diagnosis of Raynaud phenomenon who presented to the perioperative area for débridement and distal amputation of the right third finger, which had become necrotic and gangrenous. An ultrasound-guided supraclavicular nerve block was performed preoperatively with 15 mL of 1.5% mepivacaine and 15 mL of 0.5% bupivacaine. The block was performed without complication, and the patient tolerated the procedure. A review of literature related to the use of peripheral nerve blockade for the treatment of digital ischemia is discussed. Ultrasound-guided technique is considered the gold standard for the performance of peripheral nerve blocks because this technique provides better efficacy and safety. These same ultrasonographic skills can expand anesthesia providers' practice beyond nerve blockade for anesthesia and analgesia with the addition of treatment and management of digital ischemia. CI - Copyright © by the American Association of Nurse Anesthetists. FAU - Stephas, Sarah A AU - Stephas SA AD - was a DNAP student at the University of Kansas, Kansas City, Kansas, at the time she wrote this manuscript. She is currently practicing as a CRNA in West Des Moines, Iowa. FAU - Hertel, Paul AU - Hertel P AD - is a clinical assistant professor in the University of Kansas Department of Nurse Anesthesia Education. Email: phertel@kumc.edu. FAU - Bennetts, Paul AU - Bennetts P AD - is a director of research and clinical assistant professor in the University of Kansas Department of Nurse Anesthesia Education. Email: pbennetts@kumc.edu. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - AANA J JT - AANA journal JID - 0431420 MH - *Anesthesia, Conduction MH - Humans MH - Ischemia MH - *Nerve Block MH - Peripheral Nerves MH - *Raynaud Disease/surgery OTO - NOTNLM OT - Anesthesiology OT - Raynaud phenomenon OT - chemical sympathectomy OT - digital ischemia OT - peripheral nerve block COIS- Name: Sarah A. Stephas, DNP, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author. Disclosures: None. Name: Paul Hertel, DNP, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author. Disclosures: None. Name: Paul Bennetts, PhD, CRNA Contribution: This author made significant contributions to the conception, synthesis, writing, and final editing and approval of the manuscript to justify inclusion as an author. Disclosures: None The authors did not discuss off-label use within the article. Disclosure statements are available for viewing upon request. EDAT- 2021/09/30 06:00 MHDA- 2021/11/26 06:00 CRDT- 2021/09/29 17:16 PHST- 2021/09/29 17:16 [entrez] PHST- 2021/09/30 06:00 [pubmed] PHST- 2021/11/26 06:00 [medline] PST - ppublish SO - AANA J. 2021 Oct;89(5):391-395. PMID- 31211383 OWN - NLM STAT- MEDLINE DCOM- 20200320 LR - 20200320 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 58 IP - 9 DP - 2019 Sep 1 TI - Clinical impact of artifactual hypoglycaemia and its diagnosis at the bedside. PG - 1691-1692 LID - 10.1093/rheumatology/kez118 [doi] FAU - Drenthen, Linda C A AU - Drenthen LCA AD - Department of Internal Medicine, Division of Diabetology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Verheggen, Rebecca J H M AU - Verheggen RJHM AD - Department of Internal Medicine, Division of Diabetology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - de Galan, Bastiaan E AU - de Galan BE AD - Department of Internal Medicine, Division of Diabetology, Radboud University Medical Center, Nijmegen, The Netherlands. LA - eng PT - Case Reports PT - Letter PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Blood Glucose) SB - IM MH - Artifacts MH - Blood Glucose/metabolism MH - Blood Specimen Collection/*methods MH - Diagnostic Errors MH - Humans MH - Hypoglycemia/*diagnosis MH - Male MH - Middle Aged MH - Point-of-Care Systems MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications PMC - PMC6736385 EDAT- 2019/06/19 06:00 MHDA- 2020/03/21 06:00 PMCR- 2019/04/09 CRDT- 2019/06/19 06:00 PHST- 2019/03/07 00:00 [accepted] PHST- 2019/06/19 06:00 [pubmed] PHST- 2020/03/21 06:00 [medline] PHST- 2019/06/19 06:00 [entrez] PHST- 2019/04/09 00:00 [pmc-release] AID - 5435710 [pii] AID - kez118 [pii] AID - 10.1093/rheumatology/kez118 [doi] PST - ppublish SO - Rheumatology (Oxford). 2019 Sep 1;58(9):1691-1692. doi: 10.1093/rheumatology/kez118. PMID- 29888702 OWN - NLM STAT- MEDLINE DCOM- 20190403 LR - 20190403 IS - 1952-4013 (Electronic) IS - 1167-1122 (Linking) VI - 28 IP - 4 DP - 2018 Aug 1 TI - Irreversible bilateral cyanosis of the hands caused by hypothenar hammer syndrome with systemic sclerosis. PG - 525-526 LID - 10.1684/ejd.2018.3311 [doi] FAU - Jin, Kayo AU - Jin K AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Matsuzaki, Yasushi AU - Matsuzaki Y AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Akasaka, Eijiro AU - Akasaka E AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Fukui, Tomohisa AU - Fukui T AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Sagara, Chihiro AU - Sagara C AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Nakano, Hajime AU - Nakano H AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. FAU - Sawamura, Daisuke AU - Sawamura D AD - Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan. LA - eng PT - Case Reports PT - Letter PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 SB - IM MH - Aged MH - Arterial Occlusive Diseases/*diagnostic imaging MH - Cumulative Trauma Disorders/complications MH - Cyanosis/*etiology MH - Hand MH - Humans MH - Male MH - Occupational Diseases/complications MH - Raynaud Disease/*etiology MH - Scleroderma, Systemic/*complications MH - Syndrome MH - Ulnar Artery/*diagnostic imaging EDAT- 2018/06/12 06:00 MHDA- 2019/04/04 06:00 CRDT- 2018/06/12 06:00 PHST- 2018/06/12 06:00 [pubmed] PHST- 2019/04/04 06:00 [medline] PHST- 2018/06/12 06:00 [entrez] AID - ejd.2018.3311 [pii] AID - 10.1684/ejd.2018.3311 [doi] PST - ppublish SO - Eur J Dermatol. 2018 Aug 1;28(4):525-526. doi: 10.1684/ejd.2018.3311. PMID- 19833286 OWN - NLM STAT- MEDLINE DCOM- 20100115 LR - 20091016 IS - 1873-4529 (Electronic) IS - 0952-8180 (Linking) VI - 21 IP - 6 DP - 2009 Sep TI - Sequential one-lung ventilation using one Arndt endobronchial blocker in a pediatric patient undergoing bilateral, video-assisted thoracoscopic surgery (VATS). PG - 464 LID - 10.1016/j.jclinane.2009.02.005 [doi] FAU - Li, Pei-Ying AU - Li PY FAU - Gu, Hua-Hua AU - Gu HH FAU - Liang, Wei-Min AU - Liang WM LA - eng PT - Case Reports PT - Letter PL - United States TA - J Clin Anesth JT - Journal of clinical anesthesia JID - 8812166 SB - IM MH - Adolescent MH - Fiber Optic Technology MH - Humans MH - Intubation, Intratracheal/instrumentation/*methods MH - Raynaud Disease/surgery MH - Respiration, Artificial/*instrumentation MH - Sympathectomy/methods MH - Thoracic Surgery, Video-Assisted/*methods EDAT- 2009/10/17 06:00 MHDA- 2010/01/16 06:00 CRDT- 2009/10/17 06:00 PHST- 2008/11/17 00:00 [received] PHST- 2009/02/16 00:00 [revised] PHST- 2009/02/23 00:00 [accepted] PHST- 2009/10/17 06:00 [entrez] PHST- 2009/10/17 06:00 [pubmed] PHST- 2010/01/16 06:00 [medline] AID - S0952-8180(09)00221-9 [pii] AID - 10.1016/j.jclinane.2009.02.005 [doi] PST - ppublish SO - J Clin Anesth. 2009 Sep;21(6):464. doi: 10.1016/j.jclinane.2009.02.005. PMID- 16804998 OWN - NLM STAT- MEDLINE DCOM- 20060727 LR - 20191110 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 1 DP - 2006 Feb TI - [Therapy for Reynaud syndrome]. PG - 80 FAU - Riemekasten, G AU - Riemekasten G LA - ger PT - Comment PT - Letter TT - Therapie des Reynaud-Syndroms. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Anticoagulants) RN - 0 (Antirheumatic Agents) RN - 0 (Vasodilator Agents) SB - IM CON - Z Rheumatol. 2005 Mar;64(2):90-2. doi: 10.1007/s00393-005-0692-x. PMID: 15793673 MH - Anticoagulants/*therapeutic use MH - Antirheumatic Agents/*therapeutic use MH - Drug Therapy, Combination MH - Fingers/blood supply MH - Humans MH - Necrosis/prevention & control MH - *Practice Guidelines as Topic MH - Raynaud Disease/*drug therapy MH - Rheumatology/standards MH - Skin/blood supply MH - Skin Ulcer/*drug therapy MH - *Societies, Medical MH - Toes/blood supply MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use EDAT- 2006/06/29 09:00 MHDA- 2006/07/28 09:00 CRDT- 2006/06/29 09:00 PHST- 2006/06/29 09:00 [pubmed] PHST- 2006/07/28 09:00 [medline] PHST- 2006/06/29 09:00 [entrez] AID - 10.1007/s00393-006-0032-9 [doi] PST - ppublish SO - Z Rheumatol. 2006 Feb;65(1):80. doi: 10.1007/s00393-006-0032-9. PMID- 29241781 OWN - NLM STAT- MEDLINE DCOM- 20180629 LR - 20220410 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 78 IP - 1 DP - 2018 Jan TI - Nifedipine cream versus sildenafil cream for patients with secondary Raynaud phenomenon: A randomized, double-blind, controlled pilot study. PG - 189-190 LID - S0190-9622(17)32207-7 [pii] LID - 10.1016/j.jaad.2017.08.018 [doi] FAU - Wortsman, Ximena AU - Wortsman X AD - Institute for Diagnostic Imaging and Research of the Skin and Soft Tissues, Pontificia Universidad Católica de Chile, Santiago, Chile; Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Del Barrio-Díaz, Pablo AU - Del Barrio-Díaz P AD - Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Meza-Romero, Rodrigo AU - Meza-Romero R AD - Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Poehls-Risco, Christine AU - Poehls-Risco C AD - Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Vera-Kellet, Cristián AU - Vera-Kellet C AD - Connective Tissue Diseases Unit, Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago, Chile. Electronic address: cristianverakellet@gmail.com. LA - eng PT - Comparative Study PT - Letter PT - Randomized Controlled Trial PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Dosage Forms) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Dosage Forms MH - Double-Blind Method MH - Female MH - Humans MH - Middle Aged MH - Nifedipine/*administration & dosage MH - Pilot Projects MH - Prospective Studies MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate/*administration & dosage MH - Vasodilator Agents/*administration & dosage MH - Young Adult EDAT- 2017/12/16 06:00 MHDA- 2018/06/30 06:00 CRDT- 2017/12/16 06:00 PHST- 2017/01/28 00:00 [received] PHST- 2017/07/16 00:00 [revised] PHST- 2017/08/08 00:00 [accepted] PHST- 2017/12/16 06:00 [entrez] PHST- 2017/12/16 06:00 [pubmed] PHST- 2018/06/30 06:00 [medline] AID - S0190-9622(17)32207-7 [pii] AID - 10.1016/j.jaad.2017.08.018 [doi] PST - ppublish SO - J Am Acad Dermatol. 2018 Jan;78(1):189-190. doi: 10.1016/j.jaad.2017.08.018. PMID- 20859221 OWN - NLM STAT- MEDLINE DCOM- 20110325 LR - 20211203 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 16 IP - 7 DP - 2010 Oct TI - A patient with Henoch-Schönlein purpura manifesting unusual symptoms and clinical course. PG - 338-40 LID - 10.1097/RHU.0b013e3181f4de99 [doi] AB - We evaluated and treated a girl with Henoch-Schönlein purpura (HSP), who initially developed redness, swelling, and pain in all 4 limbs accompanied by Raynaud syndrome and then had convulsions and disturbance of consciousness. HSP was diagnosed based on later findings of purpura in both legs and a decrease in factor XIII activity not accompanied by thrombocytopenia. She was normotensive. A skin biopsy specimen showed small-vessel vasculitis accompanied by immunoglobulin A deposition. The cause of erythema and limb pain, convulsions, and disturbed consciousness presumably was vasculitis. The possibility of HSP should be considered in patients with limb pain despite initial absence of purpura and in patients with central nervous system symptoms such as convulsions. FAU - Miyazawa, Tomoki AU - Miyazawa T AD - Department of Pediatrics, Kinki University School of Medicine, Osaka-Sayama, Japan. FAU - Sugimoto, Keisuke AU - Sugimoto K FAU - Fujita, Shinsuke AU - Fujita S FAU - Miyazaki, Kohei AU - Miyazaki K FAU - Takemura, Yutaka AU - Takemura Y FAU - Yanagida, Hidehiko AU - Yanagida H FAU - Sakata, Naoki AU - Sakata N FAU - Wada, Norihisa AU - Wada N FAU - Okada, Mitsuru AU - Okada M FAU - Takemura, Tsukasa AU - Takemura T LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Adolescent MH - Female MH - Humans MH - IgA Vasculitis/*complications/*diagnosis/therapy MH - Pain/etiology MH - Raynaud Disease/etiology EDAT- 2010/09/23 06:00 MHDA- 2011/03/26 06:00 CRDT- 2010/09/23 06:00 PHST- 2010/09/23 06:00 [entrez] PHST- 2010/09/23 06:00 [pubmed] PHST- 2011/03/26 06:00 [medline] AID - 10.1097/RHU.0b013e3181f4de99 [doi] PST - ppublish SO - J Clin Rheumatol. 2010 Oct;16(7):338-40. doi: 10.1097/RHU.0b013e3181f4de99. PMID- 21296436 OWN - NLM STAT- MEDLINE DCOM- 20120210 LR - 20181201 IS - 1874-1754 (Electronic) IS - 0167-5273 (Linking) VI - 147 IP - 3 DP - 2011 Mar 17 TI - Bosentan improves systemic sclerosis-related peripheral circulation insufficiency. PG - 472-5 LID - 10.1016/j.ijcard.2011.01.025 [doi] FAU - Yagi, Shusuke AU - Yagi S FAU - Akaike, Masashi AU - Akaike M FAU - Aihara, Ken-ichi AU - Aihara K FAU - Iwase, Takashi AU - Iwase T FAU - Sumitomo-Ueda, Yuka AU - Sumitomo-Ueda Y FAU - Yoshida, Sumiko AU - Yoshida S FAU - Matsumoto, Toshio AU - Matsumoto T FAU - Sata, Masataka AU - Sata M LA - eng PT - Comparative Study PT - Letter DEP - 20110205 PL - Netherlands TA - Int J Cardiol JT - International journal of cardiology JID - 8200291 RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Blood Circulation/*drug effects/physiology MH - Bosentan MH - Female MH - Humans MH - Middle Aged MH - Pain Measurement/drug effects MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Sulfonamides/pharmacology/*therapeutic use EDAT- 2011/02/08 06:00 MHDA- 2012/02/11 06:00 CRDT- 2011/02/08 06:00 PHST- 2011/01/04 00:00 [received] PHST- 2011/01/08 00:00 [accepted] PHST- 2011/02/08 06:00 [entrez] PHST- 2011/02/08 06:00 [pubmed] PHST- 2012/02/11 06:00 [medline] AID - S0167-5273(11)00055-6 [pii] AID - 10.1016/j.ijcard.2011.01.025 [doi] PST - ppublish SO - Int J Cardiol. 2011 Mar 17;147(3):472-5. doi: 10.1016/j.ijcard.2011.01.025. Epub 2011 Feb 5. PMID- 17960415 OWN - NLM STAT- MEDLINE DCOM- 20090421 LR - 20211020 IS - 1432-1246 (Electronic) IS - 0340-0131 (Linking) VI - 81 IP - 5 DP - 2008 Apr TI - Multicenter study on finger systolic blood pressure test for diagnosis of vibration-induced white finger. PG - 639-44 AB - OBJECTIVE: A multicenter study (six Rosai hospitals around Japan) was performed to investigate the diagnostic value of changes in finger systolic blood pressure (FSBP) after segmental local cooling for vibration-induced white finger (VWF). METHODS: Subjects were 154 men without exposure to vibration and 135 men with occupational vibration exposure. They were classified into four groups: Group A, 154 unexposed control cases; Group B, 21 exposed cases without VWF; Group C, 31 cases with a history of VWF but without any signs of VWF within the last year; and Group D, 83 cases with active VWF within the last year. FSBP% measurements were taken at room temperatures of 23 +/- 1 and 21 +/- 1 degrees C, using a strain-gauge Digimatic 2000 plethysmograph (Medimatic). RESULTS: At a room temperature of 23 +/- 1 degrees C, there was a significant difference between Groups A and D, and B and D. At a room temperature of 21 +/- 1 degrees C, there was a significant difference between Groups A and C, A and D, and B and D. The values in Group D were the lowest at both room temperatures. Assuming a cut-off value of 75% at 23 +/- 1 degrees C, the sensitivity and specificity were 65.2 and 87.5%, respectively. Assuming the same cut-off value at 21 +/- 1 degrees C, the sensitivity and specificity were 73.9 and 82.5%, respectively. These values were not too high. Most of the subjects with WVF in this study were retired and had not used vibratory tools for many years. The situation of the subjects may affect the results of the FSBP test. Our data did not confirm a difference in diagnostic accuracy between room temperatures of 23 +/- 1 and 21 +/- 1 degrees C. CONCLUSIONS: Our study showed that the sensitivity and specificity of the FSBP test with a cut-off value of 75% at 23 +/- 1 degrees C, were 65.2 and 87.5%, respectively, and at 21 +/- 1 degrees C, they were 73.9 and 82.5%, respectively. FAU - Nasu, Yoshiro AU - Nasu Y AD - Clinical Research Center for Hand-Arm Vibration Syndrome, Japanese Labor, Health and Welfare Organization, San-in Rosai Hospital, Yonago, Japan. nasu378@sanin-rosai-h.yonago.tottori.jp FAU - Kurozawa, Youichi AU - Kurozawa Y FAU - Fujiwara, Yutaka AU - Fujiwara Y FAU - Honma, Hiroki AU - Honma H FAU - Yanai, Toshiro AU - Yanai T FAU - Kido, Kenji AU - Kido K FAU - Ikeda, Takashi AU - Ikeda T LA - eng PT - Evaluation Study PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20071025 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - Aged MH - Blood Pressure/*physiology MH - Cold Temperature MH - Cumulative Trauma Disorders/*diagnosis/etiology/physiopathology MH - Fingers/*blood supply MH - Hand-Arm Vibration Syndrome/*diagnosis/etiology/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Occupational Exposure/adverse effects MH - Predictive Value of Tests MH - ROC Curve MH - Raynaud Disease/*diagnosis/etiology/physiopathology MH - Reproducibility of Results MH - Severity of Illness Index MH - Vibration/*adverse effects EDAT- 2007/10/26 09:00 MHDA- 2009/04/22 09:00 CRDT- 2007/10/26 09:00 PHST- 2007/10/14 00:00 [received] PHST- 2007/10/15 00:00 [accepted] PHST- 2007/10/26 09:00 [pubmed] PHST- 2009/04/22 09:00 [medline] PHST- 2007/10/26 09:00 [entrez] AID - 10.1007/s00420-007-0273-1 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2008 Apr;81(5):639-44. doi: 10.1007/s00420-007-0273-1. Epub 2007 Oct 25. PMID- 17899159 OWN - NLM STAT- MEDLINE DCOM- 20090421 LR - 20181113 IS - 1432-1246 (Electronic) IS - 0340-0131 (Linking) VI - 81 IP - 5 DP - 2008 Apr TI - A longitudinal study of finger systolic blood pressure and exposure to hand-transmitted vibration. PG - 613-23 AB - OBJECTIVES: To investigate prospectively the relation between vibration-induced white finger (VWF), exposure to hand-transmitted vibration (HTV) and the cold response of digital arteries in users of vibrating tools. METHODS: Two-hundred and sixteen HTV workers and 133 control men of the same companies underwent initially a medical examination and a standardised cold test with measurement of the change in finger systolic blood pressure (FSBP) after finger cooling from 30 to 10 degrees C. They were re-examined 1 year later. Tool vibration magnitudes were expressed as frequency-weighted and unweighted r.m.s. accelerations. From the vibration magnitudes and exposure durations, alternative measures of cumulative vibration dose were calculated for each HTV worker, according to the expression: Sigma(alpha)(m)(i)(t)(i), where a ( i ) is the acceleration magnitude on tool i, t ( i ) is the lifetime exposure duration for tool i, and m = 0, 1, 2 or 4. RESULTS: Among the HTV workers, the initial prevalence and the 1-year incidence of VWF were 18.1 and 1.7%, respectively. At the first examination, the HTV workers with moderate or severe score for VWF showed a significantly increased cold reaction in the fingers when compared with the controls and the HTV workers with no vascular symptoms. At the follow-up, the controls, the asymptomatic HTV workers, and the prevalent cases of VWF did not show significant changes in the cold response of digital arteries. A deterioration of cold-induced digital vasoconstriction was found in the incident cases of VWF. In the HTV workers, vibration doses with high powers of acceleration (i.e., Sigma(alpha)(m)(i)(t)(i) with m > 1) were major predictors of the vasoconstrictor response to cold at the follow-up examination. CONCLUSIONS: The measurement of FSBP after local cooling may be a helpful objective test to monitor prospectively the change in vibration-induced vascular symptoms. The findings of this longitudinal study suggest a dose-effect relationship between cold-induced digital arterial hyperresponsiveness over time and measures of cumulative vibration exposure. In the controls, the cold response of the digital arteries was stable over 1-year follow-up period. FAU - Bovenzi, Massimo AU - Bovenzi M AD - Department of Public Health Sciences, University of Trieste, Centro Tumori, Trieste, Italy. bovenzi@units.it FAU - D'Agostin, Flavia AU - D'Agostin F FAU - Rui, Francesca AU - Rui F FAU - Negro, Corrado AU - Negro C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070926 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - Adult MH - Arteries/physiopathology MH - Blood Pressure/*physiology MH - Cold Temperature MH - Cumulative Trauma Disorders/diagnosis/*etiology/physiopathology MH - Fingers/*blood supply MH - Forestry MH - Hand-Arm Vibration Syndrome/*diagnosis/etiology/physiopathology MH - Humans MH - Italy MH - Longitudinal Studies MH - Male MH - Raynaud Disease/diagnosis/*etiology/physiopathology MH - Vibration/*adverse effects EDAT- 2007/09/28 09:00 MHDA- 2009/04/22 09:00 CRDT- 2007/09/28 09:00 PHST- 2007/08/02 00:00 [received] PHST- 2007/09/05 00:00 [accepted] PHST- 2007/09/28 09:00 [pubmed] PHST- 2009/04/22 09:00 [medline] PHST- 2007/09/28 09:00 [entrez] AID - 10.1007/s00420-007-0255-3 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2008 Apr;81(5):613-23. doi: 10.1007/s00420-007-0255-3. Epub 2007 Sep 26. PMID- 22955486 OWN - NLM STAT- MEDLINE DCOM- 20130207 LR - 20161125 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 18 IP - 6 DP - 2012 Sep TI - Hand angiography in connective tissue disease. PG - 321 LID - 10.1097/RHU.0b013e3182685ccd [doi] FAU - Pua, Uei AU - Pua U AD - Tan Tock Seng Hospital, Singapore, Singapore. druei@yahoo.com LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Contrast Media) RN - 0 (Triiodobenzoic Acids) RN - 0 (Vasodilator Agents) RN - HW8W27HTXX (iodixanol) RN - SD6QCT3TSU (Pentoxifylline) SB - IM MH - Adult MH - Angiography MH - Arterial Occlusive Diseases/*diagnostic imaging/drug therapy MH - Contrast Media MH - Female MH - Hand/blood supply/*diagnostic imaging MH - Humans MH - Mixed Connective Tissue Disease/*diagnosis MH - Pentoxifylline/therapeutic use MH - Photoplethysmography MH - Raynaud Disease/*diagnosis MH - Triiodobenzoic Acids MH - Vasculitis/*diagnostic imaging/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2012/09/08 06:00 MHDA- 2013/02/08 06:00 CRDT- 2012/09/08 06:00 PHST- 2012/09/08 06:00 [entrez] PHST- 2012/09/08 06:00 [pubmed] PHST- 2013/02/08 06:00 [medline] AID - 00124743-201209000-00015 [pii] AID - 10.1097/RHU.0b013e3182685ccd [doi] PST - ppublish SO - J Clin Rheumatol. 2012 Sep;18(6):321. doi: 10.1097/RHU.0b013e3182685ccd. PMID- 19145954 OWN - NLM STAT- MEDLINE DCOM- 20090312 LR - 20260128 IS - 0362-4064 (Print) IS - 0362-4064 (Linking) VI - 77 IP - 12 DP - 2008 Dec TI - Controlling hand-arm vibration. PG - 47-8 FAU - Brauch, Rob AU - Brauch R AD - Larson Davis, Depew, NY, USA. rbrauch@pcb.com LA - eng PT - Journal Article PL - United States TA - Occup Health Saf JT - Occupational health & safety (Waco, Tex.) JID - 7610574 SB - IM MH - Environmental Monitoring MH - Epidemiological Monitoring MH - *Hand-Arm Vibration Syndrome/epidemiology/etiology/prevention & control MH - Humans MH - Male MH - Occupational Exposure/*adverse effects/prevention & control/standards MH - Raynaud Disease/etiology MH - United Kingdom/epidemiology MH - United States/epidemiology MH - Vibration/*adverse effects EDAT- 2009/01/17 09:00 MHDA- 2009/03/13 09:00 CRDT- 2009/01/17 09:00 PHST- 2009/01/17 09:00 [entrez] PHST- 2009/01/17 09:00 [pubmed] PHST- 2009/03/13 09:00 [medline] PST - ppublish SO - Occup Health Saf. 2008 Dec;77(12):47-8. PMID- 16463794 OWN - NLM STAT- MEDLINE DCOM- 20060323 LR - 20061115 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 2 IP - 48 DP - 2006 Jan 11 TI - [Dermatology]. PG - 107-8, 111-3 AB - We are going over therapeutic acquisitions in a club-journal including relevant publications in different fields: mecanism of action, therapeutic perspectives in a near future, and side effects. FAU - Kuenzli, S AU - Kuenzli S FAU - Saurat, J-H AU - Saurat JH LA - fre PT - English Abstract PT - Journal Article TT - Dermatologie. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 RN - 0 (Dermatologic Agents) RN - 0 (Herpesvirus Vaccines) SB - IM MH - Depression/chemically induced/diagnosis MH - Depression, Postpartum/complications MH - Dermatologic Agents/*therapeutic use MH - Female MH - Herpes Zoster/prevention & control MH - Herpesvirus Vaccines MH - Humans MH - Liver Cirrhosis/prevention & control MH - Raynaud Disease/drug therapy MH - Skin Diseases/*drug therapy EDAT- 2006/02/09 09:00 MHDA- 2006/03/24 09:00 CRDT- 2006/02/09 09:00 PHST- 2006/02/09 09:00 [pubmed] PHST- 2006/03/24 09:00 [medline] PHST- 2006/02/09 09:00 [entrez] PST - ppublish SO - Rev Med Suisse. 2006 Jan 11;2(48):107-8, 111-3. PMID- 22127901 OWN - NLM STAT- MEDLINE DCOM- 20120710 LR - 20120229 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 64 IP - 3 DP - 2012 Mar TI - Timing of transition between capillaroscopic patterns in systemic sclerosis. PG - 821-5 LID - 10.1002/art.33463 [doi] AB - OBJECTIVE: To investigate the timing of transition through different patterns of nailfold microvascular damage in patients with systemic sclerosis (SSc). METHODS: In this medium-term longitudinal study, 38 SSc patients (median disease duration 12 months) with the early scleroderma pattern of microangiopathy seen on baseline nailfold videocapillaroscopy (NVC) were followed up by NVC for a median of 84 months. The evolution of the NVC pattern over time was monitored and recorded. RESULTS: At the end of followup, the NVC pattern was still that of early scleroderma in 47% of the patients. The active scleroderma pattern was seen in 34%, the late scleroderma pattern in 13%, and a normal pattern in 5%. The mean± SD time of progression from the early to the active pattern and from the early to the late pattern was of 28 ± 20 months and 36± 29 months, respectively. In the subgroup of patients whose microangiopathy progressed from the early to the late NVC pattern, the time of progression from the early to the active pattern was only 8± 1 months (P = 0.01), demonstrating that there is a subset of patients with rapid progression of microangiopathy. Clinical symptoms progressed in accordance with the nailfold morphologic changes in 60% of the SSc patients. CONCLUSION: The results of this longitudinal study demonstrate dynamic transition of microvascular damage through different NVC patterns of microangiopathy in ∼50% of SSc patients. It is recommended that patients exhibiting rapid progression from the early to the active NVC pattern (<1 year) should be monitored closely, since the evidence suggests that they are at risk of rapid progression to the advanced (late) NVC pattern of microangiopathy that is associated with further clinical manifestations of SSc. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Smith, Vanessa AU - Smith V FAU - Zampogna, Giuseppe AU - Zampogna G FAU - Ravera, Francesca AU - Ravera F FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adult MH - Capillaries/*pathology MH - Disease Progression MH - Humans MH - Longitudinal Studies MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis/etiology MH - Scleroderma, Systemic/complications/*diagnosis MH - Time Factors MH - Video Recording/methods EDAT- 2011/12/01 06:00 MHDA- 2012/07/11 06:00 CRDT- 2011/12/01 06:00 PHST- 2011/12/01 06:00 [entrez] PHST- 2011/12/01 06:00 [pubmed] PHST- 2012/07/11 06:00 [medline] AID - 10.1002/art.33463 [doi] PST - ppublish SO - Arthritis Rheum. 2012 Mar;64(3):821-5. doi: 10.1002/art.33463. PMID- 27660946 OWN - NLM STAT- MEDLINE DCOM- 20170531 LR - 20181202 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 22 IP - 7 DP - 2016 Oct TI - Iatrogenic Thenar Eminence Atrophy After Botox A Injection for Secondary Raynaud Phenomenon. PG - 396-7 LID - 10.1097/RHU.0000000000000450 [doi] FAU - Eickhoff, Jeffrey C AU - Eickhoff JC AD - US Navy Medical Corps and Rheumatology Service Walter Reed National Military Medical Center Bethesda, MD Jeffrey.c.eickhoff.mil@mail.mil US Army Medical Corps and Department of Neurology Fort Belvoir Community Hospital Fort Belvoir, VA. Department of Neurology Walter Reed National Military Medical Center Bethesda, MD US Army Medical Corps and Rheumatology Service Walter Reed National Military Medical Center Bethesda, MD. FAU - Smith, Jonathan K AU - Smith JK FAU - Landau, Mark E AU - Landau ME FAU - Edison, Jess D AU - Edison JD LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Atrophy/chemically induced MH - Botulinum Toxins, Type A/administration & dosage/*adverse effects MH - Female MH - Hand/*pathology MH - Humans MH - Iatrogenic Disease MH - Raynaud Disease/*drug therapy EDAT- 2016/09/24 06:00 MHDA- 2017/06/01 06:00 CRDT- 2016/09/24 06:00 PHST- 2016/09/24 06:00 [entrez] PHST- 2016/09/24 06:00 [pubmed] PHST- 2017/06/01 06:00 [medline] AID - 00124743-201610000-00017 [pii] AID - 10.1097/RHU.0000000000000450 [doi] PST - ppublish SO - J Clin Rheumatol. 2016 Oct;22(7):396-7. doi: 10.1097/RHU.0000000000000450. PMID- 32149383 OWN - NLM STAT- MEDLINE DCOM- 20210607 LR - 20210607 IS - 1576-6578 (Electronic) IS - 0210-0010 (Linking) VI - 70 IP - 6 DP - 2020 Mar 16 TI - [Recurrent stroke, acrocyanosis and livedo racemosa: is it always Sneddon's syndrome?]. PG - 231-232 LID - 10.33588/rn.7006.2019505 [doi] FAU - Martínez-Martín, A AU - Martínez-Martín A AD - Complejo Hospitalario Universitario de Albacete, Albacete, España. FAU - Hernández-Fernández, F AU - Hernández-Fernández F AD - Complejo Hospitalario Universitario de Albacete, Albacete, España. FAU - Molina-Nuevo, J D AU - Molina-Nuevo JD AD - Complejo Hospitalario Universitario de Albacete, Albacete, España. FAU - López-Martínez, A AU - López-Martínez A AD - Hospital Virgen de la Luz, Cuenca, España. FAU - Segura, T AU - Segura T AD - Complejo Hospitalario Universitario de Albacete, Albacete, España. LA - spa PT - Case Reports PT - Journal Article TT - Ictus recidivante, acrocianosis y livedo racemosa: ¿siempre síndrome de Sneddon? PL - Singapore TA - Rev Neurol JT - Revista de neurologia JID - 7706841 SB - IM MH - Adult MH - Angiomatosis/complications/*diagnosis/diagnostic imaging MH - Brain Diseases/complications/*diagnosis/diagnostic imaging MH - Cerebral Angiography MH - Cognition Disorders/etiology MH - Diagnosis, Differential MH - Female MH - Headache/etiology MH - Humans MH - Magnetic Resonance Imaging MH - Neuroimaging MH - Raynaud Disease/*etiology MH - Recurrence MH - Sneddon Syndrome/*diagnosis MH - Stroke/*etiology MH - Syndrome EDAT- 2020/03/10 06:00 MHDA- 2021/06/08 06:00 CRDT- 2020/03/10 06:00 PHST- 2020/03/10 06:00 [entrez] PHST- 2020/03/10 06:00 [pubmed] PHST- 2021/06/08 06:00 [medline] AID - rn2019505 [pii] AID - 10.33588/rn.7006.2019505 [doi] PST - ppublish SO - Rev Neurol. 2020 Mar 16;70(6):231-232. doi: 10.33588/rn.7006.2019505. PMID- 29873115 OWN - NLM STAT- MEDLINE DCOM- 20190409 LR - 20190409 IS - 1440-1746 (Electronic) IS - 0815-9319 (Linking) VI - 33 IP - 8 DP - 2018 Aug TI - Gastrointestinal: Hide-bound bowel. PG - 1433 LID - 10.1111/jgh.14283 [doi] FAU - Hoversten, P AU - Hoversten P AD - Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. FAU - Bledsoe, A AU - Bledsoe A AD - Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. AD - Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. FAU - Sweetser, S AU - Sweetser S AD - Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. AD - Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20180605 PL - Australia TA - J Gastroenterol Hepatol JT - Journal of gastroenterology and hepatology JID - 8607909 SB - IM MH - Enteral Nutrition MH - Female MH - Fibrosis MH - Humans MH - Jejunal Diseases/diagnostic imaging/*etiology/therapy MH - Microstomia/etiology MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/diagnostic imaging/therapy MH - Tomography, X-Ray Computed EDAT- 2018/06/07 06:00 MHDA- 2019/04/10 06:00 CRDT- 2018/06/07 06:00 PHST- 2018/03/12 00:00 [received] PHST- 2018/05/07 00:00 [accepted] PHST- 2018/06/07 06:00 [pubmed] PHST- 2019/04/10 06:00 [medline] PHST- 2018/06/07 06:00 [entrez] AID - 10.1111/jgh.14283 [doi] PST - ppublish SO - J Gastroenterol Hepatol. 2018 Aug;33(8):1433. doi: 10.1111/jgh.14283. Epub 2018 Jun 5. PMID- 28039415 OWN - NLM STAT- MEDLINE DCOM- 20180504 LR - 20181202 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 56 IP - 7 DP - 2017 Jul 1 TI - Could nailfold videocapillaroscopy usher in a new era of preventative disease-modifying therapeutic intervention in systemic sclerosis? PG - 1053-1055 LID - 10.1093/rheumatology/kew461 [doi] FAU - Pauling, John D AU - Pauling JD AD - Royal United Hospitals NHS Foundation Trust, Royal National Hospital for Rheumatic Diseases. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. LA - eng PT - Comment PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CON - Rheumatology (Oxford). 2017 Jul 1;56(7):1081-1088. doi: 10.1093/rheumatology/kew402. PMID: 27940596 MH - Humans MH - *Microscopic Angioscopy MH - Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2017/01/01 06:00 MHDA- 2018/05/05 06:00 CRDT- 2017/01/01 06:00 PHST- 2016/10/17 00:00 [received] PHST- 2016/11/16 00:00 [accepted] PHST- 2017/01/01 06:00 [pubmed] PHST- 2018/05/05 06:00 [medline] PHST- 2017/01/01 06:00 [entrez] AID - kew461 [pii] AID - 10.1093/rheumatology/kew461 [doi] PST - ppublish SO - Rheumatology (Oxford). 2017 Jul 1;56(7):1053-1055. doi: 10.1093/rheumatology/kew461. PMID- 18571768 OWN - NLM STAT- MEDLINE DCOM- 20080930 LR - 20151119 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 59 IP - 4 DP - 2008 Oct TI - A potential role for imatinib and other small molecule tyrosine kinase inhibitors in the treatment of systemic and localized sclerosis. PG - 654-8 LID - 10.1016/j.jaad.2008.04.034 [doi] AB - Small molecule tyrosine kinase (TK) inhibitor, such as imatinib, is well established in the treatment of malignancy. Oral administration, high efficacy, and an excellent safety profile have made imatinib a drug of choice for several malignancies and benign conditions. Recent progress in the understanding of several benign conditions has led to the use of TK inhibitors in the treatment of hypereosinophilic syndrome and mastocytosis. Systemic sclerosis (SS) is a recalcitrant disease featuring multiorgan fibrosis and dysfunction. Molecular and biological evidence point to a central role for platelet-derived growth factor receptor, a TK-associated entity, in the pathogenesis of SS. The ability of several TK inhibitors, namely imatinib, to abrogate the activation of platelet-derived growth factor receptor-TK may entail their use in the treatment of SS and possibly more limited forms of sclerosis. Several human studies aiming to examine the use of imatinib in the treatment of SS are currently underway. FAU - Bibi, Yuval AU - Bibi Y AD - Department of Dermatology at Boston University School of Medicine, Boston, Massachusetts 02118, USA. yuvalbn@yahoo.com FAU - Gottlieb, Alice B AU - Gottlieb AB LA - eng PT - Journal Article PT - Review DEP - 20080620 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Benzamides) RN - 0 (Piperazines) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Pyrimidines) RN - 8A1O1M485B (Imatinib Mesylate) SB - IM MH - Benzamides MH - Fibrosis/drug therapy/metabolism/pathology MH - Humans MH - Imatinib Mesylate MH - Piperazines/*therapeutic use MH - Protein Kinase Inhibitors/*therapeutic use MH - Pyrimidines/*therapeutic use MH - Raynaud Disease/etiology/prevention & control MH - Scleroderma, Localized/complications/*drug therapy/pathology MH - Scleroderma, Systemic/*drug therapy MH - Skin/pathology RF - 40 EDAT- 2008/06/24 09:00 MHDA- 2008/10/01 09:00 CRDT- 2008/06/24 09:00 PHST- 2008/04/23 00:00 [received] PHST- 2008/04/27 00:00 [revised] PHST- 2008/04/30 00:00 [accepted] PHST- 2008/06/24 09:00 [pubmed] PHST- 2008/10/01 09:00 [medline] PHST- 2008/06/24 09:00 [entrez] AID - S0190-9622(08)00598-7 [pii] AID - 10.1016/j.jaad.2008.04.034 [doi] PST - ppublish SO - J Am Acad Dermatol. 2008 Oct;59(4):654-8. doi: 10.1016/j.jaad.2008.04.034. Epub 2008 Jun 20. PMID- 20605509 OWN - NLM STAT- MEDLINE DCOM- 20110203 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 77 IP - 5 DP - 2010 Oct TI - Silica-associated limited systemic sclerosis after occupational exposure to calcined diatomaceous earth. PG - 472-3 LID - 10.1016/j.jbspin.2010.02.044 [doi] AB - Silica-associated systemic sclerosis can occur in persons using calcined diatomaceous earth for filtration purpose. A limited systemic sclerosis was diagnosed in a 52-year-old male winegrower who had a combination of Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangectasia. The anti-centromere antibodies titre was 1/5000. The patient was frequently exposed to high atmospheric concentrations of calcined diatomaceous earth when performing the filtration of wines. Calcined diatomaceous earth is almost pure crystalline silica under the cristobalite form. The diagnosis of silica-associated limited systemic sclerosis after exposure to calcined diatomaceous earth was made. The patient's disease met the medical, administrative and occupational criteria given in the occupational diseases list 22 bis of the agriculture Social Security scheme and thence was presumed to be occupational in origin, without need to be proved. The diagnosis of occupational disease had been recognized by the compensation system of the agricultural health insurance. CI - Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Moisan, Stéphanie AU - Moisan S AD - Service de santé au travail, CHU d'Angers, 4, rue Larrey, 49933 Angers cedex 9, France. FAU - Rucay, Pierre AU - Rucay P FAU - Ghali, Alaa AU - Ghali A FAU - Penneau-Fontbonne, Dominique AU - Penneau-Fontbonne D FAU - Lavigne, Christian AU - Lavigne C LA - eng PT - Case Reports PT - Journal Article DEP - 20100604 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 61790-53-2 (Diatomaceous Earth) SB - IM MH - Agricultural Workers' Diseases/*chemically induced MH - CREST Syndrome/*chemically induced/diagnosis MH - Diatomaceous Earth/*adverse effects MH - Filtration MH - Humans MH - Male MH - Middle Aged MH - *Occupational Exposure MH - Wine EDAT- 2010/07/08 06:00 MHDA- 2011/02/04 06:00 CRDT- 2010/07/08 06:00 PHST- 2009/03/27 00:00 [received] PHST- 2010/02/03 00:00 [accepted] PHST- 2010/07/08 06:00 [entrez] PHST- 2010/07/08 06:00 [pubmed] PHST- 2011/02/04 06:00 [medline] AID - S1297-319X(10)00129-6 [pii] AID - 10.1016/j.jbspin.2010.02.044 [doi] PST - ppublish SO - Joint Bone Spine. 2010 Oct;77(5):472-3. doi: 10.1016/j.jbspin.2010.02.044. Epub 2010 Jun 4. PMID- 27694069 OWN - NLM STAT- MEDLINE DCOM- 20171016 LR - 20171016 IS - 1952-4013 (Electronic) IS - 1167-1122 (Linking) VI - 27 IP - 1 DP - 2017 Feb 1 TI - Mixed connective tissue disease with bilateral erythematous palpebral oedema and targetoid skin lesions. PG - 64-65 LID - 10.1684/ejd.2016.2902 [doi] FAU - Sakaguchi, Masanobu AU - Sakaguchi M AD - Division of Dermatology, Tohoku Medical and Pharmaceutical University, 1-12-1 Fukumuro, Miyagino-ku, Sendai 983-8512. FAU - Takeda, Tomoki AU - Takeda T AD - Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, 1-12-1 Fukumuro, Miyagino-ku, Sendai 983-8512. FAU - Kodera, Takao AU - Kodera T AD - Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, 1-12-1 Fukumuro, Miyagino-ku, Sendai 983-8512. FAU - Murakami, Kazuhiro AU - Murakami K AD - Division of Clinical Pathology, Tohoku Medical and Pharmaceutical University, 1-12-1 Fukumuro, Miyagino-ku, Sendai 983-8512, Japan. FAU - Oka, Masahiro AU - Oka M AD - Division of Dermatology, Tohoku Medical and Pharmaceutical University, 1-12-1 Fukumuro, Miyagino-ku, Sendai 983-8512. LA - eng PT - Case Reports PT - Letter PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adult MH - Edema/*etiology MH - Eyelid Diseases/*etiology MH - Glucocorticoids/therapeutic use MH - Humans MH - Male MH - Mixed Connective Tissue Disease/*complications/diagnosis/drug therapy MH - Prednisolone/therapeutic use MH - Raynaud Disease/etiology MH - Skin Diseases/*etiology/pathology EDAT- 2016/10/04 06:00 MHDA- 2017/10/17 06:00 CRDT- 2016/10/04 06:00 PHST- 2016/10/04 06:00 [pubmed] PHST- 2017/10/17 06:00 [medline] PHST- 2016/10/04 06:00 [entrez] AID - ejd.2016.2902 [pii] AID - 10.1684/ejd.2016.2902 [doi] PST - ppublish SO - Eur J Dermatol. 2017 Feb 1;27(1):64-65. doi: 10.1684/ejd.2016.2902. PMID- 19225060 OWN - NLM STAT- MEDLINE DCOM- 20090707 LR - 20220317 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 135 IP - 6 DP - 2009 Jun TI - Clinical profile of anti-PL-12 autoantibody. Cohort study and review of the literature. PG - 1550-1556 LID - S0012-3692(09)60360-3 [pii] LID - 10.1378/chest.08-2233 [doi] AB - BACKGROUND: The antisynthetase syndrome consists of interstitial lung disease (ILD), arthritis, myositis, fever, mechanic's hands, and Raynaud phenomenon in the presence of an antisynthetase autoantibody, most commonly anti-Jo-1. It is believed that all the antisynthetases are associated with a similar clinical profile, but definitive data in this diverse group are lacking. The purpose of this study was to examine the clinical profile of anti-PL-12, an antisynthetase autoantibody directed against alanyl-transfer RNA synthetase. METHODS: Thirty-one subjects with anti-PL-12 autoantibody were identified from the databases at the Medical University of South Carolina, the University of Pittsburgh Medical Center, Johns Hopkins Medical Center, and Brigham and Women's Hospital. The medical charts were reviewed and the following data were recorded: demographic information; pulmonary and rheumatologic symptoms; connective tissue disease (CTD) diagnoses; serologic autoantibody findings; CT scan results; BAL findings; pulmonary function test results; lung histopathology; and treatment interventions. RESULTS: The median age at symptom onset was 51 years; 81% were women and 52% were African American. Ninety percent of anti-PL-12-positive patients had ILD, 65% of whom presented initially to a pulmonologist. Ninety percent of anti-PL-12-positive patients had an underlying CTD. Polymyositis and dermatomyositis were the most common underlying diagnoses. Raynaud phenomenon occurred in 65% of patients, fever in 45% of patients, and mechanic's hands in 16% of patients. Test results for the presence of antinuclear antibody were positive in 48% of cases. CONCLUSIONS: Anti-PL-12 is strongly associated with the presence of ILD, but less so with myositis and arthritis. Idiopathic ILD diagnosed as idiopathic pulmonary fibrosis may, in fact, be associated with anti-PL-12 and be a "forme fruste" of an underlying autoimmune disorder. FAU - Kalluri, Meena AU - Kalluri M AD - Division of Pulmonary and Critical Care Medicine, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC. Electronic address: meenakalluri@med.ualberta.ca. FAU - Sahn, Steven A AU - Sahn SA AD - Division of Pulmonary and Critical Care Medicine, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC. FAU - Oddis, Chester V AU - Oddis CV AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA. FAU - Gharib, Suzanne L AU - Gharib SL AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA. FAU - Christopher-Stine, Lisa AU - Christopher-Stine L AD - Divisions of Rheumatology, Johns Hopkins University, Baltimore, MD. FAU - Danoff, Sonye K AU - Danoff SK AD - Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Divisions of Rheumatology, Johns Hopkins University, Baltimore, MD. FAU - Hong, Grace AU - Hong G AD - Divisions of Rheumatology, Johns Hopkins University, Baltimore, MD. FAU - Dellaripa, Paul F AU - Dellaripa PF AD - Division of Rheumatology, Allergy, and Immunology, Brigham and Women's Hospital, Boston, MA. FAU - Highland, Kristin B AU - Highland KB AD - Division of Pulmonary and Critical Care Medicine, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, SC. LA - eng GR - R01 AR044684/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Review DEP - 20090218 PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Amino Acyl-tRNA Synthetases/*immunology MH - Arthritis/complications/immunology MH - Autoantibodies/*immunology MH - Autoimmune Diseases/*diagnosis/epidemiology MH - Cohort Studies MH - Female MH - Fever/complications/immunology MH - Hand/physiopathology MH - Humans MH - Incidence MH - Lung Diseases, Interstitial/complications/immunology MH - Male MH - Middle Aged MH - Polymyositis/complications/immunology MH - Prognosis MH - Raynaud Disease/complications/immunology MH - Retrospective Studies MH - Risk Factors MH - Sensitivity and Specificity MH - Severity of Illness Index MH - Sex Factors MH - Syndrome MH - Young Adult RF - 33 EDAT- 2009/02/20 09:00 MHDA- 2009/07/08 09:00 CRDT- 2009/02/20 09:00 PHST- 2009/02/20 09:00 [entrez] PHST- 2009/02/20 09:00 [pubmed] PHST- 2009/07/08 09:00 [medline] AID - S0012-3692(09)60360-3 [pii] AID - 10.1378/chest.08-2233 [doi] PST - ppublish SO - Chest. 2009 Jun;135(6):1550-1556. doi: 10.1378/chest.08-2233. Epub 2009 Feb 18. PMID- 30323370 OWN - NLM STAT- MEDLINE DCOM- 20191021 LR - 20191022 IS - 0030-9982 (Print) IS - 0030-9982 (Linking) VI - 68 IP - 6 DP - 2018 Jun TI - Clinical and immunological profile in patients with mixed connective tissue disease. PG - 959-962 AB - Mixed connective tissue disease (MCTD) is a rare disease and presents with varied overlapping symptoms of different connective tissue disorders. Many patients evolve into other connective tissue disorders with the passage of time. The case series included 20 patients with the diagnosis of MCTD, registered at the Rheumatology Clinic of Jinnah Postgraduate Medical Centre (JPMC), Karachi, from June 2010 to May 2015. Of these, 16 (80.0%) were female and 4 (20.0%) patients were male. The mean age was 30.5±8.9 years and the mean duration of illness was 4.5±2 years. Commonest presenting symptom was arthralgia in 17 (85%) patients. All the patients had positive ANA and anti-RNP antibodies. Over the disease course of 6 years, 2 (10%) patients evolved into Systemic lupus erythematosus (SLE); One each (5%) into Sjogren's syndrome, Scleroderma and Rheumatoid arthritis. FAU - Ahsan, Tasnim AU - Ahsan T AD - Medical Unit-II, Jinnah Postgraduate Medical Centre, Karachi. FAU - Erum, Uzma AU - Erum U AD - Medical Unit-II, Jinnah Postgraduate Medical Centre, Karachi. FAU - Dahani, Assadullah AU - Dahani A AD - Medical Unit-II, Jinnah Postgraduate Medical Centre, Karachi. FAU - Khowaja, Danish AU - Khowaja D AD - Medical Unit-II, Jinnah Postgraduate Medical Centre, Karachi. LA - eng PT - Journal Article PL - Pakistan TA - J Pak Med Assoc JT - JPMA. The Journal of the Pakistan Medical Association JID - 7501162 RN - 0 (Autoantibodies) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM MH - Adult MH - Arthralgia/etiology/physiopathology MH - Autoantibodies/immunology MH - Erythema/etiology/physiopathology MH - Facial Dermatoses/etiology/physiopathology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/complications/immunology/*physiopathology MH - Oral Ulcer/etiology/physiopathology MH - Pakistan MH - Photosensitivity Disorders/etiology/physiopathology MH - Raynaud Disease/etiology/physiopathology MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Synovitis/etiology/physiopathology MH - Young Adult OTO - NOTNLM OT - Mixed connective tissue disease (MCTD), Un-differentiated connective tissue disease (UDCT), anti-ribonucleoprotien antibody (RNP). EDAT- 2018/10/17 06:00 MHDA- 2019/10/23 06:00 CRDT- 2018/10/17 06:00 PHST- 2018/10/17 06:00 [entrez] PHST- 2018/10/17 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] AID - 8742 [pii] PST - ppublish SO - J Pak Med Assoc. 2018 Jun;68(6):959-962. PMID- 18971190 OWN - NLM STAT- MEDLINE DCOM- 20091005 LR - 20081030 IS - 1673-4254 (Print) IS - 1673-4254 (Linking) VI - 28 IP - 10 DP - 2008 Oct TI - [Clinical analysis of patients with systemic lupus erythematosus and concomitant pulmonary hypertension]. PG - 1860-3 AB - OBJECTIVE: To investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension (PAH) in patients with systemic lupus erythematosus (SLE). METHODS: From January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively. RESULTS: The incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34-/+12 years. Positive correlations were found between the occurrence of PAH and the Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia (P < 0.05). Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis. CONCLUSION: PAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis. FAU - Luo, Ri-qiang AU - Luo RQ AD - Department of Rheumatology, Guangdong Provincial People's Hospital, Guangzhou 510080, China. luorq@tom.com FAU - Lei, Yun-xia AU - Lei YX FAU - Zhang, Xiao AU - Zhang X FAU - Liang, Fei AU - Liang F LA - chi PT - English Abstract PT - Journal Article PL - China TA - Nan Fang Yi Ke Da Xue Xue Bao JT - Nan fang yi ke da xue xue bao = Journal of Southern Medical University JID - 101266132 SB - IM MH - Adolescent MH - Adult MH - Aged MH - China/epidemiology MH - Early Diagnosis MH - Echocardiography MH - Female MH - Humans MH - Hypertension, Pulmonary/*complications/*diagnosis/epidemiology MH - Lupus Erythematosus, Systemic/*complications/*diagnosis MH - Male MH - Middle Aged MH - Raynaud Disease/complications MH - Retrospective Studies EDAT- 2008/10/31 09:00 MHDA- 2009/10/06 06:00 CRDT- 2008/10/31 09:00 PHST- 2008/10/31 09:00 [pubmed] PHST- 2009/10/06 06:00 [medline] PHST- 2008/10/31 09:00 [entrez] PST - ppublish SO - Nan Fang Yi Ke Da Xue Xue Bao. 2008 Oct;28(10):1860-3. PMID- 26525594 OWN - NLM STAT- MEDLINE DCOM- 20161101 LR - 20161230 IS - 1773-0597 (Electronic) IS - 0181-5512 (Linking) VI - 39 IP - 1 DP - 2016 Jan TI - [Retinal arteriolar tortuosity in a patient with HANAC syndrome (dominant hereditary angiopathy with nephropathy, aneurysms and muscle cramps)]. PG - 129-30 LID - S0181-5512(15)00335-6 [pii] LID - 10.1016/j.jfo.2015.04.014 [doi] FAU - Beuste, T AU - Beuste T AD - Service d'ophtalmologie (15(e) étage), CHU de Caen, avenue de la Côte-de-Nacre, 14000 Caen, France. Electronic address: thomas.beuste@hotmail.fr. FAU - Denion, É AU - Denion É AD - Service d'ophtalmologie (15(e) étage), CHU de Caen, avenue de la Côte-de-Nacre, 14000 Caen, France. FAU - Saguet, P AU - Saguet P AD - Service d'ophtalmologie (15(e) étage), CHU de Caen, avenue de la Côte-de-Nacre, 14000 Caen, France. FAU - Miocque, S AU - Miocque S AD - Service d'ophtalmologie (15(e) étage), CHU de Caen, avenue de la Côte-de-Nacre, 14000 Caen, France. LA - fre PT - Journal Article TT - Tortuosité artériolaire dans le cadre du syndrome HANAC (angiopathie héréditaire, néphropathie, anévrisme et crampe musculaire). DEP - 20151031 PL - France TA - J Fr Ophtalmol JT - Journal francais d'ophtalmologie JID - 7804128 RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Arterioles/pathology MH - Humans MH - Muscle Cramp/*pathology MH - Raynaud Disease/*pathology MH - Retinal Vessels/*pathology EDAT- 2015/11/04 06:00 MHDA- 2016/11/02 06:00 CRDT- 2015/11/04 06:00 PHST- 2015/04/15 00:00 [received] PHST- 2015/04/23 00:00 [revised] PHST- 2015/04/24 00:00 [accepted] PHST- 2015/11/04 06:00 [entrez] PHST- 2015/11/04 06:00 [pubmed] PHST- 2016/11/02 06:00 [medline] AID - S0181-5512(15)00335-6 [pii] AID - 10.1016/j.jfo.2015.04.014 [doi] PST - ppublish SO - J Fr Ophtalmol. 2016 Jan;39(1):129-30. doi: 10.1016/j.jfo.2015.04.014. Epub 2015 Oct 31. PMID- 20114113 OWN - NLM STAT- MEDLINE DCOM- 20100218 LR - 20151119 IS - 1549-4713 (Electronic) IS - 0161-6420 (Linking) VI - 117 IP - 1 DP - 2010 Jan TI - Combination brimonidine and timolol. PG - 193-193.e1 LID - 10.1016/j.ophtha.2009.08.038 [doi] FAU - Kerr, Nathan M AU - Kerr NM FAU - Gross, Keith A AU - Gross KA FAU - Tombleson, Michael AU - Tombleson M FAU - Danesh-Meyer, Helen V AU - Danesh-Meyer HV LA - eng PT - Case Reports PT - Letter PL - United States TA - Ophthalmology JT - Ophthalmology JID - 7802443 RN - 0 (Adrenergic alpha-Agonists) RN - 0 (Adrenergic beta-Antagonists) RN - 0 (Drug Combinations) RN - 0 (Quinoxalines) RN - 4S9CL2DY2H (Brimonidine Tartrate) RN - 817W3C6175 (Timolol) SB - IM MH - Adrenergic alpha-Agonists/*adverse effects MH - Adrenergic beta-Antagonists/*adverse effects MH - Aged MH - Brimonidine Tartrate MH - Drug Combinations MH - Drug Hypersensitivity/*etiology/physiopathology MH - Humans MH - Male MH - Necrosis/chemically induced MH - Quinoxalines/*adverse effects MH - Raynaud Disease/complications MH - Skin/*pathology MH - Timolol/*adverse effects EDAT- 2010/02/02 06:00 MHDA- 2010/02/19 06:00 CRDT- 2010/02/02 06:00 PHST- 2009/07/30 00:00 [received] PHST- 2009/08/27 00:00 [accepted] PHST- 2010/02/02 06:00 [entrez] PHST- 2010/02/02 06:00 [pubmed] PHST- 2010/02/19 06:00 [medline] AID - S0161-6420(09)00976-2 [pii] AID - 10.1016/j.ophtha.2009.08.038 [doi] PST - ppublish SO - Ophthalmology. 2010 Jan;117(1):193-193.e1. doi: 10.1016/j.ophtha.2009.08.038. PMID- 20453602 OWN - NLM STAT- MEDLINE DCOM- 20100520 LR - 20210108 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 89 IP - 3 DP - 2010 May TI - Systemic sclerosis: establishing diagnostic criteria. PG - 159-165 LID - 10.1097/MD.0b013e3181dde28d [doi] AB - We designed the current study to describe the spectrum of disease expression in systemic sclerosis (SSc) in a large cohort and to develop diagnostic criteria for SSc. We assessed patients in the Canadian Scleroderma Research Group Registry by standardized history, physical examination, and laboratory testing. We performed regression tree analysis to determine the sensitivity of various clinical and serologic features for diagnosing SSc. Over 1000 (n = 1048) patients were included: mean age 55 (+/- 12) years, 87% female, 90% white, mean disease duration 11 (+/- 10) years, and 38% with diffuse skin involvement. Common clinical features were Raynaud phenomenon (98%), sclerodactyly (92%), clinically visible mat-like telangiectasias (78%), skin involvement above the fingers (58%), lung fibrosis (35%), pulmonary hypertension (15%), and gastrointestinal tract involvement (mean number of self-reported symptoms, 4 (+/- 3) out of a possible 14). Almost 90% of patients had at least 1 SSc-related autoantibody, including 34% with anti-centromere and 16% with anti-topoisomerase I. The sensitivity of Raynaud and proximal finger skin thickening for the diagnosis of SSc was only 57%. Addition of clinically visible mat-like telangiectasias and SSc-related antibodies improved the sensitivity to 97%. We conclude that important diagnostic clues in patients with SSc include Raynaud phenomenon, skin involvement, clinically visible mat-like telangiectasias, and SSc-related autoantibodies. FAU - Hudson, Marie AU - Hudson M AD - From Jewish General Hospital and McGill University (MH, MB), Montreal, Quebec; and University of Calgary (MJF), Calgary, Alberta, Canada. FAU - Fritzler, Marvin J AU - Fritzler MJ FAU - Baron, Murray AU - Baron M CN - Canadian Scleroderma Research Group (CSRG) LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Canada MH - Cohort Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/*diagnosis/immunology MH - Skin Diseases/etiology MH - Telangiectasis/etiology EDAT- 2010/05/11 06:00 MHDA- 2010/05/21 06:00 CRDT- 2010/05/11 06:00 PHST- 2010/05/11 06:00 [entrez] PHST- 2010/05/11 06:00 [pubmed] PHST- 2010/05/21 06:00 [medline] AID - 00005792-201005000-00003 [pii] AID - 10.1097/MD.0b013e3181dde28d [doi] PST - ppublish SO - Medicine (Baltimore). 2010 May;89(3):159-165. doi: 10.1097/MD.0b013e3181dde28d. PMID- 19888500 OWN - NLM STAT- MEDLINE DCOM- 20100115 LR - 20220316 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 61 IP - 3 DP - 2009 Jul-Sep TI - [Plasma-exchange in the treatment of systemic rheumatic diseases: past and present experience]. PG - 161-4 FAU - Cozzi, F AU - Cozzi F FAU - Marson, P AU - Marson P LA - ita PT - Editorial TT - Il trattamento con plasma-exchange delle malattie reumatiche sistemiche, ieri e oggi. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Antiphospholipid Syndrome/therapy MH - Arthritis, Rheumatoid/therapy MH - Autoimmune Diseases/therapy MH - Cryoglobulinemia/therapy MH - Dermatomyositis/therapy MH - Granulomatosis with Polyangiitis/therapy MH - Humans MH - Lupus Erythematosus, Systemic/therapy MH - *Plasma Exchange MH - Raynaud Disease/therapy MH - Rheumatic Diseases/immunology/*therapy MH - Scleroderma, Systemic/therapy MH - Sjogren's Syndrome/therapy MH - Systemic Vasculitis/therapy MH - Treatment Outcome EDAT- 2009/11/06 06:00 MHDA- 2010/01/16 06:00 CRDT- 2009/11/06 06:00 PHST- 2009/11/06 06:00 [entrez] PHST- 2009/11/06 06:00 [pubmed] PHST- 2010/01/16 06:00 [medline] AID - 10.4081/reumatismo.2009.161 [doi] PST - ppublish SO - Reumatismo. 2009 Jul-Sep;61(3):161-4. doi: 10.4081/reumatismo.2009.161. PMID- 18018319 OWN - NLM STAT- MEDLINE DCOM- 20071120 LR - 20151119 IS - 1089-1102 (Print) IS - 1089-1102 (Linking) VI - 12 IP - 1 DP - 2007 Aug TI - Viagra and health: beyond ED. PG - 1-4 LA - eng PT - Journal Article PL - United States TA - Harv Mens Health Watch JT - Harvard men's health watch JID - 9802701 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) MH - Altitude Sickness/drug therapy MH - Erectile Dysfunction/drug therapy MH - Heart Diseases/drug therapy MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Male MH - Piperazines/adverse effects/*therapeutic use MH - Purines/adverse effects/therapeutic use MH - Raynaud Disease/drug therapy MH - Sildenafil Citrate MH - Stroke/drug therapy MH - Sulfones/adverse effects/*therapeutic use MH - Vasodilator Agents/adverse effects/*therapeutic use EDAT- 2007/11/21 09:00 MHDA- 2007/12/06 09:00 CRDT- 2007/11/21 09:00 PHST- 2007/11/21 09:00 [pubmed] PHST- 2007/12/06 09:00 [medline] PHST- 2007/11/21 09:00 [entrez] PST - ppublish SO - Harv Mens Health Watch. 2007 Aug;12(1):1-4. PMID- 17437659 OWN - NLM STAT- MEDLINE DCOM- 20070626 LR - 20191110 IS - 1523-3774 (Print) IS - 1523-3774 (Linking) VI - 9 IP - 1 DP - 2007 Apr TI - Novel paradigm for treating vasculopathy in systemic sclerosis: vascular progenitor cells and statins. PG - 1-3 FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Division of Rheumatology, Northwestern University Feinberg School of Medicine, McGaw Pavilion, 240 East Huron Street, Chicago, IL 60611-2909, USA. FAU - Varga, John AU - Varga J LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) SB - IM MH - Endothelial Cells/drug effects MH - Humans MH - Hydroxymethylglutaryl-CoA Reductase Inhibitors/*therapeutic use MH - Neovascularization, Physiologic/*drug effects MH - Pilot Projects MH - Raynaud Disease/drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Stem Cells/drug effects RF - 12 EDAT- 2007/04/18 09:00 MHDA- 2007/06/27 09:00 CRDT- 2007/04/18 09:00 PHST- 2007/04/18 09:00 [pubmed] PHST- 2007/06/27 09:00 [medline] PHST- 2007/04/18 09:00 [entrez] AID - 10.1007/s11926-007-0014-1 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2007 Apr;9(1):1-3. doi: 10.1007/s11926-007-0014-1. PMID- 17395311 OWN - NLM STAT- MEDLINE DCOM- 20070822 LR - 20070423 IS - 1874-1754 (Electronic) IS - 0167-5273 (Linking) VI - 118 IP - 2 DP - 2007 May 31 TI - Treatment of refractory vasoespastic angina with corticosteroids. A case report. PG - e51-3 FAU - Domínguez Franco, Antonio J AU - Domínguez Franco AJ FAU - Gómez Doblas, Juan J AU - Gómez Doblas JJ FAU - García Pinilla, J M AU - García Pinilla JM FAU - Hernández García, J M AU - Hernández García JM FAU - Jiménez Navarro, Manuel AU - Jiménez Navarro M FAU - Alonso Briales, Juan H AU - Alonso Briales JH FAU - de Teresa Galvan, Eduardo AU - de Teresa Galvan E LA - eng PT - Case Reports PT - Letter DEP - 20070328 PL - Netherlands TA - Int J Cardiol JT - International journal of cardiology JID - 8200291 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Administration, Oral MH - Adrenal Cortex Hormones/*therapeutic use MH - Angina Pectoris/complications/diagnosis/*drug therapy MH - Coronary Vasospasm/complications/diagnosis/*drug therapy MH - Eosinophilia/complications MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/complications MH - Treatment Outcome EDAT- 2007/03/31 09:00 MHDA- 2007/08/23 09:00 CRDT- 2007/03/31 09:00 PHST- 2006/11/09 00:00 [received] PHST- 2006/12/31 00:00 [accepted] PHST- 2007/03/31 09:00 [pubmed] PHST- 2007/08/23 09:00 [medline] PHST- 2007/03/31 09:00 [entrez] AID - S0167-5273(07)00372-5 [pii] AID - 10.1016/j.ijcard.2006.12.058 [doi] PST - ppublish SO - Int J Cardiol. 2007 May 31;118(2):e51-3. doi: 10.1016/j.ijcard.2006.12.058. Epub 2007 Mar 28. PMID- 16614599 OWN - NLM STAT- MEDLINE DCOM- 20060531 LR - 20191026 IS - 0755-4982 (Print) IS - 0755-4982 (Linking) VI - 35 IP - 4 Pt 1 DP - 2006 Apr TI - [Bosentan for treatment of active digital ulcers in patients with systemic sclerosis]. PG - 587-92 AB - OBJECTIVES: To describe the effect of bosentan and its dual inhibition of endothelin-1 ETA and ETB receptors on digital ulcers in patients with systemic sclerosis (SSc). METHODS: Patients receiving bosentan for SSc-related digital ulcers were identified in eight centers, and their characteristics and follow-up were recorded. RESULTS: Nine (six with diffuse and three with limited cutaneous forms of SSc) patients (median age: 54 years) had received bosentan for digital ulcers. Complete healing occurred in seven (median time to improvement: 4 weeks). Another experienced a significant decrease in the number of ulcers (from 22 to 5) in 8 weeks, while one had no improvement. After a median follow-up of 24.3 months, only one recurrence was observed. Raynaud phenomenon improved in all but one patient. DISCUSSION: These data suggest that some patients may benefit from bosentan to treat digital ulcers. The short time to healing in these patients with rather chronic ulcers argues strongly in favor of its use. These results also strengthen the evidence that endothelin-1 plays an important role in the vascular manifestations of SSc. CONCLUSION: Bosentan can be effective in the treatment of digital ulcers in some SSc patients with SSc, probably especially those involving substantial ischemia. Bosentan is not a first-line drug in this indication yet and must be carefully used by specialists in SSc. Forthcoming results from the international RAPIDS-2 study should clarify the indications for bosentan in the treatment of SSc-related digital ulcers. FAU - Launay, David AU - Launay D AD - Service de Médecine Interne, Centre National de Référence Atteintes Vasculaires de la Sclérodermie, Hôpital Claude-Huriez, CHU de Lille, Université Lille 2. d-launay@chru-lille.fr FAU - Diot, Elisabeth AU - Diot E FAU - Pasquier, Elisabeth AU - Pasquier E FAU - Mouthon, Luc AU - Mouthon L FAU - Boullanger, Nadine AU - Boullanger N FAU - Fain, Olivier AU - Fain O FAU - Jego, Patrick AU - Jego P FAU - Carpentier, Patrick AU - Carpentier P FAU - Hatron, Pierre-Yves AU - Hatron PY FAU - Hachulla, Eric AU - Hachulla E LA - fre PT - Journal Article TT - Le bosentan dans le traitement des ulcères digitaux évolutifs au cours de la sclérodermie systémique (9 cas). PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Antibodies, Antinuclear) RN - 0 (Endothelin-1) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/immunology MH - Bosentan MH - Endothelin-1/*antagonists & inhibitors MH - Female MH - Fingers/*blood supply/physiopathology MH - Follow-Up Studies MH - Humans MH - Ischemia/physiopathology MH - Male MH - Middle Aged MH - Raynaud Disease/chemically induced/epidemiology MH - Scleroderma, Systemic/*drug therapy/*epidemiology/immunology MH - Skin Ulcer/*epidemiology/physiopathology MH - Sulfonamides/adverse effects/*therapeutic use MH - Time Factors MH - Treatment Outcome EDAT- 2006/04/15 09:00 MHDA- 2006/06/01 09:00 CRDT- 2006/04/15 09:00 PHST- 2006/04/15 09:00 [pubmed] PHST- 2006/06/01 09:00 [medline] PHST- 2006/04/15 09:00 [entrez] AID - S0755-4982(06)74645-0 [pii] AID - 10.1016/s0755-4982(06)74645-0 [doi] PST - ppublish SO - Presse Med. 2006 Apr;35(4 Pt 1):587-92. doi: 10.1016/s0755-4982(06)74645-0. PMID- 28981361 OWN - NLM STAT- MEDLINE DCOM- 20171205 LR - 20171205 IS - 1546-3141 (Electronic) IS - 0361-803X (Linking) VI - 209 IP - 6 DP - 2017 Dec TI - Follow-Up of Treatment Response With Dynamic Doppler Ultrasound in Raynaud Phenomenon. PG - W388-W394 LID - 10.2214/AJR.17.18143 [doi] AB - OBJECTIVE: The purpose of this study was to investigate the role of flow parameters obtained with dynamic Doppler ultrasound in the objective follow-up of treatment response in patients with Raynaud phenomenon (RP). SUBJECTS AND METHODS: The study included 33 patients with newly diagnosed primary RP, 31 with secondary RP, and 26 healthy participants (control subjects). Both groups of patients with RP underwent sonography before and after treatment. The control group underwent sonography once. Baseline digital arterial diameter and flow volume were measured at room temperature. After cold provocation, diameter and flow volume were measured again, and flow starting time and flow normalizing time were recorded. Data were measured as mean (± SD) values. RESULTS: Baseline diameter did not significantly increase in either group after treatment (p > 0.05) (primary RP pretreatment, 0.79 ± 0.17 mm; posttreatment, 0.82 ± 0.19 mm; secondary RP pretreatment, 0.66 ± 0.13 mm; posttreatment, 0.68 ± 0.14 mm). Baseline flow volume increased significantly in both groups (p < 0.01) (primary RP pretreatment, 3.08 ± 2.96 mL/min; posttreatment, 3.91 ± 3.39 mL/min; secondary RP pretreatment, 2.14 ± 1.94 mL/min; posttreatment, 2.80 ± 2.15 mL/min). Cold provocation diameter increased significantly in both groups after treatment (p < 0.01) (primary RP pretreatment, 0.63 ± 0.15 mm; posttreatment, 0.70 ± 0.16 mm; secondary RP pretreatment, 0.56 ± 0.15 mm; posttreatment, 0.63 ± 0.13 mm). Cold provocation flow volume increased significantly in both groups after treatment (p < 0.01) (primary RP pretreatment, 1.18 ± 1.26 mL/min; posttreatment, 2.17 ± 2.16 mL/min; secondary RP pretreatment, 1.07 ± 1.40 mL/min; posttreatment, 1.46 ± 1.67 mL/min). After treatment, there was no statistically significant increase in flow starting time in patients with primary RP (p > 0.05), but there was a significant increase in patients with secondary RP (p < 0.05) (primary RP pretreatment, 1.15 ± 2.27 minutes; posttreatment, 0.61 ± 1.41 minutes; secondary RP pretreatment, 3.13 ± 4.81 minutes; posttreatment, 1.58 ± 2.36 minutes). After treatment, flow volume normalizing time improved significantly in both groups (p < 0.01) (primary RP pretreatment, 7.24 ± 7.60 minutes; posttreatment, 3.84 ± 3.39 minutes; secondary RP pretreatment, 9.58 ± 8.49 minutes; posttreatment, 4.32 ± 3.56 minutes). Among patients with primary RP, the posttreatment flow starting time was similar to that in the control group. Despite improvements, all remaining parameters differed in the treatment group compared with the control group. CONCLUSION: Doppler ultrasound can be used effectively to monitor RP treatment. Blood flow volume can be measured without cold provocation to facilitate follow-up care of patients with RP. FAU - Toprak, Ugur AU - Toprak U AD - 1 Department of Radiology, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, 26480, Turkey. FAU - Ozbalkan, Zeynep AU - Ozbalkan Z AD - 2 Department of Rheumatology, Ankara Numune Education and Research Hospital, Ankara, Turkey. FAU - Erdugan, Murat AU - Erdugan M AD - 3 Department of Rheumatology, Istanbul University Capa School of Medicine, Istanbul, Turkey. FAU - Parlak, Selcuk AU - Parlak S AD - 4 Department of Radiology, Ankara Numune Education and Research Hospital, Ankara, Turkey. FAU - Sandıkcı, Sevinc Can AU - Sandıkcı SC AD - 2 Department of Rheumatology, Ankara Numune Education and Research Hospital, Ankara, Turkey. FAU - Kaya, Tunca AU - Kaya T AD - 4 Department of Radiology, Ankara Numune Education and Research Hospital, Ankara, Turkey. FAU - Şaylısoy, Suzan AU - Şaylısoy S AD - 1 Department of Radiology, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, 26480, Turkey. LA - eng PT - Journal Article DEP - 20171005 PL - United States TA - AJR Am J Roentgenol JT - AJR. American journal of roentgenology JID - 7708173 SB - IM MH - Adult MH - Arm/*blood supply MH - Blood Flow Velocity MH - Case-Control Studies MH - Female MH - Humans MH - Male MH - Raynaud Disease/*diagnostic imaging/*therapy MH - Treatment Outcome MH - *Ultrasonography, Doppler OTO - NOTNLM OT - Doppler OT - Raynaud phenomenon OT - digital artery OT - treatment OT - ultrasound EDAT- 2017/10/06 06:00 MHDA- 2017/12/06 06:00 CRDT- 2017/10/06 06:00 PHST- 2017/10/06 06:00 [pubmed] PHST- 2017/12/06 06:00 [medline] PHST- 2017/10/06 06:00 [entrez] AID - 10.2214/AJR.17.18143 [doi] PST - ppublish SO - AJR Am J Roentgenol. 2017 Dec;209(6):W388-W394. doi: 10.2214/AJR.17.18143. Epub 2017 Oct 5. PMID- 20544242 OWN - NLM STAT- MEDLINE DCOM- 20101123 LR - 20260128 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 29 IP - 9 DP - 2010 Sep TI - Anti-Ro antibody and clinical manifestations: lessons from systemic lupus erythematosus in the elderly. PG - 1077 LID - 10.1007/s10067-010-1520-5 [doi] FAU - Leng, Rui-Xue AU - Leng RX FAU - Qin, Wei-Zi AU - Qin WZ FAU - Pan, Hai-Feng AU - Pan HF FAU - Ye, Dong-Qing AU - Ye DQ LA - eng PT - Letter DEP - 20100612 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies) RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantigens) RN - 0 (Ribonucleoproteins) RN - 0 (SS-B Antigen) RN - 0 (SS-A antibodies) SB - IM MH - Aged MH - Aged, 80 and over MH - Antibodies/blood MH - Antibodies, Antinuclear/*blood MH - Autoantigens/immunology MH - Humans MH - Lupus Erythematosus, Systemic/*immunology MH - Photosensitivity Disorders/immunology MH - Raynaud Disease/immunology MH - Ribonucleoproteins/immunology MH - Vasculitis, Leukocytoclastic, Cutaneous/immunology MH - SS-B Antigen EDAT- 2010/06/15 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/06/15 06:00 PHST- 2010/05/31 00:00 [received] PHST- 2010/06/02 00:00 [accepted] PHST- 2010/06/15 06:00 [entrez] PHST- 2010/06/15 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 10.1007/s10067-010-1520-5 [doi] PST - ppublish SO - Clin Rheumatol. 2010 Sep;29(9):1077. doi: 10.1007/s10067-010-1520-5. Epub 2010 Jun 12. PMID- 18799073 OWN - NLM STAT- MEDLINE DCOM- 20090205 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 3 Suppl 49 DP - 2008 May-Jun TI - Significant improvement of axillary artery stenosis in a 67 year-old woman with giant cell arteritis. PG - S151-2 FAU - Papadopoulos, C G AU - Papadopoulos CG FAU - Voulgari, P V AU - Voulgari PV FAU - Zioga, A AU - Zioga A FAU - Miltiadous, G A AU - Miltiadous GA FAU - Nicolopoulos, P AU - Nicolopoulos P FAU - Elisaf, M S AU - Elisaf MS FAU - Drosos, A A AU - Drosos AA LA - eng PT - Case Reports PT - Letter PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 9PHQ9Y1OLM (Prednisolone) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Aged MH - Axillary Artery/*pathology MH - Constriction, Pathologic/complications/drug therapy MH - Drug Therapy, Combination MH - Female MH - Giant Cell Arteritis/*drug therapy/*pathology MH - Humans MH - Methotrexate/*therapeutic use MH - Prednisolone/*therapeutic use MH - Raynaud Disease/complications MH - Remission Induction EDAT- 2008/10/24 09:00 MHDA- 2009/02/06 09:00 CRDT- 2008/10/24 09:00 PHST- 2008/10/24 09:00 [pubmed] PHST- 2009/02/06 09:00 [medline] PHST- 2008/10/24 09:00 [entrez] AID - 2377 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 May-Jun;26(3 Suppl 49):S151-2. PMID- 20349257 OWN - NLM STAT- MEDLINE DCOM- 20110512 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 30 IP - 2 DP - 2011 Feb TI - Cutaneous vasculitis in systemic lupus erythematosus: association with anti-ribosomal P protein antibody and Raynaud phenomenon. PG - 173-7 LID - 10.1007/s10067-010-1432-4 [doi] AB - Ninety-one consecutive systemic lupus erythematosus (SLE) patients (American College of Rheumatology criteria) with a history of cutaneous vasculitis were compared to 163 SLE controls without this clinical manifestation from July to December 2007 in order to determine the possible clinical and serological association of this manifestation. Data were obtained in an ongoing electronic database protocol and autoantibodies to anti-double-stranded DNA, anti-Sm, anti-RNP, anti-Ro/SS-A, anti-La/SS-B, and anticardiolipin and ribosomal P protein antibody (anti-P) were detected by standard techniques. Exclusion criteria were the presence of anti-phospholipid syndrome or antibodies, Sjögren syndrome, and a history of thrombosis. The mean age (38.5 ± 11.5 vs. 37.8 ± 11.6 years, p = 0.635), disease duration (12.5 ± 7.8 vs. 11.8 ± 7.9 years, p = 0.501), and frequency of white race (71.4% vs. 70.5%, p = 0.872) and female sex (96.8% vs. 93.7%, p = 0.272) were comparable in both groups. The vasculitis group had a higher frequency of malar rash (97.9% vs. 87.4%, p = 0.004), photosensitivity (91.4% vs. 81.6%, p = 0.030), and Raynaud phenomenon (RP; 27.7% vs. 7.5%, p < 0.001), whereas all other clinical manifestation including renal and central nervous system involvements were similar to the control group. Laboratorial data revealed that only anti-P (35.1% vs. 12.1%, p < 0.001) was more frequent in patients with vasculitis. In a multivariate logistic regression model, cutaneous vasculitis was associated to the presence of RP (OR = 3.70; 95% confidence interval [CI] = 1.73-8.00) and anti-P (OR = 3.42; 95% CI = 1.76-6.66). In summary, SLE cutaneous vasculitis characterizes a subgroup of patients with more RP and anti-P antibodies but not accompanied by a higher frequency of renal and central nervous system involvements. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AD - Division of Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, Sala 3190, 3° andar, CEP 01246-903, São Paulo, Brazil. samuel.shinjo@gmail.com FAU - Bonfá, Eloísa AU - Bonfá E LA - eng PT - Journal Article DEP - 20100328 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) RN - 0 (Ribosomal Proteins) RN - 0 (ribosomal protein P0) SB - IM MH - Adult MH - Antibodies, Antinuclear/*immunology MH - Chi-Square Distribution MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/complications/*immunology MH - Male MH - Middle Aged MH - Odds Ratio MH - Raynaud Disease/complications/*immunology MH - Ribosomal Proteins/*immunology MH - Skin Diseases, Vascular/complications/*immunology EDAT- 2010/03/30 06:00 MHDA- 2011/05/13 06:00 CRDT- 2010/03/30 06:00 PHST- 2009/09/26 00:00 [received] PHST- 2010/03/10 00:00 [accepted] PHST- 2009/12/29 00:00 [revised] PHST- 2010/03/30 06:00 [entrez] PHST- 2010/03/30 06:00 [pubmed] PHST- 2011/05/13 06:00 [medline] AID - 10.1007/s10067-010-1432-4 [doi] PST - ppublish SO - Clin Rheumatol. 2011 Feb;30(2):173-7. doi: 10.1007/s10067-010-1432-4. Epub 2010 Mar 28. PMID- 27307535 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20220410 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 43 IP - 9 DP - 2016 Sep TI - The Recurrence of Digital Ulcers in Patients with Systemic Sclerosis after Discontinuation of Oral Treprostinil. PG - 1665-71 LID - 10.3899/jrheum.151437 [doi] AB - OBJECTIVE: Prior studies investigating the efficacy of oral treprostinil to treat digital ulcers (DU) in systemic sclerosis (SSc)-associated Raynaud phenomenon have yielded conflicting results. In this investigation, we examined whether DU burden increased after patients withdrew from oral treprostinil that was administered during an open-label extension study. METHODS: A multicenter, retrospective study was conducted to determine DU burden in the year after withdrawal from oral treprostinil. DU burden 3-6 months (Time A) and > 6-12 months (Time B) after drug withdrawal was compared with DU burden at baseline, defined as the last day receiving drug in the open-label extension study, by a paired Student t test. Changes in DU burden while receiving drug in the open-label study were compared with changes in DU burden at Time B by a paired Student t test. RESULTS: Fifty-one patients from 9 clinical sites were included for analysis. DU burden increased significantly from baseline (mean 0.47) to Time A (mean 2.1, p = 0.002, n = 23) and Time B (mean 1.45, p = 0.013, n = 30). Total DU burden decreased during oral treprostinil exposure (mean change -0.6) and then increased by Time B (mean change 1.05, p = 0.0027 for comparison, n = 30). In the year after drug withdrawal, many patients required vasodilator therapy and pain medications. Three patients were hospitalized for complications from DU, and 4 patients required surgery for DU. CONCLUSION: Total DU burden increased significantly after discontinuation of oral treprostinil. These data provide supportive evidence of a beneficial effect of oral treprostinil for the vascular complications of SSc and suggest that further study is warranted. FAU - Shah, Ami A AU - Shah AA AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. Ami.Shah@jhmi.edu. FAU - Schiopu, Elena AU - Schiopu E AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Chatterjee, Soumya AU - Chatterjee S AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Csuka, Mary Ellen AU - Csuka ME AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Frech, Tracy AU - Frech T AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Goldberg, Avram AU - Goldberg A AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Spiera, Robert AU - Spiera R AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Peng, Stanford L AU - Peng SL AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - McBride, Ryan J AU - McBride RJ AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Cleveland, Jody M AU - Cleveland JM AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. FAU - Steen, Virginia AU - Steen V AD - From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland; University of Michigan, Ann Arbor, Michigan; Cleveland Clinic, Cleveland, Ohio; Medical College of Wisconsin, Milwaukee, Wisconsin; University of Utah, Salt Lake City, Utah; New York University (NYU) Langone Medical Center, Lake Success; Hospital for Special Surgery, New York, New York; Benaroya Research Institute at Virginia Mason, Seattle, Washington; Georgetown University, Washington, DC; University of North Carolina at Chapel Hill, Chapel Hill; United Therapeutics Corporation, Research Triangle Park, North Carolina, USA.A.A. Shah, MD, MHS, Assistant Professor of Medicine, Director of Clinical and Translational Research, Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University School of Medicine; E. Schiopu, MD, Assistant Professor of Internal Medicine, University of Michigan; S. Chatterjee, MD, Associate Professor of Medicine, Cleveland Clinic; M.E. Csuka, MD, Professor of Medicine, Medical College of Wisconsin; T. Frech, MD, Associate Professor of Medicine, University of Utah; A. Goldberg, MD, Clinical Assistant Professor, NYU Langone Medical Center; R. Spiera, MD, Professor of Clinical Medicine, Hospital for Special Surgery; S.L. Peng, MD, PhD, Vice President, Clinical Development, Benaroya Research Institute at Virginia Mason; R.J. McBride, DrPH, Student, University of North Carolina at Chapel Hill; J.M. Cleveland, MS, Director, Biostatistics, United Therapeutics Corp.; V. Steen, MD, Professor of Medicine, Georgetown University. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20160615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antihypertensive Agents) RN - DCR9Z582X0 (Epoprostenol) RN - RUM6K67ESG (treprostinil) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*therapeutic use MH - Epoprostenol/*analogs & derivatives/therapeutic use MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Recurrence MH - Retrospective Studies MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology OTO - NOTNLM OT - DIGITAL ULCERS OT - RAYNAUD PHENOMENON OT - SYSTEMIC SCLEROSIS OT - TREPROSTINIL DIOLAMINE EDAT- 2016/06/17 06:00 MHDA- 2017/12/22 06:00 CRDT- 2016/06/17 06:00 PHST- 2016/05/19 00:00 [accepted] PHST- 2016/06/17 06:00 [entrez] PHST- 2016/06/17 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] AID - jrheum.151437 [pii] AID - 10.3899/jrheum.151437 [doi] PST - ppublish SO - J Rheumatol. 2016 Sep;43(9):1665-71. doi: 10.3899/jrheum.151437. Epub 2016 Jun 15. PMID- 40386907 OWN - NLM STAT- MEDLINE DCOM- 20251029 LR - 20260306 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 77 IP - 11 DP - 2025 Nov TI - Hand Swelling and Other Non-Raynaud Phenomenon Symptoms as the Initial Presentation of Systemic Sclerosis: Prevalence and Clinical Associations in Two US Cohorts. PG - 1585-1595 LID - 10.1002/art.43237 [doi] AB - OBJECTIVE: Raynaud phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc), but some patients develop other manifestations first. To help elucidate the diversity of SSc presentation in its early stages, we describe the initial clinical manifestations and antinuclear antibody (ANA) profiles of patients in two early SSc cohorts. METHODS: All patient data in the Genetics vs Environment in Scleroderma Outcomes Study (GENISOS) and Collaborative National Quality and Efficacy Registry (CONQUER) cohorts were reviewed. Both studies enrolled patients within five years of the first non-RP symptom. RESULTS: In GENISOS and CONQUER, respectively, 194 (44.2%) of 439 and 292 (31.1%) of 938 patients had a non-RP initial symptom, most commonly puffy fingers/hands. Black patients had a non-RP symptom before RP more commonly than patients in other race and ethnicity categories. Non-RP first patients were more likely than RP first patients to have diffuse cutaneous involvement and joint contractures at enrollment and had a higher prevalence of RNA polymerase III antibody positivity. CONCLUSION: In two large US cohorts, >30% of patients began to manifest SSc with puffy fingers/hands or other symptoms, without the "warning sign" of RP as their initial symptom. These patients presented with more severe skin and musculoskeletal disease on average, highlighting the importance of early recognition. The most common autoantibody associated with this presentation was RNA polymerase III. These results should be considered in efforts to recognize SSc in its earliest stages. Puffy fingers/hands, even in the absence of RP, should prompt consideration of early SSc and testing for ANA and SSc-associated autoantibodies, including RNA polymerase III. CI - © 2025 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Hanif, Iqtidar AU - Hanif I AD - University of Texas Health Science Center at Houston. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - University of Texas Health Science Center at Houston. FAU - Mayes, Maureen D AU - Mayes MD AUID- ORCID: 0000-0001-5070-2535 AD - University of Texas Health Science Center at Houston. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AUID- ORCID: 0000-0001-6461-8940 AD - University of Texas Health Science Center at Houston. FAU - Zhang, Meng AU - Zhang M AD - University of Texas Health Science Center at Houston. FAU - Charles, Julio AU - Charles J AD - University of Texas Health Science Center at Houston. FAU - VanBuren, John M AU - VanBuren JM AD - University of Utah, Salt Lake City. FAU - Alvey, Jessica S AU - Alvey JS AD - University of Utah, Salt Lake City. FAU - Ghaffari, Kimia AU - Ghaffari K AD - University of Utah, Salt Lake City. FAU - Bernstein, Elana J AU - Bernstein EJ AD - Columbia University Irving Medical Center, New York City, New York. FAU - Castelino, Flavia V AU - Castelino FV AUID- ORCID: 0000-0001-9862-0299 AD - Massachusetts General Hospital, Boston. FAU - Chung, Lorinda AU - Chung L AD - Palo Alto VA Health Care System and Stanford University, Palo Alto, California. FAU - Evnin, Luke AU - Evnin L AD - Scleroderma Research Foundation, San Francisco, California. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Vanderbilt University Medical Center and Veterans Affairs Medical Center, VA Tennessee Valley Healthcare System, Nashville. FAU - Gordon, Jessica K AU - Gordon JK AUID- ORCID: 0000-0001-8068-0592 AD - Hospital for Special Surgery, New York City, New York. FAU - Hant, Faye N AU - Hant FN AD - Medical University of South Carolina, Charleston. FAU - Hummers, Laura K AU - Hummers LK AUID- ORCID: 0000-0002-4864-4011 AD - Johns Hopkins University, Baltimore, Maryland. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan, Ann Arbor. FAU - Lakin, Kimberly S AU - Lakin KS AD - Hospital for Special Surgery, New York City, New York. FAU - Lebiedz-Odrobina, Dorota AU - Lebiedz-Odrobina D AD - University of Utah, Salt Lake City. FAU - Luo, Yiming AU - Luo Y AD - Columbia University Irving Medical Center, New York City, New York. FAU - Makol, Ashima AU - Makol A AD - Mayo Clinic, Rochester, Minnesota. FAU - Molitor, Jerry A AU - Molitor JA AD - University of Minnesota, Minneapolis. FAU - Moore, Duncan F AU - Moore DF AD - Northwestern University, Chicago, Illinois. FAU - Richardson, Carrie AU - Richardson C AD - Northwestern University, Chicago, Illinois. FAU - Sandorfi, Nora AU - Sandorfi N AD - University of Pennsylvania, Philadelphia. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University, Baltimore, Maryland. FAU - Shah, Ankoor AU - Shah A AD - Duke University, Durham, North Carolina. FAU - Shanmugam, Victoria K AU - Shanmugam VK AUID- ORCID: 0000-0002-5882-4884 AD - The George Washington University, Washington, DC. FAU - Steen, Virginia D AU - Steen VD AD - Georgetown University Medical Center, Washington, DC. FAU - Volkmann, Elizabeth R AU - Volkmann ER AUID- ORCID: 0000-0003-3750-6569 AD - University of California, Los Angeles. FAU - Zahn, Carleigh AU - Zahn C AUID- ORCID: 0009-0005-9683-6973 AD - University of Michigan, Ann Arbor. FAU - Skaug, Brian AU - Skaug B AUID- ORCID: 0000-0002-6689-4655 AD - University of Texas Health Science Center at Houston. LA - eng GR - K24 AR080217/AR/NIAMS NIH HHS/United States GR - DOD-W81XWH-22-1-0163/U.S. Department of Defense/ GR - K08AR081402/AR/NIAMS NIH HHS/United States GR - UM1 TR004409/TR/NCATS NIH HHS/United States GR - Rheumatology Research Foundation/ GR - RO1HL164758/AR/NIAMS NIH HHS/United States GR - K23 AR075112/AR/NIAMS NIH HHS/United States GR - Scleroderma Research Foundation/ GR - R01AR081280/AR/NIAMS NIH HHS/United States GR - R01 HL164758/HL/NHLBI NIH HHS/United States GR - R01 AR081280/AR/NIAMS NIH HHS/United States GR - DOD-W81XWH-22-1-0162/U.S. Department of Defense/ GR - K23AR075112/AR/NIAMS NIH HHS/United States GR - K08 AR081402/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article DEP - 20250722 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antibodies, Antinuclear) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Antibodies, Antinuclear/blood/immunology MH - Black or African American/statistics & numerical data MH - Cohort Studies MH - Contracture/etiology MH - *Edema/etiology/epidemiology MH - *Hand/pathology MH - Prevalence MH - *Raynaud Disease/etiology/epidemiology/immunology MH - RNA Polymerase III/immunology MH - *Scleroderma, Systemic/complications/immunology/diagnosis/epidemiology MH - United States/epidemiology PMC - PMC12353935 MID - NIHMS2082976 EDAT- 2025/05/19 12:30 MHDA- 2025/10/29 12:29 CRDT- 2025/05/19 06:33 PHST- 2025/04/18 00:00 [revised] PHST- 2024/10/09 00:00 [received] PHST- 2025/04/30 00:00 [accepted] PHST- 2025/10/29 12:29 [medline] PHST- 2025/05/19 12:30 [pubmed] PHST- 2025/05/19 06:33 [entrez] AID - 10.1002/art.43237 [doi] PST - ppublish SO - Arthritis Rheumatol. 2025 Nov;77(11):1585-1595. doi: 10.1002/art.43237. Epub 2025 Jul 22. PMID- 29513930 OWN - NLM STAT- MEDLINE DCOM- 20190703 LR - 20190703 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 70 IP - 6 DP - 2018 Jun TI - Reply. PG - 974 LID - 10.1002/art.40480 [doi] FAU - Denton, Christopher P AU - Denton CP AD - Royal Free Hospital, London, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - University of Manchester Salford Royal NHS Foundation Trust, and NIHR Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Comment PT - Letter DEP - 20180502 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Acetamides) RN - 0 (Pyrazines) RN - 5EXC0E384L (selexipag) SB - IM CON - Arthritis Rheumatol. 2017 Dec;69(12):2370-2379. doi: 10.1002/art.40242. PMID: 29193819 CON - Arthritis Rheumatol. 2018 Jun;70(6):973-974. doi: 10.1002/art.40481. PMID: 29513932 MH - Acetamides MH - Adult MH - Humans MH - Pyrazines MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2018/03/08 06:00 MHDA- 2019/07/04 06:00 CRDT- 2018/03/08 06:00 PHST- 2018/03/08 06:00 [pubmed] PHST- 2019/07/04 06:00 [medline] PHST- 2018/03/08 06:00 [entrez] AID - 10.1002/art.40480 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Jun;70(6):974. doi: 10.1002/art.40480. Epub 2018 May 2. PMID- 33928623 OWN - NLM STAT- MEDLINE DCOM- 20210507 LR - 20211129 IS - 2284-0729 (Electronic) IS - 1128-3602 (Linking) VI - 25 IP - 8 DP - 2021 Apr TI - Cardiovascular, hematological and neurosensory impact of COVID-19 and variants. PG - 3350-3364 LID - 25747 [pii] LID - 10.26355/eurrev_202104_25747 [doi] AB - OBJECTIVE: The purpose of this article was to review our clinical experience with COVID-19 patients observed in the Cardiovascular Division of Pompidou Hospital (University of Paris, France) and the Department of Neurology of the Eastern Piedmont University (Novara, Italy), related to the impact on the cardiovascular, hematological, and neurologic systems and sense organs. PATIENTS AND METHODS: We sought to characterize cardiovascular, hematological, and neurosensory manifestations in patients with COVID-19 and variants. Special attention was given to initial signs and symptoms to facilitate early diagnosis and therapy. Indications of ECMO (extracorporeal membrane oxygenation) for cardiorespiratory support were evaluated. RESULTS: Preliminary neurosensorial symptoms, such as anosmia and dysgeusia, are useful for diagnosis, patient isolation, and treatment. Early angiohematological acro-ischemic syndrome includes hand and foot cyanosis, Raynaud digital ischemia phenomenon, skin bullae, and dry gangrene. This was associated with neoangiogenesis, vasculitis, and vessel thrombosis related to immune dysregulation, resulting from "cytokine storm syndrome". The most dangerous complication is disseminated intravascular coagulation, with mortality risks for both children and adults. CONCLUSIONS: COVID-19 is a prothrombotic disease with unique global lethality. A strong inflammatory response to viral infection severely affects cardiovascular and neurological systems, as well as respiratory, immune, and hematological systems. Rapid identification of acro-ischemic syndrome permits the treatment of disseminated intravascular coagulation complications. Early sensorial symptoms, such as gustatory and olfactory loss, are useful for COVID-19 diagnosis. New variants of SARS-CoV-2 are emerging, principally from United Kingdom, South Africa, and Brazil. These variants seem to spread more easily and quickly, which may lead to more cases of COVID. FAU - Chachques, J C AU - Chachques JC AD - Cardiovascular Division, Pompidou Hospital, University of Paris, Paris, France. j.chachques@aphp.fr. FAU - Mazzini, L AU - Mazzini L FAU - Mitrecic, D AU - Mitrecic D FAU - Zavan, B AU - Zavan B FAU - Rogante, M AU - Rogante M FAU - Latremouille, C AU - Latremouille C FAU - Rustichelli, F AU - Rustichelli F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Eur Rev Med Pharmacol Sci JT - European review for medical and pharmacological sciences JID - 9717360 RN - 0 (Spike Glycoprotein, Coronavirus) RN - 0 (spike protein, SARS-CoV-2) RN - EC 3.4.22.28 (Coronavirus 3C Proteases) SB - IM EIN - Eur Rev Med Pharmacol Sci. 2021 Jul;25(13):4440. doi: 10.26355/eurrev_202107_26226. PMID: 34286481 MH - Anosmia/*physiopathology MH - COVID-19/pathology/*physiopathology/therapy/virology MH - Coronavirus 3C Proteases/ultrastructure MH - Cyanosis/*physiopathology MH - Cytokine Release Syndrome MH - Disseminated Intravascular Coagulation/pathology/*physiopathology MH - Dysgeusia/*physiopathology MH - Extracorporeal Membrane Oxygenation MH - Foot/blood supply MH - France MH - Gangrene/pathology/physiopathology MH - Hand/blood supply MH - Humans MH - Ischemia/pathology/physiopathology MH - Myocarditis/*physiopathology MH - Noninvasive Ventilation MH - Plasma Exchange MH - Raynaud Disease/pathology/*physiopathology MH - SARS-CoV-2 MH - Spike Glycoprotein, Coronavirus/ultrastructure MH - Synchrotrons MH - Vasculitis/pathology/*physiopathology EDAT- 2021/05/01 06:00 MHDA- 2021/05/08 06:00 CRDT- 2021/04/30 07:09 PHST- 2021/04/30 07:09 [entrez] PHST- 2021/05/01 06:00 [pubmed] PHST- 2021/05/08 06:00 [medline] AID - 25747 [pii] AID - 10.26355/eurrev_202104_25747 [doi] PST - ppublish SO - Eur Rev Med Pharmacol Sci. 2021 Apr;25(8):3350-3364. doi: 10.26355/eurrev_202104_25747. PMID- 38000832 OWN - NLM STAT- MEDLINE DCOM- 20231127 LR - 20250328 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 44 IP - 12 DP - 2023 Dec TI - [White and blue fingers]. PG - 679-681 LID - S0248-8663(23)00737-3 [pii] LID - 10.1016/j.revmed.2023.09.005 [doi] FAU - Bertolino, J AU - Bertolino J AD - Service de médecine vasculaire, hôpital de la Timone, Assistance publique-Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), Marseille, France. FAU - Leclercq, B AU - Leclercq B AD - Service de médecine vasculaire, hôpital de la Timone, Assistance publique-Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), Marseille, France. FAU - Bartoli, M AU - Bartoli M AD - Service de chirurgie vasculaire, hôpital de la Timone, Assistance publique-Hôpitaux de Marseille (AP-HM), Aix-Marseille Université (AMU), Marseille, France. FAU - Jacquier, A AU - Jacquier A AD - Service de radiologie, hôpital de la Timone, Assistance publique-Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), Marseille, France. FAU - Benyamine, A AU - Benyamine A AD - Service de médecine interne, Hôpital Nord, Assistance Publique Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), chemin des Bourrely, 1305 Marseille, France. FAU - Sarlon, G AU - Sarlon G AD - Service de médecine vasculaire, hôpital de la Timone, Assistance publique-Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), Marseille, France. FAU - Granel, B AU - Granel B AD - Service de médecine interne, Hôpital Nord, Assistance Publique Hôpitaux de Marseille (AP-HM), Aix-Marseille université (AMU), chemin des Bourrely, 1305 Marseille, France. Electronic address: bgranel@ap-hm.fr. LA - fre PT - Letter TT - Des doigts blancs et bleus. DEP - 20231122 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Humans MH - *Fingers MH - *Raynaud Disease EDAT- 2023/11/25 07:42 MHDA- 2023/11/27 12:43 CRDT- 2023/11/24 20:55 PHST- 2023/07/12 00:00 [received] PHST- 2023/09/25 00:00 [accepted] PHST- 2023/11/27 12:43 [medline] PHST- 2023/11/25 07:42 [pubmed] PHST- 2023/11/24 20:55 [entrez] AID - S0248-8663(23)00737-3 [pii] AID - 10.1016/j.revmed.2023.09.005 [doi] PST - ppublish SO - Rev Med Interne. 2023 Dec;44(12):679-681. doi: 10.1016/j.revmed.2023.09.005. Epub 2023 Nov 22. PMID- 20567796 OWN - NLM STAT- MEDLINE DCOM- 20110201 LR - 20220310 IS - 1795-990X (Electronic) IS - 0355-3140 (Linking) VI - 36 IP - 6 DP - 2010 Nov TI - White fingers, cold environment, and vibration--exposure among Swedish construction workers. PG - 509-13 AB - OBJECTIVES: The aim of this study was to examine the association between white fingers, cold environment, and exposure to hand-arm vibration (HAV). The hypothesis was that working in cold climate increases the risk of white fingers. METHODS: The occurrence of white fingers was investigated as a cross-sectional study in a cohort of Swedish male construction workers (N=134 757). Exposure to HAV was based on a job-exposure matrix. Living in the north or south of Sweden was, in a subgroup of the cohort, used as an indicator of the exposure to cold environment (ie, living in the north meant a higher exposure to cold climate). The analyses were adjusted for age and use of nicotine products (smoking and snuff). RESULTS: HAV-exposed workers living in a colder climate had a higher risk for white fingers than those living in a warmer climate [odds ratio (OR) 1.71, 95% confidence interval (95% CI) 1.42-2.06]. As expected, we found that HAV-exposed workers had an increased risk compared to controls (OR 2.02, 95% CI 1.75-2.34). The risk for white fingers increased with increased level of exposure to HAV and also age. CONCLUSIONS: Cold environment increases the risk for white fingers in workers occupationally exposed to HAV. The results underscore the need to keep exposure to HAV at workplaces as low as possible especially in cold climate. FAU - Burström, Lage AU - Burström L AD - Department of Public Health & Clinical Medicine, Occupational and Environmental Medicine, Umeå University,Umeå, Sweden. lage.burstrom@envmed.umu.se FAU - Järvholm, Bengt AU - Järvholm B FAU - Nilsson, Tohr AU - Nilsson T FAU - Wahlström, Jens AU - Wahlström J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100622 PL - Finland TA - Scand J Work Environ Health JT - Scandinavian journal of work, environment & health JID - 7511540 SB - IM MH - Adult MH - Aged MH - *Cold Temperature MH - Cross-Sectional Studies MH - Facility Design and Construction MH - Fingers/blood supply/*physiopathology MH - Hand-Arm Vibration Syndrome/complications/*epidemiology MH - Humans MH - Male MH - Middle Aged MH - Occupations MH - Raynaud Disease/epidemiology/*etiology MH - Risk Factors MH - Sweden/epidemiology MH - *Vibration EDAT- 2010/06/23 06:00 MHDA- 2011/02/02 06:00 CRDT- 2010/06/23 06:00 PHST- 2010/06/23 06:00 [entrez] PHST- 2010/06/23 06:00 [pubmed] PHST- 2011/02/02 06:00 [medline] AID - 3072 [pii] AID - 10.5271/sjweh.3072 [doi] PST - ppublish SO - Scand J Work Environ Health. 2010 Nov;36(6):509-13. doi: 10.5271/sjweh.3072. Epub 2010 Jun 22. PMID- 18078627 OWN - NLM STAT- MEDLINE DCOM- 20080410 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 25 IP - 5 DP - 2007 Sep-Oct TI - Improving the sensitivity of the American College of Rheumatology classification criteria for systemic sclerosis. PG - 754-7 AB - OBJECTIVE: A large proportion of patients with limited systemic sclerosis (SSc) do not meet the current American College of Rheumatology (ACR) classification criteria for SSc. We undertook this study to determine whether the addition of easily available clinical variables, namely nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias, could improve the sensitivity of the current ACR classification criteria for patients with limited SSc. METHODS: Patients in the Canadian Scleroderma Research Group Registry with skin involvement distal to the metacarpophalangeal joints were identified and divided into two groups according to whether they fulfilled the current ACR classification criteria for SSc or not. Sensitivity of the criteria was calculated. Regression tree analysis was performed to determine whether the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias could improve the sensitivity of the criteria. RESULTS: One hundred and one (101) patients were included, in majority women with a mean age of 59 (+/- 13). Of these, 68 (67%) met the ACR classification criteria. The sensitivity of the criteria increased from 67% to 99% with the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias. CONCLUSIONS: The SSc research community would benefit from having better classification criteria to identify patients with limited SSc. The current classification criteria for SSc may be significantly improved by the inclusion of easily identified clinical variables including nailfold capillary abnormalities using a dermatoscope. FAU - Hudson, M AU - Hudson M AD - McGill University, Montreal, Quebec, Canada. marie.hudson@mcgill.ca FAU - Taillefer, S AU - Taillefer S FAU - Steele, R AU - Steele R FAU - Dunne, J AU - Dunne J FAU - Johnson, S R AU - Johnson SR FAU - Jones, N AU - Jones N FAU - Mathieu, J-P AU - Mathieu JP FAU - Baron, M AU - Baron M LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM CIN - Clin Exp Rheumatol. 2007 Sep-Oct;25(5):663-5. PMID: 18078610 MH - Aged MH - Capillaries/pathology MH - Dermoscopy MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Nails/*blood supply/pathology MH - Raynaud Disease/diagnosis/pathology MH - Scleroderma, Systemic/*classification/*diagnosis/pathology MH - Sensitivity and Specificity MH - Societies, Medical MH - Telangiectasis/diagnosis/pathology MH - United States EDAT- 2007/12/15 09:00 MHDA- 2008/04/11 09:00 CRDT- 2007/12/15 09:00 PHST- 2007/12/15 09:00 [pubmed] PHST- 2008/04/11 09:00 [medline] PHST- 2007/12/15 09:00 [entrez] AID - 2191 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2007 Sep-Oct;25(5):754-7. PMID- 18431096 OWN - NLM STAT- MEDLINE DCOM- 20080603 LR - 20131121 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 14 IP - 1 DP - 2008 Feb TI - Methylphenidate and dextroamphetamine-induced peripheral vasculopathy. PG - 30-3 LID - 10.1097/RHU.0b013e3181639aaa [doi] AB - Methylphenidate and dextroamphetamine are central nervous system stimulants used in the treatment of attention deficit hyperactivity disorders in children. These medications have been associated with cerebral arteritis, renal necrotizing vasculitis, and systemic and pulmonary hypertension. We report 4 patients, 2 on methylphenidate and 2 on dextroamphetamine who presented with acral cyanosis, livedo reticularis, or Raynaud phenomenon. Two patients were found to have a positive ANA at low titers, 1 of whom had histopathologic evidence of stratum malgiphian necrosis with perivascular lymphocytic infiltration on skin biopsy. Two had positive antihistone antibodies. One patient improved after withdrawal of dextroamphetamine; others had worsening of their symptoms on higher doses of medications. These cases indicate the potential for development of acral cyanosis, livedo reticularis, or Raynaud symptoms with these medications and their potential contribution to a vasculopathy. FAU - Syed, Reema H AU - Syed RH AD - Division of Rheumatology, Saint Louis University Medical Center, Saint Louis, Missouri 63104, USA. FAU - Moore, Terry L AU - Moore TL LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) RN - TZ47U051FI (Dextroamphetamine) SB - IM MH - Adult MH - Attention Deficit Disorder with Hyperactivity/drug therapy MH - Central Nervous System Stimulants/*adverse effects MH - Child MH - Dextroamphetamine/*adverse effects MH - Female MH - Humans MH - Livedo Reticularis/*chemically induced MH - Methylphenidate/*adverse effects MH - Peripheral Vascular Diseases/*chemically induced MH - Raynaud Disease/*chemically induced EDAT- 2008/04/24 09:00 MHDA- 2008/06/05 09:00 CRDT- 2008/04/24 09:00 PHST- 2008/04/24 09:00 [pubmed] PHST- 2008/06/05 09:00 [medline] PHST- 2008/04/24 09:00 [entrez] AID - 00124743-200802000-00008 [pii] AID - 10.1097/RHU.0b013e3181639aaa [doi] PST - ppublish SO - J Clin Rheumatol. 2008 Feb;14(1):30-3. doi: 10.1097/RHU.0b013e3181639aaa. PMID- 30028256 OWN - NLM STAT- MEDLINE DCOM- 20181231 LR - 20220409 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 27 IP - 11 DP - 2018 Oct TI - Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus in a patient with progressive systemic sclerosis. PG - 1860-1863 LID - 10.1177/0961203318789766 [doi] AB - A 60-year-old female was diagnosed with progressive systemic sclerosis and interstitial lung disease of two months' duration. The patient was treated for Raynaud phenomenon with aspirin, nifedipine, colchicine, and naproxen. Two weeks after treatment, she developed widespread erythematous patches with blistering eruptions on the face, torso, and extremities, and also had erosion on the oral mucosa. Skin biopsy for histopathology and direct immunofluorescent studies were suggestive of lupus erythematosus. To the best of our knowledge, this is the first case of toxic epidermal necrolysis-like acute cutaneous lupus erythematosus in a patient with progressive systemic sclerosis. FAU - Aiempanakit, K AU - Aiempanakit K AUID- ORCID: 0000-0001-5256-827X AD - 1 Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, Thailand. FAU - Chiratikarnwong, K AU - Chiratikarnwong K AD - 1 Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, Thailand. FAU - Juthong, S AU - Juthong S AD - 2 Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, Thailand. FAU - Auepemkiate, S AU - Auepemkiate S AD - 3 Department of Pathology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, Thailand. LA - eng PT - Case Reports PT - Journal Article DEP - 20180720 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Diagnosis, Differential MH - Female MH - Humans MH - Lupus Erythematosus, Cutaneous/*diagnosis/*pathology MH - Middle Aged MH - Raynaud Disease/pathology MH - Scleroderma, Systemic/*complications MH - Skin/*pathology MH - Stevens-Johnson Syndrome/pathology OTO - NOTNLM OT - Cutaneous lupus erythematosus OT - overlap syndrome OT - systemic lupus erythematosus OT - systemic sclerosis OT - toxic epidermal necrolysis EDAT- 2018/07/22 06:00 MHDA- 2019/01/01 06:00 CRDT- 2018/07/21 06:00 PHST- 2018/07/22 06:00 [pubmed] PHST- 2019/01/01 06:00 [medline] PHST- 2018/07/21 06:00 [entrez] AID - 10.1177/0961203318789766 [doi] PST - ppublish SO - Lupus. 2018 Oct;27(11):1860-1863. doi: 10.1177/0961203318789766. Epub 2018 Jul 20. PMID- 26015278 OWN - NLM STAT- MEDLINE DCOM- 20151013 LR - 20150727 IS - 1523-6838 (Electronic) IS - 0272-6386 (Linking) VI - 66 IP - 2 DP - 2015 Aug TI - Distal Angiopathy and Atypical Hemolytic Uremic Syndrome: Clinical and Functional Properties of an Anti-Factor H IgAλ Antibody. PG - 331-6 LID - S0272-6386(15)00706-4 [pii] LID - 10.1053/j.ajkd.2015.03.039 [doi] AB - Abnormal regulation of the alternative pathway of the complement system is a well-described trigger of microangiopathy leading to atypical hemolytic uremic syndrome (aHUS). However, the involvement of complement dysregulation in distal angiopathy has not been reported in adults. We describe the clinical course of a patient with severe distal angiopathy (amputation of all fingers and toes) followed 3 years later by aHUS with end-stage renal disease. This course was attributed to a circulating monoclonal immunoglobulin A λ light chain (IgAλ) with unusual properties: it bound complement factor H (CFH) and impaired CFH-glycosaminoglycan interaction and cell-surface protection. Local complement activation with distal angiopathy and microvascular injury was suggested by deposition of IgA, C4d, and C5b-9 in limb and preglomerular arteries. We therefore postulated that the monoclonal IgAλ inhibited activity of endothelial cell-bound CFH, which led to local activation of complement, vasoconstriction (distal angiopathy), and aHUS. While the patient was dependent on dialysis and plasma exchange, treatment with the anti-C5 antibody eculizumab induced remission of distal angiopathy and aHUS. During eculizumab treatment, kidney transplantation was performed. The patient had normal kidney function at the 3-year follow-up. We suggest that the association of distal angiopathy and aHUS in this patient is clearly linked to anti-CFH properties of the monoclonal IgAλ. CI - Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved. FAU - Rigothier, Claire AU - Rigothier C AD - Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France; INSERM U1026, Université de Bordeaux, Bordeaux, France. Electronic address: claire.rigothier@chu-bordeaux.fr. FAU - Delmas, Yahsou AU - Delmas Y AD - Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. FAU - Roumenina, Lubka T AU - Roumenina LT AD - Complement and Diseases Team, INSERM UMRS 1138, Cordeliers Research Center, Paris, France; Université Paris Descartes Sorbonne Paris-Cité, Paris, France; Université Pierre et Marie Curie (UPMC-Paris-6), Paris, France. FAU - Contin-Bordes, Cécile AU - Contin-Bordes C AD - Laboratoire d'Immunologie, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. FAU - Lepreux, Sébastien AU - Lepreux S AD - INSERM U1026, Université de Bordeaux, Bordeaux, France; Laboratoire de Pathologie, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. FAU - Bridoux, Frank AU - Bridoux F AD - Service de Néphrologie, Hémodialyse et Transplantation Rénale, Centre Hospitalier Universitaire de Poitiers, Poitiers, France; Centre de référence des amyloses primitives et des maladies de dépôts d'immunoglobulines monoclonales, Poitiers, France. FAU - Goujon, Jean Michel AU - Goujon JM AD - Centre de référence des amyloses primitives et des maladies de dépôts d'immunoglobulines monoclonales, Poitiers, France; Laboratoire d'Anatomie et cytologie Pathologiques, Centre Hospitalier Universitaire de Poitiers, Poitiers, France. FAU - Bachelet, Thomas AU - Bachelet T AD - Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. FAU - Touchard, Guy AU - Touchard G AD - Service de Néphrologie, Hémodialyse et Transplantation Rénale, Centre Hospitalier Universitaire de Poitiers, Poitiers, France; Centre de référence des amyloses primitives et des maladies de dépôts d'immunoglobulines monoclonales, Poitiers, France. FAU - Frémeaux-Bacchi, Véronique AU - Frémeaux-Bacchi V AD - Complement and Diseases Team, INSERM UMRS 1138, Cordeliers Research Center, Paris, France; Assistance Publique-Hopitaux de Paris, Service d'Immunologie biologique, Hôpital Européen Georges Pompidou, Paris, France. FAU - Combe, Christian AU - Combe C AD - Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France; INSERM U1026, Université de Bordeaux, Bordeaux, France. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150523 PL - United States TA - Am J Kidney Dis JT - American journal of kidney diseases : the official journal of the National Kidney Foundation JID - 8110075 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Immunoglobulin A) RN - 0 (Immunoglobulin Light Chains) RN - 80295-65-4 (Complement Factor H) RN - A3ULP0F556 (eculizumab) SB - IM MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Atypical Hemolytic Uremic Syndrome/*etiology/immunology/therapy MH - Complement Factor H/*immunology MH - Humans MH - Immunoglobulin A/*immunology MH - Immunoglobulin Light Chains/*immunology MH - Kidney Failure, Chronic/*etiology/immunology/therapy MH - Male MH - Middle Aged MH - Paraproteinemias/*complications/immunology/therapy MH - Plasma Exchange MH - Raynaud Disease/*etiology/immunology/therapy MH - Thrombotic Microangiopathies/etiology/immunology/therapy OTO - NOTNLM OT - Gammopathy OT - alternative complement pathway OT - atypical hemolytic uremic syndrome (aHUS) OT - autoantibody OT - complement activation OT - complement factor H (CFH) OT - distal angiopathy OT - factor H antibody OT - macroangiopathy OT - microvascular injury EDAT- 2015/05/28 06:00 MHDA- 2015/10/16 06:00 CRDT- 2015/05/28 06:00 PHST- 2014/11/27 00:00 [received] PHST- 2015/03/11 00:00 [accepted] PHST- 2015/05/28 06:00 [entrez] PHST- 2015/05/28 06:00 [pubmed] PHST- 2015/10/16 06:00 [medline] AID - S0272-6386(15)00706-4 [pii] AID - 10.1053/j.ajkd.2015.03.039 [doi] PST - ppublish SO - Am J Kidney Dis. 2015 Aug;66(2):331-6. doi: 10.1053/j.ajkd.2015.03.039. Epub 2015 May 23. PMID- 24085811 OWN - NLM STAT- MEDLINE DCOM- 20140604 LR - 20131002 IS - 1939-2869 (Electronic) IS - 0891-1150 (Linking) VI - 80 IP - 10 DP - 2013 Oct TI - Antisynthetase syndrome: not just an inflammatory myopathy. PG - 655-66 LID - 10.3949/ccjm.80a.12171 [doi] AB - In recent years, antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibodies to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, "mechanic's hands," Raynaud phenomenon, and polyarthritis. Physicians should be familiar with its variety of clinical presentations and should include it in the differential diagnosis in patients presenting with unexplained interstitial lung disease. FAU - Chatterjee, Soumya AU - Chatterjee S AD - Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH; Staff, Department of Rheumatic and Immunologic Diseases, Orthopedics and Rheumatology, Institute, Cleveland Clinic. FAU - Prayson, Richard AU - Prayson R FAU - Farver, Carol AU - Farver C LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - Cleve Clin J Med JT - Cleveland Clinic journal of medicine JID - 8703441 RN - 0 (Antibodies, Antinuclear) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Aged MH - Antibodies, Antinuclear/blood MH - Arthritis/etiology MH - Fatal Outcome MH - Glucocorticoids/*therapeutic use MH - Hand Dermatoses/etiology MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/*diagnosis/*drug therapy/etiology MH - Male MH - Myositis/blood/complications/*diagnosis/*drug therapy MH - Raynaud Disease/etiology EDAT- 2013/10/03 06:00 MHDA- 2014/06/05 06:00 CRDT- 2013/10/03 06:00 PHST- 2013/10/03 06:00 [entrez] PHST- 2013/10/03 06:00 [pubmed] PHST- 2014/06/05 06:00 [medline] AID - 80/10/655 [pii] AID - 10.3949/ccjm.80a.12171 [doi] PST - ppublish SO - Cleve Clin J Med. 2013 Oct;80(10):655-66. doi: 10.3949/ccjm.80a.12171. PMID- 29470737 OWN - NLM STAT- MEDLINE DCOM- 20180918 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 5 DP - 2018 May TI - The safety of iloprost in systemic sclerosis in a real-life experience. PG - 1249-1255 LID - 10.1007/s10067-018-4043-0 [doi] AB - Iloprost (ILO) is employed intravenously for the treatment of severe Raynaud phenomenon (RP) and digital ulcers (DU) in systemic sclerosis (SSc). The aim of this study was to evaluate the safety and tolerability of the intravenous treatment with ILO in different phases of SSc. Eighty-one consecutive non-selected SSc patients, all on nifedipine, with moderate RP, treated with ILO infusion, were retrospectively evaluated. Patients were sub classified according to the edematous or fibrotic/atrophic cutaneous phase of the disease. ILO was infused with a progressive increase of the dosage up to the achievement of patient's tolerance, 1 day/week. In cases of slower infusion regimen due to adverse events (AE) at the beginning of the administration, patients received a lower dose of the drug (not possible to quantify precisely the final cumulative dosage). 16/81 SSc patients presented digital edema, 5 developed diarrhea, and 9 developed transient hypotension during the infusion at 20 ml/h that ameliorated when the drug was withdrawn. Moreover, 10/16 edematous patients experienced significant and painful digital swelling, unlike patients in the fibrotic group (p < 0.0001); 11/16 patients reported flushing and 7/16 headache, always controlled with dose tapering below 10 ml/h. In the atrophic/fibrotic phase patients (65/81), 10 developed diarrhea and 24 hypotension at infusion rate of 20 ml/h that led to temporary withdrawal of the drug. When ILO was restarted and kept below 10 ml/h, no side effects were experienced. 23/65 patients experienced flushing and 8/65 headache, all controlled with infusion reduction below 10 ml/h. In these patients, adverse events were significantly less frequent than in the edematous group (p = 0.023 and p = 0.008, respectively). Our data suggest that calcium channel blockers should be transitorily stopped while using ILO and that a pre-treatment approach might reduce or control adverse events. In patients with digital edema, ILO infusion should be carefully employed after the evaluation of patient's drug tolerance. FAU - Bellando-Randone, S AU - Bellando-Randone S AUID- ORCID: 0000-0002-5926-6263 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. s.bellandorandone@gmail.com. FAU - Bruni, C AU - Bruni C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Lepri, G AU - Lepri G AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Fiori, G AU - Fiori G AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Bartoli, F AU - Bartoli F AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Conforti, M L AU - Conforti ML AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Moggi-Pignone, A AU - Moggi-Pignone A AD - Internal Medicine Unit 3, Careggi University Hospital Florence, Florence, Italy. FAU - Guiducci, S AU - Guiducci S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. FAU - Giuggioli, D AU - Giuggioli D AD - Rheumatology Unit, Scleroderma Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Colaci, M AU - Colaci M AD - Rheumatology Unit, Scleroderma Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Spinella, A AU - Spinella A AD - Rheumatology Unit, Scleroderma Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Ferri, C AU - Ferri C AD - Rheumatology Unit, Scleroderma Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence and Division of Rheumatology AOUC, Florence, Italy. LA - eng PT - Journal Article DEP - 20180222 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Diarrhea/chemically induced MH - Female MH - Fingers MH - Humans MH - Iloprost/*adverse effects/therapeutic use MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Raynaud Disease/*drug therapy/etiology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology MH - Treatment Outcome OTO - NOTNLM OT - Digital edema OT - Iloprost OT - Safety OT - Systemic sclerosis EDAT- 2018/02/23 06:00 MHDA- 2018/09/19 06:00 CRDT- 2018/02/23 06:00 PHST- 2017/11/04 00:00 [received] PHST- 2018/02/14 00:00 [accepted] PHST- 2018/02/01 00:00 [revised] PHST- 2018/02/23 06:00 [pubmed] PHST- 2018/09/19 06:00 [medline] PHST- 2018/02/23 06:00 [entrez] AID - 10.1007/s10067-018-4043-0 [pii] AID - 10.1007/s10067-018-4043-0 [doi] PST - ppublish SO - Clin Rheumatol. 2018 May;37(5):1249-1255. doi: 10.1007/s10067-018-4043-0. Epub 2018 Feb 22. PMID- 24720999 OWN - NLM STAT- MEDLINE DCOM- 20150709 LR - 20161125 IS - 2214-8116 (Electronic) IS - 0398-0499 (Linking) VI - 39 IP - 3 DP - 2014 May TI - [Hypothenar hammer syndrome: case report and review of the literature]. PG - 220-3 LID - S0398-0499(14)00054-7 [pii] LID - 10.1016/j.jmv.2014.03.004 [doi] AB - INTRODUCTION: Hypothenar hammer syndrome is a rare cause of upper extremity digital ischemia or Raynaud phenomenon, a diagnosis which should be considered in cases of iterative palmar trauma. Its treatment can be medical or surgical and should not suffer any delay. The best options remain controversial. METHODS: A 65-year-old patient presented with an ischemia of the last three fingers of the left hand. A partially thrombosed aneurysm of the left ulnar artery was diagnosed at imaging. After a partially effective medical treatment, a surgical treatment was performed with resection of the aneurysm and a vascular reconstruction with an autologous vein graft. The postoperative course was uneventful with disappearance of the symptoms and revascularization of the hypothenar area. CONCLUSION: Appropriate treatment for hypothenar hammer syndrome is controversial but whould always begin with medical care. The decision to perform surgery should be based on evidence of ulnar artery lesions and the associated symptoms. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Malgras, B AU - Malgras B AD - Service de chirurgie viscérale et vasculaire, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. Electronic address: bricemalgras@hotmail.com. FAU - Mlynski, A AU - Mlynski A AD - Service de chirurgie viscérale et vasculaire, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. FAU - Pierret, C AU - Pierret C AD - Service de chirurgie viscérale et vasculaire, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. FAU - Fossat, S AU - Fossat S AD - Service de chirurgie viscérale et vasculaire, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. FAU - de Kérangal, X AU - de Kérangal X AD - Service de chirurgie viscérale et vasculaire, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. LA - fre PT - Case Reports PT - Journal Article PT - Review TT - Syndrome du marteau hypothénarien : cas clinique et revue de la littérature. DEP - 20140408 PL - France TA - J Mal Vasc JT - Journal des maladies vasculaires JID - 7707965 SB - IM MH - Aged MH - Aneurysm/*complications/diagnostic imaging/surgery MH - Blood Vessel Prosthesis Implantation MH - Hand/*blood supply/surgery MH - Humans MH - Ischemia/*diagnosis/etiology MH - Male MH - Plaque, Atherosclerotic/complications MH - Raynaud Disease/*complications MH - Thrombectomy MH - Thrombosis/*etiology/surgery MH - Ulnar Artery/diagnostic imaging/*pathology/surgery MH - Ultrasonography OTO - NOTNLM OT - Digital ischemia OT - Hypothenar hammer OT - Ischémie digitale OT - Marteau hypothénarien OT - Phénomène de Raynaud OT - Raynaud phenomenon EDAT- 2014/04/12 06:00 MHDA- 2015/07/15 06:00 CRDT- 2014/04/12 06:00 PHST- 2013/09/20 00:00 [received] PHST- 2014/02/28 00:00 [accepted] PHST- 2014/04/12 06:00 [entrez] PHST- 2014/04/12 06:00 [pubmed] PHST- 2015/07/15 06:00 [medline] AID - S0398-0499(14)00054-7 [pii] AID - 10.1016/j.jmv.2014.03.004 [doi] PST - ppublish SO - J Mal Vasc. 2014 May;39(3):220-3. doi: 10.1016/j.jmv.2014.03.004. Epub 2014 Apr 8. PMID- 35429067 OWN - NLM STAT- MEDLINE DCOM- 20220719 LR - 20221015 IS - 1468-3083 (Electronic) IS - 0926-9959 (Print) IS - 0926-9959 (Linking) VI - 36 IP - 8 DP - 2022 Aug TI - A case of acrocyanosis in a painting of the 17(th) century. PG - 1169-1170 LID - 10.1111/jdv.18120 [doi] FAU - Kluger, Nicolas AU - Kluger N AUID- ORCID: 0000-0002-5225-8316 AD - Department of Dermatology, Allergology and Venereology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. AD - Société Française Des Sciences Humaines Sur la Peau (SFSHP), Société Française de Dermatologie, Paris, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20220415 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 SB - IM MH - *Cyanosis MH - Humans MH - *Raynaud Disease PMC - PMC9540773 COIS- None. EDAT- 2022/04/17 06:00 MHDA- 2022/07/20 06:00 PMCR- 2022/10/07 CRDT- 2022/04/16 05:29 PHST- 2022/02/27 00:00 [revised] PHST- 2022/01/07 00:00 [received] PHST- 2022/03/25 00:00 [accepted] PHST- 2022/04/17 06:00 [pubmed] PHST- 2022/07/20 06:00 [medline] PHST- 2022/04/16 05:29 [entrez] PHST- 2022/10/07 00:00 [pmc-release] AID - JDV18120 [pii] AID - 10.1111/jdv.18120 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2022 Aug;36(8):1169-1170. doi: 10.1111/jdv.18120. Epub 2022 Apr 15. PMID- 27185438 OWN - NLM STAT- MEDLINE DCOM- 20170413 LR - 20170817 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 74 IP - 6 DP - 2016 Jun TI - Characterization of patients with clinical overlap of morphea and systemic sclerosis: A case series. PG - 1272-4 LID - S0190-9622(16)00006-2 [pii] LID - 10.1016/j.jaad.2015.12.051 [doi] FAU - Chen, Jennifer K AU - Chen JK AD - Department of Dermatology, Stanford University, Stanford, CA. Electronic address: jenniferkchen@stanford.edu. FAU - Chung, Lorinda AU - Chung L AD - Department of Dermatology, Stanford University, Stanford, CA; Division of Immunology and Rheumatology, Stanford University, Stanford, CA; Palo Alto VA Health Care System, Palo Alto, CA. FAU - Fiorentino, David F AU - Fiorentino DF AD - Department of Dermatology, Stanford University, Stanford, CA. LA - eng PT - Case Reports PT - Letter PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Adult MH - Aged, 80 and over MH - Female MH - Humans MH - Male MH - Middle Aged MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Scleroderma, Localized/*complications/pathology/physiopathology MH - Scleroderma, Systemic/*complications/pathology/physiopathology EDAT- 2016/05/18 06:00 MHDA- 2017/04/14 06:00 CRDT- 2016/05/18 06:00 PHST- 2015/09/18 00:00 [received] PHST- 2015/12/09 00:00 [revised] PHST- 2015/12/31 00:00 [accepted] PHST- 2016/05/18 06:00 [entrez] PHST- 2016/05/18 06:00 [pubmed] PHST- 2017/04/14 06:00 [medline] AID - S0190-9622(16)00006-2 [pii] AID - 10.1016/j.jaad.2015.12.051 [doi] PST - ppublish SO - J Am Acad Dermatol. 2016 Jun;74(6):1272-4. doi: 10.1016/j.jaad.2015.12.051. PMID- 18783099 OWN - NLM STAT- MEDLINE DCOM- 20080930 LR - 20080911 IS - 1750-8460 (Print) IS - 1750-8460 (Linking) VI - 69 IP - 8 DP - 2008 Aug TI - Systemic sclerosis: an update for clinicians. PG - 464-70 AB - Recent advances in the management of systemic sclerosis highlight the importance of early diagnosis and assessment, before irreversible tissue injury has occurred. This review will discuss diagnosis, subtyping, and the major clinical features and their management. FAU - Herrick, Ariane L AU - Herrick AL AD - Rheumatic Diseases Centre, Clinical Sciences Building, University of Manchester, Salford Royal NHS Foundation Trust, Salford M6 8HD. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Singapore TA - Br J Hosp Med (Lond) JT - British journal of hospital medicine (London, England : 2005) JID - 101257109 SB - IM MH - Adult MH - Early Diagnosis MH - Female MH - Fibrosis MH - Fingers/*blood supply/pathology MH - Humans MH - Ischemia/*etiology/therapy MH - Male MH - Raynaud Disease/*complications MH - Scleroderma, Systemic/complications/*diagnosis RF - 30 EDAT- 2008/09/12 09:00 MHDA- 2008/10/01 09:00 CRDT- 2008/09/12 09:00 PHST- 2008/09/12 09:00 [pubmed] PHST- 2008/10/01 09:00 [medline] PHST- 2008/09/12 09:00 [entrez] AID - 10.12968/hmed.2008.69.8.30744 [doi] PST - ppublish SO - Br J Hosp Med (Lond). 2008 Aug;69(8):464-70. doi: 10.12968/hmed.2008.69.8.30744. PMID- 17977626 OWN - NLM STAT- MEDLINE DCOM- 20080411 LR - 20161018 IS - 0248-8663 (Print) IS - 0248-8663 (Linking) VI - 28 Suppl 4 DP - 2007 Dec TI - [Treatment of systemic sclerosis (except pulmonary arterial hypertension)]. PG - S277-84 FAU - Hachulla, E AU - Hachulla E AD - Service de médecine interne A, hôpital Claude-Huriez, CHRU de Lille, université de Lille-2, avenue Oscar-Lambret, 59037 Lille cedex, France. ehachulla@chru-lille.fr LA - fre PT - Journal Article PT - Review TT - Traitement de la sclérodermie systémique (en dehors de l'hypertension artérielle pulmonaire). DEP - 20071011 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Immunologic Factors) RN - 0 (Immunosuppressive Agents) SB - IM MH - Acute Kidney Injury/drug therapy MH - Gastrointestinal Diseases/drug therapy MH - Humans MH - Immunologic Factors/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Raynaud Disease/drug therapy MH - Scleroderma, Systemic/*drug therapy MH - Skin Ulcer/drug therapy RF - 57 EDAT- 2007/11/06 09:00 MHDA- 2008/04/12 09:00 CRDT- 2007/11/06 09:00 PHST- 2007/11/06 09:00 [pubmed] PHST- 2008/04/12 09:00 [medline] PHST- 2007/11/06 09:00 [entrez] AID - S0248-8663(07)00798-9 [pii] AID - 10.1016/j.revmed.2007.09.003 [doi] PST - ppublish SO - Rev Med Interne. 2007 Dec;28 Suppl 4:S277-84. doi: 10.1016/j.revmed.2007.09.003. Epub 2007 Oct 11. PMID- 25385415 OWN - NLM STAT- MEDLINE DCOM- 20160111 LR - 20181113 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 11 IP - 1 DP - 2015 Jan TI - Connective tissue diseases: New criteria improve recognition of early systemic sclerosis. PG - 3-4 LID - 10.1038/nrrheum.2014.191 [doi] FAU - Asano, Yoshihide AU - Asano Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. FAU - Sato, Shinichi AU - Sato S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. LA - eng PT - News DEP - 20141111 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/immunology MH - Biomarkers/metabolism MH - Connective Tissue Diseases/*diagnosis/immunology/metabolism/pathology MH - Humans MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/pathology MH - Scleroderma, Systemic/*diagnosis/immunology/metabolism/pathology EDAT- 2014/11/12 06:00 MHDA- 2016/01/12 06:00 CRDT- 2014/11/12 06:00 PHST- 2014/11/12 06:00 [entrez] PHST- 2014/11/12 06:00 [pubmed] PHST- 2016/01/12 06:00 [medline] AID - nrrheum.2014.191 [pii] AID - 10.1038/nrrheum.2014.191 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2015 Jan;11(1):3-4. doi: 10.1038/nrrheum.2014.191. Epub 2014 Nov 11. PMID- 19657126 OWN - NLM STAT- MEDLINE DCOM- 20090811 LR - 20101118 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 361 IP - 6 DP - 2009 Aug 6 TI - Clinical problem-solving. A hard diagnosis. PG - 613-7 LID - 10.1056/NEJMcps0804137 [doi] FAU - Margaretten, Mary E AU - Margaretten ME AD - Division of Rheumatology, University of California, San Francisco, San Francisco, CA 94118, USA. mary.margaretten@ucsf.edu FAU - Tierney, Lawrence M Jr AU - Tierney LM Jr FAU - Dhaliwal, Gurpreet AU - Dhaliwal G LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/adverse effects/therapeutic use MH - Blood Chemical Analysis MH - Diagnosis, Differential MH - Edema/etiology MH - Female MH - Humans MH - Hypertension/*etiology MH - Kidney/pathology MH - Middle Aged MH - Raynaud Disease/complications MH - Renal Insufficiency/*etiology/pathology MH - Scleroderma, Systemic/complications/*diagnosis MH - Self Medication MH - Urticaria/etiology EDAT- 2009/08/07 09:00 MHDA- 2009/08/12 09:00 CRDT- 2009/08/07 09:00 PHST- 2009/08/07 09:00 [entrez] PHST- 2009/08/07 09:00 [pubmed] PHST- 2009/08/12 09:00 [medline] AID - 361/6/613 [pii] AID - 10.1056/NEJMcps0804137 [doi] PST - ppublish SO - N Engl J Med. 2009 Aug 6;361(6):613-7. doi: 10.1056/NEJMcps0804137. PMID- 28027937 OWN - NLM STAT- MEDLINE DCOM- 20171002 LR - 20250530 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 111 DP - 2017 May TI - Reduced perfusion in systemic sclerosis digital ulcers (both fingertip and extensor) can be increased by topical application of glyceryl trinitrate. PG - 32-36 LID - S0026-2862(16)30197-2 [pii] LID - 10.1016/j.mvr.2016.12.008 [doi] AB - OBJECTIVES: In patients with systemic sclerosis (SSc), fingertip digital ulcers (DUs) are believed to be ischaemic, and extensor surface DUs a result of mechanical factors/microtrauma. Our aim was to assess blood flow response to topical glyceryl trinitrate (GTN) compared to placebo in SSc DUs, looking for differences in pathophysiology between fingertip and extensor lesions. METHOD: This was a double-blind, randomised, crossover, placebo-controlled study. Sixteen (6 fingertip, 10 extensor) DUs were each studied twice (one day apart): once with GTN and once with placebo ointment. Perfusion at the DU centre ('DUCore') and periphery ('DUPeriphery'), as measured by laser Doppler imaging was performed before and immediately after ointment application, then every 10min, up to 90min post-application. We calculated the area under the response curve (AUC) and the ratio of peak perfusion to baseline, then compared these between GTN and placebo. RESULTS: Perfusion was lower in the DUCore compared to the DUPeriphery (ratio of 0.52). The microvessels of the DUCore were responsive to GTN, with an increase in perfusion, with a similar effect in both fingertip and extensor DUs. The AUC and peak/baseline perfusion difference in means (ratio, 95% confidence interval) between GTN and placebo at the DUCore were 1.2 (1.0-1.6) and 1.2 (1.0-1.5) respectively, and at the DUPeriphery were 1.1 (0.8-1.6) and 1.0 (0.9-1.2) respectively. CONCLUSION: DUs (both fingertip and extensor) were responsive to topical GTN, with an increase in perfusion to the ischaemic DU centre. If both fingertip and extensor DUs have a (potentially reversible) ischaemic aetiology, this has important treatment implications. CI - Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Hughes, M AU - Hughes M AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom. Electronic address: Michael.Hughes-6@postgrad.manchester.ac.uk. FAU - Moore, T AU - Moore T AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, United Kingdom. FAU - Manning, J AU - Manning J AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, United Kingdom. FAU - Wilkinson, J AU - Wilkinson J AD - Research and Development, Salford Royal NHS Foundation Trust, Salford, United Kingdom. FAU - Dinsdale, G AU - Dinsdale G AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom. FAU - Roberts, C AU - Roberts C AD - Centre for Biostatistics, Institute of Population Health, School of Medicine, The University of Manchester, Manchester, United Kingdom. FAU - Murray, A AU - Murray A AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; Photon Science Institute, The University of Manchester, United Kingdom. FAU - Herrick, A L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, United Kingdom. LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom GR - 20482/VAC_/Versus Arthritis/United Kingdom GR - 20482/ARC_/Arthritis Research UK/United Kingdom PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20161224 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Administration, Cutaneous MH - Adult MH - Aged MH - Blood Flow Velocity MH - Cross-Over Studies MH - Double-Blind Method MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/diagnosis/*drug therapy/physiopathology MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation/*drug effects MH - Middle Aged MH - Nitroglycerin/*administration & dosage MH - Perfusion Imaging/methods MH - Raynaud Disease/diagnosis/*drug therapy/physiopathology MH - Recovery of Function MH - Regional Blood Flow MH - Scleroderma, Systemic/diagnosis/*drug therapy/physiopathology MH - Skin Ulcer/diagnosis/*drug therapy/physiopathology MH - Time Factors MH - Treatment Outcome MH - Vasodilation/*drug effects MH - Vasodilator Agents/*administration & dosage PMC - PMC5351498 OTO - NOTNLM OT - Digital ischaemia OT - Digital ulcers OT - Glyceryl trinitrate OT - Microvascular OT - Scleroderma OT - Systemic sclerosis EDAT- 2016/12/29 06:00 MHDA- 2017/10/03 06:00 PMCR- 2017/05/01 CRDT- 2016/12/29 06:00 PHST- 2016/11/12 00:00 [received] PHST- 2016/12/20 00:00 [revised] PHST- 2016/12/20 00:00 [accepted] PHST- 2016/12/29 06:00 [pubmed] PHST- 2017/10/03 06:00 [medline] PHST- 2016/12/29 06:00 [entrez] PHST- 2017/05/01 00:00 [pmc-release] AID - S0026-2862(16)30197-2 [pii] AID - 10.1016/j.mvr.2016.12.008 [doi] PST - ppublish SO - Microvasc Res. 2017 May;111:32-36. doi: 10.1016/j.mvr.2016.12.008. Epub 2016 Dec 24. PMID- 29060127 OWN - NLM STAT- MEDLINE DCOM- 20180824 LR - 20200928 IS - 2694-0604 (Electronic) IS - 2375-7477 (Linking) VI - 2017 DP - 2017 Jul TI - Development of hand blood circulation measurement system for Raynaud syndrome using infrared imaging. PG - 1352-1355 LID - 10.1109/EMBC.2017.8037083 [doi] AB - Raynaud syndrome refers to a medical condition where arterial smooth muscle contraction induces blood flow reduction. Thermal images and questionnaires have been used to assess the efficacy of treatment. In this research, a potential way to measure whole-hand blood circulation in real-time using infrared image is introduced. Normalized average pixel intensity of the regions of interest in hand was measured during or after blood circulation changes brought by temperature alteration and pressure applied to the hand or forearm. The intensity of regions has shown the tendency of the expected amount of blood in the hand in each blood circulation changes. Possible uses of this system are measuring severity of Raynaud syndrome and the efficacy of treatments. FAU - Yunchan Hwang AU - Yunchan Hwang FAU - Minwon Seo AU - Minwon Seo FAU - Jong-Mo Seo AU - Jong-Mo Seo LA - eng PT - Journal Article PL - United States TA - Annu Int Conf IEEE Eng Med Biol Soc JT - Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference JID - 101763872 SB - IM MH - *Cardiovascular System MH - Forearm MH - Hand MH - Humans MH - Raynaud Disease MH - Regional Blood Flow MH - Temperature EDAT- 2017/10/25 06:00 MHDA- 2018/08/25 06:00 CRDT- 2017/10/25 06:00 PHST- 2017/10/25 06:00 [entrez] PHST- 2017/10/25 06:00 [pubmed] PHST- 2018/08/25 06:00 [medline] AID - 10.1109/EMBC.2017.8037083 [doi] PST - ppublish SO - Annu Int Conf IEEE Eng Med Biol Soc. 2017 Jul;2017:1352-1355. doi: 10.1109/EMBC.2017.8037083. PMID- 33506859 OWN - NLM STAT- MEDLINE DCOM- 20211221 LR - 20240404 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 60 IP - 10 DP - 2021 Oct 2 TI - Defining the optimal disease duration of early diffuse systemic sclerosis for clinical trial design. PG - 4662-4670 LID - 10.1093/rheumatology/keab075 [doi] AB - OBJECTIVES: Clinical trials in early diffuse cutaneous systemic sclerosis (SSc) using the modified Rodnan skin score (mRSS) as the primary outcome measure have most often been negative. We wanted to assess how the definition of disease onset (first SSc manifestation vs first non-Raynaud manifestation) and varying lengths of disease duration at trial entry as an inclusion criteria functioned. Our objective was to optimize trial inclusion criteria. METHODS: We used the prospective, observational University of Pittsburgh Scleroderma Cohort to identify early diffuse SSc patients first evaluated between 1980 and 2015. All had <3 years from first SSc (n = 481) or first non-Raynaud manifestation (n = 514) and three or more mRSS scores. We used descriptive, survival and group-based trajectory analyses to compare the different definitions of disease onset and disease duration as inclusion criteria for clinical trials. RESULTS: There was no appreciable difference between using first SSc manifestation compared with first non-Raynaud manifestation as the definition of disease onset. Compared with other disease durations, <18 months of disease had >70% of patients fitting into trajectories with worsening cutaneous disease over 6 months of follow-up. Longer disease durations demonstrated the majority of patients with trajectories showing an improvement in mRSS (regression to the mean) over 6 months. CONCLUSIONS: Regardless of whether the first SSc or first non-Raynaud manifestation is used to define disease onset, duration of <18 months at enrolment is preferable. A longer disease duration criterion more frequently results in regression to the mean of the mRSS score, and likely contributes to negative trial outcomes. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Domsic, Robyn T AU - Domsic RT AUID- ORCID: 0000-0002-2765-0922 AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine. FAU - Gao, Shiyao AU - Gao S AD - Department of Epidemiology, University of Pittsburgh Graduate School of Public Health, Pittsburgh, PA. FAU - Laffoon, Maureen AU - Laffoon M AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine. FAU - Wisniewski, Steven AU - Wisniewski S AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine. FAU - Zhang, Yuqing AU - Zhang Y AD - Division of Epidemiology, Massachusetts General Hospital, Boston, MA. FAU - Steen, Virginia AU - Steen V AD - Division of Rheumatology, Georgetown University, Washington, DC, USA. FAU - Lafyatis, Robert AU - Lafyatis R AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine. FAU - Medsger, Thomas A AU - Medsger TA AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine. LA - eng GR - P50 AR060780/AR/NIAMS NIH HHS/United States GR - R01 AR069874/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Observational Study PT - Research Support, N.I.H., Extramural PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Clinical Trials as Topic/*methods MH - Early Diagnosis MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Patient Acuity MH - *Patient Selection MH - Prospective Studies MH - Raynaud Disease/diagnosis MH - Scleroderma, Diffuse/*diagnosis MH - *Time Factors PMC - PMC8677444 OTO - NOTNLM OT - clinical trial design OT - diffuse scleroderma OT - scleroderma OT - systemic sclerosis EDAT- 2021/01/29 06:00 MHDA- 2021/12/22 06:00 PMCR- 2021/01/28 CRDT- 2021/01/28 08:52 PHST- 2020/09/23 00:00 [received] PHST- 2020/12/15 00:00 [revised] PHST- 2021/01/29 06:00 [pubmed] PHST- 2021/12/22 06:00 [medline] PHST- 2021/01/28 08:52 [entrez] PHST- 2021/01/28 00:00 [pmc-release] AID - 6121911 [pii] AID - keab075 [pii] AID - 10.1093/rheumatology/keab075 [doi] PST - ppublish SO - Rheumatology (Oxford). 2021 Oct 2;60(10):4662-4670. doi: 10.1093/rheumatology/keab075. PMID- 17901977 OWN - NLM STAT- MEDLINE DCOM- 20090421 LR - 20250103 IS - 1432-1246 (Electronic) IS - 0340-0131 (Linking) VI - 81 IP - 5 DP - 2008 Apr TI - Negligent exposures to hand-transmitted vibration. PG - 645-59 AB - OBJECTIVES: If the negligence of an employer results in the disability in an employee, the employer is responsible, in whole or in part, for the disability. The employer is wholly responsible when the worker would not have developed the disability if the employer had taken all reasonable preventative measures. The employer is only partly responsible if the worker would probably have developed some disability even if the employer had taken all reasonable precautions. The employer's responsibility may be estimated from the difference between the actual disability of the worker and the disability that the worker would have suffered if the employer had taken all reasonable preventative measures. This paper considers alternative ways of apportioning negligent and non-negligent exposures to hand-transmitted vibration. RESULTS: The equivalent daily vibration exposure, A(8), used in current EU Directives is shown to be unsuitable for distinguishing between the consequences of negligent and non-negligent exposures because the risks of developing a disorder from hand-transmitted vibration also depend on the years of exposure. Furthermore, daily exposures take no account of individual susceptibility or the practicality of reducing exposure. The consequences of employer negligence may be estimated from the delay in the onset and progression of disorder that would have been achieved if the employer had acted reasonably, such as by reducing vibration magnitude and exposure duration to the minimum that was reasonably achievable in the circumstances. This seems to be fair and reasonable for both employers and employees and indicates the consequences of negligence-the period of the worker's life with disease as a result of negligence and the period for which their employment opportunities may be restricted as a result of the onset of the disorder due to negligence. CONCLUSIONS: The effects of negligence may be estimated from the delay in the onset of disease or disability that would have occurred if the employer had behaved reasonably. This definition of negligence encourages employers to reduce risks to the lowest reasonably practical level, consistent with EU Directives. FAU - Griffin, Michael J AU - Griffin MJ AD - Human Factors Research Unit, Institute of Sound and Vibration Research, University of Southampton, Southampton, UK. M.J.Griffin@soton.ac.uk LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070928 PL - Germany TA - Int Arch Occup Environ Health JT - International archives of occupational and environmental health JID - 7512134 SB - IM MH - Cumulative Trauma Disorders/*etiology/physiopathology MH - Disability Evaluation MH - Persons with Disabilities MH - European Union MH - Fingers/blood supply MH - Hand-Arm Vibration Syndrome/*diagnosis/etiology/physiopathology MH - Humans MH - *Malpractice MH - *Occupational Exposure/adverse effects/legislation & jurisprudence/standards MH - Occupational Health/*legislation & jurisprudence MH - Raynaud Disease/*etiology/physiopathology MH - Vibration/*adverse effects EDAT- 2007/09/29 09:00 MHDA- 2009/04/22 09:00 CRDT- 2007/09/29 09:00 PHST- 2007/08/08 00:00 [received] PHST- 2007/09/05 00:00 [accepted] PHST- 2007/09/29 09:00 [pubmed] PHST- 2009/04/22 09:00 [medline] PHST- 2007/09/29 09:00 [entrez] AID - 10.1007/s00420-007-0251-7 [doi] PST - ppublish SO - Int Arch Occup Environ Health. 2008 Apr;81(5):645-59. doi: 10.1007/s00420-007-0251-7. Epub 2007 Sep 28. PMID- 20562195 OWN - NLM STAT- MEDLINE DCOM- 20110923 LR - 20110118 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 50 IP - 2 DP - 2011 Feb TI - Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features. PG - 317-23 LID - 10.1093/rheumatology/keq176 [doi] AB - OBJECTIVE: To assess internal organ involvement in early SSc at presentation. METHODS: One hundred and fifteen patients admitted to a tertiary centre because of RP, who did not present any routinely detectable scleroderma-related internal organ involvement, were investigated for ANA and videocapillaroscopy, and underwent history and physical examination to detect symptoms/signs suggestive of SSc. Patients were then subdivided into three groups: (i) early SSc, constituted by patients without clinical manifestations other than RP, but with scleroderma marker autoantibodies and/or typical capillaroscopic abnormalities; (ii) probable SSc, constituted by patients with the same autoantibody and/or capillaroscopic status as early SSc patients, but with any of the following manifestations: digital ulcers/scars, puffy fingers, arthritis, telangiectasia, dysphagia/heartburn, shortness of breath; (iii) UCTD, constituted by patients with a specific (i.e. disease antibody marker) ANA and capillaroscopic findings plus any disease manifestation. All patients were investigated by lung functional study and B-mode echo-Doppler-cardiography. Patients who consented underwent oesophageal manometry. RESULTS: An inverted mitral E : A ratio (i.e. early scleroderma cardiac involvement) and/or a diffusing lung capacity for CO <80% of the predictive value (i.e. early lung involvement) and/or basal low oesophageal sphincter pressure <15 mmHg (i.e. early oesophageal involvement) were detected in 37/51 probable SSc patients (72%), 8/19 early SSc patients (42%) and 12/45 UCTD patients (27%). CONCLUSION: A scleroderma-related internal organ involvement was detected in patients from each group and, more importantly, was pre-clinical in a number of cases. FAU - Valentini, Gabriele AU - Valentini G AD - Unità di Reumatologia della Seconda Università di Napoli, Policlinico, Via Pansini, 5-80131 Napoli, Italy. gabriele.valentini@unina2.it FAU - Cuomo, Giovanna AU - Cuomo G FAU - Abignano, Giuseppina AU - Abignano G FAU - Petrillo, Ambrogio AU - Petrillo A FAU - Vettori, Serena AU - Vettori S FAU - Capasso, Alessia AU - Capasso A FAU - Cozzolino, Domenico AU - Cozzolino D FAU - Del Genio, Gianmattia AU - Del Genio G FAU - Santoriello, Carlo AU - Santoriello C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100618 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2011 Feb;50(2):250-1. doi: 10.1093/rheumatology/keq374. PMID: 21115465 MH - Adolescent MH - Adult MH - Aged MH - Child MH - Disease Progression MH - Early Diagnosis MH - Female MH - Gastrointestinal Diseases/physiopathology MH - Heart Diseases/physiopathology MH - Humans MH - Kidney Diseases/physiopathology MH - Lung Diseases/physiopathology MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Raynaud Disease/*complications/physiopathology MH - Scleroderma, Systemic/*physiopathology MH - Time Factors MH - Young Adult EDAT- 2010/06/22 06:00 MHDA- 2011/09/29 06:00 CRDT- 2010/06/22 06:00 PHST- 2010/06/22 06:00 [entrez] PHST- 2010/06/22 06:00 [pubmed] PHST- 2011/09/29 06:00 [medline] AID - keq176 [pii] AID - 10.1093/rheumatology/keq176 [doi] PST - ppublish SO - Rheumatology (Oxford). 2011 Feb;50(2):317-23. doi: 10.1093/rheumatology/keq176. Epub 2010 Jun 18. PMID- 27307533 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20180207 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 43 IP - 8 DP - 2016 Aug TI - Nailfold Videocapillaroscopy Alterations in Dermatomyositis and Systemic Sclerosis: Toward Identification of a Specific Pattern. PG - 1575-80 LID - 10.3899/jrheum.160122 [doi] AB - OBJECTIVE: The term scleroderma pattern typically defines capillary abnormalities of scleroderma spectrum disorders, mainly systemic sclerosis (SSc) and dermatomyositis (DM). Our study aimed to investigate differences in nailfold videocapillaroscopy (NVC) between DM and SSc, with a cross-sectional and longitudinal evaluation. METHODS: NVC features of 29 consecutive patients with DM were compared with 90 patients with SSc categorized into the 3 subsets of scleroderma pattern: early, active, and late. Twenty patients with DM and all with SSc were also longitudinally reevaluated after 30 months of followup. RESULTS: At baseline, all SSc groups showed giant capillaries, with significant differences with DM only for early and active pattern. Ramified capillaries were significantly more frequent and severe in DM than in early and active patterns, while DM showed an opposite trend compared with late pattern. Capillary loss was lower in early pattern and higher in active and late, compared with DM. Finally, giant-ramified capillaries were almost exclusive of DM. During followup, NVC showed a different evolution in DM and SSc. In DM we recorded a reduction of giant capillaries, while ramified capillaries increased both in DM and in early and active SSc pattern. The number of capillaries recovered in DM; conversely, capillary loss slightly worsened in all SSc patterns. Giant-ramified capillaries significantly decreased in patients with DM, remaining rare in patients with SSc. CONCLUSION: Our study strengthens the specificity of DM and SSc microangiopathy and points out the need for large prospective studies to confirm our results and possibly to revise current terminology by distinguishing between "scleroderma" and "dermatomyositis" patterns. FAU - Manfredi, Andreina AU - Manfredi A AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. andreina.manfredi@gmail.com. FAU - Sebastiani, Marco AU - Sebastiani M AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Campomori, Federica AU - Campomori F AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Pipitone, Nicolò AU - Pipitone N AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Giuggioli, Dilia AU - Giuggioli D AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Colaci, Michele AU - Colaci M AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Praino, Emanuela AU - Praino E AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. FAU - Ferri, Clodoveo AU - Ferri C AD - From the Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena; Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; Rheumatology Unit, University of Bari, Bari, Italy.A. Manfredi, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Sebastiani, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; F. Campomori, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; N. Pipitone, MD, Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia; D. Giuggioli, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; M. Colaci, MD, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena; E. Praino, MD, Rheumatology Unit, University of Bari; C. Ferri, MD, Professor, Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena. LA - eng PT - Journal Article DEP - 20160615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Dermatomyositis/*diagnostic imaging/physiopathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Raynaud Disease/*diagnostic imaging/physiopathology MH - Scleroderma, Systemic/*diagnostic imaging/physiopathology OTO - NOTNLM OT - DERMATOMYOSITIS OT - MICROANGIOPATHY OT - SCLERODERMA PATTERN OT - SYSTEMIC SCLEROSIS OT - VIDEOCAPILLAROSCOPY EDAT- 2016/06/17 06:00 MHDA- 2017/12/22 06:00 CRDT- 2016/06/17 06:00 PHST- 2016/04/19 00:00 [accepted] PHST- 2016/06/17 06:00 [entrez] PHST- 2016/06/17 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] AID - jrheum.160122 [pii] AID - 10.3899/jrheum.160122 [doi] PST - ppublish SO - J Rheumatol. 2016 Aug;43(8):1575-80. doi: 10.3899/jrheum.160122. Epub 2016 Jun 15. PMID- 18203763 OWN - NLM STAT- MEDLINE DCOM- 20090126 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 68 IP - 1 DP - 2009 Jan TI - Sclerosing skin disorders in association with multiple sclerosis. Coincidence, underlying autoimmune pathology or interferon induced? PG - 47-50 LID - 10.1136/ard.2007.083246 [doi] AB - OBJECTIVES: To describe and analyse the manifestation of sclerosing skin disorders in patients with multiple sclerosis (MS). CASE REPORTS: We describe three patients with relapsing-remitting MS who developed skin sclerosis while receiving interferon (IFN)-beta treatment and review nine further cases of systemic sclerosis (SSc) in MS from the literature. Of all 12 patients reported, eight had limited cutaneous SSc, three had diffuse cutaneous SSc and one patient had an antisynthetase syndrome. Localised scleroderma such as morphoea was not described. The mean age at diagnosis was 25.2 years for MS (range 12 to 51) and 38.3 years for SSc (range 16 to 66). Eleven patients developed SSc after the onset of MS and manifested with skin sclerosis after a mean of 14.9 years (range 1 to 45). In five patients IFN-beta was commenced before the development of skin sclerosis (mean 4.6 years, range 1 to 8 years). There was no relationship between the onset of skin sclerosis and MS activity. With the exception of one individual, all patients had antinuclear antibodies. CONCLUSIONS: Sclerosing skin disorders may develop in the course of MS. The relatively early age of SSc onset in patients with MS suggests a genetic predisposition and/or an IFN-associated trigger. FAU - Hügle, T AU - Hügle T AD - Felix Platter Spital, Basel University Department of Rheumatology, Switzerland. FAU - Gratzl, S AU - Gratzl S FAU - Daikeler, T AU - Daikeler T FAU - Frey, D AU - Frey D FAU - Tyndall, A AU - Tyndall A FAU - Walker, U A AU - Walker UA LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20080118 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Adjuvants, Immunologic) RN - 77238-31-4 (Interferon-beta) SB - IM MH - Adjuvants, Immunologic/adverse effects/therapeutic use MH - Aged MH - Female MH - Humans MH - Interferon-beta/adverse effects/therapeutic use MH - Male MH - Middle Aged MH - Multiple Sclerosis, Relapsing-Remitting/*complications/immunology MH - Raynaud Disease/complications/immunology MH - Scleroderma, Diffuse/*complications/immunology MH - Time Factors RF - 23 EDAT- 2008/01/22 09:00 MHDA- 2009/01/27 09:00 CRDT- 2008/01/22 09:00 PHST- 2008/01/22 09:00 [entrez] PHST- 2008/01/22 09:00 [pubmed] PHST- 2009/01/27 09:00 [medline] AID - S0003-4967(24)42657-5 [pii] AID - 10.1136/ard.2007.083246 [doi] PST - ppublish SO - Ann Rheum Dis. 2009 Jan;68(1):47-50. doi: 10.1136/ard.2007.083246. Epub 2008 Jan 18. PMID- 23324736 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - Nailfold capillaroscopy abnormalities as predictors of mortality in patients with systemic sclerosis. PG - 103-8 AB - OBJECTIVES: Peripheral microangiopathy is a hallmark of systemic sclerosis (SSc) and can be early detected by nailfold capillaroscopy (NFC). This study aimed to examine whether more severe peripheral microangiopathy at NFC are predictive factor for death in SSc patients. METHODS: 135 SSc patients who performed NFC between June 2001 and July 2009 were included. The following NFC parameters were evaluated: number of capillary loops/mm, avascular score (scored from 0 to 3), and number of enlarged and giant capillary loops. Univariate and multivariate regression models were used to analyse the association of mortality with NFC and clinical parameters. RESULTS: At the time of the analysis (August 2010), 123 patients were alive, and 12 were dead. By univariate analysis, male gender, forced vital capacity <75% predicted, higher number of giant capillary loops, and an avascular score >1.5 on NFC were associated with a significantly increase risk of death. By multivariate analysis, an avascular score >1.5 was the only independent predictor of death (hazard ratio 2.265). Survival rates from diagnosis at 1, 5 and 10 years were lower in patients with avascular score >1.5 (97%, 86%, and 59%, respectively) compared with those with avascular score ≤1.5 (97%, 97%, and 91% respectively) (p=0.009 by log rank test). CONCLUSIONS: Avascular scores higher than 1.5 at NFC was an independent predictor of death in SSc, suggesting that NFC can be useful for predicting SSc outcome. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil. cristiane.kayser@unifesp.br FAU - Sekiyama, Juliana Y AU - Sekiyama JY FAU - Próspero, Lucas C AU - Próspero LC FAU - Camargo, Cintia Z AU - Camargo CZ FAU - Andrade, Luis E C AU - Andrade LE LA - eng PT - Journal Article DEP - 20130116 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Female MH - Follow-Up Studies MH - Humans MH - Kaplan-Meier Estimate MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Multivariate Analysis MH - Nails/*blood supply MH - Predictive Value of Tests MH - Raynaud Disease/mortality/physiopathology MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*mortality/*physiopathology MH - Vital Capacity EDAT- 2013/01/18 06:00 MHDA- 2013/09/11 06:00 CRDT- 2013/01/18 06:00 PHST- 2012/02/18 00:00 [received] PHST- 2012/09/05 00:00 [accepted] PHST- 2013/01/18 06:00 [entrez] PHST- 2013/01/18 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 5874 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):103-8. Epub 2013 Jan 16. PMID- 23321020 OWN - NLM STAT- MEDLINE DCOM- 20140212 LR - 20130719 IS - 1545-7206 (Electronic) IS - 0033-3182 (Linking) VI - 54 IP - 4 DP - 2013 Jul-Aug TI - Neuropsychiatric symptoms in scleroderma. PG - 382-6 LID - S0033-3182(12)00165-X [pii] LID - 10.1016/j.psym.2012.09.002 [doi] FAU - McNair, Susan AU - McNair S AD - Department of Psychiatry, Behavioral Neurosciences, Michael G. DeGroote School of Medicine, Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada. susan.mcnair@medportal.ca FAU - Hategan, Ana AU - Hategan A FAU - Bourgeois, James A AU - Bourgeois JA FAU - Losier, Bruno AU - Losier B LA - eng PT - Case Reports PT - Journal Article DEP - 20130112 PL - England TA - Psychosomatics JT - Psychosomatics JID - 0376506 RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Aged MH - Antibodies, Anticardiolipin/metabolism MH - Antibodies, Antinuclear/metabolism MH - Bipolar Disorder/psychology MH - CREST Syndrome/complications/diagnosis/*physiopathology MH - Cognition Disorders/diagnosis/etiology MH - Dementia, Vascular/diagnosis/*etiology MH - Diagnosis, Differential MH - Female MH - Hospitalization MH - Humans MH - Medication Adherence MH - Neuropsychological Tests MH - Raynaud Disease/pathology EDAT- 2013/01/17 06:00 MHDA- 2014/02/13 06:00 CRDT- 2013/01/17 06:00 PHST- 2012/08/16 00:00 [received] PHST- 2012/09/08 00:00 [revised] PHST- 2012/09/10 00:00 [accepted] PHST- 2013/01/17 06:00 [entrez] PHST- 2013/01/17 06:00 [pubmed] PHST- 2014/02/13 06:00 [medline] AID - S0033-3182(12)00165-X [pii] AID - 10.1016/j.psym.2012.09.002 [doi] PST - ppublish SO - Psychosomatics. 2013 Jul-Aug;54(4):382-6. doi: 10.1016/j.psym.2012.09.002. Epub 2013 Jan 12. PMID- 23268365 OWN - NLM STAT- MEDLINE DCOM- 20140120 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 72 IP - 12 DP - 2013 Dec TI - A multi-centre, blinded, randomised, placebo-controlled, laboratory-based study of MQX-503, a novel topical gel formulation of nitroglycerine, in patients with Raynaud phenomenon. PG - 1962-7 LID - 10.1136/annrheumdis-2012-201536 [doi] AB - OBJECTIVE: MQX-503 is a novel nitroglycerine preparation designed to absorb quickly and allow local vasodilatation in the skin. We examined the efficacy and tolerability of this medication in Raynaud phenomenon (RP) in a laboratory-based study. METHODS: In this multi-centre, double-blind, randomised, placebo-controlled, cross-over study, subjects were treated with 0.5% or 1.25% nitroglycerine or placebo gel. Subjects received each dose twice in a randomised order. Each study session consisted of baseline laser Doppler measurements, study gel application and 5 min of cold chamber exposure (-20°C). Blood flow (BF) was measured at the end of exposure and for the next 120 min at set intervals. Other outcome measures included achievement of baseline BF; the time to achieve 50% and 70% baseline skin temperature (ST); and pain, tingling and numbness scores. RESULTS: 37 subjects completed 214 treatment periods. Time to achieve baseline BF was significantly shorter in the two treated groups (HR=1.77 and 2.02 for 0.5% and 1.25% vs placebo, respectively). The proportion of subjects achieving baseline BF was 45.8% for placebo, 66.2% for 0.5% and 69% for 1.25% (p=0.01 and p=0.002 for 0.5% and 1.25% vs placebo, respectively). No meaningful differences were seen in ST or pain/numbness/tingling scores. Treatment was well tolerated with no serious adverse events. CONCLUSIONS: Treatment with MQX-503 caused a significant improvement in skin BF compared with placebo. Data from this proof of concept study suggest benefit of MQX-503 in subjects with RP. FAU - Hummers, Laura K AU - Hummers LK AD - Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, , Baltimore, Maryland, USA. FAU - Dugowson, Carin E AU - Dugowson CE FAU - Dechow, Frederick J AU - Dechow FJ FAU - Wise, Robert A AU - Wise RA FAU - Gregory, Jeffrey AU - Gregory J FAU - Michalek, Joel AU - Michalek J FAU - Yenokyan, Gayane AU - Yenokyan G FAU - McGready, John AU - McGready J FAU - Wigley, Fredrick M AU - Wigley FM LA - eng PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial DEP - 20121225 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Gels) RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Administration, Cutaneous MH - Adult MH - Aged MH - Chemistry, Pharmaceutical MH - Cross-Over Studies MH - Dose-Response Relationship, Drug MH - Double-Blind Method MH - Female MH - Gels MH - Humans MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Nitroglycerin/administration & dosage/adverse effects/*therapeutic use MH - Raynaud Disease/*drug therapy/physiopathology MH - Regional Blood Flow/drug effects MH - Skin/blood supply MH - Skin Temperature/drug effects MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage/adverse effects/*therapeutic use MH - Young Adult OTO - NOTNLM OT - Systemic Lupus Erythematosus OT - Systemic Sclerosis OT - Treatment EDAT- 2012/12/27 06:00 MHDA- 2014/01/21 06:00 CRDT- 2012/12/27 06:00 PHST- 2012/12/27 06:00 [entrez] PHST- 2012/12/27 06:00 [pubmed] PHST- 2014/01/21 06:00 [medline] AID - S0003-4967(24)21281-4 [pii] AID - 10.1136/annrheumdis-2012-201536 [doi] PST - ppublish SO - Ann Rheum Dis. 2013 Dec;72(12):1962-7. doi: 10.1136/annrheumdis-2012-201536. Epub 2012 Dec 25. PMID- 38394270 OWN - NLM STAT- MEDLINE DCOM- 20240226 LR - 20260430 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 92 IP - 3 DP - 2024 Mar 1 TI - Medium- and Long-Term Outcomes of Autologous Fat Grafting to Hands and Feet for Patients With Raynaud Phenomenon. PG - 287-293 LID - 10.1097/SAP.0000000000003752 [doi] AB - BACKGROUND: Autologous fat grafting (AFG) has emerged as a promising treatment option for Raynaud phenomenon. However, existing studies are limited by short follow-up, and there is little evidence regarding predictive factors for successful outcomes. METHODS: A retrospective chart review and standardized phone interviews were performed for all patients (n = 17, 65% response rate) treated with AFG to the hands or feet at our institution for primary or secondary Raynaud from 2010 to 2021. Each occurrence of AFG was defined as a separate surgery (n = 23), with an average follow-up of 3.7 years. RESULTS: At follow-up, patients reported a 31% reduction in cold attack frequency, a 45% reduction in the intensity of individual attacks, a 29% reduction in the duration of attacks, and a 40% improvement in overall Raynaud Condition Score (P < 0.01). Although initial AFG to an extremity significantly improved symptoms, subsequent attempts were not shown to statistically improve outcomes. Digital ulcers were present in 65% of cases, and AFG resulted in ulcer healing in 87% of those cases. Median duration of maximum symptom relief was 1 year postoperatively, with 74% of patients reporting diminishing symptom relief by 4 years postoperatively. Those with a BMI ≥25, with primary Raynaud phenomenon or without preoperative ulcers experienced significantly longer symptom relief (P < 0.05). Average patient satisfaction was 7.7 of 10, and 91% would recommend the procedure to others. CONCLUSIONS: Autologous fat grafting is an effective, albeit sometimes temporary, treatment for Raynaud and digital ulcers. Certain patients may be more likely to experience lasting symptom relief beyond 1 year. CI - Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved. FAU - Haravu, Pranav N AU - Haravu PN AD - From the Pritzker School of Medicine, University of Chicago. FAU - Bond, Stephanie AU - Bond S AD - Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago, Chicago, IL. FAU - Hendren-Santiago, Bryce AU - Hendren-Santiago B AD - From the Pritzker School of Medicine, University of Chicago. FAU - Prescher, Hannes AU - Prescher H AD - Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago, Chicago, IL. FAU - Bank, Jonathan AU - Bank J AD - New York Breast Reconstruction and Aesthetic Plastic Surgery, Great Neck, NY. FAU - Zachary, Lawrence S AU - Zachary LS AD - Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago, Chicago, IL. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231220 PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - digital ulcers SB - IM MH - Humans MH - *Adipose Tissue/transplantation MH - Retrospective Studies MH - Hand/surgery MH - Transplantation, Autologous/methods MH - *Raynaud Disease/surgery MH - *Skin Ulcer COIS- Financial disclosure statement: The authors have no financial conflicts of interest to disclose. EDAT- 2024/02/23 18:42 MHDA- 2024/02/26 06:44 CRDT- 2024/02/23 14:06 PHST- 2024/02/26 06:44 [medline] PHST- 2024/02/23 18:42 [pubmed] PHST- 2024/02/23 14:06 [entrez] AID - 00000637-202403000-00008 [pii] AID - 10.1097/SAP.0000000000003752 [doi] PST - ppublish SO - Ann Plast Surg. 2024 Mar 1;92(3):287-293. doi: 10.1097/SAP.0000000000003752. Epub 2023 Dec 20. PMID- 16220222 OWN - NLM STAT- MEDLINE DCOM- 20061012 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 3 DP - 2006 May TI - Incidence and clinical correlation of anticentromere antibody in Thai patients. PG - 325-8 AB - Anticentromere antibodies (ACA) are useful in assessing and classifying patients with mild variant of systemic sclerosis called calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias (CREST) syndrome. From their prognostic significance, we are interested in the prevalence and disease correlation in Thai patients. A total of 3,233 serum samples of patients with any musculoskeletal symptoms were sent for antinuclear antibody determination at Ramathibodi Immunology Laboratory Service between the years 1998 and 2001. Forty sera (1.23%) were ACA positive. These sera were from 27 patients with autoimmune diseases and 13 with nonautoimmune diseases. Among autoimmune group, scleroderma was the most common diagnosis (33.3%) with limited sclerosis being the most frequent variant. The percentages of autoimmune disease were almost the same among the low-titer (1:40) and the high-titer (1:640) groups. The study suggests that the prevalence of ACA in Thai patients is low. The presence of ACA detected in patients with vague musculoskeletal symptoms does not suggest a diagnosis of CREST syndrome. Even high-titer ACA can be found in nonautoimmune diseases. FAU - Pakunpanya, Krisaree AU - Pakunpanya K AD - Division of Allergy, Immunology and Rheumatology, Faculty of Medicine, Ramathibodi Hospital, Rama 6 Road, Bangkok 10400, Thailand. rasjw@mahidol.ac.th FAU - Verasertniyom, Oravan AU - Verasertniyom O FAU - Vanichapuntu, Monchand AU - Vanichapuntu M FAU - Pisitkun, Prapaporn AU - Pisitkun P FAU - Totemchokchyakarn, Kitti AU - Totemchokchyakarn K FAU - Nantiruj, Kanokrat AU - Nantiruj K FAU - Janwityanujit, Suchela AU - Janwityanujit S LA - eng PT - Journal Article DEP - 20051012 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/*blood MH - Autoimmune Diseases/epidemiology/*immunology MH - CREST Syndrome/epidemiology/*immunology MH - Centromere/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Limited/epidemiology/*immunology MH - Thailand/epidemiology EDAT- 2005/10/13 09:00 MHDA- 2006/10/13 09:00 CRDT- 2005/10/13 09:00 PHST- 2004/05/24 00:00 [received] PHST- 2005/05/14 00:00 [accepted] PHST- 2005/05/14 00:00 [revised] PHST- 2005/10/13 09:00 [pubmed] PHST- 2006/10/13 09:00 [medline] PHST- 2005/10/13 09:00 [entrez] AID - 10.1007/s10067-005-0005-4 [doi] PST - ppublish SO - Clin Rheumatol. 2006 May;25(3):325-8. doi: 10.1007/s10067-005-0005-4. Epub 2005 Oct 12. PMID- 27245655 OWN - NLM STAT- MEDLINE DCOM- 20161110 LR - 20181202 IS - 1524-4539 (Electronic) IS - 0009-7322 (Linking) VI - 133 IP - 22 DP - 2016 May 31 TI - Response to Letter Regarding Article, "Relation of Nailfold Capillaries and Autoantibodies to Mortality in Patients With Raynaud Phenomenon". PG - e669 LID - 10.1161/CIRCULATIONAHA.116.022853 [doi] FAU - Schlager, Oliver AU - Schlager O AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. FAU - Mueller, Markus AU - Mueller M AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. FAU - Gamper, Jutta AU - Gamper J AD - Center of Medical Statistics, Informatics and Intelligent Systems Medical University of Vienna Vienna, Austria. FAU - Giurgea, Georgiana-Aura AU - Giurgea GA AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. FAU - Charwat-Resl, Silvia AU - Charwat-Resl S AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. FAU - Kiener, Hans P AU - Kiener HP AD - Division of Rheumatology Department of Medicine III Medical University of Vienna Vienna, Austria. FAU - Smolen, Josef S AU - Smolen JS AD - Division of Rheumatology Department of Medicine III Medical University of Vienna Vienna, Austria. FAU - Perkmann, Thomas AU - Perkmann T AD - Department of Laboratory Medicine Medical University of Vienna Vienna, Austria. FAU - Koppensteiner, Renate AU - Koppensteiner R AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. FAU - Gschwandtner, Michael E AU - Gschwandtner ME AD - Division of Angiology Department of Medicine II Medical University of Vienna Vienna, Austria. LA - eng PT - Comment PT - Letter PL - United States TA - Circulation JT - Circulation JID - 0147763 RN - 0 (Autoantibodies) SB - IM CON - Circulation. 2016 Feb 2;133(5):509-17. doi: 10.1161/CIRCULATIONAHA.115.017816. PMID: 26733605 CON - Circulation. 2016 May 31;133(22):e668. doi: 10.1161/CIRCULATIONAHA.116.021987. PMID: 27245654 MH - *Autoantibodies MH - *Capillaries MH - Connective Tissue Diseases MH - Humans MH - Raynaud Disease EDAT- 2016/06/02 06:00 MHDA- 2016/11/11 06:00 CRDT- 2016/06/02 06:00 PHST- 2016/06/02 06:00 [entrez] PHST- 2016/06/02 06:00 [pubmed] PHST- 2016/11/11 06:00 [medline] AID - CIRCULATIONAHA.116.022853 [pii] AID - 10.1161/CIRCULATIONAHA.116.022853 [doi] PST - ppublish SO - Circulation. 2016 May 31;133(22):e669. doi: 10.1161/CIRCULATIONAHA.116.022853. PMID- 19152463 OWN - NLM STAT- MEDLINE DCOM- 20090128 LR - 20190724 IS - 0021-5384 (Print) IS - 0021-5384 (Linking) VI - 97 IP - 10 DP - 2008 Oct 10 TI - [Clinical and diagnostic findings in rheumatism]. PG - 2587-9 FAU - Takahashi, Yuichi AU - Takahashi Y LA - jpn PT - Journal Article PL - Japan TA - Nihon Naika Gakkai Zasshi JT - Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine JID - 19130210R RN - 0 (Biomarkers) RN - 9007-41-4 (C-Reactive Protein) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Arthralgia MH - Biomarkers/blood MH - Blood Sedimentation MH - C-Reactive Protein/analysis MH - Fever of Unknown Origin MH - Humans MH - Muscle Rigidity MH - Prednisolone/administration & dosage MH - Raynaud Disease MH - Rheumatic Diseases/*diagnosis/drug therapy EDAT- 2009/01/21 09:00 MHDA- 2009/01/29 09:00 CRDT- 2009/01/21 09:00 PHST- 2009/01/21 09:00 [entrez] PHST- 2009/01/21 09:00 [pubmed] PHST- 2009/01/29 09:00 [medline] AID - 10.2169/naika.97.2587 [doi] PST - ppublish SO - Nihon Naika Gakkai Zasshi. 2008 Oct 10;97(10):2587-9. doi: 10.2169/naika.97.2587. PMID- 19683883 OWN - NLM STAT- MEDLINE DCOM- 20091230 LR - 20221207 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 34 IP - 8 DP - 2009 Oct TI - Long-term results of periarterial sympathectomy. PG - 1454-60 LID - 10.1016/j.jhsa.2009.05.003 [doi] AB - PURPOSE: To compare long-term results (minimum follow-up of 23 months) of periarterial sympathectomy for patients with digital vasospasm secondary to either an autoimmune disease or generalized atherosclerotic disease. Patients with posttraumatic or localized occlusive disease and vasospasm were not evaluated. METHODS: Twenty-eight patients had periarterial sympathectomy at 1 hospital by 1 senior surgeon. Periarterial sympathectomy was targeted to the areas of ulceration. Twenty patients (with 24 involved extremities and 42 ulcerated digits) had a documented autoimmune disease; 17 patients had scleroderma or an undifferentiated mixed connective tissue disorder, 2 had systemic lupus erythematosus, and 1 had rheumatoid arthritis. Eight patients (with 9 involved extremities and 17 ulcerated digits) had atherosclerotic disease. The primary outcomes were complete healing of all ulcers, a decrease in the number of ulcers, and need for amputation by the end of follow-up. Statistical analysis was done using the Fischer exact t-test. RESULTS: The average follow-up for all patients was 96 months (90 months for the autoimmune group and 113 months for the atherosclerotic group). Fifteen of the 20 patients (28 of 42 digits) in the autoimmune group had complete healing or decrease in ulcer number. Conversely, only 1 of the 8 patients (2 of 17 digits) in the atherosclerotic group had complete healing or decrease in ulcer number. Eleven of the 42 (26%) digits treated in the autoimmune group required amputation. In contrast, 10 of the 17 (59%) digits treated in the atherosclerotic group ultimately required amputation. CONCLUSIONS: Periarterial sympathectomy can lead to complete healing and decrease in ulcer number in autoimmune disease patients with digital ischemia from vasospasm. However, our data suggest that periarterial sympathectomy may be of little or no benefit in patients with chronic digital ischemia and vasospasm secondary to severe atherosclerotic disease. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III. FAU - Hartzell, Tristan L AU - Hartzell TL AD - Department of Surgery, Division of Plastic Surgery, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA. thartzell@partners.org FAU - Makhni, Eric C AU - Makhni EC FAU - Sampson, Christian AU - Sampson C LA - eng PT - Journal Article DEP - 20090815 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 SB - IM MH - Adult MH - Aged MH - Amputation, Surgical MH - Arteries/innervation MH - Atherosclerosis/complications/surgery MH - Autoimmune Diseases/complications/surgery MH - Chronic Disease MH - Female MH - Fingers/*blood supply MH - Follow-Up Studies MH - Humans MH - Ischemia/*surgery MH - Male MH - Middle Aged MH - Postoperative Complications/*etiology/surgery MH - Raynaud Disease/complications/surgery MH - Reoperation MH - Retrospective Studies MH - Skin Ulcer/surgery MH - *Sympathectomy MH - Wound Healing/physiology EDAT- 2009/08/18 09:00 MHDA- 2009/12/31 06:00 CRDT- 2009/08/18 09:00 PHST- 2008/10/31 00:00 [received] PHST- 2009/04/30 00:00 [revised] PHST- 2009/05/04 00:00 [accepted] PHST- 2009/08/18 09:00 [entrez] PHST- 2009/08/18 09:00 [pubmed] PHST- 2009/12/31 06:00 [medline] AID - S0363-5023(09)00438-9 [pii] AID - 10.1016/j.jhsa.2009.05.003 [doi] PST - ppublish SO - J Hand Surg Am. 2009 Oct;34(8):1454-60. doi: 10.1016/j.jhsa.2009.05.003. Epub 2009 Aug 15. PMID- 28921059 OWN - NLM STAT- MEDLINE DCOM- 20180103 LR - 20230215 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 19 IP - 10 DP - 2017 Sep 18 TI - Very Early Systemic Sclerosis and Pre-systemic Sclerosis: Definition, Recognition, Clinical Relevance and Future Directions. PG - 65 LID - 10.1007/s11926-017-0684-2 [doi] AB - PURPOSE OF REVIEW: The approach to systemic sclerosis (SSc) has changed over the years with an increasing focus on the very early diagnosis of the disease. The terminology identifying patients in the early phase of SSc has been significantly confusing in the last three decades. The purpose of this article is to analyze how the concept of "very early SSc" has evolved over the years, which is the role of an early diagnosis and how early treat patients. RECENT FINDINGS: Several attempts have been made over time, to create more sensitive and specific classification criteria to include the largest number of SSc patients, also in the earliest phase. An algorythm for the very early diagnosis of SSc was identified, diagnostic preliminary criteria proposed, and new 2013 ACR/EULAR SSc classification criteria published, including new items and adding emphasis to the vasculopathic manifestations. True biomarkers that could predict the disease evolution are still missing. Treat or not to treat patients in the earliest phases still remain a dilemma. For the moment, the only feasible clinical strategy in very early SSc remains a tight follow up program to detect in "real time" the early internal organ involvement which may allow an aggressive therapeutic agenda. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy. s.bellandorandone@gmail.com. AD - Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy. s.bellandorandone@gmail.com. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology, AOUC, Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20170918 PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 SB - IM MH - Algorithms MH - Early Diagnosis MH - Humans MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/classification/*diagnosis/drug therapy OTO - NOTNLM OT - Classification criteria OT - Early diagnosis OT - Puffy fingers OT - Raynaud phenomenon OT - Systemic sclerosis EDAT- 2017/09/19 06:00 MHDA- 2018/01/04 06:00 CRDT- 2017/09/19 06:00 PHST- 2017/09/19 06:00 [entrez] PHST- 2017/09/19 06:00 [pubmed] PHST- 2018/01/04 06:00 [medline] AID - 10.1007/s11926-017-0684-2 [pii] AID - 10.1007/s11926-017-0684-2 [doi] PST - epublish SO - Curr Rheumatol Rep. 2017 Sep 18;19(10):65. doi: 10.1007/s11926-017-0684-2. PMID- 25651660 OWN - NLM STAT- MEDLINE DCOM- 20150218 LR - 20150205 IS - 0043-3284 (Print) IS - 0043-3284 (Linking) VI - 110 IP - 6 DP - 2014 Nov-Dec TI - Lupus coagulopathy post-tasing: a case study. PG - 20-2 AB - While severe adverse effects are rare, evidence suggests significant physiological effects may be associated with the use of electrical incapacitation devices, or TASERs. In this case, a 28 year old Caucasian female with chronic, stable systemic lupus erythematosus (SLE) was voluntarily tased in a work-related training procedure. Days later, she presented to the emergency room with an acute flare of her lupus and a constellation of symptoms that lead to new diagnoses of antiphospholipid syndrome (APS), Raynaud phenomenon, acute lower left extremity deep vein thrombosis (DVT), and diffuse bilateral pulmonary emboli (PEs). Due to the temporal relationship of these complications and this patient's history of autoimmune disorders, it is reasonable to believe that an APS was both induced by the tasing event and associated with her lupus. FAU - Bryant, Jordan AU - Bryant J FAU - Forty, Theresa AU - Forty T FAU - Ignacio, Shanley AU - Ignacio S FAU - Shelton, Julie AU - Shelton J LA - eng PT - Case Reports PT - Journal Article PL - United States TA - W V Med J JT - The West Virginia medical journal JID - 0413777 SB - IM MH - Adult MH - Antiphospholipid Syndrome/*diagnosis/etiology MH - Conducted Energy Weapon Injuries/*complications MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Pulmonary Embolism/etiology MH - Raynaud Disease/etiology MH - Sjogren's Syndrome/*complications MH - Venous Thrombosis/etiology EDAT- 2015/02/06 06:00 MHDA- 2015/02/19 06:00 CRDT- 2015/02/06 06:00 PHST- 2015/02/06 06:00 [entrez] PHST- 2015/02/06 06:00 [pubmed] PHST- 2015/02/19 06:00 [medline] PST - ppublish SO - W V Med J. 2014 Nov-Dec;110(6):20-2. PMID- 18066551 OWN - NLM STAT- MEDLINE DCOM- 20080724 LR - 20211020 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 28 IP - 7 DP - 2008 May TI - Onset and enhancement of systemic sclerosis after treatments for multiple sclerosis. PG - 703-7 AB - The coexistence of systemic sclerosis (SSc) and multiple sclerosis (MS) in the same patient has been described in few cases. We refer here a further case of association between these diseases, which highlight the difficulty of treating such patients. A 57-year-old male with relapsing-remitting MS since 20 years, shortly after having received high-dose corticosteroids for a relapse of MS, suddenly developed SSc, with onset of Raynaud phenomenon simultaneous to that of scleroderma skin involvement, new appearance of accelerated arterial hypertension, and rapidly progressive oliguric renal failure, indicative of scleroderma renal crisis, that was controlled with ramipril, irbesartan and amlodipin. A further disabling relapse of MS was treated with interferon-beta, but 19 months later he developed multiple severe digital necrotic ulcers, that resolved with interferon discontinuation and therapy with iloprost. This case report shows that some form of treatment useful for MS might enhance the manifestations of SSc. FAU - Airo', Paolo AU - Airo' P AD - Rheumatology and Clinical Immunology, Spedali Civili, Piazza Spedali Civili, 25124 Brescia, Italy. airo@bresciareumatologia.it FAU - Scarsi, Mirko AU - Scarsi M FAU - Rossi, Mara AU - Rossi M FAU - Mondini, Michele AU - Mondini M LA - eng PT - Case Reports PT - Journal Article DEP - 20071208 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Interferon Type I) SB - IM MH - Humans MH - Interferon Type I/adverse effects MH - Male MH - Middle Aged MH - Multiple Sclerosis/complications/*drug therapy MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*etiology RF - 25 EDAT- 2007/12/11 09:00 MHDA- 2008/07/25 09:00 CRDT- 2007/12/11 09:00 PHST- 2007/06/08 00:00 [received] PHST- 2007/11/24 00:00 [accepted] PHST- 2007/12/11 09:00 [pubmed] PHST- 2008/07/25 09:00 [medline] PHST- 2007/12/11 09:00 [entrez] AID - 10.1007/s00296-007-0507-2 [doi] PST - ppublish SO - Rheumatol Int. 2008 May;28(7):703-7. doi: 10.1007/s00296-007-0507-2. Epub 2007 Dec 8. PMID- 35023438 OWN - NLM STAT- MEDLINE DCOM- 20220301 LR - 20220301 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 18 IP - 1 DP - 2022 Jan TI - Early diagnosis of systemic sclerosis, where do we stand today? PG - 1-3 LID - 10.1080/1744666X.2022.2015327 [doi] FAU - Lepri, Gemma AU - Lepri G AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Florence, Italy. FAU - Bellando Randone, Silvia AU - Bellando Randone S AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Florence, Italy. FAU - Matucci Cerinic, Marco AU - Matucci Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Florence, Italy. AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UNIRAR), Irccs San Raffaele Hospital, Milan, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit AOUC, Florence, Italy. LA - eng PT - Editorial DEP - 20220113 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - Early Diagnosis MH - Humans MH - *Raynaud Disease MH - *Scleroderma, Systemic/diagnosis OTO - NOTNLM OT - Systemic sclerosis OT - classification criteria OT - early diagnosis OT - organ damage OT - prediction OT - treatment EDAT- 2022/01/14 06:00 MHDA- 2022/03/03 06:00 CRDT- 2022/01/13 08:44 PHST- 2022/01/14 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2022/01/13 08:44 [entrez] AID - 10.1080/1744666X.2022.2015327 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2022 Jan;18(1):1-3. doi: 10.1080/1744666X.2022.2015327. Epub 2022 Jan 13. PMID- 31370770 OWN - NLM STAT- MEDLINE DCOM- 20201207 LR - 20201214 IS - 2043-6289 (Electronic) IS - 0266-7681 (Linking) VI - 44 IP - 9 DP - 2019 Nov TI - Vascular compromise during wide-awake local anaesthesia no tourniquet technique for distal radial plating: a case report. PG - 980-983 LID - 10.1177/1753193419865082 [doi] FAU - Liu, Wen-Chih AU - Liu WC AD - Division of Orthopedic Surgery, Kaohsiung Municipal Siaogang Hospital, Taiwan. AD - Department Orthopedic Surgery, Kaohsiung Medical University Hospital, Taiwan. FAU - Fu, Yin-Chih AU - Fu YC AD - Department Orthopedic Surgery, Kaohsiung Medical University Hospital, Taiwan. AD - School of Medicine, College of Medicine, Kaohsiung Medical University, Taiwan. FAU - Lu, Chun-Kuan AU - Lu CK AD - Department Orthopedic Surgery, Kaohsiung Medical University Hospital, Taiwan. LA - eng PT - Case Reports PT - Letter DEP - 20190801 PL - England TA - J Hand Surg Eur Vol JT - The Journal of hand surgery, European volume JID - 101315820 RN - 0 (Anesthetics, Local) SB - IM MH - Adult MH - Anesthesia, Local/*methods MH - Anesthetics, Local/*administration & dosage/adverse effects MH - Female MH - Fracture Fixation, Internal/*methods MH - Hand/*blood supply MH - Humans MH - Radius Fractures/*surgery MH - Raynaud Disease/*complications MH - Wakefulness EDAT- 2019/08/03 06:00 MHDA- 2020/12/15 06:00 CRDT- 2019/08/03 06:00 PHST- 2019/08/03 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2019/08/03 06:00 [entrez] AID - 10.1177/1753193419865082 [doi] PST - ppublish SO - J Hand Surg Eur Vol. 2019 Nov;44(9):980-983. doi: 10.1177/1753193419865082. Epub 2019 Aug 1. PMID- 23388228 OWN - NLM STAT- MEDLINE DCOM- 20131205 LR - 20161125 IS - 1557-2501 (Electronic) IS - 1042-3931 (Linking) VI - 25 IP - 2 DP - 2013 Feb TI - Acute hand ischemia after radial intervention in patient with CREST-associated pulmonary hypertension: successful treatment with manual thromboaspiration. PG - 89-91 AB - We describe the case of a 60-year-old woman with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) associated pulmonary hypertension undergoing transradial coronary angiography. The day after the procedure, the patient complained of severe symptoms and signs of acute hand ischemia. Urgent right upper extremity angiography showed the lack of ulnar palmar arch and a severe narrowed radial artery with endoluminal filling defect. The patient was successfully treated with manual thromboaspiration leading to a complete flow restoration and symptom relief. This case shows that radial occlusion, one of the most common and usually asymptomatic complications following transradial cardiac catheterization, may cause severe hand ischemia in patients with small-vessel inflammatory disease. FAU - Taglieri, Nevio AU - Taglieri N AD - Institute of Cardiology, Bologna University, St Orsola/Malpighi Hospital, Via Massarenti 9, 40138, Bologna, Italy. vbneviotaglieri@hotmail.it FAU - Galiè, Nazzareno AU - Galiè N FAU - Marzocchi, Antonio AU - Marzocchi A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Invasive Cardiol JT - The Journal of invasive cardiology JID - 8917477 SB - IM MH - Acute Disease MH - Arterial Occlusive Diseases/complications/diagnostic imaging MH - Calcinosis/complications MH - Catheterization, Peripheral/*adverse effects MH - Esophageal Motility Disorders/complications MH - Female MH - Hand/*blood supply MH - Humans MH - Hypertension, Pulmonary/complications/*therapy MH - Iatrogenic Disease MH - Ischemia/diagnostic imaging/*etiology MH - Middle Aged MH - Radial Artery/diagnostic imaging/*injuries MH - Radiography MH - Raynaud Disease/complications MH - Telangiectasis/*complications MH - Thrombectomy/*adverse effects/methods EDAT- 2013/02/08 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/02/08 06:00 PHST- 2013/02/08 06:00 [entrez] PHST- 2013/02/08 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] PST - ppublish SO - J Invasive Cardiol. 2013 Feb;25(2):89-91. PMID- 24445877 OWN - NLM STAT- MEDLINE DCOM- 20140626 LR - 20210106 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 133 IP - 5 DP - 2014 May TI - Fat grafting to the hand in patients with Raynaud phenomenon: a novel therapeutic modality. PG - 1109-1118 LID - 10.1097/PRS.0000000000000104 [doi] AB - BACKGROUND: Raynaud phenomenon causes progressively decreasing blood flow to the extremities, resulting from an imbalance between vasoconstriction and vasodilation. Treatment options include biofeedback, phosphodiesterase inhibitors, calcium channel inhibitors, botulinum toxin injection, or surgical sympathectomy. The authors propose fat grafting to the hands as a method to delay progression of the disease. METHODS: Indications included symptomatic Raynaud phenomenon with failure of previous management. Fat is harvested from abdominal depots. Approximately 30 ml of decanted fat is injected by means of blunt cannulae: 10 to 15 ml in the dorsum of the hand, 2 to 3 ml in the snuffbox, 1 to 2 ml in each dorsal webspace, 3 to 4 ml along the superficial palmar arch, 1 to 2 ml in volar webspaces 2 to 4, and 2 to 3 ml in the first webspace. Patients underwent preoperative and postoperative laser speckle imaging study to assess changes in perfusion. RESULTS: A total of 13 patients were treated (21 hands). Twelve patients had undergone prior botulinum toxin injection, and 11 patients had prior sympathectomies. Findings included reduced pain (average reduction, 6.86 of 10 to 2.38 of 10), fewer cold attacks, improved skin and soft-tissue texture, decrease in ulcerations, and patient-reported improved function. Three patients had no changes. Increased blood flow per imaging was noted in five of 11 hands tested. Six patients had decreased readings on laser imaging. None of the laser speckle imaging changes were statistically significant, and they did not correlate clinically. There were no major complications. CONCLUSIONS: Preliminary results of fat grafting to the hands of patients with Raynaud phenomenon revealed improved symptomatology with evidence suggestive of measurably increased perfusion in some cases. Fat grafting may benefit the management of this patient population. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV. FAU - Bank, Jonathan AU - Bank J AD - Chicago, Ill. From the Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago Medical Center. FAU - Fuller, Sam M AU - Fuller SM FAU - Henry, Ginard I AU - Henry GI FAU - Zachary, Lawrence S AU - Zachary LS LA - eng PT - Clinical Trial PT - Journal Article PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM CIN - Plast Reconstr Surg. 2015 Jan;135(1):229e-230e. doi: 10.1097/PRS.0000000000000814. PMID: 25539337 CIN - Plast Reconstr Surg. 2015 Jan;135(1):230e-231e. doi: 10.1097/PRS.0000000000000789. PMID: 25539338 MH - Abdomen MH - Adipose Tissue/*transplantation MH - Adult MH - Aged MH - Female MH - Fingers/blood supply/surgery MH - Hand/*blood supply/*surgery MH - Humans MH - Ischemia/*surgery MH - Male MH - Middle Aged MH - Optical Imaging MH - Raynaud Disease/*surgery MH - Skin Ulcer/surgery MH - Treatment Outcome MH - Vasoconstriction MH - Vasodilation EDAT- 2014/01/22 06:00 MHDA- 2014/06/27 06:00 CRDT- 2014/01/22 06:00 PHST- 2014/01/22 06:00 [entrez] PHST- 2014/01/22 06:00 [pubmed] PHST- 2014/06/27 06:00 [medline] AID - 00006534-201405000-00013 [pii] AID - 10.1097/PRS.0000000000000104 [doi] PST - ppublish SO - Plast Reconstr Surg. 2014 May;133(5):1109-1118. doi: 10.1097/PRS.0000000000000104. PMID- 30543199 OWN - NLM STAT- MEDLINE DCOM- 20200220 LR - 20200220 IS - 1897-9483 (Electronic) IS - 0032-3772 (Linking) VI - 129 IP - 1 DP - 2019 Jan 31 TI - Microcirculation disorders of the oral cavity in patients with primary Raynaud phenomenon. PG - 36-42 LID - 10.20452/pamw.4389 [doi] AB - INTRODUCTION Raynaud phenomenon is a medical condition in which the spasm of the arteries causes episodes of reduced blood flow. Potential disorders in the microcirculation of the oral mucosa may promote the occurrence of lesions. OBJECTIVES The aim of the study was to investigate the association of the frequency of oral cavity lesions with oral microcirculatory dysfunction in patients with primary Raynaud phenomenon (PRP) in comparison with healthy control group. PATIENTS AND METHODS Measurements of oral capillary flow were performed using laser doppler flowmetry (LDF) in 61 patients with PRP. In a group of 31 of 61 patients (group 1), the measurements were made during a Raynaud phenomenon (RP) attack. The RP attack was caused by stress initiated by the examination or the first visit itself. The RP attack was not deliberately caused by a cold test, vibration40w56 or any stress test. After 10 to 14 days, the measurements were repeated in all 61 patients and in the control group, and a dental examination was performed. Follow‑up visits were conducted every 3 months for a period of 12 months to monitor oral mucosa. RESULTS Differences in LDF were found between various anatomical points in both the PRP and control groups. On the first visit, the LDF flow in group 1 was significantly lower at all examined points in comparison with those in the control group. On the second visit, differences were observed in the LDF of the teeth and oral mucosa temperature in all patients with PRP in comparison with controls. Oral cavity lesions reported in the past and at follow‑up were significantly more common in patients with PRP. CONCLUSIONS Patients with PRP have dysfunction in the microcirculation of the oral mucosa and they more often have lesions in the oral cavity. FAU - Gregorczyk-Maga, Iwona AU - Gregorczyk-Maga I AD - Institute of Dentistry, Jagiellonian University Medical College, Kraków, Poland FAU - Frołow, Marzena AU - Frołow M AD - Department of Angiology, Jagiellonian University Medical College, Kraków, Poland FAU - Kaczmarczyk, Paweł AU - Kaczmarczyk P AD - Department of Angiology, Jagiellonian University Medical College, Kraków, Poland FAU - Maga, Paweł AU - Maga P AD - Department of Angiology, Jagiellonian University Medical College, Kraków, Poland LA - eng PT - Journal Article DEP - 20181213 PL - Poland TA - Pol Arch Intern Med JT - Polish archives of internal medicine JID - 101700960 SB - IM MH - Adult MH - Capillaries MH - Female MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - *Microcirculation MH - Mouth/*blood supply MH - Mouth Mucosa/blood supply MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow MH - Young Adult EDAT- 2018/12/14 06:00 MHDA- 2020/02/23 06:00 CRDT- 2018/12/14 06:00 PHST- 2018/12/14 06:00 [pubmed] PHST- 2020/02/23 06:00 [medline] PHST- 2018/12/14 06:00 [entrez] AID - 10.20452/pamw.4389 [doi] PST - ppublish SO - Pol Arch Intern Med. 2019 Jan 31;129(1):36-42. doi: 10.20452/pamw.4389. Epub 2018 Dec 13. PMID- 17060652 OWN - NLM STAT- MEDLINE DCOM- 20061107 LR - 20081120 IS - 1488-2329 (Electronic) IS - 0820-3946 (Print) IS - 0820-3946 (Linking) VI - 175 IP - 9 DP - 2006 Oct 24 TI - Soft-tissue infection and underlying calcinosis of CREST syndrome. PG - 1059 FAU - Tangri, Navdeep AU - Tangri N AD - Department of Medicine, McGill University, Montréal, Que. FAU - Young, Barbara M AU - Young BM LA - eng PT - Case Reports PT - Journal Article PL - Canada TA - CMAJ JT - CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne JID - 9711805 SB - IM MH - Calcinosis/*complications MH - Esophageal Motility Disorders MH - Female MH - Humans MH - Middle Aged MH - Raynaud Disease MH - Scleroderma, Localized MH - Soft Tissue Infections/*etiology MH - Staphylococcal Infections/diagnosis/drug therapy/*etiology MH - Syndrome MH - Thumb/microbiology/pathology PMC - PMC1609158 EDAT- 2006/10/25 09:00 MHDA- 2006/11/09 09:00 PMCR- 2006/10/24 CRDT- 2006/10/25 09:00 PHST- 2006/10/25 09:00 [pubmed] PHST- 2006/11/09 09:00 [medline] PHST- 2006/10/25 09:00 [entrez] PHST- 2006/10/24 00:00 [pmc-release] AID - 175/9/1059-b [pii] AID - 0002792-200610240-00016 [pii] AID - 10.1503/cmaj.060521 [doi] PST - ppublish SO - CMAJ. 2006 Oct 24;175(9):1059. doi: 10.1503/cmaj.060521. PMID- 17047894 OWN - NLM STAT- MEDLINE DCOM- 20070731 LR - 20220716 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 6 DP - 2007 Jun TI - Correlation between homocysteine plasma levels and nailfold videocapillaroscopic patterns in systemic sclerosis. PG - 902-7 AB - The aim of the study is to investigate the relationship between microangiopathy as assessed by nailfold videocapillaroscopy (NVC) and plasma level of homocysteine (Hcy) in systemic sclerosis (SSc). As known, Hcy is a nonessential amino acid that interferes with normal properties of a vascular tree. Sixty patients affected by SSc (4 men and 56 women, mean age 54.6) underwent the determination of plasma Hcy level; at the same time, NVC was performed. Hcy level was also determined in 30 sex- and age-matched controls. In patients affected by SSc the plasma Hcy level was significantly higher than in healthy controls (11.8 and 6.5 micromol/l, respectively; p < 0.001). A significant correlation was found between plasma Hcy concentration and the pattern of NVC with a progressive increase from early to active and above all to late pattern (10.7, 11.8, and 17.4 micromol/l, respectively; p < 0.001). Subjects with high Hcy level (i.e., >75th percentile of Hcy level in controls and in patients considered altogether) were mostly represented in the scleroderma patients with late nailfold videocapillaroscopic pattern; the crude odds ratio was 9.0 (significant; 95% CI from 2.1 to 38.8). In conclusion, Hcy plasma level is related to microvascular involvement in patients affected by SSc; the concentration increases with the progression of the nailfold videocapillaroscopic pattern. Hyperhomocysteinemia may represent an aggravating factor among the complex mechanisms involved in scleroderma damage contributing to the injury of endothelium. FAU - Caramaschi, Paola AU - Caramaschi P AD - Dipartimento di Medicina Clinica e Sperimentale, Università di Verona, Policlinico GB Rossi, P.le Scuro, Verona, 37134, Italy. paola.caramaschi@azosp.vr.it FAU - Volpe, Alessandro AU - Volpe A FAU - Canestrini, Sabrina AU - Canestrini S FAU - Bambara, Lisa M AU - Bambara LM FAU - Faccini, Giovanni AU - Faccini G FAU - Carletto, Antonio AU - Carletto A FAU - Biasi, Domenico AU - Biasi D LA - eng PT - Journal Article DEP - 20061018 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0LVT1QZ0BA (Homocysteine) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Capillaries/*pathology MH - Case-Control Studies MH - Endothelium, Vascular/pathology MH - Female MH - Homocysteine/*blood MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Raynaud Disease/blood/pathology MH - Scleroderma, Systemic/*blood/*pathology EDAT- 2006/10/19 09:00 MHDA- 2007/08/01 09:00 CRDT- 2006/10/19 09:00 PHST- 2006/06/07 00:00 [received] PHST- 2006/08/14 00:00 [accepted] PHST- 2006/07/26 00:00 [revised] PHST- 2006/10/19 09:00 [pubmed] PHST- 2007/08/01 09:00 [medline] PHST- 2006/10/19 09:00 [entrez] AID - 10.1007/s10067-006-0425-9 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Jun;26(6):902-7. doi: 10.1007/s10067-006-0425-9. Epub 2006 Oct 18. PMID- 19464224 OWN - NLM STAT- MEDLINE DCOM- 20100108 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 76 IP - 5 DP - 2009 Oct TI - Long-term efficacy of mycophenolate mofetil in a case of refractory antisynthetase syndrome. PG - 575-6 LID - 10.1016/j.jbspin.2009.02.004 [doi] FAU - Hervier, Baptiste AU - Hervier B FAU - Masseau, Agathe AU - Masseau A FAU - Mussini, Jean-Marie AU - Mussini JM FAU - Audrain, Marie AU - Audrain M FAU - Hamidou, Mohamed A AU - Hamidou MA LA - eng PT - Case Reports PT - Letter DEP - 20090521 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) RN - 83HN0GTJ6D (Cyclosporine) RN - 9007-41-4 (C-Reactive Protein) RN - HU9DX48N0T (Mycophenolic Acid) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adult MH - C-Reactive Protein/metabolism MH - Cyclosporine/therapeutic use MH - Diabetes Mellitus, Type 1/complications MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - Immunosuppressive Agents/*therapeutic use MH - Male MH - Methotrexate/therapeutic use MH - Mycophenolic Acid/*analogs & derivatives/therapeutic use MH - Protoporphyria, Erythropoietic/*drug therapy MH - Raynaud Disease/complications EDAT- 2009/05/26 09:00 MHDA- 2010/01/09 06:00 CRDT- 2009/05/26 09:00 PHST- 2009/01/21 00:00 [received] PHST- 2009/02/04 00:00 [accepted] PHST- 2009/05/26 09:00 [entrez] PHST- 2009/05/26 09:00 [pubmed] PHST- 2010/01/09 06:00 [medline] AID - S1297-319X(09)00055-4 [pii] AID - 10.1016/j.jbspin.2009.02.004 [doi] PST - ppublish SO - Joint Bone Spine. 2009 Oct;76(5):575-6. doi: 10.1016/j.jbspin.2009.02.004. Epub 2009 May 21. PMID- 28391171 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20181202 IS - 1878-0180 (Electronic) IS - 1878-0180 (Linking) VI - 71 DP - 2017 Jul TI - A two scale modeling and computational framework for vibration-induced Raynaud syndrome. PG - 320-328 LID - S1751-6161(17)30129-7 [pii] LID - 10.1016/j.jmbbm.2017.03.019 [doi] AB - Hand-Arm Vibration syndrome (HAVS), usually caused by long-term use of hand-held power tools, can in certain manifestations alter the peripheral blood circulation in the hand-arm region. HAVS typically occurs after exposure to cold, causing an abnormally strong vasoconstriction of blood vessels. A pathoanatomical mechanism suggests that a reduction of the lumen of the blood vessels in VWF (Vibration White Finger) subjects, due to either hypertrophy or thickening of the vessel wall, may be at the origin of the disease. However, the direct and indirect effects of the load of the hand-held tools on the structure of blood vessels remain controversial:.one hypothesis is the mechanical action of vibration on the local acral dysregulation and/or on the vessel histomorphological modifications. Another hypothesis is the participation of the sympathetic nervous system to this dysregulation. In this paper, we assume the modifications as mechanobiological growth and the load-effect relationship may be interpreted as directly or indirectly induced. This work is the first attempt to model the effect of vibration through soft tissues onto the distal capillaries, addressing the double paradigm of multi space-time scales, i.e. low period vibration versus high time constant of the growth phenomenon as well as vibrations propagating in the macroscopic tissue including the microscopic capillary structures subjected to a pathological microstructural evolution. The objective is to lay down the theoretical basis of growth modeling for the small distal artery, with the ability to predict the geometrical and structural changes of the arterial walls caused by vibration exposure. We adopt the key idea of splitting the problem into one global vibration problem at the macroscopic scale and one local growth problem at the micro level. The macroscopic hyperelastic viscous dynamic model of the fingertip cross-section is validated by fitting experimental data. It is then used in steady-state vibration conditions to predict the mechanical fields in the close vicinity of capillaries. The space scale transfer from macroscopic to microscopic levels is ensured by considering a representative volume element (RVE) embedding a single capillary in its center. The vibrations emitted by the hand held power tool are next linked to the capillary growth through the adopted biomechanical growth model at the capillary level. The obtained results show that vibrations induce an increase of the thickness of the capillary's wall, thereby confirming the scenario of vibrations induced reduction of the lumen of blood vessels. CI - Copyright © 2017 Elsevier Ltd. All rights reserved. FAU - Hua, Yue AU - Hua Y AD - INRS, Institut National de Recherche et de Sécurité, 1, rue du Morvan, 54519 Vandœuvre Cedex, France; CNRS, LEMTA, UMR 7563, Université de Lorraine, 2, Avenue de la forêt de Haye, BP 90161, 54505 Vandoeuvre-lès-Nancy, France. FAU - Lemerle, Pierre AU - Lemerle P AD - INRS, Institut National de Recherche et de Sécurité, 1, rue du Morvan, 54519 Vandœuvre Cedex, France. FAU - Ganghoffer, Jean-François AU - Ganghoffer JF AD - CNRS, LEMTA, UMR 7563, Université de Lorraine, 2, Avenue de la forêt de Haye, BP 90161, 54505 Vandoeuvre-lès-Nancy, France. Electronic address: jean-francois.ganghoffer@univ-lorraine.fr. LA - eng PT - Journal Article DEP - 20170328 PL - Netherlands TA - J Mech Behav Biomed Mater JT - Journal of the mechanical behavior of biomedical materials JID - 101322406 SB - IM MH - Fingers/blood supply MH - Finite Element Analysis MH - Hand-Arm Vibration Syndrome/*physiopathology MH - Humans MH - Models, Biological MH - Raynaud Disease/etiology/*physiopathology MH - Vasoconstriction MH - Vibration/*adverse effects OTO - NOTNLM OT - Capillary OT - Finite-element method OT - Global and local scales OT - Growth OT - Vibration OT - White finger EDAT- 2017/04/10 06:00 MHDA- 2017/12/22 06:00 CRDT- 2017/04/10 06:00 PHST- 2017/01/03 00:00 [received] PHST- 2017/03/15 00:00 [revised] PHST- 2017/03/23 00:00 [accepted] PHST- 2017/04/10 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/04/10 06:00 [entrez] AID - S1751-6161(17)30129-7 [pii] AID - 10.1016/j.jmbbm.2017.03.019 [doi] PST - ppublish SO - J Mech Behav Biomed Mater. 2017 Jul;71:320-328. doi: 10.1016/j.jmbbm.2017.03.019. Epub 2017 Mar 28. PMID- 26232308 OWN - NLM STAT- MEDLINE DCOM- 20170807 LR - 20171116 IS - 1578-2190 (Electronic) IS - 0001-7310 (Linking) VI - 106 IP - 10 DP - 2015 Dec TI - Mixed Connective Tissue Disease in a Patient With Castleman Disease and Hodgkin Lymphoma: Excellent Clinical Response to Rituximab. PG - 843-6 LID - S0001-7310(15)00283-5 [pii] LID - 10.1016/j.ad.2015.03.017 [doi] FAU - Gracia-Cazaña, T AU - Gracia-Cazaña T AD - Servicio de Dermatología, Hospital de Barbastro, Huesca, España. Electronic address: tamara_gracia@hotmail.com. FAU - Delgado-Beltrán, C AU - Delgado-Beltrán C AD - Servicio de Reumatología, Hospital Clínico Lozano Blesa, Zaragoza, España. FAU - Concellón, M A AU - Concellón MA AD - Servicio de Dermatología, Hospital Clínico Lozano Blesa, Zaragoza, España. FAU - Fuertes, M A AU - Fuertes MA AD - Servicio de Hematología, Hospital Clínico Lozano Blesa, Zaragoza, España. LA - eng LA - spa PT - Case Reports PT - Letter DEP - 20150729 PL - Spain TA - Actas Dermosifiliogr JT - Actas dermo-sifiliograficas JID - 0373062 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antimalarials) RN - 4F4X42SYQ6 (Rituximab) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Antimalarials/therapeutic use MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Castleman Disease/drug therapy/*etiology/immunology MH - Drug Therapy, Combination MH - Hodgkin Disease/*complications/drug therapy MH - Humans MH - Male MH - Methylprednisolone/therapeutic use MH - Middle Aged MH - Mixed Connective Tissue Disease/drug therapy/*etiology MH - Raynaud Disease/etiology MH - Remission Induction MH - Rituximab/*therapeutic use EDAT- 2015/08/02 06:00 MHDA- 2017/08/08 06:00 CRDT- 2015/08/02 06:00 PHST- 2015/01/20 00:00 [received] PHST- 2015/03/25 00:00 [revised] PHST- 2015/03/29 00:00 [accepted] PHST- 2015/08/02 06:00 [entrez] PHST- 2015/08/02 06:00 [pubmed] PHST- 2017/08/08 06:00 [medline] AID - S0001-7310(15)00283-5 [pii] AID - 10.1016/j.ad.2015.03.017 [doi] PST - ppublish SO - Actas Dermosifiliogr. 2015 Dec;106(10):843-6. doi: 10.1016/j.ad.2015.03.017. Epub 2015 Jul 29. PMID- 21487301 OWN - NLM STAT- MEDLINE DCOM- 20111122 LR - 20250529 IS - 1536-0210 (Electronic) IS - 0020-9996 (Print) IS - 0020-9996 (Linking) VI - 46 IP - 8 DP - 2011 Aug TI - Noncontrast magnetic resonance angiography of the hand: improved arterial conspicuity by multidirectional flow-sensitive dephasing magnetization preparation in 3D balanced steady-state free precession imaging. PG - 515-23 LID - 10.1097/RLI.0b013e318217daee [doi] AB - PURPOSE: : To develop a flow-sensitive dephasing (FSD) preparative scheme to facilitate multidirectional flow-signal suppression in 3-dimensional balanced steady-state free precession imaging and to validate the feasibility of the refined sequence for noncontrast magnetic resonance angiography (NC-MRA) of the hand. MATERIALS AND METHODS: : A new FSD preparative scheme was developed that combines 2 conventional FSD modules. Studies using a flow phantom (gadolinium-doped water 15 cm/s) and the hands of 11 healthy volunteers (6 males and 5 females) were performed to compare the proposed FSD scheme with its conventional counterpart with respect to the signal suppression of multidirectional flow. In 9 of the 11 healthy subjects and 2 patients with suspected vasculitis and documented Raynaud phenomenon, respectively, 3-dimensional balanced steady-state free precession imaging coupled with the new FSD scheme was compared with spatial-resolution-matched (0.94 × 0.94 × 0.94 mm) contrast-enhanced magnetic resonance angiography (0.15 mmol/kg gadopentetate dimeglumine) in terms of overall image quality, venous contamination, motion degradation, and arterial conspicuity. RESULTS: : The proposed FSD scheme was able to suppress 2-dimensional flow signal in the flow phantom and hands and yielded significantly higher arterial conspicuity scores than the conventional scheme did on NC-MRA at the regions of common digitals and proper digitals. Compared with contrast-enhanced magnetic resonance angiography, the refined NC-MRA technique yielded comparable overall image quality and motion degradation, significantly less venous contamination, and significantly higher arterial conspicuity score at digital arteries. CONCLUSION: : The FSD-based NC-MRA technique is improved in the depiction of multidirectional flow by applying a 2-module FSD preparation, which enhances its potential to serve as an alternative magnetic resonance angiography technique for the assessment of hand vascular abnormalities. FAU - Fan, Zhaoyang AU - Fan Z AD - Biomedical Imaging Research Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA. FAU - Hodnett, Philip A AU - Hodnett PA FAU - Davarpanah, Amir H AU - Davarpanah AH FAU - Scanlon, Timothy G AU - Scanlon TG FAU - Sheehan, John J AU - Sheehan JJ FAU - Varga, John AU - Varga J FAU - Carr, James C AU - Carr JC FAU - Li, Debiao AU - Li D LA - eng GR - R01 HL096119/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Invest Radiol JT - Investigative radiology JID - 0045377 RN - K2I13DR72L (Gadolinium DTPA) SB - IM MH - Adult MH - Arteries/pathology/*physiology MH - Female MH - Gadolinium DTPA MH - Hand/*blood supply/pathology MH - *Hemodynamics MH - Humans MH - Image Processing, Computer-Assisted MH - Imaging, Three-Dimensional/*instrumentation MH - Magnetic Resonance Angiography/*instrumentation/methods MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/diagnosis/pathology MH - Statistics, Nonparametric MH - Vasculitis/diagnosis/pathology MH - Young Adult PMC - PMC3129329 MID - NIHMS285511 EDAT- 2011/04/14 06:00 MHDA- 2011/12/13 00:00 PMCR- 2012/08/01 CRDT- 2011/04/14 06:00 PHST- 2011/04/14 06:00 [entrez] PHST- 2011/04/14 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] PHST- 2012/08/01 00:00 [pmc-release] AID - 10.1097/RLI.0b013e318217daee [doi] PST - ppublish SO - Invest Radiol. 2011 Aug;46(8):515-23. doi: 10.1097/RLI.0b013e318217daee. PMID- 24847906 OWN - NLM STAT- MEDLINE DCOM- 20151030 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 6 Suppl 86 DP - 2014 Nov-Dec TI - Dysfunctional arteriovenous anastomoses in hands of systemic sclerosis patients with digital ulcers. PG - S-53-9 AB - OBJECTIVES: Previous studies indicate that the arteriovenous anastomoses (AVAs) and the arterioles with the nutritive flow are involved in the pathophysiologic process disturbing hand blood flow in systemic sclerosis (SSc). However, impact of different part of the microvascular system involved in digital ulcers (DU) is not well known. Here, we aimed to assess the vasomotor activity of the AVAs in the hands of patients with and without DU in SSc. METHODS: Simultaneous recordings were made of laser Doppler flux in the finger pulp and thenar eminence, together with ipsilateral radial artery blood velocity and mean arterial blood pressure (MAP) in 22 non-smoking SSc patients and 13 aged-matched healthy controls. RESULTS: AVA responses in the finger pulp to spontaneous vasoconstrictor nerve impulses were abolished in 64% of the SSc patients. Correlation and cross-spectra analysis showed positive correlation between blood flow changes and MAP changes, indicating a passive vascular bed in the SSc finger pulp with blood flow variations depending on short-term variability in MAP. Dysfunctional AVAs were identified in all the patients with a history of DU (n=8), while none of the patients with normal AVA function had episodes of DU (n=8) (p= 0.017). CONCLUSIONS: We found that in SSc patients with DU there is a dysfunction of the AVAs of the finger pulp. This proof-of-concept study supports the notion that AVA dysfunction may play a critical role in SSc related DU. AVA dysfunction may be a part of autonomic dysfunction in SSc. FAU - Bergersen, T K AU - Bergersen TK AD - Department of Dermatology, Oslo University Hospital, Oslo, Norway. kbergers@ous-hf.no. FAU - Hoffmann-Vold, A-M AU - Hoffmann-Vold AM FAU - Midtvedt, Ø AU - Midtvedt Ø FAU - Gran, J T AU - Gran JT FAU - Mørk, C AU - Mørk C FAU - Toska, K AU - Toska K FAU - Elstad, M AU - Elstad M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140521 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Aged MH - Arteriovenous Anastomosis/*physiopathology MH - Case-Control Studies MH - Female MH - Fingers/*blood supply MH - Hand Dermatoses/etiology/*physiopathology MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - *Microcirculation MH - Middle Aged MH - Raynaud Disease/etiology/*physiopathology MH - Regional Blood Flow MH - Scleroderma, Systemic/complications/*physiopathology MH - Skin Ulcer/etiology/*physiopathology EDAT- 2014/05/23 06:00 MHDA- 2015/10/31 06:00 CRDT- 2014/05/23 06:00 PHST- 2013/10/25 00:00 [received] PHST- 2013/12/18 00:00 [accepted] PHST- 2014/05/23 06:00 [entrez] PHST- 2014/05/23 06:00 [pubmed] PHST- 2015/10/31 06:00 [medline] AID - 7693 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-53-9. Epub 2014 May 21. PMID- 28053333 OWN - NLM STAT- MEDLINE DCOM- 20190729 LR - 20190729 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 13 IP - 3 DP - 2017 Mar TI - Connective tissue disease: Reflections on the EULAR recommendations for the treatment of systemic sclerosis. PG - 134-136 LID - 10.1038/nrrheum.2016.212 [doi] FAU - Pope, Janet E AU - Pope JE AD - St. Joseph's Health Care, 268 Grosvenor St, London, Ontario N6A 4V2, Canada; and at University of Western Ontario, Schulich School of Medicine &Dentistry, 1151 Richmond St, London, Ontario, N6A 5C1, Canada. LA - eng PT - Journal Article DEP - 20170105 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - 0 (Platelet Aggregation Inhibitors) RN - JED5K35YGL (Iloprost) MH - Administration, Intravenous MH - Connective Tissue Diseases/classification/*drug therapy/epidemiology/therapy MH - Disease Management MH - Humans MH - Iloprost/administration & dosage/therapeutic use MH - Platelet Aggregation Inhibitors/therapeutic use MH - Practice Guidelines as Topic MH - Raynaud Disease/*drug therapy MH - Scleroderma, Systemic/*drug therapy/epidemiology/therapy EDAT- 2017/01/06 06:00 MHDA- 2019/07/30 06:00 CRDT- 2017/01/06 06:00 PHST- 2017/01/06 06:00 [pubmed] PHST- 2019/07/30 06:00 [medline] PHST- 2017/01/06 06:00 [entrez] AID - nrrheum.2016.212 [pii] AID - 10.1038/nrrheum.2016.212 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2017 Mar;13(3):134-136. doi: 10.1038/nrrheum.2016.212. Epub 2017 Jan 5. PMID- 24347137 OWN - NLM STAT- MEDLINE DCOM- 20150108 LR - 20141112 IS - 1708-5381 (Print) IS - 1708-5381 (Linking) VI - 22 IP - 6 DP - 2014 Dec TI - Hypothenar hammer syndrome and basilic bypass. PG - 448-9 LID - 10.1177/1708538113516323 [doi] AB - We report a case of hypothenar hammer syndrome. The case presents necessary diagnostic measures and discusses the etiology of this syndrome. Additionally, the case reviews treatments, which culminated in the eventual use of ulnar artery bypass with autogenous basilica vein to treat and resolve the ischemic fingers of the patient. CI - © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Chander, R K AU - Chander RK AD - Department of Vascular Surgery, University of Maryland, Baltimore, MD, USA rchander@smail.umaryland.edu. FAU - Phair, J AU - Phair J AD - Albert Einstein College of Medicine, Bronx, NY, USA. FAU - Oza, P AU - Oza P AD - Department of Surgery, Montefiore Medical Center, Bronx, NY, USA. FAU - Patel, M AU - Patel M AD - Department of Surgery, Montefiore Medical Center, Bronx, NY, USA. FAU - Balar, N AU - Balar N AD - Department of Surgery, Montefiore Medical Center, Bronx, NY, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20131217 PL - England TA - Vascular JT - Vascular JID - 101196722 SB - IM MH - Aged MH - Arteries/surgery MH - Blood Vessel Prosthesis Implantation MH - Dilatation, Pathologic MH - Fingers/*blood supply/*pathology MH - Gangrene MH - Humans MH - Male MH - Peripheral Arterial Disease/complications/*surgery MH - Raynaud Disease/*etiology MH - Skin Ulcer/*pathology MH - Syndrome MH - Thrombosis/complications/surgery MH - Veins/surgery/transplantation OTO - NOTNLM OT - Acute blue finger syndrome OT - hypothenar hammer syndrome OT - water hammer pulse EDAT- 2013/12/19 06:00 MHDA- 2015/01/09 06:00 CRDT- 2013/12/19 06:00 PHST- 2013/12/19 06:00 [entrez] PHST- 2013/12/19 06:00 [pubmed] PHST- 2015/01/09 06:00 [medline] AID - 1708538113516323 [pii] AID - 10.1177/1708538113516323 [doi] PST - ppublish SO - Vascular. 2014 Dec;22(6):448-9. doi: 10.1177/1708538113516323. Epub 2013 Dec 17. PMID- 21238823 OWN - NLM STAT- MEDLINE DCOM- 20110223 LR - 20220331 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 64 IP - 2 DP - 2011 Feb TI - Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. PG - 217-28; quiz 229-30 LID - 10.1016/j.jaad.2010.05.045 [doi] AB - Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues. Morphea is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes. Patients with morphea commonly have systemic symptoms, such as malaise, fatigue, arthralgias, and myalgias, as well as positive autoantibody serologies. However, involvement of morphea is almost uniformly limited to those tissues derived from the mesoderm. The underlying pathogenesis of morphea is incompletely understood at this time, but ultimately results in an imbalance of collagen production and destruction. CI - Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. FAU - Fett, Nicole AU - Fett N AD - Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. Nicole.Fett@uphs.upenn.edu FAU - Werth, Victoria P AU - Werth VP LA - eng GR - K24-AR 02207/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Review PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 9007-34-5 (Collagen) SB - IM MH - Adolescent MH - Adult MH - Autoimmunity MH - Child MH - Child, Preschool MH - Chimerism MH - Collagen/metabolism MH - Female MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Raynaud Disease/diagnosis MH - *Scleroderma, Localized/epidemiology/pathology MH - Scleroderma, Systemic/diagnosis EDAT- 2011/01/18 06:00 MHDA- 2011/02/24 06:00 CRDT- 2011/01/18 06:00 PHST- 2010/01/22 00:00 [received] PHST- 2010/05/17 00:00 [revised] PHST- 2010/05/25 00:00 [accepted] PHST- 2011/01/18 06:00 [entrez] PHST- 2011/01/18 06:00 [pubmed] PHST- 2011/02/24 06:00 [medline] AID - S0190-9622(10)00745-0 [pii] AID - 10.1016/j.jaad.2010.05.045 [doi] PST - ppublish SO - J Am Acad Dermatol. 2011 Feb;64(2):217-28; quiz 229-30. doi: 10.1016/j.jaad.2010.05.045. PMID- 38134088 OWN - NLM STAT- MEDLINE DCOM- 20231225 LR - 20250503 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 102 IP - 51 DP - 2023 Dec 22 TI - Risk factors of systemic lupus erythematosus patients with pulmonary arterial hypertension: A systematic review and meta-analysis. PG - e36654 LID - 10.1097/MD.0000000000036654 [doi] LID - e36654 AB - BACKGROUND: To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE). METHODS: The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI. RESULTS: A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05]. CONCLUSION: Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE. CI - Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Lun, Xueping AU - Lun X AD - Ji'nan Zhangqiu District Hospital of Traditional Chinese Medicine, Jinan City, China. FAU - Yang, Jianguo AU - Yang J AD - First Clinical Medical College, Shandong university of traditional Chinese medicine, Jinan City, China. FAU - Liu, Ying AU - Liu Y AD - Rheumatology and immunology Department, The affiliated hospital of Shandong university of traditional Chinese medicine, Jinan City, China. FAU - Zhao, Fuyu AU - Zhao F AD - Guanghua Clinical Medical College, Shanghai University of Traditional Chinese Medicine, Shanghai, China. AD - Shanghai University of Traditional Chinese Medicine, Shanghai, China. FAU - Wei, Zhiliang AU - Wei Z AD - Radiological department, Shanghe county hospital of traditional Chinese medicine, Jinan City, China. FAU - Sun, Yuying AU - Sun Y AD - First Clinical Medical College, Shandong university of traditional Chinese medicine, Jinan City, China. FAU - Zhou, Xinpeng AU - Zhou X AD - Rheumatology and immunology Department, The affiliated hospital of Shandong university of traditional Chinese medicine, Jinan City, China. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Humans MH - *Pulmonary Arterial Hypertension/etiology/complications MH - Cohort Studies MH - *Hypertension, Pulmonary/epidemiology/etiology/diagnosis MH - *Serositis/complications MH - *Pericardial Effusion MH - Rheumatoid Factor MH - *Lupus Erythematosus, Systemic/complications/epidemiology/diagnosis MH - Familial Primary Pulmonary Hypertension/complications MH - Risk Factors MH - *Raynaud Disease/complications/epidemiology MH - *Vasculitis/complications PMC - PMC10735116 COIS- The authors have no conflicts of interest to disclose. EDAT- 2023/12/22 19:43 MHDA- 2023/12/25 06:42 PMCR- 2023/12/22 CRDT- 2023/12/22 13:43 PHST- 2023/12/25 06:42 [medline] PHST- 2023/12/22 19:43 [pubmed] PHST- 2023/12/22 13:43 [entrez] PHST- 2023/12/22 00:00 [pmc-release] AID - 00005792-202312220-00037 [pii] AID - 10.1097/MD.0000000000036654 [doi] PST - ppublish SO - Medicine (Baltimore). 2023 Dec 22;102(51):e36654. doi: 10.1097/MD.0000000000036654. PMID- 38334058 OWN - NLM STAT- MEDLINE DCOM- 20240412 LR - 20240429 IS - 1477-0377 (Electronic) IS - 1358-863X (Print) IS - 1358-863X (Linking) VI - 29 IP - 2 DP - 2024 Apr TI - Capillaroscopic differences between primary Raynaud phenomenon and healthy controls indicate potential microangiopathic involvement in benign vasospasms. PG - 200-207 LID - 10.1177/1358863X231223523 [doi] AB - BACKGROUND: For primary Raynaud phenomenon (PRP), an otherwise unexplained vasospastic disposition is assumed. To test the hypothesis of an additional involvement of distinct ultrastructural microvascular alterations, we compared the nailfold capillary pattern of patients with PRP and healthy controls. METHODS: A total of 120 patients with PRP (with a median duration of vasospastic symptoms of 60 [IQR: 3-120] months) were compared against 125 controls. In both groups, nailfold capillaroscopy was performed to record the presence of dilatations, capillary edema, tortuous capillaries, ramifications, hemorrhages, and reduced capillary density and to determine a semiquantitative rating score. Further, the capacity of finger skin rewarming was investigated by performing infrared thermography in combination with cold provocation. RESULTS: Unspecific morphologic alterations were found in both, PRP, such as controls, whereby the risk for PRP was four times as high in the presence of capillary dilations (CI: 2.3-7.6) and five times as high if capillary density was reduced (CI: 1.9-13.5). Capillary density correlated with thermoregulatory capacity in both hands in the PRP group, but not in controls. In addition, a negative correlation between the microangiopathy score and the percentage degree of rewarming in both hands was found for patients with PRP only. CONCLUSION: We found specific differences within the microvascular architecture between patients with PRP and controls. As a conclusion, PRP may not be an entirely benign vasospastic phenomenon, but might be associated with subtle microcirculatory vasculopathy. In addition, we suggest that the implementation of a scoring system might serve as guidance in the diagnostic process at least of patients with long-standing PRP. FAU - Brunner-Ziegler, Sophie AU - Brunner-Ziegler S AUID- ORCID: 0000-0001-7825-1254 AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. AD - Current: Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria. FAU - Dassler, Eva AU - Dassler E AUID- ORCID: 0000-0002-5422-3889 AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Müller, Markus AU - Müller M AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Pratscher, Marco AU - Pratscher M AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Forstner, Nikolaus Franz-Ferdinand Maria AU - Forstner NFM AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Koppensteiner, Renate AU - Koppensteiner R AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Schlager, Oliver AU - Schlager O AD - Department of Internal Medicine II, Division of Angiology, Medical University of Vienna, Vienna, Austria. FAU - Jilma, Bernd AU - Jilma B AD - Current: Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria. LA - eng PT - Journal Article DEP - 20240209 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 SB - IM MH - Humans MH - Microscopic Angioscopy MH - Capillaries MH - Microcirculation MH - *Raynaud Disease/diagnosis MH - *Vascular Diseases PMC - PMC11010550 OTO - NOTNLM OT - Raynaud phenomenon OT - microangiopathy OT - nailfold capillary microscopy OT - vasospasm COIS- Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2024/02/09 06:43 MHDA- 2024/04/12 06:44 PMCR- 2024/04/12 CRDT- 2024/02/09 05:53 PHST- 2024/04/12 06:44 [medline] PHST- 2024/02/09 06:43 [pubmed] PHST- 2024/02/09 05:53 [entrez] PHST- 2024/04/12 00:00 [pmc-release] AID - 10.1177_1358863X231223523 [pii] AID - 10.1177/1358863X231223523 [doi] PST - ppublish SO - Vasc Med. 2024 Apr;29(2):200-207. doi: 10.1177/1358863X231223523. Epub 2024 Feb 9. PMID- 17954957 OWN - NLM STAT- MEDLINE DCOM- 20080108 LR - 20131121 IS - 0300-2977 (Print) IS - 0300-2977 (Linking) VI - 65 IP - 9 DP - 2007 Oct TI - Oxybutynin for hyperhidrosis. PG - 356 FAU - Lefrandt, J D AU - Lefrandt JD FAU - Maurer, J M AU - Maurer JM LA - eng PT - Comment PT - Letter PL - Netherlands TA - Neth J Med JT - The Netherlands journal of medicine JID - 0356133 RN - 0 (Mandelic Acids) RN - K9P6MC7092 (oxybutynin) RN - V92SO9WP2I (Glycopyrrolate) SB - IM CON - Neth J Med. 2006 Oct;64(9):326-8. PMID: 17057269 MH - Adolescent MH - Adult MH - Depression/complications MH - Female MH - Glycopyrrolate/administration & dosage/*therapeutic use MH - Humans MH - Hyperhidrosis/*drug therapy/etiology MH - Male MH - Mandelic Acids/administration & dosage/adverse effects/*therapeutic use MH - Raynaud Disease MH - Sympathectomy/*adverse effects MH - Thoracic Nerves/surgery EDAT- 2007/10/24 09:00 MHDA- 2008/01/09 09:00 CRDT- 2007/10/24 09:00 PHST- 2007/10/24 09:00 [pubmed] PHST- 2008/01/09 09:00 [medline] PHST- 2007/10/24 09:00 [entrez] PST - ppublish SO - Neth J Med. 2007 Oct;65(9):356. PMID- 23237785 OWN - NLM STAT- MEDLINE DCOM- 20130802 LR - 20161209 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 42 IP - 5 DP - 2013 May TI - Telangiectasic mastocytosis with systemic sclerosis. PG - 902-4 LID - S0755-4982(12)00615-X [pii] LID - 10.1016/j.lpm.2012.10.014 [doi] FAU - Frigui, Makram AU - Frigui M FAU - Dupin, Nicolas AU - Dupin N FAU - Carlotti, Agnès AU - Carlotti A FAU - Bussone, Guillaume AU - Bussone G FAU - Pestre, Vincent AU - Pestre V FAU - Charles, Pierre AU - Charles P FAU - Crabol, Yoann AU - Crabol Y FAU - Bérezné, Alice AU - Bérezné A FAU - Guillevin, Loïc AU - Guillevin L FAU - Mouthon, Luc AU - Mouthon L LA - eng PT - Case Reports PT - Letter DEP - 20121211 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Antibodies, Antinuclear) RN - 0 (Histamine Antagonists) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Female MH - Histamine Antagonists/therapeutic use MH - Humans MH - Male MH - Mast Cells/ultrastructure MH - Mastocytosis, Cutaneous/diagnosis/drug therapy/*etiology/pathology MH - Middle Aged MH - Pruritus/etiology MH - Raynaud Disease/etiology MH - Scleroderma, Diffuse/blood/*complications/diagnosis MH - Skin/pathology MH - Telangiectasis/*etiology/pathology EDAT- 2012/12/15 06:00 MHDA- 2013/08/03 06:00 CRDT- 2012/12/15 06:00 PHST- 2012/05/21 00:00 [received] PHST- 2012/10/10 00:00 [revised] PHST- 2012/10/12 00:00 [accepted] PHST- 2012/12/15 06:00 [entrez] PHST- 2012/12/15 06:00 [pubmed] PHST- 2013/08/03 06:00 [medline] AID - S0755-4982(12)00615-X [pii] AID - 10.1016/j.lpm.2012.10.014 [doi] PST - ppublish SO - Presse Med. 2013 May;42(5):902-4. doi: 10.1016/j.lpm.2012.10.014. Epub 2012 Dec 11. PMID- 19337287 OWN - NLM STAT- MEDLINE DCOM- 20090717 LR - 20211020 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 5 IP - 4 DP - 2009 Apr TI - Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis. PG - 225-8 LID - 10.1038/nrrheum.2009.30 [doi] AB - BACKGROUND: A 36-year-old woman developed new-onset Raynaud phenomenon and rapidly progressive dyspnea over a 2-week period. A lung biopsy demonstrated pauci-inflammatory nonspecific pneumonitis, which proved refractory to systemic corticosteroid and intravenous cyclophosphamide therapy. Her preterminal course in an intensive care unit was typified by sequential organ failure. Postmortem examination showed extensive organ fibrosis, including severe diffuse alveolar damage and parenchymal fibrosis, and a notable lack of potentially treatable tissue inflammation. INVESTIGATIONS: Chest radiography, physical examination, screening for autoantibodies, measurement of serum creatinine, creatine phosphokinase, and brain natriuretic peptide levels, cardiac examination, pulmonary function tests, electrocardiography, transthoracic Doppler echocardiography, right heart catheterization, high-resolution thoracic CT, pulmonary ventilation/perfusion scan, lung biopsy. DIAGNOSIS: Interstitial lung disease associated with diffuse systemic sclerosis. MANAGEMENT: Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide. FAU - Phillips, Kristine AU - Phillips K AD - University of Michigan, Ann Arbor, MI 48103, USA. FAU - Byrne-Dugan, Cathryn AU - Byrne-Dugan C FAU - Batterson, Eric AU - Batterson E FAU - Seibold, James R AU - Seibold JR LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - Adult MH - Disease Progression MH - Fatal Outcome MH - Female MH - Humans MH - Lung Diseases, Interstitial/diagnosis/*etiology/therapy MH - Raynaud Disease/etiology MH - Scleroderma, Diffuse/*complications/diagnosis/therapy EDAT- 2009/04/02 09:00 MHDA- 2009/07/18 09:00 CRDT- 2009/04/02 09:00 PHST- 2009/04/02 09:00 [entrez] PHST- 2009/04/02 09:00 [pubmed] PHST- 2009/07/18 09:00 [medline] AID - nrrheum.2009.30 [pii] AID - 10.1038/nrrheum.2009.30 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2009 Apr;5(4):225-8. doi: 10.1038/nrrheum.2009.30. PMID- 33497033 OWN - NLM STAT- MEDLINE DCOM- 20210812 LR - 20210812 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 73 IP - 6 DP - 2021 Jun TI - Lindsay's Nails in Early Limited Cutaneous Systemic Sclerosis With Severe Digital Vasculopathy. PG - 1004 LID - 10.1002/art.41658 [doi] FAU - Ellis, Jessica C AU - Ellis JC AUID- ORCID: 0000-0002-9343-809X AD - Royal National Hospital for Rheumatic Diseases. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Bath NHS Foundation Trust and University of Bath, Bath, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20210408 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Acro-Osteolysis/*diagnostic imaging MH - Antibodies, Antinuclear/immunology MH - Finger Phalanges/diagnostic imaging MH - Humans MH - Hyperpigmentation/pathology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nail Diseases/diagnostic imaging/*pathology MH - Raynaud Disease/*pathology MH - Scleroderma, Limited/diagnostic imaging/immunology/*pathology MH - Thermography EDAT- 2021/01/27 06:00 MHDA- 2021/08/13 06:00 CRDT- 2021/01/26 12:13 PHST- 2021/01/05 00:00 [revised] PHST- 2020/12/08 00:00 [received] PHST- 2021/01/05 00:00 [accepted] PHST- 2021/01/27 06:00 [pubmed] PHST- 2021/08/13 06:00 [medline] PHST- 2021/01/26 12:13 [entrez] AID - 10.1002/art.41658 [doi] PST - ppublish SO - Arthritis Rheumatol. 2021 Jun;73(6):1004. doi: 10.1002/art.41658. Epub 2021 Apr 8. PMID- 25882676 OWN - NLM STAT- MEDLINE DCOM- 20170412 LR - 20170412 IS - 1578-2190 (Electronic) IS - 0001-7310 (Linking) VI - 106 IP - 7 DP - 2015 Sep TI - Paraneoplastic Acral Vascular Syndrome. PG - 601-2 LID - S0001-7310(15)00124-6 [pii] LID - 10.1016/j.ad.2014.12.019 [doi] FAU - Rodríguez Martín, A M AU - Rodríguez Martín AM AD - Servicio de Dermatología, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, España. Electronic address: rodriguezmartinanamaria@gmail.com. FAU - Guirao Arrabal, E AU - Guirao Arrabal E AD - Servicio de Medicina Interna, Hospital Universitario Reina Sofía de Córdoba, Córdoba, España. FAU - Jiménez Puya, R AU - Jiménez Puya R AD - Servicio de Dermatología, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, España. FAU - Vélez García-Nieto, A AU - Vélez García-Nieto A AD - Servicio de Dermatología, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, España. LA - eng LA - spa PT - Case Reports PT - Letter DEP - 20150414 PL - Spain TA - Actas Dermosifiliogr JT - Actas dermo-sifiliograficas JID - 0373062 SB - IM MH - Alcoholism/complications MH - Arterial Occlusive Diseases/diagnostic imaging/*etiology MH - Carcinoma, Large Cell/*complications/diagnosis/surgery MH - Hand/blood supply MH - Humans MH - Lung Neoplasms/*complications/diagnosis/surgery MH - Male MH - Middle Aged MH - Paraneoplastic Syndromes/*etiology MH - Pneumonectomy MH - Raynaud Disease/*etiology MH - Remission Induction MH - Smoking/adverse effects EDAT- 2015/04/18 06:00 MHDA- 2017/04/13 06:00 CRDT- 2015/04/18 06:00 PHST- 2014/08/01 00:00 [received] PHST- 2014/12/27 00:00 [revised] PHST- 2014/12/30 00:00 [accepted] PHST- 2015/04/18 06:00 [entrez] PHST- 2015/04/18 06:00 [pubmed] PHST- 2017/04/13 06:00 [medline] AID - S0001-7310(15)00124-6 [pii] AID - 10.1016/j.ad.2014.12.019 [doi] PST - ppublish SO - Actas Dermosifiliogr. 2015 Sep;106(7):601-2. doi: 10.1016/j.ad.2014.12.019. Epub 2015 Apr 14. PMID- 30644670 OWN - NLM STAT- MEDLINE DCOM- 20190425 LR - 20190425 IS - 0807-7096 (Electronic) IS - 0029-2001 (Linking) VI - 138 IP - 1 DP - 2019 Jan 15 TI - A young woman with white fingers and dyspnoea. LID - 10.4045/tidsskr.18.0247 [doi] FAU - Frich, Lars AU - Frich L FAU - Bjørkedal, Bjørn Tore AU - Bjørkedal BT FAU - Gagama, Ragnhild AU - Gagama R FAU - Andersson, Helena AU - Andersson H LA - eng LA - nor PT - Case Reports PT - Journal Article TT - En ung kvinne med hvite fingre og kortpustethet. DEP - 20181220 PL - Norway TA - Tidsskr Nor Laegeforen JT - Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke JID - 0413423 SB - IM MH - Adult MH - Dyspnea/etiology MH - Female MH - Humans MH - *Lymphatic Metastasis/diagnosis/diagnostic imaging MH - *Melanoma/complications/diagnosis/diagnostic imaging/surgery MH - Microscopic Angioscopy MH - Neoplasm Staging MH - *Neoplasms, Unknown Primary/complications/diagnosis/diagnostic imaging/surgery MH - Positron Emission Tomography Computed Tomography MH - Raynaud Disease/etiology MH - Tomography, X-Ray Computed EDAT- 2019/01/16 06:00 MHDA- 2019/04/26 06:00 CRDT- 2019/01/16 06:00 PHST- 2019/01/16 06:00 [pubmed] PHST- 2019/04/26 06:00 [medline] PHST- 2019/01/16 06:00 [entrez] AID - 18-0247 [pii] AID - 10.4045/tidsskr.18.0247 [doi] PST - epublish SO - Tidsskr Nor Laegeforen. 2018 Dec 20;138(1). doi: 10.4045/tidsskr.18.0247. Print 2019 Jan 15. PMID- 20828893 OWN - NLM STAT- MEDLINE DCOM- 20110809 LR - 20161018 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 31 IP - 12 DP - 2010 Dec TI - [Bullous lesions]. PG - 863-4 LID - 10.1016/j.revmed.2010.07.014 [doi] FAU - Elqatni, M AU - Elqatni M AD - Service de médecine interne B, hôpital militaire d'instruction Mohammed V, Rabat, Maroc. elqatni@yahoo.fr FAU - Fatihi, J AU - Fatihi J FAU - Sekkach, Y AU - Sekkach Y FAU - Hammi, S AU - Hammi S FAU - Ameziane, T AU - Ameziane T FAU - Abouzahir, A AU - Abouzahir A FAU - Ghafir, D AU - Ghafir D FAU - Ohayon, V AU - Ohayon V LA - fre PT - Case Reports PT - Journal Article TT - Des lésions bulleuses. DEP - 20100909 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Antimalarials) RN - 0 (Glucocorticoids) SB - IM MH - Adult MH - Antimalarials/therapeutic use MH - Arthralgia/pathology MH - Blister/etiology/*pathology MH - Diagnosis, Differential MH - Drug Therapy, Combination MH - Glucocorticoids/therapeutic use MH - Humans MH - Lupus Erythematosus, Systemic/complications/diagnosis/drug therapy/*pathology MH - Male MH - Mouth Mucosa/pathology MH - Neck/pathology MH - Raynaud Disease/pathology MH - Thorax/pathology MH - Treatment Outcome MH - Upper Extremity/pathology EDAT- 2010/09/11 06:00 MHDA- 2011/08/10 06:00 CRDT- 2010/09/11 06:00 PHST- 2010/05/23 00:00 [received] PHST- 2010/07/17 00:00 [accepted] PHST- 2010/09/11 06:00 [entrez] PHST- 2010/09/11 06:00 [pubmed] PHST- 2011/08/10 06:00 [medline] AID - S0248-8663(10)00868-4 [pii] AID - 10.1016/j.revmed.2010.07.014 [doi] PST - ppublish SO - Rev Med Interne. 2010 Dec;31(12):863-4. doi: 10.1016/j.revmed.2010.07.014. Epub 2010 Sep 9. PMID- 19643651 OWN - NLM STAT- MEDLINE DCOM- 20100108 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 76 IP - 5 DP - 2009 Oct TI - Intravenous immune globulins to treat eosinophilic fasciitis: a case report. PG - 572-4 LID - 10.1016/j.jbspin.2009.06.001 [doi] FAU - Pimenta, Sofia AU - Pimenta S FAU - Bernardes, Miguel AU - Bernardes M FAU - Bernardo, Alexandra AU - Bernardo A FAU - Brito, Iva AU - Brito I FAU - Castro, Lígia AU - Castro L FAU - Simões-Ventura, Francisco AU - Simões-Ventura F LA - eng PT - Case Reports PT - Letter DEP - 20090729 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunologic Factors) RN - 0 (Immunosuppressive Agents) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adult MH - Arm MH - Electromyography MH - Eosinophilia/*drug therapy MH - Foot MH - Hand MH - Humans MH - Immunoglobulins, Intravenous/*therapeutic use MH - Immunologic Factors/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Inflammation MH - Joint Diseases/drug therapy/physiopathology MH - Male MH - Methotrexate/therapeutic use MH - Muscle, Skeletal/pathology/physiopathology MH - Muscular Diseases/drug therapy/physiopathology MH - Myositis/diagnosis/drug therapy/pathology MH - Raynaud Disease/diagnosis EDAT- 2009/08/01 09:00 MHDA- 2010/01/09 06:00 CRDT- 2009/08/01 09:00 PHST- 2009/01/21 00:00 [received] PHST- 2009/01/21 00:00 [accepted] PHST- 2009/08/01 09:00 [entrez] PHST- 2009/08/01 09:00 [pubmed] PHST- 2010/01/09 06:00 [medline] AID - S1297-319X(09)00109-2 [pii] AID - 10.1016/j.jbspin.2009.06.001 [doi] PST - ppublish SO - Joint Bone Spine. 2009 Oct;76(5):572-4. doi: 10.1016/j.jbspin.2009.06.001. Epub 2009 Jul 29. PMID- 38556712 OWN - NLM STAT- MEDLINE DCOM- 20241119 LR - 20260210 IS - 1754-4505 (Electronic) IS - 0275-1879 (Linking) VI - 44 IP - 5 DP - 2024 Sep TI - CREST syndrome diagnosed by oral lesions: A case report and review of the literature. PG - 1368-1376 LID - 10.1111/scd.12998 [doi] AB - BACKGROUND: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is an acronym for the clinical features that are seen. Its etiology is unknown, affecting women three times more than men. CREST syndrome is often diagnosed by systemic symptoms. However, oral manifestations could be helpful in the early diagnosis of the disease. Less than 20 cases of CREST syndrome with oral symptoms were described. OBJECTIVE: To report a case of a 26-year-old female, who was diagnosed with CREST syndrome based on findings of the oral mucosa. CASE REPORT: Clinical examination revealed sclerodactyly and nail alterations. Oral findings were associated with hypochromic and paleness mucosa. The tongue was strongly rigid and reddish areas compatible with telangiectasias were found. The mouth opening was particularly compromised. Histological findings were suggestive of systemic sclerosis in the context of CREST syndrome. Considering the available diagnosis criteria, this case was diagnosed as CREST syndrome based on oral manifestations. CONCLUSIONS: Oral manifestations of CREST syndrome could help in early clinical diagnosis, preventing a delay in the onset of an aggressive form of the disease. Accurate referrals of patients to specialists are needed for a multidisciplinary approach. CI - © 2024 Special Care Dentistry Association and Wiley Periodicals LLC. FAU - Gilligan, Gerardo AU - Gilligan G AUID- ORCID: 0000-0002-5201-1444 AD - Oral Medicine Department, Facultad de Odontología, Universidad Nacional de Córdoba, Cordoba, Argentina. FAU - Leonardi, Nicolás AU - Leonardi N AD - Oral Medicine Department, School of Dentistry, Health Sciences Faculty, Universidad Católica de Córdoba, Córdoba, Argentina. FAU - Sambuelli, Gabriela AU - Sambuelli G AD - General Pathology Department, Clínica Universitaria Reyna Fabiola, Córdoba, Argentina. FAU - Panico, René AU - Panico R AD - Oral Medicine Department, Facultad de Odontología, Universidad Nacional de Córdoba, Cordoba, Argentina. AD - Oral Medicine Department, School of Dentistry, Health Sciences Faculty, Universidad Católica de Córdoba, Córdoba, Argentina. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20240331 PL - United States TA - Spec Care Dentist JT - Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry JID - 8103755 SB - IM MH - Humans MH - *CREST Syndrome/complications MH - Adult MH - Female MH - Mouth Diseases/diagnosis/pathology MH - Diagnosis, Differential EDAT- 2024/04/01 06:42 MHDA- 2024/11/19 13:17 CRDT- 2024/04/01 00:32 PHST- 2024/02/16 00:00 [revised] PHST- 2024/01/31 00:00 [received] PHST- 2024/03/14 00:00 [accepted] PHST- 2024/11/19 13:17 [medline] PHST- 2024/04/01 06:42 [pubmed] PHST- 2024/04/01 00:32 [entrez] AID - 10.1111/scd.12998 [doi] PST - ppublish SO - Spec Care Dentist. 2024 Sep;44(5):1368-1376. doi: 10.1111/scd.12998. Epub 2024 Mar 31. PMID- 30806445 OWN - NLM STAT- MEDLINE DCOM- 20200513 LR - 20200513 IS - 2175-8239 (Electronic) IS - 0101-2800 (Print) IS - 0101-2800 (Linking) VI - 41 IP - 4 DP - 2019 Oct-Dec TI - Endothelial lesion and complement activation in patients with Scleroderma Renal Crisis. PG - 580-584 LID - 10.1590/2175-8239-JBN-2018-0202 [doi] AB - In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation. FAU - Pérez, Ney Arencibia AU - Pérez NA AUID- ORCID: 0000-0002-8878-8263 AD - Reina Sofia University Hospital, Nephrology, Av. Menendez Pidal, s/n Córdoba, Spain. FAU - Morales, María Luisa Agüera AU - Morales MLA AD - Reina Sofia University Hospital, Nephrology, Av. Menendez Pidal, s/n Córdoba, Spain. FAU - Sánchez, Rafael Sánchez AU - Sánchez RS AD - Reina Sofia University Hospital, Pathological Anatomy, Cordoba, Spain. FAU - Salas, Rosa María Ortega AU - Salas RMO AD - Reina Sofia University Hospital, Pathological Anatomy, Cordoba, Spain. FAU - Puebla, Rafael Ángel Fernández de la AU - Puebla RÁF AD - Reina Sofia University Hospital, Internal Medicine, Cordoba, Spain. FAU - Hernández, Mario Espinosa AU - Hernández ME AD - Reina Sofia University Hospital, Nephrology, Av. Menendez Pidal, s/n Córdoba, Spain. LA - eng LA - por PT - Case Reports PT - Journal Article PL - Brazil TA - J Bras Nefrol JT - Jornal brasileiro de nefrologia JID - 9426946 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) SB - IM MH - Acute Kidney Injury/diagnosis/*etiology MH - Anemia, Hemolytic/diagnosis/etiology MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Capillaries/metabolism MH - Dermatomyositis/complications/immunology MH - Humans MH - Hypertensive Retinopathy/diagnosis/drug therapy/pathology MH - Immunohistochemistry MH - Kidney/*blood supply/diagnostic imaging/pathology MH - Male MH - Middle Aged MH - Papilledema/pathology MH - Raynaud Disease/*complications/pathology MH - Scleroderma, Systemic/complications/immunology MH - Treatment Outcome MH - Vision Disorders/diagnosis/*etiology PMC - PMC6979577 EDAT- 2019/02/27 06:00 MHDA- 2020/05/14 06:00 PMCR- 2019/10/01 CRDT- 2019/02/27 06:00 PHST- 2018/10/04 00:00 [received] PHST- 2018/11/13 00:00 [accepted] PHST- 2019/02/27 06:00 [pubmed] PHST- 2020/05/14 06:00 [medline] PHST- 2019/02/27 06:00 [entrez] PHST- 2019/10/01 00:00 [pmc-release] AID - S0101-28002019005009101 [pii] AID - 10.1590/2175-8239-JBN-2018-0202 [doi] PST - ppublish SO - J Bras Nefrol. 2019 Oct-Dec;41(4):580-584. doi: 10.1590/2175-8239-JBN-2018-0202. PMID- 25749623 OWN - NLM STAT- MEDLINE DCOM- 20151002 LR - 20250529 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 54 IP - 8 DP - 2015 Aug TI - A study comparing videocapillaroscopy and dermoscopy in the assessment of nailfold capillaries in patients with systemic sclerosis-spectrum disorders. PG - 1435-42 LID - 10.1093/rheumatology/keu533 [doi] AB - OBJECTIVES: Nailfold videocapillaroscopy (NVC), the current gold standard for detection of capillary abnormalities suggestive of an SSc-spectrum disorder, is not widely available: a key question is whether lower-magnification, easy-to-use dermoscopy compares favourably. This is especially relevant given the inclusion of capillaroscopic abnormality within the 2013 classification criteria for SSc. Our objectives were to examine the ability to classify capillaries and to evaluate abnormality (severity), by both NVC and dermoscopy, to determine whether these differ between general and specialist rheumatologists, and to compare intra- and interrater reliability of both techniques. METHODS: NVC and dermoscopy images were acquired from all 10 nailbeds of 32 subjects with a range of capillary abnormalities. Images were graded (using a web-based interface) on a 0-3 scale of severity: normal (0), mildly (1), definitely (2) and grossly abnormal (3), and an unclassifiable category. Raters graded images from four subjects (40 nailbeds) using each technique, with five repeated images to estimate intrarater reliability. RESULTS: Forty-eight rheumatologists from 12 countries participated in the study (22 generalists, 26 specialists). While most images could be graded by both techniques, more were graded by NVC (84% vs 70%) and were systematically scored higher by NVC (mean difference 0.43 between the ratings). Agreement between the techniques was moderate. Intra- and interrater reliability were comparable for the two techniques in the classifiability of images and the grading of severity. CONCLUSION: Our results suggest that dermoscopy is comparable to NVC, although NVC images were more likely to be classifiable and were graded more severely. CI - © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and michael.hughes-6@postgrad.manchester.ac.uk. FAU - Moore, Tonia AU - Moore T AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. FAU - O'Leary, Neil AU - O'Leary N AD - Centre for Biostatistics, Institute of Population Health, School of Medicine, The University of Manchester, Manchester, UK. FAU - Tracey, Andrew AU - Tracey A AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. FAU - Ennis, Holly AU - Ennis H AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. FAU - Dinsdale, Graham AU - Dinsdale G AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. FAU - Murray, Andrea AU - Murray A AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. FAU - Roberts, Christopher AU - Roberts C AD - Centre for Biostatistics, Institute of Population Health, School of Medicine, The University of Manchester, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre and. LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom PT - Comparative Study PT - Evaluation Study PT - Journal Article DEP - 20150306 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Capillaries/*pathology MH - Case-Control Studies MH - Dermoscopy/*methods MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Observer Variation MH - Raynaud Disease/diagnosis/pathology MH - Reproducibility of Results MH - Scleroderma, Systemic/diagnosis/*pathology MH - Severity of Illness Index MH - Video Recording/methods OTO - NOTNLM OT - capillaroscopy OT - dermoscopy OT - scleroderma OT - systemic sclerosis OT - videocapillaroscopy EDAT- 2015/03/10 06:00 MHDA- 2015/10/03 06:00 CRDT- 2015/03/10 06:00 PHST- 2014/06/03 00:00 [received] PHST- 2015/03/10 06:00 [entrez] PHST- 2015/03/10 06:00 [pubmed] PHST- 2015/10/03 06:00 [medline] AID - keu533 [pii] AID - 10.1093/rheumatology/keu533 [doi] PST - ppublish SO - Rheumatology (Oxford). 2015 Aug;54(8):1435-42. doi: 10.1093/rheumatology/keu533. Epub 2015 Mar 6. PMID- 21622523 OWN - NLM STAT- MEDLINE DCOM- 20111017 LR - 20151119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 50 IP - 9 DP - 2011 Sep TI - Laser Doppler perfusion imaging in systemic sclerosis impaired response to cold stimulation involves digits and hand dorsum. PG - 1654-8 LID - 10.1093/rheumatology/ker188 [doi] AB - OBJECTIVES: To assess by Laser Doppler perfusion imaging (LDPI) skin blood perfusion of hands in patients with SSc and primary RP (PRP) at baseline and after cold stimulation (CS). In SSc patients, the associations between skin perfusion and nailfold video capillaroscopy (NVC) patterns were also evaluated. METHODS: Forty patients with SSc, 38 patients with PRP and 32 healthy controls were recruited. Skin blood flow of the hands was detected by Lisca Laser Doppler Perfusion Imager at baseline and after CS. Further laser Doppler scanning was performed for each hand at 0 (T(1)), 3 (T(2)), 7 (T(3)) and 15 min (T(4)). RESULTS: Baseline mean perfusion is significantly (P < 0.000 l) lower in SSc patients than in healthy controls. In SSc patients, mean perfusion is reduced after CS (P < 0.0001) and skin flow recovery (significant difference between T(0) and T(4), P < 0.0001) is incomplete. In SSc patients with low vascular damage (early and active capillaroscopic groups), the abnormal microvascular response to CS involves only the digits, while the perfusion of hands dorsum is normal. With the progression of vascular damage (late capillaroscopic groups), the abnormal microvascular response to CS also appears in the hand dorsum skin. In PRP patients, baseline hand perfusion is very low and the skin flow recovery after CS is absent (P < 0.05). CONCLUSION: In early SSc, the thermoregulation of finger skin is impaired, but only in advanced stages of microangiopathy does the skin of the hand dorsum show a vasomotor control failure. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Clinical Medicine, Clinical Immunology Unit - Scleroderma Center, Sapienza University of Rome, Viale dell'Università 37, 00185 Rome, Italy. edoardo.rosato@uniroma1.it FAU - Rossi, Carmelina AU - Rossi C FAU - Molinaro, Ilenia AU - Molinaro I FAU - Giovannetti, Antonello AU - Giovannetti A FAU - Pisarri, Simonetta AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article DEP - 20110526 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Cold Temperature MH - Female MH - Fingers/*blood supply MH - Hand/blood supply MH - Humans MH - Laser-Doppler Flowmetry/methods MH - Male MH - Microcirculation MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/*blood supply MH - Perfusion Imaging/methods MH - Raynaud Disease/complications/*physiopathology MH - Scleroderma, Systemic/complications/*physiopathology MH - Skin/*blood supply MH - Young Adult EDAT- 2011/05/31 06:00 MHDA- 2011/10/18 06:00 CRDT- 2011/05/31 06:00 PHST- 2011/05/31 06:00 [entrez] PHST- 2011/05/31 06:00 [pubmed] PHST- 2011/10/18 06:00 [medline] AID - ker188 [pii] AID - 10.1093/rheumatology/ker188 [doi] PST - ppublish SO - Rheumatology (Oxford). 2011 Sep;50(9):1654-8. doi: 10.1093/rheumatology/ker188. Epub 2011 May 26. PMID- 32238542 OWN - NLM STAT- MEDLINE DCOM- 20200819 LR - 20200819 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 47 IP - 4 DP - 2020 Apr TI - The "Renocentric Theory" of Renal Resistive Index: Is It Time for a Copernican Revolution? PG - 486-489 LID - 10.3899/jrheum.190930 [doi] FAU - Geraci, Giulio AU - Geraci G AUID- ORCID: 0000-0003-3806-1151 AD - Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Unit of Nephrology and Hypertension, European Society of Hypertension Excellence Centre, University of Palermo, Palermo, Italy. giulio.geraci@unipa.it. FAU - Sorce, Alessandra AU - Sorce A AUID- ORCID: 0000-0002-5267-3616 AD - Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Unit of Nephrology and Hypertension, European Society of Hypertension Excellence Centre, University of Palermo, Palermo, Italy. FAU - Mulè, Giuseppe AU - Mulè G AUID- ORCID: 0000-0002-8500-8671 AD - Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Unit of Nephrology and Hypertension, European Society of Hypertension Excellence Centre, University of Palermo, Palermo, Italy. LA - eng PT - Comment PT - Editorial PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CON - J Rheumatol. 2020 Apr;47(4):567-571. doi: 10.3899/jrheum.190165. PMID: 31203218 MH - Hemodynamics MH - Humans MH - Kidney MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2020/04/03 06:00 MHDA- 2020/08/20 06:00 CRDT- 2020/04/03 06:00 PHST- 2020/04/03 06:00 [entrez] PHST- 2020/04/03 06:00 [pubmed] PHST- 2020/08/20 06:00 [medline] AID - 47/4/486 [pii] AID - 10.3899/jrheum.190930 [doi] PST - ppublish SO - J Rheumatol. 2020 Apr;47(4):486-489. doi: 10.3899/jrheum.190930. PMID- 18828905 OWN - NLM STAT- MEDLINE DCOM- 20081112 LR - 20181113 IS - 1477-7819 (Electronic) IS - 1477-7819 (Linking) VI - 6 DP - 2008 Oct 1 TI - Adenocarcinoma of the third portion of the duodenum in a man with CREST syndrome. PG - 106 LID - 10.1186/1477-7819-6-106 [doi] AB - BACKGROUND: CREST (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias) syndrome has been rarely associated with other malignancies (lung, esophagus). This is the first report of a primary adenocarcinoma of the third portion of the duodenum in a patient with CREST syndrome. CASE PRESENTATION: A 54-year-old male patient with CREST syndrome presented with colicky postprandial pain of the upper abdomen, diminished food uptake and a 6-Kg-body weight loss during the previous 2 months. An ulcerative lesion in the third portion of the duodenum was revealed during duodenoscopy, with a diagnosis of adenocarcinoma on biopsy specimen histology. The patient underwent a partial pancreatoduodenectomy. No adjuvant therapy was instituted and follow-up is negative for local recurrence or metastases 21 months postoperatively. CONCLUSION: CREST syndrome has been associated with colon cancer, gastric polyps, familial adenomatous polyposis (FAP) syndrome and Crohn's disease; however, this is the first report of a primary adenocarcinoma of the duodenum in a patient with CREST syndrome. However, any etiologic relationship remains to be further investigated. FAU - Anastasopoulos, Georgios AU - Anastasopoulos G AD - Second Department of Surgery, Areteion University Hospital, Athens, Greece. FAU - Marinis, Athanasios AU - Marinis A FAU - Konstantinidis, Christos AU - Konstantinidis C FAU - Theodosopoulos, Theodosios AU - Theodosopoulos T FAU - Fragulidis, Georgios AU - Fragulidis G FAU - Vassiliou, Ioannis AU - Vassiliou I LA - eng PT - Case Reports PT - Journal Article DEP - 20081001 PL - England TA - World J Surg Oncol JT - World journal of surgical oncology JID - 101170544 SB - IM MH - Adenocarcinoma/*complications/surgery MH - CREST Syndrome/*complications MH - Duodenal Neoplasms/*complications/surgery MH - Humans MH - Male MH - Middle Aged MH - Pancreaticoduodenectomy PMC - PMC2566573 EDAT- 2008/10/03 09:00 MHDA- 2008/11/13 09:00 PMCR- 2008/10/01 CRDT- 2008/10/03 09:00 PHST- 2008/06/03 00:00 [received] PHST- 2008/10/01 00:00 [accepted] PHST- 2008/10/03 09:00 [pubmed] PHST- 2008/11/13 09:00 [medline] PHST- 2008/10/03 09:00 [entrez] PHST- 2008/10/01 00:00 [pmc-release] AID - 1477-7819-6-106 [pii] AID - 10.1186/1477-7819-6-106 [doi] PST - epublish SO - World J Surg Oncol. 2008 Oct 1;6:106. doi: 10.1186/1477-7819-6-106. PMID- 16823697 OWN - NLM STAT- MEDLINE DCOM- 20061018 LR - 20161021 IS - 1641-4640 (Print) IS - 1509-572X (Linking) VI - 44 IP - 2 DP - 2006 TI - Multiple mononeuropathy due to vasculitis associated with anticardiolipin antibodies: a case report. PG - 140-3 AB - This report illustrates a case of peripheral nerve vasculitis associated with elevated anticardiolipin antibodies. A 49-year-old female with a history of seven spontaneous abortions initially complained of pain and numbness in her right calf that later spread to the left foot and ankle. Over the next few months, she developed a Raynaud phenomenon and livedo reticularis. Clinical examination revealed signs of multiple mononeuropathy. Right sural nerve biopsy performed two months after the beginning of the disease revealed active necrotizing arteritis of the epineural arteries with transmural inflammatory infiltrate and thrombosis. Vasculitis is a rare finding in sural nerve biopsies, usually in patients with systemic vasculitis or autoimmune connective tissue diseases. However, vasculitis restricted to the peripheral nerves has also been described. Our patient had no clinical or laboratory features of any autoimmune disorder and also no signs of systemic vasculitis. We discuss the potential role of anticardiolipin antibodies in the pathogenesis of vasculitis. FAU - Jeruc, Jera AU - Jeruc J AD - Institute of Pathology, Faculty of Medicine, Ljubljana, Korytkova 2, SI-1000 Ljubljana, Slovenia. Jera.Jeruc@mf.uni-lj.si FAU - Popovic, Mara AU - Popovic M FAU - Vizjak, Alenka AU - Vizjak A FAU - Jurcić, Vesna AU - Jurcić V FAU - Lestan, Boris AU - Lestan B FAU - Ferluga, Dusan AU - Ferluga D LA - eng PT - Case Reports PT - Journal Article PL - Poland TA - Folia Neuropathol JT - Folia neuropathologica JID - 9437431 RN - 0 (Antibodies, Anticardiolipin) SB - IM MH - Abortion, Spontaneous MH - Antibodies, Anticardiolipin/*blood MH - Antiphospholipid Syndrome/complications MH - Exanthema/etiology MH - Female MH - Humans MH - Immunohistochemistry MH - Middle Aged MH - Mononeuropathies/blood/*etiology/pathology MH - Pain/etiology MH - Pregnancy MH - Raynaud Disease/etiology MH - Skin Diseases, Vascular/etiology MH - Sural Nerve/blood supply/pathology MH - Vasculitis/blood/*complications/pathology EDAT- 2006/07/11 09:00 MHDA- 2006/10/19 09:00 CRDT- 2006/07/11 09:00 PHST- 2006/07/11 09:00 [pubmed] PHST- 2006/10/19 09:00 [medline] PHST- 2006/07/11 09:00 [entrez] AID - 6220 [pii] PST - ppublish SO - Folia Neuropathol. 2006;44(2):140-3. PMID- 19940282 OWN - NLM STAT- MEDLINE DCOM- 20091229 LR - 20220414 IS - 1460-2105 (Electronic) IS - 0027-8874 (Print) IS - 0027-8874 (Linking) VI - 101 IP - 24 DP - 2009 Dec 16 TI - Observational study of prevalence of long-term Raynaud-like phenomena and neurological side effects in testicular cancer survivors. PG - 1682-95 LID - 10.1093/jnci/djp413 [doi] AB - BACKGROUND: Sensory neuropathy (paresthesias), tinnitus, hearing impairment, and Raynaud phenomena are side effects of cisplatin-based chemotherapy used to treat testicular cancer patients. We assessed the long-term occurrence of these side effects among testicular cancer survivors according to the treatment they received. METHODS: A total of 1814 men who were treated for unilateral testicular cancer in Norway during 1980-1994 were invited to participate in a national multicenter follow-up survey conducted during 1998-2002. The men were allocated to six groups according to the treatment they had received. Self-reported symptoms were assessed by a mailed questionnaire that included the Scale for Chemotherapy-Induced Neurotoxicity. A total of 1409 participants who responded to the questionnaire and/or underwent audiometry were assessable in this study. Respondents to the questionnaire (n = 1402) scored the relevant symptoms according to how troubled they were by each (not at all, a little, quite a bit, or very much). Hearing impairment was objectively assessed by audiometry at 4000 Hz in 755 men (seven of whom did not respond to the questionnaire). Group comparisons of symptom assessments were performed with chi2 or Kruskal-Wallis tests. Associations between relevant factors and self-reported symptoms or hearing impairment measured by audiometry were assessed using proportional odds ordinal logistic regression models and linear regression models, respectively. All statistical tests were two-sided. RESULTS: The median follow-up for the 1409 assessable men was 10.7 years (range = 4-21 years). All chemotherapy groups had statistically significantly higher odds for increasing severity of all assessed symptoms and inferior audiometric results compared with men who did not receive chemotherapy. Among chemotherapy-treated men, 39% (95% confidence interval [CI] = 35% to 43%) reported Raynaud-like phenomena (defined as white or cold hands or fingers [or feet or toes] on cold exposure), 29% (95% CI = 25% to 33%) reported paresthesias in the hands or feet, 21% (95% CI = 18% to 25%) reported hearing impairment, and 22% (95% CI = 19% to 26%) reported tinnitus as major symptoms troubling them quite a bit or very much. Hearing impairment (odds ratio [OR] = 5.3, 95% CI = 3.0 to 9.2) and tinnitus (OR = 7.1, 95% CI = 4.1 to 12.4) were particularly common in the dose-intensive chemotherapy group compared with the no chemotherapy group. Men who were treated with radiotherapy had higher odds of self-reported paresthesias in feet compared with those not treated with radiotherapy (OR = 1.5, 95% CI = 1.01 to 2.1, P = .04). CONCLUSION: Long-term survivors of testicular cancer who were treated with cisplatin-based chemotherapy were more often troubled by dose-dependent neurological side effects and Raynaud-like phenomena compared with those who were not treated with chemotherapy. FAU - Brydøy, Marianne AU - Brydøy M AD - Department of Oncology, Haukeland University Hospital, N-5021 Bergen, Norway. marianne.brydoy@helse-bergen.no FAU - Oldenburg, Jan AU - Oldenburg J FAU - Klepp, Olbjørn AU - Klepp O FAU - Bremnes, Roy M AU - Bremnes RM FAU - Wist, Erik A AU - Wist EA FAU - Wentzel-Larsen, Tore AU - Wentzel-Larsen T FAU - Hauge, Erik R AU - Hauge ER FAU - Dahl, Olav AU - Dahl O FAU - Fosså, Sophie D AU - Fosså SD LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - J Natl Cancer Inst JT - Journal of the National Cancer Institute JID - 7503089 RN - Q20Q21Q62J (Cisplatin) SB - IM MH - Adult MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*adverse effects MH - Audiometry MH - Cisplatin/administration & dosage/*adverse effects MH - Cross-Sectional Studies MH - Drug Administration Schedule MH - Follow-Up Studies MH - Hearing Loss/chemically induced/epidemiology MH - Humans MH - Male MH - Middle Aged MH - Norway/epidemiology MH - Odds Ratio MH - Paresthesia/chemically induced/epidemiology MH - Prevalence MH - Raynaud Disease/*chemically induced/*epidemiology MH - Regression Analysis MH - Research Design MH - Seminoma/drug therapy MH - Sensation Disorders/*chemically induced/*epidemiology MH - Severity of Illness Index MH - Surveys and Questionnaires MH - Survivors/*statistics & numerical data MH - Testicular Neoplasms/*drug therapy MH - Tinnitus/chemically induced/epidemiology PMC - PMC2794301 EDAT- 2009/11/27 06:00 MHDA- 2009/12/30 06:00 PMCR- 2009/12/16 CRDT- 2009/11/27 06:00 PHST- 2009/11/27 06:00 [entrez] PHST- 2009/11/27 06:00 [pubmed] PHST- 2009/12/30 06:00 [medline] PHST- 2009/12/16 00:00 [pmc-release] AID - djp413 [pii] AID - 10.1093/jnci/djp413 [doi] PST - ppublish SO - J Natl Cancer Inst. 2009 Dec 16;101(24):1682-95. doi: 10.1093/jnci/djp413. PMID- 25419330 OWN - NLM STAT- MEDLINE DCOM- 20150716 LR - 20181113 IS - 1937-8688 (Electronic) VI - 18 DP - 2014 TI - [Aseptic necrosis of the femoral head in a patient with Takayasu arteritis]. PG - 203 LID - 10.11604/pamj.2014.18.203.4679 [doi] LID - 203 FAU - Loukil, Hanen AU - Loukil H AD - Service de Médecine Interne CHU Hédi Chaker, Sfax, Tunisie. FAU - Frikha, Faten AU - Frikha F AD - Service de Médecine Interne CHU Hédi Chaker, Sfax, Tunisie. FAU - Snoussi, Mouna AU - Snoussi M AD - Service de Médecine Interne CHU Hédi Chaker, Sfax, Tunisie. FAU - Ben Salah, Raida AU - Ben Salah R AD - Service de Médecine Interne CHU Hédi Chaker, Sfax, Tunisie. FAU - Bahloul, Zouhir AU - Bahloul Z AD - Service de Médecine Interne CHU Hédi Chaker, Sfax, Tunisie. LA - fre PT - Case Reports PT - Journal Article TT - Ostéonécrose aseptique de la tête fémorale chez une patiente atteinte d'artérite de Takayasu. DEP - 20140706 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/adverse effects/therapeutic use MH - Aged MH - Coronary Disease/complications MH - Dyslipidemias/complications MH - Female MH - Femur Head Necrosis/chemically induced/diagnostic imaging/*etiology MH - Humans MH - Raynaud Disease/complications MH - Takayasu Arteritis/*complications/drug therapy MH - Tomography, X-Ray Computed PMC - PMC4237577 OTO - NOTNLM OT - Takayasu arteritis OT - aseptic osteonecrosis OT - femoral head EDAT- 2014/11/25 06:00 MHDA- 2015/07/17 06:00 PMCR- 2014/07/06 CRDT- 2014/11/25 06:00 PHST- 2014/05/25 00:00 [received] PHST- 2014/06/23 00:00 [accepted] PHST- 2014/11/25 06:00 [entrez] PHST- 2014/11/25 06:00 [pubmed] PHST- 2015/07/17 06:00 [medline] PHST- 2014/07/06 00:00 [pmc-release] AID - PAMJ-18-203 [pii] AID - 10.11604/pamj.2014.18.203.4679 [doi] PST - epublish SO - Pan Afr Med J. 2014 Jul 6;18:203. doi: 10.11604/pamj.2014.18.203.4679. eCollection 2014. PMID- 24645948 OWN - NLM STAT- MEDLINE DCOM- 20140422 LR - 20161125 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 370 IP - 12 DP - 2014 Mar 20 TI - Case records of the Massachusetts General Hospital. Case 9-2014. A 34-year-old woman with increasing dyspnea. PG - 1149-57 LID - 10.1056/NEJMcpc1305992 [doi] FAU - Saukkonen, Kai AU - Saukkonen K FAU - Tan, Timothy C AU - Tan TC FAU - Sharma, Amita AU - Sharma A FAU - Channick, Richard N AU - Channick RN FAU - Murali, Mandakolathur R AU - Murali MR FAU - Zukerberg, Lawrence R AU - Zukerberg LR LA - eng PT - Case Reports PT - Clinical Conference PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 RN - 0 (Antibodies, Antinuclear) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM EIN - N Engl J Med. 2014 Jun 5;370(23):2253 CIN - N Engl J Med. 2014 Jul 24;371(4):388-9. doi: 10.1056/NEJMc1404715. PMID: 25054729 CIN - N Engl J Med. 2014 Jul 24;371(4):387. doi: 10.1056/NEJMc1404715. PMID: 25054730 CIN - N Engl J Med. 2014 Jul 24;371(4):387-8. doi: 10.1056/NEJMc1404715. PMID: 25054731 MH - Adult MH - Antibodies, Antinuclear/blood MH - Diagnosis, Differential MH - Dyspnea/etiology MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/*etiology MH - Hypertrophy, Left Ventricular/diagnostic imaging MH - Lymph Nodes/*pathology MH - Mixed Connective Tissue Disease/complications/*pathology MH - Pericardial Effusion/diagnostic imaging MH - Radiography, Thoracic MH - Raynaud Disease/complications MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Ultrasonography EDAT- 2014/03/22 06:00 MHDA- 2014/04/23 06:00 CRDT- 2014/03/21 06:00 PHST- 2014/03/21 06:00 [entrez] PHST- 2014/03/22 06:00 [pubmed] PHST- 2014/04/23 06:00 [medline] AID - 10.1056/NEJMcpc1305992 [doi] PST - ppublish SO - N Engl J Med. 2014 Mar 20;370(12):1149-57. doi: 10.1056/NEJMcpc1305992. PMID- 28913747 OWN - NLM STAT- MEDLINE DCOM- 20180606 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 36 IP - 11 DP - 2017 Nov TI - Primary Sjögren's syndrome: Extraglandular manifestations and hydroxychloroquine therapy. PG - 2455-2460 LID - 10.1007/s10067-017-3822-3 [doi] AB - The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded. Demographics and pSS characteristics were recorded. The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients were divided into two groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. A descriptive analysis of demographics and pSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi(2) test or Fisher's exact test. A total of 221 patients were included (97.3% women), mean age, 55.7 years (SD 14). Mean age at diagnosis, 48.8 years (SD 15); median disease duration, 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the sicca syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p < 0.001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p < 0.001), fatigue (p < 0.001), purpura (p = 0.01), Raynaud phenomenon (p = 0.003), and hypergammaglobulinemia (p = 0.006). Immunosuppressive treatment was indicated on 28 patients (12.7%), 13 of which were receiving also HCQ. The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis, and interstitial lung disease. None of the patients received biologic therapy. The lower incidence of EGM was observed in patients on HCQ therapy supports its efficacy in pSS. However, further large scale prospective studies are needed to confirm these findings. FAU - Demarchi, J AU - Demarchi J AUID- ORCID: 0000-0002-6906-2096 AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. juliademarchireum@gmail.com. FAU - Papasidero, S AU - Papasidero S AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. FAU - Medina, M A AU - Medina MA AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. FAU - Klajn, D AU - Klajn D AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. FAU - Chaparro Del Moral, R AU - Chaparro Del Moral R AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. FAU - Rillo, O AU - Rillo O AD - Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina. FAU - Martiré, V AU - Martiré V AD - Hospital B. Rivadavia, Buenos Aires, Argentina. FAU - Crespo, G AU - Crespo G AD - Hospital B. Rivadavia, Buenos Aires, Argentina. FAU - Secco, A AU - Secco A AD - Hospital B. Rivadavia, Buenos Aires, Argentina. FAU - Catalan Pellet, A AU - Catalan Pellet A AD - Hospital B. Rivadavia, Buenos Aires, Argentina. FAU - Amitrano, C AU - Amitrano C AD - Hospital Alemán, Buenos Aires, Argentina. FAU - Crow, C AU - Crow C AD - Hospital Alemán, Buenos Aires, Argentina. FAU - Asnal, C AU - Asnal C AD - Hospital Alemán, Buenos Aires, Argentina. FAU - Pucci, P AU - Pucci P AD - Hospital Alemán, Buenos Aires, Argentina. FAU - Caeiro, F AU - Caeiro F AD - Hospital Privado de Córdoba, Córdoba, Argentina. FAU - Benzanquen, N AU - Benzanquen N AD - Hospital Privado de Córdoba, Córdoba, Argentina. FAU - Pirola, J P AU - Pirola JP AD - Hospital Privado de Córdoba, Córdoba, Argentina. FAU - Mayer, M AU - Mayer M AD - Hospital Británico de Buenos Aires, Buenos Aires, Argentina. FAU - Zazzetti, F AU - Zazzetti F AD - Hospital Británico de Buenos Aires, Buenos Aires, Argentina. FAU - Velez, S AU - Velez S AD - Hospital Británico de Buenos Aires, Buenos Aires, Argentina. FAU - Barreira, J AU - Barreira J AD - Hospital Británico de Buenos Aires, Buenos Aires, Argentina. FAU - Tamborenea, N AU - Tamborenea N AD - Organización Médica de Investigación (OMI), Buenos Aires, Argentina. FAU - Santiago, L AU - Santiago L AD - Organización Médica de Investigación (OMI), Buenos Aires, Argentina. FAU - Raiti, L AU - Raiti L AD - Clínica Bessone, Buenos Aires, Argentina. LA - eng PT - Journal Article DEP - 20170914 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antirheumatic Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Adult MH - Antirheumatic Agents/*therapeutic use MH - Fatigue/epidemiology/etiology MH - Female MH - Humans MH - Hydroxychloroquine/*therapeutic use MH - Hypergammaglobulinemia/epidemiology/etiology MH - Incidence MH - Male MH - Middle Aged MH - Purpura/epidemiology/etiology MH - Raynaud Disease/epidemiology/etiology MH - Retrospective Studies MH - Sjogren's Syndrome/*complications/*drug therapy OTO - NOTNLM OT - Extraglandular manifestations OT - Hydroxychloroquine OT - Primary Sjögren’s syndrome OT - Treatment EDAT- 2017/09/16 06:00 MHDA- 2018/06/07 06:00 CRDT- 2017/09/16 06:00 PHST- 2017/05/09 00:00 [received] PHST- 2017/09/04 00:00 [accepted] PHST- 2017/08/29 00:00 [revised] PHST- 2017/09/16 06:00 [pubmed] PHST- 2018/06/07 06:00 [medline] PHST- 2017/09/16 06:00 [entrez] AID - 10.1007/s10067-017-3822-3 [pii] AID - 10.1007/s10067-017-3822-3 [doi] PST - ppublish SO - Clin Rheumatol. 2017 Nov;36(11):2455-2460. doi: 10.1007/s10067-017-3822-3. Epub 2017 Sep 14. PMID- 41043651 OWN - NLM STAT- MEDLINE DCOM- 20251213 LR - 20260105 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 43 IP - 6 DP - 2025 Nov-Dec TI - Are systemic sclerosis and localized scleroderma (morphea) part of a common disease spectrum? A systematic review on their coexistence. PG - 850-858 LID - S0738-081X(25)00265-2 [pii] LID - 10.1016/j.clindermatol.2025.09.032 [doi] AB - Scleroderma encompasses systemic sclerosis (SSc), which may be divided into limited cutaneous and diffuse cutaneous forms, as well as localized scleroderma (LoS), also known as morphea, each with distinct yet overlapping features. Although SSc affects internal organs, LoS (morphea) is typically confined to skin and the underlying tissues. We reviewed the records from 23 studies of 57 patients for coexistence of SSc and LoS (morphea). The majority of patients were women (84%) with a mean disease onset age of 44 years. Limited cutaneous SSc (46%) and nodular morphea (25%) were the most frequent subtypes. Raynaud phenomenon (63%) and sclerodactyly (53%) were common systemic manifestations, with severe complications, such as interstitial lung disease (25%) and esophageal dysmotility (18%), observed in 35% of the patients. Autoantibodies were detected in 67% of the patients, with anti-centromere (28%) and anti-nuclear (23%) antibodies as the most frequent markers. Disease sequencing showed SSc preceding LoS (morphea) in 47% of cases (mean latency: five years), LoS (morphea) preceding SSc in 26% (latency: seven years), and concurrent onset in 26%, often with generalized morphea. The coexistence suggests shared pathogenic mechanisms involving autoimmunity and TGF-β-mediated fibrosis. Treatment data were limited but indicated partial to complete improvement with systemic steroids, immunosuppressants, and other modalities. These findings highlight the importance of monitoring LoS (morphea) patients, particularly those with Raynaud phenomenon or autoantibody positivity, for potential progression to SSc. The overlap supports considering SSc and LoS (morphea) as a continuum rather than distinct entities. CI - Copyright © 2025 Elsevier Inc. All rights reserved. FAU - De Rosa, Carolina AU - De Rosa C AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Di Marco, Giorgia AU - Di Marco G AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Bottino, Vanessa AU - Bottino V AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Pesce, Nazario AU - Pesce N AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Brunetti, Antonio Podo AU - Brunetti AP AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Stabile, Giorgio AU - Stabile G AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Guida, Stefania AU - Guida S AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Rongioletti, Franco AU - Rongioletti F AD - School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Dermatology Clinic, IRCCS San Raffaele Hospital, Milan, Italy. Electronic address: rongioletti.franco@hsr.it. LA - eng PT - Journal Article PT - Systematic Review DEP - 20251001 PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Scleroderma, Localized/complications/immunology/drug therapy MH - *Scleroderma, Systemic/complications/immunology/drug therapy MH - Female MH - Raynaud Disease/etiology MH - Autoantibodies/blood MH - Adult MH - Male COIS- Declaration of competing interest The authors declare no conflict of interest. EDAT- 2025/10/04 00:30 MHDA- 2025/12/14 00:39 CRDT- 2025/10/03 19:36 PHST- 2025/12/14 00:39 [medline] PHST- 2025/10/04 00:30 [pubmed] PHST- 2025/10/03 19:36 [entrez] AID - S0738-081X(25)00265-2 [pii] AID - 10.1016/j.clindermatol.2025.09.032 [doi] PST - ppublish SO - Clin Dermatol. 2025 Nov-Dec;43(6):850-858. doi: 10.1016/j.clindermatol.2025.09.032. Epub 2025 Oct 1. PMID- 29991707 OWN - NLM STAT- MEDLINE DCOM- 20190910 LR - 20200401 IS - 1745-7254 (Electronic) IS - 1671-4083 (Print) IS - 1671-4083 (Linking) VI - 40 IP - 4 DP - 2019 Apr TI - Expression of the oxygen-sensitive transcription factor subunit HIF-1α in patients suffering from secondary Raynaud syndrome. PG - 500-506 LID - 10.1038/s41401-018-0055-1 [doi] AB - Anti-ischemic therapy remains a challenge due to the complexity of hypoxia response pathways. Hypoxia-inducible factor (HIF)-1 is a heterodimer transcription factor consisting of 2 subunits, HIF-1α and HIF-1β. Hypoxia-dependent activation of HIF-1α regulates cellular O(2) homeostasis. Raynaud syndrome (RS), as a comorbidity of the autoimmune disease systemic sclerosis (SS), is characterized by vasospasms that limit blood flow to the limbs, resulting in hypoxia. A single-center randomized study was conducted to compare prostaglandin E1 (PgE1) therapy with a treatment combining PgE1 and an endothelin-1 blocker, bosentan. A total of 30 patients suffering from SS with RS were enrolled. We examined the regulation of HIF-1α, its target heme oxygenase-1 (HMOX-1), and the serum levels of the HIF-1α protein in a subset of patients as well as in ten healthy individuals. The expression of HIF-1α and HMOX-1 in monocytes was measured using absolute plasmid-based quantitative real-time PCR, whereas serum HIF-1α levels were measured with ELISA. Samples were taken at the time of randomization and after 24 weeks. We found that HIF-1α and HMOX-1 mRNA expression in monocytes and serum HIF-1α protein levels were significantly higher in the SS/RS patients compared to the healthy control group. Single-drug therapy significantly increased HIF-1α and HMOX-1 mRNA expression in monocytes and serum HIF-1α protein levels in the SS/RS patients compared to those at the time of randomization, whereas combining PgE1 with an endothelin-1 blocker prevented the further increases in HIF-1α and HMOX-1 expression. We propose HIF-1α and HMOX-1 as novel markers for anti-ischemic therapy in RS. FAU - Heger, Lukas Andreas AU - Heger LA AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. Lukas.heger@uniklinik-freiburg.de. FAU - Kerber, Mark AU - Kerber M AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Hortmann, Marcus AU - Hortmann M AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Robinson, Samuel AU - Robinson S AD - Department of Medicine, Monash University, Melbourne, 3800, Australia. FAU - Mauler, Maximilian AU - Mauler M AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. AD - Faculty of Biology, University of Freiburg, Freiburg, 79104, Germany. FAU - Stallmann, Daniela AU - Stallmann D AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Duerschmied, Daniel AU - Duerschmied D AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Bode, Christoph AU - Bode C AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Hehrlein, Christoph AU - Hehrlein C AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. FAU - Ahrens, Ingo AU - Ahrens I AD - Department of Cardiology and Angiology I, Heart Center Freiburg University, Faculty of Medicine, University of Freiburg, Freiburg, 79106, Germany. AD - Augustinerinnen Hospital, Academic Teaching Hospital, University of Cologne, Cologne, 50678, Germany. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20180710 PL - United States TA - Acta Pharmacol Sin JT - Acta pharmacologica Sinica JID - 100956087 RN - 0 (Hypoxia-Inducible Factor 1, alpha Subunit) RN - S88TT14065 (Oxygen) SB - IM MH - Female MH - Humans MH - Hypoxia-Inducible Factor 1, alpha Subunit/*metabolism MH - Male MH - Middle Aged MH - Oxygen/*metabolism MH - Prospective Studies MH - Raynaud Disease/*metabolism PMC - PMC6461843 OTO - NOTNLM OT - HIF-1alpha OT - HMOX-1 OT - Raynaud syndrome OT - anti-ischemic therapy OT - hypoxia OT - monocytes COIS- The authors declare no competing interests. EDAT- 2018/07/12 06:00 MHDA- 2019/09/11 06:00 PMCR- 2020/04/01 CRDT- 2018/07/12 06:00 PHST- 2017/12/23 00:00 [received] PHST- 2018/05/30 00:00 [accepted] PHST- 2018/07/12 06:00 [pubmed] PHST- 2019/09/11 06:00 [medline] PHST- 2018/07/12 06:00 [entrez] PHST- 2020/04/01 00:00 [pmc-release] AID - 10.1038/s41401-018-0055-1 [pii] AID - 55 [pii] AID - 10.1038/s41401-018-0055-1 [doi] PST - ppublish SO - Acta Pharmacol Sin. 2019 Apr;40(4):500-506. doi: 10.1038/s41401-018-0055-1. Epub 2018 Jul 10. PMID- 31308205 OWN - NLM STAT- MEDLINE DCOM- 20200512 LR - 20200518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 11 DP - 2019 Nov TI - Pauling and Frech reply. PG - 1544-1545 LID - 10.3899/jrheum.190565 [doi] FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath, Bath, UK; JohnPauling@nhs.net. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - University of Utah and Salt Lake Regional Veterans Affairs Medical Center, Salt Lake City, Utah, USA. LA - eng PT - Comment PT - Letter DEP - 20190715 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CON - J Rheumatol. 2019 Oct;46(10):1326-1334. doi: 10.3899/jrheum.180818. PMID: 30824643 CON - J Rheumatol. 2019 Nov;46(11):1543-1544. doi: 10.3899/jrheum.190463. PMID: 31308207 MH - Humans MH - *Raynaud Disease MH - *Scleroderma, Systemic EDAT- 2019/07/17 06:00 MHDA- 2020/05/19 06:00 CRDT- 2019/07/17 06:00 PHST- 2019/07/17 06:00 [pubmed] PHST- 2020/05/19 06:00 [medline] PHST- 2019/07/17 06:00 [entrez] AID - jrheum.190565 [pii] AID - 10.3899/jrheum.190565 [doi] PST - ppublish SO - J Rheumatol. 2019 Nov;46(11):1544-1545. doi: 10.3899/jrheum.190565. Epub 2019 Jul 15. PMID- 23492397 OWN - NLM STAT- MEDLINE DCOM- 20130909 LR - 20131121 IS - 1743-0593 (Electronic) IS - 1743-0585 (Linking) VI - 98 IP - 2 DP - 2013 Apr TI - How to use... antinuclear antibodies in paediatric rheumatic diseases. PG - 64-70 LID - 10.1136/archdischild-2012-303326 [doi] FAU - Weaver, Lehn K AU - Weaver LK AD - Department of Pediatric Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. FAU - Behrens, Edward M AU - Behrens EM LA - eng GR - K08AI079396/AI/NIAID NIH HHS/United States GR - T32 HD043021/HD/NICHD NIH HHS/United States GR - Howard Hughes Medical Institute/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - England TA - Arch Dis Child Educ Pract Ed JT - Archives of disease in childhood. Education and practice edition JID - 101220684 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*analysis MH - Arthritis, Juvenile/diagnosis MH - Autoimmune Diseases/diagnosis MH - Autoimmunity MH - Child MH - Fluorescent Antibody Technique, Indirect MH - Humans MH - Predictive Value of Tests MH - Raynaud Disease/diagnosis MH - Rheumatic Diseases/*diagnosis/immunology MH - Sensitivity and Specificity EDAT- 2013/03/16 06:00 MHDA- 2013/09/10 06:00 CRDT- 2013/03/16 06:00 PHST- 2013/03/16 06:00 [entrez] PHST- 2013/03/16 06:00 [pubmed] PHST- 2013/09/10 06:00 [medline] AID - archdischild-2012-303326 [pii] AID - 10.1136/archdischild-2012-303326 [doi] PST - ppublish SO - Arch Dis Child Educ Pract Ed. 2013 Apr;98(2):64-70. doi: 10.1136/archdischild-2012-303326. PMID- 17319437 OWN - NLM STAT- MEDLINE DCOM- 20070424 LR - 20181201 IS - 0034-9356 (Print) IS - 0034-9356 (Linking) VI - 54 IP - 1 DP - 2007 Jan TI - [Anesthetic management for mediastinoscopy in a patient with severe pulmonary hypertension]. PG - 55-6 FAU - Murcia Sánchez, E AU - Murcia Sánchez E FAU - Germán, M J AU - Germán MJ FAU - Ibáñez, V AU - Ibáñez V FAU - Pérez-Cerdá, F AU - Pérez-Cerdá F LA - spa PT - Case Reports PT - Letter TT - Manejo anestésico para la realización de mediastinoscopia en un caso de hipertensión pulmonar severa. PL - Spain TA - Rev Esp Anestesiol Reanim JT - Revista espanola de anestesiologia y reanimacion JID - 0134516 RN - 0 (Methyl Ethers) RN - 0 (Piperidines) RN - 27O7W4T232 (Spironolactone) RN - 38LVP0K73A (Sevoflurane) RN - 7LXU5N7ZO5 (Furosemide) RN - DCR9Z582X0 (Epoprostenol) RN - P10582JYYK (Remifentanil) RN - UF599785JZ (Fentanyl) SB - IM MH - Anesthesia, General/*methods MH - Bundle-Branch Block/complications MH - Dermatomyositis/complications MH - Dyslipidemias/complications MH - Epoprostenol/therapeutic use MH - Female MH - Fentanyl MH - Furosemide/therapeutic use MH - Humans MH - Hypertension/complications MH - Hypertension, Pulmonary/*complications/drug therapy/therapy MH - Hypertrophy, Right Ventricular/complications MH - Mediastinal Diseases/diagnosis/surgery MH - *Mediastinoscopy MH - Methyl Ethers MH - Middle Aged MH - Oxygen Inhalation Therapy MH - Piperidines MH - Preoperative Care MH - Raynaud Disease/complications MH - Remifentanil MH - Sevoflurane MH - Spironolactone/therapeutic use EDAT- 2007/02/27 09:00 MHDA- 2007/04/25 09:00 CRDT- 2007/02/27 09:00 PHST- 2007/02/27 09:00 [pubmed] PHST- 2007/04/25 09:00 [medline] PHST- 2007/02/27 09:00 [entrez] PST - ppublish SO - Rev Esp Anestesiol Reanim. 2007 Jan;54(1):55-6. PMID- 16750953 OWN - NLM STAT- MEDLINE DCOM- 20060609 LR - 20131121 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 119 IP - 6 DP - 2006 Jun TI - Primary Sjögren's syndrome and vitamin B12 deficiency: preliminary results in 80 patients. PG - e9-10 FAU - Andrès, Emmanuel AU - Andrès E FAU - Blicklé, Frédéric AU - Blicklé F FAU - Sordet, Christelle AU - Sordet C FAU - Cohen-Solal, Julien AU - Cohen-Solal J FAU - Sibilia, Jean AU - Sibilia J FAU - Sapin, Rémy AU - Sapin R LA - eng PT - Letter PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantigens) RN - P6YC3EG204 (Vitamin B 12) SB - IM MH - Antibodies, Antinuclear/blood MH - Arthralgia/etiology MH - Arthritis/etiology MH - Autoantigens/blood MH - Female MH - France/epidemiology MH - Humans MH - Male MH - Mental Disorders/etiology MH - Middle Aged MH - Nervous System Diseases/etiology MH - Prevalence MH - Raynaud Disease/etiology MH - Sjogren's Syndrome/blood/*complications/immunology MH - Vitamin B 12/*blood MH - Vitamin B 12 Deficiency/blood/*complications/immunology EDAT- 2006/06/06 09:00 MHDA- 2006/06/10 09:00 CRDT- 2006/06/06 09:00 PHST- 2005/09/01 00:00 [received] PHST- 2005/09/07 00:00 [revised] PHST- 2005/09/08 00:00 [accepted] PHST- 2006/06/06 09:00 [pubmed] PHST- 2006/06/10 09:00 [medline] PHST- 2006/06/06 09:00 [entrez] AID - S0002-9343(05)00810-7 [pii] AID - 10.1016/j.amjmed.2005.09.015 [doi] PST - ppublish SO - Am J Med. 2006 Jun;119(6):e9-10. doi: 10.1016/j.amjmed.2005.09.015. PMID- 26972993 OWN - NLM STAT- MEDLINE DCOM- 20170816 LR - 20250626 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 45 IP - 6 DP - 2016 Jun TI - A systematic review and meta-analysis of cutaneous manifestations in late- versus early-onset systemic lupus erythematosus. PG - 691-7 LID - S0049-0172(16)00030-5 [pii] LID - 10.1016/j.semarthrit.2016.01.004 [doi] AB - OBJECTIVES: Although systemic lupus erythematosus (SLE) most commonly occurs in reproductive-age women, some are diagnosed after the age of 50. Recognizing that greater than one-third of SLE criteria are cutaneous, we undertook a systematic review and meta-analysis to evaluate differences in cutaneous manifestations in early- and late-onset SLE patients. METHODS: We searched the literature using PubMed, CINAHL, Web of Science, and Cochrane Library. We excluded studies that did not include ACR SLE classification criteria, early-onset controls, that defined late-onset SLE as <50 years of age, or were not written in English. Two authors rated study quality using the Newcastle Ottawa Quality Scale. We used Forest plots to compare odds ratios (95% CI) of cutaneous manifestations by age. Study heterogeneity was assessed using I(2). RESULTS: Overall, 35 studies, representing 11,189 early-onset and 1727 late-onset patients with SLE, met eligibility criteria. The female:male ratio was lower in the late-onset group (5:1 versus 8:1). Most cutaneous manifestations were less prevalent in the late-onset group. In particular, malar rash [OR = 0.43 (0.35, 0.52)], photosensitivity [OR = 0.72 (0.59, 0.88)], and livedo reticularis [OR = 0.33 (0.17, 0.64)] were less common in late-onset patients. In contrast, sicca symptoms were more common [OR = 2.45 (1.91, 3.14)]. The mean Newcastle Ottawa Quality Scale score was 6.3 ± 0.5 (scale: 0-9) with high inter-rater reliability for the score (0.96). CONCLUSIONS: Overall, cutaneous manifestations are less common in late-onset SLE patients, except sicca symptoms. Future studies should investigate etiologies for this phenomenon including roles of immune senescence, environment, gender, and immunogenetics. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Medlin, Jennifer L AU - Medlin JL AD - University of Wisconsin Hospital and Clinics, Madison, WI. FAU - Hansen, Karen E AU - Hansen KE AD - Department of Medicine, University of Wisconsin School of Medicine and Public Health, 1625 Highland Ave #4132, Madison, WI 53705. FAU - Fitz, Sara R AU - Fitz SR AD - Department of Dermatology, Medical Associates Clinic, Dubuque, IA. FAU - Bartels, Christie M AU - Bartels CM AD - Department of Medicine, University of Wisconsin School of Medicine and Public Health, 1625 Highland Ave #4132, Madison, WI 53705. LA - eng GR - K23 AR062381/AR/NIAMS NIH HHS/United States GR - L30 AR063469/AR/NIAMS NIH HHS/United States GR - UL1 TR000427/TR/NCATS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20160121 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Age of Onset MH - Alopecia/etiology/physiopathology MH - Exanthema/etiology/*physiopathology MH - Female MH - Humans MH - Late Onset Disorders/complications/*physiopathology MH - Livedo Reticularis/etiology/*physiopathology MH - Lupus Erythematosus, Systemic/complications/*physiopathology MH - Male MH - Middle Aged MH - Odds Ratio MH - Photosensitivity Disorders/etiology/*physiopathology MH - Raynaud Disease/etiology/physiopathology MH - Skin Diseases/etiology/physiopathology MH - Vasculitis/etiology/physiopathology PMC - PMC4879060 MID - NIHMS767968 OTO - NOTNLM OT - Alopecia OT - Cutaneous OT - Late onset OT - Malar rash OT - Photosensitivity OT - Raynaud OT - Sicca OT - Systemic lupus erythematosus COIS- The authors have no direct financial, consultant, or institutional conflict of interest pertaining to this article. EDAT- 2016/03/15 06:00 MHDA- 2017/08/17 06:00 PMCR- 2017/06/01 CRDT- 2016/03/15 06:00 PHST- 2015/08/19 00:00 [received] PHST- 2015/12/10 00:00 [revised] PHST- 2016/01/15 00:00 [accepted] PHST- 2016/03/15 06:00 [entrez] PHST- 2016/03/15 06:00 [pubmed] PHST- 2017/08/17 06:00 [medline] PHST- 2017/06/01 00:00 [pmc-release] AID - S0049-0172(16)00030-5 [pii] AID - 10.1016/j.semarthrit.2016.01.004 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Jun;45(6):691-7. doi: 10.1016/j.semarthrit.2016.01.004. Epub 2016 Jan 21. PMID- 26639766 OWN - NLM STAT- MEDLINE DCOM- 20170216 LR - 20181202 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 63 IP - 4 DP - 2016 Oct 5 TI - HTR1B gene variants associate with the susceptibility of Raynauds' phenomenon in workers exposed hand-arm vibration. PG - 335-347 AB - OBJECTIVE: To explore whether polymorphic variants of the HTR1B gene are associated with the susceptibility of Raynauds' Phenomenon (RP) coursed by vibration. METHODS: 148 subjects exposed to vibration for more than 2 years were classified into either induced white finger (VWF) group (n = 72), or non-VWF group (n = 76). Vibration exposure levels were measured and assessed following ISO 5349-1:2001 protocol. All workers were genotyped by sequencing for the single nucleotide polymorphisms (SNPs) in the 5'-flanking and coding region of HTR1B. Genetic characteristics and linkage disequilibrium (LD) were analyzed with Haploview. Serum serotonin levels of each subject were detected using ELISA. The association between the susceptibility of vascular damage and genotype was analyzed via logistic regression. RESULTS: 7 known SNPs were obtained and their allele frequencies were inserted into the Hardy-Weinberg equilibrium. rs6297 variant genotype had an increased risk of VWF compared with wild genotype (OR = 2.14, 95% CI = 1.04- 4.58, P < 0.05). rs6298 mutant type (AG+GG) was found to have a significant interaction on vibration exposure LN(CEI), accounting for VWF occurrence. LN(5-HT) level is significantly different between the VWF group (x¯±s= 1.99±1.09 ng/mL) and the non-VWF group (x¯±s= 2.72±1.47 ng/mL). CONCLUSIONS: Serotonin levels may affect the progression of secondary RP. Polymorphic variants of the HTR1B gene are associated with the susceptibility of secondary RP in vibration-exposed occupational populations of Chinese Han people. FAU - Chen, Qingsong AU - Chen Q AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Lang, Li AU - Lang L AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Xiao, Bin AU - Xiao B AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Lin, Hansheng AU - Lin H AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Yang, Aichu AU - Yang A AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Li, Hongling AU - Li H AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. FAU - Tang, Shichuan AU - Tang S AD - Key Laboratory of Occupational Health and Safety, Beijing Municipal Institute of Labor Protection, Beijing, China. FAU - Huang, Hanlin AU - Huang H AD - Guangdong Prevention and Cure Centre of Occupational Diseases, Guangzhou, China. LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 0 (HTR1B protein, human) RN - 0 (Receptor, Serotonin, 5-HT1B) SB - IM MH - Adult MH - Arm/*pathology MH - Female MH - Genetic Predisposition to Disease MH - Hand/*pathology MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*genetics/pathology MH - Polymorphism, Single Nucleotide MH - Raynaud Disease/*genetics/pathology MH - Receptor, Serotonin, 5-HT1B/*genetics MH - Vibration/*adverse effects OTO - NOTNLM OT - HTR1B OT - Raynauds’ Phenomenon (RP) OT - single nucleotide polymorphisms OT - vascular damage OT - vibration-induced white finger (VWF) EDAT- 2015/12/08 06:00 MHDA- 2017/02/17 06:00 CRDT- 2015/12/08 06:00 PHST- 2015/12/08 06:00 [pubmed] PHST- 2017/02/17 06:00 [medline] PHST- 2015/12/08 06:00 [entrez] AID - CH2021 [pii] AID - 10.3233/CH-152021 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2016 Oct 5;63(4):335-347. doi: 10.3233/CH-152021. PMID- 18848159 OWN - NLM STAT- MEDLINE DCOM- 20090203 LR - 20131121 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 19 IP - 6 DP - 2008 Oct TI - Persistently low glucose levels despite high-dose methylprednisolone in a woman with mixed connective tissue disease. PG - e18-9 LID - 10.1016/j.ejim.2007.12.003 [doi] FAU - Cumbo-Nacheli, Gustavo AU - Cumbo-Nacheli G AD - Department of Internal Medicine, 4201 Saint Antoine UHC 2E, Detroit, MI 48201, USA. gcumbona@med.wayne.edu FAU - Torres-Heisecke, Raul AU - Torres-Heisecke R FAU - Attallah, Hamdee AU - Attallah H LA - eng PT - Case Reports PT - Journal Article DEP - 20080130 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Blood Glucose) RN - 0 (Glucocorticoids) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Blood Glucose/*analysis MH - Female MH - Glucocorticoids/*therapeutic use MH - Humans MH - Methylprednisolone/*therapeutic use MH - Mixed Connective Tissue Disease/*blood/*drug therapy MH - Raynaud Disease EDAT- 2008/10/14 09:00 MHDA- 2009/02/04 09:00 CRDT- 2008/10/14 09:00 PHST- 2007/11/30 00:00 [received] PHST- 2007/12/15 00:00 [accepted] PHST- 2008/10/14 09:00 [pubmed] PHST- 2009/02/04 09:00 [medline] PHST- 2008/10/14 09:00 [entrez] AID - S0953-6205(07)00390-1 [pii] AID - 10.1016/j.ejim.2007.12.003 [doi] PST - ppublish SO - Eur J Intern Med. 2008 Oct;19(6):e18-9. doi: 10.1016/j.ejim.2007.12.003. Epub 2008 Jan 30. PMID- 17238361 OWN - NLM STAT- MEDLINE DCOM- 20070928 LR - 20220331 IS - 1942-597X (Electronic) IS - 1559-4076 (Linking) VI - 2006 DP - 2006 TI - Exploiting semantic relations for literature-based discovery. PG - 349-53 AB - We propose using semantic predications to enhance literature-based discovery (LBD) systems, which currently depend exclusively on co-occurrence of words or concepts in target documents. In this paper, the predications, which are produced by the combined application of two natural language processing systems, BioMedLEE and SemRep, are coupled with an LBD system BITOLA. Initial experiments suggest this approach can uncover new associations that were not possible using previous methods. FAU - Hristovski, Dimitar AU - Hristovski D AD - Institure of Biomedical Informatics, Medical Faculty, University of Ljubljana, Slovenia. dimitar.hristovski@mf.uni-lf.si FAU - Friedman, Carol AU - Friedman C FAU - Rindflesch, Thomas C AU - Rindflesch TC FAU - Peterlin, Borut AU - Peterlin B LA - eng GR - R01 LM007659/LM/NLM NIH HHS/United States GR - R01 LM008635/LM/NLM NIH HHS/United States GR - LM007659/LM/NLM NIH HHS/United States GR - LM008635/LM/NLM NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - AMIA Annu Symp Proc JT - AMIA ... Annual Symposium proceedings. AMIA Symposium JID - 101209213 SB - IM MH - Biomedical Research MH - Blood Viscosity MH - Databases, Bibliographic MH - Humans MH - Huntington Disease MH - Information Storage and Retrieval/*methods MH - *Natural Language Processing MH - Platelet Aggregation MH - Publications MH - Raynaud Disease/blood MH - Semantics PMC - PMC1839258 EDAT- 2007/01/24 09:00 MHDA- 2007/09/29 09:00 PMCR- 2006/01/01 CRDT- 2007/01/24 09:00 PHST- 2007/01/24 09:00 [pubmed] PHST- 2007/09/29 09:00 [medline] PHST- 2007/01/24 09:00 [entrez] PHST- 2006/01/01 00:00 [pmc-release] AID - 86414 [pii] AID - amia2006_0349 [pii] PST - ppublish SO - AMIA Annu Symp Proc. 2006;2006:349-53. PMID- 16711151 OWN - NLM STAT- MEDLINE DCOM- 20061030 LR - 20061115 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 2 IP - 62 DP - 2006 Apr 19 TI - [Pathogenic mechanisms in systemic sclerosis and their therapeutical consequences. Part 2: treatment]. PG - 1058, 1060-6 AB - Systemic sclerosis (scleroderma) is considered as the most severe connective tissue disease. It is characterized by an abnormal immune activation, a vasculopathy and a fibrosis of the skin and of multiple internal organs. Numerous progress in the understanding of the pathogenesis with identification of key molecules have permit to introduce novel treatments that improve the management of some aspects of the disease. ACE inhibitors are effective in resolving renal crisis. Cyclophosphamide is useful for treatment of fibrosing alveolitis. Prostaglandins, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors permit to improve the treatment of the vascular complications (digital ulcerations, pulmonary arterial hypertension) of scleroderma. FAU - Zuber, J P AU - Zuber JP AD - Service d'immunologie et d'allergie Département de médecine CHUV, BH 18/707. jean-philippe.zuber@chuv.ch FAU - Chizzolini, C AU - Chizzolini C FAU - Leimgruber, A AU - Leimgruber A FAU - Bart, P A AU - Bart PA FAU - Spertini, F AU - Spertini F LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Mécanismes pathogéniques de la sclérodermie et leurs conséquences thérapeutiques. 2e partie: traitement. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 SB - IM MH - Fingers MH - Humans MH - Hypertension, Pulmonary/etiology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/*therapy MH - Skin Ulcer/etiology RF - 42 EDAT- 2006/05/23 09:00 MHDA- 2006/10/31 09:00 CRDT- 2006/05/23 09:00 PHST- 2006/05/23 09:00 [pubmed] PHST- 2006/10/31 09:00 [medline] PHST- 2006/05/23 09:00 [entrez] PST - ppublish SO - Rev Med Suisse. 2006 Apr 19;2(62):1058, 1060-6. PMID- 26034146 OWN - NLM STAT- MEDLINE DCOM- 20160406 LR - 20220410 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 42 IP - 7 DP - 2015 Jul TI - Use of Laser Speckle Contrast Imaging to Assess Digital Microvascular Function in Primary Raynaud Phenomenon and Systemic Sclerosis: A Comparison Using the Raynaud Condition Score Diary. PG - 1163-8 LID - 10.3899/jrheum.141437 [doi] AB - OBJECTIVE: Evaluate objective assessment of digital microvascular function using laser speckle contrast imaging (LSCI) in a cross-sectional study of patients with primary Raynaud phenomenon (RP) and systemic sclerosis (SSc), comparing LSCI with both infrared thermography (IRT) and subjective assessment using the Raynaud Condition Score (RCS) diary. METHODS: Patients with SSc (n = 25) and primary RP (n = 18) underwent simultaneous assessment of digital perfusion using LSCI and IRT with a cold challenge on 2 occasions, 2 weeks apart. The RCS diary was completed between assessments. The relationship between objective and subjective assessments of RP was evaluated. Reproducibility of LSCI/IRT was assessed, along with differences between primary RP and SSc, and the effect of sex. RESULTS: There was moderate-to-good correlation between LSCI and IRT (Spearman rho 0.58-0.84, p < 0.01), but poor correlation between objective assessments and the RCS diary (p > 0.05 for all analyses). Reproducibility of IRT and LSCI was moderate at baseline (ICC 0.51-0.63) and immediately following cold challenge (ICC 0.56-0.86), but lower during reperfusion (ICC 0.3-0.7). Neither subjective nor objective assessments differentiated between primary RP and SSc. Men reported lower median daily frequency of RP attacks (0.82 vs 1.93, p = 0.03). Perfusion using LSCI/IRT was higher in men for the majority of assessments. CONCLUSION: Objective and subjective methods provide differing information on microvascular function in RP. There is good convergent validity of LSCI with IRT and acceptable reproducibility of both modalities. Neither subjective nor objective assessments could differentiate between primary RP and SSc. Influence of sex on subjective and objective assessment of RP warrants further evaluation. FAU - Pauling, John D AU - Pauling JD AD - From the Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; and the Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.J.D. Pauling, BMedSci, BMBS, MRCP, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath; J.A. Shipley, BSc, PhD; D.J. Hart, BSc, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; A. McGrogan, PhD, Department of Pharmacy and Pharmacology, University of Bath; N.J. McHugh, BMBS, FRCP, FRCPath, MD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath. John.Pauling@rnhrd.nhs.uk. FAU - Shipley, Jacqueline A AU - Shipley JA AD - From the Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; and the Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.J.D. Pauling, BMedSci, BMBS, MRCP, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath; J.A. Shipley, BSc, PhD; D.J. Hart, BSc, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; A. McGrogan, PhD, Department of Pharmacy and Pharmacology, University of Bath; N.J. McHugh, BMBS, FRCP, FRCPath, MD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath. FAU - Hart, Darren J AU - Hart DJ AD - From the Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; and the Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.J.D. Pauling, BMedSci, BMBS, MRCP, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath; J.A. Shipley, BSc, PhD; D.J. Hart, BSc, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; A. McGrogan, PhD, Department of Pharmacy and Pharmacology, University of Bath; N.J. McHugh, BMBS, FRCP, FRCPath, MD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath. FAU - McGrogan, Anita AU - McGrogan A AD - From the Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; and the Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.J.D. Pauling, BMedSci, BMBS, MRCP, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath; J.A. Shipley, BSc, PhD; D.J. Hart, BSc, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; A. McGrogan, PhD, Department of Pharmacy and Pharmacology, University of Bath; N.J. McHugh, BMBS, FRCP, FRCPath, MD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath. FAU - McHugh, Neil J AU - McHugh NJ AD - From the Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; and the Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.J.D. Pauling, BMedSci, BMBS, MRCP, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath; J.A. Shipley, BSc, PhD; D.J. Hart, BSc, PhD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases; A. McGrogan, PhD, Department of Pharmacy and Pharmacology, University of Bath; N.J. McHugh, BMBS, FRCP, FRCPath, MD, Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, and Department of Pharmacy and Pharmacology, University of Bath. LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150601 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Cross-Sectional Studies MH - Diagnostic Imaging/methods MH - Female MH - Fingers/*blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation/*physiology MH - Middle Aged MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/*physiopathology OTO - NOTNLM OT - IMAGING OT - OUTCOMES MEASUREMENT OT - RAYNAUD PHENOMENON OT - REPRODUCIBILITY OT - SYSTEMIC SCLEROSIS EDAT- 2015/06/03 06:00 MHDA- 2016/04/07 06:00 CRDT- 2015/06/03 06:00 PHST- 2015/03/02 00:00 [accepted] PHST- 2015/06/03 06:00 [entrez] PHST- 2015/06/03 06:00 [pubmed] PHST- 2016/04/07 06:00 [medline] AID - jrheum.141437 [pii] AID - 10.3899/jrheum.141437 [doi] PST - ppublish SO - J Rheumatol. 2015 Jul;42(7):1163-8. doi: 10.3899/jrheum.141437. Epub 2015 Jun 1. PMID- 30811376 OWN - NLM STAT- MEDLINE DCOM- 20190920 LR - 20190920 IS - 1533-712X (Electronic) IS - 0271-0749 (Linking) VI - 39 IP - 2 DP - 2019 Mar/Apr TI - Methylphenidate-Induced Raynaud Phenomenon Developed After Increasing Methylphenidate in an Adult With Attention-Deficit Hyperactivity Disorder. PG - 178-179 LID - 10.1097/JCP.0000000000001007 [doi] FAU - Monteerarat, Yuwarat AU - Monteerarat Y AD - Department of Orthopaedic SurgeryFaculty of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand. Department of Psychiatry Faculty of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand pornjirap@gmail.com. FAU - Pariwatcharakul, Pornjira AU - Pariwatcharakul P LA - eng PT - Case Reports PT - Letter PL - United States TA - J Clin Psychopharmacol JT - Journal of clinical psychopharmacology JID - 8109496 RN - 0 (Central Nervous System Stimulants) RN - 207ZZ9QZ49 (Methylphenidate) SB - IM MH - Adult MH - Attention Deficit Disorder with Hyperactivity/*drug therapy MH - Central Nervous System Stimulants/administration & dosage/*adverse effects MH - Dose-Response Relationship, Drug MH - Female MH - Humans MH - Methylphenidate/administration & dosage/*adverse effects MH - Raynaud Disease EDAT- 2019/02/28 06:00 MHDA- 2019/09/21 06:00 CRDT- 2019/02/28 06:00 PHST- 2019/02/28 06:00 [entrez] PHST- 2019/02/28 06:00 [pubmed] PHST- 2019/09/21 06:00 [medline] AID - 00004714-201903000-00020 [pii] AID - 10.1097/JCP.0000000000001007 [doi] PST - ppublish SO - J Clin Psychopharmacol. 2019 Mar/Apr;39(2):178-179. doi: 10.1097/JCP.0000000000001007. PMID- 33600677 OWN - NLM STAT- MEDLINE DCOM- 20211210 LR - 20211214 IS - 2501-062X (Electronic) IS - 1220-4749 (Linking) VI - 59 IP - 3 DP - 2021 Sep 1 TI - Nailfold capillaroscopy in systemic diseases: short overview for internal medicine. PG - 201-217 LID - 10.2478/rjim-2021-0007 [doi] AB - Nailfold capillaroscopy (NFC) is now one of the main imaging tools in systemic sclerosis and imposed over time as an easy, non-invasive method for the nailfold microvascular bed assessment. In qualitative NFC normal pattern is characterized by homogeneous, parallel fashion arrangement of the last capillaries row as well as by capillaries with hairpin or non-specific variations like tortuous and/ or crossing shape. Nailfold capillaroscopy is strongly recommended for evaluation of all patients with Raynaud phenomenon. Appearance of giant capillaries is chronologically the first relevant finding for scleroderma spectrum disorders development (systemic sclerosis, dermatomyositis, undifferentiated and mixed connective tissue disease). Collapses of the giant loops generate microhemorrhages and further capillary loss with subsequent hypoxia, and neoangiogenesis seen as ramified/ bushy capillaries. Nailfold capillaroscopy is indicated especially in systemic sclerosis, being also included in the classification criteria. Based on these major NFC pathologic findings (giant capillaries, microhemorrhages, avascularity and neoangiogenesis), three evolutive stages were described in systemic sclerosis, namely the early, active, and late scleroderma pattern. In other connective tissue diseases than those scleroderma-related, like systemic lupus erythematosus, psoriatic arthritis, or antiphospholipid syndrome, the interest for capillaroscopy is growing, but the attempts of defining specific characteristics failed until now. Besides qualitative NFC, semiquantitative and quantitative capillaroscopic assessments were proposed for more accurate evaluation. Lately, automated systems are under development. There is still need of more studies to sustain the nailfold capillaroscopy validity as diagnostic and prognostic test. CI - © 2021 Alina Dima et al., published by Sciendo. FAU - Dima, Alina AU - Dima A AD - Department of Rheumatology, Colentina Clinical Hospital, Bucharest, Romania. FAU - Berza, Ioana AU - Berza I AD - Department of Rheumatology, Colentina Clinical Hospital, Bucharest, Romania. FAU - Popescu, Daniela Nicoleta AU - Popescu DN AD - Department of Rheumatology, Colentina Clinical Hospital, Bucharest, Romania. FAU - Parvu, Magda Ileana AU - Parvu MI AD - Department of Rheumatology, Colentina Clinical Hospital, Bucharest, Romania. LA - eng PT - Journal Article PT - Review DEP - 20210826 PL - Germany TA - Rom J Intern Med JT - Romanian journal of internal medicine = Revue roumaine de medecine interne JID - 9304507 SB - IM MH - Capillaries/*diagnostic imaging MH - Humans MH - Microscopic Angioscopy/*methods MH - Raynaud Disease/*diagnostic imaging MH - Scleroderma, Systemic/*diagnostic imaging OTO - NOTNLM OT - microangiopathy OT - nailfold capillaroscopy OT - systemic sclerosis OT - video capillaroscopy EDAT- 2021/02/19 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/02/18 17:16 PHST- 2020/12/03 00:00 [received] PHST- 2021/02/19 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/02/18 17:16 [entrez] AID - rjim-2021-0007 [pii] AID - 10.2478/rjim-2021-0007 [doi] PST - epublish SO - Rom J Intern Med. 2021 Aug 26;59(3):201-217. doi: 10.2478/rjim-2021-0007. Print 2021 Sep 1. PMID- 21456859 OWN - NLM STAT- MEDLINE DCOM- 20110809 LR - 20191210 IS - 1560-2281 (Electronic) IS - 1083-3668 (Linking) VI - 16 IP - 3 DP - 2011 Mar TI - Segmental analysis of indocyanine green pharmacokinetics for the reliable diagnosis of functional vascular insufficiency. PG - 030504 LID - 10.1117/1.3556718 [doi] AB - Accurate and reliable diagnosis of functional insufficiency of peripheral vasculature is essential since Raynaud phenomenon (RP), most common form of peripheral vascular insufficiency, is commonly associated with systemic vascular disorders. We have previously demonstrated that dynamic imaging of near-infrared fluorophore indocyanine green (ICG) can be a noninvasive and sensitive tool to measure tissue perfusion. In the present study, we demonstrated that combined analysis of multiple parameters, especially onset time and modified T(max) which means the time from onset of ICG fluorescence to T(max), can be used as a reliable diagnostic tool for RP. To validate the method, we performed the conventional thermographic analysis combined with cold challenge and rewarming along with ICG dynamic imaging and segmental analysis. A case-control analysis demonstrated that segmental pattern of ICG dynamics in both hands was significantly different between normal and RP case, suggesting the possibility of clinical application of this novel method for the convenient and reliable diagnosis of RP. FAU - Kang, Yujung AU - Kang Y FAU - Lee, Jungsul AU - Lee J FAU - An, Yuri AU - An Y FAU - Jeon, Jongwook AU - Jeon J FAU - Choi, Chulhee AU - Choi C LA - eng PT - Evaluation Study PT - Letter PT - Research Support, Non-U.S. Gov't PT - Validation Study PL - United States TA - J Biomed Opt JT - Journal of biomedical optics JID - 9605853 RN - 0 (Coloring Agents) RN - 0 (Fluorescent Dyes) RN - IX6J1063HV (Indocyanine Green) SB - IM MH - Adult MH - *Coloring Agents/pharmacokinetics MH - Female MH - Fluorescent Dyes/pharmacokinetics MH - Humans MH - *Indocyanine Green/pharmacokinetics MH - Male MH - Optical Phenomena MH - Peripheral Vascular Diseases/*diagnosis MH - Raynaud Disease/diagnosis MH - Spectroscopy, Near-Infrared EDAT- 2011/04/05 06:00 MHDA- 2011/08/10 06:00 CRDT- 2011/04/05 06:00 PHST- 2011/04/05 06:00 [entrez] PHST- 2011/04/05 06:00 [pubmed] PHST- 2011/08/10 06:00 [medline] AID - 10.1117/1.3556718 [doi] PST - ppublish SO - J Biomed Opt. 2011 Mar;16(3):030504. doi: 10.1117/1.3556718. PMID- 19002303 OWN - NLM STAT- MEDLINE DCOM- 20090106 LR - 20151119 IS - 0300-8495 (Print) IS - 0300-8495 (Linking) VI - 37 IP - 10 DP - 2008 Oct TI - Red flags in scleroderma. PG - 831-4 AB - BACKGROUND: Scleroderma (systemic sclerosis) is an uncommon connective tissue disease characterised by vascular, inflammatory and fibrotic dysfunction of multiple organ systems. Systemic sclerosis is often recognised late in the course of the disease. OBJECTIVE: This article outlines the clinical features of systemic sclerosis, in particular 'red flags' that indicate the presence of significant organ disease. DISCUSSION: Common clinical features include Raynaud phenomenon and skin thickening, often with calcinosis and telangiectasia. These features should alert the physician to look for red flag features. In the general practice setting, early recognition of scleroderma will enable timely referral to specialist centres for regular screening and effective management of its many serious visceral complications. FAU - Li, Qiang AU - Li Q AD - Rheumatology Department, Monash Medical Centre, Melbourne, Victoria. drqiang@hotmail.com FAU - Sahhar, Joanne AU - Sahhar J FAU - Littlejohn, Geoffrey AU - Littlejohn G LA - eng PT - Journal Article PT - Review PL - Australia TA - Aust Fam Physician JT - Australian family physician JID - 0326701 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (Biomarkers) SB - IM MH - Antibodies, Antinuclear/analysis MH - Antigens, Nuclear/analysis MH - Biomarkers/analysis MH - Calcinosis MH - Early Diagnosis MH - Gastrointestinal Diseases MH - Heart Diseases MH - Humans MH - Lung Diseases MH - Raynaud Disease MH - *Scleroderma, Systemic/diagnosis/pathology/therapy MH - Skin/pathology MH - Telangiectasis RF - 17 EDAT- 2008/11/13 09:00 MHDA- 2009/01/07 09:00 CRDT- 2008/11/13 09:00 PHST- 2008/11/13 09:00 [pubmed] PHST- 2009/01/07 09:00 [medline] PHST- 2008/11/13 09:00 [entrez] PST - ppublish SO - Aust Fam Physician. 2008 Oct;37(10):831-4. PMID- 28502374 OWN - NLM STAT- MEDLINE DCOM- 20180321 LR - 20180321 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 46 IP - 5 DP - 2017 May TI - [Severe membranoproliferative glomerulonephritis with polyadenopathy associated with hypocomplementemic urticarial vasculitis syndrome]. PG - 547-550 LID - S0755-4982(17)30105-7 [pii] LID - 10.1016/j.lpm.2017.01.020 [doi] FAU - Gheerbrant, Hubert AU - Gheerbrant H AD - CHU de Grenoble, service de pneumologie, BP 2017, 38043 Grenoble cedex 09, France. Electronic address: hgheerbrant@chu-grenoble.fr. FAU - Giovannini, Diane AU - Giovannini D AD - CHU de Grenoble, service d'anatomo-pathologie, BP 2017, 38043 Grenoble cedex 09, France. FAU - Falque, Loic AU - Falque L AD - CHU de Grenoble, service de pneumologie, BP 2017, 38043 Grenoble cedex 09, France. FAU - Andry, Fanny AU - Andry F AD - CHU de Grenoble, service de médecine interne, BP 2017, 38043 Grenoble cedex 09, France. FAU - Lugosi, Maxime AU - Lugosi M AD - CHU de Grenoble, service de médecine interne, BP 2017, 38043 Grenoble cedex 09, France. FAU - Deroux, Alban AU - Deroux A AD - CHU de Grenoble, service de médecine interne, BP 2017, 38043 Grenoble cedex 09, France. LA - fre PT - Case Reports PT - Letter TT - Vascularite hypocomplémentémique urticarienne associée à une glomérulonéphrite membrano-proliférative sévère et polyadénopathies. DEP - 20170510 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 80295-33-6 (Complement C1q) SB - IM MH - Adult MH - Arthralgia/etiology MH - Autoantibodies/immunology MH - Autoantigens/immunology MH - Biopsy MH - Complement C1q/immunology MH - Diagnosis, Differential MH - Female MH - Glomerulonephritis, Membranoproliferative/*etiology/immunology/pathology MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis MH - Lymphadenopathy/diagnostic imaging/*etiology MH - Multidetector Computed Tomography MH - Pancytopenia/etiology MH - Positron Emission Tomography Computed Tomography MH - Raynaud Disease/etiology MH - Syndrome MH - Urticaria/*etiology MH - Vasculitis, Leukocytoclastic, Cutaneous/*complications/diagnosis/immunology EDAT- 2017/05/16 06:00 MHDA- 2018/03/22 06:00 CRDT- 2017/05/16 06:00 PHST- 2016/07/18 00:00 [received] PHST- 2017/01/05 00:00 [accepted] PHST- 2017/05/16 06:00 [pubmed] PHST- 2018/03/22 06:00 [medline] PHST- 2017/05/16 06:00 [entrez] AID - S0755-4982(17)30105-7 [pii] AID - 10.1016/j.lpm.2017.01.020 [doi] PST - ppublish SO - Presse Med. 2017 May;46(5):547-550. doi: 10.1016/j.lpm.2017.01.020. Epub 2017 May 10. PMID- 18645312 OWN - NLM STAT- MEDLINE DCOM- 20080909 LR - 20131121 IS - 1533-0311 (Electronic) IS - 0193-1091 (Linking) VI - 30 IP - 4 DP - 2008 Aug TI - Nodular scleroderma: a report of 2 cases. PG - 385-8 LID - 10.1097/DAD.0b013e3181766177 [doi] AB - Nodular scleroderma, also known as keloidal scleroderma, is a rare form of scleroderma that may occur with either systemic sclerosis or localized scleroderma. Clinically, this disorder is characterized by keloidal nodules that form in sclerodermatous areas. These nodules may histologically show the presence of keloidal collagen. Because of the rarity of this condition, clinicians may not be familiar with the clinical and histologic features relevant to this scleroderma variant. In this report, we describe 2 cases of nodular scleroderma. FAU - Wriston, Cooper C AU - Wriston CC AD - Division of Dermatopathology, Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA. FAU - Rubin, Adam I AU - Rubin AI FAU - Elenitsas, Rosalie AU - Elenitsas R FAU - Crawford, Glen H AU - Crawford GH LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Dermatopathol JT - The American Journal of dermatopathology JID - 7911005 RN - 0 (Anti-Inflammatory Agents) RN - 1ZK20VI6TY (Triamcinolone) SB - IM MH - Adult MH - Anti-Inflammatory Agents/therapeutic use MH - Arthralgia/complications MH - Female MH - Humans MH - Keloid/complications/drug therapy/*pathology MH - Male MH - Middle Aged MH - Pericarditis/complications MH - Raynaud Disease/complications MH - Scleroderma, Systemic/complications/drug therapy/*pathology MH - Triamcinolone/therapeutic use EDAT- 2008/07/23 09:00 MHDA- 2008/09/10 09:00 CRDT- 2008/07/23 09:00 PHST- 2008/07/23 09:00 [pubmed] PHST- 2008/09/10 09:00 [medline] PHST- 2008/07/23 09:00 [entrez] AID - 00000372-200808000-00011 [pii] AID - 10.1097/DAD.0b013e3181766177 [doi] PST - ppublish SO - Am J Dermatopathol. 2008 Aug;30(4):385-8. doi: 10.1097/DAD.0b013e3181766177. PMID- 19806949 OWN - NLM STAT- MEDLINE DCOM- 20091201 LR - 20091007 IS - 1027-6661 (Print) IS - 1027-6661 (Linking) VI - 15 IP - 2 DP - 2009 TI - [Nonreconstructive revascularizing operations in limb ischaemia]. PG - 108-12 AB - Presented herein are the findings of experimental studies substantiating interrelationship between osteogenesis and blood circulation, as well as effects of surgical interventions based on excitation of reparative regeneration of the bone and aimed at improvement of blood circulation in the extremities. Reflected also are the main indications for the use of each operative intervention concerned. Based on the clinical findings obtained by follow up of 1,477 patients operated on for limb ischaemia secondary to obliterating arterial lesions efficacy of the methods of treatment devised is demonstrated. FAU - Shevtsov, V I AU - Shevtsov VI FAU - Popkov, A V AU - Popkov AV FAU - Bunov, V S AU - Bunov VS LA - rus PT - Comparative Study PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Angiol Sosud Khir JT - Angiologiia i sosudistaia khirurgiia = Angiology and vascular surgery JID - 9604504 SB - IM MH - Animals MH - Arteriosclerosis Obliterans/*surgery MH - Bone Regeneration MH - Bone and Bones/*blood supply/*surgery MH - Collateral Circulation MH - Dogs MH - Extremities/*blood supply MH - Follow-Up Studies MH - Humans MH - Ischemia/*surgery MH - Osteogenesis MH - Prognosis MH - Raynaud Disease/*surgery MH - Thromboangiitis Obliterans/*surgery MH - Time Factors MH - Treatment Outcome EDAT- 2009/10/08 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/10/08 06:00 PHST- 2009/10/08 06:00 [entrez] PHST- 2009/10/08 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] PST - ppublish SO - Angiol Sosud Khir. 2009;15(2):108-12. PMID- 18478312 OWN - NLM STAT- MEDLINE DCOM- 20090331 LR - 20211020 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 10 DP - 2008 Oct TI - Asymmetric scleroderma in a CVA patient. PG - 1321-3 LID - 10.1007/s10067-008-0915-z [doi] AB - We describe a systemic sclerosis and cerebral vascular accident case in which the cutaneous manifestation and the distal acroosteolysis occurred in an asymmetrical way in the non-paretic limb. The subsequent sclerodermic alterations and the acroosteolysis acquired an asymmetric pattern, sparing the patient's hemiparetic side. Although a number of definitions of this protective effect may be found in other rheumatic diseases, such as rheumatoid arthritis and gout, we found in the literature only one previous case describing the protective effect of the hemiplegia in scleroderma. FAU - Azevedo, V F AU - Azevedo VF AD - Rheumatology Unit, Hospital de Clínicas-Federal University of Paraná, Curitiba, Paraná, Brazil. valderilio@hotmail.com FAU - Mueller, C AU - Mueller C FAU - Aragão, S C AU - Aragão SC LA - eng PT - Case Reports PT - Journal Article DEP - 20080514 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Acro-Osteolysis/*pathology MH - Female MH - Hemiplegia/complications/etiology MH - Humans MH - Middle Aged MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications/*pathology MH - Stroke/*complications EDAT- 2008/05/15 09:00 MHDA- 2009/04/01 09:00 CRDT- 2008/05/15 09:00 PHST- 2008/03/11 00:00 [received] PHST- 2008/04/21 00:00 [accepted] PHST- 2008/04/20 00:00 [revised] PHST- 2008/05/15 09:00 [pubmed] PHST- 2009/04/01 09:00 [medline] PHST- 2008/05/15 09:00 [entrez] AID - 10.1007/s10067-008-0915-z [doi] PST - ppublish SO - Clin Rheumatol. 2008 Oct;27(10):1321-3. doi: 10.1007/s10067-008-0915-z. Epub 2008 May 14. PMID- 21798623 OWN - NLM STAT- MEDLINE DCOM- 20120410 LR - 20210409 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 66 IP - 3 DP - 2012 Mar TI - Pediatric erythromelalgia: a retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period. PG - 416-23 LID - 10.1016/j.jaad.2011.01.010 [doi] AB - BACKGROUND: Erythromelalgia has not been well characterized in the pediatric population. OBJECTIVE: We sought to review our experience of erythromelalgia in the pediatric age group. METHODS: We conducted a retrospective review of patients 18 years of age and younger with a diagnosis of erythromelalgia who were examined at Mayo Clinic in Rochester, MN, from 1970 to 2007. RESULTS: The records of 32 patients (girls, 22 [69%]) were evaluated. Mean age was 14.1 years (range, 5-18 years) and mean time to diagnosis was 5.2 years. Seven patients (22%) had a first-degree relative with erythromelalgia; 4 were from the same family. Physical activity was limited because of discomfort in 21 patients (66%) and school attendance was affected in 11 patients (34%). Noninvasive vascular studies, which compared temperature, laser Doppler flow, and transcutaneous oximetry in the toes, identified vascular abnormalities in 13 (93%) of 14 patients. Neurophysiologic studies with autonomic reflex screening (including quantitative sudomotor axon reflex test and thermoregulatory sweat testing) showed evidence of a small-fiber neuropathy involving the skin in 10 (59%) of 17 patients studied; there was no evidence of large-fiber neuropathy in 20 patients in whom electromyographic and nerve conduction studies were performed. Topical lidocaine was the most commonly prescribed treatment (44%). Fifteen patients were monitored for an average of 9.1 years (median, 5.0 years; range, 0.4-23.7 years). At last follow-up, 5 patients had stable disease, 4 showed improvement, two had resolution, one reported worsening of symptoms, and 3 had died (one suicide). LIMITATIONS: Conclusions are limited because this was a retrospective chart review. CONCLUSION: Erythromelalgia in pediatric patients is associated with substantial morbidity and even death. The majority of cases are not inherited. Most patients studied have associated small-fiber neuropathy. The disease course is variable. A reliable and safe treatment has not been determined. CI - Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. FAU - Cook-Norris, Robert H AU - Cook-Norris RH AD - Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA. FAU - Tollefson, Megha M AU - Tollefson MM FAU - Cruz-Inigo, Andres E AU - Cruz-Inigo AE FAU - Sandroni, Paola AU - Sandroni P FAU - Davis, Mark D P AU - Davis MD FAU - Davis, Dawn M R AU - Davis DM LA - eng PT - Journal Article DEP - 20110727 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Anesthetics, Local) RN - 98PI200987 (Lidocaine) SB - IM MH - Adolescent MH - Anesthetics, Local/therapeutic use MH - Cellulitis/diagnosis/mortality MH - Child MH - Child, Preschool MH - Comorbidity MH - Disease Progression MH - Electromyography MH - Erythromelalgia/*diagnosis/*drug therapy/mortality MH - Female MH - Follow-Up Studies MH - Humans MH - Lidocaine/*therapeutic use MH - Male MH - Nerve Fibers/physiology MH - Neural Conduction MH - Oximetry MH - Raynaud Disease/diagnosis/mortality MH - Retrospective Studies MH - Treatment Outcome EDAT- 2011/07/30 06:00 MHDA- 2012/04/11 06:00 CRDT- 2011/07/30 06:00 PHST- 2010/05/28 00:00 [received] PHST- 2011/01/03 00:00 [revised] PHST- 2011/07/27 00:00 [accepted] PHST- 2011/07/30 06:00 [entrez] PHST- 2011/07/30 06:00 [pubmed] PHST- 2012/04/11 06:00 [medline] AID - S0190-9622(11)00041-7 [pii] AID - 10.1016/j.jaad.2011.01.010 [doi] PST - ppublish SO - J Am Acad Dermatol. 2012 Mar;66(3):416-23. doi: 10.1016/j.jaad.2011.01.010. Epub 2011 Jul 27. PMID- 26808752 OWN - NLM STAT- MEDLINE DCOM- 20170913 LR - 20170913 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 77 IP - 3 DP - 2016 Sep TI - The Role of Botulinum Toxin A in the Treatment of Raynaud Phenomenon. PG - 318-23 LID - 10.1097/SAP.0000000000000715 [doi] AB - Raynaud phenomenon (RP) is a transient digital ischemia that occurs after exposure to cold temperature or emotional distress. It presents with a triphasic course: the initial white phase is followed by cyanotic discoloration and, subsequently, erythema. The attacks may be associated with pain, paresthesia, and complicate with nonhealing ulceration often leading to amputation. To date, there are no clear-cut therapeutic guidelines and many medications are used off-label. Encouraging results were reported with the use of botulinum neurotoxin-A (BoNT-A). However, there is still ongoing debate regarding indications, contraindications, best injection technique, and mechanism of action. The aim of this study was to address these issues by providing an up-to-date and detailed overview of the use of BoNT-A in RP.A PubMed database search was conducted. The available studies and techniques were evaluated and compared.The search yielded a total of 29 studies. Ten papers, published between 2004 and 2014, were considered relevant. A total of 128 patients underwent BoNT-A injections. Seventy-five percent to 100 % of the patients reported pain reduction after treatment. Healing of ulcers was reported in 75% to 100% of the affected patients. The most common complication was temporary hand weakness, with an average incidence of 14.1%. Injections targeting the neurovascular bundle at or slightly proximal to the A1 pulley were the most commonly performed.Botulinum neurotoxin-A injection proved to be a valid approach in both primary and secondary RP. The available evidence shows the achievement of both symptomatic and functional improvements in this debilitating condition. However, the patient should be adequately informed about the risk of transient hand weakness. FAU - Segreto, Francesco AU - Segreto F AD - From the Department of Plastic, Reconstructive and Aesthetic Surgery, "Campus Bio-Medico di Roma" University, Rome, Italy. FAU - Marangi, Giovanni Francesco AU - Marangi GF FAU - Cerbone, Vincenzo AU - Cerbone V FAU - Persichetti, Paolo AU - Persichetti P LA - eng PT - Journal Article PT - Review PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Botulinum Toxins, Type A/*therapeutic use MH - Humans MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Treatment Outcome EDAT- 2016/01/26 06:00 MHDA- 2017/09/14 06:00 CRDT- 2016/01/26 06:00 PHST- 2016/01/26 06:00 [entrez] PHST- 2016/01/26 06:00 [pubmed] PHST- 2017/09/14 06:00 [medline] AID - 10.1097/SAP.0000000000000715 [doi] PST - ppublish SO - Ann Plast Surg. 2016 Sep;77(3):318-23. doi: 10.1097/SAP.0000000000000715. PMID- 23994112 OWN - NLM STAT- MEDLINE DCOM- 20141103 LR - 20140217 IS - 1873-5967 (Electronic) IS - 1386-6532 (Linking) VI - 59 IP - 3 DP - 2014 Mar TI - Virus infection and autoimmunity: is there a cause-and-effect relationship? PG - 137-40 LID - S1386-6532(13)00326-0 [pii] LID - 10.1016/j.jcv.2013.08.002 [doi] FAU - Magira, Eleni E AU - Magira EE AD - Department of First Critical Care, Evangelismos Hospital, School of Medicine, National University of Athens, Athens, Greece. Electronic address: elmagira@yahoo.com. FAU - Pitsolis, Theodoros AU - Pitsolis T AD - Department of First Critical Care, Evangelismos Hospital, School of Medicine, National University of Athens, Athens, Greece. FAU - Delimpasi, Sosana AU - Delimpasi S AD - Department of Hematology, Evangelismos Hospital, Athens, Greece. FAU - Vourlakou, Christina AU - Vourlakou C AD - Department of Pathology, Evangelismos Hospital, Athens, Greece. FAU - Vlachoyiannopoulos, Panayiotis AU - Vlachoyiannopoulos P AD - Department of Pathophysiology, Laiko Hospital, School of Medicine, National University of Athens, Athens, Greece. FAU - Zakynthinos, Spyros AU - Zakynthinos S AD - Department of First Critical Care, Evangelismos Hospital, School of Medicine, National University of Athens, Athens, Greece. LA - eng PT - Case Reports PT - Journal Article DEP - 20130812 PL - Netherlands TA - J Clin Virol JT - Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology JID - 9815671 RN - Sclerocornea SB - IM MH - Autoimmunity MH - *Colitis MH - Cornea/*abnormalities MH - *Corneal Diseases MH - Cytomegalovirus MH - *Cytomegalovirus Infections MH - Female MH - Humans MH - Length of Stay MH - Middle Aged MH - *Pneumonia, Ventilator-Associated MH - Raynaud Disease MH - Ventilator Weaning OTO - NOTNLM OT - Cytomegalovirus OT - Scleroderma renal crisis OT - Ventilator-associated pneumonia EDAT- 2013/09/03 06:00 MHDA- 2014/11/05 06:00 CRDT- 2013/09/03 06:00 PHST- 2013/05/23 00:00 [received] PHST- 2013/07/31 00:00 [revised] PHST- 2013/08/03 00:00 [accepted] PHST- 2013/09/03 06:00 [entrez] PHST- 2013/09/03 06:00 [pubmed] PHST- 2014/11/05 06:00 [medline] AID - S1386-6532(13)00326-0 [pii] AID - 10.1016/j.jcv.2013.08.002 [doi] PST - ppublish SO - J Clin Virol. 2014 Mar;59(3):137-40. doi: 10.1016/j.jcv.2013.08.002. Epub 2013 Aug 12. PMID- 21790279 OWN - NLM STAT- MEDLINE DCOM- 20111115 LR - 20110727 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 7 IP - 4 DP - 2011 Jul TI - Evaluating microangiopathy in systemic sclerosis: what have we learnt and what is left to discover? PG - 395-7 LID - 10.1586/eci.11.35 [doi] FAU - Cutolo, Maurizio AU - Cutolo M FAU - Sulli, Alberto AU - Sulli A FAU - Smith, Vanessa AU - Smith V LA - eng PT - Editorial PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - Apoptosis/immunology MH - Blood Vessels/*immunology/pathology MH - Diagnosis, Differential MH - Early Diagnosis MH - Endothelial Cells/pathology MH - Humans MH - Inflammation MH - Microcirculation/immunology MH - *Microscopic Angioscopy MH - Raynaud Disease MH - Scleroderma, Systemic/*diagnosis/*immunology/pathology/physiopathology MH - Skin/*blood supply/immunology MH - Thrombotic Microangiopathies EDAT- 2011/07/28 06:00 MHDA- 2011/11/16 06:00 CRDT- 2011/07/28 06:00 PHST- 2011/07/28 06:00 [entrez] PHST- 2011/07/28 06:00 [pubmed] PHST- 2011/11/16 06:00 [medline] AID - 10.1586/eci.11.35 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2011 Jul;7(4):395-7. doi: 10.1586/eci.11.35. PMID- 20506174 OWN - NLM STAT- MEDLINE DCOM- 20101019 LR - 20111209 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 62 IP - 9 DP - 2010 Sep TI - A 39-year-old woman with lupus, myositis, and a recalcitrant vasculopathy. PG - 1351-6 LID - 10.1002/acr.20236 [doi] FAU - Sokolove, Jeremy AU - Sokolove J AD - Stanford University School of Medicine, Palo Alto, California, USA. sokolove@stanford.edu FAU - Copland, Andrew AU - Copland A FAU - Shirvani, Shervin AU - Shirvani S FAU - Brown, Janice AU - Brown J FAU - Posley, Keith AU - Posley K FAU - Chung, Lorinda AU - Chung L LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Diagnosis, Differential MH - Female MH - Fever of Unknown Origin/complications/diagnosis/microbiology MH - Fingers MH - Hand/blood supply MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Muscle, Skeletal/pathology MH - Mycobacterium tuberculosis/*isolation & purification MH - Myositis/complications/*diagnosis/drug therapy/microbiology MH - Peripheral Vascular Diseases/complications/*diagnosis/drug therapy MH - Raynaud Disease/*complications MH - Skin Ulcer/complications/*diagnosis MH - Thigh MH - Treatment Outcome EDAT- 2010/05/28 06:00 MHDA- 2010/10/20 06:00 CRDT- 2010/05/28 06:00 PHST- 2010/05/28 06:00 [entrez] PHST- 2010/05/28 06:00 [pubmed] PHST- 2010/10/20 06:00 [medline] AID - 10.1002/acr.20236 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2010 Sep;62(9):1351-6. doi: 10.1002/acr.20236. PMID- 18412718 OWN - NLM STAT- MEDLINE DCOM- 20080519 LR - 20100429 IS - 1601-5037 (Electronic) IS - 1601-5029 (Linking) VI - 6 IP - 2 DP - 2008 May TI - Scleroderma: considerations for dental hygienists. PG - 77-83 LID - 10.1111/j.1601-5037.2008.00292.x [doi] AB - Scleroderma, the general name of a group of progressive diseases affecting the connective tissues is the most deadly of the varying connective tissue disorders. Characterized by abnormal thickening of the skin, this collagen-vascular disease is associated with immune dysfunction. Hallmark signs of scleroderma include fibrosis, vascular instability and initial inflammation resulting from excessive collagen deposition. Oral facial involvement is considerable, necessitating adaptations in patient oral self-care and influencing oral hygiene. Appropriate dental hygiene management of patients with this autoimmune disorder requires an understanding of clinical characteristics, the recognition of oral facial involvement, treatment considerations and pharmacological interventions. With this information, dental hygienists will be better prepared to provide compassionate, safe and effective dental hygiene management and care to patients with scleroderma. FAU - Tolle, S L AU - Tolle SL AD - School of Dental Hygiene, Old Dominion University, Norfolk, VA 23529-0499, USA. ltolle@odu.edu LA - eng PT - Journal Article PT - Review PL - England TA - Int J Dent Hyg JT - International journal of dental hygiene JID - 101168070 SB - IM MH - *Dental Care for Chronically Ill MH - *Dental Hygienists MH - Dental Prophylaxis/*methods MH - Humans MH - Mandibular Diseases/etiology MH - Microstomia/*etiology MH - Periodontal Ligament/pathology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/pathology MH - Trigeminal Nerve Diseases/etiology RF - 37 EDAT- 2008/04/17 09:00 MHDA- 2008/05/20 09:00 CRDT- 2008/04/17 09:00 PHST- 2008/04/17 09:00 [pubmed] PHST- 2008/05/20 09:00 [medline] PHST- 2008/04/17 09:00 [entrez] AID - IDH292 [pii] AID - 10.1111/j.1601-5037.2008.00292.x [doi] PST - ppublish SO - Int J Dent Hyg. 2008 May;6(2):77-83. doi: 10.1111/j.1601-5037.2008.00292.x. PMID- 31474612 OWN - NLM STAT- MEDLINE DCOM- 20200420 LR - 20200420 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 9 DP - 2019 Sep TI - Might Nailfold Capillaroscopy Be a "Proxy" for Lung Involvement in Connective Tissue Diseases? PG - 1061-1063 LID - 10.3899/jrheum.181408 [doi] FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center, Ghent, Belgium; vanessa.smith@ugent.be. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Institute for Research and Health Care San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy. LA - eng PT - Comment PT - Editorial PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CON - J Rheumatol. 2019 Sep;46(9):1109-1116. doi: 10.3899/jrheum.180615. PMID: 30554151 MH - *Connective Tissue Diseases MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease MH - *Respiration Disorders MH - Respiratory Function Tests EDAT- 2019/09/03 06:00 MHDA- 2020/04/21 06:00 CRDT- 2019/09/03 06:00 PHST- 2019/09/03 06:00 [entrez] PHST- 2019/09/03 06:00 [pubmed] PHST- 2020/04/21 06:00 [medline] AID - 46/9/1061 [pii] AID - 10.3899/jrheum.181408 [doi] PST - ppublish SO - J Rheumatol. 2019 Sep;46(9):1061-1063. doi: 10.3899/jrheum.181408. PMID- 21855729 OWN - NLM STAT- MEDLINE DCOM- 20111223 LR - 20110822 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 29 IP - 5 DP - 2011 Sep-Oct TI - Skin signs of systemic diseases. PG - 531-40 LID - 10.1016/j.clindermatol.2010.09.021 [doi] AB - The skin should not be considered as an isolated organ but rather as a definite functioning system that communicates with the internal environment. Skin signs of systemic diseases occur frequently and sometimes feature the first symptoms of an internal disease; furthermore, these manifestations may be the sole expressions of otherwise asymptomatic systemic disorders. A number of dermatologic signs, symptoms, and disorders can be invaluable as markers of systemic disease. Although a plethora of specialized modern diagnostic tests are available, the skin still remains the only organ of the body that is immediately and completely accessible to direct clinical examination. This contribution reviews the skin signs of systemic diseases. The description of the clinical features of skin lesions observed in several internal diseases will be useful to general physicians, internists, and dermatologists in the diagnosis of a systemic disease. CI - Copyright © 2011 Elsevier Inc. All rights reserved. FAU - Rigopoulos, Dimitris AU - Rigopoulos D AD - Department of Dermatology, University of Athens, Andreas Sygros Hospital, 5th Ionos Dragoumi St, 16121 Athens, Greece. drigop@hol.gr FAU - Larios, George AU - Larios G FAU - Katsambas, Andreas AU - Katsambas A LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 SB - IM MH - Arthritis/diagnosis MH - Cardiovascular Diseases/diagnosis MH - Connective Tissue Diseases/diagnosis MH - Cutis Laxa/diagnosis MH - Ehlers-Danlos Syndrome/diagnosis MH - Endocrine System Diseases/diagnosis MH - Female MH - Gastrointestinal Diseases/diagnosis MH - Humans MH - LEOPARD Syndrome MH - Lung Diseases/diagnosis MH - Male MH - Mucocutaneous Lymph Node Syndrome/diagnosis MH - Raynaud Disease/diagnosis MH - Skin Diseases/*diagnosis EDAT- 2011/08/23 06:00 MHDA- 2011/12/24 06:00 CRDT- 2011/08/23 06:00 PHST- 2011/08/23 06:00 [entrez] PHST- 2011/08/23 06:00 [pubmed] PHST- 2011/12/24 06:00 [medline] AID - S0738-081X(10)00173-2 [pii] AID - 10.1016/j.clindermatol.2010.09.021 [doi] PST - ppublish SO - Clin Dermatol. 2011 Sep-Oct;29(5):531-40. doi: 10.1016/j.clindermatol.2010.09.021. PMID- 30442823 OWN - NLM STAT- MEDLINE DCOM- 20200626 LR - 20200626 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 46 IP - 3 DP - 2019 Mar TI - Nailfold Capillaroscopy Characteristics of Antisynthetase Syndrome and Possible Clinical Associations: Results of a Multicenter International Study. PG - 279-284 LID - 10.3899/jrheum.180355 [doi] AB - OBJECTIVE: To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. METHODS: We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. RESULTS: NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. CONCLUSION: NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS. FAU - Sebastiani, Marco AU - Sebastiani M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Triantafyllias, Konstantinos AU - Triantafyllias K AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. ktriantafyllias@gmail.com. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. ktriantafyllias@gmail.com. FAU - Manfredi, Andreina AU - Manfredi A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - González-Gay, Miguel Angel AU - González-Gay MA AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Palmou-Fontana, Natalia AU - Palmou-Fontana N AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Cassone, Giulia AU - Cassone G AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Drott, Ulrich AU - Drott U AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Delbrück, Christiane AU - Delbrück C AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Bertolazzi, Chiara AU - Bertolazzi C AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Nuño, Laura AU - Nuño L AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Giannini, Margherita AU - Giannini M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Iannone, Florenzo AU - Iannone F AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Vicente, Esther F AU - Vicente EF AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Castañeda, Santos AU - Castañeda S AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Trallero Araguas, Ernesto AU - Trallero Araguas E AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Emmi, Giacomo AU - Emmi G AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Iuliano, Annamaria AU - Iuliano A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Bauhammer, Jutta AU - Bauhammer J AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Miehle, Nikolaus AU - Miehle N AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Parisi, Simone AU - Parisi S AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Cavagna, Lorenzo AU - Cavagna L AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Codullo, Veronica AU - Codullo V AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Lopez-Longo, Francisco Javier AU - Lopez-Longo FJ AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Martínez-Barrio, Julia AU - Martínez-Barrio J AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Nieto-González, Juan Carlos AU - Nieto-González JC AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Vichi, Silvia AU - Vichi S AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Confalonieri, Marco AU - Confalonieri M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Tomietto, Paola AU - Tomietto P AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Bergner, Raoul AU - Bergner R AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Sulli, Alberto AU - Sulli A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Bonella, Francesco AU - Bonella F AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Furini, Federica AU - Furini F AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Scirè, Carlo Alberto AU - Scirè CA AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Bortoluzzi, Alessandra AU - Bortoluzzi A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Specker, Christof AU - Specker C AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Barsotti, Simone AU - Barsotti S AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Neri, Rossella AU - Neri R AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Mosca, Marta AU - Mosca M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Caproni, Marzia AU - Caproni M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Weinmann-Menke, Julia AU - Weinmann-Menke J AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Schwarting, Andreas AU - Schwarting A AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Smith, Vanessa AU - Smith V AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. FAU - Cutolo, Maurizio AU - Cutolo M AD - From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium. AD - M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital. CN - American and European Network of Antisynthetase Syndrome Collaborative Group LA - eng PT - Journal Article PT - Multicenter Study DEP - 20181115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Aged MH - Amino Acyl-tRNA Synthetases/immunology MH - Antibodies, Antinuclear/blood MH - Capillaries/diagnostic imaging MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Myositis/*diagnostic imaging/*immunology MH - Nails/blood supply MH - Raynaud Disease/diagnostic imaging/immunology MH - Retrospective Studies OTO - NOTNLM OT - ANTISYNTHETASE ANTIBODIES OT - ANTISYNTHETASE SYNDROME OT - NAILFOLD VIDEOCAPILLAROSCOPY OT - RAYNAUD PHENOMENON OT - SYSTEMIC SCLEROSIS PATTERN EDAT- 2018/11/18 06:00 MHDA- 2020/06/27 06:00 CRDT- 2018/11/17 06:00 PHST- 2018/08/13 00:00 [accepted] PHST- 2018/11/18 06:00 [pubmed] PHST- 2020/06/27 06:00 [medline] PHST- 2018/11/17 06:00 [entrez] AID - jrheum.180355 [pii] AID - 10.3899/jrheum.180355 [doi] PST - ppublish SO - J Rheumatol. 2019 Mar;46(3):279-284. doi: 10.3899/jrheum.180355. Epub 2018 Nov 15. PMID- 36967074 OWN - NLM STAT- MEDLINE DCOM- 20230501 LR - 20230510 IS - 2210-741X (Electronic) IS - 2210-7401 (Linking) VI - 47 IP - 5 DP - 2023 May TI - Noncirrhotic portal hypertension in primary biliary cholangitis with coexisting CREST syndrome. PG - 102114 LID - S2210-7401(23)00039-6 [pii] LID - 10.1016/j.clinre.2023.102114 [doi] AB - Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion. Liver biopsy excluded cirrhosis, resulting in a diagnosis of noncirrhotic portal hypertension. This case report describes the pathophysiology of presinusoidal portal hypertension as a rare complication of PBC and its association with coexisiting CREST. CI - Copyright © 2023 Elsevier Masson SAS. All rights reserved. FAU - Malkani, Kabir V AU - Malkani KV AD - Division of Gastroenterology and Hepatology, Weill Cornell Medical College, New York, NY, USA. Electronic address: Ycs9004@nyp.org. FAU - Jesudian, Arun B AU - Jesudian AB AD - Division of Gastroenterology and Hepatology, Weill Cornell Medical College, New York, NY, USA. FAU - Luo, Jean J AU - Luo JJ AD - Department of Pathology, New York Presbyterian Queens, Flushing, USA. FAU - Schonfeld, Emily A AU - Schonfeld EA AD - Division of Gastroenterology and Hepatology, Weill Cornell Medical College, New York, NY, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230324 PL - France TA - Clin Res Hepatol Gastroenterol JT - Clinics and research in hepatology and gastroenterology JID - 101553659 SB - IM MH - Adult MH - Humans MH - *CREST Syndrome/complications MH - *Liver Cirrhosis, Biliary/complications MH - *Esophageal and Gastric Varices/complications MH - Gastrointestinal Hemorrhage/etiology MH - *Hypertension, Portal/complications MH - Liver Cirrhosis/complications MH - *Portasystemic Shunt, Transjugular Intrahepatic MH - Treatment Outcome OTO - NOTNLM OT - CREST syndrome OT - Noncirrhotic portal hypertension OT - Primary biliary cholangitis OT - TIPS OT - Variceal bleeding COIS- Declaration of Competing Interest This work has not been presented or published elsewhere. The authors of this manuscript have no disclaimers or conflicts of interest. EDAT- 2023/03/27 06:00 MHDA- 2023/05/01 06:42 CRDT- 2023/03/26 20:28 PHST- 2023/02/09 00:00 [received] PHST- 2023/03/12 00:00 [revised] PHST- 2023/03/21 00:00 [accepted] PHST- 2023/05/01 06:42 [medline] PHST- 2023/03/27 06:00 [pubmed] PHST- 2023/03/26 20:28 [entrez] AID - S2210-7401(23)00039-6 [pii] AID - 10.1016/j.clinre.2023.102114 [doi] PST - ppublish SO - Clin Res Hepatol Gastroenterol. 2023 May;47(5):102114. doi: 10.1016/j.clinre.2023.102114. Epub 2023 Mar 24. PMID- 28340695 OWN - NLM STAT- MEDLINE DCOM- 20190603 LR - 20220409 IS - 2452-3186 (Electronic) IS - 2452-3186 (Linking) VI - 65 IP - 1 DP - 2017 Jan-Mar TI - Long-term follow-up after autologous adipose-derived stromal vascular fraction injection into fingers in systemic sclerosis patients. PG - 40-43 LID - S2452-3186(16)30073-3 [pii] LID - 10.1016/j.retram.2016.10.006 [doi] AB - INTRODUCTION: Hand involvement confers a substantial handicap in work and daily activities in patients with Systemic sclerosis (SSc). Autologous adipose-derived stromal vascular fraction is as an easily accessible source of cells with regenerative effects. We previously performed a phase I open-label clinical trial (NTC01813279) assessing the safety of subcutaneous injection of autologous adipose-derived stromal vascular fraction. Six and 12-month data have been reported. As patients were followed in our medical centre, we report their longer-term outcome beyond the end of the trial. PATIENTS AND METHOD: Twelve females, mean age 54.5±10.3 years, initially enrolled in the clinical trial were assessed during a scheduled medical care, which took place between 22 and 30months after treatment. RESULTS: Multiple patient-reported outcomes showed sustained improvement, in comparison with the assessment performed just before surgery: 62.5% in the Cochin Hand Function Scale, 51.1% in the Scleroderma Health Assessment Questionnaire, 33.1% in hand pain, and 88.3% in the Raynaud Condition Score. A decrease in the number of digital ulcers number was noted. Mobility, strength and fibrosis of the hand also showed improvement. None of the 8 patients who had previously received iloprost infusion required new infusion. CONCLUSION: Despite the limits of an open label study, the data are in favour of the long-term safety of the adipose-derived stromal vascular fraction injection. Two randomized double blind, placebo-controlled trials of this therapeutic agent are ongoing in the USA (NCT02396238) and in France (NCT02558543) and will help determine the place of this innovative therapy for SSc patients. CI - Copyright © 2016 Elsevier Masson SAS. All rights reserved. FAU - Daumas, A AU - Daumas A AD - Internal Medicine Department, Assistance publique-Hôpitaux de Marseille (AP-HM), CHU of Marseille, 13915 Marseille, France. FAU - Magalon, J AU - Magalon J AD - Culture and Cell Therapy Laboratory, INSERM CBT-1409, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Aix Marseille University, France; Inserm, VRCM, UMR_S 1076, Aix Marseille University, 13005 Marseille, France. FAU - Jouve, E AU - Jouve E AD - Centre d'investigation clinique - centre de pharmacologie clinique et d'évaluations thérapeutiques (CIC-CPCET), AP-HM, CHU of Marseille, 13005 Marseille, France. FAU - Truillet, R AU - Truillet R AD - Centre d'investigation clinique - centre de pharmacologie clinique et d'évaluations thérapeutiques (CIC-CPCET), AP-HM, CHU of Marseille, 13005 Marseille, France. FAU - Casanova, D AU - Casanova D AD - Plastic Surgery Department, AP-HM, CHU of Marseille, 13005 Marseille, France. FAU - Giraudo, L AU - Giraudo L AD - Culture and Cell Therapy Laboratory, INSERM CBT-1409, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Aix Marseille University, France. FAU - Veran, J AU - Veran J AD - Culture and Cell Therapy Laboratory, INSERM CBT-1409, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Aix Marseille University, France. FAU - Benyamine, A AU - Benyamine A AD - Internal Medicine Department, Assistance publique-Hôpitaux de Marseille (AP-HM), CHU of Marseille, 13915 Marseille, France. FAU - Dignat-George, F AU - Dignat-George F AD - Hematology and Vascular Biology Laboratory, AP-HM, CHU of Marseille, 13005 Marseille, France; Inserm, VRCM, UMR_S 1076, Aix Marseille University, 13005 Marseille, France. FAU - Magalon, G AU - Magalon G AD - Centre d'investigation clinique - centre de pharmacologie clinique et d'évaluations thérapeutiques (CIC-CPCET), AP-HM, CHU of Marseille, 13005 Marseille, France; Plastic Surgery Department, AP-HM, CHU of Marseille, 13005 Marseille, France. FAU - Sabatier, F AU - Sabatier F AD - Culture and Cell Therapy Laboratory, INSERM CBT-1409, Assistance publique-Hôpitaux de Marseille (AP-HM), 13005 Aix Marseille University, France; Inserm, VRCM, UMR_S 1076, Aix Marseille University, 13005 Marseille, France. FAU - Granel, B AU - Granel B AD - Internal Medicine Department, Assistance publique-Hôpitaux de Marseille (AP-HM), CHU of Marseille, 13915 Marseille, France; Inserm, VRCM, UMR_S 1076, Aix Marseille University, 13005 Marseille, France. Electronic address: bgranel@ap-hm.fr. LA - eng PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20161128 PL - France TA - Curr Res Transl Med JT - Current research in translational medicine JID - 101681234 SB - IM MH - Adipose Tissue/*cytology MH - Adult MH - Aged MH - Cell Fractionation MH - Endothelial Cells/cytology/transplantation MH - Female MH - *Fingers/pathology MH - Follow-Up Studies MH - Hand MH - Humans MH - Injections, Subcutaneous MH - Middle Aged MH - Patient Reported Outcome Measures MH - Raynaud Disease/etiology/*therapy MH - Scleroderma, Systemic/complications/*therapy MH - Skin Ulcer/etiology/therapy MH - Stromal Cells/*transplantation MH - Transplantation, Autologous MH - Treatment Outcome OTO - NOTNLM OT - Adipose tissue OT - Stromal vascular fraction OT - Systemic sclerosis EDAT- 2017/03/28 06:00 MHDA- 2019/06/04 06:00 CRDT- 2017/03/26 06:00 PHST- 2016/08/10 00:00 [received] PHST- 2016/10/14 00:00 [revised] PHST- 2016/10/17 00:00 [accepted] PHST- 2017/03/26 06:00 [entrez] PHST- 2017/03/28 06:00 [pubmed] PHST- 2019/06/04 06:00 [medline] AID - S2452-3186(16)30073-3 [pii] AID - 10.1016/j.retram.2016.10.006 [doi] PST - ppublish SO - Curr Res Transl Med. 2017 Jan-Mar;65(1):40-43. doi: 10.1016/j.retram.2016.10.006. Epub 2016 Nov 28. PMID- 20120409 OWN - NLM STAT- MEDLINE DCOM- 20100302 LR - 20100202 IS - 0350-6134 (Print) IS - 0350-6134 (Linking) VI - 33 Suppl 2 DP - 2009 Dec TI - Neurological manifestation of Fabry disease--a case report. PG - 177-9 AB - Fabry disease is an X-linked recessive glycolipid storage disease. It is caused by deficiency of the lysosomal enzyme alpha-galactosidase A and leads to the accumulation of the enzyme substrate, globotriasylceramide (Gb3) in many tissues including endothelial cells, pericytes and smooth muscle cells of blood vessels, renal epithelial cells, cardiac myocytes and numerous neuronal cells. In this report, we present 20-year-old male patient with ischemic stroke in pons. The case had previously been misdiagnosed as polimyositis and vasculitis. Angiokeratomas, neuropathic pain and ischemic stroke in young age suggested a Fabry disease. The diagnosis was confirmed biochemically and genetically. All young adults with stroke, especially if they have additional symptoms like angiokeratomas, proteinuria, neuropathic pain in toes and fingers should be tested for Fabry disease. FAU - Demarin, Vida AU - Demarin V AD - Department of Neurology, University Hospital 'Sestre milosrdnice", Zagreb, Croatia. FAU - Kes, Vanja Basić AU - Kes VB FAU - Bitunjac, Milan AU - Bitunjac M FAU - Ivanković, Mira AU - Ivanković M LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Coll Antropol JT - Collegium antropologicum JID - 8003354 SB - IM MH - Adult MH - Croatia MH - Diagnostic Errors MH - Diplopia/etiology MH - Fabry Disease/complications/genetics/*pathology MH - Humans MH - Male MH - Neuralgia/etiology MH - Pedigree MH - Polymyositis/diagnosis MH - Pons/*pathology MH - Raynaud Disease/diagnosis MH - Stroke/*etiology/pathology MH - Vasculitis/diagnosis MH - Vertigo/etiology EDAT- 2010/02/03 06:00 MHDA- 2010/03/03 06:00 CRDT- 2010/02/03 06:00 PHST- 2010/02/03 06:00 [entrez] PHST- 2010/02/03 06:00 [pubmed] PHST- 2010/03/03 06:00 [medline] PST - ppublish SO - Coll Antropol. 2009 Dec;33 Suppl 2:177-9. PMID- 29196550 OWN - NLM STAT- MEDLINE DCOM- 20190705 LR - 20190705 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 44 IP - 12 DP - 2017 Dec TI - Progression of Organ Involvement in Systemic Sclerosis Patients with Persistent "Late" Nailfold Capillaroscopic Pattern of Microangiopathy: A Prospective Study. PG - 1941-1942 LID - 10.3899/jrheum.170485 [doi] FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino. FAU - Ruaro, Barbara AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. FAU - Trombetta, Amelia Chiara AU - Trombetta AC AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino, Genoa, Italy. V. Smith is a Senior Clinical Investigator of the Research Foundation -Flanders, Brussels, Belgium (FWO) [1802915N]. C. Pizzorni, M. Cutolo, B. Ruaro, V. Smith, and A. Sulli are members of the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases. mcutolo@unige.it. LA - eng PT - Letter PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Aged MH - Capillaries/diagnostic imaging MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Prospective Studies MH - Raynaud Disease/*diagnostic imaging MH - Scleroderma, Systemic/*diagnostic imaging EDAT- 2017/12/03 06:00 MHDA- 2019/07/06 06:00 CRDT- 2017/12/03 06:00 PHST- 2017/12/03 06:00 [entrez] PHST- 2017/12/03 06:00 [pubmed] PHST- 2019/07/06 06:00 [medline] AID - 44/12/1941 [pii] AID - 10.3899/jrheum.170485 [doi] PST - ppublish SO - J Rheumatol. 2017 Dec;44(12):1941-1942. doi: 10.3899/jrheum.170485. PMID- 28602360 OWN - NLM STAT- MEDLINE DCOM- 20180627 LR - 20180723 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 47 IP - 3 DP - 2017 Dec TI - The role of platelets in autoimmunity, vasculopathy, and fibrosis: Implications for systemic sclerosis. PG - 409-417 LID - S0049-0172(17)30110-5 [pii] LID - 10.1016/j.semarthrit.2017.05.004 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, autoimmunity, and widespread dermal and visceral fibrosis. This article summarizes the current knowledge about the potential contribution of platelets in the disease process and the rationale of targeting platelets as an adjunct treatment for SSc. METHODS: We performed an electronic search (Medline) using the keywords platelets, systemic sclerosis, autoimmunity, fibrosis, Raynaud, and pulmonary arterial hypertension. RESULTS: The link that connects vasculopathy, autoimmunity, and fibrosis in SSc remains obscure. Experimental data suggest that platelets are not solely cell fragments regulating hemostasis but they have a pleiotropic role in several biologic processes including immune regulation, vasculopathy, fibrosis, and all key features of SSc. Platelets interplay with the impaired endothelium, can interact with immune cells, and they are storages of bioactive molecules involved in tissue injury and remodeling. The potential role of platelets in the pathogenesis of SSc is further supported by experimental data in animal models of SSc. Platelet-derived serotonin represents a novel target in SSc and serotonin blockade is currently being tested in clinical trials. CONCLUSION: Platelets may be actively involved in the pathogenesis of SSc by activating immune responses and facilitating the fibrotic process. However, definite conclusions cannot be drawn until more data from both basic and clinical research are available. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Ntelis, Konstantinos AU - Ntelis K AD - Division of Rheumatology, Department of Internal Medicine, University of Patras Medical School, Patras University Hospital, 26504 Rion, Patras, Greece. FAU - Solomou, Elena E AU - Solomou EE AD - Department of Internal Medicine, University of Patras Medical School, Patras University Hospital, Patras, Greece. FAU - Sakkas, Lazaros AU - Sakkas L AD - Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece. FAU - Liossis, Stamatis-Nick AU - Liossis SN AD - Division of Rheumatology, Department of Internal Medicine, University of Patras Medical School, Patras University Hospital, 26504 Rion, Patras, Greece. FAU - Daoussis, Dimitrios AU - Daoussis D AD - Division of Rheumatology, Department of Internal Medicine, University of Patras Medical School, Patras University Hospital, 26504 Rion, Patras, Greece. Electronic address: jimdaoussis@hotmail.com. LA - eng PT - Journal Article PT - Review DEP - 20170523 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Autoantibodies) RN - 333DO1RDJY (Serotonin) SB - IM MH - Animals MH - Autoantibodies/immunology MH - *Autoimmunity MH - B-Lymphocytes/immunology MH - Blood Platelets/*immunology/metabolism/pathology MH - Fibrosis/blood/complications/*immunology MH - Humans MH - Hypertension, Pulmonary/blood/complications MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*blood/complications MH - Serotonin/immunology OTO - NOTNLM OT - Autoimmunity OT - Fibrosis OT - Platelets OT - Scleroderma OT - Systemic sclerosis OT - Vasculopathy EDAT- 2017/06/13 06:00 MHDA- 2018/06/28 06:00 CRDT- 2017/06/13 06:00 PHST- 2017/02/11 00:00 [received] PHST- 2017/04/12 00:00 [revised] PHST- 2017/05/16 00:00 [accepted] PHST- 2017/06/13 06:00 [pubmed] PHST- 2018/06/28 06:00 [medline] PHST- 2017/06/13 06:00 [entrez] AID - S0049-0172(17)30110-5 [pii] AID - 10.1016/j.semarthrit.2017.05.004 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2017 Dec;47(3):409-417. doi: 10.1016/j.semarthrit.2017.05.004. Epub 2017 May 23. PMID- 25726305 OWN - NLM STAT- MEDLINE DCOM- 20160906 LR - 20151127 IS - 1578-8989 (Electronic) IS - 0025-7753 (Linking) VI - 145 IP - 11 DP - 2015 Dec 7 TI - [Large vessels vasculopathy in systemic sclerosis]. PG - 488-92 LID - S0025-7753(15)00036-6 [pii] LID - 10.1016/j.medcli.2014.12.016 [doi] AB - Vasculopathy in systemic sclerosis is a severe, in many cases irreversible, manifestation that can lead to amputation. While the classical clinical manifestations of the disease have to do with the involvement of microcirculation, proximal vessels of upper and lower limbs can also be affected. This involvement of large vessels may be related to systemic sclerosis, vasculitis or atherosclerotic, and the differential diagnosis is not easy. To conduct a proper and early diagnosis, it is essential to start prompt appropriate treatment. In this review, we examine the involvement of large vessels in scleroderma, an understudied manifestation with important prognostic and therapeutic implications. CI - Copyright © 2015 Elsevier España, S.L.U. All rights reserved. FAU - Tejera Segura, Beatriz AU - Tejera Segura B AD - Servicio de Reumatología, Hospital Universitario de Canarias, Santa Cruz de Tenerife, España. Electronic address: btejerasegura@gmail.com. FAU - Ferraz-Amaro, Iván AU - Ferraz-Amaro I AD - Servicio de Reumatología, Hospital Universitario de Canarias, Santa Cruz de Tenerife, España. LA - spa PT - English Abstract PT - Journal Article PT - Review TT - Vasculopatía de grandes vasos en la esclerosis sistémica. DEP - 20150226 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM CIN - Med Clin (Barc). 2016 Mar 18;146(6):282-3. doi: 10.1016/j.medcli.2015.10.021. PMID: 26723947 CIN - Med Clin (Barc). 2016 Mar 18;146(6):283. doi: 10.1016/j.medcli.2015.11.003. PMID: 26726114 MH - Arteriosclerosis/etiology MH - Arteritis/etiology MH - Diagnosis, Differential MH - Disease Progression MH - Endothelium, Vascular/physiopathology MH - Extremities/blood supply MH - Fingers/blood supply MH - Hand Dermatoses/etiology MH - Humans MH - Microcirculation MH - Neovascularization, Pathologic/etiology MH - Pericytes/pathology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/etiology MH - Vascular Diseases/*etiology OTO - NOTNLM OT - Esclerosis sistémica OT - Gran vaso OT - Large vessels OT - Systemic sclerosis OT - Vasculopathy OT - Vasculopatía EDAT- 2015/03/03 06:00 MHDA- 2016/09/07 06:00 CRDT- 2015/03/02 06:00 PHST- 2014/09/30 00:00 [received] PHST- 2014/11/25 00:00 [revised] PHST- 2014/12/11 00:00 [accepted] PHST- 2015/03/02 06:00 [entrez] PHST- 2015/03/03 06:00 [pubmed] PHST- 2016/09/07 06:00 [medline] AID - S0025-7753(15)00036-6 [pii] AID - 10.1016/j.medcli.2014.12.016 [doi] PST - ppublish SO - Med Clin (Barc). 2015 Dec 7;145(11):488-92. doi: 10.1016/j.medcli.2014.12.016. Epub 2015 Feb 26. PMID- 35364109 OWN - NLM STAT- MEDLINE DCOM- 20220603 LR - 20220607 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 142 DP - 2022 Jul TI - Capillaroscopic analysis of the microvascular status in mixed versus undifferentiated connective tissue disease. PG - 104367 LID - S0026-2862(22)00057-7 [pii] LID - 10.1016/j.mvr.2022.104367 [doi] AB - INTRODUCTION: Raynaud phenomenon (RP), typically, precede the clinical onset of systemic manifestations in several connective tissue diseases (CTDs). These autoimmune disorders usually share a microvascular damage whose alterations can be detected by nailfold videocapillaroscopy (NVC). The aim of the study was to compare the NVC microvascular status in Mixed Connective Tissue Disease (MCTD) versus the Undifferentiated Connective Tissue Disease (UCTD), and to search correlations between NVC findings and specific autoantibodies in UCTD patients. METHODS: Clinical data and NCV patterns were retrospectively obtained from the files of 46 MCTD patients, 47 stable UCTD patients and 51 individuals with primary RP (PRP) as controls collected in a central database (VideoCap®, DS Medica, Milan, Italy). ANA and ENA Abs were tested respectively by indirect immunofluorescence and enzyme-linked immunosorbent assay. RESULTS: "Scleroderma-like" (SSc-like) NVC pattern was significantly more frequent in MCTD than in UCTD patients (48% vs 11%, p < 0.001). Giant capillaries, abnormal shapes (i.e. neoangiogenesis) and lower capillary density were predominantly detected among MCTD versus UCTD patients (48% vs 11%, 49% vs 13%, 52% vs 9%, respectively, p < 0.001). The absolute number of capillaries was significantly lower in MCTD versus UCTD patients (mean 7 ± 1.7 SD vs mean 9.2 ± 1.3 SD, respectively, p < 0.001). Fully normal NVC pattern and non-specific NVC alterations were respectively observed in 6% and 46% of MCTD and in 6% and 83% of UCTD. Moreover, PRP patients showed normal NVC pattern and non-specific capillary abnormalities in 23% and in 77%, respectively. No statistically significant correlations were observed between NVC patterns and ANA patterns/specific ENA-Abs among the UCTD patients. CONCLUSIONS: The significant presence of the SSc-like NVC pattern and reduced number of capillaries seem the most typical NVC findings in MCTD in comparison to UCTD patients, suggesting a reflection of more complex and severe disease in MCTD ones. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. Electronic address: carmen.pizzorni@unige.it. FAU - Ferrari, Giorgia AU - Ferrari G AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Schenone, Carlotta AU - Schenone C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Hysa, Elvis AU - Hysa E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Carmisciano, Luca AU - Carmisciano L AD - Department of Health Sciences (DiSSal), Biostatistics Section, University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Pacini, Greta AU - Pacini G AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. Electronic address: albertosulli@unige.it. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. Electronic address: sabrina.paolino@unige.it. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital - Department of Internal Medicine, Ghent University, Belgium - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. Electronic address: vanessa.smith@ugent.be. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova, IRCCS San Martino Polyclinic, Genoa, Italy. Electronic address: mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20220329 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Capillaries MH - Humans MH - Microscopic Angioscopy MH - *Mixed Connective Tissue Disease/diagnosis MH - Nails/blood supply MH - *Raynaud Disease/diagnosis MH - Retrospective Studies MH - *Scleroderma, Systemic MH - *Undifferentiated Connective Tissue Diseases OTO - NOTNLM OT - Autoantibodies OT - Microvascular damage OT - Mixed connective tissue disease OT - Nailfold capillaroscopy OT - Primary Raynaud phenomenon OT - Undifferentiated connective tissue disease EDAT- 2022/04/02 06:00 MHDA- 2022/06/07 06:00 CRDT- 2022/04/01 20:11 PHST- 2022/01/07 00:00 [received] PHST- 2022/03/25 00:00 [revised] PHST- 2022/03/25 00:00 [accepted] PHST- 2022/04/02 06:00 [pubmed] PHST- 2022/06/07 06:00 [medline] PHST- 2022/04/01 20:11 [entrez] AID - S0026-2862(22)00057-7 [pii] AID - 10.1016/j.mvr.2022.104367 [doi] PST - ppublish SO - Microvasc Res. 2022 Jul;142:104367. doi: 10.1016/j.mvr.2022.104367. Epub 2022 Mar 29. PMID- 21586218 OWN - NLM STAT- MEDLINE DCOM- 20110906 LR - 20220409 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 29 IP - 2 Suppl 65 DP - 2011 Mar-Apr TI - Abnormal plasma levels of different angiogenic molecules are associated with different clinical manifestations in patients with systemic sclerosis. PG - S46-52 AB - OBJECTIVES: Systemic Sclerosis (SSc) is characterized by a microvascular damage due to an impairment of different angiogenic and angiostatic factors. Aim of this study was to measure plasma levels of nine molecules involved in these vascular processes in a group of SSc patients, respect to healthy controls (NC). METHODS: Sixty-five patients (M/F = 2/63; mean age = 57.29 yrs; mean disease duration = 9,63 yrs) with established SSc according to ARA criteria, and sixteen age- and sex-matched NC were enrolled. Plasma levels of vascular endothelial growth factor (VEGF), angiopoietin-2 (Ang-2), platelet derived growth factor- bb (PDGF-BB), platelet endothelial cellular adhesion molecule-1 (PECAM-1), leptin, hepathocyte growth factor (HGF), follistatin, granulocyte-colony stimulating factor (G-CSF) and interleukin 8 (IL-8) were measured using commercially available immunoassay kits (Human Angiogenesis 9-Plex Panel, Bio-Rad Laboratories). RESULTS: We detected a significant increase of Ang-2 (median value 1315.4 pg/ml vs 538.73 pg/ml; p=0.0292), HGF (median value 2886.16 pg/ml vs 1296.16 pg/ml; p=0.0001), IL-8 (median value 32.22 pg/ml vs 16.86 pg/ml; p=0.02), leptin (median value 32589,1 pg/mg vs 10679.61 pg/ml; p=0.0065), PDGF-BB (median value 7258.6 pg/ml vs 2913.44 pg/ml; p=0.0005), PECAM-1(median value 21681.81 pg/ml vs 10354.53 pg/ml; p=0.0003) and VEGF (median value 236.72 pg/ml vs 122.905 pg/ml; p=0.0073) in patients with SSc respect to NC. Higher levels of PDGF-BB (p=0.03) and PECAM-1 (p=0.05) were found in patients with digital ulcers while lower levels of PECAM-1 were found in patients with pulmonary hypertension (PH). Besides levels of IL-8 were higher in patients with PH (p=0.04) and lower in those with pulmonary fibrosis (p=0.5), while levels of Ang-2 were higher in those with a 'late' nailfold video-capillaroscopy (NVC) pattern respect to those with an 'early/active' one (p=0.05). Moreover, plasma levels of VEGF (p=0.02) and PDGF-BB (p= 0.04) were significantly higher in those patients positive for anti-topoisomerase 1 antibodies. CONCLUSIONS: Our findings show significantly higher circulating levels of seven angiogenic parameters in SSc patients, thus reflecting the disregulation of endothelium in this disease. Abnormal levels of these molecules may be considered an attempt for compensatory although ineffective mechanisms of vascular function, leading to the development of the main clinical manifestations of SSc. FAU - Riccieri, Valeria AU - Riccieri V AD - Rheumatological Unit, Department of Internal Medicine and Clinical Specialities, University of Rome 'Sapienza', Rome, Italy. valeria.riccieri@uniroma1.it FAU - Stefanantoni, Katia AU - Stefanantoni K FAU - Vasile, Massimiliano AU - Vasile M FAU - Macrì, Valeria AU - Macrì V FAU - Sciarra, Iliana AU - Sciarra I FAU - Iannace, N AU - Iannace N FAU - Alessandri, Cristiano AU - Alessandri C FAU - Valesini, Guido AU - Valesini G LA - eng PT - Journal Article DEP - 20110513 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Angiogenesis Inducing Agents) RN - 0 (Angiogenesis Inhibitors) RN - 0 (Interleukin-8) RN - 0 (Platelet Endothelial Cell Adhesion Molecule-1) RN - 0 (Platelet-Derived Growth Factor) RN - 0 (Proto-Oncogene Proteins c-sis) RN - 0 (Vascular Endothelial Growth Factor A) RN - 143011-72-7 (Granulocyte Colony-Stimulating Factor) RN - 1B56C968OA (Becaplermin) RN - 67256-21-7 (Hepatocyte Growth Factor) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Angiogenesis Inducing Agents/*blood MH - Angiogenesis Inhibitors/*blood MH - Becaplermin MH - Female MH - Granulocyte Colony-Stimulating Factor/blood MH - Hepatocyte Growth Factor/blood MH - Humans MH - Hypertension, Pulmonary/etiology/*metabolism/*physiopathology MH - Interleukin-8/blood MH - Male MH - Microscopic Angioscopy MH - Microvessels/pathology/physiopathology MH - Middle Aged MH - Platelet Endothelial Cell Adhesion Molecule-1/blood MH - Platelet-Derived Growth Factor/metabolism MH - Proto-Oncogene Proteins c-sis MH - Raynaud Disease/etiology/*metabolism/*physiopathology MH - *Scleroderma, Systemic/complications/metabolism/physiopathology MH - Vascular Endothelial Growth Factor A/blood EDAT- 2011/08/04 06:00 MHDA- 2011/09/07 06:00 CRDT- 2011/05/19 06:00 PHST- 2010/09/20 00:00 [received] PHST- 2011/04/21 00:00 [accepted] PHST- 2011/05/19 06:00 [entrez] PHST- 2011/08/04 06:00 [pubmed] PHST- 2011/09/07 06:00 [medline] AID - 4359 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2011 Mar-Apr;29(2 Suppl 65):S46-52. Epub 2011 May 13. PMID- 27548652 OWN - NLM STAT- MEDLINE DCOM- 20170605 LR - 20181202 IS - 1531-6963 (Electronic) IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 28 IP - 6 DP - 2016 Nov TI - Aberrant immune response with consequent vascular and connective tissue remodeling - causal to scleroderma and associated syndromes such as Raynaud phenomenon and other fibrosing syndromes? PG - 571-6 LID - 10.1097/BOR.0000000000000333 [doi] AB - PURPOSE OF REVIEW: Scleroderma and other autoimmune-induced connective tissue diseases are characterized by dysfunctions in the immune system, connective tissue and the vasculature. We are focusing on systemic sclerosis (SSc)-associated pulmonary hypertension, which remains a leading cause of death with only a 50-60% of 2-year survival rate. RECENT FINDINGS: Much research and translational efforts have been directed at understanding the immune response that causes SSc and the networked interactions with the connective tissue and the vasculature. One of the unexpected findings was that in some cases the pathogenic immune response in SSc resembles the immune response to helminth parasites. During coevolution, means of communication were developed which protect the host from over-colonization with parasites and which protect the parasite from excessive host responses. One explanation for the geographically clustered occurrence of SSc is that environmental exposures combined with genetic predisposition turn on triggers of molecular and cellular modules that were once initiated by parasites. SUMMARY: Future research is needed to further understand the parasite-derived signals that dampen the host response. Therapeutic helminth infection or treatment with parasite-derived response modifiers could be promising new management tools for autoimmune connective tissue diseases. FAU - Durmus, Nedim AU - Durmus N AD - aDepartment of Environmental Medicine bDepartment of Medicine, New York University Langone Medical Center, Tuxedo, New York, USA. FAU - Park, Sung-Hyun AU - Park SH FAU - Reibman, Joan AU - Reibman J FAU - Grunig, Gabriele AU - Grunig G LA - eng GR - R01 HL095764/HL/NHLBI NIH HHS/United States GR - R21 HL092370/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Connective Tissue/immunology/physiopathology MH - Connective Tissue Diseases/immunology MH - Fibrosis MH - Genetic Predisposition to Disease MH - Humans MH - Hypertension, Pulmonary/immunology MH - Raynaud Disease/*immunology MH - Scleroderma, Localized/immunology MH - Scleroderma, Systemic/*immunology MH - Syndrome MH - Vascular Remodeling/immunology PMC - PMC5114306 MID - NIHMS821879 COIS- Conflicts of Interest: None declared. EDAT- 2016/08/23 06:00 MHDA- 2017/06/06 06:00 PMCR- 2017/11/01 CRDT- 2016/08/23 06:00 PHST- 2016/08/23 06:00 [entrez] PHST- 2016/08/23 06:00 [pubmed] PHST- 2017/06/06 06:00 [medline] PHST- 2017/11/01 00:00 [pmc-release] AID - 10.1097/BOR.0000000000000333 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2016 Nov;28(6):571-6. doi: 10.1097/BOR.0000000000000333. PMID- 25342013 OWN - NLM STAT- MEDLINE DCOM- 20150901 LR - 20190918 IS - 1882-0654 (Electronic) IS - 0009-918X (Linking) VI - 54 IP - 10 DP - 2014 TI - [A case of an anti-SRP myopathy with enlargement of the thymus]. PG - 798-802 AB - A 54-year-old female was admitted to our hospital because of the Raynaud phenomenon and muscle weakness of the upper limbs. The neurological findings showed somatic and proximal limb weakness. Laboratory studies showed a high serum creatine kinase level. Computerized tomography (CT) revealed enlargement of the thymus. A muscle biopsy showed a small number of degenerating and regenerating fibers but no inflammatory infiltrations. At first, she was initially treated with a three-day course of intravenous methylprednisolone (1 g/day). However, the weakness progressed and the serum creatine kinase level remained high. She was subsequently treated with a combination of tacrolimus (3 mg/day) and prednisolone, but showed no any improvement of the muscle weakness. Following additional treatment with intravenous immunoglobulin, she showed improvement in her muscle weakness. Further, anti-signal recognition particle antibodies were identified after treatment. There have been no previous reports of myopathy with antibodies against the signal recognition particle and enlargement of the thymus, so we herein report the details of this unique case. FAU - Ohta, Rie AU - Ohta R AD - Section of Neurology, Japanese Red Cross Nagasaki Genbaku Hospital. FAU - Mukaino, Akihiro AU - Mukaino A FAU - Kinoshita, Ikuo AU - Kinoshita I FAU - Tsujihata, Mitsuhiro AU - Tsujihata M FAU - Suzuki, Shigeaki AU - Suzuki S LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 RN - 0 (Autoantibodies) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Signal Recognition Particle) SB - IM MH - *Autoantibodies MH - Female MH - Humans MH - Immunoglobulins, Intravenous/*administration & dosage MH - Middle Aged MH - Muscular Diseases/complications/*immunology/pathology/*therapy MH - Raynaud Disease/etiology MH - Signal Recognition Particle/*immunology MH - Treatment Outcome EDAT- 2014/10/25 06:00 MHDA- 2015/09/02 06:00 CRDT- 2014/10/25 06:00 PHST- 2014/10/25 06:00 [entrez] PHST- 2014/10/25 06:00 [pubmed] PHST- 2015/09/02 06:00 [medline] AID - DN/JST.JSTAGE/clinicalneurol/54.798 [pii] AID - 10.5692/clinicalneurol.54.798 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2014;54(10):798-802. doi: 10.5692/clinicalneurol.54.798. PMID- 29796907 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20190308 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 38 IP - 7 DP - 2018 Jul TI - The impact of anti-U1-RNP positivity: systemic lupus erythematosus versus mixed connective tissue disease. PG - 1169-1178 LID - 10.1007/s00296-018-4059-4 [doi] AB - Anti-U1-RNP positivity remains mandatory for the mixed connective tissue disease (MCTD) diagnosis, reason for which anti-U1-RNP occurrence in patients with lupus clinical features might determine diagnostic issues. Thus, the prevalence of 25-30% for anti-RNP was reported in John Hopkins and LUMINA lupus cohorts and also 13% prevalence for the anti-U1-RNP in Euro-Lupus cohort. Presence of anti-U1-RNP antibodies in patients fulfilling SLE criteria (but not the MCTD ones) was associated with manifestations such as Raynaud phenomenon, musculoskeletal and lung impairment or nail fold capillaroscopy changes, some clinical features frequently encountered in MCTD patients and only rarely described in lupus population. The use of more specific markers such as 70 kDa anti-U1-RNP or anti-Sm-D was proposed for discriminating between SLE and MCTD. In addition, the IgM serotype of anti-U1-RNP seems more frequently expressed in SLE, while the IgG serotype alone in MCTD. Better acknowledgement of possible clinical involvements in lupus subsets, such as the peculiarities related to the anti-U1-RNP positivity, could provide access to early diagnosis of rather rare but possible severe lupus organ impairments (e.g. pulmonary arterial hypertension). FAU - Dima, Alina AU - Dima A AUID- ORCID: 0000-0001-8743-3236 AD - Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Dionisie Lupu Street 37, 020022, Bucharest S2, Romania. alina_dima@outlook.com. FAU - Jurcut, Ciprian AU - Jurcut C AUID- ORCID: 0000-0001-9613-7420 AD - Internal Medicine Department, Carol Davila Central University Emergency Military Hospital, Mircea Vulcanescu Street 88, 010825, Bucharest S2, Romania. FAU - Baicus, Cristian AU - Baicus C AUID- ORCID: 0000-0002-7954-0098 AD - Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Dionisie Lupu Street 37, 020022, Bucharest S2, Romania. AD - Internal Medicine Department, Colentina Research Center, Colentina Clinical Hospital, Stefan cel Mare Street 19-21, 020125, Bucharest S2, Romania. LA - eng PT - Journal Article PT - Review DEP - 20180523 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*blood MH - Diagnosis, Differential MH - Humans MH - Lupus Erythematosus, Discoid/diagnosis MH - Lupus Erythematosus, Systemic/*diagnosis/immunology MH - Mixed Connective Tissue Disease/*diagnosis/immunology MH - Raynaud Disease/diagnosis OTO - NOTNLM OT - Criteria MCTD OT - Scleroderma OT - Systemic lupus erythematosus OT - U1 70 k OT - U1 RNP EDAT- 2018/05/26 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/05/26 06:00 PHST- 2018/04/21 00:00 [received] PHST- 2018/05/17 00:00 [accepted] PHST- 2018/05/26 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/05/26 06:00 [entrez] AID - 10.1007/s00296-018-4059-4 [pii] AID - 10.1007/s00296-018-4059-4 [doi] PST - ppublish SO - Rheumatol Int. 2018 Jul;38(7):1169-1178. doi: 10.1007/s00296-018-4059-4. Epub 2018 May 23. PMID- 41187239 OWN - NLM STAT- MEDLINE DCOM- 20251104 LR - 20251218 IS - 1545-9616 (Print) IS - 1545-9616 (Linking) VI - 24 IP - 11 DP - 2025 Nov 1 TI - Treatment of Refractory Pruritic Dermatitis in the Setting of Primary Biliary Cholangitis and CREST Syndrome With Upadacitinib. PG - 1138-1140 LID - 10.36849/JDD.9129 [doi] AB - Primary biliary cholangitis (PBC) can present with overlapping features of limited systemic sclerosis, commonly known as calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST). Here, we discuss the case of a PBC patient with CREST who experienced treatment-resistant and progressively worsening pruritic dermatitis that responded to upadacitinib with associated improvement of her liver enzymes and symptoms. This is the first documented report of a Janus kinase (JAK) inhibitor used to treat cutaneous symptoms in the setting of an autoimmune liver disease. FAU - Zhou, Albert E AU - Zhou AE FAU - Ravi, Sowmya AU - Ravi S FAU - Murphy, Michael AU - Murphy M FAU - Lu, Jun AU - Lu J FAU - Ferenczi, Katalin AU - Ferenczi K LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Drugs Dermatol JT - Journal of drugs in dermatology : JDD JID - 101160020 RN - 0 (Heterocyclic Compounds, 3-Ring) RN - 0 (Janus Kinase Inhibitors) RN - 4RA0KN46E0 (upadacitinib) SB - IM MH - Female MH - Humans MH - *CREST Syndrome/complications/drug therapy/diagnosis MH - *Dermatitis/drug therapy/diagnosis/etiology MH - *Heterocyclic Compounds, 3-Ring/therapeutic use/administration & dosage MH - *Janus Kinase Inhibitors/therapeutic use MH - *Liver Cirrhosis, Biliary/complications/drug therapy/diagnosis MH - *Pruritus/drug therapy/diagnosis/etiology MH - Treatment Outcome MH - Aged EDAT- 2025/11/04 18:26 MHDA- 2025/11/04 18:27 CRDT- 2025/11/04 14:18 PHST- 2025/11/04 18:27 [medline] PHST- 2025/11/04 18:26 [pubmed] PHST- 2025/11/04 14:18 [entrez] AID - S1545961625P9129X [pii] AID - 10.36849/JDD.9129 [doi] PST - ppublish SO - J Drugs Dermatol. 2025 Nov 1;24(11):1138-1140. doi: 10.36849/JDD.9129. PMID- 20818663 OWN - NLM STAT- MEDLINE DCOM- 20101116 LR - 20220408 IS - 1552-4833 (Electronic) IS - 1552-4825 (Linking) VI - 152A IP - 10 DP - 2010 Oct TI - Novel COL4A1 mutations associated with HANAC syndrome: a role for the triple helical CB3[IV] domain. PG - 2550-5 LID - 10.1002/ajmg.a.33659 [doi] AB - The COL4A1 gene encodes the α1-chain of type IV collagen, which is ubiquitously expressed in basement membranes. Mutations in COL4A1 have been reported in autosomal-dominant porencephaly and in patients with symptomatic small vessel brain disease, inconstantly associated with eye defects. We have previously reported three COL4A1 mutations associated with a systemic phenotype that we called HANAC (Hereditary Angiopathy, Nephropathy, Aneurysms, and Cramps). We carried out a clinical and genetic study of three families presenting with characteristic features of HANAC syndrome. Common systemic signs included arterial retinal tortuosity and muscle cramps, with a variable combination of small vessel brain disease, Raynaud phenomena, and kidney defects. Three novel COL4A1 missense substitutions are described, which affect highly conserved glycine residues within the collagenous domain of the protein. All six known mutations associated with the HANAC phenotype are localized within the CB3[IV] fragment of COL4A1, which encompasses major integrin-binding sites. Our results confirm that HANAC syndrome is a distinct clinical entity within the COL4A1-related disorders, which is characterized by systemic involvement and usually asymptomatic brain disease. The restricted distribution of COL4A1 mutations within the CB3[IV] region is a characteristic of the reports of patients with HANAC, which suggests that abnormal cell-type IV collagen interactions may underlie the systemic defects observed in this syndrome. CI - Copyright © 2010 Wiley-Liss, Inc. FAU - Plaisier, Emmanuelle AU - Plaisier E AD - AP HP, Department of Nephrology, Tenon Hospital, INSERM U702, UMRS702, Université Pierre et Marie Curie-Paris 6, Paris, France. emmanuelle.plaisier@tnn.aphp.fr FAU - Chen, Zhiyong AU - Chen Z FAU - Gekeler, Florian AU - Gekeler F FAU - Benhassine, Safa AU - Benhassine S FAU - Dahan, Karine AU - Dahan K FAU - Marro, Béatrice AU - Marro B FAU - Alamowitch, Sonia AU - Alamowitch S FAU - Paques, Michel AU - Paques M FAU - Ronco, Pierre AU - Ronco P LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Am J Med Genet A JT - American journal of medical genetics. Part A JID - 101235741 RN - 0 (COL4A1 protein, human) RN - 0 (Collagen Type IV) SB - IM MH - Abnormalities, Multiple/*genetics MH - Adult MH - Amino Acid Sequence MH - Animals MH - Brain Diseases/genetics MH - Collagen Type IV/chemistry/*genetics MH - Conserved Sequence MH - Female MH - Humans MH - Male MH - Middle Aged MH - Mutation MH - Pedigree MH - Raynaud Disease/genetics MH - Sequence Alignment MH - Sequence Homology, Amino Acid EDAT- 2010/09/08 06:00 MHDA- 2010/11/17 06:00 CRDT- 2010/09/07 06:00 PHST- 2010/09/07 06:00 [entrez] PHST- 2010/09/08 06:00 [pubmed] PHST- 2010/11/17 06:00 [medline] AID - 10.1002/ajmg.a.33659 [doi] PST - ppublish SO - Am J Med Genet A. 2010 Oct;152A(10):2550-5. doi: 10.1002/ajmg.a.33659. PMID- 21199634 OWN - NLM STAT- MEDLINE DCOM- 20110607 LR - 20131121 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 16 IP - 12 DP - 2010 Dec 15 TI - Hypocomplementemic urticarial vasculitis in mixed connective tissue disease. PG - 8 AB - Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence. FAU - Calistru, Ana Maria AU - Calistru AM AD - Department of Dermatology and Venereology, Hospital São João, Porto, Portugal. FAU - Lisboa, Carmen AU - Lisboa C FAU - Cruz, Maria João AU - Cruz MJ FAU - Delgado, Luis AU - Delgado L FAU - Poças, Licínio AU - Poças L FAU - Azevedo, Filomena AU - Azevedo F LA - eng PT - Case Reports PT - Journal Article DEP - 20101215 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Complement C4) RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 80295-44-9 (Complement C3c) RN - VB0R961HZT (Prednisone) SB - IM MH - Complement C3c/deficiency MH - Complement C4/deficiency MH - Female MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications/drug therapy/immunology MH - Prednisone/therapeutic use MH - Raynaud Disease/etiology MH - Urticaria/drug therapy/*etiology/immunology MH - Vasculitis, Leukocytoclastic, Cutaneous/drug therapy/*etiology/immunology EDAT- 2011/01/05 06:00 MHDA- 2011/06/08 06:00 CRDT- 2011/01/05 06:00 PHST- 2011/01/05 06:00 [entrez] PHST- 2011/01/05 06:00 [pubmed] PHST- 2011/06/08 06:00 [medline] PST - epublish SO - Dermatol Online J. 2010 Dec 15;16(12):8. PMID- 20391683 OWN - NLM STAT- MEDLINE DCOM- 20100514 LR - 20201209 IS - 0034-1193 (Print) IS - 0034-1193 (Linking) VI - 101 IP - 1 DP - 2010 Jan TI - [Helicobacter pylori infection: from gastric to systemic disease]. PG - 27-33 AB - H. pylori infection, through a chronic stimulation of the immune system and the occurrence of molecular mimicry mechanisms, is responsible for the majority of the gastroduodenal diseases and also for some extragastric disorders, including sideropenic anemia and idiopathic thrombocytopenic purpura; other diseases are under investigation. FAU - Gasbarrini, Giovanni AU - Gasbarrini G AD - Istituto di Medicina Interna, Policlinico Agostino Gemelli, Università Cattolica del Sacro Cuore, Roma. FAU - Racco, Simona AU - Racco S FAU - Franceschi, Francesco AU - Franceschi F FAU - Miele, Luca AU - Miele L FAU - Cammarota, Giovanni AU - Cammarota G FAU - Grieco, Antonio AU - Grieco A FAU - Gasbarrini, Antonio AU - Gasbarrini A LA - ita PT - Journal Article PT - Review TT - Manifestazioni extragastriche dell'infezione da Helicobacter pylori. PL - Italy TA - Recenti Prog Med JT - Recenti progressi in medicina JID - 0401271 SB - IM MH - Adenocarcinoma/microbiology MH - Anemia, Iron-Deficiency/microbiology MH - Atherosclerosis/microbiology MH - Duodenal Diseases/microbiology MH - Female MH - Helicobacter Infections/*complications/drug therapy MH - *Helicobacter pylori MH - Hepatic Encephalopathy/microbiology MH - Humans MH - Lymphoma, B-Cell, Marginal Zone/microbiology MH - Migraine Disorders/microbiology MH - Practice Guidelines as Topic MH - Pre-Eclampsia/microbiology MH - Pregnancy MH - Purpura, Thrombocytopenic, Idiopathic/microbiology MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/microbiology MH - Stomach Diseases/*microbiology MH - Stomach Neoplasms/microbiology MH - Thyroiditis, Autoimmune/microbiology RF - 67 EDAT- 2010/04/16 06:00 MHDA- 2010/05/15 06:00 CRDT- 2010/04/16 06:00 PHST- 2010/04/16 06:00 [entrez] PHST- 2010/04/16 06:00 [pubmed] PHST- 2010/05/15 06:00 [medline] PST - ppublish SO - Recenti Prog Med. 2010 Jan;101(1):27-33. PMID- 21349774 OWN - NLM STAT- MEDLINE DCOM- 20120221 LR - 20121115 IS - 1873-1449 (Electronic) IS - 1538-4721 (Linking) VI - 10 IP - 6 DP - 2011 Nov-Dec TI - Partial breast irradiation in a patient with bilateral breast cancers and CREST syndrome. PG - 486-90 LID - 10.1016/j.brachy.2011.01.010 [doi] AB - PURPOSE: To describe the first documented use of partial breast irradiation (PBI) in a patient with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias (CREST) syndrome. METHODS AND MATERIALS: A 50-year-old woman with well-controlled CREST syndrome for 6 years was diagnosed with bilateral early-staged breast cancers. She underwent bilateral lumpectomies, sentinel lymph node biopsies, and PBI delivered via bilateral MammoSite catheters (Cytyc Corp., Marlborough, MA) followed by chemotherapy. She was monitored perioperatively, at 6 months and at 1 year for worsening of her CREST-related symptoms and complications associated with surgery and radiation therapy. Both surgeon and patient's opinion of her cosmetic outcome were also recorded at 1-year followup. RESULTS: The patient experienced mild acute cellulitic changes in the perioperative period, which resolved with antibiotics. At 6 months, she exhibited a Grade 1 late toxicity, which has remained stable at 1-year followup. The patient and surgeon are very pleased with her cosmetic outcome. CONCLUSIONS: Accelerated PBI was delivered safely to a patient with collagen vascular disease. By decreasing the surface area receiving radiation with accelerated PBI, we believe that the toxicity associated with the treatment was minimized. Future studies will be necessary to clarify the use of PBI in patients with collagen vascular disease. CI - Copyright © 2011 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved. FAU - Kounalakis, Nicole AU - Kounalakis N AD - Department of Surgery, University of Colorado Medical Center, Aurora, CO 80045, USA. nicole.kounalakis@ucdenver.edu FAU - Pezner, Richard AU - Pezner R FAU - Staud, Cecil L AU - Staud CL FAU - Kruper, Laura AU - Kruper L LA - eng PT - Case Reports PT - Journal Article DEP - 20110224 PL - United States TA - Brachytherapy JT - Brachytherapy JID - 101137600 SB - IM MH - Brachytherapy/instrumentation/*methods MH - Breast Neoplasms/*epidemiology/*radiotherapy/surgery MH - CREST Syndrome/*epidemiology MH - Carcinoma, Ductal, Breast/*epidemiology/*radiotherapy MH - Catheterization MH - Comorbidity MH - Female MH - Humans MH - Mastectomy, Segmental MH - Middle Aged MH - Radiotherapy Dosage MH - Radiotherapy, Adjuvant EDAT- 2011/02/26 06:00 MHDA- 2012/02/22 06:00 CRDT- 2011/02/26 06:00 PHST- 2010/12/10 00:00 [received] PHST- 2011/01/18 00:00 [revised] PHST- 2011/01/19 00:00 [accepted] PHST- 2011/02/26 06:00 [entrez] PHST- 2011/02/26 06:00 [pubmed] PHST- 2012/02/22 06:00 [medline] AID - S1538-4721(11)00013-4 [pii] AID - 10.1016/j.brachy.2011.01.010 [doi] PST - ppublish SO - Brachytherapy. 2011 Nov-Dec;10(6):486-90. doi: 10.1016/j.brachy.2011.01.010. Epub 2011 Feb 24. PMID- 35475519 OWN - NLM STAT- MEDLINE DCOM- 20220803 LR - 20250728 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 49 IP - 8 DP - 2022 Aug TI - Paraneoplastic acral vascular syndrome in a patient with uterine cancer. PG - e274-e275 LID - 10.1111/1346-8138.16401 [doi] FAU - Yoshizaki, Ai AU - Yoshizaki A AUID- ORCID: 0000-0003-0144-1345 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Ibusuki, Atsuko AU - Ibusuki A AUID- ORCID: 0000-0003-2349-1765 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Baba, Naoko AU - Baba N AUID- ORCID: 0000-0001-8799-3284 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Takeda, Koichiro AU - Takeda K AD - Department of Dermatology, Imamura General Hospital, Kagoshima, Japan. FAU - Fujii, Kazuyasu AU - Fujii K AUID- ORCID: 0000-0002-6482-8953 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Kamio, Masaki AU - Kamio M AD - Department of obstetrics and gynecology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Maruyama, Yumi AU - Maruyama Y AD - Department of obstetrics and gynecology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Higashi, Yuko AU - Higashi Y AUID- ORCID: 0000-0003-3867-0555 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. FAU - Kanekura, Takuro AU - Kanekura T AUID- ORCID: 0000-0003-3904-5954 AD - Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. LA - eng PT - Letter DEP - 20220427 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - Female MH - Humans MH - *Paraneoplastic Syndromes/diagnosis/etiology MH - *Raynaud Disease MH - *Uterine Neoplasms/complications/diagnosis EDAT- 2022/04/28 06:00 MHDA- 2022/08/04 06:00 CRDT- 2022/04/27 08:42 PHST- 2022/04/05 00:00 [revised] PHST- 2022/03/19 00:00 [received] PHST- 2022/04/10 00:00 [accepted] PHST- 2022/04/28 06:00 [pubmed] PHST- 2022/08/04 06:00 [medline] PHST- 2022/04/27 08:42 [entrez] AID - 10.1111/1346-8138.16401 [doi] PST - ppublish SO - J Dermatol. 2022 Aug;49(8):e274-e275. doi: 10.1111/1346-8138.16401. Epub 2022 Apr 27. PMID- 17396337 OWN - NLM STAT- MEDLINE DCOM- 20070523 LR - 20151119 IS - 0026-4725 (Print) IS - 0026-4725 (Linking) VI - 54 IP - 6 DP - 2006 Dec TI - [Dysthyroidism and connective pathology]. PG - 811-4 AB - AIM: The aim of the study is to evaluate the degree of the relationship between dysthyroidism and connective pathology, both autoimmune diseases, presenting, sometimes, an early common symptom: the Raynaud phenomenon. METHOD: We studied 30 patients subdivided as follows: 18 affected by mixed connective pathology, 6 by scleroderma, 2 by CREST, 4 by scleroderma and Sjogren syndrome. We focused our attention on the chronology and the duration of the diagnosis, correlated to the laboratory parameters and the hypothesis of the pathogenetic pathway. RESULTS: 36.6% were positive to both the pathologies. One of the 11 patients resulted positive to hyperthyroidism and mixed connective, 10 patients resulted positive to hyperthyroidism subdivided in: 6 affected by sclerodermia and by 4 sclerodermia and S. of Sjogren. All the patients were positive to ANA without correlation between those values and the microsomal or antithyreoglobulin antibodies: the last two were present in 33%. The laboratory parameters indicating the inflammatory state were in the normal range, a light hypercholesterolemia was found in all patients. CONCLUSION: There is a suggestive relationship between the connective pathology and dysthyroidism,therefore it can be useful to evaluate always the association of both pathologies for the future identification of the immunogenetic alteration factors, the evaluation of the composition and the turnover of the collagen, subdividing the patients in relation to the clinical and the laboratory parameters, follow up to middle and long term. FAU - Schachter, I AU - Schachter I AD - UOC di Angiologia, Azienda Ospedaliera S. Giovanni-Addolorata, Roma. FAU - Antignani, P L AU - Antignani PL LA - ita PT - English Abstract PT - Journal Article TT - Connettiviti e distiroidismo. PL - Italy TA - Minerva Cardioangiol JT - Minerva cardioangiologica JID - 0400725 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Autoantibodies/blood MH - Biomarkers/blood MH - Connective Tissue Diseases/blood/complications/*diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Scleroderma, Systemic/diagnosis MH - Sjogren's Syndrome/diagnosis MH - Thyroid Diseases/blood/complications/*diagnosis EDAT- 2007/04/03 09:00 MHDA- 2007/05/24 09:00 CRDT- 2007/04/03 09:00 PHST- 2007/04/03 09:00 [pubmed] PHST- 2007/05/24 09:00 [medline] PHST- 2007/04/03 09:00 [entrez] PST - ppublish SO - Minerva Cardioangiol. 2006 Dec;54(6):811-4. PMID- 27938812 OWN - NLM STAT- MEDLINE DCOM- 20170620 LR - 20170620 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 35 IP - 1 DP - 2017 Jan-Feb TI - Acral manifestations of paraneoplastic and collagen vascular diseases. PG - 50-54 LID - S0738-081X(16)30226-7 [pii] LID - 10.1016/j.clindermatol.2016.09.007 [doi] AB - The skin often signals a number of systemic disease, making skin findings of paramount significance. Paraneoplastic diseases and collagen vascular diseases are vitally important illnesses. Paraneoplastic diseases and collagen vascular diseases may also occur with many different acral skin findings. Paraneoplastic skin diseases, associated with some cancers, are by definition nonmalignant skin disorders. These diseases can occur before, simultaneously, or after the diagnosis of cancer. Acral paraneoplastic diseases include acanthosis nigricans maligna, acquired pachydermatoglyphia, acrokeratosis paraneoplastica, palmoplantar keratoderma, and paraneoplastic nail disorders. Collagen vascular diseases include the acral skin findings of lupus erythematosus, scleroderma, dermatomyositis, and rheumatoid arthritis. Any acral skin finding may be encountered as the first finding of an undiagnosed malignancy or collagen vascular diseases. The role of the dermatologist is significant for often being the first physician to suspect a malignancy or collagen vascular disease. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Gül, Ülker AU - Gül Ü AD - Department of Dermatology, Akdeniz University, Faculty of Medicine, Antalya, Turkey. Electronic address: ulkergul@yahoo.com. LA - eng PT - Journal Article DEP - 20160910 PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 SB - IM MH - Acanthosis Nigricans/etiology MH - Arthritis, Rheumatoid/complications MH - Foot Dermatoses/*etiology MH - Hand Dermatoses/*etiology MH - Humans MH - Lupus Erythematosus, Cutaneous/*complications MH - Nail Diseases/etiology MH - Paraneoplastic Syndromes/*complications MH - Raynaud Disease/etiology MH - Scleroderma, Localized/complications MH - Scleroderma, Systemic/*complications EDAT- 2016/12/13 06:00 MHDA- 2017/06/21 06:00 CRDT- 2016/12/13 06:00 PHST- 2016/12/13 06:00 [entrez] PHST- 2016/12/13 06:00 [pubmed] PHST- 2017/06/21 06:00 [medline] AID - S0738-081X(16)30226-7 [pii] AID - 10.1016/j.clindermatol.2016.09.007 [doi] PST - ppublish SO - Clin Dermatol. 2017 Jan-Feb;35(1):50-54. doi: 10.1016/j.clindermatol.2016.09.007. Epub 2016 Sep 10. PMID- 22483176 OWN - NLM STAT- MEDLINE DCOM- 20130128 LR - 20181201 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 37 IP - 9 DP - 2012 Sep TI - Nonoperative treatment of digital ischemia in systemic sclerosis. PG - 1907-9 LID - 10.1016/j.jhsa.2012.02.009 [doi] FAU - Wall, Lindley B AU - Wall LB AD - Stern Hand Surgery Fellowship, Department of Orthopaedic Surgery, University of Cincinnati College of Medicine, 538 Oak Street, Suite 200, Cincinnati, OH 45219, USA. lindley.wall@gmail.com FAU - Stern, Peter J AU - Stern PJ LA - eng PT - Case Reports PT - Comparative Study PT - Journal Article DEP - 20120405 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Calcium Channel Blockers) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - JED5K35YGL (Iloprost) RN - Q326023R30 (Bosentan) SB - IM MH - Bosentan MH - Botulinum Toxins, Type A/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Education, Medical, Continuing MH - Endothelin Receptor Antagonists MH - Evidence-Based Medicine MH - Female MH - Fingers/*blood supply MH - Humans MH - Iloprost/therapeutic use MH - Ischemia/*therapy MH - Life Style MH - Middle Aged MH - Orthopedics/education MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Prostaglandins/therapeutic use MH - Randomized Controlled Trials as Topic MH - Raynaud Disease/*therapy MH - Scleroderma, Systemic/*therapy MH - Sulfonamides/therapeutic use MH - Vascular Resistance/drug effects MH - Vasodilator Agents/therapeutic use EDAT- 2012/04/10 06:00 MHDA- 2013/01/29 06:00 CRDT- 2012/04/10 06:00 PHST- 2012/01/11 00:00 [received] PHST- 2012/02/04 00:00 [accepted] PHST- 2012/04/10 06:00 [entrez] PHST- 2012/04/10 06:00 [pubmed] PHST- 2013/01/29 06:00 [medline] AID - S0363-5023(12)00219-5 [pii] AID - 10.1016/j.jhsa.2012.02.009 [doi] PST - ppublish SO - J Hand Surg Am. 2012 Sep;37(9):1907-9. doi: 10.1016/j.jhsa.2012.02.009. Epub 2012 Apr 5. PMID- 31637898 OWN - NLM STAT- MEDLINE DCOM- 20200409 LR - 20200409 IS - 1827-1618 (Electronic) IS - 0026-4725 (Linking) VI - 67 IP - 5 DP - 2019 Oct TI - Management of mild, primary Raynaud Syndrome: supplementation with Pycnogenol®. PG - 392-398 LID - 10.23736/S0026-4725.19.04991-0 [doi] AB - BACKGROUND: Raynaud syndrome (RS) is associated with vasospasm of the hand and fingers as a response to cold or stress. RS may cause discomfort and color changes (pallor, cyanosis, erythema, as single symptoms, but usually in combination, localized to one or more fingers). The aim of this 4-week registry study was the evaluation of subjects with mild, primary RS and their treatment with a standard management (SM) plan in comparison with SM associated with supplementation with Pycnogenol®. METHODS: A group of 67 females with mild, primary RS was included. All subjects were working in shops with refrigerators. No skin lesions were present. The age range was between 30 and 40; the vasospastic changes were symmetrical; no other physical findings were present. RESULTS: The two groups, receiving standard management (N.=33) or SM+Pycnogenol®, 100 mg/day, (N.=34) were comparable at inclusion. Considering the main symptoms, the decrease in coldness, burning pain, paresthesias and irregular color changes was more significant with Pycnogenol® (P<0.05) at 4 weeks. At thermography, low temperature areas and discrepancies in temperature and color were decreased with Pycnogenol® more than in controls (P<0.05). In the Pycnogenol® group, one subject (2.94%) decided to use drug treatment (PGE1) in 4 weeks in comparison with 5 controls (15.15%). The lowest finger temperature improved from 20.3° C at inclusion to 26.4° C at 4 weeks (+30.04%) with Pycnogenol® in comparison with lower values (from 20.5 to 23.1 [+12.7%] in controls [P<0.05]). The fingertip skin flux increased significantly (+ 1.55 flux units) with Pycnogenol® (P<0.05), in controls just by +0.14 (ns). Supplementation with Pycnogenol® decreased oxidative stress and increased transcutaneous oxygen pressure (TcPO2) more than in controls. Compliance and tolerability were optimal. CONCLUSIONS: This pilot registry study suggests that Pycnogenol® may be used with significant advantages in primary, mild RS. Subjects using Pycnogenol® may control their symptoms and may avoid the need for more complex and, potentially dangerous or expensive treatments. FAU - Hu, Shu AU - Hu S AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. FAU - Hosoi, Morio AU - Hosoi M AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. FAU - Belcaro, Gianni AU - Belcaro G AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy - cardres@abol.it. FAU - Dugall, Mark AU - Dugall M AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. FAU - Feragalli, Beatrice AU - Feragalli B AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. FAU - Cotellese, Roberto AU - Cotellese R AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. FAU - Luzzi, Roberta AU - Luzzi R AD - IRVINE3 Labs, IAPSS, Nicolaides's Lab, Chieti, Italy. LA - eng PT - Journal Article PL - Italy TA - Minerva Cardioangiol JT - Minerva cardioangiologica JID - 0400725 RN - 0 (Antioxidants) RN - 0 (Flavonoids) RN - 0 (Plant Extracts) RN - 50JZ5Z98QY (pycnogenols) SB - IM MH - Adult MH - Antioxidants/*therapeutic use MH - *Dietary Supplements MH - Female MH - Flavonoids/*therapeutic use MH - Humans MH - Pilot Projects MH - Plant Extracts/*therapeutic use MH - Raynaud Disease/*drug therapy MH - Severity of Illness Index MH - Syndrome EDAT- 2019/10/23 06:00 MHDA- 2020/04/10 06:00 CRDT- 2019/10/23 06:00 PHST- 2019/10/23 06:00 [entrez] PHST- 2019/10/23 06:00 [pubmed] PHST- 2020/04/10 06:00 [medline] AID - S0026-4725.19.04991-0 [pii] AID - 10.23736/S0026-4725.19.04991-0 [doi] PST - ppublish SO - Minerva Cardioangiol. 2019 Oct;67(5):392-398. doi: 10.23736/S0026-4725.19.04991-0. PMID- 33476821 OWN - NLM STAT- MEDLINE DCOM- 20210519 LR - 20210519 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 20 IP - 3 DP - 2021 Mar TI - Undifferentiated connective tissue disease at risk for systemic sclerosis: Development of a short-term predictive score and a risk stratification tool. PG - 102751 LID - S1568-9972(21)00010-0 [pii] LID - 10.1016/j.autrev.2021.102751 [doi] FAU - Riccardi, Antonella AU - Riccardi A AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Italy. FAU - Marcoccia, Antonella AU - Marcoccia A AD - Centro di Riferimento Interdisciplinare, Interdipartimentale per la diagnosi precoce della Sclerodermia (CRIIS), Sandro Pertini Hospital, Rome, Italy. FAU - Modesti, Mariagrazia AU - Modesti M AD - Centro di Riferimento Interdisciplinare, Interdipartimentale per la diagnosi precoce della Sclerodermia (CRIIS), Sandro Pertini Hospital, Rome, Italy. FAU - Bondanini, Francesco AU - Bondanini F AD - Centro di Riferimento Interdisciplinare, Interdipartimentale per la diagnosi precoce della Sclerodermia (CRIIS), Sandro Pertini Hospital, Rome, Italy. FAU - Irace, Rosaria AU - Irace R AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Italy. FAU - Messiniti, Valentina AU - Messiniti V AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Italy. FAU - Vitali, Claudio AU - Vitali C AD - Mater Domini' Humanitas Hospital, Rheumatology Outpatient Clinics, Castellanza, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AD - Department of Rheumatology, UOC Day Hospital of Rheumatology, ASST G.Pini-CTO, Milan, Italy. FAU - Valentini, Gabriele AU - Valentini G AD - Formerly Professor of Rheumatology, University of Campania "Luigi Vanvitelli", Italy. Electronic address: gabrielevalentini981@gmail.com. LA - eng PT - Letter DEP - 20210118 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - *Connective Tissue Diseases/diagnosis/epidemiology MH - Humans MH - *Raynaud Disease MH - Risk Assessment MH - *Scleroderma, Systemic/diagnosis/epidemiology MH - *Undifferentiated Connective Tissue Diseases EDAT- 2021/01/22 06:00 MHDA- 2021/05/20 06:00 CRDT- 2021/01/21 20:11 PHST- 2020/10/30 00:00 [received] PHST- 2020/11/05 00:00 [accepted] PHST- 2021/01/22 06:00 [pubmed] PHST- 2021/05/20 06:00 [medline] PHST- 2021/01/21 20:11 [entrez] AID - S1568-9972(21)00010-0 [pii] AID - 10.1016/j.autrev.2021.102751 [doi] PST - ppublish SO - Autoimmun Rev. 2021 Mar;20(3):102751. doi: 10.1016/j.autrev.2021.102751. Epub 2021 Jan 18. PMID- 19166140 OWN - NLM STAT- MEDLINE DCOM- 20090304 LR - 20250623 IS - 0041-4131 (Print) IS - 0041-4131 (Linking) VI - 85 IP - 11 DP - 2007 Nov TI - Usefulness of video-capillaroscopy in clinical practice: systematic review of indications and results in rheumatology and in non-rheumatic disorders. PG - 913-9 AB - BACKGROUND: Nailfold video-capillaroscopy (VCP) is nowadays worldwide considered as one of the best diagnostic noninvasive imaging technique able to study microcirculation in vivo. AIM: To review the applications of VCP in the clinical practice and its results in rheumatic and non-rheumatic diseases. METHODS: Review of literature RESULTS: The possibility of managing the imaging, by means of dedicated software able to characterize quantitative and qualitative data, represents another relevant property of VCP. This technique is very useful at the identification of microvascular involvement in many rheumatic diseases, particularly in systemic sclerosis and related disorders. At the same time, VCP has been showed valuable in many other extra-rheumatic diseases. The authors review the applications of VCP in the clinical practice and its results in rheumatic and non-rheumatic diseases. FAU - Russo, Romualdo AU - Russo R AD - Department of Internal Medicine, Rheumatology, Cardarelli Hospital, Naples, Italy. FAU - Gallucci, Fernando AU - Gallucci F FAU - Uomo, Generoso AU - Uomo G FAU - Hadj Yahia, Chiraz Ben AU - Hadj Yahia CB FAU - Abdelmoula, Leila AU - Abdelmoula L FAU - Chaabouni, Lilia AU - Chaabouni L FAU - Zouari, Rafik AU - Zouari R LA - eng PT - Journal Article PT - Systematic Review PL - Tunisia TA - Tunis Med JT - La Tunisie medicale JID - 0413766 SB - IM MH - Autoimmune Diseases/diagnosis MH - Capillaries/*pathology MH - Crohn Disease/diagnosis MH - Diabetic Angiopathies/diagnosis MH - Diagnosis, Differential MH - Familial Mediterranean Fever/diagnosis MH - Humans MH - Hypertension/diagnosis MH - Microscopic Angioscopy/*methods MH - Microvascular Angina/diagnosis MH - Raynaud Disease/diagnosis MH - Reproducibility of Results MH - Rheumatic Diseases/*diagnosis/pathology MH - *Rheumatology/methods MH - *Video Recording RF - 43 EDAT- 2007/11/01 00:00 MHDA- 2009/03/05 09:00 CRDT- 2009/01/27 09:00 PHST- 2009/01/27 09:00 [entrez] PHST- 2007/11/01 00:00 [pubmed] PHST- 2009/03/05 09:00 [medline] PST - ppublish SO - Tunis Med. 2007 Nov;85(11):913-9. PMID- 21291650 OWN - NLM STAT- MEDLINE DCOM- 20110407 LR - 20220419 IS - 1203-4754 (Print) IS - 1203-4754 (Linking) VI - 15 IP - 1 DP - 2011 Jan-Feb TI - Dermatologic manifestations of Sjögren syndrome. PG - 8-14 AB - Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.OBJECTIVE AND CONCLUSIONS:In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality. FAU - Kittridge, Ashley AU - Kittridge A AD - West Penn Allegheny Health System, Department of Medicine , Pittsburg, PA, USA. FAU - Routhouska, Shannon B AU - Routhouska SB FAU - Korman, Neil J AU - Korman NJ LA - eng PT - Journal Article PT - Review PL - United States TA - J Cutan Med Surg JT - Journal of cutaneous medicine and surgery JID - 9614685 SB - IM MH - Humans MH - Ichthyosis/etiology MH - Raynaud Disease/diagnosis MH - Sjogren's Syndrome/*complications MH - Skin Diseases/*etiology MH - Vasculitis/complications MH - Xerostomia/therapy EDAT- 2011/02/05 06:00 MHDA- 2011/04/08 06:00 CRDT- 2011/02/05 06:00 PHST- 2011/02/05 06:00 [entrez] PHST- 2011/02/05 06:00 [pubmed] PHST- 2011/04/08 06:00 [medline] AID - 10.2310/7750.2010.09033 [doi] PST - ppublish SO - J Cutan Med Surg. 2011 Jan-Feb;15(1):8-14. doi: 10.2310/7750.2010.09033. PMID- 19779284 OWN - NLM STAT- MEDLINE DCOM- 20091215 LR - 20190606 IS - 1349-8029 (Electronic) IS - 0470-8105 (Linking) VI - 49 IP - 9 DP - 2009 Sep TI - Intracranial aneurysms in patients with CREST syndrome. PG - 402-6 AB - CREST syndrome is a variant of scleroderma characterized by calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia, and is a collagen vascular disease characterized by inflammation and fibrosis of multiple organs/tissues. Neurological and cerebrovascular abnormalities are uncommon in CREST syndrome. Here, we report two patients with CREST syndrome harboring intracranial aneurysms. A 53-year-old woman with a 6-month history of CREST syndrome had multiple intracranial aneurysms that arose from the right middle cerebral artery, the left middle cerebral artery, the choroidal segment of the left internal carotid artery, and the left anterior cerebral artery. A 64-year-old woman with a 2-year history of CREST syndrome had a fusiform aneurysm located on the insular segment of the left middle cerebral artery. These patients were treated surgically and good outcome was achieved in both cases. The pathogenesis of cerebral aneurysms associated with collagen diseases, including CREST syndrome, remains unclear. Early treatment of CREST syndrome and other collagen diseases may prevent arteritis from progressing to affect the intracranial arteries and thus reduce the occurrence of aneurysms. The prognosis for patients with collagen diseases after rupture of cerebral aneurysm seems to be poor because the multiplicity, atypical morphology, and atypical location of their aneurysms make treatment difficult. Thus, early detection and treatment are important to improve the prognosis. FAU - Nakae, Ryuta AU - Nakae R AD - Department of Neurosurgery, Chugoku Rosai Hospital, Hiroshima. nakae@nms.ac.jp FAU - Idei, Masaru AU - Idei M FAU - Kumano, Kiyoshi AU - Kumano K FAU - Okita, Shinji AU - Okita S FAU - Yamane, Kanji AU - Yamane K LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 RN - 9007-34-5 (Collagen) SB - IM MH - Arteritis/complications/physiopathology MH - CREST Syndrome/*complications/physiopathology MH - Cerebral Angiography MH - Cerebral Arteries/diagnostic imaging/*pathology/surgery MH - Collagen/genetics/metabolism MH - Craniotomy MH - Disease Progression MH - Early Diagnosis MH - Female MH - Humans MH - Intracranial Aneurysm/*diagnosis/*etiology/surgery MH - Magnetic Resonance Angiography MH - Middle Aged MH - Prognosis MH - Surgical Instruments MH - Treatment Outcome MH - Vascular Surgical Procedures EDAT- 2009/09/26 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/26 06:00 PHST- 2009/09/26 06:00 [entrez] PHST- 2009/09/26 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - JST.JSTAGE/nmc/49.402 [pii] AID - 10.2176/nmc.49.402 [doi] PST - ppublish SO - Neurol Med Chir (Tokyo). 2009 Sep;49(9):402-6. doi: 10.2176/nmc.49.402. PMID- 25435750 OWN - NLM STAT- MEDLINE DCOM- 20160728 LR - 20181113 IS - 2092-9382 (Electronic) IS - 1011-8942 (Print) IS - 1011-8942 (Linking) VI - 28 IP - 6 DP - 2014 Dec TI - Comparison of the thickness of the lamina cribrosa and vascular factors in early normal-tension glaucoma with low and high intraocular pressures. PG - 473-8 LID - 10.3341/kjo.2014.28.6.473 [doi] AB - PURPOSE: To compare the thickness of the lamina cribrosa (LC) and vascular factors of early normal-tension glaucoma (NTG) patients with high and low intraocular pressure (IOP) that are expected to be associated with the development of glaucoma. METHODS: Seventy-one Korean NTG patients with low IOP (the highest IOP <15 mmHg, 40 patients) and high IOP (the lowest IOP >15 mmHg, 31 patients) were included in this study. The thickness of LC and vascular factors were compared. The thickness of the LC was measured using the enhanced depth imaging method with spectral domain optical coherence tomography (Heidelberg Spectralis). RESULTS: The mean thickness of the central LC was 190.0 ± 19.2 µm in the low IOP group and 197.8 ± 23.6 µm in the high IOP group, but there was no statistical significant difference between the two groups (p > 0.05). The prevalence of self-reported Raynaud phenomenon was significantly higher in the low IOP group (33.0%) than the high IOP group (10.3%, p = 0.04). CONCLUSIONS: The laminar thickness did not significantly differ between the high and low IOP groups. However, the prevalence of Raynaud phenomenon was higher in the low IOP groups. These results suggest that the development of glaucoma with low IOP patients may be more influenced by peripheral vasospasm, such as Raynaud phenomenon, rather than laminar thickness in NTG. FAU - Kim, Jee Hyun AU - Kim JH AD - Cheil Eye Hospital and Cheil Eye Research Institute, Daegu, Korea. FAU - Lee, Tae Yoon AU - Lee TY AD - Cheil Eye Hospital and Cheil Eye Research Institute, Daegu, Korea. FAU - Lee, Jong Wook AU - Lee JW AD - Cheil Eye Hospital and Cheil Eye Research Institute, Daegu, Korea. FAU - Lee, Kyoo Won AU - Lee KW AD - Cheil Eye Hospital and Cheil Eye Research Institute, Daegu, Korea. LA - eng PT - Comparative Study PT - Journal Article DEP - 20141119 PL - Korea (South) TA - Korean J Ophthalmol JT - Korean journal of ophthalmology : KJO JID - 8804513 SB - IM MH - Aged MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Intraocular Pressure MH - Low Tension Glaucoma/*diagnosis MH - Male MH - Middle Aged MH - Nerve Fibers/pathology MH - Optic Disk/*pathology MH - Optic Nerve Diseases/*diagnosis MH - Raynaud Disease/*diagnosis MH - Retinal Ganglion Cells/pathology MH - Tomography, Optical Coherence MH - Tonometry, Ocular MH - Vision Disorders/diagnosis MH - Visual Fields PMC - PMC4239466 OTO - NOTNLM OT - Enhanced depth imaging method OT - Lamina cribrosa OT - Low tension glaucoma OT - Spectral domain optical coherence tomography COIS- No potential conflict of interest relevant to this article was reported. EDAT- 2014/12/02 06:00 MHDA- 2016/07/29 06:00 PMCR- 2014/12/01 CRDT- 2014/12/02 06:00 PHST- 2013/11/10 00:00 [received] PHST- 2014/06/03 00:00 [accepted] PHST- 2014/12/02 06:00 [entrez] PHST- 2014/12/02 06:00 [pubmed] PHST- 2016/07/29 06:00 [medline] PHST- 2014/12/01 00:00 [pmc-release] AID - 10.3341/kjo.2014.28.6.473 [doi] PST - ppublish SO - Korean J Ophthalmol. 2014 Dec;28(6):473-8. doi: 10.3341/kjo.2014.28.6.473. Epub 2014 Nov 19. PMID- 34342883 OWN - NLM STAT- MEDLINE DCOM- 20220304 LR - 20220304 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 47 IP - 2 DP - 2022 Feb TI - Clinical and histopathological features of adult patients with dermatomyositis and melanoma differentiation associated-5 autoantibody seropositivity status, as determined by commercially available testing: a retrospective, single-institution comparative cohort study. PG - 282-288 LID - 10.1111/ced.14870 [doi] AB - BACKGROUND: Although melanoma differentiation associated (MDA)-5 autoantibodies have been widely explored in dermatomyositis (DM), most studies have relied on MDA-5 autoantibody testing performed in research settings, rather than the now-available commercial laboratory tests. AIM: To characterize the clinical and histopathological data in patients with DM and circulating MDA-5 autoantibodies, as defined by commercially available testing. METHODS: This was a retrospective review of patients with DM who underwent MDA-5 antibody testing. All available skin biopsy slides were reviewed. RESULTS: Cutaneous features more prevalent in MDA-5-positive DM included Raynaud phenomenon (RP) (P < 0.001), cutaneous ulcerations (P = 0.01), mechanic hands (P < 0.02), palmar papules (P < 0.01), oral ulcers (P = 0.024) and alopecia (P = 0.03). Joint and pulmonary involvement were more common in patients with MDA-5-positive DM (both P < 0.001) as was dysphagia (P < 0.01). Myopathy (P = 0.4) and malignancy (P = 0.34) were not statistically different between the cohorts. Vasculopathy was more common in MDA-5-positive DM (P < 0.01), while spongiosis was less common (P < 0.02). CONCLUSION: This study not only confirms some known associations between disease manifestations and MDA-5 autoantibody status, as determined by commercially available tests, but also identifies new associations, including RP and dysphagia. CI - © 2021 British Association of Dermatologists. FAU - Shakshouk, H AU - Shakshouk H AUID- ORCID: 0000-0002-0047-3292 AD - Department of Dermatology, Mayo Clinic, Rochester, MN, USA. FAU - Deschaine, M A AU - Deschaine MA AD - Department of Dermatology, Florida State University, Tallahassee, FL, USA. FAU - Wetter, D A AU - Wetter DA AD - Department of Dermatology, Mayo Clinic, Rochester, MN, USA. FAU - Drage, L A AU - Drage LA AD - Department of Dermatology, Mayo Clinic, Rochester, MN, USA. FAU - Ernste, F C AU - Ernste FC AD - Division of Rheumatology, Mayo Clinic, Rochester, MN, USA. FAU - Lehman, J S AU - Lehman JS AD - Department of Dermatology, Mayo Clinic, Rochester, MN, USA. AD - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA. LA - eng PT - Comparative Study PT - Journal Article DEP - 20211005 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 RN - 0 (Autoantibodies) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) SB - IM MH - Autoantibodies/*blood MH - Biopsy MH - Deglutition Disorders/complications MH - Dermatomyositis/complications/immunology/*pathology MH - Female MH - Humans MH - Interferon-Induced Helicase, IFIH1/*immunology MH - Male MH - Raynaud Disease/complications MH - Retrospective Studies MH - Skin/*pathology EDAT- 2021/08/04 06:00 MHDA- 2022/03/05 06:00 CRDT- 2021/08/03 12:29 PHST- 2021/07/26 00:00 [accepted] PHST- 2021/08/04 06:00 [pubmed] PHST- 2022/03/05 06:00 [medline] PHST- 2021/08/03 12:29 [entrez] AID - 10.1111/ced.14870 [doi] PST - ppublish SO - Clin Exp Dermatol. 2022 Feb;47(2):282-288. doi: 10.1111/ced.14870. Epub 2021 Oct 5. PMID- 29602651 OWN - NLM STAT- MEDLINE DCOM- 20190516 LR - 20190516 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 43 IP - 10 DP - 2018 Oct TI - Anatomical Variation of the Radial Artery Associated With Clinically Significant Ischemia. PG - 952.e1-952.e5 LID - S0363-5023(17)30618-4 [pii] LID - 10.1016/j.jhsa.2018.02.036 [doi] AB - PURPOSE: The purpose of this retrospective review was to investigate the incidence of radial artery anatomical variations in patients with clinically significant distal upper extremity (UE) ischemia. Available anatomical studies report that high takeoff of the radial artery occurs in up to 15% of the population. We hypothesized that there is a higher incidence of high origin of the radial artery in patients with clinically significant ischemia compared with the reported frequency in the general population. METHODS: We performed a retrospective review of all patients who underwent UE angiography for clinically significant hand and digital ischemia in our institution from 2012 to 2016. Data collected included patient age, sex, comorbidities, and modality of treatment. RESULTS: Twenty-six angiograms were performed for UE ischemia meeting inclusion criteria. Eight patients had Raynaud disease or scleroderma. Ten patients (38%) had high radial artery takeoff with radial artery origin proximal to the antecubital fossa. The need for surgical intervention was similar in patients with normal anatomy and those with high takeoff of the radial artery. CONCLUSIONS: Incidence of high radial artery takeoff was found more frequently in patients with distal UE ischemia requiring angiogram than in reported population data. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV. CI - Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved. FAU - Polfer, Elizabeth M AU - Polfer EM AD - Walter Reed National Military Medical Center, Bethesda, MD; The Curtis National Hand Center, Baltimore, MD. FAU - Sabino, Jennifer M AU - Sabino JM AD - The Curtis National Hand Center, Baltimore, MD. FAU - Giladi, Aviram M AU - Giladi AM AD - The Curtis National Hand Center, Baltimore, MD. FAU - Higgins, James P AU - Higgins JP AD - The Curtis National Hand Center, Baltimore, MD. Electronic address: anne.mattson@medstar.net. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180327 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 SB - IM MH - Angiography MH - Female MH - Humans MH - Ischemia/*etiology MH - Male MH - Middle Aged MH - Radial Artery/*abnormalities/*diagnostic imaging MH - Raynaud Disease MH - Retrospective Studies MH - Scleroderma, Limited MH - Upper Extremity/*blood supply OTO - NOTNLM OT - Radial artery OT - upper extremity ischemia EDAT- 2018/04/01 06:00 MHDA- 2019/05/17 06:00 CRDT- 2018/04/01 06:00 PHST- 2017/04/13 00:00 [received] PHST- 2018/01/23 00:00 [revised] PHST- 2018/02/28 00:00 [accepted] PHST- 2018/04/01 06:00 [pubmed] PHST- 2019/05/17 06:00 [medline] PHST- 2018/04/01 06:00 [entrez] AID - S0363-5023(17)30618-4 [pii] AID - 10.1016/j.jhsa.2018.02.036 [doi] PST - ppublish SO - J Hand Surg Am. 2018 Oct;43(10):952.e1-952.e5. doi: 10.1016/j.jhsa.2018.02.036. Epub 2018 Mar 27. PMID- 21693547 OWN - NLM STAT- MEDLINE DCOM- 20111017 LR - 20191210 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 50 IP - 9 DP - 2011 Sep TI - High-resolution magnetic resonance angiography of digital arteries in SSc patients on 3 Tesla: preliminary study. PG - 1712-9 LID - 10.1093/rheumatology/keq453 [doi] AB - OBJECTIVE: We sought to evaluate the use of high-resolution three-dimensional time of flight (3D TOF) magnetic resonance angiography (MRA) at 3 Tesla in the visualization of digital arteries in SSc patients. METHODS: A total of 33 patients with SSc [32 females and 1 male; mean (s.d.) age 37.0 (10.2) years; median number of years since onset of RP 3.4 (2.8) years] and 7 healthy controls [6 females and 1 male; mean (s.d.) age 30.7 (3.5) years] were examined on a 3T MR system. A modified high spatial resolution (voxel size = 0.35 × 0.35 × 0.5 mm(3)) 3D TOF MRA (repetition time/echo time = 19/4.4 ms, flip angle = 15°, slice thickness = 1 mm) was performed during a total scan time of 8 min 22 s. The source images and maximum intensity projection reconstruction were studied; the digital arteries count and lumen area of the selective section of the vessel were measured independently by two experienced radiologists and compared with that of the control ones; and a four-level grading system was made according to the severity. Statistical analysis was performed with t-test and P < 0.05 was used as the criterion. RESULTS: We detected the eight digital arteries in the four fingers (without the thumb) of each case and got a 47.58% presentation in general in the SSc group, and artery No. 5 had the highest presentation rate (70.97%). Statistics showed that the digital arteries No. 1 (P = 0.058) and No. 3 (P = 0.093) had no difference in the lumen area (P > 0.05). We finally got 3 cases in Grade 1, 14 cases in Grade 2, 9 cases in Grade 3 and 5 cases in Grade 4. CONCLUSION: MRA of the digital arteries in the described technique is a promising method for us to judge the severity of microvascular involvement in finger vessels of SSc patients. FAU - Zhang, Wei AU - Zhang W AD - Department of Radiology, Shanghai Clinical Centre of Rheumatic Diseases and Institute of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Xu, Jian Rong AU - Xu JR FAU - Lu, Qing AU - Lu Q FAU - Ye, Shuang AU - Ye S FAU - Liu, Xiao Sheng AU - Liu XS LA - eng PT - Evaluation Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110621 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Arteries MH - Case-Control Studies MH - Female MH - Fingers/*blood supply MH - Humans MH - Image Processing, Computer-Assisted MH - Imaging, Three-Dimensional MH - Magnetic Resonance Angiography/*methods MH - Male MH - Raynaud Disease/complications/*physiopathology MH - Scleroderma, Systemic/complications/diagnosis/*physiopathology EDAT- 2011/06/23 06:00 MHDA- 2011/10/18 06:00 CRDT- 2011/06/23 06:00 PHST- 2011/06/23 06:00 [entrez] PHST- 2011/06/23 06:00 [pubmed] PHST- 2011/10/18 06:00 [medline] AID - keq453 [pii] AID - 10.1093/rheumatology/keq453 [doi] PST - ppublish SO - Rheumatology (Oxford). 2011 Sep;50(9):1712-9. doi: 10.1093/rheumatology/keq453. Epub 2011 Jun 21. PMID- 32541075 OWN - NLM STAT- MEDLINE DCOM- 20210831 LR - 20220202 IS - 1499-2752 (Electronic) IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 48 IP - 2 DP - 2021 Feb TI - Raynaud Phenomenon in Systemic Sclerosis: Does Digital Thermal Monitoring Correlate to Specific Nailfold Videocapillaroscopy Abnormalities? PG - 247-250 LID - 10.3899/jrheum.191371 [doi] AB - OBJECTIVE: Early diagnosis of systemic sclerosis (SSc) is imperative, and Raynaud phenomenon (RP) is an important component of progressive vasculopathy. Nailfold videocapillaroscopy (NVC) is a well-established tool that can quantify structural vascular abnormalities. Digital thermal monitoring (DTM) assesses microvascular functional dysfunction related to thermoregulation. In this study, we investigated the correlation of NVC patterns and DTM variables in patients with SSc. METHODS: Patients with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism criteria who consented and enrolled in the clinical care registry had NVC and DTM performed. For NVC, the number of capillaries (density), measurement of apical diameter (dimension), presence or absence of hemorrhages, and number of abnormal shapes were assessed to categorize 3 different qualitative patterns: early, active, and late. For DTM, Doppler ultrasound hyperemic, low frequency, blood velocity of radial artery, and fingertip vascular function were assessed, and a vascular reactivity index (VRI) measurement was automated. Statistical evaluation was performed by nonparametric tests to assess the correlation of NVC and VRI. RESULTS: Thirty-one SSc subjects with interpretable NVC and DTM performed on the same day were included in the study. VRI was progressively higher in SSc patients with early, active, and late NVC patterns of microangiopathy (P < 0.0001). There was a significant negative correlation between VRI and microhemorrhages scores (r = -0.363, P = 0.044). CONCLUSION: Our study suggests that more advanced vasculopathy correlates to reduced microvascular function as detected by DTM and more advanced structural abnormalities detected by NVC. NVC and DTM may provide different aspects of vasculopathy quantification and complement each other as investigative tools. CI - Copyright © 2021 by the Journal of Rheumatology. FAU - Thomas, Julie K AU - Thomas JK AD - J.K. Thomas, MD, University of Utah, Department of Internal Medicine, Division of Rheumatology, Salt Lake City, Utah, USA. FAU - Radic, Mislav AU - Radic M AD - M. Radic, MD, University of Utah, Department of Internal Medicine, Division of Rheumatology, Salt Lake City, Utah, USA, and University Hospital Split, Split, Croatia. FAU - Tucker, Jordan R AU - Tucker JR AD - J.R. Tucker, Salt Lake Veterans Affair Medical Center, Utah Vascular Research Laboratory, Salt Lake City, Utah, USA. FAU - Overbury, Rebecca AU - Overbury R AD - R. Overbury, MD, University of Utah, Department of Internal Medicine, Division of Rheumatology, and University of Utah, Department of Pediatrics, Division of Rheumatology, Salt Lake City, Utah, USA. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - T. Frech, MD, MS, University of Utah, Department of Internal Medicine, Division of Rheumatology, and Salt Lake Veterans Affair Medical Center, Utah Vascular Research Laboratory, Salt Lake City, Utah, USA. tracy.frech@hsc.utah.edu. LA - eng GR - I01 CX001183/CX/CSRD VA/United States GR - K23 AR067889/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20200615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Capillaries/diagnostic imaging MH - Humans MH - Microscopic Angioscopy MH - Nails/diagnostic imaging MH - *Raynaud Disease/diagnostic imaging MH - *Scleroderma, Systemic/complications/diagnostic imaging PMC - PMC7736231 MID - NIHMS1600493 OTO - NOTNLM OT - Raynaud phenomenon OT - capillaries OT - systemic sclerosis COIS- Disclosures: The authors have no conflict of interest to disclose. EDAT- 2020/06/17 06:00 MHDA- 2021/09/01 06:00 PMCR- 2022/02/01 CRDT- 2020/06/17 06:00 PHST- 2020/06/01 00:00 [accepted] PHST- 2020/06/17 06:00 [pubmed] PHST- 2021/09/01 06:00 [medline] PHST- 2020/06/17 06:00 [entrez] PHST- 2022/02/01 00:00 [pmc-release] AID - jrheum.191371 [pii] AID - 10.3899/jrheum.191371 [doi] PST - ppublish SO - J Rheumatol. 2021 Feb;48(2):247-250. doi: 10.3899/jrheum.191371. Epub 2020 Jun 15. PMID- 19858403 OWN - NLM STAT- MEDLINE DCOM- 20091224 LR - 20151119 IS - 1527-7755 (Electronic) IS - 0732-183X (Linking) VI - 27 IP - 35 DP - 2009 Dec 10 TI - Health-related quality of life in long-term survivors of testicular cancer. PG - 5993-9 LID - 10.1200/JCO.2008.19.6931 [doi] AB - PURPOSE: A growing number of patients with testicular cancer (TC) become long-term survivors. As a consequence, quality-of-life (QOL) issues become increasingly important. The objective of this study was to investigate QOL among Danish TC survivors. METHODS: A long-term follow-up assessment of all patients with TC treated at Aarhus University Hospital in Denmark between 1990 and 2000 was conducted. A total of 401 survivors (response rate, 66%) completed questionnaires concerning QOL (European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire C30), depression (Beck Depression Inventory-II), fatigue (Multidimensional Fatigue Inventory-20), and health-related issues such as neurotoxic symptoms and Raynaud-like phenomena. On the basis of their treatment, participants were categorized as having received surveillance, radiotherapy, or chemotherapy. RESULTS: QOL among patients with TC was equal to that of men from the general population. Although patients who received chemotherapy reported higher levels of peripheral sensory neuropathy, ototoxicity, and Raynaud-like phenomena, treatment strategies were generally unrelated to QOL and depressive symptoms. CONCLUSION: Overall, the patients in this study reported high levels of QOL. The results suggest that patients treated for TC should be informed about the anticipated good post-therapeutic QOL and the low risk of psychosocial and physical long-term effects. FAU - Rossen, Philip Blach AU - Rossen PB AD - Department of Oncology, Aarhus University Hospital, Aarhus, Denmark. philiprossen@dadlnet.dk FAU - Pedersen, Anette Fischer AU - Pedersen AF FAU - Zachariae, Robert AU - Zachariae R FAU - von der Maase, Hans AU - von der Maase H LA - eng PT - Journal Article DEP - 20091026 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 SB - IM MH - Adult MH - Chemotherapy, Adjuvant/adverse effects MH - Denmark MH - Depression/etiology MH - Fatigue/etiology MH - Follow-Up Studies MH - Hearing Disorders/etiology MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Orchiectomy/adverse effects MH - Peripheral Nervous System Diseases/etiology MH - *Quality of Life MH - Radiotherapy, Adjuvant/adverse effects MH - Raynaud Disease/etiology MH - Registries MH - Risk Assessment MH - Risk Factors MH - Surveys and Questionnaires MH - Survivors/*psychology MH - Testicular Neoplasms/*psychology/*therapy MH - Time Factors MH - Treatment Outcome EDAT- 2009/10/28 06:00 MHDA- 2009/12/25 06:00 CRDT- 2009/10/28 06:00 PHST- 2009/10/28 06:00 [entrez] PHST- 2009/10/28 06:00 [pubmed] PHST- 2009/12/25 06:00 [medline] AID - JCO.2008.19.6931 [pii] AID - 10.1200/JCO.2008.19.6931 [doi] PST - ppublish SO - J Clin Oncol. 2009 Dec 10;27(35):5993-9. doi: 10.1200/JCO.2008.19.6931. Epub 2009 Oct 26. PMID- 32182400 OWN - NLM STAT- MEDLINE DCOM- 20201005 LR - 20201005 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 72 IP - 7 DP - 2020 Jul TI - Giant Calcinosis in Dermatomyositis and Scleroderma Overlap. PG - 1236 LID - 10.1002/art.41262 [doi] FAU - Fernández-Codina, Andreu AU - Fernández-Codina A AUID- ORCID: 0000-0001-7939-7837 AD - University of Western Ontario, London, Ontario, Canada. AD - Windsor Regional Hospital, Windsor, Ontario, Canada. AD - Vall d'Hebron University Hospital, Barcelona, Spain. FAU - Camprodon-Gómez, Maria AU - Camprodon-Gómez M AD - Vall d'Hebron University Hospital, Barcelona, Spain. FAU - Pope, Janet E AU - Pope JE AUID- ORCID: 0000-0003-1479-5302 AD - University of Western Ontario, London, Ontario, Canada. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200527 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) RN - 83HN0GTJ6D (Cyclosporine) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Calcinosis/*diagnostic imaging/etiology/surgery MH - Cyclosporine/therapeutic use MH - Dermatomyositis/complications/*therapy MH - Drainage MH - Female MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/etiology MH - Methylprednisolone/therapeutic use MH - Middle Aged MH - Muscle Weakness/etiology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/complications/*therapy MH - Skin Diseases MH - Subcutaneous Tissue/*diagnostic imaging/surgery MH - Symptom Flare Up MH - Thoracic Wall/*diagnostic imaging/surgery MH - Tomography, X-Ray Computed EDAT- 2020/03/18 06:00 MHDA- 2020/10/06 06:00 CRDT- 2020/03/18 06:00 PHST- 2020/03/18 06:00 [pubmed] PHST- 2020/10/06 06:00 [medline] PHST- 2020/03/18 06:00 [entrez] AID - 10.1002/art.41262 [doi] PST - ppublish SO - Arthritis Rheumatol. 2020 Jul;72(7):1236. doi: 10.1002/art.41262. Epub 2020 May 27. PMID- 21982057 OWN - NLM STAT- MEDLINE DCOM- 20120724 LR - 20131121 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 66 IP - 6 DP - 2012 Jun TI - Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications. PG - 959-65 LID - 10.1016/j.jaad.2011.07.013 [doi] AB - BACKGROUND: Fibroblastic rheumatism is a rare dermatoarthropathy characterized by the sudden onset of cutaneous nodules, flexion contractures, and polyarthritis. Histopathology in the correct clinical context confirms the diagnosis. Treatment is based on observational data from single case reports. OBJECTIVE: We describe 4 cases, review histologic findings, and discuss therapeutic responses. METHODS: Cases coded as fibroblastic rheumatism were retrieved from institutional and consultation files. Medical charts and biopsy specimens were reviewed. Elastic stains and immunostains for smooth muscle actin, S100, CD34, desmin, and epithelial membrane antigen were performed on selected cases. RESULTS: Four cases were identified. Patients displayed cutaneous nodules and arthralgias. Flexion contractures/decreased motion were present in two patients; one patient had associated Raynaud phenomenon and erosive joint disease. Biopsy specimens demonstrated a fibroblastic proliferation associated with a collagenous stroma. Growth patterns varied from cellular fascicles to paucicellular randomly arranged spindle cells. Elastic fibers were absent in all cases tested (3/3). Immunohistochemical stains demonstrated immunoreactivity for smooth muscle actin in one of 3 cases in a myofibroblastic pattern. Other stains were negative. One patient had complete resolution of disease with methotrexate. One patient partially responded to interferon-alfa and ribavirin and was subsequently treated with methotrexate with additional improvement. One patient had limited response to all therapies attempted. One patient was lost to follow-up. LIMITATIONS: Small sample size (n = 4) is a limitation. CONCLUSION: Our data expand the clinical, histologic, and therapeutic response data on fibroblastic rheumatism. Correlation with clinical history is critical to avoid misdiagnosis as other fibrosing lesions. Methotrexate and interferon-alfa are potential therapies. CI - Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. FAU - Jurado, Sara A AU - Jurado SA AD - Department of Dermatology, Cleveland Clinic, Cleveland, Ohio 44195, USA. FAU - Alvin, G K Glen AU - Alvin GK FAU - Selim, M Angelica AU - Selim MA FAU - Pipkin, Clare A AU - Pipkin CA FAU - Kress, Douglas AU - Kress D FAU - Jamora, Maria Jasmin J AU - Jamora MJ FAU - Billings, Steven D AU - Billings SD LA - eng PT - Case Reports PT - Journal Article DEP - 20111007 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Antirheumatic Agents) RN - 0 (Immunologic Factors) RN - 0 (Interferon-alpha) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adolescent MH - Antirheumatic Agents/therapeutic use MH - Arthritis/epidemiology MH - Child MH - Contracture/*epidemiology MH - Diagnosis, Differential MH - Fibroblasts MH - Humans MH - Immunologic Factors/therapeutic use MH - Interferon-alpha/therapeutic use MH - Male MH - Methotrexate/therapeutic use MH - Raynaud Disease/epidemiology MH - Rheumatic Diseases/diagnosis/drug therapy/*epidemiology/pathology EDAT- 2011/10/11 06:00 MHDA- 2012/07/25 06:00 CRDT- 2011/10/11 06:00 PHST- 2011/01/20 00:00 [received] PHST- 2011/07/14 00:00 [revised] PHST- 2011/07/18 00:00 [accepted] PHST- 2011/10/11 06:00 [entrez] PHST- 2011/10/11 06:00 [pubmed] PHST- 2012/07/25 06:00 [medline] AID - S0190-9622(11)00802-4 [pii] AID - 10.1016/j.jaad.2011.07.013 [doi] PST - ppublish SO - J Am Acad Dermatol. 2012 Jun;66(6):959-65. doi: 10.1016/j.jaad.2011.07.013. Epub 2011 Oct 7. PMID- 21794782 OWN - NLM STAT- MEDLINE DCOM- 20120201 LR - 20110728 IS - 1885-1398 (Electronic) IS - 1699-258X (Linking) VI - 7 IP - 1 DP - 2011 Jan-Feb TI - [Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis]. PG - 61-7 LID - 10.1016/j.reuma.2010.02.003 [doi] AB - A 31-year-old woman with a prior history of an overlap syndrome of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) developed fever, pericarditis with pericardial effusion and a rapidly progressive fatal interstitial lung disease. Diagnostic test and procedures, differential diagnosis and therapeutic approach are discussed. CI - Copyright © 2010 Elsevier España, S.L. All rights reserved. FAU - Sacnun, Mónica P AU - Sacnun MP AD - Servicio de Reumatología y Colagenopatías, Hospital Provincial de Rosario, Rosario, Argentina. FAU - Ferrer, Jaime AU - Ferrer J FAU - Ferrer, Marisol AU - Ferrer M FAU - Pons-Estel, Bernardo A AU - Pons-Estel BA LA - spa PT - Case Reports PT - Clinical Conference PT - English Abstract PT - Journal Article TT - Enfermedad pulmonar intersticial rápidamente progresiva y fatal en una paciente con síndrome de superposición de lupus eritematoso sistémico y esclerosis sistémica. DEP - 20100619 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Biopsy MH - Disease Progression MH - Drug Therapy, Combination MH - Fatal Outcome MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnosis/*etiology/pathology MH - Immunosuppressive Agents/therapeutic use MH - Lung/pathology MH - Lupus Erythematosus, Systemic/*complications/drug therapy MH - Pericarditis/etiology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/drug therapy MH - Tomography, X-Ray Computed EDAT- 2011/07/29 06:00 MHDA- 2012/02/02 06:00 CRDT- 2011/07/29 06:00 PHST- 2010/02/17 00:00 [received] PHST- 2010/02/26 00:00 [accepted] PHST- 2011/07/29 06:00 [entrez] PHST- 2011/07/29 06:00 [pubmed] PHST- 2012/02/02 06:00 [medline] AID - S1699-258X(10)00103-8 [pii] AID - 10.1016/j.reuma.2010.02.003 [doi] PST - ppublish SO - Reumatol Clin. 2011 Jan-Feb;7(1):61-7. doi: 10.1016/j.reuma.2010.02.003. Epub 2010 Jun 19. PMID- 36670487 OWN - NLM STAT- MEDLINE DCOM- 20230124 LR - 20231116 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 25 IP - 1 DP - 2023 Jan 20 TI - Hyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon. PG - 10 LID - 10.1186/s13075-023-02990-3 [doi] LID - 10 AB - BACKGROUND/PURPOSE: Lack of robust, feasible, and quantitative outcomes impedes Raynaud phenomenon (RP) clinical trials in systemic sclerosis (SSc) patients. Hyperspectral imaging (HSI) non-invasively measures oxygenated and deoxygenated hemoglobin (oxyHb and deoxyHb) concentrations and oxygen saturation (O(2) sat) in the skin and depicts data as oxygenation heatmaps. This study explored the potential role of HSI in quantifying SSc-RP disease severity and activity. METHODS: Patients with SSc-RP (n = 13) and healthy control participants (HC; n = 12) were prospectively recruited in the clinic setting. Using a hand-held camera, bilateral hand HSI (HyperMed™, Waltham, MA) was performed in a temperature-controlled room (22 °C). OxyHb, deoxyHb, and O(2) sat values were calculated for 78-mm(2) regions of interest for the ventral fingertips and palm (for normalization). Subjects underwent a cold provocation challenge (gloved hand submersion in 15 °C water bath for 1 min), and repeated HSI was performed at 0, 10, and 20 min. Patients completed two patient-reported outcome (PRO) instruments: the Raynaud Condition Score (RCS) and the Cochin Hand Function Scale (CHFS) for symptom burden assessment. Statistical analyses were performed using the Mann-Whitney U test and a mixed effects model (Stata, College Station, TX). RESULTS: Ninety-two percent of participants were women in their 40s. For SSc-RP patients, 69% had limited cutaneous SSc, the mean ± SD SSc duration was 11 ± 5 years, and 38% had prior digital ulcers-none currently. Baseline deoxyHb was higher, and O(2) sat was lower, in SSc patients versus HC (p < 0.05). SSc patients had a greater decline in oxyHb and O(2) sat from baseline to time 0 (after cold challenge) with distinct rewarming oxyHb, O(2) sat, and deoxyHb trajectories versus HCs (p < 0.01). There were no significant correlations between oxyHb, deoxyHb, and O(2) sat level changes following cold challenge and RCS or CHFS scores. CONCLUSION: Hyperspectral imaging is a feasible approach for SSc-RP quantification in the clinic setting. The RCS and CHFS values did not correlate with HSI parameters. Our data suggest that HSI technology for the assessment of SSc-RP at baseline and in response to cold provocation is a potential quantitative measure for SSc-RP severity and activity, though longitudinal studies that assess sensitivity to change are needed. CI - © 2023. The Author(s). FAU - Teaw, Shannon AU - Teaw S AD - Section of Rheumatology, Allergy & Immunology, Department of Medicine, Yale School of Medicine, 300 Cedar Street, The Anlyan Center PO Box 208031, New Haven, CT, 06520, USA. FAU - Gupta, Akash AU - Gupta A AD - Section of Rheumatology, Allergy & Immunology, Department of Medicine, Yale School of Medicine, 300 Cedar Street, The Anlyan Center PO Box 208031, New Haven, CT, 06520, USA. FAU - Williams, Alyssa AU - Williams A AD - Section of Rheumatology, Allergy & Immunology, Department of Medicine, Yale School of Medicine, 300 Cedar Street, The Anlyan Center PO Box 208031, New Haven, CT, 06520, USA. FAU - Wilson, F Perry AU - Wilson FP AD - Clinical and Translational Research Accelerator, Department of Medicine, Yale School of Medicine, New Haven, CT, USA. AD - Department of Internal Medicine, Section of Nephrology, Yale School of Medicine, New Haven, CT, USA. FAU - Sumpio, Brandon J AU - Sumpio BJ AD - Division of Vascular and Endovascular Surgery, Massachusetts General Hospital, Boston, MA, USA. FAU - Sumpio, Bauer E AU - Sumpio BE AD - Division of Vascular Surgery, Department of Surgery, Yale School of Medicine, New Haven, CT, USA. FAU - Hinchcliff, Monique AU - Hinchcliff M AUID- ORCID: 0000-0002-8652-9890 AD - Section of Rheumatology, Allergy & Immunology, Department of Medicine, Yale School of Medicine, 300 Cedar Street, The Anlyan Center PO Box 208031, New Haven, CT, 06520, USA. Monique.hinchcliff@yale.edu. AD - Clinical and Translational Research Accelerator, Department of Medicine, Yale School of Medicine, New Haven, CT, USA. Monique.hinchcliff@yale.edu. LA - eng GR - UL1 TR001863/TR/NCATS NIH HHS/United States GR - R01 AR073270/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20230120 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - digital ulcers SB - IM MH - Male MH - *Raynaud Disease/diagnostic imaging MH - Female MH - Skin Ulcer MH - Humans MH - Hyperspectral Imaging MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/complications/diagnostic imaging/drug therapy PMC - PMC9854186 OTO - NOTNLM OT - Cochin hand function scale OT - Digital ulcers OT - Hyperspectral imaging OT - Imaging OT - Outcomes OT - Patient-reported outcome instruments OT - Raynaud condition score OT - Raynaud phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- The authors declare that they have no competing interests. EDAT- 2023/01/21 06:00 MHDA- 2023/01/25 06:00 PMCR- 2023/01/20 CRDT- 2023/01/20 23:40 PHST- 2022/10/01 00:00 [received] PHST- 2023/01/06 00:00 [accepted] PHST- 2023/01/20 23:40 [entrez] PHST- 2023/01/21 06:00 [pubmed] PHST- 2023/01/25 06:00 [medline] PHST- 2023/01/20 00:00 [pmc-release] AID - 10.1186/s13075-023-02990-3 [pii] AID - 2990 [pii] AID - 10.1186/s13075-023-02990-3 [doi] PST - epublish SO - Arthritis Res Ther. 2023 Jan 20;25(1):10. doi: 10.1186/s13075-023-02990-3. PMID- 21489936 OWN - NLM STAT- MEDLINE DCOM- 20110919 LR - 20110414 IS - 1708-5381 (Print) IS - 1708-5381 (Linking) VI - 19 IP - 2 DP - 2011 Apr TI - Asymptomatic lower extremity acrocyanosis: report of two cases and review of the literature. PG - 105-10 LID - 10.1258/vasc.2010.cr0240 [doi] AB - Vasospastic disorders affecting the lower extremities are unusual entities. Two cases of asymptomatic foot discoloration with lower extremity dependency are presented. In the first case, an elderly gentleman was admitted to the hospital with a contralateral foot infection. His physical exam revealed pulse examination within normal limits and his blood work failed to identify any known causes for vasospasm. His foot infection was successfully treated with sensitivity-directed antibiotics. The second case is that of a young man with a history of quadriplegia secondary to a remote diving accident, who presented with a three-month history of foot discoloration while sitting up in his wheelchair, which resolved with recumbent leg positioning. A review of the possible diagnoses for these patients' presentation is presented herein. FAU - Glazer, Evan AU - Glazer E AD - University of Arizona Health Science Center, Tucson, AZ 85712, USA. FAU - Pacanowski, John P AU - Pacanowski JP FAU - Leon, Luis R AU - Leon LR LA - eng PT - Case Reports PT - Journal Article PT - Review PL - England TA - Vascular JT - Vascular JID - 101196722 SB - IM MH - Adult MH - Aged, 80 and over MH - Cyanosis/*diagnosis/etiology MH - Humans MH - Leg/*blood supply MH - Livedo Reticularis MH - Male MH - Peripheral Vascular Diseases/*diagnosis/physiopathology MH - Postural Orthostatic Tachycardia Syndrome/diagnosis MH - Posture MH - Raynaud Disease/diagnosis MH - Regional Blood Flow MH - Vasoconstriction/physiology EDAT- 2011/04/15 06:00 MHDA- 2011/09/20 06:00 CRDT- 2011/04/15 06:00 PHST- 2011/04/15 06:00 [entrez] PHST- 2011/04/15 06:00 [pubmed] PHST- 2011/09/20 06:00 [medline] AID - 19/2/105 [pii] AID - 10.1258/vasc.2010.cr0240 [doi] PST - ppublish SO - Vascular. 2011 Apr;19(2):105-10. doi: 10.1258/vasc.2010.cr0240. PMID- 17604287 OWN - NLM STAT- MEDLINE DCOM- 20080114 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 67 IP - 1 DP - 2008 Jan TI - Can CCL2 serum levels be used in risk stratification or to monitor treatment response in systemic sclerosis? PG - 105-9 AB - OBJECTIVE: The chemokine CCL2 has been consistently found to be up-regulated in systemic sclerosis. To explore the potential value of serum CCL2 measurement in disease assessment, we have compared CCL2 levels with clinical phenotype and investigated effect of therapy on circulating CCL2. METHODS: Serum samples from a well characterised cohort of 94 systemic sclerosis (SSc) patients, 16 patients with primary Raynaud phenomenon and 11 healthy controls were examined by ELISA. Our cohort of patients included 50 patients with limited cutaneous (lc)SSc (20 with lcSSc alone and 30 with pulmonary arterial hypertension-lcSSc), and 44 with diffuse cutaneous (dc)SSc, 30 of which had early-onset dcSSc. RESULTS: Serum levels of CCL2 were increased in both major SSc subsets. In early stage dcSSc 18/30 (60%) cases demonstrated markedly elevated CCL2, and this was associated with anti-topoisomerase or anti-RNA polymerase I/III antibody reactivity, and with greater frequency of major organ-based complications. Elevation of CCL2 serum levels in the lcSSc subset was not associated with pulmonary arterial hypertension, although there was a trend for reduction following treatment with prostacyclin analogues or bosentan. CONCLUSION: These findings suggest that the CCL2/CCR2 axis is a potential therapeutic target in SSc, particularly in the early dcSSc subset. CCL2 measurement may be useful for risk stratification in early stage disease, but its value in disease monitoring is questionable. FAU - Carulli, M T AU - Carulli MT AD - Centre for Rheumatology, Royal Free and University College Medical School, Rowland Hill Street, Hampstead, London, NW3 2PF, UK. FAU - Handler, C AU - Handler C FAU - Coghlan, J G AU - Coghlan JG FAU - Black, C M AU - Black CM FAU - Denton, C P AU - Denton CP LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070629 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Biomarkers) RN - 0 (Chemokine CCL2) SB - IM MH - Adult MH - Aged MH - Biomarkers/blood MH - Case-Control Studies MH - Chemokine CCL2/*blood MH - Chi-Square Distribution MH - Cross-Sectional Studies MH - Female MH - Humans MH - Hypertension, Pulmonary/blood MH - Male MH - Middle Aged MH - Raynaud Disease/blood MH - Risk Assessment/methods MH - Scleroderma, Diffuse/blood MH - Scleroderma, Limited/blood MH - Scleroderma, Systemic/*blood MH - Treatment Outcome EDAT- 2007/07/03 09:00 MHDA- 2008/01/15 09:00 CRDT- 2007/07/03 09:00 PHST- 2007/07/03 09:00 [pubmed] PHST- 2008/01/15 09:00 [medline] PHST- 2007/07/03 09:00 [entrez] AID - S0003-4967(24)21951-8 [pii] AID - 10.1136/ard.2006.067967 [doi] PST - ppublish SO - Ann Rheum Dis. 2008 Jan;67(1):105-9. doi: 10.1136/ard.2006.067967. Epub 2007 Jun 29. PMID- 20635726 OWN - NLM STAT- MEDLINE DCOM- 20101207 LR - 20161125 IS - 1027-6661 (Print) IS - 1027-6661 (Linking) VI - 16 IP - 1 DP - 2010 TI - [Falconer-Weddel's costoclavicular syndrome]. PG - 121-4 AB - Analysed herein are the findings of examination and outcomes of surgical management of fifty-eight patients (25 men and 33 women) presenting with Falconer-Weddel's costoclavicular syndrome in which the subclavian artery and vein and the brachial plexus at the thoracic outlet appear to be compressed. Of the 58 patients,five subjects were found to have a rudimentary cervical rib and three more (5.3%) patients had trophic disorders on their digits fingers: dystrophy of the nail plates, their fragility, periodically opening trophic ulcers at the digital tips and one patient presented with gangrene of the inguinal phalanx of the middle finger. Three patients had hyperemia of the face. A further four patients had roughening, hyperkeratosis of the skin of the hands, cracks. The presence of the supernumerary ribs was determined roentgenologically. Haemodynamics was studied using Doppler ultrasonography making it possible to reveal disordered blood flow in the upper-limb arteries in the physiological position assumed, in the Adson test as well as with Raynaud syndrome, which was observed in 39 patients. The function of the nerves was studied using electroneuromyography (ENMG). All patients were operated on under endotracheal anaesthesia. Decompression-medical operations were carried outperformed in all 58 patients, with the following four types of interventions being performed: transaxillary resection of the first rib combined with sympathectomy carried out in 23 patients, resection of the first rib without sympathectomy in eighteen patients, resection of the first rib via a surpraclavicular approach in four patients, scalenotomy and selective cervicothoracic sympathectomy in 13 patients. FAU - Sultanov, D D AU - Sultanov DD FAU - Kurbanov, N R AU - Kurbanov NR FAU - Abdulloev, N K AU - Abdulloev NK LA - rus PT - Comparative Study PT - Journal Article PL - Russia (Federation) TA - Angiol Sosud Khir JT - Angiologiia i sosudistaia khirurgiia = Angiology and vascular surgery JID - 9604504 SB - IM MH - *Cervical Rib/diagnostic imaging/physiopathology MH - Cervical Rib Syndrome/complications/diagnosis/diagnostic imaging/physiopathology/surgery MH - Decompression, Surgical MH - Electromyography MH - Female MH - Hemodynamics MH - Humans MH - Male MH - Radiography MH - Raynaud Disease/diagnosis MH - Sympathectomy MH - Thoracic Outlet Syndrome/complications/diagnosis/diagnostic imaging/physiopathology/*surgery EDAT- 2010/07/20 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/07/20 06:00 PHST- 2010/07/20 06:00 [entrez] PHST- 2010/07/20 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] PST - ppublish SO - Angiol Sosud Khir. 2010;16(1):121-4. PMID- 19247207 OWN - NLM STAT- MEDLINE DCOM- 20091012 LR - 20090721 IS - 1539-2031 (Electronic) IS - 0192-0790 (Linking) VI - 43 IP - 7 DP - 2009 Aug TI - Primary biliary cirrhosis complicated with interstitial lung disease: a prospective study in 178 patients. PG - 676-9 LID - 10.1097/MCG.0b013e31818aa11e [doi] AB - OBJECTIVE: To analyze the clinical features and prognosis of patients with primary biliary cirrhosis (PBC) complicated with interstitial lung disease (ILD). METHODS: One hundred and seventy-eight consecutive PBC patients, who were admitted to Peking Union Medical College Hospital from January 2001 to March 2007, were included in this prospective study. A structured interview, systemic rheumatologic examination, laboratory tests (including autoantibodies), and high resolution computed tomography were conducted for each patient and compared between patients with and without ILD. RESULTS: Twenty-eight (15.7%) PBC patients had ILD, and 53.6% of them had respiratory symptoms including dyspnea and cough, 88.2% showed diffusing and restrictive ventilation impairment on pulmonary function test. PBC patients with ILD were older and had higher erythrocyte sedimentation rate levels than those without ILD, P<0.05. There was no significant difference in liver biochemical parameters, positive rates of antinuclear antibodies, antimitochondrial antibodies (AMA), AMA-M2, anticentromere antibodies, anticardiolipin antibodies, and Mayo risk score between the 2 groups, P>0.05. Logistic regression analysis found that Raynaud phenomenon and association with other connective tissue diseases (CTDs) were risk factors for PBC patients to develop ILD (P=0.04, odds ratio=3.12 and P=0.01, odds ratio=3.18, respectively), though 42.9% of ILD-PBC patients did not have other CTDs. CONCLUSIONS: ILD is a common and clinically significant complication of PBC. PBC patients who have Raynaud phenomenon and other CTDs are more likely to develop ILD. FAU - Shen, Min AU - Shen M AD - Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China. FAU - Zhang, Fengchun AU - Zhang F FAU - Zhang, Xuan AU - Zhang X LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Clin Gastroenterol JT - Journal of clinical gastroenterology JID - 7910017 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Autoantibodies/blood MH - Blood Sedimentation MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/*complications/diagnosis/physiopathology MH - Logistic Models MH - Lung Diseases, Interstitial/diagnosis/*etiology/physiopathology MH - Male MH - Middle Aged MH - Prognosis MH - Prospective Studies MH - Raynaud Disease/complications MH - Respiratory Function Tests MH - Risk Factors MH - Tomography, X-Ray Computed MH - Young Adult EDAT- 2009/02/28 09:00 MHDA- 2009/10/13 06:00 CRDT- 2009/02/28 09:00 PHST- 2009/02/28 09:00 [entrez] PHST- 2009/02/28 09:00 [pubmed] PHST- 2009/10/13 06:00 [medline] AID - 00004836-200908000-00013 [pii] AID - 10.1097/MCG.0b013e31818aa11e [doi] PST - ppublish SO - J Clin Gastroenterol. 2009 Aug;43(7):676-9. doi: 10.1097/MCG.0b013e31818aa11e. PMID- 26210124 OWN - NLM STAT- MEDLINE DCOM- 20160509 LR - 20250529 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 41 IP - 3 DP - 2015 Aug TI - The Pathogenesis of Systemic Sclerosis. PG - 367-82 LID - S0889-857X(15)00023-X [pii] LID - 10.1016/j.rdc.2015.04.002 [doi] AB - Systemic sclerosis is a multisystem disorder with a high associated mortality. The hallmark abnormalities of the disease are in the immune system, vasculature, and connective tissue. Systemic sclerosis occurs in susceptible individuals and is stimulated by initiating events that are poorly understood at present. In order for the disease phenotype to appear there is dysfunction in the homoeostatic mechanisms of immune tolerance, endothelial physiology, and extracellular matrix turnover. The progression of disease is not sequential but requires simultaneous dysfunction in these normal regulatory mechanisms. Better understanding of the interplay of these factors is likely to contribute to improved treatment options. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Stern, Edward P AU - Stern EP AD - Centre for Rheumatology, UCL Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, UCL Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK. Electronic address: c.denton@ucl.ac.uk. LA - eng GR - MR/K015230/1/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Review DEP - 20150520 PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 RN - 0 (Autoantibodies) RN - 0 (Chemokines) RN - 0 (Cytokines) RN - 0 (HLA Antigens) RN - 0 (Interleukins) RN - 0 (Vasoconstrictor Agents) RN - 139568-91-5 (Connective Tissue Growth Factor) SB - IM MH - Autoantibodies/immunology MH - Chemokines/physiology MH - Connective Tissue Growth Factor/physiology MH - Cytokines/physiology MH - Disease Progression MH - Endocrine System/physiology MH - Female MH - Fibroblasts/physiology MH - Genome-Wide Association Study MH - HLA Antigens/immunology MH - Humans MH - Immunity, Cellular/physiology MH - Infections/complications MH - Interleukins/physiology MH - Neoplasms/complications MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*etiology/genetics/immunology MH - Vasoconstrictor Agents/pharmacology OTO - NOTNLM OT - Autoimmunity OT - Fibrosis OT - Pathogenesis OT - Scleroderma OT - Systemic sclerosis OT - Vasculopathy EDAT- 2015/07/27 06:00 MHDA- 2016/05/10 06:00 CRDT- 2015/07/27 06:00 PHST- 2015/07/27 06:00 [entrez] PHST- 2015/07/27 06:00 [pubmed] PHST- 2016/05/10 06:00 [medline] AID - S0889-857X(15)00023-X [pii] AID - 10.1016/j.rdc.2015.04.002 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2015 Aug;41(3):367-82. doi: 10.1016/j.rdc.2015.04.002. Epub 2015 May 20. PMID- 25559920 OWN - NLM STAT- MEDLINE DCOM- 20150601 LR - 20260128 IS - 1933-0715 (Electronic) IS - 1933-0707 (Linking) VI - 15 IP - 3 DP - 2015 Mar TI - Mechanical thrombectomy for pediatric stroke arising from an atrial myxoma: case report. PG - 301-5 LID - 10.3171/2014.10.PEDS14292 [doi] AB - Children experiencing severe neurological deficit due to acute ischemic stroke may benefit from endovascular intervention. The authors describe the use of mechanical thrombectomy in the treatment of embolic occlusion secondary to an atrial myxoma in a pediatric patient. This case involved an 11-year-old boy with a history notable for Raynaud syndrome and a distal extremity rash who presented to the emergency department with dense hemiparesis secondary to thromboembolic occlusion of the M1 segment of the middle cerebral artery. Following mechanical thrombectomy, the patient's pediatric National Institutes of Health Stroke Scale score improved from a 16 to a 7. In the setting of acute pediatric stroke due to atrial myxoma emboli, mechanical thrombectomy may be a first-line therapy. FAU - Vega, Rafael A AU - Vega RA AD - Departments of 1 Neurosurgery and. FAU - Chan, Julie L AU - Chan JL FAU - Anene-Maidoh, Tony I AU - Anene-Maidoh TI FAU - Grimes, Margaret M AU - Grimes MM FAU - Reavey-Cantwell, John F AU - Reavey-Cantwell JF LA - eng PT - Case Reports PT - Journal Article DEP - 20150110 PL - United States TA - J Neurosurg Pediatr JT - Journal of neurosurgery. Pediatrics JID - 101463759 SB - IM MH - Cerebral Angiography MH - Child MH - Heart Neoplasms/*complications/*diagnosis MH - Humans MH - Male MH - Middle Cerebral Artery/diagnostic imaging/*surgery MH - Myxoma/*complications/*diagnosis MH - *Neoplastic Cells, Circulating MH - Raynaud Disease/etiology MH - Stroke/*etiology/surgery MH - *Thrombectomy MH - Treatment Outcome MH - United States OTO - NOTNLM OT - AIS = acute ischemic stroke OT - AP = anteroposterior OT - IV = intravenous OT - MCA = middle cerebral artery OT - NIHSS = National Institutes of Health Stroke Scale OT - PCoA = posterior communicating artery OT - atrial myxoma OT - cerebral emboli OT - mechanical thrombectomy OT - pediatric stroke OT - r-tPA = recombinant tissue plasminogen activator OT - vascular disorders EDAT- 2015/01/07 06:00 MHDA- 2015/06/02 06:00 CRDT- 2015/01/07 06:00 PHST- 2015/01/07 06:00 [entrez] PHST- 2015/01/07 06:00 [pubmed] PHST- 2015/06/02 06:00 [medline] AID - 10.3171/2014.10.PEDS14292 [doi] PST - ppublish SO - J Neurosurg Pediatr. 2015 Mar;15(3):301-5. doi: 10.3171/2014.10.PEDS14292. Epub 2015 Jan 10. PMID- 24953719 OWN - NLM STAT- MEDLINE DCOM- 20150421 LR - 20140725 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 94 DP - 2014 Jul TI - The digital thermal hyperemia pattern is associated with the onset of digital ulcerations in systemic sclerosis during 3 years of follow-up. PG - 119-22 LID - S0026-2862(14)00091-0 [pii] LID - 10.1016/j.mvr.2014.06.005 [doi] AB - OBJECTIVES: One of the most important skin complications in systemic sclerosis (SSc) is digital ulceration. Local thermal hyperemia (LTH) in the skin is a biphasic response to local heating involving both neurovascular and endothelial responses. Since LTH is abnormal in SSc patients, we aimed at testing whether LTH could be a prognostic tool for the onset of digital ulcers. METHODS: We prospectively enrolled 51 patients with SSc. Nailfold capillaroscopy and LTH were recorded at baseline, and patients were followed for 3 years. RESULTS: No patient with a LTH peak/plateau ratio ≥1 (n=19) developed digital ulcerations during the 3 year follow-up (100% negative predictive value), while 6 out of 32 patients with a LTH peak/plateau ratio <1 at enrolment presented with finger pad ulcerations within 3 years (p=0.05). In contrast, when lidocaine/prilocaine was applied to the finger pad, no relationship between thermal hyperemia and digital ulcerations was observed. CONCLUSIONS: A LTH peak/plateau ratio on the finger pad greater than 1, which can easily be determined in routine clinical practice, could be used to reassure patients, whatever the subtype of SSc, about the low probability of future digital ulceration. However, the prognostic value of this parameter should be confirmed in a larger cohort. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Blaise, S AU - Blaise S AD - Vascular Medicine Department, Grenoble University Hospital, F-38000 Grenoble, France; Inserm ERI17, Grenoble Medical School, F-38000 Grenoble, France. Electronic address: SBlaise@chu-grenoble.fr. FAU - Roustit, M AU - Roustit M AD - Inserm ERI17, Grenoble Medical School, F-38000 Grenoble, France; Inserm CIC3, Grenoble Clinical Research Center, Grenoble University Hospital, F-38000 Grenoble, France. FAU - Carpentier, P AU - Carpentier P AD - Vascular Medicine Department, Grenoble University Hospital, F-38000 Grenoble, France. FAU - Seinturier, C AU - Seinturier C AD - Vascular Medicine Department, Grenoble University Hospital, F-38000 Grenoble, France. FAU - Imbert, B AU - Imbert B AD - Vascular Medicine Department, Grenoble University Hospital, F-38000 Grenoble, France. FAU - Cracowski, J L AU - Cracowski JL AD - Inserm ERI17, Grenoble Medical School, F-38000 Grenoble, France; Inserm CIC3, Grenoble Clinical Research Center, Grenoble University Hospital, F-38000 Grenoble, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140619 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Female MH - Fingers/*pathology MH - Follow-Up Studies MH - Humans MH - Hyperemia/*etiology MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation/physiology MH - Microscopic Angioscopy MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*pathology MH - Skin/blood supply MH - Skin Ulcer/diagnosis/*pathology MH - Temperature MH - Time Factors MH - Vasodilation/physiology OTO - NOTNLM OT - Capillaroscopy OT - Digital ulcer OT - Fluxmetry OT - Laser Doppler OT - Microcirculation OT - Microvascular blood flow OT - Prognosis value OT - Raynaud OT - Systemic sclerosis OT - Thermal hyperemia EDAT- 2014/06/24 06:00 MHDA- 2015/04/22 06:00 CRDT- 2014/06/24 06:00 PHST- 2014/04/23 00:00 [received] PHST- 2014/06/06 00:00 [revised] PHST- 2014/06/10 00:00 [accepted] PHST- 2014/06/24 06:00 [entrez] PHST- 2014/06/24 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] AID - S0026-2862(14)00091-0 [pii] AID - 10.1016/j.mvr.2014.06.005 [doi] PST - ppublish SO - Microvasc Res. 2014 Jul;94:119-22. doi: 10.1016/j.mvr.2014.06.005. Epub 2014 Jun 19. PMID- 22078040 OWN - NLM STAT- MEDLINE DCOM- 20120330 LR - 20111114 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 138 IP - 11 DP - 2011 Nov TI - [Digital ulcers in systemic scleroderma]. PG - 762-8; quiz 761, 769 LID - 10.1016/j.annder.2011.06.011 [doi] FAU - Lok, C AU - Lok C AD - Service de dermatologie, hôpital sud, CHU, université Picardie-Jules-Verne, 80054 Amiens cedex 1, France. Lok.catherine@chu-amiens.fr FAU - Mouthon, L AU - Mouthon L FAU - Ségard, M AU - Ségard M FAU - Richard, M-A AU - Richard MA FAU - Guillevin, L AU - Guillevin L LA - fre PT - Journal Article PT - Review TT - Les ulcères digitaux de la sclérodermie systémique. DEP - 20111011 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Analgesics) RN - 0 (Anti-Anxiety Agents) RN - 0 (Emollients) RN - 0 (Vasodilator Agents) SB - IM MH - Analgesics/therapeutic use MH - Anti-Anxiety Agents/therapeutic use MH - Arterioles/pathology MH - Emollients/therapeutic use MH - Female MH - Fingers/*blood supply/pathology MH - Gangrene MH - Hand Deformities, Acquired/etiology/prevention & control MH - Humans MH - Ischemia/etiology MH - Male MH - Necrosis MH - Occlusive Dressings MH - Patient Education as Topic MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/epidemiology/physiopathology MH - Skin Diseases, Infectious/etiology/prevention & control MH - Skin Transplantation MH - Skin Ulcer/drug therapy/*etiology/physiopathology/therapy MH - Vasodilator Agents/therapeutic use EDAT- 2011/11/15 06:00 MHDA- 2012/03/31 06:00 CRDT- 2011/11/15 06:00 PHST- 2011/03/01 00:00 [received] PHST- 2011/06/13 00:00 [revised] PHST- 2011/06/24 00:00 [accepted] PHST- 2011/11/15 06:00 [entrez] PHST- 2011/11/15 06:00 [pubmed] PHST- 2012/03/31 06:00 [medline] AID - S0151-9638(11)00362-0 [pii] AID - 10.1016/j.annder.2011.06.011 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2011 Nov;138(11):762-8; quiz 761, 769. doi: 10.1016/j.annder.2011.06.011. Epub 2011 Oct 11. PMID- 32856492 OWN - NLM STAT- MEDLINE DCOM- 20201204 LR - 20201214 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 49 IP - 6 DP - 2020 Nov TI - Impact of the COVID-19 outbreak on an Italian cohort of systemic sclerosis patients. PG - 505-506 LID - 10.1080/03009742.2020.1800083 [doi] FAU - Bellan, M AU - Bellan M AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - CAAD, Centre for Autoimmune and Allergic Diseases , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Parisi, S AU - Parisi S AD - Rheumatology Unit, AOU City of Health and Science University Hospital of Turin , Turn, Italy. FAU - Stobbione, P AU - Stobbione P AD - Rheumatology Unit, SS Antonio e Biagio and Cesare Arrigo Hospital , Alessandria, Italy. FAU - Pedrinelli, A R AU - Pedrinelli AR AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Rizzi, E AU - Rizzi E AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Casciaro, G F AU - Casciaro GF AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Vassia, V AU - Vassia V AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Landi, R AU - Landi R AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Cittone, M G AU - Cittone MG AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Rigamonti, C AU - Rigamonti C AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Patrucco, F AU - Patrucco F AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. FAU - Ditto, M C AU - Ditto MC AD - Rheumatology Unit, AOU City of Health and Science University Hospital of Turin , Turn, Italy. FAU - Finucci, A AU - Finucci A AD - Rheumatology Unit, AOU City of Health and Science University Hospital of Turin , Turn, Italy. FAU - Realmuto, C AU - Realmuto C AD - Rheumatology Unit, AOU City of Health and Science University Hospital of Turin , Turn, Italy. FAU - Todoerti, M AU - Todoerti M AD - Rheumatology Unit, SS Antonio e Biagio and Cesare Arrigo Hospital , Alessandria, Italy. FAU - Parodi, M AU - Parodi M AD - Rheumatology Unit, SS Antonio e Biagio and Cesare Arrigo Hospital , Alessandria, Italy. FAU - Rossi, P AU - Rossi P AD - Rheumatology Unit, SS Antonio e Biagio and Cesare Arrigo Hospital , Alessandria, Italy. FAU - Pirisi, M AU - Pirisi M AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - CAAD, Centre for Autoimmune and Allergic Diseases , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. FAU - Fusaro, E AU - Fusaro E AD - Rheumatology Unit, AOU City of Health and Science University Hospital of Turin , Turn, Italy. FAU - Sainaghi, P P AU - Sainaghi PP AD - Department of Translational Medicine, University of Eastern Piedmont UPO , Novara, Italy. AD - CAAD, Centre for Autoimmune and Allergic Diseases , Novara, Italy. AD - Internal Medicine Division and Rheumatology Unit, AOU University Hospital Maggiore della Carita , Novara, Italy. LA - eng PT - Letter DEP - 20200828 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antirheumatic Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Antirheumatic Agents/therapeutic use MH - *COVID-19 MH - Cohort Studies MH - Female MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Italy MH - Lung Diseases, Interstitial/*physiopathology/therapy MH - Male MH - Middle Aged MH - Oxygen Inhalation Therapy MH - Pulmonary Arterial Hypertension/*physiopathology/therapy MH - Raynaud Disease/*physiopathology MH - Retrospective Studies MH - SARS-CoV-2 MH - Scleroderma, Systemic/drug therapy/*physiopathology MH - Telemedicine MH - Telephone MH - Ulcer/*physiopathology EDAT- 2020/08/29 06:00 MHDA- 2020/12/15 06:00 CRDT- 2020/08/29 06:00 PHST- 2020/08/29 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2020/08/29 06:00 [entrez] AID - 10.1080/03009742.2020.1800083 [doi] PST - ppublish SO - Scand J Rheumatol. 2020 Nov;49(6):505-506. doi: 10.1080/03009742.2020.1800083. Epub 2020 Aug 28. PMID- 23731933 OWN - NLM STAT- MEDLINE DCOM- 20130801 LR - 20220408 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 27 IP - 2 DP - 2013 Apr TI - How to perform and interpret capillaroscopy. PG - 237-48 LID - S1521-6942(13)00029-6 [pii] LID - 10.1016/j.berh.2013.03.001 [doi] AB - The essence of capillaroscopy is to examine, noninvasively and safely the morphology of nailfold dermal papillary capillaries using a magnification system (microscopical lenses). Capillaroscopy may be performed with lenses with low (×20) and with high magnification (×200 up to ×600). The video-capillaroscope consists of an optical/digital probe which is moved to the finger of the patient and allows direct contact with the nailfold. Through qualitative assessment a normal capillaroscopy can be distinguished from a pathognomonic abnormal one due most frequently to systemic sclerosis (SSc). This pattern recognition relies on evaluating the morphology of the capillaries, their density (number) and dimensions 'at sight' of the capillaries and their architecture. In SSc three progressive capillaroscopic patterns have been described ('early', 'active' and 'late'). Quantitative assessment (quantitation of certain characteristics and semi-quantitative scoring) of the capillaroscopic pictures may also be performed. Qualitative and semi-quantitative assessments are used to predict SSc clinical complications. In other connective tissue diseases (CTDs) prospective clinical studies resulting in indices which can predict future clinical complications have not been published, as yet. CI - Copyright © 2013 Elsevier Ltd. All rights reserved. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genova, Viale Benedetto XV, no 6, 16132 Genova, Italy. mcutolo@unige.it FAU - Sulli, Alberto AU - Sulli A FAU - Smith, Vanessa AU - Smith V LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Capillaries/*pathology MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Raynaud Disease/*diagnosis MH - Scleroderma, Systemic/*diagnosis EDAT- 2013/06/05 06:00 MHDA- 2013/08/02 06:00 CRDT- 2013/06/05 06:00 PHST- 2013/06/05 06:00 [entrez] PHST- 2013/06/05 06:00 [pubmed] PHST- 2013/08/02 06:00 [medline] AID - S1521-6942(13)00029-6 [pii] AID - 10.1016/j.berh.2013.03.001 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2013 Apr;27(2):237-48. doi: 10.1016/j.berh.2013.03.001. PMID- 35488070 OWN - NLM STAT- MEDLINE DCOM- 20221104 LR - 20221109 IS - 1476-5640 (Electronic) IS - 0954-3007 (Linking) VI - 76 IP - 11 DP - 2022 Nov TI - Serum vitamin B12 and D levels in children with Primary Raynaud Phenomenon: a retrospective cohort study. PG - 1615-1617 LID - 10.1038/s41430-022-01151-0 [doi] AB - Primary Raynaud phenomenon (RP) is resultant from transient vasospasm of peripheral arteries and arterioles, is usually precipitated by cold exposure or emotional stress, without any clue for autoimmune connective tissue diseases. We aimed to determine the frequency of vitamin D and B12 deficiencies in pediatric patients with primary RP, and to investigate their roles on the disease course. Vitamin B12 and D were supplemented if the patients had deficiencies. The study included 40 children with primary RP, 29 (72.5%) female and 11 (27.5%) male. The mean and median age were 15.1 ± 1.8 and 15.5 (range, 11.5-17.8) years. Symptoms were improved in 31 (77.5%) patients with warming procedures. Seventeen (41.5%) and 16 (39%) patients had low serum vitamin B12 and D levels, respectively. Vasodilator treatment requirement did not change by vitamin B12 status but was significantly lower in vitamin D deficient and replaced patients. Further studies are needed to clarify our results. CI - © 2022. The Author(s), under exclusive licence to Springer Nature Limited. FAU - Kisla Ekinci, Rabia Miray AU - Kisla Ekinci RM AUID- ORCID: 0000-0001-6234-822X AD - Department of Pediatric Rheumatology, Adana City Training and Research Hospital, Adana, Turkey. mir_kisla@hotmail.com. FAU - Taskin Karacay, Isil Ezel AU - Taskin Karacay IE AD - Department of Pediatrics, Adana City Training and Research Hospital, Adana, Turkey. FAU - Celik, Umit AU - Celik U AD - Department of Pediatric Infectious Diseases, Adana City Training and Research Hospital, Adana, Turkey. LA - eng PT - Journal Article DEP - 20220429 PL - England TA - Eur J Clin Nutr JT - European journal of clinical nutrition JID - 8804070 RN - P6YC3EG204 (Vitamin B 12) RN - 1406-16-2 (Vitamin D) RN - 935E97BOY8 (Folic Acid) SB - IM MH - Humans MH - Male MH - Female MH - Child MH - Adolescent MH - *Vitamin B 12 Deficiency/epidemiology MH - Retrospective Studies MH - Vitamin B 12 MH - Vitamin D MH - *Raynaud Disease MH - Folic Acid EDAT- 2022/04/30 06:00 MHDA- 2022/11/05 06:00 CRDT- 2022/04/29 23:26 PHST- 2022/01/26 00:00 [received] PHST- 2022/04/20 00:00 [accepted] PHST- 2022/04/15 00:00 [revised] PHST- 2022/04/30 06:00 [pubmed] PHST- 2022/11/05 06:00 [medline] PHST- 2022/04/29 23:26 [entrez] AID - 10.1038/s41430-022-01151-0 [pii] AID - 10.1038/s41430-022-01151-0 [doi] PST - ppublish SO - Eur J Clin Nutr. 2022 Nov;76(11):1615-1617. doi: 10.1038/s41430-022-01151-0. Epub 2022 Apr 29. PMID- 18044155 OWN - NLM STAT- MEDLINE DCOM- 20080214 LR - 20190724 IS - 0021-5384 (Print) IS - 0021-5384 (Linking) VI - 96 IP - 10 DP - 2007 Oct 10 TI - [Diagnosis and therapy for mixed connective tissue diseases]. PG - 2196-200 FAU - Nonomura, Yoshinori AU - Nonomura Y FAU - Miyasaka, Nobuyuki AU - Miyasaka N LA - jpn PT - Journal Article PT - Review PL - Japan TA - Nihon Naika Gakkai Zasshi JT - Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine JID - 19130210R RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Antinuclear) RN - 0 (Calcium Channel Blockers) RN - 0 (Immunosuppressive Agents) RN - 0 (Platelet Aggregation Inhibitors) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 1406-18-4 (Vitamin E) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Anti-Inflammatory Agents/therapeutic use MH - Antibodies, Antinuclear MH - Calcium Channel Blockers/therapeutic use MH - Epoprostenol/therapeutic use MH - Female MH - Humans MH - Hypertension, Pulmonary MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Mixed Connective Tissue Disease/*diagnosis/immunology/physiopathology/*therapy MH - Platelet Aggregation Inhibitors/therapeutic use MH - Raynaud Disease MH - Referral and Consultation MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Syndrome MH - Vitamin E/therapeutic use RF - 5 EDAT- 2007/11/30 09:00 MHDA- 2008/02/15 09:00 CRDT- 2007/11/30 09:00 PHST- 2007/11/30 09:00 [pubmed] PHST- 2008/02/15 09:00 [medline] PHST- 2007/11/30 09:00 [entrez] AID - 10.2169/naika.96.2196 [doi] PST - ppublish SO - Nihon Naika Gakkai Zasshi. 2007 Oct 10;96(10):2196-200. doi: 10.2169/naika.96.2196. PMID- 26377236 OWN - NLM STAT- MEDLINE DCOM- 20161101 LR - 20170103 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 25 IP - 2 DP - 2016 Feb TI - A case of mixed connective tissue disease with pseudo-pseudo Meigs' syndrome (PPMS)-like features. PG - 214-6 LID - 10.1177/0961203315606441 [doi] AB - Pseudo-pseudo Meigs' syndrome (PPMS) has been reported to be a rare presentation of patients with systemic lupus erythematosus (SLE). However, such a presentation is not common in other forms of connective tissue disease. We presented a case of gross ascites, pleural effusion, and marked elevation of CA-125 level (PPMS-like features) that led to a diagnosis of MCTD. The patient responded to systemic steroid therapy. CI - © The Author(s) 2015. FAU - Cheah, C K AU - Cheah CK AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia cheeken78@gmail.com. FAU - Ramanujam, S AU - Ramanujam S AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia. FAU - Mohd Noor, N AU - Mohd Noor N AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia. FAU - Gandhi, C AU - Gandhi C AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia. FAU - D Souza, Beryl A AU - D Souza BA AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia. FAU - Gun, S C AU - Gun SC AD - Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia. LA - eng PT - Case Reports PT - Journal Article DEP - 20150915 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Anti-Inflammatory Agents) RN - 9PHQ9Y1OLM (Prednisolone) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Anti-Inflammatory Agents/administration & dosage MH - Diagnosis, Differential MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/pathology MH - Meigs Syndrome/*diagnosis/diagnostic imaging/drug therapy/pathology MH - Methylprednisolone/administration & dosage MH - Mixed Connective Tissue Disease/*diagnosis/drug therapy/pathology MH - Prednisolone/administration & dosage MH - Radiography MH - Raynaud Disease/*pathology OTO - NOTNLM OT - CA-125 OT - Mixed connective tissue disease OT - pseudo-pseudo Meigs’ syndrome EDAT- 2015/09/18 06:00 MHDA- 2016/11/02 06:00 CRDT- 2015/09/18 06:00 PHST- 2015/07/05 00:00 [received] PHST- 2015/08/25 00:00 [accepted] PHST- 2015/09/18 06:00 [entrez] PHST- 2015/09/18 06:00 [pubmed] PHST- 2016/11/02 06:00 [medline] AID - 0961203315606441 [pii] AID - 10.1177/0961203315606441 [doi] PST - ppublish SO - Lupus. 2016 Feb;25(2):214-6. doi: 10.1177/0961203315606441. Epub 2015 Sep 15. PMID- 24964485 OWN - NLM STAT- MEDLINE DCOM- 20140905 LR - 20161125 IS - 2158-1797 (Electronic) IS - 1548-8578 (Linking) VI - 33 IP - 10 DP - 2012 Nov-Dec TI - Complete transition from failing restorations to implant-supported fixed prostheses in a patient with scleroderma. PG - 746-56 AB - In a case involving a patient with scleroderma, the authors demonstrate how to treatment plan and sequence the transition from a failing restored dentition to complete implant-supported fixed prostheses with sequential extractions and implant placements. The article also presents surgical and prosthodontic considerations for a complete-mouth implant-supported fixed rehabilitation while achieving optimal esthetics without compromising function in patients with this condition. Sequential treatment provided the patient with fixed provisional restorations during treatment in multiple, short surgical appointments with less psychosocial trauma. This case posed treatment challenges due to limited oral access, unpredictable disease progression, and limited data on the success of treatment with endosseous dental implants in these patients. FAU - Nam, Jung AU - Nam J FAU - Janakievski, Jim AU - Janakievski J FAU - Raigrodski, Ariel J AU - Raigrodski AJ LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Compend Contin Educ Dent JT - Compendium of continuing education in dentistry (Jamesburg, N.J. : 1995) JID - 9600713 MH - Aged MH - *Dental Prosthesis, Implant-Supported MH - Dental Restoration Failure MH - Denture Design MH - *Denture, Partial, Fixed MH - Female MH - Humans MH - Jaw, Edentulous, Partially/diagnostic imaging/*rehabilitation MH - Malocclusion, Angle Class II/diagnostic imaging/rehabilitation MH - Patient Care Planning MH - Radiography, Panoramic MH - Raynaud Disease/complications MH - Scleroderma, Systemic/*complications MH - Sinus Floor Augmentation MH - Sjogren's Syndrome/complications MH - Tooth Extraction EDAT- 2012/11/01 00:00 MHDA- 2014/09/06 06:00 CRDT- 2014/06/27 06:00 PHST- 2014/06/27 06:00 [entrez] PHST- 2012/11/01 00:00 [pubmed] PHST- 2014/09/06 06:00 [medline] PST - ppublish SO - Compend Contin Educ Dent. 2012 Nov-Dec;33(10):746-56. PMID- 28420068 OWN - NLM STAT- MEDLINE DCOM- 20180507 LR - 20260127 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 26 IP - 10 DP - 2017 Sep TI - Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus. PG - 1089-1094 LID - 10.1177/0961203317699286 [doi] AB - Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters. We aimed to ascertain if PTX3 might be a significant mediator in cSLE and if it might indicate active vasculitis during the course of the disease. Methods Serum PTX3 levels were measured in 76 patients with cSLE and 41 healthy subjects. We have investigated its relation with disease activity, damage, clinical features, laboratory parameters and medications. Results Serum levels of PTX3 were found to be increased in cSLE compared to healthy controls (mean ± SD; 10.6 ± 8.2 ng/mL vs 2.7 ± 1.3 ng/mL, p < 0.001). PTX3 concentrations were also in correlation with SLEDAI-2K ( r = 0.57, p < 0.001). When viewed from the clinical perspective, serum PTX3 levels were significantly higher only in patients with active vasculitis ( p < 0.001), Raynaud phenomenon ( p = 0.006) and mucocutaneous manifestations ( p < 0.001). However, an association between PTX3 and age, age at disease onset, disease duration, complement levels, PedSDI score (pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), ESR, CRP, procalcitonin levels, anti-ds DNA antibody, anticardiolipin antibodies was not detected. Conclusions Patients with cSLE have increased levels of serum PTX3 compared to healthy controls. Thus, serum PTX-3 level might be a significant mediator in cSLE. Apart from these, the results support that PTX3 reflects active cutaneous vasculitis in cSLE and correlates with disease activity. FAU - Sahin, S AU - Sahin S AD - 1 Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Adrovic, A AU - Adrovic A AD - 1 Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Barut, K AU - Barut K AD - 1 Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Durmus, S AU - Durmus S AD - 2 Department of Biochemistry, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Gelisgen, R AU - Gelisgen R AD - 2 Department of Biochemistry, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Uzun, H AU - Uzun H AD - 2 Department of Biochemistry, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. FAU - Kasapcopur, O AU - Kasapcopur O AD - 1 Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. LA - eng PT - Journal Article DEP - 20170314 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 9007-41-4 (C-Reactive Protein) RN - 0 (Serum Amyloid P-Component) RN - 0 (Pentraxins) SB - IM MH - Adolescent MH - Age Factors MH - Age of Onset MH - C-Reactive Protein/*metabolism MH - Case-Control Studies MH - Child MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*blood/physiopathology MH - Male MH - Raynaud Disease/etiology MH - Serum Amyloid P-Component/*metabolism MH - Severity of Illness Index MH - Vasculitis/blood/*etiology MH - Young Adult MH - Pentraxins OTO - NOTNLM OT - Biomarker OT - Childhood-onset systemic lupus erythematosus OT - Pentraxin-3 OT - SLEDAI OT - Vasculitis EDAT- 2017/04/20 06:00 MHDA- 2018/05/08 06:00 CRDT- 2017/04/20 06:00 PHST- 2017/04/20 06:00 [pubmed] PHST- 2018/05/08 06:00 [medline] PHST- 2017/04/20 06:00 [entrez] AID - 10.1177/0961203317699286 [doi] PST - ppublish SO - Lupus. 2017 Sep;26(10):1089-1094. doi: 10.1177/0961203317699286. Epub 2017 Mar 14. PMID- 25830246 OWN - NLM STAT- MEDLINE DCOM- 20160708 LR - 20150402 IS - 1085-5629 (Print) IS - 1085-5629 (Linking) VI - 33 IP - 4 DP - 2014 Dec TI - Update on botulinum neurotoxin use in aesthetic dermatology. PG - 152-6 LID - 10.12788/j.sder.0115 [doi] AB - Botulinum toxins are among the most widely studied and versatile drugs in the medicinal market. Since their extraction from Clostridium botulinum, they have been harnessed and incorporated into different formulations with varied properties and actions. These products have been used to treat countless disorders such as musculoskeletal disorders, headaches, and eye disorders, among many others. In the realm of aesthetic cutaneous medicine, the evolution and creativity in the use of botulinum toxins has been swift and ever changing. Knowledge of the science and innovation behind this toxin enables the user to provide the patient with a variety of treatment options founded in evidence-based medicine. This review will highlight the properties and actions of the newer, more recent neurotoxin preparations, as well as some of the latest and novel therapeutic applications of botulinum toxins. FAU - Ibrahim, Omer AU - Ibrahim O AD - Cleveland Clinic, 9500 Euclic Ave, A61, Cleveland, Ohio 44114, USA. ibrahio@ccf.org. FAU - Keller, Emily C AU - Keller EC AD - SkinCare Physicians, Chestnut Hill, Massachusetts, USA. FAU - Arndt, Kenneth A AU - Arndt KA AD - SkinCare Physicians, Chestnut Hill, Massachusetts, USA. LA - eng PT - Journal Article PT - Review PL - United States TA - Semin Cutan Med Surg JT - Seminars in cutaneous medicine and surgery JID - 9617260 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - EC 3.4.24.69 (incobotulinumtoxinA) SB - IM MH - Administration, Topical MH - Botulinum Toxins, Type A/therapeutic use MH - *Cosmetic Techniques MH - Hot Flashes/drug therapy MH - Humans MH - Injections MH - Keloid/drug therapy MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/drug therapy MH - Skin Aging/*drug effects OTO - NOTNLM OT - RT001 OT - aesthetic dermatology OT - botulinum toxin OT - cosmetic dermatology OT - incobotulinumtoxinA OT - onabotulinumtoxinA EDAT- 2015/04/02 06:00 MHDA- 2016/07/09 06:00 CRDT- 2015/04/02 06:00 PHST- 2015/04/02 06:00 [entrez] PHST- 2015/04/02 06:00 [pubmed] PHST- 2016/07/09 06:00 [medline] AID - 10.12788/j.sder.0115 [doi] PST - ppublish SO - Semin Cutan Med Surg. 2014 Dec;33(4):152-6. doi: 10.12788/j.sder.0115. PMID- 17388920 OWN - NLM STAT- MEDLINE DCOM- 20070905 LR - 20191210 IS - 0007-0963 (Print) IS - 0007-0963 (Linking) VI - 156 IP - 5 DP - 2007 May TI - Assessment of nailfold capillaroscopy by x 30 digital epiluminescence (dermoscopy) in patients with Raynaud phenomenon. PG - 892-8 AB - BACKGROUND: Dermoscopy is a useful tool for dermatologists to study melanocytic lesions. Its possible usefulness in the assessment of capillary nailfold morphological changes (capillaroscopy) has recently been advocated. OBJECTIVES: To assess the practical utility of digital epiluminescence microscopy as a capillaroscopic instrument in patients with Raynaud phenomenon (RP). To compare the sensitivity and specificity rates obtained by epiluminescence microscopy with those previously reported with conventional capillaroscopic devices. METHODS: Fifty-six consecutive patients with primary RP (PRP; n = 5) or secondary RP (SRP; n = 51) (11 men and 45 women in total) were included in the study. A control group of 10 healthy subjects was also evaluated. Twenty-six patients (46%) had systemic sclerosis (SS), 12 (21%) presystemic sclerosis (pre-SS), one (2%) dermatopolymyositis-SS, one (2%) mixed connective tissue disease, two (4%) Sjögren syndrome, two (4%) an overlap syndrome, one (2%) rheumatoid arthritis and six (11%) other connective tissue diseases. Capillary nailfold changes were studied using a nonportable digital epiluminescence device (magnification x 30). Following a systematized protocol, capillary nailfold morphology, density and distribution were evaluated. Several capillaroscopic patterns were identified (normal, sclerodermic, nonspecific, nondiagnostic) as previously defined. A possible relationship between capillary nailfold changes and the intensity of RP or the presence of associated autoimmune diseases was assessed. RESULTS: The sclerodermic pattern showed a sensitivity of 76.9% and a specificity of 90.9% in SS. A typical capillaroscopic SS pattern was observed in 73% of cases of limited SS and in 82% of cases of diffuse SS. Patients with Sjögren syndrome and dermatopolymyositis-SS showed a nonspecific capillaroscopic pattern. All patients with PRP presented a normal capillaroscopic pattern. A normal capillaroscopic pattern was also observed in 11 of 12 patients with pre-SS. In one of two patients presenting severe sclerodactyly and in all patients showing hand oedema (three of 56), capillaroscopic changes could not be evaluated. Avascular areas correlated significantly with severe RP (P < 0.002), bone resorption (P < 0.007) and diffuse SS (P < 0.008). CONCLUSIONS: Digital epiluminescence seems to be a useful and reliable technique in the evaluation of capillary nailfold morphological changes. This technical variation allows the identification of specific capillaroscopic patterns associated with connective tissue diseases. It also permits us to differentiate PRP from SRP. The results obtained with this technique are similar to those previously reported using standard capillaroscopy devices. FAU - Beltrán, E AU - Beltrán E AD - Department of Rheumatology, Hospital del Mar, Passeig Maritim 25-29, Barcelona, Spain. FAU - Toll, A AU - Toll A FAU - Pros, A AU - Pros A FAU - Carbonell, J AU - Carbonell J FAU - Pujol, R M AU - Pujol RM LA - eng PT - Evaluation Study PT - Journal Article DEP - 20070328 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 SB - IM MH - Capillaries/pathology MH - Case-Control Studies MH - Dermoscopy/*methods MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/*blood supply MH - Prospective Studies MH - Raynaud Disease/*diagnosis MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Spain EDAT- 2007/03/29 09:00 MHDA- 2007/09/06 09:00 CRDT- 2007/03/29 09:00 PHST- 2007/03/29 09:00 [pubmed] PHST- 2007/09/06 09:00 [medline] PHST- 2007/03/29 09:00 [entrez] AID - BJD7819 [pii] AID - 10.1111/j.1365-2133.2007.07819.x [doi] PST - ppublish SO - Br J Dermatol. 2007 May;156(5):892-8. doi: 10.1111/j.1365-2133.2007.07819.x. Epub 2007 Mar 28. PMID- 25425218 OWN - NLM STAT- MEDLINE DCOM- 20150819 LR - 20200930 IS - 1552-4833 (Electronic) IS - 1552-4825 (Linking) VI - 167A IP - 1 DP - 2015 Jan TI - Porencephaly in a fetus and HANAC in her father: variable expression of COL4A1 mutation. PG - 156-8 LID - 10.1002/ajmg.a.36823 [doi] AB - COL4A1-associated disorders encompass a wide range of hereditary vasculopathy, including porencephaly and HANAC (adult-onset hemorrhagic stroke with cerebral aneurysm and retinal arterial tortuosity, renal cysts, and thenar muscle cramp). It remains elusive whether or not porencephaly and HANAC are molecularly distinctive disorders due to different classes of mutations. We report on a girl with porencephaly and an episode of microangiopathic hemolysis in infancy and her father with HANAC, both of whom had a heterozygous missense mutation of COL4A1 (c.3715G>A, p.G1239R). The current observation implies phenotypic diversities of COL4A1 mutations. CI - © 2014 Wiley Periodicals, Inc. FAU - Takenouchi, Toshiki AU - Takenouchi T AD - Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan; Center for Medical Genetics, Keio University School of Medicine, Tokyo, Japan. FAU - Ohyagi, Masaki AU - Ohyagi M FAU - Torii, Chiharu AU - Torii C FAU - Kosaki, Rika AU - Kosaki R FAU - Takahashi, Takao AU - Takahashi T FAU - Kosaki, Kenjiro AU - Kosaki K LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141125 PL - United States TA - Am J Med Genet A JT - American journal of medical genetics. Part A JID - 101235741 RN - 0 (COL4A1 protein, human) RN - 0 (Collagen Type IV) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Adult MH - Base Sequence MH - Collagen Type IV/*genetics MH - *Fathers MH - Female MH - Fetus/*abnormalities MH - Humans MH - Infant MH - Infant, Newborn MH - Magnetic Resonance Imaging MH - Male MH - Molecular Sequence Data MH - Muscle Cramp/*genetics MH - Mutation/*genetics MH - Pedigree MH - Porencephaly/*genetics MH - Pregnancy MH - Raynaud Disease/*genetics MH - Young Adult OTO - NOTNLM OT - COL4A1 OT - HANAC OT - Porencephaly EDAT- 2014/11/27 06:00 MHDA- 2015/08/20 06:00 CRDT- 2014/11/27 06:00 PHST- 2014/01/14 00:00 [received] PHST- 2014/09/18 00:00 [accepted] PHST- 2014/11/27 06:00 [entrez] PHST- 2014/11/27 06:00 [pubmed] PHST- 2015/08/20 06:00 [medline] AID - 10.1002/ajmg.a.36823 [doi] PST - ppublish SO - Am J Med Genet A. 2015 Jan;167A(1):156-8. doi: 10.1002/ajmg.a.36823. Epub 2014 Nov 25. PMID- 21956352 OWN - NLM STAT- MEDLINE DCOM- 20120207 LR - 20131121 IS - 0026-4970 (Print) IS - 0026-4970 (Linking) VI - 60 IP - 9 DP - 2011 Sep TI - Scleroderma and CREST syndrome: a case report in dentistry. PG - 443-65 AB - CREST syndrome is part of the heterogeneous scleroderma group of autoimmune diseases that cause thickening, hardening and tightening of the connective tissue in different parts of the body, and it may lead to complex disorders. CREST syndrome is characterized by the coexistence of calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactily and telangectasia. A 72-year-old caucasian woman is referred to the S. Gerardo Hospital of Monza, with a chief complaint of oral pain and difficulties in deglutition and eating, associated with denture instability and difficulties to fit it. She had been previously diagnosed with Raynaud's phenomenon, and afterwards with CREST syndrome. Extra-oral examination underlined taut, thickened and rigid skin, pallid-red irregular maculae all over the face, telangiectasias and acrocyanosis. Intra-oral examination showed no alteration of the mucosa, but we can observe tongue rigidity and some speckled red alternating with white spots on the hard palate and in the vestibule. We undermitted the patient the dental treatment of Sjogren's syndrome. The management of the Sjogren's syndrome is symptomatic and empirical, and involves the use of saliva secretion stimulators, salivary substitutes and coadjuvants. Dental treatment and prophylaxis are important to prevent the consequences of xerostomia, such as rampant caries, based on the administration of topical fluoride in toothpastes and rinses, and supplemented by fluoride gels and varnishes. Instruction and reinforcement of oral hygiene, along with frequent dental assessment and management by the dentist are essential measures to preserve the oral health of those affected with CREST syndrome in progression to SS, complicated with Sjogren's syndrome. FAU - Lauritano, D AU - Lauritano D AD - Dental Clinic Neuroscience, Milano-Bicocca University, Monza, Monza e Brianza, Italy. dorina.lauritano@unimib.it FAU - Bussolati, A AU - Bussolati A FAU - Baldoni, M AU - Baldoni M FAU - Leonida, A AU - Leonida A LA - eng LA - ita PT - Case Reports PT - Journal Article PT - Review PL - Italy TA - Minerva Stomatol JT - Minerva stomatologica JID - 0421071 RN - 0 (Immunosuppressive Agents) RN - 0 (Ophthalmic Solutions) RN - 0 (Saliva, Artificial) RN - 01MI4Q9DI3 (Pilocarpine) SB - IM MH - Aged MH - CREST Syndrome/*complications/pathology MH - Combined Modality Therapy MH - Deglutition Disorders/etiology MH - Dental Caries/prevention & control MH - Dentures MH - Disease Progression MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Ophthalmic Solutions/therapeutic use MH - Oral Hygiene MH - Palate/pathology MH - Pilocarpine/therapeutic use MH - Saliva, Artificial/therapeutic use MH - Scleroderma, Diffuse/classification/epidemiology MH - Sjogren's Syndrome/classification/*complications/drug therapy MH - Skin/pathology MH - Tongue/pathology MH - Viscera/pathology MH - Xerostomia/drug therapy/etiology EDAT- 2011/10/01 06:00 MHDA- 2012/02/09 06:00 CRDT- 2011/09/30 06:00 PHST- 2011/09/30 06:00 [entrez] PHST- 2011/10/01 06:00 [pubmed] PHST- 2012/02/09 06:00 [medline] AID - R18113359 [pii] PST - ppublish SO - Minerva Stomatol. 2011 Sep;60(9):443-65. PMID- 23914890 OWN - NLM STAT- MEDLINE DCOM- 20140407 LR - 20130806 IS - 1529-8019 (Electronic) IS - 1396-0296 (Linking) VI - 26 IP - 4 DP - 2013 Jul-Aug TI - Dermatoses of the breast in lactation. PG - 331-6 LID - 10.1111/dth.12071 [doi] AB - Dermatoses of the breast during lactation can be difficult to diagnose because of their overlapping clinical appearances. It is important to properly diagnose and treat nipple dermatitis since it can be a significant source of pain when nursing. Poorly controlled nipple pain in nursing mothers is one of the primary reasons for breastfeeding to be discontinued earlier than is recommended. Therefore, it is relevant for practicing dermatologists to be aware of certain facts in a patient's history, specific physical exam findings, and the most appropriate laboratory tests used to diagnose these conditions. In addition, the therapeutic approach should be effective and safe for the mother and infant. This review article provides dermatologists with a detailed discussion on the clinical features and management of various breast dermatoses seen in lactation, including atopic dermatitis, irritant contact dermatitis, allergic contact dermatitis, psoriasis, bacterial infections, yeast infections and herpes simplex virus infections. CI - © 2013 Wiley Periodicals, Inc. FAU - Barrett, Meagan E AU - Barrett ME AD - Keck School of Medicine, University of Southern California, Los Angeles, California, USA. FAU - Heller, Misha M AU - Heller MM FAU - Fullerton Stone, Honor AU - Fullerton Stone H FAU - Murase, Jenny E AU - Murase JE LA - eng PT - Journal Article PT - Review PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 SB - IM MH - Breast Diseases/*therapy MH - Dermatitis, Allergic Contact/therapy MH - Dermatitis, Atopic/therapy MH - Eczema/therapy MH - Female MH - Humans MH - *Lactation MH - Nipples MH - Pregnancy MH - Psoriasis/therapy MH - Raynaud Disease/therapy MH - Skin Diseases/*therapy OTO - NOTNLM OT - Candida albicans OT - Staphylococccus aureus OT - lactation OT - mastitis OT - nipple dermatitis EDAT- 2013/08/07 06:00 MHDA- 2014/04/08 06:00 CRDT- 2013/08/07 06:00 PHST- 2013/08/07 06:00 [entrez] PHST- 2013/08/07 06:00 [pubmed] PHST- 2014/04/08 06:00 [medline] AID - 10.1111/dth.12071 [doi] PST - ppublish SO - Dermatol Ther. 2013 Jul-Aug;26(4):331-6. doi: 10.1111/dth.12071. PMID- 22158453 OWN - NLM STAT- MEDLINE DCOM- 20121113 LR - 20260128 IS - 1827-6806 (Print) IS - 1827-6806 (Linking) VI - 12 IP - 12 DP - 2011 Dec TI - [Nitrates at high altitude, that is the inappropriate use of a drug]. PG - 824-8 LID - 10.1714/996.10827 [doi] FAU - Donegani, Enrico AU - Donegani E AD - A.S.O. Maggiore della Carita, Novara. donegani@hotmail.com LA - ita PT - Biography PT - Case Reports PT - Historical Article PT - Journal Article PT - Portrait TT - Nitrati in alta quota, ovvero l'uso inappropriato di un farmaco. PL - Italy TA - G Ital Cardiol (Rome) JT - Giornale italiano di cardiologia (2006) JID - 101263411 RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM CIN - G Ital Cardiol (Rome). 2012 Apr;13(4):306-7. doi: 10.1714/1056.11564. PMID: 22495649 MH - Administration, Cutaneous MH - Altitude Sickness/*chemically induced/etiology/physiopathology/prevention & control MH - Angina Pectoris/drug therapy/prevention & control MH - Brain Edema/*chemically induced/diagnosis/etiology/physiopathology MH - Disorders of Excessive Somnolence/chemically induced MH - Expeditions MH - Frostbite/prevention & control MH - History, 19th Century MH - Humans MH - Hypoxia/etiology/prevention & control MH - Italy/ethnology MH - Male MH - Middle Aged MH - Mountaineering/*physiology MH - Nitroglycerin/administration & dosage/*adverse effects/history/therapeutic use MH - Pakistan MH - Raynaud Disease/drug therapy MH - Transdermal Patch MH - Vasodilator Agents/administration & dosage/*adverse effects/history/therapeutic use PS - Sobrero A FPS - Sobrero, Ascanio EDAT- 2011/12/14 06:00 MHDA- 2012/11/14 06:00 CRDT- 2011/12/14 06:00 PHST- 2011/12/14 06:00 [entrez] PHST- 2011/12/14 06:00 [pubmed] PHST- 2012/11/14 06:00 [medline] AID - 10.1714/996.10827 [doi] PST - ppublish SO - G Ital Cardiol (Rome). 2011 Dec;12(12):824-8. doi: 10.1714/996.10827. PMID- 36535538 OWN - NLM STAT- MEDLINE DCOM- 20230320 LR - 20230324 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 365 IP - 4 DP - 2023 Apr TI - PACK syndrome: A case series and review. PG - 321-328 LID - S0002-9629(22)00489-X [pii] LID - 10.1016/j.amjms.2022.12.005 [doi] AB - A rare overlap syndrome between CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and primary biliary cholangitis (PBC) is described as PACK syndrome, an acronym for primary biliary cholangitis, anticentromere antibodies, CREST syndrome, and keratoconjunctivitis sicca. In this retrospective cohort analysis and review, we present fourteen patients who meet diagnostic criteria for PACK syndrome in one of the largest case series of this group. All patients were female, 86% of whom were White with an average age of 66.7 years (range 39-78 years). The prevalence was 5.08% in our PBC cohort (n=256) similar to previous findings. CREST syndrome was diagnosed prior to PBC in 58% of our patients and limited pulmonary and renal involvement were observed. This syndrome is rare, but given its insidious development, clinicians should be aware of this potential overlap in CREST-only and PBC-only patients. CI - Copyright © 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved. FAU - Collins, B AU - Collins B AD - Department of Medicine, Medical University of South Carolina, Charleston, SC, USA; Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, USA. Electronic address: collibra@musc.edu. FAU - Dillon, D AU - Dillon D AD - Department of Medicine, Medical University of South Carolina, Charleston, SC, USA; Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, USA. FAU - Silver, R M AU - Silver RM AD - Department of Medicine, Medical University of South Carolina, Charleston, SC, USA; Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, USA. LA - eng PT - Journal Article PT - Review DEP - 20221216 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Humans MH - Female MH - Adult MH - Middle Aged MH - Aged MH - Male MH - *CREST Syndrome MH - *Liver Cirrhosis, Biliary MH - Retrospective Studies MH - *Telangiectasis MH - *Raynaud Disease OTO - NOTNLM OT - CREST syndrome OT - PACK syndrome OT - PBC COIS- Declaration of Conflicting Interest The Authors declare that there is no conflict of interest. EDAT- 2022/12/20 06:00 MHDA- 2023/03/21 06:00 CRDT- 2022/12/19 19:25 PHST- 2022/01/27 00:00 [received] PHST- 2022/08/29 00:00 [revised] PHST- 2022/12/10 00:00 [accepted] PHST- 2022/12/20 06:00 [pubmed] PHST- 2023/03/21 06:00 [medline] PHST- 2022/12/19 19:25 [entrez] AID - S0002-9629(22)00489-X [pii] AID - 10.1016/j.amjms.2022.12.005 [doi] PST - ppublish SO - Am J Med Sci. 2023 Apr;365(4):321-328. doi: 10.1016/j.amjms.2022.12.005. Epub 2022 Dec 16. PMID- 20201267 OWN - NLM STAT- MEDLINE DCOM- 20100416 LR - 20110113 IS - 0048-7848 (Print) IS - 0048-7848 (Linking) VI - 112 IP - 3 DP - 2008 Jul-Sep TI - [New trends for the occupational health in accordance with the European program]. PG - 769-74 AB - The development of the European schedule of the occupational diseases has the aim to harmonize the data from all Member States. For this purpose European Agency for Safety and Health at work recommends for all the Member States to progressively make their statistics concerning occupational diseases, compatible with European schedule, in order to evaluate the incidence of the recognised occupational diseases and to define quantified criteria for the recognising each occupational disease. The presented data allude to the incidence of the recognised occupational diseases for the first time in 2001, supposed reference year, and to the deaths due to occupational diseases. The used indicators were the number and incidence rate of occupational diseases by sex, age, economic activity, occupation and causative agents, workplaces, exposed professions. FAU - Teslariu, Elena AU - Teslariu E AD - Facultatea de Medicină Disciplina Medicina Muncii, Universitatea de Medicină si Farmacie "Gr. T. Popa" Iaşi. FAU - Constantin, Brânduşa AU - Constantin B FAU - Mihalache, Cornelia AU - Mihalache C LA - rum PT - English Abstract PT - Journal Article TT - Noi orientări privind bolile profesionale in concordanţă cu programul European. PL - Romania TA - Rev Med Chir Soc Med Nat Iasi JT - Revista medico-chirurgicala a Societatii de Medici si Naturalisti din Iasi JID - 0413735 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Asbestosis/epidemiology MH - Asthma/epidemiology MH - Dermatitis, Occupational/epidemiology MH - European Union MH - Female MH - Government Agencies MH - Hearing Loss/epidemiology MH - Humans MH - Incidence MH - Male MH - Mesothelioma/epidemiology MH - Middle Aged MH - Musculoskeletal Diseases/epidemiology MH - Occupational Diseases/*epidemiology/etiology/mortality/prevention & control MH - Occupational Exposure/*adverse effects/legislation & jurisprudence MH - *Occupational Health/legislation & jurisprudence MH - Pleural Neoplasms/epidemiology MH - Policy Making MH - Raynaud Disease/epidemiology MH - Romania/epidemiology EDAT- 2008/07/01 00:00 MHDA- 2010/04/17 06:00 CRDT- 2010/03/06 06:00 PHST- 2010/03/06 06:00 [entrez] PHST- 2008/07/01 00:00 [pubmed] PHST- 2010/04/17 06:00 [medline] PST - ppublish SO - Rev Med Chir Soc Med Nat Iasi. 2008 Jul-Sep;112(3):769-74. PMID- 39420564 OWN - NLM STAT- MEDLINE DCOM- 20241231 LR - 20241231 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 77 IP - 1 DP - 2025 Jan TI - Impact of Season, Environmental Temperature, and Humidity on Raynaud Phenomenon in an Australian Systemic Sclerosis Cohort. PG - 61-68 LID - 10.1002/acr.25452 [doi] AB - OBJECTIVE: The aim of this study was to determine the impact of season, temperature and humidity on the severity of Raynaud phenomenon (RP) in systemic sclerosis. METHODS: Data from the Australian Scleroderma Cohort Study were used to assess associations of patient-reported worsened RP in the month preceding each study visit. Mean monthly weather data were obtained from the closest weather station to the patient's address. We evaluated the relationship between worsened RP and health-related quality of life (HRQoL) measured using the Short Form 36 instrument. RESULTS: Among 1,972 patients with systemic sclerosis, RP was a near-universal finding, and worsened RP in the preceding month was reported in 26.7% of 9,175 visits. "Worsened RP" showed significant environmental variability. On multivariable analysis, worsened RP was associated with low mean maximum temperatures (odds ratio [OR] 0.91, 95% confidence interval [95% CI] 0.90-0.92, P < 0.001), high relative humidity (OR 1.05, 95% CI 1.04-1.05, P < 0.001) and lower mean daily evaporation (OR 0.77, 95% CI 0.73-0.81, P < 0.001). Worsened RP was strongly associated with telangiectasia, calcinosis, and digital ulceration, as well as demonstrating an association with anticentromere antibody and gastroesophageal reflux disease and a negative correlation with diffuse disease. Worsened RP was also strongly associated with worse HRQoL. CONCLUSION: Lower environmental temperature and higher relative humidity had significant associations with worsened RP in this systemic sclerosis cohort, suggesting an important role for dry warmth in managing this condition. CI - © 2024 American College of Rheumatology. FAU - Taylor, Laura AU - Taylor L AUID- ORCID: 0000-0002-4282-5356 AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Hansen, Dylan AU - Hansen D AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Morrisroe, Kathleen AU - Morrisroe K AUID- ORCID: 0000-0003-3840-3967 AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Fairley, Jessica AU - Fairley J AUID- ORCID: 0000-0003-4140-2711 AD - St. Vincent's Hospital Melbourne and The University of Melbourne, Melbourne, Victoria, Australia. FAU - Calderone, Alicia AU - Calderone A AUID- ORCID: 0000-0001-8925-6771 AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Oon, Shereen AU - Oon S AUID- ORCID: 0000-0002-6822-5711 AD - St. Vincent's Hospital Melbourne and The University of Melbourne, Melbourne, Victoria, Australia. FAU - Ross, Laura AU - Ross L AUID- ORCID: 0000-0003-4636-729X AD - St. Vincent's Hospital Melbourne and The University of Melbourne, Melbourne, Victoria, Australia. FAU - Stevens, Wendy AU - Stevens W AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Ferdowsi, Nava AU - Ferdowsi N AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Quinlivan, Alannah AU - Quinlivan A AUID- ORCID: 0000-0002-7750-507X AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Sahhar, Joanne AU - Sahhar J AD - Monash Health and Monash University, Melbourne, Victoria, Australia. FAU - Ngian, Gene-Siew AU - Ngian GS AD - Monash Health and Monash University, Melbourne, Victoria, Australia. FAU - Apostolopoulos, Diane AU - Apostolopoulos D AD - Monash Health and Monash University, Melbourne, Victoria, Australia. FAU - Host, Lauren V AU - Host LV AD - Fiona Stanley Hospital, Perth, Western Australia, Australia. FAU - Walker, Jennifer AU - Walker J AD - Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - Tabesh, Maryam AU - Tabesh M AD - St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Proudman, Susanna AU - Proudman S AUID- ORCID: 0000-0002-3046-9884 AD - Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia. FAU - Nikpour, Mandana AU - Nikpour M AD - The University of Sydney and Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia. LA - eng GR - Musculoskeletal Australia/ GR - Australian Rheumatology Association/ GR - Research Endowment Fund/St Vincent's Hospital Melbourne/ GR - GNT1176538/National Health and Medical Research Council/ GR - GNT2013842/National Health and Medical Research Council/ GR - GNT1197169/National Health and Medical Research Council/ GR - Boehringer Ingelheim/ GR - Scleroderma Australia/ GR - Scleroderma Victoria/ GR - Arthritis Australia/ GR - Janssen Pharmaceutica/ PT - Journal Article DEP - 20241110 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Humans MH - *Raynaud Disease/epidemiology MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology/physiopathology MH - *Humidity MH - Female MH - Male MH - Middle Aged MH - Australia/epidemiology MH - *Seasons MH - *Temperature MH - Aged MH - Adult MH - Quality of Life MH - Cohort Studies MH - Severity of Illness Index EDAT- 2024/10/18 06:23 MHDA- 2024/12/31 12:21 CRDT- 2024/10/18 01:43 PHST- 2024/10/07 00:00 [revised] PHST- 2024/05/31 00:00 [received] PHST- 2024/10/08 00:00 [accepted] PHST- 2024/12/31 12:21 [medline] PHST- 2024/10/18 06:23 [pubmed] PHST- 2024/10/18 01:43 [entrez] AID - 10.1002/acr.25452 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2025 Jan;77(1):61-68. doi: 10.1002/acr.25452. Epub 2024 Nov 10. PMID- 25584528 OWN - NLM STAT- MEDLINE DCOM- 20160119 LR - 20220318 IS - 1540-7330 (Electronic) IS - 1085-2352 (Print) IS - 1085-2352 (Linking) VI - 43 IP - 1 DP - 2015 TI - An investigation of symptoms predating CFS onset. PG - 54-61 LID - 10.1080/10852352.2014.973240 [doi] AB - The Fukuda et al. (1994) criteria for chronic fatigue syndrome (CFS) specifies that a symptom can only be included within a diagnosis if it is experienced concurrently or following the onset of fatigue. In order to investigate this issue, participants provided information on persisting symptoms (lasting greater than six months) and whether those symptoms occurred prior to, concurrently, or following the onset of their fatigue. More symptoms were experienced after the fatigue onset than prior to the fatigue onset; however, a considerable number of participants reported experiencing persisting symptoms prior to the onset of CFS. Particularly, rates of hay fever and asthma were higher prior to the illness. Investigating symptoms prior to the onset of the illness might provide investigators with ways to better understand the etiology of this illness. FAU - Evans, Meredyth AU - Evans M AD - a Center for Community Research, DePaul University , Chicago , Illinois , USA. FAU - Barry, Morgan AU - Barry M FAU - Im, Young AU - Im Y FAU - Brown, Abigail AU - Brown A FAU - Jason, Leonard A AU - Jason LA LA - eng GR - AI055735/AI/NIAID NIH HHS/United States GR - R01 AI055735/AI/NIAID NIH HHS/United States GR - R01 AI049720/AI/NIAID NIH HHS/United States GR - R01 AI105781/AI/NIAID NIH HHS/United States GR - AI 49720/AI/NIAID NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - J Prev Interv Community JT - Journal of prevention & intervention in the community JID - 9702085 SB - IM MH - Adult MH - Fatigue/*physiopathology MH - Fatigue Syndrome, Chronic/*diagnosis/*physiopathology MH - Female MH - Health Status MH - Humans MH - Hypersensitivity/physiopathology MH - Male MH - Mental Health MH - Middle Aged MH - Raynaud Disease/physiopathology MH - Rhinitis, Allergic, Seasonal/physiopathology MH - Severity of Illness Index MH - Socioeconomic Factors PMC - PMC4830334 MID - NIHMS775178 OTO - NOTNLM OT - Myalgic Encephalomyelitis OT - Myalgic Encephalomyelitis/chronic fatigue syndrome OT - Predating Symptoms OT - chronic fatigue syndrome EDAT- 2015/01/15 06:00 MHDA- 2016/01/20 06:00 PMCR- 2016/04/13 CRDT- 2015/01/14 06:00 PHST- 2015/01/14 06:00 [entrez] PHST- 2015/01/15 06:00 [pubmed] PHST- 2016/01/20 06:00 [medline] PHST- 2016/04/13 00:00 [pmc-release] AID - 10.1080/10852352.2014.973240 [doi] PST - ppublish SO - J Prev Interv Community. 2015;43(1):54-61. doi: 10.1080/10852352.2014.973240. PMID- 17039313 OWN - NLM STAT- MEDLINE DCOM- 20070117 LR - 20161020 IS - 1439-7595 (Print) IS - 1439-7595 (Linking) VI - 16 IP - 5 DP - 2006 TI - Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis. PG - 309-11 AB - We have encountered a 68-year-old Japanese woman with limited cutaneous systemic sclerosis who developed de novo onset of accelerated hypertension and renal dysfunction; thus we diagnosed scleroderma renal crisis. Anticentromere antibody alone was identified, and not anti-DNA topoisomerase I antibody, anti-RNA polymerase antibodies, anti-Th/To antibodies, or antiribonucleoprotein antibodies, even with use of immunoprecipitation assay. She was successfully treated with angiotensin-converting enzyme inhibitor. This case, scleroderma renal crisis with detection of anticentromere antibody, is thought to be extremely uncommon. FAU - Sugimoto, Toshiro AU - Sugimoto T AD - Department of Medicine, Shiga University of Medical Science, Seta, Otsu, 520-2192, Japan. toshiro@belle.shiga-med.ac.jp FAU - Soumura, Mariko AU - Soumura M FAU - Danno, Kiichiro AU - Danno K FAU - Kaji, Kenzo AU - Kaji K FAU - Kondo, Miki AU - Kondo M FAU - Hirata, Kunio AU - Hirata K FAU - Nakazawa, Jun AU - Nakazawa J FAU - Uzu, Takashi AU - Uzu T FAU - Nishio, Yoshihiko AU - Nishio Y FAU - Kashiwagi, Atsunori AU - Kashiwagi A LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) SB - IM MH - Aged MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Autoantibodies/*blood MH - Autoantigens/immunology MH - Centromere/*immunology MH - Esophagitis, Peptic/complications MH - Female MH - Humans MH - Hypertension/etiology MH - Kidney Diseases/*etiology MH - Liver Cirrhosis, Biliary/complications MH - Pruritus/complications MH - Raynaud Disease/complications MH - Scleroderma, Limited/*complications/immunology/*physiopathology EDAT- 2006/10/14 09:00 MHDA- 2007/01/18 09:00 CRDT- 2006/10/14 09:00 PHST- 2006/04/27 00:00 [received] PHST- 2006/06/15 00:00 [accepted] PHST- 2006/10/14 09:00 [pubmed] PHST- 2007/01/18 09:00 [medline] PHST- 2006/10/14 09:00 [entrez] AID - 10.1007/s10165-006-0504-4 [doi] PST - ppublish SO - Mod Rheumatol. 2006;16(5):309-11. doi: 10.1007/s10165-006-0504-4. PMID- 26607041 OWN - NLM STAT- MEDLINE DCOM- 20160815 LR - 20181113 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 9 DP - 2015 Nov 25 TI - Primary sclerosing cholangitis associated with CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) in an elderly woman: a case report. PG - 272 LID - 10.1186/s13256-015-0747-9 [doi] LID - 272 AB - INTRODUCTION: CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia) syndrome comprising calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia and primary sclerosing cholangitis are both chronic fibrotic diseases but the association between them is extremely rare. While primary sclerosing cholangitis has been associated with diffuse cutaneous scleroderma, the association with limited cutaneous scleroderma or CREST has not been previously reported in the literature. This case report illustrates the association between CREST and primary sclerosing cholangitis. CASE PRESENTATION: We report the case of an 84-year-old Asian woman with a long history of CREST who was admitted with abdominal pain, fatigue and progressive derangement of her liver enzymes. This was initially thought to be secondary to her bosentan therapy for pulmonary hypertension but it persisted despite bosentan being ceased. Primary sclerosing cholangitis was subsequently diagnosed on magnetic resonance cholangiopancreatography and she was referred to a hepatologist for treatment. CONCLUSIONS: This case highlights the need to consider primary sclerosing cholangitis in patients with CREST who present with abdominal symptoms and deranged liver enzymes when other causes have been excluded. Relevant differential diagnoses for this presentation, which can be difficult to exclude, include immunoglobulin G4-associated cholangitis and antimitochondrial antibody negative primary biliary cirrhosis. It is of particular significance to rheumatologists and gastroenterologists but has broader relevance to all medical specialists involved in the care of patients with CREST. FAU - Powell, Alice AU - Powell A AD - The Queen Elizabeth Hospital, Adelaide, SA, Australia. alice.c.powell@gmail.com. FAU - McNeil, Julian AU - McNeil J AD - University Department of Medicine, Modbury Hospital, Adelaide, SA, Australia. julian.mcneil@adelaide.edu.au. LA - eng PT - Case Reports PT - Journal Article DEP - 20151125 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 SB - IM MH - Abdominal Pain/*etiology MH - Aged, 80 and over MH - Calcinosis MH - *Cholangiopancreatography, Magnetic Resonance MH - Cholangitis, Sclerosing/complications/*diagnosis/pathology MH - Esophageal Motility Disorders MH - Fatigue/*etiology MH - Female MH - Humans MH - Raynaud Disease MH - Referral and Consultation MH - Telangiectasis PMC - PMC4660807 EDAT- 2015/11/27 06:00 MHDA- 2016/08/16 06:00 PMCR- 2015/11/25 CRDT- 2015/11/27 06:00 PHST- 2015/05/03 00:00 [received] PHST- 2015/10/26 00:00 [accepted] PHST- 2015/11/27 06:00 [entrez] PHST- 2015/11/27 06:00 [pubmed] PHST- 2016/08/16 06:00 [medline] PHST- 2015/11/25 00:00 [pmc-release] AID - 10.1186/s13256-015-0747-9 [pii] AID - 747 [pii] AID - 10.1186/s13256-015-0747-9 [doi] PST - epublish SO - J Med Case Rep. 2015 Nov 25;9:272. doi: 10.1186/s13256-015-0747-9. PMID- 23136105 OWN - NLM STAT- MEDLINE DCOM- 20130507 LR - 20121108 IS - 1438-9592 (Electronic) IS - 0044-409X (Linking) VI - 137 IP - 5 DP - 2012 Oct TI - [Immunoadsorption in thrombangiitis obliterans: a promising therapeutic option: results of a consecutive patient cohort treated in clinical routine care]. PG - 460-5 LID - 10.1055/s-0032-1315141 [doi] AB - BACKGROUND: Thrombangiitis obliterans or Buerger's disease is a segmental inflammatory disease affecting small and medium-sized veins and arteries, which most often affects young smokers leading to thrombophlebitis and acral ischaemic syndromes, inducing high amputation rates. Based on positive results of a former pilot study we report on our results of immunoadsorption (IA) in clinical routine care, where IA was offered as a treatment option. PATIENTS AND METHODS: The uncontrolled course of 12 consecutive TAO-patients treated by IA on a series of 5 consecutive days was observed. Follow-up period was 14.1 (ranging from 1-26) months. RESULTS: Eight patients were treated with one, four patients completed 2 IA-series. In 9 patients an early onset and lasting clinical improvement and an improvement of ischaemia was noted. The intake of pain-relievers (especially opioids) sank drastically. Eight patients returned to work. Retrospectively, in two out of three treatment failures the correct diagnosis of TAO was questionable. CONCLUSION: IA seems to be a promising treatment option for patients suffering from TAO which should be further evaluated in controlled clinical trials. CI - Georg Thieme Verlag KG Stuttgart · New York. FAU - Klein-Weigel, P F AU - Klein-Weigel PF AD - Klinik für Angiologie, HELIOS Klinikum Berlin-Buch, Deutschland. peter.klein-weigel@helios-kliniken.de FAU - Köning, C AU - Köning C FAU - Härtwig, A AU - Härtwig A FAU - Krüger, K AU - Krüger K FAU - Gutsche-Petrak, B AU - Gutsche-Petrak B FAU - Dreusicke, S AU - Dreusicke S FAU - Thieme, U AU - Thieme U FAU - Enke-Melzer, K AU - Enke-Melzer K FAU - Urbach, B AU - Urbach B FAU - Kron, J AU - Kron J LA - ger PT - English Abstract PT - Journal Article TT - Immunadsorption bei Thrombangiitis obliterans - eine vielversprechende therapeutische Option. Behandlungsergebnisse einer konsekutiven Patientenkohorte der klinischen Routineversorgung. DEP - 20121107 PL - Germany TA - Zentralbl Chir JT - Zentralblatt fur Chirurgie JID - 0413645 SB - IM MH - Adult MH - Cohort Studies MH - Female MH - Fingers/blood supply MH - Follow-Up Studies MH - Foot/blood supply MH - Humans MH - *Immunosorbent Techniques MH - Ischemia/etiology/therapy MH - Male MH - Middle Aged MH - Raynaud Disease/therapy MH - Thromboangiitis Obliterans/*therapy MH - Toes/blood supply EDAT- 2012/11/09 06:00 MHDA- 2013/05/08 06:00 CRDT- 2012/11/09 06:00 PHST- 2012/11/09 06:00 [entrez] PHST- 2012/11/09 06:00 [pubmed] PHST- 2013/05/08 06:00 [medline] AID - 10.1055/s-0032-1315141 [doi] PST - ppublish SO - Zentralbl Chir. 2012 Oct;137(5):460-5. doi: 10.1055/s-0032-1315141. Epub 2012 Nov 7. PMID- 32234170 OWN - NLM STAT- MEDLINE DCOM- 20200512 LR - 20200512 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 100 IP - 12 DP - 2020 Mar 31 TI - [Clinical analysis of 12 cases of mixed connective tissue disease-associated trigeminal neuropathy]. PG - 938-941 LID - 10.3760/cma.j.cn112137-20191113-02471 [doi] AB - Objective: The aim of present study is to analyze clinical and laboratory features of mixed connective tissue disease (MCTD)-associated trigeminal neuropathy (TN). Methods: Clinical records of 12 cases of MCTD complicated with TN diagnosed in Peking University People's Hospital from January 2008 to October 2019 were analyzed retrospectively. Results: The present study included 12 cases, 1 males and 11 females, average age was(40±13)years. TN was developed before the diagosis of MCTD in 1 case. TN and MCTD were occurred simutaneously in 1 case. Raynaud phenomenon (12 cases), arthritis (10cases), edema of fingers (9cases), myositis (6 cases), and pulmonary involvement (11cases) were main cinical feature of MCTD-associate TN. Antinuclear antibody (ANA) and high titer anti-U1-RNP antibody could be detected in serum of all patients. Elevated erythrocyte sedimentation rate (ESR) and creatine kinase were found in serum of 7cases and 5cases, respectively. Blink reflex tests were positive in 6 cases. Neurological symptoms improve slowly without any progress by using glucocorticoid combined immunosuppressants or intravenous gamma globulin. Conclusions: TN is often associted with actived MCTD. Positive ANA and anti-U1-RNP antibody were common in MCTD-associated TN. Blink reflex test is essential to diagnose MCTD-associated TN. Intensive treatment of MCTD contributes to control the progress of TN. FAU - Gao, X J AU - Gao XJ AD - Department of Rheumatology, Ningde Hospital, Affiliated Hospital of Fujian Medical University, Ningde 352000, China. FAU - Li, Y H AU - Li YH AD - Department of Rheumatology, People's Hospital of Peking University, Beijing 100044, China. FAU - Zhang, X W AU - Zhang XW AD - Department of Rheumatology, People's Hospital of Peking University, Beijing 100044, China. FAU - Chen, S AU - Chen S AD - Department of Rheumatology, People's Hospital of Peking University, Beijing 100044, China. FAU - Liu, Y Y AU - Liu YY AD - Department of Rheumatology, People's Hospital of Peking University, Beijing 100044, China. LA - chi GR - 81801617/National Natural Science Foundation of China/ GR - 2017YFA0105802/National Key R&D Program of China/ GR - RDY2018-01, RS2018-02, RDKP2019-02, RDE2019-02/Research and Development Fund of Peking University People's Hospital/ GR - RDH 2017-02/Peking University People's Hospital Research and Development Funds/ PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Mixed Connective Tissue Disease MH - *Raynaud Disease MH - Retrospective Studies MH - *Trigeminal Nerve Diseases OTO - NOTNLM OT - Blink reflex OT - Mixed connective tissue disease OT - Raynaud phenomenon OT - Trigeminal neuropathy EDAT- 2020/04/03 06:00 MHDA- 2020/05/13 06:00 CRDT- 2020/04/03 06:00 PHST- 2020/04/03 06:00 [entrez] PHST- 2020/04/03 06:00 [pubmed] PHST- 2020/05/13 06:00 [medline] AID - 10.3760/cma.j.cn112137-20191113-02471 [doi] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2020 Mar 31;100(12):938-941. doi: 10.3760/cma.j.cn112137-20191113-02471. PMID- 32007943 OWN - NLM STAT- MEDLINE DCOM- 20210510 LR - 20210510 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 47 IP - 2 DP - 2020 Feb TI - Targetoid-like Lesions in the Setting of Systemic Lupus Erythematosus: A Case of Rowell Syndrome. PG - 298-299 LID - 10.3899/jrheum.181021 [doi] FAU - Ward, Jordan M AU - Ward JM AUID- ORCID: 0000-0003-4417-3141 AD - Resident physician in Internal Medicine Residency Program at the Medical College of Georgia at Augusta University; joward@augusta.edu. FAU - Hess, Jaclyn N AU - Hess JN AUID- ORCID: 0000-0003-4417-3141 AD - Chief Resident in the Department of Dermatology at the Medical College of Georgia at Augusta University. FAU - Davis, Loretta S AU - Davis LS AUID- ORCID: 0000-0003-4417-3141 AD - Chief of the Department of Dermatology and Residency Program Director at the Medical College of Georgia at Augusta University, Augusta, Georgia, USA. LA - eng PT - Case Reports PT - Journal Article PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antirheumatic Agents) RN - 0 (Immunosuppressive Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Antirheumatic Agents/therapeutic use MH - Diagnosis, Differential MH - Erythema Multiforme/*complications/drug therapy MH - Female MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/*complications/drug therapy MH - Methotrexate/therapeutic use MH - Raynaud Disease/*complications MH - Sjogren's Syndrome/*complications/drug therapy MH - Sunlight/adverse effects MH - Syndrome MH - Treatment Outcome EDAT- 2020/02/03 06:00 MHDA- 2021/05/11 06:00 CRDT- 2020/02/03 06:00 PHST- 2020/02/03 06:00 [entrez] PHST- 2020/02/03 06:00 [pubmed] PHST- 2021/05/11 06:00 [medline] AID - 47/2/298 [pii] AID - 10.3899/jrheum.181021 [doi] PST - ppublish SO - J Rheumatol. 2020 Feb;47(2):298-299. doi: 10.3899/jrheum.181021. PMID- 22214803 OWN - NLM STAT- MEDLINE DCOM- 20130617 LR - 20130118 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 70 IP - 2 DP - 2013 Feb TI - Long-term results of arterial sympathectomy and artery reconstruction with vein bypass technique as a salvage procedure for severe digital ischemia. PG - 168-71 LID - 10.1097/SAP.0b013e318234e906 [doi] AB - The aim of this study is to present a combined treatment of arterial sympathectomy and artery reconstruction with vein bypass technique in patients with severe digital ischemia. In all, 22 patients representing 53 digits received treatment during a 15-year period. Skin color and trophic changes including ulcers, necrosis, and amputation; pain level according to the visual analog scale; and functional assessment based on the disabilities of the arm, shoulder, and hand questionnaire were recorded. After surgery, the skin discoloration improved, and the trophic changes healed in the majority of patients. Amputation was required in 2 cases. Pain and function improved significantly postoperatively. Based on the results of this study, vascular reconstruction combined with sympathectomy may be indicated on an individualized basis for patients with digital ischemia. This procedure can lead to healing of skin trophic changes, diminution of pain, avoidance of amputation, and long-term improvement in life quality. FAU - Savvidou, Christiana AU - Savvidou C AD - Christine M. Kleinert Institute for Hand & Microsurgery, Louisville, KY 40202, USA. csavvidou24@gmail.com FAU - Tsai, Tsu-Min AU - Tsai TM LA - eng PT - Journal Article PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 SB - IM MH - Adolescent MH - Adult MH - Arteries/innervation/*surgery MH - Blood Vessel Prosthesis Implantation MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*surgery MH - Male MH - Middle Aged MH - Raynaud Disease/*surgery MH - Retrospective Studies MH - *Sympathectomy MH - Veins/*transplantation MH - Young Adult EDAT- 2012/01/05 06:00 MHDA- 2013/06/19 06:00 CRDT- 2012/01/05 06:00 PHST- 2012/01/05 06:00 [entrez] PHST- 2012/01/05 06:00 [pubmed] PHST- 2013/06/19 06:00 [medline] AID - 10.1097/SAP.0b013e318234e906 [doi] PST - ppublish SO - Ann Plast Surg. 2013 Feb;70(2):168-71. doi: 10.1097/SAP.0b013e318234e906. PMID- 18292730 OWN - NLM STAT- MEDLINE DCOM- 20080325 LR - 20161124 IS - 1341-1098 (Print) IS - 1341-1098 (Linking) VI - 13 IP - 6 DP - 2007 Dec TI - A case of biatrial multiple myxomas with glandular structure. PG - 423-7 AB - A 45-year-old male, who had been indicated by brain magnetic resonance imaging to have cerebral infarctions, was found by echocardiography to have a tumor in the left atrium. He had experienced several of the constitutional disturbances associated with myxoma. At the ages of 19 and 35 he had had two episodes associated with embolisms, and at the later one he was diagnosed as having multiple cerebral aneurysms. He received an urgent operation in which three left atrial tumors and one right atrial tumor were resected. Histologically, the tumors were myxomas, and the left atrial main tumor had glandular structure. In view of his clinical history, this patient seems to have had cardiac myxomas for a long period. The multiple growths that occurred in this case may be a good argument for allowing this condition to last for so long. To our knowledge, the present case was the first report of cardiac myxoma with glandular structure in Japan. FAU - Namura, Osamu AU - Namura O AD - Division of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. FAU - Saitoh, Masayuki AU - Saitoh M FAU - Moro, Hisanaga AU - Moro H FAU - Watanabe, Hiroshi AU - Watanabe H FAU - Sogawa, Masakazu AU - Sogawa M FAU - Nishikura, Ken AU - Nishikura K FAU - Hayashi, Jun-Ichi AU - Hayashi J LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Ann Thorac Cardiovasc Surg JT - Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia JID - 9703158 SB - IM MH - Cerebral Infarction/epidemiology MH - Comorbidity MH - *Heart Atria MH - Heart Neoplasms/diagnostic imaging/epidemiology/*pathology/surgery MH - Humans MH - Intracranial Aneurysm/epidemiology MH - Intracranial Embolism/epidemiology MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Myxoma/diagnostic imaging/epidemiology/*pathology/surgery MH - Raynaud Disease/epidemiology MH - Ultrasonography EDAT- 2008/02/23 09:00 MHDA- 2008/03/26 09:00 CRDT- 2008/02/23 09:00 PHST- 2006/09/21 00:00 [received] PHST- 2007/02/05 00:00 [accepted] PHST- 2008/02/23 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2008/02/23 09:00 [entrez] AID - atcs/2007_13_6/423 [pii] PST - ppublish SO - Ann Thorac Cardiovasc Surg. 2007 Dec;13(6):423-7. PMID- 33563461 OWN - NLM STAT- MEDLINE DCOM- 20210913 LR - 20210913 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 159 IP - 2 DP - 2021 Feb TI - Cement Factory Worker Presenting With Raynaud Phenomenon, Breathlessness, and Digital Ulcers. PG - e93-e96 LID - S0012-3692(20)34484-6 [pii] LID - 10.1016/j.chest.2020.09.076 [doi] AB - A 54-year-old man who had worked in a cement factory for the past 30 years, presented to the chest clinic with complaints of insidious onset, gradually progressive breathlessness with intermittent dry cough of three years' duration. The symptoms were associated with bluish discoloration of fingers on exposure to cold. He also gave a history of digital ulcers at the fingertips of the same duration. These ulcers used to heal, leaving behind pitted scars. There was also an associated history of progressive tightening of skin involving the face, extremities, and trunk. He also complained of food getting stuck in the throat, and he had to take frequent sips of water while eating, along with a feeling of early satiety. There was also a history of skin pruritus. There was no history of arthritis, rash, or alopecia. He had been treated 15 years ago for pulmonary TB, with 9 months of anti-tubercular therapy. He denied any similar illness in the family. On eliciting his occupational history, he revealed that other coworkers in his workspace had complained of a similar illness. He was a nonsmoker and teetotaller with no known addictions or exposure to chemicals. CI - Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. FAU - Marwah, Vikas AU - Marwah V AD - Department of Pulmonary, Critical Care, and Sleep Medicine, Army Institute of Cardiothoracic Sciences, Maharashtra, India. FAU - Katoch, Chandan Dev Singh AU - Katoch CDS AD - Department of Pulmonary, Critical Care, and Sleep Medicine, Army Institute of Cardiothoracic Sciences, Maharashtra, India. FAU - Hegde, Arun AU - Hegde A AD - Department of Rheumatology, Army Institute of Cardiothoracic Sciences, Maharashtra, India. FAU - Choudhary, Robin AU - Choudhary R AD - Department of Pulmonary, Critical Care, and Sleep Medicine, Army Institute of Cardiothoracic Sciences, Maharashtra, India. Electronic address: robinch19@gmail.com. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 SB - IM MH - Diagnosis, Differential MH - Dyspnea MH - Fingers MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*diagnosis/therapy MH - Occupational Exposure/*adverse effects MH - Raynaud Disease MH - Scleroderma, Systemic/*diagnosis/therapy MH - Silicosis/*diagnosis/therapy MH - Syndrome MH - Ulcer EDAT- 2021/02/11 06:00 MHDA- 2021/09/14 06:00 CRDT- 2021/02/10 05:38 PHST- 2020/05/11 00:00 [received] PHST- 2020/08/25 00:00 [revised] PHST- 2020/09/06 00:00 [accepted] PHST- 2021/02/10 05:38 [entrez] PHST- 2021/02/11 06:00 [pubmed] PHST- 2021/09/14 06:00 [medline] AID - S0012-3692(20)34484-6 [pii] AID - 10.1016/j.chest.2020.09.076 [doi] PST - ppublish SO - Chest. 2021 Feb;159(2):e93-e96. doi: 10.1016/j.chest.2020.09.076. PMID- 33871613 OWN - NLM STAT- MEDLINE DCOM- 20211222 LR - 20211222 IS - 2574-3805 (Electronic) IS - 2574-3805 (Linking) VI - 4 IP - 4 DP - 2021 Apr 1 TI - Evaluation of the Safety of Calcitonin Gene-Related Peptide Antagonists for Migraine Treatment Among Adults With Raynaud Phenomenon. PG - e217934 LID - 10.1001/jamanetworkopen.2021.7934 [doi] LID - e217934 AB - IMPORTANCE: Calcitonin gene-related peptide (CGRP) antagonists have demonstrated tremendous promise in migraine management. However, these medications decrease reflex vasodilatory response, which may lead to exacerbation of microvascular disease in susceptible patients, such as patients with Raynaud phenomenon (RP). OBJECTIVE: To investigate the microvascular complications of CGRP antagonists in patients with underlying RP. DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study was performed from May 18, 2018, to September 15, 2020, in Mayo Clinic Health System patients with Raynaud phenomenon while undergoing CGRP antagonist therapy to treat migraine. Inclusion criteria were age older than 18 years, history of migraine, past or current treatment with CGRP antagonists, and diagnosis of primary or secondary RP. EXPOSURE: Treatment with CGRP antagonists. MAIN OUTCOMES AND MEASURES: The main outcome measure was microvascular complications (eg, worsening RP, digital ulcerations, and gangrenous necrosis) after initiation of treatment with a CGRP antagonist. Patient demographic and clinical characteristics were compared between those who experienced complications and those who did not. RESULTS: A total of 169 patients (163 [96.4%] female; 151 [89.3%] non-Hispanic White; mean [SD] age, 46 [13] years) were identified. Of the 169 patients, 9 (5.3%) exhibited microvascular complications, ranging from worsening RP to gangrene and autonecrosis that required distal digit amputation. Comparative analysis did not find statistically significant differences in demographic or clinical characteristics between the 2 cohorts. All 9 patients with complications were female (mean [SD] age, 40 [12] years). Five of the 9 patients (55.6%) had previously diagnosed RP; in 3 the RP was primary, and 2 it was secondary to scleroderma. The other 4 patients (44.4%) were newly diagnosed with RP. Eight of the 9 patients (88.9%) had chronic migraine; 4 had migraine with aura, and 5 had migraine without aura. The CGRP antagonist agents temporally associated with the microvascular complications included galcanezumab (in 3 patients), erenumab (in 5 patients), and fremanezumab (in 1 patient). CONCLUSIONS AND RELEVANCE: The results of this study indicate that microvascular complications of CGRP antagonist use in patients with underlying RP are uncommon. The incidence of serious adverse events, although rare, warrant caution when considering the use of these agents in patients with RP. FAU - Breen, Ilana D AU - Breen ID AD - Department of Dermatology, Mayo Clinic Arizona, Scottsdale. FAU - Brumfiel, Caitlin M AU - Brumfiel CM AD - Department of Dermatology, Mayo Clinic Arizona, Scottsdale. FAU - Patel, Meera H AU - Patel MH AD - Department of Dermatology, Mayo Clinic Arizona, Scottsdale. FAU - Butterfield, Richard J AU - Butterfield RJ AD - Department of Biostatistics, Mayo Clinic Arizona, Scottsdale. FAU - VanderPluym, Juliana H AU - VanderPluym JH AD - Department of Neurology, Mayo Clinic Arizona, Phoenix. FAU - Griffing, Leroy AU - Griffing L AD - Department of Rheumatology, Mayo Clinic Arizona, Phoenix. FAU - Pittelkow, Mark R AU - Pittelkow MR AD - Department of Dermatology, Mayo Clinic Arizona, Scottsdale. FAU - Mangold, Aaron R AU - Mangold AR AD - Department of Dermatology, Mayo Clinic Arizona, Scottsdale. LA - eng PT - Journal Article DEP - 20210401 PL - United States TA - JAMA Netw Open JT - JAMA network open JID - 101729235 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - 0 (fremanezumab) RN - 55KHL3P693 (galcanezumab) RN - I5I8VB78VT (erenumab) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Adult MH - Antibodies, Monoclonal/*adverse effects MH - Antibodies, Monoclonal, Humanized/adverse effects MH - Calcitonin Gene-Related Peptide/antagonists & inhibitors MH - Calcitonin Gene-Related Peptide Receptor Antagonists/*adverse effects MH - *Contraindications, Drug MH - *Drug-Related Side Effects and Adverse Reactions MH - Female MH - Fingers MH - Humans MH - Male MH - Microcirculation MH - Middle Aged MH - Migraine Disorders/*drug therapy MH - Necrosis/etiology MH - Raynaud Disease/*pathology MH - Ulcer/etiology MH - Vascular Diseases/pathology PMC - PMC8056280 COIS- Conflict of Interest Disclosures: Dr VanderPluym reported receiving grants from Amgen and personal fees from Teva outside the submitted work. No other disclosures were reported. EDAT- 2021/04/20 06:00 MHDA- 2021/12/24 06:00 PMCR- 2021/04/19 CRDT- 2021/04/19 12:23 PHST- 2021/04/19 12:23 [entrez] PHST- 2021/04/20 06:00 [pubmed] PHST- 2021/12/24 06:00 [medline] PHST- 2021/04/19 00:00 [pmc-release] AID - 2778841 [pii] AID - zoi210252 [pii] AID - 10.1001/jamanetworkopen.2021.7934 [doi] PST - epublish SO - JAMA Netw Open. 2021 Apr 1;4(4):e217934. doi: 10.1001/jamanetworkopen.2021.7934. PMID- 28240972 OWN - NLM STAT- MEDLINE DCOM- 20170728 LR - 20260127 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 35 IP - 11 DP - 2017 Apr 10 TI - Multi-Institutional Assessment of Adverse Health Outcomes Among North American Testicular Cancer Survivors After Modern Cisplatin-Based Chemotherapy. PG - 1211-1222 LID - 10.1200/JCO.2016.70.3108 [doi] AB - Purpose To provide new information on adverse health outcomes (AHOs) in testicular cancer survivors (TCSs) after four cycles of etoposide and cisplatin (EPX4) or three or four cycles of bleomycin, etoposide, cisplatin (BEPX3/BEPX4). Methods Nine hundred fifty-two TCSs > 1 year postchemotherapy underwent physical examination and completed a questionnaire. Multinomial logistic regression estimated AHOs odds ratios (ORs) in relation to age, cumulative cisplatin and/or bleomycin dose, time since chemotherapy, sociodemographic factors, and health behaviors. Results Median age at evaluation was 37 years; median time since chemotherapy was 4.3 years. Chemotherapy consisted largely of BEPX3 (38.2%), EPX4 (30.9%), and BEPX4 (17.9%). None, one to two, three to four, or five or more AHOs were reported by 20.4%, 42.0%, 25.1%, and 12.5% of TCSs, respectively. Median number after EPX4 or BEPX3 was two (range, zero to nine and zero to 11, respectively; P > .05) and two (range, zero to 10) after BEPX4. When comparing individual AHOs for EPX4 versus BEPX3, Raynaud phenomenon (11.6% v 21.4%; P < .01), peripheral neuropathy (29.2% v 21.4%; P = .02), and obesity (25.5% v 33.0%; P = .04) differed. Larger cumulative bleomycin doses (OR, 1.44 per 90,000 IU) were significantly associated with five or more AHOs. Increasing age was a significant risk factor for one to two, three to four, or five or more AHOs versus zero AHOs (OR, 1.22, 1.50, and 1.87 per 5 years, respectively; P < .01); vigorous physical activity was protective (OR, 0.62, 0.51, and 0.41, respectively; P < .05). Significant risk factors for three to four and five or more AHOs included current (OR, 3.05 and 3.73) or former (OR, 1.61 and 1.76) smoking ( P < .05). Self-reported health was excellent/very good in 59.9% of TCSs but decreased as AHOs increased ( P < .001). Conclusion Numbers of AHOs after EPX4 or BEPX3 appear similar, with median follow-up of 4.3 years. A healthy lifestyle was associated with reduced number of AHOs. FAU - Fung, Chunkit AU - Fung C AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Sesso, Howard D AU - Sesso HD AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Williams, Annalynn M AU - Williams AM AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Kerns, Sarah L AU - Kerns SL AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Monahan, Patrick AU - Monahan P AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Abu Zaid, Mohammad AU - Abu Zaid M AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Feldman, Darren R AU - Feldman DR AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Hamilton, Robert J AU - Hamilton RJ AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Vaughn, David J AU - Vaughn DJ AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Beard, Clair J AU - Beard CJ AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Kollmannsberger, Christian K AU - Kollmannsberger CK AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Cook, Ryan AU - Cook R AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Althouse, Sandra AU - Althouse S AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Ardeshir-Rouhani-Fard, Shirin AU - Ardeshir-Rouhani-Fard S AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Lipshultz, Steve E AU - Lipshultz SE AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Einhorn, Lawrence H AU - Einhorn LH AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Fossa, Sophie D AU - Fossa SD AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Travis, Lois B AU - Travis LB AD - Chunkit Fung, Annalynn M. Williams, and Sarah L. Kerns, University of Rochester Medical Center, James P. Wilmot Cancer Institute, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Howard D. Sesso, Brigham and Women's Hospital; Clair J. Beard, Dana-Farber Cancer Institute, Boston, MA; Patrick Monahan, Mohammad Abu Zaid, Ryan Cook, Sandra Althouse, Shirin Ardeshir-Rouhani-Fard, Lawrence H. Einhorn, and Lois B. Travis, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN; Robert J. Hamilton, Princess Margaret Cancer Center, Toronto, Ontario; Christian K. Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Steve E. Lipshultz, Wayne State University School of Medicine; Steve E. Lipshultz, Children's Hospital of Michigan; Steve E. Lipshultz, Karmanos Cancer Institute, Detroit, MI; and Sophie D. Fossa, Oslo University Hospital, Radium Hospital, Oslo, Norway. CN - Platinum Study Group LA - eng GR - K07 CA187546/CA/NCI NIH HHS/United States GR - R01 CA157823/CA/NCI NIH HHS/United States PT - Journal Article PT - Multicenter Study DEP - 20170227 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - 11056-06-7 (Bleomycin) RN - 6PLQ3CP4P3 (Etoposide) RN - Q20Q21Q62J (Cisplatin) SB - IM CIN - Eur Urol. 2017 Nov;72(5):857-858. doi: 10.1016/j.eururo.2017.06.017. PMID: 28655542 CIN - J Urol. 2018 Jan;199(1):29. doi: 10.1016/j.juro.2017.09.121. PMID: 29310185 MH - Adult MH - Age Factors MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*adverse effects MH - Bleomycin/administration & dosage/adverse effects MH - Canada/epidemiology MH - Case-Control Studies MH - Cisplatin/administration & dosage/adverse effects MH - Etoposide/administration & dosage MH - Exercise MH - Health Status MH - Hearing Loss/chemically induced/epidemiology MH - Humans MH - Long Term Adverse Effects/chemically induced/epidemiology MH - Male MH - Middle Aged MH - Obesity/chemically induced/*epidemiology MH - Peripheral Nervous System Diseases/chemically induced/*epidemiology MH - Prevalence MH - Protective Factors MH - Raynaud Disease/chemically induced/*epidemiology MH - Risk Factors MH - Smoking/epidemiology MH - Surveys and Questionnaires MH - Survivors/*statistics & numerical data MH - Testicular Neoplasms/*drug therapy MH - Tinnitus/chemically induced/epidemiology MH - United States/epidemiology MH - Young Adult PMC - PMC5455601 EDAT- 2017/02/28 06:00 MHDA- 2017/07/29 06:00 PMCR- 2018/03/10 CRDT- 2017/02/28 06:00 PHST- 2017/02/28 06:00 [pubmed] PHST- 2017/07/29 06:00 [medline] PHST- 2017/02/28 06:00 [entrez] PHST- 2018/03/10 00:00 [pmc-release] AID - 703108 [pii] AID - 10.1200/JCO.2016.70.3108 [doi] PST - ppublish SO - J Clin Oncol. 2017 Apr 10;35(11):1211-1222. doi: 10.1200/JCO.2016.70.3108. Epub 2017 Feb 27. PMID- 21626532 OWN - NLM STAT- MEDLINE DCOM- 20120706 LR - 20220330 IS - 1097-0258 (Electronic) IS - 0277-6715 (Linking) VI - 31 IP - 7 DP - 2012 Mar 30 TI - Random effects models for assessing diagnostic accuracy of traditional Chinese doctors in absence of a gold standard. PG - 661-71 LID - 10.1002/sim.4275 [doi] AB - Two common problems in assessing the accuracy of traditional Chinese medicine (TCM) doctors in detecting a particular symptom are the unknown true symptom status and the ordinal-scale of the symptom status. Wang et al. (Biostatistics 2011; DOI: 10.1093/biostatistics/kxq075) proposed a nonparametric maximum likelihood method for estimating the accuracy of different TCM doctors without a gold standard when the true symptom status is measured on an ordinal-scale. A key assumption of their work is that the diagnosis results are independent conditional on the gold standard. This assumption can be violated in many practical situations.In this paper, we propose a random effects modeling approach that extends their method to incorporate dependence structure among different tests or doctors. The proposed method is illustrated on a real data set from TCM, which contains the diagnostic results from five doctors for the same patients regarding symptoms related to Chills disease. The same data set was analyzed by Wang et al. under the conditional independence assumption. In addition, we also discuss an ad hoc test for the model fitting and a likelihood ratio test on the random effects. FAU - Wang, Zheyu AU - Wang Z AD - Department of Biostatistics, University of Washington, Box 357232, Seattle, WA 98195, USA. FAU - Zhou, Xiao-Hua AU - Zhou XH LA - eng PT - Journal Article DEP - 20110531 PL - England TA - Stat Med JT - Statistics in medicine JID - 8215016 SB - IM MH - Clinical Competence/*statistics & numerical data MH - Diagnosis, Differential MH - Diagnostic Errors/statistics & numerical data MH - Humans MH - *Medicine, Chinese Traditional MH - *Models, Biological MH - *Models, Statistical MH - Physicians/statistics & numerical data MH - ROC Curve MH - Raynaud Disease/*diagnosis EDAT- 2011/06/01 06:00 MHDA- 2012/07/07 06:00 CRDT- 2011/06/01 06:00 PHST- 2010/09/06 00:00 [received] PHST- 2011/03/17 00:00 [accepted] PHST- 2011/06/01 06:00 [entrez] PHST- 2011/06/01 06:00 [pubmed] PHST- 2012/07/07 06:00 [medline] AID - 10.1002/sim.4275 [doi] PST - ppublish SO - Stat Med. 2012 Mar 30;31(7):661-71. doi: 10.1002/sim.4275. Epub 2011 May 31. PMID- 18520574 OWN - NLM STAT- MEDLINE DCOM- 20081020 LR - 20161124 IS - 0883-5993 (Print) IS - 0883-5993 (Linking) VI - 23 IP - 2 DP - 2008 May TI - Pulmonary extramedullary hematopoiesis. PG - 138-41 LID - 10.1097/RTI.0b013e31815b89aa [doi] AB - Extramedullary hematopoiesis (EMH) is the formation and development of blood cells outside of the bone marrow. Of particular interest to chest physicians and radiologists is the occurrence of EMH in the lungs and pleura. There have been several reports of patients presenting with pulmonary EMH published in the literature and the majority are due to a secondary process, such as myeloproliferative disorders, hemolytic anemias, hereditary spherocytosis, and Gaucher disease. We present a case report of pulmonary EMH and a review of the literature concentrating on the etiology, clinical presentation, pathophysiology, diagnosis, and therapy for this uncommon disorder. FAU - Bowling, Mark Rollin AU - Bowling MR AD - Department of Internal Medicine, Section on Pulmonary and Critical Care, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157, USA. MBowling@wfumbc.edu FAU - Cauthen, Carlton Gregory AU - Cauthen CG FAU - Perry, Christopher David AU - Perry CD FAU - Patel, Nilesh Prahlad AU - Patel NP FAU - Bergman, Simon AU - Bergman S FAU - Link, Kerry Michael AU - Link KM FAU - Sane, Aneysa Christine AU - Sane AC FAU - Conforti, John Frank AU - Conforti JF LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Thorac Imaging JT - Journal of thoracic imaging JID - 8606160 RN - 0 (Antirheumatic Agents) RN - MRK240IY2L (Azathioprine) SB - IM MH - Anemia, Hemolytic/etiology MH - Antirheumatic Agents/therapeutic use MH - Arthritis/complications MH - Azathioprine/therapeutic use MH - Biopsy, Fine-Needle MH - Diagnosis, Differential MH - Dry Eye Syndromes/etiology MH - Dyspnea/etiology MH - Female MH - Hematopoiesis, Extramedullary/*drug effects MH - Humans MH - Lung/*diagnostic imaging/pathology MH - Lung Diseases/*diagnosis/drug therapy MH - Middle Aged MH - Pleural Effusion/etiology MH - Pulmonary Fibrosis/complications MH - Raynaud Disease/etiology MH - Sjogren's Syndrome/complications/drug therapy MH - Sleep Apnea, Obstructive/complications MH - Tomography, X-Ray Computed MH - Vasculitis/complications EDAT- 2008/06/04 09:00 MHDA- 2008/10/22 09:00 CRDT- 2008/06/04 09:00 PHST- 2008/06/04 09:00 [pubmed] PHST- 2008/10/22 09:00 [medline] PHST- 2008/06/04 09:00 [entrez] AID - 00005382-200805000-00012 [pii] AID - 10.1097/RTI.0b013e31815b89aa [doi] PST - ppublish SO - J Thorac Imaging. 2008 May;23(2):138-41. doi: 10.1097/RTI.0b013e31815b89aa. PMID- 30383134 OWN - NLM STAT- MEDLINE DCOM- 20190903 LR - 20190903 IS - 1539-3704 (Electronic) IS - 0003-4819 (Linking) VI - 169 IP - 10 DP - 2018 Nov 20 TI - On-Demand Sildenafil as a Treatment for Raynaud Phenomenon: A Series of n-of-1 Trials. PG - 694-703 LID - 10.7326/M18-0517 [doi] AB - BACKGROUND: Treatment of Raynaud phenomenon (RP) with phosphodiesterase-5 inhibitors has shown moderate efficacy. Adverse effects decrease the risk-benefit profile of these drugs, and patients may not be willing to receive long-term treatment. On-demand single doses before or during exposure to cold may be a good alternative. OBJECTIVE: To assess the efficacy and safety of on-demand sildenafil in RP. DESIGN: Series of randomized, double-blind, n-of-1 trials. (ClinicalTrials.gov: NCT02050360). SETTING: Outpatients at a French university hospital. PARTICIPANTS: Patients with primary or secondary RP. INTERVENTION: Each trial consisted of a multiple crossover study in a single patient. Repeated blocks of 3 periods of on-demand treatment were evaluated: 1 week of placebo, 1 week of sildenafil at 40 mg per dose, and 1 week of sildenafil at 80 mg per dose, with a maximum of 2 doses daily. MEASUREMENTS: Raynaud Condition Score (RCS) and frequency and daily duration of attacks. Skin blood flow in response to cooling also was assessed with laser speckle contrast imaging. Mixed-effects models were used and parameters were estimated in a Bayesian framework to determine individual and aggregated efficacy. RESULTS: 38 patients completed 2 to 5 treatment blocks. On the basis of aggregated data, the probability that sildenafil at 40 mg or 80 mg was more effective than placebo was greater than 90% for all outcomes (except for RCS with sildenafil, 80 mg). However, the aggregated effect size was not clinically relevant. Yet, substantial heterogeneity in sildenafil's efficacy was observed among participants, with clinically relevant efficacy in some patients. LIMITATION: The response to sildenafil was substantially heterogeneous among patients. CONCLUSION: Despite a high probability that sildenafil is superior to placebo, substantial heterogeneity was observed in patient response and aggregated results did not show that on-demand sildenafil has clinically relevant efficacy. In this context, the use of n-of-1 trials may be an original and relevant approach in RP. PRIMARY FUNDING SOURCE: GIRCI (Groupement Interrégional de Recherche Clinique et d'Innovation) Auvergne Rhône-Alpes (academic funding) and Pfizer. FAU - Roustit, Matthieu AU - Roustit M AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Giai, Joris AU - Giai J AD - Université de Lyon and Hospices Civils de Lyon, Lyon, France (J.G., F.S.). FAU - Gaget, Olivier AU - Gaget O AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Khouri, Charles AU - Khouri C AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Mouhib, Myriam AU - Mouhib M AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Lotito, Adrien AU - Lotito A AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Blaise, Sophie AU - Blaise S AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Seinturier, Christophe AU - Seinturier C AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Subtil, Fabien AU - Subtil F AD - Université de Lyon and Hospices Civils de Lyon, Lyon, France (J.G., F.S.). FAU - Paris, Adeline AU - Paris A AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Cracowski, Claire AU - Cracowski C AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Imbert, Bernard AU - Imbert B AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Carpentier, Patrick AU - Carpentier P AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). FAU - Vohra, Sunita AU - Vohra S AD - University of Alberta, Edmonton, Alberta, Canada (S.V.). FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Université Grenoble Alpes and Grenoble Alpes University Hospital, Grenoble, France (M.R., O.G., C.K., M.M., A.L., S.B., C.S., A.P., C.C., B.I., P.C., J.C.). LA - eng SI - ClinicalTrials.gov/NCT02050360 PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20181030 PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM CIN - Ann Intern Med. 2019 Feb 19;170(4):JC21. doi: 10.7326/ACPJ201902190-021. PMID: 30776810 MH - Adult MH - Cross-Over Studies MH - Data Interpretation, Statistical MH - Double-Blind Method MH - Drug Administration Schedule MH - Female MH - Humans MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors/*administration & dosage/*adverse effects MH - Prospective Studies MH - Raynaud Disease/*drug therapy MH - Sildenafil Citrate/*administration & dosage/*adverse effects MH - Treatment Outcome MH - Vasodilator Agents/*administration & dosage/*adverse effects EDAT- 2018/11/02 06:00 MHDA- 2019/09/04 06:00 CRDT- 2018/11/02 06:00 PHST- 2018/11/02 06:00 [pubmed] PHST- 2019/09/04 06:00 [medline] PHST- 2018/11/02 06:00 [entrez] AID - 2709822 [pii] AID - 10.7326/M18-0517 [doi] PST - ppublish SO - Ann Intern Med. 2018 Nov 20;169(10):694-703. doi: 10.7326/M18-0517. Epub 2018 Oct 30. PMID- 23395500 OWN - NLM STAT- MEDLINE DCOM- 20130730 LR - 20130211 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 140 IP - 2 DP - 2013 Feb TI - [Systemic sclerosis autoantibodies: what dermatologists must know]. PG - 143-9 LID - S0151-9638(12)01382-8 [pii] LID - 10.1016/j.annder.2012.11.012 [doi] FAU - Hüe, S AU - Hüe S AD - Laboratoire d'immunologie biologique, hôpital Henri-Mondor, AP-HP, université Paris-Est-Créteil UPEC, CHU, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94000 Créteil, France. FAU - Ingen-Housz-Oro, S AU - Ingen-Housz-Oro S FAU - Cosnes, A AU - Cosnes A LA - fre PT - Journal Article PT - Review TT - Anticorps de la sclérodermie systémique: ce que le dermatologue doit savoir. DEP - 20130116 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantigens) RN - 0 (Chromosomal Proteins, Non-Histone) RN - 0 (Pol1 Transcription Initiation Complex Proteins) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 0 (fibrillarin) RN - 0 (transcription factor UBF) RN - EC 2.7.7.6 (DNA-Directed RNA Polymerases) RN - EC 3.1.- (Exoribonucleases) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - EC 3.1.13.- (EXOSC10 protein, human) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 5.99.1.2 (TOP1 protein, human) SB - IM MH - Antibodies, Antinuclear/blood/*immunology MH - Autoantigens/immunology MH - Cell Nucleolus/immunology MH - Centromere/immunology MH - Chromosomal Proteins, Non-Histone/immunology MH - DNA Topoisomerases, Type I/immunology MH - DNA-Directed RNA Polymerases/immunology MH - Endothelium, Vascular/pathology MH - Exoribonucleases/immunology MH - Exosome Multienzyme Ribonuclease Complex/immunology MH - Fibroblasts/pathology MH - Fibrosis MH - Fluorescent Antibody Technique, Indirect MH - Humans MH - Lymphocyte Subsets/immunology MH - Oxidative Stress MH - Pol1 Transcription Initiation Complex Proteins/immunology MH - Raynaud Disease/etiology MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Scleroderma, Systemic/complications/*immunology/physiopathology EDAT- 2013/02/12 06:00 MHDA- 2013/07/31 06:00 CRDT- 2013/02/12 06:00 PHST- 2012/09/07 00:00 [received] PHST- 2012/11/05 00:00 [revised] PHST- 2012/11/23 00:00 [accepted] PHST- 2013/02/12 06:00 [entrez] PHST- 2013/02/12 06:00 [pubmed] PHST- 2013/07/31 06:00 [medline] AID - S0151-9638(12)01382-8 [pii] AID - 10.1016/j.annder.2012.11.012 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2013 Feb;140(2):143-9. doi: 10.1016/j.annder.2012.11.012. Epub 2013 Jan 16. PMID- 20225230 OWN - NLM STAT- MEDLINE DCOM- 20100525 LR - 20220414 IS - 0008-543X (Print) IS - 0008-543X (Linking) VI - 116 IP - 10 DP - 2010 May 15 TI - Long-term neurologic and peripheral vascular toxicity after chemotherapy treatment of testicular cancer. PG - 2322-31 LID - 10.1002/cncr.24981 [doi] AB - BACKGROUND: Testicular cancer is curable in the majority of men, and persisting treatment toxicity is a concern. The authors report a cross-sectional study of the long-term effects of chemotherapy (C) on neurologic function and development of Raynaud phenomenon. METHODS: Seven hundred thirty-nine patients who were treated between 1982 and 1992 gave consent to enter the study. Patients were classified according to the receipt of C (n = 384) or no C (n = 355). Patients completed a general health questionnaire and a quality-of-life form (the European Organization for Research and Treatment of Cancer Quality-of-Life C30 questionnaire with testicular module). Symptom scores of 3 or 4 were considered clinically significant. Patients were assessed in the clinic, and clinical history was used to diagnose Raynaud phenomenon (RP) and tinnitus. Examinations included peripheral nerve function testing for light touch and vibration sense. Five hundred seventy-seven patients underwent audiometry. RESULTS: On physician assessment, peripheral neuropathy and RP were more common after C (21.7% vs 9.1% [P<.001] and 20.3% vs 1.7% [P<.001], respectively). Similar results were obtained for symptom scores (12.5% vs 5.5% [P = .002] and 9.7% vs 3.7% [P<.001], respectively). On multivariate analysis, for peripheral neuropathy, the significant predictors were cisplatin dose, carboplatin dose, and age. For RP, the significant predictor was bleomycin. Significant differences in hearing thresholds were noted at 8000 hertz only and, on multivariate analysis, were related to age, cisplatin dose, and vincristine dose. Auditory symptom scores did not differ between groups. CONCLUSIONS: With long-term follow-up, peripheral neuropathy and RP remained detectable in approximately 20% of patients and caused significant symptoms in 10% of patients. Detectable effects on high frequency remained but caused little symptomatic problem. These effects persisted and were related to the cumulative chemotherapy dose. CI - (c) 2010 American Cancer Society. FAU - Glendenning, Jennifer L AU - Glendenning JL AD - Urology Unit, The Royal Marsden National Health Service Foundation Trust, Sutton, Surrey, United Kingdom. FAU - Barbachano, Yolanda AU - Barbachano Y FAU - Norman, Andy R AU - Norman AR FAU - Dearnaley, David P AU - Dearnaley DP FAU - Horwich, Alan AU - Horwich A FAU - Huddart, Robert A AU - Huddart RA LA - eng GR - C46/A2131/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Cancer JT - Cancer JID - 0374236 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use MH - Hearing Loss/chemically induced MH - Humans MH - Male MH - Microcirculation/drug effects MH - Middle Aged MH - Peripheral Nervous System Diseases/*chemically induced MH - Raynaud Disease/*chemically induced MH - Testicular Neoplasms/*drug therapy MH - Time MH - Tinnitus/chemically induced EDAT- 2010/03/13 06:00 MHDA- 2010/05/26 06:00 CRDT- 2010/03/13 06:00 PHST- 2010/03/13 06:00 [entrez] PHST- 2010/03/13 06:00 [pubmed] PHST- 2010/05/26 06:00 [medline] AID - 10.1002/cncr.24981 [doi] PST - ppublish SO - Cancer. 2010 May 15;116(10):2322-31. doi: 10.1002/cncr.24981. PMID- 32965452 OWN - NLM STAT- MEDLINE DCOM- 20200929 LR - 20201218 IS - 1678-9849 (Electronic) IS - 0037-8682 (Print) IS - 0037-8682 (Linking) VI - 53 DP - 2020 TI - Covid-19 overlapping with systemic sclerosis. PG - e20200450 LID - S0037-86822020000100826 [pii] LID - 10.1590/0037-8682-0450-2020 [doi] LID - e20200450 FAU - Mariano, Rachel Zerbini AU - Mariano RZ AD - Universidade Estadual de Campinas, Faculdade de Ciências Médicas, Departamento de Radiologia, Campinas, SP, Brasil. FAU - Rio, Ana Paula Toledo Del AU - Rio APTD AD - Universidade Estadual de Campinas, Faculdade de Ciências Médicas, Departamento de Medicina Interna, Campinas, SP, Brasil. FAU - Reis, Fabiano AU - Reis F AUID- ORCID: 0000-0003-2256-4379 AD - Universidade Estadual de Campinas, Faculdade de Ciências Médicas, Departamento de Radiologia, Campinas, SP, Brasil. LA - eng PT - Case Reports PT - Journal Article DEP - 20200921 PL - Brazil TA - Rev Soc Bras Med Trop JT - Revista da Sociedade Brasileira de Medicina Tropical JID - 7507456 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Aged MH - Antibodies, Antinuclear MH - Betacoronavirus/genetics/*isolation & purification MH - Bronchiectasis MH - COVID-19 MH - Coronavirus Infections/*diagnosis MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/*diagnostic imaging MH - Lung/*diagnostic imaging MH - Microscopic Angioscopy MH - *Pandemics MH - Pneumonia, Viral/*diagnosis MH - Raynaud Disease MH - Real-Time Polymerase Chain Reaction MH - SARS-CoV-2 MH - Scleroderma, Systemic/complications/*diagnostic imaging MH - Tomography, X-Ray Computed PMC - PMC7508197 COIS- Conflict of Interest: The authors declare that there is no conflict of interest. EDAT- 2020/09/24 06:00 MHDA- 2020/09/30 06:00 PMCR- 2020/09/21 CRDT- 2020/09/23 12:12 PHST- 2020/07/09 00:00 [received] PHST- 2020/08/10 00:00 [accepted] PHST- 2020/09/23 12:12 [entrez] PHST- 2020/09/24 06:00 [pubmed] PHST- 2020/09/30 06:00 [medline] PHST- 2020/09/21 00:00 [pmc-release] AID - S0037-86822020000100826 [pii] AID - 10.1590/0037-8682-0450-2020 [doi] PST - epublish SO - Rev Soc Bras Med Trop. 2020 Sep 21;53:e20200450. doi: 10.1590/0037-8682-0450-2020. eCollection 2020. PMID- 26550776 OWN - NLM STAT- MEDLINE DCOM- 20160613 LR - 20250626 IS - 1539-3704 (Electronic) IS - 0003-4819 (Linking) VI - 164 IP - 3 DP - 2016 Feb 2 TI - Long-Term Health Outcomes in Women With Silicone Gel Breast Implants: A Systematic Review. PG - 164-75 LID - 10.7326/M15-1169 [doi] AB - BACKGROUND: Silicone gel breast implants were removed from the U.S. market for cosmetic use in 1992 owing to safety concerns. They were reintroduced in 2006, with a call for improved surveillance of clinical outcomes. PURPOSE: To systematically review the literature regarding specific long-term health outcomes in women with silicone gel breast implants, including cancer; connective tissue, rheumatologic, and autoimmune diseases; neurologic diseases; reproductive issues, including lactation; offspring issues; and mental health issues (depression and suicide). DATA SOURCES: MEDLINE, EMBASE, and Ovid Healthstar (inception through 30 June 2015), and the Cochrane Central Register of Controlled Trials and Cochrane Database of Systematic Reviews (through the first quarter of 2015). STUDY SELECTION: 4 researchers double-screened articles for longitudinal studies that compared women with and without breast implants and reported long-term health outcomes of interest. DATA EXTRACTION: 4 researchers extracted data on participant and implant characteristics, analytic methods, and results. DATA SYNTHESIS: 32 studies (in 58 publications) met eligibility criteria. Random-effects model meta-analyses of effect sizes were conducted when feasible. For most outcomes, there was at most only a single adequately adjusted study, which usually found no significant associations. There were possible associations with decreased risk for primary breast and endometrial cancers and increased risks for lung cancer, rheumatoid arthritis, Sjögren syndrome, and Raynaud syndrome. Evidence on breast implants and other outcomes either was limited or did not exist. LIMITATION: The evidence was most frequently not specific to silicone gel implants, and studies were rarely adequately adjusted for potential confounders. CONCLUSION: The evidence remains inconclusive about any association between silicone gel implants and long-term health outcomes. Better evidence is needed from existing large studies, which can be reanalyzed to clarify the strength of associations between silicone gel implants and health outcomes. PRIMARY FUNDING SOURCE: The Plastic Surgery Foundation. FAU - Balk, Ethan M AU - Balk EM FAU - Earley, Amy AU - Earley A FAU - Avendano, Esther A AU - Avendano EA FAU - Raman, Gowri AU - Raman G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20151110 PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - 0 (Silicone Gels) SB - IM CIN - Ann Intern Med. 2016 Feb 2;164(3):201-2. doi: 10.7326/M15-2307. PMID: 26551020 CIN - Ann Intern Med. 2016 Feb 2;164(3):199-200. doi: 10.7326/M15-2427. PMID: 26551242 CIN - Ann Intern Med. 2016 Oct 4;165(7):527. doi: 10.7326/L16-0195. PMID: 27699392 CIN - Ann Intern Med. 2016 Oct 4;165(7):527-528. doi: 10.7326/L16-0196. PMID: 27699393 MH - Arthritis, Rheumatoid/epidemiology MH - Autoimmune Diseases/epidemiology MH - *Breast Implants/adverse effects MH - Connective Tissue Diseases/epidemiology MH - Female MH - *Health Status MH - Humans MH - Lactation Disorders MH - Mental Health MH - Neoplasms/epidemiology MH - Raynaud Disease/epidemiology MH - Reproductive Health MH - Risk Factors MH - *Silicone Gels MH - Sjogren's Syndrome/epidemiology EDAT- 2015/11/10 06:00 MHDA- 2016/06/14 06:00 CRDT- 2015/11/10 06:00 PHST- 2015/11/10 06:00 [entrez] PHST- 2015/11/10 06:00 [pubmed] PHST- 2016/06/14 06:00 [medline] AID - 2468813 [pii] AID - 10.7326/M15-1169 [doi] PST - ppublish SO - Ann Intern Med. 2016 Feb 2;164(3):164-75. doi: 10.7326/M15-1169. Epub 2015 Nov 10. PMID- 17426360 OWN - NLM STAT- MEDLINE DCOM- 20071005 LR - 20220716 IS - 1080-0549 (Print) IS - 1080-0549 (Linking) VI - 32 IP - 1 DP - 2007 Feb TI - Increased prevalence of anti-third generation cyclic citrullinated peptide antibodies in patients with rheumatoid arthritis and CREST syndrome. PG - 47-56 AB - To investigate the prevalence of anti-third generation cyclic citrullinated peptide antibodies (anti-CCP3) in patients with systemic connective tissue diseases, we assembled a training set consisting of 115 patients with rheumatoid arthritis (RA), 52 with Calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, 21 with scleroderma, 20 with ankylosing spondylitis, 18 with reactive arthritis, 25 with juvenile rheumatoid arthritis (RA), 51 with osteoarthritis, 26 with mixed connective tissue disease, 23 with primary Sjogren's syndrome, 74 with systemic lupus erythematosus, 49 with Polymyalgia rheumatica, and 39 with polymyositis/dermatomyositis. The commercial enzyme-linked immunosorbent assay (ELISA) was used to detect anti-CCP antibodies, including anti-CCP2 (regular, second generation of CCP antigen) and anti-CCP3 (third generation of CCP antigen) in disease-related specimens and normal controls. These serum samples were also evaluated for anti-centomere antibodies by anti-centromere ELISA kit. The higher frequencies of anti-CCP3 and anti-CCP2 were detected in 75.6 and 70.4% patients with RA, respectively. At the same time, anti-CCP3 (not anti-CCP2) was significantly increased in samples isolated from patients with CREST syndrome. The clinical sensitivity of IgG anti-CCP3 for the patients with CREST syndrome was 29% (15 of 52) and the specificity was 96% (384 of 397), with the exception of the RA group. The anti-centromere antibodies were significantly higher in patients with CREST only. The results of our study suggest that compared to anti-CCP2 assay, the new anti-CCP3 assay can enhance the clinical sensitivity for diagnosis of RA and, as an associate marker combined with anticentromere, can distinguish CREST syndrome from other systemic connective tissue diseases, especially RA. The clinical specificity of anti-CCP3 was lower than anti-CCP2 assay in diagnosis of RA because of the crossreaction to the patients with CREST syndrome. FAU - Wu, R AU - Wu R AD - Pathway Diagnostics Inc., Malibu, CA, USA. FAU - Shovman, O AU - Shovman O FAU - Zhang, Y AU - Zhang Y FAU - Gilburd, B AU - Gilburd B FAU - Zandman-Goddard, G AU - Zandman-Goddard G FAU - Shoenfeld, Yehuda AU - Shoenfeld Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Autoantibodies) RN - 0 (Peptides, Cyclic) RN - 0 (cyclic citrullinated peptide) SB - IM MH - Arthritis, Rheumatoid/blood/complications/*immunology MH - Autoantibodies/*analysis/*immunology MH - CREST Syndrome/blood/complications/*immunology MH - Humans MH - Peptides, Cyclic/*immunology EDAT- 2007/04/12 09:00 MHDA- 2007/10/06 09:00 CRDT- 2007/04/12 09:00 PHST- 1999/11/30 00:00 [received] PHST- 1999/11/30 00:00 [revised] PHST- 1999/11/30 00:00 [accepted] PHST- 2007/04/12 09:00 [pubmed] PHST- 2007/10/06 09:00 [medline] PHST- 2007/04/12 09:00 [entrez] AID - CRIAI:32:1:47 [pii] AID - 10.1007/BF02686081 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2007 Feb;32(1):47-56. doi: 10.1007/BF02686081. PMID- 21869710 OWN - NLM STAT- MEDLINE DCOM- 20120223 LR - 20110902 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 17 IP - 6 DP - 2011 Sep TI - Capillaroscopic pattern at the toes of systemic sclerosis patients: does it "tell" more than those of fingers? PG - 311-4 LID - 10.1097/RHU.0b013e31822be4e8 [doi] AB - PURPOSE: The aim of the study was to compare nail fold capillaroscopic findings of the fingers with those of the toes in patients with systemic sclerosis (SSc). METHODS: Thirty-six patients with SSc were included in the study: 30 patients had limited SSc, 5 patients had diffuse SSc, and 1 patient had an overlap syndrome. Of these 36 patients, 30 were women and 6 were men (mean [SD] age, 56 [14] years). The severity of the Raynaud phenomenon (RP), the presence of digital ulcers, and skin scores at the fingers and feet were assessed. Nail fold capillaroscopy was performed with a videocapillaroscope (Videocap 3.0; DS Medica). RESULTS: All 36 patients (100%) reported about symptoms of RP in the hands and 34 (94.4%) reported episodes of RP in the feet; the difference is not significant (P > 0.05). In most patients with RP symptoms of both hands and feet, the symptoms were more severe at the hands (82%, 28/34). Digital ulcers of the fingers were found in 36% (13/36) of the case and those of the toes were found in 8.3% (3/36) of the cases. Nail fold capillaroscopy of the hands showed the classic "scleroderma"-type capillaroscopic pattern in 97.2% (35/36) of the patients. In the toes, a scleroderma-type capillaroscopic pattern was found only in 66.7% (24/36) of patients (P < 0.05). With respect to distinct differences, in the toes, the dilated capillaries were found in 72.2% (26/36) of the cases, giant capillaries in 30.6% (11/36) of the cases, hemorrhages in 8.3% (3/36) of the cases, avascular areas in 41.7% (15/36) of the cases, and neoangiogenesis in 22.1% (8/36) of the cases. This difference in frequency of the findings regarding the toes and the fingers of patients with SSc was statistically significant for all parameters. CONCLUSIONS: Capillaroscopy of the toes of SSc also shows patterns characteristic of SSc. However, these patterns differ from the respective patterns of the fingers, which is probably related to less-severe RP and lower skin score at the feet. FAU - Lambova, Sevdalina AU - Lambova S AD - Department for Propedeutics of Internal Medicine, Clinic of Rheumatology, Medical University - Plovdiv, Bulgaria. ssevdalina_n@abv.bg FAU - Hermann, Walter AU - Hermann W FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Comparative Study PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - Fingers/*blood supply MH - Hemorrhage/epidemiology MH - Humans MH - Incidence MH - Male MH - Microcirculation/*physiology MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/blood supply MH - Neovascularization, Pathologic/epidemiology MH - Raynaud Disease/epidemiology MH - Regional Blood Flow/physiology MH - Scleroderma, Systemic/*physiopathology MH - Severity of Illness Index MH - Toes/*blood supply EDAT- 2011/08/27 06:00 MHDA- 2012/02/24 06:00 CRDT- 2011/08/27 06:00 PHST- 2011/08/27 06:00 [entrez] PHST- 2011/08/27 06:00 [pubmed] PHST- 2012/02/24 06:00 [medline] AID - 10.1097/RHU.0b013e31822be4e8 [doi] PST - ppublish SO - J Clin Rheumatol. 2011 Sep;17(6):311-4. doi: 10.1097/RHU.0b013e31822be4e8. PMID- 28292796 OWN - NLM STAT- MEDLINE DCOM- 20170330 LR - 20260127 IS - 1715-5258 (Electronic) IS - 0008-350X (Print) IS - 0008-350X (Linking) VI - 63 IP - 3 DP - 2017 Mar TI - Hand-arm vibration syndrome: What family physicians should know. PG - 206-210 AB - OBJECTIVE: To provide family physicians with an understanding of the epidemiology, pathogenesis, symptoms, diagnosis, and management of hand-arm vibration syndrome (HAVS), an important and common occupational disease in Canada. SOURCES OF INFORMATION: A MEDLINE search was conducted for research and review articles on HAVS. A Google search was conducted to obtain gray literature relevant to the Canadian context. Additional references were obtained from the articles identified. MAIN MESSAGE: Hand-arm vibration syndrome is a prevalent occupational disease affecting workers in multiple industries in which vibrating tools are used. However, it is underdiagnosed in Canada. It has 3 components-vascular, in the form of secondary Raynaud phenomenon; sensorineural; and musculoskeletal. Hand-arm vibration syndrome in its more advanced stages contributes to substantial disability and poor quality of life. Its diagnosis requires careful history taking, in particular occupational history, physical examination, laboratory tests to rule out alternative diagnoses, and referral to an occupational medicine specialist for additional investigations. Management involves reduction of vibration exposure, avoidance of cold conditions, smoking cessation, and medication. CONCLUSION: To ensure timely diagnosis of HAVS and improve prognosis and quality of life, family physicians should be aware of this common occupational disease and be able to elicit the relevant occupational history, refer patients to occupational medicine clinics, and appropriately initiate compensation claims. CI - Copyright© the College of Family Physicians of Canada. FAU - Shen, Shixin Cindy AU - Shen SC AD - Resident in the Department of Family and Community Medicine at St Michael's Hospital and in the Public Health and Preventive Medicine Residency Program at the University of Toronto in Ontario. cindy.shen@mail.utoronto.ca. FAU - House, Ronald A AU - House RA AD - Occupational medicine specialist in the Department of Occupational and Environmental Health at St Michael's Hospital and Associate Professor Emeritus in the Department of Occupational and Environmental Health at the University of Toronto. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Canada TA - Can Fam Physician JT - Canadian family physician Medecin de famille canadien JID - 0120300 SB - IM MH - Adult MH - Canada/epidemiology MH - *Family Practice MH - Hand-Arm Vibration Syndrome/*diagnosis/epidemiology/etiology/*therapy MH - Humans MH - Male MH - Occupational Diseases/*diagnosis/epidemiology/etiology/*therapy MH - Prognosis MH - Raynaud Disease/etiology PMC - PMC5349719 EDAT- 2017/03/16 06:00 MHDA- 2017/03/31 06:00 PMCR- 2017/03/01 CRDT- 2017/03/16 06:00 PHST- 2017/03/16 06:00 [entrez] PHST- 2017/03/16 06:00 [pubmed] PHST- 2017/03/31 06:00 [medline] PHST- 2017/03/01 00:00 [pmc-release] AID - 63/3/206 [pii] AID - 206 [pii] PST - ppublish SO - Can Fam Physician. 2017 Mar;63(3):206-210. PMID- 16782771 OWN - NLM STAT- MEDLINE DCOM- 20070823 LR - 20060825 IS - 0962-7480 (Print) IS - 0962-7480 (Linking) VI - 56 IP - 6 DP - 2006 Sep TI - Modification of the Stockholm Vascular Scale. PG - 422-5 AB - BACKGROUND: Staging hand-arm vibration syndrome (HAVS) depends upon accurate reporting of the extent and frequency of blanching attacks. Reporting may not be repeatable and not all individuals classifiable using the Stockholm Workshop Scale (SWS). For Department of Trade and Industry (Dti) coal miners' assessments, the SWS was modified to include a blanching score. Further modifications, which involve splitting Stage 2V into 'early' and 'late' have been proposed but the impact of this on classification has not been investigated. AIM: To investigate the impact of modifications in the SWS on HAVS classification. METHODS: Staging of individuals with HAVS according to the SWS using two modified scales. Two different cut-offs for defining 'frequent' blanching attacks (>or=3 or >or=7 attacks/week, respectively) were used. RESULTS: One hundred and sixty-five individuals were staged. Using the SWS, 58 and 31% of the population were unclassifiable using the two cut-offs, respectively. The modification splitting Stage 2V reduced the proportions that were unclassifiable to 2 and 9%, respectively, and increased those classified as Stage 2V. The cut-off for frequent attacks used (3 or 7) affected the proportion of individuals falling into the subdivisions of Stage 2 with 17 and 42% being classified as 2Vearly and 45 and 20% as 2Vlate, respectively. CONCLUSIONS: Subdividing Stage 2V enables more individuals to be classified, but the proportion falling into each category is susceptible to the cut-off used for defining frequent attacks. Caution may need to be applied if this categorization is used to make decisions regarding fitness to work. FAU - Poole, Kerry AU - Poole K AD - Health and Safety Laboratory, Harpur Hill, Buxton, Derbyshire SK17 9JN, UK. kerry.poole@hsl.gov.uk FAU - Elms, Joanne AU - Elms J FAU - Mason, Howard AU - Mason H LA - eng PT - Journal Article DEP - 20060616 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Arm MH - Cumulative Trauma Disorders/diagnosis MH - Hand MH - Hand Injuries/complications MH - Humans MH - Occupational Diseases/*diagnosis MH - Occupational Exposure MH - Raynaud Disease/*diagnosis MH - *Work Capacity Evaluation EDAT- 2006/06/20 09:00 MHDA- 2007/08/24 09:00 CRDT- 2006/06/20 09:00 PHST- 2006/06/20 09:00 [pubmed] PHST- 2007/08/24 09:00 [medline] PHST- 2006/06/20 09:00 [entrez] AID - kql044 [pii] AID - 10.1093/occmed/kql044 [doi] PST - ppublish SO - Occup Med (Lond). 2006 Sep;56(6):422-5. doi: 10.1093/occmed/kql044. Epub 2006 Jun 16. PMID- 39875750 OWN - NLM STAT- MEDLINE DCOM- 20250504 LR - 20250524 IS - 1179-1934 (Electronic) IS - 1172-7047 (Linking) VI - 39 IP - 3 DP - 2025 Mar TI - Raynaud Syndrome Associated with Medication for Attention-Deficit/Hyperactivity Disorder: A Systematic Review. PG - 213-241 LID - 10.1007/s40263-024-01154-4 [doi] AB - BACKGROUND: Raynaud syndrome (RS) is a peripheral vasculopathy characterised be impaired acral perfusion typically manifesting as skin discolouration with pallor, cyanosis and/or erythema, and increased sensitivity to cold. RS may be primary or secondary to systemic disease, lifestyle and environmental factors or medication. RS has been reported with medication to treat ADHD, but we found no recent comprehensive overview of the literature. The aim of this review is to evaluate the evidence in the published literature for Raynaud syndrome associated with medication for ADHD. METHODS: We systematically searched PubMed and Embase from inception to 12 June 2024 for articles published in English describing cases of RS in individuals treated with stimulant medication, atomoxetine, guanfacine or clonidine. Identified cases were assessed against the Naranjo Adverse Drug Reaction Scale criteria to determine the probability of a causal relationship with the medication. RESULTS: The initial search identified 197 articles. A total of 61 cases were identified from 15 case reports, 5 case series, 1 retrospective case-control study, and 1 retrospective cohort study. No randomised, controlled studies were identified. Implicated medications included methylphenidate, (dex)amfetamine and, more rarely, atomoxetine. Most cases were mild and resolved within weeks of discontinuation, dose reduction or switch to an alternative medication. A few cases associated with systemic disease were reported, leading to ulceration, gangrene and the need for amputation or revascularisation in some individuals. Assessment of 28 cases using the Naranjo criteria suggested a 'possible' causative role of ADHD medication in 13 cases, a 'probable' role in 13 cases and a 'definite' role in two cases. CONCLUSIONS: Due to the uncontrolled nature of all but one of the available studies, a causal relationship between medication for ADHD and RS could not be determined reliably. However, in view of the possibility of severe sequelae, albeit in rare cases, routine monitoring for signs of RS is recommended in individuals treated with CNS stimulants or atomoxetine, especially when initiating treatment or increasing the dose. Large database studies in which individuals act as their own controls should be conducted to clarify any association between treatment with these medications and RS, controlling for confounding factors. CI - © 2025. The Author(s), under exclusive licence to Springer Nature Switzerland AG. FAU - Besag, Frank M C AU - Besag FMC AUID- ORCID: 0000-0002-4530-7933 AD - East London NHS Foundation Trust, Bedford, UK. fbesag@aol.com. AD - University College London, London, UK. fbesag@aol.com. AD - King's College London, London, UK. fbesag@aol.com. FAU - Vasey, Michael J AU - Vasey MJ AUID- ORCID: 0000-0003-1983-3531 AD - East London NHS Foundation Trust, Bedford, UK. FAU - Roy, Sulagna AU - Roy S AUID- ORCID: 0009-0005-6824-7266 AD - Faculty of Environmental and Life Sciences, Centre for Innovation in Mental Health, School of Psychology, University of Southampton, Southampton, UK. FAU - Cortese, Samuele AU - Cortese S AUID- ORCID: 0000-0001-5877-8075 AD - Faculty of Environmental and Life Sciences, Centre for Innovation in Mental Health, School of Psychology, University of Southampton, Southampton, UK. AD - Clinical and Experimental Sciences (CNS and Psychiatry), Faculty of Medicine, University of Southampton, Southampton, UK. AD - Solent NHS Trust, Southampton, UK. AD - Hassenfeld Children's Hospital at NYU Langone, New York University Child Study Center, New York City, New York, USA. AD - DiMePRe-J-Department of Precision and Rigenerative Medicine-Jonic Area, University of Bari "Aldo Moro", Bari, Italy. LA - eng PT - Journal Article PT - Systematic Review DEP - 20250129 PL - New Zealand TA - CNS Drugs JT - CNS drugs JID - 9431220 RN - 0 (Central Nervous System Stimulants) RN - 57WVB6I2W0 (Atomoxetine Hydrochloride) SB - IM MH - Humans MH - *Attention Deficit Disorder with Hyperactivity/drug therapy MH - *Raynaud Disease/chemically induced MH - *Central Nervous System Stimulants/adverse effects MH - Atomoxetine Hydrochloride/adverse effects COIS- Declarations. Funding: No funding was received in relation to the preparation of this manuscript. Conflicts of Interest: S.C., NIHR Research Professor (NIHR303122) is funded by the NIHR for this research project. The views expressed in this publication are those of the author(s) and not necessarily those of the NIHR, NHS or the UK Department of Health and Social Care. S.C. is also supported by NIHR grants NIHR203684, NIHR203035, NIHR130077, NIHR128472, RP-PG-0618-20003 and by grant 101095568-HORIZONHLTH- 2022-DISEASE-07-03 from the European Research Executive Agency. S.C. has declared reimbursement for travel and accommodation expenses from the Association for Child and Adolescent Central Health (ACAMH) in relation to lectures delivered for ACAMH, the Canadian AADHD Alliance Resource, the British Association of Psychopharmacology, and from Healthcare Convention for educational activity on ADHD, and has received honoraria from Medice. S.C. is an Editorial Board member of CNS Drugs. S.C. was not involved in the selection of peer reviewers for the manuscript nor any of the subsequent editorial decisions. The other authors declare that they have no conflicts of interest. Ethics Approval: Not applicable. Consent to Participate: Not applicable. Consent to Publish: Not applicable. Availability of Data and Material: Data sharing not applicable to this article as no datasets were generated or analysed during the current study. Code Availability: Not applicable. Authors Contributions: The authors contributed equally to the drafting of the review text. The primary literature search and article screening was conducted by F.B. and M.V. S.R. and M.V. conducted the Naranjo assessment with mediation provided by F.B. All authors read and approved the final manuscript and agree to be accountable for the work presented. EDAT- 2025/01/29 00:20 MHDA- 2025/02/24 12:26 CRDT- 2025/01/28 23:30 PHST- 2024/12/19 00:00 [accepted] PHST- 2025/02/24 12:26 [medline] PHST- 2025/01/29 00:20 [pubmed] PHST- 2025/01/28 23:30 [entrez] AID - 10.1007/s40263-024-01154-4 [pii] AID - 10.1007/s40263-024-01154-4 [doi] PST - ppublish SO - CNS Drugs. 2025 Mar;39(3):213-241. doi: 10.1007/s40263-024-01154-4. Epub 2025 Jan 29. PMID- 17909740 OWN - NLM STAT- MEDLINE DCOM- 20081003 LR - 20220317 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 5 DP - 2008 May TI - Pregnancy in systemic lupus erythematosus: a retrospective study from a developing community. PG - 577-80 AB - Little data exists from the developing world on pregnancy in systemic lupus erythematosus (SLE). A 10-year review of pregnancies in lupus patients was conducted at a tertiary hospital in a developing country. Forty-seven pregnancies in 31 patients were identified. Eleven (23%) booked after 20 weeks gestation. There were no maternal deaths; six (13%) mothers experienced flares-all mild. Twelve women developed preeclampsia of which one experienced an intrauterine death. One patient was diagnosed with lupus and nephritis during pregnancy. She required an abortion to control the disease. Another with active nephritis delivered a normal but premature infant despite cyclophosphamide therapy. There was only minor deterioration in renal function. There were 36 (77%) live births, 8 first trimester abortions, 2 elective abortions and 1 still birth. Fourteen (39%) of live births were premature, and five (14%) experienced intrauterine growth retardation (IUGR). Two live-born babies experienced neonatal heartblock, and one, a neonatal lupus rash. We discuss these finding in relation to risk factors and to results from the developed world. FAU - Whitelaw, D A AU - Whitelaw DA AD - Division of Rheumatology, Stellenbosch University and Tygerberg Hospital, Parow Valley, Bellville 7505, South Africa. dwhit@sun.ac.za FAU - Hall, D AU - Hall D FAU - Kotze, T AU - Kotze T LA - eng PT - Journal Article DEP - 20071002 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antiphospholipid) RN - 9007-36-7 (Complement System Proteins) SB - IM MH - Adolescent MH - Adult MH - Antibodies, Antiphospholipid/blood MH - Complement System Proteins/metabolism MH - *Developing Countries MH - Female MH - Humans MH - Infant, Low Birth Weight MH - Infant, Newborn MH - Infant, Premature MH - Lupus Erythematosus, Systemic/blood/*complications/immunology MH - Pre-Eclampsia/etiology MH - Pregnancy MH - Pregnancy Complications/blood/*etiology/immunology MH - Pregnancy Outcome MH - Raynaud Disease/etiology MH - Retrospective Studies MH - South Africa EDAT- 2007/10/03 09:00 MHDA- 2008/10/04 09:00 CRDT- 2007/10/03 09:00 PHST- 2007/06/14 00:00 [received] PHST- 2007/09/10 00:00 [accepted] PHST- 2007/08/15 00:00 [revised] PHST- 2007/10/03 09:00 [pubmed] PHST- 2008/10/04 09:00 [medline] PHST- 2007/10/03 09:00 [entrez] AID - 10.1007/s10067-007-0749-0 [doi] PST - ppublish SO - Clin Rheumatol. 2008 May;27(5):577-80. doi: 10.1007/s10067-007-0749-0. Epub 2007 Oct 2. PMID- 21570655 OWN - NLM STAT- MEDLINE DCOM- 20110915 LR - 20161125 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 22 IP - 3 DP - 2011 Jun TI - Erectile dysfunction of sclerodermic patients correlates with digital vascular damage. PG - 318-21 LID - 10.1016/j.ejim.2010.09.013 [doi] AB - BACKGROUND: The prevalence of erectile dysfunction (ED) in men with systemic sclerosis (SSc) can be considered a manifestation of endothelium damage. Aim of the study is to investigate ED in SSc patients by color Doppler ultrasound examination and to correlate it with disease severity and digital vascular damage. METHODS: In 20 males SSc patients blood flow velocity in the cavernous artery was determined with Duplex ultrasonography. Naifold videocapillaroscopy, Sexual Health Inventory for Men (SHIM) and Medsger Disease Severity Scale (DSS) were performed. Arteriogenic ED was defined by the presence of a reduced peak systolic velocity (PSVs), while diastolic velocity (EDV) and the resistive index (RI) were estimated to evaluate venocclusive dysfunction. SSc patients are classified by capillaroscopic pattern and vascular domain of DSS into two groups: low vascular damage (early or active capillaroscopic pattern and score of vascular domain of DSS≤2) and high vascular damage (late capillaroscopic pattern and score of vascular domain of DSS≥3). RESULTS: In all SSc patients a reduction of SHIM is present (mean 13.5±6.3). Patients with less vascular damage have a significantly (p<0.001) higher score of SHIM than patients with greater vascular damage (19.2±2.4 vs 7.9±2.7). No significant difference (p>0.5) between the two groups of vascular damage was found in PSVs. Venocclusive dysfunction was present only (p<0.001) in the group with high vascular damage. CONCLUSION: We can assert that there is a relationship between SSc vascular digital damage and ED. CI - Copyright © 2010 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. FAU - Rosato, Edoardo AU - Rosato E AD - Sapienza University of Rome, Department of Clinical Medicine, Clinical Immunology Unit, Scleroderma Center, Italy. FAU - Aversa, Antonio AU - Aversa A FAU - Molinaro, Ilenia AU - Molinaro I FAU - Pisarri, Simonetta AU - Pisarri S FAU - Spera, Giovanni AU - Spera G FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article DEP - 20101016 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 SB - IM MH - Adult MH - Arteries/diagnostic imaging MH - Erectile Dysfunction/diagnostic imaging MH - Humans MH - Impotence, Vasculogenic/*diagnostic imaging MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Penis/*blood supply/diagnostic imaging MH - Raynaud Disease/*diagnostic imaging MH - Scleroderma, Systemic/*diagnostic imaging MH - *Severity of Illness Index MH - Ultrasonography, Doppler, Color EDAT- 2011/05/17 06:00 MHDA- 2011/09/16 06:00 CRDT- 2011/05/17 06:00 PHST- 2010/05/13 00:00 [received] PHST- 2010/07/29 00:00 [revised] PHST- 2010/09/24 00:00 [accepted] PHST- 2011/05/17 06:00 [entrez] PHST- 2011/05/17 06:00 [pubmed] PHST- 2011/09/16 06:00 [medline] AID - S0953-6205(10)00196-2 [pii] AID - 10.1016/j.ejim.2010.09.013 [doi] PST - ppublish SO - Eur J Intern Med. 2011 Jun;22(3):318-21. doi: 10.1016/j.ejim.2010.09.013. Epub 2010 Oct 16. PMID- 17704623 OWN - NLM STAT- MEDLINE DCOM- 20071009 LR - 20220331 IS - 1880-6791 (Print) IS - 1880-6791 (Linking) VI - 26 IP - 4 DP - 2007 Jun TI - Perception of foot temperature in young women with cold constitution: analysis of skin temperature and warm and cold sensation thresholds. PG - 449-57 AB - To examine the disease state of cold constitution, physiological measurements of the foot were conducted by investigating thermal sensations under an environmental condition of 25 degrees C-26 degrees C (neutral temperature) in 29 young women with and without cold constitution. The subjects were classified into 3 groups according to their experiences with cold constitution: cold constitution, intermediate, and normal groups. Foot skin temperature was measured by thermography. Thermal sensations were measured on the dorsum of the left foot using a thermal stimulator. Cold and warm spots on the dorsum of the right foot were ascertained. Thermal stimulation was delivered by a copper probe. No significant differences in foot skin temperature among these 3 groups were identified as measured in a laboratory under neutral temperature conditions. However, the mean warm sensation threshold was +6.3+/-1.09 degrees C (mean+/-SEM) for the cold constitution group (n=14), +3.4+/-2.10 degrees C (mean+/-SEM) for the intermediate group (n=7), and -0.25+/-1.96 degrees C (mean+/-SEM) for the normal group (n=6). The difference was significant between the cold constitution and normal groups. No significant differences among the 3 groups were found in the cold sensation threshold. This may be attributable to the distribution of thermal receptors and to chronically reduced blood flow in subcutaneous tissues, where the skin temperature receptors responsible for temperature sensation are located. FAU - Sadakata, Mieko AU - Sadakata M AD - School of Health Sciences, Faculty of Medicine, Niigata University, Niigata, Niigata 951-8518, Japan. atom@clg.niigata-u.ac.jp FAU - Yamada, Yoshiaki AU - Yamada Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - J Physiol Anthropol JT - Journal of physiological anthropology JID - 101269653 SB - IM MH - Adult MH - Body Temperature Regulation/*physiology MH - Case-Control Studies MH - *Cold Temperature MH - Female MH - Foot/innervation/physiology MH - *Hot Temperature MH - Humans MH - Raynaud Disease/*physiopathology MH - *Skin Temperature MH - *Thermosensing EDAT- 2007/08/21 09:00 MHDA- 2007/10/10 09:00 CRDT- 2007/08/21 09:00 PHST- 2007/08/21 09:00 [pubmed] PHST- 2007/10/10 09:00 [medline] PHST- 2007/08/21 09:00 [entrez] AID - JST.JSTAGE/jpa2/26.449 [pii] AID - 10.2114/jpa2.26.449 [doi] PST - ppublish SO - J Physiol Anthropol. 2007 Jun;26(4):449-57. doi: 10.2114/jpa2.26.449. PMID- 17280777 OWN - NLM STAT- MEDLINE DCOM- 20070614 LR - 20131121 IS - 0303-8467 (Print) IS - 0303-8467 (Linking) VI - 109 IP - 4 DP - 2007 May TI - Myasthenia gravis and scleroderma: two cases and a review of the literature. PG - 388-91 AB - Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. We report two new cases and review clinical features of 12 other reported cases of co-existing scleroderma and myasthenia gravis, unrelated to previous d-penicillamine therapy. Co-occurrence of myasthenia and scleroderma was reported almost exclusively (13/14) in women with a mean latency of 7.03 years. Most patients (10/11) had seropositive generalized myasthenia, and there were no cases with exclusively ocular symptoms. Three patients with pre-existing myasthenia were safely treated with d-penicillamine. Myasthenia and scleroderma occur in the context of an underlying autoimmune diathesis, but their co-occurrence could be underreported as the recognition of either disorder may be delayed by overlapping clinical symptoms. Our findings also suggest that d-penicillamine may be cautiously used in selected patients with pre-existing scleroderma and myasthenia, when potential benefits outweigh the risk of possible myasthenia exacerbation. FAU - Zivković, Sasa A AU - Zivković SA AD - VA Pittsburgh Healthcare System, Pittsburgh, PA 15240, United States. zivkovics@upmc.edu FAU - Medsger, Thomas A Jr AU - Medsger TA Jr LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20070205 PL - Netherlands TA - Clin Neurol Neurosurg JT - Clinical neurology and neurosurgery JID - 7502039 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 34138-28-8 (penicillamide) RN - ONL14K3AFD (Penicillic Acid) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Autoantibodies/blood MH - Comorbidity MH - Follow-Up Studies MH - Hashimoto Disease/diagnosis MH - Humans MH - Middle Aged MH - Myasthenia Gravis/*diagnosis/surgery MH - Neurologic Examination MH - Penicillic Acid/administration & dosage/analogs & derivatives MH - Raynaud Disease/diagnosis MH - Scleroderma, Systemic/*diagnosis/drug therapy MH - Sjogren's Syndrome/diagnosis MH - Thymectomy MH - Thymus Hyperplasia/diagnosis/surgery RF - 30 EDAT- 2007/02/07 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/02/07 09:00 PHST- 2006/11/01 00:00 [received] PHST- 2007/01/02 00:00 [revised] PHST- 2007/01/05 00:00 [accepted] PHST- 2007/02/07 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/02/07 09:00 [entrez] AID - S0303-8467(07)00017-0 [pii] AID - 10.1016/j.clineuro.2007.01.006 [doi] PST - ppublish SO - Clin Neurol Neurosurg. 2007 May;109(4):388-91. doi: 10.1016/j.clineuro.2007.01.006. Epub 2007 Feb 5. PMID- 30791856 OWN - NLM STAT- MEDLINE DCOM- 20190724 LR - 20190724 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 28 IP - 4 DP - 2019 Apr TI - Uveal effusion and transient myopia as the initial presentation of systemic lupus erythematosus patient with pulmonary arterial hypertension: case report. PG - 560-564 LID - 10.1177/0961203319829819 [doi] AB - We reported uveal effusion and transient myopia as the initial presentation of systemic lupus erythematosus with pulmonary arterial hypertension. Choroidal retinopathy is rare but extremely destructive to visual function. Therefore, prompt diagnosis and effective treatments will result in complete resolution of the uveal effusion and functional restoration of vision. FAU - Wang, Y AU - Wang Y AUID- ORCID: 0000-0001-5629-451X AD - Department of Ophthalmology, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. FAU - Yan, Y AU - Yan Y AD - Department of Ophthalmology, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. FAU - Zhang, J AU - Zhang J AD - Department of Ophthalmology, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. FAU - Li, J AU - Li J AD - Department of Ophthalmology, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20190221 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antirheumatic Agents) RN - 0 (Glucocorticoids) RN - VB0R961HZT (Prednisone) MH - Adult MH - Antibodies, Antinuclear/blood MH - Antirheumatic Agents/therapeutic use MH - Choroid/diagnostic imaging/pathology MH - Choroid Diseases/*diagnosis/drug therapy/etiology MH - Ciliary Body/diagnostic imaging/pathology MH - Female MH - Fluorescein Angiography MH - Glucocorticoids/therapeutic use MH - Humans MH - Hypertension, Pulmonary/drug therapy/*etiology MH - Hypertrophy, Right Ventricular/diagnostic imaging MH - Lupus Erythematosus, Systemic/blood/*complications/*diagnosis/drug therapy MH - Microscopy, Acoustic MH - Myopia/*diagnosis/drug therapy/*etiology MH - Prednisone/therapeutic use MH - Quality of Life MH - Raynaud Disease/complications MH - Retina/diagnostic imaging/pathology MH - Retinal Detachment/diagnostic imaging/drug therapy/etiology MH - Tomography, Optical Coherence MH - Treatment Outcome MH - Uvea/*pathology MH - Vision, Low/drug therapy/etiology OTO - NOTNLM OT - Uveal effusion OT - ciliochoroidal disease OT - pulmonary arterial hypertension OT - systemic lupus erythematosus OT - vision loss EDAT- 2019/02/23 06:00 MHDA- 2019/07/25 06:00 CRDT- 2019/02/23 06:00 PHST- 2019/02/23 06:00 [pubmed] PHST- 2019/07/25 06:00 [medline] PHST- 2019/02/23 06:00 [entrez] AID - 10.1177/0961203319829819 [doi] PST - ppublish SO - Lupus. 2019 Apr;28(4):560-564. doi: 10.1177/0961203319829819. Epub 2019 Feb 21. PMID- 25582037 OWN - NLM STAT- MEDLINE DCOM- 20161110 LR - 20170103 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 42 IP - 3 DP - 2015 Mar TI - Clinical and laboratory features of systemic sclerosis complicated with localized scleroderma. PG - 283-7 LID - 10.1111/1346-8138.12775 [doi] AB - Localized scleroderma (LSc) primarily affects skin, whereas systemic sclerosis (SSc) affects skin and various internal organs. LSc and SSc are considered to be basically different diseases, and there is no transition between them. However, LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc. Clinical and laboratory characteristics of SSc patients with LSc remain unclear. We investigated the clinical and laboratory features of 8 SSc patients with LSc among 220 SSc patients (3.6%). The types of LSc included plaque (5/8), guttate (2/8), and linear type (1/8). All cases were diagnosed as having SSc within 5 years before or after the appearance of LSc. In three cases of SSc with LSc (37.5%), LSc skin lesions preceded clinical symptoms of SSc. Young age, negative antinuclear antibody, and positive anti-RNA polymerase III antibody were significantly prevalent in SSc patients with LSc. The positivity of anticentromere antibody tended to be prevalent in SSc patients without LSc. No significant difference in the frequency of complications, such as interstitial lung disease, reflux esophagitis, and pulmonary artery hypertension, was observed. The awareness of these characteristic of SSc with LSc are essential to establish an early diagnosis and treatment. CI - © 2015 Japanese Dermatological Association. FAU - Toki, Sayaka AU - Toki S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Motegi, Sei-ichiro AU - Motegi S FAU - Yamada, Kazuya AU - Yamada K FAU - Uchiyama, Akihiko AU - Uchiyama A FAU - Kanai, Sahori AU - Kanai S FAU - Yamanaka, Masayoshi AU - Yamanaka M FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article DEP - 20150113 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Adult MH - Age of Onset MH - Antibodies, Antinuclear/*blood MH - Autoimmune Diseases/complications MH - Female MH - Humans MH - Male MH - Middle Aged MH - RNA Polymerase III/*immunology MH - Raynaud Disease/etiology MH - Scleroderma, Localized/*blood/complications/pathology MH - Scleroderma, Systemic/*blood/complications OTO - NOTNLM OT - localized scleroderma OT - morphea OT - systemic sclerosis EDAT- 2015/01/15 06:00 MHDA- 2016/11/12 06:00 CRDT- 2015/01/14 06:00 PHST- 2014/10/30 00:00 [received] PHST- 2014/12/09 00:00 [accepted] PHST- 2015/01/14 06:00 [entrez] PHST- 2015/01/15 06:00 [pubmed] PHST- 2016/11/12 06:00 [medline] AID - 10.1111/1346-8138.12775 [doi] PST - ppublish SO - J Dermatol. 2015 Mar;42(3):283-7. doi: 10.1111/1346-8138.12775. Epub 2015 Jan 13. PMID- 25529235 OWN - NLM STAT- MEDLINE DCOM- 20160419 LR - 20221207 IS - 1098-2752 (Electronic) IS - 0738-1085 (Linking) VI - 35 IP - 5 DP - 2015 Jul TI - Intraoral reconstruction with "thinned" peroneal artery perforator flaps: An alternative to classic donor areas in comorbid patients. PG - 399-402 LID - 10.1002/micr.22366 [doi] AB - Free fasciocutaneous flaps like the radial forearm free flap (RFFF) and the anterolateral thigh (ALT) are the most commonly used flaps in intraoral reconstruction. However, certain conditions preclude the use of either of these flaps. The aim of this report was to show applicability of "thinned" peroneal artery perforator (PAP) flaps in intraoral reconstruction. We report two cases of squamous cell carcinoma involving the tongue and floor of the mouth, where one patient had advanced scleroderma with tight forearm skin and the other with a history of Reynaud's disease precluding the use of RFFF. In addition, both patients were morbidly obese with thick adipose tissue in the thigh making ALT flap not a suitable option. Instead, a PAP flap was chosen. After the harvest, the subcutaneous tissue thickness was measured to be 2.2 and 1.8 cm, respectively. The thinning was performed by removing the deep fat lobules of the superficial fat layer down to a final thickness of 0.4 and 0.3 cm, respectively. A 2 × 2 cm area surrounding the perforators were kept untouched. Both patients had uneventful postoperative course with one patient having a small donor area dehiscence that healed with local wound care. The functional outcomes at 1 year were good. "Thinned" PAP flap is a unique and novel application that may be an alternative in intraoral reconstruction when primary choices are not available. CI - © 2014 Wiley Periodicals, Inc. FAU - Acartürk, Tahsin Oğuz AU - Acartürk TO AD - Department of Plastic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA. FAU - Maldonado, Andrés A AU - Maldonado AA AD - Department of Plastic Surgery and Burns, University Hospital of Getafe, Madrid, Spain. FAU - Ereso, Alex AU - Ereso A AD - Department of Plastic Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA. LA - eng PT - Case Reports PT - Journal Article DEP - 20141220 PL - United States TA - Microsurgery JT - Microsurgery JID - 8309230 SB - IM MH - Aged MH - Carcinoma, Squamous Cell/complications/*surgery MH - Female MH - Humans MH - Leg/*blood supply/surgery MH - Middle Aged MH - Mouth Neoplasms/complications/*surgery MH - Perforator Flap/*blood supply/transplantation MH - Raynaud Disease/*complications MH - Plastic Surgery Procedures/*methods MH - Scleroderma, Systemic/*complications MH - Tongue Neoplasms/complications/surgery EDAT- 2014/12/23 06:00 MHDA- 2016/04/20 06:00 CRDT- 2014/12/23 06:00 PHST- 2013/12/14 00:00 [received] PHST- 2014/11/30 00:00 [revised] PHST- 2014/12/02 00:00 [accepted] PHST- 2014/12/23 06:00 [entrez] PHST- 2014/12/23 06:00 [pubmed] PHST- 2016/04/20 06:00 [medline] AID - 10.1002/micr.22366 [doi] PST - ppublish SO - Microsurgery. 2015 Jul;35(5):399-402. doi: 10.1002/micr.22366. Epub 2014 Dec 20. PMID- 20159671 OWN - NLM STAT- MEDLINE DCOM- 20100407 LR - 20211020 IS - 0949-2321 (Print) IS - 2047-783X (Electronic) IS - 0949-2321 (Linking) VI - 15 IP - 1 DP - 2010 Jan 29 TI - Absolute count of T and B lymphocyte subsets is decreased in systemic sclerosis. PG - 44-6 AB - BACKGROUND: Previous reports on lymphocyte subpopulations in systemic sclerosis (SSc) are conflicting. Therefore, we aimed to investigate the lymphocyte subsets in SSc patients who were not on immunosuppressive therapy. METHODS: Lymphocyte subsets were assessed in the peripheral blood of SSc patients (n = 29) and healthy controls (n = 29) using the four colour flow cytometry method. Correlation studies were also performed in order to assess the relationship between lymphocyte subsets and clinical parameters. RESULTS: The absolute count of lymphocytes (P = 0.0042), CD3+ (P = 0.0014), CD4+ (P = 0.0070), CD8+ (P = 0.021), and CD19+ cells (P = 0.024) was significantly decreased in SSc patients when compared to healthy controls. CD4+/CD8+ ratio and the absolute count of CD56+ cells observed in SSc patients did not significantly differ from controls (P=0.165; P = 0.632, respectively). There was no substantial relationship between the lymphocyte subset levels and clinical features (i.e., SSc subtype, autoantibody profiles, organ involvement), except for a significant inverse correlation of CD19+ cells and the modified Rodnan skin score (r = -0.43, P = 0.020). CONCLUSION: Our data support previous reports indicating that subsets of T lymphocytes as well as B lymphocytes play a role in the pathogenesis of SSc. FAU - Gambichler, Thilo AU - Gambichler T AD - Department of Dermatology, Ruhr-University Bochum, 44791 Bochum, Germany. t.gambichler@klinikum-bochum.de FAU - Tigges, C AU - Tigges C FAU - Burkert, B AU - Burkert B FAU - Höxtermann, S AU - Höxtermann S FAU - Altmeyer, P AU - Altmeyer P FAU - Kreuter, A AU - Kreuter A LA - eng PT - Journal Article PL - England TA - Eur J Med Res JT - European journal of medical research JID - 9517857 SB - IM MH - Adult MH - Aged MH - B-Lymphocyte Subsets/*cytology MH - Female MH - Humans MH - *Lymphocyte Count MH - Male MH - Middle Aged MH - Raynaud Disease/immunology MH - Scleroderma, Systemic/*immunology MH - Severity of Illness Index MH - T-Lymphocyte Subsets/*cytology PMC - PMC3351847 EDAT- 2010/02/18 06:00 MHDA- 2010/04/08 06:00 PMCR- 2010/01/29 CRDT- 2010/02/18 06:00 PHST- 2010/02/18 06:00 [entrez] PHST- 2010/02/18 06:00 [pubmed] PHST- 2010/04/08 06:00 [medline] PHST- 2010/01/29 00:00 [pmc-release] AID - 2047-783X-15-1-44 [pii] AID - 10.1186/2047-783x-15-1-44 [doi] PST - ppublish SO - Eur J Med Res. 2010 Jan 29;15(1):44-6. doi: 10.1186/2047-783x-15-1-44. PMID- 18937632 OWN - NLM STAT- MEDLINE DCOM- 20090114 LR - 20081021 IS - 1936-9719 (Print) IS - 1936-9719 (Linking) VI - 66 IP - 3 DP - 2008 TI - Systemic sclerosis: an update. PG - 198-202 AB - Systemic sclerosis (SSc) is a complex and heterogeneous chronic illness characterized by substantial patient to patient variability in clinical manifestations, internal organ involvement, and outcome. Genetic factors contribute to disease susceptibility, but environmental influences also play a significant role. The pathogenesis of SSc encompasses vascular, immunological, and fibrotic processes, which contribute to clinical manifestations and morbidity and must be addressed in the treatment plan. Although vascular interventions appear to reduce the frequency and severity of complications, such as scleroderma renal crisis and pulmonary hypertension, current therapies generally target the immune component of SSc in a non-selective fashion and have largely failed as diseases-modifying interventions. Newer insights into the mechanisms underlying autoimmunity, vascular injury and destruction, and particularly tissue fibrosis provide novel potential targets for therapy. Transforming growth factor-ss is a ubiquitous cytokine that appears to contribute to fibroblast activation, collagen overproduction, and pathological tissue fibrosis. Neutralizing antibodies and small molecules that block TGF-beta activation or function are effective in shutting down TGF-beta signaling and selectively inhibit the progression of fibrosis and may be entering clinical trials for the treatment of SSc. FAU - Varga, John AU - Varga J AD - Northwest University, Feinberg School of Medicine, Chicago, Illinois, USA. j-varga@northwestern.edu LA - eng PT - Journal Article PT - Review PL - United States TA - Bull NYU Hosp Jt Dis JT - Bulletin of the NYU hospital for joint diseases JID - 101300541 RN - 0 (Cardiovascular Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Transforming Growth Factor beta) SB - IM MH - Blood Vessels/pathology MH - Cardiovascular Agents/therapeutic use MH - Fibrosis MH - Genetic Predisposition to Disease MH - Humans MH - Hypertension, Pulmonary/etiology MH - Immunosuppressive Agents/therapeutic use MH - Inflammation/etiology MH - Kidney Diseases/etiology MH - Lung Diseases, Interstitial/etiology MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*complications/drug therapy/genetics/pathology/physiopathology MH - Skin/pathology MH - Transforming Growth Factor beta/metabolism MH - Treatment Outcome RF - 15 EDAT- 2008/10/22 09:00 MHDA- 2009/01/15 09:00 CRDT- 2008/10/22 09:00 PHST- 2008/10/22 09:00 [pubmed] PHST- 2009/01/15 09:00 [medline] PHST- 2008/10/22 09:00 [entrez] PST - ppublish SO - Bull NYU Hosp Jt Dis. 2008;66(3):198-202. PMID- 16717051 OWN - NLM STAT- MEDLINE DCOM- 20070823 LR - 20161124 IS - 0962-7480 (Print) IS - 0962-7480 (Linking) VI - 56 IP - 5 DP - 2006 Aug TI - Hand-arm vibration syndrome with concomitant arterial thrombosis in the hands. PG - 317-21 AB - BACKGROUND: Hand-arm vibration syndrome (HAVS) refers to the vascular, neurological and musculoskeletal effects that may occur in workers with prolonged exposure to vibrating tools. Hypothenar hammer syndrome (HHS) is a lesion of the ulnar artery at the level of the hamate bone secondary to single or repeated episodes of trauma to the hypothenar eminence. The literature suggests that digital arterial thrombosis and HHS may be associated with the use of vibrating tools. AIM: This study will familiarize investigators with the range of vascular abnormalities seen in workers using vibrating tools, and highlight the importance of screening for arterial thrombosis in the hand when assessing hand-arm vibration-exposed patients. METHODS: In the patients referred to our clinic for HAVS assessment, three were identified during the period 2001 to 2004 who had vascular occlusions in the hands in addition to HAVS. In addition to standardized HAVS vascular investigations, all three patients had arteriograms based on a significantly positive Allen's test. RESULTS: All three cases had documented HAVS based on vascular testing. Arteriograms revealed a spectrum of severity of arterial thromboses from severe HHS, to occlusion limited to the digital arteries. CONCLUSION: Our study reports three cases of HAVS with concomitant HHS and/or digital artery thrombosis. These findings support previous reports of an association between HAVS and vascular thrombosis in the hands. Screening for arterial occlusive problems in the hands should be included in the HAVS work up. FAU - Thompson, Aaron AU - Thompson A AD - Aaron.thompson@utoronto.ca FAU - House, Ron AU - House R LA - eng PT - Case Reports PT - Journal Article DEP - 20060522 PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 SB - IM MH - Adult MH - Cumulative Trauma Disorders/*etiology MH - Hand/*blood supply MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/*etiology MH - Radiography MH - Raynaud Disease/etiology MH - Syndrome MH - Thrombosis/diagnostic imaging/*etiology MH - Vibration/*adverse effects EDAT- 2006/05/24 09:00 MHDA- 2007/08/24 09:00 CRDT- 2006/05/24 09:00 PHST- 2006/05/24 09:00 [pubmed] PHST- 2007/08/24 09:00 [medline] PHST- 2006/05/24 09:00 [entrez] AID - kql022 [pii] AID - 10.1093/occmed/kql022 [doi] PST - ppublish SO - Occup Med (Lond). 2006 Aug;56(5):317-21. doi: 10.1093/occmed/kql022. Epub 2006 May 22. PMID- 31203218 OWN - NLM STAT- MEDLINE DCOM- 20210831 LR - 20210831 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 47 IP - 4 DP - 2020 Apr TI - Renal Parenchymal Thickness in Patients with Systemic Sclerosis Is Related to Intrarenal Hemodynamic Variables and Raynaud Renal Phenomenon. PG - 567-571 LID - 10.3899/jrheum.190165 [doi] AB - OBJECTIVE: Renal involvement in systemic sclerosis (SSc) ranges from urinary abnormalities, reduction of glomerular filtration rate, and high renal resistive index, to scleroderma renal crisis. Intrarenal resistance indices are considered markers of renal SSc-associated vasculopathy. The aim of this study is to evaluate renal morphological variables, such as renal length, parenchymal thickness, atrophy index, and renal sinus in patients with SSc and to correlate it with renal function and hemodynamic variables. METHODS: There were 92 patients with SSc and 40 healthy controls (HC) enrolled in this study. Doppler and renal ultrasound (US) including renal length, parenchymal thickness, atrophy index, renal sinus, and intrarenal resistive index were measured in patients with SSc and HC. RESULTS: Renal US showed significant differences between HC and patients with SSc. The renal length (mm; 106.7 ± 5.1 vs 102.3 ± 8.4) and renal sinus (70.7 ± 7.9 vs 65.3 ± 7.7 mm) were significantly (p = 0.001) higher in HC than patients with SSc. The parenchymal thickness was significantly (p = 0.004) higher in HC than patients with SSc (18 ± 3.1 vs 16.3 ± 2.5 mm). Pulsatility index, resistive index, and systolic/diastolic ratio were significantly (p < 0.0001) lower in HC than patients with SSc. The renal length was significantly (p = 0.004) higher in diffuse cutaneous SSc (105 ± 8.4) than in limited cutaneous SSc (99.5 ± 7.5). CONCLUSION: In SSc, kidney involvement is subclinical and is related to vascular injury, Raynaud phenomenon, and chronic hypoxia that can modify renal morphology. Serum creatinine is a poor marker of renal damage, and renal US could be a useful tool - together with Doppler - to evaluate renal involvement in a systemic and chronic disease such as SSc. FAU - Gigante, Antonietta AU - Gigante A AUID- ORCID: 0000-0002-2015-765X AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Barbano, Biagio AU - Barbano B AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Gasperini, Maria Ludovica AU - Gasperini ML AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Zingaretti, Viviana AU - Zingaretti V AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Cianci, Rosario AU - Cianci R AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Rosato, Edoardo AU - Rosato E AUID- ORCID: 0000-0002-7417-8093 AD - From the Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. edoardo.rosato@uniroma1.it. AD - A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; B. Barbano, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; M.L. Gasperini, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; V. Zingaretti, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; R. Cianci, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; E. Rosato, PhD, Department of Translational and Precision Medicine, Sapienza University of Rome. edoardo.rosato@uniroma1.it. LA - eng PT - Journal Article DEP - 20190615 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2020 Apr;47(4):486-489. doi: 10.3899/jrheum.190930. PMID: 32238542 MH - Glomerular Filtration Rate MH - Hemodynamics MH - Humans MH - Kidney/diagnostic imaging/physiology MH - *Raynaud Disease MH - *Scleroderma, Systemic/complications/diagnostic imaging OTO - NOTNLM OT - ATROPHIC INDEX OT - CORTICAL THICKNESS OT - RENAL LENGTH OT - RENAL RESISTIVE INDEX OT - RENAL ULTRASONOGRAPHY OT - SYSTEMIC SCLEROSIS EDAT- 2019/06/17 06:00 MHDA- 2021/09/01 06:00 CRDT- 2019/06/17 06:00 PHST- 2019/05/30 00:00 [accepted] PHST- 2019/06/17 06:00 [pubmed] PHST- 2021/09/01 06:00 [medline] PHST- 2019/06/17 06:00 [entrez] AID - jrheum.190165 [pii] AID - 10.3899/jrheum.190165 [doi] PST - ppublish SO - J Rheumatol. 2020 Apr;47(4):567-571. doi: 10.3899/jrheum.190165. Epub 2019 Jun 15. PMID- 22089061 OWN - NLM STAT- MEDLINE DCOM- 20120913 LR - 20221207 IS - 1885-1398 (Electronic) IS - 1699-258X (Linking) VI - 8 IP - 2 DP - 2012 Mar-Apr TI - Cryoglobulinemia with acronecrosis not associated with hepatitis C infection: a case report. PG - 84-6 LID - 10.1016/j.reuma.2011.06.006 [doi] AB - Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation. CI - Copyright © 2011 Elsevier España, S.L. All rights reserved. FAU - Ramírez Huaranga, Marco A AU - Ramírez Huaranga MA AD - Reumatología, Hospital General de Ciudad Real, Ciudad Real, Spain. hurauma@hotmail.com FAU - Ramos Rodríguez, Claudia C AU - Ramos Rodríguez CC FAU - Bellido Pastrana, David AU - Bellido Pastrana D LA - eng PT - Case Reports PT - Journal Article DEP - 20111103 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) RN - 4F4X42SYQ6 (Rituximab) RN - 8N3DW7272P (Cyclophosphamide) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adrenal Cortex Hormones/administration & dosage/therapeutic use MH - Amputation, Surgical MH - Antibodies, Monoclonal, Murine-Derived/therapeutic use MH - Combined Modality Therapy MH - Cryoglobulinemia/*complications/drug therapy/pathology/surgery/therapy MH - Cyclophosphamide/administration & dosage/therapeutic use MH - Diabetes Mellitus, Type 2/complications MH - Drug Resistance MH - Drug Therapy, Combination MH - Female MH - Hepatitis C MH - Humans MH - Hypertension/complications MH - Immunosuppressive Agents/administration & dosage/therapeutic use MH - Ischemia/drug therapy/*etiology/surgery MH - Methylprednisolone/administration & dosage/therapeutic use MH - Middle Aged MH - Necrosis MH - Paresthesia/drug therapy/etiology MH - Plasmapheresis MH - Raynaud Disease/etiology MH - Rituximab MH - Toes/*blood supply/pathology/surgery MH - Vasodilator Agents/administration & dosage/therapeutic use EDAT- 2011/11/18 06:00 MHDA- 2012/09/14 06:00 CRDT- 2011/11/18 06:00 PHST- 2011/02/24 00:00 [received] PHST- 2011/05/16 00:00 [revised] PHST- 2011/06/14 00:00 [accepted] PHST- 2011/11/18 06:00 [entrez] PHST- 2011/11/18 06:00 [pubmed] PHST- 2012/09/14 06:00 [medline] AID - S1699-258X(11)00252-X [pii] AID - 10.1016/j.reuma.2011.06.006 [doi] PST - ppublish SO - Reumatol Clin. 2012 Mar-Apr;8(2):84-6. doi: 10.1016/j.reuma.2011.06.006. Epub 2011 Nov 3. PMID- 40617586 OWN - NLM STAT- MEDLINE DCOM- 20250705 LR - 20260513 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 11 IP - 3 DP - 2025 Jul 5 TI - Differences in nailfold capillaroscopy findings between limited and diffuse cutaneous systemic sclerosis: a detailed analysis. LID - 10.1136/rmdopen-2025-005716 [doi] LID - e005716 AB - OBJECTIVE: To investigate and distinguish detailed nailfold videocapillaroscopy (NVC) findings in patients with limited (lcSSc) and diffuse (dcSSc) cutaneous systemic sclerosis (SSc). METHODS: A total of 157 patients was recruited, 100 with lcSSc, 27 with dcSSc and 30 with primary Raynaud phenomenon (pRP). The NVC SSc pattern and the absolute number of capillaries (per linear millimetre) were performed at the first NVC analysis. 'Early'/'Active' NVC status (capillary dilations, microhaemorrhages and giant capillaries) and 'Late' NVC status (number of capillaries, altered microvascular architecture and abnormal capillary shapes) were scored. RESULTS: A statistically significant difference in the absolute number of capillaries between patients with lcSSc, dcSSc and pRP was found (p<0.001). Capillary number loss was present in both SSc subgroups and it was significantly higher in patients with dcSSc compared with lcSSc (4.89±1.53 vs 6.18±1.75, p<0.001). A significantly higher 'Late' NVC status score was observed in patients with dcSSc (p<0.001), including lower capillary density (p<0.001), altered shapes (p<0.001) and presence of abnormal shapes (p=0.005). Correlations showed that higher modified Rodnan Skin Score is associated with decreased capillary number and higher 'Late' NVC status score (p<0.001). Additionally, a statistically significant association was established between 'Late' SSc pattern and dcSSc (p=0.004) and between 'Early' SSc pattern and lcSSc (p=0.010). The absolute capillary number was normal and significantly higher in patients with pRP (p<0.001) than in all patients with SSc. CONCLUSIONS: The current investigation underlines the importance of NVC detailed analysis and scoring in discriminating the severity of microvascular damage between lcSSc and dcSSc. CI - © World Health Organization 2025. Licensee BMJ. FAU - Correia, Ana Margarida AU - Correia AM AD - Rheumatology Department, Unidade Local de Saúde de Braga, Braga, Portugal. FAU - Campitiello, Rosanna AU - Campitiello R AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy. AD - Department of Experimental Medicine (DIMES), University of Genoa, Genoa, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AUID- ORCID: 0000-0002-4732-0306 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy. AD - IRCCS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Rheumatology and Internal Medicine, Ghent University, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, VIB, Ghent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Medical Specialties (DiMI), University of Genoa, Genoa, Italy mcutolo@unige.it. AD - IRCCS Ospedale Policlinico San Martino, Genoa, Italy. LA - eng PT - Journal Article DEP - 20250705 PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Male MH - Female MH - Middle Aged MH - Capillaries/pathology/diagnostic imaging MH - *Nails/blood supply/pathology MH - Adult MH - *Scleroderma, Diffuse/diagnosis/pathology MH - Aged MH - Raynaud Disease/diagnosis MH - *Scleroderma, Limited/diagnosis/pathology MH - Scleroderma, Systemic PMC - PMC12228432 OTO - NOTNLM OT - Autoimmunity OT - Connective Tissue Diseases OT - Microcirculation OT - Scleroderma, Systemic COIS- Competing interests: None declared. EDAT- 2025/07/06 06:18 MHDA- 2025/07/06 06:19 PMCR- 2025/07/05 CRDT- 2025/07/05 20:33 PHST- 2025/03/24 00:00 [received] PHST- 2025/06/23 00:00 [accepted] PHST- 2025/07/06 06:19 [medline] PHST- 2025/07/06 06:18 [pubmed] PHST- 2025/07/05 20:33 [entrez] PHST- 2025/07/05 00:00 [pmc-release] AID - rmdopen-2025-005716 [pii] AID - 10.1136/rmdopen-2025-005716 [doi] PST - epublish SO - RMD Open. 2025 Jul 5;11(3):e005716. doi: 10.1136/rmdopen-2025-005716. PMID- 30961944 OWN - NLM STAT- MEDLINE DCOM- 20200501 LR - 20200501 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 150 DP - 2019 Apr TI - Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients. PG - 154-160 LID - S0954-6111(19)30086-1 [pii] LID - 10.1016/j.rmed.2019.03.011 [doi] AB - BACKGROUND: The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. This cohort is then compared to a group of patients with Idiopathic Pulmonary Fibrosis (IPF). MATERIAL AND METHODS: From 626 consecutive ILD patients evaluated, 45 IPAF and a comparison cohort of 143 IPF patients were enrolled. All patients underwent clinical assessment with rheumatologic and respiratory evaluation, HRCT, Pulmonary Function Tests and Nailfold Videocapillaroscopy. RESULTS: The IPAF patients had a predominance of female gender (62.12%) with a median age of 66 years. The most common findings were: Nonspecific Interstitial Pneumonia (NSIP, 68.89%), Antinuclear Antibody positivity (17.77%) and Raynaud Phenomenon (31.11%). In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O(2) support and predominance of female sex and NSIP pattern. DISCUSSION: This is the first report of a prospective cohort of IPAF patients. IPAF patients seem to have a less severe lung disease than IPF. IPAF criteria probably need to be revisited and validated, but their capacity to recruit patients with incomplete forms or early onset of CTD could be useful for further research. CI - Copyright © 2019 Elsevier Ltd. All rights reserved. FAU - Sambataro, Gianluca AU - Sambataro G AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy; Artroreuma S.R.L., Rheumatology Outpatient registered with the National Health System, Corso S. Vito 53, 95030, Mascalucia, CT, Italy. Electronic address: dottorsambataro@gmail.com. FAU - Sambataro, Domenico AU - Sambataro D AD - Artroreuma S.R.L., Rheumatology Outpatient registered with the National Health System, Corso S. Vito 53, 95030, Mascalucia, CT, Italy; Department of Clinical and Experimental Medicine, Internal Medicine Unit, Section of Rheumatology, Cannizzaro Hospital, University ofCatania, Via Messina 829, 95126, Catania, Italy. FAU - Torrisi, Sebastiano Emanuele AU - Torrisi SE AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Vancheri, Ada AU - Vancheri A AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Colaci, Michele AU - Colaci M AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Section of Rheumatology, Cannizzaro Hospital, University ofCatania, Via Messina 829, 95126, Catania, Italy. FAU - Pavone, Mauro AU - Pavone M AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Pignataro, Francesca AU - Pignataro F AD - Day Hospital of Rheumatology, Dept. of Rheumatology, ASST G. Pini-CTO, Milan, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AD - Day Hospital of Rheumatology, Dept. of Rheumatology, ASST G. Pini-CTO, Milan, Italy. FAU - Palmucci, Stefano AU - Palmucci S AD - Department of Radiology, "Policlinico-Vittorio Emanuele", University of Catania, Italy. FAU - Vancheri, Carlo AU - Vancheri C AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190325 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Aged MH - Antibodies, Antinuclear/immunology MH - Autoimmune Diseases/immunology MH - Connective Tissue Diseases/classification/epidemiology MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/blood/*diagnostic imaging/*immunology/physiopathology MH - Lung Diseases, Interstitial/blood/*diagnostic imaging/*immunology/physiopathology MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Prospective Studies MH - Radiography/methods MH - Raynaud Disease/epidemiology MH - Respiratory Function Tests/methods MH - Tomography, X-Ray Computed/methods OTO - NOTNLM OT - Classification criteria OT - Diagnosis OT - Idiopathic pulmonary fibrosis OT - Interstitial Pneumonia with autoimmune features OT - Interstitial lung disease OT - Undifferentiated connective tissue disease EDAT- 2019/04/10 06:00 MHDA- 2020/05/02 06:00 CRDT- 2019/04/10 06:00 PHST- 2018/11/18 00:00 [received] PHST- 2019/03/18 00:00 [revised] PHST- 2019/03/20 00:00 [accepted] PHST- 2019/04/10 06:00 [entrez] PHST- 2019/04/10 06:00 [pubmed] PHST- 2020/05/02 06:00 [medline] AID - S0954-6111(19)30086-1 [pii] AID - 10.1016/j.rmed.2019.03.011 [doi] PST - ppublish SO - Respir Med. 2019 Apr;150:154-160. doi: 10.1016/j.rmed.2019.03.011. Epub 2019 Mar 25. PMID- 40489831 OWN - NLM STAT- MEDLINE DCOM- 20250609 LR - 20250630 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 104 IP - 23 DP - 2025 Jun 6 TI - Correlation study between clinical phenotypes and autoantibodies in systemic sclerosis in Southwest China. PG - e42639 LID - 10.1097/MD.0000000000042639 [doi] LID - e42639 AB - This study investigates the characteristics of baseline data and the correlation between autoantibodies and clinical phenotypes in patients with systemic sclerosis (SSc) from Southwest China. A total of 346 patients diagnosed with SSc between 2021 and 2023 were included. Clinical data and laboratory data were collected to analyze the relationship between autoantibodies and clinical phenotypes. The prevalence of hypertension and osteoporosis in female patients with SSc was significantly higher than that in male patients. Anti-Scl-70 antibody was positively correlated with Raynaud phenomenon, facial or acral swelling and interstitial lung disease. Anti-Ro52 antibody is positively correlated with pulmonary hypertension. Anti-Scl-70, anti-U1-nrNP, anti-SSA, and anti-Ro52 were associated with overlap syndrome of SSc and systemic lupus erythematosus. Anti-SSA and anti-Ro52 are associated with overlap syndrome of SSc and Sjogren syndrome. Anti-Scl-70 was found to be statistically different in platelet count (PLT), C3, C4, albumin, and C-reactive protein, as was anti-U1-nRNP in hemoglobin, immunoglobulin G, C3, total triglyceride, high-density lipoprotein and low-density lipoprotein, as was anti-SSA in hemoglobin, immunoglobulin G and erythrocyte sedimentation rate, as was anti-ro52 in white blood cell count (WBC) and IgG, and anti-CENP B in PLT. Our study showed that autoantibodies anti-Scl-70 and anti-Ro52 in SSc patients were associated with the clinical phenotypes of Raynaud phenomenon, facial or acral swelling, interstitial lung disease and pulmonary hypertension. CI - Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Sui, Jingzhe AU - Sui J AUID- ORCID: 0000-0001-9822-558 AD - Department of Clinical Laboratory, The First Affiliated Hospital of Guangxi Medical University, Key Laboratory of Clinical Laboratory Medicine of Guangxi Department of Education, Nanning, Guangxi, People's Republic of China. FAU - Li, Haiwei AU - Li H FAU - Liu, Yanqiong AU - Liu Y FAU - Chen, Zhijian AU - Chen Z FAU - Chen, Dan AU - Chen D FAU - Mo, Cuiju AU - Mo C FAU - Huang, Li AU - Huang L FAU - Li, Xi AU - Li X AUID- ORCID: 0000-0002-1981-9273 LA - eng GR - Z-A20220436/Scientific Research Projects of Health Commission of Guangxi Zhuang autonomous region/ PT - Journal Article PT - Observational Study PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Female MH - Male MH - *Scleroderma, Systemic/immunology/epidemiology/blood MH - China/epidemiology MH - *Autoantibodies/blood/immunology MH - Middle Aged MH - Phenotype MH - Adult MH - Aged MH - Raynaud Disease MH - Osteoporosis/epidemiology MH - Lung Diseases, Interstitial/epidemiology MH - Hypertension/epidemiology PMC - PMC12150969 OTO - NOTNLM OT - autoantibodies OT - baseline data OT - clinical phenotypes OT - systemic sclerosis COIS- The authors have no conflicts of interest to disclose. EDAT- 2025/06/09 18:28 MHDA- 2025/06/09 18:29 PMCR- 2025/06/06 CRDT- 2025/06/09 17:13 PHST- 2025/06/09 18:29 [medline] PHST- 2025/06/09 18:28 [pubmed] PHST- 2025/06/09 17:13 [entrez] PHST- 2025/06/06 00:00 [pmc-release] AID - 00005792-202506060-00030 [pii] AID - MD-D-24-15125 [pii] AID - 10.1097/MD.0000000000042639 [doi] PST - ppublish SO - Medicine (Baltimore). 2025 Jun 6;104(23):e42639. doi: 10.1097/MD.0000000000042639. PMID- 26142117 OWN - NLM STAT- MEDLINE DCOM- 20160621 LR - 20150815 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 101 DP - 2015 Sep TI - Short-term follow-up of digital ulcers by laser speckle contrast analysis in systemic sclerosis patients. PG - 82-5 LID - S0026-2862(15)30004-2 [pii] LID - 10.1016/j.mvr.2015.06.009 [doi] AB - OBJECTIVE: To monitor in systemic sclerosis (SSc) the evolution of digital ulcer (DU) status by analysing blood perfusion (BP) using laser speckle contrast analysis (LASCA). METHODS: Hand BP was recorded by LASCA in twenty SSc patients with recent onset fingertip DUs before and after 10days of local/systemic treatment. Regions of interest (ROIs) to analyse BP were created al the level of ulcer, peri-ulcer, periungual and fingertip areas. Visual analogue pain scale (VAS) was also administered to patients before and after follow-up. RESULTS: A statistically significant increase of BP was observed from T0 to T1 in the ROIs created at the level of the ulcer area (p<0.0001), as well as a significant decrease of BP was observed in the peri-ulcer area (p<0.0001). A statistically significant decrease of both ulcer size (p<0.0001) and VAS (p=0.001) was observed, whereas no significant variation of both periungual and fingertip BP was detected. CONCLUSIONS: LASCA may safely monitor DU evolution in SSc patients, by evaluating its blood perfusion and area during standard treatment. This may be useful to monitor DU evolution during treatment in clinical trials. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Ruaro, Barbara AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy. Electronic address: mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20150630 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Aged MH - Clinical Trials as Topic MH - Female MH - Fingers/pathology MH - Follow-Up Studies MH - Hand/*pathology MH - Humans MH - Lasers MH - Male MH - Middle Aged MH - Pain Measurement MH - Perfusion MH - Raynaud Disease/pathology MH - Scleroderma, Systemic/*pathology MH - Skin Ulcer/*diagnosis/pathology MH - Ulcer/pathology OTO - NOTNLM OT - Blood perfusion OT - Digital ulcers OT - Laser speckle contrast analysis OT - Microcirculation OT - Systemic sclerosis EDAT- 2015/07/05 06:00 MHDA- 2016/06/22 06:00 CRDT- 2015/07/05 06:00 PHST- 2015/03/04 00:00 [received] PHST- 2015/06/26 00:00 [revised] PHST- 2015/06/26 00:00 [accepted] PHST- 2015/07/05 06:00 [entrez] PHST- 2015/07/05 06:00 [pubmed] PHST- 2016/06/22 06:00 [medline] AID - S0026-2862(15)30004-2 [pii] AID - 10.1016/j.mvr.2015.06.009 [doi] PST - ppublish SO - Microvasc Res. 2015 Sep;101:82-5. doi: 10.1016/j.mvr.2015.06.009. Epub 2015 Jun 30. PMID- 21698702 OWN - NLM STAT- MEDLINE DCOM- 20111104 LR - 20250529 IS - 1522-2586 (Electronic) IS - 1053-1807 (Print) IS - 1053-1807 (Linking) VI - 34 IP - 1 DP - 2011 Jul TI - High temporal and spatial resolution 3D time-resolved contrast-enhanced magnetic resonance angiography of the hands and feet. PG - 2-12 LID - 10.1002/jmri.22469 [doi] AB - Methods are described for generating 3D time-resolved contrast-enhanced magnetic resonance (MR) angiograms of the hands and feet. Given targeted spatial resolution and frame times, it is shown that acceleration of about one order of magnitude or more is necessary. This is obtained by a combination of 2D sensitivity encoding (SENSE) and homodyne (HD) acceleration methods. Image update times from 3.4-6.8 seconds are provided in conjunction with view sharing. Modular receiver coil arrays are described which can be designed to the targeted vascular region. Images representative of the technique are generated in the vasculature of the hands and feet in volunteers and in patient studies. CI - Copyright © 2011 Wiley-Liss, Inc. FAU - Haider, Clifton R AU - Haider CR AD - Department of Radiology, Mayo Clinic, Rochester, Minnesota 55905, USA. FAU - Riederer, Stephen J AU - Riederer SJ FAU - Borisch, Eric A AU - Borisch EA FAU - Glockner, James F AU - Glockner JF FAU - Grimm, Roger C AU - Grimm RC FAU - Hulshizer, Thomas C AU - Hulshizer TC FAU - Macedo, Thanila A AU - Macedo TA FAU - Mostardi, Petrice M AU - Mostardi PM FAU - Rossman, Phillip J AU - Rossman PJ FAU - Vrtiska, Terri J AU - Vrtiska TJ FAU - Young, Phillip M AU - Young PM LA - eng GR - R56 HL070620/HL/NHLBI NIH HHS/United States GR - EB000212/EB/NIBIB NIH HHS/United States GR - C06 RR018898/RR/NCRR NIH HHS/United States GR - RR018898/RR/NCRR NIH HHS/United States GR - HL070620/HL/NHLBI NIH HHS/United States GR - R01 HL070620/HL/NHLBI NIH HHS/United States GR - T32 GM065841/GM/NIGMS NIH HHS/United States GR - R01 EB000212/EB/NIBIB NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review PL - United States TA - J Magn Reson Imaging JT - Journal of magnetic resonance imaging : JMRI JID - 9105850 RN - 0 (Contrast Media) SB - IM MH - Acceleration MH - Algorithms MH - Angiography/*methods MH - Contrast Media/*pharmacology MH - Foot/diagnostic imaging/*pathology MH - Hand/diagnostic imaging/*pathology MH - Humans MH - Image Processing, Computer-Assisted/methods MH - Imaging, Three-Dimensional/*methods MH - Magnetic Resonance Imaging/*methods MH - Raynaud Disease/pathology MH - Time Factors PMC - PMC3122144 MID - NIHMS256131 EDAT- 2011/06/24 06:00 MHDA- 2011/11/05 06:00 PMCR- 2012/07/01 CRDT- 2011/06/24 06:00 PHST- 2011/06/24 06:00 [entrez] PHST- 2011/06/24 06:00 [pubmed] PHST- 2011/11/05 06:00 [medline] PHST- 2012/07/01 00:00 [pmc-release] AID - 10.1002/jmri.22469 [doi] PST - ppublish SO - J Magn Reson Imaging. 2011 Jul;34(1):2-12. doi: 10.1002/jmri.22469. PMID- 22489252 OWN - NLM STAT- MEDLINE DCOM- 20130301 LR - 20220409 IS - 1740-2530 (Electronic) IS - 1740-2522 (Print) IS - 1740-2522 (Linking) VI - 2012 DP - 2012 TI - Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. PG - 854941 LID - 10.1155/2012/854941 [doi] LID - 854941 AB - Pulmonary arterial hypertension (PAH) is commonly associated with connective tissue diseases (CTDs) including systemic sclerosis and systemic lupus erythematosus (SLE). The prevalence of PAH in SLE is estimated to be 0.5% to 17.5%. The pathophysiology of PAH involves multiple mechanisms from vasculitis and in-situ thrombosis to interstitial pulmonary fibrosis which increases pulmonary vascular resistance, potentially leading to right heart failure. Immune and inflammatory mechanisms may play a significant role in the pathogenesis or progression of PAH in patients with CTDs, establishing a role for anti-inflammatory and immunosuppressive therapies. The leading predictors of PAH in SLE are Raynaud phenomenon, anti-U1RNP antibody, and anticardiolipin antibody positivity. The first-line of diagnostic testing for patients with suspected SLE-associated PAH (SLE-aPAH) involves obtaining a Doppler echocardiogram. Once the diagnosis is confirmed by right heart catheterization, SLE-aPAH patients are generally treated with oxygen, anticoagulants, and vasodilators. Although the prognosis and therapeutic responsiveness of these patients have improved with the addition of intensive immunosuppressive therapies, these treatments are still largely unproven. Recent data put the one-year survival rate for SLE-aPAH patients at 94%. Pregnant women are most at risk of dying due to undiagnosed SLE-aPAH, and screening should be considered essential in this population. FAU - Dhala, Atiya AU - Dhala A AD - Department of Medicine, North Bronx Healthcare Network, Jacobi Medical Center and North Central Bronx Hospital, 3424 Kossuth Avenue, Room 9C-01, Bronx, NY 10467, USA. atiya.dhala@nbhn.net LA - eng PT - Journal Article PT - Review DEP - 20120322 PL - Egypt TA - Clin Dev Immunol JT - Clinical & developmental immunology JID - 101183692 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Anti-Inflammatory Agents/administration & dosage/therapeutic use MH - Antibodies, Anticardiolipin/*blood/immunology MH - Biomarkers/blood MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hypertension, Pulmonary/complications/*diagnosis/drug therapy/mortality MH - Immunosuppressive Agents/administration & dosage/therapeutic use MH - Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy/mortality MH - Pregnancy MH - Prognosis MH - Raynaud Disease/physiopathology MH - Survival Rate MH - Vasodilator Agents/administration & dosage/therapeutic use PMC - PMC3318206 EDAT- 2012/04/11 06:00 MHDA- 2013/03/02 06:00 PMCR- 2012/03/22 CRDT- 2012/04/11 06:00 PHST- 2011/11/09 00:00 [received] PHST- 2012/01/02 00:00 [accepted] PHST- 2012/04/11 06:00 [entrez] PHST- 2012/04/11 06:00 [pubmed] PHST- 2013/03/02 06:00 [medline] PHST- 2012/03/22 00:00 [pmc-release] AID - 10.1155/2012/854941 [doi] PST - ppublish SO - Clin Dev Immunol. 2012;2012:854941. doi: 10.1155/2012/854941. Epub 2012 Mar 22. PMID- 19567653 OWN - NLM STAT- MEDLINE DCOM- 20091022 LR - 20211020 IS - 1527-1315 (Electronic) IS - 0033-8419 (Print) IS - 0033-8419 (Linking) VI - 252 IP - 3 DP - 2009 Sep TI - Three-dimensional electrocardiographically gated variable flip angle FSE imaging for MR angiography of the hands at 3.0 T: initial experience. PG - 874-81 LID - 10.1148/radiol.2531090290 [doi] AB - After institutional review board approval and informed consent were obtained for this HIPAA-compliant investigation, a three-dimensional electrocardiographically gated variable flip angle (VFA) fast spin-echo magnetic resonance (MR) angiography technique was evaluated as an unenhanced method for imaging hand arteries in 13 subjects (including four patients) at 3.0 T; this included evaluation of vessel visualization with warming and cooling in seven subjects. Examinations were evaluated for image quality and vessel conspicuity. Clear separation of arteries from veins was achieved in all subjects, with excellent vessel conspicuity and depiction of stenoses. Warming improved vessel visualization in healthy volunteers. VFA MR angiography is a high-spatial-resolution technique that enables the assessment of vascular reactivity in response to temperature challenge. FAU - Lim, Ruth P AU - Lim RP AD - Department of Radiology, New York University Langone Medical Center, 530 First Ave, Basement Schwartz Bldg, New York, NY 10016, USA. ruth.lim@nyumc.org FAU - Storey, Pippa AU - Storey P FAU - Atanasova, Iliyana P AU - Atanasova IP FAU - Xu, Jian AU - Xu J FAU - Hecht, Elizabeth M AU - Hecht EM FAU - Babb, James S AU - Babb JS FAU - Stoffel, David R AU - Stoffel DR FAU - Chang, Hugo AU - Chang H FAU - McGorty, Kellyanne AU - McGorty K FAU - Chen, Qun AU - Chen Q FAU - Rusinek, Henry AU - Rusinek H FAU - Belmont, H Michael AU - Belmont HM FAU - Lee, Vivian S AU - Lee VS LA - eng GR - R01 HL092439/HL/NHLBI NIH HHS/United States GR - HL092439/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20090630 PL - United States TA - Radiology JT - Radiology JID - 0401260 SB - IM MH - Adult MH - Aged MH - Blood Flow Velocity MH - Cold Temperature MH - *Electrocardiography MH - Female MH - Hand/*blood supply MH - Heart Rate/physiology MH - Hot Temperature MH - Humans MH - Image Processing, Computer-Assisted MH - *Imaging, Three-Dimensional MH - Logistic Models MH - Magnetic Resonance Angiography/*methods MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/physiopathology MH - Scleroderma, Limited/physiopathology PMC - PMC2734893 EDAT- 2009/07/02 09:00 MHDA- 2009/10/23 06:00 PMCR- 2010/09/01 CRDT- 2009/07/02 09:00 PHST- 2009/07/02 09:00 [entrez] PHST- 2009/07/02 09:00 [pubmed] PHST- 2009/10/23 06:00 [medline] PHST- 2010/09/01 00:00 [pmc-release] AID - 2531090290 [pii] AID - 07068 [pii] AID - 10.1148/radiol.2531090290 [doi] PST - ppublish SO - Radiology. 2009 Sep;252(3):874-81. doi: 10.1148/radiol.2531090290. Epub 2009 Jun 30. PMID- 18084002 OWN - NLM STAT- MEDLINE DCOM- 20080305 LR - 20080122 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 2 DP - 2008 Feb TI - Home parenteral nutrition--an effective and safe long-term therapy for systemic sclerosis-related intestinal failure. PG - 176-9 AB - OBJECTIVES: To examine the outcome in patients with SSc requiring parenteral nutrition (PN), and to compare their clinical characteristics with those of other SSc patients and of patients requiring PN/home parenteral nutrition (HPN) for other conditions. METHODS: Retrospective review of SSc and Intestinal Failure Unit databases at a tertiary referral centre for SSc/national unit for intestinal failure over a 13-yr period. RESULTS: Eight patients with SSc requiring PN during the study period were identified (2 males, 6 females: median age at commencement of PN 51 yrs, range 42-56 yrs). All patients commencing PN had bacterial overgrowth and malabsorption not responding to antibiotic therapy. The median duration of PN therapy in the eight patients was 40 months (range 0.8-192 months). Between them the eight patients had a total of 13,851 catheter-use days and only two line infections (0.14/1000 catheter days), a lower rate of line infection than in other HPN-treated patients at Hope Hospital (0.52/1000 catheter days). Three patients died during the 13-yr period, none of causes related to their PN. Six were unable to manage their HPN regime themselves, mainly because of problems with hand function. CONCLUSIONS: Although patient numbers were small, our findings suggest that HPN can be safely and successfully used long-term in patients with SSc and should be considered for patients unable to maintain their nutritional status because of severe gastrointestinal involvement. Impaired hand function should not preclude SSc patients from receiving HPN: family members or community nurses may be trained in the care of the HPN line. FAU - Brown, M AU - Brown M AD - University of Manchester Rheumatic Diseases Centre, Hope Hospital, Salford M6 8HD, Manchester, UK. FAU - Teubner, A AU - Teubner A FAU - Shaffer, J AU - Shaffer J FAU - Herrick, A L AU - Herrick AL LA - eng PT - Journal Article PT - Multicenter Study DEP - 20071214 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Female MH - Gastrointestinal Diseases/epidemiology/etiology/therapy MH - Humans MH - Intestinal Diseases/*etiology/*therapy MH - Male MH - Middle Aged MH - Nutritional Status MH - *Parenteral Nutrition, Home MH - Raynaud Disease/therapy MH - Retrospective Studies MH - Safety MH - Scleroderma, Systemic/*complications MH - Treatment Outcome EDAT- 2007/12/18 09:00 MHDA- 2008/03/06 09:00 CRDT- 2007/12/18 09:00 PHST- 2007/12/18 09:00 [pubmed] PHST- 2008/03/06 09:00 [medline] PHST- 2007/12/18 09:00 [entrez] AID - kem329 [pii] AID - 10.1093/rheumatology/kem329 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Feb;47(2):176-9. doi: 10.1093/rheumatology/kem329. Epub 2007 Dec 14. PMID- 18766120 OWN - NLM STAT- MEDLINE DCOM- 20090325 LR - 20080903 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 14 IP - 4 DP - 2008 Aug TI - Health-related quality of life in primary Raynaud phenomenon. PG - 206-10 LID - 10.1097/RHU.0b013e31817a2485 [doi] AB - OBJECTIVE: To assess health-related quality of life (HRQL) in patients with primary Raynaud phenomenon (RP). METHODS: The Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) questionnaire and the EQ-5D questionnaire were administered to 81 patients with primary RP who had been referred to a rheumatological department. The scores were compared with data of control subjects. RESULTS: In the physical dimensions of the SF-36, the subjects who had RP had significantly lower scores (worse HRQL) than controls (47.6 +/- 9.8 vs. 52.2 +/- 9.3, P = 0.021). The physical component score (PCS) was correlated with age (P = 0.003) and the number of comorbidities (P = 0.004). The mental health component score (MCS) was significantly lower in RP subjects than in controls (41.9 +/- 9.4 vs. 46.1 +/- 10.7, P = 0.006). As regards anxiety/depression (of the EQ-5DA/D), considerably more RP patients (77.7%) reported at least some related problems as compared with controls (60.0%) (chi = 7.93; P = 0.005). Fifty-two RP patients (64.2%) and 178 controls (57.8%) described themselves as moderately anxious/depressed, whereas 11 RP patients (13.5%), and 7 controls (2.2%) reported extreme anxiety/depression (P < 0.0001). CONCLUSIONS: This study shows a reduction of the HRQL in patients with primary RP, compared with control subjects. The psychologic wellbeing represents the most involved area, whereas physical functioning and symptoms are significantly influenced by age and comorbidities. Moreover, these patients are more anxious/depressed (EQ-5DA/D). Longitudinal studies are needed to validate our findings, to explore the causal relationship between primary RP and emotional factors, and to possibly lead to encouraging behavioral therapies. FAU - De Angelis, Rossella AU - De Angelis R AD - Dipartimento di Patologia Molecolare e Terapie Innovative, Cattedra di Reumatologia, Università Politecnica delle Marche, Italy. rossella.deangelis@asl5.marche.it FAU - Salaffi, Fausto AU - Salaffi F FAU - Grassi, Walter AU - Grassi W LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - *Health Surveys MH - Humans MH - Male MH - Middle Aged MH - Pain Measurement MH - *Quality of Life MH - *Raynaud Disease EDAT- 2008/09/04 09:00 MHDA- 2009/03/26 09:00 CRDT- 2008/09/04 09:00 PHST- 2008/09/04 09:00 [pubmed] PHST- 2009/03/26 09:00 [medline] PHST- 2008/09/04 09:00 [entrez] AID - 00124743-200808000-00003 [pii] AID - 10.1097/RHU.0b013e31817a2485 [doi] PST - ppublish SO - J Clin Rheumatol. 2008 Aug;14(4):206-10. doi: 10.1097/RHU.0b013e31817a2485. PMID- 22190689 OWN - NLM STAT- MEDLINE DCOM- 20120508 LR - 20121120 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 51 IP - 4 DP - 2012 Apr TI - A multicentre study on the reliability of qualitative and quantitative nail-fold videocapillaroscopy assessment. PG - 749-55 LID - 10.1093/rheumatology/ker403 [doi] AB - OBJECTIVE: To investigate the inter- and intra-observer reliability of both qualitative and quantitative parameters used in the assessment of nail-fold capillaroscopy images. METHODS: Fifty mosaic nail-fold images of healthy controls (n = 10), patients with primary RP (n = 10) and SSc (n = 30) were assessed in random order by two blinded observers on two occasions at centres in Sweden, UK and The Netherlands. Each image was therefore scored by six observers twice. RESULTS: Inter- and intra-observer reliability of quantitative parameters showed substantial to almost perfect agreement [inter- and intra-observer weighted κ's for the number of widened capillaries was 0.75 and 0.87 and giant capillaries was 0.84 and 0.92, intra-class correlation coefficients (ICCs) for capillary density was 0.87 and 0.92 and total loop width was 0.94 and 0.98, respectively]. Qualitative parameters including architecture, avascularity, haemorrhage, crossed, ramified and bushy capillaries showed moderate to substantial inter-observer reproducibility (weighted κ ranging from 0.47 to 0.73), and substantial intra-observer repeatability (weighted κ ranging from 0.71 to 0.80), whereas the scoring of tortuous and bizarre capillaries showed poor inter-observer and substantial intra-observer agreement (inter-observer weighted κ's was 0.39 and 0.21 and intra-observer weighted κ's was 0.68 and 0.76, respectively). CONCLUSION: All quantitative and certain qualitative parameters are highly reliable in terms of inter- and intra-observer agreement. A combination of parameters with the highest reliability should be incorporated into future capillaroscopic scoring systems in studies of prediction and monitoring of SSc spectrum disorders. FAU - Hofstee, Herman M A AU - Hofstee HM AD - Department of Internal Medicine, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands. hma.hofstee@vumc.nl FAU - Serné, Erik H AU - Serné EH FAU - Roberts, Christopher AU - Roberts C FAU - Hesselstrand, Roger AU - Hesselstrand R FAU - Scheja, Agneta AU - Scheja A FAU - Moore, Tonia L AU - Moore TL FAU - Wildt, Marie AU - Wildt M FAU - Manning, Joanne B AU - Manning JB FAU - Vonk Noordegraaf, Anton AU - Vonk Noordegraaf A FAU - Voskuyl, Alexandre E AU - Voskuyl AE FAU - Herrick, Ariane L AU - Herrick AL LA - eng PT - Journal Article PT - Multicenter Study DEP - 20111220 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - Rheumatology (Oxford). 2012 Oct;51(10):1921-2; author reply 1922. doi: 10.1093/rheumatology/kes172. PMID: 22772318 MH - Capillaries/pathology MH - Humans MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply MH - Observer Variation MH - Raynaud Disease/*pathology MH - Reproducibility of Results MH - Scleroderma, Systemic/*pathology MH - Single-Blind Method MH - Video Recording EDAT- 2011/12/23 06:00 MHDA- 2012/05/09 06:00 CRDT- 2011/12/23 06:00 PHST- 2011/12/23 06:00 [entrez] PHST- 2011/12/23 06:00 [pubmed] PHST- 2012/05/09 06:00 [medline] AID - ker403 [pii] AID - 10.1093/rheumatology/ker403 [doi] PST - ppublish SO - Rheumatology (Oxford). 2012 Apr;51(4):749-55. doi: 10.1093/rheumatology/ker403. Epub 2011 Dec 20. PMID- 31806155 OWN - NLM STAT- MEDLINE DCOM- 20210427 LR - 20210427 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 50 IP - 3 DP - 2020 Jun TI - Concurrent anti-PM-Scl antibody-associated systemic sclerosis and inclusion body myositis - report of two cases and review of the literature. PG - 498-502 LID - S0049-0172(19)30689-4 [pii] LID - 10.1016/j.semarthrit.2019.11.008 [doi] AB - OBJECTIVES: To describe two cases of anti-PM-Scl antibody-associated systemic sclerosis with evidence of inclusion body myositis on muscle biopsy. METHODS: Two female patients with anti-PM-Scl antibody-associated systemic sclerosis developed progressive proximal myopathy. Both patients had profound muscle weakness that was refractory to treatment with glucocorticoids with or without other oral immunosuppressive agents. Quadriceps muscle biopsy in both cases indicated inclusion body myositis (IBM). Monthly intravenous immunoglobulin (IVIG) infusions were added to the treatment regimen. RESULTS: One patient's myopathy appears to have temporarily stabilized with IVIG infusions (2 g/Kg) every four weeks, though severe residual muscle weakness has persisted. The other patient's myopathy continues to progress slowly, despite being on 4-weekly IVIG infusions, along with weekly oral methotrexate and 6-monthly rituximab infusions. In this report, we compare these two patients with two similar cases that were previously reported and hypothesize a possible pathomechanism of this association. CONCLUSION: This extremely rare association of IBM and anti-PM-Scl antibody-associated scleromyositis sheds new light on the possible pathogenesis of IBM. It strengthens the hypothesis that autoimmune muscle disease can potentially trigger myodegeneration. Whether early intervention with aggressive immunosuppressive therapy can prevent progression to treatment-refractory IBM, should be a subject of future research. CI - Copyright © 2019 Elsevier Inc. All rights reserved. FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195, USA. Electronic address: chattes@ccf.org. FAU - Prayson, Richard A AU - Prayson RA AD - Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH 44195, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20191116 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) SB - IM MH - Disease Progression MH - Female MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage MH - Immunosuppressive Agents/therapeutic use MH - Middle Aged MH - Muscle Weakness/*etiology MH - Myositis, Inclusion Body/*diagnosis/drug therapy MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/*diagnosis/drug therapy OTO - NOTNLM OT - Anti-PM-Scl antibody OT - Dermatomyositis OT - Inclusion body myositis OT - Polymyositis OT - Scleroderma (systemic sclerosis) OT - Scleromyositis COIS- Declaration of competing interest Dr. Soumya Chatterjee and Dr. Richard Prayson both have nothing to disclose. EDAT- 2019/12/07 06:00 MHDA- 2021/04/28 06:00 CRDT- 2019/12/07 06:00 PHST- 2019/05/25 00:00 [received] PHST- 2019/09/08 00:00 [revised] PHST- 2019/11/08 00:00 [accepted] PHST- 2019/12/07 06:00 [pubmed] PHST- 2021/04/28 06:00 [medline] PHST- 2019/12/07 06:00 [entrez] AID - S0049-0172(19)30689-4 [pii] AID - 10.1016/j.semarthrit.2019.11.008 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2020 Jun;50(3):498-502. doi: 10.1016/j.semarthrit.2019.11.008. Epub 2019 Nov 16. PMID- 25794968 OWN - NLM STAT- MEDLINE DCOM- 20160419 LR - 20150508 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 99 DP - 2015 May TI - New insights into systemic sclerosis related microcirculatory dysfunction by assessment of sublingual micr\ocirculation and vascular glycocalyx layer. Results from a preliminary study. PG - 72-7 LID - S0026-2862(15)00029-1 [pii] LID - 10.1016/j.mvr.2015.03.002 [doi] AB - BACKGROUND: Initial morphological and functional markers of systemic sclerosis (SSc) are evidenced in microvascular structural damage. However, nailfold videocapillaroscopy (NVC) explores only morphological abnormalities. Sidestream Dark Field (SDF) imaging of sublingual microcirculation enables assessment of both morphological and functional capillary impairment and allows measurement of the glycocalyx layer, which is an indicator of endothelial dysfunction. OBJECTIVE: To describe and validate sublingual abnormalities assessed by SDF device in comparison with NVC findings and to measure the thickness of the glycocalyx layer. METHODS: From February to May 2014, 26 subjects (16 SSc patients and 10 healthy controls) underwent standardised NVC and SDF imaging of sublingual microcirculation. Glycocalyx thickness was also measured. RESULTS: Capillary density and percentage of perfused vessels were significantly reduced in patients with SSc (n = 13) compared to controls. Correlation between nailfold capillary density assessed by NVC and sublingual capillary density assessed by SDF was observed (r(2) = 0.59; P = 0.023). According to the NVC pattern, patients with "active" disease experienced greater reduction in capillary density than patients with "late" disease as suggested by the de Backer score (9.17 ± 0.81 vs 10.86 ± 1.19; P = 0.03). Additionally, the decrease in glycocalyx thickness was measured in SSc patients (n = 13) compared to controls (n = 10) (0.41 ± 0.03 versus 0.76 ± 0.29 P = 0.003). CONCLUSION: Our results suggest for the first time in SSc, that sublingual microcirculation and glycocalyx are impaired and that SDF imaging findings correlate with those of NVC. Nevertheless, further studies are required for the validation of our preliminary results. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Miranda, S AU - Miranda S AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; Inserm U1096, University of Rouen, France; Institute for Research and Innovation in Biomedicine, University of Rouen, France. FAU - Armengol, G AU - Armengol G AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; Inserm U1096, University of Rouen, France; Institute for Research and Innovation in Biomedicine, University of Rouen, France. FAU - Le Besnerais, M AU - Le Besnerais M AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; Inserm U1096, University of Rouen, France; Institute for Research and Innovation in Biomedicine, University of Rouen, France. FAU - Lévesque, H AU - Lévesque H AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; Inserm U1096, University of Rouen, France; Institute for Research and Innovation in Biomedicine, University of Rouen, France. FAU - Benhamou, Y AU - Benhamou Y AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; Inserm U1096, University of Rouen, France; Institute for Research and Innovation in Biomedicine, University of Rouen, France. Electronic address: ygal.benhamou@chu-rouen.fr. LA - eng PT - Journal Article DEP - 20150317 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - Capillaries/pathology MH - Case-Control Studies MH - Female MH - Glycocalyx/chemistry MH - Humans MH - Male MH - *Microcirculation MH - Microscopic Angioscopy MH - Middle Aged MH - Mouth Floor/*blood supply MH - Nails/blood supply MH - Raynaud Disease/physiopathology MH - Reproducibility of Results MH - Scleroderma, Systemic/*physiopathology MH - Tongue/*blood supply OTO - NOTNLM OT - Endothelial glycocalyx OT - Microcirculation OT - Nailfold videocapillaroscopy OT - SDF imaging OT - Systemic sclerosis EDAT- 2015/03/22 06:00 MHDA- 2016/04/20 06:00 CRDT- 2015/03/22 06:00 PHST- 2014/11/08 00:00 [received] PHST- 2015/03/09 00:00 [revised] PHST- 2015/03/10 00:00 [accepted] PHST- 2015/03/22 06:00 [entrez] PHST- 2015/03/22 06:00 [pubmed] PHST- 2016/04/20 06:00 [medline] AID - S0026-2862(15)00029-1 [pii] AID - 10.1016/j.mvr.2015.03.002 [doi] PST - ppublish SO - Microvasc Res. 2015 May;99:72-7. doi: 10.1016/j.mvr.2015.03.002. Epub 2015 Mar 17. PMID- 22391471 OWN - NLM STAT- MEDLINE DCOM- 20120427 LR - 20210108 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 91 IP - 2 DP - 2012 Mar TI - Inflammatory myopathies with anti-Ku antibodies: a prognosis dependent on associated lung disease. PG - 95-102 LID - 10.1097/MD.0b013e31824d9cec [doi] AB - Anti-Ku antibodies have been reported in a wide spectrum of autoimmune diseases, sometimes in association with inflammatory myopathies (IM). We studied the clinical, laboratory, and muscle histologic features of all anti-Ku-positive patients detected in our hospital during the last 10 years, as well as their treatment and outcomes. Anti-Ku antibodies were found in 34 patients (0.46% of 20,600 sera positive for antinuclear antibodies), and complete data were available for 30 patients; 86.7% were female, mean age was 49 years (range, 20-73 yr). The most frequent clinical manifestations were arthralgia (77%) and Raynaud phenomenon (53%). Eleven (37%) patients had IM, 8 of them as part of an overlap syndrome defined as IM associated with connective autoimmune disease (5 systemic sclerosis [SSc], 2 Sjögren syndrome (SS), and 1 systemic lupus erythematosus [SLE]). Of 21 patients without IM, 19 had autoimmune diseases (including 6 SLE, 2 SSc, 2 SS, and 2 rheumatoid arthritis), 1 had bronchial neoplasia, and 1 had nephroangiosclerosis. Clinical features of the 9 patients with IM were myalgia (91%), proximal muscle weakness (89%), and dysphagia (36%). All had increased creatine kinase (median, 2210 U/L; range, 194-4073 U/L). Muscle biopsy showed necrosis, inflammation, and positive HLA class I immunostaining. Interstitial lung disease (ILD) was detected on computed tomography (CT) scan in 11 patients (37%) and was significantly more frequent in patients with IM (82% vs. 10.5%, p < 0.001). Fourteen (47%) patients required no immunosuppressive treatment or only a low corticosteroid dose (<15 mg/d, n = 3). A high dose of corticosteroids was more frequently administered in patients with IM (10/11 cases, 80% with associated ILD) than in patients without IM (4/19 cases, 0 with ILD). Complete muscle remission after steroids occurred in 73% of patients with IM. Lung disease was corticoresistant in 6 of 8 (75%) treated cases.Anti-Ku antibodies remain rarely detected, but their presence can be frequently associated with corticosensitive IM and severe, corticoresistant ILD. FAU - Rigolet, Aude AU - Rigolet A AD - From the Service de Médecine Interne 1 (AR, SH, OB); Département d'Immunologie (LM, JLC), Neuropathologie (OD, TM), and Radiologie (PG); Centre deRéférence des Pathologies Neuromusculaires Paris Est, Institut de Myologie(AB, SH, OB); Service de Médecine Interne 2 (ZA); Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Paris, France. FAU - Musset, Lucile AU - Musset L FAU - Dubourg, Odile AU - Dubourg O FAU - Maisonobe, Thierry AU - Maisonobe T FAU - Grenier, Philippe AU - Grenier P FAU - Charuel, Jean-Luc AU - Charuel JL FAU - Behin, Anthony AU - Behin A FAU - Herson, Serge AU - Herson S FAU - Amoura, Zahir AU - Amoura Z FAU - Benveniste, Olivier AU - Benveniste O LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) RN - 0 (DNA-Binding Proteins) RN - 0 (Immunosuppressive Agents) RN - EC 2.7.3.2 (Creatine Kinase) RN - EC 3.6.4.12 (Xrcc6 protein, human) RN - EC 4.2.99.- (Ku Autoantigen) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood MH - Antigens, Nuclear/*immunology MH - Arthralgia/etiology MH - Autoantibodies/blood MH - Autoimmune Diseases/complications MH - Biopsy MH - Creatine Kinase/blood MH - DNA-Binding Proteins/*immunology MH - Deglutition Disorders/etiology MH - Drug Resistance MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/drug therapy MH - Immunosuppressive Agents/therapeutic use MH - Ku Autoantigen MH - Lung Diseases, Interstitial/*diagnosis/drug therapy MH - Male MH - Middle Aged MH - Muscle Weakness/etiology MH - Muscle, Skeletal/pathology MH - Myositis/complications/*drug therapy/*immunology MH - Necrosis MH - Prognosis MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Young Adult EDAT- 2012/03/07 06:00 MHDA- 2012/04/28 06:00 CRDT- 2012/03/07 06:00 PHST- 2012/03/07 06:00 [entrez] PHST- 2012/03/07 06:00 [pubmed] PHST- 2012/04/28 06:00 [medline] AID - 00005792-201203000-00004 [pii] AID - 10.1097/MD.0b013e31824d9cec [doi] PST - ppublish SO - Medicine (Baltimore). 2012 Mar;91(2):95-102. doi: 10.1097/MD.0b013e31824d9cec. PMID- 21978705 OWN - NLM STAT- MEDLINE DCOM- 20111202 LR - 20170214 IS - 0394-6320 (Print) IS - 0394-6320 (Linking) VI - 24 IP - 3 DP - 2011 Jul-Sep TI - Long-term N-acetylcysteine therapy in systemic sclerosis interstitial lung disease: a retrospective study. PG - 727-33 AB - Systemic sclerosis (SSc) is associated with interstitial lung diseases. The primary endpoints of this study were changes between baseline and month 24 in single-breath carbon monoxide diffusing capacity (DLco). The secondary endpoints were: vital capacity (VC), forced expired volume in 1 sec (FEV1), total lung capacity (TLC), scores of high resolution computed tomography (HRCT) of the chest, number of adverse effects. In this study, we retrospectively investigated data from SSc patients who had undergone therapy with high-dose intravenous N-acetylcysteine (NAC) at a dosage of 15 mg/Kg/h for 5 consecutive hours every 14 days. After NAC therapy median values of DLco (69.5 vs 77.7%), VC (99 vs 101.3%) and TLC (93 vs 98.3%) significantly increased. We did not observe any significant changes from baseline in FEV1 value and HRTC score. The improvement in lung function was more evident in SSc patients without radiological signs of pulmonary fibrosis than in patients with pulmonary fibrosis. In SSc patients with mild-moderate pulmonary fibrosis intravenous NAC administration slows the rate of deterioration of DLco, VC and TLC. In conclusion, this retrospective study demonstrates that long-term therapy with intravenous NAC ameliorates pulmonary function tests in SSc patients. FAU - Rosato, E AU - Rosato E AD - Sapienza University of Rome, Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center, Rome, Italy. FAU - Rossi, C AU - Rossi C FAU - Molinaro, I AU - Molinaro I FAU - Giovannetti, A AU - Giovannetti A FAU - Pisarri, S AU - Pisarri S FAU - Salsano, Felice AU - Salsano F LA - eng PT - Journal Article PL - England TA - Int J Immunopathol Pharmacol JT - International journal of immunopathology and pharmacology JID - 8911335 RN - 0 (Antioxidants) RN - 0 (Calcium Channel Blockers) RN - I9ZF7L6G2L (Nifedipine) RN - WYQ7N0BPYC (Acetylcysteine) SB - IM MH - Acetylcysteine/*therapeutic use MH - Adult MH - Aged MH - Antioxidants/*therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - Endpoint Determination MH - Female MH - Fingers/pathology MH - Humans MH - Lung/physiopathology MH - Lung Diseases, Interstitial/*drug therapy/physiopathology MH - Male MH - Middle Aged MH - Nifedipine/therapeutic use MH - Pulmonary Fibrosis/pathology MH - Raynaud Disease/drug therapy MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/*drug therapy/physiopathology MH - Total Lung Capacity MH - Treatment Outcome MH - Ulcer/drug therapy/pathology MH - Vital Capacity MH - Young Adult EDAT- 2011/10/08 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/10/08 06:00 PHST- 2011/10/08 06:00 [entrez] PHST- 2011/10/08 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 19 [pii] AID - 10.1177/039463201102400319 [doi] PST - ppublish SO - Int J Immunopathol Pharmacol. 2011 Jul-Sep;24(3):727-33. doi: 10.1177/039463201102400319. PMID- 30221850 OWN - NLM STAT- MEDLINE DCOM- 20191031 LR - 20191031 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 71 IP - 1 DP - 2019 Jan TI - Arthritis Mutilans in Systemic Sclerosis. PG - 120 LID - 10.1002/art.40723 [doi] FAU - Abignano, Giuseppina AU - Abignano G AD - Rheumatology Institute of Lucania (IReL), San Carlo Hospital, Potenza, Italy. AD - LIRMM, University of Leeds, Leeds, UK. FAU - Mennillo, Gianna A AU - Mennillo GA AD - IReL, San Carlo Hospital. FAU - Lettieri, Giovanni AU - Lettieri G AD - Radiology Department, San Giovanni di Dio e Ruggi d'Aragona University Hospital, Salerno, Italy. FAU - Padula, Angela AU - Padula A AD - IReL, San Carlo Hospital. FAU - McGonagle, Dennis AU - McGonagle D AD - LIRMM, University of Leeds and NIHR LBRC, Leeds Teaching Hospitals NHS Trust. FAU - D'Angelo, Salvatore AU - D'Angelo S AD - IReL, San Carlo Hospital. LA - eng PT - Case Reports PT - Journal Article DEP - 20181124 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Acro-Osteolysis/complications/diagnosis/*diagnostic imaging MH - Adult MH - Arthritis/complications/diagnosis/*diagnostic imaging/drug therapy MH - Autoantibodies/immunology MH - Calcinosis/complications/diagnostic imaging MH - Contracture/complications/diagnosis/diagnostic imaging MH - DNA Topoisomerases, Type I/immunology MH - Hand Joints/*diagnostic imaging MH - Humans MH - Immunologic Factors/therapeutic use MH - Lung Diseases, Interstitial/complications/drug therapy MH - Male MH - Radiography MH - Raynaud Disease MH - Rituximab/therapeutic use MH - Scleroderma, Diffuse/complications/*diagnosis/drug therapy/immunology EDAT- 2018/09/18 06:00 MHDA- 2019/11/02 06:00 CRDT- 2018/09/18 06:00 PHST- 2018/09/18 06:00 [pubmed] PHST- 2019/11/02 06:00 [medline] PHST- 2018/09/18 06:00 [entrez] AID - 10.1002/art.40723 [doi] PST - ppublish SO - Arthritis Rheumatol. 2019 Jan;71(1):120. doi: 10.1002/art.40723. Epub 2018 Nov 24. PMID- 23588147 OWN - NLM STAT- MEDLINE DCOM- 20130823 LR - 20151119 IS - 0392-0488 (Print) IS - 0392-0488 (Linking) VI - 148 IP - 2 DP - 2013 Apr TI - Iloprost treatment summer-suspension: effects on skin thermal properties and cytokine profile in systemic sclerosis patients. PG - 209-16 AB - AIM: Aim of the study was to assess whether Iloprost treatment summer suspension modifies systemic cytokines levels, cutaneous thermal properties and functional response to a cold-induced stress in patients affected by systemic sclerosis (SSc). METHODS: Twenty-eight patients fulfilling the American College of Rheumatology (ACR) criteria for SSc were included in the study. Patients recorded number, duration and pain-severity of Raynaud phenomenon (RP). Pain-severity was determined by a visual analog scale. Cytokines expression and production in peripheral blood mononuclear cells and serum were evaluated by RT-PCR and ELISA assay. Basal finger temperature (Tb), distal-dorsal difference temperature (DTdd) and thermal recovery time (tr) from cold stress were measured by means of functional infrared imaging (fIR). Measurements were performed in late spring, during routine Iloprost therapy (1-3 days infusion of 0.5-2 ng/kg every month), and in late summer after a therapy-withdrawal period. RESULTS: Deterioration of SSc patients' skin thermal properties was observed in the period of therapy withdrawal (Tb reduction and tr enhancement; no DTdd differences) despite the improvement in symptoms of RP. A reduction in IL-12/23p40 gene expression was recorded after therapy withdrawal and a direct correlation between IL-12/23p40 and IL-23p19 gene expression was observed, stronger after therapy suspension. CONCLUSION: Our data suggest that Iloprost treatment summer suspension may induce the loss of the therapy beneficial effect on microcirculation despite the objective reduction of RP, thus favouring a continuous use of Iloprost in absence of severe side effects. Iloprost showed to modulate only IL-23 expression corroborating the idea that this cytokine is crucial for SSc development and progression. FAU - Auriemma, M AU - Auriemma M AD - Department of Experimental and Clinical Sciences University "G. D'Annunzio" Chieti-Pescara, Italy. matteo.auriemma@gmail.com FAU - Vianale, G AU - Vianale G FAU - Reale, M AU - Reale M FAU - Costantini, E AU - Costantini E FAU - Di Nicola, M AU - Di Nicola M FAU - Romani, G L AU - Romani GL FAU - Merla, A AU - Merla A FAU - Muraro, R AU - Muraro R FAU - Amerio, P AU - Amerio P LA - eng PT - Journal Article PL - Italy TA - G Ital Dermatol Venereol JT - Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia JID - 8102852 RN - 0 (Adjuvants, Immunologic) RN - 0 (Biomarkers) RN - 0 (Cytokines) RN - 0 (Interleukin-23) RN - 0 (Transforming Growth Factor beta) RN - 0 (Tumor Necrosis Factor-alpha) RN - 0 (Vasodilator Agents) RN - 187348-17-0 (Interleukin-12) RN - JED5K35YGL (Iloprost) SB - IM MH - Adjuvants, Immunologic/blood MH - Aged MH - Biomarkers/blood MH - Cold Temperature/adverse effects MH - Cytokines/*blood/drug effects MH - Female MH - Humans MH - Iloprost/*administration & dosage/adverse effects MH - Interleukin-12/blood MH - Interleukin-23/blood MH - Male MH - Middle Aged MH - Pain Measurement MH - Raynaud Disease/etiology MH - Scleroderma, Systemic/blood/*drug therapy/*immunology MH - Seasons MH - Time Factors MH - Transforming Growth Factor beta/blood MH - Treatment Outcome MH - Tumor Necrosis Factor-alpha/blood MH - Vasodilator Agents/*administration & dosage/adverse effects MH - *Withholding Treatment EDAT- 2013/04/17 06:00 MHDA- 2013/08/24 06:00 CRDT- 2013/04/17 06:00 PHST- 2013/04/17 06:00 [entrez] PHST- 2013/04/17 06:00 [pubmed] PHST- 2013/08/24 06:00 [medline] AID - R23134452 [pii] PST - ppublish SO - G Ital Dermatol Venereol. 2013 Apr;148(2):209-16. PMID- 32576213 OWN - NLM STAT- MEDLINE DCOM- 20230711 LR - 20230711 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 15 IP - 1 DP - 2020 Jun 23 TI - Raising rare disease awareness using red flags, role play simulation and patient educators: results of a novel educational workshop on Raynaud phenomenon and systemic sclerosis. PG - 159 LID - 10.1186/s13023-020-01439-z [doi] LID - 159 AB - BACKGROUND: As lack of awareness of rare diseases (RDs) among healthcare professionals results in delayed diagnoses, there is a need for a more efficient approach to RD training during academic education. We designed an experimental workshop that used role-play simulation with patient educators and focused on teaching "red flags" that should raise the suspicion of an RD when faced with a patient with frequently encountered symptoms. Our objective was to report our experience, and to assess the improvement in learners' knowledge and the satisfaction levels of the participants. RESULTS: The workshop consisted of 2 simulated consultations that both started with the same frequent symptom (Raynaud phenomenon, RP) but led to different diagnoses: a frequent condition (idiopathic RP) and an RD (systemic sclerosis, SSc). In the second simulated consultation, the role of the patient was played by a patient educator with SSc. By juxtaposing 2 seemingly similar situations, the training particularly highlighted the elements that help differentiate SSc from idiopathic RP. When answering a clinical case exam about RP and SSc, students that had participated in the workshop had a higher mean mark than those who had not (14 ± 3.7 vs 9.6 ± 5.5 points out of 20, p = 0.001). Participants mostly felt "very satisfied" with this training (94%), and "more comfortable" about managing idiopathic RP and SSc (100%). They considered the workshop "not very stressful" and "very formative" (both 71%). When asked about the strengths of this training, they mentioned the benefits of being put in an immersive situation, allowing a better acquisition of practical skills and a more interactive exchange with teachers, as well as the confrontation with a real patient, leading to a better retention of semiological findings and associating a relational component with this experience. CONCLUSIONS: Through the use of innovative educational methods, such as role-play simulation and patient educators, and by focusing on teaching "red flags", our workshop successfully improved RP and SSc learning in a way that satisfied students. By modifying the workshop's scenarios, its template can readily be applied to other clinical situations, making it an interesting tool to teach other RDs. FAU - Sanges, S AU - Sanges S AUID- ORCID: 0000-0003-0280-411X AD - Centre de Simulation PRESAGE, Univ. Lille, UFR Médecine, F-59000, Lille, France. sebastien.sanges@univ-lille.fr. AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. sebastien.sanges@univ-lille.fr. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. sebastien.sanges@univ-lille.fr. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. sebastien.sanges@univ-lille.fr. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. sebastien.sanges@univ-lille.fr. FAU - Farhat, M-M AU - Farhat MM AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Assaraf, M AU - Assaraf M AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. FAU - Galland, J AU - Galland J AD - Service de médecine interne, Hôpital Lariboisière, Assistance Publique - Hôpitaux de Paris, F-75010, Paris, France. AD - Université de Paris Diderot, F-75010, Paris, France. FAU - Rivière, E AU - Rivière E AD - Service de médecine interne et maladies infectieuses, CHU de Bordeaux, F-33600, Pessac, France. AD - Centre de simulation SimBA-S de Bordeaux, CHU de Bordeaux et Université de Bordeaux, F-33000, Bordeaux, France. FAU - Roubille, C AU - Roubille C AD - Département de médecine interne, CHU de Montpellier, hôpital Lapeyronie, 371, avenue du Doyen Gaston Giraud, F-34295, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR 9214, Cedex 5, Montpellier, France. FAU - Lambert, M AU - Lambert M AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Yelnik, C AU - Yelnik C AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Maillard, H AU - Maillard H AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Sobanski, V AU - Sobanski V AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Lefèvre, G AU - Lefèvre G AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. AD - CHU Lille, Institut d'Immunologie, F-59000, Lille, France. FAU - Launay, D AU - Launay D AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Morell-Dubois, S AU - Morell-Dubois S AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. FAU - Hachulla, E AU - Hachulla E AD - Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000, Lille, France. AD - Département de Médecine Interne et Immunologie Clinique, CHU Lille, F-59037, Lille Cedex, France. AD - Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Oust de France (CeRAINO), F-59000, Lille, France. AD - Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), Lille, France. LA - eng PT - Journal Article PT - Review DEP - 20200623 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 SB - IM MH - Humans MH - Rare Diseases MH - *Raynaud Disease MH - *Scleroderma, Systemic/diagnosis PMC - PMC7310378 OTO - NOTNLM OT - Big data OT - Medical education OT - Patient educators OT - Rare diseases OT - Raynaud phenomenon OT - Role play OT - Simulated patients OT - Simulation OT - Systemic sclerosis COIS- The authors declare that they have no competing interests in relation to this work. EDAT- 2020/06/25 06:00 MHDA- 2021/06/22 06:00 PMCR- 2020/06/23 CRDT- 2020/06/25 06:00 PHST- 2020/05/03 00:00 [received] PHST- 2020/06/15 00:00 [accepted] PHST- 2020/06/25 06:00 [entrez] PHST- 2020/06/25 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/06/23 00:00 [pmc-release] AID - 10.1186/s13023-020-01439-z [pii] AID - 1439 [pii] AID - 10.1186/s13023-020-01439-z [doi] PST - epublish SO - Orphanet J Rare Dis. 2020 Jun 23;15(1):159. doi: 10.1186/s13023-020-01439-z. PMID- 22371290 OWN - NLM STAT- MEDLINE DCOM- 20130122 LR - 20211021 IS - 1573-2592 (Electronic) IS - 0271-9142 (Linking) VI - 32 IP - 4 DP - 2012 Aug TI - Evaluation of the reactivity of sera from patients with systemic lupus erythematosus against the human MCP1. PG - 721-8 LID - 10.1007/s10875-012-9665-4 [doi] AB - This study evaluates metaphase chromosome protein 1 (MCP1), a nuclear antigen, as a diagnostic marker for systemic lupus erythematosus (SLE). Reactivity of sera from 114 Portuguese patients with autoimmune rheumatic disease or from healthy blood donors (HBD), against MCP1, produced in bacteria (bact-MCP1) or in its native form (native-MCP1), was determined by immunoblotting. Predictive and discriminative power of MCP1 reactivity for SLE diagnosis in disease-control groups was evaluated by logistic regression, its diagnostic value determined by receiver-operating characteristic analysis and compared with similar analysis of antinuclear antibody and double-stranded DNA (dsDNA). We demonstrated that native-MCP1, in contrast to bact-MCP1, reacts with SLE sera with significant predictive and discriminative power versus other autoimmune diseases (odds ratio [OR] ≤3.537 and ≥3.265; area under the receiver-operating characteristic curve [AUC] ≤0.643 and ≥0.636) or versus HBD (OR = 5.006; AUC = 0.671), showing a good diagnostic power with high specificity (82.1% versus HBD) and low sensitivity for SLE, similar to those of dsDNA. The reactivity of SLE sera with native-MCP1 was shown to be dependent on the presence of phosphorylated residues. Native-MCP1 was shown to have diagnostic value as a specific marker for SLE diagnosis and, therefore, is a suitable substrate for a new antibody test. The widely reported importance of phosphorylated epitopes as targets for autoantibodies in SLE could also be confirmed for native-MCP1. FAU - Bronze-da-Rocha, Elsa AU - Bronze-da-Rocha E AD - Departamento de Ciências Biológicas, Laboratório de Bioquímica, Faculdade de Farmácia, Universidade do Porto, Rua Aníbal Cunha, 164, 4050-047, Porto, Portugal. elsa.rocha@ff.up.pt FAU - Nóvoa, Ana AU - Nóvoa A FAU - Teixeira, Natércia AU - Teixeira N FAU - Vasconcelos, Carlos Silva AU - Vasconcelos CS FAU - Cerveira, Conceição AU - Cerveira C FAU - Castro e Melo, João AU - Castro e Melo J FAU - Carvalho, Manuel Cirne AU - Carvalho MC LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120229 PL - Netherlands TA - J Clin Immunol JT - Journal of clinical immunology JID - 8102137 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (Autoantigens) RN - 0 (Biomarkers) RN - 0 (metaphase chromosome protein 1, human) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood/immunology MH - Antigens, Nuclear/*immunology MH - Arthritis, Rheumatoid/immunology MH - Autoantigens/*immunology MH - Biomarkers/blood MH - CREST Syndrome/immunology MH - Cell Line, Tumor MH - Dermatomyositis/immunology MH - Female MH - HeLa Cells MH - Humans MH - Lupus Erythematosus, Systemic/*diagnosis/*immunology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/immunology MH - Portugal MH - Raynaud Disease/immunology MH - Scleroderma, Systemic/immunology MH - Sjogren's Syndrome/immunology MH - Young Adult EDAT- 2012/03/01 06:00 MHDA- 2013/01/23 06:00 CRDT- 2012/02/29 06:00 PHST- 2011/11/25 00:00 [received] PHST- 2012/02/03 00:00 [accepted] PHST- 2012/02/29 06:00 [entrez] PHST- 2012/03/01 06:00 [pubmed] PHST- 2013/01/23 06:00 [medline] AID - 10.1007/s10875-012-9665-4 [doi] PST - ppublish SO - J Clin Immunol. 2012 Aug;32(4):721-8. doi: 10.1007/s10875-012-9665-4. Epub 2012 Feb 29. PMID- 32173655 OWN - NLM STAT- MEDLINE DCOM- 20210831 LR - 20210831 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 48 IP - 1 DP - 2021 Jan 1 TI - The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease? PG - 82-86 LID - 10.3899/jrheum.190976 [doi] AB - OBJECTIVE: To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients. METHODS: We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom. RESULTS: One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation. CONCLUSION: This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc. FAU - Blaja, Elisabeth AU - Blaja E AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Jordan, Suzana AU - Jordan S AUID- ORCID: 0000-0002-8114-8239 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Mihai, Carmen-Marina AU - Mihai CM AUID- ORCID: 0000-0002-8627-8817 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Dobrota, Rucsandra AU - Dobrota R AUID- ORCID: 0000-0001-9819-7574 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Becker, Mike Oliver AU - Becker MO AUID- ORCID: 0000-0001-9102-3088 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Maurer, Britta AU - Maurer B AUID- ORCID: 0000-0001-9385-8097 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; Oliver.Distler@usz.ch. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200315 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2020 Nov 1;47(11):1724. doi: 10.3899/jrheum.200345. PMID: 32669449 MH - Humans MH - Microscopic Angioscopy MH - *Raynaud Disease MH - *Rheumatic Diseases MH - *Rheumatology MH - *Scleroderma, Systemic/diagnosis OTO - NOTNLM OT - autoimmune diseases OT - disease duration OT - systemic sclerosis EDAT- 2020/03/17 06:00 MHDA- 2021/09/01 06:00 CRDT- 2020/03/17 06:00 PHST- 2020/03/06 00:00 [accepted] PHST- 2020/03/17 06:00 [pubmed] PHST- 2021/09/01 06:00 [medline] PHST- 2020/03/17 06:00 [entrez] AID - jrheum.190976 [pii] AID - 10.3899/jrheum.190976 [doi] PST - ppublish SO - J Rheumatol. 2021 Jan 1;48(1):82-86. doi: 10.3899/jrheum.190976. Epub 2020 Mar 15. PMID- 35633574 OWN - NLM STAT- MEDLINE DCOM- 20220531 LR - 20220531 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 1 IP - 1 DP - 2022 Jan-Mar TI - Clinical features and outcome of 1054 patients with Systemic Sclerosis: analysis of Reuma.pt/SSc registry. PG - 21-29 AB - BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disorder with heterogeneous manifestations and outcomes. Besides differences in disease characteristics among distinct ethnic groups and geographical regions, several questions regarding the impact of the disease and the effectiveness of treatments remain unanswered. To address these questions, the Rheumatic Diseases Portuguese Register (Reuma.pt) launched a specific protocol for the prospective follow-up of SSc patients. OBJECTIVES: To describe the baseline characteristics, disease subsets, treatments used and survival of SSc patients registered in Reuma.pt/SSc. METHODS: Data from adult patients with SSc included in Reuma.pt up to November 2020 were analysed. Demographic features, SSc subsets, fulfilment of classification criteria, main clinical and immunological features, comorbidities, treatments used and survival data were described and compared between diffuse cutaneous (dc) and limited cutaneous (lc) disease subsets. Survival was calculated for patients included in Reuma.pt within the first two years of diagnosis. RESULTS: In total, 1054 patients were included, 87.5% female, with a mean age at diagnosis of 52.7 +/- 14.8 years. The most common subset was lcSSc (56.3%), followed by dcSSc (17.5%), preclinical SSc (13%), overlap syndrome (9.8%) and SSc sine scleroderma (3.3%). Raynaud's phenomenon (93.4%) and skin thickening (76.9%) were the most frequently observed clinical manifestations. Gastrointestinal (62.8% versus 47.8%), pulmonary (59.5% versus 23%) and cardiac (12.8% versus 6.9%) involvements were significantly more prevalent in dcSSc than lcSSc. Ninety per-cent of patients were Antinuclear antibody positive, 52.5% were Anti-centromere antibody positive and 21% anti-topoisomerase positive, with significant differences between lcSSc and dcSSc. One-third of patients were treated with immunomodulators, 53.6% with vasodilators, 23% with glucocorticoids and 2.3% with biologics. During follow-up, 83 deaths (7.9%) were reported. The overall 1-, 2- and 5-year survivals were 98.0%, 96.8% and 92.6%, respectively, without significant differences between lcSSc and dcSSc. CONCLUSION: Reuma.pt/SSc data highlights the importance of registries in improving knowledge about rare and complex diseases, such as SSc. Clinical features of Portuguese SSc patients are similar to those of other populations. In recently diagnosed patients, 5-year survival is over 92%. To the best of our knowledge, this is the first study showing that clinical features of Portuguese SSc are similar to those of other cohorts. FAU - Freitas, Raquel AU - Freitas R AD - Serviço de Reumatologia, Hospital Garcia de Orta. FAU - Martins, Patrícia AU - Martins P AD - Serviço de Reumatologia, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa. FAU - Dourado, Eduardo AU - Dourado E AD - Serviço de Reumatologia, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa. FAU - Santiago, Tânia AU - Santiago T AD - Serviço de Reumatologia, Centro Hospitalar e Universitário de Coimbra; Institute for Clinical and Biomedical Research, Faculty of Medicine, University of Coimbra. FAU - Guimarães, Francisca AU - Guimarães F AD - Serviço de Reumatologia, Unidade Local de Saúde do Alto Minho. FAU - Fernandes, Bruno Miguel AU - Fernandes BM AD - Serviço de Reumatologia, Centro Hospitalar Universitário São João. FAU - Garcia, Salomé AU - Garcia S AD - Serviço de Reumatologia, Centro Hospitalar Universitário São João. FAU - Samões, Beatriz AU - Samões B AD - Serviço de Reumatologia, Centro Hospitalar Vila Nova de Gaia. FAU - Pinto, Ana Sofia AU - Pinto AS AD - Serviço de Reumatologia, Unidade de Saúde Local da Guarda. FAU - Gonçalves, Nuno AU - Gonçalves N AD - Serviço de Reumatologia, Centro Hospitalar Lisboa Ocidental; Hospital Central do Funchal. FAU - Lourenço, Maria Helena AU - Lourenço MH AD - Serviço de Reumatologia, Centro Hospitalar Lisboa Ocidental. FAU - Costa, Emanuel AU - Costa E AD - Serviço de Reumatologia, Hospital de Braga. FAU - Rocha, Margarida AU - Rocha M AD - Serviço de Reumatologia, Centro Hospitalar Universitário do Algarve. FAU - Couto, Maura AU - Couto M AD - Serviço de Reumatologia, Centro Hospitalar Tondela - Viseu. FAU - Duarte, Ana Catarina AU - Duarte AC AD - Serviço de Reumatologia, Hospital Garcia de Orta. FAU - Araújo, Filipe AU - Araújo F AD - Serviço de Reumatologia, Hospital de Sant'Ana. FAU - Cordeiro, Inês AU - Cordeiro I AD - Serviço de Reumatologia, Centro Hospitalar Universitário Lisboa Norte. FAU - Godinho, Fátima AU - Godinho F AD - Serviço de Reumatologia, Hospital Garcia de Orta. FAU - Resende, Catarina AU - Resende C AD - Serviço de Reumatologia, Centro Hospitalar Universitário Lisboa Norte. FAU - Salvador, Maria João AU - Salvador MJ AD - Serviço de Reumatologia, Centro Hospitalar e Universitário de Coimbra. FAU - Cordeiro, Ana AU - Cordeiro A AD - Serviço de Reumatologia, Hospital Garcia de Orta. FAU - Santos, Maria José AU - Santos MJ AD - Serviço de Reumatologia, Hospital Garcia de Orta; Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa. LA - eng PT - Journal Article TT - Clinical features and outcome of 1054 patients with Systemic Sclerosis: analysis of Reuma.pt/SSc registry. PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - *CREST Syndrome MH - *Connective Tissue Diseases MH - Female MH - Humans MH - Male MH - Prospective Studies MH - Registries MH - *Scleroderma, Diffuse/diagnosis MH - *Scleroderma, Systemic/diagnosis MH - *Skin Diseases EDAT- 2022/05/29 06:00 MHDA- 2022/06/01 06:00 CRDT- 2022/05/28 17:32 PHST- 2022/05/28 17:32 [entrez] PHST- 2022/05/29 06:00 [pubmed] PHST- 2022/06/01 06:00 [medline] AID - AO210205 [pii] PST - ppublish SO - ARP Rheumatol. 2022 Jan-Mar;1(1):21-29. PMID- 29930420 OWN - NLM STAT- MEDLINE DCOM- 20180625 LR - 20181202 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 50 IP - 3 DP - 2018 Jun 18 TI - [Correlation between nailfold capillaroscopic findings and presence of interstitial lung disease in systemic sclerosis patients]. PG - 501-506 AB - OBJECTIVE: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. METHODS: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters. RESULTS: Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array. CONCLUSION: Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc. FAU - Ji, L L AU - Ji LL AD - Department of Rheumatology and Immunology, Peking University First Hospital, Beijing 100034, China. FAU - Wang, H AU - Wang H AD - Department of Radiology, Peking University First Hospital, Beijing 100034, China. FAU - Zhang, X H AU - Zhang XH AD - Department of Rheumatology and Immunology, Peking University First Hospital, Beijing 100034, China. FAU - Zhang, Z L AU - Zhang ZL AD - Department of Rheumatology and Immunology, Peking University First Hospital, Beijing 100034, China. LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Adult MH - Aged MH - Capillaries MH - Female MH - Hemorrhage MH - Humans MH - Lung Diseases, Interstitial/*complications MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nail Diseases/*complications MH - Nails MH - Raynaud Disease MH - Scleroderma, Systemic/*complications EDAT- 2018/06/23 06:00 MHDA- 2018/06/26 06:00 CRDT- 2018/06/23 06:00 PHST- 2018/06/23 06:00 [entrez] PHST- 2018/06/23 06:00 [pubmed] PHST- 2018/06/26 06:00 [medline] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2018 Jun 18;50(3):501-506. PMID- 36394746 OWN - NLM STAT- MEDLINE DCOM- 20221121 LR - 20221216 IS - 2284-0729 (Electronic) IS - 1128-3602 (Linking) VI - 26 IP - 21 DP - 2022 Nov TI - Intravenous iloprost in systemic sclerosis and its effect in cardiopulmonary function: a retrospective observational study. PG - 7967-7973 LID - 30149 [pii] LID - 10.26355/eurrev_202211_30149 [doi] AB - OBJECTIVE: This study aims at evaluating the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutively enrolled systemic sclerosis (SSc) patients treated with the approved iloprost regimen. PATIENTS AND METHODS: A retrospective observational study was performed on 68 SSc patients treated with 5-6 infusions of iloprost per month for 6 hours per day at a dosage of 0.5-2.0 ng/kg/min through a portable syringe pump. All patients were evaluated for modified Rodnan skin score, systolic pulmonary arterial pressure, tricuspid annular plane systolic excursion, diffusing capacity of the lungs for carbon monoxide, forced vital capacity, alveolar volume, diffusing capacity of the lungs for carbon monoxide/alveolar volume, pro-brain natriuretic peptide (pBNP), New York Heart Association class, and the presence or absence of digital ulcers (DUs). RESULTS: After a follow-up period of 9.9±2.9 years, all patients improved in frequency and severity of the Raynaud phenomenon and showed a stabilization or improvement of cardiopulmonary parameters. The pulmonary arterial pressure and pBNP improved significantly from baseline (30.91±6.4 mmHg vs. 27.36±7.1 mmHg, and 97.20±69.3 pg/ml vs. 66.65±44.3 pg/ml, respectively; p<0.0001 for both). A significant improvement was observed in the modified Rodnan skin score in 57 patients who continued the treatment during the entire follow-up (5.09±5.7 vs. 3.30±4.2, p<0.0001). CONCLUSIONS: Despite the retrospective design and the lack of a control group, the regular and continued administration of iloprost maintained the stability of the cardiopulmonary and cutaneous parameters in SSc. It significantly reduced pBNP levels, a prognostic cardiac biomarker of SSc. Future research should be addressed to demonstrate a stronger causality of this effect. FAU - Foti, R AU - Foti R AD - Rheumatology Unit, San Marco Hospital, Policlinico University of Catania, Catania, Italy. elivisa21@gmail.com. FAU - Amato, G AU - Amato G FAU - Benenati, A AU - Benenati A FAU - Dal Bosco, Y AU - Dal Bosco Y FAU - Piazza, R AU - Piazza R FAU - Gagliano, C AU - Gagliano C FAU - Foti, R AU - Foti R FAU - Bellofiore, S AU - Bellofiore S FAU - Visalli, E AU - Visalli E LA - eng PT - Journal Article PT - Observational Study PL - Italy TA - Eur Rev Med Pharmacol Sci JT - European review for medical and pharmacological sciences JID - 9717360 RN - JED5K35YGL (Iloprost) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Humans MH - Iloprost MH - Retrospective Studies MH - Carbon Monoxide MH - *Scleroderma, Systemic/drug therapy MH - *Raynaud Disease EDAT- 2022/11/18 06:00 MHDA- 2022/11/22 06:00 CRDT- 2022/11/17 13:58 PHST- 2022/11/17 13:58 [entrez] PHST- 2022/11/18 06:00 [pubmed] PHST- 2022/11/22 06:00 [medline] AID - 30149 [pii] AID - 10.26355/eurrev_202211_30149 [doi] PST - ppublish SO - Eur Rev Med Pharmacol Sci. 2022 Nov;26(21):7967-7973. doi: 10.26355/eurrev_202211_30149. PMID- 40410650 OWN - NLM STAT- MEDLINE DCOM- 20250707 LR - 20250707 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 7 DP - 2025 Jul TI - Positive thyroid autoantibodies in primary Sjögren syndrome define a subset of patients with milder disease. PG - 2889-2895 LID - 10.1007/s10067-025-07500-1 [doi] AB - OBJECTIVE: To investigate the prevalence, clinical and immune characteristics of patients with primary Sjögren syndrome (pSjS) with positive thyroid autoantibodies (TABs) in Chinese population. METHODS: 391 cases of pSjS were retrospectively analyzed. Patients with positive thyroid autoantibodies (TABs), the presence of either thyroglobulin antibody (TG-Ab), thyroid peroxidase antibody (TPO-Ab), or thyrotropin receptor antibody (TR-Ab), or any combination of these were defined as positive TABs group, and patients without any TABs were defined as negative TABs group. The clinical manifestations and laboratory results were compared between the two groups. RESULTS: Among the 391 pSjS patients, the TABs were positive in 32.99% (129/391) patients. In the positive TABs group, the prevalence of Raynaud phenomenon was higher, the prevalence of interstitial lung disease (ILD) and low complement 4 (C4) level were significantly lower. Additionally, the levels of serum globulin, immunoglobulin (Ig) G and IgM were higher compared with patients with negative TABs. Age, ILD, RF and anti-SSA were negatively correlated with the coexistence of TABs, while C-reactive protein (CRP) was positively correlated. CONCLUSION: PSjS was closely related to autoimmune thyroid abnormalities. The prevalence of autoimmune thyroid diseases (AITDs) and TABs in pSjS was high, pSjS patients with positive TABs might represent a milder subset of the disease with a better prognosis with low risk of ILD. Key Points • PSjS was closely related to autoimmune thyroid abnormalities, the prevalence of autoimmune thyroid diseases (AITDs) and TABs in pSjS were high. • The prevalence of interstitial lung disease (ILD) and low complement 4 (C4) level were significantly lower in the positive TABs group. PSjS patients with positive TABs may represent a milder subset of the disease with a better prognosis. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Gu, Xiaojing AU - Gu X AUID- ORCID: 0009-0002-4608-5479 AD - Department of Rheumatology Immunology, The First Affiliated Hospital of Gannan Medical University, Ganzhou, Jiangxi, China. FAU - Chen, Yangchun AU - Chen Y AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. FAU - Xuan, Jingxiu AU - Xuan J AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. FAU - Shi, Yingying AU - Shi Y AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. FAU - Chen, Shiju AU - Chen S AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. shiju@xmu.edu.cn. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. shiju@xmu.edu.cn. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. shiju@xmu.edu.cn. FAU - Liu, Yuan AU - Liu Y AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. liuyuan@xmu.edu.cn. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. liuyuan@xmu.edu.cn. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. liuyuan@xmu.edu.cn. FAU - Shi, Guixiu AU - Shi G AD - Department of Rheumatology and Clinical Immunology, School of Medicine, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, China. gshi@xmu.edu.cn. AD - Xiamen Municipal Clinical Research Center for Immune Diseases, Xiamen, Fujian, China. gshi@xmu.edu.cn. AD - Xiamen Key Laboratory of Rheumatology and Clinical Immunology, Xiamen, Fujian, China. gshi@xmu.edu.cn. LA - eng PT - Journal Article DEP - 20250523 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (anti-thyroid autoantibodies) RN - 0 (Complement C4) SB - IM MH - Humans MH - *Sjogren's Syndrome/immunology/blood/complications MH - Female MH - *Autoantibodies/blood/immunology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Adult MH - Aged MH - China/epidemiology MH - Prevalence MH - Lung Diseases, Interstitial/epidemiology MH - Raynaud Disease MH - Complement C4 OTO - NOTNLM OT - Interstitial lung diseases OT - Primary Sjögren syndrome OT - Thyroid autoantibodies COIS- Declarations. Competing interests: The authors declare no competing interests. EDAT- 2025/05/24 00:45 MHDA- 2025/07/07 12:27 CRDT- 2025/05/23 23:37 PHST- 2024/10/08 00:00 [received] PHST- 2025/05/15 00:00 [accepted] PHST- 2025/03/20 00:00 [revised] PHST- 2025/07/07 12:27 [medline] PHST- 2025/05/24 00:45 [pubmed] PHST- 2025/05/23 23:37 [entrez] AID - 10.1007/s10067-025-07500-1 [pii] AID - 10.1007/s10067-025-07500-1 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Jul;44(7):2889-2895. doi: 10.1007/s10067-025-07500-1. Epub 2025 May 23. PMID- 28057519 OWN - NLM STAT- MEDLINE DCOM- 20180222 LR - 20180525 IS - 1095-953X (Electronic) IS - 0969-9961 (Linking) VI - 100 DP - 2017 Apr TI - Col4a1 mutation generates vascular abnormalities correlated with neuronal damage in a mouse model of HANAC syndrome. PG - 52-61 LID - S0969-9961(16)30295-9 [pii] LID - 10.1016/j.nbd.2016.12.014 [doi] AB - The HANAC syndrome is caused by mutations in the gene coding for collagen4a1, a major component of blood vessel basement membranes. Ocular symptoms include an increase in blood vessel tortuosity and occasional hemorrhages. To examine how vascular defects can affect neuronal function, we analyzed the retinal phenotype of a HANAC mouse model. Heterozygous mutant mice displayed both a thinning of the basement membrane in retinal blood vessels and in Bruch's membrane resulting in vascular leakage. Homozygous mice had additional vascular changes, including greater vessel coverage and tortuosity. This greater tortuosity was associated to higher expression levels of vascular endothelial growth factor (VEGF). These major changes to the blood vessels were correlated with photoreceptor dysfunction and degeneration. The neuronal damage was associated with reactive gliosis in astrocytes and Müller glial cells, and by the migration of microglial cells into the outer retina. This study illustrates how vascular changes can trigger neuronal degeneration in a new model of HANAC syndrome that can be used to further study dysfunctions of neurovascular coupling. SUMMARY STATEMENT: This study provides a phenotypic analysis of a novel mouse model of HANAC syndrome focusing on the retinal aspect. It recapitulates most of the aspects of the human disease and is therefore a great tool to study and to address this condition. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Trouillet, Alix AU - Trouillet A AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Lorach, Henri AU - Lorach H AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Dubus, Elisabeth AU - Dubus E AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - El Mathari, Brahim AU - El Mathari B AD - Fovea Sanofi, Paris, France. FAU - Ivkovic, Ivana AU - Ivkovic I AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Dégardin, Julie AU - Dégardin J AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Simonutti, Manuel AU - Simonutti M AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Paques, Michel AU - Paques M AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France; CHNO des Quinze-Vingts, Paris, France. FAU - Guillonneau, Xavier AU - Guillonneau X AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Sennlaub, Florian AU - Sennlaub F AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. FAU - Sahel, José-Alain AU - Sahel JA AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France; CHNO des Quinze-Vingts, Paris, France; Fondation Ophtalmologique Adolphe de Rothschild, Paris, France; Academie des Sciences, Paris, France. FAU - Ronco, Pierre AU - Ronco P AD - AP HP, Department of Nephrology, Tenon Hospital, Paris, France; INSERM, U1155, Paris F-75020, France; Sorbonne Universités, UPMC Univ Paris 06, UMRS_1155, Paris F-75020, France. FAU - Plaisier, Emmanuelle AU - Plaisier E AD - AP HP, Department of Nephrology, Tenon Hospital, Paris, France; INSERM, U1155, Paris F-75020, France; Sorbonne Universités, UPMC Univ Paris 06, UMRS_1155, Paris F-75020, France. Electronic address: emmanuelle.plaisier@tnn.aphp.fr. FAU - Picaud, Serge AU - Picaud S AD - INSERM, U968, Institut de la Vision, Paris F-75012, France; Sorbonne Universités, UPMC Univ Paris 06, UMR_S968, Institut de la Vision, Paris, F-75012, France; CNRS UMR7210, Institut de la Vision, Paris 75012, France. Electronic address: serge.picaud@inserm.fr. LA - eng PT - Journal Article DEP - 20170103 PL - United States TA - Neurobiol Dis JT - Neurobiology of disease JID - 9500169 RN - 0 (Col4a1 protein, mouse) RN - 0 (Collagen Type IV) RN - 0 (Vascular Endothelial Growth Factor A) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Animals MH - Collagen Type IV/*genetics MH - Disease Models, Animal MH - Mice, Transgenic MH - Muscle Cramp/*genetics MH - Mutation/*genetics MH - Neuroglia/metabolism MH - Neurons/metabolism/*pathology MH - Raynaud Disease/*genetics MH - Retina/metabolism MH - Retinal Vessels/*abnormalities/metabolism MH - Vascular Endothelial Growth Factor A/metabolism OTO - NOTNLM OT - Collagen OT - Mouse model OT - Retinal damage OT - Vascular permeability EDAT- 2017/01/07 06:00 MHDA- 2018/02/23 06:00 CRDT- 2017/01/07 06:00 PHST- 2016/07/21 00:00 [received] PHST- 2016/12/01 00:00 [revised] PHST- 2016/12/18 00:00 [accepted] PHST- 2017/01/07 06:00 [pubmed] PHST- 2018/02/23 06:00 [medline] PHST- 2017/01/07 06:00 [entrez] AID - S0969-9961(16)30295-9 [pii] AID - 10.1016/j.nbd.2016.12.014 [doi] PST - ppublish SO - Neurobiol Dis. 2017 Apr;100:52-61. doi: 10.1016/j.nbd.2016.12.014. Epub 2017 Jan 3. PMID- 17527081 OWN - NLM STAT- MEDLINE DCOM- 20070716 LR - 20070528 IS - 0147-5185 (Print) IS - 0147-5185 (Linking) VI - 31 IP - 6 DP - 2007 Jun TI - Role of human papillomavirus in squamous cell metaplasia-dysplasia-carcinoma of the rectum. PG - 919-25 AB - Primary colorectal squamous cell carcinoma (SCC) and squamous dysplasia are uncommon and little is known about their pathogenesis. Most have been reported in association with ulcerative colitis and other chronic disease states. Although cervical and anal SCC have been strongly linked to human papillomavirus (HPV) infection, the role of HPV in rectal squamous carcinoma has not been well-examined. We evaluated 3 cases of primary rectal SCC for the presence of high-risk HPV by immunohistochemistry for p16(INK4A), in situ hybridization, and polymerase chain reaction. HPV type 16 was detected by polymerase chain reaction in all cases. In addition, all cases exhibited diffuse strong reactivity for p16(INK4A) and punctate nuclear staining by Ventana HPVIII in situ hybridization. The presence of HPV 16 in all three cases suggests that high-risk HPV infection is a risk factor for rectal SCC, particularly in patients with underlying chronic inflammatory disease processes or altered immune status. Further studies are warranted to determine if SCC occurring more proximal in the colon are also HPV-dependent or occur via another, HPV-independent pathway. FAU - Kong, Christina S AU - Kong CS AD - Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305-5324, USA. ckong@stanford.edu FAU - Welton, Mark L AU - Welton ML FAU - Longacre, Teri A AU - Longacre TA LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Surg Pathol JT - The American journal of surgical pathology JID - 7707904 RN - 0 (Cyclin-Dependent Kinase Inhibitor p16) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Carcinoma, Squamous Cell/metabolism/pathology/*virology MH - Cyclin-Dependent Kinase Inhibitor p16/metabolism MH - Female MH - Hepatitis C, Chronic/pathology MH - Humans MH - Hypothyroidism/pathology MH - Immunohistochemistry MH - In Situ Hybridization MH - Male MH - Metaplasia/metabolism/pathology/virology MH - Middle Aged MH - Papillomaviridae/isolation & purification MH - Papillomavirus Infections/*complications/metabolism/pathology MH - Polymerase Chain Reaction MH - Precancerous Conditions/metabolism/pathology/*virology MH - Raynaud Disease/pathology MH - Rectal Neoplasms/pathology/*virology MH - Rectum/pathology/*virology MH - Tumor Virus Infections/*complications/metabolism/pathology MH - Uterine Cervical Dysplasia/pathology/virology EDAT- 2007/05/29 09:00 MHDA- 2007/07/17 09:00 CRDT- 2007/05/29 09:00 PHST- 2007/05/29 09:00 [pubmed] PHST- 2007/07/17 09:00 [medline] PHST- 2007/05/29 09:00 [entrez] AID - 00000478-200706000-00014 [pii] AID - 10.1097/01.pas.0000213441.86030.fc [doi] PST - ppublish SO - Am J Surg Pathol. 2007 Jun;31(6):919-25. doi: 10.1097/01.pas.0000213441.86030.fc. PMID- 30092330 OWN - NLM STAT- MEDLINE DCOM- 20190306 LR - 20190306 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 80 IP - 2 DP - 2019 Feb TI - Association of skin hyperpigmentation disorders with digital ulcers in systemic sclerosis: Analysis of a cohort of 239 patients. PG - 478-484 LID - S0190-9622(18)32335-1 [pii] LID - 10.1016/j.jaad.2018.07.033 [doi] AB - BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc. CI - Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Leroy, Vaianu AU - Leroy V AD - Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. FAU - Henrot, Pauline AU - Henrot P AD - Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. FAU - Barnetche, Thomas AU - Barnetche T AD - Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France. FAU - Cario, Muriel AU - Cario M AD - Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. FAU - Darrigade, Anne-Sophie AU - Darrigade AS AD - Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. FAU - Manicki, Pauline AU - Manicki P AD - Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France. FAU - Doutre, Marie-Sylvie AU - Doutre MS AD - Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. FAU - Lazaro, Estibaliz AU - Lazaro E AD - Department of Internal Medicine, Hôpital Haut-Levêque, CHU de Bordeaux, Bordeaux, France. FAU - Constans, Joel AU - Constans J AD - Department of Vascular Medicine, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. FAU - Barcat, Damien AU - Barcat D AD - Department of Internal Medicine, Hôpital Robert Boulin, Libourne, France. FAU - Vernhes, Jean-Philippe AU - Vernhes JP AD - Department of Rheumatology, Hôpital Robert Boulin, Libourne. FAU - Richez, Christophe AU - Richez C AD - Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France. FAU - Taieb, Alain AU - Taieb A AD - Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France; Immunoconcept, CNRS UMR 5164, Bordeaux University, Bordeaux, France. FAU - Seneschal, Julien AU - Seneschal J AD - Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. Electronic address: julien.seneschal@chu-bordeaux.fr. CN - Fédération Hospitalo-Universitaire–Aquitaine's Care and Research Organisation for Inflammatory and Immune-Mediated Diseases LA - eng PT - Journal Article DEP - 20180807 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Adult MH - Age Distribution MH - Aged MH - Cohort Studies MH - Comorbidity MH - Female MH - Fingers/*pathology MH - France MH - Humans MH - Hyperpigmentation/diagnosis/*epidemiology/therapy MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Prevalence MH - Raynaud Disease/physiopathology MH - Retrospective Studies MH - Scleroderma, Systemic/diagnosis/*epidemiology/therapy MH - Severity of Illness Index MH - Sex Distribution MH - Skin Ulcer/diagnosis/*epidemiology/therapy MH - Statistics, Nonparametric OTO - NOTNLM OT - digital ulcers OT - pigmentation OT - systemic sclerosis EDAT- 2018/08/10 06:00 MHDA- 2019/03/07 06:00 CRDT- 2018/08/10 06:00 PHST- 2018/02/19 00:00 [received] PHST- 2018/06/14 00:00 [revised] PHST- 2018/07/26 00:00 [accepted] PHST- 2018/08/10 06:00 [pubmed] PHST- 2019/03/07 06:00 [medline] PHST- 2018/08/10 06:00 [entrez] AID - S0190-9622(18)32335-1 [pii] AID - 10.1016/j.jaad.2018.07.033 [doi] PST - ppublish SO - J Am Acad Dermatol. 2019 Feb;80(2):478-484. doi: 10.1016/j.jaad.2018.07.033. Epub 2018 Aug 7. PMID- 38272842 OWN - NLM STAT- MEDLINE DCOM- 20240529 LR - 20260518 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 76 IP - 6 DP - 2024 Jun TI - Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality. PG - 963-972 LID - 10.1002/art.42813 [doi] AB - OBJECTIVE: Myositis-associated autoantibodies (MAAs) have been associated with overlap myositis, certain disease manifestations such as interstitial lung disease (ILD), and worse prognosis in the idiopathic inflammatory myopathies. MAAs overall remain largely uncharacterized in patients with juvenile-onset myositis. Moreover, it is unknown whether the number of MAAs is associated with disease severity. METHODS: Patients with juvenile myositis in cross-sectional natural history studies who underwent testing for myositis autoantibodies were included. Demographics, myositis autoantibodies, clinical characteristics, medications received, and outcomes of those with and without MAAs were compared. Multivariable logistic regression was performed to determine whether the number of MAAs detected was associated with severe disease features. RESULTS: Among 551 patients, 36% had an MAA and 13% had more than one MAA. Among those who were MAA positive, there was a higher frequency of overlap myositis (18% vs 5.9%, P < 0.001). MAA positivity was associated with certain clinical features, including Raynaud phenomenon (odds ratio [OR] 2.44, 95% confidence interval [CI] 1.41-4.28) and ILD (OR 3.43, 95% CI 1.75-6.96), as well as a chronic disease course (OR 1.72, 95% CI 1.10-2.72) and mortality (OR 3.76, 95% CI 1.72-8.43). The number of MAAs was also associated with mortality (OR 1.83, 95% CI 1.16-2.86). CONCLUSION: MAAs were prevalent in a large cohort of patients with juvenile myositis. ILD, refractory disease, and mortality were associated with MAA positivity. Prospective studies are needed to determine whether early detection of MAAs may lead to improved outcomes for patients with juvenile myositis. CI - © 2024 Oklahoma Medical Research Foundation and The Authors. Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA. FAU - Sherman, Matthew A AU - Sherman MA AUID- ORCID: 0000-0002-5448-1538 AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. FAU - Noroozi Farhadi, Payam AU - Noroozi Farhadi P AD - National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Pak, Katherine AU - Pak K AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. FAU - Trieu, Edward P AU - Trieu EP AD - Oklahoma Medical Research Foundation, Oklahoma City. FAU - Sarkar, Kakali AU - Sarkar K AD - National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Targoff, Ira N AU - Targoff IN AD - Veterans Affairs Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City. FAU - Neely, Megan L AU - Neely ML AD - Duke University School of Medicine, Durham, North Carolina. FAU - Mammen, Andrew L AU - Mammen AL AUID- ORCID: 0000-0003-3732-3252 AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland, and Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Rider, Lisa G AU - Rider LG AUID- ORCID: 0000-0002-6912-2458 AD - National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. CN - Childhood Myositis Heterogeneity Collaborative Study Group LA - eng GR - ZIA-AR-041203/AR/NIAMS NIH HHS/United States GR - ZIA-ES-101074/ES/NIEHS NIH HHS/United States GR - ZIA ES101074/ImNIH/Intramural NIH HHS/United States GR - NIH/ GR - Z01 ES101081/ImNIH/Intramural NIH HHS/United States GR - ZIA AR041203/ImNIH/Intramural NIH HHS/United States GR - ZIA ES101081/ImNIH/Intramural NIH HHS/United States GR - ZIA-ES-101081/ES/NIEHS NIH HHS/United States PT - Journal Article DEP - 20240312 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Autoantibodies/immunology/blood MH - Male MH - Female MH - Child MH - Adolescent MH - Cross-Sectional Studies MH - *Myositis/immunology/mortality MH - Dermatomyositis/immunology/complications/mortality MH - Severity of Illness Index MH - Lung Diseases, Interstitial/immunology/mortality/etiology MH - Logistic Models MH - Child, Preschool MH - Raynaud Disease/immunology PMC - PMC11136598 MID - NIHMS1962001 FIR - Albert, Daniel A IR - Albert DA FIR - Arabshahi, Bita IR - Arabshahi B FIR - Ardalan, Kaveh IR - Ardalan K FIR - Baer, Alan N IR - Baer AN FIR - Balboni, Imelda M IR - Balboni IM FIR - Ballinger, Susan H IR - Ballinger SH FIR - Bayat, Nastaran IR - Bayat N FIR - Becker, Mara L IR - Becker ML FIR - April Bingham, C IR - April Bingham C FIR - Bohnsack, John F IR - Bohnsack JF FIR - Cartwright, Victoria W IR - Cartwright VW FIR - Cron, Randy Q IR - Cron RQ FIR - Curiel, Rodolfo IR - Curiel R FIR - Dare, Jason A IR - Dare JA FIR - de Guzman, Marietta M IR - de Guzman MM FIR - Dedeoglu, Fatma IR - Dedeoglu F FIR - DeMarco, Paul J IR - DeMarco PJ FIR - Eade, Kaleo IR - Eade K FIR - Eberhard, Barbara Anne IR - Eberhard BA FIR - Edelheit, Barbara S IR - Edelheit BS FIR - Elder, Melissa E IR - Elder ME FIR - Ferguson, Polly IR - Ferguson P FIR - Finkel, Terri H IR - Finkel TH FIR - Fuhlbrigge, Robert C IR - Fuhlbrigge RC FIR - Gedalia, Abraham IR - Gedalia A FIR - Goldmuntz, Ellen A IR - Goldmuntz EA FIR - Gottlieb, Beth S IR - Gottlieb BS FIR - Guirola, Ricardo IR - Guirola R FIR - Hannan, William P IR - Hannan WP FIR - Henrickson, Michael IR - Henrickson M FIR - Hobday, Patricia M IR - Hobday PM FIR - Hong, Sandy IR - Hong S FIR - Huber, Adam M IR - Huber AM FIR - Jansen, Anna IR - Jansen A FIR - Jarvis, James IR - Jarvis J FIR - Jones, Olcay Y IR - Jones OY FIR - Kamdar, Ankur IR - Kamdar A FIR - Kim, Hanna IR - Kim H FIR - Kim, Susan IR - Kim S FIR - Kingsbury, Daniel J IR - Kingsbury DJ FIR - Kishi, Takayuki IR - Kishi T FIR - Lang, Bianca A IR - Lang BA FIR - Lerman, Melissa IR - Lerman M FIR - Lindsley, Carol B IR - Lindsley CB FIR - Miller, Frederick W IR - Miller FW FIR - Mitchell, Stephen R IR - Mitchell SR FIR - Nanda, Kabita IR - Nanda K FIR - Nativ, Simona IR - Nativ S FIR - Onel, Karen B IR - Onel KB FIR - de la Pena, Wendy IR - de la Pena W FIR - Perez, Maria D IR - Perez MD FIR - Person, Donald A IR - Person DA FIR - Pinal-Fernandez, Iago IR - Pinal-Fernandez I FIR - Ronis, Tova IR - Ronis T FIR - Sabbagh, Sara E IR - Sabbagh SE FIR - Schiffenbauer, Adam IR - Schiffenbauer A FIR - Schmeling, Heinrike IR - Schmeling H FIR - Shaham, Bracha IR - Shaham B FIR - Shenoi, Susan IR - Shenoi S FIR - Soep, Jennifer IR - Soep J FIR - Stoll, Matthew L IR - Stoll ML FIR - Sule, Sangeeta IR - Sule S FIR - Tarvin, Stacey E IR - Tarvin SE FIR - Taylor-Albert, Elizabeth IR - Taylor-Albert E FIR - Volochayev, Rita IR - Volochayev R FIR - Wahezi, Dawn IR - Wahezi D FIR - White, Patience H IR - White PH FIR - Zhao, Yongdong IR - Zhao Y EDAT- 2024/01/26 00:43 MHDA- 2024/05/29 06:42 PMCR- 2025/06/01 CRDT- 2024/01/25 22:42 PHST- 2024/01/08 00:00 [revised] PHST- 2023/11/16 00:00 [received] PHST- 2024/01/22 00:00 [accepted] PHST- 2024/05/29 06:42 [medline] PHST- 2024/01/26 00:43 [pubmed] PHST- 2024/01/25 22:42 [entrez] PHST- 2025/06/01 00:00 [pmc-release] AID - 10.1002/art.42813 [doi] PST - ppublish SO - Arthritis Rheumatol. 2024 Jun;76(6):963-972. doi: 10.1002/art.42813. Epub 2024 Mar 12. PMID- 31115358 OWN - NLM STAT- MEDLINE DCOM- 20191227 LR - 20220408 IS - 0973-3922 (Electronic) IS - 0378-6323 (Linking) VI - 85 IP - 4 DP - 2019 Jul-Aug TI - Nail fold dermoscopy in collagen vascular disorders: A cross-sectional study. PG - 439 LID - 10.4103/ijdvl.IJDVL_495_18 [doi] AB - BACKGROUND: The collagen vascular disorders, particularly systemic sclerosis, dermatomyositis, systemic lupus erythematosus and mixed connective tissue disorder, are often characterized by microangiopathic abnormalities of the nail folds. Nail fold dermoscopy is a well-established technique to assess these vascular changes. AIMS: To evaluate finger nail capillary vascular abnormalities by dermoscopy and their correlation with cutaneous and systemic involvement in the patients of collagen vascular disorders. METHODS: This was a cross-sectional study involving patients of collagen vascular disorders presenting to Government Medical College, Amritsar over a period of 2 years. Nail fold dermoscopy was done in these patients and correlated with cutaneous and systemic involvement. Statistical analysis was done using SPSS 17.0 version. RESULTS: A total of 30 patients were enrolled in the study. Sixteen (53.3%), 11 (36.7%) and 3 (10%) patients of systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disorder, respectively were included for nail fold dermoscopy. The commonest change recorded in our study was dilated capillaries in 21 (70%) patients, followed by capillary dropouts in 17 (56.7%) patients and avascular areas in 16 (53.3%) patients. Of 17 patients presenting with sclerodactyly, active, early and late patterns were seen in 7 (41.2%), 2 (11.8%) and 7 (41.2%) patients, respectively. Out of 13 patients with respiratory involvement, active, early and late patterns were seen in 1, 1 and 7 (53.8%) patients, respectively (P value = 0.004). LIMITATIONS: Owing to lesser number of patients in our study, it is difficult to draw conclusive recommendations, and more studies with a larger sample size are required. CONCLUSION: Dermoscopy is a valuable tool not only to diagnose collagen vascular disorders but also for prognostication by correlating with systemic involvement. FAU - Chojer, Parul AU - Chojer P AD - Department of Dermatology, Venereology and Leprology, Government Medical College, Amritsar, Punjab, India. FAU - Mahajan, B B AU - Mahajan BB AD - Department of Dermatology, Venereology and Leprology, Government Medical College, Amritsar, Punjab, India. LA - eng PT - Journal Article PL - United States TA - Indian J Dermatol Venereol Leprol JT - Indian journal of dermatology, venereology and leprology JID - 7701852 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Capillaries/*diagnostic imaging MH - Connective Tissue Diseases/complications/*diagnostic imaging MH - Cross-Sectional Studies MH - *Dermoscopy/methods MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/diagnostic imaging MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Mixed Connective Tissue Disease/diagnostic imaging MH - Nails/*blood supply MH - Raynaud Disease/etiology MH - Respiratory Tract Diseases/etiology MH - Scleroderma, Systemic/diagnostic imaging MH - Young Adult OTO - NOTNLM OT - Collagen vascular disorders OT - dermoscopy OT - systemic lupus erythematosus OT - systemic sclerosis COIS- None EDAT- 2019/05/23 06:00 MHDA- 2019/12/28 06:00 CRDT- 2019/05/23 06:00 PHST- 2019/05/23 06:00 [pubmed] PHST- 2019/12/28 06:00 [medline] PHST- 2019/05/23 06:00 [entrez] AID - 258646 [pii] AID - 10.4103/ijdvl.IJDVL_495_18 [doi] PST - ppublish SO - Indian J Dermatol Venereol Leprol. 2019 Jul-Aug;85(4):439. doi: 10.4103/ijdvl.IJDVL_495_18. PMID- 17605011 OWN - NLM STAT- MEDLINE DCOM- 20090730 LR - 20220311 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 28 IP - 2 DP - 2007 Dec TI - Microvascular dysfunction in rheumatoid arthritis assessed by laser Doppler anemometry: relationship to soluble adhesion molecules and extraarticular manifestations. PG - 145-52 AB - In search of a noninvasive diagnostic test for rheumatoid vasculitis (RV), this study addressed the questions whether changes in capillary blood cell velocity (CBV) detected by laser Doppler anemometry in patients with rheumatoid arthritis (RA) were correlated with the levels of soluble adhesion molecules and whether cutaneous flow abnormalities may reflect extraarticular manifestations in RA. In 31 RA patients and 20 patients with osteoarthritis (OA), CBV was measured in the skin above the left ring finger at rest and after 3-min arterial occlusion. Serum levels of soluble intercellular adhesion molecule-1 (sICAM-1), soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble E-selectin (sE-selectin), and soluble P-selectin (sP-selectin) were assessed by enzyme linked immunosorbent assay. Peak CBV was reduced in RA patients compared to OA patients (0.42 +/- 0.07 mm/s vs. 0.70 +/- 0.13 mm/s; P = 0.013). Both CBV during rest and reactive hyperemia were not correlated with the levels of soluble adhesion molecules. There were no significant differences in resting or peak CBV between RA patients with or without extraarticular manifestations. The lack of an inverse correlation between the levels of soluble adhesion molecules and CBV during rest and reactive hyperemia contradicts the assumption that inflammatory vascular damage indicated by increased levels of soluble adhesion molecules was the main reason for the impairment of microcirculation. The present results do not suggest that cutaneous flow abnormalities may reflect extraarticular manifestations in RA. FAU - Meyer, Martin F AU - Meyer MF AD - Department of Internal Medicine, University Clinic Bergmannsheil, Ruhr-University Bochum, Bochum, Germany. martin.meyer-2@rub.de FAU - Schmidt, Olga AU - Schmidt O FAU - Hellmich, Bernhard AU - Hellmich B FAU - Schatz, Helmut AU - Schatz H FAU - Klein, Harald H AU - Klein HH FAU - Braun, Jürgen AU - Braun J LA - eng PT - Journal Article DEP - 20070629 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Cell Adhesion Molecules) SB - IM MH - Adult MH - Aged MH - Arthritis, Rheumatoid/*physiopathology MH - Blood Flow Velocity MH - Capillaries/*physiopathology MH - Cell Adhesion Molecules/*blood MH - Female MH - Humans MH - *Laser-Doppler Flowmetry MH - Male MH - Microcirculation MH - Middle Aged MH - Raynaud Disease/*physiopathology MH - Regional Blood Flow MH - Rheumatoid Nodule/*physiopathology MH - Sjogren's Syndrome/*physiopathology MH - Skin/blood supply MH - Solubility MH - Vasculitis/physiopathology EDAT- 2007/07/03 09:00 MHDA- 2009/07/31 09:00 CRDT- 2007/07/03 09:00 PHST- 2007/02/08 00:00 [received] PHST- 2007/06/12 00:00 [accepted] PHST- 2007/07/03 09:00 [pubmed] PHST- 2009/07/31 09:00 [medline] PHST- 2007/07/03 09:00 [entrez] AID - 10.1007/s00296-007-0397-3 [doi] PST - ppublish SO - Rheumatol Int. 2007 Dec;28(2):145-52. doi: 10.1007/s00296-007-0397-3. Epub 2007 Jun 29. PMID- 17255677 OWN - NLM STAT- MEDLINE DCOM- 20070220 LR - 20220318 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 119 IP - 1 DP - 2007 Jan TI - Management of vasospastic disorders with botulinum toxin A. PG - 217-226 LID - 10.1097/01.prs.0000244860.00674.57 [doi] AB - BACKGROUND: Surgical digital artery sympathectomy is indicated when medical management has failed to control rest pain, impending infarction of digits, or healing of ischemic ulcerations caused by profound vasospasm that is associated with other systemic diseases. After digital artery sympathectomy, recurrence or persistence of vasospasm may compromise hand function and ultimately result in amputation of all or portions of both lower and upper extremities. METHODS: The authors present a case series of 11 patients with vasospasm producing intractable rest pain, digital ulcerations, and digit infarctions that failed aggressive medical therapy and that were then treated by perivascular injections of botulinum toxin A (Botox). Before Botox injection, the level of pain, cutaneous temperatures, color, and ulcerations and infarctions were documented RESULTS: The authors' longest follow-up was 30 months. All patients reported highly significant pain reduction, 10 of 10 to 0 to 2 of 10, within 24 to 48 hours after injection, persisting for months after the injection. Nine of 11 patients with nonhealing ulcers spontaneously healed small ulcers and areas of infarction after surgical debridement. Two cases required small skin grafts. Nine of 11 patients reported decreased severity and frequency of vasospastic episodes. CONCLUSIONS: Hand injection of botulinum toxin A appears to be an effective treatment for intractable digital ulcerations and rest pain in patients with severe vasospastic disorders. Because of the complexity of surgical digital artery sympathectomy along with its associated high risk of persistent symptoms, the authors conclude that the therapeutic use of botulinum toxin A injections represents an attractive alternative therapy. FAU - Van Beek, Allen L AU - Van Beek AL AD - Minneapolis, Minn. From the Division of Plastic Surgery, Department of Cardiology, and Division of Heart Failure, Transplantation, and Investigational Therapeutics, Minneapolis Heart Institute Foundation, and Section of Cardiology, Department of Medicine, University of Minnesota. FAU - Lim, Paul K AU - Lim PK FAU - Gear, Andrew J L AU - Gear AJL FAU - Pritzker, Marc R AU - Pritzker MR LA - eng PT - Journal Article PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Adult MH - Aged MH - Botulinum Toxins, Type A/*therapeutic use MH - Female MH - Fingers/blood supply MH - Humans MH - Male MH - Middle Aged MH - Neuromuscular Agents/*therapeutic use MH - Raynaud Disease/complications MH - Spasm/*drug therapy/etiology MH - Toes/blood supply MH - Vascular Diseases/*drug therapy/etiology EDAT- 2007/01/27 09:00 MHDA- 2007/02/21 09:00 CRDT- 2007/01/27 09:00 PHST- 2007/01/27 09:00 [pubmed] PHST- 2007/02/21 09:00 [medline] PHST- 2007/01/27 09:00 [entrez] AID - 00006534-200701000-00032 [pii] AID - 10.1097/01.prs.0000244860.00674.57 [doi] PST - ppublish SO - Plast Reconstr Surg. 2007 Jan;119(1):217-226. doi: 10.1097/01.prs.0000244860.00674.57. PMID- 31043545 OWN - NLM STAT- MEDLINE DCOM- 20210510 LR - 20210510 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 47 IP - 2 DP - 2020 Feb TI - Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis. PG - 249-254 LID - 10.3899/jrheum.181173 [doi] AB - OBJECTIVE: To evaluate agreement of the updated European League Against Rheumatism and European Scleroderma Trials and Research group (EUSTAR) recommendations for treatment of systemic sclerosis (SSc) among international experts. In addition, to determine factors that might influence agreement. METHODS: Level of agreement (10-point scale: 0 = not at all, 10 = completely agree) and local drug availability (yes/no) were assessed using an online survey. The Web link to the survey was shared with 481 unique e-mail addresses and SSc networks (Scleroderma Clinical Trials Consortium, Australian Scleroderma Interest Group, International Systemic Sclerosis Inception Cohort). Level of agreement was compared between subgroups stratified for participant characteristics. RESULTS: In total, 263 experts participated, of whom n = 209 (79%) completed each single item. The majority were rheumatologists (n = 200, 76%) working in Europe (n = 185; 71%); 59% (n = 156) were EUSTAR members; and 57% (n = 151) had > 10 years of clinical experience. Overall level of agreement was high (mean 8.0, SD 2.5). The 3 highest mean agreements included (1) angiotensin-converting enzyme inhibitors for scleroderma renal crisis (9.2, SD 2.1); (2) blood pressure control in SSc-patients treated with corticosteroids (9.0, SD 2.2); (3) proton pump inhibitors to prevent reflux complications (9.0, SD 2.2). The 3 lowest mean agreements included (1) fluoxetine for Raynaud phenomenon (RP; 4.6, SD 2.8); (2) hematopoietic stem cell transplantation (HSCT) for severe SSc (7.1, SD 2.9); (3) phosphodiesterase inhibitors 5 for RP (7.3, SD 2.7). Agreement differed between Europe and non-Europe for the use of iloprost, bosentan, methotrexate, HSCT, and cyclophosphamide. Treatment availability could partially explain differential agreement for iloprost, bosentan, and HSCT. CONCLUSION: In general, worldwide expert agreement on updated recommendations for treatment of SSc is high, supporting their value. Differences in agreement are partially explained by geographical area and treatment availability. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AUID- ORCID: 0000-0002-5624-1415 AD - From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. j.k.de_vries-bouwstra@lumc.nl. AD - J.K. de Vries-Bouwstra, MD, PhD, Leiden University Medical Center, Department of Rheumatology; Y. Allanore, MD, PhD, Professor, Paris Descartes University, Cochin Hospital, Rheumatology Department; M. Matucci-Cerinic, MD, PhD, Professor, Department of Experimental and Clinical Medicine, University of Florence; A. Balbir-Gurman, MD, PhD, Professor, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine. j.k.de_vries-bouwstra@lumc.nl. FAU - Allanore, Yannick AU - Allanore Y AD - From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. AD - J.K. de Vries-Bouwstra, MD, PhD, Leiden University Medical Center, Department of Rheumatology; Y. Allanore, MD, PhD, Professor, Paris Descartes University, Cochin Hospital, Rheumatology Department; M. Matucci-Cerinic, MD, PhD, Professor, Department of Experimental and Clinical Medicine, University of Florence; A. Balbir-Gurman, MD, PhD, Professor, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. AD - J.K. de Vries-Bouwstra, MD, PhD, Leiden University Medical Center, Department of Rheumatology; Y. Allanore, MD, PhD, Professor, Paris Descartes University, Cochin Hospital, Rheumatology Department; M. Matucci-Cerinic, MD, PhD, Professor, Department of Experimental and Clinical Medicine, University of Florence; A. Balbir-Gurman, MD, PhD, Professor, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. AD - J.K. de Vries-Bouwstra, MD, PhD, Leiden University Medical Center, Department of Rheumatology; Y. Allanore, MD, PhD, Professor, Paris Descartes University, Cochin Hospital, Rheumatology Department; M. Matucci-Cerinic, MD, PhD, Professor, Department of Experimental and Clinical Medicine, University of Florence; A. Balbir-Gurman, MD, PhD, Professor, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine. LA - eng PT - Journal Article DEP - 20190501 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Proton Pump Inhibitors) RN - 01K63SUP8D (Fluoxetine) SB - IM CIN - J Rheumatol. 2020 Feb;47(2):164-165. doi: 10.3899/jrheum.190582. PMID: 32007942 MH - Adrenal Cortex Hormones/*therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/*therapeutic use MH - Europe MH - Fluoxetine/*therapeutic use MH - Gastroesophageal Reflux/prevention & control MH - Health Services Accessibility MH - Hematopoietic Stem Cell Transplantation/*methods MH - Humans MH - Hypertension/prevention & control MH - Phosphodiesterase Inhibitors/*therapeutic use MH - *Practice Guidelines as Topic MH - Proton Pump Inhibitors/*therapeutic use MH - Raynaud Disease/prevention & control MH - Renal Insufficiency/prevention & control MH - Rheumatologists/*psychology MH - Scleroderma, Systemic/complications/*drug therapy/pathology MH - Severity of Illness Index OTO - NOTNLM OT - DRUG THERAPY OT - GUIDELINE ADHERENCE OT - PRACTICE GUIDELINES OT - SYSTEMIC SCLEROSIS EDAT- 2019/05/03 06:00 MHDA- 2021/05/11 06:00 CRDT- 2019/05/03 06:00 PHST- 2019/04/16 00:00 [accepted] PHST- 2019/05/03 06:00 [pubmed] PHST- 2021/05/11 06:00 [medline] PHST- 2019/05/03 06:00 [entrez] AID - jrheum.181173 [pii] AID - 10.3899/jrheum.181173 [doi] PST - ppublish SO - J Rheumatol. 2020 Feb;47(2):249-254. doi: 10.3899/jrheum.181173. Epub 2019 May 1. PMID- 31317425 OWN - NLM STAT- MEDLINE DCOM- 20201106 LR - 20201106 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 39 IP - 1 DP - 2020 Jan TI - Is there a role for laser speckle contrast analysis (LASCA) in predicting the outcome of digital ulcers in patients with systemic sclerosis? PG - 69-75 LID - 10.1007/s10067-019-04662-7 [doi] AB - OBJECTIVE: Digital ulcers (DUs) represent one major burden for patients with systemic sclerosis (SSc). The objectives of our study were to evaluate blood flow in SSc-DUs with laser speckle contrast analysis (LASCA) and to correlate the skin perfusion to clinical and laboratory data. METHODS: Forty DUs in 31 consecutive patients with SSc according to 2013 ACR/EULAR criteria (20 with limited cutaneous disease, 3 males) were prospectively examined with LASCA. Clinical and laboratory data were collected at the same time. DUs were classified according to clinical features and presence of infection. RESULTS: At LASCA analysis, patients with diffuse SSc had lower mean values of blood flow compared with those with limited disease at the finger affected by DUs (88.80 vs 44.40, p = 0.036) and at the periulcer area (p = 0.041). The presence of infection was associated to a higher flow at the finger with DU (103.02 vs 58.05 p = 0.04), at the level of ulcer (217.63 vs 67.15, p < 0.001), and at the periulcer area (p = 0.001). The ratio between the blood flow at the ulcer area and the finger base (UA/FB) showed a bimodal trend in patients with infected DUs and in those without infections. Infection was positive correlated to the time of healing (HT) (r = 0.648, p = 0.023), while in DUs without infection a negative correlation to HT (r = - 0.46, p = 0.015) was identified. CONCLUSIONS: This study demonstrates for the first time that the UA/FB ratio may predict the healing time of DUs in SSc patients and may be crucial for the prognostic stratification of patients. Infection remains one of the main predictors of DU healing.Key Points• The prognostic value of laser speckle contrast analysis (LASCA) in patients with digital ulcers (DUs) in systemic sclerosis remains to be clarified.• LASCA may be able to predict the haling time of the digital ulcers.• The presence of infection of the wound bed may greatly influence the LASCA parameters and the healing time of the digital ulcer. FAU - Barsotti, Simone AU - Barsotti S AUID- ORCID: 0000-0002-4864-4505 AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. AD - Department of Medical Biotechnologies, University of Siena, Siena, Italy. FAU - d'Ascanio, Anna AU - d'Ascanio A AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Valentina, Venturini AU - Valentina V AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Chiara, Stagnaro AU - Chiara S AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Silvia, Bilia AU - Silvia B AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Laura, Amanzi AU - Laura A AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma, 67, 56126, Pisa, Italy. a.dellarossa@ao-pisa.toscana.it. LA - eng PT - Journal Article DEP - 20190717 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Diagnostic Imaging/*methods MH - Female MH - Fingers/pathology MH - Hand/*pathology MH - Humans MH - Lasers MH - Linear Models MH - Male MH - Middle Aged MH - Pain Measurement MH - Perfusion MH - Prospective Studies MH - Raynaud Disease/pathology MH - Scleroderma, Systemic/*pathology MH - Skin Ulcer/*diagnostic imaging/pathology MH - Ulcer/pathology OTO - NOTNLM OT - Digital ulcers OT - LASCA OT - Laser Doppler OT - Outcome assessment OT - Systemic sclerosis EDAT- 2019/07/19 06:00 MHDA- 2020/11/11 06:00 CRDT- 2019/07/19 06:00 PHST- 2019/04/04 00:00 [received] PHST- 2019/06/25 00:00 [accepted] PHST- 2019/06/19 00:00 [revised] PHST- 2019/07/19 06:00 [pubmed] PHST- 2020/11/11 06:00 [medline] PHST- 2019/07/19 06:00 [entrez] AID - 10.1007/s10067-019-04662-7 [pii] AID - 10.1007/s10067-019-04662-7 [doi] PST - ppublish SO - Clin Rheumatol. 2020 Jan;39(1):69-75. doi: 10.1007/s10067-019-04662-7. Epub 2019 Jul 17. PMID- 28917576 OWN - NLM STAT- MEDLINE DCOM- 20180911 LR - 20181202 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 145 IP - 1 DP - 2018 Jan TI - [Cancers associated with systemic sclerosis involving anti-RNA polymerase III antibodies]. PG - 33-36 LID - S0151-9638(17)30315-0 [pii] LID - 10.1016/j.annder.2017.08.005 [doi] AB - BACKGROUND: The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population. PATIENTS AND METHODS: Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Clinical symptoms led us to prescribe more laboratory exams and both patients were diagnosed with cancer of the nasopharyngeal area. DISCUSSION: Anti-RNA polymerase III antibodies are useful for SSc diagnosis in patients without anti-centromere or anti-Scl70 antibodies. Their presence must lead physicians to screen for associated cancer, even in the absence of clinical signs. CI - Copyright © 2017 Elsevier Masson SAS. All rights reserved. FAU - Monfort, J-B AU - Monfort JB AD - Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France. Electronic address: jean-benoit.monfort@aphp.fr. FAU - Mathian, A AU - Mathian A AD - Service de médecine interne, hôpital Pitié-Salpétrière, 47-83, boulevard de l'hôpital, 75013 Paris, France. FAU - Amoura, Z AU - Amoura Z AD - Service de médecine interne, hôpital Pitié-Salpétrière, 47-83, boulevard de l'hôpital, 75013 Paris, France. FAU - Francès, C AU - Francès C AD - Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France. FAU - Barbaud, A AU - Barbaud A AD - Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France. FAU - Senet, P AU - Senet P AD - Service de dermatologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France. LA - fre PT - Case Reports PT - Journal Article TT - Néoplasies associées à une sclérodermie systémique avec anticorps anti-ARN polymérase III. DEP - 20170913 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Taxoids) RN - 15H5577CQD (Docetaxel) RN - EC 2.7.7.6 (RNA Polymerase III) RN - Q20Q21Q62J (Cisplatin) RN - U3P01618RT (Fluorouracil) SB - IM MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Autoantibodies/*immunology MH - Autoantigens/*immunology MH - Carcinoma/drug therapy/*etiology/radiotherapy/secondary MH - Carcinoma, Squamous Cell/etiology/surgery/therapy MH - Chemoradiotherapy MH - Cisplatin/administration & dosage MH - Combined Modality Therapy MH - Docetaxel MH - Fluorouracil/administration & dosage MH - Humans MH - Lung Neoplasms/drug therapy/radiotherapy/secondary MH - Male MH - Middle Aged MH - Nasopharyngeal Neoplasms/drug therapy/*etiology/immunology/radiotherapy MH - RNA Polymerase III/*immunology MH - Radiotherapy, Adjuvant MH - Raynaud Disease/etiology MH - Remission Induction MH - Scleroderma, Systemic/*complications/diagnosis/immunology MH - Taxoids/administration & dosage MH - Tonsillectomy MH - Young Adult OTO - NOTNLM OT - Anti-RNA polymerase III OT - Anticorps anti-ARN polymérase III OT - Cancer OT - Scleroderma OT - Sclérodermie EDAT- 2017/09/18 06:00 MHDA- 2018/09/12 06:00 CRDT- 2017/09/18 06:00 PHST- 2017/02/08 00:00 [received] PHST- 2017/04/13 00:00 [revised] PHST- 2017/08/21 00:00 [accepted] PHST- 2017/09/18 06:00 [pubmed] PHST- 2018/09/12 06:00 [medline] PHST- 2017/09/18 06:00 [entrez] AID - S0151-9638(17)30315-0 [pii] AID - 10.1016/j.annder.2017.08.005 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2018 Jan;145(1):33-36. doi: 10.1016/j.annder.2017.08.005. Epub 2017 Sep 13. PMID- 18046019 OWN - NLM STAT- MEDLINE DCOM- 20080327 LR - 20200930 IS - 0363-6119 (Print) IS - 0363-6119 (Linking) VI - 294 IP - 2 DP - 2008 Feb TI - Chronobiological characterization of women with primary vasospastic syndrome: body heat loss capacity in relation to sleep initiation and phase of entrainment. PG - R630-8 AB - Women with primary vasospastic syndrome (VS), but otherwise healthy, exhibit a functional disorder of vascular regulation (main symptom: cold extremities) and often suffer from difficulties initiating sleep (DIS). Diverse studies have shown a close association between distal vasodilatation before lights off and a rapid onset of sleep. Therefore, we hypothesized that DIS in women with VS could be due to a reduced heat loss capacity in the evening, i.e., subjects are physiologically not ready for sleep. The aim of the study was to elucidate whether women having both VS and DIS (WVD) or not (controls) show different circadian characteristics (e.g., phase delay of the circadian thermoregulatory system with respect to the sleep-wake cycle). Healthy young women (n = 9 WVD and n = 9 control) completed a 40-h constant routine protocol (adjusted to habitual bedtime) before and after an 8-h sleep episode. Skin temperatures [off-line calculated as distal-proximal skin temperature gradient (DPG)] and core body temperature (CBT; rectal) were continuously recorded. Half-hourly saliva samples were collected for melatonin assay and subjective sleepiness was assessed on the Karolinska Sleepiness Scale (KSS). Compared with control, WVD showed no differences in habitual bed times, but a 1-h circadian phase delay of dim light-melatonin onset (hours after lights on: WVD 14.6 +/- 0.3 h; control 13.5 +/- 0.2 h; P = 0.01). Similar phase shifts were observed in CBT, DPG, and KSS ratings. In conclusion, WVD exhibit a phase delay of the endogenous circadian system with respect to their habitual sleep-wake cycle, which could be a cause of DIS. FAU - Vollenweider, Stephanie AU - Vollenweider S AD - Psychiatric University Clinics, Thermophysiological Chronobiology, Centre for Chronobiology, Wilhelm Klein-Strasse 27, Basel, Switzerland. FAU - Wirz-Justice, Anna AU - Wirz-Justice A FAU - Flammer, Josef AU - Flammer J FAU - Orgül, Selim AU - Orgül S FAU - Kräuchi, Kurt AU - Kräuchi K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20071128 PL - United States TA - Am J Physiol Regul Integr Comp Physiol JT - American journal of physiology. Regulatory, integrative and comparative physiology JID - 100901230 RN - JL5DK93RCL (Melatonin) SB - IM MH - Adult MH - Body Temperature/physiology MH - Body Temperature Regulation/*physiology MH - Circadian Rhythm/*physiology MH - Female MH - Humans MH - Jet Lag Syndrome/physiopathology MH - Melatonin/metabolism MH - Raynaud Disease/*physiopathology MH - Saliva/metabolism MH - Sleep/*physiology MH - Sleep Disorders, Circadian Rhythm/*physiopathology MH - Vasodilation/physiology EDAT- 2007/11/30 09:00 MHDA- 2008/03/28 09:00 CRDT- 2007/11/30 09:00 PHST- 2007/11/30 09:00 [pubmed] PHST- 2008/03/28 09:00 [medline] PHST- 2007/11/30 09:00 [entrez] AID - 00609.2007 [pii] AID - 10.1152/ajpregu.00609.2007 [doi] PST - ppublish SO - Am J Physiol Regul Integr Comp Physiol. 2008 Feb;294(2):R630-8. doi: 10.1152/ajpregu.00609.2007. Epub 2007 Nov 28. PMID- 29804150 OWN - NLM STAT- MEDLINE DCOM- 20190111 LR - 20200306 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 37 IP - 9 DP - 2018 Sep TI - Vascular complications in systemic sclerosis: a prospective cohort study. PG - 2429-2437 LID - 10.1007/s10067-018-4148-5 [doi] AB - Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician's ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. At baseline, patients lacked evidence of PH and were without a current DL. At each 6-month visit, the patient was examined for signs/symptoms of PH and/or a DL. The primary outcomes were (1) PH defined as a mean pulmonary artery pressure ≥ 25 mmHg by right heart catheterization and (2) ≥ 1 DL defined as new onset of severe vascular compromise. Thirty patients (10%) developed PH (11 group 1/PAH, 4 group II, 15 group III) and 69 developed DL. The average time from enrollment until diagnosis of PH was 3.2 ± 2 years. In multivariable analyses, patients who developed PH were more likely to have diffuse disease (HR 3.2, p = 0.004), a forced vital capacity (FVC)/diffusing capacity of the lungs for carbon monoxide (DLCO) ratio > 1.6 (HR 1.7, p = 0.008), and elevated RVSP (HR = 1.07, p = 0.007). Patients who developed PAH were more likely to have a FVC/DLCO ratio > 1.6 (HR = 5.8, p = 0.014), and patients who developed group III PH were less likely to have an elevated FVC (HR = 0.92, p = 0.001). Patients were more likely to develop a DL if they had a history of prior DL (HR = 7.0, p < 0.001), or were men (HR = 2.3, p = 0.007). In a prevalent cohort of scleroderma patients, individuals who develop PH or DL have simple to measure clinical features that can predict these complications years before they occur. FAU - Mecoli, Christopher A AU - Mecoli CA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Boin, Francesco AU - Boin F AD - Division of Rheumatology, Department of Medicine, University of California, San Francisco, CA, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, MFL Bldg, Center Tower, Suite 4100, Baltimore, MD, 21224, USA. lhummers@jhmi.edu. LA - eng GR - P50 HL084946/HL/NHLBI NIH HHS/United States GR - K23 AR052742/AR/NIAMS NIH HHS/United States GR - T32AR048522/AR/NIAMS NIH HHS/United States GR - P50-HL084946-01/HL/NHLBI NIH HHS/United States GR - T32 AR048522/AR/NIAMS NIH HHS/United States GR - 5K23AR52742-5/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Observational Study DEP - 20180526 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Female MH - Fingers/*blood supply MH - Humans MH - Hypertension, Pulmonary/*etiology MH - Ischemia/*etiology MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/etiology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications PMC - PMC6166238 MID - NIHMS984740 OTO - NOTNLM OT - Cohort studies OT - Pulmonary hypertension OT - Scleroderma OT - Wounds and injuries EDAT- 2018/05/29 06:00 MHDA- 2019/01/12 06:00 PMCR- 2019/09/01 CRDT- 2018/05/28 06:00 PHST- 2018/03/22 00:00 [received] PHST- 2018/05/11 00:00 [accepted] PHST- 2018/05/08 00:00 [revised] PHST- 2018/05/29 06:00 [pubmed] PHST- 2019/01/12 06:00 [medline] PHST- 2018/05/28 06:00 [entrez] PHST- 2019/09/01 00:00 [pmc-release] AID - 10.1007/s10067-018-4148-5 [pii] AID - 10.1007/s10067-018-4148-5 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Sep;37(9):2429-2437. doi: 10.1007/s10067-018-4148-5. Epub 2018 May 26. PMID- 21478179 OWN - NLM STAT- MEDLINE DCOM- 20110726 LR - 20221207 IS - 1465-3621 (Electronic) IS - 0368-2811 (Linking) VI - 41 IP - 6 DP - 2011 Jun TI - A case of progressive digital ischemia after early withdrawal of gemcitabine and S-1 in a patient with systemic sclerosis. PG - 803-6 LID - 10.1093/jjco/hyr045 [doi] AB - The safety of chemotherapy for patients with systemic sclerosis is unclear, and there are few published reports documenting the side effects of chemotherapy in patients with this condition. Here, we report the case of a patient with systemic sclerosis who developed severe digital ischemia during combination gemcitabine/S-1 chemotherapy for pancreatic cancer. In spite of aggressive treatment, the digital ischemia progressively worsened and gangrenous changes developed in multiple fingers and toes. In this patient, the systemic sclerosis had been well controlled, with no digital ischemic symptoms for the previous 6 years, so this progressive clinical course in spite of aggressive treatment strongly suggests that the chemotherapy triggered or aggravated the digital necrosis. To the best of our knowledge, this is only the third reported case of a patient with systemic sclerosis developing digital necrosis after gemcitabine-based chemotherapy. The incidence of digital necrosis during chemotherapy in patients with systemic sclerosis is unknown, and the mechanism by which it occurs is unclear, but the three reports published to date, including the present case, suggest that physicians should be very cautious about administering gemcitabine-based chemotherapy to patients with systemic sclerosis. Any resulting digital ischemia might be refractory to treatment and worsen progressively, even if chemotherapy is withdrawn in the early stages of digital ischemia. FAU - Zaima, C AU - Zaima C AD - Kyoto University Hospital, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, Japan. FAU - Kanai, M AU - Kanai M FAU - Ishikawa, S AU - Ishikawa S FAU - Kawaguchi, Y AU - Kawaguchi Y FAU - Masui, T AU - Masui T FAU - Mori, Y AU - Mori Y FAU - Nishimura, T AU - Nishimura T FAU - Matsumoto, S AU - Matsumoto S FAU - Yanagihara, K AU - Yanagihara K FAU - Chiba, T AU - Chiba T FAU - Mimori, T AU - Mimori T LA - eng PT - Case Reports PT - Journal Article DEP - 20110407 PL - England TA - Jpn J Clin Oncol JT - Japanese journal of clinical oncology JID - 0313225 RN - 0 (Antimetabolites, Antineoplastic) RN - 0 (Drug Combinations) RN - 0W860991D6 (Deoxycytidine) RN - 150863-82-4 (S 1 (combination)) RN - 1548R74NSZ (Tegafur) RN - 5VT6420TIG (Oxonic Acid) RN - 0 (Gemcitabine) SB - IM MH - Aged MH - Antimetabolites, Antineoplastic/administration & dosage/*adverse effects MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Deoxycytidine/administration & dosage/adverse effects/*analogs & derivatives MH - Disease Progression MH - Drug Administration Schedule MH - Drug Combinations MH - Fatal Outcome MH - Fingers/*pathology MH - Gangrene/chemically induced MH - Humans MH - Ischemia/*chemically induced/drug therapy/etiology/therapy MH - Male MH - Necrosis/chemically induced MH - Oxonic Acid/administration & dosage/*adverse effects MH - Pancreatic Neoplasms/complications/drug therapy/pathology MH - Peritoneal Neoplasms/drug therapy/secondary MH - Raynaud Disease/chemically induced MH - Scleroderma, Systemic/*complications MH - Tegafur/administration & dosage/*adverse effects MH - Toes/*pathology MH - Gemcitabine EDAT- 2011/04/12 06:00 MHDA- 2011/07/27 06:00 CRDT- 2011/04/12 06:00 PHST- 2011/04/12 06:00 [entrez] PHST- 2011/04/12 06:00 [pubmed] PHST- 2011/07/27 06:00 [medline] AID - hyr045 [pii] AID - 10.1093/jjco/hyr045 [doi] PST - ppublish SO - Jpn J Clin Oncol. 2011 Jun;41(6):803-6. doi: 10.1093/jjco/hyr045. Epub 2011 Apr 7. PMID- 23026233 OWN - NLM STAT- MEDLINE DCOM- 20140205 LR - 20250529 IS - 1532-0480 (Electronic) IS - 1532-0464 (Print) IS - 1532-0464 (Linking) VI - 46 IP - 2 DP - 2013 Apr TI - A graph-based recovery and decomposition of Swanson's hypothesis using semantic predications. PG - 238-51 LID - S1532-0464(12)00151-7 [pii] LID - 10.1016/j.jbi.2012.09.004 [doi] AB - OBJECTIVES: This paper presents a methodology for recovering and decomposing Swanson's Raynaud Syndrome-Fish Oil hypothesis semi-automatically. The methodology leverages the semantics of assertions extracted from biomedical literature (called semantic predications) along with structured background knowledge and graph-based algorithms to semi-automatically capture the informative associations originally discovered manually by Swanson. Demonstrating that Swanson's manually intensive techniques can be undertaken semi-automatically, paves the way for fully automatic semantics-based hypothesis generation from scientific literature. METHODS: Semantic predications obtained from biomedical literature allow the construction of labeled directed graphs which contain various associations among concepts from the literature. By aggregating such associations into informative subgraphs, some of the relevant details originally articulated by Swanson have been uncovered. However, by leveraging background knowledge to bridge important knowledge gaps in the literature, a methodology for semi-automatically capturing the detailed associations originally explicated in natural language by Swanson, has been developed. RESULTS: Our methodology not only recovered the three associations commonly recognized as Swanson's hypothesis, but also decomposed them into an additional 16 detailed associations, formulated as chains of semantic predications. Altogether, 14 out of the 19 associations that can be attributed to Swanson were retrieved using our approach. To the best of our knowledge, such an in-depth recovery and decomposition of Swanson's hypothesis has never been attempted. CONCLUSION: In this work therefore, we presented a methodology to semi-automatically recover and decompose Swanson's RS-DFO hypothesis using semantic representations and graph algorithms. Our methodology provides new insights into potential prerequisites for semantics-driven Literature-Based Discovery (LBD). Based on our observations, three critical aspects of LBD include: (1) the need for more expressive representations beyond Swanson's ABC model; (2) an ability to accurately extract semantic information from text; and (3) the semantic integration of scientific literature and structured background knowledge. CI - Published by Elsevier Inc. FAU - Cameron, Delroy AU - Cameron D AD - Ohio Center of Excellence in Knowledge-enabled Computing (Kno.e.sis), Wright State University, Dayton, OH 45435, USA. delroy@knoesis.org FAU - Bodenreider, Olivier AU - Bodenreider O FAU - Yalamanchili, Hima AU - Yalamanchili H FAU - Danh, Tu AU - Danh T FAU - Vallabhaneni, Sreeram AU - Vallabhaneni S FAU - Thirunarayan, Krishnaprasad AU - Thirunarayan K FAU - Sheth, Amit P AU - Sheth AP FAU - Rindflesch, Thomas C AU - Rindflesch TC LA - eng GR - R21 DA030571/DA/NIDA NIH HHS/United States GR - Z99 LM999999/ImNIH/Intramural NIH HHS/United States GR - R21 DA030571-01A1/DA/NIDA NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20120928 PL - United States TA - J Biomed Inform JT - Journal of biomedical informatics JID - 100970413 SB - IM MH - Blood Viscosity MH - Computational Biology/*methods/trends MH - Data Mining/*methods/trends MH - Humans MH - Knowledge Discovery/*methods MH - *Models, Theoretical MH - Platelet Aggregation MH - Raynaud Disease MH - *Semantics PMC - PMC4031661 MID - NIHMS411603 EDAT- 2012/10/03 06:00 MHDA- 2014/02/06 06:00 PMCR- 2014/05/23 CRDT- 2012/10/03 06:00 PHST- 2012/03/22 00:00 [received] PHST- 2012/09/05 00:00 [revised] PHST- 2012/09/08 00:00 [accepted] PHST- 2012/10/03 06:00 [entrez] PHST- 2012/10/03 06:00 [pubmed] PHST- 2014/02/06 06:00 [medline] PHST- 2014/05/23 00:00 [pmc-release] AID - S1532-0464(12)00151-7 [pii] AID - 10.1016/j.jbi.2012.09.004 [doi] PST - ppublish SO - J Biomed Inform. 2013 Apr;46(2):238-51. doi: 10.1016/j.jbi.2012.09.004. Epub 2012 Sep 28. PMID- 31365695 OWN - NLM STAT- MEDLINE DCOM- 20190903 LR - 20200225 IS - 1414-431X (Electronic) IS - 0100-879X (Print) IS - 0100-879X (Linking) VI - 52 IP - 8 DP - 2019 TI - Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension. PG - e8513 LID - S0100-879X2019000800601 [pii] LID - 10.1590/1414-431X20198513 [doi] LID - e8513 AB - Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=-0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients. FAU - Andrade, F M AU - Andrade FM AUID- ORCID: 0000-0002-3947-6458 AD - Programa de Pós-Graduação em Ciências da Reabilitação, Centro Universitário Augusto Motta, Rio de Janeiro, RJ, Brasil. FAU - Oliveira, A D AU - Oliveira AD AUID- ORCID: 0000-0001-7770-2789 AD - Programa de Pós-Graduação em Ciências da Reabilitação, Centro Universitário Augusto Motta, Rio de Janeiro, RJ, Brasil. FAU - Lopes, A J AU - Lopes AJ AUID- ORCID: 0000-0001-8598-4878 AD - Programa de Pós-Graduação em Ciências da Reabilitação, Centro Universitário Augusto Motta, Rio de Janeiro, RJ, Brasil. AD - Programa de Pós-Graduação em Ciências Médicas, Faculdade de Ciências Médicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brasil. LA - eng PT - Journal Article DEP - 20190729 PL - Brazil TA - Braz J Med Biol Res JT - Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas JID - 8112917 SB - IM MH - Adult MH - Case-Control Studies MH - Exercise Tolerance/*physiology MH - Female MH - Humans MH - Hypertension, Pulmonary/etiology/*physiopathology MH - Lung/*physiology/physiopathology MH - Lung Diseases, Interstitial/etiology/*physiopathology MH - Lung Volume Measurements/methods MH - Middle Aged MH - Pulmonary Ventilation MH - Raynaud Disease/complications MH - Respiratory Function Tests/*methods MH - Scleroderma, Systemic/*complications MH - Tomography, X-Ray Computed/methods MH - Vital Capacity/physiology MH - Walk Test/methods PMC - PMC6668959 EDAT- 2019/08/01 06:00 MHDA- 2019/09/04 06:00 PMCR- 2019/07/29 CRDT- 2019/08/01 06:00 PHST- 2019/01/16 00:00 [received] PHST- 2019/06/05 00:00 [accepted] PHST- 2019/08/01 06:00 [entrez] PHST- 2019/08/01 06:00 [pubmed] PHST- 2019/09/04 06:00 [medline] PHST- 2019/07/29 00:00 [pmc-release] AID - S0100-879X2019000800601 [pii] AID - 10.1590/1414-431X20198513 [doi] PST - ppublish SO - Braz J Med Biol Res. 2019;52(8):e8513. doi: 10.1590/1414-431X20198513. Epub 2019 Jul 29. PMID- 19958891 OWN - NLM STAT- MEDLINE DCOM- 20100118 LR - 20221207 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 122 IP - 12 DP - 2009 Dec TI - Cryofibrinogenemia: new insights into clinical and pathogenic features. PG - 1128-35 LID - 10.1016/j.amjmed.2009.03.040 [doi] AB - OBJECTIVE: Cryofibrinogenemia is an under-recognized cryoprotein that can be life-threatening when untreated. Our aim was to describe the prevalence and clinical findings of patients with cryofibrinogenemia and to clarify the mechanisms involved. METHODS: Between 1996 and 2006, 2312 patients were tested for cryofibrinogenemia in a single university hospital. A total of 515 patients had positive test results, of whom 455 (88.3%) had an associated cryoglobulin. RESULTS: Sixty patients (11.7%) with persistent cryofibrinogenemia and without cryoglobulin were included in the study. Main clinical manifestations related to cryofibrinogenemia included purpura (46.6%), skin necrosis (36.6%), and arthralgia (31.6%) with cold sensitivity in 40%. Overall thrombotic events occurred in up to 40% of cases. Cryofibrinogen plasma concentration was 2 times greater in patients with thrombotic events (P=.012). Complications included gangrene (5%), septicemia (5%), and leg amputation (3.3%). Complete remission of cryofibrinogenemia was achieved in 78% of patients receiving antithrombotic agents, steroids, or immunosuppressants, whereas 41.6% of patients experienced a relapse after a median time of 9 months (range 7-42 months). After a mean follow-up of 85 months, 3 patients died of sepsis (n=2) and cardiovascular disease (n=1). Fibrinolysis status analyzed in a patient with cryofibrinogenemia showed an increase in fibrinolysis inhibitor levels, plasminogen activator inhibitor-1, alpha-2 macroglobulin, and euglobulin lysis time, which normalized after fibrinolytic therapy. CONCLUSION: Essential cryofibrinogenemia represents 12% of all the cryoproteins at Pitie-Salpêtriere Hospital. Thrombotic events are frequent and could be associated with the amount of plasma cryofibrinogen. Defects in the fibrinolysis process might lead to cryofibrinogen accumulation and clotting in small and medium arteries. FAU - Saadoun, David AU - Saadoun D AD - Pierre et Marie Curie-Paris 6 University, Department of Internal Medicine, Hôpital Pitié-Salpétrière, Paris, France. FAU - Elalamy, Ismail AU - Elalamy I FAU - Ghillani-Dalbin, Pascale AU - Ghillani-Dalbin P FAU - Sene, Damien AU - Sene D FAU - Delluc, Aurelien AU - Delluc A FAU - Cacoub, Patrice AU - Cacoub P LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Cryoglobulins) RN - 0 (Fibrinolytic Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Plasminogen Activator Inhibitor 1) RN - 0 (SERPINE1 protein, human) RN - 0 (Serum Globulins) RN - 0 (alpha-Macroglobulins) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Amputation, Surgical/statistics & numerical data MH - Arthralgia/epidemiology MH - Cold Temperature/adverse effects MH - Cryoglobulinemia/*diagnosis/epidemiology/etiology/*therapy MH - Cryoglobulins/analysis MH - Female MH - Fibrinolysis MH - Fibrinolytic Agents/therapeutic use MH - Gangrene MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Leg/surgery MH - Male MH - Middle Aged MH - Necrosis MH - Plasmapheresis MH - Plasminogen Activator Inhibitor 1 MH - Purpura/epidemiology MH - Raynaud Disease/epidemiology MH - Recurrence MH - Remission Induction MH - Sepsis/epidemiology/mortality MH - Serum Globulins/metabolism MH - Skin/pathology MH - Thrombosis/epidemiology MH - Urticaria/epidemiology MH - alpha-Macroglobulins/analysis EDAT- 2009/12/05 06:00 MHDA- 2010/01/19 06:00 CRDT- 2009/12/05 06:00 PHST- 2008/11/09 00:00 [received] PHST- 2009/02/21 00:00 [revised] PHST- 2009/03/30 00:00 [accepted] PHST- 2009/12/05 06:00 [entrez] PHST- 2009/12/05 06:00 [pubmed] PHST- 2010/01/19 06:00 [medline] AID - S0002-9343(09)00774-8 [pii] AID - 10.1016/j.amjmed.2009.03.040 [doi] PST - ppublish SO - Am J Med. 2009 Dec;122(12):1128-35. doi: 10.1016/j.amjmed.2009.03.040. PMID- 24709277 OWN - NLM STAT- MEDLINE DCOM- 20150423 LR - 20220408 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 44 IP - 1 DP - 2014 Aug TI - Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. PG - 55-62 LID - S0049-0172(14)00042-0 [pii] LID - 10.1016/j.semarthrit.2014.03.002 [doi] AB - OBJECTIVES: PHAROS registry is a prospective longitudinal cohort study to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). METHODS: "At-risk" pulmonary arterial hypertension (PAH) is defined by these entry criteria: echocardiogram (echo) systolic pulmonary arterial pressure (sPAP) >40 mmHg, diffusion lung capacity of carbon monoxide (DLco) <55% predicted, or ratio of percentage forced vital capacity (FVC)/percentage DLco >1.6, as measured by pulmonary function testing (PFT). Patients were followed up annually and right heart catheterization (RHC) performed if PH was suspected. We used descriptive statistics and Kaplan-Meier estimate of time to PH diagnosis. RESULTS: A total of 251 "at-risk" subjects were enrolled between 2005 and 2012 and followed up for mean of 2.5 ± 1.2 years. The mean age at entry was 56.7 ± 11.0 and disease duration was 9.9 ± 8.7 years. Overall, 82 patients had RHC, and 35 were confirmed to have new PH. There were no differences in age, gender, SSc subtypes, antibodies, and disease duration between the "at-risk" and new PH groups. Using Kaplan-Meier survival, the time to PH was 10% at 2 years, 13% at 3 years, and 25% at 5 years. Most new PH patients at entry met the PFT criteria (76%), had significantly higher sPAP (p = 0.013), had shorter 6-min walk distance, and had exercise-induced hypoxia (p = 0.003) than "at-risk" PAH group. CONCLUSIONS: A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP > 40 were strongly associated with future PH, though RHC was necessary to confirm PH. This ongoing prospective study confirms that these high-risk factors do predict future PH. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Hsu, Vivien M AU - Hsu VM AD - Department of Medicine, Division of Rheumatology, Adult Clinical Research Center, Rutgers-RWJ Medical School, Acute Care Building, 51 French St, New Brunswick, NJ 08903. Electronic address: hsuvm@rwjms.rutgers.edu. FAU - Chung, Lorinda AU - Chung L AD - Department of Medicine, Division of Immnunology & Rheumatology, Stanford University, Stanford, CA. FAU - Hummers, Laura K AU - Hummers LK AD - Department of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, MD. FAU - Wigley, Fredrick AU - Wigley F AD - Department of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, MD. FAU - Simms, Robert AU - Simms R AD - Department of Medicine, Division of Rheumatology, Boston University, Boston, MA. FAU - Bolster, Marcy AU - Bolster M AD - Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, SC. FAU - Silver, Rick AU - Silver R AD - Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, SC. FAU - Fischer, Aryeh AU - Fischer A AD - Department of Medicine, Division of Rheumatology, National Jewish Health, University of Colorado, Denver, CO. FAU - Hinchcliff, Monique E AU - Hinchcliff ME AD - Department of Medicine, Division of Rheumatology, Northwestern University, Chicago, IL. FAU - Varga, John AU - Varga J AD - Department of Medicine, Division of Rheumatology, Northwestern University, Chicago, IL. FAU - Goldberg, Avram Z AU - Goldberg AZ AD - Department of Medicine, Division of Rheumatology, North Shore-LIJ Health System, Long Island, NY. FAU - Derk, Chris T AU - Derk CT AD - Department of Medicine, Division of Rheumatology, Jefferson Medical College, Philadelphia, PA. FAU - Schiopu, Elena AU - Schiopu E AD - Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, MI. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, MI. FAU - Shapiro, Lee S AU - Shapiro LS AD - Department of Medicine, Division of Rheumatology, Albany Medical College, Albany, NY. FAU - Domsic, Robyn T AU - Domsic RT AD - Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA. FAU - Medsger, Thomas AU - Medsger T AD - Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA. FAU - Mayes, Maureen D AU - Mayes MD AD - Department of Medicine, Division of Rheumatology, University of Texas, Houston, TX. FAU - Furst, Daniel AU - Furst D AD - Department of Medicine, Division of Rheumatology, University of California, Los Angeles, CA. FAU - Csuka, Mary E AU - Csuka ME AD - Department of Medicine, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI. FAU - Molitor, Jerry A AU - Molitor JA AD - Department of Medicine, Division of Rheumatology, University of Minnesota, Minneapolis, MN. FAU - Alkassab, Firas AU - Alkassab F AD - Department of Medicine, Division of Rheumatology, University of Massachusetts Medical School, Worcester, MA. FAU - Steen, Virginia D AU - Steen VD AD - Department of Medicine, Division of Rheumatology, Immunology & Allergy, Georgetown University, Washington, DC. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140305 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Hypertension, Pulmonary/*etiology MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Prospective Studies MH - Raynaud Disease/*etiology MH - Registries MH - Risk MH - Scleroderma, Systemic/*complications OTO - NOTNLM OT - Pulmonary arterial hypertension OT - Registry OT - Scleroderma OT - Systemic EDAT- 2014/04/09 06:00 MHDA- 2015/04/24 06:00 CRDT- 2014/04/09 06:00 PHST- 2013/12/13 00:00 [received] PHST- 2014/02/21 00:00 [revised] PHST- 2014/03/03 00:00 [accepted] PHST- 2014/04/09 06:00 [entrez] PHST- 2014/04/09 06:00 [pubmed] PHST- 2015/04/24 06:00 [medline] AID - S0049-0172(14)00042-0 [pii] AID - 10.1016/j.semarthrit.2014.03.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2014 Aug;44(1):55-62. doi: 10.1016/j.semarthrit.2014.03.002. Epub 2014 Mar 5. PMID- 28831595 OWN - NLM STAT- MEDLINE DCOM- 20180717 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 37 IP - 10 DP - 2017 Oct TI - Juvenile systemic sclerosis: experience from a tertiary care center from India. PG - 1687-1691 LID - 10.1007/s00296-017-3793-3 [doi] AB - Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms). Controlled: ability to perform ADL and absence of B features for at least 6 months. Partly controlled: inability to perform ADL or any of the B features. Non-responsive/progressive disease: presence of both A and any of B features. Thirty-two children (21, girls) diagnosed as systemic sclerosis for whom follow-up of more than 6 months was available were included for this retrospective analysis. Mean (SD) age at presentation was 112.79 (30.05) months, while the median (IQR) delay in diagnosis was 28.5 (9-47.25) months. Of the 32 children 17 (53.12%) had diffuse systemic sclerosis (dSSc), 5 (15.62%) had limited systemic sclerosis (lSSc) and 10 (31.25%) had sclerosis with overlap syndrome. The common clinical features apart from sclerosis/induration proximal to metacarpophalangeal joint were Raynauds phenomenon (n = 22, 68.7%), skin rash (n = 20, 62%), arthritis or arthralgia (n = 16, 50%), and muscular weakness (n = 10, 31.2%). Among those for whom data regarding investigations were available; ANA was positive in 50% (12/24), whereas Anti Scl70 was positive in one out three cases. Treatment regimen included naproxen, methotrexate, calcium channel blockers with or without steroids. HCQ was added in children with skin rash or in children with partial control. Median (IQR) follow-up period was 19.75 (12-31.75) months. With the above treatment protocol, 19 (59.3%) children achieved disease control on treatment, 8 (26.6%) had partial control while 5 (16.6%) showed no response or progressive disease. Esophageal dysmotility and intertitial lung disease (ILD) were documented in three children each. Complication (cataract and herpes zoster) related to immunosuppressive therapy were observed in two children. There was no mortality during the study period. Juvenile Sclerosis though rare is associated with significant morbidities and lacks a curative treatment but a reasonable quality of life to perform daily activities can be achieved using methotrexate and steroid-based immuosuppressive therapy. FAU - Bagri, Narendra Kumar AU - Bagri NK AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. drnarendrabagri@yahoo.co.in. FAU - Raj, Dinesh AU - Raj D AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. FAU - Kaur, Jasmeet AU - Kaur J AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. FAU - Punia, Harish AU - Punia H AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. FAU - Saini, Isha AU - Saini I AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. FAU - Lodha, Rakesh AU - Lodha R AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. FAU - Kabra, S K AU - Kabra SK AD - Division of Pediatric Rheumatology, Department of Pediatrics, All India Institute of Medical Sciences, AIIMS, New Delhi, India. LA - eng PT - Journal Article DEP - 20170822 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Calcium Channel Blockers) RN - 0 (Immunosuppressive Agents) SB - IM MH - Activities of Daily Living MH - Adolescent MH - Anti-Inflammatory Agents/*therapeutic use MH - Arthralgia/*etiology MH - Arthritis/*etiology MH - Calcium Channel Blockers/therapeutic use MH - Child MH - Child, Preschool MH - Disease Progression MH - Female MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - India MH - Male MH - Quality of Life MH - Raynaud Disease/*etiology MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*diagnosis/*drug therapy MH - Tertiary Care Centers MH - Treatment Outcome OTO - NOTNLM OT - Complications OT - Juvenile OT - Pulse dexamethasone OT - Scleroderma EDAT- 2017/08/24 06:00 MHDA- 2018/07/18 06:00 CRDT- 2017/08/24 06:00 PHST- 2017/03/21 00:00 [received] PHST- 2017/08/09 00:00 [accepted] PHST- 2017/08/24 06:00 [pubmed] PHST- 2018/07/18 06:00 [medline] PHST- 2017/08/24 06:00 [entrez] AID - 10.1007/s00296-017-3793-3 [pii] AID - 10.1007/s00296-017-3793-3 [doi] PST - ppublish SO - Rheumatol Int. 2017 Oct;37(10):1687-1691. doi: 10.1007/s00296-017-3793-3. Epub 2017 Aug 22. PMID- 19796555 OWN - NLM STAT- MEDLINE DCOM- 20100105 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 27 IP - 3 Suppl 54 DP - 2009 May-Jun TI - Evidence of a selective nociceptive impairment in systemic sclerosis. PG - 9-14 AB - OBJECTIVE: To test for the autonomic neuropathy in systemic sclerosis (SSc) using cardiovascular reflex evaluation including the "cold face test", which elicits forehead cold receptors (C-fibres). These tests examine the induced bradycardia-hypertensive response and the integrity of nociceptive afferent and parasympathetic-sympathetic efferent pathways. METHODS: Twelve SSc patients were studied; including 5 with the limited cutaneous (lcSSc) involvement, and 7 with diffuse cutaneous involvement (dcSSc). All patients were matched with healthy controls. We performed cardiovascular autonomic tests (tilt-test, Valsalva manoeuver, deep breathing, sustained handgrip and cold face) with continuous monitoring of beat-to-beat blood pressure (BP) and heart rate (HR). Baroreceptor sensitivity index (BRSI) and power spectral analysis (PSA) of heart rate variability (HRV) were also evaluated. RESULTS: SSc patients showed a statistically significant higher HR at rest (p<0.01), a lower increase of diastolic BP during tilt test (p<0.01). They had suboptimal hypertensive and bradycardic response to the cold face test (Systolic BP: p<0.05; Diastolic BP: p<0.01; HR: p=0.08). The Valsalva manoeuver, deep breathing, isometric handgrip, BRSI and PSA of HRV results were within normal limits in the majority of SSc patients. CONCLUSION: In this group of SSc patients cardiovascular reflexes were normal, whereas the cold face test which acts through cutaneous nociceptive sensory fibres was abnormal in almost all patients. These results suggest that insufficiency of epidermal small fibres (C-fibres) is involved in SSc. FAU - Bajocchi, G AU - Bajocchi G AD - Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy. baiocchi.gianluigi@asmn.re.it FAU - Terlizzi, R AU - Terlizzi R FAU - Zanigni, S AU - Zanigni S FAU - Barletta, G AU - Barletta G FAU - Grimaldi, D AU - Grimaldi D FAU - Pierangeli, G AU - Pierangeli G FAU - Cortelli, P AU - Cortelli P LA - eng PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Autonomic Nervous System Diseases/complications/*physiopathology MH - Blood Pressure MH - Cardiovascular System/*innervation/physiopathology MH - Cold Temperature MH - Face/physiology MH - Female MH - Heart Rate MH - Humans MH - Male MH - Middle Aged MH - Pain Measurement MH - Parasympathetic Nervous System/physiopathology MH - Raynaud Disease MH - Reflex, Abnormal MH - Scleroderma, Systemic/complications/diagnosis/*physiopathology MH - Sympathetic Nervous System/physiopathology MH - Valsalva Maneuver EDAT- 2009/12/04 06:00 MHDA- 2010/01/06 06:00 CRDT- 2009/10/03 06:00 PHST- 2009/10/03 06:00 [entrez] PHST- 2009/12/04 06:00 [pubmed] PHST- 2010/01/06 06:00 [medline] AID - 2737 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2009 May-Jun;27(3 Suppl 54):9-14. PMID- 24530379 OWN - NLM STAT- MEDLINE DCOM- 20160104 LR - 20150316 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 98 DP - 2015 Mar TI - Effect of capillaroscopic patterns on the pulse oximetry measurements in systemic sclerosis patients. PG - 183-6 LID - S0026-2862(14)00033-8 [pii] LID - 10.1016/j.mvr.2014.02.002 [doi] AB - Nailfold capillaroscopy is a simple method for determining microvascular damage in systemic sclerosis (SSc). The pulse oximeter is a noninvasive tool that is used to estimate a patient's arterial blood oxygen saturation. The aim of this study was to investigate the effect of capillaroscopic patterns on pulse oximetry measurements in SSc patients. The nailfolds from 2nd to 5th fingers in both hands were examined in all subjects by using capillaroscopy. Patients were categorized according to their capillaroscopy findings in 3 groups as having early, active or late patterns. Oxygen saturation (SpO2) measurements were performed from 2nd to 5th fingers in both hands with a finger probe. We studied 53 SSc patients (F/M: 48/5). According to capillaroscopy findings 18 patients were classified as having early pattern (34.0%), 19 active pattern (35.8%), and 15 late pattern (28.3%). Only 1 (1.9%) patient had normal capillaroscopy findings. SpO2 could not be measured in 47 (11.0%) fingers and in 20 (37.7%) SSc patients. There were 20 (37.7%) patients with a ≥4% difference between the minimum and maximum SpO2 measurements among fingers. There were no difference between the groups of SSc patients defined by capillaroscopy findings in terms of mean maximal SpO2 or mean minimal SpO2 measured from fingers (p NS, for all). Assessment of SpO2 values in patients with SSc is challenging. We did not detect any effect of capillaroscopic patterns on mean SpO2 values. On the other hand ≥4% difference between minimum and maximum SpO2 values measured from fingers of a patient may be considered as an indirect sign of microvascular damage. Assessment of the highest measured SpO2 values among the fingers of a patient may be more suitable in practice. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Akdogan, Ali AU - Akdogan A AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Kilic, Levent AU - Kilic L AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: drleventkilic@yahoo.com. FAU - Dogan, Ismail AU - Dogan I AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Karadag, Omer AU - Karadag O AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Bilgen, Sule Apras AU - Bilgen SA AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Kiraz, Sedat AU - Kiraz S AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Ertenli, Insan AU - Ertenli I AD - Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. LA - eng PT - Journal Article DEP - 20140214 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - S88TT14065 (Oxygen) SB - IM MH - Adult MH - Female MH - Fingers/blood supply MH - Humans MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/blood supply MH - *Oximetry MH - Oxygen/chemistry MH - Raynaud Disease MH - Scleroderma, Systemic/*complications/*diagnosis/*physiopathology EDAT- 2014/02/18 06:00 MHDA- 2016/01/05 06:00 CRDT- 2014/02/18 06:00 PHST- 2013/04/28 00:00 [received] PHST- 2013/12/19 00:00 [revised] PHST- 2014/02/06 00:00 [accepted] PHST- 2014/02/18 06:00 [entrez] PHST- 2014/02/18 06:00 [pubmed] PHST- 2016/01/05 06:00 [medline] AID - S0026-2862(14)00033-8 [pii] AID - 10.1016/j.mvr.2014.02.002 [doi] PST - ppublish SO - Microvasc Res. 2015 Mar;98:183-6. doi: 10.1016/j.mvr.2014.02.002. Epub 2014 Feb 14. PMID- 21337913 OWN - NLM STAT- MEDLINE DCOM- 20110725 LR - 20200326 IS - 0125-877X (Print) IS - 0125-877X (Linking) VI - 28 IP - 4 DP - 2010 Dec TI - Juvenile scleroderma: experience in one institution. PG - 279-86 AB - BACKGROUND: Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group. OBJECTIVE: In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma. METHODS: Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinical manifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes. RESULTS: Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55 +/- 3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after D-penicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions. CONCLUSIONS: While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study. FAU - Lo, Chia-Yi AU - Lo CY AD - Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan. FAU - Shyur, Shyh-Dar AU - Shyur SD FAU - Chu, Szu-Hung AU - Chu SH FAU - Huang, Li-Hsin AU - Huang LH FAU - Kao, Yu-Hsuan AU - Kao YH FAU - Lei, Wei-Te AU - Lei WT FAU - Cheng, Chieh-Han AU - Cheng CH FAU - Lee, Kuo-Hsi AU - Lee KH FAU - Chen, Chen-Kuan AU - Chen CK FAU - Liu, Ling-Chun AU - Liu LC LA - eng PT - Journal Article PL - Thailand TA - Asian Pac J Allergy Immunol JT - Asian Pacific journal of allergy and immunology JID - 8402034 RN - Juvenile-onset scleroderma SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Child MH - Child, Preschool MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Prognosis MH - Proteinuria MH - Raynaud Disease MH - Retrospective Studies MH - Scleroderma, Localized/*diagnosis/*epidemiology/physiopathology MH - Scleroderma, Systemic/diagnosis/epidemiology/physiopathology MH - Sex Factors MH - Taiwan EDAT- 2011/02/23 06:00 MHDA- 2011/07/26 06:00 CRDT- 2011/02/23 06:00 PHST- 2011/02/23 06:00 [entrez] PHST- 2011/02/23 06:00 [pubmed] PHST- 2011/07/26 06:00 [medline] PST - ppublish SO - Asian Pac J Allergy Immunol. 2010 Dec;28(4):279-86. PMID- 28056338 OWN - NLM STAT- MEDLINE DCOM- 20170529 LR - 20181113 IS - 1525-2191 (Electronic) IS - 0002-9440 (Print) IS - 0002-9440 (Linking) VI - 187 IP - 3 DP - 2017 Mar TI - HANAC Col4a1 Mutation in Mice Leads to Skeletal Muscle Alterations due to a Primary Vascular Defect. PG - 505-516 LID - S0002-9440(16)30525-9 [pii] LID - 10.1016/j.ajpath.2016.10.020 [doi] AB - Collagen IV is a major component of basement membranes (BMs). The α1(IV) chain, encoded by the COL4A1 gene, is expressed ubiquitously and associates with the α2(IV) chain to form the α1α1α2(IV) heterotrimer. Several COL4A1 mutations affecting a conformational domain containing integrin-binding sites are responsible for the systemic syndrome of hereditary angiopathy, nephropathy, aneurysms, and cramps (HANAC). To analyze the pathophysiology of HANAC, Col4a1 mutant mice bearing the p.Gly498Val mutation were generated. Analysis of the skeletal muscles of Col4a1(G498V) mutant animals showed morphologic characteristics of a muscular dystrophy phenotype with myofiber atrophy, centronucleation, focal inflammatory infiltrates, and fibrosis. Abnormal ultrastructural aspects of muscle BMs was associated with reduced extracellular secretion of the mutant α1α1α2(IV) trimer. In addition to muscular dystrophic features, endothelial cell defects of the muscle capillaries were observed, with intracytoplasmic accumulation of the mutant α1α1α2(IV) molecules, endoplasmic reticulum cisternae dilation, and up-regulation of endoplasmic reticulum stress markers. Induction of the unfolded protein response in Col4a1 mutant muscle tissue resulted in an excess of apoptosis in endothelial cells. HANAC mutant animals also presented with a muscular functional impairment and increased serum creatine kinase levels reflecting altered muscle fiber sarcolemma. This extensive description of the muscular phenotype of the Col4a1 HANAC murine model suggests a potential contribution of primary endothelial cell defects, together with muscle BM alterations, to the development of COL4A1-related myopathy. CI - Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved. FAU - Guiraud, Simon AU - Guiraud S AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France; Medical Research Council Functional Genomics Unit, Department of Physiology, Anatomy, and Genetics, University of Oxford, Oxford, United Kingdom. FAU - Migeon, Tiffany AU - Migeon T AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France. FAU - Ferry, Arnaud AU - Ferry A AD - Research Center in Myology, Institut de Myologie, the Inserm UMRS974, CNRS FRE3617, Pitié-Salpêtrière Hospital Group, University Pierre and Marie Curie Paris 06, Paris Descartes University, The Sorbonne University, Paris, France. FAU - Chen, Zhiyong AU - Chen Z AD - Mixed Research Unit S1155, INSERM, Paris, France. FAU - Ouchelouche, Souhila AU - Ouchelouche S AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France. FAU - Verpont, Marie-Christine AU - Verpont MC AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France. FAU - Sado, Yoshikazu AU - Sado Y AD - Shigei Medical Research Institute, Okayama, Japan. FAU - Allamand, Valérie AU - Allamand V AD - Research Center in Myology, Institut de Myologie, the Inserm UMRS974, CNRS FRE3617, Pitié-Salpêtrière Hospital Group, University Pierre and Marie Curie Paris 06, Paris Descartes University, The Sorbonne University, Paris, France. FAU - Ronco, Pierre AU - Ronco P AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France; Department of Nephrology and Dialysis, Assistance Publique-Hôpitaux de Paris, Tenon Hospital, Paris, France. FAU - Plaisier, Emmanuelle AU - Plaisier E AD - Mixed Research Unit S1155, INSERM, Paris, France; University Pierre and Marie Curie Paris 06, Sorbonne University, Paris, France; Department of Nephrology and Dialysis, Assistance Publique-Hôpitaux de Paris, Tenon Hospital, Paris, France. Electronic address: emmanuelle.plaisier@tnn.aphp.fr. LA - eng PT - Journal Article DEP - 20170103 PL - United States TA - Am J Pathol JT - The American journal of pathology JID - 0370502 RN - 0 (Col4a1 protein, mouse) RN - 0 (Collagen Type IV) RN - 0 (Dystrophin) RN - 0 (Integrin beta1) RN - 0 (Platelet Endothelial Cell Adhesion Molecule-1) RN - EC 2.7.3.2 (Creatine Kinase) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Animals MH - Apoptosis MH - Blood Vessels/*abnormalities/pathology MH - Body Weight MH - Collagen Type IV/*genetics MH - Creatine Kinase/blood MH - Dystrophin/metabolism MH - Endoplasmic Reticulum Stress MH - Endothelial Cells/pathology/ultrastructure MH - Extracellular Matrix/metabolism MH - Integrin beta1/metabolism MH - Mice MH - Mice, Mutant Strains MH - Muscle Cramp/*genetics MH - Muscle, Skeletal/*blood supply/*pathology/ultrastructure MH - Mutation/*genetics MH - Organ Size MH - Platelet Endothelial Cell Adhesion Molecule-1/metabolism MH - Raynaud Disease/*genetics PMC - PMC5389361 EDAT- 2017/01/06 06:00 MHDA- 2017/05/30 06:00 PMCR- 2017/09/01 CRDT- 2017/01/06 06:00 PHST- 2016/03/14 00:00 [received] PHST- 2016/10/24 00:00 [revised] PHST- 2016/10/25 00:00 [accepted] PHST- 2017/01/06 06:00 [pubmed] PHST- 2017/05/30 06:00 [medline] PHST- 2017/01/06 06:00 [entrez] PHST- 2017/09/01 00:00 [pmc-release] AID - S0002-9440(16)30525-9 [pii] AID - 10.1016/j.ajpath.2016.10.020 [doi] PST - ppublish SO - Am J Pathol. 2017 Mar;187(3):505-516. doi: 10.1016/j.ajpath.2016.10.020. Epub 2017 Jan 3. PMID- 29802482 OWN - NLM STAT- MEDLINE DCOM- 20190110 LR - 20190110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 8 DP - 2018 Aug TI - Systemic sclerosis-rheumatoid arthritis overlap syndrome complicated with Sweet's syndrome. PG - 2281-2284 LID - 10.1007/s10067-018-4150-y [doi] AB - Herein, we report a case of a 34-year-old woman with systemic sclerosis (SSc)-rheumatoid arthritis (RA) overlap syndrome (OS) complicated with Sweet's syndrome. OS has been defined as entities satisfying classification criteria of at least two connective tissue diseases (CTD) occurring at the same or at different times in the same patient. The CTD include RA, SSc, systemic lupus erythematosus (SLE), polymyositis, and dermatomyositis. Sweet's syndrome also known as acute febrile neutrophilic dermatosis was first described by Robert Sweet in 1964. Sweet's syndrome is characterized by fever, neutrophilia, erythematous skin lesions, and a diffuse dermal infiltrate of mature neutrophils. There are sets of associations that we will discuss in this article between OS and Sweet's syndrome. FAU - Zhu, T AU - Zhu T AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. FAU - Zhao, W L AU - Zhao WL AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. FAU - Zeng, Y P AU - Zeng YP AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. FAU - Liu, Y H AU - Liu YH AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. FAU - Jin, H Z AU - Jin HZ AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. FAU - Li, L AU - Li L AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China. lilipumch2007@sina.com. LA - eng GR - 81371731/National Natural Science Foundation of China/ GR - 2016zlgc0106/Education Reform Projects of Peking Union Medical College/ PT - Case Reports PT - Journal Article DEP - 20180525 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Arthritis, Rheumatoid/drug therapy/*pathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/drug therapy/*pathology MH - Raynaud Disease MH - Scleroderma, Systemic/drug therapy/*pathology MH - Sweet Syndrome/*complications MH - Undifferentiated Connective Tissue Diseases/drug therapy/*pathology OTO - NOTNLM OT - Overlap syndrome OT - Rheumatoid arthritis OT - Sweet’s syndrome OT - Systemic sclerosis EDAT- 2018/05/29 06:00 MHDA- 2019/01/11 06:00 CRDT- 2018/05/27 06:00 PHST- 2018/02/02 00:00 [received] PHST- 2018/05/15 00:00 [accepted] PHST- 2018/04/27 00:00 [revised] PHST- 2018/05/29 06:00 [pubmed] PHST- 2019/01/11 06:00 [medline] PHST- 2018/05/27 06:00 [entrez] AID - 10.1007/s10067-018-4150-y [pii] AID - 10.1007/s10067-018-4150-y [doi] PST - ppublish SO - Clin Rheumatol. 2018 Aug;37(8):2281-2284. doi: 10.1007/s10067-018-4150-y. Epub 2018 May 25. PMID- 39711361 OWN - NLM STAT- MEDLINE DCOM- 20250428 LR - 20250519 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 3 DP - 2025 Mar TI - Nailfold videocapillaroscopy findings and associations with organ involvement in mixed connective tissue disease. PG - 418-424 LID - 10.55563/clinexprheumatol/xshsd7 [doi] AB - OBJECTIVES: To investigate nailfold videocapillaroscopy (NVC) abnormalities in mixed connective tissue disease (MCTD). METHODS: Patients with MCTD followed at the Rheumatology Department in Cochin Hospital (Paris, France) were identified based on individual record review. Diagnosis of MCTD required fulfillment of one of the three sets of classification criteria. Clinical and laboratory data were collected and NVC was performed on 4 fingers of both hands by one assessor. NVC patterns were analysed by two independent observers. RESULTS: We identified 51 MCTD patients [mean age 51±12 years, 86% female, 31% had interstitial lung disease (ILD)]. NVC images were available for 40 patients. Three different NVC patterns were identified: 'normal' (15 %); 'non-specific microangiopathy' (40%) and 'scleroderma pattern' (45%). 'Scleroderma pattern' was associated with skin sclerosis (9/18 vs. 5/32; p=0.008) and digital ulcers (6/18 vs. 2/32; p=0.017). We observed a reduction in the number of capillaries in patients with ILD (4.80±1.87 vs. 6.03±1.47; p=0.039). Patients with severe reduction of capillary density (≤4/mm) were more likely to have ILD (5/7 vs. 5/33; p=0.002). Neoangiogenesis was more frequent in patients with ILD (6/13 vs. 4/27; p=0.034). The association between severe reduction of capillary density and ILD was observed independently of the presence of the 'scleroderma pattern' and skin sclerosis. CONCLUSIONS: We identified three NVC patterns in MCTD patients. 'Scleroderma pattern' was associated with presence of skin sclerosis and digital ulcers. Severe capillary loss was significantly associated with the presence of ILD. Our results indicates that NVC may be helpful for disease risk stratification in MCTD. FAU - Kasser, Camille AU - Kasser C AD - Service de Rhumatologie, Hôpital Cochin, AP-HP Centre Université Paris Cité, Paris, France. FAU - Boleto, Gonçalo AU - Boleto G AD - Service de Rhumatologie, Hôpital Cochin, AP-HP Centre Université Paris Cité, Paris, France; and Department of Rheumatology, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal. FAU - Allanore, Yannick AU - Allanore Y AD - Service de Rhumatologie, Hôpital Cochin, AP-HP Centre Université Paris Cité, Paris, and INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France. FAU - Avouac, Jérôme AU - Avouac J AD - Service de Rhumatologie, Hôpital Cochin, AP-HP Centre Université Paris Cité, Paris, and INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France. jerome.avouac@aphp.fr. LA - eng PT - Journal Article DEP - 20241221 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Female MH - *Microscopic Angioscopy/methods MH - Middle Aged MH - Male MH - *Capillaries/pathology/diagnostic imaging MH - *Nails/blood supply MH - Adult MH - *Mixed Connective Tissue Disease/complications/diagnosis/pathology/physiopathology/diagnostic imaging MH - Aged MH - *Lung Diseases, Interstitial/etiology MH - Predictive Value of Tests MH - Scleroderma, Systemic MH - Retrospective Studies MH - Skin Ulcer/etiology/pathology MH - Raynaud Disease EDAT- 2024/12/23 06:20 MHDA- 2025/03/12 18:17 CRDT- 2024/12/23 05:03 PHST- 2024/02/27 00:00 [received] PHST- 2024/09/12 00:00 [accepted] PHST- 2025/03/12 18:17 [medline] PHST- 2024/12/23 06:20 [pubmed] PHST- 2024/12/23 05:03 [entrez] AID - 20964 [pii] AID - 10.55563/clinexprheumatol/xshsd7 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Mar;43(3):418-424. doi: 10.55563/clinexprheumatol/xshsd7. Epub 2024 Dec 21. PMID- 36922284 OWN - NLM STAT- MEDLINE DCOM- 20230615 LR - 20230615 IS - 1872-7131 (Electronic) IS - 0387-7604 (Linking) VI - 45 IP - 7 DP - 2023 Aug TI - Clinical characteristics of muscle cramps in hereditary angiopathy with nephropathy, aneurysms, and muscle cramps syndrome associated with a novel COL4A1 pathogenic variant: A family case study. PG - 390-394 LID - S0387-7604(23)00043-8 [pii] LID - 10.1016/j.braindev.2023.02.008 [doi] AB - BACKGROUND: Muscle cramps are a common problem characterized by a sudden, painful, and involuntary contraction of a muscle or muscle group. Most muscle cramps develop in the calf muscles, particularly in situations of prolonged exercise; however, some may be related to underlying systemic conditions such as the hereditary angiopathy with nephropathy, aneurysms, and muscle cramps (HANAC) syndrome. Muscle cramps appear to be the initial symptom of the HANAC syndrome; however, the clinical characteristics of these muscle cramps have rarely been described in detail. CASE PRESENTATION: We report a familial case of autosomal-dominant muscle cramps in four members of a Japanese family spanning across three generations. The muscle cramps were recognized as systemic symptoms of the HANAC syndrome associated with a novel COL4A1 pathogenic variant, NM_001845:c.1538G > A, p.(Gly513Asp). The four affected individuals indicated that the first episodes of the muscle cramps occurred in early childhood. In addition, they reported that the muscle cramps are characterized by an abrupt onset of severe pain without muscle contraction. The painful recurrent attacks occurred spontaneously in various muscles throughout the body, but rarely in the calf muscle. The muscle pain lasts for several minutes, cannot be ameliorated by stretching the affected muscle, and leaves a feeling of discomfort that lasts for 24-48 h. The serum creatine kinase levels of the patients were persistently elevated; however, their electromyography results did not reveal any specific abnormalities. CONCLUSIONS: Recognition of the clinical characteristics of the muscle cramps in the HANAC syndrome may facilitate early diagnosis of the syndrome and enable proper treatment of the patients, improve their long-term outcomes, and facilitate the design and adaption of appropriate genetic counseling. CI - Copyright © 2023 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved. FAU - Haga, Shunsuke AU - Haga S AD - Department of Pediatrics, Asahikawa Medical University, Hokkaido, Japan. Electronic address: haga5p@asahikawa-med.ac.jp. FAU - Takeguchi, Ryo AU - Takeguchi R AD - Department of Pediatrics, Asahikawa Medical University, Hokkaido, Japan. FAU - Tanaka, Ryosuke AU - Tanaka R AD - Department of Pediatrics, Asahikawa Medical University, Hokkaido, Japan. FAU - Satake, Akira AU - Satake A AD - Department of Pediatrics, Nakashibetsu Town Hospital, Hokkaido, Japan. FAU - Makita, Yoshio AU - Makita Y AD - Department of Genetic Counseling, Asahikawa Medical University Hospital, Hokkaido, Japan. FAU - Yanagi, Kumiko AU - Yanagi K AD - Department of Genome Medicine, National Center for Child Health and Development, Tokyo, Japan. FAU - Kaname, Tadashi AU - Kaname T AD - Department of Genome Medicine, National Center for Child Health and Development, Tokyo, Japan. FAU - Takahashi, Satoru AU - Takahashi S AD - Department of Pediatrics, Asahikawa Medical University, Hokkaido, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20230314 PL - Netherlands TA - Brain Dev JT - Brain & development JID - 7909235 RN - 0 (Collagen Type IV) RN - 0 (COL4A1 protein, human) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Muscle Cramp/genetics MH - *Aneurysm/complications MH - Raynaud Disease MH - *Kidney Diseases MH - Mutation/genetics MH - Child, Preschool MH - Collagen Type IV/genetics MH - Syndrome MH - Humans OTO - NOTNLM OT - COL4A1 OT - Familial case OT - Hereditary angiopathy with nephropathy, aneurysms, and muscle cramps syndrome OT - Muscle cramp COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2023/03/16 06:00 MHDA- 2023/06/15 06:42 CRDT- 2023/03/15 23:04 PHST- 2022/11/08 00:00 [received] PHST- 2023/02/13 00:00 [revised] PHST- 2023/02/26 00:00 [accepted] PHST- 2023/06/15 06:42 [medline] PHST- 2023/03/16 06:00 [pubmed] PHST- 2023/03/15 23:04 [entrez] AID - S0387-7604(23)00043-8 [pii] AID - 10.1016/j.braindev.2023.02.008 [doi] PST - ppublish SO - Brain Dev. 2023 Aug;45(7):390-394. doi: 10.1016/j.braindev.2023.02.008. Epub 2023 Mar 14. PMID- 25712813 OWN - NLM STAT- MEDLINE DCOM- 20160414 LR - 20161125 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 44 IP - 6 DP - 2015 Jun TI - Extra-cranial giant cell arteritis and Takayasu arteritis: How similar are they? PG - 724-8 LID - S0049-0172(15)00006-2 [pii] LID - 10.1016/j.semarthrit.2015.01.005 [doi] AB - OBJECTIVE: To compare clinical and imaging characteristics of patients with giant cell arteritis (GCA) and upper extremity (UE) arterial involvement to patients with Takayasu arteritis (TAK). METHODS: A cohort of patients seen at the Mayo Clinic with TAK diagnosed between 1984 and 2009 and a cohort of patients with GCA and UE arterial involvement diagnosed between 1999 and 2008 were studied. RESULTS: The TAK cohort consisted of 125 patients (91% female); the mean age (±SD) at diagnosis was 30.9 (±10) years. The cohort of patients with GCA and UE involvement comprised of 120 patients (80% female); the mean age (±SD) at diagnosis was 67.8 (±7.5) years. The mean time from onset of symptoms to diagnosis was significantly longer in TAK (3.2 years) than GCA (0.5 years), p < 0.001. UE claudication was reported in 40% with TAK and 53% with GCA, p = 0.04. UE blood pressure discrepancy was present in 65% with TAK versus 28% with GCA, p < 0.001. Involvement of the thoracic aorta, abdominal aorta, carotid arteries, innominate artery, mesenteric artery, and left renal artery was more frequently observed in TAK (p < 0.05). Among patients with luminal changes of the thoracic aorta, stenotic/occlusive lesions were predominant in TAK (81% compared to 0% in GCA), whereas aneurysmal disease was more common in GCA (100% compared with 19% in TAK, p < 0.001). CONCLUSION: Patients with GCA and UE involvement differ from patients with TAK in clinical and imaging characteristics. Aortic aneurysms were more common in GCA, while stenotic changes of the aorta were more common in TAK, suggesting different pathophysiologic mechanisms or vascular response to injury. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Kermani, Tanaz A AU - Kermani TA AD - Department of Medicine, David Geffen School of Medicine, University of California Los Angeles, 2020 Santa Monica Boulevard, Suite 540, Los Angeles, CA 90404. Electronic address: TKermani@mednet.ucla.edu. FAU - Crowson, Cynthia S AU - Crowson CS AD - Department of Medicine, Mayo Clinic, Rochester, MN; Department of Health Sciences Research, Mayo Clinic, Rochester, MN. FAU - Muratore, Francesco AU - Muratore F AD - Department of Medicine, Arcispedale S. Maria Nuova IRCCS, Reggio Emilia, Italy. FAU - Schmidt, Jean AU - Schmidt J AD - Department of Medicine, Amiens University Hospital, Amiens, France. FAU - Matteson, Eric L AU - Matteson EL AD - Department of Medicine, Mayo Clinic, Rochester, MN; Department of Health Sciences Research, Mayo Clinic, Rochester, MN. FAU - Warrington, Kenneth J AU - Warrington KJ AD - Department of Medicine, Mayo Clinic, Rochester, MN. LA - eng PT - Comparative Study PT - Journal Article DEP - 20150123 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Age Distribution MH - Aged MH - Angiography MH - Aorta MH - Blood Pressure MH - Brachiocephalic Trunk/diagnostic imaging MH - Carotid Arteries/diagnostic imaging MH - Cohort Studies MH - Female MH - Giant Cell Arteritis/diagnostic imaging/epidemiology/*physiopathology MH - Humans MH - Intermittent Claudication/etiology MH - Male MH - Mesenteric Arteries/diagnostic imaging MH - Middle Aged MH - Raynaud Disease MH - Renal Artery/diagnostic imaging MH - Retrospective Studies MH - Takayasu Arteritis/diagnostic imaging/epidemiology/*physiopathology MH - Tomography, X-Ray Computed MH - Upper Extremity/*blood supply MH - Young Adult OTO - NOTNLM OT - Clinical characteristics OT - Giant cell arteritis OT - Imaging OT - Takayasu arteritis EDAT- 2015/02/26 06:00 MHDA- 2016/04/15 06:00 CRDT- 2015/02/26 06:00 PHST- 2014/10/15 00:00 [received] PHST- 2015/01/04 00:00 [revised] PHST- 2015/01/16 00:00 [accepted] PHST- 2015/02/26 06:00 [entrez] PHST- 2015/02/26 06:00 [pubmed] PHST- 2016/04/15 06:00 [medline] AID - S0049-0172(15)00006-2 [pii] AID - 10.1016/j.semarthrit.2015.01.005 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2015 Jun;44(6):724-8. doi: 10.1016/j.semarthrit.2015.01.005. Epub 2015 Jan 23. PMID- 36372235 OWN - NLM STAT- MEDLINE DCOM- 20221201 LR - 20230202 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 145 DP - 2023 Jan TI - Structural and functional analysis of retinal vasculature in HANAC syndrome with a novel intronic COL4A1 mutation. PG - 104450 LID - S0026-2862(22)00142-X [pii] LID - 10.1016/j.mvr.2022.104450 [doi] AB - PURPOSE: Mutations of the COL4A1 gene, a major structural protein of vessels, may cause hereditary angiopathy with nephropathy, aneurysms and muscle cramps (HANAC) syndrome. The vascular structure and function of patients with HANAC is poorly known. Here, we report a family with HANAC syndrome associated to a previously unreported mutation in COL4A1. The structure and function of retinal vessels were detailed by adaptive optics ophthalmoscopy (AOO) and optical coherence tomography (OCT) angiography. METHODS: Clinical data from six affected individuals (43 to 72 years old) from a single family comprising two generations were collected. Imaging charts including conventional fundus imaging, OCT-angiography and AOO in static and dynamic (flicker) mode were reviewed. DNA sequencing was done in the proband. RESULTS: DNA sequencing of the proband revealed a heterozygous deletion of COL4A1 (NM_001845) at position 1120 in the intron 20 resulting in the loss of splicing donor site for exon 20 (c.1120 + 2_1120 + 8del heterozygote). Four patients had arterial hypertension, and three had kidney dysfunction, one of which under dialysis. By fundus examination, five had typical retinal arteriolar tortuosity with arteriolar loops. Wall-to-lumen ratio of arteries was within normal limits, that is, lower than expected for hypertensive patients. Several foci of arteriolar irregularities were noted in the two oldest patients. In three affected subjects, evaluation of the neurovascular coupling showed a higher flicker-induced vasodilation than a control population (6 % to 11 %; n < 5 %). CONCLUSIONS: Structural and dynamic analysis of retinal vessels in a HANAC family bearing a previously unreported intronic COL4 mutation was done. In addition to arteriolar tortuosity, we found reduced wall-to-lumen ratio, arteriolar irregularity and increased vasodilatory response to flicker light. These abnormalities were more marked in the oldest subjects. This abnormal flicker response affected also non-tortuous arteries, suggesting that microvascular dysfunction extends beyond tortuosity. Such explorations may help to better vascular dysfunction related to HANAC and hence better understand the mechanisms of end-organ damage. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Faure, Céline AU - Faure C AD - Ophthalmology department, Saint Martin hospital, Ramsay Générale de Santé, 18 rue des Roquemonts, 14000 Caen, France; Clinical Investigation Center 1423, Quinze-Vingts hospital, INSERM-DHOS, Paris F-75012, France. Electronic address: celinefaureoph@gmail.com. FAU - Castrale, Cindy AU - Castrale C AD - Nephrology department, Saint Martin hospital, Ramsay Générale de Santé, 18 rue des Roquemonts, 14000 Caen, France. FAU - Benabed, Anaïs AU - Benabed A AD - Nephrology department, Saint Martin hospital, Ramsay Générale de Santé, 18 rue des Roquemonts, 14000 Caen, France. FAU - Cognard, Pauline AU - Cognard P AD - Ophthalmology department, Saint Martin hospital, Ramsay Générale de Santé, 18 rue des Roquemonts, 14000 Caen, France. FAU - Lezé, Romain AU - Lezé R AD - Ophthalmology department, Saint Martin hospital, Ramsay Générale de Santé, 18 rue des Roquemonts, 14000 Caen, France. FAU - Castro-Farias, Daniela AU - Castro-Farias D AD - Clinical Investigation Center 1423, Quinze-Vingts hospital, INSERM-DHOS, Paris F-75012, France. FAU - Gérard, Marion AU - Gérard M AD - Genetics department, CHU de Caen-Hôpital Clémenceau, avenue Georges Clémenceau, France. FAU - Louapre, Céline AU - Louapre C AD - Sorbonne University, APHP, Pitié-Salpêtrière Hospital, Department of neurology, CIC Neurosciences, Paris Brain Institute, Paris, France. FAU - Paques, Michel AU - Paques M AD - Clinical Investigation Center 1423, Quinze-Vingts hospital, INSERM-DHOS, Paris F-75012, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20221111 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Collagen Type IV) RN - 0 (COL4A1 protein, human) RN - Angiopathy, Hereditary, With Nephropathy, Aneurysms, And Muscle Cramps SB - IM MH - Raynaud Disease MH - Tomography, Optical Coherence MH - Mutation MH - Retinal Vessels MH - Humans MH - Aged MH - *Muscle Cramp/complications/genetics MH - Middle Aged MH - Adult MH - Introns MH - *Aneurysm/complications/genetics MH - Collagen Type IV/genetics OTO - NOTNLM OT - Adaptive optics ophthalmoscopy OT - Aneurysms and muscle cramps (HANAC) OT - COL4A1 OT - Hereditary angiopathy with nephropathy OT - Neurovascular coupling OT - Optical coherence tomography angiography OT - Retinal vessels COIS- Declaration of competing interest The authors have no competing interests. EDAT- 2022/11/14 06:00 MHDA- 2022/12/02 06:00 CRDT- 2022/11/13 19:33 PHST- 2022/09/04 00:00 [received] PHST- 2022/10/17 00:00 [revised] PHST- 2022/11/01 00:00 [accepted] PHST- 2022/11/14 06:00 [pubmed] PHST- 2022/12/02 06:00 [medline] PHST- 2022/11/13 19:33 [entrez] AID - S0026-2862(22)00142-X [pii] AID - 10.1016/j.mvr.2022.104450 [doi] PST - ppublish SO - Microvasc Res. 2023 Jan;145:104450. doi: 10.1016/j.mvr.2022.104450. Epub 2022 Nov 11. PMID- 18520322 OWN - NLM STAT- MEDLINE DCOM- 20080717 LR - 20210108 IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 87 IP - 3 DP - 2008 May TI - Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies. PG - 131-141 LID - 10.1097/MD.0b013e318173aa56 [doi] AB - Audiovestibular dysfunction has been reported in patients with connective tissue disease. Systemic sclerosis (SSc; scleroderma) is a rare connective tissue disease of unknown etiology. In the current study we assess whether audiovestibular involvement is present in patients with limited scleroderma (lSSc). To answer this question we studied a series of 35 consecutive patients who fulfilled well-established classification criteria for lSSc and had antibodies against the major centromere protein-B (CENP-B), and 59 matched controls. Individuals with a history of cerebrovascular complications, syphilis, Ménière and other vestibular syndromes, infections involving the inner ear, barotrauma, or in treatment with ototoxic drugs were excluded. The majority of patients with lSSc were women (94%). The mean age at time of study was 64.5 years, and the mean age at time of disease diagnosis was 56.9 years. Besides Raynaud phenomenon, most patients with lSSc had other typical features of CREST (calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia) syndrome. Twenty-seven (77%) patients showed abnormal hearing loss in the audiogram compared with only 15 (26%) of the controls (p < 0.001). Values of audiometric tests (pure-tone average and speech reception threshold) yielded significant differences between patients and controls (p < 0.001). The typical pattern of hearing impairment in our series of lSSc patients was a bilateral and symmetrical sensorineural hearing loss with a flat pattern in the audiogram. Abnormal tympanogram and abnormal stapedial reflex were more commonly observed in patients than controls (p < or = 0.001). Similarly, a significantly increased frequency of abnormal oculocephalic response (10 patients, 29%) and head-shaking nystagmus (9 patients, 26%) was observed in patients compared with controls (p < 0.001 for both comparisons). Finally, a significantly increased frequency of abnormal caloric test and clinical test of sensory integration and balance was observed in lSSc patients (31% and 46% of patients, respectively) compared with controls (0% and 12%, respectively) (p < 0.001 for both comparisons). The current study demonstrates strong evidence for inner ear compromise in patients with lSSc. FAU - Amor-Dorado, Juan C AU - Amor-Dorado JC AD - From the Divisions of Otolaryngology (JCAD), Internal Medicine (MCAN), Rheumatology (JAMF, MAGG), and Cardiology (CGJ), Hospital Xeral-Calde, Lugo; and the Division of Epidemiology and Computational Biology (JL), School of Medicine, University of Cantabria, Santander, Spain. FAU - Arias-Nuñez, Maria C AU - Arias-Nuñez MC FAU - Miranda-Filloy, Jose A AU - Miranda-Filloy JA FAU - Gonzalez-Juanatey, Carlos AU - Gonzalez-Juanatey C FAU - Llorca, Javier AU - Llorca J FAU - Gonzalez-Gay, Miguel A AU - Gonzalez-Gay MA LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) RN - 0 (Centromere Protein B) SB - IM MH - Autoantibodies/*blood MH - CREST Syndrome/complications/diagnosis MH - Centromere Protein B/*immunology MH - Female MH - Hearing Loss, Sensorineural/*complications/diagnosis MH - Humans MH - Male MH - Middle Aged MH - Nystagmus, Pathologic/complications/diagnosis MH - Scleroderma, Systemic/*complications/immunology MH - Vestibular Diseases/*complications/diagnosis EDAT- 2008/06/04 09:00 MHDA- 2008/07/18 09:00 CRDT- 2008/06/04 09:00 PHST- 2008/06/04 09:00 [pubmed] PHST- 2008/07/18 09:00 [medline] PHST- 2008/06/04 09:00 [entrez] AID - 00005792-200805000-00001 [pii] AID - 10.1097/MD.0b013e318173aa56 [doi] PST - ppublish SO - Medicine (Baltimore). 2008 May;87(3):131-141. doi: 10.1097/MD.0b013e318173aa56. PMID- 18625944 OWN - NLM STAT- MEDLINE DCOM- 20080724 LR - 20151119 IS - 1538-3601 (Electronic) IS - 0003-9950 (Linking) VI - 126 IP - 7 DP - 2008 Jul TI - Bilateral infusion pump implants as therapy for refractory corneal ulcers in a patient with CREST syndrome: an interdisciplinary approach. PG - 964-7 LID - 10.1001/archopht.126.7.964 [doi] AB - Internal infusion pumps are implantable and programmable systems that have been widely used for years in the management of chronic pain. During the past few years, these devices have had an increasingly prominent role given the possibility of insulin infusions in patients with diabetes mellitus because they provide patients with higher autonomy in the management of their disease, despite the fact that they are expensive systems and require surgery for implantation. These features make internal infusion pumps a suitable therapeutic option for those patients who need to use artificial tears continuously because of severe dry eyes. We report a case of severe eye pain due to xerophthalmia in a patient with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome who was treated with an implanted pump reservoir. FAU - De Andrés, José AU - De Andrés J AD - Departments of Anesthesia, Critical Care, and PainManagement, University General Hospital, Valencia, Spain. FAU - García-Delpech, Salvador AU - García-Delpech S FAU - Pérez, Vicente Luis Villanueva AU - Pérez VL FAU - Díaz-Llopis, Manuel AU - Díaz-Llopis M FAU - Udaondo, Patricia AU - Udaondo P FAU - Sánchez, María Teresa Serrano AU - Sánchez MT FAU - Salom, David AU - Salom D LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arch Ophthalmol JT - Archives of ophthalmology (Chicago, Ill. : 1960) JID - 7706534 RN - 451W47IQ8X (Sodium Chloride) SB - IM MH - CREST Syndrome/*complications MH - Corneal Ulcer/*drug therapy/etiology MH - Female MH - Humans MH - *Infusion Pumps, Implantable MH - Middle Aged MH - Patient Care Team/organization & administration MH - Recurrence MH - Sjogren's Syndrome/drug therapy/etiology MH - Sodium Chloride/*administration & dosage MH - Subcutaneous Tissue MH - Xerophthalmia/drug therapy/etiology EDAT- 2008/07/16 09:00 MHDA- 2008/07/25 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2008/07/25 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] AID - 126/7/964 [pii] AID - 10.1001/archopht.126.7.964 [doi] PST - ppublish SO - Arch Ophthalmol. 2008 Jul;126(7):964-7. doi: 10.1001/archopht.126.7.964. PMID- 22221908 OWN - NLM STAT- MEDLINE DCOM- 20120924 LR - 20151119 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 41 IP - 6 DP - 2012 Jun TI - Treatment of systemic sclerosis-associated calcinosis: a case report of rituximab-induced regression of CREST-related calcinosis and review of the literature. PG - 822-9 LID - 10.1016/j.semarthrit.2011.11.007 [doi] AB - OBJECTIVES: Calcinosis is frequently encountered in patients with systemic sclerosis (SSc) and may be associated with significant morbidity. No treatment has shown so far an unequivocal beneficial effect. METHODS: We performed an extensive internet search (MEDLINE) using the keywords calcinosis, calcification, scleroderma, systemic sclerosis, and treatment. RESULTS: Our patient had extensive Calcinosis, Raynaud, Esophagitis, Sclerodactyly, telangiectasia (CREST)-related calcinosis, frequently ulcerating and painful. Following 2 rituximab courses (consisting of 4 weekly infusions, 375 mg/m(2) each), calcinosis significantly improved and pain disappeared. Pharmacologic agents used in the treatment of SSc-associated calcinosis include diltiazem, minocycline, warfarin, biphosphonates, and intravenous immunoglobulin. Other therapeutic approaches include surgical excision, laser vaporization, and extracorporeal shock wave lithotripsy. CONCLUSIONS: Evidence for all existing therapies is weak and therefore larger scale controlled studies are needed. Rituximab appears as a promising treatment especially in view of recent evidence that this therapy may be also effective in the underlying disease. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Daoussis, Dimitrios AU - Daoussis D AD - Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Patras, Greece. jimdaoussis@hotmail.com FAU - Antonopoulos, Ioannis AU - Antonopoulos I FAU - Liossis, Stamatis-Nick C AU - Liossis SN FAU - Yiannopoulos, Georgios AU - Yiannopoulos G FAU - Andonopoulos, Andrew P AU - Andonopoulos AP LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20120104 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) SB - IM CIN - Semin Arthritis Rheum. 2014 Oct;44(2):e5-6. doi: 10.1016/j.semarthrit.2014.04.007. PMID: 24842570 MH - Antibodies, Monoclonal, Murine-Derived/*therapeutic use MH - Antirheumatic Agents/*therapeutic use MH - CREST Syndrome/complications/*drug therapy MH - Calcinosis/complications/*drug therapy MH - Female MH - Humans MH - Middle Aged MH - Pain Measurement MH - Rituximab MH - Scleroderma, Systemic/*complications MH - Treatment Outcome EDAT- 2012/01/10 06:00 MHDA- 2012/09/25 06:00 CRDT- 2012/01/07 06:00 PHST- 2011/09/22 00:00 [received] PHST- 2011/11/07 00:00 [revised] PHST- 2011/11/15 00:00 [accepted] PHST- 2012/01/07 06:00 [entrez] PHST- 2012/01/10 06:00 [pubmed] PHST- 2012/09/25 06:00 [medline] AID - S0049-0172(11)00357-X [pii] AID - 10.1016/j.semarthrit.2011.11.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2012 Jun;41(6):822-9. doi: 10.1016/j.semarthrit.2011.11.007. Epub 2012 Jan 4. PMID- 40836485 OWN - NLM STAT- MEDLINE DCOM- 20250905 LR - 20250905 IS - 1096-9101 (Electronic) IS - 0196-8092 (Linking) VI - 57 IP - 8 DP - 2025 Oct TI - Fractional Carbon Dioxide Laser for the Treatment of Microstomia in Limited-Cutaneous Systemic Sclerosis. PG - 658-662 LID - 10.1002/lsm.70057 [doi] AB - OBJECTIVES: Limited-cutaneous systemic sclerosis (lcSSc), formerly known as CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, scleroderma, and telangiectasias) is an auto-immune connective tissue disease characterized by cutaneous fibrosis and systemic fibrovascular dysfunction. The cutaneous fibrosis of lcSSc is commonly limited to acral sites, with perioral involvement seen in 70%-85% of cases. Patients often seek treatment of lcSSc-associated microstomia for functional and cosmetic purposes. Treatments for the cutaneous sclerosis in systemic sclerosis (SSc) include topical agents, systemic immunosuppressants, phototherapy, and other less commonly used systemic medications. More recently fractional ablative laser therapy with the carbon dioxide (CO(2)) laser has been utilized in scarring disorders of the skin with notable improvement in cutaneous fibrosis. Here we report a case of a 54-year-old female with lcSSc-associated microstomia treated with fractional ablative CO(2) laser. The goal of treatment was to improve the objective size of the oral aperture and improve subjective symptoms, including oral mobility, speech, and eating. MATERIALS AND METHODS: The Lumenis Ultra Pulse CO(2) laser was used to fractionally ablate the target peri-oral skin. Pulses were performed up to but not crossing the vermillion border circumferentially around the mouth. Measurements in centimeters were taken pretreatment as well as 2 and 5 months posttreatment. Appropriate infectious prophylaxis was provided to the patient. RESULTS: Notable increases were observed at 2 and 5 months follow-ups in horizontal width (+14.3%), vertical height (+12.1%), and cross-sectional areas (+28.1%) of the oral aperture following a single treatment with CO(2) laser. Subjective improvements in oral mobility, speech, and daily activities such as eating were noted by the patient. CONCLUSION: Ablative fractional CO(2) laser is an effective and efficient method for the treatment of microstomia secondary to systemic sclerosis with minimal adverse effects. Utilizing this therapeutic treatment can significantly impact the quality of life in patients with lcSSc-associated microstomia. CI - © 2025 Wiley Periodicals LLC. FAU - Fisher, Craig AU - Fisher C AUID- ORCID: 0009-0004-7694-7812 AD - Department of Dermatology, Wilford Hall Ambulatory Surgical Center, San Antonio Uniformed Health Education Consortium, Lackland AFB, Texas, USA. FAU - Woodside, Sarah S AU - Woodside SS AD - Department of Dermatology, Wilford Hall Ambulatory Surgical Center, San Antonio Uniformed Health Education Consortium, Lackland AFB, Texas, USA. FAU - Martin, Yizhen E AU - Martin YE AD - Department of Dermatology, Wilford Hall Ambulatory Surgical Center, San Antonio Uniformed Health Education Consortium, Lackland AFB, Texas, USA. FAU - Mak, Evan L AU - Mak EL AD - Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. FAU - Roberts, Jared AU - Roberts J AD - Department of Dermatology, Wilford Hall Ambulatory Surgical Center, San Antonio Uniformed Health Education Consortium, Lackland AFB, Texas, USA. FAU - Hivnor, Chad AU - Hivnor C AD - Department of Dermatology, Veterans Health Administration, San Antonio, Texas, USA. LA - eng GR - The authors received no specific funding for this work./ PT - Case Reports PT - Journal Article DEP - 20250820 PL - United States TA - Lasers Surg Med JT - Lasers in surgery and medicine JID - 8007168 SB - IM MH - Humans MH - *Lasers, Gas/therapeutic use MH - Female MH - Middle Aged MH - *Microstomia/etiology/surgery/radiotherapy MH - *Scleroderma, Systemic/complications MH - *CREST Syndrome/complications OTO - NOTNLM OT - CO2 OT - CREST syndrome OT - ablative OT - carbon dioxide laser OT - resurfacing OT - scar OT - scleroderma OT - systemic sclerosis EDAT- 2025/08/21 06:28 MHDA- 2025/09/05 06:35 CRDT- 2025/08/21 02:13 PHST- 2025/08/05 00:00 [revised] PHST- 2025/06/24 00:00 [received] PHST- 2025/08/14 00:00 [accepted] PHST- 2025/09/05 06:35 [medline] PHST- 2025/08/21 06:28 [pubmed] PHST- 2025/08/21 02:13 [entrez] AID - 10.1002/lsm.70057 [doi] PST - ppublish SO - Lasers Surg Med. 2025 Oct;57(8):658-662. doi: 10.1002/lsm.70057. Epub 2025 Aug 20. PMID- 39275841 OWN - NLM STAT- MEDLINE DCOM- 20250103 LR - 20250103 IS - 1724-6016 (Electronic) IS - 1120-6721 (Linking) VI - 35 IP - 1 DP - 2025 Jan TI - Long-term prognosis of penetrating keratoplasty in a patient with limited form of Scleroderma- a case report. PG - NP1-NP4 LID - 10.1177/11206721241284405 [doi] AB - PURPOSE: To explore the challenges of managing recurrent graft rejections in patients with Macular Corneal Dystrophy (MCD) undergoing Penetrating Keratoplasty (PKP) who also have an underlying diagnosis of Systemic Sclerosis, specifically the limited form known as CREST syndrome. METHODS: The case of a 47-year-old female diagnosed with MCD who underwent multiple PKPs over a 13 year period was reviewed. The patients treatment included extensive surgical interventions (PKPs, amniotic membrane transplatation, tarsorrhaphy) and medical management involving systemic and topical steroids and immunosuppressive therapy (Tacrolimus ointment). RESULTS: Initial PKP surgeries improved the patients vision, but subsequently graft rejections,both acute and chronic, required further surgical and medical interventions. Despite aggressive management, the patient experienced multiple graft failures, with the final visual outcome being significantly compromised (vision 6/60). the presence of CREST syndrome complicated the management and prognosis of graft survival. CONCLUSION: This case illustrates the significant impact of systemic autoimmune disorders like CREST syndrome on the prognosis of PKP in patients with MCD. It highlights the necessity for diligent systemic evaluation and possibly more aggressive immunosuppresive strategies to manage graft rejections and prolong graft survival in such complex clinical scenarios. FAU - Anitha, Venugopal AU - Anitha V AUID- ORCID: 0000-0002-7412-380X AD - Cornea and Refractive Services, Aravind Eye Hospital and Post graduate Institute of Ophthalmology, Tirunelveli, India. RINGGOLD: 75197 FAU - Patwardhan, Veena AU - Patwardhan V AD - Cornea and Refractive Services, Aravind Eye Hospital and Post graduate Institute of Ophthalmology, Tirunelveli, India. RINGGOLD: 75197 FAU - Ravindran, Meenakshi AU - Ravindran M AD - Chief Medical Officer, Paediatric and Strabismus surgery, Aravind Eye Hospital and Post graduate Institute of Ophthalmology, Tirunelveli, India. RINGGOLD: 75197 LA - eng PT - Case Reports PT - Journal Article DEP - 20240913 PL - United States TA - Eur J Ophthalmol JT - European journal of ophthalmology JID - 9110772 RN - 0 (Immunosuppressive Agents) SB - IM MH - Humans MH - Female MH - *Keratoplasty, Penetrating MH - Middle Aged MH - *Visual Acuity/physiology MH - *Graft Survival MH - Prognosis MH - Graft Rejection/diagnosis MH - CREST Syndrome/complications MH - Corneal Dystrophies, Hereditary/surgery/diagnosis/physiopathology MH - Immunosuppressive Agents/therapeutic use OTO - NOTNLM OT - Corneal dystrophies < CORNEA / EXTERNAL DISEASE OT - corneal transplantation < CORNEA / EXTERNAL DISEASE OT - immune disease of the cornea < CORNEA / EXTERNAL DISEASE OT - immune diseases of the sclera < CORNEA / EXTERNAL DISEASE OT - penetrating keratoplasty < CORNEA / EXTERNAL DISEASE OT - surgical trauma < CORNEA / EXTERNAL DISEASE COIS- Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2024/09/14 10:46 MHDA- 2025/01/03 06:21 CRDT- 2024/09/14 02:42 PHST- 2025/01/03 06:21 [medline] PHST- 2024/09/14 10:46 [pubmed] PHST- 2024/09/14 02:42 [entrez] AID - 10.1177/11206721241284405 [doi] PST - ppublish SO - Eur J Ophthalmol. 2025 Jan;35(1):NP1-NP4. doi: 10.1177/11206721241284405. Epub 2024 Sep 13. PMID- 34250789 OWN - NLM STAT- MEDLINE DCOM- 20210722 LR - 20240521 IS - 1933-0693 (Electronic) IS - 0022-3085 (Linking) VI - 135 IP - 1 DP - 2021 Jul 1 TI - Cochlear implantation after radiosurgery for vestibular schwannoma. PG - 126-135 LID - 10.3171/2020.4.JNS201069 [doi] AB - OBJECTIVE: The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS). METHODS: The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. RESULTS: Seventeen patients (18 ears) underwent SRS and ipsilateral CI during the study period. Thirteen patients (76%) had neurofibromatosis type 2 (NF2). Median age at SRS and CI were 44 and 48 years, respectively. Median time from SRS to CI was 60 days, but notably, 4 patients underwent SRS and CI within 1 day and 5 patients underwent CI more than 7 years after SRS. Median marginal dose was 13 Gy. Median treatment volume at the time of SRS was 1400 mm3 (range 84-6080 mm3, n = 15 patients). Median post-CI PTA was 28 dB HL, improved from 101 dB HL preoperatively (p < 0.001). Overall, 11 patients (12 ears) exhibited open-set speech understanding. Sentence testing was performed at a median of 10 months (range 1-143 months) post-CI. The median AzBio sentence score for patients with open-set speech understanding was 76% (range 19%-95%, n = 10 ears). Two ears exhibited Hearing in Noise Test (HINT) sentence scores of 49% and 95%, respectively. Four patients achieved environmental sound awareness without open-set speech recognition. Two had no detectable auditory percepts. CONCLUSIONS: Most patients who underwent CI following SRS for VS enjoyed access to sound at near-normal levels, with the majority achieving good open-set speech understanding. Implantation can be performed immediately following SRS or in a delayed fashion, depending on hearing status as well as other factors. This strategy may be applied to cases of sporadic or NF2-associated VS. ABBREVIATIONS: AAO-HNS = American Academy of Otolaryngology-Head and Neck Surgery; ABI = auditory brainstem implant; CI = cochlear implantation; CN = cranial nerve; CNC = consonant-nucleus-consonant; CPA = cerebellopontine angle; EPS = electrical promontory stimulation; ESA = environmental sound awareness; HINT = Hearing in Noise Test; IAC = internal auditory canal; NF2 = neurofibromatosis type 2; OSP = open-set speech perception; PTA = pure tone average; SRS = stereotactic radiosurgery; VS = vestibular schwannoma; WRS = word recognition score. FAU - Patel, Neil S AU - Patel NS AD - 1Department of Otolaryngology-Head and Neck Surgery, and. FAU - Carlson, Matthew L AU - Carlson ML AD - 1Department of Otolaryngology-Head and Neck Surgery, and. AD - 2Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota. FAU - Link, Michael J AU - Link MJ AD - 1Department of Otolaryngology-Head and Neck Surgery, and. AD - 2Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota. FAU - Neff, Brian A AU - Neff BA AD - 1Department of Otolaryngology-Head and Neck Surgery, and. FAU - Van Gompel, Jamie J AU - Van Gompel JJ AD - 1Department of Otolaryngology-Head and Neck Surgery, and. AD - 2Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota. FAU - Driscoll, Colin L W AU - Driscoll CLW AD - 1Department of Otolaryngology-Head and Neck Surgery, and. AD - 2Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota. LA - eng PT - Case Reports PT - Journal Article DEP - 20210624 PL - United States TA - J Neurosurg JT - Journal of neurosurgery JID - 0253357 SB - IM MH - Adolescent MH - Adult MH - Aged MH - CREST Syndrome/complications MH - *Cochlear Implantation MH - Cochlear Nerve/diagnostic imaging/physiopathology MH - Female MH - Hearing Loss, Sensorineural/etiology/*rehabilitation MH - Hearing Loss, Unilateral/etiology/*rehabilitation MH - Hearing Tests MH - Humans MH - Male MH - Middle Aged MH - Neurofibromatosis 2/complications MH - Neuroma, Acoustic/complications/diagnostic imaging/rehabilitation/*surgery MH - *Radiosurgery MH - Retrospective Studies MH - Speech Perception MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - cochlear implant OT - cranial base OT - hearing loss OT - neurofibromatosis OT - skull base OT - vestibular schwannoma COIS- Dr. Carlson is a consultant for Advanced Bionics, Cochlear Corp., and MED-EL GmbH. Dr. Driscoll is a consultant for Advanced Bionics, Cochlear Corp., and Envoy Medical. EDAT- 2021/07/13 06:00 MHDA- 2021/07/23 06:00 CRDT- 2021/07/12 08:01 PHST- 2021/07/12 08:01 [entrez] PHST- 2021/07/13 06:00 [pubmed] PHST- 2021/07/23 06:00 [medline] AID - 10.3171/2020.4.JNS201069 [doi] PST - ppublish SO - J Neurosurg. 2021 Jul 1;135(1):126-135. doi: 10.3171/2020.4.JNS201069. Epub 2021 Jun 24. PMID- 33963836 OWN - NLM STAT- MEDLINE DCOM- 20210927 LR - 20210927 IS - 2047-2412 (Electronic) IS - 2047-2404 (Linking) VI - 22 IP - 9 DP - 2021 Aug 14 TI - Biventricular affection in CREST syndrome. PG - e146 LID - 10.1093/ehjci/jeab068 [doi] FAU - Brugger, Marcus AU - Brugger M AD - Department of Cardiology, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Straße 22, 81675 Munich, Germany. FAU - Steger, Alexander AU - Steger A AD - Department of Cardiology, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Straße 22, 81675 Munich, Germany. FAU - Okrojek, Rainer AU - Okrojek R AD - Department of Cardiology, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Straße 22, 81675 Munich, Germany. FAU - Gaa, Jochen AU - Gaa J AD - Department of Radiology, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Straße 22, 81675 Munich, Germany. FAU - Ibrahim, Tareq AU - Ibrahim T AD - Department of Cardiology, Klinikum rechts der Isar, Technical University of Munich, Ismaninger Straße 22, 81675 Munich, Germany. LA - eng PT - Journal Article PL - England TA - Eur Heart J Cardiovasc Imaging JT - European heart journal. Cardiovascular Imaging JID - 101573788 SB - IM MH - *CREST Syndrome MH - Humans EDAT- 2021/05/09 06:00 MHDA- 2021/09/28 06:00 CRDT- 2021/05/08 12:05 PHST- 2021/03/18 00:00 [received] PHST- 2021/03/23 00:00 [accepted] PHST- 2021/05/09 06:00 [pubmed] PHST- 2021/09/28 06:00 [medline] PHST- 2021/05/08 12:05 [entrez] AID - 6272475 [pii] AID - 10.1093/ehjci/jeab068 [doi] PST - ppublish SO - Eur Heart J Cardiovasc Imaging. 2021 Aug 14;22(9):e146. doi: 10.1093/ehjci/jeab068. PMID- 32883767 OWN - NLM STAT- MEDLINE DCOM- 20220301 LR - 20220301 IS - 1469-0756 (Electronic) IS - 0032-5473 (Linking) VI - 97 IP - 1153 DP - 2021 Nov TI - CREST syndrome. PG - 746 LID - 10.1136/postgradmedj-2020-138817 [doi] FAU - Kumar, Ashok AU - Kumar A AD - Rheumatology, Fortis FLT LT Rajan Dhall Hospital, New Delhi, India. FAU - Sharma, Ashish AU - Sharma A AUID- ORCID: 0000-0002-4810-8921 AD - Rheumatology, Fortis Hospital, Noida, India ash.blueney@gmail.com. FAU - Agarwal, Anunay AU - Agarwal A AUID- ORCID: 0000-0002-2310-4748 AD - Internal Medicine, Aakash Hospital, New Delhi, India. LA - eng PT - Journal Article DEP - 20200903 PL - England TA - Postgrad Med J JT - Postgraduate medical journal JID - 0234135 SB - IM MH - *CREST Syndrome MH - *Gastroenterology MH - Humans OTO - NOTNLM OT - CT OT - Gastroenterology OT - Internal medicine OT - Motility disorders OT - Oesophageal disease OT - Radiology & imaging OT - Rheumatology COIS- Competing interests: None declared. EDAT- 2020/09/05 06:00 MHDA- 2022/03/03 06:00 CRDT- 2020/09/05 06:00 PHST- 2020/08/05 00:00 [received] PHST- 2020/08/07 00:00 [accepted] PHST- 2020/09/05 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2020/09/05 06:00 [entrez] AID - postgradmedj-2020-138817 [pii] AID - 10.1136/postgradmedj-2020-138817 [doi] PST - ppublish SO - Postgrad Med J. 2021 Nov;97(1153):746. doi: 10.1136/postgradmedj-2020-138817. Epub 2020 Sep 3. PMID- 30862460 OWN - NLM STAT- MEDLINE DCOM- 20200228 LR - 20200228 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 132 IP - 10 DP - 2019 Oct TI - Acute Herbal Therapy Efficacy: A Case Report. PG - 1126-1127 LID - S0002-9343(18)31178-1 [pii] LID - 10.1016/j.amjmed.2018.11.046 [doi] FAU - Stern, Robert G AU - Stern RG AD - Department of Medical Imaging, University of Arizona College of Medicine, Tucson. Electronic address: sternr@cox.net. LA - eng PT - Case Reports PT - Editorial DEP - 20190309 PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 RN - 0 (Teas, Herbal) SB - IM CIN - Am J Med. 2019 Oct;132(10):1128. doi: 10.1016/j.amjmed.2019.03.018. PMID: 30953630 MH - Aged MH - CREST Syndrome/*drug therapy MH - Chronic Pain/*drug therapy MH - Female MH - Humans MH - *Phytotherapy MH - *Teas, Herbal EDAT- 2019/03/14 06:00 MHDA- 2020/02/29 06:00 CRDT- 2019/03/14 06:00 PHST- 2018/11/30 00:00 [received] PHST- 2018/11/30 00:00 [accepted] PHST- 2019/03/14 06:00 [pubmed] PHST- 2020/02/29 06:00 [medline] PHST- 2019/03/14 06:00 [entrez] AID - S0002-9343(18)31178-1 [pii] AID - 10.1016/j.amjmed.2018.11.046 [doi] PST - ppublish SO - Am J Med. 2019 Oct;132(10):1126-1127. doi: 10.1016/j.amjmed.2018.11.046. Epub 2019 Mar 9. PMID- 29067967 OWN - NLM STAT- MEDLINE DCOM- 20181009 LR - 20181113 IS - 2542-5641 (Electronic) IS - 0366-6999 (Print) IS - 0366-6999 (Linking) VI - 130 IP - 21 DP - 2017 Nov 5 TI - Pulmonary Capillary Hemangiomatosis Associated with CREST Syndrome: A Challenge of Diagnosis and Treatment. PG - 2645-2646 LID - 10.4103/0366-6999.217082 [doi] FAU - Diao, Xiao-Li AU - Diao XL AD - Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China. FAU - Mu, Xiang-Dong AU - Mu XD AD - Department of Pulmonary and Critical Care Medicine, Peking University First Hospital, Beijing 100034, China. FAU - Jin, Mu-Lan AU - Jin ML AD - Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China. LA - eng PT - Case Reports PT - Letter PL - China TA - Chin Med J (Engl) JT - Chinese medical journal JID - 7513795 SB - IM MH - Aged MH - CREST Syndrome/*complications/*diagnostic imaging MH - Echocardiography MH - Female MH - Hemangioma, Capillary/*diagnostic imaging/*etiology MH - Humans MH - Hypertension, Pulmonary/*complications/*diagnostic imaging MH - Lung Neoplasms/complications/diagnostic imaging PMC - PMC5678270 COIS- There are no conflicts of interest. EDAT- 2017/10/27 06:00 MHDA- 2018/10/10 06:00 PMCR- 2017/11/05 CRDT- 2017/10/26 06:00 PHST- 2017/10/26 06:00 [entrez] PHST- 2017/10/27 06:00 [pubmed] PHST- 2018/10/10 06:00 [medline] PHST- 2017/11/05 00:00 [pmc-release] AID - ChinMedJ_2017_130_21_2645_217082 [pii] AID - CMJ-130-2645 [pii] AID - 10.4103/0366-6999.217082 [doi] PST - ppublish SO - Chin Med J (Engl). 2017 Nov 5;130(21):2645-2646. doi: 10.4103/0366-6999.217082. PMID- 28742726 OWN - NLM STAT- MEDLINE DCOM- 20190816 LR - 20190816 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 23 IP - 5 DP - 2017 Aug TI - CREST Syndrome: Clinical Expression of the Disease. PG - 285 LID - 10.1097/RHU.0000000000000491 [doi] FAU - Arana-Guajardo, Ana AU - Arana-Guajardo A AD - From the Servicio de Reumatología, Departamento de Medicina Interna, Hospital Universitario "Dr. José Eleuterio González," Monterrey, Nuevo León, México. FAU - Villarreal-Alarcón, Miguel AU - Villarreal-Alarcón M LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) MH - Antibodies, Antinuclear/blood MH - *CREST Syndrome/diagnosis/immunology/physiopathology MH - Diagnosis, Differential MH - *Esophageal Motility Disorders/diagnosis/etiology MH - Female MH - Humans MH - Middle Aged MH - *Scleroderma, Limited/diagnosis/etiology MH - *Telangiectasis/diagnosis/etiology EDAT- 2017/07/26 06:00 MHDA- 2019/08/17 06:00 CRDT- 2017/07/26 06:00 PHST- 2017/07/26 06:00 [entrez] PHST- 2017/07/26 06:00 [pubmed] PHST- 2019/08/17 06:00 [medline] AID - 00124743-201708000-00009 [pii] AID - 10.1097/RHU.0000000000000491 [doi] PST - ppublish SO - J Clin Rheumatol. 2017 Aug;23(5):285. doi: 10.1097/RHU.0000000000000491. PMID- 25110926 OWN - NLM STAT- MEDLINE DCOM- 20150130 LR - 20161125 IS - 2168-6114 (Electronic) IS - 2168-6106 (Linking) VI - 174 IP - 10 DP - 2014 Oct TI - Looking for trouble -- patient preference, misdiagnosis and overtesting: a teachable moment. PG - 1548-9 LID - 10.1001/jamainternmed.2014.3429 [doi] FAU - Mason, Mysha K AU - Mason MK AD - Department of Medicine, University of Colorado Denver School of Medicine, Aurora. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - JAMA Intern Med JT - JAMA internal medicine JID - 101589534 SB - IM MH - Aged MH - CREST Syndrome/complications/*diagnosis/diagnostic imaging/pathology MH - Dilatation, Pathologic/diagnostic imaging/etiology MH - *Echocardiography MH - Esophagus/diagnostic imaging/pathology MH - Female MH - Humans MH - Multiple Pulmonary Nodules/diagnostic imaging/etiology MH - *Practice Patterns, Physicians'/standards/trends MH - Stress, Psychological/*etiology MH - *Tomography, X-Ray Computed MH - *Unnecessary Procedures EDAT- 2014/08/12 06:00 MHDA- 2015/01/31 06:00 CRDT- 2014/08/12 06:00 PHST- 2014/08/12 06:00 [entrez] PHST- 2014/08/12 06:00 [pubmed] PHST- 2015/01/31 06:00 [medline] AID - 1893926 [pii] AID - 10.1001/jamainternmed.2014.3429 [doi] PST - ppublish SO - JAMA Intern Med. 2014 Oct;174(10):1548-9. doi: 10.1001/jamainternmed.2014.3429. PMID- 26548213 OWN - NLM STAT- MEDLINE DCOM- 20160107 LR - 20151109 IS - 1029-4864 (Print) IS - 1029-4864 (Linking) VI - 69 IP - 7 DP - 2014 Aug TI - Oral medicine case book 62: CREST syndrome. PG - 324-5 FAU - Bunn, B K AU - Bunn BK FAU - van Zyl, A W AU - van Zyl AW FAU - Rahman, L AU - Rahman L FAU - van Heerden, W F P AU - van Heerden WF LA - eng PT - Case Reports PT - Journal Article PL - South Africa TA - SADJ JT - SADJ : journal of the South African Dental Association = tydskrif van die Suid-Afrikaanse Tandheelkundige Vereniging JID - 9812497 MH - Adult MH - CREST Syndrome/*complications MH - Connective Tissue/transplantation MH - Dental Care for Chronically Ill MH - Female MH - Gingiva/transplantation MH - Gingival Recession/*surgery MH - Humans EDAT- 2014/08/01 00:00 MHDA- 2016/01/08 06:00 CRDT- 2015/11/10 06:00 PHST- 2015/11/10 06:00 [entrez] PHST- 2014/08/01 00:00 [pubmed] PHST- 2016/01/08 06:00 [medline] PST - ppublish SO - SADJ. 2014 Aug;69(7):324-5. PMID- 23190708 OWN - NLM STAT- MEDLINE DCOM- 20130910 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 31 IP - 2 Suppl 76 DP - 2013 Mar-Apr TI - Incidence of scleroderma spectrum disorders in Slovenia. PG - 8-11 AB - OBJECTIVES: This paper aims to investigate the incidence of scleroderma spectrum disorders (SDS) in Slovenia. METHODS: From 01.01.2007 to 31.12.2009 we prospectively examined all patients over 18 years of age suspected of suffering from SDS who were referred to our department, which is the only Rheumatology referral centre in the Ljubljana region serving a population of 518.921 Caucasians over 18. Patient work-up consisted of clinical assessment, laboratory and imaging studies, and functional tests. The working classification of SDS proposed by Maricq and Valter was used to classify patients as having CREST syndrome, digital scleroderma disease (SD), intermediate SD, diffuse SD, undifferentiated connective tissue disease with SD features, and SD sine scleroderma. Patients with certain features of SDS who did not fit any specific class were classified as having prescleroderma using LeRoy's classification of early systemic sclerosis. RESULTS: We examined 100 patients. Forty-one new cases of SDS were diagnosed (37 females, 4 males), aged 58.9±15.1 years (24-86 years). CONCLUSIONS: The overall age-adjusted annual incidence of SDS in Slovenia is 2.6 per 100.000 adults per year (95%CI=1.7-3.5). FAU - Sipek Dolničar, Alenka AU - Sipek Dolničar A AD - University Medical Centre Ljubljana, Department of Rheumatology, Vodnikova cesta 62, 1000 Ljubljana, Slovenia. alenka.sipek@kclj.si FAU - Rotar, Ziga AU - Rotar Z FAU - Tomsič, Matija AU - Tomsič M LA - eng PT - Journal Article DEP - 20121128 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - CREST Syndrome/*epidemiology/pathology MH - Female MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Prospective Studies MH - Scleroderma, Diffuse/*epidemiology/pathology MH - Scleroderma, Systemic/*epidemiology/pathology MH - Slovenia/epidemiology MH - Young Adult EDAT- 2012/11/30 06:00 MHDA- 2013/09/11 06:00 CRDT- 2012/11/30 06:00 PHST- 2011/12/11 00:00 [received] PHST- 2012/06/14 00:00 [accepted] PHST- 2012/11/30 06:00 [entrez] PHST- 2012/11/30 06:00 [pubmed] PHST- 2013/09/11 06:00 [medline] AID - 5658 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):8-11. Epub 2012 Nov 28. PMID- 21511428 OWN - NLM STAT- MEDLINE DCOM- 20111028 LR - 20181201 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 40 IP - 9 Pt 1 DP - 2011 Sep TI - [Tuberculous tenosynovitis]. PG - 877-81 LID - 10.1016/j.lpm.2011.02.032 [doi] FAU - Samson, Maxime AU - Samson M AD - CHU de Dijon, service de médecine interne et immunologie clinique, 21000 Dijon, France. samsonmaxime@gmail.com FAU - Roch, Nathalie AU - Roch N FAU - Audia, Sylvain AU - Audia S FAU - Berthier, Sabine AU - Berthier S FAU - Leguy, Vanessa AU - Leguy V FAU - Bonnotte, Bernard AU - Bonnotte B FAU - Lorcerie, Bernard AU - Lorcerie B LA - fre PT - Case Reports PT - Letter TT - Une ténosynovite tuberculeuse. DEP - 20110420 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antihypertensive Agents) RN - 0 (Antitubercular Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Aged MH - Anti-Inflammatory Agents/adverse effects/therapeutic use MH - Antihypertensive Agents/adverse effects/therapeutic use MH - Antitubercular Agents/therapeutic use MH - Bosentan MH - CREST Syndrome/diagnosis/drug therapy MH - Combined Modality Therapy MH - Diagnosis, Differential MH - Female MH - *Hand/surgery MH - Humans MH - Hypertension, Pulmonary/diagnosis/drug therapy MH - Opportunistic Infections/diagnosis MH - Sulfonamides/adverse effects/therapeutic use MH - Tenosynovitis/*diagnosis/drug therapy/surgery MH - Tuberculosis, Osteoarticular/*diagnosis/drug therapy/surgery EDAT- 2011/04/23 06:00 MHDA- 2011/10/29 06:00 CRDT- 2011/04/23 06:00 PHST- 2010/09/21 00:00 [received] PHST- 2011/02/09 00:00 [revised] PHST- 2011/02/21 00:00 [accepted] PHST- 2011/04/23 06:00 [entrez] PHST- 2011/04/23 06:00 [pubmed] PHST- 2011/10/29 06:00 [medline] AID - S0755-4982(11)00151-5 [pii] AID - 10.1016/j.lpm.2011.02.032 [doi] PST - ppublish SO - Presse Med. 2011 Sep;40(9 Pt 1):877-81. doi: 10.1016/j.lpm.2011.02.032. Epub 2011 Apr 20. PMID- 21154474 OWN - NLM STAT- MEDLINE DCOM- 20110309 LR - 20101214 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 62 IP - 12 DP - 2010 Dec TI - Clinical images: a rare presentation of colonic pseudoobstruction in CREST. PG - 3836 LID - 10.1002/art.27725 [doi] FAU - Nanda, Sudip AU - Nanda S AD - St. Luke's Hospital, Bethlehem, PA, USA. FAU - Longo, Santo AU - Longo S LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - CREST Syndrome/*complications MH - Colonic Pseudo-Obstruction/*diagnosis/*etiology MH - Colonoscopy MH - Female MH - Humans MH - Middle Aged EDAT- 2010/12/15 06:00 MHDA- 2011/03/10 06:00 CRDT- 2010/12/15 06:00 PHST- 2010/12/15 06:00 [entrez] PHST- 2010/12/15 06:00 [pubmed] PHST- 2011/03/10 06:00 [medline] AID - 10.1002/art.27725 [doi] PST - ppublish SO - Arthritis Rheum. 2010 Dec;62(12):3836. doi: 10.1002/art.27725. PMID- 20920690 OWN - NLM STAT- MEDLINE DCOM- 20101025 LR - 20101005 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 123 IP - 10 DP - 2010 Oct TI - Images in dermatology. A plethora of protein. Primary localized cutaneous nodular amyloidosis. PG - 904-6 LID - 10.1016/j.amjmed.2010.06.009 [doi] FAU - Damian, Diona L AU - Damian DL AD - Department of Dermatology, Sydney Cancer Centre, University of Sydney at Royal Prince Alfred Hospital, Camperdown, NSW, Australia. diona.damian@sswahs.nsw.gov.au FAU - Bertouch, Jim V AU - Bertouch JV LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 SB - IM MH - Amyloidosis/*diagnosis/pathology/therapy MH - CREST Syndrome/diagnosis/pathology MH - Combined Modality Therapy MH - Ear, External/pathology MH - Humans MH - Leg MH - Male MH - Middle Aged MH - Skin Diseases/*diagnosis/pathology/therapy EDAT- 2010/10/06 06:00 MHDA- 2010/10/26 06:00 CRDT- 2010/10/06 06:00 PHST- 2010/02/24 00:00 [received] PHST- 2010/06/16 00:00 [revised] PHST- 2010/06/16 00:00 [accepted] PHST- 2010/10/06 06:00 [entrez] PHST- 2010/10/06 06:00 [pubmed] PHST- 2010/10/26 06:00 [medline] AID - S0002-9343(10)00560-7 [pii] AID - 10.1016/j.amjmed.2010.06.009 [doi] PST - ppublish SO - Am J Med. 2010 Oct;123(10):904-6. doi: 10.1016/j.amjmed.2010.06.009. PMID- 20453487 OWN - NLM STAT- MEDLINE DCOM- 20110204 LR - 20131121 IS - 1423-0356 (Electronic) IS - 0025-7931 (Linking) VI - 80 IP - 5 DP - 2010 TI - Pulmonary capillary hemangiomatosis associated with CREST syndrome: a case report and review of the literature. PG - 435-8 LID - 10.1159/000314587 [doi] AB - This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease. CI - Copyright © 2010 S. Karger AG, Basel. FAU - McGuire, Franklin AU - McGuire F AD - University of South Carolina School of Medicine, 8 Medical Park, Columbia, SC 29203, USA. franklin.mcguire@uscmed.sc.edu FAU - Kennelly, Tina AU - Kennelly T FAU - Tillack, Thomas AU - Tillack T FAU - Robbins, Mark AU - Robbins M LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20100506 PL - Switzerland TA - Respiration JT - Respiration; international review of thoracic diseases JID - 0137356 RN - 0 (Antihypertensive Agents) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Antihypertensive Agents/administration & dosage/*adverse effects MH - Autopsy MH - CREST Syndrome/*complications MH - Epoprostenol/administration & dosage/*adverse effects MH - Fatal Outcome MH - Female MH - Hemangioma, Capillary/*diagnosis MH - Humans MH - Hypertension, Pulmonary/etiology MH - Lung Diseases, Interstitial/complications MH - Lung Neoplasms/*diagnosis MH - Pulmonary Edema/chemically induced MH - Pulmonary Veno-Occlusive Disease/diagnosis EDAT- 2010/05/11 06:00 MHDA- 2011/02/05 06:00 CRDT- 2010/05/11 06:00 PHST- 2009/06/25 00:00 [received] PHST- 2010/01/04 00:00 [accepted] PHST- 2010/05/11 06:00 [entrez] PHST- 2010/05/11 06:00 [pubmed] PHST- 2011/02/05 06:00 [medline] AID - 000314587 [pii] AID - 10.1159/000314587 [doi] PST - ppublish SO - Respiration. 2010;80(5):435-8. doi: 10.1159/000314587. Epub 2010 May 6. PMID- 20430883 OWN - NLM STAT- MEDLINE DCOM- 20100810 LR - 20211203 IS - 1083-351X (Electronic) IS - 0021-9258 (Print) IS - 0021-9258 (Linking) VI - 285 IP - 27 DP - 2010 Jul 2 TI - Regulation of aurora B kinase by the lipid raft protein flotillin-1. PG - 20683-90 LID - 10.1074/jbc.M110.130591 [doi] AB - The lipid raft protein Flotillin-1 was previously shown to be required for cell proliferation. Here we show that it is critical for the maintenance of the levels of the mitotic regulator Aurora B. Knockdown of Flotillin-1 induced aberrant mitotic events similar to those produced by Aurora B depletion and led to a marked decline in Aurora B levels and activity. Transfection of wild-type full-length Flotillin-1 or forms directed to the nucleus increased Aurora B levels and activity. Flotillin-1 interacted with Aurora B directly through its SPFH domain in a complex distinct from the chromosomal passenger protein complex, and the two proteins co-purified in nuclear, non-raft fractions. These observations are the first evidence for a function of Flotillin-1 outside of lipid rafts and suggest its critical role in the maintenance of a pool of active Aurora B. FAU - Gómez, Valentí AU - Gómez V AD - Unitat de Recerca Biomèdica, Institut de Recerca Hospital Vall d'Hebrón, 08035 Barcelona, Spain. FAU - Sesé, Marta AU - Sesé M FAU - Santamaría, Anna AU - Santamaría A FAU - Martínez, Juan D AU - Martínez JD FAU - Castellanos, Elisabet AU - Castellanos E FAU - Soler, Marta AU - Soler M FAU - Thomson, Timothy M AU - Thomson TM FAU - Paciucci, Rosanna AU - Paciucci R LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100429 PL - United States TA - J Biol Chem JT - The Journal of biological chemistry JID - 2985121R RN - 0 (Anti-Bacterial Agents) RN - 0 (BIRC5 protein, human) RN - 0 (DNA Primers) RN - 0 (Inhibitor of Apoptosis Proteins) RN - 0 (Membrane Proteins) RN - 0 (Microtubule-Associated Proteins) RN - 0 (Oligopeptides) RN - 0 (RNA, Small Interfering) RN - 0 (Survivin) RN - 0 (flotillins) RN - 133343-34-7 (lactacystin) RN - EC 2.7.11.1 (AURKB protein, human) RN - EC 2.7.11.1 (Aurora Kinase B) RN - EC 2.7.11.1 (Aurora Kinases) RN - EC 2.7.11.1 (Protein Serine-Threonine Kinases) RN - WYQ7N0BPYC (Acetylcysteine) RN - Y0900I3U8U (epoxomicin) SB - IM MH - Acetylcysteine/analogs & derivatives/pharmacology MH - Anti-Bacterial Agents/pharmacology MH - Aurora Kinase B MH - Aurora Kinases MH - CREST Syndrome/blood MH - Cell Division MH - Cell Nucleus/physiology MH - DNA Primers MH - Down-Regulation MH - Gene Knockdown Techniques MH - HeLa Cells MH - Humans MH - In Situ Nick-End Labeling MH - Inhibitor of Apoptosis Proteins MH - Membrane Proteins/genetics/metabolism/*pharmacology MH - Microtubule-Associated Proteins/pharmacology MH - Oligopeptides/pharmacology MH - Protein Serine-Threonine Kinases/deficiency/drug effects/*genetics/*metabolism MH - RNA, Small Interfering/chemistry/genetics MH - Reverse Transcriptase Polymerase Chain Reaction MH - Survivin MH - Transfection PMC - PMC2898292 EDAT- 2010/05/01 06:00 MHDA- 2010/08/11 06:00 PMCR- 2011/07/02 CRDT- 2010/05/01 06:00 PHST- 2010/05/01 06:00 [entrez] PHST- 2010/05/01 06:00 [pubmed] PHST- 2010/08/11 06:00 [medline] PHST- 2011/07/02 00:00 [pmc-release] AID - S0021-9258(20)57355-9 [pii] AID - M110.130591 [pii] AID - 10.1074/jbc.M110.130591 [doi] PST - ppublish SO - J Biol Chem. 2010 Jul 2;285(27):20683-90. doi: 10.1074/jbc.M110.130591. Epub 2010 Apr 29. PMID- 20524284 OWN - NLM STAT- MEDLINE DCOM- 20100707 LR - 20100607 IS - 0890-9016 (Print) IS - 0890-9016 (Linking) DP - 2009 TI - Lung and heart-lung transplantation at University of Pittsburgh: 1982-2009. PG - 179-95 AB - The Lung Transplantation program at the University of Pittsburgh began in 1982. From the beginning to December 31, 2009, 1347, lung and heart lung transplantations have been done. (674 double-lung, 310 left single-lung, 227 right single-lung and 130 heart-lung transplantations. University of Pittsburgh maintains a data base, from the time of the inception of the program, of all recipients and donors. There is an increasing trend to do double-lung transplantation, as 5- and 10-year survival is better with double than single lungs. Our experience with heart-lung transplantation is considerable. The 4 most common indications for lung transplantation are: chronic obstructive disease (COPD), idiopathic pulmonary fibrosis (IPF), cystic fibrosis (CF) and pulmonary arterial hypertension. (PAH). Potential recipients are evaluated over 2 weeks. There is also a pathway for accelerated evaluation of sicker patients. Our recipient criteria are expanded and flexible; as many patients, who have been denied lung transplantation, at other major centers have been successfully transplanted by us with good outcomes. The median waiting time on the list in 2009 was 37 days. Our altruistic flexibility in recipient selection, balanced with respect to the altruistic gift of donor families, has been described in considerable detail. To solve the problem of shortage of donor lungs, we have expanded our donor selection criteria beyond the historic ideal donor, without comprising on outcomes. These selection criteria and our donor-lungs management protocol are also described in reasonable detail. We started using lungs from donors after cardiac death (DCD) in January 2007. Recently we reviewed our experience and literature, and devised a protocol; which is given in a robust table. We also participate as faculty in the educational program initiated by the Organ Procurement Organizations (OPO) of Pennsylvania. During procurement we use 500 microg of prostaglandin E-1 into the pulmonary artery just before X clamp, Perfedex infusion after X-clamp, at 70 ml/kg, with 500 microg of prostaglandin and 50 mg of nitroglycerine in the first bag, and retrograde flush of 500 ccs in each pulmonary vein. We have refined our recipient operation, from clam shell incision to bilateral antero-axillary incisions, preserving the sternum and internal mammary arteries, for both double and single-lung transplantation, with good outcomes. This technique and results have been described in generous detail. We use pneumoplegia, similar to cardioplegia, to protect the allograft from ischemic and reperfusion injury (Appendix III). Our technique of bronchial anastomosis and intraoperative management of septic lung disease has remained unchanged. Post-operatively we continue to use the ventilatory management of low FiO2 and high PEEP. Our immunosuppression and infection prophylaxis protocol is the same since 2003, when we started using Alemtuzumab (Campath) for induction, with minimization of the use of steroids. For maintenance, we use Tacrolimus and Mycophenolate Mofetil. Now, we also monitor the functional activity of the T cell by Cylex ImmuKnow. Lowered activity (< 100 ng/ml) suggests an increased risk of infection; and higher activity (> 200 ng/ml) suggests greater risks for rejection. Although we have expanded our recipient and donor pools, our outcomes have continued to improve. The overall survival of double-lung transplantation from 2003-2009 was 82.8% at one year and 56.8% at 5 years. This compares well with the international data which shows an overall survival rate of 80% at one year and 56% at 5 years. Results of lung transplantation will continue to improve, with our increasing understanding of mechanisms and management of ischemic-reperfusion injury, acute rejection and bronchiolitis obliterans syndrome. FAU - Thacker, Jnanesh AU - Thacker J AD - The Heart, Lung and Esophageal Surgery Institute, Division of Cardiac Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA. FAU - Toyoda, Yoshiya AU - Toyoda Y LA - eng PT - Journal Article PL - United States TA - Clin Transpl JT - Clinical transplants JID - 8812419 RN - 0 (Immunosuppressive Agents) SB - IM MH - CREST Syndrome/surgery MH - Death MH - Heart-Lung Transplantation/immunology/mortality/*statistics & numerical data MH - Hospitals, University MH - Humans MH - Hypertension, Pulmonary/surgery MH - Immunosuppressive Agents/therapeutic use MH - Living Donors MH - Lung Diseases/classification/surgery MH - Lung Transplantation/immunology/mortality/*statistics & numerical data MH - Patient Selection MH - Pennsylvania MH - Sarcoidosis/surgery MH - Scleroderma, Systemic/mortality/surgery MH - Survival Rate MH - Survivors MH - Tissue Donors/statistics & numerical data EDAT- 2009/01/01 00:00 MHDA- 2010/07/08 06:00 CRDT- 2010/06/08 06:00 PHST- 2010/06/08 06:00 [entrez] PHST- 2009/01/01 00:00 [pubmed] PHST- 2010/07/08 06:00 [medline] PST - ppublish SO - Clin Transpl. 2009:179-95. PMID- 18480507 OWN - NLM STAT- MEDLINE DCOM- 20080805 LR - 20080515 IS - 0889-5899 (Print) IS - 0889-5899 (Linking) VI - 54 IP - 4 DP - 2008 Apr TI - Acoustic pressure wound therapy in the treatment of a vasculopathy-associated digital ulcer: a case study. PG - 62-5 AB - Digital vasculopathy and subsequent digital ulceration are common and painful complications of limited cutaneous systemic sclerosis. Although the use of hydrocolloid occlusive dressings has been found to reduce pain, frequently required surgical or chemical debridement can be intensely painful in such ulcers. Acoustic pressure wound therapy is a noncontact, low-frequency ultrasound therapy used for painless debridement in a variety of acute and chronic wounds. It was administered to treat an intensely painful, methicillin-resistant Staphylococcus aureus-infected finger ulcer resulting from peripheral, bilateral vasculopathy in a 68-year-old man with a history of three prior fingertip amputations secondary to limited cutaneous systemic sclerosis-associated digital vasculopathy. At treatment initiation, 90% of the 11 cm2 wound was covered with firmly adherent fibrin slough. Acoustic pressure wound therapy was performed three times weekly for 5 minutes per treatment and the wound was covered with a hydrocolloid occlusive dressings. Pain scores decreased from 10 (visual analog scale, 0 = none, 10 = extreme) at the beginning of treatment to 0 at the week 8 assessment and his analgesics were discontinued. After 10 weeks (31 acoustic pressure wound therapy treatments), the wound was completely closed. FAU - Fleming, Christopher Paul AU - Fleming CP AD - Monticello-Big Lake Hospital Wound Care Center, Monticello, Minnesota, USA. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Ostomy Wound Manage JT - Ostomy/wound management JID - 8912029 MH - *Acoustics MH - Aged MH - CREST Syndrome/complications/*therapy MH - Fingers MH - Humans MH - Male MH - *Negative-Pressure Wound Therapy MH - Ulcer/complications/*therapy MH - Wound Healing EDAT- 2008/05/16 09:00 MHDA- 2008/08/06 09:00 CRDT- 2008/05/16 09:00 PHST- 2008/05/16 09:00 [pubmed] PHST- 2008/08/06 09:00 [medline] PHST- 2008/05/16 09:00 [entrez] PST - ppublish SO - Ostomy Wound Manage. 2008 Apr;54(4):62-5. PMID- 18265812 OWN - NLM STAT- MEDLINE DCOM- 20080401 LR - 20131121 IS - 0035-3639 (Print) IS - 0035-3639 (Linking) VI - 28 IP - 6 DP - 2007 Nov-Dec TI - [Autoimmune hepatitis and CREST syndrome]. PG - 528-31 AB - We report the case of an autoimmune hepatitis in a 59-year old woman who was referred for a progressive jaundice. The patient had an history of CREST syndrome. The particularity of this case report is the rare association between these two autoimmune diseases. FAU - Ngo Mandag, N AU - Ngo Mandag N AD - Service de Gastroentérologie, C.H.U. Saint-Pierre, Site César De Paepe. FAU - Van Gossum, M AU - Van Gossum M FAU - Rickaert, F AU - Rickaert F FAU - Golstein, M AU - Golstein M LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Hépatite auto-immune associée au syndrome de CREST. PL - Belgium TA - Rev Med Brux JT - Revue medicale de Bruxelles JID - 8003474 RN - 0 (Immunosuppressive Agents) RN - 9PHQ9Y1OLM (Prednisolone) RN - MRK240IY2L (Azathioprine) SB - IM MH - Azathioprine/therapeutic use MH - CREST Syndrome/*complications/drug therapy MH - Female MH - Hepatitis, Autoimmune/*complications/drug therapy MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Jaundice/*complications/drug therapy MH - Middle Aged MH - Prednisolone/therapeutic use EDAT- 2008/02/13 09:00 MHDA- 2008/04/02 09:00 CRDT- 2008/02/13 09:00 PHST- 2008/02/13 09:00 [pubmed] PHST- 2008/04/02 09:00 [medline] PHST- 2008/02/13 09:00 [entrez] PST - ppublish SO - Rev Med Brux. 2007 Nov-Dec;28(6):528-31. PMID- 16757644 OWN - NLM STAT- MEDLINE DCOM- 20060803 LR - 20181113 IS - 0095-1137 (Print) IS - 1098-660X (Electronic) IS - 0095-1137 (Linking) VI - 44 IP - 6 DP - 2006 Jun TI - Fatal myocardial necrosis caused by Staphylococcus lugdunensis and cytomegalovirus in a patient with scleroderma. PG - 2295-7 AB - A 42-year-old woman developed a rapidly progressing fatal heart failure. At the autopsy extensive necrosis of the myocardium was seen, with an almost complete absence of inflammatory cells and the presence of bacterial structures identified as Staphylococcus lugdunensis by PCR. In addition, the cytomegalovirus genome was found to be located inside the cardiomyocytes. FAU - Pirilä, Laura AU - Pirilä L AD - Department of Medicine, University of Turku, Turku, Finland. FAU - Söderström, Karl-Ove AU - Söderström KO FAU - Hietarinta, Marja AU - Hietarinta M FAU - Jalava, Jari AU - Jalava J FAU - Kytö, Ville AU - Kytö V FAU - Toivanen, Auli AU - Toivanen A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Microbiol JT - Journal of clinical microbiology JID - 7505564 SB - IM MH - Adult MH - CREST Syndrome/*complications MH - Cytomegalovirus/*isolation & purification MH - Cytomegalovirus Infections/microbiology MH - Fatal Outcome MH - Female MH - Humans MH - Muscle Cells/microbiology/virology MH - Myocarditis/*microbiology/*virology MH - Myocardium/pathology MH - Necrosis MH - Staphylococcal Infections/microbiology MH - Staphylococcus/*isolation & purification PMC - PMC1489446 EDAT- 2006/06/08 09:00 MHDA- 2006/08/04 09:00 PMCR- 2006/10/01 CRDT- 2006/06/08 09:00 PHST- 2006/06/08 09:00 [pubmed] PHST- 2006/08/04 09:00 [medline] PHST- 2006/06/08 09:00 [entrez] PHST- 2006/10/01 00:00 [pmc-release] AID - 44/6/2295 [pii] AID - 0002-06 [pii] AID - 10.1128/JCM.00002-06 [doi] PST - ppublish SO - J Clin Microbiol. 2006 Jun;44(6):2295-7. doi: 10.1128/JCM.00002-06. PMID- 16391265 OWN - NLM STAT- MEDLINE DCOM- 20060207 LR - 20160512 IS - 0021-9355 (Print) IS - 0021-9355 (Linking) VI - 88 IP - 1 DP - 2006 Jan TI - Tumoral calcinosis in the cervical spine in a patient with CREST syndrome. A case report. PG - 193-7 FAU - Teng, Andelle L AU - Teng AL AD - Department of Orthopaedic Surgery, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA. ateng_md@yahoo.com FAU - Robbin, Mark R AU - Robbin MR FAU - Furey, Christopher G AU - Furey CG FAU - Easley, Samantha E AU - Easley SE FAU - Abdul-Karim, Fadi W AU - Abdul-Karim FW FAU - Bohlman, Henry H AU - Bohlman HH LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Bone Joint Surg Am JT - The Journal of bone and joint surgery. American volume JID - 0014030 RN - 9007-34-5 (Collagen) RN - H0G9379FGK (Calcium Carbonate) SB - IM MH - Biopsy MH - CREST Syndrome/*diagnosis MH - Calcinosis/*diagnosis MH - Calcium Carbonate/analysis MH - Cervical Vertebrae/*pathology MH - Collagen/analysis MH - Connective Tissue/pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Image Processing, Computer-Assisted MH - Magnetic Resonance Imaging MH - Middle Aged MH - Muscle, Skeletal/pathology MH - Neck/*pathology MH - Spinal Diseases/*diagnosis MH - Spinal Fusion MH - Tomography, X-Ray Computed/methods EDAT- 2006/01/05 09:00 MHDA- 2006/02/08 09:00 CRDT- 2006/01/05 09:00 PHST- 2006/01/05 09:00 [pubmed] PHST- 2006/02/08 09:00 [medline] PHST- 2006/01/05 09:00 [entrez] AID - 88/1/193 [pii] AID - 10.2106/JBJS.E.00536 [doi] PST - ppublish SO - J Bone Joint Surg Am. 2006 Jan;88(1):193-7. doi: 10.2106/JBJS.E.00536. PMID- 38850086 OWN - NLM STAT- MEDLINE DCOM- 20240608 LR - 20240619 IS - 1651-2057 (Electronic) IS - 0001-5555 (Print) IS - 0001-5555 (Linking) VI - 104 DP - 2024 Jun 8 TI - A 71-year-old Woman with CREST Syndrome and Multiple Waxy Facial Papules and Plaques: A Quiz. PG - adv40419 LID - 10.2340/actadv.v104.40419 [doi] LID - 40419 FAU - Planella-Fontanillas, Nidia AU - Planella-Fontanillas N AD - Department of Dermatology, Hospital del Mar, Barcelona, Spain; Universitat Autònoma de Barcelona (UAB), Barcelona Spain. nidia.planella.fontanillas@psmar.cat. FAU - Pesqué, David AU - Pesqué D AD - Department of Dermatology, Hospital del Mar, Barcelona, Spain; Universitat Autònoma de Barcelona (UAB), Barcelona Spain. FAU - Parra-Navarro, Laura AU - Parra-Navarro L AD - Department of Dermatology, Hospital del Mar, Barcelona, Spain; Universitat Autònoma de Barcelona (UAB), Barcelona Spain. FAU - Pujol, Ramon M AU - Pujol RM AD - Department of Dermatology, Hospital del Mar, Barcelona, Spain; Universitat Pompeu Fabra (UPF). Barcelona, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20240608 PL - Sweden TA - Acta Derm Venereol JT - Acta dermato-venereologica JID - 0370310 SB - IM MH - Humans MH - Female MH - Aged MH - *CREST Syndrome/complications/pathology MH - Facial Dermatoses/pathology/diagnosis MH - Biopsy MH - Skin/pathology PMC - PMC11181914 EDAT- 2024/06/08 10:45 MHDA- 2024/06/08 10:46 PMCR- 2024/06/08 CRDT- 2024/06/08 04:53 PHST- 2024/03/26 00:00 [received] PHST- 2024/05/15 00:00 [accepted] PHST- 2024/06/08 10:46 [medline] PHST- 2024/06/08 10:45 [pubmed] PHST- 2024/06/08 04:53 [entrez] PHST- 2024/06/08 00:00 [pmc-release] AID - ActaDV-104-40419 [pii] AID - 10.2340/actadv.v104.40419 [doi] PST - epublish SO - Acta Derm Venereol. 2024 Jun 8;104:adv40419. doi: 10.2340/actadv.v104.40419. PMID- 35144811 OWN - NLM STAT- MEDLINE DCOM- 20230131 LR - 20230202 IS - 1943-4723 (Electronic) IS - 0002-8177 (Linking) VI - 154 IP - 2 DP - 2023 Feb TI - Red and white lesion of the tongue in a patient with cutaneous findings. PG - 174-179 LID - S0002-8177(21)00659-0 [pii] LID - 10.1016/j.adaj.2021.10.007 [doi] FAU - Menon, Reshma S AU - Menon RS FAU - Tavares, Tiffany AU - Tavares T FAU - Woo, Sook-Bin AU - Woo SB LA - eng PT - Case Reports PT - Journal Article DEP - 20220208 PL - England TA - J Am Dent Assoc JT - Journal of the American Dental Association (1939) JID - 7503060 SB - IM MH - Humans MH - *Tongue/pathology MH - Tongue Neoplasms/pathology MH - Female MH - Aged MH - *CREST Syndrome/diagnosis EDAT- 2022/02/12 06:00 MHDA- 2023/01/31 06:00 CRDT- 2022/02/11 05:32 PHST- 2021/06/25 00:00 [received] PHST- 2021/09/22 00:00 [revised] PHST- 2021/10/21 00:00 [accepted] PHST- 2022/02/12 06:00 [pubmed] PHST- 2023/01/31 06:00 [medline] PHST- 2022/02/11 05:32 [entrez] AID - S0002-8177(21)00659-0 [pii] AID - 10.1016/j.adaj.2021.10.007 [doi] PST - ppublish SO - J Am Dent Assoc. 2023 Feb;154(2):174-179. doi: 10.1016/j.adaj.2021.10.007. Epub 2022 Feb 8. PMID- 32557599 OWN - NLM STAT- MEDLINE DCOM- 20210617 LR - 20210617 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 59 IP - 9 DP - 2020 Sep TI - Pseudo-ainhum in a patient with CREST syndrome. PG - 1163-1164 LID - 10.1111/ijd.15014 [doi] FAU - Darlenski, Razvigor AU - Darlenski R AD - Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria. AD - Department of Dermatology and Venereology, Medical Faculty, Trakia University, Stara Zagora, Bulgaria. FAU - Popov, Yanko AU - Popov Y AD - Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria. FAU - Dourmishev, Lyubomir AU - Dourmishev L AD - Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria. FAU - Balabanova, Maria AU - Balabanova M AD - Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria. FAU - Vassileva, Snejina AU - Vassileva S AD - Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria. LA - eng PT - Letter DEP - 20200618 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 SB - IM MH - *Ainhum MH - *CREST Syndrome MH - Constriction, Pathologic MH - Humans EDAT- 2020/06/20 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/06/20 06:00 PHST- 2020/03/23 00:00 [received] PHST- 2020/05/11 00:00 [revised] PHST- 2020/05/28 00:00 [accepted] PHST- 2020/06/20 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/06/20 06:00 [entrez] AID - 10.1111/ijd.15014 [doi] PST - ppublish SO - Int J Dermatol. 2020 Sep;59(9):1163-1164. doi: 10.1111/ijd.15014. Epub 2020 Jun 18. PMID- 30961884 OWN - NLM STAT- MEDLINE DCOM- 20200605 LR - 20200605 IS - 1578-1275 (Electronic) IS - 0212-6567 (Print) IS - 0212-6567 (Linking) VI - 51 IP - 4 DP - 2019 Apr TI - [Calcinosis and scleroderma]. PG - 259-260 LID - S0212-6567(18)30354-8 [pii] LID - 10.1016/j.aprim.2018.05.011 [doi] FAU - Pujol Olivares, Xavier AU - Pujol Olivares X AD - Medicina de Familia y Comunitaria, EAP Ripoll, Sant Joan de les Abadesses, Girona, España. Electronic address: xpujol.girona.ics@gencat.cat. LA - spa PT - Case Reports PT - Journal Article TT - Calcinosis y esclerodermia. DEP - 20181026 PL - Spain TA - Aten Primaria JT - Atencion primaria JID - 9111075 SB - IM MH - CREST Syndrome/diagnosis MH - Calcinosis/*complications/diagnosis/pathology MH - Diagnosis, Differential MH - Elbow MH - Female MH - Gout/diagnosis MH - Humans MH - Middle Aged MH - Scleroderma, Localized/*complications/pathology PMC - PMC6836910 EDAT- 2019/04/10 06:00 MHDA- 2020/06/06 06:00 PMCR- 2018/10/26 CRDT- 2019/04/10 06:00 PHST- 2018/05/23 00:00 [received] PHST- 2018/05/25 00:00 [accepted] PHST- 2019/04/10 06:00 [entrez] PHST- 2019/04/10 06:00 [pubmed] PHST- 2020/06/06 06:00 [medline] PHST- 2018/10/26 00:00 [pmc-release] AID - S0212-6567(18)30354-8 [pii] AID - 10.1016/j.aprim.2018.05.011 [doi] PST - ppublish SO - Aten Primaria. 2019 Apr;51(4):259-260. doi: 10.1016/j.aprim.2018.05.011. Epub 2018 Oct 26. PMID- 30691950 OWN - NLM STAT- MEDLINE DCOM- 20211006 LR - 20211006 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 16 IP - 6 DP - 2020 Nov-Dec TI - Chronic Anterior Uveitis in a Patient with CREST Syndrome. PG - 497-498 LID - S1699-258X(18)30234-1 [pii] LID - 10.1016/j.reuma.2018.10.005 [doi] AB - Systemic sclerosis is a connective tissue pathology with very heterogeneous clinical manifestations, associated in a small percentage with inflammatory eye diseases. In the specific case of uveitis, only isolated cases have been reported in the literature, especially in relation to the CREST syndrome. We present the case of a 53-year-old woman with CREST syndrome and chronic anterior uveitis, which we consider of clinical relevance given its low prevalence. CI - Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Fuente Cosío, Sara AU - Fuente Cosío S AD - Medicina Interna, Hospital Comarcal de Jarrio, Coaña, Asturias, España. Electronic address: sarafuentecosio@gmail.com. FAU - Arca Barca, Beatriz AU - Arca Barca B AD - Reumatología, Hospital Universitario San Agustín, Avilés, Asturias, España. FAU - Martínez García, Paula AU - Martínez García P AD - Medicina Interna, Hospital Universitario San Agustín, Avilés, Asturias, España. FAU - Sampedro López, Antonio AU - Sampedro López A AD - Oftalmología, Hospital Universitario San Agustín, Avilés, Asturias, España. LA - eng LA - spa PT - Case Reports PT - Journal Article TT - Uveítis anterior crónica en paciente con síndrome de CREST. DEP - 20190125 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Aged, 80 and over MH - CREST Syndrome/*complications MH - Chronic Disease MH - Female MH - Humans MH - Uveitis, Anterior/*etiology OTO - NOTNLM OT - CREST syndrome OT - Esclerosis sistémica OT - Systemic sclerosis OT - Síndrome de CREST OT - Uveitis OT - Uveítis EDAT- 2019/01/30 06:00 MHDA- 2021/10/07 06:00 CRDT- 2019/01/30 06:00 PHST- 2018/07/11 00:00 [received] PHST- 2018/09/25 00:00 [revised] PHST- 2018/10/18 00:00 [accepted] PHST- 2019/01/30 06:00 [pubmed] PHST- 2021/10/07 06:00 [medline] PHST- 2019/01/30 06:00 [entrez] AID - S1699-258X(18)30234-1 [pii] AID - 10.1016/j.reuma.2018.10.005 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2020 Nov-Dec;16(6):497-498. doi: 10.1016/j.reuma.2018.10.005. Epub 2019 Jan 25. PMID- 30343710 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1532-1916 (Electronic) IS - 1521-6918 (Linking) VI - 34-35 DP - 2018 Jun-Aug TI - PBC and related extrahepatic diseases. PG - 49-54 LID - S1521-6918(18)30009-X [pii] LID - 10.1016/j.bpg.2018.05.013 [doi] AB - Patients with PBC have at least 60% of probability to have an autoimmune extrahepatic condition. The pathogenesis of these conditions includes a common mechanism involving both innate and adaptive immune responses targeting cholangiocytes and different extrahepatic tissues. The recent EASL guidelines recommend the management of these conditions, although detailed practical treatments have not been indicated. Autoimmune extrahepatic conditions may include: rheumatologic, endocrine, pulmonary, gastrointestinal, dermatologic diseases. This review aims to focus the most important extrahepatic autoimmune conditions associated to PBC with practical recommendation regarding diagnostic approach and management. CI - Copyright © 2018 Elsevier Ltd. All rights reserved. FAU - Floreani, Annarosa AU - Floreani A AD - Dept. of Surgery, Oncology and Gastroenterology, University of Padova, Italy. Electronic address: annarosa.floreani@unipd.it. FAU - Cazzagon, Nora AU - Cazzagon N AD - Dept. of Surgery, Oncology and Gastroenterology, University of Padova, Italy. LA - eng PT - Journal Article PT - Review DEP - 20180523 PL - Netherlands TA - Best Pract Res Clin Gastroenterol JT - Best practice & research. Clinical gastroenterology JID - 101120605 SB - IM MH - Arthritis, Rheumatoid/diagnosis/etiology MH - Autoimmune Diseases/diagnosis/*etiology MH - CREST Syndrome/diagnosis/etiology MH - Humans MH - Liver Cirrhosis, Biliary/*complications/diagnosis MH - Lung Diseases/diagnosis/etiology MH - Scleroderma, Systemic/diagnosis/etiology MH - Sjogren's Syndrome/diagnosis/etiology MH - Skin Diseases/diagnosis/etiology MH - Thyroid Diseases/diagnosis/etiology OTO - NOTNLM OT - CREST disease OT - Dermatological diseases OT - Extrahepatic autoimmune conditions OT - Primary biliary cholangitis OT - Primary biliary cirrhosis OT - Pulmonary diseases OT - Rheumatoid arthritis OT - Scleroderma OT - Sjogren's syndrome OT - Thyroid diseases EDAT- 2018/10/23 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/10/23 06:00 PHST- 2018/01/17 00:00 [received] PHST- 2018/05/22 00:00 [accepted] PHST- 2018/10/23 06:00 [entrez] PHST- 2018/10/23 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] AID - S1521-6918(18)30009-X [pii] AID - 10.1016/j.bpg.2018.05.013 [doi] PST - ppublish SO - Best Pract Res Clin Gastroenterol. 2018 Jun-Aug;34-35:49-54. doi: 10.1016/j.bpg.2018.05.013. Epub 2018 May 23. PMID- 29987861 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20181211 IS - 1479-8301 (Electronic) IS - 1346-3500 (Linking) VI - 18 IP - 5 DP - 2018 Sep TI - Imaging studies of kleptomania in a middle-aged woman with obsessive-compulsive disorder: a case report. PG - 430-433 LID - 10.1111/psyg.12339 [doi] AB - A 57-year-old woman who had been arrested for shoplifting visited our hospital. She was diagnosed with kleptomania. She had previously been diagnosed with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and obsessive-compulsive disorder. Cranial magnetic resonance imaging showed mild atrophy of the bilateral dorsolateral prefrontal cortices, left hippocampus, and occipital cortex, as well as diffuse mild T(2) hyperintensity in the deep and subcortical white matter, including the frontal region. During a single-photon emission computed tomography scan, significant hyperperfusion was observed in the right ventral striatum, including the nucleus accumbens, ventral thalamus, and right ventrolateral prefrontal areas. Patchy hypoperfusion was found in the bilateral posterior cingulate, parietal, and occipital regions. The patient's neurocognitive function was normal, except for slight impairment of her executive function. Her symptoms and neuroimaging findings were not suggestive of a specific neurocognitive disorder. Hyperactivity of the right ventral striatum may contribute to both obsessive-compulsive disorder and kleptomania. Although frontotemporal lobar degeneration is a major neurocognitive disorder related to illegal behaviours, CREST syndrome-induced white matter microstructural damage in the orbitofrontal lobe could have caused our patient's kleptomania. CI - © 2018 Japanese Psychogeriatric Society. FAU - Midorikawa, Haruhiko AU - Midorikawa H AD - University of Tsukuba Hospital, Tsukuba, Japan. FAU - Ide, Masayuki AU - Ide M AUID- ORCID: 0000-0002-8659-8247 AD - Department of Psychiatry, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan. FAU - Nemoto, Kiyotaka AU - Nemoto K AUID- ORCID: 0000-0001-8623-9829 AD - Department of Psychiatry, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan. FAU - Mizukami, Katsuyoshi AU - Mizukami K AD - Faculty of Health and Sport Sciences, University of Tsukuba, Tsukuba, Japan. FAU - Asada, Takashi AU - Asada T AD - Department of Psychiatry, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan. FAU - Arai, Tetsuaki AU - Arai T AD - Department of Psychiatry, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20180710 PL - England TA - Psychogeriatrics JT - Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society JID - 101230058 SB - IM MH - Atrophy/diagnostic imaging MH - Brain/*diagnostic imaging MH - CREST Syndrome/diagnosis MH - Disruptive, Impulse Control, and Conduct Disorders/*diagnostic imaging MH - Female MH - Humans MH - *Magnetic Resonance Imaging MH - Middle Aged MH - Obsessive-Compulsive Disorder/complications/*diagnosis MH - *Tomography, Emission-Computed, Single-Photon OTO - NOTNLM OT - CREST syndrome OT - SPECT OT - kleptomania OT - nucleus accumbens OT - obsessive-compulsive disorder OT - orbitofrontal cortex EDAT- 2018/07/11 06:00 MHDA- 2018/12/12 06:00 CRDT- 2018/07/11 06:00 PHST- 2017/09/06 00:00 [received] PHST- 2018/02/12 00:00 [revised] PHST- 2018/02/16 00:00 [accepted] PHST- 2018/07/11 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/07/11 06:00 [entrez] AID - 10.1111/psyg.12339 [doi] PST - ppublish SO - Psychogeriatrics. 2018 Sep;18(5):430-433. doi: 10.1111/psyg.12339. Epub 2018 Jul 10. PMID- 27583424 OWN - NLM STAT- MEDLINE DCOM- 20170317 LR - 20170817 IS - 1532-0650 (Electronic) IS - 0002-838X (Linking) VI - 94 IP - 5 DP - 2016 Sep 1 TI - Nontender Elbow Nodules. PG - 375-6 FAU - Cordts, Scott AU - Cordts S AD - Deaconess Family Medicine Residency, Evansville, Indiana, USA. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 SB - IM MH - Aged MH - Arthritis/*diagnosis/diagnostic imaging MH - Arthritis, Rheumatoid/diagnosis/diagnostic imaging MH - CREST Syndrome MH - Diagnosis, Differential MH - *Elbow MH - Female MH - Humans MH - Osteoarthritis/diagnosis/diagnostic imaging EDAT- 2016/09/02 06:00 MHDA- 2017/03/18 06:00 CRDT- 2016/09/02 06:00 PHST- 2016/09/02 06:00 [entrez] PHST- 2016/09/02 06:00 [pubmed] PHST- 2017/03/18 06:00 [medline] AID - d12722 [pii] PST - ppublish SO - Am Fam Physician. 2016 Sep 1;94(5):375-6. PMID- 27072730 OWN - NLM STAT- MEDLINE DCOM- 20180913 LR - 20181202 IS - 1540-9740 (Print) IS - 1540-9740 (Linking) VI - 14 IP - 1 DP - 2016 TI - Crest Syndrome. PG - 43 FAU - Kumar, Piyush AU - Kumar P AD - Department of Dermatology, Katihar Medical College and Hospital, Katihar, India; docpiyush99@gmail.com. FAU - Das, Anupam AU - Das A AD - KPC Medical College and Hospital, Kolkata, West Bengal, India. LA - eng PT - Journal Article DEP - 20160201 PL - United States TA - Skinmed JT - Skinmed JID - 101168327 SB - IM MH - *CREST Syndrome MH - Humans EDAT- 2016/04/14 06:00 MHDA- 2018/09/14 06:00 CRDT- 2016/04/14 06:00 PHST- 2016/04/14 06:00 [entrez] PHST- 2016/04/14 06:00 [pubmed] PHST- 2018/09/14 06:00 [medline] PST - epublish SO - Skinmed. 2016 Feb 1;14(1):43. eCollection 2016. PMID- 26513367 OWN - NLM STAT- MEDLINE DCOM- 20160829 LR - 20161126 IS - 1423-0291 (Electronic) IS - 1015-2008 (Linking) VI - 82 IP - 6 DP - 2015 TI - Amyloidosis of the Breast: Three Different and Unusual Presentations of a Rare Entity. PG - 264-8 LID - 10.1159/000440866 [doi] AB - BACKGROUND: Amyloidosis involving the breast is a rare finding and it may present as a solitary mass called 'amyloid tumor'. According to the largest case series, the amyloid deposits are usually of the AL type (commonly x03BA; light chain). METHODS: We report 3 cases diagnosed at our institution in the period from 2000 to 2015. Radiological, histological and immunohistochemical studies were performed. RESULTS AND CONCLUSIONS: Together with a case presenting in a patient with multiple myeloma, we describe 2 unique presentations including 1 associated with CREST syndrome in a patient with a previous history of breast carcinoma and another, also associated with cancer, with transthyretin deposits in a woman with a TTR gene mutation and a family history of familial amyloidotic polyneuropathy. These cases are an example of the vast heterogeneity of this disorder regarding its clinical presentation, the type of amyloid deposits and other diseases associated with breast amyloidosis. CI - © 2015 S. Karger AG, Basel. FAU - Herrero, Laura AU - Herrero L AD - Department of Pathology, Hospital Clinic, University of Barcelona, Barcelona, Spain. FAU - Naranjo-Hans, Dolores AU - Naranjo-Hans D FAU - Solé, Manel AU - Solé M FAU - Santamaría, Gorane AU - Santamaría G FAU - Bargalló, Xavier AU - Bargalló X FAU - Velasco, Martín AU - Velasco M FAU - Fernández, Pedro Luis AU - Fernández PL LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20151030 PL - Switzerland TA - Pathobiology JT - Pathobiology : journal of immunopathology, molecular and cellular biology JID - 9007504 RN - 0 (Prealbumin) SB - IM MH - Aged MH - Amyloid Neuropathies/complications/congenital MH - Amyloidosis/complications/*diagnosis/*pathology MH - Breast/*pathology/ultrastructure MH - Breast Neoplasms/complications MH - CREST Syndrome/complications/diagnostic imaging/pathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Multiple Myeloma/complications MH - Mutation MH - Prealbumin/genetics MH - Radiography MH - Rare Diseases EDAT- 2015/10/30 06:00 MHDA- 2016/08/30 06:00 CRDT- 2015/10/30 06:00 PHST- 2015/08/18 00:00 [received] PHST- 2015/09/04 00:00 [accepted] PHST- 2015/10/30 06:00 [entrez] PHST- 2015/10/30 06:00 [pubmed] PHST- 2016/08/30 06:00 [medline] AID - 000440866 [pii] AID - 10.1159/000440866 [doi] PST - ppublish SO - Pathobiology. 2015;82(6):264-8. doi: 10.1159/000440866. Epub 2015 Oct 30. PMID- 26125693 OWN - NLM STAT- MEDLINE DCOM- 20151222 LR - 20150701 IS - 2325-7237 (Electronic) IS - 2325-7237 (Linking) VI - 5 IP - 1 DP - 2015 Jul 1 TI - Perioperative Glucose Management: Point-of-Care Testing and Pseudohypoglycemia. PG - 13-4 LID - 10.1213/XAA.0000000000000164 [doi] AB - Perioperative hypoglycemia has been associated with adverse outcomes. Consequently, perioperative monitoring of blood glucose using convenient point-of-care (POC) monitors is frequently used. Although venous or arterial glucose POC testing has been cleared for use in critically ill hospitalized patients, the results of capillary glucose POC testing should be interpreted with caution because capillary POC samples are usually less reliable than those obtained from arterial or venous sites. We describe a case of pseudohypoglycemia using such testing. This case highlights the importance of individualizing perioperative glucose management to venous or arterial rather than capillary sampling in certain clinical situations. FAU - Radosevich, Misty A AU - Radosevich MA AD - From the Department of Anesthesiology, Mayo Clinic, Rochester, Minnesota. FAU - Narr, Bradly J AU - Narr BJ FAU - Curry, Timothy B AU - Curry TB FAU - Johnson, Rebecca L AU - Johnson RL LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - A A Case Rep JT - A & A case reports JID - 101637720 RN - 0 (Blood Glucose) SB - IM MH - Aged MH - Arthroplasty, Replacement, Knee MH - Blood Glucose/*analysis MH - CREST Syndrome/complications MH - Critical Illness MH - Female MH - Humans MH - Hypoglycemia/complications/*diagnosis MH - Perioperative Care MH - *Point-of-Care Testing EDAT- 2015/07/01 06:00 MHDA- 2015/12/23 06:00 CRDT- 2015/07/01 06:00 PHST- 2015/07/01 06:00 [entrez] PHST- 2015/07/01 06:00 [pubmed] PHST- 2015/12/23 06:00 [medline] AID - 01720097-201507010-00005 [pii] AID - 10.1213/XAA.0000000000000164 [doi] PST - ppublish SO - A A Case Rep. 2015 Jul 1;5(1):13-4. doi: 10.1213/XAA.0000000000000164. PMID- 25744697 OWN - NLM STAT- MEDLINE DCOM- 20150713 LR - 20191210 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 44 IP - 3 DP - 2015 May TI - Validation of the ICD-9-CM code for systemic sclerosis using updated ACR/EULAR classification criteria. PG - 253-5 LID - 10.3109/03009742.2015.1008038 [doi] FAU - Valenzuela, A AU - Valenzuela A AD - Division of Immunology and Rheumatology , Palo Alto, CA , USA. FAU - Yaqub, A AU - Yaqub A FAU - Fiorentino, D AU - Fiorentino D FAU - Krishnan, E AU - Krishnan E FAU - Chung, L AU - Chung L LA - eng PT - Letter PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Validation Study DEP - 20150306 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Aged MH - CREST Syndrome/*diagnosis MH - Europe MH - Female MH - Humans MH - International Classification of Diseases MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Retrospective Studies MH - Rheumatology MH - Scleroderma, Systemic/*diagnosis MH - Sensitivity and Specificity MH - Societies, Medical MH - United States EDAT- 2015/03/07 06:00 MHDA- 2015/07/15 06:00 CRDT- 2015/03/07 06:00 PHST- 2015/03/07 06:00 [entrez] PHST- 2015/03/07 06:00 [pubmed] PHST- 2015/07/15 06:00 [medline] AID - 10.3109/03009742.2015.1008038 [doi] PST - ppublish SO - Scand J Rheumatol. 2015 May;44(3):253-5. doi: 10.3109/03009742.2015.1008038. Epub 2015 Mar 6. PMID- 24852904 OWN - NLM STAT- MEDLINE DCOM- 20150603 LR - 20140912 IS - 1532-2653 (Electronic) IS - 0967-5868 (Linking) VI - 21 IP - 10 DP - 2014 Oct TI - Cryoglobulinemic vasculitis in a patient with CREST syndrome. PG - 1821-3 LID - S0967-5868(14)00179-9 [pii] LID - 10.1016/j.jocn.2014.01.015 [doi] AB - Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. CI - Copyright © 2014 Elsevier Ltd. All rights reserved. FAU - Hurst, Rebecca L AU - Hurst RL AD - Department of Neurology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL 32224, USA. Electronic address: hurst.rebecca@mayo.edu. FAU - Berianu, Florentina AU - Berianu F AD - Department of Rheumatology, Mayo Clinic, Jacksonville, FL, USA. FAU - Ginsburg, William W AU - Ginsburg WW AD - Department of Rheumatology, Mayo Clinic, Jacksonville, FL, USA. FAU - Klein, Christopher J AU - Klein CJ AD - Department of Neurology, Mayo Clinic, Rochester, MN, USA; Peripheral Nerve Center, Mayo Clinic, Rochester, MN, USA. FAU - Englestad, Janean K AU - Englestad JK AD - Peripheral Nerve Center, Mayo Clinic, Rochester, MN, USA. FAU - Kennelly, Kathleen D AU - Kennelly KD AD - Department of Neurology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL 32224, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20140519 PL - Scotland TA - J Clin Neurosci JT - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JID - 9433352 RN - 0 (Cryoglobulins) SB - IM MH - CREST Syndrome/*complications/pathology MH - Cryoglobulins/*metabolism MH - Female MH - Humans MH - Middle Aged MH - Sural Nerve/pathology MH - Vasculitis/*complications/drug therapy/*metabolism/pathology OTO - NOTNLM OT - CREST OT - Cryoglobulinemia OT - Cryoglobulins OT - Peripheral neuropathy OT - Sural nerve biopsy OT - Systemic sclerosis OT - Vasculitis EDAT- 2014/05/24 06:00 MHDA- 2015/06/04 06:00 CRDT- 2014/05/24 06:00 PHST- 2013/08/21 00:00 [received] PHST- 2014/01/08 00:00 [revised] PHST- 2014/01/15 00:00 [accepted] PHST- 2014/05/24 06:00 [entrez] PHST- 2014/05/24 06:00 [pubmed] PHST- 2015/06/04 06:00 [medline] AID - S0967-5868(14)00179-9 [pii] AID - 10.1016/j.jocn.2014.01.015 [doi] PST - ppublish SO - J Clin Neurosci. 2014 Oct;21(10):1821-3. doi: 10.1016/j.jocn.2014.01.015. Epub 2014 May 19. PMID- 23849984 OWN - NLM STAT- MEDLINE DCOM- 20140217 LR - 20131125 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 66 IP - 12 DP - 2013 Dec TI - Surgical treatment of extensive subcutaneous calcification of the forearm in CREST syndrome. PG - 1817-8 LID - S1748-6815(13)00388-4 [pii] LID - 10.1016/j.bjps.2013.06.035 [doi] FAU - Selig, Harald F AU - Selig HF AD - Clinic for Hand Surgery, Rhoen-Klinikum AG, Bad Neustadt/Saale, Germany; Clinic for Plastic, Hand and Reconstructive Surgery, Burn and Trauma Center, Eberhard-Karls-University Tuebingen, Tuebingen, Germany. Electronic address: selighf@gmail.com. FAU - Pillukat, Thomas AU - Pillukat T FAU - Mühldorfer-Fodor, Marion AU - Mühldorfer-Fodor M FAU - Schmitt, Stefanie AU - Schmitt S FAU - van Schoonhoven, Jörg AU - van Schoonhoven J LA - eng PT - Case Reports PT - Letter DEP - 20130711 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 SB - IM MH - Aged, 80 and over MH - CREST Syndrome/pathology/*surgery MH - Fascia/pathology MH - Female MH - Forearm/*pathology MH - Humans MH - Subcutaneous Tissue/*pathology/*surgery EDAT- 2013/07/16 06:00 MHDA- 2014/02/18 06:00 CRDT- 2013/07/16 06:00 PHST- 2013/05/15 00:00 [received] PHST- 2013/06/10 00:00 [revised] PHST- 2013/06/17 00:00 [accepted] PHST- 2013/07/16 06:00 [entrez] PHST- 2013/07/16 06:00 [pubmed] PHST- 2014/02/18 06:00 [medline] AID - S1748-6815(13)00388-4 [pii] AID - 10.1016/j.bjps.2013.06.035 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2013 Dec;66(12):1817-8. doi: 10.1016/j.bjps.2013.06.035. Epub 2013 Jul 11. PMID- 22381693 OWN - NLM STAT- MEDLINE DCOM- 20120904 LR - 20131121 IS - 1167-1122 (Print) IS - 1167-1122 (Linking) VI - 22 IP - 3 DP - 2012 May-Jun TI - Primary cutaneous cryptococcosis in a patient with CREST syndrome. PG - 421-2 LID - 10.1684/ejd.2012.1684 [doi] FAU - Ishikawa, Emiko AU - Ishikawa E FAU - Yoneda, Kozo AU - Yoneda K FAU - Nakai, Kozo AU - Nakai K FAU - Moriue, Junko AU - Moriue J FAU - Yokoi, Ikumi AU - Yokoi I FAU - Munehiro, Asuka AU - Munehiro A FAU - Kubota, Yasuo AU - Kubota Y LA - eng PT - Case Reports PT - Letter PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 RN - 0 (Antifungal Agents) RN - 8VZV102JFY (Fluconazole) SB - IM MH - Aged MH - Antifungal Agents/administration & dosage MH - CREST Syndrome/*complications MH - Cryptococcosis/*complications/pathology MH - Dermatomycoses/*complications/pathology MH - Fingers/pathology MH - Fluconazole/administration & dosage MH - Humans MH - Immunocompromised Host MH - Male EDAT- 2012/03/03 06:00 MHDA- 2012/09/05 06:00 CRDT- 2012/03/03 06:00 PHST- 2012/03/03 06:00 [entrez] PHST- 2012/03/03 06:00 [pubmed] PHST- 2012/09/05 06:00 [medline] AID - ejd.2012.1684 [pii] AID - 10.1684/ejd.2012.1684 [doi] PST - ppublish SO - Eur J Dermatol. 2012 May-Jun;22(3):421-2. doi: 10.1684/ejd.2012.1684. PMID- 22214628 OWN - NLM STAT- MEDLINE DCOM- 20120524 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 51 IP - 1 DP - 2012 TI - Slowly progressive insulin-dependent diabetes in a patient with primary biliary cirrhosis with portal hypertension-type progression. PG - 79-82 AB - A 73-year-old woman had previously been diagnosed with CREST syndrome, PBC and diabetes. Hepatic fibrosis was not evident, in spite of the transudative ascites and active esophageal varices. ACA were positive, whereas AMA and anti-gp210 antibodies were negative. She showed low urinary excretion of C-peptide and was weakly positive for anti-GAD antibody. She was diagnosed with a form of PBC that progresses via portal hypertension rather than liver failure and with SPIDDM. Her HLA type did not contain risk allele for IDDM or PBC. SPIDDM should be considered when patients with PBC with portal hypertension-type progression develop diabetes. FAU - Takeshita, Yumie AU - Takeshita Y AD - Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Japan. FAU - Takamura, Toshinari AU - Takamura T FAU - Inoue, Oto AU - Inoue O FAU - Okumura, Miki AU - Okumura M FAU - Kato, Kenichiro AU - Kato K FAU - Sunagozaka, Hajime AU - Sunagozaka H FAU - Arai, Kuniaki AU - Arai K FAU - Misu, Hirofumi AU - Misu H FAU - Nakamura, Minoru AU - Nakamura M FAU - Nakanuma, Yasuni AU - Nakanuma Y FAU - Kaneko, Shuichi AU - Kaneko S LA - eng PT - Case Reports PT - Journal Article DEP - 20120101 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (C-Peptide) SB - IM MH - Aged MH - C-Peptide/blood MH - CREST Syndrome/complications MH - Diabetes Mellitus, Type 1/*complications MH - Disease Progression MH - Female MH - Histocompatibility Testing MH - Humans MH - Hypertension, Portal/*complications MH - Liver Cirrhosis, Biliary/*complications MH - Polyendocrinopathies, Autoimmune/complications/diagnosis/immunology EDAT- 2012/01/05 06:00 MHDA- 2012/05/25 06:00 CRDT- 2012/01/05 06:00 PHST- 2012/01/05 06:00 [entrez] PHST- 2012/01/05 06:00 [pubmed] PHST- 2012/05/25 06:00 [medline] AID - JST.JSTAGE/internalmedicine/51.6477 [pii] AID - 10.2169/internalmedicine.51.6477 [doi] PST - ppublish SO - Intern Med. 2012;51(1):79-82. doi: 10.2169/internalmedicine.51.6477. Epub 2012 Jan 1. PMID- 21827631 OWN - NLM STAT- MEDLINE DCOM- 20120126 LR - 20220419 IS - 1742-481X (Electronic) IS - 1742-4801 (Print) IS - 1742-4801 (Linking) VI - 8 IP - 5 DP - 2011 Oct TI - Non-healing leg ulcers in a patient with dystrophic calcification and crest syndrome: a challenging clinical case. PG - 537-41 LID - 10.1111/j.1742-481X.2011.00834.x [doi] AB - The management of non-healing leg ulcers in patients with CREST syndrome and subdermal calcification is rarely reported in medical literature. Only one similar case was found in the literature (1). Dealing with such patients can be a challenge for wound specialists. In this article, we discuss the clinical progress of an interesting case of extensive non-healing leg ulcers in a CREST patient with dystrophic calcification. The combination of systemic physiological deficits and immune compromise, along with the local physical abnormalities associated with the wound pose a complex multifactorial aetiological mix. There is no conclusive data on the optimal management of these wounds in CREST patients. It seems that ablation of the calcific deposits may offer some hope. CI - © 2011 The Authors. © 2011 Blackwell Publishing Ltd and Medicalhelplines.com Inc. FAU - Al-Najjar, Mahmoud AU - Al-Najjar M AD - Department of Vascular Surgery, Gold Coast Hospital, Southport, Queensland, Australia. FAU - Jackson, Mark J AU - Jackson MJ LA - eng PT - Case Reports PT - Journal Article DEP - 20110809 PL - England TA - Int Wound J JT - International wound journal JID - 101230907 RN - SML2Y3J35T (Colchicine) SB - IM MH - Aged MH - Bandages MH - CREST Syndrome/*complications MH - Calcinosis/*complications MH - Colchicine/therapeutic use MH - Diagnosis, Differential MH - Female MH - Follow-Up Studies MH - Humans MH - Leg Ulcer/diagnosis/*etiology/therapy MH - Magnetic Resonance Angiography MH - Skin Diseases/*complications/diagnosis/drug therapy MH - *Wound Healing PMC - PMC7950557 EDAT- 2011/08/11 06:00 MHDA- 2012/01/27 06:00 PMCR- 2011/08/09 CRDT- 2011/08/11 06:00 PHST- 2011/08/11 06:00 [entrez] PHST- 2011/08/11 06:00 [pubmed] PHST- 2012/01/27 06:00 [medline] PHST- 2011/08/09 00:00 [pmc-release] AID - IWJ834 [pii] AID - 10.1111/j.1742-481X.2011.00834.x [doi] PST - ppublish SO - Int Wound J. 2011 Oct;8(5):537-41. doi: 10.1111/j.1742-481X.2011.00834.x. Epub 2011 Aug 9. PMID- 21628611 OWN - NLM STAT- MEDLINE DCOM- 20110802 LR - 20211020 IS - 1942-5546 (Electronic) IS - 0025-6196 (Print) IS - 0025-6196 (Linking) VI - 86 IP - 6 DP - 2011 Jun TI - 56-year-old woman with positional headache. PG - e35-8 LID - 10.4065/mcp.2010.0801 [doi] FAU - Cullan, Allison M AU - Cullan AM AD - Mayo School of Graduate Medical Education, Mayo Clinic Arizona, Scottsdale, AZ, USA. FAU - Grover, Michael L AU - Grover ML LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mayo Clin Proc JT - Mayo Clinic proceedings JID - 0405543 SB - IM MH - *Blood Patch, Epidural MH - Brain/diagnostic imaging/*pathology MH - CREST Syndrome/complications MH - Cerebrospinal Fluid Leak MH - Cerebrospinal Fluid Rhinorrhea/complications/diagnosis/pathology MH - Diagnosis, Differential MH - Female MH - Headache/*etiology/pathology MH - Hematoma, Subdural/etiology MH - Humans MH - Intracranial Hypotension/complications/*diagnosis/etiology/pathology/therapy MH - Magnetic Resonance Imaging MH - Middle Aged MH - Nausea/etiology MH - Pain Measurement MH - *Posture MH - Severity of Illness Index MH - Tomography, X-Ray Computed MH - Vertigo/etiology MH - Vomiting/etiology PMC - PMC3104917 EDAT- 2011/06/02 06:00 MHDA- 2011/08/04 06:00 PMCR- 2011/12/01 CRDT- 2011/06/02 06:00 PHST- 2011/06/02 06:00 [entrez] PHST- 2011/06/02 06:00 [pubmed] PHST- 2011/08/04 06:00 [medline] PHST- 2011/12/01 00:00 [pmc-release] AID - S0025-6196(11)60061-X [pii] AID - 10.4065/mcp.2010.0801 [doi] PST - ppublish SO - Mayo Clin Proc. 2011 Jun;86(6):e35-8. doi: 10.4065/mcp.2010.0801. PMID- 20711101 OWN - NLM STAT- MEDLINE DCOM- 20101222 LR - 20100816 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 35 IP - 2 DP - 2010 Apr-Jun TI - [Calcinosis in scleroderma patient: to treat or not to treat?]. PG - 267-8 FAU - Gomes, Olga AU - Gomes O AD - Interna do Internato Complementar de Medicina Interna, Serviço de Reumatologia, Hospital Infante D. Pedro - E.P.E., Aveiro. olgagms@yahoo.com FAU - Barcelos, Anabela AU - Barcelos A LA - por PT - Case Reports PT - Journal Article TT - Calcinose em doente com esclerodermia: tratar ou aguardar? PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adult MH - CREST Syndrome/*complications MH - Calcinosis/*etiology MH - Female MH - Humans MH - Skin Diseases/*etiology EDAT- 2010/08/17 06:00 MHDA- 2010/12/24 06:00 CRDT- 2010/08/17 06:00 PHST- 2010/08/17 06:00 [entrez] PHST- 2010/08/17 06:00 [pubmed] PHST- 2010/12/24 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2010 Apr-Jun;35(2):267-8. PMID- 20510616 OWN - NLM STAT- MEDLINE DCOM- 20101019 LR - 20161125 IS - 1532-2653 (Electronic) IS - 0967-5868 (Linking) VI - 17 IP - 8 DP - 2010 Aug TI - Multiple cerebral aneurysms in a patient with CREST syndrome. PG - 1049-51 LID - 10.1016/j.jocn.2009.12.008 [doi] AB - Systemic sclerosis (SSc) associated with cerebral aneurysm is rare. We describe a patient with multiple cerebral aneurysms with the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) variant of SSc. A 61-year-old woman with a 20-year history of CREST syndrome was incidentally found to have four cerebral aneurysms located at the C2, C3 and C5 segments of the right internal carotid artery (ICA) and the C2 segment of the left ICA. The bilateral C2 segment aneurysms were successfully clipped using 2-stage surgery. To date, intracranial aneurysms have been reported in only two other patients with CREST syndrome. We hypothesize that the pathogenesis of the aneurysm is related to CREST syndrome. Elucidating the true incidence of cerebral aneurysms associated with CREST syndrome would help to clarify the relationship between SSc-related autoantibodies and aneurysm formation. CI - Copyright 2010 Elsevier Ltd. All rights reserved. FAU - Masuoka, Jun AU - Masuoka J AD - Department of Neurosurgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. masuoka@cc.saga-u.ac.jp FAU - Murao, Kenichi AU - Murao K FAU - Nagata, Izumi AU - Nagata I FAU - Iihara, Koji AU - Iihara K LA - eng PT - Case Reports PT - Journal Article DEP - 20100526 PL - Scotland TA - J Clin Neurosci JT - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JID - 9433352 SB - IM MH - CREST Syndrome/*complications MH - Female MH - Humans MH - Incidental Findings MH - Intracranial Aneurysm/*complications/*diagnostic imaging/surgery MH - Magnetic Resonance Angiography MH - Middle Aged MH - Radiography EDAT- 2010/06/01 06:00 MHDA- 2010/10/20 06:00 CRDT- 2010/06/01 06:00 PHST- 2009/09/19 00:00 [received] PHST- 2009/12/19 00:00 [accepted] PHST- 2010/06/01 06:00 [entrez] PHST- 2010/06/01 06:00 [pubmed] PHST- 2010/10/20 06:00 [medline] AID - S0967-5868(10)00095-0 [pii] AID - 10.1016/j.jocn.2009.12.008 [doi] PST - ppublish SO - J Clin Neurosci. 2010 Aug;17(8):1049-51. doi: 10.1016/j.jocn.2009.12.008. Epub 2010 May 26. PMID- 20346544 OWN - NLM STAT- MEDLINE DCOM- 20110303 LR - 20161021 IS - 0210-5691 (Print) IS - 0210-5691 (Linking) VI - 34 IP - 9 DP - 2010 Dec TI - [Cor pulmonale-PHT secondary to crest syndrome. Treatment with slow continuous ultrafiltration]. PG - 629-31 LID - 10.1016/j.medin.2009.12.006 [doi] AB - Pulmonary hypertension is a severe disease with complex treatment based on general measurements, anticoagulation and use of specific vasodilator drugs. Right heart failure initiated in final stages of the disease is treated with diuretics. We present the case of slow continuous ultrafilitration as treatment in right heart failure secondary to pulmonary hypertension associated to collagen disease refractory to diuretic treatment. CI - 2009 Elsevier España, S.L. y SEMICYUC. All rights reserved. FAU - Fernández Aguirre, C AU - Fernández Aguirre C AD - Servicio de Neumología, Unidad de Hipertensión Pulmonar, Hospital Universitario Virgen de la Victoria, Málaga, España. FAU - Daga Ruiz, D AU - Daga Ruiz D FAU - Velasco Garrido, J L AU - Velasco Garrido JL FAU - Cota Delgado, F AU - Cota Delgado F FAU - Hidalgo Sanjuán, M V AU - Hidalgo Sanjuán MV FAU - Salazar Ramírez, C AU - Salazar Ramírez C LA - spa PT - Case Reports PT - English Abstract PT - Journal Article TT - Cor pulmonale hipertensión pulmonar secundaria a síndrome crest. Tratamiento con ultrafiltración lenta continua. DEP - 20100325 PL - Spain TA - Med Intensiva JT - Medicina intensiva JID - 9207689 SB - IM MH - CREST Syndrome/*complications MH - Female MH - *Hemofiltration/methods MH - Humans MH - Hypertension, Pulmonary/etiology/*therapy MH - Middle Aged MH - Pulmonary Heart Disease/etiology/*therapy MH - Time Factors EDAT- 2010/03/30 06:00 MHDA- 2011/03/04 06:00 CRDT- 2010/03/30 06:00 PHST- 2009/06/15 00:00 [received] PHST- 2009/12/18 00:00 [accepted] PHST- 2010/03/30 06:00 [entrez] PHST- 2010/03/30 06:00 [pubmed] PHST- 2011/03/04 06:00 [medline] AID - S0210-5691(10)00022-7 [pii] AID - 10.1016/j.medin.2009.12.006 [doi] PST - ppublish SO - Med Intensiva. 2010 Dec;34(9):629-31. doi: 10.1016/j.medin.2009.12.006. Epub 2010 Mar 25. PMID- 20130953 OWN - NLM STAT- MEDLINE DCOM- 20100615 LR - 20240505 IS - 1129-2377 (Electronic) IS - 1129-2369 (Print) IS - 1129-2369 (Linking) VI - 11 IP - 2 DP - 2010 Apr TI - Sporadic hemiplegic migraine and CREST syndrome. PG - 171-3 LID - 10.1007/s10194-010-0188-1 [doi] AB - Hemiplegic migraines are characterised by attacks of migraine with aura accompanied by transient motor weakness. There are both familial and sporadic subtypes, which are now recognised as separate entities by the International Classification of Headache Disorders, edition II (ICHD-II). The sporadic subtype has been associated with other medical conditions, particularly rheumatological diseases. We report the case of a woman with sporadic hemiplegic migraine associated with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Since there is a close relationship between migraine and Raynaud's phenomenon, it could be speculated that the sporadic hemiplegic migraines in our patient might be secondary to CREST syndrome. FAU - Grecco, Martin Pablo AU - Grecco MP AD - Neurology, Hospital de Clínicas, Av. Córdoba 2351, Buenos Aires, Argentina. martingrecco@yahoo.com FAU - Pieroni, Miguel AU - Pieroni M FAU - Otero, Marcela AU - Otero M FAU - Ferreiro, Jorge Luis AU - Ferreiro JL FAU - Figuerola, María de Lourdes AU - Figuerola Mde L LA - eng PT - Case Reports PT - Journal Article DEP - 20100204 PL - England TA - J Headache Pain JT - The journal of headache and pain JID - 100940562 RN - 0 (Neuroprotective Agents) RN - 0H73WJJ391 (Topiramate) RN - 30237-26-4 (Fructose) SB - IM MH - Adult MH - Brain/pathology/physiopathology MH - CREST Syndrome/*complications/*physiopathology MH - Diffusion Magnetic Resonance Imaging MH - Female MH - Fructose/analogs & derivatives/therapeutic use MH - Humans MH - Ischemic Attack, Transient/etiology MH - Magnetic Resonance Imaging MH - Migraine with Aura/*etiology/*physiopathology MH - Neuroprotective Agents/therapeutic use MH - Paresis/etiology MH - Topiramate MH - Treatment Outcome PMC - PMC3452292 EDAT- 2010/02/05 06:00 MHDA- 2010/06/16 06:00 PMCR- 2010/04/01 CRDT- 2010/02/05 06:00 PHST- 2009/10/29 00:00 [received] PHST- 2010/01/06 00:00 [accepted] PHST- 2010/02/05 06:00 [entrez] PHST- 2010/02/05 06:00 [pubmed] PHST- 2010/06/16 06:00 [medline] PHST- 2010/04/01 00:00 [pmc-release] AID - 188 [pii] AID - 10.1007/s10194-010-0188-1 [doi] PST - ppublish SO - J Headache Pain. 2010 Apr;11(2):171-3. doi: 10.1007/s10194-010-0188-1. Epub 2010 Feb 4. PMID- 19786719 OWN - NLM STAT- MEDLINE DCOM- 20091029 LR - 20131121 IS - 1468-3296 (Electronic) IS - 0040-6376 (Linking) VI - 64 IP - 10 DP - 2009 Oct TI - Iloprost-induced rash. PG - 917-8 LID - 10.1136/thx.2009.116111 [doi] FAU - Finn, R S AU - Finn RS FAU - Beckert, L AU - Beckert L FAU - Troughton, R AU - Troughton R LA - eng PT - Case Reports PT - Letter PL - England TA - Thorax JT - Thorax JID - 0417353 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - CREST Syndrome/*drug therapy MH - Drug Eruptions/*etiology MH - Exanthema/*chemically induced MH - Female MH - Humans MH - Iloprost/*adverse effects MH - Middle Aged MH - Skin Diseases, Vascular/*chemically induced MH - Vasodilator Agents/*adverse effects EDAT- 2009/09/30 06:00 MHDA- 2009/10/30 06:00 CRDT- 2009/09/30 06:00 PHST- 2009/09/30 06:00 [entrez] PHST- 2009/09/30 06:00 [pubmed] PHST- 2009/10/30 06:00 [medline] AID - 64/10/917 [pii] AID - 10.1136/thx.2009.116111 [doi] PST - ppublish SO - Thorax. 2009 Oct;64(10):917-8. doi: 10.1136/thx.2009.116111. PMID- 19753757 OWN - NLM STAT- MEDLINE DCOM- 20100914 LR - 20161125 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 57 DP - 2009 Jan TI - Scleroderma esophagus. PG - 37 FAU - Santra, G AU - Santra G AD - Dept. of Medicine, North Bengal Medical College, Sushrutanagar, Darjeeling, PIN-734012. LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 RN - 0 (Proton Pump Inhibitors) SB - IM MH - Adult MH - CREST Syndrome/*complications/drug therapy MH - *Esophageal Sphincter, Lower MH - Esophageal Stenosis/*diagnosis/diagnostic imaging/etiology MH - Esophagitis, Peptic/*diagnosis/diagnostic imaging/etiology MH - Female MH - Humans MH - Proton Pump Inhibitors/therapeutic use MH - Radiography EDAT- 2009/09/17 06:00 MHDA- 2010/09/15 06:00 CRDT- 2009/09/17 06:00 PHST- 2009/09/17 06:00 [entrez] PHST- 2009/09/17 06:00 [pubmed] PHST- 2010/09/15 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2009 Jan;57:37. PMID- 19371916 OWN - NLM STAT- MEDLINE DCOM- 20090924 LR - 20090601 IS - 0025-7753 (Print) IS - 0025-7753 (Linking) VI - 132 IP - 20 DP - 2009 May 30 TI - [Calcinosis. Limited systemic sclerosis (CREST syndrome)]. PG - 805 LID - 10.1016/j.medcli.2008.12.012 [doi] FAU - Arias Núñez, M Carmen AU - Arias Núñez MC AD - Servicio de Medicina Interna, Complexo Hospitalario Xeral Calde, Lugo, España. mcarinun@hotmail.com FAU - González-Gay, Miguel Angel AU - González-Gay MA LA - spa PT - Case Reports PT - Journal Article TT - Calcinosis. Esclerosis sistémica limitada (síndrome CREST). DEP - 20090416 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM MH - *CREST Syndrome/diagnosis MH - Female MH - Humans MH - Middle Aged EDAT- 2009/04/18 09:00 MHDA- 2009/09/25 06:00 CRDT- 2009/04/18 09:00 PHST- 2008/11/12 00:00 [received] PHST- 2008/12/03 00:00 [accepted] PHST- 2009/04/18 09:00 [entrez] PHST- 2009/04/18 09:00 [pubmed] PHST- 2009/09/25 06:00 [medline] AID - S0025-7753(09)00390-X [pii] AID - 10.1016/j.medcli.2008.12.012 [doi] PST - ppublish SO - Med Clin (Barc). 2009 May 30;132(20):805. doi: 10.1016/j.medcli.2008.12.012. Epub 2009 Apr 16. PMID- 19289776 OWN - NLM STAT- MEDLINE DCOM- 20090410 LR - 20220419 IS - 1538-3652 (Electronic) IS - 0003-987X (Linking) VI - 145 IP - 3 DP - 2009 Mar TI - Intravenous immunoglobulin therapy for dystrophic calcinosis cutis: unreliable in our hands. PG - 334; author reply 335 LID - 10.1001/archdermatol.2008.620 [doi] FAU - Kalajian, Andrew H AU - Kalajian AH FAU - Perryman, Jennifer H AU - Perryman JH FAU - Callen, Jeffrey P AU - Callen JP LA - eng PT - Comment PT - Letter PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 RN - 0 (Immunoglobulins, Intravenous) SB - IM CON - Arch Dermatol. 2008 May;144(5):585-7. doi: 10.1001/archderm.144.5.585. PMID: 18490584 MH - CREST Syndrome/complications MH - Calcinosis/*drug therapy/pathology MH - Dermatomyositis/complications MH - Humans MH - Immunoglobulins, Intravenous/*therapeutic use MH - Skin Diseases/*drug therapy/pathology EDAT- 2009/03/18 09:00 MHDA- 2009/04/11 09:00 CRDT- 2009/03/18 09:00 PHST- 2009/03/18 09:00 [entrez] PHST- 2009/03/18 09:00 [pubmed] PHST- 2009/04/11 09:00 [medline] AID - 145/3/334 [pii] AID - 10.1001/archdermatol.2008.620 [doi] PST - ppublish SO - Arch Dermatol. 2009 Mar;145(3):334; author reply 335. doi: 10.1001/archdermatol.2008.620. PMID- 18548216 OWN - NLM STAT- MEDLINE DCOM- 20081007 LR - 20080612 IS - 0723-5003 (Print) IS - 0723-5003 (Linking) VI - 103 IP - 6 DP - 2008 Jun 15 TI - [Bedside visualization of altered microflow in autoimmune hemolysis by sidestream dark-field technology]. PG - 447-9 LID - 10.1007/s00063-008-1065-4 [doi] FAU - Jung, Christian AU - Jung C AD - Klinik für Innere Medizin I, Universitätsklinik der Friedrich-Schiller-Universität Jena, Erlanger Allee 101, Jena. christian.jung@med.uni-jena.de FAU - Ferrari, Markus AU - Ferrari M FAU - Rödiger, Christoph AU - Rödiger C FAU - Bahrmann, Philipp AU - Bahrmann P FAU - Goebel, Bjoern AU - Goebel B FAU - Lauten, Alexander AU - Lauten A FAU - Hutschenreuther, Jan AU - Hutschenreuther J FAU - Fritzenwanger, Michael AU - Fritzenwanger M FAU - Pfeifer, Rüdiger AU - Pfeifer R FAU - Figulla, Hans-Reiner AU - Figulla HR LA - ger PT - Case Reports PT - Journal Article TT - Bedside-Visualisierung der Alteration des Mikroflusses bei Autoimmunhämolyse mittels Sidestream-dark-Field-Technologie. PL - Germany TA - Med Klin (Munich) JT - Medizinische Klinik (Munich, Germany : 1983) JID - 8303501 SB - IM MH - Anemia, Hemolytic, Autoimmune/*diagnosis/therapy MH - Blood Flow Velocity/physiology MH - CREST Syndrome/*diagnosis/therapy MH - Dermatomyositis/*diagnosis/therapy MH - Extracorporeal Membrane Oxygenation MH - Fatal Outcome MH - Female MH - Humans MH - Microcirculation/*physiology MH - *Microscopic Angioscopy MH - *Microscopy, Polarization MH - *Microscopy, Video MH - Middle Aged MH - Mouth Mucosa/*blood supply MH - *Online Systems MH - Plasmapheresis MH - *Point-of-Care Systems MH - Polymyositis/*diagnosis/therapy MH - Pulmonary Fibrosis/diagnosis/therapy EDAT- 2008/06/13 09:00 MHDA- 2008/10/08 09:00 CRDT- 2008/06/13 09:00 PHST- 2008/06/13 09:00 [pubmed] PHST- 2008/10/08 09:00 [medline] PHST- 2008/06/13 09:00 [entrez] AID - 10.1007/s00063-008-1065-4 [doi] PST - ppublish SO - Med Klin (Munich). 2008 Jun 15;103(6):447-9. doi: 10.1007/s00063-008-1065-4. PMID- 18490584 OWN - NLM STAT- MEDLINE DCOM- 20080624 LR - 20090410 IS - 1538-3652 (Electronic) IS - 0003-987X (Linking) VI - 144 IP - 5 DP - 2008 May TI - Response of dystrophic calcification to intravenous immunoglobulin. PG - 585-7 LID - 10.1001/archderm.144.5.585 [doi] FAU - Schanz, Stefan AU - Schanz S AD - University of Tuebingen, Liebermeisterstrasse 25, Tuebingen 72076, Germany. Stefan.schanz@med.uni-tuebingen.de FAU - Ulmer, Anja AU - Ulmer A FAU - Fierlbeck, Gerhard AU - Fierlbeck G LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 RN - 0 (Immunoglobulins, Intravenous) SB - IM CIN - Arch Dermatol. 2009 Mar;145(3):334; author reply 335. doi: 10.1001/archdermatol.2008.620. PMID: 19289776 MH - CREST Syndrome/complications MH - Calcinosis/complications/*drug therapy/pathology MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Edema/etiology/pathology MH - Female MH - Humans MH - *Immunoglobulins, Intravenous/administration & dosage MH - Inflammation/etiology/pathology MH - Middle Aged MH - Treatment Outcome EDAT- 2008/05/21 09:00 MHDA- 2008/06/25 09:00 CRDT- 2008/05/21 09:00 PHST- 2008/05/21 09:00 [pubmed] PHST- 2008/06/25 09:00 [medline] PHST- 2008/05/21 09:00 [entrez] AID - 144/5/585 [pii] AID - 10.1001/archderm.144.5.585 [doi] PST - ppublish SO - Arch Dermatol. 2008 May;144(5):585-7. doi: 10.1001/archderm.144.5.585. PMID- 17572041 OWN - NLM STAT- MEDLINE DCOM- 20071003 LR - 20161124 IS - 1769-6623 (Electronic) IS - 0750-7658 (Linking) VI - 26 IP - 7-8 DP - 2007 Jul-Aug TI - [Splenic rupture associated with CMV infection: case report and review]. PG - 674-6 AB - Splenic ruptures secondary to infection are rare, life threatening and difficult to diagnose. The main management is surgery, however non-operative management in the stable patient is possible. We report the case of a 60-yr-woman with spontaneous splenic rupture during CMV primary infection. Non-operative treatment failed and splenectomy was done. FAU - Amathieu, R AU - Amathieu R AD - Service de réanimation polyvalente, CHU Jean-Verdier, APHP, Bondy, France. roland.amathieu@jvr.aphp.fr FAU - Tual, L AU - Tual L FAU - Rouaghe, S AU - Rouaghe S FAU - Stirnemann, J AU - Stirnemann J FAU - Fain, O AU - Fain O FAU - Dhonneur, G AU - Dhonneur G LA - fre PT - Case Reports PT - Journal Article PT - Review TT - Rupture spontanée de la rate au cours d'une infection à cytomégalovirus: cas clinique et revue de la littérature. DEP - 20070614 PL - France TA - Ann Fr Anesth Reanim JT - Annales francaises d'anesthesie et de reanimation JID - 8213275 SB - IM MH - CREST Syndrome/complications MH - Cytomegalovirus Infections/*complications MH - Female MH - Humans MH - Hypotension/etiology MH - Infectious Mononucleosis/*complications/virology MH - Middle Aged MH - Rupture, Spontaneous MH - Splenectomy MH - Splenic Rupture/diagnostic imaging/*etiology/surgery MH - Ultrasonography RF - 12 EDAT- 2007/06/19 09:00 MHDA- 2007/10/04 09:00 CRDT- 2007/06/19 09:00 PHST- 2006/11/19 00:00 [received] PHST- 2007/03/01 00:00 [accepted] PHST- 2007/06/19 09:00 [pubmed] PHST- 2007/10/04 09:00 [medline] PHST- 2007/06/19 09:00 [entrez] AID - S0750-7658(07)00189-X [pii] AID - 10.1016/j.annfar.2007.03.037 [doi] PST - ppublish SO - Ann Fr Anesth Reanim. 2007 Jul-Aug;26(7-8):674-6. doi: 10.1016/j.annfar.2007.03.037. Epub 2007 Jun 14. PMID- 17425211 OWN - NLM STAT- MEDLINE DCOM- 20070507 LR - 20260128 IS - 0001-5547 (Print) IS - 0001-5547 (Linking) VI - 51 IP - 2 DP - 2007 Mar-Apr TI - Myxoinflammatory fibroblastic sarcoma: report of a case with fine needle aspiration cytology. PG - 231-4 AB - BACKGROUND: Myxoinflammatory fibroblastic sarcoma (MFS) is a distinct neoplasm that usually arises in the acral zones of distalextremities. We report, for the first time, the preoperative fine needle a,spiration cytology (FNAC) findings of an MFS case that was confirmed after surgical excision. CASE: An 81-year-old woman presented with a multinodular tumor in the distal right extremity that had been present for 1 year. FNA C of the lesion was performed and followed by local excision. The fine needle aspiration smears contained 2 of the 3 types of neoplastic cells that have been observed in MFS: spindled and ganglionlike cells. The background was myxoid, with a prominent inflammatory infiltrate. Histopathologic examination of the surgical specimen confirmed the diagnosis of MFS. CONCLUSION: Although the cytologic diagnosis was "pleomorphic sarcoma," MFS was considered and local excision recommended, given the reported low grade nature of this entity. However, the need for extreme caution in the diagnosis of soft tissue lesions on cytologic grounds alone cannot be overemphasized. FAU - García-García, Elena AU - García-García E AD - Department of Pathology, 12 de Octubre University Hospital, Madrid, Spain. FAU - Rodríguez-Gil, Yolanda AU - Rodríguez-Gil Y FAU - Suárez-Gauthier, Ana AU - Suárez-Gauthier A FAU - Martínez-Tello, Francisco José AU - Martínez-Tello FJ FAU - López-Ríos, Fernando AU - López-Ríos F FAU - Ballestín, Claudio AU - Ballestín C LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Switzerland TA - Acta Cytol JT - Acta cytologica JID - 0370307 RN - 0 (Antigens, CD) RN - 0 (Antigens, Differentiation, Myelomonocytic) RN - 0 (Biomarkers, Tumor) RN - 0 (Vimentin) RN - 0 (CD68 antigen, human) RN - 0 (CD68 Molecule) SB - IM MH - Aged, 80 and over MH - Antigens, CD/analysis/metabolism MH - Antigens, Differentiation, Myelomonocytic/analysis/metabolism MH - Biomarkers, Tumor/analysis/metabolism MH - Biopsy, Fine-Needle MH - CREST Syndrome/complications MH - Cell Nucleus/pathology MH - Cytoplasm/pathology MH - Diagnosis, Differential MH - Female MH - Fibroblasts/*pathology MH - Humans MH - Leg/*pathology/physiopathology/surgery MH - Myxosarcoma/*pathology/physiopathology/surgery MH - Neoplasm Invasiveness/physiopathology MH - Neoplasm Metastasis/physiopathology MH - Orthopedic Procedures MH - Predictive Value of Tests MH - Sarcoma/*pathology/physiopathology/surgery MH - Treatment Outcome MH - Vimentin/analysis/metabolism MH - CD68 Molecule EDAT- 2007/04/12 09:00 MHDA- 2007/05/08 09:00 CRDT- 2007/04/12 09:00 PHST- 2007/04/12 09:00 [pubmed] PHST- 2007/05/08 09:00 [medline] PHST- 2007/04/12 09:00 [entrez] AID - 10.1159/000325724 [doi] PST - ppublish SO - Acta Cytol. 2007 Mar-Apr;51(2):231-4. doi: 10.1159/000325724. PMID- 17309890 OWN - NLM STAT- MEDLINE DCOM- 20070703 LR - 20161124 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 5 DP - 2007 May TI - Local implantation of autologous mononuclear cells from bone marrow and peripheral blood for treatment of ischaemic digits in patients with connective tissue diseases. PG - 882-4 AB - OBJECTIVE: CD34-positive bone marrow mononuclear cells (MNCs) have been successfully used for regeneration of small arteries in Buerger's disease. The objective of this study is to examine the angiogenetic potential of autologous MNCs from bone marrow and peripheral blood implanted into the ischaemic digits from patients with connective tissue diseases. METHODS: Three patients with systemic sclerosis, two with mixed connective tissue disease, and one with CREST syndrome were enrolled who had painful ischaemic digits with necrosis refractory to several vasodilators including intravenous prostaglandins. MNCs obtained from 7 ml/kg bone marrow blood and 400 ml peripheral blood were implanted into 20 different sites in palms and/or soles. The study was performed open-labelled. RESULTS: Pain in the numeric rating scale improved remarkably up to 1 month after implantation of bone marrow or peripheral MNCs to the same extent, although no significant differences were found in transcutaneous oxygen pressure and thermogram before and after the implantation. Bone marrow MNCs increased blood flow of the hand determined by intra-arterial digital subtraction angiography, while peripheral MNCs did not. CONCLUSIONS: Implantation of autologous MNCs from peripheral and bone marrow into the ischaemic digits was so effective in pain-relief and more clinical trials would be warranted to see whether this could be a new treatment modality for angiogenesis in connective tissue diseases as in Buerger's disease. FAU - Kamata, Y AU - Kamata Y AD - Division of Rheumatology and Clinical Immunology, Jichi Medical University, Shimotsuke-shi, Tochigi-ken, Japan. y.kamata@jichi.ac.jp FAU - Takahashi, Y AU - Takahashi Y FAU - Iwamoto, M AU - Iwamoto M FAU - Matsui, K AU - Matsui K FAU - Murakami, Y AU - Murakami Y FAU - Muroi, K AU - Muroi K FAU - Ikeda, U AU - Ikeda U FAU - Shimada, K AU - Shimada K FAU - Yoshio, T AU - Yoshio T FAU - Okazaki, H AU - Okazaki H FAU - Minota, S AU - Minota S LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070219 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antigens, CD34) SB - IM MH - Aged MH - Angiography, Digital Subtraction MH - Antigens, CD34/analysis MH - *Bone Marrow Transplantation MH - CREST Syndrome/therapy MH - Connective Tissue Diseases/complications/*therapy MH - Female MH - Fingers/blood supply MH - Humans MH - Ischemia/diagnostic imaging/etiology/*therapy MH - Male MH - Middle Aged MH - Neovascularization, Physiologic MH - *Peripheral Blood Stem Cell Transplantation MH - Toes/blood supply MH - Treatment Outcome EDAT- 2007/02/21 09:00 MHDA- 2007/07/04 09:00 CRDT- 2007/02/21 09:00 PHST- 2007/02/21 09:00 [pubmed] PHST- 2007/07/04 09:00 [medline] PHST- 2007/02/21 09:00 [entrez] AID - kel436 [pii] AID - 10.1093/rheumatology/kel436 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 May;46(5):882-4. doi: 10.1093/rheumatology/kel436. Epub 2007 Feb 19. PMID- 16882593 OWN - NLM STAT- MEDLINE DCOM- 20061027 LR - 20061115 IS - 0300-9742 (Print) IS - 0300-9742 (Linking) VI - 35 IP - 4 DP - 2006 Jul-Aug TI - Anti-centromere antibodies in patients with systemic lupus erythematosus. PG - 290-4 AB - BACKGROUND: Anti-centromere autoantibodies (ACA) are frequently detected in systemic sclerosis (SScl), especially in the calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, in which a prevalence of 55% has been reported. The presence of ACA in systemic lupus erythematosus (SLE) is so rare that its detection can raise serious doubts about the validity of the diagnosis. OBJECTIVE: To determine the frequency of ACA positive subjects from a wide monocentric cohort of SLE patients and analyse the clinical and biological characteristics of this group. METHODS: Five hundred and sixty consecutive SLE patients were systematically analysed for the presence of ACA and other autoantibodies using indirect immunofluorescence, counter-immunoelectrophoresis, double immunodiffusion, enzyme-linked immunosorbent assay (ELISA), and Western-blot. RESULTS: ACA were detected in 11 SLE patients (1.9%); all of them were women. The CENP-B-specific ELISA was positive in all patients. The main clinical features of scleroderma (cutaneous sclerosis, sclerodactylia, digital ulcers, or pulmonary fibrosis) were not present in these patients, who did not differ clinically from the whole SLE group. CONCLUSIONS: ACA can be detected in patients with genuine SLE without concurrent scleroderma. Therefore, the presence of this antibody does not preclude the possibility of the diagnosis of SLE. In addition, SLE patients with ACA do not represent a different clinical subgroup. FAU - Respaldiza, N AU - Respaldiza N AD - Department of Immunology, Hospitales Universitarios Virgen del Rocio, Avenida Manuel Siurot s/n, 41013 Seville, Spain. FAU - Wichmann, I AU - Wichmann I FAU - Ocaña, C AU - Ocaña C FAU - Garcia-Hernandez, F J AU - Garcia-Hernandez FJ FAU - Castillo, M J AU - Castillo MJ FAU - Magariño, M I AU - Magariño MI FAU - Magariño, R AU - Magariño R FAU - Torres, A AU - Torres A FAU - Sanchez-Roman, J AU - Sanchez-Roman J FAU - Nuñez-Roldan, A AU - Nuñez-Roldan A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood MH - CREST Syndrome/diagnosis/immunology MH - Centromere/*immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/*immunology/physiopathology MH - Male MH - Middle Aged EDAT- 2006/08/03 09:00 MHDA- 2006/10/28 09:00 CRDT- 2006/08/03 09:00 PHST- 2006/08/03 09:00 [pubmed] PHST- 2006/10/28 09:00 [medline] PHST- 2006/08/03 09:00 [entrez] AID - U357330Q07232442 [pii] AID - 10.1080/03009740600588376 [doi] PST - ppublish SO - Scand J Rheumatol. 2006 Jul-Aug;35(4):290-4. doi: 10.1080/03009740600588376. PMID- 16871407 OWN - NLM STAT- MEDLINE DCOM- 20071023 LR - 20181113 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 27 IP - 2 DP - 2006 Dec TI - Scleroderma and chronic myeloid leukemia: a sheer coincidence, a consequence of long lasting D-penicillamine therapy or a plausible relationship of both diseases? PG - 175-7 AB - Systemic sclerosis is a chronic multisystem disorder of unknown etiology characterized by the involvement of skin and visceral organs caused by an accumulation of collagen. It has been reported that the incidence of solid and hematological malignancy increased in systemic sclerosis. Multiple myeloma and chronic lymphocytic leukemia are the most common hematological malignancies seen in patients with systemic sclerosis. Chronic myeloid leukemia (CML) has only rarely been reported so far. We here report a case with CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactly, telangiectasia) who developed CML 7 years after the onset of CREST. Ours is the second case with CML developing after the onset of CREST in the literature. We also briefly discuss the possible tendency to hematological malignancy in systemic sclerosis. FAU - Kaşifoğlu, Timuçin AU - Kaşifoğlu T AD - Division of Rheumatology, Department of Internal Medicine, Eskişehir Osmangazi University Medical Faculty, Vişnelik Mah. Alifuat Güven C. Akasya Sok. 11/11, 26020 Eskişehir, Turkey. FAU - Korkmaz, Cengiz AU - Korkmaz C FAU - Yaşar, Sule AU - Yaşar S FAU - Gülbaş, Zafer AU - Gülbaş Z LA - eng PT - Case Reports PT - Journal Article DEP - 20060727 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antirheumatic Agents) RN - GNN1DV99GX (Penicillamine) SB - IM MH - Antirheumatic Agents/*adverse effects MH - CREST Syndrome/*complications/drug therapy MH - Female MH - Humans MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/chemically induced/*complications MH - Middle Aged MH - Penicillamine/*adverse effects EDAT- 2006/07/28 09:00 MHDA- 2007/10/24 09:00 CRDT- 2006/07/28 09:00 PHST- 2005/09/27 00:00 [received] PHST- 2006/07/01 00:00 [accepted] PHST- 2006/07/28 09:00 [pubmed] PHST- 2007/10/24 09:00 [medline] PHST- 2006/07/28 09:00 [entrez] AID - 10.1007/s00296-006-0167-7 [doi] PST - ppublish SO - Rheumatol Int. 2006 Dec;27(2):175-7. doi: 10.1007/s00296-006-0167-7. Epub 2006 Jul 27. PMID- 16804698 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20181113 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 4 DP - 2006 Jul TI - [Pulmonary arterial hypertension in collagenoses: clinical features, epidemiology, pathogenesis, diagnosis and treatment]. PG - 297-300, 302-5 AB - Pulmonary arterial hypertension (PAH) is a severe vasculopathy, which is characterised by progressive narrowing and obliteration of the pulmonary arterioles and increased endothelin-1 levels. The increase of vascular resistance in the lung vessels leads to chronic pressure overload and to right heart failure, if untreated. PAH often occurs in association with rheumatic-inflammatory diseases (e.g., in 15% of patients with systemic sclerosis (SSc), especially in the limited form or in CREST patients) and determines their prognosis: in advanced stages, untreated patients die within a short period. Therefore all SSc patients, particularly the newly diagnosed ones, should be screened for PAH with echocardiography. If PAH is suspected, a right heart catheter should be performed, and if PAH is confirmed, adequate treatment should be initiated. While few years ago lung transplantation was the only option for patients with severe PAH, in recent years enormous progress was seen in drug treatment. Today prostanoids (Ventavis) and the endothelin receptor antagonist bosentan (Tracleer) are available for patients with PAH in WHO/NYHA stage III: they have substantially improved the prognosis of PAH in the last years. Since few months, also the phosphodiesterase inhibitor sildenafil (Revatio) is available. The combination of drugs with different mode of action will likely further improve the prognosis of PAH patients. FAU - Ahmadi-Simab, K AU - Ahmadi-Simab K AD - Poliklinik für Rheumatologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck und Rheumaklinik Bad Bramstedt, Oskar-Alexander-Strasse 26, 24576, Bad Bramstedt. ahmadi@r-on-klinik.de FAU - Gross, W L AU - Gross WL LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Pulmonale arterielle Hypertonie bei Kollagenosen: Klinik, Epidemiologie, Pathogenese, Diagnostik und Therapie. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Vasodilator Agents) SB - IM MH - Algorithms MH - CREST Syndrome/diagnosis/epidemiology/physiopathology/therapy MH - Cross-Sectional Studies MH - Echocardiography MH - Endothelium, Vascular MH - Evidence-Based Medicine MH - Humans MH - Hypertension, Pulmonary/diagnosis/*epidemiology/physiopathology/therapy MH - Lupus Erythematosus, Systemic/diagnosis/*epidemiology/physiopathology/therapy MH - Mixed Connective Tissue Disease/diagnosis/*epidemiology/physiopathology/therapy MH - Prognosis MH - Scleroderma, Systemic/diagnosis/*epidemiology/physiopathology/therapy MH - Vasoconstriction/physiology MH - Vasodilator Agents/therapeutic use RF - 61 EDAT- 2006/06/29 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/06/29 09:00 PHST- 2006/06/29 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/06/29 09:00 [entrez] AID - 10.1007/s00393-006-0069-9 [doi] PST - ppublish SO - Z Rheumatol. 2006 Jul;65(4):297-300, 302-5. doi: 10.1007/s00393-006-0069-9. PMID- 16489463 OWN - NLM STAT- MEDLINE DCOM- 20061215 LR - 20181113 IS - 1432-9417 (Print) IS - 1432-9417 (Linking) VI - 10 IP - 2 DP - 2006 Mar TI - Solitary extramedullary plasmacytoma and granulomatous sialadenitis of the parotid gland preceding a B-cell non-Hodgkin's lymphoma. PG - 122-5 AB - A patient with swelling of the left parotid gland of four-months' duration, sicca syndrome (xerophthalmia and xerostomia) and a history of progressive systemic sclerosis with an incomplete form of the CREST syndrome was referred to our department. On ultrasound a parotid mass of reduced echogenicity without any enlarged cervical lymph nodes was found. Ultrasonographically guided fine-needle biopsy could not provide any definitive diagnosis. After partial parotidectomy with complete tumor removal the histologic exam showed an extramedullary plasmacytoma with concurrent non-necrotizing granulomatous sialadenitis of the parotid gland. Complete systemic work-up excluded multiple myeloma, leukemia, lymphoma and sarcoidosis. Post-operative radiotherapy of the left parotid region and left neck including the supraclavicular lymph node area was performed. Six months after surgery an aggressive B-cell non-Hodgkin's lymphoma was diagnosed. FAU - Gouveris, H AU - Gouveris H AD - Universitäts-HNO-Klinik, Johannes-Gutenberg-Universität Mainz, Mainz. FAU - Hansen, T AU - Hansen T FAU - Franke, K AU - Franke K LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Mund Kiefer Gesichtschir JT - Mund-, Kiefer- und Gesichtschirurgie : MKG JID - 9716576 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 4F4X42SYQ6 (Rituximab) RN - 5J49Q6B70F (Vincristine) RN - 80168379AG (Doxorubicin) RN - 8N3DW7272P (Cyclophosphamide) RN - VB0R961HZT (Prednisone) RN - CHOP protocol SB - IM MH - Aged MH - Antibodies, Monoclonal/administration & dosage MH - Antibodies, Monoclonal, Murine-Derived MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/therapeutic use MH - Biopsy, Needle MH - Bone Marrow/pathology MH - CREST Syndrome/diagnosis MH - Cyclophosphamide/administration & dosage MH - Doxorubicin/administration & dosage MH - Follow-Up Studies MH - Granuloma/*diagnosis/pathology/radiotherapy/surgery MH - Humans MH - Lymphoma, B-Cell/*diagnosis/pathology MH - Male MH - Neoplasms, Radiation-Induced/*diagnosis/drug therapy/pathology MH - Neoplasms, Second Primary/*diagnosis/drug therapy/pathology MH - Parotid Gland/pathology/radiation effects/surgery MH - Parotid Neoplasms/*diagnosis/pathology/radiotherapy/surgery MH - Parotitis/*diagnosis/pathology/radiotherapy/surgery MH - Plasmacytoma/*diagnosis/pathology/radiotherapy/surgery MH - Postoperative Complications/diagnosis/pathology MH - Prednisone/administration & dosage MH - Rituximab MH - Vincristine/administration & dosage EDAT- 2006/02/21 09:00 MHDA- 2006/12/16 09:00 CRDT- 2006/02/21 09:00 PHST- 2006/02/21 09:00 [pubmed] PHST- 2006/12/16 09:00 [medline] PHST- 2006/02/21 09:00 [entrez] AID - 10.1007/s10006-006-0673-5 [doi] PST - ppublish SO - Mund Kiefer Gesichtschir. 2006 Mar;10(2):122-5. doi: 10.1007/s10006-006-0673-5. PMID- 16445773 OWN - NLM STAT- MEDLINE DCOM- 20060721 LR - 20060131 IS - 0007-0963 (Print) IS - 0007-0963 (Linking) VI - 154 IP - 3 DP - 2006 Mar TI - The presence of dominant T-cell clones in peripheral blood of patients with collagen vascular disorders: a prospective study of 97 cases. PG - 445-9 AB - BACKGROUND: T-lymphocyte dysfunction has been seldom investigated in collagen vascular disorders. The search for dominant T-cell clones has been scarcely reported, although the presence of such clones might be expected in disorders showing immune responses directed against a variety of autoantigens. OBJECTIVES: We conducted a systematic search for dominant T-cell clones in peripheral blood in patients with collagen vascular disorders. Patients and methods Ninety-seven patients with collagen vascular disorders were studied (7 cutaneous and 38 systemic lupus erythematosus; 8 multiple morphea; 12 regional scleroderma; 32 systemic sclerosis of the CREST type). A dominant T-cell clone was searched for in peripheral blood by polymerase chain reaction targeting the T-cell receptor gamma chain followed by a size analysis of amplified fragments. Peripheral blood from patients with nonlymphocyte-dependent disorders and matched by age and sex was assessed in the same conditions. Results in both groups were compared using nonparametric statistical tests. RESULTS: Overall, a circulating dominant T-cell clone was found in 52% of patients compared with 16.9% in controls. More precisely, such a dominant clone was present in 43% and 37% of cutaneous and systemic lupus erythematosus, respectively, in 75% of multiple morphea, 75% of regional scleroderma and 60% of CREST syndrome patients. The percentages in all subsets of patients were significantly higher than in the control group. CONCLUSIONS: The presence of a dominant T-cell clone in peripheral blood is significantly more frequent in collagen vascular disorders than in controls, especially in patients with scleroderma, whatever the clinical subset, which suggests T-cell involvement in the immune response dysfunction in these diseases classically characterized by disturbances of B lymphocytes. The relevance of such a dominant clone regarding diagnosis, pathomechanisms, long-term outcome and visceral prognosis of these diseases as well as therapeutic decisions remains to be evaluated. FAU - Dereure, O AU - Dereure O AD - Department of Dermatology, University Hospital of Montpellier, France. o-dereure@chu-montpellier.fr FAU - Gubler, B AU - Gubler B FAU - Bessis, D AU - Bessis D FAU - Guillot, B AU - Guillot B FAU - Guilhou, J-J AU - Guilhou JJ FAU - Clot, J AU - Clot J FAU - Eliaou, J-F AU - Eliaou JF LA - eng PT - Journal Article PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Receptors, Antigen, T-Cell, gamma-delta) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoimmune Diseases/*immunology MH - CREST Syndrome/immunology MH - Child MH - Child, Preschool MH - Clone Cells/immunology MH - Connective Tissue Diseases/*immunology MH - Female MH - Humans MH - Lupus Erythematosus, Cutaneous/immunology MH - Lupus Erythematosus, Systemic/immunology MH - Male MH - Middle Aged MH - Prospective Studies MH - Receptors, Antigen, T-Cell, gamma-delta/genetics MH - Scleroderma, Localized/immunology MH - Scleroderma, Systemic/immunology MH - T-Lymphocytes/*immunology EDAT- 2006/02/01 09:00 MHDA- 2006/07/22 09:00 CRDT- 2006/02/01 09:00 PHST- 2006/02/01 09:00 [pubmed] PHST- 2006/07/22 09:00 [medline] PHST- 2006/02/01 09:00 [entrez] AID - BJD7044 [pii] AID - 10.1111/j.1365-2133.2005.07044.x [doi] PST - ppublish SO - Br J Dermatol. 2006 Mar;154(3):445-9. doi: 10.1111/j.1365-2133.2005.07044.x. PMID- 38898064 OWN - NLM STAT- MEDLINE DCOM- 20240619 LR - 20240711 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 14 IP - 1 DP - 2024 Jun 19 TI - Long-term outcomes in patients with primary biliary cholangitis complicated with CREST syndrome. PG - 14124 LID - 10.1038/s41598-024-64976-8 [doi] LID - 14124 AB - Primary biliary cholangitis (PBC) is frequently associated with autoimmune disease. Although PBC complicated with CREST syndrome (PBC-CREST) has been reported, the long-term outcomes of the affected patients have not been fully investigated. Herein, the long-term outcomes of PBC-CREST were evaluated. Next, the GLOBE and UK-PBC scores were validated and compared between the PBC alone and PBC-CREST groups. A total of 302 patients who were diagnosed with PBC between December 1990 and August 2021 at Fukushima Medical University Hospital were included. The liver transplantation (LT)-free survival rates were compared between patients with PBC alone (n = 245) and those with PBC-CREST (n = 57). Moreover, 173 patients, excluding those with liver-related death/LT within 1 year after ursodeoxycholic acid administration, were divided into two subgroups (PBC alone (n = 147) and PBC-CREST (n = 26)), and the GLOBE and UK-PBC scores were compared between the subgroups. The survival rates without LT (3/5/10 years) were 92/87/80% for the PBC-alone group and 98/96/96% for the PBC-CREST group, with a significantly better prognosis in the PBC-CREST group (log-rank P = 0.0172). Multivariate analysis revealed that the presence of CREST syndrome is an independent protective factor for the presence of cirrhosis. The predicted 5/10/15-year risks of liver-related death or LT based on the UK-PBC score were significantly lower in the PBC-CREST group (2.4/7.6/13.2%) than in the PBC-alone group (4.8/11.8/18.8%) (P < 0.05). The predicted 3/5-year LT-free survival rates based on the GLOBE score were significantly higher in the PBC-CREST group (93/88%) than in the PBC-alone group (88/81%) (P < 0.05). Patients with PBC-CREST may have better long-term outcomes than those with PBC alone. CI - © 2024. The Author(s). FAU - Abe, Kazumichi AU - Abe K AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. k-abe@fmu.ac.jp. FAU - Hayashi, Manabu AU - Hayashi M AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Sugaya, Tatsuro AU - Sugaya T AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Abe, Naoto AU - Abe N AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Takahata, Yosuke AU - Takahata Y AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Fujita, Masashi AU - Fujita M AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Takahashi, Atsushi AU - Takahashi A AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. FAU - Migita, Kiyoshi AU - Migita K AD - Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Ohira, Hiromasa AU - Ohira H AD - Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan. LA - eng PT - Journal Article DEP - 20240619 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Humans MH - Female MH - Male MH - *Liver Cirrhosis, Biliary/complications/mortality MH - Middle Aged MH - Aged MH - *Liver Transplantation MH - *CREST Syndrome/complications MH - Prognosis MH - Adult MH - Survival Rate MH - Retrospective Studies PMC - PMC11187228 OTO - NOTNLM OT - CREST syndrome OT - GLOBE score OT - Primary biliary cholangitis OT - Prognosis OT - UK-PBC risk score COIS- The authors declare no competing interests. EDAT- 2024/06/20 00:42 MHDA- 2024/06/20 00:43 PMCR- 2024/06/19 CRDT- 2024/06/19 23:17 PHST- 2023/11/07 00:00 [received] PHST- 2024/06/14 00:00 [accepted] PHST- 2024/06/20 00:43 [medline] PHST- 2024/06/20 00:42 [pubmed] PHST- 2024/06/19 23:17 [entrez] PHST- 2024/06/19 00:00 [pmc-release] AID - 10.1038/s41598-024-64976-8 [pii] AID - 64976 [pii] AID - 10.1038/s41598-024-64976-8 [doi] PST - epublish SO - Sci Rep. 2024 Jun 19;14(1):14124. doi: 10.1038/s41598-024-64976-8. PMID- 38054306 OWN - NLM STAT- MEDLINE DCOM- 20240320 LR - 20241112 IS - 1445-2197 (Electronic) IS - 1445-1433 (Linking) VI - 94 IP - 3 DP - 2024 Mar TI - The effect of CREST syndrome in planning a DIEP flap reconstruction. PG - 474-475 LID - 10.1111/ans.18773 [doi] FAU - Cheung, Michael Y AU - Cheung MY AUID- ORCID: 0000-0002-3217-4561 AD - Department of Burns, Reconstructive and Plastic Surgery, Royal North Shore Hospital, Sydney, New South Wales, Australia. FAU - Aggarwala, Shivani AU - Aggarwala S AUID- ORCID: 0000-0002-3978-1794 AD - Department of Burns, Reconstructive and Plastic Surgery, Royal North Shore Hospital, Sydney, New South Wales, Australia. FAU - French, James AU - French J AD - Westmead Breast Cancer Institute, Westmead Hospital, Sydney, New South Wales, Australia. FAU - Hsieh, Frank AU - Hsieh F AD - Department of Plastic and Reconstructive Surgery, Westmead Hospital, Sydney, New South Wales, Australia. AD - Department of Plastic & Reconstructive Surgery, Bankstown-Lidcombe Hospital, Sydney, New South Wales, Australia. FAU - Soliman, Bishoy AU - Soliman B AD - Department of Burns, Reconstructive and Plastic Surgery, Royal North Shore Hospital, Sydney, New South Wales, Australia. AD - Department of Plastic and Reconstructive Surgery, Westmead Hospital, Sydney, New South Wales, Australia. LA - eng PT - Case Reports PT - Journal Article DEP - 20231206 PL - Australia TA - ANZ J Surg JT - ANZ journal of surgery JID - 101086634 SB - IM MH - Humans MH - *CREST Syndrome MH - *Mammaplasty MH - *Perforator Flap MH - Retrospective Studies EDAT- 2023/12/06 06:41 MHDA- 2024/03/20 06:45 CRDT- 2023/12/06 05:27 PHST- 2023/10/30 00:00 [revised] PHST- 2023/08/14 00:00 [received] PHST- 2023/10/31 00:00 [accepted] PHST- 2024/03/20 06:45 [medline] PHST- 2023/12/06 06:41 [pubmed] PHST- 2023/12/06 05:27 [entrez] AID - 10.1111/ans.18773 [doi] PST - ppublish SO - ANZ J Surg. 2024 Mar;94(3):474-475. doi: 10.1111/ans.18773. Epub 2023 Dec 6. PMID- 33472228 OWN - NLM STAT- MEDLINE DCOM- 20210906 LR - 20221207 IS - 1526-6702 (Electronic) IS - 0730-2347 (Print) IS - 0730-2347 (Linking) VI - 47 IP - 4 DP - 2020 Aug 1 TI - Acute Hand Ischemia and Digital Amputation After Transradial Coronary Intervention in a Patient With CREST Syndrome. PG - 319-321 LID - 10.14503/THIJ-19-6988 [doi] AB - The radial artery approach for coronary angiography and intervention is rapidly replacing the femoral artery approach, largely because it reduces bleeding and vascular access site complications. However, complications associated with transradial access warrant attention, notably radial artery occlusion. This report focuses on a case of radial artery occlusion after percutaneous coronary intervention in a 46-year-old woman with CREST (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome, which ultimately led to acute hand ischemia necessitating amputation of her middle and index fingers. CI - © 2020 by the Texas Heart® Institute, Houston. FAU - Earl, Thomas J AU - Earl TJ AD - Michigan Heart & Vascular Specialists, McLaren Northern Michigan, Petoskey, Michigan 49770. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Tex Heart Inst J JT - Texas Heart Institute journal JID - 8214622 SB - IM MH - Amputation, Surgical/*methods MH - Animals MH - Arterial Occlusive Diseases/complications/diagnosis/*surgery MH - CREST Syndrome/*complications MH - Female MH - Finger Phalanges/blood supply/*surgery MH - Humans MH - Middle Aged MH - Percutaneous Coronary Intervention/adverse effects MH - Radial Artery PMC - PMC7819447 OTO - NOTNLM OT - Cardiac catheterization/adverse effects/methods OT - catheterization, peripheral/adverse effects OT - percutaneous coronary intervention OT - radial artery EDAT- 2021/01/21 06:00 MHDA- 2021/09/07 06:00 PMCR- 2020/08/01 CRDT- 2021/01/20 20:08 PHST- 2021/01/20 20:08 [entrez] PHST- 2021/01/21 06:00 [pubmed] PHST- 2021/09/07 06:00 [medline] PHST- 2020/08/01 00:00 [pmc-release] AID - 450160 [pii] AID - 10.14503/THIJ-19-6988 [doi] PST - ppublish SO - Tex Heart Inst J. 2020 Aug 1;47(4):319-321. doi: 10.14503/THIJ-19-6988. PMID- 30967221 OWN - NLM STAT- MEDLINE DCOM- 20191031 LR - 20191031 IS - 1879-355X (Electronic) IS - 0360-3016 (Linking) VI - 104 IP - 1 DP - 2019 May 1 TI - Is Postoperative Breast Radiation Therapy Safe in Patients With Scleroderma? PG - 10-11 LID - S0360-3016(19)30244-5 [pii] LID - 10.1016/j.ijrobp.2019.02.018 [doi] FAU - Belkacemi, Yazid AU - Belkacemi Y AD - Department of Radiation Oncology and Henri Mondor Breast Center, University of Paris-Est (UPEC), Créteil, France; INSERM Unit 955, EQ 07, IMRB, University of Paris-Est (UPEC), Créteil, France. Electronic address: yazid.belkacemi@aphp.fr. FAU - Majdoul, Soufya AU - Majdoul S AD - Department of Radiation Oncology and Henri Mondor Breast Center, University of Paris-Est (UPEC), Créteil, France. FAU - Tsoutsou, Pelagia Pauletta AU - Tsoutsou PP AD - Department of Radiation Oncology of HNE (La Chaux-de-Fonds) and University Hospital of Vaudois CHUV, Lausanne, France. FAU - Abouegylah, Mohamed AU - Abouegylah M AD - Department of Radiation Oncology, Harvard Medical School, Boston, Massachusetts; Breast Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts. FAU - Kaidar-Person, Orit AU - Kaidar-Person O AD - Oncology Institute, Radiation Oncology Unit, Rambam Medical Center, Haifa, Israel. FAU - Meattini, Icro AU - Meattini I AD - Radiation Oncology Unit, Oncology Department, AOU Careggi-University of Florence, Florence, Italy. FAU - Poortmans, Philip M P AU - Poortmans PMP AD - Department of Radiation Oncology, Institut Curie, Paris, France. FAU - Taghian, Alphonse AU - Taghian A AD - Department of Radiation Oncology, Harvard Medical School, Boston, Massachusetts; Breast Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts. LA - eng PT - Case Reports PT - Editorial PL - United States TA - Int J Radiat Oncol Biol Phys JT - International journal of radiation oncology, biology, physics JID - 7603616 SB - IM CIN - Int J Radiat Oncol Biol Phys. 2019 May 1;104(1):12-13. doi: 10.1016/j.ijrobp.2019.02.029. PMID: 30967222 CIN - Int J Radiat Oncol Biol Phys. 2019 May 1;104(1):13-15. doi: 10.1016/j.ijrobp.2019.02.028. PMID: 30967223 CIN - Int J Radiat Oncol Biol Phys. 2019 May 1;104(1):15-16. doi: 10.1016/j.ijrobp.2019.02.027. PMID: 30967224 MH - Aged MH - Breast Carcinoma In Situ/*radiotherapy/surgery MH - Breast Neoplasms/*radiotherapy/surgery MH - CREST Syndrome/*complications MH - Clinical Decision-Making MH - Conservative Treatment MH - Female MH - Humans MH - Mastectomy MH - Postoperative Care MH - Radiation Dose Hypofractionation MH - Radiotherapy, Adjuvant EDAT- 2019/04/11 06:00 MHDA- 2019/11/02 06:00 CRDT- 2019/04/11 06:00 PHST- 2018/05/10 00:00 [received] PHST- 2019/01/27 00:00 [revised] PHST- 2019/02/06 00:00 [accepted] PHST- 2019/04/11 06:00 [entrez] PHST- 2019/04/11 06:00 [pubmed] PHST- 2019/11/02 06:00 [medline] AID - S0360-3016(19)30244-5 [pii] AID - 10.1016/j.ijrobp.2019.02.018 [doi] PST - ppublish SO - Int J Radiat Oncol Biol Phys. 2019 May 1;104(1):10-11. doi: 10.1016/j.ijrobp.2019.02.018. PMID- 30893393 OWN - NLM STAT- MEDLINE DCOM- 20190913 LR - 20190913 IS - 2326-6929 (Electronic) IS - 0011-4162 (Linking) VI - 103 IP - 2 DP - 2019 Feb TI - Erythematous edematous plaques on the dorsal aspects of the hands. PG - E36-E38 FAU - Bell, Austin G AU - Bell AG AD - Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. FAU - Roman, John W AU - Roman JW AD - Dermatology Department, Walter Reed National Military Medical Center, Bethesda, USA. FAU - Kentosh, Joshua B AU - Kentosh JB AD - Dermatology Department, Walter Reed National Military Medical Center, Bethesda, USA. LA - eng PT - Case Reports PT - Letter PL - United States TA - Cutis JT - Cutis JID - 0006440 RN - 0 (Glucocorticoids) RN - 0 (Photosensitizing Agents) RN - ADN79D536H (Clobetasol) SB - IM MH - Administration, Topical MH - CREST Syndrome/diagnosis MH - Citrus aurantiifolia/*adverse effects MH - Clobetasol/*administration & dosage MH - Diagnosis, Differential MH - Female MH - Fruit and Vegetable Juices/adverse effects MH - Glucocorticoids/administration & dosage MH - *Hand MH - Humans MH - Lupus Erythematosus, Cutaneous/diagnosis MH - Middle Aged MH - *Photosensitivity Disorders/diagnosis/etiology/physiopathology/therapy MH - Photosensitizing Agents/*adverse effects MH - Plant Structures/adverse effects EDAT- 2019/03/21 06:00 MHDA- 2019/09/14 06:00 CRDT- 2019/03/21 06:00 PHST- 2019/03/21 06:00 [entrez] PHST- 2019/03/21 06:00 [pubmed] PHST- 2019/09/14 06:00 [medline] PST - ppublish SO - Cutis. 2019 Feb;103(2):E36-E38. PMID- 28990976 OWN - NLM STAT- MEDLINE DCOM- 20180730 LR - 20180730 IS - 1537-2677 (Electronic) IS - 0740-9303 (Linking) VI - 34 IP - 2 DP - 2018 Mar/Apr TI - Orbital Inflammation as a Presenting Sign for CREST Syndrome. PG - e43-e45 LID - 10.1097/IOP.0000000000001007 [doi] AB - A 61-year-old male was referred with a week's history of a painful and swollen left eye. Examination revealed normal visual acuities, left proptosis and global restriction of ocular ductions, and subretinal fluid at the macula. CT imaging confirmed thickening of the posterior scleral coat, with an associated choroidal effusion. Serology revealed positive antinuclear antibodies with a centromere staining pattern; subsequent rheumatology review revealed extensive telangiectasia with digital ulceration in both hands, and a diagnosis of limited cutaneous systemic sclerosis was made. Orbital inflammatory disease is often the initial presentation of systemic diseases such as sarcoidosis, granulomatosis with polyangiitis, and IgG4 disease. Limited cutaneous systemic sclerosis is rarely encountered in the context of orbital inflammation, but is a further systemic association, reminding the clinician of the diagnostic importance of peripheral symptoms and serological markers in patients presenting with orbital inflammation and scleritis. FAU - Hamed-Azzam, Shirin W AU - Hamed-Azzam SW FAU - D'Cruz, David P AU - D'Cruz DP FAU - Verity, David H AU - Verity DH LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Ophthalmic Plast Reconstr Surg JT - Ophthalmic plastic and reconstructive surgery JID - 8508431 SB - IM MH - CREST Syndrome/*complications MH - Dacryocystitis/*etiology MH - Humans MH - Male MH - Middle Aged MH - Scleritis/*etiology EDAT- 2017/10/11 06:00 MHDA- 2018/07/31 06:00 CRDT- 2017/10/10 06:00 PHST- 2017/10/11 06:00 [pubmed] PHST- 2018/07/31 06:00 [medline] PHST- 2017/10/10 06:00 [entrez] AID - 10.1097/IOP.0000000000001007 [doi] PST - ppublish SO - Ophthalmic Plast Reconstr Surg. 2018 Mar/Apr;34(2):e43-e45. doi: 10.1097/IOP.0000000000001007. PMID- 27811572 OWN - NLM STAT- MEDLINE DCOM- 20170811 LR - 20190221 IS - 1536-481X (Electronic) IS - 1057-0829 (Linking) VI - 26 IP - 2 DP - 2017 Feb TI - Complications in Placement of a Glaucoma Drainage Device in a Patient With CREST Syndrome. PG - e82-e83 LID - 10.1097/IJG.0000000000000568 [doi] AB - This case describes difficulty with conjunctival closure in an 80-year-old woman with CREST syndrome, a subset of systemic scleroderma. The patient underwent uneventful glaucoma drainage device implantation, but friability of the conjunctiva was noted during closure. Amniotic membrane was used to ensure secure closure, and the patient had a successful outcome. There is a paucity of existing studies on patients with CREST syndrome and their ocular findings. This report sheds light on ocular findings in this autoimmune disease and the necessity for alternative closure methods in special cases. FAU - Husain, Maria Q AU - Husain MQ AD - Braverman-Terry-Oei Eye Associates, San Antonio, TX. FAU - Alsheikh, Oday AU - Alsheikh O LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Glaucoma JT - Journal of glaucoma JID - 9300903 SB - IM MH - Aged, 80 and over MH - Amnion/transplantation MH - CREST Syndrome/*complications MH - Conjunctival Diseases/*etiology/surgery MH - Female MH - Glaucoma Drainage Implants/*adverse effects MH - Glaucoma, Open-Angle/physiopathology/*surgery MH - Humans MH - Intraocular Pressure/physiology MH - Prosthesis Implantation/adverse effects MH - Retrospective Studies MH - Suture Techniques EDAT- 2016/11/05 06:00 MHDA- 2017/08/12 06:00 CRDT- 2016/11/05 06:00 PHST- 2016/11/05 06:00 [pubmed] PHST- 2017/08/12 06:00 [medline] PHST- 2016/11/05 06:00 [entrez] AID - 10.1097/IJG.0000000000000568 [doi] PST - ppublish SO - J Glaucoma. 2017 Feb;26(2):e82-e83. doi: 10.1097/IJG.0000000000000568. PMID- 26992073 OWN - NLM STAT- MEDLINE DCOM- 20170911 LR - 20170911 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 175 IP - 5 DP - 2016 Nov TI - Worsening of calcinosis cutis with teriparatide treatment in two osteoporotic patients. PG - 1049-1051 LID - 10.1111/bjd.14550 [doi] AB - We present two cases of patients with systemic autoimmune diseases (one with dermatomyositis and one with CREST syndrome) who presented with a worsening of calcinosis cutis after treatment of osteoporosis with teriparatide. To our knowledge, this association is not described in the literature and might be considered in the spectrum of adverse reactions to teriparatide. CI - © 2016 British Association of Dermatologists. FAU - Echeverri, A F AU - Echeverri AF AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. FAU - Ospina, F E AU - Ospina FE AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. AD - Clinical Investigation Institute, Fundación Valle del Lili, Cali, Colombia. FAU - Cañas, C A AU - Cañas CA AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. FAU - Agualimpia, A AU - Agualimpia A AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. FAU - Suso, J P AU - Suso JP AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. AD - Clinical Investigation Institute, Fundación Valle del Lili, Cali, Colombia. FAU - Tobón, G J AU - Tobón GJ AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. gtobon1@yahoo.com. AD - Laboratory of Immunology, Fundación Valle del Lili, Cali, Colombia. gtobon1@yahoo.com. FAU - Bonilla-Abadía, F AU - Bonilla-Abadía F AD - Unit of Rheumatology, Fundación Valle del Lili, Cali, Colombia. LA - eng PT - Case Reports PT - Journal Article DEP - 20160620 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Bone Density Conservation Agents) RN - 10T9CSU89I (Teriparatide) SB - IM MH - Aged MH - Bone Density Conservation Agents/*adverse effects MH - CREST Syndrome/complications MH - Calcinosis/*chemically induced MH - Dermatomyositis/complications MH - Female MH - Humans MH - Middle Aged MH - Osteoporosis/complications/*drug therapy MH - Skin Diseases/*chemically induced MH - Teriparatide/*adverse effects EDAT- 2016/10/30 06:00 MHDA- 2017/09/12 06:00 CRDT- 2016/03/19 06:00 PHST- 2016/02/17 00:00 [accepted] PHST- 2016/10/30 06:00 [pubmed] PHST- 2017/09/12 06:00 [medline] PHST- 2016/03/19 06:00 [entrez] AID - 10.1111/bjd.14550 [doi] PST - ppublish SO - Br J Dermatol. 2016 Nov;175(5):1049-1051. doi: 10.1111/bjd.14550. Epub 2016 Jun 20. PMID- 26885625 OWN - NLM STAT- MEDLINE DCOM- 20170117 LR - 20220311 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 34 Suppl 100 IP - 5 DP - 2016 Sep-Oct TI - Different contributions of angiostatin and endostatin in angiogenesis impairment in systemic sclerosis: a cohort study. PG - 37-42 AB - OBJECTIVES: To determine the concentrations of circulating endostatin and angiostatin in patients with systemic sclerosis (SSc) and to assess its relationship to disease subsets, evolution phase, organ involvement and nailfold capillaroscopic changes. METHODS: Endostatin and angiostatin serum levels were measured by ELISA in a cohort of 57 patients with SSc, and correlated with disease subsets, evolution phase, organ involvement and nailfold capillaroscopic changes. RESULTS: Endostatin and angiostatin serum levels were significantly higher in patients with SSc than in healthy controls. Also, angiostatin was elevated in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), but not in pre-SSc, while endostatin was increased in all SSc subsets. Moreover, endostatin was augmented in lcSSc, with or without CREST syndrome, whereas angiostatin was increased exclusively in patients with CREST. Analysis according to disease evolution phase found that endostatin was elevated in all phases while angiostatin was only significantly higher in intermediate and late phases of disease. Analysis regarding organ involvement revealed that angiostatin was significantly higher in patients with osteoarticular involvement and with more serious lung affection; no significant differences were found for endostatin. Finally, endostatin was significantly increased in all nailfold capillaroscopy stages, while angiostatin was only elevated in active and late phases. CONCLUSIONS: In accordance with previous studies, we found that endostatin and angiostatin concentrations are elevated in SSc patients. Additionally, we recognised the important role that endostatin might play as an early disease marker and realized that angiostatin is a marker of late disease and relates to lung disease severity. FAU - Almeida, Isabel AU - Almeida I AD - Clinical Immunology Unit, Dept.
 of Medicine, Hosp. de Santo António (HSA), Centro Hospitalar do Porto (CHP); and Multidisciplinary Unit for Biomedical Investigation (UMIB), Instituto de Ciências Biomédicas Abel Salazar (ICBAS), Univ. do Porto, Portugal. FAU - Oliveira Gomes, Ana AU - Oliveira Gomes A AD - Clinical Immunology Unit, Department of Medicine, Centro Hospitalar do Porto, Porto, Portugal. FAU - Lima, Margarida AU - Lima M AD - Laboratory of Cytometry, Dept. of Haematology, Hospital de Santo António (HSA), Centro Hospitalar do Porto (CHP); and Multidisciplinary Unit for Biomedical Investigation (UMIB), Inst. de Ciências Biomédicas Abel Salazar (ICBAS), Univ. do Porto, Portugal. FAU - Silva, Ivone AU - Silva I AD - Department of Vascular Surgery, Centro Hospitalar do Porto, Porto, Portugal. FAU - Vasconcelos, Carlos AU - Vasconcelos C AD - Clinical Immunology Unit, Dept.
 of Medicine, Hosp. de Santo António (HSA), Centro Hospitalar do Porto (CHP); and Multidisciplinary Unit for Biomedical Investigation (UMIB), Instituto de Ciências Biomédicas Abel Salazar (ICBAS), Univ. do Porto, Portugal. LA - eng PT - Comparative Study PT - Journal Article DEP - 20160216 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Biomarkers) RN - 0 (Endostatins) RN - 86090-08-6 (Angiostatins) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Angiostatins/*blood MH - Biomarkers/blood MH - CREST Syndrome/blood/pathology MH - Case-Control Studies MH - Cohort Studies MH - Disease Progression MH - Early Diagnosis MH - Endostatins/*blood MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - *Neovascularization, Pathologic MH - Predictive Value of Tests MH - Scleroderma, Diffuse/*blood/pathology MH - Scleroderma, Limited/*blood/pathology MH - Severity of Illness Index MH - Signal Transduction MH - Skin/*blood supply MH - Up-Regulation MH - Young Adult EDAT- 2016/10/18 06:00 MHDA- 2017/01/18 06:00 CRDT- 2016/02/18 06:00 PHST- 2015/09/16 00:00 [received] PHST- 2015/12/14 00:00 [accepted] PHST- 2016/10/18 06:00 [pubmed] PHST- 2017/01/18 06:00 [medline] PHST- 2016/02/18 06:00 [entrez] AID - 9973 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):37-42. Epub 2016 Feb 16. PMID- 27353193 OWN - NLM STAT- MEDLINE DCOM- 20180723 LR - 20180723 IS - 1885-1398 (Electronic) IS - 1699-258X (Linking) VI - 13 IP - 6 DP - 2017 Nov-Dec TI - Upper gastrointestinal bleeding (watermelon stomach) in a patient with limited scleroderma (CREST syndrome). PG - 361-362 LID - S1699-258X(16)30055-9 [pii] LID - 10.1016/j.reuma.2016.05.011 [doi] FAU - Turrión Nieves, Ana Isabel AU - Turrión Nieves AI AD - Servicio de ESI-Reumatología, Departamento de Medicina Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España; Unidad Docente de Anatomía y Embriología Humanas, Departamento de Cirugía y Ciencias Médico Sociales, Universidad de Alcalá de Henares, Alcalá de Henares, Madrid, España. Electronic address: anaturrion@hotmail.com. FAU - Moruno Cruz, Henry AU - Moruno Cruz H AD - Servicio de ESI-Reumatología, Departamento de Medicina Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España. FAU - Romero Bogado, M Liz AU - Romero Bogado ML AD - Servicio de ESI-Reumatología, Departamento de Medicina Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España. FAU - Perez Gómez, Ana AU - Perez Gómez A AD - Servicio de ESI-Reumatología, Departamento de Medicina Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, España. LA - eng LA - spa PT - Case Reports PT - Journal Article TT - Hemorragia digestiva alta (estómago de sandía) en paciente con esclerodermia limitada (síndrome de CREST). DEP - 20160625 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 SB - IM MH - Antiphospholipid Syndrome/complications MH - Argon Plasma Coagulation MH - CREST Syndrome/*complications MH - Gastric Antral Vascular Ectasia/diagnostic imaging/etiology/surgery MH - Gastrointestinal Hemorrhage/*etiology/surgery MH - Gastroscopy MH - Humans MH - Male MH - Middle Aged EDAT- 2016/06/30 06:00 MHDA- 2018/07/24 06:00 CRDT- 2016/06/30 06:00 PHST- 2016/01/16 00:00 [received] PHST- 2016/05/20 00:00 [revised] PHST- 2016/05/26 00:00 [accepted] PHST- 2016/06/30 06:00 [pubmed] PHST- 2018/07/24 06:00 [medline] PHST- 2016/06/30 06:00 [entrez] AID - S1699-258X(16)30055-9 [pii] AID - 10.1016/j.reuma.2016.05.011 [doi] PST - ppublish SO - Reumatol Clin. 2017 Nov-Dec;13(6):361-362. doi: 10.1016/j.reuma.2016.05.011. Epub 2016 Jun 25. PMID- 27337840 OWN - NLM STAT- MEDLINE DCOM- 20160729 LR - 20160624 IS - 0370-629X (Print) IS - 0370-629X (Linking) VI - 71 IP - 5 DP - 2016 May TI - [LEFT ATRIAL APPENDAGE CLOSURE IN A PATIENT WITH SYSTEMIC SCLEROSIS]. PG - 227-32 AB - We report the clinical history of a 69 year-old female who suffered from systemic sclerosis and in whom we performed a percutaneous left atrial appendage closure due to recurrent gastrointestinal bleedings under anticoagulant therapy for chronic atrial fibrillation. We review the impact of scleroderma on the cardiac and digestive systems and discuss the issue of anticoagulation and its alternatives in uncommon clinical situations. We also describe the indications, technical aspects and potential complications of percutaneous left atrial appendage closure. FAU - Haeck, G AU - Haeck G FAU - Bataille, Y AU - Bataille Y FAU - Herzet, J M AU - Herzet JM FAU - Lecoq, E AU - Lecoq E FAU - Hoffer, E AU - Hoffer E LA - fre PT - Case Reports PT - English Abstract PT - Journal Article PT - Review TT - LE CAS CLINIQUE DU MOIS. Fermeture percutanée de L'auricule gauche chez une patiente souffrant de sclérodermie. PL - Belgium TA - Rev Med Liege JT - Revue medicale de Liege JID - 0404317 RN - 0 (Anticoagulants) SB - IM MH - Aged MH - Anticoagulants/therapeutic use MH - Atrial Appendage/*surgery MH - Atrial Fibrillation/complications/drug therapy MH - CREST Syndrome/surgery MH - Endovascular Procedures/*methods MH - Female MH - Gastrointestinal Hemorrhage/complications/surgery MH - Humans MH - Recurrence MH - Scleroderma, Systemic/*surgery EDAT- 2016/06/25 06:00 MHDA- 2016/07/30 06:00 CRDT- 2016/06/25 06:00 PHST- 2016/06/25 06:00 [entrez] PHST- 2016/06/25 06:00 [pubmed] PHST- 2016/07/30 06:00 [medline] PST - ppublish SO - Rev Med Liege. 2016 May;71(5):227-32. PMID- 26476904 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 23 IP - 3 DP - 2015 TI - Tinea Incognita in a Patient with Crest Syndrome: Case Report. PG - 199-202 AB - Tinea incognita is a dermatophytic infection that is difficult to diagnose, usually modified by inappropriate topical or systemic corticosteroid therapy. We report an extensive case of tinea incognita caused by the zoophilic dermatophyte Trichophyton mentagrophytes (var. granulosa) in a 49-year-old female patient with CREST (Calcinosis; Raynaud phenomenon; Esophageal involvement; Sclerodactyly; Teleangiectasia) syndrome. Immunocompromised patients, as well as patients with keratinization disorders, seem to be especially susceptible to dermatophytic infections with atypical clinical presentation that is sometimes bizarre and difficult to recognize. Therefore, close monitoring and mycological skin examination is recommended in order to avoid misdiagnosis and to give the patient the best chance of recovery. FAU - Gorgievska-Sukarovska, Biljana AU - Gorgievska-Sukarovska B AD - Biljana Gorgievska-Sukarovska, MD, Dermatology and Venereology Unit, Zabok General Hospital, Bračak 8 , 49000 Zabok, Croatia; biljana.gs@gmail.com. FAU - Skerlev, Mihael AU - Skerlev M FAU - Žele-Starčević, Lidija AU - Žele-Starčević L LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 RN - 0 (Antifungal Agents) SB - IM MH - Antifungal Agents/therapeutic use MH - CREST Syndrome/*complications MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Tinea/*diagnosis/drug therapy/*microbiology MH - Trichophyton EDAT- 2015/10/20 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/10/19 06:00 PHST- 2015/10/19 06:00 [entrez] PHST- 2015/10/20 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] PST - ppublish SO - Acta Dermatovenerol Croat. 2015;23(3):199-202. PMID- 25393970 OWN - NLM STAT- MEDLINE DCOM- 20150115 LR - 20190930 IS - 1560-2281 (Electronic) IS - 1083-3668 (Linking) VI - 19 IP - 11 DP - 2014 TI - Pulsed versus continuous wave low-level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome): a case report. PG - 118001 LID - 10.1117/1.JBO.19.11.118001 [doi] AB - Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. The transforming growth factor beta has been identified as a major player in the pathogenic process, where low-level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940 nm using millisecond pulsing and continuous wave (CW) modes on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks using a sequential pulsing mode on one elbow and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Considerable functional and morphologic improvements were observed after LLLT, with the best results seen with the pulsing mode. No adverse effects were noted. Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome). FAU - Barolet, Daniel AU - Barolet D AD - RoseLab Skin Optics Laboratory, 3333 100th Avenue, Suite 200, Laval, Quebec, H7T 0G3, Canada. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Biomed Opt JT - Journal of biomedical optics JID - 9605853 SB - IM MH - Adult MH - *CREST Syndrome/pathology/physiopathology/therapy MH - Female MH - Humans MH - Lasers MH - Patient Satisfaction MH - Phototherapy/instrumentation/*methods MH - Skin Temperature EDAT- 2014/11/14 06:00 MHDA- 2015/01/16 06:00 CRDT- 2014/11/14 06:00 PHST- 2014/01/29 00:00 [received] PHST- 2014/10/23 00:00 [accepted] PHST- 2014/11/14 06:00 [entrez] PHST- 2014/11/14 06:00 [pubmed] PHST- 2015/01/16 06:00 [medline] AID - 1936163 [pii] AID - 10.1117/1.JBO.19.11.118001 [doi] PST - ppublish SO - J Biomed Opt. 2014;19(11):118001. doi: 10.1117/1.JBO.19.11.118001. PMID- 24842570 OWN - NLM STAT- MEDLINE DCOM- 20150521 LR - 20181202 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 44 IP - 2 DP - 2014 Oct TI - Flare of calcinosis despite rituximab therapy. PG - e5-6 LID - S0049-0172(14)00055-9 [pii] LID - 10.1016/j.semarthrit.2014.04.007 [doi] FAU - Hurabielle, Charlotte AU - Hurabielle C AD - Paris Descartes University, Rheumatology A Department, Cochin Hospital, APHP, Paris, France. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University, Rheumatology A Department, Cochin Hospital, APHP, Paris, France. FAU - Kahan, André AU - Kahan A AD - Paris Descartes University, Rheumatology A Department, Cochin Hospital, APHP, Paris, France. FAU - Avouac, Jérôme AU - Avouac J AD - Paris Descartes University, Rheumatology A Department, Cochin Hospital, APHP, Paris, France. Electronic address: jerome.avouac@cch.aphp.fr. LA - eng PT - Comment PT - Letter DEP - 20140413 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Antirheumatic Agents) SB - IM CON - Semin Arthritis Rheum. 2012 Jun;41(6):822-9. doi: 10.1016/j.semarthrit.2011.11.007. PMID: 22221908 MH - Antibodies, Monoclonal, Murine-Derived/*therapeutic use MH - Antirheumatic Agents/*therapeutic use MH - CREST Syndrome/*drug therapy MH - Calcinosis/*drug therapy MH - Female MH - Humans MH - Scleroderma, Systemic/*complications EDAT- 2014/05/21 06:00 MHDA- 2015/05/23 06:00 CRDT- 2014/05/21 06:00 PHST- 2014/02/24 00:00 [received] PHST- 2014/04/07 00:00 [accepted] PHST- 2014/05/21 06:00 [entrez] PHST- 2014/05/21 06:00 [pubmed] PHST- 2015/05/23 06:00 [medline] AID - S0049-0172(14)00055-9 [pii] AID - 10.1016/j.semarthrit.2014.04.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2014 Oct;44(2):e5-6. doi: 10.1016/j.semarthrit.2014.04.007. Epub 2014 Apr 13. PMID- 24184038 OWN - NLM STAT- MEDLINE DCOM- 20140901 LR - 20211203 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 24 IP - 8 DP - 2013 Dec TI - Clinical significance of autoantibodies to the pericentromeric heterochromatin protein 1a protein. PG - 868-71 LID - S0953-6205(13)00178-7 [pii] LID - 10.1016/j.ejim.2013.06.013 [doi] AB - OBJECTIVE: The objective of the study is to determine the frequency and the clinical significance of autoantibodies to the pericentromeric heterochromatin protein 1 (HP1). So far this antinuclear antibody specificity has been mainly reported in patients with the CREST syndrome. METHODS: We screened the sera of 199 individuals, including patients suffering from various autoimmune disorders (Group I, n=145) and non autoimmune diseases (Group II, n=44 patients) as well as healthy individuals (Group III, n=30). The sera were systematically tested by Western blot and ELISA using a GST-HP1α fusion protein as an antigen. RESULTS: Anti-HP1 antibodies were detected in 32% of patients in Group I, 11.3% in Group II and 3.3% of individuals in Group III. They could be detected in sera containing or not antinuclear antibodies detectable by indirect immunofluorescence. Anti-HP1 antibodies were mostly associated with the CREST and Sjogren's syndromes (70% and 44.4%, respectively). They could also be detected in 22.2% of patients suffering from various other autoimmune diseases. However, their negative predictive value was 94% in the CREST syndrome. CONCLUSION: Anti-HP1 autoantibodies are associated with a large spectrum of disorders. However, they have a diagnostic value in the CREST syndrome. CI - © 2013. FAU - Coppo, Paul AU - Coppo P AD - Département d'Hématologie, Hôpital Saint-Antoine, Centre de Référence des Microangiopathies thrombotiques, France; Hôpital Saint Antoine, AP-HP, Université Pierre et Marie Curie (UPMC), Paris, France; Unité Inserm U1009, Institut Gustave Roussy, Villejuif, France. FAU - Henry-Dessailly, Isabelle AU - Henry-Dessailly I FAU - Rochette, Jacques AU - Rochette J FAU - Lok, Catherine AU - Lok C FAU - Buendia, Brigitte AU - Buendia B FAU - Lassoued, Kaiss AU - Lassoued K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131031 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (CBX5 protein, human) RN - 0 (Chromosomal Proteins, Non-Histone) RN - 0 (anticentromere antibody) RN - 107283-02-3 (Chromobox Protein Homolog 5) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/*immunology MH - Antiphospholipid Syndrome/diagnosis/immunology MH - Arthritis, Rheumatoid/diagnosis/immunology MH - Autoantibodies/immunology MH - Autoimmune Diseases/diagnosis/immunology MH - CREST Syndrome/diagnosis/*immunology MH - Case-Control Studies MH - Chromobox Protein Homolog 5 MH - Chromosomal Proteins, Non-Histone/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Scleroderma, Systemic/diagnosis/immunology MH - Sjogren's Syndrome/diagnosis/*immunology MH - Young Adult OTO - NOTNLM OT - Anti-centromere antibodies OT - Antinuclear antibodies OT - Autoimmune disorders OT - CREST OT - HP1 OT - Systemic sclerosis EDAT- 2013/11/05 06:00 MHDA- 2014/09/02 06:00 CRDT- 2013/11/05 06:00 PHST- 2012/05/09 00:00 [received] PHST- 2013/05/16 00:00 [revised] PHST- 2013/06/26 00:00 [accepted] PHST- 2013/11/05 06:00 [entrez] PHST- 2013/11/05 06:00 [pubmed] PHST- 2014/09/02 06:00 [medline] AID - S0953-6205(13)00178-7 [pii] AID - 10.1016/j.ejim.2013.06.013 [doi] PST - ppublish SO - Eur J Intern Med. 2013 Dec;24(8):868-71. doi: 10.1016/j.ejim.2013.06.013. Epub 2013 Oct 31. PMID- 24142114 OWN - NLM STAT- MEDLINE DCOM- 20140716 LR - 20221207 IS - 1432-5241 (Electronic) IS - 0364-216X (Linking) VI - 37 IP - 6 DP - 2013 Dec TI - Surgical management of digital calcinosis in CREST syndrome. PG - 1214-9 LID - 10.1007/s00266-013-0224-z [doi] AB - As a limited form of sclerodermy, CREST syndrome is characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which determine the acronym CREST. Calcinosis is a particularly difficult entity to treat given the paucity of effective options described in the literature. Treatment of finger calcinosis has a wide range of possibilities depending on the extent of calcifications and the involvement of deep structures. From a surgical point of view, whereas simple removal is adequate in minor outpatient cases, a radical debridement in the major and more painful cases seems required. A cover flap is needed particularly in the thumb due to its great functional importance, also if the fingertip is not involved. The authors recommend the kite flap for the dimensions, the tissue quality, and the possibility of giving sensation to the reconstructed area. With this surgical option, the transferred skin is soft, sensate, and the right fit. Usually, no further operations are needed for flap remodeling. The time required for sensory integration is about 2 years, often related to the age of the patient. Debridement and flap reconstruction usually give total resolution of pain, with complete recovery of thumb motion and the thumb-index finger grip. FAU - Merlino, Giorgio AU - Merlino G AD - Division of Plastic Surgery and Hand Surgery, Maria Vittoria Hospital, Turin, Italy. FAU - Germano, Silvia AU - Germano S FAU - Carlucci, Salvatore AU - Carlucci S LA - eng PT - Journal Article PT - Review DEP - 20131019 PL - United States TA - Aesthetic Plast Surg JT - Aesthetic plastic surgery JID - 7701756 SB - IM MH - CREST Syndrome/diagnosis/*surgery MH - Calcinosis/diagnosis/*surgery MH - Debridement/methods MH - Female MH - Graft Survival MH - Humans MH - Male MH - Plastic Surgery Procedures/*methods MH - Recovery of Function MH - Risk Assessment MH - Severity of Illness Index MH - Surgical Flaps/blood supply/*transplantation MH - Thumb/physiopathology/*surgery MH - Treatment Outcome MH - Wound Healing/physiology EDAT- 2013/10/22 06:00 MHDA- 2014/07/17 06:00 CRDT- 2013/10/22 06:00 PHST- 2012/11/14 00:00 [received] PHST- 2013/09/08 00:00 [accepted] PHST- 2013/10/22 06:00 [entrez] PHST- 2013/10/22 06:00 [pubmed] PHST- 2014/07/17 06:00 [medline] AID - 10.1007/s00266-013-0224-z [doi] PST - ppublish SO - Aesthetic Plast Surg. 2013 Dec;37(6):1214-9. doi: 10.1007/s00266-013-0224-z. Epub 2013 Oct 19. PMID- 22392867 OWN - NLM STAT- MEDLINE DCOM- 20120424 LR - 20121115 IS - 1524-4539 (Electronic) IS - 0009-7322 (Linking) VI - 125 IP - 9 DP - 2012 Mar 6 TI - Peripheral phenomena in a woman with calcinosis, raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome-associated pulmonary hypertension. PG - e413-4 LID - 10.1161/CIRCULATIONAHA.111.054130 [doi] FAU - Cutler, Todd S AU - Cutler TS AD - New York University, Langone Medical Center, New York, NY 10016, USA. FAU - Reyentovich, Alex AU - Reyentovich A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Circulation JT - Circulation JID - 0147763 SB - IM EIN - Circulation. 2012 Apr 3;125(13):e541 MH - Aged, 80 and over MH - CREST Syndrome/*complications/pathology MH - Calcinosis/pathology MH - *Cardiac Catheterization MH - *Electrocardiography MH - Female MH - Hand/pathology MH - Humans MH - Hypertension, Pulmonary/*diagnosis/*etiology EDAT- 2012/03/07 06:00 MHDA- 2012/04/25 06:00 CRDT- 2012/03/07 06:00 PHST- 2012/03/07 06:00 [entrez] PHST- 2012/03/07 06:00 [pubmed] PHST- 2012/04/25 06:00 [medline] AID - 125/9/e413 [pii] AID - 10.1161/CIRCULATIONAHA.111.054130 [doi] PST - ppublish SO - Circulation. 2012 Mar 6;125(9):e413-4. doi: 10.1161/CIRCULATIONAHA.111.054130. PMID- 22387650 OWN - NLM STAT- MEDLINE DCOM- 20120628 LR - 20151221 IS - 0972-2823 (Electronic) IS - 0022-3859 (Linking) VI - 58 IP - 1 DP - 2012 Jan-Mar TI - Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome. PG - 57-9 LID - 10.4103/0022-3859.93254 [doi] AB - Clinical involvement of the peripheral nervous system in the calcinosis cutis, raynaud's phenomenon, esophageal dismotility, sclerodactyly and telangiectasia (CREST) variant of systemic sclerosis occurs infrequently and is characterized by axonal degeneration due to necrotizing vasculitis. We report a female patient with a known history of CREST syndrome, which developed a slowly progressive, distal symmetric demyelinating sensorimotor polyneuropathy (PN), with tremor and ataxia as prominent features, compatible with anti-myelin associated glycoprotein (MAG) PN. The diagnosis of PN was established by the presence of monoclonal immunoglobulin M anti-MAG antibodies (Thin-Layer Chromatography, Western Blot and enzyme-linked immunoabsorbent assay). Given the evidence that in CREST activation of T-helper cells is observed and that anti-MAG antibodies, despite the fact that they are T-cell-independent, may be influenced by an increase in T-helper function, the coexistence of these two rare autoimmune disorders in the same patient may not be incidental but related to the underlying immunological mechanisms involved. FAU - Andreadou, E AU - Andreadou E AD - Department of Neurology, Athens National University, Aeginition Hospital, Athens, Greece. eandread@med.uoa.gr FAU - Zouvelou, V AU - Zouvelou V FAU - Karandreas, N AU - Karandreas N FAU - Kilidireas, C AU - Kilidireas C LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Postgrad Med JT - Journal of postgraduate medicine JID - 2985196R RN - 0 (Antibodies, Anti-Idiotypic) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulin M) RN - 0 (Immunosuppressive Agents) RN - 0 (Myelin-Associated Glycoprotein) RN - 0 (anti-IgM) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Aged MH - Antibodies, Anti-Idiotypic MH - Ataxia/etiology MH - Autoantibodies/*analysis MH - Blotting, Western MH - CREST Syndrome/*complications/immunology MH - Chromatography, Thin Layer MH - Cyclophosphamide/therapeutic use MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Immunoglobulin M MH - Immunosuppressive Agents/therapeutic use MH - Myelin-Associated Glycoprotein/*immunology MH - Polyneuropathies/complications/*immunology MH - Treatment Outcome MH - Tremor/etiology EDAT- 2012/03/06 06:00 MHDA- 2012/06/29 06:00 CRDT- 2012/03/06 06:00 PHST- 2012/03/06 06:00 [entrez] PHST- 2012/03/06 06:00 [pubmed] PHST- 2012/06/29 06:00 [medline] AID - jpgm_2012_58_1_57_93254 [pii] AID - 10.4103/0022-3859.93254 [doi] PST - ppublish SO - J Postgrad Med. 2012 Jan-Mar;58(1):57-9. doi: 10.4103/0022-3859.93254. PMID- 22333384 OWN - NLM STAT- MEDLINE DCOM- 20130312 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 51 IP - 4 DP - 2012 TI - CREST syndrome with pulmonary arterial hypertension. PG - 441-2 FAU - Fukuda, Yusuke AU - Fukuda Y AD - Department of Cardiology, Fukuoka University School of Medicine, Japan. zfukusuke@minf.med.fukuoka-u.ac.jp FAU - Miura, Shin-ichiro AU - Miura S FAU - Saku, Keijiro AU - Saku K LA - eng PT - Case Reports PT - Journal Article DEP - 20120215 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM MH - Aged MH - CREST Syndrome/complications/*diagnostic imaging MH - Echocardiography MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hypertension, Pulmonary/complications/*diagnostic imaging MH - Radiography EDAT- 2012/02/16 06:00 MHDA- 2013/03/13 06:00 CRDT- 2012/02/16 06:00 PHST- 2012/02/16 06:00 [entrez] PHST- 2012/02/16 06:00 [pubmed] PHST- 2013/03/13 06:00 [medline] AID - JST.JSTAGE/internalmedicine/51.6561 [pii] AID - 10.2169/internalmedicine.51.6561 [doi] PST - ppublish SO - Intern Med. 2012;51(4):441-2. doi: 10.2169/internalmedicine.51.6561. Epub 2012 Feb 15. PMID- 21486675 OWN - NLM STAT- MEDLINE DCOM- 20110920 LR - 20220409 IS - 1879-1298 (Electronic) IS - 0045-6535 (Linking) VI - 84 IP - 5 DP - 2011 Jul TI - Detection of environmental clastogens and aneugens in human fibroblasts by cytokinesis-blocked micronucleus assay associated with immunofluorescent staining of CENP-A in micronuclei. PG - 676-80 LID - 10.1016/j.chemosphere.2011.03.027 [doi] AB - The cytokinesis-blocked micronucleus (CBMN) assay, in combination with fluorescent in situ hybridization (FISH) of human pan-centromeric DNA probes, or with CREST antibodies that specifically stain kinetochore proteins, is widely used on several cell types. It distinguishes micronuclei containing one or several whole chromosomes, which are positively labeled (centromere positive micronucleus, C+MN, due to aneugenic effect), or acentric chromosome fragments, which are unlabeled due to the absence of centromere (centromere negative micronucleus, C-MN, due to clastogenic effect). However, the very slight level of the centromeric signals obtained with the FISH technique on primary human fibroblasts, a cell type commonly used in environmental genetic toxicology, leads to great difficulties in distinguishing C+MN and C-MN. Furthermore, the CREST technique may lead to inappropriate results particularly with regards to variations in antibody composition between patient sera. Our results show that the in vitro CBMN, in combination with immunofluorescence staining of CENP-A (centromere protein A), efficiently screens genotoxicants for their ability to induce clastogenic and/or aneugenic effects. We propose the in vitro CBMN assay in combination with immunofluorescence staining of CENP-A as a suitable tool in environmental genotoxicity testing of primary human fibroblasts. CI - Copyright © 2011 Elsevier Ltd. All rights reserved. FAU - Benameur, Laila AU - Benameur L AD - Laboratoire de Biogénotoxicologie et Mutagenèse Environnementale (EA 1784/FR CNRS 3098 ECCOREV), Aix-Marseille Université, Faculté de Médecine, Marseille, France. laila.benameur@univmed.fr FAU - Orsière, Thierry AU - Orsière T FAU - Rose, Jérôme AU - Rose J FAU - Botta, Alain AU - Botta A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110412 PL - England TA - Chemosphere JT - Chemosphere JID - 0320657 RN - 0 (Aneugens) RN - 0 (Autoantigens) RN - 0 (CENPA protein, human) RN - 0 (Centromere Protein A) RN - 0 (Chromosomal Proteins, Non-Histone) RN - 0 (Environmental Pollutants) RN - 0 (Mutagens) RN - 0 (SS18L1 protein, human) RN - 0 (Trans-Activators) SB - IM MH - Aneugens/metabolism/toxicity MH - Autoantigens/*metabolism MH - CREST Syndrome/blood MH - Centromere Protein A MH - Chromosomal Proteins, Non-Histone/*metabolism MH - Cytokinesis MH - Environmental Monitoring MH - Environmental Pollutants/*metabolism MH - Fibroblasts/drug effects/*metabolism MH - Humans MH - In Situ Hybridization, Fluorescence MH - Micronucleus Tests/*methods MH - Mutagens/*metabolism/toxicity MH - Trans-Activators/metabolism EDAT- 2011/04/14 06:00 MHDA- 2011/09/21 06:00 CRDT- 2011/04/14 06:00 PHST- 2011/01/29 00:00 [received] PHST- 2011/03/07 00:00 [revised] PHST- 2011/03/14 00:00 [accepted] PHST- 2011/04/14 06:00 [entrez] PHST- 2011/04/14 06:00 [pubmed] PHST- 2011/09/21 06:00 [medline] AID - S0045-6535(11)00307-9 [pii] AID - 10.1016/j.chemosphere.2011.03.027 [doi] PST - ppublish SO - Chemosphere. 2011 Jul;84(5):676-80. doi: 10.1016/j.chemosphere.2011.03.027. Epub 2011 Apr 12. PMID- 21474271 OWN - NLM STAT- MEDLINE DCOM- 20110726 LR - 20161125 IS - 1916-7075 (Electronic) IS - 0828-282X (Linking) VI - 27 IP - 3 DP - 2011 May-Jun TI - Coronary artery abnormalities in CREST syndrome revealed by cardiovascular magnetic resonance imaging. PG - 390.e5-7 LID - 10.1016/j.cjca.2010.12.052 [doi] AB - CREST syndrome represents a subset of systemic sclerosis (SSc). Five patients with CREST syndrome and 5 with SSc without cardiac symptoms and with normal routine cardiac examination were investigated by cardiovascular magnetic resonance. All CREST patients had ectatic coronary arteries, and in 1 of them, an inferior, transmural myocardial infarction was identified. Furthermore, patchy fibrosis was identified in all the patients with SSc, although their coronary arteries were normal. Cardiovascular magnetic resonance can be a useful, noninvasive diagnostic tool in the evaluation of asymptomatic CREST and SSc patients. CI - Copyright © 2011. Published by Elsevier Inc. FAU - Mavrogeni, Sophie AU - Mavrogeni S AD - Onassis Cardiac Surgery Centre, Athens, Greece. soma13@otenet.gr FAU - Bratis, Costas AU - Bratis C FAU - Manoussakis, Menelaos AU - Manoussakis M LA - eng PT - Comparative Study PT - Journal Article DEP - 20110406 PL - England TA - Can J Cardiol JT - The Canadian journal of cardiology JID - 8510280 SB - IM MH - Adult MH - CREST Syndrome/complications/*diagnosis MH - Coronary Angiography/methods MH - Coronary Artery Disease/*diagnosis/etiology MH - Coronary Vessel Anomalies/diagnostic imaging/pathology MH - Female MH - Humans MH - *Magnetic Resonance Angiography MH - Myocardial Infarction/diagnosis/etiology MH - Reference Values MH - Risk Assessment MH - Sampling Studies MH - Scleroderma, Systemic/complications/*diagnosis EDAT- 2011/04/09 06:00 MHDA- 2011/07/27 06:00 CRDT- 2011/04/09 06:00 PHST- 2010/06/29 00:00 [received] PHST- 2010/08/28 00:00 [accepted] PHST- 2011/04/09 06:00 [entrez] PHST- 2011/04/09 06:00 [pubmed] PHST- 2011/07/27 06:00 [medline] AID - S0828-282X(10)00068-1 [pii] AID - 10.1016/j.cjca.2010.12.052 [doi] PST - ppublish SO - Can J Cardiol. 2011 May-Jun;27(3):390.e5-7. doi: 10.1016/j.cjca.2010.12.052. Epub 2011 Apr 6. PMID- 21277800 OWN - NLM STAT- MEDLINE DCOM- 20110607 LR - 20220309 IS - 1535-7732 (Electronic) IS - 1051-0443 (Linking) VI - 22 IP - 3 DP - 2011 Mar TI - Livedo reticularis and bowel ischemia after carbon dioxide arteriography in a patient with CREST syndrome. PG - 395-9 LID - 10.1016/j.jvir.2010.11.012 [doi] AB - For 30 years, CO(2) gas has been used as a safe alternative to iodinated contrast agents for angiography in patients with renal insufficiency or allergy to iodine. CO(2) angiography is well tolerated when performed properly, and serious complications are rare. However, severe complications may occur if the physical properties of CO(2) and the specific pathophysiology of an individual patient are not carefully considered. The present report describes a case in which diffuse livedo reticularis, bowel ischemia, and renal insufficiency developed following CO(2) angiography in a patient with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia). CI - Copyright © 2011 SIR. Published by Elsevier Inc. All rights reserved. FAU - Johnson, Philip L AU - Johnson PL AD - Department of Radiology, University of Kansas Medical Center, 3901 Rainbow Blvd, Kansas City, KS 66160 66160, USA. FAU - Neperud, Julie AU - Neperud J FAU - Arnold, Jill AU - Arnold J FAU - Thomas, James AU - Thomas J LA - eng PT - Case Reports PT - Journal Article DEP - 20110201 PL - United States TA - J Vasc Interv Radiol JT - Journal of vascular and interventional radiology : JVIR JID - 9203369 RN - 0 (Contrast Media) RN - 142M471B3J (Carbon Dioxide) SB - IM MH - Angiography, Digital Subtraction/*adverse effects MH - CREST Syndrome/*complications MH - Carbon Dioxide/*adverse effects MH - Contrast Media/*adverse effects MH - Humans MH - Intestines/*blood supply MH - Ischemia/*chemically induced MH - Livedo Reticularis/*chemically induced MH - Male MH - Middle Aged MH - Renal Artery Obstruction/complications/*diagnostic imaging/therapy MH - Renal Insufficiency/chemically induced EDAT- 2011/02/01 06:00 MHDA- 2011/06/08 06:00 CRDT- 2011/02/01 06:00 PHST- 2010/08/04 00:00 [received] PHST- 2010/11/11 00:00 [accepted] PHST- 2011/02/01 06:00 [entrez] PHST- 2011/02/01 06:00 [pubmed] PHST- 2011/06/08 06:00 [medline] AID - S1051-0443(10)01101-2 [pii] AID - 10.1016/j.jvir.2010.11.012 [doi] PST - ppublish SO - J Vasc Interv Radiol. 2011 Mar;22(3):395-9. doi: 10.1016/j.jvir.2010.11.012. Epub 2011 Feb 1. PMID- 20942115 OWN - NLM STAT- MEDLINE DCOM- 20101230 LR - 20151119 IS - 0047-1852 (Print) IS - 0047-1852 (Linking) VI - 68 Suppl 6 DP - 2010 Jun TI - [Anticentromere antibody]. PG - 506-9 FAU - Imafuku, Yuji AU - Imafuku Y AD - Department of Infection Control and Laboratory Medicine, Fukushima Medical University School of Medicine. LA - jpn PT - Journal Article PL - Japan TA - Nihon Rinsho JT - Nihon rinsho. Japanese journal of clinical medicine JID - 0420546 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Reagent Kits, Diagnostic) RN - 0 (anticentromere antibody) SB - IM MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - CREST Syndrome/*diagnosis/immunology MH - Enzyme-Linked Immunosorbent Assay/methods MH - Fluorescent Antibody Technique, Indirect/methods MH - Humans MH - Reagent Kits, Diagnostic MH - Reference Values MH - Specimen Handling EDAT- 2010/10/15 06:00 MHDA- 2010/12/31 06:00 CRDT- 2010/10/15 06:00 PHST- 2010/10/15 06:00 [entrez] PHST- 2010/10/15 06:00 [pubmed] PHST- 2010/12/31 06:00 [medline] PST - ppublish SO - Nihon Rinsho. 2010 Jun;68 Suppl 6:506-9. PMID- 20584022 OWN - NLM STAT- MEDLINE DCOM- 20101109 LR - 20161125 IS - 1442-9071 (Electronic) IS - 1442-6404 (Linking) VI - 38 IP - 5 DP - 2010 Jul TI - Orbital bone and soft tissue calcification in CREST syndrome. PG - 534-6 LID - 10.1111/j.1442-9071.2010.02277.x [doi] FAU - Morris, Olivia C AU - Morris OC FAU - Verity, David H AU - Verity DH FAU - Luthert, Philip J AU - Luthert PJ FAU - Rose, Geoffrey E AU - Rose GE LA - eng PT - Case Reports PT - Letter PT - Research Support, Non-U.S. Gov't PL - Australia TA - Clin Exp Ophthalmol JT - Clinical & experimental ophthalmology JID - 100896531 SB - IM MH - CREST Syndrome/diagnostic imaging/*pathology/surgery MH - Calcinosis/diagnostic imaging/*pathology/surgery MH - Dermatologic Surgical Procedures MH - Female MH - Humans MH - Middle Aged MH - Orbit/diagnostic imaging/*pathology/surgery MH - Skin/diagnostic imaging/*pathology MH - Tomography, X-Ray Computed EDAT- 2010/06/30 06:00 MHDA- 2010/11/10 06:00 CRDT- 2010/06/30 06:00 PHST- 2010/06/30 06:00 [entrez] PHST- 2010/06/30 06:00 [pubmed] PHST- 2010/11/10 06:00 [medline] AID - CEO2277 [pii] AID - 10.1111/j.1442-9071.2010.02277.x [doi] PST - ppublish SO - Clin Exp Ophthalmol. 2010 Jul;38(5):534-6. doi: 10.1111/j.1442-9071.2010.02277.x. PMID- 20465653 OWN - NLM STAT- MEDLINE DCOM- 20100818 LR - 20100514 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 49 IP - 2 DP - 2010 Feb TI - Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. PG - 229-30 LID - 10.1111/j.1365-4632.2008.04058.x [doi] FAU - Shiman, Michael AU - Shiman M FAU - Ricotti, Carlos AU - Ricotti C FAU - Miteva, Maria AU - Miteva M FAU - Kerdel, Francisco AU - Kerdel F FAU - Romanelli, Paolo AU - Romanelli P LA - eng PT - Case Reports PT - Letter PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 SB - IM MH - Aged, 80 and over MH - Amyloidosis/complications/*pathology MH - Biopsy, Needle MH - CREST Syndrome/complications/*pathology MH - Female MH - Follow-Up Studies MH - Humans MH - Immunohistochemistry MH - Monitoring, Physiologic/methods MH - Rare Diseases MH - Severity of Illness Index MH - Skin Diseases/complications/*pathology EDAT- 2010/05/15 06:00 MHDA- 2010/08/19 06:00 CRDT- 2010/05/15 06:00 PHST- 2010/05/15 06:00 [entrez] PHST- 2010/05/15 06:00 [pubmed] PHST- 2010/08/19 06:00 [medline] AID - IJD4058 [pii] AID - 10.1111/j.1365-4632.2008.04058.x [doi] PST - ppublish SO - Int J Dermatol. 2010 Feb;49(2):229-30. doi: 10.1111/j.1365-4632.2008.04058.x. PMID- 20385616 OWN - NLM STAT- MEDLINE DCOM- 20110311 LR - 20221207 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 49 IP - 8 DP - 2010 Aug TI - Enhanced cytokine responses to Toll-like and NOD-like receptor ligands in primary biliary cirrhosis-CREST overlap syndrome. PG - 1602-4 LID - 10.1093/rheumatology/keq080 [doi] FAU - Fukuhara, Manabu AU - Fukuhara M FAU - Watanabe, Tomohiro AU - Watanabe T FAU - Ueo, Taro AU - Ueo T FAU - Ida, Hiroshi AU - Ida H FAU - Kodama, Yuzo AU - Kodama Y FAU - Chiba, Tsutomu AU - Chiba T LA - eng PT - Case Reports PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20100412 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Cytokines) RN - 0 (Ligands) RN - 0 (Nod Signaling Adaptor Proteins) RN - 0 (Toll-Like Receptors) SB - IM MH - Aged MH - Asian People MH - CREST Syndrome/*complications MH - Cytokines/*immunology MH - Female MH - Humans MH - Ligands MH - Liver Cirrhosis, Biliary/*complications MH - Nod Signaling Adaptor Proteins/*immunology MH - Toll-Like Receptors/*immunology EDAT- 2010/04/14 06:00 MHDA- 2011/03/12 06:00 CRDT- 2010/04/14 06:00 PHST- 2010/04/14 06:00 [entrez] PHST- 2010/04/14 06:00 [pubmed] PHST- 2011/03/12 06:00 [medline] AID - keq080 [pii] AID - 10.1093/rheumatology/keq080 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 Aug;49(8):1602-4. doi: 10.1093/rheumatology/keq080. Epub 2010 Apr 12. PMID- 19788612 OWN - NLM STAT- MEDLINE DCOM- 20091211 LR - 20161125 IS - 1440-1746 (Electronic) IS - 0815-9319 (Linking) VI - 24 IP - 10 DP - 2009 Oct TI - Education and Imaging. Hepatobiliary and pancreatic: idiopathic portal hypertension. PG - 1699 LID - 10.1111/j.1440-1746.2009.06099.x [doi] FAU - Samanta, R AU - Samanta R AD - Department of Hepatology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom. FAU - Davies, S AU - Davies S FAU - Griffiths, W AU - Griffiths W LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - J Gastroenterol Hepatol JT - Journal of gastroenterology and hepatology JID - 8607909 SB - IM MH - Aged MH - Biopsy MH - CREST Syndrome/*complications/pathology MH - Esophageal and Gastric Varices/etiology MH - Gastrointestinal Hemorrhage/etiology MH - Humans MH - Hypertension, Portal/*diagnosis/etiology MH - Male MH - Melena/etiology/pathology MH - *Portal Vein/diagnostic imaging/pathology MH - Risk Factors MH - Tomography, X-Ray Computed EDAT- 2009/10/01 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/10/01 06:00 PHST- 2009/10/01 06:00 [entrez] PHST- 2009/10/01 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - JGH6099 [pii] AID - 10.1111/j.1440-1746.2009.06099.x [doi] PST - ppublish SO - J Gastroenterol Hepatol. 2009 Oct;24(10):1699. doi: 10.1111/j.1440-1746.2009.06099.x. PMID- 19348177 OWN - NLM STAT- MEDLINE DCOM- 20090519 LR - 20190917 IS - 0009-918X (Print) IS - 0009-918X (Linking) VI - 49 IP - 2-3 DP - 2009 Feb-Mar TI - [A case of anti-AQP4 antibody-positive recurrent myelitis overlapped with autoimmune disorders including incomplete CREST syndrome revealed multiple discontinuous cord lesions]. PG - 115-8 AB - A 65-year-old woman presenting with multiple autoimmune disorders including incomplete CREST overlapping with aquaporin 4 (AQP4) antibody-positive recurrent myelitis was reported. She also clinically suffered from Sjogren syndrome and primary biliary cirrhosis (PBC). She had dysesthesia below C4 level, mild motor weakness and hyperreflexia without pathological reflexes on bilateral lower extremities. A T2-weighted MRI indicated multiple discontinuous spinal cord lesions at C1-5 and T7/8. A visual evoked potential study disclosed bilateral prolonged latency of P100. She clinically manifested not only incomplete CREST syndrome (facial teleangiectasia, sclerodactyly in bilateral fingers, and Raynaud's phenomenon), but also Sjögren (sicca syndrome) and PBC (jaundice). Immunoserological study showed that she was positive for anti-nuclear, anti-centromere, and anti-AQP4 (= NMO-IgG) antibodies. A combination therapy with corticosteroid and plasmapheresis was effective for all clinical symptoms. Therefore, this case stresses on the relevance of anti-AQP 4 antibody to the other overlapping autoimmune disorders, such as CREST syndrome, when recurrent myelitis is clinically diagnosed. FAU - Takahashi, Makio AU - Takahashi M AD - Department of Neurology, Kitano Hospital. FAU - Nagata, Rie AU - Nagata R FAU - Ozaki, Akihiko AU - Ozaki A FAU - Kaneko, Satoshi AU - Kaneko S FAU - Saiki, Hidemoto AU - Saiki H FAU - Matsumoto, Sadayuki AU - Matsumoto S LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 RN - 0 (AQP4 protein, human) RN - 0 (Aquaporin 4) RN - 0 (Autoantibodies) SB - IM MH - Aquaporin 4/*immunology MH - Autoantibodies/*analysis MH - Autoimmune Diseases/*immunology MH - CREST Syndrome/*complications/*immunology/pathology MH - Female MH - Humans MH - Myelitis/complications/*immunology MH - Recurrence MH - Spinal Cord/*pathology EDAT- 2009/04/08 09:00 MHDA- 2009/05/20 09:00 CRDT- 2009/04/08 09:00 PHST- 2009/04/08 09:00 [entrez] PHST- 2009/04/08 09:00 [pubmed] PHST- 2009/05/20 09:00 [medline] AID - 10.5692/clinicalneurol.49.115 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2009 Feb-Mar;49(2-3):115-8. doi: 10.5692/clinicalneurol.49.115. PMID- 18799553 OWN - NLM STAT- MEDLINE DCOM- 20080924 LR - 20161124 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 359 IP - 12 DP - 2008 Sep 18 TI - Images in clinical medicine. Colonic saccular diverticula. PG - e13 LID - 10.1056/NEJMicm075753 [doi] FAU - Steensma, Elizabeth A AU - Steensma EA AD - Brigham and Women's Hospital, Boston, MA 02115, USA. FAU - Wu, Fred M AU - Wu FM LA - eng PT - Case Reports PT - Journal Article PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM MH - CREST Syndrome/complications MH - Colon/diagnostic imaging/pathology MH - Diverticulosis, Colonic/diagnostic imaging/etiology MH - Diverticulum, Colon/*diagnostic imaging/*pathology MH - Fatal Outcome MH - Female MH - Heart Failure/etiology MH - Humans MH - Hypertension, Pulmonary/complications MH - Middle Aged MH - Radiography EDAT- 2008/09/19 09:00 MHDA- 2008/09/25 09:00 CRDT- 2008/09/19 09:00 PHST- 2008/09/19 09:00 [pubmed] PHST- 2008/09/25 09:00 [medline] PHST- 2008/09/19 09:00 [entrez] AID - 359/12/e13 [pii] AID - 10.1056/NEJMicm075753 [doi] PST - ppublish SO - N Engl J Med. 2008 Sep 18;359(12):e13. doi: 10.1056/NEJMicm075753. PMID- 18716957 OWN - NLM STAT- MEDLINE DCOM- 20081124 LR - 20220331 IS - 1744-5078 (Electronic) IS - 0927-3948 (Linking) VI - 16 IP - 4 DP - 2008 Jul-Aug TI - Juxtafoveolar telangiectasis associated with CREST syndrome. PG - 195-7 LID - 10.1080/09273940802217867 [doi] AB - PURPOSE: To report a case of CREST syndrome associated with juxtafoveolar telangiectasias (JT). DESIGN: Case report. METHODS: Observational case report. RESULTS: A 64-year-old woman affected with CREST syndrome developed bilateral visual loss. Capillary dilatation and permeability changes in the outer retina were noticed during an angiographic study. Optical coherence tomography (OCT) showed thickening with loss of the foveal depression and inner lamellar cyst. The patient was diagnosed as stage 3, group 2A JT associated with CREST syndrome. CONCLUSIONS: Finding JT in association with CREST syndrome suggests a common pathophysiologic process. FAU - Huerva, Valentin AU - Huerva V AD - Department of Ophthalmology, University Hospital Arnau de Vilanova, Lleida, Spain. vhuerva@mixmail.com FAU - Sánchez, M Carmen AU - Sánchez MC LA - eng PT - Case Reports PT - Journal Article PL - England TA - Ocul Immunol Inflamm JT - Ocular immunology and inflammation JID - 9312169 SB - IM MH - CREST Syndrome/*complications MH - Female MH - Fluorescein Angiography MH - Fovea Centralis MH - Humans MH - Middle Aged MH - Retinal Diseases/*complications/diagnosis MH - Retinal Vessels/*pathology MH - Telangiectasis/*complications/diagnosis MH - Tomography, Optical Coherence MH - Visual Acuity EDAT- 2008/08/22 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/08/22 09:00 PHST- 2008/08/22 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/08/22 09:00 [entrez] AID - 901778818 [pii] AID - 10.1080/09273940802217867 [doi] PST - ppublish SO - Ocul Immunol Inflamm. 2008 Jul-Aug;16(4):195-7. doi: 10.1080/09273940802217867. PMID- 18187755 OWN - NLM STAT- MEDLINE DCOM- 20080306 LR - 20080111 IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 133 IP - 1 DP - 2008 Jan TI - Unusual dyspnea in a woman with CREST syndrome. PG - 286-90 LID - 10.1378/chest.07-1211 [doi] FAU - Tzeng, David Z AU - Tzeng DZ AD - Pulmonary and Critical Care Medicine, University of California Davis Medical Center, Sacramento, CA 95835, USA. david.tzeng@ucdmc.ucdavis.edu FAU - Leslie, Kevin O AU - Leslie KO FAU - Shelton, David AU - Shelton D FAU - Chan, Andrew AU - Chan A LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 SB - IM MH - Bronchiolitis/complications/*diagnosis MH - CREST Syndrome/complications MH - Dyspnea/etiology MH - Female MH - Humans MH - Middle Aged EDAT- 2008/01/12 09:00 MHDA- 2008/03/07 09:00 CRDT- 2008/01/12 09:00 PHST- 2008/01/12 09:00 [pubmed] PHST- 2008/03/07 09:00 [medline] PHST- 2008/01/12 09:00 [entrez] AID - S0012-3692(15)48991-3 [pii] AID - 10.1378/chest.07-1211 [doi] PST - ppublish SO - Chest. 2008 Jan;133(1):286-90. doi: 10.1378/chest.07-1211. PMID- 17592207 OWN - NLM STAT- MEDLINE DCOM- 20071101 LR - 20191110 IS - 1349-2365 (Print) IS - 1349-2365 (Linking) VI - 48 IP - 3 DP - 2007 May TI - Combination therapy with oral sildenafil and beraprost for pulmonary arterial hypertension associated with CREST syndrome. PG - 417-22 AB - Pulmonary arterial hypertension (PAH) is commonly associated with CREST (Calcinosis, Raynaud phenomenon, Esophageal motility disorders, Sclerodactyly, and Telangiectasia) syndrome. Sildenafil, an oral phosphodiesterase type-5 inhibitor, may offer benefits in the pharmacological management of PAH. However, little is known about the long-term hemodynamic effects of sildenafil, and the potential role of sildenafil in long-term combination with beraprost, an oral prostacyclin analogue, remains unclear. We therefore examined the hemodynamic effect of oral sildenafil alone and when coadministered with beraprost in a patient with PAH associated with CREST syndrome. Traces of the acute hemodynamic effects of beraprost (20 microg) disappeared after 2 hours. In contrast, the acute hemodynamic effects of sildenafil (50 mg) produced a greater reduction in PAP (31%) and PVR (40%), and these effects also disappeared after 5 hours. After 1 month of combination therapy of sildenafil (25 mg) twice daily and beraprost (20 microg) 3 times daily, the fall in pulmonary artery pressure and pulmonary vascular resistance was sustained (31% in both). Furthermore, the patient had significantly improved her 3-minute walk test and NYHA function class without significant adverse effects at the reported doses. The findings indicate that oral sildenafil is a potent pulmonary vasodilator that appears to act synergistically with oral beraprost to cause sustained pulmonary vasodilatation in a patient with PAH associated with CREST syndrome. FAU - Miwa, Kenji AU - Miwa K AD - Department of Cardiology, Ishikawa Prefectural Central Hospital, Ishikawa, Japan. FAU - Matsubara, Takashi AU - Matsubara T FAU - Uno, Yoshihide AU - Uno Y FAU - Yasuda, Toshihiko AU - Yasuda T FAU - Sakata, Kenji AU - Sakata K FAU - Tsuda, Toyonobu AU - Tsuda T FAU - Kanaya, Honin AU - Kanaya H LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Int Heart J JT - International heart journal JID - 101244240 RN - 0 (Cryoprotective Agents) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - 35E3NJJ4O6 (beraprost) RN - BW9B0ZE037 (Sildenafil Citrate) RN - DCR9Z582X0 (Epoprostenol) RN - EC 3.1.4.35 (3',5'-Cyclic-GMP Phosphodiesterases) SB - IM MH - 3',5'-Cyclic-GMP Phosphodiesterases/antagonists & inhibitors MH - Administration, Oral MH - CREST Syndrome/*complications MH - Cryoprotective Agents MH - Drug Therapy, Combination MH - Epoprostenol/administration & dosage/*analogs & derivatives MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/*drug therapy/etiology/physiopathology MH - Middle Aged MH - Phosphodiesterase Inhibitors/*administration & dosage MH - Piperazines/*administration & dosage MH - Pulmonary Wedge Pressure/drug effects MH - Purines/administration & dosage MH - Sildenafil Citrate MH - Sulfones/*administration & dosage MH - Vasodilator Agents/*administration & dosage EDAT- 2007/06/27 09:00 MHDA- 2007/11/02 09:00 CRDT- 2007/06/27 09:00 PHST- 2007/06/27 09:00 [pubmed] PHST- 2007/11/02 09:00 [medline] PHST- 2007/06/27 09:00 [entrez] AID - JST.JSTAGE/ihj/48.417 [pii] AID - 10.1536/ihj.48.417 [doi] PST - ppublish SO - Int Heart J. 2007 May;48(3):417-22. doi: 10.1536/ihj.48.417. PMID- 16873097 OWN - NLM STAT- MEDLINE DCOM- 20060926 LR - 20191110 IS - 0892-3973 (Print) IS - 0892-3973 (Linking) VI - 28 IP - 2 DP - 2006 TI - Intracellular Th1/Th2 balance of pulmonary CD4(+) T cells in patients with active interstitial pneumonia evaluated by serum KL-6. PG - 295-304 AB - The balance between CD4(+) T helper (Th1) lymphocytes producing interferon-gamma or Interleukin-4 (Th2) in the lungs may vary among diseases and during the progression of interstitial pneumonia (IP). Both idiopathic pulmonary fibrosis (IPF) and collagen vascular diseases (CVD) are associated with IP, but the clinical course and the response to treatment are different. Since Th1 or Th2 modulating drugs have been proven to alter the lymphocyte balance in vitro, it is important to elucidate the Th1/Th2 profile in patients with active IP. Bronchoalveolar lavage (BAL) was performed in patients who had IPF (n = 12) or CVD (n = 12) with IP, as well as in patients who had bronchoectasis and bronchopneumonia (n = 12). The CVD patients had rheumatoid arthritis (n = 6), Sjogren's syndrome (n = 2), dermatomyositis (n = 1), progressive systemic sclerosis (n = 2), and CREST syndrome (n = 1) as the underlying diseases. IP activity was evaluated by measuring serum KL-6, which is a clinically useful indicator for IP. The Th1/ Th2 balance and the CD4(+)/CD8(+) ratio were determined for lymphocytes obtained from BAL by flow cytometric analysis. In IPF patients, the CD4(+)/CD8(+) ratio was lower than in CVD patients. IPF patients showed Th2 dominance and CVD patients showed Th1 dominance when IP was active as evaluated by the serum KL-6 level. These data indicated that the Th1/Th2 balance of CD4(+) T cells in the BAL differs between active IPF and CVD, even though KL-6 is elevated in both diseases. Therefore, the Th1/Th2 profile should be investigated to determine the use of Th1/Th2 modulator therapy for active IP with elevation of KL-6. FAU - Shimizu, Yasuo AU - Shimizu Y AD - Numata National Hospital, Numata City, Gunma, Japan. yasuos@med.gunma-u.ac.jp FAU - Kuwabara, Hidemasa AU - Kuwabara H FAU - Ono, Akihiro AU - Ono A FAU - Higuchi, Seiichi AU - Higuchi S FAU - Hisada, Takeshi AU - Hisada T FAU - Dobashi, Kunio AU - Dobashi K FAU - Utsugi, Mitsuyoshi AU - Utsugi M FAU - Mita, Yoshinori AU - Mita Y FAU - Mori, Masatomo AU - Mori M LA - eng PT - Journal Article PL - England TA - Immunopharmacol Immunotoxicol JT - Immunopharmacology and immunotoxicology JID - 8800150 RN - 0 (Antigens, Neoplasm) RN - 0 (MUC1 protein, human) RN - 0 (Mucin-1) RN - 0 (Mucins) SB - IM MH - Aged MH - Antigens, Neoplasm/*blood MH - Arthritis, Rheumatoid/blood/complications MH - Bronchoalveolar Lavage Fluid MH - CD4-CD8 Ratio MH - CREST Syndrome/blood/complications MH - Dermatomyositis/blood/complications MH - Female MH - Humans MH - Lung Diseases, Interstitial/*blood/etiology MH - Male MH - Middle Aged MH - Mucin-1 MH - Mucins/*blood MH - Scleroderma, Diffuse/blood/complications MH - Sjogren's Syndrome/blood/complications MH - Th1 Cells/*metabolism MH - Th2 Cells/*metabolism EDAT- 2006/07/29 09:00 MHDA- 2006/09/27 09:00 CRDT- 2006/07/29 09:00 PHST- 2006/07/29 09:00 [pubmed] PHST- 2006/09/27 09:00 [medline] PHST- 2006/07/29 09:00 [entrez] AID - KJ1M6305543712W6 [pii] AID - 10.1080/08923970600809389 [doi] PST - ppublish SO - Immunopharmacol Immunotoxicol. 2006;28(2):295-304. doi: 10.1080/08923970600809389. PMID- 38127320 OWN - NLM STAT- MEDLINE DCOM- 20240214 LR - 20240214 IS - 2784-8450 (Electronic) IS - 2784-8671 (Linking) VI - 159 IP - 1 DP - 2024 Feb TI - Skin involvement in CREST syndrome: not only telangiectasias? PG - 75-76 LID - 10.23736/S2784-8671.23.07727-7 [doi] FAU - Marvisi, Maurizio AU - Marvisi M AD - Department of Internal Medicine, Istituto Figlie di San Camillo, Cremona, Italy. FAU - Ramponi, Sara AU - Ramponi S AD - Department of Internal Medicine, Istituto Figlie di San Camillo, Cremona, Italy - sararamponi79@gmail.com. FAU - Felegara, Giovanni AU - Felegara G AD - Department of Pathology, C.D.I., Milan, Italy. FAU - Cecchi, Antonella AU - Cecchi A AD - Dermatology Unit, Istituto Figlie di San Camillo, Cremona, Italy. LA - eng PT - Journal Article DEP - 20231221 PL - Italy TA - Ital J Dermatol Venerol JT - Italian journal of dermatology and venereology JID - 101778002 SB - IM MH - Humans MH - *CREST Syndrome/complications MH - *Telangiectasis/etiology EDAT- 2023/12/21 12:42 MHDA- 2024/02/12 15:44 CRDT- 2023/12/21 11:36 PHST- 2024/02/12 15:44 [medline] PHST- 2023/12/21 12:42 [pubmed] PHST- 2023/12/21 11:36 [entrez] AID - S2784-8671.23.07727-7 [pii] AID - 10.23736/S2784-8671.23.07727-7 [doi] PST - ppublish SO - Ital J Dermatol Venerol. 2024 Feb;159(1):75-76. doi: 10.23736/S2784-8671.23.07727-7. Epub 2023 Dec 21. PMID- 35959840 OWN - NLM STAT- MEDLINE DCOM- 20221103 LR - 20221123 IS - 1479-8301 (Electronic) IS - 1346-3500 (Linking) VI - 22 IP - 6 DP - 2022 Nov TI - A case of limited cutaneous systemic sclerosis with non-Fahr-type calcification in the brain and a review of the literature. PG - 882-885 LID - 10.1111/psyg.12887 [doi] FAU - Ukai, Katsuyuki AU - Ukai K AUID- ORCID: 0000-0002-7649-2238 AD - Department of Psychogeriatrics, Kamiiida Daiichi General Hospital, Nagoya, Japan. AD - Department of Psychiatry, Nagoya University Graduate School of Medicine, Nagoya, Japan. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20220812 PL - England TA - Psychogeriatrics JT - Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society JID - 101230058 SB - IM MH - Humans MH - *Basal Ganglia Diseases MH - *Calcinosis MH - Brain MH - *CREST Syndrome OTO - NOTNLM OT - Fahr's disease OT - brain calcification OT - idiopathic basal ganglia calcification OT - pontine calcification OT - primary familial brain calcification OT - systemic sclerosis EDAT- 2022/08/13 06:00 MHDA- 2022/11/04 06:00 CRDT- 2022/08/12 07:02 PHST- 2022/07/06 00:00 [revised] PHST- 2022/05/13 00:00 [received] PHST- 2022/07/22 00:00 [accepted] PHST- 2022/08/13 06:00 [pubmed] PHST- 2022/11/04 06:00 [medline] PHST- 2022/08/12 07:02 [entrez] AID - 10.1111/psyg.12887 [doi] PST - ppublish SO - Psychogeriatrics. 2022 Nov;22(6):882-885. doi: 10.1111/psyg.12887. Epub 2022 Aug 12. PMID- 35523221 OWN - NLM STAT- MEDLINE DCOM- 20221201 LR - 20221206 IS - 1438-8812 (Electronic) IS - 0013-726X (Linking) VI - 54 IP - 12 DP - 2022 Dec TI - Transesophageal endoscopic ultrasound-guided coil and cyanoacrylate treatment of challenging esophageal varices bleeding associated with CREST syndrome ulcerative esophagitis. PG - E761-E762 LID - 10.1055/a-1814-4140 [doi] FAU - Rivory, Jérôme AU - Rivory J AD - Department of Endoscopy and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Pioche, Mathieu AU - Pioche M AD - Department of Endoscopy and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Dumortier, Jérôme AU - Dumortier J AD - Department of Endoscopy and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Lambin, Thomas AU - Lambin T AD - Department of Endoscopy and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Lupu, Alexandru AU - Lupu A AD - Department of Endoscopy and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Ber, Charles-Eric AU - Ber CE AD - Department of Anesthesiology and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. FAU - Valette, Pierre-Jean AU - Valette PJ AD - Department of Radiology and Hepatogastroenterology, Edouard Herriot Hospital, Lyon, France. LA - eng PT - Journal Article DEP - 20220506 PL - Germany TA - Endoscopy JT - Endoscopy JID - 0215166 RN - 0 (Cyanoacrylates) SB - IM MH - Humans MH - *Esophageal and Gastric Varices/complications/therapy MH - Cyanoacrylates/therapeutic use MH - *CREST Syndrome/therapy MH - *Hemostasis, Endoscopic MH - *Peptic Ulcer MH - Ultrasonography, Interventional MH - *Esophagitis MH - Gastrointestinal Hemorrhage/etiology/therapy MH - Treatment Outcome MH - Recurrence COIS- J. Rivory is a consultant for Cook Medical. EDAT- 2022/05/07 06:00 MHDA- 2022/12/02 06:00 CRDT- 2022/05/06 19:02 PHST- 2022/05/07 06:00 [pubmed] PHST- 2022/12/02 06:00 [medline] PHST- 2022/05/06 19:02 [entrez] AID - 10.1055/a-1814-4140 [doi] PST - ppublish SO - Endoscopy. 2022 Dec;54(12):E761-E762. doi: 10.1055/a-1814-4140. Epub 2022 May 6. PMID- 31764379 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20230927 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 27 IP - 1 DP - 2021 Jan 1 TI - Distal Phalanx Osteolysis and Subcutaneous Calcinosis in CREST Syndrome. PG - e22 LID - 10.1097/RHU.0000000000001210 [doi] FAU - Horino, Taro AU - Horino T AD - From the Department of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University, Kochi. FAU - Ichii, Osamu AU - Ichii O AD - Laboratory of Anatomy, Department of Basic Veterinary Sciences, Faculty of Veterinary Medicine, Hokkaido University, Sapporo, Japan. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - *CREST Syndrome MH - *Calcinosis/diagnostic imaging/etiology MH - Extremities MH - *Finger Phalanges MH - Humans MH - *Osteolysis/diagnostic imaging/etiology COIS- The authors declare no conflict of interest. EDAT- 2019/11/26 06:00 MHDA- 2021/06/24 06:00 CRDT- 2019/11/26 06:00 PHST- 2019/11/26 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] PHST- 2019/11/26 06:00 [entrez] AID - 00124743-202101000-00022 [pii] AID - 10.1097/RHU.0000000000001210 [doi] PST - ppublish SO - J Clin Rheumatol. 2021 Jan 1;27(1):e22. doi: 10.1097/RHU.0000000000001210. PMID- 31249281 OWN - NLM STAT- MEDLINE DCOM- 20200113 LR - 20200113 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 44 IP - 1 DP - 2019 Jan-Mar TI - Dystrophic Calcinosis: Do We Really Know How to Treat It? PG - 86-87 FAU - Nunes, Gabriel Pacifico Seabra AU - Nunes GPS AD - Universidade Nilton Lins. FAU - Souza, Rosana Barros de AU - Souza RB AD - Universidade Federal do Amazonas. FAU - Ribeiro, Sandra Lúcia Euzébio AU - Ribeiro SLE AD - Universidade Federal do Amazonas. LA - eng PT - Case Reports PT - Journal Article TT - Dystrophic Calcinosis: Do We Really Know How to Treat It? PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - CREST Syndrome/complications MH - Calcinosis/diagnostic imaging/*therapy MH - Female MH - Humans MH - Middle Aged MH - Treatment Failure EDAT- 2019/06/30 06:00 MHDA- 2020/01/14 06:00 CRDT- 2019/06/29 06:00 PHST- 2019/06/29 06:00 [entrez] PHST- 2019/06/30 06:00 [pubmed] PHST- 2020/01/14 06:00 [medline] AID - 180289 [pii] PST - ppublish SO - Acta Reumatol Port. 2019 Jan-Mar;44(1):86-87. PMID- 31166211 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20210623 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 26 IP - 6 DP - 2020 Sep TI - Anomalous Inferior Vena Cava in a Patient With Limited Cutaneous Systemic Sclerosis and Primary Biliary Cholangitis. PG - e203 LID - 10.1097/RHU.0000000000001073 [doi] FAU - Horino, Taro AU - Horino T AD - From the Department of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University, Kochi. FAU - Ichii, Osamu AU - Ichii O AD - Laboratory of Anatomy, Department of Biomedical Sciences, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo, Japan. FAU - Terada, Yoshio AU - Terada Y AD - From the Department of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University, Kochi. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 SB - IM MH - *CREST Syndrome MH - Humans MH - *Liver Cirrhosis, Biliary/complications/diagnosis MH - Vena Cava, Inferior/diagnostic imaging EDAT- 2019/06/06 06:00 MHDA- 2021/06/24 06:00 CRDT- 2019/06/06 06:00 PHST- 2019/06/06 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] PHST- 2019/06/06 06:00 [entrez] AID - 00124743-202009000-00043 [pii] AID - 10.1097/RHU.0000000000001073 [doi] PST - ppublish SO - J Clin Rheumatol. 2020 Sep;26(6):e203. doi: 10.1097/RHU.0000000000001073. PMID- 29447644 OWN - NLM STAT- MEDLINE DCOM- 20180410 LR - 20180410 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 23 IP - 11 DP - 2017 Nov 15 TI - Cutaneous metastasis to the scalp as the primary presentation of colorectal adenocarcinoma. LID - 13030/qt31p698nz [pii] AB - Eruptaneous metastasis is an uncommon presentation of colorectal adenocarcinoma that can occur years after diagnosis of the primary cancer or manifest as the first sign of malignancy. It is essential to diagnose these metastases immediately, as this late-stage development carries a poor prognosis. The scalp is one of the less common sites for skin metastases and nodules may be mistaken for benign entities. In this case report, we report on the case of a 61-year-old woman with CREST syndrome who presented with a cutaneous metastasis to the scalp as the first sign ofcolorectal adenocarcinoma. FAU - Shah, Sarita R AU - Shah SR AD - Baylor College of Medicine, Department of Dermatology, Houston, Texas. FAU - Applebaum, Danielle S AU - Applebaum DS FAU - Potenziani, Silvia AU - Potenziani S FAU - Huttenbach, Yve T AU - Huttenbach YT FAU - Wolf, John AU - Wolf J FAU - Orengo, Ida F AU - Orengo IF LA - eng PT - Case Reports PT - Journal Article DEP - 20171115 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 SB - IM MH - Adenocarcinoma/*secondary MH - Biopsy MH - CREST Syndrome/complications MH - Colorectal Neoplasms/*pathology MH - Female MH - Head and Neck Neoplasms/*secondary MH - Humans MH - Middle Aged MH - Prognosis MH - Scalp/*pathology MH - Skin Neoplasms/*secondary EDAT- 2018/02/16 06:00 MHDA- 2018/04/11 06:00 CRDT- 2018/02/16 06:00 PHST- 2017/12/11 00:00 [received] PHST- 2017/12/11 00:00 [accepted] PHST- 2018/02/16 06:00 [entrez] PHST- 2018/02/16 06:00 [pubmed] PHST- 2018/04/11 06:00 [medline] AID - 13030/qt31p698nz [pii] PST - epublish SO - Dermatol Online J. 2017 Nov 15;23(11):13030/qt31p698nz. PMID- 29040484 OWN - NLM STAT- MEDLINE DCOM- 20180615 LR - 20180615 IS - 1442-2050 (Electronic) IS - 1120-8694 (Linking) VI - 11 IP - 1 DP - 2017 Nov 1 TI - Esophagocardioplasty, vagotomy-antrectomy and Roux-en-Y gastrojejunostomy: indication in cases with severe esophageal motor disfunction. PG - 58-61 LID - 10.1093/dote/11.1.58 [doi] AB - Almost 10% of patients with Crest syndrome associated with severe gastroesophageal reflux and 5-10% of patients with failed cardiomyotomy for achalasia present with cardial or distal esophageal organic stricture. Some of these cases are poor risk patients for surgery and therefore the surgeon must offer a safe procedure with low morbimortality, keeping in mind the pathophysiological motor pattern of these patients.In order to treat the stricture to improve the esophageal transit we treated patients with esophagocardioplasty associated with vagotomy-antrectomy and Roux-en-Y gastrojejunostomy, thereby avoiding the potential acid or biliary reflux in poor risk patients in whom esophagectomy would be a very deleterious procedure. All four patients had a good postoperative evolution and late control demonstrated good esophagogastric transit with no postoperative esophagitis. CI - © 1998 International Society for Diseases of the Esophagus/Harcourt Brace & Co. Ltd. FAU - Braghetto, I AU - Braghetto I AD - Department of Surgery, University of Chile, Santiago, Chile. FAU - Korn, O AU - Korn O AD - Department of Surgery, University of Chile, Santiago, Chile. FAU - Csendes, A AU - Csendes A AD - Department of Surgery, University of Chile, Santiago, Chile. FAU - Frias, J C AU - Frias JC AD - Department of Surgery, University of Chile, Santiago, Chile. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Dis Esophagus JT - Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus JID - 8809160 SB - IM MH - Adult MH - Aged MH - Anastomosis, Roux-en-Y MH - CREST Syndrome/surgery MH - Cardia/surgery MH - Esophageal Motility Disorders/*surgery MH - Esophageal Stenosis/*surgery MH - Esophagoplasty MH - Female MH - Gastric Bypass MH - Humans MH - Male MH - Middle Aged MH - Patient Selection MH - Pyloric Antrum/surgery MH - Vagotomy EDAT- 2017/10/19 06:00 MHDA- 2018/06/16 06:00 CRDT- 2017/10/18 06:00 PHST- 2017/10/18 06:00 [entrez] PHST- 2017/10/19 06:00 [pubmed] PHST- 2018/06/16 06:00 [medline] AID - 4371471 [pii] AID - 10.1093/dote/11.1.58 [doi] PST - ppublish SO - Dis Esophagus. 2017 Nov 1;11(1):58-61. doi: 10.1093/dote/11.1.58. PMID- 28602872 OWN - NLM STAT- MEDLINE DCOM- 20181123 LR - 20181123 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 130 IP - 11 DP - 2017 Nov TI - Coexistent Primary Biliary Cholangitis with CREST Syndrome (Reynolds Syndrome). PG - e501-e502 LID - S0002-9343(17)30597-1 [pii] LID - 10.1016/j.amjmed.2017.05.019 [doi] FAU - Kiyani, Amirali AU - Kiyani A AD - Department of Medicine, Maricopa Medical Center, Phoenix, Ariz. Electronic address: amirali.kiyani@mihs.org. FAU - Ursu, Shannon AU - Ursu S AD - Department of Medicine, Maricopa Medical Center, Phoenix, Ariz. LA - eng PT - Case Reports PT - Journal Article DEP - 20170608 PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 SB - IM MH - Biliary Tract Diseases/*complications/*diagnosis/pathology MH - CREST Syndrome/*complications/*diagnosis MH - Cholangitis/*complications/*diagnosis/pathology MH - Female MH - Humans MH - Middle Aged EDAT- 2017/06/13 06:00 MHDA- 2018/11/24 06:00 CRDT- 2017/06/13 06:00 PHST- 2017/05/02 00:00 [received] PHST- 2017/05/16 00:00 [revised] PHST- 2017/05/17 00:00 [accepted] PHST- 2017/06/13 06:00 [pubmed] PHST- 2018/11/24 06:00 [medline] PHST- 2017/06/13 06:00 [entrez] AID - S0002-9343(17)30597-1 [pii] AID - 10.1016/j.amjmed.2017.05.019 [doi] PST - ppublish SO - Am J Med. 2017 Nov;130(11):e501-e502. doi: 10.1016/j.amjmed.2017.05.019. Epub 2017 Jun 8. PMID- 25807102 OWN - NLM STAT- MEDLINE DCOM- 20151215 LR - 20150326 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 21 IP - 3 DP - 2015 Apr TI - Osteonecrosis of multiple joints in a patient with limited scleroderma/CREST syndrome. PG - 169-70 LID - 10.1097/RHU.0000000000000231 [doi] FAU - Desai, Ketan AU - Desai K AD - VA Medical Center, Wilkes Barre, Pennsylvania ketan.desai@va.gov. FAU - Mian, Nabeela Z AU - Mian NZ LA - eng PT - Case Reports PT - Letter PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/adverse effects/therapeutic use MH - Adult MH - Ankle Joint/pathology MH - CREST Syndrome/*complications/drug therapy MH - Hip Joint/pathology MH - Humans MH - Knee Joint/pathology MH - Male MH - Osteonecrosis/chemically induced/*diagnosis/*pathology MH - Risk Factors MH - Scleroderma, Limited/*complications/drug therapy MH - Treatment Outcome EDAT- 2015/03/26 06:00 MHDA- 2015/12/17 06:00 CRDT- 2015/03/26 06:00 PHST- 2015/03/26 06:00 [entrez] PHST- 2015/03/26 06:00 [pubmed] PHST- 2015/12/17 06:00 [medline] AID - 00124743-201504000-00014 [pii] AID - 10.1097/RHU.0000000000000231 [doi] PST - ppublish SO - J Clin Rheumatol. 2015 Apr;21(3):169-70. doi: 10.1097/RHU.0000000000000231. PMID- 23897225 OWN - NLM STAT- MEDLINE DCOM- 20140102 LR - 20211021 IS - 1529-0131 (Electronic) IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 65 IP - 11 DP - 2013 Nov TI - Skin gene expression correlates of severity of interstitial lung disease in systemic sclerosis. PG - 2917-27 LID - 10.1002/art.38101 [doi] AB - OBJECTIVE: We undertook this hypothesis-generating study to identify skin transcripts correlating with severity of interstitial lung disease (ILD) in systemic sclerosis (SSc). METHODS: Skin biopsy samples from 59 patients enrolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort or an open-label imatinib study (baseline visit) were examined by global gene expression analysis using Illumina HT-12 arrays. Skin transcripts correlating with concomitantly obtained forced vital capacity (FVC) values and the modified Rodnan skin thickness score (MRSS) were identified by quantitative trait analysis. Also, immunofluorescence staining for selected transcripts was performed in affected skin and lung tissue. Plasma levels of CCL2, soluble SELP, and soluble P-selectin glycoprotein ligand 1 (sPSGL-1) were examined in all patients enrolled in the GENISOS cohort (n = 266). RESULTS: Eighty-two skin transcripts correlated significantly with FVC. This gene list distinguished patients with more severe ILD (FVC <70% predicted) in unsupervised hierarchical clustering analysis (P < 0.001). These genes included SELP, CCL2, and matrix metalloproteinase 3, which are involved in extravasation and adhesion of inflammatory cells. Among the FVC correlates, 8 genes (CCL2, HAPLN3, GPR4, ADCYAP1, WARS, CDC25B, PLP1, and STXBP6) also correlated with the MRSS. Immunofluorescence staining revealed that SELP and CCL2 were also overexpressed in affected skin and lung tissue from SSc patients compared to those from controls. Plasma levels of CCL2 and sPSGL-1 correlated with concomitantly obtained FVC values (r = -0.22, P = 0.001 and r = 0.17, P = 0.015, respectively). This relationship was independent of potential confounders (age, sex, ethnicity, smoking status, anti-topoisomerase I positivity, treatment with immunosuppressive agents, MRSS, disease type, and disease duration). CONCLUSION: A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD. CI - Copyright © 2013 by the American College of Rheumatology. FAU - Assassi, Shervin AU - Assassi S AD - University of Texas Health Science Center at, Houston. FAU - Wu, Minghua AU - Wu M FAU - Tan, Filemon K AU - Tan FK FAU - Chang, Jeffrey AU - Chang J FAU - Graham, Tiffany A AU - Graham TA FAU - Furst, Daniel E AU - Furst DE FAU - Khanna, Dinesh AU - Khanna D FAU - Charles, Julio AU - Charles J FAU - Ferguson, Emma C AU - Ferguson EC FAU - Feghali-Bostwick, Carol AU - Feghali-Bostwick C FAU - Mayes, Maureen D AU - Mayes MD LA - eng GR - K24-AR-063120/AR/NIAMS NIH HHS/United States GR - K23-AR-061436/AR/NIAMS NIH HHS/United States GR - P50 AR054144/AR/NIAMS NIH HHS/United States GR - P50-AR-054144/AR/NIAMS NIH HHS/United States GR - K23 AR061436/AR/NIAMS NIH HHS/United States GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - P30-AR-061271/AR/NIAMS NIH HHS/United States GR - P30 AR061271/AR/NIAMS NIH HHS/United States GR - U01-AI-09090/AI/NIAID NIH HHS/United States GR - UL1 TR000371/TR/NCATS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antineoplastic Agents) RN - 0 (Benzamides) RN - 0 (Piperazines) RN - 0 (Pyrimidines) RN - 8A1O1M485B (Imatinib Mesylate) SB - IM MH - Adult MH - Antineoplastic Agents/therapeutic use MH - Benzamides/therapeutic use MH - Biopsy MH - CREST Syndrome/drug therapy/genetics/pathology MH - Cell Adhesion/physiology MH - Female MH - Humans MH - Imatinib Mesylate MH - Lung Diseases, Interstitial/drug therapy/*genetics/pathology MH - Male MH - Piperazines/therapeutic use MH - Pyrimidines/therapeutic use MH - Scleroderma, Systemic/drug therapy/*genetics/pathology MH - *Severity of Illness Index MH - Skin Physiological Phenomena/*genetics MH - *Transcriptome PMC - PMC3898704 MID - NIHMS541775 EDAT- 2013/07/31 06:00 MHDA- 2014/01/03 06:00 PMCR- 2014/11/01 CRDT- 2013/07/31 06:00 PHST- 2012/10/04 00:00 [received] PHST- 2013/07/18 00:00 [accepted] PHST- 2013/07/31 06:00 [entrez] PHST- 2013/07/31 06:00 [pubmed] PHST- 2014/01/03 06:00 [medline] PHST- 2014/11/01 00:00 [pmc-release] AID - 10.1002/art.38101 [doi] PST - ppublish SO - Arthritis Rheum. 2013 Nov;65(11):2917-27. doi: 10.1002/art.38101. PMID- 23494102 OWN - NLM STAT- MEDLINE DCOM- 20140926 LR - 20211021 IS - 1435-604X (Electronic) IS - 0268-8921 (Linking) VI - 29 IP - 1 DP - 2014 Jan TI - Telangiectasis in CREST syndrome and systemic sclerosis: correlation of clinical and pathological features with response to pulsed dye laser treatment. PG - 137-40 LID - 10.1007/s10103-013-1298-1 [doi] AB - Telangiectasia are cardinal features of systemic sclerosis (SS) and calcinosis, Raynaud's syndrome, esophageal motility, sclerodactyly, telangiectasias (CREST) syndrome. The etiology of telangiectasia in these syndromes is unknown, but vascular dysfunction has been proposed. However, the telangiectasia of CREST have anecdotally been considered relatively resistant to pulse dye laser (PDL), the treatment of choice for classic telangiectasia. The study was designed to test whether SS/CREST telangiectasia require more treatments than sporadic telangiectasia and to identify clinical and histological features that could explain such an effect. Nineteen skin biopsies from patients with SS or CREST and 10 control biopsies were examined and compared for features that may predict a differential response to PDL. Sixteen cases of SS or CREST treated with PDL between 1997 and 2007 were evaluated and response to treatment was compared with 20 patients with sporadic telangiectasis. Relative to normal skin, CREST/scleroderma telangiectasia exhibited thickened vessels in 17 out of 19 sections and thickened collagen fibers in the reticular or deep dermis in all sections. The number of treatments required to clear SS/CREST telangiectasia was approximately twofold higher. SS/CREST telangiectasia are more resistant to PDL but can be effectively cleared with more treatments. FAU - Halachmi, Shlomit AU - Halachmi S AD - Laser Unit, Department of Dermatology, Rabin Medical Center, Petach Tikva, Israel, drshomithalachmi@gmail.com. FAU - Gabari, Osama AU - Gabari O FAU - Cohen, Sarit AU - Cohen S FAU - Koren, Romelia AU - Koren R FAU - Amitai, Dan Ben AU - Amitai DB FAU - Lapidoth, Moshe AU - Lapidoth M LA - eng PT - Journal Article DEP - 20130314 PL - England TA - Lasers Med Sci JT - Lasers in medical science JID - 8611515 SB - IM MH - Adult MH - Aged MH - CREST Syndrome/complications/pathology/*surgery MH - Case-Control Studies MH - Endothelium, Vascular/pathology MH - Female MH - Humans MH - Lasers, Dye/*therapeutic use MH - Male MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/complications/pathology/*surgery MH - Telangiectasis/complications/pathology/*surgery MH - Treatment Outcome MH - Young Adult EDAT- 2013/03/16 06:00 MHDA- 2014/09/27 06:00 CRDT- 2013/03/16 06:00 PHST- 2013/01/06 00:00 [received] PHST- 2013/02/28 00:00 [accepted] PHST- 2013/03/16 06:00 [entrez] PHST- 2013/03/16 06:00 [pubmed] PHST- 2014/09/27 06:00 [medline] AID - 10.1007/s10103-013-1298-1 [doi] PST - ppublish SO - Lasers Med Sci. 2014 Jan;29(1):137-40. doi: 10.1007/s10103-013-1298-1. Epub 2013 Mar 14. PMID- 23070133 OWN - NLM STAT- MEDLINE DCOM- 20140102 LR - 20130423 IS - 1941-2444 (Electronic) IS - 0148-6071 (Linking) VI - 37 IP - 3 DP - 2013 May-Jun TI - Anemia and leukopenia in a long-term parenteral nutrition patient during a shortage of parenteral trace element products in the United States. PG - 425-9 LID - 10.1177/0148607112463942 [doi] AB - Recently, drug shortages in the United States have affected multiple components of the parenteral nutrition (PN) solution. A 62-year-old patient with systemic sclerosis who was dependent on home PN due to intestinal dysmotility developed anemia and leukopenia approximately 4 months after parenteral copper was withheld from her PN solution due to drug shortages. The patient was not able to tolerate a sufficient amount of oral multivitamins with trace elements due to severe dysphagia. Her serum copper and ceruloplasmin concentrations were undetectable, confirming the diagnosis of severe copper deficiency. The hematological abnormalities promptly resolved with copper supplementation. This report emphasizes the importance of close monitoring for nutrient deficiencies during drug shortages and supplementing with oral or enteral nutrition when feasible, particularly in high-risk patients such as those with intestinal malabsorption or short bowel syndrome who are dependent on long-term PN. FAU - Pramyothin, Pornpoj AU - Pramyothin P AD - Section of Endocrinology, Diabetes, and Nutrition, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA. FAU - Kim, Dong Wook AU - Kim DW FAU - Young, Lorraine S AU - Young LS FAU - Wichansawakun, Sanit AU - Wichansawakun S FAU - Apovian, Caroline M AU - Apovian CM LA - eng PT - Case Reports PT - Journal Article DEP - 20121015 PL - United States TA - JPEN J Parenter Enteral Nutr JT - JPEN. Journal of parenteral and enteral nutrition JID - 7804134 RN - 0 (Micronutrients) RN - 0 (Parenteral Nutrition Solutions) RN - 0 (Trace Elements) RN - 789U1901C5 (Copper) RN - EC 1.16.3.1 (Ceruloplasmin) SB - IM MH - Anemia/*etiology MH - CREST Syndrome/*complications/therapy MH - Ceruloplasmin/analysis MH - Copper/blood/deficiency MH - Deglutition Disorders/physiopathology/therapy MH - *Dietary Supplements MH - Female MH - Humans MH - Intestinal Absorption MH - Leukopenia/*etiology MH - Micronutrients/administration & dosage/deficiency MH - Middle Aged MH - Parenteral Nutrition MH - Parenteral Nutrition Solutions/*supply & distribution MH - Trace Elements/administration & dosage/blood/*deficiency MH - Treatment Outcome MH - United States EDAT- 2012/10/17 06:00 MHDA- 2014/01/03 06:00 CRDT- 2012/10/17 06:00 PHST- 2012/10/17 06:00 [entrez] PHST- 2012/10/17 06:00 [pubmed] PHST- 2014/01/03 06:00 [medline] AID - 0148607112463942 [pii] AID - 10.1177/0148607112463942 [doi] PST - ppublish SO - JPEN J Parenter Enteral Nutr. 2013 May-Jun;37(3):425-9. doi: 10.1177/0148607112463942. Epub 2012 Oct 15. PMID- 22198488 OWN - NLM STAT- MEDLINE DCOM- 20120503 LR - 20161125 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 18 IP - 1 DP - 2012 Jan TI - Calcinosis universalis in a patient with overlap of scleroderma/dermatomyositis. PG - 57 LID - 10.1097/RHU.0b013e31823ee767 [doi] FAU - Shenavandeh, Saeedeh AU - Shenavandeh S AD - Division of Rheumatology, Department of Internal Medicine, Medical University of Shiraz, Nemazee Hospital, Shiraz, Iran. shenavande@sums.ac.ir LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Anti-Bacterial Agents) RN - 0 (Glucocorticoids) RN - 6Q205EH1VU (Vancomycin) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Anti-Bacterial Agents/therapeutic use MH - CREST Syndrome/*complications/drug therapy MH - Calcinosis/*diagnostic imaging/etiology/microbiology MH - Dermatomyositis/*complications/drug therapy MH - Female MH - Fever/drug therapy/etiology MH - Glucocorticoids/therapeutic use MH - Humans MH - Middle Aged MH - Prednisolone/therapeutic use MH - Radiography MH - Staphylococcal Infections/*diagnosis/drug therapy/microbiology MH - Staphylococcus aureus/*isolation & purification MH - Vancomycin/therapeutic use EDAT- 2011/12/27 06:00 MHDA- 2012/05/04 06:00 CRDT- 2011/12/27 06:00 PHST- 2011/12/27 06:00 [entrez] PHST- 2011/12/27 06:00 [pubmed] PHST- 2012/05/04 06:00 [medline] AID - 00124743-201201000-00017 [pii] AID - 10.1097/RHU.0b013e31823ee767 [doi] PST - ppublish SO - J Clin Rheumatol. 2012 Jan;18(1):57. doi: 10.1097/RHU.0b013e31823ee767. PMID- 21982887 OWN - NLM STAT- MEDLINE DCOM- 20120206 LR - 20191112 IS - 2589-0646 (Electronic) IS - 2589-0646 (Linking) VI - 4 IP - 3 DP - 2011 TI - Small lymphocytic lymphoma in a patient with CREST syndrome. PG - 132-5 AB - We report a case of a 61-year-old man with a history of CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who presented for evaluation of thrombocytopenia. He had evident cervical adenopathy and lymph node biopsy showed small lymphocytic lymphoma (SLL) with evident systemic adenopathy and bone marrow involvement. The patient achieved a complete remission with FCR (fludarabine/cyclophosphamide/rituximab) chemotherapy. About 30 cases of lymphomas are reported in the literature in association with systemic sclerosis. To our knowledge, there are no reports of a small lymphocytic lymphoma (SLL) in association with limited cutaneous systemic sclerosis with classic features of the CREST syndrome. FAU - William, Basem M AU - William BM AD - University of Nebraska Medical Center and Omaha Veterans Affairs Medical Center, Omaha, Nebraska 68198-7680, USA. bwillia@unmc.edu FAU - Harbert, Tracey AU - Harbert T FAU - Ganti, Apar K AU - Ganti AK FAU - Bierman, Philip J AU - Bierman PJ LA - eng PT - Case Reports PT - Journal Article PL - India TA - Hematol Oncol Stem Cell Ther JT - Hematology/oncology and stem cell therapy JID - 101468532 SB - IM MH - Biopsy MH - Bone Marrow/pathology MH - CREST Syndrome/*complications/diagnostic imaging/pathology MH - Cell Aggregation MH - Humans MH - Leukemia, Lymphocytic, Chronic, B-Cell/*complications/diagnostic imaging/pathology MH - Lymph Nodes/pathology/surgery MH - Lymphocytes/pathology MH - Male MH - Middle Aged MH - Tomography, X-Ray Computed EDAT- 2011/10/11 06:00 MHDA- 2012/02/07 06:00 CRDT- 2011/10/11 06:00 PHST- 2011/10/11 06:00 [entrez] PHST- 2011/10/11 06:00 [pubmed] PHST- 2012/02/07 06:00 [medline] AID - 10.5144/1658-3876.2011.132 [pii] AID - 10.5144/1658-3876.2011.132 [doi] PST - ppublish SO - Hematol Oncol Stem Cell Ther. 2011;4(3):132-5. doi: 10.5144/1658-3876.2011.132. PMID- 20824416 OWN - NLM STAT- MEDLINE DCOM- 20110218 LR - 20211020 IS - 1615-6722 (Electronic) IS - 0723-5003 (Linking) VI - 105 IP - 8 DP - 2010 Aug TI - [Digital ulcers in systemic sclerosis--an interdisciplinary challenge]. PG - 578-81 LID - 10.1007/s00063-010-1096-5 [doi] AB - Digital ulcers in systemic sclerosis are painful ischemic necrotic lesions of the acra. Optimal treatment consists of conventional wound management and medication: iloprost infusions promote primary healing of the ulcers, while the dual endothelin receptor antagonist bosentan is used for secondary prophylaxis of new ulcers. The described case illustrates the essential interdisciplinary collaboration for optimal management of these patients. FAU - Wiesent, Franziska AU - Wiesent F AD - III. Medizinische Klinik, Funktionsbereich Rheumatologie/Osteologie, Zentralklinikum Augsburg, Augsburg, Germany. fwiesent@hotmail.de FAU - Weinerth, Jutta AU - Weinerth J LA - ger PT - Case Reports PT - Journal Article TT - Digitale Ulzerationen bei Sklerodermie--eine interdisziplinäre Herausforderung. DEP - 20100908 PL - Germany TA - Med Klin (Munich) JT - Medizinische Klinik (Munich, Germany : 1983) JID - 8303501 RN - 0 (Antihypertensive Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) RN - Q326023R30 (Bosentan) SB - IM MH - Angiography MH - Antihypertensive Agents/therapeutic use MH - Arm/blood supply MH - Bosentan MH - CREST Syndrome/*diagnosis/drug therapy MH - *Cooperative Behavior MH - Endothelin Receptor Antagonists MH - Fatal Outcome MH - *Fingers MH - Follow-Up Studies MH - Hand Dermatoses/*diagnosis/drug therapy MH - Humans MH - Iloprost/therapeutic use MH - *Interdisciplinary Communication MH - Leg/blood supply MH - Male MH - Middle Aged MH - Scleroderma, Limited/*diagnosis/drug therapy MH - Skin Ulcer/*diagnosis/drug therapy MH - Sulfonamides/therapeutic use MH - Vasodilator Agents/therapeutic use EDAT- 2010/09/09 06:00 MHDA- 2011/02/22 06:00 CRDT- 2010/09/09 06:00 PHST- 2010/01/03 00:00 [received] PHST- 2010/05/26 00:00 [accepted] PHST- 2010/09/09 06:00 [entrez] PHST- 2010/09/09 06:00 [pubmed] PHST- 2011/02/22 06:00 [medline] AID - 10.1007/s00063-010-1096-5 [doi] PST - ppublish SO - Med Klin (Munich). 2010 Aug;105(8):578-81. doi: 10.1007/s00063-010-1096-5. Epub 2010 Sep 8. PMID- 20734547 OWN - NLM STAT- MEDLINE DCOM- 20101222 LR - 20100824 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 35 IP - 2 DP - 2010 Apr-Jun TI - [Dystrophic calcinosis of the vulva in a patient with CREST syndrome]. PG - 264-6 FAU - Almeida, Maria do Socorro Teixeira Moreira AU - Almeida Mdo S AD - Universidade Federal do Piauí, Hospital Getúlio Vargas - Teresina - Piauí. esteios@uol.com.br FAU - Carvalho, Luciano Lima AU - Carvalho LL FAU - Arcoverde, Josué Costa AU - Arcoverde JC FAU - Capucho, Ludmilla de Figueirêdo do Vale AU - Capucho Lde F FAU - Monteiro, Camilla Tapety e Silva do Rego AU - Monteiro CT FAU - Fontenelle, Gustavo Eduardo Pires AU - Fontenelle GE LA - por PT - Case Reports PT - Journal Article TT - Calcinose distrófica de vulva em paciente com síndrome Crest. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adult MH - CREST Syndrome/*complications MH - Calcinosis/*etiology MH - Female MH - Humans MH - Vulvar Diseases/*etiology EDAT- 2010/08/26 06:00 MHDA- 2010/12/24 06:00 CRDT- 2010/08/26 06:00 PHST- 2010/08/26 06:00 [entrez] PHST- 2010/08/26 06:00 [pubmed] PHST- 2010/12/24 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2010 Apr-Jun;35(2):264-6. PMID- 20485183 OWN - NLM STAT- MEDLINE DCOM- 20101116 LR - 20100805 IS - 1473-5687 (Electronic) IS - 0954-691X (Linking) VI - 22 IP - 9 DP - 2010 Sep TI - Gastric variceal bleeding uncovering a rare association of CREST syndrome, primary biliary cirrhosis, nodular regenerative hyperplasia and pulmonary hypertension. PG - 1145-8 LID - 10.1097/MEG.0b013e32833ab83a [doi] AB - A 73-year-old woman was admitted after the first upper gastric tract haemorrhage due to gastric variceal bleeding. A CREST syndrome associated with Hashimoto's thyroiditis, Gougerot-Sjögren syndrome, cryoglobulinaemia and complicated with severe pulmonary hypertension was diagnosed. Liver histology found precirrhotic lesions of primary biliary cirrhosis (PBC) and nodular regenerative hyperplasia (NRH). Collagen diseases are often associated with liver test abnormalities and liver disease usually associated with CREST syndrome is PBC. NRH has been found in association with collagen diseases but also with haematological diseases or drugs or with autoimmune diseases, such as PBC. This case shows the association of PBC and NRH with porto pulmonary hypertension in CREST syndrome. FAU - Riviere, Etienne AU - Riviere E AD - Service d'Hépato-Gastroentérologie, Hôpital du Haut-Lévêque, 33604 Pessac cedex, France. FAU - Vergniol, Julien AU - Vergniol J FAU - Reffet, Armel AU - Reffet A FAU - Lippa, Nicolas AU - Lippa N FAU - Le Bail, Brigitte AU - Le Bail B FAU - de Ledinghen, Victor AU - de Ledinghen V LA - eng PT - Case Reports PT - Journal Article PL - England TA - Eur J Gastroenterol Hepatol JT - European journal of gastroenterology & hepatology JID - 9000874 SB - IM MH - Aged MH - Biopsy MH - CREST Syndrome/*complications MH - Esophageal and Gastric Varices/*complications MH - Female MH - Focal Nodular Hyperplasia/*complications/pathology MH - Gastrointestinal Hemorrhage/*etiology MH - Humans MH - Hyperplasia MH - Hypertension, Pulmonary/*complications MH - Liver Cirrhosis, Biliary/*complications/pathology EDAT- 2010/05/21 06:00 MHDA- 2010/11/17 06:00 CRDT- 2010/05/21 06:00 PHST- 2010/05/21 06:00 [entrez] PHST- 2010/05/21 06:00 [pubmed] PHST- 2010/11/17 06:00 [medline] AID - 10.1097/MEG.0b013e32833ab83a [doi] PST - ppublish SO - Eur J Gastroenterol Hepatol. 2010 Sep;22(9):1145-8. doi: 10.1097/MEG.0b013e32833ab83a. PMID- 20189525 OWN - NLM STAT- MEDLINE DCOM- 20100527 LR - 20100301 IS - 1097-6779 (Electronic) IS - 0016-5107 (Linking) VI - 71 IP - 3 DP - 2010 Mar TI - Complete double-balloon enteroscopy: from A 2 E. PG - 623-4; discussion 624 LID - 10.1016/j.gie.2009.10.057 [doi] FAU - McCabe, Evin J AU - McCabe EJ AD - Division of Gastroenterology and Center for Advanced Therapeutic Endoscopy, Lenox Hill Hospital, New York, New York, USA. FAU - Haber, Gregory B AU - Haber GB FAU - Ali, Aman AU - Ali A FAU - Nourani, Sam M AU - Nourani SM LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Gastrointest Endosc JT - Gastrointestinal endoscopy JID - 0010505 SB - IM MH - CREST Syndrome/*diagnosis/*therapy MH - Endoscopes, Gastrointestinal MH - Endoscopy, Gastrointestinal/*methods MH - Humans MH - Male MH - Middle Aged EDAT- 2010/03/02 06:00 MHDA- 2010/05/28 06:00 CRDT- 2010/03/02 06:00 PHST- 2009/01/15 00:00 [received] PHST- 2009/10/30 00:00 [accepted] PHST- 2010/03/02 06:00 [entrez] PHST- 2010/03/02 06:00 [pubmed] PHST- 2010/05/28 06:00 [medline] AID - S0016-5107(09)02711-4 [pii] AID - 10.1016/j.gie.2009.10.057 [doi] PST - ppublish SO - Gastrointest Endosc. 2010 Mar;71(3):623-4; discussion 624. doi: 10.1016/j.gie.2009.10.057. PMID- 20074500 OWN - NLM STAT- MEDLINE DCOM- 20100615 LR - 20100115 IS - 1533-7294 (Electronic) IS - 0094-3509 (Linking) VI - 59 IP - 1 DP - 2010 Jan TI - Elbow nodules. PG - 35-9 FAU - Diaz, Lucia AU - Diaz L AD - University of Texas Health Center at San Antonio, San Antonio, TX 78229, USA. Diazl3@uthscsa.edu FAU - Usatine, Richard P AU - Usatine RP LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Fam Pract JT - The Journal of family practice JID - 7502590 SB - IM MH - Adult MH - CREST Syndrome/*diagnosis MH - Diagnosis, Differential MH - *Elbow MH - Female MH - Humans MH - Skin/*pathology EDAT- 2010/01/16 06:00 MHDA- 2010/06/16 06:00 CRDT- 2010/01/16 06:00 PHST- 2010/01/16 06:00 [entrez] PHST- 2010/01/16 06:00 [pubmed] PHST- 2010/06/16 06:00 [medline] AID - jfp_5901e [pii] PST - ppublish SO - J Fam Pract. 2010 Jan;59(1):35-9. PMID- 19748010 OWN - NLM STAT- MEDLINE DCOM- 20091125 LR - 20161125 IS - 1365-229X (Electronic) IS - 0009-9260 (Linking) VI - 64 IP - 10 DP - 2009 Oct TI - Invasive uraemic calcinosis of the hip. PG - 1035-6 LID - 10.1016/j.crad.2008.12.016 [doi] FAU - Gibson, C AU - Gibson C AD - Sir Charles Gairdner Hospital, Shenton Park, Western Australia. gibbo56@hotmail.com FAU - Robbins, P AU - Robbins P FAU - Guy, S AU - Guy S LA - eng PT - Case Reports PT - Journal Article DEP - 20090906 PL - England TA - Clin Radiol JT - Clinical radiology JID - 1306016 SB - IM MH - Bone Diseases/*diagnostic imaging/etiology MH - CREST Syndrome/complications MH - Calcinosis/*diagnostic imaging/etiology MH - Fatal Outcome MH - Female MH - Hip Joint/*diagnostic imaging MH - Humans MH - Hyperkalemia/complications MH - Kidney Failure, Chronic/complications MH - Middle Aged MH - Tomography, X-Ray Computed/methods MH - Uremia/complications EDAT- 2009/09/15 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/09/15 06:00 PHST- 2008/10/07 00:00 [received] PHST- 2008/12/02 00:00 [revised] PHST- 2008/12/10 00:00 [accepted] PHST- 2009/09/15 06:00 [entrez] PHST- 2009/09/15 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - S0009-9260(09)00137-8 [pii] AID - 10.1016/j.crad.2008.12.016 [doi] PST - ppublish SO - Clin Radiol. 2009 Oct;64(10):1035-6. doi: 10.1016/j.crad.2008.12.016. Epub 2009 Sep 6. PMID- 19410347 OWN - NLM STAT- MEDLINE DCOM- 20091116 LR - 20191210 IS - 1579-2129 (Electronic) IS - 0300-2896 (Linking) VI - 45 IP - 8 DP - 2009 Aug TI - [Double-lung transplantation in 15 patients with pulmonary hypertension]. PG - 366-70 LID - 10.1016/j.arbres.2008.11.007 [doi] AB - BACKGROUND: Pulmonary hypertension is a serious disease that, in its terminal phase, requires lung transplantation. PATIENTS AND METHODS: A retrospective study was undertaken of 15 patients with pulmonary hypertension who underwent lung transplantation between 1994 and 2004. Clinical data recorded before the procedure and during follow-up were reviewed. RESULTS: Pulmonary hypertension was reported as idiopathic in 8 patients (53%) and related to consumption of toxic oil in 2. The remaining causes were documented as chronic peripheral pulmonary embolism, histiocytosis X, venoocclusive disease, scleroderma, and simple corrected congenital heart defect in 1 patient each. The mean values of the hemodynamic variables were 100, 50, and 67 mm Hg for systolic, diastolic, and mean pulmonary artery pressure, respectively; 2.63 L/min for cardiac output; and 20.9 Wood units for total pulmonary resistance. The mean time between diagnosis of pulmonary hypertension and lung transplantation was 5.9 years (range, 0.4-20 y). Seven patients were in functional class III and 8 in functional class IV. The mean 6-minute walk distance was 204 m (range, 0-360 m). Four patients (26%) died during the during the perioperative period and 9 (60%), 7 (46%), and 6 (40%) were still alive at 1, 3, and 5 years, respectively. CONCLUSIONS: Double-lung transplantation is a therapeutic option that, in certain cases, has similar outcomes to those achieved with the most aggressive medical treatment for pulmonary hypertension. FAU - López-Meseguer, Manuel AU - López-Meseguer M AD - Servei de Pneumologia, Hospital General Universitari Vall d'Hebron, Universitat Autonòma de Barcelona, CIBER de Enfermedades Respiratorias, Barcelona, España. FAU - Román, Antonio AU - Román A FAU - Monforte, Víctor AU - Monforte V FAU - Bravo, Carlos AU - Bravo C FAU - Solé, Joan AU - Solé J FAU - Morell, Ferran AU - Morell F LA - spa PT - Journal Article TT - Trasplante bipulmonar en hipertensión pulmonar. Una serie de 15 pacientes. DEP - 20090502 PL - Spain TA - Arch Bronconeumol JT - Archivos de bronconeumologia JID - 0354720 RN - 0 (Fatty Acids, Monounsaturated) RN - 0 (Plant Oils) RN - 0 (Rapeseed Oil) RN - DCR9Z582X0 (Epoprostenol) SB - IM MH - Adult MH - Brassica MH - Bronchiolitis Obliterans/etiology/mortality MH - CREST Syndrome/complications MH - Cardiovascular Diseases/complications MH - Combined Modality Therapy MH - Epoprostenol/therapeutic use MH - Fatty Acids, Monounsaturated MH - Female MH - Follow-Up Studies MH - Graft Rejection MH - Hemodynamics MH - Histiocytosis, Langerhans-Cell/complications MH - Humans MH - Hypertension, Pulmonary/chemically induced/drug therapy/etiology/physiopathology/*surgery MH - Infections/etiology/mortality MH - Lung Transplantation/*methods/mortality MH - Male MH - Middle Aged MH - Plant Oils/poisoning MH - Postoperative Complications/epidemiology MH - Rapeseed Oil MH - Retrospective Studies MH - Survival Analysis MH - Treatment Outcome MH - Young Adult EDAT- 2009/05/05 09:00 MHDA- 2009/11/17 06:00 CRDT- 2009/05/05 09:00 PHST- 2008/09/02 00:00 [received] PHST- 2008/11/07 00:00 [revised] PHST- 2008/12/27 00:00 [accepted] PHST- 2009/05/05 09:00 [entrez] PHST- 2009/05/05 09:00 [pubmed] PHST- 2009/11/17 06:00 [medline] AID - S0300-2896(09)00138-0 [pii] AID - 10.1016/j.arbres.2008.11.007 [doi] PST - ppublish SO - Arch Bronconeumol. 2009 Aug;45(8):366-70. doi: 10.1016/j.arbres.2008.11.007. Epub 2009 May 2. PMID- 19358063 OWN - NLM STAT- MEDLINE DCOM- 20090626 LR - 20091111 IS - 0044-2771 (Print) IS - 0044-2771 (Linking) VI - 47 IP - 4 DP - 2009 Apr TI - [A 64-year-old woman with perianal bleeding, chronic diarrhea and severe fecal incontinence]. PG - 361-4 LID - 10.1055/s-2008-1027703 [doi] AB - Unclear perianal bleeding may cause diagnostic and therapeutic difficulty, particularly when the bleeding source cannot be detected. In this case record we report on a 64-year-old woman with systemic sclerosis and incomplete CRES(T) syndrome diagnosed more than 10 years ago with no detectable teleangiectasia/angiodysplasia at that time. During the course of the disease the initially incomplete CRES(T) syndrome developed into a complete CREST syndrome with repeated bleeding from teleangiectatic/angiodysplastic lesions of the rectum, stomach/duodenum. In addition, chronic diarrhoea with malabsorption, bacterial overgrowth and severe anal incontinence were present which all were seen as intestinal manifestations of the existing underlying disease. A complete motoric and sensoric insufficiency of the anal sphincter was found manometrically, anal endosonography with elastography revealed changes compatible with sclerosis. In the absence of a causal therapy symptomatic treatment strategies are described and discussed on the basis of existing pathophysiologic knowledge. FAU - Allgayer, H AU - Allgayer H AD - Innere Medizin, Rehaklinik Ob der Tauber, Bismarckstr. 31, 97980 Bad Mergentheim. allgayer@reha-klinik-odt.de FAU - Dietrich, C F AU - Dietrich CF LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - 64-jährige Patientin mit perianalen Blutabgängen, chronischer Diarrhö und schwerer Stuhlinkontinenz. DEP - 20090408 PL - Germany TA - Z Gastroenterol JT - Zeitschrift fur Gastroenterologie JID - 0033370 SB - IM MH - Angiodysplasia/*diagnosis MH - CREST Syndrome/*diagnosis MH - Colonoscopy MH - Diagnosis, Differential MH - Diarrhea/*etiology MH - Elasticity Imaging Techniques MH - Endosonography MH - Fecal Incontinence/*etiology MH - Gastrointestinal Diseases/*diagnosis MH - Gastrointestinal Hemorrhage/*etiology MH - Humans MH - Intestinal Mucosa/pathology MH - Middle Aged MH - Scleroderma, Systemic/*diagnosis MH - Telangiectasia, Hereditary Hemorrhagic/*diagnosis EDAT- 2009/04/10 09:00 MHDA- 2009/06/27 09:00 CRDT- 2009/04/10 09:00 PHST- 2009/04/10 09:00 [entrez] PHST- 2009/04/10 09:00 [pubmed] PHST- 2009/06/27 09:00 [medline] AID - 10.1055/s-2008-1027703 [doi] PST - ppublish SO - Z Gastroenterol. 2009 Apr;47(4):361-4. doi: 10.1055/s-2008-1027703. Epub 2009 Apr 8. PMID- 19242018 OWN - NLM STAT- MEDLINE DCOM- 20090318 LR - 20090226 IS - 0019-4832 (Print) IS - 0019-4832 (Linking) VI - 60 IP - 4 DP - 2008 Jul-Aug TI - Scleroderma and coronary artery disease: a case report. PG - 363-5 AB - We present the case of a 35-year-old woman presenting with the rare combination of scleroderma and ischemic heart disease. Her ECG suggested old inferior wall myocardial infarction. Coronary angiography revealed significant coronary artery disease. FAU - Mathew, Jomal AU - Mathew J AD - Department of Medicine, JIPMER, Puducherry, India. mathewjomal@yahoo.co.in FAU - Negi, Vir Singh AU - Negi VS FAU - Balachander, Jayaraman AU - Balachander J FAU - Swaminathan, Rathinam Palamalai AU - Swaminathan RP LA - eng PT - Case Reports PT - Journal Article PL - India TA - Indian Heart J JT - Indian heart journal JID - 0374675 SB - IM MH - Adult MH - CREST Syndrome/*diagnosis/pathology MH - Coronary Angiography MH - Female MH - Humans MH - Myocardial Infarction/*diagnosis/drug therapy/physiopathology MH - Myocardial Ischemia/*diagnosis/drug therapy/pathology/physiopathology EDAT- 2009/02/27 09:00 MHDA- 2009/03/19 09:00 CRDT- 2009/02/27 09:00 PHST- 2009/02/27 09:00 [entrez] PHST- 2009/02/27 09:00 [pubmed] PHST- 2009/03/19 09:00 [medline] PST - ppublish SO - Indian Heart J. 2008 Jul-Aug;60(4):363-5. PMID- 19221282 OWN - NLM STAT- MEDLINE DCOM- 20090331 LR - 20090217 IS - 1538-3652 (Electronic) IS - 0003-987X (Linking) VI - 145 IP - 2 DP - 2009 Feb TI - Surgical debridement of painful fingertip calcinosis cutis in CREST syndrome. PG - 212-3 LID - 10.1001/archderm.145.2.212-b [doi] FAU - Saddic, Nicole AU - Saddic N FAU - Miller, Jeffrey J AU - Miller JJ FAU - Miller, O Fred 3rd AU - Miller OF 3rd FAU - Clarke, Jennie T AU - Clarke JT LA - eng PT - Case Reports PT - Letter PL - United States TA - Arch Dermatol JT - Archives of dermatology JID - 0372433 SB - IM MH - CREST Syndrome/*pathology/*surgery MH - *Debridement MH - Fingers MH - Humans MH - Male MH - Middle Aged EDAT- 2009/02/18 09:00 MHDA- 2009/04/01 09:00 CRDT- 2009/02/18 09:00 PHST- 2009/02/18 09:00 [entrez] PHST- 2009/02/18 09:00 [pubmed] PHST- 2009/04/01 09:00 [medline] AID - 145/2/212-a [pii] AID - 10.1001/archderm.145.2.212-b [doi] PST - ppublish SO - Arch Dermatol. 2009 Feb;145(2):212-3. doi: 10.1001/archderm.145.2.212-b. PMID- 18718225 OWN - NLM STAT- MEDLINE DCOM- 20081024 LR - 20080822 IS - 1365-229X (Electronic) IS - 0009-9260 (Linking) VI - 63 IP - 9 DP - 2008 Sep TI - The role of radiology in the management of systemic sclerosis. PG - 959-67 LID - 10.1016/j.crad.2008.05.007 [doi] AB - Systemic sclerosis is a multisystem connective tissue disorder. Radiology plays an integral part in its management, guiding the clinician concerning the onset and severity of visceral involvement. After skin involvement, the gastrointestinal tract is the most commonly affected system; contrast radiography and magnetic resonance imaging (MRI) play a role in diagnosis. Non-specific interstitial pneumonia is the most frequent respiratory disease and high-resolution computed tomography (CT) is the cornerstone of management. In common with other rheumatic disorders, the role of cardiac MRI is expanding. Radiography remains the main technique in the investigation of skeletal involvement, although MRI is useful as a problem-solving tool. Neurological involvement is increasingly recognized and the major role of radiology is the exclusion of coexistent pathology. We present a thorough review of the role of radiology in the management of systemic sclerosis. FAU - Madani, G AU - Madani G AD - Radiology Department, St Mary's Hospital, Royal Free Campus, University College London, London, UK. gittamadani@yahoo.com FAU - Katz, R D AU - Katz RD FAU - Haddock, J A AU - Haddock JA FAU - Denton, C P AU - Denton CP FAU - Bell, J R AU - Bell JR LA - eng PT - Journal Article PT - Review DEP - 20080726 PL - England TA - Clin Radiol JT - Clinical radiology JID - 1306016 RN - 0 (Contrast Media) SB - IM MH - CREST Syndrome/physiopathology MH - Contrast Media MH - Diagnostic Imaging/*methods MH - Disease Progression MH - Gastrointestinal Diseases/*diagnosis/etiology/pathology MH - Gastrointestinal Motility MH - Heart Diseases/diagnosis/etiology/pathology MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/etiology/pathology MH - Musculoskeletal Diseases/*diagnosis/etiology/pathology MH - Scleroderma, Systemic/complications/*diagnosis/pathology MH - Severity of Illness Index MH - Sjogren's Syndrome/diagnosis RF - 56 EDAT- 2008/08/23 09:00 MHDA- 2008/10/25 09:00 CRDT- 2008/08/23 09:00 PHST- 2007/09/25 00:00 [received] PHST- 2008/05/08 00:00 [revised] PHST- 2008/05/08 00:00 [accepted] PHST- 2008/08/23 09:00 [pubmed] PHST- 2008/10/25 09:00 [medline] PHST- 2008/08/23 09:00 [entrez] AID - S0009-9260(08)00239-0 [pii] AID - 10.1016/j.crad.2008.05.007 [doi] PST - ppublish SO - Clin Radiol. 2008 Sep;63(9):959-67. doi: 10.1016/j.crad.2008.05.007. Epub 2008 Jul 26. PMID- 18712025 OWN - NLM STAT- MEDLINE DCOM- 20081030 LR - 20130912 IS - 0011-4162 (Print) IS - 0011-4162 (Linking) VI - 82 IP - 1 DP - 2008 Jul TI - Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature. PG - 55-9 AB - Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis. FAU - Summers, Erika M AU - Summers EM AD - Department of Internal Medicine, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA. esummers@bidmc.harvard.edu FAU - Kendrick, Christina G AU - Kendrick CG LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - Cutis JT - Cutis JID - 0006440 SB - IM MH - Amyloidosis/*complications/*pathology/therapy MH - CREST Syndrome/*complications/*pathology/therapy MH - Female MH - Humans MH - Middle Aged MH - Skin Diseases, Metabolic/*complications/*pathology/therapy RF - 30 EDAT- 2008/08/21 09:00 MHDA- 2008/10/31 09:00 CRDT- 2008/08/21 09:00 PHST- 2008/08/21 09:00 [pubmed] PHST- 2008/10/31 09:00 [medline] PHST- 2008/08/21 09:00 [entrez] PST - ppublish SO - Cutis. 2008 Jul;82(1):55-9. PMID- 18677336 OWN - NLM STAT- MEDLINE DCOM- 20080813 LR - 20080821 IS - 1175-8716 (Electronic) IS - 0028-8446 (Linking) VI - 121 IP - 1277 DP - 2008 Jul 4 TI - Medical image. Genital lupus. PG - 86-7 FAU - Darwish, Tarek AU - Darwish T AD - Department of Internal Medicine, University of Missouri-Kansas City, MO, USA. darwisht@umkc.edu. LA - eng PT - Case Reports PT - Journal Article DEP - 20080704 PL - New Zealand TA - N Z Med J JT - The New Zealand medical journal JID - 0401067 SB - IM CIN - N Z Med J. 2008 Jul 25;121(1278):101-2; author reply 102. PMID: 18670481 MH - CREST Syndrome/*diagnosis MH - Diagnosis, Differential MH - Female MH - Genital Diseases, Female/*diagnosis MH - Humans MH - Lupus Erythematosus, Systemic/*diagnosis MH - Middle Aged EDAT- 2008/08/05 09:00 MHDA- 2008/08/14 09:00 CRDT- 2008/08/05 09:00 PHST- 2008/08/05 09:00 [pubmed] PHST- 2008/08/14 09:00 [medline] PHST- 2008/08/05 09:00 [entrez] PST - epublish SO - N Z Med J. 2008 Jul 4;121(1277):86-7. PMID- 18283901 OWN - NLM STAT- MEDLINE DCOM- 20080408 LR - 20190724 IS - 0021-5384 (Print) IS - 0021-5384 (Linking) VI - 97 IP - 1 DP - 2008 Jan 10 TI - [Anti-centromere antibody-positive primary biliary cirrhosis with rapid development]. PG - 144-6 FAU - Suzuki, Yasuaki AU - Suzuki Y AD - Department of Medicine, Division of Gastroenterology and Hematology/Oncology, Asahikawa Medical College, Asahikawa. FAU - Abe, Masami AU - Abe M FAU - Hosoki, Yayoi AU - Hosoki Y FAU - Miyoshi, Shigeki AU - Miyoshi S FAU - Inoue, Mitsutaka AU - Inoue M FAU - Ohhira, Masumi AU - Ohhira M FAU - Ohtake, Takaaki AU - Ohtake T FAU - Kohgo, Yutaka AU - Kohgo Y LA - jpn PT - Case Reports PT - Journal Article PL - Japan TA - Nihon Naika Gakkai Zasshi JT - Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine JID - 19130210R RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Autoantibodies/*blood MH - CREST Syndrome/*complications/immunology MH - Centromere/*immunology MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/*complications EDAT- 2008/02/21 09:00 MHDA- 2008/04/09 09:00 CRDT- 2008/02/21 09:00 PHST- 2008/02/21 09:00 [pubmed] PHST- 2008/04/09 09:00 [medline] PHST- 2008/02/21 09:00 [entrez] AID - 10.2169/naika.97.144 [doi] PST - ppublish SO - Nihon Naika Gakkai Zasshi. 2008 Jan 10;97(1):144-6. doi: 10.2169/naika.97.144. PMID- 17473489 OWN - NLM STAT- MEDLINE DCOM- 20070523 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 46 IP - 9 DP - 2007 TI - Two cases of primary biliary cirrhosis complicated by acute leukemia. PG - 561-3 AB - Case 1 was a 43-year-old woman, who was diagnosed as having PBC. After one month, she was hospitalized owing to sudden temporary unconsciousness. She was diagnosed as having acute lymphoid leukemia (ALL) by bone marrow examination. Chemotherapy was done, but she died after 6 months. Case 2 was a 54-year-old woman, who was diagnosed as having PBC with CREST syndrome. Seven years later, she was diagnosed as having acute promyelocytic leukemia (APL) by bone marrow examination. Chemotherapy was continued, and her symptoms are at present, stable. To date, there have been no reports of PBC complicated by acute leukemia. FAU - Saito, Hironobu AU - Saito H AD - Department of Internal Medicine II, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Monoe, Kyoko AU - Monoe K FAU - Kanno, Yukiko AU - Kanno Y FAU - Takahashi, Atsushi AU - Takahashi A FAU - Rai, Tsuyoshi AU - Rai T FAU - Irisawa, Atsushi AU - Irisawa A FAU - Ohira, Hiromasa AU - Ohira H LA - eng PT - Case Reports PT - Journal Article DEP - 20070501 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - EC 2.3.1.12 (Dihydrolipoyllysine-Residue Acetyltransferase) SB - IM MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Blotting, Western MH - CREST Syndrome/complications MH - Dihydrolipoyllysine-Residue Acetyltransferase/metabolism MH - Fatal Outcome MH - Female MH - Humans MH - Leukemia, Promyelocytic, Acute/*complications/drug therapy MH - Liver/pathology MH - Liver Cirrhosis, Biliary/*complications/enzymology/pathology MH - Middle Aged MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*complications/drug therapy EDAT- 2007/05/03 09:00 MHDA- 2007/05/24 09:00 CRDT- 2007/05/03 09:00 PHST- 2007/05/03 09:00 [pubmed] PHST- 2007/05/24 09:00 [medline] PHST- 2007/05/03 09:00 [entrez] AID - JST.JSTAGE/internalmedicine/46.6037 [pii] AID - 10.2169/internalmedicine.46.6037 [doi] PST - ppublish SO - Intern Med. 2007;46(9):561-3. doi: 10.2169/internalmedicine.46.6037. Epub 2007 May 1. PMID- 16980839 OWN - NLM STAT- MEDLINE DCOM- 20061005 LR - 20221207 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 118 IP - 4 DP - 2006 Sep 15 TI - Free anterolateral thigh perforator flap in a patient with systemic sclerosis and Raynaud's phenomenon. PG - 90e-92e LID - 10.1097/01.prs.0000232388.59236.3a [doi] FAU - Jandali, Shareef AU - Jandali S AD - New York, N.Y. From the Plastic and Reconstructive Surgery Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center. FAU - Mehrara, Babak AU - Mehrara B LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM MH - Bone Neoplasms/secondary/*surgery MH - CREST Syndrome MH - Carcinoma, Squamous Cell/secondary/*surgery MH - Dura Mater MH - Female MH - Forehead MH - Frontal Bone MH - Humans MH - Meningeal Neoplasms/secondary/*surgery MH - Middle Aged MH - Neoplasm Invasiveness MH - Neoplasm Recurrence, Local MH - Plastic Surgery Procedures/*methods MH - Skin Neoplasms/pathology/*surgery MH - Surgical Flaps/*blood supply MH - Thigh MH - Wounds and Injuries/surgery EDAT- 2006/09/19 09:00 MHDA- 2006/10/06 09:00 CRDT- 2006/09/19 09:00 PHST- 2006/09/19 09:00 [pubmed] PHST- 2006/10/06 09:00 [medline] PHST- 2006/09/19 09:00 [entrez] AID - 00006534-200609150-00010 [pii] AID - 10.1097/01.prs.0000232388.59236.3a [doi] PST - ppublish SO - Plast Reconstr Surg. 2006 Sep 15;118(4):90e-92e. doi: 10.1097/01.prs.0000232388.59236.3a. PMID- 16431161 OWN - NLM STAT- MEDLINE DCOM- 20070208 LR - 20161124 IS - 1525-2167 (Print) IS - 1532-2114 (Linking) VI - 7 IP - 6 DP - 2006 Dec TI - Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I). PG - 439-46 AB - Patients with autoimmune disease, and in particular limited systemic sclerosis (CREST syndrome), are at risk of developing pulmonary artery hypertension (PAH) which is associated with a poor prognosis. With improvements in therapy offering improved survival and functional capacity, there has been an emphasis on screening to identify patients at risk. Assessment of patients during exercise may enable early identification of patients with this condition. AIMS AND METHODS: We aimed to assess the ability of exercise stress echocardiography to evaluate the change in pulmonary artery pressure in 51 patients with autoimmune disease (systemic lupus erythamatosus (SLE), limited systemic sclerosis (LSS or "CREST") and diffuse systemic sclerosis (DSS)). Systolic pulmonary artery pressure (sPAP) was estimated using interrogation of the tricuspid incompetence jet before and after exercise. PAH was classified as normal, mild, moderate or severe using echocardiographic assessment of sPAP. RESULTS: We were able to estimate pre-exercise and post-exercise sPAP in 92% and 90% of patients, respectively. Pulmonary pressures rose or remained unchanged in all screened individuals, with a mean rise during stress of 14.1mmHg (+/-1.1). Pulmonary artery pressure rose significantly in each of three subgroups (p<0.05). Stress echocardiography demonstrated PAH (using a cut-off of >35mmHg) in 59% of all individuals with systemic autoimmunity. CONCLUSION: Stress echocardiography is a useful tool in identifying individuals with autoimmune disease who may have underlying pulmonary arterial disease that may be amenable to therapy. We noted a consistent elevation in sPAP across all autoimmune subtypes, suggesting an abnormal pulmonary vascular response to exercise exists in these patients. FAU - Collins, Nicholas AU - Collins N AD - Cardiovascular Unit, John Hunter Hospital, Lookout Road, New Lambton, Newcastle, NSW 2305, Australia. nandl_collins@yahoo.com.au FAU - Bastian, Bruce AU - Bastian B FAU - Quiqueree, Laurent AU - Quiqueree L FAU - Jones, Carol AU - Jones C FAU - Morgan, Renae AU - Morgan R FAU - Reeves, Glenn AU - Reeves G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060120 PL - England TA - Eur J Echocardiogr JT - European journal of echocardiography : the journal of the Working Group on Echocardiography of the European Society of Cardiology JID - 100890618 SB - IM MH - Aged MH - Autoimmune Diseases/*complications/physiopathology MH - CREST Syndrome/complications/physiopathology MH - *Echocardiography, Stress MH - Exercise Test/methods MH - Female MH - Humans MH - Hypertension, Pulmonary/*diagnostic imaging/prevention & control MH - Male MH - Middle Aged MH - Pulmonary Artery/*diagnostic imaging/physiopathology EDAT- 2006/01/25 09:00 MHDA- 2007/02/09 09:00 CRDT- 2006/01/25 09:00 PHST- 2005/06/30 00:00 [received] PHST- 2005/11/20 00:00 [revised] PHST- 2005/12/04 00:00 [accepted] PHST- 2006/01/25 09:00 [pubmed] PHST- 2007/02/09 09:00 [medline] PHST- 2006/01/25 09:00 [entrez] AID - S1525-2167(05)00229-5 [pii] AID - 10.1016/j.euje.2005.12.002 [doi] PST - ppublish SO - Eur J Echocardiogr. 2006 Dec;7(6):439-46. doi: 10.1016/j.euje.2005.12.002. Epub 2006 Jan 20. PMID- 32373479 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2214-9112 (Electronic) IS - 2214-9112 (Linking) VI - 27 DP - 2020 Jul TI - Arterial palmar arch occlusion in a woman with Raynaud's disease taking oral combined menopausal hormone therapy: A case report. PG - e00206 LID - 10.1016/j.crwh.2020.e00206 [doi] LID - e00206 AB - Patients with surgical menopause often present to their primary care provider with menopausal symptoms. Here we present an unusual case of palmar arch artery occlusion in a 55-year-old woman with primary Raynaud's disease taking oral combined menopausal hormone therapy after surgical menopause for endometriosis. She was successfully treated with intravenous infusion of prostaglandin E1 and nitroglycerin patches over two weeks. Menopausal hormone therapy was stopped and her vasomotor symptoms did not recur. CI - © 2020 Published by Elsevier B.V. FAU - Llaneza-Suarez, Cristina AU - Llaneza-Suarez C AD - Xove Centro de Salud, Hospital Da Costa Burela, Camino Real, 0, 27870 Xove, Lugo, Spain. FAU - Eiriz-Eirin, Montserrat AU - Eiriz-Eirin M AD - Xove Centro de Salud, Hospital Da Costa Burela, Camino Real, 0, 27870 Xove, Lugo, Spain. FAU - Llaneza, Placido AU - Llaneza P AD - Universidad de Oviedo, La Merced, 22, 2C, 33201 Oviedo, Gijón, Spain. LA - eng PT - Case Reports PT - Journal Article DEP - 20200421 PL - Netherlands TA - Case Rep Womens Health JT - Case reports in women's health JID - 101682122 PMC - PMC7191182 OTO - NOTNLM OT - Arterial palmar arch occlusion OT - Cardiovascular risk OT - Finger vascular lesion OT - Menopausal hormone therapy OT - Raynaud's disease EDAT- 2020/05/07 06:00 MHDA- 2020/05/07 06:01 PMCR- 2020/04/21 CRDT- 2020/05/07 06:00 PHST- 2020/04/07 00:00 [received] PHST- 2020/04/16 00:00 [revised] PHST- 2020/04/20 00:00 [accepted] PHST- 2020/05/07 06:00 [entrez] PHST- 2020/05/07 06:00 [pubmed] PHST- 2020/05/07 06:01 [medline] PHST- 2020/04/21 00:00 [pmc-release] AID - S2214-9112(20)30036-9 [pii] AID - e00206 [pii] AID - 10.1016/j.crwh.2020.e00206 [doi] PST - epublish SO - Case Rep Womens Health. 2020 Apr 21;27:e00206. doi: 10.1016/j.crwh.2020.e00206. eCollection 2020 Jul. PMID- 41986912 OWN - NLM STAT- Publisher LR - 20260416 IS - 1538-4683 (Electronic) IS - 1061-5377 (Linking) DP - 2026 Apr 16 TI - Raynaud's Disease: Review of Current Literature. LID - 10.1097/CRD.0000000000001278 [doi] AB - Raynaud's disease (RD), also referred to as Raynaud's phenomenon (RP) or Raynaud's syndrome, is a vasospastic condition characterized by episodic, reversible constriction of the peripheral digital arteries and cutaneous arterioles in response to cold temperature and/or emotional stress. The condition is classified into 2 forms. The first being primary RD, which occurs in the absence of any identifiable underlying condition, and then secondary RP, which develops in the setting of a systemic disease, most commonly connective tissue disorders such as systemic sclerosis, systemic lupus erythematosus, and Sjogren's syndrome. Primary RD is considerably more prevalent and generally follows a benign course, whereas secondary RP carries a significant risk of ischemic digital complications, including ulceration, gangrene, and permanent tissue loss. The clinical hallmark of RD is the characteristic triphasic color change of the digits, progressing from pallor to cyanosis to erythema, reflecting the sequential phases of vasospasm, deoxygenation, and reactive hyperemia. Diagnosis is largely clinical, supported by a thorough history and physical examination, serologic autoantibody testing, and nailfold capillaroscopy, which serves as the gold standard for differentiating primary from secondary disease. CI - Copyright © 2026 Wolters Kluwer Health, Inc. All rights reserved. FAU - Venkatesh, Vishnu AU - Venkatesh V AD - From the Department of Medicine, Westchester Medical Center and New York Medical College, Valhalla, NY. FAU - Frishman, William H AU - Frishman WH FAU - Aronow, Wilbert S AU - Aronow WS LA - eng PT - Journal Article DEP - 20260416 PL - United States TA - Cardiol Rev JT - Cardiology in review JID - 9304686 SB - IM OTO - NOTNLM OT - Raynaud’s disease OT - Raynaud’s phenomenon OT - calcium channel blockers OT - connective tissue disease OT - digital ischemia OT - nailfold capillaroscopy OT - primary Raynaud’s OT - secondary Raynaud’s OT - systemic sclerosis OT - vasospasm COIS- Disclosure: The authors have no conflicts of interest to report. EDAT- 2026/04/16 13:09 MHDA- 2026/04/16 13:09 CRDT- 2026/04/16 00:02 PHST- 2026/04/16 13:09 [medline] PHST- 2026/04/16 13:09 [pubmed] PHST- 2026/04/16 00:02 [entrez] AID - 00045415-990000000-00814 [pii] AID - 10.1097/CRD.0000000000001278 [doi] PST - aheadofprint SO - Cardiol Rev. 2026 Apr 16. doi: 10.1097/CRD.0000000000001278. PMID- 21057622 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 2005-7563 (Electronic) IS - 2005-6419 (Print) IS - 2005-6419 (Linking) VI - 59 IP - 4 DP - 2010 Oct TI - Sequential bipolar radiofrequency lumbar sympathectomy in Raynaud's disease -A case report-. PG - 286-9 LID - 10.4097/kjae.2010.59.4.286 [doi] AB - A 39-year-old female was suffering from cold-induced Raynaud's attacks in both hands and feet, with symptoms being most severe in her left foot. The patient did not respond to medical treatments and was referred to our department of pain medicine. We performed sequential bipolar radiofrequency lumbar sympathectomy to the patient, which offered a long duration of symptom relief. Sequential bipolar radiofrequency lesions could create continuous strip lesion, and thus, could achieve better results, while the potential risk of liquid neurolytic agents could be avoided. FAU - Kang, Sang-Soo AU - Kang SS AD - Department of Anesthesiology and Pain Medicine, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea. FAU - Shin, Keun-Man AU - Shin KM FAU - Jung, Sang-Moon AU - Jung SM FAU - Park, Jun-Hee AU - Park JH FAU - Hong, Seong-Jun AU - Hong SJ LA - eng PT - Journal Article DEP - 20101021 PL - Korea (South) TA - Korean J Anesthesiol JT - Korean journal of anesthesiology JID - 101502451 PMC - PMC2966713 OTO - NOTNLM OT - Bipolar OT - Lumbar sympathectomy OT - Radiofrequency OT - Raynaud's disease EDAT- 2010/11/09 06:00 MHDA- 2010/11/09 06:01 PMCR- 2010/10/01 CRDT- 2010/11/09 06:00 PHST- 2009/10/05 00:00 [received] PHST- 2009/10/30 00:00 [revised] PHST- 2009/11/26 00:00 [accepted] PHST- 2010/11/09 06:00 [entrez] PHST- 2010/11/09 06:00 [pubmed] PHST- 2010/11/09 06:01 [medline] PHST- 2010/10/01 00:00 [pmc-release] AID - 10.4097/kjae.2010.59.4.286 [doi] PST - ppublish SO - Korean J Anesthesiol. 2010 Oct;59(4):286-9. doi: 10.4097/kjae.2010.59.4.286. Epub 2010 Oct 21. PMID- 41546369 OWN - NLM STAT- Publisher LR - 20260117 IS - 1461-7277 (Electronic) IS - 1359-1053 (Linking) DP - 2026 Jan 16 TI - Cold Hands, Warm Heart: Quality of life, wellbeing, and mental health in Raynaud's disease - An international survey study. PG - 13591053251407736 LID - 10.1177/13591053251407736 [doi] AB - The study assessed quality of life, wellbeing, and mental health in individuals living with Raynaud's disease (RD), using a large international sample (n = 720). Participants completed Raynaud-Specific Quality of Life Questionnaire (RQLQ), WHO-5 Wellbeing Index, DASS-21 and demographics. Meteorological variables were recorded based on location. Participants with secondary RD reported lower quality of life and wellbeing, and higher anxiety, depression, and pain, than participants with primary RD or without a diagnosis. Participants without a diagnosis reported worse mental health than groups with a diagnosis. Participants in tropical climates reported the lowest quality of life, and those in temperate climates had the lowest wellbeing. Pain and symptom severity were the strongest predictors of quality of life. RD negatively affects quality of life, wellbeing, and mental health, particularly in secondary RD. Pain and symptom severity are key determinants. Meteorological factors contribute minimally. Tailored interventions focusing on symptom management should be prioritised. FAU - Vaportzis, Eleftheria AU - Vaportzis E AUID- ORCID: 0000-0002-0522-5095 AD - University of Bradford, UK. LA - eng PT - Journal Article DEP - 20260116 PL - England TA - J Health Psychol JT - Journal of health psychology JID - 9703616 SB - IM OTO - NOTNLM OT - Raynaud’s disease OT - international survey OT - mental health OT - quality of life OT - temperature OT - wellbeing EDAT- 2026/01/17 06:30 MHDA- 2026/01/17 06:30 CRDT- 2026/01/17 01:02 PHST- 2026/01/17 06:30 [medline] PHST- 2026/01/17 06:30 [pubmed] PHST- 2026/01/17 01:02 [entrez] AID - 10.1177/13591053251407736 [doi] PST - aheadofprint SO - J Health Psychol. 2026 Jan 16:13591053251407736. doi: 10.1177/13591053251407736. PMID- 30397635 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240403 IS - 2332-0885 (Print) IS - 2332-0885 (Electronic) IS - 2332-0885 (Linking) VI - 5 IP - 1 DP - 2018 TI - Hyperbaric oxygen therapy for digital ulcers due to Raynaud's disease. PG - 72-74 LID - 10.1080/23320885.2018.1525684 [doi] AB - We present a case of Raynaud's disease with digital ulcers that was successfully treated with hyperbaric oxygen therapy. Hyperbaric oxygen therapy can be considered as a safe and useful adjunct treatment for intractable digital ulcers in patients with Raynaud's disease. FAU - Sato, Tomoya AU - Sato T AD - Department of Plastic and Reconstructive Surgery, Saitama Medical University, Moroyama, Japan. FAU - Arai, Kiyohito AU - Arai K AD - Department of Cardiology, Saiseikai Kurihasi Hospital, Kuki City, Japan. FAU - Ichioka, Shigeru AU - Ichioka S AD - Department of Plastic and Reconstructive Surgery, Saitama Medical University, Moroyama, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20181025 PL - United States TA - Case Reports Plast Surg Hand Surg JT - Case reports in plastic surgery & hand surgery JID - 101655541 PMC - PMC6211215 OTO - NOTNLM OT - Addiction OT - alcohol OT - knowledge OT - media OT - qualitative interviews OT - trust EDAT- 2018/11/07 06:00 MHDA- 2018/11/07 06:01 PMCR- 2018/10/25 CRDT- 2018/11/07 06:00 PHST- 2018/06/07 00:00 [received] PHST- 2018/09/16 00:00 [accepted] PHST- 2018/11/07 06:00 [entrez] PHST- 2018/11/07 06:00 [pubmed] PHST- 2018/11/07 06:01 [medline] PHST- 2018/10/25 00:00 [pmc-release] AID - 1525684 [pii] AID - 10.1080/23320885.2018.1525684 [doi] PST - epublish SO - Case Reports Plast Surg Hand Surg. 2018 Oct 25;5(1):72-74. doi: 10.1080/23320885.2018.1525684. eCollection 2018. PMID- 40661725 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20260521 IS - 2042-8812 (Print) IS - 2042-8812 (Electronic) IS - 2042-8812 (Linking) VI - 2025 IP - 7 DP - 2025 Jul TI - Deep inferior epigastric perforator flap breast reconstruction in patients with Raynaud's disease: a case series and literature review. PG - rjaf535 LID - 10.1093/jscr/rjaf535 [doi] LID - rjaf535 AB - Free tissue transfer using the deep inferior epigastric perforator (DIEP) flap represents the gold-standard for autologous breast reconstruction. Raynaud's disease, characterized by vasospasm, thrombosis, and a hypercoagulable state, poses a theoretical risk to flap viability; however, its impact on DIEP outcomes remains poorly defined. We present the first reported case series evaluating DIEP flap reconstruction in three patients with Raynaud's disease. In the first case, complete flap loss occurred due to thrombosis, in the absence of vasoprotective strategies. The subsequent two patients received intraoperative papaverine and verapamil to mitigate vasospasm, and postoperative antiplatelet therapy with aspirin and dipyridamole. Both achieved successful flap outcomes without microvascular complications. Our findings suggest Raynaud's disease should not be considered a contraindication to DIEP flap reconstruction, provided tailored perioperative pharmacological strategies are employed. Larger studies are warranted to evaluate outcomes and guide evidence-based perioperative protocols in this understudied patient population. CI - © The Author(s) 2025. Published by Oxford University Press and JSCR Publishing Ltd. FAU - Shah, Rushabh AU - Shah R AD - Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, United Kingdom. FAU - Sosnowski, Krzysztof AU - Sosnowski K AD - School of Clinical Medicine, University of Cambridge, Hills Rd, Cambridge CB20SP, United Kingdom. FAU - Habeeb, Amir AU - Habeeb A AD - Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, United Kingdom. FAU - Smeeton, Benjamin AU - Smeeton B AD - Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, United Kingdom. FAU - Malata, Charles M AU - Malata CM AD - Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, United Kingdom. AD - Cambridge Breast Unit, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge CB2 0QQ, United Kingdom. AD - Anglia Ruskin University School of Medicine, Anglia Ruskin University, East Rd, Cambridge CB1 1PT, United Kingdom. LA - eng PT - Case Reports PT - Journal Article DEP - 20250714 PL - England TA - J Surg Case Rep JT - Journal of surgical case reports JID - 101560169 EIN - J Surg Case Rep. 2026 May 19;2026(5):rjag406. doi: 10.1093/jscr/rjag406. PMID: 42164008 PMC - PMC12257084 OTO - NOTNLM OT - DIEP flap OT - Raynaud’s disease OT - breast reconstruction OT - microvascular surgery OT - vasospasm COIS- None declared.\ EDAT- 2025/07/15 06:28 MHDA- 2025/07/15 06:29 PMCR- 2025/07/14 CRDT- 2025/07/15 05:05 PHST- 2025/05/30 00:00 [received] PHST- 2025/06/25 00:00 [accepted] PHST- 2025/07/15 06:29 [medline] PHST- 2025/07/15 06:28 [pubmed] PHST- 2025/07/15 05:05 [entrez] PHST- 2025/07/14 00:00 [pmc-release] AID - rjaf535 [pii] AID - 10.1093/jscr/rjaf535 [doi] PST - epublish SO - J Surg Case Rep. 2025 Jul 14;2025(7):rjaf535. doi: 10.1093/jscr/rjaf535. eCollection 2025 Jul. PMID- 23198043 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121203 LR - 20211021 IS - 2005-7563 (Electronic) IS - 2005-6419 (Print) IS - 2005-6419 (Linking) VI - 63 IP - 5 DP - 2012 Nov TI - Percutaneous bipolar radiofrequency T3 sympathicotomy in Raynaud's disease -A case report-. PG - 461-4 LID - 10.4097/kjae.2012.63.5.461 [doi] AB - A 54-year-old female was suffering from cold-induced Raynaud's attacks in her both hands with symptoms most severe in her left hand. As the patient did not respond to previous medical treatments and endoscopic thoracic sympathectomy, we performed percutaneous bipolar radiofrequency thoracic sympathicotomy at the left T3 vertebral level. After the procedure, the patient obtained a long duration of symptom relief over 3 years. Percutaneous bipolar radiofrequency T3 sympathicotomy is minimally invasive and effective technique by creating large continuous strip lesion. FAU - Kang, Sang-Soo AU - Kang SS AD - Department of Anesthesiology and Pain Medicine, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea. FAU - Park, Jung-Chan AU - Park JC FAU - Hong, Sung-Jun AU - Hong SJ FAU - Yoon, Young-Jun AU - Yoon YJ FAU - Shin, Keun-Man AU - Shin KM LA - eng PT - Journal Article DEP - 20121116 PL - Korea (South) TA - Korean J Anesthesiol JT - Korean journal of anesthesiology JID - 101502451 PMC - PMC3506859 OTO - NOTNLM OT - Bipolar OT - Radiofrequency OT - Raynaud's disease OT - Sympathectomy OT - Thoracic EDAT- 2012/12/01 06:00 MHDA- 2012/12/01 06:01 PMCR- 2012/11/01 CRDT- 2012/12/01 06:00 PHST- 2011/10/27 00:00 [received] PHST- 2011/11/23 00:00 [revised] PHST- 2011/12/29 00:00 [accepted] PHST- 2012/12/01 06:00 [entrez] PHST- 2012/12/01 06:00 [pubmed] PHST- 2012/12/01 06:01 [medline] PHST- 2012/11/01 00:00 [pmc-release] AID - 10.4097/kjae.2012.63.5.461 [doi] PST - ppublish SO - Korean J Anesthesiol. 2012 Nov;63(5):461-4. doi: 10.4097/kjae.2012.63.5.461. Epub 2012 Nov 16. PMID- 26618125 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20151130 LR - 20200929 IS - 2234-6163 (Print) IS - 2234-6171 (Electronic) IS - 2234-6163 (Linking) VI - 42 IP - 6 DP - 2015 Nov TI - Two-Step Incision for Periarterial Sympathectomy of the Hand. PG - 761-8 LID - 10.5999/aps.2015.42.6.761 [doi] AB - BACKGROUND: Surgical scars on the palmar surface of the hand may lead to functional and also aesthetic and psychological consequences. The objective of this study was to introduce a new incision technique for periarterial sympathectomy of the hand and to compare the results of the new two-step incision technique with those of a Koman incision by using an objective questionnaire. METHODS: A total of 40 patients (17 men and 23 women) with intractable Raynaud's disease or syndrome underwent surgery in our hospital, conducted by a single surgeon, between January 2008 and January 2013. Patients who had undergone extended sympathectomy or vessel graft were excluded. Clinical evaluation of postoperative scars was performed in both groups one year after surgery using the patient and observer scar assessment scale (POSAS) and the Wake Forest University rating scale. RESULTS: The total patient score was 8.59 (range, 6-15) in the two-step incision group and 9.62 (range, 7-18) in the Koman incision group. A significant difference was found between the groups in the total PS score (P-value=0.034) but not in the total observer score. Our analysis found no significant difference in preoperative and postoperative Wake Forest University rating scale scores between the two-step and Koman incision groups. The time required for recovery prior to returning to work after surgery was shorter in the two-step incision group, with a mean of 29.48 days in the two-step incision group and 34.15 days in the Koman incision group (P=0.03). CONCLUSIONS: Compared to the Koman incision, the new two-step incision technique provides better aesthetic results, similar symptom improvement, and a reduction in the recovery time required before returning to work. Furthermore, this incision allows the surgeon to access a wide surgical field and a sufficient exposure of anatomical structures. FAU - Jeon, Seung Bae AU - Jeon SB AD - Department of Plastic and Reconstructive Surgery, Hanyang University Seoul Hospital, Hanyang University College of Medicine, Seoul, Korea. FAU - Ahn, Hee Chang AU - Ahn HC AD - Department of Plastic and Reconstructive Surgery, Hanyang University Seoul Hospital, Hanyang University College of Medicine, Seoul, Korea. FAU - Ahn, Yong Su AU - Ahn YS AD - START Plastic Surgery Clinic, Seoul, Korea. FAU - Choi, Matthew Seung Suk AU - Choi MS AD - Department of Plastic and Reconstructive Surgery, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea. LA - eng PT - Journal Article DEP - 20151116 PL - Korea (South) TA - Arch Plast Surg JT - Archives of plastic surgery JID - 101577999 PMC - PMC4659991 OTO - NOTNLM OT - Raynaud disease OT - Sympathectomy COIS- No potential conflict of interest relevant to this article was reported. EDAT- 2015/12/01 06:00 MHDA- 2015/12/01 06:01 PMCR- 2015/11/01 CRDT- 2015/12/01 06:00 PHST- 2015/05/13 00:00 [received] PHST- 2015/08/28 00:00 [revised] PHST- 2015/09/24 00:00 [accepted] PHST- 2015/12/01 06:00 [entrez] PHST- 2015/12/01 06:00 [pubmed] PHST- 2015/12/01 06:01 [medline] PHST- 2015/11/01 00:00 [pmc-release] AID - 10.5999/aps.2015.42.6.761 [doi] PST - ppublish SO - Arch Plast Surg. 2015 Nov;42(6):761-8. doi: 10.5999/aps.2015.42.6.761. Epub 2015 Nov 16. PMID- 36908929 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230314 IS - 1178-7090 (Print) IS - 1178-7090 (Electronic) IS - 1178-7090 (Linking) VI - 16 DP - 2023 TI - Efficacy of Radiofrequency Thermocoagulation of the Thoracic Sympathetic Nerve versus Chemical Excision in Pain Caused by Raynaud's Disease. PG - 649-658 LID - 10.2147/JPR.S398298 [doi] AB - OBJECTIVE: To investigate the effectiveness and safety of computed tomography (CT)-guided radiofrequency thermocoagulation (RFTC) of the thoracic sympathetic nerve versus chemical resection (CTS) for the treatment of pain caused by Raynaud's disease. METHODS: Patients who underwent CTS or thoracic sympathetic nerve RFTC between March 2012 and March 2021 were enrolled in this retrospective study. There were 28 cases in the alcohol group (Group A) and 44 in the radiofrequency group (Group R). Visual analog scores (VAS) were collected from patients at different time points, as well as preoperative and postoperative finger end perfusion index (PI) and hand temperature (T). The efficiency, postoperative recurrence rate, complications, and improvement in postoperative quality of life were observed in both groups. RESULTS: Pain scores at different follow-up times after surgery decreased in both groups compared to the preoperative period (P < 0.05). Postoperative T and PI were higher in both groups than preoperatively all (P < 0.05). The recurrence rate was higher in the R group than in the A group. Postoperative complications were observed in 13.6% and 25% of patients in groups R and A, respectively. Meanwhile, the postoperative quality of life improved in both groups, but the radiofrequency (RF) group was better than the alcohol group in terms of improvement in quality of life (P < 0.05). CONCLUSION: Both CT-guided CTS and RFTC of the thoracic sympathetic nerve provided good treatment outcomes. However, the RF group was superior to the alcohol group in terms of complication rate and quality of life improvement. CI - © 2023 Xin et al. FAU - Xin, Bingyue AU - Xin B AUID- ORCID: 0000-0003-2232-069X AD - Department of Anesthesiology and Pain Research Center, The First Hospital of Jiaxing or The Affiliated Hospital of Jiaxing University, Jiaxing, People's Republic of China. FAU - Xie, Keyue AU - Xie K AUID- ORCID: 0000-0002-6457-2046 AD - Department of Anesthesiology and Pain Research Center, The First Hospital of Jiaxing or The Affiliated Hospital of Jiaxing University, Jiaxing, People's Republic of China. FAU - Huang, Bing AU - Huang B AD - Department of Anesthesiology and Pain Research Center, The First Hospital of Jiaxing or The Affiliated Hospital of Jiaxing University, Jiaxing, People's Republic of China. FAU - Yao, Ming AU - Yao M AD - Department of Anesthesiology and Pain Research Center, The First Hospital of Jiaxing or The Affiliated Hospital of Jiaxing University, Jiaxing, People's Republic of China. LA - eng PT - Journal Article DEP - 20230305 PL - New Zealand TA - J Pain Res JT - Journal of pain research JID - 101540514 PMC - PMC9997092 OTO - NOTNLM OT - CT guidance OT - Raynaud’s disease OT - chemical thoracic sympathectomy OT - radiofrequency thermocoagulation OT - sympathetic nerve COIS- The authors declare no conflicts of interest in this work. EDAT- 2023/03/14 06:00 MHDA- 2023/03/14 06:01 PMCR- 2023/03/05 CRDT- 2023/03/13 03:53 PHST- 2022/11/30 00:00 [received] PHST- 2023/01/28 00:00 [accepted] PHST- 2023/03/13 03:53 [entrez] PHST- 2023/03/14 06:00 [pubmed] PHST- 2023/03/14 06:01 [medline] PHST- 2023/03/05 00:00 [pmc-release] AID - 398298 [pii] AID - 10.2147/JPR.S398298 [doi] PST - epublish SO - J Pain Res. 2023 Mar 5;16:649-658. doi: 10.2147/JPR.S398298. eCollection 2023. PMID- 42164008 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260521 LR - 20260521 IS - 2042-8812 (Print) IS - 2042-8812 (Electronic) IS - 2042-8812 (Linking) VI - 2026 IP - 5 DP - 2026 May TI - Correction to: Deep inferior epigastric perforator flap breast reconstruction in patients with Raynaud's disease: a case series and literature review. PG - rjag406 LID - 10.1093/jscr/rjag406 [doi] LID - rjag406 AB - [This corrects the article DOI: 10.1093/jscr/rjaf535.]. CI - © The Author(s) 2026. Published by Oxford University Press and JSCR Publishing Ltd. LA - eng PT - Published Erratum DEP - 20260519 PL - England TA - J Surg Case Rep JT - Journal of surgical case reports JID - 101560169 EFR - J Surg Case Rep. 2025 Jul 14;2025(7):rjaf535. doi: 10.1093/jscr/rjaf535. PMID: 40661725 PMC - PMC13183579 EDAT- 2026/05/21 06:31 MHDA- 2026/05/21 06:32 PMCR- 2026/05/19 CRDT- 2026/05/21 04:36 PHST- 2026/05/21 06:32 [medline] PHST- 2026/05/21 06:31 [pubmed] PHST- 2026/05/21 04:36 [entrez] PHST- 2026/05/19 00:00 [pmc-release] AID - rjag406 [pii] AID - 10.1093/jscr/rjag406 [doi] PST - epublish SO - J Surg Case Rep. 2026 May 19;2026(5):rjag406. doi: 10.1093/jscr/rjag406. eCollection 2026 May. PMID- 36788863 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230217 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 1 DP - 2023 Jan TI - Cocaine-Induced Raynaud's Phenomenon: A Case Report. PG - e33604 LID - 10.7759/cureus.33604 [doi] LID - e33604 AB - We illustrate a notable case of a middle-aged male who presents to a community hospital with left third- and fourth-digit discoloration and pain for the past four days. On presentation to the emergency department, a urine drug screen was ordered which showed synthetic cannabinoids, cocaine, and amphetamines. Initial therapy of nitroglycerin paste, oral oxycodone, intravenous Dilaudid®, and aspirin was started, which resulted in decreased subjective pain. The pathophysiology and mechanism of cocaine-induced Raynaud's phenomenon (RP) are discussed. Our purpose in putting forward this case is to acknowledge cocaine use as a cause of secondary RP and to emphasize the importance of early recognition to reduce the occurrence of digital necrosis. CI - Copyright © 2023, Ahn et al. FAU - Ahn, Heong Jin C AU - Ahn HJC AD - Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA. FAU - Tine Iii, Albert AU - Tine Iii A AD - Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA. FAU - Patel, Arsh N AU - Patel AN AD - Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA. FAU - Le, Samantha A AU - Le SA AD - Department of Clinical Research and Quality Improvement, Alabama College of Osteopathic Medicine, Dothan, USA. FAU - Defour, Fulton AU - Defour F AD - Internal Medicine, Thomas Hospital, Fairhope, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230110 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9910812 OTO - NOTNLM OT - cocaine use OT - drug induced vasculitis OT - raynaud disease OT - raynaud’s phenomenon OT - secondary raynaud's COIS- The authors have declared that no competing interests exist. EDAT- 2023/02/16 06:00 MHDA- 2023/02/16 06:01 PMCR- 2023/01/10 CRDT- 2023/02/15 02:04 PHST- 2022/11/25 00:00 [received] PHST- 2023/01/10 00:00 [accepted] PHST- 2023/02/15 02:04 [entrez] PHST- 2023/02/16 06:00 [pubmed] PHST- 2023/02/16 06:01 [medline] PHST- 2023/01/10 00:00 [pmc-release] AID - 10.7759/cureus.33604 [doi] PST - epublish SO - Cureus. 2023 Jan 10;15(1):e33604. doi: 10.7759/cureus.33604. eCollection 2023 Jan. PMID- 31384329 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1792-0981 (Print) IS - 1792-1015 (Electronic) IS - 1792-0981 (Linking) VI - 18 IP - 2 DP - 2019 Aug TI - Immunologic adverse reactions of β-blockers and the skin. PG - 955-959 LID - 10.3892/etm.2019.7504 [doi] AB - β-Blockers are a widely utilised class of medication. They have been in use for a variety of systemic disorders including hypertension, heart failure and intention tremors. Their use in dermatology has garnered growing interest with the discovery of their therapeutic effects in the treatment of haemangiomas, their potential positive effects in wound healing, Kaposi sarcoma, melanoma and pyogenic granuloma, and, more recently, pemphigus. Since β-blockers are deployed in a variety of disorders, which have cutaneous co-morbidities such as psoriasis, their pertinence to dermatologists cannot be overstated. Likewise, β-blockers, like any other drug category, carry risks of side effects, some of which are dermatologic. These include triggering and exacerbation of psoriasis, psoriatic and rheumatoid arthritis, anaphylaxis, contact dermatitis, occupational contact dermatitis, Raynaud's disease, alopecia, lichen planus-like drug eruption, hyperhydrosis and vitiligo. While recent articles have focussed on the positive uses of β-blockers, it may also be wise to call our attention to the potential dermatologic adverse effects that may follow β-blocker use, as well as possible therapeutic approaches to these. This short review will focus on those dermatoses resulting from β-blocker use, which have an immunologic basis. FAU - Tatu, Alin Laurentiu AU - Tatu AL AD - Medical and Pharmaceutical Research Unit/Competitive, Interdisciplinary Research Integrated Platform 'Dunărea de Jos', ReForm-UDJG; Research Centre in the Field of Medical and Pharmaceutical Sciences, Faculty of Medicine and Pharmacy, Department of Pharmaceutical Sciences, 'Dunărea de Jos' University of Galați, 800010 Galati, Romania. FAU - Elisei, Alina Mihaela AU - Elisei AM AD - Medical and Pharmaceutical Research Unit/Competitive, Interdisciplinary Research Integrated Platform 'Dunărea de Jos', ReForm-UDJG; Research Centre in the Field of Medical and Pharmaceutical Sciences, Faculty of Medicine and Pharmacy, Department of Pharmaceutical Sciences, 'Dunărea de Jos' University of Galați, 800010 Galati, Romania. FAU - Chioncel, Valentin AU - Chioncel V AD - Department of Cardio-Thoracic Pathology, Faculty of Medicine, 'Carol Davila' University of Medicine and Phamacy, 050474 Bucharest, Romania. FAU - Miulescu, Magdalena AU - Miulescu M AD - Department of Morphological and Functional Sciences, Faculty of Medicine and Pharmacy, 'Dunarea de Jos University' of Galati, 800010 Galati, Romania. FAU - Nwabudike, Lawrence Chukwudi AU - Nwabudike LC AD - Department of Diabetic Foot Care, 'Prof. N. Paulescu' National Institute of Diabetes, 011233 Bucharest, Romania. LA - eng PT - Journal Article PT - Review DEP - 20190418 PL - Greece TA - Exp Ther Med JT - Experimental and therapeutic medicine JID - 101531947 PMC - PMC6639944 OTO - NOTNLM OT - Raynaud's disease OT - acrocyanosis OT - anaphylaxis OT - contact dermatitis OT - drug-induced psoriasis OT - hyperhydrosis OT - lichen planus-like drug eruption OT - vitiligo OT - β-blockers EDAT- 2019/08/07 06:00 MHDA- 2019/08/07 06:01 PMCR- 2019/04/18 CRDT- 2019/08/07 06:00 PHST- 2018/09/11 00:00 [received] PHST- 2018/11/16 00:00 [accepted] PHST- 2019/08/07 06:00 [entrez] PHST- 2019/08/07 06:00 [pubmed] PHST- 2019/08/07 06:01 [medline] PHST- 2019/04/18 00:00 [pmc-release] AID - ETM-0-0-7504 [pii] AID - 10.3892/etm.2019.7504 [doi] PST - ppublish SO - Exp Ther Med. 2019 Aug;18(2):955-959. doi: 10.3892/etm.2019.7504. Epub 2019 Apr 18. PMID- 39310508 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240924 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 8 DP - 2024 Aug TI - Primary Raynaud's Phenomenon of the Tongue. PG - e67417 LID - 10.7759/cureus.67417 [doi] LID - e67417 AB - Raynaud's phenomenon is a condition characterized by intermittent vasoconstriction of arteries in the fingers and skin, triggered by cold temperatures or emotional stress, aimed at conserving body heat. This condition is classified into primary and secondary forms, with secondary Raynaud's often linked to connective tissue diseases, medications, infections, and occupational exposures. A notable clinical case involves a 51-year-old male experiencing episodes of painful, white discoloration of the tongue, which were managed through a comprehensive diagnostic process, including rheumatological and cardiological evaluations, to rule out connective tissue diseases and cardiac dysfunction. We highlight the complex pathophysiology of Raynaud's, involving vascular, neurogenic, and immune mechanisms. Management strategies focus on lifestyle modifications and pharmacologic treatments, such as calcium channel blockers, to reduce attack frequency and severity. For refractory cases, advanced therapies, including phosphodiesterase inhibitors, intravenous prostaglandins, and surgical sympathectomy, may be considered. Effective diagnosis and individualized treatment are crucial for preventing complications and improving patient outcomes. CI - Copyright © 2024, Basharat et al. FAU - Basharat, Rehman AU - Basharat R AD - Otolaryngology, Stony Brook University, New York, USA. FAU - Riordan, Jacob AU - Riordan J AD - Otolaryngology, Stony Brook University, New York, USA. FAU - Samara, Ghassan AU - Samara G AD - Otolaryngology, Stony Brook University, New York, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240821 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11414999 OTO - NOTNLM OT - primary raynaud's OT - raynaud disease OT - raynaud's phenomena OT - tongue OT - tongue pain COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/09/23 12:48 MHDA- 2024/09/23 12:49 PMCR- 2024/08/21 CRDT- 2024/09/23 06:02 PHST- 2024/08/21 00:00 [accepted] PHST- 2024/09/23 12:49 [medline] PHST- 2024/09/23 12:48 [pubmed] PHST- 2024/09/23 06:02 [entrez] PHST- 2024/08/21 00:00 [pmc-release] AID - 10.7759/cureus.67417 [doi] PST - epublish SO - Cureus. 2024 Aug 21;16(8):e67417. doi: 10.7759/cureus.67417. eCollection 2024 Aug. PMID- 27651971 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160921 LR - 20201001 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2016 DP - 2016 TI - Severe Primary Raynaud's Disease Treated with Rituximab. PG - 2053804 LID - 10.1155/2016/2053804 [doi] LID - 2053804 AB - Raynaud's phenomenon refers to reversible spasms of the peripheral arterioles that can be primary Raynaud's phenomenon (PRP) or secondary Raynaud's phenomenon (SRP) to underlying connective tissue disease, both of which are characterized by a triphasic color response triggered by cold exposure or stress. PRP is typically a benign disease, whereas SRP may progress into digital ulcers and/or gangrene. Here, we report a case of a 55-year-old female diagnosed with PRP 7 years ago. Treatment with first-line agents, including calcium channel blocker, aspirin, and phosphodiesterase inhibitor, did not control her symptoms, which progressed to digital ulceration and gangrene. There were no symptoms of underlying autoimmune disease or malignancy, and autoimmune, serology, and immunology test results were normal; a biopsy of her left little finger was negative for vasculitis. Development to critical digital ischemia necessitated treatment with intravenous iloprost and heparin infusion followed by angioplasty, which led to a partial improvement. Due to persistent symptoms, rituximab therapy was initiated and two cycles induced a complete resolution of symptoms. FAU - Shabrawishi, Mohammed AU - Shabrawishi M AUID- ORCID: 0000-0002-0050-2698 AD - Department of Internal Medicine, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia. FAU - Albeity, Abdurahman AU - Albeity A AUID- ORCID: 0000-0001-5415-6383 AD - Department of Internal Medicine, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia. FAU - Almoallim, Hani AU - Almoallim H AD - Department of Internal Medicine, Medical College, Umm Alqura University, Makkah, Saudi Arabia. LA - eng PT - Journal Article DEP - 20160829 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC5019931 EDAT- 2016/09/22 06:00 MHDA- 2016/09/22 06:01 PMCR- 2016/08/29 CRDT- 2016/09/22 06:00 PHST- 2016/05/08 00:00 [received] PHST- 2016/08/09 00:00 [accepted] PHST- 2016/09/22 06:00 [entrez] PHST- 2016/09/22 06:00 [pubmed] PHST- 2016/09/22 06:01 [medline] PHST- 2016/08/29 00:00 [pmc-release] AID - 10.1155/2016/2053804 [doi] PST - ppublish SO - Case Rep Rheumatol. 2016;2016:2053804. doi: 10.1155/2016/2053804. Epub 2016 Aug 29. PMID- 32582494 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 12 IP - 5 DP - 2020 May 22 TI - The Effect of Abobotulinum Toxin A on the Symptoms of Raynaud's Phenomenon: A Case Series. PG - e8235 LID - 10.7759/cureus.8235 [doi] LID - e8235 AB - Raynaud's phenomenon (RP) is a relatively common syndrome occurring alone or in combination with autoimmune and inflammatory diseases. It is characterized by pain and ulceration due to vasospasm in response to cold and stress, most often affecting the digits. Although pharmacologic treatment for this condition exists, it is not always efficacious. Our case series demonstrates the use of abobotulinum toxin A in the treatment of RP. We report the cases of four patients who received injections of abobotulinum toxin A to treat their mild to severe RP symptoms. They experienced clinical improvement for up to one year after treatment. CI - Copyright © 2020, Winter et al. FAU - Winter, Amelia R AU - Winter AR AD - Internal Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Camargo Macias, Kathlyn AU - Camargo Macias K AD - Internal Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Kim, Sun AU - Kim S AD - Internal Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Sami, Naveed AU - Sami N AD - Internal Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Weinstein, David AU - Weinstein D AD - Dermatology, University of Central Florida College of Medicine, Orlando, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200522 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7306670 OTO - NOTNLM OT - abobotulinum toxin a OT - botulinum toxin OT - case series OT - raynaud disease COIS- The authors have declared that no competing interests exist. EDAT- 2020/06/26 06:00 MHDA- 2020/06/26 06:01 PMCR- 2020/05/22 CRDT- 2020/06/26 06:00 PHST- 2020/06/26 06:00 [entrez] PHST- 2020/06/26 06:00 [pubmed] PHST- 2020/06/26 06:01 [medline] PHST- 2020/05/22 00:00 [pmc-release] AID - 10.7759/cureus.8235 [doi] PST - epublish SO - Cureus. 2020 May 22;12(5):e8235. doi: 10.7759/cureus.8235. PMID- 41390232 OWN - NLM STAT- MEDLINE DCOM- 20251213 LR - 20251219 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 21 IP - 10 DP - 2025 Dec TI - Nailfold capillaroscopy in inflammatory bowel disease: A shared approach in gastroenterology and rheumatology. PG - 501998 LID - S2173-5743(25)00175-3 [pii] LID - 10.1016/j.reumae.2025.501998 [doi] AB - INTRODUCTION AND OBJECTIVES: Inflammatory bowel disease (IBD) has systemic consequences that extend beyond the gastrointestinal tract. While nailfold capillaroscopy (NFC) is widely utilized in many rheumatological disorders, its significance in evaluating microvascular changes in IBD remains unknown. This study aimed to standardize NFC use in IBD patients and relate NFC results to clinical and laboratory criteria. METHODS: This observational case-control study included histology- and colonoscopy-diagnosed IBD patients. We performed NFC on eight fingers of each subject using a Dino-Lite digital microscope, adhering to European Alliance of Associations for Rheumatology guidelines. Capillary characteristics, including density, morphology, and dimensions, as well as microhemorrhages, were assessed at 200×. RESULTS: The study included 50 IBD patients and 30 healthy controls. IBD patients had significantly lower capillary density (7.52±0.68capillaries/mm) than controls (8.18±0.63capillaries/mm, p<0.001), with 26% of IBD patients exhibiting densities below seven capillaries/mm. In IBD patients, microhemorrhages were more prevalent than in controls (p=0.032). Raynaud's phenomenon was more commonly detected in patients with low mean capillary density (p=0.04). CONCLUSION: IBD patients had reduced mean capillary density and increased microhemorrhages. These changes suggested that NFC could be a non-invasive way provides unique insights into IBD microvascular health. . CI - Copyright © 2025. Published by Elsevier España S.L.U. FAU - Abdelrahman, Maha S I AU - Abdelrahman MSI AD - Department of Rheumatology, Rehabilitation and Physical Medicine, Faculty of Medicine, Assiut University, Assiut, Egypt. Electronic address: mahasayed@aun.edu.eg. FAU - Abdeltawab, Doaa AU - Abdeltawab D AD - Department of Tropical Medicine and Gastroenterology, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Hamed Shehata, Rasha AU - Hamed Shehata R AD - Department of Tropical Medicine and Gastroenterology, Faculty of Medicine, Assiut University, Assiut, Egypt. LA - eng PT - Journal Article PT - Observational Study PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Case-Control Studies MH - Female MH - Male MH - *Inflammatory Bowel Diseases/diagnostic imaging/complications MH - Adult MH - Middle Aged MH - *Nails/blood supply MH - Capillaries/pathology/diagnostic imaging MH - Rheumatology MH - Gastroenterology OTO - NOTNLM OT - Angioscopia microscópica OT - Autoimmune disease OT - Capilares OT - Capillaries OT - Enfermedad autoinmune OT - Enfermedad de Raynaud OT - Enfermedades inflamatorias intestinales OT - Inflammatory bowel diseases OT - Microscopic angioscopy OT - Raynaud's disease EDAT- 2025/12/14 00:39 MHDA- 2025/12/14 00:40 CRDT- 2025/12/13 20:56 PHST- 2025/04/19 00:00 [received] PHST- 2025/09/15 00:00 [revised] PHST- 2025/10/10 00:00 [accepted] PHST- 2025/12/14 00:40 [medline] PHST- 2025/12/14 00:39 [pubmed] PHST- 2025/12/13 20:56 [entrez] AID - S2173-5743(25)00175-3 [pii] AID - 10.1016/j.reumae.2025.501998 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2025 Dec;21(10):501998. doi: 10.1016/j.reumae.2025.501998. PMID- 34085754 OWN - NLM STAT- MEDLINE DCOM- 20220118 LR - 20220122 IS - 1742-481X (Electronic) IS - 1742-4801 (Print) IS - 1742-4801 (Linking) VI - 19 IP - 2 DP - 2022 Feb TI - Segmental branches emanating from saphenous nerve morphing into sympathetic trunks for innervation of saphenous artery and its clinical implication for arterial sympathectomy. PG - 294-304 LID - 10.1111/iwj.13630 [doi] AB - Sympathectomy of arteries has been adopted for the treatment of peripheral arterial disease and Raynaud's disease. However, the exact route for sympathetic axons to reach peripheral arteries awaits further investigation that could pave the way for development of new surgical strategies. In this study, saphenous neurovascular bundles from 10 neonatal Sprague-Dawley rats first were harvested for whole-mount immunostaining to show sympathetic innervation pattern of the artery. Secondly, 40 Sprague-Dawley male rats weighing 350 to 400 g were assigned to five groups, receiving either sham, perivascular sympathectomy, nerve-artery separation, nerve transection in the saphenous neurovascular bundle, or lumbar sympathectomy surgery that removes the lumbar sympathetic trunks. Immediately after surgery, the arterial perfusion and diameter were measured using laser speckling contrast imaging, and 1 week later the saphenous neurovascular bundles were harvested for immunostaining using antibodies against TH, neuron-specific β-tubulin (Tuj 1), and α-SMA to show the presence or absence of the TH-immuopositive staining in the adventitia. The differences among the five groups were determined using one-way analysis of variance (ANOVA). We found that an average of 2.8 ± 0.8 branches with a diameter of 4.8 ± 1.2 μm derived from the saphenous nerve that morphed into a primary and a secondary sympathetic trunk for innervation of the saphenous artery. Nerve-artery separation, nerve transection, and lumbar sympathectomy could eradicate TH-immunopositive staining of the artery, resulting, respectively, in a 12%, 36%, and 59% increase in diameter (P < .05), and a 52%, 63%, and 201% increase in perfusion compared with sham surgery (P < .01). In contrast, perivascular sympathectomy did not have a significant impact on the TH-immunopositive staining, the diameter, and perfusion of the distal part of the artery (P > .05). We conclude that the sympathetic innervation of an artery derives from segmental branches given off from its accompanying nerve. Nerve-artery disconnection is a theoretic option in sympathectomy of an artery. CI - © 2021 The Authors. International Wound Journal published by Medicalhelplines.com Inc (3M) and John Wiley & Sons Ltd. FAU - Xie, Yun AU - Xie Y AD - Orthopedic Department, First Affiliated Hospital, Fujian Medical University, Fuzhou, China. FAU - Fang, Fang AU - Fang F AD - Department of Pharmacology, Fujian Medical University, Fuzhou, China. FAU - Lin, Peisen AU - Lin P AD - Orthopedic Department, First Affiliated Hospital, Fujian Medical University, Fuzhou, China. FAU - Zhang, Zhiming AU - Zhang Z AD - Department of Anesthesiology, Chenzhou No. 1 People's Hospital, University of South China, Chenzhou, China. FAU - Zhuang, Yuehong AU - Zhuang Y AUID- ORCID: 0000-0003-3997-7663 AD - Orthopedic Department, First Affiliated Hospital, Fujian Medical University, Fuzhou, China. AD - Fujian Key Laboratory of Brain Aging and Neurodegenerative Diseases, Institute of Clinical Applied Anatomy, Fujian Medical University, Fuzhou, China. LA - eng GR - 2020J01625/Fujian Natural Science Foundation/ GR - 2019JJ40010/Hunan Natural Science Foundation/ PT - Journal Article DEP - 20210604 PL - England TA - Int Wound J JT - International wound journal JID - 101230907 SB - IM MH - Animals MH - Arteries MH - Male MH - Rats MH - Rats, Sprague-Dawley MH - *Sympathectomy MH - *Sympathetic Nervous System/surgery PMC - PMC8762570 OTO - NOTNLM OT - Raynaud's disease OT - arterial innervation OT - peripheral arterial disease OT - sympathectomy OT - sympathetic nerve COIS- The authors have no conflict of interest to declare. EDAT- 2021/06/05 06:00 MHDA- 2022/01/19 06:00 PMCR- 2021/06/04 CRDT- 2021/06/04 09:09 PHST- 2021/04/19 00:00 [received] PHST- 2021/05/25 00:00 [accepted] PHST- 2021/06/05 06:00 [pubmed] PHST- 2022/01/19 06:00 [medline] PHST- 2021/06/04 09:09 [entrez] PHST- 2021/06/04 00:00 [pmc-release] AID - IWJ13630 [pii] AID - 10.1111/iwj.13630 [doi] PST - ppublish SO - Int Wound J. 2022 Feb;19(2):294-304. doi: 10.1111/iwj.13630. Epub 2021 Jun 4. PMID- 36035460 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220830 IS - 2090-6986 (Print) IS - 2090-6994 (Electronic) IS - 2090-6994 (Linking) VI - 2022 DP - 2022 TI - Lingual Raynaud's Phenomenon after Surgical and Radiotherapeutic Intervention for Oral Squamous Cell Carcinoma. PG - 1567581 LID - 10.1155/2022/1567581 [doi] LID - 1567581 AB - Raynaud's phenomenon of the tongue after radiation therapy with or without chemotherapy is an exceedingly rare complication. Symptoms are similar to Raynaud's disease of other sites and involve pallor and discomfort on exposure to cold temperatures that resolve with rewarming. Presentation occurs approximately 18-24 months after radiotherapy on average and can usually be managed effectively with lifestyle modification and pharmacotherapy. Here, we present a case of lingual Raynaud's following surgery and adjuvant radiation therapy in a patient with squamous cell carcinoma of the oral cavity. CI - Copyright © 2022 Nicholas J. Murphy et al. FAU - Murphy, Nicholas J AU - Murphy NJ AUID- ORCID: 0000-0002-9265-994X AD - Wayne State University School of Medicine, 540 E. Canfield Ave. Detroit, MI 48201, USA. FAU - Kabbani, Loay S AU - Kabbani LS AD - Department of Surgery, Henry Ford Hospital, 2799 W Grand Blvd Detroit, MI 48202, USA. FAU - Shepard, Alexander D AU - Shepard AD AD - Department of Surgery, Henry Ford Hospital, 2799 W Grand Blvd Detroit, MI 48202, USA. FAU - Siddiqui, Farzan AU - Siddiqui F AUID- ORCID: 0000-0003-1547-835X AD - Department of Radiation Oncology, Henry Ford Hospital, 2799 W Grand Blvd Detroit, MI 48202, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20220819 PL - United States TA - Case Rep Vasc Med JT - Case reports in vascular medicine JID - 101585357 PMC - PMC9417770 COIS- The authors declare that there is no conflict of interest regarding the publication of this article. EDAT- 2022/08/30 06:00 MHDA- 2022/08/30 06:01 PMCR- 2022/08/19 CRDT- 2022/08/29 05:24 PHST- 2022/06/04 00:00 [received] PHST- 2022/08/09 00:00 [accepted] PHST- 2022/08/29 05:24 [entrez] PHST- 2022/08/30 06:00 [pubmed] PHST- 2022/08/30 06:01 [medline] PHST- 2022/08/19 00:00 [pmc-release] AID - 10.1155/2022/1567581 [doi] PST - epublish SO - Case Rep Vasc Med. 2022 Aug 19;2022:1567581. doi: 10.1155/2022/1567581. eCollection 2022. PMID- 36452251 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221202 IS - 2052-4374 (Print) IS - 2052-4374 (Electronic) IS - 2052-4374 (Linking) VI - 34 DP - 2022 TI - Technetium-99m hand perfusion scintigraphy (Raynaud's scan) as a method of verification in hand arm vibration syndrome: a review. PG - e26 LID - 10.35371/aoem.2022.34.e26 [doi] LID - e26 AB - It is important to assess the blood flow of fingers in the verification of hand-arm vibration syndrome. In the Republic of Korea, most assessments of the blood flow in the fingers are performed using a cold provocation test with finger skin color change. However, this test is a non-objective method with a relatively low sensitivity, leading to possible social and legal problems. Thus, we reviewed the characteristics of several tests that assess the blood flow in the fingers. Among these tests, using the radioactive isotope method, Raynaud's scan has a relatively higher sensitivity and specificity than other tests, provides objective results, and is approachable in many hospitals. So we suggest using Raynaud's scan as an alternative test when cold provocation test with finger skin color change is negative in vibration exposed worker. CI - Copyright © 2022 Korean Society of Occupational & Environmental Medicine. FAU - Ha, Taewoong AU - Ha T AUID- ORCID: 0000-0003-4680-3290 AD - Department of Occupational and Environmental Medicine, Kosin University Gospel Hospital, Busan, Korea. FAU - Oh, Hyeoncheol AU - Oh H AUID- ORCID: 0000-0003-2360-2787 AD - Department of Occupational and Environmental Medicine, Kosin University Gospel Hospital, Busan, Korea. FAU - Kim, Jungwon AU - Kim J AUID- ORCID: 0000-0003-3836-8890 AD - Department of Occupational and Environmental Medicine, Kosin University Gospel Hospital, Busan, Korea. AD - Department of Occupational and Environmental Medicine, Kosin University College of Medicine, Busan, Korea. LA - eng PT - Journal Article PT - Review DEP - 20221011 PL - Korea (South) TA - Ann Occup Environ Med JT - Annals of occupational and environmental medicine JID - 101609244 PMC - PMC9685291 OTO - NOTNLM OT - Cold temperature OT - Hand-arm vibration syndrome OT - Radionuclide imaging OT - Raynaud disease COIS- Competing interests: The authors declare that they have no competing interests. EDAT- 2022/12/02 06:00 MHDA- 2022/12/02 06:01 PMCR- 2022/10/11 CRDT- 2022/12/01 02:41 PHST- 2022/04/14 00:00 [received] PHST- 2022/07/10 00:00 [revised] PHST- 2022/09/07 00:00 [revised] PHST- 2022/09/11 00:00 [accepted] PHST- 2022/12/01 02:41 [entrez] PHST- 2022/12/02 06:00 [pubmed] PHST- 2022/12/02 06:01 [medline] PHST- 2022/10/11 00:00 [pmc-release] AID - 10.35371/aoem.2022.34.e26 [doi] PST - epublish SO - Ann Occup Environ Med. 2022 Oct 11;34:e26. doi: 10.35371/aoem.2022.34.e26. eCollection 2022. PMID- 36819449 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230224 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 1 DP - 2023 Jan TI - Worsening of Primary Raynaud's Phenomenon During Episodes of Pyrexia and Rigors in SARS-CoV-2 Infection. PG - e33781 LID - 10.7759/cureus.33781 [doi] LID - e33781 AB - Acute SARS-CoV-2 infection is associated with several cutaneous manifestations, including vasculitic digital ischemia. The reversal of digital ischemia in the primary Raynaud phenomenon (RP) arises from endothelial hypersensitivity to circulating adrenaline and noradrenaline and diminished vasodilatory innervation. SARS-CoV-2 can infect endothelial cells and be associated with raised adrenaline levels, reduced microvascular dilatory responses, and exaggerated clotting mechanisms. We report worsened RP in a patient with previously mild winter-time primary RP during the periods of fever and rigors from an acute SARS-CoV-2 infection contracted in a warm summer period. This reverted to the mild phenotype after recovery, and we discussed possible mechanisms for the brief exacerbation. CI - Copyright © 2023, Bansal et al. FAU - Bansal, Ciara J AU - Bansal CJ AD - Rheumatology, Haematology, Royal Melbourne Hospital, Melbourne, AUS. FAU - Kamel, Kirollos AU - Kamel K AD - Haematology, Royal Melbourne Hospital, Melbourne, AUS. LA - eng PT - Case Reports PT - Journal Article DEP - 20230114 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9928217 OTO - NOTNLM OT - coronavirus OT - coronavirus disease OT - covid-19 OT - digital ischemia OT - mechanism OT - pyrexia OT - raynaud disease OT - raynaud phenomenon OT - rigors OT - sars-cov-2 COIS- The authors have declared that no competing interests exist. EDAT- 2023/02/24 06:00 MHDA- 2023/02/24 06:01 PMCR- 2023/01/14 CRDT- 2023/02/23 10:03 PHST- 2023/01/10 00:00 [accepted] PHST- 2023/02/23 10:03 [entrez] PHST- 2023/02/24 06:00 [pubmed] PHST- 2023/02/24 06:01 [medline] PHST- 2023/01/14 00:00 [pmc-release] AID - 10.7759/cureus.33781 [doi] PST - epublish SO - Cureus. 2023 Jan 14;15(1):e33781. doi: 10.7759/cureus.33781. eCollection 2023 Jan. PMID- 25902774 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150424 LR - 20150423 IS - 1044-7946 (Print) IS - 1044-7946 (Linking) VI - 21 IP - 11 DP - 2009 Nov TI - Use of Equine Derived Pericardium as a Biological Cover To Promote Closure of a Complicated Wound With Associated Scleroderma and Raynaud's Disease . PG - 297-301 AB - A 39-year-old man with previously undiagnosed scleroderma was admitted to the UCSD Medical Center with bilateral, limb-threatening necrotic lower extremity ulcers extending to underlying fascia and muscle. Rather than amputate the extremities, the patient requested alternative treatment and underwent extensive tissue debridement followed by placement of an equine pericardium xenograft. Subsequent to treatment, the patient underwent weekly examinations and dressing changes without additional treatment. The patient was ambulating without assistance and with complete closure of all wounds in 10 weeks. The patient remained without wound recurrence at a recent 6-month follow-up visit . FAU - Mulder, Gerit AU - Mulder G AD - UCSD Medical Center, San Diego, California; Email:gmulder@ucsd.edu. FAU - Lee, Daniel K AU - Lee DK LA - eng PT - Journal Article PL - United States TA - Wounds JT - Wounds : a compendium of clinical research and practice JID - 9010276 EDAT- 2009/11/01 00:00 MHDA- 2009/11/01 00:01 CRDT- 2015/04/24 06:00 PHST- 2015/04/24 06:00 [entrez] PHST- 2009/11/01 00:00 [pubmed] PHST- 2009/11/01 00:01 [medline] PST - ppublish SO - Wounds. 2009 Nov;21(11):297-301. PMID- 20842555 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1534-3189 (Electronic) IS - 1092-8464 (Linking) VI - 12 IP - 2 DP - 2010 Apr TI - Management of Raynaud's Phenomenon in the Patient with Connective Tissue Disease. PG - 185-204 LID - 10.1007/s11936-010-0065-x [doi] AB - Raynaud's phenomenon is characterized by intense vasospasm of digital arteries on cold exposure or with emotional stress, leading to well-defined color changes of digital skin. It may be primary (Raynaud's disease) or secondary to an underlying condition, including autoimmune rheumatic diseases. Although Raynaud's disease is predominantly a vasospastic condition, Raynaud's phenomenon in connective tissue diseases often is a result of an underlying vaso-occlusive process. As a result, the manifestations are more severe and persistent and often warrant pharmacologic therapy. Dihydropyridine calcium channel blockers are by far the most commonly studied and prescribed class of agents for the treatment of Raynaud's phenomenon. There is some evidence for the efficacy of other classes of drugs, such as topical nitrates, α-antagonists, angiotensin receptor blockers, selective serotonin reuptake inhibitors, and pentoxifylline. However, the data on the efficacy of these agents are not as convincing, and they are not proven to be more effective than calcium channel blockers. Hence, their place in the therapy of Raynaud's phenomenon is limited to patients who fail to respond adequately to or are unable to tolerate calcium channel blockers. More expensive second-line agents, such as phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and intravenous prostanoids, are reserved for refractory cases of secondary Raynaud's phenomenon with severe digital ischemia leading to ulceration or gangrene. These drugs may be used in isolation or as adjunct therapy to the first-line agents. Chemical and/or surgical sympathectomy may be considered if sympathetically driven digital ischemia is severe and resistant to pharmacologic intervention. These procedures may temporarily reverse the digital ischemia and help tide over the crisis, whereas the improvement thus achieved can be maintained by continuing medical therapy. In cases of ischemic digital ulceration, it is important to achieve adequate analgesia and to identify and treat superadded infection. FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatology Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, Cleveland, OH, 44195, USA, chattes@ccf.org. LA - eng PT - Journal Article PL - United States TA - Curr Treat Options Cardiovasc Med JT - Current treatment options in cardiovascular medicine JID - 9815942 EDAT- 2010/09/16 06:00 MHDA- 2010/09/16 06:01 CRDT- 2010/09/16 06:00 PHST- 2010/09/16 06:00 [entrez] PHST- 2010/09/16 06:00 [pubmed] PHST- 2010/09/16 06:01 [medline] AID - 10.1007/s11936-010-0065-x [doi] PST - ppublish SO - Curr Treat Options Cardiovasc Med. 2010 Apr;12(2):185-204. doi: 10.1007/s11936-010-0065-x. PMID- 37547779 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230808 IS - 1735-8949 (Print) IS - 1735-9392 (Electronic) IS - 1735-9392 (Linking) VI - 17 IP - 2 DP - 2023 Jun TI - Raynaud Phenomenon of the Nipple: A Clinical Case Report. PG - 113-115 LID - 10.18502/jfrh.v17i2.12876 [doi] AB - OBJECTIVE: In Raynaud's phenomenon of the nipple there is a change in color, accompanied by pain or discomfort during breastfeeding. CASE REPORT: A 29-years old female patient, breastfeeding, develops a severe bilateral nipple pain during and after breastfeeding and biphasic change in nipple color, with difficulties in the breastfeeding technique. She was medicated with nifedipine and recommended application of warm compresses to the nipples and use of electric breast pump, showing complete resolution after four weeks of treatment. CONCLUSION: Raynaud's phenomenon of the nipple should be considered in breastfeeding women who report nipple pain or discomfort. In clinical practice, nipple pain is a very frequent complaint, and responsible for many cases of early abandonment of breastfeeding. It is therefore essential to make an early diagnosis and implement a correct and immediate treatment. CI - Copyright © 2023 Tehran University of Medical Sciences. Published by Tehran University of Medical Sciences. FAU - Quental, Carolina AU - Quental C AD - Family Health Unit, Prelada, Porto, Portugal. FAU - Brito, Daniel Bertoluci AU - Brito DB AD - Family Health Unit, Espaço Saúde, Porto, Portugal. FAU - Sobral, João AU - Sobral J AD - Family Health Unit, Baltar, Paredes, Portugal. FAU - Macedo, Ana Mafalda AU - Macedo AM AD - Family Health Unit, Prelada, Porto, Portugal. LA - eng PT - Case Reports PT - Journal Article PL - Iran TA - J Family Reprod Health JT - Journal of family & reproductive health JID - 101496684 PMC - PMC10397528 OTO - NOTNLM OT - Breast Feeding OT - Nifedipine OT - Nipples OT - Raynaud Disease OT - Vasoconstriction COIS- Conflict of Interests Authors declare no conflict of interests. EDAT- 2023/08/07 06:41 MHDA- 2023/08/07 06:42 PMCR- 2023/06/01 CRDT- 2023/08/07 04:58 PHST- 2023/08/07 06:42 [medline] PHST- 2023/08/07 06:41 [pubmed] PHST- 2023/08/07 04:58 [entrez] PHST- 2023/06/01 00:00 [pmc-release] AID - JFRH-17-113 [pii] AID - 10.18502/jfrh.v17i2.12876 [doi] PST - ppublish SO - J Family Reprod Health. 2023 Jun;17(2):113-115. doi: 10.18502/jfrh.v17i2.12876. PMID- 27478469 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160801 LR - 20220408 IS - 1734-1922 (Print) IS - 1896-9151 (Electronic) IS - 1734-1922 (Linking) VI - 12 IP - 4 DP - 2016 Aug 1 TI - Botulinum toxin A in the treatment of Raynaud's phenomenon: a systematic review. PG - 864-70 LID - 10.5114/aoms.2015.48152 [doi] AB - INTRODUCTION: The management of Raynaud's phenomenon in its most severe form is challenging, and current medical and surgical treatment methods frequently do not lead to optimal symptom control and prevention of ischemic complications. The aim of the study was to critically evaluate all existing evidence on the use of botulinum toxin A in the management of Raynaud's phenomenon. MATERIAL AND METHODS: We adopted the PRISMA methodology and searched Cochrane Library, MEDLINE, SCOPUS, EULAR and ACR congresses abstract archives for Raynaud* AND botulinum toxin OR onabotulinum. All studies that contained reports of botulinum toxin A use and its outcome in Raynaud's phenomenon were included in the review. RESULTS: Eleven studies met our inclusion criteria and involved a total of 125 patients. Two reviewers extracted data from the studies under review and achieved a consensus in their selection. The main outcomes measured were pain reduction and healing of digital ulcers. The level of evidence across studies was very low to moderate. CONCLUSIONS: There is insufficient evidence to assess the efficacy of botulinum toxin A in Raynaud's phenomenon. Despite many promising reports, further research in the form of randomized controlled trials is warranted in order to investigate this new treatment method for Raynaud's phenomenon. FAU - Żebryk, Paweł AU - Żebryk P AD - Department of Rheumatology and Internal Medicine, Poznan University of Medical Sciences, Poznan, Poland. FAU - Puszczewicz, Mariusz J AU - Puszczewicz MJ AD - Department of Rheumatology and Internal Medicine, Poznan University of Medical Sciences, Poznan, Poland. LA - eng PT - Journal Article DEP - 20150108 PL - Poland TA - Arch Med Sci JT - Archives of medical science : AMS JID - 101258257 PMC - PMC4947604 OTO - NOTNLM OT - Raynaud disease/etiology/therapy OT - botulinum toxins OT - scleroderma OT - systemic/*therapy OT - type A/therapeutic use OT - vasodilator agents/therapeutic use EDAT- 2016/08/02 06:00 MHDA- 2016/08/02 06:01 PMCR- 2016/08/01 CRDT- 2016/08/02 06:00 PHST- 2014/07/29 00:00 [received] PHST- 2014/08/13 00:00 [accepted] PHST- 2016/08/02 06:00 [entrez] PHST- 2016/08/02 06:00 [pubmed] PHST- 2016/08/02 06:01 [medline] PHST- 2016/08/01 00:00 [pmc-release] AID - 24344 [pii] AID - 10.5114/aoms.2015.48152 [doi] PST - ppublish SO - Arch Med Sci. 2016 Aug 1;12(4):864-70. doi: 10.5114/aoms.2015.48152. Epub 2015 Jan 8. PMID- 35585632 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 6 IP - 1 DP - 2022 May 19 TI - Raynaud's phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report. PG - 26 LID - 10.1186/s41927-022-00258-y [doi] LID - 26 AB - BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis. CASE PRESENTATION: A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud's phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain-Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud's phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud's phenomenon, acrocyanosis, and white nails, but the neurological response was partial. CONCLUSIONS: POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud's phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud's phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation. CI - © 2022. The Author(s). FAU - Torres-Saavedra, Fabio AU - Torres-Saavedra F AUID- ORCID: 0000-0003-0385-8160 AD - Division of Rheumatology, GRUA Investigation Group, Universidad de Antioquia, 050010, Medellín, Colombia. fabio.torressav@gmail.com. AD - IPS Artmedica, Medellín, Colombia. fabio.torressav@gmail.com. FAU - León-Sierra, Lina AU - León-Sierra L AD - Internal Medicine, Universidad Nacional de Colombia, Bogotá, Colombia. LA - eng PT - Journal Article DEP - 20220519 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC9118802 OTO - NOTNLM OT - Autoimmune disease OT - Case report OT - POEMS syndrome OT - Paraneoplastic polyneuropathy OT - Raynaud disease COIS- The authors declare no competing interests. EDAT- 2022/05/19 06:00 MHDA- 2022/05/19 06:01 PMCR- 2022/05/19 CRDT- 2022/05/18 23:45 PHST- 2021/12/04 00:00 [received] PHST- 2022/03/08 00:00 [accepted] PHST- 2022/05/18 23:45 [entrez] PHST- 2022/05/19 06:00 [pubmed] PHST- 2022/05/19 06:01 [medline] PHST- 2022/05/19 00:00 [pmc-release] AID - 10.1186/s41927-022-00258-y [pii] AID - 258 [pii] AID - 10.1186/s41927-022-00258-y [doi] PST - epublish SO - BMC Rheumatol. 2022 May 19;6(1):26. doi: 10.1186/s41927-022-00258-y. PMID- 40520078 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250617 IS - 2050-313X (Print) IS - 2050-313X (Electronic) IS - 2050-313X (Linking) VI - 13 DP - 2025 TI - Upadacitinib as a potential management option for diffuse cutaneous systemic sclerosis: A case report. PG - 2050313X251343300 LID - 10.1177/2050313X251343300 [doi] LID - 2050313X251343300 AB - Systemic sclerosis (SSc) is a complex disease involving vasculopathy, immune dysfunction, and fibrosis, with varied clinical presentations that complicate treatment standardization. It often affects multiple organs, including the skin, lungs, gastrointestinal tract, and kidneys. We present a 52-year-old woman with a 14-year history of diffuse cutaneous SSc with severe, treatment-resistant manifestations. She had Raynaud's disease with digital ulceration and auto-amputation, telangiectasias, sclerodactyly, esophageal scleroderma, interstitial lung disease, and extensive calcinosis requiring multiple surgeries. Her disease remained poorly controlled despite treatment with nintedanib, sevelamer, colchicine, tadalafil, and prior immunosuppressants such as prednisone and mycophenolate mofetil. We initiated a trial of upadacitinib which resulted in improved vascular and cutaneous symptoms. Overall, upadacitinib provided meaningful clinical benefits despite her refractory, multisystem disease. CI - © The Author(s) 2025. FAU - Sarfaraz, Sidra AU - Sarfaraz S AD - Division of Dermatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada. FAU - Chang, Janis AU - Chang J AD - Division of Dermatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada. FAU - Kirchhof, Mark G AU - Kirchhof MG AUID- ORCID: 0000-0002-4015-8377 AD - Division of Dermatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada. LA - eng PT - Case Reports PT - Journal Article DEP - 20250612 PL - England TA - SAGE Open Med Case Rep JT - SAGE open medical case reports JID - 101638686 PMC - PMC12163272 OTO - NOTNLM OT - Janus kinase inhibitors OT - scleroderma OT - systemic sclerosis OT - upadacitinib COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/06/16 12:27 MHDA- 2025/06/16 12:28 PMCR- 2025/06/12 CRDT- 2025/06/16 06:36 PHST- 2025/02/17 00:00 [received] PHST- 2025/02/22 00:00 [accepted] PHST- 2025/06/16 12:28 [medline] PHST- 2025/06/16 12:27 [pubmed] PHST- 2025/06/16 06:36 [entrez] PHST- 2025/06/12 00:00 [pmc-release] AID - 10.1177_2050313X251343300 [pii] AID - 10.1177/2050313X251343300 [doi] PST - epublish SO - SAGE Open Med Case Rep. 2025 Jun 12;13:2050313X251343300. doi: 10.1177/2050313X251343300. eCollection 2025. PMID- 34745793 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211110 IS - 2169-7574 (Print) IS - 2169-7574 (Electronic) IS - 2169-7574 (Linking) VI - 9 IP - 11 DP - 2021 Nov TI - The Use of a Neurotized Arterio-venous Flow-through Flap for Concurrent Pulp Revascularization and Reconstruction. PG - e3894 LID - 10.1097/GOX.0000000000003894 [doi] LID - e3894 AB - Digital fingertip soft tissue defects requiring both reconstruction and revascularization pose challenges to the reconstructive surgeon. Traditional options, including terminalization, vein graft and cross-finger flap, and free flow-through flaps, maybe unsuitable or unavailable, with potential for significant donor site morbidity. Venous free flaps rely on venous circulation alone, with no sacrifice of an artery. We present a unique case of a self-employed tradesman with Raynaud's disease, with four-finger injury, and three-finger ischemia for whom we performed a neurotized arterialized venous flow-through flap to revascularize and reconstruct a pulp defect (with a concomitant vessel gap of 2 cm). After allowing for a period of intrinsic delay, the neurotized arterialized venous flow-through flap was inset after 10 days. The flap survived and the patient began to return to his activities within a month of the injury. CI - Copyright © 2021 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. FAU - Khajuria, Ankur AU - Khajuria A AD - Kellogg College, University of Oxford, Oxford, UK. AD - Department of Plastic Surgery, Queen Victoria Hospital, East Grinstead, UK. FAU - Sethu, Arun AU - Sethu A AD - Department of Plastic Surgery, Queen Victoria Hospital, East Grinstead, UK. FAU - Kannan, Ruben Y AU - Kannan RY AD - Department of Plastic Surgery, Queen Victoria Hospital, East Grinstead, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20211102 PL - United States TA - Plast Reconstr Surg Glob Open JT - Plastic and reconstructive surgery. Global open JID - 101622231 PMC - PMC8563065 EDAT- 2021/11/09 06:00 MHDA- 2021/11/09 06:01 PMCR- 2021/11/02 CRDT- 2021/11/08 06:45 PHST- 2021/06/18 00:00 [received] PHST- 2021/09/07 00:00 [accepted] PHST- 2021/11/08 06:45 [entrez] PHST- 2021/11/09 06:00 [pubmed] PHST- 2021/11/09 06:01 [medline] PHST- 2021/11/02 00:00 [pmc-release] AID - 10.1097/GOX.0000000000003894 [doi] PST - epublish SO - Plast Reconstr Surg Glob Open. 2021 Nov 2;9(11):e3894. doi: 10.1097/GOX.0000000000003894. eCollection 2021 Nov. PMID- 31259013 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240717 IS - 2153-4063 (Print) IS - 2153-4063 (Electronic) VI - 2019 DP - 2019 TI - A Literature Based Discovery Visualization System with Hierarchical Clustering and Linking Set Associations. PG - 582-591 AB - Literature Based discovery (LBD) seeks to find information implicit in text, but never explicitly stated. In this work, we develop a method of visually summarizing LBD output in an automatically generated tree structure. This structure promotes a comprehensive understanding of LBD output as a whole, and encourages the user to explore branches of the hierarchy they find most interesting or surprising. This novel visualization system requires the development and integration of automatic functional group discovery, set associations, and linking set associations. Specifically, we perform hierarchical clustering on the potential discoveries generated by an LBD system to create a tree of potential hypotheses. We weight the tree by developing set association measures, and extending them to linking set association measures. This weighted tree is displayed in an interactive visual environment, and validated by replicating the historic Raynaud's Disease - fish oil discovery. FAU - Henry, Sam AU - Henry S AD - Dept. Computer Science, Virginia Commonwealth University, VA, USA. FAU - Panahi, Aliakbar AU - Panahi A AD - Dept. Computer Science, Virginia Commonwealth University, VA, USA. FAU - Wijesinghe, D Shanaka AU - Wijesinghe DS AD - Dept. of Pharmacotherapy & Outcomes Science, Virginia Commonwealth University, VA, USA. FAU - McInnes, Bridget T AU - McInnes BT AD - Dept. Computer Science, Virginia Commonwealth University, VA, USA. LA - eng PT - Journal Article DEP - 20190506 PL - United States TA - AMIA Jt Summits Transl Sci Proc JT - AMIA Joint Summits on Translational Science proceedings. AMIA Joint Summits on Translational Science JID - 101539486 PMC - PMC6568119 EDAT- 2019/07/02 06:00 MHDA- 2019/07/02 06:01 PMCR- 2019/05/06 CRDT- 2019/07/02 06:00 PHST- 2019/07/02 06:00 [entrez] PHST- 2019/07/02 06:00 [pubmed] PHST- 2019/07/02 06:01 [medline] PHST- 2019/05/06 00:00 [pmc-release] AID - 3055683 [pii] PST - epublish SO - AMIA Jt Summits Transl Sci Proc. 2019 May 6;2019:582-591. eCollection 2019. PMID- 38201402 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240121 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 14 IP - 1 DP - 2023 Dec 30 TI - Analysis of Hand Function, Upper Limb Disability, and Its Relationship with Peripheral Vascular Alterations in Raynaud's Phenomenon. LID - 10.3390/diagnostics14010093 [doi] LID - 93 AB - This study aimed to compare vascular involvement, hand functionality, and upper limb disability between Raynaud's phenomenon participants and controls. Also, we analyzed the relationships between vascular impairment, mobility, and strength with disability in this Raynaud population. We conducted a case-control study with fifty-seven participants. We registered sociodemographic and clinical data; vascular variables (temperature, cold test, blood flow, and oxygen saturation); functional variables (pinch strength, range of motion), and disability (Shortened Disabilities of the Arm, Shoulder and Hand Questionnaire) (Q-DASH). Raynaud participants present more disability in all Q-DASH subscales, lower hands' temperature pre and post cold test, decreased blood flow on radial artery, decreased ranges of motions at passive extension of index finger, and active flexion and extension of thumb than the healthy controls. The multivariate regression analysis showed that extension of the index finger, lateral pinch strength, and oxygen saturation were significantly associated with disability in RP, almost the 55% of the total variance on the upper limb, 27% at sports/arts, and 42% at work. Our findings suggest that RP has a disabling effect on the upper extremities and a practice of activities in people who suffer it. Also, disability in Raynaud seems to be more related with hand mobility and strength impairment than vascular injury. FAU - Tapia-Haro, Rosa Mª AU - Tapia-Haro RM AD - Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), 18071 Granada, Spain. AD - ibs.GRANADA Instituto de Investigación Biosanitaria, 18012 Granada, Spain. FAU - García-Ríos, Mª Carmen AU - García-Ríos MC AUID- ORCID: 0000-0003-2913-1410 AD - Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), 18071 Granada, Spain. AD - ibs.GRANADA Instituto de Investigación Biosanitaria, 18012 Granada, Spain. FAU - Castro-Sánchez, Adelaida Mª AU - Castro-Sánchez AM AUID- ORCID: 0000-0002-9607-3241 AD - Department of Nursing, Physical Therapy and Medicine, University of Almeria, 04120 Almeria, Spain. FAU - Toledano-Moreno, Sonia AU - Toledano-Moreno S AD - ibs.GRANADA Instituto de Investigación Biosanitaria, 18012 Granada, Spain. AD - Biomedicine Program, Department of Physical Therapy, Faculty of Health Science, University of Granada (UGR), 18071 Granada, Spain. FAU - Casas-Barragán, Antonio AU - Casas-Barragán A AUID- ORCID: 0000-0002-6761-4811 AD - Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), 18071 Granada, Spain. AD - ibs.GRANADA Instituto de Investigación Biosanitaria, 18012 Granada, Spain. FAU - Aguilar-Ferrándiz, Mª Encarnación AU - Aguilar-Ferrándiz ME AD - Department of Physical Therapy, Faculty of Health Sciences, University of Granada (UGR), 18071 Granada, Spain. AD - ibs.GRANADA Instituto de Investigación Biosanitaria, 18012 Granada, Spain. LA - eng PT - Journal Article DEP - 20231230 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10795737 OTO - NOTNLM OT - Raynaud disease OT - activities of daily living OT - disability evaluation OT - hand OT - systemic scleroderma OT - upper extremity COIS- The authors declare no conflicts of interest. EDAT- 2024/01/11 07:43 MHDA- 2024/01/11 07:44 PMCR- 2023/12/30 CRDT- 2024/01/11 01:05 PHST- 2023/11/16 00:00 [received] PHST- 2023/12/28 00:00 [revised] PHST- 2023/12/29 00:00 [accepted] PHST- 2024/01/11 07:44 [medline] PHST- 2024/01/11 07:43 [pubmed] PHST- 2024/01/11 01:05 [entrez] PHST- 2023/12/30 00:00 [pmc-release] AID - diagnostics14010093 [pii] AID - diagnostics-14-00093 [pii] AID - 10.3390/diagnostics14010093 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Dec 30;14(1):93. doi: 10.3390/diagnostics14010093. PMID- 33564517 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210211 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 1 DP - 2021 Jan 5 TI - Use of Botulinum Toxin A to Treat Chemotherapy-Induced Raynaud's Phenomenon. PG - e12511 LID - 10.7759/cureus.12511 [doi] LID - e12511 AB - Raynaud's phenomenon (RP) is a vasospastic disorder of the digital blood vessels leading to pain, paresthesias, and pallor in response to cold or stress. RP can develop secondary to a number of pathologies or factors, including the use of chemotherapy agents. Typical first-line therapies for secondary RP may be contraindicated in patients with certain comorbidities. Here, we discuss a case in which botulinum toxin A (BTX-A) was used to treat chemotherapy-induced RP in a patient with non-small cell lung cancer (NSCLC). We provide a review of the existing literature on the clinical course and treatment modalities, including the use of BTX-A, for patients with secondary RP. A 56-year-old female with NSCLC received treatment with bevacizumab and pemetrexed. Her initial symptoms included progressive discoloration and pain in her fingertips, which hastily progressed to ischemia and subsequent dry gangrene. She was diagnosed with chemotherapy-induced RP, but traditional management options were complicated by acute congestive heart failure. BTX-A injections were administered at key locations on the wrist and hand, significantly improving her symptoms and slowing the progression of the gangrenous changes. RP can develop as sequelae of chemotherapy regimens. Clinical management may be complicated by underlying pathology and/or patient symptoms. BTX-A injections are an excellent non-operative therapeutic option for patients with secondary RP in cases where mainstay therapies may be contraindicated, thus decreasing pain, improving patient quality of life, and slowing the progression of gangrenous changes. CI - Copyright © 2021, Potluri et al. FAU - Potluri, Thrisha K AU - Potluri TK AD - Department of Plastic Surgery and Reconstructive Medicine, University of South Florida Morsani College of Medicine, Tampa, USA. FAU - Lee, Frank G AU - Lee FG AD - Department of Plastic Surgery and Reconstructive Medicine, University of South Florida Morsani College of Medicine, Tampa, USA. FAU - Song, Ethan AU - Song E AD - Department of Plastic Surgery and Reconstructive Medicine, University of South Florida Morsani College of Medicine, Tampa, USA. FAU - Wallace, Sean J AU - Wallace SJ AD - Division of Plastic & Reconstructive Surgery, Lehigh Valley Health Network, Allentown, USA. FAU - Miller, Nathan AU - Miller N AD - Division of Plastic & Reconstructive Surgery, Lehigh Valley Health Network, Allentown, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210105 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7863048 OTO - NOTNLM OT - botulinum toxin type a OT - chemotherapy OT - raynaud disease COIS- The authors have declared that no competing interests exist. EDAT- 2021/02/11 06:00 MHDA- 2021/02/11 06:01 PMCR- 2021/01/05 CRDT- 2021/02/10 05:56 PHST- 2021/02/10 05:56 [entrez] PHST- 2021/02/11 06:00 [pubmed] PHST- 2021/02/11 06:01 [medline] PHST- 2021/01/05 00:00 [pmc-release] AID - 10.7759/cureus.12511 [doi] PST - epublish SO - Cureus. 2021 Jan 5;13(1):e12511. doi: 10.7759/cureus.12511. PMID- 29300294 OWN - NLM STAT- MEDLINE DCOM- 20180417 LR - 20180417 IS - 1528-3976 (Electronic) IS - 1071-5754 (Linking) VI - 45 IP - 1 DP - 2018 Jan/Feb TI - Calcinosis Cutis: WOC Nurse Management. PG - 83-86 LID - 10.1097/WON.0000000000000403 [doi] AB - BACKGROUND: Calcinosis cutis is characterized by deposition of calcium in the dermis and the subcutaneous tissue. This condition may be initially identified by the WOC nurse, and its management requires a team approach. Calcinosis cutis is a debilitating and painful condition; it is difficult to manage, and widely agreed-upon standards for treatment have not been established. CASES: Two patients who presented with calcium deposits in the dermis and the subcutaneous tissue are discussed. The first was initially misdiagnosed as venous ulcerations; the second presented with a confirmed diagnoses of calcinosis cutis, Raynaud's disease, and scleroderma. CONCLUSIONS: Despite the lack of a standardized approach to treatment of calcinosis cutis, successful management of these patients was achieved by adhering to evidence-based wound-healing principles: (1) find and treat the underlying cause, (2) support the host, and (3) adhere to moist wound-healing principles. FAU - Netsch, Debra AU - Netsch D AD - Debra Netsch, DNP, APRN, FNP-BC, CWOCN, CFCN, WEB WOC Education Programs, Minneapolis, Minnesota; and Ridgeview Medical Center, Waconia, Minnesota. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Wound Ostomy Continence Nurs JT - Journal of wound, ostomy, and continence nursing : official publication of The Wound, Ostomy and Continence Nurses Society JID - 9435679 MH - Aged MH - Calcinosis/*diagnosis/*physiopathology MH - Female MH - Humans MH - Middle Aged MH - Scleroderma, Systemic/complications/physiopathology MH - Skin Diseases/*etiology EDAT- 2018/01/05 06:00 MHDA- 2018/04/18 06:00 CRDT- 2018/01/05 06:00 PHST- 2018/01/05 06:00 [entrez] PHST- 2018/01/05 06:00 [pubmed] PHST- 2018/04/18 06:00 [medline] AID - 00152192-201801000-00015 [pii] AID - 10.1097/WON.0000000000000403 [doi] PST - ppublish SO - J Wound Ostomy Continence Nurs. 2018 Jan/Feb;45(1):83-86. doi: 10.1097/WON.0000000000000403. PMID- 35858907 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220724 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 6 IP - 1 DP - 2022 Jul 21 TI - Incidence, remission, and persistence of Raynaud's phenomenon in the general population of northern Sweden: a prospective study. PG - 41 LID - 10.1186/s41927-022-00272-0 [doi] LID - 41 AB - BACKGROUND: Raynaud's phenomenon is common condition, but little is known about the natural course. The primary aim of this study was to determine the incidence, remission, and persistence proportions of Raynaud's phenomenon in the general population of northern Sweden. Secondary aims were to determine how individual and exposure factors affect the course of Raynaud's phenomenon, and to assess gender differences. METHODS: A prospective, survey-based, closed-cohort study was conducted on a sample of men and women between 18-70 years of age, living in northern Sweden. Data on Raynaud's phenomenon characteristics and general health status were collected during the winters of 2015 (baseline) and 2021 (follow-up). Rates of incidence, remission, and persistence were calculated. Binary logistic regression was used to determine the association between baseline variables and the course of Raynaud's phenomenon. RESULTS: The study population consisted of 2703 women (53.9%) and 2314 men. There were 390 women (14.5%) and 290 men (12.7%) reporting Raynaud's phenomenon in the follow-up survey. The annual incidence proportion was 0.7% among women and 0.9% among men (gender difference p = 0.04). The annual remission proportion was 4.4% and 5.5%, respectively (p = 0.05). Having sustained a cold injury affecting the hands since baseline was significantly associated with incident Raynaud's phenomenon (OR 3.92; 95% CI 2.60-5.90), after adjusting for age and gender. CONCLUSIONS: In the general population of northern Sweden, Raynaud's phenomenon is a common but variable condition, where symptoms may remit over time. Men had a higher incidence proportion than women. The results support a possible causal pathway where cold injury can precede the onset of Raynaud's phenomenon. CI - © 2022. The Author(s). FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. albin.stjernbrandt@umu.se. FAU - Pettersson, Hans AU - Pettersson H AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Lundström, Ronnie AU - Lundström R AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Liljelind, Ingrid AU - Liljelind I AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. FAU - Wahlström, Jens AU - Wahlström J AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, 901 87, Umeå, Sweden. LA - eng GR - 646641/Region Västerbotten/ GR - 939839/Healthcare Research in Regional Collaboration in the North/ PT - Journal Article DEP - 20220721 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC9301854 OTO - NOTNLM OT - Cold climate OT - Incidence OT - Longitudinal studies OT - Occupational exposure OT - Peripheral vascular diseases OT - Raynaud disease OT - Remission, Spontaneous OT - Sweden COIS- The authors declare that they have no competing interests. EDAT- 2022/07/21 06:00 MHDA- 2022/07/21 06:01 PMCR- 2022/07/21 CRDT- 2022/07/20 23:38 PHST- 2022/01/10 00:00 [received] PHST- 2022/04/21 00:00 [accepted] PHST- 2022/07/20 23:38 [entrez] PHST- 2022/07/21 06:00 [pubmed] PHST- 2022/07/21 06:01 [medline] PHST- 2022/07/21 00:00 [pmc-release] AID - 10.1186/s41927-022-00272-0 [pii] AID - 272 [pii] AID - 10.1186/s41927-022-00272-0 [doi] PST - epublish SO - BMC Rheumatol. 2022 Jul 21;6(1):41. doi: 10.1186/s41927-022-00272-0. PMID- 27682649 OWN - NLM STAT- MEDLINE DCOM- 20171222 LR - 20181018 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 76 DP - 2017 Jan TI - Autoantibody to scaffold attachment factor B (SAFB): A novel connective tissue disease-related autoantibody associated with interstitial lung disease. PG - 101-107 LID - S0896-8411(16)30149-4 [pii] LID - 10.1016/j.jaut.2016.09.006 [doi] AB - OBJECTIVE: To identify and characterize a novel connective tissue disease (CTD)-related autoantibody (autoAb) directed against scaffold attachment factor B (SAFB). METHODS: AutoAb specificity was analyzed using RNA and protein-immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry. RESULTS: By immunoprecipitation assay, 10 sera reacted with a protein with a molecular weight of approximately 160 kDa. Liquid chromatography mass spectrometry of the partially purified autoantigen and additional immunoblot-based analyses revealed that the Ab specifically recognized SAFB. Anti-SAFB Abs were detected in 2 of 646 patients with systemic sclerosis (SSc) (0.3%), 1 of 1570 patients with polymyositis/dermatomyositis (0.06%), 4 of 270 patients with interstitial lung disease (ILD) (1.5%), 1 of 43 patients with overlap syndrome (2.3%) and 2 patients with other diseases including primary Raynaud's disease and eosinophilic pneumonia. Five patients with anti-SAFB Abs had Raynaud's phenomenon and 3 had nail fold punctate hemorrhage. Of note, 8 of the 10 patients (80%) suffered from ILD. None of the patients with anti-SAFB Abs had pulmonary arterial hypertension, heart disease, or renal involvement. CONCLUSIONS: Anti-SAFB Ab is a novel CTD-related autoAb possibly associated with ILD. CI - Copyright © 2016 Elsevier Ltd. All rights reserved. FAU - Takeuchi, Akiko AU - Takeuchi A AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Matsushita, Takashi AU - Matsushita T AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Kaji, Kenzo AU - Kaji K AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Okamoto, Yoshinobu AU - Okamoto Y AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Yasui, Masahide AU - Yasui M AD - Division of Respiratory Medicine, Department of Internal Medicine, Kanazawa Municipal Hospital, Kanazawa, Japan. FAU - Hirata, Masayoshi AU - Hirata M AD - Division of Rheumatology, Department of Internal Medicine, Takaoka Municipal Hospital, Takaoka, Japan. FAU - Oishi, Naoto AU - Oishi N AD - Department of Dermatology, Kurobe Municipal Hospital, Kurobe, Japan. FAU - Higashi, Akira AU - Higashi A AD - Department of Dermatology, Toyama Red Cross Hospital, Toyama, Japan. FAU - Seishima, Mariko AU - Seishima M AD - Department of Dermatology, Gifu University, Gifu, Japan. FAU - Asano, Tomoya AU - Asano T AD - Division of Functional Genomics, Advanced Science Research Center, Kanazawa University, Kanazawa, Japan. FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan; Department of Dermatology, University of Tsukuba, Tsukuba, Japan. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Takehara, Kazuhiko AU - Takehara K AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Hamaguchi, Yasuhito AU - Hamaguchi Y AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. Electronic address: yasuhito@med.kanazawa-u.ac.jp. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160925 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) RN - 0 (Matrix Attachment Region Binding Proteins) RN - 0 (Nuclear Matrix-Associated Proteins) RN - 0 (Receptors, Estrogen) RN - 0 (SAFB protein, human) SB - IM MH - Aged MH - Autoantibodies/*immunology MH - Autoantigens/*immunology MH - Biomarkers MH - Case-Control Studies MH - Connective Tissue Diseases/diagnosis/immunology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Lung Diseases, Interstitial/diagnosis/*immunology MH - Male MH - Matrix Attachment Region Binding Proteins/*immunology MH - Middle Aged MH - Nuclear Matrix-Associated Proteins/*immunology MH - Phenotype MH - Receptors, Estrogen/*immunology OTO - NOTNLM OT - Anti-SAFB Ab OT - Anti-nuclear Ab OT - Interstitial lung disease OT - Raynaud's phenomenon EDAT- 2016/09/30 06:00 MHDA- 2017/12/23 06:00 CRDT- 2016/09/30 06:00 PHST- 2016/05/21 00:00 [received] PHST- 2016/09/02 00:00 [revised] PHST- 2016/09/13 00:00 [accepted] PHST- 2016/09/30 06:00 [pubmed] PHST- 2017/12/23 06:00 [medline] PHST- 2016/09/30 06:00 [entrez] AID - S0896-8411(16)30149-4 [pii] AID - 10.1016/j.jaut.2016.09.006 [doi] PST - ppublish SO - J Autoimmun. 2017 Jan;76:101-107. doi: 10.1016/j.jaut.2016.09.006. Epub 2016 Sep 25. PMID- 25385354 OWN - NLM STAT- MEDLINE DCOM- 20160803 LR - 20161125 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 29 IP - 11 DP - 2015 Nov TI - Acroosteolysis presenting with brachyonychia following exposure to cold. PG - 2252-4 LID - 10.1111/jdv.12826 [doi] AB - BACKGROUND: A vast number of conditions ranging from simple trauma to hereditary and collagen vascular disease had been described in association with acroosteolysis. OBJECTIVE: To demonstrate that severe cold exposure not mounting to frostbite may be associated with acroosteolysis. METHODS: A 16-year-old girl with acroosteolysis presenting with brachyonychia was fully investigated for possible cause of her nail and bone deformity. RESULTS: Lab investigations including Parathormone levels, Anti Scl 70, ANA, Anti-CCP and RF levels were all normal. X-ray imaging revealed resorption of the tufts of the terminal phalanges bilaterally. Disruption of nail fold capillaries with sluggish flow in capillary loops was evident on capillaroscopy. CONCLUSION: It had been repeatedly reported that frostbite, Raynaud's disease and diseases associated with repeated chilblains may be associated with secondary cold-induced acroosteolysis. Here, we present a case of acroosteolysis associated with brachyonychia following exposure to severe cold not mounting to frostbite. CI - © 2014 European Academy of Dermatology and Venereology. FAU - El-Komy, M H M AU - El-Komy MH AD - Department of Dermatology, Faculty of Medicine, Cairo University, Cairo, Egypt. FAU - Baran, R AU - Baran R AD - Nail Disease Center, Cannes, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20141110 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 SB - IM MH - Acro-Osteolysis/diagnostic imaging/*etiology MH - Adolescent MH - Capillaries/pathology MH - Cold Temperature/*adverse effects MH - Female MH - Finger Phalanges/diagnostic imaging MH - Humans MH - Nail Diseases/diagnostic imaging/*etiology MH - Nails/blood supply MH - Radiography EDAT- 2014/11/12 06:00 MHDA- 2016/08/04 06:00 CRDT- 2014/11/12 06:00 PHST- 2014/08/05 00:00 [received] PHST- 2014/10/06 00:00 [accepted] PHST- 2014/11/12 06:00 [entrez] PHST- 2014/11/12 06:00 [pubmed] PHST- 2016/08/04 06:00 [medline] AID - 10.1111/jdv.12826 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2015 Nov;29(11):2252-4. doi: 10.1111/jdv.12826. Epub 2014 Nov 10. PMID- 39850714 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250302 IS - 1687-6105 (Print) IS - 1687-6113 (Electronic) IS - 1687-6113 (Linking) VI - 2025 DP - 2025 TI - Acrocyanosis: The Least Known Acrosyndrome Revisited With a Dermatologic Perspective. PG - 2904301 LID - 10.1155/drp/2904301 [doi] LID - 2904301 AB - Background: Acrocyanosis is a functional peripheral vascular disorder, currently categorized under the canopy of acrosyndromes, i.e., a group of clinically similar and significantly overlapping vascular disorders involving the acral skin. The disorder might be primary or secondary, depending on the cause. Recently, there has been a remarkable surge in acrocyanosis prevalence along with the COVID-19 pandemic. Both COVID-19 infection and vaccines for COVID-19 have been affixed to the list of disorders instigating acrocyanosis. Objectives: The goal of this narrative review was to evaluate the existing literature, project acrocyanosis from the viewpoint of dermatologists in the face of the COVID-19 pandemic, and assess the need for targeted research, education, and/or clinical practice. Methods: An English literature search was conducted using PubMed and Google. All abstracts on acrocyanosis, irrespective of the article type and publication date, were retrieved and reviewed and those most relevant for the focus of this article were selected and summarized. Discussion/Results: A narrative review was carried out. There is paucity of randomized, double-blind, placebo-controlled studies on acrocyanosis in the English literature, implicating the need for targeted research. Pertinent information still relies on anecdotal observations, case reports, case series, or scarce reviews, which are dated rather old and published in vascular-oriented journals. The scarcity of published literature on acrocyanosis in dermatology-oriented journals points to the necessity of professional education and improvement of clinical diagnostic skills for dermatologists. Conclusions: Although acrocyanosis is the least known and the least studied acrosyndrome, it is increasingly more commonly confronted in the COVID-19 era. The diagnosis still largely relies on clinical findings. Accordingly, it has become a growing necessity for a dermatologist to remain updated on this peculiar disorder and be able to differentiate acrocyanosis from clinically similar cold-induced or cold-exacerbated acrosyndromes. Acrocyanosis is still misdiagnosed, underdiagnosed, underreported, and undertreated by the dermatology community. CI - Copyright © 2025 Deniz Demircioğlu and Emel Öztürk Durmaz. Dermatology Research and Practice published by John Wiley & Sons Ltd. FAU - Demircioğlu, Deniz AU - Demircioğlu D AUID- ORCID: 0000-0001-8290-3075 AD - Department of Dermatology, Acıbadem University School of Medicine, İstanbul, Turkey. FAU - Öztürk Durmaz, Emel AU - Öztürk Durmaz E AUID- ORCID: 0000-0003-0661-9720 AD - Department of Dermatology, Acıbadem University School of Medicine, İstanbul, Turkey. LA - eng PT - Journal Article PT - Review DEP - 20250116 PL - United States TA - Dermatol Res Pract JT - Dermatology research and practice JID - 101312803 PMC - PMC11756949 OTO - NOTNLM OT - BASCULE syndrome OT - Bier spots OT - COVID fingers OT - COVID toes OT - acrocyanosis OT - acrosyndromes OT - chilblain-like lesions OT - chilblains OT - coronavirus OT - raynaud's disease COIS- The authors declare no conflicts of interest. EDAT- 2025/01/24 06:26 MHDA- 2025/01/24 06:27 PMCR- 2025/01/16 CRDT- 2025/01/24 04:25 PHST- 2024/06/22 00:00 [received] PHST- 2024/12/07 00:00 [accepted] PHST- 2025/01/24 06:27 [medline] PHST- 2025/01/24 06:26 [pubmed] PHST- 2025/01/24 04:25 [entrez] PHST- 2025/01/16 00:00 [pmc-release] AID - 10.1155/drp/2904301 [doi] PST - epublish SO - Dermatol Res Pract. 2025 Jan 16;2025:2904301. doi: 10.1155/drp/2904301. eCollection 2025. PMID- 35242466 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230917 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 1 DP - 2022 Jan TI - Raynaud's Phenomenon: Reviewing the Pathophysiology and Management Strategies. PG - e21681 LID - 10.7759/cureus.21681 [doi] LID - e21681 AB - Raynaud's phenomenon (RP) is a multifactorial vasospastic disorder characterized by a transient, recurrent, and reversible constriction of peripheral blood vessels. RP is documented to affect up to 5% of the general population, but variation in its prevalence is commonly recognized owing to many factors, including varied definitions, gender, genetics, hormones, and region. Furthermore, RP may be idiopathic or be a clinical manifestation of an underlying illness. Patients with RP classically describe a triphasic discoloration of the affected area, beginning with pallor, followed by cyanosis, and finally ending with erythema. This change in color spares the thumb and is often associated with pain. Each attack may persist from several minutes to hours. Moreover, the transient cessation of blood flow in RP is postulated to be mediated by neural and vascular mechanisms. Both structural and functional alterations observed in the blood vessels contribute to the vascular abnormalities documented in RP. However, functional impairment serves as a primary contributor to the pathophysiology of primary Raynaud's. Substances like endothelin-1, angiotensin, and angiopoietin-2 play a significant role in the vessel-mediated pathophysiology of RP. The role of nitric oxide in the development of this phenomenon is still complex. Neural abnormalities resulting in RP are recognized as either being concerned with central mechanisms or peripheral mechanisms. CNS involvement in RP may be suggested by the fact that emotional distress and low temperature serve as major triggers for an attack, but recent observations have highlighted the importance of locally produced factors in this regard as well. Impaired vasodilation, increased vasoconstriction, and several intravascular abnormalities have been documented as potential contributors to the development of this disorder. RP has also been observed to occur as a side effect of various drugs. Recent advances in understanding the mechanism of RP have yielded better pharmacological therapies. However, general lifestyle modifications along with other nonpharmacological interventions remain first-line in the management of these patients. Calcium channel blockers, alpha-1 adrenoreceptor antagonists, angiotensin-converting enzyme inhibitors, nitric oxide, prostaglandin analogs, and phosphodiesterase inhibitors are some of the common classes of drugs that have been found to be therapeutically significant in the management of RP. Additionally, anxiety management, measures to avoid colder temperatures, and smoking cessation, along with other simple modifications, have proven to be effective non-drug strategies in patients experiencing milder symptoms. CI - Copyright © 2022, Nawaz et al. FAU - Nawaz, Iqra AU - Nawaz I AD - Medicine, Quaid-e-Azam Medical College, Bahawalpur, PAK. FAU - Nawaz, Yashfa AU - Nawaz Y AD - Medicine, Army Medical College, Rawalpindi, PAK. FAU - Nawaz, Eisha AU - Nawaz E AD - Medicine, Quaid-e-Azam Medical College, Bahawalpur, PAK. FAU - Manan, Muhammad Romail AU - Manan MR AD - Medicine, Services Institute of Medical Sciences, Lahore, PAK. FAU - Mahmood, Adil AU - Mahmood A AD - Medicine, Bahawal Victoria Hospital, Bahawalpur, PAK. LA - eng PT - Journal Article PT - Review DEP - 20220128 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8884459 OTO - NOTNLM OT - cold fingers OT - hereditary raynaud phenomenon OT - pathology OT - raynaud disease OT - rheumatology COIS- The authors have declared that no competing interests exist. EDAT- 2022/03/05 06:00 MHDA- 2022/03/05 06:01 PMCR- 2022/01/28 CRDT- 2022/03/04 05:41 PHST- 2021/09/26 00:00 [received] PHST- 2022/01/25 00:00 [accepted] PHST- 2022/03/04 05:41 [entrez] PHST- 2022/03/05 06:00 [pubmed] PHST- 2022/03/05 06:01 [medline] PHST- 2022/01/28 00:00 [pmc-release] AID - 10.7759/cureus.21681 [doi] PST - epublish SO - Cureus. 2022 Jan 28;14(1):e21681. doi: 10.7759/cureus.21681. eCollection 2022 Jan. PMID- 40460899 OWN - NLM STAT- MEDLINE DCOM- 20250711 LR - 20260515 IS - 1089-8646 (Electronic) IS - 0888-7543 (Linking) VI - 117 IP - 4 DP - 2025 Jul TI - MiR-100-5p as a biomarker in hand-arm vibration disease: Mediating pathogenesis through angiogenesis suppression. PG - 111067 LID - S0888-7543(25)00083-7 [pii] LID - 10.1016/j.ygeno.2025.111067 [doi] AB - Hand-arm vibration disease (HAVD), also known as occupational Raynaud's disease, is an occupational disease that can cause injuries to the microcirculation in the fingertips, might leading to severe vascular and neurological damage. While HAVD has been defined as a peripheral vascular injury disorders, its comprehensive disease mechanisms-spanning molecular drivers, pathophysiological cascades, and clinically actionable biomarkers remain substantially unelucidated. Our study has used miRNA sequencing (miRNA-seq) to investigate the differential expression of miRNAs in HAVD patients compared to workers exposed to hand-transmitted vibration. In vitro, human umbilical vein endothelial cells (HUVECs) have been utilized to explore the role of miR-100-5p in endothelial cell dysfunction. Our results reveal that 36 miRNAs were upregulated and 2 miRNAs were downregulated in HAVD patients. Notably, miR-100-5p and miR-4735-5p exhibited the most significant differential expression.The area under the curve (AUC) for miR-100-5p in distinguishing hand-transmitted vibration-exposed workers from healthy individuals was 0.9906, while its AUC for identifying HAVD was 0.9922. The combination of those two miRNAs as the diagnostic marker of HAVD showed great potential has higher AUC, with high sensitivity, and specificity. Furthermore, miR-100-5p might mediate the pathology of HAVD by inhibiting vascular cell angiogenesis via VEGFA/VEGFR pathway, which could be regulated by decreased TRIB2 expression and inhibition of the p38 MAPK signaling pathway. CI - Copyright © 2024. Published by Elsevier Inc. FAU - Zheng, Hanjun AU - Zheng H AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China; Department of Nutrition, School of Public Health (Shenzhen), Sun Yat-sen University, Shenzhen, China; Guangdong Provincial Key Laboratory of Food, Nutrition and Health, Guangzhou, Guangdong, China; Guangdong Engineering Technology Center of Nutrition Transformation, Guangzhou, Guangdong, China. FAU - Lang, Li AU - Lang L AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China. FAU - Li, Wankang AU - Li W AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China; Department of Public Health, Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Wang, Qia AU - Wang Q AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China. FAU - Xiao, Bin AU - Xiao B AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China. FAU - Jia, Yanxia AU - Jia Y AD - Department of Public Health, Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Pan, Siyu AU - Pan S AD - Department of Public Health, Guangdong Pharmaceutical University, Guangzhou, Guangdong, China. FAU - Yan, Maosheng AU - Yan M AD - Guangdong Provincial Hospital for Occupational Disease Prevention and Treatment, Guangzhou, Guangdong, China. Electronic address: yanmsh@gdoh.org. LA - eng SI - GEO/GSE231390 PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20250601 PL - United States TA - Genomics JT - Genomics JID - 8800135 RN - 0 (MicroRNAs) RN - 0 (Biomarkers) RN - 0 (MIRN100 microRNA, human) SB - IM MH - Humans MH - *MicroRNAs/genetics/metabolism MH - Biomarkers/metabolism MH - Human Umbilical Vein Endothelial Cells/metabolism MH - *Hand-Arm Vibration Syndrome/genetics/metabolism/pathology/diagnosis MH - Male MH - Female MH - Adult MH - Middle Aged MH - *Neovascularization, Pathologic/genetics MH - Angiogenesis OTO - NOTNLM OT - Angiogenesis OT - Biomarker OT - Early diagnosis OT - Hand-arm vibration disease (HAVD) OT - Peripheral vascular injuries OT - Raynaud's phenomenon (RP) OT - TRIB2 OT - miR-100-5p COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/06/04 00:29 MHDA- 2025/07/12 16:44 CRDT- 2025/06/03 19:27 PHST- 2024/12/04 00:00 [received] PHST- 2025/05/29 00:00 [revised] PHST- 2025/05/30 00:00 [accepted] PHST- 2025/07/12 16:44 [medline] PHST- 2025/06/04 00:29 [pubmed] PHST- 2025/06/03 19:27 [entrez] AID - S0888-7543(25)00083-7 [pii] AID - 10.1016/j.ygeno.2025.111067 [doi] PST - ppublish SO - Genomics. 2025 Jul;117(4):111067. doi: 10.1016/j.ygeno.2025.111067. Epub 2025 Jun 1. PMID- 28286685 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200929 IS - 2090-6765 (Print) IS - 2090-6773 (Electronic) IS - 2090-6773 (Linking) VI - 2017 DP - 2017 TI - Acute Vision Loss Following Endoscopic Sinus Surgery. PG - 4935123 LID - 10.1155/2017/4935123 [doi] LID - 4935123 AB - A 41-year-old female with a history of uterine cancer and Celiac and Raynaud's Disease presented to our institution with frequent migraines and nasal congestion. She underwent functional endoscopic sinus surgery (FESS) and experienced acute unilateral vision loss postoperatively. Rapid recognition of the etiology and effective treatment are paramount given the permanent and irreversible vision loss that can result. Arterial vasospasm following FESS is rare. Patients with autoimmune diseases have perhaps an increased risk for vasospasm secondary to an increased vasoreactive profile. We present the first documented case of nitroglycerin sublingual therapy to successfully treat ophthalmic artery vasospasm following FESS. Nitroglycerin sublingual therapy is a promising treatment for ophthalmic vasospasm secondary to its ability to cross the blood-ocular barrier, its rapid onset of action, and its ability to promote relaxation of vascular smooth muscle. FAU - Byrd, Serena AU - Byrd S AUID- ORCID: 0000-0002-1535-0949 AD - Department of Otolaryngology-Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, MO, USA. FAU - Hussaini, Adnan S AU - Hussaini AS AUID- ORCID: 0000-0002-2400-3615 AD - Department of Otolaryngology-Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, MO, USA. FAU - Antisdel, Jastin AU - Antisdel J AD - Department of Otolaryngology-Head and Neck Surgery, Saint Louis University School of Medicine, Saint Louis, MO, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20170213 PL - United States TA - Case Rep Otolaryngol JT - Case reports in otolaryngology JID - 101576603 PMC - PMC5327765 COIS- The authors declare that there is no conflict of interests regarding the publication of this paper. EDAT- 2017/03/14 06:00 MHDA- 2017/03/14 06:01 PMCR- 2017/02/13 CRDT- 2017/03/14 06:00 PHST- 2016/11/19 00:00 [received] PHST- 2017/01/14 00:00 [revised] PHST- 2017/01/19 00:00 [accepted] PHST- 2017/03/14 06:00 [entrez] PHST- 2017/03/14 06:00 [pubmed] PHST- 2017/03/14 06:01 [medline] PHST- 2017/02/13 00:00 [pmc-release] AID - 10.1155/2017/4935123 [doi] PST - ppublish SO - Case Rep Otolaryngol. 2017;2017:4935123. doi: 10.1155/2017/4935123. Epub 2017 Feb 13. PMID- 18335184 OWN - NLM STAT- MEDLINE DCOM- 20080804 LR - 20211020 IS - 0020-9554 (Print) IS - 0020-9554 (Linking) VI - 49 IP - 5 DP - 2008 May TI - [Pharmacology and clinical relevance of vasopressin antagonists]. PG - 628, 629-30, 632-4 LID - 10.1007/s00108-008-2017-z [doi] AB - As vasopressin receptors are found in many different tissues, vasopressin antagonists may benefit the treatment of numerous disorders. Effects of vasopressin via V1(a) and V2 receptors are closely implicated in a variety of water-retaining diseases and cardiovascular diseases, including heart failure, hyponatremia, hypertension, renal diseases, syndrome of inappropriate antidiuretic hormone secretion, cirrhosis, and ocular hypertension. Furthermore, V1(a) vasopressin antagonists might be useful in cerebral ischemia and stroke, Raynaud's disease, dysmenorrhoea and tocolytic treatment. V1(b) selective vasopressin antagonists are discussed in terms of their usefulness in the treatment of emotional and psychiatric disorders. The vaptans are vasopressin receptor antagonists with V1(a) (relcovaptan) or V2 (tolvaptan, lixivaptan, satavaptan) selectivity or non-selective activity (conivaptan). Conivaptan is the first vaptan which has been approved by the FDA for the treatment of euvolemic hyponatremia. For further indications such as congenital heart failure, studies are going on. FAU - Lemmens-Gruber, R AU - Lemmens-Gruber R AD - Department für Pharmakologie und Toxikologie, Universität Wien, Althanstrasse 14, 1090, Wien, Osterreich. rosa.lemmens@univie.ac.at FAU - Kamyar, M AU - Kamyar M LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Pharmakologie und klinische Bedeutung von Vasopressinantagonisten. PL - Germany TA - Internist (Berl) JT - Der Internist JID - 0264620 RN - 0 (Antidiuretic Agents) RN - 0 (Antidiuretic Hormone Receptor Antagonists) RN - 11000-17-2 (Vasopressins) SB - IM MH - Antidiuretic Agents/*therapeutic use MH - *Antidiuretic Hormone Receptor Antagonists MH - Heart Failure/*prevention & control MH - Humans MH - Hyponatremia/*prevention & control MH - Vasopressins/*therapeutic use RF - 30 EDAT- 2008/03/13 09:00 MHDA- 2008/08/05 09:00 CRDT- 2008/03/13 09:00 PHST- 2008/03/13 09:00 [pubmed] PHST- 2008/08/05 09:00 [medline] PHST- 2008/03/13 09:00 [entrez] AID - 10.1007/s00108-008-2017-z [doi] PST - ppublish SO - Internist (Berl). 2008 May;49(5):628, 629-30, 632-4. doi: 10.1007/s00108-008-2017-z. PMID- 32467814 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 12 IP - 4 DP - 2020 Apr 26 TI - Right Ventricular Perforation Presenting as Tingling of the Left Breast. PG - e7839 LID - 10.7759/cureus.7839 [doi] LID - e7839 AB - Pacemaker lead-associated cardiac perforation is a rare phenomenon. Lead perforations can be acute, subacute, or chronic following lead placement. Symptoms are typically nonspecific and depend on the location of the displaced lead. Diagnostic workup requires interrogation of the pacemaker and imaging studies. Management of lead displacement is dependent on multiple risk factors such as age, gender, corticosteroid use, and anticoagulation therapy. A 74-year-old female with a history of myosin light chain kinase (MYLK) 2 hypertrophic cardiomyopathy, Sjogren's syndrome, Raynaud's disease, and sick sinus syndrome was evaluated for an abnormal finding on pacemaker interrogation. The patient's only symptom was tingling of her left breast. Imaging studies confirmed pacemaker lead perforation. Right ventricle perforation due to a pacemaker lead displacement can cause severe complications. Early identification and treatment by physicians can reduce the risk of mortality. CI - Copyright © 2020, Shah et al. FAU - Shah, Rony AU - Shah R AD - Internal Medicine, Citrus Memorial Hospital, Inverness, USA. FAU - Barnes, Andrew AU - Barnes A AD - Internal Medicine, Citrus Memorial Hospital, Inverness, USA. FAU - Modi, Fagunkumar AU - Modi F AD - Internal Medicine, Citrus Memorial Hospital, Inverness, USA. FAU - Royalty, John AU - Royalty J AD - Cardiovascular Surgery, Citrus Memorial Hospital, Inverness, USA. FAU - Jordan, Jeffrey AU - Jordan J AD - Internal Medicine, Citrus Memorial Hospital, Inverness, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200426 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7250524 OTO - NOTNLM OT - pacemaker lead displacement OT - paresthesia of left breast OT - right ventricular perforation OT - tingling of left breast COIS- The authors have declared that no competing interests exist. EDAT- 2020/05/30 06:00 MHDA- 2020/05/30 06:01 PMCR- 2020/04/26 CRDT- 2020/05/30 06:00 PHST- 2020/05/30 06:00 [entrez] PHST- 2020/05/30 06:00 [pubmed] PHST- 2020/05/30 06:01 [medline] PHST- 2020/04/26 00:00 [pmc-release] AID - 10.7759/cureus.7839 [doi] PST - epublish SO - Cureus. 2020 Apr 26;12(4):e7839. doi: 10.7759/cureus.7839. PMID- 31346706 OWN - NLM STAT- MEDLINE DCOM- 20191007 LR - 20200429 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 78 IP - 7 DP - 2019 Sep TI - [Antisynthetase syndromes]. PG - 645-655 LID - 10.1007/s00393-019-0665-0 [doi] AB - Antisynthetase syndromes (ASS) are rare autoimmune diseases. Characteristic is the presence of at least one of the three main symptoms myositis, interstitial lung disease (ILD) and arthritis with possible accompanying symptoms, such as mechanic's hands and feet, Raynaud's disease and/or fever in combination with detection of an aminoacyl-tRNA synthetase antibody in peripheral blood. In addition to myositis, ILD is a frequent and often predominant organ involvement and is responsible for morbidity and mortality. Autoantibodies to 11 aminoacyl-tRNA synthetases are known of which 8 have so far been associated with the clinical manifestation of ASS. The Jo-1 antibody is by far the most frequent one. The antibodies differ in the rate and severity of the main and accompanying symptoms. Treatment with selected immunosuppressive medication depends on the extent and severity of organ involvement. With a 5-year survival rate of approximately 90%, the Jo-1 syndrome has the best prognosis. FAU - Bauhammer, Jutta AU - Bauhammer J AD - Abteilung für Innere Medizin/Rheumatologie, ACURA-Rheumazentrum Baden-Baden, Rotenbachtalstr. 5, 76530, Baden-Baden, Deutschland. J.Bauhammer@acura-kliniken.de. FAU - Fiehn, Christoph AU - Fiehn C AD - Praxis für Rheumatologie, Tätigkeitsschwerpunkt Klinische Immunologie, Medical Center, Baden-Baden, Deutschland. LA - ger PT - Journal Article PT - Review TT - Antisynthetasesyndrome. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - *Amino Acyl-tRNA Synthetases/blood/immunology MH - Autoantibodies/*immunology MH - Humans MH - Lung Diseases, Interstitial MH - *Myositis/enzymology/immunology OTO - NOTNLM OT - Antisynthetase antibodies OT - Interstitial lung disease OT - Jo-1 OT - Mechanic’s hands OT - Polymyositis EDAT- 2019/07/28 06:00 MHDA- 2019/10/08 06:00 CRDT- 2019/07/27 06:00 PHST- 2019/07/28 06:00 [pubmed] PHST- 2019/10/08 06:00 [medline] PHST- 2019/07/27 06:00 [entrez] AID - 10.1007/s00393-019-0665-0 [pii] AID - 10.1007/s00393-019-0665-0 [doi] PST - ppublish SO - Z Rheumatol. 2019 Sep;78(7):645-655. doi: 10.1007/s00393-019-0665-0. PMID- 35676726 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230916 IS - 1745-6673 (Print) IS - 1745-6673 (Electronic) IS - 1745-6673 (Linking) VI - 17 IP - 1 DP - 2022 Jun 8 TI - The impact of Raynaud's phenomenon on work ability - a longitudinal study. PG - 12 LID - 10.1186/s12995-022-00354-2 [doi] LID - 12 AB - OBJECTIVE: To determine if having Raynaud's phenomenon (RP) affects the work ability, job retainment, or occurrence of sick leave. METHODS: Surveys on the working-age general population of northern Sweden were conducted in 2015 and 2021, gathering data on RP, occupation and sick leave. Work ability was assessed using the Work Ability Score. RESULTS: The study population consisted of 2,703 women and 2,314 men, among which 390 women and 290 men reported RP at follow-up. For women, the mean [standard deviation (SD)] Work Ability Score was 8.02 (2.24) for subjects reporting RP and 7.68 (2.46) for those without RP. For men, the corresponding numbers were 7.37 (2.03) and 7.61 (2.14), respectively. Multiple linear regression did not show an association between RP status and work ability (p = 0.459 for women and p = 0.254 for men), after adjusting for age, body mass index, physical workload, cardiovascular disease, and perceived stress. Having retained the same main livelihood since baseline was reported by 227 (58.5%) women with RP, 1,163 (51.2%) women without RP, 152 (52.6%) men with RP, and 1,075 (54.1%) men without RP (p = 0.002 for women and p = 0.127 for men). At follow-up, any occurrence of sick leave during the last year was reported by 80 (21.4%) women with RP, 410 (18.6%) women without RP, 48 (17.1%) men with RP, and 268 (13.7%) men without RP (p = 0.208 for women and p = 0.133 for men). Among those reporting sick leave, the mean (SD) duration in months was 2.93 (3.76) for women with RP, 3.00 (4.64) for women without RP, 2.77 (3.79) for men with RP, and 2.91 (12.45) for men without RP (p = 0.849 for women and p = 0.367 for men). CONCLUSION: For neither women nor men was there a significant effect of having RP on work ability. Women with RP reported a slightly higher job retainment compared to those without the condition, while there was no difference in job retainment among men. For neither gender did the presence of RP influence the occurrence of recent sick leave, nor did it affect the length of time away from work. CI - © 2022. The Author(s). FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Department of Public Health and Clinical Medicine, Section of Sustainable Health, Umeå University, 901 87, Umeå, Sweden. albin.stjernbrandt@umu.se. FAU - Wahlström, Jens AU - Wahlström J AD - Department of Public Health and Clinical Medicine, Section of Sustainable Health, Umeå University, 901 87, Umeå, Sweden. LA - eng GR - 646641/Västerbotten Läns Landsting/ GR - 939839/Healthcare Research in Regional Collaboration in the North/ PT - Journal Article DEP - 20220608 PL - England TA - J Occup Med Toxicol JT - Journal of occupational medicine and toxicology (London, England) JID - 101245790 PMC - PMC9175314 OTO - NOTNLM OT - Longitudinal Studies OT - Raynaud Disease OT - Sick Leave OT - Sweden OT - Work COIS- The authors declare no conflict of interest. EDAT- 2022/06/09 06:00 MHDA- 2022/06/09 06:01 PMCR- 2022/06/08 CRDT- 2022/06/08 23:43 PHST- 2022/02/06 00:00 [received] PHST- 2022/06/01 00:00 [accepted] PHST- 2022/06/08 23:43 [entrez] PHST- 2022/06/09 06:00 [pubmed] PHST- 2022/06/09 06:01 [medline] PHST- 2022/06/08 00:00 [pmc-release] AID - 10.1186/s12995-022-00354-2 [pii] AID - 354 [pii] AID - 10.1186/s12995-022-00354-2 [doi] PST - epublish SO - J Occup Med Toxicol. 2022 Jun 8;17(1):12. doi: 10.1186/s12995-022-00354-2. PMID- 27143949 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160504 LR - 20201001 IS - 1178-7058 (Print) IS - 1178-7058 (Electronic) IS - 1178-7058 (Linking) VI - 9 DP - 2016 TI - Angioplasty of forearm arteries as a finger salvage procedure for patient with end-stage renal failure. PG - 105-9 LID - 10.2147/IJNRD.S102257 [doi] AB - Due to the relatively low metabolic demand and extensive collaterals of the upper limb, peripheral arterial disease seldom leads to tissue loss, except in patients with end-stage renal failure (ESRF), rheumatologic diseases, Raynaud's disease, frostbites, or distal emboli. We report a case of a 51-year-old lady with ESRF who presented to our tertiary referral vascular center with infected gangrene of her right ring finger. Duplex ultrasound showed that her forearm arteries were severely diseased. Digital subtraction angiogram showed severe multilevel stenoses/occlusions in her forearm radial and ulnar arteries. These lesions were successfully angioplastized with 2 mm × 25 mm angioplasty balloon. Completion angiogram showed good radiological results with some post-dilatation spasm which improved with intra-arterial glyceryl trinitrate. The sepsis improved after revascularization, and the distal phalanx was allowed to self-demarcate with dressings and autoamputate with good clinical results. Our case illustrated that even in delayed setting, patients could still benefit from specialist vascular care with a combination of expert care and angioplasty of forearm arteries, with successful salvage of her finger. FAU - Law, Yuk AU - Law Y AD - Division of Vascular and Endovascular Surgery, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong. FAU - Chan, Yiu Che AU - Chan YC AD - Division of Vascular and Endovascular Surgery, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong. FAU - Cheng, Stephen Wing-Keung AU - Cheng SW AD - Division of Vascular and Endovascular Surgery, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong. LA - eng PT - Case Reports PT - Journal Article DEP - 20160418 PL - New Zealand TA - Int J Nephrol Renovasc Dis JT - International journal of nephrology and renovascular disease JID - 101550217 PMC - PMC4846062 OTO - NOTNLM OT - angioplasty OT - end-stage renal disease OT - finger septic gangrene OT - peripheral arterial disease OT - salvage EDAT- 2016/05/05 06:00 MHDA- 2016/05/05 06:01 PMCR- 2016/04/18 CRDT- 2016/05/05 06:00 PHST- 2016/05/05 06:00 [entrez] PHST- 2016/05/05 06:00 [pubmed] PHST- 2016/05/05 06:01 [medline] PHST- 2016/04/18 00:00 [pmc-release] AID - ijnrd-9-105 [pii] AID - 10.2147/IJNRD.S102257 [doi] PST - epublish SO - Int J Nephrol Renovasc Dis. 2016 Apr 18;9:105-9. doi: 10.2147/IJNRD.S102257. eCollection 2016. PMID- 27413384 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160714 LR - 20201001 IS - 1741-427X (Print) IS - 1741-4288 (Electronic) IS - 1741-427X (Linking) VI - 2016 DP - 2016 TI - Synergistic Effect of Ferulic Acid and Z-Ligustilide, Major Components of A. sinensis, on Regulating Cold-Sensing Protein TRPM8 and TPRA1 In Vitro. PG - 3160247 LID - 10.1155/2016/3160247 [doi] LID - 3160247 AB - Angelica sinensis has been used to attenuate cold-induced cutaneous vasospasm syndrome, such as Raynaud's disease and frostbite, in China for many years. Ferulic acid (PubChem CID: 445858) and Z-ligustilide (PubChem CID: 529865), two major components extracted from Angelica sinensis, had been reported to inhibit vasoconstriction induced by vasoconstrictors. In this study, the pharmacological interaction in regulating cold-induced vascular smooth muscle cell contraction via cold-sensing protein TRPM8 and TRPA1 was analyzed between ferulic acid and Z-ligustilide. Pharmacological interaction on inhibiting [Ca(2+)]i influx evoked by TRPM8 agonist WS-12 or TRPA1 agonist ASP 7663 as well as cold-induced upregulation of TRPM8 was determined using isobolographic analysis. The isobolograms demonstrated that the combinations investigated in this study produced a synergistic interaction. Combination effect of two components in inhibiting RhoA activation and phosphorylation of MLC20 induced by WS-12 or ASP 7663 was also being quantified. These findings suggest that the therapeutic effect of Angelica sinensis on cold-induced vasospasm may be partially attributed to combinational effect, via TRPM8 and TPRA1 way, between ferulic acid and Z-ligustilide. FAU - Pan, Yuwei AU - Pan Y AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. FAU - Zhao, Guoping AU - Zhao G AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. FAU - Cai, Zejian AU - Cai Z AD - Shipai District Community Health Service Center, Guangzhou 510630, China. FAU - Chen, Fengguo AU - Chen F AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. FAU - Xu, Dandan AU - Xu D AD - Guangdong Food and Drug Vocational College, Guangzhou 510520, China. FAU - Huang, Si AU - Huang S AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. FAU - Lan, Hai AU - Lan H AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. FAU - Tong, Yi AU - Tong Y AD - Department of Traditional Chinese Medicine, College of Medicine, Jinan University, Guangzhou 510630, China. LA - eng PT - Journal Article DEP - 20160620 PL - United States TA - Evid Based Complement Alternat Med JT - Evidence-based complementary and alternative medicine : eCAM JID - 101215021 PMC - PMC4931054 EDAT- 2016/07/15 06:00 MHDA- 2016/07/15 06:01 PMCR- 2016/06/20 CRDT- 2016/07/15 06:00 PHST- 2016/02/23 00:00 [received] PHST- 2016/05/16 00:00 [revised] PHST- 2016/05/31 00:00 [accepted] PHST- 2016/07/15 06:00 [entrez] PHST- 2016/07/15 06:00 [pubmed] PHST- 2016/07/15 06:01 [medline] PHST- 2016/06/20 00:00 [pmc-release] AID - 10.1155/2016/3160247 [doi] PST - ppublish SO - Evid Based Complement Alternat Med. 2016;2016:3160247. doi: 10.1155/2016/3160247. Epub 2016 Jun 20. PMID- 39803022 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250114 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 12 DP - 2024 Dec TI - Exploring New and Potential Indications for Botulinum Toxin Treatment: An Updated Literature Review. PG - e75549 LID - 10.7759/cureus.75549 [doi] LID - e75549 AB - Botulinum toxin (BoNT) has traditionally been utilized to relieve tension in muscular and connective tissue diseases (CTD). However, its usage has rapidly expanded and now encompasses usage for neurological, gastrointestinal, psychological, cardiovascular, ophthalmology, orthopedics, and more. More recently, its usage has been utilized for sequelae of CTDs such as Raynaud's disease and reduced oral aperture secondary to scleroderma/systemic sclerosis. Beyond its current applications, BoNT holds promise in various medical fields but is not FDA-approved in these conditions. Thus, the design and conduction of well-designed randomized controlled trials are essential in establishing the efficacy and safety of BoNT treatment which can help accelerate regulatory approval for new indications. The versatility of BoNT suggests that its therapeutic applications continue to expand, offering novel therapies for a wide range of conditions. This review aims to comprehensively evaluate the literature on BoNT's current FDA-approved indications and potential non-FDA uses in other medical conditions. Additionally, this review offers potential insights and future possibilities for BoNT treatment. CI - Copyright © 2024, Wen et al. FAU - Wen, Jimmy AU - Wen J AD - Physical Medicine and Rehabilitation, California Northstate University College of Medicine, Elk Grove, USA. FAU - Nadora, Dawnica AU - Nadora D AD - Dermatology, California Northstate University College of Medicine, Elk Grove, USA. FAU - Ansari, Ubaid AU - Ansari U AD - Neurology, California Northstate University College of Medicine, Elk Grove, USA. FAU - Syed, Burhaan AU - Syed B AD - Surgery, California Northstate University College of Medicine, Elk Grove, USA. FAU - Shehabat, Mouhamad AU - Shehabat M AD - Surgery, California Northstate University College of Medicine, Elk Grove, USA. FAU - Razick, Daniel I AU - Razick DI AD - Surgery, California Northstate University College of Medicine, Elk Grove, USA. FAU - Razick, Adam A AU - Razick AA AD - Life Sciences, University of California, Los Angeles, Los Angeles, USA. FAU - Rajagopal, Thiru AU - Rajagopal T AD - General Surgery, Mercy General Hospital, Sacramento, USA. LA - eng PT - Journal Article PT - Review DEP - 20241211 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11722663 OTO - NOTNLM OT - botox injections OT - botulinum (botox) OT - botulinum neurotoxin type-a OT - botulinum toxin OT - connective tissue disease COIS- Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/01/13 06:16 MHDA- 2025/01/13 06:17 PMCR- 2024/12/11 CRDT- 2025/01/13 05:38 PHST- 2024/12/11 00:00 [accepted] PHST- 2025/01/13 06:17 [medline] PHST- 2025/01/13 06:16 [pubmed] PHST- 2025/01/13 05:38 [entrez] PHST- 2024/12/11 00:00 [pmc-release] AID - 10.7759/cureus.75549 [doi] PST - epublish SO - Cureus. 2024 Dec 11;16(12):e75549. doi: 10.7759/cureus.75549. eCollection 2024 Dec. PMID- 31703554 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 1865-1372 (Print) IS - 1865-1380 (Electronic) IS - 1865-1372 (Linking) VI - 12 IP - 1 DP - 2019 Nov 8 TI - Rhabdomyolysis as an initial presentation of systemic lupus erythematosus: a case report. PG - 33 LID - 10.1186/s12245-019-0251-x [doi] LID - 33 AB - BACKGROUND: Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease which most commonly presents in women of reproductive age. It takes a relapsing-remitting course and may manifest as a variety of clinical symptoms, making it difficult to diagnose at first presentation, particularly in the emergency department (ED) setting. In active SLE, rhabdomyolysis has, thus far, not been reported as the sole initial presentation. CASE PRESENTATION: A 28-year-old presented to the ED with bilateral proximal arm swelling and pain. She had a previous history of Raynaud's disease. Creatine kinase was > 13,000 units/l (normal range 25-200), but renal function was preserved. She did not require hospital admission so was encouraged to take oral fluids and discontinue the combined oral contraceptive pill. Antinuclear antibody and anti-double-stranded DNA titres were highly elevated with low complement demonstrated. She was diagnosed with SLE and treated with an oral prednisolone course. Antibody titres remained high 6 months later, provoking the initiation of hydroxychloroquine therapy. CONCLUSIONS: We report with a view to recommend autoimmune screening in young patients for whom a cause of rhabdomyolysis is not clearly identified. FAU - Saxena, Gayatri AU - Saxena G AUID- ORCID: 0000-0002-7731-5284 AD - St George's Hospital, Blackshaw Road, Tooting, London, SW17 0QT, UK. gayatri.saxena@nhs.net. AD - Royal Surrey County Hospital, Egerton Road, Guildford, GU2 7XX, UK. gayatri.saxena@nhs.net. FAU - Mahdi, Ahmed AU - Mahdi A AD - St George's Hospital, Blackshaw Road, Tooting, London, SW17 0QT, UK. LA - eng PT - Journal Article DEP - 20191108 PL - England TA - Int J Emerg Med JT - International journal of emergency medicine JID - 101469435 PMC - PMC6842242 OTO - NOTNLM OT - Myopathy OT - Oral contraceptive OT - Rhabdomyolysis OT - Systemic lupus erythematosus COIS- The authors declare that they have no competing interests. EDAT- 2019/11/11 06:00 MHDA- 2019/11/11 06:01 PMCR- 2019/11/08 CRDT- 2019/11/10 06:00 PHST- 2019/09/21 00:00 [received] PHST- 2019/10/23 00:00 [accepted] PHST- 2019/11/10 06:00 [entrez] PHST- 2019/11/11 06:00 [pubmed] PHST- 2019/11/11 06:01 [medline] PHST- 2019/11/08 00:00 [pmc-release] AID - 10.1186/s12245-019-0251-x [pii] AID - 251 [pii] AID - 10.1186/s12245-019-0251-x [doi] PST - epublish SO - Int J Emerg Med. 2019 Nov 8;12(1):33. doi: 10.1186/s12245-019-0251-x. PMID- 34301693 OWN - NLM STAT- MEDLINE DCOM- 20210727 LR - 20230724 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 14 IP - 7 DP - 2021 Jul 23 TI - Alternative cause for hypoglycaemia in insulin-treated diabetes mellitus. LID - 10.1136/bcr-2020-241439 [doi] LID - e241439 AB - We present a case of a 73-year-old woman who developed recurrent hypoglycaemia during a prolonged hospital stay following a mechanical fall. She had a complex history of insulin-treated diabetes mellitus, hypothyroidism, diffuse systemic cutaneous sclerosis, Raynaud's disease, previous breast cancer, Barrett's oesophagus and previous partial gastrectomy for a benign mass. Hypoglycaemia persisted despite weaning of insulin. She had no clinical features of adrenal or pituitary insufficiency with an acceptable cortisol on stopping prednisolone and had an optimal thyroid replacement. A 72-hour fast elicited hypoglycaemia with corresponding low insulin level. Although the C-peptide was detectable, there were no clinical, biochemical or radiological features suggestive of insulinoma. Reactive hypoglycaemia post partial gastrectomy was ruled out based on limited relation of the hypoglycaemia to meals and the low insulin levels. Hydroxychloroquine (HCQ)-induced hypoglycaemia was considered based on previous case reports and the recent literature, with a successful resolution of hypoglycaemia on discontinuation of HCQ. CI - © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Parmar, Viraj AU - Parmar V AD - General Medicine, Luton and Dunstable University Hospital, Bedfordshire Hospitals NHS Foundation Trust, Luton, UK. FAU - Sritharan, Vasanth AU - Sritharan V AUID- ORCID: 0000-0002-0458-4168 AD - General Medicine, Luton and Dunstable University Hospital, Bedfordshire Hospitals NHS Foundation Trust, Luton, UK. FAU - Lawrence, Christopher AU - Lawrence C AD - Renal Department, Luton and Dunstable University Hospital, Bedfordshire Hospitals NHS Foundation Trust, Luton, UK. FAU - Dhere, Archana AU - Dhere A AD - Endocrinology & Diabetes, Luton and Dunstable University Hospital, Bedfordshire Hospitals NHS Foundation Trust, Luton, UK archana.dhere@nhs.net. LA - eng PT - Case Reports PT - Journal Article DEP - 20210723 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (C-Peptide) RN - 0 (Insulin) SB - IM MH - Aged MH - C-Peptide MH - *Diabetes Mellitus, Type 2 MH - Female MH - Humans MH - *Hypoglycemia/chemically induced/drug therapy MH - Insulin MH - *Pancreatic Neoplasms PMC - PMC8311326 OTO - NOTNLM OT - diabetes OT - drugs: endocrine system OT - endocrine system OT - endocrinology OT - metabolic disorders COIS- Competing interests: None declared. EDAT- 2021/07/25 06:00 MHDA- 2021/07/28 06:00 PMCR- 2023/07/23 CRDT- 2021/07/24 05:38 PHST- 2021/07/24 05:38 [entrez] PHST- 2021/07/25 06:00 [pubmed] PHST- 2021/07/28 06:00 [medline] PHST- 2023/07/23 00:00 [pmc-release] AID - 14/7/e241439 [pii] AID - bcr-2020-241439 [pii] AID - 10.1136/bcr-2020-241439 [doi] PST - epublish SO - BMJ Case Rep. 2021 Jul 23;14(7):e241439. doi: 10.1136/bcr-2020-241439. PMID- 40739662 OWN - NLM STAT- Publisher LR - 20250731 IS - 2194-802X (Electronic) IS - 2194-802X (Linking) DP - 2025 Aug 1 TI - Misdiagnosis in carpal tunnel syndrome: amyloidosis and other red flags. A narrative review. LID - 10.1515/dx-2025-0063 [doi] AB - INTRODUCTION: Carpal tunnel syndrome (CTS) involves the entrapment of the median nerve at the wrist. Despite acceptable sensitivity and specificity in diagnostics tests, errors persist, leading to unsuccessful treatments, especially when CTS is an early sign of other conditions. CONTENT: This review aims to identify red flags that may manifest as CTS or coexist with it, and to describe their clinical presentations. SUMMARY: A PubMed search (2000-2025) yielded 622 articles, with 24 included in the review. Of these, 12 articles explored CTS and amyloidosis, three with neurological pathologies, three with tumours, two with rheumatic diseases, one with Raynaud's disease, one on CTS in children, and two with other conditions. OUTLOOK: Many conditions can be mistaken for CTS. Given its high prevalence, healthcare professionals must distinguish these to reduce surgical failures and improve early detection of conditions like cardiac amyloidosis or multiple sclerosis. CI - © 2025 Walter de Gruyter GmbH, Berlin/Boston. FAU - Menéndez-Cámara, Jorge AU - Menéndez-Cámara J AD - Department of Physical Therapy, Occupational Therapy, Rehabilitation and Physical Medicine, Universidad Rey Juan Carlos, Alcorcón, Spain. AD - Cognitive Neuroscience, Pain and Rehabilitation Research Group (NECODOR), Faculty of Health Sciences, Universidad Rey Juan Carlos, Madrid, Spain. AD - Department of Physical Therapy, Occupational Therapy, Rehabilitation and Physical Medicine, Escuela Internacional de Doctorado, Universidad Rey Juan Carlos, Alcorcón, Spain. FAU - Cecilia-López, David AU - Cecilia-López D AD - Unit of Elbow-Hand, Service of Traumatology and Orthopedics, Hospital 12 de Octubre, Madrid, Spain. AD - Complutense University of Madrid, Madrid, Spain. AD - Department of Surgery, Hospital Vithas La Milagrosa, Madrid, Spain. AD - Hospital Viamed Santa Elena, Madrid, Spain. FAU - García-Lamas, Lorena AU - García-Lamas L AD - Unit of Elbow-Hand, Service of Traumatology and Orthopedics, Hospital 12 de Octubre, Madrid, Spain. FAU - Castillo-Fernández, Celia AU - Castillo-Fernández C AD - Unit of Elbow-Hand, Service of Traumatology and Orthopedics, Hospital 12 de Octubre, Madrid, Spain. FAU - Matesanz-García, Luis AU - Matesanz-García L AUID- ORCID: 0000-0001-5148-6336 AD - Department of Physical Therapy, Occupational Therapy, Rehabilitation and Physical Medicine, Universidad Rey Juan Carlos, Alcorcón, Spain. AD - Cognitive Neuroscience, Pain and Rehabilitation Research Group (NECODOR), Faculty of Health Sciences, Universidad Rey Juan Carlos, Madrid, Spain. AD - 165113 CranioSPain Research Group. Centro Superior de Estudios Universitarios La Salle , Alcorcón, Spain. AD - Department of Physiotherapy, Centro Superior de Estudios Universitarios La Salle, Universidad Autónoma de Madrid, Madrid, Spain. FAU - Fernández-Carnero, Josué AU - Fernández-Carnero J AD - Department of Physical Therapy, Occupational Therapy, Rehabilitation and Physical Medicine, Universidad Rey Juan Carlos, Alcorcón, Spain. AD - Cognitive Neuroscience, Pain and Rehabilitation Research Group (NECODOR), Faculty of Health Sciences, Universidad Rey Juan Carlos, Madrid, Spain. AD - La Paz Hospital Institute for Health Research, IdiPAZ, Madrid, Spain. AD - Musculoskeletal Pain and Motor Control Research Group, Faculty of Sport Sciences, Universidad Europea de Madrid, Villaviciosa de Odón, Spain. LA - eng PT - Journal Article PT - Review DEP - 20250801 PL - Germany TA - Diagnosis (Berl) JT - Diagnosis (Berlin, Germany) JID - 101654734 SB - IM OTO - NOTNLM OT - amyloidosis OT - carpal tunnel syndrome OT - clinical reasoning OT - diagnosis OT - red flags EDAT- 2025/07/31 06:27 MHDA- 2025/07/31 06:27 CRDT- 2025/07/31 01:03 PHST- 2025/04/23 00:00 [received] PHST- 2025/06/27 00:00 [accepted] PHST- 2025/07/31 06:27 [medline] PHST- 2025/07/31 06:27 [pubmed] PHST- 2025/07/31 01:03 [entrez] AID - dx-2025-0063 [pii] AID - 10.1515/dx-2025-0063 [doi] PST - aheadofprint SO - Diagnosis (Berl). 2025 Aug 1. doi: 10.1515/dx-2025-0063. PMID- 38620644 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20260311 IS - 2772-6134 (Electronic) IS - 2772-6134 (Linking) VI - 1 DP - 2021 Dec TI - Pfizer-biontech COVID-19 RNA vaccination induces phosphatidylserine autoantibodies, cryoglobulinemia, and digital necrosis in a patient with pre-existing autoimmunity. PG - 1-3 LID - 10.1016/j.clicom.2021.08.001 [doi] AB - We describe a 64-year-old Caucasian female with a history of Raynaud's disease, hand arthritis, photosensitivity, Sjogren's syndrome and leukocytoclastic vasculitis who presented with progressively worsening fingertip necrosis that began three days after receiving a first dose of Pfizer-BioNTech COVID-19 RNA vaccine. Our workup revealed cryoglobulinemia, hypocomplementemia, elevated antinuclear antibodies (ANA) and IgM antiphospholipid autoantibodies (aPL) directed against phosphatidylserine (aPL-PS), suggesting a diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). The patient failed to develop anti-spike IgG antibodies up to two months following vaccination. Disease progression was halted by plasmapheresis, anticoagulation, and immune suppression. We conclude that the vaccine RNA moiety may induce SLE manifesting in APS, cryoglobulinemia, hypocomplementemia, and digital necrosis. CI - © 2021 The Authors. FAU - Nasr, Sandy AU - Nasr S AD - Department of Medicine, College of Medicine, State University of New York, 750 East Adams Street, Syracuse, NY 13210, United States. FAU - Khalil, Sara AU - Khalil S AD - Department of Medicine, College of Medicine, State University of New York, 750 East Adams Street, Syracuse, NY 13210, United States. FAU - Poiesz, Bernard J AU - Poiesz BJ AD - Department of Medicine, College of Medicine, State University of New York, 750 East Adams Street, Syracuse, NY 13210, United States. FAU - Banki, Katalin AU - Banki K AD - Department of Medicine, College of Medicine, State University of New York, 750 East Adams Street, Syracuse, NY 13210, United States. FAU - Perl, Andras AU - Perl A AD - Department of Medicine, College of Medicine, State University of New York, 750 East Adams Street, Syracuse, NY 13210, United States. LA - eng GR - R01 AT004332/AT/NCCIH NIH HHS/United States GR - R01 AI122176/AI/NIAID NIH HHS/United States GR - R34 AI141304/AI/NIAID NIH HHS/United States GR - R01 AI048079/AI/NIAID NIH HHS/United States GR - R21 AI061066/AI/NIAID NIH HHS/United States GR - R01 AI072648/AI/NIAID NIH HHS/United States PT - Case Reports PT - Journal Article DEP - 20210927 PL - United States TA - Clin Immunol Commun JT - Clinical immunology communications JID - 9918697481206676 EIN - Clin Immunol Commun. 2023 Dec;3:59-60. doi: 10.1016/j.clicom.2023.05.001. PMID: 38014401 PMC - PMC8486180 OTO - NOTNLM OT - Antiphospholipid antibody OT - Antiphospholipid syndrome OT - Cryoglobulinemia OT - Digital necrosis OT - Pfizer–BioNTech COVID-19 RNA vaccine OT - Phosphatidylserine antibody OT - Systemic lupus erythematosus OT - Vasculitis COIS- The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2021/12/01 00:00 MHDA- 2021/12/01 00:01 PMCR- 2021/09/27 CRDT- 2024/04/15 14:41 PHST- 2021/08/21 00:00 [received] PHST- 2021/08/25 00:00 [accepted] PHST- 2021/12/01 00:01 [medline] PHST- 2021/12/01 00:00 [pubmed] PHST- 2024/04/15 14:41 [entrez] PHST- 2021/09/27 00:00 [pmc-release] AID - S2772-6134(21)00002-0 [pii] AID - 10.1016/j.clicom.2021.08.001 [doi] PST - ppublish SO - Clin Immunol Commun. 2021 Dec;1:1-3. doi: 10.1016/j.clicom.2021.08.001. Epub 2021 Sep 27. PMID- 38179435 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250402 IS - 0972-978X (Print) IS - 0972-978X (Electronic) IS - 0972-978X (Linking) VI - 50 DP - 2024 Apr TI - Distal periarterial sympathectomy surgery for chronic digital ischemia: A systematic review of the literature. PG - 76-83 LID - 10.1016/j.jor.2023.11.072 [doi] AB - OBJECTIVE: This study assesses the efficacy of distal periarterial sympathectomy in treating chronic digital ischemia by evaluating clinical outcomes of surgery. METHODS: A systematic literature review of distal sympathectomy for chronic digital ischemia was conducted. Data extracted included study design, patient statistics, aetiology, follow-up duration, sympathectomy level, and surgical outcomes. RESULTS: 21 studies were analysed, containing a total of 337 patients, 324 hands, and 398 digits. Patient age ranged from 23.2 to 56.6 years. Causes of ischemia included Scleroderma, Raynaud's disease, atherosclerosis/Buerger's disease, systemic lupus erythematosus/discoid lupus, undifferentiated rheumatic disorder/mixed connective tissue disease, CREST syndrome, trauma and unknown diagnoses. Common digital artery sympathectomy was mostly performed. Follow-up spanned 12-120 months. OUTCOMES: Distal sympathectomy led to reduced pain in 94.7 % patients. Complete resolution of ulceration was seen in 73 % patients. Subsequent amputation was required in 28 % patients. Other complications were reported in 24.1 % patients. CONCLUSIONS: This study indicates that distal periarterial sympathectomy may effectively treat chronic digital ischemia, offering pain relief and resolution of digital ulceration. However, risks of complications and amputation persist. Further research is required to inform patient selection and establish the optimal technique and extent of distal sympathectomy surgery, before it can be considered a valid treatment option. CI - Crown Copyright © 2023 Published by Elsevier B.V. on behalf of Professor P K Surendran Memorial Education Foundation. All rights reserved. FAU - Cereceda-Monteoliva, Nicholas AU - Cereceda-Monteoliva N AD - Queen Victoria Hospital NHS Trust, Holtye Rd, East Grinstead, UK. FAU - Smart, Yat Wing AU - Smart YW AD - West Hertfordshire Hospitals NHS Trust, Vicarage Rd, Watford, UK. FAU - Ojelade, Elizabeth AU - Ojelade E AD - West Hertfordshire Hospitals NHS Trust, Vicarage Rd, Watford, UK. FAU - Schaller, Gavin AU - Schaller G AD - Queen Victoria Hospital NHS Trust, Holtye Rd, East Grinstead, UK. FAU - Berber, Onur AU - Berber O AD - Royal Free Hospital NHS Foundation Trust, Pond St, London, UK. LA - eng PT - Journal Article DEP - 20231206 PL - India TA - J Orthop JT - Journal of orthopaedics JID - 101233220 PMC - PMC10762319 OTO - NOTNLM OT - Chronic digital ischemia OT - Digital sympathectomy OT - Hand surgery OT - Periarterial sympathectomy OT - Ulcer COIS- The authors declare that they have no competing interests. EDAT- 2024/01/05 06:42 MHDA- 2024/01/05 06:43 PMCR- 2025/04/01 CRDT- 2024/01/05 04:00 PHST- 2023/10/09 00:00 [received] PHST- 2023/11/26 00:00 [revised] PHST- 2023/11/27 00:00 [accepted] PHST- 2024/01/05 06:43 [medline] PHST- 2024/01/05 06:42 [pubmed] PHST- 2024/01/05 04:00 [entrez] PHST- 2025/04/01 00:00 [pmc-release] AID - S0972-978X(23)00348-3 [pii] AID - 10.1016/j.jor.2023.11.072 [doi] PST - epublish SO - J Orthop. 2023 Dec 6;50:76-83. doi: 10.1016/j.jor.2023.11.072. eCollection 2024 Apr. PMID- 38130955 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231223 IS - 2233-4718 (Electronic) IS - 2093-940X (Print) IS - 2233-4718 (Linking) VI - 31 IP - 1 DP - 2024 Jan 1 TI - Clinical characteristics of juvenile systemic sclerosis in Korea: 31-year single-center study. PG - 25-32 LID - 10.4078/jrd.2023.0046 [doi] AB - OBJECTIVE: To evaluate the clinical and laboratory characteristics, therapeutic drugs, and prognosis of juvenile systemic sclerosis (JSSc) at a single center in Korea. METHODS: This study was a retrospective analysis of patients with JSSc aged <16 years at disease onset and who were treated at our hospital between January 1992 and April 2023. All patients met the Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc, and those with localized scleroderma (morphea) were excluded. RESULTS: Among the 13 patients, proximal skin sclerosis (100%), Raynaud's phenomenon (RP) (84.6%), and sclerodactyly (69.2%) were present at the time of diagnosis. The most common symptom before diagnosis was RP, which was present in 10 patients (76.9%), whereas proximal skin sclerosis was observed in only five patients (38.5%). Thirteen patients had positive anti-nuclear antibody (ANA). At the time of diagnosis, five individuals had findings suggestive of interstitial lung disease (ILD) on a pulmonary function test (PFT) or chest computed tomography (CT), two of whom were asymptomatic. During follow-up, three patients developed ILD, one developed renal dysfunction, one developed heart disease, and none died. CONCLUSION: This study was the first descriptive analysis of clinical features of JSSc in South Korea. Clinical suspicion is essential for diagnosing JSSc in patients with RP, especially if ANA is positive; however, proximal skin sclerosis, which is crucial for diagnosing JSSc, was unrecognized in the early phase of the disease. PFT should be considered even if a patient is asymptomatic or has normal chest CT. CI - Copyright © 2024 by The Korean College of Rheumatology. All rights reserved. FAU - Jeong, Ji Eun AU - Jeong JE AUID- ORCID: 0009-0004-2667-8618 AD - Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. FAU - Kim, Seong Heon AU - Kim SH AUID- ORCID: 0000-0001-8003-3010 AD - Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. LA - eng PT - Journal Article DEP - 20231206 PL - Korea (South) TA - J Rheum Dis JT - Journal of rheumatic diseases JID - 101571816 PMC - PMC10730803 OTO - NOTNLM OT - Anti-nuclear antibody OT - Interstitial lung diseases OT - Juvenile systemic scleroderma OT - Raynaud disease COIS- CONFLICT OF INTEREST No potential conflict of interest relevant to this article was reported. EDAT- 2023/12/22 06:42 MHDA- 2023/12/22 06:43 PMCR- 2023/12/06 CRDT- 2023/12/22 04:08 PHST- 2023/08/10 00:00 [received] PHST- 2023/09/28 00:00 [revised] PHST- 2023/10/30 00:00 [accepted] PHST- 2023/12/22 06:43 [medline] PHST- 2023/12/22 06:42 [pubmed] PHST- 2023/12/22 04:08 [entrez] PHST- 2023/12/06 00:00 [pmc-release] AID - jrd-31-1-25 [pii] AID - 10.4078/jrd.2023.0046 [doi] PST - ppublish SO - J Rheum Dis. 2024 Jan 1;31(1):25-32. doi: 10.4078/jrd.2023.0046. Epub 2023 Dec 6. PMID- 23106952 OWN - NLM STAT- MEDLINE DCOM- 20130619 LR - 20191112 IS - 1482-1826 (Electronic) IS - 1482-1826 (Linking) VI - 15 IP - 4 DP - 2012 TI - An old drug for a new application: potential benefits of sildenafil in wound healing. PG - 483-98 AB - PURPOSE: Several studies have evaluated the effects of sildenafil on the tissue repair and wound healing. In the present review, the impact of sildenafil on the wound healing in all available clinical and non-clinical (experimental) studies has been discussed. METHODS: A literature search was performed using PubMed, Scopus, Medline, Embase, Cochrane central register of controlled trials and Cochrane database systematic reviews. Related articles indexed in Google Scholar were also included. Key words used as search terms were 'phosphodiesterase inhibitor', 'sildenafil', 'skin', 'cutaneous', 'skin lesion', 'skin damage', 'wound', and 'wound healing'. No time limitation was considered in this review. RESULTS: A total of 15 animal studies, 7 case reports, and 2 small clinical studies have reported the effects of sildenafil on the wound healing. The effects included skin flaps and grafts, anastomosis, systemic sclerosis and Raynaud's disease. CONCLUSIONS: The available data support the beneficial effects of sildenafil in improvement of tissue healing in various conditions. This article is open to POST-PUBLICATION REVIEW. Registered readers (see "For Readers") may comment by clicking on ABSTRACT on the issue's contents page. FAU - Farsaie, Shadi AU - Farsaie S AD - Department of Clinical Pharmacy, Faculty of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran. FAU - Khalili, Hossein AU - Khalili H FAU - Karimzadeh, Iman AU - Karimzadeh I FAU - Dashti-Khavidaki, Simin AU - Dashti-Khavidaki S LA - eng PT - Journal Article PT - Review PL - Switzerland TA - J Pharm Pharm Sci JT - Journal of pharmacy & pharmaceutical sciences : a publication of the Canadian Society for Pharmaceutical Sciences, Societe canadienne des sciences pharmaceutiques JID - 9807281 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Animals MH - Humans MH - Piperazines/*pharmacology/*therapeutic use MH - Purines/pharmacology/therapeutic use MH - Sildenafil Citrate MH - Sulfones/*pharmacology/*therapeutic use MH - Wound Healing/*drug effects EDAT- 2012/10/31 06:00 MHDA- 2013/06/20 06:00 CRDT- 2012/10/31 06:00 PHST- 2012/10/31 06:00 [entrez] PHST- 2012/10/31 06:00 [pubmed] PHST- 2013/06/20 06:00 [medline] AID - 10.18433/j3tc7v [doi] PST - ppublish SO - J Pharm Pharm Sci. 2012;15(4):483-98. doi: 10.18433/j3tc7v. PMID- 18468546 OWN - NLM STAT- MEDLINE DCOM- 20080522 LR - 20220409 IS - 1474-547X (Electronic) IS - 0140-6736 (Linking) VI - 371 IP - 9624 DP - 2008 May 10 TI - Non-peptide arginine-vasopressin antagonists: the vaptans. PG - 1624-32 LID - 10.1016/S0140-6736(08)60695-9 [doi] AB - Arginine-vasopressin is a hormone that plays an important part in circulatory and water homoeostasis. The three arginine-vasopressin-receptor subtypes--V1a, V1b, and V2--all belong to the large rhodopsin-like G-protein-coupled receptor family. The vaptans are orally and intravenously active non-peptide vasopressin receptor antagonists that are in development. Relcovaptan is a selective V1a-receptor antagonist, which has shown initial positive results in the treatment of Raynaud's disease, dysmenorrhoea, and tocolysis. SSR-149415 is a selective V1b-receptor antagonist, which could have beneficial effects in the treatment of psychiatric disorders. V2-receptor antagonists--mozavaptan, lixivaptan, satavaptan, and tolvaptan--induce a highly hypotonic diuresis without substantially affecting the excretion of electrolytes (by contrast with the effects of diuretics). These drugs are all effective in the treatment of euvolaemic and hypervolaemic hyponatraemia. Conivaptan is a V1a/V2 non-selective vasopressin-receptor antagonist that has been approved by the US Food and Drug Administration as an intravenous infusion for the inhospital treatment of euvolaemic or hypervolaemic hyponatraemia. FAU - Decaux, Guy AU - Decaux G AD - Department of Internal Medicine, Erasmus University Hospital, Brussels, Belgium. guy.decaux@skynet.be FAU - Soupart, Alain AU - Soupart A FAU - Vassart, Gilbert AU - Vassart G LA - eng PT - Journal Article PT - Review PL - England TA - Lancet JT - Lancet (London, England) JID - 2985213R RN - 0 (Antidiuretic Agents) RN - 0 (Antidiuretic Hormone Receptor Antagonists) RN - 0 (Benzazepines) RN - 0 (Receptors, Vasopressin) RN - 0NJ98Y462X (conivaptan) RN - 113-79-1 (Arginine Vasopressin) SB - IM MH - Animals MH - Antidiuretic Agents/therapeutic use MH - Antidiuretic Hormone Receptor Antagonists MH - *Arginine Vasopressin/physiology/therapeutic use MH - Benzazepines/adverse effects/*therapeutic use MH - Clinical Trials as Topic MH - Contraindications MH - Humans MH - Hyponatremia/drug therapy MH - Models, Molecular MH - *Receptors, Vasopressin/physiology RF - 79 EDAT- 2008/05/13 09:00 MHDA- 2008/05/23 09:00 CRDT- 2008/05/13 09:00 PHST- 2008/05/13 09:00 [pubmed] PHST- 2008/05/23 09:00 [medline] PHST- 2008/05/13 09:00 [entrez] AID - S0140-6736(08)60695-9 [pii] AID - 10.1016/S0140-6736(08)60695-9 [doi] PST - ppublish SO - Lancet. 2008 May 10;371(9624):1624-32. doi: 10.1016/S0140-6736(08)60695-9. PMID- 41879202 OWN - NLM STAT- Publisher LR - 20260502 IS - 1615-7109 (Electronic) IS - 1203-4754 (Linking) DP - 2026 Mar 25 TI - L-Carnitine in Dermatology: A Systematic Review of Therapeutic Potential and Biomarker Applications. PG - 12034754261427402 LID - 10.1177/12034754261427402 [doi] AB - L-Carnitine, an amino acid derivative critical for fatty acid metabolism, exhibits anti-inflammatory, antioxidant, and cytoprotective properties, with emerging applications in dermatology. This review explores its therapeutic potential, both topically and systemically, and its role as a biomarker in various skin disorders. A systematic search of Medline, EMBASE, and PubMed (1946-January 2025) was conducted, focusing on L-carnitine and dermatologic conditions, excluding acetyltransferases, acyltransferases, breast, and genetic metabolic diseases. From 420 articles, 59 were included after screening in Covidence. Data on study design, sample size, carnitine type, and outcomes were extracted and validated by two authors. Topically, L-carnitine (1%-5%) reduces acne lesions, sebum production, post-inflammatory hyperpigmentation, and cellulite, particularly when combined with agents like licochalcone-A or salicylic acid. Systemic L-carnitine mitigates inflammation, oxidative stress, and treatment-related side effects (eg, from corticosteroids, isotretinoin, vismodegib) while improving skin elasticity, microcirculation, wound healing, and fibrosis in conditions like Raynaud's disease, venous leg ulcers, and sclerotic disorders. As a biomarker, altered carnitine levels and metabolites reflect disease mechanisms in genodermatoses (eg, restrictive dermopathy, recessive dystrophic epidermolysis bullosa), inflammatory dermatoses, and melanoma, aiding diagnosis and monitoring. L-Carnitine shows promise as a therapeutic and diagnostic tool in dermatology, with benefits in acne, sebum control, post-inflammatory hyperpigmentation, cellulite, inflammation, and tissue repair. Its biomarker potential enhances disease insights and personalized care. Further dermatology-specific trials are needed to optimize dosing, formulations, and long-term safety, particularly regarding trimethylamine-N-oxide-related cardiovascular risks. FAU - Chow, Eunice Y AU - Chow EY AUID- ORCID: 0000-0001-9222-1828 AD - Department of Medicine, Division of Dermatology, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB, Canada. FAU - Sapijaszko, Mariusz AU - Sapijaszko M AUID- ORCID: 0000-0002-8624-1205 AD - Department of Medicine, Division of Dermatology, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB, Canada. LA - eng PT - Journal Article PT - Review DEP - 20260325 PL - United States TA - J Cutan Med Surg JT - Journal of cutaneous medicine and surgery JID - 9614685 SB - IM OTO - NOTNLM OT - L-carnitine OT - acetyl-L-carnitine OT - acne OT - amino acid OT - biomarker OT - carnitine OT - dermatology OT - inflammatory dermatoses OT - sebum OT - wound healing EDAT- 2026/03/25 12:48 MHDA- 2026/03/25 12:48 CRDT- 2026/03/25 08:33 PHST- 2026/03/25 12:48 [pubmed] PHST- 2026/03/25 12:48 [medline] PHST- 2026/03/25 08:33 [entrez] AID - 10.1177/12034754261427402 [doi] PST - aheadofprint SO - J Cutan Med Surg. 2026 Mar 25:12034754261427402. doi: 10.1177/12034754261427402. PMID- 20874808 OWN - NLM STAT- MEDLINE DCOM- 20111207 LR - 20250529 IS - 1748-1716 (Electronic) IS - 1748-1708 (Print) IS - 1748-1708 (Linking) VI - 203 IP - 1 DP - 2011 Sep TI - Sex, hormones and neuroeffector mechanisms. PG - 155-65 LID - 10.1111/j.1748-1716.2010.02192.x [doi] AB - Incidence and rate of cardiovascular disease differ between men and women across the life span. Although hypertension is more prominent in men than women, there is a group of vasomotor disorders [i.e. Raynaud's disease, postural orthostatic tachycardia syndrome and vasomotor symptoms (hot flashes) of menopause and migraine] with a female predominance. Both sex and hormones interact to modulate neuroeffector mechanisms including integrated regulation of the Sry gene and direct effect of sex steroid hormones on synthesis, release and disposition of monoamine neurotransmitters, and distribution and sensitivity of their receptors in brain areas associated with autonomic control. The interaction of the sex chromosomes and steroids also modulates these effector tissues, that is, the heart, vascular smooth muscle and endothelium. Although involvement of central serotonergic centres has been studied in regard to mood disorders such as depression, their contribution to cardiovascular risk is gaining attention. Studies are needed to further evaluate how hormonal treatments and drugs used to modulate adrenergic and serotonergic activity affect progression and risk for cardiovascular disease in men and women. CI - © 2010 Mayo Clinic. Acta Physiologica © 2010 Scandinavian Physiological Society. FAU - Hart, E C AU - Hart EC AD - Department of Anesthesia, Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN 55905, USA. FAU - Charkoudian, N AU - Charkoudian N FAU - Miller, V M AU - Miller VM LA - eng GR - R01 HL083947/HL/NHLBI NIH HHS/United States GR - R01 HL090639/HL/NHLBI NIH HHS/United States GR - UL1 RR024150/RR/NCRR NIH HHS/United States GR - HL 083947/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20101109 PL - England TA - Acta Physiol (Oxf) JT - Acta physiologica (Oxford, England) JID - 101262545 RN - 0 (Gonadal Steroid Hormones) SB - IM MH - Animals MH - Cardiovascular Diseases/*metabolism/physiopathology MH - Cardiovascular System/*metabolism/physiopathology MH - Female MH - Gonadal Steroid Hormones/*physiology MH - Humans MH - Male MH - *Sex Characteristics PMC - PMC3025263 MID - NIHMS240078 COIS- Conflict of Interest: This work was supported in part by grants from NIH HL 083947, UL1 RR024150 and the Mayo Clinic. Dr. Miller is President of the Organization for the Study of Sex Differences and serves on the Board of the Society for Women’rsquo;s Health Research. EDAT- 2010/09/30 06:00 MHDA- 2011/12/13 00:00 PMCR- 2012/09/01 CRDT- 2010/09/30 06:00 PHST- 2010/09/30 06:00 [entrez] PHST- 2010/09/30 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] PHST- 2012/09/01 00:00 [pmc-release] AID - 10.1111/j.1748-1716.2010.02192.x [doi] PST - ppublish SO - Acta Physiol (Oxf). 2011 Sep;203(1):155-65. doi: 10.1111/j.1748-1716.2010.02192.x. Epub 2010 Nov 9. PMID- 24585475 OWN - NLM STAT- MEDLINE DCOM- 20141027 LR - 20140303 IS - 1522-1229 (Electronic) IS - 1043-4046 (Linking) VI - 38 IP - 1 DP - 2014 Mar TI - The sympathetic release test: a test used to assess thermoregulation and autonomic control of blood flow. PG - 87-92 LID - 10.1152/advan.00095.2013 [doi] AB - When a subject is heated, the stimulation of temperature-sensitive nerve endings in the skin, and the raising of the central body temperature, results in the reflex release of sympathetic vasoconstrictor tone in the skin of the extremities, causing a measurable temperature increase at the site of release. In the sympathetic release test, the subject is gently heated by placing the feet and calves in a commercially available foot warming pouch or immersing the feet and calves in warm water and wrapping the subject in blankets. Skin blood flow is estimated from measurements of skin temperature in the fingers. Normally skin temperature of the fingers is 65-75°F in cool conditions (environmental temperature: 59-68°F) and rises to 85-95°F during body heating. Deviations in this pattern may mean that there is abnormal sympathetic vasoconstrictor control of skin blood flow. Abnormal skin blood flow can substantially impair an individual's ability to thermoregulate and has important clinical implications. During whole body heating, the skin temperature from three different skin sites is monitored and oral temperature is monitored as an index of core temperature. Students determine the fingertip temperature at which the reflex release of sympathetic activity occurs and its maximal attainment, which reflects the vasodilating capacity of this cutaneous vascular bed. Students should interpret typical sample data for certain clinical conditions (Raynaud's disease, peripheral vascular disease, and postsympathectomy) and explain why there may be altered skin blood flow in these disorders. FAU - Tansey, E A AU - Tansey EA AD - Centre for Biomedical Sciences Education, School of Medicine Dentistry and Biomedical Science, Queen's University Belfast, Belfast, Northern Ireland, United Kingdom. FAU - Roe, S M AU - Roe SM FAU - Johnson, C J AU - Johnson CJ LA - eng PT - Journal Article PL - United States TA - Adv Physiol Educ JT - Advances in physiology education JID - 100913944 SB - IM MH - Blood Flow Velocity MH - Blood Vessels/*innervation MH - *Body Temperature Regulation MH - Comprehension MH - Curriculum MH - *Hemodynamics MH - Humans MH - Learning MH - Lower Extremity MH - Models, Cardiovascular MH - Physiology/*education MH - Reflex MH - Regional Blood Flow MH - Skin/*blood supply MH - Students MH - Sympathetic Nervous System/*physiology MH - Teaching/*methods MH - Vasoconstriction MH - Vasodilation OTO - NOTNLM OT - heat stress OT - skin blood flow OT - skin temperature OT - sympathetic vasoconstrictor nerves EDAT- 2014/03/04 06:00 MHDA- 2014/10/28 06:00 CRDT- 2014/03/04 06:00 PHST- 2014/03/04 06:00 [entrez] PHST- 2014/03/04 06:00 [pubmed] PHST- 2014/10/28 06:00 [medline] AID - 38/1/87 [pii] AID - 10.1152/advan.00095.2013 [doi] PST - ppublish SO - Adv Physiol Educ. 2014 Mar;38(1):87-92. doi: 10.1152/advan.00095.2013. PMID- 16794787 OWN - NLM STAT- MEDLINE DCOM- 20060929 LR - 20260128 IS - 1420-682X (Print) IS - 1420-9071 (Electronic) IS - 1420-682X (Linking) VI - 63 IP - 15 DP - 2006 Aug TI - Vasopressin antagonists. PG - 1766-79 LID - 1766 AB - Effects of vasopressin via V1a- and V2-receptors are closely implicated in a variety of water-retaining diseases and cardiovascular diseases, including heart failure, hyponatraemia, hypertension, renal diseases, syndrome of inappropriate antidiuretic hormone secretion, cirrhosis and ocular hypertension. As vasopressin receptors are found in many different tissues, vasopressin antagonists may benefit the treatment of disorders such as cerebral ischaemia and stroke, Raynaud's disease, dysmenorrhoea and tocolytic treatment. V1b selective vasopressin antagonists are discussed in terms of their usefulness in the treatment of emotional and psychiatric disorders. The vaptans are vasopressin receptor antagonists with V1a (relcovaptan) or V2 (tolvaptan, lixivaptan) selectivity or non-selective activity (conivaptan) which may be advantageous in some disorders. The V1a/V2 non-selective vasopressin antagonist conivaptan is the first vaptan which is approved by the FDA for the treatment of euvolaemic hyponatraemia. FAU - Lemmens-Gruber, R AU - Lemmens-Gruber R AD - Department of Pharmacology and Toxicology, University of Vienna, 1090 Vienna, Austria. rosa.lemmens@univie.ac.at FAU - Kamyar, M AU - Kamyar M LA - eng PT - Journal Article PT - Review PL - Switzerland TA - Cell Mol Life Sci JT - Cellular and molecular life sciences : CMLS JID - 9705402 RN - 0 (Antidiuretic Hormone Receptor Antagonists) RN - 0 (Azepines) RN - 0 (Benzamides) RN - 0 (Benzazepines) RN - 0 (Hormone Antagonists) RN - 0 (Indoles) RN - 0 (Pyrroles) RN - 0 (Pyrrolidines) RN - 21G72T1950 (Tolvaptan) RN - 11000-17-2 (Vasopressins) RN - 0NJ98Y462X (conivaptan) RN - 8F5X4B082E (lixivaptan) RN - 12794-10-4 (Benzodiazepines) RN - C1GL8G6G0O (relcovaptan) SB - IM MH - Animals MH - *Antidiuretic Hormone Receptor Antagonists MH - Azepines/chemistry/therapeutic use MH - Benzamides/chemistry/therapeutic use MH - Benzazepines/chemistry/therapeutic use MH - Binding Sites MH - Cardiovascular Diseases/drug therapy/metabolism MH - Clinical Trials as Topic MH - Hormone Antagonists/chemistry/*therapeutic use MH - Humans MH - Indoles/chemistry/therapeutic use MH - Pyrroles MH - Pyrrolidines/chemistry/therapeutic use MH - Tolvaptan MH - Vasopressins/*antagonists & inhibitors MH - Water-Electrolyte Imbalance/drug therapy/metabolism MH - Benzodiazepines PMC - PMC11136164 EDAT- 2006/06/24 09:00 MHDA- 2006/09/30 09:00 PMCR- 2006/06/23 CRDT- 2006/06/24 09:00 PHST- 2006/06/24 09:00 [pubmed] PHST- 2006/09/30 09:00 [medline] PHST- 2006/06/24 09:00 [entrez] PHST- 2006/06/23 00:00 [pmc-release] AID - 6054 [pii] AID - 10.1007/s00018-006-6054-2 [doi] PST - ppublish SO - Cell Mol Life Sci. 2006 Aug;63(15):1766-79. doi: 10.1007/s00018-006-6054-2. PMID- 16808991 OWN - NLM STAT- MEDLINE DCOM- 20070208 LR - 20220408 IS - 0266-7681 (Print) IS - 0266-7681 (Linking) VI - 31 IP - 5 DP - 2006 Oct TI - Cold intolerance of the hand measured by the CISS questionnaire in a normative study population. PG - 533-6 AB - Cold intolerance has been recognized as one of the most disabling sequelae of upper extremity trauma, especially when neurovascular structures are involved. In this study, we aimed to describe cold intolerance in a normative study population, validate the Cold Intolerance Symptom Severity (CISS) questionnaire and define the threshold for abnormal cold intolerance. One hundred and eight volunteers participated in our study. In addition to the CISS score, information about age, gender and previous surgery or trauma to the upper extremity was obtained. There were no volunteers with previous peripheral nerve injury and subjects with a history of Raynaud's disease, upper extremity injury or surgery were excluded (n=40). The CISS scores of the study population (n=68) averaged 12.9 (SD 8.2). Age and gender were not correlated with CISS score. The upper 95% confidence interval of the CISS scores for healthy subjects is about 30. We suggest this value as a threshold for pathological cold intolerance. FAU - Ruijs, A C J AU - Ruijs AC AD - Department of Plastic and Reconstructive Surgery, Erasmus Medical Center Rotterdam and Erasmus University Medical School, Rotterdam, The Netherlands. AleidRuijs@hotmail.com FAU - Jaquet, J-B AU - Jaquet JB FAU - Daanen, H A M AU - Daanen HA FAU - Hovius, S E R AU - Hovius SE LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060630 PL - Scotland TA - J Hand Surg Br JT - Journal of hand surgery (Edinburgh, Scotland) JID - 8403839 SB - IM MH - Adolescent MH - Adult MH - Cold Temperature/*adverse effects MH - Female MH - Hand/*physiopathology MH - Humans MH - Male MH - Middle Aged MH - Reference Values MH - Severity of Illness Index MH - *Surveys and Questionnaires MH - Thermosensing/*physiology EDAT- 2006/07/01 09:00 MHDA- 2007/02/09 09:00 CRDT- 2006/07/01 09:00 PHST- 2005/12/12 00:00 [received] PHST- 2006/04/12 00:00 [revised] PHST- 2006/04/18 00:00 [accepted] PHST- 2006/07/01 09:00 [pubmed] PHST- 2007/02/09 09:00 [medline] PHST- 2006/07/01 09:00 [entrez] AID - S0266-7681(06)00264-6 [pii] AID - 10.1016/j.jhsb.2006.04.013 [doi] PST - ppublish SO - J Hand Surg Br. 2006 Oct;31(5):533-6. doi: 10.1016/j.jhsb.2006.04.013. Epub 2006 Jun 30. PMID- 37669310 OWN - NLM STAT- MEDLINE DCOM- 20230907 LR - 20230908 IS - 2242-3982 (Electronic) IS - 1239-9736 (Print) IS - 1239-9736 (Linking) VI - 82 IP - 1 DP - 2023 Dec TI - Neurosensory and vascular symptoms and clinical findings in the hands of Arctic open-pit miners in Sweden and Norway - a descriptive study. PG - 2254916 LID - 10.1080/22423982.2023.2254916 [doi] LID - 2254916 AB - This cross-sectional study aimed to describe exposure to cold climate and hand-arm vibration (HAV) as well as neurosensory and vascular symptoms and clinical findings among open-pit Arctic miners. It was based on data from questionnaires and physical examinations, including 177 men and 75 women from two open-pit mines in Sweden and Norway (response rate 54%). Working outdoors or in an unheated building or machine for at least two hours per day was reported by 44% and HAV exposure of the same duration by 10%. Neurosensory symptoms (e.g. reduced perception of touch) in the hands were reported by 47% and Raynaud's phenomenon by 14%. In brief conclusion, the study showed that Arctic miners were commonly exposed to both cold temperatures and HAV. They also reported a broad range of neurosensory and vascular symptoms in their hands and had abnormal clinical findings related to the symptoms. The results emphasise the need for additional preventive measures in this occupational setting. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Johnsen, Magnar AU - Johnsen M AUID- ORCID: 0000-0002-2776-0750 AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Tromsø, Norway. FAU - Liljelind, Ingrid AU - Liljelind I AUID- ORCID: 0000-0002-5936-1172 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Aminoff, Anna AU - Aminoff A AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Tromsø, Norway. AD - Department of Community Medicine, UiT The Arctic University of Norway, Tromsø, Norway. FAU - Wahlström, Jens AU - Wahlström J AUID- ORCID: 0000-0002-2359-509X AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Höper, Anje Christina AU - Höper AC AUID- ORCID: 0000-0002-8962-5853 AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Tromsø, Norway. AD - Department of Community Medicine, UiT The Arctic University of Norway, Tromsø, Norway. FAU - Pettersson, Hans AU - Pettersson H AUID- ORCID: 0000-0001-7077-2389 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. FAU - Nilsson, Tohr AU - Nilsson T AUID- ORCID: 0000-0003-2789-6321 AD - Section of Sustainable Health, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Int J Circumpolar Health JT - International journal of circumpolar health JID - 9713056 SB - IM MH - Male MH - Female MH - Humans MH - Sweden MH - Cross-Sectional Studies MH - *Hand MH - Norway MH - *Cold Climate PMC - PMC10481761 OTO - NOTNLM OT - Cold climate OT - Norway OT - Raynaud disease OT - Sweden OT - mining OT - peripheral nervous system diseases OT - vibration COIS- No potential conflict of interest was reported by the author(s). EDAT- 2023/09/05 18:41 MHDA- 2023/09/07 06:42 PMCR- 2023/09/05 CRDT- 2023/09/05 13:43 PHST- 2023/09/07 06:42 [medline] PHST- 2023/09/05 18:41 [pubmed] PHST- 2023/09/05 13:43 [entrez] PHST- 2023/09/05 00:00 [pmc-release] AID - 2254916 [pii] AID - 10.1080/22423982.2023.2254916 [doi] PST - ppublish SO - Int J Circumpolar Health. 2023 Dec;82(1):2254916. doi: 10.1080/22423982.2023.2254916. PMID- 36897675 OWN - NLM STAT- MEDLINE DCOM- 20230314 LR - 20230916 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 102 IP - 10 DP - 2023 Mar 10 TI - Adrenaline auto-injector injuries: Practical considerations in emergency management in a tertiary hand surgery unit. PG - e32977 LID - 10.1097/MD.0000000000032977 [doi] LID - e32977 AB - Adrenaline auto-injectors are the first line treatment for anaphylaxis in the community setting. Both anaphylaxis and auto-injector carriage are increasing in prevalence. Adrenaline auto-injector injuries are common and most often involve the hand or digits. Such injuries carry a risk of ischemic necrosis due to profound vasoconstriction, especially if there is undying vascular pathology such as Raynaud's disease. The effects can be readily reversed with local infiltration of phentolamine. A survey was circulated to 40 clinicians working in the emergency and hand surgery departments of a major urban center. Knowledge of adrenaline duration of action and its reversal (agent, dose and location in the hospital) was assessed. All clinicians working within the two departments were eligible for participation. Only 25% of clinicians surveyed were aware of the duration of action of adrenaline. Half were aware of the correct reversal agent and only 20% knew the correct dose. Only one person was aware of phentolamine's location within the hospital. There is relatively poor clinician knowledge surrounding adrenaline reversal and a lack of easily accessible information available about dosing and drug location within the hospital. Given the time dependent nature of adrenaline auto-injector injuries Emergency Departments should consider stocking phentolamine in an emergency drugs fridge within the department along with a dosing guide. This is likely to greatly reduce time from presentation to treatment and thus the chances of digital ischemia progressing to necrosis. CI - Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - McCaughran, Pierre William AU - McCaughran PW AUID- ORCID: 0000-0002-2599-1301 AD - Royal Free Hospital Department of Plastic Surgery, London, United Kingdom. FAU - Ellis, Kate AU - Ellis K AD - University College London Medical School, London, United Kingdom. FAU - Southall, Clea AU - Southall C AD - Royal Free Hospital Department of Plastic Surgery, London, United Kingdom. FAU - Zargaran, David AU - Zargaran D AD - University College London, Royal Free Hospital Department of Plastic Surgery, London, United Kingdom. FAU - Nikkhah, Dariush AU - Nikkhah D AD - Royal Free Hospital Department of Plastic Surgery, London, United Kingdom. FAU - Mosahebi, Afshin AU - Mosahebi A AD - Royal Free Hospital Department of Plastic Surgery, London, United Kingdom. LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - YKH834O4BH (Epinephrine) RN - Z468598HBV (Phentolamine) SB - IM MH - Humans MH - *Epinephrine MH - *Anaphylaxis/drug therapy MH - Phentolamine/therapeutic use MH - Hand/surgery MH - Injections, Intramuscular MH - Necrosis PMC - PMC9997809 EDAT- 2023/03/11 06:00 MHDA- 2023/03/15 06:00 PMCR- 2023/03/10 CRDT- 2023/03/10 12:04 PHST- 2023/03/10 12:04 [entrez] PHST- 2023/03/11 06:00 [pubmed] PHST- 2023/03/15 06:00 [medline] PHST- 2023/03/10 00:00 [pmc-release] AID - 00005792-202303100-00010 [pii] AID - 10.1097/MD.0000000000032977 [doi] PST - ppublish SO - Medicine (Baltimore). 2023 Mar 10;102(10):e32977. doi: 10.1097/MD.0000000000032977. PMID- 27800198 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2090-6668 (Print) IS - 2090-6676 (Electronic) IS - 2090-6676 (Linking) VI - 2016 DP - 2016 TI - A Case Report of Neurosarcoidosis Presenting as a Lymphoma Mimic. PG - 7464587 LID - 7464587 AB - Objective. To describe a unique presentation of neurosarcoidosis. Background. Central nervous system involvement is rare in sarcoidosis. Sarcoidosis can be severe and can be mistaken for systemic lymphoma. Case Description. A 55-year-old right-handed white male with past medical history of obstructive sleep apnea, Raynaud's disease, and Hashimoto's thyroiditis was noted to have cognitive decline over a duration of few weeks and 20 lb weight loss. His neurologic exam (including cranial nerves) was normal except for five-minute recall. Head CT revealed a lacrimal gland mass, confirmed on brain MRI, which was suspicious for lymphoma on brain PET/MRI. Subsequent whole-body FDG PET/CT scan showed multiple enlarged lymph nodes. Bone marrow biopsy was negative. Serum and CSF ACE levels were within normal limits. Supraclavicular lymph node biopsy before steroids therapy was initiated and revealed multiple noncaseating granulomas, diagnostic of "sarcoidosis." He was treated with daily prednisone for two months, followed by weekly infliximab. Brain MRI two months after treatment with prednisone showed decrease in size of lacrimal lesion, and brain PET/MRI showed normal brain metabolism pattern after five months. Neurocognitive evaluation three months after diagnosis demonstrated improvements in memory abilities. Discussion. Both clinically and radiographically, neurosarcoidosis can mimic systemic lymphoma. Biopsy in these types of cases is necessary to establish the diagnosis. FAU - Kaur, Gurcharanjeet AU - Kaur G AUID- ORCID: 0000-0003-3552-1423 AD - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY, USA. FAU - Cameron, Lauren AU - Cameron L AD - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY, USA. FAU - Syritsyna, Olga AU - Syritsyna O AD - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY, USA. FAU - Coyle, Patricia AU - Coyle P AUID- ORCID: 0000-0002-3801-6177 AD - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY, USA. FAU - Kowalska, Agnes AU - Kowalska A AUID- ORCID: 0000-0003-0576-0423 AD - Department of Neurology, Stony Brook University Hospital, Stony Brook, NY, USA. LA - eng PT - Journal Article DEP - 20161012 PL - United States TA - Case Rep Neurol Med JT - Case reports in neurological medicine JID - 101576451 PMC - PMC5069375 EDAT- 2016/11/02 06:00 MHDA- 2016/11/02 06:01 PMCR- 2016/10/12 CRDT- 2016/11/02 06:00 PHST- 2016/04/10 00:00 [received] PHST- 2016/08/14 00:00 [accepted] PHST- 2016/11/02 06:00 [pubmed] PHST- 2016/11/02 06:01 [medline] PHST- 2016/11/02 06:00 [entrez] PHST- 2016/10/12 00:00 [pmc-release] AID - 10.1155/2016/7464587 [doi] PST - ppublish SO - Case Rep Neurol Med. 2016;2016:7464587. doi: 10.1155/2016/7464587. Epub 2016 Oct 12. PMID- 21885275 OWN - NLM STAT- MEDLINE DCOM- 20120416 LR - 20141120 IS - 1464-3405 (Electronic) IS - 0960-894X (Linking) VI - 21 IP - 19 DP - 2011 Oct 1 TI - Discovery of PF-184563, a potent and selective V1a antagonist for the treatment of dysmenorrhoea. The influence of compound flexibility on microsomal stability. PG - 5684-7 LID - 10.1016/j.bmcl.2011.08.038 [doi] AB - The V1a receptor has emerged as an attractive target for a range of indications including Raynaud's disease and dysmenorrhoea. As part of an effort to discover a new class of orally active V1a antagonist, we optimised a highly lipophilic, metabolically unstable lead into a range of potent, selective and metabolically stable V1a antagonists. In this communication, we demonstrate the series-dependent effect of limiting the number of rotatable bonds in order to decrease Cytochrome P450-mediated metabolism. This effort culminated in the discovery of PF-184563, a novel, selective V1a antagonist with excellent in vitro and in vivo properties. CI - Copyright © 2011 Elsevier Ltd. All rights reserved. FAU - Johnson, Patrick S AU - Johnson PS AD - Pfizer Worldwide Medicinal Chemistry, Ramsgate Road, Sandwich Kent CT139NJ, UK. FAU - Ryckmans, Thomas AU - Ryckmans T FAU - Bryans, Justin AU - Bryans J FAU - Beal, Dave M AU - Beal DM FAU - Dack, Kevin N AU - Dack KN FAU - Feeder, Neil AU - Feeder N FAU - Harrison, Anthony AU - Harrison A FAU - Lewis, Mark AU - Lewis M FAU - Mason, Helen J AU - Mason HJ FAU - Mills, James AU - Mills J FAU - Newman, Julie AU - Newman J FAU - Pasquinet, Christelle AU - Pasquinet C FAU - Rawson, Dave J AU - Rawson DJ FAU - Roberts, Lee R AU - Roberts LR FAU - Russell, Rachel AU - Russell R FAU - Spark, Deborah AU - Spark D FAU - Stobie, Alan AU - Stobie A FAU - Underwood, Toby J AU - Underwood TJ FAU - Ward, Robin AU - Ward R FAU - Wheeler, Simon AU - Wheeler S LA - eng PT - Journal Article DEP - 20110812 PL - England TA - Bioorg Med Chem Lett JT - Bioorganic & medicinal chemistry letters JID - 9107377 RN - 0 (Antidiuretic Hormone Receptor Antagonists) RN - 0 (Hormone Antagonists) RN - 0 (PF-184563) RN - 0 (Triazoles) RN - 12794-10-4 (Benzodiazepines) RN - 9035-51-2 (Cytochrome P-450 Enzyme System) SB - IM MH - *Antidiuretic Hormone Receptor Antagonists MH - Benzodiazepines/*chemical synthesis/chemistry/metabolism/*pharmacology MH - Cytochrome P-450 Enzyme System/*metabolism MH - *Drug Design MH - *Drug Discovery MH - Drug Stability MH - Dysmenorrhea/*drug therapy MH - Female MH - Hormone Antagonists/*chemical synthesis/chemistry/metabolism/*pharmacology MH - Humans MH - Microsomes/physiology MH - Molecular Structure MH - Triazoles/*chemical synthesis/chemistry/metabolism/*pharmacology EDAT- 2011/09/03 06:00 MHDA- 2012/04/17 06:00 CRDT- 2011/09/03 06:00 PHST- 2011/07/11 00:00 [received] PHST- 2011/08/06 00:00 [accepted] PHST- 2011/09/03 06:00 [entrez] PHST- 2011/09/03 06:00 [pubmed] PHST- 2012/04/17 06:00 [medline] AID - S0960-894X(11)01123-1 [pii] AID - 10.1016/j.bmcl.2011.08.038 [doi] PST - ppublish SO - Bioorg Med Chem Lett. 2011 Oct 1;21(19):5684-7. doi: 10.1016/j.bmcl.2011.08.038. Epub 2011 Aug 12. PMID- 27175977 OWN - NLM STAT- MEDLINE DCOM- 20170911 LR - 20260410 IS - 1827-1618 (Electronic) IS - 0026-4725 (Linking) VI - 64 IP - 6 DP - 2016 Dec TI - Thoracic sympathectomy for upper extremity ischemia. PG - 676-85 AB - INTRODUCTION: Thoracic sympathectomy is performed in the management of a variety of disorders of the upper extremity. To evaluate the contemporary results of thoracic sympathectomy for upper extremity ischemia a systematic review of the literature was conducted. EVIDENCE AQUISITION: We performed a PubMed, EMBASE and Cochrane search of the literature written in the English language from January 1975 to December 2015. All articles presenting original patient data regarding the effect of treatment on symptoms or on the healing of ulcers were eligible for inclusion. Individual analyses for Primary Raynaud's Disease (PRD) and Secondary Raynaud's Phenomenon (SRP) were performed. EVIDENCE SYNTHESIS: We included 6 prospective and 23 retrospective series with a total of 753 patients and 1026 affected limbs. Early beneficial effects of thoracic sympathectomy were noticed in 63-100% (median 94%) of all patients, in 73-100% (median 98%) of PRD patients and in 63-100% (median 94%) of SRP patients. The beneficial effect was noted to lessen over time. Long-term beneficial effects were reported in 13-100% (median 75%) of all patients, in 22-100% (median 58%) of PRD patients, and in 13-100% (median 79%) of SRD patients. Complete or improved ulcer healing was achieved in 33-100% and 25-67% respectively, of all patients. CONCLUSIONS: Thoracic sympathectomy can be beneficial in the treatment of upper extremity ischemia in select patients. Although the effect in patients with PRD will lessen over time, it may still reduce the severity of symptoms. In SRD, effects are more often long-lasting. In addition, thoracic sympathectomy may maximize tissue preservation or prevent amputation in cases of digital ulceration. FAU - Hoexum, Frank AU - Hoexum F AD - Department of Vascular Surgery, Vrije Universiteit Medical Center, Amsterdam, The Netherlands - frankhoexum@hotmail.com. FAU - Coveliers, Hans M AU - Coveliers HM FAU - Lu, Joyce J AU - Lu JJ FAU - Jongkind, Vincent AU - Jongkind V FAU - Yeung, Kakkhee K AU - Yeung KK FAU - Wisselink, Willem AU - Wisselink W LA - eng PT - Journal Article PT - Systematic Review DEP - 20160513 PL - Italy TA - Minerva Cardioangiol JT - Minerva cardioangiologica JID - 0400725 SB - IM MH - Humans MH - Ischemia/*surgery MH - Regional Blood Flow MH - Sympathectomy/*methods MH - Upper Extremity/blood supply/*surgery EDAT- 2016/05/14 06:00 MHDA- 2017/09/12 06:00 CRDT- 2016/05/14 06:00 PHST- 2016/05/14 06:00 [entrez] PHST- 2016/05/14 06:00 [pubmed] PHST- 2017/09/12 06:00 [medline] AID - R05Y9999N00A16051302 [pii] PST - ppublish SO - Minerva Cardioangiol. 2016 Dec;64(6):676-85. Epub 2016 May 13. PMID- 41763898 OWN - NLM STAT- Publisher LR - 20260418 IS - 1558-9455 (Electronic) IS - 1558-9447 (Print) IS - 1558-9447 (Linking) DP - 2026 Feb 28 TI - The Incidence of Painful Neuroma After Digital Amputation Due to Trauma Versus Chronic Ischemia. PG - 15589447261416977 LID - 10.1177/15589447261416977 [doi] LID - 15589447261416977 AB - BACKGROUND: Finger amputation is one of the most common surgical procedures conducted by hand surgeons. The purpose of the current study was to compare the incidence of painful neuromas requiring surgical interventions after finger amputation secondary to acute trauma versus chronic digital ischemia. METHODS: A retrospective chart review was conducted at a single tertiary academic medical center. Patients who underwent finger amputation due to acute trauma or chronic ischemia were identified using Current Procedural Terminology codes. The primary outcome was the incidence of neuroma formation. Patient demographics, treatment types, and postoperative complications, such as infection, residual pain, and phantom limb, were also collected. RESULTS: Between January 2013 and December 2023, there were 1150 patients who underwent finger or thumb amputations, 917 due to acute trauma and 233 associated with chronic ischemia. Men were more likely involved in traumatic amputations. Raynaud's disease, scleroderma, and end-stage renal disease were the common causes for ischemic finger amputation. No significant difference was found between the 2 groups in postamputation complications. Thirty-eight in the traumatic group (4.1%) and 4 in the chronic ischemia group (1.7%) developed painful neuroma requiring surgical intervention. However, the difference was not statistically significant (P = .09). CONCLUSIONS: The overall incidence of painful stump neuroma after digital amputation was low. While there was a stronger tendency in developing painful neuromas after finger amputation due to trauma compared with chronic ischemia, the difference was not statistically significant. FAU - Shanmugam, Sujeeth Krishna AU - Shanmugam SK AUID- ORCID: 0009-0009-4842-6207 AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. FAU - Martin, Sarah A AU - Martin SA AUID- ORCID: 0000-0002-7885-5055 AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. FAU - Mendoza, Andy AU - Mendoza A AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. FAU - Do, Andrew AU - Do A AUID- ORCID: 0000-0002-0241-1407 AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. FAU - Bullock, Garrett Scott AU - Bullock GS AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. FAU - Li, Zhongyu AU - Li Z AUID- ORCID: 0000-0002-1514-4651 AD - Wake Forest University School of Medicine, Winston-Salem, NC, USA. LA - eng PT - Journal Article DEP - 20260228 PL - United States TA - Hand (N Y) JT - Hand (New York, N.Y.) JID - 101264149 SB - IM PMC - PMC12950525 OTO - NOTNLM OT - finger amputation OT - ischemia OT - neuroma OT - trauma COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2026/03/01 00:35 MHDA- 2026/03/01 00:35 PMCR- 2027/02/28 CRDT- 2026/02/28 21:13 PHST- 2027/02/28 00:00 [pmc-release] PHST- 2026/03/01 00:35 [medline] PHST- 2026/03/01 00:35 [pubmed] PHST- 2026/02/28 21:13 [entrez] AID - 10.1177_15589447261416977 [pii] AID - 10.1177/15589447261416977 [doi] PST - aheadofprint SO - Hand (N Y). 2026 Feb 28:15589447261416977. doi: 10.1177/15589447261416977. PMID- 30758773 OWN - NLM STAT- MEDLINE DCOM- 20190904 LR - 20190904 IS - 0065-2598 (Print) IS - 0065-2598 (Linking) VI - 1153 DP - 2019 TI - Systemic Sclerosis and Serum Content of Transforming Growth Factor. PG - 63-67 LID - 10.1007/5584_2019_341 [doi] AB - Systemic sclerosis is a connective tissue disease characterized by tissue fibrosis leading to interstitial lung disease. Transforming growth factor-β (TGF-β) has been of interest as a potential diagnostic marker and also as a drug target in systemic sclerosis. The aim of this study was to assess the serum content of TGF-β1 in patients with systemic sclerosis and to assess its potential role in tissue fibrosis. The study included 30 patients, 5 men and 25 women, of the mean age of 46.9 ± 12.8 years, diagnosed with systemic sclerosis. The control group consisted of 19 women of the mean age of 28.4 ± 7.8 years, diagnosed with primary Raynaud's disease. TGF-β1 serum levels were measured, chest imaging examinations were performed, and fibrotic tissue changes were assessed using the modified Rodnan Skin Score. We found that the mean serum TGF-β1 content in patients with systemic sclerosis was 598.7 ± 242.6 pg/mL, whereas it was 568.4 ± 322.2 pg/mL in the control group (p = 0.378). We also failed to substantiate any significant relationship between TGF-β1 serum levels and the severity of pulmonary and skin fibrosis in systemic sclerosis. In conclusion, systemic sclerosis does not seem a disease that would be accompanied by a specific enhancement of serum TGF-β1. Thus, this cytokine is rather unlikely to play an essential role in the development and course of the disease, nor can it be considered diagnostic or prognostic marker. FAU - Majewski, Dominik AU - Majewski D AD - Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland. dmajes@poczta.onet.pl. FAU - Majewska, Katarzyna A AU - Majewska KA AD - Department of Clinical Auxology and Pediatric Nursing, Poznan University of Medical Sciences, Poznan, Poland. FAU - Kuznar-Kaminska, Barbara AU - Kuznar-Kaminska B AD - Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Runowska, Marta AU - Runowska M AD - Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland. FAU - Piorunek, Tomasz AU - Piorunek T AD - Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Batura-Gabryel, Halina AU - Batura-Gabryel H AD - Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Puszczewicz, Mariusz AU - Puszczewicz M AD - Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland. LA - eng PT - Journal Article PL - United States TA - Adv Exp Med Biol JT - Advances in experimental medicine and biology JID - 0121103 RN - 0 (Transforming Growth Factor beta) RN - 0 (Transforming Growth Factor beta1) RN - 76057-06-2 (Transforming Growth Factors) SB - IM MH - Adult MH - Female MH - Fibrosis MH - Humans MH - Male MH - Middle Aged MH - *Scleroderma, Systemic/blood MH - Skin/pathology MH - Transforming Growth Factor beta MH - Transforming Growth Factor beta1/blood MH - *Transforming Growth Factors/blood MH - Young Adult OTO - NOTNLM OT - Connective tissue disease OT - Cytokines OT - Fibroblasts OT - Pulmonary hypertension OT - Rheumatology OT - Scleroderma EDAT- 2019/02/14 06:00 MHDA- 2019/09/05 06:00 CRDT- 2019/02/14 06:00 PHST- 2019/02/14 06:00 [pubmed] PHST- 2019/09/05 06:00 [medline] PHST- 2019/02/14 06:00 [entrez] AID - 10.1007/5584_2019_341 [doi] PST - ppublish SO - Adv Exp Med Biol. 2019;1153:63-67. doi: 10.1007/5584_2019_341. PMID- 24401675 OWN - NLM STAT- MEDLINE DCOM- 20140929 LR - 20260128 IS - 1347-8648 (Electronic) IS - 1347-8613 (Linking) VI - 124 IP - 1 DP - 2014 TI - Therapeutic potential of vasopressin-receptor antagonists in heart failure. PG - 1-6 AB - Arginine vasopressin (AVP) is a 9-amino acid peptide that is secreted from the posterior pituitary in response to high plasma osmolality and hypotension. AVP has important roles in circulatory and water homoeostasis, which are mediated by oxytocin receptors and by AVP receptor subtypes: V(1a) (mainly vascular), V(1b) (pituitary), and V(2) (renal). Vaptans are orally and intravenously active nonpeptide vasopressin-receptor antagonists. Recently, subtype-selective nonpeptide vasopressin-receptor agonists have been developed. A selective V(1a)-receptor antagonist, relcovaptan, has shown initial positive results in the treatment of Raynaud's disease, dysmenorrhea, and tocolysis. A selective V(1b)-receptor antagonist, nelivaptan, has beneficial effects in the treatment of psychiatric disorders. Selective V2-receptor antagonists including mozavaptan, lixivaptan, satavaptan, and tolvaptan induce highly hypotonic diuresis without substantially affecting the excretion of electrolytes. A nonselective V(1a)/V(2)-receptor antagonist, conivaptan, is used in the treatment for euvolaemic or hypervolemic hyponatremia. Recent basic and clinical studies have shown that AVP-receptor antagonists, especially V2-receptor antagonists, may have therapeutic potential for heart failure. This review presents current information about AVP and its antagonists. FAU - Izumi, Yasukatsu AU - Izumi Y AD - Department of Pharmacology, Osaka City University Medical School, Japan. FAU - Miura, Katsuyuki AU - Miura K FAU - Iwao, Hiroshi AU - Iwao H LA - eng PT - Journal Article PT - Review DEP - 20140107 PL - Japan TA - J Pharmacol Sci JT - Journal of pharmacological sciences JID - 101167001 RN - 0 (Antidiuretic Hormone Receptor Antagonists) RN - 113-79-1 (Arginine Vasopressin) RN - 0 (Benzamides) RN - 0 (Benzazepines) RN - 0 (Morpholines) RN - 0 (Pyrroles) RN - 0 (Receptors, Vasopressin) RN - 0 (Spiro Compounds) RN - 21G72T1950 (Tolvaptan) RN - 0NJ98Y462X (conivaptan) RN - 17OJ42922Y (mozavaptan) RN - 8F5X4B082E (lixivaptan) RN - 12794-10-4 (Benzodiazepines) RN - AJS8S3P31H (satavaptan) SB - IM MH - Animals MH - *Antidiuretic Hormone Receptor Antagonists MH - Arginine Vasopressin/*physiology MH - Benzamides/*pharmacology/*therapeutic use MH - Benzazepines/*pharmacology/*therapeutic use MH - Body Water/metabolism MH - Clinical Trials as Topic MH - Disease Models, Animal MH - Heart Failure/*drug therapy MH - Homeostasis/drug effects MH - Humans MH - Hyponatremia/drug therapy MH - Morpholines/pharmacology/therapeutic use MH - Pyrroles/*pharmacology/*therapeutic use MH - Receptors, Vasopressin/physiology MH - Spiro Compounds/pharmacology/therapeutic use MH - Tolvaptan MH - Ventricular Remodeling/drug effects MH - Benzodiazepines EDAT- 2014/01/10 06:00 MHDA- 2014/09/30 06:00 CRDT- 2014/01/10 06:00 PHST- 2014/01/10 06:00 [entrez] PHST- 2014/01/10 06:00 [pubmed] PHST- 2014/09/30 06:00 [medline] AID - DN/JST.JSTAGE/jphs/13R13CP [pii] AID - 10.1254/jphs.13r13cp [doi] PST - ppublish SO - J Pharmacol Sci. 2014;124(1):1-6. doi: 10.1254/jphs.13r13cp. Epub 2014 Jan 7. PMID- 36588566 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230103 IS - 2297-055X (Print) IS - 2297-055X (Electronic) IS - 2297-055X (Linking) VI - 9 DP - 2022 TI - Premature stroke and cardiovascular risk in primary Sjögren's syndrome. PG - 1048684 LID - 10.3389/fcvm.2022.1048684 [doi] LID - 1048684 AB - INTRODUCTION: Primary Sjögren's syndrome (pSS) is associated with an increased prevalence of traditional risk factors and cardiovascular diseases (CVDs). The study aimed to identify specific risk factors for CVD in pSS patients. METHODS: PSS patients with and without CVD were compared. All patients fulfilled the EULAR/ACR classification criteria. Patients with CVD presented at least one of the following manifestations: myocardial infarction, transient ischemic attacks, ischemic or hemorrhagic stroke, peripheral artery disease, coronary artery disease, and carotid plaques. Data were collected by a standardized protocol and review of medical records. RESULTS: 61/312 (19.6%) pSS patients presented with CVD. Traditional risk factors such as hypertension, hypercholesterinemia and diabetes (p < 0.05), pSS manifestations, in particular vasculitis (p = 0.033) and Raynaud's phenomenon (p = 0.018) were associated with CVD. Among patients with ischemic events (28/312, 9%), particularly cerebrovascular disease (n = 12/28, 42.9%), correlations with increased EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) (p = 0.039) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) (p = 0.048) were observed. Age at first cerebrovascular event was 55.2 [48.9-69.6] years. Multivariate analysis confirmed hypertension [odds ratio (OR) 3.7, 95% confidence interval (CI) 1.87-7.18, p < 0.001], hypercholesterinemia (OR 3.1, 95% CI 1.63-5.72, p < 0.001), male gender (OR 0.4, 95% CI 0.17-0.78, p = 0.009), Raynaud's phenomenon (OR 2.5, 95% CI 1.28-4.82, p = 0.007), and CNS involvement (OR 2.7, 95% CI 1.00-7.15, p = 0.048) as independent CVD predictors. CONCLUSION: Raynaud's phenomen as well as vasculitis and high ESSDAI have shown a significant association to CVD. PSS patients with cerebrovascular events were younger than expected. Knowledge about risk factors may help clinicians to identify pSS patients at risk for CVD. After diagnosis of pSS, patients should be screened for risk factors such as hypertension and receive appropriate therapy to prevent or at least reduce sequelae such as infarction. However, further investigations are necessary in order to achieve a reliable risk stratification for these patients. CI - Copyright © 2022 Zippel, Beider, Kramer, Konen, Seeliger, Skripuletz, Hirsch, Jablonka, Witte, Sonnenschein and Ernst. FAU - Zippel, Clara L AU - Zippel CL AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Beider, Sonja AU - Beider S AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Kramer, Emelie AU - Kramer E AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Konen, Franz F AU - Konen FF AD - Department of Neurology, Hannover Medical School, Hannover, Germany. FAU - Seeliger, Tabea AU - Seeliger T AD - Department of Neurology, Hannover Medical School, Hannover, Germany. FAU - Skripuletz, Thomas AU - Skripuletz T AD - Department of Neurology, Hannover Medical School, Hannover, Germany. FAU - Hirsch, Stefanie AU - Hirsch S AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Jablonka, Alexandra AU - Jablonka A AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Witte, Torsten AU - Witte T AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. FAU - Sonnenschein, Kristina AU - Sonnenschein K AD - Department of Cardiology, Hannover Medical School, Hannover, Germany. FAU - Ernst, Diana AU - Ernst D AD - Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany. LA - eng PT - Journal Article DEP - 20221214 PL - Switzerland TA - Front Cardiovasc Med JT - Frontiers in cardiovascular medicine JID - 101653388 PMC - PMC9794609 OTO - NOTNLM OT - EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) OT - Raynaud disease OT - Sjögren syndrome OT - atherosclerosis OT - cardiovasclar disease OT - cardiovascular risk factors OT - stroke OT - vasculitis COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/01/03 06:00 MHDA- 2023/01/03 06:01 PMCR- 2022/01/01 CRDT- 2023/01/02 03:35 PHST- 2022/09/19 00:00 [received] PHST- 2022/11/28 00:00 [accepted] PHST- 2023/01/02 03:35 [entrez] PHST- 2023/01/03 06:00 [pubmed] PHST- 2023/01/03 06:01 [medline] PHST- 2022/01/01 00:00 [pmc-release] AID - 10.3389/fcvm.2022.1048684 [doi] PST - epublish SO - Front Cardiovasc Med. 2022 Dec 14;9:1048684. doi: 10.3389/fcvm.2022.1048684. eCollection 2022. PMID- 24746866 OWN - NLM STAT- MEDLINE DCOM- 20150204 LR - 20220410 IS - 1769-6666 (Electronic) IS - 1297-3203 (Linking) VI - 33 IP - 3 DP - 2014 Jun TI - The hand in systemic diseases other than rheumatoid arthritis. PG - 155-73 LID - S1297-3203(14)00039-0 [pii] LID - 10.1016/j.main.2014.01.008 [doi] AB - This review outlines the skin, vascular and musculoskeletal symptoms affecting the hand during systemic inflammatory diseases other than rheumatoid arthritis. Skin lesions are diagnosed clinically and their symptomatology is documented through an extensive series of photographs. These conditions may require specific care before a surgical procedure can be performed. Vascular lesions are also diagnosed clinically and their symptomatology is described in detail. It is important to recognize that acrocyanosis is always benign. The surgeon should be able to distinguish between primary, but benign Raynaud's disease and secondary Raynaud's syndrome, which has a high risk of finger necrosis. Current preventative and curative treatments for finger necrosis are described. The clinical, radiological, progressive and therapeutic features of musculoskeletal lesions are reviewed, namely those associated with psoriatic arthritis, systemic sclerosis and lupus. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Fontaine, C AU - Fontaine C AD - Service de chirurgie de la main et du membre supérieur, pôle des neurosciences et de l'appareil locomoteur, hôpital Roger-Salengro, CHRU de Lille, rue du Pr-Emile-Laine, 59037 Lille cedex, France. Electronic address: christian.fontaine@chru-lille.fr. FAU - Staumont-Sallé, D AU - Staumont-Sallé D AD - Service de dermatologie, hôpital Huriez, CHRU de Lille, 59037 Lille cedex, France. FAU - Hatron, P-Y AU - Hatron PY AD - Centre national de référence maladies rares : maladies auto-immunes systémiques, service de médecine interne, hôpital Huriez, CHRU de Lille, 59037 Lille cedex, France. FAU - Cotten, A AU - Cotten A AD - Service de radiologie et imagerie musculosquelettique, CCIAL, CHRU de Lille, 59037 Lille cedex, France. FAU - Couturier, C AU - Couturier C AD - SOS Main, hôpital privé Ouest Parisien, 14, avenue Castiglione-del-Lago, 78190 Trappes, France; Espace médical Vauban, 2A, avenue de Ségur, 75007 Paris, France. LA - eng PT - Journal Article PT - Review DEP - 20140324 PL - France TA - Chir Main JT - Chirurgie de la main JID - 100937750 SB - IM MH - Arthritis, Psoriatic/classification/complications/diagnosis MH - Cooperative Behavior MH - Cyanosis/diagnosis MH - Dermatomyositis/complications MH - Diagnosis, Differential MH - Diagnostic Imaging MH - Fingers/*pathology MH - Hand/surgery MH - Hand Dermatoses/*etiology/therapy MH - Humans MH - Interprofessional Relations MH - Lupus Erythematosus, Cutaneous/complications MH - Necrosis/etiology MH - Osteoarthritis/diagnosis MH - Osteonecrosis/diagnosis MH - Psoriasis/complications MH - Sarcoidosis/complications MH - Scleroderma, Diffuse/complications MH - Scleroderma, Systemic/complications MH - Skin Diseases, Infectious/prevention & control MH - Ulcer/etiology MH - Vascular Diseases/diagnosis/etiology/therapy MH - Wound Healing MH - Wrist Joint/surgery OTO - NOTNLM OT - Arthrite OT - Arthritis OT - Chirurgie OT - Dermatomyosite OT - Dermatomyositis OT - Hand OT - Ischemia OT - Ischémie OT - Lupus OT - Main OT - Necrosis OT - Nécrose OT - Poignet OT - Polymyosite OT - Polymyositis OT - Psoriasis OT - Raynaud OT - Scleroderma OT - Sclérodermie OT - Surgery OT - Wrist EDAT- 2014/04/22 06:00 MHDA- 2015/02/05 06:00 CRDT- 2014/04/22 06:00 PHST- 2013/08/19 00:00 [received] PHST- 2013/12/24 00:00 [revised] PHST- 2014/01/13 00:00 [accepted] PHST- 2014/04/22 06:00 [entrez] PHST- 2014/04/22 06:00 [pubmed] PHST- 2015/02/05 06:00 [medline] AID - S1297-3203(14)00039-0 [pii] AID - 10.1016/j.main.2014.01.008 [doi] PST - ppublish SO - Chir Main. 2014 Jun;33(3):155-73. doi: 10.1016/j.main.2014.01.008. Epub 2014 Mar 24. PMID- 16899912 OWN - NLM STAT- MEDLINE DCOM- 20061031 LR - 20071115 IS - 1386-0291 (Print) IS - 1386-0291 (Linking) VI - 35 IP - 1-2 DP - 2006 TI - Gender differences in hemorheological parameters of coronary artery disease patients. PG - 99-103 AB - Plasma fibrinogen concentration, plasma and whole blood viscosity (WBV) are independent risk factors of coronary artery disease (CAD). Fibrinogen seems to be a relatively stronger risk factor for women than for men, but men are more endangered by higher hematocrit (Hct) and WBV than women are. We have previously reported that a theoretically optimal Hct value can be determined using Hct/WBV ratio in healthy subjects, hyperlipidemic and Raynaud's disease patients. Our aim was to examine whether Hct/WBV ratio is differently correlated with Hct in men and women with proven CAD. In a retrospective study we analysed the hemorheological data of 162 CAD outpatients (107 men and 55 women). Coronary angiography, echocardiography and impedance cardiography were performed. Hemorheological parameters (Hct, fibrinogen level, plasma viscosity, WBV), blood picture, serum lipid concentrations were determined and Hct/WBV ratio was calculated. Mean ages of male and female patients were similar (54.9 and 55.4 years, respectively), but men had significantly higher coronary angiography score than women. Mean left ventricular ejection fraction, stroke volume index and cardiac index showed no significant differences in men and women. Similarly, lipid concentrations, fibrinogen levels and plasma viscosities demonstrated no statistical differences. However, Hct, WBV and Hct/WBV ratios were significantly higher in male than in female patients (p < 0.00001; p < 0.00001 and p < 0.005, respectively). The most striking gender difference was found in the correlation between Hct/WBV ratio and cardiac index. Men older than 56 years showed negative, women positive correlation (r = -0.485, p = 0.01; r = 0.468, p = 0.006, respectively). This study demonstrates that Hct/WBV ratio as a rheological oxygen carrying capacity parameter is positively correlated with the cardiac index as it can be expected. However, the correlation is negative in elder men indicating an unhealthy relation between hemodynamic and hemorheologic parameters. FAU - Bogar, L AU - Bogar L AD - Department of Anesthesiology and Intensive Care, University of Pécs, Hungary. lajos.bogar@aok.pte.hu FAU - Juricskay, I AU - Juricskay I FAU - Kesmarky, G AU - Kesmarky G FAU - Feher, G AU - Feher G FAU - Kenyeres, P AU - Kenyeres P FAU - Toth, K AU - Toth K LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 9001-32-5 (Fibrinogen) SB - IM MH - Aged MH - Blood Viscosity/*physiology MH - Cohort Studies MH - Coronary Artery Disease/*blood MH - Female MH - Fibrinogen/*analysis MH - *Hematocrit MH - *Hemorheology MH - Humans MH - Linear Models MH - Male MH - Middle Aged MH - Retrospective Studies MH - Sex Factors MH - Stroke Volume/*physiology EDAT- 2006/08/11 09:00 MHDA- 2006/11/01 09:00 CRDT- 2006/08/11 09:00 PHST- 2006/08/11 09:00 [pubmed] PHST- 2006/11/01 09:00 [medline] PHST- 2006/08/11 09:00 [entrez] PST - ppublish SO - Clin Hemorheol Microcirc. 2006;35(1-2):99-103. PMID- 23025425 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121022 LR - 20211021 IS - 1868-7083 (Electronic) IS - 1868-7075 (Print) IS - 1868-7075 (Linking) VI - 4 IP - 1 DP - 2012 Oct 1 TI - Sirtuin1 single nucleotide polymorphism (A2191G) is a diagnostic marker for vibration-induced white finger disease. PG - 18 LID - 10.1186/1868-7083-4-18 [doi] AB - BACKGROUND: Vibration-induced white finger disease (VWF), also known as hand-arm vibration syndrome, is a secondary form of Raynaud's disease, affecting the blood vessels and nerves. So far, little is known about the pathogenesisof the disease. VWF is associated with an episodic reduction in peripheral blood flow. Sirtuin 1, a class III histone deacetylase, has been described to regulate the endothelium dependent vasodilation by targeting endothelial nitric oxide synthase. We assessed Sirt1single nucleotide polymorphisms in patients with VWF to further elucidate the role of sirtuin 1 in the pathogenesis of VWF. METHODS: Peripheral blood samples were obtained from 74 patients with VWF (male 93.2%, female 6.8%, median age 53 years) and from 317 healthy volunteers (gender equally distributed, below 30 years of age). Genomic DNA was extracted from peripheral blood mononuclear cells and screened for potential Sirt1single nucleotide polymorphisms. Four putative genetic polymorphisms out of 113 within the Sirt1 genomic region (NCBI Gene Reference: NM_012238.3) were assessed. Allelic discrimination was performed by TaqMan-polymerasechainreaction-based allele-specific genotyping single nucleotide polymorphism assays. RESULTS: Sirt1single nucleotide polymorphism A2191G (Assay C_25611590_10, rs35224060) was identified within Sirt1 exon 9 (amino acid position 731, Ile → Val), with differing allelic frequencies in the VWF population (A/A: 70.5%, A/G: 29.5%, G/G: 0%) and the control population (A/A: 99.7%, A/G: 0.3%, G/G: 0.5%), with significance levels of P < 0.001 (Mann-Whitney U test (two-tailed) P <0.001; F-exact t-test and Chi-square test with Yates correction (all two-tailed): P <0.0001). The heterogeneous A/G genotype in base pair position 2191 is significantly overrepresented in the VWF patient population when compared with healthy controls. CONCLUSION: We identified theSirt1A2191Gsingle nucleotide polymorphism as a diagnostic marker for VWF. FAU - Voelter-Mahlknecht, Susanne AU - Voelter-Mahlknecht S AD - Institute of Occupational, Social and Environmental Health, University of Mainz, Obere Zahlbacher Strasse 67, D-55131 Mainz, Germany. susanne.voelter-mahlknecht@med.uni-tuebingen.de. FAU - Rossbach, Bernd AU - Rossbach B FAU - Schleithoff, Christina AU - Schleithoff C FAU - Dransfeld, Christian L AU - Dransfeld CL FAU - Letzel, Stephan AU - Letzel S FAU - Mahlknecht, Ulrich AU - Mahlknecht U LA - eng PT - Journal Article DEP - 20121001 PL - Germany TA - Clin Epigenetics JT - Clinical epigenetics JID - 101516977 PMC - PMC3475079 EDAT- 2012/10/03 06:00 MHDA- 2012/10/03 06:01 PMCR- 2012/10/01 CRDT- 2012/10/03 06:00 PHST- 2012/04/25 00:00 [received] PHST- 2012/07/25 00:00 [accepted] PHST- 2012/10/03 06:00 [entrez] PHST- 2012/10/03 06:00 [pubmed] PHST- 2012/10/03 06:01 [medline] PHST- 2012/10/01 00:00 [pmc-release] AID - 1868-7083-4-18 [pii] AID - 10.1186/1868-7083-4-18 [doi] PST - epublish SO - Clin Epigenetics. 2012 Oct 1;4(1):18. doi: 10.1186/1868-7083-4-18. PMID- 38251786 OWN - NLM STAT- MEDLINE DCOM- 20240614 LR - 20240614 IS - 1533-2500 (Electronic) IS - 1530-7085 (Linking) VI - 24 IP - 5 DP - 2024 Jun TI - Novel ultrasound-guided supraclavicular stellate ganglion block. PG - 808-814 LID - 10.1111/papr.13350 [doi] AB - INTRODUCTION: Stellate ganglion block (SGB) provides diagnostic and therapeutic benefits in pain syndromes in the head, neck, and upper extremity, including complex regional pain syndrome Types I and II, Raynaud's disease, hyperhidrosis, arterial embolism in the region of the arm. METHODS: We present a novel ultrasound-guided supraclavicular stellate ganglion block. Considering the existing anatomical structures of the targeted area. RESULTS AND CONCLUSIONS: We hope that we can provide fewer complications and additional benefits with this new approach. CI - © 2024 World Institute of Pain. FAU - Fajardo Pérez, Mario AU - Fajardo Pérez M AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. FAU - Yamak-Altinpulluk, Ece AU - Yamak-Altinpulluk E AUID- ORCID: 0000-0002-6993-0762 AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Anesthesiology Clinical Research Office, Ataturk University, Erzurum, Turkey. AD - Outcomes Research Consortium, Cleveland, Ohio, USA. FAU - Díez Tafur, Rodrigo AU - Díez Tafur R AUID- ORCID: 0000-0002-0681-8941 AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Centro MDRS - Sports, Spine & Pain Center: Lima Pain Institute, Lima, Peru. AD - Clínica Angloamericana British American Hospital, Lima, Peru. AD - Latin American Pain Society (LAPS), New York, New York, USA. FAU - Salazar-Zamorano, Carlos H AU - Salazar-Zamorano CH AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Department of Anesthesia, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Espinosa Morales, Karla AU - Espinosa Morales K AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Department of Anesthesia and Pain Medicine, Hospital de Trauma, Centro Integral de Salud de Puriscal, San José, Costa Rica. FAU - Oliver-Fornies, Pablo AU - Oliver-Fornies P AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Department of Anesthesiology, Critical Care and Pain Medicine, Móstoles University Hospital, Móstoles, Spain. AD - Aragon Institute for Health Research, Zaragoza, Spain. FAU - Rocha-Romero, Andrés AU - Rocha-Romero A AUID- ORCID: 0000-0001-9647-1752 AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Department of Anesthesia and Pain Medicine, Hospital de Trauma, Centro Integral de Salud de Puriscal, San José, Costa Rica. AD - Department of Anesthesia and Pain Management, Centro Nacional de Rehabilitacion, Hospital de Trauma, San José, Costa Rica. FAU - Aguilar Ureña, Ricardo AU - Aguilar Ureña R AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Department of Anesthesiology, Critical Care and Pain Medicine, Centro Nacional de Rehabilitacion, San José, Costa Rica. FAU - Juarez-Lemus, Angel AU - Juarez-Lemus A AD - Department of Pain Medicine, National Cancer Institute, Mexico City, Mexico. FAU - Galluccio, Felice AU - Galluccio F AUID- ORCID: 0000-0001-7485-471X AD - Morphological Madrid Research Center (MoMaRC), Ultradissection Spain EchoTraining School, Madrid, Spain. AD - Fisiotech Lab Studio, Rheumatology and Pain Management, Firenze, Italy. AD - Center for Regional Anesthesia and Pain Medicine (CRAPM), Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan. FAU - Abd-Elsayed, Alaa AU - Abd-Elsayed A AD - Anesthesiology Department, University of Wisconsin, Madison, Wisconsin, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240122 PL - United States TA - Pain Pract JT - Pain practice : the official journal of World Institute of Pain JID - 101130835 RN - 0 (Anesthetics, Local) SB - IM MH - Humans MH - *Stellate Ganglion/diagnostic imaging MH - *Autonomic Nerve Block/methods MH - *Ultrasonography, Interventional/methods MH - Anesthetics, Local/administration & dosage OTO - NOTNLM OT - sympathetic chain OT - sympathetic ganglia EDAT- 2024/01/22 12:42 MHDA- 2024/06/14 06:42 CRDT- 2024/01/22 08:18 PHST- 2024/06/14 06:42 [medline] PHST- 2024/01/22 12:42 [pubmed] PHST- 2024/01/22 08:18 [entrez] AID - 10.1111/papr.13350 [doi] PST - ppublish SO - Pain Pract. 2024 Jun;24(5):808-814. doi: 10.1111/papr.13350. Epub 2024 Jan 22. PMID- 18854969 OWN - NLM STAT- MEDLINE DCOM- 20090420 LR - 20181113 IS - 0340-2592 (Print) IS - 0340-2592 (Linking) VI - 47 IP - 12 DP - 2008 Dec TI - [The basics of phosphodiesterase type 5 (PDE5) inhibition in urology]. PG - 1582-7 LID - 10.1007/s00120-008-1797-z [doi] AB - Phosphodiesterase (PDE) isoenzymes hold a central role in controlling levels of the cyclic nucleotide monophosphates cyclic AMP and cyclic GMP, which are important second messengers in many transmitter pathways involved in regulating biological processes in urogenital tissues. The development of the PDE5 inhibitors Sildenafil (Viagra), Vardenafil (Levitra), and Tadalafil (Cialis), combining a high response rate and the advantage of on-demand intake, is the result of the scientific characterization of the physiology of penile erectile smooth muscle.The introduction of these compounds as safe and well-tolerated orally active drugs for the treatment of erectile dysfunction has not only become a worldwide clinical success, but it provided the basis for the development and introduction of several new therapeutic modalities into the management of male and female sexual dysfunction. It has also brought further attention to cyclic nucleotide phosphodiesterases as putative pharmacological targets in a variety of disorders, such as pulmonary arterial hypertension, Raynaud's disease, Peyronie's disease, the so-called benign prostatic syndrome, endothelial dysfunction, disturbances of male ejaculatory function (premature ejaculation), and female sexual dysfunction.Because the concept of taking a pill to cure an illness or relieve disease symptoms has become widely accepted by consumers, pharmacological research and development is focusing primarily on selective orally available drugs that influence peripheral intracellular or central regulatory mechanisms. This review briefly describes the current and evolving advances in the field of PDE5 pharmacotherapy in urology and other fields of medicine. FAU - Becker, A J AU - Becker AJ AD - Urologische Klinik & Poliklinik, Klinikum Grosshadern, Medizinische Fakultät, Ludwig-Maximilians-Universität (LMU) München, Marchioninistrasse 15, 81377 München. armin.becker@med.uni-muenchen.de FAU - Uckert, S AU - Uckert S FAU - Stief, C G AU - Stief CG LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Grundlagen der Phosphodiesterase-5- (PDE5-)Inhibitoren. PL - Germany TA - Urologe A JT - Der Urologe. Ausg. A JID - 1304110 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) SB - IM MH - Administration, Oral MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Erectile Dysfunction/blood/*drug therapy MH - Female MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Male MH - *Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/adverse effects/pharmacokinetics/*therapeutic use MH - Randomized Controlled Trials as Topic MH - Sexual Dysfunction, Physiological/*drug therapy RF - 34 EDAT- 2008/10/16 09:00 MHDA- 2009/04/21 09:00 CRDT- 2008/10/16 09:00 PHST- 2008/10/16 09:00 [pubmed] PHST- 2009/04/21 09:00 [medline] PHST- 2008/10/16 09:00 [entrez] AID - 10.1007/s00120-008-1797-z [doi] PST - ppublish SO - Urologe A. 2008 Dec;47(12):1582-7. doi: 10.1007/s00120-008-1797-z. PMID- 31341499 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211231 IS - 1741-427X (Print) IS - 1741-4288 (Electronic) IS - 1741-427X (Linking) VI - 2019 DP - 2019 TI - Acupuncture Point "Hegu" (LI4) Is Close to the Vascular Branch from the Superficial Branch of the Radial Nerve. PG - 6879076 LID - 10.1155/2019/6879076 [doi] LID - 6879076 AB - The acupuncture point "Hegu" (LI4) has been used for treating peripheral circulatory failure, which is located in the area covered by the superficial branch of the radial nerve (SBRN). SBRN has branches reaching arteries, so-called vascular branches (VBs), which are thought to be involved in the arterial constriction. The distribution areas of the VBs from the SBRN have been reported, but the positional relationship between these distribution areas and the acupuncture points are not known. To examine the positional relationship between LI4 and VBs from the SBRN, forty hands were examined to assess the positional relationship between the acupuncture points "Erjian" (LI2), "Sanjian" (LI3), LI4, and "Yangxi" (LI5) in the Yangming Large Intestine Meridian of Hand, which are located in the area covered by SBRN, and the VBs from the SBRN. After the VBs were identified, the distances from the acupuncture points (LI2, LI3, LI4, and LI5) to the point where the VBs reached the radial artery or the first dorsal metacarpal artery were measured. VBs reaching the radial arteries were observed in all specimens. The mean distances from LI2, LI3, LI4, and LI5 to the point where the VBs reached the radial artery were 64.2 ± 8.2 mm, 42.0 ± 7.5 mm, 4.3 ± 4.3 mm, and 33.0 ± 4.8 mm, respectively. LI4 was significantly closer than the other acupuncture points (P<0.01). The nerve fibers of the VBs adjacent to the radial artery were confirmed using hematoxylin and eosin staining. Our findings provide anatomical evidence that stimulation at LI4 is used for treating peripheral circulatory failure such as Raynaud's disease. LI4 is significant because it is located at a source point, making it clinically important. FAU - Umemoto, Kanae AU - Umemoto K AUID- ORCID: 0000-0001-5855-5496 AD - Department of Anatomy, Division of Basic Medical Science, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, Japan. FAU - Naito, Munekazu AU - Naito M AD - Department of Anatomy, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, Japan. FAU - Tano, Kaori AU - Tano K AD - Department of Acupuncture and Moxibustion, Faculty of Health Science, Suzuka University of Medical Science, 1001-1 Kishioka, Suzuka, Mie, Japan. FAU - Terayama, Hayato AU - Terayama H AUID- ORCID: 0000-0002-7053-8418 AD - Department of Anatomy, Division of Basic Medical Science, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, Japan. FAU - Koike, Taro AU - Koike T AD - Department of Anatomy, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, Japan. FAU - Ohmichi, Mika AU - Ohmichi M AD - Department of Anatomy, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, Japan. FAU - Ohmichi, Yusuke AU - Ohmichi Y AUID- ORCID: 0000-0002-9503-8022 AD - Department of Anatomy, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, Japan. FAU - Sakabe, Kou AU - Sakabe K AD - Department of Anatomy, Division of Basic Medical Science, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, Japan. FAU - Nakano, Takashi AU - Nakano T AD - Department of Anatomy, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi, Japan. LA - eng PT - Journal Article DEP - 20190625 PL - United States TA - Evid Based Complement Alternat Med JT - Evidence-based complementary and alternative medicine : eCAM JID - 101215021 CIN - Evid Based Complement Alternat Med. 2021 Dec 21;2021:9857079. doi: 10.1155/2021/9857079. PMID: 34970328 PMC - PMC6614981 EDAT- 2019/07/26 06:00 MHDA- 2019/07/26 06:01 PMCR- 2019/06/25 CRDT- 2019/07/26 06:00 PHST- 2019/04/24 00:00 [received] PHST- 2019/06/16 00:00 [accepted] PHST- 2019/07/26 06:00 [entrez] PHST- 2019/07/26 06:00 [pubmed] PHST- 2019/07/26 06:01 [medline] PHST- 2019/06/25 00:00 [pmc-release] AID - 10.1155/2019/6879076 [doi] PST - epublish SO - Evid Based Complement Alternat Med. 2019 Jun 25;2019:6879076. doi: 10.1155/2019/6879076. eCollection 2019. PMID- 40236261 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250417 IS - 2666-3643 (Electronic) IS - 2666-3643 (Linking) VI - 6 IP - 5 DP - 2025 May TI - Durvalumab Combined With Pemetrexed-Based Chemotherapy in Trial-Ineligible Patients With Mesothelioma: A Brief Report. PG - 100775 LID - 10.1016/j.jtocrr.2024.100775 [doi] LID - 100775 AB - INTRODUCTION: Chemoimmunotherapy is associated with promising activity in mesothelioma in phase II to III trials. Studies exploring this approach in patients ineligible for clinical trials are lacking. We assembled a cohort of patients receiving pemetrexed-based chemotherapy with durvalumab outside of clinical trials. METHODS: Patients with pleural mesothelioma received pemetrexed plus durvalumab or carboplatin plus pemetrexed plus durvalumab via off-label authorization at Massachusetts General Hospital. Response to chemoimmunotherapy was assessed per modified Response Evaluation Criteria in Solid Tumors version 1.1. A retrospective chart review was conducted to assess safety per Common Terminology Criteria for Adverse Events version 5.0. RESULTS: Twelve patients were included in the series. Nine patients were treated with triplet chemoimmunotherapy. Three patients received doublet chemoimmunotherapy because of platinum ineligibility. Concurrent active malignancies and symptomatic cardiac disease were present in three patients (25%) and two patients (17%), respectively. Ten patients had measurable disease at baseline. With the triplet regimen, partial responses were observed in four of the seven (57%) patients with measurable disease. All three patients receiving pemetrexed plus durvalumab had measurable disease and experienced a partial response. Primary progression was not observed with either regimen. Overall, eight patients (75%) remained on treatment for more than 6 months without progression. Five patients developed immune-related adverse events (n = 1 each pyrexia, arthritis, neutropenia, Raynaud's disease, stomatitis). Three patients discontinued treatment because of toxicity or symptomatic comorbid conditions (n = 1 grade 3 heart failure, n = 1 grade 2 fever + progressive kidney cancer, n = 1 grade 2 fatigue). CONCLUSIONS: Antitumor activity of chemoimmunotherapy reported in phase II to III clinical trials is generalizable to the broader patient population with mesothelioma. However, the tolerability of chemoimmunotherapy is impacted by comorbid conditions in real-world patients. CI - © 2024 The Authors. FAU - Dagogo-Jack, Ibiayi AU - Dagogo-Jack I AD - Massachusetts General Hospital Cancer Center, Boston, Massachusetts. AD - Harvard Medical School, Boston, Massachusetts. FAU - Lasko, Aubrey AU - Lasko A AD - Massachusetts General Hospital Cancer Center, Boston, Massachusetts. FAU - Krueger, Elizabeth A AU - Krueger EA AD - Massachusetts General Hospital Cancer Center, Boston, Massachusetts. FAU - Tsang, Kitman AU - Tsang K AD - Massachusetts General Hospital Cancer Center, Boston, Massachusetts. FAU - Rao, Revati AU - Rao R AD - Harvard Medical School, Boston, Massachusetts. AD - Department of Medicine, Newton Wellesley Hospital, Newton, Massachusetts. FAU - Hambelton, Grace AU - Hambelton G AD - Massachusetts General Hospital Cancer Center, Boston, Massachusetts. FAU - Digumarthy, Subba R AU - Digumarthy SR AD - Harvard Medical School, Boston, Massachusetts. AD - Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts. LA - eng PT - Journal Article DEP - 20241122 PL - United States TA - JTO Clin Res Rep JT - JTO clinical and research reports JID - 101769967 PMC - PMC11998111 OTO - NOTNLM OT - Chemoimmunotherapy OT - Durvalumab OT - Immunotherapy OT - Mesothelioma COIS- Dr. Dagogo-Jack has received honoraria from Foundation Medicine, Creative Education Concepts, OncLive, ASCO Post, DAVA Oncology, Medscape, Research to Practice, Total Health, Aptitude Health, American Lung Association, PeerView; consulting fees from AstraZeneca, Boehringer Ingelheim, Bayer, BostonGene, Bristol Myers Squibb, Catalyst, Genentech, Gilead, Janssen, Merus, Novocure, Pfizer, Roche, Sanofi-Genzyme, Syros, ThermoFisher Scientific, and Xcovery, research support from Array, Genentech, Novartis, Pfizer, and Guardant Health; and travel support from Array and Pfizer. Mrs. Lasko has received consulting fees from Janssen. Mrs. Krueger has received consulting fees from Pfizer. Dr. Digumarthy provides independent image analysis for hospital-contracted clinical research trials programs for Merck, Pfizer, Bristol Myers Squibb, Novartis, Roche, Polaris, Cascadian, Abbvie, Gradalis, Bayer, Zai laboratories, Biengen, Riverain, Resonance, AstraZeneca, Analise. Research grants from Lunit Inc, GE, Qure AI, Vuno, honorarium from Siemens, and book royalties from Elsevier. The remaining authors declare no conflict of interest. EDAT- 2025/04/16 06:19 MHDA- 2025/04/16 06:20 PMCR- 2024/11/22 CRDT- 2025/04/16 05:23 PHST- 2024/07/30 00:00 [received] PHST- 2024/10/29 00:00 [revised] PHST- 2024/11/16 00:00 [accepted] PHST- 2025/04/16 06:20 [medline] PHST- 2025/04/16 06:19 [pubmed] PHST- 2025/04/16 05:23 [entrez] PHST- 2024/11/22 00:00 [pmc-release] AID - S2666-3643(24)00145-0 [pii] AID - 100775 [pii] AID - 10.1016/j.jtocrr.2024.100775 [doi] PST - epublish SO - JTO Clin Res Rep. 2024 Nov 22;6(5):100775. doi: 10.1016/j.jtocrr.2024.100775. eCollection 2025 May. PMID- 36507132 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221221 IS - 2296-2360 (Print) IS - 2296-2360 (Electronic) IS - 2296-2360 (Linking) VI - 10 DP - 2022 TI - Effectiveness of unilateral sequential video-assisted sympathetic chain blockage for primary palmar hyperidrosis in children and adolescents. PG - 1067141 LID - 10.3389/fped.2022.1067141 [doi] LID - 1067141 AB - INTRODUCTION: Primary palmar hyperhidrosis (PPH) is a severely debilitating condition that can affect patients of any age. Thoracoscopic sympathectomy provides a definitive treatment for PPH. Aim of this study is to investigate the effectiveness of unilateral sequential video-assisted thoracic sympathetic chain clamping (VATSCC) by clips application in pediatric population. METHODS: The surgical procedure was done in the semi-sitting position, under general anesthesia with orotracheal intubation. Mean operation time was 23 ± 6 min (range 12-45). Two 5 mm ports were inserted at the level of the middle axillary line in the second and fourth intercostal space respectively. The sympathetic chain was identified, and two clips were applied, the first one at the level of the third and the second one, at the level of the fourth rib. No chest tube was used. Resolution of symptoms, complications, recurrence rate, onset and duration of compensatory hyperhidrosis were analyzed. RESULTS: From August 2017 to September 2021, 58 patients (male:female ratio 32:26), mean age 16.5 years (range 14-19), with PPH underwent unilateral sequential VATSCC by clips application, starting on the dominant hand. The contralateral side was operated 2 months after. All patients except one (transient pneumothorax) were discharged on the first post-op day. No immediate or late complications have been recorded. Mean follow-up was 32 months (range 6-41). All patients except one (1,7%), affected by Raynaud's disease, showed a complete resolution of the symptom. Seven patients (12%) developed transient moderate compensative hyperhidrosis (CH) that spontaneously disappeared in the postoperative period. CONCLUSIONS: Unilateral sequential thoracoscopic sympathetic chain clamping for PPH in pediatric patients is a safe and very effective procedure with a low complication rate and low incidence of postoperative CH that, in our experience, resolved spontaneously in the postoperative period, after the second surgery leading to an improvement in the quality of life. CI - © 2022 Adorisio, Davoli, Ceriati, Battaglia, Camanni and De Peppo. FAU - Adorisio, Ottavio AU - Adorisio O AD - Department of Pediatric Surgery, Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. FAU - Davoli, Enrico AU - Davoli E AD - Department of General Surgery, Campus Biomedico University Hospital, Rome, Italy. FAU - Ceriati, Emanuela AU - Ceriati E AD - Department of Pediatric Surgery, Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. FAU - Battaglia, Sonia AU - Battaglia S AD - Department of Pediatric Surgery, Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. FAU - Camanni, Daniela AU - Camanni D AD - Department of Pediatric Surgery, Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. FAU - De Peppo, Francesco AU - De Peppo F AD - Department of Pediatric Surgery, Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. LA - eng PT - Journal Article DEP - 20221123 PL - Switzerland TA - Front Pediatr JT - Frontiers in pediatrics JID - 101615492 PMC - PMC9727172 OTO - NOTNLM OT - compensatory hyperhidrosis OT - palmar hyperhidrosis OT - sweating OT - sympathecotomy OT - thoracoscopy COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/12/13 06:00 MHDA- 2022/12/13 06:01 PMCR- 2022/11/23 CRDT- 2022/12/12 11:32 PHST- 2022/10/11 00:00 [received] PHST- 2022/11/08 00:00 [accepted] PHST- 2022/12/12 11:32 [entrez] PHST- 2022/12/13 06:00 [pubmed] PHST- 2022/12/13 06:01 [medline] PHST- 2022/11/23 00:00 [pmc-release] AID - 10.3389/fped.2022.1067141 [doi] PST - epublish SO - Front Pediatr. 2022 Nov 23;10:1067141. doi: 10.3389/fped.2022.1067141. eCollection 2022. PMID- 16084666 OWN - NLM STAT- MEDLINE DCOM- 20060810 LR - 20131121 IS - 0306-9877 (Print) IS - 0306-9877 (Linking) VI - 66 IP - 1 DP - 2006 TI - High-dose zinc to terminate angina pectoris: a review and hypothesis for action by ICAM inhibition. PG - 169-72 AB - We reviewed the literature related to the effects of high-dose zinc in arteriosclerosis-induced angina pectoris. Lipid peroxidation and LDL oxidation are believed to be critical for arteriosclerosis, and consequently angina pectoris. Administration of biologically available zinc was a beneficial treatment in a significant percentage of patients with severely symptomatic, inoperable atherosclerotic disease. In these patients, there was no difference in zinc concentration between patients with and without atherosclerosis in whole blood, erythocytes or hair, but there was a major difference between normal aorta and diseased aortas (40.6 ppm zinc in normal aorta vs. 23.2 ppm zinc in atherosclerotic aorta, 40.6 ppm zinc in normal aorta vs. 19.4 ppm zinc in atherosclerotic aneurysm aorta, and no difference between normal and aneurysm aorta), although copper was low in aneurysm aorta. Medication with high-dose zinc sulfate to raise zinc serum concentrations from 95 to 177 microg/dl resulted in objective improvement in 12 of 16 of these patients, including a patient that also had Raynaud's disease. Long term environmental exposure to zinc resulted in a 40% reduction in the incidence of angina of effort compared to people not exposed to environmental zinc (P<0.01) and a 40% reduction in the incidence of probable ischemia in exercise (P<0.001). Lead had no effect while cadmium exposure resulted in more than tripling the incidence of angina of effort (P<0.001). The antioxidative action of zinc prevents oxidation of LDL cholesterol and consequently stops the main mechanism of atherogenesis. Zinc blocks calcium and its several actions on atherogenesis. Increased amounts of cytotoxic cytokines such as TNF-alpha, IL-beta and IL-8, often produced in the elderly, are blocked by high-dose zinc. We hypothesize that higher serum concentrations of LDL cholesterol resulting from administration of 300 mg of zinc per day is caused by a release of low density cholesterol from cardiovascular tissues, beneficially flushing it into the serum where it is readily observed, thus decreasing arteriosclerosis, increasing circulation, terminating angina pectoris and restoring more youthful cardiac function. Although prevention of cholesterol-induced arteriosclerosis by zinc is predicted from findings related to oxidative stress and lipid peroxidation, removal of LDL might be attributable to action of ionic zinc on ICAM inhibition. In stark contrast to current practice, high-dose zinc should be considered as basic in the strategy of prophylaxis and therapy of the atherosclerosis process to terminate angina pectoris and restore youthful cardiac function. FAU - Eby, George A AU - Eby GA AD - George Eby Research, 14909-C Fitzhugh Road, Austin, TX 78704, USA. george.eby@coldcure.com FAU - Halcomb, William W AU - Halcomb WW LA - eng PT - Journal Article DEP - 20050805 PL - United States TA - Med Hypotheses JT - Medical hypotheses JID - 7505668 RN - 0 (Cholesterol, LDL) RN - J41CSQ7QDS (Zinc) SB - IM MH - Angina Pectoris/*drug therapy/etiology MH - Aorta/*metabolism/pathology MH - Atherosclerosis/drug therapy MH - Cholesterol, LDL/blood/metabolism MH - Humans MH - Zinc/metabolism/*therapeutic use EDAT- 2005/08/09 09:00 MHDA- 2006/08/11 09:00 CRDT- 2005/08/09 09:00 PHST- 2005/06/03 00:00 [received] PHST- 2005/06/07 00:00 [revised] PHST- 2005/06/09 00:00 [accepted] PHST- 2005/08/09 09:00 [pubmed] PHST- 2006/08/11 09:00 [medline] PHST- 2005/08/09 09:00 [entrez] AID - S0306-9877(05)00323-3 [pii] AID - 10.1016/j.mehy.2005.06.013 [doi] PST - ppublish SO - Med Hypotheses. 2006;66(1):169-72. doi: 10.1016/j.mehy.2005.06.013. Epub 2005 Aug 5. PMID- 39592536 OWN - NLM STAT- MEDLINE DCOM- 20250922 LR - 20250925 IS - 1573-501X (Electronic) IS - 1381-1991 (Print) IS - 1381-1991 (Linking) VI - 29 IP - 5 DP - 2025 Oct TI - An overview on pharmaceutical applications of phosphodiesterase enzyme 5 (PDE5) inhibitors. PG - 4765-4785 LID - 10.1007/s11030-024-11016-2 [doi] AB - Phosphodiesterase enzyme 5 (PDE5) inhibitors have emerged as one of the leading molecules for the treatment of erectile dysfunction (ED). PDE5 inhibitors are categorized structurally into several classes. PDE5 inhibitors have been a multidisciplinary endeavor that attracts the attention of researchers because of their multiple pharmaceutical applications. Beyond their action on ED, PDE5 inhibitors are widely used in treatment of benign prostatic hypertrophy (BPH), Eisenmenger's syndrome, Raynaud's Disease, Intrauterine growth retardation (IUGR), Mountain sickness, Bladder pain syndrome/interstitial cystitis (BPS/IC), pulmonary arterial hypertension and type II diabetes (insulin resistance). In addition, PDE5 inhibitors also show promising antiproliferative activity, anti-Alzheimer and COX-1/COX-2 inhibitory activity (anti-inflammatory). Pharmacokinetics, Pharmacogenetics and toxicity of PDE5 inhibitors were finally explored. The diverse therapeutic applications, the high feasibility of structural modification and the appropriate pharmacokinetic properties of PDE5 inhibitors have motivated researchers to develop new scaffolds that have been either under clinical trials or approved by FDA and utilize them to overcome some recent global concerns, such as COVID-19. CI - © 2024. The Author(s). FAU - Nemr, Mohamed T M AU - Nemr MTM AD - Pharmaceutical Organic Chemistry Department, Faculty of Pharmacy, Cairo University, Kasr El-Eini Street 11562, Cairo, Egypt. mohamed.nemr@pharma.cu.edu.eg. FAU - Abdelaziz, Mostafa A AU - Abdelaziz MA AD - Department of Chemistry, College of Wooster, Wooster, OH, 44691, USA. FAU - Teleb, Mohamed AU - Teleb M AD - Department of Pharmaceutical Chemistry, Faculty of Pharmacy, Alexandria University, Alexandria, 21521, Egypt. AD - Faculty of Pharmacy, Alamein International University (AIU), Alamein City, Alamein City, 5060310, Egypt. FAU - Elmasry, Ahmed E AU - Elmasry AE AD - Organic & Medicinal Chemistry Department, Faculty of Pharmacy, University of Sadat City, Menoufia, Egypt. FAU - Elshaier, Yaseen A A M AU - Elshaier YAAM AD - Organic & Medicinal Chemistry Department, Faculty of Pharmacy, University of Sadat City, Menoufia, Egypt. yaseen.elshaier@fop.usc.edu.eg. LA - eng PT - Journal Article PT - Review DEP - 20241127 PL - Netherlands TA - Mol Divers JT - Molecular diversity JID - 9516534 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) SB - IM MH - Humans MH - *Phosphodiesterase 5 Inhibitors/therapeutic use/pharmacology/chemistry/pharmacokinetics MH - Erectile Dysfunction/drug therapy MH - COVID-19 Drug Treatment MH - Animals MH - Male MH - SARS-CoV-2 MH - COVID-19 MH - Cyclic Nucleotide Phosphodiesterases, Type 5/metabolism PMC - PMC12454544 OTO - NOTNLM OT - Anti-Alzheimer and anti-inflammatory OT - Antiproliferative OT - Erectile dysfunction OT - PDE5 enzymes OT - Type 2 diabetes COIS- Declarations. Conflict of interest: The authors declare no competing interests. EDAT- 2024/11/27 00:20 MHDA- 2025/09/23 03:17 PMCR- 2024/11/27 CRDT- 2024/11/26 23:15 PHST- 2024/08/24 00:00 [received] PHST- 2024/10/12 00:00 [accepted] PHST- 2025/09/23 03:17 [medline] PHST- 2024/11/27 00:20 [pubmed] PHST- 2024/11/26 23:15 [entrez] PHST- 2024/11/27 00:00 [pmc-release] AID - 10.1007/s11030-024-11016-2 [pii] AID - 11016 [pii] AID - 10.1007/s11030-024-11016-2 [doi] PST - ppublish SO - Mol Divers. 2025 Oct;29(5):4765-4785. doi: 10.1007/s11030-024-11016-2. Epub 2024 Nov 27. PMID- 29239149 OWN - NLM STAT- MEDLINE DCOM- 20191031 LR - 20191031 IS - 1827-1596 (Electronic) IS - 0375-9393 (Linking) VI - 84 IP - 4 DP - 2018 Apr TI - Agreement between ccNexfin CO-trek cardiac output and intermittent cold-bolus pulmonary thermodilution in a prospective multicenter study. PG - 473-480 LID - 10.23736/S0375-9393.17.12051-1 [doi] AB - BACKGROUND: The ccNexfin System uses the CO-trek algorithm to analyze a non-invasively obtained arterial pressure waveform and calculate cardiac output (NEXCO). It remains matter of debate whether NEXCO can replace invasive, pulmonary artery catheter-derived, cold-bolus pulmonary thermodilution cardiac output measurement (PACCO). This study aimed at testing NEXCO-PACCO agreement in a large sample size, multicenter study. We hypothesized that agreement between NEXCO and PACCO would be demonstrated by a mean accuracy (bias) <0.6 L/min with a percentage error <30%. METHODS: Patients undergoing cardiac surgery in three academic hospitals clinically requiring pulmonary artery catheterization were included. Exclusion criteria were aortic, pulmonary and tricuspid (valve) abnormalities, non-sinus rhythm and insufficient perfusion of the digits such as in Raynaud's disease. After induction of anesthesia, cardiac output was measured with four cold bolus thermodilution measurements and four averaged 30-second ccNexfin measurements randomized through the respiratory cycle to obtain one measurement pair. Mean accuracy and precision of ccNexfin were expressed as bias (mean of all NEXCO-PACCO differences) and limits of agreement (LOA) (1.96·SD of bias). Percentage error was calculated as [LOA / (NEXCO-PACCO average)]. RESULTS: Fifty-five patients were enrolled in the study, 51 completed the protocol. Median PACCO was 3.7 (IQR: 3.2 to 4.6) L/min and median NEXCO was 3.8 (IQR: 3.1 to 4.7) L/min. NEXCO-PACCO bias was 0.1 (LOA: -1.4 to +1.6) L/min with a 37% percentage error. CONCLUSIONS: In this study, cardiac output measurement with ccNexfin failed to meet the predefined criteria for agreement with cold-bolus pulmonary artery thermodilution. FAU - Sperna Weiland, Nicolaas H AU - Sperna Weiland NH AD - Department of Anesthesiology, Academic Medical Center (AMC) Amsterdam, University of Amsterdam, Amsterdam, The Netherlands - n.h.spernaweiland@amc.uva.nl. FAU - de Wever, Jim W AU - de Wever JW AD - Department of Anesthesiology, Free University Medical Center, Amsterdam, The Netherlands. FAU - van Duivenvoorde, Yoni AU - van Duivenvoorde Y AD - Department of Anesthesiology, Free University Medical Center, Amsterdam, The Netherlands. FAU - Boer, Christa AU - Boer C AD - Department of Anesthesiology, Free University Medical Center, Amsterdam, The Netherlands. FAU - Mitrev, Ludmil AU - Mitrev L AD - Department of Anesthesiology, Cooper University Hospital, Cooper Medical School of Rowan University, Camden, NJ, USA. FAU - Muntazar, Muhammad AU - Muntazar M AD - Department of Anesthesiology, Cooper University Hospital, Cooper Medical School of Rowan University, Camden, NJ, USA. FAU - Patel, Kinjal AU - Patel K AD - Department of Anesthesiology, Cooper University Hospital, Cooper Medical School of Rowan University, Camden, NJ, USA. FAU - Hollmann, Markus W AU - Hollmann MW AD - Department of Anesthesiology, Academic Medical Center (AMC) Amsterdam, University of Amsterdam, Amsterdam, The Netherlands. FAU - Preckel, Benedikt AU - Preckel B AD - Department of Anesthesiology, Academic Medical Center (AMC) Amsterdam, University of Amsterdam, Amsterdam, The Netherlands. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20171213 PL - Italy TA - Minerva Anestesiol JT - Minerva anestesiologica JID - 0375272 SB - IM MH - Aged MH - *Algorithms MH - *Cardiac Output MH - *Cardiac Surgical Procedures MH - Female MH - Heart Function Tests/*methods MH - Humans MH - Male MH - Monitoring, Intraoperative/*methods MH - Prospective Studies MH - Pulmonary Artery MH - Thermodilution/*methods EDAT- 2017/12/15 06:00 MHDA- 2019/11/02 06:00 CRDT- 2017/12/15 06:00 PHST- 2017/12/15 06:00 [pubmed] PHST- 2019/11/02 06:00 [medline] PHST- 2017/12/15 06:00 [entrez] AID - S0375-9393.17.12051-1 [pii] AID - 10.23736/S0375-9393.17.12051-1 [doi] PST - ppublish SO - Minerva Anestesiol. 2018 Apr;84(4):473-480. doi: 10.23736/S0375-9393.17.12051-1. Epub 2017 Dec 13. PMID- 36204695 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221009 IS - 2352-5126 (Print) IS - 2352-5126 (Electronic) IS - 2352-5126 (Linking) VI - 29 DP - 2022 Nov TI - Lower limb ulcer and Raynaud's phenomenon. PG - 67-69 LID - 10.1016/j.jdcr.2022.08.054 [doi] FAU - Chérif, Mohammad Yassine AU - Chérif MY AD - Department of Rheumatology, CHU Brugmann, Université Libre de Bruxelles, Brussels, Belgium. FAU - Weynand, Marjolaine AU - Weynand M AD - Department of Rheumatology, CHU Brugmann, Université Libre de Bruxelles, Brussels, Belgium. FAU - Kamgang, Prochore AU - Kamgang P AD - Department of Internal Medecine, Université Libre de Bruxelles, Brussels, Belgium. FAU - Badot, Valérie AU - Badot V AD - Department of Rheumatology, CHU Brugmann, Université Libre de Bruxelles, Brussels, Belgium. AD - Department of Immunology and Allergology, Université Libre de Bruxelles, Brussels, Belgium. FAU - Mostmans, Yora AU - Mostmans Y AD - Department of Immunology and Allergology, Université Libre de Bruxelles, Brussels, Belgium. AD - Department of Dermatology, Centre Hospitalier Universitaire Brugmann, Université Libre de Bruxelles, Brussels, Belgium. LA - eng PT - Journal Article DEP - 20220907 PL - United States TA - JAAD Case Rep JT - JAAD case reports JID - 101665210 PMC - PMC9529539 OTO - NOTNLM OT - LLU, lower limb ulcer OT - NA, Necrotic angiodermatitis OT - RP, Raynaud's phenomenon OT - Raynaud's phenomenon OT - SSc, systemic sclerosis OT - arteriopathy OT - botulinum toxin (botox) OT - calcinosis OT - connective tissue disease OT - lower limb ulcers OT - lymphangiopathy OT - macroangiopathy OT - microangiopathy OT - naifold videocapillaroscopy OT - systemic sclerosis OT - venous insufficiency COIS- None disclosed. EDAT- 2022/10/08 06:00 MHDA- 2022/10/08 06:01 PMCR- 2022/09/07 CRDT- 2022/10/07 02:59 PHST- 2022/10/07 02:59 [entrez] PHST- 2022/10/08 06:00 [pubmed] PHST- 2022/10/08 06:01 [medline] PHST- 2022/09/07 00:00 [pmc-release] AID - S2352-5126(22)00410-6 [pii] AID - 10.1016/j.jdcr.2022.08.054 [doi] PST - epublish SO - JAAD Case Rep. 2022 Sep 7;29:67-69. doi: 10.1016/j.jdcr.2022.08.054. eCollection 2022 Nov. PMID- 40174776 OWN - NLM STAT- In-Process LR - 20250911 IS - 1578-2190 (Electronic) IS - 0001-7310 (Linking) VI - 116 IP - 6 DP - 2025 Jun TI - [Translated article] RF-Update on the Treatment of Raynaud's Phenomenon. PG - T643-T644 LID - S0001-7310(25)00213-3 [pii] LID - 10.1016/j.ad.2025.03.026 [doi] FAU - Merlo-Gómez, P AU - Merlo-Gómez P AD - Servicio de Dermatología, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Madrid, Spain. Electronic address: merlogomezp@gmail.com. FAU - Polo Rodríguez, I AU - Polo Rodríguez I AD - Servicio de Dermatología, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Madrid, Spain. FAU - Lucía Pinto-Pulido, E AU - Lucía Pinto-Pulido E AD - Servicio de Dermatología, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Madrid, Spain. LA - eng LA - spa PT - Journal Article DEP - 20250331 PL - Spain TA - Actas Dermosifiliogr JT - Actas dermo-sifiliograficas JID - 0373062 SB - IM OTO - NOTNLM OT - Connective tissue disease OT - Enfermedad del tejido conectivo OT - Fenómeno de Raynaud OT - Opciones terapéuticas OT - Raynaud's phenomenon OT - Therapeutic options EDAT- 2025/04/03 00:25 MHDA- 2025/04/03 00:25 CRDT- 2025/04/02 19:30 PHST- 2024/03/22 00:00 [received] PHST- 2024/05/28 00:00 [revised] PHST- 2024/06/01 00:00 [accepted] PHST- 2025/04/03 00:25 [pubmed] PHST- 2025/04/03 00:25 [medline] PHST- 2025/04/02 19:30 [entrez] AID - S0001-7310(25)00213-3 [pii] AID - 10.1016/j.ad.2025.03.026 [doi] PST - ppublish SO - Actas Dermosifiliogr. 2025 Jun;116(6):T643-T644. doi: 10.1016/j.ad.2025.03.026. Epub 2025 Mar 31. PMID- 35387220 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221003 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 6 IP - 3 DP - 2021 Oct TI - Is axillary botulinum toxin efficient in controlling secondary Raynaud's phenomenon? A case report. PG - 327-329 LID - 10.1177/23971983211034077 [doi] AB - Raynaud's phenomenon when secondary to underlying systemic disease such as systemic sclerosis occurs early in the disease course and progression can bring significant morbidity such as pain, digital ulceration, and necrosis. Standard medical therapies are aimed at promoting distal arterial vasodilation but are often inadequate in managing Raynaud's phenomenon. Options for refractory cases include surgical and chemical sympathectomy with Botulinum neurotoxin type A (BoNT/A) hand injections but the latter can be associated with transient hand weakness. We describe the case of a 35-year-old woman with undifferentiated connective tissue disease, Raynaud's phenomenon, and concomitant primary focal axillary hyperhidrosis for which she received axillary BoNT/A therapy every 6 months who noted significant improvement in her Raynaud's phenomenon and hand arthralgias for 5 months following the axillary injections. This effect remained durable after 24 months of therapy. This improvement in Raynaud's phenomenon after axillary BoNT/A has not been previously described. CI - © The Author(s) 2021. FAU - DeMasters, David AU - DeMasters D AUID- ORCID: 0000-0002-8479-3452 AD - Wright-Patterson Medical Center, Wright-Patterson AFB, OH, USA. FAU - Sturgill, Emily AU - Sturgill E AD - Wright-Patterson Medical Center, Wright-Patterson AFB, OH, USA. FAU - Bartholomew, Alan AU - Bartholomew A AD - Wright-Patterson Medical Center, Wright-Patterson AFB, OH, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210729 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922664 OTO - NOTNLM OT - Onabotulinum toxin A OT - Raynaud’s phenomenon OT - botulinum toxin A OT - hyperhidrosis OT - systemic sclerosis OT - undifferentiated connective tissue disease COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2022/04/08 06:00 MHDA- 2022/04/08 06:01 PMCR- 2022/10/01 CRDT- 2022/04/07 05:22 PHST- 2021/04/19 00:00 [received] PHST- 2021/07/03 00:00 [accepted] PHST- 2022/04/07 05:22 [entrez] PHST- 2022/04/08 06:00 [pubmed] PHST- 2022/04/08 06:01 [medline] PHST- 2022/10/01 00:00 [pmc-release] AID - 10.1177_23971983211034077 [pii] AID - 10.1177/23971983211034077 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2021 Oct;6(3):327-329. doi: 10.1177/23971983211034077. Epub 2021 Jul 29. PMID- 38322394 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240210 IS - 2616-163X (Electronic) IS - 0016-9560 (Print) IS - 0016-9560 (Linking) VI - 55 IP - 1 DP - 2021 Mar TI - Management of severe Raynaud's Phenomenon secondary to autoimmune vasculopathy in a young woman. PG - 96-100 LID - 10.4314/gmj.v55i1.16 [doi] AB - Raynaud's phenomenon as a cause of acute limb ischaemia in the warmer climates of Sub-Saharan Africa region is uncommon because it is usually thought of as a disease common in cold weather. The prevalence of connective tissue diseases among Black Africans is increasing, and these conditions are associated with secondary Raynaud's phenomenon and ischaemic digital lesions. We present the case of a 36-year old female with dermatomyositis/systemic sclerosis overlap and secondary Raynaud's phenomenon who presented with acute limb ischemia (wet gangrene of all digits) in a Tertiary Hospital in Ghana. Young patients presenting with acute limb ischaemia should also be screened for an underlying connective tissue disease. In patients with connective tissue disease, the onset of digital vasculopathy can be rapid and progressive, hence treatment must be prompt and comprehensive to enable better clinical outcomes. FUNDING: None declared. CI - Copyright © The Author(s). FAU - Amissah-Arthur, Maame-Boatemaa AU - Amissah-Arthur MB AD - School of Medicine and Dentistry, University of Ghana Medical School, Accra, Ghana. FAU - Wu, Lily P AU - Wu LP AD - School of Medicine and Dentistry, University of Ghana Medical School, Accra, Ghana. LA - eng PT - Case Reports PT - Journal Article PL - Ghana TA - Ghana Med J JT - Ghana medical journal JID - 0073210 SB - IM PMC - PMC10665273 OTO - NOTNLM OT - Acute limb ischaemia OT - autoimmune OT - connective tissue disease OT - digital vasculopathy OT - secondary Raynaud's phenomenon COIS- Conflict of interest: None declared EDAT- 2021/03/01 00:00 MHDA- 2021/03/01 00:01 PMCR- 2021/03/01 CRDT- 2024/02/07 04:00 PHST- 2021/03/01 00:01 [medline] PHST- 2021/03/01 00:00 [pubmed] PHST- 2024/02/07 04:00 [entrez] PHST- 2021/03/01 00:00 [pmc-release] AID - jGMJ.v55.i1.pg96 [pii] AID - 10.4314/gmj.v55i1.16 [doi] PST - ppublish SO - Ghana Med J. 2021 Mar;55(1):96-100. doi: 10.4314/gmj.v55i1.16. PMID- 34413734 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210821 IS - 1662-6567 (Print) IS - 1662-6567 (Electronic) IS - 1662-6567 (Linking) VI - 13 IP - 2 DP - 2021 May-Aug TI - A Self-Limited Facial Rash in a Lupus Patient: The Case of Primary Facial Raynaud's Phenomenon. PG - 366-371 LID - 10.1159/000517553 [doi] AB - Skin is involved in 80% of systemic lupus erythematosus (SLE) and the second most affected after joint disease. Lupus-specific lesions include (a) acute ones viz. malar rash (80%), (b) subacute ones viz. photosensitive maculopapular dermatitis (50%), and (c) chronic ones viz. discoid rash. The lupus nonspecific lesions include; (a) nonscarring alopecia (86.67%), oral ulcers (56.67%), vasculitic lesions (33.34%), bullous lesions (10%), and Raynaud's phenomenon (6.67%). In this case report, we describe a patient with SLE and antiphospholipid antibodies that had developed a transient facial form of Raynaud's phenomenon that was not associated with disease activity and digital changes. Its association with SLE is discussed. CI - Copyright © 2021 by S. Karger AG, Basel. FAU - El-Reshaid, Kamel AU - El-Reshaid K AD - Department of Medicine, Faculty of Medicine, Kuwait University, Safat, Kuwait. FAU - Al-Bader, Shaikha AU - Al-Bader S AD - Department of Medicine, Nephrology Unit, Amiri Hospital, Ministry of Health, Kuwait City, Kuwait. FAU - Sallam, Hossameldin Tawfik AU - Sallam HT AD - Department of Medicine, Nephrology Unit, Amiri Hospital, Ministry of Health, Kuwait City, Kuwait. LA - eng PT - Case Reports PT - Journal Article DEP - 20210716 PL - Switzerland TA - Case Rep Dermatol JT - Case reports in dermatology JID - 101517685 PMC - PMC8339507 OTO - NOTNLM OT - Autoimmunity OT - Face OT - Raynaud's OT - Skin OT - Systemic lupus erythematosis COIS- The authors have no conflicts of interest to declare. EDAT- 2021/08/21 06:00 MHDA- 2021/08/21 06:01 PMCR- 2021/07/16 CRDT- 2021/08/20 07:01 PHST- 2021/03/22 00:00 [received] PHST- 2021/06/01 00:00 [accepted] PHST- 2021/08/20 07:01 [entrez] PHST- 2021/08/21 06:00 [pubmed] PHST- 2021/08/21 06:01 [medline] PHST- 2021/07/16 00:00 [pmc-release] AID - cde-0013-0366 [pii] AID - 10.1159/000517553 [doi] PST - epublish SO - Case Rep Dermatol. 2021 Jul 16;13(2):366-371. doi: 10.1159/000517553. eCollection 2021 May-Aug. PMID- 26283854 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150818 LR - 20201001 IS - 0976-9668 (Print) IS - 2229-7707 (Electronic) IS - 0976-9668 (Linking) VI - 6 IP - 2 DP - 2015 Jul-Dec TI - Monostotic fibrous dysplasia with Raynaud's phenomenon. PG - 462-4 LID - 10.4103/0976-9668.160042 [doi] AB - Fibrous dysplasia (FD) is a benign bone disorder characterized by alteration in bone morphology. Monostotic FD is the commonest variant and affects the craniofacial bones. Raynaud's phenomenon is recurrent vasospasm of the fingers and toes due to cold exposure. The disease is usually idiopathic or secondary to connective tissue disorders. Raynaud's phenomenon is not described previously with FD. We recently encountered two interesting patients of craniofacial monostotic FD with Raynaud's phenomenon and report the same in this report. FAU - Kumar, K V S Hari AU - Kumar KV AD - Department of Endocrinology, Command Hospital, Chandimandir, Haryana, India. FAU - Aravinda, K AU - Aravinda K AD - Department of Oral Medicine and Radiology, SDD Dental College, Barwala, Punjab, India. FAU - Narayanan, K AU - Narayanan K AD - Department of Rheumatology, Command Hospital, Chandimandir, Haryana, India. LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Nat Sci Biol Med JT - Journal of natural science, biology, and medicine JID - 101571070 PMC - PMC4518434 OTO - NOTNLM OT - Bisphosphonates OT - Raynaud's phenomenon OT - craniofacial dysplasia OT - monostotic fibrous dysplasia COIS- Conflict of Interest: None declared. EDAT- 2015/08/19 06:00 MHDA- 2015/08/19 06:01 PMCR- 2015/07/01 CRDT- 2015/08/19 06:00 PHST- 2015/08/19 06:00 [entrez] PHST- 2015/08/19 06:00 [pubmed] PHST- 2015/08/19 06:01 [medline] PHST- 2015/07/01 00:00 [pmc-release] AID - JNSBM-6-462 [pii] AID - 10.4103/0976-9668.160042 [doi] PST - ppublish SO - J Nat Sci Biol Med. 2015 Jul-Dec;6(2):462-4. doi: 10.4103/0976-9668.160042. PMID- 29044769 OWN - NLM STAT- MEDLINE DCOM- 20190911 LR - 20220330 IS - 1365-2796 (Electronic) IS - 0954-6820 (Linking) VI - 283 IP - 2 DP - 2018 Feb TI - Human papillomavirus vaccination of adult women and risk of autoimmune and neurological diseases. PG - 154-165 LID - 10.1111/joim.12694 [doi] AB - BACKGROUND: Since 2006, human papillomavirus (HPV) vaccines have been introduced in many countries worldwide. Whilst safety studies have been reassuring, focus has been on the primary target group, the young adolescent girls. However, it is also important to evaluate safety in adult women where background disease rates and safety issues could differ significantly. OBJECTIVE: We took advantage of the unique Danish and Swedish nationwide healthcare registers to conduct a cohort study comparing incidence rate ratios (RRs) of 45 preselected serious chronic diseases in quadrivalent HPV (qHPV)-vaccinated and qHPV-unvaccinated adult women 18-44 years of age. METHODS: We used Poisson regression to estimate RRs according to qHPV vaccination status with two-sided 95% confidence intervals (95% CIs). RESULTS: The study cohort comprised 3 126 790 women (1 195 865 [38%] Danish and 1 930 925 [62%] Swedish) followed for 16 386 459 person-years. Vaccine uptake of at least one dose of qHPV vaccine was 8% in the cohort: 18% amongst Danish women and 2% amongst Swedish. We identified seven adverse events with statistically significant increased risks following vaccination-Hashimoto's thyroiditis, coeliac disease, localized lupus erythematosus, pemphigus vulgaris, Addison's disease, Raynaud's disease and other encephalitis, myelitis or encephalomyelitis. After taking multiple testing into account and conducting self-controlled case series analyses, coeliac disease (RR 1.56 [95% confidence interval 1.29-1.89]) was the only remaining association. CONCLUSION: Unmasking of conditions at vaccination visits is a plausible explanation for the increased risk associated with qHPV in this study because coeliac disease is underdiagnosed in Scandinavian populations. In conclusion, our study of serious adverse event rates in qHPV-vaccinated and qHPV-unvaccinated adult women 18-44 years of age did not raise any safety issues of concern. CI - © 2017 The Association for the Publication of the Journal of Internal Medicine. FAU - Hviid, A AU - Hviid A AUID- ORCID: 0000-0002-7509-9127 AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. FAU - Svanström, H AU - Svanström H AUID- ORCID: 0000-0003-1015-7215 AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. FAU - Scheller, N M AU - Scheller NM AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. FAU - Grönlund, O AU - Grönlund O AD - Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden. FAU - Pasternak, B AU - Pasternak B AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. AD - Department of Medicine Solna, Clinical Epidemiology Unit, Karolinska Institutet, Stockholm, Sweden. FAU - Arnheim-Dahlström, L AU - Arnheim-Dahlström L AD - Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20171018 PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 RN - 0 (Papillomavirus Vaccines) SB - IM MH - Adolescent MH - Adult MH - Autoimmune Diseases/epidemiology/*etiology MH - Cohort Studies MH - Denmark/epidemiology MH - Female MH - Humans MH - Mass Vaccination/*adverse effects MH - Nervous System Diseases/epidemiology/*etiology MH - Papillomavirus Infections/*prevention & control MH - Papillomavirus Vaccines/administration & dosage/*adverse effects MH - Registries MH - Risk Factors MH - Sweden/epidemiology MH - Young Adult OTO - NOTNLM OT - Cohort study OT - Epidemiology OT - Human papillomavirus OT - Vaccine Safety EDAT- 2017/10/19 06:00 MHDA- 2019/09/12 06:00 CRDT- 2017/10/19 06:00 PHST- 2017/10/19 06:00 [pubmed] PHST- 2019/09/12 06:00 [medline] PHST- 2017/10/19 06:00 [entrez] AID - 10.1111/joim.12694 [doi] PST - ppublish SO - J Intern Med. 2018 Feb;283(2):154-165. doi: 10.1111/joim.12694. Epub 2017 Oct 18. PMID- 32002464 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2332-0885 (Print) IS - 2332-0885 (Electronic) IS - 2332-0885 (Linking) VI - 6 IP - 1 DP - 2019 TI - Pacinian hyperplasia presenting with Raynaud's phenomenon. PG - 148-152 LID - 10.1080/23320885.2019.1698958 [doi] AB - Pacinian corpuscle pathology is a rare clinical entity and an uncommonly reported cause of digital pain. While many prior reports implicate hand trauma, we describe a case of Pacinian hyperplasia found in a patient with Raynaud's phenomenon and propose a potential mechanism of disease. CI - © 2019 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group. FAU - Pickrell, Brent B AU - Pickrell BB AD - Division of Plastic Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA. FAU - Talbot, Simon G AU - Talbot SG AD - Division of Plastic Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA. FAU - Costigan, Danielle C AU - Costigan DC AD - Department of Pathology, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA. FAU - Sampson, Christian E AU - Sampson CE AD - Division of Plastic Surgery, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20191213 PL - United States TA - Case Reports Plast Surg Hand Surg JT - Case reports in plastic surgery & hand surgery JID - 101655541 PMC - PMC6968677 OTO - NOTNLM OT - CREST syndrome OT - Pacinian corpuscle OT - Raynaud’s phenomenon OT - digital ischemia OT - hand pain OT - hyperplasia EDAT- 2020/02/01 06:00 MHDA- 2020/02/01 06:01 PMCR- 2019/12/13 CRDT- 2020/02/01 06:00 PHST- 2019/03/31 00:00 [received] PHST- 2019/11/25 00:00 [accepted] PHST- 2020/02/01 06:00 [entrez] PHST- 2020/02/01 06:00 [pubmed] PHST- 2020/02/01 06:01 [medline] PHST- 2019/12/13 00:00 [pmc-release] AID - 1698958 [pii] AID - 10.1080/23320885.2019.1698958 [doi] PST - epublish SO - Case Reports Plast Surg Hand Surg. 2019 Dec 13;6(1):148-152. doi: 10.1080/23320885.2019.1698958. eCollection 2019. PMID- 29201156 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231112 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 9 IP - 12 DP - 2017 Dec TI - Evidence-based management of Raynaud's phenomenon. PG - 317-329 LID - 10.1177/1759720X17740074 [doi] AB - Raynaud's phenomenon (RP) is relevant to the rheumatologist because it may signify an underlying connective tissue disease and also because it can be very challenging to treat, especially when it has progressed to digital ulceration or critical ischaemia. This review article discusses diagnosis (does this patient have an underlying connective tissue disease?), including the role for nailfold capillaroscopy, and treatment. Management of 'uncomplicated' RP is first described and then treatment of RP complicated by progression to digital ulceration or critical ischaemia, highlighting recent advances (including phosphodiesterase type 5 inhibition, and endothelin 1 receptor antagonism) and the evidence base underpinning these. Possible future therapies are briefly discussed. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester UK, M13 9PT and NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester, UK. LA - eng PT - Journal Article PT - Review DEP - 20171120 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC5700788 OTO - NOTNLM OT - Raynaud’s phenomenon OT - critical ischaemia OT - digital ulceration OT - systemic sclerosis OT - treatment COIS- Conflict of interest statement: ALH has done consultancy work for Actelion, served on a Data Safety Monitoring Board for Apricus, received research funding and speaker’s fees from Actelion, and speaker’s fees from GSK. EDAT- 2017/12/05 06:00 MHDA- 2017/12/05 06:01 PMCR- 2017/12/01 CRDT- 2017/12/05 06:00 PHST- 2017/04/20 00:00 [received] PHST- 2017/09/07 00:00 [accepted] PHST- 2017/12/05 06:00 [entrez] PHST- 2017/12/05 06:00 [pubmed] PHST- 2017/12/05 06:01 [medline] PHST- 2017/12/01 00:00 [pmc-release] AID - 10.1177_1759720X17740074 [pii] AID - 10.1177/1759720X17740074 [doi] PST - ppublish SO - Ther Adv Musculoskelet Dis. 2017 Dec;9(12):317-329. doi: 10.1177/1759720X17740074. Epub 2017 Nov 20. PMID- 39341355 OWN - NLM STAT- In-Process LR - 20241220 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 92 IP - 1 DP - 2025 Jan TI - Botulinum toxin for Raynaud's phenomenon: A decade of real-world evidence. PG - 173-175 LID - S0190-9622(24)02890-1 [pii] LID - 10.1016/j.jaad.2024.09.034 [doi] FAU - Pinto-Pulido, Elena Lucía AU - Pinto-Pulido EL AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. Electronic address: elucia.pinto95@gmail.com. FAU - Polo-Rodríguez, Isabel AU - Polo-Rodríguez I AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. FAU - García-Verdú, Elena AU - García-Verdú E AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. FAU - Merlo-Gómez, Paola AU - Merlo-Gómez P AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. FAU - Martínez-Alcalde, Laura AU - Martínez-Alcalde L AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. FAU - Ruiz-Gutiérrez, Lucía AU - Ruiz-Gutiérrez L AD - Department of Rheumatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. FAU - Medina-Montalvo, Susana AU - Medina-Montalvo S AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Spain. LA - eng PT - Journal Article DEP - 20240926 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM OTO - NOTNLM OT - Raynaud's phenomenon OT - botulinum toxin OT - connective tissue disease OT - dermatologic therapy OT - systemic sclerosis COIS- Conflicts of interest None disclosed. EDAT- 2024/09/29 00:42 MHDA- 2024/09/29 00:42 CRDT- 2024/09/28 19:17 PHST- 2024/06/30 00:00 [received] PHST- 2024/08/22 00:00 [revised] PHST- 2024/09/18 00:00 [accepted] PHST- 2024/09/29 00:42 [pubmed] PHST- 2024/09/29 00:42 [medline] PHST- 2024/09/28 19:17 [entrez] AID - S0190-9622(24)02890-1 [pii] AID - 10.1016/j.jaad.2024.09.034 [doi] PST - ppublish SO - J Am Acad Dermatol. 2025 Jan;92(1):173-175. doi: 10.1016/j.jaad.2024.09.034. Epub 2024 Sep 26. PMID- 42199593 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260527 LR - 20260527 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 4 DP - 2026 Apr TI - Rheumatoid Arthritis-Associated Interstitial Lung Disease Presenting With Raynaud's Phenomenon: A Case Report. PG - e107697 LID - 10.7759/cureus.107697 [doi] LID - e107697 AB - Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a recognized extra-articular manifestation of rheumatoid arthritis. Pulmonary involvement may occasionally precede overt articular disease, creating diagnostic challenges. A patient presented with breathlessness for 2-3 months and joint pain of similar duration, along with bluish discoloration of the left middle finger, suggestive of Raynaud's phenomenon. Respiratory examination revealed bilateral inspiratory crepitations in the infrascapular and infra-axillary areas. Rheumatoid factor was positive, while antinuclear antibody was negative. High-resolution computed tomography (HRCT) of the thorax demonstrated microcystic honeycombing, basal subpleural fibrosis, and traction bronchiectasis, consistent with a usual interstitial pneumonia (UIP) pattern. This case highlights ILD as a potential early manifestation of rheumatoid arthritis and emphasizes the importance of autoimmune evaluation in patients presenting with fibrotic ILD and systemic features such as Raynaud's phenomenon. CI - Copyright © 2026, Ghule et al. FAU - Ghule, Prashant AU - Ghule P AD - Respiratory Medicine, N.K.P. Salve Institute of Medical Sciences and Research Centre and Lata Mangeshkar Hospital (NKPSIMS and LMH), Nagpur, IND. FAU - Sontakke, Anil AU - Sontakke A AD - Respiratory Medicine, N.K.P. Salve Institute of Medical Sciences and Research Centre and Lata Mangeshkar Hospital (NKPSIMS and LMH), Nagpur, IND. FAU - Ali, Saood AU - Ali S AD - Respiratory Medicine, N.K.P. Salve Institute of Medical Sciences and Research Centre and Lata Mangeshkar Hospital (NKPSIMS and LMH), Nagpur, IND. FAU - Kolte, Sagar AU - Kolte S AD - Respiratory Medicine, N.K.P. Salve Institute of Medical Sciences and Research Centre and Lata Mangeshkar Hospital (NKPSIMS and LMH), Nagpur, IND. FAU - Mashalkar, Somesh AU - Mashalkar S AD - Respiratory Medicine, N.K.P. Salve Institute of Medical Sciences and Research Centre and Lata Mangeshkar Hospital (NKPSIMS and LMH), Nagpur, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20260425 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13200223 OTO - NOTNLM OT - autoimmune lung disease OT - high-resolution ct OT - interstitial lung disease OT - raynaud's phenomenon OT - rheumatoid arthritis OT - usual interstitial pneumonia COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/05/27 12:20 MHDA- 2026/05/27 12:21 PMCR- 2026/04/25 CRDT- 2026/05/27 04:29 PHST- 2026/04/25 00:00 [accepted] PHST- 2026/05/27 12:21 [medline] PHST- 2026/05/27 12:20 [pubmed] PHST- 2026/05/27 04:29 [entrez] PHST- 2026/04/25 00:00 [pmc-release] AID - 10.7759/cureus.107697 [doi] PST - epublish SO - Cureus. 2026 Apr 25;18(4):e107697. doi: 10.7759/cureus.107697. eCollection 2026 Apr. PMID- 35566614 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220518 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 11 IP - 9 DP - 2022 Apr 28 TI - Raynaud's Phenomenon with Focus on Systemic Sclerosis. LID - 10.3390/jcm11092490 [doi] LID - 2490 AB - Raynaud's phenomenon is a painful vascular condition in which abnormal vasoconstriction of the digital arteries causes blanching of the skin. The treatment approach can vary depending on the underlying cause of disease. Raynaud's phenomenon can present as a primary symptom, in which there is no evidence of underlying disease, or secondary to a range of medical conditions or therapies. Systemic sclerosis is one of the most frequent causes of secondary Raynaud's phenomenon; its appearance may occur long before other signs and symptoms. Timely, accurate identification of secondary Raynaud's phenomenon may accelerate a final diagnosis and positively alter prognosis. Capillaroscopy is fundamental in the diagnosis and differentiation of primary and secondary Raynaud's phenomenon. It is helpful in the very early stages of systemic sclerosis, along with its role in disease monitoring. An extensive range of pharmacotherapies with various routes of administration are available for Raynaud's phenomenon but a standardized therapeutic plan is still lacking. This review provides insight into recent advances in the understanding of Raynaud's phenomenon pathophysiology, diagnostic methods, and treatment approaches. FAU - Maciejewska, Magdalena AU - Maciejewska M AUID- ORCID: 0000-0003-1009-6395 AD - Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland. FAU - Sikora, Mariusz AU - Sikora M AUID- ORCID: 0000-0002-6162-9916 AD - National Institute of Geriatrics, Rheumatology and Rehabilitation, Spartańska 1, 02-637 Warsaw, Poland. FAU - Maciejewski, Cezary AU - Maciejewski C AUID- ORCID: 0000-0003-2619-7190 AD - 1st Department of Cardiology, Medical University of Warsaw, 02-091 Warsaw, Poland. FAU - Alda-Malicka, Rosanna AU - Alda-Malicka R AD - Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland. FAU - Czuwara, Joanna AU - Czuwara J AD - Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland. FAU - Rudnicka, Lidia AU - Rudnicka L AUID- ORCID: 0000-0002-8308-1023 AD - Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland. LA - eng GR - Medical University of Warsaw./The publication was financed from the subsidy granted to the Medical University of Warsaw./ PT - Journal Article PT - Review DEP - 20220428 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC9105786 OTO - NOTNLM OT - Raynaud’s phenomenon OT - alprostadyl OT - capillaroscopy OT - iloprost OT - microcirculation OT - sulodexide OT - systemic sclerosis OT - vasculopathy COIS- The authors declare no conflict of interest. EDAT- 2022/05/15 06:00 MHDA- 2022/05/15 06:01 PMCR- 2022/04/28 CRDT- 2022/05/14 01:26 PHST- 2022/03/15 00:00 [received] PHST- 2022/04/26 00:00 [revised] PHST- 2022/04/27 00:00 [accepted] PHST- 2022/05/14 01:26 [entrez] PHST- 2022/05/15 06:00 [pubmed] PHST- 2022/05/15 06:01 [medline] PHST- 2022/04/28 00:00 [pmc-release] AID - jcm11092490 [pii] AID - jcm-11-02490 [pii] AID - 10.3390/jcm11092490 [doi] PST - epublish SO - J Clin Med. 2022 Apr 28;11(9):2490. doi: 10.3390/jcm11092490. PMID- 37012951 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230405 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 3 DP - 2023 Mar TI - Progressive Systemic Sclerosis With Negative Antinuclear Antibodies and Absence of Raynaud's Phenomenon: A Case Report and Literature Review. PG - e35663 LID - 10.7759/cureus.35663 [doi] LID - e35663 AB - Systemic sclerosis (SSc) is typically characterized by positive antinuclear antibodies (ANA) and Raynaud's phenomenon (RP). We present the case of a male patient with progressive diffuse skin tightening, interstitial lung disease (ILD), pericardial tamponade, renal failure, and gastrointestinal dysmotility who was diagnosed with severe, rapidly progressive SSc despite negative ANA, absent RP, and a negative malignancy workup. The patient's clinical course was complicated by scleroderma renal crisis (SRC) requiring dialysis and eventual kidney transplantation. He also had severe gastrointestinal dysmotility requiring gastrostomy tube placement and total parenteral nutrition. Multiple agents were required for treatment, including mycophenolate mofetil (MMF) and rituximab. The patient eventually had improvement in his skin fibrosis and has been doing well in follow-up after kidney transplantation. Treatment of SSc can be challenging given the heterogeneity of the disease, and recognition of this subset of SSc patients is needed to help prevent early mortality among them. CI - Copyright © 2023, Falls et al. FAU - Falls, Anna C AU - Falls AC AD - Division of Immunology, Allergy and Rheumatology, University of Cincinnati Medical Center, Cincinnati, USA. FAU - Wrigley, Catherine AU - Wrigley C AD - Department of Rheumatology, Christie Clinic, Champaign, USA. FAU - Khanna, Surabhi A AU - Khanna SA AD - Division of Immunology, Allergy and Rheumatology, University of Cincinnati College of Medicine, Cincinnati, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230301 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10065867 OTO - NOTNLM OT - anti-nuclear antibody OT - interstitial lung disease OT - raynaud’s phenomenon OT - scleroderma renal crisis OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2023/04/05 06:00 MHDA- 2023/04/05 06:01 PMCR- 2023/03/01 CRDT- 2023/04/04 01:57 PHST- 2023/03/01 00:00 [accepted] PHST- 2023/04/05 06:01 [medline] PHST- 2023/04/04 01:57 [entrez] PHST- 2023/04/05 06:00 [pubmed] PHST- 2023/03/01 00:00 [pmc-release] AID - 10.7759/cureus.35663 [doi] PST - epublish SO - Cureus. 2023 Mar 1;15(3):e35663. doi: 10.7759/cureus.35663. eCollection 2023 Mar. PMID- 21217755 OWN - NLM STAT- MEDLINE DCOM- 20110331 LR - 20250529 IS - 1546-1718 (Electronic) IS - 1061-4036 (Print) IS - 1061-4036 (Linking) VI - 43 IP - 2 DP - 2011 Feb TI - Tartrate-resistant acid phosphatase deficiency causes a bone dysplasia with autoimmunity and a type I interferon expression signature. PG - 127-31 LID - 10.1038/ng.748 [doi] AB - We studied ten individuals from eight families showing features consistent with the immuno-osseous dysplasia spondyloenchondrodysplasia. Of particular note was the diverse spectrum of autoimmune phenotypes observed in these individuals (cases), including systemic lupus erythematosus, Sjögren's syndrome, hemolytic anemia, thrombocytopenia, hypothyroidism, inflammatory myositis, Raynaud's disease and vitiligo. Haplotype data indicated the disease gene to be on chromosome 19p13, and linkage analysis yielded a combined multipoint log(10) odds (LOD) score of 3.6. Sequencing of ACP5, encoding tartrate-resistant acid phosphatase, identified biallelic mutations in each of the cases studied, and in vivo testing confirmed a loss of expressed protein. All eight cases assayed showed elevated serum interferon alpha activity, and gene expression profiling in whole blood defined a type I interferon signature. Our findings reveal a previously unrecognized link between tartrate-resistant acid phosphatase activity and interferon metabolism and highlight the importance of type I interferon in the genesis of autoimmunity. FAU - Briggs, Tracy A AU - Briggs TA AD - Manchester Academic Heath Science Centre, University of Manchester, Genetic Medicine, Manchester, UK. FAU - Rice, Gillian I AU - Rice GI FAU - Daly, Sarah AU - Daly S FAU - Urquhart, Jill AU - Urquhart J FAU - Gornall, Hannah AU - Gornall H FAU - Bader-Meunier, Brigitte AU - Bader-Meunier B FAU - Baskar, Kannan AU - Baskar K FAU - Baskar, Shankar AU - Baskar S FAU - Baudouin, Veronique AU - Baudouin V FAU - Beresford, Michael W AU - Beresford MW FAU - Black, Graeme C M AU - Black GC FAU - Dearman, Rebecca J AU - Dearman RJ FAU - de Zegher, Francis AU - de Zegher F FAU - Foster, Emily S AU - Foster ES FAU - Francès, Camille AU - Francès C FAU - Hayman, Alison R AU - Hayman AR FAU - Hilton, Emma AU - Hilton E FAU - Job-Deslandre, Chantal AU - Job-Deslandre C FAU - Kulkarni, Muralidhar L AU - Kulkarni ML FAU - Le Merrer, Martine AU - Le Merrer M FAU - Linglart, Agnes AU - Linglart A FAU - Lovell, Simon C AU - Lovell SC FAU - Maurer, Kathrin AU - Maurer K FAU - Musset, Lucile AU - Musset L FAU - Navarro, Vincent AU - Navarro V FAU - Picard, Capucine AU - Picard C FAU - Puel, Anne AU - Puel A FAU - Rieux-Laucat, Frederic AU - Rieux-Laucat F FAU - Roifman, Chaim M AU - Roifman CM FAU - Scholl-Bürgi, Sabine AU - Scholl-Bürgi S FAU - Smith, Nigel AU - Smith N FAU - Szynkiewicz, Marcin AU - Szynkiewicz M FAU - Wiedeman, Alice AU - Wiedeman A FAU - Wouters, Carine AU - Wouters C FAU - Zeef, Leo A H AU - Zeef LA FAU - Casanova, Jean-Laurent AU - Casanova JL FAU - Elkon, Keith B AU - Elkon KB FAU - Janckila, Anthony AU - Janckila A FAU - Lebon, Pierre AU - Lebon P FAU - Crow, Yanick J AU - Crow YJ LA - eng GR - 084366/WT_/Wellcome Trust/United Kingdom GR - BB/H006818/1/BB_/Biotechnology and Biological Sciences Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110109 PL - United States TA - Nat Genet JT - Nature genetics JID - 9216904 RN - 0 (Interferon Type I) RN - 0 (Isoenzymes) RN - EC 3.1.3.2 (ACP5 protein, human) RN - EC 3.1.3.2 (Acid Phosphatase) RN - EC 3.1.3.2 (Tartrate-Resistant Acid Phosphatase) SB - IM CIN - Nat Genet. 2011 Feb;43(2):90-1. doi: 10.1038/ng0211-90. PMID: 21270835 MH - Acid Phosphatase/*deficiency/*genetics MH - Animals MH - Autoimmunity MH - Bone Diseases, Developmental/enzymology/*genetics/*pathology MH - Cattle MH - Chromosomes, Human, Pair 19 MH - Female MH - *Gene Expression Regulation MH - Humans MH - Inflammation MH - Interferon Type I/*metabolism MH - Isoenzymes/*deficiency/*genetics MH - Lupus Erythematosus, Systemic/metabolism MH - Male MH - Models, Molecular MH - Mutation MH - Mutation, Missense MH - Phenotype MH - Sclerosis/pathology MH - Tartrate-Resistant Acid Phosphatase PMC - PMC3030921 MID - UKMS33798 OID - NLM: UKMS33798 EDAT- 2011/01/11 06:00 MHDA- 2011/04/01 06:00 PMCR- 2011/08/01 CRDT- 2011/01/11 06:00 PHST- 2010/08/19 00:00 [received] PHST- 2010/12/06 00:00 [accepted] PHST- 2011/01/11 06:00 [entrez] PHST- 2011/01/11 06:00 [pubmed] PHST- 2011/04/01 06:00 [medline] PHST- 2011/08/01 00:00 [pmc-release] AID - ng.748 [pii] AID - 10.1038/ng.748 [doi] PST - ppublish SO - Nat Genet. 2011 Feb;43(2):127-31. doi: 10.1038/ng.748. Epub 2011 Jan 9. PMID- 38558753 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240403 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 2 DP - 2024 Feb TI - To Test or Not to Test: A Case Report on Hereditary Hemorrhagic Telangiectasia. PG - e55118 LID - 10.7759/cureus.55118 [doi] LID - e55118 AB - A 50-year-old female patient presenting with joint pains, Raynaud's phenomenon, epistaxis, and telangiectasias was posed with a diagnostic conundrum, i.e., whether to accept the diagnosis of mixed connective tissue disease (MCTD), for which she fulfilled all the criteria, or test for another probable disease, namely hereditary hemorrhagic telangiectasia (HHT), even though only some clinical features were present and all diagnostic criteria were not satisfied. Taking the patient's onset of epistaxis as an important clue, the patient was counseled for genetic testing for HHT, which was positive. Treatment for both MCTD and HHT is underway, and appropriate surveillance is planned for the patient. CI - Copyright © 2024, Chadalavada et al. FAU - Chadalavada, Bhavya AU - Chadalavada B AD - Internal Medicine, Gandhi Medical College, Hyderabad, IND. FAU - Baddam, Ritesh AU - Baddam R AD - Internal Medicine, Gandhi Medical College, Hyderabad, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20240228 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10979317 OTO - NOTNLM OT - autosomal dominant disorder OT - hereditary hemorrhagic telangiectasia (hht) OT - mixed connective tissue disease OT - raynaud’s phenomenon OT - severe epistaxis COIS- The authors have declared that no competing interests exist. EDAT- 2024/04/01 18:42 MHDA- 2024/04/01 18:43 PMCR- 2024/02/28 CRDT- 2024/04/01 17:03 PHST- 2024/02/28 00:00 [accepted] PHST- 2024/04/01 18:43 [medline] PHST- 2024/04/01 18:42 [pubmed] PHST- 2024/04/01 17:03 [entrez] PHST- 2024/02/28 00:00 [pmc-release] AID - 10.7759/cureus.55118 [doi] PST - epublish SO - Cureus. 2024 Feb 28;16(2):e55118. doi: 10.7759/cureus.55118. eCollection 2024 Feb. PMID- 24610647 OWN - NLM STAT- MEDLINE DCOM- 20141102 LR - 20151119 IS - 1644-4345 (Electronic) IS - 1506-9680 (Linking) VI - 17 IP - 1 DP - 2014 TI - The own method and program of isotope quantitatively assessment of perfusion in muscles of upper limbs (initial report). PG - 13-7 LID - 10.5603/NMR.2014.0004 [doi] AB - BACKGROUND: The issue of blood flow in muscles has been dealt with for many years. However, most often it was assessed qualitatively with standard vascular examinations. The quantitatively perfusion assessment is indispensable in the normal and pathological conditions. Some diseases impair the perfusion mainly in the area of upper limbs. It can be observed in Raynaud's disease, vascular occlusive diseases, neurological disturbances, and thermal injuries. Hyperhidrosis of upper limbs after sympathectomy of thoracic part of sympathetic trunk may bring closer the diagnosis statement. Nuclear medicine has the markers and methods that allow for the assessment of the volume of perfusion in muscles. The aim for creating this method and program was the development of radioisotope method allowing for quantitative assessment of perfusion in muscles of upper limbs. This should lead to calculating the perfusion index and its range of normal values in a greater group of patients and to using this method both in the healthy and pathological conditions. MATERIAL AND METHODS: 20 patients, age 30.4 ± 7.1 years, who underwent following examinations: qualification to the group, medical history, subject examinations, USG of upper limb vessels, anthropometric examinations, biochemical and hormonal blood tests, the assessment of upper limbs with USG Doppler and FMD (flow mediated dilatation), radioisotope examinations with gamma camera BrightView XCT by own program RAPUL (Radioisotope Assessment Perfusion of Upper Limb). Acquisitions were started five minutes after intravenous injection of 99mTc-MIBI (metoxyisobutylnitrite). The whole body scintigram and scintigrams of arm and forearm muscles in A-P projections were taken. RESULTS: In the examined patients, the results of anthropometric, biochemical and hormonal test were within the range of normal values. In radioisotope quantitative assessment of perfusion, perfusion indexes of left arm were 20 < PI(_left) < 11.90 and of the right arm 7.00 < PI(_right) <12.30. The dependency correlation PI(_left) vs. PI(_right) was strong r = 0.92036.The values of perfusion symmetry index (IPS) had a normal distribution and were within the following range: 0.9231 < IPS < 1.1019. CONCLUSIONS: The RAPUL method and program allow for quantitative assessment of perfusion in muscles of upper limbs. Developed program has a high repeatability of results. The results in the bigger group will allow for defining the range of normal values of perfusion index in muscles of upper limbs at rest. These will be diagnostically used both in healthy (sports medicine, military services) and pathological conditions. FAU - Niewiadomski, Dariusz AU - Niewiadomski D FAU - Tryniszewski, Wieslaw AU - Tryniszewski W FAU - Marzec, Wojciech AU - Marzec W FAU - Brocki, Marian AU - Brocki M FAU - Mikosiński, Jacek AU - Mikosiński J FAU - Raciborska, Iwona AU - Raciborska I FAU - Maziarz, Zbigniew AU - Maziarz Z AD - Department of Radiological and Isotopic Diagnostics and Therapy, Medical University of Lodz. zbigniew.maziarz@umed.lodz.pl. LA - eng PT - Journal Article PL - Poland TA - Nucl Med Rev Cent East Eur JT - Nuclear medicine review. Central & Eastern Europe JID - 100886103 RN - 971Z4W1S09 (Technetium Tc 99m Sestamibi) SB - IM MH - Adult MH - Arm/*blood supply MH - Female MH - Humans MH - Male MH - Middle Aged MH - Muscles/*blood supply MH - Perfusion Imaging/*methods MH - *Regional Blood Flow MH - Technetium Tc 99m Sestamibi MH - Young Adult EDAT- 2014/03/13 06:00 MHDA- 2014/11/05 06:00 CRDT- 2014/03/11 06:00 PHST- 2013/12/09 00:00 [received] PHST- 2014/01/03 00:00 [accepted] PHST- 2013/12/22 00:00 [revised] PHST- 2014/03/11 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2014/11/05 06:00 [medline] AID - VM/OJS/J/36454 [pii] AID - 10.5603/NMR.2014.0004 [doi] PST - ppublish SO - Nucl Med Rev Cent East Eur. 2014;17(1):13-7. doi: 10.5603/NMR.2014.0004. PMID- 27558461 OWN - NLM STAT- MEDLINE DCOM- 20181008 LR - 20181008 IS - 1545-1550 (Electronic) IS - 1526-6028 (Linking) VI - 23 IP - 6 DP - 2016 Dec TI - Comparison of Transradial vs Transfemoral Access for Aortoiliac and Femoropopliteal Interventions: A Single-Center Experience. PG - 880-888 AB - PURPOSE: To compare the procedure and safety outcomes of the transradial approach (TRA) with the femoral approach (FA) for treating aortoiliac and femoropopliteal stenoses and occlusions. METHODS: A single-center retrospective study was conducted involving 188 patients (mean age 66.4±10.8 years; 116 men) with lower limb claudication or critical limb ischemia who underwent aortoiliac (131, 62.4%) or femoropopliteal (79, 37.6%) interventions on 210 lesions over a 3-year period. Operator discretion determined TRA suitability; exclusions included Raynaud's disease, upper limb occlusive disease, previous TRA difficulties, or planned hemodialysis. Lesion characteristics, clinical endpoints, and access site complications were compared. RESULTS: FA was used primarily in 123 patients and the TRA (12 left and 53 right radial arteries) in 65 procedures. Eleven (16.9%) TRAs failed vs 9 (7.3%) FAs (p=0.42). Crossover to FA was due to occlusive lesions requiring alternative equipment in 9 cases and to tortuosity of the aortic arch vessels in 2 patients. The 134 FA interventions (balloon angioplasty, stents) were retrograde (112, 83.6%) or antegrade (22, 16.4%). There were significantly more TASC C/D lesions in the FA group (p=0.02). Sheath sizes (5-F to 8-F) did not differ between groups, and no significant differences were found between FA vs TRA in terms of procedure time (50.0±28.9 vs 46.8±25.1 minutes, p=0.50) or length of stay (2.2±0.6 vs 2.1±0.3 days, p=0.24). While there were no strokes, access site complications occurred in 6.0% of the FA patients vs 3.7% of the TRA patients (p=0.12). CONCLUSION: The transradial approach for aortoiliac and femoropopliteal interventions is safe and efficacious compared with the transfemoral approach for a range of lesion subtypes. Nevertheless, there remains a need for improvements in peripheral device and catheter technology to decrease transradial failure rates. CI - © The Author(s) 2016. FAU - Roy, Andrew K AU - Roy AK AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Garot, Phillipe AU - Garot P AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Louvard, Yves AU - Louvard Y AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Neylon, Antoinette AU - Neylon A AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Spaziano, Marco AU - Spaziano M AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Sawaya, Fadi J AU - Sawaya FJ AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Fernandez, Leticia AU - Fernandez L AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Roux, Yann AU - Roux Y AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Blanc, Raphael AU - Blanc R AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. AD - Department of Interventional Radiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Piotin, Michel AU - Piotin M AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. AD - Department of Interventional Radiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Champagne, Stephane AU - Champagne S AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Tavolaro, Oscar AU - Tavolaro O AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Benamer, Hakim AU - Benamer H AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. FAU - Hovasse, Thomas AU - Hovasse T AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Chevalier, Bernard AU - Chevalier B AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Lefèvre, Thierry AU - Lefèvre T AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. FAU - Unterseeh, Thierry AU - Unterseeh T AD - Department of Cardiology, Ramsay Générale de Santé, Institut Cardiovasculaire Paris-Sud, Hôpital Privé Jacques Cartier, Massy, France t.unterseeh@angio-icps.com. AD - Hôpital Claude-Galien, Quincy Sous-Sénart, France. LA - eng PT - Journal Article DEP - 20160824 PL - United States TA - J Endovasc Ther JT - Journal of endovascular therapy : an official journal of the International Society of Endovascular Specialists JID - 100896915 SB - IM MH - Aged MH - *Angioplasty, Balloon MH - Constriction, Pathologic/*surgery MH - Femoral Artery/surgery MH - Humans MH - Male MH - Middle Aged MH - Popliteal Artery/*surgery MH - Retrospective Studies MH - *Stents MH - Treatment Outcome OTO - NOTNLM OT - angioplasty OT - aortoiliac bifurcation OT - endovascular intervention OT - femoral artery access OT - iliac artery OT - occlusion OT - peripheral artery disease OT - popliteal artery OT - radial artery access OT - stenosis OT - stent OT - superficial femoral artery EDAT- 2016/08/26 06:00 MHDA- 2018/10/09 06:00 CRDT- 2016/08/26 06:00 PHST- 2016/08/26 06:00 [pubmed] PHST- 2018/10/09 06:00 [medline] PHST- 2016/08/26 06:00 [entrez] AID - 1526602816665617 [pii] AID - 10.1177/1526602816665617 [doi] PST - ppublish SO - J Endovasc Ther. 2016 Dec;23(6):880-888. doi: 10.1177/1526602816665617. Epub 2016 Aug 24. PMID- 22121371 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20211021 IS - 1687-9279 (Electronic) IS - 1687-9260 (Print) IS - 1687-9260 (Linking) VI - 2011 DP - 2011 TI - Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis. PG - 201787 LID - 10.1155/2011/201787 [doi] LID - 201787 AB - Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis. FAU - Arefiev, Kait AU - Arefiev K AD - Division of Immunology and Rheumatology, Departments of Dermatology and Medicine, Stanford University School of Medicine, Palo Alto VA Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94304, USA. FAU - Fiorentino, David F AU - Fiorentino DF FAU - Chung, Lorinda AU - Chung L LA - eng PT - Journal Article DEP - 20111027 PL - United States TA - Int J Rheumatol JT - International journal of rheumatology JID - 101519204 PMC - PMC3205679 EDAT- 2011/11/29 06:00 MHDA- 2011/11/29 06:01 PMCR- 2011/10/27 CRDT- 2011/11/29 06:00 PHST- 2011/06/02 00:00 [received] PHST- 2011/08/17 00:00 [accepted] PHST- 2011/11/29 06:00 [entrez] PHST- 2011/11/29 06:00 [pubmed] PHST- 2011/11/29 06:01 [medline] PHST- 2011/10/27 00:00 [pmc-release] AID - 10.1155/2011/201787 [doi] PST - ppublish SO - Int J Rheumatol. 2011;2011:201787. doi: 10.1155/2011/201787. Epub 2011 Oct 27. PMID- 36460440 OWN - NLM STAT- MEDLINE DCOM- 20221206 LR - 20221230 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 43 IP - 12 DP - 2022 Dec TI - [An alarming necrosis]. PG - 752-754 LID - S0248-8663(22)00577-X [pii] LID - 10.1016/j.revmed.2022.07.012 [doi] FAU - Deroux, A AU - Deroux A AD - Service de médecine interne et polyvalente, CHU Grenoble-Alpes, 38000 Grenoble, France. Electronic address: aderoux@chu-grenoble.fr. FAU - Madelon, A AU - Madelon A AD - Service de médecine interne et polyvalente, CH de Briançon, 05100 Briançon, France. FAU - Colombe, B AU - Colombe B AD - Service de médecine interne et polyvalente, CHU Grenoble-Alpes, 38000 Grenoble, France. FAU - Lugosi, M AU - Lugosi M AD - Service de médecine interne et polyvalente, CHU Grenoble-Alpes, 38000 Grenoble, France. FAU - Bouillet, L AU - Bouillet L AD - Service de médecine interne et polyvalente, CHU Grenoble-Alpes, 38000 Grenoble, France; Université Grenoble-Alpes, 38000 Grenoble, France. LA - fre PT - Letter TT - Une nécrose inquiétante. DEP - 20221110 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Humans MH - *Necrosis OTO - NOTNLM OT - Buerger's disease OT - Digital ulcers OT - Maladie de Buerger OT - Raynaud's phenomenon OT - Syndrome de Raynaud OT - Ulcères digitaux EDAT- 2022/12/03 06:00 MHDA- 2022/12/07 06:00 CRDT- 2022/12/02 21:08 PHST- 2022/04/21 00:00 [received] PHST- 2022/05/27 00:00 [revised] PHST- 2022/07/12 00:00 [accepted] PHST- 2022/12/02 21:08 [entrez] PHST- 2022/12/03 06:00 [pubmed] PHST- 2022/12/07 06:00 [medline] AID - S0248-8663(22)00577-X [pii] AID - 10.1016/j.revmed.2022.07.012 [doi] PST - ppublish SO - Rev Med Interne. 2022 Dec;43(12):752-754. doi: 10.1016/j.revmed.2022.07.012. Epub 2022 Nov 10. PMID- 37809341 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231030 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 10 DP - 2023 TI - Editorial: Update in microcirculation in dermatology. PG - 1285005 LID - 10.3389/fmed.2023.1285005 [doi] LID - 1285005 FAU - Trevisan, Giusto AU - Trevisan G AD - Department of Medical Sciences, University of Trieste, Trieste, Italy. FAU - Bilancini, Salvino AU - Bilancini S AD - Research Center of Vascular Disease, Jean François Merlen, Frosinone, Italy. LA - eng PT - Editorial DEP - 20230921 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 CON - Editorial on the Research Topic Update in microcirculation in dermatology PMC - PMC10558206 OTO - NOTNLM OT - Nailfold Capillaroscopy OT - Raynaud's OT - connective tissue disease OT - microangiopathies OT - microcirculation COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. EDAT- 2023/10/09 06:41 MHDA- 2023/10/09 06:42 PMCR- 2023/09/21 CRDT- 2023/10/09 05:54 PHST- 2023/08/29 00:00 [received] PHST- 2023/09/04 00:00 [accepted] PHST- 2023/10/09 06:42 [medline] PHST- 2023/10/09 06:41 [pubmed] PHST- 2023/10/09 05:54 [entrez] PHST- 2023/09/21 00:00 [pmc-release] AID - 10.3389/fmed.2023.1285005 [doi] PST - epublish SO - Front Med (Lausanne). 2023 Sep 21;10:1285005. doi: 10.3389/fmed.2023.1285005. eCollection 2023. PMID- 41989281 OWN - NLM STAT- Publisher LR - 20260416 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) DP - 2026 Apr 16 TI - Vasospastic and Ischemic Disorders of the Upper Extremity: Vascular Radiology Collaborations Guiding Raynaud's Treatment Strategies. LID - 10.1097/PRS.0000000000013126 [doi] AB - BACKGROUND: Raynaud's phenomenon (RP) encompasses vasospastic disorders that can progress to digital ischemia, ulceration, and tissue loss. Despite multiple therapeutic options, variability in diagnostic evaluation and procedural indications can delay appropriate treatment. This review summarizes current evidence on RP and presents a structured diagnostic pathway and treatment algorithm for patients with vasospastic disease and digital ischemia. METHODS: A review of contemporary literature on the epidemiology, pathophysiology, diagnostic studies and therapeutic interventions for RP was collected. Additionally, we describe our institution's algorithm which integrates noninvasive vascular diagnostics, catheter angiography with vasodilation challenges and a stepwise approach to procedure selection based on patterns of vasospasm and occlusion. RESULTS: Primary RP is a vasospastic disorder whereas secondary RP involves vasospasm and fixed occlusive disease. Noninvasive studies help assess wound healing potential and abnormal results will lead to an interventional radiology consultation for catheter angiography. Angiography defines the level of disease, responsiveness to vasodilators and suitability for intervention. Interventions include chemical and mechanical sympathectomy which is most suitable in patients with vasospastic disease and less effective in secondary RP. Patients with segmental occlusions and patent distal targets or with secondary RP will benefit from open arterial bypass or arterial reconstruction. Patients with disease isolated to the proper digital arteries are not amenable to targeted surgical interventions due to the risk of worsening ischemia. CONCLUSIONS: A structured, physiology-guided diagnostic and treatment algorithm can improve the precision of RP management. Integrating noninvasive testing with angiography allows tailored use of sympathectomy and reconstruction in these patients. CI - Copyright © 2026 by the American Society of Plastic Surgeons. FAU - Le, Elliot L H AU - Le ELH AD - . Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Jabbari, Kayvon AU - Jabbari K AD - . Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Reyes, Daniel AU - Reyes D AD - . Division of Vascular and Interventional Radiology, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Allenby, Taylor H AU - Allenby TH AD - . Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Fisher, Marlie H AU - Fisher MH AD - . Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Greyson, Mark A AU - Greyson MA AD - . Division of Plastic and Reconstructive Surgery, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Walker, Lisa K AU - Walker LK AD - . Division of Vascular and Interventional Radiology, University of Colorado School of Medicine, Anschutz Medical Center, Aurora, CO. FAU - Iorio, Matthew L AU - Iorio ML AD - . Peak Rejuvenation, Durango, CO. LA - eng PT - Journal Article DEP - 20260416 PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 SB - IM OTO - NOTNLM OT - Raynaud’s OT - angiography OT - digital ischemia OT - sympathectomy EDAT- 2026/04/17 13:25 MHDA- 2026/04/17 13:25 CRDT- 2026/04/16 09:53 PHST- 2026/04/17 13:25 [medline] PHST- 2026/04/17 13:25 [pubmed] PHST- 2026/04/16 09:53 [entrez] AID - 00006534-990000000-03222 [pii] AID - 10.1097/PRS.0000000000013126 [doi] PST - aheadofprint SO - Plast Reconstr Surg. 2026 Apr 16. doi: 10.1097/PRS.0000000000013126. PMID- 27407233 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160713 LR - 20181113 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 53 IP - 2 DP - 2015 TI - Raynaud's phenomenon: new aspects of pathogenesis and the role of nailfold videocapillaroscopy. PG - 87-93 LID - 10.5114/reum.2015.51508 [doi] AB - Raynaud's phenomenon (RP) refers to paroxysmal pallor or cyanosis of the digits of the hands or feet and, infrequently, the tips of the nose or ears (acral parts) owing to cold-induced vasoconstriction of the digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts. Raynaud's phenomenon reflects an exaggeration of normal central and local vasomotor responses to cold or emotion. Raynaud's phenomenon has been classified as primary or secondary, depending on whether it occurs as an isolated condition or is associated mainly with a connective tissue disease. Dysregulation of autonomic and sensitive nerve fibers, functional and structural vessel changes, and intravascular alterations can be observed in the pathogenesis of RP. Nailfold videocapillaroscopy (NVC) is the best non-invasive and repetitive diagnostic technique for detecting morpho-functional changes in the microcirculation. Nailfold videocapillaroscopy is accepted in early diagnosis and monitoring of primary and secondary RP. FAU - Kuryliszyn-Moskal, Anna AU - Kuryliszyn-Moskal A AD - Department of Rehabilitation, Medical University of Bialystok, Bialystok, Poland. FAU - Kita, Jacek AU - Kita J AD - Department of Rehabilitation, Medical University of Bialystok, Bialystok, Poland. FAU - Hryniewicz, Anna AU - Hryniewicz A AD - Department of Rehabilitation, Medical University of Bialystok, Bialystok, Poland. LA - eng PT - Journal Article PT - Review DEP - 20150518 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC4847279 OTO - NOTNLM OT - Raynaud's phenomenon OT - microvascular abnormalities OT - nailfold videocapillaroscopy EDAT- 2015/01/01 00:00 MHDA- 2015/01/01 00:01 PMCR- 2015/01/01 CRDT- 2016/07/14 06:00 PHST- 2015/03/16 00:00 [received] PHST- 2015/05/04 00:00 [accepted] PHST- 2016/07/14 06:00 [entrez] PHST- 2015/01/01 00:00 [pubmed] PHST- 2015/01/01 00:01 [medline] PHST- 2015/01/01 00:00 [pmc-release] AID - 51508 [pii] AID - 10.5114/reum.2015.51508 [doi] PST - ppublish SO - Reumatologia. 2015;53(2):87-93. doi: 10.5114/reum.2015.51508. Epub 2015 May 18. PMID- 27899893 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 1663-9812 (Print) IS - 1663-9812 (Electronic) IS - 1663-9812 (Linking) VI - 7 DP - 2016 TI - Raynaud's Phenomenon: A Brief Review of the Underlying Mechanisms. PG - 438 LID - 438 AB - Raynaud's phenomenon (RP) is characterized by exaggerated cold-induced vasoconstriction. This augmented vasoconstriction occurs by virtue of a reflex response to cooling via the sympathetic nervous system as well as by local activation of α(2C) adrenoceptors (α(2C)-AR). In a cold-initiated, mitochondrion-mediated mechanism involving reactive oxygen species and the Rho/ROCK pathway, cytoskeletal rearrangement in vascular smooth muscle cells orchestrates the translocation of α(2C)-AR to the cell membrane, where this receptor readily interacts with its ligand. Different parameters are involved in this spatial and functional rescue of α(2C)-AR. Of notable relevance is the female hormone, 17β-estradiol, or estrogen. This is consistent with the high prevalence of RP in premenopausal women compared to age-matched males. In addition to dissecting the role of these various players, the contribution of pollution as well as genetic background to the onset and prevalence of RP are also discussed. Different therapeutic approaches employed as treatment modalities for this disease are also highlighted and analyzed. The lack of an appropriate animal model for RP mandates that more efforts be undertaken in order to better understand and eventually treat this disease. Although several lines of treatment are utilized, it is important to note that precaution is often effective in reducing severity or frequency of RP attacks. FAU - Fardoun, Manal M AU - Fardoun MM AD - Department of Biology, Faculty of Arts and Sciences, American University of Beirut Beirut, Lebanon. FAU - Nassif, Joseph AU - Nassif J AD - Department of Obstetrics and Gynecology, Faculty of Medicine, American University of Beirut Beirut, Lebanon. FAU - Issa, Khodr AU - Issa K AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut Beirut, Lebanon. FAU - Baydoun, Elias AU - Baydoun E AD - Department of Biology, Faculty of Arts and Sciences, American University of Beirut Beirut, Lebanon. FAU - Eid, Ali H AU - Eid AH AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut Beirut, Lebanon. LA - eng PT - Journal Article PT - Review DEP - 20161116 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC5110514 OTO - NOTNLM OT - Raynaud’s Phenomenon OT - Rho kinase OT - alpha 2-adrenergic receptors OT - estrogen OT - peripheral vascular disease OT - thermoregulation EDAT- 2016/12/03 06:00 MHDA- 2016/12/03 06:01 PMCR- 2016/11/16 CRDT- 2016/12/01 06:00 PHST- 2016/08/25 00:00 [received] PHST- 2016/11/03 00:00 [accepted] PHST- 2016/12/01 06:00 [entrez] PHST- 2016/12/03 06:00 [pubmed] PHST- 2016/12/03 06:01 [medline] PHST- 2016/11/16 00:00 [pmc-release] AID - 10.3389/fphar.2016.00438 [doi] PST - epublish SO - Front Pharmacol. 2016 Nov 16;7:438. doi: 10.3389/fphar.2016.00438. eCollection 2016. PMID- 34924902 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211221 IS - 1611-2156 (Print) IS - 1611-2156 (Electronic) IS - 1611-2156 (Linking) VI - 20 DP - 2021 TI - Raynaud's phenomenon in a drummer player: Microvascular disorder and nailfold video capillaroscopic findings. PG - 1526-1531 LID - 10.17179/excli2021-4208 [doi] AB - Drummers are usually exposed to intensive physical stress and occupational diseases that have been only partially investigated. The majority of studies focus on musculoskeletal problems while microvascular abnormalities have been less considered. We report on a case of a 19-year-old drummer affected by Raynaud's phenomenon. The patient underwent nailfold video capillaroscopy that showed a non-specific pattern, with granular flow, dyshomogeneous capillary morphology, increased efferent/afferent loop ratio and many enlarged capillaries. The continuous exposition to vibration in drummers could determinate microvascular abnormalities with related cold induced disorders and Raynaud's phenomenon. Nailfold video capillaroscopy is a tool that allows to detect the alterations of the microcirculation and to carry out the follow-up of the patients with low cost, non-invasiveness, repeatability, high sensibility and specificity. CI - Copyright © 2021 Sirufo et al. FAU - Sirufo, Maria Maddalena AU - Sirufo MM AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04, 64100 Teramo, Italy. FAU - Catalogna, Alessandra AU - Catalogna A AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04, 64100 Teramo, Italy. FAU - De Pietro, Francesca AU - De Pietro F AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04, 64100 Teramo, Italy. FAU - Ginaldi, Lia AU - Ginaldi L AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04, 64100 Teramo, Italy. FAU - De Martinis, Massimo AU - De Martinis M AUID- ORCID: 0000-0003-4253-1312 AD - Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy. AD - Allergy and Clinical Immunology Unit, Center for the Diagnosis and Treatment of Osteoporosis, AUSL 04, 64100 Teramo, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20211028 PL - Germany TA - EXCLI J JT - EXCLI journal JID - 101299402 PMC - PMC8678061 OTO - NOTNLM OT - Raynaud's phenomenon OT - arts medicine OT - drummer OT - hand-arm vibration syndrome OT - microcirculation OT - musician OT - nailfold video capillaroscopy OT - occupational disease EDAT- 2021/12/21 06:00 MHDA- 2021/12/21 06:01 PMCR- 2021/10/28 CRDT- 2021/12/20 06:03 PHST- 2021/08/17 00:00 [received] PHST- 2021/10/21 00:00 [accepted] PHST- 2021/12/20 06:03 [entrez] PHST- 2021/12/21 06:00 [pubmed] PHST- 2021/12/21 06:01 [medline] PHST- 2021/10/28 00:00 [pmc-release] AID - 2021-4208 [pii] AID - Doc1526 [pii] AID - 10.17179/excli2021-4208 [doi] PST - epublish SO - EXCLI J. 2021 Oct 28;20:1526-1531. doi: 10.17179/excli2021-4208. eCollection 2021. PMID- 36186832 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230413 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 60 IP - 4 DP - 2022 TI - Association between systemic sclerosis, palmar fasciitis with polyarthritis, Raynaud's phenomenon and erythromelalgia with underlying malignancy. PG - 275-280 LID - 10.5114/reum.2022.119044 [doi] AB - The symptoms of a rheumatic disease may also be a sign of a proliferative process. These include conditions that present with skin and vascular changes such as systemic sclerosis and Raynaud's phenomenon with peripheral ischaemia and ulceration. Furthermore, the less common conditions - erythromelalgia or palmar fasciitis with polyarthritis may also accompany cancer. In this article, we discuss the association of diffuse systemic sclerosis with anorectal tumor, palmar fasciitis and polyarthritis with ovarian cancer, erythromelalgia with underlying ovarian malignancy and Raynaud's phenomenon and digital ischemia associated with renal carcinoma. Based on the literature review on this topic we highlighted the importance of recognizing paraneoplastic syndromes at an early stage. This is a crucial point in the adequate management of a patient. Many of the paraneoplastic symptoms of rheumatic and other described conditions may regress with the management of the underlying malignancy. CI - Copyright: © 2022 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. FAU - Nowak, Katarzyna AU - Nowak K AD - Musgrave Park Hospital, Belfast, United Kingdom. FAU - Wright, Gary AU - Wright G AD - Musgrave Park Hospital, Belfast, United Kingdom. LA - eng PT - Case Reports PT - Journal Article DEP - 20220908 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC9494792 OTO - NOTNLM OT - Raynaud’s syndrome OT - anti-RNA polymerase III antibody OT - erythromelalgia OT - palmar fibromatosis OT - paraneoplastic syndrome OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2022/10/04 06:00 MHDA- 2022/10/04 06:01 PMCR- 2022/01/01 CRDT- 2022/10/03 04:40 PHST- 2022/06/09 00:00 [received] PHST- 2022/08/17 00:00 [accepted] PHST- 2022/10/03 04:40 [entrez] PHST- 2022/10/04 06:00 [pubmed] PHST- 2022/10/04 06:01 [medline] PHST- 2022/01/01 00:00 [pmc-release] AID - 47708 [pii] AID - 10.5114/reum.2022.119044 [doi] PST - ppublish SO - Reumatologia. 2022;60(4):275-280. doi: 10.5114/reum.2022.119044. Epub 2022 Sep 8. PMID- 33015303 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201006 IS - 2352-6475 (Print) IS - 2352-6475 (Electronic) IS - 2352-6475 (Linking) VI - 6 IP - 4 DP - 2020 Sep TI - Dermoscopy of onychodystrophy in a patient with connective tissue disease. PG - 342-343 LID - 10.1016/j.ijwd.2020.05.007 [doi] FAU - Stewart, Claire AU - Stewart C AD - Shari Lipner to Weill Cornell Medicine, New York, NY, United States. FAU - Lipner, Shari R AU - Lipner SR AD - Department of Dermatology, Weill Cornell Medical College, New York, NY, United States. LA - eng PT - Journal Article PT - Review DEP - 20200518 PL - United States TA - Int J Womens Dermatol JT - International journal of women's dermatology JID - 101654170 PMC - PMC7522860 OTO - NOTNLM OT - Dermoscopy OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Ventral pterygium EDAT- 2020/10/06 06:00 MHDA- 2020/10/06 06:01 PMCR- 2020/05/18 CRDT- 2020/10/05 06:22 PHST- 2020/03/28 00:00 [received] PHST- 2020/05/12 00:00 [revised] PHST- 2020/05/13 00:00 [accepted] PHST- 2020/10/05 06:22 [entrez] PHST- 2020/10/06 06:00 [pubmed] PHST- 2020/10/06 06:01 [medline] PHST- 2020/05/18 00:00 [pmc-release] AID - S2352-6475(20)30090-3 [pii] AID - 10.1016/j.ijwd.2020.05.007 [doi] PST - epublish SO - Int J Womens Dermatol. 2020 May 18;6(4):342-343. doi: 10.1016/j.ijwd.2020.05.007. eCollection 2020 Sep. PMID- 24082203 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20131001 LR - 20211021 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 58 IP - 5 DP - 2013 Sep TI - Progressive systemic sclerosis in a child. PG - 406 LID - 10.4103/0019-5154.117333 [doi] AB - Systemic sclerosis is a clinically heterogeneous systemic disease affecting the connective tissues of skin, walls of blood vessels and internal organs like lung, heart and kidneys. Systemic sclerosis is very unusual in pediatric population. Children represent fewer than 10% of all cases. We report a case of 11 years old girl of progressive systemic sclerosis presenting with features of cutaneous sclerosis, microstomia, mask-like facies, sclerodactyly, esophageal dysmotility, Raynaud's phenomenon, arthralgia and pulmonary fibrosis. FAU - De, Arun K AU - De AK AD - Department of Pediatric Medicine, Medical College, Kolkata, India. FAU - Das, Kallol AU - Das K FAU - Sil, Archan AU - Sil A FAU - Joardar, Swarnali AU - Joardar S LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3778798 OTO - NOTNLM OT - Microstomia OT - Raynaud's phenomenon OT - progressive systemic sclerosis OT - pulmonary fibrosis OT - sclerodactyly COIS- Conflict of Interest: Nil. EDAT- 2013/10/02 06:00 MHDA- 2013/10/02 06:01 PMCR- 2013/09/01 CRDT- 2013/10/02 06:00 PHST- 2013/10/02 06:00 [entrez] PHST- 2013/10/02 06:00 [pubmed] PHST- 2013/10/02 06:01 [medline] PHST- 2013/09/01 00:00 [pmc-release] AID - IJD-58-406f [pii] AID - 10.4103/0019-5154.117333 [doi] PST - ppublish SO - Indian J Dermatol. 2013 Sep;58(5):406. doi: 10.4103/0019-5154.117333. PMID- 40786022 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250811 IS - 1535-2188 (Print) IS - 1536-0067 (Electronic) IS - 1535-2188 (Linking) VI - 39 IP - 3 DP - 2025 Aug TI - Advancements in Managing Intractable Raynaud Phenomenon: The Role of Integrated Neurectomy and Sympathectomy. PG - 170-174 LID - 10.1055/s-0045-1809371 [doi] AB - Raynaud's phenomenon (RP) is characterized by digit discoloration including pallor, cyanosis, and rubor. While some experience only mild numbness or tingling during RP attacks, many progress to severe conditions, including intractable pain, nonhealing digital ulcers, and even self-amputation. Although mild symptoms can often be managed conservatively through lifestyle modifications, such as avoiding triggering factors, or with pharmacological interventions, surgical treatments are often required for those with persistent pain and ulcers. Advances in microsurgical techniques have introduced peripheral sympathectomy, peripheral neurectomy, and distal artery bypass as promising options for managing refractory and severe cases of RP. This review explores the epidemiology, pathophysiology, and particularly the evolution of microsurgical interventions for the treatment of RP. CI - Thieme. All rights reserved. FAU - Lien, Po-Hao AU - Lien PH AD - Department of Plastic and Reconstructive Surgery, Chang-Gung Memorial Hospital, Taoyuan, Taiwan. FAU - Chen, Shih-Heng AU - Chen SH AD - Department of Plastic and Reconstructive Surgery, Chang-Gung Memorial Hospital, Taoyuan, Taiwan. LA - eng PT - Journal Article PT - Review DEP - 20250613 PL - United States TA - Semin Plast Surg JT - Seminars in plastic surgery JID - 101131275 PMC - PMC12334259 OTO - NOTNLM OT - Raynaud's phenomenon OT - arterial bypass OT - connective tissue disease OT - microsurgical intervention OT - sympathectomy COIS- Conflict of Interest None declared. EDAT- 2025/08/11 12:32 MHDA- 2025/08/11 12:33 PMCR- 2026/06/13 CRDT- 2025/08/11 06:12 PHST- 2026/06/13 00:00 [pmc-release] PHST- 2025/08/11 12:33 [medline] PHST- 2025/08/11 12:32 [pubmed] PHST- 2025/08/11 06:12 [entrez] AID - sps01471 [pii] AID - 10.1055/s-0045-1809371 [doi] PST - epublish SO - Semin Plast Surg. 2025 Jun 13;39(3):170-174. doi: 10.1055/s-0045-1809371. eCollection 2025 Aug. PMID- 35382391 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 4 IP - 2 DP - 2019 Jun TI - Raynaud's phenomenon. PG - 89-101 LID - 10.1177/2397198319826467 [doi] AB - Raynaud's phenomenon can be either primary (idiopathic) or secondary to underlying disease including systemic sclerosis. Primary Raynaud's phenomenon is very common, affecting approximately 3%-5% of the general population. Although much rarer, systemic sclerosis-related Raynaud's phenomenon can be particularly severe, progressing to digital ulceration in approximately 50% of patients. Raynaud's phenomenon can have a major impact on quality of life. This review has a focus on the systemic sclerosis-related Raynaud's phenomenon (which is the most researched form of Raynaud's phenomenon and probably the most challenging to treat) and on recent advances. Epidemiology (including transition from 'isolated' to systemic sclerosis-related Raynaud's phenomenon), pathogenesis, diagnosis and assessment are discussed, followed by the treatment of both 'uncomplicated' and 'complicated' Raynaud's phenomena (i.e. Raynaud's phenomenon which has progressed to digital ulceration and/or critical ischaemia). Finally, some of the major challenges for the next 5-10 years are highlighted. CI - © The Author(s) 2019. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Biomedical Research Centre, Manchester, UK. LA - eng PT - Journal Article PT - Review DEP - 20190213 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922643 OTO - NOTNLM OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - assessment OT - diagnosis OT - management COIS- Declaration of conflicting interests: A.L.H. received research funding from Actelion. EDAT- 2019/06/01 00:00 MHDA- 2019/06/01 00:01 PMCR- 2020/06/01 CRDT- 2022/04/06 05:15 PHST- 2018/09/30 00:00 [received] PHST- 2018/11/27 00:00 [accepted] PHST- 2022/04/06 05:15 [entrez] PHST- 2019/06/01 00:00 [pubmed] PHST- 2019/06/01 00:01 [medline] PHST- 2020/06/01 00:00 [pmc-release] AID - 10.1177_2397198319826467 [pii] AID - 10.1177/2397198319826467 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2019 Jun;4(2):89-101. doi: 10.1177/2397198319826467. Epub 2019 Feb 13. PMID- 24130596 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20131016 LR - 20211021 IS - 1735-3327 (Print) IS - 2008-0255 (Electronic) IS - 1735-3327 (Linking) VI - 10 IP - 4 DP - 2013 Jul TI - A rare case of hidebound disease with dental implications. PG - 556-61 AB - Systemic sclerosis (also called as Scleroderma or hidebound disease) is a chronic sclerotic disease of unknown etiology which causes diffuse, increased deposition of extra cellular matrix in connective tissue with vascular abnormalities, resulting in tissue hypoxia. The disease is characterized by diffuse fibrosis; degenerative changes; and vascular abnormalities in the skin (scleroderma), articular structures, and internal organs. Aesthetic and facial dysfunctions are followed by important oral and facial manifestations. Most oral manifestations begin with tongue rigidity and facial skin changes. Bone resorption of mandibular angle and widening of periodontal ligament space on periapical radiographs are important radiological findings. Other systemic changes include the involvement of internal organs, which lead to serious complications as well as disorders in the cardiac muscle and Raynaud΄s phenomenon. This is a case report of 30-year-old female patient with the classical features of this disease. This case is reported for its rarity and variable expressivity. The main aim of this article is to describe thorough presentation of the case report, various forms of scleroderma, pathogenesis, oral, extraoral, periodontal manifestations of scleroderma, and its treatment options. A brief review of the literature, focusing on dental alterations is also presented. FAU - Bali, Vikram AU - Bali V AD - Department of Periodontics, Christian Dental College, Ludhiana, India. FAU - Dabra, Sarita AU - Dabra S FAU - Behl, Ashima Bali AU - Behl AB FAU - Bali, Rajiv AU - Bali R LA - eng PT - Case Reports PT - Journal Article PL - Iran TA - Dent Res J (Isfahan) JT - Dental research journal JID - 101471186 PMC - PMC3793424 OTO - NOTNLM OT - Acroosteolysis OT - Raynaud's phenomenon OT - connective tissue OT - crest syndrome OT - hidebound disease OT - periodontitis OT - scleroderma COIS- Conflict of Interest: None declared. EDAT- 2013/10/17 06:00 MHDA- 2013/10/17 06:01 PMCR- 2013/07/01 CRDT- 2013/10/17 06:00 PHST- 2013/10/17 06:00 [entrez] PHST- 2013/10/17 06:00 [pubmed] PHST- 2013/10/17 06:01 [medline] PHST- 2013/07/01 00:00 [pmc-release] AID - DRJ-10-556 [pii] PST - ppublish SO - Dent Res J (Isfahan). 2013 Jul;10(4):556-61. PMID- 32185363 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 30 IP - 3 DP - 2019 Sep TI - Interstitial Lung Disease in Anti-Synthetase Syndrome. PG - 186-189 LID - 10.31138/mjr.30.3.186 [doi] AB - Anti-synthetase syndrome is an autoimmune disorder characterized by the presence of autoantibodies against aminoacyl transfer RNA (tRNA) synthetases, and myositis, interstitial lung disease (ILD), arthritis, fever and Raynaud's phenomenon (RP). We present a 54-year-old woman, who complained of fatigue, low-grade fever, myalgias, arthralgias, RP and dyspnoea on exertion. Chest CT scan revealed features of interstitial lung disease. Due to rapid deterioration of her lung function, she required oxygen support. The patient did not respond to empiric treatment with antibiotics. Autoantibody testing was remarkable for ANA positivity (1/160) and high-titre anti-Jo1 positivity. A diagnosis of anti-synthetase syndrome was made and the patient was placed on high-dose corticosteroids and rituximab with significant improvement. At 1-year follow up, she remains in good condition, without the need for oxygen supplementation. CI - © 2019 The Mediterranean Journal of Rheumatology (MJR). FAU - Kourkouni, Evangelia AU - Kourkouni E AD - Department of Rheumatology and Clinical Immunology. FAU - Mitsogiannis, Georgios AU - Mitsogiannis G AD - Department of Rheumatology and Clinical Immunology. FAU - Simopoulou, Theodora AU - Simopoulou T AD - Department of Rheumatology and Clinical Immunology. FAU - Liaskos, Christos AU - Liaskos C AD - Department of Rheumatology and Clinical Immunology. FAU - Katsiari, Christina G AU - Katsiari CG AD - Department of Rheumatology and Clinical Immunology. FAU - Daniil, Zoi AU - Daniil Z AD - Department of Respiratory Medicine, University General Hospital of Larissa, Faculty of Medicine, University of Thessaly, Larissa, Greece. FAU - Gourgoulianis, Konstantinos AU - Gourgoulianis K AD - Department of Respiratory Medicine, University General Hospital of Larissa, Faculty of Medicine, University of Thessaly, Larissa, Greece. FAU - Bogdanos, Dimitrios P AU - Bogdanos DP AD - Department of Rheumatology and Clinical Immunology. FAU - Sakkas, Lazaros I AU - Sakkas LI AD - Department of Rheumatology and Clinical Immunology. LA - eng PT - Case Reports PT - Journal Article DEP - 20190930 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7045864 OTO - NOTNLM OT - Raynaud’s phenomenon OT - anti-Jo1 OT - anti-synthetase syndrome OT - interstitial lung disease EDAT- 2020/03/19 06:00 MHDA- 2020/03/19 06:01 PMCR- 2019/09/30 CRDT- 2020/03/19 06:00 PHST- 2019/04/12 00:00 [received] PHST- 2019/06/25 00:00 [revised] PHST- 2019/07/09 00:00 [accepted] PHST- 2020/03/19 06:00 [entrez] PHST- 2020/03/19 06:00 [pubmed] PHST- 2020/03/19 06:01 [medline] PHST- 2019/09/30 00:00 [pmc-release] AID - MJR-30-3-186 [pii] AID - 10.31138/mjr.30.3.186 [doi] PST - epublish SO - Mediterr J Rheumatol. 2019 Sep 30;30(3):186-189. doi: 10.31138/mjr.30.3.186. eCollection 2019 Sep. PMID- 35382145 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 4 IP - 1 DP - 2019 Feb TI - Iloprost infusion through elastomeric pump in the treatment of Raynaud's phenomenon and digital ulcers. PG - NP1-NP4 LID - 10.1177/2397198318765596 [doi] AB - INTRODUCTION: Systemic sclerosis is a systemic autoimmune disease characterized by microangiopathy and fibroblast dysfunction resulting in fibrosis of the skin and internal organs. Raynaud's phenomenon and digital ulcers are the main clinical features of vascular involvement. Treatment with iloprost is recommended to reduce the frequency and severity of Raynaud's phenomenon attacks and to heal active digital ulcer. Classical forms of treatment require admission to a ward or day-hospital unit to ensure safety during infusion, causing disruption of the patient's normal daily life and resulting in significant costs with hospitalization. Nowadays, new portable devices, of which the elastomeric pump is an example, are becoming available in order to avoid hospitalization. CASE REPORTS: We describe five cases of patients with systemic sclerosis or mixed connective tissue disease and severe Raynaud's phenomenon/critical ischaemia or active digital ulcers that were successfully treated with iloprost infusion through elastomeric pump without experiencing any side effects. We present our unit's protocol for ambulatory infusion. DISCUSSION/CONCLUSION: Our case reports and a brief review of literature prove that iloprost infusion through elastomeric pump is safe, easy and well tolerated and might even improve patient compliance with treatment. Meanwhile, it may also decrease the economic burden of hospitalization with these patients. CI - © The Author(s) 2018. FAU - Fartura Braga Temido, Maria Helena AU - Fartura Braga Temido MH AD - Internal Medicine Department A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Gomes, Manuel AU - Gomes M AD - Internal Medicine Department, Trofa Saúde Hospital, Trofa, Portugal. FAU - Parente, Francisco AU - Parente F AD - Internal Medicine Department A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Santos, Lèlita AU - Santos L AD - Internal Medicine Department A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20180410 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922585 OTO - NOTNLM OT - Digital ulcers OT - Raynaud’s phenomenon OT - elastomeric pump OT - iloprost COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2019/02/01 00:00 MHDA- 2019/02/01 00:01 PMCR- 2020/02/01 CRDT- 2022/04/06 05:12 PHST- 2017/12/19 00:00 [received] PHST- 2018/02/26 00:00 [accepted] PHST- 2022/04/06 05:12 [entrez] PHST- 2019/02/01 00:00 [pubmed] PHST- 2019/02/01 00:01 [medline] PHST- 2020/02/01 00:00 [pmc-release] AID - 10.1177_2397198318765596 [pii] AID - 10.1177/2397198318765596 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2019 Feb;4(1):NP1-NP4. doi: 10.1177/2397198318765596. Epub 2018 Apr 10. PMID- 39557079 OWN - NLM STAT- In-Process LR - 20250225 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 92 IP - 3 DP - 2025 Mar TI - A global retrospective study identifies higher rates of pernio in individuals with connective tissue disease. PG - 636-640 LID - S0190-9622(24)03217-1 [pii] LID - 10.1016/j.jaad.2024.11.014 [doi] FAU - Arora, Jagmeet S AU - Arora JS AD - Department of Dermatology, University of California Irvine, Irvine, California. FAU - Kesler, Alice AU - Kesler A AD - Department of Graduate Medical Education, Lakeland Regional Medical Center, Lakeland, Florida. FAU - Min, Michelle S AU - Min MS AD - Department of Dermatology, University of California Irvine, Irvine, California. Electronic address: michellesmin@gmail.com. LA - eng PT - Journal Article DEP - 20241117 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM OTO - NOTNLM OT - Behcet's OT - Raynaud's phenomenon OT - antiphospholipid syndrome OT - chilblains OT - connective tissue disease OT - dermatomyositis OT - eosinophilic fasciitis OT - lupus OT - morphea OT - pernio OT - rheumatoid arthritis OT - scleroderma OT - vasculitis COIS- Conflicts of interest Dr Min is on the advisory boards of Horizon and BMS. She is an investigator for Amgen, Priovant, and BI. Author Arora and Dr Kesler have no conflicts of interest to declare. EDAT- 2024/11/19 00:22 MHDA- 2024/11/19 00:22 CRDT- 2024/11/18 19:22 PHST- 2024/08/13 00:00 [received] PHST- 2024/11/02 00:00 [revised] PHST- 2024/11/10 00:00 [accepted] PHST- 2024/11/19 00:22 [pubmed] PHST- 2024/11/19 00:22 [medline] PHST- 2024/11/18 19:22 [entrez] AID - S0190-9622(24)03217-1 [pii] AID - 10.1016/j.jaad.2024.11.014 [doi] PST - ppublish SO - J Am Acad Dermatol. 2025 Mar;92(3):636-640. doi: 10.1016/j.jaad.2024.11.014. Epub 2024 Nov 17. PMID- 40386534 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250520 IS - 2050-313X (Print) IS - 2050-313X (Electronic) IS - 2050-313X (Linking) VI - 13 DP - 2025 TI - A middle-aged woman with the initial symptom of acrocyanosis diagnosed with cold agglutinin disease: A case report. PG - 2050313X251342166 LID - 10.1177/2050313X251342166 [doi] LID - 2050313X251342166 AB - Autoimmune hemolytic anemia is when autoantibodies attack red blood cells, leading to anemia. Cold agglutinin disease, a subtype of autoimmune hemolytic anemia, accounts for 13%-32% of cases. We present a middle-aged woman with a history of Raynaud's phenomenon who was admitted to the hospital due to acrocyanosis, weakness, and lethargy. Her blood tests revealed indirect hyperbilirubinemia, severe anemia, an increase in lactate dehydrogenase, a positive direct Coombs test, increased inflammatory factors, and a positive cold agglutinin antibody, confirming the diagnosis of cold agglutinin disease. Despite a 5-day hospital stay and initiation of treatments, she did not show a satisfactory response to the treatments. Cold agglutinin disease is a chronic disease that can initially present with mild symptoms. Early identification through appropriate laboratory tests is crucial. In severe cases, prompt initiation of proper treatment is necessary to prevent fatal outcomes. CI - © The Author(s) 2025. FAU - Salimi, Babak AU - Salimi B AD - Research Center of Thoracic Oncology (RCTO), National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Salimi, Maryam AU - Salimi M AD - Department of Hematology and Oncology, Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Tavakoli Avval, Mahta AU - Tavakoli Avval M AD - School of Medicine, Ardabil University of Medical Sciences, Iran. FAU - Jannati, Rona AU - Jannati R AD - Department of Internal Medicine, School of Medicine, Ardabil University of Medical Sciences, Iran. FAU - Negaresh, Mohammad AU - Negaresh M AUID- ORCID: 0000-0002-8293-9139 AD - Department of Internal Medicine, School of Medicine, Ardabil University of Medical Sciences, Iran. LA - eng PT - Case Reports PT - Journal Article DEP - 20250517 PL - England TA - SAGE Open Med Case Rep JT - SAGE open medical case reports JID - 101638686 PMC - PMC12085749 OTO - NOTNLM OT - Raynaud’s phenomenon OT - acrocyanosis OT - autoimmune hemolytic anemia OT - cold agglutinin disease OT - hemolysis COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/05/19 12:29 MHDA- 2025/05/19 12:30 PMCR- 2025/05/17 CRDT- 2025/05/19 06:25 PHST- 2025/03/12 00:00 [received] PHST- 2025/04/28 00:00 [accepted] PHST- 2025/05/19 12:30 [medline] PHST- 2025/05/19 12:29 [pubmed] PHST- 2025/05/19 06:25 [entrez] PHST- 2025/05/17 00:00 [pmc-release] AID - 10.1177_2050313X251342166 [pii] AID - 10.1177/2050313X251342166 [doi] PST - epublish SO - SAGE Open Med Case Rep. 2025 May 17;13:2050313X251342166. doi: 10.1177/2050313X251342166. eCollection 2025. PMID- 27895669 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1687-9627 (Print) IS - 1687-9635 (Electronic) VI - 2016 DP - 2016 TI - Myeloproliferative Disease: An Unusual Cause of Raynaud's Phenomenon and Digital Ischaemia. PG - 9675171 LID - 9675171 AB - We describe a 59-year-old female who presented with ischaemic digits, preceded by a 6-month history of Raynaud's phenomenon affecting her fingers and toes. There were no clinical or laboratory features of primary vasculitis or connective tissue disease, Doppler imaging was normal, and bloods were unremarkable aside from a platelet count of 786 × 109/L (150-400) and white cells of 16 × 109/L (4-11). In view of the thrombocytosis a JAK2 mutation assay was requested which confirmed a JAK2 V617F mutation, suggesting essential thrombocytosis (ET) as the cause. She received treatment with hydroxycarbamide which normalised her platelet count and led to a complete resolution of her Raynaud's symptoms. Raynaud's phenomenon is a rare manifestation of ET. Myeloproliferative disorders such as ET should be considered in the differential diagnosis of Raynaud's phenomenon and vasculitis. FAU - Beynon, Celia AU - Beynon C AUID- ORCID: 0000-0001-9043-3210 AD - Department of Rheumatology, Princess of Wales Hospital, Coity Road, Bridgend CF31 1RQ, UK. FAU - Huws, Gwenan AU - Huws G AD - Department of Rheumatology, Royal Gwent Hospital, Cardiff Road, Newport NP20 2UB, UK. FAU - Lawson, Tom AU - Lawson T AD - Department of Rheumatology, Princess of Wales Hospital, Coity Road, Bridgend CF31 1RQ, UK. LA - eng PT - Journal Article DEP - 20161108 PL - United States TA - Case Rep Med JT - Case reports in medicine JID - 101512910 PMC - PMC5118510 COIS- The authors have no competing interests to declare. EDAT- 2016/11/30 06:00 MHDA- 2016/11/30 06:01 PMCR- 2016/11/08 CRDT- 2016/11/30 06:00 PHST- 2016/06/29 00:00 [received] PHST- 2016/09/22 00:00 [revised] PHST- 2016/10/27 00:00 [accepted] PHST- 2016/11/30 06:00 [entrez] PHST- 2016/11/30 06:00 [pubmed] PHST- 2016/11/30 06:01 [medline] PHST- 2016/11/08 00:00 [pmc-release] AID - 10.1155/2016/9675171 [doi] PST - ppublish SO - Case Rep Med. 2016;2016:9675171. doi: 10.1155/2016/9675171. Epub 2016 Nov 8. PMID- 41760496 OWN - NLM STAT- Publisher LR - 20260227 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) DP - 2026 Feb 26 TI - Raynaud's phenomenon and digital ulceration in systemic sclerosis. PG - 102121 LID - S1521-6942(26)00006-9 [pii] LID - 10.1016/j.berh.2026.102121 [doi] AB - Systemic sclerosis (SSc) is a vascular disease. Immune-mediated endothelial injury leading to vasculopathy characterised by a progressive obliterative microangiopathy is a key aetiopathogenic driver of SSc. This typically manifests clinically as Raynaud's phenomenon (RP), which occurs in virtually all individuals with SSc and digital ulcers (DU), which develop in around half of patients at some stage in the disease course. The term RP encompasses a constellation of clinical features associated with digital vasospasm, typically in response to cold exposure. People with SSc-DU typically report more severe RP symptoms but it is an over-simplification to suggest that DU are simply a consequence of profound RP. The level of ischaemic tissue injury required to cause DU requires more protracted tissue hypoxia and there is a large body of work linking SSc-DU disease with progression of the obliterative microangiopathy and irreversible capillary loss typical of established SSc. The present chapter shall discuss the burden, aetiopathogenesis, assessment and management of SSc-RP and SSc-DU. We shall highlight practical considerations for the assessment and management of these digital vascular complications in routine clinical practice. The complexity of SSc-related digital vasculopathy is illustrated through examples from the clinic setting. The management of these complications shall focus on recently published clinical guidelines, highlighting nuances of management and the therapeutic rationale underpinning the use of unlicensed therapies for which the evidence base is sometimes scant. CI - Copyright © 2026 Elsevier Ltd. All rights reserved. FAU - Turnbull, Amy AU - Turnbull A AD - Department of Internal Medicine, Bristol Royal Infirmary, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK; Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. Electronic address: John.Pauling@nbt.nhs.uk. LA - eng PT - Journal Article PT - Review DEP - 20260226 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM OTO - NOTNLM OT - Digital ulcers OT - Digital vasculopathy OT - Practical management OT - Raynaud's phenomenon OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: AT has no relevant conflicts of interest. JDP reports speaker honoraria from Johnson & Johnson and Boehringer Ingelheim. JDP has undertaken consultancy work for Johnson & Johnson, Abbvie, Celexor Bio, Liquidia, Astra Zeneca, Boehringer-Ingelheim, Sojournix Pharma, IsoMab pharma and Permeatus, Inc. JDP has received educational support from CSL Vifor outside the submitted work. EDAT- 2026/02/28 00:41 MHDA- 2026/02/28 00:41 CRDT- 2026/02/27 22:01 PHST- 2026/02/06 00:00 [received] PHST- 2026/02/06 00:00 [accepted] PHST- 2026/02/28 00:41 [medline] PHST- 2026/02/28 00:41 [pubmed] PHST- 2026/02/27 22:01 [entrez] AID - S1521-6942(26)00006-9 [pii] AID - 10.1016/j.berh.2026.102121 [doi] PST - aheadofprint SO - Best Pract Res Clin Rheumatol. 2026 Feb 26:102121. doi: 10.1016/j.berh.2026.102121. PMID- 33521578 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210202 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 31 IP - 4 DP - 2020 Dec TI - The Role of Novel Autoantibodies in the Diagnostic Approach and Prognosis of Patients with Raynaud's Phenomenon. PG - 427-429 LID - 10.31138/mjr.31.4.427 [doi] AB - Raynaud's phenomenon (RP) is a condition characterised by distinct colour changes of the digits upon exposure to sympathomimetic conditions, such as cold temperature. It can be either primary or secondary, depending on whether it presents alone or as part of an underlying disorder. One of the most common causes of secondary RP are systemic autoimmune rheumatic diseases (SARDs), in which RP may precede the onset of other autoimmune features by many years. Thus, timely and accurate recognition of secondary RP is of great importance as it alters patient management and prognosis. An important step in the diagnostic approach of RP is the detection of antinuclear antibodies (ANAs) by indirect immunofluorescence. However, identification of specific autoantibodies is not yet common practice, though many of them have shown important clinical associations. Moreover, the role of some autoantibodies has not yet been elucidated, given their relatively recent discovery and low reported prevalence rates in autoimmune population. The goal of this study is to reveal clinical associations of these novel autoantibodies in SARDs through the application of an extended serology workup in patients presenting with RP. CI - © 2020 The Mediterranean Journal of Rheumatology (MJR). FAU - Koulouri, Vasiliki AU - Koulouri V AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Nezos, Adrianos AU - Nezos A AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Marketos, Nikolaos AU - Marketos N AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Rheumatology Outpatient Department, Henry Dunant Hospital Centre, Athens, Greece. FAU - Argyriou, Evangelia AU - Argyriou E AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Rheumatology Unit, Sismanogleio General Hospital, Athens, Greece. FAU - Boki, Kyriaki AU - Boki K AD - Rheumatology Unit, Sismanogleio General Hospital, Athens, Greece. FAU - Ioakimidis, Dimitrios AU - Ioakimidis D AD - Rheumatology Outpatient Department, Henry Dunant Hospital Centre, Athens, Greece. FAU - Koutsilieris, Michalis AU - Koutsilieris M AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Mavragani, Clio P AU - Mavragani CP AD - Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Rheumatology Outpatient Unit, Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Laikon Hospital, Athens, Greece. LA - eng PT - Journal Article DEP - 20201228 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7841088 OTO - NOTNLM OT - Autoantibodies OT - Raynaud’s phenomenon OT - systemic autoimmune rheumatic disease EDAT- 2021/02/02 06:00 MHDA- 2021/02/02 06:01 PMCR- 2020/12/28 CRDT- 2021/02/01 06:02 PHST- 2020/05/30 00:00 [received] PHST- 2020/06/14 00:00 [revised] PHST- 2020/06/30 00:00 [accepted] PHST- 2021/02/01 06:02 [entrez] PHST- 2021/02/02 06:00 [pubmed] PHST- 2021/02/02 06:01 [medline] PHST- 2020/12/28 00:00 [pmc-release] AID - MJR-31-4-427 [pii] AID - 10.31138/mjr.31.4.427 [doi] PST - epublish SO - Mediterr J Rheumatol. 2020 Dec 28;31(4):427-429. doi: 10.31138/mjr.31.4.427. eCollection 2020 Dec. PMID- 38514374 OWN - NLM STAT- MEDLINE DCOM- 20240515 LR - 20240515 IS - 0965-206X (Print) IS - 0965-206X (Linking) VI - 33 IP - 2 DP - 2024 May TI - Impact of foot functionality in patients with systemic sclerosis: Cross-sectional study. PG - 202-207 LID - S0965-206X(24)00029-9 [pii] LID - 10.1016/j.jtv.2024.03.004 [doi] AB - BACKGROUND: Progressive systemic sclerosis or systemic scleroderma (SS) is a chronic and rare autoimmune disease that mainly affects the skin and various internal organs. Raynaud's phenomenon and digital ulcers are some of the symptoms that affect the foot, causing a decrease in the quality of life of patients. The objective of this study is to determine the functionality of the feet in patients with SS and determine the impact on their daily lives. METHODS: A sample of 165 patients (154 women, 11 men) diagnosed with SS with a mean age of 46.29 ± 11.36 years and a mean body mass index (BMI) of 24.90 ± 5.77 was recruited. Each participant completed the Foot Function Index (FFI) questionnaire and the Systemic Sclerosis Questionnaire (SySQ). A multivariate analysis was performed to determine which factors were related to a higher score in both questionnaires. RESULTS: 32.1% of the participants (n = 53) had claw toe deformities, 79.4% (n = 131) Raynaud's disease and 20% (n = 33) a history of foot ulcers. 51.5% of the participants (n = 85) presented symptoms in their nails, the most frequent sign being thickening, hardening and yellow coloration. The final score of the FFI questionnaire was 3.51 ± 2.41 (0-9.9), the pain subscale being the highest, with a score of 5.06 ± 2.75, followed by foot disability (3.26 ± 2.91) and difficulty performing activities (1.55 ± 2.22). The final score of the SySQ questionnaire was 0.95 ± 0.45 (0.18-2.45), and the subscales with the highest score were symptom frequency (1.30 ± 0.47), symptom intensity (1.11 ± 0.55), and general skill limitation (0.47 ± 0.51). A high correlation was observed between the final FFI score and the final SySQ score (r = 0.712; p=<0.001). Also, between foot activity limitation and general skill limitation (r = 0.658; p=<0.001). A moderate correlation was observed between foot pain score and overall symptom intensity (r = 0.482; p=<0.001). Also, between foot disability and overall symptom frequency (r = 0.556; p=<0.001). The multivariate analysis (R(2) 0.51) showed that the final FFI score had a significant relationship with the final SySQ score (p < 0.001). No significant correlation was found between age (p = 0.15), gender (p = 0.49), BMI (p = 0.74) or time of diagnosis (p = 0.57) and FFI. CONCLUSION: SS is a disease that affects foot functionality in patients, with a greater impact on the pain scale. There is a correlation between the final FFI score and the final SySQ score, so improving foot functionality could help to improve the overall functionality of the patient with sclerosis. CI - Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Chicharro-Luna, Esther AU - Chicharro-Luna E AD - Department Behavioral Sciences and Health, Nursing Area, Faculty of Medicine, University Miguel Hernández, Spain. Electronic address: ec.luna@umh.es. FAU - Gracia-Vesga, Miguel Ángel AU - Gracia-Vesga MÁ AD - Occupational Therapy, University Miguel Hernández, Spain. Electronic address: ocupaterapia@gmail.com. FAU - Ramos-Petersen, Laura AU - Ramos-Petersen L AD - Department of Nursing and Podiatry, Faculty of Health Sciences, University of Málaga, Spain. Electronic address: lauraramos.94@uma.es. FAU - Gijón-Nogueron, Gabriel AU - Gijón-Nogueron G AD - Department of Nursing and Podiatry, Faculty of Health Sciences, IBIMA, University of Málaga, Spain. Electronic address: gagijon@uma.es. LA - eng PT - Journal Article DEP - 20240307 PL - England TA - J Tissue Viability JT - Journal of tissue viability JID - 9306822 SB - IM MH - Humans MH - Female MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - *Scleroderma, Systemic/complications/physiopathology MH - Surveys and Questionnaires MH - Adult MH - Quality of Life/psychology MH - Foot/physiopathology OTO - NOTNLM OT - Foot OT - Foot function index OT - Prevalence OT - Quality of life OT - Sclerosis OT - Systemic sclerosis questionnaire EDAT- 2024/03/22 00:44 MHDA- 2024/05/16 00:44 CRDT- 2024/03/21 23:00 PHST- 2023/09/04 00:00 [received] PHST- 2023/11/27 00:00 [revised] PHST- 2024/03/01 00:00 [accepted] PHST- 2024/05/16 00:44 [medline] PHST- 2024/03/22 00:44 [pubmed] PHST- 2024/03/21 23:00 [entrez] AID - S0965-206X(24)00029-9 [pii] AID - 10.1016/j.jtv.2024.03.004 [doi] PST - ppublish SO - J Tissue Viability. 2024 May;33(2):202-207. doi: 10.1016/j.jtv.2024.03.004. Epub 2024 Mar 7. PMID- 33403182 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231110 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 12 IP - 11 DP - 2020 Nov 28 TI - COVID-19-Induced Vestibular Neuritis, Hemi-Facial Spasms and Raynaud's Phenomenon: A Case Report. PG - e11752 LID - 10.7759/cureus.11752 [doi] LID - e11752 AB - The coronavirus disease 2019 (COVID-19) pandemic has created a global health crisis. Though respiratory symptoms have been the usual manifestations, the presentation in some cases may be atypical with various neurological and cutaneous manifestations. We present a case of a 63-year-old female diagnosed with COVID-19 and associated rare manifestations during her visit to Europe. CI - Copyright © 2020, Vanaparthy et al. FAU - Vanaparthy, Rachana AU - Vanaparthy R AD - Pulmonology, Oregon Health & Science University, Portland, USA. FAU - Malayala, Srikrishna V AU - Malayala SV AD - Internal Medicine, Temple University Hospital, Philadelphia, USA. FAU - Balla, Mamtha AU - Balla M AD - Internal Medicine, ProMedica Toledo Hospital, Toledo, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20201128 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7773294 OTO - NOTNLM OT - covid-19 OT - cutaneous manifestations OT - hemifacial spasms OT - neurological manifestations OT - raynaud’s phenomenon OT - urticaria. OT - vestibular neuritis COIS- The authors have declared that no competing interests exist. EDAT- 2021/01/07 06:00 MHDA- 2021/01/07 06:01 PMCR- 2020/11/28 CRDT- 2021/01/06 05:53 PHST- 2021/01/06 05:53 [entrez] PHST- 2021/01/07 06:00 [pubmed] PHST- 2021/01/07 06:01 [medline] PHST- 2020/11/28 00:00 [pmc-release] AID - 10.7759/cureus.11752 [doi] PST - epublish SO - Cureus. 2020 Nov 28;12(11):e11752. doi: 10.7759/cureus.11752. PMID- 26019859 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150528 LR - 20201001 IS - 2048-8505 (Print) IS - 2048-8513 (Electronic) IS - 2048-8505 (Linking) VI - 6 IP - 2 DP - 2013 Apr TI - Joint pain, digital necrosis and acute kidney failure in small-vessel autoimmune disease. PG - 240-2 LID - 10.1093/ckj/sft015 [doi] FAU - Bartsch, Peter AU - Bartsch P AD - Department of Nephrology and Hypertension, Diabetes and Endocrinology , Otto-von-Guericke University Magdeburg , Magdeburg , Germany. FAU - Schossee, Aileen AU - Schossee A AD - Department of Nephrology and Hypertension, Diabetes and Endocrinology , Otto-von-Guericke University Magdeburg , Magdeburg , Germany. FAU - Sitte, Joana AU - Sitte J AD - Department of Nephrology and Hypertension, Diabetes and Endocrinology , Otto-von-Guericke University Magdeburg , Magdeburg , Germany. FAU - Mertens, Peter R AU - Mertens PR AD - Department of Nephrology and Hypertension, Diabetes and Endocrinology , Otto-von-Guericke University Magdeburg , Magdeburg , Germany. LA - eng PT - Journal Article PL - England TA - Clin Kidney J JT - Clinical kidney journal JID - 101579321 PMC - PMC4432452 OTO - NOTNLM OT - ANCA-associated necrotizing granulomatosis OT - Raynaud's phenomenon OT - digital gangrene EDAT- 2013/04/01 00:00 MHDA- 2013/04/01 00:01 PMCR- 2013/04/01 CRDT- 2015/05/29 06:00 PHST- 2012/07/16 00:00 [received] PHST- 2013/01/24 00:00 [accepted] PHST- 2015/05/29 06:00 [entrez] PHST- 2013/04/01 00:00 [pubmed] PHST- 2013/04/01 00:01 [medline] PHST- 2013/04/01 00:00 [pmc-release] AID - sft015 [pii] AID - 10.1093/ckj/sft015 [doi] PST - ppublish SO - Clin Kidney J. 2013 Apr;6(2):240-2. doi: 10.1093/ckj/sft015. PMID- 33281997 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211203 IS - 1869-3474 (Print) IS - 1869-3482 (Electronic) IS - 1869-3474 (Linking) VI - 54 IP - 6 DP - 2020 Dec TI - Perfusion Scintigraphy for the Evaluation of Patients with Raynaud's Phenomenon. PG - 269-273 LID - 10.1007/s13139-020-00671-6 [doi] AB - Raynaud's phenomenon (RP) is a functional vascular disorder, which can be defined as transient vasospasm of the peripheral arteries and arterioles in the affected areas exposed to the cold or other stress. The diagnosis of RP is mainly based on symptoms. Perfusion scintigraphy, with or without cold stimulation, can be used to evaluate RP. Studies with perfusion scintigraphy for RP have shown that patients with RP showed lower finger-to-palm ratio than patients without RP. Responses after cold stimulation were also different in patients with RP. Not only decreased perfusion or blood pool after cold stimulation but also paradoxically increased perfusion can be shown in patients with RP. Some studies have shown that primary and secondary RP can be differentiated by perfusion scintigraphy. Correlation between duration of disease and findings on perfusion scintigraphy was reported. Perfusion scintigraphy can show differences before and after treatment as well. However, the protocols for perfusion scintigraphy for PR vary among studies. The standard protocol of perfusion scintigraphy for RP should be established. CI - © Korean Society of Nuclear Medicine 2020. FAU - Chong, Ari AU - Chong A AUID- ORCID: 0000-0001-9327-9810 AD - Department of Nuclear Medicine, Chosun University Hospital, Chosun University School of Medicine, 365, Pilmun-daero, Dong-gu, Gwangju Metropolitan City, 61453 Republic of Korea. GRID: grid.254187.d. ISNI: 0000 0000 9475 8840 LA - eng PT - Journal Article DEP - 20201022 PL - Germany TA - Nucl Med Mol Imaging JT - Nuclear medicine and molecular imaging JID - 101530478 PMC - PMC7704978 OTO - NOTNLM OT - Diagnostic imaging OT - Perfusion imaging OT - Raynaud’s phenomenon COIS- Conflict of InterestAri Chong declares that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript. EDAT- 2020/12/08 06:00 MHDA- 2020/12/08 06:01 PMCR- 2021/12/01 CRDT- 2020/12/07 05:32 PHST- 2020/08/04 00:00 [received] PHST- 2020/10/06 00:00 [revised] PHST- 2020/10/16 00:00 [accepted] PHST- 2020/12/07 05:32 [entrez] PHST- 2020/12/08 06:00 [pubmed] PHST- 2020/12/08 06:01 [medline] PHST- 2021/12/01 00:00 [pmc-release] AID - 671 [pii] AID - 10.1007/s13139-020-00671-6 [doi] PST - ppublish SO - Nucl Med Mol Imaging. 2020 Dec;54(6):269-273. doi: 10.1007/s13139-020-00671-6. Epub 2020 Oct 22. PMID- 26169898 OWN - NLM STAT- MEDLINE DCOM- 20160607 LR - 20150911 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 142 IP - 8-9 DP - 2015 Aug-Sep TI - [Diagnosis of vascular acrosyndromes]. PG - 513-8 LID - S0151-9638(15)00450-0 [pii] LID - 10.1016/j.annder.2015.06.006 [doi] AB - Vascular acrosyndromes are associated with vasomotor disorders. They may be paroxysmal, like Raynaud's phenomenon, whitening of the fingers on exposure to cold, or erythromelalgia, a painful form of erythema induced by exposure to heat. Others are permanent or semi-permanent, such as acrocyanosis, chilblains, spontaneous haematoma of the fingers, acrocholose and digital ischaemia or necrosis. Diagnosis of the type of acrosyndrome at issue is based primarily on clinical examination and history-taking. Capillaroscopy and antinuclear antibody assay are key examinations essential for distinguishing between primary and secondary Raynaud's phenomenon and connective tissue disorders. Complete blood counts, screening for thyroid dysthyroidism, and antinuclear antibody assay can help distinguish between primary erythromelalgia and erythromelalgia secondary to a systemic disease, principally myeloproliferative syndrome. In the case of acrocyanosis, spontaneous digital haematomas and typical bilateral chilblains, examinations are of no value. For the other permanent and semi-permanent acrosyndromes such as digital ischaemia and purpuric or livedoid lesions, screening for arterial or thrombotic disease is necessary. CI - Copyright © 2015 Elsevier Masson SAS. All rights reserved. FAU - Senet, P AU - Senet P AD - Service de dermatologie, UF de dermatologie vasculaire, hôpital Tenon, 4, rue de la Chine, 75970 Paris cedex 20, France. Electronic address: patricia.senet@tnn.aphp.fr. LA - fre PT - English Abstract PT - Journal Article TT - Diagnostic des acrosyndromes vasculaires. DEP - 20150711 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 SB - IM MH - Algorithms MH - Chilblains/*diagnosis MH - Fingers/blood supply MH - Hematoma/*diagnosis MH - Humans MH - Ischemia/*diagnosis MH - Necrosis MH - Peripheral Vascular Diseases/*diagnosis MH - Physical Examination/methods OTO - NOTNLM OT - Acrocyanose OT - Acrocyanosis OT - Acrosyndromes vasculaires OT - Erythromelalgia OT - Phénomène de Raynaud OT - Raynaud's phenomenon OT - Vascular acrosyndromes OT - Érythromélalgie EDAT- 2015/07/15 06:00 MHDA- 2016/06/09 06:00 CRDT- 2015/07/15 06:00 PHST- 2015/07/15 06:00 [entrez] PHST- 2015/07/15 06:00 [pubmed] PHST- 2016/06/09 06:00 [medline] AID - S0151-9638(15)00450-0 [pii] AID - 10.1016/j.annder.2015.06.006 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2015 Aug-Sep;142(8-9):513-8. doi: 10.1016/j.annder.2015.06.006. Epub 2015 Jul 11. PMID- 21794380 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20121031 IS - 1699-258X (Print) IS - 1699-258X (Linking) VI - 2 Suppl 3 DP - 2006 Nov TI - [Raynaud's phenomenon]. PG - S10-5 LID - 10.1016/S1699-258X(06)73101-1 [doi] AB - Raynaud's phenomenon is characterized by repeated daily attacks of ischemia followed by reperfusion at the acrallevel. It is a frequent syndrome found in medical practice; and it can be considered as primary or secondary to other conditions, including rheumatic autoimmune diseases. Current classification had clinical and therapeutic implications. Careful clinical evaluation is the most reliable and reproducible method in the diagnosis of Raynaud's phenomenon. Several risk factors had been associated in the genesis of Raynaud's phenomenon; however, its pathogenesis remains elusive although recently, considerable progress in disease mechanism had been described. Such advances are directing new lines of therapy. CI - Copyright © 2006 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved. FAU - Saavedra Salinas, Miguel Ángel AU - Saavedra Salinas MÁ AD - Departamento de Reumatología. Unidad Médica de Alta Especialidad. Hospital de Especialidades Dr. Antonio Fraga Mouret. Centro Médico Nacional La Raza. Instituto Mexicano del Seguro Social. México DF. México. FAU - Carrillo Vázquez, Sandra Miriam AU - Carrillo Vázquez SM LA - spa PT - English Abstract PT - Journal Article TT - Fenómeno de Raynaud. DEP - 20081210 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 EDAT- 2006/11/01 00:00 MHDA- 2006/11/01 00:01 CRDT- 2011/07/29 06:00 PHST- 2011/07/29 06:00 [entrez] PHST- 2006/11/01 00:00 [pubmed] PHST- 2006/11/01 00:01 [medline] AID - S1699-258X(06)73101-1 [pii] AID - 10.1016/S1699-258X(06)73101-1 [doi] PST - ppublish SO - Reumatol Clin. 2006 Nov;2 Suppl 3:S10-5. doi: 10.1016/S1699-258X(06)73101-1. Epub 2008 Dec 10. PMID- 24353496 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20131219 LR - 20211021 IS - 1642-395X (Print) IS - 2299-0046 (Electronic) IS - 1642-395X (Linking) VI - 30 IP - 5 DP - 2013 Oct TI - Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl. PG - 329-36 LID - 10.5114/pdia.2013.38365 [doi] AB - Mixed connective tissue disease (MCTD) is a systemic inflammatory disease affecting connective tissue with the underlying autoimmunological mechanism. The core of MCTD is an appearance of symptoms of several other inflammatory diseases of connective tissue - systemic lupus erythematosus, systemic scleroderma, poly- or dermatomyositis, rheumatoid arthritis at the same time, accompanied by a high level of anti-ribonucleoprotein antibodies (anti-U1RNP). The disease was described more than 40 years ago by Sharp et al. During recent years, many efforts to better understand clinical and serological features of MCTD have been made. Diagnosis of MCTD can be difficult. Obligatory international diagnostic criteria are required to be fulfilled. Several versions of such criteria have been proposed, but the most widely used one was described by Kasukawa. There is no consensus about treatment - a choice of drugs depends on symptoms. We present a case of a 10-year-old girl with sclerodactyly and trophic damages of fingers accompanied by symptoms of Raynaud's phenomenon. After an almost 2-year course of the disease, a diagnosis of MCTD has been established. FAU - Latuśkiewicz-Potemska, Joanna AU - Latuśkiewicz-Potemska J AD - Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD. FAU - Zygmunt, Agnieszka AU - Zygmunt A AD - Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD. FAU - Biernacka-Zielińska, Małgorzata AU - Biernacka-Zielińska M AD - Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD. FAU - Stańczyk, Jerzy AU - Stańczyk J AD - Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD. FAU - Smolewska, Elżbieta AU - Smolewska E AD - Department of Pediatric Cardiology and Rheumatology, Clinical Hospital No. 4 of Medical University of Lodz, Poland. Head of Department: Prof. Jerzy Stańczyk MD, PhD. LA - eng PT - Case Reports PT - Journal Article DEP - 20131030 PL - Poland TA - Postepy Dermatol Alergol JT - Postepy dermatologii i alergologii JID - 101168357 PMC - PMC3858664 OTO - NOTNLM OT - Raynaud's phenomenon OT - mixed connective tissue disease OT - sclerodactyly OT - trophic damages of fingers EDAT- 2013/12/20 06:00 MHDA- 2013/12/20 06:01 PMCR- 2013/10/01 CRDT- 2013/12/20 06:00 PHST- 2013/01/03 00:00 [received] PHST- 2013/06/15 00:00 [revised] PHST- 2013/08/20 00:00 [accepted] PHST- 2013/12/20 06:00 [entrez] PHST- 2013/12/20 06:00 [pubmed] PHST- 2013/12/20 06:01 [medline] PHST- 2013/10/01 00:00 [pmc-release] AID - 21585 [pii] AID - 10.5114/pdia.2013.38365 [doi] PST - ppublish SO - Postepy Dermatol Alergol. 2013 Oct;30(5):329-36. doi: 10.5114/pdia.2013.38365. Epub 2013 Oct 30. PMID- 39712791 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250104 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 11 DP - 2024 Nov TI - Lymphoplasmacytic Lymphoma Presenting as Severe Secondary Raynaud's Phenomenon. PG - e74144 LID - 10.7759/cureus.74144 [doi] LID - e74144 AB - Primary Raynaud's phenomenon (RP) is a common and self-limiting condition, which is not secondary to any other disease process. In contrast, secondary RP has an underlying etiology. Several conditions can lead to secondary RP, which creates a challenging landscape for clinicians. Differentiation between primary and secondary RP is vital as failure to do so can lead to delays in treatment and poor patient outcomes. We present a case of a 77-year-old male who experienced digit discoloration when exposed to cold temperatures. He had been initially diagnosed with primary RP, but his symptoms had increased in frequency and progressed to digit necrosis requiring amputation. He was admitted to our tertiary care center for further workup. Subsequently, a diagnosis of type I cryoglobulinemia secondary to lymphoplasmacytic lymphoma (LPL) was made instead of the initial diagnosis of primary RP as the cause of his digit necrosis. This report emphasizes the importance of differentiating between primary and secondary RP and highlights the need for comprehensive workup in patients with RP, especially those presenting with atypical features. CI - Copyright © 2024, Rorah et al. FAU - Rorah, Drayton J AU - Rorah DJ AD - Internal Medicine, University of Kansas Medical Center, Kansas City, USA. FAU - Daghlas, Salah AU - Daghlas S AD - Internal Medicine, University of Kansas Medical Center, Kansas City, USA. FAU - Badshah, Mashood AU - Badshah M AD - Division of Allergy, Clinical Immunology, & Rheumatology, University of Kansas Medical Center, Kansas City, USA. FAU - Schmidt, Paul AU - Schmidt P AD - Division of Allergy, Clinical Immunology, & Rheumatology, University of Kansas Medical Center, Kansas City, USA. FAU - Maz, Mehrdad AU - Maz M AD - Division of Allergy, Clinical Immunology, & Rheumatology, University of Kansas Medical Center, Kansas City, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20241121 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11662510 OTO - NOTNLM OT - clinical rheumatology OT - cryoglobulins OT - delayed diagnosis OT - diagnosis OT - digital gangrene OT - lymphoplasmacytic lymphoma OT - raynaud’s phenomenon OT - secondary raynaud’s phenomenon COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/12/23 17:24 MHDA- 2024/12/23 17:25 PMCR- 2024/11/21 CRDT- 2024/12/23 06:10 PHST- 2024/11/21 00:00 [accepted] PHST- 2024/12/23 17:25 [medline] PHST- 2024/12/23 17:24 [pubmed] PHST- 2024/12/23 06:10 [entrez] PHST- 2024/11/21 00:00 [pmc-release] AID - 10.7759/cureus.74144 [doi] PST - epublish SO - Cureus. 2024 Nov 21;16(11):e74144. doi: 10.7759/cureus.74144. eCollection 2024 Nov. PMID- 28386141 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 55 IP - 1 DP - 2017 TI - Infrared thermal imaging in connective tissue diseases. PG - 38-43 LID - 10.5114/reum.2017.66686 [doi] AB - Infrared thermal imaging (IRT) is a non-invasive, non-contact technique which allows one to measure and visualize infrared radiation. In medicine, thermal imaging has been used for more than 50 years in various clinical settings, including Raynaud's phenomenon and systemic sclerosis. Imaging and quantification of surface body temperature provides an indirect measure of the microcirculation's overall performance. As such, IRT is capable of confirming the diagnosis of Raynaud's phenomenon, and, with additional cold or heat challenge, of differentiating between the primary and secondary condition. In systemic sclerosis IRT has a potential role in assessing disease activity and monitoring treatment response. Despite certain limitations, thermal imaging can find a place in clinical practice, and with the introduction of small, low-cost infrared cameras, possibly become a part of routine rheumatological evaluation. FAU - Chojnowski, Marek AU - Chojnowski M AD - Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. LA - eng PT - Journal Article PT - Review DEP - 20170322 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC5380771 OTO - NOTNLM OT - Raynaud’s phenomenon OT - systemic sclerosis OT - thermal imaging OT - thermography COIS- The author declares no conflict of interest. EDAT- 2017/04/08 06:00 MHDA- 2017/04/08 06:01 PMCR- 2017/01/01 CRDT- 2017/04/08 06:00 PHST- 2016/10/19 00:00 [received] PHST- 2017/01/30 00:00 [accepted] PHST- 2017/04/08 06:00 [entrez] PHST- 2017/04/08 06:00 [pubmed] PHST- 2017/04/08 06:01 [medline] PHST- 2017/01/01 00:00 [pmc-release] AID - 29676 [pii] AID - 10.5114/reum.2017.66686 [doi] PST - ppublish SO - Reumatologia. 2017;55(1):38-43. doi: 10.5114/reum.2017.66686. Epub 2017 Mar 22. PMID- 42136449 OWN - NLM STAT- Publisher LR - 20260515 IS - 2772-2716 (Electronic) IS - 2772-2708 (Linking) DP - 2026 May 7 TI - Revisiting Raynaud's Phenomenon: Emerging Insights and Evolving Management Strategies. LID - 10.2174/0127722708444234260422070300 [doi] AB - Raynaud's Phenomenon (RP) is a multifactorial vasoconstrictive disease that is associated with recurrent and reversible ischemic crises of the extremities, in most cases, the fingers and toes. Its pathophysiology remains a complex issue involving neurovascular loss of regulation, endothelial dysfunction, increased sympathetic activity, and, in some instances, immune-mediated processes. Significant attention should be paid to the timely and proper identification, as Raynaud's can be the primary diagnosis or the initial symptom of another underlying connective tissue disease. This review is a thorough evaluation of the clinical presentation, diagnostic modalities, and differential diagnosis to help identify and intervene on time. Present management treatment includes non-pharmacological methods, such as thermal protection, quitting smoking, and behavioral modification, as well as pharmacological treatment, including calcium channel blockers, Phosphodiesterase (PDE) inhibitors, prostacyclin analogues, and new targeted agents. Recent innovations and patent literature highlight progress in topical compositions, wearable thermotherapy apparatus, and drug-delivery systems that will help improve peripheral circulation and reduce vasospastic events. Potentially valuable approaches to refractory or severe cases include promising novel therapies, such as botulinum toxin injections, gene-based approaches, nitric-oxide modulating approaches, and rho-kinase inhibitors. Moreover, current changing knowledge about molecular pathways is fueling the quest to design precision-medicine interventions that are patient-specific. The review concludes by outlining future directions that emphasize early diagnosis, integrative therapeutic algorithms, advanced biomedical technologies, and innovation in patented treatment strategies to optimize long-term outcomes for individuals affected by RP. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Kumar, Aman AU - Kumar A AD - Department of Pharmaceutics, ISF College of Pharmacy, GT Road, Moga, 142001, Punjab, India. FAU - Sood, Nayan AU - Sood N AD - Department of Pharmaceutics, ISF College of Pharmacy, GT Road, Moga, 142001, Punjab, India. FAU - Thakur, Ritik Kumar AU - Thakur RK AD - Department of Pharmaceutics, ISF College of Pharmacy, GT Road, Moga, 142001, Punjab, India. FAU - Singh, Nitin AU - Singh N AD - Department of Pharmaceutics, ISF College of Pharmacy, GT Road, Moga, 142001, Punjab, India. FAU - Patel, Preeti AU - Patel P AD - Department of Pharmaceutical Chemistry, ISF College of Pharmacy, GT Road Moga, 142001, Punjab, India. FAU - Kurmi, Balak Das AU - Kurmi BD AUID- ORCID: 0000-0001-8470-6622 AD - Department of Pharmaceutics, ISF College of Pharmacy, GT Road, Moga, 142001, Punjab, India. LA - eng PT - Journal Article DEP - 20260507 PL - Netherlands TA - Recent Adv Inflamm Allergy Drug Discov JT - Recent advances in inflammation & allergy drug discovery JID - 101776469 SB - IM OTO - NOTNLM OT - Raynaud’s phenomenon OT - emerging treatments OT - endothelial dysfunction OT - pharmacological therapy OT - precision medicine. OT - vasospasm EDAT- 2026/05/15 06:29 MHDA- 2026/05/15 06:29 CRDT- 2026/05/15 04:04 PHST- 2025/09/03 00:00 [received] PHST- 2026/01/03 00:00 [revised] PHST- 2026/01/12 00:00 [accepted] PHST- 2026/05/15 06:29 [medline] PHST- 2026/05/15 06:29 [pubmed] PHST- 2026/05/15 04:04 [entrez] AID - RAIAD-EPUB-155323 [pii] AID - 10.2174/0127722708444234260422070300 [doi] PST - aheadofprint SO - Recent Adv Inflamm Allergy Drug Discov. 2026 May 7. doi: 10.2174/0127722708444234260422070300. PMID- 41531633 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260114 LR - 20260116 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 12 DP - 2025 Dec TI - Paraneoplastic Raynaud's Phenomenon: A Case Report. PG - e99075 LID - 10.7759/cureus.99075 [doi] LID - e99075 AB - Raynaud's phenomenon is a vasospastic disorder that may occur as a primary benign condition or as a secondary manifestation of systemic disease. We describe the case of a 63-year-old man, with stage IV squamous cell carcinoma of the left lung undergoing palliative chemotherapy, who developed asymmetric and transient discoloration of the distal upper extremities. The patient's clinical history of active cancer, combined with exclusion of other secondary causes, supported a paraneoplastic etiology. Paraneoplastic Raynaud's phenomenon should be considered in adults with atypical or new-onset vasospastic symptoms, especially in the context of established malignancy. Early recognition is essential to optimize symptom management and enhance understanding of paraneoplastic vascular syndromes associated with lung cancer. CI - Copyright © 2025, Vitó Madureira et al. FAU - Vitó Madureira, Beatriz AU - Vitó Madureira B AD - Internal Medicine, Local Health Unit of Entre Douro e Vouga, Santa Maria da Feira, PRT. FAU - Aranha, Rita AU - Aranha R AD - Oncology, Local Health Unit of Entre Douro e Vouga, Santa Maria da Feira, PRT. FAU - Soares Costa, Rita AU - Soares Costa R AD - Internal Medicine, Local Health Unit of Entre Douro e Vouga, Santa Maria da Feira, PRT. LA - eng PT - Case Reports PT - Journal Article DEP - 20251212 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12791199 OTO - NOTNLM OT - lung cancer OT - oncologic complications OT - paraneoplastic syndromes OT - raynaud’s phenomenon OT - vasospasm COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/01/14 07:05 MHDA- 2026/01/14 07:06 PMCR- 2025/12/12 CRDT- 2026/01/14 04:07 PHST- 2025/12/11 00:00 [accepted] PHST- 2026/01/14 07:06 [medline] PHST- 2026/01/14 07:05 [pubmed] PHST- 2026/01/14 04:07 [entrez] PHST- 2025/12/12 00:00 [pmc-release] AID - 10.7759/cureus.99075 [doi] PST - epublish SO - Cureus. 2025 Dec 12;17(12):e99075. doi: 10.7759/cureus.99075. eCollection 2025 Dec. PMID- 42190748 OWN - NLM STAT- Publisher LR - 20260526 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) DP - 2026 May 25 TI - Impact of Phosphodiesterase-5 Inhibitor Treatment Delay on Secondary Raynaud's Phenomenon Outcomes: A Retrospective Cohort Study. LID - S0190-9622(26)02739-8 [pii] LID - 10.1016/j.jaad.2026.05.056 [doi] FAU - Zappi, Isabella AU - Zappi I AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY; University of Rochester School of Medicine, Rochester, NY. Electronic address: Isabella.Zappi@nyulangone.org. FAU - Maas, Derek AU - Maas D AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Sikora, Michelle AU - Sikora M AD - Department of Internal Medicine, Zucker and Barbara School of Medicine at Hofstra-Lenox Hill Hospital, New York, NY. FAU - Spindler, Archie AU - Spindler A AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Nohria, Ambika AU - Nohria A AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Desai, Deesha D AU - Desai DD AD - Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, PA. FAU - Obijiofor, Chinemelum AU - Obijiofor C AD - Department of Dermatology, Harvard University, Boston, MA. FAU - Richardson, William Mark AU - Richardson WM AD - Department of Dermatology, SUNY Downstate Health Sciences University, Brooklyn, NY. FAU - Shapiro, Jerry AU - Shapiro J AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Femia, Alisa N AU - Femia AN AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Saxena, Amit AU - Saxena A AD - Division of Rheumatology, Department of Medicine, New York University Grossman School of Medicine, New York, NY. FAU - Caplan, Avrom S AU - Caplan AS AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Mazori, Daniel R AU - Mazori DR AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. FAU - Lo Sicco, Kristen I AU - Lo Sicco KI AD - The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, NY. LA - eng PT - Journal Article DEP - 20260525 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM OTO - NOTNLM OT - amputation OT - auto-amputation OT - dermatomyositis OT - gangrene OT - mixed connective tissue disease OT - phosphodiesterase-5 inhibitors OT - rheumatoid arthritis OT - secondary Raynaud’s phenomenon OT - sildenafil OT - systemic lupus erythematosus OT - systemic sclerosis OT - tadalafil OT - treatment delay OT - ulcers EDAT- 2026/05/27 00:30 MHDA- 2026/05/27 00:30 CRDT- 2026/05/26 19:33 PHST- 2026/01/15 00:00 [received] PHST- 2026/05/20 00:00 [revised] PHST- 2026/05/21 00:00 [accepted] PHST- 2026/05/27 00:30 [medline] PHST- 2026/05/27 00:30 [pubmed] PHST- 2026/05/26 19:33 [entrez] AID - S0190-9622(26)02739-8 [pii] AID - 10.1016/j.jaad.2026.05.056 [doi] PST - aheadofprint SO - J Am Acad Dermatol. 2026 May 25:S0190-9622(26)02739-8. doi: 10.1016/j.jaad.2026.05.056. PMID- 36440300 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221129 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 11 DP - 2022 Nov TI - Rapid and Sustained Effect of Ozone Major Autohemotherapy for Raynaud and Hand Edema in Systemic Sclerosis Patient: A Case Report. PG - e31831 LID - 10.7759/cureus.31831 [doi] LID - e31831 AB - Systemic sclerosis (SSc) is a complex disease characterized by vascular injury with endothelial cell and platelet activation, immune dysregulation with inflammatory cytokines and fibroblast activation. The Raynaud phenomenon and puffy hands and fingers are common early manifestations of the disease that have a negative impact on patients' quality of life. Vasodilators such as calcium channel blockers, PDE5 inhibitors, and prostacyclin analogs are recommended treatments, but they often have side effects and are not always effective. Ozone is an oxygen donor, an immunomodulator, an inducer of antioxidant enzymes and the endothelial nitric oxide synthase, a metabolic booster, and a stem cell activator. I report the case of a scleroderma patient treated effectively with autohemotherapy with ozone and a clear reduction of Raynaud's episodes and resolution of the edema of the hands. Furthermore, the capillaroscopic evaluation showed a rapid modification of the microcirculation which remained unchanged for months. Ozone therapy is effective to treat the Raynaud phenomenon and hand edema and should be considered, at least, as a complementary therapy to the standard of care, especially in patients who are unresponsive or with frequent adverse drug reactions. Further studies will be needed to confirm the efficacy of ozone therapy in scleroderma vasculopathy. CI - Copyright © 2022, Galluccio et al. FAU - Galluccio, Felice AU - Galluccio F AD - Rheumatology & Rehabilitation, Fisiotech Lab Studio, Firenze, ITA. AD - Pain Medicine, Morphological Madrid Research Center (MoMaRC), Madrid, ESP. LA - eng PT - Case Reports PT - Journal Article DEP - 20221123 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9685362 OTO - NOTNLM OT - microcirculation and inflammation OT - ozone therapy OT - puffy fingers OT - puffy hands OT - raynaud’s phenomenon OT - systemic scleroderma OT - systemic sclerosis OT - videocapillaroscopy COIS- The authors have declared that no competing interests exist. EDAT- 2022/11/29 06:00 MHDA- 2022/11/29 06:01 PMCR- 2022/11/23 CRDT- 2022/11/28 04:52 PHST- 2022/11/23 00:00 [accepted] PHST- 2022/11/28 04:52 [entrez] PHST- 2022/11/29 06:00 [pubmed] PHST- 2022/11/29 06:01 [medline] PHST- 2022/11/23 00:00 [pmc-release] AID - 10.7759/cureus.31831 [doi] PST - epublish SO - Cureus. 2022 Nov 23;14(11):e31831. doi: 10.7759/cureus.31831. eCollection 2022 Nov. PMID- 24834253 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140516 LR - 20211021 IS - 2008-2258 (Print) IS - 2008-4234 (Electronic) IS - 2008-2258 (Linking) VI - 6 IP - 2 DP - 2013 Spring TI - Crohn's disease of the small bowel, complicated by primary biliary cirrhosis, Hashimoto thyroiditis, and Raynaud's phenomenon: favorable response of all disorders to adalimumab treatment. PG - 101-5 AB - We describe the case of a male patient suffering from long-lasting Crohn's disease of the small bowel who developed thyroiditis Hassimoto, Raynaud's phenomenon, and primary biliary cirrhosis, during the course of the underlying bowel disease. It is not clear whether these co-morbidities appeared coincidentally, or because they share some common immunopathogenetic mechanisms. In this patient, Crohn's disease favorably responded to the treatment with an anti-TNF-α agent (adalimumab). The serum titers of antimitochondrial antibodyies and cholestatic enzymes considerably reduced during the 3-year treatment with the biologic agent. Raynaud's phenomenon, also, completely disappeared. Bearing in mind the possible involvement of TNF-α in the pathogenesis of primary biliary cirrhosis, it could be argued that the clinical and laboratory improvement of liver disease, as well as the reduction in serum titers of antimitochondrial antibodies, might be due to the anti-TNF-α action of adalimumab. We suggest that it would be worth further investigating the role of biologic agents in the treatment of patients with primary biliary cirrhosis. FAU - Triantafillidis, John K AU - Triantafillidis JK AD - Department of Gastroenterology, "Saint Panteleimon" General Hospital, Nicea, Greece. FAU - Durakis, Spyros AU - Durakis S AD - Department of Medicine, "Hipokrateion" Hospital, University of Athens, Medical School, Athens, Greece. FAU - Merikas, Emmanuel AU - Merikas E AD - Department of Gastroenterology, "Saint Panteleimon" General Hospital, Nicea, Greece. LA - eng PT - Case Reports PT - Journal Article PL - Iran TA - Gastroenterol Hepatol Bed Bench JT - Gastroenterology and hepatology from bed to bench JID - 101525875 PMC - PMC4017500 OTO - NOTNLM OT - Crohn's disease OT - Extraintestinal manifestations OT - Inflammatory bowel disease OT - Infliximab OT - Primary biliary cirrhosis EDAT- 2014/05/17 06:00 MHDA- 2014/05/17 06:01 PMCR- 2013/03/01 CRDT- 2014/05/17 06:00 PHST- 2012/11/25 00:00 [received] PHST- 2013/02/14 00:00 [accepted] PHST- 2014/05/17 06:00 [entrez] PHST- 2014/05/17 06:00 [pubmed] PHST- 2014/05/17 06:01 [medline] PHST- 2013/03/01 00:00 [pmc-release] AID - GHFBB-6-101 [pii] PST - ppublish SO - Gastroenterol Hepatol Bed Bench. 2013 Spring;6(2):101-5. PMID- 40718204 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250731 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 6 DP - 2025 Jun TI - A Rare Case of Cold Antibody-Mediated Autoimmune Haemolytic Anaemia in Early Systemic Lupus Erythematosus. PG - e86842 LID - 10.7759/cureus.86842 [doi] LID - e86842 AB - Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterised by autoantibody production, leading to multiorgan inflammation and tissue damage. Autoimmune haemolytic anaemia (AIHA) is a recognised haematologic complication of SLE. This report describes a 16-year-old female who presented with four weeks of generalised weakness, polyarthralgia, recurrent syncopal episodes, and Raynaud's phenomenon. This case highlights the diagnostic challenges of SLE presenting with AIHA, underscoring the importance of prompt, comprehensive autoimmune and haematologic evaluation in adolescents with unexplained anaemia and multi-system involvement. CI - Copyright © 2025, Rajaram et al. FAU - Rajaram, Harisanth AU - Rajaram H AD - Internal Medicine, Royal Berkshire NHS Foundation Trust, Reading, GBR. FAU - Ganegoda, Sherwin AU - Ganegoda S AD - Internal Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, GBR. FAU - Andev, Rajinder AU - Andev R AD - Rheumatology, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, GBR. LA - eng PT - Case Reports PT - Journal Article DEP - 20250627 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12296916 OTO - NOTNLM OT - autoimmune hemolytic anemia OT - cold agglutinin disease OT - coombs-positive anaemia OT - corticosteroid therapy OT - pediatric systemic lupus erythematosus (psle) OT - raynaud's phenomenon OT - systemic lupus erythromatosus COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: Dr. Rajinder Andev declare(s) personal fees from Novartis. He received honoraria and speaker fees from Novartis. Dr. Rajinder Andev declare(s) personal fees from UCB. He received honoraria from UCB. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/07/28 18:51 MHDA- 2025/07/28 18:52 PMCR- 2025/06/27 CRDT- 2025/07/28 05:00 PHST- 2025/06/27 00:00 [accepted] PHST- 2025/07/28 18:52 [medline] PHST- 2025/07/28 18:51 [pubmed] PHST- 2025/07/28 05:00 [entrez] PHST- 2025/06/27 00:00 [pmc-release] AID - 10.7759/cureus.86842 [doi] PST - epublish SO - Cureus. 2025 Jun 27;17(6):e86842. doi: 10.7759/cureus.86842. eCollection 2025 Jun. PMID- 28775907 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2090-6986 (Print) IS - 2090-6994 (Electronic) IS - 2090-6994 (Linking) VI - 2017 DP - 2017 TI - Bilateral Brachial Artery Disease Presenting with Features of Raynaud's Phenomenon: A Case Report and Review of the Literature. PG - 7461082 LID - 10.1155/2017/7461082 [doi] LID - 7461082 AB - OBJECTIVE: To present a case of bilateral brachial artery disease presenting with features of Raynaud's phenomenon which was successfully treated with angioplasty and stenting, together with a review of the relevant literature. CASE: A 71-year-old female presented with a one-year history of intermittent pallor of both hands precipitated with cold objects. On examination, bilateral radial pulses were reduced. Prior photos showed pallor of the distal aspect of both palms. Angiogram showed high grade stenosis of the right brachial artery and focal occlusion with likely dissection of the left brachial artery. She underwent angioplasty and stenting for both lesions. She was asymptomatic without further episodes of Raynaud's phenomenon after five months on dual antiplatelet therapy. Upper-extremity vascular stenosis is uncommon. Structural changes in the vessel wall can cause vasospastic attacks, a mechanism described in secondary Raynaud's phenomenon. We hypothesize that these attacks may have been precipitated by the bilateral brachial artery disease. Furthermore, resolution of the symptoms after stent further supports our theory. CONCLUSION: Bilateral brachial artery disease is uncommon. Physicians should consider this in patients presenting with Raynaud's phenomenon. Brachial artery stenosis and occlusion is a treatable disease with good symptomatic outcomes after angioplasty and stenting. FAU - Seegobin, Karan AU - Seegobin K AUID- ORCID: 0000-0002-2739-7357 AD - Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA. FAU - Lyons, Brittany AU - Lyons B AUID- ORCID: 0000-0002-8129-9723 AD - Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA. FAU - Maharaj, Satish AU - Maharaj S AUID- ORCID: 0000-0002-2872-3011 AD - Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA. FAU - Baldeo, Cherisse AU - Baldeo C AUID- ORCID: 0000-0002-3326-012X AD - Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA. FAU - Reddy, Pramod AU - Reddy P AD - Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA. FAU - Cunningham, James AU - Cunningham J AUID- ORCID: 0000-0002-6258-2048 AD - Department of Interventional Radiology, University of Florida College of Medicine, Jacksonville, FL 32209, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20170710 PL - United States TA - Case Rep Vasc Med JT - Case reports in vascular medicine JID - 101585357 PMC - PMC5523346 EDAT- 2017/08/05 06:00 MHDA- 2017/08/05 06:01 PMCR- 2017/07/10 CRDT- 2017/08/05 06:00 PHST- 2017/04/01 00:00 [received] PHST- 2017/04/19 00:00 [accepted] PHST- 2017/08/05 06:00 [entrez] PHST- 2017/08/05 06:00 [pubmed] PHST- 2017/08/05 06:01 [medline] PHST- 2017/07/10 00:00 [pmc-release] AID - 10.1155/2017/7461082 [doi] PST - ppublish SO - Case Rep Vasc Med. 2017;2017:7461082. doi: 10.1155/2017/7461082. Epub 2017 Jul 10. PMID- 41393621 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251215 LR - 20251217 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 11 DP - 2025 Nov TI - A Clinical Case of Anti-synthetase Syndrome: Polysymptomatic and Underdiagnosed. PG - e96690 LID - 10.7759/cureus.96690 [doi] LID - e96690 AB - Antisynthetase syndrome (ASSD) encompasses a group of autoimmune diseases associated with the formation of antibodies against aminoacyl-tRNA synthetases (ARS). The medical literature describes a constellation of symptoms, including myositis, arthritis, Raynaud's phenomenon, interstitial lung disease (ILD), fever, skin rash, or "mechanic's hands." We present a case of a 51-year-old man admitted with SAS with a consumptive clinical picture, rhabdomyolysis, ILD, and myositis. Positive anti-PL-7 and anti-nuclear antibodies (ANA) were detected. After diagnostic confirmation, the patient was treated with high doses of methylprednisolone and subsequently with cyclophosphamide, with a good response to immunosuppressive therapy. This clinical case is of great importance given the rarity of the pathology and the diagnostic difficulty of a disease with such a wide range of clinical manifestations. CI - Copyright © 2025, Oliveira et al. FAU - Oliveira, Nuno AU - Oliveira N AD - Internal Medicine, Unidade Local de Saude Do Oeste - Unidade Caldas da Rainha, Caldas da Rainha, PRT. FAU - San Martin, Francisco AU - San Martin F AD - Internal Medicine, Unidade Local de Saúde do Oeste - Unidade Caldas da Rainha, Caldas da Rainha, PRT. FAU - Amorim, Rosa AU - Amorim R AD - Internal Medicine, Unidade Local de Saúde do Oeste - Unidade Caldas da Rainha, Caldas da Rainha, PRT. LA - eng PT - Case Reports PT - Journal Article DEP - 20251112 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12700491 OTO - NOTNLM OT - anti-pl-7 OT - antisynthetase syndrome OT - interstitial lung disease OT - raynaud's OT - rhabdomyolysis COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/12/15 13:09 MHDA- 2025/12/15 13:10 PMCR- 2025/11/12 CRDT- 2025/12/15 06:11 PHST- 2025/11/11 00:00 [accepted] PHST- 2025/12/15 13:10 [medline] PHST- 2025/12/15 13:09 [pubmed] PHST- 2025/12/15 06:11 [entrez] PHST- 2025/11/12 00:00 [pmc-release] AID - 10.7759/cureus.96690 [doi] PST - epublish SO - Cureus. 2025 Nov 12;17(11):e96690. doi: 10.7759/cureus.96690. eCollection 2025 Nov. PMID- 37568970 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240923 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 13 IP - 15 DP - 2023 Aug 5 TI - Optical Coherence Tomography Angiography Is a Useful Tool for Distinguishing Primary Raynaud's Phenomenon from Systemic Sclerosis and/or Very Early Disease of Systemic Sclerosis. LID - 10.3390/diagnostics13152607 [doi] LID - 2607 AB - This cross-sectional study aimed to compare optical coherence tomography angiography (OCT-A) findings in patients with primary Raynaud's phenomenon (PRP; n = 22), very early disease of systemic sclerosis (VEDOSS; n = 19), and systemic sclerosis (SSc; 25 patients with limited cutaneous SSc (lcSSc) and 13 patients with diffuse cutaneous SSc (dcSSc)). Whole, parafoveal, and perifoveal superficial capillary plexus (SCP) vessel densities (VDs), deep capillary plexus VDs, and whole, inside, and peripapillary VDs were significantly higher in the PRP group (p < 0.001). In the lcSSc group, the FAZ perimeter was significantly higher than that in the VEDOSS group (p = 0.017). Retinal nerve fiber layer VDs were significantly lower in the lcSSc group than in the PRP and VEDOSS groups (p < 0.001). The whole and peripapillary optic disc VDs of the VEDOSS group were significantly higher than in the lcSSc group (p < 0.001). Whole SCP VDs (94.74% sensitivity, 100.00% specificity) and parafoveal SCP VDs (89.47% sensitivity, 100.00% specificity) showed the best performance in distinguishing patients with SSc from those with PRP. OCT-A seems to have potential diagnostic value in differentiating patients with PRP from patients with SSc and VEDOSS, and there is potential value in assessing prognostic roles, since findings from OCT-A images could be early indicators of retinal vascular injury long before overt SSc symptoms develop. FAU - Erturk, Adem AU - Erturk A AUID- ORCID: 0000-0001-8882-0692 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar 03030, Turkey. FAU - Erogul, Ozgur AU - Erogul O AUID- ORCID: 0000-0002-0875-1517 AD - Department of Ophthalmology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar 03030, Turkey. FAU - Kasikci, Murat AU - Kasikci M AD - Department of Ophthalmology, Mugla Training and Research Hospital, Mugla 48000, Turkey. LA - eng PT - Journal Article DEP - 20230805 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10417700 OTO - NOTNLM OT - Raynaud’s phenomenon OT - ocular microvasculature OT - optical coherence tomography angiography OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2023/08/12 10:49 MHDA- 2023/08/12 10:50 PMCR- 2023/08/05 CRDT- 2023/08/12 01:05 PHST- 2023/06/23 00:00 [received] PHST- 2023/07/20 00:00 [revised] PHST- 2023/07/29 00:00 [accepted] PHST- 2023/08/12 10:50 [medline] PHST- 2023/08/12 10:49 [pubmed] PHST- 2023/08/12 01:05 [entrez] PHST- 2023/08/05 00:00 [pmc-release] AID - diagnostics13152607 [pii] AID - diagnostics-13-02607 [pii] AID - 10.3390/diagnostics13152607 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Aug 5;13(15):2607. doi: 10.3390/diagnostics13152607. PMID- 33632387 OWN - NLM STAT- Publisher LR - 20240222 IS - 1866-0452 (Electronic) IS - 1866-0452 (Linking) VI - 118 IP - Forthcoming DP - 2021 Apr 9 TI - Raynaud's Phenomenon: A Vascular Acrosyndrome That Requires Long-Term Care. PG - 273-80 LID - arztebl.m2021.0023 [pii] LID - 10.3238/arztebl.m2021.0023 [doi] AB - BACKGROUND: Some 5-10% of the German population are affected by Raynaud's phenomenon (RP). In around 10-20% of cases RP arises from an underlying disease, most commonly a connective tissue disease. This review encompasses the diagnosis and differential diagnosis of RP and examines the efficacy of the currently available pharmaceutical and non-pharmaceutical treatment options. METHODS: We conducted a selective literature search in PubMed using the search terms "Raynaud's phenomenon", "Raynaud's syndrome," "vasospasm," "vascular acrosyndrome," and "systemic sclerosis," together with a search of the Cochrane Database of Systematic Reviews up to April 2020. RESULTS: Raynaud's phenomenon mainly affects the fingers or toes and is typically triggered by cold or emotional stressors. The most important diagnostic steps are demonstration of a tendency towards digital vasospasm, exclusion of occlusions in the afferent arteries and acral vessels, nail-fold capillaroscopy, and determination of autoantibody status. Tumor screening should be arranged in the presence of B symptoms or first manifestation of RP in old age. The onset of RP in childhood is a rare occurrence and points to a secondary origin. The principal options for treatment are protection against cold and administration of calcium antagonists, which reduces the occurrence of RP by around 20-40 %. The treatment of RP in patients with systemic sclerosis is described in the recommendations of the European League Against Rheumatism (EULAR). CONCLUSION: At onset or after years of latency, patients with Raynaud phenomenon may have an underlying disease (most commonly a connective tissue disease). Long-term specialist care is necessary for asymptomatic patients with risk factors and for those with clinically manifest symptoms of an underlying condition alike. FAU - Klein-Weigel, Peter AU - Klein-Weigel P FAU - Sander, Oliver AU - Sander O FAU - Reinhold, Simone AU - Reinhold S FAU - Nielitz, Jessica AU - Nielitz J FAU - Steindl, Julia AU - Steindl J FAU - Richter, Jutta AU - Richter J LA - eng PT - Journal Article DEP - 20210409 PL - Germany TA - Dtsch Arztebl Int JT - Deutsches Arzteblatt international JID - 101475967 SB - IM CIN - Dtsch Arztebl Int. 2021 Sep 6;118I processed the file.I'm waiting for feedback tomorrow(35-36):604. doi: 10.3238/arztebl.m2021.0281. PMID: 34789377 CIN - Dtsch Arztebl Int. 2021 Sep 6;118I processed the file.I'm waiting for feedback tomorrow(35-36):604. doi: 10.3238/arztebl.m2021.0283. PMID: 34789378 CIN - Dtsch Arztebl Int. 2021 Sep 6;118I processed the file.I'm waiting for feedback tomorrow(35-36):605. doi: 10.3238/arztebl.m2021.0282. PMID: 34789379 PMC - PMC8287761 EDAT- 2021/02/27 06:00 MHDA- 2021/02/27 06:00 PMCR- 2021/04/01 CRDT- 2021/02/26 05:40 PHST- 2021/02/26 05:40 [entrez] PHST- 2021/02/27 06:00 [pubmed] PHST- 2021/02/27 06:00 [medline] PHST- 2021/04/01 00:00 [pmc-release] AID - arztebl.m2021.0023 [pii] AID - 10.3238/arztebl.m2021.0023 [doi] PST - aheadofprint SO - Dtsch Arztebl Int. 2021 Apr 9;118(Forthcoming):273-80. doi: 10.3238/arztebl.m2021.0023. PMID- 24119054 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140926 LR - 20200930 IS - 1386-6346 (Print) IS - 1386-6346 (Linking) VI - 44 IP - 10 DP - 2014 Oct TI - Evaluation of nail fold capillaroscopy findings in patients with primary biliary cirrhosis. PG - E129-36 LID - 10.1111/hepr.12255 [doi] AB - AIM: Some patients with primary biliary cirrhosis (PBC) experience Raynaud's phenomenon. The objective of this study was to clarify the relationships between nail fold capillaroscopy findings and clinical presentations of PBC. METHODS: A total of 70 patients with PBC and 57 patients with non-PBC liver diseases, including 44 patients with chronic viral hepatic disease, eight with autoimmune hepatitis and five with non-alcoholic fatty liver disease, were included in this study. Nail fold capillaroscopy findings were classified as normal or abnormal and were further graded as mild, moderate or severe, and the relationships between frequency of abnormal blood vessel and their clinical presentations were examined. RESULTS: The frequency of abnormal nail fold capillaroscopy findings was significantly higher in PBC patients (54.3%) than in patients with non-PBC liver disease (13.8%) (P < 0.01). These abnormal findings observed in PBC patients were graded as mild in 15 patients, moderate in 18 patients and severe in five patients. Significantly more PBC patients with abnormal capillaroscopy findings (19/38, 50%) were positive for anticentromere antibody than were those with normal capillaroscopy findings (3/32, 9.4%) (P < 0.01). CONCLUSION: PBC patients had significantly higher frequency of abnormal nail fold capillaroscopy findings than did patients with non-PBC liver disease. CI - © 2013 The Japan Society of Hepatology. FAU - Monoe, Kyoko AU - Monoe K AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Takahashi, Atsushi AU - Takahashi A FAU - Abe, Kazumichi AU - Abe K FAU - Kanno, Yukiko AU - Kanno Y FAU - Watanabe, Hiroshi AU - Watanabe H FAU - Ohira, Hiromasa AU - Ohira H LA - eng PT - Journal Article DEP - 20131108 PL - Netherlands TA - Hepatol Res JT - Hepatology research : the official journal of the Japan Society of Hepatology JID - 9711801 OTO - NOTNLM OT - Raynaud's phenomenon OT - anticentromere antibody OT - nail fold capillaroscopy OT - primary biliary cirrhosis EDAT- 2013/10/15 06:00 MHDA- 2013/10/15 06:01 CRDT- 2013/10/15 06:00 PHST- 2013/08/21 00:00 [received] PHST- 2013/09/26 00:00 [revised] PHST- 2013/09/30 00:00 [accepted] PHST- 2013/10/15 06:00 [entrez] PHST- 2013/10/15 06:00 [pubmed] PHST- 2013/10/15 06:01 [medline] AID - 10.1111/hepr.12255 [doi] PST - ppublish SO - Hepatol Res. 2014 Oct;44(10):E129-36. doi: 10.1111/hepr.12255. Epub 2013 Nov 8. PMID- 33736700 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210323 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 5 IP - 1 DP - 2021 Mar 19 TI - Capillaroscopy as a diagnostic tool in the diagnosis of mixed connective tissue disease (MCTD): a case report. PG - 9 LID - 10.1186/s41927-021-00179-2 [doi] LID - 9 AB - BACKGROUND: The concept of mixed connective tissue disease (MCTD) as a unique connective tissue disease has endured for half a century. Disease onset can be in adulthood (MCTD) or of juvenile onset (jMCTD) and is characterized by overlapping features of systemic lupus erythematosus (SLE), polymyositis or dermatomyositis (PM/DM) and systemic sclerosis (SSc). No universally accepted classification criteria for MCTD exists, however agreed upon overlapping disease features include the presence of high titers of U1 small nuclear ribonucleoprotein particle antibodies (U1snRNP) in the peripheral blood, Raynaud's phenomenon, synovitis, myositis and swollen hands or fingers. Characteristic capillaroscopy findings are commonly seen in MCTD and jMCTD, which may represent a crucial and key clue for classification as well as prognosis in these patients. CASE PRESENTATION: We present a young male patient, with symptom onset as early as age 13, who was diagnosed with MCTD at age 16 and found to have high titers of anti-U1snRNP antibodies, Raynaud's phenomenon, synovitis, and swollen hands and fingers. Most interestingly, his video capillaroscopy at diagnosis was abnormal and revealed an active SSc-like pattern. His presentation and course are described. CONCLUSIONS: We conclude that based on existing data, and as highlighted by this case presentation, nailfold video capillaroscopy should be included as an early screening tool for the detection of microangiopathy in patients with the diagnosis MCTD and jMCTD. Additionally, given its prevalence in this population at disease diagnosis, we recommend consideration be given to nailfold video capillaroscopy as a potentially important classification criteria and prognostic tool for jMCTD and MCTD. FAU - Radić, Mislav AU - Radić M AD - Department of Rheumatology and Clınıcal Immunollogy, University of Split, Split, Croatia. FAU - Overbury, Rebecca S AU - Overbury RS AUID- ORCID: 0000-0003-3008-9432 AD - Department of Internal Medicine, Division of Rheumatology, University of Utah, 30 N 1900 E, Ste 4B200, UT, 84132, Salt Lake City, USA. rebecca.overbury@hsc.utah.edu. LA - eng PT - Journal Article DEP - 20210319 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC7977576 OTO - NOTNLM OT - Capillaroscopy OT - Classification criteria OT - Juvenile mixed connective tissue disease OT - Mixed connective tissue disease OT - Raynaud’s phenomenon COIS- The authors declare that they have no competing interests. EDAT- 2021/03/20 06:00 MHDA- 2021/03/20 06:01 PMCR- 2021/03/19 CRDT- 2021/03/19 05:53 PHST- 2020/07/21 00:00 [received] PHST- 2021/01/28 00:00 [accepted] PHST- 2021/03/19 05:53 [entrez] PHST- 2021/03/20 06:00 [pubmed] PHST- 2021/03/20 06:01 [medline] PHST- 2021/03/19 00:00 [pmc-release] AID - 10.1186/s41927-021-00179-2 [pii] AID - 179 [pii] AID - 10.1186/s41927-021-00179-2 [doi] PST - epublish SO - BMC Rheumatol. 2021 Mar 19;5(1):9. doi: 10.1186/s41927-021-00179-2. PMID- 19180481 OWN - NLM STAT- MEDLINE DCOM- 20090401 LR - 20250529 IS - 0004-3591 (Print) IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 60 IP - 2 DP - 2009 Feb TI - B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis. PG - 578-83 LID - 10.1002/art.24249 [doi] AB - OBJECTIVE: To determine the safety of rituximab, to provide preliminary data regarding the potential efficacy of rituximab, and to investigate the effects of rituximab on autoimmunity and fibrosis in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Fifteen patients with dcSSc, all of whom experienced their first non-Raynaud's disease-associated disease manifestation within 18 months of trial entry, were recruited to receive 2 intravenous doses of rituximab (1,000 mg), administered 2 weeks apart. Safety, clinical, and exploratory outcomes were evaluated at baseline and at 6 months. The primary outcome was the change in the modified Rodnan skin thickness score (MRSS) at 6 months compared with baseline. RESULTS: Adverse events included frequent infusion reactions and rare infections (urinary tract infection and dental abscess occurred in 1 patient each). The mean change in the MRSS between baseline and 6 months was not significant. Results of pulmonary function tests and other measures of major organ involvement were stable. The modest B cell infiltrates that were present in most skin biopsy specimens at baseline were completely depleted at 6 months in most patients. Autoantibody titers showed only modest and variable changes after treatment. CONCLUSION: In this pilot study, treatment with rituximab appeared to be safe and well tolerated among patients with dcSSc. Rituximab treatment resulted in both depletion of circulating B cells and depletion of dermal B cells but had little effect on the levels of SSc-associated autoantibodies. Rituximab treatment did not appear to result in a significant beneficial effect on skin disease. The potential efficacy of rituximab in other organs such as the lung could not be clearly evaluated in this small open-label trial. FAU - Lafyatis, Robert AU - Lafyatis R AD - Boston University School of Medicine, Boston, Massachusetts 02118, USA. lafyatis@bu.edu FAU - Kissin, Eugene AU - Kissin E FAU - York, Michael AU - York M FAU - Farina, Giuseppina AU - Farina G FAU - Viger, Kerry AU - Viger K FAU - Fritzler, Marvin J AU - Fritzler MJ FAU - Merkel, Peter A AU - Merkel PA FAU - Simms, Robert W AU - Simms RW LA - eng GR - M01-RR-00533/RR/NCRR NIH HHS/United States GR - M01 RR001066/RR/NCRR NIH HHS/United States GR - U01-AR-055063/AR/NIAMS NIH HHS/United States GR - U01 AR055063/AR/NIAMS NIH HHS/United States GR - M01 RR000533/RR/NCRR NIH HHS/United States PT - Clinical Trial PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Autoantibodies) RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) SB - IM CIN - Curr Rheumatol Rep. 2011 Feb;13(1):1-3. doi: 10.1007/s11926-010-0150-x. PMID: 21107765 MH - Adult MH - Antibodies, Monoclonal/*therapeutic use MH - Antibodies, Monoclonal, Murine-Derived MH - Autoantibodies/blood MH - Autoimmunity/drug effects MH - B-Lymphocytes/*drug effects/pathology MH - Cell Survival/drug effects MH - Female MH - Humans MH - Immunologic Factors/*therapeutic use MH - Injections, Intravenous MH - Leukocyte Count MH - Lymphocyte Depletion MH - Male MH - Middle Aged MH - Pilot Projects MH - Respiratory Function Tests MH - Rituximab MH - Scleroderma, Diffuse/*drug therapy/immunology/physiopathology MH - Skin/drug effects/pathology PMC - PMC2637937 MID - NIHMS87469 EDAT- 2009/01/31 09:00 MHDA- 2009/04/02 09:00 PMCR- 2010/02/01 CRDT- 2009/01/31 09:00 PHST- 2009/01/31 09:00 [entrez] PHST- 2009/01/31 09:00 [pubmed] PHST- 2009/04/02 09:00 [medline] PHST- 2010/02/01 00:00 [pmc-release] AID - 10.1002/art.24249 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Feb;60(2):578-83. doi: 10.1002/art.24249. PMID- 33758813 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220421 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 35 IP - 4 DP - 2020 Dec TI - Pattern of Nailfold Capillaroscopy in Patients With Systemic Lupus Erythematosus. PG - 568-574 LID - 10.46497/ArchRheumatol.2020.7763 [doi] AB - OBJECTIVES: This study aims to assess the nailfold capillary changes in patients with systemic lupus erythematosus (SLE), particularly among those with Raynaud's phenomenon (RP), and the correlation between nailfold capillary changes and autoantibodies and disease activity. PATIENTS AND METHODS: A total of 85 patients (9 males, 76 females; median age 31 years; range, 15 to 58 years) with newly diagnosed SLE were selected between July 2016 and July 2018 from our hospital. Disease activity was scored by the SLE Disease Activity Index. Nailfold capillaroscopy (NFC) was performed in all patients. RESULTS: Normal pattern, non-specific pattern, and scleroderma pattern were found in 13 (15.3%), 64 (75.3%), and eight (9.4%) patients, respectively. There was no significant difference between anti-double stranded deoxyribonucleic acid, anti-Smith antibodies, and low complements (all p>0.05), while significant differences of NFC pattern were found between low disease activity and high disease activity (p=0.002). RP was present in 31.7% of SLE patients, and the NFC findings in SLE patients with and without RP were significantly different in dilatation (81.5% vs. 14.0%). CONCLUSION: The results of our study showed that capillary changes were very common in patients with SLE, which seem to associate with disease activity and RP condition. CI - Copyright © 2020, Turkish League Against Rheumatism. FAU - Zhao, Ting AU - Zhao T AD - Department of Rheumatology and Immunology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou, China. FAU - Lin, Fu-An AU - Lin FA AD - Department of Rheumatology and Immunology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou, China. FAU - Chen, Hong-Pu AU - Chen HP AD - Department of Rheumatology and Immunology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou, China. LA - eng PT - Journal Article DEP - 20200420 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC7945704 OTO - NOTNLM OT - Nailfold capillaroscopy OT - Raynaud’s phenomenon OT - systemic lupus erythematosus COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2021/03/25 06:00 MHDA- 2021/03/25 06:01 PMCR- 2020/04/20 CRDT- 2021/03/24 06:49 PHST- 2019/08/28 00:00 [received] PHST- 2019/12/02 00:00 [accepted] PHST- 2021/03/24 06:49 [entrez] PHST- 2021/03/25 06:00 [pubmed] PHST- 2021/03/25 06:01 [medline] PHST- 2020/04/20 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2020.7763 [doi] PST - epublish SO - Arch Rheumatol. 2020 Apr 20;35(4):568-574. doi: 10.46497/ArchRheumatol.2020.7763. eCollection 2020 Dec. PMID- 17911571 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20080118 LR - 20071003 IS - 1531-0035 (Print) IS - 1521-5768 (Linking) VI - 19 IP - 3 DP - 2007 Sep TI - Commentary. Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease. Results of ten years of prospective surveillance. PG - 341-2 FAU - Moneta, Gregory L AU - Moneta GL AD - Oregon Health & Science University Portland, Oregon, USA. LA - eng PT - Comment PT - Journal Article PL - United States TA - Perspect Vasc Surg Endovasc Ther JT - Perspectives in vascular surgery and endovascular therapy JID - 100970607 CON - Arthritis Rheum. 2006 Jun;54(6):1974-81. doi: 10.1002/art.21912. PMID: 16732585 EDAT- 2007/10/04 09:00 MHDA- 2007/10/04 09:01 CRDT- 2007/10/04 09:00 PHST- 2007/10/04 09:00 [pubmed] PHST- 2007/10/04 09:01 [medline] PHST- 2007/10/04 09:00 [entrez] AID - 19/3/341 [pii] AID - 10.1177/1531003507301668 [doi] PST - ppublish SO - Perspect Vasc Surg Endovasc Ther. 2007 Sep;19(3):341-2. doi: 10.1177/1531003507301668. PMID- 30485025 STAT- Publisher ISBN- 978-0-9871718-2-5 PB - University of Adelaide Press DP - 2011 TI - Pathophysiology and Principles of Management of Vasculitides and Raynaud’s Syndrome. BTI - Mechanisms of Vascular Disease: A Reference Book for Vascular Specialists CP - 16 CI - © The Contributors 2011. FED - Fitridge, Robert ED - Fitridge R AD - The University of Adelaide, The Queen Elizabeth Hospital, Woodville, Australia FED - Thompson, Matthew ED - Thompson M AD - St George’s Hospital Medical School, London, UK FAU - Veller, Martin AU - Veller M LA - eng PT - Review PT - Book Chapter PL - Adelaide (AU) EDAT- 2011/01/01 00:00 CRDT- 2011/01/01 00:00 AID - NBK534271 [bookaccession] PMID- 21430889 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 55 IP - 4 DP - 2010 Oct TI - Dexamethasone pulse therapy in patients of systemic sclerosis: is it a viable proposition? A study from kashmir. PG - 355-8 LID - 10.4103/0019-5154.74543 [doi] AB - BACKGROUND: Systemic sclerosis is a multisystemic autoimmune disorder. Intravenous dexamethasone pulse therapy has been used since 1998. AIM: The aim wasto report the beneficial effects of dexamethasone pulse in patients of systemic sclerosis vis-à-vis the side effects. MATERIALS AND METHODS: Forty-seven patients of systemic sclerosis were included. After looking at the history and physical examination, the patients were submitted to various relevant investigations. Clinical scoring of the patient was done at baseline and 6-month interval according to Furst's organ indices score. RESULTS: A total of47 patients of systemic sclerosis were included (45 females, 2 males). In majority, acrosclerosis was seen. Severe sclerosis and contractures were seen in two patients. Moderate proteinuria, restrictive lung disease, dysphagia, and valvular heart involvement were seen.A total of13 patients on dexamethasone pulse therapy developed tuberculosis. Improvement in skin scoring and decreased severity of Raynaud's phenomenon was seen. No improvement in dysphagia, severe vascular symptoms, or restrictive lung disease was seen. CONCLUSION: Thus, beneficial effects of dexamethasone pulse therapy seem to be merely cosmetic. FAU - Sameem, Farah AU - Sameem F AD - Department of Dermatology, STD and Leprosy, Government Medical College, Srinagar, Jammu and Kashmir, India. FAU - Hassan, Iffat AU - Hassan I FAU - Ahmad, Qazi Masood AU - Ahmad QM FAU - Khan, Dilshad AU - Khan D FAU - Majeed, Imran AU - Majeed I FAU - Kamili, M A AU - Kamili MA FAU - Shah, Parvaiz A AU - Shah PA LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3051296 OTO - NOTNLM OT - Dexamethasone pulse OT - Raynaud's phenomenon OT - systemic sclerosis COIS- Conflict of Interest: Nil. EDAT- 2011/03/25 06:00 MHDA- 2011/03/25 06:01 PMCR- 2010/10/01 CRDT- 2011/03/25 06:00 PHST- 2011/03/25 06:00 [entrez] PHST- 2011/03/25 06:00 [pubmed] PHST- 2011/03/25 06:01 [medline] PHST- 2010/10/01 00:00 [pmc-release] AID - IJD-55-355 [pii] AID - 10.4103/0019-5154.74543 [doi] PST - ppublish SO - Indian J Dermatol. 2010 Oct;55(4):355-8. doi: 10.4103/0019-5154.74543. PMID- 36595033 OWN - NLM STAT- MEDLINE DCOM- 20230116 LR - 20230116 IS - 2731-7013 (Electronic) IS - 2731-7005 (Print) IS - 2731-7005 (Linking) VI - 74 IP - 1 DP - 2023 Jan TI - [Nailfold capillaroscopy-Principles and clinical application]. PG - 55-64 LID - 10.1007/s00105-022-05091-5 [doi] AB - Nailfold capillaroscopy is a rapid and easily applicable differential diagnostic technique that allows direct visualization of the microcirculation. Abnormal findings in nailfold capillaroscopy are closely associated with connective tissue diseases, such as systemic sclerosis. The clinical manifestation of impaired microcirculation is Raynaud's phenomenon, which is a classical symptom of connective tissue diseases. Nailfold capillaroscopy is increasingly used in various fields of medicine, therefore it is important to define methods for the acquisition and analysis of the results of nailfold capillary and to have a uniform definition of abnormal capillaries. This article discusses image acquisition and analysis, various capillaroscopic techniques, normal and abnormal capillaroscopic features and their significance, scoring systems and reliability of image acquisition and interpretation. CI - © 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature. FAU - Hasseli-Fräbel, R AU - Hasseli-Fräbel R AD - Medizinische Klinik und Poliklinik II, Universitätsklinikum Gießen, Justus-Liebig-Universität Gießen, Gießen, Deutschland. FAU - Hermann, W AU - Hermann W AD - Abteilung für Rheumatologie und Klinische Immunologie, Kerckhoff-Klinik GmbH, Benekestr. 2-8, 61231, Bad Nauheim, Deutschland. w.hermann@kerckhoff-klinik.de. FAU - Sander, O AU - Sander O AD - Klinik für Rheumatologie, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität, Düsseldorf, Deutschland. FAU - Triantafyllias, K AU - Triantafyllias K AD - RZ Rheumazentrum Rheinland-Pfalz, Bad Kreuznach, Deutschland. LA - ger PT - English Abstract PT - Journal Article TT - Kapillarmikroskopie – Grundlagen und klinische Anwendung. PL - Germany TA - Dermatologie (Heidelb) JT - Dermatologie (Heidelberg, Germany) JID - 9918384885206676 SB - IM MH - Humans MH - Nails/diagnostic imaging MH - Microscopic Angioscopy/methods MH - Reproducibility of Results MH - *Connective Tissue Diseases/diagnosis MH - *Scleroderma, Systemic/diagnosis PMC - PMC9808688 OTO - NOTNLM OT - Connective tissue disease OT - Inflammatory rheumatic diseases OT - Microangiopathy OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2023/01/04 06:00 MHDA- 2023/01/17 06:00 PMCR- 2023/01/03 CRDT- 2023/01/03 11:14 PHST- 2023/01/04 06:00 [pubmed] PHST- 2023/01/17 06:00 [medline] PHST- 2023/01/03 11:14 [entrez] PHST- 2023/01/03 00:00 [pmc-release] AID - 10.1007/s00105-022-05091-5 [pii] AID - 5091 [pii] AID - 10.1007/s00105-022-05091-5 [doi] PST - ppublish SO - Dermatologie (Heidelb). 2023 Jan;74(1):55-64. doi: 10.1007/s00105-022-05091-5. PMID- 41996261 OWN - NLM STAT- MEDLINE DCOM- 20260417 LR - 20260417 IS - 1210-7875 (Print) IS - 1210-7875 (Linking) VI - 62 IP - 1 DP - 2026 TI - Castleman-Like Lymphadenopathy in a Patient with Mixed Connective Tissue Disease: A Case Report and Review of the Literature. PG - 50-57 AB - Differentiating reactive lymphadenopathies in the context of autoimmune disease from Idiopathic Multicentric Castleman Disease (iMCD) poses a significant diagnostic challenge. Castleman-like histological features have been described in various autoimmune disorders, necessitating a strict and comprehensive integration of clinical and laboratory findings to reach the correct diagnosis. Although the Castleman Disease Collaborative Network (CDCN) consensus guidelines list several autoimmune conditions as exclusion criteria for an iMCD diagnosis, mixed connective tissue disease (MCTD) is not currently among them. We report the case of a  77-year-old woman presenting with fatigue, Raynaud's  phenomenon, sclerodactyly, mild generalized lymphadenopathy, in whom the lymph node biopsy revealed a Castleman-like histology. The absence of systemic inflammatory symptoms and the presence of high-titer anti-U1- RNP antibodies were, however, inconsistent with iMCD, favouring the diagnosis of a reactive Castleman-like lymphadenitis secondary to MCTD. This report highlights that Castleman-like lymphadenopathy can occur in MCTD, closely mimicking iMCD. Therefore, in patients with autoimmune diseases not explicitly listed among the CDCN exclusion criteria, comprehensive clinicopathological integration is essential to avoid misdiagnosis and potentially inappropriate antiIL-6-based therapy. FAU - Rogges, Evelina AU - Rogges E FAU - Pelliccia, Sabrina AU - Pelliccia S FAU - Lopez, Gianluca AU - Lopez G FAU - Soscia, Roberta AU - Soscia R FAU - Di Napoli, Arianna AU - Di Napoli A LA - eng PT - Case Reports PT - Journal Article PT - Review TT - Castleman-like lymfadenopatie u pacienta se smíšenou chorobou pojiva: kazuistika a přehled literatury. PL - Czech Republic TA - Cesk Patol JT - Ceskoslovenska patologie JID - 0050734 RN - Multi-centric Castleman's Disease SB - IM MH - Humans MH - Female MH - Aged MH - *Castleman Disease/diagnosis/pathology MH - *Lymphadenopathy/etiology/pathology/diagnosis MH - *Mixed Connective Tissue Disease/complications/diagnosis MH - Diagnosis, Differential MH - Lymph Nodes/pathology OTO - NOTNLM OT - Castleman-like lymphadenopathy OT - Raynaud’s phenomenon - anti-U1-RNP OT - Sclerodactyly OT - autoimmune disease OT - mixed connective tissue disease EDAT- 2026/04/18 13:10 MHDA- 2026/04/18 13:11 CRDT- 2026/04/17 12:43 PHST- 2026/04/18 13:11 [medline] PHST- 2026/04/18 13:10 [pubmed] PHST- 2026/04/17 12:43 [entrez] AID - 143335 [pii] PST - ppublish SO - Cesk Patol. 2026;62(1):50-57. PMID- 24900125 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140606 LR - 20211021 IS - 1869-3474 (Print) IS - 1869-3482 (Electronic) IS - 1869-3474 (Linking) VI - 47 IP - 4 DP - 2013 Dec TI - Conversion to Paradoxical Finding on Technetium-99m-labeled RBC Scintigraphy after Treatment for Secondary Raynaud's Phenomenon. PG - 278-80 LID - 10.1007/s13139-013-0215-7 [doi] AB - An 18-year-old woman reported that after exposure to cold temperatures her fingers appeared blue and her hands and feet felt cold. Secondary Raynaud's phenomenon (RP) associated with peripheral vascular disease was suspected. Technetium (Tc)-99m-labeled RBC hand scintigraphy after cold change showed decreased blood pool activity in her fingers. The patient's symptoms improved after she received sarpogrelate HCL (200 mg/day) and nifedifine (40 mg/day). Follow-up scintigraphy performed 7 months after the patient started treatment showed paradoxically increased blood pool activity in her fingers after cold challenge. To the best of our knowledge, this is the first case report of a patient with secondary RP showing paradoxical change on scintigraphy after she received medication that improved her symptoms. FAU - Chong, Ari AU - Chong A AD - Department of Nuclear Medicine, Chosun University Hospital, 588 Seoseok-dong, Dong-gu, Gwangju, 501-757 Republic of Korea. FAU - Ha, Jung-Min AU - Ha JM AD - Department of Nuclear Medicine, Chosun University Hospital, 588 Seoseok-dong, Dong-gu, Gwangju, 501-757 Republic of Korea. FAU - Song, Ho-Chun AU - Song HC AD - Department of Nuclear Medicine, Chonnam National University Hospital, Gwangju, Republic of Korea. FAU - Kim, Jahae AU - Kim J AD - Department of Nuclear Medicine, Chonnam National University Hospital, Gwangju, Republic of Korea. FAU - Choi, Soo Jin Na AU - Choi SJ AD - Division of Transplantation & Vascular Surgery, Department of Surgery, Chonnam National University Hospital, Gwangju, Republic of Korea. LA - eng PT - Journal Article DEP - 20130821 PL - Germany TA - Nucl Med Mol Imaging JT - Nuclear medicine and molecular imaging JID - 101530478 PMC - PMC4035180 OTO - NOTNLM OT - Paradoxical reaction OT - Raynaud’s phenomenon OT - Tc-99 m RBC scintigraphy EDAT- 2014/06/06 06:00 MHDA- 2014/06/06 06:01 PMCR- 2014/12/01 CRDT- 2014/06/06 06:00 PHST- 2013/04/30 00:00 [received] PHST- 2013/07/08 00:00 [revised] PHST- 2013/07/10 00:00 [accepted] PHST- 2014/06/06 06:00 [entrez] PHST- 2014/06/06 06:00 [pubmed] PHST- 2014/06/06 06:01 [medline] PHST- 2014/12/01 00:00 [pmc-release] AID - 215 [pii] AID - 10.1007/s13139-013-0215-7 [doi] PST - ppublish SO - Nucl Med Mol Imaging. 2013 Dec;47(4):278-80. doi: 10.1007/s13139-013-0215-7. Epub 2013 Aug 21. PMID- 35629370 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2075-1729 (Print) IS - 2075-1729 (Electronic) IS - 2075-1729 (Linking) VI - 12 IP - 5 DP - 2022 May 8 TI - Animal Models of Systemic Sclerosis: Using Nailfold Capillaroscopy as a Potential Tool to Evaluate Microcirculation and Microangiopathy: A Narrative Review. LID - 10.3390/life12050703 [doi] LID - 703 AB - Systemic sclerosis (SSc) is an autoimmune disease with three pathogenic hallmarks, i.e., inflammation, vasculopathy, and fibrosis. A wide plethora of animal models have been developed to address the complex pathophysiology and for the development of possible anti-fibrotic treatments. However, no current model comprises all three pathological mechanisms of the disease. To highlight the lack of a complete model, a review of some of the most widely used animal models for SSc was performed. In addition, to date, no model has accomplished the recreation of primary or secondary Raynaud's phenomenon, a key feature in SSc. In humans, nailfold capillaroscopy (NFC) has been used to evaluate secondary Raynaud's phenomenon and microvasculature changes in SSc. Being a non-invasive technique, it is widely used both in clinical studies and as a tool for clinical evaluation. Because of this, its potential use in animal models has been neglected. We evaluated NFC in guinea pigs to investigate the possibility of applying this technique to study microcirculation in the nailfold of animal models and in the future, development of an animal model for Raynaud's phenomenon. The applications are not only to elucidate the pathophysiological mechanisms of vasculopathy but can also be used in the development of novel treatment options. FAU - Mandujano, Angélica AU - Mandujano A AUID- ORCID: 0000-0001-8006-7683 AD - Departamento de Atención a la Salud, Universidad Autónoma Metropolitana-Xochimilco, Mexico City 04960, Mexico. FAU - Golubov, Melissa AU - Golubov M AD - Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico City 04510, Mexico. LA - eng PT - Journal Article PT - Review DEP - 20220508 PL - Switzerland TA - Life (Basel) JT - Life (Basel, Switzerland) JID - 101580444 PMC - PMC9147447 OTO - NOTNLM OT - Raynaud’s phenomenon OT - animal models OT - fibrosis OT - nailfold capillaroscopy OT - systemic sclerosis OT - vasculopathy COIS- The authors declare no conflict of interest. EDAT- 2022/05/29 06:00 MHDA- 2022/05/29 06:01 PMCR- 2022/05/08 CRDT- 2022/05/28 01:27 PHST- 2022/04/07 00:00 [received] PHST- 2022/05/05 00:00 [revised] PHST- 2022/05/06 00:00 [accepted] PHST- 2022/05/28 01:27 [entrez] PHST- 2022/05/29 06:00 [pubmed] PHST- 2022/05/29 06:01 [medline] PHST- 2022/05/08 00:00 [pmc-release] AID - life12050703 [pii] AID - life-12-00703 [pii] AID - 10.3390/life12050703 [doi] PST - epublish SO - Life (Basel). 2022 May 8;12(5):703. doi: 10.3390/life12050703. PMID- 33466727 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210126 IS - 1996-1944 (Print) IS - 1996-1944 (Electronic) IS - 1996-1944 (Linking) VI - 14 IP - 2 DP - 2021 Jan 14 TI - Design of Electro-Thermal Glove with Sensor Function for Raynaud's Phenomenon Patients. LID - 10.3390/ma14020377 [doi] LID - 377 AB - Raynaud's phenomenon (RP) is a disease that mainly affects human fingertips during cold weather. It is difficult to treat this disease using medicine, apart from keeping the body in a warm environment. In this research, conductive knitted fabrics were fabricated to help relax the vessels of the patient's fingertips by providing proper heat, and also serving as a sensor to detect finger motion after relaxation of the blood vessels of patients. Four different structures, termed plain, purl, interlock, and rib were produced using conductive silver/PE (polyethylene) yarn and wool yarn, with a computerized flat knitting machine. The effect of knitted structure on the electro-thermal behavior, sensitivity, and stability of resistance change (∆R/R) under different tensile forces was investigated. By comprehensive comparison, the purl structure was identified as the preferred structure for the heating glove for RP patients, owing to superior electro-thermal behavior. Additionally, the purl structure had a greater capacity to detect different motions with stable resistance change. This potential electro-thermal glove could be used for functional, as well as aesthetic (fashion) purposes, and could be worn at any time and occasion with complete comfort. FAU - Dawit, Hewan AU - Dawit H AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. AD - Key Laboratory of Textile Industry for Biomedical Textile Materials and Technology, Donghua University, Shanghai 201620, China. FAU - Zhang, Qian AU - Zhang Q AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. AD - Key Laboratory of Textile Industry for Biomedical Textile Materials and Technology, Donghua University, Shanghai 201620, China. FAU - Li, Yimeng AU - Li Y AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. AD - Key Laboratory of Textile Industry for Biomedical Textile Materials and Technology, Donghua University, Shanghai 201620, China. FAU - Islam, Syed Rashedul AU - Islam SR AUID- ORCID: 0000-0002-8784-426X AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. FAU - Mao, Jifu AU - Mao J AUID- ORCID: 0000-0001-5143-4149 AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. AD - Key Laboratory of Textile Industry for Biomedical Textile Materials and Technology, Donghua University, Shanghai 201620, China. FAU - Wang, Lu AU - Wang L AD - Key Laboratory of Textile Science & Technology of Ministry of Education and College of Textiles, Donghua University, 2999 North Renmin Road, Songjiang District, Shanghai 201620, China. AD - Key Laboratory of Textile Industry for Biomedical Textile Materials and Technology, Donghua University, Shanghai 201620, China. LA - eng GR - 2232020G-01; BP0719035/Fundamental Research Funds for the Central Universities； 111 Project 2.0; Fundamental Research Funds for DHU Distinguished Young Professor Program; Fnancial support from the Chinese government scholarship council/ PT - Journal Article DEP - 20210114 PL - Switzerland TA - Materials (Basel) JT - Materials (Basel, Switzerland) JID - 101555929 PMC - PMC7828797 OTO - NOTNLM OT - Raynaud’s phenomenon OT - contact resistance OT - electro-thermal property OT - knitted structure OT - strain sensor COIS- The authors declare no conflict of interest. EDAT- 2021/01/21 06:00 MHDA- 2021/01/21 06:01 PMCR- 2021/01/14 CRDT- 2021/01/20 01:07 PHST- 2020/12/08 00:00 [received] PHST- 2020/12/29 00:00 [revised] PHST- 2021/01/12 00:00 [accepted] PHST- 2021/01/20 01:07 [entrez] PHST- 2021/01/21 06:00 [pubmed] PHST- 2021/01/21 06:01 [medline] PHST- 2021/01/14 00:00 [pmc-release] AID - ma14020377 [pii] AID - materials-14-00377 [pii] AID - 10.3390/ma14020377 [doi] PST - epublish SO - Materials (Basel). 2021 Jan 14;14(2):377. doi: 10.3390/ma14020377. PMID- 37525698 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230802 IS - 2253-1556 (Print) IS - 2253-1556 (Electronic) IS - 2253-1556 (Linking) VI - 12 DP - 2023 TI - Towards Early Diagnosis of Mixed Connective Tissue Disease: Updated Perspectives. PG - 79-89 LID - 10.2147/ITT.S390023 [doi] AB - Mixed Connective Tissue Disease (MCTD) is an autoimmune disease first described by Sharp et al in 1972, characterized by the presence of anti-Ribonucleoprotein antibodies directed against the U1 complex (anti-U1RNP). The condition shares clinical characteristics with Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Systemic Sclerosis. Diagnosis is quite difficult due to its rarity, the lack of validated classification criteria, and its heterogeneous clinical presentation. While in the early stages its nuanced clinical features might lead to it being incorrectly classified as other Connective Tissue Diseases (CTDs) or even not recognized, in cases of longstanding disease its classification as a CTD is clear but challenging to discriminate from overlap syndromes. MCTD should be considered a distinct entity due to the presence of a specific genetic substrate and the presence of the high titer of a specific autoantibody, anti-U1RNP, present in all the commercial kits for Extractable Nuclear Antigens, and almost always associated with Antinuclear Antibody positivity with a coarse speckled pattern. Except for anti-U1RNP, no specific biomarkers are available to guide clinicians to a correct classification of MCTD, which is arrived at by the association of clinical, serological and instrumental evaluation. In the first stages, the disease is mainly characterized by Raynaud's phenomenon, inflammatory arthritis, puffy fingers, myalgia and/or myositis, and rarely, trigeminal neuropathy. Longstanding disease is generally associated with the development of Pulmonary Hypertension and Interstitial Lung Disease, which are the two main causes of mortality in MCTD. The aim of this review is to summarize current knowledge on the early recognition of MCTD. CI - © 2023 Ferrara et al. FAU - Ferrara, Chiara Alfia AU - Ferrara CA AD - Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. "Policlinico-San Marco", University of Catania, Catania, Italy. FAU - La Rocca, Gaetano AU - La Rocca G AD - Department of Rheumatology, University of Pisa, Pisa, Italy. FAU - Ielo, Giuseppe AU - Ielo G AD - Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. "Policlinico-San Marco", University of Catania, Catania, Italy. FAU - Libra, Alessandro AU - Libra A AD - Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. "Policlinico-San Marco", University of Catania, Catania, Italy. FAU - Sambataro, Gianluca AU - Sambataro G AUID- ORCID: 0000-0001-9933-1202 AD - Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. "Policlinico-San Marco", University of Catania, Catania, Italy. LA - eng PT - Journal Article PT - Review DEP - 20230726 PL - New Zealand TA - Immunotargets Ther JT - ImmunoTargets and therapy JID - 101606565 PMC - PMC10387239 OTO - NOTNLM OT - Raynaud’s phenomenon OT - anti-RNP OT - diagnosis OT - inflammatory arthritis OT - mixed connective tissue disease OT - nailfold videocapillaroscopy COIS- Gianluca Sambataro received honoraria from Boheringer Ingelheim, outside of the submitted work. Gaetano La Rocca, Chiara Alfia Ferrara, Giuseppe Ielo, and Alessandro Libra have no conflicts of interest to declare for this work. EDAT- 2023/08/01 06:44 MHDA- 2023/08/01 06:45 PMCR- 2023/07/26 CRDT- 2023/08/01 03:36 PHST- 2023/05/25 00:00 [received] PHST- 2023/07/21 00:00 [accepted] PHST- 2023/08/01 06:45 [medline] PHST- 2023/08/01 06:44 [pubmed] PHST- 2023/08/01 03:36 [entrez] PHST- 2023/07/26 00:00 [pmc-release] AID - 390023 [pii] AID - 10.2147/ITT.S390023 [doi] PST - epublish SO - Immunotargets Ther. 2023 Jul 26;12:79-89. doi: 10.2147/ITT.S390023. eCollection 2023. PMID- 37966656 OWN - NLM STAT- MEDLINE DCOM- 20240920 LR - 20240920 IS - 0973-7693 (Electronic) IS - 0019-5456 (Linking) VI - 91 IP - 10 DP - 2024 Oct TI - Juvenile Scleroderma. PG - 1049-1055 LID - 10.1007/s12098-023-04894-1 [doi] AB - Localised scleroderma and systemic sclerosis are rare chronic fibrosing disorders seen in children, and are collectively referred to as Juvenile Scleroderma. Histopathology of the two forms is non-distinct but they differ in terms of vasculopathy, internal organ involvement, morbidity and mortality. Raynaud's phenomenon with digital tip ulcers is considered hallmark of systemic sclerosis. Quality of life gets greatly affected by these diseases. Early identification in the inflammatory phase of the disease, effective treatment and strict surveillance remain crucial for better outcomes. Emerging vascular and immunosuppressive strategies, coupled with efforts from scientific community to develop better biomarkers and monitoring tools, help constantly to improve survival rates. CI - © 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation. FAU - Bhat, Aruna AU - Bhat A AD - Department of Pediatric Rheumatology, Narayana Health City, Bangalore, Karnataka, India. hegdearuna@hotmail.com. LA - eng PT - Journal Article PT - Review DEP - 20231115 PL - India TA - Indian J Pediatr JT - Indian journal of pediatrics JID - 0417442 RN - 0 (Immunosuppressive Agents) RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/complications MH - Child MH - *Scleroderma, Localized/diagnosis/therapy MH - Quality of Life MH - Immunosuppressive Agents/therapeutic use OTO - NOTNLM OT - Digital ulcers OT - Juvenile scleroderma OT - Nail-fold capillaroscopy OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2023/11/15 12:42 MHDA- 2024/09/20 19:08 CRDT- 2023/11/15 11:11 PHST- 2023/03/17 00:00 [received] PHST- 2023/10/03 00:00 [accepted] PHST- 2024/09/20 19:08 [medline] PHST- 2023/11/15 12:42 [pubmed] PHST- 2023/11/15 11:11 [entrez] AID - 10.1007/s12098-023-04894-1 [pii] AID - 10.1007/s12098-023-04894-1 [doi] PST - ppublish SO - Indian J Pediatr. 2024 Oct;91(10):1049-1055. doi: 10.1007/s12098-023-04894-1. Epub 2023 Nov 15. PMID- 36438809 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221129 IS - 1663-9812 (Print) IS - 1663-9812 (Electronic) IS - 1663-9812 (Linking) VI - 13 DP - 2022 TI - G protein estrogen receptor as a potential therapeutic target in Raynaud's phenomenon. PG - 1061374 LID - 10.3389/fphar.2022.1061374 [doi] LID - 1061374 AB - Exaggerated cold-induced vasoconstriction can precipitate a pathogenesis called Raynaud's phenomenon (RP). Interestingly, RP is significantly more prevalent in females than age-matched men, highlighting the potential implication of 17β-estradiol (E(2)) in the etio-pathogenesis of this disease. Indeed, we have previously reported that E(2) stimulates the expression of vascular alpha 2C-adrenoceptors (α(2C)-AR), the sole mediator of cold-induced constriction of cutaneous arterioles. This induced expression occurs through the cyclic adenosine monophosphate → exchange protein activated by cAMP→ Ras-related protein 1→ c-Jun N-terminal kinase→ activator protein-1 (cAMP/Epac/Rap/JNK/AP-1 pathway). On the basis that estrogen-induced rapid cAMP accumulation and JNK activation occurs so rapidly we hypothesized that a non-classic, plasma membrane estrogen receptor was the mediator. We then showed that an impermeable form of E(2), namely E(2):BSA, mimics E(2) effects suggesting a role for the membranous G-protein coupled estrogen receptor (GPER) in E(2)-induced α(2C)-AR expression. Our current working hypothesis and unpublished observations further cement this finding, as G1, a GPER agonist, mimics while G15, a GPER antagonist, abrogates estrogen's effect on the expression of vascular α(2C)-AR. These, and other observations, highlight the potential of GPER as a tractable target in the management of RP, particularly in pre-menopausal women. CI - Copyright © 2022 Fardoun, Mondello, Kobeissy and Eid. FAU - Fardoun, Manal AU - Fardoun M AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - Mondello, Stefania AU - Mondello S AD - Department of Biomedical and Dental Sciences and Morphofunctional Imaging, University of Messina, Messina, Italy. FAU - Kobeissy, Firas AU - Kobeissy F AD - Department of Emergency Medicine, University of Florida, Gainesville, FL, United States. FAU - Eid, Ali H AU - Eid AH AD - Department of Basic Medical Sciences, College of Medicine, QU Health, Qatar University, Doha, Qatar. LA - eng PT - Journal Article DEP - 20221110 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC9685397 OTO - NOTNLM OT - GPER OT - VSMC OT - alpha 2C adrenoceptor OT - cardiovascular disease OT - estrogen OT - gender bias OT - raynaud’s phenomenon OT - vasoconstriction COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/11/29 06:00 MHDA- 2022/11/29 06:01 PMCR- 2022/11/10 CRDT- 2022/11/28 04:24 PHST- 2022/10/04 00:00 [received] PHST- 2022/10/31 00:00 [accepted] PHST- 2022/11/28 04:24 [entrez] PHST- 2022/11/29 06:00 [pubmed] PHST- 2022/11/29 06:01 [medline] PHST- 2022/11/10 00:00 [pmc-release] AID - 1061374 [pii] AID - 10.3389/fphar.2022.1061374 [doi] PST - epublish SO - Front Pharmacol. 2022 Nov 10;13:1061374. doi: 10.3389/fphar.2022.1061374. eCollection 2022. PMID- 41522843 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260112 LR - 20260114 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 14 IP - 1 DP - 2026 Jan TI - Beyond Raynaud's: Atypical Peripheral Vascular Manifestations in a Case of CREST Syndrome. PG - e71815 LID - 10.1002/ccr3.71815 [doi] LID - e71815 AB - Peripheral vascular disease (PVD) is a rare but severe symptom of CREST syndrome, which itself is a limited cutaneous sclerosis. Even though Raynaud's phenomenon is the gold standard of CREST, the development of critical limb ischemia and self-amputation is rare. We report a case of a 48-year-old female with CREST syndrome who presented with progressive ischemia in both upper and lower limbs, leading to spontaneous auto-amputation of multiple fingers/toes. The patient exhibited sclerodactyly, dry gangrene, delayed capillary refill, and tactile deficits in peripheral pulses. ANA and anti-Scl 70 antibody were positive. CT angiography demonstrated segmental and progressive obstruction of multifocal peripheral arteries. The patient was operated on for amputation of necrotic digits and was initiated on immunomodulatory therapy. Following treatment, monitoring revealed stabilization without further progression. This case highlights the possible seriousness of vascular complications in CREST syndrome. Prompt diagnosis and treatment are necessary to prevent irreversible ischemic damage. CI - © 2026 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Kumari, Sakshi AU - Kumari S AD - Shimoga Institute of Medical Sciences Shimoga India. FAU - Henna, Fathimathul AU - Henna F AD - Dubai Medical College for Girls Dubai UAE. FAU - Alam, Umama AU - Alam U AUID- ORCID: 0009-0001-0456-022X AD - Khyber Medical College Peshawar Pakistan. FAU - Khattak, Fazia AU - Khattak F AUID- ORCID: 0009-0004-0508-4765 AD - Khyber Medical College Peshawar Pakistan. FAU - Kamil, Kamil Ahmad AU - Kamil KA AUID- ORCID: 0009-0000-8208-0689 AD - Internal Medicine Department Mirwais Regional Hospital Kandahar Afghanistan. LA - eng PT - Journal Article DEP - 20260110 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC12789933 OTO - NOTNLM OT - Raynaud's phenomenon OT - amputation OT - crest syndrome OT - ischemia OT - sclerosis COIS- The authors declare no conflicts of interest. EDAT- 2026/01/12 06:29 MHDA- 2026/01/12 06:30 PMCR- 2026/01/10 CRDT- 2026/01/12 05:41 PHST- 2025/04/14 00:00 [received] PHST- 2025/11/09 00:00 [revised] PHST- 2025/12/29 00:00 [accepted] PHST- 2026/01/12 06:30 [medline] PHST- 2026/01/12 06:29 [pubmed] PHST- 2026/01/12 05:41 [entrez] PHST- 2026/01/10 00:00 [pmc-release] AID - CCR371815 [pii] AID - 10.1002/ccr3.71815 [doi] PST - epublish SO - Clin Case Rep. 2026 Jan 10;14(1):e71815. doi: 10.1002/ccr3.71815. eCollection 2026 Jan. PMID- 41583572 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260126 LR - 20260128 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 63 IP - 6 DP - 2025 TI - Prevalence and severity of complicated Raynaud's phenomenon in limited and diffuse systemic sclerosis: a multicenter study in Iraq. PG - 383-388 LID - 10.5114/reum/207625 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multiple internal organ involvement, vasculopathy, and fibrosis. Two major types are present, limited systemic sclerosis (lSSc) and diffuse systemic sclerosis (dSSc), according to the limit of skin fibrosis, with variability in internal organ involvement. Raynaud's phenomenon (RP) is almost always present in either type as a presenting feature; it may precede the onset by years. It affects the quality of life for the patient and has a variable range of complications as well, with the most severe being tissue gangrene and finger amputation. The aim of the study was to investigate the prevalence of RP complications and predictors of outcome in lSSc and dSSc. MATERIAL AND METHODS: Patients diagnosed with SSc were included in the study at 3 rheumatology centers in Iraq over a 3-year period. Data collection was conducted through questionnaires and interviews. All patients underwent clinical assessments to determine the presence or absence of RP complications, including pit scars, ulcers, ischemia, and amputated digits at the time of interview as well as the previous records. Subjects with concomitant autoimmune diseases were excluded from the study. RESULTS: Of the 105 patients, 55 (52%) had dSSc, and 92% of those were female. The mean age was in the fourth decade. Digital complications were recorded. Ischemia was the most frequently observed complication in limited scleroderma, while amputation was the least common. These complications exhibited a significant association with the duration of the disease, with the highest prevalence occurring within the first 10 years of diagnosis. Cardiac complications were associated with RP. Notably, 27% of patients with dSSc had hypertension, while 64% of patients with limited scleroderma did not have cardiac complications. CONCLUSIONS: Raynaud's phenomenon is a defining characteristic of both limited and diffuse scleroderma. It is particularly complex in the diffuse form, underscoring the necessity for aggressive treatment to prevent debilitating complications. It is crucial to educate patients about the significance of adhering to treatment regimens and cessation of smoking. CI - Copyright: © 2025 Termedia & Banach. FAU - Jaafar, Farah AU - Jaafar F AUID- ORCID: 0009-0004-1577-6933 AD - Rheumatology Unit, Department of Medicine, College of Medicine, University of Mustansiriyah, Iraq. FAU - Moayad, Zakariya AU - Moayad Z AD - Rheumatology Unit, Department of Medicine, College of Medicine, University of Babylon, Iraq. FAU - Gorial, Faiq AU - Gorial F AUID- ORCID: 0000-0002-2760-5566 AD - Rheumatology Unit, Department of Medicine, College of Medicine, University of Baghdad, Iraq. FAU - Alkazzaz, Ali AU - Alkazzaz A AUID- ORCID: 0000-0002-7852-1581 AD - Rheumatology Unit, Department of Medicine, College of Medicine, University of Babylon, Iraq. LA - eng PT - Journal Article DEP - 20251204 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC12828900 OTO - NOTNLM OT - Raynaud’s phenomenon OT - ischemia OT - scleroderma COIS- The authors declare no conflict of interest. EDAT- 2026/01/26 14:54 MHDA- 2026/01/26 14:55 PMCR- 2025/12/04 CRDT- 2026/01/26 06:49 PHST- 2025/03/07 00:00 [received] PHST- 2025/06/27 00:00 [accepted] PHST- 2026/01/26 14:55 [medline] PHST- 2026/01/26 14:54 [pubmed] PHST- 2026/01/26 06:49 [entrez] PHST- 2025/12/04 00:00 [pmc-release] AID - 207625 [pii] AID - 10.5114/reum/207625 [doi] PST - epublish SO - Reumatologia. 2025 Dec 4;63(6):383-388. doi: 10.5114/reum/207625. eCollection 2025. PMID- 23346430 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130125 LR - 20211021 IS - 2090-6579 (Electronic) IS - 2090-6560 (Print) IS - 2090-6579 (Linking) VI - 2012 DP - 2012 TI - Deep Vein Thrombosis, Raynaud's Phenomenon, and Prinzmetal Angina in a Patient with Glanzmann Thrombasthenia. PG - 156290 LID - 10.1155/2012/156290 [doi] LID - 156290 AB - Patients with Glanzmann thrombasthenia fail to form large platelet thrombi due to mutations that affect the biosynthesis and/or function of the αIIbβ3 integrin. The result is a moderate to severe bleeding syndrome. We now report unusual vascular behaviour in a 55-year-old woman with classic type I disease (with no platelet αIIbβ3 expression) and a homozygous ITGA2B missense mutation (E324K) affecting the terminal β-propeller domain of αIIb. While exhibiting classic bleeding symptoms as a child, in later life this woman first developed deep vein thrombosis after a long air flight then showed vascular problems characteristic of Raynaud's phenomenon, and finally this year she presented with chest pains suggestive of coronary heart disease. Yet while coronary angiography first showed a stenosis, this was not seen on a second examination when she was diagnosed with coronary spastic angina and Prinzmetal phenomenon. It is significant that the absence of platelet aggregation with physiologic agonists had not prevented any of the above cardiovascular or vascular diseases. FAU - Nurden, Alan AU - Nurden A AD - Plateforme Technologique et d'Innovation Biomédicale, Hôpital Xavier Arnozan, 33600 Pessac, France. FAU - Mercié, Patrick AU - Mercié P FAU - Zely, Pascal AU - Zely P FAU - Nurden, Paquita AU - Nurden P LA - eng PT - Journal Article DEP - 20121231 PL - United States TA - Case Rep Hematol JT - Case reports in hematology JID - 101576456 PMC - PMC3549378 EDAT- 2013/01/25 06:00 MHDA- 2013/01/25 06:01 PMCR- 2012/12/31 CRDT- 2013/01/25 06:00 PHST- 2012/11/28 00:00 [received] PHST- 2012/12/19 00:00 [accepted] PHST- 2013/01/25 06:00 [entrez] PHST- 2013/01/25 06:00 [pubmed] PHST- 2013/01/25 06:01 [medline] PHST- 2012/12/31 00:00 [pmc-release] AID - 10.1155/2012/156290 [doi] PST - ppublish SO - Case Rep Hematol. 2012;2012:156290. doi: 10.1155/2012/156290. Epub 2012 Dec 31. PMID- 40270661 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250425 IS - 2284-2594 (Electronic) IS - 2284-2594 (Linking) VI - 12 IP - 4 DP - 2025 TI - Improvement of Microangiopathy in A Systemic Sclerosis Patient Following Hysterectomy for Leiomyoma: A Potential Role of Neoplasia. PG - 005286 LID - 10.12890/2025_005286 [doi] LID - 005286 AB - BACKGROUND: Systemic sclerosis (SSc) is a complex autoimmune disease characterised by vascular alterations, immune dysregulation and fibrosis. Microangiopathy is a hallmark of SSc and can be assessed non-invasively using nailfold videocapillaroscopy (NVC). CASE DESCRIPTION: This is a case of a 50-year-old Caucasian woman with anticentromere-positive SSc, experiencing Raynaud's phenomenon and hand oedema. She met the 2013 ACR/EULAR classification criteria for SSc. Despite stable nifedipine therapy, her vasospastic episodes were only partially controlled. In March 2024, she underwent a total hysterectomy for a symptomatic uterine leiomyoma. Postoperatively, there was a marked improvement in Raynaud's phenomenon and NVC findings. DISCUSSION: This case suggests a potential link between neoplastic processes and exacerbation of SSc-related microangiopathy. It is hypothesised that tumour-derived factors, such as inflammatory cytokines and angiogenic mediators, may contribute to endothelial dysfunction and worsen vascular impairment. The observed microvascular improvement following tumour removal supports the hypothesis that neoplasms may act as disease-modifying factors in SSc. Further studies are needed to determine whether systematic malignancy screening in selected SSc patients may help identify modifiable triggers of microangiopathy. LEARNING POINTS: Observation of clinical and microvascular improvement in a systemic sclerosis patient is recommended following the removal of a uterine leiomyoma.The potential aggravating role of neoplastic processes in systemic sclerosis-associated microangiopathy.The importance of timely identification and management of neoplasms in systemic sclerosis patients to modulate disease progression. CI - © EFIM 2025. FAU - Nigro, Angelo AU - Nigro A AD - Department of Rheumatology of Lucania, UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Santarcangelo, Pasquale AU - Santarcangelo P AD - Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Bonelli, Antonio AU - Bonelli A AD - Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Digregorio, Serena AU - Digregorio S AD - Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Mazzoccoli, Carmela AU - Mazzoccoli C AD - Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Nicoletti, Giuseppe AU - Nicoletti G AD - Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. LA - eng PT - Journal Article DEP - 20250326 PL - Italy TA - Eur J Case Rep Intern Med JT - European journal of case reports in internal medicine JID - 101648453 PMC - PMC12013226 OTO - NOTNLM OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - capillaroscopy OT - microangiopathy OT - neoplasia COIS- Conflicts of Interests: The Authors declare that there are no competing interests. EDAT- 2025/04/24 12:30 MHDA- 2025/04/24 12:31 PMCR- 2025/03/26 CRDT- 2025/04/24 06:17 PHST- 2025/02/25 00:00 [received] PHST- 2025/03/03 00:00 [accepted] PHST- 2025/04/24 12:31 [medline] PHST- 2025/04/24 12:30 [pubmed] PHST- 2025/04/24 06:17 [entrez] PHST- 2025/03/26 00:00 [pmc-release] AID - 5286 [pii] AID - 10.12890/2025_005286 [doi] PST - epublish SO - Eur J Case Rep Intern Med. 2025 Mar 26;12(4):005286. doi: 10.12890/2025_005286. eCollection 2025. PMID- 39759709 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250107 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 12 DP - 2024 Dec TI - Digital Ulcers and Microvascular Abnormalities Presenting As the Initial Manifestations of Pre-scleroderma. PG - e75061 LID - 10.7759/cureus.75061 [doi] LID - e75061 AB - The term Raynaud's phenomenon (RP) is used to describe complex symptoms related to vascular compromise, which are typically exacerbated by cold-induced vasoconstriction, emotional stress, or other sympathomimetic factors. In almost all patients with limited cutaneous systemic sclerosis (SSc), the first symptom is RP, often two to five years before any other symptom of scleroderma. The clinical course and severity of this disease are variable and highly fatal in some individuals, which has led to the development of strategies for timely diagnosis; hence, criteria for the very early diagnosis of systemic sclerosis have been established. Nevertheless, some individuals with this disease may lack classification findings (Raynaud's phenomenon, puffy fingers, and positive antinuclear antibodies), making their condition less apparent. This study reports a different clinical scenario involving a previously healthy 42-year-old woman with recent monophasic RP and digital ulcers in the absence of clinical or immunological manifestations of systemic autoimmune disease. The diagnosis of preclinical systemic sclerosis was made possible based on evidence of capillaroscopic abnormalities. The patient demonstrated favorable evolution following the initiation of treatment with hydroxychloroquine, a statin, and a calcium antagonist. Four years later, she had no recurrences of digital ulcers, a noticeable improvement in her RP, and no clinical features of systemic sclerosis, highlighting the paradigm of the natural history of scleroderma in its preclinical, clinical, advanced severe stages, and immunological abnormalities. CI - Copyright © 2024, Arango et al. FAU - Arango, Alejandro AU - Arango A AD - Department of Internal Medicine, Pontifical Bolivarian University, Medellin, COL. FAU - Yaman, Reena N AU - Yaman RN AD - Department of Rheumatology, Mayo Clinic, Jacksonville, USA. FAU - Mumtaz, Sehreen AU - Mumtaz S AD - Department of Rheumatology, Mayo Clinic, Jacksonville, USA. FAU - Abril, Andy AU - Abril A AD - Department of Rheumatology, Mayo Clinic, Jacksonville, USA. FAU - Berianu, Florentina AU - Berianu F AD - Department of Rheumatology, Mayo Clinic, Jacksonville, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20241203 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11695195 OTO - NOTNLM OT - immunosuppressive agents OT - nailfold videocapillaroscopy OT - raynaud's phenomenon OT - scleroderma OT - vasculopathy COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/01/06 10:08 MHDA- 2025/01/06 10:09 PMCR- 2024/12/03 CRDT- 2025/01/06 05:40 PHST- 2024/12/03 00:00 [accepted] PHST- 2025/01/06 10:09 [medline] PHST- 2025/01/06 10:08 [pubmed] PHST- 2025/01/06 05:40 [entrez] PHST- 2024/12/03 00:00 [pmc-release] AID - 10.7759/cureus.75061 [doi] PST - epublish SO - Cureus. 2024 Dec 3;16(12):e75061. doi: 10.7759/cureus.75061. eCollection 2024 Dec. PMID- 18325317 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20100630 LR - 20211020 IS - 1092-8464 (Print) IS - 1092-8464 (Linking) VI - 10 IP - 2 DP - 2008 Apr TI - Raynaud's phenomenon. PG - 146-55 AB - Treatment of Raynaud's phenomenon in any one individual depends on the severity of symptoms and whether or not there is an underlying disease process requiring specific intervention. Many patients with primary (idiopathic) Raynaud's phenomenon respond well to treatment with general measures, perhaps with the addition of a calcium channel blocker. Conversely, others with underlying structural vascular abnormality (as occurs in connective tissue diseases such as systemic sclerosis ) are often refractory to treatment and may progress to irreversible tissue injury, sometimes with gangrene. Because these patients are the most challenging and because a large proportion of the research into Raynaud's phenomenon has been conducted in patients with SSc-spectrum disorders, much of this review is weighted toward them. The key principles of management are removal/treatment of any triggering factor/underlying cause, general (nondrug) measures, drug treatment, and surgery, although the last is rarely indicated. New insights into pathogenesis have led, and continue to lead, to new approaches to treatment, including endothelin-1 receptor antagonism and nitric oxide supplementation. FAU - Herrick, Ariane AU - Herrick A AD - Rheumatic Diseases Centre, University of Manchester, Salford Royal NHS Foundation Trust, Stott Lane, Salford M6 8HD, UK. ariane.herrick@manchester.ac.uk LA - eng PT - Journal Article PL - United States TA - Curr Treat Options Cardiovasc Med JT - Current treatment options in cardiovascular medicine JID - 9815942 EDAT- 2008/03/08 09:00 MHDA- 2008/03/08 09:01 CRDT- 2008/03/08 09:00 PHST- 2008/03/08 09:00 [pubmed] PHST- 2008/03/08 09:01 [medline] PHST- 2008/03/08 09:00 [entrez] AID - 10.1007/s11936-008-0016-y [doi] PST - ppublish SO - Curr Treat Options Cardiovasc Med. 2008 Apr;10(2):146-55. doi: 10.1007/s11936-008-0016-y. PMID- 37097526 OWN - NLM STAT- MEDLINE DCOM- 20230616 LR - 20230616 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 42 IP - 7 DP - 2023 Jul TI - Cannabis arteritis presenting with Raynaud's and digital ulcerations: a case-based review of a controversial thromboangiitis obliterans-like condition. PG - 1981-1985 LID - 10.1007/s10067-023-06603-x [doi] AB - Thromboangiitis obliterans (TAO), or Buerger's disease, is a non-atherosclerotic inflammatory disease of the small and medium-sized arteries, veins, and nerves of the legs and arms, strongly associated with the use of tobacco products in young adults. Cannabis arteritis (CA), an entity with similar clinical and pathological features, has been described in marijuana users as a subtype of TAO. Distinction between TAO and CA is challenging, given that most patients use tobacco and marijuana products concomitantly. Herein, we report the case of a male in his late forties who was referred to rheumatology with a 2-month history of hand swelling and bilateral painful digital ulcers with blue discoloration on his fingers and toes. The patient reported daily use of marijuana in blunt wraps and denied tobacco use. His laboratory work-up was negative for scleroderma and other connective tissue diseases. His angiogram confirmed the diagnosis of thromboangiitis obliterans, which was attributed to cannabis arteritis. The patient was started on aspirin and nifedipine daily and discontinued marijuana use. His symptoms resolved within 6 months and have not recurred for more than a year with continued avoidance of marijuana. Our case is one of the few that features primarily marijuana-driven CA and highlights the importance of not only considering marijuana use but also blunt wrap use in patients presenting with Raynaud's phenomenon and ulcerations as cannabis use rises globally. CI - © 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Pilitsi, Eleni AU - Pilitsi E AUID- ORCID: 0000-0003-0047-3388 AD - Department of Dermatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, 609 Albany Street, Boston, MA, 02118, USA. eleni.pilitsi@bmc.org. FAU - Kennamer, Brooke AU - Kennamer B AUID- ORCID: 0000-0002-1404-5475 AD - Department of Dermatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, 609 Albany Street, Boston, MA, 02118, USA. FAU - Trepanowski, Nicole AU - Trepanowski N AUID- ORCID: 0000-0001-5885-1009 AD - Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, Boston, MA, USA. FAU - Gonzalez, Reina AU - Gonzalez R AUID- ORCID: 0000-0001-8150-2341 AD - Department of Dermatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, 609 Albany Street, Boston, MA, 02118, USA. FAU - Trojanowski, Marcin AU - Trojanowski M AUID- ORCID: 0000-0003-2583-1591 AD - Department of Rheumatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, Boston, MA, USA. FAU - Phillips, Tania AU - Phillips T AUID- ORCID: 0000-0003-2744-4931 AD - Department of Dermatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, 609 Albany Street, Boston, MA, 02118, USA. FAU - Lam, Christina S AU - Lam CS AUID- ORCID: 0000-0002-4761-5163 AD - Department of Dermatology, Boston University Aram V. Chobanian & Edward Avedisian School of Medicine, 609 Albany Street, Boston, MA, 02118, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20230425 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - digital ulcers SB - IM MH - *Thromboangiitis Obliterans/diagnosis MH - Lower Extremity MH - *Cannabis MH - *Arteritis/diagnosis MH - Skin Ulcer MH - Humans MH - Male MH - Young Adult OTO - NOTNLM OT - Cannabis OT - Cannabis arteritis OT - Raynaud’s OT - Thromboangiitis obliterans OT - Ulcerations EDAT- 2023/04/25 17:43 MHDA- 2023/06/16 06:42 CRDT- 2023/04/25 11:16 PHST- 2023/02/10 00:00 [received] PHST- 2023/04/13 00:00 [accepted] PHST- 2023/04/12 00:00 [revised] PHST- 2023/06/16 06:42 [medline] PHST- 2023/04/25 17:43 [pubmed] PHST- 2023/04/25 11:16 [entrez] AID - 10.1007/s10067-023-06603-x [pii] AID - 10.1007/s10067-023-06603-x [doi] PST - ppublish SO - Clin Rheumatol. 2023 Jul;42(7):1981-1985. doi: 10.1007/s10067-023-06603-x. Epub 2023 Apr 25. PMID- 40205717 OWN - NLM STAT- MEDLINE DCOM- 20250522 LR - 20250522 IS - 1749-6632 (Electronic) IS - 0077-8923 (Linking) VI - 1547 IP - 1 DP - 2025 May TI - A case of mixed connective tissue disease with usual interstitial pneumonia and methotrexate-induced erythema multiforme. PG - 100-104 LID - 10.1111/nyas.15333 [doi] AB - Mixed connective tissue disease (MCTD) is a rare autoimmune disorder characterized by overlapping features of various connective tissue diseases. We present a case of a 62-year-old Indian male with a 20-year history of skin tightness and dysphagia, accompanied by a low-grade fever persisting for 1 year. Physical examination revealed sclerodactyly, Raynaud's phenomenon, and perioral sclerosis. High-resolution computed tomography findings indicated a pattern consistent with usual interstitial pneumonia (UIP), characterized by honeycombing and traction bronchiectasis. Laboratory tests confirmed the presence of anti-U1 RNP antibodies. The patient was initially treated with methotrexate, which led to bullous drug eruptions on the palms, lips, and soles, diagnosed as methotrexate-induced erythema multiforme. Following the discontinuation of methotrexate, treatment with mycophenolate mofetil and prednisolone resulted in the resolution of fever and synovitis within 1 month. Over 6 months, there was significant improvement in Raynaud's phenomenon and sclerodactyly. This case highlights the uncommon presentation of UIP in MCTD and the potential for methotrexate to induce bullous eruptions, emphasizing the necessity for cautious use of this medication in patients with connective tissue diseases. CI - © 2025 The New York Academy of Sciences. FAU - Shah, Kavish AU - Shah K AD - Byramjee Jeejeebhoy Medical College & Civil Hospital, Ahmedabad, India. FAU - Vaja, HariOm AU - Vaja H AUID- ORCID: 0000-0002-2139-4438 AD - Byramjee Jeejeebhoy Medical College & Civil Hospital, Ahmedabad, India. FAU - Patel, Kahan AU - Patel K AD - Byramjee Jeejeebhoy Medical College & Civil Hospital, Ahmedabad, India. FAU - Banker, Ahan AU - Banker A AD - Byramjee Jeejeebhoy Medical College & Civil Hospital, Ahmedabad, India. FAU - Shah, Jay AU - Shah J AD - Department of Medicine, Byramjee Jeejeebhoy Medical College & Civil Hospital, Ahmedabad, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20250409 PL - United States TA - Ann N Y Acad Sci JT - Annals of the New York Academy of Sciences JID - 7506858 RN - YL5FZ2Y5U1 (Methotrexate) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Humans MH - Male MH - *Methotrexate/adverse effects MH - Middle Aged MH - *Mixed Connective Tissue Disease/drug therapy/complications/diagnosis MH - *Erythema Multiforme/chemically induced/diagnosis/complications MH - *Idiopathic Pulmonary Fibrosis/diagnosis/complications MH - Prednisolone/therapeutic use OTO - NOTNLM OT - Raynaud's phenomenon OT - methotrexate‐induced erythema multiforme OT - mixed connective tissue disease OT - usual interstitial pattern pneumonia EDAT- 2025/04/10 06:25 MHDA- 2025/05/22 12:32 CRDT- 2025/04/10 01:22 PHST- 2025/05/22 12:32 [medline] PHST- 2025/04/10 06:25 [pubmed] PHST- 2025/04/10 01:22 [entrez] AID - 10.1111/nyas.15333 [doi] PST - ppublish SO - Ann N Y Acad Sci. 2025 May;1547(1):100-104. doi: 10.1111/nyas.15333. Epub 2025 Apr 9. PMID- 32884802 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240329 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 8 IP - 8 DP - 2020 Aug TI - A case of antisynthetase syndrome. PG - 1586-1587 LID - 10.1002/ccr3.2778 [doi] AB - Respiratory complaints alone or in association with musculoskeletal complaints can be the predominant presenting feature of antisynthetase syndrome. Therefore, antibodies to cellular antigens should be evaluated in such clinical settings. CI - © 2020 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Mavragani, Clio P AU - Mavragani CP AUID- ORCID: 0000-0002-8214-7406 AD - Departments of Physiology and Pathophysiology School of Medicine National and Kapodistrian University of Athens Athens Greece. FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM AD - Chair Medical Sciences/Immunology Athens Academy Athens Greece. LA - eng PT - Journal Article DEP - 20200603 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC7455397 OTO - NOTNLM OT - Raynaud's OT - antibodies against cellular antigens OT - antisynthetase syndrome OT - interstitial lung disease COIS- None declared. EDAT- 2020/09/05 06:00 MHDA- 2020/09/05 06:01 PMCR- 2020/06/03 CRDT- 2020/09/05 06:00 PHST- 2019/08/21 00:00 [received] PHST- 2020/01/16 00:00 [revised] PHST- 2020/02/05 00:00 [accepted] PHST- 2020/09/05 06:00 [entrez] PHST- 2020/09/05 06:00 [pubmed] PHST- 2020/09/05 06:01 [medline] PHST- 2020/06/03 00:00 [pmc-release] AID - CCR32778 [pii] AID - 10.1002/ccr3.2778 [doi] PST - epublish SO - Clin Case Rep. 2020 Jun 3;8(8):1586-1587. doi: 10.1002/ccr3.2778. eCollection 2020 Aug. PMID- 26523153 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1758-4272 (Print) IS - 1758-4280 (Electronic) IS - 1758-4280 (Linking) VI - 5 IP - 3 DP - 2010 TI - Raynaud's phenomenon and digital ischemia: a practical approach to risk stratification, diagnosis and management. PG - 355-370 AB - Digital ischemia is a painful and often disfiguring event. Such an ischemic event often leads to tissue loss and can significantly affect the patient's quality of life. Digital ischemia can be secondary to a vasculopathy, vasculitis, embolic disease, trauma, or extrinsic vascular compression. It is an especially serious complication in patients with scleroderma. Risk stratification of patients with scleroderma at risk for digital ischemia is now possible with clinical assessment and autoantibody profiles. Because there are a variety of conditions that lead to digital ischemia, it is important to understand the pathophysiology underlying each ischemic presentation in order to target therapy appropriately. Significant progress has been made in the last two decades in defining the pathophysiological processes leading to digital ischemia in rheumatic diseases. In this article we review the risk stratification, diagnosis, and management of patients with digital ischemia and provide a practical approach to therapy, particularly in scleroderma. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224. LA - eng GR - T32 AR048522/AR/NIAMS NIH HHS/United States PT - Journal Article PL - England TA - Int J Clin Rheumtol JT - International journal of clinical rheumatology JID - 101503080 PMC - PMC4624318 MID - NIHMS638014 OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ischemia OT - digital ulcers OT - scleroderma EDAT- 2010/01/01 00:00 MHDA- 2010/01/01 00:01 PMCR- 2015/10/28 CRDT- 2015/11/03 06:00 PHST- 2015/11/03 06:00 [entrez] PHST- 2010/01/01 00:00 [pubmed] PHST- 2010/01/01 00:01 [medline] PHST- 2015/10/28 00:00 [pmc-release] AID - 10.2217/ijr.10.17 [doi] PST - ppublish SO - Int J Clin Rheumtol. 2010;5(3):355-370. doi: 10.2217/ijr.10.17. PMID- 41170218 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251031 LR - 20251102 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 9 DP - 2025 Sep TI - Dilemmas in the Management of Digital Ulcers in Systemic Sclerosis-Mixed Connective Tissue Disease: Lessons From a Case Report. PG - e93477 LID - 10.7759/cureus.93477 [doi] LID - e93477 AB - Systemic sclerosis (SSc) is a rare autoimmune disorder marked by fibrosis and microvascular injury, commonly manifesting as digital ulcers and skin thickening. Lower limb ulcers are uncommon but can contribute to significant morbidity, need prolonged healing, and carry the risk of progression to necrosis or amputation, particularly in systemic sclerosis-mixed connective tissue disease (SSc-MCTD). We report a case of a 57-year-old woman with Raynaud's phenomenon, inflammatory small-joint arthritis, alopecia, photosensitive rash, and exertional dyspnea. Despite improvement in arthritis with disease-modifying antirheumatic therapy, a lower limb ulcer progressed while she was on vasoactive therapy (α-adrenergic blockade, phosphodiesterase-5 inhibition, endothelin-receptor antagonism) and immunomodulation, culminating in necrosis and pain that required amputation of the great toe and subsequently the forefoot. Laboratory evaluation revealed systemic inflammation and dyslipidemia, while vascular imaging demonstrated both microvascular and macrovascular compromise. Histopathology confirmed acute inflammatory changes without primary vasculitis. Rheumatology evaluation and serology established a diagnosis of SSc-MCTD, after which management was refocused on targeted vasodilation, immunomodulatory therapy, and multidisciplinary wound care. This case underscores the central role of vasculopathy in SSc-MCTD, highlights the limited efficacy of surgical intervention without optimized medical management, and emphasizes the importance of early involvement of Rheumatology and coordinated multidisciplinary care to prevent progression to irreversible ischemic injury. CI - Copyright © 2025, Dadhich et al. FAU - Dadhich, Khushali AU - Dadhich K AD - Medicine, K. J. Somaiya Hospital and Research Centre, Mumbai, IND. AD - General Surgery, Bedfordshire Hospitals NHS Foundation Trust, Luton, GBR. FAU - Shah, Miet AU - Shah M AD - Medicine, K. J. Somaiya Hospital and Research Centre, Mumbai, IND. FAU - Jain, Ashish AU - Jain A AD - Medicine, K. J. Somaiya Hospital and Research Centre, Mumbai, IND. FAU - Gill, Niharika AU - Gill N AD - Medicine, K. J. Somaiya Hospital and Research Centre, Mumbai, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20250929 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12570281 OTO - NOTNLM OT - amputation OT - digital ulcers OT - lower-limb ulcers OT - mixed connective tissue disease OT - multidisciplinary care OT - raynaud’s phenomenon OT - systemic sclerosis OT - vasculopathy COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/10/31 06:26 MHDA- 2025/10/31 06:27 PMCR- 2025/09/29 CRDT- 2025/10/31 04:54 PHST- 2025/09/28 00:00 [accepted] PHST- 2025/10/31 06:27 [medline] PHST- 2025/10/31 06:26 [pubmed] PHST- 2025/10/31 04:54 [entrez] PHST- 2025/09/29 00:00 [pmc-release] AID - 10.7759/cureus.93477 [doi] PST - epublish SO - Cureus. 2025 Sep 29;17(9):e93477. doi: 10.7759/cureus.93477. eCollection 2025 Sep. PMID- 42226011 OWN - NLM STAT- Publisher LR - 20260601 IS - 1097-4598 (Electronic) IS - 0148-639X (Linking) DP - 2026 Jun 1 TI - A Rare Association of Anti-SRP Antibody-Positive Immune-Mediated Necrotizing Myopathy and Sjögren Disease: Two Case Reports. LID - 10.1002/mus.70304 [doi] AB - INTRODUCTION/AIMS: Immune-mediated necrotizing myopathy (IMNM) is an autoimmune myositis which is characterized by severe muscle fiber necrosis and refractoriness to immunotherapies. Overlap between myositis and other autoimmune disorders is well-recognized; however, the coexistence of IMNM and Sjögren disease (SjD) is rare. We investigated the clinicopathological features of anti-signal recognition particle antibody-positive IMNM associated with SjD. METHODS: We retrospectively reviewed previously reported cases and analyzed the clinical and pathological features of two patients: a 68-year-old man with a 2-year history of sicca symptoms, who presented a 3-month history of dysphagia, difficulty arising from a sitting position and gait disturbance, and a 67-year-old woman with a 20-year history of sicca symptoms, who subsequently developed muscle weakness, dysphagia, and hyperCKemia. RESULTS: Muscle pathological findings in both patients were consistent with those of IMNM without SjD. In contrast, they exhibited relatively mild limb weakness and showed a better response to immunotherapies compared with patients with typical IMNM. Notably, Patient 1 developed severe Raynaud's phenomenon and digital ulcers, requiring additional treatment. DISCUSSION: IMNM associated with SjD may present with relatively mild muscle involvement compared with IMNM without SjD; however, SjD-related disease activity may become more prominent during the clinical course. CI - © 2026 Wiley Periodicals LLC. FAU - Tanaka, Hiroaki AU - Tanaka H AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Nishimori, Yukako AU - Nishimori Y AUID- ORCID: 0009-0003-4418-4462 AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Eura, Nobuyuki AU - Eura N AUID- ORCID: 0000-0002-4943-3152 AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Sugata, Mayu AU - Sugata M AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Takeuchi, Katsuya AU - Takeuchi K AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Kobayashi, Masaki AU - Kobayashi M AD - Department of Neurology, Nara Medical University, Kashihara, Japan. FAU - Sugie, Kazuma AU - Sugie K AUID- ORCID: 0000-0003-0148-4687 AD - Department of Neurology, Nara Medical University, Kashihara, Japan. LA - eng PT - Journal Article DEP - 20260601 PL - United States TA - Muscle Nerve JT - Muscle & nerve JID - 7803146 SB - IM OTO - NOTNLM OT - Raynaud's phenomenon OT - Sjögren disease OT - immune‐mediated necrotizing myopathy OT - muscle pathology OT - myositis EDAT- 2026/06/02 00:35 MHDA- 2026/06/02 00:35 CRDT- 2026/06/01 23:42 PHST- 2026/05/16 00:00 [revised] PHST- 2026/01/24 00:00 [received] PHST- 2026/05/25 00:00 [accepted] PHST- 2026/06/02 00:35 [medline] PHST- 2026/06/02 00:35 [pubmed] PHST- 2026/06/01 23:42 [entrez] AID - 10.1002/mus.70304 [doi] PST - aheadofprint SO - Muscle Nerve. 2026 Jun 1. doi: 10.1002/mus.70304. PMID- 31708452 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20211008 LR - 20211008 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 17 IP - 5 DP - 2021 May TI - Clinical impact of nailfold capillaroscopy in daily clinical practice. PG - 258-262 LID - S1699-258X(19)30126-3 [pii] LID - 10.1016/j.reuma.2019.07.008 [doi] AB - INTRODUCTION: Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice. MATERIAL AND METHODS: All patients that undergone at least one NC between June 2012 and December 2017 were included. Clinical records were reviewed and analysed in a dichotomous way (yes/no), to see whether the NC contributed to a change of diagnosis in subsequent consultations. In addition, demographic, clinical and laboratory data were collected, and the relationship with NC patterns evaluated. RESULTS: Of the 530 patients who had undergone at least one NC, 266 had Raynaud's phenomenon as primary indication for the technique. Of those, 20 patients (3.8%) had a diagnostic change in the post-NC consultation; 15 were diagnosed with systemic sclerosis, 4 with undifferentiated connective tissue disease and one with mixed connective tissue disease. All patients had, except for one patient diagnosed with undifferentiated connective tissue disease, positive antinuclear antibodies titres, 11 of them had disease specific antibodies (9 anti-centromere, one anti-Scl70 and other anti-RNPC). The positivity of antinuclear antibodies titres was associated with a higher probability of presenting a scleroderma pattern in the NC, and all patients with a specific rheumatological diagnosis had an abnormal NC. CONCLUSION: NC is a useful technique, but with limited impact in the diagnosis of connective tissue diseases. Autoantibody positivity is associated with a greater likelihood of presenting pathological NC patterns. CI - Copyright © 2019 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Torrens Cid, Luis A AU - Torrens Cid LA AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. Electronic address: luistorrens.25@gmail.com. FAU - Soleto K, Christian Y AU - Soleto K CY AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Montoro-Álvarez, María AU - Montoro-Álvarez M AD - Pfizer, Madrid, España. FAU - Sáenz Tenorio, Claudia AU - Sáenz Tenorio C AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Silva-Riveiro, Alicia AU - Silva-Riveiro A AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - López-Cerón, Ana AU - López-Cerón A AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Anzola Alfaro, Ana M AU - Anzola Alfaro AM AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Caballero Motta, Liz Rocío AU - Caballero Motta LR AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Serrano Benavente, Belén AU - Serrano Benavente B AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Martínez-Barrio, Julia AU - Martínez-Barrio J AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Ovalles-Bonilla, Juan G AU - Ovalles-Bonilla JG AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - González Fernández, Carlos M AU - González Fernández CM AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Monteagudo Sáez, Indalecio AU - Monteagudo Sáez I AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Nieto-González, Juan Carlos AU - Nieto-González JC AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España. LA - eng LA - spa PT - Journal Article TT - Impacto clínico de la capilaroscopia periungueal en la práctica clínica diaria. DEP - 20191107 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM OTO - NOTNLM OT - Anticuerpos OT - Autoantibodies OT - Capilaroscopia periungueal OT - Clinical practice OT - Esclerodermia OT - Fenómeno de Raynaud OT - Nailfold capillaroscopy OT - Práctica clínica OT - Raynaud's phenomenon OT - Scleroderma EDAT- 2019/11/12 06:00 MHDA- 2019/11/12 06:01 CRDT- 2019/11/12 06:00 PHST- 2018/12/26 00:00 [received] PHST- 2019/04/20 00:00 [revised] PHST- 2019/07/29 00:00 [accepted] PHST- 2019/11/12 06:00 [pubmed] PHST- 2019/11/12 06:01 [medline] PHST- 2019/11/12 06:00 [entrez] AID - S1699-258X(19)30126-3 [pii] AID - 10.1016/j.reuma.2019.07.008 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2021 May;17(5):258-262. doi: 10.1016/j.reuma.2019.07.008. Epub 2019 Nov 7. PMID- 16618258 OWN - NLM STAT- MEDLINE DCOM- 20060726 LR - 20090202 IS - 0017-8748 (Print) IS - 0017-8748 (Linking) VI - 46 IP - 3 DP - 2006 Mar TI - Migraine is associated with menorrhagia and endometriosis. PG - 422-8 AB - OBJECTIVE: To evaluate the frequency of menorrhagia and endometriosis in female migraineurs compared to age-matched women without headache. BACKGROUND: Migraine predominantly affects women of childbearing age and is often associated with the menstrual period, yet there is a paucity of data regarding the relationship of migraine and menstrual disorders. METHODS: Women diagnosed with migraine, using International Headache Society criteria and an age- and sex-matched control group, were administered a semistructured questionnaire regarding migraine and migraine-related disability, menstrual history, other bleeding history, vascular event history, and vascular risk factors. RESULTS: Fifty female migraineurs between the ages of 22 and 50 years and 52 age-matched women (mean age 37 years) were enrolled in the study. Similar proportions of women in each group reported using hormone contraceptives (30% vs. 33%, P = .77) and hormone replacement therapy (12% vs. 8%, P = .69). The proportions presently menstruating (64 % vs. 80%, P = .20) and status after hysterectomy were similar (24% vs. 14%, P = .84). Menorrhagia (defined as at least three consecutive heavy periods), both current and prior, was more commonly reported in migraineurs (63% vs. 37%, P = .009), with higher likelihood of staining clothes by menses (35% vs. 8%, P = .003), and significant impact of menses on activities of daily living (on a 10-point Likert scale) with work/school participation (P = .02), family activities (P < .0001), sleep (P = .003), life enjoyment (P = .001), mood (P = .02), and overall quality of life (P = .003). Endometriosis, which may be associated with menorrhagia, was also more commonly diagnosed in the migraineurs (30% vs. 4%, P = .001). The migraineurs more frequently described bruising (40% vs. 10%, P < .001) and rectal bleeding (18% vs. 2%, P = .017) but not more serious bleeding problems. Nonsteroidal anti-inflammatory drug (NSAID) use was more frequent in the migraine group (28% vs. 12%, P = .036), and significance for increased menorrhagia, endometriosis, menstrual interference, and bruising was maintained, even when controlling for the use of NSAIDs. With logistic regression, menorrhagia was significantly associated with migraine, adjusted odds ratio (OR) = 2.8 (95% CI 1.2 to 6.5), and with endometriosis, adjusted OR = 10.5 (95% CI 2.2 to 51.4). There were no differences in vascular events and risk factors, except for trends of increased hypertension (25% vs. 10%, P = .05), transient ischemic attack/stroke (10% vs. 2%, P = .08), and Raynaud's disease (10% vs. 2%, P = .08) in the migraineurs. CONCLUSION: Women with migraine have a higher frequency of menorrhagia, endometriosis, and associated psychosocial consequences. These findings suggest that there should be further study of factors influencing endometriosis and menstrual blood flow, such as eicosanoids and platelet function, in migraineurs. FAU - Tietjen, Gretchen E AU - Tietjen GE AD - Department of Neurology, Medical College of Ohio, Toledo 43614, USA. FAU - Conway, Anita AU - Conway A FAU - Utley, Christine AU - Utley C FAU - Gunning, William T AU - Gunning WT FAU - Herial, Nabeel A AU - Herial NA LA - eng PT - Journal Article PL - United States TA - Headache JT - Headache JID - 2985091R SB - IM EIN - Headache. 2006 Mar;46(3):548 CIN - Headache. 2007 Mar;47(3):447-8. doi: 10.1111/j.1526-4610.2007.00736.x. PMID: 17371366 MH - Adult MH - Endometriosis/*complications MH - Female MH - Humans MH - Menorrhagia/*complications MH - Middle Aged MH - Migraine Disorders/*complications EDAT- 2006/04/19 09:00 MHDA- 2006/07/27 09:00 CRDT- 2006/04/19 09:00 PHST- 2006/04/19 09:00 [pubmed] PHST- 2006/07/27 09:00 [medline] PHST- 2006/04/19 09:00 [entrez] AID - HED290 [pii] AID - 10.1111/j.1526-4610.2006.00290.x [doi] PST - ppublish SO - Headache. 2006 Mar;46(3):422-8. doi: 10.1111/j.1526-4610.2006.00290.x. PMID- 28344932 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2220-3184 (Print) IS - 2220-3184 (Electronic) IS - 2220-3184 (Linking) VI - 6 IP - 2 DP - 2016 May 27 TI - Mutation in TNXB gene causes moderate to severe Ehlers-Danlos syndrome. PG - 17-21 LID - 10.5496/wjmg.v6.i2.17 [doi] AB - We report a 28-year-old female who presented with severe joint pain, chronic muscle weakness, Raynaud's phenomenon, and hypermobility. She was found to have a 6074A > T nucleotide transition in the TNXB gene causing an amino acid protein change at Asp2025Val classified as likely pathogenic. We add this clinical report to the literature and classical human disease gene catalogs to identify this specific mutation as disease-causing. This gene variant was reported previously in a different 36-year-old patient who shared our patient's symptoms of joint hypermobility, skeletal and joint pain, skin elasticity and musculoskeletal problems, thereby causing a more severe presentation than seen in the hypermobility type of Ehlers-Danlos syndrome (EDS). At the time of writing, a few mutations in the TNXB gene have been recognized as pathogenic causing EDS due to tenascin-X deficiency, but the variant identified in our patient has not been recognized as pathogenic in online genetic databases. Our case study in combination with peer-reviewed literature suggests that the 6074A > T nucleotide transition in the TNXB gene may be classified as disease-causing for EDS due to tenascin-X deficiency. FAU - Kaufman, Carolyn S AU - Kaufman CS AD - Department of Psychiatry, Behavioral Sciences and Pediatrics, University of Kansas Medical Center, Kansas City, KS 66160, United States. FAU - Butler, Merlin G AU - Butler MG AD - Department of Psychiatry, Behavioral Sciences and Pediatrics, University of Kansas Medical Center, Kansas City, KS 66160, United States. LA - eng GR - P30 HD002528/HD/NICHD NIH HHS/United States PT - Journal Article PL - United States TA - World J Med Genet JT - World journal of medical genetics JID - 101692087 PMC - PMC5363719 MID - NIHMS814447 OTO - NOTNLM OT - Ehlers-Danlos syndrome OT - Ehlers-Danlos syndrome due to tenascin-X deficiency OT - Genetic variants OT - Hypermobility OT - Joint pain OT - Muscle weakness OT - Mutations OT - Raynaud’s phenomenon OT - TNXB OT - Tenascin-X COIS- Conflict-of-interest statement: There are no conflicts of interest. EDAT- 2017/03/28 06:00 MHDA- 2017/03/28 06:01 PMCR- 2017/03/23 CRDT- 2017/03/28 06:00 PHST- 2017/03/28 06:00 [entrez] PHST- 2017/03/28 06:00 [pubmed] PHST- 2017/03/28 06:01 [medline] PHST- 2017/03/23 00:00 [pmc-release] AID - 10.5496/wjmg.v6.i2.17 [doi] PST - ppublish SO - World J Med Genet. 2016 May 27;6(2):17-21. doi: 10.5496/wjmg.v6.i2.17. PMID- 35382024 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250530 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 5 IP - 2 DP - 2020 Jun TI - Combination therapy with bosentan and sildenafil for refractory digital ulcers and Raynaud's phenomenon in a 30-year-old woman with systemic sclerosis: Case report and literature review. PG - 159-164 LID - 10.1177/2397198319876738 [doi] AB - BACKGROUND: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud's phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud's phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. METHODS: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud's phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. RESULTS: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud's phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. CONCLUSION: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results. CI - © The Author(s) 2019. FAU - Rademacher, Jan-Gerd AU - Rademacher JG AD - Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany. FAU - Wincup, Chris AU - Wincup C AD - Department of Rheumatology, University College London, London, UK. AD - Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, UK. FAU - Tampe, Björn AU - Tampe B AD - Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany. FAU - Korsten, Peter AU - Korsten P AUID- ORCID: 0000-0001-6065-5680 AD - Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany. LA - eng GR - 21992/VAC_/Versus Arthritis/United Kingdom PT - Journal Article DEP - 20190919 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922606 OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ulcers OT - endothelin receptor antagonists OT - phosphodiesterase inhibitors OT - systemic sclerosis COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2020/06/01 00:00 MHDA- 2020/06/01 00:01 PMCR- 2021/06/01 CRDT- 2022/04/06 05:10 PHST- 2019/07/31 00:00 [received] PHST- 2019/08/20 00:00 [accepted] PHST- 2022/04/06 05:10 [entrez] PHST- 2020/06/01 00:00 [pubmed] PHST- 2020/06/01 00:01 [medline] PHST- 2021/06/01 00:00 [pmc-release] AID - 10.1177_2397198319876738 [pii] AID - 10.1177/2397198319876738 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2020 Jun;5(2):159-164. doi: 10.1177/2397198319876738. Epub 2019 Sep 19. PMID- 33823201 OWN - NLM STAT- MEDLINE DCOM- 20210902 LR - 20210902 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 85 IP - 2 DP - 2021 Aug TI - The catch-22 of limited Food and Drug Administration approval for connective tissue disease therapies. PG - 517-519 LID - S0190-9622(21)00671-X [pii] LID - 10.1016/j.jaad.2021.03.103 [doi] FAU - Min, Michelle S AU - Min MS AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Kassamali, Bina AU - Kassamali B AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Mazori, Daniel R AU - Mazori DR AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Vleugels, Ruth Ann AU - Vleugels RA AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Merola, Joseph F AU - Merola JF AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; Division of Rheumatology, Immunology, and Allergy, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - Cobos, Gabriela AU - Cobos G AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. FAU - LaChance, Avery H AU - LaChance AH AD - Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address: avery.lachance@gmail.com. LA - eng PT - Journal Article DEP - 20210403 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Connective Tissue Diseases/*therapy MH - *Drug Approval MH - Humans MH - United States OTO - NOTNLM OT - Raynaud's OT - connective tissue disease OT - dermatomyositis OT - health policy prior authorization OT - lupus erythematosus OT - morphea OT - patient access OT - systemic sclerosis OT - vasculitis COIS- Conflicts of interest Dr Vleugels is a principal investigator for Pfizer. Dr Merola is a consultant for Abbvie, Dermavant, Eli Lilly, Novartis, Janssen, UCB, Celgene, Biogen, Pfizer, and Leo Pharma (honoraria). Drs Min, Mazori, Cobos, LaChance and author Kassamali have no conflicts of interest to declare. EDAT- 2021/04/07 06:00 MHDA- 2021/09/03 06:00 CRDT- 2021/04/06 20:09 PHST- 2021/02/25 00:00 [received] PHST- 2021/03/26 00:00 [revised] PHST- 2021/03/30 00:00 [accepted] PHST- 2021/04/07 06:00 [pubmed] PHST- 2021/09/03 06:00 [medline] PHST- 2021/04/06 20:09 [entrez] AID - S0190-9622(21)00671-X [pii] AID - 10.1016/j.jaad.2021.03.103 [doi] PST - ppublish SO - J Am Acad Dermatol. 2021 Aug;85(2):517-519. doi: 10.1016/j.jaad.2021.03.103. Epub 2021 Apr 3. PMID- 31046487 OWN - NLM STAT- MEDLINE DCOM- 20200611 LR - 20200611 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 15 IP - 7 DP - 2019 Jul TI - Pathophysiology of systemic sclerosis: current understanding and new insights. PG - 753-764 LID - 10.1080/1744666X.2019.1614915 [doi] AB - Introduction: Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease characterized by chronic and progressive tissue and organ fibrosis with broad patient-to-patient variability. Some risk factors are known and include combination of persistent Raynaud's phenomenon, steroid hormone imbalance, selected chemicals, thermal, or other injuries. Endogenous and/or exogenous environmental trigger/risk factors promote epigenetic mechanisms in genetically primed subjects. Disease pathogenesis presents early microvascular changes with endothelial cell dysfunction, followed by the activation of mechanisms promoting their transition into myofibroblasts. A complex autoimmune response, involving innate and adaptive immunity with specific/functional autoantibody production, characterizes the disease. Progressive fibrosis and ischemia involve skin and visceral organs resulting in their irreversible damage/failure. Progenitor circulating cells (monocytes, fibrocytes), together with growth factors and cytokines participate in disease diffusion and evolution. Epigenetic, vascular and immunologic mechanisms implicated in systemic fibrosis, represent major targets for incoming disease modifying therapeutic approaches. Areas covered: This review discusses current understanding and new insights of SSc pathogenesis, through an overview of the most relevant advancements to present aspects and mechanisms involved in disease pathogenesis. Expert opinion: Considering SSc intricacy/heterogeneity, early combination therapy with vasodilators, immunosuppressive and antifibrotic drugs should successfully downregulate the disease progression, especially if started from the beginning. FAU - Cutolo, Maurizio AU - Cutolo M AD - a Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine , University of Genova, IRCCS San Martino Polyclinic Hospital Genova , Genova , Italy. FAU - Soldano, Stefano AU - Soldano S AD - a Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine , University of Genova, IRCCS San Martino Polyclinic Hospital Genova , Genova , Italy. FAU - Smith, Vanessa AU - Smith V AD - b Department of Internal Medicine , Ghent University , Ghent , Belgium. AD - c Department of Rheumatology , Ghent University Hospital , Ghent , Belgium. AD - d Unit for Molecular Immunology and Inflammation , VIB Inflammation Research Center (IRC) , Ghent , Belgium. LA - eng PT - Journal Article PT - Review DEP - 20190513 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - 0 (Autoantibodies) RN - 0 (Gonadal Steroid Hormones) SB - IM MH - Animals MH - Autoantibodies/immunology MH - *Autoimmunity MH - Gonadal Steroid Hormones/immunology MH - Humans MH - *Scleroderma, Systemic/drug therapy/immunology/pathology/physiopathology MH - *Stem Cells/immunology/pathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - autoimmune rheumatic diseases OT - connective tissue diseases OT - endothelial cells OT - epigenetic OT - fibrocytes myofibroblasts OT - growth factors OT - immune response OT - macrophages OT - nailfold capillaroscopy EDAT- 2019/05/03 06:00 MHDA- 2020/06/12 06:00 CRDT- 2019/05/04 06:00 PHST- 2019/05/03 06:00 [pubmed] PHST- 2020/06/12 06:00 [medline] PHST- 2019/05/04 06:00 [entrez] AID - 10.1080/1744666X.2019.1614915 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2019 Jul;15(7):753-764. doi: 10.1080/1744666X.2019.1614915. Epub 2019 May 13. PMID- 35299881 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220319 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 14 DP - 2022 TI - Prevalence of Raynaud's Phenomenon in Saudi Arabia. PG - 17-24 LID - 10.2147/OARRR.S352655 [doi] AB - PURPOSE: Raynaud's phenomenon (RP) is defined as frequent ischaemic attacks in the fingers and toes due to vascular vasospasm. Studies have been conducted in many countries worldwide to determine the prevalence of RP. The aim of the current study was to assess the prevalence of RP in the Saudi Arabian population. PATIENTS AND METHODS: An online survey based on international consensus criteria used to diagnose RP was conducted to collect data from individuals from the Saudi population. Participants were considered positive if they had triphasic or biphasic colours of the extremities with cold-related sensitivity. Awareness of RP was also assessed. RESULTS: A total of 1025 responses were collected and included in the final analysis. The prevalence of RP was 4.29%, including 22% men and 77% women. The most common age group among women was 26-40 years (36.3%). Familiarity with RP was low, with 56.82% of participants lacking adequate awareness regarding RP. Only 32% of patients with RP reported attending doctor visits regarding symptoms of the disease. CONCLUSION: The RP prevalence in Saudi Arabia is comparable to that reported in the international literature. Public awareness activities should be conducted to increase knowledge about RP. TRIAL REGISTRATION: Not applicable. CI - © 2022 Mustafa et al. FAU - Mustafa, Mohammad AU - Mustafa M AUID- ORCID: 0000-0003-0170-3747 AD - Department of Medicine, Rheumatology Unit, University of Jeddah, Jeddah, Saudi Arabia. FAU - Alsulaimani, Hadeel AU - Alsulaimani H AUID- ORCID: 0000-0002-8979-6011 AD - Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia. FAU - Alhaddad, Abdulrahman AU - Alhaddad A AD - Faculty of Medicine, King Saud Bin Abdulaziz University for Health and Science, Jeddah, Saudi Arabia. FAU - Almujil, Sara AU - Almujil S AD - Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia. FAU - Albar, Zainab AU - Albar Z AUID- ORCID: 0000-0002-2685-7382 AD - School of Medicine, Umm Al Qura University, Makkah, Saudi Arabia. FAU - Bawazir, Yasser AU - Bawazir Y AUID- ORCID: 0000-0002-5060-3884 AD - Rheumatology Unit, Department of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia. FAU - Alsolaimani, Roaa AU - Alsolaimani R AD - Rheumatology Unit, Department of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia. FAU - Omair, Mohammed A AU - Omair MA AUID- ORCID: 0000-0002-9373-5473 AD - Division of Rheumatology, Department of Medicine, King Saud University, Riyadh, Saudi Arabia. LA - eng PT - Journal Article DEP - 20220309 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC8922445 OTO - NOTNLM OT - Raynaud’s phenomenon OT - awareness OT - cold sensitivity COIS- The authors report no conflicts of interest in this work. EDAT- 2022/03/19 06:00 MHDA- 2022/03/19 06:01 PMCR- 2022/03/09 CRDT- 2022/03/18 05:12 PHST- 2021/12/09 00:00 [received] PHST- 2022/03/01 00:00 [accepted] PHST- 2022/03/18 05:12 [entrez] PHST- 2022/03/19 06:00 [pubmed] PHST- 2022/03/19 06:01 [medline] PHST- 2022/03/09 00:00 [pmc-release] AID - 352655 [pii] AID - 10.2147/OARRR.S352655 [doi] PST - epublish SO - Open Access Rheumatol. 2022 Mar 9;14:17-24. doi: 10.2147/OARRR.S352655. eCollection 2022. PMID- 30534427 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2067-0656 (Print) IS - 2069-4032 (Electronic) VI - 41 IP - 3 DP - 2015 Jul-Sep TI - Contribution of Nail Fold Videocapillaroscopy in Patients with Early Inflammatory Arthritis. PG - 233-238 LID - 10.12865/CHSJ.41.03.07 [doi] AB - BACKGROUND: Early inflammatory arthritis (EIA) a condition defined by joint swelling, with or without morning stiffness, and/or swelling of metacarpophalangeal and/or metatarsophalangeal joints. Nail fold video-capillaroscopy (NVC) is important for the evaluation of microcirculation in vivo. There are limited data about the role of NVC in EIA. OBJECTIVES: To study the capillaroscopic pattern in patients with EIA. PATIENTS AND METHODS: 27 patients with EIA - 21 women, 6 men, mean age of 41.6±4.2 yrs, mean disease duration of 6.9±3.1 months. Anamnesis and clinical examination were perform to identify clinical signs associated with connective tissue diseases. All the patients were nonsmokers and had no personal medical history of diabetes or non-immune mediated occlusive vascular disease. Blood and urine samples were collected for bio-chemical and immunological evaluation. All the patients were interrogated by NVC. RESULTS: Raynaud's phenomenon was found in 9 patients, puffy fingers in 2 pts, telangiectasia in 2 pts, Sicca symptoms in 6 pts, malar rash in 2 pts, photosensitivity and psoriasis plaque in one patient. Combined information from clinical exam, NVC and immunological assessment allowed a specific diagnosis in 5 patients. CONCLUSIONS: Nail fold capillaroscopy assessment can provide further information and has diagnostic value in some cases of early arthritis. Nail fold capillaroscopy assessment contribution to the differential diagnosis in patients with early arthritis is sometimes significant, especially in poorly clinical and immunological defined cases. FAU - Florea, M AU - Florea M AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. FAU - Roşu, A AU - Roşu A AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. FAU - Vreju, F A AU - Vreju FA AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. FAU - Muşetescu, A E AU - Muşetescu AE AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. FAU - Criveanu, C AU - Criveanu C AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. FAU - Ciurea, P AU - Ciurea P AD - Rheumatology Department, University of Medicine and Pharmacy of Craiova, Faculty of Medicine, Romania. LA - eng PT - Journal Article DEP - 20150315 PL - Romania TA - Curr Health Sci J JT - Current health sciences journal JID - 101597164 PMC - PMC6246983 OTO - NOTNLM OT - early inflammatory arthritis OT - Raynaud`s phenomenon OT - antinuclear antibodies OT - capillaroscopy EDAT- 2015/07/01 00:00 MHDA- 2015/07/01 00:01 PMCR- 2015/07/01 CRDT- 2018/12/12 06:00 PHST- 2015/02/27 00:00 [received] PHST- 2015/03/15 00:00 [accepted] PHST- 2018/12/12 06:00 [entrez] PHST- 2015/07/01 00:00 [pubmed] PHST- 2015/07/01 00:01 [medline] PHST- 2015/07/01 00:00 [pmc-release] AID - 2015.03.07 [pii] AID - 10.12865/CHSJ.41.03.07 [doi] PST - ppublish SO - Curr Health Sci J. 2015 Jul-Sep;41(3):233-238. doi: 10.12865/CHSJ.41.03.07. Epub 2015 Mar 15. PMID- 39350525 OWN - NLM STAT- Publisher LR - 20250104 IS - 1533-2500 (Electronic) IS - 1530-7085 (Print) IS - 1530-7085 (Linking) VI - 25 IP - 1 DP - 2024 Sep 30 TI - 12. Vascular pain: Ischemic pain in the extremities and Raynaud's syndrome. LID - 10.1111/papr.13421 [doi] LID - e13421 AB - INTRODUCTION: Peripheral artery diseases (PAD) and Raynaud's syndrome are associated with substantial morbidity. PAD, through the restriction of blood flow to the extremities, may lead to critical limb ischemia with symptoms of pain at rest which may eventually progress to severe limb ischemia with gangrene. This serious and painful clinical condition requires extensive medical care, is limb-threatening and, in case of delayed or unsuccessful treatment, is associated with a high mortality rate. In Raynaud's syndrome, the blood supply to certain parts of the body, usually the fingers and toes and less frequently the nose or ears, is restricted because of vasculopathy of the smaller vessels at acral sites. Under certain circumstances, with cold as the most well-known provoking factor, blood flow restriction occurs, leading to demarcated color changes and symptoms such as pain, paresthesia, and numbness. In severe cases of Raynaud syndrome tissue ischemia may lead to necrosis and the need for amputation of the affected area. METHODS: In this narrative review, the literature on the diagnosis and interventional pain treatment of PAD and Raynaud's syndrome was updated and summarized. OBJECTIVES: This review focused on interventional pain treatment. In PAD, the effects of the intervention on limb salvage, ulcer healing, and ischemic pain were summarized. Additionally, results with respect to skin microcirculation and quality of life were reported if available. In Raynaud's syndrome, we focused on the effect of the intervention on peripheral blood flow metrics and pain intensity during attacks. RESULTS: In PAD, prevention and treatment of risk factors are important. Initially, conservative treatment and pharmacological therapy are preferred first-line therapies. However, when disease progression occurs, interventional management may be considered. The literature search yielded conflicting evidence for sympathectomy as a treatment for PAD. Spinal cord stimulation (SCS) as a treatment modality for advanced PAD had high-quality evidence for limb salvage in subgroups of patients but conflicting evidence for other outcome measures such as pain, wound healing, and quality of life. The literature search for interventional pain management in Raynaud's syndrome was limited to only one randomized controlled trial (RCT) studying the effect of thoracic sympathectomy. This study had several limitations and hence the level of evidence for this interventional treatment is very low. No RCTs studying SCS in patients with Raynaud's syndrome were found. CONCLUSIONS: In both PAD and Raynaud's syndrome, additional RCTs are needed to substantiate interventional (pain) management and bolster the evidence base for sympathectomy and SCS as treatment options. CI - © 2024 The Author(s). Pain Practice published by Wiley Periodicals LLC on behalf of World Institute of Pain. FAU - van der Gaag, Antal AU - van der Gaag A AD - Anesthesiology and Pain Medicine, Catharina Ziekenhuis, Eindhoven, The Netherlands. FAU - Cohen, Steven P AU - Cohen SP AD - Anesthesiology, Neurology, Physical Medicine & Rehabilitation and Psychiatry and Behavioral Sciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA. AD - Anesthesiology and Physical Medicine & Rehabilitation, Walter Reed National Military Medical Center, Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA. FAU - Stojanovic, Milan P AU - Stojanovic MP AD - Department of Anesthesiology, Critical Care and Pain Medicine Service, VA Boston Healthcare System, Boston, Massachusetts, USA. AD - VA Bedford Healthcare System, Harvard Medical School, Boston, Massachusetts, USA. FAU - Huygen, Frank J P M AU - Huygen FJPM AD - Department of Anesthesiology and Pain Management, Erasmus Medical Centre, Rotterdam, The Netherlands. AD - Department of Anesthesiology and Pain Management, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Kallewaard, Jan Willem AU - Kallewaard JW AD - Anesthesiology and Pain Medicine, Rijnstate Ziekenhuis, Elst, The Netherlands. AD - Anesthesiology and Pain Medicine, Amsterdam University Medical Centers, Amsterdam, The Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20240930 PL - United States TA - Pain Pract JT - Pain practice : the official journal of World Institute of Pain JID - 101130835 SB - IM PMC - PMC11683193 OTO - NOTNLM OT - Raynaud's syndrome OT - evidence‐based medicine OT - ischemic pain OT - peripheral arterial disease COIS- Frank Huygen is an Editorial Board member of Pain Practice and a co‐author of this article. To minimize bias, he was excluded from all editorial decision‐making related to the acceptance of this article for publication. The other authors declare no conflicts of interest. EDAT- 2024/10/01 06:55 MHDA- 2024/10/01 06:55 PMCR- 2024/12/30 CRDT- 2024/10/01 02:12 PHST- 2024/10/01 06:55 [medline] PHST- 2024/10/01 06:55 [pubmed] PHST- 2024/10/01 02:12 [entrez] PHST- 2024/12/30 00:00 [pmc-release] AID - PAPR13421 [pii] AID - 10.1111/papr.13421 [doi] PST - aheadofprint SO - Pain Pract. 2024 Sep 30;25(1):e13421. doi: 10.1111/papr.13421. PMID- 41921883 OWN - NLM STAT- Publisher LR - 20260409 IS - 1879-3649 (Electronic) IS - 1537-1891 (Linking) VI - 163 DP - 2026 Mar 30 TI - Nanocarrier-based drug delivery strategies for Raynaud's phenomenon: Overcoming microvascular and perfusion barriers. PG - 107604 LID - S1537-1891(26)00024-8 [pii] LID - 10.1016/j.vph.2026.107604 [doi] AB - Raynaud's phenomenon (RP) is a functional vascular disorder characterized by episodic vasospasm of the digital microcirculation, resulting in transient ischemia, sensory disturbances, and pain. Despite long-standing clinical recognition, effective pharmacological management of RP remains challenging due to perfusion-limited drug delivery, short drug residence time at ischemic sites, and frequent systemic adverse effects associated with conventional vasodilator therapies. These limitations are particularly pronounced in secondary RP, where structural microvasculature damage further compromises therapeutic efficacy. Emerging nanocarrier-based drug delivery systems offer a promising strategy to address these challenges by enhancing drug stability, improving site-specific accumulation, and enabling controlled or stimuli-responsive release in affected tissues. This review examines RP from a drug-delivery-oriented perspective, highlighting how nanocarriers such as liposomes, polymeric nanoparticles, dendrimers, micelles, and nanoemulsions can overcome RP-specific vascular and cutaneous barriers. Particular emphasis is placed on aligning nanocarrier design with RP pathophysiology, including episodic ischemia, endothelial dysfunction, oxidative stress, and differences between primary and secondary disease. Route-specific delivery strategies and translational considerations, including manufacturing and regulatory challenges, are also discussed. By reframing RP as a delivery-limited disorder rather than solely a pharmacological one, this review provides a translational framework for the rational development of next-generation, personalized nanomedicine-based therapies for RP. CI - Copyright © 2026. Published by Elsevier Inc. FAU - Shanmugam, Sangeetha AU - Shanmugam S AD - SRM College of Pharmacy, Faculty of Medicine and Health Sciences, SRM Institute of Science and Technology, Kattankulathur, Chengalpattu, Tamil Nadu, India. Electronic address: sangeets2@srmist.edu.in. FAU - Muthu, Sandya AU - Muthu S AD - SRM College of Pharmacy, Faculty of Medicine and Health Sciences, SRM Institute of Science and Technology, Kattankulathur, Chengalpattu, Tamil Nadu, India. Electronic address: sm3991@srmist.edu.in. LA - eng PT - Journal Article PT - Review DEP - 20260330 PL - United States TA - Vascul Pharmacol JT - Vascular pharmacology JID - 101130615 SB - IM OTO - NOTNLM OT - Drug delivery OT - Liposomes OT - Nanocarriers OT - Oxidative stress OT - Personalized medicine OT - Polymeric nanoparticles OT - Raynaud's phenomenon COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/04/02 00:29 MHDA- 2026/04/02 00:29 CRDT- 2026/04/01 19:16 PHST- 2026/02/04 00:00 [received] PHST- 2026/03/24 00:00 [revised] PHST- 2026/03/24 00:00 [accepted] PHST- 2026/04/02 00:29 [pubmed] PHST- 2026/04/02 00:29 [medline] PHST- 2026/04/01 19:16 [entrez] AID - S1537-1891(26)00024-8 [pii] AID - 10.1016/j.vph.2026.107604 [doi] PST - aheadofprint SO - Vascul Pharmacol. 2026 Mar 30;163:107604. doi: 10.1016/j.vph.2026.107604. PMID- 35371438 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220405 IS - 2048-8505 (Print) IS - 2048-8513 (Electronic) IS - 2048-8505 (Linking) VI - 15 IP - 4 DP - 2022 Apr TI - Raynaud's phenomenon triggered by the vasopressin V(2) receptor antagonist tolvaptan in a patient with autosomal dominant polycystic kidney disease and Sjögren's syndrome. PG - 827-828 LID - 10.1093/ckj/sfab260 [doi] FAU - Roca Oporto, F J AU - Roca Oporto FJ AUID- ORCID: 0000-0001-8297-3129 AD - Hospital Universitario Virgen del Rocio, Nephrology, Sevilla, Spain. FAU - Rocha, Jose L AU - Rocha JL AD - Hospital Universitario Virgen del Rocio, Nephrology, Sevilla, Spain. LA - eng PT - Journal Article DEP - 20211209 PL - England TA - Clin Kidney J JT - Clinical kidney journal JID - 101579321 PMC - PMC8967664 EDAT- 2022/04/05 06:00 MHDA- 2022/04/05 06:01 PMCR- 2021/12/09 CRDT- 2022/04/04 05:30 PHST- 2021/11/20 00:00 [received] PHST- 2022/04/04 05:30 [entrez] PHST- 2022/04/05 06:00 [pubmed] PHST- 2022/04/05 06:01 [medline] PHST- 2021/12/09 00:00 [pmc-release] AID - sfab260 [pii] AID - 10.1093/ckj/sfab260 [doi] PST - epublish SO - Clin Kidney J. 2021 Dec 9;15(4):827-828. doi: 10.1093/ckj/sfab260. eCollection 2022 Apr. PMID- 36601192 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230111 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 12 DP - 2022 Dec TI - Positive Regadenoson Stress Test in a Patient on Phentermine With Normal Coronaries. PG - e32138 LID - 10.7759/cureus.32138 [doi] LID - e32138 AB - Obesity is a well-established cardiovascular (CV) risk factor with greater mortality and morbidity rates than the general population. Phentermine is a weight loss medication that is approved for short-term obesity treatment in conjunction with lifestyle modifications to decrease CV risk. A 51-year-old female with Raynaud's phenomenon who was started on phentermine one week prior presented with a one-day history of palpitations. Subsequent workup revealed non-ST elevation myocardial infarction (NSTEMI) on presentation and worsening ST segment depressions following regadenoson injection during pharmacological stress testing. Although current evidence suggests that the use of phentermine is safe and may even reduce the risk of CV disease in obese patients, it still may pose adverse CV effects. A detailed medical history, including medications used and predisposing conditions, is crucial to help identify and possibly prevent exacerbation of such CV side effects. CI - Copyright © 2022, Sendil et al. FAU - Sendil, Selin AU - Sendil S AD - Cardiology, Mount Sinai Medical Center, Miami, USA. FAU - Gjergjindreaj, Medeona AU - Gjergjindreaj M AD - Cardiology, Mount Sinai Medical Center, Miami, USA. FAU - Lozier, Matthew AU - Lozier M AD - Cardiology, Mount Sinai Medical Center, Miami, USA. FAU - Fernandez, Rafle AU - Fernandez R AD - Cardiology, Mount Sinai Medical Center, Miami, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20221202 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9805544 OTO - NOTNLM OT - coronary artery vasospasm OT - non-st elevation myocardial infarction OT - nstemi OT - phentermine OT - raynaud’s phenomenon OT - regadenoson COIS- The authors have declared that no competing interests exist. EDAT- 2023/01/06 06:00 MHDA- 2023/01/06 06:01 PMCR- 2022/12/02 CRDT- 2023/01/05 02:26 PHST- 2022/12/01 00:00 [accepted] PHST- 2023/01/05 02:26 [entrez] PHST- 2023/01/06 06:00 [pubmed] PHST- 2023/01/06 06:01 [medline] PHST- 2022/12/02 00:00 [pmc-release] AID - 10.7759/cureus.32138 [doi] PST - epublish SO - Cureus. 2022 Dec 2;14(12):e32138. doi: 10.7759/cureus.32138. eCollection 2022 Dec. PMID- 36465326 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221206 IS - 2719-4523 (Electronic) IS - 2719-4523 (Linking) VI - 3 IP - 2 DP - 2022 Jun TI - The Relationship between Smoking, Raynaud's Phenomenon, Digital Ulcers, and Skin Thickness in the Waikato Systemic Sclerosis Cohort. PG - 84-89 LID - 10.2478/rir-2022-0014 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a heterogeneous complex autoimmune connective tissue disease with variable presentation as a consequence of multisystem involvement. One of the key features of SSc is Raynaud's phenomenon along with vascular endothelial dysfunction that leads to digital ulcers (DUs). Raynaud's tends to be triggered by decreasing thermal gradient exposure, while stress and smoking also play a role. DUs arising as a consequence of severe Raynaud's and vasculopathy are a major cause of morbidity and disability in SSc. We set out to determine the relationship between smoking, Raynaud's phenomenon, DUs, and skin thickness in our Waikato Systemic Sclerosis cohort. METHODS: The Waikato Systemic Sclerosis (SSc) database was used to extract data. Variables collected included demographics, age of diagnosis, SSc subtypes, age at first non-Raynaud's phenomenon, medications used for treatment of Raynaud's phenomenon or ulcers, and maximal modified Rodnan skin score (mRSS). Raynaud's phenomenon and finger DUs (severity for each over the past week and since diagnosis) and a Scleroderma Health Assessment Questionnaire (SHAQ) visual analog 10 cm scale were collected. The lead rheumatologist completed a physician's assessment of Raynaud's and the disease severity questionnaire. RESULTS: Of the cohort of 143 patients, 100 patients were eligible to complete the questionnaires. Seventy-five patients returned completed questionnaires. Of these, the majority were female (88%), 52 (69.3%) had limited cutaneous systemic sclerosis (lcSSc), 17 (22.7%) had diffuse cutaneous systemic sclerosis (dcSSc), and 6 (8%) had an overlap syndrome. Thirty-six (48%) had a smoking history (in the time frame of collection of serial data). Mean ± standard deviation (SD) pack-years smoked were 17.11 ± 15.29 years. Thirty-five participants had a history of DUs, with a median of 4 DU (range 1-20). Of 17 patients with dcSSc, 12 (70.6%) had ulcers in comparison with 17 of 52 (32.7%) patients with lcSSc. There was a significant relationship between SSc subtype and the number with ulcers (X(2) = 10.1, P = 0.007). There was also a significant relationship between physician severity of Raynaud's and presence of ulcers (t = 6.1, P < 0.001), which was not evident between patients' severity of Raynaud's and presence of ulcers (t = 1.9, P = 0.06). On the SHAQ score, smokers had significantly worse Raynaud's phenomenon over the prior week (t = 3.08, P = 0.03) and were more likely to note DUs over the preceding week, although the latter was not statistically significant (t = 1.95, P = 0.055). There was no association between smoking and skin thickness as measured by mRSS (r = 0.23, P = 0.19). CONCLUSION: Our study demonstrates that smokers have had worse Raynaud's phenomenon over the past week and they were also more likely to note DUs with a trend toward significance but not statistically significant most likely due to our small sample size. Our study also demonstrated that patients with dcSSc had more ulcers in comparison with lcSSc. This study justifies physicians strongly recommending smoking cessation in patients with SSc. CI - © 2022 Cherumi Silva et al., published by Sciendo. FAU - Silva, Cherumi AU - Silva C AD - Rheumatology Department, Waikato Hospital, Private Bag, Pembroke Street, Hamilton, New Zealand. FAU - Solanki, Kamal K AU - Solanki KK AD - Rheumatology Department, Waikato Hospital, Private Bag, Pembroke Street, Hamilton, New Zealand. FAU - White, Douglas H N AU - White DHN AD - Rheumatology Department, Waikato Hospital, Private Bag, Pembroke Street, Hamilton, New Zealand. LA - eng PT - Journal Article DEP - 20220706 PL - Germany TA - Rheumatol Immunol Res JT - Rheumatology and immunology research JID - 9918419176206676 PMC - PMC9524819 OTO - NOTNLM OT - Raynaud's; skin thickness OT - digital ulcers OT - sclerosis OT - smoking OT - systemic COIS- Conflict of Interest None. EDAT- 2022/12/06 06:00 MHDA- 2022/12/06 06:01 PMCR- 2022/07/06 CRDT- 2022/12/05 03:38 PHST- 2022/03/17 00:00 [received] PHST- 2022/05/09 00:00 [accepted] PHST- 2022/12/05 03:38 [entrez] PHST- 2022/12/06 06:00 [pubmed] PHST- 2022/12/06 06:01 [medline] PHST- 2022/07/06 00:00 [pmc-release] AID - rir-2022-0014 [pii] AID - 10.2478/rir-2022-0014 [doi] PST - epublish SO - Rheumatol Immunol Res. 2022 Jul 6;3(2):84-89. doi: 10.2478/rir-2022-0014. eCollection 2022 Jun. PMID- 24381778 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140101 LR - 20211021 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2013 DP - 2013 TI - Coexistence of sarcoidosis and systemic sclerosis. PG - 684216 LID - 10.1155/2013/684216 [doi] LID - 684216 AB - Sarcoidosis is a multisystem granulomatous disease characterized by hilar lymphadenopathy, involvement of internal organs, and diverse skin lesions. Systemic sclerosis is an autoimmune disease characterized by skin hardening and different internal organ fibrosis, including vascular abnormality. Immune response associated with Th-2 has been shown in the early and active stage of the disease. In this paper, we report coexistence of systemic sclerosis with sarcoidosis in a female patient presenting with granulomatous dermatitis, interstitial lung disease, and Raynaud's phenomenon complaints. FAU - Kobak, Senol AU - Kobak S AD - Department of Rheumatology, Faculty of Medicine, Sifa University, Bornova, 35100 Izmir, Turkey. FAU - Sever, Fidan AU - Sever F AD - Department of Pulmonary Diseases, Faculty of Medicine, Sifa University, 35100 Izmir, Turkey. FAU - Sivrikoz, Oya AU - Sivrikoz O AD - Department of Pathology, Faculty of Medicine, Sifa University, 35100 Izmir, Turkey. FAU - Karaarslan, Ahmet AU - Karaarslan A AD - Department of Ortopedics, Faculty of Medicine, Sifa University, 35100 Izmir, Turkey. LA - eng PT - Journal Article DEP - 20131205 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC3870080 EDAT- 2014/01/02 06:00 MHDA- 2014/01/02 06:01 PMCR- 2013/12/05 CRDT- 2014/01/02 06:00 PHST- 2013/10/23 00:00 [received] PHST- 2013/11/14 00:00 [accepted] PHST- 2014/01/02 06:00 [entrez] PHST- 2014/01/02 06:00 [pubmed] PHST- 2014/01/02 06:01 [medline] PHST- 2013/12/05 00:00 [pmc-release] AID - 10.1155/2013/684216 [doi] PST - ppublish SO - Case Rep Rheumatol. 2013;2013:684216. doi: 10.1155/2013/684216. Epub 2013 Dec 5. PMID- 39524967 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241114 IS - 2514-1775 (Electronic) IS - 2514-1775 (Linking) VI - 8 IP - 4 DP - 2024 TI - GP consultations and prescribed treatments for Raynaud's: a survey among primary Raynaud's patients in the community. PG - rkae126 LID - 10.1093/rap/rkae126 [doi] LID - rkae126 FAU - Chen, Anthony AU - Chen A AUID- ORCID: 0000-0002-7466-6476 AD - Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. FAU - Lax, Stephanie J AU - Lax SJ AD - Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. FAU - Grainge, Matthew J AU - Grainge MJ AUID- ORCID: 0000-0001-7181-4042 AD - Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. FAU - Lanyon, Peter C AU - Lanyon PC AD - Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. FAU - Pearce, Fiona A AU - Pearce FA AUID- ORCID: 0000-0003-2884-1998 AD - Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. FAU - Jeffries-Owens, Nick AU - Jeffries-Owens N AD - Scleroderma and Raynaud's UK, London, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK. AD - Federation of European Scleroderma Associations (FESCA), Belgium. AD - Rare Autoimmune Rheumatic Disease Alliance (RAIRDA) Patient Information Forum, London, UK. LA - eng PT - Journal Article DEP - 20241008 PL - England TA - Rheumatol Adv Pract JT - Rheumatology advances in practice JID - 101736676 PMC - PMC11543524 EDAT- 2024/11/14 03:55 MHDA- 2024/11/14 03:56 PMCR- 2024/10/08 CRDT- 2024/11/11 05:35 PHST- 2024/09/20 00:00 [accepted] PHST- 2024/11/14 03:56 [medline] PHST- 2024/11/14 03:55 [pubmed] PHST- 2024/11/11 05:35 [entrez] PHST- 2024/10/08 00:00 [pmc-release] AID - rkae126 [pii] AID - 10.1093/rap/rkae126 [doi] PST - epublish SO - Rheumatol Adv Pract. 2024 Oct 8;8(4):rkae126. doi: 10.1093/rap/rkae126. eCollection 2024. PMID- 34646694 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211015 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 9 DP - 2021 Sep TI - Persistent Raynaud's Phenomenon Following Methylphenidate Hydrochloride Use During the COVID-19 Pandemic. PG - e17647 LID - 10.7759/cureus.17647 [doi] LID - e17647 AB - Raynaud's phenomenon (RP) is a medical condition characterized by vasospasm of the digital vessels in the fingers and toes. The prevalence of RP in the general population is estimated at 3-5% and can vary based on climate. It is classified into primary and secondary RP based on causality. RP has been reported in some cases diagnosed with coronavirus disease 2019 (COVID-19) infection. We report the case of a 14-year-old Caucasian female who presented during the pandemic with chief complaints of suicidal ideations and attempted suicide and had a history of attention-deficit hyperactivity disorder (ADHD) and persistent RP after a stimulant trial. After an initial failure of treatment with lisdexamfetamine, she was switched to methylphenidate hydrochloride (MPH). Within two months of starting MPH, the patient noticed skin discoloration of the lower legs and feet along with numbness. The discoloration of skin was mainly limited to her feet and gradually moved up her legs. She was advised to discontinue the MPH, but her symptoms persisted for four more months until her admission. Other etiologies were ruled out by multi-specialties and during her hospitalization. She was started on atomoxetine and buspirone with appropriate dose titration. Post-discharge from the hospital, no improvement was observed in the patient's RP at an outpatient follow-up performed within a month. The development of RP following MPH treatment and its persistence after stopping MPH is a fascinating event. Clinicians should be aware of the potential rare side effects of stimulants and stimulant-like medications, including vascular, hematological, and dermatological effects. Adolescents with ADHD may be particularly distressed by the COVID-19 pandemic and display increased behavioral issues. Stress can be a trigger for RP; therefore, minimizing stress in at-risk patients is essential. CI - Copyright © 2021, Laboe et al. FAU - Laboe, Christopher AU - Laboe C AD - Psychiatry, Penn State College of Medicine, Hershey, USA. FAU - Batchelder, Emma AU - Batchelder E AD - Psychiatry, University of Virginia, Charlottesville, USA. FAU - Vasireddy, Deepa AU - Vasireddy D AD - Pediatrics, Pediatric Group of Acadiana, Lafayette, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210901 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8486148 OTO - NOTNLM OT - adhd OT - covid-19 OT - methylphenidate hydrochloride OT - raynaud’s phenomenon OT - stimulant OT - suicide and depression COIS- The authors have declared that no competing interests exist. EDAT- 2021/10/15 06:00 MHDA- 2021/10/15 06:01 PMCR- 2021/09/01 CRDT- 2021/10/14 06:37 PHST- 2021/09/01 00:00 [accepted] PHST- 2021/10/14 06:37 [entrez] PHST- 2021/10/15 06:00 [pubmed] PHST- 2021/10/15 06:01 [medline] PHST- 2021/09/01 00:00 [pmc-release] AID - 10.7759/cureus.17647 [doi] PST - epublish SO - Cureus. 2021 Sep 1;13(9):e17647. doi: 10.7759/cureus.17647. eCollection 2021 Sep. PMID- 41346899 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251205 LR - 20251207 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 11 DP - 2025 Nov TI - Gastric Antral Vascular Ectasia Preceding the Diagnosis of Limited Cutaneous Systemic Sclerosis. PG - e96021 LID - 10.7759/cureus.96021 [doi] LID - e96021 AB - Systemic sclerosis is an autoimmune connective tissue disease that affects multiple organ systems, leading to diverse clinical presentations. Raynaud's phenomenon is one of the most common early manifestations, reflecting the underlying vasculopathy. In rare cases, vascular changes also involve internal organs, including the gastrointestinal tract. We present the case of a 79-year-old female who first presented in 2015 with recurrent iron-deficiency anaemia requiring multiple blood transfusions. Oesophago-gastro-duodenoscopy revealed features of "watermelon stomach," consistent with gastric antral vascular ectasia (GAVE). She underwent multiple sessions of argon plasma coagulation to control bleeding. Seven to eight years later, she developed classical features of systemic sclerosis, including sclerodactyly, worsening Raynaud's phenomenon, skin calcifications, and digital ulcerations. Autoimmune testing was positive for antinuclear antibodies, anti-Ro, and anti-centromere antibodies, confirming limited cutaneous systemic sclerosis. This case demonstrates the potential for GAVE to appear before other features of systemic sclerosis become clinically evident. Patients presenting with idiopathic or recurrent GAVE should be evaluated for underlying connective tissue disease, as timely recognition can influence monitoring, treatment, and long-term outcomes. CI - Copyright © 2025, Khan et al. FAU - Khan, Nudrat AU - Khan N AD - Acute Medicine, North West Anglia NHS Foundation Trust, Peterborough City Hospital, Peterborough, GBR. FAU - Ather, Arslan AU - Ather A AD - Rheumatology, North West Anglia NHS Foundation Trust, Peterborough City Hospital, Peterborough, GBR. FAU - Pradeep, John AU - Pradeep J AD - Rheumatology, North West Anglia NHS Foundation Trust, Peterborough City Hospital, Peterborough, GBR. LA - eng PT - Case Reports PT - Journal Article DEP - 20251103 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12674828 OTO - NOTNLM OT - argon plasma coagulation (apc) OT - gastric antral vascular ectasia OT - iron-deficiency anaemia OT - oesophago-gastro-duodenoscopy (ogd) OT - raynaud’s phenomenon OT - systemic sclerosis COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/12/05 06:27 MHDA- 2025/12/05 06:28 PMCR- 2025/11/03 CRDT- 2025/12/05 04:31 PHST- 2025/11/03 00:00 [accepted] PHST- 2025/12/05 06:28 [medline] PHST- 2025/12/05 06:27 [pubmed] PHST- 2025/12/05 04:31 [entrez] PHST- 2025/11/03 00:00 [pmc-release] AID - 10.7759/cureus.96021 [doi] PST - epublish SO - Cureus. 2025 Nov 3;17(11):e96021. doi: 10.7759/cureus.96021. eCollection 2025 Nov. PMID- 28641551 OWN - NLM STAT- MEDLINE DCOM- 20180925 LR - 20180925 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 14 IP - 1 DP - 2018 Apr 20 TI - Nailfold Capillaroscopy Within and Beyond the Scope of Connective Tissue Diseases. PG - 12-21 LID - 10.2174/1573397113666170615093600 [doi] AB - Nailfold capillaroscopy is a noninvasive instrumental method for morphological analysis of the nutritive capillaries in the nailfold area. In rheumatology, it is a method of choice among instrumental modalities for differential diagnosis between primary and secondary Raynaud's phenomenon (RP) in systemic rheumatic diseases. RP is a common diagnostic problem in rheumatology. Defining the proper diagnosis is a prerequisite for administration of the appropriate treatment. Thus, nailfold capillaroscopic examination is of crucial importance for the every-day practice of the rheumatologists and is currently gaining increasing attention. The most specific capillaroscopic changes are observed in Systemic Sclerosis (SSc). Due to the high prevalence of the capillaroscopic changes in this clinical entity and their early appearance, they could be used for early and very early diagnosis of the disease. More recently, "scleroderma" type capillaroscopic changes have been defined as diagnostic criterion in the new EULAR/ACR classification criteria for SSc together with the presence of scleroderma-related autoantibodies, RP, telangiectasia and other clinical signs. Capillaroscopic changes in other connective tissue diseases and in different rheumatic-like conditions like those in diabetes mellitus (e.g., diabetic stiff-hand syndrome) and paraneoplastic syndromes associated with microvascular pathology should be interpreted properly in order to obtain precise diagnosis in the shortest possible differential diagnostic process. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University, Plovdiv, Bulgaria. AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany. FAU - Muller-Ladner, Ulf AU - Muller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Connective Tissue Diseases/diagnostic imaging MH - Diabetes Complications/*diagnostic imaging MH - Humans MH - Microscopic Angioscopy/*methods MH - Paraneoplastic Syndromes/*diagnostic imaging MH - Scleroderma, Systemic/*diagnostic imaging OTO - NOTNLM OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - connective tissue disease OT - diabetic stiff-hand syndrome OT - rheumatic-like conditions OT - systemic sclerosis. EDAT- 2017/06/24 06:00 MHDA- 2018/09/27 06:00 CRDT- 2017/06/24 06:00 PHST- 2017/02/17 00:00 [received] PHST- 2017/02/17 00:00 [revised] PHST- 2017/05/16 00:00 [accepted] PHST- 2017/06/24 06:00 [pubmed] PHST- 2018/09/27 06:00 [medline] PHST- 2017/06/24 06:00 [entrez] AID - CRR-EPUB-84109 [pii] AID - 10.2174/1573397113666170615093600 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2018 Apr 20;14(1):12-21. doi: 10.2174/1573397113666170615093600. PMID- 33961845 OWN - NLM STAT- MEDLINE DCOM- 20211018 LR - 20211018 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 362 IP - 3 DP - 2021 Sep TI - Thymic Carcinoma With Multiple Paraneoplastic Disorders. PG - 324-330 LID - S0002-9629(21)00152-X [pii] LID - 10.1016/j.amjms.2021.04.012 [doi] AB - Thymic neoplasms are rare and may run an indolent course. Among them, thymic epithelial carcinoma is exceptional as it may be presented with extensive local invasion and distant metastases. There is a wide spectrum of autoimmune/paraneoplastic syndromes associated with thymic tumors including autoimmune diseases, some of which may precede the diagnosis of thymoma. This article describes a 37-year-old woman with metastatic malignant thymoma and a combination of manifestations from different organs. Vitiligo, Raynaud's phenomenon and anti-centromere antibodies were preceded while eosinophilia, interstitial lung disease, rash, thickening of the skin and asymptomatic cryoglobulinemia were diagnosed concomitantly with the neoplasm. We have reviewed the literature and found only twenty case reports with a cluster of three or more autoimmune/paraneoplastic syndromes in the same patient but none with this unique constellation of disorders. The diversity of thymoma's clinical presentation and laboratory/histological features may cause diagnostic dilemmas and therapeutic challenges. CI - Copyright © 2021. Published by Elsevier Inc. FAU - Alexakou, Zoe AU - Alexakou Z AD - 2(nd) Department of Internal Medicine, Medical School, National & Kapodistrian University of Athens, Hippokration General Hospital, Athens, Greece. Electronic address: zoeeee.al@gmail.com. FAU - Liatsos, George AU - Liatsos G AD - 2(nd) Department of Internal Medicine, Medical School, National & Kapodistrian University of Athens, Hippokration General Hospital, Athens, Greece. Electronic address: geoliatsos@yahoo.gr. FAU - Vasileiou, Nick AU - Vasileiou N AD - 2(nd) Department of Internal Medicine, Medical School, National & Kapodistrian University of Athens, Hippokration General Hospital, Athens, Greece. Electronic address: nickmorton01@hotmail.com. FAU - Vamvakaris, Ioannis AU - Vamvakaris I AD - Pathology Department, Athens Chest Hospital "Sotiria", Athens, Greece. Electronic address: i.vamvakaris@yahoo.gr. FAU - Mani, Iliana AU - Mani I AD - 2(nd) Department of Internal Medicine, Medical School, National & Kapodistrian University of Athens, Hippokration General Hospital, Athens, Greece. Electronic address: ilianamani@windowslive.com. FAU - Alexopoulou, Alexandra AU - Alexopoulou A AD - 2(nd) Department of Internal Medicine, Medical School, National & Kapodistrian University of Athens, Hippokration General Hospital, Athens, Greece. Electronic address: alexopou@ath.forthnet.gr. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210504 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage MH - Female MH - Humans MH - Paraneoplastic Syndromes/complications/*diagnostic imaging/drug therapy MH - Thymoma/complications/*diagnostic imaging/drug therapy MH - Thymus Neoplasms/complications/*diagnostic imaging/drug therapy OTO - NOTNLM OT - Anticentromere antibodies OT - Eosinophilia OT - Interstitial lung disease OT - Raynaud's phenomenon OT - Thymic carcinoma OT - Vitiligo COIS- Declaration of Competing Interest Authors have no conflict of interest. EDAT- 2021/05/08 06:00 MHDA- 2021/10/21 06:00 CRDT- 2021/05/07 20:19 PHST- 2020/07/03 00:00 [received] PHST- 2021/02/20 00:00 [revised] PHST- 2021/04/25 00:00 [accepted] PHST- 2021/05/08 06:00 [pubmed] PHST- 2021/10/21 06:00 [medline] PHST- 2021/05/07 20:19 [entrez] AID - S0002-9629(21)00152-X [pii] AID - 10.1016/j.amjms.2021.04.012 [doi] PST - ppublish SO - Am J Med Sci. 2021 Sep;362(3):324-330. doi: 10.1016/j.amjms.2021.04.012. Epub 2021 May 4. PMID- 31077005 OWN - NLM STAT- MEDLINE DCOM- 20200511 LR - 20210524 IS - 0973-7693 (Electronic) IS - 0019-5456 (Linking) VI - 86 IP - 9 DP - 2019 Sep TI - Fatal Myocarditis in an Adolescent Girl with Evolving Connective Tissue Disease. PG - 857-859 LID - 10.1007/s12098-019-02974-9 [doi] AB - Connective tissue diseases are rarely suspected and diagnosed in childhood and adolescence. Rarity of occurrence and poor disease acceptance among parents make them extremely difficult to treat in the early stages. An adolescent girl presented with features of pneumonia, was worked up and diagnosed as an evolving connective tissue disease. Her clinical characteristics did not fit into any specific disease. She was started on steroids and immunoglobulin as she had fulminant myocarditis with rapid downhill clinical course. High index of suspicion and aggressive immunosuppression can be life saving in exceptional situations even though a specific diagnosis cannot be ascertained. FAU - Jose, Bipin AU - Jose B AD - Department of Pediatrics, Rajagiri Hospital, Aluva, Kerala, 683112, India. bipin015@yahoo.co.in. FAU - Emmanuel, Dantis AU - Emmanuel D AD - Department of Clinical Immunology and Rheumatology, Rajagiri Hospital, Aluva, Kerala, India. FAU - Harrison, Preethy AU - Harrison P AD - Department of Dermatology, Rajagiri Hospital, Aluva, Kerala, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20190510 PL - India TA - Indian J Pediatr JT - Indian journal of pediatrics JID - 0417442 RN - 0 (Immunoglobulins) RN - 0 (Steroids) SB - IM CIN - Indian J Pediatr. 2021 Feb;88(2):202-203. doi: 10.1007/s12098-020-03421-w. PMID: 32651865 MH - Adolescent MH - Connective Tissue Diseases/*complications/*diagnosis/drug therapy/physiopathology MH - Cyanosis/diagnostic imaging MH - Female MH - Humans MH - Immunoglobulins/therapeutic use MH - Myocarditis/*complications/*diagnosis/drug therapy/physiopathology MH - Steroids/therapeutic use OTO - NOTNLM OT - *Children OT - *Connective tissue disease OT - *Gottron’s papules OT - *Myocarditis OT - *Raynaud’s EDAT- 2019/05/12 06:00 MHDA- 2020/05/12 06:00 CRDT- 2019/05/12 06:00 PHST- 2019/01/06 00:00 [received] PHST- 2019/04/25 00:00 [accepted] PHST- 2019/05/12 06:00 [pubmed] PHST- 2020/05/12 06:00 [medline] PHST- 2019/05/12 06:00 [entrez] AID - 10.1007/s12098-019-02974-9 [pii] AID - 10.1007/s12098-019-02974-9 [doi] PST - ppublish SO - Indian J Pediatr. 2019 Sep;86(9):857-859. doi: 10.1007/s12098-019-02974-9. Epub 2019 May 10. PMID- 19711597 OWN - NLM STAT- MEDLINE DCOM- 20091022 LR - 20090828 IS - 0012-7183 (Print) IS - 0012-7183 (Linking) VI - 125 IP - 12 DP - 2009 TI - [Shoemaker's vibration disease]. PG - 1289-93 AB - Exposure to vibration at work with associated health hazards is rather common. Severe symptoms can significantly impair working and functional capacity, whereby occupational health services should invest in the primary and secondary prevention of vibration-induced handicaps. Patients with work-related numbness of the fingers and Raynaud's syndrome should be referred to occupational health service. We describe a shoe worker diagnosed with occupational disease caused by vibration. His profession and way of exposure are unusual, but the symptoms are typical for vibration disease. FAU - Sauni, Riitta AU - Sauni R AD - Työterveyslaitos, Tampere ja TAYS, työlääketieteen poliklinikka, PL 486, 33101, Tampere. FAU - Humalajoki, Riitta AU - Humalajoki R FAU - Pääkkönen, Rauno AU - Pääkkönen R FAU - Peltoniemi, Niina AU - Peltoniemi N FAU - Lindholm, Tuula AU - Lindholm T FAU - Uitti, Jukka AU - Uitti J LA - fin PT - English Abstract PT - Journal Article TT - Kengäntekijän tärinätauti. PL - Finland TA - Duodecim JT - Duodecim; laaketieteellinen aikakauskirja JID - 0373207 SB - IM MH - Humans MH - Male MH - Occupational Diseases/*etiology MH - Shoes MH - Vibration/*adverse effects EDAT- 2009/08/29 09:00 MHDA- 2009/10/23 06:00 CRDT- 2009/08/29 09:00 PHST- 2009/08/29 09:00 [entrez] PHST- 2009/08/29 09:00 [pubmed] PHST- 2009/10/23 06:00 [medline] PST - ppublish SO - Duodecim. 2009;125(12):1289-93. PMID- 27574560 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160830 LR - 20200929 IS - 2053-8855 (Print) IS - 2053-8855 (Electronic) IS - 2053-8855 (Linking) VI - 2016 IP - 8 DP - 2016 Aug TI - Undifferentiated vasculitis or an evolving systemic autoimmune rheumatic disease? PG - omw066 LID - 10.1093/omcr/omw066 [doi] LID - omw066 AB - Undifferentiated connective tissue diseases usually present with arthralgias, sicca symptoms, Raynaud's phenomenon and leucopenia. This case presents the atypical presentation of an undifferentiated connective tissue disease with extensive cutaneous involvement of fingers and toes leading to gangrene with absence of typical rheumatological symptoms. The autoimmune profile showed positive ANA and anti-Ro/SS-A. Thromboembolism was ruled out on the basis of transthoracic and transesophageal echo. She was treated with I/V corticosteroids and cyclophosphamide that halted the disease progression. FAU - Fatimah, Nafeesah AU - Fatimah N AD - Department of Internal Medicine, Shaikh Zayed Medical Complex , 54600 Lahore , Pakistan. FAU - Ussaid, Ahmad AU - Ussaid A AD - Department of Internal Medicine, Shaikh Zayed Medical Complex , 54600 Lahore , Pakistan. FAU - Rasheed, Aflak AU - Rasheed A AD - Department of Rheumatology and Immunology, Shaikh Zayed Medical Complex , 54600 Lahore , Pakistan. LA - eng PT - Case Reports PT - Journal Article DEP - 20160825 PL - England TA - Oxf Med Case Reports JT - Oxford medical case reports JID - 101642070 PMC - PMC5002063 OTO - NOTNLM OT - Autoimmunity OT - Undifferentiated connective tissue disease OT - Vasculitis EDAT- 2016/08/31 06:00 MHDA- 2016/08/31 06:01 PMCR- 2016/08/25 CRDT- 2016/08/31 06:00 PHST- 2016/02/23 00:00 [received] PHST- 2016/05/17 00:00 [revised] PHST- 2016/06/23 00:00 [accepted] PHST- 2016/08/31 06:00 [entrez] PHST- 2016/08/31 06:00 [pubmed] PHST- 2016/08/31 06:01 [medline] PHST- 2016/08/25 00:00 [pmc-release] AID - omw066 [pii] AID - 10.1093/omcr/omw066 [doi] PST - epublish SO - Oxf Med Case Reports. 2016 Aug 25;2016(8):omw066. doi: 10.1093/omcr/omw066. eCollection 2016 Aug. PMID- 41984810 OWN - NLM STAT- In-Process LR - 20260508 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 65 IP - 5 DP - 2026 May 5 TI - Vaping and vascular health: emerging risks for Raynaud's, chilblains and Buerger's disease. LID - keag182 [pii] LID - 10.1093/rheumatology/keag182 [doi] FAU - Amin, Meshva AU - Amin M AUID- ORCID: 0000-0003-1266-1837 AD - Department of Rheumatology, Oxford University Hospitals NHS FT, Oxford, UK. FAU - Dubey, Shirish AU - Dubey S AUID- ORCID: 0000-0002-9979-6066 AD - Department of Rheumatology, Oxford University Hospitals NHS FT, Oxford, UK. AD - Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK. FAU - Clark, Kristina AU - Clark K AUID- ORCID: 0000-0002-5926-3900 AD - Department of Rheumatology, Oxford University Hospitals NHS FT, Oxford, UK. AD - Kennedy Institute of Rheumatology, University of Oxford, Oxford, UK. FAU - Ong, Voon AU - Ong V AUID- ORCID: 0000-0001-5474-0053 AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - Joshi, Nilay AU - Joshi N AUID- ORCID: 0000-0003-2214-2360 AD - Department of Rheumatology, Kettering General Hospital NHS FT, Kettering, UK. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM EDAT- 2026/04/15 18:32 MHDA- 2026/04/15 18:32 CRDT- 2026/04/15 13:35 PHST- 2026/03/27 00:00 [accepted] PHST- 2026/04/15 18:32 [pubmed] PHST- 2026/04/15 18:32 [medline] PHST- 2026/04/15 13:35 [entrez] AID - 8654539 [pii] AID - 10.1093/rheumatology/keag182 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 May 5;65(5):keag182. doi: 10.1093/rheumatology/keag182. PMID- 41746197 OWN - NLM STAT- In-Process LR - 20260226 IS - 1680-0745 (Electronic) IS - 1015-9657 (Linking) VI - 36 IP - 3 DP - 2025 Sep 3 TI - Evaluation of abnormal complete blood count values in primary Raynaud's Phenomenon. PG - 225-230 LID - 10.5830/CVJA-2025-029 [doi] AB - BACKGROUND: The primary objective of this study was to evaluate changes in complete blood count (CBC) values in patients diagnosed with primary Raynaud's phenomenon (PRP). Most diagnostic tests for Raynaud's phenomenon (RP) aim to identify underlying diseases. When no such disease is detected, the condition is termed primary Raynaud's phenomenon (PRP). METHODS: Data were collected from a single-centre database, consisting of 2215 patients evaluated between 2010 and 2019. This study included 471 patients diagnosed with PRP and 339 healthy controls. A total of 1069 patients diagnosed with RP at our hospital were initially considered. Exclusion criteria were applied, resulting in the selection of 471 PRP patients for the study group. During the same period, 1146 healthy individuals with similar demographic characteristics were also evaluated, and 339 were included as the control group after applying exclusion criteria. CBC data of both groups were compared. The study retrospectively analysed patients diagnosed with PRP. Statistical analyses were performed using the SPSS software. RESULTS: Significant differences were observed between the PRP and control groups in platelet distribution width (PDW), red cell distribution width (RDW), platelet count (PLT), and mean platelet volume (MPV) (p < 0.00001). PRP patients exhibited increased PDW, RDW, and PLT values, and decreased MPV values. Regression analysis identified PDW (CI = 0.958-0.988) as the best predictive parameter for PRP, followed by RDW (CI = 0.769-0.835). Other parameters showed lower predictive power. CONCLUSION: The pathophysiology of PRP remains unclear. However, the observed changes in CBC values may enhance understanding of PRP mechanisms. These haematological parameters could potentially serve as biomarkers for PRP diagnosis and prognosis. FAU - Vezir, Ozden AU - Vezir O AD - Mersin City Training and Research Hospital, Department of Cardiovascular Surgery, Mersin / Turkey. FAU - Tekin, Esra Ertürk AU - Tekin EE AD - Mersin City Training and Research Hospital, Department of Cardiovascular Surgery, Mersin / Turkey. Email: dresraer@yahoo.com. LA - eng PT - Journal Article DEP - 20250815 PL - South Africa TA - Cardiovasc J Afr JT - Cardiovascular journal of Africa JID - 101313864 SB - IM OTO - NOTNLM OT - Blood Count OT - Haematological Parameters OT - Platelet Distribution Width OT - Primary Raynaud's Phenomenon OT - Red Cell Distribution Width EDAT- 2026/02/26 13:40 MHDA- 2026/02/26 13:40 CRDT- 2026/02/26 10:12 PHST- 2024/07/27 00:00 [received] PHST- 2025/10/25 00:00 [accepted] PHST- 2026/02/26 13:40 [medline] PHST- 2026/02/26 13:40 [pubmed] PHST- 2026/02/26 10:12 [entrez] AID - 10.5830/CVJA-2025-029 [doi] PST - ppublish SO - Cardiovasc J Afr. 2025 Sep 3;36(3):225-230. doi: 10.5830/CVJA-2025-029. Epub 2025 Aug 15. PMID- 32185286 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 28 IP - 4 DP - 2017 Dec TI - Anti-synthetase syndrome presenting with interstitial lung disease. PG - 212-216 LID - 10.31138/mjr.28.4.212 [doi] AB - Anti-synthetase syndrome is an idiopathic inflammatory myopathy characterized by interstitial lung involvement, arthritis, Raynaud's phenomenon, mechanic's hands and fever. The case of anti-synthetase syndrome in a young female is reported. The patient presented with interstitial lung disease, initially subclinical muscle involvement, Raynaud's phenomenon, arthritis and mechanic's hands. Induction therapy was administrated, that consisted of intravenous methylprednisolone and cyclophosphamide, followed by azathioprine and prednisone with good response, and finally, complete remission. The presence of mechanic's hands in a patient with lung involvement should prompt for specific investigations such as anti-synthetase antibodies and muscle enzymes. Anti-synthetase syndrome should be aggressively managed by immunosuppressive therapy, as interstitial lung disease is a severe manifestation of the disease which may affect quality of life and life expectancy. CI - © 2017 The Mediterranean Journal of Rheumatology (MJR). FAU - Athanassiou, Panagiotis AU - Athanassiou P AD - Department of Rheumatology, St. Paul's Hospital, Thessaloniki, Greece. FAU - Kostopoulos, Markos AU - Kostopoulos M AD - Department of Rheumatology, St. Paul's Hospital, Thessaloniki, Greece. FAU - Tzanavari, Aikaterini AU - Tzanavari A AD - Department of Rheumatology, St. Paul's Hospital, Thessaloniki, Greece. FAU - Spyridis, Anestis AU - Spyridis A AD - Department of Rheumatology, St. Paul's Hospital, Thessaloniki, Greece. FAU - Kostoglou-Athanassiou, Ifigenia AU - Kostoglou-Athanassiou I AD - Department of Endocrinology, Asclepeion Hospital, Athens, Greece. LA - eng PT - Case Reports PT - Journal Article DEP - 20171222 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7046003 OTO - NOTNLM OT - anti-synthetase syndrome OT - azathioprine OT - interstitial lung disease OT - mechanic’s hands OT - myopathy EDAT- 2017/12/22 00:00 MHDA- 2017/12/22 00:01 PMCR- 2017/12/22 CRDT- 2020/03/19 06:00 PHST- 2017/09/12 00:00 [received] PHST- 2017/11/04 00:00 [revised] PHST- 2017/11/13 00:00 [accepted] PHST- 2020/03/19 06:00 [entrez] PHST- 2017/12/22 00:00 [pubmed] PHST- 2017/12/22 00:01 [medline] PHST- 2017/12/22 00:00 [pmc-release] AID - MJR-28-4-212 [pii] AID - 10.31138/mjr.28.4.212 [doi] PST - epublish SO - Mediterr J Rheumatol. 2017 Dec 22;28(4):212-216. doi: 10.31138/mjr.28.4.212. eCollection 2017 Dec. PMID- 40212482 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250412 IS - 2250-0685 (Print) IS - 2321-3817 (Electronic) IS - 2250-0685 (Linking) VI - 15 IP - 4 DP - 2025 Apr TI - Systemic Sclerosis Presenting as Osteomyelitis of the Finger: Physicians Must Maintain a High Index of Suspicion for Systemic Sclerosis when Evaluating Patients with Fingertip Ulceration or Infection. PG - 52-55 LID - 10.13107/jocr.2025.v15.i04.5442 [doi] AB - INTRODUCTION: Osteomyelitis is a serious bone infection commonly caused by bacterial pathogens, with Staphylococcus aureus being the most prevalent. The condition poses significant challenges in patients with underlying autoimmune disorders such as scleroderma and Raynaud's syndrome, where vascular dysfunction and immunosuppression heighten infection risks. This case report illustrates the complex interplay between these conditions and underscores the importance of early diagnosis and comprehensive management to prevent severe complications. CASE REPORT: We present the case of a 79-year-old female with a history of Raynaud's syndrome and suspected scleroderma who developed osteomyelitis of the left middle finger following a paronychial infection. The patient experienced persistent pain and swelling despite multiple debridement procedures. Physical examination revealed necrosis and gangrene of the affected finger, and magnetic resonance imaging confirmed osteomyelitis. Cultures identified Corynebacterium accolens, an uncommon pathogen in this context. The patient underwent surgical debridement, followed by antibiotic therapy and vasodilators. Her condition improved, with no signs of infection at follow-up. CONCLUSION: This case highlights the critical need for early and thorough assessment of infections in patients with autoimmune conditions such as scleroderma and Raynaud's syndrome. The vascular dysfunction inherent in these diseases can exacerbate infections, leading to severe outcomes such as osteomyelitis. A multidisciplinary approach involving early surgical intervention and tailored medical management is essential to optimize patient outcomes. CI - Copyright: © Indian Orthopaedic Research Group. FAU - Poucke, Logan Van AU - Poucke LV AD - Department of Medicine, University of Illinois College of Medicine, Chicago, Illinois, USA. FAU - Hinton, Jared B AU - Hinton JB AD - Department of Medicine, Northeast Ohio Medical University, Rootstown, Ohio, USA. FAU - Heck, Holly C AU - Heck HC AD - Department of Medicine, University of Toledo College of Medicine and Life Sciences. Toledo, Ohio, USA. FAU - Heck, Benjamin E AU - Heck BE AD - Department of Medicine, University of Toledo College of Medicine and Life Sciences. Toledo, Ohio, USA. FAU - Heck, Bruce E AU - Heck BE AD - Northwest Ohio Orthopedics & Sports Medicine. Department of Orthopedic Surgery, University of Toledo Medical Center, Toledo, Ohio, USA. LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Orthop Case Rep JT - Journal of orthopaedic case reports JID - 101641392 PMC - PMC11981491 OTO - NOTNLM OT - Osteomyelitis OT - Raynaud’s syndrome OT - autoimmune disease OT - scleroderma OT - vascular dysfunction COIS- Conflict of Interest: Nil EDAT- 2025/04/11 06:25 MHDA- 2025/04/11 06:26 PMCR- 2025/04/01 CRDT- 2025/04/11 05:38 PHST- 2025/01/15 00:00 [received] PHST- 2025/02/28 00:00 [revised] PHST- 2025/04/11 06:26 [medline] PHST- 2025/04/11 06:25 [pubmed] PHST- 2025/04/11 05:38 [entrez] PHST- 2025/04/01 00:00 [pmc-release] AID - JOCR-15-52 [pii] AID - 10.13107/jocr.2025.v15.i04.5442 [doi] PST - ppublish SO - J Orthop Case Rep. 2025 Apr;15(4):52-55. doi: 10.13107/jocr.2025.v15.i04.5442. PMID- 32959188 OWN - NLM STAT- MEDLINE DCOM- 20210518 LR - 20210518 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 40 IP - 6 DP - 2021 Jun TI - Anti-phospholipid syndrome leading to digital ischaemia and rare organ complications in systemic sclerosis and related disorders. PG - 2457-2465 LID - 10.1007/s10067-020-05399-4 [doi] AB - Antiphospholipid syndrome (APS) is an acquired, autoimmune thrombophilia that can occur as a primary disorder (with no associated disease) or secondary to infection, medication usage and autoimmune rheumatic diseases (ARDs). The association between APS and systemic lupus erythematosus (SLE) is well established, and practicing rheumatologists check for APS antibodies in the routine assessment of SLE, particularly if clinical features such as thrombotic events or pregnancy loss are present. APS secondary to systemic sclerosis (SSc)-related disorders is less widely recognised and easily overlooked. We describe 5 cases that highlight the varied breadth of clinical manifestations of APS in the context of SSc and related disorders. These cases range from uncomplicated Raynaud's phenomenon, digital ulceration/necrosis, critical digital ischaemia/gangrene and rare internal organ complications of APS in SSc-spectrum disorders. To our knowledge, our cases include the first reported case of secondary APS contributing to digital necrosis in the context of RACAND syndrome (Raynaud's phenomenon, anti-centromere antibodies and necrosis of the digits) and the first reported case of secondary APS in SSc causing posterior reversible encephalopathy syndrome (PRES). The case series is accompanied by a comprehensive review of the literature relevant to each case. Rheumatologists should be alert to the possibility of APS in SSc-spectrum disorders and should routinely check APS antibodies in all patients at diagnosis, and again later in the disease course if new features emerge that could indicate the presence of thrombotic events or other recognised APS manifestations. Key points • APS should be considered in all patients with digital ischaemic symptoms. • APS may be an important driver of SSc-related digital ulceration/necrosis. • Identification of SSc-associated APS opens up new therapeutic options for acute management and secondary prevention. FAU - Chatterjee, Saion AU - Chatterjee S AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK. jdp32@bath.ac.uk. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. jdp32@bath.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20200921 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Antibodies, Antiphospholipid MH - *Antiphospholipid Syndrome/complications/diagnosis MH - Female MH - Humans MH - Ischemia/etiology MH - *Lupus Erythematosus, Systemic/complications MH - *Posterior Leukoencephalopathy Syndrome MH - Pregnancy MH - *Scleroderma, Systemic/complications OTO - NOTNLM OT - Anti-phospholipid syndrome OT - Clinical phenotype OT - Critical digital ischaemia OT - Digital necrosis OT - Digital ulceration OT - Overlap syndromes OT - Posterior reversible encephalopathy syndrome OT - Raynaud’s phenomenon OT - Systemic sclerosis EDAT- 2020/09/23 06:00 MHDA- 2021/05/19 06:00 CRDT- 2020/09/22 05:44 PHST- 2020/07/15 00:00 [received] PHST- 2020/09/12 00:00 [accepted] PHST- 2020/09/08 00:00 [revised] PHST- 2020/09/23 06:00 [pubmed] PHST- 2021/05/19 06:00 [medline] PHST- 2020/09/22 05:44 [entrez] AID - 10.1007/s10067-020-05399-4 [pii] AID - 10.1007/s10067-020-05399-4 [doi] PST - ppublish SO - Clin Rheumatol. 2021 Jun;40(6):2457-2465. doi: 10.1007/s10067-020-05399-4. Epub 2020 Sep 21. PMID- 35966055 OWN - NLM STAT- MEDLINE DCOM- 20220816 LR - 20250728 IS - 1664-2392 (Print) IS - 1664-2392 (Electronic) IS - 1664-2392 (Linking) VI - 13 DP - 2022 TI - Case Report: Artifactual Hypoglycemia: A Condition That Should Not Be Forgotten. PG - 951377 LID - 10.3389/fendo.2022.951377 [doi] LID - 951377 AB - BACKGROUND: Hypoglycemia is uncommon in people who are not being treated for diabetes mellitus and, when present, the differential diagnosis is broad. Artifactual hypoglycemia describes discrepancy between low capillary and normal plasma glucose levels regardless of symptoms and should be considered in patients with Raynaud's phenomenon. CASE PRESENTATION: A 46-year-old female patient with a history of a sleeve gastrectomy started complaining about episodes of lipothymias preceded by sweating, nausea, and dizziness. During one of these episodes, a capillary blood glucose was obtained with a value of 24 mg/dl. She had multiple emergency admissions with low-capillary glycemia. An exhaustive investigation for possible causes of hypoglycemia was made for 18 months. The 72h fasting test was negative for hypoglycemia. A Raynaud's phenomenon was identified during one appointment. CONCLUSION: Artifactual hypoglycemia has been described in various conditions including Raynaud's phenomenon, peripheral arterial disease, Eisenmenger syndrome, acrocyanosis, or hypothermia. With this case report, we want to reinforce the importance of being aware of this diagnosis to prevent anxiety, unnecessary treatment, and diagnostic tests. CI - Copyright © 2022 Amaral, Palha, Bernardino and Silva-Nunes. FAU - Amaral, Sara AU - Amaral S AD - Department of Endocrinology, Diabetes and Metabolism; Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal. AD - Nova Medical School/Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal. FAU - Palha, Ana AU - Palha A AD - Department of Endocrinology, Diabetes and Metabolism; Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal. AD - Nova Medical School/Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal. FAU - Bernardino, Vera AU - Bernardino V AD - Functional Unit of Internal Medicine 7.2, Centro Hospitalar Universitário Lisboa Central, , Lisbon, Portugal. FAU - Silva-Nunes, José AU - Silva-Nunes J AD - Department of Endocrinology, Diabetes and Metabolism; Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal. AD - Nova Medical School/Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal. AD - Health and Technology Research Center (H&TRC), Escola Superior de Tecnologia da Saude de Lisboa, Lisbon, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20220728 PL - Switzerland TA - Front Endocrinol (Lausanne) JT - Frontiers in endocrinology JID - 101555782 RN - 0 (Blood Glucose) SB - IM MH - Awareness MH - Blood Glucose MH - *Diabetes Mellitus MH - Female MH - Humans MH - *Hypoglycemia/diagnosis/etiology MH - Memory Disorders MH - Middle Aged PMC - PMC9371975 OTO - NOTNLM OT - artifactual hypoglycemia OT - autoimmune diseases OT - capillary blood glucose OT - hypoglycemia OT - raynaud’s phenomenon COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/08/16 06:00 MHDA- 2022/08/17 06:00 PMCR- 2022/01/01 CRDT- 2022/08/15 03:34 PHST- 2022/05/23 00:00 [received] PHST- 2022/06/24 00:00 [accepted] PHST- 2022/08/15 03:34 [entrez] PHST- 2022/08/16 06:00 [pubmed] PHST- 2022/08/17 06:00 [medline] PHST- 2022/01/01 00:00 [pmc-release] AID - 10.3389/fendo.2022.951377 [doi] PST - epublish SO - Front Endocrinol (Lausanne). 2022 Jul 28;13:951377. doi: 10.3389/fendo.2022.951377. eCollection 2022. PMID- 39534828 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241114 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 10 DP - 2024 Oct TI - Improvement of Post-sympathectomy Raynaud's Syndrome With Spinal Cord Stimulation. PG - e71340 LID - 10.7759/cureus.71340 [doi] LID - e71340 AB - Raynaud's phenomenon is a vascular disorder, characterized by vasospasm-induced discoloration, numbness, and pain in the extremities. While pharmacological treatments and sympathectomy are commonly employed, many patients experience symptom recurrence, and effective therapies for refractory cases remain limited. This case study presents a 60-year-old male with severe Raynaud's symptoms, including fingertip necrosis, unresponsive to pharmacotherapy and endoscopic thoracic sympathectomy. Despite initial symptom relief, the patient's condition worsened, leading to finger necrosis. Spinal cord stimulation (SCS) was introduced as an alternative treatment, significantly reducing the patient's pain and improving blood flow to the affected areas. The mechanisms of SCS remain largely speculative, but it is believed to modulate the sympathetic nervous system, promoting vasodilation by releasing neuropeptides. This case highlights the potential of SCS as a therapeutic option for managing severe and recurrent Raynaud's phenomenon, especially in patients unresponsive to conventional treatments. CI - Copyright © 2024, Pan et al. FAU - Pan, Kai AU - Pan K AD - Neurosurgery, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Zhejiang Hangzhou, CHN. AD - Neurosurgery, Clinical Research Center of Neurological Disease of Zhejiang Province, Zhejiang Hangzhou, CHN. FAU - Jiang, Hongjie AU - Jiang H AD - Neurosurgery, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou Zhejiang, CHN. AD - Neurosurgery, Clinical Research Center of Neurological Disease of Zhejiang Province, Hangzhou Zhejiang, CHN. FAU - Wu, Hemmings AU - Wu H AD - Neurosurgery, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou Zhejiang, CHN. AD - Neurosurgery, Clinical Research Center of Neurological Disease of Zhejiang Province, Hangzhou Zhejiang, CHN. FAU - Zhu, Junming AU - Zhu J AD - Neurosurgery, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou Zhejiang, CHN. AD - Neurosurgery, Clinical Research Center of Neurological Disease of Zhejiang Province, Hangzhou Zhejiang, CHN. FAU - Zhang, Jianmin AU - Zhang J AD - Neurosurgery, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou Zhejiang, CHN. AD - Neurosurgery, Clinical Research Center of Neurological Disease of Zhejiang Province, Hangzhou Zhejiang, CHN. LA - eng PT - Case Reports PT - Journal Article DEP - 20241012 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11555124 OTO - NOTNLM OT - finger necrosis OT - raynaud's phenomenon OT - spinal cord stimulation (scs) OT - sympathectomy OT - vascular pain COIS- Human subjects: Consent was obtained or waived by all participants in this study. Human Research Ethics Committee, The Second Affiliated Hospital of Zhejiang University School of Medicine issued approval 2020/021. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/11/14 03:55 MHDA- 2024/11/14 03:56 PMCR- 2024/10/12 CRDT- 2024/11/13 04:28 PHST- 2024/10/12 00:00 [accepted] PHST- 2024/11/14 03:56 [medline] PHST- 2024/11/14 03:55 [pubmed] PHST- 2024/11/13 04:28 [entrez] PHST- 2024/10/12 00:00 [pmc-release] AID - 10.7759/cureus.71340 [doi] PST - epublish SO - Cureus. 2024 Oct 12;16(10):e71340. doi: 10.7759/cureus.71340. eCollection 2024 Oct. PMID- 36843820 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230228 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 1 DP - 2023 Jan TI - Tofacitinib Is an Effective Treatment for Refractory Scleromyositis Associated With Anti-PM/Scl. PG - e34125 LID - 10.7759/cureus.34125 [doi] LID - e34125 AB - Scleromyositis is a rare autoimmune disease characterized by overlapping scleroderma and myositis. This case report discusses the presentation and management of a 28-year-old male with scleromyositis presenting with myositis, arthritis, Raynaud's phenomenon, refractory calcinosis, interstitial lung disease, and myocarditis. This case highlights key points in the systematic approach to immunosuppressive treatment and proposes a novel therapeutic option. CI - Copyright © 2023, Álvarez Troncoso et al. FAU - Álvarez Troncoso, Jorge AU - Álvarez Troncoso J AD - Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario La Paz, Madrid, ESP. FAU - Nuño González, Almudena AU - Nuño González A AD - Dermatology Department, Hospital Universitario La Paz, Madrid, ESP. FAU - Martínez Robles, Elena AU - Martínez Robles E AD - Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario La Paz, Madrid, ESP. FAU - Sorriguieta Torre, Raquel AU - Sorriguieta Torre R AD - Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario La Paz, Madrid, ESP. FAU - Robles Marhuenda, Ángel AU - Robles Marhuenda Á AD - Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario La Paz, Madrid, ESP. LA - eng PT - Case Reports PT - Journal Article DEP - 20230124 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9947514 OTO - NOTNLM OT - calcinosis OT - connective tissue disease associated interstitial lung disease OT - myocarditis OT - myositis OT - scleroderma OT - scleromyositis OT - tofacitinib COIS- The authors have declared that no competing interests exist. EDAT- 2023/02/28 06:00 MHDA- 2023/02/28 06:01 PMCR- 2023/01/24 CRDT- 2023/02/27 03:55 PHST- 2023/01/23 00:00 [accepted] PHST- 2023/02/27 03:55 [entrez] PHST- 2023/02/28 06:00 [pubmed] PHST- 2023/02/28 06:01 [medline] PHST- 2023/01/24 00:00 [pmc-release] AID - 10.7759/cureus.34125 [doi] PST - epublish SO - Cureus. 2023 Jan 24;15(1):e34125. doi: 10.7759/cureus.34125. eCollection 2023 Jan. PMID- 36179074 OWN - NLM STAT- MEDLINE DCOM- 20230103 LR - 20231003 IS - 1471-8405 (Electronic) IS - 0962-7480 (Linking) VI - 72 IP - 9 DP - 2022 Dec 31 TI - Cryoglobulins and cold agglutinins for hand arm vibration syndrome. PG - 609-613 LID - 10.1093/occmed/kqac083 [doi] AB - BACKGROUND: Hand arm vibration syndrome (HAVS) is a condition caused by hand transmitted vibration from the use of hand-held vibrating tools or workpieces. The disease affects the vascular, neurological and musculoskeletal systems. The vascular component of HAVS is a form of secondary Raynaud's phenomenon. Other causes of disease must be excluded before attributing the cause to hand transmitted vibration. AIMS: To evaluate the prevalence, and utility of testing for, cryoglobulins and cold agglutinins in patients with HAVS symptoms. METHODS: A retrospective cohort study of 1183 patients referred for HAVS clinical assessment at St. Michael's Hospital, Toronto, Canada, between 2014 and 2020. The standard operating procedure at the clinic includes a detailed clinical and exposure history, physical examination, objective investigations and blood tests. Data were retrieved from patient chart review and laboratory investigation results for all cases with cryoglobulin and cold agglutinin testing. RESULTS: A total of 1183 patients had a serum cryoglobulin measurement. Eleven patients (1%) were positive. Seven positive results were 'low titre' (1% positive) and the other four results were 2%, 6%, 9% and 18%. The patient with a 9% positive cryoglobulin titre had previously diagnosed Sjögren's syndrome. There were no positive cold agglutinin tests in the 795 patients tested. CONCLUSIONS: Routine testing for cryoglobulins and cold agglutinins in patients with HAVS symptoms is not recommended because test positivity rates are negligible. Testing may be considered if the clinical history or routine blood investigations suggest evidence of underlying cryoglobulinaemia or cold agglutinin disease. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Alsaidi, Y AU - Alsaidi Y AD - Division of Occupational Medicine, Department of Medicine, University of Toronto and St. Michael's Hospital, Toronto, Canada. FAU - Thompson, A AU - Thompson A AUID- ORCID: 0000-0002-4414-6330 AD - Division of Occupational Medicine, Department of Medicine, University of Toronto and St. Michael's Hospital, Toronto, Canada. FAU - Spilchuk, V AU - Spilchuk V AD - Division of Occupational Medicine, Department of Medicine, University of Toronto and St. Michael's Hospital, Toronto, Canada. FAU - House, R A AU - House RA AD - Division of Occupational Medicine, Department of Medicine, University of Toronto and St. Michael's Hospital, Toronto, Canada. FAU - Adisesh, A AU - Adisesh A AUID- ORCID: 0000-0002-4973-8474 AD - Division of Occupational Medicine, Department of Medicine, University of Toronto and St. Michael's Hospital, Toronto, Canada. LA - eng PT - Journal Article PL - England TA - Occup Med (Lond) JT - Occupational medicine (Oxford, England) JID - 9205857 RN - 0 (cold agglutinins) RN - 0 (Cryoglobulins) RN - 0 (Agglutinins) SB - IM MH - Humans MH - *Hand-Arm Vibration Syndrome/complications/diagnosis/epidemiology MH - Cryoglobulins MH - Retrospective Studies MH - Arm MH - Vibration MH - Agglutinins MH - Hand MH - Cold Temperature MH - *Occupational Diseases/epidemiology/etiology/diagnosis OTO - NOTNLM OT - Hand arm vibration syndrome OT - Raynaud’s phenomenon OT - cold agglutinin OT - cryoglobulin EDAT- 2022/10/01 06:00 MHDA- 2023/01/04 06:00 CRDT- 2022/09/30 14:42 PHST- 2022/10/01 06:00 [pubmed] PHST- 2023/01/04 06:00 [medline] PHST- 2022/09/30 14:42 [entrez] AID - 6731905 [pii] AID - 10.1093/occmed/kqac083 [doi] PST - ppublish SO - Occup Med (Lond). 2022 Dec 31;72(9):609-613. doi: 10.1093/occmed/kqac083. PMID- 37761318 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231003 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 13 IP - 18 DP - 2023 Sep 14 TI - Reversible Cerebral Vasoconstriction Syndrome and Raynaud's Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review. LID - 10.3390/diagnostics13182951 [doi] LID - 2951 AB - Reversible cerebral vasoconstriction syndrome (RCVS) typically manifests as a sudden, severe thunderclap headache due to narrowing of the cerebral arteries. Symptoms usually resolve within three months. An imbalance in cerebral vascular tone, an abnormal endothelial function, and a decreased autoregulation of cerebral blood flow are thought to be involved in the pathogenesis of RCVS. However, the precise origin of this condition is not yet fully understood. Symptoms of Raynaud's phenomenon (RP) include vasospasm of arterioles of the digits. The pathophysiology of RP includes interactions between the endothelium, smooth muscle, and autonomic and sensory neurons that innervate arteries to help maintain vasomotor homeostasis. RP may occur before the clinical manifestation of a rheumatic condition. RCVS is rare in patients with autoimmune rheumatic disease. We describe a 54-year-old female who had a history of Raynaud's phenomenon affecting her fingers and toes since the age of 12 years. The patient was diagnosed with RCVS in 2012. She described RCVS precipitants, including the regular use of cannabis, cocaine, and amphetamine and tobacco smoking. In 2021, she presented with oral ulcers, intermittent swallowing difficulties, and Raynaud's phenomenon. Clinical examination revealed early sclerodactyly, and abnormal nail-fold capillaroscopy showed multiple giant capillaries, dilated capillary loops, and areas of capillary hemorrhage with capillary drop-out. The investigation revealed positive ANA, strongly positive SRP antibodies, and Ro60 antibodies. Our case report indicates that there may be a correlation between RCVS and Raynaud's phenomenon, and a potential connection between RCVS and autoimmune rheumatic diseases. Hence, physicians must be aware of the red flags and subtle differences in neurological abnormalities, such as headaches, in patients with autoimmune rheumatic diseases who have an inactive clinical status to improve patient care and outcomes. FAU - Alenzi, Fahidah AU - Alenzi F AUID- ORCID: 0000-0001-8495-9064 AD - Clinical Sciences Department, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh 11564, Saudi Arabia. FAU - D'Cruz, David P AU - D'Cruz DP AD - Louise Coote Lupus Unit, Guy's Hospital, Guy's and St. Thomas' Hospitals, NHS Foundation Trust, London SE1 9RT, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20230914 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10528332 OTO - NOTNLM OT - Raynaud’s phenomenon OT - autoimmune rheumatic diseases OT - nail-fold capillaroscopy OT - reversible cerebral vasoconstriction syndrome OT - systemic lupus erythematosus COIS- The authors declare no conflict of interest. EDAT- 2023/09/28 06:42 MHDA- 2023/09/28 06:43 PMCR- 2023/09/14 CRDT- 2023/09/28 01:12 PHST- 2023/08/02 00:00 [received] PHST- 2023/09/10 00:00 [revised] PHST- 2023/09/13 00:00 [accepted] PHST- 2023/09/28 06:43 [medline] PHST- 2023/09/28 06:42 [pubmed] PHST- 2023/09/28 01:12 [entrez] PHST- 2023/09/14 00:00 [pmc-release] AID - diagnostics13182951 [pii] AID - diagnostics-13-02951 [pii] AID - 10.3390/diagnostics13182951 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Sep 14;13(18):2951. doi: 10.3390/diagnostics13182951. PMID- 29691079 OWN - NLM STAT- MEDLINE DCOM- 20190509 LR - 20190509 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 43 IP - 6 DP - 2018 Jun TI - The Scleroderma Hand: Manifestations of Disease and Approach to Management. PG - 550-557 LID - S0363-5023(17)31450-8 [pii] LID - 10.1016/j.jhsa.2018.03.021 [doi] AB - Scleroderma is a rare autoimmune connective tissue disorder that often affects the hands. Manifestations in the hands include calcium deposits within the soft tissues that cause pain and may ulcerate through the skin, digital ischemia resulting in chronic wounds and digital gangrene, and joint contracture. Because of the underlying disease, patients with scleroderma have poorly vascularized tissue and a deficient soft tissue envelope, which make surgery particularly challenging. However, when undertaken with care, surgical intervention is often the best option for addressing the disabling hand conditions that so often accompany this disease. CI - Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved. FAU - Williams, Ariel A AU - Williams AA AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins Medicine, Baltimore, MD. FAU - Carl, Hannah M AU - Carl HM AD - Johns Hopkins University School of Medicine, Baltimore, MD. FAU - Lifchez, Scott D AU - Lifchez SD AD - Department of Plastic and Reconstructive Surgery, Johns Hopkins Medicine, Baltimore, MD. Electronic address: slifche1@jhmi.edu. LA - eng PT - Journal Article PT - Review PT - Video-Audio Media PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 SB - IM MH - Calcinosis/diagnostic imaging/etiology/surgery/therapy MH - Contracture/complications/etiology/surgery MH - Gangrene/etiology MH - Hand/blood supply MH - Hand Deformities, Acquired/etiology/surgery MH - Hand Dermatoses/*etiology/*therapy MH - Humans MH - Ischemia/etiology/therapy MH - Scleroderma, Localized/*complications MH - Sympathectomy OTO - NOTNLM OT - Raynaud’s OT - Scleroderma OT - calcinosis OT - contracture OT - vasculopathy EDAT- 2018/04/25 06:00 MHDA- 2019/05/10 06:00 CRDT- 2018/04/26 06:00 PHST- 2017/08/16 00:00 [received] PHST- 2018/03/17 00:00 [accepted] PHST- 2018/04/25 06:00 [pubmed] PHST- 2019/05/10 06:00 [medline] PHST- 2018/04/26 06:00 [entrez] AID - S0363-5023(17)31450-8 [pii] AID - 10.1016/j.jhsa.2018.03.021 [doi] PST - ppublish SO - J Hand Surg Am. 2018 Jun;43(6):550-557. doi: 10.1016/j.jhsa.2018.03.021. PMID- 24108159 OWN - NLM STAT- MEDLINE DCOM- 20131211 LR - 20220330 IS - 1756-1833 (Electronic) IS - 0959-8138 (Print) IS - 0959-8138 (Linking) VI - 347 DP - 2013 Oct 9 TI - Autoimmune, neurological, and venous thromboembolic adverse events after immunisation of adolescent girls with quadrivalent human papillomavirus vaccine in Denmark and Sweden: cohort study. PG - f5906 LID - bmj.f5906 [pii] LID - 10.1136/bmj.f5906 [doi] LID - f5906 AB - OBJECTIVE: To assess the risk of serious adverse events after vaccination of adolescent girls with quadrivalent human papillomavirus (qHPV) vaccine. DESIGN: Register based cohort study. SETTING: Denmark and Sweden, October 2006 to December 2010. PARTICIPANTS: 997,585 girls aged 10-17, among whom 296,826 received a total of 696,420 qHPV vaccine doses. MAIN OUTCOME MEASURES: Incident hospital diagnosed autoimmune, neurological, and venous thromboembolic events (53 different outcomes) up to 180 days after each qHPV vaccine dose. Only events with at least five vaccine exposed cases were considered for further assessment. Rate ratios adjusted for age, country, calendar year, and parental country of birth, education, and socioeconomic status were estimated, comparing vaccinated and unvaccinated person time. For outcomes where the rate ratio was significantly increased, we regarded three criteria as signal strengthening: analysis based on 20 or more vaccine exposed cases (reliability), rate ratio 3.0 or more (strength), and significantly increased rate ratio in country specific analyses (consistency). We additionally assessed clustering of events in time and estimated rate ratios for a risk period that started on day 181. RESULTS: Among the 53 outcomes, at least five vaccine exposed cases occurred in 29 and these were analysed further. Whereas the rate ratios for 20 of 23 autoimmune events were not significantly increased, exposure to qHPV vaccine was significantly associated with Behcet's syndrome, Raynaud's disease, and type 1 diabetes. Each of these three outcomes fulfilled only one of three predefined signal strengthening criteria. Furthermore, the pattern of distribution in time after vaccination was random for all three and the rate ratios for these outcomes in the period from day 181 after vaccination were similar to the rate ratios in the primary risk period. The rate ratios for five neurological events were not significantly increased and there were inverse associations with epilepsy (rate ratio 0.66, 95% confidence interval 0.54 to 0.80) and paralysis (0.56, 0.35 to 0.90). There was no association between exposure to qHPV vaccine and venous thromboembolism (0.86, 0.55 to 1.36). CONCLUSIONS: This large cohort study found no evidence supporting associations between exposure to qHPV vaccine and autoimmune, neurological, and venous thromboembolic adverse events. Although associations for three autoimmune events were initially observed, on further assessment these were weak and not temporally related to vaccine exposure. Furthermore, the findings need to be interpreted considering the multiple outcomes assessed. FAU - Arnheim-Dahlström, Lisen AU - Arnheim-Dahlström L AD - Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm 171 77, Sweden. FAU - Pasternak, Björn AU - Pasternak B FAU - Svanström, Henrik AU - Svanström H FAU - Sparén, Pär AU - Sparén P FAU - Hviid, Anders AU - Hviid A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131009 PL - England TA - BMJ JT - BMJ (Clinical research ed.) JID - 8900488 RN - 0 (Papillomavirus Vaccines) SB - IM CIN - BMJ. 2013 Oct 09;347:f5631. doi: 10.1136/bmj.f5631. PMID: 24108153 CIN - J Pediatr. 2014 May;164(5):1240. doi: 10.1016/j.jpeds.2014.02.043. PMID: 24742654 MH - Adolescent MH - Autoimmune Diseases/*epidemiology/immunology MH - Child MH - Cohort Studies MH - Denmark/epidemiology MH - Female MH - Humans MH - Incidence MH - Nervous System Diseases/*epidemiology/immunology MH - Papillomavirus Infections/*prevention & control MH - Papillomavirus Vaccines/*adverse effects MH - Risk Factors MH - Sweden/epidemiology MH - Venous Thromboembolism/*epidemiology/immunology PMC - PMC3805482 COIS- Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: no support from any organisation for the submitted work; LAD and PS are and have been involved in other studies with unconditional grants from GlaxoSmithKline, Sanofi Pasteur MSD, and Merck; and no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2013/10/11 06:00 MHDA- 2013/12/16 06:00 PMCR- 2013/10/09 CRDT- 2013/10/11 06:00 PHST- 2013/10/11 06:00 [entrez] PHST- 2013/10/11 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] PHST- 2013/10/09 00:00 [pmc-release] AID - bmj.f5906 [pii] AID - arnl010885 [pii] AID - 10.1136/bmj.f5906 [doi] PST - epublish SO - BMJ. 2013 Oct 9;347:f5906. doi: 10.1136/bmj.f5906. PMID- 32344844 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240328 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 10 IP - 5 DP - 2020 Apr 25 TI - Nailfold Videocapillaroscopy is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients. LID - 10.3390/diagnostics10050253 [doi] LID - 253 AB - Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud's phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP. FAU - Sambataro, Domenico AU - Sambataro D AD - Artroreuma S.R.L., Outpatient of Rheumatology associated with the National Health System corso S. Vito 53, 95030 Mascalucia (Catania), Italy. AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Cannizzaro Hospital, University of Catania, via Messina 829, 95100 Catania, Italy. FAU - Sambataro, Gianluca AU - Sambataro G AD - Artroreuma S.R.L., Outpatient of Rheumatology associated with the National Health System corso S. Vito 53, 95030 Mascalucia (Catania), Italy. AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Libra, Alessandro AU - Libra A AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Vignigni, Giovanna AU - Vignigni G AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Pino, Fabio AU - Pino F AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Fagone, Evelina AU - Fagone E AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Fruciano, Mary AU - Fruciano M AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Gili, Elisa AU - Gili E AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. FAU - Pignataro, Francesca AU - Pignataro F AD - Scleroderma Clinic, Department of Rheumatology, ASST G. Pini-CTO, 20122 Milan, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AUID- ORCID: 0000-0003-1549-8852 AD - Scleroderma Clinic, Department of Rheumatology, ASST G. Pini-CTO, 20122 Milan, Italy. FAU - Vancheri, Carlo AU - Vancheri C AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. Of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy. LA - eng PT - Journal Article DEP - 20200425 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC7277171 OTO - NOTNLM OT - Raynaud’s phenomenon OT - antisynthetase syndrome OT - bushy capillary OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - interstitial pneumonia with autoimmune features OT - multidisciplinary team OT - nailfold videocapillaroscopy OT - polymyositis OT - systemic sclerosis COIS- C.V. is part of the F. Hoffmann-La Roche Ltd. Scientific board. He has received consulting fees and/or speaker fees from AstraZeneca, Boehringer Ingelheim, Chiesi, F. Hoffmann-La Roche Ltd and Menarini. The authors G.S., D.S., A.L., G.V., Fa.P., Fr.P., N.D.P., M.F., E.F. and E.G. declare that they have no conflicts of interest. EDAT- 2020/04/30 06:00 MHDA- 2020/04/30 06:01 PMCR- 2020/04/25 CRDT- 2020/04/30 06:00 PHST- 2020/03/23 00:00 [received] PHST- 2020/04/23 00:00 [revised] PHST- 2020/04/23 00:00 [accepted] PHST- 2020/04/30 06:00 [entrez] PHST- 2020/04/30 06:00 [pubmed] PHST- 2020/04/30 06:01 [medline] PHST- 2020/04/25 00:00 [pmc-release] AID - diagnostics10050253 [pii] AID - diagnostics-10-00253 [pii] AID - 10.3390/diagnostics10050253 [doi] PST - epublish SO - Diagnostics (Basel). 2020 Apr 25;10(5):253. doi: 10.3390/diagnostics10050253. PMID- 41985270 OWN - NLM STAT- Publisher LR - 20260415 IS - 1878-7541 (Electronic) IS - 1550-8307 (Linking) VI - 22 IP - 4 DP - 2026 Apr 11 TI - Integrative management of microvascular spasm-driven Raynaud's-like digital ischemia in an elderly smoker with mixed-pattern vasculopathy: A case report. PG - 103418 LID - S1550-8307(26)00105-9 [pii] LID - 10.1016/j.explore.2026.103418 [doi] AB - BACKGROUND: Chronic smoking is a key driver of endothelial dysfunction and microcirculatory impairment, contributing to "mixed-pattern vasculopathy," a condition characterized by coexisting macrovascular stenosis and microvascular tone dysregulation. This pathology significantly increases the risk of refractory distal ulceration, dry gangrene, and amputation. This case report aimed to describe the clinical course and therapeutic outcome of integrative treatment with Chinese herbal medicine (CHM) in a patient with refractory ischemia associated with mixed-pattern vasculopathy. CASE PRESENTATION: An 80-year-old man with a 100-pack-year smoking history presented with Raynaud's-like digital ischemia, including severe pain, cyanosis, and skin ulceration. Diagnostic imaging revealed multifocal atherosclerotic stenoses of the lower extremity arteries, emphysematous lung changes, cerebral small-vessel disease, and mild heart myocardial perfusion abnormalities. Although percutaneous transluminal angioplasty was performed, only partial revascularization was achieved because of unfavorable vascular architecture, and distal perfusion remained inadequate, with persistent cyanosis and pain. Integrative treatment with CHM, aimed at improving vascular circulation, was subsequently initiated. The intervention resulted in rapid pain reduction, improved peripheral skin perfusion, and complete healing of ulcers within 1 year. No recurrence of ischemic symptoms was observed during >2 years of follow-up. CONCLUSION: This case report suggests that integrative CHM may serve as a clinically meaningful adjunctive therapy for microvascular-predominant ischemia, particularly when conventional revascularization and standard vasodilator therapies fail to achieve adequate symptom control in patients with mixed-pattern vasculopathy. CI - Copyright © 2026. Published by Elsevier Inc. FAU - Chen, Ting-Ching AU - Chen TC AD - Department of Chinese Medicine, Tri-Service General Hospital, National Defense Medical University, Taipei, Taiwan. Electronic address: DOC60041@mail.ndmutsgh.edu.tw. FAU - Hsu, Chih-Hsueng AU - Hsu CH AD - Division of Cardiology, Department of Medicine, Tri-Service General Hospital, National Defense Medical University, Taipei, Taiwan. Electronic address: jhon10298@gmail.com. FAU - Lu, Chun-Chi AU - Lu CC AD - Division of Rheumatology, Immunology and Allergy, Tri-Service General Hospital, National Defense Medical University, Taipei, Taiwan; Division of Rheumatology, Immunology and Allergy, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. Electronic address: jameslutaiwan@gmail.com. FAU - Wang, Chih-Wei AU - Wang CW AD - Department of Radiology, Tri-Service General Hospital, National Defense Medical University, Taipei, Taiwan. Electronic address: davidwang666@gmail.com. FAU - Lin, Sunny Jui-Shan AU - Lin SJ AD - Department of Chinese Medicine, Tri-Service General Hospital, National Defense Medical University, Taipei, Taiwan; Graduate Institute of Medical Sciences, College of Medicine, National Defense Medical University, Taipei, Taiwan; Chinese Medical Advancement Foundation, Taichung, Taiwan. Electronic address: sunnylin@mail.ndmutsgh.edu.tw. LA - eng PT - Journal Article DEP - 20260411 PL - United States TA - Explore (NY) JT - Explore (New York, N.Y.) JID - 101233160 SB - IM OTO - NOTNLM OT - Case report OT - Chinese herbal medicine OT - Digital ischemia OT - Microcirculation OT - Mixed-pattern vasculopathy OT - Raynaud’s phenomenon COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/04/16 13:09 MHDA- 2026/04/16 13:09 CRDT- 2026/04/15 18:04 PHST- 2026/03/24 00:00 [received] PHST- 2026/04/09 00:00 [accepted] PHST- 2026/04/16 13:09 [medline] PHST- 2026/04/16 13:09 [pubmed] PHST- 2026/04/15 18:04 [entrez] AID - S1550-8307(26)00105-9 [pii] AID - 10.1016/j.explore.2026.103418 [doi] PST - aheadofprint SO - Explore (NY). 2026 Apr 11;22(4):103418. doi: 10.1016/j.explore.2026.103418. PMID- 35585956 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230602 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 7 IP - 2 DP - 2022 Jun TI - Nailfold capillaroscopy for the early diagnosis of the scleroderma spectrum of diseases in patients without Raynaud's phenomenon. PG - 144-150 LID - 10.1177/23971983221088460 [doi] AB - BACKGROUND: The utility of nailfold capillaroscopy in the evaluation of patients without Raynaud's phenomenon is unclear. OBJECTIVE: This study aims to compare the utility of nailfold capillaroscopy for the early diagnosis of the scleroderma-spectrum of diseases in patients who present with Raynaud's phenomenon, undifferentiated non-Raynaud's phenomenon features and positive systemic sclerosis-associated antibodies without scleroderma-spectrum of disease features. METHODS: Eligible patients were divided into three referral criteria groups: (I) Raynaud's phenomenon; (II) Undifferentiated non-Raynaud's phenomenon features and (III) Positive systemic sclerosis-associated autoantibodies without features to suggest scleroderma-spectrum of diseases. This includes systemic sclerosis, mixed connective tissue disease and dermatomyositis. The association between baseline scleroderma pattern on nailfold capillaroscopy (systemic sclerosis-nailfold capillaroscopy) and final diagnosis at follow-up was determined using logistic regression analysis. Test characteristics of nailfold capillaroscopy were compared and stratified by referral groups. RESULTS: Of 95 patients followed-up for a mean of 1.6 years, 28 (29.5%) patients developed scleroderma-spectrum of diseases, 36 (37.9%) patients had suspected/other connective tissue disease and 27 (28.4%) patients had no connective tissue disease. Baseline systemic sclerosis-nailfold capillaroscopy was significantly associated with the development of scleroderma-spectrum of diseases in patients from Group I (odds ratio, 7.1, p = 0.01) and Group II (odds ratio 7.3, p = 0.005). In Group II patients, nailfold capillaroscopy had a sensitivity, specificity, positive and negative predictive values of 71.4%, 76.5%, 55.6% and 86.7%, respectively. Specificity (81.8%) and PPV (69.2%) were the highest in Group I patients. Nailfold capillaroscopy had the highest negative predictive value in Group III (100%), followed by Group II (86.7%) and Group I (78.3%) patients. CONCLUSION: In addition to evaluating patients with Raynaud's phenomenon, nailfold capillaroscopy was useful in the evaluation and exclusion of scleroderma-spectrum of diseases in patients with undifferentiated non-Raynaud phenomenon features and those with systemic sclerosis-associated antibodies without features to suggest scleroderma-spectrum of diseases. CI - © The Author(s) 2022. FAU - Hong, Cassandra AU - Hong C AUID- ORCID: 0000-0002-2542-6521 AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore. AD - Yong Loo Lin School of Medicine, National University of Singapore, Singapore. AD - Duke-NUS Medical School, National University of Singapore, Singapore. FAU - Xiang, Ling AU - Xiang L AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore. AD - Yong Loo Lin School of Medicine, National University of Singapore, Singapore. FAU - Saffari, Seyed Ehsan AU - Saffari SE AUID- ORCID: 0000-0002-6473-4375 AD - Centre for Quantitative Medicine, Duke-NUS Medical School, National University of Singapore, Singapore. FAU - Low, Andrea Hl AU - Low AH AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore. AD - Yong Loo Lin School of Medicine, National University of Singapore, Singapore. AD - Duke-NUS Medical School, National University of Singapore, Singapore. LA - eng PT - Journal Article DEP - 20220421 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC9109506 OTO - NOTNLM OT - Systemic sclerosis OT - early diagnosis OT - nailfold capillaroscopy COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2022/05/20 06:00 MHDA- 2022/05/20 06:01 PMCR- 2023/06/01 CRDT- 2022/05/19 02:15 PHST- 2021/12/06 00:00 [received] PHST- 2022/03/01 00:00 [accepted] PHST- 2022/05/19 02:15 [entrez] PHST- 2022/05/20 06:00 [pubmed] PHST- 2022/05/20 06:01 [medline] PHST- 2023/06/01 00:00 [pmc-release] AID - 10.1177_23971983221088460 [pii] AID - 10.1177/23971983221088460 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2022 Jun;7(2):144-150. doi: 10.1177/23971983221088460. Epub 2022 Apr 21. PMID- 25065013 OWN - NLM STAT- MEDLINE DCOM- 20160121 LR - 20220309 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 54 IP - 11 DP - 2015 Nov TI - Old medications and new targeted therapies in systemic sclerosis. PG - 1944-53 LID - 10.1093/rheumatology/keu285 [doi] AB - SSc is a multiorgan disease with significant morbidity that is associated with poor health-related quality of life. Treatment of this condition is often organ based and non-curative. However, there are newer, potentially disease-modifying therapies available to treat certain aspects of the disease. This review focuses on old and new therapies in the management of SSc in clinical practice. CI - © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Nagaraja, Vivek AU - Nagaraja V AD - Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA and. FAU - Denton, Christopher P AU - Denton CP AD - Division of Medicine, University College London, London, UK. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA and khannad@med.umich.edu. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Review DEP - 20140726 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antirheumatic Agents) SB - IM MH - Antirheumatic Agents/*therapeutic use MH - Drug Therapy/*trends MH - Fibrosis/etiology MH - Humans MH - Scleroderma, Systemic/complications/diagnosis/*drug therapy MH - Skin/pathology MH - Vascular Diseases/etiology PMC - PMC4603276 OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ulcers OT - immunosuppressive therapy OT - interstitial lung disease OT - pulmonary arterial hypertension OT - renal crisis OT - skin fibrosis OT - systemic sclerosis OT - treatment EDAT- 2014/07/30 06:00 MHDA- 2016/01/23 06:00 PMCR- 2016/11/01 CRDT- 2014/07/28 06:00 PHST- 2014/01/20 00:00 [received] PHST- 2014/07/28 06:00 [entrez] PHST- 2014/07/30 06:00 [pubmed] PHST- 2016/01/23 06:00 [medline] PHST- 2016/11/01 00:00 [pmc-release] AID - keu285 [pii] AID - 10.1093/rheumatology/keu285 [doi] PST - ppublish SO - Rheumatology (Oxford). 2015 Nov;54(11):1944-53. doi: 10.1093/rheumatology/keu285. Epub 2014 Jul 26. PMID- 32508995 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 1792-0981 (Print) IS - 1792-1015 (Electronic) IS - 1792-0981 (Linking) VI - 20 IP - 1 DP - 2020 Jul TI - Dynamics of digital ulcers in systemic sclerosis. PG - 61-67 LID - 10.3892/etm.2020.8572 [doi] AB - Systemic sclerosis (SSc) is a collagenosis with insufficiently known etiopathogenesis, characterized by microvasculopathy and excessive fibrosis in the context of an autoimmune disorder. The incompletely elucidated pathogenesis and limited therapeutic options, disabling aspects, skin lesions and pain determine important functional and psychological deficiencies which affect the quality of life. It is imperative to observe and correlate individual clinical and paraclinical data to optimize disease management. A group of 22 patients diagnosed with SSc, hospitalized in a university clinic in Bucharest was included in an observational study. The evolution of digital ulcers was evaluated as an indicator of vasculopathy and their status and dynamics were correlated with clinical elements reflecting the fibrotic aspect of the disease. The present study shows that the Raynaud phenomenon is almost always present during the course of the disease, but its presence is not always associated with digital ulcers. The existing data in the literature show that fibrosis is subsequent to vasculopathy, but this study did not reveal causality between these two aspects of pathogenesis. The presence of microstomia and digital contracture was identified in the presence of digital ulcers, but also in their absence. The etiopathogenic mechanisms with multiple unknown involved factors open the opportunity to investigate many aspects of SSc for optimal aiming of therapeutic interventions. CI - Copyright: © Bobeică et al. FAU - Bobeică, Carmen AU - Bobeică C AD - Department of Dermato-Venereology, Doctoral School, University of Medicine and Pharmacy 'Gr. T. Popa' Iaşi, 700115 Iaşi, Romania. AD - Department of Morphological and Functional Sciences, 'Dunărea de Jos' University of Galaţi, Faculty of Medicine and Pharmacy, 800216 Galaţi, Romania. FAU - Tatu, Alin Laurențiu AU - Tatu AL AD - Department of Clinical Dermato-Venereology, 'Dunărea de Jos' University of Galaţi, Faculty of Medicine and Pharmacy, ReForm UDJ, 800216 Galaţi, Romania. AD - Department of Clinical Dermato-Venereology, University of Medicine and Pharmacy 'Gr. T. Popa', Faculty of Medicine and Pharmacy, 700115 Iaşi, Romania. FAU - Crăescu, Mihaela AU - Crăescu M AD - Department of Morphological and Functional Sciences, 'Dunărea de Jos' University of Galaţi, Faculty of Medicine and Pharmacy, 800216 Galaţi, Romania. AD - Department of Clinical Radiotherapy, 'Sf. Ap. Andrei' Emergency Clinical Hospital of Galaţi, 800578 Galaţi; , Romania. FAU - Solovăstru, Laura AU - Solovăstru L AD - Department of Clinical Dermato-Venereology, University of Medicine and Pharmacy 'Gr. T. Popa', Faculty of Medicine and Pharmacy, 700115 Iaşi, Romania. AD - Department of Clinical Dermato-Venereology, 'Sf. Spiridon' Emergency Clinical Hospital of Iaşi, 700111 Iaşi, Romania. LA - eng PT - Journal Article DEP - 20200227 PL - Greece TA - Exp Ther Med JT - Experimental and therapeutic medicine JID - 101531947 EIN - Exp Ther Med. 2020 Oct;20(4):3894. doi: 10.3892/etm.2020.9127. PMID: 32905209 PMC - PMC7271728 OTO - NOTNLM OT - Raynaud's phenomen OT - digital ulcers OT - microangiopaty OT - microstomia OT - microvasculopathy OT - systemic sclerosis EDAT- 2020/06/09 06:00 MHDA- 2020/06/09 06:01 PMCR- 2020/02/27 CRDT- 2020/06/09 06:00 PHST- 2020/01/07 00:00 [received] PHST- 2020/02/11 00:00 [accepted] PHST- 2020/06/09 06:00 [entrez] PHST- 2020/06/09 06:00 [pubmed] PHST- 2020/06/09 06:01 [medline] PHST- 2020/02/27 00:00 [pmc-release] AID - ETM-0-0-8572 [pii] AID - 10.3892/etm.2020.8572 [doi] PST - ppublish SO - Exp Ther Med. 2020 Jul;20(1):61-67. doi: 10.3892/etm.2020.8572. Epub 2020 Feb 27. PMID- 30190774 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1682-024X (Print) IS - 1681-715X (Electronic) IS - 1681-715X (Linking) VI - 34 IP - 4 DP - 2018 Jul-Aug TI - An elusive case of digital ischemia in a patient with Rheumatoid Arthritis. PG - 1024-1026 LID - 10.12669/pjms.344.15281 [doi] AB - Essential thrombocytosis (ET) has rarely been reported with autoimmune rheumatic disorders. We report a case of young female, diagnosed case of Rheumatoid arthritis (RA), who had been overlooked for her raised platelet counts. Later her symptoms of impending digital gangrene led to an active search for her thrombocytosis. JAK2 mutation came out to be positive and she was diagnosed as ET associated with RA. She was treated with Hydroxyurea and Aspirin, in addition to her RA treatment. Patient responded well to the treatment and her platelet counts have been gradually improved, however, she developed gangrene of toe, for which amputation of distal phalanx of toe and nail excision was done, later in the disease course. FAU - Azam, Waleed AU - Azam W AD - Dr. Waleed Azam, MBBS, FCPS Trainee. Medicine Department, Patel Hospital, ST-18, Block-4, Gulshan-e-Iqbal, Karachi, Pakistan. FAU - Erum, Uzma AU - Erum U AD - Dr. Uzma Erum, MBBS, FCPS (Medicine). Medicine Department, Patel Hospital, ST-18, Block-4, Gulshan-e-Iqbal, Karachi, Pakistan. FAU - Muhammad, Asif Jan AU - Muhammad AJ AD - Dr. Asif Jan Muhammad, MBBS, MCPS, MRCP (UK). Medicine Department, Patel Hospital, ST-18, Block-4, Gulshan-e-Iqbal, Karachi, Pakistan. LA - eng PT - Case Reports PT - Journal Article PL - Pakistan TA - Pak J Med Sci JT - Pakistan journal of medical sciences JID - 100913117 PMC - PMC6115556 OTO - NOTNLM OT - Erythromelalgia OT - Essential thrombocytosis OT - Raynaud’s phenomenon OT - Rheumatoid arthritis EDAT- 2018/09/08 06:00 MHDA- 2018/09/08 06:01 PMCR- 2018/07/01 CRDT- 2018/09/08 06:00 PHST- 2018/09/08 06:00 [entrez] PHST- 2018/09/08 06:00 [pubmed] PHST- 2018/09/08 06:01 [medline] PHST- 2018/07/01 00:00 [pmc-release] AID - PJMS-34-1024 [pii] AID - 10.12669/pjms.344.15281 [doi] PST - ppublish SO - Pak J Med Sci. 2018 Jul-Aug;34(4):1024-1026. doi: 10.12669/pjms.344.15281. PMID- 41392995 OWN - NLM STAT- In-Process LR - 20260308 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 53 IP - 3 DP - 2026 Mar TI - A Case of Anti-SRP Antibody-Positive Necrotizing Myopathy That Initially Presented With Raynaud's Phenomenon and a Digital Ulcer. PG - e173-e174 LID - 10.1111/1346-8138.70114 [doi] FAU - Shimizu, Tomomichi AU - Shimizu T AUID- ORCID: 0000-0001-7142-7800 AD - Department of Dermatology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Yamazaki, Fumikazu AU - Yamazaki F AUID- ORCID: 0000-0001-7292-6732 AD - Department of Dermatology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Kondoh, Akio AU - Kondoh A AD - Department of Dermatology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Kato, Eimi AU - Kato E AD - Department of Neurology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Mizuma, Atsushi AU - Mizuma A AD - Department of Neurology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Nagata, Eiichiro AU - Nagata E AD - Department of Neurology, Tokai University School of Medicine, Kanagawa, Japan. FAU - Mabuchi, Tomotaka AU - Mabuchi T AUID- ORCID: 0000-0002-0945-1137 AD - Department of Dermatology, Tokai University School of Medicine, Kanagawa, Japan. LA - eng GR - National Center of Neurology and Psychiatry/ PT - Letter DEP - 20251215 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM OTO - NOTNLM OT - anti‐signal recognition particle antibodies OT - cutaneous involvement OT - immune‐mediated necrotizing myopathy OT - interstitial lung disease OT - skin ulcer EDAT- 2025/12/15 13:10 MHDA- 2025/12/15 13:10 CRDT- 2025/12/15 06:03 PHST- 2025/11/18 00:00 [revised] PHST- 2025/08/19 00:00 [received] PHST- 2025/11/26 00:00 [accepted] PHST- 2025/12/15 13:10 [pubmed] PHST- 2025/12/15 13:10 [medline] PHST- 2025/12/15 06:03 [entrez] AID - 10.1111/1346-8138.70114 [doi] PST - ppublish SO - J Dermatol. 2026 Mar;53(3):e173-e174. doi: 10.1111/1346-8138.70114. Epub 2025 Dec 15. PMID- 37575740 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230815 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 7 DP - 2023 Jul TI - Leukocytoclastic Vasculitis: A Case Report. PG - e41736 LID - 10.7759/cureus.41736 [doi] LID - e41736 AB - Leukocytoclastic vasculitis, also known as hypersensitivity angiitis, is a cutaneous, small vessel vasculitis of the dermal capillaries and venules. The predominant clinical presentation is palpable purpura. Multiple medications can cause leukocytoclastic vasculitis, as well as autoimmune diseases, infections, and malignancy. The disease process may be limited to only the skin or a manifestation of a systemic vasculitis or process. Treatment is centered on symptom management. Our patient is a 60-year-old female who presented with bilateral dry and wet tender ulcerations. She was previously treated with paclizumab. CI - Copyright © 2023, Malik et al. FAU - Malik, Mona J AU - Malik MJ AD - Internal Medicine, Univeristy of California, Riverside, Riverside, USA. FAU - Pasha, Muhammad Nabeel AU - Pasha MN AD - Pulmonary and Critical Care Medicine, One Brooklyn Health, New York, USA. FAU - Salib, Victor AU - Salib V AD - Family Medicine, Univeristy of California, Riverside, Riverside, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230711 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10415167 OTO - NOTNLM OT - avascular necrosis OT - dermal hypersensitivity OT - fibrinoid OT - iga-mediated immune complex OT - leukocytocsis vasculitis OT - neutrophils OT - raynaud's phenomenon OT - skin necrosis OT - systemic COIS- The authors have declared that no competing interests exist. EDAT- 2023/08/14 06:43 MHDA- 2023/08/14 06:44 PMCR- 2023/07/11 CRDT- 2023/08/14 04:33 PHST- 2023/07/08 00:00 [accepted] PHST- 2023/08/14 06:44 [medline] PHST- 2023/08/14 06:43 [pubmed] PHST- 2023/08/14 04:33 [entrez] PHST- 2023/07/11 00:00 [pmc-release] AID - 10.7759/cureus.41736 [doi] PST - epublish SO - Cureus. 2023 Jul 11;15(7):e41736. doi: 10.7759/cureus.41736. eCollection 2023 Jul. PMID- 35707502 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2022 DP - 2022 TI - A Case of Anti-Jo-1 Myositis with Unique Biopsy Findings. PG - 9096643 LID - 10.1155/2022/9096643 [doi] LID - 9096643 AB - Antisynthetase syndrome (ASS) or anti-Jo-1 antibody syndrome has a classic clinical presentation including arthritis, myositis, interstitial lung disease, mechanic hands, and/or Raynaud's phenomenon. The biopsy findings are distinctive from polymyositis or dermatomyositis. We describe an interesting case of ASS where a patient presented with significant muscle weakness, proteinuria, and interstitial lung disease. She also had positive Ro-52 antibodies in addition to anti-Jo-1 antibodies. Her biopsy findings were consistent with inflammatory necrotizing myositis. CI - Copyright © 2022 Beenish Zulfiqar et al. FAU - Zulfiqar, Beenish AU - Zulfiqar B AUID- ORCID: 0000-0003-1330-2078 AD - Department of Rheumatology, University of Tennessee, Memphis, Tennessee, USA. FAU - Aksionav, Pavel AU - Aksionav P AD - Department of Rheumatology, University of Tennessee, Memphis, Tennessee, USA. FAU - Bittar, Mohamad AU - Bittar M AD - Department of Rheumatology, University of Tennessee, Memphis, Tennessee, USA. FAU - Chapman, Cathy AU - Chapman C AD - Department of Rheumatology, University of Tennessee, Memphis, Tennessee, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20220606 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC9192263 COIS- The authors declare that they have no conflicts of interest. EDAT- 2022/06/17 06:00 MHDA- 2022/06/17 06:01 PMCR- 2022/06/06 CRDT- 2022/06/16 02:40 PHST- 2022/04/21 00:00 [received] PHST- 2022/05/24 00:00 [accepted] PHST- 2022/06/16 02:40 [entrez] PHST- 2022/06/17 06:00 [pubmed] PHST- 2022/06/17 06:01 [medline] PHST- 2022/06/06 00:00 [pmc-release] AID - 10.1155/2022/9096643 [doi] PST - epublish SO - Case Rep Rheumatol. 2022 Jun 6;2022:9096643. doi: 10.1155/2022/9096643. eCollection 2022. PMID- 26833587 OWN - NLM STAT- MEDLINE DCOM- 20161219 LR - 20171116 IS - 1549-8719 (Electronic) IS - 1073-9688 (Linking) VI - 23 IP - 3 DP - 2016 Apr TI - Treprostinil Iontophoresis Improves Digital Blood Flow during Local Cooling in Systemic Sclerosis. PG - 266-70 LID - 10.1111/micc.12272 [doi] AB - INTRODUCTION: Severe Raynaud's syndrome and DUs are the most prevalent manifestations of SSc peripheral microvascular disease. We tested whether treprostinil iontophoresis on the finger pad of patients with SSc would improve digital blood flow during hand cooling. METHODS: Eleven patients with limited cutaneous SSc underwent a double-blinded iontophoresis of treprostinil (2.56 × 10(-4) M during two hours) and placebo (NaCl 0.9%) on two finger pads. Then, the hand was inserted for 30 minutes in a fenestrated cooling box at 8 °C, and skin blood flow was recorded continuously using LSCI. RESULTS: During the local cooling, CVC was significantly higher at the treprostinil site than at the placebo site and remained higher 30 minutes after the test. CONCLUSIONS: In patients with SSc, digital treprostinil iontophoresis shifts skin blood flow upward during local cooling of the hand and during the initial rewarming phase. Digital treprostinil iontophoresis should now be tested in larger scale studies. CI - © 2016 John Wiley & Sons Ltd. FAU - Gaillard-Bigot, Florence AU - Gaillard-Bigot F AD - University Grenoble Alpes, Grenoble, France. AD - Clinical Pharmacology Unit, INSERM CIC1406, Grenoble University Hospital, Grenoble, France. AD - INSERM, U1042, HP2, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AD - University Grenoble Alpes, Grenoble, France. AD - Clinical Pharmacology Unit, INSERM CIC1406, Grenoble University Hospital, Grenoble, France. AD - INSERM, U1042, HP2, Grenoble, France. FAU - Blaise, Sophie AU - Blaise S AD - INSERM, U1042, HP2, Grenoble, France. AD - Vascular Medicine Department, Grenoble University Hospital, Grenoble, France. FAU - Cracowski, Claire AU - Cracowski C AD - Clinical Pharmacology Unit, INSERM CIC1406, Grenoble University Hospital, Grenoble, France. AD - INSERM, U1042, HP2, Grenoble, France. FAU - Seinturier, Christophe AU - Seinturier C AD - Vascular Medicine Department, Grenoble University Hospital, Grenoble, France. FAU - Imbert, Bernard AU - Imbert B AD - Vascular Medicine Department, Grenoble University Hospital, Grenoble, France. FAU - Carpentier, Patrick AU - Carpentier P AD - Vascular Medicine Department, Grenoble University Hospital, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - University Grenoble Alpes, Grenoble, France. AD - Clinical Pharmacology Unit, INSERM CIC1406, Grenoble University Hospital, Grenoble, France. AD - INSERM, U1042, HP2, Grenoble, France. LA - eng PT - Clinical Trial PT - Journal Article PL - United States TA - Microcirculation JT - Microcirculation (New York, N.Y. : 1994) JID - 9434935 RN - DCR9Z582X0 (Epoprostenol) RN - RUM6K67ESG (treprostinil) SB - IM MH - Aged MH - Blood Flow Velocity/drug effects MH - Epoprostenol/administration & dosage/*analogs & derivatives MH - Female MH - Humans MH - *Hypothermia, Induced MH - Iontophoresis MH - Male MH - Microcirculation/*drug effects MH - Middle Aged MH - *Scleroderma, Systemic/physiopathology/therapy MH - *Skin/blood supply/physiopathology OTO - NOTNLM OT - Raynaud's phenomenon OT - laser speckle contrast imaging OT - microcirculation OT - prostacyclin OT - systemic sclerosis EDAT- 2016/02/03 06:00 MHDA- 2016/12/20 06:00 CRDT- 2016/02/03 06:00 PHST- 2015/10/21 00:00 [received] PHST- 2016/01/23 00:00 [accepted] PHST- 2016/02/03 06:00 [entrez] PHST- 2016/02/03 06:00 [pubmed] PHST- 2016/12/20 06:00 [medline] AID - 10.1111/micc.12272 [doi] PST - ppublish SO - Microcirculation. 2016 Apr;23(3):266-70. doi: 10.1111/micc.12272. PMID- 40970064 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250919 LR - 20250921 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 8 DP - 2025 Aug TI - Raynaud's Phenomenon, Anticentromere Antibodies and Digital Necrosis (RACAND) Is a Distinct Clinical Syndrome From Systemic Sclerosis: A Case Report and Literature Review. PG - e90362 LID - 10.7759/cureus.90362 [doi] LID - e90362 AB - The occurrence of digital necrosis and Raynaud's phenomenon with anticentromere (ACA) antibodies has been recognized as a rare clinical entity coined RACAND syndrome. Although these features occur in systemic sclerosis (SSc), RACAND syndrome lacks other features characteristic of SSc, such as sclerodactyly, skin thickening or organ involvement. Reports of this syndrome are scarce throughout the literature, with only 10 described cases of RACAND syndrome, but several more reports of digital gangrene associated with ACA lacking SSc features. In this article, we present the case of an 80-year-old woman who developed digital gangrene, with Raynaud's phenomenon and ACA in the absence of other signs and symptoms and no SSc features. The patient was diagnosed with RACAND syndrome and responded well to treatment with iloprost and moderate doses of prednisone. Additionally, a review of the available literature of similar cases is shown in order for clinicians to gain better insight of disease characteristics and possible treatment options, as there are no established treatment guidelines. The majority of patients were females of older age, mostly without medical history which would predispose them to a peripheral vasculopathy. Treatment including prostanoids might yield more promising results, however this requires further dedicated studies. We argue that RACAND syndrome is distinct from SSc and that the absence of other typical diagnostic features of SSc or other systemic autoimmune disease is necessary for diagnosis. Clinician awareness of this entity is needed in the treatment of digital necrosis, as well as further studies to determine the best treatment options. CI - Copyright © 2025, Hrkac et al. FAU - Hrkac, Stela AU - Hrkac S AD - Department of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, HRV. FAU - Mitrovic, Josko AU - Mitrovic J AD - Department of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, HRV. AD - School of Medicine, University of Zagreb, Zagreb, HRV. AD - Faculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, HRV. FAU - Golob, Majda AU - Golob M AD - Department of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, HRV. FAU - Pitesa Kosutic, Ivana AU - Pitesa Kosutic I AD - Department of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, HRV. FAU - Salamon, Lea AU - Salamon L AD - Department of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, HRV. AD - School of Medicine, University of Zagreb, Zagreb, HRV. LA - eng PT - Case Reports PT - Journal Article DEP - 20250818 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12441989 OTO - NOTNLM OT - anti-centromere antibodies OT - digital ischemia OT - digital necrosis OT - racand syndrome OT - systemic sclerosis COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Ethics Committee of Dubrava University Hospital issued approval N/A. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/09/19 06:33 MHDA- 2025/09/19 06:34 PMCR- 2025/08/18 CRDT- 2025/09/19 05:09 PHST- 2025/08/17 00:00 [accepted] PHST- 2025/09/19 06:34 [medline] PHST- 2025/09/19 06:33 [pubmed] PHST- 2025/09/19 05:09 [entrez] PHST- 2025/08/18 00:00 [pmc-release] AID - 10.7759/cureus.90362 [doi] PST - epublish SO - Cureus. 2025 Aug 18;17(8):e90362. doi: 10.7759/cureus.90362. eCollection 2025 Aug. PMID- 19820657 OWN - NLM STAT- MEDLINE DCOM- 20100126 LR - 20091012 IS - 1539-0713 (Electronic) IS - 0884-741X (Linking) VI - 27 IP - 9 DP - 2009 Oct TI - Update on rheumatology: part 2. PG - 535-9; quiz 540-1 LID - 10.1097/01.NHH.0000361924.22174.92 [doi] AB - Ms. J. is a 60-year-old woman with severe Raynaud's phenomenon, bibasilar pulmonary fibrosis, gastroesophageal reflux disease (GERD) with dysphagia, and muscle weakness. What is a possible rheumatological diagnosis and what might the home health clinician do next? FAU - Neal-Boylan, Leslie AU - Neal-Boylan L AD - Yale University School of Nursing, New Haven, CT 06519, USA. Leslie.neal-boylan@yale.edu LA - eng PT - Journal Article PT - Review PL - United States TA - Home Healthc Nurse JT - Home healthcare nurse JID - 8403379 MH - Comorbidity MH - Home Care Services/*standards MH - Humans MH - Nursing Assessment MH - Rheumatic Diseases/diagnosis/*nursing MH - Rheumatology/*standards RF - 8 EDAT- 2009/10/13 06:00 MHDA- 2010/01/27 06:00 CRDT- 2009/10/13 06:00 PHST- 2009/10/13 06:00 [entrez] PHST- 2009/10/13 06:00 [pubmed] PHST- 2010/01/27 06:00 [medline] AID - 00004045-200910000-00006 [pii] AID - 10.1097/01.NHH.0000361924.22174.92 [doi] PST - ppublish SO - Home Healthc Nurse. 2009 Oct;27(9):535-9; quiz 540-1. doi: 10.1097/01.NHH.0000361924.22174.92. PMID- 33549135 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20211101 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 61 IP - 1 DP - 2021 Feb 6 TI - Hematopoietic stem cell transplantation for systemic sclerosis: Brazilian experience. PG - 9 LID - 10.1186/s42358-021-00166-8 [doi] AB - BACKGROUND: In the past 20 years, hematopoietic stem cell transplantation (HSCT) has been investigated as treatment for systemic sclerosis (SSc). The goal of HSCT is to eradicate the autoreactive immune system, which is replaced by a new immune repertoire with long-lasting regulation and tolerance to autoantigens. Here, we describe the clinical outcomes of severe and refractory SSc patients that underwent HSCT at a single Brazilian center. PATIENTS AND METHODS: This is a longitudinal and retrospective study, including 70 adult SSc patients, with an established diagnosis of SSc, and who underwent autologous HSCT from 2009 to 2016. The procedure included harvesting and cryopreservation of autologous hematopoietic progenitor cells, followed by administration of an immunoablative regimen and subsequent infusion of the previously collected cells. Patients were evaluated immediately before transplantation, at 6 months and then yearly until at least 5-years of post-transplantation follow-up. At each evaluation time point, patients underwent clinical examination, including modified Rodnan's skin score (mRSS) assessment, echocardiography, high-resolution computed tomography of the lungs and pulmonary function. RESULTS: Median (range) age was 35.9 (19-59), with 57 (81.4%) female and median (range) non-Raynaud's disease duration of 2 (1-7) years. Before transplantation, 96% of the patients had diffuse skin involvement, 84.2%, interstitial lung disease and 67%, positive anti-topoisomerase I antibodies. Skin involvement significantly improved, with a decline in mRSS at all post-transplantation time points until at least 5-years of follow-up. When patients with pre-HSCT interstitial lung disease were analyzed, there was an improvement in pulmonary function (forced vital capacity and diffusing capacity of lung for carbon monoxide) over the 5-year follow-up. Overall survival was 81% and progression-free survival was 70.5% at 8-years after HSCT. Three patients died due to transplant-related toxicity, 9 patients died over follow-up due to disease reactivation and one patient died due to thrombotic thrombocytopenic purpura. CONCLUSIONS: Autologous hematopoietic progenitor cell transplantation improves skin and interstitial lung involvement. These results are in line with the international experience and support HSCT as a viable therapeutic alternative for patients with severe and progressive systemic sclerosis. FAU - Henrique-Neto, Álvaro AU - Henrique-Neto Á AD - Graduate Program in Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Vasconcelos, Marianna Yumi Kawashima AU - Vasconcelos MYK AD - Graduate Program in Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Dias, Juliana Bernardes Elias AU - Dias JBE AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - de Moraes, Daniela Aparecida AU - de Moraes DA AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Gonçalves, Maynara Santana AU - Gonçalves MS AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Graduate Program in Oncology, Stem Cells and Cell Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Zanin-Silva, Djúlio César AU - Zanin-Silva DC AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Graduate Program in Basic and Applied Immunology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Zucoloto, Talita Graminha AU - Zucoloto TG AD - Graduate Program in Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - de Oliveira, Marília de Fátima Cirioli AU - de Oliveira MFC AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Ribeirão Preto School of Nursing, University of São Paulo, Ribeirão Preto, Brazil. FAU - Dotoli, Giuliana Martinelli AU - Dotoli GM AD - Graduate Program in Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Weffort, Luiz Fernando AU - Weffort LF AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Leopoldo, Vanessa Cristina AU - Leopoldo VC AD - Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Ribeirão Preto School of Nursing, University of São Paulo, Ribeirão Preto, Brazil. FAU - Oliveira, Maria Carolina AU - Oliveira MC AUID- ORCID: 0000-0003-0691-2222 AD - Center for Cell-Based Therapy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. mcarolor@usp.br. AD - Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Avenida dos Bandeirantes 3900, Ribeirão Preto, SP, 14048-900, Brazil. mcarolor@usp.br. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210206 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 SB - IM MH - Adult MH - Brazil MH - Cause of Death MH - Female MH - *Hematopoietic Stem Cell Transplantation/methods/mortality MH - Humans MH - Longitudinal Studies MH - Lung Diseases, Interstitial/physiopathology/*therapy MH - Male MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/immunology/mortality/*therapy MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - Hematopoietic stem cell transplantation OT - Progenitor cells OT - Stem cells OT - Systemic sclerosis OT - Transplantation EDAT- 2021/02/08 06:00 MHDA- 2021/11/03 06:00 CRDT- 2021/02/07 20:27 PHST- 2020/11/13 00:00 [received] PHST- 2021/01/26 00:00 [accepted] PHST- 2021/02/07 20:27 [entrez] PHST- 2021/02/08 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] AID - 10.1186/s42358-021-00166-8 [pii] AID - 10.1186/s42358-021-00166-8 [doi] PST - epublish SO - Adv Rheumatol. 2021 Feb 6;61(1):9. doi: 10.1186/s42358-021-00166-8. PMID- 33847003 OWN - NLM STAT- MEDLINE DCOM- 20210705 LR - 20220531 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 48 IP - 7 DP - 2021 Jul TI - The clinical characteristics and predictors of severe digital ischemia in patients with anti-aminoacyl transfer RNA synthetase antibodies. PG - 1044-1051 LID - 10.1111/1346-8138.15884 [doi] AB - Severe digital ischemia (SDI), which presents with digital ulcers, necrosis, or gangrene, has been reported to be a rare manifestation of anti-aminoacyl transfer RNA synthetase (ARS) antibody-positive polymyositis/dermatomyositis or anti-synthetase syndrome. A retrospective study was conducted between 2009 and 2020 at our department to investigate the clinical features of anti-ARS antibody-positive patients with SDI and identify their predictors. A total of 46 patients who were positive for anti-ARS antibody were included, four of whom (8.7%) presented with SDI. The characteristics of the patients with SDI were as follows: the median age was 74 years, with 75% being female; anti-Jo-1 antibody, Raynaud's phenomenon, interstitial lung disease, and myositis were observed in two (50%), four (100%), four (100%), and three patients (75%), respectively. Next, we reviewed the literature of anti-ARS antibody-positive patients with SDI and investigated the predictors of SDI by analyzing a total of 51 patients, including the previously reported five patients with SDI. Multivariable analyses revealed that Raynaud's phenomenon and myositis independently predicted the development of SDI in patients with anti-ARS antibody. In conclusion, digital ulcers, necrosis, or gangrene seem to be more common presentations in our study, and Raynaud's phenomenon and myositis can predict the complications of SDI in anti-ARS antibody-positive patients. CI - © 2021 Japanese Dermatological Association. FAU - Suma, Harumichi AU - Suma H AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Yoshida, Yusuke AU - Yoshida Y AUID- ORCID: 0000-0002-0774-0509 AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Sugimoto, Tomohiro AU - Sugimoto T AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Matsuo, Yoshimi AU - Matsuo Y AD - Department of Dermatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Law, Sze-Ming AU - Law SM AD - Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. FAU - Nakashima, Ran AU - Nakashima R AD - Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. FAU - Kobayashi, Hiroki AU - Kobayashi H AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Hosokawa, Yohei AU - Hosokawa Y AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Ishitoku, Michinori AU - Ishitoku M AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Kohno, Hiroki AU - Kohno H AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Watanabe, Hirofumi AU - Watanabe H AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Tokunaga, Tadahiro AU - Tokunaga T AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Mokuda, Sho AU - Mokuda S AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Nojima, Takaki AU - Nojima T AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Hirata, Shintaro AU - Hirata S AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. FAU - Sugiyama, Eiji AU - Sugiyama E AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan. LA - eng GR - 19K08908/JSPS KAKENHI/ GR - 19K18499/JSPS KAKENHI/ GR - 19K07940/JSPS KAKENHI/ PT - Journal Article PT - Review DEP - 20210413 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Aged MH - *Amino Acyl-tRNA Synthetases MH - Autoantibodies MH - *Dermatomyositis MH - Female MH - Humans MH - Ischemia MH - Male MH - *Myositis MH - Retrospective Studies OTO - NOTNLM OT - Raynaud's phenomenon OT - aminoacyl transfer RNA synthetase OT - gangrene OT - necrosis OT - ulcer EDAT- 2021/04/14 06:00 MHDA- 2021/07/06 06:00 CRDT- 2021/04/13 06:41 PHST- 2021/03/12 00:00 [revised] PHST- 2021/01/30 00:00 [received] PHST- 2021/03/19 00:00 [accepted] PHST- 2021/04/14 06:00 [pubmed] PHST- 2021/07/06 06:00 [medline] PHST- 2021/04/13 06:41 [entrez] AID - 10.1111/1346-8138.15884 [doi] PST - ppublish SO - J Dermatol. 2021 Jul;48(7):1044-1051. doi: 10.1111/1346-8138.15884. Epub 2021 Apr 13. PMID- 35220958 OWN - NLM STAT- MEDLINE DCOM- 20220404 LR - 20220405 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 22 IP - 1 DP - 2022 Feb 27 TI - Interstitial lung disease in Primary Sjögren's syndrome. PG - 73 LID - 10.1186/s12890-022-01868-5 [doi] LID - 73 AB - BACKGROUND: Interstitial lung disease (ILD) may cause life-threatening complications of primary Sjogren's syndrome (pSS), and has a poor prognosis in terms of survival and quality of life. To date, few studies have investigated the risk factors for ILD detected by high-resolution computed tomography (HRCT) in pSS patients with or without respiratory symptoms. METHODS: Data of 333 patients with newly diagnosed pSS were retrospectively analysed. Interstitial lung disease involvement was defined as typical abnormalities on HRCT and/or pulmonary function tests. Multivariate regression model was used to evaluate the association between interstitial lung disease and pSS characteristics. RESULTS: Sixty-six patients (19.82%) were diagnosed with pSS-ILD. Ground glass opacities (87.88%) and septal/sub pleural lines (81.82%) were most frequent. Based on pulmonary high-resolution computed tomography, patients were divided into nonspecific (n = 42), usual (n = 20), lymphocytic interstitial pneumonia (n = 3) and cryptogenic organising pneumonia (n = 1) groups. There was a strong association between erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) and the HRCT-score. Pulmonary function tests revealed impaired diffusion capacity for carbon monoxide and total lung capacity, and coexistence of small airway lesions in pSS-interstitial lung disease. On logistic regression analysis, age, Raynaud's phenomenon, lymphopenia, cough, dyspnoea and rampant dental caries were risk factors associated with pSS-interstitial lung disease. CONCLUSIONS: Interstitial lung disease involvement in pSS is a common clinical occurrence. The clinical manifestation is nonspecific and variable; Raynaud's phenomenon and lymphopenia may predict its onset. pSS patients with advanced age, dry cough and dyspnoea should be systematically evaluated for ILD involvement and managed according to their symptoms. CI - © 2022. The Author(s). FAU - Lin, Wei AU - Lin W AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Xin, Zhifei AU - Xin Z AD - Department of Thoracic Surgery, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Zhang, Jianlong AU - Zhang J AD - Department of Medical Imaging, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Liu, Ning AU - Liu N AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Ren, Xiuying AU - Ren X AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Liu, Meilu AU - Liu M AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Su, Yashuang AU - Su Y AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Liu, Yixuan AU - Liu Y AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Yang, Liu AU - Yang L AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Guo, Shaoying AU - Guo S AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Yang, Yupeng AU - Yang Y AD - Department of Stomatology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Li, Yang AU - Li Y AD - Department of Oncology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Cao, Jingjing AU - Cao J AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Ning, Xiaoran AU - Ning X AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Li, Jingjing AU - Li J AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Xue, He AU - Xue H AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Niu, Nannan AU - Niu N AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Chen, Yingmin AU - Chen Y AD - Department of Medical Imaging, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Li, Fang AU - Li F AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Sun, Lijun AU - Sun L AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. slj8042@163.com. FAU - Zhang, Xiaopeng AU - Zhang X AD - Department of Thoracic Surgery, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. zxp8756@163.com. FAU - Zhang, Fengxiao AU - Zhang F AD - Department of Rheumatology and Immunology, Hebei General Hospital, Hebei, 050051, Shijiazhuang, China. FAU - Zhang, Wen AU - Zhang W AD - Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, 100730, China. LA - eng GR - No.81801630/National Nature Science Foundation of China/ GR - No. H2018307047/National Nature Science Foundation of Hebei Province/ PT - Journal Article DEP - 20220227 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 SB - IM MH - *Dental Caries/complications/pathology MH - Humans MH - Lung MH - *Lung Diseases, Interstitial/epidemiology/etiology/pathology MH - Quality of Life MH - Retrospective Studies MH - *Sjogren's Syndrome/complications/epidemiology PMC - PMC8882286 OTO - NOTNLM OT - Interstitial lung disease OT - Primary Sjögren syndrome OT - Raynaud's phenomenon OT - Risk factors OT - Warrick score COIS- The authors declare no potential conflicts of interest. EDAT- 2022/03/01 06:00 MHDA- 2022/04/05 06:00 PMCR- 2022/02/27 CRDT- 2022/02/28 05:27 PHST- 2021/11/13 00:00 [received] PHST- 2022/02/18 00:00 [accepted] PHST- 2022/02/28 05:27 [entrez] PHST- 2022/03/01 06:00 [pubmed] PHST- 2022/04/05 06:00 [medline] PHST- 2022/02/27 00:00 [pmc-release] AID - 10.1186/s12890-022-01868-5 [pii] AID - 1868 [pii] AID - 10.1186/s12890-022-01868-5 [doi] PST - epublish SO - BMC Pulm Med. 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID- 34849114 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240818 IS - 1642-395X (Print) IS - 2299-0046 (Electronic) IS - 1642-395X (Linking) VI - 38 IP - 5 DP - 2021 Oct TI - Controversy around the morphea. PG - 716-720 LID - 10.5114/ada.2021.106242 [doi] AB - Morphea, also known as localized scleroderma, is a chronic, autoimmune disease of connective tissue. It is characterized by a typical clinical feature. In morphea, there is no Raynaud's phenomenon, no sclerodactyly or no ulcerations on the fingertips. Although morphea and systemic sclerosis have been perceived as separate disease entities for years, they are still confused both by patients (which is a source of unnecessary stress) and doctors. This may be due to, in part, misunderstood terminology. The controversy around morphea also concerns the division of this disease entity, including its less common subtypes, such as eosinophilic fasciitis. Discussions also revolve around the diagnostic aspects and possible treatment options. The paper attempts to present the debatable aspects regarding nomenclature, classification, diagnosis and treatment of morphea. CI - Copyright: © 2021 Termedia Sp. z o. o. FAU - Dańczak-Pazdrowska, Aleksandra AU - Dańczak-Pazdrowska A AD - Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Cieplewicz, Paulina AU - Cieplewicz P AD - Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Żaba, Ryszard AU - Żaba R AD - Department of Dermatology and Venereology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Adamski, Zygmunt AU - Adamski Z AD - Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland. FAU - Polańska, Adriana AU - Polańska A AD - Department of Dermatology and Venereology, Poznan University of Medical Sciences, Poznan, Poland. LA - eng PT - Journal Article PT - Review DEP - 20210518 PL - Poland TA - Postepy Dermatol Alergol JT - Postepy dermatologii i alergologii JID - 101168357 PMC - PMC8610066 OTO - NOTNLM OT - eosinophilic fasciitis OT - localized scleroderma OT - morphea OT - penicillin COIS- The authors declare no conflict of interest. EDAT- 2021/12/02 06:00 MHDA- 2021/12/02 06:01 PMCR- 2021/10/01 CRDT- 2021/12/01 06:54 PHST- 2021/03/10 00:00 [received] PHST- 2021/03/23 00:00 [accepted] PHST- 2021/12/01 06:54 [entrez] PHST- 2021/12/02 06:00 [pubmed] PHST- 2021/12/02 06:01 [medline] PHST- 2021/10/01 00:00 [pmc-release] AID - 44121 [pii] AID - 10.5114/ada.2021.106242 [doi] PST - ppublish SO - Postepy Dermatol Alergol. 2021 Oct;38(5):716-720. doi: 10.5114/ada.2021.106242. Epub 2021 May 18. PMID- 41229634 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251113 LR - 20251115 IS - 2284-2594 (Electronic) IS - 2284-2594 (Linking) VI - 12 IP - 11 DP - 2025 TI - Raynaud's Phenomenon and Pericardial Effusion Requiring Management with A Pericardial Window as the only Manifestations of Systemic Sclerosis without Scleroderma for over 10 Years, after which Pulmonary Hypertension Developed. PG - 005866 LID - 10.12890/2025_005866 [doi] LID - 005866 AB - INTRODUCTION: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterised by vasculopathy and progressive fibrosis affecting the skin and internal organs. Systemic sclerosis sine scleroderma (ssSSc) is an uncommon variant, accounting for 5-10% of cases and is defined by the absence of cutaneous involvement despite internal organ manifestations. CASE DESCRIPTION: We present a case of a patient who initially presented with recurrent pericardial effusion and late-onset Raynaud's phenomenon. Despite serological markers, SSc was not diagnosed, due to absence of skin involvement, until pulmonary hypertension (PH) developed over a decade later. Nailfold capillaroscopy confirmed ssSSc. Surgical pericardial drainage and targeted PH therapy led to clinical improvement. DISCUSSION: Pericardial effusion may be an early and under-recognised manifestation of ssSSc. While typically attributed to inflammation, our patient had a transudative effusion with a poor response to steroids, suggesting a non-inflammatory mechanism. The effusion preceded PH by over a decade, challenging current pathophysiological theories. Surgical drainage was effective, contrasting with poorer outcomes in cutaneous SSc. CONCLUSION: This case highlights the diagnostic complexity and heterogeneity of cardiac involvement in ssSSc. It underscores the importance of maintaining a high index of suspicion in patients with unexplained cardiopulmonary symptoms and demonstrates the indolent course of the disease in atypical SSc. LEARNING POINTS: Systemic sclerosis sine scleroderma (ssSSc) may be diagnosed late due to the absence of skin involvement, underscoring the need for clinical vigilance.Visceral manifestations can precede Raynaud's phenomenon, challenging the traditional diagnostic sequence in systemic sclerosis.Pericardial effusion may be an early and under-recognised feature of ssSSc, often presenting with non-specific symptoms and requiring a high index of suspicion. CI - © EFIM 2025. FAU - Moriarty, Natassja AU - Moriarty N AD - Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom. FAU - Vadiveloo, Thivya Dharshini V AU - Vadiveloo TDV AD - Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom. FAU - Osman, Fathelrahman AU - Osman F AD - Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom. FAU - Benson, Rosalind AU - Benson R AD - Rheumatology, Aintree University Hospital, Liverpool, United Kingdom. FAU - Nelson, Katherine AU - Nelson K AD - Rheumatology, Broadgreen Hospital, Liverpool, United Kingdom. LA - eng PT - Journal Article DEP - 20251028 PL - Italy TA - Eur J Case Rep Intern Med JT - European journal of case reports in internal medicine JID - 101648453 PMC - PMC12604890 OTO - NOTNLM OT - Systemic sclerosis OT - pericardial effusion OT - pulmonary hypertension OT - systemic sclerosis sine scleroderma COIS- Conflicts of Interests: The Authors declare that there are no competing interests. EDAT- 2025/11/13 06:30 MHDA- 2025/11/13 06:31 PMCR- 2025/10/28 CRDT- 2025/11/13 04:36 PHST- 2025/09/24 00:00 [received] PHST- 2025/10/07 00:00 [accepted] PHST- 2025/11/13 06:31 [medline] PHST- 2025/11/13 06:30 [pubmed] PHST- 2025/11/13 04:36 [entrez] PHST- 2025/10/28 00:00 [pmc-release] AID - 5866 [pii] AID - 10.12890/2025_005866 [doi] PST - epublish SO - Eur J Case Rep Intern Med. 2025 Oct 28;12(11):005866. doi: 10.12890/2025_005866. eCollection 2025. PMID- 38524014 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240405 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 2 DP - 2024 Feb TI - Exploring the Impact of Occupational Silica Exposure Progressing to Systemic Sclerosis: A Report on the Development of Silica-Induced Systemic Sclerosis Cases. PG - e54595 LID - 10.7759/cureus.54595 [doi] LID - e54595 AB - Erasmus syndrome is an uncommon disease brought on by exposure to silica and later manifests as systemic sclerosis (SSc) with or without silicosis. The body of literature on Erasmus syndrome is scarce. Here, we report two cases of male patients presenting with SSc after silica exposure. One of the patients had worked in the steel industry, and another had worked in the sculpture manufacturing for a decade before the presentation. It is imperative to raise awareness of this uncommon illness because avoiding further exposure remains the mainstay of management. Our case reports reemphasize the importance of occupational history in all patients of SSc. CI - Copyright © 2024, Patro et al. FAU - Patro, Mahismita AU - Patro M AD - Pulmonary Medicine & Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND. FAU - Girija, Aswathy AU - Girija A AD - Pulmonary Medicine & Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND. FAU - Sarkar, Subho AU - Sarkar S AD - Pulmonary Medicine & Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND. FAU - Mohapatra, Prasanta R AU - Mohapatra PR AD - Pulmonary Medicine & Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND. FAU - Shirgaokar, Rohit AU - Shirgaokar R AD - Pulmonary Medicine & Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20240221 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10958239 OTO - NOTNLM OT - erasmus syndrome OT - raynaud’s phenomena OT - sclerodactyly OT - silicosis OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2024/03/25 06:43 MHDA- 2024/03/25 06:44 PMCR- 2024/02/21 CRDT- 2024/03/25 04:21 PHST- 2024/02/20 00:00 [accepted] PHST- 2024/03/25 06:44 [medline] PHST- 2024/03/25 06:43 [pubmed] PHST- 2024/03/25 04:21 [entrez] PHST- 2024/02/21 00:00 [pmc-release] AID - 10.7759/cureus.54595 [doi] PST - epublish SO - Cureus. 2024 Feb 21;16(2):e54595. doi: 10.7759/cureus.54595. eCollection 2024 Feb. PMID- 34840747 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240404 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 71 DP - 2021 Nov TI - Systemic sclerosis cutaneous expression: Management of skin fibrosis and digital ulcers. PG - 102984 LID - 10.1016/j.amsu.2021.102984 [doi] LID - 102984 AB - Systemic sclerosis is a connective tissue disease with cutaneous involvement. Clinical manifestations result from the balance of inflammations/autoimmunity process and fibrogenesis. Patients suffer from skin ulcers, non-ulcerative lesions including digital pitting scars, telangiectasias, subungual hyperkeratosis, abrasions, fissures, and subcutaneous calcinosis. A review about the pathophysiology of the disease, the physical examination of the patients, the instrumental assessment, and possible treatments is performed. CI - © 2021 The Authors. FAU - Starnoni, Marta AU - Starnoni M AD - Department of Medical and Surgical Sciences, Division of Plastic Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. AD - Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy. FAU - Pappalardo, Marco AU - Pappalardo M AD - Department of Medical and Surgical Sciences, Division of Plastic Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Spinella, Amelia AU - Spinella A AD - Department of Rheumatology, Division of Rheumatology, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Testoni, Sofia AU - Testoni S AD - Department of Rheumatology, Division of Rheumatology, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Lattanzi, Melba AU - Lattanzi M AD - Department of Medical and Surgical Sciences, Division of Plastic Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Feminò, Raimondo AU - Feminò R AD - Department of Anesthesiology, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - De Santis, Giorgio AU - De Santis G AD - Department of Medical and Surgical Sciences, Division of Plastic Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Department of Rheumatology, Division of Rheumatology, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Department of Rheumatology, Division of Rheumatology, University of Modena and Reggio Emilia, Policlinico of Modena, Largo Pozzo 71, 41124, Modena, Italy. LA - eng PT - Journal Article PT - Review DEP - 20211102 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC8606707 OTO - NOTNLM OT - Autologous fat grafting OT - DU, digital ulcer OT - Digital ulcers OT - MHISS, Mouth Handicap in Systemic Sclerosis scale OT - ROM, Range of Motion OT - RP, Raynaud's phenomenon OT - SSc, Systemic sclerosis OT - SU, skin ulcer OT - Scleroderma OT - Skin fibrosis OT - Systemic sclerosis COIS- The authors declares that there is no conflict of interest regarding the publication of this paper. EDAT- 2021/11/30 06:00 MHDA- 2021/11/30 06:01 PMCR- 2021/11/02 CRDT- 2021/11/29 06:28 PHST- 2021/07/30 00:00 [received] PHST- 2021/10/25 00:00 [revised] PHST- 2021/10/28 00:00 [accepted] PHST- 2021/11/29 06:28 [entrez] PHST- 2021/11/30 06:00 [pubmed] PHST- 2021/11/30 06:01 [medline] PHST- 2021/11/02 00:00 [pmc-release] AID - S2049-0801(21)00934-1 [pii] AID - 102984 [pii] AID - 10.1016/j.amsu.2021.102984 [doi] PST - epublish SO - Ann Med Surg (Lond). 2021 Nov 2;71:102984. doi: 10.1016/j.amsu.2021.102984. eCollection 2021 Nov. PMID- 30050814 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220318 IS - 2229-5178 (Print) IS - 2249-5673 (Electronic) IS - 2229-5178 (Linking) VI - 9 IP - 4 DP - 2018 Jul-Aug TI - Evaluation of Serum Vitamin D Levels in Patients with Systemic Sclerosis and Healthy Controls: Results of a Pilot Study. PG - 250-255 LID - 10.4103/idoj.IDOJ_328_17 [doi] AB - BACKGROUND: The anti-inflammatory, immunomodulatory, and anti-proliferative effects of vitamin D in pathogenesis of autoimmune diseases have been highlighted in recent years but implications of vitamin D deficiency in systemic sclerosis (SSc) remain understudied. OBJECTIVES: To evaluate serum vitamin D levels in SSc patients and matched controls. MATERIALS AND METHODS: Serum vitamin D levels were estimated in 38 (M:F 5:33) patients aged 23-70 years of untreated SSc and age and gender matched healthy controls. Clinical and investigative evaluation for skin sclerosis by modified Rodnan skin score (mRSS), presence of digital ulcers, Raynaud's phenomenon, type of auto-antibodies, systemic involvement, and serum vitamin D levels were performed. Serum vitamin D levels were defined as normal (30-100 ng/ml), insufficient (10-30 ng/ml), and deficient (<10 ng/ml). RESULTS: Serum vitamin D levels (median ± IQR) were 19.5 ± 77.8 ng/ml in 38 patients and 100 ± 31.3 ng/ml in controls each. Vitamin D deficiency in 13 (34.2%) and insufficiency in 10 (26.3%) patients were identified. Only 2 (5.3%) controls had vitamin D insufficiency and the difference was statistically significant (P = 0.001). An inverse relationship was observed between mRSS and serum vitamin D levels. CONCLUSIONS: Patients with SSc have significantly lower serum vitamin D levels than healthy controls. Serum vitamin D levels do not correlate well with age, gender, disease duration or its variants, type of auto antibodies, presence of digital ulceration, or systemic involvement but has inverse correlation with skin sclerosis. Better-designed studies will perhaps resolve issues of potential benefits of vitamin D supplementation in modification of disease activity or severity in SSc. FAU - Gupta, Sarita AU - Gupta S AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Mahajan, Vikram K AU - Mahajan VK AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Yadav, Rajinder S AU - Yadav RS AD - Department of Biochemistry, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Mehta, Karaninder S AU - Mehta KS AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Bhushan, Satya AU - Bhushan S AD - Department of Biochemistry, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Chauhan, Pushpinder S AU - Chauhan PS AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Rawat, Ritu AU - Rawat R AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. FAU - Sharma, Vikas AU - Sharma V AD - Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh, India. LA - eng PT - Journal Article PL - India TA - Indian Dermatol Online J JT - Indian dermatology online journal JID - 101586880 PMC - PMC6042194 OTO - NOTNLM OT - Autoimmune disorders OT - Raynaud's phenomenon OT - connective tissue diseases OT - dysphagia OT - dyspnea OT - scleroderma COIS- There are no conflicts of interest. EDAT- 2018/07/28 06:00 MHDA- 2018/07/28 06:01 PMCR- 2018/07/01 CRDT- 2018/07/28 06:00 PHST- 2018/07/28 06:00 [entrez] PHST- 2018/07/28 06:00 [pubmed] PHST- 2018/07/28 06:01 [medline] PHST- 2018/07/01 00:00 [pmc-release] AID - IDOJ-9-250 [pii] AID - 10.4103/idoj.IDOJ_328_17 [doi] PST - ppublish SO - Indian Dermatol Online J. 2018 Jul-Aug;9(4):250-255. doi: 10.4103/idoj.IDOJ_328_17. PMID- 32104228 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231103 IS - 1792-0981 (Print) IS - 1792-1015 (Electronic) IS - 1792-0981 (Linking) VI - 19 IP - 3 DP - 2020 Mar TI - A patient-centered approach to the burden of symptoms in patients with scleroderma treated with Bosentan: A prospective single-center observational study. PG - 1739-1746 LID - 10.3892/etm.2019.8361 [doi] AB - Systemic sclerosis (SSc) is a rare and complex autoimmune disease associated with poor vital and functional outcomes. The functional hindrance in patients derives from various disease-specific manifestations, including Raynaud's phenomenon and digital ulcers (DUs). Bosentan is an endothelin receptor antagonist capable of preventing the appearance of new DUs in patients with scleroderma. The present study aimed to evaluate the effects of Bosentan on the severity of Raynaud's phenomenon, DU-related symptoms and functional impairment during the first year of treatment. A prospective study that included adult patients with SSc admitted to the Rheumatology Department between January 2016 and January 2017 that were candidates for Bosentan therapy, was performed. All patients were asked to evaluate the burden of symptoms secondary to Raynaud's and DUs using a visual analogue scale (VAS), whereas functional hindrance was assessed via Health Assessment Questionnaire Disability Index (HAQ-DI). The outcomes were assessed at baseline and every 3 months during 1 year of therapy. Among the 41 patients included initially, 2 participants discontinued the treatment after 1 month due to adverse events (elevation of liver enzymes). The study cohort exhibited a significant improvement in HAQ-DI, VAS-R and VAS-DU scores in response to Bosentan therapy over the 1-year follow-up period. Higher scores at baseline predicted a weaker treatment-related improvement, with the risk of a poor outcome being increased by 220% for VAS-R, 116% for VAS-DU, whereas no increase was observed for HAQ-DI. The post-treatment improvement in VAS-DU levels was associated with a better outcome for HAQ-DI (R=0.44; P=0.005). This association was not identified for VAS-R (R=0.24; P=0.137). Throughout the follow-up period, patients with dyspnea presented with significantly higher HAQ-DI scores compared with non-dyspneic patients. Bosentan therapy may indirectly influence functionality and quality of life in patients with scleroderma by reducing the burden of Raynaud's and DU-related symptoms. Nonetheless, patients with SSc with a decreased symptom burden at baseline exhibited improved outcomes. CI - Copyright: © Rezus et al. FAU - Rezus, Elena AU - Rezus E AD - Department of Rheumatology and Physiotherapy, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Burlui, Alexandra Maria AU - Burlui AM AD - Department of Rheumatology and Physiotherapy, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Gafton, Bogdan AU - Gafton B AD - Department of Medical Oncology-Radiotherapy, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Stratulat, Teodora Alexa AU - Stratulat TA AD - Department of Medical Oncology-Radiotherapy, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Zota, Gabriela Rusu AU - Zota GR AD - Department of Pharmacology, Clinical Pharmacology and Algesiology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Cardoneanu, Anca AU - Cardoneanu A AD - Department of Rheumatology and Physiotherapy, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Rezus, Ciprian AU - Rezus C AD - Department of Internal Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania. LA - eng PT - Journal Article DEP - 20191220 PL - Greece TA - Exp Ther Med JT - Experimental and therapeutic medicine JID - 101531947 PMC - PMC7027142 OTO - NOTNLM OT - Raynaud's symptoms OT - bosentan OT - dyspnea OT - microvascular changes OT - systemic sclerosis EDAT- 2020/02/28 06:00 MHDA- 2020/02/28 06:01 PMCR- 2019/12/20 CRDT- 2020/02/28 06:00 PHST- 2019/05/24 00:00 [received] PHST- 2019/11/12 00:00 [accepted] PHST- 2020/02/28 06:00 [entrez] PHST- 2020/02/28 06:00 [pubmed] PHST- 2020/02/28 06:01 [medline] PHST- 2019/12/20 00:00 [pmc-release] AID - ETM-0-0-8361 [pii] AID - 10.3892/etm.2019.8361 [doi] PST - ppublish SO - Exp Ther Med. 2020 Mar;19(3):1739-1746. doi: 10.3892/etm.2019.8361. Epub 2019 Dec 20. PMID- 29392863 OWN - NLM STAT- MEDLINE DCOM- 20180919 LR - 20260127 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 21 IP - 4 DP - 2018 Apr TI - Stem cell transplant in systemic sclerosis: An Indian experience. PG - 859-865 LID - 10.1111/1756-185X.13262 [doi] AB - AIM: To prospectively evaluate long term outcomes in a cohort of patients with Systemic sclerosis treated with Hematopoietic stem cell transplant (HSCT). METHOD: This is a prospective observational study of four SSc patients who underwent HSCT at a tertiary care center in India between 2008-2012. The selection criteria included young individuals with rapidly progressive disease and at least one major organ involvement. We used granulocyte colony-stimulating factor for peripheral blood stem cell mobilization, pre-transplant conditioning with fludarabine, cyclophosphamide and rabbit anti-thymocyte globulin followed by re-infusion of autologous stem cells as per standard institute protocol. RESULTS: A total of four patients (one male and three females) underwent autologous HSCT for SSc. Patients had heterogeneous disease manifestations including severe Raynaud's phenomenon with vasculopathic ulcers, gastrointestinal problems and mild interstitial lung disease (ILD). Patients were followed up for a mean duration of 7 years. There was significant sustained improvement in skin score, vasculopathy and gastrointestinal manifestations. Interstitial lung disease did not show any deterioration. The quality of life indices showed remarkable improvement in all subjects. No complications related to transplant were noted. CONCLUSION: In absence of an effective pharmacotherapy for SSc, autologous HSCT has a huge potential in management of cutaneous and internal organ manifestations. CI - © 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Nair, Velu AU - Nair V AD - Internal Medicine & Haematology, Armed Forces Medical College, Pune, India. FAU - Vasdev, Vivek AU - Vasdev V AUID- ORCID: 0000-0001-5001-189X AD - Rheumatology & Clinical Immunology, Army Hospital R & R, New Delhi, India. FAU - Kumar, Abhishek AU - Kumar A AUID- ORCID: 0000-0002-7883-5118 AD - Rheumatology & Clinical Immunology, Army Hospital R & R, New Delhi, India. FAU - Shankar, Subramanian AU - Shankar S AD - Rheumatology, Command Hospital (Air Force), Bengaluru, India. FAU - Nair, Vivek AU - Nair V AD - Dermatology, Maulana Azad Medical College, New Delhi, India. FAU - Sharma, Ajay AU - Sharma A AD - Rheumatology & Clinical Immunology, Army Hospital R & R, New Delhi, India. LA - eng PT - Journal Article PT - Observational Study DEP - 20180202 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Immunosuppressive Agents) RN - 0 (Myeloablative Agonists) RN - 143011-72-7 (Granulocyte Colony-Stimulating Factor) SB - IM MH - Adult MH - Female MH - Granulocyte Colony-Stimulating Factor/therapeutic use MH - Hematopoietic Stem Cell Mobilization MH - *Hematopoietic Stem Cell Transplantation/adverse effects/methods MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - India MH - Male MH - Myeloablative Agonists/therapeutic use MH - Prospective Studies MH - Quality of Life MH - Remission Induction MH - Scleroderma, Systemic/diagnosis/*surgery MH - Tertiary Care Centers MH - Time Factors MH - Transplantation Conditioning MH - Transplantation, Autologous MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - India OT - Raynaud's OT - scleroderma OT - stem cell transplant OT - systemic sclerosis EDAT- 2018/02/03 06:00 MHDA- 2018/09/20 06:00 CRDT- 2018/02/03 06:00 PHST- 2018/02/03 06:00 [pubmed] PHST- 2018/09/20 06:00 [medline] PHST- 2018/02/03 06:00 [entrez] AID - 10.1111/1756-185X.13262 [doi] PST - ppublish SO - Int J Rheum Dis. 2018 Apr;21(4):859-865. doi: 10.1111/1756-185X.13262. Epub 2018 Feb 2. PMID- 35339493 OWN - NLM STAT- MEDLINE DCOM- 20220603 LR - 20221221 IS - 1095-9319 (Electronic) IS - 0026-2862 (Print) IS - 0026-2862 (Linking) VI - 142 DP - 2022 Jul TI - Detailed videocapillaroscopic microvascular changes detectable in adult COVID-19 survivors. PG - 104361 LID - S0026-2862(22)00051-6 [pii] LID - 10.1016/j.mvr.2022.104361 [doi] AB - OBJECTIVE: COVID-19 is a multisystem disease that causes endothelial dysfunction and organ damage. Aim of the study was to evaluate the microvascular status in COVID-19 survivors with past different disease severity, in comparison with age and sex-matched primary Raynaud's phenomenon (PRP) patients and control subjects (CNT), including possible effects of concomitant therapies. METHODS: Sixty-one COVID-19 survivors (mean age 58 ± 13 years, mean days from disease onset 126 ± 53 and mean days from recovery 104 ± 53), thirty-one PRP patients (mean age 59 ± 15 years, mean disease duration 11 ± 10 years) and thirty CNT (mean age 58 ± 13 years) underwent nailfold videocapillaroscopy (NVC) examination. The following capillaroscopic parameters were searched and scored (0-3): dilated capillaries, giant capillaries, isolated microhemorrhages, capillary ramifications (angiogenesis) and capillary number, including absolute capillary number per linear millimeter at the nailfold bed. RESULTS: The mean nailfold capillary number per linear millimeter was significantly lower in COVID-19 survivors when compared with PRP patients and CNT (univariate and multivariate analysis p < 0.001). On the contrary, COVID-19 survivors showed significantly less isolated microhemorrhages than PRP patients and CNT (univariate and multivariate analysis, p = 0.005 and p = 0.012, respectively). No statistically significant difference was observed between COVID-19 survivors and control groups concerning the frequency of dilated capillaries and capillary ramifications. COVID-19 selective therapies showed a promising trend on preserving capillary loss and deserving further investigations. CONCLUSIONS: SARS-CoV-2 seems to mainly induce a significant loss of capillaries in COVID-19 survivors at detailed NVC analysis in comparison to controls. The presence of a significant reduced score for isolated microhaemorrhages in COVID-19 survivors deserves further analysis. CI - Copyright © 2022 Elsevier Inc. All rights reserved. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. Electronic address: albertosulli@unige.it. FAU - Gotelli, Emanuele AU - Gotelli E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. FAU - Bica, Pietro Francesco AU - Bica PF AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. FAU - Schiavetti, Irene AU - Schiavetti I AD - Department of Health Sciences, University of Genova, Genova, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. Electronic address: carmen.pizzorni@unige.it. FAU - Aloè, Teresita AU - Aloè T AD - Interventional Pneumology Unit, IRCCS San Martino Polyclinic Hospital, Genova, Italy. FAU - Grosso, Marco AU - Grosso M AD - Interventional Pneumology Unit, IRCCS San Martino Polyclinic Hospital, Genova, Italy. FAU - Barisione, Emanuela AU - Barisione E AD - Interventional Pneumology Unit, IRCCS San Martino Polyclinic Hospital, Genova, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. Electronic address: sabrina.paolino@unige.it. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium; Department of Internal Medicine, Ghent University Hospital, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, Vlaams Instituut voor Biotechnologie (VIB), Inflammation Research Center (IRC), Ghent, Belgium. Electronic address: vanessa.smith@ugent.be. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy. Electronic address: mcutolo@unige.it. LA - eng PT - Journal Article DEP - 20220324 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - *COVID-19/diagnosis MH - Capillaries MH - Humans MH - Microscopic Angioscopy MH - Middle Aged MH - *Nails/blood supply MH - SARS-CoV-2 MH - Survivors PMC - PMC8942583 OTO - NOTNLM OT - COVID-19 OT - Capillaries OT - Endothelial cells OT - Glucocorticoids OT - Microcirculation OT - Nailfold capillaroscopy OT - Raynaud's phenomenon OT - SARS-CoV-2 COIS- All Authors declare no competing interests concerning this manuscript. EDAT- 2022/03/28 06:00 MHDA- 2022/06/07 06:00 PMCR- 2022/03/24 CRDT- 2022/03/27 20:20 PHST- 2022/01/03 00:00 [received] PHST- 2022/03/08 00:00 [revised] PHST- 2022/03/14 00:00 [accepted] PHST- 2022/03/28 06:00 [pubmed] PHST- 2022/06/07 06:00 [medline] PHST- 2022/03/27 20:20 [entrez] PHST- 2022/03/24 00:00 [pmc-release] AID - S0026-2862(22)00051-6 [pii] AID - 104361 [pii] AID - 10.1016/j.mvr.2022.104361 [doi] PST - ppublish SO - Microvasc Res. 2022 Jul;142:104361. doi: 10.1016/j.mvr.2022.104361. Epub 2022 Mar 24. PMID- 33505743 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210130 IS - 2090-6641 (Print) IS - 2090-665X (Electronic) IS - 2090-665X (Linking) VI - 2021 DP - 2021 TI - Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors. PG - 8862405 LID - 10.1155/2021/8862405 [doi] LID - 8862405 AB - Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. We present a case of MCTD with chronic features of Raynaud's phenomenon, dermatomyositis, and thrombocytopenia complicated with acute SRC which showed a great response to ACE inhibitors. Here, we advise the early and aggressive use of ACE inhibitors as soon as SRC is suspected. CI - Copyright © 2021 Jomana Madieh et al. FAU - Madieh, Jomana AU - Madieh J AD - Department of Internal Medicine, Faculty of Medicine, Al-Quds University, Abu Dis, State of Palestine. FAU - Khamayseh, Iman AU - Khamayseh I AD - Department of Internal Medicine, Faculty of Medicine, Al-Quds University, Abu Dis, State of Palestine. FAU - Hrizat, Alaa AU - Hrizat A AD - Department of Internal Medicine, Faculty of Medicine, Al-Quds University, Abu Dis, State of Palestine. FAU - Hamadah, Abdurrahman AU - Hamadah A AD - Department of Internal Medicine, Faculty of Medicine, Hashemite University, Zarqa, Jordan. FAU - Gharaibeh, Kamel AU - Gharaibeh K AUID- ORCID: 0000-0002-9600-9901 AD - Department of Internal Medicine, Faculty of Medicine, Al-Quds University, Abu Dis, State of Palestine. LA - eng PT - Case Reports PT - Journal Article DEP - 20210106 PL - United States TA - Case Rep Nephrol JT - Case reports in nephrology JID - 101598418 PMC - PMC7808802 COIS- The authors declare that they have no conflicts of interest. EDAT- 2021/01/29 06:00 MHDA- 2021/01/29 06:01 PMCR- 2021/01/06 CRDT- 2021/01/28 05:43 PHST- 2020/09/08 00:00 [received] PHST- 2020/12/10 00:00 [revised] PHST- 2020/12/23 00:00 [accepted] PHST- 2021/01/28 05:43 [entrez] PHST- 2021/01/29 06:00 [pubmed] PHST- 2021/01/29 06:01 [medline] PHST- 2021/01/06 00:00 [pmc-release] AID - 10.1155/2021/8862405 [doi] PST - epublish SO - Case Rep Nephrol. 2021 Jan 6;2021:8862405. doi: 10.1155/2021/8862405. eCollection 2021. PMID- 23888582 OWN - NLM STAT- MEDLINE DCOM- 20130827 LR - 20150826 IS - 0370-629X (Print) IS - 0370-629X (Linking) VI - 68 IP - 5-6 DP - 2013 May-Jun TI - [Risking the skin for some joints]. PG - 311-4 AB - Marijuana, cannabis, hemp designate some plants and their extracts enriched in cannabinoids including change 9-tetrahydrocannabinol (THC). This soft drug exerts psychoactive effects and is responsible for adverse events appearing on the skin, mucosae and eyes. Contact allergic urticaria possibly occurs as well as Raynaud's phenomenon and arteritis resembling Buerger's disease. Glossitis and atrophic stomatitis may be associated with paronditis and uvular angioedema. FAU - Hermanns-Lé, T AU - Hermanns-Lé T AD - (1) Maître de Conférence, Consultant Expert clinique, Service de Dermatopathologie, CHU de Liege, BElgique. FAU - Delvenne, P AU - Delvenne P FAU - Piérard, G E AU - Piérard GE FAU - Rousseau, A F AU - Rousseau AF FAU - Piérard-franchimont, C AU - Piérard-franchimont C LA - fre PT - English Abstract PT - Journal Article TT - Risquer sa peau pour quelques ((joints)). PL - Belgium TA - Rev Med Liege JT - Revue medicale de Liege JID - 0404317 SB - IM MH - Arteritis/*chemically induced MH - Cannabis/*adverse effects MH - Humans MH - Marijuana Smoking/*adverse effects MH - Urticaria/*chemically induced EDAT- 2013/07/31 06:00 MHDA- 2013/08/28 06:00 CRDT- 2013/07/30 06:00 PHST- 2013/07/30 06:00 [entrez] PHST- 2013/07/31 06:00 [pubmed] PHST- 2013/08/28 06:00 [medline] PST - ppublish SO - Rev Med Liege. 2013 May-Jun;68(5-6):311-4. PMID- 33022722 OWN - NLM STAT- MEDLINE DCOM- 20210325 LR - 20210325 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 145 IP - 20 DP - 2020 Oct TI - [Upper extremity peripheral arterial disease]. PG - 1437-1442 LID - 10.1055/a-1068-4230 [doi] AB - Peripheral arterial disease (PAD) of the upper extremity is much less frequent and aetiologically more heterogeneous than lower extremity PAD. The clinical approach to patients with upper extremity PAD must consider a range of distinctive features regarding symptoms, physical findings and diagnostic strategies. This review focusses on these specific characteristics of upper extremity PAD and the new developments in this field. Arteriosclerotic subclavian artery obstruction, large vessel vasculitis, thoracic outlet syndrome and secondary Raynaud's phenomenon are four pivotal causes and manifestations of upper extremity PAD. These four entities are exemplarily discussed. CI - Thieme. All rights reserved. FAU - Tatò, Federico AU - Tatò F AD - Gefäßpraxis im Tal. LA - ger PT - Journal Article PT - Review TT - Durchblutungsstörungen der oberen Extremitäten. DEP - 20201006 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Angiography, Digital Subtraction MH - Humans MH - *Peripheral Arterial Disease MH - *Upper Extremity/blood supply/diagnostic imaging/physiopathology/surgery COIS- Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht. EDAT- 2020/10/07 06:00 MHDA- 2021/03/26 06:00 CRDT- 2020/10/06 20:18 PHST- 2020/10/06 20:18 [entrez] PHST- 2020/10/07 06:00 [pubmed] PHST- 2021/03/26 06:00 [medline] AID - 10.1055/a-1068-4230 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2020 Oct;145(20):1437-1442. doi: 10.1055/a-1068-4230. Epub 2020 Oct 6. PMID- 22798974 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20240318 IS - 2689-9175 (Electronic) IS - 1941-2789 (Print) IS - 1941-2789 (Linking) VI - 5 IP - 7 DP - 2012 Jul TI - An Update on the Treatment of the Cutaneous Manifestations of Systemic Sclerosis: The Dermatologist's Point of View. PG - 33-43 AB - Systemic sclerosis is a connective tissue disorder that affects multiple organs. Although the initial symptoms of the disease are vascular, skin involvement is almost universally present in patients with systemic sclerosis. The presence of Raynaud's phenomenon, progressive thickening of the skin, digital ulcers, and calcinosis all correlate proportionally with disease severity. Since no treatment is available to completely prevent the natural course of the disease, emphasis is often placed on managing symptoms and complications. In this review, the authors focus on the management of each one of the skin manifestations seen in systemic sclerosis, as the dermatologist may facilitate the early recognition and treatment of these complications. FAU - Vitiello, Magalys AU - Vitiello M FAU - Abuchar, Adriana AU - Abuchar A FAU - Santana, Néstor AU - Santana N FAU - Dehesa, Luis AU - Dehesa L FAU - Kerdel, Francisco A AU - Kerdel FA LA - eng PT - Journal Article PL - United States TA - J Clin Aesthet Dermatol JT - The Journal of clinical and aesthetic dermatology JID - 101518173 PMC - PMC3396456 COIS- DISCLOSURE:Dr. Kerdel has received grants, participated in advisory boards and is a speaker for Abbott, Amgen, Wyeth, Astellas, Centocor, Genentech, Stiefel, Eisai, Pfizer, Celgene, Merck, and Novartis. Drs. Vitiello, Abuchar, Santana, and Dehesa report no relevant conflicts of interest. EDAT- 2012/07/17 06:00 MHDA- 2012/07/17 06:01 PMCR- 2012/07/01 CRDT- 2012/07/17 06:00 PHST- 2012/07/17 06:00 [entrez] PHST- 2012/07/17 06:00 [pubmed] PHST- 2012/07/17 06:01 [medline] PHST- 2012/07/01 00:00 [pmc-release] PST - ppublish SO - J Clin Aesthet Dermatol. 2012 Jul;5(7):33-43. PMID- 29123575 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1895-5770 (Print) IS - 1897-4317 (Electronic) IS - 1895-5770 (Linking) VI - 12 IP - 3 DP - 2017 TI - Systemic sclerosis and the gastrointestinal tract. PG - 163-168 LID - 10.5114/pg.2017.70467 [doi] AB - Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. The disease results in heterogeneous clinical findings and different courses. Systemic sclerosis usually begins with the onset of Raynaud's phenomenon (RP), followed by skin sclerosis and internal organ involvement, although it may appear synchronously with RP. Gastrointestinal involvement is a serious and prevalent complication of SSc, and the oesophagus is the most frequently affected organ. Both limited and diffuse cutaneous SSc involve internal organs, with the involvement of the gastrointestinal tract as a leading cause of morbidity. At present, treatment is mainly symptomatic with no disease-modifying drugs. FAU - Walecka, Irena AU - Walecka I AD - Clinical Department of Dermatology, Central Clinical Hospital of the MSWiA, Warsaw, Poland. LA - eng PT - Journal Article PT - Review DEP - 20170930 PL - Poland TA - Prz Gastroenterol JT - Przeglad gastroenterologiczny JID - 101280380 PMC - PMC5672703 OTO - NOTNLM OT - autoantibodies OT - gastrointestinal involvement OT - systemic sclerosis EDAT- 2017/11/11 06:00 MHDA- 2017/11/11 06:01 PMCR- 2017/01/01 CRDT- 2017/11/11 06:00 PHST- 2017/04/25 00:00 [received] PHST- 2017/06/01 00:00 [accepted] PHST- 2017/11/11 06:00 [entrez] PHST- 2017/11/11 06:00 [pubmed] PHST- 2017/11/11 06:01 [medline] PHST- 2017/01/01 00:00 [pmc-release] AID - 30723 [pii] AID - 10.5114/pg.2017.70467 [doi] PST - ppublish SO - Prz Gastroenterol. 2017;12(3):163-168. doi: 10.5114/pg.2017.70467. Epub 2017 Sep 30. PMID- 17474176 OWN - NLM STAT- MEDLINE DCOM- 20070628 LR - 20070502 IS - 1427-440X (Print) IS - 1427-440X (Linking) VI - 52 Suppl 2 DP - 2006 TI - [A review of paraneoplastic rheumatic syndromes]. PG - 17-22 AB - Malignant neoplasms are sometimes associated with a variety of paraneoplastic rheumatic syndromes. The most frequently diagnosed ones include hypertrophic osteoarthropathy, polyarthritis, dermatomyositis/polymyositis, and paraneoplastic vasculitis. Fasciitis, panniculitis, erythema nodosum, lupus-like syndrome, and Raynaud's syndrome are rare. Rheumatic manifestations of cancer are sometimes indistinguishable from idiopathic rheumatic disease. Hence, the neoplasm is not discovered because it is masked by another disease implicating improper therapeutic decisions and worsening the prognosis. The aim of this work was to review the literature on paraneoplastic rheumatic syndromes. FAU - Dabrowska-Zimoń, Agnieszka AU - Dabrowska-Zimoń A AD - Klinika Reumatologii Pomorskiej Akademii Medycznej ul. Unii Lubelskiej 1, 71-252 Szczecin. FAU - Brzosko, Marek AU - Brzosko M LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Reumatologiczne zespoły paranowotworowe--przeglad literatury. PL - Poland TA - Ann Acad Med Stetin JT - Annales Academiae Medicae Stetinensis JID - 7506854 SB - IM MH - Causality MH - Comorbidity MH - Dermatomyositis/epidemiology MH - Erythema Nodosum/epidemiology MH - Humans MH - Paraneoplastic Syndromes/*diagnosis/*epidemiology MH - Polymyositis/epidemiology MH - Rheumatic Diseases/*epidemiology MH - Synovitis/epidemiology MH - Vasculitis/epidemiology RF - 58 EDAT- 2007/05/03 09:00 MHDA- 2007/06/29 09:00 CRDT- 2007/05/03 09:00 PHST- 2007/05/03 09:00 [pubmed] PHST- 2007/06/29 09:00 [medline] PHST- 2007/05/03 09:00 [entrez] PST - ppublish SO - Ann Acad Med Stetin. 2006;52 Suppl 2:17-22. PMID- 34079662 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210605 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 4 DP - 2021 Apr 19 TI - Successful Spinal Cord Stimulation for Necrotizing Raynaud's Phenomenon in COVID-19 Affected Patient: The Nightmare Comes Back. PG - e14569 LID - 10.7759/cureus.14569 [doi] LID - e14569 AB - Necrotizing Raynaud's phenomenon is a vascular clinical syndrome characterized by vasospasm of distal resistance vessels, usually triggered by cold temperatures or by psychological conditions such as anxiety and stress. Pain is the first reported symptom, related to insufficient oxygen delivery to the extremities that leads to ischemia of the peripheral tissues. The initial treatment is conservative, but if the symptoms persist, necrosis and distal amputation can occur. In selected patients, neuromodulation with spinal cord stimulation (SCS) can be an effective treatment by reducing pain and amputation rate. Recent evidence suggests that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can cause endotheliopathy with microvascular and macrovascular thrombotic events and can present as a systemic inflammatory vascular disease. We present a case of a severe necrotizing Raynaud's phenomenon successfully treated and controlled with SCS that abruptly reappeared during SARS-CoV-2 infection. The report of this case is suggestive for potential treatment in case of peripheral ischemia consequent to COVID-19 vasculopathy. The interaction between SCS and SARS-CoV-2-related endotheliopathy is unknown and would deserve further studies. CI - Copyright © 2021, Giglio et al. FAU - Giglio, Mariateresa AU - Giglio M AD - Department of Anaesthesiology, University of Bari Aldo Moro, Bari, ITA. FAU - Preziosa, Angela AU - Preziosa A AD - Department of Anesthesiology, University of Bari Aldo Moro, Bari, ITA. FAU - Rekatsina, Martina AU - Rekatsina M AD - Pain Management, Whipps Cross Hospital Barts Health National Health Service (NHS), London, GBR. FAU - Viswanath, Omar AU - Viswanath O AD - Department of Anesthesiology, University of Arizona, Phoenix, USA. FAU - Urits, Ivan AU - Urits I AD - Department of Anesthesia, Critical Care and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, USA. FAU - Varrassi, Giustino AU - Varrassi G AD - Department of Research and Development, Paolo Procacci Foundation, Roma, ITA. FAU - Paladini, Antonella AU - Paladini A AD - Dipartimento di Medicina clinica, sanità pubblica, scienze della vita e dell'ambiente, University of L'Aquila, L'Aquila, ITA. FAU - Puntillo, Filomena AU - Puntillo F AD - Department of Intedisciplinary Medicine, University of Bari Aldo Moro, Bari, ITA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210419 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8159305 OTO - NOTNLM OT - covid-19 OT - pain OT - raynaud phenomenon OT - spinal cord stimulation OT - vascular pain COIS- The authors have declared that no competing interests exist. EDAT- 2021/06/04 06:00 MHDA- 2021/06/04 06:01 PMCR- 2021/04/19 CRDT- 2021/06/03 06:42 PHST- 2021/06/03 06:42 [entrez] PHST- 2021/06/04 06:00 [pubmed] PHST- 2021/06/04 06:01 [medline] PHST- 2021/04/19 00:00 [pmc-release] AID - 10.7759/cureus.14569 [doi] PST - epublish SO - Cureus. 2021 Apr 19;13(4):e14569. doi: 10.7759/cureus.14569. PMID- 21558747 OWN - NLM STAT- MEDLINE DCOM- 20121011 LR - 20200928 IS - 0446-6586 (Print) IS - 0446-6586 (Linking) VI - 108 IP - 5 DP - 2011 May TI - [A case of superior mesenteric artery occlusion associated with idiopathic chronic cold agglutinin disease]. PG - 791-8 AB - A 64-year-old man who had been given a diagnosis of idiopathic chronic cold agglutinin disease in a medical clinic suffered from Raynaud's phenomenon and acrocyanosis in winter. He was admitted to our hospital with unbearable abdominal pain. Blood tests showed liver dysfunction with jaundice and severe acidosis. Abdominal angiogram and contrast-enhanced CT revealed superior mesenteric artery occlusion. These findings suggest that thrombosis due to cold agglutinin disease could be the cause of superior mesenteric artery occlusion. FAU - Hirono, Haruka AU - Hirono H AD - Department of Internal Medicine, Sado General Hospital. haruka@ngt.ndu.ac.jp FAU - Kubota, Tomoyuki AU - Kubota T FAU - Funakoshi, Kazuhiro AU - Funakoshi K FAU - Watanabe, Takuya AU - Watanabe T FAU - Hasegawa, Katsuhiko AU - Hasegawa K FAU - Soga, Kenji AU - Soga K FAU - Shibasaki, Koichi AU - Shibasaki K LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PT - Review PL - Japan TA - Nihon Shokakibyo Gakkai Zasshi JT - Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology JID - 2984683R SB - IM MH - Anemia, Hemolytic, Autoimmune/*complications MH - Chronic Disease MH - Humans MH - Male MH - Mesenteric Artery, Superior MH - Mesenteric Vascular Occlusion/*etiology MH - Middle Aged EDAT- 2011/05/12 06:00 MHDA- 2012/10/12 06:00 CRDT- 2011/05/12 06:00 PHST- 2011/05/12 06:00 [entrez] PHST- 2011/05/12 06:00 [pubmed] PHST- 2012/10/12 06:00 [medline] AID - JST.JSTAGE/nisshoshi/108.791 [pii] PST - ppublish SO - Nihon Shokakibyo Gakkai Zasshi. 2011 May;108(5):791-8. PMID- 16010443 OWN - NLM STAT- MEDLINE DCOM- 20060818 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 2 DP - 2006 Mar TI - Malignant thymoma associated with mixed connective tissue disease: a case report. PG - 262-4 AB - Many autoimmune diseases have been reported to be associated with malignancy. Mixed connective tissue disease (MCTD), however, has rarely been associated with malignancy. Thymoma is one of the neoplasms often reported to be related to various immunological disorders. Among the types of thymoma defined by WHO, malignant thymoma (thymoma type C) is the one least reported to be associated with autoimmune disease. Here, we report a case of malignant thymoma with concurrent MCTD, which manifested with acrosclerosis, Raynaud's phenomenon, arthritis (synovitis), and a high titer of anti-ribonucleoprotein antibody. FAU - Lin, Yu-Chih AU - Lin YC AD - Department of Internal Medicine, Rheumatology Unit, Kaohsiung Medical University Hospital, Kaohsiung City 807, Taiwan, ROC. FAU - Wu, Chen-Ching AU - Wu CC FAU - Ou, Tsan-Teng AU - Ou TT FAU - Yen, Jeng-Hsien AU - Yen JH FAU - Liu, Hong-Wen AU - Liu HW FAU - Tsai, Wen-Chan AU - Tsai WC LA - eng PT - Case Reports PT - Journal Article DEP - 20050712 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Female MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications MH - Thymoma/*complications MH - Thymus Neoplasms/*complications EDAT- 2005/07/13 09:00 MHDA- 2006/08/19 09:00 CRDT- 2005/07/13 09:00 PHST- 2004/10/25 00:00 [received] PHST- 2005/01/06 00:00 [accepted] PHST- 2004/01/06 00:00 [revised] PHST- 2005/07/13 09:00 [pubmed] PHST- 2006/08/19 09:00 [medline] PHST- 2005/07/13 09:00 [entrez] AID - 10.1007/s10067-005-1159-9 [doi] PST - ppublish SO - Clin Rheumatol. 2006 Mar;25(2):262-4. doi: 10.1007/s10067-005-1159-9. Epub 2005 Jul 12. PMID- 33788193 OWN - NLM STAT- MEDLINE DCOM- 20210402 LR - 20210402 IS - 0065-2598 (Print) IS - 0065-2598 (Linking) VI - 1303 DP - 2021 TI - Systemic Sclerosis and Pulmonary Disease. PG - 173-182 LID - 10.1007/978-3-030-63046-1_10 [doi] AB - Systemic sclerosis is a complex, often progressive, multisystem autoimmune disease. It is commonly categorized into limited cutaneous or diffuse cutaneous systemic sclerosis. There is near universal involvement of skin fibrosis and gastrointestinal dysfunction, but lung disease is not only common but also a most serious complication. Severe lung disease is the top cause of mortality, displacing scleroderma renal crisis as the leading cause of death. Whether there is limited cutaneous or diffuse cutaneous manifestations can be predictive of what type of lung disease that can present in the patient. Limited cutaneous systemic sclerosis patients tend to have pulmonary hypertension whereas diffuse cutaneous systemic sclerosis patients tend to have interstitial lung disease. There are more rare phenotypes associated with antibodies Th/To and U3RNP that can have both pulmonary hypertension and interstitial lung disease concomitantly. There are inherent challenges in the management for both pulmonary hypertension and interstitial lung disease but with the focus on early diagnosis for each of these lung complications, treatment may have a higher chance of efficacy. FAU - Ngo, Khoa AU - Ngo K AD - Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, NY, USA. ngok@amc.edu. LA - eng PT - Journal Article PL - United States TA - Adv Exp Med Biol JT - Advances in experimental medicine and biology JID - 0121103 SB - IM MH - Humans MH - *Hypertension, Pulmonary/etiology MH - *Lung Diseases, Interstitial/diagnosis/etiology MH - *Scleroderma, Systemic/complications/diagnosis MH - Skin MH - *Skin Diseases OTO - NOTNLM OT - American College of Rheumatology OT - Autologous hematopoietic stem cell transplantation OT - Cyclophosphamide OT - Diffuse cutaneous systemic sclerosis OT - European League Against Rheumatism OT - Fibrosis OT - Interstitial lung disease OT - Limited cutaneous systemic sclerosis OT - Mycophenolate mofetil OT - Pulmonary arterial hypertension OT - Raynaud’s phenomenon OT - Scleroderma Lung Study OT - Vasculopathy EDAT- 2021/04/01 06:00 MHDA- 2021/04/07 06:00 CRDT- 2021/03/31 13:31 PHST- 2021/03/31 13:31 [entrez] PHST- 2021/04/01 06:00 [pubmed] PHST- 2021/04/07 06:00 [medline] AID - 10.1007/978-3-030-63046-1_10 [doi] PST - ppublish SO - Adv Exp Med Biol. 2021;1303:173-182. doi: 10.1007/978-3-030-63046-1_10. PMID- 21794549 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20121031 IS - 1699-258X (Print) IS - 1699-258X (Linking) VI - 4 Suppl 1 DP - 2008 Mar TI - [Sjögren's Syndrome]. PG - 22-7 LID - 10.1016/S1699-258X(08)76135-7 [doi] AB - Sjögren's syndrome is a systemic autoimmune disease that is characterized by the presence of keratoconjunctivitis sicca, xerostomy and a large spectrum of signs and symptoms that translate into a very heterogeneous disease. The mild form that affects mucosal tissues is the most frequent, but there are more severe and active patterns, manifested by the presence of extraglandular affection with a worse prognosis. The clinical spectrum includes anything from mucosal alterations, Raynaud's phenomenon, parotid enlargement or arthritis, but can be aggravated by the presence of neurological, lung or renal affection. Initial therapy includes topical treatment with artificial tears, nocturnal cream and drugs that stimulate secretion for important glandular affection, while severe systemic affection merits immunosuppressant therapy. There has been recent evidence that biologic therapy is useful for the treatment of severe and resistant cases. CI - Copyright © 2008 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved. FAU - Corominas, Hèctor AU - Corominas H AD - Unitat de Reumatologia. Hospital Dos de Maig. Barcelona. España. FAU - Fíguls, Ramon AU - Fíguls R FAU - Riera, Manel AU - Riera M LA - spa PT - English Abstract PT - Journal Article TT - Síndrome de Sjögren. DEP - 20090203 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 EDAT- 2008/03/01 00:00 MHDA- 2008/03/01 00:01 CRDT- 2011/07/29 06:00 PHST- 2011/07/29 06:00 [entrez] PHST- 2008/03/01 00:00 [pubmed] PHST- 2008/03/01 00:01 [medline] AID - S1699-258X(08)76135-7 [pii] AID - 10.1016/S1699-258X(08)76135-7 [doi] PST - ppublish SO - Reumatol Clin. 2008 Mar;4 Suppl 1:22-7. doi: 10.1016/S1699-258X(08)76135-7. Epub 2009 Feb 3. PMID- 41018143 OWN - NLM STAT- Publisher LR - 20260417 IS - 1753-495X (Print) IS - 1753-4968 (Electronic) IS - 1753-495X (Linking) DP - 2025 Sep 25 TI - A case of antisynthetase syndrome with interstitial lung disease in pregnancy. PG - 1753495X251380063 LID - 10.1177/1753495X251380063 [doi] LID - 1753495X251380063 AB - Antisynthetase syndrome (anti-SS) is an autoimmune condition characterised by autoantibodies against aminoacyl tRNA synthetases. Clinical features can include interstitial lung disease (ILD), myositis, Raynaud's phenomenon and arthritis. Described here is a 25-year-old pregnant woman with anti-SS with worsening ILD in the latter part of pregnancy, requiring emergency delivery at 36 weeks and 3 days of gestation. CI - © The Author(s) 2025. FAU - Park, Esther AU - Park E AUID- ORCID: 0000-0003-3954-9001 AD - The de Swiet Obstetric Medicine Centre, Centre for Fetal Care, Queen Charlotte's and Chelsea Hospital, London, UK. FAU - Munro, Kerry AU - Munro K AD - Imperial College Healthcare NHS Trust, UK. RINGGOLD: 8946 FAU - Wickremasinghe, Melissa AU - Wickremasinghe M AD - Imperial College Healthcare NHS Trust, UK. RINGGOLD: 8946 FAU - Frise, Charlotte Jane AU - Frise CJ AUID- ORCID: 0000-0002-7650-5780 AD - Imperial College Healthcare NHS Trust, UK. RINGGOLD: 8946 LA - eng PT - Journal Article DEP - 20250925 PL - England TA - Obstet Med JT - Obstetric medicine JID - 101464191 PMC - PMC12463919 OTO - NOTNLM OT - Antisynthetase syndrome OT - interstitial lung disease OT - pregnancy COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/09/29 13:19 MHDA- 2025/09/29 13:19 PMCR- 2026/09/25 CRDT- 2025/09/29 06:40 PHST- 2025/03/20 00:00 [received] PHST- 2025/09/02 00:00 [accepted] PHST- 2026/09/25 00:00 [pmc-release] PHST- 2025/09/29 13:19 [medline] PHST- 2025/09/29 13:19 [pubmed] PHST- 2025/09/29 06:40 [entrez] AID - 10.1177_1753495X251380063 [pii] AID - 10.1177/1753495X251380063 [doi] PST - aheadofprint SO - Obstet Med. 2025 Sep 25:1753495X251380063. doi: 10.1177/1753495X251380063. PMID- 22958322 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20211021 IS - 2049-6958 (Electronic) IS - 1828-695X (Print) IS - 1828-695X (Linking) VI - 6 IP - 3 DP - 2011 Aug 31 TI - Rituximab treatment in a case of antisynthetase syndrome with severe interstitial lung disease and acute respiratory failure. PG - 183-8 LID - 10.1186/2049-6958-6-3-183 [doi] AB - We present a case of severe interstitial pneumonitis, mild polyarthritis and polymyositis, and Raynaud's syndrome with the presence of anti-Jo-1 antibodies, which had been diagnosed as anti-synthetase syndrome. The presence, however, of anti-Ro/SSA antibodies led us to understand that we were dealing here with a more severe form of interstitial lung disease. The patient was treated for acute respiratory failure but he showed resistance to glucocorticoids and cyclosporine. Thus, he was treated with infusions of anti-CD20 therapy (rituximab): his clinical conditions improved very rapidly and a significant decrease in the activity of pulmonary disease was detected using high-resolution computerized tomography (HRCT) of the thorax and pulmonary function tests. FAU - Zappa, Maria Cristina AU - Zappa MC AD - Division of Lung Disease, Sandro Pertini Hospital, Rome, Italy. mczappa@inwind.it. FAU - Trequattrini, Tiziana AU - Trequattrini T FAU - Mattioli, Francesco AU - Mattioli F FAU - Rivitti, Rosario AU - Rivitti R FAU - Vigliarolo, Rossana AU - Vigliarolo R FAU - Marcoccia, Antonella AU - Marcoccia A FAU - D'Arcangelo, Giovanni AU - D'Arcangelo G LA - eng PT - Journal Article DEP - 20110831 PL - Italy TA - Multidiscip Respir Med JT - Multidisciplinary respiratory medicine JID - 101477642 PMC - PMC3463069 EDAT- 2011/01/01 00:00 MHDA- 2011/01/01 00:01 PMCR- 2011/08/31 CRDT- 2012/09/11 06:00 PHST- 2010/09/14 00:00 [received] PHST- 2011/02/24 00:00 [accepted] PHST- 2012/09/11 06:00 [entrez] PHST- 2011/01/01 00:00 [pubmed] PHST- 2011/01/01 00:01 [medline] PHST- 2011/08/31 00:00 [pmc-release] AID - 2049-6958-6-3-183 [pii] AID - 10.1186/2049-6958-6-3-183 [doi] PST - epublish SO - Multidiscip Respir Med. 2011 Aug 31;6(3):183-8. doi: 10.1186/2049-6958-6-3-183. PMID- 17450762 OWN - NLM STAT- MEDLINE DCOM- 20070621 LR - 20070424 IS - 0303-464X (Print) IS - 0303-464X (Linking) VI - 32 IP - 1 DP - 2007 Jan-Mar TI - Capillarosecopic patterns in rheumatic diseases. PG - 29-36 AB - Nailfold capillaroscopy (NVC) is a simple, non-invasive, inexpensive and useful method for the analysis of microvascular abnormalities found in several rheumatic disorders. The well-known Raynaud's phenomenon is a clinical condition that should promptly lead to a microvascular analysis, in order to distinguish its primary form (functional, not disease associated) from the secondary Raynaud's phenomenon (disease associated). NVC has an exceptional predictive value in this early distinction, and this may be the best advantage this technique can offer. Microvascular damage is a typical feature of Systemic Sclerosis (SSc) and more than 95% of the patients present architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, avascular areas and neovascularization, as main microvascular abnormalities. These sequential capillaroscopic changes characterize the "scleroderma pattern" and reflect the SSc microangiopathy. In dermatomyositis and undifferentiated connective tissue disease the capillaroscopic aspects are generally named as "scleroderma-like pattern". Capillaroscopy changes have also been found in other systemic rheumatic diseases such as Systemic Lupus Erythematosus, Antiphospholipid Syndrome and Sjögren's Syndrome, further epidemiological and clinical studies are needed to better characterize and standardize nailfold capillaroscopy patterns in these disorders. FAU - Cortes, Sara AU - Cortes S AD - Instituto Português de Reumatologia, Lisboa. sarafgcortes@gmail.com FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article PT - Review PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Humans MH - *Microscopic Angioscopy/methods MH - Rheumatic Diseases/*pathology MH - Scleroderma, Localized/pathology RF - 26 EDAT- 2007/04/25 09:00 MHDA- 2007/06/22 09:00 CRDT- 2007/04/25 09:00 PHST- 2007/04/25 09:00 [pubmed] PHST- 2007/06/22 09:00 [medline] PHST- 2007/04/25 09:00 [entrez] PST - ppublish SO - Acta Reumatol Port. 2007 Jan-Mar;32(1):29-36. PMID- 19906168 OWN - NLM STAT- MEDLINE DCOM- 20100125 LR - 20091112 IS - 1600-0781 (Electronic) IS - 0905-4383 (Linking) VI - 25 IP - 6 DP - 2009 Dec TI - Eosinophilic fascitis: a report of two cases treated with ultraviolet A1 phototherapy. PG - 325-7 LID - 10.1111/j.1600-0781.2009.00463.x [doi] AB - Eosinophilic fascitis (EF) (synonyms: Shulman's syndrome, diffuse fascitis with eosinophilia) is a disease characterized by a complex set of symptoms with scleroderma-like skin lesions, the absence of Raynaud's phenomenon and other non-mandatory symptoms including eosinophilia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia and high levels of circulating immune complexes. EF is probably not a separate disease entity, but an acute variant of localized scleroderma. This rare disease of unknown etiology is usually seen in middle-aged adults. Sclerodermiform indurations without Raynaud's symptoms develop rapidly usually on the extremities and more rarely on the trunk or the face. The skin becomes hard, tightly bound to the underlying structures, so that contractures can develop in as little as a few weeks. The course of the disease is usually chronic but spontaneous remission is possible. Standard therapy includes high doses of corticosteroids, immunosuppressive drugs such as methotrexate, cyclosporin A, cyclophosphamide or azathioprine and others such as psoralen and ultraviolet A radiation. FAU - Silny, Wojciech AU - Silny W AD - Department of Dermatology, University of Medical Science, Poznan, Poland. wsilny@ump.edu.pl FAU - Osmola-Mankowska, Agnieszka AU - Osmola-Mankowska A FAU - Czarnecka-Operacz, Magdalena AU - Czarnecka-Operacz M FAU - Zaba, Ryszard AU - Zaba R FAU - Danczak-Pazdrowska, Aleksandra AU - Danczak-Pazdrowska A FAU - Marciniak, Adrianna AU - Marciniak A LA - eng PT - Case Reports PT - Journal Article PL - England TA - Photodermatol Photoimmunol Photomed JT - Photodermatology, photoimmunology & photomedicine JID - 9013641 SB - IM MH - Adult MH - Eosinophilia/*radiotherapy MH - Fasciitis/*radiotherapy MH - Female MH - Humans MH - *Ultraviolet Therapy EDAT- 2009/11/13 06:00 MHDA- 2010/01/26 06:00 CRDT- 2009/11/13 06:00 PHST- 2009/11/13 06:00 [entrez] PHST- 2009/11/13 06:00 [pubmed] PHST- 2010/01/26 06:00 [medline] AID - PPP463 [pii] AID - 10.1111/j.1600-0781.2009.00463.x [doi] PST - ppublish SO - Photodermatol Photoimmunol Photomed. 2009 Dec;25(6):325-7. doi: 10.1111/j.1600-0781.2009.00463.x. PMID- 31966953 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 12 IP - 1 DP - 2020 Jan 15 TI - Peripheral Gangrene as the Initial Presentation of Systemic Lupus Erythematosus in Emergency Department. PG - e6667 LID - 10.7759/cureus.6667 [doi] LID - e6667 AB - Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease. Among the cutaneous manifestations of SLE, digital gangrene is considered to be very rare. This complication, which may lead to severe ischemic necrosis and amputation, is suggested to be the result of poor perfusion that is usually caused by vasculitis, vasospasm, thromboembolism, or atherosclerosis. Digital gangrene is seen mostly at a late stage of the disease proposing that a long history of SLE is a considered risk factor. Only 0.2% of patients with SLE presented initially as digital necrosis. This is a case report of a 20-year-old Saudi female who presented to the emergency room primarily with acute painful localized dry digital gangrene associated with bilateral lower limbs petechial rash. Her medical history was not suggestive of autoimmune diseases. Serology was positive for SLE. A diagnosis of SLE, lupus nephritis, and vasculitis has been established clinically and serologically. The patient adequately responded to rituximab and steroids as a medical therapy. To our knowledge, cases of acute peripheral gangrene as the initial and only presentation of SLE have rarely been documented in Emergency Medicine. CI - Copyright © 2020, Alalawi et al. FAU - Alalawi, Zainab M AU - Alalawi ZM AD - Emergency Medicine, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Al-Khobar, SAU. FAU - Alkenany, Samar AU - Alkenany S AD - Emergency Medicine, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Al-Khobar, SAU. FAU - Almahroos, Fatemah AU - Almahroos F AD - Emergency Medicine, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Al-Khobar, SAU. FAU - Albloushi, Basmah AU - Albloushi B AD - Emergency Medicine, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, SAU. LA - eng PT - Case Reports PT - Journal Article DEP - 20200115 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC6964793 OTO - NOTNLM OT - digital gangrene OT - raynaud’s phenomenon OT - systemic lupus erythematosus OT - vasculitis COIS- The authors have declared that no competing interests exist. EDAT- 2020/01/23 06:00 MHDA- 2020/01/23 06:01 PMCR- 2020/01/15 CRDT- 2020/01/23 06:00 PHST- 2020/01/23 06:00 [entrez] PHST- 2020/01/23 06:00 [pubmed] PHST- 2020/01/23 06:01 [medline] PHST- 2020/01/15 00:00 [pmc-release] AID - 10.7759/cureus.6667 [doi] PST - epublish SO - Cureus. 2020 Jan 15;12(1):e6667. doi: 10.7759/cureus.6667. PMID- 34158959 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210624 IS - 2053-8855 (Print) IS - 2053-8855 (Electronic) IS - 2053-8855 (Linking) VI - 2021 IP - 6 DP - 2021 Jun TI - A case of macrophage activation syndrome in a patient with anti-synthetase syndrome. PG - omab045 LID - 10.1093/omcr/omab045 [doi] LID - omab045 AB - Anti-synthetase syndrome (ASS) is an autoimmune disease characterized by autoantibodies against an aminoacyl transfer RNA synthetase with clinical features including interstitial lung disease, non-erosive arthritis, myositis, Raynaud's phenomenon, unexplained fever and/or mechanic's hands. Macrophage activation syndrome (MAS) is a potentially fatal hyper- inflammatory syndrome that can occur as a complication of systemic rheumatic diseases. However, the association of MAS and ASS has rarely been reported in the literature. Here, we report this association in a patient with overlap ASS and anti-CCP positive rheumatoid arthritis. First line management with steroids was complicated by diabetic ketoacidosis, hence requiring use of anti-IL1 therapy (anakinra) for disease control. CI - © The Author(s) 2021. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Ahmad, Nadia AU - Ahmad N AD - Rheumatology, Royal Berkshire NHS Foundation Trust, Reading RG1 5AN, UK. FAU - Parmar, Aneel AU - Parmar A AD - Rheumatology, Royal Berkshire NHS Foundation Trust, Reading RG1 5AN, UK. FAU - Kitchen, Joanne AU - Kitchen J AD - Rheumatology, Royal Berkshire NHS Foundation Trust, Reading RG1 5AN, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20210618 PL - England TA - Oxf Med Case Reports JT - Oxford medical case reports JID - 101642070 PMC - PMC8212681 EDAT- 2021/06/24 06:00 MHDA- 2021/06/24 06:01 PMCR- 2021/06/18 CRDT- 2021/06/23 07:20 PHST- 2020/11/26 00:00 [received] PHST- 2020/03/22 00:00 [revised] PHST- 2021/04/11 00:00 [accepted] PHST- 2021/06/23 07:20 [entrez] PHST- 2021/06/24 06:00 [pubmed] PHST- 2021/06/24 06:01 [medline] PHST- 2021/06/18 00:00 [pmc-release] AID - omab045 [pii] AID - 10.1093/omcr/omab045 [doi] PST - epublish SO - Oxf Med Case Reports. 2021 Jun 18;2021(6):omab045. doi: 10.1093/omcr/omab045. eCollection 2021 Jun. PMID- 29641934 OWN - NLM STAT- MEDLINE DCOM- 20190314 LR - 20190314 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 14 IP - 5 DP - 2018 May TI - The challenge of establishing treatment efficacy for cutaneous vascular manifestations of systemic sclerosis. PG - 431-442 LID - 10.1080/1744666X.2018.1464390 [doi] AB - The cutaneous vascular manifestations of systemic sclerosis (SSc) comprise Raynaud's phenomenon, cutaneous ulceration, telangiectasia formation and critical digital ischaemia; each of which are associated with significant disease-related morbidity. Despite the availability of multiple classes of vasodilator therapy, many of which have been the subject of RCTs, a limited number of pharmacological interventions are currently approved for the management of cutaneous vascular manifestations of SSc. Areas covered: A major challenge has been demonstrating treatment efficacy with examples of promising therapies yielding contrasting results in controlled trial settings. Differences between consensus best-practice guidelines, evidence-based recommendations and marketing approvals in different jurisdictions has resulted in geographic variation in clinical practice concerning the management of cutaneous vascular manifestations of SSc. Difficulty demonstrating treatment efficacy risks waning industry engagement for drug development programmes in this field. This article highlights the key challenges in establishing treatment efficacy and barriers that must be overcome to support successful clinical trial programmes across the spectrum of cutaneous vascular manifestations of SSc. Expert commentary: The paucity of approved treatments for cutaneous vascular manifestations of SSc relates as much to challenges in clinical trial design and the need for reliable clinical trial endpoints, as to lack of therapeutic options. FAU - Pauling, John D AU - Pauling JD AD - a Department of Rheumatology, Royal National Hospital for Rheumatic Diseases , Royal United Hospitals NHS Foundation Trust , Bath , UK. AD - b Department of Pharmacy and Pharmacology , University of Bath , Bath , UK. LA - eng PT - Journal Article PT - Review DEP - 20180426 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM MH - Humans MH - *Ischemia/diagnosis/etiology/pathology/therapy MH - *Scleroderma, Systemic/complications/diagnosis/pathology/therapy MH - *Skin Ulcer/diagnosis/etiology/pathology/therapy MH - *Vascular Diseases/diagnosis/etiology/pathology/therapy OTO - NOTNLM OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - clinical trials OT - critical digital ischaemia OT - digital ulceration OT - outcome measures OT - telangiectasia EDAT- 2018/04/12 06:00 MHDA- 2019/03/15 06:00 CRDT- 2018/04/12 06:00 PHST- 2018/04/12 06:00 [pubmed] PHST- 2019/03/15 06:00 [medline] PHST- 2018/04/12 06:00 [entrez] AID - 10.1080/1744666X.2018.1464390 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2018 May;14(5):431-442. doi: 10.1080/1744666X.2018.1464390. Epub 2018 Apr 26. PMID- 31673412 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20200427 IS - 2056-5933 (Print) IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 5 IP - 2 DP - 2019 TI - Association of antiphospholipid antibodies with active digital ulceration in systemic sclerosis. PG - e001012 LID - 10.1136/rmdopen-2019-001012 [doi] LID - e001012 FAU - Martin, Mickaël AU - Martin M AUID- ORCID: 0000-0002-3826-4584 AD - Internal Medicine, Poitiers University Hospital, Poitiers, France. FAU - Martinez, Camille AU - Martinez C AD - Internal Medicine, Colmar Civilian Hospitals, Colmar, France. FAU - Arnaud, Laurent AU - Arnaud L AD - Rheumatology, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France. FAU - Weber, Jean-Christophe AU - Weber JC AD - Internal Medicine, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France. FAU - Poindron, Vincent AU - Poindron V AD - Clinical Immunology, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France. FAU - Blaison, Gilles AU - Blaison G AD - Internal Medicine, Colmar Civilian Hospitals, Colmar, France. FAU - Kieffer, Pierre AU - Kieffer P AD - Internal Medicine, Mulhouse Regional Hospital Center, Mulhouse, France. FAU - Bonnotte, Bernard AU - Bonnotte B AD - Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France. FAU - Berthier, Sabine AU - Berthier S AD - Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France. FAU - Wahl, Denis AU - Wahl D AD - Vascular Medicine, Regional Center for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Vandoeuvre-Les-Nancy, France. AD - Medicine Faculty, University of Lorraine, Vandoeuvre-Les-Nancy, France. FAU - Maurier, Francois AU - Maurier F AD - Internal Medicine, Metz-Thionville Regional Hospital Center, Metz, France. FAU - Pennaforte, Jean-Loup AU - Pennaforte JL AD - Rheumatology, Reims University Hospital, Reims, France. FAU - Bielefeld, Philip AU - Bielefeld P AD - Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France. FAU - Magy-Bertrand, Nadine AU - Magy-Bertrand N AD - Internal Medicine, Besancon University Hospital, Besançon, France. FAU - Devilliers, Hervé AU - Devilliers H AD - Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France. FAU - Martin, Thierry AU - Martin T AD - Clinical Immunology, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20190920 PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) MH - Adult MH - Aged MH - Antibodies, Antiphospholipid/*immunology MH - Autoantibodies/immunology MH - Biomarkers MH - Cross-Sectional Studies MH - Disease Susceptibility MH - Female MH - Fingers/*pathology MH - Humans MH - Male MH - Middle Aged MH - Risk Factors MH - Scleroderma, Systemic/*complications/diagnosis/epidemiology/*immunology MH - Skin Ulcer/diagnosis/epidemiology/*etiology PMC - PMC6802995 OTO - NOTNLM OT - Raynaud’s phenomenon OT - antiphospholipid antibodies OT - antiphospholipid syndrome OT - digital ulcer OT - systemic sclerosis COIS- Competing interests: None declared. EDAT- 2019/11/02 06:00 MHDA- 2019/11/02 06:01 PMCR- 2019/09/20 CRDT- 2019/11/02 06:00 PHST- 2019/05/15 00:00 [received] PHST- 2019/08/23 00:00 [revised] PHST- 2019/09/06 00:00 [accepted] PHST- 2019/11/02 06:00 [entrez] PHST- 2019/11/02 06:00 [pubmed] PHST- 2019/11/02 06:01 [medline] PHST- 2019/09/20 00:00 [pmc-release] AID - rmdopen-2019-001012 [pii] AID - 10.1136/rmdopen-2019-001012 [doi] PST - epublish SO - RMD Open. 2019 Sep 20;5(2):e001012. doi: 10.1136/rmdopen-2019-001012. eCollection 2019. PMID- 27284161 OWN - NLM STAT- MEDLINE DCOM- 20180716 LR - 20250530 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 55 IP - 10 DP - 2016 Oct TI - BSR and BHPR guideline for the treatment of systemic sclerosis. PG - 1906-10 LID - 10.1093/rheumatology/kew224 [doi] FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Royal Free Hospital, London c.denton@ucl.ac.uk. FAU - Hughes, Michael AU - Hughes M AD - Rheumatology Department, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester Academic Health Science Centre, Manchester. FAU - Gak, Nataliya AU - Gak N AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Vila, Josephine AU - Vila J AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne. FAU - Buch, Maya H AU - Buch MH AD - Leeds Institute of Musculoskeletal and Rheumatic Medicine, Chapel Allerton Hospital, Leeds. FAU - Chakravarty, Kuntal AU - Chakravarty K AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Fligelstone, Kim AU - Fligelstone K AD - Centre for Rheumatology, Royal Free Hospital, London. FAU - Gompels, Luke L AU - Gompels LL AD - Rheumatology Department, Musgrove Park Hospital, Taunton. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne. FAU - Herrick, Ariane L AU - Herrick AL AD - Rheumatology Department, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester Academic Health Science Centre, Manchester. FAU - Pang, Jay AU - Pang J AD - Pharmacy Department. FAU - Parker, Louise AU - Parker L AD - Centre for Rheumatology, Royal Free Hospital, London, UK. FAU - Redmond, Anthony AU - Redmond A AD - Leeds Institute of Musculoskeletal and Rheumatic Medicine, Chapel Allerton Hospital, Leeds. FAU - van Laar, Jacob AU - van Laar J AD - Rheumatology and Immunology, UMC Utrecht, Utrecht, The Netherlands. FAU - Warburton, Louise AU - Warburton L AD - Primary Care, Telford and Wrekin NHS Trust, Telford, Shropshire, UK. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology, Royal Free Hospital, London. CN - BSR and BHPR Standards, Guidelines and Audit Working Group LA - eng GR - 20482/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20160609 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - *Disease Management MH - Humans MH - *Practice Guidelines as Topic MH - *Rheumatology MH - Scleroderma, Systemic/*therapy MH - *Societies, Medical OTO - NOTNLM OT - Raynaud’s phenomenon OT - digital ulcers OT - lung fibrosis OT - management OT - pulmonary hypertension OT - scleroderma OT - systemic sclerosis EDAT- 2016/06/11 06:00 MHDA- 2018/07/17 06:00 CRDT- 2016/06/11 06:00 PHST- 2016/06/11 06:00 [entrez] PHST- 2016/06/11 06:00 [pubmed] PHST- 2018/07/17 06:00 [medline] AID - kew224 [pii] AID - 10.1093/rheumatology/kew224 [doi] PST - ppublish SO - Rheumatology (Oxford). 2016 Oct;55(10):1906-10. doi: 10.1093/rheumatology/kew224. Epub 2016 Jun 9. PMID- 37443554 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230718 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 13 IP - 13 DP - 2023 Jun 25 TI - Self-Reported Systemic Sclerosis-Related Symptoms Are More Prevalent in Subjects with Raynaud's Phenomenon in the Lifelines Population: Focus on Pulmonary Complications. LID - 10.3390/diagnostics13132160 [doi] LID - 2160 AB - Puffy fingers and Raynaud's phenomenon (RP) are important clinical predictors of the development of systemic sclerosis (SSc). We aim to assess the prevalence of SSc-related symptoms, explore pulmonary symptoms, and test the usefulness of skin autofluorescence (SAF) as a non-invasive marker for Advanced Glycation Endproducts (AGEs). Subjects from the Lifelines Cohort Study with known connective tissue disease (CTD) were excluded. Patient characteristics, SAF, self-reported pulmonary symptoms, and spirometry were obtained. Subjects (n = 73,948) were categorized into definite RP (5.3%) with and without SSc-related symptoms and non-RP. Prevalence of at least one potential SSc-related symptom (other than RP) was 8.7%; 23.5% in subjects with RP and 7.1% without RP (p < 0.001). Subjects with RP and additional SSc-related symptoms more frequently reported dyspnea at rest, dyspnea after exertion, and self-reported pulmonary fibrosis, and had the lowest mean forced vital capacity compared to the other groups (RP without SSc-related symptoms and no RP, both p < 0.001). In multivariate regression, dyspnea at rest/on exertion remained associated with an increased risk of SSc-related symptoms in subjects with RP (both p < 0.001). SAF was higher in subjects with RP and SSc-related symptoms compared to the other groups (p < 0.001), but this difference was not significant after correction for potential confounders. The prevalence of SSc-related symptoms was approximately three-fold higher in subjects with RP. Pulmonary symptoms are more prevalent in subjects with RP who also reported additional potential SSc-related symptoms. This might suggest that (suspected) early SSc develops more insidiously than acknowledged. According to this study, SAF is no marker for early detection of SSc. FAU - van de Zande, Saskia Corine AU - van de Zande SC AUID- ORCID: 0000-0002-3063-0260 AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Abdulle, Amaal Eman AU - Abdulle AE AUID- ORCID: 0000-0003-3766-2238 AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Al-Adwi, Yehya AU - Al-Adwi Y AUID- ORCID: 0000-0001-5406-7604 AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Stel, Alja AU - Stel A AD - Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - de Leeuw, Karina AU - de Leeuw K AD - Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Brouwer, Elisabeth AU - Brouwer E AD - Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Arends, Suzanne AU - Arends S AUID- ORCID: 0000-0002-4422-7640 AD - Department of Rheumatology and Clinical Immunology, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Gan, Christiaan Tji AU - Gan CT AD - Department of Pulmonary Diseases and Tuberculosis, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - van Goor, Harry AU - van Goor H AUID- ORCID: 0000-0002-6670-1577 AD - Department of Pathology and Medical Biology, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. FAU - Mulder, Douwe Johannes AU - Mulder DJ AUID- ORCID: 0000-0003-3715-6474 AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, 9712 Groningen, The Netherlands. LA - eng GR - 115142-2/Thermo Fisher Scientific (Germany)/ PT - Journal Article DEP - 20230625 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10340274 OTO - NOTNLM OT - Raynaud’s phenomenon OT - epidemiology OT - pulmonary involvement OT - skin autofluorescence OT - systemic sclerosis COIS- Brouwer, as an employee of the UMCG, received speaker fees and consulting fees from Roche in 2016 and 2017 which were paid to the UMCG. Mulder, as an employee of the UMCG, received research grants from Sanofi Genzyme and Actelion which were paid to the UMCG. The other authors declared no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results. EDAT- 2023/07/14 13:06 MHDA- 2023/07/14 13:07 PMCR- 2023/06/25 CRDT- 2023/07/14 01:01 PHST- 2023/04/25 00:00 [received] PHST- 2023/06/13 00:00 [revised] PHST- 2023/06/20 00:00 [accepted] PHST- 2023/07/14 13:07 [medline] PHST- 2023/07/14 13:06 [pubmed] PHST- 2023/07/14 01:01 [entrez] PHST- 2023/06/25 00:00 [pmc-release] AID - diagnostics13132160 [pii] AID - diagnostics-13-02160 [pii] AID - 10.3390/diagnostics13132160 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Jun 25;13(13):2160. doi: 10.3390/diagnostics13132160. PMID- 26331941 OWN - NLM STAT- MEDLINE DCOM- 20170331 LR - 20220321 IS - 1098-2825 (Electronic) IS - 0887-8013 (Print) IS - 0887-8013 (Linking) VI - 30 IP - 5 DP - 2016 Sep TI - Endocan, Novel Potential Biomarker for Systemic Sclerosis: Results of a Pilot Study. PG - 368-73 LID - 10.1002/jcla.21864 [doi] AB - BACKGROUND: Systemic sclerosis (Ssc) is an autoimmune disease characterized by vascular alterations of small arteries and microvessels with subsequent tissue fibrosis. Endocan is expressed by endothelial cells and associated with endothelial dysfunction; therefore it could be a potential biomarker for Ssc patients. METHODS: Twenty-one Ssc patients and 20 sex- and age-matched healthy controls were recruited for the study. Serum endocan levels were determined using ELISA method in all patients and controls. RESULTS: Serum endocan levels were superior in Ssc patients (median 2.53 (1.10-7 ng/ml)) compared with controls (0.79 (0-2 ng/ml), P < 0.05). Higher serum endocan expression was seen in diffuse Ssc subset and associated with the presence of digital ulcers and daily Raynaud's phenomenon (P < 0.05). Higher serum endocan levels were associated with a modified Rodnan skin score >14 and longer disease duration (P < 0.05). Values of areas under the receiver operating curves showed that serum endocan had good discriminative power for Ssc diagnosis, differentiating diffuse from limited subset type and differentiating patients with modified Rodnan skin score above and under 14 (area under curve: 0.94, 0.81, 0.75, respectively). CONCLUSION: The results of this pilot study suggest endocan as a potential biomarker for microvascular manifestations and complications in Ssc patients. These encouraging results could promote future prospective studies in order to determine the exact role played by endocan as a biomarker for Ssc. CI - © 2015 Wiley Periodicals, Inc. FAU - Bălănescu, Paul AU - Bălănescu P AD - CDPC Clinical Immunology Department, Colentina Clinical Hospital, Bucharest, Romania. plbalanescu@gmail.com. AD - Internal Medicine Chair, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania. plbalanescu@gmail.com. AD - Clinical Research Unit, RECIF (Réseau d' Epidémiologie Clinique International Francophone), Bucharest, Romania. plbalanescu@gmail.com. FAU - Lădaru, Anca AU - Lădaru A AD - Pediatrics Department, Institute for Mother and Child Protection "Alfred Rusescu," Bucharest, Romania. FAU - Bălănescu, Eugenia AU - Bălănescu E AD - CDPC Clinical Immunology Department, Colentina Clinical Hospital, Bucharest, Romania. FAU - Voiosu, Theodor AU - Voiosu T AD - Internal Medicine Chair, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania. AD - Clinical Research Unit, RECIF (Réseau d' Epidémiologie Clinique International Francophone), Bucharest, Romania. FAU - Băicuş, Cristian AU - Băicuş C AD - Internal Medicine Chair, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania. AD - Clinical Research Unit, RECIF (Réseau d' Epidémiologie Clinique International Francophone), Bucharest, Romania. AD - Department of Internal Medicine, Colentina Clinical Hospital, Bucharest, Romania. FAU - Dan, Gheorghe Andrei AU - Dan GA AD - Internal Medicine Chair, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania. AD - Clinical Research Unit, RECIF (Réseau d' Epidémiologie Clinique International Francophone), Bucharest, Romania. AD - Department of Internal Medicine, Colentina Clinical Hospital, Bucharest, Romania. LA - eng PT - Journal Article DEP - 20150901 PL - United States TA - J Clin Lab Anal JT - Journal of clinical laboratory analysis JID - 8801384 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (ESM1 protein, human) RN - 0 (Neoplasm Proteins) RN - 0 (Proteoglycans) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Biomarkers/blood MH - Case-Control Studies MH - Enzyme-Linked Immunosorbent Assay/methods MH - Female MH - Humans MH - Male MH - Middle Aged MH - Neoplasm Proteins/*blood MH - Pilot Projects MH - Proteoglycans/*blood MH - Scleroderma, Systemic/*blood MH - Statistics, Nonparametric PMC - PMC6807019 OTO - NOTNLM OT - Raynaud's phenomenon OT - biomarker OT - endocan OT - ischemic digital ulcer OT - skin fibrosis OT - systemic sclerosis OT - vascular alteration EDAT- 2015/09/04 06:00 MHDA- 2017/04/01 06:00 PMCR- 2015/09/01 CRDT- 2015/09/03 06:00 PHST- 2014/07/23 00:00 [received] PHST- 2015/06/27 00:00 [accepted] PHST- 2015/09/03 06:00 [entrez] PHST- 2015/09/04 06:00 [pubmed] PHST- 2017/04/01 06:00 [medline] PHST- 2015/09/01 00:00 [pmc-release] AID - JCLA21864 [pii] AID - 10.1002/jcla.21864 [doi] PST - ppublish SO - J Clin Lab Anal. 2016 Sep;30(5):368-73. doi: 10.1002/jcla.21864. Epub 2015 Sep 1. PMID- 31720162 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 11 IP - 9 DP - 2019 Sep 18 TI - Scurvy: A Rare Cause of Anemia. PG - e5694 LID - 10.7759/cureus.5694 [doi] LID - e5694 AB - Scurvy is a rather uncommon disease today, and its symptoms can certainly be exhibited with vitamin C deficiency. Dangerously low levels of the vitamin can cause serious health complications and have been proven to be fatal. We present the case of a 42-year-old female with multiple primary diagnoses: easy bruising and Raynaud's syndrome without gangrene. The purpose of this report is to call attention to the clinical presentation, diagnosis, and treatment of scurvy. CI - Copyright © 2019, Ricaurte et al. FAU - Ricaurte, Frank R AU - Ricaurte FR AD - Westlake High School, Cleveland, USA. FAU - Kewan, Tariq AU - Kewan T AD - Internal Medicine, Cleveland Clinic - Fairview Hospital, Cleveland, USA. FAU - Daw, Hamed AU - Daw H AD - Hematology and Oncology, Cleveland Clinic - Fairview Hospital, Cleveland, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20190918 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC6823021 OTO - NOTNLM OT - anemia OT - deficiency OT - hemorrhage OT - perifollicular OT - scurvy OT - vitamin c supplementation COIS- The authors have declared that no competing interests exist. EDAT- 2019/11/14 06:00 MHDA- 2019/11/14 06:01 PMCR- 2019/09/18 CRDT- 2019/11/14 06:00 PHST- 2019/11/14 06:00 [entrez] PHST- 2019/11/14 06:00 [pubmed] PHST- 2019/11/14 06:01 [medline] PHST- 2019/09/18 00:00 [pmc-release] AID - 10.7759/cureus.5694 [doi] PST - epublish SO - Cureus. 2019 Sep 18;11(9):e5694. doi: 10.7759/cureus.5694. PMID- 24742450 OWN - NLM STAT- MEDLINE DCOM- 20140612 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 32 IP - 2 Suppl 81 DP - 2014 Mar-Apr TI - Current management strategies for systemic sclerosis. PG - 156-64 AB - Systemic sclerosis remains a challenging disease despite progress that has taken place in the management of organ-based complications. Overall management strategies need to take into account the features of the disease that are common to almost all patients such as skin involvement, gastro-oesophageal manifestations and secondary Raynaud's, as well as identify less frequent but critical manifestations that impact on survival including heart, lung, renal and more severe GI involvement. Treatments can be considered to be disease-modifying or symptomatic. In addition, it is important to address more generic problems such as the emotional, psychological and economic impact of a chronic autoimmune rheumatic disease. This article reviews general approaches to disease assessment and management and relates this to subset and stage of the condition. FAU - Nihtyanova, Svetlana I AU - Nihtyanova SI AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK. FAU - Ong, Voon H AU - Ong VH FAU - Denton, Christopher P AU - Denton CP LA - eng PT - Journal Article PT - Review DEP - 20140411 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Immunosuppressive Agents) RN - 0 (Steroids) SB - IM MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Scleroderma, Systemic/diagnosis/*drug therapy MH - Steroids/*therapeutic use EDAT- 2014/04/20 06:00 MHDA- 2014/06/13 06:00 CRDT- 2014/04/19 06:00 PHST- 2014/01/09 00:00 [received] PHST- 2014/03/26 00:00 [accepted] PHST- 2014/04/19 06:00 [entrez] PHST- 2014/04/20 06:00 [pubmed] PHST- 2014/06/13 06:00 [medline] AID - 7892 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2014 Mar-Apr;32(2 Suppl 81):156-64. Epub 2014 Apr 11. PMID- 26180702 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150716 LR - 20200930 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 7 IP - 6 DP - 2015 Jun TI - Hereditary Hemorrhagic Telangiectasia with Unusual Associations. PG - e278 LID - 10.7759/cureus.278 [doi] LID - e278 AB - We describe a report of an elderly lady who was hospitalized with progressive worsening of breathlessness and fatigue of one month's duration. Clinical evaluation of the patient revealed hereditary hemorrhagic telangiectasia, interstitial lung disease, pulmonary hypertension without left heart failure, and bilateral gluteal calcinosis cutis. Initially, CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome was considered in view of the telangiectasia and calcinosis cutis, but a strong autosomal inheritance pattern and endoscopies (nasal and upper gastrointestinal) favored a diagnosis of hereditary hemorrhagic telangiectasia with rare associations. FAU - Jain, Dheeraj AU - Jain D AD - Department of General Medicine, Indira Gandhi Medical College & Research Institute. FAU - Viswanathan, Stalin AU - Viswanathan S AD - Department of General Medicine, Indira Gandhi Medical College & Research Institute. FAU - Ramasamy, Chandramohan AU - Ramasamy C AD - Department of Cardiology, JIPMER. LA - eng PT - Case Reports PT - Journal Article DEP - 20150616 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC4494561 OTO - NOTNLM OT - calcinosis cutis OT - hereditary hemorrhagic telangiectasia OT - interstitial lung disease OT - pulmonary hypertension COIS- The authors have declared that no competing interests exist. EDAT- 2015/07/17 06:00 MHDA- 2015/07/17 06:01 PMCR- 2015/06/16 CRDT- 2015/07/17 06:00 PHST- 2015/06/16 00:00 [accepted] PHST- 2015/07/17 06:00 [entrez] PHST- 2015/07/17 06:00 [pubmed] PHST- 2015/07/17 06:01 [medline] PHST- 2015/06/16 00:00 [pmc-release] AID - 10.7759/cureus.278 [doi] PST - epublish SO - Cureus. 2015 Jun 16;7(6):e278. doi: 10.7759/cureus.278. eCollection 2015 Jun. PMID- 34650852 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211016 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 9 DP - 2021 Sep TI - A Case of Longstanding Idiopathic Pernio/Chilblain Disease. PG - e17674 LID - 10.7759/cureus.17674 [doi] LID - e17674 AB - Pernio, also known as chilblains, presents as erythematous macules at sites of cold exposure, mainly in women. It is a diagnosis that is often overlooked, and when suspecting a patient with pernio, other conditions such as lupus nephritis and Raynaud's must be ruled out. A 46-year-old lady presented to the clinic with skin findings suggestive of pernio. She had erythematous macules on the dorsum of her hands, which appeared during cold weather and lasted for three weeks. She had been suffering with this condition for over 18 years and nothing has helped her condition, other than preventing cold exposure. There are limited treatment options for pernio, and current management includes using steroids, calcium-channel blockers and cold avoidance. Current research has suggested that pernio could also be linked to the severe acute respiratory syndrome coronavirus 2. CI - Copyright © 2021, Maraj et al. FAU - Maraj, Diva C AU - Maraj DC AD - Allergy and Immunology, St. Joseph Mercy Oakland Hospital, Pontiac, USA. FAU - Barak-Norris, Ronda AU - Barak-Norris R AD - Allergy and Immunology, St. Joseph Mercy Oakland Hospital, Pontiac, USA. AD - Allergy and Immunology, Beaumont Hospital, Royal Oak, USA. AD - Allergy and Immunology, Ascension Providence Hospital, Southfield, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210903 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8487589 OTO - NOTNLM OT - blisters OT - chilblain's disease OT - pernio OT - rheumatology OT - skin COIS- The authors have declared that no competing interests exist. EDAT- 2021/10/16 06:00 MHDA- 2021/10/16 06:01 PMCR- 2021/09/03 CRDT- 2021/10/15 06:37 PHST- 2021/09/02 00:00 [accepted] PHST- 2021/10/15 06:37 [entrez] PHST- 2021/10/16 06:00 [pubmed] PHST- 2021/10/16 06:01 [medline] PHST- 2021/09/03 00:00 [pmc-release] AID - 10.7759/cureus.17674 [doi] PST - epublish SO - Cureus. 2021 Sep 3;13(9):e17674. doi: 10.7759/cureus.17674. eCollection 2021 Sep. PMID- 41267036 OWN - NLM STAT- In-Process LR - 20251124 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 25 IP - 1 DP - 2025 Nov 20 TI - Remote monitoring of spirometry and oximetry in patients with connective tissue disease related interstitial lung disease: a prospective observational cohort study. PG - 539 LID - 10.1186/s12890-025-03966-6 [doi] LID - 539 AB - BACKGROUND: Remote monitoring of forced vital capacity (FVC) and oximetry (SpO2) in patients with idiopathic pulmonary fibrosis (IPF) is emerging as an adjunct to routine in-person ambulatory care. Patients with connective tissue diseases (CTD) commonly have impaired hand function, diminished oral aperture and Raynaud's phenomenon, which can uniquely impair the user’s ability to use these devices. We aimed to assess the acceptability, usability and adherence to home monitoring in patients with CTD associated interstitial lung disease (CTD-ILD) compared to patients with IPF. METHODS: Participants were recruited to a prospective observational cohort study (NCT06702228), provided with a portable handheld spirometer and finger pulse oximeter connected to a smart phone application (app), received training in their use, asked to use these at least once a week, and were reviewed at 6-months. RESULTS: Ninety-seven participants completed the 6-month study. Participants in the CTD-ILD group were slightly younger than those with IPF with a mean and standard deviation (SD) of 62.44 (15.25) compared to 70.21 (9.39) years (p = 0.005). The number of days the devices were used was lower in the CTD-ILD (69.2 versus 99.4, p = 0.028). In line with study instructions, there was no significant difference in weekly adherence with FVC or SpO2 measurement (71.5% versus 75%, p = 0.65). We found a strong correlation between home and hospital-based FVC (r = 0.76, p < 0.0001). Both groups reported finding the devices and app easy to use (77% versus 72%, p = 0.65), but more participants with CTD-ILD reported difficulty obtaining SpO2 readings compared to those with IPF (33.33% versus 12.77%, p = 0.02). Six (16.7%) participants from the CTD-ILD group experienced difficulty using the devices due to hand function impairment (p = 0.004), 3 (8.3%) were unable to use a finger probe at all and required an ear oximeter. We found no differences in the correlation of FVC adherence with age, ILD-GAP score, or participant reported symptom scores between the groups. CONCLUSIONS: While participants with CTD-ILD had lower device use than those with IPF, they did have high adherence, satisfaction and reported ease of use despite Raynaud's phenomenon and impaired hand function. Therefore, remote monitoring has potential as an adjunct to routine care in CTD-ILD. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12890-025-03966-6. FAU - Ng, Wan Lin AU - Ng WL AD - Department of Rheumatology, Beaumont Hospital, Dublin, Ireland. AD - Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - Carolan, Aoife AU - Carolan A AD - Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland. AD - Tissue Engineering Research Group, Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - Sulaiman, Imran AU - Sulaiman I AD - Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - Greene, Garrett AU - Greene G AD - School of Mathematics and Statistics, University College Dublin, Dublin, Ireland. FAU - Morgan, Ross K AU - Morgan RK AD - Department of Medicine, Beaumont Hospital, Dublin, Ireland. FAU - Howard, Donough AU - Howard D AD - Department of Rheumatology, Beaumont Hospital, Dublin, Ireland. FAU - Durcan, Laura AU - Durcan L AD - Department of Rheumatology, Beaumont Hospital, Dublin, Ireland. FAU - Hurley, Killian AU - Hurley K AD - Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland. killianhurley@rcsi.ie. AD - Tissue Engineering Research Group, Royal College of Surgeons in Ireland, Dublin, Ireland. killianhurley@rcsi.ie. LA - eng GR - ECSA-2020-011/HRBI_/Health Research Board/Ireland GR - 101078300 - STAR-TEL/HORIZON EUROPE European Research Council/ PT - Journal Article DEP - 20251120 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 SB - IM PMC - PMC12632022 OTO - NOTNLM OT - Acceptability OT - Adherence OT - Connective tissue disease associated interstitial lung disease OT - Idiopathic pulmonary fibrosis OT - Oximetry OT - Patient survey OT - Raynaud's phenomenon OT - Remote monitoring OT - Spirometry COIS- Declarations: Ethical approval and consent to participate. This study received ethical approval from Beaumont Hospital Research Ethics Committee (REC 20/24) and was conducted in accordance with the ethical principles of the Declaration of Helsinki. All participants provided written informed consent. Consent for publication: Not applicable. Competing interests: KH has received grants to his institution from Moderna and patientMpower Ltd not related to this study. KH reports lecture fees from Boehringer Ingelheim, and consultancy fees from patientMpower Ltd. EDAT- 2025/11/21 06:27 MHDA- 2025/11/21 06:27 PMCR- 2025/11/20 CRDT- 2025/11/21 00:05 PHST- 2025/05/09 00:00 [received] PHST- 2025/09/26 00:00 [accepted] PHST- 2025/11/21 06:27 [medline] PHST- 2025/11/21 06:27 [pubmed] PHST- 2025/11/21 00:05 [entrez] PHST- 2025/11/20 00:00 [pmc-release] AID - 10.1186/s12890-025-03966-6 [pii] AID - 3966 [pii] AID - 10.1186/s12890-025-03966-6 [doi] PST - epublish SO - BMC Pulm Med. 2025 Nov 20;25(1):539. doi: 10.1186/s12890-025-03966-6. PMID- 29290695 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 9 DP - 2017 TI - Systematic review of the role of rituximab in treatment of antineutrophil cytoplasmic autoantibody-associated vasculitis, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch-Schönlein purpura, ankylosing spondylitis, and Raynaud's phenomenon. PG - 201-214 LID - 10.2147/OARRR.S149373 [doi] AB - Rituximab (RTX) is established for the treatment of rheumatoid arthritis. This systematic review of the literature since 2006 summarizes evidence for the use of RTX in the treatment of additional rheumatological diseases: antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV), hepatitis C virus-related cryoglobulinemic vasculitis, Henoch-Schönlein purpura, ankylosing spondylitis, and Raynaud's phenomenon. Data from randomized controlled trials are available only for AAV, confirming efficacy for remission induction, including in disease resistant to conventional treatment, and maintenance of remission. Further studies are required to confirm optimal maintenance regimens in AAV, important questions needing to be addressed including protocol administration versus treatment in response to clinical relapse and the importance of maintaining B-cell depletion. Sufficient data are available in other diseases to suggest RTX to be useful and that randomized controlled trials should be conducted. FAU - Taha, Rbab AU - Taha R AD - Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah. FAU - El-Haddad, Hadeel AU - El-Haddad H AD - Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah. FAU - Almuallim, Abdulqader AU - Almuallim A AD - Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca. FAU - Alshaiki, Fatma AU - Alshaiki F AD - Department of Medicine, East Jeddah Hospital, Jeddah. FAU - Obaid, Elaf AU - Obaid E AD - Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca. FAU - Almoallim, Hani AU - Almoallim H AD - Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah. AD - Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca. AD - Rheumatic Diseases, Umm Al-Qura University, Mecca, Saudi Arabia. LA - eng PT - Journal Article PT - Review DEP - 20171215 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC5735990 OTO - NOTNLM OT - anti-CD20 monoclonal antibody OT - anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis OT - refractory ankylosing spondylitis OT - refractory rheumatological diseases OT - resistant cryoglobulinemic vasculitis COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2018/01/02 06:00 MHDA- 2018/01/02 06:01 PMCR- 2017/12/15 CRDT- 2018/01/02 06:00 PHST- 2018/01/02 06:00 [entrez] PHST- 2018/01/02 06:00 [pubmed] PHST- 2018/01/02 06:01 [medline] PHST- 2017/12/15 00:00 [pmc-release] AID - oarrr-9-201 [pii] AID - 10.2147/OARRR.S149373 [doi] PST - epublish SO - Open Access Rheumatol. 2017 Dec 15;9:201-214. doi: 10.2147/OARRR.S149373. eCollection 2017. PMID- 32067119 OWN - NLM STAT- MEDLINE DCOM- 20210615 LR - 20210615 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 22 IP - 3 DP - 2020 Feb 14 TI - Carnitine Palmitoyl Transferase Deficiency in a University Immunology Practice. PG - 8 LID - 10.1007/s11926-020-0879-9 [doi] AB - PURPOSE: This report describes the clinical manifestations of 35 patients sent to a University Immunology clinic with a diagnosis of fatigue and exercise intolerance who were identified to have low carnitine palmitoyl transferase activity on muscle biopsies. RECENT FINDINGS: All of the patients presented with fatigue and exercise intolerance and many had been diagnosed with fibromyalgia. Their symptoms responded to treatment of the metabolic disease. Associated symptoms included bloating, diarrhea, constipation, gastrointestinal reflux symptoms, recurrent infections, arthritis, dyspnea, dry eye, visual loss, and hearing loss. Associated medical conditions included Hashimoto thyroiditis, Sjogren's syndrome, seronegative arthritis, food hypersensitivities, asthma, sleep apnea, and vasculitis. This study identifies clinical features that should alert physicians to the possibility of an underlying metabolic disease. Treatment of the metabolic disease leads to symptomatic improvement. FAU - Bax, Kiley AU - Bax K AD - Department of Medicine, SUNY at Buffalo School of Medicine, Buffalo, NY, USA. FAU - Isackson, Paul J AU - Isackson PJ AD - Department of Pediatrics, SUNY at Buffalo School of Medicine, Buffalo, NY, USA. FAU - Moore, Molly AU - Moore M AD - Department of Surgery, SUNY at Buffalo School of Medicine, Buffalo, NY, USA. FAU - Ambrus, Julian L Jr AU - Ambrus JL Jr AD - Department of Medicine, SUNY at Buffalo School of Medicine, Buffalo, NY, USA. jambrus@buffalo.edu. AD - Division of Allergy, Immunology and Rheumatology SUNY at Buffalo School of Medicine, Room 8030C, Center for Translational Research, 875 Ellicott Street, Buffalo, NY, 14203, USA. jambrus@buffalo.edu. LA - eng PT - Journal Article PT - Review DEP - 20200214 PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - EC 2.3.1.21 (Carnitine O-Palmitoyltransferase) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Biopsy MH - Carnitine O-Palmitoyltransferase/*deficiency MH - Female MH - Humans MH - Lipid Metabolism Disorders/*diagnosis/therapy MH - Male MH - Metabolic Diseases/diagnosis/therapy MH - Middle Aged MH - Muscles/*pathology OTO - NOTNLM OT - Asthma OT - Carnitine palmitoyl transferase OT - Gastrointestinal dysmotility OT - Mitochondria OT - Raynaud’s OT - Sjogren’s syndrome EDAT- 2020/02/19 06:00 MHDA- 2021/06/16 06:00 CRDT- 2020/02/19 06:00 PHST- 2020/02/19 06:00 [entrez] PHST- 2020/02/19 06:00 [pubmed] PHST- 2021/06/16 06:00 [medline] AID - 10.1007/s11926-020-0879-9 [pii] AID - 10.1007/s11926-020-0879-9 [doi] PST - epublish SO - Curr Rheumatol Rep. 2020 Feb 14;22(3):8. doi: 10.1007/s11926-020-0879-9. PMID- 41658246 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260209 LR - 20260211 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 18 DP - 2026 TI - Age and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR). PG - 1759720X251410243 LID - 10.1177/1759720X251410243 [doi] LID - 1759720X251410243 AB - BACKGROUND: The sequence and temporal relationship between Raynaud's phenomenon (RP) and the first non-Raynaud's sign/symptom (NRP) in systemic sclerosis (SSc) have been partially investigated. OBJECTIVES: To evaluate whether the mode and ages of clinical onset are associated with disease endotype and survival in SSc. DESIGN: We included SSc patients from the Systemic sclerosis Progression INvestiGation registry of the Italian Society of Rheumatology (SPRING-SIR) registry in a cohort study, with post hoc cross-sectional and longitudinal analysis. METHODS: Patients were grouped based on age-RP and age-NRP quartiles. Additionally, categories were defined based on mode of onset: RP group-RP onset at least 1 year before NRP; Simultaneous group-RP onset within the same year of NRP; NRP group-RP onset after at least 1 year after NRP. Comparisons were made using Chi-square and ANOVA tests. Logistic, linear, and multinomial regression models were applied to assess associations, while Kaplan-Meier curves and Cox regression were used to assess mortality. RESULTS: A total of 1748 patients were eligible: 682 (39.0%) in the RP group, 1026 (58.8%) in the simultaneous group, and 39 (2.2%) in the NRP group. A higher prevalence of anti-centromere antibodies was found In the RP group, while the simultaneous group had more diffuse cutaneous SSc (dcSSc), anti-topoisomerase-I antibodies, and higher Rodnan's skin score (mRSS). The NRP group presented higher prevalence of pulmonary arterial hypertension. On logistic regression, the simultaneous group was associated with a higher prevalence of dcSSc compared to the RP group (odds ratio, 1.491, 95% confidence interval (CI): 1.032-2.154). Younger age at RP onset was associated with lower systolic pulmonary artery pressure and mRSS. In 943 patients with available follow-up (median 24 months), the simultaneous group had higher mortality compared to the RP group (hazard ratio, 1.975, 95% CI: 1.002-3.893). CONCLUSION: The timing of RP and NRP onset may help define SSc endotype and survival. Patients with simultaneous RP-NRP onset have more severe disease features and higher mortality risk, emphasizing the relevance of onset timing in disease stratification. CI - © The Author(s), 2026. FAU - Peretti, Silvia AU - Peretti S AUID- ORCID: 0009-0007-8159-9754 AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology, Department of Medical and Geriatric specialties, Careggi University Hospital, University of Florence, Florence, Italy. FAU - Bruni, Cosimo AU - Bruni C AUID- ORCID: 0000-0003-2813-2083 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich 8091, Switzerland. AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. FAU - Bonomi, Francesco AU - Bonomi F AUID- ORCID: 0000-0001-7744-8549 AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology, Department of Medical and Geriatric specialties, Careggi University Hospital, University of Florence, Florence, Italy. FAU - De Angelis, Rossella AU - De Angelis R AD - Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy. FAU - Bajocchi, Gianluigi AU - Bajocchi G AD - Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Orlandi, Martina AU - Orlandi M AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AUID- ORCID: 0000-0001-5042-1282 AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Foti, Roberta AU - Foti R AD - Rheumatology Unit, AOU Policlinico San Marco, Catania, Italy. FAU - Visalli, Elisa AU - Visalli E AD - Rheumatology Unit, AOU Policlinico San Marco, Catania, Italy. FAU - Cuomo, Giovanna AU - Cuomo G AD - Department of Precision Medicine, University of Campania-Luigi Vanvitelli University, Naples, Italy. FAU - Ariani, Alarico AU - Ariani A AD - Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Lepri, Gemma AU - Lepri G AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology, Department of Medical and Geriatric specialties, Careggi University Hospital, University of Florence, Florence, Italy. FAU - Girelli, Francesco AU - Girelli F AD - Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni-L Pierantoni, Forlì, Italy. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Internal Medicine, Anesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Department of Rheumatology, University of Padua, Padova, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Rheumatology Division, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Rome, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Brescia, Italy. AD - Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Clinica Reumatologica, Dipartimento di Reumatologia e Scienze Mediche, ASST Gaetano Pini-CTO, Dipartimento di Scienze Cliniche e di Comunità, Dipartimento di Eccellenza, Università degli Studi di Milano, Milan, Italy. FAU - De Santis, Maria AU - De Santis M AD - Department of Biomedical Sciences, Humanitas University, Pieve Emanuele-Milan and Research Hospital, Milan, Italy. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AUID- ORCID: 0000-0001-7479-4462 AD - Rheumatology Unit, Department of Precision and Regenerative Medicine-Ionian Area, University of Bari "Aldo Moro," Bari, Italy. AD - Rheumatology Service, Internal Medicine Unit "F. Miulli" General Hospital, Acquaviva delle Fonti, Bari, Italy. AD - Department of Medicine and Surgery, LUM "G. De Gennaro," Casamassima, Bari, Italy. FAU - Murdaca, Giuseppe AU - Murdaca G AD - Department of Internal Medicine, University of Genoa and Allergology, and Clinical Immunology Unit, Ospedale San Bartolomeo, Sarzana, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - Rheumatology Unit, Department of Health Science, San Carlo Hospital, University of Basilicata, Potenza, Italy. FAU - Pettiti, Giorgio AU - Pettiti G AD - Rheumatology Unit ASO S., Croce e Carle Hospital, Cuneo, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Department of Rheumatology, University of Pisa, Pisa, Italy. FAU - Caminiti, Maurizio AU - Caminiti M AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy. FAU - Iuliano, Annamaria AU - Iuliano A AD - Rheumatology Unit, San Camillo-Forlanini Hospital, Rome, Italy. FAU - Ciano, Giovanni AU - Ciano G AD - Local Health Department, Hospital of Ariano Irpino, Ariano Irpino, Italy. FAU - Beretta, Lorenzo AU - Beretta L AD - Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Bagnato, Gianluca AU - Bagnato G AD - Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy. FAU - Lubrano, Ennio AU - Lubrano E AD - Department of Rheumatology, University of Molise, Campobasso, Italy. FAU - De Andres, Ilenia AU - De Andres I AD - Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione Garibaldi, Catania, Italy. FAU - Idolazzi, Luca AU - Idolazzi L AUID- ORCID: 0000-0002-7254-4686 AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Saracco, Marta AU - Saracco M AD - Rheumatology Unit, Mauriziano-Umberto I Hospital, Turin, Italy. FAU - Agnes, Cecilia AU - Agnes C AD - Division of Rehabilitation, Department of Medicine, Torino, ASL TO5, Carmagnola, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, & Inflammation, Fibrosis and Ageing initiative, Milan, Italy. AD - Vita-Salute San Raffaele University, Milan, Italy. FAU - De Luca, Giacomo AU - De Luca G AD - IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, & Inflammation, Fibrosis and Ageing initiative, Milan, Italy. AD - Vita-Salute San Raffaele University, Milan, Italy. FAU - Cipolletta, Edoardo AU - Cipolletta E AD - Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy. FAU - Fornaro, Marco AU - Fornaro M AD - Rheumatology Unit, Department of Precision and Regenerative Medicine-Ionian Area, University of Bari "Aldo Moro," Bari, Italy. FAU - Lumetti, Federica AU - Lumetti F AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Spinella, Amelia AU - Spinella A AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Magnani, Luca AU - Magnani L AD - Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Iandoli, Carlo AU - Iandoli C AD - Department of Precision Medicine, University of Campania-Luigi Vanvitelli University, Naples, Italy. FAU - Gigante, Antonietta AU - Gigante A AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Pellegrino, Greta AU - Pellegrino G AD - Department of Rheumatology, IRCCS Ospedale Galeazzi-Sant'Ambrogio, Milan, Italy. AD - Dipartimento di Scienze Biomediche e Cliniche, Università degli Studi di Milano, Milan, Italy. FAU - Pigatto, Erika AU - Pigatto E AD - Department of Medicine, Villa Salus Hospital, Mestre, Italy. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Brescia, Italy. AD - Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - De Lorenzis, Enrico AU - De Lorenzis E AD - Rheumatology Division, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Rome, Italy. FAU - Mennillo, Gianna Angela AU - Mennillo GA AD - Rheumatology Unit, Department of Health Science, San Carlo Hospital, University of Basilicata, Potenza, Italy. FAU - Battista, Marco Di AU - Battista MD AD - Department of Rheumatology, University of Pisa, Pisa, Italy. FAU - Pagano Mariano, Giuseppa AU - Pagano Mariano G AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy. FAU - Furini, Federica AU - Furini F AD - Department of Medicine, Villa Salus Hospital, Mestre, Italy. FAU - Vultaggio, Licia AU - Vultaggio L AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna, Ferrara, Italy. FAU - Parisi, Simone AU - Parisi S AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e della Scienza di Torino, Turin, Italy. FAU - Peroni, Clara Lisa AU - Peroni CL AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e della Scienza di Torino, Turin, Italy. FAU - Bianchi, Gerolamo AU - Bianchi G AD - Rheumatology Unit, Department of Medical Specialities, Local Health Trust 3, Genoa, Italy. FAU - Fusaro, Enrico AU - Fusaro E AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e della Scienza di Torino, Turin, Italy. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AD - Rheumatology Unit, San Camillo-Forlanini Hospital, Rome, Italy. FAU - Govoni, Marcello AU - Govoni M AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna, Ferrara, Italy. FAU - D'Angelo, Salvatore AU - D'Angelo S AUID- ORCID: 0000-0002-7442-1110 AD - Rheumatology Unit, Department of Health Science, San Carlo Hospital, University of Basilicata, Potenza, Italy. FAU - Cozzi, Franco AU - Cozzi F AD - Department of Medicine, Villa Salus Hospital, Mestre, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Brescia, Italy. AD - Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology, Department of Medical and Geriatric specialties, Careggi University Hospital, University of Florence, Florence, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, & Inflammation, Fibrosis and Ageing initiative, Milan, Italy. AD - Vita-Salute San Raffaele University, Milan, Italy. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - Department of Rheumatology, University of Padua, Padova, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - D'Agostino, Maria Antonietta AU - D'Agostino MA AD - Rheumatology Division, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Rome, Italy. FAU - Iannone, Florenzo AU - Iannone F AUID- ORCID: 0000-0003-0474-5344 AD - Rheumatology Unit, Department of Precision and Regenerative Medicine-Ionian Area, University of Bari "Aldo Moro," Bari, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, & Inflammation, Fibrosis and Ageing initiative, Milan, Italy. AD - Vita-Salute San Raffaele University, Milan, Italy. FAU - Ferri, Clodoveo AU - Ferri C AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. AD - Rheumatology Clinic "Madonna dello Scoglio" Crotonei, Crotone, Italy. FAU - Randone, Silvia Bellando AU - Randone SB AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology, Department of Medical and Geriatric specialties, Careggi University Hospital, University of Florence, Florence, Italy. LA - eng PT - Journal Article DEP - 20260205 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC12881321 OAB - How systemic sclerosis starts impacts how the disease will behave Systemic sclerosis (SSc) is a rare autoimmune disease that affects the skin and internal organs. In most cases the disease starts with Raynaud’s phenomenon (RP), a discolouring of the fingers in reaction to cold temperatures; some other patients may experience different symptoms first, such as joint pain, skin changes or shortness of breath. Understanding which symptom appears first, and at what age, may help doctors identify patients who are more likely to develop severe disease. This study included 1,748 SSc patients enrolled in the Italian SPRING-SIR registry, divided patients into three groups: those who first developed RP (RP group), those who developed RP and other symptoms in the same year (Simultaneous), and those who developed non-RP symptoms first (NRP group). We found that patients in the RP group were younger at disease onset and often had milder disease. Patients in the Simultaneous group were more likely to develop diffuse skin disease, lung complications, and had a higher risk of death. Patients in the NRPgroup were rare but often had aggressive involvement of heart and lung. Overall, our results show that the timing of RP and non-RP symptoms can help predict disease course and survival in SSc. These findings may help doctors recognize higher-risk patients earlier and provide closer monitoring and tailored treatments. OABL- eng OTO - NOTNLM OT - Raynaud’s phenomenon OT - disease onset OT - non-Raynaud’s symptoms OT - risk assessment OT - systemic sclerosis COIS- C.B. consultant of: Boehringer Ingelheim and Glaxo Smith Klein, Grant/research support from: Scleroderma Clinical Trials Consortium (SCTC), Scleroderma Research Foundation (SRF), EMDO Foundation, Iten-Kohaut Foundation, Kurt und Senta Herrmann Foundation, Jubileum Foundation of Swisslife. Congress support from Boehringer-Ingelheim. All outside the content of this manuscript. All other authors declare no conflicts of interest. EDAT- 2026/02/09 12:53 MHDA- 2026/02/09 12:54 PMCR- 2026/02/05 CRDT- 2026/02/09 06:43 PHST- 2025/05/28 00:00 [received] PHST- 2025/12/08 00:00 [accepted] PHST- 2026/02/09 12:54 [medline] PHST- 2026/02/09 12:53 [pubmed] PHST- 2026/02/09 06:43 [entrez] PHST- 2026/02/05 00:00 [pmc-release] AID - 10.1177_1759720X251410243 [pii] AID - 10.1177/1759720X251410243 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2026 Feb 5;18:1759720X251410243. doi: 10.1177/1759720X251410243. eCollection 2026. PMID- 33768864 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240331 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 9 IP - 3 DP - 2021 Mar TI - A case of antisynthetase syndrome with chilblains-like lesions and microangiopathy. PG - 1446-1449 LID - 10.1002/ccr3.3790 [doi] AB - We present the case of a 50-year-old woman with febrile myalgia, chilblains-like lesions, and cough. Antinuclear antibodies and anti-PL-12 antisynthetase autoantibodies were found in complementary examinations. Interstitial lung disease was found on chest computed tomography. Nailfold capillaroscopy showed microangiopathic lesions. Antisynthetase syndrome is a recently described entity in inflammatory myopathies, with specific clinical criteria. Interstitial lung disease is very common, especially in anti-PL-12 associated antisynthetase syndrome. Raynaud's phenomenon is another well-defined criterion. However, microangiopathic damage is probably underestimated and the role of nailfold capillaroscopy in the diagnosis has not been established yet. CI - © 2021 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Abrassart, Tom AU - Abrassart T AD - Department of Internal Medicine and Infectious Diseases Centre Hospitalier Universitaire Ambroise Paré Mons Belgium. FAU - Roland, Thomas AU - Roland T AUID- ORCID: 0000-0002-2660-5476 AD - Department of Internal Medicine and Infectious Diseases Centre Hospitalier Universitaire Ambroise Paré Mons Belgium. FAU - Laurent, France AU - Laurent F AD - Department of Internal Medicine and Infectious Diseases Centre Hospitalier Universitaire Ambroise Paré Mons Belgium. FAU - Rossi, Camelia AU - Rossi C AD - Department of Internal Medicine and Infectious Diseases Centre Hospitalier Universitaire Ambroise Paré Mons Belgium. LA - eng PT - Case Reports PT - Journal Article DEP - 20210202 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC7981682 OTO - NOTNLM OT - acrosyndrome OT - antisynthetase syndrome OT - microangiopathy OT - myalgia COIS- None declared. EDAT- 2021/03/27 06:00 MHDA- 2021/03/27 06:01 PMCR- 2021/02/02 CRDT- 2021/03/26 07:44 PHST- 2020/10/13 00:00 [received] PHST- 2020/12/19 00:00 [revised] PHST- 2020/12/27 00:00 [accepted] PHST- 2021/03/26 07:44 [entrez] PHST- 2021/03/27 06:00 [pubmed] PHST- 2021/03/27 06:01 [medline] PHST- 2021/02/02 00:00 [pmc-release] AID - CCR33790 [pii] AID - 10.1002/ccr3.3790 [doi] PST - epublish SO - Clin Case Rep. 2021 Feb 2;9(3):1446-1449. doi: 10.1002/ccr3.3790. eCollection 2021 Mar. PMID- 27134916 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160502 LR - 20220408 IS - 2249-782X (Print) IS - 0973-709X (Electronic) IS - 0973-709X (Linking) VI - 10 IP - 3 DP - 2016 Mar TI - Antisynthetase Syndrome: A Rare Cause for ILD. PG - OD08-9 LID - 10.7860/JCDR/2016/16872.7361 [doi] AB - Anti-Synthetase Syndrome (ASS) is a rare autoimmune disorder characterized by Interstitial Lung Disease (ILD), inflammatory myositis, fever, Raynaud's phenomenon, mechanic's hand, and inflammatory polyarthritis in the setting of antibodies against amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. It can sometimes present as interstitial lung disease without any other expression of the syndrome. Clinical and radiological features can be similar to atypical pneumonia and could be a challenge to diagnose at an early stage. Prognosis is generally poor, especially when there is associated ILD and delay in diagnosis can lead to increase in morbidity due to progression of pulmonary involvement. We report a patient of ASS presenting with ILD, diagnosed early and treated successfully with immunosupression. FAU - Devi, Hj Gayathri AU - Devi HG AD - Associate Professor, Department of Respiratory Medicine, MS Ramaiah Medical College , Bangalore, India . FAU - Pasha, Md Majeed AU - Pasha MM AD - Resident, Department of Respiratory Medicine, MS Ramaiah Medical College , Bangalore, India . FAU - Padmaja, Mantha Sathya AU - Padmaja MS AD - Resident, Department of Respiratory Medicine, MS Ramaiah Medical College , Bangalore, India . FAU - Halappa, Sujith AU - Halappa S AD - Resident, Department of Respiratory Medicine, MS Ramaiah Medical College , Bangalore, India . LA - eng PT - Case Reports PT - Journal Article DEP - 20160301 PL - India TA - J Clin Diagn Res JT - Journal of clinical and diagnostic research : JCDR JID - 101488993 PMC - PMC4843302 OTO - NOTNLM OT - Anti-jo-1 OT - Cryptogenic OT - Organizing OT - Pneumonia EDAT- 2016/05/03 06:00 MHDA- 2016/05/03 06:01 PMCR- 2016/05/01 CRDT- 2016/05/03 06:00 PHST- 2015/09/18 00:00 [received] PHST- 2016/01/06 00:00 [accepted] PHST- 2016/05/03 06:00 [entrez] PHST- 2016/05/03 06:00 [pubmed] PHST- 2016/05/03 06:01 [medline] PHST- 2016/05/01 00:00 [pmc-release] AID - 10.7860/JCDR/2016/16872.7361 [doi] PST - ppublish SO - J Clin Diagn Res. 2016 Mar;10(3):OD08-9. doi: 10.7860/JCDR/2016/16872.7361. Epub 2016 Mar 1. PMID- 40256100 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250422 IS - 2249-4863 (Print) IS - 2278-7135 (Electronic) IS - 2249-4863 (Linking) VI - 14 IP - 3 DP - 2025 Mar TI - Paraneoplastic systemic sclerosis: A distinct entity or a mere association - A case report. PG - 1128-1130 LID - 10.4103/jfmpc.jfmpc_1527_24 [doi] AB - Systemic sclerosis (SS) is a multi-systemic rheumatological disorder that progresses to interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and scleroderma renal crisis. However, the association between systemic sclerosis and malignancies remains unclear. Although many malignancies can cause skin thickening as a paraneoplastic syndrome, the presence of a positive Scl 70 antibody in this case is intriguing. Traditionally, Scl 70 antibodies have been absent in paraneoplastic SS. However, there have been few reports of diffuse SS with Scl 70 positivity in the context of malignancy. Whether it is a coexisting pathology or a paraneoplastic entity remains debatable. Here, we describe the case of a male in his 50's presenting with diffuse systemic sclerosis with Autosomal Dominant Polycystic Kidney Disease (ADPKD) and hypothyroidism with metastatic epithelial neoplasm of unknown primary origin and a positive autoantibody profile for scleroderma. This case highlights a complex association between SS and malignancy. Although the exact mechanism remains unclear, it is crucial for clinicians to be aware of the possibility of malignancy in patients with SS, especially in those with rapid progression or atypical presentation, irrespective of the autoantibody profiles. Early detection and appropriate management can improve patient outcome. CI - Copyright: © 2025 Journal of Family Medicine and Primary Care. FAU - Sahoo, Debananda AU - Sahoo D AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. FAU - Devi, Sujata AU - Devi S AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. FAU - Anupam, Anurag AU - Anupam A AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. FAU - Dash, Arpita AU - Dash A AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. FAU - Dey, Anupam AU - Dey A AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. FAU - Mishra, Bodhisattwa AU - Mishra B AD - Department of General Medicine, AIIMS, Bhubaneswar, Odisha, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20250325 PL - India TA - J Family Med Prim Care JT - Journal of family medicine and primary care JID - 101610082 PMC - PMC12007760 OTO - NOTNLM OT - Malignancy OT - Raynaud’s phenomenon OT - nail-fold capillaroscopy OT - paraneoplastic systemic sclerosis OT - scleroderma renal crisis COIS- There are no conflicts of interest. EDAT- 2025/04/21 06:25 MHDA- 2025/04/21 06:26 PMCR- 2025/03/01 CRDT- 2025/04/21 05:25 PHST- 2024/09/03 00:00 [received] PHST- 2024/10/20 00:00 [revised] PHST- 2024/10/24 00:00 [accepted] PHST- 2025/04/21 06:26 [medline] PHST- 2025/04/21 06:25 [pubmed] PHST- 2025/04/21 05:25 [entrez] PHST- 2025/03/01 00:00 [pmc-release] AID - JFMPC-14-1128 [pii] AID - 10.4103/jfmpc.jfmpc_1527_24 [doi] PST - ppublish SO - J Family Med Prim Care. 2025 Mar;14(3):1128-1130. doi: 10.4103/jfmpc.jfmpc_1527_24. Epub 2025 Mar 25. PMID- 35276077 OWN - NLM STAT- MEDLINE DCOM- 20230110 LR - 20230224 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 365 IP - 2 DP - 2023 Feb TI - Chronic dyspnea with Raynaud's phenomenon and elevated ANA: A diagnosis of systemic sclerosis sine scleroderma. PG - 198-204 LID - S0002-9629(22)00098-2 [pii] LID - 10.1016/j.amjms.2022.01.023 [doi] AB - Systemic sclerosis (SSc) should be considered in all patients initially diagnosed with idiopathic interstitial lung disease (ILD), even in the absence of classical scleroderma cutaneous features. Systemic sclerosis sine scleroderma (ssSSc) is a rare subtype of SSc, and the diagnosis requires the absence of characteristic skin thickening but the presence of the three following criteria: (A) Raynaud's phenomenon or the equivalent of abnormal nail fold capillaries, (B) positive antinuclear antibody (ANA), typically with nucleolar or speckled immunofluorescence pattern, and (C) at least one internal organ involvement of ILD, renal dysfunction, esophageal/bowel dysmotility or pulmonary arterial hypertension; in the absence of an alternative rheumatological diagnosis. The radiological and histopathological features of systemic sclerosis sine scleroderma-associated interstitial lung disease (ssSSc-ILD) are commonly those of non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) that cannot help distinguish between idiopathic interstitial pneumonia, different types of connective tissue diseases, or even different subsets of SSc. Therefore, other than chest imaging, the use of nail fold capillaroscopy, positive serum ANA antibody, echocardiogram, and esophagram are essential, in conjunction with the clinical presentation for facilitating the diagnosis of ssSSc. We present a case of a 58-year-old woman presenting with chronic dyspnea, a positive review of systems for Raynaud's phenomenon, and found to have elevated nucleolar immunofluorescence pattern of ANA with chest imaging consistent with the diagnosis of ssSSc-ILD. The uniqueness of this case is that despite symptomatic alleviation with oral mycophenolate therapy, our patient's restrictive lung disease on pulmonary function tests continued to decline, requiring initiation of oral nintedanib therapy leading to stability and improvement. However, due to the rarity of ssSSc, the use of oral nintedanib for systemic sclerosis-associated ILD has only been formally assessed on patients with diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis. CI - Copyright © 2022. Published by Elsevier Inc. FAU - Chong, Woon Hean AU - Chong WH AD - Department of Pulmonary and Critical Care Medicine; Albany Medical Center, 43 New Scotland Avenue, 12208 Albany, NY, USA. Electronic address: Keenan_chong_woon_hean@nuhs.edu.sg. FAU - Saha, Biplab K AU - Saha BK AD - Department of Pulmonary and Critical Care, Ozarks Medical Center, West Plains, MO, USA. FAU - Beegle, Scott AU - Beegle S AD - Department of Pulmonary and Critical Care Medicine; Albany Medical Center, 43 New Scotland Avenue, 12208 Albany, NY, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20220308 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Female MH - Humans MH - Middle Aged MH - *Scleroderma, Systemic/complications/diagnosis MH - *Scleroderma, Diffuse MH - *Lung Diseases, Interstitial/complications/diagnosis MH - Skin MH - Dyspnea/complications OTO - NOTNLM OT - Systemic sclerosis OT - Systemic sclerosis sine scleroderma OT - Systemic sclerosis sine scleroderma-associated with interstitial lung disease OT - Systemic sclerosis-associated with interstitial lung disease COIS- Declaration of Competing Interest No author has a conflict of interest to disclose. EDAT- 2022/03/12 06:00 MHDA- 2023/01/11 06:00 CRDT- 2022/03/11 20:10 PHST- 2020/12/15 00:00 [received] PHST- 2021/09/10 00:00 [revised] PHST- 2022/01/31 00:00 [accepted] PHST- 2022/03/12 06:00 [pubmed] PHST- 2023/01/11 06:00 [medline] PHST- 2022/03/11 20:10 [entrez] AID - S0002-9629(22)00098-2 [pii] AID - 10.1016/j.amjms.2022.01.023 [doi] PST - ppublish SO - Am J Med Sci. 2023 Feb;365(2):198-204. doi: 10.1016/j.amjms.2022.01.023. Epub 2022 Mar 8. PMID- 24627847 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140314 LR - 20211021 IS - 2277-9175 (Print) IS - 2277-9175 (Electronic) IS - 2277-9175 (Linking) VI - 3 DP - 2014 TI - A case of sine scleroderma with parenchymal lung disease. PG - 39 LID - 10.4103/2277-9175.125728 [doi] LID - 39 AB - Systemic sclerosis sine scleroderma is a subtype of scleroderma, which is characterized by involvement of visceral organs, but no characteristic skin alteration. The involved organs could be kidneys, heart, gastrointestinal system, and lungs. Interstitial lung disease (ILD) is one of the pulmonary manifestations of sine scleroderma. We report a 38-year-old woman presenting with chill, fever, generalized malaise, dyspnea on exertion, and dry cough with a history of Raynaud's phenomenon, who was evaluated by physical examination, spirometry, and computed tomography scan, that all lead to the diagnosis of ILD. Combination of high-titer positive anti-nuclear antibody, high erythrocyte sedimentation rate, positive C-reactive protein, and ILD could be explained by sine scleroderma. FAU - Karimifar, Mansoor AU - Karimifar M AD - Department of Rheumatology, Alzahra Hospital, Isfahan University of Medical Sciences, Soffe Avenue, Isfahan, Iran. FAU - Hashemi, Hourosadat AU - Hashemi H AD - Department of Rheumatology, Alzahra Hospital, Isfahan University of Medical Sciences, Soffe Avenue, Isfahan, Iran. FAU - Karimifar, Mozhgan AU - Karimifar M AD - Department of Rheumatology, Alzahra Hospital, Isfahan University of Medical Sciences, Soffe Avenue, Isfahan, Iran. FAU - Kazizadeh, Amir AU - Kazizadeh A AD - Department of Rheumatology, Alzahra Hospital, Isfahan University of Medical Sciences, Soffe Avenue, Isfahan, Iran. LA - eng PT - Case Reports PT - Journal Article DEP - 20140124 PL - India TA - Adv Biomed Res JT - Advanced biomedical research JID - 101586897 PMC - PMC3949340 OTO - NOTNLM OT - Interstitial lung disease OT - rituximab OT - systemic sclerosis sine scleroderma COIS- Conflict of Interest: None declared. EDAT- 2014/03/15 06:00 MHDA- 2014/03/15 06:01 PMCR- 2014/01/24 CRDT- 2014/03/15 06:00 PHST- 2012/12/06 00:00 [received] PHST- 2013/02/26 00:00 [accepted] PHST- 2014/03/15 06:00 [entrez] PHST- 2014/03/15 06:00 [pubmed] PHST- 2014/03/15 06:01 [medline] PHST- 2014/01/24 00:00 [pmc-release] AID - ABR-3-39 [pii] AID - 10.4103/2277-9175.125728 [doi] PST - epublish SO - Adv Biomed Res. 2014 Jan 24;3:39. doi: 10.4103/2277-9175.125728. eCollection 2014. PMID- 21794387 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20121031 IS - 1699-258X (Print) IS - 1699-258X (Linking) VI - 2 Suppl 3 DP - 2006 Nov TI - [Treatment of systemic sclerosis]. PG - S42-5 LID - 10.1016/S1699-258X(06)73107-2 [doi] AB - Treatment of systemic sclerosis is based upon 3 types of medications modifiers of disease: drugs which prevent vascular damage, antifibrotric agents, and immunomodulators and immunosupressors. Drugs that prevent vascular damage such as: calcium antagonists, prostaglandins analogues, receptors of endothelin blockers (bosentan), inhibitors of angiotensin converting enzyme, receptors of angiotensin antagonists and inhibitors of 5'-phosphodiesterase have been successful in treating the Raynaud's phenomenon, renal crisis and pulmonary arterial hypertension. In contrast, the results of treatment of fibrosis are discouraging and the D-penicilamine continues being a matter of controversy. The immunosupressor therapy with cyclophosphamide and the transplant of hematopoietic cells, may be beneficial. The knowledge of the pathogenesis of systemic sclerosis to molecular level will lead to new treatment strategies. CI - Copyright © 2006 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved. FAU - Medrano Ramírez, Gabriel AU - Medrano Ramírez G AD - Departamento de Reumatología. Hospital General de México. SS. México DF. México. FAU - Lidia Vera-Lastra, Olga AU - Lidia Vera-Lastra O FAU - Jara, Luis J AU - Jara LJ LA - spa PT - English Abstract PT - Journal Article TT - Tratamiento de la esclerosis sistémica. DEP - 20081210 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 EDAT- 2006/11/01 00:00 MHDA- 2006/11/01 00:01 CRDT- 2011/07/29 06:00 PHST- 2011/07/29 06:00 [entrez] PHST- 2006/11/01 00:00 [pubmed] PHST- 2006/11/01 00:01 [medline] AID - S1699-258X(06)73107-2 [pii] AID - 10.1016/S1699-258X(06)73107-2 [doi] PST - ppublish SO - Reumatol Clin. 2006 Nov;2 Suppl 3:S42-5. doi: 10.1016/S1699-258X(06)73107-2. Epub 2008 Dec 10. PMID- 33458769 OWN - NLM STAT- MEDLINE DCOM- 20210628 LR - 20210628 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 60 IP - 4 DP - 2021 Apr 6 TI - The aetiopathogenic significance, clinical relevance and therapeutic implications of vasculopathy in idiopathic inflammatory myopathy. PG - 1593-1607 LID - 10.1093/rheumatology/keaa816 [doi] AB - It is 120 years since 'angiomyositis' was included alongside 'polymyositis' and 'dermatomyositis' in an attempt to propose a taxonomy that reflected the major clinical characteristics of idiopathic inflammatory myopathy (IIM). Endothelial injury, perivascular inflammation and capillary loss are important histological findings in affected tissues in IIM. Overt vascular clinical features including RP and abnormal nailfold capillaroscopy (NC) are also common in IIM. Despite the presence of endothelial injury, perivascular inflammation and capillary loss in affected tissues in IIM, and the presence of clinical features such as RP and NC abnormalities, the pathogenic and therapeutic implications of vasculopathy in IIM have been somewhat overlooked. RP and NC abnormalities are not always present, providing a valuable opportunity to explore aetiopathogenic factors driving vasculopathy within autoimmune rheumatic disease. The present review examines the aetiopathogenic, prognostic and therapeutic significance of vasculopathy in IIM. We describe the prevalence and clinical relevance of vasculopathy in IIM, and consider how vasculopathy may be better utilized to support improved IIM diagnosis and disease classification. Areas of unmet research need are highlighted where relevant. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (Part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Christopher-Stine, Lisa AU - Christopher-Stine L AD - Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM EIN - Rheumatology (Oxford). 2021 Aug 2;60(8):3960. doi: 10.1093/rheumatology/keab431. PMID: 34056651 MH - Capillaries/*pathology MH - Dermatomyositis/pathology MH - Humans MH - Myositis/*pathology MH - Polymyositis/pathology MH - Vascular Diseases/*pathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - classification OT - dermatomyositis OT - inflammatory myopathy OT - nailfold capillaroscopy OT - polymyositis OT - vasculopathy EDAT- 2021/01/19 06:00 MHDA- 2021/06/29 06:00 CRDT- 2021/01/18 05:37 PHST- 2020/07/08 00:00 [received] PHST- 2020/09/16 00:00 [revised] PHST- 2020/10/15 00:00 [accepted] PHST- 2021/01/19 06:00 [pubmed] PHST- 2021/06/29 06:00 [medline] PHST- 2021/01/18 05:37 [entrez] AID - 6103235 [pii] AID - 10.1093/rheumatology/keaa816 [doi] PST - ppublish SO - Rheumatology (Oxford). 2021 Apr 6;60(4):1593-1607. doi: 10.1093/rheumatology/keaa816. PMID- 24218729 OWN - NLM STAT- MEDLINE DCOM- 20131217 LR - 20131113 IS - 0012-7183 (Print) IS - 0012-7183 (Linking) VI - 129 IP - 19 DP - 2013 TI - [Systemic sclerosis]. PG - 1981-91 AB - Systemic sclerosis (scleroderma) is a rare connective tissue disease characterized by vasculopathy and immune dysfunction, leading to fibrosis with damage of multiple organs. Two major clinical subtypes are the diffuse and limited forms. The combination of Raynaud's phenomenon, puffy fingers and positive antinuclear antibodies are red flag features that should alert the clinician to the presence of very early systemic sclerosis, which can be treated with vasodilator, antithrombotic, and immunosuppressive drugs. Progress has been made even in the management of the most severe manifestations, including interstitial lung disease, pulmonary artery hypertension and scleroderma renal crisis. FAU - Peltomaa, Ritva AU - Peltomaa R AD - HUS Yleissisätautien Klininen Laitos. FAU - Pettersson, Tom AU - Pettersson T FAU - Tuompo, Riitta AU - Tuompo R FAU - Luosujärvi, Riitta AU - Luosujärvi R LA - fin PT - English Abstract PT - Journal Article PT - Review TT - Systeeminen skleroosi. PL - Finland TA - Duodecim JT - Duodecim; laaketieteellinen aikakauskirja JID - 0373207 RN - 0 (Fibrinolytic Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Diagnosis, Differential MH - Fibrinolytic Agents/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Prognosis MH - Scleroderma, Systemic/complications/*diagnosis/drug therapy MH - Vasodilator Agents/therapeutic use EDAT- 2013/11/14 06:00 MHDA- 2013/12/18 06:00 CRDT- 2013/11/14 06:00 PHST- 2013/11/14 06:00 [entrez] PHST- 2013/11/14 06:00 [pubmed] PHST- 2013/12/18 06:00 [medline] PST - ppublish SO - Duodecim. 2013;129(19):1981-91. PMID- 39838473 OWN - NLM STAT- MEDLINE DCOM- 20250122 LR - 20250623 IS - 1466-609X (Electronic) IS - 1364-8535 (Print) IS - 1364-8535 (Linking) VI - 29 IP - 1 DP - 2025 Jan 21 TI - The association of capillary refill time and return of spontaneous circulation during out-of-hospital cardiac arrest: an observational study. PG - 37 LID - 10.1186/s13054-025-05255-4 [doi] LID - 37 AB - INTRODUCTION: Microcirculatory alterations are predictive of poor outcomes in patients with shock and after cardiac arrest in animal models. However, microcirculatory alterations during human cardiac arrest have not yet been studied. METHODS: We prospectively included adult patients receiving resuscitation after witnessed out-of-hospital cardiac arrest. Exclusion criteria were hypovolemia, hypo- or hyperthermia (< 34.0 °C, > 37.5 °C), peripheral arterial disease, Raynaud's disease, and logistical issues (e.g., shortage of space). Capillary refill time was measured on the finger (CRT-F) and the earlobe (CRT-E) every other minute until return of spontaneous circulation (any ROSC) or termination of resuscitation. The primary endpoint was any ROSC, secondary endpoints were 30-day-mortality and good neurological outcome (defined as cerebral performance category 1-2). Based on the data structure, CRT-F and CRT-E values were grouped post-hoc into quartiles and tertiles. A cluster-robust standard error logistic regression was performed for the primary outcome. Trend analyses were made for each individual. RESULTS: After screening of 141 patients, 50 were included in the analysis (median age 75 years, 28% female, any ROSC 32%). The median CRT-F was > 10 [7-> 10] seconds; the median CRT-E was 3 [3-4] seconds. The any ROSC rate for patients in CRT-F quartile 1 (3-5 s) was 71.4%, 31.7% in quartile 2 (6-8 s), 23.1% in quartile 3 (9-10 s), and 10% in quartile 4 (> 10 s). The odds ratio of 0.39 (95% CI 0.20-0.73, p = 0.004) indicated, that with an increase of CRT-F by a quartile, the chance of achieving any ROSC decreased by 61%. Patients with a decreasing CRT-F achieved any ROSC in 70%, whereas patients with constant or increasing CRT-F had any ROSC in only 21% (p = 0.008). In contrast, CRT-E showed no association with any ROSC (T1 [1-2 s.]: 16.7%, T2 [3 s.]: 27.5%, T3 [4-> 10 s.]: 22.4%, OR by tertiles: 1.18, 95% CI 0.58-2.44, p = 0.646). CONCLUSION: During out-of-hospital cardiac arrest, shorter CRT-F, but not CRT-E, is associated with a higher chance of any ROSC. TRIAL REGISTRATION: This trial was pre-registered on clinicaltrials.gov with the number: NCT04791995 on March 2nd, 2021. CI - © 2025. The Author(s). FAU - Mueller, Matthias AU - Mueller M AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Holzer, Michael AU - Holzer M AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. Michael.holzer@meduniwien.ac.at. FAU - Losert, Heidrun AU - Losert H AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Grassmann, Daniel AU - Grassmann D AD - Emergency Medical Service Vienna, Vienna, Austria. AD - PULS - Austrian Cardiac Arrest Awareness Association, Vienna, Austria. FAU - Ettl, Florian AU - Ettl F AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. AD - PULS - Austrian Cardiac Arrest Awareness Association, Vienna, Austria. FAU - Gatterbauer, Mathias AU - Gatterbauer M AD - Emergency Medical Service Vienna, Vienna, Austria. FAU - Magnet, Ingrid AU - Magnet I AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Nuernberger, Alexander AU - Nuernberger A AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Kienbacher, Calvin Lukas AU - Kienbacher CL AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Gelbenegger, Georg AU - Gelbenegger G AD - Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria. FAU - Girsa, Michael AU - Girsa M AD - Emergency Medical Service Vienna, Vienna, Austria. FAU - Herkner, Harald AU - Herkner H AD - Department of Emergency Medicine, Medical University of Vienna, Waehringer Guertel 18-20/6D, 1090, Vienna, Austria. FAU - Krammel, Mario AU - Krammel M AD - Emergency Medical Service Vienna, Vienna, Austria. AD - PULS - Austrian Cardiac Arrest Awareness Association, Vienna, Austria. LA - eng SI - ClinicalTrials.gov/NCT04791995 GR - 21171/Medical Scientific Fund of the Mayor of the City of Vienna/ PT - Journal Article PT - Observational Study DEP - 20250121 PL - England TA - Crit Care JT - Critical care (London, England) JID - 9801902 SB - IM MH - Humans MH - *Out-of-Hospital Cardiac Arrest/physiopathology/therapy/mortality MH - Female MH - Male MH - Aged MH - Middle Aged MH - Prospective Studies MH - *Return of Spontaneous Circulation/physiology MH - *Capillaries/physiology/physiopathology MH - *Microcirculation/physiology MH - Time Factors MH - Cardiopulmonary Resuscitation/methods PMC - PMC11748279 OTO - NOTNLM OT - Capillary refill time OT - Cardiac arrest OT - Microcirculation OT - Out-of-hospital cardiac arrest OT - Resuscitation outcomes OT - Return of spontaneous circulation COIS- Declarations. Ethics approval and consent to participate: This study and related amendments were submitted and approved by the ethics committee of the Medical University of Vienna (EK 2427/2020) and the ethics committee of the City of Vienna (EK 21–050-0321). This trial was pre-registered at clinicaltrials.gov under the number NCT04791995 and conducted in full conformance with the ‘Declaration of Helsinki’ and its amendments. Initial consent was waived. Post-hoc written informed consent was obtained as soon as possible after neurological recovery. Patients who died prior to a possible informed consent were included in the study. Competing Interests: The authors declare no competing interests. EDAT- 2025/01/22 06:26 MHDA- 2025/01/22 06:27 PMCR- 2025/01/21 CRDT- 2025/01/22 00:01 PHST- 2024/09/18 00:00 [received] PHST- 2025/01/04 00:00 [accepted] PHST- 2025/01/22 06:27 [medline] PHST- 2025/01/22 06:26 [pubmed] PHST- 2025/01/22 00:01 [entrez] PHST- 2025/01/21 00:00 [pmc-release] AID - 10.1186/s13054-025-05255-4 [pii] AID - 5255 [pii] AID - 10.1186/s13054-025-05255-4 [doi] PST - epublish SO - Crit Care. 2025 Jan 21;29(1):37. doi: 10.1186/s13054-025-05255-4. PMID- 22424193 OWN - NLM STAT- MEDLINE DCOM- 20130410 LR - 20220317 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 26 IP - 1 DP - 2012 Feb TI - Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. PG - 61-72 LID - 10.1016/j.berh.2012.01.009 [doi] AB - The most common clinical manifestations of mixed connective disease are Raynaud's phenomenon, arthralgias, swollen joints, esophageal dysfunction, muscle weakness and fingers sausage-like appearance together with the presence of anti-ribonucleoprotein (RNP) antibodies. However, organ involvement is more extensive than first descriptions reported. The disease can be serious with development of pulmonary, kidney, cardiovascular, gastrointestinal and central nervous system manifestations. The worst prognosis and high mortality are associated with the presence of pulmonary disease. Although a different set of clinical criteria have been proposed, there is no consensus about the most accurate. There is no full agreement about treatment and the initial impression of a satisfactory response to low doses of steroids is not always the rule. Herein, we review available evidence to a better approach to all previous topics. CI - © 2012. Published by Elsevier Ltd. All rights reserved. FAU - Ortega-Hernandez, Oscar-Danilo AU - Ortega-Hernandez OD AD - The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel. FAU - Shoenfeld, Yehuda AU - Shoenfeld Y LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/immunology MH - Humans MH - *Mixed Connective Tissue Disease/diagnosis/immunology/therapy EDAT- 2012/03/20 06:00 MHDA- 2013/04/11 06:00 CRDT- 2012/03/20 06:00 PHST- 2012/01/03 00:00 [revised] PHST- 2012/01/04 00:00 [accepted] PHST- 2012/03/20 06:00 [entrez] PHST- 2012/03/20 06:00 [pubmed] PHST- 2013/04/11 06:00 [medline] AID - S1521-6942(12)00010-1 [pii] AID - 10.1016/j.berh.2012.01.009 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2012 Feb;26(1):61-72. doi: 10.1016/j.berh.2012.01.009. PMID- 37364617 OWN - NLM STAT- MEDLINE DCOM- 20240214 LR - 20250219 IS - 1097-6787 (Electronic) IS - 0190-9622 (Linking) VI - 90 IP - 3 DP - 2024 Mar TI - Erythromelalgia. Part I: Pathogenesis, clinical features, evaluation, and complications. PG - 453-462 LID - S0190-9622(23)01186-6 [pii] LID - 10.1016/j.jaad.2023.02.071 [doi] AB - Erythromelalgia is a rare pain disorder that is underrecognized and difficult-to-treat. It is characterized by episodes of extremity erythema and pain that can be disabling; it may be genetic, related to an underlying systemic disease, or idiopathic. Considering the prominent cutaneous features characteristic of the condition, dermatologists can play an important role in early recognition and limitation of morbidity. The first article in this 2-part continuing medical education series reviews the epidemiology, pathogenesis, clinical manifestations, evaluation, and complications. CI - Published by Elsevier Inc. FAU - Caldito, Elena Gonzalez AU - Caldito EG AD - Division of Dermatology, John H Stroger Hospital of Cook County, Chicago, Illinois. FAU - Kaul, Subuhi AU - Kaul S AD - Division of Dermatology, John H Stroger Hospital of Cook County, Chicago, Illinois. FAU - Caldito, Natalia Gonzalez AU - Caldito NG AD - Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. FAU - Piette, Warren AU - Piette W AD - Division of Dermatology, John H Stroger Hospital of Cook County, Chicago, Illinois; Department of Dermatology, Rush University Medical Center, Chicago, Illinois. FAU - Mehta, Shilpa AU - Mehta S AD - Division of Dermatology, John H Stroger Hospital of Cook County, Chicago, Illinois. Electronic address: shilpaderm@gmail.com. LA - eng PT - Journal Article PT - Review DEP - 20230624 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 SB - IM MH - Humans MH - *Erythromelalgia/diagnosis/epidemiology/etiology MH - Pain/diagnosis/etiology MH - Erythema MH - Skin/pathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - SCN9A gene OT - complex regional pain syndrome OT - erythermalgia OT - erythromelalgia OT - red ear syndrome OT - voltage gated sodium channel COIS- Conflicts of interest None disclosed. EDAT- 2023/06/27 01:06 MHDA- 2024/02/12 05:43 CRDT- 2023/06/26 19:12 PHST- 2022/10/12 00:00 [received] PHST- 2023/01/27 00:00 [revised] PHST- 2023/02/12 00:00 [accepted] PHST- 2024/02/12 05:43 [medline] PHST- 2023/06/27 01:06 [pubmed] PHST- 2023/06/26 19:12 [entrez] AID - S0190-9622(23)01186-6 [pii] AID - 10.1016/j.jaad.2023.02.071 [doi] PST - ppublish SO - J Am Acad Dermatol. 2024 Mar;90(3):453-462. doi: 10.1016/j.jaad.2023.02.071. Epub 2023 Jun 24. PMID- 29087124 OWN - NLM STAT- MEDLINE DCOM- 20180716 LR - 20210616 IS - 1668-3501 (Electronic) IS - 0325-0075 (Linking) VI - 115 IP - 6 DP - 2017 Dec 1 TI - [Antiphospholipid syndrome in Pediatrics: a case report]. PG - e412-e415 LID - 10.5546/aap.2017.e412 [doi] AB - The antiphospholipid syndrome is a multisystem autoimmune disease in which autoantibodies against a variety of phospholipids and phospholipid binding proteins are produced. It occurs in 1.8% of the population and only 2% of the cases are pediatric. The spectrum of clinical manifestations is wide from asymptomatic patients to a life-threatening disease like the catastrophic antiphospholipid syndrome. Any organ can be affected. The most frequent manifestations in pediatrics correspond to venous thrombosis in 60% of patients, arterial thrombosis in 32%, hematological disease in 38% (thrombocytopenia, leucopenia), skin alterations in 18% (livedo reticularis, Raynaud's phenomenon) and neurological signs in 16%. We describe the case of a previously healthy 14-year-old female patient diagnosed with antiphospholipid syndrome. CI - Sociedad Argentina de Pediatría. FAU - Micheletti, Eugenia AU - Micheletti E AD - Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires. FAU - Blanco, Luciana AU - Blanco L AD - Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires. FAU - Cechinni, Ana AU - Cechinni A AD - Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires. FAU - Acerenza, Marcelo AU - Acerenza M AD - Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires. FAU - Canda, Paula AU - Canda P AD - Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires. paulacanda@hotmail.com. LA - spa PT - Case Reports PT - Journal Article TT - Síndrome antifosfolipídico en pediatría: a propósito de un caso clínico. PL - Argentina TA - Arch Argent Pediatr JT - Archivos argentinos de pediatria JID - 0372460 SB - IM MH - Adolescent MH - Antiphospholipid Syndrome/complications/*diagnosis MH - Female MH - Humans MH - Pulmonary Embolism/*diagnostic imaging/etiology OTO - NOTNLM OT - antiphospholipid syndrome OT - pediatrics EDAT- 2017/11/01 06:00 MHDA- 2018/07/17 06:00 CRDT- 2017/11/01 06:00 PHST- 2016/11/04 00:00 [received] PHST- 2017/06/13 00:00 [accepted] PHST- 2017/11/01 06:00 [entrez] PHST- 2017/11/01 06:00 [pubmed] PHST- 2018/07/17 06:00 [medline] AID - 10.5546/aap.2017.e412 [doi] PST - ppublish SO - Arch Argent Pediatr. 2017 Dec 1;115(6):e412-e415. doi: 10.5546/aap.2017.e412. PMID- 27828653 OWN - NLM STAT- MEDLINE DCOM- 20170530 LR - 20220408 IS - 1806-4841 (Electronic) IS - 0365-0596 (Print) IS - 0365-0596 (Linking) VI - 91 IP - 5 DP - 2016 Sep-Oct TI - Syndrome in question: antisynthetase syndrome (anti-PL-7). PG - 683-685 LID - S0365-05962016000500683 [pii] LID - 10.1590/abd1806-4841.20164449 [doi] AB - Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies. FAU - Esposito, Ana Cláudia Cavalcante AU - Esposito AC AD - Universidade Estadual Paulista "Júlio de Mesquita Filho" (Unesp), Botucatu, SP, Brazil. FAU - Gige, Tatiana Cristina AU - Gige TC AD - Universidade Estadual Paulista "Júlio de Mesquita Filho" (Unesp), Botucatu, SP, Brazil. FAU - Miot, Hélio Amante AU - Miot HA AD - Universidade Estadual Paulista "Júlio de Mesquita Filho" (Unesp), Botucatu, SP, Brazil. LA - eng PT - Case Reports PT - Journal Article PL - Spain TA - An Bras Dermatol JT - Anais brasileiros de dermatologia JID - 0067662 RN - Antisynthetase syndrome SB - IM MH - Adult MH - Female MH - Hand Dermatoses/complications MH - Humans MH - Lung Diseases, Interstitial/complications/diagnostic imaging MH - Myositis/complications/*diagnosis MH - Nail Diseases/complications MH - Tomography PMC - PMC5087238 COIS- None EDAT- 2016/11/10 06:00 MHDA- 2017/05/31 06:00 PMCR- 2016/09/01 CRDT- 2016/11/10 06:00 PHST- 2015/02/08 00:00 [received] PHST- 2015/04/22 00:00 [accepted] PHST- 2016/11/10 06:00 [entrez] PHST- 2016/11/10 06:00 [pubmed] PHST- 2017/05/31 06:00 [medline] PHST- 2016/09/01 00:00 [pmc-release] AID - S0365-05962016000500683 [pii] AID - 10.1590/abd1806-4841.20164449 [doi] PST - ppublish SO - An Bras Dermatol. 2016 Sep-Oct;91(5):683-685. doi: 10.1590/abd1806-4841.20164449. PMID- 29497844 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 38 IP - 8 DP - 2018 Aug TI - Pain, fatigue and functional disability are associated with higher educational needs in systemic sclerosis: a cross-sectional study. PG - 1471-1478 LID - 10.1007/s00296-018-3998-0 [doi] AB - In the process of the planned and systematic education of patients/families, it is extremely important to identify patients' health problems as well as their needs and expectations. The objective of this study was to determine the relationship between functional disability, health problems and perceived educational needs in people with systemic sclerosis (SSc). This was a cross-sectional analytic study conducted in six rheumatology centers in Poland. Functional disability was measured using HAQ-DI, and the magnitude of other health problems (pain, fatigue, intestinal problems, breathing problems, Raynaud's phenomenon, finger ulcerations) was measured using 0-100 mm visual analogue scales. The educational needs were measured using the Polish version of the Educational Needs Assessment Tool (Pol-ENAT). Spearman's correlation coefficient (r(s)) was used to report associations. The sample comprised 140 patients, 125 (89.28%) were women. They had a mean (SD) age of 54 (14.23) and disease duration of 11 (10.27) years. The median (IQR) HAQ-DI was 1.12 (0.62-1.62) and mean ENAT score was 71.54 (SD 27.72). Patients needed to know more about the disease process, self-help measures and managing pain. All health problems had significant correlations with the overall educational needs, pain, functional disability and fatigue having the highest r(s) = 0.359, p < 0.0001; r(s) = 0.314, p < 0.001 and r(s) = 0.270, p = 0.001, respectively. Health problems in people with SSc are associated with considerable educational needs; therefore, health professionals should take this into account when planning patient education. Group interventions should consider providing patient education related to disease process as a minimum. FAU - Sierakowska, Matylda AU - Sierakowska M AUID- ORCID: 0000-0003-2816-8588 AD - Department of Integrated Care Medical, Medical University of Bialystok, 7a Maria Sklodowska-Curie Street, 15-096, Bialystok, Poland. matyldasierakowska@gmail.com. FAU - Sierakowski, Stanisław AU - Sierakowski S AUID- ORCID: 0000-0002-4310-3777 AD - Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland. FAU - Sierakowska, Justyna AU - Sierakowska J AUID- ORCID: 0000-0003-0446-7188 AD - Department of Foreign Languages, Medical University of Bialystok, Bialystok, Poland. FAU - Krajewska-Kułak, Elżbieta AU - Krajewska-Kułak E AUID- ORCID: 0000-0002-9425-2430 AD - Department of Integrated Care Medical, Medical University of Bialystok, 7a Maria Sklodowska-Curie Street, 15-096, Bialystok, Poland. FAU - Ndosi, Mwidimi AU - Ndosi M AUID- ORCID: 0000-0002-7764-3173 AD - Department of Nursing and Midwifery, University of the West of England, Bristol, UK. AD - Academic Rheumatology Unit, University Hospitals Bristol, Bristol, UK. LA - eng GR - N/ST/ZB/17/002/3310/Uniwersytet Medyczny w Bialymstoku/ PT - Journal Article DEP - 20180301 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Cross-Sectional Studies MH - Disability Evaluation MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain MH - Patient Education as Topic/*organization & administration MH - Poland MH - Quality of Life MH - Scleroderma, Systemic/*physiopathology/psychology MH - Severity of Illness Index MH - Surveys and Questionnaires PMC - PMC6060785 OTO - NOTNLM OT - Disability OT - Educational needs OT - Fatigue OT - Pain OT - Raynaud’s phenomenon OT - Systemic sclerosis COIS- All authors declare that they have no competing interests. EDAT- 2018/03/03 06:00 MHDA- 2018/12/12 06:00 PMCR- 2018/03/01 CRDT- 2018/03/03 06:00 PHST- 2017/12/06 00:00 [received] PHST- 2018/02/17 00:00 [accepted] PHST- 2018/03/03 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2018/03/03 06:00 [entrez] PHST- 2018/03/01 00:00 [pmc-release] AID - 10.1007/s00296-018-3998-0 [pii] AID - 3998 [pii] AID - 10.1007/s00296-018-3998-0 [doi] PST - ppublish SO - Rheumatol Int. 2018 Aug;38(8):1471-1478. doi: 10.1007/s00296-018-3998-0. Epub 2018 Mar 1. PMID- 35079180 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240823 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 59 IP - 6 DP - 2021 TI - Juvenile systemic sclerosis - observations of one clinical centre. PG - 367-372 LID - 10.5114/reum.2021.112350 [doi] AB - OBJECTIVES: The systemic form of scleroderma (SSc) in children is a very rare disease; therefore, it is recognized relatively late, which increases the risk of complications. The aim of the study was to assess the clinical symptoms of juvenile systemic sclerosis (JSSc) in our cohort patients. MATERIAL AND METHODS: A group of (N = 22) scleroderma patients aged between 2 and 16 years were observed. Demographic data and all clinical results obtained during 16 years of observation in the clinic of rheumatic diseases of developmental age were collected and analysed. RESULTS: In all observed children the major JSSc criterion was found, i.e. skin thickening proximal to the metacarpal phalangeal and/or metatarsophalangeal joints. Other symptoms are presented as follows: nailfold capillary abnormalities - 100%, Raynaud's phenomenon - 90.9%, sclerodactyly - 27.3%, digital tip ulcers - 27.3%, dysphagia - 18.2%, gastroesophageal reflux - 27.3% (assessed in only 10 children), arrhythmias - 22.7%, heart failure - 9.1%, new-onset arterial hypertension - 9.1%, pulmonary fibrosis - 72.7%, pulmonary arterial hypertension - 9.1%, neuropathy - 13.6%, carpal tunnel syndrome - 4.5%, tendon friction rubs - 4.5%, arthritis - 22.7%, and myositis - 13.6%. There were no cases of renal crisis. Decreased diffusing capacity of oxygen was confirmed in 12 patients (58.3%). The presence of antinuclear antibodies was noticed in 86.7% of patients, and among SSc selective autoantibodies: anticentromere - 31.8%, anti-topoisomerase I - 18.2%, anti-PM-Scl 100 or 75 - 45.5%, anti-RP11, Th/To, PCNA in total in 27.3% were presented. In 4.5% of cases, apart from the presence of anti-PM-Scl autoantibodies, positive lupus band test, reduced concentration of complement, and antiphospholipid antibodies were also found. In 59% of studied children, the body mass index was below the 25th percentile. CONCLUSIONS: The presented retrospective analysis shows that the occurrence of Raynaud's phenomenon with changes in nailfold capillaroscopy is the best screening toll for the assessment of risk of JSSc. All patients of developmental age with Raynaud's phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests. CI - Copyright: © 2021 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. FAU - Rutkowska-Sak, Lidia AU - Rutkowska-Sak L AD - Clinic of Developmental-Age Rheumatology, National Institute of Geriatrics, Rheumatology, and Rehabilitation, Warsaw, Poland. FAU - Gietka, Piotr AU - Gietka P AD - Clinic of Developmental-Age Rheumatology, National Institute of Geriatrics, Rheumatology, and Rehabilitation, Warsaw, Poland. FAU - Gazda, Agnieszka AU - Gazda A AD - Clinic of Developmental-Age Rheumatology, National Institute of Geriatrics, Rheumatology, and Rehabilitation, Warsaw, Poland. FAU - Kołodziejczyk, Beata AU - Kołodziejczyk B AD - Clinic of Developmental-Age Rheumatology, National Institute of Geriatrics, Rheumatology, and Rehabilitation, Warsaw, Poland. LA - eng PT - Journal Article DEP - 20220112 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC8768038 OTO - NOTNLM OT - Raynaud’s phenomenon OT - capillaroscopy OT - clinical picture OT - juvenile systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2022/01/27 06:00 MHDA- 2022/01/27 06:01 PMCR- 2021/01/01 CRDT- 2022/01/26 05:30 PHST- 2021/09/01 00:00 [received] PHST- 2021/12/06 00:00 [accepted] PHST- 2022/01/26 05:30 [entrez] PHST- 2022/01/27 06:00 [pubmed] PHST- 2022/01/27 06:01 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 46067 [pii] AID - 10.5114/reum.2021.112350 [doi] PST - ppublish SO - Reumatologia. 2021;59(6):367-372. doi: 10.5114/reum.2021.112350. Epub 2022 Jan 12. PMID- 22747554 OWN - NLM STAT- MEDLINE DCOM- 20130521 LR - 20120703 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 41 IP - 2 DP - 2011 Feb TI - Antitopoisomerase antibody positivity predates nailfold capillaroscopy abnormalities in scleroderma. Postulated classification of 'prescleroderma'. PG - 197-9 LID - 10.1111/j.1445-5994.2010.02381.x [doi] AB - In a patient with early topoisomerase antibody-positive scleroderma, antinuclear antibody positivity was fortuitously observed to predate nailfold capillaroscopy changes. Using this case as a template, the prediagnostic phase of the presumed multifactorial disease may be divided into 5 temporal phases--phase 1 representing conception and intrauterine environment, phase 2 representing the extrauterine environment predating environmental exposure; phase 3 representing the early post-environmental exposure interval with no detectable perturbed body status; phase 4 representing the post-environmental exposure interval characterized by autoantibody production and microvascular changes, and phase 5, the symptomatic clinical prediagnostic interval (Raynaud's, skin, musculoskeletal, gastrointestinal, cardiorespiratory) prompting scleroderma diagnosis. Temporal classification of prescleroderma aids in both the understanding and definition of scleroderma 'onset'. If altered nailfold capillaries and autoantibodies develop at comparable rates, and if the findings from this case--that autoantibody changes precede microvascular changes--are truly representative of the preclinical disease phase, then these findings argue that the evolution of the disease is from within the vessel outwards, rather than vice versa. CI - © 2011 The Authors. Internal Medicine Journal © 2011 Royal Australasian College of Physicians. FAU - Englert, H AU - Englert H AD - Rheumatology Department, Westmead Hospital, Sydney, New South Wales, Australia. Email: helen.englert@gmail.com FAU - Champion, D AU - Champion D FAU - Wu, J C AU - Wu JC FAU - Giallussi, J AU - Giallussi J FAU - McGrath, M AU - McGrath M FAU - Manolios, N AU - Manolios N LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - 0 (Autoantibodies) RN - EC 5.99.1.- (DNA Topoisomerases) SB - IM MH - Adult MH - Autoantibodies/*biosynthesis MH - DNA Topoisomerases/*immunology MH - Female MH - Humans MH - *Microscopic Angioscopy/methods MH - Nails/blood supply/immunology/*pathology MH - Scleroderma, Systemic/*classification/*enzymology/immunology EDAT- 2011/02/01 00:00 MHDA- 2013/05/23 06:00 CRDT- 2012/07/04 06:00 PHST- 2012/07/04 06:00 [entrez] PHST- 2011/02/01 00:00 [pubmed] PHST- 2013/05/23 06:00 [medline] AID - 10.1111/j.1445-5994.2010.02381.x [doi] PST - ppublish SO - Intern Med J. 2011 Feb;41(2):197-9. doi: 10.1111/j.1445-5994.2010.02381.x. PMID- 18068860 OWN - NLM STAT- MEDLINE DCOM- 20080313 LR - 20071210 IS - 1521-6942 (Print) IS - 1521-6942 (Linking) VI - 21 IP - 6 DP - 2007 Dec TI - Mixed connective tissue disease: what is behind the curtain? PG - 1037-49 AB - Although there is still an emotional debate over the existence of mixed connective tissue disease, the evidence from animal models suggests that anti-U1RNP antibodies, similar to other autoantibodies in other connective tissue diseases (such as antisynthetase, anticentromere, and antitopoisomerase), play a pathophysiological role in this disease. Despite an antiendothelial effect of anti-U1RNP antibodies, which is reminiscent of anticentromere antibodies, patients with high-titer autoantibodies to U1RNP in the absence of anti-Sm antibodies do not usually have or develop typical systemic sclerosis. Instead, their severe Raynaud's syndrome is commonly accompanied by arthritis, which can be erosive, and by swollen/puffy hands and myositis. Pulmonary arterial hypertension is the major life-threatening complication in these patients and regular screening for this condition is essential. FAU - Aringer, Martin AU - Aringer M AD - Division of Rheumatology, Department of Medicine III, University Center Carl Gustav Carus, Technical University of Dresden, Germany. martin.aringer@uniklinikum-dresden.de FAU - Smolen, Josef S AU - Smolen JS LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Autoimmunity/*immunology MH - Diagnosis, Differential MH - Humans MH - Immunity, Cellular/immunology MH - *Mixed Connective Tissue Disease/classification/diagnosis/immunology RF - 80 EDAT- 2007/12/11 09:00 MHDA- 2008/03/14 09:00 CRDT- 2007/12/11 09:00 PHST- 2007/12/11 09:00 [pubmed] PHST- 2008/03/14 09:00 [medline] PHST- 2007/12/11 09:00 [entrez] AID - S1521-6942(07)00110-6 [pii] AID - 10.1016/j.berh.2007.10.002 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2007 Dec;21(6):1037-49. doi: 10.1016/j.berh.2007.10.002. PMID- 37958228 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241013 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 13 IP - 21 DP - 2023 Oct 28 TI - A Multidisciplinary Approach as a Goal for the Management of Complications in Systemic Scleroderma: A Literature Review and Case Scenario. LID - 10.3390/diagnostics13213332 [doi] LID - 3332 AB - Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement. Scleroderma has the highest mortality among all rheumatic diseases. The pathophysiology mechanism of systemic sclerosis is a progressive self-amplifying process, which involves widespread microvascular damage, followed by a dysregulation of innate and adaptive immunity and inflammation and diffuse fibrosis of the skin and visceral organs. Fibrosis of internal organs is a hint for systemic sclerosis, moreover associated with interstitial lung disease (SSc-ILD) is a complex process. In order to correlate scientific data from the literature with clinical experience, we present the case of a 56-year-old woman who was diagnosed with systemic sclerosis 16 years ago. The association of numerous comorbidities characterized by a considerable level of seriousness characterizes this case: the highly extensive systemic damage, the cardiovascular impact of the illness, and the existence of severe pulmonary arterial hypertension. The systemic and clinical manifestations, respiratory functional tests, radiological features, and specific therapy are discussed. FAU - Pătrîntașu, Dariana-Elena AU - Pătrîntașu DE AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. FAU - Sárközi, Hédi Katalin AU - Sárközi HK AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. AD - Pneumology Department, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Târgu Mures, 540139 Targu Mures, Romania. FAU - Lupușor, Eugeniu AU - Lupușor E AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. FAU - Vlangăr, Irina Elena AU - Vlangăr IE AD - Cardiology Department, Elias University Emergency Hospital, 011461 Bucharest, Romania. FAU - Rotariu, Gheorghe-Marian AU - Rotariu GM AD - Faculty of Medicine, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Târgu Mures, 540139 Targu Mures, Romania. FAU - Rența, Ionuț-Alexandru AU - Rența IA AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. FAU - Nan, Anda-Nicoleta AU - Nan AN AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. FAU - Budin, Corina Eugenia AU - Budin CE AUID- ORCID: 0000-0002-7785-0121 AD - Pneumology Department, Mures Clinical County Hospital, 540142 Targu Mures, Romania. AD - Pathophysiology Department, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Târgu Mures, 540139 Targu Mures, Romania. LA - eng PT - Journal Article PT - Review DEP - 20231028 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10648338 OTO - NOTNLM OT - Raynaud’s syndrome OT - interstitial lung disease OT - lung diseases OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2023/11/14 06:42 MHDA- 2023/11/14 06:43 PMCR- 2023/10/28 CRDT- 2023/11/14 02:06 PHST- 2023/10/12 00:00 [received] PHST- 2023/10/23 00:00 [revised] PHST- 2023/10/26 00:00 [accepted] PHST- 2023/11/14 06:43 [medline] PHST- 2023/11/14 06:42 [pubmed] PHST- 2023/11/14 02:06 [entrez] PHST- 2023/10/28 00:00 [pmc-release] AID - diagnostics13213332 [pii] AID - diagnostics-13-03332 [pii] AID - 10.3390/diagnostics13213332 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Oct 28;13(21):3332. doi: 10.3390/diagnostics13213332. PMID- 34336409 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210803 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 6 DP - 2021 Jun TI - Diagnosis of Undifferentiated Connective Tissue Disease in a Patient With Digital Gangrene and Positive Antinuclear Antibodies. PG - e15883 LID - 10.7759/cureus.15883 [doi] LID - e15883 AB - The occurrence of ischemia of the digits or digital gangrene is a well-known complication of systemic autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, and anti-phospholipid syndrome, among others. The pathophysiological mechanisms are small vessel vasculitis, vasospasm of Raynaud's phenomenon, microthrombi due to antiphospholipid syndrome, and/or accompanying accelerated atherosclerosis. Digital ischemia can also occur in the context of disseminated bacterial infections and sepsis. We present here the case of a patient who had digital ischemia and positive antinuclear antibodies but without well-defined clinical features of a connective tissue disease. A diagnosis of undifferentiated connective tissue disease was made. CI - Copyright © 2021, Ravi et al. FAU - Ravi, Pradeep AU - Ravi P AD - Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND. FAU - Thabah, Molly Mary AU - Thabah MM AD - Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND. FAU - Verghese, Rohan J AU - Verghese RJ AD - Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND. FAU - Dineshbabu, Sekar AU - Dineshbabu S AD - Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND. FAU - Kadhiravan, Tamilarasu AU - Kadhiravan T AD - Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20210624 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8312813 OTO - NOTNLM OT - connective tissue disorder OT - gangrene OT - ischemia OT - lupus OT - systemic lupus erythematosus OT - uctd COIS- The authors have declared that no competing interests exist. EDAT- 2021/08/03 06:00 MHDA- 2021/08/03 06:01 PMCR- 2021/06/24 CRDT- 2021/08/02 06:04 PHST- 2021/06/24 00:00 [accepted] PHST- 2021/08/02 06:04 [entrez] PHST- 2021/08/03 06:00 [pubmed] PHST- 2021/08/03 06:01 [medline] PHST- 2021/06/24 00:00 [pmc-release] AID - 10.7759/cureus.15883 [doi] PST - epublish SO - Cureus. 2021 Jun 24;13(6):e15883. doi: 10.7759/cureus.15883. eCollection 2021 Jun. PMID- 22408869 OWN - NLM STAT- MEDLINE DCOM- 20120501 LR - 20120313 IS - 0035-2640 (Print) IS - 0035-2640 (Linking) VI - 62 IP - 2 DP - 2012 Feb TI - [Sjögren's syndrome: diagnosis and systemic manifestations]. PG - 221-4 AB - Sjögren's syndrome is an autoimmune exocrinopathy characterized by keratoconjunctivis sicca, xerostomia and immune-inflammatory systemic manifestations. The diagnosis is easy to establish when the patient presents with sicca complex as a main symptom, or recurring attacks of parotitis. However, it is way more complex when the disease begins with extraglandular features, such as non erosive polyarticular arthritis, Raynaud's phenomenon, peripheral or central nervous system involvement, kidney disease or interstitial pneumonary disease, or even vasculitis. In such circumstances, diagnosis is often delayed several years. FAU - Hatron, Pierre-Yves AU - Hatron PY AD - CHRU de Lille, hôpital Claude-Huriez, service de médecine interne, 59037 Lille cedex. pierre-yves.hatron@chru-lille.fr LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Circonstances de découverte et manifestations systémiques du syndrome de Gougerot-Sjögren. PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Brain Diseases/complications/diagnosis MH - Exocrine Glands/pathology MH - Humans MH - Incidental Findings MH - Joint Diseases/complications/diagnosis MH - Sjogren's Syndrome/*complications/*diagnosis/pathology MH - Vascular Diseases/complications/diagnosis MH - Xerophthalmia/complications/diagnosis/pathology MH - Xerostomia/complications/diagnosis/pathology EDAT- 2012/03/14 06:00 MHDA- 2012/05/02 06:00 CRDT- 2012/03/14 06:00 PHST- 2012/03/14 06:00 [entrez] PHST- 2012/03/14 06:00 [pubmed] PHST- 2012/05/02 06:00 [medline] PST - ppublish SO - Rev Prat. 2012 Feb;62(2):221-4. PMID- 19487839 OWN - NLM STAT- MEDLINE DCOM- 20090820 LR - 20090602 IS - 0021-4884 (Print) IS - 0021-4884 (Linking) VI - 58 IP - 5 DP - 2009 May TI - [Thrombotic thrombocytopenic purpura with mixed connective tissue disease. A case report]. PG - 567-72 AB - We reported a very rare case of mixed connective tissue disease (MCTD) with thrombotic thrombocytopenic purpura (TTP). The patient was a 24-year-old female who admitted to our hospital in February 2007 because of swelling of her fingers and Raynaud's phenomenon, and was diagnosed as MCTD. Her symptom was improved with the oral administration of prednisolone 10 mg/day. In September 2007, her blood examination test showed remarkable thrombocytopenia and hemolytic anemia. A significant number of schistocytes were observed in her peripheral blood smear, and a disintegrin-like and metalloproteinase with thrombospondin type1 motifs13 (ADAMTS13) activity in her serum was below the measurement sensitivity, resulted in the diagnosis of TTP. Seven-time plasma exchanges so far cured TTP clinically without any relapse, with remarkable improving of all laboratory data relating to TTP. FAU - Kajita, Naomi AU - Kajita N AD - Department of Dermatology, Nagoya University Hospital. nao_sas2000@yahoo.com FAU - Muro, Yoshinao AU - Muro Y FAU - Tomita, Akihiro AU - Tomita A FAU - Hirashima, Kanji AU - Hirashima K FAU - Matsushita, Tadashi AU - Matsushita T FAU - Naoe, Tomoki AU - Naoe T FAU - Tomita, Yasushi AU - Tomita Y LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Arerugi JT - Arerugi = [Allergy] JID - 0241212 SB - IM MH - Female MH - Humans MH - Mixed Connective Tissue Disease/*complications MH - Purpura, Thrombotic Thrombocytopenic/*complications MH - Young Adult EDAT- 2009/06/03 09:00 MHDA- 2009/08/21 09:00 CRDT- 2009/06/03 09:00 PHST- 2008/06/02 00:00 [received] PHST- 2009/02/16 00:00 [accepted] PHST- 2009/06/03 09:00 [entrez] PHST- 2009/06/03 09:00 [pubmed] PHST- 2009/08/21 09:00 [medline] AID - 058050567e [pii] PST - ppublish SO - Arerugi. 2009 May;58(5):567-72. PMID- 29763008 STAT- Publisher CTDT- 20230808 PB - StatPearls Publishing DP - 2026 Jan TI - Raynaud Disease. BTI - StatPearls AB - In response to cold temperatures, the body adapts by restricting blood flow to the skin. This is done as a thermoregulating mechanism to prevent further loss of body heat and to sustain the core body temperature. In Raynaud phenomenon, blood-flow restriction occurs during cold temperatures and emotional stress. Specifically, in Raynaud phenomenon, there is vasoconstriction of the digital arteries and cutaneous arterioles. This phenomenon was first described by Maurice Raynaud in 1862 and later studied by Sir Thomas Lewis in 1930. Overall, Raynaud phenomenon is a transient and peripheral vasoconstrictive response to cold temperatures or emotional stress. Raynaud phenomenon can be categorized as either primary or secondary.  CI - Copyright © 2026, StatPearls Publishing LLC. FAU - Musa, Rina AU - Musa R AD - Creighton University School of Medicine FAU - Qurie, Ahmad AU - Qurie A LA - eng PT - Study Guide PT - Book Chapter PL - Treasure Island (FL) COIS- Disclosure: Rina Musa declares no relevant financial relationships with ineligible companies. Disclosure: Ahmad Qurie declares no relevant financial relationships with ineligible companies. EDAT- 2023/08/08 00:00 CRDT- 2023/08/08 00:00 AID - NBK499833 [bookaccession] PMID- 27159488 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20170928 LR - 20170928 IS - 0024-6921 (Print) IS - 0024-6921 (Linking) VI - 167 IP - 3 DP - 2015 May-Jun TI - SYSTEMIC SCLEROSIS RELATED CALCINOSIS: PATIENTS PROVIDE WHAT SPECIALISTS WANT TO LEARN. PG - 158-9 AB - BACKGROUND: Calcinosis is a disabling, rarely discussed manifestation of systemic sclerosis (SSc) for which the natural history and management is understood poorly. OBJECTIVES: To develop a calcinosis specific patient reported outcome measure (PROM) that can be used for future clinical research to test the effects of therapy on scleroderma related calcinosis. METHODS: Patients were selected for participation by their scleroderma physicians. Four focus groups and individual interviews were recorded and transcribed verbatim. Patients were asked to frame questions to help a physician learn if calcinosis was better, worse or the same. Patient transcripts underwent an iterative inductive process (no preconceived coding, content drives coding and analysis) by at least five independent analysts including at least one research team member with SSc. Concepts were triangulated to identify a comprehensive set of meaningful concepts with occurrence quantified per participant. RESULTS: Twenty-three patients (22/23 female, 19/23 white, with mean disease duration 14.8 years) consented and were interviewed. Responses included concepts of self-management strategies and recurrent hypotheses relating calcinosis development to trauma, Raynaud's and cold exposure. We identified discrete concepts such as the perceived association between cold exposure, Raynaud's and calcinosis severity. Calcinosis tended to present along with or soon after SSc diagnosis and remained throughout disease duration - though was not yet compared to report of first Raynaud experience. CONCLUSIONS: Patient observations and self-management behavior provide opportunities for experts to learn from and to preemptively educate physicians and patients. Patients are eager for self-management guidance. These concepts are the groundwork for PROM development. However, patients suggested a composite of scales anchored in pain, size, frequency, number and related impairment may reasonably serve as an interim instrument for SSc calcinosis. FAU - Christensen, A AU - Christensen A AD - Tulane University School of Medicine, New Orleans, LA. FAU - Khalique, S AU - Khalique S AD - Louisiana University Health Sciences Center, New Orleans, LA. FAU - Cenac, S AU - Cenac S AD - Louisiana University Health Sciences Center, New Orleans. FAU - Fligelstone, K AU - Fligelstone K AD - Royal Free Hospital, Scleroderma Unit and The Scleroderma Society UK, London, UK. FAU - Mawdsley, A AU - Mawdsley A AD - Raynaud's and Scleroderma - Care and Support, UK. FAU - French, T AU - French T AD - University of Utah, Salt Lake City, UT. FAU - Gordon, J K AU - Gordon JK AD - Hospital for Special Surgery, NYC, NY. FAU - Baron, M AU - Baron M AD - Jewish General Hospital, McGill University, Montreal, Canada. FAU - Busman, E AU - Busman E AD - Atlanta Scleroderma Support Group, Atlanta, GA. FAU - Steen, V D AU - Steen VD AD - Georgetown University Medical Center, Washington, DC. FAU - Saketkoo, L A AU - Saketkoo LA AD - University Medical Center Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans. LA - eng PT - Journal Article DEP - 20150615 PL - United States TA - J La State Med Soc JT - The Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society JID - 7505618 EDAT- 2016/05/10 06:00 MHDA- 2016/05/10 06:01 CRDT- 2016/05/10 06:00 PHST- 2016/05/10 06:00 [entrez] PHST- 2016/05/10 06:00 [pubmed] PHST- 2016/05/10 06:01 [medline] PST - ppublish SO - J La State Med Soc. 2015 May-Jun;167(3):158-9. Epub 2015 Jun 15. PMID- 22389797 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20240315 IS - 2090-5475 (Electronic) IS - 2090-5467 (Print) IS - 2090-5467 (Linking) VI - 2011 DP - 2011 TI - Results of a Pilot Randomized Placebo-Controlled Trial in Primary and Secondary Raynaud's Phenomenon with St. John's Wort: Detecting Changes in Angiogenic Cytokines When RP Improves. PG - 580704 LID - 10.5402/2011/580704 [doi] LID - 580704 AB - Objectives.To perform a 6-week double-blind RCT in Raynaud's phenomenon (RP) comparing the plant extract St. John's Wort (SJW) to placebo. Methods. RP patients having at least 7 attacks per week were stratified by primary and secondary RP and within secondary by systemic sclerosis or other connective tissue disease. Subjects completed a daily standardized diary recording all RP attacks (frequency, duration and severity). Serum levels of 18 inflammatory and angiogenic cytokines were measured pre- and post-treatment. Results. Eighteen patients completed the study; 8 received SJW and 10 placebo. The decrease in mean number of attacks per day was 0.75 with SJW and 1.01 with placebo, P = 0.06. Attack duration and severity were not different between groups. Cytokine analyses demonstrated no between-groups differences. Combining treatment groups, those with >50% improvement in frequency of attacks yielded a significant increase in E-selectin (P = 0.049), MMP-9 (P = 0.011), G-CSF (P = 0.02), and VEGF (P = 0.012) pre- versus post-treatment. A ≥50% improvement in severity of attacks corresponded to a significant increase in levels of sVCAM-1 (P = 0.003), sICAM-1 (P = 0.007), and MCP-1 (P = 0.004). Conclusions. There were no clinical or biomarker benefit of SJW versus placebo in RP. However, combining all patients, there were changes in some cytokines that may be further investigated. FAU - Malenfant, Déanne AU - Malenfant D AD - Department of Medicine, University of Western Ontario, London, ON, Canada N6A 5C1. FAU - Summers, Kelly AU - Summers K FAU - Seney, Shannon AU - Seney S FAU - McBain, Donna AU - McBain D FAU - Petrlich, Lisa AU - Petrlich L FAU - Watson, Sharon AU - Watson S FAU - Vanderhoek, Louise AU - Vanderhoek L FAU - Samadi, Nooshin AU - Samadi N FAU - Bonner, Ashley AU - Bonner A FAU - Pope, Janet AU - Pope J LA - eng PT - Journal Article DEP - 20110912 PL - Egypt TA - ISRN Rheumatol JT - ISRN rheumatology JID - 101570649 PMC - PMC3263747 EDAT- 2011/01/01 00:00 MHDA- 2011/01/01 00:01 PMCR- 2011/09/12 CRDT- 2012/03/06 06:00 PHST- 2011/05/06 00:00 [received] PHST- 2011/06/27 00:00 [accepted] PHST- 2012/03/06 06:00 [entrez] PHST- 2011/01/01 00:00 [pubmed] PHST- 2011/01/01 00:01 [medline] PHST- 2011/09/12 00:00 [pmc-release] AID - 10.5402/2011/580704 [doi] PST - ppublish SO - ISRN Rheumatol. 2011;2011:580704. doi: 10.5402/2011/580704. Epub 2011 Sep 12. PMID- 34649624 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250928 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 5 IP - 1 DP - 2021 Oct 15 TI - The frequency of Raynaud's phenomenon, very early diagnosis of systemic sclerosis, and systemic sclerosis in a large Veteran Health Administration database. PG - 42 LID - 10.1186/s41927-021-00209-z [doi] LID - 42 AB - BACKGROUND: We describe Raynauds phenomenon (RP), potential very early diagnosis of systemic sclerosis (VEDOSS), and systemic sclerosis (SSc) in Veterans deployed in support of Post-9/11 operations. We sought to describe the military occupation specialty, clinical features, and vasodilator use across the three diagnoses. METHODS: Individual Veterans medical records were assessed for RP (ICD-9443.0), VEDOSS with swelling of hands (ICD-9729.81) and RP (ICD-9443.0), and SSc (ICD-9710.1). The distribution of sociodemographic, military service branch, job classification, vasodilator use, and comorbidities were examined across the three classifications of disease. The chi-squared test and Fisher's exact compared frequency of these categorical variables. Logistic regression assessed the likelihood of characteristics of the three classifications. RESULTS: In this population of 607,665 individual Veteran medical records, 857 had RP, 45 met possible VEDOSS criteria, and 71 had a diagnosis of SSc. The majority of RP, potential VEDOSS and SSc cases were white males. Those in craftworks, engineering or maintenance, and healthcare had a greater likelihood of RP. Less than half of RP and VEDOSS patients were on vasodilators. The most common comorbidities in this population were the diagnostic code for pain (highest in the potential VEDOSS group [81.6%]), followed by depression in all groups. CONCLUSION: This is a unique Veteran population of predominately-male patients. Our data suggests that vasodilator medications are potentially being under-utilized for RP and potential VEDOSS. Our data highlights mood and pain management as an important aspect of SSc care. CI - © 2021. The Author(s). FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Department of Internal Medicine, Division of Rheumatology, University of Utah and Salt Lake Veterans Affair Medical Center, 1900 E 30 N, SOM 4b200, Salt Lake City, UT, 84132, USA. tracy.frech@hsc.utah.edu. FAU - Murtaugh, Maureen A AU - Murtaugh MA AD - Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Division of Epidemiology, Salt Lake City, UT, USA. FAU - Amuan, Megan AU - Amuan M AD - Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Division of Epidemiology, Salt Lake City, UT, USA. FAU - Pugh, Mary Jo AU - Pugh MJ AD - Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Division of Epidemiology, Salt Lake City, UT, USA. LA - eng GR - UL1 TR002538/TR/NCATS NIH HHS/United States GR - KL2 TR002539/TR/NCATS NIH HHS/United States GR - I01 CX001183/CX/CSRD VA/United States GR - K23AR067889/AR/NIAMS NIH HHS/United States GR - K23 AR067889/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20211015 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC8518247 OTO - NOTNLM OT - Occupational health OT - Raynaud’s phenomenon OT - Scleroderma OT - Systemic sclerosis COIS- The authors declare that they have no competing interests. EDAT- 2021/10/16 06:00 MHDA- 2021/10/16 06:01 PMCR- 2021/10/15 CRDT- 2021/10/15 05:31 PHST- 2021/01/17 00:00 [received] PHST- 2021/06/28 00:00 [accepted] PHST- 2021/10/15 05:31 [entrez] PHST- 2021/10/16 06:00 [pubmed] PHST- 2021/10/16 06:01 [medline] PHST- 2021/10/15 00:00 [pmc-release] AID - 10.1186/s41927-021-00209-z [pii] AID - 209 [pii] AID - 10.1186/s41927-021-00209-z [doi] PST - epublish SO - BMC Rheumatol. 2021 Oct 15;5(1):42. doi: 10.1186/s41927-021-00209-z. PMID- 35006287 OWN - NLM STAT- MEDLINE DCOM- 20220228 LR - 20220429 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 42 IP - 2 DP - 2022 Feb TI - Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review. PG - 359-364 LID - 10.1007/s00296-021-05075-z [doi] AB - Autoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud's, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction. CI - © 2022. The Author(s). FAU - Srikantharajah, Denesh AU - Srikantharajah D AD - Department of Rheumatology, St George's University Hospitals NHS Foundation Trust, London, SW17 0QT, UK. FAU - Lloyd, Mark E AU - Lloyd ME AD - Department of Rheumatology, Frimley Healthcare NHS Foundation Trust, Frimley, UK. FAU - Kiely, Patrick D W AU - Kiely PDW AUID- ORCID: 0000-0002-8091-2717 AD - Department of Rheumatology, St George's University Hospitals NHS Foundation Trust, London, SW17 0QT, UK. patrick.kiely@nhs.net. AD - Institute of Medical and Biomedical Education, St George's, University of London, London, UK. patrick.kiely@nhs.net. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20220110 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antirheumatic Agents) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulins, Intravenous) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Adult MH - Antirheumatic Agents/*administration & dosage MH - Autoantibodies/immunology MH - Female MH - Humans MH - Immunoglobulins, Intravenous/*administration & dosage MH - Male MH - Middle Aged MH - Muscle Weakness/etiology MH - Myositis/complications/*drug therapy MH - Rituximab/*administration & dosage MH - Scleroderma, Systemic/complications/*drug therapy MH - Young Adult PMC - PMC8800925 OTO - NOTNLM OT - Intravenous immunoglobulin OT - Myositis OT - PM/Scl autoantibody OT - Raynaud’s OT - Rituximab OT - Systemic sclerosis COIS- The authors declare that there is no conflict of interest. EDAT- 2022/01/11 06:00 MHDA- 2022/03/01 06:00 PMCR- 2022/01/10 CRDT- 2022/01/10 12:26 PHST- 2021/10/28 00:00 [received] PHST- 2021/12/18 00:00 [accepted] PHST- 2022/01/11 06:00 [pubmed] PHST- 2022/03/01 06:00 [medline] PHST- 2022/01/10 12:26 [entrez] PHST- 2022/01/10 00:00 [pmc-release] AID - 10.1007/s00296-021-05075-z [pii] AID - 5075 [pii] AID - 10.1007/s00296-021-05075-z [doi] PST - ppublish SO - Rheumatol Int. 2022 Feb;42(2):359-364. doi: 10.1007/s00296-021-05075-z. Epub 2022 Jan 10. PMID- 26181868 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150721 LR - 20181113 IS - 1865-7257 (Print) IS - 1865-7265 (Linking) VI - 5 IP - 1 DP - 2012 Feb TI - Involvement of the liver in rheumatic diseases. PG - 9-14 LID - 10.1007/s12328-011-0271-4 [doi] AB - Liver dysfunction is often observed in patients with rheumatic diseases. Underlying disease is the major cause of liver dysfunction associated with adult-onset Still's disease, polymyositis/dermatomyositis, and vasculitis syndrome, whereas drug-induced liver injury is common in rheumatoid arthritis and systemic lupus erythematosus. These diseases may be accompanied by autoimmune hepatitis and primary biliary cirrhosis (PBC). PBC is particularly common in CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactylia, and telangiectasia) syndrome and Sjögren's syndrome. The recent widespread use of biological drugs such as tumor necrosis factor inhibitors has raised concerns about reactivation of hepatitis B. Given the varying causes and degrees of liver dysfunction associated with rheumatic diseases, medical intervention should be undertaken with adequate understanding of the characteristics of each type of liver disease. FAU - Ohira, Hiromasa AU - Ohira H AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan. h-ohira@fmu.ac.jp. FAU - Abe, Kazumichi AU - Abe K AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan. FAU - Takahashi, Atsushi AU - Takahashi A AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan. LA - eng PT - Journal Article DEP - 20111126 PL - Japan TA - Clin J Gastroenterol JT - Clinical journal of gastroenterology JID - 101477246 OTO - NOTNLM OT - Autoimmune liver disease OT - CREST syndrome OT - Rheumatoid arthritis OT - Systemic lupus erythematosus EDAT- 2012/02/01 00:00 MHDA- 2012/02/01 00:01 CRDT- 2015/07/17 06:00 PHST- 2011/11/10 00:00 [received] PHST- 2011/11/10 00:00 [accepted] PHST- 2015/07/17 06:00 [entrez] PHST- 2012/02/01 00:00 [pubmed] PHST- 2012/02/01 00:01 [medline] AID - 10.1007/s12328-011-0271-4 [pii] AID - 10.1007/s12328-011-0271-4 [doi] PST - ppublish SO - Clin J Gastroenterol. 2012 Feb;5(1):9-14. doi: 10.1007/s12328-011-0271-4. Epub 2011 Nov 26. PMID- 36268301 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231106 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 82 DP - 2022 Oct TI - Antisynthetase syndrome and interstitial lung disease: A case report. PG - 104571 LID - 10.1016/j.amsu.2022.104571 [doi] LID - 104571 AB - INTRODUCTION: Myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands, and arthropathy are symptoms of Antisynthetase Syndrome (ASS), which is defined by the development of antibodies against t-ribonucleic acid (RNA) synthetase, particularly anti-Jo-1. CASE PRESENTATION: The case is about 29 years female with 1 month history of non-productive cough and dyspnea on exertion which was later diagnosed as ASS. DISCUSSION: The diagnosis of an inflammatory myopathy is based on clinical findings such as subacute development of symmetrical muscle weakness and signs such as laboratory investigations revealing skeletal muscle inflammation. Creatinine phosphokinase (CPK) is mainly used to demonstrate skeletal muscle involvement. CONCLUSION: Interstitial lung disease is a frequent occurrence and is associated with a bad prognosis during the course of antisynthetase syndrome. CI - © 2022 The Authors. FAU - Dahal, Krishna AU - Dahal K AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Chaudhary, Anand AU - Chaudhary A AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Rawal, Laba AU - Rawal L AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Ray, Umesh AU - Ray U AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Paudel, Sandip AU - Paudel S AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Khanal, Pitambar AU - Khanal P AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Gyawali, Pawan AU - Gyawali P AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Sah, Sanjit Kumar AU - Sah SK AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. FAU - Shrestha, Kishan Kumar AU - Shrestha KK AD - Nepal Medical College, Kathmandu, Nepal. FAU - Pandey, Shailendra AU - Pandey S AD - Tribhuvan University, Institute of Medicine, Maharajgunj, 44600, Nepal. LA - eng PT - Case Reports PT - Journal Article DEP - 20220908 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC9577423 OTO - NOTNLM OT - Antisynthetase syndrome OT - Case report OT - Interstitial lung disease OT - Non-specific interstitial pneumonia EDAT- 2022/10/22 06:00 MHDA- 2022/10/22 06:01 PMCR- 2022/09/08 CRDT- 2022/10/21 03:13 PHST- 2022/07/16 00:00 [received] PHST- 2022/08/30 00:00 [revised] PHST- 2022/09/04 00:00 [accepted] PHST- 2022/10/21 03:13 [entrez] PHST- 2022/10/22 06:00 [pubmed] PHST- 2022/10/22 06:01 [medline] PHST- 2022/09/08 00:00 [pmc-release] AID - S2049-0801(22)01331-0 [pii] AID - 104571 [pii] AID - 10.1016/j.amsu.2022.104571 [doi] PST - epublish SO - Ann Med Surg (Lond). 2022 Sep 8;82:104571. doi: 10.1016/j.amsu.2022.104571. eCollection 2022 Oct. PMID- 40584538 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250702 IS - 2050-313X (Print) IS - 2050-313X (Electronic) IS - 2050-313X (Linking) VI - 13 DP - 2025 TI - Nailfold capillaroscopy in myositis: A case series. PG - 2050313X251353297 LID - 10.1177/2050313X251353297 [doi] LID - 2050313X251353297 AB - Nailfold capillaroscopy is well established in systemic sclerosis; however, abnormalities (scleroderma or scleroderma-like patterns) are identified in other connective tissue diseases, such as myositis, even in the absence of Raynaud's phenomenon. Although this may be known within the rheumatology world, myositis cases often present to other subspecialists who may be unfamiliar with the value of capillaroscopy. We present four cases of myositis that initially presented to non-rheumatology subspecialists where capillaroscopy played a role in confirming the diagnosis. Nailfold capillaroscopy is especially useful in cases of isolated interstitial lung disease, atypical and isolated rashes, and to delineate between myositis mimickers, whether pathologic or clinical. It is a noninvasive tool, which is useful early in the work-up of myositis to identify features consistent with a connective tissue disease. CI - © The Author(s) 2025. FAU - Cotton, Thaisa AU - Cotton T AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montreal, QC, Canada. FAU - Hudson, Marie AU - Hudson M AUID- ORCID: 0000-0001-6718-2468 AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montreal, QC, Canada. AD - Lady Davis Institute for Medical Research, Montreal, QC, Canada. FAU - Troyanov, Yves AU - Troyanov Y AD - Division of Rheumatology, Department of Medicine, Hôpital du Sacre-Coeur de Montreal, University of Montreal, QC, Canada. FAU - Leclair, Valérie AU - Leclair V AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montreal, QC, Canada. AD - Lady Davis Institute for Medical Research, Montreal, QC, Canada. FAU - Gyger, Geneviève AU - Gyger G AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montreal, QC, Canada. LA - eng PT - Case Reports PT - Journal Article DEP - 20250627 PL - England TA - SAGE Open Med Case Rep JT - SAGE open medical case reports JID - 101638686 PMC - PMC12205182 OTO - NOTNLM OT - connective tissue disease OT - dermatomyositis OT - idiopathic inflammatory myopathies OT - interstitial lung disease OT - nailfold videocapillaroscopy COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/07/01 04:06 MHDA- 2025/07/01 04:07 PMCR- 2025/06/27 CRDT- 2025/06/30 06:12 PHST- 2025/04/14 00:00 [received] PHST- 2025/06/11 00:00 [accepted] PHST- 2025/07/01 04:07 [medline] PHST- 2025/07/01 04:06 [pubmed] PHST- 2025/06/30 06:12 [entrez] PHST- 2025/06/27 00:00 [pmc-release] AID - 10.1177_2050313X251353297 [pii] AID - 10.1177/2050313X251353297 [doi] PST - epublish SO - SAGE Open Med Case Rep. 2025 Jun 27;13:2050313X251353297. doi: 10.1177/2050313X251353297. eCollection 2025. PMID- 24765399 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140425 LR - 20211021 IS - 2039-7275 (Print) IS - 2039-7283 (Electronic) IS - 2039-7275 (Linking) VI - 1 IP - 4 DP - 2011 Sep 28 TI - Systemic sclerosis with hemoptysis and a huge lung cavity. PG - e99 LID - 10.4081/cp.2011.e99 [doi] LID - e99 AB - Systemic sclerosis or scleroderma is associated with distal vasculitis, Raynaud's phenomenon, and inflammation of internal organs and the skin. We present on a 58-year-old Thai woman with systemic sclerosis who came to the 10(th) Zonal Tuberculosis and Chest Disease Center, Chiang Mai, Thailand in 2009 and presented with hemoptysis and a solitary huge-lung cavity as the predominant clinical manifestations which spontaneously resoluted 2 months later. This case demonstrates a solitary huge-lung cavity with hemoptysis and looked like from non-tuberculous Mycobacterial infections or malignancy with spontaneous resolution of hemoptysis and the lung cavity, which does not need invasive investigations. FAU - Cheepsattayakorn, Attapon AU - Cheepsattayakorn A AD - 10 Zonal Tuberculosis and Chest Disease Center, Chiang Mai, 10 Office of Disease Prevention and Control, Department of Disease Control, Ministry of Public Health; FAU - Cheepsattayakorn, Ruangrong AU - Cheepsattayakorn R AD - Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. LA - eng PT - Case Reports PT - Journal Article DEP - 20111107 PL - Switzerland TA - Clin Pract JT - Clinics and practice JID - 101563282 PMC - PMC3981401 OTO - NOTNLM OT - hemoptysis. OT - huge lung cavity OT - systemic sclerosis EDAT- 2011/09/28 00:00 MHDA- 2011/09/28 00:01 PMCR- 2011/11/07 CRDT- 2014/04/26 06:00 PHST- 2011/09/19 00:00 [received] PHST- 2011/10/18 00:00 [accepted] PHST- 2014/04/26 06:00 [entrez] PHST- 2011/09/28 00:00 [pubmed] PHST- 2011/09/28 00:01 [medline] PHST- 2011/11/07 00:00 [pmc-release] AID - cp.2011.e99 [pii] AID - 10.4081/cp.2011.e99 [doi] PST - epublish SO - Clin Pract. 2011 Nov 7;1(4):e99. doi: 10.4081/cp.2011.e99. eCollection 2011 Sep 28. PMID- 32714443 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240329 IS - 1753-495X (Print) IS - 1753-4968 (Electronic) IS - 1753-495X (Linking) VI - 13 IP - 2 DP - 2020 Jun TI - Antisynthetase syndrome in pregnancy: A case and review of the literature. PG - 96-100 LID - 10.1177/1753495X18808646 [doi] AB - Antisynthetase syndrome is a rare autoimmune, multisystem, inflammatory condition, characterised by autoantibodies against aminoacyl tRNA synthetases. The predominant features are myositis and interstitial lung disease but other symptoms such as Raynaud's phenomenon may also be present. Described here is a 36-year-old woman with antisynthetase syndrome who planned and underwent a successful pregnancy, during which a multidisciplinary team approach secured a good outcome for both mother and baby. CI - © The Author(s) 2018. FAU - Green, Lauren J AU - Green LJ AUID- ORCID: 0000-0003-0230-2662 AD - Oxford University Hospitals NHS Foundation Trust, John Radcliffe Hospital, Oxford, UK. FAU - O'Neill, Lorraine AU - O'Neill L AD - Department of Rheumatology, Oxford University Hospitals NHS Foundation Trust, Nuffield Orthopaedic Centre, Oxford, UK. FAU - Frise, Charlotte J AU - Frise CJ AD - Oxford University Hospitals NHS Foundation Trust, John Radcliffe Hospital, Oxford, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20181108 PL - England TA - Obstet Med JT - Obstetric medicine JID - 101464191 PMC - PMC7359659 OTO - NOTNLM OT - Antisynthetase syndrome OT - anti-Ro antibodies OT - inflammatory myopathy OT - pregnancy EDAT- 2020/07/28 06:00 MHDA- 2020/07/28 06:01 PMCR- 2021/06/01 CRDT- 2020/07/28 06:00 PHST- 2018/05/12 00:00 [received] PHST- 2018/09/28 00:00 [accepted] PHST- 2020/07/28 06:00 [entrez] PHST- 2020/07/28 06:00 [pubmed] PHST- 2020/07/28 06:01 [medline] PHST- 2021/06/01 00:00 [pmc-release] AID - 10.1177_1753495X18808646 [pii] AID - 10.1177/1753495X18808646 [doi] PST - ppublish SO - Obstet Med. 2020 Jun;13(2):96-100. doi: 10.1177/1753495X18808646. Epub 2018 Nov 8. PMID- 25349765 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20141028 LR - 20201001 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2014 DP - 2014 TI - Systemic sclerosis and silicone breast implant: a case report and review of the literature. PG - 809629 LID - 10.1155/2014/809629 [doi] LID - 809629 AB - Environmentally induced systemic sclerosis is a well-recognized condition, which is correlated with exposure to various chemical compounds or drugs. However, development of scleroderma-like disease after exposure to silicone has always been a controversial issue and, over time, it has triggered spirited debate whether there is a certain association or not. Herein, we report the case of a 35-year-old female who developed Raynaud's phenomenon and, finally, systemic sclerosis shortly after silicone breast implantation surgery. FAU - Psarras, Antonios AU - Psarras A AUID- ORCID: 0000-0002-6318-0171 AD - Clinical Immunology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos Street 49, 546 42 Thessaloniki, Greece. FAU - Gkougkourelas, Ioannis AU - Gkougkourelas I AD - Clinical Immunology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos Street 49, 546 42 Thessaloniki, Greece. FAU - Tselios, Konstantinos AU - Tselios K AD - Clinical Immunology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos Street 49, 546 42 Thessaloniki, Greece. FAU - Sarantopoulos, Alexandros AU - Sarantopoulos A AUID- ORCID: 0000-0002-1179-3195 AD - Clinical Immunology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos Street 49, 546 42 Thessaloniki, Greece. FAU - Boura, Panagiota AU - Boura P AD - Clinical Immunology Unit, 2nd Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Konstantinoupoleos Street 49, 546 42 Thessaloniki, Greece. LA - eng PT - Journal Article DEP - 20140930 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC4199071 EDAT- 2014/10/29 06:00 MHDA- 2014/10/29 06:01 PMCR- 2014/09/30 CRDT- 2014/10/29 06:00 PHST- 2014/08/27 00:00 [received] PHST- 2014/09/14 00:00 [revised] PHST- 2014/09/19 00:00 [accepted] PHST- 2014/10/29 06:00 [entrez] PHST- 2014/10/29 06:00 [pubmed] PHST- 2014/10/29 06:01 [medline] PHST- 2014/09/30 00:00 [pmc-release] AID - 10.1155/2014/809629 [doi] PST - ppublish SO - Case Rep Rheumatol. 2014;2014:809629. doi: 10.1155/2014/809629. Epub 2014 Sep 30. PMID- 37291891 OWN - NLM STAT- MEDLINE DCOM- 20230630 LR - 20230630 IS - 1744-7666 (Electronic) IS - 1465-6566 (Linking) VI - 24 IP - 10 DP - 2023 May-Aug TI - Pharmacological management of digital ulcers in systemic sclerosis - what is new? PG - 1159-1170 LID - 10.1080/14656566.2023.2213434 [doi] AB - INTRODUCTION: Digital ulcers (DUs) develop in approximately 50% of patients with systemic sclerosis (SSc). DUs are painful and disfiguring, with a major impact on hand function and quality of life. Although some pharmacological treatments have been shown to confer benefit, new treatments are badly needed: SSc-related DUs are an area of major unmet clinical need. This review focuses on advances in pharmacological management. AREAS COVERED: DU definition, types of DU, and clinical burden are briefly described and the general approach to multidisciplinary management, followed by a more detailed description of pharmacological management, with particular reference to blocking the endothelin pathway, and supplementing the nitric oxide and prostacyclin pathways. Other aspects of pharmacological management, including analgesia and botulinum toxin injections are also discussed. To inform the review, the MEDLINE database was searched for English-language papers published between 1946 and December 2022 using search terms: 'systemic sclerosis (scleroderma)' and 'digital ulcer' or 'finger ulcer' or 'digital vasculopathy.' EXPERT OPINION: The key challenges to preventing and treating DUs are to develop and validate reliable, sensitive outcome measures to facilitate clinical trials, and then to undertake trials of emerging new approaches to treatment, including topical therapies and (in early disease) vascular remodeling therapies. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK. FAU - Philobos, Mariana AU - Philobos M AD - Department of Rheumatology, Aberdeen Royal Infirmary, Aberdeen, UK. LA - eng PT - Journal Article PT - Review DEP - 20230609 PL - England TA - Expert Opin Pharmacother JT - Expert opinion on pharmacotherapy JID - 100897346 RN - digital ulcers SB - IM MH - Humans MH - Ulcer MH - *Skin Ulcer/drug therapy/etiology MH - Quality of Life MH - *Scleroderma, Systemic/complications/drug therapy MH - Pain OTO - NOTNLM OT - Digital ulcers OT - Raynaud’s phenomenon OT - digital vasculopathy OT - systemic sclerosis OT - treatment EDAT- 2023/06/09 06:42 MHDA- 2023/06/30 06:42 CRDT- 2023/06/09 03:52 PHST- 2023/06/30 06:42 [medline] PHST- 2023/06/09 06:42 [pubmed] PHST- 2023/06/09 03:52 [entrez] AID - 10.1080/14656566.2023.2213434 [doi] PST - ppublish SO - Expert Opin Pharmacother. 2023 May-Aug;24(10):1159-1170. doi: 10.1080/14656566.2023.2213434. Epub 2023 Jun 9. PMID- 33493310 OWN - NLM STAT- MEDLINE DCOM- 20210629 LR - 20210629 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 60 IP - 5 DP - 2021 May 14 TI - Quantitative nailfold capillaroscopy-update and possible next steps. PG - 2054-2065 LID - 10.1093/rheumatology/keab006 [doi] AB - We review the exciting potential (and challenges) of quantitative nailfold capillaroscopy, focusing on its role in systemic sclerosis. Quantifying abnormality, including automated analysis of nailfold images, overcomes the subjectivity of qualitative/descriptive image interpretation. First we consider the rationale for quantitative analysis, including the potential for precise discrimination between normal and abnormal capillaries and for reliable measurement of disease progression and treatment response. We discuss nailfold image acquisition and interpretation, and describe how early work on semi-quantitative and quantitative analysis paved the way for semi-automated and automated analysis. Measurement of red blood cell velocity is described briefly. Finally we give a personal view on 'next steps'. From a clinical perspective, increased uptake of nailfold capillaroscopy by general rheumatologists could be achieved via low-cost hand-held devices with cloud-based automated analysis. From a research perspective, automated analysis could facilitate large-scale prospective studies using capillaroscopic parameters as possible biomarkers of systemic sclerosis-spectrum disorders. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre. FAU - Berks, Michael AU - Berks M AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, The University of Manchester, Manchester, UK. FAU - Taylor, Chris J AU - Taylor CJ AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, The University of Manchester, Manchester, UK. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Disease Progression MH - Humans MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Scleroderma, Systemic/diagnostic imaging/*physiopathology OTO - NOTNLM OT - Raynaud’s phenomenon OT - USB microscope OT - automated OT - nailfold capillaroscopy OT - quantitative OT - systemic sclerosis OT - videocapillaroscopy EDAT- 2021/01/26 06:00 MHDA- 2021/06/30 06:00 CRDT- 2021/01/25 17:16 PHST- 2020/10/11 00:00 [received] PHST- 2020/12/16 00:00 [revised] PHST- 2020/12/18 00:00 [accepted] PHST- 2021/01/26 06:00 [pubmed] PHST- 2021/06/30 06:00 [medline] PHST- 2021/01/25 17:16 [entrez] AID - 6119380 [pii] AID - 10.1093/rheumatology/keab006 [doi] PST - ppublish SO - Rheumatology (Oxford). 2021 May 14;60(5):2054-2065. doi: 10.1093/rheumatology/keab006. PMID- 29317057 OWN - NLM STAT- MEDLINE DCOM- 20180827 LR - 20180827 IS - 1532-2777 (Electronic) IS - 0306-9877 (Linking) VI - 110 DP - 2018 Jan TI - The complex associations between obstructive sleep apnea and auto-immune disorders: A review. PG - 138-143 LID - S0306-9877(17)30474-7 [pii] LID - 10.1016/j.mehy.2017.12.004 [doi] AB - Obstructive sleep apnea is known to be associated with diseases such as hypertension, metabolic disorder, and cancer. A more controversial and less understood association is that of sleep apneas and the development and worsening of autoimmune and rheumatologic disorders. Through the main pathways of intermittent hypoxia and sleep deprivation, we hypothesize that obstructive sleep apnea creates a chronic inflammatory state that worsens or incites autoimmune disorders. This thorough review of the available literature highlights our current understanding of the relationship between these disease processes in order to demonstrate the importance of diagnosis and appropriate management of sleep disorders in patients suffering from rheumatologic diseases. CI - Copyright © 2017 Elsevier Ltd. All rights reserved. FAU - Vakil, Mayand AU - Vakil M AD - Department of Otorhinolaryngology and Sleep Medicine, Albert Einstein College of Medicine, Bronx, NY 10461, United States. Electronic address: Mayand.Vakil@gmail.com. FAU - Park, Steven AU - Park S AD - Department of Otorhinolaryngology and Sleep Medicine, Albert Einstein College of Medicine, Bronx, NY 10461, United States. FAU - Broder, Anna AU - Broder A AD - Department of Rheumatology, Albert Einstein College of Medicine, Bronx, NY 10461, United States. LA - eng PT - Journal Article PT - Review DEP - 20171205 PL - United States TA - Med Hypotheses JT - Medical hypotheses JID - 7505668 SB - IM MH - Alzheimer Disease/complications/immunology/physiopathology MH - Animals MH - Arthritis, Rheumatoid/complications/immunology/physiopathology MH - Autoimmune Diseases/*complications/immunology/physiopathology MH - Humans MH - Inflammation/complications/immunology/physiopathology MH - Lupus Erythematosus, Systemic/complications/immunology/physiopathology MH - Models, Biological MH - Psoriasis/complications/immunology/physiopathology MH - Sleep Apnea, Obstructive/*complications/immunology/physiopathology OTO - NOTNLM OT - Chronic inflammation OT - Intermittent hypoxia OT - Neuro-inflammation OT - Obesity OT - Obstructive sleep apnea OT - Psoriasis OT - Raynaud’s phenomenon OT - Rheumatoid arthritis OT - Sleep deprivation OT - Systemic lupus erythematosus EDAT- 2018/01/11 06:00 MHDA- 2018/08/28 06:00 CRDT- 2018/01/11 06:00 PHST- 2017/05/09 00:00 [received] PHST- 2017/10/27 00:00 [revised] PHST- 2017/12/01 00:00 [accepted] PHST- 2018/01/11 06:00 [entrez] PHST- 2018/01/11 06:00 [pubmed] PHST- 2018/08/28 06:00 [medline] AID - S0306-9877(17)30474-7 [pii] AID - 10.1016/j.mehy.2017.12.004 [doi] PST - ppublish SO - Med Hypotheses. 2018 Jan;110:138-143. doi: 10.1016/j.mehy.2017.12.004. Epub 2017 Dec 5. PMID- 26977918 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 1997-7298 (Print) IS - 1997-7298 (Linking) VI - 115 IP - 10 Pt 2 DP - 2015 TI - [A case report of bilateral trigeminal sensory neuropathy as one of the initial manifestation of systemic scleroderma (the difficulties of early diagnosis of the primary disease)]. PG - 59-63 LID - 10.17116/jnevro201511510259-63 [doi] AB - The article describes the case of a patient with bilateral trigeminal sensory neuropathy (TSN), as a possible neurological manifestation of systemic scleroderma (SS). In this patient, intense non-paroxysmal facial pain caused by TSN, subjectively dominated over other manifestations of SS, including Raynaud's syndrome, for at least 1.5 years, thus hampering the diagnosis of the primary disease. In addition to pain, which was not relieved by analgesic medication, TSN was manifested by marked sensory deficit on the face (hypoesthesia / anesthesia) and bilateral sensory deficits in the oral cavity, including the anterior third of the tongue. TSN was also combined with disorders of taste perception. The assumption of rheumatic origin of TSN occurred during a primary neurological examination: a standard examination revealed generalized sensory polyneuropathy with bilateral involvement of the trigeminal nerve; the additional study identified no neurological signs of rheumatic diseases, including Raynaud's phenomenon. SS met all the criteria for the diagnosis (2013), high titers of nuclear ribonucleoprotein were determined as well. Thus, TSN as early and subjectively dominant manifestation of SS can complicate the diagnosis of primary rheumatic diseases. Therefore, in cases of distal sensory polyneuropathy with bilateral involvement of the trigeminal nerve, it is necessary to conduct an additional survey to identify the signs of possible rheumatic diseases: signs of vascular lesion (Raynaud's syndrome), lesions of skin, joints and muscles. FAU - Grachev, Ju V AU - Grachev JV AD - FGBNU 'NII obschej patologii i patofiziologii', Moskva. FAU - Anan'eva, L P AU - Anan'eva LP AD - FGBNU 'NII revmatologii im. V.A. Nasonovoj', Moskva. FAU - Tjurnikov, V M AU - Tjurnikov VM AD - FGBNU 'Nauchnyj tsentr nevrologii', Moskva. FAU - Zaharova, A Ju AU - Zaharova AJ AD - FGBNU 'NII revmatologii im. V.A. Nasonovoj', Moskva. LA - rus PT - English Abstract PT - Journal Article TT - Dvustoronnyaya trigeminal'naya sensornaya neiropatiya kak odno iz nachal'nykh proyavlenii sistemnoi sklerodermii (trudnosti rannego ustanovleniya diagnoza osnovnogo zabolevaniya). PL - Russia (Federation) TA - Zh Nevrol Psikhiatr Im S S Korsakova JT - Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova JID - 9712194 SB - IM EDAT- 2015/01/01 00:00 MHDA- 2015/01/01 00:01 CRDT- 2016/03/16 06:00 PHST- 2016/03/16 06:00 [entrez] PHST- 2015/01/01 00:00 [pubmed] PHST- 2015/01/01 00:01 [medline] AID - 10.17116/jnevro201511510259-63 [doi] PST - ppublish SO - Zh Nevrol Psikhiatr Im S S Korsakova. 2015;115(10 Pt 2):59-63. doi: 10.17116/jnevro201511510259-63. PMID- 23257531 OWN - NLM STAT- MEDLINE DCOM- 20130528 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 51 IP - 24 DP - 2012 TI - Rapidly progressive respiratory failure in mixed connective tissue disease: report of an autopsy case. PG - 3415-9 AB - A 64-year-old woman presented with exertional dyspnea. The case was diagnosed as mixed connective tissue disease (MCTD) due to presence of swollen fingers, Raynaud's phenomenon, muscle weakness, positive anti-U1RNP antibody, pericarditis and interstitial pneumonia. Although the histology from a transbronchial lung biopsy (TBLB) indicated organizing pneumonia, corticosteroid therapy was postponed for two months at the patient's request. She died 8 weeks later from acute progressive interstitial pneumonia in spite of the administration of intravenous cyclophosphamide combined with prednisolone. The autopsy revealed exudative and organizing diffuse alveolar damage (DAD). Previous reports have shown that DAD is an extremely rare pulmonary complication in MCTD. This report presents a case of MCTD with acute respiratory failure. This case thus suggests that this therapy should be administered as soon as possible. FAU - Watanabe, Yasutaka AU - Watanabe Y AD - Division of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan. yasuyasu@omiya.jichi.ac.jp FAU - Koyama, Shinichiro AU - Koyama S FAU - Moriguchi, Masato AU - Moriguchi M FAU - Miwa, Chihiro AU - Miwa C FAU - Shiraishi, Mamoru AU - Shiraishi M FAU - Nomura, Motoko AU - Nomura M FAU - Nokubi, Mitsuhiro AU - Nokubi M FAU - Terai, Chihiro AU - Terai C FAU - Kawabata, Yoshinori AU - Kawabata Y LA - eng PT - Case Reports PT - Journal Article DEP - 20121215 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM MH - Autopsy MH - Disease Progression MH - Fatal Outcome MH - Female MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications MH - Respiratory Insufficiency/*etiology MH - Time Factors EDAT- 2012/12/22 06:00 MHDA- 2013/05/29 06:00 CRDT- 2012/12/22 06:00 PHST- 2012/12/22 06:00 [entrez] PHST- 2012/12/22 06:00 [pubmed] PHST- 2013/05/29 06:00 [medline] AID - DN/JST.JSTAGE/internalmedicine/51.8728 [pii] AID - 10.2169/internalmedicine.51.8728 [doi] PST - ppublish SO - Intern Med. 2012;51(24):3415-9. doi: 10.2169/internalmedicine.51.8728. Epub 2012 Dec 15. PMID- 16704598 OWN - NLM STAT- MEDLINE DCOM- 20060707 LR - 20220310 IS - 0003-2409 (Print) IS - 0003-2409 (Linking) VI - 61 IP - 6 DP - 2006 Jun TI - Major colorectal surgery in a patient with cold agglutinin disease. PG - 593-6 AB - We present the case of a 62-year-old man with severe cold agglutinin disease who underwent major colorectal surgery. Cold agglutinin disease is a condition in which auto-antibodies, usually immunoglobulin M, cause red blood cell agglutination at decreased body temperature. Haemolysis may result. Agglutination results in impaired perfusion, resulting in symptomatic Raynaud's phenomenon and acrocyanosis. Haemolysis can result in anaemia and thrombotic events caused by microvascular occlusion, in addition to haemoglobinuria and renal failure. Peri-operative hypothermia is common in all patients and may be associated with significant morbidity, but is potentially catastrophic in a patient suffering from cold agglutinin disease. FAU - Young, S AU - Young S AD - Department of Anaesthesia, Hairmyres Hospital, East Kilbride, UK. FAU - Haldane, G AU - Haldane G LA - eng PT - Case Reports PT - Journal Article PL - England TA - Anaesthesia JT - Anaesthesia JID - 0370524 SB - IM MH - Anemia, Hemolytic, Autoimmune/*complications MH - Anesthesia, General/methods MH - Body Temperature MH - Humans MH - Hypothermia/etiology/*prevention & control MH - Intraoperative Complications/prevention & control MH - Male MH - Middle Aged MH - Perioperative Care/methods MH - Postoperative Complications/prevention & control MH - Rectal Neoplasms/*surgery EDAT- 2006/05/18 09:00 MHDA- 2006/07/11 09:00 CRDT- 2006/05/18 09:00 PHST- 2006/05/18 09:00 [pubmed] PHST- 2006/07/11 09:00 [medline] PHST- 2006/05/18 09:00 [entrez] AID - ANA4650 [pii] AID - 10.1111/j.1365-2044.2006.04650.x [doi] PST - ppublish SO - Anaesthesia. 2006 Jun;61(6):593-6. doi: 10.1111/j.1365-2044.2006.04650.x. PMID- 27275213 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160609 LR - 20200929 IS - 1857-9655 (Print) IS - 1857-9655 (Electronic) IS - 1857-9655 (Linking) VI - 3 IP - 1 DP - 2015 Mar 15 TI - Work-Related Upper Limb Disorders: A Case Report. PG - 146-50 LID - 10.3889/oamjms.2015.033 [doi] AB - In this study the complex interrelationship between physical factors, job stress, lifestyle and genetic factors on symptoms of work-related musculoskeletal disorders of the upper limbs is demonstrated by a case report and discussion of the literature. A 58 year old woman with long lasting complaints of the upper limbs with increasing intensity and duration, generalisation, combined with skin thickness, Raynaud's phenomenon, joint disorders, arterial and pulmonary hypertension, metabolic lipid dysfunctions is presented. Occupational history proves continuous duration of service at a job with occupational physical static load with numerous repetitive monotonous systematic motions of fingers and hands as a weaver of Persian rugs followed by work at an automated loom and variable labour activities. Though the complaints dated since the time she was a manual weaver, the manifestations of generalized joint degenerative changes, system sclerosis with Raynaud's phenomenon with similar upper extremities signs and symptoms discount upper limbs musculoskeletal disorder as caused only or mainly by occupational risk factors. The main principles and criteria for occupational diagnosis of musculoskeletal upper limb disorders and legislative requirements for their reglamentation are discussed. FAU - Stoyneva, Zlatka Borisova AU - Stoyneva ZB AD - University Hospital St. Ivan Rilsky, Clinic of Occupational Diseases, Medical University - Sofia, Bulgaria; University Hospital St George, Medical University - Plovdiv, Bulgaria. FAU - Dermendjiev, Svetlan AU - Dermendjiev S AD - University Hospital St George, Medical University - Plovdiv, Bulgaria. FAU - Dermendjiev, Tihomir AU - Dermendjiev T AD - University Hospital St George, Medical University - Plovdiv, Bulgaria. FAU - Dobrev, Hristo AU - Dobrev H AD - University Hospital St George, Medical University - Plovdiv, Bulgaria. LA - eng PT - Case Reports PT - Journal Article DEP - 20150311 PL - North Macedonia TA - Open Access Maced J Med Sci JT - Open access Macedonian journal of medical sciences JID - 101662294 PMC - PMC4877775 OTO - NOTNLM OT - connective tissue disease OT - musculoskeletal upper limb disorders OT - occupational disease OT - occupational risk OT - work-related disease EDAT- 2015/03/15 00:00 MHDA- 2015/03/15 00:01 PMCR- 2015/03/15 CRDT- 2016/06/09 06:00 PHST- 2015/01/06 00:00 [received] PHST- 2015/02/22 00:00 [revised] PHST- 2015/03/05 00:00 [accepted] PHST- 2016/06/09 06:00 [entrez] PHST- 2015/03/15 00:00 [pubmed] PHST- 2015/03/15 00:01 [medline] PHST- 2015/03/15 00:00 [pmc-release] AID - OAMJMS-3-146 [pii] AID - 10.3889/oamjms.2015.033 [doi] PST - ppublish SO - Open Access Maced J Med Sci. 2015 Mar 15;3(1):146-50. doi: 10.3889/oamjms.2015.033. Epub 2015 Mar 11. PMID- 38336186 OWN - NLM STAT- MEDLINE DCOM- 20240226 LR - 20240226 IS - 1872-7573 (Electronic) IS - 0378-8741 (Linking) VI - 325 DP - 2024 May 10 TI - Fingerprint analysis of dang-gui-Si-Ni decoction and its anticoagulant activity in vivo-in vitro. PG - 117890 LID - S0378-8741(24)00189-2 [pii] LID - 10.1016/j.jep.2024.117890 [doi] AB - ETHNOPHARMACOLOGICAL RELEVANCE: Dang-Gui-Si-Ni (DGSN) decoction is a classic prescription in the clinical practice of traditional Chinese Medicine (TCM). DGSN decoction is often used to relieve symptoms of cold coagulation and blood stasis recorded by Treatise on Febrile Diseases (Shang Han Lun) and treat Raynaud's disease, dysmenorrhea, arthritis, migraine in TCM clinic. Accumulated evidences have suggested that this diseases are related to microcirculation disturbance. However, the anticoagulant activity and underlying mechanisms of DGSN decoction responsible for the therapeutic not well understood. AIM OF THE STUDY: The fingerprint and anticoagulant activity in vivo-in vitro of DGSN decoction were evaluated to strengthen the quality control and activity study of formulas. MATERIALS AND METHODS: The chemical components of DGSN decoction were analyzed by HPLC and its fingerprint similarity were evaluated by "Chinese Medicine Chromatographic Fingerprint Similarity Evaluation Software (2012 Edition)". The anticoagulant activity of DGSN decoction was assessed by measuring four coagulation factors (PT, TT, APTT, FIB) in vitro. Zebrafish thrombosis model induced by punatinib was established to evaluate the activity of improving microvascular hemodynamics in vivo. Quantitative real-time polymerase chain reaction (q-PCR) were adopted to compare the changes in the RNA expression levels of coagulation factor II (FII), VII (FVII), IX (FIX) and X (FX) in zebrafish thrombosis model. RESULTS: The fingerprint similarity evaluation method of DGSN decoction was established. The results showed that 18 samples had higher similarity (S1-S18 > 0.878). Pharmacodynamic results showed that DGSN decoction could extend PT, TT and APTT, and reduce FIB content in vitro. Meanwhile, it markedly enhanced the cardiac output and blood flow velocity at low dosage (500 μg mL(-1)) in vivo. q-PCR data demonstrated that DGSN decoction (500 μg mL(-1)) could downregulate the RNA expression of FII, FVII, FIX and FX. Interestingly, there were a bidirectional regulation of FII, FIX and FX in a certain concentration range. In general, DGSN decoction can significantly improve hemodynamics and downregulate coagulation factors, and the results were consistent both in vitro - in vivo. CONCLUSION: The fingerprint study provide a new perspective for improving the quality control of DGSN decoction. DGSN decoction possess anticoagulant activity by regulating multiple coagulation factors simultaneously. Thus, it has the potential to develop into the novel raw material of anticoagulant drugs. CI - Copyright © 2024 Elsevier B.V. All rights reserved. FAU - Li, Yun AU - Li Y AD - School of Pharmaceutical Sciences, Shandong University of Traditional Chinese Medicine, Jinan, 250355, China; National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. Electronic address: liyun137934@163.com. FAU - Ren, Teng-Teng AU - Ren TT AD - Shandong Provincial Third Hospital Cheeloo College of Medicine, Shandong University, 11 Wuyingshan Road, Jinan, 250031, Shandong, China. FAU - Liu, Shan-Shan AU - Liu SS AD - Institute of Analysis and Testing, Beijing Academy of Science and Technology (Beijing Center for Physical &Chemical Analysis), No.27, North Xisanhuan Road, Beijing, 100089, China. FAU - Zhang, Ling AU - Zhang L AD - School of Pharmaceutical Sciences, Shandong University of Traditional Chinese Medicine, Jinan, 250355, China. FAU - Yi, Hong AU - Yi H AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Li, Chun AU - Li C AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Chen, Liang-Mian AU - Chen LM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Gao, Hui-Min AU - Gao HM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Yan, Li-Hua AU - Yan LH AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. FAU - Liu, Xiao-Qian AU - Liu XQ AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. Electronic address: lianyu1127@126.com. FAU - Wang, Zhi-Min AU - Wang ZM AD - National Engineering Laboratory for Quality Control Technology of Chinese Herbal Medicines, Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China. Electronic address: zmwang@icmm.ac.cn. LA - eng PT - Journal Article DEP - 20240207 PL - Ireland TA - J Ethnopharmacol JT - Journal of ethnopharmacology JID - 7903310 RN - B66F4574UG (angelicae sinensis extract) RN - 0 (Blood Coagulation Factors) RN - 0 (Drugs, Chinese Herbal) RN - 0 (Anticoagulants) RN - 9001-26-7 (Prothrombin) RN - 63231-63-0 (RNA) SB - IM MH - Female MH - Animals MH - Zebrafish MH - Blood Coagulation Factors MH - *Drugs, Chinese Herbal/pharmacology/therapeutic use/chemistry MH - Anticoagulants/pharmacology/therapeutic use MH - Prothrombin MH - *Thrombosis/drug therapy MH - RNA MH - *Angelica sinensis OTO - NOTNLM OT - Anticoagulant activity OT - Coagulation factor OT - Dang-gui-Si-Ni decoction OT - Fingerprint COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/02/10 13:18 MHDA- 2024/02/26 06:44 CRDT- 2024/02/09 19:16 PHST- 2023/11/25 00:00 [received] PHST- 2024/01/30 00:00 [revised] PHST- 2024/02/06 00:00 [accepted] PHST- 2024/02/26 06:44 [medline] PHST- 2024/02/10 13:18 [pubmed] PHST- 2024/02/09 19:16 [entrez] AID - S0378-8741(24)00189-2 [pii] AID - 10.1016/j.jep.2024.117890 [doi] PST - ppublish SO - J Ethnopharmacol. 2024 May 10;325:117890. doi: 10.1016/j.jep.2024.117890. Epub 2024 Feb 7. PMID- 32335662 OWN - NLM STAT- MEDLINE DCOM- 20200819 LR - 20201201 IS - 1815-672X (Electronic) IS - 0028-2715 (Print) IS - 0028-2715 (Linking) VI - 57 IP - 220 DP - 2019 Nov-Dec TI - Diffuse Systemic Sclerosis with Left Ventricular Diastolic Dysfunction: A Case Report. PG - 457-459 AB - Systemic sclerosis is a connective tissue disease characterized by wide-spread vascular lesions and fibrosis of the skin and internal organs. It is an immune mediated rheumatic disease with the presence of an immunological dysfunction of T lymphocytes, especially Th1 and Th17 subtypes. It affects gastrointestinal, pulmonary, vascular, musculoskeletal, cardiac and various other systems. This disease is rare but has high morbidity and mortality with less known effective management. We report a case of 70-year-old female with systemic sclerosis presented with pain along with swelling over multiple joints since 18 months which exacerbated since last 6 months and wound over finger tips since last 2 weeks. We present here other various signs, investigations and management of this uncommon disease systemic sclerosis, also known as scleroderma. Various systems are evident to be involved including cardiac (left ventricular diastolic dysfunction) and peripheral vascular system (Raynaud's phenomenon). Keywords: connective tissue disease; immune-mediated; rheumatic disease; scleroderma; systemic sclerosis. FAU - Neupane, Asmita AU - Neupane A AD - Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal. FAU - Luintel, Prabesh AU - Luintel P AD - Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal. FAU - Dhakal, Subodh Sagar AU - Dhakal SS AD - Department of Internal Medicine, Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal. LA - eng PT - Case Reports PT - Journal Article PL - Nepal TA - JNMA J Nepal Med Assoc JT - JNMA; journal of the Nepal Medical Association JID - 0045233 SB - IM MH - Aged MH - Diastole/*physiology MH - Echocardiography, Doppler MH - Female MH - Humans MH - Scleroderma, Diffuse/complications/*diagnosis/pathology MH - Ventricular Dysfunction, Left/*diagnostic imaging/etiology/physiopathology PMC - PMC7580412 COIS- None. EDAT- 2020/04/27 06:00 MHDA- 2020/08/20 06:00 PMCR- 2019/11/01 CRDT- 2020/04/27 06:00 PHST- 2020/04/27 06:00 [entrez] PHST- 2020/04/27 06:00 [pubmed] PHST- 2020/08/20 06:00 [medline] PHST- 2019/11/01 00:00 [pmc-release] AID - 10.31729/jnma.4768 [doi] PST - ppublish SO - JNMA J Nepal Med Assoc. 2019 Nov-Dec;57(220):457-459. doi: 10.31729/jnma.4768. PMID- 32185274 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 28 IP - 3 DP - 2017 Sep TI - Oxygen-Dependent Patient with Antisynthetase Syndrome Associated Interstitial Lung Disease Responds Promptly to Rituximab with Rapid Pulmonary Function Improvement. PG - 153-156 LID - 10.31138/mjr.28.3.153 [doi] AB - Antisynthetase syndrome (anti-SS) is a rare systemic autoimmune disorder characterized by myositis, Raynaud's phenomenon, fever, interstitial lung disease (ILD), polyarthralgia, and presence of antibodies against tRNA synthetase, especially anti-Jo-1. Rarely, anti-SS can present as isolated ILD, with clinical features very similar to atypical pneumonia, making diagnosis extremely challenging. We report a patient originally diagnosed with atypical pneumonia, requiring oxygen supplementation, who failed treatment with antibiotics. Radiological findings were suspicious for ILD and a comprehensive rheumatological work-up revealed the diagnosis of anti-SS associated ILD. Prompt treatment was initiated with steroids and rituximab. Follow up pulmonary function tests showed an improvement in her diffusing capacity of the lung for carbon monoxide and forced vital capacity allowing her to resume her daily life without supplemental oxygen. CI - © 2017 The Mediterranean Journal of Rheumatology (MJR). FAU - Chao, Robert AU - Chao R AD - Department of Medicine, Weill Cornell Medicine/New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA. FAU - Das, Mukund AU - Das M AD - Department of Medicine, Weill Cornell Medicine/New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA. FAU - Philip, Cecil AU - Philip C AD - Department of Medicine, Weill Cornell Medicine/New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA. FAU - Efthimiou, Petros AU - Efthimiou P AD - Department of Medicine, Weill Cornell Medicine/New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20170929 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7046064 OTO - NOTNLM OT - Antisynthetase syndrome OT - interstitial lung disease OT - myositis OT - rituximab EDAT- 2017/09/29 00:00 MHDA- 2017/09/29 00:01 PMCR- 2017/09/29 CRDT- 2020/03/19 06:00 PHST- 2017/05/14 00:00 [received] PHST- 2017/07/11 00:00 [revised] PHST- 2017/07/25 00:00 [accepted] PHST- 2020/03/19 06:00 [entrez] PHST- 2017/09/29 00:00 [pubmed] PHST- 2017/09/29 00:01 [medline] PHST- 2017/09/29 00:00 [pmc-release] AID - MJR-28-3-153 [pii] AID - 10.31138/mjr.28.3.153 [doi] PST - epublish SO - Mediterr J Rheumatol. 2017 Sep 29;28(3):153-156. doi: 10.31138/mjr.28.3.153. eCollection 2017 Sep. PMID- 39574421 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241123 IS - 2754-0413 (Electronic) IS - 2754-0413 (Linking) VI - 3 IP - 1 DP - 2024 TI - Human papillomavirus nonavalent (HPV9) vaccination and risk of immune mediated diseases, myocarditis, pericarditis, and thromboembolic outcomes in Denmark: self-controlled case series study. PG - e000854 LID - 10.1136/bmjmed-2024-000854 [doi] LID - e000854 AB - OBJECTIVE: To assess the associations between vaccination with the nonavalent human papillomavirus (HPV9) vaccine and immune mediated diseases, myocarditis, pericarditis, arterial thromboembolism, and venous thromboembolism with or without thrombocytopenia, in adolescent girls and boys in Denmark. DESIGN: Self-controlled case series study. SETTING: Population based study of linked nationwide health registers in Denmark for HPV vaccination and hospital diagnosis data, 1 October 2017 (or age 10 years) to 31 December 2022 or censored. Personal data were obtained from the Central Person Register. Information on dates of HPV vaccination and type of vaccine were obtained from the Danish Vaccination Register. Primary or secondary diagnoses of inpatient or outpatient hospital contact were sourced from the Danish National Patient Register. PARTICIPANTS: Source cohort 854 586. 350 687 individuals aged 10-17 years living in Denmark received at least one dose of HPV9 vaccine. Self-controlled case series analysis of 3354 individuals (1913 girls and 1441 boys) who received at least one dose of HPV9 vaccine and had at least one outcome. MAIN OUTCOME MEASURES: Rate ratios of the study outcomes in a 28 day or 180 day risk period (depending on the type of outcome) after HPV9 vaccination compared with the reference period were calculated. 47 immune mediated diseases, myocarditis, pericarditis, and seven thromboembolic outcomes were assessed. A safety signal for a specific outcome was identified if at least three outcomes were seen in the risk period after vaccination, the rate ratio was significantly increased (lower bound of the 95% confidence interval (CI) for the self-controlled case series rate ratio >1.0), and the false discovery rate adjusted P value was significant (<0.05). RESULTS: 696 776 doses of any HPV vaccine were given during the study period, including 673 530 doses of HPV9 vaccine in 350 687 individuals who received at least one dose. In the self-controlled case series analysis, rate ratios of all immune mediated outcomes combined were 0.99 (95% CI 0.86 to 1.13) and 1.03 (0.89 to 1.20) in girls and boys, respectively, after HPV9 vaccination. Rate ratios for any of the 47 analysed immune mediated outcomes were not increased in the risk periods in girls after vaccination. The only increased rate ratio seen was for Raynaud's disease (rate ratio 2.62, 95% CI 1.07 to 6.40) after HPV9 vaccination in boys, which did not fulfil the criteria of a safety signal. These findings should be interpreted in the light of the study limitations. None of the other 55 outcomes examined showed an association with HPV9 vaccination. CONCLUSIONS: The results of this study did not suggest an association between HPV9 vaccination and the study outcomes in adolescent boys and girls aged 10-17 years. This study contributes to the evidence on the safety of the HPV9 vaccine. CI - Copyright © Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Faksová, Kristýna AU - Faksová K AUID- ORCID: 0009-0007-8900-5017 AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. FAU - Laksafoss, Anna D AU - Laksafoss AD AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. FAU - Hviid, Anders AU - Hviid A AUID- ORCID: 0000-0002-7509-9127 AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark. AD - Pharmacovigilance Research Center, Department of Drug Design and Pharmacology, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark. LA - eng PT - Journal Article DEP - 20241022 PL - England TA - BMJ Med JT - BMJ medicine JID - 9918487584306676 PMC - PMC11579538 OTO - NOTNLM OT - Drug-related side effects and adverse reactions OT - Preventive medicine OT - Public health OT - Sexually transmitted diseases COIS- All authors have completed the ICMJE uniform disclosure form at www.icmje.org/disclosure-of-interest/ and declare: support from the Lundbeck Foundation for the submitted work; AH holds unrelated grants from the Novo Nordisk Foundation and Independent Research Fund Denmark; AH is a scientific board member of VAC4EU; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/11/22 06:22 MHDA- 2024/11/22 06:23 PMCR- 2024/10/22 CRDT- 2024/11/22 04:14 PHST- 2024/01/03 00:00 [received] PHST- 2024/09/17 00:00 [accepted] PHST- 2024/11/22 06:23 [medline] PHST- 2024/11/22 06:22 [pubmed] PHST- 2024/11/22 04:14 [entrez] PHST- 2024/10/22 00:00 [pmc-release] AID - bmjmed-2024-000854 [pii] AID - 10.1136/bmjmed-2024-000854 [doi] PST - epublish SO - BMJ Med. 2024 Oct 22;3(1):e000854. doi: 10.1136/bmjmed-2024-000854. eCollection 2024. PMID- 36628007 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230112 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 12 DP - 2022 Dec TI - Early Diagnosis of Systemic Sclerosis: The Role of General Practitioner. PG - e32291 LID - 10.7759/cureus.32291 [doi] LID - e32291 AB - Systemic sclerosis (SSc) is a chronic, rare, and idiopathic disease characterized by the presence of microcirculatory and immune alterations followed by fibrosis. It is clinically heterogeneous and may present a rapid and progressive involvement that leads to disability and death. Over the years, the approach has changed with an increasing focus on early diagnosis. Raynaud's phenomenon (RP) and puffy fingers are "red flags" to refer the patients to rheumatology to detect and start the appropriate treatment of such a rare and complex disease. We present a case of a 75-year-old woman with edema and bright erythema on the back and palm of the hands, telangiectasias of the face, and RP with three years of evolution. The aim of this case is to recall the importance of primary care physicians in recognizing the main clinical manifestations of SSc that are sometimes undervalued. CI - Copyright © 2022, Freixo et al. FAU - Freixo, Armanda AU - Freixo A AD - Unidade de Saúde Familiar (USF) Lethes, Unidade Local de Saúde do Alto Minho, Ponte de Lima, PRT. FAU - Abreu, Cecília AU - Abreu C AD - Unidade de Saúde Familiar (USF) Lethes, Unidade Local de Saúde do Alto Minho, Ponte de Lima, PRT. LA - eng PT - Case Reports PT - Journal Article DEP - 20221207 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9820582 OTO - NOTNLM OT - classification OT - early diagnosis OT - red flags OT - systemic sclerosis OT - very early systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2023/01/12 06:00 MHDA- 2023/01/12 06:01 PMCR- 2022/12/07 CRDT- 2023/01/11 01:45 PHST- 2022/12/07 00:00 [accepted] PHST- 2023/01/11 01:45 [entrez] PHST- 2023/01/12 06:00 [pubmed] PHST- 2023/01/12 06:01 [medline] PHST- 2022/12/07 00:00 [pmc-release] AID - 10.7759/cureus.32291 [doi] PST - epublish SO - Cureus. 2022 Dec 7;14(12):e32291. doi: 10.7759/cureus.32291. eCollection 2022 Dec. PMID- 41704982 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260218 LR - 20260218 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 1 DP - 2026 Jan TI - An Under-Recognized Manifestation of Systemic Sclerosis: Macrovascular Peripheral Arterial Disease. PG - e101734 LID - 10.7759/cureus.101734 [doi] LID - e101734 AB - Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by skin and visceral fibrosis and by a well-known microangiopathy leading to Raynaud's phenomenon, digital ischemia, pulmonary arterial hypertension, and scleroderma renal crisis. By contrast, macrovascular involvement has been less extensively described and may be under-recognized. We report the case of a 66-year-old woman with a 16-year history of SSc. The diagnosis was based on cutaneous sclerosis, Raynaud's phenomenon complicated by digital necrosis, positive antinuclear antibodies, and interstitial lung disease with pulmonary fibrosis. She was admitted for intermittent claudication of the lower limbs. CT angiography of the lower extremities revealed diffusely small-caliber arteries with long, thread-like stenoses, consistent with macrovascular peripheral arterial involvement in the context of SSc. This case illustrates that SSc may be associated not only with microvascular but also with clinically significant macrovascular disease. Awareness of this complication and the use of vascular imaging in symptomatic patients are essential for early diagnosis and appropriate management. CI - Copyright © 2026, Mouharir et al. FAU - Mouharir, Meriem AU - Mouharir M AD - Department of Internal Medicine, Avicenne Military Hospital / Cadi Ayyad University, Marrakesh, MAR. FAU - Kaddouri, Said AU - Kaddouri S AD - Department of Internal Medicine, Avicenne Military Hospital / Cadi Ayyad University, Marrakesh, MAR. FAU - Chahbi, Zakaria AU - Chahbi Z AD - Department of Internal Medicine, Avicenne Military Hospital / Cadi Ayyad University, Marrakesh, MAR. FAU - Qacif, Hassan AU - Qacif H AD - Department of Internal Medicine, Avicenne Military Hospital / Cadi Ayyad University, Marrakesh, MAR. FAU - Zyani, Mohamed AU - Zyani M AD - Department of Internal Medicine, Avicenne Military Hospital / Cadi Ayyad University, Marrakesh, MAR. LA - eng PT - Case Reports PT - Journal Article DEP - 20260117 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12908610 OTO - NOTNLM OT - case report OT - ct angiography OT - intermittent claudication OT - macrovascular involvement OT - peripheral arterial disease OT - systemic sclerosis COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/02/18 10:08 MHDA- 2026/02/18 10:09 PMCR- 2026/01/17 CRDT- 2026/02/18 04:31 PHST- 2026/01/17 00:00 [accepted] PHST- 2026/02/18 10:09 [medline] PHST- 2026/02/18 10:08 [pubmed] PHST- 2026/02/18 04:31 [entrez] PHST- 2026/01/17 00:00 [pmc-release] AID - 10.7759/cureus.101734 [doi] PST - epublish SO - Cureus. 2026 Jan 17;18(1):e101734. doi: 10.7759/cureus.101734. eCollection 2026 Jan. PMID- 28643532 OWN - NLM STAT- MEDLINE DCOM- 20180723 LR - 20181113 IS - 1473-2300 (Electronic) IS - 0300-0605 (Print) IS - 0300-0605 (Linking) VI - 45 IP - 6 DP - 2017 Dec TI - Dermatomyositis with renal infarction: a case report and literature review. PG - 2153-2157 LID - 10.1177/0300060517709673 [doi] AB - Renal infarction is a rare clinical entity that is not easily detected by low-sensitivity ultrasound. We herein report a case of dermatomyositis with renal infarction detected during corticosteroid therapy. The patient was followed up for 18 months. A woman who was clinically diagnosed with dermatomyositis complained of severe pain in the right flank of the low back and abdomen, accompanied by nausea and vomiting during corticosteroid therapy. Based on the findings of routine blood tests, abdominal X-ray radiography, and abdominal ultrasound, the patient was diagnosed with acute gastroenteritis and treated with levofloxacin. However, her symptoms were not relieved. Abdominal contrast-enhanced computed tomography revealed renal infarction. Clinicians should be alert to the occurrence of thrombosis, especially when it manifests as vasculitis in patients with rheumatic disease who complain of severe abdominal pain, because it may suggest the presence of renal infarction. FAU - Zhang, Ti AU - Zhang T AD - Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, the Second Military Medical University, Shanghai, China. FAU - Liu, Xin AU - Liu X AD - Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, the Second Military Medical University, Shanghai, China. FAU - Xu, Huji AU - Xu H AD - Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, the Second Military Medical University, Shanghai, China. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20170623 PL - England TA - J Int Med Res JT - The Journal of international medical research JID - 0346411 SB - IM MH - Dermatomyositis/*complications/diagnostic imaging MH - Female MH - Humans MH - Kidney Diseases/*complications/diagnostic imaging MH - Middle Aged MH - Tomography, X-Ray Computed PMC - PMC5805205 OTO - NOTNLM OT - Dermatomyositis OT - Raynaud’s phenomenon OT - renal infarction OT - vasculitis EDAT- 2017/06/24 06:00 MHDA- 2018/07/24 06:00 PMCR- 2017/12/01 CRDT- 2017/06/24 06:00 PHST- 2017/06/24 06:00 [pubmed] PHST- 2018/07/24 06:00 [medline] PHST- 2017/06/24 06:00 [entrez] PHST- 2017/12/01 00:00 [pmc-release] AID - 10.1177_0300060517709673 [pii] AID - 10.1177/0300060517709673 [doi] PST - ppublish SO - J Int Med Res. 2017 Dec;45(6):2153-2157. doi: 10.1177/0300060517709673. Epub 2017 Jun 23. PMID- 24147535 OWN - NLM STAT- MEDLINE DCOM- 20140714 LR - 20151119 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 9 IP - 11 DP - 2013 Nov TI - A systematic overview on the use and relevance of capillaroscopy in systemic sclerosis. PG - 1091-7 LID - 10.1586/1744666X.2013.849198 [doi] AB - Capillaroscopy is a non-invasive and safe technique that allows the detection and quantification of the early microvascular abnormalities that characterize secondary Raynaud's phenomenon. The well-established role of capillaroscopy for the early diagnosis of systemic sclerosis, its inclusion in the classification criteria, combined with its predictive value for clinical complications of the disease and its potential for monitoring disease progression and treatment response, makes nailfold capillaroscopy an important assessment in clinical practice and research. Capillaroscopy provides a unique window into the microcirculation and its application in diseases in which a microvascular component is suspected; it also may provide new insights into their pathophysiology and natural history. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Department of Clinical Sciences & Community Health, Division of Rheumatology, University of Milan, Piazza Cardinal Ferrari, 1, 20122 Milan, Italy. FAU - Gualtierotti, Roberta AU - Gualtierotti R LA - eng PT - Journal Article PT - Review DEP - 20131022 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - 0 (Biomarkers) RN - 0 (Biomarkers, Pharmacological) SB - IM MH - Animals MH - Biomarkers/*metabolism MH - Biomarkers, Pharmacological/*metabolism MH - Humans MH - Microcirculation MH - Microscopic Angioscopy/*methods/trends MH - Microvessels/*metabolism MH - Scleroderma, Systemic/*diagnosis EDAT- 2013/10/24 06:00 MHDA- 2014/07/16 06:00 CRDT- 2013/10/24 06:00 PHST- 2013/10/24 06:00 [entrez] PHST- 2013/10/24 06:00 [pubmed] PHST- 2014/07/16 06:00 [medline] AID - 10.1586/1744666X.2013.849198 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2013 Nov;9(11):1091-7. doi: 10.1586/1744666X.2013.849198. Epub 2013 Oct 22. PMID- 33164735 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240330 IS - 2147-9720 (Print) IS - 2148-4279 (Electronic) IS - 2147-9720 (Linking) VI - 7 IP - Suppl 3 DP - 2020 Oct TI - New perspectives in the imaging of Raynaud's phenomenon. PG - S212-S221 LID - 10.5152/eurjrheum.2020.19124 [doi] AB - The last 10-20 years have seen huge strides in imaging science. The aim of this review article is to share with the reader the key recent advances in non-invasive imaging of the digital (finger) vasculature in patients with Raynaud's phenomenon (RP), including in systemic sclerosis (SSc)-related digital vasculopathy. For the rheumatologist, seeing a patient with RP is an opportunity for early diagnosis of an underlying SSc-spectrum disorder or (conversely) for reassuring the patient with primary (idiopathic) RP. Non-invasive imaging techniques can help to provide diagnostic certainty. In addition, they can provide new insights into pathophysiology and have the potential to facilitate the development of much needed effective treatments by providing primary and secondary endpoints for randomized controlled trials: validation studies are ongoing. This review article focuses on nailfold capillaroscopy, thermography, and laser Doppler methods but also discusses (briefly) other technologies, including optical coherence tomography, multispectral imaging, and photoacoustic imaging. Key recent advances are the increasing use/availability of nailfold capillaroscopy (and better understanding of the role of low-cost hand-held devices), increased accessibility of thermography (including mobile phone thermography), and increased application of laser Doppler methods to the study of RP/digital vasculopathy (in particular of laser Doppler imaging and laser speckle contrast imaging, both of which measure blood flow over an area rather than at a single site). In an era of precision medicine, non-invasive imaging techniques can help stratify risk of (a) SSc in the patient with RP and (b) digital vascular disease progression in the patient with an SSc-spectrum disorder. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK. AD - Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Dinsdale, Graham AU - Dinsdale G AUID- ORCID: 0000-0001-6245-2721 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK. AD - Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK. FAU - Murray, Andrea AU - Murray A AUID- ORCID: 0000-0001-9244-2882 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK. AD - Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK. LA - eng PT - Journal Article PT - Review DEP - 20200706 PL - Turkey TA - Eur J Rheumatol JT - European journal of rheumatology JID - 101656068 PMC - PMC7647685 COIS- Conflict of Interest: A.L.H. has received research funding from Gesynta, speaker’s fees from Actelion, and consultancy fees from Boehringer-Ingelheim, Gesynta and Camurus. A.M. has received research funding from Actelion and speaker’s fees from Actelion. EDAT- 2020/11/10 06:00 MHDA- 2020/11/10 06:01 PMCR- 2020/10/01 CRDT- 2020/11/09 13:51 PHST- 2019/06/30 00:00 [received] PHST- 2020/03/05 00:00 [accepted] PHST- 2020/11/09 13:51 [entrez] PHST- 2020/11/10 06:00 [pubmed] PHST- 2020/11/10 06:01 [medline] PHST- 2020/10/01 00:00 [pmc-release] AID - eurjrheum.2020.19124 [pii] AID - ejr-7-suppl3-s212 [pii] AID - 10.5152/eurjrheum.2020.19124 [doi] PST - ppublish SO - Eur J Rheumatol. 2020 Oct;7(Suppl 3):S212-S221. doi: 10.5152/eurjrheum.2020.19124. Epub 2020 Jul 6. PMID- 22291739 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20211021 IS - 1896-9151 (Electronic) IS - 1734-1922 (Print) IS - 1734-1922 (Linking) VI - 7 IP - 1 DP - 2011 Feb TI - Angiogenin and SDF-1α serum concentration in patients with systemic sclerosis in relation to clinical status. PG - 92-6 LID - 10.5114/aoms.2011.20610 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a connective tissue disorder characterized by tissue hypoxia due to vascular changes and excessive fibrosis of the skin and internal organs. Damage to blood vessels and endothelium, as well as imbalance of vascular homeostasis, impairment of angiogenesis and vasculogenesis are observed in the course of the disease. The aim of the study was to investigate the pro-angiogenic factors angiogenin and SDF-1α in patients with SSc. MATERIAL AND METHODS: Serum samples were collected from 50 patients with dSSc (diffuse SSc) and lSSc (limited SSc) and from 38 patients used as a healthy control group. We explored: 1) how the serum concentrations of SDF-1α and angiogenin differ in the investigated groups; 2) the correlation among chemokines in SSc and the duration of the disease, Raynaud's phenomenon, sclerosis of the skin and TSS (total skin score). RESULTS: Patients with SSc showed statistically significantly higher serum angiogenin concentration and there was no correlation between duration of the disease and Raynaud's phenomenon, skin sclerosis or TSS. There was also no difference or no correlation between serum level of SDF-1α and the investigated groups. CONCLUSIONS: The increase in angiogenin concentration in the serum in patients with SSc may confirm endothelial damage caused by hypoxia and reduced vascular perfusion due to the course of SSc without contributing to compensatory revascularization. FAU - Dziankowska-Bartkowiak, Bożena AU - Dziankowska-Bartkowiak B AD - Department of Immunodermatology, Medical University of Lodz, Poland. FAU - Gerlicz-Kowalczuk, Zofia AU - Gerlicz-Kowalczuk Z FAU - Waszczykowska, Elżbieta AU - Waszczykowska E LA - eng PT - Journal Article DEP - 20110308 PL - Poland TA - Arch Med Sci JT - Archives of medical science : AMS JID - 101258257 PMC - PMC3258685 OTO - NOTNLM OT - SDF-1α OT - angiogenesis OT - angiogenin OT - systemic sclerosis OT - vasculogenesis EDAT- 2012/02/01 06:00 MHDA- 2012/02/01 06:01 PMCR- 2011/02/01 CRDT- 2012/02/01 06:00 PHST- 2009/09/14 00:00 [received] PHST- 2009/10/07 00:00 [revised] PHST- 2009/12/12 00:00 [accepted] PHST- 2012/02/01 06:00 [entrez] PHST- 2012/02/01 06:00 [pubmed] PHST- 2012/02/01 06:01 [medline] PHST- 2011/02/01 00:00 [pmc-release] AID - AMS-7-1-92 [pii] AID - 10.5114/aoms.2011.20610 [doi] PST - ppublish SO - Arch Med Sci. 2011 Feb;7(1):92-6. doi: 10.5114/aoms.2011.20610. Epub 2011 Mar 8. PMID- 30501848 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2147-9720 (Print) IS - 2148-4279 (Electronic) IS - 2147-9720 (Linking) VI - 5 IP - 4 DP - 2018 Dec TI - Botulinum toxin type A in the treatment of Raynaud's phenomenon: A three-year follow-up study. PG - 224-229 LID - 10.5152/eurjrheum.2018.18013 [doi] AB - OBJECTIVE: Raynaud's phenomenon consists of vasospastic disease of the digital arteries after exposure to cold or stress. It causes an important reduction in the patient's quality of life when severe. The available treatments do not always offer favorable results. METHODS: A 3-year retrospective study was presented. A total of 15 patients with severe Raynaud's phenomenon who required infiltration with botulinum toxin type A participated in the study. In the first and follow-up visits (30 min, 7 days, 3 months, 6 months, and annual), the overall response by the patient was assessed as was the reduction in the number of weekly episodes of Raynaud's phenomenon, improvement in pain by means of the Visual Analogue Scale, and resolution of ulcers and necrosis as efficacy variables. RESULTS: A total of 15 patients were included in the study. After 30 min of infiltration, the immediate results showed a very good perception of response in four patients. After 1 month of treatment, eight patients had obtained and maintained a very good response, persisting throughout the study. A statistically significant reduction in pain was obtained, as well as the number of weekly episodes of Raynaud's phenomenon. Of the seven patients with basal ulcers, five were completely healed at 3 months. Of the patients, 64.3% showed an overall satisfaction level of >8 at the end of treatment. No serious adverse events were observed. CONCLUSION: Botulinum toxin is a useful treatment for severe Raynaud's phenomenon that is generally well tolerated. Its mechanism of action is not based exclusively on vasodilation. Further studies are necessary to define the ideal patient for this treatment, the most appropriate method of administration, and the number of units and frequency of the infiltrations. FAU - Medina, Susana AU - Medina S AUID- ORCID: 0000-0003-3165-5387 AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Gómez-Zubiaur, Alba AU - Gómez-Zubiaur A AUID- ORCID: 0000-0003-3371-7676 AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Valdeolivas-Casillas, Nuria AU - Valdeolivas-Casillas N AUID- ORCID: 0000-0002-8452-7541 AD - Department of Dermatology, Hospital Universitario del Sureste, Arganda del Rey, Madrid, Spain. FAU - Polo-Rodríguez, Isabel AU - Polo-Rodríguez I AUID- ORCID: 0000-0001-9353-4367 AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Ruíz, Lucia AU - Ruíz L AUID- ORCID: 0000-0001-8618-1452 AD - Department of Rheumatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Izquierdo, Carmen AU - Izquierdo C AUID- ORCID: 0000-0002-7367-2938 AD - Department of Radiology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Guirado, Cristina AU - Guirado C AUID- ORCID: 0000-0002-3421-3544 AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Cabrera, Alicia AU - Cabrera A AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. FAU - Trasobares, Lidia AU - Trasobares L AUID- ORCID: 0000-0003-2282-3764 AD - Department of Dermatology, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain. LA - eng PT - Journal Article DEP - 20181012 PL - Turkey TA - Eur J Rheumatol JT - European journal of rheumatology JID - 101656068 PMC - PMC6267752 COIS- Conflict of Interest: The authors have no conflict of interest to declare. EDAT- 2018/12/07 06:00 MHDA- 2018/12/07 06:01 PMCR- 2018/12/01 CRDT- 2018/12/04 06:00 PHST- 2018/04/15 00:00 [received] PHST- 2018/05/06 00:00 [accepted] PHST- 2018/12/04 06:00 [entrez] PHST- 2018/12/07 06:00 [pubmed] PHST- 2018/12/07 06:01 [medline] PHST- 2018/12/01 00:00 [pmc-release] AID - eurjrheum.2018.18013 [pii] AID - ejr-5-4-224 [pii] AID - 10.5152/eurjrheum.2018.18013 [doi] PST - ppublish SO - Eur J Rheumatol. 2018 Dec;5(4):224-229. doi: 10.5152/eurjrheum.2018.18013. Epub 2018 Oct 12. PMID- 21794314 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20110728 IS - 1699-258X (Print) IS - 1699-258X (Linking) VI - 2 IP - 3 DP - 2006 May TI - [Ozone therapy in rheumatic diseases: a systematic review]. PG - 119-23 LID - 10.1016/S1699-258X(06)73032-7 [doi] AB - OBJECTIVE: To perform a systematic review to analyze the efficacy on which the use of ozone therapy in musculoskeletal diseases is based. METHODS: A literature search was performed in PubMed, Embase and the Cochrane Library using highly sensitive search terms to identify all studies on ozone therapy. All studies showing the efficacy or effectiveness of ozone therapy in any musculoskeletal disease were selected. RESULTS: Only 6 relevant studies were identified, 5 in lumbar disk herniation and 1 in Raynaud's syndrome. Of the 5 studies in disk herniation, only 3 were clinical trials and none used random allocation. Study participants were generally patients with symptomatic small discal hernias. There was wide variability in the dose of ozone injected as well as in the controls used for comparison. All outcome measures were subjective and there was no blinded evaluation of the results. The study in Raynaud's syndrome included only 4 patients. Adverse effects were not evaluated in detail. CONCLUSIONS: The use of ozone therapy in musculoskeletal diseases is based on poor quality studies. Currently, data supporting an adequate risk/benefit ratio for ozone therapy in rheumatic diseases is lacking. CI - Copyright © 2006 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved. FAU - Carmona, Loreto AU - Carmona L AD - Unidad de Investigación. Fundación Española de Reumatología. Madrid. España. LA - spa PT - English Abstract PT - Journal Article TT - Revisión sistemática: ozonoterapia en enfermedades reumáticas. DEP - 20081210 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 EDAT- 2006/05/01 00:00 MHDA- 2006/05/01 00:01 CRDT- 2011/07/29 06:00 PHST- 2005/07/19 00:00 [received] PHST- 2005/11/29 00:00 [accepted] PHST- 2011/07/29 06:00 [entrez] PHST- 2006/05/01 00:00 [pubmed] PHST- 2006/05/01 00:01 [medline] AID - S1699-258X(06)73032-7 [pii] AID - 10.1016/S1699-258X(06)73032-7 [doi] PST - ppublish SO - Reumatol Clin. 2006 May;2(3):119-23. doi: 10.1016/S1699-258X(06)73032-7. Epub 2008 Dec 10. PMID- 24009812 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130906 LR - 20211021 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 14 DP - 2013 TI - Peripheral gangrene: A rare presentation of systemic lupus erythematosus in a child. PG - 337-40 LID - 10.12659/AJCR.889290 [doi] AB - PATIENT: Female, 12 FINAL DIAGNOSIS: Antiphospholipid positive SLE Symptoms: Gangrene • Raynaud's phenomenon • autoamputation of the terminal phalanx of the second left hand finger MEDICATION: Prednisolone • mycophenolate mofetil • captopril Clinical Procedure: Renal Biopsy • treatment of lupus nephritis • control of hypertension Specialty: Pediatric rheumatology. OBJECTIVE: Unusual clinical course. BACKGROUND: SLE in children has many manifestations. In several studies on SLE in children, gangrene and Raynaud phenomenon have been described as a rare manifestation of SLE during its course in children. CASE REPORT: We present the case of a 12-year-old girl referred to our center, presenting with peripheral gangrene plus Raynaud's phenomenon, who proved to have SLE. Our patient was treated with steroids and mycophenolate mofetil. She appeared to respond to this combination judging by the disappearance of the digital cyanosis, appearance of extremity pulses, and return of renal function. CONCLUSIONS: This case highlights the importance of precise management and awareness of very rare manifestations of a common disease like SLE. Gangrene can be initial symptom of SLE in children. We recommend SLE evolution in all children with gangrene symptom. FAU - Ziaee, Vahid AU - Ziaee V AD - Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran ; Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran ; Children's Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran. FAU - Yeganeh, Mehrnoush Hassas AU - Yeganeh MH FAU - Moradinejad, Mohammad-Hassan AU - Moradinejad MH LA - eng PT - Journal Article DEP - 20130830 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 PMC - PMC3762519 OTO - NOTNLM OT - Raynaud phenomenon OT - children OT - gangrene OT - systemic lupus erythematosus EDAT- 2013/09/07 06:00 MHDA- 2013/09/07 06:01 PMCR- 2013/08/30 CRDT- 2013/09/07 06:00 PHST- 2013/04/15 00:00 [received] PHST- 2013/05/27 00:00 [accepted] PHST- 2013/09/07 06:00 [entrez] PHST- 2013/09/07 06:00 [pubmed] PHST- 2013/09/07 06:01 [medline] PHST- 2013/08/30 00:00 [pmc-release] AID - 889290 [pii] AID - 10.12659/AJCR.889290 [doi] PST - epublish SO - Am J Case Rep. 2013 Aug 30;14:337-40. doi: 10.12659/AJCR.889290. eCollection 2013. PMID- 16598411 OWN - NLM STAT- MEDLINE DCOM- 20070327 LR - 20220409 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 2 DP - 2007 Feb TI - MCTD: is it rare in India? PG - 205-7 AB - Mixed connective tissue disease (MCTD) has been rarely reported from India. Thus, we did a retrospective analysis of cases of MCTD seen at our hospital during the last 13 years. We found 16 cases among 441 patients with connective tissue disease. All the 16 patients (15 females) of MCTD fulfilled classification criteria by Kasukawa and at least one of the other two (Sharp's and Alarcon-Sergovia). Raynaud's phenomenon, sclerodactyly, puffy fingers, esophageal hypomotility, and pulmonary disease were the most common manifestations. At a median follow-up of 12 months (1-172), 12 patients developed features of limited scleroderma and three patients had pulmonary hypertension. FAU - Lawrence, A AU - Lawrence A AD - Department of Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226 014, India. FAU - Aggarwal, A AU - Aggarwal A FAU - Misra, R AU - Misra R LA - eng PT - Journal Article DEP - 20060406 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/complications/diagnosis/epidemiology MH - India/epidemiology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/complications/diagnosis/*epidemiology MH - Retrospective Studies MH - Scleroderma, Limited/complications/diagnosis/epidemiology EDAT- 2006/04/07 09:00 MHDA- 2007/03/28 09:00 CRDT- 2006/04/07 09:00 PHST- 2005/03/17 00:00 [received] PHST- 2005/07/18 00:00 [accepted] PHST- 2006/04/07 09:00 [pubmed] PHST- 2007/03/28 09:00 [medline] PHST- 2006/04/07 09:00 [entrez] AID - 10.1007/s10067-006-0276-4 [doi] PST - ppublish SO - Clin Rheumatol. 2007 Feb;26(2):205-7. doi: 10.1007/s10067-006-0276-4. Epub 2006 Apr 6. PMID- 19077087 OWN - NLM STAT- MEDLINE DCOM- 20090727 LR - 20090610 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 34 IP - 5 DP - 2009 Jul TI - Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis. PG - e192-5 LID - 10.1111/j.1365-2230.2008.03008.x [doi] AB - Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES. FAU - Ito, K AU - Ito K AD - Department of Dermatology, Kawaguchi Medical Center, Saitama, Japan. ito_kei@jcom.home.ne.jp FAU - Hara, H AU - Hara H FAU - Okada, T AU - Okada T FAU - Terui, T AU - Terui T LA - eng PT - Case Reports PT - Journal Article DEP - 20081209 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 SB - IM MH - Adult MH - Giant Cell Arteritis/*pathology MH - Humans MH - Hypereosinophilic Syndrome/*pathology MH - Leg Dermatoses/*pathology MH - Male MH - Pruritus/pathology EDAT- 2008/12/17 09:00 MHDA- 2009/07/28 09:00 CRDT- 2008/12/17 09:00 PHST- 2008/12/17 09:00 [entrez] PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/07/28 09:00 [medline] AID - CED3008 [pii] AID - 10.1111/j.1365-2230.2008.03008.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2009 Jul;34(5):e192-5. doi: 10.1111/j.1365-2230.2008.03008.x. Epub 2008 Dec 9. PMID- 40148917 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250330 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 9 IP - 1 DP - 2025 Mar 27 TI - A case of digital vasculitis in anti-synthetase syndrome (Anti-OJ subtype). PG - 36 LID - 10.1186/s41927-025-00484-0 [doi] LID - 36 AB - Anti-synthetase syndrome is a rare autoimmune disorder characterised by the presence of autoantibodies against aminoacyl transfer RNA synthetases. We report a unique case of a 54-year-old woman with anti-OJ anti-synthetase syndrome, characterised by the atypical occurrence of digital vasculitis in conjunction with the classic manifestations of anti-synthetase syndrome. Our patient presented with digital vasculitis affecting the right third and fourth fingers, rapidly evolving interstitial lung disease of the organising pneumonia subtype, sub-clinical myositis, arthritis and mechanic's hands. Notably, she had no prior history of Raynaud's phenomenon. Serological tests revealed positive anti-OJ antibodies and weakly positive anti-MI2 antibodies. Our patient's condition was managed with intravenous methylprednisolone then after stepped down to prednisolone and mycophenolate mofetil with successful therapeutic response.Current literature primarily highlights Raynaud's phenomenon and vasculopathy-related ischemia, whether occlusive or non-occlusive in anti-synthetase syndrome. This case study identifies digital vasculitis as a distinctive complication of anti-synthetase syndrome, anti-OJ subtype. It emphasises the importance of recognising vascular complications, including vasculitis, even when classic signs like Raynaud's phenomenon are absent. Further research is crucial to fully understand the range of vascular manifestations associated with anti-synthetase syndrome. CI - © 2025. The Author(s). FAU - Ak, Deniz AU - Ak D AD - Rheumatology, Royal Free Hospital London, London, UK. Deniz.ak2@nhs.net. FAU - Stratton, Richard J AU - Stratton RJ AD - Rheumatology, Royal Free Hospital London, London, UK. AD - Division of Medicine, University College London, London, UK. LA - eng PT - Journal Article DEP - 20250327 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC11948678 OTO - NOTNLM OT - Anti-OJ OT - Anti-synthetase syndrome OT - Digital vasculitis OT - Interstitial lung disease OT - Organising pneumonia OT - Vasculopathy COIS- Declarations. Ethical approval: Not applicable. Consent for publication: Informed and written consent was obtained from the patient for the publication of this case report including their image use. Competing interests: The authors declare no competing interests. EDAT- 2025/03/28 06:25 MHDA- 2025/03/28 06:26 PMCR- 2025/03/27 CRDT- 2025/03/28 00:59 PHST- 2024/10/24 00:00 [received] PHST- 2025/03/14 00:00 [accepted] PHST- 2025/03/28 06:26 [medline] PHST- 2025/03/28 06:25 [pubmed] PHST- 2025/03/28 00:59 [entrez] PHST- 2025/03/27 00:00 [pmc-release] AID - 10.1186/s41927-025-00484-0 [pii] AID - 484 [pii] AID - 10.1186/s41927-025-00484-0 [doi] PST - epublish SO - BMC Rheumatol. 2025 Mar 27;9(1):36. doi: 10.1186/s41927-025-00484-0. PMID- 41180029 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251103 LR - 20251105 IS - 2050-313X (Print) IS - 2050-313X (Electronic) IS - 2050-313X (Linking) VI - 13 DP - 2025 TI - Fever and rhabdomyolysis in a recent traveler: A case report of an uncommon presentation of anti-synthetase syndrome. PG - 2050313X251392319 LID - 10.1177/2050313X251392319 [doi] LID - 2050313X251392319 AB - Anti-synthetase syndrome is a rare systemic autoimmune disease characterized by aminoacyl-tRNA synthetase autoantibodies. As an overlap disease, it can present heterogeneously with Raynaud's phenomenon, rash, arthritis, interstitial lung disease, or myositis. Rhabdomyolysis is not commonly seen. Diagnosis of anti-synthetase syndrome is based on autoantibody positivity and clinical presentation and may be supported by muscle biopsy. We describe an unusual case of a 28-year-old woman with anti-Jo-1 anti-synthetase syndrome presenting with isolated rhabdomyolysis without lung or skin involvement. Our case also describes concurrent influenza infection as a potential trigger for anti-synthetase syndrome. This case illustrates the variability and potential severity of this rare syndrome. CI - © The Author(s) 2025. FAU - Prasad, Rohit AU - Prasad R AUID- ORCID: 0009-0008-9126-7013 AD - Dell Medical School, The University of Texas at Austin, USA. FAU - Mantilla, Bryanna AU - Mantilla B AD - Snoqualmie Valley Health, WA, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20251031 PL - England TA - SAGE Open Med Case Rep JT - SAGE open medical case reports JID - 101638686 PMC - PMC12579117 OTO - NOTNLM OT - anti-synthetase syndrome OT - case report OT - inflammatory myositis OT - rheumatology/clinical immunology COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/11/03 13:42 MHDA- 2025/11/03 13:43 PMCR- 2025/10/31 CRDT- 2025/11/03 07:05 PHST- 2025/07/29 00:00 [received] PHST- 2025/10/14 00:00 [accepted] PHST- 2025/11/03 13:43 [medline] PHST- 2025/11/03 13:42 [pubmed] PHST- 2025/11/03 07:05 [entrez] PHST- 2025/10/31 00:00 [pmc-release] AID - 10.1177_2050313X251392319 [pii] AID - 10.1177/2050313X251392319 [doi] PST - epublish SO - SAGE Open Med Case Rep. 2025 Oct 31;13:2050313X251392319. doi: 10.1177/2050313X251392319. eCollection 2025. PMID- 36874716 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230307 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 1 DP - 2023 Jan TI - Vasculitis Leading to Gangrene: An Early Presentation in a Rheumatoid Arthritis Patient. PG - e34438 LID - 10.7759/cureus.34438 [doi] LID - e34438 AB - Vasculitis is a late complication in rheumatoid arthritis (RA) and is seen in RA patients with long-standing disease. Rheumatoid vasculitis affects small-to-medium-sized vessels. In a few patients, vasculitis develops early in the course of the disease. Here, we report the case of a 32-year-old female who presented with gangrene in the second and third digits of the right foot and gangrene in the second digit of the left foot. She was on hydroxychloroquine and methotrexate for one year since the diagnosis of RA. The patient then developed Raynaud's phenomenon and blackish discoloration of toes. She was started on pulse methylprednisolone, aspirin, nifedipine, and pentoxifylline. As no improvement was seen, intravenous cyclophosphamide was started. There was no improvement even after starting cyclophosphamide, and the gangrene further worsened. Eventually, after consulting the surgical team, it was decided to amputate the digits. The second digits in both feet were subsequently amputated. Hence, a physician should always be careful in checking for signs of vasculitis in RA patients early in the course of the disease as well. CI - Copyright © 2023, Vadher et al. FAU - Vadher, Abhishek AU - Vadher A AD - Internal Medicine, B. J. Medical College, Ahmedabad, IND. FAU - Yeruva, Karthik AU - Yeruva K AD - Internal Medicine, Merit Health River Region Hospital, Vicksburg, USA. FAU - Vora, Chitralekha AU - Vora C AD - Internal Medicine, B. J. Medical and Civil Hospital, Ahmedabad, IND. FAU - Gangu, Karthik AU - Gangu K AD - Internal Medicine, University of Kansas Medical Center, Kansas City, USA. FAU - Baraiya, Swati AU - Baraiya S AD - Family Medicine, Bombay Hospital and Medical Research Center, Mumbai, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20230131 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9979587 OTO - NOTNLM OT - amputation OT - arthritis OT - gangrene OT - rheumatoid OT - vasculitis COIS- The authors have declared that no competing interests exist. EDAT- 2023/03/07 06:00 MHDA- 2023/03/07 06:01 PMCR- 2023/01/31 CRDT- 2023/03/06 03:59 PHST- 2023/01/31 00:00 [accepted] PHST- 2023/03/06 03:59 [entrez] PHST- 2023/03/07 06:00 [pubmed] PHST- 2023/03/07 06:01 [medline] PHST- 2023/01/31 00:00 [pmc-release] AID - 10.7759/cureus.34438 [doi] PST - epublish SO - Cureus. 2023 Jan 31;15(1):e34438. doi: 10.7759/cureus.34438. eCollection 2023 Jan. PMID- 30071936 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2147-9720 (Print) IS - 2148-4279 (Electronic) IS - 2147-9720 (Linking) VI - 5 IP - 4 DP - 2018 Dec TI - Novel case of anti-synthetase syndrome. PG - 275-277 LID - 10.5152/eurjrheum.2018.17167 [doi] AB - Anti-synthetase syndrome (AS) is a heterogeneous group of systemic autoimmune diseases associated with anti-aminoacyl-transfer RNA synthetases. These inflammatory myopathies present with a constellation of symptoms including myositis, arthritis, Raynaud's phenomenon, and interstitial lung disease (ILD). We present a novel case of a 44-year-old female, who presented with Anti-OJ AS with severe myopathy and rhabdomyolysis without evidence of ILD, which, in our literature review and to the best of our knowledge, has not been previously reported. Furthermore, our patient was initially misdiagnosed, highlighting the paucity of cases and physicians' unfamiliarity with this disease. After her diagnosis was confirmed, the patient was successfully treated with high-dose steroids and transitioned to azathioprine, and she continues to do well. This case report emphasizes a novel presentation of the rarely diagnosed AS. We also discuss the significant overlap between the inflammatory myopathies and consolidate relevant pathophysiology and current trends in the management of this disease. FAU - Kapoor, Aniruddh AU - Kapoor A AUID- ORCID: 0000-0003-1372-9485 AD - Department of Internal Medicine, SSM Health St Mary's Hospital, St. Louis, Missouri, USA. FAU - Vaidyan, Philip AU - Vaidyan P AD - Department of Internal Medicine, SSM Health St Mary's Hospital, St. Louis, Missouri, USA. FAU - Jalil, Basmah AU - Jalil B AD - Department of Rheumatology, SSM Health St Mary's Hospital, St. Louis, Missouri, USA. FAU - Upaluri, Chitra AU - Upaluri C AD - Department of Internal Medicine, SSM Health St Mary's Hospital, St. Louis, Missouri, USA. LA - eng PT - Journal Article DEP - 20180620 PL - Turkey TA - Eur J Rheumatol JT - European journal of rheumatology JID - 101656068 PMC - PMC6267745 COIS- Conflict of Interest: The authors have no conflict of interest to declare. EDAT- 2018/08/04 06:00 MHDA- 2018/08/04 06:01 PMCR- 2018/12/01 CRDT- 2018/08/04 06:00 PHST- 2017/12/13 00:00 [received] PHST- 2018/04/04 00:00 [accepted] PHST- 2018/08/04 06:00 [pubmed] PHST- 2018/08/04 06:01 [medline] PHST- 2018/08/04 06:00 [entrez] PHST- 2018/12/01 00:00 [pmc-release] AID - eurjrheum.2018.17167 [pii] AID - ejr-5-4-275 [pii] AID - 10.5152/eurjrheum.2018.17167 [doi] PST - ppublish SO - Eur J Rheumatol. 2018 Dec;5(4):275-277. doi: 10.5152/eurjrheum.2018.17167. Epub 2018 Jun 20. PMID- 17403257 OWN - NLM STAT- MEDLINE DCOM- 20070531 LR - 20220310 IS - 1396-0296 (Print) IS - 1396-0296 (Linking) VI - 20 IP - 1 DP - 2007 Jan-Feb TI - Autoimmune disorders: nail signs and therapeutic approaches. PG - 17-30 AB - Systemic sclerosis (scleroderma, SSc) is an autoimmune disease that targets small and medium-sized arteries and arterioles in the involved tissues, resulting in a fibrotic vasculopathy and tissue fibrosis. Several prominent nail and periungual changes are apparent in scleroderma. Examination of the nail fold capillaries can reveal the nature and extent of microvascular pathology in patients with collagen vascular disease and Raynaud's phenomenon. Among the complications stemming from Raynaud's phenomenon can be painful ischemic digital ulcers. This can be managed, and potentially prevented, through pharmacologic and nonpharmacologic means. Whereas oral calcium channel blockers remain the most convenient therapy, oral endothelin receptor antagonists and intravenous prostaglandins may be important therapeutic advances for ischemic digital vascular lesions. FAU - Sherber, Noëlle S AU - Sherber NS AD - Columbia University Medical Center, New York, New York 10032, USA. ns2292@columbia.edu FAU - Wigley, Fredrick M AU - Wigley FM FAU - Scher, Richard K AU - Scher RK LA - eng PT - Journal Article PT - Review PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 SB - IM MH - Autoimmune Diseases/complications/*diagnosis/*therapy MH - Dermatomyositis/complications/diagnosis/therapy MH - Humans MH - Lupus Erythematosus, Systemic/complications/diagnosis/therapy MH - Nail Diseases/*etiology/pathology MH - Scleroderma, Systemic/complications/diagnosis/therapy RF - 26 EDAT- 2007/04/04 09:00 MHDA- 2007/06/01 09:00 CRDT- 2007/04/04 09:00 PHST- 2007/04/04 09:00 [pubmed] PHST- 2007/06/01 09:00 [medline] PHST- 2007/04/04 09:00 [entrez] AID - DTH108 [pii] AID - 10.1111/j.1529-8019.2007.00108.x [doi] PST - ppublish SO - Dermatol Ther. 2007 Jan-Feb;20(1):17-30. doi: 10.1111/j.1529-8019.2007.00108.x. PMID- 39555179 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241119 IS - 1930-0433 (Print) IS - 1930-0433 (Electronic) IS - 1930-0433 (Linking) VI - 20 IP - 1 DP - 2025 Jan TI - Mixed connective tissue disease: A case of aggressive progression and multisystem involvement. PG - 488-491 LID - 10.1016/j.radcr.2024.09.148 [doi] AB - Mixed connective tissue disease (MCTD) is a rare autoimmune syndrome characterized by overlapping features of systemic sclerosis, systemic lupus erythematosus (SLE), and polymyositis, first described by Sharp et al. in 1972. This case report discusses a 38-year-old female who presented with symptoms indicative of these overlapping conditions, including sclerodactyly, Raynaud's phenomenon, and muscle weakness, confirmed by laboratory findings and imaging. Despite aggressive treatment with immunosuppressive agents such as methotrexate, corticosteroids, and infliximab, the patient's condition progressively deteriorated, leading to severe complications, including interstitial lung disease and gastrointestinal involvement. Mixed connective tissue disease remains a complex and poorly understood condition with a range of clinical presentations and no distinct risk factors. The prognosis varies significantly, with some patients experiencing life-threatening complications while others may achieve complete remission. Diagnostic criteria, such as the Alarcon-Segovia criteria, and serological markers like anti-U1RNP antibodies, are critical in identifying and managing this rare and challenging condition. CI - © 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington. FAU - Odah, Ali Bani AU - Odah AB AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Awashra, Ameer AU - Awashra A AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Sawaftah, Zaid AU - Sawaftah Z AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Sawafta, Ahmed AU - Sawafta A AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Sawafta, Omar AU - Sawafta O AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Hamdan, Dawoud AU - Hamdan D AD - Department of Medicine, An Najah National University, Nablus, Palestine. FAU - Khamaysa, Jehad AU - Khamaysa J AD - Department of Radiology, Tubas Turkish Governmental Hospital, Tubas, Palestine. FAU - Alwani, Aziz AU - Alwani A AD - Department of Medicine, Tubas Turkish Governmental Hospital, Tubas, Palestine. LA - eng PT - Case Reports PT - Journal Article DEP - 20241031 PL - Netherlands TA - Radiol Case Rep JT - Radiology case reports JID - 101467888 PMC - PMC11564058 OTO - NOTNLM OT - Mixed connective tissue disease OT - Systemic lupus erythematosus OT - Systemic sclerosis EDAT- 2024/11/18 16:20 MHDA- 2024/11/18 16:21 PMCR- 2024/10/31 CRDT- 2024/11/18 06:34 PHST- 2024/09/09 00:00 [received] PHST- 2024/09/27 00:00 [revised] PHST- 2024/09/28 00:00 [accepted] PHST- 2024/11/18 16:21 [medline] PHST- 2024/11/18 16:20 [pubmed] PHST- 2024/11/18 06:34 [entrez] PHST- 2024/10/31 00:00 [pmc-release] AID - S1930-0433(24)01096-3 [pii] AID - 10.1016/j.radcr.2024.09.148 [doi] PST - epublish SO - Radiol Case Rep. 2024 Oct 31;20(1):488-491. doi: 10.1016/j.radcr.2024.09.148. eCollection 2025 Jan. PMID- 35546334 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230515 IS - 2147-9720 (Print) IS - 2148-4279 (Electronic) IS - 2147-9720 (Linking) VI - 9 IP - 2 DP - 2022 Apr TI - Adalimumab-Induced Lupus Nephritis: Case Report and Review of the Literature. PG - 108-110 LID - 10.5152/eurjrheum.2022.21059 [doi] AB - Tumor necrosis factor-alpha inhibitors are known causative agents of systemic lupus erythemato- sus but have rarely been implicated in lupus nephritis. A patient with Crohn's disease on long-term adalimumab treatment presented with new-onset Raynaud's phenomenon and was found to have hematuria and proteinuria. Elevated antinuclear, anti-dsDNA, and MPO antibodies were found. A renal biopsy confirmed the diagnosis of lupus nephritis. Adalimumab was discontinued ensuing improvement in urine studies and resolution of dsDNA and MPO antibodies. Adalimumab can induce systemic lupus erythematosus and lupus nephritis. FAU - Kazzi, Brigitte AU - Kazzi B AD - Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Gudenkauf, Brent AU - Gudenkauf B AD - Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Fine, Derek AU - Fine D AD - Division of Nephrology, Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Monroy-Trujillo, Jose Manuel AU - Monroy-Trujillo JM AD - Division of Nephrology, Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Azar, Antoine AU - Azar A AD - Division of Allergy and Clinical Immunology, Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Giannini, Gabriel AU - Giannini G AD - Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA. FAU - Timlin, Homa AU - Timlin H AD - Division of Rheumatology, Department of Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA. LA - eng PT - Journal Article PL - Turkey TA - Eur J Rheumatol JT - European journal of rheumatology JID - 101656068 PMC - PMC10176219 EDAT- 2022/05/14 06:00 MHDA- 2022/05/14 06:01 PMCR- 2022/03/23 CRDT- 2022/05/13 01:39 PHST- 2022/05/13 01:39 [entrez] PHST- 2022/05/14 06:00 [pubmed] PHST- 2022/05/14 06:01 [medline] PHST- 2022/03/23 00:00 [pmc-release] AID - ejr-9-2-108 [pii] AID - 10.5152/eurjrheum.2022.21059 [doi] PST - ppublish SO - Eur J Rheumatol. 2022 Apr;9(2):108-110. doi: 10.5152/eurjrheum.2022.21059. PMID- 38741509 OWN - NLM STAT- MEDLINE DCOM- 20240514 LR - 20260127 IS - 1881-6096 (Print) IS - 1881-6096 (Linking) VI - 76 IP - 5 DP - 2024 May TI - [Treatment Strategies for Anti-Synthetase Syndrome]. PG - 655-659 LID - 10.11477/mf.1416202656 [doi] AB - Anti-aminoacyl tRNA synthetase (ARS) antibodies are the most frequent in idiopathic inflammatory myopathy, notably associated with anti-synthetase syndrome (ASyS), which is characterized by six clinical features: arthritis, myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanical hands. Although patients with ASyS often respond well to initial glucocorticoid (GC) therapy, they tend to have a chronic, recurrent disease course. In anti-ARS-positive patients, the treatment goal involves suppressing disease recurrence and progression while achieving a minimal GC dose. In this regard, the administration and continuation of immunosuppressants, such as calcineurin inhibitors, have been suggested. B-cell depletion therapies are expected to be valuable in patients with refractory ASyS. Moreover, additional antifibrotic agents may be beneficial for patients with progressive fibrosing ILD. FAU - Nakashima, Ran AU - Nakashima R AD - Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University. LA - jpn PT - English Abstract PT - Journal Article PT - Review PL - Japan TA - Brain Nerve JT - Brain and nerve = Shinkei kenkyu no shinpo JID - 101299709 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Humans MH - *Myositis/drug therapy/immunology MH - *Amino Acyl-tRNA Synthetases/immunology/antagonists & inhibitors EDAT- 2024/05/14 06:42 MHDA- 2024/05/14 06:43 CRDT- 2024/05/14 02:53 PHST- 2024/05/14 06:43 [medline] PHST- 2024/05/14 06:42 [pubmed] PHST- 2024/05/14 02:53 [entrez] AID - 1416202656 [pii] AID - 10.11477/mf.1416202656 [doi] PST - ppublish SO - Brain Nerve. 2024 May;76(5):655-659. doi: 10.11477/mf.1416202656. PMID- 23370948 OWN - NLM STAT- MEDLINE DCOM- 20130916 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2013 DP - 2013 Jan 30 TI - Purpura fulminans in a patient with mixed connective tissue disease. LID - 10.1136/bcr-2012-007947 [doi] LID - bcr2012007947 AB - A 43-year-old lady was admitted to the intensive care unit with sepsis. She had a history of mixed connective tissue disease, Raynaud's syndrome and hypothyroidism. 2 days later, she developed a purpuric rash on her face and extremities with a livedoid background. Few days later, her distal fingers and toes became gangrenous which then had to be amputated. Laboratory investigations showed that she was coagulopathic and had multiple organ dysfunctions. Antiphospholipid antibodies were negative; however, protein C and antithrombin III levels were low. A skin biopsy showed fibrinoid necrosis in the vessel wall with microthrombi and red-cell extravasation. A diagnosis of purpura fulminans was made. FAU - Murad, Aizuri A AU - Murad AA AD - Dermatology Department, Adelaide and Meath Hospital, Dublin, Ireland. FAU - Jeffers, Michael AU - Jeffers M FAU - Tobin, Anne-Marie AU - Tobin AM FAU - Connolly, Maureen AU - Connolly M LA - eng PT - Case Reports PT - Journal Article DEP - 20130130 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Anticoagulants) RN - 0 (Protein C) SB - IM MH - Adult MH - Anticoagulants/therapeutic use MH - Diagnosis, Differential MH - Face MH - Female MH - Fingers MH - Humans MH - Mixed Connective Tissue Disease/*complications MH - Protein C/therapeutic use MH - Purpura Fulminans/*complications/diagnosis/drug therapy/pathology MH - Sepsis/complications MH - Skin/pathology MH - Toes PMC - PMC3604251 EDAT- 2013/02/02 06:00 MHDA- 2013/09/17 06:00 PMCR- 2015/01/30 CRDT- 2013/02/02 06:00 PHST- 2013/02/02 06:00 [entrez] PHST- 2013/02/02 06:00 [pubmed] PHST- 2013/09/17 06:00 [medline] PHST- 2015/01/30 00:00 [pmc-release] AID - bcr-2012-007947 [pii] AID - 10.1136/bcr-2012-007947 [doi] PST - epublish SO - BMJ Case Rep. 2013 Jan 30;2013:bcr2012007947. doi: 10.1136/bcr-2012-007947. PMID- 18159702 OWN - NLM STAT- MEDLINE DCOM- 20080206 LR - 20071227 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 3 IP - 134 DP - 2007 Nov 21 TI - [The antisynthetase syndrome: a cause of rapidly progressive interstitial lung disease]. PG - 2675-6, 2679-81 AB - The anti-synthetase syndrome is associated with inflammatory myopathy, interstitial lung disease, polyarthritis, Raynaud's phenomenon and typical skin lesions known as "mechanic's hands". Anti-Jo1 antibodies are frequently present. Interstitial lung disease is the most important feature, as it determines survival and the therapeutic attitude. We report here two cases of anti-synthetase syndrome with acute respiratory failure. Immunosuppressive treatment combining corticosteroids and cyclosporine lead to clinical improvement, regression of radiological lesions and improvement in pulmonary functions. FAU - Jordan Greco, Anne-Sophie AU - Jordan Greco AS AD - Département de médecine interne, CHCVs/Hôpital de Sierre. asjordan@freesurf.ch FAU - Métrailler, Jean-Claude AU - Métrailler JC FAU - Dayer, Eric AU - Dayer E LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Syndrome des antisynthétases: une cause de pneumopathie interstitielle rapidement progressive. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Antibodies, Antinuclear/blood MH - Arthritis/immunology MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/drug therapy/*immunology MH - Middle Aged MH - Myositis/immunology MH - Respiratory Insufficiency/drug therapy/immunology MH - Syndrome EDAT- 2007/12/28 09:00 MHDA- 2008/02/07 09:00 CRDT- 2007/12/28 09:00 PHST- 2007/12/28 09:00 [pubmed] PHST- 2008/02/07 09:00 [medline] PHST- 2007/12/28 09:00 [entrez] PST - ppublish SO - Rev Med Suisse. 2007 Nov 21;3(134):2675-6, 2679-81. PMID- 17401716 OWN - NLM STAT- MEDLINE DCOM- 20070531 LR - 20070402 IS - 0300-8126 (Print) IS - 0300-8126 (Linking) VI - 35 IP - 2 DP - 2007 Apr TI - Chronic vasculitis and polyneuropathy due to infection with Bartonella henselae. PG - 107-9 AB - Bartonella henselae, the causative agent of cat scratch disease and bacillary angiomatosis, is associated with an expanding spectrum of diseases. Here, we report on a 40-year-old patient suffering from chronic recurrent painful ulcers of the toes, distal axonal sensomotor polyneuropathy and Raynaud's phenomenon. Biopsy of the sural nerve demonstrated an axonal neuropathy with a neurogenic muscular atrophy. Treatment with high dose corticosteroids had no beneficial effect. A biopsy taken from a recurring ulcer 7 years after the beginning of the disease revealed superficial ulcerated hyperkeratosis with subepithelial proliferation of small vessels compatible with a diagnosis of verruca peruana, however, without detection of microorganism. Serologic analysis revealed an elevated IFT titer of 1:1,024 against B. henselae. Treatment with erythromycin induced healing of the ulcer, remission of the vasculitis and the polyneuropathy, and a decline of the IFT titer. This case illustrates that B. henselae infection should be considered in patients with vasculitis and polyneuropathic syndromes. FAU - Stockmeyer, B AU - Stockmeyer B AD - Dept. of Medicine III, University Hospital Erlangen, Krankenhausstr. 12, 91054, Erlangen, Germany. Thomas.Harrer@med3.imed.uni-erlangen.de FAU - Schoerner, C AU - Schoerner C FAU - Frangou, P AU - Frangou P FAU - Moriabadi, T AU - Moriabadi T FAU - Heuss, D AU - Heuss D FAU - Harrer, T AU - Harrer T LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Infection JT - Infection JID - 0365307 SB - IM MH - Adult MH - Angiomatosis, Bacillary/*complications MH - Bartonella henselae/isolation & purification MH - Chronic Disease MH - Foot Ulcer/etiology MH - Humans MH - Male MH - Polyneuropathies/*etiology MH - Recurrence MH - Vasculitis/*etiology EDAT- 2007/04/03 09:00 MHDA- 2007/06/01 09:00 CRDT- 2007/04/03 09:00 PHST- 2006/01/13 00:00 [received] PHST- 2006/09/18 00:00 [accepted] PHST- 2007/04/03 09:00 [pubmed] PHST- 2007/06/01 09:00 [medline] PHST- 2007/04/03 09:00 [entrez] AID - 10.1007/s15010-007-6021-3 [doi] PST - ppublish SO - Infection. 2007 Apr;35(2):107-9. doi: 10.1007/s15010-007-6021-3. PMID- 17710842 OWN - NLM STAT- MEDLINE DCOM- 20070928 LR - 20071115 IS - 0300-9009 (Print) IS - 0300-9009 (Linking) VI - 107 IP - 2 DP - 2007 Jun TI - Carotid thrombus formation and extension during anticoagulation: a case report of large vessel disease and hypercoagulable state in systemic sclerosis. PG - 55-7 AB - Systemic sclerosis (SSc) is an autoimmune multisystem disorder of connective tissue characterized by widespread vascular lesions and fibrosis. Limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dsSSC) are both subsets of SSc. The vascular component of SSc is an important part of the disease. Especially Raynaud's phenomenon and microcirculatory abnormalities are well recognized. Enhanced coagulation pathways, decreased fibrinolysis, and endothelial dysfunction probably contribute to vascular events in SSc. Macrovascular disease is not recognized as a major feature of SSc. However, several studies report an increase in large vessel disease and mortality rate attributable to cardiovascular causes. We present a patient with lcSSc with an acute embolic stroke due to a large carotid thrombus. A hypercoagulable state was suspected because of thrombus formation during oral anticoagulation and extension during intravenous heparin treatment. This is one of the few reports of large vessel disease in systemic sclerosis. The hypercoagulable state may be related to systemic sclerosis. FAU - Renard, Dimitri AU - Renard D AD - Department of Neurology, Hôpital Gui de Chauliac, CHU Montpellier, Montpellier, France. dimitrirenard@hotmail.com FAU - Heroum, Chérif AU - Heroum C LA - eng PT - Case Reports PT - Journal Article PL - Italy TA - Acta Neurol Belg JT - Acta neurologica Belgica JID - 0247035 RN - 0 (Anticoagulants) SB - IM MH - Aged MH - Anticoagulants/therapeutic use MH - Carotid Artery Diseases/*etiology MH - Female MH - Humans MH - Hypertension/complications/drug therapy MH - Myocardial Infarction/complications MH - Scleroderma, Systemic/*complications/*physiopathology MH - Smoking MH - Stroke/*etiology MH - Thrombophilia/*etiology MH - Thrombosis/etiology EDAT- 2007/08/23 09:00 MHDA- 2007/09/29 09:00 CRDT- 2007/08/23 09:00 PHST- 2007/08/23 09:00 [pubmed] PHST- 2007/09/29 09:00 [medline] PHST- 2007/08/23 09:00 [entrez] PST - ppublish SO - Acta Neurol Belg. 2007 Jun;107(2):55-7. PMID- 18298553 OWN - NLM STAT- MEDLINE DCOM- 20080527 LR - 20080402 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 38 IP - 4 DP - 2008 Apr TI - Familial scleroderma: nature, nurture or both? PG - 235-42 LID - 10.1111/j.1445-5994.2007.01525.x [doi] AB - BACKGROUND: The aim of the study was to investigate: (i) familial scleroderma (FS) risk factors, (ii) subtype concordance and (iii) relationship between dates (DSO) and ages (ASO) at scleroderma onset. METHODS: Forty-seven cases (23 families; 25 FS pairs) were identified. Scleroderma disease onset was defined by (i) Raynaud's onset, (ii) first symptom onset (1SxO), (iii) second symptom onset (2SxO) and (iv) scleroderma diagnosis (SDx). RESULTS: Female : male and limited : diffuse (L : D) ratios were 8.4:1 and 3.3:1. The Raynaud's onset - SDx interval was longer in limited disease (L : D = 14.6:3.1 years; P = 0.01). Raynaud's first occurred in 36% women > or =50 years. The median differences in ASO between affected family members were 10-12 years. Disease subtype concordance exceeded discordance (16:9 clusters; (P = 0.32) 16:7 families; (P = 0.17)). The observed/expected LL : LD : DD ratios were 14: 8:1/11:7:1 (P = 0.66). FS affected 34% (95% confidence interval 19-50) sister-sister and 44% (95% confidence interval 27-75) mother-daughter pairs. The second family member's SDx was made at the same (9%) or a younger age (80%) than the first family member. In 14 LL disease families ASO was closer between sisters than mothers-daughters (P = 0.07). There was a trend towards closer ages - than dates - at Raynaud's and 1SxO in scleroderma-affected family members (P = 0.054) and closer dates - than ages - at 2SxO (P = 0.02) and SDx. CONCLUSION: FS showed female predominance, relatively late onset Raynaud's, subtype ratios similar to idiopathic scleroderma and earlier SDx in younger family members. Familial L scleroderma has a longer prediagnostic latency than familial D scleroderma. FS is likely under-ascertained. In LL scleroderma, Raynaud's/1SxO is possibly more genetically determined and 2SxO/SDx more environmentally determined. FAU - Englert, H AU - Englert H AD - Department of Rheumatology, Westmead Hospital, Sydney, New South Wales, Australia. helenen@westgate.wh.usyd.edu.au FAU - Roberts-Thomson, P J AU - Roberts-Thomson PJ FAU - Byth, K AU - Byth K FAU - Manolios, N AU - Manolios N LA - eng PT - Journal Article DEP - 20080220 PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Family MH - Female MH - Genetic Predisposition to Disease MH - Humans MH - Male MH - Middle Aged MH - Risk Factors MH - Scleroderma, Localized/*etiology/genetics EDAT- 2008/02/27 09:00 MHDA- 2008/05/28 09:00 CRDT- 2008/02/27 09:00 PHST- 2008/02/27 09:00 [pubmed] PHST- 2008/05/28 09:00 [medline] PHST- 2008/02/27 09:00 [entrez] AID - IMJ1525 [pii] AID - 10.1111/j.1445-5994.2007.01525.x [doi] PST - ppublish SO - Intern Med J. 2008 Apr;38(4):235-42. doi: 10.1111/j.1445-5994.2007.01525.x. Epub 2008 Feb 20. PMID- 27594777 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 1068-0640 (Print) IS - 1068-0640 (Linking) VI - 23 IP - 5 DP - 2016 Sep TI - The Diagnosis and Treatment of Antisynthetase Syndrome. PG - 218-226 AB - Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud's phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results. Patients with anti-synthetase syndrome often require multi-modality immunosuppressive therapy in order to control the muscle and/or pulmonary manifestations of their disease. The long-term care of these patients mandates careful attention to the adverse effects and complications of chronic immunosuppressive therapy, as well as disease-related sequelae that can include progressive interstitial lung disease necessitating lung transplantation, pulmonary hypertension, malignancy and decreased survival. It is hoped that greater awareness of the clinical features of this syndrome will allow for earlier diagnosis and appropriate treatment to improve outcomes in patients with anti-synthetase syndrome. FAU - Witt, Leah J AU - Witt LJ AD - Section of Pulmonary and Critical Care Medicine, Department of Medicine, The University of Chicago Medicine, Chicago, IL 60637. FAU - Curran, James J AU - Curran JJ AD - Section of Rheumatology, Department of Medicine, The University of Chicago Medicine, Chicago, IL 60637. FAU - Strek, Mary E AU - Strek ME AD - Section of Pulmonary and Critical Care Medicine, Department of Medicine, The University of Chicago Medicine, Chicago, IL 60637. LA - eng GR - T32 HL007605/HL/NHLBI NIH HHS/United States PT - Journal Article PL - United States TA - Clin Pulm Med JT - Clinical pulmonary medicine JID - 9421562 PMC - PMC5006392 MID - NIHMS800523 COIS- no authors have conflicts of interest to disclose EDAT- 2016/09/07 06:00 MHDA- 2016/09/07 06:01 PMCR- 2017/09/01 CRDT- 2016/09/06 06:00 PHST- 2016/09/06 06:00 [entrez] PHST- 2016/09/07 06:00 [pubmed] PHST- 2016/09/07 06:01 [medline] PHST- 2017/09/01 00:00 [pmc-release] AID - 10.1097/CPM.0000000000000171 [doi] PST - ppublish SO - Clin Pulm Med. 2016 Sep;23(5):218-226. doi: 10.1097/CPM.0000000000000171. PMID- 25874345 OWN - NLM STAT- MEDLINE DCOM- 20160112 LR - 20191210 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 17 IP - 5 DP - 2015 May TI - New ACR EULAR guidelines for systemic sclerosis classification. PG - 32 LID - 10.1007/s11926-015-0506-3 [doi] AB - The American College of Rheumatology and European League Against Rheumatism classification criteria for systemic sclerosis are a significant advancement in the field. This article describes the innovative, rigorous, criteria development strategy that was used. The new criteria build upon previous criteria by incorporating important elements (proximal scleroderma, sclerodactyly, digital pits, pulmonary fibrosis, Raynaud's phenomenon, and scleroderma specific autoantibodies). The new criteria add emphasis to the vasculopathic manifestations, and include the early manifestation of puffy fingers. Together, these enhancements have resulted in a shift in the conceptual framework of the disease for the next generation. The new criteria have improved sensitivity and specifically, particularly among cases with early disease, mild disease, or limited disease. The ability to classify more cases, at an earlier stage, may confer the opportunity to intervene and prevent disease progression. Undoubtedly, this will lead to a paradigm shift in the conduct of clinical trials in systemic sclerosis. FAU - Johnson, Sindhu R AU - Johnson SR AD - Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto, Ground Floor, East Wing, 399 Bathurst Street, Toronto, ON, Canada, M5T 2S8, Sindhu.Johnson@uhn.ca. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Conflict, Psychological MH - Humans MH - *Practice Guidelines as Topic MH - Scleroderma, Systemic/classification/*diagnosis/immunology MH - Sensitivity and Specificity EDAT- 2015/04/16 06:00 MHDA- 2016/01/13 06:00 CRDT- 2015/04/16 06:00 PHST- 2015/04/16 06:00 [entrez] PHST- 2015/04/16 06:00 [pubmed] PHST- 2016/01/13 06:00 [medline] AID - 10.1007/s11926-015-0506-3 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2015 May;17(5):32. doi: 10.1007/s11926-015-0506-3. PMID- 35382020 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240825 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 5 IP - 2 DP - 2020 Jun TI - Patient-reported outcome instruments in clinical trials of systemic sclerosis. PG - 90-102 LID - 10.1177/2397198319886496 [doi] AB - Patient-reported outcome instruments provide valuable insight into disease-related morbidity known only to the patient and complement more objective outcome tools in the clinical trial setting. They are of particular importance in systemic sclerosis owing to the challenges around defining disease activity, the episodic nature of many disease-specific manifestations and the paucity of validated objective surrogate outcome measures for use in clinical trials. Early clinical trials of systemic sclerosis often incorporated legacy patient-reported outcome instruments, but the last 20 years has witnessed the emergence of several scleroderma-specific instruments that are now being routinely used alongside other outcomes in systemic sclerosis clinical trials. More recently, the value of patient-reported outcomes has been highlighted by their prominence in the American College of Rheumatology Combined Response Index for Systemic Sclerosis that has been utilized as the primary endpoint of recent clinical trials of early diffuse systemic sclerosis. This review considers the role and performance of the various patient-reported outcome instruments utilized in systemic sclerosis clinical trials, the current positioning of patient-reported outcome instruments within clinical trial endpoint models across the range of systemic sclerosis disease manifestations and, where applicable, we shall highlight areas for future research. CI - © The Author(s) 2019. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Royal National Hospital for Rheumatic Diseases, Bath, UK. AD - Department of Pharmacy & Pharmacology, University of Bath, Bath, UK. FAU - Caetano, Joana AU - Caetano J AD - Systemic Immune-Mediated Diseases Unit, Department of Medicine IV, Fernando Fonseca Hospital, Amadora, Portugal. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy. FAU - De Luca, Giacomo AU - De Luca G AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy. FAU - Gheorghiu, Ana Maria AU - Gheorghiu AM AD - Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AD - Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, Brescia, Italy. AD - Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy. FAU - Khanna, Dinesh AU - Khanna D AD - Scleroderma Program, Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. LA - eng PT - Journal Article PT - Review DEP - 20191125 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922614 OTO - NOTNLM OT - Patient-reported outcome instruments OT - Raynaud’s phenomenon OT - clinical trials OT - outcome measures OT - systemic sclerosis COIS- Declaration of conflicting interests: J.P. has consultancy relationships with Actelion and Boehringer Ingelheim. None of the other authors declared any relevant conflicts of interest. EDAT- 2020/06/01 00:00 MHDA- 2020/06/01 00:01 PMCR- 2021/06/01 CRDT- 2022/04/06 05:10 PHST- 2019/03/19 00:00 [received] PHST- 2019/10/04 00:00 [accepted] PHST- 2022/04/06 05:10 [entrez] PHST- 2020/06/01 00:00 [pubmed] PHST- 2020/06/01 00:01 [medline] PHST- 2021/06/01 00:00 [pmc-release] AID - 10.1177_2397198319886496 [pii] AID - 10.1177/2397198319886496 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2020 Jun;5(2):90-102. doi: 10.1177/2397198319886496. Epub 2019 Nov 25. PMID- 30989453 OWN - NLM STAT- MEDLINE DCOM- 20191125 LR - 20200225 IS - 1591-9528 (Electronic) IS - 1591-8890 (Linking) VI - 19 IP - 3 DP - 2019 Aug TI - Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study. PG - 357-366 LID - 10.1007/s10238-019-00553-y [doi] AB - Vasculopathy is a crucial feature of systemic sclerosis (SSc), and Raynaud's phenomenon (RP) and digital ulcers (DU) have a deep impact on the quality of patients' life. The management of vascular disease can be challenging for the clinician because of the suboptimal tolerability of the treatments and lack of consensus on the best therapeutic approach. Intravenous iloprost, a synthetic analogue of prostacyclin, is broadly used for the treatment of RP and ischemic ulcers secondary to SSc. However, no standardized protocol on iloprost use is currently available and, consequently, the management of this treatment is largely based on the experience of each single center. The PROSIT project is an observational, multicenter study aiming to investigate the current treatments for SSc vasculopathy, the use of prostanoids, with special regard to iloprost, and the perception of the treatment from a patient's perspective. The study was conducted on a cohort of 346 patients from eight Italian centers and included a structured survey addressed to physicians, data collected from patient's medical records and two patient-administered questionnaires assessing the level of satisfaction, tolerability and perception of the efficacy of Iloprost. PROSIT data confirmed that in the contest of SSc iloprost represents the first-line choice for the management of severe RP and DU. Moreover, it is a well-tolerated treatment as reported by patients' experience. Although a standard protocol for the treatment of SSc-related vasculopathy is lacking, PROSIT study identified different therapeutic approaches largely supported by tertiary Italian centers. Further studies are needed in order to optimize the best treatment for SSc vascular diseases, in particular to improve the best iloprost schedule management. FAU - Negrini, Simone AU - Negrini S AUID- ORCID: 0000-0003-1267-4320 AD - Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy. negrini@unige.it. FAU - Magnani, Ottavia AU - Magnani O AD - Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, AOU Careggi - University of Florence, Florence, Italy. FAU - Carignola, Renato AU - Carignola R AD - Internal Medicine, San Luigi Gonzaga Hospital Orbassano, Turin, Italy. FAU - Data, Valeria AU - Data V AD - Internal Medicine, San Luigi Gonzaga Hospital Orbassano, Turin, Italy. FAU - Montabone, Erika AU - Montabone E AD - Internal Medicine, San Luigi Gonzaga Hospital Orbassano, Turin, Italy. FAU - Santaniello, Alessandro AU - Santaniello A AD - Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Adorni, Giuditta AU - Adorni G AD - Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Murdaca, Giuseppe AU - Murdaca G AD - Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy. FAU - Puppo, Francesco AU - Puppo F AD - Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy. FAU - Indiveri, Francesco AU - Indiveri F AD - Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - D'Ascanio, Anna AU - D'Ascanio A AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero Universitaria, Modena, Italy. FAU - Ferri, Clodoveo AU - Ferri C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero Universitaria, Modena, Italy. FAU - Lumetti, Federica AU - Lumetti F AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero Universitaria, Modena, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Rheumatology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. FAU - Canestrari, Giovanni AU - Canestrari G AD - Rheumatology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. FAU - Bellando Randone, Silvia AU - Bellando Randone S AD - Department of Experimental and Clinical Medicine, AOU Careggi - University of Florence, Florence, Italy. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Experimental and Clinical Medicine, AOU Careggi - University of Florence, Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, AOU Careggi - University of Florence, Florence, Italy. FAU - Battaglia, Elisabetta AU - Battaglia E AD - Rheumatology Unit, ARNAS Garibaldi, Catania, Italy. FAU - De Andres, Maria Ilenia AU - De Andres MI AD - Rheumatology Unit, ARNAS Garibaldi, Catania, Italy. FAU - Russo, Alessandra Azzurra AU - Russo AA AD - Rheumatology Unit, ARNAS Garibaldi, Catania, Italy. FAU - Beretta, Lorenzo AU - Beretta L AD - Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20190415 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - *Disease Management MH - Female MH - Humans MH - Iloprost/*therapeutic use MH - Italy MH - Male MH - Middle Aged MH - Peripheral Vascular Diseases/*drug therapy/*pathology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/*drug therapy MH - Tertiary Care Centers MH - Treatment Outcome MH - Vasodilator Agents/*therapeutic use MH - Young Adult OTO - NOTNLM OT - Digital ulcers OT - Iloprost OT - PROSIT OT - Raynaud’s phenomenon OT - Systemic sclerosis OT - Vasculopathy EDAT- 2019/04/17 06:00 MHDA- 2019/11/26 06:00 CRDT- 2019/04/17 06:00 PHST- 2018/12/21 00:00 [received] PHST- 2019/04/10 00:00 [accepted] PHST- 2019/04/17 06:00 [pubmed] PHST- 2019/11/26 06:00 [medline] PHST- 2019/04/17 06:00 [entrez] AID - 10.1007/s10238-019-00553-y [pii] AID - 10.1007/s10238-019-00553-y [doi] PST - ppublish SO - Clin Exp Med. 2019 Aug;19(3):357-366. doi: 10.1007/s10238-019-00553-y. Epub 2019 Apr 15. PMID- 41049026 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251006 LR - 20251008 IS - 2051-3380 (Print) IS - 2051-3380 (Electronic) IS - 2051-3380 (Linking) VI - 13 IP - 10 DP - 2025 Oct TI - The Case for Autoantibody Screening in Stone Benchtop Workers. PG - e70363 LID - 10.1002/rcr2.70363 [doi] LID - e70363 AB - Occupational silica exposure is a recognised cause of respiratory diseases such as silicosis, with increasing evidence linking it to systemic autoimmune diseases. We report a case of a 40-year-old stone benchtop fabricator diagnosed with complicated silicosis through a screening program. He was initially asymptomatic apart from Raynaud's phenomenon but had an elevated antinuclear antibody (ANA) titre. Over time, he developed progressive autoimmune disease with multisystem involvement and respiratory decline. This case highlights the potential for silica exposure to trigger severe autoimmune disease and the importance of proactive autoimmune screening in high-risk occupational groups. ANA positivity in otherwise asymptomatic individuals may signal underlying or future autoimmune disease. Clinicians should maintain a high index of suspicion for autoimmune conditions in workers exposed to silica, as early detection offers the potential to mitigate long-term morbidity through earlier intervention. CI - © 2025 The Author(s). Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. FAU - Lin, Tiffany AU - Lin T AUID- ORCID: 0000-0002-5255-0130 AD - Department of Respiratory Medicine Alfred Health Melbourne Australia. FAU - Khoo, Jun Keng AU - Khoo JK AD - Department of Respiratory Medicine Alfred Health Melbourne Australia. FAU - Hoy, Ryan AU - Hoy R AD - Department of Respiratory Medicine Alfred Health Melbourne Australia. AD - Monash Centre for Occupational and Environmental Health Monash University Melbourne Australia. LA - eng PT - Journal Article DEP - 20251002 PL - United States TA - Respirol Case Rep JT - Respirology case reports JID - 101631052 PMC - PMC12490934 OTO - NOTNLM OT - antinuclear antibody OT - autoimmune disease OT - silica OT - systemic sclerosis OT - vasculitis COIS- The authors declare no conflicts of interest. EDAT- 2025/10/06 12:31 MHDA- 2025/10/06 12:32 PMCR- 2025/10/02 CRDT- 2025/10/06 07:16 PHST- 2025/08/17 00:00 [received] PHST- 2025/09/15 00:00 [revised] PHST- 2025/09/19 00:00 [accepted] PHST- 2025/10/06 12:32 [medline] PHST- 2025/10/06 12:31 [pubmed] PHST- 2025/10/06 07:16 [entrez] PHST- 2025/10/02 00:00 [pmc-release] AID - RCR270363 [pii] AID - 10.1002/rcr2.70363 [doi] PST - epublish SO - Respirol Case Rep. 2025 Oct 2;13(10):e70363. doi: 10.1002/rcr2.70363. eCollection 2025 Oct. PMID- 37667720 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230906 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 8 DP - 2023 Aug TI - Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus. PG - e42922 LID - 10.7759/cureus.42922 [doi] LID - e42922 AB - This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE). This is the case of a young female with lupus and end-stage renal disease secondary to lupus nephritis who presented to the emergency department for shortness of breath and bilateral leg swelling that eventually progressed to catastrophic antiphospholipid syndrome. She developed pulmonary embolisms, axillary hematoma, and bilateral lower extremity digital gangrene. The treatment course consisted of anticoagulation, steroids, intravenous immunoglobulin (IVIG), above-knee amputation, and eventually rituximab. Diagnosis and treatment of digital ischemia can be complex, especially, in the setting of lupus where the differential diagnosis is broad. A high index of suspicion for CAPS is essential for early diagnosis and treatment. CI - Copyright © 2023, Liang et al. FAU - Liang, Jessica AU - Liang J AD - Internal Medicine, Wayne State University Detroit Medical Center, Detroit, USA. FAU - Mahmood, Raai AU - Mahmood R AD - Rheumatology, Wayne State University Detroit Medical Center, Detroit, USA. FAU - Benchaala, Ilyes AU - Benchaala I AD - Rheumatology, Wayne State University Detroit Medical Center, Detroit, USA. FAU - York, Russel AU - York R AD - Rheumatology, Wayne State University Detroit Medical Center, Detroit, USA. FAU - Sarakbi, Housam AU - Sarakbi H AD - Rheumatology, Wayne State University Detroit Medical Center, Detroit, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230803 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10474971 OTO - NOTNLM OT - antiphospholipid antibody syndrome (aps) OT - catastrophic antiphospholipid syndrome (caps) OT - intravenous immunoglobulins (ivig) OT - lupus mesenteric vasculitis OT - raynaud’s phenomenon OT - rituximab therapy OT - sle and lupus nephritis OT - systemic lupus erythema COIS- The authors have declared that no competing interests exist. EDAT- 2023/09/05 06:42 MHDA- 2023/09/05 06:43 PMCR- 2023/08/03 CRDT- 2023/09/05 03:35 PHST- 2023/07/20 00:00 [accepted] PHST- 2023/09/05 06:43 [medline] PHST- 2023/09/05 06:42 [pubmed] PHST- 2023/09/05 03:35 [entrez] PHST- 2023/08/03 00:00 [pmc-release] AID - 10.7759/cureus.42922 [doi] PST - epublish SO - Cureus. 2023 Aug 3;15(8):e42922. doi: 10.7759/cureus.42922. eCollection 2023 Aug. PMID- 38939289 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240629 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 5 DP - 2024 May TI - Anti-Ha Antisynthetase Syndrome: A Case Report. PG - e61251 LID - 10.7759/cureus.61251 [doi] LID - e61251 AB - Anti-synthetase syndrome (ASyS) is a rare systemic autoimmune myopathy characterized by the involvement of muscles, lungs, and joints, in addition to Raynaud's phenomenon, "mechanics' hand," and fever. Laboratory ASyS is defined by the positivity of anti-aminoacyl-tRNA synthetase autoantibodies, of which anti-Jo-1 is the most common. Herein, we reported an ASyS defined by an anti-Ha autoantibody, which has rarely been described in the literature. Moreover, to the best of our knowledge, we reported the first case of anti-Ha ASyS in Brazil. CI - Copyright © 2024, De Andrade et al. FAU - De Andrade, Vanessa P AU - De Andrade VP AD - Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, BRA. FAU - Miossi, Renata AU - Miossi R AD - Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, BRA. FAU - De Souza, Fernando H AU - De Souza FH AD - Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, BRA. FAU - Shinjo, Samuel K AU - Shinjo SK AD - Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, BRA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240528 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11210826 OTO - NOTNLM OT - antisythetase syndrome OT - autoimmune rheumatic disease OT - case report OT - inflammatory myopathy OT - rheumatic disease COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/06/28 06:42 MHDA- 2024/06/28 06:43 PMCR- 2024/05/28 CRDT- 2024/06/28 04:57 PHST- 2024/05/28 00:00 [accepted] PHST- 2024/06/28 06:43 [medline] PHST- 2024/06/28 06:42 [pubmed] PHST- 2024/06/28 04:57 [entrez] PHST- 2024/05/28 00:00 [pmc-release] AID - 10.7759/cureus.61251 [doi] PST - epublish SO - Cureus. 2024 May 28;16(5):e61251. doi: 10.7759/cureus.61251. eCollection 2024 May. PMID- 22131856 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20211021 IS - 1918-1515 (Electronic) IS - 1205-6626 (Print) IS - 1205-6626 (Linking) VI - 16 IP - 4 DP - 2011 Winter TI - Emerging new uses of phosphodiesterase-5 inhibitors in cardiovascular diseases. PG - e30-5 AB - Phosphodiesterase type-5 (PDE-5) is an enzyme that catalyzes the hydrolytic degradation of cyclic GMP - an essential intracellular second messenger that modulates diverse biological processes in living cells. Three selective inhibitors of PDE-5 - sildenafil, vardenafil and tadalafil - have been successfully used by millions of men worldwide for the treatment of erectile dysfunction. Also, sildenafil and tadalafil are currently approved for the treatment of pulmonary hypertension. Recent powerful basic science data and clinical studies suggest potential nonurological applications of PDE-5 inhibitors, including ischemia/reperfusion injury, myocardial infarction, cardiac hypertrophy, cardiomyopathy, heart failure, stroke, neurodegenerative diseases and other circulatory disorders including Raynaud's phenomenon. Future carefully controlled clinical trials would hopefully expedite their expanding therapeutic use in patients with cardiovascular disease. FAU - Kukreja, Rakesh C AU - Kukreja RC AD - Department of Internal Medicine, Division of Cardiology, Virginia Commonwealth University Pauley Heart Center, Richmond, Virginia, USA. FAU - Salloum, Fadi N AU - Salloum FN FAU - Das, Anindita AU - Das A FAU - Koka, Saisudha AU - Koka S FAU - Ockaili, Ramzi A AU - Ockaili RA FAU - Xi, Lei AU - Xi L LA - eng GR - R01 HL051045/HL/NHLBI NIH HHS/United States GR - R01 HL079424/HL/NHLBI NIH HHS/United States GR - R01 HL093685/HL/NHLBI NIH HHS/United States GR - R37 HL051045/HL/NHLBI NIH HHS/United States PT - Journal Article PL - Canada TA - Exp Clin Cardiol JT - Experimental and clinical cardiology JID - 9715903 PMC - PMC3206106 OTO - NOTNLM OT - Cardiovascular disease OT - Ischemia/reperfusion injury OT - Phosphodiesterase-5 inhibitors EDAT- 2011/12/02 06:00 MHDA- 2011/12/02 06:01 PMCR- 2011/12/01 CRDT- 2011/12/02 06:00 PHST- 2011/12/02 06:00 [entrez] PHST- 2011/12/02 06:00 [pubmed] PHST- 2011/12/02 06:01 [medline] PHST- 2011/12/01 00:00 [pmc-release] AID - ecc16e030 [pii] PST - ppublish SO - Exp Clin Cardiol. 2011 Winter;16(4):e30-5. PMID- 28018840 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2198-6002 (Print) IS - 2198-6002 (Linking) VI - 2 IP - 3 DP - 2016 Sep TI - Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment. PG - 252-269 LID - 10.1007/s40674-016-0052-9 [doi] AB - Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life. However, despite this knowledge, most diagnostic and treatment principles in SSc are focused on visceral manifestations due to known associations with morbidity and mortality. Treatment of inflammatory arthritis is symptom based and involves corticosteroids ≤10mg daily, methotrexate, tumor necrosis factor inhibitors, tocilizumab, and abatacept. Small joint contractures are managed by principles of occupational hand therapy and rarely surgical procedures. TFRs may be treated similar to inflammatory arthritis with corticosteroids. All patients with RP and DU should keep digits covered and warm and avoid vasoconstrictive agents. Pharmacologic management of RP begins with use of calcium channel blockers, but additional agents that may be considered are fluoxetine and phosphodiesterase 5 (PDE5) inhibitors. DU management also involves vasodilators including calcium channel blockers and PDE5 inhibitors; bosentan has also been shown to prevent DU. In patients with severe RP and active DU, intravenous epoprostenol or iloprost can be used and surgical procedures, such as botulinum injections and digital sympathectomies, may be considered. For those with early diffuse cutaneous SSc needing immunosuppression for skin sclerosis, methotrexate or mycophenolate mofetil can be used, but the agent of choice depends on co-existing manifestations, such as inflammatory arthritis and/or lung involvement. Various pharmacologic agents for calcinosis have been considered but are generally ineffective; however, surgical options, including excision of areas of calcinosis, can be considered. Overall management of hand impairment for all patients with SSc should include occupational hand therapy techniques such as range of motion exercises, paraffin wax, and devices to assist in ADL. Thus, treatment options for the various manifestations contributing to hand impairment in SSc are limited and often modestly efficacious at best. Robust studies are needed to address the manifestations of SSc that contribute to hand impairment. FAU - Young, Amber AU - Young A AD - Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; University of Michigan Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Namas, Rajaie AU - Namas R AD - Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; University of Michigan Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Dodge, Carole AU - Dodge C AD - Division of Occupational and Physical Therapy, University of Michigan, Ann Arbor, MI, USA. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; University of Michigan Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - T32 AR007080/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20160719 PL - Switzerland TA - Curr Treatm Opt Rheumatol JT - Current treatment options in rheumatology JID - 101649954 PMC - PMC5176259 MID - NIHMS804241 OTO - NOTNLM OT - Raynaud's phenomenon OT - acro-osteolysis OT - arthralgias OT - calcinosis OT - digital ulcers OT - hand impairment OT - hand involvement OT - inflammatory arthritis OT - joint contractures OT - puffy hands OT - scleroderma OT - skin sclerosis OT - systemic sclerosis OT - tendon friction rubs EDAT- 2016/12/27 06:00 MHDA- 2016/12/27 06:01 PMCR- 2017/09/01 CRDT- 2016/12/27 06:00 PHST- 2016/12/27 06:00 [entrez] PHST- 2016/12/27 06:00 [pubmed] PHST- 2016/12/27 06:01 [medline] PHST- 2017/09/01 00:00 [pmc-release] AID - 10.1007/s40674-016-0052-9 [doi] PST - ppublish SO - Curr Treatm Opt Rheumatol. 2016 Sep;2(3):252-269. doi: 10.1007/s40674-016-0052-9. Epub 2016 Jul 19. PMID- 19041071 OWN - NLM STAT- MEDLINE DCOM- 20090212 LR - 20161124 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 22 IP - 6 DP - 2008 Dec TI - Modern imaging techniques: a revolution for rheumatology practice. PG - 951-9 LID - 10.1016/j.berh.2008.08.007 [doi] AB - Imaging has changed rheumatology practice in terms of both diagnostic approach and knowledge of disease mechanisms. Clinical symptoms, disease signs and the results of physical examination have been more closely related to their anatomical basis. In particular, ultrasonography (US), magnetic resonance imaging and videocapillaroscopy allow diagnosis of disease in its early phase. Novel imaging studies have contributed to elucidate several pathogenetic mechanisms in musculoskeletal diseases; allow evaluation of the real degree of joint inflammation, which is often uncoupled from clinical signs; and possibly reduce the need for large clinical trials. US-guided intra-articular and soft tissue injections nearly always reach the right target, improving the efficacy of the injection and avoiding several possible side-effects. Videocapillaroscopy allow differential diagnosis in Raynaud's phenomenon and may predict disease progression. In conclusion, new imaging techniques and refinements of the established techniques have opened exciting perspectives in our understanding and treatment of many rheumatic diseases. FAU - Cimmino, Marco A AU - Cimmino MA AD - Clinica Reumatologica, Dipartimento di Medicina Interna, Università di Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. cimmino@unige.it FAU - Grassi, Walter AU - Grassi W FAU - Cutolo, Maurizio AU - Cutolo M LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Diagnostic Imaging/instrumentation/*methods/trends MH - Humans MH - Joints/*pathology MH - Radiography MH - Rheumatic Diseases/*diagnosis/diagnostic imaging MH - Rheumatology/methods MH - Ultrasonography RF - 49 EDAT- 2008/12/02 09:00 MHDA- 2009/02/13 09:00 CRDT- 2008/12/02 09:00 PHST- 2008/12/02 09:00 [pubmed] PHST- 2009/02/13 09:00 [medline] PHST- 2008/12/02 09:00 [entrez] AID - S1521-6942(08)00095-8 [pii] AID - 10.1016/j.berh.2008.08.007 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2008 Dec;22(6):951-9. doi: 10.1016/j.berh.2008.08.007. PMID- 26932287 OWN - NLM STAT- MEDLINE DCOM- 20170215 LR - 20220330 IS - 1573-3971 (Print) IS - 1573-3971 (Linking) VI - 9 IP - 4 DP - 2013 TI - Novel Aspects in the Pathophysiology of Peripheral Vasculopathy in Systemic Sclerosis. PG - 237-44 AB - Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis, autoimmunity and vascular damage. Although fibrosis is often considered the main feature of the disease, there is evidence that the underlying vasculopathy plays an important role in the initiation and perpetuation of SSc. Vascular manifestations such as Raynaud's phenomenon and digital ulcers are prominent in early disease stages and might substantially contribute to the SSc related mortality in later disease stages when pulmonary arterial hypertension becomes clinically evident. Vascular damage is thought to start with endothelial cell injury and apoptosis resulting in tissue hypoxia. Hypoxia is considered a main stimulus for vascular regenerative processes. However, despite the significant deterioration in number and quality of microvessels, there is a lack of appropriate compensatory repair processes by angiogenesis and vasculogenesis. In this review, we will discuss recent data about the pathophysiology of peripheral (acral) microvascular damage in SSc, highlight novel aspects behind the defective repair mechanisms in the vascular system in SSc and focus on SSc animal models with peripheral vascular changes. FAU - Suliman, Yossra A AU - Suliman YA FAU - Distler, Oliver AU - Distler O AD - Division of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Gloriastrasse 25, 8091 Zurich, Switzerland. oliver.distler@usz.ch. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Animals MH - Humans MH - Peripheral Vascular Diseases/*physiopathology MH - Scleroderma, Systemic/*physiopathology EDAT- 2013/01/01 00:00 MHDA- 2017/02/16 06:00 CRDT- 2016/03/03 06:00 PHST- 2016/03/03 06:00 [entrez] PHST- 2013/01/01 00:00 [pubmed] PHST- 2017/02/16 06:00 [medline] AID - CRR-EPUB-60154 [pii] AID - 10.2174/157339710904140417123932 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2013;9(4):237-44. doi: 10.2174/157339710904140417123932. PMID- 20924723 OWN - NLM STAT- MEDLINE DCOM- 20110217 LR - 20220801 IS - 0973-7693 (Electronic) IS - 0019-5456 (Linking) VI - 77 IP - 10 DP - 2010 Oct TI - Approach to a patient with connective tissue disease. PG - 1157-64 LID - 10.1007/s12098-010-0207-x [doi] AB - Connective tissue disease (CTDs), though rare in childhood, are an important cause of morbidity. Most of them involve multiple organ systems and are associated with presence of autoantibodies. Systemic lupus eryethematosus (SLE) is the most common CTD, the others being Juvenile dermatomyositis, systemic sclerosis, mixed connective disease and Sjogren syndrome. The clinical presentation of CTD in childhood can range from an acute severe illness mimicking a serious infection, to an insidious onset of disease with gradual accumulation of symptoms and signs over wks to months. The presence of multi-system involvement, evidence of inflammation and lack of any obvious cause should alert a clinician to the possibility of CTD. Diagnosis is usually clinical and features like malar rash, Raynaud's phenomenon, Gottron's rash, photosensitivity, oral ulcers suggest a possibility of CTD. Presence of autoantibodies like anti-nuclear antibodies, anti-dsDNA etc. provide supportive evidence to a diagnosis of CTD. Most CTDs are treated with immunosuppressive drugs with good success. Early recognition and prompt treatment results in excellent outcome. FAU - Kumar, T Sathish AU - Kumar TS AD - Department of Child Health, Christian Medical College, Vellore, India. FAU - Aggarwal, Amita AU - Aggarwal A LA - eng PT - Journal Article PT - Review DEP - 20101006 PL - India TA - Indian J Pediatr JT - Indian journal of pediatrics JID - 0417442 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/analysis MH - Child MH - Connective Tissue Diseases/*diagnosis MH - Dermatomyositis/diagnosis MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis MH - Scleroderma, Localized/diagnosis MH - Scleroderma, Systemic/diagnosis MH - Sjogren's Syndrome/diagnosis EDAT- 2010/10/07 06:00 MHDA- 2011/02/18 06:00 CRDT- 2010/10/07 06:00 PHST- 2010/06/25 00:00 [received] PHST- 2010/07/06 00:00 [accepted] PHST- 2010/10/07 06:00 [entrez] PHST- 2010/10/07 06:00 [pubmed] PHST- 2011/02/18 06:00 [medline] AID - 10.1007/s12098-010-0207-x [doi] PST - ppublish SO - Indian J Pediatr. 2010 Oct;77(10):1157-64. doi: 10.1007/s12098-010-0207-x. Epub 2010 Oct 6. PMID- 36601602 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230111 IS - 2156-7514 (Print) IS - 2156-5597 (Electronic) IS - 2156-5597 (Linking) VI - 12 DP - 2022 TI - Phalangeal microgeodic syndrome, COVID-19, and antinuclear antibodies in a child: A case report. PG - 64 LID - 10.25259/JCIS_122_2022 [doi] LID - 64 AB - Phalangeal microgeodic syndrome (PMS) is a rare rheumatological disease affecting children strongly associated with cold weather. It is considered to be benign and self-limiting, with most cases resolving in warmer months, and no studies have investigated its association to autoantibodies or viral infection. In this peculiar case, a 12-year-old child with the previous COVID-19 infection developed PMS that did not improve in the warm weather and, subsequently, Raynaud's phenomenon and facial rash. With the increasing number of new studies showing the correlation between COVID-19 and autoimmunity, this case report highlights a plausible link between COVID-19, PMS, and autoimmunity in the pediatric population that should be investigated further. CI - © 2022 Published by Scientific Scholar on behalf of Journal of Clinical Imaging Science. FAU - Hanife, Nura AU - Hanife N AD - Department of Radiology, Watford General Hospital, Watford, Hertfordshire, United Kingdom. FAU - Bessame, Khaoula AU - Bessame K AD - Department of Radiology, Watford General Hospital, Watford, Hertfordshire, United Kingdom. FAU - Mandalia, Uday Yashwant AU - Mandalia UY AD - Department of Radiology, Watford General Hospital, Watford, Hertfordshire, United Kingdom. LA - eng PT - Case Reports PT - Journal Article DEP - 20221205 PL - United States TA - J Clin Imaging Sci JT - Journal of clinical imaging science JID - 101564708 PMC - PMC9805605 OTO - NOTNLM OT - Antinuclear antibodies OT - COVID-19 OT - Phalangeal microgeodic syndrome COIS- There are no conflicts of interest. EDAT- 2023/01/06 06:00 MHDA- 2023/01/06 06:01 PMCR- 2022/12/05 CRDT- 2023/01/05 02:36 PHST- 2022/10/11 00:00 [received] PHST- 2022/11/20 00:00 [accepted] PHST- 2023/01/05 02:36 [entrez] PHST- 2023/01/06 06:00 [pubmed] PHST- 2023/01/06 06:01 [medline] PHST- 2022/12/05 00:00 [pmc-release] AID - 10.25259/JCIS_122_2022 [pii] AID - 10.25259/JCIS_122_2022 [doi] PST - epublish SO - J Clin Imaging Sci. 2022 Dec 5;12:64. doi: 10.25259/JCIS_122_2022. eCollection 2022. PMID- 20814538 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1687-9279 (Electronic) IS - 1687-9260 (Print) IS - 1687-9260 (Linking) VI - 2010 DP - 2010 TI - Vascular Complications of Systemic Sclerosis during Pregnancy. LID - 287248 [pii] LID - 10.1155/2010/287248 [doi] AB - Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis of the skin and visceral tissues as well as a noninflammatory vasculopathy. Vascular disease in systemic sclerosis is a major cause of morbidity and mortality among nonpregnant patients with SSc and is even a bigger concern in the pregnant SSc patient, as the underlying vasculopathy may prevent the required hemodynamic changes necessary to support a growing pregnancy. Vascular manifestations including scleroderma renal crisis and pulmonary arterial hypertension should be considered relative contraindications against pregnancy due to the high associations of both maternal and fetal morbidity and mortality. In contrast, Raynaud's phenomenon may actually improve somewhat during pregnancy. Women with SSc who are considering a pregnancy or discover they are pregnant require evaluation for the presence and extent of underlying vasculopathy. In the absence of significant visceral vasculopathy, most women with SSc can expect to have reasonable pregnancy outcomes. FAU - Chakravarty, Eliza F AU - Chakravarty EF AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, Suite 203, Palo Alto, CA 94304, USA. LA - eng PT - Journal Article DEP - 20100811 PL - United States TA - Int J Rheumatol JT - International journal of rheumatology JID - 101519204 PMC - PMC2931377 EDAT- 2010/09/04 06:00 MHDA- 2010/09/04 06:01 PMCR- 2010/08/11 CRDT- 2010/09/04 06:00 PHST- 2010/04/15 00:00 [received] PHST- 2010/07/02 00:00 [accepted] PHST- 2010/09/04 06:00 [entrez] PHST- 2010/09/04 06:00 [pubmed] PHST- 2010/09/04 06:01 [medline] PHST- 2010/08/11 00:00 [pmc-release] AID - 287248 [pii] AID - 10.1155/2010/287248 [doi] PST - ppublish SO - Int J Rheumatol. 2010;2010:287248. doi: 10.1155/2010/287248. Epub 2010 Aug 11. PMID- 28864092 OWN - NLM STAT- MEDLINE DCOM- 20180727 LR - 20220408 IS - 1578-8989 (Electronic) IS - 0025-7753 (Linking) VI - 150 IP - 1 DP - 2018 Jan 12 TI - Facts and controversies in mixed connective tissue disease. PG - 26-32 LID - S0025-7753(17)30588-2 [pii] LID - 10.1016/j.medcli.2017.06.066 [doi] AB - Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity. Others, however, suggest that these patients may belong to subgroups or early stages of certain definite connective diseases, such as SLE or SSc, or are, in fact, SARD overlap syndromes. CI - Copyright © 2017 Elsevier España, S.L.U. All rights reserved. FAU - Martínez-Barrio, Julia AU - Martínez-Barrio J AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España; Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, España; Universidad Complutense de Madrid, Madrid, España. Electronic address: juliamartinezbarrio@gmail.com. FAU - Valor, Lara AU - Valor L AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España; Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, España. FAU - López-Longo, F Javier AU - López-Longo FJ AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España; Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, España; Universidad Complutense de Madrid, Madrid, España. LA - eng LA - spa PT - Journal Article PT - Review TT - Hechos y controversias en la enfermedad mixta del tejido conectivo. DEP - 20170831 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM CIN - Med Clin (Barc). 2018 Dec 14;151(11):e63. doi: 10.1016/j.medcli.2018.02.026. PMID: 29685309 MH - Diagnosis, Differential MH - Dissent and Disputes MH - Humans MH - Mixed Connective Tissue Disease/*diagnosis/mortality/therapy OTO - NOTNLM OT - Anti-U1-RNP OT - Enfermedad mixta del tejido conectivo OT - Mixed connective tissue disease OT - Seronegative OT - Seronegativo EDAT- 2017/09/03 06:00 MHDA- 2018/07/28 06:00 CRDT- 2017/09/03 06:00 PHST- 2017/05/17 00:00 [received] PHST- 2017/06/25 00:00 [revised] PHST- 2017/06/26 00:00 [accepted] PHST- 2017/09/03 06:00 [pubmed] PHST- 2018/07/28 06:00 [medline] PHST- 2017/09/03 06:00 [entrez] AID - S0025-7753(17)30588-2 [pii] AID - 10.1016/j.medcli.2017.06.066 [doi] PST - ppublish SO - Med Clin (Barc). 2018 Jan 12;150(1):26-32. doi: 10.1016/j.medcli.2017.06.066. Epub 2017 Aug 31. PMID- 21888685 OWN - NLM STAT- MEDLINE DCOM- 20120329 LR - 20211020 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 13 IP - 4 DP - 2011 Aug 26 TI - Cardiovascular disease in systemic sclerosis--an emerging association? PG - 237 LID - 10.1186/ar3445 [doi] AB - Microvascular disease is a prominent feature of systemic sclerosis (SSc) and leads to Raynaud's phenomenon, pulmonary arterial hypertension, and scleroderma renal crisis. The presence of macrovascular disease is less well established, and, in particular, it is not known whether the prevalence of coronary heart disease in SSc is increased. Furthermore, in terms of cardiac involvement in SSc, there remains conjecture about the relative contributions of atherosclerotic macrovascular disease and myocardial microvascular disease. In this review, we summarize the literature describing cardiovascular disease in SSc, discuss the pathophysiological mechanisms common to SSc and atherosclerosis, and review the surrogate markers of cardiovascular disease which have been examined in SSc. Proposed mediators of the vasculopathy of SSc which have also been implicated in atherosclerosis include endothelial dysfunction, a reduced number of circulating endothelial progenitor cells, and an increased number of microparticles. Excess cardiovascular risk in SSc is suggested by increased arterial stiffness and carotid intima thickening and reduced flow-mediated dilatation. Cohort studies of adequate size are required to resolve whether this translates into an increased incidence of cardiovascular events in patients with SSc. FAU - Ngian, Gene-Siew AU - Ngian GS AD - The University of Melbourne, Department of Medicine (Royal Melbourne Hospital/Western Hospital), 4th Floor, Clinical Sciences Building, Royal Melbourne Hospital, Royal Parade, Parkville, Victoria 3050, Australia. gngian@student.unimelb.edu.au FAU - Sahhar, Joanne AU - Sahhar J FAU - Wicks, Ian P AU - Wicks IP FAU - Van Doornum, Sharon AU - Van Doornum S LA - eng PT - Journal Article PT - Review DEP - 20110826 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Cardiovascular Diseases/*complications/pathology MH - Humans MH - Scleroderma, Systemic/*complications/pathology PMC - PMC3239376 EDAT- 2011/09/06 06:00 MHDA- 2012/03/30 06:00 PMCR- 2012/02/26 CRDT- 2011/09/06 06:00 PHST- 2011/09/06 06:00 [entrez] PHST- 2011/09/06 06:00 [pubmed] PHST- 2012/03/30 06:00 [medline] PHST- 2012/02/26 00:00 [pmc-release] AID - ar3445 [pii] AID - 10.1186/ar3445 [doi] PST - epublish SO - Arthritis Res Ther. 2011 Aug 26;13(4):237. doi: 10.1186/ar3445. PMID- 24811467 OWN - NLM STAT- MEDLINE DCOM- 20150807 LR - 20140509 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 39 IP - 1 DP - 2014 Jan-Mar TI - Paraneoplastic sclerodermiform syndrome--case report. PG - 87-90 AB - Occasionally, auto-immune diseases may emerge as paraneoplastic syndromes. This is especially recognized in the case of polymyositis/dermatomyostis, but it is an extremely rare event in systemic sclerosis (SSc). The authors report the case of a sixty-year-old woman who presented with Raynaud's phenomenon and rapidly progressing skin thickness of the forearms, hands and lower limbs. Patient evaluation revealed a colorectal carcinoma. The patient was referred to the oncology department. This concomitance of cancer and SSc with rapid progression of the latter, suggests that the scleroderma might have a paraneoplastic origin. Such an hypothesis deserves consideration in every case as early diagnosis may be decisive to control the progression of either disease. FAU - Rovisco, João AU - Rovisco J FAU - Serra, Sara AU - Serra S FAU - Abreu, Pedro AU - Abreu P FAU - Coutinho, Margarida AU - Coutinho M FAU - Santiago, Tânia AU - Santiago T FAU - Inês, Luís AU - Inês L FAU - da Silva, José António Pereira AU - da Silva JA LA - eng PT - Case Reports PT - Journal Article PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adenocarcinoma/*complications MH - Colorectal Neoplasms/*complications MH - Female MH - Humans MH - Middle Aged MH - Paraneoplastic Syndromes/*etiology MH - Scleroderma, Systemic/*etiology EDAT- 2014/05/09 06:00 MHDA- 2015/08/08 06:00 CRDT- 2014/05/10 06:00 PHST- 2014/05/10 06:00 [entrez] PHST- 2014/05/09 06:00 [pubmed] PHST- 2015/08/08 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2014 Jan-Mar;39(1):87-90. PMID- 42256633 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260608 LR - 20260608 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 11 IP - 1 DP - 2026 TI - Influence of comorbidities and patient demographics on Raynaud's symptom characteristics: implications for diagnosis and management. PG - e000008 LID - 10.1136/jsrd-2026-000008 [doi] LID - e000008 AB - OBJECTIVES: Associations between Raynaud's phenomenon (RP) and body mass index (BMI), fibromyalgia syndrome (FMS) and migraine have been reported but their influence on the lived experience of RP is unknown. DESIGN: Cross-sectional electronic survey. SETTING: A social media-based awareness campaign organised by Scleroderma & Raynaud's UK. PARTICIPANTS: 4141 respondents with RP, including 3279 with primary RP (PRP), 476 with systemic sclerosis-related RP (SSc-RP) and 386 with other systemic autoimmune rheumatic disease-related RP (SARD-RP). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: RP symptom characteristics (colour change patterns, pain, numbness, tingling and thumb involvement) and their associations with BMI, FMS and migraine. RESULTS: In PRP, low BMI correlated with higher prevalence of cyanosis (55.1% vs 41.2%), hyperaemia (48.2% vs 41.3%), triphasic change (30.4% vs 23.0%) and thumb involvement (36.0% vs 27.0%) compared with high BMI. In PRP, concomitant FMS was associated with higher prevalence of pain (77.7% vs 57.4%), cyanosis (53.8% vs 45.4%), tingling (72.6% vs 62.8%) and thumb involvement (46.2% vs 27.2%) compared with those without FMS. Higher prevalence of pain was reported by FMS respondents with SSc-RP (82.8% vs 69.9%) and SARD-RP (84.1% and 70.9%, respectively). In PRP, migraine was associated with higher prevalence of cyanosis (50.0% vs 44.9%), hyperaemia (50.3% vs 43.7%), pain (69.0% vs 56.2%) and thumb involvement (33.4% vs 27.1%). Migraine was associated with higher prevalence of hyperaemia and pain in SARD-RP. CONCLUSION: BMI, FMS and migraine influence the lived experience of RP, particularly in PRP. FMS is associated with a greater pain burden, whereas low BMI and migraine are associated with prominent vasospastic features. These aetiopathogenic drivers influence RP symptomatology, with implications for management. CI - Copyright © Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Seomore, Reshma AU - Seomore R AUID- ORCID: 0009-0004-8494-3475 AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Wells, Matthew AU - Wells M AUID- ORCID: 0000-0002-8683-3358 AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. AD - Department of Clinical Specialties & Related Sciences, University of Bristol, Bristol, UK. FAU - Tymoszuk, Ula AU - Tymoszuk U AD - Scleroderma and Raynaud's UK, London, UK. FAU - Ahmed-Richards, Mithi AU - Ahmed-Richards M AD - Scleroderma and Raynaud's UK, London, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, University College London, London, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK and National Institute for Health and Care Research (NIHR), Manchester, UK. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK and National Institute for Health and Care Research (NIHR), Manchester, UK. AD - Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK. FAU - Jeffries-Owen, Nick AU - Jeffries-Owen N AD - Scleroderma and Raynaud's UK, London, UK. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. AD - Musculoskeletal Research Unit, University of Bristol, Bristol, UK. LA - eng PT - Journal Article DEP - 20260526 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC13231726 OTO - NOTNLM OT - Body Mass Index OT - Classification OT - Diagnosis COIS- JP has undertaken consultancy work, received educational support and/or speaker honoraria from Johnson & Johnson, AstraZeneca, Boehringer Ingelheim, CSL Vifor, IsoMab, Permeatus and Sojournix Pharma. MH has received conference support from UCB and consultancy fees from Boehringer Ingelheim and Novartis (none are relevant to this manuscript). EDAT- 2026/06/08 12:38 MHDA- 2026/06/08 12:39 PMCR- 2026/05/26 CRDT- 2026/06/08 07:42 PHST- 2026/04/09 00:00 [received] PHST- 2026/05/06 00:00 [accepted] PHST- 2026/06/08 12:39 [medline] PHST- 2026/06/08 12:38 [pubmed] PHST- 2026/06/08 07:42 [entrez] PHST- 2026/05/26 00:00 [pmc-release] AID - jsrd-2026-000008 [pii] AID - 10.1136/jsrd-2026-000008 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2026 May 26;11(1):e000008. doi: 10.1136/jsrd-2026-000008. eCollection 2026. PMID- 32226169 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200403 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 57 IP - 6 DP - 2019 TI - Hand-arm vibration syndrome. PG - 347-349 LID - 10.5114/reum.2019.90364 [doi] AB - Use of vibrating tools often leads to development of hand-arm vibration syndrome. It manifests with vascular symptoms, neurologic (carpal tunnel syndrome) and musculoskeletal symptoms (impaired grip strength, osteoarthritis, bone necrosis). Kienböck's disease is osteonecrosis of the lunate. A 61-year-old construction worker was referred to a rheumatologist because of suspected arthritis. On examination tenderness and swelling of the dorsal aspect of the right wrist were recorded without features of inflammation. The patient reported paresthesia in the right hand when working with a pneumatic drill. He reported no morning stiffness or Raynaud's phenomenon. He had undergone surgery because of right carpal tunnel syndrome two years earlier. Rheumatoid factor was negative, CRP 0.2 mg/l, uric acid 4.7 mg/dl. In magnetic resonance avascular necrosis of the lunate was diagnosed and scaphoid fracture. Kienböck's disease was diagnosed. Non-steroidal anti-inflammatory drugs were used. The patient did not give consent for surgery. CI - Copyright: © 2019 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. FAU - Nieradko-Iwanicka, Barbara AU - Nieradko-Iwanicka B AD - Chair and Department of Hygiene, Medical University of Lublin, Poland. LA - eng PT - Case Reports PT - Journal Article DEP - 20191231 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC7091485 OTO - NOTNLM OT - Kienböck’s disease OT - carpal tunnel syndrome OT - hand-arm vibration syndrome COIS- The author declares no conflict of interest. EDAT- 2019/01/01 00:00 MHDA- 2019/01/01 00:01 PMCR- 2019/01/01 CRDT- 2020/04/01 06:00 PHST- 2019/09/01 00:00 [received] PHST- 2019/11/18 00:00 [accepted] PHST- 2020/04/01 06:00 [entrez] PHST- 2019/01/01 00:00 [pubmed] PHST- 2019/01/01 00:01 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - 90364 [pii] AID - 10.5114/reum.2019.90364 [doi] PST - ppublish SO - Reumatologia. 2019;57(6):347-349. doi: 10.5114/reum.2019.90364. Epub 2019 Dec 31. PMID- 37746430 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230926 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 8 DP - 2023 Aug TI - Antisynthetase Syndrome With Predominant Pulmonary Involvement: A Case Report. PG - e43966 LID - 10.7759/cureus.43966 [doi] LID - e43966 AB - Antisynthetase syndrome (ASyS) is an autoimmune disease characterized by the presence of aminoacyl-transfer RNA synthetase antibodies. Its clinical presentation is variable and may include interstitial lung disease (ILD), myositis, arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." ILD is more prevalent in this entity when compared to other idiopathic inflammatory myopathies and imparts greater severity to the condition. Here, we report the case of a 42-year-old female patient who sought care for severe ILD and persistent fever. Her diagnosis was made only after the detection of anti-Jo1 autoantibodies. Treatment was refractory to both prednisone monotherapy and cyclophosphamide pulse therapy, requiring the introduction of rituximab. A high degree of clinical suspicion is required to allow early diagnosis of ASyS in patients with pulmonary involvement in the absence of accompanying muscle weakness or other clinical symptoms. CI - Copyright © 2023, Lima Corrêa de Araújo et al. FAU - Lima Corrêa de Araújo, Beatriz AU - Lima Corrêa de Araújo B AD - Department of Internal Medicine, Hospital Barão de Lucena, Recife, BRA. FAU - Victor, David R AU - Victor DR AD - Medical School, Universidade de Pernambuco, Recife, BRA. FAU - Farias Fontes, Heloísa Maria AU - Farias Fontes HM AD - Medical School, Universidade de Pernambuco, Recife, BRA. FAU - Caminha Mendes Gomes, Rayana Maria AU - Caminha Mendes Gomes RM AD - Medical School, Universidade de Pernambuco, Recife, BRA. FAU - Lima Corrêa de Araújo, Leonardo AU - Lima Corrêa de Araújo L AD - Medical School, Universidade Federal de Pernambuco, Recife, BRA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230823 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10515292 OTO - NOTNLM OT - anti-jo1 OT - antisynthetase syndrome OT - interstitial lung disease OT - myositis OT - rituximab COIS- The authors have declared that no competing interests exist. EDAT- 2023/09/25 06:42 MHDA- 2023/09/25 06:43 PMCR- 2023/08/23 CRDT- 2023/09/25 05:16 PHST- 2023/08/21 00:00 [accepted] PHST- 2023/09/25 06:43 [medline] PHST- 2023/09/25 06:42 [pubmed] PHST- 2023/09/25 05:16 [entrez] PHST- 2023/08/23 00:00 [pmc-release] AID - 10.7759/cureus.43966 [doi] PST - epublish SO - Cureus. 2023 Aug 23;15(8):e43966. doi: 10.7759/cureus.43966. eCollection 2023 Aug. PMID- 27068736 OWN - NLM STAT- MEDLINE DCOM- 20170220 LR - 20220409 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 35 IP - 9 DP - 2016 Sep TI - Heterogeneous clinical spectrum of interstitial lung disease in patients with anti-EJ anti-synthetase syndrome: a case series. PG - 2363-7 LID - 10.1007/s10067-016-3258-1 [doi] AB - Auto-antibodies against aminoacyl-tRNA-synthetases (anti-ARS Abs) represent the hallmark of the anti-synthetase syndrome that is defined as the clinical association of fever, Raynaud's phenomenon, myositis, interstitial lung disease (ILD), arthritis and mechanic's hands. Recently, differences in clinical features depending on specific anti-ARS Abs have been reported. We describe three cases of anti-EJ (anti-glycyl) antibody-positive patients presenting with ILD as a common feature, but with heterogeneous histopathological and radiographic patterns and with different responses to treatment. Relapsing-remittent fever, refractory muscle involvement and seronegative arthritis were also striking clinical manifestations. FAU - Giannini, Margherita AU - Giannini M AD - Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy. FAU - Notarnicola, Antonella AU - Notarnicola A AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Dastmalchi, Maryam AU - Dastmalchi M AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Lundberg, Ingrid E AU - Lundberg IE AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. FAU - Lopalco, Giuseppe AU - Lopalco G AD - Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Rheumatology Unit-DIM, University of Bari, P.zza G Cesare 11, 70124, Bari, Italy. florenzo.iannone@uniba.it. LA - eng PT - Case Reports PT - Journal Article DEP - 20160411 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/*immunology MH - Female MH - Humans MH - Lung Diseases, Interstitial/complications/*immunology MH - Middle Aged MH - Myositis/complications/*immunology MH - Young Adult OTO - NOTNLM OT - Anti-EJ OT - Anti-synthetase syndrome OT - Interstitial lung disease EDAT- 2016/04/14 06:00 MHDA- 2017/10/14 06:00 CRDT- 2016/04/13 06:00 PHST- 2015/12/10 00:00 [received] PHST- 2016/04/04 00:00 [accepted] PHST- 2016/04/03 00:00 [revised] PHST- 2016/04/13 06:00 [entrez] PHST- 2016/04/14 06:00 [pubmed] PHST- 2017/10/14 06:00 [medline] AID - 10.1007/s10067-016-3258-1 [pii] AID - 10.1007/s10067-016-3258-1 [doi] PST - ppublish SO - Clin Rheumatol. 2016 Sep;35(9):2363-7. doi: 10.1007/s10067-016-3258-1. Epub 2016 Apr 11. PMID- 24480058 OWN - NLM STAT- MEDLINE DCOM- 20150515 LR - 20140402 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 41 IP - 4 DP - 2014 Apr TI - Case of lepromatous leprosy misdiagnosed as systemic sclerosis. PG - 343-5 LID - 10.1111/1346-8138.12406 [doi] AB - Hansen's disease (HD) is a chronic granulomatous infectious disease caused by Mycobacterium leprae. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of HD are extensive, with involvement of the skin and various organs, and these can resemble those of many rheumatic diseases. Our patient initially presented with gradual sclerotic skin change and slight sclerodactyly with Raynaud's phenomenon, which is frequently observed in systemic sclerosis. However, a skin biopsy with acid-fast stain later confirmed lepromatous leprosy. We report this case to emphasize the role of dermatologists for applying a systematic approach to the skin lesions of HD, which has become difficult to detect because of its rapidly declining prevalence rate. CI - © 2014 Japanese Dermatological Association. FAU - Lee, Jin Yong AU - Lee JY AD - Department of Dermatology, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea. FAU - Park, So Eun AU - Park SE FAU - Shin, Soo Jung AU - Shin SJ FAU - Kim, Chul Woo AU - Kim CW FAU - Kim, Sang Seok AU - Kim SS LA - eng PT - Case Reports PT - Journal Article DEP - 20140131 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - Aged, 80 and over MH - Diagnostic Errors MH - Female MH - Humans MH - Leprosy, Lepromatous/*diagnosis/pathology/physiopathology MH - Neural Conduction MH - Polyneuropathies/physiopathology MH - Scleroderma, Systemic/*diagnosis MH - Skin/pathology OTO - NOTNLM OT - Hansen's disease OT - lepromatous leprosy OT - sclerodactyly OT - scleroderma OT - systemic sclerosis EDAT- 2014/02/01 06:00 MHDA- 2015/05/16 06:00 CRDT- 2014/02/01 06:00 PHST- 2013/03/04 00:00 [received] PHST- 2013/12/20 00:00 [accepted] PHST- 2014/02/01 06:00 [entrez] PHST- 2014/02/01 06:00 [pubmed] PHST- 2015/05/16 06:00 [medline] AID - 10.1111/1346-8138.12406 [doi] PST - ppublish SO - J Dermatol. 2014 Apr;41(4):343-5. doi: 10.1111/1346-8138.12406. Epub 2014 Jan 31. PMID- 20137654 OWN - NLM STAT- MEDLINE DCOM- 20100518 LR - 20100208 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 89 IP - 41 DP - 2009 Nov 10 TI - [Clinical analysis of 79 pulmonary arterial hypertension cases from 1892 connective tissue disease patients]. PG - 2934-7 AB - OBJECTIVE: To understand the prevalence, investigate the correlation of clinical features, explore the early-stage diagnosis and treatment of pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD). METHODS: All cases with pulmonary arterial hypertension in 1892 CTD patients were analyzed retrospectively. The risk factor of PAH was evaluated and the prognostic influence of different treatments and primary diseases analyzed. RESULTS: The prevalence of PAH in patients with connective tissue disease was about 4.2%(79/1892). In these patients, systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) had the highest incidence of PAH (18.18% and 12.00%) (P < 0.01). It was obviously higher than polymyositis/dermatomyositis (6.2%), systemic lupus erythematosus (4.4%), Sjogren syndrome (3.8%), rheumatoid arthritis (0.8%) and anti-phospholipid syndrome (0.5%), etc. (P < 0.01). Raynaud's phenomenon was related to a higher pulmonary arterial pressure (P < 0.01). There was a positive correlation (P < 0.01) between the presence of Raynaud's phenomenon and pulmonary arterial pressure. Abnormal lung function was a common finding. There were associations (P < 0.05) between the degree of pulmonary hypertension and IgG, anti-U1RNP antibody positive, antiphospholipid antibody positive, pericardial effusion and interstitial pneumonia. CONCLUSION: PAH is common in connective tissue disease. SSc and MCTD have the highest prevalence of PAH. The presence of Raynaud's phenomenon anti-U1RNP antibody is positively correlated with pulmonary arterial pressure. It can predict the development of PAH. It is useful to perform ultraechocardiogrphy for an early-stage diagnosis and prognostic analysis. FAU - Lei, Yun-xia AU - Lei YX AD - Department of Rheumatology, Guangdong Provincial People's Hospital, Guangzhou 510080, China. FAU - Zhang, Xiao AU - Zhang X FAU - Cui, Yang AU - Cui Y FAU - Dong, Guang-fu AU - Dong GF FAU - Luo, Ri-qiang AU - Luo RQ LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Connective Tissue Diseases/*complications/*diagnosis MH - Female MH - Humans MH - Hypertension, Pulmonary/*diagnosis/*etiology MH - Male MH - Middle Aged MH - Prevalence MH - Retrospective Studies MH - Risk Factors MH - Young Adult EDAT- 2010/02/09 06:00 MHDA- 2010/05/19 06:00 CRDT- 2010/02/09 06:00 PHST- 2010/02/09 06:00 [entrez] PHST- 2010/02/09 06:00 [pubmed] PHST- 2010/05/19 06:00 [medline] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2009 Nov 10;89(41):2934-7. PMID- 17325434 OWN - NLM STAT- MEDLINE DCOM- 20070410 LR - 20131121 IS - 1386-0291 (Print) IS - 1386-0291 (Linking) VI - 36 IP - 2 DP - 2007 TI - Nifedipine effect on red cell rheological properties in patients with systemic scleroderma. PG - 105-10 AB - Systemic scleroderma is an autoimmune disease, due to a connective tissue alteration characterized by extracellular matrix increase in the skin and internal organs. It is already known that the Raynaud's phenomenon and the microcapillary obliteration lead to ischemia and peripheral tissue injury. The ischemia-reperfusion phenomenon releases free radicals, that react with red blood cells (RBCs) membrane components originating lipid peroxidation and impairment of the ATP-Ca(++) pump, two possible mechanisms responsible of disease pathogenesis. Nifedipine is a Ca(++)-channel antagonist that has been used for a long time in Raynaud's phenomenon treatment. In the present study we were able to demonstrate that erythrocyte deformability and two other related variables such as membrane fluidity and osmotic fragility improve significantly with nifedipine therapy. It is likely that nifedipine inhibiting cytoplasmic calcium accumulation could restore some red blood cell membrane properties. FAU - Spengler, M I AU - Spengler MI AD - Departamento de Ciencias Fisiológicas, Cátedra de Física Biológica, Facultad de Ciencias Médicas, Universidad Nacional de Rosario, Santa Fe 3100, CP 2000, Rosario, Argentina. isabelspengler@hotmail.com FAU - Leroux, M B AU - Leroux MB FAU - Svetaz, M J AU - Svetaz MJ FAU - Contesti, J F AU - Contesti JF FAU - Parente, F M AU - Parente FM FAU - Bertoluzzo, S M AU - Bertoluzzo SM LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 0 (Calcium Channel Blockers) RN - I9ZF7L6G2L (Nifedipine) SB - IM MH - Adult MH - Blood Viscosity/*drug effects MH - Calcium Channel Blockers/*pharmacology MH - Erythrocyte Deformability/*drug effects MH - Female MH - Hemorheology/drug effects MH - Humans MH - Nifedipine/*pharmacology MH - Osmotic Fragility/*drug effects MH - Scleroderma, Systemic/*drug therapy EDAT- 2007/02/28 09:00 MHDA- 2007/04/11 09:00 CRDT- 2007/02/28 09:00 PHST- 2007/02/28 09:00 [pubmed] PHST- 2007/04/11 09:00 [medline] PHST- 2007/02/28 09:00 [entrez] PST - ppublish SO - Clin Hemorheol Microcirc. 2007;36(2):105-10. PMID- 34659821 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240901 IS - 2072-1439 (Print) IS - 2077-6624 (Electronic) IS - 2072-1439 (Linking) VI - 13 IP - 9 DP - 2021 Sep TI - A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach. PG - 5556-5571 LID - 10.21037/jtd-20-3328 [doi] AB - Anti-synthetase syndrome (AS) is a rare autoimmune disorder characterized by the presence of aminoacyl-transfer RNA synthetase antibodies in conjunction with clinical features such as interstitial lung disease (ILD), Raynaud's phenomenon, nonerosive arthritis, and myopathy. AS distinguishes itself from other inflammatory myopathies by its significant lung involvement and rapidly progressive interstitial lung disease (AS-ILD), therefore the management of AS-ILD requires careful clinical, serologic and radiologic assessment. Glucocorticoids are considered the mainstay of therapy; however, additional immunosuppressive agents are often required to achieve disease control. Patient prognosis is highly dependent on early diagnosis and symptom recognition as the antibody profile is thought to influence therapy response. Since progressive ILD is the leading cause of morbidity and mortality, this review will discuss the clinical approach to patient with suspected AS, with particular emphasis on diagnosis and management of AS-ILD. CI - 2021 Journal of Thoracic Disease. All rights reserved. FAU - Sawal, Naina AU - Sawal N AD - Department of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY, USA. FAU - Mukhopadhyay, Sanjay AU - Mukhopadhyay S AD - Department of Pathology, Cleveland Clinic, Cleveland, OH, USA. FAU - Rayancha, Sheetal AU - Rayancha S AD - Department of Rheumatology, SUNY Upstate Medical University, Syracuse, NY, USA. FAU - Moore, Alastair AU - Moore A AD - Department of Radiology, Baylor Scott and White Health, Dallas, TX, USA. FAU - Garcha, Puneet AU - Garcha P AD - Department of Pulmonary Critical-Care, Baylor College of Medicine, Houston, TX, USA. FAU - Kumar, Anupam AU - Kumar A AD - Department of Pulmonary Critical-Care, Baylor College of Medicine, Houston, TX, USA. FAU - Kaul, Viren AU - Kaul V AD - Department of Pulmonary Critical-Care, Crouse Health/SUNY Upstate Medical University, Syracuse, NY, USA. LA - eng PT - Journal Article PT - Review PL - China TA - J Thorac Dis JT - Journal of thoracic disease JID - 101533916 PMC - PMC8482343 OTO - NOTNLM OT - Antisynthetase syndrome OT - autoimmune disease OT - computed tomography OT - immunosuppressive agents OT - interstitial lung disease COIS- Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://dx.doi.org/10.21037/jtd-20-3328). The authors have no conflicts of interest to declare. EDAT- 2021/10/19 06:00 MHDA- 2021/10/19 06:01 PMCR- 2021/09/01 CRDT- 2021/10/18 09:01 PHST- 2020/11/18 00:00 [received] PHST- 2021/07/23 00:00 [accepted] PHST- 2021/10/18 09:01 [entrez] PHST- 2021/10/19 06:00 [pubmed] PHST- 2021/10/19 06:01 [medline] PHST- 2021/09/01 00:00 [pmc-release] AID - jtd-13-09-5556 [pii] AID - 10.21037/jtd-20-3328 [doi] PST - ppublish SO - J Thorac Dis. 2021 Sep;13(9):5556-5571. doi: 10.21037/jtd-20-3328. PMID- 29104809 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2017 DP - 2017 TI - Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma. PG - 4029271 LID - 10.1155/2017/4029271 [doi] LID - 4029271 AB - This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare. While she was receiving high-dose prednisolone, as worsening of her connective tissue disease was suspected to be the cause of the inflammatory changes, she had Listeria monocytogenes meningitis and was hospitalized for several weeks, but she recovered from this without sequelae. After Mycobacterium intracellulare infection was diagnosed, she was treated with clarithromycin and rifampicin. Her skin manifestations, arthralgias, and fatigue improved considerably, and the wounds of the right leg healed, unfortunately with significant scarring. Immunodeficiency testing was unremarkable. In summary, an infection with Mycobacterium intracellulare was mistaken for an unusually severe progression of scleroderma. FAU - Krabbe, Simon AU - Krabbe S AUID- ORCID: 0000-0002-2877-1582 AD - Center for Rheumatology and Spine Diseases, Rigshospitalet, 2600 Glostrup, Denmark. FAU - Engelhart, Merete AU - Engelhart M AD - Center for Rheumatology and Spine Diseases, Gentofte Hospital, 2900 Hellerup, Denmark. FAU - Thybo, Sören AU - Thybo S AD - Department of Infectious Diseases, Rigshospitalet, 2100 Copenhagen, Denmark. FAU - Jacobsen, Søren AU - Jacobsen S AD - Center for Rheumatology and Spine Diseases, Rigshospitalet, 2100 Copenhagen, Denmark. LA - eng PT - Case Reports PT - Journal Article DEP - 20170927 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC5635288 EDAT- 2017/11/07 06:00 MHDA- 2017/11/07 06:01 PMCR- 2017/09/27 CRDT- 2017/11/07 06:00 PHST- 2017/04/02 00:00 [received] PHST- 2017/09/09 00:00 [revised] PHST- 2017/09/12 00:00 [accepted] PHST- 2017/11/07 06:00 [entrez] PHST- 2017/11/07 06:00 [pubmed] PHST- 2017/11/07 06:01 [medline] PHST- 2017/09/27 00:00 [pmc-release] AID - 10.1155/2017/4029271 [doi] PST - ppublish SO - Case Rep Rheumatol. 2017;2017:4029271. doi: 10.1155/2017/4029271. Epub 2017 Sep 27. PMID- 19896327 OWN - NLM STAT- MEDLINE DCOM- 20100422 LR - 20161125 IS - 1615-5947 (Electronic) IS - 0890-5096 (Linking) VI - 24 IP - 2 DP - 2010 Feb TI - Brachial artery fibromuscular dysplasia. PG - 255.e1-4 LID - 10.1016/j.avsg.2009.05.012 [doi] AB - Fibromuscular dysplasia is a rare vascular disease that is characterized as nonatherosclerotic and noninflammatory in nature. This disease most commonly afflicts the renal and cerebrovascular beds but can rarely affect the upper extremity. We present the case of a 76-year-old woman who complained of a symptom complex, congruent with Raynaud's phenomenon on the right side. The patient had evidence of distal ischemia without the classic angiographic evidence of fibromuscular dysplasia on arteriography. The abnormal arterial section of the right brachial artery was resected and grafted with reversed saphenous vein. She has had no reoccurrence of her symptoms and no stenosis of her graft over a 3-year follow-up period. CI - Published by Elsevier Inc. FAU - Rice, Robert D AU - Rice RD AD - Department of Surgery, Vascular Surgery Service, Dwight David Eisenhower Army Medical Center, Fort Gordon, GA, USA. robert.d.rice@us.army.mil FAU - Armstrong, Peter J AU - Armstrong PJ LA - eng PT - Case Reports PT - Journal Article DEP - 20091105 PL - Netherlands TA - Ann Vasc Surg JT - Annals of vascular surgery JID - 8703941 SB - IM MH - Adult MH - Aged MH - Angioplasty, Balloon MH - Brachial Artery/diagnostic imaging/pathology/*surgery MH - Female MH - Fibromuscular Dysplasia/diagnostic imaging/pathology/*surgery MH - Humans MH - Male MH - Middle Aged MH - Radiography MH - Saphenous Vein/*transplantation MH - Treatment Outcome MH - *Vascular Surgical Procedures EDAT- 2009/11/10 06:00 MHDA- 2010/04/23 06:00 CRDT- 2009/11/10 06:00 PHST- 2009/03/12 00:00 [received] PHST- 2009/05/27 00:00 [accepted] PHST- 2009/11/10 06:00 [entrez] PHST- 2009/11/10 06:00 [pubmed] PHST- 2010/04/23 06:00 [medline] AID - S0890-5096(09)00170-8 [pii] AID - 10.1016/j.avsg.2009.05.012 [doi] PST - ppublish SO - Ann Vasc Surg. 2010 Feb;24(2):255.e1-4. doi: 10.1016/j.avsg.2009.05.012. Epub 2009 Nov 5. PMID- 38918941 OWN - NLM STAT- MEDLINE DCOM- 20240626 LR - 20240626 IS - 1581-2979 (Electronic) IS - 1318-4458 (Linking) VI - 33 IP - 2 DP - 2024 Jun TI - Dermatomyositis: nailfold capillaroscopy patterns and a general survey. PG - 69-79 AB - INTRODUCTION: Dermatomyositis (DM) is a group of autoimmune idiopathic inflammatory myopathies characterized by typical cutaneous signs and symptoms of muscle involvement. The diseases can be associated with cancer in the paraneoplastic syndrome, calcinosis, interstitial lung disease, other autoimmune connective tissue diseases (in overlap syndrome), and Raynaud's phenomenon. METHODS: Clinical and capillaroscopic data were gathered from 43 patients with DM. The diagnosis was based on the Bohan‒Peter and European League against Rheumatism / American College of Rheumatology (EULAR/ACR) classification criteria. In addition, nailfold capillaroscopy was performed in all patients. RESULTS: In our cohort, eight patients had overlap syndrome, six had paraneoplastic syndrome, eight presented with interstitial lung disease, and nine had calcinosis, two of whom also had a cancerous pathology. Raynaud's phenomenon was reported in 74% of patients. Upon nailfold capillaroscopy, 84% of patients presented giant capillaries, 81% ramified capillaries, and 70% both. The latter, notably giant ramified capillaries, could be considered specific for DM. The detection of prominent subpapillary venous plexuses was associated with pulmonary involvement. In contrast, alterations of the pericapillary spaces were associated with the severity and prognosis of DM. CONCLUSIONS: Our results underline the usefulness of nailfold capillaroscopy in the diagnosis and prognosis of DM. Based on the results and literature data, specific nailfold capillaroscopy features should be included in DM diagnostic criteria. FAU - Trevisan, Giusto AU - Trevisan G AD - Department of Medical Sciences, University of Trieste, Trieste, Italy. FAU - Bonin, Serena AU - Bonin S AD - Department of Medical Sciences, University of Trieste, Trieste, Italy. FAU - Tucci, Sandro AU - Tucci S AD - Jean Françoise Merlen Research Center of Vascular Diseases, Frosinone, Italy. FAU - Bilancini, Salvino AU - Bilancini S AD - Jean Françoise Merlen Research Center of Vascular Diseases, Frosinone, Italy. LA - eng PT - Journal Article PL - Slovenia TA - Acta Dermatovenerol Alp Pannonica Adriat JT - Acta dermatovenerologica Alpina, Pannonica, et Adriatica JID - 9422563 SB - IM MH - Humans MH - *Microscopic Angioscopy MH - *Dermatomyositis/pathology/diagnosis MH - Female MH - Male MH - Middle Aged MH - Aged MH - Adult EDAT- 2024/06/26 06:41 MHDA- 2024/06/26 06:42 CRDT- 2024/06/26 01:25 PHST- 2024/06/26 06:42 [medline] PHST- 2024/06/26 06:41 [pubmed] PHST- 2024/06/26 01:25 [entrez] AID - 680 [pii] PST - ppublish SO - Acta Dermatovenerol Alp Pannonica Adriat. 2024 Jun;33(2):69-79. PMID- 23279029 OWN - NLM STAT- MEDLINE DCOM- 20131126 LR - 20160511 IS - 1442-200X (Electronic) IS - 1328-8067 (Linking) VI - 54 IP - 6 DP - 2012 Dec TI - Child-onset systemic sclerosis positive for anticentromere antibodies: two Japanese cases. PG - 941-4 LID - 10.1111/j.1442-200X.2012.03604.x [doi] AB - Systemic sclerosis (SSc) is an uncommon connective tissue disease of childhood. Moreover, pediatric SSc positive for anticentromere antibodies (ACA) is extremely rare. We describe two cases of ACA-positive SSc in Japanese girls with clinical findings. Case 1 is a 15-year-old female. From disease onset at 7 years, she developed limited cutaneous (no internal involvement) SSc positive for ACA and anti-U1RNP antibodies, but negative for antitopoisomerase I antibodies (ATA). She also showed calcinosis, sclerodactyly, and telangiectasia, but not Raynaud's phenomenon. Case 2 is also a 15-year-old female with onset at the same age. Her diagnosis was limited cutaneous SSc positive for ACA, anti-SSA/Ro antibodies, and anti-thyroid antibodies, but not ATA. She showed sclerodactyly, telangiectasia, and Raynaud phenomenon, as well as complicated Sjögren's syndrome and chronic thyroiditis with euthyroidism. We reported that two Japanese girls with SSc were positive for ACA and other antibodies with the exception of ATA. CI - © 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society. FAU - Inamo, Yasuji AU - Inamo Y AD - Department of General Pediatrics, Nihon University of School Medicine, Nihon University Nerima-Hikarigaoka Hospital, Tokyo, Japan. y-inamo@pb3.so-net.ne.jp LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - Pediatr Int JT - Pediatrics international : official journal of the Japan Pediatric Society JID - 100886002 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Adolescent MH - Antibodies, Antinuclear/blood/*immunology MH - Female MH - Humans MH - Japan MH - Scleroderma, Systemic/blood/diagnosis/*immunology EDAT- 2013/01/03 06:00 MHDA- 2013/12/16 06:00 CRDT- 2013/01/03 06:00 PHST- 2013/01/03 06:00 [entrez] PHST- 2013/01/03 06:00 [pubmed] PHST- 2013/12/16 06:00 [medline] AID - 10.1111/j.1442-200X.2012.03604.x [doi] PST - ppublish SO - Pediatr Int. 2012 Dec;54(6):941-4. doi: 10.1111/j.1442-200X.2012.03604.x. PMID- 31498665 OWN - NLM STAT- MEDLINE DCOM- 20200213 LR - 20200213 IS - 1759-7390 (Electronic) IS - 1750-8460 (Linking) VI - 80 IP - 9 DP - 2019 Sep 2 TI - Systemic sclerosis. PG - 530-536 LID - 10.12968/hmed.2019.80.9.530 [doi] AB - Systemic sclerosis is a complex autoimmune connective tissue disease which carries a significant burden of disease-related morbidity including potentially life-threatening complications. Systemic sclerosis can affect all the major organs and therefore, although the disease is uncommon, many hospital-based specialists are involved in patient care. Vascular disease (e.g. Raynaud's phenomenon) is an almost universal symptom in patients with systemic sclerosis and is often the earliest manifestation of the disease. Systemic sclerosis not uncommonly can overlap with other rheumatological conditions (e.g. rheumatoid arthritis and myositis). During the past few decades there have been major advances in understanding the pathogenesis of systemic sclerosis and these are driving advances in treatment. There are now a number of effective treatments to manage many of the different organ-based complications. Autologous haemopoietic stem cell transplantation is a potential treatment option in highly selected patients. This review updates the clinician about epidemiology, pathogenesis, differential diagnosis, the wide clinical spectrum of disease, and current and emerging treatments for systemic sclerosis. FAU - Hughes, Michael AU - Hughes M AD - Consultant Rheumatologist, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF. FAU - Herrick, Ariane L AU - Herrick AL AD - Professor of Rheumatology, Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester and NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester. LA - eng PT - Journal Article PT - Review PL - Singapore TA - Br J Hosp Med (Lond) JT - British journal of hospital medicine (London, England : 2005) JID - 101257109 SB - IM MH - Humans MH - Scleroderma, Systemic/diagnosis/epidemiology/*physiopathology/therapy EDAT- 2019/09/10 06:00 MHDA- 2020/02/14 06:00 CRDT- 2019/09/10 06:00 PHST- 2019/09/10 06:00 [entrez] PHST- 2019/09/10 06:00 [pubmed] PHST- 2020/02/14 06:00 [medline] AID - 10.12968/hmed.2019.80.9.530 [doi] PST - ppublish SO - Br J Hosp Med (Lond). 2019 Sep 2;80(9):530-536. doi: 10.12968/hmed.2019.80.9.530. PMID- 34584436 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240403 IS - 1178-7015 (Print) IS - 1178-7015 (Electronic) IS - 1178-7015 (Linking) VI - 14 DP - 2021 TI - Utilities of Botulinum Toxins in Dermatology and Cosmetology. PG - 1319-1330 LID - 10.2147/CCID.S332247 [doi] AB - Botulinum toxin (BoNT) is a neurotoxin produced by the Clostridium botulinum bacterium with a well-known efficacy and safety profile in the focal idiopathic hyperhidrosis treatment. BoNT comprises seven different neurotoxins; however, only toxins A and B are clinically employed. BoNT is lately practiced in off-label therapies for a variety of skin diseases. Scar prevention, hyperhidrosis, rhytides, eccrine nevus, alopecia, psoriasis, Darier disease, bullous skin disease, pompholyx and Raynaud's phenomenon are some of the novel indications for BoNT in cosmetic and notably non-cosmetic aspects of dermatology. To employ BoNT correctly in clinical practice, we must have a thorough understanding of the functional anatomy of the mimetic muscles. An intensive literature search was conducted to update all dermatology-oriented experiments and clinical trials on the described element of BoNT for this general overview of BoNT use in dermatology. This review aims to analyse the role of BoNT in dermatology and cosmetology. CI - © 2021 Naik. FAU - Naik, Piyu Parth AU - Naik PP AUID- ORCID: 0000-0002-6499-4062 AD - Department of Dermatology, Saudi German Hospital and Clinic, Dubai, United Arab Emirates. LA - eng PT - Journal Article PT - Review DEP - 20210921 PL - New Zealand TA - Clin Cosmet Investig Dermatol JT - Clinical, cosmetic and investigational dermatology JID - 101543449 PMC - PMC8464334 OTO - NOTNLM OT - BoNT OT - Clostridium botulinum OT - botulinum toxin OT - cosmetology OT - dermatology OT - neurotoxin COIS- Dr. Piyu Parth Naik reports no conflicts of interest for this work. EDAT- 2021/09/30 06:00 MHDA- 2021/09/30 06:01 PMCR- 2021/09/21 CRDT- 2021/09/29 06:52 PHST- 2021/08/02 00:00 [received] PHST- 2021/09/04 00:00 [accepted] PHST- 2021/09/29 06:52 [entrez] PHST- 2021/09/30 06:00 [pubmed] PHST- 2021/09/30 06:01 [medline] PHST- 2021/09/21 00:00 [pmc-release] AID - 332247 [pii] AID - 10.2147/CCID.S332247 [doi] PST - epublish SO - Clin Cosmet Investig Dermatol. 2021 Sep 21;14:1319-1330. doi: 10.2147/CCID.S332247. eCollection 2021. PMID- 18622933 OWN - NLM STAT- MEDLINE DCOM- 20080923 LR - 20080714 IS - 0040-5930 (Print) IS - 0040-5930 (Linking) VI - 65 IP - 5 DP - 2008 May TI - [Systemic sclerosis]. PG - 283-7 LID - 10.1024/0040-5930.65.5.283 [doi] AB - Systemic sclerosis (scleroderma) is characterized by autoimmune phenomena and a progressive fibrosis. Clinical signs include Raynaud's phenomenon, pathognomonic nail fold capillary changes, pulmonal, renal, cardial and intestinal involvement. The two forms of systemic sclerosis, the diffuse and the limited show distinct disease phenomena and evolution. The diffuse form typically leads to a rapid sclerosis of the whole skin, it is associated with the auto-antibody Scl-70, it presents with an interstitial fibrosis of the lung, myositis and cardial fibrosis, a progressive loss of intestinal motility with maldigestion and it bears the risk of renal crisis. The limited form is characterized by sclerosis of the skin of the distal parts of the limbs and the face only, it is associated with the centromer auto-antibody and a much slower progression of visceral fibrosis. A typical late stage manifestation is pulmonary arterial hypertension. New drugs have improved quality of life. Their correct indication is based on a systematic monitoring of disease. FAU - Villiger, Peter M AU - Villiger PM AD - Klinik für Rheumatologie und Klinische Immunologie & Allergologie, Universitätsspital, Bern. peter.villiger@insel.ch LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Systemsklerose. PL - Switzerland TA - Ther Umsch JT - Therapeutische Umschau. Revue therapeutique JID - 0407224 RN - 0 (Immunosuppressive Agents) SB - IM MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Scleroderma, Systemic/*diagnosis/*drug therapy RF - 6 EDAT- 2008/07/16 09:00 MHDA- 2008/09/24 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2008/09/24 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] AID - 10.1024/0040-5930.65.5.283 [doi] PST - ppublish SO - Ther Umsch. 2008 May;65(5):283-7. doi: 10.1024/0040-5930.65.5.283. PMID- 30705970 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 3 IP - 3 DP - 2018 Oct TI - Patient-reported outcome instruments for assessing Raynaud's phenomenon in systemic sclerosis: A SCTC Vascular Working Group Report. PG - 249-252 LID - 10.1177/2397198318774307 [doi] AB - The episodic nature of Raynaud's phenomenon (RP) in systemic sclerosis (SSc) has led to a reliance on patient-reported outcome (PRO) instruments such as the Raynaud's Condition Score (RCS) diary. Little is known about the utilisation in routine clinical practice and health professional attitudes towards existing PRO instruments for assessing SSc-RP. Members of the Scleroderma Clinical Trials Consortium Vascular Working Group (SCTC-VWG, n=28) were invited to participate in a survey gauging attitudes towards the RCS diary and the perceived need for novel PRO instruments for assessing SSc-RP. Nineteen SCTC-VWG members (68% response rate) from academic units based in North America (n=9), Europe (n=8), South America (n=1) and Australasia (n=1) took part in the survey. There was broad consensus that RCS diary returns could be influenced by factors including seasonal variation in weather, efforts made by patients to avoid or ameliorate attacks of RP, habituation to RP symptoms, evolution of RP symptom characteristics with progressive obliterative microangiopathy, patient coping strategies, respondent burden and placebo effect. There was consensus that limitations of the RCS diary might be a barrier to drug development (79% of respondents agree/strongly agree) and that a novel PRO instrument for assessing SSc-RP should be developed with the input of both clinicians and patients (84% agree/strongly agree). Perceived potential limitations of the RCS diary have been identified along with concerns that such factors might impede drug development programs for SSc-RP. There is support within the systemic sclerosis community for the development of a novel PRO instrument for assessing SSc-RP. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Frech, Tracy M AU - Frech TM AD - University of Utah and Salt Lake Regional Veterans Affair Medical Center, Salt Lake City, UT. FAU - Hughes, Michael AU - Hughes M AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester. FAU - Gordon, Jessica K AU - Gordon JK AD - Hospital for Special Surgery, New York, NY. FAU - Domsic, Robyn T AU - Domsic RT AD - University of Pittsburgh Medical Center, Pittsburgh, PA. FAU - Anderson, Marina E AU - Anderson ME AD - Institute of Ageing and Chronic Disease, Faculty of Health and Life Sciences, University of Liverpool & Aintree University Hospital. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, Dept. of Clinical Sciences and Community Health, University of Milano, Milan, Italy. FAU - McHugh, Neil J AU - McHugh NJ AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Johnson, Sindhu R AU - Johnson SR AD - Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Canada. FAU - Hudson, Marie AU - Hudson M AD - Jewish General Hospital, Lady Davis Institute and McGill University, Montreal, Canada. FAU - Boin, Francesco AU - Boin F AD - UCSF Scleroderma Center, University of California San Francisco, San Francisco, CA. FAU - Ong, Voon H AU - Ong VH AD - University College London Medical School, Royal Free Hospital, London, UK. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, University of Florence, Florence, Italy. FAU - Altorok, Nezam AU - Altorok N AD - University of Toledo Medical Center, Toledo, OH. FAU - Scolnik, Marina AU - Scolnik M AD - Rheumatology Section, Hospital Italiano de Buenos Aires, Argentina. FAU - Nikpour, Mandana AU - Nikpour M AD - The University of Melbourne at St. Vincent's Hospital, Melbourne, Victoria, Australia. FAU - Shah, Ankoor AU - Shah A AD - Duke University Medical Center, Durham, NC. FAU - Pope, Janet E AU - Pope JE AD - Western University, London, Ontario, Canada. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan Scleroderma Program, Ann Arbor, MI. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester. AD - NIHR Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, UK. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20180524 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC6350902 MID - NIHMS982916 OTO - NOTNLM OT - Patient Reported Outcomes OT - Raynaud’s Phenomenon OT - Scleroderma OT - Survey OT - Systemic Sclerosis EDAT- 2019/02/02 06:00 MHDA- 2019/02/02 06:01 CRDT- 2019/02/02 06:00 PHST- 2019/02/02 06:00 [entrez] PHST- 2019/02/02 06:00 [pubmed] PHST- 2019/02/02 06:01 [medline] AID - 10.1177/2397198318774307 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2018 Oct;3(3):249-252. doi: 10.1177/2397198318774307. Epub 2018 May 24. PMID- 32748967 OWN - NLM STAT- MEDLINE DCOM- 20210427 LR - 20210427 IS - 1525-1470 (Electronic) IS - 0736-8046 (Linking) VI - 37 IP - 5 DP - 2020 Sep TI - Chondrodysplasia punctata and neonatal lupus in an infant with positive anti-RNP and negative anti-Ro/SSA and -La/SSB antibodies, a case report. PG - 925-928 LID - 10.1111/pde.14312 [doi] AB - Rhizomelic chondrodysplasia punctata is a rare, often fatal disease that shares many clinical dysmorphologic features with the rare often non-lethal chondrodysplasia punctata due to maternal autoimmune disease. Characteristic findings of both conditions include mid-face hypoplasia, stippled epiphyses of the vertebrae and long bones, and growth failure. A growing association with anti-ribonucleoprotein antibodies is emerging amongst patients with chondrodysplasia punctata due to maternal autoimmune disease and also neonatal lupus that have potential important screening implications. We present a unique case of chondrodysplasia punctata with neonatal lupus in the setting of positive anti-RNP antibodies and negative anti-Ro/SSA and -La/SSB antibodies born to a mother with mixed connective tissue disease and Raynaud's syndrome. CI - © 2020 Wiley Periodicals LLC. FAU - Milliken, Michael AU - Milliken M AUID- ORCID: 0000-0002-2116-8107 AD - Department of Dermatology, University of Utah, Salt Lake City, UT, USA. FAU - Lee, Jack AU - Lee J AD - Department of Dermatology, University of Virginia, Charlottesville, VA, USA. FAU - Cipriano, Sarah D AU - Cipriano SD AUID- ORCID: 0000-0003-0888-1458 AD - Department of Dermatology, University of Utah, Salt Lake City, UT, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200804 PL - United States TA - Pediatr Dermatol JT - Pediatric dermatology JID - 8406799 RN - 0 (Antibodies, Antinuclear) RN - Neonatal Systemic lupus erythematosus SB - IM MH - Antibodies, Antinuclear MH - *Chondrodysplasia Punctata/diagnosis MH - Female MH - Humans MH - Infant MH - Infant, Newborn MH - Lupus Erythematosus, Systemic/*congenital MH - Mothers OTO - NOTNLM OT - Neonatal lupus OT - chondrodysplasia punctata OT - maternal autoantibodies OT - maternal autoimmune disease EDAT- 2020/08/05 06:00 MHDA- 2021/04/28 06:00 CRDT- 2020/08/05 06:00 PHST- 2020/08/05 06:00 [pubmed] PHST- 2021/04/28 06:00 [medline] PHST- 2020/08/05 06:00 [entrez] AID - 10.1111/pde.14312 [doi] PST - ppublish SO - Pediatr Dermatol. 2020 Sep;37(5):925-928. doi: 10.1111/pde.14312. Epub 2020 Aug 4. PMID- 18223527 OWN - NLM STAT- MEDLINE DCOM- 20080402 LR - 20091211 IS - 1698-6946 (Electronic) IS - 1698-4447 (Linking) VI - 13 IP - 2 DP - 2008 Feb 1 TI - Oral complaints in progressive systemic sclerosis: two cases report. PG - E114-8 AB - Progressive systemic sclerosis is a chronic sclerotic disease which causes diffuse, increased deposition of extracellular matrix in connective tissue with vascular abnormalities, resulting in tissue hypoxia. Aesthetic and facial dysfunction are followed by important oral and facial manifestation of disturbances such as xerostomia, the lack of saliva in the mouth, and its associated complications. Most clinical manifestations begin with tongue rigidity. The facial skin changes and bone resorption of mandible angle are often reported. Other systemic changes include the involvement of internal organs which leads to serious complications as well as disorders in the cardiac muscle and Raynaud's phenomenon. The objective of the this paper is to report two cases of systemic sclerosis in patients with oral and facial manifestations of the disease. A brief review of the literature, focusing on deontological alterations is also presented. FAU - Cazal, Cláudia AU - Cazal C AD - Oral Pathology Department of the Dentistry School of CESMAC, Brazil. FAU - Sobral, Ana Paula Veras AU - Sobral AP FAU - Neves, Ridel Frota Sá Nogueira AU - Neves RF FAU - Freire Filho, Francisco Wagner Vasconcelos AU - Freire Filho FW FAU - Cardoso, Alvaro Bezerra AU - Cardoso AB FAU - da Silveira, Márcia Maria Fonseca AU - da Silveira MM LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20080201 PL - Spain TA - Med Oral Patol Oral Cir Bucal JT - Medicina oral, patologia oral y cirugia bucal JID - 101231694 SB - IM MH - Adult MH - Disease Progression MH - Female MH - Humans MH - Mouth Diseases/*etiology MH - Scleroderma, Diffuse/*complications RF - 11 EDAT- 2008/01/29 09:00 MHDA- 2008/04/03 09:00 CRDT- 2008/01/29 09:00 PHST- 2008/01/29 09:00 [pubmed] PHST- 2008/04/03 09:00 [medline] PHST- 2008/01/29 09:00 [entrez] AID - 10489616 [pii] PST - epublish SO - Med Oral Patol Oral Cir Bucal. 2008 Feb 1;13(2):E114-8. PMID- 41196296 OWN - NLM STAT- MEDLINE DCOM- 20251106 LR - 20251231 IS - 1420-908X (Electronic) IS - 1023-3830 (Linking) VI - 74 IP - 1 DP - 2025 Nov 6 TI - Systemic sclerosis, main culprits and involved signaling pathways. PG - 158 LID - 10.1007/s00011-025-02126-0 [doi] AB - Systemic sclerosis is an autoimmune connective tissue disease of unknown cause and diverse clinical manifestations. Vasospastic episodes (Raynaud's phenomenon), often triggered by cold or stress, typically appear at disease onset. Cytokines, particularly TGF-β, act in the inflammatory and hypoxic microenvironment to drive fibrosis, which predominantly develops at inflammatory sites. Several cell types contribute to disease pathogenesis and fibrosis, including vascular endothelial cells, vascular smooth muscle cells, and fibroblasts in the extracellular matrix. Multiple signaling pathways are activated in these cells and promote disease progression. Endothelial and vascular smooth muscle cells respond to diverse ligands through pathways such as AKT, MAPK, and GPCR signaling, which promote fibrosis progression in the profibrotic and proinflammatory milieu. Cytokines are also important mediators of inflammation and fibrosis, particularly by acting on activated monocytes in the ECM and guiding them toward M1 or M2 macrophage polarization. In the early inflammatory stage, M1 macrophages predominate, while the fibrotic stage is characterized by increased M2 macrophage presence. ECM accumulation, resulting from TGF-β signaling in fibroblasts, provides integrins with ligands and promotes enhanced adhesion and migration of these cells. TGF-β, on the other hand, can transactivate the Ras pathway, promoting myofibroblast differentiation and enhancing pro-fibrotic effects. CI - © 2025. The Author(s), under exclusive licence to Springer Nature Switzerland AG. FAU - Sadatpour, Omid AU - Sadatpour O AD - Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. AD - Research Center for Chronic Inflammatory Diseases, Tehran University of Medical Sciences, Tehran, Iran. FAU - Azizan, Amin AU - Azizan A AD - Research Center for Chronic Inflammatory Diseases, Tehran University of Medical Sciences, Tehran, Iran. AD - Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Ave, P.O. BOX: 1411713137, Tehran, Iran. FAU - Kavosi, Hoda AU - Kavosi H AD - Research Center for Chronic Inflammatory Diseases, Tehran University of Medical Sciences, Tehran, Iran. AD - Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Ave, P.O. BOX: 1411713137, Tehran, Iran. FAU - Vodjgani, Mohammad AU - Vodjgani M AD - Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. FAU - Farhadi, Elham AU - Farhadi E AD - Research Center for Chronic Inflammatory Diseases, Tehran University of Medical Sciences, Tehran, Iran. farhadie@tums.ac.ir. AD - Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Kargar Ave, P.O. BOX: 1411713137, Tehran, Iran. farhadie@tums.ac.ir. FAU - Mahmoudi, Mahdi AU - Mahmoudi M AD - Research Center for Chronic Inflammatory Diseases, Tehran University of Medical Sciences, Tehran, Iran. mahmoudim@tums.ac.ir. LA - eng PT - Journal Article PT - Review DEP - 20251106 PL - Switzerland TA - Inflamm Res JT - Inflammation research : official journal of the European Histamine Research Society ... [et al.] JID - 9508160 RN - 0 (Cytokines) RN - 0 (Transforming Growth Factor beta) SB - IM MH - Humans MH - *Scleroderma, Systemic/pathology/immunology/metabolism MH - *Signal Transduction MH - Animals MH - Fibrosis MH - Cytokines/metabolism MH - Transforming Growth Factor beta/metabolism OTO - NOTNLM OT - Fibrosis OT - Signaling pathways OT - Systemic sclerosis OT - TGF-β COIS- Declarations. Conflict of interest: The authors declare no competing interests. EDAT- 2025/11/06 12:33 MHDA- 2025/11/06 12:34 CRDT- 2025/11/06 11:13 PHST- 2025/04/01 00:00 [received] PHST- 2025/10/15 00:00 [accepted] PHST- 2025/10/11 00:00 [revised] PHST- 2025/11/06 12:34 [medline] PHST- 2025/11/06 12:33 [pubmed] PHST- 2025/11/06 11:13 [entrez] AID - 10.1007/s00011-025-02126-0 [pii] AID - 10.1007/s00011-025-02126-0 [doi] PST - epublish SO - Inflamm Res. 2025 Nov 6;74(1):158. doi: 10.1007/s00011-025-02126-0. PMID- 34854586 OWN - NLM STAT- MEDLINE DCOM- 20220106 LR - 20220531 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 165 DP - 2021 Sep 23 TI - [An asymptomatic patient with lactate acidosis and hypercapnia]. LID - D5587 [pii] AB - BACKGROUND: Blood gas analyses are used to identify acid-base and respiratory disturbances. Blood gas abnormalities can be first signs of serious underlying disease. CASE DESCRIPTION: An 82-year old women with chronic kidney disease (eGFR 35 ml/min/1.73m2) and rheumatoid arthritis was admitted to the geriatric ward due to a urinary tract infection and bloody diarrhea. She repeatedly had a low oxygen saturation, although without symptoms. Capillary blood gas analysis was evidently disturbed (pH 6.96, pCO2 9.3 kPa, lactate 8.4 mmol/l), and led to consultation of the intensivist. Careful physical examination demonstrated that the patients' hands had blue discoloration, typical for Raynaud's phenomenon. Arterial blood gas demonstrated normal results. CONCLUSION: Capillary blood gas analysis is less reliable when peripheral circulation is disturbed. When the results are aberrant, it is important to distinguish between peripheral vascular disease and diminished systemic circulation. Arterial blood gas analysis can be used to support the diagnosis. FAU - Gant, Christina M AU - Gant CM AD - Meander Medisch Centrum, afd. Interne Geneeskunde, Amersfoort. AD - Contact: Christina M. Gant (cm.gant@meandermc.nl). FAU - Gijzen, Karlijn AU - Gijzen K AD - Meander Medisch Centrum, afd. Klinische Chemie, Amersfoort. FAU - Josephus Jitta, Nienke AU - Josephus Jitta N AD - Meander Medisch Centrum, afd. Intensive Care,Amersfoort. LA - dut PT - Case Reports PT - Journal Article TT - Een asymptomatische patiënt met lactaatacidose en hypercapnie. DEP - 20210923 PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 33X04XA5AT (Lactic Acid) SB - IM MH - *Acidosis MH - Aged, 80 and over MH - Blood Gas Analysis MH - Female MH - Humans MH - *Hypercapnia MH - Lactic Acid MH - Oxygen Saturation EDAT- 2021/12/03 06:00 MHDA- 2022/01/07 06:00 CRDT- 2021/12/02 08:59 PHST- 2021/12/02 08:59 [entrez] PHST- 2021/12/03 06:00 [pubmed] PHST- 2022/01/07 06:00 [medline] AID - D5587 [pii] PST - epublish SO - Ned Tijdschr Geneeskd. 2021 Sep 23;165:D5587. PMID- 23257529 OWN - NLM STAT- MEDLINE DCOM- 20130528 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 51 IP - 24 DP - 2012 TI - The clinical characteristics of two anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibody-positive interstitial lung disease patients with polymyositis/dermatomyositis. PG - 3405-10 AB - We herein report the clinical and laboratory characteristics of two anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibody-positive interstitial lung disease patients with polymyositis/dermatomyositis (PM/DM). We compared these characteristics with previously published findings. Previous reports and our present cases show that anti-OJ autoantibody-positive interstitial lung disease (ILD) patients with PM/DM lack the manifestations of Raynaud's phenomenon and sclerodactyly and show good prognoses and responses to glucocorticoid therapy. These results indicate that the presence of anti-OJ autoantibodies may be useful for predicting the prognosis of ILD and its clinical course in PM/DM patients. FAU - Kunimasa, Kei AU - Kunimasa K AD - Department of Respiratory Medicine, Kurashiki Central Hospital, Japan. keikunimasa@gmail.com FAU - Arita, Machiko AU - Arita M FAU - Nakazawa, Takashi AU - Nakazawa T FAU - Tanaka, Maki AU - Tanaka M FAU - Tsubouchi, Kazuya AU - Tsubouchi K FAU - Konishi, Satoshi AU - Konishi S FAU - Fukuda, Yasushi AU - Fukuda Y FAU - Saigusa, Mika AU - Saigusa M FAU - Nakagawa, Hiroaki AU - Nakagawa H FAU - Ubukata, Satoshi AU - Ubukata S FAU - Korogi, Yohei AU - Korogi Y FAU - Fujii, Takao AU - Fujii T FAU - Mimori, Tsuneyo AU - Mimori T FAU - Ishida, Tadashi AU - Ishida T LA - eng PT - Case Reports PT - Journal Article DEP - 20121215 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Autoantibodies) RN - EC 6.1.1.5 (Isoleucine-tRNA Ligase) SB - IM MH - Aged MH - Autoantibodies/*immunology MH - Dermatomyositis/complications/*enzymology/*immunology MH - Female MH - Humans MH - Isoleucine-tRNA Ligase/*immunology MH - Lung Diseases, Interstitial/complications/*enzymology/*immunology MH - Male MH - Polymyositis EDAT- 2012/12/22 06:00 MHDA- 2013/05/29 06:00 CRDT- 2012/12/22 06:00 PHST- 2012/12/22 06:00 [entrez] PHST- 2012/12/22 06:00 [pubmed] PHST- 2013/05/29 06:00 [medline] AID - DN/JST.JSTAGE/internalmedicine/51.7452 [pii] AID - 10.2169/internalmedicine.51.7452 [doi] PST - ppublish SO - Intern Med. 2012;51(24):3405-10. doi: 10.2169/internalmedicine.51.7452. Epub 2012 Dec 15. PMID- 22957292 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120910 LR - 20220408 IS - 2090-6897 (Electronic) IS - 2090-6889 (Print) IS - 2090-6897 (Linking) VI - 2012 DP - 2012 TI - Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report. PG - 549298 LID - 10.1155/2012/549298 [doi] LID - 549298 AB - Acute fibrinous and organizing pneumonia (AFOP), recently described, is a histologic pattern characterized by the presence of fibrin "balls" within alveolar spaces. The term undifferentiated connective tissue disease (UCTD) is used to identify autoimmune systemic diseases that do not fulfill the criteria to be classified as a definitive connective tissue disease. The AFOP has never been reported in association with UCTD. The present reported case is a 39-year-old Caucasian, female with dry cough and progressive dyspnea. Eight months later, she was diagnosed with "organizing pneumonia" based on clinical history and radiologic images. She manifested Raynaud's Phenomenon, sicca syndrome, boot and gloves neuropathic pain, and previous hypothyroidism. Antinuclear antibody, rheumatoid factor, and specific autoantibodies were negative. Salivary gland biopsy and electroneuromyiography were normal. The capillaroscopy showed a "scleroderma" pattern with capillary deletion and ectasia. She experienced clinical and radiologic worsening. Despite being submitted to cyclophosphamide pulse, she developed hemorrhage and then died. Thoracotomy pulmonary specimen showed histological pattern of AFOP. This paper shows a rare association of AFOP with UCTD. FAU - Valim, Valéria AU - Valim V AD - Department of Internal Medicine, Universidade Federal do Espirito Santo, Marechal Campos Avenue 1355, 29040-715 Vitoria, ES, Brazil. FAU - Rocha, Roberta Hora AU - Rocha RH FAU - Couto, Roberta Barcelos AU - Couto RB FAU - Paixão, Thaysa Simões AU - Paixão TS FAU - Serrano, Erica Vieira AU - Serrano EV LA - eng PT - Case Reports PT - Journal Article DEP - 20120404 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC3420729 EDAT- 2012/09/08 06:00 MHDA- 2012/09/08 06:01 PMCR- 2012/04/04 CRDT- 2012/09/08 06:00 PHST- 2011/11/30 00:00 [received] PHST- 2012/01/29 00:00 [accepted] PHST- 2012/09/08 06:00 [entrez] PHST- 2012/09/08 06:00 [pubmed] PHST- 2012/09/08 06:01 [medline] PHST- 2012/04/04 00:00 [pmc-release] AID - 10.1155/2012/549298 [doi] PST - ppublish SO - Case Rep Rheumatol. 2012;2012:549298. doi: 10.1155/2012/549298. Epub 2012 Apr 4. PMID- 41694889 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260216 LR - 20260218 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 1 DP - 2026 Jan TI - Facial Swelling in a Young Adult With Type 1 Diabetes: Morbihan Disease as a Scleroderma Mimic. PG - e101635 LID - 10.7759/cureus.101635 [doi] LID - e101635 AB - Scleredema diabeticorum and Morbihan disease (solid facial edema) can mimic scleroderma, creating diagnostic challenges for rheumatologists. We report a 19-year-old male with poorly controlled type 1 diabetes (glycated hemoglobin (HbA1c) >9%) presenting with progressive facial swelling. Extensive workup excluded superior vena cava syndrome and connective tissue diseases. Histopathological examination of the skin fragment was suggestive of scleredema diabeticorum or Morbihan disease secondary to rosacea. Key differentiating features included sparing of hands/feet, absence of Raynaud's phenomenon, and non-specific low-titer anti-nuclear antibody (ANA). This case emphasizes recognizing scleroderma mimics to ensure appropriate therapy. CI - Copyright © 2026, Shammas et al. FAU - Shammas, Rania AU - Shammas R AD - Rheumatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA. FAU - Gudino-Rosales, Santiago AU - Gudino-Rosales S AD - Dermatology, University of California Riverside, Riverside, USA. FAU - Kneiber, Diana AU - Kneiber D AD - Dermatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA. FAU - Sarantopoulos, G Peter AU - Sarantopoulos GP AD - Pathology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA. FAU - Aung, Thanda AU - Aung T AD - Rheumatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20260115 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12906367 OTO - NOTNLM OT - facial swelling OT - morbihan OT - rosacea OT - scleredema diabeticorum OT - scleroderma mimic COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/02/16 12:30 MHDA- 2026/02/16 12:31 PMCR- 2026/01/15 CRDT- 2026/02/16 06:45 PHST- 2025/11/01 00:00 [received] PHST- 2026/01/14 00:00 [accepted] PHST- 2026/02/16 12:31 [medline] PHST- 2026/02/16 12:30 [pubmed] PHST- 2026/02/16 06:45 [entrez] PHST- 2026/01/15 00:00 [pmc-release] AID - 10.7759/cureus.101635 [doi] PST - epublish SO - Cureus. 2026 Jan 15;18(1):e101635. doi: 10.7759/cureus.101635. eCollection 2026 Jan. PMID- 23047635 OWN - NLM STAT- MEDLINE DCOM- 20130807 LR - 20200928 IS - 1349-7693 (Electronic) IS - 0446-6586 (Linking) VI - 109 IP - 10 DP - 2012 Oct TI - [A case of protein-losing gastroenteropathy accompanied by Sjögren syndrome and mixed connective tissue disease]. PG - 1770-5 AB - Case reports of protein-losing gastroenteropathy (PLGE) associated with not only mixed connective tissue disease (MCTD) but also Sjögren syndrome (SjS) are very rare. We report a first case of PLGE in a patient with both MCTD and SjS. A 58-year-old Japanese woman was referred and admitted to our hospital because of abdominal fullness and lower leg edema. Her past medical history revealed SjS at age 40. Physical examination demonstrated lower leg edema and Raynaud's phenomenon. Blood chemistry data showed severe hypoproteinemia. Anti RNP antibody was positive. MCTD was diagnosed. The alpha-1 antitrypsin clearance level was high. The (99m)Tc-DTPA human serum albumin scintigraphy demonstrated abnormal accumulation in the intestine. PLGE associated with both MCTD and SjS was diagnosed, but she was successfully treated by prednisolone. FAU - Kakigao, Kana AU - Kakigao K AD - Clinical Research Center, National Hospital Organization Kyushu Medical Center, Japan. FAU - Fukushima, Nobuyoshi AU - Fukushima N FAU - Mizutani, Takahiro AU - Mizutani T FAU - Haraguchi, Kazuhiro AU - Haraguchi K FAU - Okamoto, Risa AU - Okamoto R FAU - Sawamura, Noriko AU - Sawamura N FAU - Oohashi, Tomoko AU - Oohashi T FAU - Mitsuyasu, Aya AU - Mitsuyasu A FAU - Fujiyama, Takashi AU - Fujiyama T FAU - Yoshimoto, Tsuyoshi AU - Yoshimoto T FAU - Koujima, Motoyuki AU - Koujima M FAU - Kawabe, Ken AU - Kawabe K FAU - Fukuizumi, Kunitaka AU - Fukuizumi K FAU - Nakamuta, Makoto AU - Nakamuta M FAU - Harada, Naohiko AU - Harada N LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Nihon Shokakibyo Gakkai Zasshi JT - Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology JID - 2984683R SB - IM MH - Female MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications MH - Protein-Losing Enteropathies/*complications MH - Sjogren's Syndrome/*complications EDAT- 2012/10/11 06:00 MHDA- 2013/08/08 06:00 CRDT- 2012/10/11 06:00 PHST- 2012/10/11 06:00 [entrez] PHST- 2012/10/11 06:00 [pubmed] PHST- 2013/08/08 06:00 [medline] AID - DN/JST.JSTAGE/nisshoshi/109.1770 [pii] PST - ppublish SO - Nihon Shokakibyo Gakkai Zasshi. 2012 Oct;109(10):1770-5. PMID- 30017344 OWN - NLM STAT- MEDLINE DCOM- 20191223 LR - 20210525 IS - 2255-534X (Electronic) IS - 2255-534X (Linking) VI - 84 IP - 2 DP - 2019 Apr-Jun TI - Autoimmune associations in a Mexican cohort with primary biliary cholangitis. PG - 130-135 LID - S0375-0906(18)30126-5 [pii] LID - 10.1016/j.rgmx.2018.03.008 [doi] AB - INTRODUCTION: Several groups have reported associations of primary biliary cholangitis with other autoimmune entities, particularly Sjögren's syndrome and hypothyroidism. Its prevalence and characteristics in Mexican patients is unknown. AIM: To determine the frequency and characteristics of autoimmune diseases in a Mexican cohort of patients with primary biliary cholangitis. MATERIALS AND METHODS: The medical records of patients that presented with primary biliary cholangitis within the time frame of 2005 and 2012 were reviewed and assessed for other autoimmune diseases. RESULTS: Seventy-eight patients, 75 women and 3 men, were included. Their mean age was 55.8 years. Seventy-three cases had positive antimitochondrial antibodies (94.8%) and disease was confirmed in 5 through liver biopsy. Five patients (8%) had anti-smooth muscle antibodies and 55/78 (70.5%) had antinuclear antibodies by indirect immunofluorescence. Forty-nine patients (62.8%) were positive for an autoimmune disease other than primary biliary cholangitis. Among those, 20 patients had one associated disease, 14 had 2, and 15 patients had 3 concomitant diseases. They included: Sjögren's syndrome in 23/78 patients (29.5%), dysthyroidism in 21/78 cases (26.9%), Raynaud syndrome in 11/78 (14.1%), CREST syndrome in 9/78 patients (11.4%), rheumatoid arthritis in 6/78 patients (7.7%), vitiligo in 5/78 (6.4%), scleroderma in 4/78 patients (5.1%), and other diseases in 8 patients. In 12/78 patients (15.4%), there was a documented family background of autoimmune disease. CONCLUSIONS: The presence of autoimmune associations in our cohort was frequent, and similar in characteristics to the information reported by other groups. The clinical implications of those findings remain to be determined. CI - Copyright © 2018 Asociación Mexicana de Gastroenterología. Publicado por Masson Doyma México S.A. All rights reserved. FAU - González-Huezo, M S AU - González-Huezo MS AD - Departamento de Gastroenterología, Centro Médico ISSEMyM, Metepec, México. Electronic address: saraigh69@yahoo.com. FAU - Delgado-Ayala, L Y AU - Delgado-Ayala LY AD - Departamento de Medicina Interna, Centro Médico ISSEMyM, Metepec, México. FAU - Osorio-Núñez, A L AU - Osorio-Núñez AL AD - Departamento de Gastroenterología, Centro Médico ISSEMyM, Metepec, México. FAU - Meléndez-Mercado, C AU - Meléndez-Mercado C AD - Departamento de Reumatología, Centro Médico ISSEMyM, Metepec, México. LA - eng LA - spa PT - Journal Article PT - Observational Study TT - Asociaciones autoinmunes en una cohorte mexicana con colangitis biliar primaria. DEP - 20180714 PL - Mexico TA - Rev Gastroenterol Mex (Engl Ed) JT - Revista de gastroenterologia de Mexico (English) JID - 101778603 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Autoantibodies/analysis MH - Autoimmune Diseases/complications/*epidemiology/*immunology MH - Cohort Studies MH - Comorbidity MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/complications/*epidemiology/*immunology MH - Male MH - Mexico/epidemiology MH - Middle Aged MH - Prevalence MH - Retrospective Studies MH - Sjogren's Syndrome OTO - NOTNLM OT - Cirrosis biliar primaria OT - Connective tissue diseases OT - Enfermedades del tejido conectivo OT - Fenómeno de Raynaud OT - Hipotiroidismo OT - Hypothyroidism OT - Primary biliary cirrhosis OT - Raynaud's phenomenon OT - Sjögren's syndrome OT - Síndrome de Sjögren EDAT- 2018/07/19 06:00 MHDA- 2019/12/24 06:00 CRDT- 2018/07/19 06:00 PHST- 2017/12/12 00:00 [received] PHST- 2018/03/06 00:00 [revised] PHST- 2018/03/07 00:00 [accepted] PHST- 2018/07/19 06:00 [pubmed] PHST- 2019/12/24 06:00 [medline] PHST- 2018/07/19 06:00 [entrez] AID - S0375-0906(18)30126-5 [pii] AID - 10.1016/j.rgmx.2018.03.008 [doi] PST - ppublish SO - Rev Gastroenterol Mex (Engl Ed). 2019 Apr-Jun;84(2):130-135. doi: 10.1016/j.rgmx.2018.03.008. Epub 2018 Jul 14. PMID- 26785763 OWN - NLM STAT- MEDLINE DCOM- 20171102 LR - 20171102 IS - 1130-0108 (Print) IS - 1130-0108 (Linking) VI - 108 IP - 9 DP - 2016 Sep TI - Rare association of celiac disease with myasthenia gravis in a patient with other immune disorders: a case report. PG - 586-8 LID - 10.17235/reed.2016.3929/2015 [doi] AB - BACKGROUND: Celiac disease is described in association with several autoimmune diseases, but rarely with myasthenia gravis. CASE REPORT: We describe the case of a 31-year-old white woman with celiac disease who presented manifestations related to a hyperactive immune system, including macroamylasemia, false-positive anti-HCV, positive antinuclear antibody, and Raynaud's phenomenon. The introduction of a gluten-free diet (GFD) resolved these features, but myasthenia gravis (MG) symptoms unexpectedly occurred on that occasion. DISCUSSION: The role of a GFD in the course of autoimmune diseases has been studied and improvement has been reported in many diseases. However, there is no consensus in the literature regarding the course of neurological disorders associated with celiac disease. In the present case, a GFD did not prevent the appearance of symptoms related to myasthenia gravis. There are few reports on the association of celiac disease with myasthenia gravis and therefore little is known about the course and time of onset of myasthenia in celiac patients. The present case increases the knowledge about this unusual autoimmune neurological disease associated with celiac disease. FAU - de Almeida Menezes, Marcela AU - de Almeida Menezes M AD - Internal Medicine Department, State University of Campinas (UNICAMP), Brazil. FAU - Ribeiro Cabral, Vírginia Lúcia AU - Ribeiro Cabral VL AD - Department of Internal Medicine, State University of Campinas, Brazil. FAU - Lorena, Sônia S AU - Lorena SS AD - Department of Internal Medicine, State University of Campinas, Brazil. FAU - Nucci, Anamarli AU - Nucci A AD - Department of Neurology, State University of Campinas, Brazil. FAU - Andrade Santana, Priscila AU - Andrade Santana P AD - Department of Internal Medicine, State University of Campinas, Brazil. FAU - Queiroz Silva, Cecília AU - Queiroz Silva C AD - Department of Internal Medicine, State University of Campinas, Brazil. LA - eng PT - Case Reports PT - Journal Article PL - Spain TA - Rev Esp Enferm Dig JT - Revista espanola de enfermedades digestivas JID - 9007566 SB - IM MH - Adult MH - Celiac Disease/blood/*complications/diet therapy MH - Diet, Gluten-Free MH - Female MH - Humans MH - Immune System Diseases/*complications MH - Myasthenia Gravis/*complications/diet therapy/drug therapy EDAT- 2016/01/21 06:00 MHDA- 2017/11/03 06:00 CRDT- 2016/01/21 06:00 PHST- 2016/01/21 06:00 [entrez] PHST- 2016/01/21 06:00 [pubmed] PHST- 2017/11/03 06:00 [medline] AID - 10.17235/reed.2016.3929/2015 [doi] PST - ppublish SO - Rev Esp Enferm Dig. 2016 Sep;108(9):586-8. doi: 10.17235/reed.2016.3929/2015. PMID- 34367753 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210810 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 7 DP - 2021 Jul TI - A Rare Case Presentation: Diagnosing Primary Biliary Cholangitis in a Male Patient With Concomitant Type 1 Diabetes. PG - e16109 LID - 10.7759/cureus.16109 [doi] LID - e16109 AB - Primary biliary cholangitis (PBC) is characterized as an autoimmune disease that involves the destruction of intrahepatic bile ducts, characteristically leading to a cholestatic liver. The presence of disease-specific antimitochondrial antibodies (AMA) is the gold standard to diagnose PBC. Typically, PBC is known to affect female populations exceedingly over their male counterparts. Associated autoimmune diseases include Sjogren's and Raynaud's syndrome, which are also more prevalent in women. The low incidence rates of men affected with PBC, especially with a concomitant type 1 diabetes diagnosis, have resulted in little being known about the clinical course of the disease in this particular population group. Current research suggests no significant histological, serological, or biochemical differences between PBC in males and females. However, some symptoms and clinical associations may be different. This case report presents the rare case of a male patient with type 1 diabetes recently diagnosed with PBC. CI - Copyright © 2021, Johnson et al. FAU - Johnson, Adejoke M AU - Johnson AM AD - Medicine, All Saints University School of Medicine, Roseau, DMA. FAU - Akpan, Ezekiel J AU - Akpan EJ AD - Medicine, All Saints University College of Medicine, Kingstown, VCT. FAU - Kale, Supriya AU - Kale S AD - School of Medicine, Windsor University, Canyon, KNA. FAU - Patel, Anna AU - Patel A AD - Gastroenterology and Hepatology, Community First Medical Center, Chicago, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210702 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8330503 OTO - NOTNLM OT - antimitochondrial antibody OT - autoimmune disease OT - cholestyramine OT - liver cirrhosis OT - male primary biliary cholangitis OT - primary biliary cholangitis OT - pruritis OT - type 1 diabetes OT - ursodiol COIS- The authors have declared that no competing interests exist. EDAT- 2021/08/10 06:00 MHDA- 2021/08/10 06:01 PMCR- 2021/07/02 CRDT- 2021/08/09 06:38 PHST- 2021/07/02 00:00 [accepted] PHST- 2021/08/09 06:38 [entrez] PHST- 2021/08/10 06:00 [pubmed] PHST- 2021/08/10 06:01 [medline] PHST- 2021/07/02 00:00 [pmc-release] AID - 10.7759/cureus.16109 [doi] PST - epublish SO - Cureus. 2021 Jul 2;13(7):e16109. doi: 10.7759/cureus.16109. eCollection 2021 Jul. PMID- 40006015 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250228 IS - 1424-8247 (Print) IS - 1424-8247 (Electronic) IS - 1424-8247 (Linking) VI - 18 IP - 2 DP - 2025 Feb 2 TI - Assessment of Treatment Effects of Aminaphtone by Capillaroscopy in a Patient with Raynaud's Phenomenon. LID - 10.3390/ph18020203 [doi] LID - 203 AB - Background: Aminaphtone is a well-established pharmaceutical agent that has been utilized for over 40 years, primarily due to its effectiveness in treating microvascular disorders. Recent studies have explored its impact on various conditions, including chronic venous insufficiency, diabetic microangiopathy, leg ulcers, systemic sclerosis, and Raynaud's phenomenon. These investigations have consistently demonstrated that aminaphtone enhances skin blood perfusion and mitigates endothelial damage, all while maintaining a robust safety profile over time. Case Summary: This report highlights the potential of aminaphtone in improving microcirculation in a young patient who experienced spontaneous capillary rupture in her second finger. A 38-year-old woman with undifferentiated connective tissue disease presented to the clinic for periungual videocapillaroscopy (NVC). Given the microangiopathic changes observed during the NVC, she was prescribed aminaphtone. After seven months of treatment, a follow-up NVC revealed significant improvement in the capillaroscopic findings. A comprehensive literature review on aminaphtone was conducted using electronic databases (PUBMED, Google Scholar, ResearchGate, UpToDate), along with manual searches, focusing on articles published until November 2024. Conclusion: Treatment with aminaphtone led to notable improvements in microangiopathic health. Following the introduction of this medication, the nailfold microvascular bed, which previously exhibited severe alterations, showed a remarkable transition to only mild abnormalities. FAU - Screm, Gianluca AU - Screm G AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Mondini, Lucrezia AU - Mondini L AUID- ORCID: 0000-0002-7043-9639 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Salton, Francesco AU - Salton F AUID- ORCID: 0000-0002-0864-9766 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Confalonieri, Paola AU - Confalonieri P AUID- ORCID: 0000-0002-7284-5016 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Bozzi, Chiara AU - Bozzi C AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Torregiani, Chiara AU - Torregiani C AUID- ORCID: 0000-0001-9110-3029 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Antonaglia, Caterina AU - Antonaglia C AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Geri, Pietro AU - Geri P AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - D'Oria, Mario AU - D'Oria M AUID- ORCID: 0000-0002-7156-7827 AD - Division of Vascular and Endovascular Surgery, Department of Clinical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy. FAU - Bandini, Giulia AU - Bandini G AUID- ORCID: 0000-0002-2076-7319 AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine, Azienda Ospedaliero Universitaria Careggi, University of Florence, 50134 Florence, Italy. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester M6 8HD, UK. FAU - Confalonieri, Marco AU - Confalonieri M AUID- ORCID: 0000-0002-4791-768X AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20250202 PL - Switzerland TA - Pharmaceuticals (Basel) JT - Pharmaceuticals (Basel, Switzerland) JID - 101238453 PMC - PMC11859383 OTO - NOTNLM OT - aminaphtone OT - capillary disorders OT - nailfold video capillaroscopy (NVC) COIS- The authors declare no conflicts of interest. EDAT- 2025/02/26 06:28 MHDA- 2025/02/26 06:29 PMCR- 2025/02/02 CRDT- 2025/02/26 01:26 PHST- 2024/12/09 00:00 [received] PHST- 2025/01/30 00:00 [revised] PHST- 2025/01/30 00:00 [accepted] PHST- 2025/02/26 06:29 [medline] PHST- 2025/02/26 06:28 [pubmed] PHST- 2025/02/26 01:26 [entrez] PHST- 2025/02/02 00:00 [pmc-release] AID - ph18020203 [pii] AID - pharmaceuticals-18-00203 [pii] AID - 10.3390/ph18020203 [doi] PST - epublish SO - Pharmaceuticals (Basel). 2025 Feb 2;18(2):203. doi: 10.3390/ph18020203. PMID- 19393210 OWN - NLM STAT- MEDLINE DCOM- 20090911 LR - 20161125 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 8 IP - 7 DP - 2009 Jun TI - Anti-RNA polymerase III antibodies: a marker of systemic sclerosis with rapid onset and skin thickening progression. PG - 580-4 LID - 10.1016/j.autrev.2009.02.002 [doi] AB - Anti-RNA polymerase III antibodies (ARA) are a specific marker for Systemic Sclerosis (SSc), associated to severe disease with major organ and diffuse cutaneous involvement. In our series, ARA were found in 19 of 216 sera, in 15 cases as isolated antibodies' specificity, with a statistically negative association with other SSc-specific autoantibodies (p: 0.00003). The prevalence of ARA among 73 anticentromere and anti-topoisomerase I (topo I) negative sera, was 20.5%. Patients with isolated ARA had more rapid disease onset, defined as the interval from the appearance of Raynaud's phenomenon to the first symptom other than Raynaud's, than patients with isolated anti-topo I antibodies (median: 2 months vs 13 months; p: 0.0013). A rapid onset of SSc (within 6 months from Raynaud's phenomenon onset) was found in all patients with isolated ARA and only in 34% of those with anti-topo I (p<0.00001). Moreover, the skin thickening in the first months after SSc onset was faster in the ARA group (p<0.0001). Nevertheless, the rates of internal organ involvement and of survival rates were similar between the two groups. Our experience therefore suggests that ARA are a marker of very rapid onset of disease and skin thickening progression in SSc. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology Unit and Chair, Spedali Civili, Brescia, Italy. ilariacava@virgilio.it FAU - Ceribelli, Angela AU - Ceribelli A FAU - Airo', Paolo AU - Airo' P FAU - Zingarelli, Stefania AU - Zingarelli S FAU - Tincani, Angela AU - Tincani A FAU - Franceschini, Franco AU - Franceschini F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090209 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM EIN - Autoimmun Rev. 2013 Jan;12(3):457. Angela, Ceribelli [corrected to Ceribelli, Angela]; Paolo, Airo' [corrected to Airo', Paolo]; Stefania, Zingarelli [corrected to Zingarelli, Stefania]; Angela, Tincani [corrected to Tincani, Angela]; Franco, Franceschini [corrected to Franceschini, Franco] MH - Antibody Specificity MH - Autoantibodies/*blood/immunology MH - Biomarkers/blood MH - Disease Progression MH - Female MH - Humans MH - Male MH - Middle Aged MH - RNA Polymerase III/*immunology MH - Scleroderma, Systemic/blood/*immunology/*pathology MH - Skin/immunology/*pathology EDAT- 2009/04/28 09:00 MHDA- 2009/09/12 06:00 CRDT- 2009/04/28 09:00 PHST- 2009/01/03 00:00 [received] PHST- 2009/02/01 00:00 [accepted] PHST- 2009/04/28 09:00 [entrez] PHST- 2009/04/28 09:00 [pubmed] PHST- 2009/09/12 06:00 [medline] AID - S1568-9972(09)00034-2 [pii] AID - 10.1016/j.autrev.2009.02.002 [doi] PST - ppublish SO - Autoimmun Rev. 2009 Jun;8(7):580-4. doi: 10.1016/j.autrev.2009.02.002. Epub 2009 Feb 9. PMID- 30105937 OWN - NLM STAT- MEDLINE DCOM- 20190528 LR - 20221207 IS - 1477-0377 (Electronic) IS - 1358-863X (Linking) VI - 24 IP - 1 DP - 2019 Feb TI - Peripheral vascular manifestation in patients receiving an amphetamine analog: A case series. PG - 50-55 LID - 10.1177/1358863X18790101 [doi] AB - Amphetamine and its related derivatives and analogues (ADRA) are highly addictive central nervous system stimulants that are used commonly in the treatment of attention-deficit/hyperactivity disorder and narcolepsy. These medications are associated with many side effects but reports of peripheral arterial manifestations associated with ADRA usage are scarce. We retrospectively reviewed the records of 16 patients (median age 37 years (IQR 31-47), 13 females) referred to a single tertiary referral service while receiving ADRA. Follow-up was available for a median of 3 years (IQR 3-4.5). The most common presentation (62.5%) was mild vasospastic symptoms involving the upper, lower or both extremities. Six patients developed severe manifestations including tissue loss and the need for lower extremity amputation. Most patients (75%) refused to stop the medication during follow-up. Underlying rheumatologic disorders were found in 25% of the patients, and the presence of rheumatologic disease seemed to be associated with more severe vascular manifestations. In conclusion, it is important to search for ADRA usage as part of the differential diagnosis of digital ischemia. FAU - Tan, GuangMing AU - Tan G AUID- ORCID: 0000-0001-6828-6269 AD - 1 Cardiology Division, Massachusetts General Hospital, Boston, MA, USA. FAU - Mintz, Ari J AU - Mintz AJ AD - 2 Department of Cardiovascular Medicine, Lahey Hospital and Medical Center, Burlington, MA, USA. FAU - Mintz, Bruce AU - Mintz B AD - 3 Cardiovascular Medicine, Gagnon Heart Hospital, Morristown, NJ, USA. FAU - Borges, Jorge C AU - Borges JC AD - 1 Cardiology Division, Massachusetts General Hospital, Boston, MA, USA. FAU - Hines, Mary Deborah AU - Hines MD AD - 1 Cardiology Division, Massachusetts General Hospital, Boston, MA, USA. FAU - Schainfeld, Robert M AU - Schainfeld RM AD - 1 Cardiology Division, Massachusetts General Hospital, Boston, MA, USA. FAU - Jaff, Michael R AU - Jaff MR AD - 4 Department of Medicine, Newton-Wellesley Hospital, Newton, MA, USA. FAU - Weinberg, Ido AU - Weinberg I AD - 1 Cardiology Division, Massachusetts General Hospital, Boston, MA, USA. LA - eng PT - Journal Article DEP - 20180814 PL - England TA - Vasc Med JT - Vascular medicine (London, England) JID - 9610930 RN - 0 (Amphetamines) RN - 0 (Central Nervous System Stimulants) SB - IM MH - Adult MH - Amphetamines/*adverse effects MH - Amputation, Surgical MH - Central Nervous System Stimulants/*adverse effects MH - Female MH - Humans MH - Ischemia/*chemically induced/diagnostic imaging/physiopathology/therapy MH - Limb Salvage MH - Lower Extremity/*blood supply MH - Male MH - Middle Aged MH - Peripheral Arterial Disease/*chemically induced/diagnostic imaging/physiopathology/therapy MH - Retrospective Studies MH - Risk Factors MH - Severity of Illness Index MH - Upper Extremity/*blood supply MH - Vasoconstriction/*drug effects MH - Young Adult OTO - NOTNLM OT - Raynaud’s phenomenon OT - acrocyanosis OT - amphetamine OT - digital ischemia EDAT- 2018/08/15 06:00 MHDA- 2019/05/29 06:00 CRDT- 2018/08/15 06:00 PHST- 2018/08/15 06:00 [pubmed] PHST- 2019/05/29 06:00 [medline] PHST- 2018/08/15 06:00 [entrez] AID - 10.1177/1358863X18790101 [doi] PST - ppublish SO - Vasc Med. 2019 Feb;24(1):50-55. doi: 10.1177/1358863X18790101. Epub 2018 Aug 14. PMID- 36105282 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220917 IS - 1664-042X (Print) IS - 1664-042X (Electronic) IS - 1664-042X (Linking) VI - 13 DP - 2022 TI - The application of artificial gravity in medicine and space. PG - 952723 LID - 10.3389/fphys.2022.952723 [doi] LID - 952723 AB - Gravity plays a crucial role in physiology. The lack of gravity, like in long duration spaceflight missions, cause pathologies in e.g., the musculoskeletal system, cardiovascular deconditioning, immune system deprivation or brain abnormalities, to just mention a few. The application of artificial gravity through short-arm human centrifugation (SAHC) has been studied as a possible countermeasure to treat spaceflight deconditioning. However, hypergravity protocols applied by using SAHC have also been used to treat different, ground-based pathologies. Such gravitational therapies have been applied in Uruguay for more than four decades now. The aim of this overview is to summarize the most important findings about the effects of gravitational therapy in different, mainly vascular based pathologies according to the experience in the Gravitational Therapy Center and to discuss the current research in the field of hypergravity applications in medicine but also as multisystem countermeasure for near weightlessness pathologies. New insight is needed on the use of hypergravity in medicine and space research and application. CI - Copyright © 2022 Isasi, Isasi and van Loon. FAU - Isasi, Eugenia AU - Isasi E AD - Centro de Terapia Gravitacional, Montevideo, Uruguay. AD - Departamento de Histología y Embriología, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay. FAU - Isasi, Maria E AU - Isasi ME AD - Centro de Terapia Gravitacional, Montevideo, Uruguay. FAU - van Loon, Jack J W A AU - van Loon JJWA AD - Department of Oral and Maxillofacial Surgery/Pathology, Amsterdam Movement Sciences & Amsterdam Bone Center (ABC), Amsterdam UMC location Vrije Universiteit Amsterdam & Academic Center for Dentistry Amsterdam (ACTA), Amsterdam, Netherlands. AD - Life Support and Physical Sciences Section (TEC-MMG), European Space Agency (ESA), European Space Research and Technology Centre (ESTEC), Noordwijk, Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20220829 PL - Switzerland TA - Front Physiol JT - Frontiers in physiology JID - 101549006 PMC - PMC9465481 OTO - NOTNLM OT - Complex Regional Pain Syndrome (CRPS) OT - Coronary Artery Disease (CAD) OT - Lymphedema OT - Peripheral Artery Disease (PAD) OT - Secondary Raynaud’s Phenomenon OT - Systemic Sclerosis OT - human centrifugation OT - microgravity COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/09/16 06:00 MHDA- 2022/09/16 06:01 PMCR- 2022/08/29 CRDT- 2022/09/15 02:19 PHST- 2022/05/25 00:00 [received] PHST- 2022/07/18 00:00 [accepted] PHST- 2022/09/15 02:19 [entrez] PHST- 2022/09/16 06:00 [pubmed] PHST- 2022/09/16 06:01 [medline] PHST- 2022/08/29 00:00 [pmc-release] AID - 952723 [pii] AID - 10.3389/fphys.2022.952723 [doi] PST - epublish SO - Front Physiol. 2022 Aug 29;13:952723. doi: 10.3389/fphys.2022.952723. eCollection 2022. PMID- 21189705 OWN - NLM STAT- MEDLINE DCOM- 20110111 LR - 20171116 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 58 DP - 2010 Aug TI - Mixed connective tissue disorder and Castleman's disease. PG - 515-7 AB - We present a 16-year-old girl who presented with polyarthritis in association with Raynaud's phenomenon, malar rash, oral ulcers, photosensitivity and alopecia of 6 months duration. On evaluation, it emerged that she had a mixed connective tissue disorder with a mesangio-proliferative glomerulonephritis. Her Chest radiograph revealed a well defined left mid and lower zone opacity with evidence of a hilar mass on CT Thorax. Histopathological examination following CT guided biopsy of the mass revealed a hyaline vascular type of Castleman's disease. Mixed Connective Tissue Disorder with Castleman's Disease is a rare association; the patient presenting with varied and interesting manifestations. It is important to understand this association in view of management. The exact etio-pathogenesis of the autoimmune manifestations in patients with Castleman's disease is not clear. Treatment with immunosuppression can suppress both immune manifestations and result in tumour regression as well. FAU - Chrispal, Anugrah AU - Chrispal A AD - Department of Medicine, Christian Medical College, Vellore. FAU - Vasuki, Zoya AU - Vasuki Z FAU - Thomas, Elsa Mary AU - Thomas EM FAU - Boorugu, Hari Kishan AU - Boorugu HK LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 9PHQ9Y1OLM (Prednisolone) RN - MRK240IY2L (Azathioprine) SB - IM MH - Adolescent MH - Azathioprine/therapeutic use MH - Biopsy MH - Castleman Disease/complications/*pathology MH - Female MH - Glomerulonephritis, Membranoproliferative/*pathology MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Mixed Connective Tissue Disease/complications/drug therapy/*pathology MH - Prednisolone/therapeutic use MH - Tomography, X-Ray Computed MH - Treatment Outcome EDAT- 2010/12/31 06:00 MHDA- 2011/01/12 06:00 CRDT- 2010/12/31 06:00 PHST- 2010/12/31 06:00 [entrez] PHST- 2010/12/31 06:00 [pubmed] PHST- 2011/01/12 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2010 Aug;58:515-7. PMID- 25461837 OWN - NLM STAT- MEDLINE DCOM- 20150526 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 14 IP - 3 DP - 2015 Mar TI - Undifferentiated Connective Tissue Disease at risk for systemic sclerosis (SSc) (so far referred to as very early/early SSc or pre-SSc). PG - 210-3 LID - S1568-9972(14)00260-2 [pii] LID - 10.1016/j.autrev.2014.11.002 [doi] AB - In the last few years, a number of studies have been published on a condition characterized by Raynaud's phenomenon (RP) associated with systemic sclerosis (SSc) marker autoantibodies and/or scleroderma-type capillaroscopic abnormalities and referred to as very early/early SSc. The present review is devoted to analyze pathophysiologic, clinical, and evolutive aspects of the condition that would induce to label it as Undifferentiated Connective Tissue Disease at risk for SSc and to split it into 3 subsets (i.e. RP associated to marker autoantibodies and scleroderma-type capillaroscopic abnormalities; RP associated to marker autoantibodies in the absence of scleroderma-type capillaroscopic abnormalities; and RP associated to scleroderma-type capillaroscopic abnormalities without any detectable marker autoantibody), which have been shown to carry different degrees of risk, but not the certainty, to develop overt SSc over time. This nosographic approach is instrumental to plan future studies devoted to investigate validated biomarkers heralding the development of major vascular disease manifestations as well as skin and/or organ fibrosis in patients at risk. CI - Copyright © 2014. Published by Elsevier B.V. FAU - Valentini, Gabriele AU - Valentini G AD - Rheumatology Unit, Second University of Naples, Naples, Italy. Electronic address: gabriele.valentini@unina2.it. LA - eng PT - Journal Article PT - Review DEP - 20141118 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Animals MH - Autoantibodies/immunology MH - Biomarkers/analysis MH - Connective Tissue Diseases/complications/*immunology MH - Humans MH - Risk Factors MH - Scleroderma, Systemic/complications/*immunology OTO - NOTNLM OT - Nailfold videocapillaroscopy OT - Scleroderma OT - Scleroderma spectrum antibodies OT - Undifferentiated Connective Tissue Disease EDAT- 2014/12/03 06:00 MHDA- 2015/05/27 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/11/01 00:00 [received] PHST- 2014/11/05 00:00 [accepted] PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/05/27 06:00 [medline] AID - S1568-9972(14)00260-2 [pii] AID - 10.1016/j.autrev.2014.11.002 [doi] PST - ppublish SO - Autoimmun Rev. 2015 Mar;14(3):210-3. doi: 10.1016/j.autrev.2014.11.002. Epub 2014 Nov 18. PMID- 30603599 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2213-0071 (Print) IS - 2213-0071 (Electronic) IS - 2213-0071 (Linking) VI - 26 DP - 2019 TI - Anti-Ku antibody-positive desquamative interstitial pneumonia. PG - 115-117 LID - 10.1016/j.rmcr.2018.12.007 [doi] AB - A 66-year-old man, an ex-smoker, was referred to our hospital for slightly progressive respiratory symptoms of cough and dyspnea on exertion and chest abnormal shadow. Chest high-resolution computed tomography showed wide-ranging ground-glass attenuation and reticulation with lower lobe predominance. Bronchoalveolar lavage (BAL) fluid revealed a marked increase in lymphocytes (53.0%), and a surgical lung biopsy revealed a pattern of desquamative interstitial pneumonia (DIP) with hyperplasia of the lymphoid follicles. His serum was positive for anti-Ku and anti-SS-A antibodies, and he had signs (such as Raynaud's phenomenon, joint pain, and mechanic's hand) suspicious of connective tissue disease (CTD) although a definitive diagnosis of CTD had not been established. On the basis of the findings in our patient obtained from the serologic domain, BAL, and pathological examination, clinicians should consider the important correlation of DIP with CTD as well as with smoking. FAU - Otoshi, Ryota AU - Otoshi R AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Yamakawa, Hideaki AU - Yamakawa H AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. AD - Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan. FAU - Takemura, Tamiko AU - Takemura T AD - Department of Pathology, Japan Red Cross Medical Center, Tokyo, Japan. FAU - Katano, Takuma AU - Katano T AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Aiko, Naoto AU - Aiko N AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Matama, Goushi AU - Matama G AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Isomoto, Kohsuke AU - Isomoto K AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Baba, Tomohisa AU - Baba T AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Hagiwara, Eri AU - Hagiwara E AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Ogura, Takashi AU - Ogura T AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20181216 PL - England TA - Respir Med Case Rep JT - Respiratory medicine case reports JID - 101604463 PMC - PMC6302154 OTO - NOTNLM OT - Anti-Ku antibody OT - Connective tissue disease OT - Desquamative interstitial pneumonia EDAT- 2019/01/04 06:00 MHDA- 2019/01/04 06:01 PMCR- 2018/12/16 CRDT- 2019/01/04 06:00 PHST- 2018/09/06 00:00 [received] PHST- 2018/12/14 00:00 [accepted] PHST- 2019/01/04 06:00 [entrez] PHST- 2019/01/04 06:00 [pubmed] PHST- 2019/01/04 06:01 [medline] PHST- 2018/12/16 00:00 [pmc-release] AID - S2213-0071(18)30277-6 [pii] AID - 10.1016/j.rmcr.2018.12.007 [doi] PST - epublish SO - Respir Med Case Rep. 2018 Dec 16;26:115-117. doi: 10.1016/j.rmcr.2018.12.007. eCollection 2019. PMID- 35083289 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220128 IS - 2296-9705 (Print) IS - 2296-9705 (Electronic) VI - 11 IP - 3 DP - 2021 Sep-Dec TI - A Case of Deficiency of Adenosine Deaminase 2: 28 years of Diagnostic Challenges. PG - 340-347 LID - 10.1159/000517141 [doi] AB - Deficiency of adenosine deaminase 2 (DADA2) is a unique monogenic autoinflammatory disease caused by autosomal recessive loss-of-function mutations in the CECR1 gene which presents as childhood-onset small- and medium-vessel vasculitis. Previously, many of these patients were misdiagnosed and thought to have clinical features of systemic polyarteritis nodosum, which negatively influenced its outcome, since TNF inhibitors seem to have efficacy on the vasculitic phenotype of DADA2. We present a case of a 28-year-old woman with a lifelong unknown syndrome and unique clinical manifestations recently recognized as DADA2. The first manifestation, at 3 months of age, was an episode of facial paralysis during which renovascular hypertension was diagnosed. Later, she developed episodes of prolonged fever, polyarthritis, Raynaud's phenomenon, gastrointestinal bleeding, and intracerebral hemorrhage. This inflammatory state ultimately led to the development of amyloid A amyloidosis and renal insufficiency. CI - Copyright © 2021 by S. Karger AG, Basel. FAU - Pardinhas, Clara AU - Pardinhas C AD - Neurology Department, Coimbra University Hospital Center, Coimbra, Portugal. FAU - Santo, Gustavo AU - Santo G AD - Neurology Department, Coimbra University Hospital Center, Coimbra, Portugal. AD - Center of Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal. FAU - Escada, Luís AU - Escada L AD - Neurology Department, Coimbra University Hospital Center, Coimbra, Portugal. FAU - Rodrigues, Jorge AU - Rodrigues J AD - Pediatric Rheumatology Unit, Coimbra University Hospital Center, Coimbra, Portugal. FAU - Almeida, Maria Rosário AU - Almeida MR AD - Center of Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal. FAU - Alves, Rui AU - Alves R AD - Neurology Department, Coimbra University Hospital Center, Coimbra, Portugal. AD - Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal. FAU - Salgado, Manuel AU - Salgado M AD - Pediatric Rheumatology Unit, Coimbra University Hospital Center, Coimbra, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20211118 PL - Switzerland TA - Case Rep Nephrol Dial JT - Case reports in nephrology and dialysis JID - 101636294 PMC - PMC8739640 OTO - NOTNLM OT - Adenosine deaminase 2 deficiency OT - Amyloid A amyloidosis OT - Autoinflammatory disease OT - Kidney injury OT - Vasculitis COIS- The authors have no conflicts of interest to declare. EDAT- 2022/01/28 06:00 MHDA- 2022/01/28 06:01 PMCR- 2021/11/18 CRDT- 2022/01/27 05:50 PHST- 2021/02/16 00:00 [received] PHST- 2021/05/07 00:00 [accepted] PHST- 2022/01/27 05:50 [entrez] PHST- 2022/01/28 06:00 [pubmed] PHST- 2022/01/28 06:01 [medline] PHST- 2021/11/18 00:00 [pmc-release] AID - cnd-0011-0340 [pii] AID - 10.1159/000517141 [doi] PST - epublish SO - Case Rep Nephrol Dial. 2021 Nov 18;11(3):340-347. doi: 10.1159/000517141. eCollection 2021 Sep-Dec. PMID- 25161760 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140827 LR - 20211021 IS - 1941-2789 (Print) IS - 1941-2789 (Linking) VI - 7 IP - 8 DP - 2014 Aug TI - Postural orthostatic tachycardia syndrome: a dermatologic perspective and successful treatment with losartan. PG - 41-7 AB - The postural orthostatic tachycardia syndrome is a disease characterized by excessively increased heart rate during orthostatic challenge associated with symptoms of orthostatic intolerance including dizziness, exercise intolerance, headache, fatigue, memory problems, nausea, blurred vision, pallor, and sweating, which improve with recumbence. Postural orthostatic tachycardia syndrome patients may present with a multitude of additional symptoms that are attributable to vascular vasoconstriction. Observed signs and symptoms in a patient with postural orthostatic tachycardia syndrome include tachycardia at rest, exaggerated heart rate increase with upright position and exercise, crushing chest pain, tremor, syncope, loss of vision, confusion, migraines, fatigue, heat intolerance, parasthesia, dysesthesia, allodynia, altered traditional senses, and thermoregulatory abnormalities. There are a number of possible dermatological manifestations of postural orthostatic tachycardia syndrome easily explained by its recently discovered pathophysiology. The author reports the case of a 22-year-old woman with moderate-to-severe postural orthostatic tachycardia syndrome with numerous dermatological manifestations attributable to the disease process. The cutaneous manifestations observed in this patient are diverse and most noticeable during postural orthostatic tachycardia syndrome flares. The most distinct are evanescent, hyperemic, sharply demarcated, irregular patches on the chest and neck area that resolve upon diascopy. This distinct "evanescent hyperemia" disappears spontaneously after seconds to minutes and reappears unexpectedly. Other observed dermatological manifestations of this systemic disease include Raynaud's phenomenon, koilonychia, onychodystrophy, madarosis, dysesthesia, allodynia, telogen effluvium, increased capillary refill time, and livedo reticularis. The treatment of this disease poses a great challenge. The author reports the unprecedented use of an oral angiotensin II type 1 receptor antagonist resulting in remarkable improvement. FAU - Landero, James AU - Landero J AD - Abilene, Texas. LA - eng PT - Journal Article PL - United States TA - J Clin Aesthet Dermatol JT - The Journal of clinical and aesthetic dermatology JID - 101518173 PMC - PMC4142820 EDAT- 2014/08/28 06:00 MHDA- 2014/08/28 06:01 PMCR- 2014/08/01 CRDT- 2014/08/28 06:00 PHST- 2014/08/28 06:00 [entrez] PHST- 2014/08/28 06:00 [pubmed] PHST- 2014/08/28 06:01 [medline] PHST- 2014/08/01 00:00 [pmc-release] PST - ppublish SO - J Clin Aesthet Dermatol. 2014 Aug;7(8):41-7. PMID- 40099047 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250319 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 2 DP - 2025 Feb TI - Erasmus Syndrome, An Autoimmunity Paradox: A Case Report and Literature Review. PG - e79036 LID - 10.7759/cureus.79036 [doi] LID - e79036 AB - Erasmus syndrome (ES) is a rare condition characterized by the link between crystalline silica exposure, with or without silicosis, and systemic sclerosis (SSc). Although first noted over a century ago, its underlying mechanisms remain unclear. However, it is indistinguishable from idiopathic SSc in the general population. Its clinical presentation is heterogeneous, depending on the affected systems, with notable features, including skin fibrosis, microstomia, telangiectasia, Raynaud's phenomenon, arthralgia, and interstitial lung disease. Currently, there is no unified consensus on its treatment; however, organ-specific therapy is a reasonable approach. We report the case of a 43-year-old miner diagnosed with diffuse cutaneous SSc, where ES was diagnosed after an exhaustive history was taken, occupational exposure was characterized, differential diagnoses were excluded, and radiological and histopathological evidence of pulmonary silicosis was presented. CI - Copyright © 2025, Arango et al. FAU - Arango, Alejandro AU - Arango A AD - Internal Medicine, Pontifical Bolivarian University, Medellín, COL. FAU - Villa-Pérez, Simon AU - Villa-Pérez S AD - Internal Medicine, Hospital Pablo Tobón Uribe, Medellín, COL. AD - Internal Medicine, Universidad EIA, Envigado, COL. FAU - Garcia Rueda, Jhon Edwar AU - Garcia Rueda JE AD - Internal Medicine, Pontifical Bolivarian University, Medellín, COL. FAU - Cardona Palacio, Alejandro AU - Cardona Palacio A AD - Pathology, Hospital Pablo Tobón Uribe, Medellín, COL. AD - Pathology, Universidad EIA, Envigado, COL. FAU - Benavides, Roberto AU - Benavides R AD - Pulmonology, Universidad CES, Medellín, COL. LA - eng PT - Case Reports PT - Journal Article DEP - 20250215 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11912300 OTO - NOTNLM OT - erasmus syndrome OT - ild interstitial lung disease OT - immunosuppressive agents OT - silicosis OT - systemic sclerosis COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/03/18 06:22 MHDA- 2025/03/18 06:23 PMCR- 2025/02/15 CRDT- 2025/03/18 04:41 PHST- 2025/02/14 00:00 [accepted] PHST- 2025/03/18 06:23 [medline] PHST- 2025/03/18 06:22 [pubmed] PHST- 2025/03/18 04:41 [entrez] PHST- 2025/02/15 00:00 [pmc-release] AID - 10.7759/cureus.79036 [doi] PST - epublish SO - Cureus. 2025 Feb 15;17(2):e79036. doi: 10.7759/cureus.79036. eCollection 2025 Feb. PMID- 24503734 OWN - NLM STAT- MEDLINE DCOM- 20140404 LR - 20140207 IS - 1827-6806 (Print) IS - 1827-6806 (Linking) VI - 15 IP - 1 DP - 2014 Jan TI - [Cardiac involvement at rest in patients with systemic sclerosis: differences between the limited and the diffuse form of the disease]. PG - 44-50 LID - 10.1714/1394.15518 [doi] AB - BACKGROUND: Heart involvement confers a poor prognosis in patients affected by systemic sclerosis (SSc). Nevertheless, the prevalence of heart involvement in these patients is not fully known. In this study we analyzed the most frequent manifestations of heart involvement at rest in a cohort of patients with SSc, comparing their prevalence in patients with the limited form (lcSSc) and in patients with the diffuse form (dcSSc) of the disease, taking also into account the duration of the disease. METHODS: We thoroughly evaluated with electrocardiographic and echocardiographic examinations 174 patients affected by SSc who were followed at our hospital between 2001 and 2011. They were divided according to the disease subtype (dcSSc vs lcSSc) and information about the disease duration was available for 121 of them (calculated from the onset of the Raynaud's phenomenon). RESULTS: Patients with dcSSc had a greater prevalence of heart involvement at rest when evaluated within 5 and 10 years after the onset of the Raynaud's phenomenon (p=0.0051 within 5 years and p=0.035 within 10 years). Indeed, patients with dcSSc had a greater prevalence of atrioventricular conduction abnormalities within 20 years after the onset of the Raynaud's phenomenon (p=0.03 within 10 years and p=0.04 within 20 years) and a greater prevalence of valvular abnormalities within 5 years (p=0.04). In dcSSc there was a greater prevalence of rhythm disturbances in patients with a disease duration ≤20 or >20 years (p=0.04 within 20 years and p=0.04 after 20 years), but not in those with a disease duration ≤5 and ≤10 years. Left ventricular hypertrophy had a greater prevalence in dcSSc after 20 years of disease duration (p=0.02). CONCLUSIONS: Cardiac manifestation occurs earlier and more frequently in patients affected by dcSSc than in patients with lcSSc. FAU - Faccini, Alessia AU - Faccini A FAU - Franchini, Stefano AU - Franchini S FAU - Sabbadini, Maria Grazia AU - Sabbadini MG FAU - Camici, Paolo G AU - Camici PG LA - ita PT - English Abstract PT - Journal Article TT - Coinvolgimento cardiaco a riposo in pazienti affetti da sclerosi sistemica: differenze fra forma diffusa e forma limitata. PL - Italy TA - G Ital Cardiol (Rome) JT - Giornale italiano di cardiologia (2006) JID - 101263411 SB - IM MH - Adult MH - Aged MH - Electrocardiography MH - Female MH - Heart Diseases/epidemiology/*etiology/physiopathology MH - Humans MH - Hypertrophy, Left Ventricular/epidemiology/etiology/physiopathology MH - Male MH - Middle Aged MH - Prevalence MH - Prognosis MH - Retrospective Studies MH - Scleroderma, Diffuse/*complications/physiopathology MH - Scleroderma, Limited/*complications/physiopathology MH - Time Factors EDAT- 2014/02/08 06:00 MHDA- 2014/04/05 06:00 CRDT- 2014/02/08 06:00 PHST- 2014/02/08 06:00 [entrez] PHST- 2014/02/08 06:00 [pubmed] PHST- 2014/04/05 06:00 [medline] AID - 10.1714/1394.15518 [doi] PST - ppublish SO - G Ital Cardiol (Rome). 2014 Jan;15(1):44-50. doi: 10.1714/1394.15518. PMID- 33200774 OWN - NLM STAT- MEDLINE DCOM- 20210705 LR - 20210705 IS - 1930-613X (Electronic) IS - 0026-4075 (Linking) VI - 186 IP - 7-8 DP - 2021 Jul 1 TI - Interstitial Lung Disease and Myositis in a Patient With Antisynthetase Syndrome and PL12 and Ro52 Co-positivity in a Retired Medical Officer. PG - e836-e839 LID - 10.1093/milmed/usaa412 [doi] AB - Antisynthetase syndrome (ASS) is an idiopathic inflammatory myopathy characterized by myositis, arthritis, interstitial lung disease (ILD), Raynaud's phenomenon, and distinctive cutaneous manifestations. Anti-PL12 is a rare myositis-specific autoantibody classically associated with an amyopathic presentation and rapidly progressive ILD. Anti-Ro52 is a myositis-associated antibody that has been postulated to be directly pathogenic in inflammatory myopathy patients. The disease phenotype, course, and response to treatment associated with anti-PL12 and anti-Ro52 co-positivity is not well described. A 58-year-old man with anti-PL12 and anti-Ro52 ASS presented with rapidly progressive ILD and myositis refractory to high-dose prednisone. He ultimately required a dexamethasone burst with intravenous immunoglobulin and mycophenolate mofetil for disease control. Severe and rapidly progressive myositis is infrequently reported in anti-PL12 ASS. This case suggests that concurrent anti-Ro52 positivity predicts a more aggressive disease phenotype and may require more initial immunosuppression. If rapid progression of this disease were to occur in an active duty service member, it would have significant implications for readiness and potentially catastrophic outcomes in the deployed setting. Early identification and treatment of the disease are imperative. The question must also be raised of an occupational exposure from military service. CI - Published by Oxford University Press on behalf of the Association of Military Surgeons of the United States 2020. This work is written by (a) US Government employee(s) and is in the public domain in the US. FAU - Loncharich, Michael F AU - Loncharich MF AD - Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. AD - Rheumatology Service, Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. FAU - Anderson, Caleb W AU - Anderson CW AD - Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. AD - Rheumatology Service, Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. FAU - Collins, Jeannette AU - Collins J AD - Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. AD - Pulmonology and Critical Care, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. FAU - Edison, Jess AU - Edison J AD - Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. AD - Rheumatology Service, Department of Medicine, Walter Reed National Military Medical Center, Bethesda, MD 20889, USA. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mil Med JT - Military medicine JID - 2984771R RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies MH - Humans MH - *Lung Diseases, Interstitial MH - Male MH - Middle Aged MH - *Myositis MH - Retrospective Studies EDAT- 2020/11/18 06:00 MHDA- 2021/07/06 06:00 CRDT- 2020/11/17 08:41 PHST- 2020/05/05 00:00 [received] PHST- 2020/07/13 00:00 [revised] PHST- 2020/09/26 00:00 [accepted] PHST- 2020/11/18 06:00 [pubmed] PHST- 2021/07/06 06:00 [medline] PHST- 2020/11/17 08:41 [entrez] AID - 5985307 [pii] AID - 10.1093/milmed/usaa412 [doi] PST - ppublish SO - Mil Med. 2021 Jul 1;186(7-8):e836-e839. doi: 10.1093/milmed/usaa412. PMID- 21960047 OWN - NLM STAT- MEDLINE DCOM- 20130220 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 10 DP - 2012 Oct TI - Evaluation of skin thickness lesions in patients with Lyme disease measured by modified Rodnan total skin score. PG - 3189-91 AB - Recently, a possible etiological connection between infection with Borrelia burgdorferi and various skin lesions, including morphea and systemic sclerosis (SSc), has been discussed. The aim of our study was the evaluation of frequency of skin thickening typical of SSc or morphea in the group of patients with Lyme disease (LD) with frequent exposition to tick bites. The group consisted of 110 patients with LD frequently exposed to tick bites form the northeastern Poland, which is an endemic area for this disease. To measure the skin lesions, the modified Rodnan total skin score (RTSS) was used. In the analyzed group, no skin changes typical of morphea or skin thickening were found. According to RTSS, all patients scored 0 points. Raynaud's phenomenon in all patients was not found. The relationship between scleroderma or morphea and LD is still a matter of controversy. Described by some authors, cases with LD and scleroderma may be associated with co-existence of B. burgdorferi infection with autoimmune process. FAU - Moniuszko, A AU - Moniuszko A AD - Department of Infectious Diseases and Neuroinfections, Medical University in Białystok, Żurawia Street 14, 15-540 Bialystok, Poland. annamoniuszko@op.pl FAU - Gińdzieńska-Sieśkiewicz, E AU - Gińdzieńska-Sieśkiewicz E FAU - Pancewicz, S A AU - Pancewicz SA FAU - Czupryna, P AU - Czupryna P FAU - Zajkowska, J AU - Zajkowska J FAU - Sierakowski, S AU - Sierakowski S LA - eng PT - Journal Article DEP - 20110930 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Borrelia burgdorferi/immunology MH - Female MH - Humans MH - Lyme Disease/*complications/immunology/microbiology/pathology MH - Male MH - Middle Aged MH - Scleroderma, Localized/immunology/microbiology/*pathology MH - Scleroderma, Systemic/immunology/microbiology/*pathology MH - Severity of Illness Index MH - Skin/immunology/microbiology/*pathology EDAT- 2011/10/01 06:00 MHDA- 2013/02/21 06:00 CRDT- 2011/10/01 06:00 PHST- 2011/05/19 00:00 [received] PHST- 2011/09/10 00:00 [accepted] PHST- 2011/10/01 06:00 [entrez] PHST- 2011/10/01 06:00 [pubmed] PHST- 2013/02/21 06:00 [medline] AID - 10.1007/s00296-011-2157-7 [doi] PST - ppublish SO - Rheumatol Int. 2012 Oct;32(10):3189-91. doi: 10.1007/s00296-011-2157-7. Epub 2011 Sep 30. PMID- 41940214 OWN - NLM STAT- Publisher LR - 20260418 IS - 1753-495X (Print) IS - 1753-4968 (Electronic) IS - 1753-495X (Linking) DP - 2026 Apr 1 TI - Maternal and fetal outcomes in pregnancies affected by mixed connective tissue disease. PG - 1753495X261427244 LID - 10.1177/1753495X261427244 [doi] LID - 1753495X261427244 AB - INTRODUCTION: Mixed connective tissue disease (MCTD) is an autoimmune condition with overlapping features of lupus, systemic sclerosis, and polymyositis. Maternal-fetal outcomes specific to MCTD remain poorly characterized; a scoping review was conducted. METHODS: Five databases were queried. Extracted data included demographics, antibody profiles, symptoms, treatments, and maternal-fetal outcomes. Descriptive analysis was performed. RESULTS: Of 269 articles, 33 met inclusion criteria, including 375 pregnancies. Common symptoms and disease manifestations included Raynaud's phenomenon, arthralgias, and sclerodactyly. Treatments included hydroxychloroquine, azathioprine, and corticosteroids. Maternal complications included interstitial lung disease (21.4%), gestational hypertension (HTN) (15.6%), chronic HTN (7.0%), pulmonary HTN (9.8%), eclampsia (1.4%), miscarriage (20.3%), preterm birth (19.6%), and stillbirth (8.1%). Fetal complications included chondrodysplasia punctata (18.2%), demise (6.5%), neonatal lupus erythematosus (3.3%), and congenital heart block (1.0%). CONCLUSION: While most MCTD pregnancies resulted in live birth, they carry elevated maternal and perinatal risks that warrant heightened surveillance and tailored management. CI - © The Author(s) 2026. FAU - Zhou, Shannon Y AU - Zhou SY AUID- ORCID: 0000-0003-0068-6578 AD - Department of Obstetrics and Gynecology, University of Minnesota Medical School, Minneapolis, MN, USA. RINGGOLD: 12269 FAU - Holden, Lily AU - Holden L AD - Department of Obstetrics and Gynecology, University of Minnesota Medical School, Minneapolis, MN, USA. RINGGOLD: 12269 FAU - Colon, Melody AU - Colon M AD - University of Minnesota, Minneapolis, MN, USA. RINGGOLD: 5635 FAU - Cregan, Alexandra AU - Cregan A AUID- ORCID: 0009-0008-7496-0465 AD - Department of Obstetrics and Gynecology, University of Minnesota Medical School, Minneapolis, MN, USA. RINGGOLD: 12269 FAU - Burn, Sabrina C AU - Burn SC AUID- ORCID: 0000-0001-9419-2944 AD - Department of Obstetrics and Gynecology, University of Minnesota Medical School, Minneapolis, MN, USA. RINGGOLD: 12269 LA - eng PT - Journal Article PT - Review DEP - 20260401 PL - England TA - Obstet Med JT - Obstetric medicine JID - 101464191 PMC - PMC13043632 OTO - NOTNLM OT - Mixed connective tissue disease OT - autoimmune disease OT - hypertensive disorders OT - neonatal lupus OT - pregnancy complications COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2026/04/06 12:36 MHDA- 2026/04/06 12:36 PMCR- 2027/04/01 CRDT- 2026/04/06 06:24 PHST- 2025/09/28 00:00 [received] PHST- 2026/02/07 00:00 [accepted] PHST- 2027/04/01 00:00 [pmc-release] PHST- 2026/04/06 12:36 [medline] PHST- 2026/04/06 12:36 [pubmed] PHST- 2026/04/06 06:24 [entrez] AID - 10.1177_1753495X261427244 [pii] AID - 10.1177/1753495X261427244 [doi] PST - aheadofprint SO - Obstet Med. 2026 Apr 1:1753495X261427244. doi: 10.1177/1753495X261427244. PMID- 39726702 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250104 IS - 2234-943X (Print) IS - 2234-943X (Electronic) IS - 2234-943X (Linking) VI - 14 DP - 2024 TI - Postoperative unexplained sigmoid stenosis in a patient with rectal cancer complicated with connective tissue disease: a case report and literature review. PG - 1406098 LID - 10.3389/fonc.2024.1406098 [doi] LID - 1406098 AB - It is well established that host immunity plays a critical role in defending against colorectal cancer (CRC) progression. Connective tissue disease (CTD) encompasses a group of heterogeneous, immune-mediated disorders that present with diverse and often non-specific initial symptoms. Raynaud's phenomenon is a common feature, complicating early diagnosis. As CTD progresses, it can damage the skin, muscles, and blood vessels and may extend to the lungs, heart, kidneys, and other abdominal organs. Several studies have reported that CTD can lead to intestinal vascular occlusion and related inflammation, but the occurrence of related complications after intestinal surgery has been reported rarely. In this study, an elderly female patient with rectal cancer complicated with CTD was found to have unexplained proximal anastomotic stenosis during an attempt at fistula restoration 3 months after laparoscopy-assisted transanal total mesorectal excision (TaTME) and preventive terminal ileostomy, resulting in fistula failure. This case study aims to serve as a reference for clinicians in their future practice. CI - Copyright © 2024 Tong, Li, Gao and Zhang. FAU - Tong, Deming AU - Tong D AD - Department of General Surgery, General Hospital of Northern Theater Command, Shenyang, China. FAU - Li, Jian AU - Li J AD - Department of General Surgery, General Hospital of Northern Theater Command, Shenyang, China. FAU - Gao, Guangrong AU - Gao G AD - Department of General Surgery, General Hospital of Northern Theater Command, Shenyang, China. FAU - Zhang, Cheng AU - Zhang C AD - Department of General Surgery, General Hospital of Northern Theater Command, Shenyang, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20241211 PL - Switzerland TA - Front Oncol JT - Frontiers in oncology JID - 101568867 PMC - PMC11670202 OTO - NOTNLM OT - CTD (connective tissue disease) OT - TaTME OT - anastomotic stenosis OT - rectal cancer (RC) OT - temporary ileostomy COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2024/12/27 06:19 MHDA- 2024/12/27 06:20 PMCR- 2024/01/01 CRDT- 2024/12/27 03:57 PHST- 2024/03/24 00:00 [received] PHST- 2024/11/18 00:00 [accepted] PHST- 2024/12/27 06:20 [medline] PHST- 2024/12/27 06:19 [pubmed] PHST- 2024/12/27 03:57 [entrez] PHST- 2024/01/01 00:00 [pmc-release] AID - 10.3389/fonc.2024.1406098 [doi] PST - epublish SO - Front Oncol. 2024 Dec 11;14:1406098. doi: 10.3389/fonc.2024.1406098. eCollection 2024. PMID- 34131653 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230506 IS - 2732-432X (Electronic) IS - 2732-432X (Linking) VI - 10 DP - 2021 TI - Emerging treatments for scleroderma/systemic sclerosis. PG - 43 LID - 10.12703/r/10-43 [doi] LID - 43 AB - Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae. Management of SSc cutaneous disease remains challenging and often is driven by extracutaneous manifestations. Methotrexate is the typical first-line therapy for patients with early progressive cutaneous disease. However, in patients with diffuse progressive skin disease and inflammatory arthritis, methotrexate or rituximab monotherapy should be considered. First-line therapy for patients with concomitant myositis includes methotrexate or intravenous immunoglobulin (IVIG). For patients with both cutaneous findings and interstitial lung disease, studies have suggested the efficacy of mycophenolate mofetil or rituximab. Second-line therapies, including UVA-1 phototherapy, IVIG, or rituximab, can be considered in patients with disease refractory to first-line treatments. Clinical trials investigating the utility of emerging therapies such as abatacept and tocilizumab in the treatment of SSc are under way, and preliminary results are promising. Nonetheless, all patients with SSc benefit from a gentle skin-care regimen to alleviate pruritis, which is a commonly reported symptom. Additional cutaneous manifestations of SSc include telangiectasias, calcinosis cutis, microstomia, and Raynaud's phenomenon. Telangiectasia may be managed with camouflage techniques, pulse dye laser, and intense pulse light. Calcinosis cutis therapy is guided by the size of the calcium deposits, although treatment options are limited. Mouth augmentation and oral stretching exercises are recommended for patients with reduced oral aperture. Raynaud's phenomenon is treated with a combination of lifestyle modification and calcium channel blockers, such as amlodipine. Overall, SSc is a clinically heterogenous disease that affects multiple organ systems. Providers should assess extracutaneous involvement and use evidence-based recommendations to select the most appropriate therapy for patients with SSc. CI - Copyright: © 2021 Jacobe HT et al. FAU - Zhu, Jane L AU - Zhu JL AD - Department of Dermatology, University of Texas at Southwestern Medical Center, Dallas, TX, USA. FAU - Black, Samantha M AU - Black SM AD - Department of Dermatology, University of Texas at Southwestern Medical Center, Dallas, TX, USA. FAU - Chen, Henry W AU - Chen HW AD - Department of Dermatology, University of Texas at Southwestern Medical Center, Dallas, TX, USA. FAU - Jacobe, Heidi T AU - Jacobe HT AD - Department of Dermatology, University of Texas at Southwestern Medical Center, Dallas, TX, USA. LA - eng GR - R01 AR073516/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Review DEP - 20210505 PL - England TA - Fac Rev JT - Faculty reviews JID - 101769226 PMC - PMC8170563 OTO - NOTNLM OT - Scleroderma OT - Systemic Sclerosis COIS- The authors declare that they have no competing interests.No competing interests were disclosed.No competing interests were disclosed. EDAT- 2021/06/17 06:00 MHDA- 2021/06/17 06:01 PMCR- 2021/05/05 CRDT- 2021/06/16 06:51 PHST- 2021/06/16 06:51 [entrez] PHST- 2021/06/17 06:00 [pubmed] PHST- 2021/06/17 06:01 [medline] PHST- 2021/05/05 00:00 [pmc-release] AID - 10.12703/r/10-43 [doi] PST - epublish SO - Fac Rev. 2021 May 5;10:43. doi: 10.12703/r/10-43. eCollection 2021. PMID- 36042067 OWN - NLM STAT- MEDLINE DCOM- 20230110 LR - 20260127 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 42 IP - 1 DP - 2023 Jan TI - Fingerprint comparison between before disease onset and after systemic sclerosis diagnosis: a monocentric cross-sectional study. PG - 117-124 LID - 10.1007/s10067-022-06353-2 [doi] AB - BACKGROUND: Skin tightness is a hallmark of systemic sclerosis (SSc), and the fingers are an affected body part, so much so that fingerprints can be significantly affected among those with extensive skin tightness of the finger. OBJECTIVE: We aimed to compare the difference between the current and past (pre-disease onset) fingerprints of SSc patients. METHODS: We conducted a cross-sectional study among adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between October 2019, and September 2020. All eligible patients consented to obtaining their current and previous prints from the Central Registration Bureau, Department of Provincial Administration, Ministry of the Interior. The current prints were obtained using the Crossmatch (Lite UE) live scan from the Central Institute of Forensic Science of Thailand. We investigated the concordance between the prints before disease onset and the current (enroll date) via the Printquest AFIS system with officers from the Central Institute of Forensic Science, Thailand. RESULTS: One hundred SSc patients, according to the sample size calculation, were enrolled (mean age 59.4 ± 9.6 years; 66% female). Most (70%) had the diffuse cutaneous SSc subset. A respective 59%, 55%, and 6% presented acro-osteolysis, hand deformities, and digital ulcers. Some challenges were experienced in obtaining prints from patients with loss of the fingertip fat pad, finger joint contracture, and/or acro-osteolysis; notwithstanding, all fingerprints were usable and without individualized changes. CONCLUSION: Fingerprints were affected by fingertip lesions and finger joint contractures; notwithstanding, the prints remained usable for personal identification. Key Points • Skin involvement in systemic sclerosis (SSc) affects the prints, particularly at the fingertip. • Despite disease onset, the fingerprints of SSc patients do not change significantly. • Fingerprints are inadequate for personal identification among SSc patients with hand deformities due to poor quality or difficulty acquiring them. CI - © 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Sriwong, Warut T AU - Sriwong WT AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Srisangwarn, Pattayarat AU - Srisangwarn P AD - The Identification of Fingerprints and Palm Automation, Central Institute of Forensic Science, Bangkok, Thailand. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AUID- ORCID: 0000-0002-1964-4389 AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. fching@kku.ac.th. LA - eng PT - Journal Article DEP - 20220831 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - digital ulcers SB - IM MH - Adult MH - Humans MH - Female MH - Middle Aged MH - Aged MH - Male MH - Cross-Sectional Studies MH - Thailand MH - *Scleroderma, Systemic/complications/diagnosis MH - *Contracture MH - *Acro-Osteolysis MH - Skin Ulcer OTO - NOTNLM OT - Clinical trial and method OT - Hand OT - Raynaud’s syndrome OT - Scleroderma and related disorders OT - Skin EDAT- 2022/08/31 06:00 MHDA- 2023/01/11 06:00 CRDT- 2022/08/30 23:15 PHST- 2022/07/13 00:00 [received] PHST- 2022/08/20 00:00 [accepted] PHST- 2022/08/14 00:00 [revised] PHST- 2022/08/31 06:00 [pubmed] PHST- 2023/01/11 06:00 [medline] PHST- 2022/08/30 23:15 [entrez] AID - 10.1007/s10067-022-06353-2 [pii] AID - 10.1007/s10067-022-06353-2 [doi] PST - ppublish SO - Clin Rheumatol. 2023 Jan;42(1):117-124. doi: 10.1007/s10067-022-06353-2. Epub 2022 Aug 31. PMID- 30632525 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220331 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 33 IP - 3 DP - 2018 Sep TI - Juvenile Scleroderma: A Referral Center Experience. PG - 344-351 LID - 10.5606/ArchRheumatol.2018.6578 [doi] AB - OBJECTIVES: This study aims to evaluate the demographic and clinical features, laboratory data, treatment modalities, and outcomes of juvenile systemic sclerosis (JSS) and juvenile localized scleroderma (JLS) patients at a referral pediatric rheumatology center in Turkey. PATIENTS AND METHODS: Medical records of a total of 57 patients, including 29 with JSS (1 male, 28 females; mean age 18.3±3.2 years; range 14 to 27 years) and 28 with JLS (6 males, 22 females; mean age 14.4±4.8 years; range 6 to 23 years), diagnosed betweenJanuary 2006 and Mart 2015 and followed-up for at least six months were evaluated in this retrospective longitudinal study. All medical records were retrospectively analyzed for demographic, clinical, and laboratory findings. RESULTS: Mean age at disease onset was 9.9±4.2 years and 7.7±3.9 years for JSS and JLS, respectively. Mean ages at diagnosis and at the time of study were lower in JLS: 9.1±3.5 years vs. 11.7±3.7 years and 14.4±4.8 years vs. 18.3±3.2 years, respectively. Mean disease duration was 7.8±5.2 years and 8.0±4.3 years for JSS and JLS, respectively. Among JSS patients, interstitial lung disease was seen in eight (27%), pulmonary hypertension in three (10%), and arrhythmia in one (3%). One JSS patient (3%) died as a consequence of cardiac sclerosis. Corticosteroids with methotrexate were used in 29 JSS patients (100%) and in 21 JLS patients (75%). Patients with vasculopathy were treated with nifedipine (n=18, 62%) and bosentan (n=12, 41%). Internal organ involvement was treated with high-dose cyclophosphamide (n=10, 34%) or biological agent (n=3, 10%). CONCLUSION: Close monitoring of internal organ involvement is of great importance in preventing disease-related complications in JSS and JLS. Although rare, vital organ involvement has a devastating effect on prognosis. Biological agents represent an option for patients resistant to standard immunosuppressive treatment. FAU - Adrovic, Amra AU - Adrovic A AD - Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey. FAU - Şahin, Sezgin AU - Şahin S AD - Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey. FAU - Barut, Kenan AU - Barut K AD - Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey. FAU - Kasapçopur, Özgür AU - Kasapçopur Ö AD - Department of Pediatric Rheumatology, İstanbul University, Cerrahpasa Medical School, İstanbul, Turkey. LA - eng PT - Journal Article DEP - 20180118 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC6328218 OTO - NOTNLM OT - Juvenile localized scleroderma OT - Raynaud's phenomenon OT - juvenile systemic sclerosis OT - skin stiffening COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2019/01/12 06:00 MHDA- 2019/01/12 06:01 PMCR- 2018/01/18 CRDT- 2019/01/12 06:00 PHST- 2017/07/13 00:00 [received] PHST- 2017/12/13 00:00 [accepted] PHST- 2019/01/12 06:00 [entrez] PHST- 2019/01/12 06:00 [pubmed] PHST- 2019/01/12 06:01 [medline] PHST- 2018/01/18 00:00 [pmc-release] AID - 10.5606/ArchRheumatol.2018.6578 [doi] PST - epublish SO - Arch Rheumatol. 2018 Jan 18;33(3):344-351. doi: 10.5606/ArchRheumatol.2018.6578. eCollection 2018 Sep. PMID- 26933723 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160321 LR - 20191027 IS - 0256-9574 (Print) VI - 105 IP - 12 DP - 2015 Dec TI - Meeting the challenges in the diagnosis of inflammatory myopathies. PG - 1076 AB - Inflammatory myopathy (IM) is a rubric term to describe a heterogeneous group of muscle diseases typified by dermatomyositis and polymyositis. The current classifications are unsatisfactory, but IM associated with other connective tissue diseases (CTDs), such as systemic lupus erythematosus, underlying malignancy and HIV, should also be included. Although uncommon, IM should always be considered in a patient who presents with proximal weakness of gradual onset and has raised serum muscle enzymes. The diagnosis may be obvious if the patient has diagnostic skin signs such as heliotropic rash (peri-orbital discoloration) and Gottron’s lesions (typically on the extensor surfaces of the fingers). In the absence of obvious skin manifestations, other features of a CTD such as Raynaud’s phenomenon, abnormal capilloroscopy and the presence of serum antinuclear factor antibody should be searched for. Conditions that mimic IM include other causes of myopathy such as endocrine disorders, adverse effects of medication, metabolic myopathies and muscle dystrophies. Atypical features suggesting an alternative diagnosis are acute onset, severe pain, asymmetrical involvement, distal weakness and wasting. Appropriate investigations include a chest radiograph indicating interstitial lung disease or malignancy. Electromyography and muscle biopsy are useful in cases where other diagnoses are suspected. FAU - Manie, M AU - Manie M LA - eng PT - Journal Article PL - South Africa TA - S Afr Med J JT - South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde JID - 0404520 EDAT- 2016/03/05 06:00 MHDA- 2016/03/05 06:01 CRDT- 2016/03/03 06:00 PHST- 2016/03/03 06:00 [entrez] PHST- 2016/03/05 06:00 [pubmed] PHST- 2016/03/05 06:01 [medline] AID - 10.7196/samj.2015.v105i12.10226 [doi] PST - ppublish SO - S Afr Med J. 2015 Dec;105(12):1076. doi: 10.7196/samj.2015.v105i12.10226. PMID- 19348178 OWN - NLM STAT- MEDLINE DCOM- 20090519 LR - 20190917 IS - 0009-918X (Print) IS - 0009-918X (Linking) VI - 49 IP - 2-3 DP - 2009 Feb-Mar TI - [Elevated serum aldolase activity in a patient of non-eosinophilic myofasciitis and synovitis with perifascicular atrophy]. PG - 119-22 AB - A 35-year-old man suffered from myalgia and joint pain on walking for 5 months. Physical and neurological examinations revealed dermal sclerosis, skin swelling, redness of forearms, Raynaud's phenomenon, joint pain, myalgia and muscle weakness. Eosinophilia was not found and serum creatine kinase activity was normal, while aldolase was markedly elevated. Abnormal signals suggesting synovitis and myofasciitis were found on MRI images. Biopsy of the fascia of quadriceps femoris showed perivascular mononuclear cell infiltration. A muscle biopsy showed mononuclear cell infiltration mainly in the perimysium extending to the endmysium. Eosinophilic cells were not found, Perifascicular atrophy was observed. Corticosteroid therapy improved clinical symptoms and serum aldolase level. We diagnosed him as non-eosinophilic myofasciitis and synovitis with perifascicular atrophy. The serum aldolase activity is usuful for diagnosis and for monitoring the disease activity. FAU - Ono, Hirohiko AU - Ono H AD - Department of Neurology, Tohoku University School of Medicine. FAU - Suzuki, Naoki AU - Suzuki N FAU - Mizuno, Hideki AU - Mizuno H FAU - Tateyama, Maki AU - Tateyama M FAU - Aoki, Masashi AU - Aoki M FAU - Itoyama, Yasuto AU - Itoyama Y LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 RN - EC 4.1.2.13 (Fructose-Bisphosphate Aldolase) SB - IM MH - Adult MH - Fasciitis/*enzymology/pathology MH - Fructose-Bisphosphate Aldolase/*blood MH - Humans MH - Male MH - Myositis/*enzymology/pathology MH - Synovitis/enzymology/*pathology EDAT- 2009/04/08 09:00 MHDA- 2009/05/20 09:00 CRDT- 2009/04/08 09:00 PHST- 2009/04/08 09:00 [entrez] PHST- 2009/04/08 09:00 [pubmed] PHST- 2009/05/20 09:00 [medline] AID - 10.5692/clinicalneurol.49.119 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2009 Feb-Mar;49(2-3):119-22. doi: 10.5692/clinicalneurol.49.119. PMID- 18311040 OWN - NLM STAT- MEDLINE DCOM- 20080422 LR - 20191110 IS - 0911-4300 (Print) IS - 0911-4300 (Linking) VI - 31 IP - 1 DP - 2008 Feb TI - The roles of chemokines in the development of systemic sclerosis. PG - 23-36 AB - Systemic sclerosis (SSc, scleroderma) is an autoimmune disease characterized by excessive extracellular matrix deposition and vascular injury in the skin and internal organs. Although the pathogenesis remains unclear, Raynaud's phenomenon, a kind of ischemia-reperfusion, usually precedes the development of skin sclerosis. Therefore, it is possible that endothelial cell injury caused by recurring ischemia-reperfusion induces inflammatory cell infiltration and subsequent cytokine production, leading to the development of tissue fibrosis. During this process, chemokines likely have important roles via mediating chemotaxis and activation of leukocytes, result in the interaction between leukocytes and fibroblasts. While chemokine abnormalities of SSc have been reported in amounts of literatures, monocyte chemoattractant protein-1 (MCP-1/CCL2) and its receptor, CCR2, likely have the most critical role for the development of SSc. Here recent data will be reviewed on the potential role of chemokines and their receptors in SSc. FAU - Hasegawa, Minoru AU - Hasegawa M AD - Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa, Japan. LA - eng PT - Journal Article PT - Review PL - Japan TA - Nihon Rinsho Meneki Gakkai Kaishi JT - Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology JID - 9505992 RN - 0 (Chemokines) SB - IM MH - Animals MH - Chemokines/*physiology MH - Humans MH - Scleroderma, Systemic/*physiopathology RF - 130 EDAT- 2008/03/04 09:00 MHDA- 2008/04/23 09:00 CRDT- 2008/03/04 09:00 PHST- 2008/03/04 09:00 [pubmed] PHST- 2008/04/23 09:00 [medline] PHST- 2008/03/04 09:00 [entrez] AID - JST.JSTAGE/jsci/31.23 [pii] AID - 10.2177/jsci.31.23 [doi] PST - ppublish SO - Nihon Rinsho Meneki Gakkai Kaishi. 2008 Feb;31(1):23-36. doi: 10.2177/jsci.31.23. PMID- 35387217 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221003 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 6 IP - 3 DP - 2021 Oct TI - Ascending aorta aneurysm in scleroderma. PG - 322-326 LID - 10.1177/23971983211023322 [doi] AB - Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. She had other features like arthritis, Raynaud's phenomena, dyspnoea, heaviness of chest, and dysphagia. On investigation, she was strongly positive for antinuclear antibody and Scl 70. Imaging revealed interstitial lung disease (nonspecific interstitial pneumonia pattern) and a fusiform dilatation of ascending aorta of 6.5 cm. Patient was offered surgical correction for aneurysm, for which she refused. To the best of our knowledge, our case report adds up to the few cases of ascending aorta aneurysm in scleroderma available in world literature. CI - © The Author(s) 2021. FAU - Chintala, Bhavya AU - Chintala B AUID- ORCID: 0000-0002-0014-4325 AD - Department of Rheumatology and clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India. FAU - Duggal, Lalit AU - Duggal L AD - Department of Rheumatology and clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India. FAU - Jain, Neeraj AU - Jain N AD - Department of Rheumatology and clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20210621 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922660 OTO - NOTNLM OT - Ascending aorta OT - aneurysm OT - scleroderma COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2022/04/08 06:00 MHDA- 2022/04/08 06:01 PMCR- 2022/10/01 CRDT- 2022/04/07 05:22 PHST- 2021/01/28 00:00 [received] PHST- 2021/05/16 00:00 [accepted] PHST- 2022/04/07 05:22 [entrez] PHST- 2022/04/08 06:00 [pubmed] PHST- 2022/04/08 06:01 [medline] PHST- 2022/10/01 00:00 [pmc-release] AID - 10.1177_23971983211023322 [pii] AID - 10.1177/23971983211023322 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2021 Oct;6(3):322-326. doi: 10.1177/23971983211023322. Epub 2021 Jun 21. PMID- 24722688 OWN - NLM STAT- MEDLINE DCOM- 20150224 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 33 IP - 7 DP - 2014 Jul TI - Rituximab for refractory digital infarcts and ulcers in systemic sclerosis. PG - 1019-20 LID - 10.1007/s10067-014-2579-1 [doi] AB - Systemic sclerosis is an uncommon connective tissue disease characterised by excessive deposition of collagen and vasculopathy which affects the skin and multiple internal organs. It is associated with autoimmunity, inflammation, vasculopathy and fibrosis. Managing Raynaud's phenonemon, digital infarcts and ulcers in systemic sclerosis patients is often a challenge particularly among rheumatologists. We report a case of systemic sclerosis patient with refractory digital infarcts and ulcers responded successfully with rituximab. FAU - Khor, Chiew-Gek AU - Khor CG AD - Rheumatology Unit, Department of Internal Medicine, Pulau Pinang General Hospital, Georgetown, Malaysia. FAU - Chen, Xena Lung-Fang AU - Chen XL FAU - Lin, Ting-Syuan AU - Lin TS FAU - Lu, Cheng-Hsun AU - Lu CH FAU - Hsieh, Song-Chou AU - Hsieh SC LA - eng PT - Case Reports PT - Journal Article DEP - 20140412 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 4F4X42SYQ6 (Rituximab) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Adult MH - Antibodies, Monoclonal, Murine-Derived/*therapeutic use MH - Autoimmunity MH - Blood Sedimentation MH - C-Reactive Protein/metabolism MH - Fingers/pathology MH - Humans MH - Inflammation MH - Male MH - Rituximab MH - Scleroderma, Systemic/complications/*drug therapy MH - Skin/pathology MH - Skin Ulcer/complications/*drug therapy MH - Treatment Outcome EDAT- 2014/04/12 06:00 MHDA- 2015/02/25 06:00 CRDT- 2014/04/12 06:00 PHST- 2014/02/13 00:00 [received] PHST- 2014/03/10 00:00 [accepted] PHST- 2014/03/07 00:00 [revised] PHST- 2014/04/12 06:00 [entrez] PHST- 2014/04/12 06:00 [pubmed] PHST- 2015/02/25 06:00 [medline] AID - 10.1007/s10067-014-2579-1 [doi] PST - ppublish SO - Clin Rheumatol. 2014 Jul;33(7):1019-20. doi: 10.1007/s10067-014-2579-1. Epub 2014 Apr 12. PMID- 38907515 OWN - NLM STAT- MEDLINE DCOM- 20240622 LR - 20240926 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 25 DP - 2024 Jun 22 TI - Blue Toe Syndrome in Behçet's Disease: A Case Report. PG - e943813 LID - 10.12659/AJCR.943813 [doi] AB - BACKGROUND Vascular Behçet's disease (VBD) is a rare but potentially life-threatening subtype of Behçet's disease that is characterized by multisystemic vasculitis. It primarily affects males with ancestry traced back to regions along the ancient Silk Road. Both arteries and veins, regardless of size, may exhibit complications, including aneurysmal degeneration or occlusion. While venous involvement is observed in two-thirds of VBD cases, arterial complications are notably the most severe and lethal. Arterial aneurysmal degeneration is more common than occlusive complications, with larger arteries being predominantly affected in VBD. Data regarding isolated small-vessel arterial occlusive disease in VBD are limited. Given the rarity of this presentation in this patient population, it becomes mandatory to thoroughly evaluate such patients to differentiate small-vessel vasculitis from other similar diseases, such as Raynaud's phenomenon, which has a different etiology and management and generally has a more benign course. Here, we delineate the concept of isolated small-vessel vasculitis as a cause of blue toe syndrome in patients with VBD. CASE REPORT This report describes a distinctive case of vascular Behçet's disease in a 51-year-old man who initially exhibited unilateral blue toe syndrome, which swiftly progressed to dry gangrene of the toes. Despite reports of large-vessel involvement, there is a paucity of data on isolated small-vessel vasculitis-induced digital ischemia in VBD. CONCLUSIONS This atypical case underscores the necessity of clinical discernment in differentiating inflammatory microvascular occlusive disease from vasospastic Raynaud's syndrome, both of which can complicate Behçet's disease. FAU - Aljarrah, Qusai AU - Aljarrah Q AUID- ORCID: 0000-0003-0234-423X AD - Department of General and Vascular Surgery, King Abdullah University Hospital (KAUH), Jordan University of Science and Technology (JUST), Irbid, Jordan. FAU - Ba-Shammakh, Saleh A AU - Ba-Shammakh SA AD - Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan. FAU - Allouh, Mohammed Z AU - Allouh MZ AUID- ORCID: 0000-0003-0105-6260 AD - Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan. AD - Department of Anatomy, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates. FAU - Afaneh, Mohammed W AU - Afaneh MW AD - Department of General and Vascular Surgery, King Abdullah University Hospital (KAUH), Jordan University of Science and Technology (JUST), Irbid, Jordan. LA - eng PT - Case Reports PT - Journal Article DEP - 20240622 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - Humans MH - *Behcet Syndrome/complications/diagnosis MH - Male MH - Middle Aged MH - *Blue Toe Syndrome/etiology PMC - PMC11334092 COIS- Conflict of interest: None declared EDAT- 2024/06/22 19:43 MHDA- 2024/06/22 19:44 PMCR- 2024/06/22 CRDT- 2024/06/22 01:22 PHST- 2024/06/22 19:44 [medline] PHST- 2024/06/22 19:43 [pubmed] PHST- 2024/06/22 01:22 [entrez] PHST- 2024/06/22 00:00 [pmc-release] AID - 943813 [pii] AID - 10.12659/AJCR.943813 [doi] PST - epublish SO - Am J Case Rep. 2024 Jun 22;25:e943813. doi: 10.12659/AJCR.943813. PMID- 42110615 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260511 LR - 20260511 IS - 1734-1922 (Print) IS - 1896-9151 (Electronic) IS - 1734-1922 (Linking) VI - 22 IP - 1 DP - 2026 Jan TI - Relationship between clinical characteristics and serum tumor markers in connective tissue disease with interstitial lung disease as the initial manifestation. PG - 155-162 LID - 10.5114/aoms/204199 [doi] AB - INTRODUCTION: Interstitial lung disease (ILD) is a common complication of connective tissue disease (CTD), which seriously affects the prognosis of patients. The abnormal expression of tumor markers in non-neoplastic diseases may be related to the occurrence and development of CTD-ILD. This study aimed to explore the detailed clinical characteristics of CTD-ILD, and to analyze their association with serum tumor markers. MATERIAL AND METHODS: The clinical data of 128 patients with CTD-ILD were retrospectively analyzed. Seventy-nine ILD patients without CTD were enrolled as the non-combined group. Clinical data included imaging manifestations, laboratory indices and tumor markers such as carbohydrate antigen (CA) 125, CA153, carcinoembryonic antigen (CEA), neuron-specific enolase (NSE), and squamous cell carcinoma (SCC) antigen were collected. ROC curve analysis was used to assess the clinical value of these markers. RESULTS: The proportion of clinical manifestations such as arthralgia, rash, Raynaud's phenomenon, dry mouth and dry eyes in the combined group was higher than in the non-combined group (p < 0.05). The serum albumin and total protein levels in the combined group were lower than in the non-combined group (p < 0.001). The levels of CA125, CA153, CEA, SCC, and NSE in the combined group were higher than in the non-combined group (p < 0.001). The AUC of combined detection of each index was 0.917, with a sensitivity of 97.47% and a specificity of 76.56%. CONCLUSIONS: The main clinical manifestations for CTD-ILD patients were arthralgia, rash, Raynaud's phenomenon, and dry mouth and eyes. The combined detection of tumor markers had high evaluation value. CI - Copyright: © 2025 Termedia & Banach. FAU - Wang, Pengfei AU - Wang P AD - Shanxi Bethune Hospital, China. FAU - Duan, Wei AU - Duan W AD - Shanxi Bethune Hospital, China. FAU - Guo, Qian AU - Guo Q AD - Shanxi Bethune Hospital, China. FAU - Wei, Yaona AU - Wei Y AD - Shanxi Bethune Hospital, China. FAU - Hao, Yanyan AU - Hao Y AD - Shanxi Bethune Hospital, China. LA - eng PT - Journal Article DEP - 20250819 PL - Poland TA - Arch Med Sci JT - Archives of medical science : AMS JID - 101258257 PMC - PMC13154795 OTO - NOTNLM OT - clinical characteristics OT - connective tissue disease OT - interstitial lung disease OT - serum tumor markers COIS- The authors declare no conflict of interest. EDAT- 2026/05/11 13:00 MHDA- 2026/05/11 13:01 PMCR- 2025/08/19 CRDT- 2026/05/11 06:45 PHST- 2024/05/27 00:00 [received] PHST- 2025/04/20 00:00 [accepted] PHST- 2026/05/11 13:01 [medline] PHST- 2026/05/11 13:00 [pubmed] PHST- 2026/05/11 06:45 [entrez] PHST- 2025/08/19 00:00 [pmc-release] AID - 204199 [pii] AID - 10.5114/aoms/204199 [doi] PST - epublish SO - Arch Med Sci. 2025 Aug 19;22(1):155-162. doi: 10.5114/aoms/204199. eCollection 2026 Jan. PMID- 33969130 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220422 IS - 2322-3480 (Print) IS - 2322-3480 (Electronic) IS - 2322-3480 (Linking) VI - 9 IP - 4 DP - 2021 Jan TI - Para-Clinical and Immunological Evaluation in Buerger's Disease as a Suspected Autoimmune Disease: Case Series. PG - 379-384 LID - 10.52547/rbmb.9.4.373 [doi] AB - BACKGROUND: Autoimmunity causes the loss of normal immune homeostasis and involves the presence of autoantibodies and inflammation. Thromboangiitis obliterans or Buerger's disease (BD) refers to a type of vascular obstructive syndrome, with tobacco exposure accounting for disease formation and progression. However, the current understanding of autoimmunity is unclear in the context of BD, and the scientific findings are not enough to support autoimmune mechanisms. This study was aimed at investigating autoimmunity factors in patients with BD. METHODS: Clinical and experimental examinations were performed on 80 patients with BD. The diagnostic work-up for autoimmunity was composed of IgM rheumatoid factor (RF), anti-nuclear antibodies (ANA), The erythrocyte sedimentation rate (ESR), anti-cyclic citrullinated peptide (CCP) antibodies, Antiphospholipid antibodies (APA), Anti-cardiolipin antibodies (ACLA), anti-double-stranded DNA (ds-DNA), and extractable nuclear antigen (ENA) profile. Immunomarkers were detected using the quantitative enzyme-linked immunosorbent assay (ELISA). RESULTS: Raynaud's phenomenon (84.93%), cold sensitivity (76.25%), and claudication (73.75%) were the most common symptoms in the BD patients. Also, 64.29% represented with high ANA levels and positive RF, while 42.11% were found with increased ANA and ESR levels. The ANA/RF positive BD patients had ESR> 15 mm/hr and a high prevalence of cold sensitivity, claudication, and Raynaud's phenomenon (p> 0.05). CONCLUSION: There is a possibility of a non-specific autoimmune disposition among BD patients. RF and ANA could be considered for predicting disease progression. FAU - Shapouri-Moghaddam, Abbas AU - Shapouri-Moghaddam A AD - Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. FAU - Tavakkol Afshari, Seyed Jalil AU - Tavakkol Afshari SJ AD - Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. FAU - Rahimi, Hamid Reza AU - Rahimi HR AD - Department of Medical Genetics and Molecular Medicine, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. FAU - Saeed Modaghegh, Mohammad-Hadi AU - Saeed Modaghegh MH AD - Vascular and Endovascular Surgery Research Center, Alavi Hospital, Mashhad University of Medical Sciences, Mashhad, Iran. FAU - Mahmoudi, Mahmoud AU - Mahmoudi M AD - Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. FAU - Ehteshamfar, Seyed Morteza AU - Ehteshamfar SM AD - Immunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. LA - eng PT - Journal Article PL - Iran TA - Rep Biochem Mol Biol JT - Reports of biochemistry & molecular biology JID - 101637937 PMC - PMC8068440 OTO - NOTNLM OT - Antibodies OT - Autoimmunity OT - Buerger's Disease OT - Immune System EDAT- 2021/05/11 06:00 MHDA- 2021/05/11 06:01 PMCR- 2021/01/01 CRDT- 2021/05/10 06:30 PHST- 2021/05/10 06:30 [entrez] PHST- 2021/05/11 06:00 [pubmed] PHST- 2021/05/11 06:01 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - rbmb-9-379 [pii] AID - 10.52547/rbmb.9.4.373 [doi] PST - ppublish SO - Rep Biochem Mol Biol. 2021 Jan;9(4):379-384. doi: 10.52547/rbmb.9.4.373. PMID- 32110458 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2020 DP - 2020 TI - PL-7 Antisynthetase Syndrome in Association with Sjögren's, Systemic Lupus Erythematosus, and Rheumatoid Arthritis. PG - 4736476 LID - 10.1155/2020/4736476 [doi] LID - 4736476 AB - We present a rare case of PL-7 antisynthetase syndrome (ASS) in association with Sjögren's, systemic lupus erythematosus (SLE), and seropositive rheumatoid arthritis (RA). Initially, the patient was diagnosed with Sjögren's followed by Sjögren's/SLE overlap and then Sjögren's/SLE/RA overlap. She was eventually diagnosed with Sjögren's/SLE/RA overlap with PL-7 ASS with interstitial lung disease (ILD). ILD was discovered after complaints of pleuritic chest pain with subsequent workup with coronary computed tomography (CT) revealing pulmonary fibrosis. This case demonstrates the ambiguity with which symptoms of ASS can present; given the high respiratory morbidity and mortality of ASS especially in non-Jo-1 patients, those who present with Raynaud's, myositis, or joint pain, whether together or in isolation, should be assessed for presence of additional features of ASS and potentially undergo testing for ASS antibodies if appropriate. CI - Copyright © 2020 Mehrin Jawaid et al. FAU - Jawaid, Mehrin AU - Jawaid M AUID- ORCID: 0000-0002-2259-9478 AD - WellStar Kennestone Regional Medical Center, Marietta, GA 30060, USA. FAU - Ross, Yael AU - Ross Y AD - WellStar Kennestone Regional Medical Center, Marietta, GA 30060, USA. FAU - Kamran, Mohammad AU - Kamran M AD - WellStar Kennestone Regional Medical Center, Marietta, GA 30060, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20200213 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC7042534 COIS- The authors declare that there are no conflicts of interest regarding the publication of this article. EDAT- 2020/02/29 06:00 MHDA- 2020/02/29 06:01 PMCR- 2020/02/13 CRDT- 2020/02/29 06:00 PHST- 2019/07/21 00:00 [received] PHST- 2019/12/01 00:00 [revised] PHST- 2020/01/10 00:00 [accepted] PHST- 2020/02/29 06:00 [entrez] PHST- 2020/02/29 06:00 [pubmed] PHST- 2020/02/29 06:01 [medline] PHST- 2020/02/13 00:00 [pmc-release] AID - 10.1155/2020/4736476 [doi] PST - epublish SO - Case Rep Rheumatol. 2020 Feb 13;2020:4736476. doi: 10.1155/2020/4736476. eCollection 2020. PMID- 26738265 OWN - NLM STAT- MEDLINE DCOM- 20160201 LR - 20200507 IS - 0370-629X (Print) IS - 0370-629X (Linking) VI - 70 IP - 11 DP - 2015 Nov TI - [A case of Sneddon's syndrome associated with Moskowitz's syndrome]. PG - 546-9 AB - Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion. The two main features of this syndrome are livedo racemosa and ischemic stroke. Here, we describe the case of a 37-year-old woman with a past medical history of Moskowitz's syndrome and migraine, and a 2-year history of livedo racemosa and Raynaud's phenomenon. She presented with acute aphasia related to cortical ischemic stroke. Extensive diagnostic workup allowed to diagnose a Sneddon's syndrome. Her symptoms improved on a platelet aggregation inhibitor (aspirin), and speech therapy. This condition can be associated with other disorders affecting small vessels such as illustrated in this case with Moskowitz's syndrome. FAU - Belfeki, N AU - Belfeki N FAU - Dieudonné, L AU - Dieudonné L LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - LE CAS CLINIQUE DU MOIS. Un syndrome de Sneddon associé à un syndrome de Moskowitz. PL - Belgium TA - Rev Med Liege JT - Revue medicale de Liege JID - 0404317 SB - IM MH - Adult MH - Female MH - Humans MH - Magnetic Resonance Imaging MH - Purpura, Thrombotic Thrombocytopenic/*complications MH - Sneddon Syndrome/*complications/*diagnosis MH - Stroke/pathology MH - White Matter/pathology EDAT- 2016/01/08 06:00 MHDA- 2016/02/02 06:00 CRDT- 2016/01/08 06:00 PHST- 2016/01/08 06:00 [entrez] PHST- 2016/01/08 06:00 [pubmed] PHST- 2016/02/02 06:00 [medline] PST - ppublish SO - Rev Med Liege. 2015 Nov;70(11):546-9. PMID- 40270665 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250425 IS - 2284-2594 (Electronic) IS - 2284-2594 (Linking) VI - 12 IP - 4 DP - 2025 TI - Inhaled Iloprost for Digital Necrosis in Systemic Sclerosis - An Effective Alternative. PG - 004913 LID - 10.12890/2025_004913 [doi] LID - 004913 AB - INTRODUCTION: Vascular issues in systemic sclerosis (SSc), notably Raynaud's phenomenon, can lead to digital ulcers (DU) and necrosis, causing pain and infections. Intravenous prostacyclin (iloprost) is used for established DUs. We report a case where severe Raynaud's phenomenon revealed SSc sine scleroderma, which improved with inhaled iloprost. CASE DESCRIPTION: A 52-year-old former smoker with protein C deficiency, on anticoagulation since 2016 for a previous deep vein thrombosis, presented with severe Raynaud's phenomenon and pulp necrosis of the third right finger. The capillaroscopy revealed megacapillaries suggestive of a connective tissue disease, and antinuclear antibodies were positive for centromere fluorescence. Doppler ultrasound and cardiopulmonary assessments were normal. The patient underwent necrosectomy but had ischemic pain and cyanosis post-surgery. Inhaled iloprost (2 ampoules of 10 mg/10 ml daily in 3 cycles of 5 days) led to significant clinical improvement. CONCLUSION: Inhaled iloprost could be an effective and better-tolerated alternative to intravenous iloprost for treating SSc DUs. Further studies are needed to confirm this potential. LEARNING POINTS: This case report shows the efficacy of inhaled prostacyclin in the treatment of digital ulcers in systemic sclerosis as an equally effective, better tolerated, and safer alternative to the usual intravenous route. CI - © EFIM 2025. FAU - Yousfi, Jaouad AU - Yousfi J AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Alami Idrissi, Soukaina AU - Alami Idrissi S AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Dakir, Fatima AU - Dakir F AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Bott, Zineb AU - Bott Z AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Taouil, Chadyne AU - Taouil C AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Zahlane, Mouna AU - Zahlane M AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. FAU - Essaadouni, Lamiaa AU - Essaadouni L AD - Internal Medicine Department, Mohamed VI hospital, Marrakech, Morocco. LA - eng PT - Journal Article DEP - 20250319 PL - Italy TA - Eur J Case Rep Intern Med JT - European journal of case reports in internal medicine JID - 101648453 PMC - PMC12013218 OTO - NOTNLM OT - Systemic sclerosis OT - digital ulcers OT - inhaled prostacyclin OT - necrosis COIS- Conflicts of Interests: The Authors declare that there are no competing interests. EDAT- 2025/04/24 12:29 MHDA- 2025/04/24 12:30 PMCR- 2025/03/19 CRDT- 2025/04/24 06:17 PHST- 2024/09/23 00:00 [received] PHST- 2024/10/22 00:00 [accepted] PHST- 2025/04/24 12:30 [medline] PHST- 2025/04/24 12:29 [pubmed] PHST- 2025/04/24 06:17 [entrez] PHST- 2025/03/19 00:00 [pmc-release] AID - 4913 [pii] AID - 10.12890/2025_004913 [doi] PST - epublish SO - Eur J Case Rep Intern Med. 2025 Mar 19;12(4):004913. doi: 10.12890/2025_004913. eCollection 2025. PMID- 23065452 OWN - NLM STAT- MEDLINE DCOM- 20130520 LR - 20211021 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 14 IP - 6 DP - 2012 Dec TI - Is there evidence for vasculitis in systemic sclerosis? PG - 516-25 LID - 10.1007/s11926-012-0296-9 [doi] AB - Systemic sclerosis (SSc) is a devastating and potentially life-threatening multi-organ system disease. SSc is marked by skin thickening and tightening, Raynaud's phenomenon and digital ischemia with ulceration, gastrointestinal dysmotility, cardiopulmonary involvement with pulmonary fibrosis and pulmonary arterial hypertension, as well as renal failure. Fibrosis is the most obvious manifestation of SSc. Vascular involvement and inflammation are other prominent components of SSc pathology, and both features are also seen in vasculitis. This review analyzes whether there is evidence for vasculitis especially with particular organ manifestations and subgroups of patients. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - LSU Scleroderma and Sarcoidosis Patient Care and Research Center, Louisiana State University Health Sciences Center, New Orleans, LA, USA. ctd.ild@gmail.com FAU - Distler, Oliver AU - Distler O LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 SB - IM MH - Humans MH - Inflammation/physiopathology MH - Scleroderma, Systemic/*complications/physiopathology MH - Vascular Diseases/etiology/physiopathology MH - Vasculitis/*etiology/physiopathology EDAT- 2012/10/16 06:00 MHDA- 2013/05/22 06:00 CRDT- 2012/10/16 06:00 PHST- 2012/10/16 06:00 [entrez] PHST- 2012/10/16 06:00 [pubmed] PHST- 2013/05/22 06:00 [medline] AID - 10.1007/s11926-012-0296-9 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2012 Dec;14(6):516-25. doi: 10.1007/s11926-012-0296-9. PMID- 18504941 OWN - NLM STAT- MEDLINE DCOM- 20080701 LR - 20200103 IS - 0204-8043 (Print) IS - 0204-8043 (Linking) VI - 49 IP - 3-4 DP - 2007 TI - Capi text V.1--data analysis software for nailfold skin capillaroscopy. PG - 84-7 AB - INTRODUCTION: Nailfold skin capillaroscopy is a simple non-invasive method used to assess conditions of disturbed microcirculation such as Raynaud's phenomenon, acrocyanosis, perniones, connective tissue diseases, psoriasis, diabetes mellitus, neuropathy and vibration disease. AIM: To develop data analysis software aimed at assisting the documentation and analysis of a capillaroscopic investigation. SOFTWARE DESCRIPTION: The programme is based on a modular principle. The module "Nomenclatures" includes menus for the patients' data. The module "Examinations" includes menus for all general and specific aspects of the medical examination and capillaroscopic investigations. The modules "Settings" and "Information" include customization menus for the programme. The results of nailfold capillaroscopy can be printed in a short or expanded form. This software allows physicians to perform quick search by using various specified criteria and prepare analyses and reports. CONCLUSION: This software programme will facilitate any practitioner who performs nailfold skin capillaroscopy. FAU - Dobrev, Hristo P AU - Dobrev HP AD - Department of Dermatology and Venereology, Medical University, Plovdiv, Bulgaria. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Bulgaria TA - Folia Med (Plovdiv) JT - Folia medica JID - 2984761R SB - IM MH - Capillaries/pathology MH - Humans MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Software MH - Statistics as Topic EDAT- 2008/05/29 09:00 MHDA- 2008/07/02 09:00 CRDT- 2008/05/29 09:00 PHST- 2008/05/29 09:00 [pubmed] PHST- 2008/07/02 09:00 [medline] PHST- 2008/05/29 09:00 [entrez] PST - ppublish SO - Folia Med (Plovdiv). 2007;49(3-4):84-7. PMID- 31344020 OWN - NLM STAT- MEDLINE DCOM- 20200129 LR - 20200225 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 20 DP - 2019 Jul 25 TI - Antisynthetase Syndrome and Autoantibodies: A Literature Review and Report of 4 Cases. PG - 1094-1103 LID - 10.12659/AJCR.916178 [doi] AB - BACKGROUND With the advent and advancement of autoantibodies, there has been progress in the diagnosis, prognosis, and treatment of rheumatologic diseases. Antisynthetase syndrome (ASS) is a great example of a disease that initially was described as arthritis, myositis, interstitial lung disease, mechanic's hands, Raynaud's phenomenon, and fever in the presence of the anti-JO-1 antibody, but nowadays it presents with a different spectrum related to new antibodies. CASE REPORT We describe 4 patients with antisynthetase syndrome who were diagnosed with antibodies specific for myositis associated with different clinical findings. All patients responded to immunosuppressive therapy, and rituximab was the most used. CONCLUSIONS It is necessary to search for specific autoantibodies related to the syndrome in suspected clinical cases and in other rheumatological diseases refractory to the usual treatment. FAU - Marin, Flávia Luiza AU - Marin FL AD - Postgraduate Program in Pathophysiology in Medical Clinic, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil. FAU - Sampaio, Henrique Pereira AU - Sampaio HP AD - Department of Rheumatology, Division of Medical Clinic, Section of Medicine, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20190725 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 RN - 0 (Autoantibodies) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - VB0R961HZT (Prednisone) RN - YL5FZ2Y5U1 (Methotrexate) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Autoantibodies/*blood MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Methotrexate/therapeutic use MH - Myositis/*diagnosis/drug therapy MH - Prednisone/therapeutic use MH - Rare Diseases PMC - PMC6676984 COIS- Conflict of interest: None declared Conflict of interest None. EDAT- 2019/07/26 06:00 MHDA- 2020/01/30 06:00 PMCR- 2019/07/25 CRDT- 2019/07/26 06:00 PHST- 2019/07/26 06:00 [entrez] PHST- 2019/07/26 06:00 [pubmed] PHST- 2020/01/30 06:00 [medline] PHST- 2019/07/25 00:00 [pmc-release] AID - 916178 [pii] AID - 10.12659/AJCR.916178 [doi] PST - epublish SO - Am J Case Rep. 2019 Jul 25;20:1094-1103. doi: 10.12659/AJCR.916178. PMID- 32467874 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 30 IP - 4 DP - 2019 Dec TI - Giant Cell Hepatitis - A Rare Association with Connective Tissue Disease. PG - 224-227 LID - 10.31138/mjr.30.4.224 [doi] AB - A 68-year-old gentleman presented to hepatology department with asymptomatic year-long history of stably deranged liver function tests. His peak alkaline phosphatase (ALP), was 828 with alanine transaminase (ALT) of 141. Full liver workup was negative; hence, a liver biopsy was organised, which confirmed giant cell hepatitis (GCH). A computed tomography (CT) scan revealed non-specific interstitial pneumonitis (NSIP) pattern interstitial lung disease supported by lung function tests. Antibody testing showed strongly positive antinuclear antibody (ANA) with anti-polymyositis/scleroderma (anti-PM-SCL) antibody. Clinical picture was in keeping with likely undifferentiated connective tissue disease (UCTD) with polyarthralgia, early morning stiffness, Raynaud's and nailfold infarcts with capillaritis on nail bed examination. Further testing confirmed triple-positive antiphospholipid antibodies twice 12 weeks apart (immunoglobulin M [IgM] anti beta-2 glycoprotein antibodies, lupus anticoagulant and IgM anticardiolipin antibody). He was treated with mycophenolate and hydroxychloroquine with resolution of symptoms. Giant cell hepatitis is uncommon, with only 100 cases reported worldwide. To our knowledge, this is the only report of GCH in the context of UCTD, highlighting the significance of careful evaluation of liver disease overlap and the successful role of mycophenolate mofetil (MMF) in this setting. CI - © 2019 The Mediterranean Journal of Rheumatology (MJR). FAU - Rauf, Maria AU - Rauf M AD - Histopathology Department, Luton & Dunstable University Hospital, Luton, United Kingdom. FAU - Sen, Sambit AU - Sen S AD - Hepatology Department, Luton & Dunstable University Hospital, Luton, United Kingdom. FAU - Levene, Adam AU - Levene A AD - Histopathology Department, Luton & Dunstable University Hospital, Luton, United Kingdom. FAU - Nisar, Muhammad K AU - Nisar MK AD - Rheumatology Department, Luton & Dunstable University Hospital, Luton, United Kingdom. LA - eng PT - Case Reports PT - Journal Article DEP - 20200331 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7241662 OTO - NOTNLM OT - antiphospholipid antibodies OT - connective tissue disease OT - giant cell hepatitis EDAT- 2020/05/30 06:00 MHDA- 2020/05/30 06:01 PMCR- 2019/12/31 CRDT- 2020/05/30 06:00 PHST- 2019/04/25 00:00 [received] PHST- 2019/11/09 00:00 [revised] PHST- 2019/11/11 00:00 [accepted] PHST- 2020/05/30 06:00 [entrez] PHST- 2020/05/30 06:00 [pubmed] PHST- 2020/05/30 06:01 [medline] PHST- 2019/12/31 00:00 [pmc-release] AID - MJR-30-4-224 [pii] AID - 10.31138/mjr.30.4.224 [doi] PST - epublish SO - Mediterr J Rheumatol. 2020 Mar 31;30(4):224-227. doi: 10.31138/mjr.30.4.224. eCollection 2019 Dec. PMID- 32284267 OWN - NLM STAT- MEDLINE DCOM- 20210305 LR - 20210305 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 34 IP - 4 DP - 2020 Aug TI - Clinical manifestations and treatment of antisynthetase syndrome. PG - 101503 LID - S1521-6942(20)30020-6 [pii] LID - 10.1016/j.berh.2020.101503 [doi] AB - Antisynthetase syndrome (ASyS) is an autoimmune disease clinically manifested most often by interstitial lung disease, myositis, and arthritis. Raynaud's syndrome, fever, and rashes are also commonly seen. This syndrome is characterized by the highly specific presence of antibodies against various aminoacyl transfer RNA synthetases, including Jo-1 and others. In this chapter, we provide an overview of ASyS, including pathogenesis, common clinical manifestations, and treatment strategies. We discuss the spectrum of disease seen with specific antisynthetase antibodies and examine the differences in phenotype between patients with different antisynthetase antibodies. We outline common treatment strategies, which should generally target the most severe and life- or organ-threatening disease manifestations. Finally, we discuss short- and long-term prognosis in ASyS. CI - Copyright © 2020 Elsevier Ltd. All rights reserved. FAU - Marco, Joanna L AU - Marco JL AD - University of Washington, Department of Medicine, Division of Rheumatology, 1959 NE Pacific Street, Box 356428, Seattle, WA, 98195-6522, USA. Electronic address: JLMarco@uw.edu. FAU - Collins, Bridget F AU - Collins BF AD - University of Washington Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, 1959 NE Pacific Street, Box 356166, Seattle, WA, 98195, USA. Electronic address: bfc3@uw.edu. LA - eng PT - Journal Article PT - Review DEP - 20200411 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - *Amino Acyl-tRNA Synthetases/immunology MH - Antibodies, Antinuclear MH - Arthritis/diagnosis/etiology/therapy MH - Autoantibodies MH - Humans MH - Lung Diseases, Interstitial/diagnosis/etiology/therapy MH - *Myositis/diagnosis/pathology/therapy OTO - NOTNLM OT - Anti-Jo-1 OT - Antisynthetase antibody OT - Antisynthetase syndrome OT - Arthritis OT - Idiopathic inflammatory myopathy OT - Interstitial lung disease OT - Mechanic's hands EDAT- 2020/04/15 06:00 MHDA- 2021/03/06 06:00 CRDT- 2020/04/15 06:00 PHST- 2020/04/15 06:00 [pubmed] PHST- 2021/03/06 06:00 [medline] PHST- 2020/04/15 06:00 [entrez] AID - S1521-6942(20)30020-6 [pii] AID - 10.1016/j.berh.2020.101503 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2020 Aug;34(4):101503. doi: 10.1016/j.berh.2020.101503. Epub 2020 Apr 11. PMID- 17550632 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20070730 LR - 20240405 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 5 DP - 2007 May 1 TI - Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center. PG - 6 AB - BACKGROUND: Juvenile systemic sclerosis (JSS) is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. It has a low prevalence, even in a tertiary facility setting. The purpose of the present study is to describe and analyze the clinical and laboratory characteristics of a group of children with JSS followed in a single center. METHODS: Clinical charts of children with a diagnosis of JSS who were seen at a tertiary referral center between 1995 and 2005 were reviewed. Clinical features were recorded and analysed. RESULTS: Twenty-three patients who met preliminary classification criteria for JSS were included. Age at first symptom attributable to JSS was 6 (1-14) years, The first symptom attributable to JSS was Raynaud's phenomenon in 14 cases. Proximal sclerosis (23 patients, 100%), sclerodactyly (21, 91%), Raynaud's phenomenon (19, 83%), and periungual capillaropathy (17, 74%) were the most consistent clinical findings during follow-up. Respiratory involvement occurred in two thirds of our patients, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests; pulmonary hypertension was an infrequent finding. Dysphagia was the commonest gastrointestinal symptom (9 patients, 39%). The most frequent musculoskeletal symptom was arthralgia (14 children, 6%); symmetrical arthritis was found in 8 (35%) patients. Periungual capillary abnormalities were evident during physical examination in 17 children; capillaroscopy revealed abnormalities in all 19 examined patients. ANA were present in 17 (74%) children: homogeneous pattern was the most frequent (8 patients), nucleolar (5) and speckled (4) were less common. CONCLUSION: Raynaud's phenomenon heralds the beginning of the disease. Capilaroscopy is a major adjuvant in the diagnosis, since autoantibody determination may not offer sensitive and specific markers. Skin and vascular manifestations are the most common clinical features, while internal organ involvement is more rare. Cardiopulmonary disease is the most frequent visceral involvement, leading to significant morbidity. FAU - Russo, Ricardo A G AU - Russo RA AD - Service of Immunology and Rheumatology, Hospital de Pediatría Prof, Dr, Juan P, Garrahan, Combate de los Pozos 1881, (1245) Buenos Aires, Argentina. rrusso@garrahan.gov.ar FAU - Katsicas, María M AU - Katsicas MM LA - eng PT - Journal Article DEP - 20070501 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 PMC - PMC1869029 EDAT- 2007/06/07 09:00 MHDA- 2007/06/07 09:01 PMCR- 2007/05/01 CRDT- 2007/06/07 09:00 PHST- 2007/02/13 00:00 [received] PHST- 2007/05/01 00:00 [accepted] PHST- 2007/06/07 09:00 [pubmed] PHST- 2007/06/07 09:01 [medline] PHST- 2007/06/07 09:00 [entrez] PHST- 2007/05/01 00:00 [pmc-release] AID - 1546-0096-5-6 [pii] AID - 10.1186/1546-0096-5-6 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2007 May 1;5:6. doi: 10.1186/1546-0096-5-6. PMID- 39981635 OWN - NLM STAT- MEDLINE DCOM- 20250509 LR - 20250509 IS - 1744-7666 (Electronic) IS - 1465-6566 (Linking) VI - 26 IP - 5 DP - 2025 Apr TI - Systemic pharmacotherapy approaches for the treatment of systemic sclerosis. PG - 551-566 LID - 10.1080/14656566.2025.2470846 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) represents a complex, multisystem rheumatologic disorder characterized by immune dysregulation, vascular dysfunction, and multi-organ fibrosis. This review discusses the efficacy of the available therapeutic options and the significance of developing effective strategies against its varied manifestations, pivotal to improving patient outcomes. AREAS COVERED: The review elaborates on the pharmacological treatments available for managing key manifestations of SSc, including skin and lung involvement, and vascular complications, as well as the most recent findings in the field. We evaluated recent literature and clinical trials from the past decade, as well as most recent guidelines from entities like EULAR and the ACR, to provide a comprehensive overview of current management strategies. EXPERT OPINION: Despite advancements in therapeutic options, SSc remains a challenging disease to manage due to its complexity, our relatively limited understanding of disease pathogenesis, and its severe impact on quality of life. The development of targeted therapies and the refinement of existing treatment protocols offer hope for better management. Future research should focus on personalized medicine approaches and refining treatment algorithms to optimize outcomes for patients. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. AD - CNRS, ImmunoConcEpT, Univ. Bordeaux, Bordeaux, Nouvelle-Aquitaine, France. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - Department of Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Department of Rheumatology, Salford, UK. LA - eng PT - Journal Article PT - Review DEP - 20250227 PL - England TA - Expert Opin Pharmacother JT - Expert opinion on pharmacotherapy JID - 100897346 SB - IM MH - Humans MH - *Scleroderma, Systemic/drug therapy/physiopathology/complications MH - Quality of Life MH - Precision Medicine MH - Practice Guidelines as Topic MH - Molecular Targeted Therapy MH - Animals OTO - NOTNLM OT - Raynaud’s Phenomenon OT - Systemic sclerosis OT - digital ulcer OT - scleroderma OT - systemic pharmacotherapy EDAT- 2025/02/21 06:22 MHDA- 2025/04/01 06:26 CRDT- 2025/02/21 05:42 PHST- 2025/04/01 06:26 [medline] PHST- 2025/02/21 06:22 [pubmed] PHST- 2025/02/21 05:42 [entrez] AID - 10.1080/14656566.2025.2470846 [doi] PST - ppublish SO - Expert Opin Pharmacother. 2025 Apr;26(5):551-566. doi: 10.1080/14656566.2025.2470846. Epub 2025 Feb 27. PMID- 23984162 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130828 LR - 20211021 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2013 DP - 2013 TI - A case of subacute cutaneous lupus erythematosus in a patient with mixed connective tissue disease: successful treatment with plasmapheresis and rituximab. PG - 857694 LID - 10.1155/2013/857694 [doi] LID - 857694 AB - A 30-year-old woman affected by Mixed Connective Tissue Disease with scleroderma spectrum developed a facial eruption, a clinical and histological characteristic of subacute cutaneous lupus erythematosus (SCLE). Speckled anti-nuclear antibodies, high-titer anti-ribonucleoprotein1, anti-Sm, anti-Cardiolipin (aCL) IgG/IgM, and anti-Ro/SSA antibodies were positive. SCLE was resistant to Azathioprine, Hydroxychloroquine, and Methotrexate while Mycophenolate Mofetil was suspended due to side effects. Subsequently, the patient was treated with three cycles of therapeutic plasma exchange (TPE) followed, one month after the last TPE, by the anti-CD20 antibody Rituximab (RTX) (375 mg/m(2) weekly for 4 weeks). Eight and 16 months later the patient received other two TPE and RTX cycles, respectively. This therapeutic approach has allowed to obtain a complete skin healing persistent even after 8-month follow-up. Moreover, mitigation of Raynaud's phenomenon, resolution of alopecia, and a decline of aCL IgG/IgM and anti-Ro/SSA antibodies were observed. FAU - Fantò, M AU - Fantò M AD - Department of Allergy, Clinical Immunology and Rheumatology, S. Andrea Hospital, Sapienza University of Rome, Italy. FAU - Salemi, S AU - Salemi S FAU - Socciarelli, F AU - Socciarelli F FAU - Bartolazzi, A AU - Bartolazzi A FAU - Natale, G A AU - Natale GA FAU - Casorelli, I AU - Casorelli I FAU - Pavan, A AU - Pavan A FAU - Vaglio, S AU - Vaglio S FAU - Di Rosa, R AU - Di Rosa R FAU - D'Amelio, R AU - D'Amelio R LA - eng PT - Journal Article DEP - 20130728 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC3745888 EDAT- 2013/08/29 06:00 MHDA- 2013/08/29 06:01 PMCR- 2013/07/28 CRDT- 2013/08/29 06:00 PHST- 2013/06/04 00:00 [received] PHST- 2013/06/26 00:00 [accepted] PHST- 2013/08/29 06:00 [entrez] PHST- 2013/08/29 06:00 [pubmed] PHST- 2013/08/29 06:01 [medline] PHST- 2013/07/28 00:00 [pmc-release] AID - 10.1155/2013/857694 [doi] PST - ppublish SO - Case Rep Rheumatol. 2013;2013:857694. doi: 10.1155/2013/857694. Epub 2013 Jul 28. PMID- 18651055 OWN - NLM STAT- MEDLINE DCOM- 20081103 LR - 20191111 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 60 IP - 2 DP - 2008 Apr-Jun TI - [Long-term effects of cyclic therapy with iloprost in systemic sclerosis]. PG - 114-9 AB - OBJECTIVE: To assess the long-term effects of cyclic infusion of iloprost, a derivative of prostacyclin, on Raynaud's phenomenon-related symptoms and ischemic ulcers in patients with Systemic Sclerosis (SSc). METHODS: A retrospective analysis of prospectively collected parameters in 59 consecutive SSc patients, followed at one institution, who were treated for a median time of 52 months with iloprost for severe Raynaud's phenomenon and ischemic ulcers. RESULTS: Among the 50 patients with ischemic ulcers at the start of therapy, 35 (70%) did not show lesions at the last observation. Despite therapy, four patients underwent amputations (two of forefoot, two of finger distal phalanges). Compared to the pre-treatment point, we observed: decrease of the Raynaud's phenomenon VAS (p<0.001), and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score (p=0.002). The Health Assessment Questionnaire was not significantly improved. CONCLUSION: Treatment with cyclic iloprost can control Raynaud's phenomenon-related symptoms and ischemic ulcers in the large majority of patients with SSc. However, a disease-modifying effect of this therapy could not be demonstrated. FAU - Scarsi, M AU - Scarsi M AD - Servizio di Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italia. scarsi@bresciareumatologia.it FAU - Rossi, M AU - Rossi M FAU - Danieli, E AU - Danieli E FAU - Frigè, A AU - Frigè A FAU - Grottolo, A AU - Grottolo A FAU - Zambruni, A AU - Zambruni A FAU - Airò, P AU - Airò P LA - ita PT - English Abstract PT - Journal Article TT - Effetti a lungo termine della terapia ciclica con iloprost nella sclerosi sistemica. PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Iloprost/*administration & dosage MH - Male MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/*drug therapy MH - Time Factors EDAT- 2008/07/25 09:00 MHDA- 2008/11/04 09:00 CRDT- 2008/07/25 09:00 PHST- 2008/07/25 09:00 [pubmed] PHST- 2008/11/04 09:00 [medline] PHST- 2008/07/25 09:00 [entrez] AID - 10.4081/reumatismo.2008.114 [doi] PST - ppublish SO - Reumatismo. 2008 Apr-Jun;60(2):114-9. doi: 10.4081/reumatismo.2008.114. PMID- 25434081 OWN - NLM STAT- MEDLINE DCOM- 20150129 LR - 20200103 IS - 0204-8043 (Print) IS - 0204-8043 (Linking) VI - 56 IP - 3 DP - 2014 Jul-Sep TI - Association of eosinophilic fasciitis with morphea. PG - 220-4 AB - Eosinophilic fasciitis is a rare inflammatory disease of unknown etiology, described for the first time by Shulman in 1974. The disease presents with induration of the skin, connective tissue and the underlying muscle fascia, sometimes accompanied by myalgia, most commonly in the lower extremities. Unlike scleroderma, it presents with absence of visceral organ involvement and Raynaud's phenomenon. Hypergammaglobulinemia and eosinophilia have been reported. Eosinophilic fasciitis is often associated with hematological disorders--there are reports of combinations with other autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, Hashimoto thyroiditis, Sjogren syndrome, vitiligo, etc. Occurrence of morphea, in the course of eosinophil fasciitis is considered a rarity. We have observed such a case with the simultaneous presence of both types of lesions. A 20-year-old female patient is reported, wherein the clinical picture developed for 6 months. The initial erythematous edema and subsequently the livedo-like painful plaques in both lower legs gradually swell, thicken and hyperpigment. Almost simultaneously with these complaints small brown livid body plaques emerged. The patient was diagnosed based on history, clinical picture, peripheral eosinophilia and histological findings from the affected areas. There was no systemic involvement and accompanying hematologic or other disease. Therapeutic management and significant clinical improvement were achieved using systemic corticosteroid therapy combined with methotrexate. FAU - Zisova, Lilia G AU - Zisova LG AD - Department of Dermatology and Venereology, Faculty of Medicine, Medical University, Plovdiv FAU - Abadjieva, Cvetana I AU - Abadjieva CI AD - Department of Dermatology and Venereology, Faculty of Medicine, Medical University, Plovdiv FAU - Obreshkova, Elena V AU - Obreshkova EV AD - Department of Dermatology and Venereology, Military Medical Academy, Sofia FAU - Chernev, Georgi K AU - Chernev GK AD - Clinic of Dermatology and Venereology, Lozenetz, University Hospital Center, Sofi a, Bulgaria FAU - Vutova, Nina I AU - Vutova NI AD - Department of Dermatology and Venereology, Faculty of Medicine, Medical University, Plovdiv LA - eng PT - Case Reports PT - Journal Article PL - Bulgaria TA - Folia Med (Plovdiv) JT - Folia medica JID - 2984761R RN - Eosinophilic Fasciitis SB - IM EIN - Folia Med (Plovdiv). 2014 Oct-Dec;56(4):309. Abadjieva, Cvetana I [corrected to Abadjieva, Tsvetana I]; Chernev, Georgi K [corrected to Tchernev, Georgi K] MH - Adult MH - Eosinophilia/*etiology MH - Fasciitis/*etiology MH - Female MH - Humans MH - Scleroderma, Localized/*complications EDAT- 2014/12/02 06:00 MHDA- 2015/01/30 06:00 CRDT- 2014/12/02 06:00 PHST- 2014/12/02 06:00 [entrez] PHST- 2014/12/02 06:00 [pubmed] PHST- 2015/01/30 06:00 [medline] AID - 10.2478/folmed-2014-0032 [doi] PST - ppublish SO - Folia Med (Plovdiv). 2014 Jul-Sep;56(3):220-4. doi: 10.2478/folmed-2014-0032. PMID- 35549794 OWN - NLM STAT- MEDLINE DCOM- 20230207 LR - 20230207 IS - 1646-0758 (Electronic) IS - 0870-399X (Linking) VI - 36 IP - 2 DP - 2023 Feb 1 TI - Erasmus Syndrome: An Underrecognized Entity. PG - 122-126 LID - 10.20344/amp.16896 [doi] AB - We present a case of a 33-year-old male who worked as a plumber and a locksmith. The patient presented with diffuse myalgia and asthenia, skin sclerosis and puffy fingers, Raynaud's phenomenon, exertional dyspnea and erectile dysfunction. The presence of specific autoantibodies enabled the diagnosis of systemic sclerosis. Chest-computed tomography revealed upper lobe consolidation. After extensive evaluation, the multidisciplinary interstitial lung disease team concluded that the patient also had advanced silicosis. After a year, there was significant clinical, radiologic, and functional deterioration of the lung disease. The patient was referred for lung transplant. Silica inhalation is the cause of silicosis but is also implicated in the development of systemic sclerosis (Erasmus syndrome). Although they share a common risk factor, it is rare to find both diseases co-existing. We present this case of a young patient where both diseases presented aggressively in order to raise awareness to this association. FAU - Magalhães, Ana AU - Magalhães A AD - Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa. Portugal. FAU - Moreira, Inês AU - Moreira I AD - Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa. Portugal. FAU - Pinheiro, Sofia AU - Pinheiro S AD - Internal Medicine Department. Hospital Santo António dos Capuchos. Centro Hospitalar Universitário Lisboa Central. Lisboa. Portugal. FAU - Borba, Alexandra AU - Borba A AD - Pulmonology Department. Hospital de Santa Marta. Centro Hospitalar Universitário Lisboa Central. Lisboa. Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20220513 PL - Portugal TA - Acta Med Port JT - Acta medica portuguesa JID - 7906803 RN - 0 (Autoantibodies) SB - IM MH - Male MH - Humans MH - Adult MH - *Scleroderma, Systemic/diagnosis MH - *Silicosis/complications/diagnosis MH - *Lung Diseases, Interstitial MH - Lung MH - Autoantibodies MH - Syndrome OTO - NOTNLM OT - Connective Tissue Diseases OT - Lung Diseases, Interstitial OT - Scleroderma, Systemic OT - Silicosis EDAT- 2022/05/14 06:00 MHDA- 2023/02/08 06:00 CRDT- 2022/05/13 12:07 PHST- 2021/07/23 00:00 [received] PHST- 2022/01/07 00:00 [accepted] PHST- 2022/04/09 00:00 [revised] PHST- 2022/05/14 06:00 [pubmed] PHST- 2023/02/08 06:00 [medline] PHST- 2022/05/13 12:07 [entrez] AID - 10.20344/amp.16896 [doi] PST - ppublish SO - Acta Med Port. 2023 Feb 1;36(2):122-126. doi: 10.20344/amp.16896. Epub 2022 May 13. PMID- 33093861 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210903 IS - 1753-495X (Print) IS - 1753-4968 (Electronic) IS - 1753-495X (Linking) VI - 13 IP - 3 DP - 2020 Sep TI - Systemic sclerosis in pregnancy. PG - 105-111 LID - 10.1177/1753495X19878042 [doi] AB - Systemic sclerosis is a rare multisystem connective tissue disease. It predominantly affects women and poses a significant risk to mother and baby during pregnancy if not managed appropriately. The commonest manifestations are skin fibrosis and Raynaud's phenomenon. Subgroups of women have an increased risk of organ involvement, especially interstitial lung disease, pulmonary arterial hypertension and renal crises. Pregnancy increases the risk to the mother, especially those with established organ involvement, but also the development of new organ dysfunction; and risks to the fetus. Optimising these women prior to conception, along with careful management and surveillance during pregnancy, is vital for optimising pregnancy outcome. Women with scleroderma need to be managed in a specialised centre with coordinated care from the multi-disciplinary teams including physicians, obstetricians, anaesthetists, neonatologists and midwives. This review aims to describe the risks associated with systemic sclerosis and pregnancy, with management advice for physicians looking after pregnant women with this chronic condition. CI - © The Author(s) 2019. FAU - Clark, Kristina En AU - Clark KE AUID- ORCID: 0000-0002-5926-3900 AD - Centre of Rheumatology and Connective Tissue Diseases, UCL Royal Free Medical School, London, UK. FAU - Etomi, Oseme AU - Etomi O AD - Department of Obstetric Medicine, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Centre of Rheumatology and Connective Tissue Diseases, UCL Royal Free Medical School, London, UK. LA - eng PT - Journal Article PT - Review DEP - 20191030 PL - England TA - Obstet Med JT - Obstetric medicine JID - 101464191 PMC - PMC7543163 OTO - NOTNLM OT - High-risk pregnancy OT - perinatal medicine OT - rheumatology OT - systemic sclerosis EDAT- 2020/10/24 06:00 MHDA- 2020/10/24 06:01 PMCR- 2021/09/01 CRDT- 2020/10/23 05:57 PHST- 2018/08/01 00:00 [received] PHST- 2019/08/31 00:00 [accepted] PHST- 2020/10/23 05:57 [entrez] PHST- 2020/10/24 06:00 [pubmed] PHST- 2020/10/24 06:01 [medline] PHST- 2021/09/01 00:00 [pmc-release] AID - 10.1177_1753495X19878042 [pii] AID - 10.1177/1753495X19878042 [doi] PST - ppublish SO - Obstet Med. 2020 Sep;13(3):105-111. doi: 10.1177/1753495X19878042. Epub 2019 Oct 30. PMID- 19707370 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1177-5475 (Print) IS - 1177-5491 (Electronic) IS - 1177-5475 (Linking) VI - 2 IP - 3 DP - 2008 Sep TI - The role of B cells in systemic sclerosis. PG - 389-95 AB - Systemic sclerosis (SSc) is a connective disease characterized by features of autoimmunity, vasculopathy, inflammation, and fibrosis. The disease typically starts with Raynaud's phenomenon, followed by skin thickening in the extremities due to inflammation and fibrosis. Fibrosis results from excessive collagen production by fibroblasts, which constitutes the final common pathway of complex cellular interactions including B cells. Several studies have indicated that B cells may play a role in SSc. Lesional skin infiltrates from SSc patients consist of a variety of cells, including eosinophils, neutrophils, lymphocytes, plasma cells, and macrophages. Autoantibodies of several specificities are present in the serum of SSc patients of which antitopoisomerase 1 is the most common, and evidence has been gathered for a potential pathogenic role of some autoantibodies, eg, anti-PDGF antibodies. The blood of SSc patients contains an increased proportion of naïve B cells but a decreased proportion of memory B cells. Furthermore, serum levels of interleukin-6, an important pro-inflammatory cytokine, have been shown to correlate with skin fibrosis. Animal models of SSc have provided more in-depth information on the role of B lymphocytes, eg, through disruption of B cell function. In this review we will discuss the evidence that B cells are involved in the pathogenesis of SSc. FAU - Kraaij, Marina D AU - Kraaij MD AD - Musculoskeletal Research Group, Institute of Cellular Medicine, School of Clinical Medical Sciences, Newcastle University, Newcastle upon, Tyne NE2 4HH, United Kingdom. FAU - van Laar, Jacob M AU - van Laar JM LA - eng PT - Journal Article PL - New Zealand TA - Biologics JT - Biologics : targets & therapy JID - 101321511 PMC - PMC2721390 OTO - NOTNLM OT - B lymphocyte OT - fibrosis OT - systemic sclerosis EDAT- 2009/08/27 09:00 MHDA- 2009/08/27 09:01 PMCR- 2008/09/01 CRDT- 2009/08/27 09:00 PHST- 2009/08/27 09:00 [entrez] PHST- 2009/08/27 09:00 [pubmed] PHST- 2009/08/27 09:01 [medline] PHST- 2008/09/01 00:00 [pmc-release] AID - btt-2-389 [pii] PST - ppublish SO - Biologics. 2008 Sep;2(3):389-95. PMID- 18218275 OWN - NLM STAT- MEDLINE DCOM- 20080529 LR - 20191027 IS - 0210-5705 (Print) IS - 0210-5705 (Linking) VI - 31 IP - 1 DP - 2008 Jan TI - [Acute portal thrombosis in patients with hepatitis C treated with pegylated interferon and ribavirin]. PG - 18-21 AB - The combination of pegylated interferon and ribavirin is the first option treatment for chronic hepatitis C (HCC). Although not frequent, several vascular adverse events, such as Raynaud's phenomenon, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and central retinal vein occlusion, have been associated with the use of pegylated and standard interferon. We report the cases of two patients with HCC who, while receiving treatment with pegylated interferon and ribavirin, developed decompensated liver disease. Radiological tests revealed acute portal thrombosis. After the diagnosis was established, anticoagulant therapy was started, which resolved the portal thrombosis. FAU - Merino Rodríguez, Beatriz AU - Merino Rodríguez B AD - Sección de Hepatología, Servicio de Aparato Digestivo, Hospital General Universitario Gregorio Marañón, Madrid, España. FAU - Díaz Sánchez, Antonio AU - Díaz Sánchez A FAU - Matilla Peña, Ana AU - Matilla Peña A FAU - Clemente Ricote, Gerardo AU - Clemente Ricote G FAU - Núñez Martínez, Oscar AU - Núñez Martínez O LA - spa PT - Case Reports PT - Journal Article TT - Trombosis portal aguda en pacientes con hepatitis C crónica en tratamiento con interferón pegilado y ribavirina. PL - Spain TA - Gastroenterol Hepatol JT - Gastroenterologia y hepatologia JID - 8406671 RN - 0 (Antiviral Agents) RN - 0 (Interferon alpha-2) RN - 0 (Interferon-alpha) RN - 0 (Recombinant Proteins) RN - 3WJQ0SDW1A (Polyethylene Glycols) RN - 49717AWG6K (Ribavirin) RN - Q46947FE7K (peginterferon alfa-2a) SB - IM MH - Acute Disease MH - Adult MH - Antiviral Agents/*adverse effects MH - Hepatitis C, Chronic/*drug therapy MH - Humans MH - Interferon alpha-2 MH - Interferon-alpha/*adverse effects MH - Male MH - Polyethylene Glycols/*adverse effects MH - *Portal Vein MH - Recombinant Proteins MH - Ribavirin/*adverse effects MH - Thrombosis/*chemically induced EDAT- 2008/01/26 09:00 MHDA- 2008/05/30 09:00 CRDT- 2008/01/26 09:00 PHST- 2008/01/26 09:00 [pubmed] PHST- 2008/05/30 09:00 [medline] PHST- 2008/01/26 09:00 [entrez] AID - 13114566 [pii] AID - 10.1157/13114566 [doi] PST - ppublish SO - Gastroenterol Hepatol. 2008 Jan;31(1):18-21. doi: 10.1157/13114566. PMID- 17156671 OWN - NLM STAT- MEDLINE DCOM- 20080424 LR - 20061212 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 86 IP - 35 DP - 2006 Sep 19 TI - [Clinical features of undifferentiated connective tissue diseases: analysis of 145 patients]. PG - 2458-61 AB - OBJECTIVE: To investigate the clinical features and prognosis of undifferentiated connective tissue disease. (UCTD) METHODS: 1105 connective tissue disease (CTD) patients, including 751 cases of systemic lupus erythrematosus (SLE), 63 cases of systemic sclerosis (SSc), 103 cases of polymyositis/dermatomyositis (PM/DM), 159 cases of primary Sjögren's syndrome (pSS), and 29 cases of overlap syndrome (29), were randomly selected. The clinical data of these patients were analyzed to identify those who displayed the manifestations of UCTD as the onset manifestations so as to summarize the clinical manifestation, immunological parameters, and long term development of UCTD. RESULTS: These 145 patients with UCTD developed SLE, SSc, SS, PM/DM, or overlap syndrome within two to five years. The patients with arthritis and arthralgia often developed into SLE. Raynaud's syndrome was often related to SSc. The patients with rash or face edema were more likely turned out to be PM/DM patients. The patients with dry eyes or dry mouth often developed into pSS patients. CONCLUSION: UCTD can develop into various autoimmune diseases, such as SLE, SSc, pSS or PM/DM. Some clinical features of onset are related with the outcome. FAU - Zhang, Xue-wu AU - Zhang XW AD - Department of Rheumatology, People's Hospital of Beijing University, Beijing, China. FAU - Liu, Xu AU - Liu X FAU - Li, Zhan-guo AU - Li ZG LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 SB - IM MH - Adult MH - Aged MH - Connective Tissue Diseases/complications/*pathology MH - Disease Progression MH - Edema/etiology MH - Exanthema/etiology MH - Face MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/pathology MH - Male MH - Middle Aged MH - Sjogren's Syndrome/pathology EDAT- 2006/12/13 09:00 MHDA- 2008/04/25 09:00 CRDT- 2006/12/13 09:00 PHST- 2006/12/13 09:00 [pubmed] PHST- 2008/04/25 09:00 [medline] PHST- 2006/12/13 09:00 [entrez] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2006 Sep 19;86(35):2458-61. PMID- 37744621 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240925 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 11 IP - 9 DP - 2023 Sep TI - Erasmus syndrome: A rare case report of silicosis and systemic sclerosis. PG - e7935 LID - 10.1002/ccr3.7935 [doi] LID - e7935 AB - People with silicosis may develop Erasmus syndrome, a condition characterized by the emergence of systemic sclerosis (SSc) after silica exposure. This case study emphasizes the significance of understanding the connection between occupational silica exposure, silicosis, and SSc. A 24-year-old male stonecutter got silicosis and a form of SSc following 8 years on his job as a stonecutter. The signs and symptoms the patient experienced were Raynaud's phenomenon, cutaneous fibrosis, arthralgia, digital pitting, and respiratory distress. High-resolution computed tomography (HRCT) revealed interstitial lung disease and calcified mediastinal lymph nodes. This case study demonstrates the clinical importance of the relationship between occupational silica exposure, silicosis, SSc, and Erasmus syndrome. Healthcare providers need to be aware of the possible difficulties and issues that may result from silica exposure. They should prioritize quick detection and efficient treatment plans for those who have been exposed to silica while on the job. CI - © 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Rauniyar, Somee AU - Rauniyar S AUID- ORCID: 0009-0007-5466-3183 AD - Rangpur Medical College Rangpur Bangladesh. FAU - Thapa, Biyas AU - Thapa B AD - Rangpur Medical College Rangpur Bangladesh. FAU - Gupta, Prakash AU - Gupta P AUID- ORCID: 0000-0002-1267-3769 AD - Virgen Milagrosa University Foundation College of Medicine San Carlos City Philippines. FAU - Subedi, Rupak AU - Subedi R AD - Madan Bhandari Academy of Health Sciences Hetauda Nepal. FAU - Baral, Bishwodip AU - Baral B AD - Sukraraj Tropical and Infectious Disease Hospital Kathmandu Nepal. LA - eng PT - Case Reports PT - Journal Article DEP - 20230922 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC10517219 OTO - NOTNLM OT - occupational lung disease OT - scleroderma OT - silicosis OT - systemic sclerosis COIS- The authors have no conflict of interest to declare. EDAT- 2023/09/25 06:42 MHDA- 2023/09/25 06:43 PMCR- 2023/09/22 CRDT- 2023/09/25 04:46 PHST- 2023/07/11 00:00 [received] PHST- 2023/09/01 00:00 [revised] PHST- 2023/09/07 00:00 [accepted] PHST- 2023/09/25 06:43 [medline] PHST- 2023/09/25 06:42 [pubmed] PHST- 2023/09/25 04:46 [entrez] PHST- 2023/09/22 00:00 [pmc-release] AID - CCR37935 [pii] AID - 10.1002/ccr3.7935 [doi] PST - epublish SO - Clin Case Rep. 2023 Sep 22;11(9):e7935. doi: 10.1002/ccr3.7935. eCollection 2023 Sep. PMID- 17983453 OWN - NLM STAT- MEDLINE DCOM- 20080729 LR - 20220408 IS - 0307-6938 (Print) IS - 0307-6938 (Linking) VI - 33 IP - 1 DP - 2008 Jan TI - A review of the cutaneous paraneoplastic associations and metastatic presentations of ovarian carcinoma. PG - 10-5 AB - Ovarian carcinoma possesses cutaneous and paraneoplastic associations. The aim of this study was to review the paraneoplastic associations and metastatic presentations of ovarian carcinoma. PubMed was searched through December 2006 for references to cutaneous metastatic ovarian carcinoma (CMOC). CMOC occurs in 2-7% of cases, manifests in advanced disease and indicates a poor prognosis. The paraneoplastic associations of ovarian carcinoma include acanthosis nigricans, Raynaud's phenomenon, scleroderma, dermatomyositis and palmar fasciitis with polyarthritis. Dermatomyositis, in particular, can precede the diagnosis of ovarian carcinoma. Ovarian carcinoma has many cutaneous paraneoplastic effects and metastatic presentations, all of which portend a poor prognosis. Dermatomyositis is sometimes the initial manifestation of ovarian cancer, thus women > 40 years of age with dermatomyositis should be checked for ovarian carcinoma. It is possible that paraneoplastic dermtomyosititis can be distinguished from nonparaneoplastic dermatomyostitis by the former's lack of (i) associated Raynaud's phenomenon, (ii) response to treatment, (iii) autoantibodies, (iv) overlap and association with other collagen vascular diseases and (v) the presence of the prodromal symptoms of ovarian carcinoma such as gastrointestinal symptoms, urinary symptoms and/or fatigue or malaise. FAU - Scheinfeld, N AU - Scheinfeld N AD - Department of Dermatology, Columbia University School of Medicine, St Luke's Roosevelt Hospital Center, New York, NY, USA. scheinfeld@earthlink.net LA - eng PT - Journal Article PT - Review DEP - 20071103 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 SB - IM MH - Adult MH - Dermatomyositis/pathology MH - Female MH - Humans MH - Middle Aged MH - Ovarian Neoplasms/*pathology MH - Paraneoplastic Syndromes/classification/*pathology MH - Skin Neoplasms/classification/*secondary RF - 31 EDAT- 2007/11/07 09:00 MHDA- 2008/07/30 09:00 CRDT- 2007/11/07 09:00 PHST- 2007/11/07 09:00 [pubmed] PHST- 2008/07/30 09:00 [medline] PHST- 2007/11/07 09:00 [entrez] AID - CED2560 [pii] AID - 10.1111/j.1365-2230.2007.02560.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2008 Jan;33(1):10-5. doi: 10.1111/j.1365-2230.2007.02560.x. Epub 2007 Nov 3. PMID- 22137282 OWN - NLM STAT- MEDLINE DCOM- 20120405 LR - 20161125 IS - 1776-2561 (Electronic) IS - 0761-8417 (Linking) VI - 67 IP - 6 DP - 2011 Dec TI - [Acute interstitial lung disease revealing antisynthetase syndrome]. PG - 367-70 LID - 10.1016/j.pneumo.2011.01.003 [doi] AB - INTRODUCTION: The antisynthetase syndrome is characterized by the presence of myositis, interstitial lung disease, arthritis, Raynaud's phenomenon, mechanics hands and anti-Jo1 antibody (histidyl tRNA synthetase). The prognosis of this syndrome is closely related to the severity of lung disease. Myositis can occur several years after lung disease and some patients with interstitial lung disease associated with anti-Jo1 antibodies will not suffer from muscle disease. CASE-REPORT: We report the case of a 69-year-old man admitted to the medical intensive care unit for acute respiratory insufficiency related to rapidly progressive interstitial lung disease. Antisynthetase syndrome was diagnosed the presence of wrists' arthritis, 'mechanic's hands and anti-Jo1 antibodies. Despite the dramatic efficacy of corticosteroid therapy on ventilation parameters, the patient died from a Pseudomonas Aeruginosa nosocomial ventilator-acquired pneumonia. CONCLUSION: Our case emphasizes the importance to search for anti-Jo1 antibodies in the presence of interstitial lung disease. During the course of antisynthetase syndrome, the occurrence of interstitial lung disease is almost always constant and is correlated with poor prognosis. CI - Copyright © 2011 Elsevier Masson SAS. All rights reserved. FAU - Bizien, N AU - Bizien N AD - Département de médecine interne et pneumologie, CHU de Brest, France. nicolas.bizien@chu-brest.fr FAU - Renault, A AU - Renault A FAU - Boles, J-M AU - Boles JM FAU - Delluc, A AU - Delluc A LA - fre PT - Case Reports PT - Journal Article TT - Pneumopathie interstitielle aiguë révélatrice d'un syndrome des antisynthétases. DEP - 20110429 PL - France TA - Rev Pneumol Clin JT - Revue de pneumologie clinique JID - 8406312 RN - Antisynthetase syndrome SB - IM MH - Acute Disease MH - Aged MH - Diagnosis, Differential MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/diagnostic imaging/etiology MH - Male MH - Myositis/complications/*diagnosis/diagnostic imaging MH - Radiography, Thoracic EDAT- 2011/12/06 06:00 MHDA- 2012/04/06 06:00 CRDT- 2011/12/06 06:00 PHST- 2010/07/22 00:00 [received] PHST- 2011/01/13 00:00 [revised] PHST- 2011/01/19 00:00 [accepted] PHST- 2011/12/06 06:00 [entrez] PHST- 2011/12/06 06:00 [pubmed] PHST- 2012/04/06 06:00 [medline] AID - S0761-8417(11)00028-9 [pii] AID - 10.1016/j.pneumo.2011.01.003 [doi] PST - ppublish SO - Rev Pneumol Clin. 2011 Dec;67(6):367-70. doi: 10.1016/j.pneumo.2011.01.003. Epub 2011 Apr 29. PMID- 33407281 OWN - NLM STAT- MEDLINE DCOM- 20210113 LR - 20210113 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 21 IP - 1 DP - 2021 Jan 6 TI - Anti-synthetase syndrome: a rare and challenging diagnosis for bilateral ground-glass opacities-a case report with literature review. PG - 11 LID - 10.1186/s12890-020-01388-0 [doi] LID - 11 AB - BACKGROUND: Anti-synthetase syndrome (ASS) is an uncommon immune-mediated entity characterized by myositis, interstitial lung disease (ILD), non-erosive arthritis, and less common features such as fever, Raynaud's phenomenon, and skin changes in association with anti-aminoacyl-transfer-RNA antibodies, most commonly anti-Jo-1 antibodies. CASE PRESENTATION: We present a challenging and rare case of ASS-associated ILD presenting with unexplained respiratory symptoms and bilateral infiltrates on chest imaging during the COVID-19 pandemic. High clinical suspicion for ASS with early appropriate therapy with corticosteroids and immunosuppressive agents led to marked clinical improvement. CONCLUSION: High index of suspicion for ASS is mandated in patients with unexplained ILD. A comprehensive autoimmune work-up is important as an early treatment with corticosteroids with or without immunomodulators improves patient outcomes and survival in an otherwise poor prognostic disease. FAU - Alfraji, Nasam AU - Alfraji N AD - Department of Internal Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, 07753, USA. nasam.alfraji@hmhn.org. FAU - Mazahir, Usman AU - Mazahir U AD - Department of Pulmonology and Critical Care, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA. FAU - Chaudhri, Moiuz AU - Chaudhri M AD - Department of Pulmonology and Critical Care, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA. FAU - Miskoff, Jeffrey AU - Miskoff J AD - Department of Pulmonology and Critical Care, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210106 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/*immunology MH - COVID-19/*epidemiology MH - Comorbidity MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung/*diagnostic imaging MH - Middle Aged MH - Myositis/*diagnosis/epidemiology/immunology MH - *Pandemics MH - Prognosis MH - *Rare Diseases MH - *SARS-CoV-2 PMC - PMC7787399 OTO - NOTNLM OT - Anti-synthetase syndrome OT - Autoimmune disease OT - Corticosteroids OT - Interstitial lung disease COIS- The authors declare that they have no competing interests. EDAT- 2021/01/08 06:00 MHDA- 2021/01/14 06:00 PMCR- 2021/01/06 CRDT- 2021/01/07 05:44 PHST- 2020/12/18 00:00 [received] PHST- 2020/12/21 00:00 [accepted] PHST- 2021/01/07 05:44 [entrez] PHST- 2021/01/08 06:00 [pubmed] PHST- 2021/01/14 06:00 [medline] PHST- 2021/01/06 00:00 [pmc-release] AID - 10.1186/s12890-020-01388-0 [pii] AID - 1388 [pii] AID - 10.1186/s12890-020-01388-0 [doi] PST - epublish SO - BMC Pulm Med. 2021 Jan 6;21(1):11. doi: 10.1186/s12890-020-01388-0. PMID- 29507032 OWN - NLM STAT- MEDLINE DCOM- 20180913 LR - 20200305 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2018 DP - 2018 Mar 5 TI - Following leads: connecting dysphagia to mixed connective tissue disease. LID - bcr-2017-223699 [pii] LID - 10.1136/bcr-2017-223699 [doi] LID - bcr2017223699 AB - Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis. Also, a subset of patients evolve into a clinical picture more consistent with a major diffuse connective tissue disease. We present the case of a 50-year-old black woman whose inaugural presentation of MCDT was oropharyngeal dysphagia, symmetrical proximal muscle weakness, tongue atrophy and skin sclerosis. High-dose corticosteroids and methotrexate were given with little improvement, maintaining disabling dysphagia leading to a percutaneous endoscopic gastrostomy tube placement. She was then started on intravenous immunoglobulin with progressive remission of symptoms. CI - © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. FAU - Gameiro, Rita de Sousa AU - Gameiro RS AD - Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal. FAU - Reis, Ana Isabel Alves AU - Reis AIA AD - Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal. FAU - Grilo, Ana Cristina AU - Grilo AC AD - Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal. FAU - Noronha, Carla AU - Noronha C AD - Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20180305 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Deglutition Disorders/*etiology MH - Delayed Diagnosis MH - *Disease Progression MH - Female MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications/*diagnosis/drug therapy MH - Muscle Weakness/etiology PMC - PMC5847916 OTO - NOTNLM OT - connective tissue disease OT - medical management OT - oesophagus COIS- Competing interests: None declared. EDAT- 2018/03/07 06:00 MHDA- 2018/09/14 06:00 PMCR- 2020/03/05 CRDT- 2018/03/07 06:00 PHST- 2018/03/07 06:00 [entrez] PHST- 2018/03/07 06:00 [pubmed] PHST- 2018/09/14 06:00 [medline] PHST- 2020/03/05 00:00 [pmc-release] AID - bcr-2017-223699 [pii] AID - 10.1136/bcr-2017-223699 [doi] PST - epublish SO - BMJ Case Rep. 2018 Mar 5;2018:bcr2017223699. doi: 10.1136/bcr-2017-223699. PMID- 23486724 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130317 LR - 20211021 IS - 2074-1804 (Print) IS - 2074-1812 (Electronic) IS - 2074-1804 (Linking) VI - 15 IP - 1 DP - 2013 Jan TI - Serum homocystein level in patients with scleroderma. PG - 29-31 LID - 10.5812/ircmj.3672 [doi] AB - BACKGROUND: Systemic Sclerosis (SSc) is a systemic connective tissue disease. In this study, we compared the serum Homocystein (Hcy) level between patients with SSc and normal control group. OBJECTIVES: The current study was conducted to determine whether serum Hcy levels are elevated in SSc patients and whether there is any correlation between Hcy levels and RP, Gastro intestinal and lung involvement. PATIENTS AND METHODS: Forty one patients who fulfilled the diagnostic criteria for SSc (39 females and 5 males) and Forty four community-based healthy individuals (sex and age matched) were enrolled in to the study. Serum Hcy, vitamin B12, and folate levels were determined. RESULTS: Thirty three patients (70.45%) had GI involvement, twenty two patients (50%) had lung involvement and twenty seven patients (61.36%) had Raynaud's phenomena. Mean serum Hcy level in control group was 22.78 ± 6.018 μmol/L and in case group was 19.43 ± 7.205 μmol/L, shows that the serum Hcy level in control group was significantly higher than patients (P = 0.020). CONCLUSIONS: Serum Hcy level is significantly lower in SSc patients than in control group. There is no statistically significant correlation between serum Hcy level and organ involvements. FAU - Nazarinia, Mohammadali AU - Nazarinia M AD - Shiraz University of Medical Sciences, Shiraz, IR Iran. FAU - Shams, Mesbah AU - Shams M FAU - Kamali Sarvestani, Eskandar AU - Kamali Sarvestani E FAU - Shenavande, Saeede AU - Shenavande S FAU - Khademalhosseini, Maryam AU - Khademalhosseini M FAU - Khademalhosseini, Zeinab AU - Khademalhosseini Z LA - eng PT - Journal Article DEP - 20130105 PL - Estonia TA - Iran Red Crescent Med J JT - Iranian Red Crescent medical journal JID - 101319850 PMC - PMC3589775 OTO - NOTNLM OT - Autoimmunity OT - Homocysteine OT - Raynauds Disease OT - Scleroderma, Systemic EDAT- 2013/03/15 06:00 MHDA- 2013/03/15 06:01 PMCR- 2013/01/01 CRDT- 2013/03/15 06:00 PHST- 2011/11/30 00:00 [received] PHST- 2012/04/27 00:00 [revised] PHST- 2012/05/19 00:00 [accepted] PHST- 2013/03/15 06:00 [entrez] PHST- 2013/03/15 06:00 [pubmed] PHST- 2013/03/15 06:01 [medline] PHST- 2013/01/01 00:00 [pmc-release] AID - 10.5812/ircmj.3672 [doi] PST - ppublish SO - Iran Red Crescent Med J. 2013 Jan;15(1):29-31. doi: 10.5812/ircmj.3672. Epub 2013 Jan 5. PMID- 42261453 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260609 LR - 20260609 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 6 DP - 2026 Jun TI - Atypical Cutaneous Manifestation Leading to the Diagnosis of Chronic Lymphocytic Leukemia. PG - e110370 LID - 10.7759/cureus.110370 [doi] LID - e110370 AB - We report a 64-year-old male with rheumatoid arthritis and Raynaud's phenomenon who presented with diffuse arthralgias and progressive ulcerating lesions involving the palms and elbows. Differential diagnoses included recurrent Rickettsial infection, vasculitis, pyoderma gangrenosum, and cutaneous tuberculosis. Laboratory studies demonstrated marked leukocytosis (135.4 ×10⁹/L), while computed tomography (CT) imaging revealed hepatomegaly, lymphadenopathy, and pulmonary micronodules concerning for infiltrative disease. Additional history revealed a 40-pound unintentional weight loss over three months. Peripheral flow cytometry established a new diagnosis of chronic lymphocytic leukemia (CLL), and skin punch biopsy demonstrated epidermal necrosis with dense dermal lymphoid infiltrates consistent with leukemia cutis. This case highlights leukemia cutis as a rare initial manifestation of CLL that may mimic infectious or autoimmune conditions, emphasizing the importance of thorough evaluation of unexplained cutaneous lesions and significant leukocytosis to facilitate earlier diagnosis and treatment consideration. CI - Copyright © 2026, Memon et al. FAU - Memon, Muhammad AU - Memon M AD - Internal Medicine, Christiana Care Health System, Newark, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20260606 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13242892 OTO - NOTNLM OT - chronic lymphocytic leukemia OT - clinical hematology OT - dermatology OT - diffuse rash OT - hematology-oncology OT - skin disease COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/06/09 06:34 MHDA- 2026/06/09 06:35 PMCR- 2026/06/06 CRDT- 2026/06/09 03:56 PHST- 2026/06/06 00:00 [accepted] PHST- 2026/06/09 06:35 [medline] PHST- 2026/06/09 06:34 [pubmed] PHST- 2026/06/09 03:56 [entrez] PHST- 2026/06/06 00:00 [pmc-release] AID - 10.7759/cureus.110370 [doi] PST - epublish SO - Cureus. 2026 Jun 6;18(6):e110370. doi: 10.7759/cureus.110370. eCollection 2026 Jun. PMID- 31363430 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 11 IP - 5 DP - 2019 May 24 TI - Anxiety, Depression, and Obsessive-compulsive Disorder in a Recently Diagnosed Case of Systemic Sclerosis. PG - e4748 LID - 10.7759/cureus.4748 [doi] LID - e4748 AB - Systemic sclerosis is an autoimmune condition that frequently affects women. It is a progressive, debilitating disease that has widespread manifestations, targeting different organs of the body with potentially fatal consequences due to lung and kidney involvement. Women with this disease mostly present with Raynaud's phenomenon along with symptoms of gastro-esophageal reflux disease (GERD). Just like any chronic debilitating condition, patients with systemic sclerosis often suffer from mental health issues that can further worsen their condition, significantly affecting their quality of life. Further research regarding the effects and severity of the disease should be encouraged for a better understanding of the illness, its diagnosis, and treatment. We present a rare case of a 55-year-old woman who presented with complaints of a major depressive episode. She was diagnosed with systemic sclerosis last year and has a history of generalized anxiety disorder. She was prescribed Mirtazapine, an antidepressant. On her follow-up after one month, she started complaining of obsessive ruminations that were causing her significant distress. She was prescribed an add-on therapy with a selective serotonin reuptake inhibitor (SSRI) antidepressant with the emphasis being placed on cognitive behavioral therapy (CBT). She reported an improvement in her obsessive symptoms as well as depression after two months. FAU - Khan, Omair A AU - Khan OA AD - Internal Medicine, Fauji Foundation Hospital, Rawalpindi, PAK. FAU - Majeed, Ramsha AU - Majeed R AD - Internal Medicine, Fauji Foundation Hospital, Rawalpindi, PAK. FAU - Kamal, Kaynat AU - Kamal K AD - Public Health, Health Services Academy, Quaid-E-Azam University, Islamabad, PAK. FAU - Sherazi, Mahnoor AU - Sherazi M AD - Internal Medicine, Fauji Foundation Hospital, Rawalpindi, PAK. FAU - Saad, Muhammad AU - Saad M AD - Internal Medicine, Fauji Foundation Hospital, Rawalpindi, PAK. LA - eng PT - Case Reports PT - Journal Article DEP - 20190524 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC6663282 OTO - NOTNLM OT - anxiety OT - depression OT - obsessive compulsive disorder OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2019/08/01 06:00 MHDA- 2019/08/01 06:01 PMCR- 2019/05/24 CRDT- 2019/08/01 06:00 PHST- 2019/08/01 06:00 [entrez] PHST- 2019/08/01 06:00 [pubmed] PHST- 2019/08/01 06:01 [medline] PHST- 2019/05/24 00:00 [pmc-release] AID - 10.7759/cureus.4748 [doi] PST - epublish SO - Cureus. 2019 May 24;11(5):e4748. doi: 10.7759/cureus.4748. PMID- 36727567 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230315 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 5 IP - 3 DP - 2023 Mar TI - Qualitative Interviews to Assess the Content Validity and Usability of the Electronic Raynaud Diary in Patients with Systemic Sclerosis. PG - 132-141 LID - 10.1002/acr2.11522 [doi] AB - OBJECTIVE: To better understand the symptoms and impacts of Raynaud phenomenon (RP) in patients with systemic sclerosis (SSc) and to evaluate the content validity and usability of a new electronic patient-reported outcome (PRO) measure for RP: the Raynaud Diary. METHODS: The Raynaud Diary was developed as a daily eDiary for assessing the number and duration of symptomatic Raynaud attacks; worst pain, numbness, tingling, and discomfort in the fingers; and overall disease severity, captured using the Raynaud's Condition Score. The Raynaud Diary was debriefed in two waves of qualitative interviews with adults with self-reported RP secondary to SSc. All interviews included open-ended questions about participants' experiences of RP. RESULTS: Participants (N = 39) had a mean age of 55.1 years, and 87% were female. Frequently reported RP symptoms were color change (reported by all participants), numbness (90%), tingling (82%), pain (77%), and discomfort (72%). Common attack triggers included temperature-related factors and stress. Participants reported being unable to be outside or do outdoor activities and had problems gripping objects. All participants demonstrated understanding of the Raynaud Diary instructions. Most participants indicated that they would be able to use the Raynaud Diary to record the worst severity of individual RP symptoms in the previous 24 hours. CONCLUSION: Patients with RP secondary to SSc bear a heavy symptom burden. The Raynaud Diary is a content valid PRO measure that captures the most frequent symptoms of RP in patients with SSc. CI - © 2023 Eicos Sciences, Inc. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Domsic, Robyn T AU - Domsic RT AUID- ORCID: 0000-0002-2765-0922 AD - University of Pittsburgh, Pennsylvania, Pittsburgh. FAU - Pokrzywinski, Robin AU - Pokrzywinski R AD - Evidera, Maryland, Bethesda. FAU - Stassek, Larissa AU - Stassek L AD - Evidera, Maryland, Bethesda. FAU - Benton, Wade W AU - Benton WW AD - Eicos Sciences, Inc, California, San Mateo. FAU - Vampola, Christa-Lynn AU - Vampola CL AD - Eicos Sciences, Inc, California, San Mateo. FAU - Furst, Daniel E AU - Furst DE AUID- ORCID: 0000-0001-7857-2373 AD - University of California, Los Angeles, California. FAU - Chung, Lorinda AU - Chung L AD - Stanford University, California, Palo Alto. FAU - Steen, Virginia AU - Steen V AD - Georgetown University, D.C, Washington. FAU - Mayes, Maureen D AU - Mayes MD AUID- ORCID: 0000-0001-5070-2535 AD - University of Texas Health Science Center, Houston, Texas. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University School of Medicine, Maryland, Baltimore. FAU - Molitor, Jerry A AU - Molitor JA AD - University of Minnesota, Minneapolis, Minnesota. FAU - Oliver, Kelly AU - Oliver K AD - Eicos Sciences, Inc, California, San Mateo. FAU - Nagaraja, Vivek AU - Nagaraja V AUID- ORCID: 0000-0002-4930-3200 AD - University of Michigan Scleroderma Program, Ann Arbor, Michigan. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Eicos Sciences, Inc., San Mateo, California, and University of Michigan Scleroderma Program, Ann Arbor, Michigan. LA - eng GR - Eicos Sciences, Inc./ PT - Journal Article DEP - 20230202 PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC10010488 EDAT- 2023/02/03 06:00 MHDA- 2023/02/03 06:01 PMCR- 2023/02/02 CRDT- 2023/02/02 05:42 PHST- 2022/08/17 00:00 [revised] PHST- 2022/03/30 00:00 [received] PHST- 2022/09/04 00:00 [accepted] PHST- 2023/02/03 06:00 [pubmed] PHST- 2023/02/03 06:01 [medline] PHST- 2023/02/02 05:42 [entrez] PHST- 2023/02/02 00:00 [pmc-release] AID - ACR211522 [pii] AID - 10.1002/acr2.11522 [doi] PST - ppublish SO - ACR Open Rheumatol. 2023 Mar;5(3):132-141. doi: 10.1002/acr2.11522. Epub 2023 Feb 2. PMID- 26524895 OWN - NLM STAT- MEDLINE DCOM- 20160211 LR - 20151103 IS - 0047-1860 (Print) IS - 0047-1860 (Linking) VI - 63 IP - 5 DP - 2015 May TI - [Progress of Autoantibody Examinations for Connective Tissue Diseases]. PG - 562-9 AB - Connective tissue diseases are chronic inflammatory diseases that can affect multiple organs and, thus, have a broad spectrum of clinical presentations. Various autoantibodies are detected in patients with connective tissue diseases, represented by anti-nuclear antibody for systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), Sjögren's syndrome, and mixed connective tissue disease. Assessment of the autoantibody profile is fundamental for the clinical management of patients with connective tissue diseases, providing important data for the diagnosis, clinical characterization, and disease activity evaluation. Anti-ribosomal P antibody and anti-NMDA receptor antibody are associated with neuropsychiatric SLE. Anti-synthetase syndrome comprises the association of myositis (PM/DM), interstitial lung disease, fever, Raynaud's phenomenon, mechanic's hands, and anti-aminoacyl tRNA synthetase antibodies. Anti-MDA5 antibody is detected in patients with clinically amyopathic DM, often complicated by rapidly progressive interstitial lung disease. Between 50 and 75% of malignancy-associated DM patients are positive for anti-TIF1-γ antibody. Anti-RNA polymerase III antibody is associated with diffuse cutaneous SSc and renal crisis. This review focuses on the importance and usefulness of these autoantibodies for the diagnosis and management of patients with connective tissue diseases in clinical practice. FAU - Akashi, Kengo AU - Akashi K FAU - Saegusa, Jun AU - Saegusa J FAU - Morinobu, Akio AU - Morinobu A LA - jpn PT - English Abstract PT - Journal Article PT - Review PL - Japan TA - Rinsho Byori JT - Rinsho byori. The Japanese journal of clinical pathology JID - 2984781R RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Connective Tissue Diseases/*diagnosis/*immunology MH - Humans EDAT- 2015/11/04 06:00 MHDA- 2016/02/13 06:00 CRDT- 2015/11/04 06:00 PHST- 2015/11/04 06:00 [entrez] PHST- 2015/11/04 06:00 [pubmed] PHST- 2016/02/13 06:00 [medline] PST - ppublish SO - Rinsho Byori. 2015 May;63(5):562-9. PMID- 22083296 OWN - NLM STAT- MEDLINE DCOM- 20120508 LR - 20211021 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 14 IP - 1 DP - 2012 Feb TI - Angiogenesis and vasculopathy in systemic sclerosis: evolving concepts. PG - 56-63 LID - 10.1007/s11926-011-0219-1 [doi] AB - Systemic sclerosis (scleroderma [SSc]) is a multifactorial disease characterized by inflammation, extensive and progressive fibrosis, and multiple vasculopathies. The vascular manifestations can be seen early in the pathogenesis of the disease and include malformed capillaries, Raynaud's phenomenon, and digital ulcers. As the disease progresses, the vasculopathy proceeds to significant clinical manifestations, including renal crisis and pulmonary arterial hypertension. Moreover, later stages of the disease are marked by increasingly avascular areas. Despite the obliteration of microvascular structures, compensatory vasculogenesis and angiogenesis do not occur normally. This is in spite of a general increase in many potent angiogenic factors. Recent studies are beginning to examine this paradox and subsequent paucity of an angiogenic response in SSc. In this review, we discuss these findings and examine the role that chemokine and growth factor receptors, proteases, adhesion molecules, and transcription factors play in the dysregulation of angiogenesis in SSc. FAU - Rabquer, Bradley J AU - Rabquer BJ AD - Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI 48109-2200, USA. brabquer@med.umich.edu FAU - Koch, Alisa E AU - Koch AE LA - eng GR - HL094017/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 SB - IM MH - Humans MH - Neovascularization, Pathologic/*etiology MH - Peripheral Vascular Diseases/*etiology MH - Scleroderma, Systemic/*complications EDAT- 2011/11/16 06:00 MHDA- 2012/05/09 06:00 CRDT- 2011/11/16 06:00 PHST- 2011/11/16 06:00 [entrez] PHST- 2011/11/16 06:00 [pubmed] PHST- 2012/05/09 06:00 [medline] AID - 10.1007/s11926-011-0219-1 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2012 Feb;14(1):56-63. doi: 10.1007/s11926-011-0219-1. PMID- 21455765 OWN - NLM STAT- MEDLINE DCOM- 20110830 LR - 20220408 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 13 IP - 3 DP - 2011 Jun TI - Antisynthetase syndrome. PG - 175-81 LID - 10.1007/s11926-011-0176-8 [doi] AB - Autoantibodies to eight of the aminoacyl-transfer RNA synthetases-the most well-recognized of which is anti-histidyl (Jo-1)-have all been implicated in the pathogenesis of antisynthetase syndrome (AS). AS is characterized by varying degrees of interstitial lung disease, myositis, arthropathy, fever, Raynaud's phenomenon, and mechanic's hands, and the morbidity and mortality of the disease are usually linked to the pulmonary findings. The value of a lung biopsy in AS cannot be overemphasized, as it serves to describe the underlying etiology of the interstitial lung disease, guide therapy, and estimate prognosis. Muscle disease shares many clinical features of polymyositis, yet histologically, the inflammatory involvement resembles that of dermatomyositis. Because inflammatory arthritis mimics rheumatoid arthritis, AS should be considered in atypical cases. Corticosteroids are the mainstay of acute therapy, although treatment often requires immunosuppressant medications such as cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, or rituximab. FAU - Katzap, Elena AU - Katzap E AD - Division of Rheumatology and Allergy-Clinical Immunology, North Shore Long Island Jewish Health System, 2800 Marcus Avenue, Suite 200, Lake Success, NY 11042, USA. ebogach@nshs.edu FAU - Barilla-LaBarca, Maria-Louise AU - Barilla-LaBarca ML FAU - Marder, Galina AU - Marder G LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - 0 (Antibodies, Antinuclear) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Antibodies, Antinuclear/*immunology MH - Arthritis/immunology/pathology MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung/pathology MH - Lung Diseases, Interstitial/immunology/pathology MH - Myositis/drug therapy/immunology/pathology EDAT- 2011/04/02 06:00 MHDA- 2011/08/31 06:00 CRDT- 2011/04/02 06:00 PHST- 2011/04/02 06:00 [entrez] PHST- 2011/04/02 06:00 [pubmed] PHST- 2011/08/31 06:00 [medline] AID - 10.1007/s11926-011-0176-8 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2011 Jun;13(3):175-81. doi: 10.1007/s11926-011-0176-8. PMID- 18789123 OWN - NLM STAT- MEDLINE DCOM- 20081103 LR - 20181201 IS - 1744-9987 (Electronic) IS - 1744-9979 (Linking) VI - 12 IP - 4 DP - 2008 Aug TI - A case report of successful treatment with immunoadsorption onto protein A in mixed connective tissue disease in childhood. PG - 337-42 LID - 10.1111/j.1744-9987.2008.00597.x [doi] AB - An 11-year-old male patient suffering mixed connective tissue disease with life-threatening pulmonary arterial hypertension, progressive heart failure (New York Heart Association class III-IV), skin ulcers, Raynaud's phenomenon and arthritis, showing no improvement after intensive immunosuppressive therapy or high dose steroids, was treated with immunoadsorption onto protein A. With a combined therapy of low-dose cortisone and bosentan and 22 sessions of immunoadsorption, his condition improved significantly and he continues in clinical remission. At the time of writing no further immunosuppressive therapy or immunoadsorption had been necessary. The patient is now 15 years old and healthy with an age-based constitution comparable to the normal population. FAU - Rummler, Silke AU - Rummler S AD - Institute of Transfusion Medicine, University Hospital, Jena, Germany. silke.rummler@med.uni-jena.de FAU - Althaus, Karina AU - Althaus K FAU - Maak, Bernhard AU - Maak B FAU - Barz, Dagmar AU - Barz D LA - eng PT - Case Reports PT - Journal Article PL - Australia TA - Ther Apher Dial JT - Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy JID - 101181252 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antihypertensive Agents) RN - 0 (Staphylococcal Protein A) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) RN - V27W9254FZ (Cortisone) SB - IM MH - Adolescent MH - Anti-Inflammatory Agents/therapeutic use MH - Antihypertensive Agents/therapeutic use MH - Bosentan MH - Child MH - Combined Modality Therapy MH - Cortisone/therapeutic use MH - Humans MH - Immunosorbent Techniques MH - Male MH - Mixed Connective Tissue Disease/immunology/*therapy MH - Remission Induction/methods MH - *Staphylococcal Protein A MH - Sulfonamides/therapeutic use EDAT- 2008/09/16 09:00 MHDA- 2008/11/04 09:00 CRDT- 2008/09/16 09:00 PHST- 2008/09/16 09:00 [pubmed] PHST- 2008/11/04 09:00 [medline] PHST- 2008/09/16 09:00 [entrez] AID - TAP597 [pii] AID - 10.1111/j.1744-9987.2008.00597.x [doi] PST - ppublish SO - Ther Apher Dial. 2008 Aug;12(4):337-42. doi: 10.1111/j.1744-9987.2008.00597.x. PMID- 32851373 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220416 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 35 IP - 2 DP - 2020 Jun TI - Association of Interstitial Lung Disease With Clinical Characteristics of Chinese Patients With Systemic Lupus Erythematosus. PG - 239-246 LID - 10.46497/ArchRheumatol.2020.7583 [doi] AB - OBJECTIVES: This study aims to evaluate the frequency and clinical and laboratory features of interstitial lung disease (ILD) in Chinese patients with systemic lupus erythematosus (SLE) and to evaluate the association of ILD with the clinical features. PATIENTS AND METHODS: The study included 505 SLE patients (64 males, 441 females; mean age 35.3±15.3 years; range, 14 to 87 years) who were categorized into two groups as 449 patients without ILD and 56 patients with ILD based on evidence obtained from high-resolution computed tomography images. The demographic data, clinical and laboratory findings, SLE disease activity index score, and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index of all patients were also recorded and statistically analyzed. RESULTS: The ILD frequency in patients with SLE was 11.1%. Compared to the group of SLE patients without ILD, the group of SLE patients with ILD possessed the following statistical differences: elderly age, longer illness duration, lower level of anti-double-stranded deoxyribonucleic acid, and higher level of serum complement 3, increased ratios of Raynaud's phenomenon, moist rales and tachypnea. Multivariate logistic regression results suggested that elderly age (≥60 years), long illness duration (1-10 years, ≥10 years), Raynaud's phenomenon, and tachypnea were statistically associated with the occurrence of ILD in SLE patients. CONCLUSION: Chinese SLE patients who possessed the factors that were statistically associated with ILD, namely, elderly age (≥60 years old), long illness duration (≥1 years), Raynaud's phenomenon, and tachypnea, were recommended to be monitored for the possibility of ILD. CI - Copyright © 2020, Turkish League Against Rheumatism. FAU - Chen, Yaling AU - Chen Y AUID- ORCID: 0000-0002-4320-7664 AD - Department of Rheumatology, Fujian Medical University Union Hospital, Fuzhou, China. FAU - Wang, Yanqing AU - Wang Y AUID- ORCID: 0000-0002-3331-1206 AD - Department of Rheumatology, Fujian Medical University Union Hospital, Fuzhou, China. FAU - Chen, Xiangfang AU - Chen X AUID- ORCID: 0000-0001-6449-9267 AD - Department of Rheumatology, Fujian Medical University Union Hospital, Fuzhou, China. FAU - Liang, Huishun AU - Liang H AUID- ORCID: 0000-0002-9308-0999 AD - Department of Radiology, Fujian Medical University Union Hospital, Fuzhou, China. FAU - Yang, Xuwei AU - Yang X AUID- ORCID: 0000-0002-0904-2763 AD - Department of Rheumatology, Fujian Medical University Union Hospital, Fuzhou, China. LA - eng PT - Journal Article DEP - 20200108 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC7406170 OTO - NOTNLM OT - Interstitial lung disease OT - systemic lupus erythematosus COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2020/08/28 06:00 MHDA- 2020/08/28 06:01 PMCR- 2020/01/08 CRDT- 2020/08/28 06:00 PHST- 2019/05/01 00:00 [received] PHST- 2019/07/16 00:00 [accepted] PHST- 2020/08/28 06:00 [entrez] PHST- 2020/08/28 06:00 [pubmed] PHST- 2020/08/28 06:01 [medline] PHST- 2020/01/08 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2020.7583 [doi] PST - epublish SO - Arch Rheumatol. 2020 Jan 8;35(2):239-246. doi: 10.46497/ArchRheumatol.2020.7583. eCollection 2020 Jun. PMID- 39027693 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240720 IS - 1642-395X (Print) IS - 2299-0046 (Electronic) IS - 1642-395X (Linking) VI - 41 IP - 3 DP - 2024 Jun TI - The usefulness of nailfold capillaroscopy in scleroderma-spectrum disorders: a single-centre observational study. PG - 296-300 LID - 10.5114/ada.2024.141158 [doi] AB - INTRODUCTION: Nail-fold capillaroscopy is a non-invasive method for assessment of the microcirculation in nail folds. This examination is particularly useful for diagnosis, assessment of activity, evaluation of the response to treatment, and assessment of the correlation of changes in microvessels with changes in organs in systemic sclerosis and in scleroderma-spectrum diseases, i.e. dermatomyositis, polymyositis, mixed connective tissue disease, and undifferentiated connective tissue disease. AIM: To perform capillaroscopic analyses of lesions in patients with scleroderma-spectrum diseases and determine the correlation of the capillaroscopic image with organ manifestations and the serological profile. MATERIAL AND METHODS: The study involved 15 patients with scleroderma-spectrum disorders. RESULTS: Mixed systemic connective tissue disease was diagnosed in 8 patients, and dermatomyositis was detected in 7 patients. The study assessed the frequency of clinical symptoms, e.g. interstitial lung disease or arthritis, and the presence of ANA antibodies. Scleroderma-like microangiopathy was diagnosed in 47% of patients with scleroderma-spectrum disorders. The early pattern was found in patients with mixed systemic connective tissue disease, whereas dermatomyositis was characterized by the late pattern. Non-specific changes were found in 27% of the patients, and a normal image was observed in 27% of the patients. CONCLUSIONS: The analysis also revealed that the reduced number of vessels correlated with the occurrence of interstitial lung disease, and the incidence of Raynaud's phenomenon and arthritis was statistically significantly higher in patients with systemic connective tissue disease than in those with dermatomyositis. CI - Copyright: © 2024 Termedia Sp. z o. o. FAU - Wiąk-Walerowicz, Katarzyna AU - Wiąk-Walerowicz K AD - Department of Rheumatology and Systemic Connective Tissue Diseases, Medical University of Lublin, Lublin, Poland. FAU - Wielosz, Ewa AU - Wielosz E AD - Department of Rheumatology and Systemic Connective Tissue Diseases, Medical University of Lublin, Lublin, Poland. FAU - Majdan, Maria AU - Majdan M AD - Department of Rheumatology and Systemic Connective Tissue Diseases, Medical University of Lublin, Lublin, Poland. LA - eng PT - Journal Article DEP - 20240630 PL - Poland TA - Postepy Dermatol Alergol JT - Postepy dermatologii i alergologii JID - 101168357 PMC - PMC11253315 OTO - NOTNLM OT - capillaroscopy OT - dermatomyositis OT - polymyositis OT - scleroderma OT - scleroderma-spectrum disorders COIS- The authors declare no conflict of interest. EDAT- 2024/07/19 06:42 MHDA- 2024/07/19 06:43 PMCR- 2024/06/01 CRDT- 2024/07/19 04:50 PHST- 2023/03/02 00:00 [received] PHST- 2024/03/11 00:00 [accepted] PHST- 2024/07/19 06:43 [medline] PHST- 2024/07/19 06:42 [pubmed] PHST- 2024/07/19 04:50 [entrez] PHST- 2024/06/01 00:00 [pmc-release] AID - 54420 [pii] AID - 10.5114/ada.2024.141158 [doi] PST - ppublish SO - Postepy Dermatol Alergol. 2024 Jun;41(3):296-300. doi: 10.5114/ada.2024.141158. Epub 2024 Jun 30. PMID- 42131627 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260514 LR - 20260514 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 4 DP - 2026 Apr TI - Severe Peripheral Arterial Ischemia Leading to Digital Gangrene in Mixed Connective Tissue Disease: A Case Report. PG - e106861 LID - 10.7759/cureus.106861 [doi] LID - e106861 AB - Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder characterized by overlapping clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis, in association with anti-U1 ribonucleoprotein (anti-U1-RNP) antibodies. Although vascular manifestations such as Raynaud's phenomenon are common, severe peripheral ischemic complications are rare. We report the case of a 40-year-old woman with known MCTD who presented with a three-month history of progressive necrotic lesions affecting the lower extremities, associated with severe pain, impaired ambulation, and ulcerative lesions of the hands. Her disease course was complicated by interstitial lung disease and pulmonary arterial hypertension. On admission, physical examination revealed extensive gangrenous lesions affecting multiple toes, along with cutaneous and musculoskeletal features consistent with advanced disease. Despite multidisciplinary management, the ischemic lesions progressed, and surgical intervention was planned. The patient subsequently developed sudden cardiorespiratory arrest and died before surgery. This report highlights a rare and severe vascular manifestation of MCTD characterized by peripheral arterial ischemia leading to digital gangrene. Clinicians should maintain a high index of suspicion for systemic autoimmune diseases in patients presenting with unexplained ischemic or necrotic lesions. Early recognition and multidisciplinary management are essential to improve clinical outcomes. CI - Copyright © 2026, Tamayo-Gómez et al. FAU - Tamayo-Gómez, Manuel Esaú AU - Tamayo-Gómez ME AD - General Surgery, "Dr. Javier Buenfil Osorio" General Hospital of Specialties, Campeche, MEX. FAU - Sandoval, Alfonso AU - Sandoval A AD - Medicine, Tecnológico de Monterrey Campus Ciudad de Mexico, Mexico City, MEX. FAU - Arjona-Bojorquez, Lilian Priscilla AU - Arjona-Bojorquez LP AD - General Surgery, "Dr. Javier Buenfil Osorio" General Hospital of Specialties, Campeche, MEX. FAU - Pablos-López, Luis Manuel AU - Pablos-López LM AD - General Surgery, "Dr. Javier Buenfil Osorio" General Hospital of Specialties, Campeche, MEX. FAU - Mangas-Sosa, María Abril AU - Mangas-Sosa MA AD - Surgery, Autonomous University of Campeche, Campeche, MEX. FAU - González Flores, José Emiliano AU - González Flores JE AD - Medicine, Tecnológico de Monterrey Campus Ciudad de Mexico, Mexico City, MEX. LA - eng PT - Case Reports PT - Journal Article DEP - 20260411 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13161721 OTO - NOTNLM OT - autoimmune vascular injury OT - digital gangrene OT - mixed connective tissue disease OT - peripheral ischemia OT - raynaud phenomenon. COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/05/14 14:21 MHDA- 2026/05/14 14:22 PMCR- 2026/04/11 CRDT- 2026/05/14 04:55 PHST- 2026/04/11 00:00 [accepted] PHST- 2026/05/14 14:22 [medline] PHST- 2026/05/14 14:21 [pubmed] PHST- 2026/05/14 04:55 [entrez] PHST- 2026/04/11 00:00 [pmc-release] AID - 10.7759/cureus.106861 [doi] PST - epublish SO - Cureus. 2026 Apr 11;18(4):e106861. doi: 10.7759/cureus.106861. eCollection 2026 Apr. PMID- 27012074 OWN - NLM STAT- MEDLINE DCOM- 20160408 LR - 20160325 IS - 0017-7768 (Print) IS - 0017-7768 (Linking) VI - 155 IP - 1 DP - 2016 Jan TI - [THROMBOANGIITIS OBLITERANS OVERLAPPING WITH ATHEROSCLEROTIC OCCLUSIVE ARTERIAL DISEASE: SMALL MESENTERIC ARTERY INVOLVEMENT BY THROMBOANGIITIS OBLITERANS]. PG - 41-4, 67, 66 AB - INTRODUCTION: Thromboangiitis obliterans is an inflammatory occlusive vascular disease of young smokers that commonly involves the small and medium sized arteries and veins of the extremities. An important differential diagnosis of thromboangiitis obliterans is atherosclerotic arterial disease. An atypical presentation of thromboangiitis obliterans by involvement of mesenteric arteries has been described sporadically. CASE PRESENTATION: We report the case of a patient presenting with Raynaud's phenomenon, ischemia of the upper and lower extremities, as well as mesenteric ischemia. The dramatic course of the disease advanced to gangrene of the calves and intestinal infarction. In this patient, angiographic and histologic features were consistent with thromboangiitis obliterans associated with atherosclerotic arteriopathy. DISCUSSION: A review of the literature revealed 31 reported cases of mesenteric artery involvement by thromboangiitis obliterans. The overlap between thromboangiitis obliterans and atherosclerotic arteriopathy is rare but has recently focused attention in the literature. CONCLUSION: In the differential diagnosis of mesenteric ischemia, thromboangiitis obliterans is a rare but important diagnosis that should be considered. In view of shared features of thromboangiitis obliterans and peripheral artery disease, awareness of their possible coexistence is needed in order to make the right diagnosis and offer proper treatment. FAU - Rimar, Doron AU - Rimar D FAU - Rozenbaum, Michael AU - Rozenbaum M FAU - Slobodin, Gleb AU - Slobodin G FAU - Boulman, Nina AU - Boulman N FAU - KaLy, Lisa AU - KaLy L FAU - Rosner, Itzhak AU - Rosner I LA - heb PT - Case Reports PT - English Abstract PT - Journal Article PT - Review PL - Israel TA - Harefuah JT - Harefuah JID - 0034351 SB - IM MH - Arteriosclerosis/*diagnosis/pathology MH - Diagnosis, Differential MH - Gangrene/pathology MH - Humans MH - Ischemia/etiology/pathology MH - Male MH - Mesenteric Arteries/pathology MH - Mesenteric Vascular Occlusion/*diagnosis/etiology/pathology MH - Middle Aged MH - Peripheral Arterial Disease/diagnosis/pathology MH - Thromboangiitis Obliterans/complications/*diagnosis/pathology EDAT- 2016/03/26 06:00 MHDA- 2016/04/09 06:00 CRDT- 2016/03/26 06:00 PHST- 2016/03/26 06:00 [entrez] PHST- 2016/03/26 06:00 [pubmed] PHST- 2016/04/09 06:00 [medline] PST - ppublish SO - Harefuah. 2016 Jan;155(1):41-4, 67, 66. PMID- 19542803 OWN - NLM STAT- MEDLINE DCOM- 20090831 LR - 20090622 IS - 1536-5409 (Electronic) IS - 0749-8047 (Linking) VI - 25 IP - 6 DP - 2009 Jul-Aug TI - Effectiveness of interventions of specific complaints of the arm, neck, or shoulder (CANS): musculoskeletal disorders of the hand. PG - 537-52 LID - 10.1097/AJP.0b013e31819ff52c [doi] AB - OBJECTIVES: The aim of this study was to provide an evidence-based overview of the effectiveness of (conservative and surgical) interventions for the 4 specific pain disorders of the hand: trigger finger, primary Raynaud's phenomenon, Dupuytren disease, and De Quervain's disease. This information can help clinicians in the selection of interventions in daily practice, and may give direction to future research. METHODS: Relevant review publications and randomized clinical trials (RCTs) in PubMed were searched. Data extraction and quality assessment were performed. To summarize the results of the included reviews and RCTs, a best-evidence synthesis was used. RESULTS: For primary Raynaud's phenomenon (1 review, 20 RCTs), we found strong evidence for calcium channel blockers and moderate evidence for laser therapy. Limited evidence was found for Ketanserin, Prozasin, Buflomedil, transdermal glyceryl trinitrate patches, Ginkgo biloba, and behavioral treatment with temperature feedback. Other interventions did not show clear favorable treatment effects. For Trigger finger one very small RCT was found that showed limited evidence for steroid injection. For Dupuytren disease (4 RCTs) limited evidence was found in favor of use of staples versus sutures in the Dupuytren's surgery, and for intermittent compression on the postoperative hand after surgery. For other interventions no clear positive effects could be demonstrated. For De Quervain's disease (2 RCTs), we found no efficacy of Nimesulide as addition to a Triamcinolone injection, and no clear differences between a corticosteroid injection and a splint in pregnant patients or patients breast-feeding. DISCUSSION: Well-designed and well-conducted RCTs are clearly needed in this field. FAU - van Middelkoop, Marienke AU - van Middelkoop M AD - Department of General Practice, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Huisstede, Bionka M A AU - Huisstede BM FAU - Glerum, Suzanne AU - Glerum S FAU - Koes, Bart W AU - Koes BW LA - eng PT - Journal Article PT - Review PL - United States TA - Clin J Pain JT - The Clinical journal of pain JID - 8507389 SB - IM MH - Arm/*physiopathology/surgery MH - Female MH - Hand/*physiopathology/surgery MH - Humans MH - Musculoskeletal Diseases/*pathology/surgery/*therapy MH - Neck/*physiopathology/surgery MH - Pregnancy MH - Randomized Controlled Trials as Topic MH - Shoulder/*physiopathology/surgery RF - 41 EDAT- 2009/06/23 09:00 MHDA- 2009/09/01 06:00 CRDT- 2009/06/23 09:00 PHST- 2009/06/23 09:00 [entrez] PHST- 2009/06/23 09:00 [pubmed] PHST- 2009/09/01 06:00 [medline] AID - 00002508-200907000-00013 [pii] AID - 10.1097/AJP.0b013e31819ff52c [doi] PST - ppublish SO - Clin J Pain. 2009 Jul-Aug;25(6):537-52. doi: 10.1097/AJP.0b013e31819ff52c. PMID- 24608395 OWN - NLM STAT- MEDLINE DCOM- 20150908 LR - 20181202 IS - 1672-7347 (Print) IS - 1672-7347 (Linking) VI - 39 IP - 2 DP - 2014 Feb TI - ANCA associated glomerulonephritis in a patient with mixed connective tissue disease. PG - 209-14 LID - 10.11817/j.issn.1672-7347.2014.02.018 [doi] AB - OBJECTIVE: To investigate the diagnosis and treatment of mixed connective tissue disease (MCTD) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis, which is a rare clinical entity in medical practice. METHODS: A 35-year-old female of Asian origin was admitted to our hospital due to complaints of Raynaud's phenomenon, myalgia, arthralgia and fatigue. The patient was diagnosed as MCTD in the out-patient department 8 months prior to admission based on Alarcon-Segovia classification criteria of Raynaud's phenomenon, myalgia, arthralgia and a high anti-U1 ribonucleoprotein antibody level. Interstitial lung disease was determined by chest computed tomography. Renal biopsy was performed because of marked proteinuria on 24 h urine collection. Histopathological examination revealed glomerulonephritis with fibrocellular/cellular crescents, in which moderate staining of IgM was shown by direct immunofluorescence. She was tested positive for myeloperoxidase antineutrophil cytoplasmic antibody. RESULTS: High dose of methylprednisolone (500 mg/d for 3 days) was started intravenously when the results of renal biopsy were obtained. Oral prednisone and intravenous cyclophosphamide therapy (0.8 g/month) were continued for 12 months. Daily urinary protein loss decreased dramatically and serum creatinine was maintained at a normal level. CONCLUSION: Corticosteroids and cyclophosphamide are effective in the treatment of MPO-ANCA associated glomerulonephritis in MCTD. FAU - Sun, Yining AU - Sun Y AD - Department of Rheumatology, First Affiliated Hospital of the Medical School of Xi'an Jiaotong Universtiy, Xi'an 710061, China. FAU - He, Lan AU - He L FAU - Lü, Xiaohong AU - Lü X FAU - Mo, Lingfei AU - Mo L FAU - Zhang, Jing AU - Zhang J LA - eng PT - Case Reports PT - Journal Article PL - China TA - Zhong Nan Da Xue Xue Bao Yi Xue Ban JT - Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences JID - 101230586 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 8N3DW7272P (Cyclophosphamide) RN - EC 1.11.1.7 (Peroxidase) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - *Antibodies, Antineutrophil Cytoplasmic MH - Cyclophosphamide MH - Female MH - *Glomerulonephritis MH - Humans MH - Methylprednisolone MH - *Mixed Connective Tissue Disease MH - Peroxidase MH - Proteinuria EDAT- 2014/03/13 06:00 MHDA- 2015/09/09 06:00 CRDT- 2014/03/11 06:00 PHST- 2014/03/11 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2015/09/09 06:00 [medline] AID - 10.11817/j.issn.1672-7347.2014.02.018 [doi] PST - ppublish SO - Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2014 Feb;39(2):209-14. doi: 10.11817/j.issn.1672-7347.2014.02.018. PMID- 37461764 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230719 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 6 DP - 2023 Jun TI - Undifferentiated Connective Tissue Disease With Isolated Diaphragmatic Dysfunction. PG - e40515 LID - 10.7759/cureus.40515 [doi] LID - e40515 AB - Undifferentiated connective tissue disease (UCTD) is a rare autoimmune disorder with a prevalence of about two people per 100,000 people per year. Patients present with the features of different connective tissue diseases, including systemic lupus erythematosus, systemic sclerosis, polymyositis, and rheumatoid arthritis, with some positivity in serological markers that is insufficient to fulfill the criteria of any recognized connective tissue disorder. Pulmonary involvement is usually subacute and pleomorphic, which can cause a delay in the diagnosis. A few cases of UCTD involving an isolated diaphragm in the pulmonary system have been reported. We report a case of a 48-year-old female who initially presented with various nonspecific symptoms, including fatigue, polyarthralgia, dry mouth, and Raynaud's phenomenon. Subsequently, she developed significant dyspnea and orthopnea. Laboratory, immunology, and imaging workups were negative for any specific diagnosis. Pulmonary function tests showed severely low maximum inspiratory pressure (MEP) and maximum expiratory pressure, suggesting diaphragmatic dysfunction. A diagnosis of UCTD was considered, and she was treated with hydroxychloroquine and intravenous immunoglobulin (IVIG), which improved her respiratory symptoms and pulmonary function tests. CI - Copyright © 2023, Purohit et al. FAU - Purohit, Richa AU - Purohit R AD - Department of Medicine, Concentra Urgent Care, Orlando, USA. FAU - Shahu Khal, Ravi AU - Shahu Khal R AD - Department of Rheumatology, University of Central Florida Hospital Corporation of America (HCA) Healthcare Graduate Medical Education (GME), Orlando, USA. FAU - Gokalp, Gizem AU - Gokalp G AD - Department of Medicine, University of Central Florida Hospital Corporation of America (HCA) Healthcare Graduate Medical Education (GME), Orlando, USA. AD - Department of Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Sambandan, Rajan AU - Sambandan R AD - Department of Medicine, University of Central Florida College of Medicine, Orlando, USA. FAU - Bhanusali, Neha AU - Bhanusali N AD - Department of Rheumatology, University of Central Florida College of Medicine, Orlando, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230616 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10350301 OTO - NOTNLM OT - diaphragmatic dysfunction OT - dyspnea OT - ivig OT - pulmonary function test OT - undifferentiated connective tissue disease COIS- The authors have declared that no competing interests exist. EDAT- 2023/07/18 06:42 MHDA- 2023/07/18 06:43 PMCR- 2023/06/16 CRDT- 2023/07/18 03:40 PHST- 2023/05/24 00:00 [received] PHST- 2023/06/16 00:00 [accepted] PHST- 2023/07/18 06:43 [medline] PHST- 2023/07/18 06:42 [pubmed] PHST- 2023/07/18 03:40 [entrez] PHST- 2023/06/16 00:00 [pmc-release] AID - 10.7759/cureus.40515 [doi] PST - epublish SO - Cureus. 2023 Jun 16;15(6):e40515. doi: 10.7759/cureus.40515. eCollection 2023 Jun. PMID- 22116525 OWN - NLM STAT- MEDLINE DCOM- 20131030 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 4 DP - 2013 Apr TI - Undifferentiated connective tissue disease in a rheumatology center in Cali, Colombia: clinical features of 94 patients followed for a year. PG - 1085-8 LID - 10.1007/s00296-011-2234-y [doi] AB - The aim of the study is to evaluate the clinical and serological features of patients with undifferentiated connective tissue disease (UCTD) of a rheumatology referral center in Cali, Colombia, who were followed for a year. A retrospective analysis of a cohort of patients with an initial diagnosis of UCTD and monitoring for at least 12 months was carried out. A total of 94 patients with UCTD (97.9% women) were evaluated, with an average follow-up of 51 ± 35.7 months. Only 13 patients (13.8%) evolved into a defined connective tissue disease (CTD), of which 8.5% (n:8) developed systemic lupus erythematosus (SLE), 4.2% (n:4) Sjögren syndrome (SS) and 1.1% (n:1) rheumatoid arthritis (RA). A mean period of 35.8 ± 29.2 months between UCTD diagnosis and definite develop of a CTD was found. Arthritis, Raynaud's phenomenon and photosensitivity were statistically significant (<0.001) for development of CTD. After a mean follow-up of 4.25 years, most of the patients with UCTD showed a favorable evolution. Arthritis, Raynaud's phenomenon and the presence of photosensitivity were predictors for the development of CTD. It requires a consensus to establish criteria for the classification of UCTD. FAU - Guerrero, Luis F AU - Guerrero LF AD - Department of Internal Medicine, Universidad Libre, Cali, Colombia. FAU - Rueda, Juan C AU - Rueda JC FAU - Arciniegas, Raquel AU - Arciniegas R FAU - Rueda, Jorge M AU - Rueda JM LA - eng PT - Journal Article DEP - 20111125 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Arthritis, Rheumatoid/*diagnosis/immunology MH - Colombia MH - Connective Tissue Diseases/*diagnosis/immunology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Lupus Erythematosus, Systemic/*diagnosis/immunology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Sjogren's Syndrome/*diagnosis/immunology EDAT- 2011/11/26 06:00 MHDA- 2013/10/31 06:00 CRDT- 2011/11/26 06:00 PHST- 2011/07/17 00:00 [received] PHST- 2011/10/22 00:00 [accepted] PHST- 2011/11/26 06:00 [entrez] PHST- 2011/11/26 06:00 [pubmed] PHST- 2013/10/31 06:00 [medline] AID - 10.1007/s00296-011-2234-y [doi] PST - ppublish SO - Rheumatol Int. 2013 Apr;33(4):1085-8. doi: 10.1007/s00296-011-2234-y. Epub 2011 Nov 25. PMID- 27340761 OWN - NLM STAT- MEDLINE DCOM- 20170426 LR - 20181202 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 65 IP - 2 DP - 2017 TI - Successful long-term (22 year) treatment of limited scleroderma using therapeutic plasma exchange: Is blood rheology the key? PG - 131-136 LID - 10.3233/CH-16140 [doi] AB - While a number of studies have shown short-term beneficial effects of therapeutic plasma exchange (TPE) for treating systemic scleroderma (SSc), there have been no reports on the very long-term usage of TPE as the sole systemic treatment intervention. We report the case of a male patient, originally diagnosed with limited systemic scleroderma (lcSSc) in early 1990, who has been undergoing regular plasmapheresis treatments for more than 22 years, beginning in late 1993. Prior to commencing treatment, the patient exhibited symptoms including severe gastro esophageal reflux disease (GERD) with esophagitis, frequent Raynaud's attacks, reduced lung function, and chronic chilling. With the exception of mild residual Raynaud's, all of the patient's symptoms reversed after three years of regular TPE treatments and he remains in complete remission. While the typical explanation for the therapeutic benefits seen with TPE focuses on temporary reduction of circulating antibodies or other pathogenic factors, we propose instead an explanation based on abnormal blood rheology as a novel disease pathogenesis model for SSc. FAU - Harris, Edward S AU - Harris ES AD - Scleroderma Education Project Ltd, Madison, WI, USA. FAU - Meiselman, Herbert J AU - Meiselman HJ AD - Department of Physiology & Biophysics, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA. FAU - Moriarty, Patrick M AU - Moriarty PM AD - Department of Clinical Pharmacology, University of Kansas Medical Center, Kansas City, KS, USA. FAU - Weiss, John AU - Weiss J AD - Department of Pathology and Laboratory Medicine, University of Wisconsin School of Medicine, Madison, WI, USA. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 SB - IM MH - Aged MH - Blood Viscosity MH - Humans MH - Male MH - Plasma Exchange/*methods MH - Plasmapheresis/*methods MH - *Rheology MH - Scleroderma, Limited/*therapy OTO - NOTNLM OT - CREST syndrome OT - Limited scleroderma OT - blood viscosity OT - plasma exchange OT - plasmapheresis EDAT- 2016/06/25 06:00 MHDA- 2017/04/27 06:00 CRDT- 2016/06/25 06:00 PHST- 2016/06/25 06:00 [pubmed] PHST- 2017/04/27 06:00 [medline] PHST- 2016/06/25 06:00 [entrez] AID - CH16140 [pii] AID - 10.3233/CH-16140 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2017;65(2):131-136. doi: 10.3233/CH-16140. PMID- 33547561 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231223 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 40 IP - 4 DP - 2021 Apr TI - Correction to: How do patients define Raynaud's phenomenon? Differences between primary and secondary disease. PG - 1617-1620 LID - 10.1007/s10067-021-05620-y [doi] AB - INTRODUCTION/ OBJECTIVES: To examine how people define Raynaud’s Phenomenon (RP), based on their lived experiences and explore if differences exist depending on primary or secondary RP diagnosis. METHOD: An international survey was sent to people with RP through health systems, foundations, and social media. Qualitative coding of responses to an open text question regarding one’s own definition of RP was performed and themes were identified. Prevalence of themes among the sample and then comparisons between themes among people who self-reported primary versus secondary diagnosis of RP were performed. RESULTS: There were 1345 respondents from 45 countries (mean age 51.5 years, 93% female) who defined RP in their own words; 17% reported primary RP and 83% reported secondary RP (69% of secondary RP was scleroderma-related, n = 927). Over half defined their RP by describing the body parts affected, color changes, pain, and triggers or situations in which an episode occurs. Patients with primary RP more frequently defined RP in terms of its impact on function/quality of life and pain compared to those with secondary RP (34.5% versus 25.3% respectively, p=0.004; 54.0% versus 46.8%, p=0.05). Patients with secondary RP more frequently included specific body parts, color change, the management of attacks and other digital vascular complications in their definition of RP. CONCLUSIONS: We have identified differences in how people with primary and secondary RP define RP, in terms of how they feel and function. Our findings have implications for the domains of outcome measures for assessing RP within different patient populations. FAU - Murphy, Susan L AU - Murphy SL AUID- ORCID: 0000-0001-7924-0012 AD - Department of Physical Medicine and Rehabilitation, University of Michigan, 24 Frank Lloyd Wright Drive, Lobby M Suite 3100, Ann Arbor, MI, 48105, USA. sumurphy@umich.edu. AD - VA Ann Arbor Health Care System, Geriatric Research, Education, and Clinical Center (GRECC), 2215 Fuller Rd, Ann Arbor, MI, 48105, USA. sumurphy@umich.edu. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France. AD - Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France. AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Alore, Mary AU - Alore M AD - Peer Mentors Program, University of Michigan Scleroderma Program, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Bath, UK. FAU - Sabbagh, Maya AU - Sabbagh M AD - Peer Mentors Program, University of Michigan Scleroderma Program, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, 7C27 NIB 300 North Ingalls Street, Ann Arbor, MI, 48109, USA. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - R01 AR070470/AR/NIAMS NIH HHS/United States GR - R43 AR063129/AR/NIAMS NIH HHS/United States PT - Published Erratum PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM EFR - Clin Rheumatol. 2021 Apr;40(4):1611-1616. doi: 10.1007/s10067-021-05598-7. PMID: 33486597 PMC - PMC10734887 MID - NIHMS1949008 COIS- Conflicts of interest/Competing Interests: Dr. Pauling reports personal fees from Boehringer Ingelheim; Grants, personal fees and non-financial support from Actelion Pharmaceuticals; Personal fees from Sojournix Pharma, all outside the submitted work. Dr. Hughes reports speakers fees from Actelion Pharmaceuticals. Dr. Khanna is a consultant to Acceleron, Abbvie, Actelion, Amgen, Bayer, BMS, Boehringer Ingelheim, CSL Behring, Corbus, Galapagos, Genentech/ Roche, GSK, Horizon, MitsubishiTanabe Pharma, Sanofi-Aventis, and United Therapeutics. He has stock options in Eicos Sciences, Inc. No other authors report conflicts of interest or competing interests. EDAT- 2021/02/07 06:00 MHDA- 2021/02/07 06:01 PMCR- 2023/12/21 CRDT- 2021/02/06 05:45 PHST- 2021/02/07 06:00 [pubmed] PHST- 2021/02/07 06:01 [medline] PHST- 2021/02/06 05:45 [entrez] PHST- 2023/12/21 00:00 [pmc-release] AID - 10.1007/s10067-021-05620-y [pii] AID - 10.1007/s10067-021-05620-y [doi] PST - ppublish SO - Clin Rheumatol. 2021 Apr;40(4):1617-1620. doi: 10.1007/s10067-021-05620-y. PMID- 26123342 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20181113 IS - 1432-2218 (Electronic) IS - 0930-2794 (Linking) VI - 30 IP - 4 DP - 2016 Apr TI - Thoracic sympathectomy: a review of current indications. PG - 1255-69 LID - 10.1007/s00464-015-4353-0 [doi] AB - BACKGROUND: Thoracic sympathetic ablation was introduced over a century ago. While some of the early indications have become obsolete, new ones have emerged. Sympathetic ablation is being still performed for some odd indications thus prompting the present study, which reviews the evidence base for current practice. METHODS: The literature was reviewed using the PubMed/Medline Database, and pertinent articles regarding the indications for thoracic sympathectomy were retrieved and evaluated. Old, historical articles were also reviewed as required. RESULTS AND CONCLUSIONS: Currently, thoracic sympathetic ablation is indicated mainly for primary hyperhidrosis, especially affecting the palm, and to a lesser degree, axilla and face, and for facial blushing. Despite modern pharmaceutical, endovascular and surgical treatments, sympathetic ablation has still a place in the treatment of very selected cases of angina, arrhythmias and cardiomyopathy. Thoracic sympathetic ablation is indicated in several painful conditions: the early stages of complex regional pain syndrome, erythromelalgia, and some pancreatic and other painful abdominal pathologies. Although ischaemia was historically the major indication for sympathetic ablation, its use has declined to a few selected cases of thromboangiitis obliterans (Buerger's disease), microemboli, primary Raynaud's phenomenon and Raynaud's phenomenon secondary to collagen diseases, paraneoplastic syndrome, frostbite and vibration syndrome. Thoracic sympathetic ablation for hypertension is obsolete, and direct endovascular renal sympathectomy still requires adequate clinical trials. There are rare publications of sympathetic ablation for primary phobias, but there is no scientific basis to support sympathetic surgery for any psychiatric indication. FAU - Hashmonai, Moshe AU - Hashmonai M AD - Faculty of Medicine, Technion-Israel Institute of Technology, PO Box 359, 30952119, Zikhron Ya'akov, Haifa, Israel. hasmonai@inter.net.il. FAU - Cameron, Alan E P AU - Cameron AE AD - Nuffield Hospital, Ipswich, UK. FAU - Licht, Peter B AU - Licht PB AD - Department of Cardiothoracic Surgery, Odense University Hospital, Odense, Denmark. FAU - Hensman, Chris AU - Hensman C AD - Specialist Surgeon, Melbourne, Australia. FAU - Schick, Christoph H AU - Schick CH AD - German Hyperhidrosiscenter, Munich, Germany. LA - eng PT - Journal Article PT - Review DEP - 20150627 PL - Germany TA - Surg Endosc JT - Surgical endoscopy JID - 8806653 SB - IM MH - Heart Diseases/surgery MH - Humans MH - Hyperhidrosis/surgery MH - *Sympathectomy MH - *Thoracoscopy OTO - NOTNLM OT - Hyperhidrosis OT - Sympathectomy OT - Thoracoscopy EDAT- 2015/07/01 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/07/01 06:00 PHST- 2015/03/05 00:00 [received] PHST- 2015/06/16 00:00 [accepted] PHST- 2015/07/01 06:00 [entrez] PHST- 2015/07/01 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1007/s00464-015-4353-0 [pii] AID - 10.1007/s00464-015-4353-0 [doi] PST - ppublish SO - Surg Endosc. 2016 Apr;30(4):1255-69. doi: 10.1007/s00464-015-4353-0. Epub 2015 Jun 27. PMID- 28292033 OWN - NLM STAT- MEDLINE DCOM- 20170404 LR - 20260127 IS - 1937-8688 (Electronic) VI - 25 DP - 2016 TI - [Silica-associated systemic sclerosis occurring after an occupational exposure to arc welding]. PG - 70 LID - 10.11604/pamj.2016.25.70.10390 [doi] LID - 70 AB - Crystalline silica-associated systemic sclerosis can occur in people operating arc welding. Diffuse scleroderma was diagnosed in a 57-year old plumber-welder suffering from inflammatory polyarthralgias, Raynaud's phenomenon, sclerodactyly, diffuse cutaneous scleroderma, telangiectasias, esophageal damage, pulmonary arterial hypertension and pulmonary fibrosis associated with the presence of anti-nucleosome antibodies. During his professional activity the patient was frequently exposed to high atmospheric concentrations of crystalline silica generated by arc-welding. The diagnosis of Erasmus syndrome associated with systemic sclerosis and pulmonary silicosis was retained. A report of work-related illness (table 17 in Tunisia) was made. FAU - Alaya, Zeineb AU - Alaya Z AD - Service de Rhumatologie, CHU Farhat-Hached, Sousse, Tunisie. FAU - Kalboussi, Houda AU - Kalboussi H AD - Service de Médecine du Travail, CHU Farhat-Hached, Sousse, Tunisie. FAU - Osman, Walid AU - Osman W AD - Service d'Orthopédie, CHU Sahloul, Sousse, Tunisie. FAU - Naouar, Nader AU - Naouar N AD - Service d'Orthopédie, CHU Sahloul, Sousse, Tunisie. FAU - Zeglaoui, Héla AU - Zeglaoui H AD - Service de Rhumatologie, CHU Farhat-Hached, Sousse, Tunisie. FAU - Bouajina, Elyès AU - Bouajina E AD - Service de Rhumatologie, CHU Farhat-Hached, Sousse, Tunisie. LA - fre PT - Case Reports PT - Journal Article TT - Sclérodermie systémique associée à l’exposition à la silice survenant après une exposition professionnelle à la soudure à l’arc. DEP - 20161004 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - 7631-86-9 (Silicon Dioxide) SB - IM MH - Humans MH - Male MH - Occupational Diseases/diagnosis/*physiopathology MH - Occupational Exposure/*adverse effects MH - Scleroderma, Systemic/diagnosis/*etiology/physiopathology MH - Silicon Dioxide/toxicity MH - Silicosis/*diagnosis/physiopathology MH - Tunisia MH - Welding PMC - PMC5324153 OTO - NOTNLM OT - Systemic sclerosis OT - occupational disease OT - silica crystalline COIS- Les auteurs ne déclarent aucun conflit d’intérêt en relation avec cet article. EDAT- 2017/03/16 06:00 MHDA- 2017/04/05 06:00 PMCR- 2016/10/04 CRDT- 2017/03/16 06:00 PHST- 2016/07/24 00:00 [received] PHST- 2016/08/03 00:00 [accepted] PHST- 2017/03/16 06:00 [entrez] PHST- 2017/03/16 06:00 [pubmed] PHST- 2017/04/05 06:00 [medline] PHST- 2016/10/04 00:00 [pmc-release] AID - PAMJ-25-70 [pii] AID - 10.11604/pamj.2016.25.70.10390 [doi] PST - epublish SO - Pan Afr Med J. 2016 Oct 4;25:70. doi: 10.11604/pamj.2016.25.70.10390. eCollection 2016. PMID- 41716450 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260220 LR - 20260220 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 14 IP - 2 DP - 2026 Feb TI - A Rare Case Report of Antisynthetase Syndrome With Progressive Myopathy and Interstitial Lung Disease in a 38-Year-Old Male. PG - e72059 LID - 10.1002/ccr3.72059 [doi] LID - e72059 AB - Antisynthetase syndrome is a rare autoimmune disorder characterized by a range of clinical features, most commonly associated with autoantibodies targeting aminoacyl-tRNA synthetases. The condition typically presents with a combination of symptoms, including myositis, arthritis, Raynaud's phenomenon, "mechanic's hands," fever, and interstitial lung involvement. Pulmonary manifestations, particularly interstitial lung disease (ILD), are significant prognostic determinants. Management often involves a combination of immunosuppressive agents to address both muscular and respiratory complications. We report the case of a 38-year-old man who developed progressive proximal muscle weakness, arthralgia, Raynaud's phenomenon, and ILD. Laboratory tests indicated elevated muscle enzymes, and anti-Jo-1 antibodies were detected on repeat testings. Electromyography supported a diagnosis of myopathy, while high-resolution computed tomography confirmed ILD. He was treated with corticosteroids, additional immunosuppressants, and plasmapheresis, which resulted in partial symptomatic improvement. Rituximab was considered a potential future treatment option. This case emphasizes the importance of early recognition and prompt, intensive immunosuppressive therapy, along with careful clinical monitoring, to improve outcomes for patients with severe antisynthetase syndrome. CI - © 2026 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Ali, Masab AU - Ali M AUID- ORCID: 0009-0007-8599-6861 AD - Department of Internal Medicine Punjab Medical College Faisalabad Punjab Pakistan. FAU - Tariq, Muhammad Umair AU - Tariq MU AD - Department of Neurology Allied Hospital Faisalabad Pakistan. FAU - Hassan, Muhammad AU - Hassan M AUID- ORCID: 0009-0006-6747-7980 AD - Department of Internal Medicine Punjab Medical College Faisalabad Punjab Pakistan. FAU - Ahmad, Muhammad Husnain AU - Ahmad MH AUID- ORCID: 0009-0006-5248-0749 AD - Department of Internal Medicine S Tentishev Asian Medical Institute Kant Kyrgyzstan. FAU - Jamil, Amina AU - Jamil A AD - Aziz Fatimah Medical & Dental College Faisalabad Punjab Pakistan. LA - eng PT - Journal Article DEP - 20260217 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC12913691 OTO - NOTNLM OT - antisynthetase syndrome OT - anti‐Jo‐1 OT - idiopathic inflammatory myopathy OT - interstitial lung disease OT - mechanic's hands COIS- The authors declare no conflicts of interest. EDAT- 2026/02/20 07:05 MHDA- 2026/02/20 07:06 PMCR- 2026/02/17 CRDT- 2026/02/20 04:46 PHST- 2025/12/19 00:00 [received] PHST- 2026/01/29 00:00 [revised] PHST- 2026/02/03 00:00 [accepted] PHST- 2026/02/20 07:06 [medline] PHST- 2026/02/20 07:05 [pubmed] PHST- 2026/02/20 04:46 [entrez] PHST- 2026/02/17 00:00 [pmc-release] AID - CCR372059 [pii] AID - 10.1002/ccr3.72059 [doi] PST - epublish SO - Clin Case Rep. 2026 Feb 17;14(2):e72059. doi: 10.1002/ccr3.72059. eCollection 2026 Feb. PMID- 30703839 OWN - NLM STAT- MEDLINE DCOM- 20190502 LR - 20190502 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 144 IP - 3 DP - 2019 Feb TI - [Progress in Systemic Sclerosis - Early, Targeted and Intensive Therapy is the Key to Success]. PG - 189-193 LID - 10.1055/a-0652-2488 [doi] AB - This literature review summarizes the main findings in systemic sclerosis (SSc) made in the last few years. Accordingly, the disease pathogenesis is mainly driven by the adaptive immune system, which is proven by the effects of autologous stem cell transplantation. Particularly, autoantibodies can activate both adaptive as well as innate immune cells as identified for the anti-angiotensin receptor antibodies. In addition, major achievements come from the early recognition of organ complications, which mainly appear in the first years upon Raynaud`s phenomenon. This implicates screening for organ complications such as for pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD) even without any clinical symptoms at the beginning. On the other hand, the presence of anti-polymerase III antibodies indicates a risk or the presence of malignant diseases, which should be identified. Several studies in the last years showed the high burden of the disease, which is often underscored by physicians. Pain, depressions, fatigue, and incontinence often determine quality of life and should be recognized and treated, if possible. Systemic sclerosis is a disease with the highest disease-related mortality among the rheumatic diseases. More than half of the SSc patients die from SSc manifestations particularly from cardiac and lung involvement such as PAH and ILD. Ventricular tachycardias should be recognized by Holter-ECG. Finally, intensive therapies such as autologous stem cell transplantation or combination therapies seem to be most successful in SSc as well as in SSc-related PAH. Currently, several studies are ongoing, which will hopefully change the outcome and quality of life. CI - © Georg Thieme Verlag KG Stuttgart · New York. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck. LA - ger PT - Journal Article PT - Review TT - Fortschritte bei der Systemischen Sklerose. DEP - 20190131 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/mortality/therapy COIS- Die Autorin erklärt, Honorare für Vorträge, Beratung und teilweise für Forschung von den Firmen Actelion, CellTrend, BMS, AbbVie, Celgene, Roche und UCB erhalten zu haben. EDAT- 2019/02/01 06:00 MHDA- 2019/05/03 06:00 CRDT- 2019/02/01 06:00 PHST- 2019/02/01 06:00 [entrez] PHST- 2019/02/01 06:00 [pubmed] PHST- 2019/05/03 06:00 [medline] AID - 10.1055/a-0652-2488 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2019 Feb;144(3):189-193. doi: 10.1055/a-0652-2488. Epub 2019 Jan 31. PMID- 28796005 OWN - NLM STAT- MEDLINE DCOM- 20180510 LR - 20181202 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 29 IP - 6 DP - 2017 Nov TI - Clinical spectrum of anti-Jo-1-associated disease. PG - 612-617 LID - 10.1097/BOR.0000000000000434 [doi] AB - PURPOSE OF REVIEW: To provide the most recent evidence on anti-Jo-1 syndrome. RECENT FINDINGS: Several new evidences on anti-Jo-1 syndrome have recently emerged. It has been clearly established that, at disease onset, the classic clinical triad (arthritis, myositis and interstitial lung disease - ILD) is only rarely observed. Indeed, disease onset with an isolated arthritis is common. Patients presenting with an isolated manifestation are at high risk for the subsequent occurrence of initially lacking triad findings. Moreover, the ex-novo occurrence of accompanying features such as Raynaud's phenomenon, mechanic's hands and fever during follow-up is a strong risk factor for the occurrence of overt antisynthetase syndrome (ASSD) with further triad manifestations. Several contributions on ILD involvement and prognosis have been published, as well as the distinctive muscle MRI characteristics compared with healthy controls, and a novel definition of a rare skin manifestation (hiker's feet). SUMMARY: Recent evidence has shed a light on the need for a better understanding of the clinical course, imaging modalities and prognosis of anti-Jo-1 syndrome, providing some relevant elements to allow early diagnosis of this often unrecognized disease. FAU - Monti, Sara AU - Monti S AD - Division of Rheumatology, University of Pavia, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Montecucco, Carlomaurizio AU - Montecucco C FAU - Cavagna, Lorenzo AU - Cavagna L LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) SB - IM MH - *Antibodies, Antinuclear MH - Arthritis/*diagnosis/immunology MH - Autoimmune Diseases/*diagnosis/immunology MH - Histidine-tRNA Ligase/*immunology MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/immunology MH - Myositis/*diagnosis/immunology MH - Prognosis MH - Risk Factors MH - Symptom Assessment MH - Syndrome EDAT- 2017/08/11 06:00 MHDA- 2018/05/11 06:00 CRDT- 2017/08/11 06:00 PHST- 2017/08/11 06:00 [pubmed] PHST- 2018/05/11 06:00 [medline] PHST- 2017/08/11 06:00 [entrez] AID - 10.1097/BOR.0000000000000434 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2017 Nov;29(6):612-617. doi: 10.1097/BOR.0000000000000434. PMID- 42039714 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260427 LR - 20260427 IS - 0971-6203 (Print) IS - 1998-3913 (Electronic) IS - 0971-6203 (Linking) VI - 51 IP - 1 DP - 2026 Jan-Mar TI - Heat for Healing: A Review of Infrared Thermography in Medical Diagnostics and Therapy. PG - 1-11 LID - 10.4103/jmp.jmp_175_25 [doi] AB - Infrared thermography (IRT) is an emerging noninvasive imaging modality that provides real-time, contactless assessment of skin surface temperature, reflecting underlying vascular perfusion. This narrative review explores the principles, clinical utility, advantages, limitations, and future potential of IRT in vascular diagnostics and monitoring. IRT has demonstrated diagnostic relevance across a spectrum of vascular conditions, including peripheral arterial disease, diabetic foot complications, venous insufficiency, Raynaud's phenomenon, and postoperative vascular monitoring. Its key benefits - such as radiation-free imaging, portability, and dynamic functional assessment - make it especially valuable for use in vulnerable populations and resource-limited settings. However, challenges such as environmental sensitivity, lack of standardized imaging protocols, and limited specificity necessitate further validation. With the integration of artificial intelligence and wearable technology, IRT holds significant promise as a complementary tool in modern vascular medicine. CI - Copyright: © 2026 Journal of Medical Physics. FAU - Kumar, Bitesh AU - Kumar B AD - Department of Pediatric Surgery, All India Institute of Medical Science, New Delhi, India. FAU - Jain, Vishesh AU - Jain V AD - Department of Pediatric Surgery, All India Institute of Medical Science, New Delhi, India. FAU - Yadav, Devendra Kumar AU - Yadav DK AD - Department of Pediatric Surgery, All India Institute of Medical Science, New Delhi, India. FAU - Dhua, Anjan Kumar AU - Dhua AK AD - Department of Pediatric Surgery, All India Institute of Medical Science, New Delhi, India. FAU - Goel, Prabudh AU - Goel P AD - Department of Pediatric Surgery, All India Institute of Medical Science, New Delhi, India. FAU - Jain, Divya AU - Jain D AD - Department of Ophthalmology, Post Graduate Institute of Child Health, Noida, Uttar Pradesh, India. FAU - Singh, Shubhendu AU - Singh S AD - Department of Microbiology, Santosh Deemed to be University, Ghaziabad, Uttar Pradesh, India. LA - eng PT - Journal Article PT - Review DEP - 20260331 PL - India TA - J Med Phys JT - Journal of medical physics JID - 9441104 PMC - PMC13105372 OTO - NOTNLM OT - Diagnostic OT - infrared thermography OT - monitoring OT - noninvasive imaging OT - thermal imaging COIS- There are no conflicts of interest. EDAT- 2026/04/27 12:34 MHDA- 2026/04/27 12:35 PMCR- 2026/01/01 CRDT- 2026/04/27 07:02 PHST- 2025/07/07 00:00 [received] PHST- 2025/11/19 00:00 [revised] PHST- 2025/12/13 00:00 [accepted] PHST- 2026/04/27 12:35 [medline] PHST- 2026/04/27 12:34 [pubmed] PHST- 2026/04/27 07:02 [entrez] PHST- 2026/01/01 00:00 [pmc-release] AID - JMP-51-1 [pii] AID - 10.4103/jmp.jmp_175_25 [doi] PST - ppublish SO - J Med Phys. 2026 Jan-Mar;51(1):1-11. doi: 10.4103/jmp.jmp_175_25. Epub 2026 Mar 31. PMID- 22453528 OWN - NLM STAT- MEDLINE DCOM- 20140613 LR - 20220318 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 9 DP - 2013 Sep TI - Camptocormia as a clinical manifestation of polymyositis/systemic sclerosis overlap myositis associated with anti-Ku. PG - 2411-5 LID - 10.1007/s00296-012-2412-6 [doi] AB - Camptocormia, an abnormal truncal flexion posture that occurs while walking or standing, is usually caused by various hypokinetic movement disorders, mainly Parkinson disease. We describe the case of a man with subacute onset of camptocormia. Quadriceps muscle biopsy showed significant rhabdomyolysis, few isolated inflammatory cells and mild expression of type I MHC in few fibers, a pattern usually found in immune-mediated necrotizing myopathies. Myositis was associated with Raynaud's phenomenon, mild sclerodactyly, and anti-Ku antibodies leading to the diagnosis of polymyositis/systemic sclerosis overlap myositis. The posture showed modest improvement in response to treatment. FAU - Zenone, Thierry AU - Zenone T AD - Department of Internal Medicine, Centre Hospitalier, 179 boulevard Marechal Juin, 26953, Valence Cedex 9, France. tzenone@ch-valence.fr FAU - Streichenberger, Nathalie AU - Streichenberger N FAU - Puget, Marie AU - Puget M LA - eng PT - Case Reports PT - Journal Article DEP - 20120328 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) RN - 0 (DNA-Binding Proteins) RN - EC 3.6.4.12 (Xrcc6 protein, human) RN - EC 4.2.99.- (Ku Autoantigen) RN - Camptocormia SB - IM MH - Aged MH - Antigens, Nuclear/*immunology MH - Autoantibodies/*blood MH - DNA-Binding Proteins/*immunology MH - Humans MH - Ku Autoantigen MH - Male MH - Muscular Atrophy, Spinal/*etiology MH - Polymyositis/*complications/pathology MH - Scleroderma, Systemic/*complications/pathology MH - Spinal Curvatures/*etiology EDAT- 2012/03/29 06:00 MHDA- 2014/06/15 06:00 CRDT- 2012/03/29 06:00 PHST- 2011/11/28 00:00 [received] PHST- 2012/03/11 00:00 [accepted] PHST- 2012/03/29 06:00 [entrez] PHST- 2012/03/29 06:00 [pubmed] PHST- 2014/06/15 06:00 [medline] AID - 10.1007/s00296-012-2412-6 [doi] PST - ppublish SO - Rheumatol Int. 2013 Sep;33(9):2411-5. doi: 10.1007/s00296-012-2412-6. Epub 2012 Mar 28. PMID- 21689379 OWN - NLM STAT- MEDLINE DCOM- 20111207 LR - 20110816 IS - 1748-1716 (Electronic) IS - 1748-1708 (Linking) VI - 203 IP - 1 DP - 2011 Sep TI - Paracrine control of vascular innervation in health and disease. PG - 61-86 LID - 10.1111/j.1748-1716.2011.02333.x [doi] AB - Proper vascular regulation is of paramount importance for the control of blood flow to tissues. In particular, the regulation of peripheral resistance arteries is essential for several physiological processes, including control of blood pressure, thermoregulation and increase of blood flow to central nervous system and heart under stress conditions such as hypoxia. Arterial tone is regulated by the periarterial autonomic nervous plexus, as well as by endothelium-dependent, myogenic and humoral mechanisms. Underscoring the importance of proper vascular regulation, defects in these processes can lead to diseases such as hypertension, orthostatic hypotension, Raynaud's phenomenon, defective thermoregulation, hand-foot syndrome, migraine and congestive heart failure. Here, we review the molecular mechanisms controlling the development of the periarterial nerve plexus, retrograde and localized signalling at neuro-effector junctions, the molecular and cellular mechanisms of vascular regulation and adult plasticity and maintenance of periarterial innervation. We particularly highlight a newly discovered role for vascular endothelial growth factor in the structural and functional maintenance of arterial neuro-effector junctions. Finally, we discuss how defects in neuronal vascular regulation can lead to disease. CI - © 2011 The Authors. Acta Physiologica © 2011 Scandinavian Physiological Society. FAU - Storkebaum, E AU - Storkebaum E AD - Molecular Neurogenetics Laboratory, Max Planck Institute for Molecular Biomedicine, Muenster, Germany. erik.storkebaum@mpi-muenster.mpg.de FAU - Carmeliet, P AU - Carmeliet P LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20110730 PL - England TA - Acta Physiol (Oxf) JT - Acta physiologica (Oxford, England) JID - 101262545 SB - IM MH - Animals MH - Arteries/*innervation MH - Hemodynamics/*physiology MH - Humans MH - Paracrine Communication/*physiology MH - Vascular Resistance/*physiology EDAT- 2011/06/22 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/06/22 06:00 PHST- 2011/06/22 06:00 [entrez] PHST- 2011/06/22 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 10.1111/j.1748-1716.2011.02333.x [doi] PST - ppublish SO - Acta Physiol (Oxf). 2011 Sep;203(1):61-86. doi: 10.1111/j.1748-1716.2011.02333.x. Epub 2011 Jul 30. PMID- 32755965 OWN - NLM STAT- Publisher LR - 20240227 IS - 2052-0573 (Print) IS - 2052-0573 (Electronic) IS - 2052-0573 (Linking) VI - 2020 DP - 2020 Aug 4 TI - Type B insulin resistance syndrome associated with connective tissue disease and psoriasis. LID - EDM200027 [pii] LID - 10.1530/EDM-20-0027 [doi] LID - 20-0027 AB - SUMMARY: Type B insulin resistance syndrome (TBIR) is characterised by the rapid onset of severe insulin resistance due to circulating anti-insulin receptor antibodies (AIRAs). Widespread acanthosis nigricans is normally seen, and co-occurrence with other autoimmune diseases is common. We report a 27-year-old Caucasian man with psoriasis and connective tissue disease who presented with unexplained rapid weight loss, severe acanthosis nigricans, and hyperglycaemia punctuated by fasting hypoglycaemia. Severe insulin resistance was confirmed by hyperinsulinaemic euglycaemic clamping, and immunoprecipitation assay demonstrated AIRAs, confirming TBIR. Treatment with corticosteroids, metformin and hydroxychloroquine allowed withdrawal of insulin therapy, with stabilisation of glycaemia and diminished signs of insulin resistance; however, morning fasting hypoglycaemic episodes persisted. Over three years of follow-up, metabolic control remained satisfactory on a regimen of metformin, hydroxychloroquine and methotrexate; however, psoriatic arthritis developed. This case illustrates TBIR as a rare but severe form of acquired insulin resistance and describes an effective multidisciplinary approach to treatment. LEARNING POINTS: We describe an unusual case of type B insulin resistance syndrome (TBIR) in association with mixed connective tissue disease and psoriasis. Clinical evidence of severe insulin resistance was corroborated by euglycaemic hyperinsulinaemic clamp, and anti-insulin receptor autoantibodies were confirmed by immunoprecipitation assay. Treatment with metformin, hydroxychloroquine and methotrexate ameliorated extreme insulin resistance. FAU - Łebkowska, Agnieszka AU - Łebkowska A AD - Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine. FAU - Krentowska, Anna AU - Krentowska A AD - Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine. FAU - Adamska, Agnieszka AU - Adamska A AD - Department of Endocrinology, Diabetology and Internal Medicine. FAU - Lipińska, Danuta AU - Lipińska D AD - Department of Endocrinology, Diabetology and Internal Medicine. FAU - Piasecka, Beata AU - Piasecka B AD - Department of Endocrinology, Diabetology and Internal Medicine. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland. FAU - Górska, Maria AU - Górska M AD - Department of Endocrinology, Diabetology and Internal Medicine. FAU - Semple, Robert K AU - Semple RK AD - Centre for Cardiovascular Science, Queen's Medical Research Institute, University of Edinburgh, Edinburgh, UK. FAU - Kowalska, Irina AU - Kowalska I AD - Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine. LA - eng PT - Journal Article DEP - 20200804 PL - England TA - Endocrinol Diabetes Metab Case Rep JT - Endocrinology, diabetes & metabolism case reports JID - 101618943 PMC - PMC7424346 OTO - NOTNLM OT - 2020 OT - Acanthosis nigricans OT - Adiponectin OT - Adult OT - Alanine aminotransferase* OT - Anaemia OT - Anti-insulin antibodies OT - Anti-insulin receptor antibodies* OT - Antinuclear antibody OT - Antiribonucleoprotein antibodies* OT - August OT - Autoimmune disorders OT - BMI OT - Complement* OT - Connective tissue disorders* OT - Coombs test* OT - Corticosteroids OT - DEXA scan OT - Dermatology OT - Diabetes OT - Enlarged parotid glands* OT - Fatigue OT - Glucose (urine) OT - Glucosuria OT - Haemoglobin A1c OT - Hydroxychloroquine* OT - Hyperinsulinaemic euglycaemic clamp* OT - Hypoglycaemia OT - Immunoprecipitation* OT - Insulin OT - Insulin resistance OT - Leukopenia* OT - Lymphopenia* OT - Male OT - Metformin OT - Methotrexate* OT - Methylprednisolone OT - New disease or syndrome: presentations/diagnosis/management OT - Palpable lymph nodes* OT - Pancreas OT - Platelet count OT - Poland OT - Prednisone OT - Psoriasis* OT - Psoriatic arthritis OT - Raynaud's syndrome* OT - Red blood cell count OT - Sclerodactyly* OT - Skin OT - Sodium OT - Tachycardia OT - Thrombocytopenia OT - Triglycerides OT - Urinalysis OT - Weight loss OT - White OT - White blood cell count EDAT- 2020/08/07 06:00 MHDA- 2020/08/07 06:00 PMCR- 2020/08/04 CRDT- 2020/08/07 06:00 PHST- 2020/06/15 00:00 [received] PHST- 2020/07/13 00:00 [accepted] PHST- 2020/08/07 06:00 [entrez] PHST- 2020/08/07 06:00 [pubmed] PHST- 2020/08/07 06:00 [medline] PHST- 2020/08/04 00:00 [pmc-release] AID - EDM200027 [pii] AID - 10.1530/EDM-20-0027 [doi] PST - aheadofprint SO - Endocrinol Diabetes Metab Case Rep. 2020 Aug 4;2020:20-0027. doi: 10.1530/EDM-20-0027. PMID- 29453582 OWN - NLM STAT- MEDLINE DCOM- 20180918 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 5 DP - 2018 May TI - The clinical phenotype of patients positive for antibodies to myositis and myositis-related disorders. PG - 1257-1263 LID - 10.1007/s10067-018-4032-3 [doi] AB - Inflammatory myopathies are a clinically diverse group of diseases, in which the detection of particular autoantibodies may facilitate diagnosis, treatment, and prognosis. The aim of this report is to summarize our experience with specific autoantibody testing in patients with inflammatory myopathies. Data were collected over the last decade in the Autoimmune Center of the Sheba Medical Center, a tertiary referral hospital. Data regarding patients' positive for autoantibodies against Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, and PM-Scl antigens were retrospectively collected. Patient demographics, clinical characteristics, and mortality were recorded. Descriptive statistics (mean, standard deviation, frequency, and percentage) were calculated. A total of 507 patients were surveyed for sclero-poly-synthetase antibodies, as part of the diagnostic workup of myositis/myalgia or interstitial lung disease. Forty-three patients were found positive for one or more of the abovementioned antibodies, and 23 of them (53.49%) had interstitial lung disease (ILD). Four patients were positive for anti-PL-7, three of them had ILD and Raynaud's phenomenon. Five patients were positive for anti-Ku, and four of them had both arthritis and Raynaud's phenomenon. Nine patients were positive for anti-Mi-2, and six of them were given diagnosed with dermatomyositis. Ten patients were positive for anti-SRP, and six of them had cancers of various types. Our results reiterate the previously recognized associations between anti-Mi-2 and dermatomyositis, anti-Ku and Raynaud's phenomenon, and between anti-PL-7 and ILD. In addition, our data support an association between anti-SRP autoantibody positivity and malignancy, which calls for further investigation. FAU - Gofrit, S G AU - Gofrit SG AD - Department of Medicine A, Sheba Medical Center, 5262000, Tel Hashomer, Israel. FAU - Yonath, H AU - Yonath H AD - Department of Medicine A, Sheba Medical Center, 5262000, Tel Hashomer, Israel. AD - Danek Gertner Institute of Human Genetics, Tel Hashomer, Israel. AD - Sackler School of Medicine, Ramat Aviv, Israel. FAU - Lidar, M AU - Lidar M AD - Sackler School of Medicine, Ramat Aviv, Israel. AD - The Zabludowicz Center for Autoimmune Diseases, Tel-Hashomer, Israel. FAU - Shoenfeld, Y AU - Shoenfeld Y AD - Sackler School of Medicine, Ramat Aviv, Israel. AD - The Zabludowicz Center for Autoimmune Diseases, Tel-Hashomer, Israel. AD - Incumbent of the Laura Schwarz-Kip Chair for Research of Autoimmune Diseases, Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv-Yafo, Israel. FAU - Kivity, S AU - Kivity S AD - Department of Medicine A, Sheba Medical Center, 5262000, Tel Hashomer, Israel. kivitys@gmail.com. AD - Sackler School of Medicine, Ramat Aviv, Israel. kivitys@gmail.com. AD - The Zabludowicz Center for Autoimmune Diseases, Tel-Hashomer, Israel. kivitys@gmail.com. AD - The Dr. Pinchas Borenstein Talpiot Medical Leadership Program 2013 and Sheba Medical Center, Tel-Hashomer, Israel. kivitys@gmail.com. LA - eng PT - Journal Article DEP - 20180216 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Mi-2 antibodies) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoantibodies/*analysis MH - Autoantigens/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Myositis/*diagnosis/immunology MH - Phenotype MH - Registries OTO - NOTNLM OT - Anti-synthetase OT - Dermatomyositis OT - Interstitial lung disease OT - Myositis OT - Polymyositis EDAT- 2018/02/18 06:00 MHDA- 2018/09/19 06:00 CRDT- 2018/02/18 06:00 PHST- 2017/12/14 00:00 [received] PHST- 2018/02/07 00:00 [accepted] PHST- 2018/02/02 00:00 [revised] PHST- 2018/02/18 06:00 [pubmed] PHST- 2018/09/19 06:00 [medline] PHST- 2018/02/18 06:00 [entrez] AID - 10.1007/s10067-018-4032-3 [pii] AID - 10.1007/s10067-018-4032-3 [doi] PST - ppublish SO - Clin Rheumatol. 2018 May;37(5):1257-1263. doi: 10.1007/s10067-018-4032-3. Epub 2018 Feb 16. PMID- 40144419 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250328 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 2 DP - 2025 Feb TI - Converging Inflammations: Simultaneous Myositis and Polyneuropathy as a Diagnostic Challenge. PG - e79536 LID - 10.7759/cureus.79536 [doi] LID - e79536 AB - Idiopathic inflammatory myopathies consist of a variety of autoimmune diseases with variable clinical manifestations, treatment response, and prognosis. Symmetrical proximal predominant muscle weakness is the usual presenting clinical manifestation except in the case of clinically amyopathic dermatomyositis and inclusion body myositis, which represent without muscle weakness and asymmetric muscle weakness, respectively. Other extramuscular manifestations include skin rash and necrosis, arthritis, interstitial lung disease, myocarditis, dysphagia, and Raynaud's phenomenon. However, there are only a few case reports of neuromyositis in the literature. Here, we describe a case of a 30-year-old Indian male patient with polymyositis associated with inflammatory polyneuropathy. CI - Copyright © 2025, Kumari et al. FAU - Kumari, Shikha AU - Kumari S AD - General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND. FAU - Priyanka, Gudimetla AU - Priyanka G AD - General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND. FAU - N C, Kiran AU - N C K AD - General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND. FAU - Bansal, Rachit AU - Bansal R AD - General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND. FAU - Sharma, Siddharth AU - Sharma S AD - General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20250223 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11942522 OTO - NOTNLM OT - antisynthetase syndrome OT - inflammatory myositis OT - neuromyositis OT - polymyositis OT - polyneuropathy COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/03/27 06:29 MHDA- 2025/03/27 06:30 PMCR- 2025/02/23 CRDT- 2025/03/27 05:11 PHST- 2025/02/23 00:00 [accepted] PHST- 2025/03/27 06:30 [medline] PHST- 2025/03/27 06:29 [pubmed] PHST- 2025/03/27 05:11 [entrez] PHST- 2025/02/23 00:00 [pmc-release] AID - 10.7759/cureus.79536 [doi] PST - epublish SO - Cureus. 2025 Feb 23;17(2):e79536. doi: 10.7759/cureus.79536. eCollection 2025 Feb. PMID- 31813070 OWN - NLM STAT- MEDLINE DCOM- 20201026 LR - 20201026 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 21 IP - 12 DP - 2019 Dec 7 TI - Arthritis in Idiopathic Inflammatory Myopathies. PG - 70 LID - 10.1007/s11926-019-0878-x [doi] AB - PURPOSE OF REVIEW: Arthritis is a well-recognized symptom of idiopathic inflammatory myopathies (IIM). We provide a summary of available data regarding the epidemiology, clinical characteristics, and autoantibody associations of joint involvement in various forms of IIM. RECENT FINDINGS: Arthritis is reported in 18-55% of patients with IIM. It is particularly frequent (20-70%) in those with antisynthetase syndrome (ASS); highest prevalence is associated with anti-Jo-1 positivity. Most common manifestation is non-erosive polyarthritis. X-ray erosions may be found occasionally in ASS, particularly in patients with overlap with rheumatoid arthritis (RA). Arthritis is often present at the time of IIM diagnosis and it may even precede the onset of muscle weakness. Arthritis may in some cases be the main disease manifestation responsible for the disease burden in patients with IIM. Arthritis is a frequent symptom of IIM. Polyarthritis of small joints of the hands is the most frequent clinical manifestation. Arthritis may be the first or dominant symptom in IIM and therefore patients may be initially misdiagnosed as having RA. Particularly in seronegative RA patients with interstitial lung disease or Raynaud's phenomenon, the possibility of IIM should be considered. FAU - Klein, Martin AU - Klein M AD - Institute of Rheumatology, Na Slupi 4, 128 50, Prague, Czech Republic. AD - Department of Rheumatology, 1st Medical Faculty, Charles University, Prague, Czech Republic. FAU - Mann, Heřman AU - Mann H AD - Institute of Rheumatology, Na Slupi 4, 128 50, Prague, Czech Republic. AD - Department of Rheumatology, 1st Medical Faculty, Charles University, Prague, Czech Republic. FAU - Vencovský, Jiří AU - Vencovský J AD - Institute of Rheumatology, Na Slupi 4, 128 50, Prague, Czech Republic. vencovsky@revma.cz. AD - Department of Rheumatology, 1st Medical Faculty, Charles University, Prague, Czech Republic. vencovsky@revma.cz. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20191207 PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 SB - IM MH - Arthritis/*diagnosis/*epidemiology MH - Humans MH - Myositis/*complications/*diagnosis OTO - NOTNLM OT - Anti-Jo-1 OT - Antisynthetase syndrome OT - Arthritis OT - Floppy-thumb OT - Idiopathic inflammatory myopathies EDAT- 2019/12/10 06:00 MHDA- 2020/10/27 06:00 CRDT- 2019/12/09 06:00 PHST- 2019/12/09 06:00 [entrez] PHST- 2019/12/10 06:00 [pubmed] PHST- 2020/10/27 06:00 [medline] AID - 10.1007/s11926-019-0878-x [pii] AID - 10.1007/s11926-019-0878-x [doi] PST - epublish SO - Curr Rheumatol Rep. 2019 Dec 7;21(12):70. doi: 10.1007/s11926-019-0878-x. PMID- 27115104 OWN - NLM STAT- MEDLINE DCOM- 20171019 LR - 20171019 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 41 IP - 1 DP - 2016 Jan-Mar TI - Systemic sclerosis: markers and targeted treatments. PG - 18-25 AB -

Systemic sclerosis (SSc) is characterized by autoantibody production, progressive microvasculopathy, and aberrant extracellular matrix protein (ECM) synthesis in tissues. The disease presents two major clinical hallmarks: Raynaud's phenomenon (RP) and skin involvement, followed by varying prevalences of internal organ involvement. Despite significant advances in the management of certain organ-specific involvements and symptoms, the research for efficient markers and targets, to be used for an optimized treatment, is still ongoing. Therapies targeting the vasculature (i.e. ET-1 receptor antagonists, phosphodiesterase-5 (PDE-5) inhi bitor, agiotensin-converting enzyme inhibition, prostacyclins), the immune system and/or the fibrotic process (i.e. traditional disease modifying anti-rheu - matic drugs DMARDs such as methotrexate, cyclospo - rine or mycophenolate mofetil, biologicals like rituxi - mab, tocilizumab or abatacept) have been or are being eva luated in SSc. Advanced approaches, reserved to unres ponsive SSc patients, include autologous haema - topoietic stem cell transplantation (HSTC) and intravenous immunoglobulins (IVIG). Interestingly, it is expected that new and future possible diagnostic and therapeutical approaches in SSc will come from epigenetic studies (MicroRNAs). Ideally, combination therapy in SSc seems the best approach, together with the early intervention on the major hallmarks of the disease in "at risk" patients, that consists of the microvascular damage/altered function and the autoimmune reaction, followed by the progressive and systemic fibrotic process.

. FAU - Cutolo, M AU - Cutolo M FAU - Sulli, A AU - Sulli A FAU - Pizzorni, C AU - Pizzorni C FAU - Paolino, S AU - Paolino S FAU - Smith, V AU - Smith V LA - eng PT - Journal Article PT - Review TT - Systemic sclerosis: markers and targeted treatments. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 RN - 0 (Biomarkers) SB - IM MH - Biomarkers/analysis MH - Forecasting MH - Humans MH - *Molecular Targeted Therapy MH - Scleroderma, Systemic/diagnosis/*drug therapy EDAT- 2016/04/27 06:00 MHDA- 2017/10/20 06:00 CRDT- 2016/04/27 06:00 PHST- 2016/04/27 06:00 [entrez] PHST- 2016/04/27 06:00 [pubmed] PHST- 2017/10/20 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2016 Jan-Mar;41(1):18-25. PMID- 23423441 OWN - NLM STAT- MEDLINE DCOM- 20140117 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 32 IP - 5 DP - 2013 May TI - Antisynthetase antibody syndrome: case report and review of the literature. PG - 715-9 LID - 10.1007/s10067-013-2207-5 [doi] AB - Antisynthetase antibody syndrome is a rare autoimmune disease that may present with variable systemic manifestations, mainly polymyositis, interstitial lung disease, skin lesions, and Raynaud's phenomenon. This diagnosis should always come to mind in patients that present with signs of myositis, dermatomyositis, or polymyositis associated with interstitial lung disease. On the following paper, we report the case of a 52-year-old man who presented with a 2-month history of asymmetric polyarthralgia, myalgia, weight loss of 8 kg, and progressive muscle weakness associated with dyspnea, orthopnea, and dysphonia. Further tests revealed myositis, interstitial pneumonia, and elevation of anti-Jo-1 antibodies. A diagnosis of antisynthetase antibody syndrome was made and the patient showed good response to treatment with corticoids and methotrexate. Finally, we present a short review of the literature. FAU - Uribe, Laura AU - Uribe L AD - Department of Internal Medicine, Faculty of Medicine, Fundación Universitaria de Ciencias de Salud, Bogotá, Colombia. FAU - Ronderos, Diana Maria AU - Ronderos DM FAU - Díaz, Maria Claudia AU - Díaz MC FAU - Gutierrez, Juan Martín AU - Gutierrez JM FAU - Mallarino, Christina AU - Mallarino C FAU - Fernandez-Avila, Daniel Gerardo AU - Fernandez-Avila DG LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20130220 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Antibodies, Antinuclear/immunology MH - Arthralgia/diagnosis MH - Biopsy MH - Humans MH - Lung Diseases, Interstitial/complications/*diagnosis MH - Male MH - Middle Aged MH - Muscle Weakness/diagnosis MH - Muscles/pathology MH - Myalgia/diagnosis MH - Myositis/complications/*diagnosis/therapy MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Weight Loss EDAT- 2013/02/21 06:00 MHDA- 2014/01/18 06:00 CRDT- 2013/02/21 06:00 PHST- 2013/01/14 00:00 [received] PHST- 2013/01/30 00:00 [accepted] PHST- 2013/02/21 06:00 [entrez] PHST- 2013/02/21 06:00 [pubmed] PHST- 2014/01/18 06:00 [medline] AID - 10.1007/s10067-013-2207-5 [doi] PST - ppublish SO - Clin Rheumatol. 2013 May;32(5):715-9. doi: 10.1007/s10067-013-2207-5. Epub 2013 Feb 20. PMID- 28042297 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1687-9627 (Print) IS - 1687-9635 (Electronic) VI - 2016 DP - 2016 TI - Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders. PG - 7649510 LID - 10.1155/2016/7649510 [doi] LID - 7649510 AB - Secondary amyloidosis can complicate chronic inflammatory autoimmune diseases. However, the clinical findings of primary amyloidosis may mimic those of primary rheumatologic disorders. We present the case of a 53-year-old woman who presented with dystrophic nail changes, dry eyes, bilateral carpal tunnel syndrome, Raynaud's phenomenon, and high titer positive nucleolar pattern antinuclear antibody. She was initially misdiagnosed as having Undifferentiated Connective Tissue Disease (UCTD). On further workup, she was eventually diagnosed with lambda light chain systemic amyloidosis by abdominal fat pad biopsy. Her symptoms completely resolved after autologous stem cell transplantation. With this case, we would like to highlight the similarities in the clinical features between light chain amyloidosis and rheumatological disorders. We would also like to emphasize the importance of the prompt recognition of the clinical features of amyloidosis which are crucial to triggering appropriate diagnostic procedures, since early diagnosis is a key to improving outcomes in this disease with an otherwise poor prognosis. FAU - Rao, Rohit R AU - Rao RR AUID- ORCID: 0000-0002-5118-0473 AD - Department of Hematology/Oncology, Allegheny Health Network, 320 East North Avenue, Pittsburgh, PA 15212, USA. FAU - Yong, Wai Chung AU - Yong WC AUID- ORCID: 0000-0002-8395-6897 AD - Department of Internal Medicine, The Mary Imogene Bassett Hospital, One Atwell Road, Cooperstown, NY 13326, USA. FAU - Wasko, Mary Chester AU - Wasko MC AUID- ORCID: 0000-0002-7536-9246 AD - Lupus Center of Excellence, Allegheny Health Network, 4800 Friendship Avenue, Suite 2600, North Tower, Pittsburgh, PA 15224, USA. LA - eng PT - Journal Article DEP - 20161129 PL - United States TA - Case Rep Med JT - Case reports in medicine JID - 101512910 PMC - PMC5153512 COIS- All authors declare that there are no competing interests. EDAT- 2017/01/04 06:00 MHDA- 2017/01/04 06:01 PMCR- 2016/11/29 CRDT- 2017/01/03 06:00 PHST- 2016/07/06 00:00 [received] PHST- 2016/09/26 00:00 [revised] PHST- 2016/09/28 00:00 [accepted] PHST- 2017/01/03 06:00 [entrez] PHST- 2017/01/04 06:00 [pubmed] PHST- 2017/01/04 06:01 [medline] PHST- 2016/11/29 00:00 [pmc-release] AID - 10.1155/2016/7649510 [doi] PST - ppublish SO - Case Rep Med. 2016;2016:7649510. doi: 10.1155/2016/7649510. Epub 2016 Nov 29. PMID- 34432146 OWN - NLM STAT- MEDLINE DCOM- 20220928 LR - 20221003 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 42 IP - 11 DP - 2022 Nov TI - Mixed connective tissue disease after thymectomy in refractory myasthenia gravis. PG - 2077-2084 LID - 10.1007/s00296-021-04976-3 [doi] AB - Connective tissue diseases, including systemic lupus erythematosus (SLE) and systemic sclerosis are classic models of autoimmunity; diseases with large-scale loss of tolerance and subsequent development of pathogenic autoreactive lymphocytes and tissue targeting autoantibodies. Here we report a case of mixed connective tissue disease, with features of systemic lupus erythematosus and systemic sclerosis developing in a patient 10 years post thymectomy for myasthenia gravis. The patient developed acute cutaneous lupus, Raynaud's with digital ulcers, arthritis and lymphopenia. Her myasthenia continued to be resistant to treatment and her rheumatic disease progressed despite aggressive therapy. We performed a database search of MEDLINE, EMBASE, Scopus, and Web of Science for articles of similar cases post thymectomy from inception to August 2021, using the terms "systemic lupus erythematosus" (or systemic sclerosis, or connective tissue disease) and "myasthenia gravis" and "thymectomy". We identified 41 cases, 28 of SLE post thymectomy, 8 related to systemic sclerosis, 5 with mixed connective tissue disease and highlighted their different presentation and serology. We explore the role of the thymus, tolerance and myasthenia gravis in the development of connective tissue disease. This highlights the complexity of concurrent autoimmune diseases and their autoantibodies. CI - © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Kobza, Alexandra AU - Kobza A AUID- ORCID: 0000-0001-7503-123X AD - Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada. FAU - Keenan, Marissa AU - Keenan M AD - Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada. AD - Division of Rheumatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada. FAU - Ivory, Catherine AU - Ivory C AUID- ORCID: 0000-0002-9042-7854 AD - Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada. civory@toh.ca. AD - Division of Rheumatology, Department of Medicine, The Ottawa Hospital, Ottawa, ON, Canada. civory@toh.ca. AD - Clinical Epidemiology Program, The Ottawa Hospital Research Institute, Ottawa, ON, Canada. civory@toh.ca. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210825 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - *Connective Tissue Diseases MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/complications MH - *Mixed Connective Tissue Disease/complications MH - *Myasthenia Gravis/surgery MH - *Scleroderma, Systemic/complications/surgery OTO - NOTNLM OT - Anti-RNP antibodies OT - Autoimmunity OT - Mixed connective tissue disease OT - Myasthenia gravis OT - Thymectomy EDAT- 2021/08/26 06:00 MHDA- 2022/09/28 06:00 CRDT- 2021/08/25 12:26 PHST- 2021/06/30 00:00 [received] PHST- 2021/08/16 00:00 [accepted] PHST- 2021/08/26 06:00 [pubmed] PHST- 2022/09/28 06:00 [medline] PHST- 2021/08/25 12:26 [entrez] AID - 10.1007/s00296-021-04976-3 [pii] AID - 10.1007/s00296-021-04976-3 [doi] PST - ppublish SO - Rheumatol Int. 2022 Nov;42(11):2077-2084. doi: 10.1007/s00296-021-04976-3. Epub 2021 Aug 25. PMID- 36569738 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221227 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 11 DP - 2022 Nov TI - ANCA-Negative EGPA With Pulmonary, Cutaneous, and Neurological Manifestations in a 25-Year-Old Male: A Case Report. PG - e31753 LID - 10.7759/cureus.31753 [doi] LID - e31753 AB - Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by the presence of asthma, hyper-eosinophilia, and necrotizing vasculitis with extravascular eosinophilic granulomas. We report the case of a 25-year-old male who presented to the outpatient department complaining of joint aches and numbness in the hands and legs. Physical examination revealed erythematous blanchable macular rashes on palms and soles. Raynaud's phenomenon was also observed. Lab workup revealed elevated WBC count and peripheral blood eosinophilia. Antibody tests were positive only for anti-nuclear antibodies. A diagnosis of eosinophilic granulomatosis with polyangiitis including peripheral neuropathy, arthralgia, rash, and pulmonary manifestations was established. The patient was started on a therapeutic regimen of corticosteroids and immunosuppressants, which halted the progression of the disease. Peripheral neuropathy and arthralgia also improved. CI - Copyright © 2022, Shehryar et al. FAU - Shehryar, Abdullah AU - Shehryar A AD - Internal Medicine, Allama Iqbal Medical College, Lahore, PAK. FAU - Rehman, Abdur AU - Rehman A AD - Internal Medicine, Mayo Hospital, Lahore, PAK. FAU - Sajid, Samar AU - Sajid S AD - Medicine, Dow University of Health Sciences, Karachi, PAK. FAU - Haseeb, Muhammad AU - Haseeb M AD - Internal Medicine, Jinnah Hospital Lahore, Lahore, PAK. AD - Internal Medicine, Bahria International Hospital, Lahore, PAK. FAU - Owais, Mohammad AU - Owais M AD - Medicine, Mayo Hospital, Lahore, PAK. LA - eng PT - Case Reports PT - Journal Article DEP - 20221121 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9771091 OTO - NOTNLM OT - asthma OT - churg strauss syndrome OT - egpa OT - eosinophilia OT - eosinophilic granulomatosis with polyangiitis (egpa) OT - granuloma OT - vasculitis COIS- The authors have declared that no competing interests exist. EDAT- 2022/12/27 06:00 MHDA- 2022/12/27 06:01 PMCR- 2022/11/21 CRDT- 2022/12/26 04:20 PHST- 2022/11/21 00:00 [accepted] PHST- 2022/12/26 04:20 [entrez] PHST- 2022/12/27 06:00 [pubmed] PHST- 2022/12/27 06:01 [medline] PHST- 2022/11/21 00:00 [pmc-release] AID - 10.7759/cureus.31753 [doi] PST - epublish SO - Cureus. 2022 Nov 21;14(11):e31753. doi: 10.7759/cureus.31753. eCollection 2022 Nov. PMID- 29333029 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 0971-5851 (Print) IS - 0975-2129 (Electronic) IS - 0971-5851 (Linking) VI - 38 IP - 4 DP - 2017 Oct-Dec TI - Flagellate Rash: An Unusual Complication of Bleomycin Therapy - A Case Report with Brief Review of Literature. PG - 548-551 LID - 10.4103/ijmpo.ijmpo_147_16 [doi] AB - Chemotherapy-induced skin rashes are common toxicities encountered which require careful assessment and evaluation as rashes could be a manifestation of primary malignancy itself and a variety of drugs used in combination further complicate the clinical scenario. Bleomycin is an anticancer antibiotic derived from Streptomyces verticillus and has been commonly used in the treatment of Hodgkin's disease, germ cell tumors and for pleurodesis. There are various dermatological adverse effects of bleomycin which have been previously reported in literature including skin peeling, hyperkeratosis, nail bed changes, Raynaud's phenomenon, and palmoplantar desquamation. Bleomycin-induced skin rashes are seen infrequently now a day due to its declining use in clinical practice. We report here a 29-year-old male with Stage III germ cell tumor who developed widespread flagellate rash after receiving 3 cycles of bleomycin-based chemotherapy which responded to treatment with local steroids and omission of bleomycin from further chemotherapy cycles. FAU - Agrawal, Chaturbhuj AU - Agrawal C AD - Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India. FAU - Talwar, Vineet AU - Talwar V AD - Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India. FAU - Saini, Rajeev AU - Saini R AD - Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India. FAU - Babu, Pradeep AU - Babu P AD - Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India. LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Indian J Med Paediatr Oncol JT - Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology JID - 9604571 PMC - PMC5759081 OTO - NOTNLM OT - Bleomycin OT - flagellate rash OT - germ cell tumor COIS- There are no conflicts of interest. EDAT- 2018/01/16 06:00 MHDA- 2018/01/16 06:01 PMCR- 2017/10/01 CRDT- 2018/01/16 06:00 PHST- 2018/01/16 06:00 [entrez] PHST- 2018/01/16 06:00 [pubmed] PHST- 2018/01/16 06:01 [medline] PHST- 2017/10/01 00:00 [pmc-release] AID - IJMPO-38-548 [pii] AID - 10.4103/ijmpo.ijmpo_147_16 [doi] PST - ppublish SO - Indian J Med Paediatr Oncol. 2017 Oct-Dec;38(4):548-551. doi: 10.4103/ijmpo.ijmpo_147_16. PMID- 18068864 OWN - NLM STAT- MEDLINE DCOM- 20080313 LR - 20220408 IS - 1521-6942 (Print) IS - 1521-6942 (Linking) VI - 21 IP - 6 DP - 2007 Dec TI - The contribution of capillaroscopy to the differential diagnosis of connective autoimmune diseases. PG - 1093-108 AB - Raynaud's phenomenon (RP) is one of the earliest clinical hallmarks of microvascular involvement in several connective autoimmune rheumatic diseases. The direct observation of the microvasculature with nailfold videocapillaroscopy (NVC) is useful for an early diagnosis of connective autoimmune diseases (secondary RP) and differentiation from primary (unsymptomatic) RP. Generally, to detect early pathologic capillaroscopic changes, the following parameters are considered: presence of enlarged and giant capillaries, haemorrhages, disorganization of the vascular array, ramified/bushy capillaries and loss of capillaries. Careful capillaroscopic analysis of subjects affected by primary RP can detect the earliest signs of the transition to secondary RP and thus screening procedures for further differential diagnosis within connective autoimmune diseases can be undertaken. In systemic sclerosis, the recognition of clear and different NVC morphological patterns ("early", "active", "late") should suggest including this analysis in the classification criteria of the disease. FAU - Cutolo, Maurizio AU - Cutolo M AD - Internal Medicine and Rheumatology, Research Laboratory and Clinical Academic Unit of Rheumatology, University of Genova, Italy. mcutolo@unige.it FAU - Sulli, Alberto AU - Sulli A FAU - Secchi, Maria Elena AU - Secchi ME FAU - Olivieri, Monica AU - Olivieri M FAU - Pizzorni, Carmen AU - Pizzorni C LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Autoimmune Diseases/*diagnosis MH - Connective Tissue Diseases/*diagnosis MH - Diagnosis, Differential MH - Humans MH - Microscopic Angioscopy/*methods MH - Reproducibility of Results RF - 52 EDAT- 2007/12/11 09:00 MHDA- 2008/03/14 09:00 CRDT- 2007/12/11 09:00 PHST- 2007/12/11 09:00 [pubmed] PHST- 2008/03/14 09:00 [medline] PHST- 2007/12/11 09:00 [entrez] AID - S1521-6942(07)00109-X [pii] AID - 10.1016/j.berh.2007.10.001 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2007 Dec;21(6):1093-108. doi: 10.1016/j.berh.2007.10.001. PMID- 40693094 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250724 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 6 DP - 2025 Jun TI - An Atypical Case of CREST Syndrome With Early Complete Clinical Manifestation. PG - e86500 LID - 10.7759/cureus.86500 [doi] LID - e86500 AB - CREST syndrome, the limited cutaneous subtype of systemic sclerosis, is defined by five classic clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which also forms the initialism. While diagnosis requires the presence of only three criteria, the full expression of all five is uncommon and typically develops gradually over many years. We describe the case of a 44-year-old Spanish-speaking female patient who presented to a community-based health clinic. Her initial evaluation identified the recent onset of Raynaud's phenomenon, esophageal symptoms, sclerodactyly, and facial telangiectasia. Serology was notable for positive anti-centromere and antinuclear antibodies. One year later, she developed a painful mass on her right foot, ultimately identified as dystrophic calcification consistent with calcinosis. This case highlights a rare, rapidly progressive, and complete manifestation of CREST syndrome. This atypical presentation also underscores the importance of early recognition, multidisciplinary management, and careful attention to social determinants of health in patients with autoimmune disease. CI - Copyright © 2025, Schock et al. FAU - Schock, Geil A AU - Schock GA AD - College of Osteopathic Medicine, Michigan State University, East Lansing, USA. FAU - Simon, Dana AU - Simon D AD - College of Osteopathic Medicine, Michigan State University, East Lansing, USA. FAU - Weitzman, Ethan AU - Weitzman E AD - William Beaumont School of Medicine, Oakland University, Auburn Hills, USA. FAU - Weitzman, Raymond AU - Weitzman R AD - Rheumatology, Gary Burnstein Community Health Clinic, Pontiac, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250621 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12279287 OTO - NOTNLM OT - auto immune disease OT - crest syndrome OT - cutaneous calcinosis OT - internal medicine and rheumatology OT - limited cutaneous systemic sclerosis OT - limited scleroderma OT - sclerodactyly OT - social determinants of health (sdoh) OT - systemic sclerosis (ssc) OT - telangiectasia COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/07/22 06:26 MHDA- 2025/07/22 06:27 PMCR- 2025/06/21 CRDT- 2025/07/22 04:45 PHST- 2025/06/21 00:00 [accepted] PHST- 2025/07/22 06:27 [medline] PHST- 2025/07/22 06:26 [pubmed] PHST- 2025/07/22 04:45 [entrez] PHST- 2025/06/21 00:00 [pmc-release] AID - 10.7759/cureus.86500 [doi] PST - epublish SO - Cureus. 2025 Jun 21;17(6):e86500. doi: 10.7759/cureus.86500. eCollection 2025 Jun. PMID- 37621814 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230826 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 7 DP - 2023 Jul TI - Antisynthetase Syndrome: The Classical Phenotype With a Twist. PG - e42360 LID - 10.7759/cureus.42360 [doi] LID - e42360 AB - Antisynthetase syndrome is a systemic autoimmune rheumatic disease characterized by multiple organ involvement, including interstitial lung disease, myositis, non-erosive arthritis, fever, Raynaud's phenomenon, "mechanic's hands," and the presence of autoantibodies against aminoacyl-tRNA synthetases, mainly anti-Jo1 (histidyl) antibodies. Patients with antisynthetase syndrome and active muscle inflammation are usually presented with elevated creatine phosphokinase levels, even in the range of acute rhabdomyolysis. Despite that, the presence of myoglobinuric acute kidney injury is rarely seen in patients with myositis-associated rhabdomyolysis. Herein, we report the case of a 64-year-old man who presented with acute kidney injury due to severe rhabdomyolysis in the setting of antisynthetase syndrome diagnosed by the classical clinical triad of (1) interstitial lung disease, (2) non-erosive arthritis, and (3) active myositis and the presence of anti-Jo1 antibodies. The diagnosis was confirmed by muscle biopsy histological findings as well as electromyography. In this case report, we also discuss the classical clinical manifestations of antisynthetase syndrome and a twist toward this unusual complication associated with active muscle inflammation. CI - Copyright © 2023, Betsikos et al. FAU - Betsikos, Achilleas AU - Betsikos A AD - First Department of Internal Medicine, General Hospital of Trikala, Trikala, GRC. FAU - Gazouni, Evanthia AU - Gazouni E AD - First Department of Internal Medicine, General Hospital of Trikala, Trikala, GRC. FAU - Bika, Spyridoula AU - Bika S AD - First Department of Internal Medicine, General Hospital of Trikala, Trikala, GRC. FAU - Paschou, Eleni AU - Paschou E AD - Department of General Practice and Family Medicine, 10th Local Health Unit of Giannouli, Larisa, GRC. FAU - Sabanis, Nikolaos AU - Sabanis N AD - Department of Nephrology, General Hospital of Trikala, Trikala, GRC. LA - eng PT - Case Reports PT - Journal Article DEP - 20230724 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10445298 OTO - NOTNLM OT - acute rhabdomyolysis OT - anti-jo1 antibodies OT - antisynhetase syndrome OT - inflammatory myopathies OT - myoglobinuric acute kidney injury COIS- The authors have declared that no competing interests exist. EDAT- 2023/08/25 06:42 MHDA- 2023/08/25 06:43 PMCR- 2023/07/24 CRDT- 2023/08/25 03:52 PHST- 2023/07/24 00:00 [accepted] PHST- 2023/08/25 06:43 [medline] PHST- 2023/08/25 06:42 [pubmed] PHST- 2023/08/25 03:52 [entrez] PHST- 2023/07/24 00:00 [pmc-release] AID - 10.7759/cureus.42360 [doi] PST - epublish SO - Cureus. 2023 Jul 24;15(7):e42360. doi: 10.7759/cureus.42360. eCollection 2023 Jul. PMID- 32700874 OWN - NLM STAT- MEDLINE DCOM- 20210617 LR - 20210617 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 72 IP - 2 DP - 2020 Jul 23 TI - The clinical manifestations at the onset of antisynthetase syndrome: A chameleon with multiple faces. PG - 86-92 LID - 10.4081/reumatismo.2020.1275 [doi] AB - The antisynthetase syndrome (ASS) is clinically characterized by fever, myositis, interstitial lung disease, joint involvement, mechanic's hands, or Raynaud's phenomenon, and the presence of antisynthetase autoantibodies. These clinical manifestations may not occur simultaneously. Therefore, the aim of this study was to analyze the sequence in which these clinical manifestations can develop at the onset of ASS. This retrospective, single-center cohort study enrolled 55 ASS patients. Their mean age at the onset of ASS symptoms was 42.3±11.8 years. There was a predominance of female patients (75.9%) and white patients (72.7%). At initial presentation, 41.8% of the patients had fever, 43.6% had joint symptoms, 38.2% had myositis, 36.4% had interstitial lung disease, 18.2% had Raynaud's phenomenon, and 16.4% had mechanic's hands. Subsequent clinical symptoms emerged at varying time points. In two out of 55 cases, joint, muscle, and lung manifestations developed simultaneously. The median time between the onset of symptoms and the complete ASS clinical manifestation was 19.9 (4.0-60.2) months; whereas, the timeframe between the onset of symptoms and the ASS diagnosis was 29.0 (11.0-63.0) months. The confounding misdiagnoses interfering with the initial diagnosis were polymyositis (52.7%), dermatomyositis (29.1%), nonspecific interstitial pneumopathy (23.6%), rheumatoid arthritis (18.2%), and others (10.9%). Clinical features at the onset of ASS are highly variable. Consequently, confounding factors can lead to significant delays for the final and definitive diagnosis of ASS. Therefore, ASS should be considered a differential diagnosis in patients with initial symptoms of joint, lung, and/or muscle involvements, as well as fever, mechanic's hands, and/or Raynaud's phenomenon manifestations. FAU - Baccaro, A C C D AU - Baccaro ACCD AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP. aline.capellato@gmail.com. FAU - Behrens Pinto, G L AU - Behrens Pinto GL AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP. gustavolbp@gmail.com. FAU - Carboni, R C S AU - Carboni RCS AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP. re_casseb@hotmail.com. FAU - Shinjo, S K AU - Shinjo SK AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP. samuel.shinjo@usp.br. LA - eng PT - Journal Article DEP - 20200723 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - Antisynthetase syndrome SB - IM MH - Adult MH - Cohort Studies MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - Myositis/*diagnosis MH - Retrospective Studies MH - *Symptom Assessment EDAT- 2020/07/24 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/07/24 06:00 PHST- 2020/02/20 00:00 [received] PHST- 2020/04/28 00:00 [accepted] PHST- 2020/07/24 06:00 [entrez] PHST- 2020/07/24 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] AID - 10.4081/reumatismo.2020.1275 [doi] PST - epublish SO - Reumatismo. 2020 Jul 23;72(2):86-92. doi: 10.4081/reumatismo.2020.1275. PMID- 39617425 OWN - NLM STAT- MEDLINE DCOM- 20241201 LR - 20250104 IS - 1349-7235 (Electronic) IS - 0918-2918 (Print) IS - 0918-2918 (Linking) VI - 63 IP - 23 DP - 2024 TI - Peliosis Hepatis Complicated by Scleroderma and Rapidly Progressing to Liver Failure. PG - 3171-3178 LID - 10.2169/internalmedicine.3508-24 [doi] AB - In November 2019, a 76-year-old woman was diagnosed with limited cutaneous scleroderma caused by Raynaud's phenomenon and skin hardening on the periphery of the extremities. In October 2022, blood tests and abdominal ultrasonography revealed liver dysfunction and multiple liver masses, respectively. In November 2022, a percutaneous liver mass biopsy revealed peliosis hepatitis, so the patient was referred to Kindai University Hospital, and signs of liver failure were observed. Considering her age, the patient was ineligible for liver transplantation, and she died in September 2023. Peliosis hepatis complicated by scleroderma has not been previously reported, so we report this as a valuable case. FAU - Hagiwara, Satoru AU - Hagiwara S AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Ida, Hiroshi AU - Ida H AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Chikugo, Takaaki AU - Chikugo T AD - Department of Pathology, Kindai University Faculty of Medicine, Japan. FAU - Komeda, Yoriaki AU - Komeda Y AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Nishida, Naoshi AU - Nishida N AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Yoshida, Akihiro AU - Yoshida A AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Yamamoto, Tomoki AU - Yamamoto T AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Matsubara, Takuya AU - Matsubara T AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. FAU - Kudo, Masatoshi AU - Kudo M AD - Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20241201 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 SB - IM MH - Humans MH - Female MH - Aged MH - *Peliosis Hepatis/complications/diagnosis/diagnostic imaging MH - Fatal Outcome MH - *Liver Failure/etiology MH - Disease Progression MH - Scleroderma, Systemic/complications/diagnosis PMC - PMC11671192 OTO - NOTNLM OT - computed tomography OT - liver diseases OT - peliosis hepatis OT - scleroderma COIS- The authors state that they have no Conflict of Interest (COI). EDAT- 2024/12/02 05:30 MHDA- 2024/12/02 05:31 PMCR- 2024/12/01 CRDT- 2024/12/01 21:02 PHST- 2024/12/02 05:31 [medline] PHST- 2024/12/02 05:30 [pubmed] PHST- 2024/12/01 21:02 [entrez] PHST- 2024/12/01 00:00 [pmc-release] AID - 10.2169/internalmedicine.3508-24 [doi] PST - ppublish SO - Intern Med. 2024;63(23):3171-3178. doi: 10.2169/internalmedicine.3508-24. Epub 2024 Dec 1. PMID- 26237437 OWN - NLM STAT- MEDLINE DCOM- 20160811 LR - 20161021 IS - 2532-179X (Electronic) IS - 1124-0490 (Linking) VI - 32 Suppl 1 DP - 2015 Aug 3 TI - Challenges in the classification of fibrotic ILD: Patient case 1. PG - 10-2 AB - PATIENT PRESENTATION AND CLINICAL HISTORY: The patient is a 43-year-old male non-smoker who works as a farmer and cheese-maker. He complained of a dry cough for 6-12 months without fever or other clinical signs. His medical history was unremarkable with no use of drugs or alcohol and no signs of Raynaud's syndrome. In his family history he had two first-degree relatives with pulmonary fibrosis, one of whom also had rheumatoid arthritis. FAU - Manali, Effrosyni D AU - Manali ED AD - ATTIKON University Hospital, National and Kapodistrian University of Athens, Greece. redazione@mattioli1885.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20150803 PL - Italy TA - Sarcoidosis Vasc Diffuse Lung Dis JT - Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG JID - 9610928 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Adult MH - Biopsy, Needle MH - Disease Progression MH - Humans MH - Idiopathic Pulmonary Fibrosis/classification/*drug therapy/*pathology/surgery MH - Immunohistochemistry MH - Lung Transplantation MH - Male MH - Risk Assessment MH - Severity of Illness Index MH - Tomography, X-Ray Computed/methods MH - *Waiting Lists EDAT- 2015/08/04 06:00 MHDA- 2016/08/12 06:00 CRDT- 2015/08/04 06:00 PHST- 2015/08/03 00:00 [received] PHST- 2015/08/03 00:00 [accepted] PHST- 2015/08/04 06:00 [entrez] PHST- 2015/08/04 06:00 [pubmed] PHST- 2016/08/12 06:00 [medline] PST - epublish SO - Sarcoidosis Vasc Diffuse Lung Dis. 2015 Aug 3;32 Suppl 1:10-2. PMID- 24853818 OWN - NLM STAT- MEDLINE DCOM- 20150220 LR - 20161125 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 348 IP - 4 DP - 2014 Oct TI - Measuring microangiopathy abnormalities in systemic sclerosis patients: the role of capillaroscopy-based scoring models. PG - 331-6 LID - 10.1097/MAJ.0000000000000282 [doi] AB - Capillaroscopy is a noninvasive imaging technique for the in vivo study of microcirculation. The role of a qualitative evaluation of capillaroscopy in the assessment of Raynaud's phenomenon secondary to scleroderma spectrum disorder, particularly systemic sclerosis (SSc), is well defined. The usefulness of capillaroscopy in the follow-up of SSc patients and the possible prognostic role for the appearance of typical SSc vascular and visceral involvement, namely, digital ulcers, pulmonary arterial hypertension, and mortality, is suggested by many authors but still under debate. In this regard, and for a reliable and repeatable longitudinal evaluation of SSc microangiopathy, a quantitative analysis should be required. In this review, we describe the current classifications proposed to define the SSc microvascular involvement and the scoring methods suggested for a semiquantitative and quantitative analysis of microangiopathy and its correlation with clinical manifestations of disease. FAU - Sebastiani, Marco AU - Sebastiani M AD - Rheumatology Unit, University of Modena and Reggio Emilia, Modena, Italy. FAU - Manfredi, Andreina AU - Manfredi A FAU - Cassone, Giulia AU - Cassone G FAU - Giuggioli, Dilia AU - Giuggioli D FAU - Ghizzoni, Cecilia AU - Ghizzoni C FAU - Ferri, Clodoveo AU - Ferri C LA - eng PT - Journal Article PT - Review PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 SB - IM MH - Humans MH - *Microcirculation/physiology MH - Microscopic Angioscopy/*standards MH - Radiography MH - Research Design/*standards MH - Scleroderma, Systemic/*diagnostic imaging/physiopathology EDAT- 2014/05/24 06:00 MHDA- 2015/02/24 06:00 CRDT- 2014/05/24 06:00 PHST- 2014/05/24 06:00 [entrez] PHST- 2014/05/24 06:00 [pubmed] PHST- 2015/02/24 06:00 [medline] AID - S0002-9629(15)30218-4 [pii] AID - 10.1097/MAJ.0000000000000282 [doi] PST - ppublish SO - Am J Med Sci. 2014 Oct;348(4):331-6. doi: 10.1097/MAJ.0000000000000282. PMID- 16634365 OWN - NLM STAT- MEDLINE DCOM- 20060816 LR - 20170214 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 15 IP - 3 DP - 2006 TI - Mixed connective tissue disease. PG - 132-7 AB - Mixed connective tissue disease (MCTD) was first described in 1972 as a disease syndrome with overlapping features of systemic sclerosis, systemic lupus erythematosus (SLE) and polymyositis associated with antibodies to RNAse sensitive extractable nuclear antigen. When the antigen was subsequently characterized as polypeptides on the U1 ribonuclear protein component of the splicesosome (U1RNP), MCTD became the first rheumatic disease syndrome to be defined by a serologic test. Clinical features include a high frequency of Raynaud's syndrome, swollen hands, sclerodactyly, arthritis, polymyositis and interstitial lung disease. Over the last 30 years there has been a continuing debate as to whether MCTD constitutes a 'distinct clinical entity'. Here, I will review the pathological, immunogenetic and clinical features of MCTD and conclude that the debate remains unresolved. The early misconception that it has a relatively good prognosis has not stood the test of time with long-term follow-up studies. These have identified a tendency for MCTD to evolve into SLE or systemic sclerosis and highlighted pulmonary hypertension and scleroderma renal crisis as important causes of death. Providing it is realized that our appreciation of the clinical features associated with anti-U1RNP have evolved over time, MCTD remains a useful concept in clinical practice. Whether it can be credited with the term 'disease' awaits the demonstration of common etiopathological events underlying the development of antibodies to U1 RNP and their associated clinical features. FAU - Venables, P J W AU - Venables PJ AD - Kennedy Institute, Imperial College, London, UK. p.venables@imperial.ac.uk LA - eng PT - Journal Article PT - Review PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM MH - Humans MH - Mixed Connective Tissue Disease/diagnosis/etiology/*therapy MH - Ribonucleoprotein, U1 Small Nuclear/chemistry/physiology RF - 19 EDAT- 2006/04/26 09:00 MHDA- 2006/08/17 09:00 CRDT- 2006/04/26 09:00 PHST- 2006/04/26 09:00 [pubmed] PHST- 2006/08/17 09:00 [medline] PHST- 2006/04/26 09:00 [entrez] AID - 10.1191/0961203306lu2283rr [doi] PST - ppublish SO - Lupus. 2006;15(3):132-7. doi: 10.1191/0961203306lu2283rr. PMID- 30512777 OWN - NLM STAT- MEDLINE DCOM- 20190520 LR - 20190520 IS - 2101-017X (Electronic) IS - 0035-2640 (Linking) VI - 67 IP - 7 DP - 2017 Sep TI - [Systemic sclerosis]. PG - 775-783 AB - Systemic sclerosis. Systemic sclerosis is more frequent among women (sex ratio from 3/1 to 8/1) and its prevalence varies from 30 to 240 per millions. Two types of SSc are described depending on the extent of skin involvement : limited and diffuse SSc. Clinical manifestations include fibrosing lesions (skin and pulmonary fibrosis) and vascular abnormalities (Raynaud's phenomenon and pulmonary arterial hypertension (PAH)). Auto-antibodies have been identified in SSc patients, directed at centromere proteins, topoisomerase 1 or RNA polymerase III. SSc is associated with a poor outcome particularly in diffuse forms associated with pulmonary involvement (pulmonary fibrosis and/or PAH). SSc treatment associate symptomatic treatments as well as treatment of visceral complications (PAH, renal crisis) and immunosuppressive therapy in patients with recent onset diffuse SSc and/or rapidely progressing interstitial lung disease. FAU - Legendre, Paul AU - Legendre P AD - Service de médecine interne, hôpital Cochin, Centre de référence national pour les maladies systémiques auto-immunes rares, DHU Authors, Assistance publique-Hôpitaux de Paris, Paris, France. FAU - Mouthon, Luc AU - Mouthon L AD - Institut Cochin, Inserm U1016, CNRS UMR 8104, université Paris-Descartes, Paris, France. LA - fre PT - Journal Article TT - Sclérodermie systémique. PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Female MH - Humans MH - *Hypertension, Pulmonary/etiology MH - *Lung Diseases, Interstitial/etiology MH - *Pulmonary Fibrosis/etiology MH - *Scleroderma, Systemic/complications/diagnosis/therapy MH - Skin OTO - NOTNLM OT - systemic sclerosis COIS- P. Legendre déclare n’avoir aucun lien d’intérêts. L. Mouthon déclare des interventions ponctuelles pour Pfizer, Lilly et Actelion, et avoir été pris en charge lors de congrès par Roche. EDAT- 2017/09/01 00:00 MHDA- 2019/05/21 06:00 CRDT- 2018/12/05 06:00 PHST- 2018/12/05 06:00 [entrez] PHST- 2017/09/01 00:00 [pubmed] PHST- 2019/05/21 06:00 [medline] PST - ppublish SO - Rev Prat. 2017 Sep;67(7):775-783. PMID- 38710221 OWN - NLM STAT- MEDLINE DCOM- 20240522 LR - 20260220 IS - 1098-9048 (Electronic) IS - 1069-3424 (Linking) VI - 45 IP - 3 DP - 2024 Jun TI - Review of Pulmonary Manifestations in Antisynthetase Syndrome. PG - 365-385 LID - 10.1055/s-0044-1785536 [doi] AB - Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations. CI - Thieme. All rights reserved. FAU - Ghanbar, Mohammad I AU - Ghanbar MI AD - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland. FAU - Danoff, Sonye K AU - Danoff SK AD - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland. LA - eng PT - Journal Article PT - Review DEP - 20240506 PL - United States TA - Semin Respir Crit Care Med JT - Seminars in respiratory and critical care medicine JID - 9431858 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Humans MH - *Myositis/diagnosis/complications/immunology MH - *Lung Diseases, Interstitial/diagnosis/etiology/physiopathology/complications MH - Autoantibodies/blood MH - Diagnosis, Differential COIS- None declared. EDAT- 2024/05/07 00:50 MHDA- 2024/05/23 00:42 CRDT- 2024/05/06 19:16 PHST- 2024/05/23 00:42 [medline] PHST- 2024/05/07 00:50 [pubmed] PHST- 2024/05/06 19:16 [entrez] AID - 10.1055/s-0044-1785536 [doi] PST - ppublish SO - Semin Respir Crit Care Med. 2024 Jun;45(3):365-385. doi: 10.1055/s-0044-1785536. Epub 2024 May 6. PMID- 26932289 OWN - NLM STAT- MEDLINE DCOM- 20170215 LR - 20191113 IS - 1573-3971 (Print) IS - 1573-3971 (Linking) VI - 9 IP - 4 DP - 2013 TI - Early Diagnostic and Predictive Value of Capillaroscopy in Systemic Sclerosis. PG - 249-53 AB - Nailfold microvascular impairment represents an early feature of systemic sclerosis (SSc) and its progression through different patterns of capillary damage and their validated scoring, is evaluable by nailfold videocapillaroscopy (NVC) in a safe and reliable manner. The presence of specific morphological microvascular alterations at the NVC (i.e., presence of giant capillaries) is fundamental and mandatory for the early diagnosis of SSc, together with the presence of the Raynaud's phenomenon. Furthermore, a recent longitudinal study showed a dynamic transition of microvascular damage through different NVC patterns of microangiopathy in almost 50% of SSc patients and clinical symptoms progressed in accordance with the NVC morphologic changes in 60% of the SSc patients. A pilot study was the first demonstrating an association between baseline NVC patterns and future severe, peripheral vascular and lung involvement with stronger odds according to worsening scleroderma patterns. Prognostic indexes for digital trophic lesions, especially for daily use in SSc clinics and simply limited to the mean score of capillary loss are now validated. Very recently, it has been described that efficacious potentially disease modifying therapies in SSc may interfere with progression of nailfold microvascular damage, as assessed by NVC, over long term at least in presence of digital ulcers. NVC is a safe and reliable tool for the early diagnosis of SSc and the different NVC scleroderma patterns have a predictive value for the clinical complications of the disease. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova Italy. cutolo@unige.it. FAU - Pizzorni, Carmen AU - Pizzorni C FAU - Sulli, Alberto AU - Sulli A FAU - Smith, Vanessa AU - Smith V LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Early Diagnosis MH - Humans MH - Microscopic Angioscopy/*methods MH - Scleroderma, Systemic/*diagnosis EDAT- 2013/01/01 00:00 MHDA- 2017/02/16 06:00 CRDT- 2016/03/03 06:00 PHST- 2016/03/03 06:00 [entrez] PHST- 2013/01/01 00:00 [pubmed] PHST- 2017/02/16 06:00 [medline] AID - CRR-EPUB-60156 [pii] AID - 10.2174/157339710904140417125010 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2013;9(4):249-53. doi: 10.2174/157339710904140417125010. PMID- 24021080 OWN - NLM STAT- MEDLINE DCOM- 20131112 LR - 20171116 IS - 0443-5117 (Print) IS - 0443-5117 (Linking) VI - 51 IP - 4 DP - 2013 Jul-Aug TI - [Multicentric hyaline vascular Castleman's disease. A POEMS type variant]. PG - 464-7 AB - BACKGROUND: Castleman's disease is an atypical lymphoproliferative disorder which may be compatible with paraneoplastic manifestations of POEMS syndrome. CLINICAL CASE: a 53 year old man with a history of type 2 diabetes, hypothyroidism and Addison's disease presented with numbness and weakness in limbs, dyspnea, skin hardening, Raynaud's phenomenon, weight loss and fatigue. A physical exam showed tachypnea, generalized cutaneous hyperpigmentation and skin hardening of extremities, muscle weakness, hypoesthesia and hyporeflexia. Laboratory showed hyperprolactinemia, low testosterone, hypothyroidism and Addison's disease. Electrophoresis of proteins showed polyclonal hypergammaglobulinemia. Somatosensory evoked potentials reported peripheral neuropathy and severe axonal polyneuropathy by electromyography. Chest X-rays showed bilateral reticular infiltrates and mediastinal widening. An echocardiogram displayed moderate pulmonary hypertension. Skin biopsy had no evidence of scleroderma. CT reported axillar, mediastinal and retroperitoneal nodes. The mediastinal lesion biopsy reported hyaline vascular Castleman's disease, multicentric variety. He was treated with rituximab. CONCLUSIONS: the case meet criteria for multicentric hyaline vascular Castleman's disease, POEMS variant, treated with rituximab. FAU - Gracia-Ramos, Abraham Edgar AU - Gracia-Ramos AE AD - Instituto Mexicano del Seguro Social, Distrito Federal, Mexico. ken_monsters@hotmail.com FAU - Cruz-Domínguez, María del Pilar AU - Cruz-Domínguez Mdel P FAU - Vera-Lastra, Olga Lidia AU - Vera-Lastra OL LA - spa PT - Case Reports PT - Journal Article TT - Enfermedad de Castleman multicéntrica hialina vascular. Una variante de POEMS. PL - Mexico TA - Rev Med Inst Mex Seguro Soc JT - Revista medica del Instituto Mexicano del Seguro Social JID - 101243727 RN - Multi-centric Castleman's Disease SB - IM MH - Castleman Disease/classification/*diagnosis MH - Humans MH - Male MH - Middle Aged MH - POEMS Syndrome/classification/*diagnosis OTO - NOTNLM OT - POEMS syndrome OT - giant lymph node hyperplasia OT - hiperplasia de ganglio linfático gigante OT - síndrome POEMS EDAT- 2013/09/12 06:00 MHDA- 2013/11/13 06:00 CRDT- 2013/09/12 06:00 PHST- 2013/09/12 06:00 [entrez] PHST- 2013/09/12 06:00 [pubmed] PHST- 2013/11/13 06:00 [medline] PST - ppublish SO - Rev Med Inst Mex Seguro Soc. 2013 Jul-Aug;51(4):464-7. PMID- 38057166 OWN - NLM STAT- Publisher LR - 20231206 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) DP - 2023 Dec 5 TI - [Autologous peripheral stem cell transplantation in systemic sclerosis: An important step forward, but we must temper our enthusiasm!]. LID - S0248-8663(23)01297-3 [pii] LID - 10.1016/j.revmed.2023.11.010 [doi] AB - Three prospective randomized studies have demonstrated the efficacy of autologous hematopoietic stem cell (HSC) transplantation in systemic sclerosis (SSc) on survival. These results encourage us to offer this therapy to patients who have a rapidly progressive disease and who have early symptoms but no advanced visceral involvement. HSC autograft can thus be discussed in patients with diffuse cutaneous SSc with a duration of the disease since the first visceral manifestations (cutaneous, cardiac, digestive, pulmonary, or renal) excluding Raynaud's phenomenon of less than 5 years. However, the indications for HSC autograft in SSc validated at European level and in the national diagnostic and care protocol (PNDS) are broader and some of these indications are debatable, in particular in patients with worsening diffuse interstitial lung disease. These indications are discussed in a reasoned way, taking into account the level of evidence and the toxicity of the HSC autograft. CI - Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Mouthon, L AU - Mouthon L AD - Service de médecine interne, centre de référence maladies auto-immunes systémiques rares d'Île-de-France, hôpital Cochin, Assistance publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Hôpital Cochin, université Paris Cité, AP-HP - CUP, 75014 Paris, France. Electronic address: luc.mouthon@aphp.fr. LA - fre PT - English Abstract PT - Journal Article TT - Autogreffe de cellules souches périphériques dans la sclérodermie systémique : un progrès important, mais il faut tempérer notre enthousiasme ! DEP - 20231205 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM OTO - NOTNLM OT - Autogreffe de cellules souches hématopoïétiques OT - Interstitial lung disease OT - Morbidity OT - Morbidité OT - Mortality OT - Mortalité OT - Peripheral stem cell transplantation OT - Pneumopathie interstitielle diffuse OT - Sclérodermie systémique diffuse OT - Systemic scleroderma EDAT- 2023/12/07 00:42 MHDA- 2023/12/07 00:42 CRDT- 2023/12/06 21:58 PHST- 2023/12/07 00:42 [medline] PHST- 2023/12/07 00:42 [pubmed] PHST- 2023/12/06 21:58 [entrez] AID - S0248-8663(23)01297-3 [pii] AID - 10.1016/j.revmed.2023.11.010 [doi] PST - aheadofprint SO - Rev Med Interne. 2023 Dec 5:S0248-8663(23)01297-3. doi: 10.1016/j.revmed.2023.11.010. PMID- 38597289 OWN - NLM STAT- MEDLINE DCOM- 20240524 LR - 20240610 IS - 2578-5826 (Electronic) IS - 2578-5826 (Linking) VI - 47 IP - 2 DP - 2024 Jun TI - Evolution of diagnostic criteria and new insights into clinical testing in mixed connective tissue disease; anti-survival motor neuron complex antibody as a novel marker of severity of the disease. PG - 52-57 LID - 10.1080/25785826.2024.2338593 [doi] AB - Mixed connective tissue disease (MCTD) is an autoimmune disorder characterized by a combination of clinical features from systemic lupus erythematosus, systemic sclerosis, and inflammatory muscle disease, along with the presence of positive anti-U1-ribonucleoprotein (U1-RNP) antibodies. The exact etiology of the disease remains unclear, but it is believed to involve vascular damage within the context of heightened autoimmune responses. Consequently, Raynaud's phenomenon and pulmonary arterial hypertension are observed in patients with MCTD. While specific biomarkers for MCTD have not yet been identified, the recent study of the utility of anti-survival motor neuron complex (SMN) antibodies in MCTD suggests a promising avenue for further research and the accumulation of additional evidence. FAU - Kubo, Satoshi AU - Kubo S AUID- ORCID: 0000-0001-9693-9263 AD - Department of Molecular Targeted Therapies, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. AD - The First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. FAU - Tanaka, Yoshiya AU - Tanaka Y AUID- ORCID: 0000-0002-0807-7139 AD - The First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. LA - eng PT - Journal Article PT - Review DEP - 20240410 PL - England TA - Immunol Med JT - Immunological medicine JID - 101736847 RN - 0 (Biomarkers) RN - 0 (Autoantibodies) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM MH - Humans MH - *Mixed Connective Tissue Disease/immunology/diagnosis MH - *Biomarkers/blood MH - *Autoantibodies/blood MH - Severity of Illness Index MH - Motor Neurons/immunology MH - Ribonucleoprotein, U1 Small Nuclear/immunology OTO - NOTNLM OT - Mixed connective tissue disease OT - anti-survival motor neuron complex antibodies OT - biomarker OT - survival motor neuron complex EDAT- 2024/04/10 12:42 MHDA- 2024/05/24 06:42 CRDT- 2024/04/10 07:03 PHST- 2024/05/24 06:42 [medline] PHST- 2024/04/10 12:42 [pubmed] PHST- 2024/04/10 07:03 [entrez] AID - 10.1080/25785826.2024.2338593 [doi] PST - ppublish SO - Immunol Med. 2024 Jun;47(2):52-57. doi: 10.1080/25785826.2024.2338593. Epub 2024 Apr 10. PMID- 38674039 OWN - NLM STAT- MEDLINE DCOM- 20240427 LR - 20240511 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 25 IP - 8 DP - 2024 Apr 18 TI - A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease. LID - 10.3390/ijms25084453 [doi] LID - 4453 AB - Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions. FAU - Patel, Puja AU - Patel P AD - Section of Rheumatology, Temple University Hospital, Philadelphia, PA 19140, USA. FAU - Marinock, Jenna M AU - Marinock JM AD - Department of Medicine, Temple University Hospital, Philadelphia, PA 19140, USA. FAU - Ajmeri, Aamir AU - Ajmeri A AUID- ORCID: 0000-0002-7361-5300 AD - Department of Thoracic Medicine, Temple University Hospital, Philadelphia, PA 19140, USA. FAU - Brent, Lawrence H AU - Brent LH AD - Section of Rheumatology, Temple University Hospital, Philadelphia, PA 19140, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20240418 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Female MH - Humans MH - Middle Aged MH - Amino Acyl-tRNA Synthetases/immunology MH - *Autoantibodies/immunology MH - *Lung Diseases, Interstitial/immunology/etiology/complications MH - *Myositis/immunology/complications/diagnosis PMC - PMC11050089 OTO - NOTNLM OT - IIM OT - anti-Jo-1 antibody OT - antisynthetase antibodies OT - antisynthetase syndrome OT - idiopathic inflammatory myopathy OT - interstitial lung disease COIS- Authors P.P., J.M.M. and A.A. declare no conflicts of interest. Author L.H.B. owns stocks in Johnson and Johnson. EDAT- 2024/04/27 09:50 MHDA- 2024/04/27 09:51 PMCR- 2024/04/18 CRDT- 2024/04/27 01:14 PHST- 2024/03/15 00:00 [received] PHST- 2024/04/05 00:00 [revised] PHST- 2024/04/13 00:00 [accepted] PHST- 2024/04/27 09:51 [medline] PHST- 2024/04/27 09:50 [pubmed] PHST- 2024/04/27 01:14 [entrez] PHST- 2024/04/18 00:00 [pmc-release] AID - ijms25084453 [pii] AID - ijms-25-04453 [pii] AID - 10.3390/ijms25084453 [doi] PST - epublish SO - Int J Mol Sci. 2024 Apr 18;25(8):4453. doi: 10.3390/ijms25084453. PMID- 38282947 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241023 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 34 IP - 4 DP - 2023 Dec TI - The Role of Nailfold Capillaroscopy in Evaluating Patients with Interstitial Lung Disease Related to Connective Tissue Disease. PG - 588-591 LID - 10.31138/mjr.20230804.tr [doi] AB - Capillaroscopy is a non-invasive and safe imaging method that allows the evaluation of the microcirculation of the small vessels of the skin. The method's main advantage is the early detection of microvascular changes that may occur in certain connective tissue diseases (CTDs). Today, the presence of specific autoantibodies and capillaroscopic findings are generally accepted and emerge as a powerful diagnostic tool for detecting underlying CTDs in patients with Raynaud's phenomenon. The role of capillaroscopy has also been investigated in patients with CTD and interstitial lung disease (ILD). In these patients, lung involvement is considered one of the most severe complications, potentially leading to significant morbidity and mortality. So far, studies have shown an association of the scleroderma pattern in capillaroscopy with lung involvement in Scleroderma patients. Although there are studies on the association of capillary findings in patients with other CTDs, further efforts are needed to evaluate this technique and produce high-performance algorithms in the early detection of involvement and the progression of (CTD) related ILD (CTD-ILD). The present study aims to perform capillaroscopy in CTDILD patients with different imaging patterns and to correlate the method's findings with those found in high-resolution computed tomography, pulmonary tests, and the immunological profile of patients. Furthermore, the impact of ILD treatment on the capillaroscopic findings will be evaluated. CI - © 2023 The Mediterranean Journal of Rheumatology (MJR). FAU - Venetsanopoulou, Aliki I AU - Venetsanopoulou AI AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Greece. FAU - Goules, Andreas V AU - Goules AV AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Vlachoyiannopoulos, Panayiotis G AU - Vlachoyiannopoulos PG AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece; Institute for Autoimmune Systemic and Neurologic Diseases, Athens, Greece. FAU - Drosos, Alexandros A AU - Drosos AA AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Voulgari, Paraskevi V AU - Voulgari PV AD - Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Greece. LA - eng PT - Journal Article DEP - 20230804 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC10815530 OTO - NOTNLM OT - autoimmune diseases OT - capillaroscopy OT - interstitial lung disease OT - scleroderma COIS- The authors declare no conflict of interest. EDAT- 2024/01/29 06:43 MHDA- 2024/01/29 06:44 PMCR- 2023/08/04 CRDT- 2024/01/29 04:11 PHST- 2022/11/03 00:00 [received] PHST- 2022/11/15 00:00 [revised] PHST- 2022/11/17 00:00 [accepted] PHST- 2024/01/29 06:44 [medline] PHST- 2024/01/29 06:43 [pubmed] PHST- 2024/01/29 04:11 [entrez] PHST- 2023/08/04 00:00 [pmc-release] AID - MJR-34-4-588 [pii] AID - 10.31138/mjr.20230804.tr [doi] PST - epublish SO - Mediterr J Rheumatol. 2023 Aug 4;34(4):588-591. doi: 10.31138/mjr.20230804.tr. eCollection 2023 Dec. PMID- 18798588 OWN - NLM STAT- MEDLINE DCOM- 20081118 LR - 20080918 IS - 1022-386X (Print) IS - 1022-386X (Linking) VI - 18 IP - 8 DP - 2008 Aug TI - Clinical characteristics of Buerger's disease in Iran. PG - 502-5 AB - OBJECTIVE: To determine the clinical course of Buerger's disease as observed in two vascular surgery centers located in the capital of Iran. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Sina and Imam Hospitals, Tehran, Iran, during the years 1997 to 2002. METHODOLOGY: The records of all the patients admitted with Buerger's disease diagnosed on the basis of Shionoya's clinical criteria were studied. Their clinical characteristics, treatment offered and short-term follow-up results are described as frequencies and percentages. RESULTS: A total of 116 patients, aged 41.1+/-11.3 years, were enrolled. All patients were males; 99% of them were smokers with an average of 22.9 pack-years of tobacco use. Lower-extremity was affected in 102 (87.9%) patients, upper-extremity in 3 (2.6%) patients and both in 11 (9.5%). The most frequent reasons for being referred to hospital were ischemic ulcers (90.5%), claudication (87.9%), paresthesia (75.9%), rest pain (66.4%), gangrene (60.3%), Raynaud's phenomenon (23.3%) and thrombophlebitis (9.5%). Diagnostic arteriography, vascular bypass surgery and sympathectomy were performed in 60%, 24% and 83% of the patients, respectively. Sixty-eight patients (58.6%) had one of the following amputations: toe 36 (52.9%), transmetatarsal 3 (4.4%), below knee 25 (36.8%), finger 3 (4.4%) and above knee one (1.5%) patient. CONCLUSION: Since the studied hospitals are the referral centers for vascular surgery in Iran admitting patients with severe symptoms; therefore, a higher number of complications and amputations was found in the present study. Upper extremity involvement as well as the occurrence of thrombophlebitis and Raynaud's phenomenon was rather infrequent among the studied cases. FAU - Salimi, Javed AU - Salimi J AD - Department of Surgery, Sina Hospital, Medical Sciences / University of Tehran, Tehran, Iran. swt_f@yahoo.com FAU - Tavakkoli, Hasan AU - Tavakkoli H FAU - Salimzadeh, Ahmad AU - Salimzadeh A FAU - Ghadimi, Hadi AU - Ghadimi H FAU - Habibi, Gholamreza AU - Habibi G FAU - Masoumi, Amir Ali AU - Masoumi AA LA - eng PT - Journal Article PL - Pakistan TA - J Coll Physicians Surg Pak JT - Journal of the College of Physicians and Surgeons--Pakistan : JCPSP JID - 9606447 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Female MH - Humans MH - Iran/epidemiology MH - Male MH - Middle Aged MH - Prospective Studies MH - Risk Factors MH - Thromboangiitis Obliterans/*diagnosis/epidemiology/physiopathology/surgery MH - Vascular Diseases EDAT- 2008/09/19 09:00 MHDA- 2008/11/19 09:00 CRDT- 2008/09/19 09:00 PHST- 2007/05/21 00:00 [received] PHST- 2008/07/11 00:00 [accepted] PHST- 2008/09/19 09:00 [pubmed] PHST- 2008/11/19 09:00 [medline] PHST- 2008/09/19 09:00 [entrez] AID - 040579197 [pii] PST - ppublish SO - J Coll Physicians Surg Pak. 2008 Aug;18(8):502-5. PMID- 24658990 OWN - NLM STAT- MEDLINE DCOM- 20141124 LR - 20140324 IS - 1735-9694 (Electronic) IS - 0044-6025 (Linking) VI - 52 IP - 1 DP - 2014 Feb 22 TI - An Iranian scoring system for diagnosing Buerger's disease. PG - 60-5 AB - Buerger's disease or thromboangiitis obliterans (TAO) seems to be common in IR Iran, The present study aimed to evaluate an Iranian population with Buerger's disease in order to suggest a diagnostic criterion for Buerger's disease based on the most frequent findings and to compare it with Papa diagnostic criteria. In a cross-sectional study, all patients with resting limb pain, limb ischemic ulcers, intermittent claudication and limb ischemia who referred to the Vascular Clinic of Sina Hospital during 2009-2011 were evaluated. The patients were allocated to Buerger's and non-Buerger's groups; Evaluating 122 patients (61 in each group), according to the model each clinical manifestations and risk factors in the patients with Buerger's disease obtained a score. Absent pulsation, abnormal distal Doppler sonography and ischemic ulcer were respectively present in 58 (95.1%), 58 (95.1%) and 49 (80.3%) individuals with Buerger's disease. Multivariate linear regression analysis and multivariate logistic regression analysis were used for modeling. Considering the model finding findings, diagnostic criteria including age, sex, smoking, Raynaud's phenomenon, abnormal proximal Doppler, diabetes mellitus and hyperlipidemia were suggested (R2=0.582); the sensitivity and specificity of the criteria was respectively 95.1% and 78.7%. Compared with Papa criteria, Kappa coefficient was measured at 0.66 with a P-value<0.001. It seems that the recommended criteria have an acceptable accuracy in diagnosing Buerger's disease, especially in the Iranian population; however, it is necessary to conduct more studies with larger sample sizes to evaluate the criteria, especially in other populations. FAU - Ramin, Mohammad AU - Ramin M AD - Department of Surgery, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran.. Meysamie@tums.ac.ir. FAU - Salimi, Javad AU - Salimi J AD - Department of Vascular Surgery, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran.. Meysamie@tums.ac.ir. FAU - Meysamie, Alipasha AU - Meysamie A AD - Department of Community and Preventive Medicine, Medical Faculty, Tehran University of Medical Sciences, Tehran, Iran.. Meysamie@tums.ac.ir. LA - eng PT - Journal Article DEP - 20140222 PL - Iran TA - Acta Med Iran JT - Acta medica Iranica JID - 14540050R SB - IM MH - Cross-Sectional Studies MH - Female MH - Humans MH - Iran MH - Male MH - Thromboangiitis Obliterans/*diagnosis/physiopathology EDAT- 2014/03/25 06:00 MHDA- 2014/12/15 06:00 CRDT- 2014/03/25 06:00 PHST- 2014/02/19 00:00 [received] PHST- 2014/02/19 00:00 [accepted] PHST- 2014/03/25 06:00 [entrez] PHST- 2014/03/25 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] PST - epublish SO - Acta Med Iran. 2014 Feb 22;52(1):60-5. PMID- 16823587 OWN - NLM STAT- MEDLINE DCOM- 20070330 LR - 20181113 IS - 0340-1855 (Print) IS - 0340-1855 (Linking) VI - 65 IP - 4 DP - 2006 Jul TI - [Capillaroscopy and rheumatic diseases: state of the art]. PG - 290-6 AB - Nailfold capillaroscopy (NVC) represents the best method for analyzing microvascular abnormalities in rheumatic diseases. Raynaud's phenomenon (RP) represents the most frequent clinical aspect of microvascular involvement and is a key feature of several such diseases. Under normal conditions or in primary RP (exclusion by the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops within the nail bed. However, in subjects suffering from secondary RP, one or more alterations in the capillaroscopic findings should alert the physician to search for an underlying connective tissue disease. Architectural disorganization, giant capillaries, hemorrhages, loss of capillaries and avascular areas characterize more than 95% of patients with overt systemic sclerosis (scleroderma, SSc). Therefore, the term "scleroderma pattern", includes all capillaroscopic changes typical of the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in undifferentiated connective tissue disease are generally reported as "scleroderma-like patterns". This peripheral microangiopathy can be effectively detected early in the course of the disease and studied in detail by nailfold capillaroscopy or, better, with NVC. In addition, early differential diagnosis between primary and secondary RP is the greatest advantage NVC has to offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, antiphospholipid syndrome and Sjögren's syndrome. However, further epidemiological and clinical studies are needed to better standardize NVC patterns. FAU - Cutolo, M AU - Cutolo M AD - Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV,6, 16132, Genova, Italy. mcutolo@unige.it FAU - Sulli, A AU - Sulli A FAU - Secchi, M E AU - Secchi ME FAU - Pizzorni, C AU - Pizzorni C LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Kapillarmikroskopie und rheumatische Erkrankungen: State of the art. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Antiphospholipid Syndrome/diagnosis MH - Arthritis, Psoriatic/diagnosis MH - Capillaries/pathology MH - Dermatomyositis/diagnosis MH - Diagnosis, Differential MH - Disease Progression MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis MH - *Microscopic Angioscopy MH - Prognosis MH - Rheumatic Diseases/*diagnosis MH - Scleroderma, Systemic/diagnosis RF - 35 EDAT- 2006/07/11 09:00 MHDA- 2007/03/31 09:00 CRDT- 2006/07/11 09:00 PHST- 2006/07/11 09:00 [pubmed] PHST- 2007/03/31 09:00 [medline] PHST- 2006/07/11 09:00 [entrez] AID - 10.1007/s00393-006-0071-2 [doi] PST - ppublish SO - Z Rheumatol. 2006 Jul;65(4):290-6. doi: 10.1007/s00393-006-0071-2. PMID- 39867941 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250130 IS - 2213-0071 (Print) IS - 2213-0071 (Electronic) IS - 2213-0071 (Linking) VI - 53 DP - 2025 TI - Pulmonary fibrosis as the sole manifestation of anti-Ku antibody positivity in the absence of myositis: A case report. PG - 102165 LID - 10.1016/j.rmcr.2025.102165 [doi] LID - 102165 AB - BACKGROUND: Anti-Ku antibodies are autoantibodies directed against the Ku protein complex involved in DNA repair. They are typically associated with overlap syndromes featuring polymyositis and systemic sclerosis. Isolated pulmonary involvement without myositis is exceedingly rare. CASE PRESENTATION: We report the case of a 70-year-old male, a former smoker with an 18-year smoking history who quit 20 years ago, presenting with a one-year history of progressive dyspnea and dry cough. High-resolution computed tomography (HRCT) revealed pulmonary fibrosis with areas of ground-glass opacities. Laboratory tests showed antinuclear antibodies at a titer of 1:2560 with a speckled pattern and positivity for anti-Ku antibodies. Creatine phosphokinase levels were within normal limits. There were no clinical signs of myositis, myalgia, skin manifestations, or Raynaud's phenomenon. CONCLUSION: This case underscores the rarity of pulmonary fibrosis as the sole clinical manifestation associated with anti-Ku antibody positivity in the absence of myositis. Clinicians should consider testing for anti-Ku antibodies in patients with idiopathic interstitial lung disease, even when muscular and cutaneous symptoms are absent. CI - © 2025 The Author. FAU - Nigro, Angelo AU - Nigro A AD - Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20250102 PL - England TA - Respir Med Case Rep JT - Respiratory medicine case reports JID - 101604463 PMC - PMC11759561 OTO - NOTNLM OT - Anti-Ku antibodies OT - Autoimmunity OT - Case report OT - Interstitial lung disease OT - Myositis OT - Pulmonary fibrosis COIS- The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/01/27 06:20 MHDA- 2025/01/27 06:21 PMCR- 2025/01/02 CRDT- 2025/01/27 05:59 PHST- 2024/09/27 00:00 [received] PHST- 2024/12/28 00:00 [revised] PHST- 2025/01/01 00:00 [accepted] PHST- 2025/01/27 06:21 [medline] PHST- 2025/01/27 06:20 [pubmed] PHST- 2025/01/27 05:59 [entrez] PHST- 2025/01/02 00:00 [pmc-release] AID - S2213-0071(25)00001-2 [pii] AID - 102165 [pii] AID - 10.1016/j.rmcr.2025.102165 [doi] PST - epublish SO - Respir Med Case Rep. 2025 Jan 2;53:102165. doi: 10.1016/j.rmcr.2025.102165. eCollection 2025. PMID- 38479828 OWN - NLM STAT- MEDLINE DCOM- 20240315 LR - 20260314 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 17 IP - 3 DP - 2024 Mar 13 TI - Unusual overlap of systemic sclerosis with Takayasu arteritis. LID - 10.1136/bcr-2023-256858 [doi] LID - e256858 AB - Overlap syndromes are diseases that meet the criteria of two or more rheumatic diseases. In this case report, a woman in her 20s presented with a constellation of symptoms, including skin thickening, Raynaud's phenomenon, hypertension, absent pulse in both lower limbs with bilateral renal artery bruit. The antinuclear antibody profile revealed Scl-70 positivity. CT thorax identified early interstitial lung disease, and nailfold capillaroscopy showed severe capillary loss. CT angiogram features were suggestive of Takayasu arteritis. Notably, there have been only four documented cases of systemic sclerosis coexisting with Takayasu arteritis, highlighting the rarity of this overlap syndrome. The diagnosis of overlap syndrome was made after a thorough history recording and clinical examination. In the presence of bilateral renal artery stenosis, managing the scleroderma renal crisis may be challenging . This patient received treatment with mycophenolate mofetil and oral corticosteroids, aiming to address both systemic sclerosis and Takayasu arteritis effectively. CI - © BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Chithrabhanu, Anoop AU - Chithrabhanu A AUID- ORCID: 0009-0002-1004-2282 AD - Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India anooppaloor@gmail.com arulrajamurugan@gmail.com. FAU - Ponniah Subramanian, Arul Rajamurugan AU - Ponniah Subramanian AR AD - Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India anooppaloor@gmail.com arulrajamurugan@gmail.com. FAU - Ramamoorthy, Ramesh AU - Ramamoorthy R AD - Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India. FAU - Seetharaman Varadhan, Mythili AU - Seetharaman Varadhan M AD - Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20240313 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Female MH - Humans MH - *Takayasu Arteritis/diagnosis/diagnostic imaging MH - *Scleroderma, Systemic/complications/diagnosis MH - *Connective Tissue Diseases/complications MH - *Scleroderma, Localized/complications MH - *Lung Diseases, Interstitial/complications MH - *Autoimmune Diseases/complications PMC - PMC10941143 OTO - NOTNLM OT - Connective tissue disease OT - Hypertension OT - Vasculitis COIS- Competing interests: None declared. EDAT- 2024/03/14 00:43 MHDA- 2024/03/15 06:43 PMCR- 2026/03/13 CRDT- 2024/03/13 21:23 PHST- 2024/03/15 06:43 [medline] PHST- 2024/03/14 00:43 [pubmed] PHST- 2024/03/13 21:23 [entrez] PHST- 2026/03/13 00:00 [pmc-release] AID - 17/3/e256858 [pii] AID - bcr-2023-256858 [pii] AID - 10.1136/bcr-2023-256858 [doi] PST - epublish SO - BMJ Case Rep. 2024 Mar 13;17(3):e256858. doi: 10.1136/bcr-2023-256858. PMID- 21390438 OWN - NLM STAT- MEDLINE DCOM- 20111110 LR - 20220408 IS - 1806-3756 (Electronic) IS - 1806-3713 (Print) IS - 1806-3713 (Linking) VI - 37 IP - 1 DP - 2011 Jan-Feb TI - Myositis-related interstitial lung disease and antisynthetase syndrome. PG - 100-9 LID - S1806-37132011000100015 [pii] AB - In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable. FAU - Solomon, Joshua AU - Solomon J AD - Interstitial Lung Disease Program, National Jewish Health, 1400 Jackson Street, Denver, CO 80206, USA. solomonj@njhealth.org FAU - Swigris, Jeffrey J AU - Swigris JJ FAU - Brown, Kevin K AU - Brown KK LA - eng LA - por GR - K23 HL092227/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Review PL - Brazil TA - J Bras Pneumol JT - Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia JID - 101222274 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Human Platelet) RN - 0 (Autoantibodies) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/*blood MH - Antibodies, Antinuclear/blood MH - Antigens, Human Platelet/blood MH - Autoantibodies/*blood MH - Humans MH - Lung Diseases, Interstitial/diagnosis/*immunology/therapy MH - Myositis/diagnosis/*immunology/therapy PMC - PMC3676869 MID - NIHMS474713 EDAT- 2011/03/11 06:00 MHDA- 2011/11/11 06:00 PMCR- 2013/06/09 CRDT- 2011/03/11 06:00 PHST- 2010/10/20 00:00 [received] PHST- 2010/10/21 00:00 [accepted] PHST- 2011/03/11 06:00 [entrez] PHST- 2011/03/11 06:00 [pubmed] PHST- 2011/11/11 06:00 [medline] PHST- 2013/06/09 00:00 [pmc-release] AID - S1806-37132011000100015 [pii] AID - 10.1590/s1806-37132011000100015 [doi] PST - ppublish SO - J Bras Pneumol. 2011 Jan-Feb;37(1):100-9. doi: 10.1590/s1806-37132011000100015. PMID- 40289922 OWN - NLM STAT- MEDLINE DCOM- 20250530 LR - 20250530 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 34 IP - 7 DP - 2025 Jun TI - Reversible dilated cardiomyopathy in systemic lupus erythematosus: A case report. PG - 757-760 LID - 10.1177/09612033251337516 [doi] AB - A 40-year-old woman presented with new-onset heart failure with reduced ejection fraction (HFrEF) accompanied with systemic symptoms, including arthralgia, cutaneous lesions, and Raynaud's phenomenon. Initial investigation led to the diagnosis of systemic lupus erythematosus (SLE), with subsequent finding of dilated cardiomyopathy, a rare but severe secondary manifestation of SLE. Early recognition and appropriate management of underlying autoimmune conditions can lead to substantial improvement in cardiac function, emphasizing the need for comprehensive evaluation in patients with de novo heart failure and systemic symptoms. FAU - Reis, Jorge AU - Reis J AD - Internal Medicine Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 FAU - Leite, Marta AU - Leite M AUID- ORCID: 0000-0003-4067-3069 AD - Cardiology Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 FAU - Jesus, Guilherme AU - Jesus G AD - Internal Medicine Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 FAU - Ferreira, Diogo AU - Ferreira D AD - Pneumology Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 FAU - Pereira, Sofia AU - Pereira S AD - Internal Medicine Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 FAU - Seixas, Andreia AU - Seixas A AD - Internal Medicine Department, Unidade Local de Saúde Gaia/Espinho, Vila Nova de Gaia, Portugal. RINGGOLD: 679493 LA - eng PT - Case Reports PT - Journal Article DEP - 20250428 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Humans MH - Female MH - *Lupus Erythematosus, Systemic/complications/diagnosis/drug therapy MH - *Cardiomyopathy, Dilated/etiology/diagnosis/physiopathology MH - Adult MH - Heart Failure/etiology MH - Stroke Volume OTO - NOTNLM OT - Autoimmune disease OT - cardiovascular disease OT - dilated cardiomyopathy OT - heart failure with reduced ejection fraction OT - systemic lupus erythematosus COIS- Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/04/28 06:27 MHDA- 2025/05/30 07:53 CRDT- 2025/04/28 05:53 PHST- 2025/05/30 07:53 [medline] PHST- 2025/04/28 06:27 [pubmed] PHST- 2025/04/28 05:53 [entrez] AID - 10.1177/09612033251337516 [doi] PST - ppublish SO - Lupus. 2025 Jun;34(7):757-760. doi: 10.1177/09612033251337516. Epub 2025 Apr 28. PMID- 29664231 OWN - NLM STAT- MEDLINE DCOM- 20191213 LR - 20210109 IS - 1582-4934 (Electronic) IS - 1582-1838 (Print) IS - 1582-1838 (Linking) VI - 22 IP - 7 DP - 2018 Jul TI - Oxidative stress in the pathogenesis of systemic scleroderma: An overview. PG - 3308-3314 LID - 10.1111/jcmm.13630 [doi] AB - Systemic sclerosis (SSc) is a rare disorder of the connective tissue characterized by fibrosis of the skin, skeletal muscles and visceral organs. Additional manifestations include activation of the immune system and vascular injury. SSc causes disability and death as the result of end-stage organ failure. Two clinical subsets of the SSc are accepted: limited cutaneous SSc (lc-SSc) and diffuse cutaneous SSc (dc-SSc). At present, the aetiology and pathogenesis of SSc remain obscure, and consequently, disease outcome is unpredictable. Numerous studies suggest that reactive oxidizing species (ROS) play an important role in the pathogenesis of scleroderma. Over the years, several reports have supported this hypothesis for both lc-SSc and dc-SSc, although the specific role of oxidative stress in the pathogenesis of vascular injury and fibrosis remains to be clarified. The aim of the present review was to report and comment the recent findings regarding the involvement and role of oxidative stress in SSc pathogenesis. Biomarkers proving the link between ROS and the main pathological features of SSc have been summarized. CI - © Istituto Superiore di Sanità. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine. FAU - Vona, Rosa AU - Vona R AD - Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy. FAU - Giovannetti, Antonello AU - Giovannetti A AD - Department of Clinical Medicine, La Sapienza University, Rome, Italy. FAU - Gambardella, Lucrezia AU - Gambardella L AD - Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy. FAU - Malorni, Walter AU - Malorni W AD - Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy. FAU - Pietraforte, Donatella AU - Pietraforte D AD - Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy. AD - Core Facilities, Istituto Superiore di Sanità, Rome, Italy. FAU - Straface, Elisabetta AU - Straface E AD - Center for Gender-Specific Medicine, Biomarkers Unit, Rome, Italy. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20180417 PL - England TA - J Cell Mol Med JT - Journal of cellular and molecular medicine JID - 101083777 RN - 0 (Antioxidants) RN - 0 (Biomarkers) RN - 0 (Reactive Oxygen Species) SB - IM MH - Antioxidants/metabolism/*pharmacology MH - Biomarkers/metabolism MH - Humans MH - Oxidative Stress/*physiology MH - Reactive Oxygen Species/metabolism MH - Scleroderma, Systemic/drug therapy/*metabolism/*pathology PMC - PMC6010858 OTO - NOTNLM OT - Raynaud's phenomenon OT - antioxidants OT - biomarkers OT - gender differences OT - oxidative stress OT - reactive oxidizing species OT - scleroderma EDAT- 2018/04/18 06:00 MHDA- 2019/12/18 06:00 PMCR- 2018/07/01 CRDT- 2018/04/18 06:00 PHST- 2017/08/06 00:00 [received] PHST- 2018/03/11 00:00 [accepted] PHST- 2018/04/18 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2018/04/18 06:00 [entrez] PHST- 2018/07/01 00:00 [pmc-release] AID - JCMM13630 [pii] AID - 10.1111/jcmm.13630 [doi] PST - ppublish SO - J Cell Mol Med. 2018 Jul;22(7):3308-3314. doi: 10.1111/jcmm.13630. Epub 2018 Apr 17. PMID- 33566708 OWN - NLM STAT- MEDLINE DCOM- 20211125 LR - 20220427 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 31 IP - 6 DP - 2021 Nov TI - Prevalence and clinical association with calcinosis cutis in early systemic sclerosis. PG - 1113-1119 LID - 10.1080/14397595.2021.1886654 [doi] AB - OBJECTIVES: Calcinosis cutis is often found with systemic sclerosis (SSc). However the calcinosis cutis and its clinical association among SSc patients is limited. Our aims were to assess the prevalence of calcinosis cutis and its association with clinical features of SSc patients at early onset of the disease. METHODS: A cross-sectional study on clinical characteristics and hand radiographs of 120 newly diagnosed SSc patients with the onset less than four years were evaluated. Calcinosis cutis was described based on the anatomical regions, density (level 1-3) and shapes (net, plate, stone, and amorphous). RESULTS: Among all SSc patients enrolled, 62.5% were females and 56.1% were diffuse cutaneous SSc. The mean disease duration was 2.0 ± 1.3 years. Calcinosis cutis was detected in 60 patients with the prevalence of 50% (95%confidence interval (CI), 0.41-0.59), of which 53.3% occurred at distal phalanx, 96.7% had stone shape and 48.3% were high density. Univariate analysis revealed that calcinosis cutis was associated with age (p = .02) and high-density calcinosis cutis was associated with Raynaud's phenomenon (p = .02), ischemic ulcer (p = .04), and telangiectasis (p = .02). Logistic regression analysis revealed that calcinosis cutis at distal phalanx was negatively associated with edema at the onset (odds ratio, 0.09). CONCLUSION: Occult calcinosis cutis can be detected by hand radiograph in one half of SSc patients at early onset of the disease. Elderly patient has a risk for calcinosis cutis development and Raynaud's phenomenon was associated with high density calcinosis cutis. Calcinosis cutis, particularly at distal phalanx was less likely to be detected in an edematous phase of disease. FAU - Muktabhant, Chawiporn AU - Muktabhant C AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Thammaroj, Punthip AU - Thammaroj P AUID- ORCID: 0000-0001-5532-0165 AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Chowchuen, Prathana AU - Chowchuen P AD - Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. LA - eng PT - Journal Article DEP - 20210222 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Aged MH - *Calcinosis/complications/diagnostic imaging/epidemiology MH - Cross-Sectional Studies MH - Female MH - Humans MH - Prevalence MH - *Scleroderma, Diffuse MH - *Scleroderma, Systemic/complications/diagnostic imaging/epidemiology OTO - NOTNLM OT - Calcinosis cutis OT - hand radiography OT - scleroderma related disease OT - systemic sclerosis EDAT- 2021/02/11 06:00 MHDA- 2021/11/26 06:00 CRDT- 2021/02/10 17:10 PHST- 2021/02/11 06:00 [pubmed] PHST- 2021/11/26 06:00 [medline] PHST- 2021/02/10 17:10 [entrez] AID - 10.1080/14397595.2021.1886654 [doi] PST - ppublish SO - Mod Rheumatol. 2021 Nov;31(6):1113-1119. doi: 10.1080/14397595.2021.1886654. Epub 2021 Feb 22. PMID- 33194166 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220418 IS - 2040-6223 (Print) IS - 2040-6231 (Electronic) IS - 2040-6223 (Linking) VI - 11 DP - 2020 TI - Interstitial lung disease is a major characteristic of anti-KS associated ant-synthetase syndrome. PG - 2040622320968417 LID - 10.1177/2040622320968417 [doi] LID - 2040622320968417 AB - BACKGROUND: Anti-KS autoantibodies are rare myositis-specific autoantibodies that have been described to target asparaginyl-transfer RNA synthetase. METHODS: Here, we review the published literature on critical issues concerning the detection of anti-KS antibodies and the clinical features associated with their presence. RESULTS: Seven articles are reviewed, in all of which immunoprecipitation was employed for the detection of anti-KS antibodies. A total of 47 patients were included; the ratio of females to males was 1.9:1. In total, 46 (98%) of these patients had interstitial lung disease (ILD), which was the sole manifestation in half (50%) of them. Pulmonary pathology revealed 7 (27%) with usual interstitial pneumonia, and 16 (62%) with non-specific pneumonia. Arthritis was present in about one-quarter (26%) of patients, and the incidence of Raynaud's phenomenon and mechanic's hands was 19% and 32%, respectively. However, manifestations of myositis were rare (9%). In addition, three (11%) patients had malignant tumors. Most patients responded to glucocorticoid therapy. CONCLUSIONS: Identifying anti-KS in patients with ILD may be useful for treatment, but reliable practical detection is needed. Furthermore, clinicians need to be aware of the possible presence of anti-KS antibodies in patients with ILD, either isolated or in combination with myositis. CI - © The Author(s), 2020. FAU - Ge, Yongpeng AU - Ge Y AUID- ORCID: 0000-0003-3674-9307 AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - Li, Sizhao AU - Li S AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - Li, Shanshan AU - Li S AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - He, Linrong AU - He L AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - Lu, Xin AU - Lu X AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - Wang, Guochun AU - Wang G AD - Department of Rheumatology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, 100029, Beijing, China. LA - eng PT - Journal Article PT - Review DEP - 20201029 PL - United States TA - Ther Adv Chronic Dis JT - Therapeutic advances in chronic disease JID - 101532140 PMC - PMC7605033 OTO - NOTNLM OT - anti-KS autoantibodies OT - anti-asparaginyl-tRNA synthetase OT - anti-synthetase syndrome OT - interstitial lung disease COIS- Conflict of interest statement: The authors declare that there is no conflict of interest. EDAT- 2020/11/17 06:00 MHDA- 2020/11/17 06:01 PMCR- 2020/10/29 CRDT- 2020/11/16 08:50 PHST- 2020/07/26 00:00 [received] PHST- 2020/10/01 00:00 [accepted] PHST- 2020/11/16 08:50 [entrez] PHST- 2020/11/17 06:00 [pubmed] PHST- 2020/11/17 06:01 [medline] PHST- 2020/10/29 00:00 [pmc-release] AID - 10.1177_2040622320968417 [pii] AID - 10.1177/2040622320968417 [doi] PST - epublish SO - Ther Adv Chronic Dis. 2020 Oct 29;11:2040622320968417. doi: 10.1177/2040622320968417. eCollection 2020. PMID- 25540671 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20141225 LR - 20200929 IS - 1755-8166 (Print) IS - 1755-8166 (Electronic) IS - 1755-8166 (Linking) VI - 7 IP - 1 DP - 2014 TI - Familial 1q22 microduplication associated with psychiatric disorders, intellectual disability and late-onset autoimmune inflammatory response. PG - 90 LID - 10.1186/s13039-014-0090-7 [doi] LID - 90 AB - BACKGROUND: Despite the extensive use of chromosomal microarray technologies in patients with neurodevelopmental disorders has permitted the identification of an increasing number of causative submicroscopic rearrangements throughout the genome, constitutional duplications involving chromosome 1q22 have seldom been described in those patients. RESULTS: We report on a pedigree with seven affected members showing varying degrees of behavioural and emotional disturbances including general anxiety disorder, mood disorders, and intellectual disability. Two adult female patients also showed late onset autoimmune inflammatory responses characterized by alopecia, skin ulcers secondary to inflammatory vasculitis, interstitial lung disease, and Raynaud's phenomenon. Array-CGH analysis identified in the affected individuals a 290 Kb microduplication in the chromosome 1q22. The rearrangement involves eleven known genes and is not present in the databases of polymorphic copy number variants. CONCLUSIONS: The rearrangement segregates with the neurological clinical features observed in our patients, suggesting that dosage imbalance of one or more genes in this genomic region may lead to the observed phenotype. The association between the microduplication and the inflammatory disease is much less evident. Additional reported patients carrying similar microduplications are needed to clarify this aspect. FAU - Fichera, Marco AU - Fichera M AD - Department of Biomedical and Biotechnological Sciences, Medical Genetics, University of Catania, Catania, Italy ; Laboratory of Medical Genetics, I.R.C.C.S. Associazione Oasi Maria Santissima, Troina, Italy. FAU - Barone, Rita AU - Barone R AD - Department of Clinical and Experimental Medicine, Child Neurology and Psychiatry, University of Catania, Catania, Italy. FAU - Grillo, Lucia AU - Grillo L AD - Laboratory of Medical Genetics, I.R.C.C.S. Associazione Oasi Maria Santissima, Troina, Italy. FAU - De Grandi, Mariaclara AU - De Grandi M AD - Department of Clinical and Experimental Medicine, Child Neurology and Psychiatry, University of Catania, Catania, Italy. FAU - Fiore, Valerio AU - Fiore V AD - Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy. FAU - Morana, Ignazio AU - Morana I AD - Internal Medicine Unit, Garibaldi Hospital, Catania, Italy. FAU - Maniscalchi, Tiziana AU - Maniscalchi T AD - Department of Biomedical and Biotechnological Sciences, Medical Genetics, University of Catania, Catania, Italy. FAU - Vinci, Mirella AU - Vinci M AD - Laboratory of Medical Genetics, I.R.C.C.S. Associazione Oasi Maria Santissima, Troina, Italy. FAU - Amata, Silvestra AU - Amata S AD - Laboratory of Medical Genetics, I.R.C.C.S. Associazione Oasi Maria Santissima, Troina, Italy. FAU - Spalletta, Angela AU - Spalletta A AD - Laboratory of Medical Genetics, I.R.C.C.S. Associazione Oasi Maria Santissima, Troina, Italy. FAU - Sorge, Giovanni AU - Sorge G AD - Department of Clinical and Experimental Medicine, Pediatric Clinic, University of Catania, Catania, Italy. FAU - Signorelli, Salvatore Santo AU - Signorelli SS AD - Department of Clinical and Experimental Medicine, Medical Angiology Unit, University of Catania, Catania, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20141219 PL - England TA - Mol Cytogenet JT - Molecular cytogenetics JID - 101317942 PMC - PMC4276019 OTO - NOTNLM OT - 1q22 OT - CBCL dysregulation syndrome OT - CNV OT - Duplication OT - Inflammatory disease OT - Intellectual disability OT - LAMTOR2 OT - LMNA OT - SEMA4A EDAT- 2014/12/30 06:00 MHDA- 2014/12/30 06:01 PMCR- 2014/12/19 CRDT- 2014/12/26 06:00 PHST- 2014/09/22 00:00 [received] PHST- 2014/11/16 00:00 [accepted] PHST- 2014/12/26 06:00 [entrez] PHST- 2014/12/30 06:00 [pubmed] PHST- 2014/12/30 06:01 [medline] PHST- 2014/12/19 00:00 [pmc-release] AID - 90 [pii] AID - 10.1186/s13039-014-0090-7 [doi] PST - epublish SO - Mol Cytogenet. 2014 Dec 19;7(1):90. doi: 10.1186/s13039-014-0090-7. eCollection 2014. PMID- 38280030 OWN - NLM STAT- MEDLINE DCOM- 20240129 LR - 20241202 IS - 1591-9528 (Electronic) IS - 1591-8890 (Print) IS - 1591-8890 (Linking) VI - 24 IP - 1 DP - 2024 Jan 27 TI - The relationship between peripheral T follicular helper cells and disease severity in systemic sclerosis. PG - 19 LID - 10.1007/s10238-023-01286-9 [doi] LID - 19 AB - We aimed to investigate the association between follicular T helper cells (Tfh) and disease severity in systemic sclerosis (SSc), a chronic connective tissue disease characterized by progressive fibrosis. While Tfh cells have been extensively studied in other autoimmune diseases, their role in SSc remains poorly understood. A cohort of 50 SSc patients, diagnosed based on the ACR/EULAR 2013 classification criteria, was included in the study. Patient data, including demographic information, comorbidities, treatment history and organ involvement, were collected. Disease severity was assessed using the modified Rodnan skin score and Medsger disease severity index. Statistical analyses were performed, considering a p value of < 0.05 as statistically significant. 38% had SSc with limited skin involvement, while 62% had SSc with extensive skin involvement. However, there were no statistically significant differences observed in the levels of CD4+ CXCR5+ , CD4+ ICOS+ , CD4+ CD40L+ and CD4+ PD+ lymphocytes between the two groups. Notably, SSc patients with Raynaud's phenomenon, digital ulcer and lung involvement exhibited higher levels of CD4+ CXCR5+ lymphocytes compared to those without these manifestations. Furthermore, a significant positive correlation was observed between CD4+ CXCR5+ lymphocyte levels and the severity of lung disease according to the Medsger disease severity index. Based on these findings, we conclude that elevated levels of Tfh cells are associated with lung involvement in SSc and there is a significant correlation between Tfh cell levels and the severity of lung disease. These observations suggest a potential role for Tfh cells in the pathogenesis of lung involvement in SSc and may guide the development of targeted therapies for this aspect of the disease. CI - © 2024. The Author(s). FAU - Sahinoglu, Melek AU - Sahinoglu M AD - Department of Internal Medicine, Aydin Adnan Menderes University, Aydin, Turkey. FAU - Sargin, Gokhan AU - Sargin G AD - Department of Rheumatology, Aydin Adnan Menderes University, Aydin, Turkey. gokhan_sargin@hotmail.com. FAU - Yavasoglu, Irfan AU - Yavasoglu I AD - Department of Hematology, Aydin Adnan Menderes University, Aydin, Turkey. FAU - Senturk, Taskin AU - Senturk T AD - Department of Rheumatology, Aydin Adnan Menderes University, Aydin, Turkey. LA - eng PT - Journal Article DEP - 20240127 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Interleukins) SB - IM MH - Humans MH - T Follicular Helper Cells MH - Interleukins MH - *Scleroderma, Systemic MH - Severity of Illness Index MH - *Lung Diseases PMC - PMC10822004 OTO - NOTNLM OT - Disease severity OT - Peripheral T follicular helper cells OT - Systemic sclerosis COIS- All authors declare that they have no conflict of interest. EDAT- 2024/01/28 07:42 MHDA- 2024/01/29 06:44 PMCR- 2024/01/27 CRDT- 2024/01/27 11:14 PHST- 2023/09/02 00:00 [received] PHST- 2023/11/04 00:00 [accepted] PHST- 2024/01/29 06:44 [medline] PHST- 2024/01/28 07:42 [pubmed] PHST- 2024/01/27 11:14 [entrez] PHST- 2024/01/27 00:00 [pmc-release] AID - 10.1007/s10238-023-01286-9 [pii] AID - 1286 [pii] AID - 10.1007/s10238-023-01286-9 [doi] PST - epublish SO - Clin Exp Med. 2024 Jan 27;24(1):19. doi: 10.1007/s10238-023-01286-9. PMID- 35382392 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 4 IP - 2 DP - 2019 Jun TI - The effects of a single carbon dioxide and hot water hand bath on acral perfusion in systemic sclerosis: A randomized, clinical study. PG - 160-162 LID - 10.1177/2397198318819919 [doi] AB - Secondary Raynaud's phenomenon is the most common manifestation of systemic sclerosis, affecting more than 99% of systemic sclerosis patients, and a major cause of morbidity. Frequent and prolonged secondary Raynaud's phenomenon attacks not only cause severe discomfort and pain but also ischemic acral tissue damage. In addition to vasoactive drugs, carbon dioxide (CO(2)) hand bath and hot water bath are potential non-pharmacological treatment options which can be self-administered by affected patients at any time. In order to compare the efficacy of these two physical measures, this randomized, clinical study evaluated the effects of a single CO(2) hand bath in patients with systemic sclerosis and secondary Raynaud's phenomenon and a healthy control group versus a single hot water hand bath on acral perfusion in systemic sclerosis by Doppler ultrasonography. None of the patients had currently digital ulcers, a vasoactive medication or a concomitant vascular disease. CO(2) immersion induced an acute hemodynamic response, whereas hot water immersion had no significant effect on acral perfusion in systemic sclerosis. CI - © The Author(s) 2019. FAU - Lange, Uwe AU - Lange U AUID- ORCID: 0000-0001-7904-0619 AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Kerckhoff-Klinik and Justus Liebig University Giessen, Bad Nauheim, Germany. FAU - Bogensperger, Stephanie AU - Bogensperger S AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Kerckhoff-Klinik and Justus Liebig University Giessen, Bad Nauheim, Germany. FAU - Tarner, Ingo H AU - Tarner IH AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Kerckhoff-Klinik and Justus Liebig University Giessen, Bad Nauheim, Germany. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Rheumatology, Clinical Immunology, Osteology and Physical Medicine, Kerckhoff-Klinik and Justus Liebig University Giessen, Bad Nauheim, Germany. LA - eng PT - Journal Article DEP - 20190109 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922646 OTO - NOTNLM OT - Doppler ultrasonography OT - Systemic sclerosis OT - carbon dioxide OT - hot water COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2019/06/01 00:00 MHDA- 2019/06/01 00:01 PMCR- 2020/06/01 CRDT- 2022/04/06 05:15 PHST- 2018/08/14 00:00 [received] PHST- 2018/11/27 00:00 [accepted] PHST- 2022/04/06 05:15 [entrez] PHST- 2019/06/01 00:00 [pubmed] PHST- 2019/06/01 00:01 [medline] PHST- 2020/06/01 00:00 [pmc-release] AID - 10.1177_2397198318819919 [pii] AID - 10.1177/2397198318819919 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2019 Jun;4(2):160-162. doi: 10.1177/2397198318819919. Epub 2019 Jan 9. PMID- 35393091 OWN - NLM STAT- MEDLINE DCOM- 20220411 LR - 20230213 IS - 2542-4513 (Electronic) IS - 2542-4513 (Print) IS - 2542-4513 (Linking) VI - 47 IP - 1 DP - 2022 Feb TI - Microvascular manifestations revealing vaccine-induced thrombotic thrombocytopenia after COVID-19 vaccination. PG - 36-38 LID - S2542-4513(22)00072-4 [pii] LID - 10.1016/j.jdmv.2022.01.072 [doi] FAU - Noyé, M AU - Noyé M AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France. FAU - Sauvage, A AU - Sauvage A AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France. FAU - Toussaint, M AU - Toussaint M AD - Laboratory Haematology Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France. FAU - Benoit, R AU - Benoit R AD - Laboratory Haematology Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France. FAU - Foret, T AU - Foret T AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France; Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. FAU - Lagrange, Jeremy AU - Lagrange J AD - Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. FAU - Dufrost, V AU - Dufrost V AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France; Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. FAU - Regnault, V AU - Regnault V AD - Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. FAU - Zuily, S AU - Zuily S AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France; Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. FAU - Wahl, D G AU - Wahl DG AD - Vascular Medicine Division, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Nancy, France; Inserm UMR_S 1116 DCAC, Faculté de Médecine/Maïeutique/Métiers de la santé à Nancy, University of Lorraine, Nancy, France. Electronic address: d.wahl@chru-nancy.fr. LA - eng PT - Letter DEP - 20220117 PL - France TA - J Med Vasc JT - Journal de medecine vasculaire JID - 101709200 RN - 0 (COVID-19 Vaccines) RN - 0 (Vaccines) SB - IM EIN - J Med Vasc. 2022 Jul-Aug;47(3):164-165. doi: 10.1016/j.jdmv.2022.07.006. PMID: 36055689 MH - *COVID-19/prevention & control MH - COVID-19 Vaccines/adverse effects MH - Humans MH - *Thrombocytopenia/chemically induced/diagnosis MH - *Thrombosis/chemically induced MH - Vaccination MH - *Vaccines/adverse effects PMC - PMC8761539 OTO - NOTNLM OT - Chilblain OT - Pernio OT - Raynaud disease OT - Thrombosis OT - Vaccine-induced immune thrombotic thrombocytopenia OT - thrombocytopenia EDAT- 2022/04/09 06:00 MHDA- 2022/04/12 06:00 PMCR- 2022/01/17 CRDT- 2022/04/08 05:29 PHST- 2022/01/05 00:00 [received] PHST- 2022/04/08 05:29 [entrez] PHST- 2022/04/09 06:00 [pubmed] PHST- 2022/04/12 06:00 [medline] PHST- 2022/01/17 00:00 [pmc-release] AID - S2542-4513(22)00072-4 [pii] AID - 10.1016/j.jdmv.2022.01.072 [doi] PST - ppublish SO - J Med Vasc. 2022 Feb;47(1):36-38. doi: 10.1016/j.jdmv.2022.01.072. Epub 2022 Jan 17. PMID- 32283744 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231111 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 10 IP - 4 DP - 2020 Apr 9 TI - Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them? LID - 10.3390/diagnostics10040208 [doi] LID - 208 AB - The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD. FAU - Sambataro, Domenico AU - Sambataro D AD - Artroreuma S.R.L., Outpatient clinic of Rheumatology associated with the National Health System Corso S. Vito 53, 95030 Catania, Italy. AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Cannizzaro Hospital, University of Catania, via Messina 829, 95100 Catania, Italy. FAU - Sambataro, Gianluca AU - Sambataro G AUID- ORCID: 0000-0001-9933-1202 AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, via S. Sofia 68, pavillon 3 floor 1, 95123 Catania, Italy. FAU - Pignataro, Francesca AU - Pignataro F AD - Scleroderma clinic, Department of Rheumatology, ASST G. Pini, 20122 Milan, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AUID- ORCID: 0000-0001-5042-1282 AD - Division of Rheumatology, Hospital IRCCS Policlinico S. Matteo Foundation of Pavia, 27100 Pavia, Italy. FAU - Codullo, Veronica AU - Codullo V AUID- ORCID: 0000-0003-2557-8514 AD - Division of Rheumatology, Hospital IRCCS Policlinico S. Matteo Foundation of Pavia, 27100 Pavia, Italy. FAU - Fagone, Evelina AU - Fagone E AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, via S. Sofia 68, pavillon 3 floor 1, 95123 Catania, Italy. FAU - Martorana, Emanuele AU - Martorana E AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, via S. Sofia 68, pavillon 3 floor 1, 95123 Catania, Italy. FAU - Ferro, Francesco AU - Ferro F AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy. FAU - Orlandi, Martina AU - Orlandi M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, 50139 Florence, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AD - Scleroderma clinic, Department of Rheumatology, ASST G. Pini, 20122 Milan, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Division of Rheumatology, Hospital IRCCS Policlinico S. Matteo Foundation of Pavia, 27100 Pavia, Italy. FAU - Malatino, Lorenzo AU - Malatino L AUID- ORCID: 0000-0003-2944-2729 AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Cannizzaro Hospital, University of Catania, via Messina 829, 95100 Catania, Italy. FAU - Colaci, Michele AU - Colaci M AUID- ORCID: 0000-0002-3606-3404 AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Cannizzaro Hospital, University of Catania, via Messina 829, 95100 Catania, Italy. FAU - Vancheri, Carlo AU - Vancheri C AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, via S. Sofia 68, pavillon 3 floor 1, 95123 Catania, Italy. LA - eng PT - Journal Article PT - Review DEP - 20200409 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC7235942 OTO - NOTNLM OT - Raynaud’s phenomenon OT - Sjögren’s syndrome OT - antisynthetase syndrome OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - interstitial pneumonia with autoimmune features OT - multidisciplinary team OT - myositis OT - nailfold videocapillaroscopy OT - systemic sclerosis COIS- C.V. is part of the F. Hoffmann-La Roche Ltd. Scientific board. He has received consulting fees and/or speaker fees from AstraZeneca, Boehringer Ingelheim, Chiesi, F. Hoffmann-La Roche Ltd., and Menarini. The authors G.S., D.S., E.F., G.Z., V.C., L.C., M.C., M.O., F.P., N.D.P., L.M. declare that they have no conflict of interest. EDAT- 2020/04/15 06:00 MHDA- 2020/04/15 06:01 PMCR- 2020/04/09 CRDT- 2020/04/15 06:00 PHST- 2020/03/09 00:00 [received] PHST- 2020/04/04 00:00 [revised] PHST- 2020/04/07 00:00 [accepted] PHST- 2020/04/15 06:00 [entrez] PHST- 2020/04/15 06:00 [pubmed] PHST- 2020/04/15 06:01 [medline] PHST- 2020/04/09 00:00 [pmc-release] AID - diagnostics10040208 [pii] AID - diagnostics-10-00208 [pii] AID - 10.3390/diagnostics10040208 [doi] PST - epublish SO - Diagnostics (Basel). 2020 Apr 9;10(4):208. doi: 10.3390/diagnostics10040208. PMID- 38098902 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231216 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 11 DP - 2023 Nov TI - "Knee-Ding" a Diagnosis: A Case of Nail Patella Syndrome. PG - e48805 LID - 10.7759/cureus.48805 [doi] LID - e48805 AB - Nail Patella Syndrome (NPS) is a rare genetic disorder with pathognomonic signs including dystrophic fingernails, iliac horns, and limb abnormalities, which commonly include hypoplastic development of the patellae, causing patients to experience patellar instability. This resulting patellar instability increases susceptibility to recurrent subluxations or dislocations in NPS patients. Since these anatomical abnormalities are present at birth or in childhood, early recognition may prevent the need for surgical intervention if appropriate preventive measures are taken. This case report describes a 54-year-old woman with a history of NPS, diagnosed later in adulthood, with a prior patellectomy at age 18 secondary to an unspecified left knee injury that occurred at age 4. A combination of radiographic and clinical findings are presented, which support the diagnosis of NPS, including dystrophic nails, left knee x-ray consistent with prior patellectomy, and right knee x-ray showing inferolateral subluxation of a hypoplastic patella. Additional signs associated with NPS are also discussed, including mood disorders, Raynaud's, and a high hairline which may assist in early diagnosis. This case report emphasizes earlier identification of NPS by clinicians through recognition of signs and symptoms while also considering proactive measures to lessen recurrent subluxations or dislocations to preserve patellar integrity and reduce the need for surgical intervention. CI - Copyright © 2023, Dzhugarian et al. FAU - Dzhugarian, Sosi AU - Dzhugarian S AD - Graduate Medical Education, Internal Medicine, Community Memorial Hospital, Ventura, USA. FAU - Hamamah, Sevag AU - Hamamah S AD - Graduate Medical Education, Internal Medicine, Community Memorial Hospital, Ventura, USA. FAU - Frugoli, Amanda AU - Frugoli A AD - Graduate Medical Education, Internal Medicine, Community Memorial Hospital, Ventura, USA. FAU - Shepard, Angelica AU - Shepard A AD - Graduate Medical Education, Internal Medicine, Community Memorial Hospital, Ventura, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20231114 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10721234 OTO - NOTNLM OT - dysplastic patella OT - fong disease OT - hereditary osteo-onychodysplasia OT - hood syndrome OT - nail patella syndrome COIS- The authors have declared that no competing interests exist. EDAT- 2023/12/15 06:42 MHDA- 2023/12/15 06:43 PMCR- 2023/11/14 CRDT- 2023/12/15 03:56 PHST- 2023/11/14 00:00 [accepted] PHST- 2023/12/15 06:43 [medline] PHST- 2023/12/15 06:42 [pubmed] PHST- 2023/12/15 03:56 [entrez] PHST- 2023/11/14 00:00 [pmc-release] AID - 10.7759/cureus.48805 [doi] PST - epublish SO - Cureus. 2023 Nov 14;15(11):e48805. doi: 10.7759/cureus.48805. eCollection 2023 Nov. PMID- 33795279 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 14 IP - 4 DP - 2021 Apr 1 TI - ANCA-associated vasculitis can present with episodic attacks of joint pain consistent with palindromic rheumatism. LID - 10.1136/bcr-2020-240913 [doi] LID - e240913 AB - A 64-year-old man with a 2-year history of palindromic rheumatoid arthritis, presented with recurrent flares of arthritis, weight loss, new onset Raynaud's phenomenon and one previous episode of small-volume haemoptysis. Investigations, including renal biopsy, revealed antineutrophil cytoplasmic antibodies-mediated vasculitis. This case highlights the need to consider vasculitis in patients in whom there is an atypical history of arthritis. CI - © BMJ Publishing Group Limited 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Rutter-Locher, Zoe AU - Rutter-Locher Z AUID- ORCID: 0000-0001-8251-4037 AD - Rheumatology, Guy's and St Thomas' Hospitals NHS Trust, London, UK zrutter-locher@nhs.net. FAU - Kirkham, Bruce AU - Kirkham B AD - Rheumatology, Guy's and St Thomas' Hospitals NHS Trust, London, UK. FAU - D'Cruz, David P AU - D'Cruz DP AD - Lupus Research Unit, Guy's and St Thomas' Hospitals NHS Trust, London, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20210401 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - Palindromic rheumatism SB - IM MH - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications/diagnosis/drug therapy MH - Antibodies, Antineutrophil Cytoplasmic MH - Arthralgia/etiology MH - *Arthritis, Rheumatoid/complications MH - Humans MH - Male MH - Middle Aged PMC - PMC8021579 OTO - NOTNLM OT - connective tissue disease OT - rheumatoid arthritis OT - vasculitis COIS- Competing interests: None declared. EDAT- 2021/04/03 06:00 MHDA- 2021/05/15 06:00 PMCR- 2021/04/01 CRDT- 2021/04/02 05:54 PHST- 2021/04/02 05:54 [entrez] PHST- 2021/04/03 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2021/04/01 00:00 [pmc-release] AID - 14/4/e240913 [pii] AID - bcr-2020-240913 [pii] AID - 10.1136/bcr-2020-240913 [doi] PST - epublish SO - BMJ Case Rep. 2021 Apr 1;14(4):e240913. doi: 10.1136/bcr-2020-240913. PMID- 30472907 OWN - NLM STAT- MEDLINE DCOM- 20200210 LR - 20200210 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 29 IP - 6 DP - 2019 Nov TI - Less severe disease in patients with early systemic sclerosis? PG - 977-983 LID - 10.1080/14397595.2018.1551265 [doi] AB - Objectives: To evaluate the disease severity and activity in patients with a diagnosis of systemic sclerosis (SSc) after the 2013 American College of Rheumatology and the European League Against Rheumatism (ACR/EULAR) classification criteria development compared to patients diagnosed before 2013.Methods: One hundred and fifty-four subjects were included and assigned to the following groups: 120 SSc patients meeting the 1980 ACR criteria and with a diagnosis before 2013 (historical group), and 34 patients diagnosed after 2013, fulfilling the new ACR/EULAR criteria (early SSc group). Disease activity was assessed by the 2001 European Scleroderma Study Group Activity Index (EScSG-AI) and by the revised European Scleroderma Trials and Research group (EUSTAR) activity index. Disease severity was assessed using the Medsger Disease Severity Scale (DSS) and the summed DSS score.Results: The time between the first non-Raynaud's symptom and the diagnosis was shorter in early SSc than in the historical group (p = .001). The EScSG-AI and the EUSTAR activity index were similar between groups. The summed DSS score and the general, skin and gastrointestinal tract DSS scores were significantly lower in early SSc than in the historical group.Conclusion: SSc patients with a diagnosis after the new ACR/EULAR criteria development were diagnosed earlier and had a less severe disease than historical patients. FAU - Horimoto, Alex Magno Coelho AU - Horimoto AMC AD - Rheumatology Division, Universidade Federal do Mato Grosso do Sul, Mato Grosso do Sul, Brazil. FAU - Camargo, Cintia Zumstein AU - Camargo CZ AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. LA - eng PT - Journal Article DEP - 20190103 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/classification/epidemiology/*pathology MH - United States OTO - NOTNLM OT - Disease activity OT - disease severity OT - scleroderma OT - systemic sclerosis EDAT- 2018/11/27 06:00 MHDA- 2020/02/11 06:00 CRDT- 2018/11/27 06:00 PHST- 2018/11/27 06:00 [pubmed] PHST- 2020/02/11 06:00 [medline] PHST- 2018/11/27 06:00 [entrez] AID - 10.1080/14397595.2018.1551265 [doi] PST - ppublish SO - Mod Rheumatol. 2019 Nov;29(6):977-983. doi: 10.1080/14397595.2018.1551265. Epub 2019 Jan 3. PMID- 37576433 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230815 IS - 2633-0040 (Electronic) IS - 2633-0040 (Linking) VI - 4 DP - 2023 Jan-Dec TI - A multidisciplinary approach to treating a unique case of recurrent metastatic thymic carcinoma: case report. PG - 26330040231190661 LID - 10.1177/26330040231190661 [doi] LID - 26330040231190661 AB - Thymic carcinoma (TC) is a rare and aggressive malignancy of the thymus associated with less than 25% 5 years survivability. Our case report showcases the successful treatment of advanced metastatic TC using a multidisciplinary approach and the utility of checkpoint inhibitors in treatment of recurrent TC. A 50-year-old man presented with Raynaud's phenomenon and was found to have a stage IVb TC (T3N2M0). Eight months after management with neoadjuvant chemotherapy, surgical resection and adjuvant chemoradiotherapy, patient was diagnosed with metastasis of TC to the liver and a concurrent stage III (T2N1M0) primary sigmoid colon adenocarcinoma. Following complete resection of the colon adenocarcinoma, the patient started palliative-intent treatment for TC with pembrolizumab given PD-L1 tumor proportionate score of 100%. This resulted in a sustained complete response for 38 months. Our patient did have immune-related adverse events involving multiple organs but was able to continue pembrolizumab for a standard treatment duration of 2 years with multidisciplinary care. When recurrent disease was noted in a portocaval lymph node, pembrolizumab was reinitiated and a second complete response was achieved. The patient has maintained that complete response while maintaining an acceptable quality of life, showing that treatment with pembrolizumab is effective in patients after discontinuation with prior immunotherapy. CI - © The Author(s), 2023. FAU - Wang, Carol AU - Wang C AUID- ORCID: 0000-0002-3386-6260 AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 500 Hofstra Blvd., Hempstead, NY 11549, USA. FAU - Erick Elkowitz, David AU - Erick Elkowitz D AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA. FAU - Esposito, Michael John AU - Esposito MJ AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA. FAU - Shah, Rakesh Dinesh AU - Shah RD AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA. FAU - Tannous, Henry AU - Tannous H AD - Stony Brook Medicine, Stony Brook, NY, USA. FAU - Barilla-Labarca, Maria-Louise AU - Barilla-Labarca ML AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA. FAU - Seetharamu, Nagashree AU - Seetharamu N AD - Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230811 PL - England TA - Ther Adv Rare Dis JT - Therapeutic advances in rare disease JID - 9918557474706676 PMC - PMC10422886 OAB - Fighting Thymic Carcinoma: A Story of Immunotherapy and Multidisciplinary Care Triumph The thymus is a gland located in the chest that plays a major role in the immune system, particularly before adulthood. Thymic carcinoma (TC) is a type of cancer affecting the thymus that is often challenging to treat given its inadequate response to chemotherapy and tendency to spread to other organs. A 50-year-old man was found to have advanced stage thymic carcinoma, which is associated with a less than 25% 5-year survival rate. Eight months after completing a rigorous treatment protocol of chemotherapy, surgery and radiation therapy, his original thymic cancer was found to have metastasized to the liver. Simultaneously, he was diagnosed with stage III sigmoid colon cancer. He underwent curative surgery for colon cancer and was started on pembrolizumab for thymic cancer. Pembrolizumab is an immunotherapy drug that boosts the body’s own immune system to fight against the cancer. Inadvertently, it can turn immune cells against healthy tissues, which results in symptoms called immune-related adverse events (irAEs). Indeed, he experienced various irAEs involving multiple organs. These events were effectively managed by involving multiple specialists and initiating medications to calm the immune system and allow him to continue immunotherapy. He had a complete response to treatment and was able to complete the standard treatment course of two years. He retained a complete response for over three years before his tumor recurred. He was restarted on pembrolizumab and achieved a complete response again. This case highlights a unique presentation of metastatic TC and the utility of a multidisciplinary approach for treatment to maintain a high quality of life five years after diagnosis. OABL- eng OTO - NOTNLM OT - Raynaud’s phenomenon OT - anti-PD1 inhibitors OT - case report OT - extrathymic malignancy OT - immune checkpoint inhibitors OT - pembrolizumab OT - thymic carcinoma COIS- The authors declare that there is no conflict of interest. EDAT- 2023/08/14 06:42 MHDA- 2023/08/14 06:43 PMCR- 2023/08/11 CRDT- 2023/08/14 04:42 PHST- 2023/02/05 00:00 [received] PHST- 2023/07/06 00:00 [accepted] PHST- 2023/08/14 06:43 [medline] PHST- 2023/08/14 06:42 [pubmed] PHST- 2023/08/14 04:42 [entrez] PHST- 2023/08/11 00:00 [pmc-release] AID - 10.1177_26330040231190661 [pii] AID - 10.1177/26330040231190661 [doi] PST - epublish SO - Ther Adv Rare Dis. 2023 Aug 11;4:26330040231190661. doi: 10.1177/26330040231190661. eCollection 2023 Jan-Dec. PMID- 40662043 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250717 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 6 DP - 2025 Jun TI - Gastrointestinal Hemorrhage in CREST Syndrome: A Case Report. PG - e85978 LID - 10.7759/cureus.85978 [doi] LID - e85978 AB - Systemic scleroderma is an autoimmune disorder caused by microvascular dysfunction, excessive collagen deposition, and progressive fibrosis affecting the skin and other organs. CREST syndrome, also known as limited scleroderma, is an acronym for calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Among patients with systemic sclerosis (SSc), anticentromere antibodies are typically associated with the CREST syndrome variant. These antibodies are considered disease-specific and are known to correlate with the extent of disease progression. The pathogenesis of SSc is complex, with unusual presentations increasingly seen among patients. Gastrointestinal (GI) hemorrhage can be observed in CREST syndrome as an uncommon but serious complication. Endoscopic interventions are warranted in such patients to evaluate and manage bleeding. This report aims to raise awareness among physicians that CREST syndrome can present with GI hemorrhage, with skin signs such as calcinosis and sclerodactyly. In our case, H2 blockers and proton pump inhibitors were administered as part of the management plan to reduce acid-related mucosal injury and mitigate GI bleeding. Due to chronic mucosal hemorrhage, anemia was noted and corrected with parenteral iron therapy. The prognosis of CREST syndrome is relatively good when compared to the diffuse variant. Further research is needed to understand the pathophysiology of CREST syndrome, reliable biomarkers for early diagnosis, and targeted preventive treatment for this condition. Sensitizing clinicians regarding the changing disease pattern will help in prompt diagnosis. CI - Copyright © 2025, Ravikumar et al. FAU - Ravikumar, Jayashree AU - Ravikumar J AD - Internal Medicine, Sri Varadhar Consultation Clinic, Chennai, IND. FAU - Sai Prudhvi, Thalvayapati AU - Sai Prudhvi T AD - Internal Medicine, Kilpauk Medical College, Chennai, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20250614 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12257045 OTO - NOTNLM OT - calcinosis OT - crest syndrome OT - gastrointestinal bleed OT - gastrointestinal hemorrhage OT - limited scleroderma OT - scleroderma COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/07/15 06:28 MHDA- 2025/07/15 06:29 PMCR- 2025/06/14 CRDT- 2025/07/15 05:10 PHST- 2025/06/14 00:00 [accepted] PHST- 2025/07/15 06:29 [medline] PHST- 2025/07/15 06:28 [pubmed] PHST- 2025/07/15 05:10 [entrez] PHST- 2025/06/14 00:00 [pmc-release] AID - 10.7759/cureus.85978 [doi] PST - epublish SO - Cureus. 2025 Jun 14;17(6):e85978. doi: 10.7759/cureus.85978. eCollection 2025 Jun. PMID- 31766529 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 8 IP - 12 DP - 2019 Nov 21 TI - Interferons (IFN-A/-B/-G) Genetic Variants in Patients with Mixed Connective Tissue Disease (MCTD). LID - 10.3390/jcm8122046 [doi] LID - 2046 AB - Mixed connective tissue disease (MCTD) is a rare complex autoimmune disease in which autoantigens are recognized by endosomal TLRs. Their activation induces a higher secretion of the type I interferons, IFN-γ and the up-regulation of the INF-inducible genes. The present study aimed to investigate whether SNPs that are located in the IFN-A, IFN-B, and IFN-G genes are associated with MCTD. 145 MCTD patients and 281 healthy subjects were examined for IFN-A, IFN-B, and IFN-G genetic variants by TaqMan SNP genotyping assay. ELISA determined IFN-α/-β/-γ serum levels. Among the seven tested SNPs, four polymorphisms: IFN-A rs10757212, IFN-A rs3758236, IFN-G rs2069705, IFN-G rs2069718, as well as INF-G rs1861493A/rs2069705A/rs2069718G haplotype were significantly associated with a predisposition for MCTD. Raynaud's phenomenon, erosive arthritis, swollen hands and fingers, and sclerodactyly were significantly more frequently observed in MCTD patients with IFN-G rs2069718 G allele than in patients with IFN-G rs2069718 A allele. We also found that anti-U1-A autoantibodies most frequently occurred in MCTD patients with rs2069718 GA genotype, while the IFN-G rs2069705 AG and rs2069718 GA genotypes might be a marker of anti-Ro60 presence in MCTD patients. Our results indicate that IFN-G genetic variants may be potential genetic biomarkers for MCTD susceptibility and severity. FAU - Paradowska-Gorycka, Agnieszka AU - Paradowska-Gorycka A AD - Department of Molecular Biology, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Wajda, Anna AU - Wajda A AD - Department of Molecular Biology, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Stypinska, Barbara AU - Stypinska B AD - Department of Molecular Biology, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Walczuk, Ewa AU - Walczuk E AD - Department of Molecular Biology, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Walczyk, Marcela AU - Walczyk M AD - Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Felis-Giemza, Anna AU - Felis-Giemza A AD - Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Poluch, Aleksandra AU - Poluch A AD - Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. FAU - Olesińska, Marzena AU - Olesińska M AD - Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland. LA - eng GR - -/Polpharma Scientific Fundation/ PT - Journal Article DEP - 20191121 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC6947393 OTO - NOTNLM OT - IFN OT - MCTD OT - genetics OT - pathogenesis COIS- The authors declare no conflict of interest. EDAT- 2019/11/27 06:00 MHDA- 2019/11/27 06:01 PMCR- 2019/11/21 CRDT- 2019/11/27 06:00 PHST- 2019/10/22 00:00 [received] PHST- 2019/11/06 00:00 [revised] PHST- 2019/11/20 00:00 [accepted] PHST- 2019/11/27 06:00 [entrez] PHST- 2019/11/27 06:00 [pubmed] PHST- 2019/11/27 06:01 [medline] PHST- 2019/11/21 00:00 [pmc-release] AID - jcm8122046 [pii] AID - jcm-08-02046 [pii] AID - 10.3390/jcm8122046 [doi] PST - epublish SO - J Clin Med. 2019 Nov 21;8(12):2046. doi: 10.3390/jcm8122046. PMID- 23794106 OWN - NLM STAT- MEDLINE DCOM- 20140204 LR - 20211021 IS - 1534-6307 (Electronic) IS - 1523-3774 (Linking) VI - 15 IP - 8 DP - 2013 Aug TI - Clinical heterogeneity and outcomes of antisynthetase syndrome. PG - 349 LID - 10.1007/s11926-013-0349-8 [doi] AB - The autoimmune connective tissue disease antisynthetase syndrome (ASS) is an inflammatory myopathy associated with myositis-specific autoantibodies, e.g. anti-tRNA-synthetase antibodies (ASA). Since 1976 eight different ASA have been rigorously identified, of which anti-hystidyl-tRNA synthetase (anti-Jo1) is the most prevalent. Other phenotype features of ASS include interstitial lung disease (ILD), Raynaud's phenomenon, polyarthritis, fever, and mechanic's hands. The clinical presentation of ASS varies greatly, as does the severity of involvement of different organs-both among patients and/or over the course of the disease. ILD has been associated with poor outcomes, but in general the heterogeneity of ASS prevents identification of robust prognosis indicators. Early identification of patients requiring aggressive immunosuppressive treatment is very challenging, and there are very few prospective trials available to help match treatment management to ASS clinical characteristics. This review will focus on the biological, clinical, functional, and morphological features of ASS associated with patient outcome. Our objective is to use compiled data on these subjects to discuss the usefulness of patient stratification in developing future prospective therapeutic trials. FAU - Hervier, Baptiste AU - Hervier B AD - Internal Medicine Department, French Referral Center for Lupus and Antiphospholipid Syndrome, APHP, Hôpital Pitié Salpêtrière, 47-83 Boulevard de L'Hôpital, 75651, Paris Cedex 13, Paris, France. baptiste.hervier@psl.aphp.fr FAU - Benveniste, Olivier AU - Benveniste O LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Rheumatol Rep JT - Current rheumatology reports JID - 100888970 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/blood MH - Biomarkers/blood MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/diagnosis/immunology MH - Myositis/*diagnosis/drug therapy/immunology MH - Prognosis MH - Treatment Outcome EDAT- 2013/06/26 06:00 MHDA- 2014/02/05 06:00 CRDT- 2013/06/25 06:00 PHST- 2013/06/25 06:00 [entrez] PHST- 2013/06/26 06:00 [pubmed] PHST- 2014/02/05 06:00 [medline] AID - 10.1007/s11926-013-0349-8 [doi] PST - ppublish SO - Curr Rheumatol Rep. 2013 Aug;15(8):349. doi: 10.1007/s11926-013-0349-8. PMID- 18705442 OWN - NLM STAT- MEDLINE DCOM- 20080902 LR - 20080818 IS - 0300-5283 (Print) IS - 0300-5283 (Linking) VI - 62 IP - 2 DP - 2007 Jun TI - Demography, clinical and laboratory features of systemic sclerosis in a Malaysian rheumatology centre. PG - 117-21 AB - A six year retrospective study of the demography, clinical and laboratory features of patients with systemic sclerosis (SSc) was carried out in Selayang Hospital. There were 61 cases seen between January 2000 and December 2005. Of these, 55 (90.2%) were females and 6 (9.8%) were males. Twenty-eight (45.9%) were Malays, 24 (39.3%) were Chinese and 9 (14.8%) were Indians. The mean age of onset was 38.8 years. Thirty-nine (64.0%) had limited cutaneous SSc, 21 (34.4%) had diffuse cutaneous SSc and one had localized morphoea. Raynaud's phenomenon was present in 82.6%, telangiectasia in 45.9%, calcinosis in 11.5%, sclerodactyly in 83.6%, digital pitting scars in 42.6%, digital infarcts/ulcers/gangrene in 23.0%, arthralgia/arthritis in 49.2% and gastroesophageal reflux disease (GERD) in 47.5%. Forty-three (70.5%) patients had interstitial lung disease. Seven patients had associated myositis, 7 systemic lupus erythematosus and 2 rheumatoid arthritis. Three had two other connective tissue diseases. Antinuclear antibodies were positive in 83.6% and anti-Scl 70 antibodies in 34.4%. This study demonstrates that limited cutaneous SSc is more common and there is a high incidence of interstitial lung disease in our population. FAU - Pagalavan, L AU - Pagalavan L AD - Rheumatology Unit, Department of Medicine, Hospital Selayang, 68100 Batu Caves, Selangor. FAU - Ong, S G AU - Ong SG LA - eng PT - Journal Article PL - Malaysia TA - Med J Malaysia JT - The Medical journal of Malaysia JID - 0361547 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Aged MH - Autoantibodies/blood MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications/diagnosis/immunology EDAT- 2008/08/19 09:00 MHDA- 2008/09/03 09:00 CRDT- 2008/08/19 09:00 PHST- 2008/08/19 09:00 [pubmed] PHST- 2008/09/03 09:00 [medline] PHST- 2008/08/19 09:00 [entrez] PST - ppublish SO - Med J Malaysia. 2007 Jun;62(2):117-21. PMID- 28913687 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1092-8464 (Print) IS - 1092-8464 (Linking) VI - 19 IP - 10 DP - 2017 Sep 14 TI - Non-Atherosclerotic Vascular Disease in Women. PG - 78 LID - 10.1007/s11936-017-0579-6 [doi] AB - Takayasu arteritis, fibromuscular dysplasia (FMD), spontaneous arterial dissection, Raynaud's phenomenon, and chilblains are vascular conditions that are associated with an increased predisposition in women and are often underdiagnosed. Takayasu arteritis has an incidence rate of 2.6 cases per million individuals per year in the USA and predominantly affects women of childbearing age. HLA-B5 genetic locus is linked with Takayasu arteritis susceptibility. Methods to determine active disease are limiting; currently utilized clinical and imaging findings and laboratory tests are of limited value for this purpose. Pregnancy poses risks for maternal and fetal complications, and these patients need additional monitoring and care before and after conception. Controlling hypertension and immunosuppression using steroids, biological and non-biological immunosuppressants, are key components of managing patients with this arteritis. FMD commonly affects middle-aged, white females. Its true prevalence is unknown. Renal and cerebrovascular beds are the most frequently involved vascular beds. Its clinical presentation varies from no symptoms to catastrophic events. Controlling vascular risk factors, periodic surveillance, and revascularization when indicated are important factors in FMD management. Spontaneous arterial dissections are less common, but are an important cause of morbidity and mortality in specific populations. Cervicocephalic dissection causes 10-20% of the strokes in young adults, and coronary artery dissection is the culprit in almost one fourth of young women presenting with acute myocardial infarction. Early diagnosis is key to improving prognosis in these patients, as the majority of patients have spontaneous resolution of the dissection with conservative management alone. Increased clinician awareness of the presentation features and angiographic findings are imperative for early diagnosis. Raynaud's phenomenon and chilblains are cold- or stress-induced cutaneous lesions, commonly involving distal extremities. Secondary causes such as connective tissue diseases and malignancies must be thoroughly excluded during evaluation of these conditions. Cold avoidance, systemic and local warming, and oral vasodilator therapy are the mainstays of therapy. FAU - Joseph, Lee AU - Joseph L AD - Division of Cardiovascular Diseases, Department of Internal Medicine, University of Iowa, 169 Newton Road, Iowa City, IA, 52246, USA. FAU - Kim, Esther S H AU - Kim ESH AD - Cardiovascular Division, Vanderbilt University Medical Center, 1215 21st Avenue South MCE South Tower, Nashville, TN, 37232, USA. esther.kim@vanderbilt.edu. LA - eng PT - Journal Article PT - Review DEP - 20170914 PL - United States TA - Curr Treat Options Cardiovasc Med JT - Current treatment options in cardiovascular medicine JID - 9815942 OTO - NOTNLM OT - Atherosclerosis OT - Cardiovascular disease OT - Vascular disease OT - Women’s health EDAT- 2017/09/16 06:00 MHDA- 2017/09/16 06:01 CRDT- 2017/09/16 06:00 PHST- 2017/09/16 06:00 [entrez] PHST- 2017/09/16 06:00 [pubmed] PHST- 2017/09/16 06:01 [medline] AID - 10.1007/s11936-017-0579-6 [pii] AID - 10.1007/s11936-017-0579-6 [doi] PST - epublish SO - Curr Treat Options Cardiovasc Med. 2017 Sep 14;19(10):78. doi: 10.1007/s11936-017-0579-6. PMID- 38890107 OWN - NLM STAT- MEDLINE DCOM- 20240618 LR - 20240924 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 17 IP - 6 DP - 2024 Jun 18 TI - Impressive resolution of refractory hypertrophic discoid lupus erythematosus with anifrolumab. LID - e258487 [pii] LID - 10.1136/bcr-2023-258487 [doi] AB - Hypertrophic discoid lupus erythematosus is a rare variant of chronic cutaneous lupus erythematosus and is often challenging to treat. A male in his early 60s presented with diffuse erythematous, crusty, pruritic plaques on his upper and lower extremities, face, upper back, dorsal aspect of the hands and chest. He also described prolonged morning stiffness, swelling of his fingers and wrists, oral sores and Raynaud's phenomenon. He was positive for antinuclear antibody and anti-SSA antibody and had low C3 and C4 proteins. The skin biopsy was consistent with hypertrophic discoid lupus erythematosus. He was diagnosed with systemic lupus erythematosus. Skin lesions were refractory to treatment with topical corticosteroids, topical acitretin, hydroxychloroquine, azathioprine or mycophenolate. Anifrolumab infusions were initiated with a near-complete resolution of cutaneous symptoms within 3 months. CI - © BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Ocon, Anthony AU - Ocon A AUID- ORCID: 0000-0003-2903-5429 AD - Rheumatology, Rochester Regional Health, Rochester, New York, USA anthony.ocon@rochesterregional.org. AD - Rheumatology, URMC, Rochester, New York, USA. FAU - Avalos Sugastti, Eduardo AU - Avalos Sugastti E AD - Internal Medicine, Rochester Regional Health, Rochester, New York, USA. FAU - Duffy, Nananamibia AU - Duffy N AD - Dermatology, Rochester Regional Health, Rochester, New York, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240618 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Antibodies, Monoclonal) SB - IM MH - Humans MH - Male MH - *Lupus Erythematosus, Discoid/drug therapy MH - Middle Aged MH - Treatment Outcome MH - Antibodies, Monoclonal, Humanized/therapeutic use MH - Antibodies, Monoclonal/therapeutic use/administration & dosage OTO - NOTNLM OT - connective tissue disease OT - dermatology OT - rheumatology OT - systemic lupus erythematosus COIS- Competing interests: None declared. EDAT- 2024/06/19 00:43 MHDA- 2024/06/19 00:44 CRDT- 2024/06/18 22:03 PHST- 2024/06/19 00:44 [medline] PHST- 2024/06/19 00:43 [pubmed] PHST- 2024/06/18 22:03 [entrez] AID - 17/6/e258487 [pii] AID - 10.1136/bcr-2023-258487 [doi] PST - epublish SO - BMJ Case Rep. 2024 Jun 18;17(6):e258487. doi: 10.1136/bcr-2023-258487. PMID- 28502960 OWN - NLM STAT- MEDLINE DCOM- 20170928 LR - 20170928 IS - 1347-3409 (Electronic) IS - 1345-4676 (Linking) VI - 84 IP - 2 DP - 2017 TI - Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting. PG - 56-63 LID - 10.1272/jnms.84.56 [doi] AB - The presence of circulating anti-nuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Currently, a variety of SSc-specific ANAs, including anticentromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, have been well characterized, and their commercial kits are available worldwide. Since these autoantibodies are specifically detected in SSc patients and are associated with unique sets of disease manifestations, they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis. In addition, SSc-specific ANAs are also useful in predicting future development of SSc in patients with Raynaud's phenomenon without any scleroderma skin changes, because their production often precedes onset of SSc symptoms. Application of circulating SSc-specific ANA measurement to clinical practice has greatly improved patient care, but utility of the autoantibody testing could be maximized by combining other clinical information, such as degree and extent of skin thickness and disease duration. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School. LA - eng PT - Journal Article PT - Review PL - Japan TA - J Nippon Med Sch JT - Journal of Nippon Medical School = Nippon Ika Daigaku zasshi JID - 100935589 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Reagent Kits, Diagnostic) RN - 0 (anticentromere antibody) RN - EC 2.7.7.6 (RNA Polymerase III) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Antibodies, Antinuclear/*blood/classification MH - Biomarkers/blood MH - DNA Topoisomerases, Type I/immunology MH - Humans MH - RNA Polymerase III/immunology MH - Reagent Kits, Diagnostic MH - Scleroderma, Systemic/classification/*diagnosis/pathology OTO - NOTNLM OT - anti-nuclear antibody OT - biomarker OT - diagnosis OT - scleroderma OT - systemic sclerosis EDAT- 2017/05/16 06:00 MHDA- 2017/09/29 06:00 CRDT- 2017/05/16 06:00 PHST- 2017/05/16 06:00 [entrez] PHST- 2017/05/16 06:00 [pubmed] PHST- 2017/09/29 06:00 [medline] AID - 10.1272/jnms.84.56 [doi] PST - ppublish SO - J Nippon Med Sch. 2017;84(2):56-63. doi: 10.1272/jnms.84.56. PMID- 39487058 OWN - NLM STAT- MEDLINE DCOM- 20241111 LR - 20241111 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 20 IP - 9 DP - 2024 Nov TI - Evaluation of the efficacy and safety of gravitational therapy in a cohort of patients with systemic sclerosis. PG - 463-469 LID - S2173-5743(24)00137-0 [pii] LID - 10.1016/j.reumae.2024.10.004 [doi] AB - BACKGROUND: The exposure to artificial gravity (AG) through human centrifugation is the basis of the treatment called gravity therapy (GT), in which the mechanical stimulation over the vessel wall, induces the synthesis and release of prostacyclin. It has been used for more than four decades in Uruguay in the treatment of different vascular-based pathologies. In patients with systemic sclerosis (SSc) it has shown good benefits and excellent safety profile over the years. However, there is a lack of knowledge in the scientific community about GT and its results. OBJECTIVE: To evaluate the effectiveness of GT in cutaneous and vascular involvement, in the quality of life and functional capacity and its safety profile in patients with SSc. METHODOLOGY: It is a descriptive and retrospective study of patients with SSc assisted in an autoimmunity center in Montevideo, treated with GT in the last 10 years. RESULTS: Fifty patients were included, 48 women (96%) and 2 men (4%) with a mean age of 62 ± 12 years. The mean time of evolution of SSc at the time of inclusion in the study at the beginning of GT was 6.8 ± 3.2 years and 2.8 ± 3.2 years respectively. After GT, a significant improvement in the modified Rodnan skin score (mRSS) was observed (pre-GT 19.2 ± 8.7 vs. post-GT 5.4 ± 5.0, p < 0.05), which was not related to the time of disease progression at the beginning of GT nor to the skin extension or immunological profile. The degree of improvement post-GT was related to a higher initial mRSS (R = 0.84, p < 0.05). Also, a significant improvement was observed in the number of patients with puffy fingers (pre-GT 50% vs. post-GT 20% patients, p < 0.05), but not in telangiectasias, pitting scars or sclerodactyly. The severity of Raynaud's phenomenon significantly decreased (pre-GT: grade 3-4, 43/48 (89.6%) patients vs. post-GT: grade ≤2, 42/47 (89.4%) patients, p < 0.05) as well as the vascular pain measured with VAS (0-10 scale) (pre-GT: 7.6 ± 2.2 vs. post-TG: 1.4 ± 1.2, p < 0.05). The healing of digital ulcers was also recorded. Regarding the results reported by patients, 97% reported improvement in the quality of life and 89.5% improvement in the ability to carry out activities of daily living. No significant adverse effects were recorded. CONCLUSIONS: GT improved cutaneous and vascular involvement, the quality of life and the functional capacity in patients with SSc with an excellent safety profile. Randomized, controlled clinical trials are needed to corroborate these observational results. CI - Copyright © 2024 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Servioli, Luisa Fernanda AU - Servioli LF AD - Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Militar, Montevideo, Uruguay. Electronic address: lservioli01@gmail.com. FAU - Isasi, Eugenia AU - Isasi E AD - Unidad Académica de Histología y Embriología, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay; Centro de Terapia Gravitacional, Montevideo, Uruguay. FAU - Pérez, Alejandra AU - Pérez A AD - Centro de Terapia Gravitacional, Montevideo, Uruguay. FAU - Pouquette, Silvia AU - Pouquette S AD - Centro de Terapia Gravitacional, Montevideo, Uruguay. FAU - Isasi, María Eloísa AU - Isasi ME AD - Centro de Terapia Gravitacional, Montevideo, Uruguay. LA - eng PT - Journal Article DEP - 20241031 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications MH - Female MH - Male MH - Middle Aged MH - Retrospective Studies MH - Aged MH - *Quality of Life MH - Treatment Outcome MH - Gravitation OTO - NOTNLM OT - Artificial gravity OT - Esclerosis sistémica OT - Fenómeno de Raynaud OT - Gravedad artificial OT - Gravitational therapy OT - Hipergravedad OT - Hypergravity OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Terapia gravitacional COIS- Declaration of competing interest L.F.S. is a physician and independent researcher. E.I. and M.E.I. are partners of ISASI CENTER.SRL (Gravitational Therapy Centre). S.P and A.P are physicians of the GTC. The authors declare that there is no conflict of interest. EDAT- 2024/11/02 23:13 MHDA- 2024/11/13 13:55 CRDT- 2024/11/01 22:53 PHST- 2024/02/19 00:00 [received] PHST- 2024/07/18 00:00 [accepted] PHST- 2024/11/13 13:55 [medline] PHST- 2024/11/02 23:13 [pubmed] PHST- 2024/11/01 22:53 [entrez] AID - S2173-5743(24)00137-0 [pii] AID - 10.1016/j.reumae.2024.10.004 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2024 Nov;20(9):463-469. doi: 10.1016/j.reumae.2024.10.004. Epub 2024 Oct 31. PMID- 19565953 OWN - NLM STAT- MEDLINE DCOM- 20090818 LR - 20221207 IS - 0043-3144 (Print) IS - 0043-3144 (Linking) VI - 57 IP - 2 DP - 2008 Mar TI - Systemic sclerosis in an Afro-Caribbean population: a review of demographic and clinical features. PG - 118-21 AB - OBJECTIVE: To assess the clinical and selected demographic features of patients with systemic sclerosis (SS) seen over a 10-year period at the Rheumatology service of the Queen Elizabeth Hospital, Barbados. To compare these data with what is known to obtain in other ethnic populations. DESIGN AND METHODS: A chart review involving all patients who were found to have SS based on the American College of Rheumatology clinical criteria was conducted between 1996 and 2006. RESULTS: Twenty-seven patients with SS were identified in this predominantly Afro-Caribbean population. The prevalent and incident cases numbered 10 and 17 respectively. Twenty-six of these patients were female and the mean age at diagnosis was 37.3 years. Diffuse cutaneous involvement was seen in 63% of cases and limited cutaneous involvement in 37%. The most common clinical features in descending order of frequency were Raynaud's phenomenon, gastroesophageal reflux, pigmentary skin changes, digital pitting/ulceration, telangiectasia and pulmonary disease. CONCLUSION: In a predominantly Afro-Caribbean population, SS was uncommonly seen, had a marked female preponderance and an earlier age of onset than that seen in Caucasian populations. As expected, diffuse disease was the more common subtype and digital pitting, pigmentary skin changes, and pulmonary disease were amongst the most frequent clinical features. Telangiectasia were found more frequently than the literature suggests is typical for patients of African descent. FAU - Flower, C AU - Flower C AD - School of Clinical Medicine and Research, The University of the West Indies, Queen Elizabeth Hospital, Bridgetown, Barbados. cflower@caribsurf.com FAU - Nwankwo, C AU - Nwankwo C LA - eng PT - Journal Article PL - Jamaica TA - West Indian Med J JT - The West Indian medical journal JID - 0417410 SB - IM MH - Adolescent MH - Adult MH - Barbados/epidemiology MH - *Black People MH - Female MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Prevalence MH - Scleroderma, Systemic/*ethnology/physiopathology MH - Young Adult EDAT- 2008/03/01 00:00 MHDA- 2009/08/19 09:00 CRDT- 2009/07/02 09:00 PHST- 2009/07/02 09:00 [entrez] PHST- 2008/03/01 00:00 [pubmed] PHST- 2009/08/19 09:00 [medline] AID - 716 [pii] PST - ppublish SO - West Indian Med J. 2008 Mar;57(2):118-21. PMID- 41756703 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260227 LR - 20260227 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 14 IP - 3 DP - 2026 Mar TI - Antisynthetase Syndrome Mimicking Pulmonary Infection: A Diagnostic Lesson From County-Managed Hospital. PG - e72085 LID - 10.1002/ccr3.72085 [doi] LID - e72085 AB - Antisynthetase syndrome (ASS) is a rare chronic autoimmune disease classified as a distinct subtype of idiopathic inflammatory myopathy, characterized by the presence of anti-aminoacyl-transfer RNA synthetase (anti-ARS) antibodies. Typical clinical features include myositis, interstitial lung disease (ILD), arthritis, fever, mechanic's hands, and Raynaud's phenomenon, with a predominance in middle-aged to elderly females. We report an atypical case of a 75-year-old male presenting with a 6-month history of anorexia and fatigue, followed by a 3-week progression of chest tightness and dyspnea. Initially misdiagnosed as a pulmonary infection, the patient showed no improvement after two weeks of antimicrobial therapy. Diagnosis was ultimately confirmed through bronchoscopy and detection of anti-Jo-1 antibodies. Subsequent corticosteroid treatment led to complete radiographic resolution and significant clinical improvement. This case underscores the importance of considering ASS in elderly male patients to prevent misdiagnosis and ensure timely intervention. CI - © 2026 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Cheng, Jufen AU - Cheng J AUID- ORCID: 0009-0006-5147-3475 AD - The Second Affiliated Hospital Zhejiang University School of Medicine The Second Affiliated Hospital Zhejiang University School of Medicine Kaihua Branch Kaihua County Zhejiang Province China. LA - eng PT - Journal Article DEP - 20260224 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 PMC - PMC12932121 OTO - NOTNLM OT - antisynthetase syndrome OT - case report OT - old male OT - pulmonary infection COIS- The author declares no conflicts of interest. EDAT- 2026/02/27 06:31 MHDA- 2026/02/27 06:32 PMCR- 2026/02/24 CRDT- 2026/02/27 05:02 PHST- 2025/08/07 00:00 [received] PHST- 2025/11/30 00:00 [revised] PHST- 2025/12/23 00:00 [accepted] PHST- 2026/02/27 06:32 [medline] PHST- 2026/02/27 06:31 [pubmed] PHST- 2026/02/27 05:02 [entrez] PHST- 2026/02/24 00:00 [pmc-release] AID - CCR372085 [pii] AID - 10.1002/ccr3.72085 [doi] PST - epublish SO - Clin Case Rep. 2026 Feb 24;14(3):e72085. doi: 10.1002/ccr3.72085. eCollection 2026 Mar. PMID- 28065485 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20260127 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 14 IP - 4 DP - 2018 Jul-Aug TI - Systemic Sclerosis Sine Scleroderma in Mexican Patients. Case Reports. PG - 230-232 LID - S1699-258X(16)30159-0 [pii] LID - 10.1016/j.reuma.2016.11.004 [doi] AB - Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. CONCLUSION: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc. CI - Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Vera-Lastra, Olga AU - Vera-Lastra O AD - Departamento de Medicina Interna, Unidad Médica de Alta Especialidad, Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional, La Raza, Instituto Mexicano del Seguro Social, México D.F., México; División de Estudios de posgrado, Facultad de Medicina, Universidad Nacional Autónoma de México, México D.F., México. Electronic address: Olgavera62@yahoo.com.mx. FAU - Sauceda-Casas, Christian Alexis AU - Sauceda-Casas CA AD - Universidad Autónoma de Sinaloa, México; Academia Mexicana de la Ciencia, México. FAU - Domínguez, María Del Pilar Cruz AU - Domínguez MDPC AD - División de Estudios de posgrado, Facultad de Medicina, Universidad Nacional Autónoma de México, México D.F., México; División de Investigación en Salud, Unidad Médica de Alta Especialidad, Hospital de Especialidades, Dr. Antonio Fraga Mouret, Centro Médico Nacional, La Raza. Instituto Mexicano del Seguro Social, México D.F., México. FAU - Alvarez, Sergio Alberto Mendoza AU - Alvarez SAM AD - Departamento de Medicina Interna, Unidad Médica de Alta Especialidad, Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional, La Raza, Instituto Mexicano del Seguro Social, México D.F., México; División de Estudios de posgrado, Facultad de Medicina, Universidad Nacional Autónoma de México, México D.F., México. FAU - Sepulceda-Delgado, Jesús AU - Sepulceda-Delgado J AD - Departamento de Medicina Interna, Unidad Médica de Alta Especialidad, Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional, La Raza, Instituto Mexicano del Seguro Social, México D.F., México. LA - eng LA - spa PT - Journal Article TT - Esclerosis sistémica sin esclerodermia en pacientes mexicanos. Serie de casos. DEP - 20170103 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Mexico MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*diagnosis/pathology OTO - NOTNLM OT - Clinical manifestations OT - Esclerosis sistémica sin esclerodermia OT - Manifestaciones clínicas OT - Prevalence OT - Prevalencia OT - Systemic sclerosis sine scleroderma EDAT- 2017/01/10 06:00 MHDA- 2018/12/12 06:00 CRDT- 2017/01/10 06:00 PHST- 2016/07/28 00:00 [received] PHST- 2016/11/15 00:00 [revised] PHST- 2016/11/23 00:00 [accepted] PHST- 2017/01/10 06:00 [pubmed] PHST- 2018/12/12 06:00 [medline] PHST- 2017/01/10 06:00 [entrez] AID - S1699-258X(16)30159-0 [pii] AID - 10.1016/j.reuma.2016.11.004 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2018 Jul-Aug;14(4):230-232. doi: 10.1016/j.reuma.2016.11.004. Epub 2017 Jan 3. PMID- 16769661 OWN - NLM STAT- MEDLINE DCOM- 20060802 LR - 20181201 IS - 0891-6934 (Print) IS - 0891-6934 (Linking) VI - 39 IP - 3 DP - 2006 May TI - Anti-Jo-1 antibody positive polymyositis--successful therapy with leflunomide. PG - 261-4 AB - Idiopathic inflammatory myopathies (IM), including dermatomyositis (DM) and polymyositis (PM), are a group of systemic rheumatologic diseases of unknown etiology characterized by chronic myositis. Antisynthetase antibodies such as the anti-Jo-1 antibody are known to be highly specific for inflammatory myopathies. Patients with this antibody frequently show a combination of symptoms including interstitial lung disease, fever, polyarthritis, myositis, Raynaud's phenomenon and "mechanic's hands". In the management of PM with anti-Jo-1 antibody, immunosuppressive agents are used to control the disease. Leflunomide is a new immunosuppressive drug recently introduced in the treatment of rheumatoid and psoriatic arthritis. Here, we report two cases of female patients with PM and anti-Jo-1 antibodies, who were successfully treated with leflunomide. FAU - Lange, Uwe AU - Lange U AD - Kerckhoff Clinic, University of Giessen, Department of Rheumatology and Clinical Immunology, Internal Medicine and Rheumatology, Bad Nauheim, Germany. u.lange@kerckhoff-klinik.de FAU - Piegsa, Manfred AU - Piegsa M FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Strunk, Johannes AU - Strunk J LA - eng PT - Case Reports PT - Journal Article PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - 0 (Isoxazoles) RN - 0 (Jo-1 antibody) RN - 9PHQ9Y1OLM (Prednisolone) RN - G162GK9U4W (Leflunomide) RN - MRK240IY2L (Azathioprine) SB - IM MH - Antibodies, Antinuclear/*immunology MH - Arthritis MH - Autoantibodies/immunology MH - Azathioprine/administration & dosage/therapeutic use MH - Drug Therapy, Combination MH - Female MH - Humans MH - Immunosuppressive Agents/administration & dosage/*therapeutic use MH - Isoxazoles/administration & dosage/*therapeutic use MH - Leflunomide MH - Middle Aged MH - Polymyositis/*drug therapy/immunology MH - Prednisolone/administration & dosage/therapeutic use EDAT- 2006/06/14 09:00 MHDA- 2006/08/03 09:00 CRDT- 2006/06/14 09:00 PHST- 2006/06/14 09:00 [pubmed] PHST- 2006/08/03 09:00 [medline] PHST- 2006/06/14 09:00 [entrez] AID - L16Q5UT762762U27 [pii] AID - 10.1080/08916930600623874 [doi] PST - ppublish SO - Autoimmunity. 2006 May;39(3):261-4. doi: 10.1080/08916930600623874. PMID- 24413201 OWN - NLM STAT- MEDLINE DCOM- 20151201 LR - 20160421 IS - 2299-2847 (Electronic) IS - 0032-373X (Linking) VI - 85 IP - 11 DP - 2013 Nov TI - Selected factors of fibrinolysis in the Buerger's disease. PG - 630-7 LID - /j/pjs.2013.85.issue-11/pjs-2013-0095/pjs-2013-0095.xml [pii] LID - 10.2478/pjs-2013-0095 [doi] AB - Thrombangiitis obliterans (TAO marked by coexistence of thrombotic and inflammatory changes of neurovascular tract has evoked a considerable dispute concerning pathogenesis of this disease. The aim of the study was to define the level of activation of fibirinolitic system in course of TAO disease by means of determination its basic constituents as well as to examine the essence of level of fibrinolysis disorders in pathogenesis and development of this disease. MATERIAL AND METHODS: Fifty patients with thrombangiitis obliterans (TAO), 30 patients with peripheral occlusive disease - PAOD (ASO) and 20 healthy volunteers (K) have been subjected to the examination. We determined the activity some factors of fibrinolysis: t-PA, PAI-1, PAP, plasminogen, α2-antiplasminogen, D-dimmer as well as euglobulin lysis time. The analysis comprised 7 features and 8 factors of variability: a membership to a group of patients, sex, age, smoking, aggravation of the disease within last 3 months, occurrence of Raynaud's symptom, a degree of ischemia according to Fontaine, time the disease lasted. RESULTS: The significant differences between the average were checked by means of t-Student test or variance analysis (ANOVA) and co-relation rate r (Pearson). We concluded that the average value of PAI-1 in the group TAO was significantly higher than in comparison with ASO group. The increased values were revealed in case of 76 % of patients. The euglobulin lysis time was vitally extended in case of 60% of patients in ASO group. In all three groups higher levels of α2-antiplasmin were detected in case of elderly patients compared to the younger ones. CONCLUSIONS: The obtained results allow us to ascertain the state of potentially weakened fibrinolysis in case of patients with Buerger's disease as well as with PAOD. FAU - Terlecki, Piotr AU - Terlecki P FAU - Feldo, Marcin AU - Feldo M FAU - Przywara, Stanisław AU - Przywara S FAU - Iłżecki, Marek AU - Iłżecki M FAU - Kęsik, Jan J AU - Kęsik JJ FAU - Borowski, Grzegorz AU - Borowski G FAU - Wroński, Jacek AU - Wroński J FAU - Zubilewicz, Tomasz AU - Zubilewicz T LA - eng PT - Comparative Study PT - Journal Article PL - Poland TA - Pol Przegl Chir JT - Polski przeglad chirurgiczny JID - 0376426 RN - 0 (Biomarkers) RN - 9001-31-4 (Fibrin) SB - IM MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Biomarkers/metabolism MH - Female MH - Fibrin/*metabolism MH - *Fibrinolysis MH - Humans MH - Male MH - Middle Aged MH - Sex Factors MH - Thromboangiitis Obliterans/*physiopathology MH - Young Adult EDAT- 2014/01/15 06:00 MHDA- 2015/12/15 06:00 CRDT- 2014/01/14 06:00 PHST- 2014/01/14 06:00 [entrez] PHST- 2014/01/15 06:00 [pubmed] PHST- 2015/12/15 06:00 [medline] AID - /j/pjs.2013.85.issue-11/pjs-2013-0095/pjs-2013-0095.xml [pii] AID - 10.2478/pjs-2013-0095 [doi] PST - ppublish SO - Pol Przegl Chir. 2013 Nov;85(11):630-7. doi: 10.2478/pjs-2013-0095. PMID- 35611111 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 33 IP - 1 DP - 2022 Mar TI - Clinical Phenotype and Mechanisms of Leukopenia/Neutropenia in Patients with Primary Sjögren's Syndrome. PG - 99-101 LID - 10.31138/mjr.33.1.99 [doi] AB - Sjögren's syndrome (SS) is a chronic, systemic autoimmune disease which afflicts mainly the exocrine salivary and lachrymal glands, leading to mouth and eye dryness. However, any organ can be affected during the disease course, resulting in a variety of clinical manifestations. Sjögren's syndrome clinical manifestations can be classified into glandular (sicca manifestations or parotid swelling), extra-glandular, either nonspecific (arthralgias, arthritis, Raynaud's phenomenon, fatigue) or peri-epithelial (primary biliary cirrhosis, interstitial nephritis, bronchiolitis), and extra-epithelial (palpable glomerulonephritis, peripheral neuropathy, purpura). In addition, SS patients display high risk for B cell lymphomas due to chronic antigenic stimulation. Although disease pathogenesis remains unclear, genetic, environmental, and immunologic factors are implicated. In the context of systemic autoimmune manifestations, SS patients may also present with hematologic abnormalities including anaemia, leukopenia (mainly neutropenia or lymphopenia), and thrombocytopenia. Although leukopenia has been reported as a laboratory finding in many case series or cohorts of SS patients and in very few studies it has been proposed as an independent risk factor for lymphoma, the clinical phenotype of SS patients with leukopenia/neutropenia and the implicated pathogenetic mechanisms have not been elucidated. In the current study, we intend to analyse the clinical phenotype of leukopenic/neutropenic SS patients and explore the possible pathogenetic mechanisms by detecting anti-neutrophil antibodies and investigate the role of apoptotic pathways, especially the contribution of TRAIL pathway and the cFLIP molecule. CI - © 2022 The Mediterranean Journal of Rheumatology (MJR). FAU - Stergiou, Ioanna E AU - Stergiou IE AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Kapsogeorgou, Efstathia E AU - Kapsogeorgou EE AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Voulgarelis, Michalis AU - Voulgarelis M AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Goules, Andreas V AU - Goules AV AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. LA - eng PT - Journal Article DEP - 20220331 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC9092102 OTO - NOTNLM OT - Sjögren’s syndrome OT - clinical phenotype OT - leukopenia OT - neutropenia OT - pathogenesis EDAT- 2022/05/26 06:00 MHDA- 2022/05/26 06:01 PMCR- 2022/03/31 CRDT- 2022/05/25 01:45 PHST- 2022/01/31 00:00 [received] PHST- 2022/02/15 00:00 [accepted] PHST- 2022/05/25 01:45 [entrez] PHST- 2022/05/26 06:00 [pubmed] PHST- 2022/05/26 06:01 [medline] PHST- 2022/03/31 00:00 [pmc-release] AID - MJR-33-1-99 [pii] AID - 10.31138/mjr.33.1.99 [doi] PST - epublish SO - Mediterr J Rheumatol. 2022 Mar 31;33(1):99-101. doi: 10.31138/mjr.33.1.99. eCollection 2022 Mar. PMID- 28417151 OWN - NLM STAT- MEDLINE DCOM- 20180424 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 37 IP - 8 DP - 2017 Aug TI - A review of the role and clinical utility of anti-Ro52/TRIM21 in systemic autoimmunity. PG - 1323-1333 LID - 10.1007/s00296-017-3718-1 [doi] AB - Anti-Ro52/tripartite motif-containing 21 (TRIM21) is a ubiquitous antibody found in a number of systemic autoimmune conditions including Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis, appearing in about half of these patients. Once coupled with its closely related antibody, anti-Ro60 as the anti-SSA antibody, anti-Ro52 is emerging as a unique antibody with direct pathogenic disease involvement and distinct clinical properties. As a result, recent attention has turned to this antibody and its clinical associations and utility. There is a suggestion of anti-Ro52 being associated with more clinical and laboratory markers of disease; however, marked disagreements occur about its association with various clinical entities such as interstitial lung disease and Raynaud's phenomena. Nevertheless, with a relative paucity of studies about these across the systemic autoimmunity paradigm, limited confidence can be invested in these conclusions. Although the antibody holds great potential as a biomarker, further studies examining its clinical utility are needed. This paper will review the mechanisms of Ro52 as an autoantigen and the clinical associations of anti-Ro52 in human autoimmunity. FAU - Lee, Adrian Y S AU - Lee AYS AD - Western Health, Melbourne, VIC, Australia. adrian.lee@wh.org.au. AD - School of Medicine, University of Tasmania, Hobart, TAS, Australia. adrian.lee@wh.org.au. LA - eng PT - Journal Article PT - Review DEP - 20170417 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Ribonucleoproteins) RN - 0 (SS-A Antigen) SB - IM MH - Antibodies, Antinuclear/immunology MH - Autoimmunity MH - Biomarkers/analysis MH - Enzyme-Linked Immunosorbent Assay MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/*immunology MH - Ribonucleoproteins/*immunology MH - Scleroderma, Systemic/diagnosis/*immunology MH - Sjogren's Syndrome/diagnosis/*immunology MH - SS-A Antigen OTO - NOTNLM OT - Antibody OT - Autoimmunity OT - Ro52 OT - SSA OT - TRIM21 EDAT- 2017/04/19 06:00 MHDA- 2018/04/25 06:00 CRDT- 2017/04/19 06:00 PHST- 2016/12/05 00:00 [received] PHST- 2017/04/06 00:00 [accepted] PHST- 2017/04/19 06:00 [pubmed] PHST- 2018/04/25 06:00 [medline] PHST- 2017/04/19 06:00 [entrez] AID - 10.1007/s00296-017-3718-1 [pii] AID - 10.1007/s00296-017-3718-1 [doi] PST - ppublish SO - Rheumatol Int. 2017 Aug;37(8):1323-1333. doi: 10.1007/s00296-017-3718-1. Epub 2017 Apr 17. PMID- 18852225 OWN - NLM STAT- MEDLINE DCOM- 20090305 LR - 20081014 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 17 IP - 11 DP - 2008 Nov TI - Vascular responsiveness in the microcirculation of patients with systemic lupus erythematosus is not impaired. PG - 1010-7 LID - 10.1177/0961203308091968 [doi] AB - As endothelial dysfunction is one of the earliest signs of atherosclerosis, which is accelerated in systemic lupus erythematosus (SLE), we assessed whether vascular responses of the cutaneous microcirculation are disturbed in SLE patients and influenced by Raynaud's phenomenon (RP). Laser Doppler fluxmetry (LDF) was used in combination with iontophoresis of acetylcholine (ACh) and sodium nitroprusside (SNP), an endothelium-dependent and endothelium-independent vasodilator respectively. 42 SLE patients with inactive disease, 12 of whom had RP and 19 age- and sex-matched controls were included. Furthermore, traditional and non-traditional risk factors for cardiovascular disease (CVD) were assessed, and markers of inflammation and endothelial activation were measured. Vascular responses of SLE patients without RP did not differ from controls. However, SLE patients with RP exhibited decreased vasodilatation compared with controls. SLE patients with RP also had longer arrival times of ACh and SNP than controls. Markers of inflammation and von Willebrand factor were increased in SLE patients. Smoking, the presence of SLE and RP were negatively associated with vascular responses in univariate analysis. In multivariate analyses, the only independent variable of vascular responses to ACh and SNP was the presence of RP. Despite signs of endothelial activation, SLE patients with inactive disease do not have altered vascular responses in the microcirculation compared with controls. In SLE patients with RP, cutaneous vascular responses to both ACh and SNP are impaired. Therefore, LDF of the microcirculation seems not to be the appropriate method to distinguish those SLE patients with an increased risk to develop CVD. FAU - de Leeuw, K AU - de Leeuw K AD - Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, University Medical Center, Groningen, The Netherlands. k.de.leeuw@int.umcg.nl FAU - Blaauw, J AU - Blaauw J FAU - Smit, Aj AU - Smit A FAU - Kallenberg, Cg AU - Kallenberg C FAU - Bijl, M AU - Bijl M LA - eng PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adult MH - Endothelium, Vascular/*physiopathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*physiopathology MH - *Microcirculation EDAT- 2008/10/15 09:00 MHDA- 2009/03/06 09:00 CRDT- 2008/10/15 09:00 PHST- 2008/10/15 09:00 [pubmed] PHST- 2009/03/06 09:00 [medline] PHST- 2008/10/15 09:00 [entrez] AID - 17/11/1010 [pii] AID - 10.1177/0961203308091968 [doi] PST - ppublish SO - Lupus. 2008 Nov;17(11):1010-7. doi: 10.1177/0961203308091968. PMID- 34221776 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210706 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 6 DP - 2021 Jun 25 TI - Clinical Puzzles and Decision-Making in Antisynthetase Syndrome. PG - e15931 LID - 10.7759/cureus.15931 [doi] LID - e15931 AB - We present a challenging clinical case of an antisynthetase syndrome (ASS) with a four-year follow-up. The disease debuted with skin manifestations and interstitial lung disease (ILD), then the severe Raynaud's phenomenon came to the fore with the development of occlusive vasculopathy and critical digital ischemia. After the relief of vascular lesions, the severity of the condition was determined by ILD. The use of combined pulse therapy with cyclophosphamide and methylprednisolone, treatment with intravenous immunoglobulin made it possible to reduce the activity of ASS: lung lesion and the progression of vasculopathy. However, after the termination of an unplanned pregnancy, the patient again experienced an exacerbation with ILD progression. It was decided to use rituximab, against which the patient's condition was stabilized. Clinical and laboratory remission was achieved, which was maintained for a year and a half. However, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic triggered a re-exacerbation of the pulmonary domain of the disease, which forced us to use a nintedanib with a positive clinical and instrumental effect. CI - Copyright © 2021, Yehudina et al. FAU - Yehudina, Yelyzaveta AU - Yehudina Y AD - Department of Rheumatology, Institute of Rheumatology, Kyiv, UKR. FAU - Trypilka, Svitlana AU - Trypilka S AD - Rheumatologist Policlinic Department, Rheumatologist Policlinic Department Communal Non-Commercial Enterprise of Kharkov Regional Council "Regional Clinical Hospital", Kharkiv, UKR. FAU - Isayeva, Anna AU - Isayeva A AD - Department of Cardiology, Government Institution "L.T.Malaya Therapy National Institute of the National Academy of Medical Sciences of Ukraine", Kharkiv, UKR. LA - eng PT - Case Reports PT - Journal Article DEP - 20210625 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8237923 OTO - NOTNLM OT - antisynthetase syndrome OT - digital ischemia OT - interstitial lung disease OT - intravenous immunoglobulin OT - nintedanib OT - rituximab OT - sars-cov-2 OT - treatment COIS- The authors have declared that no competing interests exist. EDAT- 2021/07/06 06:00 MHDA- 2021/07/06 06:01 PMCR- 2021/06/25 CRDT- 2021/07/05 10:10 PHST- 2021/07/05 10:10 [entrez] PHST- 2021/07/06 06:00 [pubmed] PHST- 2021/07/06 06:01 [medline] PHST- 2021/06/25 00:00 [pmc-release] AID - 10.7759/cureus.15931 [doi] PST - epublish SO - Cureus. 2021 Jun 25;13(6):e15931. doi: 10.7759/cureus.15931. PMID- 22840991 OWN - NLM STAT- MEDLINE DCOM- 20121130 LR - 20161209 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 41 IP - 9 Pt 2 DP - 2012 Sep TI - Autoantibodies in Sjögren's syndrome: clinical presentation and regulatory mechanisms. PG - e451-60 LID - 10.1016/j.lpm.2012.05.022 [doi] AB - Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease mostly affecting the exocrine glands. A large number of autoantibodies have been detected in the serum of patients with pSS. Among them, anti-Ro/SSA and anti-La/SSB autoantibodies are the most common; they serve as disease markers and are involved in the pathogenesis of neonatal lupus syndrome (NLS). Other autoantibodies are associated with significant clinical phenotypes, such as cryoglobulins with development of non-Hodgkin's lymphoma, anti-centromere antibodies with Raynaud's phenomenon and anti-mitochondrial antibodies with liver pathology. As a result, pSS patients can be schematically categorized in subgroups according to their serological profile. Although the clinical utility of these autoantibodies is appreciated, little is known about the mechanisms related to their production and the regulation of the autoimmune response. In the present review, the clinical subsets of patients with pSS related to different autoantibodies as well as the regulating mechanisms of their production with special emphasis on idiotypic/anti-idiotypic network are discussed. CI - Copyright © 2012 Elsevier Masson SAS. All rights reserved. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Medical School, University of Athens, Department of Pathophysiology, 11527 Athens, Greece. agtzi@med.uoa.gr FAU - Tatouli, Ioanna P AU - Tatouli IP FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM LA - eng PT - Journal Article PT - Review DEP - 20120725 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunoglobulin Idiotypes) RN - 0 (Immunologic Factors) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) SB - IM MH - Antibodies, Antinuclear/analysis MH - Autoantibodies/analysis/*immunology MH - Autoimmune Diseases/immunology MH - Biomarkers/analysis MH - Humans MH - Immunoglobulin Idiotypes/analysis MH - Immunologic Factors/analysis MH - Phenotype MH - Sjogren's Syndrome/*immunology EDAT- 2012/07/31 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/07/31 06:00 PHST- 2012/04/30 00:00 [received] PHST- 2012/05/02 00:00 [accepted] PHST- 2012/07/31 06:00 [entrez] PHST- 2012/07/31 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - S0755-4982(12)00383-1 [pii] AID - 10.1016/j.lpm.2012.05.022 [doi] PST - ppublish SO - Presse Med. 2012 Sep;41(9 Pt 2):e451-60. doi: 10.1016/j.lpm.2012.05.022. Epub 2012 Jul 25. PMID- 24470658 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140128 LR - 20211021 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 59 IP - 1 DP - 2014 Jan TI - Cutaneous manifestations of mixed connective tissue disease: study from a tertiary care hospital in eastern India. PG - 35-40 LID - 10.4103/0019-5154.123491 [doi] AB - CONTEXT: Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe. AIMS: This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe. SETTINGS AND DESIGN: Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years. MATERIALS AND METHODS: The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients. STATISTICAL ANALYSIS USED: SPSS (Version 17) and MedCalc (Version 11.6). RESULTS: THE MALE: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynaud's phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this disease. CONCLUSIONS: Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease. FAU - Sen, Sumit AU - Sen S AD - Department of Dermatology, Venereology and Leprosy, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Sinhamahapatra, Pradyot AU - Sinhamahapatra P AD - Department of Rheumatology, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Choudhury, Supriyo AU - Choudhury S AD - Department of Pharmacology, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Gangopadhyay, Anusree AU - Gangopadhyay A AD - Department of Dermatology, Venereology and Leprosy, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Bala, Sanchaita AU - Bala S AD - Department of Dermatology, Venereology and Leprosy, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Sircar, Geetabali AU - Sircar G AD - Department of Rheumatology, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Chatterjee, Gobinda AU - Chatterjee G AD - Department of Dermatology, Venereology and Leprosy, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. FAU - Ghosh, Alakendu AU - Ghosh A AD - Department of Rheumatology, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Salt Lake City, Kolkata, India. LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3884926 OTO - NOTNLM OT - Autoimmune disease OT - inflammatory myositis OT - mixed connective tissue disease OT - overlap syndromes OT - sausage fingers OT - synovitis COIS- Conflict of Interest: Nil. EDAT- 2014/01/29 06:00 MHDA- 2014/01/29 06:01 PMCR- 2014/01/01 CRDT- 2014/01/29 06:00 PHST- 2014/01/29 06:00 [entrez] PHST- 2014/01/29 06:00 [pubmed] PHST- 2014/01/29 06:01 [medline] PHST- 2014/01/01 00:00 [pmc-release] AID - IJD-59-35 [pii] AID - 10.4103/0019-5154.123491 [doi] PST - ppublish SO - Indian J Dermatol. 2014 Jan;59(1):35-40. doi: 10.4103/0019-5154.123491. PMID- 30210881 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240330 IS - 2090-6560 (Print) IS - 2090-6579 (Electronic) IS - 2090-6579 (Linking) VI - 2018 DP - 2018 TI - Digital Ischemia as an Unusual Manifestation of Hodgkin's Lymphoma. PG - 1980749 LID - 10.1155/2018/1980749 [doi] LID - 1980749 AB - Digital ischemia is associated with atherosclerotic, thromboembolic, or connective tissue diseases. Less often, it can be related to malignancy. Paraneoplastic vascular acrosyndromes (Raynaud's syndrome, acrocianosis, and acronecrosis) are associated with adenocarcinoma and less frequently with hematological malignancies. We report the case of a 45-year-old male, smoker, with a 10-day history of pain, cyanosis, and progressive digital necrosis in both hands. In the previous four months, he noticed painless mass in the right axillary gap, drenching night sweats, and weight loss. Physical examination at admission highlighted necrotic lesions on the distal phalanges of both hands (except the thumbs), enlarged lymph nodes in right axillary, and right supraclavicular gaps. Arteriography of upper limbs demonstrated a distal stop in all bilateral digital arteries. Digital ischemia was interpreted as a paraneoplastic phenomenon after other common etiologies were ruled out. Amputation of three phalanges was required due to necrosis. Biopsy of axillary nodes demonstrated nodular sclerosis classical Hodgkin's lymphoma (HL). The patient started conventional ABVD protocol (doxorubicin, bleomycin, vinblastine, and dacarbazine). After 6 cycles, he remained asymptomatic and symptoms of digital ischemia were completely resolved. It was concluded that the presence of acral vascular syndromes should alert the physician about the possibility of underlying malignant disease. Prompt investigation and treatment should be rapidly performed to avoid digital sequelae. FAU - Villano, Fiorella AU - Villano F AUID- ORCID: 0000-0002-6801-4455 AD - Department of Hematology, Médica Uruguaya (MUCAM), Montevideo, Uruguay. FAU - Peixoto, Adriana AU - Peixoto A AD - Department of Hematology, Médica Uruguaya (MUCAM), Montevideo, Uruguay. FAU - Riva, Eloísa AU - Riva E AD - Department of Hematology, Médica Uruguaya (MUCAM), Montevideo, Uruguay. FAU - Di Matteo, Carina AU - Di Matteo C AD - Laboratory of Pathology Carina Di Matteo, Montevideo, Uruguay. FAU - Díaz, Lilián AU - Díaz L AD - Department of Hematology, Médica Uruguaya (MUCAM), Montevideo, Uruguay. LA - eng PT - Case Reports PT - Journal Article DEP - 20180819 PL - United States TA - Case Rep Hematol JT - Case reports in hematology JID - 101576456 PMC - PMC6120276 EDAT- 2018/09/14 06:00 MHDA- 2018/09/14 06:01 PMCR- 2018/08/19 CRDT- 2018/09/14 06:00 PHST- 2018/03/14 00:00 [received] PHST- 2018/06/04 00:00 [revised] PHST- 2018/07/25 00:00 [accepted] PHST- 2018/09/14 06:00 [entrez] PHST- 2018/09/14 06:00 [pubmed] PHST- 2018/09/14 06:01 [medline] PHST- 2018/08/19 00:00 [pmc-release] AID - 10.1155/2018/1980749 [doi] PST - epublish SO - Case Rep Hematol. 2018 Aug 19;2018:1980749. doi: 10.1155/2018/1980749. eCollection 2018. PMID- 34706954 OWN - NLM STAT- MEDLINE DCOM- 20211103 LR - 20260225 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 11 IP - 10 DP - 2021 Oct 27 TI - Evaluation of sweating responses in patients with collagen disease using the quantitative sudomotor axon reflex test (QSART): a study protocol for an investigator-initiated, prospective, observational clinical study. PG - e050690 LID - 10.1136/bmjopen-2021-050690 [doi] LID - e050690 AB - INTRODUCTION: Sweat secretion is controlled by the sympathetic nervous system and is less active during winter than in the summer. Raynaud's phenomenon is affected by an excessive strain of the sympathetic nerves after exposure to a cold environment, thus reducing the quality of life of patients with collagen disease. Herein, we focus on the eccrine sweat glands that receive both adrenergic and cholinergic innervation. Our hypothesis is that excessive activation of sympathetic nerve in Raynaud's phenomenon can affect sweating, especially in winter. This study is designed to evaluate the neuroactive sweating responses in patients with collagen disease and to assess its association with skin findings in peripheral circulatory disorders. METHODS AND ANALYSIS: The study will be conducted at a single centre in Japan. Patients with systemic sclerosis, Sjogren's syndrome, systemic lupus erythematosus, mixed connective tissue disease, and dermatomyositis will be assessed using the quantitative sudomotor axon reflex test. The primary outcomes will be sweat volume and reaction time due to axon reflex and the Raynaud's condition score. The secondary outcomes will include patient background, skin symptoms (digital ulcers, pernio-like eruptions, subcutaneous calcifications, telangiectasia, nailfold capillary dilatation/bleeding and degree of skin sclerosis) and skin surface temperature. Evaluation will be done two times, during the summer and winter, allowing for the assessment of seasonal differences in sweating responses. ETHICS AND DISSEMINATION: Ethical approval of this study was certified by the clinical research review board of Nagasaki University Hospital (Reference number: CRB19-001). We will disseminate the findings of this study through peer-reviewed publications and conference presentations. TRIAL REGISTRATION NUMBER: jRCTs072190009; pre-results. CI - © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Ashida, Miwa AU - Ashida M AUID- ORCID: 0000-0002-4215-680X AD - Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan ashida@nagasaki-u.ac.jp. FAU - Koga, Tomohiro AU - Koga T AD - Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. AD - Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. FAU - Morimoto, Shimpei AU - Morimoto S AD - Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. AD - Innovation Platform & Office for Precision Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. AD - Clinical Research Center, Nagasaki University Hospital, Nagasaki, Japan. FAU - Yozaki, Mariko AU - Yozaki M AD - Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. FAU - Ehara, Daisuke AU - Ehara D AD - Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. FAU - Koike, Yuta AU - Koike Y AD - Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. FAU - Murota, H AU - Murota H AD - Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan. LA - eng PT - Clinical Trial Protocol PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211027 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - Axons MH - *Collagen Diseases MH - Humans MH - Observational Studies as Topic MH - Prospective Studies MH - Quality of Life MH - Reflex MH - Sweat Glands MH - *Sweating PMC - PMC8552179 OTO - NOTNLM OT - dermatology OT - qualitative research OT - rheumatology OT - statistics & research methods COIS- Competing interests: None declared. EDAT- 2021/10/29 06:00 MHDA- 2021/11/04 06:00 PMCR- 2021/10/27 CRDT- 2021/10/28 05:50 PHST- 2021/10/28 05:50 [entrez] PHST- 2021/10/29 06:00 [pubmed] PHST- 2021/11/04 06:00 [medline] PHST- 2021/10/27 00:00 [pmc-release] AID - bmjopen-2021-050690 [pii] AID - 10.1136/bmjopen-2021-050690 [doi] PST - epublish SO - BMJ Open. 2021 Oct 27;11(10):e050690. doi: 10.1136/bmjopen-2021-050690. PMID- 39450217 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241026 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 10 DP - 2024 Oct TI - Not Just Myocarditis: Mixed Connective Tissue Disease (MCTD) and Overlap Myositis With Anti-Ku Positivity in a Young Male With Shortness of Breath. PG - e72310 LID - 10.7759/cureus.72310 [doi] LID - e72310 AB - Mixed connective tissue disease (MCTD) is an autoimmune disorder characterized by high levels of anti-U1 ribonucleoprotein (RNP) antibodies and overlapping clinical features of autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis (PM). Anti-Ku antibodies have been associated with overlap syndromes, which can present with symptoms such as Raynaud's phenomenon, arthritis, and myositis.  A 19-year-old male athlete presented with myositis, notable for cardiac involvement. Diagnostic testing revealed elevated anti-RNP and anti-Ku antibodies, and a muscle biopsy indicated scleromyositis/overlap myositis. The patient was treated with high-dose corticosteroids, intravenous immunoglobulin (IVIG), rituximab, and mycophenolate mofetil, which led to significant improvement in muscle strength and cardiac function. This case highlights the diagnostic complexity of MCTD when associated with positive anti-Ku antibodies, overlap syndromes, and cardiac involvement. Successful management emphasizes the importance of a tailored, multi-modal therapeutic approach. CI - Copyright © 2024, Alsulami et al. FAU - Alsulami, Kawthar AU - Alsulami K AD - Rheumatology, King Abdulaziz University Faculty of Medicine, Jeddah, SAU. FAU - D'Aoust, Julie AU - D'Aoust J AD - Rheumatology, University of Ottawa, Ottawa, CAN. LA - eng PT - Case Reports PT - Journal Article DEP - 20241024 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11500815 OTO - NOTNLM OT - anti-ku antibodies OT - inflammatory myositis OT - overlap myositis OT - peri-myocarditis OT - scleromyositis COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/10/25 06:24 MHDA- 2024/10/25 06:25 PMCR- 2024/10/24 CRDT- 2024/10/25 04:24 PHST- 2024/10/24 00:00 [accepted] PHST- 2024/10/25 06:25 [medline] PHST- 2024/10/25 06:24 [pubmed] PHST- 2024/10/25 04:24 [entrez] PHST- 2024/10/24 00:00 [pmc-release] AID - 10.7759/cureus.72310 [doi] PST - epublish SO - Cureus. 2024 Oct 24;16(10):e72310. doi: 10.7759/cureus.72310. eCollection 2024 Oct. PMID- 19674983 OWN - NLM STAT- MEDLINE DCOM- 20091005 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 68 IP - 9 DP - 2009 Sep TI - The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy. PG - 1377-80 LID - 10.1136/ard.2008.106302 [doi] AB - Early diagnosis of systemic sclerosis (SSc) may allow the start of treatment that could slow disease progression. For this reason early diagnosis of the disease is of pivotal importance. However, the lack of diagnostic criteria and valid predictors significantly limit patient evaluation and the use of potentially effective drugs in the earliest phase of SSc. Early SSc may be suspected on the basis of Raynaud's phenomenon, puffy fingers, autoantibodies and SSc capillaroscopic pattern. In practice, the aim is to have criteria for the diagnosis of very early SSc. The criteria that are proposed are obviously provisional and need to be validated: (a) initially through a Delphi technique; (b) thereafter perhaps using already available datasets; but (c) of critical importance, through prospective studies. Only after prospective studies can these potential criteria be considered validated. The consensus on criteria for the classification of very early SSc might be part of the evolving EULAR/ACR project of reclassification of SSc. FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M AD - Department of Biomedicine, Division of Rheumatology AOUC, Villa Monna Tessa, Viale Pieraccini 18, Florence I-50139, Italy. cerinic@unifi.it FAU - Allanore, Y AU - Allanore Y FAU - Czirják, L AU - Czirják L FAU - Tyndall, A AU - Tyndall A FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Denton, C AU - Denton C FAU - Valentini, G AU - Valentini G FAU - Distler, O AU - Distler O FAU - Fligelstone, K AU - Fligelstone K FAU - Tyrrel-Kennedy, A AU - Tyrrel-Kennedy A FAU - Farge, D AU - Farge D FAU - Kowal-Bielecka, O AU - Kowal-Bielecka O FAU - van den Hoogen, F AU - van den Hoogen F FAU - Cutolo, M AU - Cutolo M FAU - Sampaio-Barros, P D AU - Sampaio-Barros PD FAU - Nash, P AU - Nash P FAU - Takehara, K AU - Takehara K FAU - Furst, D E AU - Furst DE LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Early Diagnosis MH - Humans MH - Scleroderma, Systemic/classification/*diagnosis/drug therapy EDAT- 2009/08/14 09:00 MHDA- 2009/10/06 06:00 CRDT- 2009/08/14 09:00 PHST- 2009/08/14 09:00 [entrez] PHST- 2009/08/14 09:00 [pubmed] PHST- 2009/10/06 06:00 [medline] AID - S0003-4967(24)21642-3 [pii] AID - 10.1136/ard.2008.106302 [doi] PST - ppublish SO - Ann Rheum Dis. 2009 Sep;68(9):1377-80. doi: 10.1136/ard.2008.106302. PMID- 36324396 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221104 IS - 2666-2450 (Electronic) IS - 2666-2450 (Linking) VI - 3 DP - 2022 TI - Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic manifestations (RVCL-S): An update on basic science and clinical perspectives. PG - 100046 LID - 10.1016/j.cccb.2022.100046 [doi] LID - 100046 AB - Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare, underrecognized, systemic small vessel disease caused by heterozygous C-terminal truncating TREX1 mutations. The disease is characterized by vascular retinopathy, focal neurological complaints, cognitive decline and a wide range of systemic manifestations, including Raynaud's phenomenon, anemia and liver and kidney disease. Eventually, RVCL-S leads to premature death. The underlying pathological finding in RVCL-S is a nonatherosclerotic, amyloid-negative angiopathy involving small arteries and capillaries. However, the exact mechanisms by which the truncated TREX1 protein causes angiopathy remains unknown. Timely recognition of this disease is important to slow down and treat complications of the disorder, but also to prevent unnecessary (invasive) diagnostic or therapeutic procedures. As we move forward, translational research combining basic science advances and clinical findings as well as studies focusing on natural history following RVCL-S patients at different disease stages, will be critical to help elucidate RVCL-S pathophysiology. These studies will also provide the tools to identify appropriate biomarkers and therapeutic agent options for RVCL-S patients. CI - © 2022 The Authors. Published by Elsevier B.V. FAU - Wilms, A E AU - Wilms AE AD - Leiden University Medical Centre, Department of Neurology the Netherlands. FAU - de Boer, I AU - de Boer I AD - Leiden University Medical Centre, Department of Neurology the Netherlands. FAU - Terwindt, G M AU - Terwindt GM AD - Leiden University Medical Centre, Department of Neurology the Netherlands. LA - eng PT - Journal Article DEP - 20220214 PL - Netherlands TA - Cereb Circ Cogn Behav JT - Cerebral circulation - cognition and behavior JID - 101774849 PMC - PMC9616387 OTO - NOTNLM OT - CT, computed tomography OT - Cerebral small vessel disease OT - Hereditary OT - MRI, magnetic resonance imaging OT - OCT, optical coherence tomography OT - RVCL-S OT - RVCL-S, retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations OT - Retinopathy OT - TMV, total macular volume OT - cSVD, cerebral small vessel disease OT - pRNFL, peripapillary retinal nerve fiber layer EDAT- 2022/11/04 06:00 MHDA- 2022/11/04 06:01 PMCR- 2022/02/14 CRDT- 2022/11/03 02:25 PHST- 2021/12/06 00:00 [received] PHST- 2022/02/07 00:00 [revised] PHST- 2022/02/13 00:00 [accepted] PHST- 2022/11/03 02:25 [entrez] PHST- 2022/11/04 06:00 [pubmed] PHST- 2022/11/04 06:01 [medline] PHST- 2022/02/14 00:00 [pmc-release] AID - S2666-2450(22)00011-3 [pii] AID - 100046 [pii] AID - 10.1016/j.cccb.2022.100046 [doi] PST - epublish SO - Cereb Circ Cogn Behav. 2022 Feb 14;3:100046. doi: 10.1016/j.cccb.2022.100046. eCollection 2022. PMID- 22217338 OWN - NLM STAT- MEDLINE DCOM- 20120606 LR - 20120111 IS - 1532-432X (Electronic) IS - 0363-0269 (Linking) VI - 36 IP - 1 DP - 2012 TI - Secondary erythrocytosis due to compound homozygosity, but not compound heterozygosity, for Hb Luton and α-thalassemia: a family study. PG - 7-17 LID - 10.3109/03630269.2011.641134 [doi] AB - We describe the hematological and clinical features of homozygous Hb Luton (OMIM 141800.0172), a high affinity α-globin variant that has not been previously described in the homozygous state. The proband was found to have a high hemoglobin (Hb) concentration following a routine blood count prior to a planned appendectomy at the age of 16 years. Investigation showed that she was homozygous for both Hb Luton [α89(FG1)His→Leu (CAC>CTC), a high oxygen affinity Hb)] and homozygous for α(+)-thalassemia (α(+)-thal), while her mother, maternal aunt and half-brother were heterozygous for these conditions. Further investigation showed that she also had Gilbert's disease and Raynaud's syndrome. As far as we are aware, this is also the first reported family with a subject homozygous for both Hb Luton and α-thal so that the proband has no nomal α-globin. The parents of the proband are first cousins and originate from Pakistan. FAU - El-Sharkawi, Dima AU - El-Sharkawi D AD - Department of Haematology, University College London, London, UK. FAU - Fisher, Chris AU - Fisher C FAU - Khambadkone, Sachin AU - Khambadkone S FAU - Stephens, Adrian D AU - Stephens AD FAU - Porter, John B AU - Porter JB LA - eng PT - Case Reports PT - Journal Article DEP - 20120104 PL - England TA - Hemoglobin JT - Hemoglobin JID - 7705865 RN - 0 (Hemoglobins, Abnormal) RN - 0 (hemoglobin Luton) SB - IM MH - Adolescent MH - Chromatography, High Pressure Liquid MH - Consanguinity MH - DNA Mutational Analysis MH - Family Health MH - Female MH - Hemoglobins, Abnormal/*genetics MH - Heterozygote MH - Homozygote MH - Humans MH - Male MH - Pedigree MH - Polycythemia/complications/*genetics MH - Polymerase Chain Reaction MH - alpha-Thalassemia/complications/*genetics EDAT- 2012/01/06 06:00 MHDA- 2012/06/07 06:00 CRDT- 2012/01/06 06:00 PHST- 2012/01/06 06:00 [entrez] PHST- 2012/01/06 06:00 [pubmed] PHST- 2012/06/07 06:00 [medline] AID - 10.3109/03630269.2011.641134 [doi] PST - ppublish SO - Hemoglobin. 2012;36(1):7-17. doi: 10.3109/03630269.2011.641134. Epub 2012 Jan 4. PMID- 18984414 OWN - NLM STAT- MEDLINE DCOM- 20090129 LR - 20191210 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 34 IP - 4 DP - 2008 Nov TI - Patient-reported outcomes including fatigue in primary Sjögren's syndrome. PG - 949-62, ix LID - 10.1016/j.rdc.2008.08.010 [doi] AB - The hallmark of Sjögren's syndrome is focal lymphocytic infiltration of exocrine glands leading to mucosal dryness, particularly of the eyes and mouth. In addition, approximately 70% of patients report fatigue as a particularly prominent and disabling feature associated with reduced health-related quality of life. Other key patient-reported extraglandular symptoms include arthralgia, myalgia, and Raynaud's phenomenon. This article reviews these patient-reported features, their relationships with objective assessment of the disease, potential therapies for these symptoms, and how measurements of these symptoms are relevant to outcome assessment in clinical therapeutic trials. FAU - Bowman, Simon J AU - Bowman SJ AD - Rheumatology Department, University Hospital Birmingham (Selly Oak), Birmingham B296JD, UK. simon.bowman@uhb.nhs.uk LA - eng PT - Journal Article PT - Review PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 SB - IM MH - Activities of Daily Living MH - Clinical Trials as Topic MH - Fatigue/*etiology/physiopathology/therapy MH - Health Status Indicators MH - Humans MH - Keratoconjunctivitis Sicca/*etiology/physiopathology MH - Outcome Assessment, Health Care MH - Patient Satisfaction MH - Sjogren's Syndrome/*complications/physiopathology/therapy MH - Surveys and Questionnaires MH - Xerostomia/*etiology/physiopathology RF - 62 EDAT- 2008/11/06 09:00 MHDA- 2009/01/30 09:00 CRDT- 2008/11/06 09:00 PHST- 2008/11/06 09:00 [pubmed] PHST- 2009/01/30 09:00 [medline] PHST- 2008/11/06 09:00 [entrez] AID - S0889-857X(08)00082-3 [pii] AID - 10.1016/j.rdc.2008.08.010 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2008 Nov;34(4):949-62, ix. doi: 10.1016/j.rdc.2008.08.010. PMID- 17110308 OWN - NLM STAT- MEDLINE DCOM- 20070215 LR - 20220409 IS - 1568-9972 (Print) IS - 1568-9972 (Linking) VI - 6 IP - 1 DP - 2006 Nov TI - Undifferentiated connective tissue diseases (UCTD). PG - 1-4 AB - The term undifferentiated connective tissue diseases is used to define conditions characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not satisfy the classificative criteria for defined connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), rheumatoid arthritis (RA) and others. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue diseases (UCTD). The most characteristic symptoms of UCTD are represented by arthritis and arthralgias, Raynaud's phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Eighty percent of these patients have a single autoantibody specificity, more frequently anti-Ro and anti-RNP antibodies. Stable UCTD are considered as distinct clinical entities and therefore it has been proposed to define those conditions as UCTD. Classificative criteria have also been proposed and a work to better define them is still under way. FAU - Mosca, M AU - Mosca M AD - Rheumatology Unit, Department of Internal Medicine, University of Pisa, Via Roma, 67, 56126 Pisa, Italy. FAU - Tani, C AU - Tani C FAU - Neri, C AU - Neri C FAU - Baldini, C AU - Baldini C FAU - Bombardieri, S AU - Bombardieri S LA - eng PT - Journal Article PT - Review DEP - 20060419 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/immunology MH - Autoimmune Diseases/immunology/physiopathology MH - Autoimmunity/*physiology MH - Connective Tissue Diseases/diagnosis/immunology/*physiopathology MH - Humans RF - 31 EDAT- 2006/11/18 09:00 MHDA- 2007/02/16 09:00 CRDT- 2006/11/18 09:00 PHST- 2006/11/18 09:00 [pubmed] PHST- 2007/02/16 09:00 [medline] PHST- 2006/11/18 09:00 [entrez] AID - S1568-9972(06)00035-8 [pii] AID - 10.1016/j.autrev.2006.03.004 [doi] PST - ppublish SO - Autoimmun Rev. 2006 Nov;6(1):1-4. doi: 10.1016/j.autrev.2006.03.004. Epub 2006 Apr 19. PMID- 35382498 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 6 IP - 3 DP - 2021 Oct TI - Circulating Gremlin-1 is elevated in systemic sclerosis patients. PG - 286-289 LID - 10.1177/23971983211036571 [doi] AB - INTRODUCTION: Systemic sclerosis is an autoimmune connective tissue disease in which there is activation of the immune system, vascular disease and fibrosis. Activation of quiescent fibroblasts to myofibroblasts is key to disease pathogenesis. Gremlin-1 is a bone morphogenetic protein antagonist which is important in development and we recently reported in skin fibrosis. The aim of this study was to determine the serum circulating levels of Gremlin-1 in early diffuse systemic sclerosis. METHODS: Twenty-one early diffuse systemic sclerosis patients (less than 2 years from first non-Raynaud's symptom) were included and age and sex-matched healthy controls. Serum was isolated from blood and measured with a specific enzyme-linked immunoassay for Gremlin-1. Clinical variables were also measured. RESULTS: Significantly elevated Gremlin-1 was found in sera of early diffuse systemic sclerosis patients (p < 0.001). In patients with interstitial lung disease, this compared to systemic sclerosis without evidence of interstitial lung disease, Gremlin-1 was significantly elevated (p < 0.0007). A correlation was found between circulating Gremlin-1 and modified Rodnan Skin Score, albeit weak. DISCUSSION: In early diffuse systemic sclerosis patients, elevated Gremlin-1 is found in serum. This is particularly prominent in systemic sclerosis-associated interstitial lung disease. This suggests that Gremlin-1 may be a biomarker for systemic sclerosis interstitial lung disease. CI - © The Author(s) 2021. FAU - O'Reilly, Steven AU - O'Reilly S AUID- ORCID: 0000-0001-7483-740X AD - Department of Biosciences, Durham University, Durham, UK. LA - eng PT - Journal Article DEP - 20210802 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922663 OTO - NOTNLM OT - Gremlin-1 OT - bone morphogenetic protein OT - lung fibrosis OT - scleroderma OT - transforming growth factor-beta COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2022/04/07 06:00 MHDA- 2022/04/07 06:01 PMCR- 2021/08/02 CRDT- 2022/04/06 05:17 PHST- 2021/05/20 00:00 [received] PHST- 2021/07/12 00:00 [accepted] PHST- 2022/04/06 05:17 [entrez] PHST- 2022/04/07 06:00 [pubmed] PHST- 2022/04/07 06:01 [medline] PHST- 2021/08/02 00:00 [pmc-release] AID - 10.1177_23971983211036571 [pii] AID - 10.1177/23971983211036571 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2021 Oct;6(3):286-289. doi: 10.1177/23971983211036571. Epub 2021 Aug 2. PMID- 25318613 OWN - NLM STAT- MEDLINE DCOM- 20150924 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 34 IP - 2 DP - 2015 Feb TI - Nailfold capillaroscopic changes in dermatomyositis and polymyositis. PG - 279-84 LID - 10.1007/s10067-014-2795-8 [doi] AB - Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study was to describe the capillaroscopic differences between PM and DM patients and possible correlation with clinical and serological features. We analyzed 52 unselected patients with IM in a cross-sectional study in a 6-month period. NVC findings of 29 DM and 23 PM patients were compared with those of 52 patients with primary Raynaud's phenomenon. Tortuosities, capillary loss, enlarged and giant capillaries, microhemorrhages, and ramified capillaries were scored by a semiquantitative rating; disorganization of the vascular array, avascular areas, and scleroderma pattern were scored as presence/absence. Sex, mean age, and mean disease duration were similar in both groups. Disorganization of the vascular array, enlarged and giant capillaries, capillary loss, and scleroderma-like pattern were observed almost only in IM patients. Significant differences were observed between PM and DM with higher frequency and mean score of NVC changes in DM. In DM patients with disease duration ≤6 months (14/29 patients), capillary density was significantly reduced (P = 0.039) and giant capillaries more frequent (P = 0.027), compared with patients with longer disease duration, while a scleroderma pattern tended to be more frequent in patients with a disease duration of less than 6 months. On the contrary, no differences were observed for ramified capillaries with regard to disease duration. Capillaroscopic alterations are identified only in DM patients as expression of diffuse microangiopathy; surprisingly, more severe changes were associated with shorter disease duration, while persistence of ramified capillaries with long-standing disease. FAU - Manfredi, A AU - Manfredi A AD - Rheumatology Unit, University of Modena and Reggio Emilia, Via del Pozzo, 71, 41121, Modena, Italy, andreina.manfredi@gmail.com. FAU - Sebastiani, M AU - Sebastiani M FAU - Cassone, G AU - Cassone G FAU - Pipitone, N AU - Pipitone N FAU - Giuggioli, D AU - Giuggioli D FAU - Colaci, M AU - Colaci M FAU - Salvarani, C AU - Salvarani C FAU - Ferri, C AU - Ferri C LA - eng PT - Journal Article DEP - 20141017 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Dermatomyositis/*physiopathology MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Polymyositis/*physiopathology EDAT- 2014/10/17 06:00 MHDA- 2015/09/25 06:00 CRDT- 2014/10/17 06:00 PHST- 2014/07/15 00:00 [received] PHST- 2014/10/05 00:00 [accepted] PHST- 2014/09/13 00:00 [revised] PHST- 2014/10/17 06:00 [entrez] PHST- 2014/10/17 06:00 [pubmed] PHST- 2015/09/25 06:00 [medline] AID - 10.1007/s10067-014-2795-8 [doi] PST - ppublish SO - Clin Rheumatol. 2015 Feb;34(2):279-84. doi: 10.1007/s10067-014-2795-8. Epub 2014 Oct 17. PMID- 42027437 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260424 LR - 20260424 IS - 1735-0344 (Print) IS - 2345-3729 (Electronic) IS - 1735-0344 (Linking) VI - 24 IP - 2 DP - 2025 Feb TI - When Silica Returns-Erasmus Syndrome, a Rare Entity. PG - 198-201 AB - INTRODUCTION: Systemic sclerosis (SS) is an autoimmune disease with vascular changes and diffuse tissue fibrosis. Silica exposure is one of the strongest recognized risk factors for the development of systemic sclerosis (relative risk 3.2). The co-occurrence of systemic sclerosis in a patient with silica exposure is termed Erasmus Syndrome. Few case reports are available in the literature. CASE PRESENTATION: We report here a case of Erasmus syndrome in a 54-year-old stone worker. The patient presented with diffuse skin thickening, hyperpigmentation, Raynaud's phenomenon, and arthralgia along with radiological features of silicosis. Serological markers of systemic sclerosis were strongly positive, and skin biopsy confirmed scleroderma. Hence, a diagnosis of Erasmus syndrome was made. The clinical, serological, and histopathological features of SA-SS in the patient were indistinguishable from those of idiopathic SS; however, a history of silica dust exposure and evidence of silicosis supported the diagnosis of SA-SS. CONCLUSION: Careful screening should be done in patients with silicosis along with systemic manifestations to rule out any associated connective tissue disorder. A detailed exposure history should be carefully obtained for all patients with systemic sclerosis. CI - ©2025 NRITLD, National Research Institute of Tuberculosis and Lung Disease, Iran. FAU - B L, Parvathi AU - B L P AD - Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India. FAU - Shahul, Aneesa AU - Shahul A AD - Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India. FAU - Asfahan, Shahir AU - Asfahan S AD - Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India. FAU - Dutt, Naveen AU - Dutt N AD - Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India. FAU - Jalandra, Ramniwas AU - Jalandra R AD - Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India. LA - eng PT - Case Reports PT - Journal Article PL - Iran TA - Tanaffos JT - Tanaffos JID - 101308232 PMC - PMC13102338 OTO - NOTNLM OT - Scleroderma OT - Silica-associated systemic sclerosis (SA-SS) OT - Silicosis OT - Systemic sclerosis (SS) EDAT- 2025/02/01 00:00 MHDA- 2025/02/01 00:01 PMCR- 2025/02/01 CRDT- 2026/04/24 04:46 PHST- 2024/01/03 00:00 [received] PHST- 2024/08/20 00:00 [accepted] PHST- 2025/02/01 00:01 [medline] PHST- 2025/02/01 00:00 [pubmed] PHST- 2026/04/24 04:46 [entrez] PHST- 2025/02/01 00:00 [pmc-release] AID - Tanaffos-24-198 [pii] PST - ppublish SO - Tanaffos. 2025 Feb;24(2):198-201. PMID- 26679670 OWN - NLM STAT- MEDLINE DCOM- 20160202 LR - 20181202 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 47 IP - 6 DP - 2015 Dec 18 TI - [Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipidus: a case report]. PG - 1028-30 AB - The male patient reported here presented as gangrene and central diabetes insipidus (CDI), who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud's phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, β2-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no sufficient evidence to diagnose any specific disease; as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis (SNV). For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0.4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pituitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor. FAU - Huang, Qing AU - Huang Q AD - Department of Gastroenterology, Peking University People's Hospital, Beijing 100044, China. FAU - Liu, Yu-lan AU - Liu YL AD - Department of Gastroenterology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Case Reports PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 8N3DW7272P (Cyclophosphamide) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - C-Reactive Protein MH - Cyclophosphamide MH - Diabetes Insipidus/*diagnosis MH - Gangrene/*diagnosis MH - Humans MH - Magnetic Resonance Imaging MH - Male MH - Pituitary Gland MH - Vasculitis/*diagnosis EDAT- 2015/12/19 06:00 MHDA- 2016/02/03 06:00 CRDT- 2015/12/19 06:00 PHST- 2015/12/19 06:00 [entrez] PHST- 2015/12/19 06:00 [pubmed] PHST- 2016/02/03 06:00 [medline] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2015 Dec 18;47(6):1028-30. PMID- 20087791 OWN - NLM STAT- MEDLINE DCOM- 20110805 LR - 20211020 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 40 IP - 2 DP - 2011 Apr TI - The pathogenesis of systemic sclerosis revisited. PG - 92-103 LID - 10.1007/s12016-009-8193-3 [doi] AB - Although infectiological stimuli, environmental factors and genotypic features are known to contribute to the initiation and perpetuation of systemic sclerosis (SSc), its etiology still remains to be enigmatic, and less elusive insights are to be achieved by ongoing and future investigations. Being characterized, however, as chronic autoimmune disease with excessive collagen accumulation in skin, synovia and visceral organs such as lung, heart, and digestive tract along with obliterating angiopathy, the pathophysiology of SSc can be summarized as being based on imbalances of the cellular and humoral immune system, vascular dysfunction and activation of resident connective tissue cells. A complex interplay between these major components manages to establish and maintain the inability of the vasculature to adequately react to the need for dilatation, constriction and growth of new vessels, to cause the increased deposition of extracellular matrix constituents as well as to facilitate immunological disarrangement. Despite parallels to the chicken and egg causality dilemma, all of these account for what later clinicians observe in patients suffering from Raynaud's phenomenon, digital ulcers, sclerodactyly, rigidity of the face, microstomia, sicca syndrome, dyspnea, dry cough, pulmonary hypertension, palpitations, syncopes, renal insufficiency, dysphagia, gastroesophageal reflux, dyspepsia, generalized arthralgias, but also dyspareunia, or erectile dysfunction. FAU - Geyer, Matthias AU - Geyer M AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 SB - IM MH - Animals MH - Autoimmunity MH - Fibrosis/physiopathology MH - Humans MH - Inflammation MH - Lymphocytes/immunology MH - Scleroderma, Systemic/etiology/*physiopathology MH - Vascular Diseases/pathology EDAT- 2010/01/21 06:00 MHDA- 2011/08/06 06:00 CRDT- 2010/01/21 06:00 PHST- 2010/01/21 06:00 [entrez] PHST- 2010/01/21 06:00 [pubmed] PHST- 2011/08/06 06:00 [medline] AID - 10.1007/s12016-009-8193-3 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2011 Apr;40(2):92-103. doi: 10.1007/s12016-009-8193-3. PMID- 32100225 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230919 IS - 2193-8237 (Print) IS - 2193-651X (Electronic) VI - 9 IP - 1 DP - 2020 Jun TI - Injection Techniques for Common Chronic Pain Conditions of the Hand: A Comprehensive Review. PG - 129-142 LID - 10.1007/s40122-020-00158-4 [doi] AB - INTRODUCTION: This compilation presents a comprehensive review of the literature on common chronic pain conditions of the hand. It briefly presents these common conditions with their biological background, diagnosis, and common management options. It then presents and compares the latest literature available for injection techniques to treat these diagnoses and compares the available evidence. METHODS: A comprehensive literature review was performed in MEDLINE, PubMed, and Cochrane databases from 1996 to 2019 using the terms "hand pain", "injection techniques", "steroid injection", "chronic pain", "osteoarthritis", "rheumatoid arthritis", "carpal tunnel syndrome", "De Quervain's tenosynovitis", "ganglion cyst", "gout", "Raynaud's", and "stenosing tenosynovitis". RESULTS: Hand pain is a common condition with 9.7% prevalence in men and 21.6% in women and can cause significant morbidity and disability. It also carries a significant cost to the individuals and the healthcare system, totaling in $4 billion dollars in 2003. Injection therapy is an alternative when conservative treatment fails. Osteoarthritis is the most common chronic hand pain syndrome and affects about 16% of the population. Its mechanism is largely mechanic, and as such, there is controversy if steroid injections are of benefit. Hyaluronic acid (HA) appears to provide substantial relief of pain and may increase functionality. More studies of HA are required to make a definite judgment on its efficacy. Similarly, steroid ganglion cyst injection may confer little benefit. Carpal tunnel syndrome is a compressive neuropathy, and only temporarily relieved with injection therapy. US-guidance provides significant improvement and, while severe cases may still require surgery, can provide a valuable bridge therapy to surgery when conservative treatment fails. Similar bridging treatments and increased efficacy under US-guidance are effective for stenosing tenosynovitis ("trigger finger"), though, interestingly, inflammatory background is associated with decreased effect in this case. When the etiology of the pain is inflammatory, such as in RA, corticosteroid (CS) injections provide significant pain relief and increased functionality. They do not, however, change the course of disease (unlike DMARDs). Another such example is De-Quervain tenosynovitis that sees good benefit from CS injections, and an increased efficacy with US-guidance, and similarly are CS injections for gout. For Raynaud's phenomenon, Botox injections have encouraging results, but more studies are needed to determine safety and efficacy, as well as the possible difference in effect between primary and secondary Raynaud's. CONCLUSIONS: Chronic hand pain is a prevalent and serious condition and can cause significant morbidity and disability and interferes with independence and activities of daily living. Conservative treatment remains the first line of treatment; however, when first-line treatments fail, steroid injections can usually provide benefit. In some cases, HA or Botox may also be beneficial. US-guidance is increasing in hand injection and almost ubiquitously provides safer, more effective injections. Hand surgery remains the alternative for refractory pain. FAU - Urits, Ivan AU - Urits I AUID- ORCID: 0000-0002-3652-6085 AD - Department of Anesthesiology, Critical Care, and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. iurits@bidmc.harvard.edu. FAU - Smoots, Daniel AU - Smoots D AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, NE, USA. FAU - Anantuni, Lekha AU - Anantuni L AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, NE, USA. FAU - Bandi, Prudhvi AU - Bandi P AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, NE, USA. FAU - Bring, Katie AU - Bring K AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, NE, USA. FAU - Berger, Amnon A AU - Berger AA AD - Department of Anesthesiology, Critical Care, and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. FAU - Kassem, Hisham AU - Kassem H AD - Department of Anesthesiology, Mount Sinai Medical Center, Miami Beach, FL, USA. FAU - Ngo, Anh L AU - Ngo AL AD - Department of Pain Medicine, Pain Specialty Group, Newington, NH, USA. AD - Harvard Medical School, Boston, MA, USA. FAU - Abd-Elsayed, Alaa AU - Abd-Elsayed A AD - Department of Anesthesiology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA. FAU - Manchikanti, Laxmaiah AU - Manchikanti L AD - Pain Management Centers of America, Paducah, KY, USA. FAU - Urman, Richard AU - Urman R AD - Department of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Boston, MA, USA. FAU - Kaye, Alan D AU - Kaye AD AD - Department of Anesthesiology, Louisiana State University Health Shreveport, Shreveport, LA, USA. FAU - Viswanath, Omar AU - Viswanath O AD - Department of Anesthesiology, Creighton University School of Medicine, Omaha, NE, USA. AD - Valley Anesthesiology and Pain Consultants-Envision Physician Services, Phoenix, AZ, USA. AD - Department of Anesthesiology, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA. LA - eng PT - Journal Article PT - Review DEP - 20200225 PL - New Zealand TA - Pain Ther JT - Pain and therapy JID - 101634491 EIN - Pain Ther. 2020 Jun;9(1):143-144. doi: 10.1007/s40122-020-00164-6. PMID: 32236888 PMC - PMC7203307 OTO - NOTNLM OT - Carpal tunnel syndrome OT - Chronic pain OT - De Quervain’s tenosynovitis OT - Ganglion cyst OT - Gout OT - Hand pain OT - Injection techniques OT - Osteoarthritis OT - Raynaud’s OT - Rheumatoid arthritis OT - Stenosing tenosynovitis OT - Steroid injection EDAT- 2020/02/27 06:00 MHDA- 2020/02/27 06:01 PMCR- 2020/02/25 CRDT- 2020/02/27 06:00 PHST- 2020/01/14 00:00 [received] PHST- 2020/02/27 06:00 [pubmed] PHST- 2020/02/27 06:01 [medline] PHST- 2020/02/27 06:00 [entrez] PHST- 2020/02/25 00:00 [pmc-release] AID - 10.1007/s40122-020-00158-4 [pii] AID - 158 [pii] AID - 10.1007/s40122-020-00158-4 [doi] PST - ppublish SO - Pain Ther. 2020 Jun;9(1):129-142. doi: 10.1007/s40122-020-00158-4. Epub 2020 Feb 25. PMID- 36523670 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221222 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 11 DP - 2022 Nov TI - Cirrhosis of the Liver: A Case Report and Literature Review of a Rare Case Presentation of Autoimmune Hepatitis With Systemic Sclerosis. PG - e31147 LID - 10.7759/cureus.31147 [doi] LID - e31147 AB - Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, heart, lungs, kidneys, gastrointestinal tract, and musculoskeletal system. Although gastrointestinal involvement has been reported in approximately 90% of scleroderma patients, liver involvement is uncommon. A 51-year-old female was admitted to the hospital due to abdominal distension and pedal edema. She had a history of Raynaud's syndrome and multiple hypopigmented and hyperpigmented patches over her body for the last year. Her ascetic fluid analysis was transudative with a serum ascites albumin gradient >1.1, and the abdomen and pelvis ultrasonography reported liver cirrhosis with splenomegaly with perisplenic varices. Her antinuclear antibody and anti-centromere antibody were positive. Skin thickening was visible. Her alanine aminotransferase (ALT), aspartate aminotransferase (AST), and serum globulin were raised. Viral serology was negative. We managed her with diuretics, beta-blockers, prednisolone (30 mg/day administered orally), angiotensin-converting enzyme inhibitors, and calcium channel blockers. Edema and abdominal distension decreased with this management, and no Raynaud's phenomenon was observed during the hospital stay. CI - Copyright © 2022, Banait et al. FAU - Banait, Shashank AU - Banait S AD - Department of Ophthalmology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND. FAU - Burriwar, Chetan AU - Burriwar C AD - Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, IND. FAU - Jain, Jyoti AU - Jain J AD - Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, IND. FAU - Verma, Priti G AU - Verma PG AD - Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND. FAU - Banait, Tanvi AU - Banait T AD - Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND. FAU - Joshi, Madhura AU - Joshi M AD - Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20221106 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9746529 OTO - NOTNLM OT - anticentromere antibody OT - antinuclear antibody OT - autoimmune hepatitis OT - cirrhosis of the liver OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2022/12/17 06:00 MHDA- 2022/12/17 06:01 PMCR- 2022/11/06 CRDT- 2022/12/16 02:25 PHST- 2022/10/07 00:00 [received] PHST- 2022/10/30 00:00 [accepted] PHST- 2022/12/16 02:25 [entrez] PHST- 2022/12/17 06:00 [pubmed] PHST- 2022/12/17 06:01 [medline] PHST- 2022/11/06 00:00 [pmc-release] AID - 10.7759/cureus.31147 [doi] PST - epublish SO - Cureus. 2022 Nov 6;14(11):e31147. doi: 10.7759/cureus.31147. eCollection 2022 Nov. PMID- 39996181 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250226 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 1 DP - 2025 Jan TI - Erasmus Syndrome: Diffuse Systemic Sclerosis With Silicosis. PG - e77900 LID - 10.7759/cureus.77900 [doi] LID - e77900 AB - This report describes the case of a 30-year-old man with a history of prolonged occupational exposure to silica and coal dust leading to the development of Erasmus syndrome, a rare condition of silicosis associated with autoimmune disease, i.e., systemic sclerosis, in a short period. The patient was a laborer in the mining and construction industry, and he presented within a decade of occupational exposure with progressive dyspnea, chronic cough, skin thickening, inflammatory polyarthritis, and Raynaud's phenomenon. Clinical, radiological, and serological tests revealed "accelerated silicosis-associated diffuse systemic sclerosis". Treatment involved stoppage of occupational exposure to silica and symptomatic and immunosuppressive therapy. The case highlights the significance of occupational silica exposure in the development of autoimmune diseases and stresses the need for early intervention, the initiation of protective measures, and the strict monitoring of permissible silica exposure by regulatory authorities. CI - Copyright © 2025, Kumar et al. FAU - Kumar, Rajesh AU - Kumar R AD - Internal Medicine, All India Institute of Medical Sciences, Deoghar, IND. FAU - Kumaran, Ruthra AU - Kumaran R AD - Internal Medicine, All India Institute of Medical Sciences, Deoghar, IND. FAU - Manchem, Shivani AU - Manchem S AD - Internal Medicine, All India Institute of Medical Sciences, Deoghar, IND. FAU - Kumar, Chandan AU - Kumar C AD - Internal Medicine, All India Institute of Medical Sciences, Deoghar, IND. FAU - Sahoo, Manoranjan AU - Sahoo M AD - Internal Medicine, All India Institute of Medical Sciences, Deoghar, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20250124 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11848243 OTO - NOTNLM OT - coal worker pneumoconiosis OT - erasmus syndrome OT - occupational diseases OT - silica exposure OT - silicosis OT - systemic sclerosis COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/02/25 06:21 MHDA- 2025/02/25 06:22 PMCR- 2025/01/24 CRDT- 2025/02/25 04:46 PHST- 2025/01/23 00:00 [accepted] PHST- 2025/02/25 06:22 [medline] PHST- 2025/02/25 06:21 [pubmed] PHST- 2025/02/25 04:46 [entrez] PHST- 2025/01/24 00:00 [pmc-release] AID - 10.7759/cureus.77900 [doi] PST - epublish SO - Cureus. 2025 Jan 24;17(1):e77900. doi: 10.7759/cureus.77900. eCollection 2025 Jan. PMID- 28831927 OWN - NLM STAT- MEDLINE DCOM- 20180730 LR - 20181202 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 161 DP - 2017 TI - [Necrotic toes in a female patient using interferon]. PG - D1523 AB - BACKGROUND: Interferon has an important role in treatment of viral hepatitis, multiple sclerosis and solid and non-solid tumours. Ischaemia and necrosis of the extremities are relatively little-known adverse effects of treatment with interferon. CASE DESCRIPTION: A 44-year-old woman was treated with interferon-beta for relapsing-remitting multiple sclerosis. She developed ischaemia and necrosis of the right lower extremity. Extensive laboratory and imaging investigations offered no clear diagnosis, leading to suspicion of a connexion with interferon-beta treatment. Discontinuation of interferon lead to rapid healing, clearly suggesting a relationship between interferon and the vascular complications. CONCLUSION: Vascular complications during treatment with interferon seem to arise fairly frequently, and can even occur many years after commencing therapy. When vascular symptoms similar to Raynaud's syndrome develop, careful monitoring of the disease course and treatment with vasodilators if required is important. If this is insufficiently effective, interferon should be discontinued. Prompt recognition can prevent significant morbidity in these patients. FAU - Auping, T J R M AU - Auping TJRM AD - Ziekenhuis Gelderse Vallei, Ede. FAU - Maat, P AU - Maat P FAU - van der Waal, K AU - van der Waal K FAU - Bemelmans, R H H AU - Bemelmans RHH LA - dut PT - Case Reports PT - Journal Article TT - Een interferongebruikster met necrose aan de tenen. PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - 0 (Antiviral Agents) RN - 77238-31-4 (Interferon-beta) RN - 9008-11-1 (Interferons) SB - IM MH - Adult MH - Antiviral Agents MH - Female MH - Humans MH - Interferon-beta/*adverse effects/therapeutic use MH - Interferons/*adverse effects MH - Multiple Sclerosis, Relapsing-Remitting/drug therapy MH - Necrosis/*chemically induced MH - *Toes EDAT- 2017/08/24 06:00 MHDA- 2018/07/31 06:00 CRDT- 2017/08/24 06:00 PHST- 2017/08/24 06:00 [entrez] PHST- 2017/08/24 06:00 [pubmed] PHST- 2018/07/31 06:00 [medline] PST - ppublish SO - Ned Tijdschr Geneeskd. 2017;161:D1523. PMID- 25962303 OWN - NLM STAT- MEDLINE DCOM- 20150612 LR - 20150512 IS - 0042-4625 (Print) IS - 0042-4625 (Linking) VI - 174 IP - 1 DP - 2015 TI - [Surgical treatment of superior thoracic outlet syndrome]. PG - 78-83 AB - The authors present immediate and long-term results of treatment of 117 patients with superior thoracic outlet syndrome (STOS). There were different reasons for compression of neu- rovascular fascicle in outlet of the thorax. The costaclavicular syndrome was a reason in 48 patients, additional cervical ribs had 36 patients. Skalenus syndrome was noted in 26 cases, rudimentary cervical ribs or hypertrophy of cervical vertebrae C7 had 7 patients. Raynaud's syndrome took place in 19 cases. The required volume of diagnostic procedures and surgical treatment of STOS were determined according to the cause of the syndrome. Differentiated approach to the different forms of STOS was used in relation to dominant symptoms of the disease and reasons for compression of neurovascular fascicle. This allowed getting positive results in majority of patients (90,4%) in long- term period. FAU - Gaibov, A D AU - Gaibov AD FAU - Kakhorov, A Z AU - Kakhorov AZ FAU - Sadriev, O N AU - Sadriev ON FAU - Yunusov, Kh A AU - Yunusov KhA LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Vestn Khir Im I I Grek JT - Vestnik khirurgii imeni I. I. Grekova JID - 0411377 SB - IM MH - Adult MH - Cervical Vertebrae/surgery MH - Decompression, Surgical/*methods MH - Female MH - Humans MH - Male MH - *Postoperative Complications/etiology/surgery MH - Ribs/*surgery MH - Sympathectomy/*methods MH - *Thoracic Outlet Syndrome/diagnosis/physiopathology/surgery MH - Thoracic Vertebrae/surgery MH - Treatment Outcome EDAT- 2015/05/13 06:00 MHDA- 2015/06/13 06:00 CRDT- 2015/05/13 06:00 PHST- 2015/05/13 06:00 [entrez] PHST- 2015/05/13 06:00 [pubmed] PHST- 2015/06/13 06:00 [medline] PST - ppublish SO - Vestn Khir Im I I Grek. 2015;174(1):78-83. PMID- 24429047 OWN - NLM STAT- MEDLINE DCOM- 20150221 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2014 DP - 2014 Jan 15 TI - Acute blue finger: a diagnostic challenge. LID - 10.1136/bcr-2013-200290 [doi] LID - bcr2013200290 AB - The management of the acute blue finger is controversial with many regarding it as a benign condition. However, we would argue that it should always be considered as an emergency. We present a challenging case of a 43-year-old woman who presented with a 1-week history of sudden onset blue discolouration of the left fifth digit, and a 6-week history of episodic joint problems. Examination showed bilateral normal radial and ulnar pulses. Following blood investigations, an initial working diagnosis of early rheumatoid arthritis with associated Raynaud's phenomenon was made. Also, infective endocarditis was considered due to temporary misleading physical signs. Later, CT angiography of the left upper limb arteries showed a significant proximal left subclavian stenosis. Subsequently, a diagnosis of the left subclavian arteritis associated with digit ischaemia from embolic debris was made and the patient underwent a left subclavian angioplasty. However, delayed management resulted in a necrotic digit, which was left to autoamputate. FAU - Farag, Mohamed AU - Farag M AD - Department of General Medicine, Diana Princess of Wales Hospital, Grimsby, North East Lincolnshire, UK. FAU - Elmasry, Mohamed AU - Elmasry M FAU - Mabote, Thato AU - Mabote T FAU - Elsayed, Ayman AU - Elsayed A FAU - Sunthareswaran, Rame AU - Sunthareswaran R LA - eng PT - Case Reports PT - Journal Article DEP - 20140115 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Acute Disease MH - Adult MH - Angioplasty MH - Arteritis/*complications/diagnosis MH - Arthralgia/etiology MH - Cyanosis/etiology MH - Embolism/*complications/diagnosis MH - Female MH - Fingers/*blood supply MH - Humans MH - Ischemia/*etiology MH - Subclavian Steal Syndrome/*complications/diagnosis PMC - PMC3902337 EDAT- 2014/01/17 06:00 MHDA- 2015/02/24 06:00 PMCR- 2016/01/15 CRDT- 2014/01/17 06:00 PHST- 2014/01/17 06:00 [entrez] PHST- 2014/01/17 06:00 [pubmed] PHST- 2015/02/24 06:00 [medline] PHST- 2016/01/15 00:00 [pmc-release] AID - bcr-2013-200290 [pii] AID - 10.1136/bcr-2013-200290 [doi] PST - epublish SO - BMJ Case Rep. 2014 Jan 15;2014:bcr2013200290. doi: 10.1136/bcr-2013-200290. PMID- 22249306 OWN - NLM STAT- MEDLINE DCOM- 20140306 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 8 DP - 2013 Aug TI - Coexistence of Crohn's disease in a patient with systemic lupus erythematosus. PG - 2145-8 LID - 10.1007/s00296-011-2357-1 [doi] AB - The concurrence of inflammatory bowel disease with systemic lupus erythematosus (SLE) is rare. The concomitant diagnosis of Crohn's disease and SLE is even more rare. The patient, a 40-year-old woman, was admitted to our hospital because of relapsing episodes of abdominal pain, diarrheas upper and lower extremities arthralgias, Raynaud's phenomenon with positive antinuclear antibodies, and fever for the last 2 years. The patient was diagnosed elsewhere with SLE and treated with hydroxychloroquine. Her medical history also included tonsillectomy and total hip replacement after a car accident. Family history was unremarkable. Physical examination was unremarkable except of very mild pain at lower left abdominal quadrant. Laboratory tests showed erythrocyte sedimentation rate at 32 mm/h, C-reactive protein at 36 mg/dl, positive rheumatoid factor, and increased C3, C4, positive antinuclear antibodies with the presence of anti-Sm and anti-RNP antibodies. Ileocolonoscopy revealed colonic inflammation with ulcers and pseudopolyps. Subsequent biopsies were diagnostic of Crohn's disease. Patient was diagnosed with Crohn's colitis concomitant to systemic lupus erythematosus and was started on therapy with azathioprine 2 mg/Kg, methylprednisolone 16 mg/d with slow tapering, mesalazine 1.5 g/day, and hydroxychloroquine. Patient is in excellent health status on the six-month follow-up. FAU - Katsanos, Konstantinos H AU - Katsanos KH AD - 1st Division of Internal Medicine and Hepato-Gastroenterology Unit, University of Ioannina, PO Box 1186, 45110 Ioannina, Greece. FAU - Voulgari, Paraskevi V AU - Voulgari PV FAU - Goussia, Anna AU - Goussia A FAU - Oikonomou, Panagiotis AU - Oikonomou P FAU - Christodoulou, Dimitrios K AU - Christodoulou DK FAU - Drosos, Alexandros A AU - Drosos AA FAU - Tsianos, Epameinondas V AU - Tsianos EV LA - eng PT - Case Reports PT - Journal Article DEP - 20120115 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Crohn Disease/*complications/*diagnosis/pathology MH - Female MH - Humans MH - Intestinal Mucosa/pathology MH - Intestine, Small/*pathology MH - Lupus Erythematosus, Systemic/*complications/pathology EDAT- 2012/01/18 06:00 MHDA- 2014/03/07 06:00 CRDT- 2012/01/18 06:00 PHST- 2011/10/09 00:00 [received] PHST- 2011/12/22 00:00 [accepted] PHST- 2012/01/18 06:00 [entrez] PHST- 2012/01/18 06:00 [pubmed] PHST- 2014/03/07 06:00 [medline] AID - 10.1007/s00296-011-2357-1 [doi] PST - ppublish SO - Rheumatol Int. 2013 Aug;33(8):2145-8. doi: 10.1007/s00296-011-2357-1. Epub 2012 Jan 15. PMID- 40336182 OWN - NLM STAT- MEDLINE DCOM- 20250508 LR - 20250515 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 26 DP - 2025 May 8 TI - A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association. PG - e946827 LID - 10.12659/AJCR.946827 [doi] LID - e946827 AB - BACKGROUND Undifferentiated connective tissue disease (UCTD) is a heterogeneous autoimmune condition characterized by clinical features of connective tissue involvement without meeting the full classification criteria for a defined connective tissue disease (CTD). It often includes arthralgias, Raynaud's phenomenon, and serologic abnormalities. In rare cases, UCTD presents with features resembling relapsing polychondritis, raising questions about a possible overlap syndrome or a shared immunopathogenic mechanism. This case report describes an atypical presentation of UCTD with bilateral auricular inflammation and its therapeutic management. CASE REPORT A 45-year-old man diagnosed with UCTD initially presented with acral cyanosis and inflammatory arthralgias, which responded well to hydroxychloroquine and low-dose prednisone. Over time, he developed progressive bilateral auricular pain, erythema, and nodular swelling, mimicking polychondritis. In the absence of systemic features of relapsing polychondritis, this was considered an unusual manifestation within the UCTD spectrum rather than a distinct overlap syndrome. His symptoms improved significantly following an increase in corticosteroid therapy. CONCLUSIONS This case highlights the importance of recognizing atypical inflammatory manifestations in UCTD and adjusting treatment accordingly. The findings suggest auricular inflammation is part of the broader UCTD spectrum rather than a separate disease entity. These observations underscore the role of shared autoimmune pathways in connective tissue disorders and the need for individualized treatment approaches. FAU - Nigro, Angelo AU - Nigro A AUID- ORCID: 0009-0001-0802-6825 AD - Department of Rheumatology of Lucania, UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Santarcangelo, Pasquale AU - Santarcangelo P AD - Department of Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Bonelli, Antonio AU - Bonelli A AD - Department of Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Digregorio, Serena AU - Digregorio S AD - Department of Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. FAU - Nicoletti, Giuseppe AU - Nicoletti G AD - Department of Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20250508 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - Humans MH - Male MH - Middle Aged MH - *Polychondritis, Relapsing/diagnosis MH - *Undifferentiated Connective Tissue Diseases/diagnosis/complications/drug therapy MH - Diagnosis, Differential MH - Ear Auricle PMC - PMC12070814 COIS- Conflict of interest: None declared EDAT- 2025/05/08 11:14 MHDA- 2025/05/08 12:24 PMCR- 2025/05/08 CRDT- 2025/05/08 01:22 PHST- 2025/05/08 12:24 [medline] PHST- 2025/05/08 11:14 [pubmed] PHST- 2025/05/08 01:22 [entrez] PHST- 2025/05/08 00:00 [pmc-release] AID - 946827 [pii] AID - 10.12659/AJCR.946827 [doi] PST - epublish SO - Am J Case Rep. 2025 May 8;26:e946827. doi: 10.12659/AJCR.946827. PMID- 25747608 OWN - NLM STAT- MEDLINE DCOM- 20161031 LR - 20161230 IS - 2185-4610 (Electronic) IS - 0016-2590 (Linking) VI - 60 IP - 2 DP - 2014 TI - A case of a child with sle presenting with hps as a primary manifestation. PG - 181-6 LID - 10.5387/fms.2013-24 [doi] AB - The primary manifestations of systemic lupus erythematosus (SLE) are various. One such manifestation is hemophagocytic syndrome (HPS). We here report a child with SLE presenting with HPS as a primary manifestation. In October 2010, an 11-year-old Japanese boy presented with pancytopenia, elevated liver enzymes, hyperferritinemia and hemophagocytosis due to macrophages in the bone marrow, and was diagnosed with HPS. A year later, he was found to have proteinuria and hematuria. Oral aphtha and Raynaud's phenomenon were observed, and the patient showed low serum complement levels and was positive for anti-nuclear antibodies (ANAs). He was subsequently diagnosed with SLE. Moreover, low serum complement levels and ANA positivity were detected in a serum sample preserved at the onset of HPS. The HPS was considered to be a primary manifestation of SLE on the basis of these findings. Based on this case, the presence of an underlying disease, such as SLE, should be investigated in cases of HPS. FAU - Ono, Atsushi AU - Ono A AD - Department of Pediatrics, Fukushima Medical University School of Medicine. FAU - Kawasaki, Yukihiko AU - Kawasaki Y FAU - Kanno, Syuto AU - Kanno S FAU - Ohara, Shinichiro AU - Ohara S FAU - Sakai, Nobuko AU - Sakai N FAU - Suyama, Kazuhide AU - Suyama K FAU - Hosoya, Mitsuaki AU - Hosoya M LA - eng PT - Case Reports PT - Journal Article DEP - 20141220 PL - Japan TA - Fukushima J Med Sci JT - Fukushima journal of medical science JID - 0374626 SB - IM MH - Child MH - Delayed Diagnosis MH - Humans MH - Lupus Erythematosus, Systemic/*complications/*diagnosis MH - Lymphohistiocytosis, Hemophagocytic/*diagnosis/*etiology MH - Male MH - Time Factors EDAT- 2015/03/10 06:00 MHDA- 2016/11/01 06:00 CRDT- 2015/03/10 06:00 PHST- 2015/03/10 06:00 [entrez] PHST- 2015/03/10 06:00 [pubmed] PHST- 2016/11/01 06:00 [medline] AID - 10.5387/fms.2013-24 [doi] PST - ppublish SO - Fukushima J Med Sci. 2014;60(2):181-6. doi: 10.5387/fms.2013-24. Epub 2014 Dec 20. PMID- 23775467 OWN - NLM STAT- MEDLINE DCOM- 20140310 LR - 20211021 IS - 1534-6285 (Electronic) IS - 1527-2737 (Linking) VI - 14 IP - 4 DP - 2013 Aug TI - Daily dosing of PDE5 inhibitors: where does it fit in? PG - 269-78 LID - 10.1007/s11934-013-0342-9 [doi] AB - To provide a critical contemporary review of daily PDE5-inhibitor (PDE5-I) use in urological and nonurological conditions. PDE5-Is can be taken up to once a day. However, at present only tadalafil is approved for use in both erectile dysfunction (ED) and benign prostate hyperplasia (BPH) with lower urinary tract symptoms (LUTS). Evolving research in penile rehabilitation, Peyronie's disease, male infertility, pulmonary arterial hypertension, muscular dystrophy and Raynaud's phenomenon shows these therapeutic areas may also benefit from PDE5i therapy. This review examines the role of chronic PDE5 inhibition in ED, BPH-LUTS and other therapeutic targets which may shape our clinical practice in the years to come. FAU - Lee, King Chien Joe AU - Lee KC AD - Department of Urology, National University Hospital, 1E Kent Ridge Road, NUHS Tower Block, Level 8, Singapore, 119228, Singapore. joe_kc_lee@nuhs.edu.sg FAU - Brock, Gerald B AU - Brock GB LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Urol Rep JT - Current urology reports JID - 100900943 RN - 0 (Carbolines) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 742SXX0ICT (Tadalafil) SB - IM MH - Carbolines/*administration & dosage MH - Erectile Dysfunction/*drug therapy MH - Humans MH - Lower Urinary Tract Symptoms/*drug therapy/etiology MH - Male MH - Phosphodiesterase 5 Inhibitors/*administration & dosage MH - Prostatic Hyperplasia/complications/*drug therapy MH - Tadalafil EDAT- 2013/06/19 06:00 MHDA- 2014/03/13 06:00 CRDT- 2013/06/19 06:00 PHST- 2013/06/19 06:00 [entrez] PHST- 2013/06/19 06:00 [pubmed] PHST- 2014/03/13 06:00 [medline] AID - 10.1007/s11934-013-0342-9 [doi] PST - ppublish SO - Curr Urol Rep. 2013 Aug;14(4):269-78. doi: 10.1007/s11934-013-0342-9. PMID- 20981315 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1687-9279 (Electronic) IS - 1687-9260 (Print) IS - 1687-9260 (Linking) VI - 2010 DP - 2010 TI - Penile involvement in Systemic Sclerosis: New Diagnostic and Therapeutic Aspects. PG - 708067 LID - 10.1155/2010/708067 [doi] LID - 708067 AB - Systemic Sclerosis (SSc) is a connective tissue disorder featuring vascular alterations and an immunological activation leading to a progressive and widespread fibrosis of several organs such as the skin, lung, gastrointestinal tract, heart, and kidney. Men with SSc are at increased risk of developing erectile dysfunction (ED) because of the evolution of early microvascular tissutal damage into corporeal fibrosis. The entity of penile vascular damage in SSc patients has been demonstrated by using Duplex ultrasonography and functional infra-red imaging and it is now clear that this is a true clinical entity invariably occurring irrespective of age and disease duration and constituting the ''sclerodermic penis". Once-daily phosphodiesterase type-5 (PDE5) inhibitors improve both sexual function and vascular measures of cavernous arteries by improving surrogate markers of endothelial dysfunction, that is, plasma endothelin-1 and adrenomedullin levels, which may play a potential role in preventing progression of penile fibrosis and ED. Also, the beneficial effect of long-term PDE5i add-on therapy to SSc therapy in the treatment of Raynaud's phenomenon is described. FAU - Aversa, Antonio AU - Aversa A AD - Department of Experimental Medicine, Internal Medicine Unit, Università degli Studi di Roma 'La Sapienza', 00161 Rome, Italy. FAU - Bruzziches, Roberto AU - Bruzziches R FAU - Francomano, Davide AU - Francomano D FAU - Rosato, Edoardo AU - Rosato E FAU - Salsano, Felice AU - Salsano F FAU - Spera, Giovanni AU - Spera G LA - eng PT - Journal Article DEP - 20101005 PL - United States TA - Int J Rheumatol JT - International journal of rheumatology JID - 101519204 PMC - PMC2958513 EDAT- 2010/10/29 06:00 MHDA- 2010/10/29 06:01 PMCR- 2010/10/05 CRDT- 2010/10/29 06:00 PHST- 2010/05/10 00:00 [received] PHST- 2010/06/22 00:00 [revised] PHST- 2010/07/27 00:00 [accepted] PHST- 2010/10/29 06:00 [entrez] PHST- 2010/10/29 06:00 [pubmed] PHST- 2010/10/29 06:01 [medline] PHST- 2010/10/05 00:00 [pmc-release] AID - 10.1155/2010/708067 [doi] PST - ppublish SO - Int J Rheumatol. 2010;2010:708067. doi: 10.1155/2010/708067. Epub 2010 Oct 5. PMID- 36013346 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220830 IS - 2075-1729 (Print) IS - 2075-1729 (Electronic) IS - 2075-1729 (Linking) VI - 12 IP - 8 DP - 2022 Jul 31 TI - Nailfold Videocapillaroscopy for the Evaluation of Peripheral Microangiopathy in Rheumatoid Arthritis. LID - 10.3390/life12081167 [doi] LID - 1167 AB - Rheumatoid arthritis (RA) is a chronic and refractory autoimmune joint disease that affects multiple organs. Several methods have been applied for the study of microvascular endothelial dysfunction, which is considered an important component of vascular disease in RA. Implementation of nailfold videocapillaroscopy (NVC) represents a viable choice, as the skin is an easily accessible window for the non-invasive, real-time assessment of subtle microcirculation abnormalities. Although NVC is routinely used in the rheumatology field, especially for the diagnostic workout of Raynaud's phenomenon, accumulating evidence suggests a role in the evaluation of systemic vasculopathy associated with autoimmune rheumatic disorders. The current paper aims to provide an overview of NVC as a valuable clinical aid for the assessment of peripheral microcirculation in RA. Previous studies characterizing the capillaroscopic pattern in RA are summarized, along with associations with disease-related characteristics. Most available reports have mainly focused on the descriptions of non-specific morphological alterations that may reflect endothelial injury over the course of the disease. Still, the exact pattern of structural and functional capillaroscopic alterations and their clinical significance in RA remains a subject of ongoing research. FAU - Anyfanti, Panagiota AU - Anyfanti P AUID- ORCID: 0000-0002-5658-4629 AD - Second Medical Department, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. FAU - Angeloudi, Elena AU - Angeloudi E AUID- ORCID: 0000-0003-3492-7915 AD - Second Medical Department, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. FAU - Dara, Athanasia AU - Dara A AD - Fourth Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. FAU - Arvanitaki, Alexandra AU - Arvanitaki A AUID- ORCID: 0000-0003-3180-7280 AD - Fourth Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. AD - First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, 54621 Thessaloniki, Greece. FAU - Bekiari, Eleni AU - Bekiari E AD - Second Medical Department, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. FAU - Kitas, George D AU - Kitas GD AD - Department of Rheumatology, Russells Hall Hospital, Dudley Group NHS Foundation Trust, Dudley DY1 2HQ, UK. AD - School of Sport, Exercise and Rehabilitation Sciences, University of Birmingham, Birmingham B15 2TT, UK. FAU - Dimitroulas, Theodoros AU - Dimitroulas T AD - Fourth Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece. LA - eng GR - MIS 5047870/P. Anyfanti has received funding from Greece and the European Union (European Social Fund- ESF) through the Operational Programme "Human Resources Development, Education and Lifelong Learning 2014-2020"/ PT - Journal Article PT - Review DEP - 20220731 PL - Switzerland TA - Life (Basel) JT - Life (Basel, Switzerland) JID - 101580444 PMC - PMC9410266 OTO - NOTNLM OT - microcirculation OT - nailfold videocapillaroscopy OT - rheumatoid arthritis COIS- The authors declare no conflict of interest. EDAT- 2022/08/27 06:00 MHDA- 2022/08/27 06:01 PMCR- 2022/07/31 CRDT- 2022/08/26 01:29 PHST- 2022/02/28 00:00 [received] PHST- 2022/06/28 00:00 [revised] PHST- 2022/07/25 00:00 [accepted] PHST- 2022/08/26 01:29 [entrez] PHST- 2022/08/27 06:00 [pubmed] PHST- 2022/08/27 06:01 [medline] PHST- 2022/07/31 00:00 [pmc-release] AID - life12081167 [pii] AID - life-12-01167 [pii] AID - 10.3390/life12081167 [doi] PST - epublish SO - Life (Basel). 2022 Jul 31;12(8):1167. doi: 10.3390/life12081167. PMID- 18625650 OWN - NLM STAT- MEDLINE DCOM- 20081104 LR - 20161124 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 17 IP - 8 DP - 2008 Aug TI - Prevalence and clinical associations of anti-Ku antibodies in systemic autoimmune diseases. PG - 727-32 LID - 10.1177/0961203308089442 [doi] AB - We retrospectively analysed the prevalence and clinical features associated to anti-Ku antibodies in patients affected by different autoimmune diseases. Anti-Ku antibodies are detected in 147 sera out of 7239 anti-ENA positive sera (2%). They are found in 2% of patients with systemic sclerosis (SSc) (8 out of 379), 1.8% of systemic lupus erythematosus (SLE) (7 out of 372) and 1.8% of undifferentiated connective tissue disease (UCTD) (9 out of 496) and more rarely in Sjögren Syndrome and rheumatoid arthritis. Most of anti-Ku positive patients were affected by UCTD and overlap syndromes, including polymyositis, SSc and SLE. Interstitial lung disease, myositis, articular symptoms, Raynaud's phenomenon and sicca represents the main clinical features detected in our cohort. The rate and severity of pulmonary disease is similar to those found in other SSc patients. Isolated anti-Ku were detected in about 47% of sera. No clinical differences were observed between these patients and subjects with multiple anti-nuclear specificities. However, anti-Ku are usually detected in association with other serological markers in SLE and Sjögren Syndrome, while they occurred isolated in SSc and polymyositis. FAU - Cavazzana, I AU - Cavazzana I AD - Rheumatology Unit and Chair, Spedali Civili, Università degli Studi di Brescia, Brescia, Italy. ilariacava@virgilio.it FAU - Ceribelli, A AU - Ceribelli A FAU - Quinzanini, M AU - Quinzanini M FAU - Scarsi, M AU - Scarsi M FAU - Airò, P AU - Airò P FAU - Cattaneo, R AU - Cattaneo R FAU - Franceschini, F AU - Franceschini F LA - eng PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (DNA-Binding Proteins) RN - 0 (anti-extractable nuclear antigen antibodies) RN - EC 3.6.4.12 (Xrcc6 protein, human) RN - EC 4.2.99.- (Ku Autoantigen) SB - IM MH - Antibodies, Antinuclear/*blood/immunology MH - Antigens, Nuclear/*immunology MH - Autoimmune Diseases/diagnosis/*immunology MH - DNA-Binding Proteins/*immunology MH - Dermatomyositis/immunology MH - Female MH - Humans MH - Ku Autoantigen MH - Lupus Erythematosus, Systemic/immunology MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/immunology EDAT- 2008/07/16 09:00 MHDA- 2008/11/05 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2008/11/05 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] AID - 17/8/727 [pii] AID - 10.1177/0961203308089442 [doi] PST - ppublish SO - Lupus. 2008 Aug;17(8):727-32. doi: 10.1177/0961203308089442. PMID- 35794813 OWN - NLM STAT- MEDLINE DCOM- 20220708 LR - 20220802 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 23 DP - 2022 Jul 7 TI - A 25-Year-Old Saudi Woman with a 2-Year History of Antisynthetase Syndrome with Interstitial Lung Disease Who Commenced Azathioprine Treatment in the Third Trimester of Pregnancy and Had a Successful Birth at Term. PG - e936833 LID - 10.12659/AJCR.936833 [doi] AB - BACKGROUND Antisynthetase syndrome (ASS) is a rare systemic autoimmune disease. The clinical features of ASS include interstitial lung disease (ILD), myositis, arthritis, Raynaud's phenomenon, mechanic's hands, and unexplained fever. There is a paucity of reported cases and management guidelines in pregnancy. This report describes the case of a 25-year-old Saudi woman with a 2-year history of ASS with ILD who commenced azathioprine treatment in the third trimester and had a successful birth at term. CASE REPORT A 25-year-old Saudi primigravida woman with a 2-year history of ASS with ILD presented at 26 weeks of gestation after being lost to prepregnancy follow-up and discontinuing her medications. Azathioprine treatment was commenced, and despite poor prepregnancy follow-up, her pregnancy remained uneventful until 39 weeks, when fetal ultrasonography showed oligohydramnios. Therefore, labor induction was initiated, and she delivered vaginally with no postpartum complications or flare-ups. CONCLUSIONS The multisystem autoimmune disease ASS is a rare condition, and there are no clinical guidelines for its management in pregnant women. This case report highlights some aspects of ASS management and the importance of a multidisciplinary approach. FAU - Alshwairikh, Lama A AU - Alshwairikh LA AD - Department of Obstetrics and Gynecology, King Saud University Medical City, Riyadh, Saudi Arabia. FAU - Babay, Zeneb AU - Babay Z AD - Department of Obstetrics and Gynecology, King Saud University Medical City, Riyadh, Saudi Arabia. LA - eng PT - Case Reports PT - Journal Article DEP - 20220707 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 RN - 0 (Autoantibodies) RN - MRK240IY2L (Azathioprine) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Autoantibodies MH - *Autoimmune Diseases/complications MH - Azathioprine/therapeutic use MH - Female MH - Humans MH - *Lung Diseases, Interstitial/complications/drug therapy MH - *Myositis/drug therapy MH - Pregnancy MH - Pregnancy Trimester, Third MH - Saudi Arabia PMC - PMC9274784 COIS- Conflict of interest: None declared EDAT- 2022/07/08 06:00 MHDA- 2022/07/09 06:00 PMCR- 2022/07/07 CRDT- 2022/07/07 01:23 PHST- 2022/07/07 01:23 [entrez] PHST- 2022/07/08 06:00 [pubmed] PHST- 2022/07/09 06:00 [medline] PHST- 2022/07/07 00:00 [pmc-release] AID - 936833 [pii] AID - 10.12659/AJCR.936833 [doi] PST - epublish SO - Am J Case Rep. 2022 Jul 7;23:e936833. doi: 10.12659/AJCR.936833. PMID- 20625308 OWN - NLM STAT- MEDLINE DCOM- 20110211 LR - 20151119 IS - 1558-2035 (Electronic) IS - 1558-2027 (Linking) VI - 11 IP - 12 DP - 2010 Dec TI - Cardiological features in idiopathic inflammatory myopathies. PG - 906-11 LID - 10.2459/JCM.0b013e32833cdca8 [doi] AB - Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of autoimmune systemic diseases characterized by chronic muscle weakness and inflammatory cell infiltrates in skeletal muscle. The most frequent IIMs, such as adult-onset polymyositis and dermatomyositis, display a wide range of clinical manifestations other than myositis, including skin changes, Raynaud's phenomenon and interstitial lung disease. Cardiac involvement is now well recognized as a clinically important manifestation in patients with polymyositis or dermatomyositis, although its actual frequency is still uncertain. Cardiovascular complications represent one of the most frequent causes of death in myositis, apart from cancer and lung involvement. Despite the fact that clinical manifestations are relatively rare, asymptomatic cardiovascular features are frequently reported in patients with polydermatomyositis and dermatomyositis. They are characterized by isolated electrocardiographic changes, valve disease, coronary vasculitis, ischemic abnormalities, heart failure and myocarditis. Chronic inflammation producing myocyte degeneration, tissues fibrosis and vascular alterations can explain the majority of reported cardiac features in myositic patients. Although previous works reported an association between heart involvement and some myositis-specific autoantibodies (namely anti-signal recognition particle), electrocardiography, echocardiography and, where necessary, heart magnetic resonance remain the mainstay for diagnosing and monitoring myocardial inflammation in these diseases. Anyway, a complete multiorgan assessment and a careful analysis of autoantibodies should be performed in every patient in order to define any possible distinct disease entities with different prognosis within the spectrum of IIMs. FAU - Bazzani, Chiara AU - Bazzani C AD - Rheumatology Unit, University of Brescia, Piazzale Spedali Civili, Brescia, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I FAU - Ceribelli, Angela AU - Ceribelli A FAU - Vizzardi, Enrico AU - Vizzardi E FAU - Dei Cas, Livio AU - Dei Cas L FAU - Franceschini, Franco AU - Franceschini F LA - eng PT - Journal Article PT - Review PL - United States TA - J Cardiovasc Med (Hagerstown) JT - Journal of cardiovascular medicine (Hagerstown, Md.) JID - 101259752 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/blood MH - Biomarkers/blood MH - Disease Progression MH - Heart Diseases/diagnosis/*etiology/immunology/physiopathology/therapy MH - Humans MH - Myositis/*complications/diagnosis/immunology/physiopathology/therapy MH - Predictive Value of Tests MH - Prognosis EDAT- 2010/07/14 06:00 MHDA- 2011/02/12 06:00 CRDT- 2010/07/14 06:00 PHST- 2010/07/14 06:00 [entrez] PHST- 2010/07/14 06:00 [pubmed] PHST- 2011/02/12 06:00 [medline] AID - 10.2459/JCM.0b013e32833cdca8 [doi] PST - ppublish SO - J Cardiovasc Med (Hagerstown). 2010 Dec;11(12):906-11. doi: 10.2459/JCM.0b013e32833cdca8. PMID- 18500432 OWN - NLM STAT- MEDLINE DCOM- 20090414 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 27 IP - 11 DP - 2008 Nov TI - Outcome of high titer antinuclear antibody positivity in individuals without connective tissue disease: a 10-year follow-up. PG - 1399-402 LID - 10.1007/s10067-008-0932-y [doi] AB - To ascertain the development of disease in a cohort of connective tissue disease (CTD)-negative patients 10 years after testing positive for antinuclear antibody (ANA) in high titer, a telephone survey and serological tests were conducted on a group of CTD-negative patients with high ANA titer identified in a previous study. Thirty-four out of 62 patients (55%) completed only the telephone survey and 27 completed both. The mean length of follow-up was 11.5 years. The ages ranged from 27 to 89 years with the mean being 58.9 years. The sex distribution included 4 (12%) men and 30 (88%) women. Twenty-one out of 27 patients (78%) remained ANA-positive. A total of five patients were diagnosed with CTD with two in the last 5 years. Common symptoms described, in order of frequency, were joint pain, Raynaud's phenomenon, and Sicca symptoms. This study shows that a considerable percentage of CTD-negative individuals with an initial high ANA titer continue to maintain their positivity even after an extended period. It is reassuring, however, that only a small number of these individuals go on to develop CTD. It confirms the fact that the ANA test, despite remaining a useful clinical tool, needs to be used in conjunction with other evidence and clinical judgement when attempting to validate the diagnosis of CTD. FAU - Wijeyesinghe, Udara AU - Wijeyesinghe U AD - Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada. FAU - Russell, Anthony S AU - Russell AS LA - eng PT - Journal Article DEP - 20080524 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/*blood MH - Connective Tissue Diseases/diagnosis/*immunology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Interviews as Topic MH - Male MH - Middle Aged EDAT- 2008/05/27 09:00 MHDA- 2009/04/15 09:00 CRDT- 2008/05/27 09:00 PHST- 2008/05/02 00:00 [received] PHST- 2008/05/05 00:00 [accepted] PHST- 2008/05/02 00:00 [revised] PHST- 2008/05/27 09:00 [pubmed] PHST- 2009/04/15 09:00 [medline] PHST- 2008/05/27 09:00 [entrez] AID - 10.1007/s10067-008-0932-y [doi] PST - ppublish SO - Clin Rheumatol. 2008 Nov;27(11):1399-402. doi: 10.1007/s10067-008-0932-y. Epub 2008 May 24. PMID- 23292189 OWN - NLM STAT- MEDLINE DCOM- 20140130 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 7 DP - 2013 Jul TI - Rheumatic manifestations of euthyroid, anti-thyroid antibody-positive patients. PG - 1745-52 LID - 10.1007/s00296-012-2616-9 [doi] AB - The aim of this study is to define the rheumatic manifestations of euthyroid patients with chronic lymphocytic thyroiditis (CLT) but without a well-defined connective tissue disease. Forty-six consecutive patients with anti-thyroid peroxidase (αTPO) and/or anti-thyroglobulin antibodies (αTG), and normal thyroid function in the absence of a well-defined connective tissue disease were included in a case-cohort study. Arthralgias were a presenting complaint in 98 % of patients. Fibromyalgia syndrome was found in 59 % of patients. Raynaud's phenomenon occurred in 28 % and sicca symptoms in 26 % of patients. Two patients had seronegative arthritis resembling rheumatoid arthritis. Arthritis was radiographically present in 88 %, affecting the spine in 45 % of patients. Thyroid-stimulating hormone (TSH) levels positively correlated with levels of αTPO, but not with erythrocyte sedimentation rate (ESR) or αTG levels. A positive ANA was found in 24 % of patients. One patient developed subclinical hypothyroidism during the study. Rheumatic manifestations frequently occur in patients with CLT in the absence of overt thyroid dysfunction and mimic the presentation of the well-defined connective tissue diseases. FAU - Tagoe, Clement E AU - Tagoe CE AD - Department of Medicine, Albert Einstein College of Medicine, Bronx, NY, USA. ctagoe@aol.com FAU - Zezon, Anna AU - Zezon A FAU - Khattri, Saakshi AU - Khattri S FAU - Castellanos, Patricia AU - Castellanos P LA - eng PT - Journal Article DEP - 20130105 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) RN - 0 (Iron-Binding Proteins) RN - 0 (anti-thyroglobulin) RN - 0 (thyroid microsomal antibodies) RN - EC 1.11.1.7 (TPO protein, human) RN - EC 1.11.1.8 (Iodide Peroxidase) SB - IM MH - Adult MH - Aged MH - Autoantibodies/*blood MH - Autoantigens/*immunology MH - Biomarkers/blood MH - Connective Tissue Diseases/complications/diagnosis MH - Diagnosis, Differential MH - Female MH - Hashimoto Disease/blood/complications/diagnosis/*immunology MH - Humans MH - Iodide Peroxidase/*immunology MH - Iron-Binding Proteins/*immunology MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Retrospective Studies MH - Rheumatic Diseases/blood/diagnosis/*immunology MH - Thyroid Function Tests MH - Thyroid Gland/enzymology/*immunology MH - Young Adult EDAT- 2013/01/08 06:00 MHDA- 2014/01/31 06:00 CRDT- 2013/01/08 06:00 PHST- 2012/07/16 00:00 [received] PHST- 2012/12/09 00:00 [accepted] PHST- 2013/01/08 06:00 [entrez] PHST- 2013/01/08 06:00 [pubmed] PHST- 2014/01/31 06:00 [medline] AID - 10.1007/s00296-012-2616-9 [doi] PST - ppublish SO - Rheumatol Int. 2013 Jul;33(7):1745-52. doi: 10.1007/s00296-012-2616-9. Epub 2013 Jan 5. PMID- 31143345 OWN - NLM STAT- MEDLINE DCOM- 20190607 LR - 20200225 IS - 1937-8688 (Electronic) VI - 32 DP - 2019 TI - [Diffuse interstitial lung disease revealing anti-synthetase syndrome: about two cases]. PG - 40 LID - 10.11604/pamj.2019.32.40.17903 [doi] LID - 40 AB - Anti-synthetase syndrome (ASS) is an inflammatory myopathy commonly associated with pulmonary involvement, especially parenchymal (diffuse infiltrative pneumonitis). Extrathoracic manifestations associated with pulmonary involvement can give an indication to the diagnosis: myalgias, polyarthralgias, Raynaud's syndrome, erythematous palmar hyperkeratosis with fissures and fever. Given the suggestive clinical and radiological picture, the presence of aminoacyl-transfer RNA (tRNA) synthetase antibodies enables to confirm, in particular, Anti Jo-1 antibody activity. Pulmonary involvement is a major prognostic factor, hence the indication for intensive immunosuppressive therapy based on corticosteroids, immunosuppressive medications or the association among them. A better awareness about this disorder revealed by pulmonary manifestations could enable early and adequate management and to improve patient's prognosis. FAU - Bouardi, Nizar El AU - Bouardi NE AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Alaoui, Amina AU - Alaoui A AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Haloua, Meriem AU - Haloua M AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Lamrani, Youssef AU - Lamrani Y AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Boubbou, Meryem AU - Boubbou M AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Maaroufi, Mustapha AU - Maaroufi M AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. FAU - Alami, Baderdine AU - Alami B AD - Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc. AD - Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc. LA - fre PT - Case Reports PT - Journal Article TT - Une pneumopathie interstitielle diffuse révélant un syndrome des antisynthétases: à propos de 2 cas. DEP - 20190122 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Adrenal Cortex Hormones/administration & dosage MH - Adult MH - Aged MH - Antibodies, Antinuclear/immunology MH - Female MH - Humans MH - Immunosuppressive Agents/*administration & dosage MH - Lung Diseases, Interstitial/drug therapy/*etiology/physiopathology MH - Male MH - Myositis/*diagnosis/drug therapy/immunology MH - Prognosis PMC - PMC6522153 OTO - NOTNLM OT - Antisynthetases syndrome OT - anti-Jo-1 OT - diffuse infiltrative lung disease OT - prognosis COIS- Les auteurs ne déclarent aucun conflit d´intérêts. EDAT- 2019/05/31 06:00 MHDA- 2019/06/08 06:00 PMCR- 2019/01/22 CRDT- 2019/05/31 06:00 PHST- 2018/12/11 00:00 [received] PHST- 2019/01/15 00:00 [accepted] PHST- 2019/05/31 06:00 [entrez] PHST- 2019/05/31 06:00 [pubmed] PHST- 2019/06/08 06:00 [medline] PHST- 2019/01/22 00:00 [pmc-release] AID - PAMJ-32-40 [pii] AID - 10.11604/pamj.2019.32.40.17903 [doi] PST - epublish SO - Pan Afr Med J. 2019 Jan 22;32:40. doi: 10.11604/pamj.2019.32.40.17903. eCollection 2019. PMID- 23587414 OWN - NLM STAT- MEDLINE DCOM- 20140106 LR - 20161125 IS - 1776-2561 (Electronic) IS - 0761-8417 (Linking) VI - 69 IP - 3 DP - 2013 Jun TI - [Diffuse infiltrative lung disease in scleroderma. Analysis of radio-clinical and functional semiology]. PG - 132-8 LID - S0761-8417(13)00042-4 [pii] LID - 10.1016/j.pneumo.2013.02.003 [doi] AB - Scleroderma (SD) is a systemic disease that predominantly affects the skin. Diffuse infiltrative lung disease (DILD) is rare and occurs most often in the course of the disease. We analyzed seven cases of DILO of SD recorded between 2003 and 2010 among 196 PID (3.6%). Functional signs were limited to respiratory dyspnea, it was associated to dysphagia in six cases, dry syndrome in five cases and Raynaud's phenomenon in four cases. Clinical examination found crackles in the bases of the thorax in all cases and specific cutaneous signs in six cases. The chest radiograph showed that interstitial disease predominates at the lung bases in all cases with a large aspect of the pulmonary arteries in two cases. The chest CT scan confirmed the predominance of basal and peripheral damage with signs of fibrosis in six cases. The pulmonary function objectified a severe restrictive ventilatory defect in all cases. Bronchoscopy showed a normal macroscopic appearance in all cases, the broncho-alveolar lavage was predominated by neutrophilic formula in four cases. SCL 70 antibodies were positive in four cases. All patients were treated by steroids with improvement of dyspnea and stabilization of radiographs. A patient had died in an array of acute respiratory failure and one patient was lost to follow-up. DILD in scleroderma is rare and seldom reveals the disease, it affects the patient's prognosis especially when associated with arterial pulmonary hypertension. CI - Copyright © 2013 Elsevier Masson SAS. All rights reserved. FAU - El Khattabi, W AU - El Khattabi W AD - Service des maladies respiratoires, hôpital du 20-Août, Casablanca, Maroc. welkhattabi@yahoo.fr FAU - Afif, H AU - Afif H FAU - Moussali, N AU - Moussali N FAU - Aichane, A AU - Aichane A FAU - Abdelouafi, A AU - Abdelouafi A FAU - Bouayad, Z AU - Bouayad Z LA - fre PT - Journal Article TT - Pneumopathie infiltrante diffuse (PID) de la sclérodermie. Analyse de la sémiologie radioclinique et fonctionnelle. DEP - 20130412 PL - France TA - Rev Pneumol Clin JT - Revue de pneumologie clinique JID - 8406312 SB - IM MH - Adult MH - Aged MH - Cohort Studies MH - Female MH - Humans MH - Lung Diseases/*diagnostic imaging/*etiology/pathology/*physiopathology MH - Male MH - Middle Aged MH - Radiography, Thoracic MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Diffuse/*complications/diagnostic imaging/pathology/physiopathology EDAT- 2013/04/17 06:00 MHDA- 2014/01/07 06:00 CRDT- 2013/04/17 06:00 PHST- 2012/02/02 00:00 [received] PHST- 2013/01/10 00:00 [revised] PHST- 2013/02/25 00:00 [accepted] PHST- 2013/04/17 06:00 [entrez] PHST- 2013/04/17 06:00 [pubmed] PHST- 2014/01/07 06:00 [medline] AID - S0761-8417(13)00042-4 [pii] AID - 10.1016/j.pneumo.2013.02.003 [doi] PST - ppublish SO - Rev Pneumol Clin. 2013 Jun;69(3):132-8. doi: 10.1016/j.pneumo.2013.02.003. Epub 2013 Apr 12. PMID- 21645423 OWN - NLM STAT- MEDLINE DCOM- 20110922 LR - 20211020 IS - 1746-1596 (Electronic) IS - 1746-1596 (Linking) VI - 6 DP - 2011 Jun 7 TI - Undifferentiated connective tissue disease presenting with prevalent interstitial lung disease: case report and review of literature. PG - 50 LID - 10.1186/1746-1596-6-50 [doi] AB - Undifferentiated connective tissue diseases (UCTDs) are clinical entities characterised by signs and symptoms suggestive of a systemic autoimmune disease, which do not fulfil the diagnostic criteria for a defined connective tissue disease. Lung involvement can complicate the course and management of the disease, often determining a worse outcome. Respiratory dysfunction as the first clinical manifestation has seldom been reported.We describe a case of a female patient who developed significant respiratory dysfunction as the principal clinical sign. Video-assisted thoracoscopy was performed and a histological pattern of nonspecific interstitial pneumonia (NSIP) was found. A pathological diagnosis suggested careful follow-up with extensive immunological screening which then detected Raynaud's phenomenon and positivity of antinuclear antibodies. After a multidisciplinary discussion (pneumologist, radiologist, pathologist and rheumatologist) a final diagnosis of NSIP associated with UCTD was made. The diagnosis of UCTD should be considered when NSIP is diagnosed even in cases with evident first clinical manifestations of severe respiratory dysfunction. A multidisciplinary approach in the field of interstitial lung disease with NSIP, also including rheumatologic expertise, is fundamental to achieve a prompt and correct diagnosis. CI - © 2011 Lunardi et al; licensee BioMed Central Ltd. FAU - Lunardi, Francesca AU - Lunardi F AD - Department of Diagnostic Medical Sciences and Special Therapies, University of Padua, Padua, Italy. FAU - Balestro, Elisabetta AU - Balestro E FAU - Nordio, Beatrice AU - Nordio B FAU - Cozzi, Franco AU - Cozzi F FAU - Polverosi, Roberta AU - Polverosi R FAU - Sfriso, Paolo AU - Sfriso P FAU - Braccioni, Fausto AU - Braccioni F FAU - Calabrese, Fiorella AU - Calabrese F LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20110607 PL - England TA - Diagn Pathol JT - Diagnostic pathology JID - 101251558 SB - IM MH - Connective Tissue Diseases/complications/*diagnosis/physiopathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung/*pathology/physiopathology MH - Lung Diseases, Interstitial/complications/*diagnosis/physiopathology MH - Middle Aged MH - Pleurisy/complications/pathology/physiopathology MH - Radiography, Thoracic MH - Respiratory Function Tests MH - Respiratory Insufficiency/complications/*diagnosis/physiopathology PMC - PMC3126759 EDAT- 2011/06/08 06:00 MHDA- 2011/09/23 06:00 PMCR- 2011/06/07 CRDT- 2011/06/08 06:00 PHST- 2011/04/04 00:00 [received] PHST- 2011/06/07 00:00 [accepted] PHST- 2011/06/08 06:00 [entrez] PHST- 2011/06/08 06:00 [pubmed] PHST- 2011/09/23 06:00 [medline] PHST- 2011/06/07 00:00 [pmc-release] AID - 1746-1596-6-50 [pii] AID - 10.1186/1746-1596-6-50 [doi] PST - epublish SO - Diagn Pathol. 2011 Jun 7;6:50. doi: 10.1186/1746-1596-6-50. PMID- 37174957 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230515 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 13 IP - 9 DP - 2023 Apr 27 TI - Description of Peripheral Blood Perfusion by Laser Speckle Contrast Analysis (LASCA) in 'Early' versus 'Clinically Overt' Systemic Sclerosis in Routine Clinics. LID - 10.3390/diagnostics13091566 [doi] LID - 1566 AB - OBJECTIVE: To investigate in an unselected, systemic sclerosis (SSc) cohort if baseline laser speckle contrast analysis (LASCA) peripheral blood perfusion (PBP) measurements differ between 'early' SSc (without skin involvement, or 'limited' SSc-LSSc) and 'clinically overt' SSc (with skin involvement, limited cutaneous SSc-LcSSc and diffuse cutaneous SSc-DcSSc) in routine setting. METHODS: A group of twenty consecutive 'early' SSc patients and forty consecutive 'clinically overt' SSc patients (twenty LcSSc and twenty DcSSc) underwent clinical and LASCA examinations (to assess the peripheral blood perfusion [PBP] of both hands volar). RESULTS: No statistically significant difference in adjusted PBP was found in the 'early' versus the 'clinically overt' group (p = 0.77) when adjusted for possible confounding factors (e.g., vasoactive medication, active smoking, history of DTL and disease duration). A wide variability was noted when observing the individual datapoints of each subset. CONCLUSION: This study with an unselected SSc population in daily routine, non-research setting, showed there was no difference in adjusted PBP at baseline between 'early' SSc and 'clinically overt' SSc when corrected for possible confounding factors. Interestingly a wide variation of individual datapoints were observed in each subset, which emphasizes the heterogeneity of SSc. FAU - Willems, Seppe AU - Willems S AD - Department of Rheumatology, Ghent University Hospital, 9000 Ghent, Belgium. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, 9000 Ghent, Belgium. AD - Department of Internal Medicine, Ghent University Hospital, 9000 Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, Inflammation Research Center (IRC), Vlaams Instituut voor Biotechnologie (VIB), 9000 Ghent, Belgium. FAU - Wallaert, Steven AU - Wallaert S AD - Biostatistics Unit, Department of Public Health and Primary Care, Ghent University, 9000 Ghent, Belgium. FAU - Gotelli, Emanuele AU - Gotelli E AUID- ORCID: 0000-0002-4732-0306 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, 16132 Genova, Italy. FAU - Du Four, Tessa AU - Du Four T AD - Department of Rheumatology, Ghent University Hospital, 9000 Ghent, Belgium. AD - Department of Internal Medicine, Ghent University Hospital, 9000 Ghent, Belgium. FAU - Wyckstandt, Kaat AU - Wyckstandt K AD - Department of Rheumatology, Ghent University Hospital, 9000 Ghent, Belgium. AD - Department of Internal Medicine, Ghent University Hospital, 9000 Ghent, Belgium. FAU - Cere, Andrea AU - Cere A AUID- ORCID: 0000-0001-7163-1051 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, 16132 Genova, Italy. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, University of Genova, IRCCS San Martino Polyclinic Hospital, 16132 Genova, Italy. LA - eng GR - Research Foundation-Flanders (Belgium) (FWO) [1.8.029.20N]/Research Foundation-Flanders (Belgium) (FWO)/ PT - Journal Article DEP - 20230427 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10177938 OTO - NOTNLM OT - Raynaud’s phenomenon OT - laser speckle contrast analysis (LASCA) OT - systemic sclerosis OT - ‘clinically overt’ SSc OT - ‘early’ SSc COIS- The authors declare no conflict of interest. EDAT- 2023/05/13 15:12 MHDA- 2023/05/13 15:13 PMCR- 2023/04/27 CRDT- 2023/05/13 01:24 PHST- 2023/04/15 00:00 [received] PHST- 2023/04/24 00:00 [revised] PHST- 2023/04/25 00:00 [accepted] PHST- 2023/05/13 15:13 [medline] PHST- 2023/05/13 15:12 [pubmed] PHST- 2023/05/13 01:24 [entrez] PHST- 2023/04/27 00:00 [pmc-release] AID - diagnostics13091566 [pii] AID - diagnostics-13-01566 [pii] AID - 10.3390/diagnostics13091566 [doi] PST - epublish SO - Diagnostics (Basel). 2023 Apr 27;13(9):1566. doi: 10.3390/diagnostics13091566. PMID- 17204309 OWN - NLM STAT- MEDLINE DCOM- 20070719 LR - 20070528 IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 36 IP - 6 DP - 2007 Jun TI - Determinants of pulmonary arterial hypertension in scleroderma. PG - 392-6 AB - OBJECTIVE: To define risk factors associated with pulmonary arterial hypertension (PAH) in a large cohort of patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for PAH by means of Doppler echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded systolic pulmonary artery pressure along with pulmonary function testing, clinical, and laboratory data. PAH was defined as right ventricular systolic pressure equal or greater than 40 mm Hg. RESULTS: Of 114 SSc patients with echocardiographic measurements, PAH was found in 33 (29%) patients. In a multiple logistic regression analysis, the presence of pulmonary fibrosis on thoracic computed tomography (OR 6.78, CI 1.54 to 29.9), forced vital capacity less than 80% predicted (OR 3.03, CI 1.1 to 8.35), and duration of Raynaud's phenomenon preceding the onset of skin changes for at least 3 years (OR 5.75, CI 1.9 to 17.41) were found to be independent predictors of PAH. Age, disease duration, disease subtype, or autoantibodies were not associated with PAH in our patients. CONCLUSIONS: The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. Screening for PAH in these high-risk patients may detect PAH at an earlier stage and guide decisions on therapeutic interventions. FAU - Plastiras, Sotiris C AU - Plastiras SC AD - Department of Pathophysiology, University of Athens School of Medicine, 75 M. Asias Street, Athens 11527, Greece. FAU - Karadimitrakis, Stylianos P AU - Karadimitrakis SP FAU - Kampolis, Christos AU - Kampolis C FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM FAU - Tzelepis, George E AU - Tzelepis GE LA - eng PT - Journal Article DEP - 20070103 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Comorbidity MH - Echocardiography, Doppler MH - Female MH - Greece/epidemiology MH - Humans MH - Hypertension, Pulmonary/*etiology/physiopathology MH - Male MH - Middle Aged MH - Respiratory Function Tests MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications/physiopathology EDAT- 2007/01/06 09:00 MHDA- 2007/07/20 09:00 CRDT- 2007/01/06 09:00 PHST- 2006/06/09 00:00 [received] PHST- 2006/09/25 00:00 [revised] PHST- 2006/10/29 00:00 [accepted] PHST- 2007/01/06 09:00 [pubmed] PHST- 2007/07/20 09:00 [medline] PHST- 2007/01/06 09:00 [entrez] AID - S0049-0172(06)00162-4 [pii] AID - 10.1016/j.semarthrit.2006.10.004 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2007 Jun;36(6):392-6. doi: 10.1016/j.semarthrit.2006.10.004. Epub 2007 Jan 3. PMID- 20819768 OWN - NLM STAT- MEDLINE DCOM- 20101122 LR - 20181023 IS - 1308-0032 (Electronic) IS - 1302-8723 (Linking) VI - 10 Suppl 1 DP - 2010 Aug TI - [Pulmonary arterial hypertension related to connective tissue diseases]. PG - 57-62 LID - 10.5152/akd.2010.120 [doi] AB - Pulmonary arterial hypertension (PAH) is an important complication of connective tissue diseases (CTD) and especially seen in systemic sclerosis, systemic lupus erythematosis (SLE), and mixed connective tissue disease (MCTD). In systemic sclerosis, PAH is isolated or accompanied by interstitial lung disease and currently, a major cause of mortality. It has been shown to be developed in approximately 10% of cases and annual screening with echocardiography has been recommended. Right heart catheterization is required for definite diagnosis. Limited skin involvement, late onset, Raynaud's phenomenon, digital ulcers, telangiectasias, diminished nail fold capillaries, anti-U3RNP and anticentromere antibodies are known as risk factors for PAH development in systemic sclerosis. Following diffusion lung capacity for carbon monoxide (DLCO) and pro-brain natriuretic peptide (pro-BNP) levels can be helpful for evaluating PAH development. PAH in SLE linked to antiphospholipid antibodies and Raynaud's phenomenon in some studies. MCTD is an overlap syndrome with features of systemic sclerosis, SLE, polymyositis and positive anti-U1RNP antibodies. PAH develops in 9-27% of the patients and the leading cause of mortality in patients with MCTD. Endothelin receptor antagonists, prostacyclin analogs and phosphodiesterase 5 inhibitors are being used in patients with systemic sclerosis. In SLE/MCTD patients with early diagnosis immunosuppressive treatments may be effective. FAU - Pehlivan, Ozlem AU - Pehlivan O AD - Istanbul Universitesi, Istanbul Tip Fakültesi, Iç Hastaliklari Anabilim Dali, Romatoloji Bilim Dali, Istanbul, Türkiye. FAU - Inanç, Murat AU - Inanç M LA - tur PT - English Abstract PT - Journal Article PT - Review TT - Bağ dokusu hastaliklarina bağli pulmoner arteriyel hipertansiyon. PL - Turkey TA - Anadolu Kardiyol Derg JT - Anadolu kardiyoloji dergisi : AKD = the Anatolian journal of cardiology JID - 101095069 RN - 0 (Antihypertensive Agents) RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Autoantibodies/analysis MH - *Cardiac Catheterization MH - Connective Tissue Diseases/*complications/immunology MH - Humans MH - Hypertension, Pulmonary/*diagnosis/drug therapy/*etiology/immunology MH - Immunosuppressive Agents/therapeutic use EDAT- 2010/09/18 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/09/08 06:00 PHST- 2010/09/08 06:00 [entrez] PHST- 2010/09/18 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 10.5152/akd.2010.120 [doi] PST - ppublish SO - Anadolu Kardiyol Derg. 2010 Aug;10 Suppl 1:57-62. doi: 10.5152/akd.2010.120. PMID- 19390908 OWN - NLM STAT- MEDLINE DCOM- 20091013 LR - 20220331 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 8 DP - 2009 Aug TI - Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients. PG - 915-21 LID - 10.1007/s10067-009-1175-2 [doi] AB - The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, anti-dsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease. FAU - Vaz, C C AU - Vaz CC AD - Department of Rheumatology, Coimbra University Hospital, Praceta Prof. Mota Pinto, Coimbra 3000-075, Portugal. claudiacvaz@gmail.com FAU - Couto, M AU - Couto M FAU - Medeiros, D AU - Medeiros D FAU - Miranda, L AU - Miranda L FAU - Costa, J AU - Costa J FAU - Nero, P AU - Nero P FAU - Barros, R AU - Barros R FAU - Santos, M J AU - Santos MJ FAU - Sousa, E AU - Sousa E FAU - Barcelos, A AU - Barcelos A FAU - Inês, L AU - Inês L LA - eng PT - Journal Article PT - Multicenter Study DEP - 20090424 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood MH - Connective Tissue Diseases/*blood/classification/diagnosis MH - Cross-Sectional Studies MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Lupus Erythematosus, Systemic/blood MH - Middle Aged MH - Prognosis MH - Scleroderma, Systemic/blood MH - *Severity of Illness Index MH - Young Adult EDAT- 2009/04/25 09:00 MHDA- 2009/10/14 06:00 CRDT- 2009/04/25 09:00 PHST- 2009/01/07 00:00 [received] PHST- 2009/03/26 00:00 [accepted] PHST- 2009/03/25 00:00 [revised] PHST- 2009/04/25 09:00 [entrez] PHST- 2009/04/25 09:00 [pubmed] PHST- 2009/10/14 06:00 [medline] AID - 10.1007/s10067-009-1175-2 [doi] PST - ppublish SO - Clin Rheumatol. 2009 Aug;28(8):915-21. doi: 10.1007/s10067-009-1175-2. Epub 2009 Apr 24. PMID- 18592136 OWN - NLM STAT- MEDLINE DCOM- 20090331 LR - 20211020 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 27 IP - 10 DP - 2008 Oct TI - Rare association of antisynthetase syndrome and Kennedy's disease. PG - 1329-31 LID - 10.1007/s10067-008-0946-5 [doi] AB - Antisynthetase syndrome is a type of Idiopathic Inflammatory Myopathy (IIM) associated with anti-Jo1 antibody. Kennedy's disease or X-linked spinal and bulbar muscular atrophy (SBMA) is a rare neuromuscular disease. We describe the case report of a 53-year-old man who presented with proximal muscle weakness and a history of bilateral hand tremor. Initial physical examination demonstrated "mechanic's hands", Raynaud's phenomenon, having elevated creatine kinase and lactate dehydrogenase levels and anti-Jo1 antibody positivity. His muscle biopsy demonstrated inflammatory infiltrate characteristic of IIM. Considering these findings, we reached the diagnosis of antisynthetase syndrome and commenced immunosuppressive therapy. On follow-up examination, he had developed dysphagia, and his tremor had worsened. His electroneurogram result was characteristic of Kennedy's disease, and the genetic test result showed an allele with 44 CAG repeat expansion in the androgen receptor gene of the X chromosome. This confirmed that in addition to antisynthetase syndrome, he also had Kennedy's disease. This patient now receives immunology and neurology follow-up. His symptoms have improved with low dose corticosteroids, propranolol for tremor, vitamin B supplementation, and physiotherapy. This article presents a rare case report of a patient with concurrent antisynthetase syndrome and Kennedy's disease, both of which lead to elevated creatine kinase levels and muscle weakness, thus, underpinning the importance of careful follow-up of patients with IIM and maintaining an open mind to other diagnoses when faced with refractory and/or new symptoms. FAU - Szabo, Nora AU - Szabo N AD - Division of Clinical Immunology, Third Department of Internal Medicine, Institute of Internal Medicine, University of Debrecen Medical and Health Science Centre, Moricz Zs. str 22, 4004, Debrecen, Hungary. szabnora@yahoo.com FAU - Lukacs, Szilveszter AU - Lukacs S FAU - Gunasekera, Wiranthi AU - Gunasekera W FAU - Danko, Katalin AU - Danko K LA - eng PT - Case Reports PT - Journal Article DEP - 20080701 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Bulbo-Spinal Atrophy, X-Linked/*complications/drug therapy MH - Humans MH - Male MH - Middle Aged MH - Myositis/*complications/drug therapy EDAT- 2008/07/02 09:00 MHDA- 2009/04/01 09:00 CRDT- 2008/07/02 09:00 PHST- 2008/02/20 00:00 [received] PHST- 2008/06/04 00:00 [accepted] PHST- 2008/05/20 00:00 [revised] PHST- 2008/07/02 09:00 [pubmed] PHST- 2009/04/01 09:00 [medline] PHST- 2008/07/02 09:00 [entrez] AID - 10.1007/s10067-008-0946-5 [doi] PST - ppublish SO - Clin Rheumatol. 2008 Oct;27(10):1329-31. doi: 10.1007/s10067-008-0946-5. Epub 2008 Jul 1. PMID- 24004551 OWN - NLM STAT- MEDLINE DCOM- 20150305 LR - 20220311 IS - 1875-8622 (Electronic) IS - 1386-0291 (Linking) VI - 56 IP - 4 DP - 2014 TI - Nitric oxide metabolites (nitrite and nitrate) in several clinical condition. PG - 359-69 LID - 10.3233/CH-131758 [doi] AB - We determined the concentration of nitric oxide metabolites (NO2-+NO3-), expressed as NOx, in several clinical conditions. Regarding this, we have examined 25 subjects with arterial hypertension, 41 subjects with chronic kidney disease in conservative treatment, 106 subjects with metabolic syndrome subdivided according to the presence (n = 43) or not (n = 63) of diabetes mellitus, 48 subjects with obstructive sleep apnea syndrome (OSAS), 14 women with systemic sclerosis complicated with Raynaud's phenomenon, 42 dialyzed subjects and 105 young subjects with acute myocardial infarction (AMI). In subjects with arterial hypertension, chronic kidney disease, metabolic syndrome, systemic sclerosis, as well as, in dialyzed and AMI subjects, we found at baseline a NOx increase. In dyalized subjects after a standard dialysis session, we observed a decrease in NOx. The increase in NOx in juvenile AMI was significantly influenced by cigarette smoking and less by cardiovascular risk factors and the extent of coronary lesions; at 3 and 12 months later than the initial event, we observed a decrease of NOx that remains significantly higher than the control group. In subjects with OSAS no difference in NOx was noted in comparison with normal controls, although their subdivision according to the apnea/hypopnea index operates a clear distinction regarding NOx concentration. FAU - Caimi, G AU - Caimi G AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Hopps, E AU - Hopps E AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Montana, M AU - Montana M AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Carollo, C AU - Carollo C AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Calandrino, V AU - Calandrino V AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Incalcaterra, E AU - Incalcaterra E AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Canino, B AU - Canino B AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. FAU - Lo Presti, R AU - Lo Presti R AD - Dipartimento Biomedico di Medicina Interna e Specialistica, Università di Palermo, Palermo, Italy. LA - eng PT - Journal Article PL - United States TA - Clin Hemorheol Microcirc JT - Clinical hemorheology and microcirculation JID - 9709206 RN - 0 (Nitrates) RN - 0 (Nitrites) RN - 31C4KY9ESH (Nitric Oxide) SB - IM MH - Adult MH - Cardiovascular Diseases/metabolism MH - Case-Control Studies MH - Diabetes Mellitus/metabolism MH - Female MH - Humans MH - Hypertension/metabolism MH - Male MH - Metabolic Syndrome/metabolism MH - Middle Aged MH - Nitrates/*metabolism MH - Nitric Oxide/*metabolism MH - Nitrites/*metabolism MH - Renal Insufficiency, Chronic/metabolism MH - Risk Factors OTO - NOTNLM OT - AMI OT - NOx OT - OSAS OT - dialysis OT - hypertension OT - kidney disease OT - metabolic syndrome OT - sistemic sclerosis EDAT- 2013/09/06 06:00 MHDA- 2015/03/07 06:00 CRDT- 2013/09/06 06:00 PHST- 2013/09/06 06:00 [entrez] PHST- 2013/09/06 06:00 [pubmed] PHST- 2015/03/07 06:00 [medline] AID - J3M178572620K32V [pii] AID - 10.3233/CH-131758 [doi] PST - ppublish SO - Clin Hemorheol Microcirc. 2014;56(4):359-69. doi: 10.3233/CH-131758. PMID- 27979657 OWN - NLM STAT- MEDLINE DCOM- 20181017 LR - 20210525 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 14 IP - 2 DP - 2018 Mar-Apr TI - Undifferentiated connective tissue disease and interstitial lung disease: Trying to define patterns. PG - 75-80 LID - S1699-258X(16)30151-6 [pii] LID - 10.1016/j.reuma.2016.10.007 [doi] AB - OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern. RESULTS: Sixty-six patients were included. Twenty-nine (43.94%) showed at least one 'highly specific' CTD manifestation, 16 (28.57%) had a 'specific' ANA staining pattern and 29 (43.94%) high ANA titer. Patients with 'highly specific' CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [-1 to 10] vs -6% [-16 to -4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern. CONCLUSION: The presence of 'highly specific' CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD. CI - Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Alberti, María Laura AU - Alberti ML AD - Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina. Electronic address: albertimlaura@gmail.com. FAU - Paulin, Francisco AU - Paulin F AD - Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina. FAU - Toledo, Heidegger Mateos AU - Toledo HM AD - Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México. FAU - Fernández, Martín Eduardo AU - Fernández ME AD - Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina. FAU - Caro, Fabián Matías AU - Caro FM AD - Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AD - Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México. FAU - Mejía, Mayra Edith AU - Mejía ME AD - Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, México. LA - eng LA - spa PT - Journal Article PT - Observational Study TT - Enfermedad indiferenciada del tejido conectivo y enfermedad pulmonar intersticial: intentando definir patrones. DEP - 20161213 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Adult MH - Aged MH - Female MH - Follow-Up Studies MH - Humans MH - Linear Models MH - Logistic Models MH - Lung Diseases, Interstitial/diagnostic imaging/*etiology/immunology MH - Male MH - Middle Aged MH - Odds Ratio MH - Retrospective Studies MH - Undifferentiated Connective Tissue Diseases/complications/*diagnosis/immunology OTO - NOTNLM OT - Anticuerpos antinucleares OT - Antinuclear antibodies OT - Autoimmune diseases OT - Connective tissue diseases OT - Enfermedad del tejido conectivo OT - Enfermedad pulmonar intersticial OT - Enfermedades autoinmunes OT - Fenómeno de Raynaud OT - Interstitial lung diseases OT - Raynaud phenomenon EDAT- 2016/12/17 06:00 MHDA- 2018/10/18 06:00 CRDT- 2016/12/17 06:00 PHST- 2016/06/28 00:00 [received] PHST- 2016/10/13 00:00 [revised] PHST- 2016/10/30 00:00 [accepted] PHST- 2016/12/17 06:00 [pubmed] PHST- 2018/10/18 06:00 [medline] PHST- 2016/12/17 06:00 [entrez] AID - S1699-258X(16)30151-6 [pii] AID - 10.1016/j.reuma.2016.10.007 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2018 Mar-Apr;14(2):75-80. doi: 10.1016/j.reuma.2016.10.007. Epub 2016 Dec 13. PMID- 41884771 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260326 LR - 20260326 IS - 2590-0595 (Electronic) IS - 2590-0595 (Linking) VI - 8 IP - 4 DP - 2026 Apr TI - Triple-Positive Serology in Rapidly Progressive Glomerulonephritis: A Diagnostic Dilemma. PG - 101270 LID - 10.1016/j.xkme.2026.101270 [doi] LID - 101270 AB - The acute onset of malignant hypertension and acute kidney injury in patients with features suggestive of systemic sclerosis often raises concern for scleroderma renal crisis, a life-threatening complication requiring prompt recognition and angiotensin-converting enzyme inhibitor therapy. However, other autoimmune vasculitides, including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and antiglomerular basement membrane (anti-GBM) disease, can present similarly but require immunosuppressive therapy. We reported a diagnostically challenging case of a 72-year-old woman with interstitial lung disease, Raynaud's phenomenon, and serologic findings of antitopoisomerase I antibody and antimyeloperoxidase (p-ANCA) antibodies. Given this clinical picture, scleroderma renal crisis was initially suspected. However, a kidney biopsy revealed pauci-immune necrotizing and crescentic glomerulonephritis with uniform crescents and without linear IgG deposition along the GBM. Given the uniformity of crescents, additional serologic testing revealed elevated anti-GBM antibody levels, posing a therapeutic dilemma regarding the presence of clinically significant anti-GBM disease in the absence of immunofluorescence confirmation. This case highlighted the critical role of early kidney biopsy in ambiguous presentations and the need for heightened awareness of overlapping autoimmune syndromes to guide timely and appropriate treatment. Systematic reporting of such overlap syndromes is essential for improving diagnostic accuracy and optimizing outcomes. CI - © 2026 The Authors. FAU - Godbole, Nisha AU - Godbole N AD - Department of Internal Medicine, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Tiwari, Mahesh AU - Tiwari M AD - Department of Internal Medicine, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Jotwani, Pooja AU - Jotwani P AD - Department of Internal Medicine, Division of Rheumatology and Connective Tissue, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Verma, Jyoti AU - Verma J AD - Department of Internal Medicine, Division of Rheumatology and Connective Tissue, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Law, Jammie AU - Law J AD - Department of Internal Medicine, Division of Rheumatology and Connective Tissue, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Roofeh, David AU - Roofeh D AD - Department of Internal Medicine, Division of Rheumatology and Connective Tissue, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. FAU - Plamm, Alex AU - Plamm A AD - Division of Nephrology, Robert Wood Johnson University Hospital, Rutgers University, New Brunswick, NJ. FAU - Maroun, Marie-Claire AU - Maroun MC AD - Department of Internal Medicine, Division of Rheumatology and Connective Tissue, Rutgers Robert Wood Johnson University Hospital, New Brunswick, NJ. LA - eng PT - Case Reports PT - Journal Article DEP - 20260121 PL - United States TA - Kidney Med JT - Kidney medicine JID - 101756300 PMC - PMC13010390 OTO - NOTNLM OT - Rapidly progressive glomerulonephritis OT - anti-glomerular basement membrane disease OT - antineutrophil cytoplasmic antibody-associated vasculitis OT - dual-positive disease EDAT- 2026/03/26 07:09 MHDA- 2026/03/26 07:10 PMCR- 2026/01/21 CRDT- 2026/03/26 05:08 PHST- 2026/03/26 07:10 [medline] PHST- 2026/03/26 07:09 [pubmed] PHST- 2026/03/26 05:08 [entrez] PHST- 2026/01/21 00:00 [pmc-release] AID - S2590-0595(26)00031-2 [pii] AID - 101270 [pii] AID - 10.1016/j.xkme.2026.101270 [doi] PST - epublish SO - Kidney Med. 2026 Jan 21;8(4):101270. doi: 10.1016/j.xkme.2026.101270. eCollection 2026 Apr. PMID- 39757967 OWN - NLM STAT- MEDLINE DCOM- 20250106 LR - 20250117 IS - 1118-4841 (Print) IS - 1118-4841 (Linking) VI - 28 IP - 11 DP - 2024 Nov 30 TI - Severe mycoplasma pneumoniae pneumonia combined with cold agglutinin disease and pulmonary embolism in childhood: A case report and review of the literature. PG - 205-215 LID - 10.29063/ajrh2024/v28i11.20 [doi] AB - This was a case report and literature review, aimed to strengthen the understanding and therapy of mycoplasma pneumoniae (MP) pneumonia combined with cold agglutinin disease and pulmonary embolism in children. A 7-year-old boy was taken to the hospital with fever for 7 days, cough for 5 days, and recurrent cyanosis at the extremities of one day duration. Pulmonary artery computed tomography angiography (CTA) showed pulmonary embolism, double pneumonia, and pleural effusion. Mycoplasma (MP) antibody IgM, Coombs test, and anti C3d were positive while anti-IgG was negative. After treatment, the patient improved. One month after discharge, the Coombs test was negative, and pulmonary CTA showed no pulmonary embolism. The diagnosis was severe MP with cold agglutinin disease and pulmonary embolism. The cases were reviewed, involving a total of 18 cases. Two patients had pulmonary embolism, and one had Raynaud's phenomenon with superior mesenteric artery and popliteal artery embolism. After treatment, 16 cases recovered while two cases were lost to follow up. We conclude that although the disease may be associated with severe complications such as embolism, acrocyanosis, and gangrene, the prognosis is relatively good for most patients. CI - African Journal of Reproductive Health © 2024. FAU - Ss, Liang AU - Ss L AD - Department of Pediatrics, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai 264000, Shandong, China. FAU - Hb, Liu AU - Hb L AD - Department of Pediatrics, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai 264000, Shandong, China. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - Nigeria TA - Afr J Reprod Health JT - African journal of reproductive health JID - 9712263 RN - 0 (Anti-Bacterial Agents) SB - IM MH - Humans MH - Male MH - *Pulmonary Embolism/diagnosis/drug therapy MH - Child MH - *Pneumonia, Mycoplasma/complications/diagnosis/drug therapy MH - *Anemia, Hemolytic, Autoimmune/diagnosis MH - Mycoplasma pneumoniae/isolation & purification MH - Anti-Bacterial Agents/therapeutic use MH - Computed Tomography Angiography OTO - NOTNLM OT - Mycoplasma pneumonia OT - cold agglutinin disease OT - pulmonary embolism COIS- The Authors declared no conflict of interest EDAT- 2025/01/06 10:07 MHDA- 2025/01/07 00:21 CRDT- 2025/01/06 04:39 PHST- 2025/01/07 00:21 [medline] PHST- 2025/01/06 10:07 [pubmed] PHST- 2025/01/06 04:39 [entrez] AID - Afr J Reprod Health 2024; 28 [11]: 205-215 [pii] AID - 10.29063/ajrh2024/v28i11.20 [doi] PST - ppublish SO - Afr J Reprod Health. 2024 Nov 30;28(11):205-215. doi: 10.29063/ajrh2024/v28i11.20. PMID- 37034360 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230411 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 33 IP - 4 DP - 2022 Dec TI - Anti-Jo1 Syndrome: Understanding a Rare Cause of Interstitial Lung Disease. PG - 437-443 LID - 10.31138/mjr.33.4.437 [doi] AB - BACKGROUND: Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed. MATERIALS AND METHODS: In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 - 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted. RESULT: A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression. CONCLUSION: Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy. CI - © 2022 The Mediterranean Journal of Rheumatology (MJR). FAU - Hegde, Arun AU - Hegde A AD - Department of Rheumatology, Command Hospital, Lucknow, India. FAU - Marwah, Vikas AU - Marwah V AD - Department of Respiratory Medicine, AICTS, Pune, India. FAU - V, Shrinath AU - V S AD - Department of Respiratory Medicine, AICTS, Pune, India. FAU - Choudhary, Robin AU - Choudhary R AD - Department of Respiratory Medicine, AICTS, Pune, India. FAU - Malik, Virendra AU - Malik V AD - Department of Radiology, AICTS, Pune, India. LA - eng PT - Journal Article DEP - 20221231 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC10075366 OTO - NOTNLM OT - anti-Jo1 antibody OT - anti-synthetase syndrome OT - idiopathic inflammatory myositis OT - interstitial lung disease COIS- The authors declare no conflict of interest. EDAT- 2023/04/11 06:00 MHDA- 2023/04/11 06:01 PMCR- 2022/12/31 CRDT- 2023/04/10 04:06 PHST- 2021/12/03 00:00 [received] PHST- 2022/06/14 00:00 [revised] PHST- 2022/06/30 00:00 [accepted] PHST- 2023/04/11 06:01 [medline] PHST- 2023/04/10 04:06 [entrez] PHST- 2023/04/11 06:00 [pubmed] PHST- 2022/12/31 00:00 [pmc-release] AID - MJR-33-4-437 [pii] AID - 10.31138/mjr.33.4.437 [doi] PST - epublish SO - Mediterr J Rheumatol. 2022 Dec 31;33(4):437-443. doi: 10.31138/mjr.33.4.437. eCollection 2022 Dec. PMID- 35750435 OWN - NLM STAT- MEDLINE DCOM- 20220628 LR - 20240624 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 15 IP - 6 DP - 2022 Jun 24 TI - Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient. LID - 10.1136/bcr-2022-249747 [doi] LID - e249747 AB - A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud's phenomenon. Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology. CI - © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Steward, Matthew AU - Steward M AUID- ORCID: 0000-0002-1873-4665 AD - Department of Respiratory Medicine, Royal Devon and Exeter NHS Foundation Trust, Exeter, Devon, UK matt.steward1@nhs.net. AD - College of Medicine and Health, University of Exeter Medical School, Exeter, Devon, UK. FAU - Yu, Johannes H AU - Yu JH AUID- ORCID: 0000-0002-0882-3955 AD - College of Medicine and Health, University of Exeter Medical School, Exeter, Devon, UK. FAU - Gibbons, Michael A AU - Gibbons MA AD - Department of Respiratory Medicine, Royal Devon and Exeter NHS Foundation Trust, Exeter, Devon, UK. LA - eng PT - Case Reports PT - Journal Article DEP - 20220624 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - Lymphoid Interstitial Pneumonia SB - IM MH - Humans MH - *Lung Diseases, Interstitial/diagnostic imaging/etiology MH - Male MH - *Multiple Myeloma/complications MH - Respiratory Function Tests MH - *Sjogren's Syndrome/complications/diagnosis MH - Tomography, X-Ray Computed/adverse effects PMC - PMC9234793 OTO - NOTNLM OT - Connective tissue disease OT - Interstitial lung disease OT - Sjogren's syndrome COIS- Competing interests: None declared. EDAT- 2022/06/25 06:00 MHDA- 2022/06/29 06:00 PMCR- 2024/06/23 CRDT- 2022/06/24 21:07 PHST- 2022/06/24 21:07 [entrez] PHST- 2022/06/25 06:00 [pubmed] PHST- 2022/06/29 06:00 [medline] PHST- 2024/06/23 00:00 [pmc-release] AID - 15/6/e249747 [pii] AID - bcr-2022-249747 [pii] AID - 10.1136/bcr-2022-249747 [doi] PST - epublish SO - BMJ Case Rep. 2022 Jun 24;15(6):e249747. doi: 10.1136/bcr-2022-249747. PMID- 18625615 OWN - NLM STAT- MEDLINE DCOM- 20090624 LR - 20260128 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 68 IP - 6 DP - 2009 Jun TI - Geographical variation of disease manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research (EUSTAR) group database. PG - 856-62 LID - 10.1136/ard.2008.091348 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe. AIMS AND METHODS: The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70). RESULTS: 3661 patients from 79 centres in 62 cities and 23 countries were analysed. There was no clear trend between geographical coordinates and SSc subsets, although there appeared to be an increased prevalence of Scl70 in the more eastern centres. There was no association between geographical longitude or latitude and the age at the onset of Raynaud's phenomenon or the onset of non-Raynaud's symptoms. There was also a trend for the more eastern centres to care for patients with a higher prevalence of more severe organ manifestations (pulmonary arterial hypertension, cardiac involvement). Between different centres within one city there was a large variability in the frequency of organ complications. CONCLUSION: This analysis suggests that eastern centres care for more severe SSc manifestations in Europe. Large differences in patient referral account for a large local variability of SSc presentations and preclude the identification of genetic or environmental factors. FAU - Walker, U A AU - Walker UA AD - Felix Platter Spital, Basel, Switzerland. ulrich.walker@fps-basel.ch FAU - Tyndall, A AU - Tyndall A FAU - Czirják, L AU - Czirják L FAU - Denton, C P AU - Denton CP FAU - Farge-Bancel, D AU - Farge-Bancel D FAU - Kowal-Bielecka, O AU - Kowal-Bielecka O FAU - Müller-Ladner, U AU - Müller-Ladner U FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M CN - EUSTAR co-authors LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20080714 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/blood MH - Cities MH - Clinical Trials as Topic MH - Cluster Analysis MH - *Databases, Factual MH - Europe/epidemiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Scleroderma, Systemic/*epidemiology/immunology MH - Sex Factors MH - *Topography, Medical EDAT- 2008/07/16 09:00 MHDA- 2009/06/25 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2009/06/25 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] AID - S0003-4967(24)21752-0 [pii] AID - 10.1136/ard.2008.091348 [doi] PST - ppublish SO - Ann Rheum Dis. 2009 Jun;68(6):856-62. doi: 10.1136/ard.2008.091348. Epub 2008 Jul 14. PMID- 41379316 OWN - NLM STAT- MEDLINE DCOM- 20260114 LR - 20260117 IS - 2731-7013 (Electronic) IS - 2731-7005 (Print) IS - 2731-7005 (Linking) VI - 77 IP - 1 DP - 2026 Jan TI - [Systemic sclerosis : A disease with multiple faces]. PG - 41-49 LID - 10.1007/s00105-025-05622-w [doi] AB - Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease within the spectrum of rheumatologic disorders. It is characterized by a combination of vasculopathy and inflammatory fibrosis affecting the skin and various internal organs. The disease presents with heterogeneous manifestations, which can complicate diagnosis and must be carefully considered during both monitoring and treatment. In recent decades, advances in research have expanded the range of therapeutic options available for SSc. Treatment strategies need to be tailored individually, depending on the specific clinical manifestations in each patient. The aim of this article is to provide an overview of the diverse disease manifestations and current treatment approaches in SSc. For optimal patient care, close collaboration between rheumatologists, pneumologists, gastroenterologists, cardiologists, nephrologists and general practitioners is essential. CI - © 2025. The Author(s). FAU - Evers, Caroline AU - Evers C AD - Klinik für Rheumatologie, Universitätsspital Zürich, Rämistr. 100, 8091, Zürich, Schweiz. FAU - Distler, Jörg AU - Distler J AD - Klinik für Rheumatologie und Hiller-Forschungszentrum, Universitätsklinikum Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland. FAU - Distler, Oliver AU - Distler O AD - Klinik für Rheumatologie, Universitätsspital Zürich, Rämistr. 100, 8091, Zürich, Schweiz. oliver.distler@usz.ch. LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Systemische Sklerose : Eine Erkrankung mit vielen Gesichtern. PL - Germany TA - Dermatologie (Heidelb) JT - Dermatologie (Heidelberg, Germany) JID - 9918384885206676 SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/therapy/complications MH - Skin/pathology PMC - PMC12804196 OTO - NOTNLM OT - Antifibrotic therapy OT - Connective tissue diseases OT - Immunosuppression therapy OT - Raynaud disease OT - Skin fibrosis EDAT- 2025/12/11 13:03 MHDA- 2026/01/14 12:30 PMCR- 2025/12/11 CRDT- 2025/12/11 11:13 PHST- 2026/01/14 12:30 [medline] PHST- 2025/12/11 13:03 [pubmed] PHST- 2025/12/11 11:13 [entrez] PHST- 2025/12/11 00:00 [pmc-release] AID - 10.1007/s00105-025-05622-w [pii] AID - 5622 [pii] AID - 10.1007/s00105-025-05622-w [doi] PST - ppublish SO - Dermatologie (Heidelb). 2026 Jan;77(1):41-49. doi: 10.1007/s00105-025-05622-w. PMID- 38558690 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240403 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 2 DP - 2024 Feb TI - Black Digits Matter: A Multispecialty Enigma. PG - e55133 LID - 10.7759/cureus.55133 [doi] LID - e55133 AB - Introduction Digital ischemia is alike any other visceral ischemic event leading to severe tissue damage ultimately causing necrosis of the involved extremity. It's like a preview of the upcoming systemic disorder and can present itself in any specialty and hence everyone, be it a physician or a surgeon must be primed toward how to proceed with a case of digital ischemia. In this case series, we present six such cases that presented with digital ischemic events either as a sole presentation or were followed by other systemic manifestations that led to their evaluation and ultimately the etiology behind it. Material and method Patients visiting Rheumatology OPD with complaints suggestive of digital ischemia were included in this study. All patients underwent thorough history taking and clinical examination to establish the cause of digital ischemia. Patients with probable infective, trauma, cardiac, and drug-induced causes and malignancies were excluded. As per probable autoimmune causes, patients underwent evaluation via antinuclear antibodies by immunofluorescence (ANA by IF), antiphospholipid antibodies like lupus anticoagulant (LAC), anticardiolipin antibodies (AcL) and anti Beta2GP1 antibodies, extractable nuclear antigens (ENA) and in cases of suspected vasculitis doppler ultrasound and angiography.  Results Six patients were identified as cases primarily presenting with digital ischemia or with a prior history of digital ischemia. Two patients were of the pediatric age group, one 16-year-old male presenting with acute arthritis and a history of digital ischemia one year back, and the other was a 12-year-old female with blackening of the second toe in her left foot with a history of similar complaints in the left great toe for which she underwent amputation of that toe. Other four cases were of the adult age group, with two cases of scleroderma, one with systemic lupus erythematosus, and one with Takayasu arteritis. All of these patients primarily presented to departments other than rheumatology. Conclusion Digital ischemia is a pan-specialty problem with the etiologies spreading across a vast spectrum of rheumatological disorders, many of which may present to different specialties initially, later discovered to be part of the systemic manifestation of autoimmune diseases. Hence, it becomes imperative to have a rheumatological perspective in these cases of digital ischemia which all specialities should be aware of, and timely referral may prevent permanent loss of the digits and in some cases the entire limb. CI - Copyright © 2024, Agrawal et al. FAU - Agrawal, Abhijeet AU - Agrawal A AD - Department of Medicine, Jawaharlal Nehru Medical College, Wardha, IND. FAU - Bhagawati, Jahnabi AU - Bhagawati J AD - Department of Medicine, Jawaharlal Nehru Medical College, Wardha, IND. FAU - Kumar, Sunil AU - Kumar S AD - Department of Medicine, Jawaharlal Nehru Medical College, Wardha, IND. FAU - Acharya, Sourya AU - Acharya S AD - Department of Medicine, Jawaharlal Nehru Medical College, Wardha, IND. LA - eng PT - Journal Article DEP - 20240228 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10979651 OTO - NOTNLM OT - antiphospholipid antibody OT - autoimmune OT - connective tissue disease OT - digital ischemia OT - digital ulcer OT - raynaud's phenomenon OT - scleroderma OT - systemic lupus erythematosus COIS- The authors have declared that no competing interests exist. EDAT- 2024/04/01 18:42 MHDA- 2024/04/01 18:43 PMCR- 2024/02/28 CRDT- 2024/04/01 17:02 PHST- 2024/01/28 00:00 [received] PHST- 2024/02/28 00:00 [accepted] PHST- 2024/04/01 18:43 [medline] PHST- 2024/04/01 18:42 [pubmed] PHST- 2024/04/01 17:02 [entrez] PHST- 2024/02/28 00:00 [pmc-release] AID - 10.7759/cureus.55133 [doi] PST - epublish SO - Cureus. 2024 Feb 28;16(2):e55133. doi: 10.7759/cureus.55133. eCollection 2024 Feb. PMID- 37744045 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241003 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 8 IP - 3 DP - 2023 Oct TI - A very rare cause of blue finger: A case-based review. PG - NP1-NP5 LID - 10.1177/23971983231162679 [doi] AB - INTRODUCTION: Cryofibrinogen is an abnormal, cold-insoluble protein composed of a combination of fibrinogen, fibrin, and fibronectin. Cryofibrinogenemia can be essential (e.g. primary) or secondary to various conditions. While low levels of cryofibrinogen can be seen in asymptomatic healthy individuals without evidence of clinical features typical of cryofibrinogenemia, cryofibrinogenemia associated with clinical features is considered very rare. The clinical features of cryofibrinogenemia ranges from skin manifestations, including Raynaud's phenomenon and livedo reticularis, to more severe organ-threatening manifestations such as tissue ischemia and gangrene. CASE DESCRIPTION: We report a case of a 48-year-old male who presented with blue finger and palpable purpura on his distal extremities. Laboratory workup was positive for anti-nuclear antibodies, anti-double-stranded DNA, anti-ribonucleoprotein, and rheumatoid factor, while antineutrophil cytoplasmic antibodies and cryoglobulins were negative. Testing for hypercoagulable states and infectious etiologies was unrevealing. Later, angiographic computed tomography showed multiple pulmonary embolisms and disruption of blood flow to the left fifth digit. As the aforementioned workup could not explain the presence of the thrombus by a thromboembolic cause, a search for an in situ cause other than antiphospholipid syndrome was initiated and concentrated mainly on cryofibrinogenemia. Blood samples collected using prewarmed anticoagulant containing tubes were sent to central lab familiar with performing the test. Two weeks later, a positive result for the presence of cryofibrinogen confirmed the diagnosis of cryofibrinogenemia. Due to the presence of multiple signs compatible with mixed connective tissue disease, he was diagnosed with cryofibrinogenemia secondary to mixed connective tissue disease, and treatment with prednisone, low-molecular-weight heparin, prostacyclin and hydroxychloroquine was initiaed with favorable outcome. CONCLUSION: Cryofibrinogenemia is a rare and underdiagnosed condition. Clinicians should be aware of this cryopathy especially in the cases of Raynaud's phenomenon and ischemic ulcers not explained by other causes. Precautions must be taken during the diagnostic process, and therapy should be given as soon as possible. CI - © The Author(s) 2023. FAU - Hassan, Fadi AU - Hassan F AUID- ORCID: 0000-0002-9648-1130 AD - The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. AD - Rheumatology Unit, Galilee Medical Center, Nahariya, Israel. FAU - Khoury, Amir AU - Khoury A AD - The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. AD - Department of Internal Medicine "F", Galilee Medical Center, Nahariya, Israel. FAU - Awad, Jamal AU - Awad J AD - The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. AD - Department of Internal Medicine "F", Galilee Medical Center, Nahariya, Israel. FAU - Jeries, Helana AU - Jeries H AUID- ORCID: 0000-0001-8624-6332 AD - The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. AD - Rheumatology Unit, Galilee Medical Center, Nahariya, Israel. FAU - Naffaa, Mohammad E AU - Naffaa ME AD - The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel. AD - Rheumatology Unit, Galilee Medical Center, Nahariya, Israel. LA - eng PT - Case Reports PT - Journal Article DEP - 20230326 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC10515991 OTO - NOTNLM OT - Cryofibrinogenemia OT - blue finger OT - ischemia OT - mixed connective tissue disease OT - purpura COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/09/25 06:42 MHDA- 2023/09/25 06:43 PMCR- 2024/10/01 CRDT- 2023/09/25 04:37 PHST- 2022/12/27 00:00 [received] PHST- 2023/02/21 00:00 [accepted] PHST- 2023/09/25 06:43 [medline] PHST- 2023/09/25 06:42 [pubmed] PHST- 2023/09/25 04:37 [entrez] PHST- 2024/10/01 00:00 [pmc-release] AID - 10.1177_23971983231162679 [pii] AID - 10.1177/23971983231162679 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2023 Oct;8(3):NP1-NP5. doi: 10.1177/23971983231162679. Epub 2023 Mar 26. PMID- 19718594 OWN - NLM STAT- MEDLINE DCOM- 20090911 LR - 20151119 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 134 IP - 36 DP - 2009 Sep TI - [Severe corticoid-refractory autoimmune thrombocytopenia associated with mixed connective tissue disease (Sharp's syndrome). Treatment with rituximab]. PG - 1734-8 LID - 10.1055/s-0029-1234008 [doi] AB - HISTORY AND CLINICAL FINDINGS: An 18-year-old woman with mixed connective tissue disease (Sharp's syndrome), diagnosed two years earlier, was admitted because of severe thrombocytopenia. At that time the only symptom typical for collagen disease was Raynaud;s syndrome. The patient was in good general condition, the clinical examination revealed no signs of bleeding or of splenomegaly. INVESTIGATIONS: Imaging procedures showed no abnormality. The platelets were decreased to 5 Gpt/l (normal range 150 - 400 Gpt/l). The bone marrow biopsy showed a secondary immunological thrombocytopenia with an increased number of megakaryocytes. TREATMENT AND CLINICAL COURSE: Treatment with prednisolone, 100 mg/day, had no significant effect. As the patient refused splenectomy, treatment with rituximab 500 mg (375 mg/m2) per week was given over a period of four weeks, followed by azathioprine 2 x 50 mg/d. All tests demonstrated continuing increase of the platelet count up to 70 Gpt/l (normal range 150 - 400 Gpt/l). The signs of Raynaud;s syndrome also regressed. CONCLUSION: Immunologic thrombocytopenia is a potentially life-threatening hematological manifestation of mixed connective tissue disease. If high-dosage prednisolone brings no response, a splenectomy is an efficacious treatment. B-cell depletion with rituximab offers another safe and adequate option. FAU - Rudolph, S E AU - Rudolph SE AD - Klinik für Innere Medizin/ Schwerpunkt Rheumatologie, Zeisigwaldkliniken Bethanien, Chemnitz. rheuma@bethanien-sachsen.de FAU - Kouba, M AU - Kouba M FAU - Hrdlicka, P AU - Hrdlicka P LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Schwere corticoidrefraktäre Autoimmunthrombozytopenie bei Sharp-Syndrom. Therapie mit Rituximab. DEP - 20090828 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Glucocorticoids) RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adolescent MH - Antibodies, Monoclonal/*therapeutic use MH - Antibodies, Monoclonal, Murine-Derived MH - B-Lymphocytes/drug effects MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunologic Factors/*therapeutic use MH - Mixed Connective Tissue Disease/*complications/drug therapy/immunology MH - Prednisolone/therapeutic use MH - Purpura, Thrombocytopenic, Idiopathic/diagnosis/*drug therapy/etiology/immunology MH - Rituximab MH - Splenectomy MH - Treatment Failure MH - Treatment Refusal EDAT- 2009/09/01 06:00 MHDA- 2009/09/12 06:00 CRDT- 2009/09/01 09:00 PHST- 2009/09/01 09:00 [entrez] PHST- 2009/09/01 06:00 [pubmed] PHST- 2009/09/12 06:00 [medline] AID - 10.1055/s-0029-1234008 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2009 Sep;134(36):1734-8. doi: 10.1055/s-0029-1234008. Epub 2009 Aug 28. PMID- 36101994 OWN - NLM STAT- MEDLINE DCOM- 20220915 LR - 20220915 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 74 IP - 2 DP - 2022 Sep 13 TI - Frequency of ANA/DFS70 autoantibodies in Colombian patients with undifferentiated connective tissue disease. LID - 10.4081/reumatismo.2022.1420 [doi] AB - The objective was to describe the clinical characteristics and the frequency of the ANA/DFS70 autoantibodies in patients affected by undifferentiated connective tissue disease (UCTD) in a tertiary hospital in Colombia. This descriptive cross-sectional study enrolled patients who fulfilled the classification criteria for UCTD. ANAHEp- 2 test and the modified assay for ANA/DFS70 autoantibodies were performed through the indirect immunofluorescence technique. Erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, and the antibodies to anti-extractable nuclear antigens, DNA, phospholipids (IgG, IgM, IgA), and cyclic citrullinated peptide were also evaluated. Fifty-three patients were studied; 42/53 (79%) tested positive for ANA and 5/42 (11.9%) for ANA/DFS70 antibodies with a dense fine speckled fluorescent pattern (AC-2) in ANA HEp-2 test that was confirmed by a modified HEp-2-DFS70 assay. Patients had arthralgia (87%, n=47), non-erosive arthritis (66%, n=34), xerostomia (64%, n=34), xerophthalmia (42%, n=22), and Raynaud's phenomenon (17%, n=9). Arthralgia, xerophthalmia, xeroderma, and absence of disease evolution to a specific disease over five years were more frequent in patients with a positive result for the anti-DFS70 antibodies. The ANA/DFS70 autoantibodies were more frequent in patients with UCTD compared to other rheumatic diseases for which they were initially evaluated. More studies are required to support the predictive role of this antibody to the absence of progression to a well-defined connective tissue disease. FAU - Rincón-Riaño, D AU - Rincón-Riaño D AD - Universidad Militar Nueva Granada. dncolombia@hotmail.com. FAU - Fernández-Ávila, D G AU - Fernández-Ávila DG AD - Professor of Pontificia Universidad Javeriana. daniel.fernandez@javeriana.edu.co. FAU - Acero-Molina, D AU - Acero-Molina D AD - Universidad Militar Nueva Granada. aleja_k2017@hotmail.com. FAU - Bello Gualtero, J M AU - Bello Gualtero JM AD - Universidad Militar Nueva Granada. Juanmabello36@gmail.com. FAU - Romero-Sánchez, C AU - Romero-Sánchez C AD - Universidad Militar Nueva Granada. romeromaria@unbosque.edu.co. LA - eng PT - Journal Article DEP - 20220913 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Adaptor Proteins, Signal Transducing) RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Transcription Factors) SB - IM MH - Adaptor Proteins, Signal Transducing MH - Antibodies, Antinuclear MH - Arthralgia MH - Autoantibodies MH - Colombia/epidemiology MH - Cross-Sectional Studies MH - Humans MH - Transcription Factors MH - *Undifferentiated Connective Tissue Diseases MH - *Xerophthalmia EDAT- 2022/09/15 06:00 MHDA- 2022/09/16 06:00 CRDT- 2022/09/14 02:55 PHST- 2021/04/08 00:00 [received] PHST- 2022/07/11 00:00 [accepted] PHST- 2022/09/14 02:55 [entrez] PHST- 2022/09/15 06:00 [pubmed] PHST- 2022/09/16 06:00 [medline] AID - 10.4081/reumatismo.2022.1420 [doi] PST - epublish SO - Reumatismo. 2022 Sep 13;74(2). doi: 10.4081/reumatismo.2022.1420. PMID- 18077496 OWN - NLM STAT- MEDLINE DCOM- 20080110 LR - 20220409 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 IP - 1 DP - 2008 Jan TI - High-frequency ultrasound of skin involvement in systemic sclerosis reflects oedema, extension and severity in early disease. PG - 84-7 AB - OBJECTIVE: The aim was to compare skin assessment by palpation and by high-frequency ultrasound in patients with SSc with disease duration <2 yrs. METHODS: Skin thickness and skin echogenicity were measured by 20 MHz ultrasound at five different anatomical sites in 106 individuals within 2 yrs from the first non-Raynaud's symptom and compared with the modified Rodnan skin score (mRss). RESULTS: The patients with short disease duration were characterized by high skin thickness and low skin echogenicity, which correlated inversely, reflecting oedema. Patients with diffuse skin involvement displayed higher skin thickness and lower skin echogenicity than did patients with limited skin involvement. The ultrasound measurements correlated to the local mRss from the corresponding anatomical region and also to the total mRss. However, there was a considerable overlap in both skin thickness and skin echogenicity between different local mRss at all five anatomical sites. Skin involvement of the chest could be detected earlier by ultrasound than by palpation. CONCLUSION: In SSc patients with short disease duration, high-frequency ultrasound can identify the oedematous phase that may precede palpable skin involvement and may thus be useful to identify patients with diffuse skin involvement very early in the disease process. Ultrasound measurements also reflect the severity of the overall skin involvement. FAU - Hesselstrand, R AU - Hesselstrand R AD - Department of Rheumatology, Lund University Hospital, S-221 85 Lund, Sweden. roger.hesselstrand@skane.se FAU - Scheja, A AU - Scheja A FAU - Wildt, M AU - Wildt M FAU - Akesson, A AU - Akesson A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Edema/*diagnosis/physiopathology MH - Female MH - Fingers/diagnostic imaging/pathology MH - Follow-Up Studies MH - Forearm/diagnostic imaging/parasitology MH - Humans MH - Leg/diagnostic imaging/pathology MH - Male MH - Middle Aged MH - Palpation/*methods MH - Reproducibility of Results MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnosis/physiopathology MH - Severity of Illness Index MH - Skin/*diagnostic imaging/*pathology/physiopathology MH - Thorax/diagnostic imaging/pathology MH - Ultrasonography/*methods EDAT- 2007/12/14 09:00 MHDA- 2008/01/11 09:00 CRDT- 2007/12/14 09:00 PHST- 2007/12/14 09:00 [pubmed] PHST- 2008/01/11 09:00 [medline] PHST- 2007/12/14 09:00 [entrez] AID - 47/1/84 [pii] AID - 10.1093/rheumatology/kem307 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Jan;47(1):84-7. doi: 10.1093/rheumatology/kem307. PMID- 25678814 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150214 LR - 20231112 IS - 1179-1349 (Print) IS - 1179-1349 (Electronic) IS - 1179-1349 (Linking) VI - 7 DP - 2015 TI - Raynaud phenomenon and mortality: 20+ years of follow-up of the Charleston Heart Study cohort. PG - 161-8 LID - 10.2147/CLEP.S75482 [doi] AB - BACKGROUND: Raynaud phenomenon (RP) is a temporary vasoconstrictive condition that often manifests itself in the fingers in response to cold or stress. It often co-occurs with certain chronic diseases that impact mortality. Our objective was to determine whether RP has any independent association with survival. METHODS: From 1987-1989, a total of 830 participants of the Charleston Heart Study cohort completed an in-person RP screening questionnaire. Two definitions of RP were used: a broad definition that included both blanching (pallor) and cyanotic color changes and a narrow definition that included only blanching. All-cause and cardiovascular disease (CVD) mortality were compared between subjects with and without RP using race-specific survival models that adjusted for age, sex, baseline CVD, and 10-year risk of coronary heart disease. RESULTS: Using the narrow RP definition, we identified a significant interaction between older age and the presence of RP on all-cause mortality. In the broad RP definition model, the presence of RP was not associated with CVD mortality among blacks; however, among whites, the presence of RP was associated with a 1.6-fold increase in the hazard associated with CVD-related death (hazard ratio: 1.55, 95% confidence interval: 1.10-2.20, P=0.013). CONCLUSION: RP was independently associated with mortality among older adults in our cohort. Among whites, RP was associated with increased CVD-related death. It is possible that RP may be a sign of undiagnosed vascular disease. FAU - Nietert, Paul J AU - Nietert PJ AD - Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, USA. FAU - Shaftman, Stephanie R AU - Shaftman SR AD - Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, USA. FAU - Silver, Richard M AU - Silver RM AD - Department of Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA. FAU - Wolf, Bethany J AU - Wolf BJ AD - Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, USA. FAU - Egan, Brent M AU - Egan BM AD - Greenville Health System, Care Coordination Institute, Greenville, SC, USA. FAU - Hunt, Kelly J AU - Hunt KJ AD - Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, USA. FAU - Smith, Edwin A AU - Smith EA AD - Department of Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA. LA - eng GR - P60 AR062755/AR/NIAMS NIH HHS/United States GR - UL1 TR000062/TR/NCATS NIH HHS/United States PT - Journal Article DEP - 20150203 PL - New Zealand TA - Clin Epidemiol JT - Clinical epidemiology JID - 101531700 PMC - PMC4322708 OTO - NOTNLM OT - Raynaud disease OT - cardiovascular diseases OT - cohort studies OT - survival analysis EDAT- 2015/02/14 06:00 MHDA- 2015/02/14 06:01 PMCR- 2015/02/03 CRDT- 2015/02/14 06:00 PHST- 2015/02/14 06:00 [entrez] PHST- 2015/02/14 06:00 [pubmed] PHST- 2015/02/14 06:01 [medline] PHST- 2015/02/03 00:00 [pmc-release] AID - clep-7-161 [pii] AID - 10.2147/CLEP.S75482 [doi] PST - epublish SO - Clin Epidemiol. 2015 Feb 3;7:161-8. doi: 10.2147/CLEP.S75482. eCollection 2015. PMID- 29390349 OWN - NLM STAT- MEDLINE DCOM- 20180212 LR - 20221005 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 96 IP - 50 DP - 2017 Dec TI - Sjögren's syndrome manifesting as clinicopathological features of TAFRO syndrome: A case report. PG - e9220 LID - 10.1097/MD.0000000000009220 [doi] LID - e9220 AB - RATIONALE: TAFRO syndrome is a newly proposed disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. In this report, we describe the development of severe TAFRO syndrome-like systemic symptoms during the clinical course of juvenile-onset Sjögren's syndrome in a 32-year-old woman. PATIENT CONCERNS: The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. She had been diagnosed with Sjögren's syndrome at the age of 14 years, based on histopathological examination of a biopsy of the minor salivary glands and the development of Raynaud's phenomenon, with no follow-up treatment required. On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly. A bone marrow examination revealed increased megakaryocytes with reticulin fibrosis, and the histopathology of an axillary lymph node was consistent with mixed-type Castleman disease. Eventually, she developed thrombocytopenia. INTERVENTIONS: Her symptoms fulfilled all of the major and minor categories of the diagnostic criteria for TAFRO syndrome. However, considering her prior diagnosis, we assumed that the clinical presentation was consistent with an acute exacerbation of Sjögren's syndrome. Unlike typical cases of TAFRO syndrome, the administration of relatively low-dose prednisolone relieved her symptoms. LESSONS: Differentiation between TAFRO syndrome and exacerbation of an autoimmune disease is clinically important, although this can be challenging. Identification of specific biomarkers for TAFRO syndrome would be clinically beneficial. CI - Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved. FAU - Fujimoto, Shino AU - Fujimoto S AD - Department of Hematology and Immunology, Kanazawa Medical University Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Daigaku, Uchinada, Ishikawa-ken, Japan. FAU - Kawabata, Hiroshi AU - Kawabata H FAU - Kurose, Nozomu AU - Kurose N FAU - Kawanami-Iwao, Haruka AU - Kawanami-Iwao H FAU - Sakai, Tomoyuki AU - Sakai T FAU - Kawanami, Takafumi AU - Kawanami T FAU - Fujita, Yoshimasa AU - Fujita Y FAU - Fukushima, Toshihiro AU - Fukushima T FAU - Masaki, Yasufumi AU - Masaki Y LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) RN - Multi-centric Castleman's Disease SB - IM MH - Adult MH - Castleman Disease/*diagnosis/drug therapy MH - Diagnosis, Differential MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Prednisolone/therapeutic use MH - Sjogren's Syndrome/*diagnosis PMC - PMC5815761 COIS- The authors have no conflicts of interest to disclose. EDAT- 2018/02/03 06:00 MHDA- 2018/02/13 06:00 PMCR- 2017/12/15 CRDT- 2018/02/03 06:00 PHST- 2018/02/03 06:00 [entrez] PHST- 2018/02/03 06:00 [pubmed] PHST- 2018/02/13 06:00 [medline] PHST- 2017/12/15 00:00 [pmc-release] AID - 00005792-201712150-00099 [pii] AID - MD-D-17-04462 [pii] AID - 10.1097/MD.0000000000009220 [doi] PST - ppublish SO - Medicine (Baltimore). 2017 Dec;96(50):e9220. doi: 10.1097/MD.0000000000009220. PMID- 30197602 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231103 IS - 1664-042X (Print) IS - 1664-042X (Electronic) IS - 1664-042X (Linking) VI - 9 DP - 2018 TI - The Role of Oxidative Stress in the Development of Systemic Sclerosis Related Vasculopathy. PG - 1177 LID - 10.3389/fphys.2018.01177 [doi] LID - 1177 AB - Systemic sclerosis (SSc) is a rare connective tissue disease characterized by autoimmunity, vasculopathy, and progressive fibrosis typically affecting multiple organs including the skin. SSc often is a lethal disorder, because effective disease-modifying treatment still remains unavailable. Vasculopathy with endothelial dysfunction, perivascular infiltration of mononuclear cells, vascular wall remodeling and rarefaction of capillaries is the hallmark of the disease. Most patients present with vasospastic attacks of the digital arteries referred to as 'Raynaud's phenomenon,' which is often an indication of an underlying widespread vasculopathy. Although autoimmune responses and inflammation are both found to play an important role in the pathogenesis of this vasculopathy, no definite initiating factors have been identified. Recently, several studies have underlined the potential role of oxidative stress in the pathogenesis of SSc vasculopathy thereby proposing a new aspect in the pathogenesis of this disease. For instance, circulating levels of reactive oxygen species (ROS) related markers have been found to correlate with SSc vasculopathy, the formation of fibrosis and the production of autoantibodies. Excess ROS formation is well-known to lead to endothelial cell (EC) injury and vascular complications. Collectively, these findings suggest a potential role of ROS in the initiation and progression of SSc vasculopathy. In this review, we present the background of oxidative stress related processes (e.g., EC injury, autoimmunity, inflammation, and vascular wall remodeling) that may contribute to SSc vasculopathy. Finally, we describe the use of oxidative stress related read-outs as clinical biomarkers of disease activity and evaluate potential anti-oxidative strategies in SSc. FAU - Abdulle, Amaal E AU - Abdulle AE AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, Groningen, Netherlands. FAU - Diercks, Gilles F H AU - Diercks GFH AD - Section Pathology, Department of Pathology and Medical Biology, University Medical Centre Groningen, University of Groningen, Groningen, Netherlands. FAU - Feelisch, Martin AU - Feelisch M AD - Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom. FAU - Mulder, Douwe J AU - Mulder DJ AD - Department of Internal Medicine, Division of Vascular Medicine, University Medical Centre Groningen, University of Groningen, Groningen, Netherlands. FAU - van Goor, Harry AU - van Goor H AD - Section Pathology, Department of Pathology and Medical Biology, University Medical Centre Groningen, University of Groningen, Groningen, Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20180824 PL - Switzerland TA - Front Physiol JT - Frontiers in physiology JID - 101549006 PMC - PMC6117399 OTO - NOTNLM OT - biomarker OT - development OT - intervention OT - reactive oxygen species OT - systemic sclerosis OT - vasculopathy EDAT- 2018/09/11 06:00 MHDA- 2018/09/11 06:01 PMCR- 2018/08/24 CRDT- 2018/09/11 06:00 PHST- 2018/03/05 00:00 [received] PHST- 2018/08/06 00:00 [accepted] PHST- 2018/09/11 06:00 [entrez] PHST- 2018/09/11 06:00 [pubmed] PHST- 2018/09/11 06:01 [medline] PHST- 2018/08/24 00:00 [pmc-release] AID - 10.3389/fphys.2018.01177 [doi] PST - epublish SO - Front Physiol. 2018 Aug 24;9:1177. doi: 10.3389/fphys.2018.01177. eCollection 2018. PMID- 21779708 OWN - NLM STAT- MEDLINE DCOM- 20111208 LR - 20120508 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 51 IP - 4 DP - 2011 Jul-Aug TI - Autoantibody profile and clinical correlation in a group of patients with systemic sclerosis in southern Brazil. PG - 314-8, 323-4 LID - S0482-50042011000400004 [pii] AB - OBJECTIVES: To assess the manifestations of systemic sclerosis (SSc), with an emphasis on the analysis of autoantibodies and their clinical correlations, in a population of patients followed up at the SSc Outpatient Clinics of the Hospital de Clínicas of the Universidade Federal do Paraná. METHODOLOGY: Cross-sectional study with 96 patients followed up at the SSc Outpatient Clinics of the hospital between September 2007 and September 2009. RESULTS: Most patients were of the female sex, in their forties or fifties, and the median time of disease was ten years. The limited cutaneous form of SSc was more prevalent. The analysis of the autoantibodies showed the association of anticentromere antibody (ACA) with the following: the limited form of SSc; more advanced age at the time of diagnosis; longer disease time; longer interval between the appearance of the Raynaud's phenomenon (RyP) and the first non-RyP symptom; systemic arterial hypertension (SAH); and cardiac conduction blocks. The antitopoisomerase-1 antibody (ATA-1, previously called anti-Scl-70) was more common in the presence of the diffuse form of SSc, active disease, and digital ulcers. The anti-RNA polymerase III antibody (anti-Pol III) correlated with the diffuse form of SSc, disease activity, and synovitis. CONCLUSIONS: This study emphasizes and confirms the important role of autoantibodies in assessing patients with SSc, allowing the correlation between the autoimmune profile of patients with SSc and specific manifestations of the disease. FAU - Müller, Carolina de Souza AU - Müller Cde S AD - The Systemic Sclerosis Outpatient Clinic of the HC-UFPR. carolinadesmuller@yahoo.com.br FAU - Paiva, Eduardo Dos Santos AU - Paiva Edos S FAU - Azevedo, Valderílio Feijó AU - Azevedo VF FAU - Radominski, Sebastião Cezar AU - Radominski SC FAU - Lima Filho, José Hermênio Cavalcante AU - Lima Filho JH LA - eng LA - por PT - Journal Article PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/*blood MH - Brazil MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*blood/*immunology EDAT- 2011/07/23 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/07/23 06:00 PHST- 2011/01/17 00:00 [received] PHST- 2011/04/30 00:00 [accepted] PHST- 2011/07/23 06:00 [entrez] PHST- 2011/07/23 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - S0482-50042011000400004 [pii] PST - ppublish SO - Rev Bras Reumatol. 2011 Jul-Aug;51(4):314-8, 323-4. PMID- 33603610 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220420 IS - 1642-395X (Print) IS - 2299-0046 (Electronic) IS - 1642-395X (Linking) VI - 37 IP - 6 DP - 2020 Dec TI - Circulating peroxisome proliferator-activated receptor γ is elevated in systemic sclerosis. PG - 921-926 LID - 10.5114/ada.2019.84746 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune connective tissue disease with distinguished fibrosis of the skin and internal organs. Vascular damage, immune dysregulation and fibroblasts activation contribute to SSc pathogenesis. Peroxisome proliferator-activated receptor γ (PPAR-γ) can be a link between cell metabolism and fibrosis in SSc due to its anti-fibrotic and immunomodulatory properties. AIM: To measure the serum level of PPAR-γ in SSc patients and correlate it with the SSc subtype, hs-CRP, disease duration, vascular and internal organ involvement. MATERIAL AND METHODS: Twenty-two SSc patients (15 limited SSc, 7 diffuse SSc) matched with healthy controls were analysed. Clinical and laboratory data were collected including specific antibodies, interstitial lung disease, oesophageal involvement, digital pitting scars, disease duration, Raynaud's phenomenon (RP) and modified Rodnan skin score (mRSS). PPAR-γ levels were analysed by ELISA. Statistical analysis was performed with χ(2), Student's t-test and Mann-Whitney-U test. Pearson and Spearman correlation analyses were used to establish variables association. The significance threshold was set at p < 0.05. RESULTS: PPAR-γ concentration was elevated in SSc patients in comparison to controls (p = 0.007) with the highest difference for diffuseSSc (p = 0.004) with significantly elevated mRSS. No association between PPAR-γ levels and hs-CRP, internal organ and vascular involvement, disease duration, autoantibodies and RP onset was found. CONCLUSIONS: The present study revealed elevated serum PPAR-γ in SSc patients, in particular those with a diffuse form, presenting highest mRSS and lowest BMI. Whether circulating PPAR-γ originates from atrophic adipose tissue, reperfused vessels or ischemic tissues needs assessing. Also the biological meaning or effect of elevated serum PPAR-γ requires further studies. CI - Copyright: © 2019 Termedia Sp. z o. o. FAU - Żółkiewicz, Jakub AU - Żółkiewicz J AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Stochmal, Anna AU - Stochmal A AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Zaremba, Michał AU - Zaremba M AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Rudnicka, Lidia AU - Rudnicka L AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Czuwara, Joanna AU - Czuwara J AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. LA - eng PT - Journal Article DEP - 20190426 PL - Poland TA - Postepy Dermatol Alergol JT - Postepy dermatologii i alergologii JID - 101168357 PMC - PMC7874880 OTO - NOTNLM OT - fibrosis OT - organ involvement OT - peroxisome proliferator-activated receptor γ OT - pits OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2021/02/20 06:00 MHDA- 2021/02/20 06:01 PMCR- 2020/12/01 CRDT- 2021/02/19 06:05 PHST- 2019/03/11 00:00 [received] PHST- 2019/04/27 00:00 [accepted] PHST- 2021/02/19 06:05 [entrez] PHST- 2021/02/20 06:00 [pubmed] PHST- 2021/02/20 06:01 [medline] PHST- 2020/12/01 00:00 [pmc-release] AID - 36492 [pii] AID - 10.5114/ada.2019.84746 [doi] PST - ppublish SO - Postepy Dermatol Alergol. 2020 Dec;37(6):921-926. doi: 10.5114/ada.2019.84746. Epub 2019 Apr 26. PMID- 32956337 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 21 DP - 2020 Sep 10 TI - Autoimmune Myelofibrosis in Sjögren's Syndrome: Report of a Case. PG - e924983 LID - 10.12659/AJCR.924983 [doi] AB - BACKGROUND Autoimmune myelofibrosis (AMF) is a rare clinicopathologic entity of bone marrow fibrosis that occurs in association with autoimmune disorders. Steroids are very effective for treatment of AMF and the disease has a good prognosis and should be distinguished from primary myelofibrosis. CASE REPORT A 49-year-old man with bleeding and petechial hemorrhage of the extremities presented to our institution. His platelet count was 1×10⁹/L. Bone marrow aspiration revealed a dry tap, and bone marrow biopsy confirmed small lymphocyte infiltration and increased reticular fibers, consistent with immune thrombocytopenia. Testing for mutations in JAK2, MPL, and CALR was negative. Because the patient had a history of Raynaud's phenomenon, he was suspected to have collagen disease. Anti-Sjögren's-syndrome-related antigen-A antibody testing, Schirmer's test, and fluorescein staining all came back positive, which led to a diagnosis of Sjögren's syndrome. Given the bone marrow findings, the patient also was diagnosed with AMF. Treatment with steroids resulted in an immediate improvement in his platelet count. CONCLUSIONS In the present case, treatment with steroids resulted in prompt improvement in platelet counts and subsequent marrow biopsy showed MF-0 reticulin fibrosis. Bone marrow fibrosis rarely is seen in association with autoimmune disease, and its significance and mechanism are still to be determined. FAU - Kakiuchi, Seiji AU - Kakiuchi S AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. FAU - Takagi, Ikumi AU - Takagi I AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. FAU - Akiyama, Hiroaki AU - Akiyama H AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. FAU - Matsuba, Hiroyuki AU - Matsuba H AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. FAU - Rikitake, Junpei AU - Rikitake J AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. FAU - Kajimoto, Kazuyoshi AU - Kajimoto K AD - Department of Pathology, Hyogo Cancer Center, Akashi, Hyogo, Japan. FAU - Hayashi, Yoshitake AU - Hayashi Y AD - Division of Molecular Medicine and Medical Genetics, Department of Pathology, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan. FAU - Iwata, Nobuko AU - Iwata N AD - Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20200910 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - *Autoimmune Diseases/diagnosis MH - Bone Marrow MH - Humans MH - Male MH - Middle Aged MH - *Primary Myelofibrosis/complications/diagnosis MH - *Sjogren's Syndrome/complications/diagnosis MH - *Thrombocytopenia PMC - PMC7505477 COIS- Conflict of interest: None declared Conflicts of interest None. EDAT- 2020/09/22 06:00 MHDA- 2021/05/15 06:00 PMCR- 2020/09/10 CRDT- 2020/09/21 17:14 PHST- 2020/09/21 17:14 [entrez] PHST- 2020/09/22 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/09/10 00:00 [pmc-release] AID - 924983 [pii] AID - 10.12659/AJCR.924983 [doi] PST - epublish SO - Am J Case Rep. 2020 Sep 10;21:e924983. doi: 10.12659/AJCR.924983. PMID- 20387195 OWN - NLM STAT- MEDLINE DCOM- 20110110 LR - 20100806 IS - 1438-8790 (Electronic) IS - 0934-8387 (Linking) VI - 64 IP - 8 DP - 2010 Aug TI - [The anti-Jo-1 syndrome - a specific form of myositis with interstitial lung disease]. PG - 496-503 LID - 10.1055/s-0029-1244054 [doi] AB - Interstitial lung disease (ILD) may be associated with systemic inflammatory disorders and autoantibody production. The development of ILD has been described in detail in patients with polymyositis and dermatomyositis. Anti-synthetase antibodies, including anti-Jo-1 antibodies (antihistidyl-tRNA syntase), are found in up to 35 % of patients with myositis, 80 % of which constitute anti-Jo-1 antibodies. The anti-Jo-1 syndrome characteristically presents with myositis, shortness of breath, fever, polyarthritis/arthralgia, mechanic's hands, dermatomyositis-like skin lesions, signs of a connective tissue disease and/or Raynaud's phenomenon. ILD is an early diagnostic sign and shows focal infiltrates and an acinar pattern in CT scan. Non-specific interstitial pneumonitis with T-lymphocytic infiltrates in lung histology (VATS) or elevated IFN-gamma-inducible chemokines are further indicators for anti-Jo-1 syndrome. Corticosteroids eventually combined with an immunosuppressant drug are often required with reported beneficial effects, although not many therapeutic studies have been performed. Here we present a review of the current literature and a case report on anti-Jo-1 syndrome. CI - Georg Thieme Verlag KG Stuttgart. New York. FAU - Häussermann, A AU - Häussermann A AD - Robert-Koch-Klinik, Thoraxzentrum des Klinikums St. Georg, Leipzig. anja.haeussermann@gmx.de FAU - Gillissen, A AU - Gillissen A FAU - Seidel, W AU - Seidel W LA - ger PT - English Abstract PT - Journal Article TT - Das Anti-Jo-1-Syndrom - eine Sonderform der Myositis mit interstitieller Lungenerkrankung. DEP - 20100412 PL - Germany TA - Pneumologie JT - Pneumologie (Stuttgart, Germany) JID - 8906641 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Anti-Inflammatory Agents/therapeutic use MH - Antibodies, Antinuclear/*immunology MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/*drug therapy/*immunology MH - Myositis/*drug therapy/*immunology MH - Syndrome EDAT- 2010/04/14 06:00 MHDA- 2011/01/11 06:00 CRDT- 2010/04/14 06:00 PHST- 2010/04/14 06:00 [entrez] PHST- 2010/04/14 06:00 [pubmed] PHST- 2011/01/11 06:00 [medline] AID - 10.1055/s-0029-1244054 [doi] PST - ppublish SO - Pneumologie. 2010 Aug;64(8):496-503. doi: 10.1055/s-0029-1244054. Epub 2010 Apr 12. PMID- 29632291 OWN - NLM STAT- MEDLINE DCOM- 20190510 LR - 20190510 IS - 1881-6096 (Print) IS - 1881-6096 (Linking) VI - 70 IP - 4 DP - 2018 Apr TI - [Idiopathic Inflammatory Myopathy and Anti-aminoacyl-tRNA Synthetase Antibody]. PG - 439-448 LID - 10.11477/mf.1416201016 [doi] AB - Anti-aminoacyl-tRNA synthetase (ARS) antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-KS, anti-OJ, anti-Ha, and anti-Zo antibodies) are found in 25 to 40% of myositis patients. The patients with these antibodies have anti-synthetase syndrome with one or more of the following clinical features: myositis, interstitial lung disease, arthritis, fever, Raynaud's phenomenon, and mechanic's hands. In Japan, health insurance coverage of treatments for patients in whom the "anti-ARS antibodies," anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, and anti-KS are detected by enzyme-linked immunosorbent assay was approved by the Ministry of Health, Labour and Welfare in 2014. Recently, clinical features have been discovered to be heterogeneous. Patients with the anti-PL-7, anti-PL-12, anti-KS, and anti-OJ antibodies exhibit interstitial lung disease rather than myositis. Interstitial lung disease is related to the prognosis of this syndrome. Regarding histopathological findings of the muscle, perimysial connective tissue fragmentation with positive staining for alkaline phosphatase is the characteristic feature. Myonuclear actin filament inclusions are also detected. A recent work demonstrated that immunization of mice with histidyl-tRNA synthetase results in muscle inflammation consistent with myositis. These findings promote understanding of the pathological mechanism of the development of myositis associated with anti-ARS antibodies. FAU - Maeda, Meiko Hashimoto AU - Maeda MH AD - Department of Neurology, Federation of National Public Service Personnel Mutual Aid Associations Toranomon Hospital Kajigaya. LA - jpn PT - Journal Article PL - Japan TA - Brain Nerve JT - Brain and nerve = Shinkei kenkyu no shinpo JID - 101299709 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Animals MH - Autoantibodies/*immunology MH - Humans MH - Japan MH - Lung Diseases, Interstitial/*immunology MH - Mice MH - Myositis/diagnosis/*immunology EDAT- 2018/04/11 06:00 MHDA- 2019/05/11 06:00 CRDT- 2018/04/11 06:00 PHST- 2018/04/11 06:00 [entrez] PHST- 2018/04/11 06:00 [pubmed] PHST- 2019/05/11 06:00 [medline] AID - 1416201016 [pii] AID - 10.11477/mf.1416201016 [doi] PST - ppublish SO - Brain Nerve. 2018 Apr;70(4):439-448. doi: 10.11477/mf.1416201016. PMID- 41780955 OWN - NLM STAT- In-Process DCOM- 20260306 LR - 20260609 IS - 0485-1439 (Print) IS - 0485-1439 (Linking) VI - 67 IP - 2 DP - 2026 TI - [Pure red cell aplasia in a patient with cold agglutinin disease-associated lymphoproliferative disorder]. PG - 118-123 LID - 10.11406/rinketsu.67.118 [doi] AB - A 51-year-old woman had been noted to have anemia since 2019 and developed Raynaud's phenomenon during winter or in air-conditioned environments from 2021. In January 2024, she experienced palpitations, tinnitus, dyspnea on exertion, and fatigue. Twenty-three days later, she was urgently admitted to hospital with severe anemia (Hb 2.8 g/dl). She was diagnosed with primary cold agglutinin disease (CAD) and CAD-associated lymphoproliferative disorder (LPD). While she was under observation, undergoing red blood cell transfusion with avoidance of cold exposure, her anemia progressed without worsening of hemolysis and she was diagnosed with pure red cell aplasia (PRCA). Cyclosporine A (CsA) was administered for 10 days without response, and treatment was switched to bendamustine-rituximab (BR) for LPD. Although her LPD improved, her PRCA persisted; CsA was therefore resumed, leading to improvement in the PRCA. In October, her CAD-LPD relapsed with hemolysis, and BR therapy with continued CsA treatment again achieved improvement in the CAD-LPD without worsening of the PRCA. This rare case of primary CAD-associated LPD complicated by PRCA highlights the importance of accurate disease assessment and appropriate treatment strategies. FAU - Ogawa, Makiko AU - Ogawa M AD - Department of Hematology, The Fraternity Memorial Hospital. FAU - Osada, Yuki AU - Osada Y AD - Department of Hematology, The Fraternity Memorial Hospital. FAU - Tomori, Aina AU - Tomori A AD - Department of Hematology, The Fraternity Memorial Hospital. FAU - Mizuki, Taro AU - Mizuki T AD - Department of Hematology, The Fraternity Memorial Hospital. FAU - Tanosaki, Sakae AU - Tanosaki S AD - Department of Hematology, The Fraternity Memorial Hospital. FAU - Suzuki, Ken AU - Suzuki K AD - Department of Hematology, The Fraternity Memorial Hospital. LA - jpn PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Ketsueki JT - [Rinsho ketsueki] The Japanese journal of clinical hematology JID - 2984782R RN - 981Y8SX18M (Bendamustine Hydrochloride) SB - IM MH - Humans MH - *Anemia, Hemolytic, Autoimmune/complications MH - Female MH - *Lymphoproliferative Disorders/complications/etiology MH - Middle Aged MH - *Red-Cell Aplasia, Pure/etiology/therapy/diagnosis/complications MH - Bendamustine Hydrochloride/administration & dosage OTO - NOTNLM OT - BR therapy OT - Cold agglutinin disease OT - Cyclosporine OT - Pure red cell aplasia EDAT- 2026/03/05 00:39 MHDA- 2026/03/07 01:37 CRDT- 2026/03/04 20:13 PHST- 2026/03/07 01:37 [medline] PHST- 2026/03/05 00:39 [pubmed] PHST- 2026/03/04 20:13 [entrez] AID - 10.11406/rinketsu.67.118 [doi] PST - ppublish SO - Rinsho Ketsueki. 2026;67(2):118-123. doi: 10.11406/rinketsu.67.118. PMID- 25620556 OWN - NLM STAT- MEDLINE DCOM- 20151228 LR - 20150309 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 142 IP - 3 DP - 2015 Mar TI - ["Mechanic's hands" during antisynthetase syndrome: a marker of systemic involvement?]. PG - 189-92 LID - S0151-9638(14)01281-2 [pii] LID - 10.1016/j.annder.2014.11.003 [doi] AB - BACKGROUND: Antisynthetase syndrome is characterized by the presence of interstitial lung disease, inflammatory myopathy, joint disease, Raynaud's phenomenon and characteristic skin lesions of the hands known as "mechanic's hands" associated with the presence of serum antibody to aminoacyl transfer-RNA synthetases. We report the case of a patient in whom cutaneous relapse consistently preceded CT evidence of pulmonary fibrosis flare-up. PATIENT AND METHODS: A 56-year-old patient consulted for recent exertional dyspnoea and dry cough. Clinical examination showed the skin of the tips and edges of the fingers to be thickened, hyperkeratotic and fissured. High-resolution computed tomography thoracic scans revealed interstitial lung lesions affecting both lower lobes. Lab tests showed elevated serum creatine kinase and positivity for anti-Jo-1 antibodies. Response to treatment with corticosteroids and cyclophosphamide was marked by an improvement in symptoms and regression of radiological anomalies together with disappearance of cutaneous signs. Nine months later, after changeover from cyclophosphamide to mycophenolate mofetil, cutaneous relapse and flare of interstitial lung disease were observed. A second course of cyclophosphamide followed by azathioprine resulted in regression of the cutaneous and pulmonary lesions. CONCLUSION: Mechanic's hands may be considered a marker of visceral involvement and should be sought in any instance of suspected antisynthetase syndrome flare-up. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Nahas, O AU - Nahas O AD - Service de médecine interne, Hôtel-Dieu de France, Achrafieh, rue Adib Ishac, Beyrouth, Liban. Electronic address: olga.nahas@gmail.com. FAU - Haddad, F AU - Haddad F AD - Service de médecine interne, Hôtel-Dieu de France, Achrafieh, rue Adib Ishac, Beyrouth, Liban. FAU - Maalouly, G AU - Maalouly G AD - Service de médecine interne, Hôtel-Dieu de France, Achrafieh, rue Adib Ishac, Beyrouth, Liban. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Les « mains de mécanicien » au cours du syndrome des anti-synthétases : un marqueur d'atteinte systémique ? DEP - 20150122 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - Antisynthetase syndrome SB - IM MH - Hand Dermatoses/*etiology MH - Humans MH - Male MH - Middle Aged MH - Myositis/*complications/diagnosis OTO - NOTNLM OT - Aminoacyl transfer-RNA synthetase antibodies OT - Anti-Jo-1 antibodies OT - Anti-synthétases (syndrome des) OT - Anticorps anti-Jo-1 OT - Anticorps anti-aminoacyl-tRNA synthétases OT - Antisynthetase syndrome OT - Fibrose pulmonaire OT - Interstitial lung disease OT - Mains de mécanicien OT - Mechanic's hands OT - Pneumopathie interstitielle EDAT- 2015/01/27 06:00 MHDA- 2015/12/29 06:00 CRDT- 2015/01/27 06:00 PHST- 2014/03/15 00:00 [received] PHST- 2014/06/23 00:00 [revised] PHST- 2014/11/07 00:00 [accepted] PHST- 2015/01/27 06:00 [entrez] PHST- 2015/01/27 06:00 [pubmed] PHST- 2015/12/29 06:00 [medline] AID - S0151-9638(14)01281-2 [pii] AID - 10.1016/j.annder.2014.11.003 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2015 Mar;142(3):189-92. doi: 10.1016/j.annder.2014.11.003. Epub 2015 Jan 22. PMID- 38238136 OWN - NLM STAT- MEDLINE DCOM- 20240214 LR - 20240214 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 45 IP - 2 DP - 2024 Feb TI - [Autologous peripheral stem cell transplantation in systemic sclerosis: An important step forward, but we must temper our enthusiasm!]. PG - 100-103 LID - S0248-8663(24)00002-X [pii] LID - 10.1016/j.revmed.2024.01.001 [doi] AB - Three prospective randomized studies have demonstrated the efficacy of autologous hematopoietic stem cell (HSC) transplantation in systemic sclerosis (SSc) on survival. These results encourage us to offer this therapy to patients who have a rapidly progressive disease and who have early symptoms but no advanced visceral involvement. HSC autograft can thus be discussed in patients with diffuse cutaneous SSc with a duration of the disease since the first visceral manifestations (cutaneous, cardiac, digestive, pulmonary, or renal) excluding Raynaud's phenomenon of less than 5 years. However, the indications for HSC autograft in SSc validated at European level and in the national diagnostic and care protocol (PNDS) are broader and some of these indications are debatable, in particular in patients with worsening diffuse interstitial lung disease. These indications are discussed in a reasoned way, taking into account the level of evidence and the toxicity of the HSC autograft. CI - Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Mouthon, L AU - Mouthon L AD - Service de médecine interne, centre de référence maladies autoimmunes et autoinflammatoires systémiques rares d'Ile de France, de l'Est et de l'Ouest, hôpital Cochin, Assistance Publique-hôpitaux de Paris (AP-HP), hôpital Cochin, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Université Paris Cité, AP-HP-CUP, Hôpital Cochin, 75014 Paris, France. Electronic address: luc.mouthon@aphp.fr. LA - fre PT - English Abstract PT - Journal Article TT - Autogreffe de cellules souches périphériques dans la sclérodermie systémique : un progrès important, mais il faut tempérer notre enthousiasme ! DEP - 20240118 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 SB - IM MH - Humans MH - *Peripheral Blood Stem Cell Transplantation MH - Prospective Studies MH - *Scleroderma, Systemic/diagnosis/therapy MH - *Hematopoietic Stem Cell Transplantation/adverse effects MH - Transplantation, Autologous OTO - NOTNLM OT - Interstitial Lung Disease OT - Morbidity OT - Mortality OT - Peripheral Stem Cell Transplantation OT - Systemic scleroderma OT - autogreffe de cellules souches hématopoïétiques OT - morbidité OT - mortalité OT - pneumopathie interstitielle diffuse OT - sclérodermie systémique diffuse EDAT- 2024/01/19 00:42 MHDA- 2024/02/10 14:45 CRDT- 2024/01/18 21:53 PHST- 2024/02/10 14:45 [medline] PHST- 2024/01/19 00:42 [pubmed] PHST- 2024/01/18 21:53 [entrez] AID - S0248-8663(24)00002-X [pii] AID - 10.1016/j.revmed.2024.01.001 [doi] PST - ppublish SO - Rev Med Interne. 2024 Feb;45(2):100-103. doi: 10.1016/j.revmed.2024.01.001. Epub 2024 Jan 18. PMID- 34583781 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211001 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 5 IP - 1 DP - 2021 Sep 29 TI - Adult onset Still's disease in a patient with scleroderma: case report. PG - 44 LID - 10.1186/s41927-021-00212-4 [doi] LID - 44 AB - BACKGROUND: Scleroderma and adult onset Still's disease (AOSD) are both uncommon autoimmune disorders. These two disorders have rarely been documented to occur simultaneously. In fact, after a thorough literature review, we discovered only one prior case report in a pregnant individual. Here, we describe the first documented case of scleroderma and AOSD in a postmenopausal patient. CASE PRESENTATION: The patient is a 61-year-old Caucasian female with a past medical history significant for peptic ulcer disease, mitral valve prolapse, chronic idiopathic pancreatitis, and limited cutaneous scleroderma with sclerodactyly, Raynaud's, and calcinosis. She was sent to the emergency room by her primary care physician due to one-week history of intermittent spiking fevers (Tmax 101°F), sore throat, myalgias, arthralgias, and non-pruritic bilateral lower extremity rash. Diagnostic evaluation in the hospital included complete blood count, comprehensive metabolic panel, respiratory viral panel, antinuclear antibody panel, bone marrow biopsy, and imaging with computerized tomography. Our patient fulfilled Yamaguchi Criteria for AOSD and all other possible etiologies were ruled out. She was treated with a steroid taper and methotrexate was initiated on post-discharge day number fourteen. Clinical and biochemical resolution was obtained at three months. CONCLUSIONS: In this report, we describe the first ever documented case of scleroderma and AOSD in a postmenopausal patient. The clinical presentation, diagnostic work up, and management discussed herein may serve as a framework for which rheumatologists and other physicians may draw upon in similar future encounters. CI - © 2021. The Author(s). FAU - Brow, Jeffrey D AU - Brow JD AUID- ORCID: 0000-0002-4712-505X AD - Department of Medicine, NorthShore University HealthSystem, 2650 Ridge Avenue, Evanston, IL, 60201, USA. JBrow@northshore.org. FAU - Zhu, Daisy AU - Zhu D AD - Department of Medicine, NorthShore University HealthSystem, 2650 Ridge Avenue, Evanston, IL, 60201, USA. FAU - Drevlow, Barbara E AU - Drevlow BE AD - Division of Rheumatology, Department of Medicine, NorthShore University HealthSystem, Evanston, IL, USA. LA - eng PT - Journal Article DEP - 20210929 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC8480026 OTO - NOTNLM OT - Adult-Onset OT - Autoimmune Diseases OT - Case report OT - Limited OT - Scleroderma OT - Still's Disease COIS- JB, DZ, and BD report no conflicts of interest. EDAT- 2021/09/30 06:00 MHDA- 2021/09/30 06:01 PMCR- 2021/09/29 CRDT- 2021/09/29 05:43 PHST- 2020/11/24 00:00 [received] PHST- 2021/06/30 00:00 [accepted] PHST- 2021/09/29 05:43 [entrez] PHST- 2021/09/30 06:00 [pubmed] PHST- 2021/09/30 06:01 [medline] PHST- 2021/09/29 00:00 [pmc-release] AID - 10.1186/s41927-021-00212-4 [pii] AID - 212 [pii] AID - 10.1186/s41927-021-00212-4 [doi] PST - epublish SO - BMC Rheumatol. 2021 Sep 29;5(1):44. doi: 10.1186/s41927-021-00212-4. PMID- 31103501 OWN - NLM STAT- MEDLINE DCOM- 20200519 LR - 20200519 IS - 1532-8635 (Electronic) IS - 1524-9042 (Linking) VI - 20 IP - 4 DP - 2019 Aug TI - Common Causes of Pain in Systemic Sclerosis: Frequency, Severity, and Relationship to Disease Status, Depression, and Quality of Life. PG - 331-336 LID - S1524-9042(18)30299-6 [pii] LID - 10.1016/j.pmn.2019.02.006 [doi] AB - BACKGROUND: In routine clinical practice, healthcare professionals draw little attention to pain in patients with systemic sclerosis (SSc). Pain has adverse effects on functional ability, social and emotional wellbeing. AIMS: This study aims to assess the frequency and severity of different types of pain in patients with SSc, and the relationship of pain with disease status, depression and quality of life. DESIGN: Consecutive patients with SSc were included in this cross-sectional study. Patients with previously diagnosed painful diseases or conditions (other rheumatic diseases, angina pectoris, neurological disorders, etc.) were excluded. SETTINGS: Patients, who visited our rheumatology outpatient clinic from February to November 2016, participated in this study. PARTICIPANTS/SUBJECTS: 42 consecutive patients with SSc (38 women and 4 men), mean age 56.5 years, mean disease duration 9.5 years, were included. METHODS: All patients filled in a questionnaire, to indicate the presence or absence of some predefined pain syndromes. Disease status was assessed using the Scleroderma Assessment Questionnaire (SAQ), symptoms of depression by the Beck's Depression Inventory (BDI), whilst the quality of life was evaluated using the EuroQol questionnaire. RESULTS: It was found that 92.9% of SSc patients suffer from different types of pain, and 45.2% of patients have pain every day. Joint pain was the most common type of pain, present in 78.6% of patients, followed by pain associated with Raynaud's phenomenon (69%), back pain (47.6%), headache (31%), chest pain (23.8%), odynophagia (21.4%) and painful digital ulcers (19%). Symptoms of neuropathic pain were noticed in 26.2% of patients. Severe joint pain, everyday pain and symptoms of neuropathic pain in SSc were associated with more severe disease and poorer quality of life. Pain related to Raynaud's phenomenon, digital ulcers, odynophagia and joint pain were associated with significant symptoms of depression. CONCLUSION: The majority of patients with SSc suffer from different types of pain. Pain is associated with more severe disease, depression and poor quality of life. CI - Copyright © 2019 American Society for Pain Management Nursing. Published by Elsevier Inc. All rights reserved. FAU - Ostojic, Predrag AU - Ostojic P AD - Institute of Rheumatology, Belgrade, Serbia; School of Medicine, University of Belgrade, Serbia. Electronic address: drpedja74@hotmail.com. FAU - Jankovic, Katarina AU - Jankovic K AD - Institute of Rheumatology, Belgrade, Serbia. FAU - Djurovic, Nada AU - Djurovic N AD - Institute of Rheumatology, Belgrade, Serbia. FAU - Stojic, Biljana AU - Stojic B AD - Institute of Rheumatology, Belgrade, Serbia. FAU - Knezevic-Apostolski, Sladjana AU - Knezevic-Apostolski S AD - Neurology Outpatient Clinic Apostolski, Belgrade, Serbia. FAU - Bartolovic, Daniela AU - Bartolovic D AD - Center for Biochemistry, Clinical Center of Serbia, Belgrade, Serbia. LA - eng PT - Journal Article DEP - 20190515 PL - United States TA - Pain Manag Nurs JT - Pain management nursing : official journal of the American Society of Pain Management Nurses JID - 100890606 SB - IM MH - Adult MH - Aged MH - Depression/*etiology/psychology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain/classification/*etiology/psychology MH - Quality of Life/*psychology MH - Scleroderma, Systemic/*complications/psychology MH - Surveys and Questionnaires EDAT- 2019/05/20 06:00 MHDA- 2020/05/20 06:00 CRDT- 2019/05/20 06:00 PHST- 2018/07/04 00:00 [received] PHST- 2019/02/07 00:00 [revised] PHST- 2019/02/13 00:00 [accepted] PHST- 2019/05/20 06:00 [pubmed] PHST- 2020/05/20 06:00 [medline] PHST- 2019/05/20 06:00 [entrez] AID - S1524-9042(18)30299-6 [pii] AID - 10.1016/j.pmn.2019.02.006 [doi] PST - ppublish SO - Pain Manag Nurs. 2019 Aug;20(4):331-336. doi: 10.1016/j.pmn.2019.02.006. Epub 2019 May 15. PMID- 21794601 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20110728 IS - 1699-258X (Print) IS - 1699-258X (Linking) VI - 5 IP - 4 DP - 2009 Jul-Aug TI - [Clinical characteristics of children with scleroderma in a referral hospital]. PG - 158-62 LID - 10.1016/j.reuma.2008.11.018 [doi] AB - INTRODUCTION: Scleroderma is an autoimmune disease that involves the connective tissue characterized by skin fibrosis, classified as localized and systemic (participation of one or more internal organs). The primary objective of this study is to describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at a referral hospital. MATERIAL AND METHODS: Extraction of data from clinical charts of children with scleroderma in the rheumatology department at the Hospital Infantil de México Federico Gómez, between January 2000 and December 2007. RESULTS: Sixty two patients were included in the group. All of them completed the classification criteria for juvenile sclerodema, both systemic and localized. The mean age at diagnosis was 7.8 (1-14) years. The mean time from disease onset to diagnosis, based on clinical manifestations, was 23 months. The lesions found were: linear scleroderma (42%), mixed morphea (22%), circumscribed morphea (19%), generalized morphea (13%) and panclerotic morphea (4%). Involvement associated with Systemic Scleroderma was gastrointestinal 100% (18 patients), pulmonary 100% (18/18), Raynaud's phenomenon 89% (16/18), proximal sclerosis 89% (16/18), sclerodactilia 67% (12/18), joint pain 28% (5/18), calcinosis 56% (10/18). Positive antinuclear antibodies (ANA) were present in 14/62 (23%) patients (10 with systemic range and 4 localized), antiSCL 70 in 2/62 (4%) cases. The most common drug used was methotrexate. CONCLUSION: The most common skin lesions found were linear morphea, followed by the mixed and circumscribed types. In systemic scleroderma the most involved systems are the gastrointestinal, respiratory and vascular (associated with Raynaud's phenomenon). There is a special need for knowledge of this disease in first contact physicians for a faster and better diagnosis and treatment, in order to avoid complications. It is also necessary to improve resources in developing countries for complimentary studies, classification, treatment and follow-up. CI - Copyright © 2008 Elsevier España, S.L. All rights reserved. FAU - Morel, Zoilo AU - Morel Z AD - Departamento de Reumatología Pediátrica, Hospital Infantil de México Federico Gómez, México D. F., México. FAU - Benadón, Eduardo AU - Benadón E FAU - Faugier, Enrique AU - Faugier E FAU - Maldonado, María Del Rocío AU - Maldonado Mdel R LA - spa PT - English Abstract PT - Journal Article TT - Características clínicas de niños con esclerodermia en un hospital de referencia. DEP - 20090508 PL - Spain TA - Reumatol Clin JT - Reumatologia clinica JID - 101293923 EDAT- 2009/07/01 00:00 MHDA- 2009/07/01 00:01 CRDT- 2011/07/29 06:00 PHST- 2008/08/09 00:00 [received] PHST- 2008/11/16 00:00 [revised] PHST- 2008/11/19 00:00 [accepted] PHST- 2011/07/29 06:00 [entrez] PHST- 2009/07/01 00:00 [pubmed] PHST- 2009/07/01 00:01 [medline] AID - S1699-258X(09)00063-1 [pii] AID - 10.1016/j.reuma.2008.11.018 [doi] PST - ppublish SO - Reumatol Clin. 2009 Jul-Aug;5(4):158-62. doi: 10.1016/j.reuma.2008.11.018. Epub 2009 May 8. PMID- 29299961 OWN - NLM STAT- MEDLINE DCOM- 20180424 LR - 20180424 IS - 0301-1526 (Print) IS - 0301-1526 (Linking) VI - 47 IP - 2 DP - 2018 Feb TI - Erythromelalgia. PG - 91-97 LID - 10.1024/0301-1526/a000675 [doi] AB - Erythromelalgia is a rare syndrome characterized by the intermittent or, less commonly, by the permanent occurrence of extremely painful hyperperfused skin areas mainly located in the distal extremities. Primary erythromelalgia is nowadays considered to be a genetically determined neuropathic disorder affecting SCN9A, SCN10A, and SCN11A coding for NaV1.7, NaV1.8, and NaV1.9 neuronal sodium channels. Secondary forms might be associated with myeloproliferative disorders, connective tissue disease, cancer, infections, and poisoning. Between the pain episodes, the affected skin areas are usually asymptomatic, but there are patients with typical features of acrocyanosis and/or Raynaud's phenomenon preceding or occurring in between the episodes of erythromelalgia. Diagnosis is made by ascertaining the typical clinical features. Thereafter, the differentiation between primary and secondary forms should be made. Genetic testing is recommended, especially in premature cases and in cases of family clustering in specialized genetic institutions after genetic counselling. Multimodal therapeutic intervention aims toward attenuation of pain and improvement of the patient's quality of life. For this purpose, a wide variety of nonpharmacological approaches and pharmacological substances for topical and systemic use have been proposed, which are usually applied individually in a step-by-step approach. Prognosis mainly depends on the underlying condition and the ability of the patients and their relatives to cope with the disease. FAU - Klein-Weigel, Peter Franz AU - Klein-Weigel PF AD - 1 Klinik für Angiologie, Helios Klinik Berlin-Buch, Berlin, Germany. FAU - Volz, Theresa Sophie AU - Volz TS AD - 1 Klinik für Angiologie, Helios Klinik Berlin-Buch, Berlin, Germany. FAU - Richter, Jutta Gisela AU - Richter JG AD - 2 Poliklinik, Funktionsbereich und Hiller Forschungszentrum für Rheumatologie, Medizinische Fakultät, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany. LA - eng PT - Journal Article PT - Review DEP - 20180104 PL - Switzerland TA - Vasa JT - VASA. Zeitschrift fur Gefasskrankheiten JID - 0317051 SB - IM MH - *Erythromelalgia/diagnosis/epidemiology/genetics/therapy MH - Genetic Predisposition to Disease MH - Humans MH - Molecular Diagnostic Techniques MH - Pain Measurement MH - Phenotype MH - Predictive Value of Tests MH - Risk Factors MH - Treatment Outcome OTO - NOTNLM OT - Erythromelalgia OT - chronic pain syndrome small fibre polyneuropathy. OT - erythermalgia OT - genetic pain syndrome OT - vascular acrosyndrome EDAT- 2018/01/05 06:00 MHDA- 2018/04/25 06:00 CRDT- 2018/01/05 06:00 PHST- 2018/01/05 06:00 [pubmed] PHST- 2018/04/25 06:00 [medline] PHST- 2018/01/05 06:00 [entrez] AID - 10.1024/0301-1526/a000675 [doi] PST - ppublish SO - Vasa. 2018 Feb;47(2):91-97. doi: 10.1024/0301-1526/a000675. Epub 2018 Jan 4. PMID- 23563250 OWN - NLM STAT- MEDLINE DCOM- 20131022 LR - 20151119 IS - 1533-0311 (Electronic) IS - 0193-1091 (Linking) VI - 35 IP - 4 DP - 2013 Jun TI - Scleroderma and IgG4-related disease. PG - 458-62 LID - 10.1097/DAD.0b013e318276cbac [doi] AB - IgG4-related disease is a syndrome which involves lymphoplasmacytic infiltrates and soft tissue sclerosis, elevated serum IgG4 titer, and increased IgG4-positive plasma cells in a variety of tissues. Scleroderma is also characterized by fibrosis and lymphoplasmacytic infiltrates. To our knowledge, the presence of IgG4-positive cells has not been well characterized in scleroderma. A retrospective review of scleroderma and related disorders (calcinosis, raynaud's syndrome, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) syndrome, progressive systemic sclerosis, morphea) was performed. Thirty-four cases of scleroderma and related disorders were identified; IgG4-positive and IgG-positive plasma cells were counted in 10 HPF and an IgG4:IgG ratio determined. A cutoff ratio of 0.3 was used to define significant elevation. Three of the scleroderma cases had IgG4:IgG greater than 0. Only 1 case had a significant elevation. Of the 3 cases with elevated ratio, IgG4-positive cells ranged from 2 to 64 (median = 14), with an IgG4:IgG ranging from 0.06 to 0.34 (median = 0.22). Similar results were produced with the other sclerosing disorders. These results suggest that scleroderma is not part of the IgG4-related disease spectrum. FAU - Reddi, Deepti M AU - Reddi DM AD - Department of Pathology, Duke University Medical Center, Durham, NC, USA. FAU - Cardona, Diana M AU - Cardona DM FAU - Burchette, James L AU - Burchette JL FAU - Puri, Puja K AU - Puri PK LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Am J Dermatopathol JT - The American Journal of dermatopathology JID - 7911005 RN - 0 (Biomarkers) RN - 0 (Immunoglobulin G) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Biomarkers/analysis MH - Biopsy MH - Female MH - Fibrosis MH - Humans MH - Immunoglobulin G/*analysis MH - Immunohistochemistry MH - Male MH - Middle Aged MH - Plasma Cells/*immunology MH - Retrospective Studies MH - Scleroderma, Systemic/*immunology/pathology MH - Skin/*immunology/pathology MH - Young Adult EDAT- 2013/04/09 06:00 MHDA- 2013/10/23 06:00 CRDT- 2013/04/09 06:00 PHST- 2013/04/09 06:00 [entrez] PHST- 2013/04/09 06:00 [pubmed] PHST- 2013/10/23 06:00 [medline] AID - 10.1097/DAD.0b013e318276cbac [doi] PST - ppublish SO - Am J Dermatopathol. 2013 Jun;35(4):458-62. doi: 10.1097/DAD.0b013e318276cbac. PMID- 38384646 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240224 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 1 DP - 2024 Jan TI - From Suspected COVID-19 to Anti-synthetase Syndrome: A Diagnostic Challenge in the Pandemic Era. PG - e52733 LID - 10.7759/cureus.52733 [doi] LID - e52733 AB - Anti-synthetase syndrome (ASS), a rare immunomediated disease, is characterized by multiple signs and symptoms. Not all patients develop the entire clinical spectrum of the syndrome, as it often varies depending on the involved antibodies. In this case report, a 53-year-old non-smoking woman had complaints of fatigue and dyspnea on exertion for five weeks. The outpatient study revealed creatine kinase (CK) 351U/L, ANAs+, anti-SSa+, normal echocardiogram, and a chest X-ray suggesting imaging suspicion of SARS-CoV-2 pneumonia. Referred to the emergency department, she was hospitalized for bilateral interstitial pneumonia without respiratory failure. Three SARS-CoV-2 polymerase chain reaction tests were negative. She underwent a five-day course of dexamethasone 6mg due to suspected coronavirus disease 2019 (COVID-19) sequelae with favorable progress. About a month later, she experienced fatigue, exertional intolerance, morning cough, and Raynaud's phenomenon episodes. Anti-SARS-CoV-2 antibodies were negative, and a follow-up chest CT showed bilateral organizing pneumonia. Bronchofibroscopy and bronchoalveolar lavage with cytology suggestive of inflammatory appearance, predominantly CD8+ lymphocytes, were performed. Subsequently, positive results for anti-OJ antibodies were obtained. A diagnosis of ASS was established, and prednisolone was initiated at 60mg/day with a tapering regimen, resulting in clinical and radiological improvement. Additional therapy with azathioprine was proposed. This case is presented due to highly suggestive COVID-19 imaging changes, emphasizing the importance of a high suspicion of ASS, despite nearly exclusive pulmonary involvement, with only one isolated elevated CK value and no musculoskeletal complaints. It is also noteworthy for the association with anti-OJ antibodies, rarely identified, often presenting interstitial lung disease as an isolated manifestation. CI - Copyright © 2024, Gomes Ferreira et al. FAU - Gomes Ferreira, Sérgio AU - Gomes Ferreira S AD - Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT. FAU - Fernandes, Luís AU - Fernandes L AD - Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT. FAU - Santos, Sara AU - Santos S AD - Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT. FAU - Ferreira, Sofia AU - Ferreira S AD - Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT. FAU - Teixeira, Mónica AU - Teixeira M AD - Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT. LA - eng PT - Case Reports PT - Journal Article DEP - 20240122 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10880742 OTO - NOTNLM OT - anti-synthetase OT - covid-19 OT - immunomediated OT - interstitial lung disease OT - pneumonia COIS- The authors have declared that no competing interests exist. EDAT- 2024/02/22 06:43 MHDA- 2024/02/22 06:44 PMCR- 2024/01/22 CRDT- 2024/02/22 03:52 PHST- 2024/01/21 00:00 [accepted] PHST- 2024/02/22 06:44 [medline] PHST- 2024/02/22 06:43 [pubmed] PHST- 2024/02/22 03:52 [entrez] PHST- 2024/01/22 00:00 [pmc-release] AID - 10.7759/cureus.52733 [doi] PST - epublish SO - Cureus. 2024 Jan 22;16(1):e52733. doi: 10.7759/cureus.52733. eCollection 2024 Jan. PMID- 28059813 OWN - NLM STAT- MEDLINE DCOM- 20170627 LR - 20250103 IS - 1875-9270 (Electronic) IS - 1051-9815 (Linking) VI - 55 IP - 4 DP - 2016 TI - Workplace barriers encountered by employed persons with systemic sclerosis. PG - 923-929 LID - 10.3233/WOR-162448 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is an auto-immune connective tissue disease characterized by fibrosis of skin, blood vessels, and internal organs that results in significant disability. OBJECTIVE: To identify the work barriers faced by people with systemic sclerosis (SSc) in maintaining employment. METHODS: Thirty-six people with SSc who were working more than 8 hours per week completed the Work Experience Survey, which contains lists of potential work barriers, including the ability to travel to and from work; get around at work; perform essential job functions, including physical, cognitive, and task-related activities; work with others; and manage work conditions. RESULTS: Thirty-three participants completed and returned the questionnaires, most of whom were female, and working full time and in professional careers. Principal disease symptoms included fatigue, Raynaud's phenomenon, esophageal involvement, and leg or hand/wrist pain. All participants reported some barriers with a mean of 18 barriers per participant. At least three quarters of participants cited outside temperature (82%), cold temperatures inside the workplace (76%), and household work (76%), as barriers. The next most common barriers were using both hands (64%), arranging and taking part in social activities (64%), being able to provide self-care (61%) and working 8 hours (58%). CONCLUSION: Participants reported a wide range of barriers, from cold temperatures, to physical job, fatigue related, and non-workplace demands, in maintaining the worker role. The barriers reflect the disease symptoms they reported. Identifying workplace barriers facilitates the creation of job accommodations or adaptations that will allow people with SSc to continue working. FAU - Poole, Janet L AU - Poole JL AD - Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, USA. FAU - Anwar, Sahar AU - Anwar S AD - University of New Mexico Hospital, Albuquerque, NM, USA. FAU - Mendelson, Cindy AU - Mendelson C AD - College of Nursing, University of New Mexico, Albuquerque, NM, USA. FAU - Allaire, Saralynn AU - Allaire S AD - College of Health and Rehabilitation Sciences, Boston University, Boston, MA, USA. LA - eng PT - Journal Article PL - United States TA - Work JT - Work (Reading, Mass.) JID - 9204382 SB - IM MH - Adult MH - Persons with Disabilities/*psychology MH - Employment/psychology/*standards MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/physiopathology/*psychology MH - Surveys and Questionnaires MH - Workplace/psychology OTO - NOTNLM OT - Employment OT - disability OT - rheumatic disease EDAT- 2017/01/07 06:00 MHDA- 2017/06/28 06:00 CRDT- 2017/01/07 06:00 PHST- 2017/01/07 06:00 [entrez] PHST- 2017/01/07 06:00 [pubmed] PHST- 2017/06/28 06:00 [medline] AID - WOR2448 [pii] AID - 10.3233/WOR-162448 [doi] PST - ppublish SO - Work. 2016;55(4):923-929. doi: 10.3233/WOR-162448. PMID- 24135060 OWN - NLM STAT- MEDLINE DCOM- 20150330 LR - 20161018 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 35 IP - 7 DP - 2014 Jul TI - [Clinical phenotypes and prognosis of antisynthetase syndrome]. PG - 453-60 LID - S0248-8663(13)00646-2 [pii] LID - 10.1016/j.revmed.2013.09.003 [doi] AB - Antisynthetase syndrome (ASS) was first described in 1989 as an inflammatory myopathy associated with the presence of specific auto-antibodies, namely the anti-tRNA-synthetase antibodies (ASA). To date, the ASA family comprises eight different auto-antibodies, among which anti-hystidyl-tRNA-synthetase (anti-Jo1) is the most prevalent. In addition to myositis, a constellation of clinical features has also been described in ASS, including interstitial lung disease, Raynaud's phenomenon, polyarthritis, fever and mechanic's hands. Large variations in the distribution and the severity of each of these symptoms are reported from one patient to another, and also over the course of the disease. The heterogeneity of this autoimmune connective tissue disease has led to difficulties in the early identification of patients with a poor outcome (those who will require the most intensive treatments). Additionally, very few prospective trials have so far compared the efficacy of the different immunosuppressive drugs available, and evidence is lacking to help adapting therapeutic strategies to all of the different ASS clinical situations. We will review the different characteristics of ASS (namely biological, clinical, functional, and morphological ASS parameters) that have recently been shown to correlate with patients' outcome, our aim being to discuss the usefulness of patient stratification for elaborating targeted therapeutic trials for ASS in the future. CI - Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved. FAU - Hervier, B AU - Hervier B AD - Service de médecine interne 2, Centre national de référence pour le lupus et le syndrome des antiphospholipides, hôpital Pitié Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France. Electronic address: baptiste.hervier@psl.aphp.fr. FAU - Benveniste, O AU - Benveniste O AD - Service de médecine interne 1, Centre national de référence des maladies musculaires, hôpital Pitié Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France. LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Phénotypes cliniques et pronostic du syndrome des antisynthétases. DEP - 20131014 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/blood MH - Biomarkers/blood MH - Diagnosis, Differential MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Myositis/*diagnosis/therapy MH - Phenotype MH - Prognosis OTO - NOTNLM OT - Anti-Jo1 OT - Antisynthetase OT - Antisynthétase OT - Inflammatory myopathy OT - Interstitial lung disease OT - Myopathie inflammatoire OT - Myosite OT - Myositis OT - Myositis-specific antibodies OT - Pneumopathie infiltrante diffuse EDAT- 2013/10/19 06:00 MHDA- 2015/03/31 06:00 CRDT- 2013/10/19 06:00 PHST- 2013/09/10 00:00 [received] PHST- 2013/09/11 00:00 [accepted] PHST- 2013/10/19 06:00 [entrez] PHST- 2013/10/19 06:00 [pubmed] PHST- 2015/03/31 06:00 [medline] AID - S0248-8663(13)00646-2 [pii] AID - 10.1016/j.revmed.2013.09.003 [doi] PST - ppublish SO - Rev Med Interne. 2014 Jul;35(7):453-60. doi: 10.1016/j.revmed.2013.09.003. Epub 2013 Oct 14. PMID- 37929840 OWN - NLM STAT- MEDLINE DCOM- 20231114 LR - 20250224 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 11 IP - 11 DP - 2023 Nov 6 TI - Phosphodiesterase 5 inhibitors (PDE5i) for the treatment of Raynaud's phenomenon. PG - CD014089 LID - 10.1002/14651858.CD014089 [doi] LID - CD014089 AB - BACKGROUND: Raynaud's phenomenon is a vasodilatory phenomenon characterised by digital pallor, cyanosis, and pain of the extremities. Primary Raynaud's phenomenon has no underlying disease associated with it, while secondary Raynaud's phenomenon is associated with connective tissue disorders such as systemic sclerosis. Systemic sclerosis causes fibrosis and commonly affects the skin and internal organs such as the gastrointestinal tract, lungs, kidney, and heart. Phosphodiesterase 5 inhibitors (PDE5i) are a class of drugs that increases blood flow to the extremities and may be beneficial in the treatment of Raynaud's phenomenon. OBJECTIVES: To assess the benefits and harms of PDE5i compared to placebo for the treatment of Raynaud's phenomenon. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials, MEDLINE, Embase, and clinical trial registries up to June 2022. We did not apply any language restrictions. We searched the bibliographies of retrieved articles and contacted key experts in the field for additional and unpublished data. SELECTION CRITERIA: Randomised controlled trials (RCTs) comparing PDE5i to placebo in people with primary and secondary Raynaud's phenomenon. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: This review included nine RCTs which ranged in duration from four to eight weeks and included a total of 411 participants. The majority had Raynaud's phenomenon secondary to systemic sclerosis. Tadalafil was assessed in four studies, sildenafil in three studies, vardenafil in one study, and a new PDE5 inhibitor known as "PF-00489791" in one study. Three studies were parallel design and six studies were cross-over. The frequency of attacks per week was 24 with placebo and PDE5i reduced the frequency of attacks by an average of three attacks per week (mean difference (MD) -3.07, 95% confidence interval (CI) -5.15 to -1.00; 8 studies; low-certainty evidence). The duration of attacks per day was 55 minutes with placebo and PDE5i reduced the duration of attacks by an average of five minutes (MD -5.31, 95% CI -8.90 to -1.71; 8 studies; low-certainty evidence). Very low-certainty evidence from one study with eight participants showed severity of Raynaud's attacks (assessed on a 10 cm visual analogue scale with lower scores indicating less severity) was 20% lower with a PDE5i (3.7 with placebo compared to 1.6 with treatment; MD -2.1, 95% CI -2.7 to 1.4; very low-certainty evidence). Pain and patient global assessment were assessed on a 10 cm visual analogue scale with lower scores indicating improvement. Low-certainty evidence showed that the use of PDE5i may result in little to no difference compared to placebo in reducing the average pain of Raynaud's attacks (3 to 2.9; MD -0.10, 95% CI -0.78 to 0.57; 4 studies). Global scores were 36% lower with the use of a PDE5i compared to placebo (9.2 to 5.6; MD -3.59, 95% CI -4.45 to -2.73; 1 study, 24 participants; low-certainty evidence). The rate of withdrawals during treatment with PDE5i ranged from 2% to 20% compared with 2% to 4% in the placebo group in five studies. Four studies reported no withdrawals due to adverse events. Seven studies reported no serious adverse events. The rate of serious adverse events reported in two studies ranFged from 2% during treatment to 4% with placebo. The majority of the studies were judged as low or unclear risk of bias for selection, performance, and detection bias. Almost half were judged at high risk of attrition bias and unclear risk for selective reporting bias. We downgraded frequency of attacks, duration of attacks, pain intensity, and patient global assessment for small sample sizes and concerns about inconsistency and graded each as low certainty of evidence. We downgraded severity of attacks to very low certainty due to serious concerns about imprecision and publication bias. We downgraded withdrawals due to adverse events and serious adverse events to moderate certainty of evidence due to a low number of reported events. AUTHORS' CONCLUSIONS: Based on low-certainty evidence, PDE5i may reduce the frequency of attacks of Raynaud's phenomenon by a small amount per week, result in a small reduction in the duration of attack, improve patients' global assessment of their disease, and result in little to no difference in pain. PDE5i probably result in little or no difference in serious adverse events but slightly increase the likelihood of withdrawing from treatment due to an adverse event. CI - Copyright © 2023 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. FAU - Maltez, Nancy AU - Maltez N AD - Department of Rheumatology, The Ottawa Hospital, Ottawa, Canada. FAU - Maxwell, Lara J AU - Maxwell LJ AD - Cochrane Musculoskeletal, Faculty of Medicine, University of Ottawa, Ottawa, Canada. FAU - Rirash, Fadumo AU - Rirash F AD - Department of Medicine, University of Western Ontario, London, Canada. FAU - Tanjong Ghogomu, Elizabeth AU - Tanjong Ghogomu E AD - Bruyère Research Institute, University of Ottawa, Ottawa, Canada. FAU - Harding, Sarah E AU - Harding SE AD - Pediatric Critical Care Medicine, SUNY Upstate Medical University, Syracuse, USA. FAU - Tingey, Paul C AU - Tingey PC AD - Department of Medicine, University of Western Ontario, London, Canada. FAU - Wells, George A AU - Wells GA AD - School of Epidemiology and Public Health, University of Ottawa, Ottawa, Canada. FAU - Tugwell, Peter AU - Tugwell P AD - Department of Medicine, Faculty of Medicine, University of Ottawa, Ottawa, Canada. FAU - Pope, Janet AU - Pope J AD - Department of Medicine and Epidemiology and Biostatistics, University of Western Ontario, London, Canada. LA - eng SI - ClinicalTrials.gov/NCT01117298 SI - ClinicalTrials.gov/NCT01347008 SI - ClinicalTrials.gov/NCT00822354 SI - ClinicalTrials.gov/NCT02050360 PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20231106 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 RN - 0 (Phosphodiesterase 5 Inhibitors) SB - IM MH - Humans MH - Pain MH - *Phosphodiesterase 5 Inhibitors/therapeutic use MH - Sample Size MH - *Scleroderma, Systemic/complications/drug therapy PMC - PMC10626647 COIS- PT: Independent Contractor/Consultant for Data And Safety Monitoring Board, Reformulary Group, Outcome Measures in Rheumatology (OMERACT). GAW: University of Ottawa (Employment) JP, NM, LJM, EG, SEH, PCT, FR: none known EDAT- 2023/11/06 12:43 MHDA- 2023/11/07 06:45 PMCR- 2024/11/06 CRDT- 2023/11/06 07:13 PHST- 2023/11/07 06:45 [medline] PHST- 2023/11/06 12:43 [pubmed] PHST- 2023/11/06 07:13 [entrez] PHST- 2024/11/06 00:00 [pmc-release] AID - CD014089 [pii] AID - 10.1002/14651858.CD014089 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2023 Nov 6;11(11):CD014089. doi: 10.1002/14651858.CD014089. PMID- 35004754 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240405 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 8 DP - 2021 TI - Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies. PG - 788250 LID - 10.3389/fmed.2021.788250 [doi] LID - 788250 AB - Systemic Sclerosis (SSc) is an autoimmune disease marked by dysregulation of the immune system, tissue fibrosis and dysfunction of the vasculature. Vascular damage, remodeling and inadequate endothelial repair are hallmarks of the disease. Since early stages of SSc, damage and apoptosis of endothelial cells (ECs) can lead to perivascular inflammation, oxidative stress and tissue hypoxia, resulting in multiple clinical manifestations. Raynaud's phenomenon, edematous puffy hands, digital ulcers, pulmonary artery hypertension, erectile dysfunction, scleroderma renal crisis and heart involvement severely affect quality of life and survival. Understanding pathogenic aspects and biomarkers that reflect endothelial damage in SSc is essential to guide therapeutic interventions. Treatment approaches described for SSc-associated vasculopathy include pharmacological options to improve blood flow and tissue perfusion and, more recently, cellular therapy to enhance endothelial repair, promote angiogenesis and heal injuries. This mini-review examines the current knowledge on cellular and molecular aspects of SSc vasculopathy, as well as established and developing therapeutic approaches for improving the vascular compartment. CI - Copyright © 2021 Zanin-Silva, Santana-Gonçalves, Kawashima-Vasconcelos and Oliveira. FAU - Zanin-Silva, Djúlio César AU - Zanin-Silva DC AD - Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Basic and Applied Immunology Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Santana-Gonçalves, Maynara AU - Santana-Gonçalves M AD - Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Oncology, Stem Cell and Cell-Therapy Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Kawashima-Vasconcelos, Marianna Yumi AU - Kawashima-Vasconcelos MY AD - Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Internal Medicine Graduate Program, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. FAU - Oliveira, Maria Carolina AU - Oliveira MC AD - Center for Cell-Based Therapy, Regional Hemotherapy Center of the Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. AD - Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil. LA - eng PT - Journal Article PT - Review DEP - 20211222 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC8727451 OTO - NOTNLM OT - cellular therapy OT - endothelial cells OT - systemic sclerosis OT - vasculopathy OT - vasodilator agent COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/01/11 06:00 MHDA- 2022/01/11 06:01 PMCR- 2021/12/22 CRDT- 2022/01/10 09:26 PHST- 2021/10/01 00:00 [received] PHST- 2021/11/29 00:00 [accepted] PHST- 2022/01/10 09:26 [entrez] PHST- 2022/01/11 06:00 [pubmed] PHST- 2022/01/11 06:01 [medline] PHST- 2021/12/22 00:00 [pmc-release] AID - 10.3389/fmed.2021.788250 [doi] PST - epublish SO - Front Med (Lausanne). 2021 Dec 22;8:788250. doi: 10.3389/fmed.2021.788250. eCollection 2021. PMID- 16987839 OWN - NLM STAT- MEDLINE DCOM- 20070607 LR - 20080325 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 Suppl 3 DP - 2006 Oct TI - The role of endothelin in connective tissue diseases. PG - iii8-10 AB - Vascular dysregulation is centrally involved in the pathogenesis of diverse rheumatological diseases. The resulting pulmonary arterial hypertension as well as Raynaud's phenomenon may be accompanied by distinct tissue fibrosis. The pleiotropic cytokine endothelin may represent a link between these vascular and fibrotic processes, which are most evidently seen in systemic sclerosis. Among three closely related isoforms, endothelin-1 (ET-1) is the most common in humans, and is often referred to as ET in the literature. ET-1 is involved in physiological processes of vascular tone and mitogenesis, whereas under pathological conditions fibrosis, vascular hypertension and inflammation are induced. Its expression is dependent on tissue and cell type as well as on the underlying disease entity and its stage. Elevated plasma and tissue levels have been demonstrated in idiopathic pulmonary hypertension, systemic sclerosis as well as in other connective tissue diseases and correlate to haemodynamic parameters and disease outcome. The biological effects are mediated by two membrane receptors (ET-1-receptor A and B) belonging to the G-protein-coupled serpentine family. Both receptors are differentially expressed by different cell types as well as in different diseases entities. Antagonizing these receptors therapeutically has already been successful. However, the differential action of ET is counterbalanced by other mediators, prominently nitric oxide. Consequently, the suspected direct relation of vascular and fibrotic processes through ET still needs to be further evaluated. FAU - Sticherling, M AU - Sticherling M AD - Department of Dermatology, University of Erlang, Germany. Michael.Sticherling@derma.imed.uni-erlangen.de LA - eng PT - Journal Article PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Endothelin-1) RN - 0 (Receptor, Endothelin A) RN - 0 (Receptor, Endothelin B) SB - IM EIN - Rheumatology (Oxford). 2008 Feb;47(2):234-5 MH - Cardiovascular System/physiopathology MH - Connective Tissue Diseases/*metabolism/*physiopathology MH - Endothelin-1/blood/*metabolism MH - Humans MH - Receptor, Endothelin A/physiology MH - Receptor, Endothelin B/physiology RF - 14 EDAT- 2006/09/22 09:00 MHDA- 2008/03/26 09:00 CRDT- 2006/09/22 09:00 PHST- 2006/09/22 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2006/09/22 09:00 [entrez] AID - 45/suppl_3/iii8 [pii] AID - 10.1093/rheumatology/kel294 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii8-10. doi: 10.1093/rheumatology/kel294. PMID- 20451141 OWN - NLM STAT- MEDLINE DCOM- 20100715 LR - 20100510 IS - 1547-4127 (Print) VI - 20 IP - 2 DP - 2010 May TI - Thoracoscopic sympathectomy. PG - 323-30 LID - 10.1016/j.thorsurg.2010.02.008 [doi] AB - With the advent of videotechnology, sympathectomy has assumed a more important role in the armamentarium of managing diseases of the autonomic system. Currently it is used primarily for hyperhydrosis, although sympathectomy for reflex sympathetic dystrophy (RSD), Raynaud disease and other diseases still are performed, but less frequently. Most of this article will refer primarily to hyperhydrosis patients. CI - 2010 Elsevier Inc. All rights reserved. FAU - Krasna, Mark J AU - Krasna MJ AD - Program of Health Policy, St. Joseph Cancer Institute, University of Maryland, 7501 Osler Drive, Suite 104, Towson, MD 21204, USA. markkrasna@catholichealth.net LA - eng PT - Journal Article PT - Review PL - United States TA - Thorac Surg Clin JT - Thoracic surgery clinics JID - 101198195 SB - IM MH - Humans MH - Hyperhidrosis/surgery MH - Postoperative Care MH - Spine/innervation MH - Sympathectomy/*methods MH - Thoracoscopy/*methods RF - 30 EDAT- 2010/05/11 06:00 MHDA- 2010/07/16 06:00 CRDT- 2010/05/11 06:00 PHST- 2010/05/11 06:00 [entrez] PHST- 2010/05/11 06:00 [pubmed] PHST- 2010/07/16 06:00 [medline] AID - S1547-4127(10)00041-1 [pii] AID - 10.1016/j.thorsurg.2010.02.008 [doi] PST - ppublish SO - Thorac Surg Clin. 2010 May;20(2):323-30. doi: 10.1016/j.thorsurg.2010.02.008. PMID- 28507447 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1178-7031 (Print) IS - 1178-7031 (Electronic) IS - 1178-7031 (Linking) VI - 10 DP - 2017 TI - Managing refractory cryoglobulinemic vasculitis: challenges and solutions. PG - 49-54 LID - 10.2147/JIR.S114067 [doi] AB - Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis. In patients with mixed cryoglobulinemia, prevalence of anti-hepatitis C virus (HCV) antibodies and/or HCV RNA, detected by polymerase chain reaction (PCR), is reported to be up to 90%, indicating a significant role of HCV in the development of this condition. The goals of therapy for mixed cryoglobulinemia include immunoglobulin level reduction and antigen elimination. CryoVas not associated with HCV infection should be treated according to treatment recommendations for small-vessel vasculitides. CryoVas associated with chronic HCV infection should be treated with antivirals along with immunosuppressive drugs, with or without plasmapheresis, depending on disease severity and organ involvement. Patients who do not respond to first-line therapy may achieve remission when treatment with rituximab is started as second-line therapy. In HCV-related CryoVas, antiviral therapy should be given along with rituximab in order to achieve complete or partial remission. Moreover, rituximab has proven to be a glucocorticoid-sparing medication. Other potential therapies for refractory CryoVas include mycophenolate mofetil and belimumab, while tumor necrosis factor (TNF) inhibitors are not effective. FAU - Ostojic, Predrag AU - Ostojic P AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia. FAU - Jeremic, Ivan R AU - Jeremic IR AD - Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia. LA - eng PT - Journal Article PT - Review DEP - 20170508 PL - New Zealand TA - J Inflamm Res JT - Journal of inflammation research JID - 101512684 PMC - PMC5428757 OTO - NOTNLM OT - cryoglobulinemia OT - treatment OT - vasculitis COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2017/05/17 06:00 MHDA- 2017/05/17 06:01 PMCR- 2017/05/08 CRDT- 2017/05/17 06:00 PHST- 2017/05/17 06:00 [entrez] PHST- 2017/05/17 06:00 [pubmed] PHST- 2017/05/17 06:01 [medline] PHST- 2017/05/08 00:00 [pmc-release] AID - jir-10-049 [pii] AID - 10.2147/JIR.S114067 [doi] PST - epublish SO - J Inflamm Res. 2017 May 8;10:49-54. doi: 10.2147/JIR.S114067. eCollection 2017. PMID- 22728492 OWN - NLM STAT- MEDLINE DCOM- 20121113 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 51 IP - 12 DP - 2012 TI - Anti-centromere antibody-positive subjects presenting with hypertensive emergency and renal dysfunction in the absence of skin manifestations: a variant of systemic sclerosis or a novel entity? PG - 1567-72 AB - Two patients with anti-centromere antibody (ACA), hypertensive emergency, and acute renal failure, mimicking scleroderma renal crisis, without Raynaud's phenomenon and typical skin manifestations of systemic sclerosis (SSc), are reported. A review of 26 ACA-positive patients between March 2003 and March 2011 in Yokosuka Kyosai Hospital identified four additional patients with similar manifestations. All patients were Japanese women between 41 and 84 years of age at presentation. Human leukocyte antigen (HLA) genotyping tests revealed the absence of the HLA-DQB1*0501 and DRB1*0101 alleles, which are associated with disease susceptibility to ACA-positive SSc among Japanese. These subjects' manifestations may represent a novel entity. FAU - Mandai, Shintaro AU - Mandai S AD - Department of Nephrology, Yokosuka Kyosai Hospital, Japan. smandai@hotmail.co.jp FAU - Arai, Yohei AU - Arai Y FAU - Hirasawa, Suguru AU - Hirasawa S FAU - Hirai, Toshiyuki AU - Hirai T FAU - Aki, Shota AU - Aki S FAU - Inaba, Naoto AU - Inaba N FAU - Aoyagi, Makoto AU - Aoyagi M FAU - Tanaka, Hiroyuki AU - Tanaka H FAU - Tamura, Teiichi AU - Tamura T FAU - Sasaki, Sei AU - Sasaki S LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20120615 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Antibodies, Antinuclear) RN - 0 (HLA Antigens) SB - IM MH - Acute Kidney Injury/*immunology/*physiopathology MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/*blood MH - Centromere/*immunology MH - Diagnosis, Differential MH - Female MH - Genetic Predisposition to Disease MH - Genotype MH - HLA Antigens/genetics MH - Humans MH - Hypertension, Malignant/*immunology/*physiopathology MH - Middle Aged MH - Scleroderma, Systemic/*diagnosis/genetics/immunology/physiopathology EDAT- 2012/06/26 06:00 MHDA- 2012/11/14 06:00 CRDT- 2012/06/26 06:00 PHST- 2012/06/26 06:00 [entrez] PHST- 2012/06/26 06:00 [pubmed] PHST- 2012/11/14 06:00 [medline] AID - DN/JST.JSTAGE/internalmedicine/51.6924 [pii] AID - 10.2169/internalmedicine.51.6924 [doi] PST - ppublish SO - Intern Med. 2012;51(12):1567-72. doi: 10.2169/internalmedicine.51.6924. Epub 2012 Jun 15. PMID- 37494518 STAT- Publisher ISBN- 978-3-030-92879-7 ISBN- 978-3-030-92880-3 CTDT- 20220506 PB - Springer DP - 2022 TI - Rheumatism and wIRA Therapy. BTI - Water-filtered Infrared A (wIRA) Irradiation: From Research to Clinical Settings CP - Chapter 19 PG - 225-32 AB - Rheumatologic disorders comprise various conditions having different etiologies and pathogenesis, the leading clinical symptoms of which are chronic joint pain and musculoskeletal impairment. In the context of a multimodal therapy concept, the use of hyperthermia (HT) is a classical and developing adjuvant symptomatic treatment option. wIRA is an effective and well-established variant of thermal therapy in different rheumatologic disorders. This article summarizes the current state of research into locally applied wIRA in the field of rheumatism and rheumatological diseases. Local and serially applied wIRA significantly relieves pain in patients with axial spondyloarthritis (axSpA), osteoarthritis (OA) and fibromyalgia (FM), which, at least reduces the requirement for analgesics and has positive effects on well-being, functional status or disease activity. wIRA has been shown to reduce levels of C-reactive protein (CRP) and proinflammatory cytokine tumour necrosis factor α (TNFα). Given its safety and tolerability, wIRA is highly amenable in combination with standard therapies. Currently, wIRA effects are assessed in OA patients, non-inflammatory arthralgia and recent-onset arthritis of the hands. Preliminary data on effects on pain, global disease burden and functional status are promising. The potential value of wIRA, for e.g., Raynaud’s phenomena and sclerotic skin changes, need further evaluation. CI - Copyright 2022, The Author(s). FED - Vaupel, Peter ED - Vaupel P AD - Department of Radiation Oncology, University Medical Center, University of Freiburg, Freiburg/Breisgau, Germany. GRID: grid.5963.9 FAU - Vogler, D AU - Vogler D AD - Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Campus Mitte, Humboldt-Universität zu Berlin, Freie Universität Berlin, Berlin, Germany FAU - Schmittat, G AU - Schmittat G AD - Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Campus Mitte, Humboldt-Universität zu Berlin, Freie Universität Berlin, Berlin, Germany FAU - Ohrndorf, S AU - Ohrndorf S AD - Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Campus Mitte, Humboldt-Universität zu Berlin, Freie Universität Berlin, Berlin, Germany LA - eng PT - Review PT - Book Chapter PL - Cham (CH) OTO - NLM OT - Water-filtered infrared A (wIRA) therapy OT - Rheumatism OT - Axial spondyloarthritis OT - Osteoarthritis OT - Fibromyalgia OT - Recent-onset arthritis EDAT- 2022/05/06 00:00 CRDT- 2022/05/06 00:00 AID - NBK593454 [bookaccession] AID - 10.1007/978-3-030-92880-3_19 [doi] PMID- 33048801 OWN - NLM STAT- MEDLINE DCOM- 20201022 LR - 20201218 IS - 1669-9106 (Electronic) IS - 0025-7680 (Linking) VI - 80 IP - 5 DP - 2020 TI - [Simultaneous diagnosis and favorable evolution of infection with Pneumocystis jirovecii, SARS-CoV-2 and advanced HIV]. PG - 554-556 AB - SARS-CoV-2 causes the disease named COVID-19, which emerged in Wuhan, China, in December 2019 and developed into the current pandemic. The manifestations of SARS-CoV-2 infection are highly variable. The worst outcomes are usually associated with advanced age and known risk factors. Among these, it would be reasonable to consider conditions compromising the immune system, particularly the immunodeficiency associated to HIV. To date, however, there is no evidence of HIV infection worsening the evolution and prognosis of COVID-19. Pneumocystis jirovecii (previously-P. carinii) pneumonia, is a fungal disease that most commonly affects immunocompromised persons and can be life-threatening. Typically, patients at risk are those with any underlying condition altering host immunity. We present the case of a middle-aged woman with Raynaud's syndrome who was admitted with pneumonia. During hospitalization she was simultaneously diagnosed with infection by HIV, COVID-19 and P. jirovecci. The patient evolved favorably upon empirical treatment without requiring invasive maneuvers or ventilatory support. Outpatient follow-up after hospital discharge was uneventful. FAU - Larzábal, Francisco J AU - Larzábal FJ AD - Servicio de Clínica Médica, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. E-mail: Fjlarzabal@gmail.com. FAU - Vilela, Andrés AU - Vilela A AD - Servicio de Clínica Médica, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. FAU - Brusca, Silvia AU - Brusca S AD - Servicio de Clínica Médica, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. FAU - Saluzzi, Irene AU - Saluzzi I AD - Servicio de Clínica Médica, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. FAU - Ghergo, Gisela E AU - Ghergo GE AD - Servicio de Clínica Médica, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. FAU - Angiono, María Antonela AU - Angiono MA AD - Servicio de Infectología, Hospital Dalmacio Vélez Sarsfield, Buenos Aires, Argentina. LA - spa PT - Case Reports PT - Journal Article TT - Diagnóstico simultáneo y evolución favorable de infección por Pneumocystis jirovecii, SARS-COV-2 y HIV avanzada. PL - Argentina TA - Medicina (B Aires) JT - Medicina JID - 0204271 SB - IM MH - Betacoronavirus MH - COVID-19 MH - COVID-19 Testing MH - Clinical Laboratory Techniques MH - Coronavirus MH - Coronavirus Infections/*diagnosis MH - Female MH - HIV Infections/*diagnosis MH - Humans MH - Middle Aged MH - *Pandemics MH - Pneumocystis carinii/*isolation & purification MH - Pneumonia, Pneumocystis/*diagnosis MH - *Pneumonia, Viral MH - SARS-CoV-2 MH - Severe Acute Respiratory Syndrome OTO - NOTNLM OT - COVID-19 OT - HIV OT - Pneumocysti jiroveccii EDAT- 2020/10/14 06:00 MHDA- 2020/10/23 06:00 CRDT- 2020/10/13 17:11 PHST- 2020/10/13 17:11 [entrez] PHST- 2020/10/14 06:00 [pubmed] PHST- 2020/10/23 06:00 [medline] PST - ppublish SO - Medicina (B Aires). 2020;80(5):554-556. PMID- 39898813 OWN - NLM STAT- MEDLINE DCOM- 20250908 LR - 20260205 IS - 1460-2105 (Electronic) IS - 0027-8874 (Print) IS - 0027-8874 (Linking) VI - 117 IP - 9 DP - 2025 Sep 1 TI - Factors associated with longitudinal progression of the cumulative burden of morbidity and overall mortality after cisplatin-based chemotherapy for testicular cancer. PG - 1797-1808 LID - 10.1093/jnci/djaf014 [doi] AB - BACKGROUND: To comprehensively evaluate the longitudinal progression of cumulative burden of morbidity (CBM) in testicular cancer survivors (TCS) following standard-dose cisplatin-based chemotherapy and the impact of modifiable risk factors on morbidity and early mortality. METHODS: Participants completed first-line chemotherapy at or longer than 6 months before baseline assessments with comprehensive questionnaires and physical examinations. Based on follow-up assessments (median: 7 years later), longitudinal progression of adverse health outcomes (AHOs) and CBM score (encompassing AHO number and severity) were examined. Baseline health behaviors and AHOs were evaluated for associations with mortality using mixed-effects parametric proportional-hazards regression to identify modifiable risk factors. RESULTS: Among 616 TCS longitudinally assessed, 23% experienced worsening CBM postchemotherapy (median = 11 years, interquartile range = 7-15). Declines were driven by worsening treatment-related AHOs: tinnitus (29.7%), hearing loss (24.4%), Raynaud's disease (22.6%), neuropathy (18.5%), and neuropathic pain (10.7%). Baseline factors associated with worsening neuropathy included lack of aerobic physical activity (odds ratio [OR] = 1.98, 95% confidence interval [CI] = 1.06 to 3.72), and obesity (OR = 1.85, 95% CI = 1.17 to 2.92). These were also related to worsening neuropathic pain (OR = 2.82, P = .009 and OR = 2.29, P = .023). Twenty-nine deaths occurred among 1830 5-year TCS (4.2% cumulative hazard) (median age = 48 years, range = 22-74). Participants reporting neuropathic pain (hazard ratio [HR] = 3.64, 95% CI = 1.45 to 9.10), no aerobic (HR = 6.56, 95% CI = 2.73 to 15.8), or no low-impact physical activity (HR = 3.96, 95% CI = 1.40 to 11.2) had significantly higher mortality, as did TCS indicating fair (HR = 9.23, 95% CI = 3.08 to 27.8) or poor (HR = 18.5, 95% CI = 3.30 to 103) health. Relationships between pain and mortality were mediated through lowered physical activity (P = .036). CONCLUSIONS: Clinically actionable factors associated with early mortality identify high-risk TCS in need of closer monitoring and targeted interventions. The significant relationship between neuropathic pain and mortality, mediated by low physical activity, is the first to our knowledge in TCS. CI - © The Author(s) 2025. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Kerns, Sarah L AU - Kerns SL AUID- ORCID: 0000-0002-6503-0011 AD - Department of Radiation Oncology, Medical College of Wisconsin, Milwaukee, WI, United States. FAU - Dinh, Paul C Jr AU - Dinh PC Jr AD - Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN, United States. FAU - Monahan, Patrick O AU - Monahan PO AD - Department of Biostatistics and Health Data Science, Indiana University, Indianapolis, IN, United States. FAU - Stump, Timothy AU - Stump T AUID- ORCID: 0000-0001-9593-3011 AD - Department of Biostatistics and Health Data Science, Indiana University, Indianapolis, IN, United States. FAU - Fung, Chunkit AU - Fung C AUID- ORCID: 0000-0002-9384-0732 AD - J.P. Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY, United States. FAU - Sesso, Howard D AU - Sesso HD AUID- ORCID: 0000-0002-9698-9954 AD - Division of Preventive Medicine, Brigham and Women's Hospital, Boston, MA, United States. FAU - Feldman, Darren R AU - Feldman DR AUID- ORCID: 0000-0003-2424-4635 AD - Memorial Sloan Kettering Cancer Center, New York, NY, United States. AD - Department of Medicine, Weill Cornell Medical College, New York, NY, United States. FAU - Hamilton, Robert J AU - Hamilton RJ AUID- ORCID: 0000-0002-6715-5934 AD - Division of Urology, Department of Surgical Oncology, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON, Canada. FAU - Vaughn, David J AU - Vaughn DJ AD - Department of Medicine, University of Pennsylvania, Philadelphia, PA, United States. FAU - Huddart, Robert AU - Huddart R AUID- ORCID: 0000-0003-3604-1990 AD - The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Kollmannsberger, Christian AU - Kollmannsberger C AD - Division of Medical Oncology, University of British Columbia, Vancouver, BC, Canada. FAU - Martin, Neil E AU - Martin NE AUID- ORCID: 0000-0002-8164-8516 AD - Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, MA, United States. FAU - Nevel, Kathryn AU - Nevel K AUID- ORCID: 0000-0003-1499-837X AD - Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN, United States. AD - Department of Neurology, Indiana University, Indianapolis, IN, United States. FAU - Kincaid, John AU - Kincaid J AD - Department of Neurology, Indiana University, Indianapolis, IN, United States. FAU - Einhorn, Lawrence H AU - Einhorn LH AUID- ORCID: 0000-0001-7971-7669 AD - Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN, United States. FAU - Travis, Lois B AU - Travis LB AUID- ORCID: 0000-0002-6010-3541 AD - Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN, United States. LA - eng GR - K07 CA187546/CA/NCI NIH HHS/United States GR - R01 CA157823/CA/NCI NIH HHS/United States GR - P30 CA008748/CA/NCI NIH HHS/United States GR - NH/NIH HHS/United States GR - 2R01 CA157823/CA/NCI NIH HHS/United States PT - Journal Article PL - United States TA - J Natl Cancer Inst JT - Journal of the National Cancer Institute JID - 7503089 RN - Q20Q21Q62J (Cisplatin) SB - IM MH - Humans MH - Male MH - *Cisplatin/administration & dosage/adverse effects MH - *Testicular Neoplasms/drug therapy/mortality/epidemiology/pathology MH - Adult MH - Longitudinal Studies MH - Middle Aged MH - Risk Factors MH - Disease Progression MH - *Antineoplastic Combined Chemotherapy Protocols/adverse effects/therapeutic use MH - Cancer Survivors/statistics & numerical data MH - Morbidity MH - Young Adult PMC - PMC12415966 COIS- S.L.K., D.R.F., D.J.V., and L.B.T. report research funding (institutional) from the National Institutes of Health. C.F. reports Open Payments Link: https://openpaymentsdata.cms.gov/physician/450635. D.R.F. reports Consulting or Advisory Role: Telix Pharmaceuticals, BioNTech, Renibus; Research Funding: Telix Pharmaceuticals, Decibel Therapeutics, Astellas Pharma, Exelixis; Royalties for authorship of topic review: UpToDate. K.N. reports Personal Fees: Aptitude Health, LLC; payment for answering survey on glioma management. J.K. reports Subinvestigator for Ionis Pharmaceuticals in a treatment trial of a compound that treats familial amyloid neuropathy. All other authors report no conflicts of interest related to the work in this publication. EDAT- 2025/02/03 12:22 MHDA- 2025/09/08 12:43 PMCR- 2026/02/03 CRDT- 2025/02/03 09:53 PHST- 2024/08/07 00:00 [received] PHST- 2024/12/09 00:00 [revised] PHST- 2025/01/11 00:00 [accepted] PHST- 2025/09/08 12:43 [medline] PHST- 2025/02/03 12:22 [pubmed] PHST- 2025/02/03 09:53 [entrez] PHST- 2026/02/03 00:00 [pmc-release] AID - 7997269 [pii] AID - djaf014 [pii] AID - 10.1093/jnci/djaf014 [doi] PST - ppublish SO - J Natl Cancer Inst. 2025 Sep 1;117(9):1797-1808. doi: 10.1093/jnci/djaf014. PMID- 33790525 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210403 IS - 0973-3698 (Print) IS - 0973-3701 (Electronic) IS - 0973-3698 (Linking) VI - 15 IP - 6 DP - 2020 Dec TI - The clinicoserological spectrum of inflammatory myopathy in the context of systemic sclerosis and systemic lupus erythematosus. PG - 81-90 LID - 10.4103/injr.injr_136_20 [doi] AB - The autoimmune rheumatic diseases (ARDs) are characterised by a pathological triad composed of autoimmunity/inflammation, microangiopathy and aberrant tissue remodelling. Disease terms such as idiopathic inflammatory myopathy (IIM), scleroderma/systemic sclerosis (SSc), and systemic lupus erythematosus (SLE) are helpful clinically but disguise the considerable overlap that exists within these 'distinct' disorders. This is perhaps best demonstrated by inflammatory myopathy, which can be present in SSc or SLE, but can itself be absent in clinically amyopathic IIM. Archetypal clinical manifestations of ARD (such as Raynaud's phenomenon) are frequently present, albeit with varying prominence, within each of these diseases. This is certainly the case for inflammatory myositis, which has long been recognised as an important clinical feature of both SSc and SLE. Progress in elucidating the clinicoserological spectrum of autoimmune rheumatic diseases has identified autoantibody specificities that are strongly associated with 'overlap' disease and the presence of inflammatory myositis in SSc and SLE. In this review, we shall describe the prevalence, burden, prognostic value and management considerations of IIM in the context of both SSc and SLE. A major emphasis on the value of autoantibodies shall highlight the value of these tools in predicting the future occurrence of inflammatory myositis in both SSc and SLE. Where applicable, unmet research needs shall be highlighted. The review emphasises the importance of myopathy as a common feature across all the ARDs, and highlights specific antibody specificities that are strongly associated with myopathy in the context of SLE and SSc. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Skeoch, Sarah AU - Skeoch S AD - Royal National Hospital for Rheumatic Diseases (part of the Royal United Hospitals NHS Foundation Trust), Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Paik, Julie J AU - Paik JJ AD - Johns Hopkins Myositis Center. 5200 Eastern Avenue, MFL Building, Center Tower Suite 4500, Baltimore, MD USA. LA - eng GR - K23 AR073927/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20210118 PL - United States TA - Indian J Rheumatol JT - Indian journal of rheumatology JID - 101279982 PMC - PMC8009601 MID - NIHMS1678135 OTO - NOTNLM OT - Classification OT - Clinical phenotype OT - Overlap syndromes OT - Polymyositis OT - Systemic Lupus Erythematosus OT - Systemic sclerosis OT - dermatomyositis COIS- Conflicts of Interest: None of the authors report any conflicts of interest relevant to the content of this work EDAT- 2021/04/02 06:00 MHDA- 2021/04/02 06:01 PMCR- 2021/03/30 CRDT- 2021/04/01 06:23 PHST- 2021/04/01 06:23 [entrez] PHST- 2021/04/02 06:00 [pubmed] PHST- 2021/04/02 06:01 [medline] PHST- 2021/03/30 00:00 [pmc-release] AID - 10.4103/injr.injr_136_20 [doi] PST - ppublish SO - Indian J Rheumatol. 2020 Dec;15(6):81-90. doi: 10.4103/injr.injr_136_20. Epub 2021 Jan 18. PMID- 29616537 OWN - NLM STAT- MEDLINE DCOM- 20180424 LR - 20180424 IS - 0374-1338 (Print) IS - 0374-1338 (Linking) VI - 63 IP - 1 DP - 2016 TI - [Polymyositis and systemic sclerosis overlap - A case report and a review of the literature]. PG - 10-3 AB - Polymyositis (PM) is an autoimmune disease which affects skeletal muscles. In young age, it usually occurs as an idiopathic disorder associated with specific autoantibodies (anti-Jo), while in older age it is often associated with neoplasms. It can present with symptoms of other autoimmune diseases, such as systemic sclerosis (SSc), a rare progressive disease characterized by collagen deposits in various tissues and organs. A 65-year-old patient, long-time smoker, came to the ER because of painful edema in the distal parts of his limbs and proximal muscle weakness of his arms and legs. Although his muscle enzymes were not increased, PM was confirmed by the characteristic pathohistological finding. The patient had sclerodermal skin lesions on his back, but he did not have other typical SSc symptoms, and the specific autoantibodies were negative. He received glucocorticoid therapy (GC) after we had finished screening for malignant tumors. He felt better, his muscle strength returned, and the limb edema disappeared. Four weeks later, he developed symptoms which are more typical of SSc, such as dysphagia, Raynaud’s phenomenon, and skin thickening of the limbs that had been swollen. PM is often associated with SSc. It is not clear if the exacerbation of latent SSc was stimulated by GC, or if it was just a simple overlap of the two diseases with different onsets. There are no therapy guidelines for the treatment of this combination of diseases. Careful use of GC is necessary even if SSc symptoms are discreet, because of the well-known effects of GC in SSc. FAU - Petrić, Marin AU - Petrić M FAU - Kaliterna, Dušanka Martinović AU - Kaliterna DM FAU - Božić, Ivona AU - Božić I FAU - Nuić, Marija AU - Nuić M FAU - Perković, Dijana AU - Perković D LA - hrv PT - Case Reports PT - Journal Article PT - Review PL - Croatia TA - Reumatizam JT - Reumatizam JID - 0216650 RN - 0 (Glucocorticoids) SB - IM MH - Aged MH - Glucocorticoids/*therapeutic use MH - Humans MH - Male MH - Polymyositis/*complications/*drug therapy MH - Scleroderma, Systemic/*complications/*drug therapy EDAT- 2016/01/01 00:00 MHDA- 2018/04/25 06:00 CRDT- 2018/04/05 06:00 PHST- 2018/04/05 06:00 [entrez] PHST- 2016/01/01 00:00 [pubmed] PHST- 2018/04/25 06:00 [medline] PST - ppublish SO - Reumatizam. 2016;63(1):10-3. PMID- 29952300 OWN - NLM STAT- MEDLINE DCOM- 20181107 LR - 20181107 IS - 1952-4013 (Electronic) IS - 1167-1122 (Linking) VI - 28 IP - 3 DP - 2018 Jun 1 TI - Systemic therapy with calcitonin has positive clinical effects on systemic sclerosis in patients with cutaneous manifestations. PG - 364-369 LID - 10.1684/ejd.2018.3300 [doi] AB - BACKGROUND: Calcitonin gene-related peptide (CGRP) is a vasodilatory neuropeptide that plays an important role in the blood vessels of heart and peripheral circulation, a lack of which may cause vasculopathies. OBJECTIVE: In this study, the clinical course of disease, as well as the efficacy, side effects, and patient satisfaction of systemic calcitonin therapy in patients with systemic sclerosis (SSc), was evaluated. METHODS: Forty-nine patients received repetitive intravenous calcitonin infusions as first-line treatment. The average number of cycles was 12.2 ± 10.3 over a period of 30 months (each cycle: 100 U/day over 10 days). Clinical examinations, laboratory tests, and organ imaging were performed before the start of, and at regular intervals during therapy in order to evaluate organ manifestations and the clinical course of the disease. In addition, patients' own experiences of the therapy, side effects, and therapy success were evaluated with standardized questionnaires. RESULTS: Over the course of the treatment, seven patients experienced improvements in their condition with a considerable reduction in digital ulceration and improved movement (14.3%). Pulmonary function in seven patients improved during the therapy (14.3%). With regards to side effects, nausea (41.7%), headaches (33.3%), fluctuations in blood pressure (29.2%), and flushing (29.2%) were observed. Overall, 45.8% of patients evaluated the therapy as good and 58.3% would undergo further courses of therapy with calcitonin. CONCLUSIONS: Systemic calcitonin treatment seems to have positive clinical effects on SSc and contributes to relieving symptoms, especially in patients with cutaneous manifestations. No severe side effects were reported during this study. FAU - Uslu, Ugur AU - Uslu U AD - Friedrich-Alexander-Universität of Erlangen-Nürnberg, Universitätsklinikum Erlangen, Department of Dermatology, Ulmenweg 18, D-91054 Erlangen, Germany. FAU - Streiff, Laura AU - Streiff L AD - LVR-Klinik Bonn, Department of Psychiatry and Psychotherapy, Kaiser-Karl-Ring 20, D-53111 Bonn, Germany. FAU - Sticherling, Michael AU - Sticherling M AD - Friedrich-Alexander-Universität of Erlangen-Nürnberg, Universitätsklinikum Erlangen, Department of Dermatology, Ulmenweg 18, D-91054 Erlangen, Germany. LA - eng PT - Journal Article PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 RN - 0 (Dermatologic Agents) RN - 9007-12-9 (Calcitonin) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Calcitonin/*administration & dosage/adverse effects MH - Dermatologic Agents/*administration & dosage/adverse effects MH - Female MH - Humans MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Patient Satisfaction MH - Scleroderma, Systemic/*drug therapy OTO - NOTNLM OT - Raynaud's phenomenon OT - calcitonin gene-related peptide OT - collagenosis OT - digital ulcers EDAT- 2018/06/29 06:00 MHDA- 2018/11/08 06:00 CRDT- 2018/06/29 06:00 PHST- 2018/06/29 06:00 [pubmed] PHST- 2018/11/08 06:00 [medline] PHST- 2018/06/29 06:00 [entrez] AID - ejd.2018.3300 [pii] AID - 10.1684/ejd.2018.3300 [doi] PST - ppublish SO - Eur J Dermatol. 2018 Jun 1;28(3):364-369. doi: 10.1684/ejd.2018.3300. PMID- 37569433 OWN - NLM STAT- MEDLINE DCOM- 20230814 LR - 20230814 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 24 IP - 15 DP - 2023 Jul 27 TI - Evidence of a Novel Mitochondrial Signature in Systemic Sclerosis Patients with Chronic Fatigue Syndrome. LID - 10.3390/ijms241512057 [doi] LID - 12057 AB - Symptoms of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) are common in rheumatic diseases, but no studies report the frequency of these in early systemic sclerosis. There are no known biomarkers that can distinguish between patients with ME/CFS, although mitochondrial abnormalities are often demonstrated. We sought to assess the prevalence of ME/CFS in limited cutaneous SSc (lcSSc) patients early in their disease (<5 years from the onset of non-Raynaud's symptoms) and to determine if alterations in mitochondrial electron transport chain (ETC) transcripts and mitochondrial DNA (mtDNA) integrity could be used to distinguish between fatigued and non-fatigued patients. All SSc patients met ACR/EULAR classification criteria. ME/CFS-related symptoms were assessed through validated questionnaires, and the expression of ETC transcripts and mtDNA integrity were quantified via qPCR. SSc patients with ME/CFS could be distinguished from non-fatigued patients through ETC gene analysis; specifically, reduced expression of ND4 and CyB and increased expression of Cox7C. ND4 and CyB expression correlated with indicators of disease severity. Further prospective and functional studies are needed to determine if this altered signature can be further utilized to better identify ME/CFS in SSc patients, and whether ME/CFS in early SSc disease could predict more severe disease outcomes. FAU - van Eeden, Charmaine AU - van Eeden C AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. FAU - Redmond, Desiree AU - Redmond D AUID- ORCID: 0000-0002-9379-051X AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. FAU - Mohazab, Naima AU - Mohazab N AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. FAU - Larché, Maggie J AU - Larché MJ AD - Division of Rheumatology, Department of Medicine, McMaster University, Hamilton, ON L8N 3Z5, Canada. FAU - Mason, Andrew L AU - Mason AL AD - Division of Gastroenterology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. FAU - Cohen Tervaert, Jan Willem AU - Cohen Tervaert JW AUID- ORCID: 0000-0001-7407-6364 AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. FAU - Osman, Mohammed S AU - Osman MS AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB T6G 2G3, Canada. LA - eng GR - IIS 1199-0431/Boehringer Ingelheim/ GR - 17PhD01/Nierstichting/ GR - 19-0558/Arthritis Society/ GR - 2022/Scleroderma Canada/ PT - Journal Article DEP - 20230727 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 RN - 0 (Biomarkers) RN - 0 (DNA, Mitochondrial) SB - IM MH - Humans MH - *Fatigue Syndrome, Chronic/diagnosis MH - Mitochondria/genetics MH - Biomarkers MH - DNA, Mitochondrial/genetics MH - Surveys and Questionnaires PMC - PMC10418481 OTO - NOTNLM OT - fatigue OT - mitochondria OT - myalgic encephalomyelitis OT - systemic sclerosis COIS- J.W.C.T. received speaker honoraria from Pfizer, Medexus and Otsuka, and advisory board honoraria from Mallinckrodt and Otsuka. M.S.O. received speaker honoraria from Boehringer Ingelheim. M.J.L. received advisory board honoraria from Boehringer Ingelheim, Janssen, Mallinckrodt and Pfizer. All other authors declare that they do not have any competing interest. EDAT- 2023/08/12 10:46 MHDA- 2023/08/14 06:42 PMCR- 2023/07/27 CRDT- 2023/08/12 01:08 PHST- 2023/06/28 00:00 [received] PHST- 2023/07/19 00:00 [revised] PHST- 2023/07/26 00:00 [accepted] PHST- 2023/08/14 06:42 [medline] PHST- 2023/08/12 10:46 [pubmed] PHST- 2023/08/12 01:08 [entrez] PHST- 2023/07/27 00:00 [pmc-release] AID - ijms241512057 [pii] AID - ijms-24-12057 [pii] AID - 10.3390/ijms241512057 [doi] PST - epublish SO - Int J Mol Sci. 2023 Jul 27;24(15):12057. doi: 10.3390/ijms241512057. PMID- 17255133 OWN - NLM STAT- MEDLINE DCOM- 20070703 LR - 20220317 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 46 IP - 5 DP - 2007 May TI - Clinical characteristics of Japanese patients with anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibodies. PG - 842-5 AB - OBJECTIVES: The clinical and laboratory characteristics of seven patients with anti-aminoacyl-tRNA synthetase (ARS) autoantibodies, specifically anti-OJ (anti-isoleucyl-tRNA synthetase), were examined and compared with previously published findings. METHODS: Serum samples from 1135 Japanese patients with various autoimmune diseases and 48 normal individuals were screened for anti-OJ antibodies using RNA and protein immunoprecipitation assays. The patients whose sera contained anti-OJ antibodies were assessed regarding clinical symptoms, clinical course, laboratory findings, chest radiography and chest computed tomography. RESULTS: Sera from seven patients were found to contain anti-OJ antibodies. These autoantibodies were associated with interstitial lung disease (ILD) and myositis. The diagnoses of the seven patients were idiopathic interstitial pneumonias (IIPs) in three, polymyositis (PM) in three and PM-rheumatoid arthritis (RA) overlap in the remaining one. All patients had ILD, but muscle weakness and polyarthritis were seen only in four. Raynaud's phenomenon and sclerodactyly were absent in all patients. CONCLUSIONS: These results indicate that the presence of anti-OJ autoantibodies may distinguish a subtype of anti-ARS syndrome that is more closely associated with ILD than myositis or Raynaud's phenomenon. FAU - Sato, S AU - Sato S AD - Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. shins@sc.itc.keio.ac.jp FAU - Kuwana, M AU - Kuwana M FAU - Hirakata, M AU - Hirakata M LA - eng PT - Journal Article PT - Multicenter Study DEP - 20070125 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.5 (Isoleucine-tRNA Ligase) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/blood MH - Arthritis, Rheumatoid/immunology MH - Autoantibodies/*blood MH - Autoimmune Diseases/*immunology MH - Female MH - Humans MH - Immunoprecipitation/methods MH - Isoleucine-tRNA Ligase/*immunology MH - Lung Diseases, Interstitial/immunology MH - Male MH - Middle Aged MH - Polymyositis/immunology MH - Retrospective Studies EDAT- 2007/01/27 09:00 MHDA- 2007/07/04 09:00 CRDT- 2007/01/27 09:00 PHST- 2007/01/27 09:00 [pubmed] PHST- 2007/07/04 09:00 [medline] PHST- 2007/01/27 09:00 [entrez] AID - kel435 [pii] AID - 10.1093/rheumatology/kel435 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 May;46(5):842-5. doi: 10.1093/rheumatology/kel435. Epub 2007 Jan 25. PMID- 19862530 OWN - NLM STAT- MEDLINE DCOM- 20110517 LR - 20260128 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 30 IP - 11 DP - 2010 Sep TI - Clinical and radiological features of Takayasu's arteritis patients in Jordan. PG - 1449-53 LID - 10.1007/s00296-009-1163-5 [doi] AB - The prevalence of Takayasu's arteritis (TA) varies greatly among world populations, and little is known about this disease in Eastern Mediterranean Arab populations. We conducted a retrospective chart review of patients diagnosed with TA from 1996 to 2008 at a single large referral center in Jordan. Eight patients (seven females, one male) with angiographically diagnosed TA were seen at the Jordan University Hospital between 1996 and 2008. All patients were of Arabic ethnicity. The age at presentation ranged from 14 to 50 years, and delay in diagnosis ranged from 1 to 10 years. Extra-vascular manifestations included nodular episcleritis, elevated liver enzymes, erythema nodosum, inflammatory-bowel-disease-like illness, Raynaud's phenomena, and constitutional symptoms. Vascular symptoms included postural dizziness, central nervous system deficits, amauroses fugax, and transient ischemic attacks. Aortic arch vessels were involved in all patients, the abdominal aorta was involved in five patients, and the renal arteries in four patients. Major clinical events including severe stroke and cardiac failure were associated with mortality in two patients. Treatment with corticosteroids and immunosuppressive agents resulted in improvement in five patients with follow-up ranging from 3 to 12 years. In conclusion, TA is seen in Arabs, and the clinical spectrum of TA in Arabs in Jordan is similar to that reported in other countries. Increased awareness of the disease may shorten the time to diagnosis and result in a more reliable estimate of disease prevalence. FAU - Mustafa, Khader N AU - Mustafa KN AD - Jordan University Hospital, Amman, Jordan. kmustafa@ju.edu.jo FAU - Hadidy, Azmy AU - Hadidy A FAU - Sweiss, Nadera J AU - Sweiss NJ LA - eng PT - Journal Article DEP - 20091028 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adolescent MH - Adult MH - Arabs/genetics MH - Comorbidity/trends MH - Female MH - Follow-Up Studies MH - Humans MH - Jordan/epidemiology/ethnology MH - Magnetic Resonance Angiography MH - Male MH - Middle Aged MH - Prevalence MH - Radiography MH - Retrospective Studies MH - Takayasu Arteritis/*diagnosis/*diagnostic imaging/mortality MH - Young Adult EDAT- 2009/10/29 06:00 MHDA- 2011/05/18 06:00 CRDT- 2009/10/29 06:00 PHST- 2009/07/24 00:00 [received] PHST- 2009/09/20 00:00 [accepted] PHST- 2009/10/29 06:00 [entrez] PHST- 2009/10/29 06:00 [pubmed] PHST- 2011/05/18 06:00 [medline] AID - 10.1007/s00296-009-1163-5 [doi] PST - ppublish SO - Rheumatol Int. 2010 Sep;30(11):1449-53. doi: 10.1007/s00296-009-1163-5. Epub 2009 Oct 28. PMID- 27085011 OWN - NLM STAT- MEDLINE DCOM- 20171108 LR - 20250703 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 82 IP - 2 DP - 2016 Aug TI - Peripheral vasoconstriction induced by β-adrenoceptor blockers: a systematic review and a network meta-analysis. PG - 549-60 LID - 10.1111/bcp.12980 [doi] AB - AIM: Peripheral vasoconstriction has long been described as a vascular adverse effect of β-adrenoceptor blockers. Whether β-adrenoceptor blockers should be avoided in patients with peripheral vascular disease depends on pharmacological properties (e.g. preferential binding to β1 -adrenoreceptors or intrinsic sympathomimetic activity). However, this has not been confirmed in experimental studies. We performed a network meta-analysis in order to assess the comparative risk of peripheral vasoconstriction of different β-adrenoceptor blockers. METHOD: We searched for randomized controlled trials (RCTs) including β-adrenoceptor blockers that were published in core clinical journals in the Pubmed database. All RCTs reporting peripheral vasoconstriction as an adverse effect of β-adrenoceptor blockers and controls were included. Sensitivity analyses were conducted including possibly confounding covariates (latitude, properties of the β-adrenoceptor blockers, e.g. intrinsic sympathomimetic activity, vasodilation, drug indication, drug doses). The protocol and the detailed search strategy are available online (PROSPERO registry CRD42014014374). RESULTS: Among 2238 records screened, 38 studies including 57 026 patients were selected. Overall, peripheral vasoconstriction was reported in 7% of patients with β-adrenoceptor blockers and 4.6% in the control groups (P < 0.001), with heterogeneity among drugs. Atenolol and propranolol had a significantly higher risk than placebo, whereas pindolol, acebutolol and oxprenolol had not. CONCLUSION: Our results suggest that β-adrenoceptor blockers have variable propensity to enhance peripheral vasoconstriction and that it is not related to preferential binding to β1 -adrenoceptors. These findings challenge FDA and European recommendations regarding precautions and contra-indications of use of β-adrenoceptor blockers and suggest that β-adrenoceptor blockers with intrinsic sympathomimetic activity could be safely used in patients with peripheral vascular disease. CI - © 2016 The British Pharmacological Society. FAU - Khouri, Charles AU - Khouri C AD - Pôle Santé Publique Pharmacovigilance, Grenoble University Hospital (CHU Grenoble-Alpes), F-38000, Grenoble, France. FAU - Jouve, Thomas AU - Jouve T AD - Pôle Recherche, Pharmacologie Clinique, INSERM CIC1406, Grenoble University Hospital (CHU Grenoble-Alpes), F-38000, Grenoble, France. FAU - Blaise, Sophie AU - Blaise S AD - Univ. Grenoble Alpes HP2, F-38000, Grenoble, France. AD - INSERM, HP2, F-38000, Grenoble, France. AD - Grenoble University Hospital (CHU Grenoble-Alpes), Clinique de Médecine Vasculaire, F-38000, Grenoble, France. FAU - Carpentier, Patrick AU - Carpentier P AD - Grenoble University Hospital (CHU Grenoble-Alpes), Clinique de Médecine Vasculaire, F-38000, Grenoble, France. FAU - Cracowski, Jean-Luc AU - Cracowski JL AD - Pôle Recherche, Pharmacologie Clinique, INSERM CIC1406, Grenoble University Hospital (CHU Grenoble-Alpes), F-38000, Grenoble, France. AD - Univ. Grenoble Alpes HP2, F-38000, Grenoble, France. AD - INSERM, HP2, F-38000, Grenoble, France. FAU - Roustit, Matthieu AU - Roustit M AD - Pôle Recherche, Pharmacologie Clinique, INSERM CIC1406, Grenoble University Hospital (CHU Grenoble-Alpes), F-38000, Grenoble, France. AD - Univ. Grenoble Alpes HP2, F-38000, Grenoble, France. AD - INSERM, HP2, F-38000, Grenoble, France. LA - eng PT - Comparative Study PT - Journal Article PT - Network Meta-Analysis PT - Systematic Review DEP - 20160531 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - 0 (Adrenergic beta-Antagonists) RN - 0 (Sympathomimetics) SB - IM MH - Adrenergic beta-Antagonists/administration & dosage/*adverse effects MH - Dose-Response Relationship, Drug MH - Humans MH - Randomized Controlled Trials as Topic MH - Sympathomimetics/administration & dosage/*adverse effects MH - Vasoconstriction/*drug effects MH - Vasodilation/drug effects PMC - PMC4972171 OTO - NOTNLM OT - Raynaud's phenomenon OT - peripheral vasoconstriction OT - β-adrenoceptor blockers EDAT- 2016/04/17 06:00 MHDA- 2017/11/09 06:00 PMCR- 2017/08/01 CRDT- 2016/04/17 06:00 PHST- 2016/01/22 00:00 [received] PHST- 2016/04/01 00:00 [revised] PHST- 2016/04/14 00:00 [accepted] PHST- 2016/04/17 06:00 [entrez] PHST- 2016/04/17 06:00 [pubmed] PHST- 2017/11/09 06:00 [medline] PHST- 2017/08/01 00:00 [pmc-release] AID - BCP12980 [pii] AID - 10.1111/bcp.12980 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2016 Aug;82(2):549-60. doi: 10.1111/bcp.12980. Epub 2016 May 31. PMID- 38155978 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231230 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 12 DP - 2023 Dec TI - Morphea Involving the Lips and Gingiva: A Rare Case Report. PG - e51202 LID - 10.7759/cureus.51202 [doi] LID - e51202 AB - Morphea is a subtype of scleroderma that does not involve Raynaud's phenomenon or internal organ involvement. It is a connective tissue disease that features the excessive deposition of collagen in the dermis and subcutaneous tissue, leading to a thickening of the dermis and subcutaneous tissue, eventually forming a scar-like lesion. We represent a 19-year-old male Saudi patient displaying a white patch on the marginal gingiva of tooth #21 and multiple yellowish papules on the outer surface of the lip. Both teeth #21 and #22 have experienced recession and bone loss. The patient's clinical history and histopathology revealed characteristic features of localized scleroderma. A treatment was proposed involving immunosuppressants, methotrexate, and pimecrolimus cream along with topical corticosteroids and excimer laser therapy (308 nm). The patient followed the treatment plan for a full month and the white patch quickly improved for the patient. Afterward, the patient has been taking only methotrexate with a significant but gradual improvement. In this paper, we discuss the differential diagnosis to be considered and present an unusual occurrence of localized scleroderma in the oral cavity. CI - Copyright © 2023, Albagieh et al. FAU - Albagieh, Hamad AU - Albagieh H AD - Oral Medicine and Diagnostic Sciences, King Saud University, Riyadh, SAU. FAU - Alshagroud, Rana S AU - Alshagroud RS AD - Oral Medicine and Diagnostic Sciences, King Saud University, Riyadh, SAU. FAU - Aladnan, Abdullah M AU - Aladnan AM AD - Dermatology, Albaha Hosptal, Albaha, SAU. FAU - Aldosari, Bader AU - Aldosari B AD - Oral Medicine and Diagnostic Sciences, King Saud University, Riyadh, SAU. FAU - Alburaykan, Yara B AU - Alburaykan YB AD - College of Dentistry, King Saud University, Riyadh, SAU. FAU - Almashham, Lama Y AU - Almashham LY AD - College of Dentistry, King Saud University, Riyadh, SAU. FAU - Alqasem, Afyaa A AU - Alqasem AA AD - College of Dentistry, King Saud University, Riyadh, SAU. FAU - Alyahya, Areen A AU - Alyahya AA AD - College of Dentistry, King Saud University, Riyadh, SAU. FAU - Aboheimed, Nada I AU - Aboheimed NI AD - College of Dentistry, King Saud University, Riyadh, SAU. LA - eng PT - Case Reports PT - Journal Article DEP - 20231227 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10754225 OTO - NOTNLM OT - en coup de sabre morphea OT - linear morphea OT - localized scleroderma OT - oral cavity lesion OT - oral pathology COIS- The authors have declared that no competing interests exist. EDAT- 2023/12/29 06:43 MHDA- 2023/12/29 06:44 PMCR- 2023/12/27 CRDT- 2023/12/29 03:45 PHST- 2023/12/27 00:00 [accepted] PHST- 2023/12/29 06:44 [medline] PHST- 2023/12/29 06:43 [pubmed] PHST- 2023/12/29 03:45 [entrez] PHST- 2023/12/27 00:00 [pmc-release] AID - 10.7759/cureus.51202 [doi] PST - epublish SO - Cureus. 2023 Dec 27;15(12):e51202. doi: 10.7759/cureus.51202. eCollection 2023 Dec. PMID- 33139373 OWN - NLM STAT- MEDLINE DCOM- 20201110 LR - 20201218 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 13 IP - 11 DP - 2020 Nov 2 TI - Chilblain-like lesions with prominent bullae in a patient with COVID-19. LID - 10.1136/bcr-2020-237917 [doi] LID - e237917 AB - A 27-year-old patient presented with acral chilblain-like lesions atypical of dermatological presentations appearing in current reports of COVID-19. Prominent bullae had formed on the dorsa of her toes and became haemorrhagic 2 days after the initial presentation. The patient had no underlying medical conditions, including any history of collagen vascular disease, Raynaud's phenomenon, chilblains or cold exposure, and was not taking any medications. The patient reported 10 days of ageusia and anosmia 6 weeks prior to the manifestation of her toe lesions, with no other symptoms. A nasopharyngeal swab test for SARS-CoV-2 RNA was positive. It is important that physicians recognise the myriad of cutaneous lesions associated with COVID-19 in this ongoing pandemic. CI - © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Rubin, Alexandra AU - Rubin A AD - Center for Dermatology, Rutgers Robert Wood Johnson Medical School, Somerset, New Jersey, USA alirubin@rwjms.rutgers.edu. FAU - Alamgir, Mahin AU - Alamgir M AD - Center for Dermatology, Rutgers Robert Wood Johnson Medical School, Somerset, New Jersey, USA. FAU - Rubin, Julia AU - Rubin J AD - Independent Researcher, New York, New York, USA. FAU - Rao, Babar K AU - Rao BK AD - Center for Dermatology, Rutgers Robert Wood Johnson Medical School, Somerset, New Jersey, USA. AD - Dermatology, Weill Cornell Medical College, New York, New York, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20201102 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 SB - IM MH - Adult MH - *Ageusia/diagnosis/virology MH - Betacoronavirus/isolation & purification MH - COVID-19 MH - COVID-19 Testing MH - Chilblains/*diagnosis MH - Clinical Laboratory Techniques/methods MH - *Coronavirus Infections/diagnosis/physiopathology MH - Diagnosis, Differential MH - Female MH - Humans MH - *Olfaction Disorders/diagnosis/virology MH - *Pandemics MH - *Pneumonia, Viral/diagnosis/physiopathology MH - SARS-CoV-2 MH - *Toes PMC - PMC7607570 OTO - NOTNLM OT - dermatology OT - infectious diseases COIS- Competing interests: None declared. EDAT- 2020/11/04 06:00 MHDA- 2020/11/11 06:00 PMCR- 2020/11/02 CRDT- 2020/11/03 05:38 PHST- 2020/11/03 05:38 [entrez] PHST- 2020/11/04 06:00 [pubmed] PHST- 2020/11/11 06:00 [medline] PHST- 2020/11/02 00:00 [pmc-release] AID - 13/11/e237917 [pii] AID - bcr-2020-237917 [pii] AID - 10.1136/bcr-2020-237917 [doi] PST - epublish SO - BMJ Case Rep. 2020 Nov 2;13(11):e237917. doi: 10.1136/bcr-2020-237917. PMID- 31801327 OWN - NLM STAT- MEDLINE DCOM- 20191223 LR - 20220411 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 67 IP - 12 DP - 2019 Dec TI - Non-ischemic Cardiomyopathy: Role of Immunologic Work-up and Cardiac MRI in Etiologic Diagnosis and Outcomes. PG - 30-34 AB - OBJECTIVE: Study etiology of Non ischemic cardiomyopathy (NICMP) and role of cardiac MRI in diagnosis and outcomes. METHODS: prospective observational study. INCLUSION CRITERIA: 1. Clinical feature of cardiac failure, 2. 2D ECHO Systolic dysfunction, EF <45% OR Diastolic dysfunction without regional wall motion abnormality, 3. Absent ischemic changes on ECG and/ or coronary angiography. Exclusion: Valvular & congenital heart disease, Cor pulmonale, Renal failure. Patient were subjected to CBC with absolute eosinophil count(AEC), ESR, Urine-r/m, NT-Pro-BNP, ANA, ANCA, ACE, Bone marrow, Amyloid fat pad biopsy etc., chest x ray, 2DE. HRCT Chest and coronary angiography, Cardiac MRI by 3 tesla MRI machine. Patients were treated with antic-failure drugs & as per etiology and followed at 6wk clinically (NYHA) and 2DE. RESULT: forty four Patients, mean age 36 yrs F: M(22:22), many patients had feature other than cardiac failure like Raynaud's. CI - © Journal of the Association of Physicians of India 2011. FAU - Malgutte, Deepak AU - Malgutte D AD - Ex-Resident, Department of Medicine , Corresponding Author. FAU - Waghmare, Irawati AU - Waghmare I AD - Registrar, Department of Medicine. FAU - Bhute, Vishnu AU - Bhute V AD - Assistant Professor in Cardiology Department. FAU - Joshi, Angaha AU - Joshi A AD - Professor in Radiology Department. FAU - Gokhale, Yojana AU - Gokhale Y AD - Professor and In-charge of Rheumatology Services, Department of Medicine, Lokamanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai, Maharashtra. LA - eng PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Adult MH - Cardiomyopathies/*diagnosis MH - Coronary Angiography MH - *Heart Failure MH - Humans MH - Magnetic Resonance Imaging MH - Prospective Studies EDAT- 2019/12/06 06:00 MHDA- 2019/12/24 06:00 CRDT- 2019/12/06 06:00 PHST- 2019/12/06 06:00 [entrez] PHST- 2019/12/06 06:00 [pubmed] PHST- 2019/12/24 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2019 Dec;67(12):30-34. PMID- 18784136 OWN - NLM STAT- MEDLINE DCOM- 20090514 LR - 20260128 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 47 Suppl 5 DP - 2008 Oct TI - Outcome measures in rheumatologic clinical trials and systemic sclerosis. PG - v29-30 LID - 10.1093/rheumatology/ken269 [doi] AB - OMERACT (Outcome Measures in Rheumatologic Clinical Trials) is a loose organization of rheumatologists, epidemiologists and statisticians whose aim is to improve measurements in the rheumatic diseases. In this context, some SSc measures of response have been found to be valid: the modified Rodnan skin score, the Raynaud's condition score, the forced vital capacity as part of pulmonary function tests, right heart catheterization haemodynamics, serum creatinine, blood pressure and complete blood counts in scleroderma renal crisis and serum creatine phosphokinase as a measure of muscle disease in SSc. Other measures are being tested and have nearly been validated, including the gastrointestinal questionnaire in SSc. Finally, some measures have been found wanting or are not fully tested--in this case for pulmonary arterial hypertension, where effort is presently being focused. These include the echocardiogram, high-resolution CT scan of the lungs, pulmonary function tests, 6-min walking test and MRI. FAU - Furst, D E AU - Furst DE AD - Geffen School of Medicine, University of California, Los Angeles, CA, USA. defurst@mednet.ucla.edu LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Consensus Statements as Topic MH - Endpoint Determination MH - Health Status Indicators MH - Humans MH - Rheumatic Diseases/*drug therapy/physiopathology MH - Scleroderma, Systemic/blood/*drug therapy/physiopathology MH - Treatment Outcome EDAT- 2008/09/17 09:00 MHDA- 2009/05/15 09:00 CRDT- 2008/09/17 09:00 PHST- 2008/09/17 09:00 [pubmed] PHST- 2009/05/15 09:00 [medline] PHST- 2008/09/17 09:00 [entrez] AID - ken269 [pii] AID - 10.1093/rheumatology/ken269 [doi] PST - ppublish SO - Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v29-30. doi: 10.1093/rheumatology/ken269. PMID- 37762589 OWN - NLM STAT- MEDLINE DCOM- 20260310 LR - 20260310 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 24 IP - 18 DP - 2023 Sep 19 TI - The Pathogenesis of Systemic Sclerosis: The Origin of Fibrosis and Interlink with Vasculopathy and Autoimmunity. LID - 10.3390/ijms241814287 [doi] LID - 14287 AB - Systemic sclerosis (SSc) is an autoimmune disease associated with increased mortality and poor morbidity, impairing the quality of life in patients. Whilst we know that SSc affects multiple organs via vasculopathy, inflammation, and fibrosis, its exact pathophysiology remains elusive. Microvascular injury and vasculopathy are the initial pathological features of the disease. Clinically, the vasculopathy in SSc is manifested as Raynaud's phenomenon (reversible vasospasm in reaction to the cold or emotional stress) and digital ulcers due to ischemic injury. There are several reports that medications for vasculopathy, such as bosentan and soluble guanylate cyclase (sGC) modulators, improve not only vasculopathy but also dermal fibrosis, suggesting that vasculopathy is important in SSc. Although vasculopathy is an important initial step of the pathogenesis for SSc, it is still unclear how vasculopathy is related to inflammation and fibrosis. In this review, we focused on the clinical evidence for vasculopathy, the major cellular players for the pathogenesis, including pericytes, adipocytes, endothelial cells (ECs), and myofibroblasts, and their signaling pathway to elucidate the relationship among vasculopathy, inflammation, and fibrosis in SSc. FAU - Ko, Junsuk AU - Ko J AD - Duke-National University of Singapore Medical School, Singapore 169857, Singapore. FAU - Noviani, Maria AU - Noviani M AD - Duke-National University of Singapore Medical School, Singapore 169857, Singapore. AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore 169608, Singapore. AD - Translational Immunology Institute, SingHealth Duke-National University of Singapore Academic Medical Centre, Singapore 169856, Singapore. FAU - Chellamuthu, Vasuki Ranjani AU - Chellamuthu VR AD - Translational Immunology Institute, SingHealth Duke-National University of Singapore Academic Medical Centre, Singapore 169856, Singapore. FAU - Albani, Salvatore AU - Albani S AD - Duke-National University of Singapore Medical School, Singapore 169857, Singapore. AD - Translational Immunology Institute, SingHealth Duke-National University of Singapore Academic Medical Centre, Singapore 169856, Singapore. FAU - Low, Andrea Hsiu Ling AU - Low AHL AUID- ORCID: 0000-0002-5244-686X AD - Duke-National University of Singapore Medical School, Singapore 169857, Singapore. AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore 169608, Singapore. LA - eng GR - MN is supported by the National Medical Research Council (NMRC) Clinician Scientist Seed Funding (MOH-001324-00). SA holds grant support from NMRC (NMRC/OFLCG/002/2018, CIRG19may0052), MOH-STaR19nov-0002, A*STAR PEC21-H22P0M0003, Duke-NUS and SingHealth A/National Medical Research Council/ GR - JK is supported by the SingHealth Medical Student Talent Development Awards (SMSTDA, Project and Travel Awards)./SingHealth Academy of Medicine/ PT - Journal Article PT - Review DEP - 20230919 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 SB - IM MH - Humans MH - *Scleroderma, Systemic/pathology/immunology/etiology/metabolism MH - Fibrosis MH - *Autoimmunity MH - Animals MH - Signal Transduction MH - Endothelial Cells/metabolism/pathology MH - *Vascular Diseases/pathology PMC - PMC10532389 OTO - NOTNLM OT - Raynaud OT - autoimmunity OT - fibrosis OT - inflammation OT - pathogenesis OT - scleroderma OT - systemic sclerosis OT - vasculopathy COIS- A.H.L. Low declares consulting fees from Boehringer Ingelheim and Johnson & Johnson. Other authors declare no competing interest. EDAT- 2023/09/28 06:42 MHDA- 2026/03/10 18:36 PMCR- 2023/09/19 CRDT- 2023/09/28 01:20 PHST- 2023/07/10 00:00 [received] PHST- 2023/09/05 00:00 [revised] PHST- 2023/09/13 00:00 [accepted] PHST- 2026/03/10 18:36 [medline] PHST- 2023/09/28 06:42 [pubmed] PHST- 2023/09/28 01:20 [entrez] PHST- 2023/09/19 00:00 [pmc-release] AID - ijms241814287 [pii] AID - ijms-24-14287 [pii] AID - 10.3390/ijms241814287 [doi] PST - epublish SO - Int J Mol Sci. 2023 Sep 19;24(18):14287. doi: 10.3390/ijms241814287. PMID- 20374346 OWN - NLM STAT- MEDLINE DCOM- 20100702 LR - 20100408 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 12 IP - 3 DP - 2009 Sep TI - The manifestations of vasculopathy in systemic sclerosis and its evidence-based therapy. PG - 192-206 LID - 10.1111/j.1756-185X.2009.01410.x [doi] AB - Fibrosis, inflammation and vascular dysfunction are major features of systemic sclerosis and the multiple organ-specific complications that characterize this disease. Several manifestations of systemic sclerosis, including Raynaud's phenomenon, digital ulceration, scleroderma renal crisis and pulmonary arterial hypertension, contribute significantly to morbidity and mortality and are understood to share similarities in their underlying vasculopathy. In recent years, a number of treatment options have become available that ease the burden of these manifestations, including calcium channel blockers, angiotensin converting enzyme inhibitors, prostanoids and prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase type-5 inhibitors. Several of these treatments have demonstrated beneficial effects against more than one complication, as a result of the similarities in the pathology underlying these manifestations. However, physicians involved in the management of patients with systemic sclerosis are faced with differing levels of evidence supporting these treatments, and historically little international consensus on the treatment of some manifestations, such as digital ulcers. The aim of this article is to evaluate the level of evidence supporting each intervention in systemic sclerosis, thereby facilitating decision-making in the clinic. FAU - Strange, Geoff AU - Strange G AD - Department of Epidemiology & Preventive Medicine, Monash University, Victoria, Australia. FAU - Nash, Peter AU - Nash P LA - eng PT - Journal Article PT - Review PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Clinical Trials as Topic MH - *Evidence-Based Medicine MH - Humans MH - Scleroderma, Systemic/complications/*physiopathology/*therapy MH - Vascular Diseases/etiology/*physiopathology/*therapy RF - 70 EDAT- 2010/04/09 06:00 MHDA- 2010/07/03 06:00 CRDT- 2010/04/09 06:00 PHST- 2010/04/09 06:00 [entrez] PHST- 2010/04/09 06:00 [pubmed] PHST- 2010/07/03 06:00 [medline] AID - APL1410 [pii] AID - 10.1111/j.1756-185X.2009.01410.x [doi] PST - ppublish SO - Int J Rheum Dis. 2009 Sep;12(3):192-206. doi: 10.1111/j.1756-185X.2009.01410.x. PMID- 40388753 OWN - NLM STAT- MEDLINE DCOM- 20250519 LR - 20250522 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 104 IP - 20 DP - 2025 May 16 TI - Systemic sclerosis complicating endometrial adenocarcinoma: A case report. PG - e42553 LID - 10.1097/MD.0000000000042553 [doi] LID - e42553 AB - RATIONALE: Systemic sclerosis (SSc) is a rare systemic autoimmune disease, and it is even more uncommon to encounter it alongside a neoplasm. PATIENT CONCERNS: A 55-year-old female patient presented to our clinic with a 7-year history of cyanosis affecting multiple fingers bilaterally, accompanied by Raynaud's phenomenon, mild pain, numbness, and morning stiffness. One and a half years ago, she was diagnosed with endometrial adenocarcinoma (Federation International of Gynecology and Obstetrics stage IIIa) for frequent abdominal pain and abnormal vaginal bleeding. DIAGNOSES: Following a comprehensive physical examination and laboratory tests, she was diagnosed with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria. INTERVENTIONS: Although we discussed potential treatment options and prognosis with her, she ultimately declined therapy. OUTCOMES: She was lost to follow-up. LESSONS: A shared pathophysiological process may underlie the development of both SSc and malignancy, possibly driven by persistent chronic inflammation and immune dysregulation. CI - Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Yan, Hui-Chang AU - Yan HC AUID- ORCID: 0000-0002-5973-1970 AD - Department of Dermatology, Henan General Hospital, Zhengzhou, Henan Province, China. FAU - Huang, Cui AU - Huang C LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Humans MH - Female MH - *Endometrial Neoplasms/complications/diagnosis MH - Middle Aged MH - *Scleroderma, Systemic/complications/diagnosis MH - *Adenocarcinoma/complications PMC - PMC12091651 OTO - NOTNLM OT - endometrial adenocarcinoma OT - malignancy OT - pathogenesis OT - scleroderma OT - systemic sclerosis COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2025/05/19 18:34 MHDA- 2025/05/19 18:35 PMCR- 2025/05/16 CRDT- 2025/05/19 16:33 PHST- 2025/05/19 18:35 [medline] PHST- 2025/05/19 18:34 [pubmed] PHST- 2025/05/19 16:33 [entrez] PHST- 2025/05/16 00:00 [pmc-release] AID - 00005792-202505160-00034 [pii] AID - MD-D-24-15953 [pii] AID - 10.1097/MD.0000000000042553 [doi] PST - ppublish SO - Medicine (Baltimore). 2025 May 16;104(20):e42553. doi: 10.1097/MD.0000000000042553. PMID- 34105473 OWN - NLM STAT- MEDLINE DCOM- 20210610 LR - 20210610 IS - 1009-2137 (Print) IS - 1009-2137 (Linking) VI - 29 IP - 3 DP - 2021 Jun TI - [Early Diagnosis of One Multiple Myeloma Patient with Cold Agglutinin Syndrome as the Initial Presentation]. PG - 787-790 LID - 10.19746/j.cnki.issn.1009-2137.2021.03.021 [doi] AB - OBJECTIVE: To analyze one case of multiple myeloma (MM) initially presenting cold agglutinin syndrome (CAS), so as to improve clinical understanding and screening of this disease. METHODS: The clinical data, laboratory examination, bone marrow result, diagnosis and treatment of the patient were analyzed and summarized to provide ideas and clinical experience for the early diagnosis and treatment of CAS secondary to MM. RESULTS: The clinical manifestations of asthenia, hemolysis, jaundice and scattered livedo reticularis were caused by CAS secondary to MM, which was different from the general Raynaud's phenomenon. IgMκ type MM was definitely diagnosed according to the morphological features of bone marrow cells and immunofixation electrophoresis. After 3 courses of chemotherapy with BAD regimen and enhanced thermal support, anemia was corrected, M protein was decreased and the cold agglutinin phenomenon was significantly reduced. The evaluation of efficacy reached very good partial response. CONCLUSION: There are very few MM patients with CAS as the initial presentation, so it is easy to misdiagnose. Early diagnosis and individual therapy are particularly important, which requires clinicians to observe and gain experience further. FAU - Wang, Ya-Ru AU - Wang YR AD - Department of Hematology, The Second Hospital of Shanxi Medical University, Taiyuan 030001, Shanxi Province, China. FAU - Ma, Yan-Ping AU - Ma YP AD - Department of Hematology, The Second Hospital of Shanxi Medical University, Taiyuan 030001, Shanxi Province, China,E-mail: myanp18@163.com. LA - chi PT - Journal Article PL - China TA - Zhongguo Shi Yan Xue Ye Xue Za Zhi JT - Zhongguo shi yan xue ye xue za zhi JID - 101084424 RN - 0 (Cryoglobulins) RN - 0 (cold agglutinins) SB - IM MH - *Anemia, Hemolytic, Autoimmune/diagnosis MH - Cryoglobulins MH - Early Diagnosis MH - Humans MH - *Multiple Myeloma/diagnosis EDAT- 2021/06/10 06:00 MHDA- 2021/06/11 06:00 CRDT- 2021/06/09 08:45 PHST- 2021/06/09 08:45 [entrez] PHST- 2021/06/10 06:00 [pubmed] PHST- 2021/06/11 06:00 [medline] AID - 1009-2137(2021)03-0787-04 [pii] AID - 10.19746/j.cnki.issn.1009-2137.2021.03.021 [doi] PST - ppublish SO - Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2021 Jun;29(3):787-790. doi: 10.19746/j.cnki.issn.1009-2137.2021.03.021. PMID- 23410557 OWN - NLM STAT- MEDLINE DCOM- 20130411 LR - 20130215 IS - 1555-7162 (Electronic) IS - 0002-9343 (Linking) VI - 126 IP - 3 DP - 2013 Mar TI - Phosphodiesterase type 5 inhibitors improve endothelial function and may benefit cardiovascular conditions. PG - 192-9 LID - S0002-9343(12)00832-7 [pii] LID - 10.1016/j.amjmed.2012.08.015 [doi] AB - The effects of phosphodiesterase type 5 inhibitors on vasodilation mediated via nitric oxide-cyclic guanosine monophosphate are well described. Less is known about other mechanisms through which phosphodiesterase type 5 inhibitors benefit endothelial function, including normalization of serum biomarkers, increased levels of endothelial progenitor cells, ischemia-reperfusion protection mechanisms, and other actions specific to patients with diabetes. These various mechanisms are reviewed. Their impact on several cardiovascular diseases, including erectile dysfunction, pulmonary hypertension, heart failure, high-altitude pulmonary edema, Raynaud's phenomenon, coronary artery disease, diabetes, and atherosclerosis, is presented. CI - Copyright © 2013 Elsevier Inc. All rights reserved. FAU - Schwartz, Bryan G AU - Schwartz BG AD - Heart Institute, Good Samaritan Hospital, Los Angeles, Calif 90017-2395, USA. FAU - Jackson, Graham AU - Jackson G FAU - Stecher, Vera J AU - Stecher VJ FAU - Campoli-Richards, Deborah M AU - Campoli-Richards DM FAU - Kloner, Robert A AU - Kloner RA LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Am J Med JT - The American journal of medicine JID - 0267200 RN - 0 (Phosphodiesterase 5 Inhibitors) SB - IM MH - Cardiovascular Diseases/*drug therapy/physiopathology MH - Endothelium, Vascular/*drug effects/physiology MH - Hemodynamics/drug effects/physiology MH - Humans MH - Ischemic Preconditioning MH - Phosphodiesterase 5 Inhibitors/pharmacology/*therapeutic use MH - Vasodilation/drug effects/physiology EDAT- 2013/02/16 06:00 MHDA- 2013/04/12 06:00 CRDT- 2013/02/16 06:00 PHST- 2012/07/30 00:00 [received] PHST- 2012/08/29 00:00 [revised] PHST- 2012/08/29 00:00 [accepted] PHST- 2013/02/16 06:00 [entrez] PHST- 2013/02/16 06:00 [pubmed] PHST- 2013/04/12 06:00 [medline] AID - S0002-9343(12)00832-7 [pii] AID - 10.1016/j.amjmed.2012.08.015 [doi] PST - ppublish SO - Am J Med. 2013 Mar;126(3):192-9. doi: 10.1016/j.amjmed.2012.08.015. PMID- 21061555 OWN - NLM STAT- MEDLINE DCOM- 20101217 LR - 20190918 IS - 0009-918X (Print) IS - 0009-918X (Linking) VI - 50 IP - 10 DP - 2010 Oct TI - [Dropped head sign and tongue atrophy in systemic sclerosis-associated myopathy: a case report]. PG - 732-5 AB - We report a 70-year-old man with a 5-year history of Raynaud's phenomenon and slow progression of weight loss, easy fatigability, muscle weakness, skin sclerosis, and dropped head sign. The patient was assumed to have motor neuron disease by his attending physician, and was referred to our hospital. Physical examination showed skin sclerosis on the fingers, hands, forearms, face, and neck. Neurological examination showed advanced systemic muscle atrophy and weakness, especially in the neck, tongue, and proximal limb muscles. Fasciculations were not observed in these involved muscles. Deep tendon reflexes were hypoactive and pathological reflexes were negative. Sensory disturbance was absent. Laboratory tests showed moderately elevated serum creatine kinase level, and increased serum antinuclear antibody titer (1:5120 with a nucleolar pattern). Needle electromyography showed a typical myogenic pattern in proximal muscles. The patient was diagnosed as having systemic sclerosis (SSc) with SSc-associated myopathy. Severe systemic muscle involvements, including dropped head sign and tongue atrophy, are rare manifestations in patients with SSc-associated myopathy. Our observations suggest that it is essential to pay attention to the dermatological findings of SSc in differential diagnosis of patients with muscular atrophy. FAU - Yoshinaga, Tsuneaki AU - Yoshinaga T AD - Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine. FAU - Miyazaki, Daigo AU - Miyazaki D FAU - Fukushima, Kazuhiro AU - Fukushima K FAU - Shimojima, Yasuhiro AU - Shimojima Y FAU - Matsuda, Masayuki AU - Matsuda M FAU - Ikeda, Shu-ichi AU - Ikeda S LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 SB - IM MH - Aged MH - Head MH - Humans MH - Male MH - Muscle Weakness/etiology MH - Muscular Diseases/*etiology/pathology MH - Scleroderma, Systemic/*complications MH - Tongue/*pathology EDAT- 2010/11/11 06:00 MHDA- 2010/12/18 06:00 CRDT- 2010/11/11 06:00 PHST- 2010/11/11 06:00 [entrez] PHST- 2010/11/11 06:00 [pubmed] PHST- 2010/12/18 06:00 [medline] AID - 10.5692/clinicalneurol.50.732 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2010 Oct;50(10):732-5. doi: 10.5692/clinicalneurol.50.732. PMID- 20187500 OWN - NLM STAT- MEDLINE DCOM- 20100409 LR - 20131121 IS - 0465-5893 (Print) IS - 0465-5893 (Linking) VI - 60 IP - 6 DP - 2009 TI - [Nervous system disorders induced by occupational exposure to arsenic and its inorganic compounds: a literature review]. PG - 519-22 AB - This paper presents a review of the effect of arsenic (As) and its inorganic compounds on the nervous system. In humans, inhalation exposure mostly occurs in occupational conditions. In the occupational environment, the most extensive exposure to this element is observed in the copper industry. Chronic As poisoning is manifested by skin and mucous membrane lesions, impairment of the nervous system in the form of disorders of psychic functions and polyneuropathies, retrobulbar neuritis, disorders of peripheral circulation and the risk for Raynaud's syndrome. Arsenic-induced polyneuropathy is usually a very serious and chronic disease. A complete recovery is observed in only 15-20% of patients. As-induced encephalopathy is an irreversible process. FAU - Sińczuk-Walczak, Halina AU - Sińczuk-Walczak H AD - Instytut Medycyny Pracy im. prof, J. Nofera, Łódź Przychodnia Chorób Zawodowych. pchz@imp.lodz.pl LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Zmiany w układzie nerwowym w nastipstwie narazenia zawodowego na arsen i zwiizki nieorganiczne arsenu w swietle piśmiennictwa. PL - Poland TA - Med Pr JT - Medycyna pracy JID - 0376642 RN - 0 (Air Pollutants, Occupational) RN - N712M78A8G (Arsenic) SB - IM MH - Air Pollutants, Occupational/*toxicity/urine MH - Arsenic/*toxicity/urine MH - Arsenic Poisoning/etiology MH - Humans MH - Nervous System Diseases/*chemically induced/prevention & control MH - Occupational Diseases/*chemically induced/prevention & control MH - Occupational Exposure/*adverse effects MH - Primary Prevention/organization & administration MH - Risk Factors RF - 24 EDAT- 2009/01/01 00:00 MHDA- 2010/04/10 06:00 CRDT- 2010/03/02 06:00 PHST- 2010/03/02 06:00 [entrez] PHST- 2009/01/01 00:00 [pubmed] PHST- 2010/04/10 06:00 [medline] PST - ppublish SO - Med Pr. 2009;60(6):519-22. PMID- 39490526 OWN - NLM STAT- MEDLINE DCOM- 20250106 LR - 20250407 IS - 2254-8874 (Electronic) IS - 2254-8874 (Linking) VI - 225 IP - 1 DP - 2025 Jan TI - Anti-NOR90 antibodies and their clinical significance: a multicenter experience in southern Spain. PG - 51-55 LID - S2254-8874(24)00138-3 [pii] LID - 10.1016/j.rceng.2024.10.008 [doi] AB - INTRODUCTION: Anti-NOR90 antibodies were initially described in patients with autoimmune diseases based on staining of a nucleolar region known as the nucleolar organizer region (NOR). This study aims to explore the clinical aspects of anti-NOR90 antibodies in patients with systemic autoimmune diseases. METHODS: Observational study of patients with positive anti-NOR90 antibodies using the EUROLINE Systemic Sclerosis profile (IgG) kit (Euroimmun, Germany). Data on demographics, comorbidities, autoimmune diseases, treatment, and clinical manifestations were collected. RESULTS: Fifteen Patients with positive anti-NOR90 antibodies were included. Majority were female (86.7%), with median age of 54 years. Most common clinical manifestations were Raynaud's phenomenon, dryness, and interstitial lung disease (ILD). Some patients had hematological or solid organ neoplasms. EPID was prevalent, with one case showing rapid progression requiring aggressive treatment. DISCUSSION AND CONCLUSION: This study highlights the association between anti-NOR90 antibodies and systemic autoimmune diseases, particularly SS and ES. EPID was a notable feature, suggesting its consideration in IPAF diagnosis for anti-NOR90 positive patients. Further multicenter studies are needed for better understanding and detection methods optimization. CI - Copyright © 2024 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved. FAU - Martínez de Victoria Carazo, J AU - Martínez de Victoria Carazo J AD - Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Clínico San Cecilio, Granada, Spain. Electronic address: javier.martinezvictoria.sspa@juntadeandalucia.es. FAU - Fernández Reyes, D AU - Fernández Reyes D AD - Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Clínico San Cecilio, Granada, Spain. FAU - de la Hera Fernández, F J AU - de la Hera Fernández FJ AD - Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Clínico San Cecilio, Granada, Spain. FAU - González Cejudo, T AU - González Cejudo T AD - Laboratorio de Análisis Clínicos, Sección de Inmunología, Hospital Universitario Clínico San Cecilio, Granada, Spain. FAU - Navarrete Navarrete, N AU - Navarrete Navarrete N AD - Laboratorio de Análisis Clínicos, Sección de Inmunología, Hospital Universitario Clínico San Cecilio, Granada, Spain; Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Virgen de las Nieves, Granada, Spain. FAU - Callejas Rubio, J L AU - Callejas Rubio JL AD - Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Clínico San Cecilio, Granada, Spain. LA - eng PT - Case Reports PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20241028 PL - Spain TA - Rev Clin Esp (Barc) JT - Revista clinica espanola JID - 101632437 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Female MH - Male MH - Middle Aged MH - Spain MH - Aged MH - Adult MH - *Autoantibodies/blood MH - Autoimmune Diseases/immunology MH - Scleroderma, Systemic/immunology MH - Clinical Relevance OTO - NOTNLM OT - Autoantibodies OT - Autoanticuerpos OT - Enfermedad pulmonar intersticial difusa OT - Esclerosis sistémica OT - Interstitial pulmonary disease OT - NOR-90 OT - Systemic sclerosis COIS- Declaration of competing interest There are no conflicts of interest. EDAT- 2024/11/04 00:31 MHDA- 2025/01/07 00:21 CRDT- 2024/11/03 23:53 PHST- 2025/01/07 00:21 [medline] PHST- 2024/11/04 00:31 [pubmed] PHST- 2024/11/03 23:53 [entrez] AID - S2254-8874(24)00138-3 [pii] AID - 10.1016/j.rceng.2024.10.008 [doi] PST - ppublish SO - Rev Clin Esp (Barc). 2025 Jan;225(1):51-55. doi: 10.1016/j.rceng.2024.10.008. Epub 2024 Oct 28. PMID- 34984145 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220107 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 11 DP - 2021 Nov TI - Diagnostic Dilemma of Paraneoplastic Rheumatic Disorders: Case Series and Narrative Review. PG - e19993 LID - 10.7759/cureus.19993 [doi] LID - e19993 AB - Paraneoplastic rheumatic disorder (RD) is a disorder that may present before, concurrent with, or after the diagnosis of malignancy. Paraneoplastic RDs are a clinical expression of occult cancer that is not directly related to a tumor or metastasis and manifests as rheumatoid symptoms. The RD is determined by the organ system affected by articular, muscular, cutaneous, vascular, or miscellaneous symptoms. Each case is challenging to diagnose because cancer may present with similar symptoms as a common rheumatic disorder. Of note, the majority of cases have minimal responsiveness or no responsiveness to standard rheumatoid treatment. Therefore, it is imperative to recognize and treat the underlying cancer accordingly. Herein, we present four different diagnostic dilemma cases of RD: case #1 - leukocytoclastic vasculitis and C3 glomerulopathy, case #2 - scleroderma, case #3 - Raynaud's syndrome and possible lupus-like syndrome, and case #4 - inflammatory myositis. Institutional IRB approval was obtained for this case series. We will discuss and review the literature on each topic. In addition, we will mention a review of paraneoplastic rheumatoid arthritis. As rheumatic disease is associated with the use of immune checkpoint inhibitors (ICIs) for cancer treatment, we will briefly discuss some of the most common rheumatic presentations in the setting of these drugs. This case review aims to inform clinicians about the atypical presentation of paraneoplastic RD and to highlight the need for interdisciplinary management between rheumatologists, oncologists, and primary care practitioners. CI - Copyright © 2021, Cho et al. FAU - Cho, Youngmin AU - Cho Y AD - Internal Medicine, Northeast Georgia Medical Center Gainesville, Gainesville, USA. FAU - Anderson, Erik W AU - Anderson EW AD - Rheumatology, Coney Island Hospital, Brooklyn, USA. FAU - Guevara, Sara J AU - Guevara SJ AD - Surgery, North Shore University Hospital, Manhasset, USA. FAU - Miyara, Santiago J AU - Miyara SJ AD - Critical Care, North Shore University Hospital, Manhasset, USA. FAU - Maria, Naomi AU - Maria N AD - Molecular Medicine, Feinstein Institutes for Medical Research, Manhasset, USA. FAU - Metz, Christine N AU - Metz CN AD - Molecular Medicine, Feinstein Institutes for Medical Research, Manhasset, USA. FAU - Zafeiropoulos, Stefanos AU - Zafeiropoulos S AD - Cardiology, Serres General Hospital, Serres, GRC. FAU - Giannis, Dimitrios AU - Giannis D AD - Molecular Medicine, Feinstein Institutes for Medical Research, Manhasset, USA. FAU - Wang, Jifeng AU - Wang J AD - Internal Medicine, Northeast Georgia Medical Center Gainesville, Gainesville, USA. FAU - Abidoye, Oluseyi AU - Abidoye O AD - Internal Medicine, Northeast Georgia Medical Center Gainesville, Gainesville, USA. FAU - Mumford, James M AU - Mumford JM AD - Family Medicine, Glen Cove Hospital, Glen Cove, USA. FAU - Aronsohn, Judith AU - Aronsohn J AD - Anesthesiology, North Shore University Hospital, Manhasset, USA. FAU - Molmenti, Ernesto AU - Molmenti E AD - Transplant Surgery, North Shore University Hospital, Manhasset, USA. FAU - Sohail, Huma AU - Sohail H AD - Rheumatology, Northeast Georgia Medical Center Gainesville, Gainesville, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20211129 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8715838 OTO - NOTNLM OT - autoimmunity OT - connective tissue disease OT - malignancy OT - paraneoplastic syndromes OT - rheumatoid disorder COIS- The authors have declared that no competing interests exist. EDAT- 2022/01/06 06:00 MHDA- 2022/01/06 06:01 PMCR- 2021/11/29 CRDT- 2022/01/05 05:56 PHST- 2021/11/29 00:00 [accepted] PHST- 2022/01/05 05:56 [entrez] PHST- 2022/01/06 06:00 [pubmed] PHST- 2022/01/06 06:01 [medline] PHST- 2021/11/29 00:00 [pmc-release] AID - 10.7759/cureus.19993 [doi] PST - epublish SO - Cureus. 2021 Nov 29;13(11):e19993. doi: 10.7759/cureus.19993. eCollection 2021 Nov. PMID- 33614339 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240330 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 1 DP - 2021 Jan 16 TI - A Clinical Case of Polymyositis Complicated by Antisynthetase Syndrome. PG - e12737 LID - 10.7759/cureus.12737 [doi] LID - e12737 AB - Antisynthetase syndrome is an autoimmune condition that manifests clinically through signs and symptoms, such as interstitial lung disease, myositis, Raynaud's phenomenon, fever, hyperkeratotic fingertips (mechanic's hands), and arthritis. It is associated with antibodies against aminoacyl tRNA synthetase enzyme, the most common autoantibody being the anti-Jo-1. An 18-year-old girl presented with weakness of both the upper and lower limb, swelling and generalized body pain, difficulty in swallowing. MRI of the thigh was highly suggestive of myositis with symmetrical bilateral involvement. Based on proximal muscle weakness, elevated creatine phosphokinase (CPK), and lactate dehydrogenase (LDH), strongly positive anti-nuclear antibodies human epithelial cell type-2 (ANA-HEp2), and a normal nerve conduction velocity test with precise MRI findings, a diagnosis of polymyositis was made. She was given bolus intravenous methylprednisolone for five days, followed by oral methylprednisolone with subcutaneous methotrexate weekly. She reported a 50% improvement in muscle weakness; however, partial bulbar weakness persisted at the time of discharge. On her next follow-up, her blood investigations for auto-antibodies were done. The autoantibodies anti-Jo-1 (3+), Ro-52 (2+), and Mi-2β (2+) were found to be positive. These investigations, coupled with the clinical features she was presenting, finally led us to conclude that it was a case of polymyositis complicated by the antisynthetase syndrome. CI - Copyright © 2021, Khan et al. FAU - Khan, Aadil M AU - Khan AM AD - Internal Medicine, Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, IND. FAU - Ahmad, Faim Jr AU - Ahmad F Jr AD - Internal Medicine, Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, IND. FAU - Rehman, Usama AU - Rehman U AD - Anesthesia, Mayo Hospital, Lahore, PAK. FAU - Jindal, Himanshu AU - Jindal H AD - General Medicine, Ganesh Shankar Vidyarthi Memorial Medical College and Lala Lajpat Rai Hospital, Kanpur, IND. FAU - Harimohan, Hridya AU - Harimohan H AD - Internal Medicine, Kerala Institute of Medical Sciences, Trivandrum, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20210116 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7883527 OTO - NOTNLM OT - aminoacyl trna synthetase OT - anti-jo-1 antibodies OT - antisynthetase syndrome OT - interstitial lung disease OT - myositis COIS- The authors have declared that no competing interests exist. EDAT- 2021/02/23 06:00 MHDA- 2021/02/23 06:01 PMCR- 2021/01/16 CRDT- 2021/02/22 05:56 PHST- 2021/02/22 05:56 [entrez] PHST- 2021/02/23 06:00 [pubmed] PHST- 2021/02/23 06:01 [medline] PHST- 2021/01/16 00:00 [pmc-release] AID - 10.7759/cureus.12737 [doi] PST - epublish SO - Cureus. 2021 Jan 16;13(1):e12737. doi: 10.7759/cureus.12737. PMID- 28904690 OWN - NLM STAT- MEDLINE DCOM- 20170926 LR - 20221207 IS - 1937-8688 (Electronic) VI - 27 DP - 2017 TI - [Mixed connective tissue disease: prevalence and clinical characteristics in African black, study of 7 cases in Gabon and review of the literature]. PG - 162 LID - 10.11604/pamj.2017.27.162.12572 [doi] LID - 162 AB - The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015. For each case of MCTD the parameters studied were articular and extra-articular manifestations, anti-U1RNP antibodies levels, patient's evolution. Over a period of 6 years, data were collected by medical records of 7 patients out of 6050 patients and 67 cases of connective tissue disorders, reflecting a prevalence of 0.11% and 10.44% respectively. the 7 patients were women (100%), with an average age of 39.5 years. Articular manifestations included: polyarthritis, myalgias, chubby fingers and Raynaud's phenomenon in 87.5%, 87.5%, 28.6% and 14% respectively. The 7 patients had high anti-U1RNP antibodies levels, ranging between 5 and 35N (N≤ 7 IU). A case of death due to pulmonary arterial hypertension (PAH) was certified. This is the largest case series of MCTD reported in Black Africa. The disease seems to be rare among the black Africans; the reason could be genetic. The demographic and clinical aspects appear similar to those in Caucasians, Asians and Blacks except for a low frequency of Raynaud?s phenomenon among Blacks. FAU - Missounga, Landry AU - Missounga L AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Ba, Josaphat Iba AU - Ba JI AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Nseng Nseng Ondo, Ingrid Rosalie AU - Nseng Nseng Ondo IR AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Nziengui Madjinou, Maria Ines Carine AU - Nziengui Madjinou MIC AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Malekou, Doris AU - Malekou D AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Mouendou Mouloungui, Emeline Gracia AU - Mouendou Mouloungui EG AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Nzengue, Emmanuel Ecke AU - Nzengue EE AD - Service de Cardiologie, Centre Hospitalier Universitaire de Libreville, BP 2228 Libreville, Gabon. FAU - Boguikouma, Jean Bruno AU - Boguikouma JB AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. FAU - Kombila, Moussavou AU - Kombila M AD - Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon. LA - fre PT - Journal Article PT - Review TT - La connectivite mixte: prévalence et caractéristiques cliniques chez le noir africain, étude de 7 cas au Gabon et revue de la littérature. DEP - 20170630 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - 0 (Autoantibodies) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) SB - IM MH - Adolescent MH - Adult MH - Autoantibodies/*immunology MH - Black People MH - Female MH - Gabon/epidemiology MH - Humans MH - Middle Aged MH - Mixed Connective Tissue Disease/epidemiology/immunology/*physiopathology MH - Prevalence MH - Retrospective Studies MH - Ribonucleoprotein, U1 Small Nuclear/*immunology PMC - PMC5567953 OTO - NOTNLM OT - Gabon OT - Mixed connective tissue disease OT - black African OT - prevalence EDAT- 2017/09/15 06:00 MHDA- 2017/09/28 06:00 PMCR- 2017/06/30 CRDT- 2017/09/15 06:00 PHST- 2017/04/20 00:00 [received] PHST- 2017/06/18 00:00 [accepted] PHST- 2017/09/15 06:00 [entrez] PHST- 2017/09/15 06:00 [pubmed] PHST- 2017/09/28 06:00 [medline] PHST- 2017/06/30 00:00 [pmc-release] AID - PAMJ-27-162 [pii] AID - 10.11604/pamj.2017.27.162.12572 [doi] PST - epublish SO - Pan Afr Med J. 2017 Jun 30;27:162. doi: 10.11604/pamj.2017.27.162.12572. eCollection 2017. PMID- 40722841 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250801 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 13 IP - 7 DP - 2025 Jul 19 TI - Systemic Sclerosis: A Key Model of Endothelial Dysfunction. LID - 10.3390/biomedicines13071771 [doi] LID - 1771 AB - Systemic sclerosis (SSc) is a heterogeneous disease characterized by vascular alterations, immune dysregulation, and fibrosis. Solid evidence supports the hypothesis that endothelial dysfunction is the key player in SSc vascular injury and a critical factor concurring to the initiation of SSc pathogenesis. This narrative review reports on persistent endothelial dysfunction, resulting from oxidative stress, autoimmunity, and impaired vascular repair, in the course of SSc, and how it can trigger and sustain fibrotic remodeling of various organs. In this paper, we also analyze the impact on SSc of impaired angiogenesis and vasculogenesis, diminished endothelial progenitor cell function, and endothelial-to-mesenchymal transition, which can collectively disrupt vascular homeostasis and promote myofibroblast activation. These pathologic events underlie the hallmark clinical manifestations, i.e., Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, and scleroderma renal crisis. The review highlights how recognizing SSc as a paradigm of systemic endothelial dysfunction may reframe our understanding of its physiopathology, modify current therapeutic strategies, and unveil new therapeutic targets. FAU - Zaccone, Vincenzo AU - Zaccone V AUID- ORCID: 0000-0002-5285-859X AD - PhD Course in Human Health, Marche Polytechnic University, 60126 Ancona, Italy. FAU - Falsetti, Lorenzo AU - Falsetti L AUID- ORCID: 0000-0003-3411-2388 AD - Clinica Medica, Department of Clinical and Molecular Sciences, Marche Polytechnic University, 60126 Ancona, Italy. FAU - Contegiacomo, Silvia AU - Contegiacomo S AD - Clinica Medica, Department of Clinical and Molecular Sciences, Marche Polytechnic University, 60126 Ancona, Italy. FAU - Cataldi, Serena AU - Cataldi S AD - Department of Pediatrics, Marche University Hospital, 60126 Ancona, Italy. FAU - Benfaremo, Devis AU - Benfaremo D AUID- ORCID: 0000-0002-9867-2360 AD - Clinica Medica, Department of Clinical and Molecular Sciences, Marche Polytechnic University, 60126 Ancona, Italy. FAU - Moroncini, Gianluca AU - Moroncini G AD - Clinica Medica, Department of Clinical and Molecular Sciences, Marche Polytechnic University, 60126 Ancona, Italy. LA - eng PT - Journal Article PT - Review DEP - 20250719 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC12292601 OTO - NOTNLM OT - SSc OT - endothelial dysfunction OT - endothelial-to-mesenchymal transition OT - systemic sclerosis COIS- The authors declare no conflicts of interest. EDAT- 2025/07/29 06:30 MHDA- 2025/07/29 06:31 PMCR- 2025/07/19 CRDT- 2025/07/29 01:05 PHST- 2025/05/13 00:00 [received] PHST- 2025/07/15 00:00 [revised] PHST- 2025/07/18 00:00 [accepted] PHST- 2025/07/29 06:31 [medline] PHST- 2025/07/29 06:30 [pubmed] PHST- 2025/07/29 01:05 [entrez] PHST- 2025/07/19 00:00 [pmc-release] AID - biomedicines13071771 [pii] AID - biomedicines-13-01771 [pii] AID - 10.3390/biomedicines13071771 [doi] PST - epublish SO - Biomedicines. 2025 Jul 19;13(7):1771. doi: 10.3390/biomedicines13071771. PMID- 37915647 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231103 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 85 IP - 11 DP - 2023 Nov TI - Systemic sclerosis sine scleroderma with atypical clinical course: a rare case report. PG - 5656-5661 LID - 10.1097/MS9.0000000000001266 [doi] AB - INTRODUCTION AND IMPORTANCE: Systemic sclerosis (SSc) is divided into three subtypes: limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and systemic sclerosis sine scleroderma (ssSSc). ssSSc is a rare subtype of SSc that presents with internal organ manifestations but no cutaneous findings. CASE PRESENTATION: We report the case of a 58-year-old patient with a history of pulmonary hypertension who presented with symptoms of fatigue, inflammatory polyarthritis, and joint swelling. Following a comprehensive clinical examination and laboratory tests, the patient was diagnosed with ssSSc. CLINICAL DISCUSSION: Due to its atypical clinical course, we present this case report, which commenced with idiopathic pulmonary hypertension. Subsequently, after 7 months, the patient presented complaints of polyarthritis with positive antinuclear antibodies. Raynaud's phenomenon was identified 2 months later during the rheumatology clinic examination. Typically, the clinical course encompasses all three features simultaneously, without any gap between them. CONCLUSION: Diagnosis of ssSSc remains challenging, and it is essential to consider this disease form in all cases involving unexplained fibrotic involvement of the internal organs. CI - Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. FAU - Samha, Raghad AU - Samha R AD - Faculty of Medicine. FAU - Ghaddar, Sawsane A AU - Ghaddar SA AD - Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon. FAU - Raya, Maria AU - Raya M AD - Faculty of Medicine, University of Kalamoon, Damascus, Syrian Arab Republic. FAU - Alhadi, Safaa Abdalhadi AU - Alhadi SA AD - Department of Rheumatology, AlBaath University Hospital, Homs. LA - eng PT - Journal Article DEP - 20230905 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC10617812 OTO - NOTNLM OT - scleroderma OT - systemic sclerosis OT - systemic sclerosis sine scleroderma OT - tadalafil COIS- The authors declare that they have no conflicts of interest. EDAT- 2023/11/02 06:43 MHDA- 2023/11/02 06:44 PMCR- 2023/09/05 CRDT- 2023/11/02 04:03 PHST- 2023/07/31 00:00 [received] PHST- 2023/08/23 00:00 [accepted] PHST- 2023/11/02 06:44 [medline] PHST- 2023/11/02 06:43 [pubmed] PHST- 2023/11/02 04:03 [entrez] PHST- 2023/09/05 00:00 [pmc-release] AID - AMSU-D-23-01659 [pii] AID - 10.1097/MS9.0000000000001266 [doi] PST - epublish SO - Ann Med Surg (Lond). 2023 Sep 5;85(11):5656-5661. doi: 10.1097/MS9.0000000000001266. eCollection 2023 Nov. PMID- 24308674 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150520 LR - 20200929 IS - 1386-6346 (Print) IS - 1386-6346 (Linking) VI - 44 IP - 4 DP - 2014 Apr TI - Pathophysiology and recent findings of primary biliary cirrhosis complicated by systemic sclerosis. PG - 377-83 LID - 10.1111/hepr.12285 [doi] AB - Primary biliary cirrhosis (PBC) can be complicated by systemic sclerosis (SSc) and, more specifically, limited cutaneous SSc (lcSSc), which was previously called CREST syndrome. Moreover, combined PBC and SSc has been described in many case reports. Although neither the etiology of PBC nor that of SSc has been elucidated, some genetic and immunological factors are known to be shared. Both disorders are autoimmune fibrotic diseases characterized by increased levels of profibrotic cytokines transforming growth factor β (TGFβ) and interleukin-6, which have recently been suggested to influence T-helper 17 cells and regulatory T cells involved in acquired immunity. lcSSc is accompanied by CREST symptoms, although complete CREST cases are rare, with relatively high prevalence of Raynaud's phenomenon, sclerodactyly and telangiectasia, and lower prevalence of calcinosis and esophageal dysmotility. Because patients with anticentromere antibody positive PBC-SSc are at a high risk of developing portal hypertension, particular attention should be paid to the management of gastroesophageal varices. In addition, the management of SSc-related non-hepatic disorders, such as pulmonary fibrosis, pulmonary hypertension, heart disorder, infection and malignancy, is also important for improved outcomes. Because PBC is often complicated by rheumatic disease, hepatologists should keep the possibility of systemic disorder in mind when examining PBC patients. CI - © 2013 The Japan Society of Hepatology. FAU - Ohira, Hiromasa AU - Ohira H AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Watanabe, Hiroshi AU - Watanabe H AD - Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan. LA - eng PT - Journal Article DEP - 20140217 PL - Netherlands TA - Hepatol Res JT - Hepatology research : the official journal of the Japan Society of Hepatology JID - 9711801 OTO - NOTNLM OT - CREST syndrome OT - anticentromere antibody OT - primary biliary cirrhosis OT - systemic sclerosis EDAT- 2013/12/07 06:00 MHDA- 2013/12/07 06:01 CRDT- 2013/12/07 06:00 PHST- 2013/10/20 00:00 [received] PHST- 2013/12/02 00:00 [revised] PHST- 2013/12/02 00:00 [accepted] PHST- 2013/12/07 06:00 [entrez] PHST- 2013/12/07 06:00 [pubmed] PHST- 2013/12/07 06:01 [medline] AID - 10.1111/hepr.12285 [doi] PST - ppublish SO - Hepatol Res. 2014 Apr;44(4):377-83. doi: 10.1111/hepr.12285. Epub 2014 Feb 17. PMID- 23213512 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121206 LR - 20220410 IS - 2090-1550 (Electronic) IS - 2090-1542 (Print) IS - 2090-1542 (Linking) VI - 2012 DP - 2012 TI - Fibromyalgia syndrome: etiology, pathogenesis, diagnosis, and treatment. PG - 426130 LID - 10.1155/2012/426130 [doi] LID - 426130 AB - Fibromyalgia syndrome is mainly characterized by pain, fatigue, and sleep disruption. The etiology of fibromyalgia is still unclear: if central sensitization is considered to be the main mechanism involved, then many other factors, genetic, immunological, and hormonal, may play an important role. The diagnosis is typically clinical (there are no laboratory abnormalities) and the physician must concentrate on pain and on its features. Additional symptoms (e.g., Raynaud's phenomenon, irritable bowel disease, and heat and cold intolerance) can be associated with this condition. A careful differential diagnosis is mandatory: fibromyalgia is not a diagnosis of exclusion. Since 1990, diagnosis has been principally based on the two major diagnostic criteria defined by the ACR. Recently, new criteria have been proposed. The main goals of the treatment are to alleviate pain, increase restorative sleep, and improve physical function. A multidisciplinary approach is optimal. While most nonsteroidal anti-inflammatory drugs and opioids have limited benefit, an important role is played by antidepressants and neuromodulating antiepileptics: currently duloxetine (NNT for a 30% pain reduction 7.2), milnacipran (NNT 19), and pregabalin (NNT 8.6) are the only drugs approved by the US Food and Drug Administration for the treatment of fibromyalgia. In addition, nonpharmacological treatments should be associated with drug therapy. FAU - Bellato, Enrico AU - Bellato E AD - Department of Orthopedics and Traumatology, Mauriziano Umberto I Hospital, University of Turin Medical School, Largo Turati 62,10128 Turin, Italy [corrected]. FAU - Marini, Eleonora AU - Marini E FAU - Castoldi, Filippo AU - Castoldi F FAU - Barbasetti, Nicola AU - Barbasetti N FAU - Mattei, Lorenzo AU - Mattei L FAU - Bonasia, Davide Edoardo AU - Bonasia DE FAU - Blonna, Davide AU - Blonna D LA - eng PT - Journal Article DEP - 20121104 PL - United States TA - Pain Res Treat JT - Pain research and treatment JID - 101566863 EIN - Pain Res Treat. 2013;2013:960270 PMC - PMC3503476 EDAT- 2012/12/06 06:00 MHDA- 2012/12/06 06:01 PMCR- 2012/11/04 CRDT- 2012/12/06 06:00 PHST- 2012/06/28 00:00 [received] PHST- 2012/09/09 00:00 [revised] PHST- 2012/09/12 00:00 [accepted] PHST- 2012/12/06 06:00 [entrez] PHST- 2012/12/06 06:00 [pubmed] PHST- 2012/12/06 06:01 [medline] PHST- 2012/11/04 00:00 [pmc-release] AID - 10.1155/2012/426130 [doi] PST - ppublish SO - Pain Res Treat. 2012;2012:426130. doi: 10.1155/2012/426130. Epub 2012 Nov 4. PMID- 36909840 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230314 IS - 2514-2119 (Electronic) IS - 2514-2119 (Linking) VI - 7 IP - 3 DP - 2023 Mar TI - Chest pain and Raynaud's phenomenon after COVID-19 vaccination in a patient previously diagnosed with systemic lupus erythematosus: a case report. PG - ytad102 LID - 10.1093/ehjcr/ytad102 [doi] LID - ytad102 AB - BACKGROUND: Cardiovascular events, including pericarditis, myocarditis, and myocardial ischaemia, have been reported as complications following COVID-19 vaccination. CASE SUMMARY: A 28-year-old Japanese woman diagnosed 10 years earlier with systemic lupus erythematosus and antiphospholipid syndrome was admitted to our hospital because of chest pain and Raynaud's phenomenon. She had received a second dose of the COVID-19 BNT162b2 mRNA vaccine 28 days earlier. (123)I-β-methyl iodophenyl pentadecanoic acid (BMIPP) and (201)thallium dual myocardial single-photon emission computed tomography demonstrated mildly reduced perfusion of BMIPP in the mid-anterior wall of the left ventricle. Coronary angiography revealed normal coronary arteries; additionally, an endomyocardial biopsy was performed. Histopathological evaluation revealed a normal myocardium without cell infiltration. However, immunostaining for the severe acute respiratory coronavirus (SARS-CoV)/severe acute respiratory coronavirus 2 (SARS-CoV-2) spike protein was positive in the small intramural coronary arteries. The administration of azathioprine (50 mg/day) and amlodipine (5 mg/day) and increases in her prednisolone (10 mg/day) and aspirin doses led to improvements in the symptoms of the patient. DISCUSSION: Our data lead us to speculate that two events in the timeline of the patient, namely, receiving COVID-19 vaccination and the presence of SARS-CoV/SARS-CoV-2 spike protein in small intramural coronary arteries, may be related to the myocardial microangiopathy observed in this patient. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. FAU - Kawano, Hiroaki AU - Kawano H AUID- ORCID: 0000-0002-0452-4384 AD - Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. FAU - Umeda, Masataka AU - Umeda M AD - Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. FAU - Okano, Shinji AU - Okano S AD - Department of Pathology, Nagasaki University Hospital, Nagasaki, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. FAU - Kudo, Takashi AU - Kudo T AUID- ORCID: 0000-0002-8484-9209 AD - Department of Radioisotope Medicine, Atomic Bomb Disease Institute, Nagasaki University, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20230224 PL - England TA - Eur Heart J Case Rep JT - European heart journal. Case reports JID - 101730741 PMC - PMC10004643 OTO - NOTNLM OT - COVID-19 vaccination OT - Case report OT - Pathology OT - SLE OT - Spike protein COIS- Conflict of interest: None declared. EDAT- 2023/03/14 06:00 MHDA- 2023/03/14 06:01 PMCR- 2023/02/24 CRDT- 2023/03/13 04:04 PHST- 2022/05/02 00:00 [received] PHST- 2022/08/02 00:00 [revised] PHST- 2023/02/22 00:00 [accepted] PHST- 2023/03/13 04:04 [entrez] PHST- 2023/03/14 06:00 [pubmed] PHST- 2023/03/14 06:01 [medline] PHST- 2023/02/24 00:00 [pmc-release] AID - ytad102 [pii] AID - 10.1093/ehjcr/ytad102 [doi] PST - epublish SO - Eur Heart J Case Rep. 2023 Feb 24;7(3):ytad102. doi: 10.1093/ehjcr/ytad102. eCollection 2023 Mar. PMID- 28495471 OWN - NLM STAT- MEDLINE DCOM- 20180319 LR - 20250530 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 113 DP - 2017 Sep TI - Quantitative outcome measures for systemic sclerosis-related Microangiopathy - Reliability of image acquisition in Nailfold Capillaroscopy. PG - 56-59 LID - S0026-2862(17)30059-6 [pii] LID - 10.1016/j.mvr.2017.05.003 [doi] AB - BACKGROUND: Nailfold capillaroscopic parameters hold increasing promise as outcome measures for clinical trials in systemic sclerosis (SSc). Their inclusion as outcomes would often naturally require capillaroscopy images to be captured at several time points during any one study. Our objective was to assess repeatability of image acquisition (which has been little studied), as well as of measurement. METHOD: 41 patients (26 with SSc, 15 with primary Raynaud's phenomenon) and 10 healthy controls returned for repeat high-magnification (300×) videocapillaroscopy mosaic imaging of 10 digits one week after initial imaging (as part of a larger study of reliability). Images were assessed in a random order by an expert blinded observer and 4 outcome measures extracted: (1) overall image grade and then (where possible) distal vessel locations were marked, allowing (2) vessel density (across the whole nailfold) to be calculated (3) apex width measurement and (4) giant vessel count. Intra-rater, intra-visit and intra-rater inter-visit (baseline vs. 1week) reliability were examined in 475 and 392 images respectively. A linear, mixed-effects model was used to estimate variance components, from which intra-class correlation coefficients (ICCs) were determined. RESULTS: Intra-visit and inter-visit reliability estimates (ICCs) were (respectively): overall image grade, 0.97 and 0.90; vessel density, 0.92 and 0.65; mean vessel width, 0.91 and 0.79; presence of giant capillary, 0.68 and 0.56. These estimates were conditional on each parameter being measurable. CONCLUSION: Within-operator image analysis and acquisition are reproducible. Quantitative nailfold capillaroscopy, at least with a single observer, provides reliable outcome measures for clinical studies including randomised controlled trials. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Dinsdale, Graham AU - Dinsdale G AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Salford Royal Hospital NHS Foundation Trust, Salford Royal Hospital, Stott Lane, Manchester, UK. Electronic address: graham.dinsdale@manchester.ac.uk. FAU - Moore, Tonia AU - Moore T AD - Salford Royal Hospital NHS Foundation Trust, Salford, UK. FAU - O'Leary, Neil AU - O'Leary N AD - Centre for Biostatistics, Division of Population Health, Health Services Research & Primary Care, University of Manchester, Manchester, UK. FAU - Berks, Michael AU - Berks M AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, University of Manchester, Manchester, UK. FAU - Roberts, Christopher AU - Roberts C AD - Centre for Biostatistics, Division of Population Health, Health Services Research & Primary Care, University of Manchester, Manchester, UK. FAU - Manning, Joanne AU - Manning J AD - Salford Royal Hospital NHS Foundation Trust, Salford, UK. FAU - Allen, John AU - Allen J AD - Microvascular Diagnostics, Northern Medical Physics and Clinical Engineering, Freeman Hospital, Newcastle upon Tyne, UK. FAU - Anderson, Marina AU - Anderson M AD - Institute of Ageing and Chronic Disease, University of Liverpool, Liverpool, UK. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden. FAU - Howell, Kevin AU - Howell K AD - Institute of Immunity and Transplantation, University College London, Royal Free Campus, London, UK. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University, Hospital, Faculty of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Wildt, Marie AU - Wildt M AD - Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden. FAU - Taylor, Christopher AU - Taylor C AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, University of Manchester, Manchester, UK. FAU - Murray, Andrea AU - Murray A AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Salford Royal Hospital NHS Foundation Trust, Salford Royal Hospital, Stott Lane, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Salford Royal Hospital NHS Foundation Trust, Salford Royal Hospital, Stott Lane, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20170508 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Capillaries/*pathology MH - Case-Control Studies MH - Humans MH - Image Interpretation, Computer-Assisted MH - Linear Models MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Observer Variation MH - Predictive Value of Tests MH - Reproducibility of Results MH - Scleroderma, Systemic/*pathology MH - Vascular Diseases/*pathology OTO - NOTNLM OT - Nailfold videocapillaroscopy OT - Raynaud's phenomenon OT - Reliability OT - Systemic sclerosis EDAT- 2017/05/13 06:00 MHDA- 2018/03/20 06:00 CRDT- 2017/05/13 06:00 PHST- 2017/03/13 00:00 [received] PHST- 2017/05/04 00:00 [revised] PHST- 2017/05/06 00:00 [accepted] PHST- 2017/05/13 06:00 [pubmed] PHST- 2018/03/20 06:00 [medline] PHST- 2017/05/13 06:00 [entrez] AID - S0026-2862(17)30059-6 [pii] AID - 10.1016/j.mvr.2017.05.003 [doi] PST - ppublish SO - Microvasc Res. 2017 Sep;113:56-59. doi: 10.1016/j.mvr.2017.05.003. Epub 2017 May 8. PMID- 20375124 OWN - NLM STAT- MEDLINE DCOM- 20101104 LR - 20220330 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 19 IP - 8 DP - 2010 Jul TI - Disease activity and damage accrual during the early disease course in a multinational inception cohort of patients with systemic lupus erythematosus. PG - 949-56 LID - 10.1177/0961203310366572 [doi] AB - An inception cohort of patients with systemic lupus erythematosus from 14 European centres was followed for up to 5 years in order to describe the current early disease course. At inclusion patients (n = 200, 89% female, mean age 35 years, 97% Caucasian, mean SLEDAI 12.2) fulfilled a mean of 6.5 ACR classification criteria. The most prevalent criteria were antinuclear Ab presence (97%) followed by anti-dsDNA Ab (74%), arthritis (69%), leukocytopenia (54%) and malar rash (53%), antiphospholipid Ab (48%) and anti-synovial membrane Ab (21.6%). Clinical signs of lupus nephritis (LN) were present in 39% with biopsy-confirmed LN seen in 25%. Frequent additional findings were hypocomplementaemia (54%), anti-SSA Ab (49%), alopecia (26%) and Raynaud's phenomenon (31%). There were few regional differences in disease presentation and management. One and 5-year survival rates were 99% and 97% respectively. During the mean follow-up of 4.1 years 25% entered a state of early disease quiescence by global physician assessment, but the overall risk of subsequent flare was 60%. Maximum SLEDAI scores decreased over time, but 45% of patients accrued damage (SDI >or=1) for which baseline presence of proteinuria and persistent disease activity were independent predictors. The results indicate minor differences in SLE presentation and treatment within various regions of Europe and a high diagnostic reliance on anti-dsDNA Ab. Despite early reductions in disease activity and improved mortality, the risk for disease flare and damage development is, however, still substantial, especially in patients not entering an early remission. FAU - Nossent, J AU - Nossent J AD - Department of Rheumatology, Institute of Clinical Medicine, University of Tromsø, Tromsø, Norway. hans.nossent@unn.no FAU - Kiss, E AU - Kiss E FAU - Rozman, B AU - Rozman B FAU - Pokorny, G AU - Pokorny G FAU - Vlachoyiannopoulos, P AU - Vlachoyiannopoulos P FAU - Olesinska, M AU - Olesinska M FAU - Marchesoni, A AU - Marchesoni A FAU - Mosca, M AU - Mosca M FAU - Påi, S AU - Påi S FAU - Manger, K AU - Manger K FAU - Schneider, M AU - Schneider M FAU - Nielsen, H AU - Nielsen H FAU - van Vollenhoven, R AU - van Vollenhoven R FAU - Swaak, T AU - Swaak T LA - eng PT - Journal Article DEP - 20100407 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Cohort Studies MH - *Disease Progression MH - Europe MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/diagnosis/*pathology/*physiopathology/therapy MH - Male MH - Middle Aged MH - Mortality MH - Young Adult EDAT- 2010/04/09 06:00 MHDA- 2010/11/05 06:00 CRDT- 2010/04/09 06:00 PHST- 2010/04/09 06:00 [entrez] PHST- 2010/04/09 06:00 [pubmed] PHST- 2010/11/05 06:00 [medline] AID - 0961203310366572 [pii] AID - 10.1177/0961203310366572 [doi] PST - ppublish SO - Lupus. 2010 Jul;19(8):949-56. doi: 10.1177/0961203310366572. Epub 2010 Apr 7. PMID- 41970136 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260413 LR - 20260413 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 3 DP - 2026 Mar TI - When the Body Hardens and the Mind Fragments: Psychosis in Systemic Sclerosis. PG - e104948 LID - 10.7759/cureus.104948 [doi] LID - e104948 AB - Systemic sclerosis is a chronic autoimmune disease primarily characterized by fibrosis and vasculopathy; neurological involvement is considered uncommon. We present the case of a 27-year-old woman with a history of asthma, Raynaud's phenomenon, and chronic facial dermatosis, who developed an acute psychotic syndrome characterized by hallucinations, disorganized speech, and persecutory delusions, with no prior psychiatric history. Physical examination revealed mucocutaneous findings consistent with systemic sclerosis, and neurological evaluation showed altered cognition and behavioral disturbances. Laboratory and cerebrospinal fluid analyses were unremarkable, including negative anti-NMDA receptor antibodies. Transcranial Doppler suggested increased cerebral vascular resistance, raising suspicion of CNS vasculitis. The patient fulfilled the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis despite negative specific autoantibodies. Neuropsychiatric symptoms persisted despite antipsychotic therapy, but improved markedly following high-dose intravenous methylprednisolone and cyclophosphamide. This case illustrates a rare and atypical neuropsychiatric manifestation of systemic sclerosis, highlighting the importance of recognizing autoimmune contributions to acute psychosis. Timely immunosuppressive treatment can lead to favorable outcomes even in the absence of definitive serological markers. Further studies are needed to elucidate the pathophysiology of central nervous system involvement in systemic sclerosis and to guide diagnostic and therapeutic strategies for such presentations. CI - Copyright © 2026, Pacheco Ortega et al. FAU - Pacheco Ortega, Gonzalo Andrés AU - Pacheco Ortega GA AD - Neurology, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX. FAU - Tamayo de León, César David AU - Tamayo de León CD AD - Neurology, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX. FAU - Molina Martínez, Cristian Alejandro AU - Molina Martínez CA AD - Neuroradiology, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX. FAU - Garcia-Santos, Raúl Anwar AU - Garcia-Santos RA AD - Neurology, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX. LA - eng PT - Case Reports PT - Journal Article DEP - 20260309 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13062475 OTO - NOTNLM OT - autoimmune disease OT - autoimmune psychosis OT - connective tissue disease OT - psychosis OT - systemic sclerosis COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/04/13 18:17 MHDA- 2026/04/13 18:18 PMCR- 2026/03/09 CRDT- 2026/04/13 06:31 PHST- 2026/03/09 00:00 [accepted] PHST- 2026/04/13 18:18 [medline] PHST- 2026/04/13 18:17 [pubmed] PHST- 2026/04/13 06:31 [entrez] PHST- 2026/03/09 00:00 [pmc-release] AID - 10.7759/cureus.104948 [doi] PST - epublish SO - Cureus. 2026 Mar 9;18(3):e104948. doi: 10.7759/cureus.104948. eCollection 2026 Mar. PMID- 40538942 OWN - NLM STAT- Publisher LR - 20260417 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 3 DP - 2025 Jun 17 TI - Hand surgical and injectable treatments in systemic sclerosis: A systematic review of published cases. PG - 23971983251348059 LID - 10.1177/23971983251348059 [doi] AB - INTRODUCTION: Systemic sclerosis is a systemic autoimmune disease that can affect the hands leading to a deterioration in function. Unfortunately, current medical treatments are considered insufficient to improve patients' quality of life. However, innovative surgical and injectable treatments are available. PATIENTS AND METHODS: A systematic review of the literature on surgical and injectable treatments available to hand surgeons was carried out according to the PRISMA criteria. All the articles selected were analysed qualitatively and descriptively, and where possible the extracted data were summarised quantitatively. RESULTS AND DISCUSSION: Twenty-nine articles were therefore included, comprising 704 patients and 546 procedures. Nine different treatments were identified. Botulinum toxin injections have shown promising clinical results in the treatment of Raynaud's phenomenon, digital ulcers and pain, but have not been confirmed in randomised placebo-controlled trials. Injections of adipose-derived stromal vascular fraction showed positive results in Raynaud's phenomenon, digital ulcer, pain and function, but not in randomised placebo-controlled trials. Autologous fat grafting has shown significant results in Raynaud Phenomenon (RP) and digital ulcers (DUs) healing and prevention. Peripheral sympathectomy has shown moderate beneficial effects with frequent adverse events, and should be reserved for severe cases. Transluminal angioplasty has shown encouraging results, but studies with a high level of evidence are needed. Finally, symptomatic calcinosis cutis should be treated by resection or carbon dioxide (CO2) laser. A treatment algorithm is provided in the Supplemental Appendix. CONCLUSION: Interventional treatments for scleroderma of the hand have shown an overall trend of efficacy, but some of the beneficial effects have not been found in studies with a high level of evidence. Further well-conducted studies are needed. CI - © The Author(s) 2025. FAU - Perrier, Adrien AU - Perrier A AUID- ORCID: 0009-0004-0049-6226 AD - Department of Plastic and Reconstructive Surgery, Hôpital Rangueil, Centre Hospitalier Universitaire de Toulouse, Toulouse, France. FAU - Pugnet, Gregory AU - Pugnet G AD - Department of Internal Medicine, Hôpital Rangueil, Centre Hospitalier Universitaire de Toulouse, Toulouse, France. FAU - Chaput, Benoit AU - Chaput B AD - Department of Plastic and Reconstructive Surgery, Hôpital Rangueil, Centre Hospitalier Universitaire de Toulouse, Toulouse, France. FAU - Gandolfi, Silvia AU - Gandolfi S AUID- ORCID: 0000-0001-5720-1741 AD - Department of Plastic and Reconstructive Surgery, Hôpital Rangueil, Centre Hospitalier Universitaire de Toulouse, Toulouse, France. LA - eng PT - Journal Article PT - Review DEP - 20250617 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC12174094 OTO - NOTNLM OT - Hand surgery OT - cellular therapy OT - peripheral sympathectomy OT - systematic review OT - systemic sclerosis COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2025/06/20 11:31 MHDA- 2025/06/20 11:31 PMCR- 2026/06/17 CRDT- 2025/06/20 04:32 PHST- 2025/04/14 00:00 [received] PHST- 2025/05/20 00:00 [accepted] PHST- 2026/06/17 00:00 [pmc-release] PHST- 2025/06/20 11:31 [medline] PHST- 2025/06/20 11:31 [pubmed] PHST- 2025/06/20 04:32 [entrez] AID - 10.1177_23971983251348059 [pii] AID - 10.1177/23971983251348059 [doi] PST - aheadofprint SO - J Scleroderma Relat Disord. 2025 Jun 17;10(3):23971983251348059. doi: 10.1177/23971983251348059. PMID- 41000386 OWN - NLM STAT- MEDLINE DCOM- 20250926 LR - 20250929 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 16 DP - 2025 TI - Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and mixed connective tissue disease: a case report. PG - 1644259 LID - 10.3389/fimmu.2025.1644259 [doi] LID - 1644259 AB - Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory demyelinating disease affecting the optic nerve and spinal cord. NMOSD frequently coexists with other autoimmune diseases. However, its concurrence with mixed connective tissue disease (MCTD) is rather rare and often overlooked. This study reports the first case in China of aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD preceding MCTD with long-term follow-up. Between 2016 and 2024, the patient successively developed left lower limb numbness, hiccups, vomiting, facial numbness, Raynaud's phenomenon, finger swelling, digital sclerosis, and synovitis. Acute-phase management involved pulse steroid therapy, while remission maintenance utilized azathioprine, mycophenolate mofetil, rituximab, and inebilizumab for relapse prevention. This paper presents this case and reviews other cases of NMOSD combined with MCTD, aiming to contribute to the clinical understanding and management of this rare condition. CI - Copyright © 2025 Wang, Wu, Zhang, Zhang, Jia and Geng. FAU - Wang, Ziyu AU - Wang Z AD - The Fifth Clinical College of Guangzhou University of Chinese Medicine, Guangzhou, China. FAU - Wu, Ling AU - Wu L AD - Department of Rheumatology, Shenzhen Hospital, The University of Hong Kong, Shenzhen, China. FAU - Zhang, Zhihao AU - Zhang Z AD - The Fifth Clinical College of Guangzhou University of Chinese Medicine, Guangzhou, China. FAU - Zhang, Chenyang AU - Zhang C AD - The Fifth Clinical College of Guangzhou University of Chinese Medicine, Guangzhou, China. FAU - Jia, Ertao AU - Jia E AD - The Fifth Clinical College of Guangzhou University of Chinese Medicine, Guangzhou, China. AD - Department of Rheumatism, Guangdong Provincial Second Hospital of Traditional Chinese Medicine, Guangzhou, China. FAU - Geng, Hongling AU - Geng H AD - Department of Gynecology, Guangdong Provincial Hospital of Chinese Medicine, Guangzhou, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20250910 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (AQP4 protein, human) RN - 0 (Aquaporin 4) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulin G) SB - IM MH - Humans MH - *Aquaporin 4/immunology MH - *Autoantibodies/immunology/blood MH - Immunoglobulin G/immunology/blood MH - *Mixed Connective Tissue Disease/immunology/diagnosis/drug therapy/complications MH - *Neuromyelitis Optica/immunology/drug therapy/diagnosis/complications PMC - PMC12457320 OTO - NOTNLM OT - AQP4 OT - case report OT - mixed connective tissue disease OT - neuromyelitis optica spectrum disorder OT - overlap syndrome COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2025/09/26 06:30 MHDA- 2025/09/26 06:31 PMCR- 2025/09/10 CRDT- 2025/09/26 05:29 PHST- 2025/06/10 00:00 [received] PHST- 2025/08/20 00:00 [accepted] PHST- 2025/09/26 06:31 [medline] PHST- 2025/09/26 06:30 [pubmed] PHST- 2025/09/26 05:29 [entrez] PHST- 2025/09/10 00:00 [pmc-release] AID - 10.3389/fimmu.2025.1644259 [doi] PST - epublish SO - Front Immunol. 2025 Sep 10;16:1644259. doi: 10.3389/fimmu.2025.1644259. eCollection 2025. PMID- 35382016 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 3 IP - 3 DP - 2018 Oct TI - Interstitial lung disease is associated with an increased risk of lung cancer in systemic sclerosis: Longitudinal data from the Canadian Scleroderma Research Group. PG - 221-227 LID - 10.1177/2397198318766825 [doi] AB - OBJECTIVE: The literature supports an increased risk of malignancy in systemic sclerosis, including lung cancer. Our objective was to identify potential independent predictors of lung cancer risk in systemic sclerosis. METHODS: We used a cohort of 1560 systemic sclerosis patients from the Canadian Scleroderma Research Group, enrolled from 2004 and followed for a maximum of 11 years. Time to lung cancer was calculated from the onset of the first non-Raynaud's symptoms. Baseline demographic, clinical, and serological characteristics of patients with and without lung cancer were compared. Cox proportional hazards models were used to estimate the effects of demographic variables, exposure to smoking, disease duration, disease subset (diffuse vs limited), immunosuppressant drug exposure, and presence of interstitial lung disease on the risk of lung cancer. RESULTS: Over the 5519 total person-years of follow-up, 18 SSc patients were diagnosed with lung cancer after cohort entry (3.2 cancers per 1000 person-years). In univariate comparisons, cancer cases were more likely to be male, to have a smoking history, and to have interstitial lung disease than non-cases. In multivariate analysis, interstitial lung disease was independently associated with the risk of lung cancer (hazard ratio: 2.95, 95% confidence interval: 1.10-7.87). CONCLUSION: In addition to known demographic (male sex) and lifestyle risk factors (smoking), interstitial lung disease is an independent risk factor for lung cancer in systemic sclerosis. These results have implications for lung cancer screening in systemic sclerosis. CI - © The Author(s) 2018. FAU - Sakr, Lama AU - Sakr L AD - Division of Respirology, Jewish General Hospital, Montréal, QC, Canada. AD - Department of Medicine, McGill University, Montréal, QC, Canada. FAU - Hudson, Marie AU - Hudson M AD - Department of Medicine, McGill University, Montréal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montréal, QC, Canada. AD - Lady Davis Institute, Jewish General Hospital, Montréal, QC, Canada. FAU - Wang, Mianbo AU - Wang M AD - Lady Davis Institute, Jewish General Hospital, Montréal, QC, Canada. FAU - Younanian, Elie AU - Younanian E AD - McGill University, Montréal, QC, Canada. FAU - Baron, Murray AU - Baron M AD - Department of Medicine, McGill University, Montréal, QC, Canada. AD - Division of Rheumatology, Jewish General Hospital, Montréal, QC, Canada. FAU - Bernatsky, Sasha AU - Bernatsky S AD - Department of Medicine, McGill University, Montréal, QC, Canada. AD - Division of Rheumatology, McGill University Health Center, Montréal, QC, Canada. AD - Division of Clinical Epidemiology, Research Institute of the McGill University Health Centre, Montréal, QC, Canada. CN - Canadian Scleroderma Research Group LA - eng PT - Journal Article DEP - 20180410 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922601 OTO - NOTNLM OT - Systemic sclerosis OT - interstitial lung disease OT - lung cancer COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2018/10/01 00:00 MHDA- 2018/10/01 00:01 PMCR- 2019/10/01 CRDT- 2022/04/06 05:09 PHST- 2017/12/15 00:00 [received] PHST- 2018/03/01 00:00 [accepted] PHST- 2022/04/06 05:09 [entrez] PHST- 2018/10/01 00:00 [pubmed] PHST- 2018/10/01 00:01 [medline] PHST- 2019/10/01 00:00 [pmc-release] AID - 10.1177_2397198318766825 [pii] AID - 10.1177/2397198318766825 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2018 Oct;3(3):221-227. doi: 10.1177/2397198318766825. Epub 2018 Apr 10. PMID- 37882716 OWN - NLM STAT- MEDLINE DCOM- 20231027 LR - 20231027 IS - 1007-8738 (Print) IS - 1007-8738 (Linking) VI - 39 IP - 10 DP - 2023 Oct TI - [Analysis of clinical characteristics and risk factors in patients with neuropsychiatric systemic lupus erythematosus (NPSLE)]. PG - 924-927 AB - Objective To analyze clinical characteristics of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) and to explore the risk factors affecting the occurrence of NPSLE. Methods A total of 63 NPSLE patients and 61 non-NPSLE patients were enrolled. The clinical manifestations and laboratory examination data of the two groups were collected, and the disease characteristics of NPSLE were summarized to analyze the risk factors affecting the occurrence of NPSLE by multivariate Logistic regression. Results The most common clinical manifestations of NPSLE patients were headache (39.7%), affective disorder (33.3%) and cognitive impairment (30.2%), with cranial magnetic resonance abnormalities (63.5%) and a high cerebrospinal fluid protein positive rate (52.4%). Compared with non-NPSLE patients, there were significantly increased levels of Raynaud's phenomenon, renal involvement, anti-RNP antibody, anti-ribosomal P protein, hypocomplementemia, lymphocyte-to-monocyte ratio (LMR) and neutrophil-to-lymphocyte ratio (NLR) in NPSLE patients. Multivariate Logistic regression analysis showed that renal involvement, Raynaud's phenomenon, positive anti-ribosomal P protein antibody, and elevated LMR and NLR were independent risk factors for NPSLE. Conclusion Headache is the most common symptom in patients with NPSLE, and abnormal cranial MRI and cerebrospinal fluid examination are more common. SLE patients who present with renal involvement, Raynaud's phenomenon, positive anti-ribosomal P protein antibodies, and elevated levels of LMR and NLR are more susceptible to developing NPSLE. FAU - Liu, Jie AU - Liu J AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China. FAU - Jia, Shuyuan AU - Jia S AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China. FAU - Wang, Pengyu AU - Wang P AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China. FAU - Lyu, Tingting AU - Lyu T AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China. FAU - Hu, Yinxiu AU - Hu Y AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China. FAU - Zhang, Yan AU - Zhang Y AD - Department of Rheumatology, Second Affiliated Hospital of Air Force Medical University, Xi'an 710038, China.*Corresponding author, E-mail: zyhphc@fmmu.edu.cn. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Xi Bao Yu Fen Zi Mian Yi Xue Za Zhi JT - Xi bao yu fen zi mian yi xue za zhi = Chinese journal of cellular and molecular immunology JID - 101139110 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - *Lupus Vasculitis, Central Nervous System MH - Risk Factors MH - Headache MH - Antibodies, Antinuclear MH - *Cognitive Dysfunction EDAT- 2023/10/26 12:42 MHDA- 2023/10/27 06:43 CRDT- 2023/10/26 10:13 PHST- 2023/10/27 06:43 [medline] PHST- 2023/10/26 12:42 [pubmed] PHST- 2023/10/26 10:13 [entrez] PST - ppublish SO - Xi Bao Yu Fen Zi Mian Yi Xue Za Zhi. 2023 Oct;39(10):924-927. PMID- 32190139 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220413 IS - 1734-1922 (Print) IS - 1896-9151 (Electronic) IS - 1734-1922 (Linking) VI - 16 IP - 2 DP - 2020 TI - Associations between nailfold capillaroscopy findings and interstitial lung disease in patients with mixed connective tissue disease. PG - 297-301 LID - 10.5114/aoms.2018.81129 [doi] AB - INTRODUCTION: Mixed connective tissue disease (MCTD) is a chronic immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features including progressive lung fibrosis. The aim of this study was to evaluate potential associations between lung fibrosis in MCTD and specific clinical and laboratory findings including results of nailfold capillaroscopy (NFC) examination. MATERIAL AND METHODS: Patients with MCTD who were admitted to the Departments of Allergy and Immunology or Dermatology at the University Hospital of Krakow (Poland) in 2015-2018 were identified based on comprehensive individual record review. Diagnosis of MCTD required fulfilment of at least one of the four widely accepted sets of diagnostic criteria. Clinical and laboratory data were collected, and statistical analysis was performed to identify potential predictors of interstitial lung disease (ILD). RESULTS: Thirty patients (90% females) aged 22-81 years met the study inclusion criteria. The mean duration of symptoms associated with MCTD was 7.3 months. Photosensitivity and Raynaud's phenomenon were the most common clinical manifestations (90% and 70%, respectively). Hand oedema, sclerodactyly and the presence of giant capillaries in NFC correlated significantly with the risk of lung involvement. In multivariate analysis, the presence of enlarged loops in NFC (giant capillaries) was identified as an independent factor for ILD (R (2) = 0.82, p < 0.0000001). CONCLUSIONS: The NFC examination should be considered in all patients with a diagnosis of MCTD. The presence of giant capillaries may be a promising marker for interstitial lung disease in these patients, especially among those with a short duration of disease (i.e. < 1 year). CI - Copyright: © 2019 Termedia & Banach. FAU - Celińska-Löwenhoff, Magdalena AU - Celińska-Löwenhoff M AD - 2 Department of Medicine, Jagiellonian University Medical College, Krakow, Poland. FAU - Pastuszczak, Maciej AU - Pastuszczak M AD - Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland. FAU - Pełka, Karolina AU - Pełka K AD - Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland. FAU - Stec-Polak, Magdalena AU - Stec-Polak M AD - Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland. FAU - Wojas-Pelc, Anna AU - Wojas-Pelc A AD - Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland. FAU - Musiał, Jacek AU - Musiał J AD - 2 Department of Medicine, Jagiellonian University Medical College, Krakow, Poland. LA - eng PT - Journal Article DEP - 20190111 PL - Poland TA - Arch Med Sci JT - Archives of medical science : AMS JID - 101258257 PMC - PMC7069420 OTO - NOTNLM OT - capillaroscopy OT - interstitial lung disease OT - mixed connective tissue disease COIS- The authors declare no conflict of interest. EDAT- 2019/01/11 00:00 MHDA- 2019/01/11 00:01 PMCR- 2019/01/11 CRDT- 2020/03/20 06:00 PHST- 2018/09/27 00:00 [received] PHST- 2018/10/25 00:00 [accepted] PHST- 2020/03/20 06:00 [entrez] PHST- 2019/01/11 00:00 [pubmed] PHST- 2019/01/11 00:01 [medline] PHST- 2019/01/11 00:00 [pmc-release] AID - 34608 [pii] AID - 10.5114/aoms.2018.81129 [doi] PST - epublish SO - Arch Med Sci. 2019 Jan 11;16(2):297-301. doi: 10.5114/aoms.2018.81129. eCollection 2020. PMID- 17488144 OWN - NLM STAT- MEDLINE DCOM- 20070628 LR - 20181113 IS - 0012-6667 (Print) IS - 0012-6667 (Linking) VI - 67 IP - 7 DP - 2007 TI - Scleroderma lung: pathogenesis, evaluation and current therapy. PG - 985-96 AB - The lungs are frequently involved in systemic sclerosis ('scleroderma'), a rare, disabling disease of unknown origin, characterised by skin thickening and Raynaud's phenomenon. The pathogenesis of scleroderma is complex, but signs and symptoms of excessive fibrosis, vasculopathy and inflammation are almost universally present. Dyspnoea in scleroderma patients can be due to chest wall tightening from skin thickening, pleural disease, cardiac involvement, myositis of intercostal muscles, or so-called scleroderma lung disease. Scleroderma lung disease encompasses vascular (pulmonary artery hypertension) or interstitial lung disease, or both. A comprehensive work-up is required to delineate the underlying cause of dyspnoea in a scleroderma patient, and to establish the contribution of each component to the symptoms. This should include a 6-minute walk test, pulmonary function testing, high-resolution thoracic CT scanning, ECG, echocardiography and, if pulmonary artery hypertension is suspected, right-heart catheterisation; bronchoalveolar lavage is optional. Lung disease in scleroderma contributes significantly to excess morbidity and early mortality, especially when diffusion capacity drops below 40% and/or forced vital capacity below 50%. However, recent clinical studies have unequivocally demonstrated that scleroderma lung disease is amenable to treatment with new vasodilatory drugs that target specific pathways involved in vasoconstriction, or with cyclophosphamide for interstitial lung disease. Uncontrolled studies have suggested that these therapies also have an impact on survival, but controlled studies with a long follow-up are needed to corroborate this point. FAU - van Laar, Jacob M AU - van Laar JM AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. j.m.van_laar@lumc.nl FAU - Stolk, Jan AU - Stolk J FAU - Tyndall, Alan AU - Tyndall A LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Drugs JT - Drugs JID - 7600076 RN - 0 (Endothelin A Receptor Antagonists) RN - 0 (Immunosuppressive Agents) RN - 0 (Prostaglandins, Synthetic) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Cyclophosphamide/administration & dosage/therapeutic use MH - Endothelin A Receptor Antagonists MH - Humans MH - *Hypertension, Pulmonary/drug therapy/etiology MH - Immunosuppressive Agents/therapeutic use MH - *Lung Diseases, Interstitial/drug therapy/etiology MH - Prostaglandins, Synthetic/therapeutic use MH - *Scleroderma, Systemic/drug therapy/etiology MH - Terminology as Topic RF - 103 EDAT- 2007/05/10 09:00 MHDA- 2007/06/29 09:00 CRDT- 2007/05/10 09:00 PHST- 2007/05/10 09:00 [pubmed] PHST- 2007/06/29 09:00 [medline] PHST- 2007/05/10 09:00 [entrez] AID - 6774 [pii] AID - 10.2165/00003495-200767070-00004 [doi] PST - ppublish SO - Drugs. 2007;67(7):985-96. doi: 10.2165/00003495-200767070-00004. PMID- 17786140 OWN - NLM STAT- MEDLINE DCOM- 20071108 LR - 20130516 IS - 1665-2681 (Print) IS - 1665-2681 (Linking) VI - 6 IP - 3 DP - 2007 Jul-Sep TI - Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome. PG - 150-5 AB - BACKGROUND/AIMS: Patients with primary Sjögren's syndrome may present liver involvement. Our goals were to establish the prevalence of abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome and correlate their presence with other clinical and laboratory features. METHODS: Ninety-five patients with diagnosis of primary Sjögren's syndrome were studied. Data on gender, age, clinical features, liver biochemistries, tests of inflammation and autoimmunity, and concomitant diseases were collected. RESULTS: Forty-two patients (44%) had abnormal hepatic biochemistries, and of these 19 patients (20%) had clinical liver disease. Patients with abnormal hepatic biochemistries had higher frequency of autoimmune hypotiroidism, arthritis, vasculitis, Raynaud's phenomenon, higher sedimentation rate,and higher frequency of antinuclear and antimitochondrial antibodies than patients with normal liver biochemistries (P < 0.05 for each). Patients with clinical liver disease had higher frequency of arthritis, vasculitis, and higher frequency of antimitochondrial antibodies than patients without clinical liver disease (P < 0.05 for each). Twenty-one patients had diagnosis of a specific liver disease, such as hepatitis C virus infection (n = 11), autoimmune hepatitis (n = 2), primary biliary cirrhosis (n =5),nonalcoholic fatty liver disease (n = 2), and hepatitis B virus infection (n = 1). In half of patients with liver involvement a definitive cause could not be identified. CONCLUSION: Liver involvement is frequently found in patients with primary Sjögren's syndrome, and its presence is associated with clinical features of systemic disease, and markers of autoimmunity and inflammation. There may be a subgroup of patients with liver involvement secondary to primary Sjögren's syndrome. FAU - Montaño-Loza, Aldo J AU - Montaño-Loza AJ AD - Department of Gastroenterology and Hepatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. montanoa@quetzal.innsz.mx FAU - Crispín-Acuña, José Carlos AU - Crispín-Acuña JC FAU - Remes-Troche, José María AU - Remes-Troche JM FAU - Uribe, Misael AU - Uribe M LA - eng PT - Journal Article PL - Mexico TA - Ann Hepatol JT - Annals of hepatology JID - 101155885 RN - EC 2.6.1.1 (Aspartate Aminotransferases) RN - EC 2.6.1.2 (Alanine Transaminase) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Alanine Transaminase/metabolism MH - Aspartate Aminotransferases/metabolism MH - Autoimmunity/physiology MH - Female MH - Humans MH - Inflammation/physiopathology MH - Liver/*metabolism MH - Liver Diseases/*etiology/metabolism MH - Male MH - Middle Aged MH - Odds Ratio MH - Retrospective Studies MH - Sjogren's Syndrome/*complications/metabolism EDAT- 2007/09/06 09:00 MHDA- 2007/11/09 09:00 CRDT- 2007/09/06 09:00 PHST- 2007/09/06 09:00 [pubmed] PHST- 2007/11/09 09:00 [medline] PHST- 2007/09/06 09:00 [entrez] AID - 497087 [pii] PST - ppublish SO - Ann Hepatol. 2007 Jul-Sep;6(3):150-5. PMID- 32322117 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240728 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 58 IP - 1 DP - 2020 TI - The clinical phenotype associated with antisynthetase autoantibodies. PG - 4-8 LID - 10.5114/reum.2020.93505 [doi] AB - OBJECTIVES: Specific systemic autoimmune syndrome characterized by inflammatory myopathy, arthritis or arthralgias, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands is called antisynthetase syndrome (AS). The aim of this study was to assess the clinical spectrum associated with presence of aminoacyl-transfer RNA synthetase autoantibodies (ASA). MATERIAL AND METHODS: A total of 305 patients with presence of myositis-specific autoantibodies were identified in the database of immunological tests performed in the Clinical Immunology and Transplantology Unit, Medical University of Gdansk between January 2011 and March 2016. In 110 patients (36%) ASA were detected. The detailed analysis included 50 patients with ASA for whom full clinical data were available. RESULTS: The incidence of specific ASA in the analyzed group was: Jo-1 46% (23 patients), PL-12 32% (16 patients), PL-7 16% (8 patients), OJ 12% (6 patients), EJ 6% (3 patients). In 10% (5 patients) there was coexistence of at least one ASA, and in another 5 patients there was coexistence of ASA with other antibodies specific for myositis (MSA). In the analyzed group of patients 11 (22%) satisfied the Bohan and Peter criteria for dermatomyositis, 1 for polymyositis. In 5 patients (10%) based on clinical presentation and ASA presence the AS was recognized. Another 3 patients met the criteria of the overlap syndrome polymyositis respectively with systemic lupus, rheumatoid arthritis, and scleroderma. In 5 patients undifferentiated connective tissue disease was diagnosed, and 14 consecutive patients were diagnosed with other connective tissue diseases, while 12 patients did not receive a definitive diagnosis. CONCLUSIONS: The clinical presentation of patients with the presence of ASA is varied. Their presence indicates not only idiopathic inflammatory myopathies, but also non-specifically other disease entities. These patients require observation for the development of idiopathic inflammatory myopathy, and ILD. CI - Copyright: © 2020 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. FAU - Masiak, Anna AU - Masiak A AD - Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, Poland. FAU - Marzec, Monika AU - Marzec M AD - Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, Poland. FAU - Kulczycka, Julia AU - Kulczycka J AD - Department of Clinical Immunology and Transplantation, Medical University of Gdansk, Gdansk, Poland. FAU - Zdrojewski, Zbigniew AU - Zdrojewski Z AD - Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, Poland. LA - eng PT - Journal Article DEP - 20200228 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC7174798 OTO - NOTNLM OT - antisynthetase antibodies OT - antisynthetase syndrome OT - idiopathic inflammatory myopathy COIS- The authors declare no conflict of interest. EDAT- 2020/04/24 06:00 MHDA- 2020/04/24 06:01 PMCR- 2020/01/01 CRDT- 2020/04/24 06:00 PHST- 2020/01/15 00:00 [received] PHST- 2020/02/10 00:00 [accepted] PHST- 2020/04/24 06:00 [entrez] PHST- 2020/04/24 06:00 [pubmed] PHST- 2020/04/24 06:01 [medline] PHST- 2020/01/01 00:00 [pmc-release] AID - 93505 [pii] AID - 10.5114/reum.2020.93505 [doi] PST - ppublish SO - Reumatologia. 2020;58(1):4-8. doi: 10.5114/reum.2020.93505. Epub 2020 Feb 28. PMID- 25740209 OWN - NLM STAT- MEDLINE DCOM- 20151215 LR - 20161125 IS - 1347-4820 (Electronic) IS - 1346-9843 (Linking) VI - 79 IP - 4 DP - 2015 TI - Coronary microvascular dysfunction in asymptomatic patients affected by systemic sclerosis - limited vs. diffuse form. PG - 825-9 LID - 10.1253/circj.CJ-14-1114 [doi] AB - BACKGROUND: This observational study was designed to evaluate the prevalence of coronary microvascular dysfunction (CMD) in asymptomatic patients affected by systemic sclerosis (SSc), stratifying the results according to the limited (lcSSc) and the diffuse (dcSSc) forms of the disease. METHODS AND RESULTS: We enrolled 19 consecutive asymptomatic patients with dcSSc (n=7) or lcSSc (n=12). In all subjects, coronary flow reserve (CFR) was assessed by measuring diastolic coronary flow velocities in the left anterior descending artery by pulsed wave Doppler at baseline and after dipyridamole infusion (0.84 mg·kg(-1)·6 min(-1)). Wall motion score index was evaluated at baseline and during stress. We enrolled 20 healthy subjects as controls. Mean CFR was 1.96±0.62 in patients and 2.69±0.47 in controls (P<0.001). Abnormal values of CFR (≤2) were significantly more prevalent in patients than in controls (10/19 vs. 0/20; P<0.001) and in the dcSSc subgroup than in the lcSSc subgroup (6/7 vs. 4/12; P=0.05). An inverse relationship between disease duration (from time of onset of Raynaud's phenomenon) and CFR value was observed in the lcSSc group (correlation coefficient -0.583; P=0.046). Neither patients nor controls had wall motion abnormalities during dipyridamole administration. CONCLUSIONS: A blunted CFR, most likely because of CMD, is more frequent in patients affected by the dcSSc form in the early stages of the disease, whereas it seems to appear later in lcSSc. FAU - Faccini, Alessia AU - Faccini A AD - Vita-Salute San Raffaele University and San Raffaele Scientific Institute. FAU - Agricola, Eustachio AU - Agricola E FAU - Oppizzi, Michele AU - Oppizzi M FAU - Margonato, Alberto AU - Margonato A FAU - Galderisi, Maurizio AU - Galderisi M FAU - Sabbadini, Maria Grazia AU - Sabbadini MG FAU - Franchini, Stefano AU - Franchini S FAU - Camici, Paolo G AU - Camici PG LA - eng PT - Comparative Study PT - Journal Article DEP - 20150206 PL - Japan TA - Circ J JT - Circulation journal : official journal of the Japanese Circulation Society JID - 101137683 SB - IM MH - Adult MH - Aged MH - *Coronary Circulation MH - Coronary Vessels/diagnostic imaging/physiopathology MH - Echocardiography MH - Female MH - Humans MH - Male MH - *Microcirculation MH - Middle Aged MH - *Multiple Sclerosis/diagnostic imaging/physiopathology EDAT- 2015/03/06 06:00 MHDA- 2015/12/17 06:00 CRDT- 2015/03/06 06:00 PHST- 2015/03/06 06:00 [entrez] PHST- 2015/03/06 06:00 [pubmed] PHST- 2015/12/17 06:00 [medline] AID - 10.1253/circj.CJ-14-1114 [doi] PST - ppublish SO - Circ J. 2015;79(4):825-9. doi: 10.1253/circj.CJ-14-1114. Epub 2015 Feb 6. PMID- 23216317 OWN - NLM STAT- MEDLINE DCOM- 20131219 LR - 20170721 IS - 1526-4610 (Electronic) IS - 0017-8748 (Linking) VI - 53 IP - 1 DP - 2013 Jan TI - QT prolongation, Torsade de Pointes, myocardial ischemia from coronary vasospasm, and headache medications. Part 1: review of serotonergic cardiac adverse events with a triptan case. PG - 208-216 LID - 10.1111/j.1526-4610.2012.02300.x [doi] AB - Serotonin (5-hydroxytryptamine)(1B/1D) agonists are vasoconstrictors that can affect coronary and cerebral arteries. Retrosternal chest, arm, and jaw pain following triptan use is generally attributed to "triptan sensations" and dismissed as noncardiac. However, triptans narrow normal coronary arteries and occasionally trigger vasospasm. They are contraindicated in atherosclerotic vascular disease. Part 1 of this review examines the relationship of medications used in migraine with the likelihood of causing vasospasm or vasoconstriction, and the triggering of cardiac arrhythmias. We report an illustrative case of polymorphic ventricular tachyarrhythmia, electrocardiogram changes consistent with cardiac ischemia, and acquired corrected QT interval lengthening following oral sumatriptan in a 53-year-old migraineur without risk factors for coronary artery disease (CAD). Extensive evaluation revealed insignificant single coronary vessel atherosclerosis and coronary artery vasospasm on ergonovine challenge. The report highlights the hidden risk that may underlie a "triptan sensation" and the possible association of the vasospastic features of Raynaud's phenomenon, migraine headaches, and coronary vasospasm. Part 1 discusses the risks for Torsade de Pointes, vasospasm, and ischemia, with a review and discussion of case reports of triptan-associated cardiovascular events in migraineurs with and without CAD risk factors or documented CAD; of the epidemiology and studies of triptans, vasospasm, and cardiovascular morbidity; and of the relationship of variant angina, migraine, and vasospastic disease. In the second part of this review, headache medications and their propensity for corrected QT prolongation will be summarized. CI - © 2012 American Headache Society. FAU - Stillman, Mark J AU - Stillman MJ AD - Headache Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA. FAU - Tepper, Stewart AU - Tepper S AD - Headache Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA. FAU - Tepper, Deborah E AU - Tepper DE AD - Headache Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA. FAU - Cho, Leslie AU - Cho L AD - Department of Cardiology, Cleveland Clinic, Cleveland, OH, USA. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20121206 PL - United States TA - Headache JT - Headache JID - 2985091R RN - 0 (Serotonin Receptor Agonists) RN - 8R78F6L9VO (Sumatriptan) SB - IM EIN - Headache. 2013 Mar;53(3):579-80 MH - Coronary Vasospasm/*chemically induced MH - Electrocardiography MH - Female MH - Humans MH - Middle Aged MH - Migraine Disorders/*drug therapy MH - Myocardial Ischemia/*chemically induced MH - Serotonin Receptor Agonists/*adverse effects MH - Sumatriptan/*adverse effects MH - Torsades de Pointes/*chemically induced EDAT- 2012/12/12 06:00 MHDA- 2013/12/20 06:00 CRDT- 2012/12/11 06:00 PHST- 2012/10/01 00:00 [accepted] PHST- 2012/12/11 06:00 [entrez] PHST- 2012/12/12 06:00 [pubmed] PHST- 2013/12/20 06:00 [medline] AID - 10.1111/j.1526-4610.2012.02300.x [doi] PST - ppublish SO - Headache. 2013 Jan;53(1):208-216. doi: 10.1111/j.1526-4610.2012.02300.x. Epub 2012 Dec 6. PMID- 17992591 OWN - NLM STAT- MEDLINE DCOM- 20080208 LR - 20211020 IS - 1080-0549 (Print) IS - 1080-0549 (Linking) VI - 32 IP - 3 DP - 2007 Jun TI - Sjögren's syndrome--study of autoantigens and autoantibodies. PG - 238-51 AB - The presence of autoantibodies is the hallmark of systemic autoimmune diseases. During the past 30 years, intense clinical and basic research have dissected the clinical value of autoantibodies in many autoimmune diseases and offered new insights into a better understanding of the molecular and functional properties of the targeted autoantigens. Unraveling the immunologic mechanisms underlying the autoimmune tissue injury, provided useful conclusions on the generation of autoantibodies and the perpetuation of the autoimmune response. Primary Sjögren's syndrome (pSS) is characterized by the presence of autoantibodies binding on a vast array of organ and non-organ specific autoantigens. The most common autoantibodies are those targeting the Ro/La RNP complex, and they serve as disease markers, as they are included in the European-American Diagnostic Criteria for pSS. Other autoantibodies are associated with particular disease manifestations, such as anti-centromere antibodies with Raynaud's phenomenon, anti-carbonic anhydrase II with distal renal tubular acidosis, anti-mitochondrial antibodies with liver pathology, and cryoglobulins with the evolution to non-Hodgkin's lymphoma. Finally, autoantibodies against autoantigens such as alpha- and beta-fodrin, islet cell autoantigen, poly(ADP)ribose polymerase (PARP), NuMA, Golgins, and NOR-90 are found in a subpopulation of SS patients without disease specificity, and their utility remains to be elucidated. In this review, the molecular and clinical characteristics (divided according to their clinical utility) of the autoantigens and autoantibodies associated with pSS are discussed. FAU - Routsias, John G AU - Routsias JG AD - Department of Pathophysiology, Medical School, National University of Athens, M.Asias 75, 11527, Athens, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/blood/*immunology MH - Autoantigens/blood/chemistry/*immunology MH - Base Sequence MH - Biomarkers/blood MH - Humans MH - Molecular Sequence Data MH - Protein Conformation MH - Sjogren's Syndrome/*diagnosis/*immunology RF - 164 EDAT- 2007/11/10 09:00 MHDA- 2008/02/09 09:00 CRDT- 2007/11/10 09:00 PHST- 2007/11/10 09:00 [pubmed] PHST- 2008/02/09 09:00 [medline] PHST- 2007/11/10 09:00 [entrez] AID - 10.1007/s12016-007-8003-8 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2007 Jun;32(3):238-51. doi: 10.1007/s12016-007-8003-8. PMID- 16980724 OWN - NLM STAT- MEDLINE DCOM- 20071113 LR - 20060918 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 45 Suppl 4 DP - 2006 Oct TI - Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement? PG - iv43-6 AB - Raynaud's phenomenon (RP) represents the most frequent clinical aspect of cardio/microvascular involvement and is a key feature of several autoimmune rheumatic diseases. Moreover, RP is associated in a statistically significant manner with many coronary diseases. In normal conditions or in primary RP (excluding during the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops along with the nailbed. On the contrary, in subjects suffering from secondary RP, one or more alterations of the capillaroscopic findings should alert the physician of the possibility of a connective tissue disease not yet detected. Nailfold capillaroscopy (NV) represents the best method to analyse microvascular abnormalities in autoimmune rheumatic diseases. Architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, angiogenesis and avascular areas characterize >95% of patients with overt scleroderma (SSc). The term 'SSc pattern' includes, all together, these sequential capillaroscopic changes typical to the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in the undifferentiated connective tissue disease are generally reported as 'SSc-like pattern'. Effectively, and early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy, or better with nailfold video capillaroscopy (NVC). The early differential diagnosis between primary and secondary RP is the best advantage NVC may offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, anti-phospholipid syndrome and Sjogren's syndrome. Further epidemiological and clinical studies are needed to better standardize the NCV patterns. In future, the evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases might represent a tool for the prediction of microvascular heart involvement by considering the systemic microvascular derangement at the capillary nailfold. FAU - Cutolo, M AU - Cutolo M AD - Research Laboratory and Division of Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. mcutolo@unige.it FAU - Sulli, A AU - Sulli A FAU - Secchi, M E AU - Secchi ME FAU - Paolino, S AU - Paolino S FAU - Pizzorni, C AU - Pizzorni C LA - eng PT - Journal Article PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Autoimmune Diseases/complications/*diagnosis/physiopathology MH - Capillaries/pathology MH - Heart Diseases/*diagnosis/etiology/physiopathology MH - Humans MH - *Microscopic Angioscopy MH - Nails/*blood supply MH - Rheumatic Diseases/complications/*diagnosis/physiopathology RF - 35 EDAT- 2006/09/19 09:00 MHDA- 2007/11/14 09:00 CRDT- 2006/09/19 09:00 PHST- 2006/09/19 09:00 [pubmed] PHST- 2007/11/14 09:00 [medline] PHST- 2006/09/19 09:00 [entrez] AID - 45/suppl_4/iv43 [pii] AID - 10.1093/rheumatology/kel310 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Oct;45 Suppl 4:iv43-6. doi: 10.1093/rheumatology/kel310. PMID- 37736350 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230923 IS - 2160-200X (Print) IS - 2160-200X (Electronic) IS - 2160-200X (Linking) VI - 13 IP - 4 DP - 2023 TI - Concomitant symptomatic cardiac sarcoidosis and systemic sclerosis with cardiac involvement: a case report. PG - 283-290 AB - Sarcoidosis and systemic sclerosis are two inflammatory multisystemic disorders of unknown etiology that may be life-threatening especially when there is cardiac involvement. Both diseases may coexist, however, there are very few case reports of patients with both cardiac sarcoidosis and systemic sclerosis in the literature. We report the case of a 72-year-old female who was initially referred for dyspnea. A chest computed tomography scan showed multiple hilar and mediastinal adenopathy with a non-specific opacity in the middle pulmonary lobe. FDG-PET-scan showed increased FDG uptake in the adenopathy, the middle lobe and the right ventricular free wall. Sarcoidosis was confirmed with a lung biopsy. Both electrocardiogram and echocardiogram were normal. Four months later, the patient developed a high-grade atrioventricular block deemed secondary to her cardiac sarcoidosis. Two years later, the patient was referred to a rheumatologist for severe Raynaud's symptoms, sclerodactyly and acrocyanosis. After thorough investigations, a diagnosis of limited cutaneous systemic sclerosis with systemic and cardiac sarcoidosis was made. This case demonstrates that both cardiac sarcoidosis and systemic sclerosis may coexist. In the literature, either disease may come first. In cases where cardiac symptoms appear after the diagnosis of concomitant sarcoidosis and systemic sclerosis, it might be difficult for clinicians to confirm which disease is responsible for the heart involvement. This is important since early cardiac sarcoidosis treatment should be done to prevent major complications and may well differ from systemic sclerosis treatment. In this review, we discuss the main clinical manifestations and imaging findings seen with cardiac disease secondary to sarcoidosis and systemic sclerosis. CI - AJCD Copyright © 2023. FAU - Lemay, Sylvain AU - Lemay S AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Jeantin, Carla AU - Jeantin C AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Kyomi Labelle, Frédérique AU - Kyomi Labelle F AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Philippon, François AU - Philippon F AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Beaudoin, Jonathan AU - Beaudoin J AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Albert, Alexandra AU - Albert A AD - Department of Rheumatology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Dion, Geneviève AU - Dion G AD - Department of Pneumology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Trottier, Mikaël AU - Trottier M AD - Department of Nuclear Medicine, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Dubois, Michelle AU - Dubois M AD - Research Center, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Charbonneau, Éric AU - Charbonneau É AD - Department of Cardiac Surgery, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Gleeton, Guylaine AU - Gleeton G AD - Department of Radiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Massé, Charles AU - Massé C AD - Research Center, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Raymond, Cédric AU - Raymond C AD - Research Center, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. FAU - Birnie, David H AU - Birnie DH AD - Department of Cardiology, University of Ottawa Heart Institute Ottawa, Ontario, Canada. FAU - Sénéchal, Mario AU - Sénéchal M AD - Department of Cardiology, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University Québec, Canada. LA - eng PT - Case Reports PT - Journal Article DEP - 20230815 PL - United States TA - Am J Cardiovasc Dis JT - American journal of cardiovascular disease JID - 101569582 PMC - PMC10509454 OTO - NOTNLM OT - Cardiac sarcoidosis OT - atrioventricular block OT - heart failure OT - systemic sclerosis OT - ventricular arrhythmias COIS- None. EDAT- 2023/09/22 06:42 MHDA- 2023/09/22 06:43 PMCR- 2023/08/15 CRDT- 2023/09/22 03:44 PHST- 2022/11/23 00:00 [received] PHST- 2023/08/15 00:00 [accepted] PHST- 2023/09/22 06:43 [medline] PHST- 2023/09/22 06:42 [pubmed] PHST- 2023/09/22 03:44 [entrez] PHST- 2023/08/15 00:00 [pmc-release] PST - epublish SO - Am J Cardiovasc Dis. 2023 Aug 15;13(4):283-290. eCollection 2023. PMID- 25416648 OWN - NLM STAT- MEDLINE DCOM- 20150818 LR - 20181113 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 8 DP - 2014 Nov 22 TI - Chilblains in Southern California: two case reports and a review of the literature. PG - 381 LID - 10.1186/1752-1947-8-381 [doi] AB - INTRODUCTION: Chilblains or perniosis is an acrally located cutaneous eruption that occurs with exposure to cold. Chilblains can be classified into primary and secondary forms. The primary or idiopathic form is not associated with an underlying disease and is clinically indistinguishable from the secondary form. The secondary form is associated with an underlying condition such as connective tissue disease, monoclonal gammopathy, cryoglobulinemia, or chronic myelomonocytic leukemia. Histopathology cannot accurately help distinguish the primary from secondary forms of chilblains. This article will raise the awareness of chilblains by presenting two unusual case reports of chilblains in men from Southern California with discussion of the appropriate evaluation and treatment of this condition. CASE PRESENTATIONS: Case 1: A 56-year-old Caucasian man presented in January to a Southern California primary care clinic with a report of tingling and burning in both feet, followed by bluish discoloration and swelling as well as blistering. He had no unusual cold exposure prior to the onset of his symptoms. He had a history of "white attacks" in his hands consistent with Raynaud's phenomenon. His symptoms gradually resolved over a 3-week period. Case 2: A 53-year-old Caucasian man also presented to a Southern California clinic in January with a 3-week history of painful tingling in his toes, and subsequent purplish-black discoloration of the toes in both feet. His symptoms occurred 1 week after a skiing trip. He had partial improvement with warming measures. His symptoms resolved 2 weeks after his initial presentation. CONCLUSIONS: Chilblains is a relatively uncommon entity in warmer climates but can present during the winter months. Primary care providers in warmer climates such as Southern California in the USA may be unfamiliar with its presentation. It can be diagnosed clinically by the appearance of typical lesions during the cold damp season. Through a thorough history, physical examination and selected laboratory evaluation, underlying connective tissue disease or a mimic such as vasculitis or cutaneous leukemia can be excluded. FAU - Gordon, Rebecca AU - Gordon R FAU - Arikian, Anne M AU - Arikian AM AD - UCLA Department of Family Medicine Family Health Center, 1920 Colorado Avenue, Santa Monica, CA 90404, USA. aarikian@mednet.ucla.edu. FAU - Pakula, Anita S AU - Pakula AS LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20141122 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 SB - IM MH - California MH - Chilblains/*diagnosis/pathology MH - Humans MH - Male MH - Middle Aged MH - Skin/*pathology PMC - PMC4275761 EDAT- 2014/11/25 06:00 MHDA- 2015/08/19 06:00 PMCR- 2014/11/22 CRDT- 2014/11/23 06:00 PHST- 2014/03/25 00:00 [received] PHST- 2014/09/01 00:00 [accepted] PHST- 2014/11/23 06:00 [entrez] PHST- 2014/11/25 06:00 [pubmed] PHST- 2015/08/19 06:00 [medline] PHST- 2014/11/22 00:00 [pmc-release] AID - 1752-1947-8-381 [pii] AID - 10.1186/1752-1947-8-381 [doi] PST - epublish SO - J Med Case Rep. 2014 Nov 22;8:381. doi: 10.1186/1752-1947-8-381. PMID- 20219319 OWN - NLM STAT- MEDLINE DCOM- 20100610 LR - 20161209 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 39 IP - 5 DP - 2010 May TI - Extraocular manifestations of birdshot chorioretinopathy in 118 French patients. PG - e97-e102 LID - 10.1016/j.lpm.2009.12.005 [doi] AB - INTRODUCTION: Published studies on birdshot chorioretinopathy (BCR) did not provide definitive information on possibly associated extraocular manifestations. METHODS: Single-center cross-sectional analysis of extraocular manifestations in a cohort of patients with BCR. RESULTS: Since 2002, 118 patients (45 men, 73 women) were enrolled. Their mean age was 51.5 years at diagnosis. The most common features of their medical histories were: hypertension (32 patients), drug allergy (19), sinusitis (17), thyroid disease (12), otitis media (11), asthma (11); diabetes (10); cancer (8); psoriasis (5); monoclonal gammopathies (3). At the time of disease onset, arthralgias were noted in 23, ENT manifestations in 26, Raynaud's phenomenon in 6, headaches in 10, psoriasis in 3 others. Between diagnosis and cross-sectional evaluation visits, only the frequency of hypertension has increased significantly (11 additional patients). DISCUSSION AND CONCLUSIONS: No predominant extraocular manifestation of BCR was identified in our patients. Their ongoing follow-up may yet discern whether BCR is definitively eye-restricted. CI - Copyright 2010 Elsevier Masson SAS. All rights reserved. FAU - Pagnoux, Christian AU - Pagnoux C AD - Department of internal medicine, National referral center for "Systemic necrotizing vasculitides and systemic scleroderma", hôpital Cochin, université Paris-Descartes, Assistance publique-Hôpitaux de Paris, 75879 Paris cedex 14, France. christian.pagnoux@cch.aphp.fr FAU - Mahr, Alfred AU - Mahr A FAU - Aouba, Achille AU - Aouba A FAU - Bérezné, Alice AU - Bérezné A FAU - Monnet, Dominique AU - Monnet D FAU - Cohen, Pascal AU - Cohen P FAU - Levinson, Ralph D AU - Levinson RD FAU - Brézin, Antoine P AU - Brézin AP FAU - Guillevin, Loïc AU - Guillevin L LA - eng PT - Journal Article DEP - 20100310 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (HLA-A Antigens) RN - 0 (HLA-A29 antigen) SB - IM MH - Arthralgia/diagnosis MH - Asthma/diagnosis MH - Choroid Diseases/diagnosis MH - Chronic Disease MH - Cohort Studies MH - Cross-Sectional Studies MH - Diabetes Mellitus/diagnosis MH - Female MH - France MH - HLA-A Antigens/analysis MH - Hearing Disorders/diagnosis MH - Humans MH - Hypersensitivity/diagnosis MH - Hypertension/diagnosis MH - Longitudinal Studies MH - Male MH - Medical History Taking MH - Middle Aged MH - Mood Disorders/diagnosis MH - Otitis Media/diagnosis MH - Sinusitis/diagnosis MH - Smoking MH - Thyroid Diseases/diagnosis MH - Uveitis, Posterior/*diagnosis EDAT- 2010/03/12 06:00 MHDA- 2010/06/11 06:00 CRDT- 2010/03/12 06:00 PHST- 2009/10/26 00:00 [received] PHST- 2009/12/07 00:00 [revised] PHST- 2009/12/17 00:00 [accepted] PHST- 2010/03/12 06:00 [entrez] PHST- 2010/03/12 06:00 [pubmed] PHST- 2010/06/11 06:00 [medline] AID - S0755-4982(10)00084-9 [pii] AID - 10.1016/j.lpm.2009.12.005 [doi] PST - ppublish SO - Presse Med. 2010 May;39(5):e97-e102. doi: 10.1016/j.lpm.2009.12.005. Epub 2010 Mar 10. PMID- 41607600 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260129 LR - 20260205 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 36 IP - 4 DP - 2025 Dec TI - Renal Vascular Affection in Systemic Sclerosis Patients and Possible Correlation with Peripheral Vascular Involvement Assessed by Nailfold Capillaroscopy. PG - 600-608 LID - 10.31138/mjr.290425.vad [doi] AB - BACKGROUND: Symptomatic renal involvement was observed in only 24% of systemic sclerosis (SSc) patients. OBJECTIVE: We evaluated renal vascular affection, which is linked to disease characteristics and nailfold capillaroscopy (NFC) outcomes in SSc patients. METHODS: Fifty SSc individuals were subjected to renal doppler ultrasound, NFC, and evaluation of disease severity utilising the Medsger disease severity scale. RESULTS: The cohort comprised 46 females and 4 males, with a mean age of 33.82 ± 11.19 years. Symptomatic renal affection [proteinuria, elevated creatinine level, and mitigated creatinine clearance, and estimated glomerular filtration rate (eGFR) ± hypertension] was observed in 24% of subjects. However, an elevated renal resistive index (RRI) was detected in 44%. Accordingly, patients were allocated into Group 1 (patients exhibiting normal RRI, n = 28) and Group 2 (patients manifesting raised RRI, n = 22). Group 2 patients experienced significantly older age, prolonged disease duration, raised severity scores, protein/creatinine ratio, and avascular NFC scores (p < 0.05). The RRI demonstrated a positive association with age, disease duration, severity, duration of Raynaud's phenomenon, and avascular scores (p < 0.05), alongside negative correlations with creatinine clearance, eGFR, and capillary density (p < 0.05). The main predictors of high RRI encompassed age > 43 years, disease severity score > 5, protein/creatinine ratio > 0.21, capillary density ≤ 6, and avascular score > 1 (p = 0.05). CONCLUSION: The RRI detected early asymptomatic renal affection, which correlated positively with age, disease duration, severity, and high NFC avascular scores and negatively with creatinine clearance and eGFR. CI - © 2025 The Author(s). FAU - Samy, Nermeen AU - Samy N AD - Rheumatology Unit, Internal Medicine Department, Ain Shams University Medical School, Cairo, Egypt. FAU - Fayez, Dalia AU - Fayez D AD - Rheumatology Unit, Internal Medicine Department, Ain Shams University Medical School, Cairo, Egypt. FAU - Tantawy, Salma Hassan AU - Tantawy SH AD - Department of Diagnostic and Interventional Radiology and Molecular Imaging, Faculty of Medicine, Ain Shams University, Cairo, Egypt. FAU - Elziaty, Rahma A AU - Elziaty RA AD - Rheumatology Unit, Internal Medicine Department, Ain Shams University Medical School, Cairo, Egypt. FAU - El-Monem, Nada Mahmoud Abd AU - El-Monem NMA AD - Rheumatology Unit, Internal Medicine Department, Ain Shams University Medical School, Cairo, Egypt. FAU - Hawwash, Amr Mohammad Mohammad AU - Hawwash AMM AD - Rheumatology Unit, Internal Medicine Department, Ain Shams University Medical School, Cairo, Egypt. LA - eng PT - Journal Article DEP - 20251231 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC12835928 OTO - NOTNLM OT - DSS OT - NFC OT - RRI OT - creatinine OT - doppler OT - glomerular filtration rate OT - microscopic angioscopy OT - proteinuria OT - renal duplex OT - scleroderma OT - systemic OT - systemic sclerosis OT - ultrasonography COIS- The authors declare no conflict of interest. EDAT- 2026/01/29 07:34 MHDA- 2026/01/29 07:35 PMCR- 2025/12/31 CRDT- 2026/01/29 04:47 PHST- 2025/04/29 00:00 [received] PHST- 2025/05/26 00:00 [revised] PHST- 2025/06/16 00:00 [accepted] PHST- 2026/01/29 07:35 [medline] PHST- 2026/01/29 07:34 [pubmed] PHST- 2026/01/29 04:47 [entrez] PHST- 2025/12/31 00:00 [pmc-release] AID - MJR-36-4-600 [pii] AID - 10.31138/mjr.290425.vad [doi] PST - epublish SO - Mediterr J Rheumatol. 2025 Dec 31;36(4):600-608. doi: 10.31138/mjr.290425.vad. eCollection 2025 Dec. PMID- 42112395 OWN - NLM STAT- MEDLINE DCOM- 20260511 LR - 20260511 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Case Report: Anti-PL-12 Antisynthetase syndrome complicated by natural killer/T cell non-Hodgkin's lymphoma. PG - 1761119 LID - 10.3389/fimmu.2026.1761119 [doi] LID - 1761119 AB - Antisynthetase syndrome (ASS) is a systemic autoimmune disorder classified as a subtype of the idiopathic inflammatory myopathies (IIM). The condition is defined by the presence of mutually exclusive autoantibodies directed against an aminoacyl-tRNA synthetase along with typical clinical manifestations, including myositis, Raynaud's phenomenon, arthritis, skin lesions such as mechanic hands, or interstitial lung disease (ILD). Anti-synthetase syndrome associated interstitial lung disease (ASS-ILD) can range from mild forms to rapidly progressive disease, which may lead to chronic pulmonary damage if misdiagnosed or inadequately treated. Patients with IIM carry an increased risk of developing neoplasms, most commonly adenocarcinoma, but not lymphoma or other hematologic malignancies. However, data on the association between ASS and malignancy remain very limited. We report the case of a patient with anti-PL-12 antisynthetase syndrome who subsequently developed NK/T-cell non-Hodgkin lymphoma, supplemented by a review of the pertinent literature. CI - Copyright © 2026 Li, Liang, Liu, Guo and Xu. FAU - Li, Kun AU - Li K AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China. FAU - Liang, Mei-E AU - Liang ME AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China. FAU - Liu, Yang AU - Liu Y AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China. FAU - Guo, Qian-Yu AU - Guo QY AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China. FAU - Xu, Ke AU - Xu K AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20260424 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Humans MH - *Myositis/immunology/complications/diagnosis MH - *Autoantibodies/immunology/blood MH - *Lymphoma, Non-Hodgkin/immunology/diagnosis MH - Lung Diseases, Interstitial MH - Male MH - Amino Acyl-tRNA Synthetases/immunology MH - Middle Aged MH - Female MH - *Killer Cells, Natural/immunology PMC - PMC13152836 OTO - NOTNLM OT - ILD OT - PL-12 OT - antisynthetase syndrome OT - natural killer/T-cell lymphoma OT - non-Hodgkin’s lymphoma COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/05/11 13:00 MHDA- 2026/05/11 13:01 PMCR- 2026/04/24 CRDT- 2026/05/11 07:01 PHST- 2025/12/05 00:00 [received] PHST- 2026/03/14 00:00 [revised] PHST- 2026/04/06 00:00 [accepted] PHST- 2026/05/11 13:01 [medline] PHST- 2026/05/11 13:00 [pubmed] PHST- 2026/05/11 07:01 [entrez] PHST- 2026/04/24 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1761119 [doi] PST - epublish SO - Front Immunol. 2026 Apr 24;17:1761119. doi: 10.3389/fimmu.2026.1761119. eCollection 2026. PMID- 38333520 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250202 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 9 IP - 1 DP - 2024 Feb TI - Approval status of essential therapeutic drugs for systemic sclerosis versus that of drugs for rheumatoid arthritis. PG - 23-28 LID - 10.1177/23971983231222368 [doi] AB - OBJECTIVE: Systemic sclerosis, a rare disease characterized by chronic multisystem fibrosis, requires lifelong management, necessitating enough insurance coverage for the patient. Official drug approval is the first step to ensuring that the drug is covered by insurance. In this study, we investigated the approval status of essential therapeutic drugs for systemic sclerosis across eight countries and compared it with that of drugs for rheumatoid arthritis. METHODS: The essential therapeutic drug lists for systemic sclerosis and rheumatoid arthritis were taken from the guidelines of the American College of Rheumatology and the European Alliance of Associations for Rheumatology. Official drug approval status for the selected drugs was confirmed by searching representative Internet databases from eight countries: the United States, the United Kingdom, Germany, France, Italy, Switzerland, Japan, and the Republic of Korea. RESULTS: A total of 21 and 16 drugs were selected for systemic sclerosis and rheumatoid arthritis, respectively. The drug approval rates of the 21 drugs for systemic sclerosis varied among countries. Most drugs used to treat pulmonary arterial hypertension, which were developed recently and are expensive, are approved by most countries; however, most older drugs-which are still essential for management of Raynaud's phenomenon, digital ulcers, interstitial lung disease, and skin fibrosis-are not approved by most countries. By contrast, almost all of the 16 drugs used to treat rheumatoid arthritis, whether old or new, are approved by most countries. CONCLUSION: Approval rates for drugs used to treat systemic sclerosis, a rare disease, are much lower than those for drugs used to treat rheumatoid arthritis. Thus, approval rates of essential therapeutic drugs for systemic sclerosis need to improve, which will benefit patients by increasing the number of drugs covered by insurance. CI - © The Author(s) 2024. FAU - Moon, Ki Won AU - Moon KW AUID- ORCID: 0000-0002-4182-8328 AD - Division of Rheumatology, Department of Internal Medicine, Kangwon National University School of Medicine, Chuncheon, Republic of Korea. FAU - Hwang, Soo-Hee AU - Hwang SH AD - HIRA Research Institute, Health Insurance Review & Assessment Service, Wonju, Republic of Korea. FAU - Yun, Jieun AU - Yun J AD - Department of Pharmaceutical Engineering, Cheongju University, Cheongju, Republic of Korea. FAU - Lee, Eun Bong AU - Lee EB AUID- ORCID: 0000-0003-0703-1208 AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea. AD - Department of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology, Seoul National University, Seoul, Republic of Korea. LA - eng PT - Journal Article DEP - 20240108 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC10848933 OTO - NOTNLM OT - Systemic sclerosis OT - cost OT - drug approval OT - insurance OT - rheumatoid arthritis COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2024/02/09 06:42 MHDA- 2024/02/09 06:43 PMCR- 2025/02/01 CRDT- 2024/02/09 04:02 PHST- 2023/10/02 00:00 [received] PHST- 2023/12/06 00:00 [accepted] PHST- 2024/02/09 06:43 [medline] PHST- 2024/02/09 06:42 [pubmed] PHST- 2024/02/09 04:02 [entrez] PHST- 2025/02/01 00:00 [pmc-release] AID - 10.1177_23971983231222368 [pii] AID - 10.1177/23971983231222368 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2024 Feb;9(1):23-28. doi: 10.1177/23971983231222368. Epub 2024 Jan 8. PMID- 36995331 OWN - NLM STAT- MEDLINE DCOM- 20231228 LR - 20240102 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 41 IP - 12 DP - 2023 Dec TI - Anticentromere antibody positive patients with primary Sjögren's syndrome have distinctive clinical and immunological characteristics. PG - 2371-2378 LID - 10.55563/clinexprheumatol/o3pxq0 [doi] AB - OBJECTIVES: To investigate the clinical manifestations, immunological characteristics, circulating lymphocyte subsets and risk factors of anticentromere antibody (ACA) positive patients with primary Sjögren's syndrome (pSS). METHODS: Data of 333 patients with newly diagnosed pSS were collected and analysed retrospectively. The demographic features, glandular dysfunction, extraglandular manifestations, laboratory data, peripheral blood lymphocyte profiles and serum cytokines were compared between ACA-positive and ACA-negative pSS patients. Logistic regression analysis was used to evaluate the association between ACA and pSS characteristics. RESULTS: The prevalence of ACA among pSS patients was 13.5%. ACA-positive pSS patients were older at diagnosis and had longer disease duration. Xerostomia, xerophthalmia, parotid enlargement, Raynaud's phenomenon (RP), lung and digestive system involvement were more common in ACA-positive group, whereas haematological involvement such as leukopenia was more common in the ACA-negative group. Less frequency of rheumatoid factor, hypergammaglobulinaemia, anti-SSA and anti-SSB positivity, as well as higher positivity rate of ANA were observed in ACA-positive pSS patients, who exhibited a lower ESSDAI. In addition, decreased B cells and elevated NK cells were found in ACA-positive patients. Multivariate analysis identified that disease duration longer than 5 years, parotid enlargement, normal immunoglobulin and the absence of anti-SSA antibody were risk factors of ACA-positive pSS. CONCLUSIONS: ACA positive pSS patients have distinctive clinical manifestations and less severe immunological features, present a lower disease activity and lower activation of the humoral immune system. Physicians should pay attention to RP, lung and liver involvement in this subset of pSS. FAU - Liu, Yixuan AU - Liu Y AD - Department of Graduate School, Hebei Medical University, Shijiazhuang, and Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Guo, Liuxiong AU - Guo L AD - Department of Urology, Hebei General Hospital, Shijiazhuang, China. FAU - Lin, Wei AU - Lin W AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Ning, Xiaoran AU - Ning X AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Liu, Meilu AU - Liu M AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Cao, Jingjing AU - Cao J AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Su, Yashuang AU - Su Y AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Zheng, Xiao AU - Zheng X AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Li, Shuo AU - Li S AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Li, Fang AU - Li F AD - Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China. FAU - Ren, Luping AU - Ren L AD - Department of Endocrinology, Hebei General Hospital, Shijiazhuang, China. FAU - Song, Guangyao AU - Song G AD - Department of Graduate School, Hebei Medical University, Shijiazhuang, and Department of Endocrinology, Hebei General Hospital, Shijiazhuang, China. sguangyao2@163.com. LA - eng PT - Journal Article DEP - 20230316 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Humans MH - *Sjogren's Syndrome/diagnosis/epidemiology MH - Retrospective Studies MH - Antibodies, Antinuclear MH - Risk Factors MH - Rheumatoid Factor EDAT- 2023/03/31 06:00 MHDA- 2023/12/28 06:42 CRDT- 2023/03/30 10:53 PHST- 2022/11/24 00:00 [received] PHST- 2023/03/06 00:00 [accepted] PHST- 2023/12/28 06:42 [medline] PHST- 2023/03/31 06:00 [pubmed] PHST- 2023/03/30 10:53 [entrez] AID - 19417 [pii] AID - 10.55563/clinexprheumatol/o3pxq0 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2023 Dec;41(12):2371-2378. doi: 10.55563/clinexprheumatol/o3pxq0. Epub 2023 Mar 16. PMID- 22127457 OWN - NLM STAT- MEDLINE DCOM- 20120716 LR - 20250626 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 21 IP - 4 DP - 2012 Apr TI - Prognostic factors for survival in systemic lupus erythematosus associated pulmonary hypertension. PG - 353-64 LID - 10.1177/0961203311429815 [doi] AB - OBJECTIVE: Pulmonary hypertension (PH) is a rare but severe manifestation of systemic lupus erythematosus (SLE) that can ultimately result in death. The identification of factors that prognosticate survival in SLE-PH is necessary for appropriate monitoring, timing of therapeutics and lung transplantation. The primary objective of this study was to identify prognostic factors for survival in SLE-PH through review of the literature. The methodological quality of the prognostic studies was also evaluated. METHODS: A systematic review of the literature was performed to identify studies evaluating prognostic factors for survival in SLE-PH. Medline, EMBASE, CINAHL, and Cochrane Central Registry of Controlled Trials (inception - week 2 2010) were searched. A standardized abstraction form was used by two independent reviewers to extract prognostic factors. Methodological quality was evaluated using a validated quality index. RESULTS: Twenty-three observational studies from 375 citations were evaluated. Elevated mean pulmonary artery pressure, Raynaud's phenomenon, thrombocytopenia, plexiform lesion, infection, thrombosis, pregnancy, pulmonary vasculitis and anticardiolipin antibodies were associated with decreased survival. Lupus disease activity, nephritis and central nervous system disease were not associated with survival. The sample sizes were small and methodological quality of the studies was variable. CONCLUSION: This study summarizes factors that may be associated with decreased survival in SLE-PH. The small sample sizes and variable methodological quality preclude definitive conclusions. This study provides the groundwork for further research using large cohorts. FAU - Chow, S L AU - Chow SL AD - Division of Rheumatology, Department of Medicine, University Health Network, University of Toronto, Toronto, Canada. FAU - Chandran, V AU - Chandran V FAU - Fazelzad, R AU - Fazelzad R FAU - Johnson, S R AU - Johnson SR LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20111129 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Disease Progression MH - Evidence-Based Medicine MH - Familial Primary Pulmonary Hypertension MH - Humans MH - Hypertension, Pulmonary/etiology/*mortality MH - Lupus Erythematosus, Systemic/complications/*mortality MH - Prognosis MH - Risk Assessment MH - Risk Factors MH - Survival Analysis EDAT- 2011/12/01 06:00 MHDA- 2012/07/17 06:00 CRDT- 2011/12/01 06:00 PHST- 2011/12/01 06:00 [entrez] PHST- 2011/12/01 06:00 [pubmed] PHST- 2012/07/17 06:00 [medline] AID - 0961203311429815 [pii] AID - 10.1177/0961203311429815 [doi] PST - ppublish SO - Lupus. 2012 Apr;21(4):353-64. doi: 10.1177/0961203311429815. Epub 2011 Nov 29. PMID- 22121273 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20240321 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 56 IP - 5 DP - 2011 Sep-Oct TI - Skin lesions in lupus erythematosus: a marker of systemic involvement. PG - 537-40 LID - 10.4103/0019-5154.87150 [doi] AB - BACKGROUND: Lupus erythematosus (LE) is an autoimmune disorder with diverse clinical manifestation ranging from mild cutaneous disorder to life-threatening systemic illness (SLE). In some patients, it remains to persist in the skin-limited form while in others it evolves into SLE. Here comes the role of identifying the markers of systemic involvement, which could determine the course and prognosis of the disease. AIM: To identify those manifestations that could be used to identify the activity of the disease SLE. MATERIALS AND METHODS: An institution based, descriptive, cross-sectional study carried out over 1 year period. Sixty patients (male : female 1 : 4) with cutaneous LE were recruited for the study. The patients were classified in two groups depending on the presence or absence of ARA criteria of SLE. Detailed account of LE-specific and nonspecific lesions were noted. Statistical significance of the results was compared between the two groups using the chi-square test. RESULTS: Among the different cutaneous manifestations, highly significant (P value <0.001) was found between SLE and nonscarring alopecia, photosensitivity, oral ulcer, malar rash (in decreasing order of odds favoring the association with SLE). Dimorphic skin lesions (P value=0.0326) also showed significant association where as discoid lesion (especially localized variant) predicted toward a skin limited form of the disease with high probability of not developing SLE (P value <0.0001). No significant association was found between SLE and papulosquamous lesions, Raynaud's phenomenon or scarring alopecia. CONCLUSION: Identification of lesions with high degree of association with SLE can alert the physician of the unfavorable prognosis and allow timely intervention and institution of appropriate management strategies. FAU - Das, Nilay Kanti AU - Das NK AD - Department of Dermatology, Institute of Post-Graduate Medical Education and Research and Medical College, Dhakuria, Kolkata, India. FAU - Dutta, Rathindra Nath AU - Dutta RN FAU - Sengupta, Sujit Ranjan AU - Sengupta SR LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3221218 OTO - NOTNLM OT - Cutaneous lupus erythematosus OT - prognostic marker OT - systemic lupus erythematosus COIS- Conflict of Interest: Nil. EDAT- 2011/11/29 06:00 MHDA- 2011/11/29 06:01 PMCR- 2011/09/01 CRDT- 2011/11/29 06:00 PHST- 2011/11/29 06:00 [entrez] PHST- 2011/11/29 06:00 [pubmed] PHST- 2011/11/29 06:01 [medline] PHST- 2011/09/01 00:00 [pmc-release] AID - IJD-56-537 [pii] AID - 10.4103/0019-5154.87150 [doi] PST - ppublish SO - Indian J Dermatol. 2011 Sep-Oct;56(5):537-40. doi: 10.4103/0019-5154.87150. PMID- 34745681 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220307 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2021 DP - 2021 TI - A Rare Case of Fatal Hemorrhagic Stroke in a Young Female with Early Mixed Connective Tissue Disease. PG - 5321438 LID - 10.1155/2021/5321438 [doi] LID - 5321438 AB - Mixed connective tissue disease (MCTD) often presents as a slow progressive illness with low morbidity and mortality. Serious central nervous system disease is uncommon, and fatal outcomes are rarely seen. Here, we report a rare case of fatal hemorrhagic stroke in a 43-year-old female with a rapidly progressive MCTD. She presented to primary care with a history of headaches, visual disturbances, and unprovoked lower extremity swelling and pain. A rheumatological workup showed positive antinuclear (ANA) and ribonucleoprotein (RNP) antibodies. Magnetic resonance imaging (MRI) found a 12 mm hemorrhage along a cortical sulcus of the right frontal lobe, and a follow-up magnetic resonance angiography (MRA) and ophthalmological exam showed no definitive signs of vasculitis. Over the course of her workup, she developed swollen hands, Raynaud's syndrome, myalgias, and synovitis characteristic of evolving MCTD. The patient then began to experience severe headaches over one month. Repeat MRI was ordered, but never completed, and the patient presented to the emergency department (ED) with a severe, right-sided headache, and left-sided visual disturbance. In the ED, she began to display evidence of delirium and seizure activity and became unresponsive. A computerized tomography scan (CT) of the brain showed a right parietal lobe intraparenchymal hemorrhage approximately 5 × 3 × 5 cm in size with secondary mass effect including mid- and hind-brain herniation. Computerized tomography angiography (CTA) of the brain showed signs of large vessel vasculitis. A craniectomy was performed; however, the patient never regained consciousness and died several days later. Vasculitis, while rare in connective tissue diseases, should be aggressively assessed for and managed in patients with any early signs and symptoms of cerebrovascular involvement to prevent fatal outcomes. CI - Copyright © 2021 James R. Agapoff IV. FAU - Agapoff Iv, James R AU - Agapoff Iv JR AUID- ORCID: 0000-0002-6026-6463 AD - Department of Psychiatry, University of Hawai'i at Mānoa, John A. Burns School of Medicine, Honolulu, HI, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20211028 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 EIN - Case Rep Rheumatol. 2022 Feb 23;2022:9762151. doi: 10.1155/2022/9762151. PMID: 35251731 PMC - PMC8568535 COIS- The author declares no conflicts of interest. EDAT- 2021/11/09 06:00 MHDA- 2021/11/09 06:01 PMCR- 2021/10/28 CRDT- 2021/11/08 06:44 PHST- 2021/07/30 00:00 [received] PHST- 2021/10/16 00:00 [accepted] PHST- 2021/11/08 06:44 [entrez] PHST- 2021/11/09 06:00 [pubmed] PHST- 2021/11/09 06:01 [medline] PHST- 2021/10/28 00:00 [pmc-release] AID - 10.1155/2021/5321438 [doi] PST - epublish SO - Case Rep Rheumatol. 2021 Oct 28;2021:5321438. doi: 10.1155/2021/5321438. eCollection 2021. PMID- 30474928 OWN - NLM STAT- MEDLINE DCOM- 20200511 LR - 20200511 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 72 IP - 1 DP - 2020 Jan TI - Ten Years of Interventional Research in Systemic Sclerosis: A Systematic Mapping of Trial Registries. PG - 140-148 LID - 10.1002/acr.23817 [doi] AB - OBJECTIVE: To provide a comprehensive overview of interventional clinical trials registered in international databases and planned and conducted within the last 10 years in patients with systemic sclerosis (SSc). METHODS: We searched the International Clinical Trials Registry Platform for all records on interventional clinical trials targeting patients with SSc performed since September 2007. Two reviewers selected studies according to the prespecified eligibility criteria. Information on start date, country of origin, funding sources, phase of development, study design, (planned) sample size, enrollment status, outcomes, disease complication, and treatments investigated were retrieved and summarized. RESULTS: Among the 198 eligible studies identified (122 randomized controlled trials [RCTs; 62%]), 87 (30%) were conducted in Europe, 165 (83%) in a single country, and 81 (41%) were industry-funded. The majority of trials investigated pharmacologic treatments (75%), mostly nonbiotherapies (57%). RCTs were mostly 2-arm (82%) placebo-controlled (71%) studies with a median number of patients enrolled or planned to be enrolled of 40 (interquartile range 25-77 [range 10-586]). Twenty-one RCTs (17%) planned to enroll or enrolled >100 patients. Time to assess the primary outcome was found to be adequate in 29% to 50% of RCTs retrieved. Patients age >65 years were excluded in 14% of studies. SSc complications more frequently investigated in overall studies were skin thickness (26%), Raynaud's phenomenon/digital ulcers (24%), and interstitial lung disease (14%). CONCLUSION: The SSc research landscape is dominated by small, short, and mainly placebo-controlled trials, especially investigating pharmacologic treatments. Some patients' needs continue to be neglected. CI - © 2018, American College of Rheumatology. FAU - Iudici, Michele AU - Iudici M AUID- ORCID: 0000-0001-5871-8806 AD - INSERM, UMR 1153, Epidemiology and Biostatistics Sorbonne Paris Cité Research Center, Paris, France. FAU - Bafeta, Aïda AU - Bafeta A AD - INSERM, UMR 1153, Epidemiology and Biostatistics Sorbonne Paris Cité Research Center, Paris, France. FAU - Atal, Ignacio AU - Atal I AD - INSERM, UMR 1153, Epidemiology and Biostatistics Sorbonne Paris Cité Research Center, Paris, France. FAU - Ravaud, Philippe AU - Ravaud P AD - INSERM, UMR 1153, Epidemiology and Biostatistics Sorbonne Paris Cité Research Center, Hôpital Hôtel-Dieu, Assistance Publique des Hôpitaux de Paris, and Cochrane France, Paris, France, and Columbia University, Mailman School of Public Health, New York, New York. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20191210 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adolescent MH - Adult MH - Aged MH - *Clinical Trials as Topic MH - *Disease Management MH - Female MH - *Forecasting MH - Humans MH - Male MH - Middle Aged MH - *Registries MH - Retrospective Studies MH - Scleroderma, Systemic/*therapy MH - Young Adult EDAT- 2018/11/27 06:00 MHDA- 2020/05/12 06:00 CRDT- 2018/11/27 06:00 PHST- 2018/08/20 00:00 [received] PHST- 2018/11/20 00:00 [accepted] PHST- 2018/11/27 06:00 [pubmed] PHST- 2020/05/12 06:00 [medline] PHST- 2018/11/27 06:00 [entrez] AID - 10.1002/acr.23817 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2020 Jan;72(1):140-148. doi: 10.1002/acr.23817. Epub 2019 Dec 10. PMID- 25627300 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 54 IP - 5 DP - 2014 Sep-Oct TI - [Hashimoto thyroiditis may be associated with a subset of patients with systemic sclerosis with pulmonary hypertension]. PG - 366-70 LID - S0482-5004(14)00104-1 [pii] LID - 10.1016/j.rbr.2014.04.001 [doi] AB - INTRODUCTION: Recent studies show an association between autoimmune thyroiditis and systemic sclerosis (SSc) and suggest that this condition may interfere with the ES phenotype. However these studies evaluate the autoimmune thyroiditis as a whole and none of them specifically addresses Hashimoto's thyroiditis (HT) in SSc. OBJECTIVE: To investigate the presence of HT in SSc patients and its possible association with disease manifestations. METHODS: Clinical manifestations of hypothyroidism, TSH and anti-thyroid auto antibodies (anti-TPO. anti TBG and TRAb) were studied in 56 patients with SSc. SSc patients with HT were compared with SSc patients without thyroiditis. RESULTS: HT was observed in 19.64% of patients with SSc. No association was observed between HT and the different forms of disease or profile of autoantibodies. Likewise, there was no difference between the mean modified Rodnan score and presence of Raynaud's phenomenon, scars, digital necrosis, myositis, arthritis, sicca symptoms, esophageal dysmotility and scleroderma renal crisis when the groups were compared. On the other hand, patients with HT had higher frequency of pulmonary hypertension in relation to patients without HT (66.6% vs 22.5%, p=0.016). CONCLUSIONS: In the studied sample patients with ES and HT had higher prevalence of pulmonary hypertension. Long-term follow-up studies with a larger number of TH and SSc patients are needed to confirm these data. CI - Copyright © 2014 Elsevier Editora Ltda. All rights reserved. FAU - Costa, Ciliana Cardoso B AU - Costa CC AD - Hospital Universitário Evangélico de Curitiba, Curitiba, PR, Brasil. FAU - Medeiros, Morgana AU - Medeiros M AD - Hospital Universitário Evangélico de Curitiba, Curitiba, PR, Brasil. FAU - Watanabe, Karen AU - Watanabe K AD - Faculdade Evangélica do Paraná, Curitiba, PR, Brasil. FAU - Martin, Patricia AU - Martin P AD - Hospital Universitário Evangélico de Curitiba, Curitiba, PR, Brasil. FAU - Skare, Thelma L AU - Skare TL AD - Hospital Universitário Evangélico de Curitiba, Curitiba, PR, Brasil. Electronic address: tskare@onda.com.br. LA - por PT - Journal Article TT - Tireoidite de Hashimoto pode estar associada a um subgrupo de pacientes de esclerose sistêmica com hipertensão pulmonar. DEP - 20140706 PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Cross-Sectional Studies MH - Female MH - Hashimoto Disease/*complications MH - Humans MH - Hypertension, Pulmonary/epidemiology/*etiology MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications MH - Young Adult OTO - NOTNLM OT - Esclerodermia OT - Esclerose sistêmica OT - Hashimoto thyroiditis OT - Hipertensão pulmonar OT - Pulmonary hypertension OT - Scleroderma OT - Systemic sclerosis OT - Tireoidite de Hashimoto EDAT- 2015/01/30 06:00 MHDA- 2016/12/15 06:00 CRDT- 2015/01/29 06:00 PHST- 2013/12/20 00:00 [received] PHST- 2014/03/01 00:00 [revised] PHST- 2014/04/24 00:00 [accepted] PHST- 2015/01/29 06:00 [entrez] PHST- 2015/01/30 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - S0482-5004(14)00104-1 [pii] AID - 10.1016/j.rbr.2014.04.001 [doi] PST - ppublish SO - Rev Bras Reumatol. 2014 Sep-Oct;54(5):366-70. doi: 10.1016/j.rbr.2014.04.001. Epub 2014 Jul 6. PMID- 36143053 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220928 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 11 IP - 18 DP - 2022 Sep 14 TI - Patterns of Outpatient Phecodes Predating the Diagnosis of Systemic Lupus Erythematosus in Taiwanese Women. LID - 10.3390/jcm11185406 [doi] LID - 5406 AB - Shortening the time to diagnosis and initiating early treatment are imperative to improve outcomes in patients with systemic lupus erythematosus (SLE). The aim of this case-control study, based on the data from the Taiwan's National Health Insurance Research Database (NHIRD), was to investigate the patterns of diagnoses of disease phenotypes in female patients with SLE up to eight years prior to its definitive diagnosis. The 547 cases were selected from the 2000-2012 NHIRD catastrophic illness datafile and frequency-matched with 2188 controls. The primary diagnosis based on the first ICD-9-CM code for each outpatient visit was converted to Phecodes. Separate regression models, based on least absolute shrinkage and selection operator (lasso) regularization, with seven different lag periods from 1-2 to 7-8 years, were conducted. Results showed that SLE was associated with 46 disease phenotypes in a lag period of 2-3 years, but fewer in other lag periods. A number of SLE-associated disease phenotypes, such as primary thrombocytopenia, thyroid diseases, Raynaud's syndrome, renal disease, and several infectious diseases, occurred mainly in the first few years prior to SLE diagnosis. In conclusion, SLE should be suspected when the disease phenotypes identified in the present study occurred concomitantly. FAU - Lu, Ming-Chi AU - Lu MC AUID- ORCID: 0000-0001-9051-0351 AD - Division of Allergy, Immunology and Rheumatology, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin 622401, Chiayi, Taiwan. AD - School of Medicine, Tzu Chi University, Hualien City 97004, Hualien, Taiwan. FAU - Hsu, Chia-Wen AU - Hsu CW AD - Department of Medical Research, Dalin Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Dalin 622401, Chiayi, Taiwan. FAU - Koo, Malcolm AU - Koo M AUID- ORCID: 0000-0002-9242-9167 AD - Graduate Institute of Long-Term Care, Tzu Chi University of Science and Technology, Hualien City 970302, Hualien, Taiwan. AD - Dalla Lana School of Public Health, University of Toronto, Toronto, ON M5T 3M7, Canada. LA - eng GR - TCMF-A 108-05/Buddhist Tzu Chi Medical Foundation, Taiwan/ PT - Journal Article DEP - 20220914 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC9506474 OTO - NOTNLM OT - National Health Insurance Research Database OT - Phecodes OT - disease phenotypes OT - lasso regression OT - systemic lupus erythematosus COIS- The authors declare no conflict of interest. EDAT- 2022/09/24 06:00 MHDA- 2022/09/24 06:01 PMCR- 2022/09/14 CRDT- 2022/09/23 01:28 PHST- 2022/08/24 00:00 [received] PHST- 2022/09/10 00:00 [revised] PHST- 2022/09/12 00:00 [accepted] PHST- 2022/09/23 01:28 [entrez] PHST- 2022/09/24 06:00 [pubmed] PHST- 2022/09/24 06:01 [medline] PHST- 2022/09/14 00:00 [pmc-release] AID - jcm11185406 [pii] AID - jcm-11-05406 [pii] AID - 10.3390/jcm11185406 [doi] PST - epublish SO - J Clin Med. 2022 Sep 14;11(18):5406. doi: 10.3390/jcm11185406. PMID- 38897945 OWN - NLM STAT- MEDLINE DCOM- 20250702 LR - 20250702 IS - 1347-3409 (Electronic) IS - 1345-4676 (Linking) VI - 92 IP - 3 DP - 2025 Jul 2 TI - Use of Tocilizumab to Treat Arthritis Associated with Mixed Connective Tissue Disease Complicated by Ovarian Teratoma: A Case Report. PG - 300-304 LID - 10.1272/jnms.JNMS.2025_92-303 [doi] AB - Mixed connective tissue disease (MCTD) is characterized by mixed features of systemic lupus erythematosus, systemic sclerosis, and polymyositis/dermatomyositis and is rare in children. Here, we report a case of MCTD in a 10-year-old girl who presented at our hospital with arthralgia, Raynaud's phenomenon, and fatigue. Blood tests were positive for anti-U1-ribonucleoprotein (RNP) antibodies and for rheumatoid factors (RFs) IgG-RF and anti-galactose-deficient IgG. Levels of myogenic enzymes and hypergammaglobulinemia were elevated. Macrophages were prominent in bone marrow, with scattered phagocytic macrophages. MCTD was diagnosed based on the patient's symptoms and laboratory findings. Methylprednisolone pulse therapy combined with oral tacrolimus was administered, which led to resolution of symptoms. Three months after pulse therapy, arthralgia worsened and methotrexate was administered. Arthralgia improved but did not resolve. Magnetic resonance imaging performed to investigate the hip pain revealed a mature ovarian teratoma, which was surgically removed. Because the pain persisted and interfered with her daily life, she was treated with tocilizumab for joint pain relief, which decreased the pain level. Tocilizumab is a candidate for additional treatment of juvenile idiopathic arthritis-like arthritis associated with childhood-onset MCTD. FAU - Ota, Haruka AU - Ota H AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Igarashi, Toru AU - Igarashi T AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Matsui, Ryosuke AU - Matsui R AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Takeshita, Hikaru AU - Takeshita H AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Hashimoto, Koji AU - Hashimoto K AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Miyao, Masaki AU - Miyao M AD - Department of Pediatric Surgery, Nippon Medical School Musashi Kosugi Hospital. FAU - Motoda, Norio AU - Motoda N AD - Department of Diagnostic Pathology, Nippon Medical School Musashi Kosugi Hospital. FAU - Takahashi, Tsubasa AU - Takahashi T AD - Department of Pediatric Surgery, Nippon Medical School Musashi Kosugi Hospital. FAU - Hayakawa, Jun AU - Hayakawa J AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Migita, Makoto AU - Migita M AD - Department of Pediatrics, Nippon Medical School Musashi Kosugi Hospital. FAU - Itoh, Yasuhiko AU - Itoh Y AD - Department of Pediatrics, Nippon Medical School. LA - eng PT - Case Reports PT - Journal Article DEP - 20240618 PL - Japan TA - J Nippon Med Sch JT - Journal of Nippon Medical School = Nippon Ika Daigaku zasshi JID - 100935589 RN - I031V2H011 (tocilizumab) RN - 0 (Antibodies, Monoclonal, Humanized) RN - Teratoma, Ovarian SB - IM MH - Humans MH - Female MH - Child MH - *Ovarian Neoplasms/complications/surgery MH - *Antibodies, Monoclonal, Humanized/therapeutic use/administration & dosage MH - *Teratoma/complications/surgery MH - *Mixed Connective Tissue Disease/complications/drug therapy/diagnosis MH - Treatment Outcome MH - *Arthritis/drug therapy/etiology/complications OTO - NOTNLM OT - MCTD OT - mixed connective tissue disease OT - ovarian teratoma OT - tocilizumab EDAT- 2024/06/20 00:42 MHDA- 2025/07/03 00:27 CRDT- 2024/06/19 22:33 PHST- 2025/07/03 00:27 [medline] PHST- 2024/06/20 00:42 [pubmed] PHST- 2024/06/19 22:33 [entrez] AID - 10.1272/jnms.JNMS.2025_92-303 [doi] PST - ppublish SO - J Nippon Med Sch. 2025 Jul 2;92(3):300-304. doi: 10.1272/jnms.JNMS.2025_92-303. Epub 2024 Jun 18. PMID- 40316396 OWN - NLM STAT- MEDLINE DCOM- 20250503 LR - 20250502 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 21 IP - 3 DP - 2025 Mar TI - Nailfold capillaroscopy changes in systemic lupus erythematosus patients: Correlation with disease activity and anti-uridin1-ribonucleoprotein antibodies. PG - 501840 LID - S2173-5743(25)00039-5 [pii] LID - 10.1016/j.reumae.2025.501840 [doi] AB - INTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune disease that causes multiple vascular complications, including endothelial cell damage. Nailfold capillaroscopy is the most effective non-invasive imaging technique for assessing the morphology of nailfold capillaries, and approximately half of the SLE patients have non-specific nailfold capillaroscopy abnormalities. Anti-uridin1-ribonucleoprotein antibodies are present in systemic lupus erythematosus patients with Raynaud's phenomenon, pulmonary artery hypertension, esophageal dysmotility, myopathy, and no severe renal involvement. AIM: To detect different patterns of nailfold capillaroscopic changes in SLE patients, their correlation with SLE disease activity, and anti-U1-RNP antibodies. PATIENTS AND METHODS: A case-control study included eighty-six SLE patients, and disease activity was assessed using the SLEDAI-2K. All patients had a nailfold capillaroscopic examination. Anti-uridin1-ribonucleoprotein antibodies were measured in all patients. RESULTS: Anti-uridin1-ribonucleoprotein antibodies had a significant inverse correlation with microhemorrhages and a significant direct relationship between anti-dsDNA antibody positivity and the presence of microhemorrhage. Additionally, a significant direct correlation was found between giant capillaries, venous plexus visibility, and higher ESR and CRP. Raynaud's phenomenon was significantly correlated with SLEDAI-2K, swollen joints, tender joints, and anti-dsDNA. Multiple linear regression analysis revealed that microhemorrhages and giant capillaries were the most significant predictors of lupus disease activity. CONCLUSION: Our findings highlight the prevalence of microvascular abnormalities in systemic lupus erythematosus, including tortuosity, crossing, elongation, microhemorrhages, and giant capillaries, emphasizing the importance of NFC in assessing microcirculation and disease activity. Also, it adds to the growing body of evidence supporting the prognostic value of capillary abnormalities, particularly microhemorrhages and giant capillaries, as predictors of disease activity in systemic lupus erythematosus patients. Nailfold capillaroscopic examination can assess lupus activity and potentially predict the risk of serious complications. CI - Copyright © 2025 Sociedad Española de Reumatología (SER), Colegio Mexicano de Reumatología (CMR) and Elsevier España, S.L.U. All rights reserved. FAU - Makarem, Yasmine S AU - Makarem YS AD - Rheumatology & Rehabilitation Department, Faculty of Medicine, Assiut University, Assiut, Egypt. Electronic address: yasmine.saad@aun.edu.eg. FAU - Selim, Zahraa I AU - Selim ZI AD - Rheumatology & Rehabilitation Department, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Ismail, Sherif AU - Ismail S AD - Rheumatology & Rehabilitation, Department of Internal Medicine, Medical Research and Clinical Studies Institute, National Research Center, Egypt. FAU - Imam Mekkawy, Amera AU - Imam Mekkawy A AD - Rheumatology & Rehabilitation Department, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Galal, Hanan AU - Galal H AD - Clinical Pathology Department, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - El Nouby, Fatma H AU - El Nouby FH AD - Rheumatology & Rehabilitation Department, Faculty of Medicine, Assiut University, Assiut, Egypt. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/blood/immunology/diagnostic imaging/complications MH - *Microscopic Angioscopy MH - Female MH - Male MH - Adult MH - Case-Control Studies MH - Middle Aged MH - *Antibodies, Antinuclear/blood MH - Severity of Illness Index MH - *Nails/blood supply MH - Young Adult MH - *Autoantibodies/blood MH - Capillaries OTO - NOTNLM OT - Anti-U1-RNP OT - Capilaroscopia ungueal OT - Lupus eritematoso sistémico OT - Nailfold capillaroscopy OT - Systemic lupus erythematosus EDAT- 2025/05/03 00:36 MHDA- 2025/05/03 22:24 CRDT- 2025/05/02 21:00 PHST- 2024/09/26 00:00 [received] PHST- 2025/02/02 00:00 [revised] PHST- 2025/02/13 00:00 [accepted] PHST- 2025/05/03 22:24 [medline] PHST- 2025/05/03 00:36 [pubmed] PHST- 2025/05/02 21:00 [entrez] AID - S2173-5743(25)00039-5 [pii] AID - 10.1016/j.reumae.2025.501840 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2025 Mar;21(3):501840. doi: 10.1016/j.reumae.2025.501840. PMID- 27608870 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 63 IP - 9 DP - 2015 Sep TI - Salt and Pepper Pigmentation - Skin Manifestation of Systemic Sclerosis. PG - 70 AB - A 50 year old male presented with progressive difficulty in swallowing both liquid and solid food with no history of Raynaud's phenomenon. A general examination revealed skin changes in the form of thickening, hyperpigmentation and tightening of skin of fingers, hand, forearm and legs. The patient had painless skin induration over the legs, forearm and hand. Salt and pepper pigmentation was seen on the upper back (Figure 1a), over mastoid process (Figure 1b) and the concha of pinna (Figure 1c). Anti-Scl 70 was positive. Anti-centromere antibodies were negative. Pulmonary function testing (PFT) revealed very severe restrictive lung disease. Barium swallow study was normal. Despite being advised to undergo oesophageal manometry test in view of dysphagia, patient was not willing for the same. Diagnosis of systemic sclerosis was made. Systemic sclerosis is a disease in which extensive fibrosis, vascular alterations and autoantibodies against various cellular antigens being the principal features with a female to male ratio of 4:1. Skin pigmentation changes among other features of skin involvement include a salt-and-pepper appearance due to diffuse hyperpigmentation with sparing of the perifollicular areas. This may be due to the richer capillary network that may warm the perifollicular skin and preserve melanogenesis producing the perifollicular pigment retention in systemic sclerosis.1,2 Both cellular and humoral immune factors in combination with external factors such as trauma or inflammation may trigger the destruction of melanocytes.3 Moreover, various physical factors like temperature changes as well as genetic, hormonal factors may influence pigment formation. Such changes in pigmentation is also seen during repigmentation around hair follicles in vitiligo. Clinically, both vitiligo and depigmented lesions of systemic sclerosis present as chalk-white macules with well-defined borders. However, mucosal involvement is commonly seen in vitiligo while depigmented lesions are usually not observed involving the lips or oral cavity in systemic sclerosis. CI - © Journal of the Association of Physicians of India 2011. FAU - Vijayaraju, D AU - Vijayaraju D AD - Assistant Professor. FAU - Prakash, G AU - Prakash G AD - Assistant Professor. FAU - Yoganandh, T AU - Yoganandh T AD - Assistant Professor. FAU - Subramanian, S R AU - Subramanian SR AD - Professor. FAU - Ramkumar, S AU - Ramkumar S AD - Postgraduate Student, Dept. of General Medicine, Govt. Mohan Kumaramangalam Medical College and Hospital, Salem, Tamil Nadu. LA - eng PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM EDAT- 2016/09/10 06:00 MHDA- 2016/09/10 06:01 CRDT- 2016/09/10 06:00 PHST- 2016/09/10 06:00 [entrez] PHST- 2016/09/10 06:00 [pubmed] PHST- 2016/09/10 06:01 [medline] PST - ppublish SO - J Assoc Physicians India. 2015 Sep;63(9):70. PMID- 31586977 OWN - NLM STAT- MEDLINE DCOM- 20191009 LR - 20191010 IS - 0043-5147 (Print) IS - 0043-5147 (Linking) VI - 72 IP - 9 cz 1 DP - 2019 TI - [Paraneoplastic rheumatologic syndromes in the elderly]. PG - 1646-1654 AB - Risk of development of malignant tumors increases in elderly people. There are numerous clinical symptoms mimicking primary rheumatic diseases in the course of cancers, referred to as paraneoplastic rheumatologic syndromes. They are not caused directly by the tumor or its metastases, but result from the action of biologically active substances released by cancer cells and abnormal immunological reactions. Paraneoplastic rheumatologic syndromes may precede the diagnosis of cancer, occur simultaneously or occur after the diagnosis of malignancy. In clinical practice, it is very difficult to distinguish paraneoplastic syndromes from idiopathic rheumatic diseases. However, some clinical features may suggest the paraneoplastic nature of rheumatic diseases, including the onset of symptoms in old age, atypical and rapidly progressive course or poor response to conventional treatment. Early and well-targeted diagnostics allow the diagnosis of often latent neoplasm, and its effective treatment may lead to resolution of paraneoplastic rheumatic symptoms. This paper discusses selected paraneoplastic rheumatologic syndromes that often occur in older people, including carcinomatous polyarthritis, remitting seronegative symmetrical synovitis with pitting edema (RS3PE), palmar fasciitis-polyarthritis syndrome, hypertrophic osteoarthropathy, tumor-induced osteomalacia, cancerassociated myositis, paraneoplastic scleroderma like syndrome, paraneoplastic vasculitis and paraneoplastic Raynaud's syndrome. FAU - Parada-Turska, Jolanta AU - Parada-Turska J AD - Katedra i Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny w Lublinie, Lublin, Polska. LA - pol PT - Journal Article PL - Poland TA - Wiad Lek JT - Wiadomosci lekarskie (Warsaw, Poland : 1960) JID - 9705467 SB - IM MH - Aged MH - Aged, 80 and over MH - Arthritis, Rheumatoid MH - Humans MH - Myositis MH - Neoplasms MH - Paraneoplastic Syndromes/*diagnosis MH - Rheumatic Diseases/*diagnosis MH - Synovitis OTO - NOTNLM OT - cancer-associated myositis OT - paraneoplastic arthritis OT - paraneoplastic bone disease OT - paraneoplastic scleroderma like syndrome OT - paraneoplastic syndrome OT - paraneoplastic vasculitis OT - rheumatic diseases EDAT- 2019/10/07 06:00 MHDA- 2019/10/11 06:00 CRDT- 2019/10/07 06:00 PHST- 2019/10/07 06:00 [entrez] PHST- 2019/10/07 06:00 [pubmed] PHST- 2019/10/11 06:00 [medline] PST - ppublish SO - Wiad Lek. 2019;72(9 cz 1):1646-1654. PMID- 28414153 OWN - NLM STAT- MEDLINE DCOM- 20171023 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 16 IP - 6 DP - 2017 Jun TI - Inflammatory myopathy associated with myasthenia gravis with and without thymic pathology: Report of four cases and literature review. PG - 644-649 LID - S1568-9972(17)30106-4 [pii] LID - 10.1016/j.autrev.2017.04.009 [doi] AB - INTRODUCTION: The association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: We described four patients with both MG and inflammatory myopathy. RESULTS: These cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma. DISCUSSION: The recognition of these neuromuscular co-morbidities contributes to (i) understanding their pathogenic mechanisms, (ii) developing better management approaches and (iii) further improving disease outcomes. CI - Copyright © 2017 Elsevier B.V. All rights reserved. FAU - Santos, Ernestina AU - Santos E AD - Neurology Department, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal; Multidisciplinary Unit for Biomedical Research (UMIB), Institute of Biomedical Sciences Abel Salazar (ICBAS), University of Porto, Portugal. Electronic address: ernestina.santos@gmail.com. FAU - Coutinho, Ester AU - Coutinho E AD - Nuffield Department of Clinical Neurosciences, Oxford University Hospitals and University of Oxford, Oxford, United Kingdom. Electronic address: estercoutinho@gmail.com. FAU - Martins da Silva, Ana AU - Martins da Silva A AD - Neurology Department, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal; Multidisciplinary Unit for Biomedical Research (UMIB), Institute of Biomedical Sciences Abel Salazar (ICBAS), University of Porto, Portugal. Electronic address: anaadmsilva@gmail.com. FAU - Marinho, António AU - Marinho A AD - Clinical Immunology Unit, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal. Electronic address: antmarinho@hotmail.com. FAU - Vasconcelos, Carlos AU - Vasconcelos C AD - Clinical Immunology Unit, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal. Electronic address: cvcarlosvasconcelos@gmail.com. FAU - Taipa, Ricardo AU - Taipa R AD - Neuropathology Unit, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal. Electronic address: ricardotaipa@gmail.com. FAU - Pires, Manuel Melo AU - Pires MM AD - Neuropathology Unit, Hospital Santo António, Centro Hospitalar Porto, Porto, Portugal. Electronic address: melopires@hotmail.com. FAU - Gonçalves, Guilherme AU - Gonçalves G AD - Multidisciplinary Unit for Biomedical Research (UMIB), Institute of Biomedical Sciences Abel Salazar (ICBAS), University of Porto, Portugal. Electronic address: aggoncalves@icbas.up.pt. FAU - Lopes, Carlos AU - Lopes C AD - Pathology and Molecular Immunology Department, Instituto de Ciências Biomédicas de Abel Salazar, Universidade do Porto, Porto, Portugal. Electronic address: lopes81241@gmail.com. FAU - Leite, Maria Isabel AU - Leite MI AD - Nuffield Department of Clinical Neurosciences, Oxford University Hospitals and University of Oxford, Oxford, United Kingdom. Electronic address: maria.leite@ndcn.ox.ac.uk. LA - eng PT - Case Reports PT - Journal Article DEP - 20170413 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Adult MH - Aged MH - Comorbidity MH - Female MH - Humans MH - Male MH - *Myasthenia Gravis/epidemiology/pathology MH - *Myositis/epidemiology/pathology MH - Thymoma/epidemiology/pathology MH - Thymus Gland/*pathology MH - Thymus Neoplasms/epidemiology/pathology OTO - NOTNLM OT - Muscle biopsy OT - Myasthenia gravis OT - Myositis OT - Thymoma EDAT- 2017/04/18 06:00 MHDA- 2017/10/24 06:00 CRDT- 2017/04/18 06:00 PHST- 2017/03/17 00:00 [received] PHST- 2017/03/22 00:00 [accepted] PHST- 2017/04/18 06:00 [pubmed] PHST- 2017/10/24 06:00 [medline] PHST- 2017/04/18 06:00 [entrez] AID - S1568-9972(17)30106-4 [pii] AID - 10.1016/j.autrev.2017.04.009 [doi] PST - ppublish SO - Autoimmun Rev. 2017 Jun;16(6):644-649. doi: 10.1016/j.autrev.2017.04.009. Epub 2017 Apr 13. PMID- 34302594 OWN - NLM STAT- MEDLINE DCOM- 20211216 LR - 20211216 IS - 1865-3774 (Electronic) IS - 0925-5710 (Linking) VI - 114 IP - 5 DP - 2021 Nov TI - Fulminant type I cryoglobulinemic glomerulonephritis with unique ultrastructural plugs: a case report. PG - 620-625 LID - 10.1007/s12185-021-03194-8 [doi] AB -  Type I cryoglobulinemia is a rare disease which affects the skin, central nervous system and kidneys. It is usually associated with lymphoproliferative disorders such as multiple myeloma, lymphoma and monoclonal gammopathy of renal significance. Proteinuria and membranoproliferative glomerulonephritis are the most common renal manifestations; Case presentation: Here we report the case of a female patient in her late 40 s who had proteinuria accompanied by Raynaud's phenomenon, high blood and plasma viscosity, hearing loss, and cardiac and central nervous system involvement. Monoclonal immunoglobulin G-λ protein was detected and serum was positive for cryoglobulin. Renal biopsy revealed massive cryo-plugs with unique ultrastructural appearance in the glomerular and peritubular capillary lumina. Immunofluorescence showed predominant IgG3/λ deposition in cryo-plugs. As reported, the clinical manifestations of this patient resulted from cryoprecipitate and hyperviscosity syndrome; Conclusion: Cryoglobulinemia should be considered as a possible diagnosis in patients with Raynaud's phenomenon, hyperviscosity syndrome and monoclonal immunoglobulin. CI - © 2021. Japanese Society of Hematology. FAU - Weng, Qinjie AU - Weng Q AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Xu, Jing AU - Xu J AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Shen, Pingyan AU - Shen P AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Xu, Tian AU - Xu T AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Gao, Chenni AU - Gao C AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Xie, Jingyuan AU - Xie J AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Ren, Hong AU - Ren H AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. FAU - Pan, Xiaoxia AU - Pan X AUID- ORCID: 0000-0002-2963-7019 AD - Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China. pxx10768@rjh.com.cn. LA - eng PT - Case Reports PT - Journal Article DEP - 20210724 PL - Japan TA - Int J Hematol JT - International journal of hematology JID - 9111627 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunoglobulin G) SB - IM MH - Autoantibodies/blood/immunology MH - Biomarkers MH - Biopsy MH - Female MH - Fluorescent Antibody Technique MH - Glomerulonephritis, Membranoproliferative/*diagnosis/etiology MH - Humans MH - Immunoglobulin G/blood/immunology MH - Immunohistochemistry MH - Kidney/metabolism/*pathology/*ultrastructure MH - Middle Aged MH - Symptom Assessment MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Cryoglobulinemic glomerulonephritis OT - Monoclonal gammopathy of renal significance OT - Type I cryoglobulinemia EDAT- 2021/07/25 06:00 MHDA- 2021/12/17 06:00 CRDT- 2021/07/24 12:10 PHST- 2021/02/01 00:00 [received] PHST- 2021/07/14 00:00 [accepted] PHST- 2021/07/13 00:00 [revised] PHST- 2021/07/25 06:00 [pubmed] PHST- 2021/12/17 06:00 [medline] PHST- 2021/07/24 12:10 [entrez] AID - 10.1007/s12185-021-03194-8 [pii] AID - 10.1007/s12185-021-03194-8 [doi] PST - ppublish SO - Int J Hematol. 2021 Nov;114(5):620-625. doi: 10.1007/s12185-021-03194-8. Epub 2021 Jul 24. PMID- 41010002 OWN - NLM STAT- MEDLINE DCOM- 20250927 LR - 20250930 IS - 2073-4425 (Electronic) IS - 2073-4425 (Linking) VI - 16 IP - 9 DP - 2025 Sep 9 TI - Angiogenic microRNAs in Systemic Sclerosis: Insights into Microvascular Dysfunction and Therapeutic Implications. LID - 10.3390/genes16091057 [doi] LID - 1057 AB - Systemic sclerosis (SSc) is a complex connective tissue disease that affects the skin and internal organs and is characterized by immune dysregulation, progressive fibrosis, and microvascular dysfunction. Chronic tissue ischemia, accompanied by impaired angiogenesis, leads to the gradual loss of small vessels, resulting in clinical complications, such as Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, and renal crisis. Emerging evidence highlights the crucial regulatory role of microRNAs (miRNAs) in vascular homeostasis through the modulation of key signaling pathways and endothelial cell activity. Dysregulated miRNAs influence fibroblast proliferation, inflammatory responses, and immune cell activity in SSc, contributing to disease progression. Current knowledge is still limited, highlighting the need for further research to elucidate the miRNAs network involved in the etiopathogenesis of SSc. The use of miRNA-based biomarkers is gaining tremendous attention for early diagnosis, risk stratification, classification, and the prediction of therapeutic responses. This review provides insights into angiogenesis-related miRNAs involved in SSc pathogenesis, discusses their relevance as biomarkers, and explores their promise as therapeutic targets. Advancing our knowledge of miRNAs-mediated regulatory networks may open new possibilities for personalized approaches to SSc management. FAU - Rusek, Marta AU - Rusek M AUID- ORCID: 0000-0001-9592-2478 AD - Independent Unit of Radiopharmacy, Department of Organic Chemistry, Faculty of Pharmacy, Medical University, 4a Chodźki Street, 20-093 Lublin, Poland. LA - eng PT - Journal Article PT - Review DEP - 20250909 PL - Switzerland TA - Genes (Basel) JT - Genes JID - 101551097 RN - 0 (MicroRNAs) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Scleroderma, Systemic/genetics/pathology/therapy/metabolism MH - *MicroRNAs/genetics/metabolism MH - *Neovascularization, Pathologic/genetics MH - Biomarkers/metabolism MH - *Microvessels/metabolism/pathology/physiopathology MH - Animals PMC - PMC12469577 OTO - NOTNLM OT - angiogenesis OT - biomarkers OT - endothelial dysfunction OT - epigenetics OT - fibrosis OT - microRNAs OT - systemic sclerosis OT - therapeutic targets COIS- The author declares no conflicts of interest. EDAT- 2025/09/27 06:33 MHDA- 2025/09/27 06:34 PMCR- 2025/09/09 CRDT- 2025/09/27 01:15 PHST- 2025/07/25 00:00 [received] PHST- 2025/08/25 00:00 [revised] PHST- 2025/08/28 00:00 [accepted] PHST- 2025/09/27 06:34 [medline] PHST- 2025/09/27 06:33 [pubmed] PHST- 2025/09/27 01:15 [entrez] PHST- 2025/09/09 00:00 [pmc-release] AID - genes16091057 [pii] AID - genes-16-01057 [pii] AID - 10.3390/genes16091057 [doi] PST - epublish SO - Genes (Basel). 2025 Sep 9;16(9):1057. doi: 10.3390/genes16091057. PMID- 20691050 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 4 DP - 2010 Aug 6 TI - Fatal cold agglutinin-induced haemolytic anaemia: a case report. PG - 252 LID - 10.1186/1752-1947-4-252 [doi] AB - INTRODUCTION: Cold agglutinin disease usually develops as a result of the production of a specific immunoglobulin M auto-antibody directed against the I/i and H antigens, precursors of the ABH and Lewis blood group substances, on red blood cells. Autoimmune and lymphoproliferative disorders, Mycoplasma pneumoniae and other infections can be associated with the production of cold agglutinins. In its classic presentation with haemolytic anaemia and Raynaud's syndrome, cold agglutinin disease is usually idiopathic. Several factors play a role in determining the ability of a cold agglutinin to induce a haemolytic anaemia such as antibody concentration and temperature range, in particular the highest temperature at which antibodies interact with red blood cells. CASE PRESENTATION: A 48-year-old Caucasian man presented to our hospital with symptoms of extreme asthenia caused by severe anaemia. The transfusion of red blood cells (O Rh-positive), started as prescribed by the emergency guidelines in force without pre-transfusion tests, induced fatal haemolysis because of the presence of high levels of anti-H antibodies in his blood, that reacted with the large amount of H antigen in universal (0) red blood cells. CONCLUSION: Emergency transfusion of universal red blood cells (0 Rh-positive or negative) is usually accepted by the international guidelines in force in emergency departments. In this report we describe a rare complication caused by the very high concentration in the recipient of cold agglutinins and the activation of the complement system, responsible for red blood cell lysis and consequent fatal cardiovascular shock. We conclude that emergency transfusion of universal red blood cells (0 Rh-positive or negative) may be dangerous and its risk should be assessed against the risk of delaying transfusion until the pre-transfusion tests are completed. FAU - Lodi, Gianluca AU - Lodi G AD - Blood Transfusion Service - Arcispedale S, Anna, 203 C,so Giovecca, 44100 Ferrara - Italy. sitfe@ospfe.it. FAU - Resca, Daniela AU - Resca D FAU - Reverberi, Roberto AU - Reverberi R LA - eng PT - Journal Article DEP - 20100806 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 PMC - PMC2923177 EDAT- 2010/08/10 06:00 MHDA- 2010/08/10 06:01 PMCR- 2010/08/06 CRDT- 2010/08/10 06:00 PHST- 2009/10/21 00:00 [received] PHST- 2010/08/06 00:00 [accepted] PHST- 2010/08/10 06:00 [entrez] PHST- 2010/08/10 06:00 [pubmed] PHST- 2010/08/10 06:01 [medline] PHST- 2010/08/06 00:00 [pmc-release] AID - 1752-1947-4-252 [pii] AID - 10.1186/1752-1947-4-252 [doi] PST - epublish SO - J Med Case Rep. 2010 Aug 6;4:252. doi: 10.1186/1752-1947-4-252. PMID- 30755166 OWN - NLM STAT- MEDLINE DCOM- 20190313 LR - 20200225 IS - 1471-2377 (Electronic) IS - 1471-2377 (Linking) VI - 19 IP - 1 DP - 2019 Feb 12 TI - Mixed cerebrovascular disease in an elderly patient with mixed vascular risk factors: a case report. PG - 26 LID - 10.1186/s12883-019-1248-z [doi] LID - 26 AB - BACKGROUND: Mixed cerebrovascular disease is a diagnostic entity that presents with hemorrhagic and ischemic stroke clinically and/or subclinically. Here, we report a patient with mixed vascular risk factors, who presented with multiple intracerebral hemorrhages and a simultaneously occurring cerebral infarction with hemorrhagic transformation. CASE PRESENTATION: A 63-year-old male with no history of trauma or prior neurological disease presented with a sudden onset of weakness in his right limbs, followed by an episode of focal seizure without impaired awareness. The patient had a 4-year history of deep vein thrombosis in the lower limbs, and a 2-year history of Raynaud's phenomenon in the hands. He also had a family history of hypertension and thrombophilia. Head computed tomography plain scans showed two high densities in the bilateral parietal lobes and one mixed density in the left frontal lobe. The patient was diagnosed with mixed cerebrovascular disease. In this report, we make a systematic clinical reasoning regarding the etiological diagnosis, and discuss the possible pathogenic mechanisms leading to mixed cerebrovascular disease. We exclude coagulopathy, endocarditis, atrial fibrillation, patent foramen ovale, brain tumor, cerebral venous thrombosis, cerebral vascular malformation, cerebral amyloid angiopathy and vasculitis as causative factors. We identify hypertension, hereditary protein S deficiency, hypercholesteremia and hyperhomocysteinemia as contributing etiologies in this case. CONCLUSION: This case presents complex underlying mechanisms of mixed cerebrovascular disease, in which hypertension and hyperhomocysteinemia are considered to play a central role. FAU - He, Dian AU - He D AD - Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Yu, YunLi AU - Yu Y AD - Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Wu, Shan AU - Wu S AD - Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Tian, ShuFen AU - Tian S AD - Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Yu, Hui AU - Yu H AD - Department of Radiology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Xu, Shu AU - Xu S AD - Department of Pathology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. FAU - Chu, Lan AU - Chu L AD - Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China. chulan8999@126.com. LA - eng GR - (2017) 5-5/The Department of Science and Technology of Guiyang City, Guizhou Province, China/ GR - (2014) 6008/The Department of Science and Technology of Guizhou Province, China/ PT - Case Reports PT - Journal Article DEP - 20190212 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 SB - IM MH - Brain Ischemia/etiology MH - Cerebral Hemorrhage/etiology MH - Humans MH - Hypercholesterolemia/*complications MH - Hyperhomocysteinemia/*complications MH - Hypertension/*complications MH - Male MH - Middle Aged MH - Protein S Deficiency/*complications MH - Stroke/*etiology PMC - PMC6371517 COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Not applicable. CONSENT FOR PUBLICATION: Written informed consent was obtained from the patient’s daughter for publication of this case report and any accompanying images. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/02/14 06:00 MHDA- 2019/03/14 06:00 PMCR- 2019/02/12 CRDT- 2019/02/14 06:00 PHST- 2018/09/24 00:00 [received] PHST- 2019/01/31 00:00 [accepted] PHST- 2019/02/14 06:00 [entrez] PHST- 2019/02/14 06:00 [pubmed] PHST- 2019/03/14 06:00 [medline] PHST- 2019/02/12 00:00 [pmc-release] AID - 10.1186/s12883-019-1248-z [pii] AID - 1248 [pii] AID - 10.1186/s12883-019-1248-z [doi] PST - epublish SO - BMC Neurol. 2019 Feb 12;19(1):26. doi: 10.1186/s12883-019-1248-z. PMID- 41033929 OWN - NLM STAT- MEDLINE DCOM- 20251215 LR - 20251215 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 39 IP - 4 DP - 2025 Dec TI - Novel therapies in SSc. PG - 102104 LID - S1521-6942(25)00072-5 [pii] LID - 10.1016/j.berh.2025.102104 [doi] AB - Systemic sclerosis (SSc) is an autoimmune connective tissue disease that is rare and targets fibrosis and vasculopathy with autoantibodies present. There are guidelines in SSc that are evidence based and can facilitate appropriate treatment for many patients with SSc. However, there is no cure and in many patients the quality and quantity of life are significantly affected. Thus, novel therapies in SSc are warranted in order to try to prevent damage and increase quality of life and improve survival. This update will expand where the standard of care treatment in SSc may be enhanced by novel therapies and recent or ongoing trials. As few studies of Raynaud's phenomenon, digital ulcers, gastrointestinal, cardiac, renal and musculoskeletal systems in SSc are ongoing, they will not be included. This paper will concentrate on immune modification for skin involvement and interstitial lung disease and pulmonary hypertension. Potentially transformative treatments will be highlighted and where they may fit into a future improved standard of care for SSc patients. CI - Copyright © 2025. Published by Elsevier Ltd. FAU - Pope, Janet AU - Pope J AD - Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada; Division of Rheumatology, Department of Medicine, St. Joseph's Health Care, 268 Grosvenor St., London, ON, N6A 4V2, Canada. Electronic address: janet.pope@sjhc.london.on.ca. LA - eng PT - Journal Article DEP - 20250930 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/immunology/therapy MH - Lung Diseases, Interstitial/etiology/immunology/therapy MH - Hypertension, Pulmonary/etiology/immunology/therapy OTO - NOTNLM OT - BiTE OT - CAR-T OT - Clinical trials OT - Diffuse OT - ILD OT - Limited OT - Novel therapies OT - PAH OT - Progressive pulmonary fibrosis OT - Pulmonary arterial hypertension OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/10/02 00:29 MHDA- 2025/12/13 00:32 CRDT- 2025/10/01 22:00 PHST- 2025/09/04 00:00 [received] PHST- 2025/09/23 00:00 [accepted] PHST- 2025/12/13 00:32 [medline] PHST- 2025/10/02 00:29 [pubmed] PHST- 2025/10/01 22:00 [entrez] AID - S1521-6942(25)00072-5 [pii] AID - 10.1016/j.berh.2025.102104 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2025 Dec;39(4):102104. doi: 10.1016/j.berh.2025.102104. Epub 2025 Sep 30. PMID- 26677892 OWN - NLM STAT- MEDLINE DCOM- 20170220 LR - 20170817 IS - 1437-4331 (Electronic) IS - 1434-6621 (Linking) VI - 54 IP - 8 DP - 2016 Aug 1 TI - Comparison of the clinical utility of the Elia CTD Screen to indirect immunofluorescence on Hep-2 cells. PG - 1365-70 LID - 10.1515/cclm-2015-1051 [doi] AB - BACKGROUND: We compared the Elia CTD Screen (ECS), a fluoroenzymeimmunoassay incorporating 17 human antinuclear antigens (ANA), with indirect immunofluorescence (IIF) on Hep-2 cells in order to determine the clinical utility of the ECS in additon to or without IIF. METHODS: We examined 1708 consecutive serum samples submitted for ANA testing using the ECS and IIF in parallel. Positive screen results were further examined by quantitative fluoroenzymeimmunoassays and/or immunoblots for antibody identification. The medical records were evaluated for systemic rheumatic disorders. RESULTS: Concordance between ECS and IIF was observed in 1344 (78.8%) samples. ECS had a better detection rate for anti-dsDNA, -SSA/Ro, -SSB/La, -U1RNP and -Jo-1 antibodies, whereas IIF was superior in the detection of anti-CENP-B antibodies as well as anti-histone, -nucleosome and -Pl-12 antibodies, which are not included in the ECS antigen panel. ECS had a 100% sensitivity for Sjögren's syndrome, systemic sclerosis and Sharp syndrome. The sensitivity for Sjögren's syndrome was slightly higher for ESC than for IIF (94%). IIF had a higher diagnostic sensitivity for systemic lupus erythematosus, indeterminated connective tissue disease, Raynaud's syndrome and limited scleroderma, compared to ESC (100% vs. 80%, 100 vs. 75%, 89 vs. 57%, 100 vs. 88.9%). CONCLUSIONS: Our results suggest that the ECS represents an appropriate diagnostic tool for ANA screening. However, since some antigens are not incorporated in the ECS panel, and some ANA can also be missed by IIF, sequential or parallel screening with ECS and IIF may be reasonable when the clinical suspicion for connective tissue disease is high. FAU - Robier, Christoph AU - Robier C FAU - Amouzadeh-Ghadikolai, Omid AU - Amouzadeh-Ghadikolai O FAU - Stettin, Mariana AU - Stettin M FAU - Reicht, Gerhard AU - Reicht G LA - eng PT - Comparative Study PT - Journal Article PL - Germany TA - Clin Chem Lab Med JT - Clinical chemistry and laboratory medicine JID - 9806306 RN - 0 (Antigens, Nuclear) SB - IM MH - Antigens, Nuclear/*blood/immunology MH - *Fluorescent Antibody Technique, Indirect MH - Hep G2 Cells MH - Humans MH - *Immunoenzyme Techniques EDAT- 2015/12/19 06:00 MHDA- 2017/08/18 06:00 CRDT- 2015/12/19 06:00 PHST- 2015/10/28 00:00 [received] PHST- 2015/11/11 00:00 [accepted] PHST- 2015/12/19 06:00 [entrez] PHST- 2015/12/19 06:00 [pubmed] PHST- 2017/08/18 06:00 [medline] AID - /j/cclm.ahead-of-print/cclm-2015-1051/cclm-2015-1051.xml [pii] AID - 10.1515/cclm-2015-1051 [doi] PST - ppublish SO - Clin Chem Lab Med. 2016 Aug 1;54(8):1365-70. doi: 10.1515/cclm-2015-1051. PMID- 17704920 OWN - NLM STAT- MEDLINE DCOM- 20080717 LR - 20181113 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 28 IP - 4 DP - 2008 Feb TI - Iloprost infusion does not reduce oxidative stress in systemic sclerosis. PG - 335-7 AB - Systemic sclerosis is a connective tissue disease in which oxidative stress represents an important player among the complex pathogenetic mechanisms of the disease. Iloprost, an analogue of natural prostacyclin, is used in systemic sclerosis for the treatment of severe Raynaud's phenomenon and ischemic ulcers. There is a clear evidence that iloprost attenuates oxidative damage induced by ischemia-reperfusion phenomena. The aim of this study is to evaluate the effect of iloprost on oxidative status in ten patients with systemic sclerosis by measuring urinary levels of 8-isoprostaglandin-F(2alpha), a member of F(2)-isoprostanes. We found that systemic sclerosis patients cyclically treated with iloprost showed increased urinary level of 8-isoprostaglandin-F(2alpha )in comparison with healthy subjects; urinary 8-isoprostaglandin-F(2alpha) did not diminish soon after the iloprost infusion as well as 3, 15 and 30 days after the drug administration. Unlike experimental studies, in vivo the strong vasodilator effect of iloprost infusion did not reduce oxidative status. FAU - Volpe, Alessandro AU - Volpe A AD - Department of Clinical and Experimental Medicine, University of Verona, P.le LA Scuro 10, 37134, Verona, Italy. reumatologia@sacrocuore.it FAU - Biasi, Domenico AU - Biasi D FAU - Caramaschi, Paola AU - Caramaschi P FAU - Bambara, Lisa Maria AU - Bambara LM FAU - Carletto, Antonio AU - Carletto A FAU - Degan, Maurizio AU - Degan M FAU - Minuz, Pietro AU - Minuz P LA - eng PT - Clinical Trial PT - Journal Article DEP - 20070818 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Vasodilator Agents) RN - 27415-26-5 (8-epi-prostaglandin F2alpha) RN - B7IN85G1HY (Dinoprost) RN - JED5K35YGL (Iloprost) SB - IM MH - Dinoprost/analogs & derivatives/urine MH - Female MH - Humans MH - Iloprost/*administration & dosage MH - Infusions, Parenteral MH - Male MH - Middle Aged MH - Oxidative Stress/*drug effects MH - Scleroderma, Systemic/*drug therapy/metabolism MH - Time Factors MH - Treatment Outcome MH - Up-Regulation MH - Vasodilator Agents/*administration & dosage EDAT- 2007/08/21 09:00 MHDA- 2008/07/18 09:00 CRDT- 2007/08/21 09:00 PHST- 2007/04/15 00:00 [received] PHST- 2007/07/30 00:00 [accepted] PHST- 2007/08/21 09:00 [pubmed] PHST- 2008/07/18 09:00 [medline] PHST- 2007/08/21 09:00 [entrez] AID - 10.1007/s00296-007-0443-1 [doi] PST - ppublish SO - Rheumatol Int. 2008 Feb;28(4):335-7. doi: 10.1007/s00296-007-0443-1. Epub 2007 Aug 18. PMID- 22960740 OWN - NLM STAT- MEDLINE DCOM- 20130213 LR - 20151119 IS - 2567-5761 (Electronic) IS - 0720-9355 (Linking) VI - 32 Suppl 1 DP - 2012 TI - [Protein Z deficiency in unexplained affinity to thromboses, bleedings or abortions]. PG - S95-7 AB - A protein Z deficiency is presumably related with a threefold risk of venous and arterial thrombosis. Mucosal bleedings and post-operative haematomas can occur more frequently. This is seen in an increased in vivo bleeding time without other plasmatic coagulation disorders or thrombopathies. Pregnancy complications, especially abortions before the 15th week of gestation, are described as well. PATIENTS, METHODS: Since May 2011 the plasmatic concentration of protein Z has been tested in 684 patients of the Hämostaseologicum. RESULTS: In 74 patients a protein Z deficiency has been found. In other 45 patients protein Z was reduced because of the intake of phenprocoumon or coumadin. Of the 74 patients with diminished protein Z concentration 39 were marginally decreased (protein Z 1000-1500 µg/l). Of the 35 patients with a protein Z concentration <1000 µg/l 12 had had a thrombosis before (6 strokes, 3 DVT or PE, 1 arterial thrombosis, 1 retinal branch vein occlusion, 1 acute hearing loss). 7 had arterial hypertension, 2 suffered from diabetes mellitus. Of the patients who had a thrombosis 6 had a heterozygous factor V Leiden mutation. 10 had a microcirculation disorder (Raynaud's phenomenon), 4 had had bleeding complications before, 3 had a von Willebrand disease type I, 6 patients had had abortions and 4 were healthy. Of the 39 patients with protein Z concentrations between 1000 and 1500 µg/l 18 had experienced a thrombosis before (9 DVT or PE, 3 myocardial infarctions, 1 CHD, 3 strokes, 1 retinal branch vein occlusion, 1 PAOD I, 1 tinnitus). 5 additionally had arterial hypertension. 13 suffered from Raynaud's phenomenon, of which 7 had a hypotension. Of the patients with thromboses 3 had a heterozygous factor V Leiden mutation and one a protein C deficiency. 7 patients had had an abortion before. Bleeding complications were seen in 4 patients, of which 3 suffered from von Willebrand disease type 1. FAU - Kiesewetter, H AU - Kiesewetter H AD - Haemostaseologicum MVZ GbR Berlin, Berlin. kiesewetter@haemostaseologicum.com FAU - Radtke, H AU - Radtke H FAU - Jainz, A AU - Jainz A FAU - Schmidt, F-P AU - Schmidt FP LA - ger PT - English Abstract PT - Journal Article TT - Protein-Z-Mangel bei ungeklärter Neigung zu Thrombosen, Blutungen oder Aborten. PL - Germany TA - Hamostaseologie JT - Hamostaseologie JID - 8204531 RN - 0 (Biomarkers) RN - 0 (Blood Proteins) RN - 0 (plasma protein Z) SB - IM MH - Abortion, Habitual/blood/*epidemiology/prevention & control MH - Adolescent MH - Adult MH - Biomarkers/blood MH - Blood Proteins/*analysis/*deficiency MH - Comorbidity MH - Female MH - Germany/epidemiology MH - Hemorrhage/blood/*epidemiology/prevention & control MH - Humans MH - Pregnancy MH - Pregnancy Complications, Hematologic/blood/*epidemiology/prevention & control MH - Risk Assessment MH - Risk Factors MH - Thrombosis/blood/*epidemiology/prevention & control MH - Young Adult EDAT- 2012/09/14 06:00 MHDA- 2013/02/14 06:00 CRDT- 2012/09/11 06:00 PHST- 2012/02/01 00:00 [received] PHST- 2012/07/02 00:00 [accepted] PHST- 2012/09/11 06:00 [entrez] PHST- 2012/09/14 06:00 [pubmed] PHST- 2013/02/14 06:00 [medline] AID - 12410S95 [pii] PST - ppublish SO - Hamostaseologie. 2012;32 Suppl 1:S95-7. PMID- 37105929 OWN - NLM STAT- MEDLINE DCOM- 20230501 LR - 20230513 IS - 1944-7930 (Electronic) IS - 1539-6509 (Linking) VI - 35 IP - 175 DP - 2023 Apr 1 TI - Clinical Features and Risk Factors of Rapidly Progressive Systemic Sclerosis in a Single Center in China: Anti-RNA Polymerase III Antibodies as a Predictor. PG - 193-200 LID - 10.24976/Discov.Med.202335175.20 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) have been classified in two clinical subsets (diffuse and limited) based on the extend of skin thickening. In this study, we classified a novel subset of SSc defined rapidly progressive systemic sclerosis (RPSSc), which based on the rate of skin thickening progression and the progressive of interstitial lung disease (ILD). We aimed to evaluate RPSSc clinical characteristics and predictive factors in a Chinese single center. METHOD: Overall, 75 patients diagnosed with SSc, classified into RPSSc (n = 14) and non-rapidly progressive SSc (non-RPSSc, n = 61) were retrospectively included in the study. Clinical characteristics, disease severity and autoantibodies were collected. Logistic regression, least absolute shrinkage, and selection operator (LASSO) regression analysis was used to identify RPSSc predictors. Receiver operating characteristic (ROC) analysis and Delong test was conducted to evaluate and compare different indexes. RESULTS: RPSSc rate was 18.7%. ILD (64.3%), cardiac involvement (42.9%) were the most common organ system involvement of RPSSc, while Raynaud's phenomenon incidence significantly decreased. Disease duration (12 vs 72, months), sex (42.9% vs 11.5%, male %), SSc subset (85.7% vs 27.9%, diffuse cutaneous SSc (dsSSc) %), modified Rodnan total skin score (mRSS) (20.5 vs 6), Raynaud's phenomenon (64.3% vs 98.4%), cardiac involvement (42.9% vs 18%), higher incidence with malignancy (28.6% vs 1.6%) and positive anti-RNA polymerase III antibodies (ARA) (64.3% vs 1.6%) were statistically significant differences among the RPSSc groups and non-RPSSc groups (p < 0.05). Univariate analysis showed that positive ARA, male, dsSSc and malignancy were RPSSc risk factors, while long-disease duration, Raynaud's phenomenon was RPSSc protective factors. ARA was the strongest factor associated to RPSSc (OR 108, 95% CI 11.287-1033.327, p < 0.001). LASSO logistic regression model identified six factors: Disease duration, dsSSc, malignancy, cardiac involvement, positivity of ARA were RPSSc risk factors, Raynaud's phenomenon was RPSSc protective factors. CONCLUSIONS: RPSSc is an SSc clinical category which should be accounted for early detection of organ involvement and close follow-up of malignancy. ARA might be used as a predictor for RPSSc and organ involvement. FAU - Yu, Qiuxia AU - Yu Q AD - Department of Rheumatology and Clinical Immunology, Ningbo Medical Center Lihuili Hospital, 315040 Ningbo, Zhejiang, China. AD - Department of Rheumatology and Clinical Immunology, The Affiliated Li Huili Hospital, Ningbo University, 315040 Ningbo, Zhejiang, China. FAU - Zhang, Jin AU - Zhang J AD - Department of Rheumatology and Clinical Immunology, Ningbo Medical Center Lihuili Hospital, 315040 Ningbo, Zhejiang, China. AD - Department of Rheumatology and Clinical Immunology, The Affiliated Li Huili Hospital, Ningbo University, 315040 Ningbo, Zhejiang, China. FAU - Fan, Liyi AU - Fan L AD - School of Medicine, Ningbo University, 315211 Ningbo, Zhejiang, China. FAU - Yu, Tianhang AU - Yu T AD - School of Medicine, Ningbo University, 315211 Ningbo, Zhejiang, China. FAU - Liu, Bingbing AU - Liu B AD - Department of Rheumatology and Clinical Immunology, Ningbo Medical Center Lihuili Hospital, 315040 Ningbo, Zhejiang, China. AD - Department of Rheumatology and Clinical Immunology, The Affiliated Li Huili Hospital, Ningbo University, 315040 Ningbo, Zhejiang, China. FAU - Ding, Jian AU - Ding J AD - Department of Rheumatology and Clinical Immunology, Ningbo Medical Center Lihuili Hospital, 315040 Ningbo, Zhejiang, China. AD - Department of Rheumatology and Clinical Immunology, The Affiliated Li Huili Hospital, Ningbo University, 315040 Ningbo, Zhejiang, China. LA - eng PT - Journal Article PL - United States TA - Discov Med JT - Discovery medicine JID - 101250006 RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Humans MH - Male MH - Female MH - *Scleroderma, Diffuse MH - RNA Polymerase III MH - Retrospective Studies MH - *Scleroderma, Systemic/diagnosis/epidemiology MH - Risk Factors MH - *Lung Diseases, Interstitial/diagnosis/epidemiology OTO - NOTNLM OT - anti-RNA polymerase III antibodies OT - clinical characteristics OT - malignancy OT - predictors OT - rapidly progressive systemic sclerosis EDAT- 2023/04/28 00:42 MHDA- 2023/05/01 06:42 CRDT- 2023/04/27 22:23 PHST- 2023/05/01 06:42 [medline] PHST- 2023/04/28 00:42 [pubmed] PHST- 2023/04/27 22:23 [entrez] AID - 1682407456917-2011043297 [pii] AID - 10.24976/Discov.Med.202335175.20 [doi] PST - ppublish SO - Discov Med. 2023 Apr 1;35(175):193-200. doi: 10.24976/Discov.Med.202335175.20. PMID- 36717930 OWN - NLM STAT- MEDLINE DCOM- 20230201 LR - 20230202 IS - 2047-783X (Electronic) IS - 0949-2321 (Print) IS - 0949-2321 (Linking) VI - 28 IP - 1 DP - 2023 Jan 30 TI - Analysis of clinical features and risk factors of peripheral neuropathy in patients with primary Sjögren's syndrome. PG - 54 LID - 10.1186/s40001-023-01013-w [doi] LID - 54 AB - OBJECTIVE: To observe the clinical features and efficacy of immunosuppressive therapy in patients with primary Sjögren's syndrome (PSS) combined with peripheral neuropathy (PN) syndrome and to explore the risk factors for PN in patients with PSS. METHODS: Sixty consecutive patients with PSS admitted to the Department of Rheumatology and Immunology, Wuhan No. 1 Hospital, from January 2014 to June 2020 were analysed retrospectively. Patients were divided into a PN group (N = 15) and a non-PN group (N = 45). The clinical characteristics of the two groups were compared, and the independent risk factors for PN combined with PSS were analysed by multivariate logistic regression. The patients with PSS combined with PN were followed up to observe the effect of immunosuppressive therapy. RESULTS: The patients with PN had a longer course of disease than those without PN (z = - 3.225, P = 0.001), and the incidence of Raynaud's phenomenon, anti-SSB antibody, rheumatoid factor and hyperglobulinaemia was higher (all P < 0.05) in patients with PN than in those without PN. Multivariate logistic regression analysis showed that hyperglobulinaemia, RF and anti-SSB antibodies were independent risk factors for PN with PSS (P < 0.05). Fourteen patients with PSS-PN were treated with immunosuppressants. The clinical symptoms of 10 patients were relieved, and mRS scores of 10 patients were decreased. CONCLUSION: PN is a common complication in PSS patients. Patients with PSS combined with PN have a longer course of disease and a significantly higher percentage of Raynaud's phenomenon, positive anti-SSB antibody, positive RF and hyperglobulinaemia. Immunosuppressive therapy was effective for partial remission of PN with PSS. CI - © 2023. The Author(s). FAU - Wu, Zhihong AU - Wu Z AD - Department of Rheumatology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. hmfl7606@126.com. FAU - Wang, Dong AU - Wang D AD - Department of Cardiology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. FAU - Chen, Lirong AU - Chen L AD - Department of Rheumatology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. FAU - Xianyu, Kaipu AU - Xianyu K AD - Department of Rheumatology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. FAU - Yang, Huiqing AU - Yang H AD - Department of Rheumatology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. LA - eng PT - Journal Article DEP - 20230130 PL - England TA - Eur J Med Res JT - European journal of medical research JID - 9517857 RN - 0 (Immunosuppressive Agents) SB - IM MH - Humans MH - *Sjogren's Syndrome/complications/diagnosis MH - Retrospective Studies MH - Risk Factors MH - Immunosuppressive Agents/therapeutic use MH - *Peripheral Nervous System Diseases/etiology/complications PMC - PMC9885688 OTO - NOTNLM OT - Immunosuppressive therapy OT - Peripheral neuropathy OT - Primary Sjögren's syndrome COIS- The authors declare that there are no competing interests. EDAT- 2023/02/01 06:00 MHDA- 2023/02/02 06:00 PMCR- 2023/01/30 CRDT- 2023/01/31 00:06 PHST- 2022/10/12 00:00 [received] PHST- 2023/01/12 00:00 [accepted] PHST- 2023/01/31 00:06 [entrez] PHST- 2023/02/01 06:00 [pubmed] PHST- 2023/02/02 06:00 [medline] PHST- 2023/01/30 00:00 [pmc-release] AID - 10.1186/s40001-023-01013-w [pii] AID - 1013 [pii] AID - 10.1186/s40001-023-01013-w [doi] PST - epublish SO - Eur J Med Res. 2023 Jan 30;28(1):54. doi: 10.1186/s40001-023-01013-w. PMID- 25846467 OWN - NLM STAT- MEDLINE DCOM- 20160506 LR - 20221207 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 142 IP - 5 DP - 2015 May TI - [Acute finger ischemia: A retrospective study of 13 patients]. PG - 332-9 LID - S0151-9638(15)00100-3 [pii] LID - 10.1016/j.annder.2015.02.018 [doi] AB - BACKGROUND: Acute ischemia of the upper limbs is rare in comparison with ischemia of the lower limbs. The origins of this condition are varied. GOALS: We retrospectively analyzed cases of acute finger ischemia (Raynaud's phenomena was excluded) in a dermatology department between 2008 and 2013 in order to evaluate the etiology and management of this phenomenon. RESULTS: Thirteen cases of finger ischemia were reported. The mean age was 54 years. Active smoking was noted in 11 cases. Ischemia was acute in 9 cases and subacute in 4 cases. The location was unilateral in 10 cases and bilateral in 2. Etiologies were: dysplasia of the palmar arch, antiphospholipid antibody syndrome, frostbite, distal arteritis linked to smoking, paraneoplastic arteritis, Buerger's disease, polyarteritis nodosa, stenosis of the subclavian artery, and 3 cases of embolic origin (ulnar, cardiac, and paraneoplastic aneurysm). In the acute phase, antiplatelets were given in 6 cases, anticoagulants in 10 cases and ilomedin in 6 cases. Sympathectomy was performed in 1 case and amputation in 2 cases. DISCUSSION: This study illustrates the diversity of etiologies of finger ischemia. The etiological test battery should be broad and include immunological and thrombophilia tests, arterial and cardiac investigations, cervical radiography and CT scan (screening for cancer). Close collaboration between dermatologists, hematologists, vascular surgeons and radiologists is essential for the management of these patients. CI - Copyright © 2015 Elsevier Masson SAS. All rights reserved. FAU - Marchal, A AU - Marchal A AD - Service de dermatologie et médecine vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Mahé, E AU - Mahé E AD - Service de dermatologie et médecine vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. Electronic address: emmanuel.mahe@ch-argenteuil.fr. FAU - Sin, C AU - Sin C AD - Service de dermatologie et médecine vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Bagan, P AU - Bagan P AD - Service de chirurgie thoracique et vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Bilan, P AU - Bilan P AD - Service de dermatologie et médecine vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Linder, J-F AU - Linder JF AD - Laboratoire d'explorations vasculaires, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Couffinhal, J-C AU - Couffinhal JC AD - Service de chirurgie thoracique et vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. FAU - Sigal, M-L AU - Sigal ML AD - Service de dermatologie et médecine vasculaire, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel-Prud'hon, 95107 Argenteuil cedex, France. LA - fre PT - English Abstract PT - Journal Article TT - Ischémie digitale aiguë : étude rétrospective de 13 cas. DEP - 20150403 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - 0 (Platelet Aggregation Inhibitors) SB - IM MH - Acute Disease MH - Adult MH - Aged MH - Aged, 80 and over MH - Amputation, Surgical MH - Antiphospholipid Syndrome MH - Arteritis/complications MH - Female MH - Fingers/*blood supply/surgery MH - Frostbite/complications MH - Humans MH - Ischemia/*etiology/therapy MH - Male MH - Middle Aged MH - Paraneoplastic Syndromes/complications MH - Platelet Aggregation Inhibitors/therapeutic use MH - Polyarteritis Nodosa/complications MH - Retrospective Studies MH - Smoking/adverse effects MH - Subclavian Steal Syndrome/complications MH - Sympathectomy MH - Thromboangiitis Obliterans/complications OTO - NOTNLM OT - Acute ischemia OT - Atheroma OT - Athérome OT - Ischémie aiguë OT - Membre supérieur OT - Paraneoplastic syndrome OT - Syndrome paranéoplasique OT - Thrombophilia OT - Thrombophilie OT - Upper limb EDAT- 2015/04/08 06:00 MHDA- 2016/05/07 06:00 CRDT- 2015/04/08 06:00 PHST- 2013/12/20 00:00 [received] PHST- 2014/12/30 00:00 [revised] PHST- 2015/02/04 00:00 [accepted] PHST- 2015/04/08 06:00 [entrez] PHST- 2015/04/08 06:00 [pubmed] PHST- 2016/05/07 06:00 [medline] AID - S0151-9638(15)00100-3 [pii] AID - 10.1016/j.annder.2015.02.018 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2015 May;142(5):332-9. doi: 10.1016/j.annder.2015.02.018. Epub 2015 Apr 3. PMID- 34290330 OWN - NLM STAT- MEDLINE DCOM- 20211117 LR - 20211117 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 11 IP - 1 DP - 2021 Jul 21 TI - The prevalence and clinical features associated of hypothyroidism among Thai systemic sclerosis patients. PG - 14902 LID - 10.1038/s41598-021-94371-6 [doi] LID - 14902 AB - Thyroid disease, particularly hypothyroidism, has been reported in systemic sclerosis (SSc). Some clinical features of SSc can also present in hypothyroidism. Our aims were to determine the prevalence of, and describe clinical features associated with, hypothyroidism in SSc patients. We conducted a historical cohort study of adult SSc patients who underwent screening thyroid function tests at the Scleroderma Clinic, Khon Kaen University, Thailand, between 2009 and 2018. The patients who had any thyroid disorders before the onset of SSc and were diagnosed as an overlap syndrome were excluded. A total of 200 SSc were included according to sample size calculation, among whom the female to male ratio was 2:1. The majority of cases (137; 69.5%) were diffuse cutaneous SSc subset. The mean age was 55.8 ± 10.7 years and the median duration of disease 4.9 (IQR 1.6-9.9) years. Of the total, 9 had primary hypothyroidism (prevalence 4.5%; 95%CI 2.1-8.4) and 22 had subclinical hypothyroidism (prevalence 11%; 95%CI 7.0-16.2). Of the latter 22, 71% had dcSSc. Logistic regression analysis indicated that unexplained anemia was significantly associated with either subclinical hypothyroid or hypothyroidism (OR 2.74; 95% CI 1.17-6.47), whereas Raynaud's phenomenon had a negative association (OR 0.28; 95% CI 0.11-0.66). Neither severity of skin tightness nor internal organ involvement were associated with hypothyroidism among SSc patients. Clinical-subclinical hypothyroidism is uncommon among SSc patients, it is frequently associated with anemia, and less so Raynaud's phenomenon. Clinical-subclinical hypothyroidism should thus be considered in cases of unexplained anemia in SSc patients. CI - © 2021. The Author(s). FAU - Paolee, Yathao AU - Paolee Y AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. fching@kku.ac.th. FAU - Charoensri, Suranut AU - Charoensri S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Mayxay, Mayfong AU - Mayxay M AD - Lao-Oxford-Mahosot Hospital-Wellcome Trust-Research Unit, Mahosot Hospital, University of Health Sciences of Laos, Vientiane, Laos. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Nanagara, Ratanavadee AU - Nanagara R AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210721 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Aged MH - Anemia/etiology MH - Female MH - Humans MH - Hypothyroidism/epidemiology/*etiology MH - Male MH - Middle Aged MH - Prevalence MH - Scleroderma, Systemic/*complications/epidemiology MH - Thailand/epidemiology PMC - PMC8295267 COIS- The authors declare no competing interests. EDAT- 2021/07/23 06:00 MHDA- 2021/11/18 06:00 PMCR- 2021/07/21 CRDT- 2021/07/22 06:14 PHST- 2021/05/16 00:00 [received] PHST- 2021/07/09 00:00 [accepted] PHST- 2021/07/22 06:14 [entrez] PHST- 2021/07/23 06:00 [pubmed] PHST- 2021/11/18 06:00 [medline] PHST- 2021/07/21 00:00 [pmc-release] AID - 10.1038/s41598-021-94371-6 [pii] AID - 94371 [pii] AID - 10.1038/s41598-021-94371-6 [doi] PST - epublish SO - Sci Rep. 2021 Jul 21;11(1):14902. doi: 10.1038/s41598-021-94371-6. PMID- 39586183 OWN - NLM STAT- MEDLINE DCOM- 20250108 LR - 20260221 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 70 DP - 2025 Feb TI - The pathogenesis, diagnostic utility and clinical relevance of cutaneous telangiectasia in systemic sclerosis. PG - 152593 LID - S0049-0172(24)00233-6 [pii] LID - 10.1016/j.semarthrit.2024.152593 [doi] AB - Cutaneous telangiectasia (Tel) are visible permanently dilated postcapillary dermal venules and are one of the most common disease-specific manifestations of systemic sclerosis (SSc). Telangiectasia have long been recognised for their utility in the diagnosis and classification of SSc, but the clinical and prognostic relevance of these aberrant cutaneous vascular manifestations has been somewhat neglected by clinicians. Similarly, the impact of SSc-Tel on body image dissatisfaction and social discomfort has been under-appreciated. The paucity of evidence-based approaches to management has limited access to potential effective treatments for SSc-Tel. The present review examines the pathogenesis, diagnostic value, impact and clinical relevance of telangiectasia in SSc. We highlight the potentially overlooked prognostic value and clinical utility of SSc-Tel, as part of a broader appraisal of areas of unmet research need. CI - Copyright © 2024. Published by Elsevier Inc. FAU - Anilkumar, Aishwarya AU - Anilkumar A AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Wells, Matthew AU - Wells M AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Domsic, Robyn T AU - Domsic RT AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Hummers, Laura K AU - Hummers LK AD - Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Pauling, John D AU - Pauling JD AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK; Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. Electronic address: JohnPauling@nhs.net. LA - eng GR - K24 AR080217/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Review DEP - 20241117 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/diagnosis/physiopathology MH - *Telangiectasis/etiology/diagnosis MH - Prognosis MH - Skin/blood supply/pathology MH - Clinical Relevance PMC - PMC12922703 MID - NIHMS2049045 OTO - NOTNLM OT - Biomarker OT - Classification OT - Clinical outcome measures OT - Diagnosis OT - Prognosis OT - Raynaud's phenomenon OT - Systemic sclerosis OT - Telangiectasia OT - Treatment efficacy COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: JDP has undertaken consultancy work, received educational support and/or speaker honoraria from Janssen, Astra Zeneca, Boehringer-Ingelheim, CSL Vifor, IsoMab, Permeatus Inc, and Sojournix Pharma. LKH has received research or consulting support from Boehringer-Ingelheim, Cumberland Pharmaceuticals, CSH Behring, Mitsubishi Tanabe Pharma Corporation, Horizon Pharmaceuticals, Biotest. AAS has received research support for clinical trials from Arena Pharmaceuticals, Kadmon Corporation, Eicos Sciences and Medpace LLC. RTD has received support in the form of consulting fees from AstraZeneca, Aisa Pharma and CSL Behring. EDAT- 2024/11/26 00:16 MHDA- 2025/01/09 00:22 PMCR- 2026/02/20 CRDT- 2024/11/25 18:04 PHST- 2024/06/29 00:00 [received] PHST- 2024/10/21 00:00 [revised] PHST- 2024/10/28 00:00 [accepted] PHST- 2025/01/09 00:22 [medline] PHST- 2024/11/26 00:16 [pubmed] PHST- 2024/11/25 18:04 [entrez] PHST- 2026/02/20 00:00 [pmc-release] AID - S0049-0172(24)00233-6 [pii] AID - 10.1016/j.semarthrit.2024.152593 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2025 Feb;70:152593. doi: 10.1016/j.semarthrit.2024.152593. Epub 2024 Nov 17. PMID- 36161596 OWN - NLM STAT- MEDLINE DCOM- 20220928 LR - 20231223 IS - 1303-6165 (Electronic) IS - 1300-0144 (Print) IS - 1300-0144 (Linking) VI - 52 IP - 1 DP - 2022 Feb TI - Interstitial lung disease in patients with systemic lupus erythematosus: a cohort study. PG - 76-82 LID - 10.3906/sag-2109-16 [doi] AB - BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease with a variety of organ/system involvement. Respiratory system involvement is common in these patients and usually manifests itself by disorders of the lung parenchyma, pleura, pulmonary vasculature or diaphragm. In this study, we sought to determine the frequency of interstitial lung disease (ILD) in patients with SLE and associated risk factors. METHODS: Three hundred randomly chosen patients with SLE were included. Chest x-ray (CXR), lung spirometry and carbon monoxide diffusion test (DLCO) were performed. High-resolution thorax computed tomography (HRCT) was performed for a definite diagnosis of ILD. . RESULTS: Of 300 patients, 16% had ILD. At the start of the study, the prevalence obtained from the patients' records showed that 4% had ILD. The median age, mean duration of disease, and follow-up time were significantly higher and longer in patients with ILD compared to patients without (p < 0.05). Forced expiratory volume (FEV1), forced vital capacity (FVC), DLCO and total lung capacity (TLC) were significantly lower in patients with ILD (p < 0.001). Patients with ILD had a significantly higher frequency of arthritis, serositis, Raynaud's phenomenon, myositis, and anti-Scl70 positivity (p = 0.01, 0.001, 0.02, 0.004, and 0.001, respectively). A significantly higher number of patients had stopped using hydroxychloroquine (HCQ) in the ILD group (p = 0.04). FAU - Şenkal, Naci AU - Şenkal N AD - Department of Internal Medicine, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - Kıyan, Esen AU - Kıyan E AD - Department of Pulmonary Medicine, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - Demir, Ali Aslan AU - Demir AA AD - Fulya Radiologic Imaging Center, İstanbul, Turkey. FAU - Yalçınkaya, Yasemin AU - Yalçınkaya Y AD - Department of Internal Medicine, Division of Rheumatology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - Gül, Ahmet AU - Gül A AD - Department of Internal Medicine, Division of Rheumatology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - İnanç, Murat AU - İnanç M AD - Department of Internal Medicine, Division of Rheumatology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - Öçal, Mahmude Lale AU - Öçal ML AD - Department of Internal Medicine, Division of Rheumatology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. FAU - Esen, Bahar Artım AU - Esen BA AD - Department of Internal Medicine, Division of Rheumatology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Turkey. LA - eng PT - Journal Article DEP - 20220222 PL - Turkey TA - Turk J Med Sci JT - Turkish journal of medical sciences JID - 9441758 RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Carbon Monoxide MH - Cohort Studies MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Lung/diagnostic imaging MH - *Lung Diseases, Interstitial/complications/epidemiology MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - Retrospective Studies PMC - PMC10734877 OTO - NOTNLM OT - SLE OT - carbon monoxide diffusion test OT - fibrosis OT - interstitial lung disease OT - spirometry EDAT- 2022/09/27 06:00 MHDA- 2022/09/28 06:00 PMCR- 2021/12/21 CRDT- 2022/09/26 18:20 PHST- 2021/09/02 00:00 [received] PHST- 2021/12/21 00:00 [accepted] PHST- 2022/09/26 18:20 [entrez] PHST- 2022/09/27 06:00 [pubmed] PHST- 2022/09/28 06:00 [medline] PHST- 2021/12/21 00:00 [pmc-release] AID - turkjmedsci-52-1-76 [pii] AID - 10.3906/sag-2109-16 [doi] PST - ppublish SO - Turk J Med Sci. 2022 Feb;52(1):76-82. doi: 10.3906/sag-2109-16. Epub 2022 Feb 22. PMID- 24106519 OWN - NLM STAT- MEDLINE DCOM- 20140217 LR - 20211021 IS - 1740-2530 (Electronic) IS - 1740-2522 (Print) IS - 1740-2522 (Linking) VI - 2013 DP - 2013 TI - A clinical analysis of risk factors for interstitial lung disease in patients with idiopathic inflammatory myopathy. PG - 648570 LID - 10.1155/2013/648570 [doi] LID - 648570 AB - Interstitial lung disease (ILD) is a common and severe complication of idiopathic inflammatory myopathies (IIM). The aim of our study was to identify risk factors for ILD by evaluating both clinical and biochemical features in IIM patients with or without ILD. From January 2008 to December 2011, medical records of 134 IIM patients in our rheumatology unit were reviewed. The patients were divided into ILD group (83 patients) and non-ILD group (51 patients). The clinical features and laboratory findings were compared. The univariable analyses indicated that arthritis/arthralgia (54.2% versus 17.6%, P < 0.05), Mechanic's hand (16.9% versus 2.0%, P < 0.05), Raynaud's phenomenon (36.1% versus 2.0%, P < 0.05), heliotrope rash (44.6% versus 19.6%, P < 0.05), fever (43.4% versus 21.6%, P < 0.05), elevated ESR (60.2% versus 35.3%, P < 0.05), elevated CRP (55.4% versus 31.4%, P < 0.05), or anti-Jo-1 antibody (20.5% versus 5.9%, P < 0.05) were risk factors for developing ILD in IIM. Multivariable unconditional logistic regression analysis that showed arthritis/arthralgia (OR 7.1, 95% CI 2.8-18.1), Raynaud's phenomenon (OR 29.1, 95% CI 3.6-233.7), and amyopathic dermatomyositis (ADM) (OR 20.2, 95% CI 2.4-171.2) were the independent risk factors for developing ILD in IIM. FAU - Cen, Xiaomin AU - Cen X AD - Department of Rheumatology and Immunology, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, China. FAU - Zuo, Chuan AU - Zuo C FAU - Yang, Min AU - Yang M FAU - Yin, Geng AU - Yin G FAU - Xie, Qibing AU - Xie Q LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130909 PL - Egypt TA - Clin Dev Immunol JT - Clinical & developmental immunology JID - 101183692 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Lung Diseases, Interstitial/diagnosis/*etiology MH - Male MH - Middle Aged MH - Myositis/*complications MH - Odds Ratio MH - Risk Factors MH - Young Adult PMC - PMC3782818 EDAT- 2013/10/10 06:00 MHDA- 2014/02/18 06:00 PMCR- 2013/09/09 CRDT- 2013/10/10 06:00 PHST- 2013/05/02 00:00 [received] PHST- 2013/08/07 00:00 [accepted] PHST- 2013/10/10 06:00 [entrez] PHST- 2013/10/10 06:00 [pubmed] PHST- 2014/02/18 06:00 [medline] PHST- 2013/09/09 00:00 [pmc-release] AID - 10.1155/2013/648570 [doi] PST - ppublish SO - Clin Dev Immunol. 2013;2013:648570. doi: 10.1155/2013/648570. Epub 2013 Sep 9. PMID- 25559063 OWN - NLM STAT- MEDLINE DCOM- 20170503 LR - 20260128 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 55 IP - 3 DP - 2015 May-Jun TI - [Autoantibodies in systemic sclerosis and their clinical correlation in patients from a Midwestern region of Brazil]. PG - 229-39 LID - S0482-5004(14)00228-9 [pii] LID - 10.1016/j.rbr.2014.09.007 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a connective tissue disease of autoimmune nature characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. Autoantibodies do not seem to be simply epiphenomena, but are involved in disease pathogenesis. It is believed that the SSc-specific autoantibodies are responsible both for amplifying immune response and targeting cell types that are relevant in the pathophysiology of SSc. OBJECTIVES: To correlate the profile of the following specific autoantibodies: anti-centromere (ACA), anti-topoisomerase I (topo I) and anti-RNA polymerase III (RNAP III) with clinical and laboratory manifestations observed in 46 patients with SSc in the Midwest region of Brazil. METHODS: The occurrence of specific autoantibodies in 46 patients with SSc was investigated, correlating the type of autoantibody with clinical and laboratory manifestations found. RESULTS: Among all patients evaluated, we found a predominance of females (97.8%), mean age 50.21 years old, Caucasian (50%), limited cutaneous SSc (47.8%), time of diagnosis between 5-10 years (50%), and disease duration of 9.38 years. According to the specific autoantibody profile, 24 patients were ACA-positive (52.2%), 15 were positive for anti-topo I (32.6%), and 7 showed positive anti-RNAP III (15.2%). The anti-topo I autoantibody correlated with diffuse scleroderma, with greater disease severity and activity, with worse quality of life measured by the SHAQ index, with a higher prevalence of objective Raynaud's phenomenon and digital pitting scars of fingertips. The ACA correlated with limited scleroderma, with earlier onset of disease, as well as higher prevalence of telangiectasias. The anti- RNAP III correlated with diffuse scleroderma, with a higher occurrence of subjective Raynaud's phenomenon and muscle atrophy. There was no association between the positivity for anti-topo I, ACA and anti-RNAP III antibodies and other variables related to laboratory abnormalities, as well as Rodnan skin score and skin, vascular, musculoskeletal, gastrointestinal, cardiopulmonary and renal manifestations. CONCLUSIONS: The clinical subtype of the disease and some clinical manifestations in SSc may correlate positively with the presence of specific autoantibodies. CI - Copyright © 2014 Elsevier Editora Ltda. All rights reserved. FAU - Coelho Horimoto, Alex Magno AU - Coelho Horimoto AM AD - Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brasil. Electronic address: clinicaactivite@gmail.com. FAU - da Costa, Izaias Pereira AU - da Costa IP AD - Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brasil; Universidade de São Paulo, São Paulo, SP, Brasil. LA - por PT - Journal Article PT - Observational Study TT - Autoanticorpos em esclerose sistêmica e sua correlação com as manifestações clínicas da doença em pacientes do Centro-Oeste do Brasil. DEP - 20141107 PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/*blood MH - Brazil MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prospective Studies MH - Scleroderma, Systemic/*blood/*immunology OTO - NOTNLM OT - Anticentrômero OT - Antitopoisomerase I OT - Anti‐RNA polimerase III OT - Anti‐RNA polymerase III OT - Anti‐centromere OT - Anti‐topoisomerase I OT - Autoantibodies OT - Autoanticorpos OT - Esclerose sistêmica OT - Systemic sclerosis EDAT- 2015/01/07 06:00 MHDA- 2017/05/04 06:00 CRDT- 2015/01/07 06:00 PHST- 2014/03/28 00:00 [received] PHST- 2014/09/02 00:00 [revised] PHST- 2014/09/21 00:00 [accepted] PHST- 2015/01/07 06:00 [entrez] PHST- 2015/01/07 06:00 [pubmed] PHST- 2017/05/04 06:00 [medline] AID - S0482-5004(14)00228-9 [pii] AID - 10.1016/j.rbr.2014.09.007 [doi] PST - ppublish SO - Rev Bras Reumatol. 2015 May-Jun;55(3):229-39. doi: 10.1016/j.rbr.2014.09.007. Epub 2014 Nov 7. PMID- 34086073 OWN - NLM STAT- MEDLINE DCOM- 20220620 LR - 20220624 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 42 IP - 7 DP - 2022 Jul TI - Myopathy associated with anti-signal recognition particle antibodies with pulmonary involvement and response to rituximab. PG - 1265-1269 LID - 10.1007/s00296-021-04904-5 [doi] AB - The authors present the case of a 76-year-old female patient with progressive decrease in proximal muscle strength, fatigue, dyspnea, diffuse hand edema and painful triphasic Raynaud's phenomenon. Anti-SRP and anti-SSA antibodies were detected, muscle biopsy revealed changes consistent with necrotizing myopathy and capillaroscopy had findings compatible with systemic sclerosis. High-resolution chest computed tomography revealed interstitial lung disease with a non-specific interstitial pneumonia pattern. Lung function tests demonstrated a forced vital capacity 93% and a diffusing capacity for carbon monoxide of 65% predicted. After multidisciplinary discussion, she was diagnosed with immune-mediated necrotizing myopathy/systemic sclerosis overlap syndrome with pulmonary involvement. Initially, dual immunomodulation therapy with high-dose steroids and intravenous immunoglobulin was started, but after 4 weeks, the patient had clinical and analytical deterioration. At this time, she was started on rituximab, with an excellent and sustained response at both muscle and lung, sustained after 12 months. CI - © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Mazeda, Carolina AU - Mazeda C AUID- ORCID: 0000-0002-9200-5022 AD - Rheumatology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. carolina_m43@hotmail.com. FAU - Cunha, Rita AU - Cunha R AUID- ORCID: 0000-0002-3855-1129 AD - Rheumatology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. FAU - Ferreira, Pedro Gonçalo AU - Ferreira PG AUID- ORCID: 0000-0002-0438-8597 AD - Pneumology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. FAU - Barcelos, Anabela AU - Barcelos A AUID- ORCID: 0000-0002-4116-8964 AD - Rheumatology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. AD - NOVA National School of Public Health, Public Health Research Centre, NOVA University of Lisboa, Lisboa, Portugal. AD - Comprehensive Health Research Center, NOVA University of Lisboa, Lisboa, Portugal. FAU - Aguiar, Renata AU - Aguiar R AUID- ORCID: 0000-0002-1374-2738 AD - Rheumatology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210604 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Autoantibodies) RN - 0 (Signal Recognition Particle) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Aged MH - Autoantibodies MH - *Autoimmune Diseases/complications MH - Female MH - Humans MH - *Muscular Diseases/complications MH - *Myositis MH - Rituximab/therapeutic use MH - *Scleroderma, Systemic/complications MH - Signal Recognition Particle OTO - NOTNLM OT - Autoantibodies OT - Interstitial lung disease OT - Myositis OT - Signal recognition particle EDAT- 2021/06/05 06:00 MHDA- 2022/06/22 06:00 CRDT- 2021/06/04 12:20 PHST- 2021/03/09 00:00 [received] PHST- 2021/05/22 00:00 [accepted] PHST- 2021/06/05 06:00 [pubmed] PHST- 2022/06/22 06:00 [medline] PHST- 2021/06/04 12:20 [entrez] AID - 10.1007/s00296-021-04904-5 [pii] AID - 10.1007/s00296-021-04904-5 [doi] PST - ppublish SO - Rheumatol Int. 2022 Jul;42(7):1265-1269. doi: 10.1007/s00296-021-04904-5. Epub 2021 Jun 4. PMID- 25488380 OWN - NLM STAT- MEDLINE DCOM- 20160406 LR - 20170822 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 44 IP - 5 DP - 2015 Apr TI - Sparing effect of hemiplegia on skin fibrosis and microvascular involvement: reports of two cases of systemic sclerosis and review of the literature. PG - 597-601 LID - S0049-0172(14)00252-2 [pii] LID - 10.1016/j.semarthrit.2014.10.011 [doi] AB - OBJECTIVES: The sparing effect of hemiplegia in rheumatic diseases has been described, but reports on systemic sclerosis (SSc)-spectrum disorders are unusual. SSc-spectrum disorders are complex diseases of unknown origin characterized by multisystem involvement, skin and organ fibrosis, microvascular alterations, and immunologic abnormalities. We describe two cases of patients with hemiplegia who developed Raynaud׳s phenomenon and skin fibrosis of the non-paretic limb. METHODS: Clinical, laboratory, and investigation findings of two cases with hemiplegia who developed scleroderma spectrum disorders of the non-paretic limb are presented. A review of the medical literature was performed in PubMed for all articles in English. RESULTS: A total of 46 reports from 1935 to 2012 were identified, especially on osteoarthritis and rheumatoid arthritis. Only two case reports on patients with SSc describe asymmetric SSc skin involvement and unilateral acro-osteolysis on x-ray images of the non-paretic limb. By contrast, we report the first description of capillaroscopic microvascular changes in patients with hemiplegia and asymmetric SSc skin involvement. CONCLUSIONS: Our cases point out the potential role of a "cross-talk" between the nervous system and the skin in SSc-spectrum disorders and suggest future directions for research in studies of pathogenesis. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Ughi, Nicola AU - Ughi N AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. Electronic address: nicola.ughi@gpini.it. FAU - Hervey, Simon A AU - Hervey SA AD - Eastbourne District General Hospital, Eastbourne, East Sussex, UK. FAU - Gualtierotti, Roberta AU - Gualtierotti R AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. FAU - Silvana, Zeni AU - Silvana Z AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. FAU - Meroni, Pierluigi AU - Meroni P AD - Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20141029 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Female MH - Fibrosis/pathology MH - Hemiplegia/complications/*pathology MH - Humans MH - Middle Aged MH - Scleroderma, Systemic/complications/*pathology MH - Skin/*pathology MH - Skin Diseases/complications/*pathology OTO - NOTNLM OT - Connective tissue disease OT - Hemiplegia OT - Nailfold capillaroscopy OT - Scleroderma OT - Scleroderma spectrum disease OT - Systemic sclerosis EDAT- 2014/12/10 06:00 MHDA- 2016/04/07 06:00 CRDT- 2014/12/10 06:00 PHST- 2014/08/02 00:00 [received] PHST- 2014/10/13 00:00 [revised] PHST- 2014/10/24 00:00 [accepted] PHST- 2014/12/10 06:00 [entrez] PHST- 2014/12/10 06:00 [pubmed] PHST- 2016/04/07 06:00 [medline] AID - S0049-0172(14)00252-2 [pii] AID - 10.1016/j.semarthrit.2014.10.011 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2015 Apr;44(5):597-601. doi: 10.1016/j.semarthrit.2014.10.011. Epub 2014 Oct 29. PMID- 39210934 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240919 IS - 2050-0904 (Print) IS - 2050-0904 (Electronic) IS - 2050-0904 (Linking) VI - 12 IP - 9 DP - 2024 Sep TI - Anti-PL-12 anti-synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature. PG - e9408 LID - 10.1002/ccr3.9408 [doi] LID - e9408 AB - KEY CLINICAL MESSAGE: Acute digital ischemia is a rare manifestation of anti-synthetase syndrome in the absence of Raynaud's phenomenon. A high index of suspicion may result in early diagnosis and better clinical outcomes. ABSTRACT: A 61-year-old male patient was admitted to the hospital for worsening arthralgias with morning stiffness lasting hours, as well as left sided headaches, and jaw pain while eating. He had significant weight loss and subjective fever at home. Multiple fingers and toes were noted to be ischemic. His laboratory workup was pertinent for significantly elevated inflammatory markers, and mild Creatinine kinase elevation. Chest imaging and later lung biopsy were notable for organizing pneumonia. Conventional angiogram showed evidence of significant digital disease without collaterals. Subsequent autoimmune screening tests with extended myositis-specific and myositis-associated panels revealed a strongly positive anti-PL-12 antibody and moderately positive anti- SSA-52KD IgG ab. After ruling out infectious etiologies and malignancy, anti-synthetase syndrome (ASyS) diagnosis was considered in the presence of ischemic digits, organizing pneumonia, polyarthralgia, constitutional symptoms, increased inflammatory markers and positive antibodies. The patient was treated with high dose prednisone and mycophenolate mofetil along with amlodipine and sildenafil for digital vasodilation. Acute digital ischemia may be the first manifestation of ASyS with ILD. A high index of suspicion is warranted for early diagnosis and better outcomes. CI - © 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. FAU - Doumeth, Sarah Abi AU - Doumeth SA AUID- ORCID: 0000-0003-1914-7400 AD - Department of Rheumatology Case Western Reserve University Hospitals Cleveland Ohio USA. AD - Department of Medicine Case Western Reserve University Hospitals Cleveland Ohio USA. FAU - Petrinec, Emily AU - Petrinec E AD - Department of Medicine Case Western Reserve University Hospitals Cleveland Ohio USA. FAU - Chaudhary, Haseeb AU - Chaudhary H AD - Department of Rheumatology Case Western Reserve University Hospitals Cleveland Ohio USA. AD - Department of Medicine Case Western Reserve University Hospitals Cleveland Ohio USA. FAU - Mattar, Maya AU - Mattar M AD - Department of Rheumatology Case Western Reserve University Hospitals Cleveland Ohio USA. AD - Department of Rheumatology Louis Stokes VA Medical Center Cleveland Ohio USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240828 PL - England TA - Clin Case Rep JT - Clinical case reports JID - 101620385 EIN - Clin Case Rep. 2024 Sep 17;12(9):e9456. doi: 10.1002/ccr3.9456. PMID: 39296473 PMC - PMC11358210 OTO - NOTNLM OT - anti‐PL12 antibody OT - anti‐synthetase syndrome OT - digital ischemia OT - immunosuppression OT - interstitial lung disease COIS- None. EDAT- 2024/09/01 16:15 MHDA- 2024/09/01 16:16 PMCR- 2024/08/28 CRDT- 2024/08/30 04:15 PHST- 2024/04/20 00:00 [received] PHST- 2024/07/24 00:00 [revised] PHST- 2024/08/12 00:00 [accepted] PHST- 2024/09/01 16:16 [medline] PHST- 2024/09/01 16:15 [pubmed] PHST- 2024/08/30 04:15 [entrez] PHST- 2024/08/28 00:00 [pmc-release] AID - CCR39408 [pii] AID - 10.1002/ccr3.9408 [doi] PST - epublish SO - Clin Case Rep. 2024 Aug 28;12(9):e9408. doi: 10.1002/ccr3.9408. eCollection 2024 Sep. PMID- 32126935 OWN - NLM STAT- MEDLINE DCOM- 20220317 LR - 20220317 IS - 2376-9130 (Electronic) IS - 1080-3548 (Linking) VI - 28 IP - 1 DP - 2022 Mar TI - Chainsaw operators' exposure to occupational risk factors and incidence of professional diseases specific to the forestry field. PG - 8-19 LID - 10.1080/10803548.2019.1703336 [doi] AB - Purpose. This article focuses on detailed studies regarding the analysis of occupational risk factors on health and occupational disease, namely, the influence of noise, hand-arm vibration, wet bulb globe temperature (WBGT) index and exposure to particulates. Methods. This study measured the equivalent acoustic level (LA(eq)), daily vibration exposure (A(8)), WBGT index and particulate concentration in the respirable area of the worker. The inferential analysis consisted of the application of specific statistical methods: a probability plot with 95% confidence interval, the Anderson-Darling statistic and 87th percentile estimation. A sample of 107 chainsaw operators was medically evaluated, out of which 30 workers were suspected of having professional pathologies and were hospitalized in the university clinic. Results. The measurements highlight: exceeding the legal limit for noise exposure; 13% of cases exceeding the limit of 2.5 m/s(2) for hand-arm vibration; dust exposure generally within legal limits; WBGT shows the thermal stress of the workers. Following the medical evaluation, osteomusculoskeletal disorders (25.23%), Raynaud's syndrome (0.93%) and bilateral hearing loss (3.74%) were identified. Conclusions. Analysis of the levels of exposure to the risk factors, the typology and the incidence of occupational diseases requires the need to adopt new preventive measures. FAU - Iftime, Marius D AU - Iftime MD AD - Department of Forest Engineering, Forest Management Planning and Terrestrial Measurements, Transilvania University of Brasov, Romania. FAU - Dumitrascu, Adela-Eliza AU - Dumitrascu AE AUID- ORCID: 0000-0002-0272-9845 AD - Department of Manufacturing Engineering, Transilvania University of Brasov, Romania. FAU - Ciobanu, Valentina D AU - Ciobanu VD AD - Department of Forest Engineering, Forest Management Planning and Terrestrial Measurements, Transilvania University of Brasov, Romania. LA - eng PT - Journal Article DEP - 20200415 PL - England TA - Int J Occup Saf Ergon JT - International journal of occupational safety and ergonomics : JOSE JID - 9507598 SB - IM MH - Forestry MH - Humans MH - Incidence MH - *Occupational Diseases/epidemiology/etiology MH - *Occupational Exposure/adverse effects/prevention & control MH - Occupations MH - Risk Factors MH - Vibration/adverse effects OTO - NOTNLM OT - 87th percentile estimation OT - Anderson–Darling statistic OT - chainsaw operator OT - exposure to risk factor OT - occupational disease OT - probability plot OT - professional pathologies EDAT- 2020/03/05 06:00 MHDA- 2022/03/18 06:00 CRDT- 2020/03/05 06:00 PHST- 2020/03/05 06:00 [pubmed] PHST- 2022/03/18 06:00 [medline] PHST- 2020/03/05 06:00 [entrez] AID - 10.1080/10803548.2019.1703336 [doi] PST - ppublish SO - Int J Occup Saf Ergon. 2022 Mar;28(1):8-19. doi: 10.1080/10803548.2019.1703336. Epub 2020 Apr 15. PMID- 22127112 OWN - NLM STAT- MEDLINE DCOM- 20120403 LR - 20111130 IS - 1944-7930 (Electronic) IS - 1539-6509 (Linking) VI - 12 IP - 66 DP - 2011 Nov TI - Capillaroscopic findings in systemic sclerosis -- are they associated with disease duration and presence of digital ulcers? PG - 413-8 AB - PURPOSE OF THE STUDY: The aim of the study was to evaluate capillaroscopic pattern in systemic sclerosis (SSc) patients and its association with disease duration as well as with presence of digital ulcers. PATIENTS AND METHODS: Thirty six patients with SSc were included in the study. The severity of Raynaud's phenomenon (RP) at the hands was assessed with VAS (100mm), and the presence of digital ulcers at the hands was documented. Nailfold capillaroscopy was performed by a videocapillaroscope. RESULTS AND DISCUSSION: RP was found as a clinical symptom in 100% (36/36) of the examined SSc patients. In SSc patients with a duration of the disease of less than 3 years, an early phase "scleroderma type" capillaroscopic pattern was found in 50% (5/10) of the cases. In the group of SSc patients with a duration of the disease of more than 3 years, late phase scleroderma type capillaroscopic pattern was found in 26.9% (7/26) of the cases, which was characterized by the presence of extensive, "desert-like" avascular areas and neoangiogenic capillaries. Scleroderma type capillaroscopic pattern was found in 97.2% (35/36) of the cases. Digital ulcers at the hands were found in 36.1% (13/36) of the patients. In 100% of those patients with digital ulcers (13/13), an active type scleroderma like pattern was observed, which is characterized by the presence of frequent giant capillaries, hemorrhages, and avascular areas. An active type scleroderma like pattern was found in 47.2% (17/36) of the patients without digital ulcers. CONCLUSION: The data show that the presence of digital ulcers at the hands of SSc patients is strongly associated with an active type scleroderma like capillaroscopic pattern. Observation of an active type scleroderma like pattern in patients without digital ulcers may therefore be used as a predictor for the development of trophic changes in the future, an indication for vasoactive medication for the prevention of the development of digital ulcers, and as an additional objective method for the evaluation of disease activity score in SSc. FAU - Lambova, Sevdalina AU - Lambova S AD - Clinic of Rheumatology, Department for Propaedeutics of Internal Medicine, Medical University, Plovdiv, Bulgaria. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Discov Med JT - Discovery medicine JID - 101250006 SB - IM MH - Adult MH - Aged MH - Female MH - Fingers/*pathology MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Scleroderma, Systemic/complications/*diagnosis/pathology MH - Skin Ulcer/complications/*diagnosis/pathology EDAT- 2011/12/01 06:00 MHDA- 2012/04/04 06:00 CRDT- 2011/12/01 06:00 PHST- 2011/12/01 06:00 [entrez] PHST- 2011/12/01 06:00 [pubmed] PHST- 2012/04/04 06:00 [medline] PST - ppublish SO - Discov Med. 2011 Nov;12(66):413-8. PMID- 40916990 OWN - NLM STAT- MEDLINE DCOM- 20251002 LR - 20251102 IS - 1531-6963 (Electronic) IS - 1040-8711 (Print) IS - 1040-8711 (Linking) VI - 37 IP - 6 DP - 2025 Nov 1 TI - Decoding vascular dysfunction in systemic sclerosis: from endothelial damage to clinical implications. PG - 373-383 LID - 10.1097/BOR.0000000000001126 [doi] AB - PURPOSE OF REVIEW: This review explores the evolving understanding of vascular dysfunction in systemic sclerosis (SSc), from early endothelial injury to clinical manifestations and emerging therapeutic strategies. RECENT FINDINGS: Endothelial cell (EC) injury, senescence, and endothelial-to-mesenchymal transition are central to SSc vasculopathy. Single-cell and spatial omics have revealed distinct EC subtypes and dysregulated pathways, including interferon signaling and chromatin remodeling. Immune-mediated damage, viral triggers, and autoantibodies contribute to vascular pathology. Clinically, complications such as Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, and renal crisis drive morbidity and healthcare burden. Diagnostic tools such as nailfold capillaroscopy enable early detection of microvascular changes. Novel therapies including CAR-T therapy, JAK inhibitors, and complement blockade, are under investigation. SUMMARY: Vascular dysfunction is a hallmark of SSc and a key driver of disease progression. Advances in molecular profiling and imaging have improved our understanding of its mechanisms and opened new avenues for targeted intervention. Early diagnosis, biomarker-guided care, and multidisciplinary management are essential to improving outcomes. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Massay, Ryan AU - Massay R AD - Division of Rheumatology, Department of Internal Medicine. FAU - Zahn, Carleigh AU - Zahn C AD - Division of Rheumatology, Department of Internal Medicine. AD - Scleroderma Program, Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA. FAU - Tsou, Pei-Suen AU - Tsou PS AD - Division of Rheumatology, Department of Internal Medicine. AD - Scleroderma Program, Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA. LA - eng GR - R01 AI183620/AI/NIAID NIH HHS/United States PT - Journal Article PT - Review DEP - 20250903 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Humans MH - *Scleroderma, Systemic/physiopathology/complications MH - *Endothelium, Vascular/physiopathology/pathology MH - *Vascular Diseases/etiology/physiopathology MH - Endothelial Cells/pathology PMC - PMC12574716 MID - NIHMS2117645 OTO - NOTNLM OT - diagnosis OT - endothelial cells OT - scleroderma OT - treatment OT - vasculopathy COIS- Conflicts of interest: None EDAT- 2025/09/08 12:43 MHDA- 2025/10/02 12:32 PMCR- 2025/11/01 CRDT- 2025/09/08 06:33 PHST- 2025/10/02 12:32 [medline] PHST- 2025/09/08 12:43 [pubmed] PHST- 2025/09/08 06:33 [entrez] PHST- 2025/11/01 00:00 [pmc-release] AID - 00002281-202511000-00006 [pii] AID - 10.1097/BOR.0000000000001126 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2025 Nov 1;37(6):373-383. doi: 10.1097/BOR.0000000000001126. Epub 2025 Sep 3. PMID- 30255410 OWN - NLM STAT- MEDLINE DCOM- 20191002 LR - 20200225 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 78 IP - 1 DP - 2019 Feb TI - [Mimetics of systemic sclerosis]. PG - 14-23 LID - 10.1007/s00393-018-0538-y [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult. OBJECTIVE: The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated. METHODS: A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described. RESULTS: Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics. CONCLUSION: An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals. FAU - Jendrek, S T AU - Jendrek ST AD - Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland. SebastianTorben.Jendrek@uksh.de. FAU - Kahle, B AU - Kahle B AD - Klinik für Dermatologie, Universitätsklinikum Schleswig-Holstein. Campus Lübeck, Lübeck, Deutschland. FAU - Riemekasten, G AU - Riemekasten G AD - Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland. LA - ger PT - Journal Article PT - Review TT - Imitatoren der systemischen Sklerose. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - Eosinophilic synovitis SB - IM MH - Connective Tissue Diseases/*diagnosis MH - Diagnosis, Differential MH - Eosinophilia/diagnosis MH - Fasciitis/diagnosis MH - Humans MH - Scleredema Adultorum/diagnosis MH - *Scleroderma, Localized/diagnosis MH - *Scleroderma, Systemic/diagnosis MH - Scleromyxedema/diagnosis MH - Skin MH - Synovitis OTO - NOTNLM OT - Morphea OT - Nephrogenic systemic fibrosis OT - Scleroderma OT - Scleromyxedema OT - Skin fibrosis EDAT- 2018/09/27 06:00 MHDA- 2019/10/03 06:00 CRDT- 2018/09/27 06:00 PHST- 2018/09/27 06:00 [pubmed] PHST- 2019/10/03 06:00 [medline] PHST- 2018/09/27 06:00 [entrez] AID - 10.1007/s00393-018-0538-y [pii] AID - 10.1007/s00393-018-0538-y [doi] PST - ppublish SO - Z Rheumatol. 2019 Feb;78(1):14-23. doi: 10.1007/s00393-018-0538-y. PMID- 29118999 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220331 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 4 IP - 1 DP - 2017 TI - Associates and predictors of pleurisy or pericarditis in SLE. PG - e000221 LID - 10.1136/lupus-2017-000221 [doi] LID - e000221 AB - BACKGROUND/PURPOSE: Serositis is one of both ACR and SLICC classification criteria for systemic lupus erythematosus (SLE) and a common type of extra renal flare. However, little is known about clinical or immunological associations of pleurisy or pericarditis. The aim of this study is to analyze associates and predictors of pleurisy versus pericarditis in Caucasians and African Americans with SLE. METHODS: 2,390 SLE patients in the Hopkins Lupus Cohort were analyzed for demographic, clinical and serologic associates of pleurisy or pericarditis, defined using the SELENA revision of the SLE Disease Activity Index (SLEDAI). The cross-sectional and prospective study using either univariate or multivariate analysis were performed to evaluate the associates of serositis in SLE. We reported associates with a p-value of less than 0.05 for pleurisy or pericarditis. RESULTS: 43% had pleurisy and 22% had pericarditis. African-American ethnicity was a predictive factor for new pericarditis. Hemolytic anemia, proteinuria, lymphadenopathy and anti-Sm were predictive only of pericarditis, whereas pulmonary fibrosis and GI infarction were predictive only of pleurisy. Fever, Raynaud's syndrome, and anti-DNA were predictors for both pericarditis and pleurisy. CONCLUSION: Our study provides further insights into the associates of pleurisy and pericarditis in SLE. Predictors of pleurisy and pericarditis are shown for the first time. The long term consequences from the cross-sectional analysis gives a lesson that serositis in SLE should not be considered benign. FAU - Ryu, Seungwon AU - Ryu S AD - Konkuk University School of Medicine, Seoul, Republic of Korea. FAU - Fu, Wei AU - Fu W AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Petri, Michelle A AU - Petri MA AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. LA - eng GR - R01 AR043727/AR/NIAMS NIH HHS/United States GR - R01 AR069572/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20171023 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 PMC - PMC5663266 OTO - NOTNLM OT - pericarditis OT - pleurisy OT - serositis OT - systemic lupus erythematosus COIS- Competing interests: None declared. EDAT- 2017/11/10 06:00 MHDA- 2017/11/10 06:01 PMCR- 2017/10/23 CRDT- 2017/11/10 06:00 PHST- 2017/03/23 00:00 [received] PHST- 2017/05/15 00:00 [revised] PHST- 2017/06/17 00:00 [accepted] PHST- 2017/11/10 06:00 [entrez] PHST- 2017/11/10 06:00 [pubmed] PHST- 2017/11/10 06:01 [medline] PHST- 2017/10/23 00:00 [pmc-release] AID - lupus-2017-000221 [pii] AID - 10.1136/lupus-2017-000221 [doi] PST - epublish SO - Lupus Sci Med. 2017 Oct 23;4(1):e000221. doi: 10.1136/lupus-2017-000221. eCollection 2017. PMID- 27178687 OWN - NLM STAT- MEDLINE DCOM- 20170515 LR - 20180222 IS - 1532-8708 (Electronic) IS - 0093-7754 (Linking) VI - 43 IP - 3 DP - 2016 Jun TI - Cutaneous manifestations of genitourinary malignancy. PG - 347-52 LID - S0093-7754(16)00052-X [pii] LID - 10.1053/j.seminoncol.2016.02.027 [doi] AB - Genitourinary cancers are associated with a range of cutaneous syndromes, which can reflect direct metastatic spread, non-metastatic manifestations of malignancy or the consequences of treatment. More than 220,000 new cases of prostate cancer occur each year in the United States, and thus the associations with cutaneous involvement are quite well documented-rare metastatic spread, vasculitic and hemorrhagic syndromes. Cancers of the bladder and kidney may be associated with direct cutaneous metastases, vasculitic syndromes, hereditary leiomyomatosis, and other familial syndromes. Testicular cancer occasionally metastasizes to the skin but more commonly is associated with the dysplastic nevus (multiple atypical nevus) syndrome. A structured approach to history-taking, examination, and investigation is essential for optimal management, especially when these syndromes precede the diagnosis of a known malignancy. A brief review of the more common iatrogenic cutaneous complications is provided, and includes Raynaud's phenomenon, purpura, rash, hand-foot syndrome, the consequences of marrow failure, and bleomycin-induced pigmentation. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Raghavan, Derek AU - Raghavan D AD - Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC. Electronic address: derek.raghavan@carolinashealthcare.org. LA - eng PT - Journal Article PT - Review DEP - 20160223 PL - United States TA - Semin Oncol JT - Seminars in oncology JID - 0420432 SB - IM MH - Female MH - Humans MH - Iatrogenic Disease MH - Kidney Neoplasms/pathology MH - Male MH - Neoplasms, Germ Cell and Embryonal/pathology MH - Paraneoplastic Syndromes/etiology/pathology MH - Prostatic Neoplasms/pathology MH - Skin Neoplasms/*pathology/secondary MH - Urinary Bladder Neoplasms/pathology MH - Urogenital Neoplasms/*pathology/therapy OTO - NOTNLM OT - Bladder OT - Genitourinary OT - Germ cell OT - Prostate OT - Renal cell OT - Testis EDAT- 2016/05/15 06:00 MHDA- 2017/05/16 06:00 CRDT- 2016/05/15 06:00 PHST- 2016/05/15 06:00 [entrez] PHST- 2016/05/15 06:00 [pubmed] PHST- 2017/05/16 06:00 [medline] AID - S0093-7754(16)00052-X [pii] AID - 10.1053/j.seminoncol.2016.02.027 [doi] PST - ppublish SO - Semin Oncol. 2016 Jun;43(3):347-52. doi: 10.1053/j.seminoncol.2016.02.027. Epub 2016 Feb 23. PMID- 25435879 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20141201 LR - 20200930 IS - 1687-9260 (Print) IS - 1687-9279 (Electronic) IS - 1687-9260 (Linking) VI - 2014 DP - 2014 TI - Are There Clinical Differences in Limited Systemic Sclerosis according to Extension of Skin Involvement? PG - 716358 LID - 10.1155/2014/716358 [doi] LID - 716358 AB - Objectives. To examine the characteristics of our patients with limited systemic sclerosis (lSSc) for differences between Barnett Type 1 (sclerodactyly only) and Type 2 or intermediate (acrosclerosis-distal but may reach up to elbows and/or knees plus face) subsets. Methods. Records of patients between January 1, 2000, and December 31, 2011, with SSc or those with anti-Scl-70, anticentromere, or antinucleolar antibodies were reviewed. Only cases fulfilling ACR 1980 criteria were included and classified as diffuse or limited according to LeRoy's criteria. Limited SSc was separated into sclerodactyly and acrosclerosis (Barnett's Types 1 and 2). Results. 234 SSc patients (216 females) fulfilled criteria. Female/male ratio was 12 : 1; 24% had dSSc and 76% lSSC (64% Type 1 and 12% Type 2). Total follow-up was 688 patient-years. Within lSSC, the Type 2 group had significantly shorter duration of Raynaud's and more anti-Scl-70 and less anticentromere antibodies. In particular, interstitial lung disease (ILD) was significantly more prevalent in Type 2 group and similar to Type 3. Conclusions. These results appear to confirm that extension of skin involvement within limited SSc may identify two different subsets with clinical and serologic characteristics. FAU - Scolnik, Marina AU - Scolnik M AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina. FAU - Catoggio, Luis J AU - Catoggio LJ AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina ; Instituto Universitario Escuela de Medicina, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina. FAU - Lancioni, Eliana AU - Lancioni E AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina. FAU - Sabelli, Mirtha R AU - Sabelli MR AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina. FAU - Saucedo, Carla M AU - Saucedo CM AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina. FAU - Marin, Josefina AU - Marin J AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina. FAU - Soriano, Enrique R AU - Soriano ER AD - Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina ; Instituto Universitario Escuela de Medicina, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina. LA - eng PT - Journal Article DEP - 20141111 PL - United States TA - Int J Rheumatol JT - International journal of rheumatology JID - 101519204 PMC - PMC4243126 EDAT- 2014/12/02 06:00 MHDA- 2014/12/02 06:01 PMCR- 2014/11/11 CRDT- 2014/12/02 06:00 PHST- 2014/08/18 00:00 [received] PHST- 2014/10/19 00:00 [accepted] PHST- 2014/12/02 06:00 [entrez] PHST- 2014/12/02 06:00 [pubmed] PHST- 2014/12/02 06:01 [medline] PHST- 2014/11/11 00:00 [pmc-release] AID - 10.1155/2014/716358 [doi] PST - ppublish SO - Int J Rheumatol. 2014;2014:716358. doi: 10.1155/2014/716358. Epub 2014 Nov 11. PMID- 18814097 OWN - NLM STAT- MEDLINE DCOM- 20081031 LR - 20080924 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 133 Suppl 6 DP - 2008 Oct TI - ["Pulmonary hypertension" of the gastrointestinal tract in patients with systemic sclerosis: thoughts on a not (yet) existing disease entity]. PG - S203-5 LID - 10.1055/s-0028-1091239 [doi] AB - Vascular changes are often found in numerous organs of patients with systemic sclerosis (SSc). It has been shown that, e.g. Raynaud's syndrome, ulcers of the digits and pulmonary arterial hypertension have similar patterns of endothelial dysfunction. There is evidence of gastrointestinal complications in almost all patients with SSc, most often caused by abnormal motility and fibrosis. Immunohistological studies have shown that gastric and intestinal mucosae have not only activation of classical activation markers and adhesion molecules, but also of growth factors demonstrated in pulmonary hypertension as well as overexpression of endothelin-1. The possible common pathogenesis of he vasculopathies in the lung and gastrointestinal tract suggests that explorative studies should be undertaken in which endothelin-receptor antagonists are administered to patients with abnormal gastrointestinal motility and SSc. FAU - Müller-Ladner, U AU - Müller-Ladner U AD - Abteilung für Rheumatologie und klinische Immunologie, Justus-Liebig-Universität Giessen, Kerckhoff-Klinik Bad Nauheim. u.mueller-ladner@kerckhoff-klinik.de LA - ger PT - English Abstract PT - Journal Article PT - Review TT - "Pulmonale Hypertonie" des Gastrointestinaltraktes bei Patienten mit Systemischer Sklerose: Uberlegungen zu einem (noch) nicht existierenden Krankheitsbild. DEP - 20080923 PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 SB - IM MH - Fibrosis MH - Gastric Mucosa/blood supply MH - Gastrointestinal Diseases/*etiology/pathology/physiopathology MH - Gastrointestinal Motility MH - Gastrointestinal Tract/pathology MH - Humans MH - Hypertension, Pulmonary/*etiology/pathology/physiopathology MH - Lung/pathology MH - Mesenteric Arteries/physiopathology MH - Scleroderma, Systemic/*complications/pathology/physiopathology MH - Telangiectasis/physiopathology RF - 10 EDAT- 2008/10/01 09:00 MHDA- 2008/11/01 09:00 CRDT- 2008/10/01 09:00 PHST- 2008/10/01 09:00 [pubmed] PHST- 2008/11/01 09:00 [medline] PHST- 2008/10/01 09:00 [entrez] AID - 10.1055/s-0028-1091239 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2008 Oct;133 Suppl 6:S203-5. doi: 10.1055/s-0028-1091239. Epub 2008 Sep 23. PMID- 41311376 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251128 LR - 20260114 IS - 2213-0071 (Print) IS - 2213-0071 (Electronic) IS - 2213-0071 (Linking) VI - 58 DP - 2025 TI - Unraveling complexity: A rare case of pulmonary sarcoidosis coinciding with systemic scleroderma. PG - 102316 LID - 10.1016/j.rmcr.2025.102316 [doi] LID - 102316 AB - We present a rare case of a 66-year-old female with systemic sclerosis and Raynaud's phenomenon, diagnosed at age 25, who was found to have multinodular pulmonary sarcoidosis. The coexistence of sarcoidosis and scleroderma, are relatively common, well-documented in medical literatures however, the relationship between the two is complex and multifactorial. She was referred to pulmonology for evaluation of a lung mass and nodule noted on chest x-ray. Chest CT revealed widespread bilateral pulmonary nodularity, more prominent on the right, with multifocal mass-like consolidation. Differential diagnoses included malignancy, infection, or inflammatory disease. A transbronchial lung biopsy of the right upper and middle lobes showed focal granulomatous inflammation with negative AFB staining, and lymph node biopsies were negative for malignancy. These findings favored a diagnosis of sarcoidosis over systemic sclerosis, which typically does not present with granulomatous inflammation. A PET CT was performed to further differentiate between inflammation and malignancy, revealing extensive perilymphatic nodularity and FDG-avid mass-like opacities, especially in the right upper and middle lobes, consistent with the sarcoid Galaxy sign. She was initiated on prednisone 5 mg and methotrexate 25 mg daily. Given the rarity of this presentation, recognizing the co-occurrence of these two autoimmune conditions was critical in reaching the correct diagnosis and initiating appropriate therapy. CI - © 2025 The Authors. FAU - Hwang, So Yeon AU - Hwang SY AD - Department of Internal Medicine, Northwest Medical Center, Tucson, AZ, USA. FAU - Ho, David AU - Ho D AD - Department of Internal Medicine, Northwest Medical Center, Tucson, AZ, USA. FAU - Mittal, Abhinav AU - Mittal A AD - Interventional Pulmonology, Northwest Medical Center, Tucson, AZ, USA. FAU - Dubyk, Favia AU - Dubyk F AD - Foothills Pathology, Northwest Medical Center, Tucson, AZ, USA. FAU - Reyes, Felix AU - Reyes F AD - Pulmonology and Critical Care, Northwest Medical Center - Houghton Hospital, Tucson, AZ, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20251104 PL - England TA - Respir Med Case Rep JT - Respiratory medicine case reports JID - 101604463 PMC - PMC12648700 COIS- The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/11/28 06:28 MHDA- 2025/11/28 06:29 PMCR- 2025/11/04 CRDT- 2025/11/28 04:33 PHST- 2025/06/06 00:00 [received] PHST- 2025/10/20 00:00 [revised] PHST- 2025/10/30 00:00 [accepted] PHST- 2025/11/28 06:29 [medline] PHST- 2025/11/28 06:28 [pubmed] PHST- 2025/11/28 04:33 [entrez] PHST- 2025/11/04 00:00 [pmc-release] AID - S2213-0071(25)00152-2 [pii] AID - 102316 [pii] AID - 10.1016/j.rmcr.2025.102316 [doi] PST - epublish SO - Respir Med Case Rep. 2025 Nov 4;58:102316. doi: 10.1016/j.rmcr.2025.102316. eCollection 2025. PMID- 40583919 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250702 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 6 DP - 2025 Jun TI - Gastrointestinal Complications of Systemic Sclerosis: A Case Report. PG - e86844 LID - 10.7759/cureus.86844 [doi] LID - e86844 AB - Systemic sclerosis (SSc) is a rare, chronic systemic rheumatic disease. Gastrointestinal (GI) involvement is highly prevalent in SSc and any part of the GI tract may be affected. This can range from mild symptoms like heartburn and dysphagia to severe issues like abdominal distention, malnutrition, and fecal incontinence. In SSc patients, GI tract dysfunction significantly impacts the quality of life and is a major contributor to both morbidity and mortality. This case report highlights an 80-year-old female patient with SSc/CREST (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia) syndrome experiencing severe GI involvement who presented to the emergency department with hypoxic respiratory failure and acute kidney injury. The patient and her family finally made the decision to transition to inpatient hospice care, focusing on comfort and dignity, leading to the patient's peaceful passing. CI - Copyright © 2025, Arbabi et al. FAU - Arbabi, Mason AU - Arbabi M AD - Internal Medicine, University of Kentucky College of Medicine, Lexington, USA. FAU - Garg, Bella AU - Garg B AD - Internal Medicine, Centinela Hospital Medical Center, Inglewood, USA. FAU - Saghari, Saviz AU - Saghari S AD - Internal Medicine, West Anaheim Medical Center, Anaheim, USA. FAU - Patel, Paryus AU - Patel P AD - Internal Medicine/Pulmonology, Centinela Hospital Medical Center, Inglewood, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250627 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12203754 OTO - NOTNLM OT - gastroparesis OT - hospice and palliative care OT - limited systemic sclerosis OT - progressive dysphagia OT - small intestinal bacterial overgrowth COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/07/01 04:06 MHDA- 2025/07/01 04:07 PMCR- 2025/06/27 CRDT- 2025/06/30 06:04 PHST- 2025/06/26 00:00 [accepted] PHST- 2025/07/01 04:07 [medline] PHST- 2025/07/01 04:06 [pubmed] PHST- 2025/06/30 06:04 [entrez] PHST- 2025/06/27 00:00 [pmc-release] AID - 10.7759/cureus.86844 [doi] PST - epublish SO - Cureus. 2025 Jun 27;17(6):e86844. doi: 10.7759/cureus.86844. eCollection 2025 Jun. PMID- 38372720 OWN - NLM STAT- MEDLINE DCOM- 20240329 LR - 20240329 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 3 DP - 2024 Mar TI - Leprosy mimicking autoimmune diseases: a case series. PG - 746-751 LID - 10.55563/clinexprheumatol/ov3kgl [doi] AB - OBJECTIVES: Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. Less frequently, there is involvement of the musculoskeletal system, and occurrence of systemic manifestation with non-specific symptoms such as fever, fatigue and myalgia. Therefore, leprosy can often mimic autoimmune diseases such as arthritis, vasculitis, or collagenosis and be mis-diagnosed. METHODS: This study describes a series of cases of leprosy mimicking autoimmune diseases in patients treated in the Rheumatology Department of our centre in the period 2019 to 2023. All patients were investigated regarding leprosy criteria and had clinical evaluation, serum markers, and histopathological analyses recorded. The diagnosis of leprosy was confirmed using skin biopsy followed by testing for acid-fast bacillus (AFB) or smear microscopy. RESULTS: Six patients who were initially investigated for autoimmune diseases were identified as diagnosed as leprosy cases, fulfilling both clinical and histopathologic criteria, two of whom presented with symptoms of polyarthritis with an inflammatory characteristic, two diffuse erythematous-violaceous lesions, three recurrent fever, three arthralgia, and one Raynaud's phenomenon, which are all characteristics present most frequently in rheumatologic diseases. CONCLUSIONS: We must consider the bacillary infection as a differential diagnosis of autoimmune diseases. Histopathological analysis is an important tool and the gold standard for diagnostic confirmation. FAU - Aride, Danielle Busato AU - Aride DB AD - Hospital Universitário Cassiano Antônio Moraes, da Universidade Federal do Espírito Santo, Brazil. daniellearide@gmail.com. FAU - Dalmaso, Bárbara Ferraço AU - Dalmaso BF AD - Hospital Universitário Cassiano Antônio Moraes, da Universidade Federal do Espírito Santo, Brazil. FAU - Moulin, Anna Carolina S AU - Moulin ACS AD - Hospital Universitário Cassiano Antônio Moraes, da Universidade Federal do Espírito Santo, Brazil. FAU - Moulaz, Isac R AU - Moulaz IR AD - Hospital Universitário Cassiano Antônio Moraes, da Universidade Federal do Espírito Santo, Brazil. FAU - Machado, Ketty Lysie Libardi Lira AU - Machado KLLL AD - Hospital Universitário Cassiano Antônio Moraes, da Universidade Federal do Espírito Santo, Brazil. LA - eng PT - Case Reports PT - Journal Article DEP - 20240212 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - *Leprosy/diagnosis/drug therapy/microbiology MH - Mycobacterium leprae MH - Skin/pathology MH - *Autoimmune Diseases/diagnosis/pathology MH - *Arthritis EDAT- 2024/02/19 12:43 MHDA- 2024/03/29 06:46 CRDT- 2024/02/19 10:53 PHST- 2023/12/12 00:00 [received] PHST- 2024/01/18 00:00 [accepted] PHST- 2024/03/29 06:46 [medline] PHST- 2024/02/19 12:43 [pubmed] PHST- 2024/02/19 10:53 [entrez] AID - 20666 [pii] AID - 10.55563/clinexprheumatol/ov3kgl [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Mar;42(3):746-751. doi: 10.55563/clinexprheumatol/ov3kgl. Epub 2024 Feb 12. PMID- 23737595 OWN - NLM STAT- MEDLINE DCOM- 20140916 LR - 20211021 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2013 DP - 2013 Jun 3 TI - Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis. LID - 10.1136/bcr-2013-010117 [doi] LID - bcr2013010117 AB - Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy. FAU - Rodríguez-Pérez, Noelia AU - Rodríguez-Pérez N AD - Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico. FAU - Rodríguez-Navedo, Yerania AU - Rodríguez-Navedo Y FAU - Font, Yvonne M AU - Font YM FAU - Vilá, Luis M AU - Vilá LM LA - eng PT - Case Reports PT - Journal Article DEP - 20130603 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 8N3DW7272P (Cyclophosphamide) RN - VB0R961HZT (Prednisone) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Cryoglobulinemia/*diagnosis/drug therapy MH - Cyclophosphamide/administration & dosage/therapeutic use MH - Female MH - Humans MH - Methylprednisolone/administration & dosage/therapeutic use MH - Myositis/*diagnosis/drug therapy MH - Prednisone/administration & dosage/therapeutic use MH - Treatment Outcome MH - Vasculitis/*diagnosis/drug therapy PMC - PMC3702929 EDAT- 2013/06/06 06:00 MHDA- 2014/09/17 06:00 PMCR- 2015/06/03 CRDT- 2013/06/06 06:00 PHST- 2013/06/06 06:00 [entrez] PHST- 2013/06/06 06:00 [pubmed] PHST- 2014/09/17 06:00 [medline] PHST- 2015/06/03 00:00 [pmc-release] AID - bcr-2013-010117 [pii] AID - 10.1136/bcr-2013-010117 [doi] PST - epublish SO - BMJ Case Rep. 2013 Jun 3;2013:bcr2013010117. doi: 10.1136/bcr-2013-010117. PMID- 16987836 OWN - NLM STAT- MEDLINE DCOM- 20070607 LR - 20141120 IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 45 Suppl 3 DP - 2006 Oct TI - Vasoactive therapies in systemic sclerosis. PG - iii49-51 AB - In systemic sclerosis (SSc), vasculopathy is a central mechanism and is a major initial event in the process of sclerosis and causing different complications such as Raynaud's phenomenon, ulcer(s) or pulmonary hypertension, the latter being life threatening. Therefore, vasoactive therapies are important when taking care of patients with SSc. However, as treatment has been difficult, numerous therapeutic modalities have been suggested. Until now, the interpretation of most studies is limited due to the heterogeneity of patient groups, the low number of patients, the short duration of the treatments and, possibly, further pathogenic mechanisms such as autoimmunity. Several drugs are now available with effects on vasculopathy and, furthermore, on specific pathogenic mechanisms in SSc. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have potential effects on fibrosis, inflammation and endothelial cells, suggesting a disease-modifying capacity in systemic sclerosis. This review summarizes evidence-based therapy recommendations. FAU - Riemekasten, G AU - Riemekasten G AD - MD. Department of Rheumatology and Clinical Immunology, Charité University Hospital, Schumannstr. 20/21, D-10117 Berlin, Germany. gabriela.riemekasten@charite.de FAU - Sunderkötter, C AU - Sunderkötter C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Prostaglandins I) SB - IM EIN - Rheumatology (Oxford). 2008 Feb;47(2):234-5 MH - *Endothelin Receptor Antagonists MH - Humans MH - Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/*therapeutic use MH - Prostaglandins I/*therapeutic use MH - Scleroderma, Systemic/complications/*drug therapy/physiopathology MH - Vascular Diseases/drug therapy/etiology RF - 26 EDAT- 2006/09/22 09:00 MHDA- 2008/03/26 09:00 CRDT- 2006/09/22 09:00 PHST- 2006/09/22 09:00 [pubmed] PHST- 2008/03/26 09:00 [medline] PHST- 2006/09/22 09:00 [entrez] AID - 45/suppl_3/iii49 [pii] AID - 10.1093/rheumatology/kel283 [doi] PST - ppublish SO - Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii49-51. doi: 10.1093/rheumatology/kel283. PMID- 39087260 OWN - NLM STAT- MEDLINE DCOM- 20250117 LR - 20250402 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 9 IP - 1 DP - 2025 Jan 16 TI - Coexistence of anti-KS and anti-TIF1-γ antibodies in clinically amyopathic dermatomyositis presenting with rapid progression of interstitial lung disease. PG - 79-83 LID - 10.1093/mrcr/rxae033 [doi] AB - Polymyositis/dermatomyositis (DM) is an idiopathic inflammatory myopathy manifesting mainly as symmetrical proximal muscle weakness and/or typical cutaneous features due to autoimmune mechanisms. Clinically amyopathic dermatomyositis (CADM) is a subset of DM that exhibits only the typical cutaneous features without any clinical muscle symptoms. Several autoantibodies have been found specifically in patients with polymyositis/DM, including CADM patients. The anti-KS antibody is one of a group of anti-aminoacyl transfer RNA antibodies that are mainly associated with fever, Raynaud's phenomenon, polyarthritis, and interstitial lung disease (ILD), whereas anti-TIF1-γ antibody is frequently found in DM patients with malignancy. Here, we report a CADM patient having both anti-KS antibody and anti-TIF1-γ antibody. This patient developed an acute exacerbation of ILD and was successfully treated with high-dose corticosteroid pulse therapy together with immunosuppressive agents. Although earlier experience had indicated that the seminal characteristic of anti-KS-positive ILD was slowly developing disease onset with little or no progression over the clinical course, the present patient suffered rapidly progressive disease. CI - © Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com. FAU - Ota, Yuichiro AU - Ota Y AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Ohisa, Toshiki AU - Ohisa T AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Ishii, Akira AU - Ishii A AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Sugiyama, Mai AU - Sugiyama M AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Kondo, Yasushi AU - Kondo Y AUID- ORCID: 0000-0002-1566-2088 AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. AD - Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. FAU - Nishikawa, Ayumi AU - Nishikawa A AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Sasaki, Noriko AU - Sasaki N AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Yamada, Chiho AU - Yamada C AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. FAU - Sato, Shinji AU - Sato S AUID- ORCID: 0000-0002-6338-7367 AD - Department of Internal Medicine, Tokai University School of Medicine, Kanagawa, Japan. LA - eng PT - Case Reports PT - Journal Article PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - 0 (Transcription Factors) RN - 0 (TRIM33 protein, human) RN - Amyopathic dermatomyositis SB - IM MH - Female MH - Humans MH - Middle Aged MH - *Autoantibodies/blood/immunology MH - *Dermatomyositis/diagnosis/immunology/complications/drug therapy MH - *Disease Progression MH - Immunosuppressive Agents/therapeutic use/administration & dosage MH - *Lung Diseases, Interstitial/diagnosis/immunology/etiology/complications MH - Transcription Factors/immunology MH - Treatment Outcome OTO - NOTNLM OT - Dermatomyositis OT - anti-KS antibody OT - anti-TIF1-γ antibody OT - autoantibody EDAT- 2024/08/01 06:42 MHDA- 2025/01/17 18:22 CRDT- 2024/08/01 04:43 PHST- 2024/02/24 00:00 [received] PHST- 2024/05/23 00:00 [revised] PHST- 2024/07/31 00:00 [accepted] PHST- 2025/01/17 18:22 [medline] PHST- 2024/08/01 06:42 [pubmed] PHST- 2024/08/01 04:43 [entrez] AID - 7725382 [pii] AID - 10.1093/mrcr/rxae033 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2025 Jan 16;9(1):79-83. doi: 10.1093/mrcr/rxae033. PMID- 35407661 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230308 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 11 IP - 7 DP - 2022 Apr 6 TI - Clinical Features of Anti-Synthetase Syndrome Associated with Prognosis in Patients with Dermatomyositis and Polymyositis. LID - 10.3390/jcm11072052 [doi] LID - 2052 AB - We evaluated whether the clinical features of anti-synthetase syndrome (ASA)—myositis, fever, arthritis, mechanic’s hand, Raynaud’s phenomenon and interstitial lung disease—are relevant to prognosis in patients with dermatomyositis/polymyositis (DM/PM). A retrospective analysis was performed to identify patients diagnosed with DM/PM according to Bohan and Peter criteria. Clinical information, laboratory data and the presence of ASA clinical features at disease diagnosis were searched, and the outcomes of all-cause mortality, intensive care unit admission and disease remission at 1 year were assessed. Among the 86 patients included, fever (36.0%) and interstitial lung disease (26.7%) were the most common ASA clinical features. During the follow-up, 12 patients experienced death, and 7 of the 12 deaths (58.3%) occurred within 3 months of DM/PM diagnosis. Mortality was more frequently observed in those presenting with fever than in those without (25.8% versus 7.3%, p = 0.024). Multivariable Cox proportional analysis revealed that male sex (hazard ratio [HR] 5.53, 95% confidence interval [CI] 1.65, 18.49, p < 0.01) and fever (HR 4.20, 95% CI 1.26, 14.01, p = 0.02) independently predicted mortality. The clinical impact of fever was consistent in both sexes. Fever could be a warning signal heralding the poor outcome of mortality in patients with DM/PM, especially in early disease phases. FAU - Ahn, Sung Soo AU - Ahn SS AUID- ORCID: 0000-0002-9002-9880 AD - Department of Internal Medicine, Yongin Severance Hospital, College of Medicine, Yonsei University, Yongin 16995, Korea. FAU - Park, Yong-Beom AU - Park YB AD - Division of Rheumatology, Department of Internal Medicine, College of Medicine, Yonsei University, Seoul 03722, Korea. AD - Institute for Immunology and Immunological Diseases, College of Medicine, Yonsei University, Seoul 03722, Korea. FAU - Lee, Sang-Won AU - Lee SW AUID- ORCID: 0000-0002-8038-3341 AD - Division of Rheumatology, Department of Internal Medicine, College of Medicine, Yonsei University, Seoul 03722, Korea. AD - Institute for Immunology and Immunological Diseases, College of Medicine, Yonsei University, Seoul 03722, Korea. LA - eng GR - HI14C1324/Korea Health Industry Development Institute/Republic of Korea GR - 6-2019-0184/Yonsei University College of Medicine/ PT - Journal Article DEP - 20220406 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC8999572 OTO - NOTNLM OT - anti-synthetase syndrome OT - clinical features OT - dermatomyositis OT - fever OT - mortality OT - polymyositis COIS- The authors declare no conflict of interest. EDAT- 2022/04/13 06:00 MHDA- 2022/04/13 06:01 PMCR- 2022/04/06 CRDT- 2022/04/12 01:07 PHST- 2022/02/17 00:00 [received] PHST- 2022/04/05 00:00 [revised] PHST- 2022/04/05 00:00 [accepted] PHST- 2022/04/12 01:07 [entrez] PHST- 2022/04/13 06:00 [pubmed] PHST- 2022/04/13 06:01 [medline] PHST- 2022/04/06 00:00 [pmc-release] AID - jcm11072052 [pii] AID - jcm-11-02052 [pii] AID - 10.3390/jcm11072052 [doi] PST - epublish SO - J Clin Med. 2022 Apr 6;11(7):2052. doi: 10.3390/jcm11072052. PMID- 28606232 OWN - NLM STAT- MEDLINE DCOM- 20170831 LR - 20240731 IS - 1008-8830 (Print) IS - 1008-8830 (Linking) VI - 19 IP - 6 DP - 2017 Jun TI - [A clinical analysis of 15 children with systemic lupus erythematosus accompanied by pulmonary hypertension]. PG - 658-662 AB - OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0.1 year (range: 0-6.5 years). Aside from PH-related symptoms, Raynaud's phenomenon was observed in 6 (40%) of the 15 patients. There was no significant difference in SLE disease activity (evaluated by complements 3 and 4 levels, erythrocyte sedimentation rate, and positive rate of anti-double-stranded DNA) between patients with mild-to-moderate PH and those with severe PH (P<0.05). As for treatment, 13 patients received immunosuppressive therapy with glucocorticoids, and among them 2 patients received PH-targeted therapy. During a median follow-up of 8.0 years (range: 0.5-18.1 years) since the diagnosis of PH, 2 deaths were noted with class III or IV cardiac function (World Health Organization), while the other patients were in a stable condition. CONCLUSIONS: Raynaud's phenomenon is a common clinical manifestation in children with SLE accompanied by pulmonary hypertension (PH). PH severity is not significantly associated with SLE disease activity, and thus greater focus should be placed upon early screening of pulmonary arterial pressure in SLE patients. Early diagnosis and early treatment can improve the prognosis of children with SLE. FAU - Li, Ji AU - Li J AD - Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China. songhm1021@hotmail.com. FAU - Ma, Jing-Ran AU - Ma JR FAU - Sun, Zhi-Xing AU - Sun ZX FAU - Jiang, Jing-Jing AU - Jiang JJ FAU - Dong, Yan-Qing AU - Dong YQ FAU - Wang, Qian AU - Wang Q FAU - Song, Hong-Mei AU - Song HM LA - chi PT - Journal Article PL - China TA - Zhongguo Dang Dai Er Ke Za Zhi JT - Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics JID - 100909956 SB - IM MH - Adolescent MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Hypertension, Pulmonary/*complications/drug therapy MH - Infant MH - Lupus Erythematosus, Systemic/*complications/drug therapy MH - Male PMC - PMC7390294 EDAT- 2017/06/14 06:00 MHDA- 2017/09/01 06:00 PMCR- 2017/06/25 CRDT- 2017/06/14 06:00 PHST- 2017/06/14 06:00 [entrez] PHST- 2017/06/14 06:00 [pubmed] PHST- 2017/09/01 06:00 [medline] PHST- 2017/06/25 00:00 [pmc-release] AID - 10.7499/j.issn.1008-8830.2017.06.009 [pii] AID - zgddekzz-19-6-658 [pii] AID - 10.7499/j.issn.1008-8830.2017.06.009 [doi] PST - ppublish SO - Zhongguo Dang Dai Er Ke Za Zhi. 2017 Jun;19(6):658-662. doi: 10.7499/j.issn.1008-8830.2017.06.009. PMID- 26932290 OWN - NLM STAT- MEDLINE DCOM- 20170215 LR - 20191113 IS - 1573-3971 (Print) IS - 1573-3971 (Linking) VI - 9 IP - 4 DP - 2013 TI - Nailfold Capillaroscopy - Its Role in Diagnosis and Differential Diagnosis of Microvascular Damage in Systemic Sclerosis. PG - 254-60 AB - In the nailfold area, specific diagnostic microvascular abnormalities are easily recognized via capillaroscopic examination in systemic sclerosis (SSc). They are termed "scleroderma" type capillaroscopic pattern, which includes presence of dilated, giant capillaries, haemorrhages, avascular areas, and neoangiogenic capillaries and are observed in the majority of SSc patients (in more than 90%). LeRoy and Medsger (2001) proposed criteria for early diagnosis of SSc with inclusion of the abnormal capillaroscopic changes and suggested to prediagnose SSc prior to the development of other manifestations of the disease. It is a new era in the diagnosis of SSc. At present, an international multicenter project is performed. It aims validation of criteria for very early diagnosis of SSc (project VEDOSS (Very Early Diagnosis of Systemic Sclerosis) and is organized by European League Against Rheumatism (EULAR) Scleroderma Trials and Reasearch. Very recently the first results of the VEDOSS project were processed and new EULAR/ACR (American College of Rheumatology) classification criteria have been validated and published (2013), in which the characteristic capillaroscopic changes have been included. Our observations confirm the high frequency of the specific capillaroscopic changes of the fingers in SSc, which have been found in 97.2% of the cases from the studied patient population. We have performed for the first time capillaroscopic examinations of the toes in SSc. Interestingly,"scleroderma type" capillaroscopic pattern was also found at the toes in a high proportion of patients - 66.7%, but it is significantly less frequent as compared with fingers (97.2%, p<0.05). In our opinion, the examination of the toes of SSc patients should be considered as it suggests an additional opportunity for evaluation of the microvascular changes in these patients although the observed changes are in a lower proportion of cases. Thus, capillaroscopic examination is a cornerstone for the very early diagnosis of SSc. Patients with clinical symptoms of peripheral vasospasm (Raynaud's phenomenon (RP)) in association with puffy fingers and/or sclerodactyly should be carefully examined. Hence, appearance of "scleroderma" type capillaroscopic changes in RP patients should be interpreted in the clinical context, because some of the components of this pattern may be observed in several other connective tissue diseases such as mixed connective tissue disease, undifferentiated connective tissue disease that are termed "scleroderma-like" capillaroscopic changes. Capillaroscopic examination is an obligatory screening method in these cases, but the pathologic capillaroscopic changes are not specific and their interpretation is in clinical context. FAU - Lambova, Sevdalina AU - Lambova S AD - Medical University - Plovdiv, Department of Propedeutics in Internal Medicine, Clinic of Rheumatology, Plovdiv, Bulgaria, Plovdiv - 4002, 15A "Vasil Aprilov" Blvd. sevdalina_n@abv.bg. FAU - Hermann, W AU - Hermann W FAU - Muller-Ladner, Ulf AU - Muller-Ladner U LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM MH - Connective Tissue Diseases/diagnosis MH - Diagnosis, Differential MH - *Early Diagnosis MH - Humans MH - Microscopic Angioscopy/*methods MH - Scleroderma, Systemic/*diagnosis EDAT- 2013/01/01 00:00 MHDA- 2017/02/16 06:00 CRDT- 2016/03/03 06:00 PHST- 2016/03/03 06:00 [entrez] PHST- 2013/01/01 00:00 [pubmed] PHST- 2017/02/16 06:00 [medline] AID - CRR-EPUB-60157 [pii] AID - 10.2174/157339710904140417125241 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2013;9(4):254-60. doi: 10.2174/157339710904140417125241. PMID- 22596213 OWN - NLM STAT- MEDLINE DCOM- 20121025 LR - 20260518 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 51 IP - 9 DP - 2012 Sep TI - Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis. PG - 1606-9 AB - OBJECTIVES: This study aims to examine the utility of von Willebrand factor (vWF) as a biomarker in lcSSc, in particular the ability of vWF to predict the future development of disease manifestations in this disease. METHODS: vWFAg concentrations were measured in the serum of patients with lcSSc at baseline and at 3 years, during the QUINs trial [Prevention of Vascular Damage in Scleroderma with Angiotensin-Converting Enzyme (ACE) Inhibition]. %DL(CO), %KCO, %FVC, pulmonary artery pressure (PAP) estimation by echocardiography, Raynaud's attack frequency, Raynaud's severity, digital ulcer frequency, urinary protein excretion, estimated glomerular filtration rate (eGFR), modified Rodnan skin score and Medsger disease activity score were also measured at baseline and 3 years. RESULTS: Baseline serum vWF concentrations were related to concurrent Medsger disease activity score, %DL(CO), %FVC, urinary protein excretion, eGFR and PAP >30 mmHg. In logistic regression models, baseline serum vWF concentrations were able to predict the future development of elevated PAP by echocardiography (PAP >40 mmHg, P = 0.001). CONCLUSIONS: Pulmonary artery hypertension is a life-threatening complication of lcSSc. vWF is a marker of endothelial cell activation. Raised serum concentrations of vWF in lcSSc increase the risk of developing subsequent elevation in PAP. Therefore screening patients with lcSSc for vWF may identify a group at risk of developing PAH. These patients could potentially be targeted with agents that stabilize the endothelium, e.g. statins. FAU - Barnes, Theresa AU - Barnes T AD - Institute of Chronic Disease and Ageing, Clinical Sciences Centre, Aintree University Hospital, Longmoor Lane, Liverpool, UK. FAU - Gliddon, Angela AU - Gliddon A FAU - Doré, Caroline J AU - Doré CJ FAU - Maddison, Peter AU - Maddison P FAU - Moots, Robert J AU - Moots RJ CN - QUINs Trial Study Group LA - eng GR - MC_U122886349/MRC_/Medical Research Council/United Kingdom GR - 13479/ARC_/Arthritis Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120516 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Biomarkers) RN - 0 (von Willebrand Factor) SB - IM MH - Biomarkers/*blood MH - Disease Progression MH - Echocardiography MH - Humans MH - Hypertension, Pulmonary/*blood/diagnosis/etiology MH - Logistic Models MH - Mass Screening/methods MH - Predictive Value of Tests MH - Prognosis MH - Pulmonary Artery/diagnostic imaging/pathology/physiopathology MH - Scleroderma, Systemic/*blood/complications/diagnosis MH - Severity of Illness Index MH - von Willebrand Factor/*metabolism PMC - PMC3418644 FIR - Barnes, T IR - Barnes T FIR - Black, C IR - Black C FIR - Borg, A IR - Borg A FIR - Camilleri, J IR - Camilleri J FIR - Chakravarty, K IR - Chakravarty K FIR - Denton, C IR - Denton C FIR - Emery, P IR - Emery P FIR - Griffiths, B IR - Griffiths B FIR - Herrick, A IR - Herrick A FIR - Hopkinson, N IR - Hopkinson N FIR - Hickling, P IR - Hickling P FIR - Lanyon, P IR - Lanyon P FIR - Laversuch, C IR - Laversuch C FIR - Lawson, T IR - Lawson T FIR - Mallya, R IR - Mallya R FIR - Maddison, P IR - Maddison P FIR - McHugh, N IR - McHugh N FIR - Moots, R IR - Moots R FIR - Nisar, M IR - Nisar M FIR - Rhys-Dillon, C IR - Rhys-Dillon C FIR - Sheeran, T IR - Sheeran T EDAT- 2012/05/19 06:00 MHDA- 2012/10/26 06:00 PMCR- 2012/05/16 CRDT- 2012/05/19 06:00 PHST- 2012/05/19 06:00 [entrez] PHST- 2012/05/19 06:00 [pubmed] PHST- 2012/10/26 06:00 [medline] PHST- 2012/05/16 00:00 [pmc-release] AID - kes068 [pii] AID - 10.1093/rheumatology/kes068 [doi] PST - ppublish SO - Rheumatology (Oxford). 2012 Sep;51(9):1606-9. doi: 10.1093/rheumatology/kes068. Epub 2012 May 16. PMID- 22525638 OWN - NLM STAT- MEDLINE DCOM- 20120813 LR - 20181201 IS - 1646-0758 (Electronic) IS - 0870-399X (Linking) VI - 24 IP - 5 DP - 2011 Sep-Oct TI - [Digital ulcers in systemic sclerosis: use of endotheline antagonists]. PG - 837-42 AB - INTRODUCTION: Systemic sclerosis (SSc) is a systemic disease, characterized by fibrosis and vasculopathy, with variable internal organ involvement. Skin is very often involved, namely digital ulcers (DU), seldom treatment resistant, responsible for important functional limitation. The DU can evolve from sclerodactily with superficial ulcers, isquemic lesions, deep necrosis, gangrene, loss of tissue, and consequently, to finger amputation. METHODS: The authors describe the case of a 36 year old female patient, with SSc diagnosed 6 years previously, with skin, lung and gut manifestations. The patient showed uncontrolled Raynaud's phenomenon (RF), despite the adequate treatment using nifedidpine and general local warming measures, with progressively worsening DU and isquemia, especially in cold seasons. Bosentan, 62.5 mg twice daily was started, and a significant improvement in the peripheral isquemic lesions was achieved. The ulcers' healing was fast, the patient totally recovered function and regained quality of life, and no further lesions developed. CONCLUSION: The authors review the RF and DU in SSc, as well as the use of bosentan, an endotheline receptor antagonist, and its indications. Although it is not formally approved, the use of bosentan in SS has shown benefits in reducing the incidence of DU, and despite no influence in the healing process, this drug prevents the development of new lesions. FAU - Mota, Joana AU - Mota J AD - Serviço de Medicina Interna, Hospitais da Universidade de Coimbra, Portugal. FAU - Castellano, Amparo AU - Castellano A FAU - Santiago, Felicidade AU - Santiago F FAU - Carvalho, Patrícia AU - Carvalho P FAU - Madeira, A Sofia AU - Madeira AS FAU - Pereira De Moura, J M AU - Pereira De Moura JM FAU - Nascimento Costa, J M AU - Nascimento Costa JM LA - por PT - Case Reports PT - Journal Article TT - Úlceras digitais na esclerodermia: papel dos antagonistas dos receptores da endotelina na terap êutica. DEP - 20111229 PL - Portugal TA - Acta Med Port JT - Acta medica portuguesa JID - 7906803 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Bosentan MH - *Endothelin Receptor Antagonists MH - Female MH - Fingers MH - Humans MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/*etiology MH - Sulfonamides/*therapeutic use EDAT- 2012/04/25 06:00 MHDA- 2012/08/14 06:00 CRDT- 2012/04/25 06:00 PHST- 2010/05/14 00:00 [received] PHST- 2010/11/15 00:00 [accepted] PHST- 2012/04/25 06:00 [entrez] PHST- 2012/04/25 06:00 [pubmed] PHST- 2012/08/14 06:00 [medline] PST - ppublish SO - Acta Med Port. 2011 Sep-Oct;24(5):837-42. Epub 2011 Dec 29. PMID- 18587230 OWN - NLM STAT- MEDLINE DCOM- 20080813 LR - 20191110 IS - 1349-7413 (Electronic) IS - 0911-4300 (Linking) VI - 31 IP - 3 DP - 2008 Jun TI - [A case of lupus cystitis in a 74-year-old woman]. PG - 183-9 AB - The patient was a 74-year-old woman. As the history of the present illness, Raynaud's phenomenon appeared in 1998, antinuclear antibody positivity was detected in 2002, and she visited our department for the first time. Leukopenia and positivity for anti-DNA and anti-RNP antibodies were present, but active lesions were not, and thus, course observation was selected. Pollakiuria and a sensation of residual urine appeared in February 2005, diarrhea and nausea developed in November, and she was admitted to our hospital. Abdominal CT detected bilateral hydronephrosis, marked hydroureter, and hypertrophy of the urinary bladder wall, cystoscopy detected trabeculation, and features of interstitial cystitis were noted on biopsy. Edematous colon mucosa was noted on lower endoscopy, submucosal inflammatory cell infiltration on biopsy, and IgG deposition in the small vascular wall on immunostaining. Systemic lupus erythematosus (SLE) that developed as lupus cystitis was diagnosed. The clinical findings were improved by 50 mg of prednisolone. Although she developed lupus cystitis at an elderly age of 74 years, IgG deposition in the small vascular wall was detected by immunostaining of the intestinal mucosa. It is a valuable case proved that causative disease of a digestive tract symptom was enterocolitis through an immune complex as autoimmune reaction by SLE immunohistologically. There are 46 cases of lupus cystitis in Japan by 2007 since Kato reported lupus cystitis in 1985. We summarize clinical features of 46 cases and discuss difference with this case. FAU - Yukawa, Sonosuke AU - Yukawa S AD - Department of Rheumatology, Tokyo Medical University, Tokyo, Japan. FAU - Tahara, Koichiro AU - Tahara K FAU - Hayashi, Haeru AU - Hayashi H FAU - Tsuji, Soichiro AU - Tsuji S FAU - Shoji, Aki AU - Shoji A FAU - Tsuboi, Norioki AU - Tsuboi N LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Nihon Rinsho Meneki Gakkai Kaishi JT - Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology JID - 9505992 SB - IM MH - Aged MH - Cystitis/*etiology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications EDAT- 2008/07/01 09:00 MHDA- 2008/08/14 09:00 CRDT- 2008/07/01 09:00 PHST- 2008/07/01 09:00 [pubmed] PHST- 2008/08/14 09:00 [medline] PHST- 2008/07/01 09:00 [entrez] AID - JST.JSTAGE/jsci/31.183 [pii] AID - 10.2177/jsci.31.183 [doi] PST - ppublish SO - Nihon Rinsho Meneki Gakkai Kaishi. 2008 Jun;31(3):183-9. doi: 10.2177/jsci.31.183. PMID- 36117395 OWN - NLM STAT- MEDLINE DCOM- 20230103 LR - 20230103 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 26 IP - 1 DP - 2023 Jan TI - Two clusters of systemic lupus erythematosus patients with muscle involvement in a Chinese cohort. PG - 51-59 LID - 10.1111/1756-185X.14443 [doi] AB - OBJECTIVES: This study aimed to depict the clinical features, including myositis specific or associated antibody (MSA/MAA) profile of systemic lupus erythematosus (SLE) patients with muscle involvement in a Chinese cohort. METHODS: We retrospectively studied a cohort of 1696 SLE inpatients and screened for concurrent myositis features from January 2013 to June 2021. Propensity score matching was applied to enroll controls without myositis features from our cohort. Demographic, clinical and laboratory variables were collected. MSA/MAA panels containing 16 autoantibodies (TIF1-γ, MDA5, NXP2, Mi-2α/β, SAE1, Jo-1, PL-7, PL-12, EJ, OJ, SRP, HMGCR, cN-1A, PM-Scl75/100, Ku and Ro52) were tested by line-blotting assay. Binary logistic regression and K-means clustering were applied. RESULTS: Forty-one of 1696 (2.42%) SLE patients in our SLE inpatient cohort showed features of myositis. Binary logistic regression revealed that new-onset SLE (odds ratio [OR] = 4.77, 95% CI = 1.10-20.57), interstitial lung disease (ILD) (OR = 10.07, 95% CI = 1.65-61.51), positive anti-U1RNP antibody (OR = 4.38, 95% CI = 1.08-17.75), and Raynaud's phenomenon (OR = 7.94, 95% CI = 1.41-44.69) were associated with muscle involvement. Except for anti-Ro52 (50%), anti-Ku antibody (38.2%) was the next frequently detected MSA/MAA in the panel, followed by anti-NXP2 antibody (11.8%). It was noteworthy that multiple MSA/MAAs (≥2, excluding anti-Ro52) coexisted in 9 patients. Patients with myositis features were clustered into 2 subgroups. Cluster 1 was characterized by anti-Ku or anti-Ro52 with high SLE Disease Activity Index, whereas cluster 2 presented with anti-U1RNP, Raynaud's phenomenon and pulmonary arterial hypertension resembling mixed connective tissue disease. CONCLUSION: In our Chinese SLE inpatient cohort, muscle involvement was infrequent. Nevertheless, distinct features in these SLE patients deserve further study. CI - © 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Zhao, Liling AU - Zhao L AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Huang, Wenyan AU - Huang W AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Wang, Haiting AU - Wang H AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Chen, Jie AU - Chen J AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Zhang, Danting AU - Zhang D AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Wang, Xiaodong AU - Wang X AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Sun, Fangfang AU - Sun F AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. FAU - Ye, Shuang AU - Ye S AD - Department of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China. LA - eng GR - 202040291/Shanghai Municipal Health Commission/ GR - SHDC2020CR1015B/Shanghai Hospital Development Center/ PT - Journal Article DEP - 20220918 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Retrospective Studies MH - East Asian People MH - *Myositis/diagnosis MH - Autoantibodies MH - *Lupus Erythematosus, Systemic/diagnosis MH - Antibodies, Antinuclear MH - Muscles OTO - NOTNLM OT - autoantibodies OT - myositis OT - systemic lupus erythematosus EDAT- 2022/09/20 06:00 MHDA- 2023/01/04 06:00 CRDT- 2022/09/19 02:49 PHST- 2022/08/25 00:00 [revised] PHST- 2022/05/30 00:00 [received] PHST- 2022/09/07 00:00 [accepted] PHST- 2022/09/20 06:00 [pubmed] PHST- 2023/01/04 06:00 [medline] PHST- 2022/09/19 02:49 [entrez] AID - 10.1111/1756-185X.14443 [doi] PST - ppublish SO - Int J Rheum Dis. 2023 Jan;26(1):51-59. doi: 10.1111/1756-185X.14443. Epub 2022 Sep 18. PMID- 31254002 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20210110 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 39 IP - 11 DP - 2019 Nov TI - Targeting very early systemic sclerosis: a case-based review. PG - 1961-1970 LID - 10.1007/s00296-019-04357-x [doi] AB - It is unknown whether treatment in very early/early systemic sclerosis (SSc) can affect long-term outcomes. A case-based review was conducted (i) to assess the effect of rituximab (RTX) in very early SSc and (ii) to explore how many clinical trials in SSc targeted early disease and whether treatment of these patients led to better clinical outcomes. We identified cases of very early SSc from our department and performed a search in MEDLINE and Scopus databases for clinical trials in SSc during 2005-2018. Two cases are reported where RTX was administered within 24 months from the appearance of Raynaud's. In the first case, there was an improvement in interstitial lung disease as indicated by the improvement in pulmonary function tests and the regression of changes in high-resolution chest computed tomography. In the second case, a good clinical response in skin fibrosis was observed. The review revealed the following: (i) only one-third of the studies were specifically designed to target early disease, (ii) there is confusion related to disease duration definition across SSc clinical trials but an obvious trend towards improvement was evident during the past years, (iii) the question of whether early implementation of therapy may lead to better clinical outcomes cannot be definitely answered based on existing data and (iv) there is still a very low level of incorporation of the new classification criteria in SSc trials. This review suggests that there may be a window of opportunity in SSc and highlights the need for clinical trials targeting very early/early disease. FAU - Melissaropoulos, Konstantinos AU - Melissaropoulos K AUID- ORCID: 0000-0002-4891-0623 AD - Department of Rheumatology, Patras University Hospital, University of Patras Medical School, Patras, Greece. FAU - Kraniotis, Pantelis AU - Kraniotis P AUID- ORCID: 0000-0001-9149-1586 AD - Department of Radiology, Patras University Hospital, Patras, Greece. FAU - Bogdanos, Dimitrios AU - Bogdanos D AUID- ORCID: 0000-0002-9697-7902 AD - Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110, Larissa, Greece. FAU - Dimitroulas, Theodoros AU - Dimitroulas T AUID- ORCID: 0000-0002-0364-1642 AD - 4th Department of Internal Medicine Hippokration Hospital, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece. FAU - Sakkas, Lazaros AU - Sakkas L AUID- ORCID: 0000-0002-7670-3314 AD - Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110, Larissa, Greece. FAU - Daoussis, Dimitrios AU - Daoussis D AUID- ORCID: 0000-0001-8027-3655 AD - Department of Rheumatology, Patras University Hospital, University of Patras Medical School, Patras, Greece. jimdaoussis@hotmail.com. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20190628 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Adult MH - Female MH - Humans MH - Immunologic Factors/*therapeutic use MH - Middle Aged MH - Rituximab/*therapeutic use MH - Scleroderma, Systemic/diagnosis/*drug therapy MH - Treatment Outcome OTO - NOTNLM OT - Early systemic sclerosis OT - Rituximab OT - Scleroderma OT - Systemic sclerosis EDAT- 2019/06/30 06:00 MHDA- 2020/04/28 06:00 CRDT- 2019/06/30 06:00 PHST- 2019/03/22 00:00 [received] PHST- 2019/06/21 00:00 [accepted] PHST- 2019/06/30 06:00 [pubmed] PHST- 2020/04/28 06:00 [medline] PHST- 2019/06/30 06:00 [entrez] AID - 10.1007/s00296-019-04357-x [pii] AID - 10.1007/s00296-019-04357-x [doi] PST - ppublish SO - Rheumatol Int. 2019 Nov;39(11):1961-1970. doi: 10.1007/s00296-019-04357-x. Epub 2019 Jun 28. PMID- 40255739 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250422 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 3 DP - 2025 Mar TI - Predictors of Vasculitis in Patients With Systemic Lupus Erythematosus in Bangladesh. PG - e80868 LID - 10.7759/cureus.80868 [doi] LID - e80868 AB - Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Sometimes vasculitis may complicate the disease. Among the manifestations of SLE, vasculitis presentation is common. This study aimed to identify the predictors of vasculitis in SLE patients. Methods This is a cross-sectional study in a single center conducted in the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from December 2019 to January 2021. The disease activity and damage were assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR DI). Study subjects were grouped into vasculitis and no vasculitis groups. The multivariate logistic regression analysis was done to determine the independent predictors of vasculitis in SLE. The p-value <0.05 was considered significant. Results The rate of lupus vasculitis was 14.3%. The significant difference in vasculitis features between vasculitis and no vasculitis groups were: acute cutaneous lupus erythematosus (ACLE) (p<0.001), oral ulcer (p<0.001), alopecia (p<0.001), Raynaud's phenomenon (p=0.011), fever (p=0.002), arthritis (p<0.001), pregnancy loss (p=0.003), lupus nephritis (p=0.032), seizure (p=0.027), pleurisy (p=0.027), leucopenia (p=0.049), anti-dsDNA positivity (p=0.008), hypocomplementemia (p=0.003), higher mean SLEDAI (p<0.001) and SLICC/ACR DI score (p<0.001). In multivariate logistic regression analysis, higher SLEDAI score (OR = 1.296, 95% CI =1.114-1.508) was positively and lupus nephritis (OR= 0.055, 95% CI =0.007-0.413) was negatively associated with lupus vasculitis. Conclusion The vasculitis flare of lupus is associated with Raynaud's phenomenon and pregnancy loss. Mucocutaneous flare, fever, arthritis, seizure, pleurisy, and lupus nephritis were also associated with vasculitis. CI - Copyright © 2025, Sunny et al. FAU - Sunny, Shadab Saud AU - Sunny SS AD - Rheumatology, Comilla General Hospital, Comilla, BGD. FAU - Azad, Mohammad Abul Kalam AU - Azad MAK AD - Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, BGD. FAU - Hassan, M Masudul AU - Hassan MM AD - Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, BGD. FAU - Asaduzzaman, Atm AU - Asaduzzaman A AD - Dermatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, BGD. FAU - Islam, Md Nazrul AU - Islam MN AD - Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, BGD. LA - eng PT - Journal Article DEP - 20250320 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12008815 OTO - NOTNLM OT - cutaneous vasculitis OT - flare of lupus OT - lupus flare OT - lupus vasculitis OT - rheumatoid arthritis OT - sle OT - sle and lupus nephritis OT - vasculitis OT - vasculitis and disease activity COIS- Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Institutional Review Board (IRB) of Bangabandhu Sheikh Mujib Medical University (BSMMU) issued approval BSMMU/2019/13905. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/04/21 06:26 MHDA- 2025/04/21 06:27 PMCR- 2025/03/20 CRDT- 2025/04/21 05:21 PHST- 2025/03/20 00:00 [accepted] PHST- 2025/04/21 06:27 [medline] PHST- 2025/04/21 06:26 [pubmed] PHST- 2025/04/21 05:21 [entrez] PHST- 2025/03/20 00:00 [pmc-release] AID - 10.7759/cureus.80868 [doi] PST - epublish SO - Cureus. 2025 Mar 20;17(3):e80868. doi: 10.7759/cureus.80868. eCollection 2025 Mar. PMID- 24851680 OWN - NLM STAT- MEDLINE DCOM- 20150205 LR - 20140523 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 94 IP - 13 DP - 2014 Apr 8 TI - [Systemic lupus erythematosus associated pulmonary arterial hypertension: clinical analysis of 91 cases]. PG - 969-72 AB - OBJECTIVE: To explore the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH). METHODS: A total of 91 cases of SLE-PAH from 2007 to 2011 were reviewed and followed up. They were divided into 2 groups: group A: New York Heart Association (NYHA) functional class 1 and 2; group B: NYHA functional class 3 and 4. RESULTS: There were 2 males and 89 females with a mean age of 37 ± 11 years. The mean duration of SLE disease process was 7 ± 6 years. PAH was the primary symptom of SLE onset in 10 cases. Pulmonary arterial systolic pressure (PASP) as measured by ultrasonic cardiography (UCG) were between 40 to 128 mmHg. Eighteen cases (19.78%) underwent right heart catheterization (RHC). There were good parallels of PASP value between RHC and UCG. The main characteristics included Raynaud's phenomenon (53.8%), pericardial effusion (51.6%) and a high titer of anti-RNP antibody (57.1%). PASP was positively associated with SLE disease activity in mild and moderate cases. Among 27 mortality cases, there were 4 in group A (14.8%) and 23 in group B (85.2%) . And the causes of immortality were mostly non-cardiac in group A and right heart failure in group B. Cyclophosphamide was effective in mild and moderate cases. Forty-four cases received PAH target treatment and it could decrease the PASP in mild and moderate cases and significantly prolong the survival for severe cases. CONCLUSION: The major clinical characteristics of SLE-PAH patients include Raynaud's phenomenon, pericardial effusion and positivity of anti-RNP antibody. PASP is positively associated with SLE disease activity in mild and moderate cases for whom intravenous pulse cyclophosphamide therapy may be effective. For severe cases, concomitant PAH target therapy may significantly improve the prognosis. FAU - Teng, Jialin AU - Teng J AD - Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200001, China. FAU - Zhang, Wei AU - Zhang W AD - Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200001, China. Email: eyysb@126.com. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 SB - IM MH - Adult MH - Familial Primary Pulmonary Hypertension/complications/*diagnosis/*therapy MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/complications/*diagnosis/*therapy MH - Male MH - Middle Aged MH - Prognosis MH - Treatment Outcome EDAT- 2014/05/24 06:00 MHDA- 2015/02/06 06:00 CRDT- 2014/05/24 06:00 PHST- 2014/05/24 06:00 [entrez] PHST- 2014/05/24 06:00 [pubmed] PHST- 2015/02/06 06:00 [medline] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2014 Apr 8;94(13):969-72. PMID- 40548108 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250625 IS - 2234-943X (Print) IS - 2234-943X (Electronic) IS - 2234-943X (Linking) VI - 15 DP - 2025 TI - Successful treatment with carfilzomib and dexamethasone for relapsed/refractory POEMS syndrome: a case report and review of literature. PG - 1570981 LID - 10.3389/fonc.2025.1570981 [doi] LID - 1570981 AB - BACKGROUND: POEMS syndrome is a rare multisystem disease secondary to plasma cell neoplasm. Due to its rarity, there are no internationally agreed treatment standards, with very limited data to guide management in the relapse setting. CASE PRESENTATION: We describe a 51-year-old woman with initially presented with fatigue, anorexia, nausea, abdominal distension, and edema of the face and both lower limbs, who was diagnosed with POEMS syndrome accompanied with Raynaud's phenomenon and cardiac involvement. After multiple lines of treatment, including bortezomib, cyclophosphamide, and dexamethasone (VCD), ixazomib, and daratumumab along with dexamethasone (DD), her clinical and laboratory features, and cardiovascular system continued to deteriorate. Then we started carfilzomib and dexamethasone, and the patient achieved a complete response. She did not develop significant cardiac toxicity and peripheral neuropathy. A total of 4 cycles of carfilzomib and dexamethasone were administered monthly, followed by autologous stem cell transplantation (ASCT). After 4 months of follow-up, a complete remission persists, and no significant complications were observed. CONCLUSION: We report on the first case of relapsed/refractory POEMS syndrome who received carfilzomib and dexamethasone, and achieved very good remission. Carfilzomib may be a safe and effective treatment option for patients with relapsed/refractory POEMS syndrome. CI - Copyright © 2025 Yu, Li, Peng, Wang and Li. FAU - Yu, Shuang AU - Yu S AD - Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. FAU - Li, Xiangxin AU - Li X AD - Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. FAU - Peng, Jun AU - Peng J AD - Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. FAU - Wang, Luqun AU - Wang L AD - Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. FAU - Li, Hao AU - Li H AD - Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20250606 PL - Switzerland TA - Front Oncol JT - Frontiers in oncology JID - 101568867 PMC - PMC12179133 OTO - NOTNLM OT - POEMS syndrome OT - VEGF OT - adverse events OT - carfilzomib OT - peripheral neuropathy COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2025/06/24 10:51 MHDA- 2025/06/24 10:52 PMCR- 2025/06/06 CRDT- 2025/06/23 06:19 PHST- 2025/02/04 00:00 [received] PHST- 2025/05/20 00:00 [accepted] PHST- 2025/06/24 10:52 [medline] PHST- 2025/06/24 10:51 [pubmed] PHST- 2025/06/23 06:19 [entrez] PHST- 2025/06/06 00:00 [pmc-release] AID - 10.3389/fonc.2025.1570981 [doi] PST - epublish SO - Front Oncol. 2025 Jun 6;15:1570981. doi: 10.3389/fonc.2025.1570981. eCollection 2025. PMID- 34630837 OWN - NLM STAT- MEDLINE DCOM- 20211019 LR - 20211019 IS - 1937-8688 (Electronic) VI - 39 DP - 2021 TI - Systemic sclerosis manifesting as intractable gastro-oesophageal reflux and diarrhoea: a case report from Kenya. PG - 225 LID - 10.11604/pamj.2021.39.225.29771 [doi] LID - 225 AB - Systemic sclerosis is a rare condition that has not been well reported in Africa, and several multisystemic manifestations, including gastrointestinal ones, have not been well documented locally. We present an unusual case of persistent gastro-oesophageal reflux and diarrhoea in a 74-year-old Kenyan female, who progressively developed abdominal distention, dysphagia and Raynaud´s phenomenon. Stool tests were unremarkable, whereas antinuclear antibody, ribonucleoproteins antibody (anti-nRNP/Sm) and anti-Sjögren's-syndrome-related antigen A autoantibody (anti-SSA) tests were positive. Endoscopic and imaging investigations revealed features of gastrointestinal dysmotility including reflux oesophagitis, gastroparesis and chronic intestinal pseudo-obstruction. A diagnosis of systemic sclerosis was made, and she responded well to medical treatment. We present this case to contribute to the limited literature of a disease associated with high morbidity and mortality, as well as encourage fellow clinicians to have a high level of suspicion in their differentials of persistent gastrointestinal dysmotility. CI - Copyright: Gloria Wangechi Mugo et al. FAU - Mugo, Gloria Wangechi AU - Mugo GW AD - Gastro and Liver Centre, Nairobi, Kenya. FAU - Murunga, Eric Mwenda AU - Murunga EM AD - Gastro and Liver Centre, Nairobi, Kenya. LA - eng PT - Case Reports PT - Journal Article DEP - 20210803 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 SB - IM MH - Aged MH - Deglutition Disorders/etiology MH - Diarrhea/*etiology MH - Female MH - Gastroesophageal Reflux/*etiology MH - Humans MH - Kenya MH - Scleroderma, Systemic/complications/*diagnosis/therapy PMC - PMC8486939 OTO - NOTNLM OT - Kenya OT - Systemic sclerosis OT - case report OT - gastrointestinal system COIS- The authors declare no competing interests. EDAT- 2021/10/12 06:00 MHDA- 2021/10/21 06:00 PMCR- 2021/08/03 CRDT- 2021/10/11 05:59 PHST- 2021/05/10 00:00 [received] PHST- 2021/07/15 00:00 [accepted] PHST- 2021/10/11 05:59 [entrez] PHST- 2021/10/12 06:00 [pubmed] PHST- 2021/10/21 06:00 [medline] PHST- 2021/08/03 00:00 [pmc-release] AID - PAMJ-39-225 [pii] AID - 10.11604/pamj.2021.39.225.29771 [doi] PST - epublish SO - Pan Afr Med J. 2021 Aug 3;39:225. doi: 10.11604/pamj.2021.39.225.29771. eCollection 2021. PMID- 18174638 OWN - NLM STAT- MEDLINE DCOM- 20080715 LR - 20220211 IS - 0019-5359 (Print) IS - 0019-5359 (Linking) VI - 61 IP - 12 DP - 2007 Dec TI - Novel phosphodiesterase-5 inhibitors: current indications and future directions. PG - 667-79 AB - Cardiovascular diseases like hypertension, hyperlipidemia, diabetes mellitus and obesity are the important predictors of erectile dysfunction (ED). Endothelial dysfunction is proposed to be the underlying cause of ED, just like coronary artery disease. Sildenafil was originally developed to treat angina pectoris but later on was recognized as novel treatment option for impotence. To date, sildenafil has been the most extensively studied PDE (phosphodiesterase)-5 inhibitor. Currently two more PDE-5 inhibitors, tadalafil and vardenafil, are under study. Newer compounds have certain advantages over sildenafil, including greater selectivity for PDE-5 compared with other isoenzymes, absence of effect of food on absorption, faster onset and longer duration of action. PDE-5 inhibitors are emerging as novel therapeutic tools with a potential to protect or enhance endothelial function in humans and to selectively improve regional blood flow. The FDA has recently approved a reformulation of sildenafil for the treatment of pulmonary arterial hypertension. Raynaud's phenomenon, respiratory disorders with ventilation/ perfusion mismatch, congestive cardiac failure, hypertension and stroke are the other conditions in which PDE-5 inhibitors are being tried. It is hoped that this group of drugs will soon emerge as a novel weapon in the armamentarium against various cardiovascular and pulmonary diseases. FAU - Sharma, Rashmi AU - Sharma R AD - Department of Pharmacology and Therapeutics, Govt. Medical College, Jammu, J and K, India. rashmichams@yahoo.com LA - eng PT - Journal Article PT - Review PL - India TA - Indian J Med Sci JT - Indian journal of medical sciences JID - 0373023 RN - 0 (Phosphodiesterase 5 Inhibitors) SB - IM MH - Cardiovascular Diseases/chemically induced MH - Clinical Trials as Topic MH - Counseling MH - Erectile Dysfunction/drug therapy MH - Forecasting MH - Heart Failure/drug therapy MH - Humans MH - Hypertension, Pulmonary/drug therapy MH - Male MH - *Phosphodiesterase 5 Inhibitors RF - 45 EDAT- 2008/01/05 09:00 MHDA- 2008/07/17 09:00 CRDT- 2008/01/05 09:00 PHST- 2008/01/05 09:00 [pubmed] PHST- 2008/07/17 09:00 [medline] PHST- 2008/01/05 09:00 [entrez] PST - ppublish SO - Indian J Med Sci. 2007 Dec;61(12):667-79. PMID- 16935804 OWN - NLM STAT- MEDLINE DCOM- 20070329 LR - 20070710 IS - 1167-1122 (Print) IS - 1167-1122 (Linking) VI - 16 IP - 4 DP - 2006 Jul-Aug TI - Jo-1 positive paraneoplastic systemic sclerosis in a patient with metastatic melanoma. PG - 428-30 AB - A link between systemic sclerosis (SSc) and malignancy is suggested by epidemiological evidence, but the underlying mechanism connecting both diseases has been a source of ongoing controversy. Here, we describe the first case of paraneoplastic SSc secondary to a primarily diagnosed melanoma. Two months after diagnosis of metastatic melanoma, a 40-year-old female presented with high serum titers of antinuclear antibodies (ANA), but no symptoms of autoimmune disease. Five months later, the onset of Raynaud's phenomenon together with highly positive Jo-1 antibodies was observed. The following clinical course of scleroderma was correlated to melanoma remission and progression. Finally, the patient developed severe pulmonary fibrosis, massive pleural effusion, severe thoracic scleroderma and necrosis of the fingertips, simultaneously with a progression of melanoma to disseminated lymph node metastases and a small brain metastasis. This rare case of SSc concurrent with melanoma suggests that besides other possible mechanisms, paraneoplastic etiology can be responsible for the association between SSc and cancer. FAU - Thoelke, Adina AU - Thoelke A AD - German Cancer Research Center, Skin Cancer Unit, University Hospital Mannheim, Dept. of Dermatology, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim. adina.thoelke@haut.ma.uni-heidelberg.de FAU - Schmid, Hans-Peter AU - Schmid HP FAU - Figl, Robert AU - Figl R FAU - Schadendorf, Dirk AU - Schadendorf D FAU - Ugurel, Selma AU - Ugurel S LA - eng PT - Case Reports PT - Journal Article PL - France TA - Eur J Dermatol JT - European journal of dermatology : EJD JID - 9206420 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) SB - IM MH - Adult MH - Antibodies, Antinuclear/*blood MH - Female MH - Humans MH - Melanoma/*blood/*secondary MH - Neoplasms, Unknown Primary/*blood MH - Paraneoplastic Syndromes/*blood MH - Scleroderma, Systemic/*blood EDAT- 2006/08/29 09:00 MHDA- 2007/03/30 09:00 CRDT- 2006/08/29 09:00 PHST- 2006/01/18 00:00 [accepted] PHST- 2006/08/29 09:00 [pubmed] PHST- 2007/03/30 09:00 [medline] PHST- 2006/08/29 09:00 [entrez] PST - ppublish SO - Eur J Dermatol. 2006 Jul-Aug;16(4):428-30. PMID- 39789667 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250111 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 9 IP - 1 DP - 2025 Jan 9 TI - Antimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort. PG - 5 LID - 10.1186/s41927-025-00455-5 [doi] LID - 5 AB - BACKGROUND: The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S. academic center. METHODS: A retrospective chart review was conducted on dermatomyositis patients hospitalized at our institution between January 2014 and June 2023. The data were analyzed via Fischer's exact test and a t test. RESULTS: Among the 195 dermatomyositis patients reviewed, 22 tested positive for the MDA5 antibody, comprising of thirteen adults and nine pediatric patients. Myositis was significantly more common in pediatric patients than in adult patients (p = 0.002). RP-ILD was more frequently observed in adult patients of African ancestry (including both Black Hispanic and Black non-Hispanic individuals) (p = 0.04). There was a significant association noted between Raynaud's phenomenon and ILD (p = 0.02). The overall mortality rate of 13.6% was more favorable than the previously reported rates of 40-60%. CONCLUSION: This study enhances our understanding of the disease by emphasizing potential racial and demographic variations, as well as delineating the similarities and differences between adult and pediatric populations. CI - © 2025. The Author(s). FAU - Koyoda, Sai K AU - Koyoda SK AD - Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA. FAU - Ezzy, Fatema AU - Ezzy F AD - Jacobi Medical Center, Department of Internal Medicine, Bronx, NY, USA. ezzy.fatema1998@gmail.com. FAU - Wahezi, Dawn AU - Wahezi D AD - Department of Pediatric Rheumatology, Children's Hospital at Montefiore, Bronx, NY, USA. FAU - Kumthekar, Anand AU - Kumthekar A AD - Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA. FAU - Xie, Xianhong AU - Xie X AD - Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, NY, USA. FAU - Tagoe, Clement E AU - Tagoe CE AD - Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA. FAU - Ayesha, Bibi AU - Ayesha B AD - Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA. LA - eng PT - Journal Article DEP - 20250109 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC11715331 OTO - NOTNLM OT - Dermatomyositis OT - Interstitial lung disease OT - MDA5 autoantibody OT - Race & ethnicity COIS- Declarations. Ethics approval and consent to participate: The study was conducted in accordance with the Declaration of Helsinki and was approved by the Montefiore/Albert Einstein College of Medicine Institutional Review Board. As this retrospective chart review study did not involve any active intervention, the requirement for consent to participate was waived. Clinical trial number: Not applicable. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2025/01/10 00:21 MHDA- 2025/01/10 00:22 PMCR- 2025/01/09 CRDT- 2025/01/09 23:44 PHST- 2024/09/13 00:00 [received] PHST- 2025/01/01 00:00 [accepted] PHST- 2025/01/10 00:22 [medline] PHST- 2025/01/10 00:21 [pubmed] PHST- 2025/01/09 23:44 [entrez] PHST- 2025/01/09 00:00 [pmc-release] AID - 10.1186/s41927-025-00455-5 [pii] AID - 455 [pii] AID - 10.1186/s41927-025-00455-5 [doi] PST - epublish SO - BMC Rheumatol. 2025 Jan 9;9(1):5. doi: 10.1186/s41927-025-00455-5. PMID- 38881966 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240618 IS - 2169-7574 (Print) IS - 2169-7574 (Electronic) IS - 2169-7574 (Linking) VI - 12 IP - 6 DP - 2024 Jun TI - Toxin for Treating Raynaud Conditions in Hands (The TORCH Study): A Systematic Review and Meta-analysis. PG - e5885 LID - 10.1097/GOX.0000000000005885 [doi] LID - e5885 AB - BACKGROUND: Raynaud disease of the hands is a complex disorder resulting in inappropriate constriction and/or insufficient dilation in microcirculation. There is an emerging role for botulinum toxin type A (BTX-A) in the treatment armamentarium for refractory Raynaud disease. The aim of this systematic review was to critically evaluate the management of primary and secondary Raynaud disease treated with BTX-A intervention. METHODS: We performed a Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant systematic review of clinical studies assessing treatment of primary or secondary Raynaud disease with BTX-A by searching Ovid MEDLINE and Embase databases from inception to first August 2023. The review protocol was prospectively registered on the PROSPERO database (CRD42022312253). RESULTS: Our search strategy identified 288 research articles, of which 18 studies [four randomized controlled trials (RCTs), two non-RCTs, five case series, and seven retrospective cohort studies] were eligible for analysis. Meta-analysis demonstrated that the probability of pain visual analog scale score improvement with BTX-A intervention was 81.95% [95% confidence interval (74.12-87.81) P = 0.19, heterogeneity I (2) = 26%] and probability of digital ulcer healing was 79.37% [95% confidence interval (62.45-89.9) P = 0.02, heterogeneity I (2) = 56%]. CONCLUSIONS: Delivery of BTX-A to digital vessels in the hand may be an effective management strategy for primary and secondary Raynaud disease. A definitive, appropriately-powered RCT with objective functional and patient-reported outcome measures is required to accurately assess and quantify the efficacy of BTX-A in Raynaud disease of the hands. CI - Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. FAU - Geary, Ellen AU - Geary E AD - From the Department of Plastic and Reconstructive Surgery, Beaumont Hospital, Dublin, Ireland. AD - Department of Surgery, Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - Wormald, Justin C R AU - Wormald JCR AD - Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, United Kingdom. AD - Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom. FAU - Cronin, Kevin J AU - Cronin KJ AD - Department of Plastic and Reconstructive Surgery, Mater Misericordiae University Hospital, Dublin, Ireland. FAU - Giele, Henk P AU - Giele HP AD - Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom. FAU - Durcan, Laura AU - Durcan L AD - Department of Rheumatology, Beaumont Hospital, Dublin, Ireland. FAU - Kennedy, Oran AU - Kennedy O AD - Department of Anatomy and Regenerative Medicine, Tissue Engineering Research Group, Royal College of Surgeons in Ireland, Dublin, Ireland. AD - Advanced Materials and Bioengineering Research Centre (AMBER), Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - O'Brien, Fergal AU - O'Brien F AD - Department of Anatomy and Regenerative Medicine, Tissue Engineering Research Group, Royal College of Surgeons in Ireland, Dublin, Ireland. FAU - Dolan, Roisin T AU - Dolan RT AD - From the Department of Plastic and Reconstructive Surgery, Beaumont Hospital, Dublin, Ireland. AD - Department of Surgery, Royal College of Surgeons in Ireland, Dublin, Ireland. LA - eng PT - Journal Article DEP - 20240614 PL - United States TA - Plast Reconstr Surg Glob Open JT - Plastic and reconstructive surgery. Global open JID - 101622231 PMC - PMC11177805 COIS- The authors have no financial interest to declare in relation to the content of this article. EDAT- 2024/06/17 06:43 MHDA- 2024/06/17 06:44 PMCR- 2024/06/14 CRDT- 2024/06/17 05:51 PHST- 2023/12/28 00:00 [received] PHST- 2024/04/22 00:00 [accepted] PHST- 2024/06/17 06:44 [medline] PHST- 2024/06/17 06:43 [pubmed] PHST- 2024/06/17 05:51 [entrez] PHST- 2024/06/14 00:00 [pmc-release] AID - GOX-D-23-01198 [pii] AID - 10.1097/GOX.0000000000005885 [doi] PST - epublish SO - Plast Reconstr Surg Glob Open. 2024 Jun 14;12(6):e5885. doi: 10.1097/GOX.0000000000005885. eCollection 2024 Jun. PMID- 25888844 OWN - NLM STAT- MEDLINE DCOM- 20160208 LR - 20220408 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 9 DP - 2015 Apr 15 TI - Mechanic's hands in a woman with undifferentiated connective tissue disease and interstitial lung disease--anti-PL7 positive antisynthetase syndrome: a case report. PG - 82 LID - 10.1186/s13256-015-0571-2 [doi] LID - 82 AB - INTRODUCTION: Interstitial lung disease can be idiopathic or occur in the setting of connective tissue diseases. In the latter case it requires a different treatment approach with a better prognosis. Interstitial lung disease can precede the onset of typical connective tissue disease features by many years, and therefore meticulous multidisciplinary follow-up is crucial. This case highlights the diagnostic challenge and the need for intensified attention for subtle clinical features when faced with interstitial lung disease in patients with characteristics of a hitherto undifferentiated connective tissue disease. CASE PRESENTATION: A 44-year-old Caucasian woman presented to our pulmonology department with dyspnea, Raynaud's phenomenon and subtle swelling of fingers and eyelids. Laboratory analysis and autoantibody screening was negative. She was diagnosed with nonspecific interstitial pneumonia with a concurring undifferentiated connective tissue disease. After four years of stable disease, she presented with rapid pulmonary deterioration, myalgia, periorbital edema, arthritis and a cracked appearance of the radial sides of the fingers of both her hands. This clinical sign was recognized as mechanic's hands and a specific search for the presence of antisynthetase antibodies was performed. She was found to harbor anti-threonyl-tRNA synthetase antibodies. A diagnosis of antisynthetase syndrome was made and she was treated with glucocorticoids and immunosuppressives. CONCLUSIONS: This case highlights the difficulty in fine-tuning the diagnosis when confronted with a patient with interstitial lung disease and the suspicion of an underlying, yet undifferentiated connective tissue disease. There is a strong need for clinical multidisciplinary follow-up of these patients, with a high level of alertness to rare and specific clinical signs. The diagnosis of the underlying connective tissue disease profoundly influences the management of the interstitial lung disease. Recent data stress that identification of the autoantibody specificity allows for further prognostic stratification and therefore should be pursued. FAU - De Langhe, Ellen AU - De Langhe E AD - Department of Rheumatology, University Hospitals Leuven, Herestraat 49, Leuven, 3000, Belgium. ellen.delanghe@uzleuven.be. FAU - Lenaerts, Jan AU - Lenaerts J AD - Department of Rheumatology, University Hospitals Leuven, Herestraat 49, Leuven, 3000, Belgium. jan.lenaerts@skynet.be. FAU - Bossuyt, Xavier AU - Bossuyt X AD - Department of Laboratory Medicine, University Hospitals Leuven, Herestraat 49, Leuven, 3000, Belgium. xavier.bossuyt@uzleuven.be. FAU - Westhovens, Rene AU - Westhovens R AD - Department of Rheumatology, University Hospitals Leuven, Herestraat 49, Leuven, 3000, Belgium. rene.westhovens@uzleuven.be. FAU - Wuyts, Wim A AU - Wuyts WA AD - Department of Pulmonary Medicine, University Hospitals Leuven, Herestraat 49, Leuven, 3000, Belgium. wim.wuyts@uzleuven.be. LA - eng PT - Case Reports PT - Journal Article DEP - 20150415 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - EC 6.1.1.3 (Threonine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Autoantibodies/blood MH - Connective Tissue Diseases/*diagnosis MH - Delayed Diagnosis MH - Female MH - Hand/pathology MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/*diagnosis MH - Myositis/*diagnosis/drug therapy MH - Threonine-tRNA Ligase/*immunology PMC - PMC4407325 EDAT- 2015/04/19 06:00 MHDA- 2016/02/09 06:00 PMCR- 2015/04/15 CRDT- 2015/04/19 06:00 PHST- 2014/11/03 00:00 [received] PHST- 2015/03/12 00:00 [accepted] PHST- 2015/04/19 06:00 [entrez] PHST- 2015/04/19 06:00 [pubmed] PHST- 2016/02/09 06:00 [medline] PHST- 2015/04/15 00:00 [pmc-release] AID - 10.1186/s13256-015-0571-2 [pii] AID - 571 [pii] AID - 10.1186/s13256-015-0571-2 [doi] PST - epublish SO - J Med Case Rep. 2015 Apr 15;9:82. doi: 10.1186/s13256-015-0571-2. PMID- 35287403 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220317 IS - 2229-5178 (Print) IS - 2249-5673 (Electronic) IS - 2229-5178 (Linking) VI - 13 IP - 2 DP - 2022 Mar-Apr TI - Nailfold Capillaroscopic Changes as a Marker of Interstitial Lung Disease in Systemic Sclerosis: A Cross-Sectional Study in a Tertiary Care Hospital in Eastern India. PG - 216-220 LID - 10.4103/idoj.idoj_393_21 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune connective tissue disorder causing microvascular abnormality leading to Raynaud's phenomenon, skin tightening, and nailfold capillary changes. The patient may have systemic involvement, among them interstitial lung disease (ILD) and pulmonary arterial hypertension are mainly associated with disease-related mortality. AIMS: To find out an association between nailfold capillaroscopic changes with ILD severity. MATERIALS AND METHODS: An institution based cross-sectional study was performed among the patients of SSc above 18 years. Detailed history was taken. Clinical examinations, nailfold capillaroscopy (NFC) with a dermatoscope and high-resolution computed tomography (HRCT) scan of thorax were done. Data were analyzed in MedCalc statistical software version 20. RESULTS: Out of total 43 patients, 74.4% (n = 32) were female. Mean age was 35.05 ± 7.24 years and mean duration 4.28 ± 2.81 years. Diffuse SSc was found in 88.37% (n = 38). On NFC, early, active, and late patterns were found in 30.2% (n = 13), 25.6% (n = 11), and 44.2% (n = 19) cases, respectively. On HRCT, early, active, and late ILD were present in 18.6% (n = 8), 37.2% (n = 16), and 30.2% (n = 13) cases, respectively. ILD changes were absent in 14% (n = 6) though NFC changes were present in them. Respiratory symptoms were absent in 20.93% (n = 9) patients though all had features of early NFC and among them 9.3% (n = 4) showed early ILD changes on HRCT. Significant associations were found between NFC changes and ILD severity (P = 0.0003), NFC changes and respiratory symptoms (P < 0.0001) and between ILD changes and respiratory symptoms (P < 0.0001). DISCUSSION: NFC, an inexpensive procedure, can be performed in all patients of SSc to detect development of early ILD even before appearance of respiratory symptoms to prevent further progression. CI - Copyright: © 2022 Indian Dermatology Online Journal. FAU - Rudra, Olympia AU - Rudra O AD - Department of Dermatology, IPGME&R, Kolkata, West Bengal, India. FAU - Baisya, Subhasmita AU - Baisya S AD - Department of Dermatology, IPGME&R, Kolkata, West Bengal, India. FAU - Mallick, Subhadeep AU - Mallick S AD - Department of Dermatology, IPGME&R, Kolkata, West Bengal, India. FAU - Chatterjee, Gobinda AU - Chatterjee G AD - Department of Dermatology, IPGME&R, Kolkata, West Bengal, India. LA - eng PT - Journal Article DEP - 20220303 PL - India TA - Indian Dermatol Online J JT - Indian dermatology online journal JID - 101586880 PMC - PMC8917487 OTO - NOTNLM OT - Interstitial lung disease OT - nailfold capillaroscopy OT - systemic sclerosis COIS- There are no conflicts of interest. EDAT- 2022/03/16 06:00 MHDA- 2022/03/16 06:01 PMCR- 2022/03/03 CRDT- 2022/03/15 05:09 PHST- 2021/06/19 00:00 [received] PHST- 2021/10/23 00:00 [revised] PHST- 2021/10/30 00:00 [accepted] PHST- 2022/03/15 05:09 [entrez] PHST- 2022/03/16 06:00 [pubmed] PHST- 2022/03/16 06:01 [medline] PHST- 2022/03/03 00:00 [pmc-release] AID - IDOJ-13-216 [pii] AID - 10.4103/idoj.idoj_393_21 [doi] PST - epublish SO - Indian Dermatol Online J. 2022 Mar 3;13(2):216-220. doi: 10.4103/idoj.idoj_393_21. eCollection 2022 Mar-Apr. PMID- 17516621 OWN - NLM STAT- MEDLINE DCOM- 20070830 LR - 20221207 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 34 IP - 6 DP - 2007 Jun TI - Ethnic disparities among patients with pulmonary hypertension associated with systemic sclerosis. PG - 1277-82 AB - OBJECTIVE: To examine a cohort of patients with systemic sclerosis (SSc) and pulmonary hypertension (PH) for ethnic disparities in clinical presentation, disease detection, or management. METHODS: Encounters of patients with SSc seen at the Medical University of South Carolina were recorded in a computerized database from November 1997 through January 2004. Patients were evaluated for discrepancy in disease manifestation and treatment. Evaluation criteria included patient ethnicity (by self report), age, disease duration from onset of first non-Raynaud's symptom, presence or absence of PH, incidence of diastolic dysfunction and left ventricular hypertrophy among patients with PH, severity of interstitial lung disease, and treatment course. RESULTS: African Americans were more likely than Caucasians to have diffuse cutaneous SSc (dcSSc) (69.9% vs 42.9%, p < 0.001) and they presented with PH (defined as right ventricular systolic pressure > 40 mm Hg by echocardiogram or mean pulmonary artery pressure > 25 mm Hg by right heart catheterization (RHC) at a younger age (60.9 yrs vs 49.0 yrs, p < 0.001). There were no ethnic disparities in time from onset of the first non-Raynaud's symptom to detection of PH, method of PH detection, or treatment modalities. Patients with PH were more likely to have diastolic dysfunction than those without PH (52.3% vs 35.9%, p = 0.011). CONCLUSION: In this cohort of patients, African Americans were more likely to have dcSSc. Among patients with PH, African Americans presented at a younger age than their Caucasian counterparts. Incidence of diastolic dysfunction was higher in the PH population. There were no significant ethnic disparities in time of progression to PH or in treatment modalities employed in our cohort. FAU - Beall, Ashley D AU - Beall AD AD - Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina 29425-2229, USA. beall@musc.edu FAU - Nietert, Paul J AU - Nietert PJ FAU - Taylor, Marian H AU - Taylor MH FAU - Mitchell, Holly C AU - Mitchell HC FAU - Shaftman, Stephanie R AU - Shaftman SR FAU - Silver, Richard M AU - Silver RM FAU - Smith, Edwin A AU - Smith EA FAU - Bolster, Marcy B AU - Bolster MB LA - eng GR - 1 P60 AR049459/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20070515 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Black or African American/ethnology MH - Age Factors MH - Aged MH - Black People/ethnology MH - Cohort Studies MH - Disease Progression MH - Female MH - Humans MH - Hypertension, Pulmonary/*ethnology/*etiology MH - Hypertrophy, Left Ventricular/ethnology/etiology MH - Incidence MH - Male MH - Middle Aged MH - Risk Factors MH - Scleroderma, Systemic/*complications/*ethnology MH - White People/ethnology EDAT- 2007/05/23 09:00 MHDA- 2007/08/31 09:00 CRDT- 2007/05/23 09:00 PHST- 2007/05/23 09:00 [pubmed] PHST- 2007/08/31 09:00 [medline] PHST- 2007/05/23 09:00 [entrez] AID - 07/13/059 [pii] PST - ppublish SO - J Rheumatol. 2007 Jun;34(6):1277-82. Epub 2007 May 15. PMID- 30039730 OWN - NLM STAT- MEDLINE DCOM- 20190314 LR - 20190314 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 48 IP - 1 DP - 2019 Jan TI - Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database. PG - 42-51 LID - 10.1080/03009742.2018.1459830 [doi] AB - OBJECTIVE: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. METHOD: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses. RESULTS: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's. CONCLUSION: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice. FAU - Carreira, P E AU - Carreira PE AD - a Rheumatology Department , University Hospital 12 de Octubre , Madrid , Spain. FAU - Carmona, L AU - Carmona L AD - b Musculoskeletal Health Institute , Madrid , Spain. FAU - Joven, B E AU - Joven BE AD - a Rheumatology Department , University Hospital 12 de Octubre , Madrid , Spain. FAU - Loza, E AU - Loza E AD - b Musculoskeletal Health Institute , Madrid , Spain. FAU - Andréu, J L AU - Andréu JL AD - c Rheumatology Department , University Hospital Puerta de Hierro , Madrid , Spain. FAU - Riemekasten, G AU - Riemekasten G AD - d Department of Rheumatology , University of Lübeck , Lübeck , Germany. FAU - Vettori, S AU - Vettori S AD - e Rheumatology Unit, Department of Internal Medicine Clinical and Experimental 'F Magrassi-A-Lanzara' , Second University of Naples , Naples , Italy. FAU - Balbir-Gurman, A AU - Balbir-Gurman A AD - f B Shine Rheumatology Unit , Rambam Health Care Campus and Rappaport Faculty of Medicine-Technion , Haifa , Israel. FAU - Airò, P AU - Airò P AD - g Rheumatology and Clinical Immunology Unit , Civil Hospitali , Brescia , Italy. FAU - Walker, U AU - Walker U AD - h Rheumatology Department , Felix Platter Hospital , Basel , Switzerland. FAU - Damjanov, N AU - Damjanov N AD - i University of Belgrade School of Medicine , Belgrade , Serbia. FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M AD - j Division of Rheumatology, Department of Clinical and Experimental Medicine , University of Florence , Florence , Italy. FAU - Ananieva, L P AU - Ananieva LP AD - k Institute of Rheumatology , Russian Academy of Medical Science , Moscow , Russia. FAU - Rednic, S AU - Rednic S AD - l Rheumatology Clinic , University of Medicine and Pharmacy 'Iuliu Hatieganu' Cluj , Cluj-Napoca , Romania. FAU - Czirják, L AU - Czirják L AD - m Department of Immunology and Rheumatology, Faculty of Medicine , University of Pécs , Pécs , Hungary. FAU - Distler, O AU - Distler O AD - n Division of Rheumatology , University Hospital Zürich , Zürich , Switzerland. FAU - Farge, D AU - Farge D AD - o Department of Internal Medicine , Saint-Louis Hospital , Paris , France. FAU - Hesselstrand, R AU - Hesselstrand R AD - p Department of Rheumatology , Lund University Hospital , Lund , Sweden. FAU - Corrado, A AU - Corrado A AD - q Rheumatology Unit , University of Foggia, 'Col. D'Avanzo' Hospital , Foggia , Italy. FAU - Caramaschi, P AU - Caramaschi P AD - r Rheumatology Unit , AOUI , Verona , Italy. FAU - Tikly, M AU - Tikly M AD - s Rheumatology Unit, Department of Medicine , Chris Hani Baragwanath Hospital and University of the Witwatersrand , Johannesburg , South Africa. FAU - Allanore, Y AU - Allanore Y AD - t Rheumatology A Department , Cochin Hospital, APHP, Paris Descartes University , Paris , France. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20180724 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adult MH - Age Distribution MH - Age Factors MH - Age of Onset MH - Cross-Sectional Studies MH - Databases, Factual MH - Europe/epidemiology MH - Female MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Prevalence MH - *Registries MH - Retrospective Studies MH - Scleroderma, Systemic/diagnosis/*epidemiology MH - Sex Distribution EDAT- 2018/07/25 06:00 MHDA- 2019/03/15 06:00 CRDT- 2018/07/25 06:00 PHST- 2018/07/25 06:00 [pubmed] PHST- 2019/03/15 06:00 [medline] PHST- 2018/07/25 06:00 [entrez] AID - 10.1080/03009742.2018.1459830 [doi] PST - ppublish SO - Scand J Rheumatol. 2019 Jan;48(1):42-51. doi: 10.1080/03009742.2018.1459830. Epub 2018 Jul 24. PMID- 24461387 OWN - NLM STAT- MEDLINE DCOM- 20141002 LR - 20220408 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 48-49 DP - 2014 Feb-Mar TI - The diagnosis and classification of mixed connective tissue disease. PG - 46-9 LID - S0896-8411(14)00010-9 [pii] LID - 10.1016/j.jaut.2014.01.008 [doi] AB - The term "mixed connective tissue disease" (MCTD) concerns a systemic autoimmune disease typified by overlapping features between two or more systemic autoimmune diseases and the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1snRNP). Since the first description of this condition in 1972, the understanding of clinical manifestations and long-term outcome of MCTD have significantly advanced. Polyarthritis, Raynaud's phenomenon, puffy fingers, lung involvement and esophageal dysmotility are the most frequently reported symptoms among the different cohorts during the course of the disease. Moreover, in recent years a growing interest has been focused on severe organ involvement such as pulmonary arterial hypertension and interstitial lung disease which can accrue during the long-term follow-up and can still significantly influence disease prognosis. Over the last years, significant advances have been made also in disease pathogenesis understanding and a central pathogenetic role of anti-U1RNP autoantibodies has clearly emerged. Although controversies on disease definition and classification still persist, MCTD identifies a group of patients in whom increased surveillance for specific manifestations and prognostic stratification became mandatory to improve patient's outcomes. CI - Copyright © 2014. Published by Elsevier Ltd. FAU - Tani, Chiara AU - Tani C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Carli, Linda AU - Carli L AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy; Dottorato Genomec, University of Siena, Italy. FAU - Vagnani, Sabrina AU - Vagnani S AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Talarico, Rosaria AU - Talarico R AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Baldini, Chiara AU - Baldini C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Bombardieri, Stefano AU - Bombardieri S AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. Electronic address: s.bombardieri@med.unipi.it. LA - eng PT - Journal Article PT - Review DEP - 20140122 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 SB - IM MH - Autoimmune Diseases/classification/diagnosis/immunology MH - Dermatomyositis/classification/diagnosis/immunology MH - Diagnosis, Differential MH - Humans MH - Lupus Erythematosus, Systemic/classification/diagnosis/immunology MH - Mixed Connective Tissue Disease/*classification/*diagnosis/epidemiology/immunology MH - Polymyositis/classification/diagnosis/immunology MH - Prognosis MH - Scleroderma, Systemic/classification/diagnosis/immunology MH - Sjogren's Syndrome/classification/diagnosis/immunology OTO - NOTNLM OT - Anti-U1RNP autoantibodies OT - Classification OT - MCTD OT - Prognosis EDAT- 2014/01/28 06:00 MHDA- 2014/10/03 06:00 CRDT- 2014/01/28 06:00 PHST- 2013/10/07 00:00 [received] PHST- 2013/11/13 00:00 [accepted] PHST- 2014/01/28 06:00 [entrez] PHST- 2014/01/28 06:00 [pubmed] PHST- 2014/10/03 06:00 [medline] AID - S0896-8411(14)00010-9 [pii] AID - 10.1016/j.jaut.2014.01.008 [doi] PST - ppublish SO - J Autoimmun. 2014 Feb-Mar;48-49:46-9. doi: 10.1016/j.jaut.2014.01.008. Epub 2014 Jan 22. PMID- 28163035 OWN - NLM STAT- MEDLINE DCOM- 20171218 LR - 20260127 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 112 DP - 2017 Jul TI - Intra-and inter-observer reliability of nailfold videocapillaroscopy - A possible outcome measure for systemic sclerosis-related microangiopathy. PG - 1-6 LID - S0026-2862(16)30209-6 [pii] LID - 10.1016/j.mvr.2017.02.001 [doi] AB - OBJECTIVES: Our aim was to assess the reliability of nailfold capillary assessment in terms of image evaluability, image severity grade ('normal', 'early', 'active', 'late'), capillary density, capillary (apex) width, and presence of giant capillaries, and also to gain further insight into differences in these parameters between patients with systemic sclerosis (SSc), patients with primary Raynaud's phenomenon (PRP) and healthy control subjects. METHODS: Videocapillaroscopy images (magnification 300×) were acquired from all 10 digits from 173 participants: 101 patients with SSc, 22 with PRP and 50 healthy controls. Ten capillaroscopy experts from 7 European centres evaluated the images. Custom image mark-up software allowed extraction of the following outcome measures: overall grade ('normal', 'early', 'active', 'late', 'non-specific', or 'ungradeable'), capillary density (vessels/mm), mean vessel apical width, and presence of giant capillaries. RESULTS: Observers analysed a median of 129 images each. Evaluability (i.e. the availability of measures) varied across outcome measures (e.g. 73.0% for density and 46.2% for overall grade in patients with SSc). Intra-observer reliability for evaluability was consistently higher than inter- (e.g. for density, intra-class correlation coefficient [ICC] was 0.71 within and 0.14 between observers). Conditional on evaluability, both intra- and inter-observer reliability were high for grade (ICC 0.93 and 0.78 respectively), density (0.91 and 0.64) and width (0.91 and 0.85). CONCLUSIONS: Evaluability is one of the major challenges in assessing nailfold capillaries. However, when images are evaluable, the high intra- and inter-reliabilities suggest that overall image grade, capillary density and apex width have potential as outcome measures in longitudinal studies. CI - Copyright © 2017 Elsevier Inc. All rights reserved. FAU - Dinsdale, Graham AU - Dinsdale G AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. Electronic address: graham.dinsdale@manchester.ac.uk. FAU - Moore, Tonia AU - Moore T AD - Salford Royal Hospital NHS Foundation Trust, Salford, UK. FAU - O'Leary, Neil AU - O'Leary N AD - Centre for Biostatistics, Division of Population Health, Health Services Research & Primary Care, University of Manchester, Manchester, UK. FAU - Tresadern, Philip AU - Tresadern P AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, University of Manchester, Manchester, UK. FAU - Berks, Michael AU - Berks M AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, University of Manchester, Manchester, UK. FAU - Roberts, Christopher AU - Roberts C AD - Centre for Biostatistics, Division of Population Health, Health Services Research & Primary Care, University of Manchester, Manchester, UK. FAU - Manning, Joanne AU - Manning J AD - Salford Royal Hospital NHS Foundation Trust, Salford, UK. FAU - Allen, John AU - Allen J AD - Microvascular Diagnostics, Northern Medical Physics and Clinical Engineering, Freeman Hospital, Newcastle upon Tyne, UK. FAU - Anderson, Marina AU - Anderson M AD - Institute of Ageing and Chronic Disease, University of Liverpool, Liverpool, UK. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden. FAU - Howell, Kevin AU - Howell K AD - Institute of Immunity and Transplantation, University College London, Royal Free Campus, London, UK. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Faculty of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Dept. Internal Medicine, University of Genova, Italy. FAU - Wildt, Marie AU - Wildt M AD - Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden. FAU - Taylor, Christopher AU - Taylor C AD - Centre for Imaging Sciences, Division of Informatics, Imaging & Data Sciences, University of Manchester, Manchester, UK. FAU - Murray, Andrea AU - Murray A AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal & Dermatological Sciences, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng GR - 19465/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20170202 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM MH - Adult MH - Aged MH - Capillaries/*pathology MH - Case-Control Studies MH - Europe MH - Female MH - Humans MH - Image Interpretation, Computer-Assisted MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Observer Variation MH - Predictive Value of Tests MH - Reproducibility of Results MH - Scleroderma, Systemic/*complications/diagnosis MH - Severity of Illness Index MH - Software MH - Vascular Diseases/*diagnosis/etiology/pathology MH - Young Adult OTO - NOTNLM OT - Capillaroscopic patterns OT - Nailfold videocapillaroscopy OT - Raynaud's phenomenon OT - Reliability OT - Systemic sclerosis EDAT- 2017/02/07 06:00 MHDA- 2017/12/19 06:00 CRDT- 2017/02/07 06:00 PHST- 2016/11/29 00:00 [received] PHST- 2017/01/12 00:00 [revised] PHST- 2017/02/01 00:00 [accepted] PHST- 2017/02/07 06:00 [pubmed] PHST- 2017/12/19 06:00 [medline] PHST- 2017/02/07 06:00 [entrez] AID - S0026-2862(16)30209-6 [pii] AID - 10.1016/j.mvr.2017.02.001 [doi] PST - ppublish SO - Microvasc Res. 2017 Jul;112:1-6. doi: 10.1016/j.mvr.2017.02.001. Epub 2017 Feb 2. PMID- 33087021 OWN - NLM STAT- MEDLINE DCOM- 20210826 LR - 20210826 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 4 IP - 2 DP - 2020 Jul TI - Two patients with mixed connective tissue disease complicated by pulmonary arterial hypertension showing contrasting responses to pulmonary vasodilators. PG - 253-261 LID - 10.1080/24725625.2020.1758388 [doi] AB - Mixed connective tissue disease (MCTD) involves various clinical manifestations, and pulmonary hypertension (PH) is an important organ dysfunction defining the prognosis of MCTD. The pathology of PH is heterogeneous. Here, we present 2 cases of MCTD complicated by PH that had contrasting clinical courses. The first case involved a 54-year-old woman with Raynaud's phenomenon and dyspnoea on exertion. She was diagnosed with MCTD accompanied by pulmonary arterial hypertension (PAH) and was treated with ambrisentan and tadalafil in addition to high-dose glucocorticoid (GC) therapy and rituximab therapy. After treatment, her PH resolved. The second case involved a 64-year-old woman with Raynaud's phenomenon and dyspnoea on exertion. She was similarly diagnosed with MCTD accompanied by PAH and was treated with ambrisentan and tadalafil in addition to high-dose GC therapy and cyclophosphamide pulse therapy. However, she showed exacerbation of her respiratory condition and manifestation of pulmonary veno-occlusive disease (PVOD). Thus, the treatment was discontinued, and subsequently, her condition improved and eventually returned to that before treatment. The findings suggest that the presence or absence of latent PVOD might be an important factor for predicting the therapeutic responsiveness of MCTD-associated PH. Evaluation of chest radiography findings, computed tomography findings, percent vital capacity, and percent carbon monoxide diffusion capacity might be useful for predicting prognosis and might aid in treatment. PVOD could be underlying in patients with CTD-PH. When the complication of PVOD is suggested by chest CT or pulmonary function test, we need a careful introduction with pulmonary vasodilators. So, combination therapy of pulmonary vasodilators should not be applied in all patients with CTD-PH since underlying PVOD could deteriorate the patient's condition. FAU - Kusaka, Katsuhide AU - Kusaka K AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Nakano, Kazuhisa AU - Nakano K AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Iwata, Shigeru AU - Iwata S AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Kubo, Satoshi AU - Kubo S AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Nishida, Tomoya AU - Nishida T AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Tanaka, Yoshiya AU - Tanaka Y AUID- ORCID: 0000-0002-0807-7139 AD - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20200504 PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Female MH - Humans MH - Immunosuppressive Agents/administration & dosage/adverse effects/therapeutic use MH - Middle Aged MH - Mixed Connective Tissue Disease/*complications/diagnosis/therapy MH - Pulmonary Arterial Hypertension/diagnosis/*drug therapy/*etiology MH - Pulmonary Veno-Occlusive Disease/diagnosis MH - Respiratory Function Tests MH - Symptom Assessment MH - Treatment Outcome MH - Vasodilator Agents/administration & dosage/*therapeutic use OTO - NOTNLM OT - Pulmonary vasodilators OT - immunosuppressive therapy OT - mixed connective tissue disease OT - pulmonary hypertension OT - pulmonary veno-occlusive disease EDAT- 2020/10/23 06:00 MHDA- 2021/08/27 06:00 CRDT- 2020/10/22 05:25 PHST- 2020/10/22 05:25 [entrez] PHST- 2020/10/23 06:00 [pubmed] PHST- 2021/08/27 06:00 [medline] AID - 10.1080/24725625.2020.1758388 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2020 Jul;4(2):253-261. doi: 10.1080/24725625.2020.1758388. Epub 2020 May 4. PMID- 39201105 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240901 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 13 IP - 16 DP - 2024 Aug 22 TI - Interstitial Lung Disease Phenotypes and Predictive Risk Factors in Primary Sjögren's Syndrome. LID - 10.3390/jcm13164963 [doi] LID - 4963 AB - Background/Objectives: The prevalence of Interstitial Lung Disease (ILD) and risk factors for its development in patients with primary Sjögren's syndrome (pSS) are still debated, possibly due to the existence of heterogeneous pSS-related ILD phenotypes. The aims of this study were: 1. To investigate the prevalence and predictive factors for ILD development in a single-center pSS cohort; 2. To characterize different pSS-ILD phenotypes. Methods: Clinical, laboratory and imaging data of pSS patients attending our center from January 2019 to September 2023 were retrospectively analyzed. ILD presence was confirmed on HRCT. Results: Forty-three out of 474 enrolled pSS patients presented ILD (M:F = 6:37), accounting for an overall ILD prevalence of 9.1%. In 19 cases, ILD was the first manifestation of pSS (ILD-onset), while in 24 ILD was diagnosed after pSS (ILD-incident). Compared to ILD-onset, ILD-incident patients more often presented pSS-related hematologic abnormalities (p = 0.012), cutaneous involvement (p = 0.027), inflammatory arthralgias (p = 0.026), C4 hypocomplementemia (p = 0.012) and positive RF (p = 0.031). On the other hand, ILD-onset patients were significantly older at pSS diagnosis (p = 0.008) and presented more severe fibrosis on HRCT (p = 0.008). On the univariate analysis, higher ESSDAI (p = 0.011), Raynaud's phenomenon (p = 0.009), anti-Ro52 (p = 0.031), hypergammaglobulinemia (p = 0.011), Rheumatoid Factor (RF) (p = 0.038) and C4 hypocomplementemia (p = 0.044) at baseline were associated to ILD development during follow-up. On the multivariate analysis, the ESSDAI at baseline (p = 0.05) and Raynaud's phenomenon (p = 0.013) at baseline were the only independent predictors of ILD development. Conclusions: ILD is a relatively common and clinically heterogenous pSS manifestation. Elevated disease activity at pSS onset is a risk factor for ILD development, prompting careful follow-up and intriguingly suggesting that immunomodulatory therapies may prevent ILD. FAU - La Rocca, Gaetano AU - La Rocca G AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Ferro, Francesco AU - Ferro F AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Sambataro, Gianluca AU - Sambataro G AUID- ORCID: 0000-0001-9933-1202 AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, AOE Cannizzaro, University of Catania, Via Messina 829, 95126 Catania, Italy. AD - Artroreuma S.R.L., Rheumatology Outpatient Clinic Associated with the National Health System, Corso S. Vito 53, 95030 Catania, Italy. FAU - Elefante, Elena AU - Elefante E AUID- ORCID: 0000-0003-0657-485X AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Fulvio, Giovanni AU - Fulvio G AUID- ORCID: 0000-0003-0130-4412 AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Navarro, Inmaculada Concepción AU - Navarro IC AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Moretti, Michele AU - Moretti M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Romei, Chiara AU - Romei C AD - Radiodiagnostic Unit 2, Department of Diagnostic Imaging, University of Pisa, Via Paradisa 2, 56124 Pisa, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. FAU - Baldini, Chiara AU - Baldini C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy. LA - eng PT - Journal Article DEP - 20240822 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC11355583 OTO - NOTNLM OT - HRCT patterns OT - clinical phenotypes OT - interstitial lung disease OT - primary Sjögren’s syndrome OT - prognosis OT - risk factors COIS- The authors declare no conflicts of interest. EDAT- 2024/08/31 09:50 MHDA- 2024/08/31 09:51 PMCR- 2024/08/22 CRDT- 2024/08/29 01:11 PHST- 2024/07/25 00:00 [received] PHST- 2024/08/17 00:00 [revised] PHST- 2024/08/21 00:00 [accepted] PHST- 2024/08/31 09:51 [medline] PHST- 2024/08/31 09:50 [pubmed] PHST- 2024/08/29 01:11 [entrez] PHST- 2024/08/22 00:00 [pmc-release] AID - jcm13164963 [pii] AID - jcm-13-04963 [pii] AID - 10.3390/jcm13164963 [doi] PST - epublish SO - J Clin Med. 2024 Aug 22;13(16):4963. doi: 10.3390/jcm13164963. PMID- 39338221 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240930 IS - 2075-4426 (Print) IS - 2075-4426 (Electronic) IS - 2075-4426 (Linking) VI - 14 IP - 9 DP - 2024 Sep 12 TI - Clinical Characteristics of Distinct Subgroups of Patients with Primary Sjögren's Syndrome Classified by Serological Profiles: A Comparison Study. LID - 10.3390/jpm14090967 [doi] LID - 967 AB - Sjögren's syndrome (SS) is an autoimmune disease characterized by heterogeneous clinical presentation and the presence of various autoantibodies. This study aimed to determine the differences in clinical findings according to antibody positivity in patients with primary Sjögren syndrome (pSS) in the Turkish population. A retrospective study was conducted and 402 patients (378 women and 24 men) with pSS were analyzed. The patients were categorized into three subgroups based on serological tests. These were (1) quadruple seropositivity (positive for anti-Sjögren's syndrome-related antigen A antibodies (anti-SSA; anti-Ro) and anti-Sjögren's syndrome-related antigen B antibodies (anti-SSB; anti-La), rheumatoid factor (RF), and antinuclear antibody (ANA); (2) double seropositivity (positive for ANA and anti-SSA/Ro antibodies); and (3) quadruple seronegativity (negative for ANA, RF, anti-SSA/Ro and anti-SSB/La antibodies). The number of quadruple-seropositive patients was 72 (18.6%), double-seropositive 174 (43.2%), and quadruple-seronegative was 85 (21.1%). The age at diagnosis of quadruple-seropositive pSS was 42.4 ± 10.8, which was significantly younger than that of patients with double-seropositive and quadruple-seronegative pSS (p = 0.021, p = 0.112). In terms of organ involvement, salivary gland enlargement, arthralgia, arthritis, Raynaud's phenomenon, lymphadenopathy, cutaneous vasculitis, interstitial lung disease, neurological involvement, autoimmune thyroiditis, renal interstitial disease, anemia, leukopenia, hypergammaglobulinemia, and hypocomplementemia were more common in quadruple-seropositive patients with pSS than in quadruple-seronegative patients (p < 0.0001). The results of this study confirmed the strong impact of immunological markers on the pSS phenotype at the time of diagnosis. Immunological patterns play a central role in the phenotypic expression of the disease, even during the initial diagnostic phase, and can guide physicians in designing personalized treatment plans for patients with pSS. FAU - Bodakçi, Erdal AU - Bodakçi E AD - Division of Rheumatology, Department of Internal Medicine, Eskisehir Cıty Hospıtal, Eskisehir 26100, Turkey. LA - eng PT - Journal Article DEP - 20240912 PL - Switzerland TA - J Pers Med JT - Journal of personalized medicine JID - 101602269 PMC - PMC11433317 OTO - NOTNLM OT - autoantibodies OT - clinical symptoms OT - primary Sjögren’s syndrome COIS- The author declares no conflict of interest. EDAT- 2024/09/28 22:43 MHDA- 2024/09/28 22:44 PMCR- 2024/09/12 CRDT- 2024/09/28 01:21 PHST- 2024/08/05 00:00 [received] PHST- 2024/09/06 00:00 [revised] PHST- 2024/09/09 00:00 [accepted] PHST- 2024/09/28 22:44 [medline] PHST- 2024/09/28 22:43 [pubmed] PHST- 2024/09/28 01:21 [entrez] PHST- 2024/09/12 00:00 [pmc-release] AID - jpm14090967 [pii] AID - jpm-14-00967 [pii] AID - 10.3390/jpm14090967 [doi] PST - epublish SO - J Pers Med. 2024 Sep 12;14(9):967. doi: 10.3390/jpm14090967. PMID- 17599771 OWN - NLM STAT- MEDLINE DCOM- 20070906 LR - 20220409 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 7 DP - 2007 Jul TI - Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. PG - 2422-31 AB - OBJECTIVE: To explore the relationship between changes in the severity of skin disease and morbidity and mortality in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: From a large single-center cohort, we identified 225 patients with dcSSc for whom serial clinical information was available from within 24 months of the onset of the first non-Raynaud's phenomenon manifestation of SSc. The end points analyzed included death and heart, lung, kidney, and gastrointestinal tract involvement. Latent linear trajectory modeling (LTM) was applied to identify patients with a similar trajectory of modified Rodnan skin thickness score (MRSS) changes over the first 3 years of followup. Clinical outcomes were compared between 3 different LTM subgroups. RESULTS: LTM permitted classification of 131 patients (58%) into 1 of 3 subgroups with different skin score trajectories. Survival was lowest in the subgroup of patients who had a high baseline skin score and experienced little improvement during followup (P = 0.003). However, the frequency of clinical end points was similar in the subgroup with the most favorable trajectory (i.e., a low initial MRSS and subsequent improvement) and the subgroup with a high baseline MRSS and no improvement. Interestingly, the end point frequency was greatest in the subgroup with a high initial MRSS and subsequent improvement, suggesting that sustained severe skin disease does not necessarily predict the number of visceral complications, and that the relationship between the skin score and internal organ involvement in dcSSc is more complex than previously thought. CONCLUSION: Although mortality was highest among patients with the worst skin-related outcomes, no simple relationship between burden of disease and change in skin score was observed. FAU - Shand, Lynne AU - Shand L AD - Royal Free Hospital, London, UK. FAU - Lunt, Mark AU - Lunt M FAU - Nihtyanova, Svetlana AU - Nihtyanova S FAU - Hoseini, Mansour AU - Hoseini M FAU - Silman, Alan AU - Silman A FAU - Black, Carol M AU - Black CM FAU - Denton, Christopher P AU - Denton CP LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adult MH - Cohort Studies MH - Female MH - Follow-Up Studies MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Models, Biological MH - Scleroderma, Diffuse/mortality/*pathology MH - Scleroderma, Systemic/mortality/*pathology MH - Skin/*pathology MH - Survival Analysis EDAT- 2007/06/30 09:00 MHDA- 2007/09/07 09:00 CRDT- 2007/06/30 09:00 PHST- 2007/06/30 09:00 [pubmed] PHST- 2007/09/07 09:00 [medline] PHST- 2007/06/30 09:00 [entrez] AID - 10.1002/art.22721 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Jul;56(7):2422-31. doi: 10.1002/art.22721. PMID- 33851273 OWN - NLM STAT- MEDLINE DCOM- 20210928 LR - 20260518 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 40 IP - 10 DP - 2021 Oct TI - Anti-mitochondrial autoantibodies are associated with cardiomyopathy, dysphagia, and features of more severe disease in adult-onset myositis. PG - 4095-4100 LID - 10.1007/s10067-021-05730-7 [doi] AB - We analyzed the prevalence of anti-mitochondrial autoantibodies (AMA) in adult- and juvenile-onset myositis longitudinal cohorts and investigated phenotypic differences in myositis patients with AMA. We screened sera from myositis patients including 619 adult- and 371 juvenile-onset dermatomyositis (DM, JDM), polymyositis (PM, JPM), inclusion body myositis (IBM), or amyopathic DM patients and from healthy controls, including 164 adults and 92 children, for AMA by ELISA. Clinical characteristics were compared between myositis patients with and without AMA. AMA were present in 5% of adult myositis patients (16 of 216 DM, 10 of 222 PM, 4 of 140 IBM, 1 of 19 amyopathic DM), 1% of juvenile myositis patients (3 of 302 JDM, 1 of 25 JPM), and 1% of both adult and juvenile healthy controls. In patients with adult-onset myositis, AMA were associated with persistent muscle weakness, Raynaud's phenomenon, dysphagia, and cardiomyopathy. Adult myositis patients with AMA may have more severe or treatment refractory disease, as they more frequently received glucocorticoids and intravenous immunoglobulin. In juvenile myositis, children with AMA often had falling episodes and dysphagia, but no other clinical features or medications were significantly associated with AMA. AMA are present in 5% of adult myositis patients and associated with cardiomyopathy, dysphagia, and other signs of severe disease. The prevalence of AMA is not increased in patients with juvenile myositis compared to age-matched healthy controls. Our data suggest that the presence of AMA in adult myositis patients should prompt screening for cardiac and swallowing involvement. Key Points • Approximately 5% of a large North American cohort of adult myositis patients have anti-mitochondrial autoantibodies. • Adults with anti-mitochondrial autoantibodies often have chronic weakness, Raynaud's, dysphagia, cardiomyopathy, and more severe disease. • Anti-mitochondrial autoantibodies are rare in juvenile myositis and not associated with a specific clinical phenotype. CI - © 2021. The Author(s). FAU - Sabbagh, Sara E AU - Sabbagh SE AUID- ORCID: 0000-0003-3176-9958 AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. AD - Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA. FAU - Pinal-Fernandez, Iago AU - Pinal-Fernandez I AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. AD - Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. AD - Faculty of Health Sciences, Universitat Oberta de Catalunya, Barcelona, Spain. FAU - Casal-Dominguez, Maria AU - Casal-Dominguez M AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. AD - Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Albayda, Jemima AU - Albayda J AD - Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Paik, Julie J AU - Paik JJ AD - Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Miller, Frederick W AU - Miller FW AD - Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Rider, Lisa G AU - Rider LG AD - Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Mammen, Andrew L AU - Mammen AL AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. AD - Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. AD - Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Christopher-Stine, Lisa AU - Christopher-Stine L AD - Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. lchrist4@jhmi.edu. AD - Johns Hopkins Myositis Center, Division of Rheumatology, Johns Hopkins University School of Medicine, Bayview Medical Office, 5200 Eastern Ave #301, Baltimore, MD, 21224, USA. lchrist4@jhmi.edu. CN - Johns Hopkins Myositis Center Group LA - eng GR - Z01 ES101074/ImNIH/Intramural NIH HHS/United States GR - Z01 ES101081/ImNIH/Intramural NIH HHS/United States PT - Journal Article DEP - 20210413 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - *Cardiomyopathies/complications MH - *Deglutition Disorders/epidemiology MH - *Dermatomyositis/complications MH - Humans MH - *Myositis/complications PMC - PMC8463345 MID - NIHMS1701635 OTO - NOTNLM OT - Anti-mitochondrial autoantibodies OT - Juvenile myositis OT - Myositis FIR - Michelle, Harlan IR - Michelle H FIR - Tiniakou, Eleni IR - Tiniakou E FIR - Danoff, Sonye K IR - Danoff SK FIR - Lloyd, Tom IR - Lloyd T EDAT- 2021/04/15 06:00 MHDA- 2021/09/29 06:00 PMCR- 2021/04/13 CRDT- 2021/04/14 06:41 PHST- 2021/01/29 00:00 [received] PHST- 2021/03/31 00:00 [accepted] PHST- 2021/03/30 00:00 [revised] PHST- 2021/04/15 06:00 [pubmed] PHST- 2021/09/29 06:00 [medline] PHST- 2021/04/14 06:41 [entrez] PHST- 2021/04/13 00:00 [pmc-release] AID - 10.1007/s10067-021-05730-7 [pii] AID - 5730 [pii] AID - 10.1007/s10067-021-05730-7 [doi] PST - ppublish SO - Clin Rheumatol. 2021 Oct;40(10):4095-4100. doi: 10.1007/s10067-021-05730-7. Epub 2021 Apr 13. PMID- 21124695 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20240320 IS - 1179-5441 (Electronic) IS - 1179-5441 (Linking) VI - 3 DP - 2010 May 24 TI - Autonomic dysfunction predicts early cardiac affection in patients with systemic sclerosis. PG - 43-54 AB - OBJECTIVE: To detect the early preclinical alterations in cardiac autonomic control as well as altered cardiac function in systemic sclerosis (SSc) patients and their relevance to the clinical features of the disease using noninvasive methods. METHODS: 30 SSc patients and 15 healthy controls matched for age and sex underwent clinical examination, serological analysis, and echocardiographic assessment including Doppler flow imaging to evaluate cardiac function, and 24-hour Holter monitoring analyzed for arrhythmia and heart rate variability (HRV) in the time and frequency domains. RESULTS: The trans-mitral Doppler of early to atrial wave (E/A) ratio was reversed in five patients (16.6%) and the tricuspid E/A ratio was reversed in 10 patients (33.3%). Holter analysis for SSc patients revealed an increased prevalence of premature ventricular contractions (PVC) ≥ 10/h (P = 0.02), supra-ventricular tachycardias (SVTs) (P = 0.2), and total PVC count (P = 0.0000). Highly significant (P = 0.000) impairment in all HRV parameters was demonstrated in the SSc patients. Total skin thickness score (TSS), Raynaud's phenomenon and anti-scleroderma 70 (anti-SCL70) showed significant positive correlations with all arrhythmia parameters, while showing a significant negative correlation with the impaired ventricular diastolic function and various HRV parameters. No correlation was found between arrhythmia and HRV parameters and disease duration, disease type, or presence of anti-centromere antibodies. CONCLUSION: Low heart rate variability, increased TSS and the presence of anti-SCL70 are correlated with preclinical cardiac involvement in SSc patients and may predict the likelihood of malignant arrhythmia and sudden cardiac death. Therefore, noninvasive HRV evaluation before clinical cardiac involvement in these patients might be beneficial when added to the clinical and laboratory assessments in detecting high-risk patients, and may allow for implementation of preventive measures and initiation of appropriate therapy early in the course of the disease. FAU - Othman, Khaled M AU - Othman KM AD - The Departments of Cardiology. FAU - Assaf, Naglaa Youssef AU - Assaf NY FAU - Farouk, Hanan Mohamed AU - Farouk HM FAU - Aly Hassan, Iman M AU - Aly Hassan IM LA - eng PT - Journal Article DEP - 20100524 PL - United States TA - Clin Med Insights Arthritis Musculoskelet Disord JT - Clinical medicine insights. Arthritis and musculoskeletal disorders JID - 101542737 PMC - PMC2989638 OTO - NOTNLM OT - autonomic dysfunction OT - heart rate variability OT - systemic sclerosis EDAT- 2010/12/03 06:00 MHDA- 2010/12/03 06:01 PMCR- 2010/05/24 CRDT- 2010/12/03 06:00 PHST- 2010/12/03 06:00 [entrez] PHST- 2010/12/03 06:00 [pubmed] PHST- 2010/12/03 06:01 [medline] PHST- 2010/05/24 00:00 [pmc-release] AID - cmamd-2010-043 [pii] AID - 10.4137/cmamd.s4940 [doi] PST - epublish SO - Clin Med Insights Arthritis Musculoskelet Disord. 2010 May 24;3:43-54. doi: 10.4137/cmamd.s4940. PMID- 19228662 OWN - NLM STAT- MEDLINE DCOM- 20090601 LR - 20260518 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 4 DP - 2009 Apr TI - Quality of life in patients with systemic sclerosis compared to the general population and patients with other chronic conditions. PG - 768-72 LID - 10.3899/jrheum.080281 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) affects multiple physical, psychological, and social domains and is associated with impaired health-related quality of life (HRQOL).We compared the HRQOL of SSc patients with individuals in the general population and patients with other common chronic diseases. METHODS: HRQOL of SSc patients in the Canadian Scleroderma Research Group registry was measured using version 2 of the Medical Outcomes Trust Short Form-36 (SF-36). Results were compared to US general population norms and scores reported for patients with other common chronic diseases, namely heart disease, lung disease, hypertension, diabetes, and depression. RESULTS: SF-36 scores were available for 504 SSc patients (86% women, mean age 56 yrs, mean disease duration since onset of first non-Raynaud's manifestation of SSc 11 yrs). The greatest impairment in SF-36 subscale scores appeared to be in the physical functioning, general health, and role physical domains. SF-36 subscale and summary scores in SSc were significantly worse compared to US general population norms for women of similar ages, except for mental health and mental component summary score, which were not significantly different, and were generally comparable to or worse than the scores of patients with other common chronic conditions. CONCLUSION: HRQOL of patients with SSc is significantly impaired compared to that of the general population and is comparable to or worse than that of patients with other common chronic conditions. FAU - Hudson, Marie AU - Hudson M AD - SMBD-Jewish General Hospital and McGill University, Room A-216, 3755 Cote Ste. Catherine Road, Montreal, Quebec H3T 1E2, Canada. marie.hudson@mcgill.ca FAU - Thombs, Brett D AU - Thombs BD FAU - Steele, Russell AU - Steele R FAU - Panopalis, Pantelis AU - Panopalis P FAU - Newton, Evan AU - Newton E FAU - Baron, Murray AU - Baron M CN - Canadian Scleroderma Research Group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090217 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Chronic Disease MH - Cross-Sectional Studies MH - Female MH - Humans MH - Middle Aged MH - Quality of Life MH - *Scleroderma, Systemic/physiopathology/psychology MH - Surveys and Questionnaires FIR - Abu-Hakima, M IR - Abu-Hakima M FIR - Docherty, P IR - Docherty P FIR - Fritzler, M J IR - Fritzler MJ FIR - Jones, N IR - Jones N FIR - Kaminska, E IR - Kaminska E FIR - Khalidi, N IR - Khalidi N FIR - LeClercq, S IR - LeClercq S FIR - Ligier, S IR - Ligier S FIR - Markland, J IR - Markland J FIR - Masetto, A IR - Masetto A FIR - Mathieu, J-P IR - Mathieu JP FIR - Pope, J IR - Pope J FIR - Robinson, D IR - Robinson D FIR - Smith, D IR - Smith D FIR - Sutton, E IR - Sutton E EDAT- 2009/02/21 09:00 MHDA- 2009/06/02 09:00 CRDT- 2009/02/21 09:00 PHST- 2009/02/21 09:00 [entrez] PHST- 2009/02/21 09:00 [pubmed] PHST- 2009/06/02 09:00 [medline] AID - jrheum.080281 [pii] AID - 10.3899/jrheum.080281 [doi] PST - ppublish SO - J Rheumatol. 2009 Apr;36(4):768-72. doi: 10.3899/jrheum.080281. Epub 2009 Feb 17. PMID- 31519721 OWN - NLM STAT- MEDLINE DCOM- 20200212 LR - 20210913 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 12 IP - 9 DP - 2019 Sep 12 TI - Jo1-antisynthetase syndrome and severe interstitial lung disease with organising pneumonia on histopathology with favourable outcome on early combined treatment with corticosteroids, mycophenolate mofetil and rituximab. LID - 10.1136/bcr-2019-231006 [doi] LID - e231006 AB - Antisynthetase syndrome is a rare autoimmune disease and represents a distinct entity within the idiopathic inflammatory myopathies. Its variable systemic manifestations are composed of myositis, interstitial lung disease, non-erosive arthritis, fever, Raynaud's phenomenon, hyperkeratotic skin changes and the presence of antibodies against aminoacyl-transfer-RNA-synthetases. Interstitial lung disease is the major determinant of morbidity and mortality. The role of lung biopsy remains controversial but it might be considered on an individual basis and may provide information regarding prognosis and treatment response. An integrated clinical, radiological and pathological approach to interstitial lung disease has to be emphasised. Due to the rarity of the disease, no standardised treatment guidelines for antisynthetase syndrome exist. We discuss a patient with anti-Jo1-autoantibody antisynthetase syndrome with proximal myositis and severe, rapid onset, interstitial lung disease with a histopathological pattern of organising pneumonia on surgical lung biopsy and good response to early combined immunosuppressive treatment with corticosteroids, mycophenolate mofetil and rituximab. CI - © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Rüegg, Christine A AU - Rüegg CA AUID- ORCID: 0000-0002-3130-9962 AD - Division of Pulmonology, University Hospital Zurich, Zurich, Switzerland. FAU - Maurer, Britta AU - Maurer B AD - Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Laube, Irène AU - Laube I AD - Division of Pulmonology, Stadtspital Triemli, Zurich, Switzerland. FAU - Scholtze, Dieter AU - Scholtze D AUID- ORCID: 0000-0003-0202-5669 AD - Division of Pulmonology, Stadtspital Triemli, Zurich, Switzerland. LA - eng PT - Case Reports PT - Journal Article DEP - 20190912 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Autoantibodies) RN - 0 (Enzyme Inhibitors) RN - 0 (Immunologic Factors) RN - 0 (Immunosuppressive Agents) RN - 4F4X42SYQ6 (Rituximab) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - HU9DX48N0T (Mycophenolic Acid) RN - Antisynthetase syndrome SB - IM MH - Administration, Intravenous MH - Adrenal Cortex Hormones/administration & dosage/therapeutic use MH - Amino Acyl-tRNA Synthetases/immunology MH - Autoantibodies MH - Combined Modality Therapy/*methods MH - Enzyme Inhibitors/therapeutic use MH - Female MH - Humans MH - Immunologic Factors/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Lung/pathology MH - Lung Diseases, Interstitial/diagnostic imaging/*drug therapy/immunology/pathology MH - Middle Aged MH - Mycophenolic Acid/*therapeutic use MH - Myositis/*drug therapy/immunology/pathology MH - Pneumonia/etiology/*pathology MH - Rituximab/therapeutic use MH - Severity of Illness Index MH - Treatment Outcome PMC - PMC6747926 OTO - NOTNLM OT - Biological Agents OT - Connective Tissue Disease OT - Interstitial Lung Disease COIS- Competing interests: None declared. EDAT- 2019/09/15 06:00 MHDA- 2020/02/13 06:00 PMCR- 2021/09/12 CRDT- 2019/09/15 06:00 PHST- 2019/09/15 06:00 [entrez] PHST- 2019/09/15 06:00 [pubmed] PHST- 2020/02/13 06:00 [medline] PHST- 2021/09/12 00:00 [pmc-release] AID - 12/9/e231006 [pii] AID - bcr-2019-231006 [pii] AID - 10.1136/bcr-2019-231006 [doi] PST - epublish SO - BMJ Case Rep. 2019 Sep 12;12(9):e231006. doi: 10.1136/bcr-2019-231006. PMID- 30303671 OWN - NLM STAT- MEDLINE DCOM- 20181211 LR - 20260127 IS - 0023-2149 (Print) IS - 0023-2149 (Linking) VI - 95 IP - 2 DP - 2017 TI - [MODERN CLINICAL EPIDEMIOLOGICAL CHARACTERISTIC OF LUPUS ERYTHEMATOSUS BASED ON THE DATA OF REGIONAL REGISTRY]. PG - 140-7 AB - Systemic lupus erythematosus (SLE) is a severe rheumatic disease characterized by polysymptomatic clinical picture. At the present stage, there are no updated epidemiological data due to the low prevalence of the disease. The aim of the study was to examine the current clinical and epidemiological characteristics of patients with systemic lupus erythematosus based on the information contained in the territorial register, analysis of occurrence and symptoms at the early stage of the disease. This study demonstrated the epidemiological and clinical characteristics of SLE from the analysis of 107 cases during the period from 2011 to 2013 and retrospective analysis of the cases for 1980-2013. The epidemiological situation was evaluated based on extensive and intensive indicators using statistical software license. The current SLE prevalence was estimated at 5,59 per 100 000 population in 2013, the incidence between 1994 and 2003 at 0,29 per 100 000 population and between 2004 and 2013 at 0,49, with the peak in 2010 up to 1,35 per 100 000 population. The average absolute growth and growth rate of SLE in the first decade was 0,05% and 0,24%, in the second decade 0,001% and 0,006% respectively, with the female to male ratio being 9:1, mean age of the patients 37,62±11,65 years), and ethnic composition of 87 Slavs and 15 Crimean Tatars. The most common symptoms at the early (polyarthritis, fever, dermatitis) and advanced (polyarthritis, Raynaud's syndrome, carditis, myalgia) stages differed from those specified by American College of Rheumatology (1997). The difference between early and late symptoms of SLE was documented . Based on the data obtained, the division of the disease into clinical subtypes (phenotypes) is proposed. FAU - Shaduro, D V AU - Shaduro DV FAU - Beloglazov, V A AU - Beloglazov VA FAU - Petrov, A V AU - Petrov AV FAU - Aliev, K A AU - Aliev KA LA - rus PT - Journal Article PL - Russia (Federation) TA - Klin Med (Mosk) JT - Klinicheskaia meditsina JID - 2985204R SB - IM MH - Adolescent MH - Adult MH - Age Factors MH - Female MH - Humans MH - Incidence MH - *Lupus Erythematosus, Systemic/diagnosis/epidemiology MH - Male MH - Middle Aged MH - Patient Acuity MH - Prevalence MH - Registries/statistics & numerical data MH - Retrospective Studies MH - Russia/epidemiology MH - Symptom Assessment/methods/statistics & numerical data EDAT- 2017/01/01 00:00 MHDA- 2017/01/01 00:01 CRDT- 2018/10/11 06:00 PHST- 2018/10/11 06:00 [entrez] PHST- 2017/01/01 00:00 [pubmed] PHST- 2017/01/01 00:01 [medline] PST - ppublish SO - Klin Med (Mosk). 2017;95(2):140-7. PMID- 22281893 OWN - NLM STAT- MEDLINE DCOM- 20120928 LR - 20211203 IS - 1806-4841 (Electronic) IS - 0365-0596 (Linking) VI - 86 IP - 6 DP - 2011 Nov-Dec TI - Autoantibodies in scleroderma and their association with the clinical profile of the disease. A study of 66 patients from southern Brazil. PG - 1075-81 LID - S0365-05962011000600003 [pii] AB - BACKGROUND: Scleroderma is a fairly rare connective tissue disease whose autoantibody profile is associated with different clinical manifestations. The prevalence of autoantibodies in scleroderma is influenced by race and genetics. OBJECTIVE: To study the prevalence of anti-Scl-70, anti-centromere (ACA) and anti-U1-RNP antibodies in patients with scleroderma in southern Brazil and verify their association with clinical manifestations of the disease. METHODS: A retrospective study involving 66 patients with scleroderma for the presence of anti-Scl-70, anti-centromere and anti-U1-RNP and of clinical manifestations such as Raynaud's phenomenon, digital micro scars, digital necrosis, telangiectasias, calcinosis, pulmonary fibrosis, pleuritis, pericarditis, cardiomyopathy, arthralgia and arthritis, skin sclerosis, joint contractures, tendon friction rubs, pulmonary hypertension, esophageal disorders and renal crisis. RESULTS: The prevalence of anti-Scl-70 was 17.8% , that of ACA was 33.3% and the prevalence of U1 RNP was 11.8%. Anti-Scl-70 was associated with the diffuse form of the disease (p = 0.015), presence of cardiomyopathies (p = 0.016) and digital micro scars (p = 0.05). Anti-centromere was more common in the limited form, although it was not statistically significant, and had a protective role associated with cardiomyopathies (p = 0.005). Anti-U1-RNP was more common in the overlap forms (p = 0.0004). CONCLUSION: The prevalence and profile of clinical associations of autoantibodies in Brazilian patients with scleroderma are similar to those found in the literature. FAU - Skare, Thelma Larocca AU - Skare TL AD - Hospital Universitário Evangélico de Curitiba, Faculdade Evangélica do Paraná, Curitiba, PR, Brazil. tskare@onda.com.br FAU - Fonseca, Adriano Erlon AU - Fonseca AE FAU - Luciano, Alan Campos AU - Luciano AC FAU - Azevedo, Pedro Ming AU - Azevedo PM LA - eng LA - por PT - Journal Article PL - Spain TA - An Bras Dermatol JT - Anais brasileiros de dermatologia JID - 0067662 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Nuclear Proteins) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 0 (Scl 70 antigen, human) RN - 0 (anticentromere antibody) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear MH - Autoantibodies/*immunology MH - Brazil/epidemiology MH - DNA Topoisomerases, Type I MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nuclear Proteins/*analysis MH - Retrospective Studies MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Scleroderma, Systemic/epidemiology/*immunology MH - Young Adult EDAT- 2012/01/28 06:00 MHDA- 2012/09/29 06:00 CRDT- 2012/01/28 06:00 PHST- 2010/07/13 00:00 [received] PHST- 2010/12/09 00:00 [accepted] PHST- 2012/01/28 06:00 [entrez] PHST- 2012/01/28 06:00 [pubmed] PHST- 2012/09/29 06:00 [medline] AID - S0365-05962011000600003 [pii] AID - 10.1590/s0365-05962011000600003 [doi] PST - ppublish SO - An Bras Dermatol. 2011 Nov-Dec;86(6):1075-81. doi: 10.1590/s0365-05962011000600003. PMID- 16874782 OWN - NLM STAT- MEDLINE DCOM- 20060915 LR - 20060828 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 55 IP - 4 DP - 2006 Aug 15 TI - Scleroderma lung: initial forced vital capacity as predictor of pulmonary function decline. PG - 598-602 AB - OBJECTIVE: To determine the ability of initial forced vital capacity (FVC) of patients with scleroderma to predict subsequent pulmonary function deterioration. METHODS: Data on 78 patients with scleroderma were retrospectively collected and analyzed. FVC (percent predicted), diffusing capacity for carbon monoxide (percent predicted), and various clinical and laboratory parameters were recorded. Pulmonary function decline (outcome) was defined as at least a 15-point sustained decrease in FVC (percent predicted). Kaplan-Meier analyses were performed separately for 60 patients initially assessed within the first 3 years from disease onset (group A) and 16 patients whose FVC values in the fourth or fifth year from disease onset were ascribed as baseline measurements (group B). RESULTS: Based on baseline FVC, patients in each group were categorized into those with normal FVC (> or =80% predicted) and those with decreased FVC (<80% predicted). In group A, the percent-predicted FVC of 89% of patients with normal initial FVC and of 75% of patients with reduced baseline FVC did not decrease by > or =15 points at 5 years (log rank P = 0.04). Four patients with decreased baseline FVC developed respiratory failure (FVC <50% predicted) versus none with normal initial FVC. Analysis of group B showed no difference between patients with normal baseline FVC and those with decreased FVC in the ability to further predict pulmonary function decline (log rank P = 0.13). Clinical and laboratory parameters (age, male sex, baseline diffusion capacity, anti-topoisomerase I, or duration of Raynaud's phenomenon preceding skin manifestations) were not associated with pulmonary function decline. CONCLUSION: Measured within the first 3 years from disease onset, baseline FVC (percent predicted) may predict deterioration of pulmonary function in patients with scleroderma. Patients with normal pulmonary function at initial assessment are at low risk to develop considerable impairment of pulmonary function. FAU - Plastiras, Sotiris C AU - Plastiras SC AD - National University of Athens School of Medicine, and Laiko General Hospital, Athens, Greece. FAU - Karadimitrakis, Stylianos P AU - Karadimitrakis SP FAU - Ziakas, Panayiotis D AU - Ziakas PD FAU - Vlachoyiannopoulos, Panayiotis G AU - Vlachoyiannopoulos PG FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM FAU - Tzelepis, George E AU - Tzelepis GE LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Adult MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Greece MH - Humans MH - Lung/*physiopathology MH - Male MH - Middle Aged MH - Outpatients MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/*physiopathology MH - Time Factors MH - *Vital Capacity EDAT- 2006/07/29 09:00 MHDA- 2006/09/16 09:00 CRDT- 2006/07/29 09:00 PHST- 2006/07/29 09:00 [pubmed] PHST- 2006/09/16 09:00 [medline] PHST- 2006/07/29 09:00 [entrez] AID - 10.1002/art.22099 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Aug 15;55(4):598-602. doi: 10.1002/art.22099. PMID- 16681482 OWN - NLM STAT- MEDLINE DCOM- 20060802 LR - 20161128 IS - 1743-6095 (Print) IS - 1743-6095 (Linking) VI - 3 IP - 3 DP - 2006 May TI - The penile vasculature in systemic sclerosis: A duplex ultrasound study. PG - 554-8 AB - INTRODUCTION: Systemic sclerosis is a connective tissue disease characterized by Raynaud's phenomenon, degenerative changes and vascular lesions in the presence of thickened, sclerotic skin lesions determined by cellular proliferation, and excess of extracellular matrix production. The role of ultrasound in the investigation of penile pathology is well established as vasculogenic impotence accounts for more than 30% out of overall causes. AIM: In this article, we report for the first time the extent of penile vascular damage in a series of 15 sclerodermic patients (mean age 47 +/- 12.5 SD) under current treatment for their disease irrespective of their sexual dysfunction complaints. METHODS: After disease classification (mean duration of disease 7.2 +/- 5.1 years), all patients were interviewed about the presence or absence of erectile dysfunction (ED) by using the Sexual Health Inventory for Men (SHIM) questionnaire, and then blood flow velocity in the cavernous artery following standardized pharmacostimulation was determined with Duplex ultrasonography along with the intima media thickness (IMT) of the common carotid artery, a valid index for atherosclerosis. RESULTS: Mean SHIM scores revealed the presence of moderate-to-severe ED (mean 13.3 +/- 6.3). Interestingly, in all patients diffuse hyperechoic "spots" inside the corpora cavernosa along with thickening of the tunica albuginea were found. Severely impaired mean peak systolic velocities (20.2 +/- 5.5 cm/second) in the presence of mild venous leakage as expressed by mean end diastolic velocities (4.6 +/- 2.9 cm/second) were found along with normal IMT (0.065 +/- 0.010 cm) and acceleration time (92.3 +/- 32.7 cm/second). CONCLUSION: Penile fibrosis almost invariably occurs in sclerodermic patients and this determines incomplete penile arterial and smooth muscle cell relaxation and ED despite the absence of indirect signs of early atherosclerosis, that is, abnormal IMT and acceleration time. FAU - Aversa, Antonio AU - Aversa A AD - Department of Medical Pathophysiology, University of Rome Lae Sapienza, Rome, Italy. antonio.aversa@uniroma1.it FAU - Proietti, Michele AU - Proietti M FAU - Bruzziches, Roberto AU - Bruzziches R FAU - Salsano, Felice AU - Salsano F FAU - Spera, Giovanni AU - Spera G LA - eng PT - Journal Article PL - Netherlands TA - J Sex Med JT - The journal of sexual medicine JID - 101230693 SB - IM MH - Adult MH - Aged MH - Fibrosis/diagnostic imaging MH - Humans MH - Impotence, Vasculogenic/*diagnostic imaging/*etiology MH - Male MH - Middle Aged MH - Penis/*blood supply/*diagnostic imaging/pathology MH - Scleroderma, Systemic/*complications/diagnostic imaging MH - Ultrasonography EDAT- 2006/05/10 09:00 MHDA- 2006/08/03 09:00 CRDT- 2006/05/10 09:00 PHST- 2006/05/10 09:00 [pubmed] PHST- 2006/08/03 09:00 [medline] PHST- 2006/05/10 09:00 [entrez] AID - S1743-6095(15)31340-0 [pii] AID - 10.1111/j.1743-6109.2005.00169.x [doi] PST - ppublish SO - J Sex Med. 2006 May;3(3):554-8. doi: 10.1111/j.1743-6109.2005.00169.x. PMID- 39337037 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240930 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 13 IP - 18 DP - 2024 Sep 19 TI - Nailfold Capillaroscopy Changes in Patients with Idiopathic Inflammatory Myopathies. LID - 10.3390/jcm13185550 [doi] LID - 5550 AB - Background/Objectives: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders characterized by progressive proximal muscle weakness and varying extra-muscular manifestations. The latest 2017 EULAR/ACR criteria classify them into subgroups. This study aims to evaluate the role of nailfold capillaroscopy (NFC) as a diagnostic and prognostic tool in IIMs by comparing capillaroscopic patterns across different IIM subtypes. Methods: We conducted an observational, cross-sectional study at the Institute of Rheumatology in Belgrade, analyzing 90 patients diagnosed with IIMs per the 2017 EULAR/ACR criteria. Patients were categorized into dermatomyositis (DM) (n = 37), polymyositis (PM) (n = 35), amyopathic dermatomyositis (ADM) (n = 13), and juvenile dermatomyositis (JDM) (n = 5). A control group of 35 patients with primary Raynaud's phenomenon was also included. NFC findings, clinical manifestations, and laboratory data were compared across the groups. Results: In DM, 81.9% exhibited a scleroderma capillaroscopic pattern, which was also present in 76.9% of ADM patients. In PM, the most common pattern was nonspecific changes (48.6%). JDM patients showed a high prevalence of scleroderma changes (n = 4 (80%)). Scleroderma patterns correlated with Gottron's papules, heliotrope rash, periungual erythema, Raynaud's phenomenon, and interstitial lung disease (ILD). No significant differences were found in laboratory parameters across capillaroscopic groups, except for a higher prevalence of anti-Jo1 antibodies in patients with nonspecific capillaroscopic changes. Conclusions: NFC is a valuable tool for differentiating IIM subtypes and correlating clinical manifestations with specific capillaroscopic patterns. The high prevalence of scleroderma changes in DM and ADM suggests their potential as a diagnostic and prognostic marker in IIMs. Further research with larger cohorts is warranted to validate these findings. FAU - Bogojevic, Milan AU - Bogojevic M AUID- ORCID: 0000-0003-1037-1342 AD - Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro. FAU - Markovic Vlaisavljevic, Milica AU - Markovic Vlaisavljevic M AUID- ORCID: 0009-0009-2488-4975 AD - Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro. FAU - Medjedovic, Rifat AU - Medjedovic R AUID- ORCID: 0009-0004-6296-1218 AD - Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro. FAU - Strujic, Elvira AU - Strujic E AD - Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro. FAU - Pravilovic Lutovac, Dragana AU - Pravilovic Lutovac D AD - Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AD - Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia. LA - eng PT - Journal Article DEP - 20240919 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC11432190 OTO - NOTNLM OT - dermatomyositis OT - idiopathic inflammatory myopathies OT - nailfold capillaroscopy OT - polymyositis OT - scleroderma pattern COIS- The authors declare no conflicts of interest. EDAT- 2024/09/28 22:44 MHDA- 2024/09/28 22:45 PMCR- 2024/09/19 CRDT- 2024/09/28 01:14 PHST- 2024/09/09 00:00 [received] PHST- 2024/09/15 00:00 [revised] PHST- 2024/09/17 00:00 [accepted] PHST- 2024/09/28 22:45 [medline] PHST- 2024/09/28 22:44 [pubmed] PHST- 2024/09/28 01:14 [entrez] PHST- 2024/09/19 00:00 [pmc-release] AID - jcm13185550 [pii] AID - jcm-13-05550 [pii] AID - 10.3390/jcm13185550 [doi] PST - epublish SO - J Clin Med. 2024 Sep 19;13(18):5550. doi: 10.3390/jcm13185550. PMID- 22933925 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120830 LR - 20211021 IS - 1581-3207 (Electronic) IS - 1318-2099 (Print) IS - 1318-2099 (Linking) VI - 44 IP - 4 DP - 2010 Dec TI - Digital ischemic events related to gemcitabine: Report of two cases and a systematic review. PG - 257-61 LID - 10.2478/v10019-010-0020-1 [doi] AB - BACKGROUND: Gemcitabine is a potent cytotoxic agent used in the treatment of many solid tumours, sarcomas and lymphomas. Vascular toxicity and thrombotic events related to gemcitabine seem to be underreported. CASE REPORT: We report two cases of gemcitabine related digital ischemic events. Case 1. A 65-year-old man was given the first-line treatment with gemcitabine for the advanced adenocarcinoma of pancreas. After four weekly doses of gemcitabine (total dose 4000 mg/m(2)) he presented with Raynaud's like phenomenon and ischemic fingertips necrosis in five digits of both hands. Symptoms resolved in all but one digit after stopping chemotherapy and treatment with iloprost trometamol infusion. Case 2. A 77-year-old man, ex-smoker, was administered a combination of gemcitabine and cisplatin as the first-line treatment for the locally advanced bladder cancer. After 4 cycles of the treatment (total dose of gemcitabine 4000 mg/m(2)) the patient suffered digital ischemia and necrosis on two digits of a right leg. Arteriography revealed preexisting peripheral arterial occlusive disease (PAOD) of both legs with very good peripheral collateral circulation and absent microcirculation of affected two digits. The gemcitabine treatment was stopped and the patient was treated with iloprost trometamol infusion and percutaneous transluminal angioplasty with dilatation of the right superficial femoral artery. Digital changes resolved without consequences. Severe thrombocytosis (platelet count 1211 × 10(9)/L) might have also contributed to the ischemic digital event in the second case. CONCLUSIONS: Digital ischemic events associated with gemcitabine chemotherapy seem to be more common in patients with tobacco-associated cancers, especially when used in combination with platinum salt. The treatment with gemcitabine in patients with evolving Raynaud's phenomenon and/or preexisting PAOD should be done with caution. FAU - Kuhar, Cvetka Grasic AU - Kuhar CG AD - Department of Medical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia. FAU - Mesti, Tanja AU - Mesti T FAU - Zakotnik, Branko AU - Zakotnik B LA - eng PT - Journal Article DEP - 20100503 PL - Poland TA - Radiol Oncol JT - Radiology and oncology JID - 9317213 PMC - PMC3423709 OTO - NOTNLM OT - chemotherapy OT - digital ischemic events OT - gemcitabine vascular toxicity COIS- Disclosure: No potential conflicts of interest were disclosed. EDAT- 2010/12/01 00:00 MHDA- 2010/12/01 00:01 PMCR- 2010/12/01 CRDT- 2012/08/31 06:00 PHST- 2010/02/01 00:00 [received] PHST- 2010/03/01 00:00 [accepted] PHST- 2012/08/31 06:00 [entrez] PHST- 2010/12/01 00:00 [pubmed] PHST- 2010/12/01 00:01 [medline] PHST- 2010/12/01 00:00 [pmc-release] AID - rado-44-04-257 [pii] AID - 10.2478/v10019-010-0020-1 [doi] PST - ppublish SO - Radiol Oncol. 2010 Dec;44(4):257-61. doi: 10.2478/v10019-010-0020-1. Epub 2010 May 3. PMID- 39867814 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250302 IS - 2090-6846 (Print) IS - 2090-6854 (Electronic) IS - 2090-6854 (Linking) VI - 2025 DP - 2025 TI - Diffuse Alveolar Hemorrhage Associated With Anti-PL-7 Antisynthetase Syndrome: A Case Report. PG - 3715449 LID - 10.1155/crpu/3715449 [doi] LID - 3715449 AB - Background: Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening condition which can present with hemoptysis, diffuse alveolar infiltrates, anemia, and hypoxic respiratory failure. Antisynthetase syndrome (AS) is a rare autoimmune disorder most often characterized by nonerosive arthritis, proximal muscle weakness with elevated muscle enzymes, Raynaud's phenomenon, hyperkeratosis of the digits (mechanic's hands), and interstitial lung disease. According to large population studies, AS has an annual incidence of 0.56 per 100,000 persons and prevalence of 9 per 100,000. The most common autoantibody is anti-aminoacyl-transfer RNA synthetase for histidine (anti-Jo-1) with a reported prevalence of 20%-30%, whereas anti-Pl-7 (for threonine) accounts for less than 5% of all autoimmune myositis. Specific myositis autoantibodies determine clinical phenotype. PL-7 is characterized by interstitial lung disease, myositis, and arthritis. Autoimmune myositis, specifically AS, is a rare cause of DAH. Herein, we describe the first reported case of PL-7-associated AS with DAH. Case Presentation: A 41-year-old female presented with worsening shortness of breath and hemoptysis. Laboratory studies included a hemoglobin of 10.5 g/dL, mildly elevated liver enzymes, and a creatine phosphokinase (CPK) of nearly 4000 U/L. CT of the chest showed diffuse ground glass opacities bilaterally. Serial aliquots of the bronchoalveolar lavage (BAL) fluid revealed progressively hemorrhagic return and histopathologic analysis consistent with DAH. Other concurrent causes of DAH were ruled out. Conclusion: Although rare, AS should be considered a cause of DAH, particularly in patients presenting with symptoms of muscle weakness and arthritis or with evidence of mechanic's hands. CI - Copyright © 2025 Paul Shiu et al. Case Reports in Pulmonology published by John Wiley & Sons Ltd. FAU - Shiu, Paul AU - Shiu P AUID- ORCID: 0000-0001-7773-1985 AD - Prisma Health, University of South Carolina-School of Medicine, Columbia, South Carolina, USA. FAU - Iriza, Shannon AU - Iriza S AUID- ORCID: 0009-0007-7975-2123 AD - Prisma Health, University of South Carolina-School of Medicine, Columbia, South Carolina, USA. FAU - Templeton, Steven AU - Templeton S AUID- ORCID: 0009-0004-1722-4454 AD - Prisma Health, University of South Carolina-School of Medicine, Columbia, South Carolina, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250117 PL - United States TA - Case Rep Pulmonol JT - Case reports in pulmonology JID - 101585355 PMC - PMC11759568 COIS- The authors declare no conflicts of interest. EDAT- 2025/01/27 06:20 MHDA- 2025/01/27 06:21 PMCR- 2025/01/17 CRDT- 2025/01/27 05:57 PHST- 2024/06/18 00:00 [received] PHST- 2024/11/11 00:00 [accepted] PHST- 2025/01/27 06:21 [medline] PHST- 2025/01/27 06:20 [pubmed] PHST- 2025/01/27 05:57 [entrez] PHST- 2025/01/17 00:00 [pmc-release] AID - 10.1155/crpu/3715449 [doi] PST - epublish SO - Case Rep Pulmonol. 2025 Jan 17;2025:3715449. doi: 10.1155/crpu/3715449. eCollection 2025. PMID- 38572051 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240405 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 69 IP - 1 DP - 2024 Jan-Feb TI - Systemic Sclerosis in Males-Deciphering the Enigma of Erasmus Syndrome. PG - 48-56 LID - 10.4103/ijd.ijd_531_23 [doi] AB - BACKGROUND: Systemic sclerosis is an autoimmune connective tissue disease characterized by fibrosis in skin and internal organs. Chronic exposure to silica may not only lead to silicosis of lungs but also systemic sclerosis. Systemic sclerosis is relatively commoner in females; however, occupational exposure to silica in males makes them vulnerable to silica--associated systemic sclerosis (Erasmus syndrome). OBJECTIVE: To describe the clinico-epidemiological aspects of systemic sclerosis in males in a retrospective cohort study. MATERIALS AND METHODS: The data were analysed retrospectively for demographic profile and clinical characteristics including examination findings, laboratory investigations, and treatments of all male patients diagnosed with systemic sclerosis with or without silica exposure, managed from January 2018 to December 2021. RESULTS: Eight out of twelve patients were having silica exposure in the form of stone cutting, cement exposure, and working with concrete. The average age was 55 ± 10.72 years with average smoking exposure of around 24.4 ± 12.8 pack years. Skin thickening was reported by all patients with an average modified Rodnan score of 18.33/51 in diffuse and 7/51 in limited cutaneous systemic sclerosis. Raynaud's phenomenon and sclerodactyly were universal findings, while 9 (75%) patients had digital pitted scars. Antinuclear antibodies were present in all patients and specific antibodies substantiated the clinical assessment in almost all patients. Interstitial lung disease was the most common systemic finding present in 11 (91%) patients and tuberculosis was diagnosed in 2 (25%) cases with silica exposure. Gastrointestinal and cardiac system involvement was seen in 5 (41.6%) and 4 (33.3%) patients, respectively. CONCLUSION: Systemic sclerosis in males against the gender predilection indicates the role of occupational exposure. Silicosis and systemic sclerosis synergistically add to lung damage, and at the same time, these patients are more prone to infections like tuberculosis. CI - Copyright: © 2024 Indian Journal of Dermatology. FAU - Sharma, Reena K AU - Sharma RK AD - From the Department of Dermatology, Venereology and Leprosy, Dr. Rajendra Prasad Government Medical College, Kangra, Himachal Pradesh, India. FAU - Gupta, Mudita AU - Gupta M AD - Department of Dermatology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India. LA - eng PT - Journal Article DEP - 20240227 PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC10986861 OTO - NOTNLM OT - Erasmus syndrome OT - and silicosis OT - systemic sclerosis COIS- There are no conflicts of interest. EDAT- 2024/04/04 06:45 MHDA- 2024/04/04 06:46 PMCR- 2024/01/01 CRDT- 2024/04/04 04:03 PHST- 2024/04/04 06:46 [medline] PHST- 2024/04/04 06:45 [pubmed] PHST- 2024/04/04 04:03 [entrez] PHST- 2024/01/01 00:00 [pmc-release] AID - IJD-69-48 [pii] AID - 10.4103/ijd.ijd_531_23 [doi] PST - ppublish SO - Indian J Dermatol. 2024 Jan-Feb;69(1):48-56. doi: 10.4103/ijd.ijd_531_23. Epub 2024 Feb 27. PMID- 32378284 OWN - NLM STAT- MEDLINE DCOM- 20201211 LR - 20241008 IS - 1529-8019 (Electronic) IS - 1396-0296 (Print) IS - 1396-0296 (Linking) VI - 33 IP - 5 DP - 2020 Sep TI - Chilblain-like lesions in pediatrics dermatological outpatients during the COVID-19 outbreak. PG - e13516 LID - 10.1111/dth.13516 [doi] LID - e13516 AB - In Spain, with full confinement measures and coinciding with the pandemic, pediatricians and dermatologists have received, through teledermatology/teleconsultation and social networks, a barrage of diverse images, which have subsequently allowed us to approach some of them by direct physical examination of early and late skin manifestations associated with SARS-Cov-2 infection. We designed a retrospective, cross-sectional study to evaluate the dermatological care of all those patients under the age of 16 who consulted, in person or telematically, for acral lesions (chilblain-like or erythema multiforme-like) in the context of the Coronavirus disease (COVID-19) pandemic, since 15 March 2020 to 24 April 2020, both included in the health area of the Hospital Universitario San Cecilio de Granada. Of all the patients collected, 18 (66%) were male and the overall mean age was 14.44 years. All lacked a personal history of interest and denied previous episodes of chilblains or Raynaud's phenomenon/disease. The clinic was limited to purpuric lesions located on acral regions distributed on hands and feet. Dermatologists and pediatricians should be aware of the lesions associated with COVID-19 infection and their possible complications. It remains to be identified if there are different dermatological patterns in the pediatric and adult population. CI - © 2020 Wiley Periodicals LLC. FAU - Garcia-Lara, G AU - Garcia-Lara G AD - Servicio de Pediatría, Hospital Universitario San Cecilio, Granada, Spain. FAU - Linares-González, Laura AU - Linares-González L AD - Servicio de Dermatología, Hospital Universitario San Cecilio, Instituto Biosanitario Ibs de Granada, Granada, Spain. FAU - Ródenas-Herranz, Teresa AU - Ródenas-Herranz T AUID- ORCID: 0000-0001-8367-2432 AD - Servicio de Dermatología, Hospital Universitario San Cecilio, Instituto Biosanitario Ibs de Granada, Granada, Spain. FAU - Ruiz-Villaverde, Ricardo AU - Ruiz-Villaverde R AUID- ORCID: 0000-0002-0381-6174 AD - Servicio de Dermatología, Hospital Universitario San Cecilio, Instituto Biosanitario Ibs de Granada, Granada, Spain. LA - eng PT - Comment PT - Journal Article DEP - 20200623 PL - United States TA - Dermatol Ther JT - Dermatologic therapy JID - 9700070 SB - IM CON - J Eur Acad Dermatol Venereol. 2020 May;34(5):e212-e213. doi: 10.1111/jdv.16387. PMID: 32215952 MH - Adolescent MH - Adult MH - *Betacoronavirus MH - *COVID-19 MH - *Chilblains/diagnosis/epidemiology MH - Child MH - *Coronavirus MH - Cross-Sectional Studies MH - Humans MH - Male MH - Outpatients MH - Pandemics MH - *Pediatrics MH - *Pneumonia, Viral/epidemiology MH - Retrospective Studies MH - SARS-CoV-2 MH - *Skin Diseases MH - Spain/epidemiology PMC - PMC7261972 OTO - NOTNLM OT - COVID 19 OT - chilblain-like lesions OT - pediatrics COIS- The authors declare no potential conflict of interest. EDAT- 2020/05/08 06:00 MHDA- 2020/12/15 06:00 PMCR- 2020/06/23 CRDT- 2020/05/08 06:00 PHST- 2020/04/26 00:00 [received] PHST- 2020/05/03 00:00 [accepted] PHST- 2020/05/08 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2020/05/08 06:00 [entrez] PHST- 2020/06/23 00:00 [pmc-release] AID - DTH13516 [pii] AID - 10.1111/dth.13516 [doi] PST - ppublish SO - Dermatol Ther. 2020 Sep;33(5):e13516. doi: 10.1111/dth.13516. Epub 2020 Jun 23. PMID- 32292667 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240728 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 12 IP - 3 DP - 2020 Mar 12 TI - The Effects of Spinal Cord Stimulators on End Organ Perfusion: A Literature Review. PG - e7253 LID - 10.7759/cureus.7253 [doi] LID - e7253 AB - Spinal cord stimulators (SCS) have been gaining momentum in the last decade as their role in the management of chronic pain has become more apparent. Our intention was to search, analyze and highlight the effects of spinal cord stimulators on end-organ perfusion. We also looked at vascular diseases of atherosclerotic and nonatherosclerotic nature by examining objective evidence of improved circulation, pain control, limb salvage, and quality of life. We paid specific attention to disease processes such as cerebral hypoperfusion, Chronic-Critical Limb Ischemia, Intractable Angina Pectoris (IAP), Raynaud's syndrome and Thromboangiitis Obliterans. We performed a Medline database search for medical literature relevant to Spinal cord stimulators encompassing the years 1950 to 2019. Search terms included "Spinal cord stimulator," plus one of the following search terms: vasculopathy, stroke, cerebral blood flow, angina pectoris, diabetic ulcers, chronic critical leg ischemia, thromboangiitis obliterans and peripheral vascular disease. We included both clinical and experimental human studies that investigated the effect of SCS's on end-organ perfusion. We also investigated the pathophysiological mechanism of action of SCS's on the vasculature. We found 497 articles of which 43 more relevant and impactful articles investigating the hemodynamic effects of SCS and its possible mechanism were selected. Animal studies were excluded from the literature review as they provided heterogeneity. In addition to reporting literature supporting the use of stimulators for currently FDA approved uses, we also actively looked for potential future uses. Spinal Cord stimulators showed improvement in cerebral blood flow, increased capillary recruitment, and better quality of life in many studies. Patients also had increased exercise capacity and a significant reduction in the use of narcotic drug use and daily anginal attacks in patients suffering from IAP. CI - Copyright © 2020, Saini et al. FAU - Saini, Harneel S AU - Saini HS AD - Neurology, Allegheny General Hospital, Pittsburgh, USA. FAU - Shnoda, Mina AU - Shnoda M AD - Internal Medicine, Allegheny Health Network, Pittsburgh, USA. FAU - Saini, Ishveen AU - Saini I AD - Internal Medicine, Lake Erie College of Osteopathic Medicine, Erie, USA. FAU - Sayre, Matthew AU - Sayre M AD - Internal Medicine, Lewis Katz School of Medicine at Temple University, Philadelphia, USA. FAU - Tariq, Shahzaib AU - Tariq S AD - Neurology, Allegheny Health Network, Pittsburgh, USA. LA - eng PT - Journal Article PT - Review DEP - 20200312 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7152574 OTO - NOTNLM OT - scs OT - spinal cord stimulator COIS- The authors have declared that no competing interests exist. EDAT- 2020/04/16 06:00 MHDA- 2020/04/16 06:01 PMCR- 2020/03/12 CRDT- 2020/04/16 06:00 PHST- 2020/04/16 06:00 [entrez] PHST- 2020/04/16 06:00 [pubmed] PHST- 2020/04/16 06:01 [medline] PHST- 2020/03/12 00:00 [pmc-release] AID - 10.7759/cureus.7253 [doi] PST - epublish SO - Cureus. 2020 Mar 12;12(3):e7253. doi: 10.7759/cureus.7253. PMID- 27747826 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220330 IS - 2199-1154 (Print) IS - 2198-9788 (Electronic) IS - 2198-9788 (Linking) VI - 3 IP - 3 DP - 2016 Sep TI - Seasonal and Geographic Variation in Adverse Event Reporting. PG - 297-306 AB - BACKGROUND: Many illnesses demonstrate seasonal and geographic variations. Pharmacovigilance is unique among public health surveillance systems in terms of the clinical diversity of the events under surveillance. Since many pharmacovigilance signal detection methodologies are geared towards looking for increased frequency of spontaneous adverse drug event (ADE) reporting over variable time frames, seasonality of ADEs may have implications for signal detection. OBJECTIVE: The aim of this study was to investigate whether a set of illnesses that might be expected to display seasonality in general, did so when spontaneously reported as ADEs. METHODS: We performed our analysis with the publically available US FDA Adverse Event Reporting System (FAERS) data. We selected a convenience sample of events possibly triggered by seasonal factors (hypothermia, Raynaud's phenomenon, photosensitivity reaction, heat exhaustion, heat stroke, and sunburn) and events for which previous literature experience suggests seasonality (anencephaly and interstitial lung disease). Our statistical procedures can be explained in terms of a simple physicogeometric setting: the unit circle divided into 6 (semiannual sinusoidal) or 12 (annual sinusoidal) arcs. When reporting frequencies (weights) are more or less evenly distributed across months, the center of mass of the circle would not be significantly displaced from the origin (0, 0). Distinct seasonal patterns will significantly displace the center of mass of the circle. RESULTS: Various patterns of seasonality were identified for some, but not all, events and region-event pairs. USA displayed the most instances of seasonality. Scandinavia did not display seasonality for any events. Seasonality was usually annual sinusoidal. Possible explanations for failure to observe seasonality are briefly considered. CONCLUSIONS: Understanding seasonality of spontaneous ADE reporting may have public health policy and research implications and may mitigate false-positive and missed true-positive pharmacovigilance signals. More systematic study of seasonality of spontaneous ADE reporting, including additional events with more or less biological rationale for seasonality, is a logical extension of this analysis. FAU - Marrero, Osvaldo AU - Marrero O AD - Department of Mathematics and Statistics, Villanova University, Villanova, PA, USA. FAU - Hung, Eric Y AU - Hung EY AD - Pfizer Inc., New York, NY, USA. eric.hung@pfizer.com. FAU - Hauben, Manfred AU - Hauben M AD - Pfizer Inc., New York, NY, USA. AD - New York University Medical Center, New York, NY, USA. LA - eng PT - Journal Article PL - Switzerland TA - Drugs Real World Outcomes JT - Drugs - real world outcomes JID - 101658456 PMC - PMC5042937 COIS- Compliance with Ethical Standards Funding No sources of funding were used to assist in the preparation of this study. Conflict of interest Manfred Hauben and Eric Hung are employees and stockholders of Pfizer, Incorporated. Osvaldo Marrero, Manfred Hauben, and Eric Hung have no other conflicts of interest that are relevant to the content of this study. EDAT- 2016/10/18 06:00 MHDA- 2016/10/18 06:01 PMCR- 2016/06/11 CRDT- 2016/10/18 06:00 PHST- 2016/10/18 06:00 [entrez] PHST- 2016/10/18 06:00 [pubmed] PHST- 2016/10/18 06:01 [medline] PHST- 2016/06/11 00:00 [pmc-release] AID - 10.1007/s40801-016-0081-6 [pii] AID - 81 [pii] AID - 10.1007/s40801-016-0081-6 [doi] PST - ppublish SO - Drugs Real World Outcomes. 2016 Sep;3(3):297-306. doi: 10.1007/s40801-016-0081-6. PMID- 24424190 OWN - NLM STAT- MEDLINE DCOM- 20140507 LR - 20260128 IS - 1873-0183 (Electronic) IS - 1568-9972 (Print) IS - 1568-9972 (Linking) VI - 13 IP - 4-5 DP - 2014 Apr-May TI - Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. PG - 367-71 LID - S1568-9972(14)00034-2 [pii] LID - 10.1016/j.autrev.2014.01.022 [doi] AB - Autoantibodies are a hallmark in the diagnosis of many systemic autoimmune rheumatic diseases (SARD) including idiopathic inflammatory myopathies (IIM). Based on their specificity, autoantibodies in IIM are grouped into myositis specific (MSA) and myositis associated autoantibodies (MAA). Among the MSA, autoantibodies against aminoacyl-tRNA synthetases (ARS) represent the most common antibodies and can be detected in 25-35% of patients. The presence of ARS and other autoantibodies has become a key feature for classification and diagnosis of IIM and is increasingly used to define clinically distinguishable IIM subsets. For example, anti-ARS autoantibodies are the key features of what has become known as anti-synthetase syndrome (aSS), characterized by multiple organ involvement, primarily interstitial lung disease, often accompanied by myositis, non-erosive arthritis, Raynaud's phenomenon, fever, and "mechanic's hands". Autoantibodies directed to eight different ARS have been described: Jo-1 (histidyl), PL-7 (threonyl), PL-12 (alanyl), OJ (isoleucyl), EJ (glycyl), KS (asparaginyl), Zo (phenylalanyl) and Ha (tyrosyl). Each anti-ARS antibody seems to define a distinctive clinical phenotype. Although several research methods and commercial tests are available, routine testing for anti-ARS autoantibodies (other than anti-Jo-1/histidyl-tRNA synthetase) is not widely available, sometimes leading to delays in diagnosis and poor disease outcomes. CI - Copyright © 2014 Elsevier B.V. All rights reserved. FAU - Mahler, Michael AU - Mahler M AD - INOVA Diagnostics, Inc., San Diego, CA, USA. FAU - Miller, Frederick W AU - Miller FW AD - NIEHS, National Institutes of Health, DHHS, Bethesda, MD, USA. FAU - Fritzler, Marvin J AU - Fritzler MJ AD - Department of Medicine, University of Calgary, Calgary, Canada. Electronic address: fritzler@ucalgary.ca. LA - eng GR - ZIA ES101074/ImNIH/Intramural NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Intramural PT - Review DEP - 20140111 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - 0 (Autoantibodies) RN - 0 (Ribonucleoproteins) RN - 0 (SS-A Antigen) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Arthritis/complications/immunology MH - Autoantibodies/immunology MH - Humans MH - Myositis/complications/diagnosis/*immunology MH - Ribonucleoproteins/immunology MH - SS-A Antigen PMC - PMC3970575 MID - NIHMS560375 OTO - NOTNLM OT - Autoantibodies OT - Immunoassay OT - Jo-1 OT - Myositis OT - Synthetase COIS- Competing interests M. Mahler, is employed at INOVA Diagnostics a manufacturer of autoantibody kits and assays. M.J. Fritzler is a paid consultant of ImmunoConcepts Inc. and INOVA Diagnostics and has received diagnostics gifts in kind from Euroimmun. EDAT- 2014/01/16 06:00 MHDA- 2014/05/08 06:00 PMCR- 2015/04/01 CRDT- 2014/01/16 06:00 PHST- 2013/11/13 00:00 [accepted] PHST- 2014/01/16 06:00 [entrez] PHST- 2014/01/16 06:00 [pubmed] PHST- 2014/05/08 06:00 [medline] PHST- 2015/04/01 00:00 [pmc-release] AID - S1568-9972(14)00034-2 [pii] AID - 10.1016/j.autrev.2014.01.022 [doi] PST - ppublish SO - Autoimmun Rev. 2014 Apr-May;13(4-5):367-71. doi: 10.1016/j.autrev.2014.01.022. Epub 2014 Jan 11. PMID- 23917809 OWN - NLM STAT- MEDLINE DCOM- 20140422 LR - 20220331 IS - 1534-3111 (Electronic) IS - 1522-6417 (Linking) VI - 15 IP - 5 DP - 2013 Oct TI - Effectiveness and safety of phosphodiesterase 5 inhibitors in patients with cardiovascular disease and hypertension. PG - 475-83 LID - 10.1007/s11906-013-0377-9 [doi] AB - Phosphodiesterase 5 (PDE 5) inhibitors are selective inhibitors of the enzyme PDE 5, which catalyzes the hydrolysis of cyclic guanosine monophosphate (cGMP), a potent vasodilator and nitric oxide (NO) donor, to its corresponding metabolites (monophosphates). The enzyme PDE 5 is widely distributed in the body, including the heart and blood vessels. Because of its distribution, it was hypothesized that its inhibition could lead to significant coronary vasodilation, which would benefit patients with coronary artery disease (CAD). This hypothesis led to the development of PDE 5 inhibitors with the first being sildenafil citrate. Subsequent studies with sildenafil in patients with CAD demonstrated a modest cardiovascular effect, but a potent action on penile erection in men, resulting in sildenafil becoming a first-line therapy of erectile dysfunction (ED). Subsequently, two more PDE 5 inhibitors (vardenafil and tadalafil) were developed and approved by the Food and Drug Administration (FDA) for the treatment of ED. Recent studies have shown several pleiotropic beneficial effects of PDE 5 inhibitors in patients with CAD, hypertension, heart failure, pulmonary arterial hypertension, diabetes mellitus and Raynaud's phenomenon. Side effects and interactions of PDE 5 inhibitors with other drugs have been minimal, with the exception of their coadministration with nitrates, which could lead to severe vasodilation and hypotension and therefore, their coadministration is prohibited. All these pleiotropic cardiovascular effects of PDE 5 inhibitors and their drug interactions will be discussed in this concise review in the context of the American College of Cardiology / American Heart Association guidelines and the recent developments in this field. FAU - Chrysant, Steven G AU - Chrysant SG AD - University of Oklahoma College of Medicine, 5700 Mistletoe Court, Oklahoma City, OK, 73142, USA, schrysant@yahoo.com. LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Hypertens Rep JT - Current hypertension reports JID - 100888982 RN - 0 (Phosphodiesterase 5 Inhibitors) SB - IM MH - Animals MH - Blood Pressure/physiology MH - Cardiovascular Diseases/complications/*drug therapy MH - Diabetes Mellitus/drug therapy MH - Humans MH - Hypertension/complications/*drug therapy MH - Phosphodiesterase 5 Inhibitors/*adverse effects/*therapeutic use MH - Treatment Outcome EDAT- 2013/08/07 06:00 MHDA- 2014/04/23 06:00 CRDT- 2013/08/07 06:00 PHST- 2013/08/07 06:00 [entrez] PHST- 2013/08/07 06:00 [pubmed] PHST- 2014/04/23 06:00 [medline] AID - 10.1007/s11906-013-0377-9 [doi] PST - ppublish SO - Curr Hypertens Rep. 2013 Oct;15(5):475-83. doi: 10.1007/s11906-013-0377-9. PMID- 23062471 OWN - NLM STAT- MEDLINE DCOM- 20130524 LR - 20201209 IS - 1776-2561 (Electronic) IS - 0761-8417 (Linking) VI - 68 IP - 6 DP - 2012 Dec TI - [Antisynthetase syndrome: a report of four cases and literature review]. PG - 351-60 LID - S0761-8417(12)00097-1 [pii] LID - 10.1016/j.pneumo.2012.07.002 [doi] AB - The antisynthetase syndrome (ASS) includes inflammatory myopathy (polymyositis or dermatomyositis), interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and mechanic's hands, associated with antibodies against aminoacyl-tRNA-synthetases, the most well-recognized being the anti-Jo1 antibody (anti-histidyl-tRNAsynthetase). We report four cases of antisynthetase syndrome and review the clinical characteristics and prognosis factors dominated by ILD. We report the cases of four women with a mean age of 42 years (28-62 years). The interstitial lung disease was found in four cases and was objectified by CT-scan in all cases. The pulmonary symptoms were consisted of dyspnea (one case) and respiratory distress (one case). The anti-Jo1 antibodies were present in the four patients. The myopathy was concomitant with pulmonary involvement (two cases), preceded it in 6 months (one case) and in the course of evolution and after 1 month (one case). All patients received corticosteroid treatment. The immunosuppressive treatment was necessary for two patients because of the severity of the pulmonary involvement. The outcome was favorable in two patients, partially favorable in a patient who presented pulmonary fibrosis. However, one patient died after developing brain abscesses. CI - Copyright © 2012 Elsevier Masson SAS. All rights reserved. FAU - Frikha, F AU - Frikha F AD - Service de médecine interne, CHU Hédi-Chaker, 3029 Sfax, Tunisie. fetenfrikha@yahoo.fr FAU - Saidi, N AU - Saidi N FAU - Snoussi, M AU - Snoussi M FAU - Ben Salah, R AU - Ben Salah R FAU - Ben Ayed, M AU - Ben Ayed M FAU - Daoud, E AU - Daoud E FAU - Hentati, Y AU - Hentati Y FAU - Makni, S AU - Makni S FAU - Mnif, Z AU - Mnif Z FAU - Boudawara, T AU - Boudawara T FAU - Masmoudi, H AU - Masmoudi H FAU - Bahloul, Z AU - Bahloul Z LA - fre PT - Case Reports PT - Journal Article PT - Review TT - Le syndrome des antisynthétases : à propos de quatre observations et revue de la littérature. DEP - 20121010 PL - France TA - Rev Pneumol Clin JT - Revue de pneumologie clinique JID - 8406312 RN - 0 (Antibodies, Antinuclear) RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Dyspnea/etiology MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/diagnosis/immunology MH - Middle Aged MH - Myositis/*diagnosis/drug therapy/immunology MH - Respiratory Distress Syndrome/etiology EDAT- 2012/10/16 06:00 MHDA- 2013/05/28 06:00 CRDT- 2012/10/16 06:00 PHST- 2012/03/16 00:00 [received] PHST- 2012/07/12 00:00 [revised] PHST- 2012/07/19 00:00 [accepted] PHST- 2012/10/16 06:00 [entrez] PHST- 2012/10/16 06:00 [pubmed] PHST- 2013/05/28 06:00 [medline] AID - S0761-8417(12)00097-1 [pii] AID - 10.1016/j.pneumo.2012.07.002 [doi] PST - ppublish SO - Rev Pneumol Clin. 2012 Dec;68(6):351-60. doi: 10.1016/j.pneumo.2012.07.002. Epub 2012 Oct 10. PMID- 20681254 OWN - NLM STAT- MEDLINE DCOM- 20100831 LR - 20151008 IS - 0029-0203 (Print) IS - 0029-0203 (Linking) VI - 114 IP - 7 DP - 2010 Jul TI - [Study on relationship between control of intraocular pressure and systemic risk factor for visual field progression in normal-tension glaucoma]. PG - 592-7 AB - PURPOSE: To study the relationship between the reduction of intraocular pressure (IOP) and visual field (VF) damage in normal-tension glaucoma (NTG) patients with or without systemic risk factors. METHODS: Ninety-two eyes of 92 patients with NTG were enrolled in this study. All patients were followed up for more than 2 years of taking topical antiglaucoma medications. Personal history regarding migraine, diabetes mellitus, hypertension, cerebrovascular disease, cardiovascular disease, Raynaud's phenomenon, arrhythmia, and family history of glaucoma were collected from the medical records. The survival data were analyzed using regression analysis based on the Cox proportional hazards model. The relationship between the presence of each systemic factor and the effect of reduction of VF progression were evaluated using the life table analysis. RESULTS: The progression of VF damage was significantly associated with IOP (p = 0.020). The eyes with larger IOP reduction showed significantly less VF progression than eyes with smaller IOP reduction both in patients with and without systemic diseases (with systemic diseases p = 0.027, without systemic diseases p = 0.025) and a family history of glaucoma (p = 0.019). In patients with a family history of glaucoma, the rate of VF progression did not show statistically differences between the eyes with larger IOP reduction and those with smaller IOP reduction. CONCLUSIONS: IOP reduction is beneficial in reducing the risk of VF progression regardless of the presence of systemic risk factors in patients with NTG. FAU - Nakagami, Takako AU - Nakagami T AD - Department of Ophthalmology, Division of Visual Science, Nihon University School of Medicine, 30-1 Oyaguchikami-machi, Itabashi-ku, Tokyo 173-8610, Japan. nakagami@med.nihon-u.ac.jp FAU - Yamazaki, Yoshio AU - Yamazaki Y FAU - Hayamizu, Fukuko AU - Hayamizu F LA - jpn PT - English Abstract PT - Journal Article PL - Japan TA - Nippon Ganka Gakkai Zasshi JT - Nippon Ganka Gakkai zasshi JID - 7505716 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Intraocular Pressure/*drug effects MH - Low Tension Glaucoma/*drug therapy/*physiopathology MH - Male MH - Middle Aged MH - Risk Factors MH - *Visual Fields EDAT- 2010/08/05 06:00 MHDA- 2010/09/02 06:00 CRDT- 2010/08/05 06:00 PHST- 2010/08/05 06:00 [entrez] PHST- 2010/08/05 06:00 [pubmed] PHST- 2010/09/02 06:00 [medline] PST - ppublish SO - Nippon Ganka Gakkai Zasshi. 2010 Jul;114(7):592-7. PMID- 18092267 OWN - NLM STAT- MEDLINE DCOM- 20080318 LR - 20071219 IS - 0300-9742 (Print) IS - 0300-9742 (Linking) VI - 36 IP - 6 DP - 2007 Nov-Dec TI - Simple counting of nailfold capillary density in suspected systemic sclerosis - 9 years' experience. PG - 452-7 AB - OBJECTIVES: Capillary damage is a characteristic feature of systemic sclerosis (SSc). This work aimed to explore the potential clinical value of simple microscopic counting of capillary density. METHODS: In 325 patients admitted because of a clinical suspicion of SSc and in 80 healthy controls, nailfold capillary microscopy (NCM) was performed using a stereo-zoom microscope in 20 x magnification and with a transparent ruler in one of the eyepieces. Capillaries were counted within 3 mm in the centre of the nailfold in eight fingers. RESULTS: Capillary density (loops/mm) was decreased in patients with diffuse cutaneous SSc [median 4.7 (range 2.2-7.3)], limited cutaneous SSc [4.9 (2.0-7.3)], earlySSc [4.7 (2.8-7.3)], and preSSc [5.9 (4.3-8.2)] compared to healthy controls [7.2 (5.8-9.0)]. Patients with morphea and with primary Raynaud's phenomenon had normal numbers of capillaries [7.0 (6.2-7.2) and 7.0 (5.3-8.7), respectively]. In only 21/325 (6%) patients was it not possible to count the capillaries because of insufficient transparency of the skin. There was no discrepancy in capillary density based on counts of two or eight fingers. When 43 patients were reassessed after 1 to 4 years, there was no difference between the two assessments. CONCLUSION: Determination of capillary density by direct microscopy counts, a simple, inexpensive and rapid method, helps to identify patients with SSc, early in the disease course and in patients with very limited skin involvement. FAU - Wildt, M AU - Wildt M AD - Department of Rheumatology, University Hospital, Lund, Sweden. FAU - Hesselstrand, R AU - Hesselstrand R FAU - Akesson, A AU - Akesson A FAU - Scheja, A AU - Scheja A LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Capillaries/pathology MH - Cell Count MH - Diagnosis, Differential MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Nails/*blood supply MH - Reproducibility of Results MH - Retrospective Studies MH - Scleroderma, Systemic/*pathology EDAT- 2007/12/20 09:00 MHDA- 2008/03/19 09:00 CRDT- 2007/12/20 09:00 PHST- 2007/12/20 09:00 [pubmed] PHST- 2008/03/19 09:00 [medline] PHST- 2007/12/20 09:00 [entrez] AID - 788636521 [pii] AID - 10.1080/03009740701483030 [doi] PST - ppublish SO - Scand J Rheumatol. 2007 Nov-Dec;36(6):452-7. doi: 10.1080/03009740701483030. PMID- 17822528 OWN - NLM STAT- MEDLINE DCOM- 20080408 LR - 20181113 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 9 IP - 5 DP - 2007 TI - Antipolymer antibody in Italian fibromyalgic patients. PG - R86 AB - The objectives of the present study were to evaluate the presence of antipolymer antibody (APA) seropositivity in 285 Italian patients affected by primary fibromyalgia (FM) and to verify whether APA levels correlate with disease severity and with cytokine levels.APA levels were determined on serum samples by an indirect ELISA kit that detects IgG APA. Cytokines (IL-1, IL-6, IL-8, IL-10 and TNFalpha) were measured by ELISA in plasma. The impact of FM on the quality of life was estimated using the Fibromyalgia Impact Questionnaire, while pain severity was evaluated using a visual analogic scale. Patients were also characterized by the presence of tiredness, stiffness, nonrestorative sleep, anxiety, depression, tension headache, irritable bowel syndrome, temporomandibular dysfunction and Raynaud's phenomena. Using a cut-off value of 30 U, APA-positive values were detected in 60 FM patients (21.05%) and in 15 healthy control individuals (15.00%) without significant differences among their levels or the percentage of seropositivity. FM patients with moderate and severe symptoms had slightly higher APA levels with respect to patients with mild symptoms. APA-seropositive patients exhibited significant correlations between APA levels and the Fibromyalgia Impact Questionnaire estimate (P = 0.042), tiredness (P = 0.003) and IL-1 levels (P = 0.0072). In conclusion, APA cannot be considered a marker of disease in Italian FM patients. The presence of APA, however, might permit the identification of a subset of FM patients with more severe symptoms and of patients who may respond differently to different therapeutic strategies. FAU - Bazzichi, Laura AU - Bazzichi L AD - Department of Internal Medicine, Division of Rheumatology, University of Pisa, Pisa, Italy. l.bazzichi@int.med.unipi.it FAU - Giacomelli, Camillo AU - Giacomelli C FAU - De Feo, Francesca AU - De Feo F FAU - Giuliano, Tiziana AU - Giuliano T FAU - Rossi, Alessandra AU - Rossi A FAU - Doveri, Marica AU - Doveri M FAU - Tani, Chiara AU - Tani C FAU - Wilson, Russell B AU - Wilson RB FAU - Bombardieri, Stefano AU - Bombardieri S LA - eng PT - Comparative Study PT - Journal Article PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Antibodies) RN - 0 (Biomarkers) RN - 0 (Cytokines) RN - 0 (Polymers) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies/*blood MH - Biomarkers/blood MH - Cohort Studies MH - Cytokines/blood MH - Female MH - Fibromyalgia/*blood/diagnosis/epidemiology MH - Humans MH - Italy/epidemiology MH - Male MH - Middle Aged MH - *Polymers PMC - PMC2212586 EDAT- 2007/09/08 09:00 MHDA- 2008/04/09 09:00 PMCR- 2007/09/06 CRDT- 2007/09/08 09:00 PHST- 2007/05/21 00:00 [received] PHST- 2007/07/27 00:00 [revised] PHST- 2007/09/06 00:00 [accepted] PHST- 2007/09/08 09:00 [pubmed] PHST- 2008/04/09 09:00 [medline] PHST- 2007/09/08 09:00 [entrez] PHST- 2007/09/06 00:00 [pmc-release] AID - ar2285 [pii] AID - 10.1186/ar2285 [doi] PST - ppublish SO - Arthritis Res Ther. 2007;9(5):R86. doi: 10.1186/ar2285. PMID- 17619801 OWN - NLM STAT- MEDLINE DCOM- 20071113 LR - 20091111 IS - 0007-4861 (Print) IS - 0007-4861 (Linking) VI - 78 IP - 6 DP - 2007 Jun TI - Monomethylarsonous acid induced cytotoxicity and endothelial nitric oxide synthase phosphorylation in endothelial cells. PG - 455-8 AB - Chronic arsenic poisoning is reported to be associated with peripheral and cardiovascular disease, arteriosclerosis, Raynaud's syndrome, hypertension, and Blackfoot disease. Monomethylarsonous acid (MMA(III)) is a reactive metabolite of inorganic arsenic and a potent inhibitor of endothelial nitric oxide synthase (eNOS). Arsenic is also reported to phosphorylate eNOS in cultured keratinocyte and Human T cell leukemia Jurkat cells, respectively. In the present study, we examined the cytotoxicity and eNOS phosphorylation by MMA(III) exposure in cultured bovine aortic endothelial cells (BAEC). Results showed that MMA(III) is more toxic than arsenite in BAEC cells. The IC(50) values for MMA(III) and arsenite were determined to be approximately 1.7 and 24.1 micromol/L, respectively. Exposure of BAEC to MMA(III) (0.75 micromol/L) caused a significant eNOS phosphorylation 15 min after MMA(III) exposure. However, a complex of MMA(III) with dithiothreitol (DTT) that lacks the reactivity with vicinal thiols unaffected eNOS phosphorylation. The present study shows that MMA(III )generated during biomethylation of arsenic is highly toxic in BAEC. Our study also suggests that MMA(III) could induce the eNOS phosphorylation through modification to cellular thiols of the eNOS enzyme. And the initial up-regulation of eNOS phosphorylation by MMA(III )seems to be an adaptive response against disruption of eNOS bioactivity during arsenic exposure. FAU - Li, B AU - Li B AD - Department of Occupational and Environmental Health, School of Public Health, China Medical University, Shenyang 110001, China. FAU - Sun, Y AU - Sun Y FAU - Sun, X AU - Sun X FAU - Wang, Y AU - Wang Y FAU - Li, X AU - Li X FAU - Kumagai, Y AU - Kumagai Y FAU - Sun, G AU - Sun G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20070710 PL - United States TA - Bull Environ Contam Toxicol JT - Bulletin of environmental contamination and toxicology JID - 0046021 RN - 0 (Organometallic Compounds) RN - 0 (monomethylarsonous acid) RN - EC 1.14.13.39 (Nitric Oxide Synthase Type III) SB - IM MH - Animals MH - Cattle MH - Endothelium, Vascular/cytology/*drug effects/enzymology MH - Humans MH - Jurkat Cells MH - Nitric Oxide Synthase Type III/*metabolism MH - Organometallic Compounds/*toxicity MH - Phosphorylation EDAT- 2007/07/11 09:00 MHDA- 2007/11/14 09:00 CRDT- 2007/07/11 09:00 PHST- 2007/02/19 00:00 [received] PHST- 2007/06/01 00:00 [accepted] PHST- 2007/07/11 09:00 [pubmed] PHST- 2007/11/14 09:00 [medline] PHST- 2007/07/11 09:00 [entrez] AID - 10.1007/s00128-007-9178-7 [doi] PST - ppublish SO - Bull Environ Contam Toxicol. 2007 Jun;78(6):455-8. doi: 10.1007/s00128-007-9178-7. Epub 2007 Jul 10. PMID- 39622261 OWN - NLM STAT- MEDLINE DCOM- 20250426 LR - 20260505 IS - 2213-2619 (Electronic) IS - 2213-2600 (Print) IS - 2213-2600 (Linking) VI - 13 IP - 3 DP - 2025 Mar TI - Idiopathic inflammatory myopathies related lung disease in adults. PG - 272-288 LID - S2213-2600(24)00267-4 [pii] LID - 10.1016/S2213-2600(24)00267-4 [doi] AB - Interstitial lung disease (ILD) is common in idiopathic inflammatory myopathies in adults, especially in patients with antisynthetase syndrome and anti-MDA5 antibody-associated dermatomyositis. Pulmonary manifestations can range from subclinical ILD to rapidly progressive respiratory failure. Coexistent myositis, characteristic skin lesions, arthritis, and Raynaud's phenomenon are common. However, 16-65% of patients present with isolated lung disease. Detection of myositis-specific and myositis-associated antibodies can aid in diagnosis and disease characterisation. Chest imaging and pathology most commonly show non-specific interstitial pneumonia and organising pneumonia patterns. Immunosuppression is the mainstay of management with aggressive combination treatment for rapidly progressive disease and incremental escalation as needed for chronic ILD. The addition of antifibrotic agents is an option in progressive fibrotic disease, and lung transplantation can be considered in severe, end-stage disease. Most patients respond to treatment, but short-term mortality remains high for patients with rapidly progressive disease associated with anti-MDA5 antibody ILD. CI - Copyright © 2024 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Sehgal, Sameep AU - Sehgal S AD - Department of Pulmonary and Critical Care Medicine, Integrated Hospital Care Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address: sehgals@ccf.org. FAU - Patel, Aditi AU - Patel A AD - Department of Rheumatologic and Immunologic Disease, Cleveland Clinic, Cleveland, OH, USA. FAU - Chatterjee, Soumya AU - Chatterjee S AD - Department of Rheumatologic and Immunologic Disease, Cleveland Clinic, Cleveland, OH, USA. FAU - Fernandez, Anthony P AU - Fernandez AP AD - Department of Dermatology, Medical Specialty Institute, Cleveland Clinic, Cleveland, OH, USA; Department of Pathology, Cleveland Clinic, Cleveland, OH, USA. FAU - Farver, Carol AU - Farver C AD - Department of Pathology, Cleveland Clinic, Cleveland, OH, USA. FAU - Yadav, Ruchi AU - Yadav R AD - Department of Diagnostic Radiology, Diagnostic Institute, Cleveland Clinic, Cleveland, OH, USA. FAU - Li, Yuebing AU - Li Y AD - Department of Neurology, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA. FAU - Danoff, Sonye K AU - Danoff SK AD - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Saygin, Didem AU - Saygin D AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Huapaya, Julio A AU - Huapaya JA AD - Critical Care Medicine and Pulmonary Branch, National Heart, Lung, and Blood, Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Wilfong, Erin M AU - Wilfong EM AD - Division of Rheumatology and Immunology, Division of Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Highland, Kristin B AU - Highland KB AD - Department of Pulmonary and Critical Care Medicine, Integrated Hospital Care Institute, Cleveland Clinic, Cleveland, OH, USA. LA - eng GR - K08 AR080808/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Review DEP - 20241129 PL - England TA - Lancet Respir Med JT - The Lancet. Respiratory medicine JID - 101605555 RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - EC 3.6.1.- (IFIH1 protein, human) RN - Antisynthetase syndrome SB - IM MH - Humans MH - *Myositis/complications/immunology MH - *Lung Diseases, Interstitial/etiology/therapy/diagnosis MH - Adult MH - Interferon-Induced Helicase, IFIH1/immunology MH - Autoantibodies MH - Immunosuppressive Agents/therapeutic use MH - Dermatomyositis/complications MH - Disease Progression PMC - PMC13138733 MID - NIHMS2165414 COIS- Declaration of interests APF received grants and contracts from Mallinckrodt, Novartis, Corbus, Alexion, Priovant, and Pfizer; consulting fees from Biogen, AbbVie, Novartis, Bristol-Myers Squibb, and UCB; is on the speakers' bureau for AbbVie, Mallinckrodt, Kyowa Kirin, and Novartis; is on the Board of Directors for American Society of Dermatopathology; is the Director at-large for the Rheumatologic Dermatology Society; and is on the OMERACT Shared-Decision Making Committee. YL received consulting fees from Argenx, Immunovant, and UCB; and grant support from Argenx. SKD received grants and contracts from Boehringer Ingelheim and United Therapeutics; is on steering committees for Avalyn and Bristol-Myers Squibb; is an educational speaker (non-branded) for Merck; and received royalties from UpToDate. EMW has grants from the National Institutes of Health (K08AR080808); serves as a consultant for AstraZeneca, Cabaletta Bio, Capstan Therapeutics, and Merck; and receives royalties from UpToDate. KBH received grants or contracts from aTyr Pharmaceuticals, Boehringer Ingelheim, Gossamer Bio, Merck (Acceleron), and United Therapeutics; consulting fees from aTyr Pharmaceuticals, Boehringer Ingelheim, Gossamer Bio, Merck (Acceleron), United Therapeutics, Johnson and Johnson (Actelion, Janssen), and Mistsubishi Pharmaceuticals; is on the speakers' bureau for Bayer Healthcare, Boehringer Ingelheim, Johnson and Johnson (Actelion, Janssen), Merck (Acceleron), and United Therapeutics; is on the steering committee for Boehringer Ingelheim, Gossamer Bio, Johnson and Johnson (Actelion, Janssen), Merck (Acceleron), and United Therapeutics; and is the co-founder of Zafer Therapeutics. RY is an educational speaker for Boehringer Ingelheim. All other authors declare no competing interests. EDAT- 2024/12/03 00:22 MHDA- 2025/03/07 00:21 PMCR- 2026/05/04 CRDT- 2024/12/02 18:53 PHST- 2024/04/30 00:00 [received] PHST- 2024/08/05 00:00 [revised] PHST- 2024/08/13 00:00 [accepted] PHST- 2025/03/07 00:21 [medline] PHST- 2024/12/03 00:22 [pubmed] PHST- 2024/12/02 18:53 [entrez] PHST- 2026/05/04 00:00 [pmc-release] AID - S2213-2600(24)00267-4 [pii] AID - 10.1016/S2213-2600(24)00267-4 [doi] PST - ppublish SO - Lancet Respir Med. 2025 Mar;13(3):272-288. doi: 10.1016/S2213-2600(24)00267-4. Epub 2024 Nov 29. PMID- 33953621 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240809 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 13 DP - 2021 TI - The Updated Role of Ultrasound in Assessing Dermatological Manifestations in Systemic Sclerosis. PG - 79-91 LID - 10.2147/OARRR.S282612 [doi] AB - Systemic sclerosis (SSc), an autoimmune connective tissue disease, characterized by skin fibrosis, increased dermal thickness and microvascular involvement. Fibroblasts and myofibroblasts deposit excessive amounts of collagenous and non-collagenous extracellular matrix components in the skin. This leads to microvascular abnormalities and Raynaud's phenomenon, with painful digital ulcers (DU) at the fingertips adding to patient discomfort. The skin involvement and severity in SSc was evaluated by the Modified Rodnan skin score (mRSS). Although high-frequency ultrasound (HUS) has been widely researched in the study of skin thickness and DU in SSc, its adoption into clinical practice is not yet common. However, novel insights into the still relatively unknown disease pathogenesis in SSc and its evaluation may be provided by HUS, including early (pre-clinical) skin involvement. It may also be useful in both the evaluation and follow-up of DU. Indeed, it is a non-invasive, safe, inexpensive and reproducible method able to assess not only SSc patients' cutaneous structural changes, but also their vascular system changes. Moreover, several recent studies have reported that elastosonography (ES) is of use when investigating skin involvement in systemic sclerosis. This review aims at providing information as to role HUS and ES play in research advancements and the clinical perspectives in the evaluation of skin thickness and DU in SSc patients. CI - © 2021 Ruaro et al. FAU - Ruaro, Barbara AU - Ruaro B AD - Unit of Pulmonology, University Hospital of Trieste, Trieste, Italy. FAU - Santiago, Tania AU - Santiago T AD - Department of Rheumatology, Centro Hospitalare Universitário de Coimbra, Coimbra, Portugal. AD - Medicine Faculty, University of Coimbra, Coimbra, Portugal. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. FAU - Lepri, Gemma AU - Lepri G AUID- ORCID: 0000-0003-4141-6937 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, Florence, Italy. FAU - Poillucci, Gabriele AU - Poillucci G AD - Department of Radiology, Department of Medicine, Surgery and Health Science, University of Trieste, Trieste, Italy. FAU - Baratella, Elisa AU - Baratella E AUID- ORCID: 0000-0002-1061-9507 AD - Department of Radiology, Department of Medicine, Surgery and Health Science, University of Trieste, Trieste, Italy. FAU - Salton, Francesco AU - Salton F AD - Unit of Pulmonology, University Hospital of Trieste, Trieste, Italy. FAU - Confalonieri, Marco AU - Confalonieri M AUID- ORCID: 0000-0002-4791-768X AD - Unit of Pulmonology, University Hospital of Trieste, Trieste, Italy. LA - eng PT - Journal Article PT - Review DEP - 20210428 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC8092351 OTO - NOTNLM OT - digital ulcers OT - elastosonography OT - skin OT - skin thickness OT - systemic sclerosis OT - ultrasound COIS- The authors report no conflicts of interest in this work. No specific funding was received from any bodies in the public, commercial, or not-for-profit sectors, to carry out the work described in this manuscript. The authors declare that the research was carried out without any commercial or financial relationship that could be construed as a potential conflict of interest. EDAT- 2021/05/07 06:00 MHDA- 2021/05/07 06:01 PMCR- 2021/04/28 CRDT- 2021/05/06 07:01 PHST- 2021/02/22 00:00 [received] PHST- 2021/03/19 00:00 [accepted] PHST- 2021/05/06 07:01 [entrez] PHST- 2021/05/07 06:00 [pubmed] PHST- 2021/05/07 06:01 [medline] PHST- 2021/04/28 00:00 [pmc-release] AID - 282612 [pii] AID - 10.2147/OARRR.S282612 [doi] PST - epublish SO - Open Access Rheumatol. 2021 Apr 28;13:79-91. doi: 10.2147/OARRR.S282612. eCollection 2021. PMID- 25622735 OWN - NLM STAT- MEDLINE DCOM- 20150908 LR - 20250103 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 94 IP - 44 DP - 2014 Dec 2 TI - [Evaluations and analyses of quality of life in 90 patients with systemic sclerosis by health assessment questionnaire-disability index]. PG - 3471-4 AB - OBJECTIVE: To evaluate health-related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using health assessment questionnaire-disability index (HAQ-DI), visual analog scale (VAS) and scleroderma assessment questionnaire (SAQ). METHODS: The questionnaires of HAQ-DI, VAS and SAQ were administered to 50 controls and 90 SSc patients from September to December 2013. And SSc patients were also evaluated for subset (limited & diffuse SSc), age, disease duration, autoantibodies and skin & internal organ involvement. RESULTS: The physical summary scores of HAQ-DI in dressing, cleaning and hand functions domains were higher in SSc patients than those in controls (all P < 0.05). As for VAS, the specific clinical manifestations (Pain,0.60 (0.20-1.40); Raynaud's phenomenon, 9.00(4.00-11.00); finger ulcers, 1.00(0.00-5.00); gastrointestinal symptoms, 5.00(1.00-6.50); respiratory symptoms, 3.00(0.00-6.00)) of SSc patients impacted their HRQOL. SAQ showed higher scores in blood vascular, respiratory, digestive and musculoskeletal systems for SSc patients (1.00(0.75-1.50), 0.50(0.33-0.67), 0.40(0.20-0.80), 0.13(0.00-0.38) vs 0.00(0.00-0.00), all P < 0.05). CONCLUSIONS: HRQOL is impaired in patients with SSc, especially in physical domains. And higher scores in SSc patients with specific organ involvement suggest that severe disease has worse HRQOL. FAU - Mao, Xiaofei AU - Mao X AD - Department of Dermatology, Peking Union Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. FAU - Sun, Qiuning AU - Sun Q AD - Department of Dermatology, Peking Union Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. Email: doctorjenny1@126.com. LA - chi PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 SB - IM MH - Disability Evaluation MH - Persons with Disabilities MH - Hand MH - Humans MH - Pain MH - Pain Measurement MH - *Quality of Life MH - *Scleroderma, Systemic MH - Severity of Illness Index MH - Surveys and Questionnaires EDAT- 2015/01/28 06:00 MHDA- 2015/09/09 06:00 CRDT- 2015/01/28 06:00 PHST- 2015/01/28 06:00 [entrez] PHST- 2015/01/28 06:00 [pubmed] PHST- 2015/09/09 06:00 [medline] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2014 Dec 2;94(44):3471-4. PMID- 24762091 OWN - NLM STAT- MEDLINE DCOM- 20150529 LR - 20140908 IS - 1366-5928 (Electronic) IS - 0049-8254 (Linking) VI - 44 IP - 10 DP - 2014 Oct TI - Clinical symptoms in fibromyalgia are associated to catechol-O-methyltransferase (COMT) gene Val158Met polymorphism. PG - 952-6 LID - 10.3109/00498254.2014.913083 [doi] AB - 1. Fibromyalgia syndrome (FMS) is a common chronic widespread pain syndrome mainly affecting women. The aim of this study was to explore the frequency and clinical significance of catechol-O-methyltransferase (COMT) gene Val158Met polymorphism in a large cohort of Turkish patients with FMS. 2. The study included 379 FMS patients and 290 controls. Genomic DNA was isolated and genotyped using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) analyses. 3. The genotype frequencies of Val158Met polymorphism showed a small difference between FMS patients and healthy controls (p = 0.047), however, the Met/Met genotype was significantly higher in FMS patients than healthy controls (p = 0.016). No difference was observed for allele frequencies between two groups. Stratification analysis according to clinical features for this disease reveals that weight, FMS Impact Questionnaire score, algometry and Raynaud's syndrome, were detected to have statistically significant associations with Val158Met polymorphism (p = 0.037, p = 0.042, p = 0.039 and p = 0.033, respectively). Pain sensitivity, measured by algometry, was statistically higher in patients with Met/Met genotype than the patients with Val/Val and Val/Met genotypes (p = 0.017). 4. The results of this study suggested that COMT gene Val158Met polymorphism is positively associated with FMS and play a relevant role in the clinical symptoms of the disease. FAU - Inanir, Ahmet AU - Inanir A AD - Department of Physical Therapy and Rehabilitation, Faculty of Medicine . FAU - Karakus, Nevin AU - Karakus N FAU - Ates, Omer AU - Ates O FAU - Sezer, Saime AU - Sezer S FAU - Bozkurt, Nihan AU - Bozkurt N FAU - Inanir, Sema AU - Inanir S FAU - Yigit, Serbulent AU - Yigit S LA - eng PT - Journal Article DEP - 20140424 PL - England TA - Xenobiotica JT - Xenobiotica; the fate of foreign compounds in biological systems JID - 1306665 RN - EC 2.1.1.6 (COMT protein, human) RN - EC 2.1.1.6 (Catechol O-Methyltransferase) SB - IM MH - Adult MH - Amino Acid Substitution MH - Case-Control Studies MH - Catechol O-Methyltransferase/*genetics MH - Female MH - Fibromyalgia/*genetics MH - Humans MH - Male MH - Middle Aged MH - Polymorphism, Genetic MH - Severity of Illness Index OTO - NOTNLM OT - Catechol-O-methyltransferase OT - Val158Met polymorphism OT - fibromyalgia OT - pain EDAT- 2014/04/26 06:00 MHDA- 2015/05/30 06:00 CRDT- 2014/04/26 06:00 PHST- 2014/04/26 06:00 [entrez] PHST- 2014/04/26 06:00 [pubmed] PHST- 2015/05/30 06:00 [medline] AID - 10.3109/00498254.2014.913083 [doi] PST - ppublish SO - Xenobiotica. 2014 Oct;44(10):952-6. doi: 10.3109/00498254.2014.913083. Epub 2014 Apr 24. PMID- 20711093 OWN - NLM STAT- MEDLINE DCOM- 20101222 LR - 20100816 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 35 IP - 2 DP - 2010 Apr-Jun TI - [Skin manifestation in systemic sclerosis]. PG - 184-90 AB - OBJECTIVE: To assess cutaneous manifestations in a population of patients with systemic sclerosis (SSc). METHOD: The study population was constituted by 50 patients with a diagnosis of SSc, according to the classification criteria for SSc established by the American College of Rheumatology. According to the criteria proposed by LeRoy and cols., the disease was classified as diffuse SSc, or limited SSc, depending on the extent of skin involvement. Through history and physical exam the following variables were assessed: skin sclerosis, Raynaud's phenomenon, digital pitting scars, ischemic ulcers, telangiectasia, leucomelanoderma, microstomy, calcinosis and pruritus. RESULTS: In the study population, 88% were women, with a mean age of 52.3+/-12.4 years. Limited SSc was the most frequent subset, being present in 70% of the evaluated patients. We found Raynaud's phenomenon in 100% of the patients, telangiectasia in 94%, leucomelanoderma in 38%, pruritus in 50%, calcinosis in 40%, microstomy in 62%, digital scars in 66% and ischemic ulcers in 58%. The modified Rodnan skin score values ranged from 3 to 32, with a median of 14, and percentile 25 of 9 and percentile 75 of 17.75. CONCLUSION: Cutaneous manifestations are very common in ES, being responsible for important limitations on daily activities and stigmatization of patients. FAU - Bertazzi, Glauce Rejane Leonardi AU - Bertazzi GR AD - Professor mestre do Serviço de Reumatologia da Faculdade de Medicina de São José do Rio Preto-SP. glbertazzi@netsite.com.br FAU - de Toledo, Roberto Acayaba AU - de Toledo RA FAU - de Godoy, Moacir Fernandes AU - de Godoy MF FAU - Geraldino, Geise Cristina AU - Geraldino GC FAU - Fernandes, Gisele Cristine Dyonisio AU - Fernandes GC FAU - Polizelli, Daniela Vichiato AU - Polizelli DV FAU - Pedroso, Camila Lobo AU - Pedroso CL LA - por PT - English Abstract PT - Journal Article TT - Manifestações cutâneas na esclerose sistêmica. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications MH - Skin Diseases/epidemiology/*etiology MH - Young Adult EDAT- 2010/08/17 06:00 MHDA- 2010/12/24 06:00 CRDT- 2010/08/17 06:00 PHST- 2010/08/17 06:00 [entrez] PHST- 2010/08/17 06:00 [pubmed] PHST- 2010/12/24 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2010 Apr-Jun;35(2):184-90. PMID- 19213862 OWN - NLM STAT- MEDLINE DCOM- 20090505 LR - 20250529 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 18 IP - 3 DP - 2009 Mar TI - A controlled comparison of brachial artery flow mediated dilation (FMD) and digital pulse amplitude tonometry (PAT) in the assessment of endothelial function in systemic lupus erythematosus. PG - 235-42 LID - 10.1177/0961203308096663 [doi] AB - The utility of flow mediated dilation (FMD) a measure of endothelial function is limited by operator dependence. Pulse amplitude tonometry (PAT) is a novel, less operator-dependent technique to assess endothelial function. This study compares PAT to FMD in SLE and controls. Thirty women with SLE and 31 controls were enrolled. Medications, cardiovascular disease and risk factors, SLE activity (SLAM-R) and damage (SLICC-DI) were recorded. FMD and PAT were performed simultaneously. Endothelium-independent function was assessed with nitroglycerin. Average age was 48.3 +/- 10.1 years, SLE duration 16.2 years, SLAM-R 8.3 and SLICC-DI 1.0. Framingham Risk Scores were < or =2% in most subjects. There were no differences between SLE cases and controls in FMD, PAT or response to nitroglycerin. This study found no association between FMD and PAT in SLE or controls. In the 17 SLE cases with a history of Raynaud's, correlation between FMD and PAT was 0.50 (P = 0.04). There was no difference in endothelial function assessed by FMD or PAT in SLE cases versus controls. FMD did not correlate with PAT except in SLE cases with a history of Raynaud's. Correlation between FMD and PAT may be stronger in populations with greater variation in endothelial function and more cardiovascular risk factors. FAU - Aizer, J AU - Aizer J AD - Division of Rheumatology, Hospital for Special Surgery, Weill Cornell Medical College, New York, New York 10021, USA. AizerJ@hss.edu FAU - Karlson, E W AU - Karlson EW FAU - Chibnik, L B AU - Chibnik LB FAU - Costenbader, K H AU - Costenbader KH FAU - Post, D AU - Post D FAU - Liang, M H AU - Liang MH FAU - Gall, V AU - Gall V FAU - Gerhard-Herman, M D AU - Gerhard-Herman MD LA - eng GR - K24 AR052403/AR/NIAMS NIH HHS/United States PT - Journal Article PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adolescent MH - Adult MH - *Brachial Artery/diagnostic imaging/physiology MH - Endothelium, Vascular/*physiology/physiopathology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*physiopathology MH - Manometry/methods MH - Middle Aged MH - Regional Blood Flow/*physiology MH - Ultrasonography MH - Vasodilation/*physiology MH - Young Adult PMC - PMC2754753 MID - NIHMS140809 EDAT- 2009/02/14 09:00 MHDA- 2009/05/06 09:00 PMCR- 2009/09/30 CRDT- 2009/02/14 09:00 PHST- 2009/02/14 09:00 [entrez] PHST- 2009/02/14 09:00 [pubmed] PHST- 2009/05/06 09:00 [medline] PHST- 2009/09/30 00:00 [pmc-release] AID - 18/3/235 [pii] AID - 10.1177/0961203308096663 [doi] PST - ppublish SO - Lupus. 2009 Mar;18(3):235-42. doi: 10.1177/0961203308096663. PMID- 40757122 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250806 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 36 IP - 2 DP - 2025 Jun TI - Clinical Application of Infrared Thermography in Rheumatic Diseases: A Systematic Review. PG - 159-192 LID - 10.31138/mjr.271024.ita [doi] AB - AIM: This systematic review aims to evaluate the clinical applications of infrared thermography (IRT) in rheumatic diseases (RDs), focusing on its potential as a non-invasive, cost-effective, and reliable tool for diagnosis, monitoring, and treatment to improve patient outcomes. METHODS: A systematic literature review was conducted following the PRISMA guidelines. A comprehensive search strategy was implemented using various databases, namely Medline/PubMed, Scopus, Web of Science, Google Scholar, PubMed Central, Cochrane Library, and ScienceDirect. After screening, data extraction and quality assessment were performed to synthesise the findings and evaluate the methodological quality of the included studies. RESULTS: The systematic review included 51 studies comprising 7 randomised controlled trials and 44 observational studies. IRT demonstrated utility in various RDs. In osteoarthritis, it detected elevated temperatures in affected joints, correlating with pain intensity. For rheumatoid arthritis, IRT was effective in diagnosing active synovitis and monitoring disease progression, although its effectiveness was limited in small joint assessments. In Sjögren's syndrome, IRT differentiated dry eye aetiologies, while in fibromyalgia, the studies yielded mixed results. IRT effectively assessed arthritis in juvenile idiopathic arthritis and aided in detecting disease activity, monitoring progression, and evaluating treatment responses in scleroderma and Raynaud's phenomenon. Additionally, IRT showed potential in assessing therapeutic interventions across several conditions. CONCLUSION: IRT showed significant potential as a non-invasive tool for diagnosing, monitoring, and evaluating treatment in RDs. While its effectiveness varied by condition, IRT complemented existing methods. Further research is needed to standardise protocols and confirm its clinical utility. CI - © 2025 The Author(s). FAU - Nallathambi, Naveenkumar AU - Nallathambi N AD - Institute of Internal Medicine, Madras Medical College, Chennai, Tamilnadu, India. FAU - Bisaralli, Rahul AU - Bisaralli R AD - Department of Clinical Immunology and Rheumatology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India. FAU - Naidu, Shriganesh Palanikumar AU - Naidu SP AD - Institute of Internal Medicine, Madras Medical College, Chennai, Tamilnadu, India. FAU - Mallikarjunaswamy, M S AU - Mallikarjunaswamy MS AD - Department of Electronics and Instrumentation Engineering, Sri Jayachamarajendra College of Engineering, JSS Science and Technology University, Mysuru, India. FAU - Praveen, P AU - Praveen P AD - Department of Electronics and Instrumentation Engineering, Sri Jayachamarajendra College of Engineering, JSS Science and Technology University, Mysuru, India. FAU - Mamadapur, Mahabaleshwar AU - Mamadapur M AD - Department of Clinical Immunology and Rheumatology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India. LA - eng PT - Journal Article DEP - 20250130 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC12312464 OTO - NOTNLM OT - diagnosis OT - infrared thermography OT - monitoring OT - rheumatic diseases OT - treatment COIS- The authors declare no conflict of interest. EDAT- 2025/08/04 12:27 MHDA- 2025/08/04 12:28 PMCR- 2025/01/30 CRDT- 2025/08/04 06:23 PHST- 2024/10/27 00:00 [received] PHST- 2025/01/15 00:00 [revised] PHST- 2025/02/14 00:00 [accepted] PHST- 2025/08/04 12:28 [medline] PHST- 2025/08/04 12:27 [pubmed] PHST- 2025/08/04 06:23 [entrez] PHST- 2025/01/30 00:00 [pmc-release] AID - MJR-36-2-159 [pii] AID - 10.31138/mjr.271024.ita [doi] PST - epublish SO - Mediterr J Rheumatol. 2025 Jan 30;36(2):159-192. doi: 10.31138/mjr.271024.ita. eCollection 2025 Jun. PMID- 38903288 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240622 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 5 DP - 2024 May TI - Antibodies in Aseptic Meningitis of Connective Tissue Disorder: A Case Report. PG - e60762 LID - 10.7759/cureus.60762 [doi] LID - e60762 AB - Mixed connective tissue disorder (MCTD) is the first overlap syndrome described with features of overlapping manifestations of at least two other autoimmune rheumatic conditions. It is an autoimmune disease of rarity and is strongly associated with specific antibodies to U1 small nuclear ribonucleoprotein (anti-U1-RNP). This disorder affects almost all organs of the body, and it has varied clinical presentations as it has an autoimmune and inflammatory background, causing heightened immune cell activation. They present more commonly with less fatal symptoms like joint pain, stiffness, and mucocutaneous changes. The majority present initially with Raynaud's phenomenon followed by muscular skeletal involvement and around half of them present with swallowing problems due to esophageal dysmotility. Rarely do they also present with more morbid symptoms of pulmonary hypertension and central nervous system involvement. MCTD on follow-up had a 10 percent association with neurological manifestations as reported by the National Organization for Rare Diseases (NORD), and the most reported diseases were trigeminal neuralgia and aseptic meningitis. Patients presenting with such symptoms and, when treated only with guideline-based antibiotics therapy, would delay the treatment, leading to a poorer prognosis. The following is an interesting case of a young female presenting with a headache, which was masquerading as an underlying undiagnosed connective tissue disorder. Headache is a predominant presentation that has several etiologies in autoimmune disease and meticulous differential diagnosis workup is a must. This case highlights the fact that any persistent atypical, unusual symptom needs to be always considered for further evaluation to arrive at a diagnosis and for a favorable outcome. CI - Copyright © 2024, Ravichandran et al. FAU - Ravichandran, Sreevinishaa AU - Ravichandran S AD - General Medicine, Sri Ramasamy Memorial (SRM) Medical College Hospital and Research Centre, Chennai, IND. FAU - Kumar, J AU - Kumar J AD - General Medicine, Sri Ramasamy Memorial (SRM) Medical College Hospital and Research Centre, Chennai, IND. FAU - Chandrasekaran, Nirmala Devi AU - Chandrasekaran ND AD - General Medicine, Sri Ramasamy Memorial (SRM) Medical College Hospital and Research Centre, Chennai, IND. FAU - Irfan, Shahul AU - Irfan S AD - Internal Medicine, Government Medical College and Hospital Cuddalore, Chidambaram, IND. FAU - A, Sathvika AU - A S AD - Internal Medicine, Sri Ramasamy Memorial (SRM) Medical College Hospital and Research Centre, Chennai, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20240521 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11188694 OTO - NOTNLM OT - anti-u1rnp autoantibody OT - aseptic meningitis OT - cns manifestations OT - mixed connective tissue disorder OT - systemic lupus erythematosis COIS- The authors have declared that no competing interests exist. EDAT- 2024/06/21 06:42 MHDA- 2024/06/21 06:43 PMCR- 2024/05/21 CRDT- 2024/06/21 04:17 PHST- 2024/05/19 00:00 [accepted] PHST- 2024/06/21 06:43 [medline] PHST- 2024/06/21 06:42 [pubmed] PHST- 2024/06/21 04:17 [entrez] PHST- 2024/05/21 00:00 [pmc-release] AID - 10.7759/cureus.60762 [doi] PST - epublish SO - Cureus. 2024 May 21;16(5):e60762. doi: 10.7759/cureus.60762. eCollection 2024 May. PMID- 37123711 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230502 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 3 DP - 2023 Mar TI - Anti-synthetase Syndrome: A Diagnostic Dilemma. PG - e36760 LID - 10.7759/cureus.36760 [doi] LID - e36760 AB - Among the various inflammatory myopathies, the anti-synthetase syndrome (ASS) is a rare entity with autoantibodies directed against aminoacyl-transfer ribonucleic acid synthetase. Its clinical spectrum ranges from myopathy and non-erosive arthritis to dyspnea and cough of pulmonary interstitial disease and from hyperkeratotic skin changes to spasms of blood vessels causing Raynaud's phenomenon. We present a case of a 21-year-old female who had been suffering from fever, night sweats, and weight loss for two years and had remained undiagnosed. She came to our hospital with new-onset muscle weakness, small joint arthralgia, and skin changes. Physical examination showed inflammation involving multiple small joints and characteristic hyperkeratotic skin changes in the distal and lateral phalanges of the hands and feet. Raised creatine phosphokinase levels indicated the possibility of myositis along with positive anti-nuclear antibodies, suggesting an autoimmune rheumatic disorder. Inflammatory myositis was later confirmed on biopsy. Further investigations revealed positive anti-Jo1 antibodies. The diagnosis of ASS was made despite the absence of pulmonary signs and symptoms. The patient was promptly started on prednisone and azathioprine. She showed some improvement in muscle weakness at the end of two months and continues to improve albeit slowly. Due to the lack of awareness about the rare disease among the non-rheumatologists, there was a significant delay in the patient's diagnosis. It is, therefore, important for primary care physicians to obtain a comprehensive history and perform a detailed clinical examination to make timely referrals to specialized healthcare professionals. CI - Copyright © 2023, Aslam et al. FAU - Aslam, Muniba AU - Aslam M AD - Internal Medicine, Jinnah Postgraduate Medical Centre, Karachi, PAK. FAU - Khan, Sulhera AU - Khan S AD - Internal Medicine, Jinnah Postgraduate Medical Centre, Karachi, PAK. FAU - Batool, Wajeeha AU - Batool W AD - Internal Medicine, Jinnah Postgraduate Medical Centre, Karachi, PAK. FAU - Ali, Zeeshan AU - Ali Z AD - Gastroenterology, Jinnah Postgraduate Medical Centre, Jinnah Sindh Medical University, Karachi, PAK. FAU - Hanif, Iqra M AU - Hanif IM AD - Dermatology, Jinnah Postgraduate Medical Centre, Karachi, PAK. LA - eng PT - Case Reports PT - Journal Article DEP - 20230327 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10132789 OTO - NOTNLM OT - anti-jo1 antibodies OT - anti-synthetase syndrome OT - inflammatory myopathy OT - non-erosive arthritis OT - polymyositis COIS- The authors have declared that no competing interests exist. EDAT- 2023/05/01 06:41 MHDA- 2023/05/01 06:42 PMCR- 2023/03/27 CRDT- 2023/05/01 03:34 PHST- 2023/03/27 00:00 [accepted] PHST- 2023/05/01 06:42 [medline] PHST- 2023/05/01 06:41 [pubmed] PHST- 2023/05/01 03:34 [entrez] PHST- 2023/03/27 00:00 [pmc-release] AID - 10.7759/cureus.36760 [doi] PST - epublish SO - Cureus. 2023 Mar 27;15(3):e36760. doi: 10.7759/cureus.36760. eCollection 2023 Mar. PMID- 36979470 OWN - NLM STAT- MEDLINE DCOM- 20230330 LR - 20230331 IS - 2218-273X (Electronic) IS - 2218-273X (Linking) VI - 13 IP - 3 DP - 2023 Mar 15 TI - Identification of Tregs-Related Genes with Molecular Patterns in Patients with Systemic Sclerosis Related to ILD. LID - 10.3390/biom13030535 [doi] LID - 535 AB - BACKGROUND: Systemic Sclerosis (SSc) is an autoimmune disease that is characterized by vasculopathy, digital ulcers, Raynaud's phenomenon, renal failure, pulmonary arterial hypertension, and fibrosis. Regulatory T (Treg) cell subsets have recently been found to play crucial roles in SSc with interstitial lung disease (ILD) pathogenesis. This study investigates the molecular mechanism of Treg-related genes in SSc patients through bioinformatic analyses. METHODS: The GSE181228 dataset of SSc was used in this study. CIBERSORT was used for assessing the category and proportions of immune cells in SSc. Random forest and least absolute shrinkage and selection operator (LASSO) regression analysis were used to select the hub Treg-related genes. RESULTS: Through bioinformatic analyses, LIPN and CLEC4D were selected as hub Treg-regulated genes. The diagnostic power of the two genes separately for SSc was 0.824 and 0.826. LIPN was associated with the pathway of aminoacyl-tRNA biosynthesis, Primary immunodeficiency, DNA replication, etc. The expression of CLEC4D was associated with the pathway of Neutrophil extracellular trap formation, PPAR signaling pathway, Staphylococcus aureus infection, Systemic lupus erythematosus, TNF signaling pathway, and Toll-like receptor signaling pathway. CONCLUSION: Through bioinformatic analyses, we identified two Treg-related hub genes (LIPN, CLEC4D) that are mainly involved in the immune response and metabolism of Tregs in SSc with ILD. Moreover, our findings may provide the potential for studying the molecular mechanism of SSc with ILD. FAU - Luo, Jiao AU - Luo J AD - Department of Rheumatology and Clinical Immunology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang 330000, China. FAU - Li, Dongdong AU - Li D AD - Medical College of Nanchang University, Nanchang 330000, China. FAU - Jiang, Lili AU - Jiang L AD - Department of Rheumatology and Clinical Immunology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang 330000, China. FAU - Shi, Chunhua AU - Shi C AD - Department of Rheumatology and Clinical Immunology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang 330000, China. FAU - Duan, Lihua AU - Duan L AD - Department of Rheumatology and Clinical Immunology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang 330000, China. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20230315 PL - Switzerland TA - Biomolecules JT - Biomolecules JID - 101596414 RN - digital ulcers SB - IM MH - T-Lymphocytes, Regulatory MH - Skin Ulcer MH - Humans MH - *Scleroderma, Systemic/genetics/complications MH - Fibrosis MH - *Lung Diseases, Interstitial PMC - PMC10046355 OTO - NOTNLM OT - SSc OT - Treg cells OT - genes COIS- The authors declare no conflict of interest regarding the publication of this paper. EDAT- 2023/03/30 06:00 MHDA- 2023/03/30 06:11 PMCR- 2023/03/15 CRDT- 2023/03/29 01:19 PHST- 2022/11/06 00:00 [received] PHST- 2023/03/10 00:00 [revised] PHST- 2023/03/11 00:00 [accepted] PHST- 2023/03/30 06:11 [medline] PHST- 2023/03/29 01:19 [entrez] PHST- 2023/03/30 06:00 [pubmed] PHST- 2023/03/15 00:00 [pmc-release] AID - biom13030535 [pii] AID - biomolecules-13-00535 [pii] AID - 10.3390/biom13030535 [doi] PST - epublish SO - Biomolecules. 2023 Mar 15;13(3):535. doi: 10.3390/biom13030535. PMID- 36482516 OWN - NLM STAT- MEDLINE DCOM- 20221215 LR - 20221223 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 101 IP - 48 DP - 2022 Dec 2 TI - Reactive perforating collagenosis and systemic lupus erythematosus: A rare case report. PG - e32138 LID - 10.1097/MD.0000000000032138 [doi] LID - e32138 AB - BACKGROUND: Transepidermal clearance of altered collagen and excessive excretion of keratin are characteristics of a rare cutaneous disorder known as reactive perforating collagenosis (RPC). There are different forms of RPC; however, the acquired form is the most prevalent and inherited. Reactive perforating collagenosis is rarely described in autoimmune rheumatic diseases; instead, it is typically linked to systemic conditions such as renal failure or hepatic disease. METHODS: A 31-year-old Saudi female patient who was initially diagnosed with undifferentiated connective tissue disease. She developed RPC with a severe diffuse itchy skin rash with numerous papules and nodules with central hyperkeratotic plugs over the lower limb, upper limb, and face. RESULTS: The patient tested positive for antinuclear antibody; however, a year later, patient developed Raynaud's phenomenon, oral and nasal ulcers, malar rash, fatigue, and lupus rash around her eyes, and systemic lupus erythematosus was diagnosed clinically. The patient was treated for reactive perforating collagenosis with systemic antihistamines (diphenhydramine 50 mg orally twice daily), topical steroid cream (betamethasone dipropionate cream), and oral isotretinoin (20 mg daily). The patient was advised to undergo phototherapy. A year later, she presented with symptoms of systemic lupus erythematosus and started taking oral hydroxychloroquine 200 mg twice daily for systemic lupus erythematosus. The patient is listed on follow-up. CONCLUSION: Variable skin rash can mimic systemic lupus erythematosus and vasculitis. Therefore, reactive perforating collagenosis is a skin condition that requires high clinical suspension for diagnosis, and it might be challenging to determine whether it is an association or a complication. Furthermore, the timing of the skin biopsy may be crucial for the diagnosis of reactive perforating collagenosis. CI - Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Alenzi, Fahidah AU - Alenzi F AUID- ORCID: 0000-0001-8495-9064 AD - Clinical Sciences Department, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - malar rash SB - IM MH - Female MH - Humans MH - Adult MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - *Exanthema PMC - PMC9726282 COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2022/12/10 06:00 MHDA- 2022/12/15 06:00 PMCR- 2022/12/02 CRDT- 2022/12/09 01:15 PHST- 2022/12/09 01:15 [entrez] PHST- 2022/12/10 06:00 [pubmed] PHST- 2022/12/15 06:00 [medline] PHST- 2022/12/02 00:00 [pmc-release] AID - 00005792-202212020-00001 [pii] AID - 10.1097/MD.0000000000032138 [doi] PST - ppublish SO - Medicine (Baltimore). 2022 Dec 2;101(48):e32138. doi: 10.1097/MD.0000000000032138. PMID- 21640054 OWN - NLM STAT- MEDLINE DCOM- 20110824 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 29 IP - 3 DP - 2011 May-Jun TI - Clinical presentation and salivary gland histopathology of paediatric primary Sjögren's syndrome. PG - 589-93 AB - OBJECTIVES: Explore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjögren's syndrome (PPSjS). METHODS: A case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded. RESULTS: Age at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 3/8 fulfilled AECG-2002 diagnostic criteria, throughout the disease course. CONCLUSIONS: PPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group. FAU - Saad Magalhães, C AU - Saad Magalhães C AD - Department of Paediatric, Botucatu Medical School, São Paulo State University (UNESP), Brazil. claudi@fmb.unesp.br FAU - de Souza Medeiros, P B AU - de Souza Medeiros PB FAU - Oliveira-Sato, J AU - Oliveira-Sato J FAU - Custódio-Domingues, M A AU - Custódio-Domingues MA LA - eng PT - Case Reports PT - Journal Article DEP - 20110630 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Biomarkers) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Adolescent MH - Algorithms MH - Biomarkers/blood MH - Blood Sedimentation MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Male MH - Parotid Gland/pathology MH - Retrospective Studies MH - Rheumatoid Factor/blood MH - Salivary Glands/*pathology MH - Sialadenitis/pathology MH - Sjogren's Syndrome/blood/*diagnosis/*pathology EDAT- 2011/06/07 06:00 MHDA- 2011/08/25 06:00 CRDT- 2011/06/07 06:00 PHST- 2010/12/03 00:00 [received] PHST- 2011/01/10 00:00 [accepted] PHST- 2011/06/07 06:00 [entrez] PHST- 2011/06/07 06:00 [pubmed] PHST- 2011/08/25 06:00 [medline] AID - 4556 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2011 May-Jun;29(3):589-93. Epub 2011 Jun 30. PMID- 18438906 OWN - NLM STAT- MEDLINE DCOM- 20080617 LR - 20080527 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 59 IP - 5 DP - 2008 May 15 TI - Assessment of unmet needs and the lack of generalizability in the design of randomized controlled trials for scleroderma treatment. PG - 706-13 LID - 10.1002/art.23567 [doi] AB - OBJECTIVE: To determine the generalizability of randomized controlled trials (RCTs) in the treatment of systemic sclerosis (SSc) using the Canadian Scleroderma Research Group (CSRG) database. METHODS: We identified articles related to SSc published from 1958 to 2006. Key points on trial design were recorded. The inclusion/exclusion criteria were used in conjunction with the CSRG database to determine the proportion of patients with SSc who would theoretically be eligible for these trials. Articles were classified into subcategories according to the target system. The CSRG database contains 438 patients with SSc from 14 Canadian centers. Results were in median (%) and mean (%) with 95% confidence intervals (95% CIs). RESULTS: In total, 210 articles were evaluated and 73 were selected for inclusion in this study. The mean percentage of eligible patients with SSc associated with other conditions was 35% (95% CI 17-53) for Raynaud's phenomenon, 24% (95% CI 1-47) for digital ulcers, 48% (95% CI 27-68) for gastrointestinal (GI) involvement, 32% (95% CI 20-43) for overall disease modification, 6% (95% CI 4-8) for pulmonary arterial hypertension, 2% (95% CI 0-4) for interstitial lung disease, and 38% (95% CI 12-64) for other categories. CONCLUSION: Except for GI trials, <38% of the identified patients with SSc would have been suitable to enter the RCTs. Although some patients would be ineligible because they lack certain organ involvement, RCTs designed to include appropriate patients with SSc are needed; there are few proven treatments and trials typically do not include the majority of those who could potentially benefit from the intervention. FAU - Villela, Renata AU - Villela R AD - University of Western Ontario, London, ON, Canada. FAU - Yuen, Sai Yan AU - Yuen SY FAU - Pope, Janet E AU - Pope JE FAU - Baron, Murray AU - Baron M CN - Canadian Scleroderma Research group LA - eng PT - Journal Article PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Data Interpretation, Statistical MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Randomized Controlled Trials as Topic MH - Research Design/*standards MH - Scleroderma, Systemic/*drug therapy MH - Treatment Outcome EDAT- 2008/04/29 09:00 MHDA- 2008/06/18 09:00 CRDT- 2008/04/29 09:00 PHST- 2008/04/29 09:00 [pubmed] PHST- 2008/06/18 09:00 [medline] PHST- 2008/04/29 09:00 [entrez] AID - 10.1002/art.23567 [doi] PST - ppublish SO - Arthritis Rheum. 2008 May 15;59(5):706-13. doi: 10.1002/art.23567. PMID- 17329943 OWN - NLM STAT- MEDLINE DCOM- 20070412 LR - 20180614 IS - 1648-9144 (Electronic) IS - 1010-660X (Linking) VI - 43 IP - 2 DP - 2007 TI - [The influence of occupational environment and professional factors on the risk of cardiovascular disease]. PG - 96-102 AB - The article reviews the recent scientific literature and the authors' studies on this topic. Occupational conditions and psychological factors have been shown to play an important role in the etiopathogenesis of cardiovascular diseases. Their effect is often indirect, through damage to the central nervous, respiratory, and neuroendocrine systems. Hot climate in the workplace and intense infrared radiation cause the water and electrolyte imbalance and chronic hyperthermia and manifests as neurovegetative dystonia. The long-term effects of low temperatures condition ischemic lesions in circulatory system, trophic organ destruction. The influence of ultrahigh-frequency electromagnetic radiation on the cardiovascular system is directly related to the central nervous system and neurohumoral lesions. "Microwave disease" often manifests as polymorphic dystonia. Exposure to occupational vibration causes "white finger" syndrome or Raynaud's phenomenon together with cerebral vascular lesions. Recent studies have confirmed that noise as a chronic stressor causes the imbalance in the central and vegetative nervous systems and changes in homeostasis. Noise increases catecholamine and cholesterol concentration in blood, has an effect on plasma lipoprotein levels, increases heart rate, arterial blood pressure, and risk of myocardial infarction. Psychophysiological changes caused by long-term stress influence constant pathological changes in the central nervous system, endocrine and cardiovascular systems. The long-term effect of psychogenic stressors is very important in the etiopathogenesis of psychosomatic diseases. FAU - Obelenis, Vytautas AU - Obelenis V AD - Department of Environmental and Occupational Medicine, Kaunas University of Medicine, Kaunas, Lithuania. vytautas.obelenis@kmu.lt FAU - Malinauskiene, Vilija AU - Malinauskiene V LA - lit PT - English Abstract PT - Journal Article PT - Review TT - Darbo salygu ir profesiniu veiksniu itaka sirdies ir kraujagysliu ligu rizikai. PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 RN - 0 (Aerosols) RN - 0 (Dust) SB - IM MH - Aerosols/adverse effects MH - Aged MH - Cardiovascular Diseases/*epidemiology/etiology/physiopathology/psychology MH - Dust MH - Electrocardiography MH - Electromagnetic Fields/adverse effects MH - Environment MH - Female MH - Hearing Loss, Noise-Induced/etiology MH - Humans MH - Male MH - Microclimate MH - Microwaves/adverse effects MH - Middle Aged MH - Noise, Occupational/adverse effects MH - Occupational Exposure/*adverse effects MH - Risk Factors MH - Stress, Psychological/complications MH - Vibration/adverse effects RF - 34 EDAT- 2007/03/03 09:00 MHDA- 2007/04/14 09:00 CRDT- 2007/03/03 09:00 PHST- 2007/03/03 09:00 [pubmed] PHST- 2007/04/14 09:00 [medline] PHST- 2007/03/03 09:00 [entrez] AID - 0702-02 [pii] PST - ppublish SO - Medicina (Kaunas). 2007;43(2):96-102. PMID- 21437686 OWN - NLM STAT- MEDLINE DCOM- 20121207 LR - 20220801 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 6 DP - 2012 Jun TI - Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus. PG - 1727-31 LID - 10.1007/s00296-011-1880-4 [doi] AB - Pulmonary arterial hypertension (PAH) is a devastating complication of systemic lupus erythematosus (SLE). We aim to estimate the putative predictors contributing to early identification of PAH, thus improve appropriate medical intervention and a better prognosis. A retrospective case-control study was conducted. Forty-one SLE patients with PAH and 106 SLE patients without PAH were enrolled. Demographic variables, clinical features, and laboratory data were compared between the two groups. Univariate and multivariate logistic regression models were used to examine the predictors contributing to PAH in SLE. Serositis, Raynaud's phenomenon, high disease activity, anticardiolipin antibodies, and anti-U1RNP were significantly associated with SLE-PAH. Univariate and multivariate analysis showed that Raynaud's phenomenon, anticardiolipin antibodies, and anti-U1RNP were independent predictors of PAH in SLE. This study highlighted the clinical pattern of SLE-PAH patients, and underlined the leading predictors of PAH development among patients with SLE. Routine echocardiography is recommended in SLE patients with the independent predictors mentioned above. FAU - Lian, Fan AU - Lian F AD - Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Sun Yat-Sen University, No. 58, Zhongshan 2nd Road, 510080, Guangzhou, China. FAU - Chen, Dongying AU - Chen D FAU - Wang, Yu AU - Wang Y FAU - Ye, Yujin AU - Ye Y FAU - Wang, Xiaodong AU - Wang X FAU - Zhan, Zhongping AU - Zhan Z FAU - Xu, Hanshi AU - Xu H FAU - Liang, Liuqin AU - Liang L FAU - Yang, Xiuyan AU - Yang X LA - eng PT - Journal Article DEP - 20110325 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Biomarkers/blood MH - Cardiac Catheterization MH - China MH - Early Diagnosis MH - Echocardiography, Doppler MH - Familial Primary Pulmonary Hypertension MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/*etiology MH - Logistic Models MH - Lupus Erythematosus, Systemic/*complications/diagnosis MH - Male MH - Multivariate Analysis MH - Odds Ratio MH - Predictive Value of Tests MH - Retrospective Studies MH - Risk Assessment MH - Risk Factors MH - Severity of Illness Index MH - Tomography, X-Ray Computed MH - Young Adult EDAT- 2011/03/26 06:00 MHDA- 2012/12/12 06:00 CRDT- 2011/03/26 06:00 PHST- 2010/12/18 00:00 [received] PHST- 2011/02/18 00:00 [accepted] PHST- 2011/03/26 06:00 [entrez] PHST- 2011/03/26 06:00 [pubmed] PHST- 2012/12/12 06:00 [medline] AID - 10.1007/s00296-011-1880-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Jun;32(6):1727-31. doi: 10.1007/s00296-011-1880-4. Epub 2011 Mar 25. PMID- 41007697 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250927 LR - 20251001 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 13 IP - 9 DP - 2025 Sep 1 TI - Anti-Nuclear Antibody (ANA) Positivity and Nuclear Antigen Reactivity in Patients with Joint Hypermobility Syndrome/Hypermobile Ehlers Danlos Syndrome (JHS/hEDS). LID - 10.3390/biomedicines13092134 [doi] LID - 2134 AB - Background/Objectives: To compare clinical features of patients with joint hypermobility syndrome/hypermobile Ehlers Danlos Syndrome (JHS/hEDS) who tested positive or negative for anti-nuclear antibodies (ANA), and to determine antibody titers, staining patterns, and reactivity to common nuclear autoantigens. Methods: ANA results were determined by Hep2 immunofluorescence assay. Reactivity to the most common nuclear autoantigens was measured by the Multiplex assay. Clinical manifestations were compared between three subgroups: total ANA+, ANA+ who did not have evidence of systemic autoimmune inflammatory disease (SAID), and ANA-. Results: Of 289 patients, 210 patients had a Beighton score > 5 and were tested for ANA antibodies. One hundred and thirty-one patients had a positive ANA test. Twenty patients in this subgroup were classified as SAID+ while the remaining 111 patients did not meet criteria for any systemic disease. Speckled staining was the most observed pattern in both ANA+SAID+ (75.00%) and ANA+SAID- (72.97%) subgroups. In the latter subgroup, the target of nuclear autoreactivity remained elusive in 80% of patients. The most common clinical manifestations were diffuse arthralgias, myofascial pain, sicca symptoms, Raynaud's phenomenon, gastrointestinal manifestations, and chronic fatigue. Joint dislocations were observed more commonly in the ANA- subgroup compared to ANA+SAID- patients (30.38% vs. 12.61%, adjusted p < 0.05). Conclusions: Similar clinical characteristics were observed in ANA+ and ANA- subgroups of JHS/hEDS, except for joint dislocations which were more common in the ANA- subgroup. The target of ANA reactivity was unknown in 80% of ANA+JHS/hEDS patients and needs to be determined in future studies. FAU - Moy, Lindsay AU - Moy L AUID- ORCID: 0000-0001-7321-4550 AD - Division of Immunology, Department of Internal Medicine, The University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242, USA. FAU - Lenert, Aleksander AU - Lenert A AD - Division of Immunology, Department of Internal Medicine, The University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242, USA. FAU - Lenert, Petar AU - Lenert P AD - Division of Immunology, Department of Internal Medicine, The University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242, USA. LA - eng GR - K23 AR082966/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20250901 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC12467105 OTO - NOTNLM OT - anti-nuclear antibody OT - hypermobile Ehlers Danlos Syndrome OT - joint hypermobility syndrome COIS- The authors declare no conflicts of interest. EDAT- 2025/09/27 06:34 MHDA- 2025/09/27 06:35 PMCR- 2025/09/01 CRDT- 2025/09/27 01:05 PHST- 2025/07/26 00:00 [received] PHST- 2025/08/26 00:00 [revised] PHST- 2025/08/28 00:00 [accepted] PHST- 2025/09/27 06:35 [medline] PHST- 2025/09/27 06:34 [pubmed] PHST- 2025/09/27 01:05 [entrez] PHST- 2025/09/01 00:00 [pmc-release] AID - biomedicines13092134 [pii] AID - biomedicines-13-02134 [pii] AID - 10.3390/biomedicines13092134 [doi] PST - epublish SO - Biomedicines. 2025 Sep 1;13(9):2134. doi: 10.3390/biomedicines13092134. PMID- 40382636 OWN - NLM STAT- MEDLINE DCOM- 20250517 LR - 20250522 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 27 IP - 1 DP - 2025 May 17 TI - Endotheliopathy in systemic sclerosis: from endothelium-dependent vasodilation to the dysfunction of the vascular reserve, is the paradise lost? PG - 107 LID - 10.1186/s13075-025-03568-x [doi] LID - 107 AB - Microvascular dysfunction is considered one of the main pathogenetic pathways in systemic sclerosis (SSc), and endothelial cells plays a pivotal role even in the early phases of the disease. Endothelial dysfunction results in an early incapacity to adapt the vascular tone and the blood flow under stress conditions, thus losing the important adaptation mechanism that is the vascular reserve.The loss of vascular tone control in systemic sclerosis is clinically evident as Raynaud's phenomenon, one of the earliest signs of the disease. An impairment of the vascular reserve has been described in the literature for the main SSc target organs. An alteration of the coronary reserve was shown in SSc asymptomatic patients undergoing a provocative cardiac stress tests. For what concerns the pulmonary circulation, in presence of normal resting pulmonary pressure values in specific subsets of SSc patients subjected to a cycle ergometer test, an abnormal elevation of pulmonary pressure has been showed. Regarding renal arterial circulation, in SSc patients with normal baseline renal function, an absence of improved glomerular filtration after the infusion of a protein load has been demonstrated. Finally, vascular reserve can be altered even in the gastrointestinal circulation as assessed by the study of the splanchnic circulation after a balanced meal.An early detection of an alteration of the physiologic protective mechanism of the vascular reserve could open a "window of opportunity" in which SSc vasculopathy can be potentially reversible, and more responsive to targeted therapeutic strategies. CI - © 2025. The Author(s). FAU - Bandini, Giulia AU - Bandini G AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine, University of Florence, Careggi Hospital, Florence, Italy. giulia.bandini@unifi.it. AD - Department of Experimental and Clinical Medicine, Azienda Ospedaliero Universitaria Careggi - Medicina Interna 4, Largo Brambilla 3, Firenze, Italy. giulia.bandini@unifi.it. FAU - Bellando Randone, Silvia AU - Bellando Randone S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology Scleroderma UNit, University of Florence, Careggi Hospital, Florence, Italy. FAU - Manetti, Mirko AU - Manetti M AD - Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Inflammation, Fibrosis and ageing initiative (INFLAGE), IRCCS San Raffaele Scientific Institute, and Vita-Salute San Raffaele University, Milan, Italy. FAU - Matucci Cerinic, Marco AU - Matucci Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Inflammation, Fibrosis and ageing initiative (INFLAGE), IRCCS San Raffaele Scientific Institute, and Vita-Salute San Raffaele University, Milan, Italy. FAU - Moggi Pignone, Alberto AU - Moggi Pignone A AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine, University of Florence, Careggi Hospital, Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20250517 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Humans MH - *Endothelium, Vascular/physiopathology/pathology MH - *Scleroderma, Systemic/physiopathology/pathology MH - *Vasodilation/physiology PMC - PMC12085023 OTO - NOTNLM OT - Endothelial dysfunction OT - Endotheliopathy OT - Systemic sclerosis OT - Vascular reserve COIS- Declarations. Ethics approval and consent to participate: Not applicable. Consent to Publish: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2025/05/18 17:01 MHDA- 2025/05/18 17:02 PMCR- 2025/05/17 CRDT- 2025/05/17 23:31 PHST- 2025/03/17 00:00 [received] PHST- 2025/05/04 00:00 [accepted] PHST- 2025/05/18 17:02 [medline] PHST- 2025/05/18 17:01 [pubmed] PHST- 2025/05/17 23:31 [entrez] PHST- 2025/05/17 00:00 [pmc-release] AID - 10.1186/s13075-025-03568-x [pii] AID - 3568 [pii] AID - 10.1186/s13075-025-03568-x [doi] PST - epublish SO - Arthritis Res Ther. 2025 May 17;27(1):107. doi: 10.1186/s13075-025-03568-x. PMID- 39466382 OWN - NLM STAT- MEDLINE DCOM- 20241204 LR - 20250507 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 44 IP - 12 DP - 2024 Dec TI - Patients with systemic sclerosis frequently use phytopharmaceuticals: a cross-sectional survey. PG - 3079-3085 LID - 10.1007/s00296-024-05745-8 [doi] AB - The use of complementary and alternative medicine (CAM) has increased. Phytotherapy, also known as herbal medicine, is one of the CAM therapies that involves using medicinal plants and plant-derived substances. This study aims to evaluate the use of phytopharmaceuticals and the factors associated with their use in patients with Systemic sclerosis (SSc). Our study is designed as a survey and consists of two parts with 32 questions. The first part gathers demographic data with 14 questions, and the second part assesses participants' knowledge about phytotherapy with 18 questions. The questionnaire was administered face-to-face to patients with SSc who consented to participate. One hundred participants completed the survey. Fifty-two (52%) participants had diffuse sclerosis. Raynaud's phenomenon affected 97 (97%) participants; 53 (53%) participants suffered from lung involvement, and 64 (64%) had musculoskeletal system involvement. A total of 55 (55%) participants had used phytopharmaceuticals after SSc diagnosis. Phytotherapy users and non-users had similar demographic and clinical characteristics in terms of age, gender, disease duration, type of SSc, organ involvement, and education level. Participants mostly used the following phytopharmaceuticals: 23 participants used Camellia sinensis (green tea), 16 used Hypericum perforatum, and 12 used Curcuma longa (curcumin). The most improved symptoms with phytopharmaceuticals were musculoskeletal system findings and skin thickness, as stated by participants. A significant portion of SSc patients used phytopharmaceuticals in their disease duration. Although patients stated that they benefited from phytopharmaceuticals, studies on the effectiveness of these treatments are insufficient. CI - © 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Yildirim, Tuba Demirci AU - Yildirim TD AUID- ORCID: 0000-0003-3186-0591 AD - Faculty of Medicine, Division of Rheumatology, Dokuz Eylül University, İzmir, Turkey. tubademirci87@gmail.com. FAU - Basibuyuk, Fatma AU - Basibuyuk F AUID- ORCID: 0000-0002-9564-1139 AD - Faculty of Medicine, Division of Rheumatology, Dokuz Eylül University, İzmir, Turkey. FAU - Birlik, A Merih AU - Birlik AM AUID- ORCID: 0000-0001-5118-9307 AD - Faculty of Medicine, Division of Rheumatology, Dokuz Eylül University, İzmir, Turkey. LA - eng PT - Journal Article DEP - 20241028 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Humans MH - Female MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - *Phytotherapy MH - Adult MH - *Scleroderma, Systemic/drug therapy MH - Surveys and Questionnaires MH - Health Knowledge, Attitudes, Practice MH - Aged OTO - NOTNLM OT - Patient opinions OT - Phytopharmaceuticals OT - Phytotherapy OT - Surveys and questionnaires OT - Systemic sclerosis EDAT- 2024/10/28 18:23 MHDA- 2024/12/04 12:29 CRDT- 2024/10/28 12:13 PHST- 2024/09/28 00:00 [received] PHST- 2024/10/19 00:00 [accepted] PHST- 2024/12/04 12:29 [medline] PHST- 2024/10/28 18:23 [pubmed] PHST- 2024/10/28 12:13 [entrez] AID - 10.1007/s00296-024-05745-8 [pii] AID - 10.1007/s00296-024-05745-8 [doi] PST - ppublish SO - Rheumatol Int. 2024 Dec;44(12):3079-3085. doi: 10.1007/s00296-024-05745-8. Epub 2024 Oct 28. PMID- 32953585 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2222-3959 (Print) IS - 2227-4898 (Electronic) IS - 2222-3959 (Linking) VI - 13 IP - 9 DP - 2020 TI - Anterior segment parameters associated with extramuscular manifestations in polymyositis and dermatomyositis. PG - 1443-1450 LID - 10.18240/ijo.2020.09.17 [doi] AB - AIM: To evaluate detailed anterior segment parameters of patients with idiopathic inflammatory myopathies (IIM), including polymyositis (PM), and dermatomyositis (DM), and to clarify the associations between these data and clinical variables of IIM. METHODS: Totally 57 PM, 41 DM patients and 62 controls were enrolled in this cross-sectional, observational, case-control study. All study participants underwent Pentacam evaluation. Laboratory investigations consisted of different antibody assays, while extramuscular clinical assessments included Raynaud's phenomenon, dysphagia, interstitial lung disease, arthritis/arthralgia, and weight loss. Objective signs and subjective symptoms of dry eye disease (DED) were also evaluated. RESULTS: All pachymetric parameters [center, apex, thinnest and maximal keratometry (K(max))] and corneal volume (CV) of both sides of PM patients proved to be significantly lower. Some pachymetric data were also noticed as significantly decreased compared to those of controls. Several significant differences were traced between anterior segment values and extramuscular manifestations of myositis, largely in case of arthritis/arthralgia and weight loss, whereas associations between anterior segment parameters and antibodies were weak. Objective clinical tests of DED were also significantly decreased in IIM patients. CONCLUSION: The results suggest that all IIM patients have thinner corneas compared with those of controls, and decreased corneal parameters are significantly associated with the occurrence of some extramuscular manifestations. In addition, IIM patients tend to develop objective signs of DED. CI - International Journal of Ophthalmology Press. FAU - Griger, Zoltan AU - Griger Z AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Danko, Katalin AU - Danko K AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Nemeth, Gabor AU - Nemeth G AD - Department of Ophthalmology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. AD - Department of Ophthalmology, Borsod-Abauj-Zemplen County Hospital and University Teaching Hospital, Miskolc 3526, Hungary. FAU - Hassan, Ziad AU - Hassan Z AD - Orbi-Dent Refractive Surgery and Medical Center, Debrecen 4032, Hungary. FAU - Aszalos, Zsuzsa AU - Aszalos Z AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Szabo, Katalin AU - Szabo K AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Bodoki, Levente AU - Bodoki L AD - Department of Rheumatology, Institute of Medicine, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Gesztelyi, Rudolf AU - Gesztelyi R AD - Department of Pharmacology and Pharmacotherapy, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. FAU - Zsuga, Judit AU - Zsuga J AD - Department of Health Systems Management and Quality Management in Health Care, Faculty of Public Health, University of Debrecen, Debrecen 4032, Hungary. FAU - Szodoray, Peter AU - Szodoray P AD - Department of Immunology, Rikshospitalet, Oslo University Hospital, Oslo 0372, Norway. FAU - Kemeny-Beke, Adam AU - Kemeny-Beke A AD - Department of Ophthalmology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary. LA - eng PT - Journal Article DEP - 20200918 PL - China TA - Int J Ophthalmol JT - International journal of ophthalmology JID - 101553860 PMC - PMC7459236 OTO - NOTNLM OT - Scheimpflug imaging OT - dermatomyositis OT - dry eye OT - extramuscular manifestations OT - polymyositis EDAT- 2020/09/22 06:00 MHDA- 2020/09/22 06:01 PMCR- 2020/09/18 CRDT- 2020/09/21 06:15 PHST- 2019/09/12 00:00 [received] PHST- 2020/05/13 00:00 [accepted] PHST- 2020/09/21 06:15 [entrez] PHST- 2020/09/22 06:00 [pubmed] PHST- 2020/09/22 06:01 [medline] PHST- 2020/09/18 00:00 [pmc-release] AID - ijo-13-09-1443 [pii] AID - 10.18240/ijo.2020.09.17 [doi] PST - epublish SO - Int J Ophthalmol. 2020 Sep 18;13(9):1443-1450. doi: 10.18240/ijo.2020.09.17. eCollection 2020. PMID- 26219969 OWN - NLM STAT- MEDLINE DCOM- 20170707 LR - 20220409 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 40 IP - 2 DP - 2015 Apr-Jun TI - Circulating adipokines and organ involvement in patients with systemic sclerosis. PG - 156-62 AB - BACKGROUND: In recent years, mediators synthesized in the adipose tissue, the so-called adipokines, have been reported to play important roles in the pathogenesis of autoimmune rheumatic diseases. OBJECTIVE: To compare serum leptin, adiponectin and resistin levels in patients with systemic sclerosis (SSc) and healthy controls. To find possible relationship between serum levels of adipokines and organ involvement with focus on interstitial lung disease in SSc patients. PATIENTS AND METHODS: Lung involvement was assessed functionally (body plethysmography, diffusing capacity of the lung for carbon monoxide (DLCO) and six-minute walk test) and radiologically (using average disease extent on high resolution computed tomography (HRCT) of the lungs according to the percentage of interstitial changes) in 29 SSc patients. Quantitative sandwich ELISA was used to measure resistin, leptin and adiponectin concentrations in sera of patients and 30 healthy controls. RESULTS: We found no statistically significant differences in serum resistin, leptin and adiponectin levels between SSc patients and the controls. However, serum adiponectin concentrations were significantly lower in active than in inactive patients, they also correlated positively with vital capacity (VC) (p=0.04) and negatively with Valentini activity score (p=0.04). Serum resistin levels were significantly elevated in patients with digital ulcers (p=0.03) and serum concentrations of leptin were associated with the duration of SSc symptoms other than Raynaud's phenomenon (p<0.01) CONCLUSIONS: Serum adiponectin should be further investigated as a candidate for SSc activity marker and resistin may play a role in ulcer development in SSc patients. FAU - Olewicz-Gawlik, A AU - Olewicz-Gawlik A FAU - Danczak-Pazdrowska, A AU - Danczak-Pazdrowska A FAU - Kuznar-Kaminska, B AU - Kuznar-Kaminska B FAU - Batura-Gabryel, H AU - Batura-Gabryel H FAU - Katulska, K AU - Katulska K FAU - Wojciech, S AU - Wojciech S FAU - Trzybulska, D AU - Trzybulska D FAU - Hrycaj, P AU - Hrycaj P LA - eng PT - Comparative Study PT - Journal Article PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 RN - 0 (ADIPOQ protein, human) RN - 0 (Adiponectin) RN - 0 (Leptin) RN - 0 (RETN protein, human) RN - 0 (Resistin) SB - IM MH - Adiponectin/*blood MH - Adult MH - Aged MH - Female MH - Humans MH - Leptin/*blood MH - Male MH - Middle Aged MH - Pilot Projects MH - Resistin/*blood MH - Scleroderma, Systemic/*blood/*complications MH - Young Adult EDAT- 2015/07/30 06:00 MHDA- 2017/07/08 06:00 CRDT- 2015/07/30 06:00 PHST- 2015/07/30 06:00 [entrez] PHST- 2015/07/30 06:00 [pubmed] PHST- 2017/07/08 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2015 Apr-Jun;40(2):156-62. PMID- 23568993 OWN - NLM STAT- MEDLINE DCOM- 20130807 LR - 20130409 IS - 1881-6096 (Print) IS - 1881-6096 (Linking) VI - 65 IP - 4 DP - 2013 Apr TI - [Myositis-specific autoantibodies]. PG - 449-60 AB - Idiopathic inflammatory myopathies are a group of acquired skeletal muscle diseases that include polymyositis, dermatomyositis, and inclusion body myositis. Studies have shown many myositis-specific autoantibodies (MSAs) that are useful for the diagnoses as well as classification of idiopathic inflammatory myopathies, because they have been shown to correlate with distinct clinical phenotypes. Anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-KS, anti-OJ, anti-Ha, and anti-Zo antibodies target aminoacyl tRNA synthetases, and represent anti-synthetase syndrome. Anti-synthetase syndrome is characterized by myositis, interstitial lung disease, arthritis, fever, Raynaud's phenomenon, and mechanic's hands. Anti-Mi-2, anti-MDA5 (anti-CADM140), anti-TIF1 (anti-155/140, anti-p155), anti-NXP-2 (anti-MJ), and anti-SAE antibodies are specific for dermatomyositis. In particular, anti-MDA5 antibodies are clinically associated with amyopathic dermatomyositis developing into rapidly progressive interstitial lung disease, whereas anti-TIF1 and anti-NXP-2 antibodies are closely correlated with cancer-associated dermatomyositis in adults. In addition, anti-TIF1 and anti-NXP-2 antibodies are predominant MSAs found in juvenile dermatomyositis, and the latter was correlated with a high incidence of calcinosis. Furthermore, anti-SRP and anti-3-hydroxy-3-methylglutaryl-coenzyme A (anti-HMG-CoA) antibodies have been found in patients with necrotizing myopathy. Moreover, a recent study suggested the presence of autoantibodies to a 43-kDa muscle protein in patients with inclusion body myositis. Although the pathogenic role of MSAs remains unknown, recent studies have shown that myositis autoantigens are expressed at high levels in regenerating muscle fibers, which may initiate or amplify autoimmune responses in idiopathic inflammatory myopathies. FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Kanazawa University Graduate School of Medicine, Japan. LA - jpn PT - English Abstract PT - Journal Article PT - Review PL - Japan TA - Brain Nerve JT - Brain and nerve = Shinkei kenkyu no shinpo JID - 101299709 RN - 0 (Acyl Coenzyme A) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 1553-55-5 (3-hydroxy-3-methylglutaryl-coenzyme A) SB - IM MH - Acyl Coenzyme A/immunology MH - Age Distribution MH - Autoantibodies/*immunology MH - Autoantigens/immunology MH - Humans MH - Lung Diseases, Interstitial/diagnosis/immunology MH - Myositis/diagnosis/*immunology/therapy EDAT- 2013/04/10 06:00 MHDA- 2013/08/08 06:00 CRDT- 2013/04/10 06:00 PHST- 2013/04/10 06:00 [entrez] PHST- 2013/04/10 06:00 [pubmed] PHST- 2013/08/08 06:00 [medline] AID - 1416101474 [pii] PST - ppublish SO - Brain Nerve. 2013 Apr;65(4):449-60. PMID- 23019977 OWN - NLM STAT- MEDLINE DCOM- 20121120 LR - 20121001 IS - 0023-2149 (Print) IS - 0023-2149 (Linking) VI - 90 IP - 7 DP - 2012 TI - [Cutaneous-visceral interplay in patients with systemic lupus erythematosus]. PG - 51-5 AB - Systemic lupus erythematosus (SLE) remains a challenging medical problem. The integral approach to the analysis of underlying pathogenetic processes allows identifying main symptom complexes of SLE and establishing relationship between skin lesions and activity of the disease. We examined 84 patients with SLE (84% women), their mean age was 42.3 +/- 2.3 yr duration of SLE 6.5 +/- 1.2 yr. The subacute and chronic SLE variants were diagnosed in 30 (36%) and 54 (64%) patients respectively. Grade 1 and 2-3 inflammatory process occurred in 53 (63%) and 31 (37%) patients respectively. Symptom complexes "systemic inflammation", "butterfly rash", "wrist petechiae", "enanthema of the oral mucous membrane", and other lesions were regarded as the markers of SLE activity. The relationship of lupus-cheilitis and facial erythema with polyserositis and pericarditis ("visceral pathology-cardiovascular lesions") requires instrumental examination of pericardium, pleural and abdominal cavities in the patients with the above skin symptoms for diagnostics of polyserositis. At the same time, the presence of teleangiectasia on the wrists (symptom complex "visceral pathology-renoparenchymatous lesions") requires thorough examination of the renal function. The presence of erythema at the major joints, mesh livedo, and Raynaud's syndrome (symptom complex "musculoskeletal disorders") implies specialized examination of the locomotor apparatus. FAU - Potekhin, N P AU - Potekhin NP FAU - Filatova, E A AU - Filatova EA FAU - Fursov, A N AU - Fursov AN FAU - Glad'ko, V V AU - Glad'ko VV FAU - Orlov, F A AU - Orlov FA LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Klin Med (Mosk) JT - Klinicheskaia meditsina JID - 2985204R SB - IM MH - Adult MH - Chronic Disease MH - Female MH - Humans MH - Lupus Erythematosus, Cutaneous/diagnosis/pathology/*physiopathology MH - Lupus Erythematosus, Systemic/diagnosis/pathology/*physiopathology MH - Male MH - Middle Aged MH - Severity of Illness Index MH - Skin/pathology/*physiopathology MH - Viscera/pathology/*physiopathology EDAT- 2012/10/02 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/10/02 06:00 PHST- 2012/10/02 06:00 [entrez] PHST- 2012/10/02 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] PST - ppublish SO - Klin Med (Mosk). 2012;90(7):51-5. PMID- 20149898 OWN - NLM STAT- MEDLINE DCOM- 20100920 LR - 20131121 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 9 IP - 7 DP - 2010 May TI - Phosphodiesterase inhibitors in the management of autoimmune disease. PG - 511-5 LID - 10.1016/j.autrev.2010.02.012 [doi] AB - Phophodiesterases inhibitors (PDEis) act by inhibiting the catabolism of cyclic nucleotides, cAMP and cGMP, which are ubiquitously expressed in cells of the immune system. Increased levels of cAMP and/or cGMP have been reported to decrease the activity of pro-inflammatory TH1 cells, attenuate experimental autoimmune encephalomyelitis and experimental arthritis. PDE5i like Sildenafil improves endothelial dysfunction and vascular remodelling in patients with pulmonary artery hypertension and refractory secondary Raynaud's phenomenon, with a potential to cause disease modification in the former. Studies in animal models of fibrosis suggest that these drugs have anti-fibrotic effect and may be potentially useful in conditions like scleroderma. They also have been shown to have renoprotective effect in animal models. The emerging trends make it necessary to exploit the full therapeutic potential of this class of drugs in various autoimmune diseases like rheumatoid arthritis, scleroderma, profibrotic conditions and PAH. CI - 2010 Elsevier B.V. All rights reserved. FAU - Shenoy, Padmanabha AU - Shenoy P AD - Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. drdpshenoy@gmail.com FAU - Agarwal, Vikas AU - Agarwal V LA - eng PT - Journal Article PT - Review DEP - 20100208 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Phosphodiesterase Inhibitors) RN - 0 (Transforming Growth Factor beta) RN - E0399OZS9N (Cyclic AMP) SB - IM MH - Animals MH - Autoimmune Diseases/*drug therapy/immunology/metabolism MH - Clinical Trials as Topic MH - Cyclic AMP/metabolism MH - Fibrosis/prevention & control MH - Humans MH - Kidney/blood supply/*drug effects/immunology MH - Neovascularization, Pathologic MH - Phosphodiesterase Inhibitors/*pharmacology/therapeutic use MH - Scleroderma, Systemic/*drug therapy/immunology/metabolism MH - Skin/blood supply/*drug effects/immunology MH - Transforming Growth Factor beta/antagonists & inhibitors RF - 40 EDAT- 2010/02/13 06:00 MHDA- 2010/09/21 06:00 CRDT- 2010/02/13 06:00 PHST- 2010/02/13 06:00 [entrez] PHST- 2010/02/13 06:00 [pubmed] PHST- 2010/09/21 06:00 [medline] AID - S1568-9972(10)00025-X [pii] AID - 10.1016/j.autrev.2010.02.012 [doi] PST - ppublish SO - Autoimmun Rev. 2010 May;9(7):511-5. doi: 10.1016/j.autrev.2010.02.012. Epub 2010 Feb 8. PMID- 17180299 OWN - NLM STAT- MEDLINE DCOM- 20070904 LR - 20220716 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 26 IP - 8 DP - 2007 Aug TI - Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. PG - 1259-62 AB - The aim of this paper was to study the spectrum of juvenile scleroderma (JSSc) seen at a tertiary care referral center in Asia. Retrospective analysis of case records of patients with systemic sclerosis, having age of onset less than 16 years and seen at our hospital from 1988 to 2004, was done. Patients with linear scleroderma and morphea were excluded. There were 23 patients (19 girls, 4 boys) with median age of onset of 12 years (range 5-16 years). The median age at presentation was 17 years (range 10-34 years). The median time from first symptoms to presentation was 4 years (range 0.2-26 years). Among these, 14 had diffuse systemic sclerosis (DSSc), while 9 had limited scleroderma (LSSc). The clinical features seen at presentation in patients were: Raynaud's phenomenon in 19, digital ulcers in 14, loss of finger tip pulp in 12, reflux in 8, dysphagia in 7, arthritis in 8, digital gangrene in 2, and pulmonary artery hypertension in 1. Antinuclear antibody was positive in 15 out of 18 patients tested. Interstitial lung disease was seen in 15 patients, 6 of whom had diffuse disease. The median skin score was 22 (range 7-48) . One patient died of primary pulmonary hypertension within 1 year of onset of symptoms. At a mean follow-up of 34 months, 14 patients were stable or had improvement in skin score or dyspnea on exertion. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good. FAU - Misra, Ramnath AU - Misra R AD - Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Singh, Gurmeet AU - Singh G FAU - Aggarwal, Parshant AU - Aggarwal P FAU - Aggarwal, Amita AU - Aggarwal A LA - eng PT - Journal Article DEP - 20061219 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Child MH - Female MH - Hospitals, Teaching MH - Humans MH - India MH - Male MH - Prognosis MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/*pathology EDAT- 2006/12/21 09:00 MHDA- 2007/09/05 09:00 CRDT- 2006/12/21 09:00 PHST- 2006/08/28 00:00 [received] PHST- 2006/11/06 00:00 [accepted] PHST- 2006/11/03 00:00 [revised] PHST- 2006/12/21 09:00 [pubmed] PHST- 2007/09/05 09:00 [medline] PHST- 2006/12/21 09:00 [entrez] AID - 10.1007/s10067-006-0483-z [doi] PST - ppublish SO - Clin Rheumatol. 2007 Aug;26(8):1259-62. doi: 10.1007/s10067-006-0483-z. Epub 2006 Dec 19. PMID- 37550754 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231121 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 7 IP - 1 DP - 2023 Aug 7 TI - Clinical diagnoses associated with a positive antinuclear antibody test in patients with and without autoimmune disease. PG - 24 LID - 10.1186/s41927-023-00349-4 [doi] LID - 24 AB - BACKGROUND: Antinuclear antibodies (ANA) are antibodies present in several autoimmune disorders. However, a large proportion of the general population (20%) also have a positive test; very few of these individuals will develop an autoimmune disease, and the clinical impact of a positive ANA in them is not known. Thus, we test the hypothesis that ANA + test reflects a state of immune dysregulation that alters risk for some clinical disorders in individuals without an autoimmune disease. METHODS: We performed high throughput association analyses in a case-control study using real world data from the de-identified electronic health record (EHR) system from Vanderbilt University Medical Center. The study population included individuals with an ANA titer ≥ 1:80 at any time (ANA +) and those with negative results (ANA-). The cohort was stratified into sub-cohorts of individuals with and without an autoimmune disease. A phenome-wide association study (PheWAS) adjusted by sex, year of birth, race, and length of follow-up was performed in the study cohort and in the sub-cohorts. As secondary analyses, only clinical diagnoses after ANA testing were included in the analyses. RESULTS: The cohort included 70,043 individuals: 49,546 without and 20,497 with an autoimmune disease, 26,579 were ANA + and 43,464 ANA-. In the study cohort and the sub-cohort with autoimmune disease, ANA + was associated (P ≤ 5 × 10(-5)) with 88 and 136 clinical diagnoses respectively, including lupus (OR ≥ 5.4, P ≤ 7.8 × 10(-202)) and other autoimmune diseases and complications. In the sub-cohort without autoimmune diseases, ANA + was associated with increased risk of Raynaud's syndrome (OR ≥ 2.1) and alveolar/perialveolar-related pneumopathies (OR ≥ 1.4) and decreased risk of hepatitis C, tobacco use disorders, mood disorders, convulsions, fever of unknown origin, and substance abuse disorders (OR ≤ 0.8). Analyses including only diagnoses after ANA testing yielded similar results. CONCLUSION: A positive ANA test, in addition to known associations with autoimmune diseases, Raynaud's phenomenon, and idiopathic fibrosing alveolitis related disorders, is associated with decreased prevalence of several non-autoimmune diseases. CI - © 2023. BioMed Central Ltd., part of Springer Nature. FAU - Zanussi, Jacy T AU - Zanussi JT AD - Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Zhao, Juan AU - Zhao J AD - Department of Biomedical Informatics, Vanderbilt University School of Medicine, Nashville, TN, USA. FAU - Wei, Wei-Qi AU - Wei WQ AD - Department of Biomedical Informatics, Vanderbilt University School of Medicine, Nashville, TN, USA. AD - Vanderbilt Genetics Institute, Vanderbilt University School of Medicine, Nashville, TN, USA. FAU - Karakoc, Gul AU - Karakoc G AD - Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Chung, Cecilia P AU - Chung CP AD - Vanderbilt Genetics Institute, Vanderbilt University School of Medicine, Nashville, TN, USA. AD - Division of Rheumatology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. AD - Tennessee Valley Healthcare System - Nashville Campus, Nashville, TN, USA. FAU - Feng, QiPing AU - Feng Q AD - Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. AD - Vanderbilt Genetics Institute, Vanderbilt University School of Medicine, Nashville, TN, USA. FAU - Olsen, Nancy J AU - Olsen NJ AD - Department of Medicine, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA. FAU - Stein, C Michael AU - Stein CM AD - Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. AD - Vanderbilt Genetics Institute, Vanderbilt University School of Medicine, Nashville, TN, USA. FAU - Kawai, Vivian K AU - Kawai VK AD - Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. vivian.k.kawai@vumc.org. AD - Vanderbilt Genetics Institute, Vanderbilt University School of Medicine, Nashville, TN, USA. vivian.k.kawai@vumc.org. LA - eng GR - S10 OD017985/OD/NIH HHS/United States GR - UL1 TR000445/TR/NCATS NIH HHS/United States GR - S10 RR025141/RR/NCRR NIH HHS/United States GR - R01AR076516/AR/NIAMS NIH HHS/United States GR - R01AR073764/AR/NIAMS NIH HHS/United States GR - S10 OD025092/OD/NIH HHS/United States GR - UL1 RR024975/RR/NCRR NIH HHS/United States GR - R01 AR076516/AR/NIAMS NIH HHS/United States GR - UL1 TR002243/TR/NCATS NIH HHS/United States PT - Journal Article DEP - 20230807 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC10405518 OTO - NOTNLM OT - Antinuclear antibodies OT - Disease risk OT - PheWAS COIS- The authors declare no competing interests. The authors declare no competing interests. EDAT- 2023/08/08 00:42 MHDA- 2023/08/08 00:43 PMCR- 2023/08/07 CRDT- 2023/08/07 23:46 PHST- 2022/11/06 00:00 [received] PHST- 2023/07/27 00:00 [accepted] PHST- 2023/08/08 00:43 [medline] PHST- 2023/08/08 00:42 [pubmed] PHST- 2023/08/07 23:46 [entrez] PHST- 2023/08/07 00:00 [pmc-release] AID - 10.1186/s41927-023-00349-4 [pii] AID - 349 [pii] AID - 10.1186/s41927-023-00349-4 [doi] PST - epublish SO - BMC Rheumatol. 2023 Aug 7;7(1):24. doi: 10.1186/s41927-023-00349-4. PMID- 41969635 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260413 LR - 20260413 IS - 2514-1775 (Electronic) IS - 2514-1775 (Linking) VI - 10 IP - 2 DP - 2026 TI - The missing link in connective tissue disease care: addressing the shortage of specialist nurses in the UK. PG - rkag031 LID - 10.1093/rap/rkag031 [doi] LID - rkag031 FAU - Finney, Diana AU - Finney D AD - Rheumatology, Sussex MSK Health, Sussex, UK. FAU - Parker, Louise AU - Parker L AD - Independent Nurse Consultant, Founder and Director, Raynaud's Clinic, Louise Parker Associates, London, UK. FAU - Gregory, Will AU - Gregory W AUID- ORCID: 0000-0002-1186-5806 AD - Rheumatology Department, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK. AD - Faculty of Health and Education, Manchester Metropolitan University, Manchester, UK. FAU - Turner, Angie AU - Turner A AD - Philanthropy, The Children's Trust, Tadworth, UK. LA - eng PT - Editorial DEP - 20260318 PL - England TA - Rheumatol Adv Pract JT - Rheumatology advances in practice JID - 101736676 PMC - PMC13064975 EDAT- 2026/04/13 18:17 MHDA- 2026/04/13 18:18 PMCR- 2026/03/18 CRDT- 2026/04/13 06:27 PHST- 2026/01/18 00:00 [accepted] PHST- 2026/04/13 18:18 [medline] PHST- 2026/04/13 18:17 [pubmed] PHST- 2026/04/13 06:27 [entrez] PHST- 2026/03/18 00:00 [pmc-release] AID - rkag031 [pii] AID - 10.1093/rap/rkag031 [doi] PST - epublish SO - Rheumatol Adv Pract. 2026 Mar 18;10(2):rkag031. doi: 10.1093/rap/rkag031. eCollection 2026. PMID- 39866260 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250128 IS - 2523-1995 (Electronic) IS - 2523-1995 (Linking) VI - 9 DP - 2025 TI - AQP4 antibody-seropositive neuromyelitis optica spectrum disorder in a patient with mixed connective tissue disease: a case report. PG - 30 LID - 10.21037/acr-23-48 [doi] LID - 30 AB - BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSDs) are degenerative diseases frequently associated with severe recurrences and high risk of progressive disability. In this report, we describe an unusual case of a patient with the coexistence between NMOSD and mixed connective tissue disease (MCTD). CASE DESCRIPTION: A 58-year-old Caucasian man was admitted to the Emergency Department (ED) with low back pain and walking inability. He had an unsteady gait, paraesthesia of the lower limbs and pain in the left lumbar area of the spine. He previously manifested repeated episodes of Raynaud's phenomenon. The neurological examination revealed pyramidal signs with asymmetric and progressive paraparesis associated with hypoesthesia and bladder dysfunction. A spine magnetic resonance imaging (MRI) revealed the presence of a long extensive cervico-dorsal myelitis. Among laboratory analyses, serum immunometric examinations came back positive for anti-RNP (272 U/mL) and anti-SSA (20 U/mL) antibodies, whereas a recombinant immunofluorescence assay revealed the presence of immunoglobulin G (IgG) antibodies against AQP4. Consequently, he was treated with high-doses of corticosteroids, with progressive resolution of symptoms. To date, his last cervico-dorsal spine MRI showed negative results. CONCLUSIONS: Only a few anecdotal cases of the coexistence between NMOSD and MCTD have so far been described, and many clinical aspects of this association are not yet fully known. Missed diagnosis of rheumatologic or neurologic diseases may lead to treatment delay and, potentially, irreversible disability. Closer collaboration between neurologists and rheumatologists is needed for the early diagnosis of both diseases. CI - 2025 AME Publishing Company. All rights reserved. FAU - Polilli, Ennio AU - Polilli E AD - Clinical Pathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Volpe, Paola AU - Volpe P AD - Reumathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Esposito, Jessica Elisabetta AU - Esposito JE AD - Clinical Pathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Di Risio, Annalisa AU - Di Risio A AD - Clinical Pathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Di Carmine, Caterina AU - Di Carmine C AD - Neurology and Stroke Unit, Pescara General Hospital, Pescara, Italy. FAU - Di Iorio, Giancarlo AU - Di Iorio G AD - Clinical Pathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Gabini, Marco AU - Gabini M AD - Reumathology Unit, Pescara General Hospital, Pescara, Italy. FAU - Tocco, Pierluigi AU - Tocco P AD - Neurology and Stroke Unit, Pescara General Hospital, Pescara, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20241206 PL - China TA - AME Case Rep JT - AME case reports JID - 101730832 PMC - PMC11761318 OTO - NOTNLM OT - AQP4-IgG OT - Devic’s syndrome OT - case report OT - mixed connective tissue disease (MCTD) OT - neuromyelitis optica spectrum disorder (NMOSD) COIS- Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-23-48/coif). The authors have no conflicts of interest to declare. EDAT- 2025/01/27 06:20 MHDA- 2025/01/27 06:21 PMCR- 2024/12/06 CRDT- 2025/01/27 05:35 PHST- 2023/04/16 00:00 [received] PHST- 2023/09/10 00:00 [accepted] PHST- 2025/01/27 06:21 [medline] PHST- 2025/01/27 06:20 [pubmed] PHST- 2025/01/27 05:35 [entrez] PHST- 2024/12/06 00:00 [pmc-release] AID - acr-09-23-48 [pii] AID - 10.21037/acr-23-48 [doi] PST - epublish SO - AME Case Rep. 2024 Dec 6;9:30. doi: 10.21037/acr-23-48. eCollection 2025. PMID- 38736967 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240514 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 35 IP - 1 DP - 2024 Mar TI - Clinical Characteristics and Outcomes of Interstitial Lung Disease in Primary Sjögren's Syndrome: A Retrospective Cohort Study. PG - 108-114 LID - 10.31138/mjr.230323.cca [doi] AB - OBJECTIVES: To describe the characteristics of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response. METHODS: All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed. RESULTS: Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud's phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders. CONCLUSION: ILD in primary Sjögren's syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort. CI - © 2024 The Mediterranean Journal of Rheumatology (MJR). FAU - Manikuppam, Prathyusha AU - Manikuppam P AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Padiyar, Shivraj AU - Padiyar S AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Yadav, Bijesh AU - Yadav B AD - Department of Biostatistics, Christian Medical College, Vellore, India. FAU - Nair, Avinash A AU - Nair AA AD - Department of Pulmonary Medicine, Christian Medical College, Vellore, India. FAU - Mane, Manisha AU - Mane M AD - Department of Radiology, Christian Medical College, Vellore, India. FAU - Mathew, John AU - Mathew J AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. LA - eng PT - Journal Article DEP - 20240116 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC11082778 OTO - NOTNLM OT - Sjögren's syndrome OT - interstitial lung disease OT - mycophenolate mofetil OT - spirometry COIS- The authors declare no conflicts of interest. EDAT- 2024/05/13 06:43 MHDA- 2024/05/13 06:44 PMCR- 2024/01/16 CRDT- 2024/05/13 04:04 PHST- 2023/03/26 00:00 [received] PHST- 2023/09/15 00:00 [revised] PHST- 2023/10/10 00:00 [accepted] PHST- 2024/05/13 06:44 [medline] PHST- 2024/05/13 06:43 [pubmed] PHST- 2024/05/13 04:04 [entrez] PHST- 2024/01/16 00:00 [pmc-release] AID - MJR-35-1-108 [pii] AID - 10.31138/mjr.230323.cca [doi] PST - epublish SO - Mediterr J Rheumatol. 2024 Jan 16;35(1):108-114. doi: 10.31138/mjr.230323.cca. eCollection 2024 Mar. PMID- 28752373 OWN - NLM STAT- MEDLINE DCOM- 20180702 LR - 20181113 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 53 IP - 3 DP - 2017 Dec TI - Skin Manifestations of Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, and Spondyloarthritides. PG - 371-393 LID - 10.1007/s12016-017-8632-5 [doi] AB - Extra-articular manifestations of rheumatoid arthritis, juvenile idiopathic arthritis, and various spondyloarthritides including psoriatic arthritis, ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated spondyloarthritis often involve the skin and may occur before or after diagnosis of these rheumatic diseases. Cutaneous manifestations encompass a wide range of reactions that may have a notable negative impact not only on the physical but especially on the emotional and psychosocial well-being of these patients. Several cutaneous manifestations have been related to rheumatoid arthritis such as subcutaneous nodules including classical rheumatoid nodules, accelerated rheumatoid nodulosis, and rheumatoid nodulosis; vascular disorders like rheumatoid vasculitis, livedo racemosa, and Raynaud's phenomenon; and neutrophilic and/or granulomatous diseases like pyoderma gangrenosum, Sweet's syndrome, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with arthritis, as well as palisaded neutrophilic and granulomatous dermatitis. In juvenile idiopathic arthritis, the main cutaneous manifestations include an evanescent rash, rheumatoid nodules, as well as plaque and guttate psoriasis. Plaque psoriasis is also the main skin disease involved in spondyloarthritides. Furthermore, other forms of psoriasis including guttate, inverse, erythrodermic, pustular, and particularly nail psoriasis may also occur. In addition, a variety of drug-induced skin reactions may also appear in these diseases. Early recognition and understanding of these different dermatologic manifestations together with an interdisciplinary approach are often needed to optimize management of these diseases. FAU - Chua-Aguilera, Carolyn Jean AU - Chua-Aguilera CJ AD - Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. FAU - Möller, Burkhard AU - Möller B AD - Department for Rheumatology, Immunology and Allergology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. FAU - Yawalkar, Nikhil AU - Yawalkar N AUID- ORCID: 0000-0003-0024-338X AD - Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland. Nikhil.yawalkar@insel.ch. LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 SB - IM MH - Arthritis, Juvenile/diagnosis/*immunology MH - Arthritis, Rheumatoid/diagnosis/*immunology MH - Diagnosis, Differential MH - Drug-Related Side Effects and Adverse Reactions/*diagnosis MH - Granuloma MH - Humans MH - Psoriasis MH - Skin/immunology/*pathology MH - Spondylitis, Ankylosing/diagnosis/*immunology MH - Vasculitis OTO - NOTNLM OT - DMARDs OT - Inflammatory bowel disease OT - Juvenile idiopathic arthritis OT - Psoriasis OT - Rheumatoid arthritis OT - Spondyloarthritis EDAT- 2017/07/29 06:00 MHDA- 2018/07/03 06:00 CRDT- 2017/07/29 06:00 PHST- 2017/07/29 06:00 [pubmed] PHST- 2018/07/03 06:00 [medline] PHST- 2017/07/29 06:00 [entrez] AID - 10.1007/s12016-017-8632-5 [pii] AID - 10.1007/s12016-017-8632-5 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2017 Dec;53(3):371-393. doi: 10.1007/s12016-017-8632-5. PMID- 41345716 OWN - NLM STAT- MEDLINE DCOM- 20260108 LR - 20260110 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 28 IP - 1 DP - 2025 Dec 4 TI - Transcriptome analysis unveils Th1 cell cycle signature as a distinctive feature of mixed connective tissue disease. PG - 5 LID - 10.1186/s13075-025-03707-4 [doi] LID - 5 AB - OBJECTIVES: Mixed connective tissue disease (MCTD) is characterized by positivity for anti-U1-RNP antibodies and a combination of symptoms of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and inflammatory myositis (IIM). The aim of this study was to elucidate the similarities and differences in gene expression profiles of peripheral blood immune cells between MCTD and its related diseases, as well as their association with clinical parameters. METHODS: Transcriptome analysis was performed in peripheral blood immune cells from 19 MCTD patients, 58 SLE patients, 63 SSc patients, 64 IIM patients, and 79 healthy controls (HC), comprising a total of 283 individuals across 27 immune cell subsets. Differential gene expression and enrichment analyses were conducted to compare MCTD with related diseases and HC. The association between dysregulated pathways in MCTD and clinical parameters was assessed. Gene modular and machine learning analyses were employed to identify related gene signatures in other immune cells. RESULTS: MCTD exhibited a higher number of differentially expressed genes (DEGs) in Th1 cells compared to other related diseases and HC. Although the DEGs between MCTD and SLE were limited, Th1 cells in MCTD shared common DEGs with each disease, and enrichment analysis revealed upregulation of cell cycle pathways in MCTD Th1 cells. This gene signature showed upregulation in high disease activity and in anti-U1-RNP antibody positive patients in related diseases, and it was associated with severity of Raynaud's phenomenon. Furthermore, the Th1 cell cycle signature correlated with interferon signature in other immune cells. CONCLUSIONS: Transcriptome analysis of peripheral blood immune cells revealed that MCTD Th1 cells share many transcriptional features with those in SLE, yet display distinctive cell cycle signature. Particularly, upregulation of cell cycle pathways was associated with characteristic clinical features of MCTD, such as positivity for anti-U1-RNP antibodies and Raynaud's phenomenon. This Th1 cell cycle signature holds promise for shedding light on the underlying pathophysiology of MCTD. CI - © 2025. The Author(s). FAU - Suwa, Yuichi AU - Suwa Y AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Nagafuchi, Yasuo AU - Nagafuchi Y AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. nagafuchi@g.ecc.u-tokyo.ac.jp. AD - Department of Functional Genomics and Immunological Diseases, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. nagafuchi@g.ecc.u-tokyo.ac.jp. AD - Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Tochigi, Japan. nagafuchi@g.ecc.u-tokyo.ac.jp. FAU - Yamada, Saeko AU - Yamada S AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Maeda, Junko AU - Maeda J AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Ota, Mineto AU - Ota M AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. AD - Department of Functional Genomics and Immunological Diseases, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Tsuchida, Yumi AU - Tsuchida Y AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Shoda, Hirofumi AU - Shoda H AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. AD - Department of Rheumatology, Tokyo Medical University Hospital, Tokyo, Japan. FAU - Okamura, Tomohisa AU - Okamura T AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. AD - Department of Functional Genomics and Immunological Diseases, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FAU - Fujio, Keishi AU - Fujio K AD - Department of Allergy and Rheumatology, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan. FUJIOK-INT@h.u-tokyo.ac.jp. LA - eng PT - Journal Article DEP - 20251204 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Humans MH - Female MH - *Mixed Connective Tissue Disease/genetics/immunology MH - Male MH - *Gene Expression Profiling/methods MH - Adult MH - Middle Aged MH - *Th1 Cells/immunology/metabolism MH - *Transcriptome MH - *Cell Cycle/genetics MH - Lupus Erythematosus, Systemic/genetics/immunology MH - Scleroderma, Systemic/genetics/immunology PMC - PMC12781259 OTO - NOTNLM OT - Mixed connective tissue disease OT - Systemic lupus erythematosus OT - Th1 OT - Transcriptome COIS- Declarations. Ethics approval and consent to participate: This study received approval from the Ethics Committee of the University of Tokyo (approval number: G10095), and written informed consent was obtained from all participants. Consent for publication: Consent to Publish declaration: not applicable. Competing interests: YN, MO and TO belonged to the Social Cooperation Program of the Department of Functional Genomics and Immunological Diseases, supported by Chugai Pharmaceutical. EDAT- 2025/12/05 01:02 MHDA- 2026/01/08 07:12 PMCR- 2025/12/04 CRDT- 2025/12/04 23:48 PHST- 2025/09/24 00:00 [received] PHST- 2025/11/26 00:00 [accepted] PHST- 2026/01/08 07:12 [medline] PHST- 2025/12/05 01:02 [pubmed] PHST- 2025/12/04 23:48 [entrez] PHST- 2025/12/04 00:00 [pmc-release] AID - 10.1186/s13075-025-03707-4 [pii] AID - 3707 [pii] AID - 10.1186/s13075-025-03707-4 [doi] PST - epublish SO - Arthritis Res Ther. 2025 Dec 4;28(1):5. doi: 10.1186/s13075-025-03707-4. PMID- 41953538 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260409 LR - 20260409 IS - 2296-861X (Print) IS - 2296-861X (Electronic) IS - 2296-861X (Linking) VI - 13 DP - 2026 TI - Association of lipid parameters with the development of disease complications in patients with limited cutaneous systemic sclerosis: a prospective exploratory cohort study. PG - 1794319 LID - 10.3389/fnut.2026.1794319 [doi] LID - 1794319 AB - BACKGROUND: Dyslipidemia may contribute to pathogenesis and disease complications in limited cutaneous systemic sclerosis (lcSSc), while data about its associations with endothelial dysfunction and clinical lcSSc-specific parameters as well as its predictive role on disease complications are limited. This study investigated the relationship between lipid parameters, endothelial dysfunction, and disease complications in patients with lcSSc. METHODS: 38 patients with lcSSc and 38 matched controls with primary Raynaud's phenomenon were analyzed at baseline regarding lipid parameters, parameters of endothelial dysfunction and clinical lcSSc-specific parameters. During a subsequent 3-year follow-up period, patients with lcSSc were prospectively observed for disease complications. RESULTS: Patients with lcSSc exhibited lower high-density lipoprotein (HDL) levels (p = 0.032) and total HDL particles (p = 0.001) as well as higher triglyceride levels (p = 0.018), triglycerides/HDL ratio (p = 0.013) and atherogenic index (p = 0.012) compared to controls. Large low-density lipoprotein (LDL) particles correlated positively with endothelial microparticles (r = 0.464, p = 0.044), while very low-density lipoprotein size correlated negatively with symmetric dimethylarginine (r = -0.480, p = 0.033). No significant correlations were observed between lipid parameters and clinical lcSSc-specific parameters. Incidence of macrovascular events and interstitial lung disease were associated with a few selected lipid parameters (all p < 0.05). CONCLUSION: Patients with lcSSc showed a more pro-atherogenic lipid profile. Although statistically significant, these results were of modest magnitude and should be interpreted in the context of the exploratory design. Associations with endothelial dysfunction and clinical disease parameters were minimal or non-significant. Selected lipid parameters may be associated with disease complications in lcSSc, but these findings require confirmation in larger, adequately powered studies due to the small sample size and exploratory analyses. CI - Copyright © 2026 Schweiger, Meinitzer, Strohmaier, Silbernagel, Moazedi-Fürst, Nemecz, Kurzmann-Gütl, Brodmann, Hafner and Jud. FAU - Schweiger, Leyla AU - Schweiger L AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Meinitzer, Andreas AU - Meinitzer A AD - Institute of Medical and Chemical Laboratory Diagnostics, Medical University Graz, Graz, Austria. FAU - Strohmaier, Heimo AU - Strohmaier H AD - Center of Medical Research (ZMF), Medical University of Graz, Graz, Austria. FAU - Silbernagel, Günther AU - Silbernagel G AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Moazedi-Fürst, Florentine AU - Moazedi-Fürst F AD - Division of Rheumatology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Nemecz, Viktoria AU - Nemecz V AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Kurzmann-Gütl, Katharina AU - Kurzmann-Gütl K AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Brodmann, Marianne AU - Brodmann M AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Hafner, Franz AU - Hafner F AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. FAU - Jud, Philipp AU - Jud P AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, Graz, Austria. LA - eng PT - Journal Article DEP - 20260324 PL - Switzerland TA - Front Nutr JT - Frontiers in nutrition JID - 101642264 PMC - PMC13053233 OTO - NOTNLM OT - cardiovascular risk OT - endothelial dysfunction OT - limited cutaneous systemic sclerosis OT - lipoprotein metabolism OT - vascular biomarkers COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author MB declared that they were an editorial board member of Frontiers at the time of submission. This had no impact on the peer review process and the final decision. EDAT- 2026/04/09 06:30 MHDA- 2026/04/09 06:31 PMCR- 2026/03/24 CRDT- 2026/04/09 04:43 PHST- 2026/01/23 00:00 [received] PHST- 2026/02/12 00:00 [revised] PHST- 2026/03/09 00:00 [accepted] PHST- 2026/04/09 06:31 [medline] PHST- 2026/04/09 06:30 [pubmed] PHST- 2026/04/09 04:43 [entrez] PHST- 2026/03/24 00:00 [pmc-release] AID - 10.3389/fnut.2026.1794319 [doi] PST - epublish SO - Front Nutr. 2026 Mar 24;13:1794319. doi: 10.3389/fnut.2026.1794319. eCollection 2026. PMID- 35987646 OWN - NLM STAT- MEDLINE DCOM- 20220823 LR - 20250728 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Aug 20 TI - Non-criteria antiphospholipid antibodies and pediatric rheumatic disease: a case series. PG - 70 LID - 10.1186/s12969-022-00732-4 [doi] LID - 70 AB - BACKGROUND: Non-criteria antiphospholipid antibodies (NC-aPL) are a relatively undefined subgroup of antiphospholipid antibodies (aPL). Knowledge about NC-aPL in adults is limited and even less is known in pediatric patients. Routine tests for antiphospholipid syndrome (APS)-a clinical state marked by the presence of aPL in association with vascular thrombosis-usually include lupus anticoagulant (LAC), anti-cardiolipin (aCL) and -beta-2 glycoprotein I (aβ2GPI). LAC is a functional screen for prothrombotic aPL, while the latter tests identify specific autoantibodies. Specific targets of NC-aPL include, but are not limited to, phosphatidylethanolamine, phosphatidylserine, and prothrombin. PRESENTATION OF CASES: We present single-center data from eight pediatric patients with NC-aPL identified during a three-year period. All patients had presenting features raising suspicion for APS. Most patients were female with a primary rheumatic disease. One patient had a stroke. Another patient had alveolar hemorrhage and pulmonary hypertension. Raynaud's phenomenon, rashes involving distal extremities, and headaches were common. Most patients had a positive LAC, yet their routine aPL tests were negative, prompting testing for NC-aPL. CONCLUSIONS: Our findings suggest NC-aPL are associated with typical signs and symptoms of APS in pediatric patients. Pediatricians and pediatric subspecialists should consider NC-aPL when clinical suspicion is high and routine aPL tests are negative, particularly when LAC is positive. While guidelines for NC-aPL do not yet exist for children or adults, these autoantibodies have pathogenic potential. Actionable items could include evaluation for the presence of other (primary) rheumatic diseases, and consultation with hematologists and/or obstetricians regarding anticoagulation/platelet inhibition and thrombosis education. Future guidelines regarding NC-aPL will only be generated by gathering more data, ideally prospectively. CI - © 2022. The Author(s). FAU - Mahmud, Shawn A AU - Mahmud SA AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. AD - Center for Immunology, University of Minnesota, Minneapolis, MN, USA. FAU - Bullock, Danielle R AU - Bullock DR AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. FAU - Correll, Colleen K AU - Correll CK AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. FAU - Hobday, Patricia M AU - Hobday PM AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. FAU - Riskalla, Mona M AU - Riskalla MM AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. FAU - Vehe, Richard K AU - Vehe RK AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. FAU - Binstadt, Bryce A AU - Binstadt BA AD - Department of Pediatrics, Division of Pediatric Rheumatology, Allergy & Immunology, University of Minnesota, AO-10 Academic Office Building, 2414 S. 7th Street, Minneapolis, MN, USA. binstadt@umn.edu. AD - Center for Immunology, University of Minnesota, Minneapolis, MN, USA. binstadt@umn.edu. LA - eng PT - Journal Article DEP - 20220820 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Autoantibodies) RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Adult MH - Antibodies, Antiphospholipid MH - *Antiphospholipid Syndrome/complications/diagnosis MH - Autoantibodies MH - Child MH - Female MH - Humans MH - Lupus Coagulation Inhibitor MH - Male MH - *Rheumatic Diseases/complications/diagnosis MH - *Thrombosis PMC - PMC9392300 OTO - NOTNLM OT - Antiphospholipid syndrome OT - Lupus anticoagulant OT - Non-criteria antiphospholipid antibodies OT - Rheumatic disease COIS- There are no conflicts of interest. EDAT- 2022/08/21 06:00 MHDA- 2022/08/24 06:00 PMCR- 2022/08/20 CRDT- 2022/08/20 23:34 PHST- 2022/05/13 00:00 [received] PHST- 2022/08/15 00:00 [accepted] PHST- 2022/08/20 23:34 [entrez] PHST- 2022/08/21 06:00 [pubmed] PHST- 2022/08/24 06:00 [medline] PHST- 2022/08/20 00:00 [pmc-release] AID - 10.1186/s12969-022-00732-4 [pii] AID - 732 [pii] AID - 10.1186/s12969-022-00732-4 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Aug 20;20(1):70. doi: 10.1186/s12969-022-00732-4. PMID- 32892413 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 48 IP - 1 DP - 2021 Jan TI - Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti-aminoacyl-tRNA synthetase antibody. PG - 34-41 LID - 10.1111/1346-8138.15602 [doi] AB - Homocysteine is a sulfhydryl-containing amino acid that is derived from dietary methionine, and there has been increasing evidence that elevated plasma homocysteine levels are associated with increased risk of central and peripheral vascular disorders, including carotid, coronary and peripheral arterial diseases, and Raynaud's phenomenon. Recently, associations of plasma homocysteine levels with autoimmune diseases such as systemic lupus erythematodes and systemic sclerosis have been reported. However, no study analyzed the association between plasma homocysteine levels and dermatomyositis (DM). The objective of this study was to examine plasma homocysteine levels and their clinical associations in patients with DM. Plasma homocysteine levels in 28 Japanese patients with DM and 22 healthy controls were examined. We found that the plasma homocysteine levels in DM patients were significantly higher than those in healthy individuals (15.8 ± 1.1 vs 8.5 ± 0.5 µmol/L, P < 0.01). Presence of mechanic's hand, complication of interstitial lung disease (ILD), high serum Krebs von den Lungen-6 (KL-6), surfactant protein-D and creatine kinase levels, and anti-aminoacyl-tRNA synthetase (ARS) antibody (Ab) positivity were significantly more prevalent among DM patients with elevated plasma homocysteine levels. The plasma homocysteine levels in DM patients with mechanic's hand, ILD and anti-ARS Ab were significantly higher than those in DM without those features. Furthermore, the plasma homocysteine levels were positively correlated with serum KL-6 levels. These results suggest that the pathogenesis of elevated plasma homocysteine levels may be associated with ILD in DM patients, especially with anti-ARS Ab, and further examination is required. CI - © 2020 Japanese Dermatological Association. FAU - Sekiguchi, Akiko AU - Sekiguchi A AUID- ORCID: 0000-0002-7016-337X AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Endo, Yukie AU - Endo Y AUID- ORCID: 0000-0003-3058-6279 AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Yamazaki, Sahori AU - Yamazaki S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Uchiyama, Akihiko AU - Uchiyama A AUID- ORCID: 0000-0002-2169-2427 AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Shimizu, Akira AU - Shimizu A AUID- ORCID: 0000-0001-7742-079X AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Motegi, Sei-Ichiro AU - Motegi SI AUID- ORCID: 0000-0001-8286-0669 AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. LA - eng PT - Journal Article DEP - 20200906 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Autoantibodies) RN - 0LVT1QZ0BA (Homocysteine) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - *Amino Acyl-tRNA Synthetases MH - Autoantibodies MH - *Dermatomyositis/complications MH - Homocysteine MH - Humans MH - *Lung Diseases, Interstitial/etiology OTO - NOTNLM OT - anti-aminoacyl-tRNA synthetase antibody OT - dermatomyositis OT - fibrosis OT - homocysteine OT - interstitial lung disease EDAT- 2020/09/07 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/09/06 20:37 PHST- 2020/05/22 00:00 [received] PHST- 2020/08/14 00:00 [accepted] PHST- 2020/09/07 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/09/06 20:37 [entrez] AID - 10.1111/1346-8138.15602 [doi] PST - ppublish SO - J Dermatol. 2021 Jan;48(1):34-41. doi: 10.1111/1346-8138.15602. Epub 2020 Sep 6. PMID- 28476103 OWN - NLM STAT- MEDLINE DCOM- 20171108 LR - 20181202 IS - 1471-2377 (Electronic) IS - 1471-2377 (Linking) VI - 17 IP - 1 DP - 2017 May 5 TI - Intracranial lesion as onset symptom in a patient with early undifferentiated connective tissue disease: a case report. PG - 85 LID - 10.1186/s12883-017-0868-4 [doi] LID - 85 AB - BACKGROUND: Undifferentiated connective tissue disease (UCTD) is widely considered to be a distinct clinical entity, and now divided into two subgroups: stable UCTD and early UCTD. The most frequent onset symptoms of UCTD include arthralgias, arthritis, Raynaud's phenomenon, mucocutaneous involvement, and sicca symptoms. However, Neurologic involvement is rare, and intracranial lesion as onset symptom in a patient with early UCTD has not yet been reported. CASE PRESENTATION: A 51-year-old Chinese female experienced progressive left leg weakness for 14 days before hospitalizing in our department. The lesion on right parietal lobe was initially detected by brain magnetic resonance imaging. Although the patient declined a cerebral biopsy, the possibility of stroke, cerebral venous sinus thrombosis, NMOSD, MS, autoimmune encephalitis, intracranial infections, and malignant tumors as cause of the lesion was excluded by intracranial angiogram, CSF study, MRI enhancement and MRS examination. Moreover, immunologic studies showed high titer of antinuclear antibody, increased erythrocyte sedimentation rate and C-reactive protein. These results led to a diagnosis of early UCTD with central nerve system (CNS) involvement. After low dose corticosteroid and azathioprine therapy, the patient's symptoms, abnormalities in immunologic tests and cerebral radiologic examinations were all greatly improved within a short duration. CONCLUSIONS: This is the first report of intracranial lesion as onset symptom in a patient with early UCTD. Our case suggested that central nerve system (CNS) involvement could be the onset symptom in early UCTD, and should be recognized quickly with exclusion of other causative factors in the differential diagnosis. Prompt and adequate treatment with low-dose steroid and immunosuppressive drugs could improve the prognosis of both early UCTD and CNS involvement. FAU - Du, Ying AU - Du Y AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. FAU - Li, Chuan AU - Li C AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. FAU - Zhao, Dai-di AU - Zhao DD AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. FAU - Lu, Jia-Rui AU - Lu JR AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. FAU - Zhang, Wei AU - Zhang W AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. zw711711@hotmail.com. FAU - Li, Zhu-Yi AU - Li ZY AD - Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an City, Shaanxi Province, 710038, China. lizhuyi@fmmu.edu.cn. LA - eng PT - Case Reports PT - Journal Article DEP - 20170505 PL - England TA - BMC Neurol JT - BMC neurology JID - 100968555 RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Biopsy MH - C-Reactive Protein MH - Connective Tissue Diseases/*diagnosis MH - Diagnosis, Differential MH - Female MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Middle Aged MH - Prognosis PMC - PMC5420101 OTO - NOTNLM OT - Intracranial lesion OT - Undifferentiated connective tissue disease EDAT- 2017/05/10 06:00 MHDA- 2017/11/09 06:00 PMCR- 2017/05/05 CRDT- 2017/05/07 06:00 PHST- 2017/02/08 00:00 [received] PHST- 2017/05/01 00:00 [accepted] PHST- 2017/05/07 06:00 [entrez] PHST- 2017/05/10 06:00 [pubmed] PHST- 2017/11/09 06:00 [medline] PHST- 2017/05/05 00:00 [pmc-release] AID - 10.1186/s12883-017-0868-4 [pii] AID - 868 [pii] AID - 10.1186/s12883-017-0868-4 [doi] PST - epublish SO - BMC Neurol. 2017 May 5;17(1):85. doi: 10.1186/s12883-017-0868-4. PMID- 18001298 OWN - NLM STAT- MEDLINE DCOM- 20080225 LR - 20071116 IS - 1744-3121 (Print) IS - 1744-3121 (Linking) VI - 34 IP - 6 DP - 2007 Dec TI - Genetic susceptibility to SLE is associated with TNF-alpha gene polymorphism -863, but not -308 and -238, in Thai population. PG - 425-30 AB - The production of cytokine varies among individuals and correlates with the polymorphism of cytokine genes. Three functional single nucleotide polymorphisms (SNPs) at position -863, -308, and -238 in the tumour necrosis factor alpha (TNF-alpha) gene promoter were analysed for association with systemic lupus erythematosus (SLE) (n = 154), and clinical manifestations in a Thai population were compared with 154 ethnically matched controls. The genotyping was determined by polymerase chain reaction-restriction fragment length polymorphism method. The association between these SNPs and SLE was analysed using chi-squared test. The -863A allele and -863A, -308G, -238G haplotype were found to be significantly increased in SLE patients (25%) compared with healthy controls (15.3%) (Pc = 0.009, OR = 1.85, 95% CI = 1.21-2.83). In addition -863A allele was found to be significantly increased in the SLE group with Raynaud's phenomenon compared to SLE without Raynaud's phenomenon (35% vs. 19.4%) (Pc = 0.048, OR = 2.23, 95% CI = 1.21-4.10). The -863A allele of TNF-alpha gene and the extended haplotype of -863A, -308G, -238G can be used as a genetic marker for SLE susceptibility in Thai populations. In addition, the -863A genotype could produce high TNF levels and potentially induce the occurrence of Raynaud's phenomenon. FAU - Hirankarn, N AU - Hirankarn N AD - Lupus Research Unit, Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. fmednpt@md.chula.ac.th FAU - Avihingsanon, Y AU - Avihingsanon Y FAU - Wongpiyabovorn, J AU - Wongpiyabovorn J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Int J Immunogenet JT - International journal of immunogenetics JID - 101232337 RN - 0 (Tumor Necrosis Factor-alpha) SB - IM MH - Adult MH - Alleles MH - Female MH - *Genetic Predisposition to Disease MH - Genotype MH - Haplotypes MH - Humans MH - Lupus Erythematosus, Systemic/*genetics MH - Male MH - Middle Aged MH - Polymorphism, Restriction Fragment Length MH - Polymorphism, Single Nucleotide/*genetics MH - Thailand MH - Tumor Necrosis Factor-alpha/blood/*genetics EDAT- 2007/11/16 09:00 MHDA- 2008/02/26 09:00 CRDT- 2007/11/16 09:00 PHST- 2007/11/16 09:00 [pubmed] PHST- 2008/02/26 09:00 [medline] PHST- 2007/11/16 09:00 [entrez] AID - EJI715 [pii] AID - 10.1111/j.1744-313X.2007.00715.x [doi] PST - ppublish SO - Int J Immunogenet. 2007 Dec;34(6):425-30. doi: 10.1111/j.1744-313X.2007.00715.x. PMID- 41976875 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260414 LR - 20260415 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 15 IP - 7 DP - 2026 Mar 27 TI - Ischemic Vascular Complications in Early Systemic Sclerosis (SSc): A Longitudinal Inception Cohort Study of Associated Clinical Factors and Mortality. LID - 10.3390/jcm15072575 [doi] LID - 2575 AB - Background/Objectives: Predictors of ischemic vascular complications (IVCs)-including coronary artery disease (CAD), ischemic stroke, and digital gangrene-in patients with early SSc remain insufficiently defined. Therefore, we aim to determine the incidence, risk factors, and mortality associated with IVCs in early SSc. Methods: An inception cohort of patients with early SSc at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, was studied from January 2010 to December 2023. Clinical, laboratory, and cardiopulmonary assessments were performed at baseline and annually thereafter. Results: A total of 146 patients (83 female, 119 DcSSc) were enrolled, with a mean disease duration of 11.5 ± 8.9 months from the first non-Raynaud's phenomenon (NRP). The mean follow-up was 8.0 ± 3.9 years. Seventeen patients (11.6%) developed IVCs, three CAD, four ischemic stroke, eight digital gangrene, and two digital gangrene plus CAD. The median time to first IVCs was two years. The overall incidence rate of IVCs from the NRP was 1.44 per 100 person-years (95% CI 0.89-2.32). Independent factors associated with IVCs included baseline (BL) digital ulcer, traumatic ulcer, LVEF < 50%, elevated pro-BNP, and any atrial fibrillation. BL pro-BNP and dyslipidemia were independently associated with CAD, whereas BL pro-BNP and any atrial fibrillation were associated with ischemic stroke. BL digital ulcer, traumatic ulcer, and any LVEF < 50% were associated with digital gangrene. All-cause mortality was higher among patients with IVCs than those without (9 [52.9%] vs. 37 [28.7], p = 0.043). Conclusions: In this study, IVCs were uncommon in early SSc, but were associated with increased mortality. Digital ulcers, traumatic ulcers, atrial fibrillation, impaired LVEF, and elevated pro-BNP identified the patients at higher risk of IVCs. FAU - Wangkaew, Suparaporn AU - Wangkaew S AUID- ORCID: 0000-0001-7030-5043 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. FAU - Preecha, Chammaliang AU - Preecha C AUID- ORCID: 0009-0006-2090-0139 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. FAU - Prasertwitayakij, Narawudt AU - Prasertwitayakij N AUID- ORCID: 0000-0002-7470-329X AD - Division of Cardiology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. FAU - Euathrongchit, Juntima AU - Euathrongchit J AD - Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand. LA - eng PT - Journal Article DEP - 20260327 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC13073291 OTO - NOTNLM OT - coronary artery disease OT - digital gangrene OT - scleroderma OT - stroke OT - systemic sclerosis COIS- All authors have declared no conflicts of interest related to this study. EDAT- 2026/04/14 06:32 MHDA- 2026/04/14 06:33 PMCR- 2026/03/27 CRDT- 2026/04/14 01:06 PHST- 2026/02/20 00:00 [received] PHST- 2026/03/14 00:00 [revised] PHST- 2026/03/25 00:00 [accepted] PHST- 2026/04/14 06:33 [medline] PHST- 2026/04/14 06:32 [pubmed] PHST- 2026/04/14 01:06 [entrez] PHST- 2026/03/27 00:00 [pmc-release] AID - jcm15072575 [pii] AID - jcm-15-02575 [pii] AID - 10.3390/jcm15072575 [doi] PST - epublish SO - J Clin Med. 2026 Mar 27;15(7):2575. doi: 10.3390/jcm15072575. PMID- 35634293 OWN - NLM STAT- MEDLINE DCOM- 20220602 LR - 20220716 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 13 DP - 2022 TI - Aminoacyl-tRNA Synthetases: On Anti-Synthetase Syndrome and Beyond. PG - 866087 LID - 10.3389/fimmu.2022.866087 [doi] LID - 866087 AB - Anti-synthetase syndrome (ASSD) is an autoimmune disease characterized by the presence of autoantibodies targeting one of several aminoacyl t-RNA synthetases (aaRSs) along with clinical features including interstitial lung disease, myositis, Raynaud's phenomenon, arthritis, mechanic's hands, and fever. The family of aaRSs consists of highly conserved cytoplasmic and mitochondrial enzymes, one for each amino acid, which are essential for the RNA translation machinery and protein synthesis. Along with their main functions, aaRSs are involved in the development of immune responses, regulation of transcription, and gene-specific silencing of translation. During the last decade, these proteins have been associated with cancer, neurological disorders, infectious responses, and autoimmune diseases including ASSD. To date, several aaRSs have been described to be possible autoantigens in different diseases. The most commonly described are histidyl (HisRS), threonyl (ThrRS), alanyl (AlaRS), glycyl (GlyRS), isoleucyl (IleRS), asparaginyl (AsnRS), phenylalanyl (PheRS), tyrosyl (TyrRS), lysyl (LysRS), glutaminyl (GlnRS), tryptophanyl (TrpRS), and seryl (SerRS) tRNA synthetases. Autoantibodies against the first eight autoantigens listed above have been associated with ASSD while the rest have been associated with other diseases. This review will address what is known about the function of the aaRSs with a focus on their autoantigenic properties. We will also describe the anti-aaRSs autoantibodies and their association to specific clinical manifestations, and discuss their potential contribution to the pathogenesis of ASSD. CI - Copyright © 2022 Galindo-Feria, Notarnicola, Lundberg and Horuluoglu. FAU - Galindo-Feria, Angeles S AU - Galindo-Feria AS AD - Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden. AD - Center for Molecular Medicine, Karolinska Institutet, and Karolinska University Hospital Solna, Stockholm, Sweden. FAU - Notarnicola, Antonella AU - Notarnicola A AD - Center for Molecular Medicine, Karolinska Institutet, and Karolinska University Hospital Solna, Stockholm, Sweden. FAU - Lundberg, Ingrid E AU - Lundberg IE AD - Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden. AD - Center for Molecular Medicine, Karolinska Institutet, and Karolinska University Hospital Solna, Stockholm, Sweden. FAU - Horuluoglu, Begum AU - Horuluoglu B AD - Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden. AD - Center for Molecular Medicine, Karolinska Institutet, and Karolinska University Hospital Solna, Stockholm, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20220513 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 63231-63-0 (RNA) RN - EC 6.- (Ligases) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - *Amino Acyl-tRNA Synthetases/genetics/metabolism MH - Autoantibodies MH - Autoantigens MH - *Ligases MH - RNA PMC - PMC9136399 OTO - NOTNLM OT - Anti-synthetase syndrome (ASSD) OT - aminoacyl-tRNA synthetase OT - autoantibodies OT - autoantigens OT - autoimmunity OT - interstitial lung disease OT - myositis COIS- IL has received consulting fees from Corbus Pharmaceuticals, Inc. and research grants from Astra Zeneca and has been serving on the advisory board for Astra Zeneca, Bristol Myers Squibb, Corbus Pharmaceutical, EMD Serono Research & Development Institute, Argenx, Octapharma, Kezaar, Orphazyme, and Janssen and has stock shares in Roche and Novartis. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/06/01 06:00 MHDA- 2022/06/03 06:00 PMCR- 2022/01/01 CRDT- 2022/05/31 09:53 PHST- 2022/01/30 00:00 [received] PHST- 2022/03/28 00:00 [accepted] PHST- 2022/05/31 09:53 [entrez] PHST- 2022/06/01 06:00 [pubmed] PHST- 2022/06/03 06:00 [medline] PHST- 2022/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2022.866087 [doi] PST - epublish SO - Front Immunol. 2022 May 13;13:866087. doi: 10.3389/fimmu.2022.866087. eCollection 2022. PMID- 27682894 OWN - NLM STAT- MEDLINE DCOM- 20170116 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 16 IP - 1 DP - 2017 Jan TI - Interstitial lung disease in primary Sjögren's syndrome. PG - 48-54 LID - S1568-9972(16)30212-9 [pii] LID - 10.1016/j.autrev.2016.09.017 [doi] AB - Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n=5), was concurrently identified in association with pSS (n=6) and developed after pSS onset (n=9). Presenting ILD manifestations were: acute/subacute (n=11) onset of ILD, symptomatic progressive onset of ILD (n=5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n=5). ILD therapy included: steroids (n=21), cyclophosphamide (n=1), azathioprine (n=4) and rituximab (n=1). The course of ILD was as follows: improvement (15.8%), stabilization (47.4%) or deterioration (36.8%). Predictive parameters of ILD onset were: older age (p=0.044), Raynaud's phenomenon (p=0.001) and esophageal involvement (p=0.001). Factors associated with ILD deterioration were: older age (p=0.038) and esophageal involvement (p=0.038). Thus, this study underscores the poor outcome of ILD during pSS; thus, systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at early stage of ILD. We also suggest that patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy. CI - Copyright © 2016 Elsevier B.V. All rights reserved. FAU - Roca, F AU - Roca F AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. FAU - Dominique, S AU - Dominique S AD - Department of Pneumology, Rouen University Hospital, Rouen, France. FAU - Schmidt, J AU - Schmidt J AD - Department of Internal Medicine, Amiens University Hospital, Amiens, France. FAU - Smail, A AU - Smail A AD - Department of Internal Medicine, Amiens University Hospital, Amiens, France. FAU - Duhaut, P AU - Duhaut P AD - Department of Internal Medicine, Amiens University Hospital, Amiens, France. FAU - Lévesque, H AU - Lévesque H AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. FAU - Marie, I AU - Marie I AD - Department of Internal Medicine, Rouen University Hospital, Rouen, France; INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. Electronic address: isabelle.marie@chu-rouen.fr. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20160925 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Diseases, Interstitial/drug therapy/epidemiology/*etiology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Sjogren's Syndrome/*complications/epidemiology MH - Young Adult OTO - NOTNLM OT - Interstitial lung disease OT - Predictive factors OT - Primary Sjögren's syndrome OT - Pulmonary involvement OT - Therapy EDAT- 2016/09/30 06:00 MHDA- 2017/01/17 06:00 CRDT- 2016/09/30 06:00 PHST- 2016/07/27 00:00 [received] PHST- 2016/08/08 00:00 [accepted] PHST- 2016/09/30 06:00 [pubmed] PHST- 2017/01/17 06:00 [medline] PHST- 2016/09/30 06:00 [entrez] AID - S1568-9972(16)30212-9 [pii] AID - 10.1016/j.autrev.2016.09.017 [doi] PST - ppublish SO - Autoimmun Rev. 2017 Jan;16(1):48-54. doi: 10.1016/j.autrev.2016.09.017. Epub 2016 Sep 25. PMID- 42240290 OWN - NLM STAT- Publisher LR - 20260608 IS - 1747-6356 (Electronic) IS - 1747-6348 (Linking) DP - 2026 Jun 7 TI - Role of myositis autoantibodies in diagnosing interstitial lung disease in patients with idiopathic inflammatory myopathies: a retrospective analysis. PG - 1-11 LID - 10.1080/17476348.2026.2684330 [doi] AB - BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are frequently complicated by interstitial lung disease (ILD). The independent clinical significance of myositis autoantibody positivity remains incompletely defined. METHODS: We performed a retrospective cohort study of patients undergoing myositis autoantibody testing at a single tertiary center (1997-2022). Multivariable logistic regression assessed the association between antibody positivity and ILD. Subgroup analyses evaluated clinical phenotypes and radiologic patterns. RESULTS: Among 1,034 patients included in the analysis, 359 (34.7%) were positive for at least one myositis autoantibody and 365 (35.5%) had ILD. Antibody-positive patients were younger, more frequently female, and had a higher prevalence of ILD compared to antibody-negative patients (41.2% vs. 32.2%, p < 0.01). Myositis autoantibody positivity was independently associated with ILD (adjusted OR 1.78, 95% CI 1.33-2.37, p < 0.001). Increasing age, higher BMI, former smoking status, and Asian ethnicity were also associated with ILD. Among patients with ILD, antibody-positive individuals had a higher prevalence of systemic autoimmune features, including Raynaud's phenomenon, arthritis, and mechanic's hands, as well as higher inflammatory markers and more impaired pulmonary function. CONCLUSION: Myositis autoantibody positivity is independently associated with ILD and identifies a distinct clinical phenotype. These findings should be interpreted in the context of the study's retrospective, single-center design. FAU - Alrehaili, Ghadah A AU - Alrehaili GA AD - Department of Internal Medicine, College of Medicine, Princess Nourah Bint Abdul Rahman University, Riyadh, MN, USA. AD - Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine Mayo Clinic, Rochester, MN, USA. FAU - Farah, Wigdan H AU - Farah WH AD - Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine Mayo Clinic, Rochester, MN, USA. FAU - Abusalih, Mohamed AU - Abusalih M AD - Evidence-Based Practice Center, Mayo Clinic, Rochester, MN, USA. FAU - Ramachandran, Neha AU - Ramachandran N AD - Evidence-Based Practice Center, Mayo Clinic, Rochester, MN, USA. FAU - Salim, Ahmed AU - Salim A AD - Division of Hospital Internal Medicine, Mayo Clinic Healthcare System, Red Wing, MN, USA. FAU - Kruger, Livia F AU - Kruger LF AD - Division of Thoracic Radiology, Mayo Clinic, Rochester, MN, USA. FAU - Kohli, Akshay AU - Kohli A AD - Division of Pulmonary and Critical Care Medicine, Mayo Clinic School of Graduate Medical Education, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Baqir, Misbah AU - Baqir M AD - Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine Mayo Clinic, Rochester, MN, USA. LA - eng PT - Journal Article DEP - 20260607 PL - England TA - Expert Rev Respir Med JT - Expert review of respiratory medicine JID - 101278196 SB - IM OTO - NOTNLM OT - Anti-Jo-1 OT - idiopathic inflammatory myopathies OT - interstitial lung disease OT - myositis, NSIP OT - pulmonary function tests EDAT- 2026/06/04 12:42 MHDA- 2026/06/04 12:42 CRDT- 2026/06/04 08:52 PHST- 2026/06/04 12:42 [pubmed] PHST- 2026/06/04 12:42 [medline] PHST- 2026/06/04 08:52 [entrez] AID - 10.1080/17476348.2026.2684330 [doi] PST - aheadofprint SO - Expert Rev Respir Med. 2026 Jun 7:1-11. doi: 10.1080/17476348.2026.2684330. PMID- 42172612 OWN - NLM STAT- In-Process LR - 20260609 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 65 IP - 6 DP - 2026 Jun 3 TI - The clinical utility of telangiectasia quantification as markers of vascular disease progression in systemic sclerosis. LID - keag267 [pii] LID - 10.1093/rheumatology/keag267 [doi] AB - OBJECTIVES: Telangiectasia are common in SSc. We explored the relationship between the site and quantity of telangiectasia with disease characteristics in SSc, and the agreement between patient- and physician-reported quantification of telangiectasia. METHODS: A retrospective analysis of a large, cross-sectional international SSc-related vasculopathy study was undertaken, including clinician and patient assessments of telangiectasia counts (0, 1-6, 7-15 or >15) in the face, forearms and hands. Relationships between telangiectasia count at each site, demographics and clinical features including calcinosis, digital ulceration (DU) and pulmonary arterial hypertension (PAH) were examined using proportional odds logistic regression. A binary logistic regression model examined the value of telangiectasia counts on the accuracy of identifying co-existent PAH. Concordance between clinician- and patient-reported telangiectasia counts at each anatomical site was tested. RESULTS: Higher telangiectasia counts over the face and hands were associated with higher prevalence of calcinosis, DU and PAH in univariate analysis (P < 0.001-0.003). In multivariate analysis, the presence of PAH, DU and calcinosis were associated with higher facial telangiectasia (P < 0.05). The logistic regression model for the detection of PAH was enhanced with inclusion of telangiectasia count and site (area under the precision recall curve 0.824-0.875). Strength of agreement between clinician- and patient-reported telangiectasia counts were moderate for face and forearms (kappa 0.648 and 0.605 respectively, P < 0.001) and relatively weaker for hands (kappa 0.584, P < 0.001). CONCLUSION: The number of telangiectasia might be considered a complementary biomarker to the presence of vascular complications of SSc including PAH, DU and calcinosis. The anatomical regions and level of instruction provided for patient-reported count of telangiectasia need further optimization to be considered reliable and feasible. In addition, future work should consider correlations with nailfold capillaroscopic patterns. CI - © The Author(s) 2026. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Wells, Matthew AU - Wells M AUID- ORCID: 0000-0002-8683-3358 AD - Department of Clinical Specialties and Related Sciences, University of Bristol, Bristol, UK. AD - Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK. AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Smith, Theresa AU - Smith T AD - Department of Mathematical Sciences, University of Bath, Bath, UK. FAU - Domsic, Robyn T AU - Domsic RT AUID- ORCID: 0000-0002-2765-0922 AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Anilkumar, Aishwarya AU - Anilkumar A AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Vanderbilt University, Nashville, TN, USA. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - National Institute for Health and Care Research (NIHR), Manchester Biomedical Research Centre, Manchester, UK. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Shah, Ami A AU - Shah AA AD - Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan Scleroderma Program, Ann Arbor, MI, USA. AD - Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. FAU - Barratt, Shaney L AU - Barratt SL AUID- ORCID: 0000-0003-3067-7349 AD - Department of Clinical Specialties and Related Sciences, University of Bristol, Bristol, UK. AD - Academic Respiratory Unit, Department of Clinical Sciences, University of Bristol, Bristol, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Clinical Specialties and Related Sciences, University of Bristol, Bristol, UK. AD - Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK. AD - Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, Bristol, UK. LA - eng GR - W81XWH-18-1-0602/US Department of Defence/ GR - Scleroderma Clinical Trials Consortium/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM OTO - NOTNLM OT - Raynaud’s syndrome OT - biomarkers OT - cardiovascular OT - quality of life OT - rare diseases OT - respiratory OT - scleroderma and related disorders OT - translational studies EDAT- 2026/05/22 19:21 MHDA- 2026/05/22 19:21 CRDT- 2026/05/22 16:53 PHST- 2025/12/15 00:00 [received] PHST- 2026/03/26 00:00 [accepted] PHST- 2026/05/22 19:21 [pubmed] PHST- 2026/05/22 19:21 [medline] PHST- 2026/05/22 16:53 [entrez] AID - 8690969 [pii] AID - 10.1093/rheumatology/keag267 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 Jun 3;65(6):keag267. doi: 10.1093/rheumatology/keag267. PMID- 38983669 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240711 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 7 DP - 2024 Jul TI - Systemic Sclerosis With a Normotensive Scleroderma Renal Crisis: A Diagnostic Dilemma. PG - e64167 LID - 10.7759/cureus.64167 [doi] LID - e64167 AB - Systemic sclerosis (SSc), also called scleroderma, is an auto-immune rheumatic disease that is characterized by fibrosis of the skin and internal organs and vasculopathy. Three of the severe manifestations of the disease include a scleroderma renal crisis (SRC), pulmonary arterial hypertension, and digital ulceration. Vascular manifestations like Raynaud's phenomenon are an almost universal symptom in patients with SSc and are often the earliest manifestation of the disease. An SRC occurs in approximately 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. However, about 10% of SRC cases present with normal blood pressure or a normotensive renal crisis. A 65-year-old man with a history of peripheral vascular disease and newly diagnosed heart failure presented to the emergency department on account of progressive discoloration of the left big toe and intermittent confusion. Initially, he was noted to be hemodynamically stable, with bluish discoloration of his left lower extremity and left big toe, which was tender to palpation with palpable distal pulses. His left toe progressively became dusky and gangrenous, necessitating ray amputation by vascular surgery. His hospital course was further complicated by worsening acute kidney injury, requiring initiation of hemodialysis, and progressive hypoxia with the transition from room air to high-flow oxygen. As part of his workup for acute kidney injury (AKI), his antinuclear antibody (ANA) was found to be positive, with high titers, as well as elevated SCl-70 IgG. Despite the initiation of hemodialysis, and post-surgical revision, he continued to deteriorate. His family opted for comfort care measures, and he died a few days later. Although SSc is a rare disease, it is associated with significant morbidity and has one of the highest mortality rates among connective tissue diseases. SSc can present with heterogeneous manifestations, mimicking several isolated organ-specific conditions. This makes the diagnosis challenging, especially early in the course of the disease. A high index of suspicion, especially in the setting of rapidly progressing multi-organ involvement without a clear cause, should prompt further evaluation of systemic sclerosis. CI - Copyright © 2024, Atencah et al. FAU - Atencah, Stanley E AU - Atencah SE AD - Internal Medicine, Piedmont Athens Regional Medical Center, Athens, USA. FAU - Robertson, Raheem AU - Robertson R AD - Internal Medicine, Piedmont Athens Regional Medical Center, Athens, USA. FAU - Ukoha, Nkechi AU - Ukoha N AD - Internal Medicine, Piedmont Athens Regional Medical Center, Athens, USA. FAU - Idolor, Osahon N AU - Idolor ON AD - Internal Medicine, Piedmont Athens Regional Medical Center, Athens, USA. FAU - Pippim, James AU - Pippim J AD - Pulmonology, Piedmont Athens Regional Medical Center, Athens, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240709 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11232918 OTO - NOTNLM OT - clinical rheumatology OT - interstitial lung disease OT - renal crisis OT - scleroderma OT - systemic sclerosis COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/07/10 06:41 MHDA- 2024/07/10 06:42 PMCR- 2024/07/09 CRDT- 2024/07/10 04:41 PHST- 2024/07/09 00:00 [accepted] PHST- 2024/07/10 06:42 [medline] PHST- 2024/07/10 06:41 [pubmed] PHST- 2024/07/10 04:41 [entrez] PHST- 2024/07/09 00:00 [pmc-release] AID - 10.7759/cureus.64167 [doi] PST - epublish SO - Cureus. 2024 Jul 9;16(7):e64167. doi: 10.7759/cureus.64167. eCollection 2024 Jul. PMID- 30498759 OWN - NLM STAT- MEDLINE DCOM- 20190308 LR - 20260127 IS - 2314-6141 (Electronic) IS - 2314-6133 (Print) VI - 2018 DP - 2018 TI - Effect of Genetic and Laboratory Findings on Clinical Course of Antisynthetase Syndrome in a Hungarian Cohort. PG - 6416378 LID - 10.1155/2018/6416378 [doi] LID - 6416378 AB - The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud's phenomenon, 43% fever, 33% mechanic's hand, and 12% dysphagia. We could detect significant correlation between anti-Jo-1 titer and the CK and CRP levels at disease onset and during disease course. HLA DRB1⁎03 positivity was present in 68.96% of patients, where the CK level at diagnosis was significantly lower compared to the HLA DRB1⁎03 negative patients. HLA DQA1⁎0501-DQB1⁎0201 haplotype was found in 58.62% of patients, but no significant correlation was found regarding any clinical or laboratory features. Higher CRP, ESR level, RF positivity, and the presence of fever or vasculitic skin lesions at the time of diagnosis indicated a higher steroid demand and the administration of higher number of immunosuppressants during the follow-up within anti-Jo-1 positive patients. The organ involvement of the disease was not different in HLA-DRB1⁎0301 positive or negative patients who were positive to the anti-Jo-1 antibody; however, initial CK level was lower in HLA-DRB1⁎0301 positive patients. Distinct laboratory and clinical parameters at diagnosis could be considered as prognostic markers. FAU - Szabó, Katalin AU - Szabó K AUID- ORCID: 0000-0002-0617-1703 AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Bodoki, Levente AU - Bodoki L AUID- ORCID: 0000-0002-4934-6032 AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Nagy-Vincze, Melinda AU - Nagy-Vincze M AUID- ORCID: 0000-0003-0316-3828 AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Vincze, Anett AU - Vincze A AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Zilahi, Erika AU - Zilahi E AUID- ORCID: 0000-0002-7376-8921 AD - University of Debrecen, Faculty of Medicine, Department of Laboratory Medicine, Nagyerdei krt. 98, 4032 Debrecen, Hungary. FAU - Szodoray, Peter AU - Szodoray P AD - Institute of Immunology, Rikshospitalet, Oslo University Hospital, Sognsvannsveien 20, 0372 Oslo, Norway. FAU - Dankó, Katalin AU - Dankó K AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Griger, Zoltán AU - Griger Z AUID- ORCID: 0000-0002-1371-7911 AD - University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. LA - eng PT - Journal Article DEP - 20181025 PL - United States TA - Biomed Res Int JT - BioMed research international JID - 101600173 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 9007-41-4 (C-Reactive Protein) RN - EC 2.7.3.2 (Creatine Kinase) RN - X4W7ZR7023 (Methylprednisolone) RN - 0 (Ribonucleoproteins) RN - 0 (SS-A Antigen) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Autoantibodies MH - C-Reactive Protein/metabolism MH - Cohort Studies MH - Creatine Kinase/blood MH - *Disease Progression MH - Dose-Response Relationship, Drug MH - Female MH - Humans MH - Hungary MH - Male MH - Methylprednisolone/therapeutic use MH - Myositis/blood/drug therapy/*genetics/*pathology MH - Ribonucleoproteins/blood MH - SS-A Antigen PMC - PMC6222225 EDAT- 2018/12/01 06:00 MHDA- 2019/03/09 06:00 PMCR- 2018/10/25 CRDT- 2018/12/01 06:00 PHST- 2018/03/17 00:00 [received] PHST- 2018/08/08 00:00 [revised] PHST- 2018/09/14 00:00 [accepted] PHST- 2018/12/01 06:00 [entrez] PHST- 2018/12/01 06:00 [pubmed] PHST- 2019/03/09 06:00 [medline] PHST- 2018/10/25 00:00 [pmc-release] AID - 10.1155/2018/6416378 [doi] PST - epublish SO - Biomed Res Int. 2018 Oct 25;2018:6416378. doi: 10.1155/2018/6416378. eCollection 2018. PMID- 23334373 OWN - NLM STAT- MEDLINE DCOM- 20140130 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 7 DP - 2013 Jul TI - Pulmonary hypertension in rheumatic diseases: epidemiology and pathogenesis. PG - 1655-67 LID - 10.1007/s00296-012-2659-y [doi] AB - The focus of this review is to increase awareness of pulmonary arterial hypertension (PAH) in patients with rheumatic diseases. Epidemiology and pathogenesis of PAH in rheumatic diseases is reviewed, with recommendations for early screening and diagnosis and suggestion of possible role of immunosuppressive therapy in treatment for PAH in rheumatic diseases. A MEDLINE search for articles published between January 1970 and June 2012 was conducted using the following keywords: pulmonary hypertension, scleroderma, systemic sclerosis, pulmonary arterial hypertension, connective tissues disease, systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis, Sjogren's syndrome, vasculitis, sarcoidosis, inflammatory myopathies, dermatomyositis, ankylosing spondylitis, spondyloarthropathies, diagnosis and treatment. Pathogenesis and disease burden of PAH in rheumatic diseases was highlighted, with emphasis on early consideration and workup of PAH. Screening recommendations and treatment were touched upon. PAH is most commonly seen in systemic sclerosis and may be seen in isolation or in association with interstitial lung disease. Several pathophysiologic processes have been identified including an obliterative vasculopathy, veno-occlusive disease, formation of microthrombi and pulmonary fibrosis. PAH in systemic lupus erythematosus is associated with higher prevalence of antiphospholipid and anticardiolipin antibodies and the presence of Raynaud's phenomenon. Endothelial proliferation with vascular remodeling, abnormal coagulation with thrombus formation and immune-mediated vasculopathy are the postulated mechanisms. Improvement with immunosuppressive medications has been reported. Pulmonary fibrosis, extrinsic compression of pulmonary arteries and granulomatous vasculitis have been reported in patients with sarcoidosis. Intimal and medial hyperplasia with luminal narrowing has been observed in Sjogren's syndrome, mixed connective tissue disease and inflammatory myopathies. Pulmonary arterial hypertension (PAH) associated with rheumatic diseases carries a particularly grim prognosis with faster progression of disease and poor response to therapy. Though largely associated with systemic sclerosis, it is being increasingly recognized in other rheumatic diseases. An underlying inflammatory component may explain the poor response to therapy in patients with rheumatic diseases and is a rationale for consideration of immunosuppressive therapy in conjunction with vasodilator therapy in treatment for PAH. Further studies identifying pathogenetic pathways and possible targets of therapy, especially the role of immunomodulatory medications, are warranted. FAU - Shahane, Anupama AU - Shahane A AD - Division of Rheumatology, University of Pennsylvania, 8 Penn Tower, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA. Anupama.Shahane@uphs.upenn.edu LA - eng PT - Journal Article PT - Review DEP - 20130119 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antihypertensive Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Animals MH - Antihypertensive Agents/therapeutic use MH - Familial Primary Pulmonary Hypertension MH - Humans MH - Hypertension, Pulmonary/diagnosis/drug therapy/*epidemiology MH - Immunosuppressive Agents/therapeutic use MH - Rheumatic Diseases/diagnosis/drug therapy/*epidemiology MH - Risk Factors MH - Treatment Outcome MH - Vasodilator Agents/therapeutic use EDAT- 2013/01/22 06:00 MHDA- 2014/01/31 06:00 CRDT- 2013/01/22 06:00 PHST- 2012/05/08 00:00 [received] PHST- 2012/12/28 00:00 [accepted] PHST- 2013/01/22 06:00 [entrez] PHST- 2013/01/22 06:00 [pubmed] PHST- 2014/01/31 06:00 [medline] AID - 10.1007/s00296-012-2659-y [doi] PST - ppublish SO - Rheumatol Int. 2013 Jul;33(7):1655-67. doi: 10.1007/s00296-012-2659-y. Epub 2013 Jan 19. PMID- 17825708 OWN - NLM STAT- MEDLINE DCOM- 20070928 LR - 20231024 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 50 IP - 11 DP - 2007 Sep 11 TI - Alcohol and cardiovascular health: the razor-sharp double-edged sword. PG - 1009-14 AB - An extensive body of data shows concordant J-shaped associations between alcohol intake and a variety of adverse health outcomes, including coronary heart disease, diabetes, hypertension, congestive heart failure, stroke, dementia, Raynaud's phenomenon, and all-cause mortality. Light to moderate alcohol consumption (up to 1 drink daily for women and 1 or 2 drinks daily for men) is associated with cardioprotective benefits, whereas increasingly excessive consumption results in proportional worsening of outcomes. Alcohol consumption confers cardiovascular protection predominately through improvements in insulin sensitivity and high-density lipoprotein cholesterol. The ethanol itself, rather than specific components of various alcoholic beverages, appears to be the major factor in conferring health benefits. Low-dose daily alcohol is associated with better health than less frequent consumption. Binge drinking, even among otherwise light drinkers, increases cardiovascular events and mortality. Alcohol should not be universally prescribed for health enhancement to nondrinking individuals owing to the lack of randomized outcome data and the potential for problem drinking. FAU - O'Keefe, James H AU - O'Keefe JH AD - Mid America Heart Institute, University of Missouri, Kansas City, Missouri, USA. jhokeefe@cc-pc.com FAU - Bybee, Kevin A AU - Bybee KA FAU - Lavie, Carl J AU - Lavie CJ LA - eng PT - Journal Article PT - Review PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 RN - 0 (Central Nervous System Depressants) RN - 3K9958V90M (Ethanol) SB - IM MH - Alcohol Drinking/*adverse effects MH - Cardiovascular Diseases/*etiology/*prevention & control MH - Cardiovascular System/drug effects MH - Central Nervous System Depressants/pharmacology MH - Ethanol/pharmacology MH - Glucose Metabolism Disorders/prevention & control MH - Humans MH - Risk Assessment RF - 52 EDAT- 2007/09/11 09:00 MHDA- 2007/09/29 09:00 CRDT- 2007/09/11 09:00 PHST- 2007/02/14 00:00 [received] PHST- 2007/04/19 00:00 [revised] PHST- 2007/04/30 00:00 [accepted] PHST- 2007/09/11 09:00 [pubmed] PHST- 2007/09/29 09:00 [medline] PHST- 2007/09/11 09:00 [entrez] AID - S0735-1097(07)02007-4 [pii] AID - 10.1016/j.jacc.2007.04.089 [doi] PST - ppublish SO - J Am Coll Cardiol. 2007 Sep 11;50(11):1009-14. doi: 10.1016/j.jacc.2007.04.089. PMID- 40124434 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250325 IS - 1108-7471 (Print) IS - 1792-7463 (Electronic) IS - 1108-7471 (Linking) VI - 38 IP - 2 DP - 2025 Mar-Apr TI - Use of nailfold capillaroscopy for the early diagnosis of systemic sclerosis in patients with primary biliary cholangitis. PG - 187-194 LID - 10.20524/aog.2025.0949 [doi] AB - BACKGROUND: Primary biliary cholangitis (PBC) is a cholestatic autoimmune disease and is often associated with systemic sclerosis (SSc). The prevalence of SSc in PBC patients ranges from 1-22% and the diagnosis is often delayed. The aim of this study was to evaluate the role of nailfold capillaroscopy (NFC) for early SSc diagnosis in PBC patients. METHODS: In this monocentric, cross-sectional study, NFC was performed in 56 PBC patients. Raynaud's phenomenon (RP) was assessed in each patient. Patients with major NFC abnormalities and those with a scleroderma pattern were screened for SSc-specific antibodies. The SSc diagnosis was established using the 2013 American College of Rheumatology and European League Against Rheumatism (ACR/AULAR) and Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) criteria. RESULTS: NFC abnormalities were found in 31 patients (55%): 11 (20%) presented minor abnormalities, 17 (30%) had major abnormalities and 3 (5%) presented a scleroderma pattern. RP was found in 12 patients. Two patients with a scleroderma pattern were newly diagnosed with SSc. All patients newly diagnosed with SSc presented RP. No patient without RP was diagnosed with SSc. CONCLUSIONS: Performing NFC in PBC patients can help anticipate the SSc diagnosis. RP should always be checked in PBC patients and should be an indication to perform NFC for early SSc diagnosis. A diagnostic algorithm is proposed. CI - Copyright: © 2025 Hellenic Society of Gastroenterology. FAU - Crescenzi, Daniele AU - Crescenzi D AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Balducci, Daniele AU - Balducci D AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Mazzetti, Marta AU - Mazzetti M AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Menghini, Denise AU - Menghini D AD - Department of Internal Medicine, Università Politecnica delle Marche, Ancona, Italy (Denise Menghini, Chiara Gelardi, Veronica Pedini, Cristina Mezzanotte, Maria Giovanna Danieli). FAU - Gelardi, Chiara AU - Gelardi C AD - Department of Internal Medicine, Università Politecnica delle Marche, Ancona, Italy (Denise Menghini, Chiara Gelardi, Veronica Pedini, Cristina Mezzanotte, Maria Giovanna Danieli). FAU - Pedini, Veronica AU - Pedini V AD - Department of Internal Medicine, Università Politecnica delle Marche, Ancona, Italy (Denise Menghini, Chiara Gelardi, Veronica Pedini, Cristina Mezzanotte, Maria Giovanna Danieli). FAU - Mezzanotte, Cristina AU - Mezzanotte C AD - Department of Internal Medicine, Università Politecnica delle Marche, Ancona, Italy (Denise Menghini, Chiara Gelardi, Veronica Pedini, Cristina Mezzanotte, Maria Giovanna Danieli). FAU - Tarantino, Giuseppe AU - Tarantino G AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Benedetti, Antonio AU - Benedetti A AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Danieli, Maria Giovanna AU - Danieli MG AD - Department of Internal Medicine, Università Politecnica delle Marche, Ancona, Italy (Denise Menghini, Chiara Gelardi, Veronica Pedini, Cristina Mezzanotte, Maria Giovanna Danieli). FAU - Marzioni, Marco AU - Marzioni M AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). FAU - Maroni, Luca AU - Maroni L AD - Department of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy (Daniele Crescenzi, Daniele Balducci, Marta Mazzetti, Giuseppe Tarantino, Antonio Benedetti, Marco Marzioni, Luca Maroni). LA - eng PT - Journal Article DEP - 20250226 PL - Greece TA - Ann Gastroenterol JT - Annals of gastroenterology JID - 101121847 PMC - PMC11928902 OTO - NOTNLM OT - Keywords Primary biliary cholangitis OT - nailfold capillaroscopy OT - systemic sclerosis COIS- Conflict of Interest: None EDAT- 2025/03/24 12:45 MHDA- 2025/03/24 12:46 PMCR- 2025/03/01 CRDT- 2025/03/24 06:05 PHST- 2024/09/09 00:00 [received] PHST- 2024/12/14 00:00 [accepted] PHST- 2025/03/24 12:46 [medline] PHST- 2025/03/24 12:45 [pubmed] PHST- 2025/03/24 06:05 [entrez] PHST- 2025/03/01 00:00 [pmc-release] AID - AnnGastroenterol-38-187 [pii] AID - 10.20524/aog.2025.0949 [doi] PST - ppublish SO - Ann Gastroenterol. 2025 Mar-Apr;38(2):187-194. doi: 10.20524/aog.2025.0949. Epub 2025 Feb 26. PMID- 31824645 OWN - NLM STAT- MEDLINE DCOM- 20200122 LR - 20200122 IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 8 DP - 2019 TI - Cutaneous manifestations of dermatomyositis characterized by myositis-specific autoantibodies. LID - F1000 Faculty Rev-1951 [pii] LID - 10.12688/f1000research.20646.1 [doi] AB - Dermatomyositis (DM) is an inflammatory myopathy with characteristic skin manifestations, the pathologies of which are considered autoimmune diseases. DM is a heterogeneous disorder with various phenotypes, including myositis, dermatitis, and interstitial lung disease (ILD). Recently identified myositis-specific autoantibodies have been associated with distinct clinical features. For example, anti-melanoma differentiation-associated protein 5 antibodies have a high specificity for clinically amyopathic DM presenting rapidly progressive ILD. Furthermore, anti-transcriptional intermediary factor 1γ antibodies found in patients with juvenile and adult DM are closely correlated with malignancies, especially in elderly patients. Finally, patients with anti-aminoacyl-transfer RNA synthetase antibodies share characteristic clinical symptoms, including myositis, ILD, arthritis/arthralgia, Raynaud's phenomenon, and fever; thus, the term "anti-synthetase syndrome" is also used. With a focus on the characteristic cutaneous manifestations in each subgroup classified according to myositis-specific autoantibodies, we introduce the findings of previous reports, including our recent analysis indicating that skin eruptions can be histopathologically classified into myositis-specific autoantibody-associated subgroups and used to determine the systemic pathologies of the different types of antibody-associated DM. CI - Copyright: © 2019 Okiyama N and Fujimoto M. FAU - Okiyama, Naoko AU - Okiyama N AUID- ORCID: 0000-0002-5398-0773 AD - Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, 305-8575, Japan. FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Integrated Medicine, Graduate School of Medicine, Osaka University, Suita, Osaka, 565-0871, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20191121 PL - England TA - F1000Res JT - F1000Research JID - 101594320 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Amino Acyl-tRNA Synthetases MH - Autoantibodies MH - *Dermatomyositis MH - Humans MH - *Lung Diseases, Interstitial MH - *Myositis PMC - PMC6880256 OTO - NOTNLM OT - dermatomyositis OT - myositis-specific autoantibodies COIS- No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed. EDAT- 2019/12/12 06:00 MHDA- 2020/01/23 06:00 PMCR- 2019/11/21 CRDT- 2019/12/12 06:00 PHST- 2019/11/20 00:00 [accepted] PHST- 2019/12/12 06:00 [entrez] PHST- 2019/12/12 06:00 [pubmed] PHST- 2020/01/23 06:00 [medline] PHST- 2019/11/21 00:00 [pmc-release] AID - F1000 Faculty Rev-1951 [pii] AID - 10.12688/f1000research.20646.1 [doi] PST - epublish SO - F1000Res. 2019 Nov 21;8:F1000 Faculty Rev-1951. doi: 10.12688/f1000research.20646.1. eCollection 2019. PMID- 38191065 OWN - NLM STAT- MEDLINE DCOM- 20240512 LR - 20250711 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 23 IP - 4 DP - 2024 Apr TI - Childhood mixed connective tissue disease at disease onset: Evidence from a systematic review. PG - 103513 LID - S1568-9972(23)00247-1 [pii] LID - 10.1016/j.autrev.2023.103513 [doi] AB - OBJECTIVE: Childhood Mixed Connective Tissue Disease (cMCTD) is the rarest pediatric connective tissue disease that includes features of systemic lupus erythematosus, polymyositis/dermatomyositis, juvenile idiopathic arthritis, and systemic sclerosis, identified by Sharp in 1972 and whose diagnosis remains challenging. This systematic review aims to identify clinical features at the onset of cMCTD and manifestations not currently included into the available diagnostic criteria. METHODS: A systematic literature review was performed in accordance with PRISMA guidelines 2020 using bibliographic databases: MEDLINE via PubMed and EMBASE. ELIGIBILITY CRITERIA: patients diagnosed with MCTD with onset before 18 years. STUDIES INCLUDED: registries, retrospective and prospective cohort studies, case series and reports with analysis of data on signs and symptoms of presentation. RESULTS: 39 articles were included (215 subjects, 82.5% female), mean age of 141 months (± 41 months DS, range 2.5-204). The most used criteria for the diagnosis of MCTD were the Kasukawa criteria (54.5%). The clinical manifestations described at onset were Raynaud's phenomenon (69.7%), arthritis (60.9%), muscular involvement (53.5%), dermatological signs (39.5%), swollen fingers or hands (29.3%), arthralgias (25.6%), fever (22.3%), lung involvement (14.4%), sclerodactily (13.5%), lymphadenopathy (10.7%) serositis (10.2%), esophageal involvement (6.9%), nervous system involvement (6.9%), xeroftalmia (3.7%), xerostomia (3.7%), hepatosplenomegaly (2.8%), cardiac involvement (2.8%), hepatitis (2.3%), parotiditis (2.3%), Hashimoto's thyroiditis (0.9%), ocular involvement (0.9%). CONCLUSIONS: The data from this systematic review suggest great heterogeneity of the clinical presentation of cMCTD for which there are no validated diagnostic criteria that may suggest a new diagnostic approach to allow earlier or more accurate diagnosis in the future. CI - Copyright © 2023. Published by Elsevier B.V. FAU - Terminiello, Alberto AU - Terminiello A AD - Department of Health Sciences, University of Florence, Florence, Italy. Electronic address: alberto.terminiello@unifi.it. FAU - Marrani, Edoardo AU - Marrani E AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy. Electronic address: edoardo.marrani@meyer.it. FAU - Pagnini, Ilaria AU - Pagnini I AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy. FAU - Maccora, Ilaria AU - Maccora I AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy; NEUROFARBA Department, University of Florence, Florence, Italy. FAU - Maniscalco, Valerio AU - Maniscalco V AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy. FAU - Rumeileh, Sarah Abu AU - Rumeileh SA AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy. FAU - Mastrolia, Maria Vincenza AU - Mastrolia MV AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy. FAU - Simonini, Gabriele AU - Simonini G AD - Rheumatology Unit, ERN-ReCONNET Center, Meyer Children's Hospital IRCCS, Florence, Italy; NEUROFARBA Department, University of Florence, Florence, Italy. LA - eng PT - Journal Article PT - Systematic Review DEP - 20240106 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Adolescent MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Male MH - Age of Onset MH - *Mixed Connective Tissue Disease/diagnosis OTO - NOTNLM OT - Dermatomyositis OT - Juvenile idiopathic arthritis OT - Lupus erythematosus, systemic OT - Mixed connective tissue disease OT - Pediatric onset OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/01/09 00:42 MHDA- 2024/05/13 00:42 CRDT- 2024/01/08 19:17 PHST- 2023/12/18 00:00 [received] PHST- 2023/12/30 00:00 [accepted] PHST- 2024/05/13 00:42 [medline] PHST- 2024/01/09 00:42 [pubmed] PHST- 2024/01/08 19:17 [entrez] AID - S1568-9972(23)00247-1 [pii] AID - 10.1016/j.autrev.2023.103513 [doi] PST - ppublish SO - Autoimmun Rev. 2024 Apr;23(4):103513. doi: 10.1016/j.autrev.2023.103513. Epub 2024 Jan 6. PMID- 42255726 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260608 LR - 20260608 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 5 DP - 2026 May TI - Anti-Ku Antibodies in a Patient With a Polymyositis-Systemic Sclerosis Overlap Syndrome in Association With Autoimmune Hepatitis. PG - e108420 LID - 10.7759/cureus.108420 [doi] LID - e108420 AB - Overlap syndromes are well-described but rare clinical entities characterized by the co-presence of two or more connective tissue diseases. The combination of systemic sclerosis and polymyositis overlapping with autoimmune hepatitis (AIH) is particularly rare, as discussed in this case report. A 76-year-old woman with a recent history of pleuro-pericarditis presented with painful Raynaud's phenomenon, lethargy, jaundice, myalgias, and weight loss. Blood tests revealed a positive antinuclear antibody and anti-Ku antibody and raised creatine kinase, together with evidence of myositis and interstitial lung disease on imaging, leading to a diagnosis of polymyositis-systemic sclerosis overlap syndrome. Raised liver transaminases, immunological tests, and liver biopsy were consistent with AIH. She was managed with corticosteroids and mycophenolate mofetil. This is only the second instance in which AIH has been reported in the context of a positive anti-Ku antibody. We discussed the complex and rare nature of this condition and the various aspects of its management. CI - Copyright © 2026, Zubair et al. FAU - Zubair, Muhammad AU - Zubair M AD - Rheumatology, Worthing Hospital, University Hospital Sussex NHS Foundation Trust, Worthing, GBR. FAU - Wazir, Maha AU - Wazir M AD - Nephrology, The Ohio State University Wexner Medical Center, Columbus, USA. FAU - Ullah, Farhan AU - Ullah F AD - Nephrology, Ochsner Medical Center, Jefferson, USA. FAU - Thomson, Sam J AU - Thomson SJ AD - Gastroenterology and Hepatology, Worthing Hospital, University Hospital Sussex NHS Foundation Trust, Worthing, GBR. FAU - Hepburn, Alistair AU - Hepburn A AD - Rheumatology, Worthing Hospital, University Hospital Sussex NHS Foundation Trust, Worthing, GBR. LA - eng PT - Case Reports PT - Journal Article DEP - 20260507 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13242160 OTO - NOTNLM OT - anti-ku antibodies OT - autoimmune hepatitis OT - autoimmune overlap syndrome OT - polymyositis with vasculitis OT - polymyositis-systemic sclerosis overlap syndrome COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/06/08 12:40 MHDA- 2026/06/08 12:41 PMCR- 2026/05/07 CRDT- 2026/06/08 07:35 PHST- 2026/05/07 00:00 [accepted] PHST- 2026/06/08 12:41 [medline] PHST- 2026/06/08 12:40 [pubmed] PHST- 2026/06/08 07:35 [entrez] PHST- 2026/05/07 00:00 [pmc-release] AID - 10.7759/cureus.108420 [doi] PST - epublish SO - Cureus. 2026 May 7;18(5):e108420. doi: 10.7759/cureus.108420. eCollection 2026 May. PMID- 38552324 OWN - NLM STAT- MEDLINE DCOM- 20250304 LR - 20260512 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 3 DP - 2025 Mar 1 TI - Efficacy of methylprednisolone in very early systemic sclerosis: results of the 'Hit Hard and Early' randomized controlled trial. PG - 1261-1269 LID - 10.1093/rheumatology/keae156 [doi] AB - OBJECTIVE: We hypothesized that glucocorticoids would induce remission in very early systemic sclerosis (SSc) patients by inhibition of inflammation driving the disease. We examined the efficacy and safety of methylprednisolone in very early SSc. METHODS: In this trial adults with puffy fingers for less than 3 years, specific auto-antibodies and meeting the Very Early Diagnosis of Systemic Sclerosis criteria were randomly assigned (2:1) to methylprednisolone 1000 mg i.v. or placebo for three consecutive days three times with monthly intervals. The primary end point was nailfold capillary density at week 12. Capillary density at 52 weeks, number of megacapillaries and patient-reported outcomes were secondary outcomes. In addition, we assessed disease progression and lung function decline over 52 weeks. We used linear regression analyses adjusted for baseline values and stratification variables to estimate differences between groups. RESULTS: Between February 2017 and February 2021, 87 patients were screened, of whom 30 (70% female, median [interquartile range, IQR] age 52.9 [40.8-60.8] years, median [IQR] disease duration 11.4 [4.6-18.6] months) were randomly assigned to methylprednisolone (n = 21) or placebo (n = 9). We found no difference in nailfold capillary density at 12 weeks (-0.5 [95% CI: -1.1, 0.2]) nor in any of the secondary outcomes. Eleven (37%) patients showed disease progression during 1 year follow-up, and seven (23%) patients had a relevant pulmonary function decline. No serious adverse events were reported. CONCLUSION: No clinically relevant effect of short-term methylprednisolone in patients with very early SSc was observed. A substantial proportion of patients showed disease progression. TRIAL REGISTRATION: clinicaltrials.gov, NCT03059979. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Kersten, Brigit E AU - Kersten BE AUID- ORCID: 0000-0002-0074-5286 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Lemmers, Jacqueline M J AU - Lemmers JMJ AUID- ORCID: 0000-0003-1183-5049 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Vanhaecke, Amber AU - Vanhaecke A AUID- ORCID: 0000-0002-1989-7060 AD - Department of Rheumatology, University Hospital Gent, Gent, Belgium. FAU - Velauthapillai, Arthiha AU - Velauthapillai A AUID- ORCID: 0009-0006-0700-7573 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - van den Hombergh, Wieneke M T AU - van den Hombergh WMT AUID- ORCID: 0000-0002-7706-7348 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - van den Hoogen, Frank H J AU - van den Hoogen FHJ AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Rheumatology, University Hospital Gent, Gent, Belgium. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. LA - eng SI - ClinicalTrials.gov/NCT03059979 GR - Boehringer Ingelheim/ GR - Janssen Pharmaceutical Companies of Johnson & Johnson and Ferrer/ PT - Clinical Trial, Phase I PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Glucocorticoids) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Capillaries MH - Disease Progression MH - Double-Blind Method MH - *Glucocorticoids/therapeutic use/administration & dosage MH - *Methylprednisolone/therapeutic use/administration & dosage MH - Nails/blood supply MH - *Scleroderma, Systemic/drug therapy/physiopathology MH - Treatment Outcome PMC - PMC11879336 OTO - NOTNLM OT - Raynaud’s syndrome OT - immunosuppressants OT - inflammation OT - interventional studies OT - scleroderma and related disorders EDAT- 2024/03/30 11:45 MHDA- 2025/03/05 04:53 PMCR- 2024/03/29 CRDT- 2024/03/29 19:05 PHST- 2023/09/14 00:00 [received] PHST- 2024/02/24 00:00 [accepted] PHST- 2025/03/05 04:53 [medline] PHST- 2024/03/30 11:45 [pubmed] PHST- 2024/03/29 19:05 [entrez] PHST- 2024/03/29 00:00 [pmc-release] AID - 7637683 [pii] AID - keae156 [pii] AID - 10.1093/rheumatology/keae156 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Mar 1;64(3):1261-1269. doi: 10.1093/rheumatology/keae156. PMID- 38478089 OWN - NLM STAT- In-Process LR - 20260609 IS - 1861-0692 (Electronic) IS - 1861-0684 (Linking) VI - 115 IP - 7 DP - 2026 Jul TI - Aortic regurgitation is associated with African American and Asian race, smoking, renal disease, and numerous autoimmune diseases in addition to traditional cardiovascular risk factors but has lower risk with alcohol intake. PG - 1061-1073 LID - 10.1007/s00392-024-02424-3 [doi] AB - BACKGROUND: Aortic regurgitation (AR) is associated with increasing age, rheumatic heart disease, and a bicuspid aortic valve, but its association with other comorbidities and race is less known. The purpose of this study was to investigate any association between AR and comorbid conditions in older adults above 40. METHOD: The large Nationwide Inpatient Sample database was utilized for our study using uni- and multivariate analysis. Data were extracted from available ICD-10 codes for the years of 2016-2020. RESULTS: The NIS data included 112,982,565 patients. A total of 660,730 were found to have AR. AR was found to be associated with male gender (OR 1.15, CI 1.14-1.16, P < 0.001), smoking (OR 1.04, CI 1.02-1.05, P < 0.001), hypertension (OR 1.65, CI 1.62-1.68, P < 0.001), hyperlipidemia (OR 1.36, CI 1.34-1.37, P < 0.001), chronic kidney disease (OR 1.22, CI 1.21-1.24, P < 0.001), antiphospholipid antibody syndrome (OR 1.56, CI 1.33-1.83, P < 0.001), rheumatoid arthritis (OR 1.1, CI 1.06-1.14, P < 0.001), scleroderma (OR 1.49, CI 1.31-1.7, P < 0.001), systemic connective tissue disorders (OR 1.32, CI 1.25-1.4, P < 0.001), Raynaud's syndrome (OR 1.62, CI 1.47-1.77, P < 0.001), and systemic lupus erythematosus (OR 1.44, CI 1.34-1.54, P < 0.001) in add to known bicuspid aortic valve. CONCLUSION: Using a very large database, we found new associations between AR and many comorbid conditions, including many inflammatory and chronic degenerative diseases in addition to the known risk factors. CI - © 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply. FAU - Timmerman, Brandon AU - Timmerman B AD - University of Arizona, College of Medicine, Phoenix, AZ, USA. FAU - Hashemzadeh, Mehrtash AU - Hashemzadeh M AD - University of Arizona, College of Medicine, Phoenix, AZ, USA. FAU - Movahed, Mohammad Reza AU - Movahed MR AUID- ORCID: 0000-0001-7492-0510 AD - University of Arizona, College of Medicine, Phoenix, AZ, USA. rmova@aol.com. AD - University of Arizona Sarver Heart Center, College of Medicine, 1501 N. Campbell Ave., Tucson, AZ, USA. rmova@aol.com. LA - eng PT - Journal Article DEP - 20240313 PL - Germany TA - Clin Res Cardiol JT - Clinical research in cardiology : official journal of the German Cardiac Society JID - 101264123 SB - IM OTO - NOTNLM OT - Aortic insufficiency OT - Aortic regurgitation OT - Aortic valve disease OT - Aortic valve insufficiency OT - Autoimmune disease OT - Diabetes OT - Hyperlipidemia OT - Hypertension OT - Obesity OT - Prevalence OT - Valve disease COIS- Declarations. Conflict of interest: The authors declare no competing interests. EDAT- 2024/03/13 18:46 MHDA- 2024/03/13 18:46 CRDT- 2024/03/13 12:16 PHST- 2023/12/04 00:00 [received] PHST- 2024/02/23 00:00 [accepted] PHST- 2024/03/13 18:46 [pubmed] PHST- 2024/03/13 18:46 [medline] PHST- 2024/03/13 12:16 [entrez] AID - 10.1007/s00392-024-02424-3 [pii] AID - 10.1007/s00392-024-02424-3 [doi] PST - ppublish SO - Clin Res Cardiol. 2026 Jul;115(7):1061-1073. doi: 10.1007/s00392-024-02424-3. Epub 2024 Mar 13. PMID- 40319350 OWN - NLM STAT- MEDLINE DCOM- 20250504 LR - 20250510 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 26 DP - 2025 May 4 TI - Refractory Pleuritis in a Patient with Silicosis, Systemic Sclerosis, and Sjögren's Syndrome: Considering the Potential Role of Adjuvant-Induced Autoimmunity. PG - e947856 LID - 10.12659/AJCR.947856 [doi] LID - e947856 AB - BACKGROUND Silicosis, which is caused by the inhalation of crystalline silica, is known to be associated with a variety of autoimmune diseases. Recently, a new pathogenesis called autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been reported, which occurs after exposure to substances with adjuvant activity, including silica, and shares clinical features observed in several autoimmune diseases. CASE REPORT A 65-year-old man with silicosis was admitted to our hospital due to fever and chronic fatigue. Symptoms such as sicca and Raynaud's phenomenon and pleural effusion appeared as new findings on admission. Examination led to a diagnosis of systemic scleroderma (SSc) and Sjögren's syndrome (SjS). Considering that SjS was the main cause of the disease, corticosteroid therapy was initiated. However, the patient's general condition deteriorated, leading finally to his death. Silica acts as an adjuvant, inducing chronic inflammatory cytokine release. Thus, prolonged exposure to silica can contribute to the development of autoimmune diseases such as SSc and SjS. In this case, the refractory pleuritis may have been related to the pathogenesis of ASIA. ASIA is difficult to manage if the causative adjuvant cannot be eliminated. CONCLUSIONS We described a case of newly diagnosed SSc and SjS with therapy-resistant pleuritis in a patient with silicosis. Silicosis complicated with corticosteroid-resistant autoimmune disease suggests that ASIA, an adjuvant disease, is involved in the pathogenesis. Therefore, not only SSc and SjS but also the pathogenesis of ASIA should be considered in such cases. Since adjuvant exposure is a causative factor in ASIA, avoiding such exposure is crucial. FAU - Morimoto, Julia AU - Morimoto J AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Suzuki, Yasuhito AU - Suzuki Y AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Togawa, Ryuichi AU - Togawa R AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Watanabe, Natsumi AU - Watanabe N AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Kumanaka, Takahiro AU - Kumanaka T AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Tanaka, Ryutaro AU - Tanaka R AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Kazama, Kentaro AU - Kazama K AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Saito, Koshi AU - Saito K AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Harigane, Rina AU - Harigane R AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Yamada, Ryuki AU - Yamada R AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Sato, Riko AU - Sato R AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Tomita, Hikaru AU - Tomita H AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Umeda, Takashi AU - Umeda T AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Rikimaru, Mami AU - Rikimaru M AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Sato, Yuki AU - Sato Y AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Minemura, Hiroyuki AU - Minemura H AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Nikaido, Takefumi AU - Nikaido T AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Saito, Junpei AU - Saito J AUID- ORCID: 0000-0002-9876-1160 AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Kanazawa, Kenya AU - Kanazawa K AUID- ORCID: 0000-0002-9574-0516 AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Wang, Xintao AU - Wang X AUID- ORCID: 0009-0009-7657-272X AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Tanino, Yoshinori AU - Tanino Y AUID- ORCID: 0000-0003-2384-601X AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. FAU - Shibata, Yoko AU - Shibata Y AUID- ORCID: 0000-0003-1794-0026 AD - Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20250504 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - Humans MH - Male MH - *Silicosis/complications MH - Aged MH - *Scleroderma, Systemic/complications/diagnosis MH - *Sjogren's Syndrome/complications/diagnosis MH - *Pleurisy/etiology/diagnosis MH - Fatal Outcome MH - *Autoimmunity PMC - PMC12060736 COIS- Conflict of interest: None declared EDAT- 2025/05/04 15:35 MHDA- 2025/05/04 15:36 PMCR- 2025/05/04 CRDT- 2025/05/04 01:22 PHST- 2025/05/04 15:36 [medline] PHST- 2025/05/04 15:35 [pubmed] PHST- 2025/05/04 01:22 [entrez] PHST- 2025/05/04 00:00 [pmc-release] AID - 947856 [pii] AID - 10.12659/AJCR.947856 [doi] PST - epublish SO - Am J Case Rep. 2025 May 4;26:e947856. doi: 10.12659/AJCR.947856. PMID- 37404175 OWN - NLM STAT- MEDLINE DCOM- 20230804 LR - 20230804 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 41 IP - 8 DP - 2023 Aug TI - Correlation between angiotensin-converting-enzyme 2 gene polymorphisms and systemic sclerosis. PG - 1652-1658 LID - 10.55563/clinexprheumatol/utmjnq [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a disease with cardiovascular impairment and polymorphisms of the gene coding of angiotensin-converting-enzyme 2 (ACE2) may account for its development. Three single nucleotide polymorphisms of ACE2 (C>G rs879922, G>A rs2285666 and A>G rs1978124) were found to increase the risk for development of arterial hypertension (AH) and cardiovascular (CVS) diseases in different ethnicities. We investigated associations of polymorphisms rs879922, rs2285666 and rs1978124 with the development of SSc. METHODS: Genomic DNA was isolated from whole blood. Restriction-fragment-length polymorphism was used for genotyping of rs1978124, while detection of rs879922 and rs2285666 was based on TaqMan SNP Genotyping Assay. Serum level of ACE2 was assayed with commercially available ELISA test. RESULTS: 81 SSc patients (60 women, 21 men) were enrolled. Allele C of rs879922 polymorphism was associated with significantly greater risk for development of AH (OR=2.5, p=0.018), but less frequent joint involvement. A strong tendency to earlier onset of Raynaud's phenomenon and SSc was seen in carriers of allele A of rs2285666 polymorphism. They had lower risk for development of any CVS disease (RR=0.4, p=0.051) and tendency to less frequent gastrointestinal involvement. Women with genotype AG of rs1978124 polymorphism had significantly more frequent digital tip ulcers and lower serum level of ACE2. CONCLUSIONS: Polymorphisms of ACE2 may account for the development of AH and CVS disorders in SSc patients. Strong tendencies to more frequent occurrence of disease specific characteristics distinct to macrovascular involvement will require further studies evaluating significance of ACE2 polymorphisms in SSc. FAU - Miziołek, Bartosz AU - Miziołek B AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. bmiziolek@gmail.com. FAU - Kruszniewska-Rajs, Celina AU - Kruszniewska-Rajs C AD - Department of Molecular Biology, Faculty of Pharmaceutical Sciences in Sosnowiec, Medical University of Silesia, Katowice, Poland. FAU - Gola, Joanna AU - Gola J AD - Department of Molecular Biology, Faculty of Pharmaceutical Sciences in Sosnowiec, Medical University of Silesia, Katowice, Poland. FAU - Bultrowicz, Monika AU - Bultrowicz M AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Miszczyk, Justyna AU - Miszczyk J AD - Institute of Nuclear Physics, Polish Academy of Sciences, Kraków, Poland. FAU - Pieczyrak, Robert AU - Pieczyrak R AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Frątczak, Aleksandra AU - Frątczak A AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Polak, Karina AU - Polak K AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Kucharz, Eugeniusz J AU - Kucharz EJ AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Bergler-Czop, Beata AU - Bergler-Czop B AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. LA - eng PT - Journal Article DEP - 20230704 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - EC 3.4.17.23 (Angiotensin-Converting Enzyme 2) RN - 0 (Angiotensins) RN - EC 3.4.17.23 (ACE2 protein, human) SB - IM MH - Female MH - Humans MH - Male MH - Angiotensin-Converting Enzyme 2/genetics MH - Angiotensins/genetics MH - *Cardiovascular Diseases MH - *Hypertension MH - Polymorphism, Single Nucleotide MH - *Scleroderma, Systemic/diagnosis/genetics EDAT- 2023/07/05 13:05 MHDA- 2023/08/04 06:43 CRDT- 2023/07/05 10:04 PHST- 2023/02/27 00:00 [received] PHST- 2023/05/02 00:00 [accepted] PHST- 2023/08/04 06:43 [medline] PHST- 2023/07/05 13:05 [pubmed] PHST- 2023/07/05 10:04 [entrez] AID - 19705 [pii] AID - 10.55563/clinexprheumatol/utmjnq [doi] PST - ppublish SO - Clin Exp Rheumatol. 2023 Aug;41(8):1652-1658. doi: 10.55563/clinexprheumatol/utmjnq. Epub 2023 Jul 4. PMID- 37337554 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230621 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 15 IP - 6 DP - 2023 Jun TI - A Case of Antisynthetase Syndrome in the Setting of SARS-Cov-2 Infection. PG - e40588 LID - 10.7759/cureus.40588 [doi] LID - e40588 AB - Antisynthetase syndrome is a complex autoimmune disorder, and one of the key criteria for diagnosis is the presence of myositis. Additionally, evidence of interstitial lung disease (ILD) is another important indicator for diagnosis; other clinical features associated with antisynthetase syndrome include arthritis, unexplained and persistent fever, Raynaud's phenomenon, and the presence of mechanic's hands. We report a case of a 36-year-old male who presented to the emergency department with shortness of breath and proximal muscle weakness in the setting of severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection, as his inflammatory markers were elevated and he exhibited features suspicious for antisynthetase syndrome, he was started on methylprednisolone 40 mg intravenously every eight hours, and a myositis panel was checked. In addition, a chest computed tomography (CT) exhibited ground-glass opacities which were compatible with coronavirus disease 2019 (COVID-19). A magnetic resonance image (MRI) of both thighs was done, revealing significant swelling and confirming the suspicion of myositis as his muscle strength in his lower extremities took significant time to improve. As days passed, his muscle strength improved significantly and his creatine phosphatase kinase (CPK) values trended down, indicating that his myositis was improving as well. He was transitioned to oral prednisone 60 mg daily and was discharged home with a rheumatology follow-up to define long-term treatment. A myositis panel revealed anti-glycyl-transferRNA synthetase (EJ) autoantibody positivity and a diagnosis was established. Our case revealed how sometimes laboratory values do not necessarily correlate with disease severity and how we have to do a thorough history of present illness and physical exam to think about unusual diagnoses before putting laboratory data into context. CI - Copyright © 2023, Peña et al. FAU - Peña, Carlos AU - Peña C AD - Internal Medicine, Mount Sinai Medical Center, Miami Beach, USA. FAU - Kalara, Niketa AU - Kalara N AD - Internal Medicine, Mount Sinai Medical Center, Miami Beach, USA. FAU - Velagapudi, Pallavi AU - Velagapudi P AD - Internal Medicine, Mount Sinai Medical Center, Miami Beach, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230618 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC10277010 OTO - NOTNLM OT - anti synthetase syndrome OT - anti-ej antibody OT - anti-jo-1 antibodies OT - inflammatory myositis OT - mechanic hands COIS- The authors have declared that no competing interests exist. EDAT- 2023/06/20 06:42 MHDA- 2023/06/20 06:43 PMCR- 2023/06/18 CRDT- 2023/06/20 02:27 PHST- 2023/06/18 00:00 [accepted] PHST- 2023/06/20 06:43 [medline] PHST- 2023/06/20 06:42 [pubmed] PHST- 2023/06/20 02:27 [entrez] PHST- 2023/06/18 00:00 [pmc-release] AID - 10.7759/cureus.40588 [doi] PST - epublish SO - Cureus. 2023 Jun 18;15(6):e40588. doi: 10.7759/cureus.40588. eCollection 2023 Jun. PMID- 20947541 OWN - NLM STAT- MEDLINE DCOM- 20110315 LR - 20220409 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 19 IP - 14 DP - 2010 Dec TI - Pregnancy outcome in 396 pregnancies in patients with SLE in Saudi Arabia. PG - 1665-73 LID - 10.1177/0961203310378669 [doi] AB - The aim of this study was to examine the pregnancy outcomes in patients with systemic lupus erythematosus (SLE) and the effect of SLE flare and treatment on pregnancy outcomes. We performed a retrospective evaluation of all pregnancies occurring in patients with SLE during the 27-year period from 1980 to 2006. Of the 319 women with SLE planning pregnancy after SLE onset, 176 (55.2%) conceived resulting in 396 pregnancies. Live births were significantly lower in proportion (70.2% vs. 85.7%) and more likely to end in fetal deaths (29.7% vs. 14.2%) and preterm births (26.7% vs. 5.8 %) in pregnancies occurring after SLE onset than in pregnancies occurring before SLE onset (p < 0.0001). With respect to different disease manifestations, we found that fetal loss was significantly higher in patients with antiphospholipid (aPL) antibodies than without (p < 0.001). Preterm deliveries were significantly more frequent in patients with lupus nephritis, anti-Ro/SSA antibodies, hypertension, history of intravenous cyclophosphamide treatment and aPL than those without these features (p < 0.05). Neonates with intrauterine growth retardation (IUGR) neonates were more common in hypertensive and Raynaud's-positive pregnancies (p < 0.05). SLE flares occurred in 30.8% pregnancies. There was increased risk of fetal loss, preterm births and IUGR in pregnancies with SLE exacerbations than without (p < 0.05). Prednisolone was found to improve the rate of live births, although it was also a predictor of prematurity. The predictors of pregnancy loss were lupus nephritis (odds ratio (OR) 7.3), aPL (OR 3.9), and SLE flares in pregnancy (OR 1.9). There was higher risk of preterm deliveries in patients with lupus nephritis (OR 18.9), anti-Ro antibodies (OR 13.9), hypertension (OR 15.7) and SLE flares (OR 2.5). IUGR was found to be associated with hypertension (OR 37.7), Raynaud's (OR 12.3), and SLE flares (OR 4.2). In conclusion, pregnancies in SLE patients with active lupus nephritis, anti-Ro/SSA antibodies, aPL, hypertension, Raynaud's phenomenon, active disease at conception and SLE exacerbations are at a higher risk of adverse pregnancy outcomes. It is important to carefully plan pregnancy, and experienced rheumatologists and obstetricians should monitor SLE patients in pregnancy and postpartum. FAU - Al Arfaj, A S AU - Al Arfaj AS AD - Division of Rheumatology, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia. zumaigahoo@yahoo.com FAU - Khalil, N AU - Khalil N LA - eng PT - Journal Article DEP - 20101014 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Abortion, Spontaneous/epidemiology/etiology MH - Adolescent MH - Adult MH - Antibodies, Antiphospholipid/*immunology MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Infant, Newborn MH - Lupus Erythematosus, Systemic/*complications/immunology MH - Lupus Nephritis/complications MH - Middle Aged MH - Prednisolone/therapeutic use MH - Pregnancy MH - *Pregnancy Complications MH - *Pregnancy Outcome MH - Premature Birth/etiology MH - Retrospective Studies MH - Risk MH - Saudi Arabia/epidemiology MH - Young Adult EDAT- 2010/10/16 06:00 MHDA- 2011/03/16 06:00 CRDT- 2010/10/16 06:00 PHST- 2010/10/16 06:00 [entrez] PHST- 2010/10/16 06:00 [pubmed] PHST- 2011/03/16 06:00 [medline] AID - 0961203310378669 [pii] AID - 10.1177/0961203310378669 [doi] PST - ppublish SO - Lupus. 2010 Dec;19(14):1665-73. doi: 10.1177/0961203310378669. Epub 2010 Oct 14. PMID- 17854742 OWN - NLM STAT- MEDLINE DCOM- 20071218 LR - 20070914 IS - 1568-9972 (Print) IS - 1568-9972 (Linking) VI - 6 IP - 8 DP - 2007 Sep TI - Vascular complications of scleroderma. PG - 520-3 AB - Systemic sclerosis (SSc) is a multiorgan disease characterized by injury to vascular wall and extensive damage of the microvessels. The injury of the vascular wall is characterized by the formation of megacapillaries and avascular areas. The reduced capillary density leads to clinical manifestations such as digital ulcers. These lesions are extremely painful and lead to substantial functional disability. Management of digital ulcers includes non-pharmacologic and pharmacologic modalities. Despite the reduced blood flow and reduced partial oxygen pressure levels, there is paradoxically no evidence for a sufficient angiogenesis in the skin of patients with SSc. Angiogenesis is strongly disturbed in SSc, as demonstrated by Nailfold Video-Capillaroscopy changes, the damage of the vessels evolves progressively from early to late stages and is characterized by different morphological aspects. Almost all patients develop Raynaud's phenomenon which, together with structural vasculopathy, results in ulceration and critical digital ischemia. Many of the severe internal organ complications of SSc are vascular, including pulmonary arterial hypertension (PAH) and scleroderma renal crisis. Structural vascular damage occurs in many vascular beds and contribute to pulmonary, renal, cardiac and gastrointestinal complications. SSc has a high case-specific mortality due to organ-based complications including PAH, lung fibrosis, renal failure and involvement of the gastrointestinal tract. FAU - Guiducci, Serena AU - Guiducci S AD - Division of Medicine I and Rheumatology, Department of Medicine and Surgery, AOUC, University of Florence, Florence, Italy. serena16@libero.it FAU - Giacomelli, Roberto AU - Giacomelli R FAU - Cerinic, Marco Matucci AU - Cerinic MM LA - eng PT - Journal Article PT - Review DEP - 20070112 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Animals MH - Humans MH - Scleroderma, Systemic/*complications/drug therapy/immunology MH - Vascular Diseases/drug therapy/*etiology RF - 25 EDAT- 2007/09/15 09:00 MHDA- 2007/12/19 09:00 CRDT- 2007/09/15 09:00 PHST- 2007/09/15 09:00 [pubmed] PHST- 2007/12/19 09:00 [medline] PHST- 2007/09/15 09:00 [entrez] AID - S1568-9972(06)00217-5 [pii] AID - 10.1016/j.autrev.2006.12.006 [doi] PST - ppublish SO - Autoimmun Rev. 2007 Sep;6(8):520-3. doi: 10.1016/j.autrev.2006.12.006. Epub 2007 Jan 12. PMID- 38139349 OWN - NLM STAT- MEDLINE DCOM- 20231225 LR - 20231225 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 24 IP - 24 DP - 2023 Dec 15 TI - The Interplay between Helicobacter pylori and Gut Microbiota in Non-Gastrointestinal Disorders: A Special Focus on Atherosclerosis. LID - 10.3390/ijms242417520 [doi] LID - 17520 AB - The discovery of Helicobacter pylori (H. pylori) in the early 1980s by Nobel Prize winners in medicine Robin Warren and Barry Marshall led to a revolution in physiopathology and consequently in the treatment of peptic ulcer disease. Subsequently, H. pylori has also been linked to non-gastrointestinal diseases, such as autoimmune thrombocytopenia, acne rosacea, and Raynaud's syndrome. In addition, several studies have shown an association with cardiovascular disease and atherosclerosis. Our narrative review aims to investigate the connection between H. pylori infection, gut microbiota, and extra-gastric diseases, with a particular emphasis on atherosclerosis. We conducted an extensive search on PubMed, Google Scholar, and Scopus, using the keywords "H. pylori", "dysbiosis", "microbiota", "atherosclerosis", "cardiovascular disease" in the last ten years. Atherosclerosis is a complex condition in which the arteries thicken or harden due to plaque deposits in the inner lining of an artery and is associated with several cardiovascular diseases. Recent research has highlighted the role of the microbiota in the pathogenesis of this group of diseases. H. pylori is able to both directly influence the onset of atherosclerosis and negatively modulate the microbiota. H. pylori is an important factor in promoting atherosclerosis. Progress is being made in understanding the underlying mechanisms, which could open the way to interesting new therapeutic perspectives. FAU - Candelli, Marcello AU - Candelli M AUID- ORCID: 0000-0001-8443-7880 AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Franza, Laura AU - Franza L AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Cianci, Rossella AU - Cianci R AUID- ORCID: 0000-0001-5378-8442 AD - Department of Translational Medicine and Surgery, Catholic University, Fondazione Policlinico Universitario A. Gemelli-IRCCS, 00168 Rome, Italy. FAU - Pignataro, Giulia AU - Pignataro G AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Merra, Giuseppe AU - Merra G AUID- ORCID: 0000-0003-4753-3528 AD - Biomedicine and Prevention Department, Section of Clinical Nutrition and Nutrigenomics, Facoltà di Medicina e Chirurgia, Università degli Studi di Roma Tor Vergata, 00133 Rome, Italy. FAU - Piccioni, Andrea AU - Piccioni A AUID- ORCID: 0000-0001-5703-8737 AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Ojetti, Veronica AU - Ojetti V AUID- ORCID: 0000-0002-8953-0707 AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Gasbarrini, Antonio AU - Gasbarrini A AD - Medical, Abdominal Surgery and Endocrine-Metabolic Science Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. FAU - Franceschi, Francesco AU - Franceschi F AD - Emergency, Anesthesiological and Reanimation Sciences Department, Fondazione Policlinico Universitario A. Gemelli-IRCCS of Rome, 00168 Rome, Italy. LA - eng PT - Journal Article PT - Review DEP - 20231215 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 SB - IM MH - Humans MH - *Gastrointestinal Microbiome MH - *Helicobacter pylori MH - *Peptic Ulcer MH - *Atherosclerosis/complications MH - *Microbiota MH - *Cardiovascular Diseases/complications MH - *Helicobacter Infections/complications PMC - PMC10744166 OTO - NOTNLM OT - Cag-A OT - H. pylori OT - atherosclerosis OT - gut microbiota OT - inflammation COIS- The authors declare no conflict of interest. EDAT- 2023/12/23 12:43 MHDA- 2023/12/25 06:42 PMCR- 2023/12/15 CRDT- 2023/12/23 01:22 PHST- 2023/10/27 00:00 [received] PHST- 2023/12/07 00:00 [revised] PHST- 2023/12/14 00:00 [accepted] PHST- 2023/12/25 06:42 [medline] PHST- 2023/12/23 12:43 [pubmed] PHST- 2023/12/23 01:22 [entrez] PHST- 2023/12/15 00:00 [pmc-release] AID - ijms242417520 [pii] AID - ijms-24-17520 [pii] AID - 10.3390/ijms242417520 [doi] PST - epublish SO - Int J Mol Sci. 2023 Dec 15;24(24):17520. doi: 10.3390/ijms242417520. PMID- 19863377 OWN - NLM STAT- MEDLINE DCOM- 20100111 LR - 20230215 IS - 1607-842X (Electronic) IS - 0891-6934 (Linking) VI - 42 IP - 7 DP - 2009 Nov TI - Mechanisms of vascular damage in systemic sclerosis. PG - 587-95 AB - Although being classified as autoimmune connective tissue disease, dominant components of the pathophysiology of systemic sclerosis (SSc) consists of mechanisms of vascular damage, which can occur early in the course of the disease. Amongst them are abnormal vasoreactivity, hypoxia, insufficient neoangiogenesis and direct damage of vascular and perivascular cells. They result in a decreased capillary blood flow, and subsequently in clinically overt symptoms such as Raynaud's syndrome and fingertip ulcers. In addition, in active disease vascular pathology can affect various other organs, predominantly the lung, the kidney, the heart but also the gastrointestinal tract. Vascular pathology contributes also significantly to overall morbidity and mortality in SSc patients and reduces life expectancy by at least a decade. Fortunately, molecular biology has revealed a number of underlying pathways on the cellular and subcellular levels, including key factors of the aberrant function of (peri)vascular cells and autoimmune effector cells, the dysregulation of vasoconstrictive molecules and their receptors, the upregulation of intracellular signaling kinases and the altered balance of hypoxia-induced vascular growth factors. This increasing knowledge of vascular pathology in SSc has also resulted in novel therapeutic approaches ranging from endothelin antagonists to application of progenitor cells to counteract this aberrant vascular pathology and to support the repair of the dysfunctional vasculature. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Internal Medicine and Rheumatology, Kerckhoff Clinic, Justus-Liebig University Giessen, Benekestrasse 2-8, D-61231, Bad Nauheim, Germany. u.mueller-ladner@kerckhoff-klinik.de FAU - Distler, Oliver AU - Distler O FAU - Ibba-Manneschi, Lidia AU - Ibba-Manneschi L FAU - Neumann, Elena AU - Neumann E FAU - Gay, Steffen AU - Gay S LA - eng PT - Journal Article PT - Review PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Autoantibodies) RN - 0 (Endothelin-1) RN - 0 (HIF1A protein, human) RN - 0 (Hypoxia-Inducible Factor 1, alpha Subunit) RN - 0 (Vascular Endothelial Growth Factor A) RN - 126547-89-5 (Intercellular Adhesion Molecule-1) SB - IM MH - Apoptosis/physiology MH - Autoantibodies/blood MH - Blood Vessels/*metabolism MH - Endothelin-1/metabolism MH - Endothelium, Vascular/*metabolism MH - Epigenesis, Genetic/physiology MH - Humans MH - Hypoxia-Inducible Factor 1, alpha Subunit/metabolism MH - Intercellular Adhesion Molecule-1/metabolism MH - Neovascularization, Pathologic/*immunology MH - Polymorphism, Single Nucleotide MH - Scleroderma, Systemic/*complications/*immunology MH - Vascular Diseases/genetics/*immunology MH - Vascular Endothelial Growth Factor A/metabolism RF - 69 EDAT- 2009/10/30 06:00 MHDA- 2010/01/12 06:00 CRDT- 2009/10/30 06:00 PHST- 2009/10/30 06:00 [entrez] PHST- 2009/10/30 06:00 [pubmed] PHST- 2010/01/12 06:00 [medline] AID - 10.1080/08916930903002487 [pii] AID - 10.1080/08916930903002487 [doi] PST - ppublish SO - Autoimmunity. 2009 Nov;42(7):587-95. doi: 10.1080/08916930903002487. PMID- 20936633 OWN - NLM STAT- MEDLINE DCOM- 20110128 LR - 20260518 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 63 IP - 1 DP - 2011 Jan TI - Cigarette smoking in patients with systemic sclerosis. PG - 230-8 LID - 10.1002/art.30071 [doi] AB - OBJECTIVE: To determine the effects of cigarette smoking on vascular, gastrointestinal, and respiratory outcomes in patients with systemic sclerosis (SSc). METHODS: Subjects were patients enrolled in the Canadian Scleroderma Research Group cohort. Smoking history was obtained by patient self-report. The effect of smoking was assessed using multiple regression analysis of each SSc clinical outcome of interest (vascular, gastrointestinal, and respiratory). Smoking was modeled both as categorical variables (current, past, and never) and using the Comprehensive Smoking Index (CSI), which integrates smoking intensity, duration of smoking, and time since cessation into a single covariate of smoking effect. All regression models were adjusted for age, sex, disease duration, and limited or diffuse skin involvement. RESULTS: This study included 606 patients with SSc, of whom 87% were women and 90% were white, and the mean age was 55 years, mean disease duration was 11 years, and 36% had diffuse disease. Of these patients, 16% were current smokers, 42% were past smokers, and 42% were never smokers. The regression analyses showed that smoking had a significant negative effect on almost all vascular, gastrointestinal, and respiratory outcomes. The effects of smoking were in some cases long-lasting (e.g., persistent respiratory abnormalities), and smoking cessation appeared beneficial with respect to some outcomes (e.g., reduced severity of Raynaud's phenomenon). CONCLUSION: Physicians caring for patients with SSc should prioritize smoking cessation as a recommendation to patients, and resources directed to supporting smoking cessation in patients with SSc should be more readily available. CI - Copyright © 2011 by the American College of Rheumatology. FAU - Hudson, Marie AU - Hudson M AD - SMBD-Jewish General Hospital and McGill University, Montreal, Quebec, Canada. marie.hudson@mcgill.ca FAU - Lo, Ernest AU - Lo E FAU - Lu, Ying AU - Lu Y FAU - Hercz, Daniel AU - Hercz D FAU - Baron, Murray AU - Baron M FAU - Steele, Russell AU - Steele R CN - Canadian Scleroderma Research Group LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM CIN - Arthritis Rheum. 2011 Jun;63(6):1758-9. doi: 10.1002/art.30352. PMID: 21437869 MH - Aged MH - Cross-Sectional Studies MH - Female MH - Gastrointestinal Diseases/complications MH - Humans MH - Lung Diseases/complications MH - Male MH - Middle Aged MH - Regression Analysis MH - Scleroderma, Systemic/*complications MH - Severity of Illness Index MH - Smoking/*adverse effects MH - Smoking Cessation MH - Vascular Diseases/complications FIR - Baron, M IR - Baron M FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Khalidi, N IR - Khalidi N FIR - Docherty, P IR - Docherty P FIR - Kaminska, E IR - Kaminska E FIR - Masetto, A IR - Masetto A FIR - Smith, D IR - Smith D FIR - Sutton, E IR - Sutton E FIR - Mathieu, J-P IR - Mathieu JP FIR - Hudson, M IR - Hudson M FIR - Ligier, S IR - Ligier S FIR - Grodzicky, T IR - Grodzicky T FIR - Mittoo, S IR - Mittoo S FIR - Fritzler, M IR - Fritzler M EDAT- 2010/10/12 06:00 MHDA- 2011/02/01 06:00 CRDT- 2010/10/12 06:00 PHST- 2010/10/12 06:00 [entrez] PHST- 2010/10/12 06:00 [pubmed] PHST- 2011/02/01 06:00 [medline] AID - 10.1002/art.30071 [doi] PST - ppublish SO - Arthritis Rheum. 2011 Jan;63(1):230-8. doi: 10.1002/art.30071. PMID- 16631934 OWN - NLM STAT- MEDLINE DCOM- 20060726 LR - 20061115 IS - 0165-5728 (Print) IS - 0165-5728 (Linking) VI - 175 IP - 1-2 DP - 2006 Jun TI - BDNF: a missing link between sympathetic dysfunction and inflammatory disease? PG - 118-27 AB - Nerve growth factor (NGF) plays a role in sympathetic neuron integrity and survival. Brain-derived neurotrophic factor (BDNF) also has trophic effects on sympathetic neurons. We report here the serendipitous finding that co-treatment of hippocampus with BDNF and the NGF antagonist TrkA-Fc leads to perivascular inflammation and marked vasoconstriction. This effect is not observed with either reagent alone or in combination with other control proteins. Because NGF supports sympathetic neuron health, we tested the hypothesis that BDNF combined with sympathetic compromise caused this effect. Superior cervical ganglia were removed bilaterally with concurrent BDNF infusion into hippocampus. Perivascular inflammation was observed at 3 days, but not 12 days post treatment, when sympathetic terminals had receded, suggesting that the presence of these terminals was necessary for inflammation. Since sympathetic dysfunction may lead to compensatory overactivity of norepinephrine (NE) signaling, we co-infused BDNF with NE in the hippocampus and observed perivascular inflammation. In humans, sympathetic overactivity has been reported in a variety of vascular diseases. Some of these diseases, e.g. primary Raynaud's, are not accompanied by serious inflammatory disease whereas others, such as scleroderma and systemic lupus, are. We speculate that BDNF may contribute to the transformation of sympathetic dysfunction to inflammatory disease. FAU - Kasselman, Lora J AU - Kasselman LJ AD - Neuropsychology Doctoral Subprogram, Graduate Center of the City University of New York, USA. FAU - Sideris, Alexandra AU - Sideris A FAU - Bruno, Chantal AU - Bruno C FAU - Perez, William R AU - Perez WR FAU - Cai, Ning AU - Cai N FAU - Nicoletti, Jamee N AU - Nicoletti JN FAU - Wiegand, Stanley J AU - Wiegand SJ FAU - Croll, Susan D AU - Croll SD LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060424 PL - Netherlands TA - J Neuroimmunol JT - Journal of neuroimmunology JID - 8109498 RN - 0 (Brain-Derived Neurotrophic Factor) SB - IM MH - Animals MH - Autonomic Nervous System Diseases/*immunology/*metabolism/pathology/physiopathology MH - Brain Edema/*immunology/*metabolism/pathology MH - Brain-Derived Neurotrophic Factor/administration & dosage/*physiology MH - Hippocampus/metabolism MH - Inflammation/metabolism/physiopathology MH - Infusion Pumps MH - Neurons/immunology/metabolism/pathology MH - Rats MH - Rats, Sprague-Dawley EDAT- 2006/04/25 09:00 MHDA- 2006/07/27 09:00 CRDT- 2006/04/25 09:00 PHST- 2005/09/09 00:00 [received] PHST- 2006/03/12 00:00 [revised] PHST- 2006/03/15 00:00 [accepted] PHST- 2006/04/25 09:00 [pubmed] PHST- 2006/07/27 09:00 [medline] PHST- 2006/04/25 09:00 [entrez] AID - S0165-5728(06)00087-7 [pii] AID - 10.1016/j.jneuroim.2006.03.008 [doi] PST - ppublish SO - J Neuroimmunol. 2006 Jun;175(1-2):118-27. doi: 10.1016/j.jneuroim.2006.03.008. Epub 2006 Apr 24. PMID- 40592721 OWN - NLM STAT- MEDLINE DCOM- 20250701 LR - 20260513 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 11 IP - 3 DP - 2025 Jul 1 TI - New horizons in systemic sclerosis treatment: advances and emerging therapies in 2025. LID - 10.1136/rmdopen-2025-005776 [doi] LID - e005776 AB - Systemic sclerosis (SSc) is a rare, multisystem autoimmune disease characterised by vasculopathy, immune dysregulation, and progressive fibrosis, leading to significant morbidity and mortality. While recent EULAR recommendations have updated the standard of care for SSc, the field is rapidly evolving with novel therapeutic strategies and precision medicine approaches.Traditional immunosuppressive therapies-including mycophenolate mofetil, cyclophosphamide and rituximab-remain essential for controlling skin and lung involvement while autologous haematopoietic stem cell transplantation offers a proven disease-modifying option for selected high-risk patients. Tocilizumab and nintedanib have established roles in lung preservation in SSc-associated interstitial lung disease (SSc-ILD). In pulmonary arterial hypertension (PAH), early combination therapy with endothelin receptor antagonists and phosphodiesterase-5 inhibitors, complemented by newer agents such as selexipag and riociguat, has improved survival and quality of life. Advances in gastrointestinal, renal and musculoskeletal management continue to evolve, with promising roles for intravenous immunoglobulin and novel prokinetics.Crucially, emerging therapies-including CD19-targeted CAR-T cells, bispecific antibodies and agents targeting interferon pathways, BAFF, melanocortin, FcRn and PDE4B-reflect a shift towards personalised and biomarker-driven approaches. These innovations offer the potential to alter disease trajectory and support early, targeted intervention in SSc.This review provides an up-to-date synthesis of both current organ-based treatment strategies in major organ domains-skin, ILD, PAH, scleroderma renal crisis, raynaud's phenomenon and digital ulcers, gastrointestinal and musculoskeletal involvement-and emerging therapies in SSc, with an emphasis on disease-modifying approaches and future directions in personalised care. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Sieiro Santos, Cristiana AU - Sieiro Santos C AUID- ORCID: 0000-0003-0889-9877 AD - The University of Manchester Centre for Musculoskeletal Research, Manchester, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK f.delgaldo@leeds.ac.uk. AD - Scleroderma Programme, NIHR Leeds Musculoskeletal Biomedical Research Centre, Leeds, UK. LA - eng SI - ClinicalTrials.gov/NCT06375005, NCT05559580, NCT05925803, NCT05878717, NCT04440592, NCT05270668, NCT06195072 PT - Clinical Trial PT - Journal Article DEP - 20250701 PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Immunosuppressive Agents) SB - IM MH - Humans MH - *Scleroderma, Systemic/therapy/diagnosis/etiology MH - Disease Management MH - Immunosuppressive Agents/therapeutic use MH - Quality of Life PMC - PMC12215137 OTO - NOTNLM OT - Clinical Trial OT - Hematopoietic Stem Cell Transplantation OT - Lung Diseases, Interstitial OT - Pulmonary Arterial Hypertension OT - Systemic Sclerosis COIS- Competing interests: None declared. EDAT- 2025/07/02 00:27 MHDA- 2025/07/02 00:28 PMCR- 2025/07/01 CRDT- 2025/07/01 22:23 PHST- 2025/04/07 00:00 [received] PHST- 2025/06/20 00:00 [accepted] PHST- 2025/07/02 00:28 [medline] PHST- 2025/07/02 00:27 [pubmed] PHST- 2025/07/01 22:23 [entrez] PHST- 2025/07/01 00:00 [pmc-release] AID - rmdopen-2025-005776 [pii] AID - 10.1136/rmdopen-2025-005776 [doi] PST - epublish SO - RMD Open. 2025 Jul 1;11(3):e005776. doi: 10.1136/rmdopen-2025-005776. PMID- 30428504 OWN - NLM STAT- MEDLINE DCOM- 20190529 LR - 20190529 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 22 IP - 1 DP - 2019 Jan TI - Initial presentation determines clinical entity in patients with anti-centromere antibody positivity. PG - 103-107 LID - 10.1111/1756-185X.13439 [doi] AB - AIM: Anti-centromere antibody (ACA) is often detected in patients with autoimmune diseases, including limited cutaneous systemic sclerosis (SSc), Sjögren's syndrome (SS), and primary biliary cholangitis (PBC). The association between autoimmune disease and ACA positivity remains unclear. We sought to clarify the clinical features of ACA-positive patients and their association with autoantibodies. METHOD: A total of 309 cases of a discrete-speckled pattern anti-nuclear antibody (ANA) test and/or positive ACA who visited our department were retrospectively enrolled. Clinical and immunological data were collected and statistically analyzed. RESULT: A proportion of second and/or third ANA patterns were speckled (16%), homogenous (7%), cytoplasmic (3%) and/or nucleolar (3%). Of the 309 patients, 186 had Raynaud's phenomenon, 149 had sclerodactyly, and 162 had oral and/or ocular dryness. A total of 214 patients were classified into 17 autoimmune diseases based on their symptoms at the initial visit, while the other 95 patients did not meet any criteria. Most of the 214 patients were diagnosed with SSc and/or SS; 25 and 22 additional patients were diagnosed with rheumatoid arthritis and PBC, respectively. Higher titers of immunoglobulins were observed in patients diagnosed with autoimmune disease compared to patients without a diagnosis. The mean observation period was 80 months. Three additional patients received interim diagnoses based on new symptoms or organ involvement. In the other patients, the diagnosis made at the first visit was not changed over the observation period. CONCLUSION: Our study confirmed that many ACA-positive cases can be classified into an autoimmune disease type on presentation. CI - © 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Tsukamoto, Masako AU - Tsukamoto M AUID- ORCID: 0000-0002-7506-6173 AD - Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. FAU - Suzuki, Katsuya AU - Suzuki K AD - Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. FAU - Takeuchi, Tsutomu AU - Takeuchi T AD - Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. LA - eng GR - Keio University/ PT - Journal Article DEP - 20181114 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/blood/*immunology MH - Autoimmune Diseases/blood/diagnosis/*immunology MH - *Autoimmunity MH - Biomarkers/blood MH - Centromere/*immunology MH - Female MH - Fluorescent Antibody Technique MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Retrospective Studies MH - Serologic Tests MH - Young Adult OTO - NOTNLM OT - Sjögren's syndrome OT - anti-nuclear/BL OT - antibodies OT - centromere/IM OT - systemic sclerosis EDAT- 2018/11/15 06:00 MHDA- 2019/05/30 06:00 CRDT- 2018/11/15 06:00 PHST- 2018/08/10 00:00 [received] PHST- 2018/09/15 00:00 [revised] PHST- 2018/10/18 00:00 [accepted] PHST- 2018/11/15 06:00 [pubmed] PHST- 2019/05/30 06:00 [medline] PHST- 2018/11/15 06:00 [entrez] AID - 10.1111/1756-185X.13439 [doi] PST - ppublish SO - Int J Rheum Dis. 2019 Jan;22(1):103-107. doi: 10.1111/1756-185X.13439. Epub 2018 Nov 14. PMID- 17785320 OWN - NLM STAT- MEDLINE DCOM- 20071002 LR - 20070905 IS - 0077-8923 (Print) IS - 0077-8923 (Linking) VI - 1109 DP - 2007 Aug TI - Serological and clinical characterization of anti-dsDNA and anti-PM/Scl double-positive patients. PG - 311-21 AB - Antibodies to double-stranded desoxyribonucleic acid (dsDNA) and to the polymyositis/scleroderma (PM/Scl) complex are regarded as serological markers for systemic lupus erythematosus (SLE) and PM/Scl overlap syndrome, respectively. In a previous study, serum samples were identified that contained antibodies specific for both dsDNA and PM/Scl. Fourteen of these sera were available for more detailed investigation including the autoantibody profile as determined by several methods including an addressable laser bead assay, Crithidia luciliae indirect immunofluorescence test (CLIFT) and a PM1-Alpha ELISA. Moreover, 300 samples from connective tissue disease patients and 30 PM/Scl positive samples were screened for anti-dsDNA(+)/PM/Scl(+) specimens by CLIFT, dsDNA ELISA, and PM1-Alpha ELISA. We confirmed anti-dsDNA and anti-PM/Scl reactivity in 2/7 samples from the previous study. One sample had also anti-chromatin and anti-SS-A reactivity and the second sample was oligoreactive. In addition, 2/300 (0.7%) unselected samples from connective tissue disease patients were identified with anti-dsDNA and anti-PM/Scl reactivity. In a panel of PM1-Alpha positive samples (n = 30) collected regardless of the diagnosis of the patients, no anti-dsDNA reactivity was found. All anti-dsDNA(+)/anti-PM/Scl(+) patients identified fulfilled sufficient criteria to be classified as definite SLE and also had at least one feature of systemic sclerosis (i.e., sclerodactyly and/or Raynaud's phenomenon). Only 1/4 patients had clinical evidence of dermatomyositis. The combination of anti-dsDNA(+)/anti-PM/Scl(+) in patients suffering from connective tissue disease is less frequently found than previously described when newer assays are used. Clinically, anti-dsDNA(+)/anti-PM/Scl(+) patients may define a small subgroup of SLE patients with additional features of systemic sclerosis. FAU - Mahler, M AU - Mahler M AD - Dr. Fooke Laboratorien GmbH, Neuss, Germany. m.mahler.job@web.de FAU - Greidinger, E L AU - Greidinger EL FAU - Szmyrka, M AU - Szmyrka M FAU - Kromminga, A AU - Kromminga A FAU - Fritzler, M J AU - Fritzler MJ LA - eng PT - Journal Article PL - United States TA - Ann N Y Acad Sci JT - Annals of the New York Academy of Sciences JID - 7506858 RN - 0 (Antibodies) RN - 9007-49-2 (DNA) SB - IM MH - Adult MH - Antibodies/*immunology MH - DNA/*immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/blood/*immunology/*pathology MH - Male MH - Middle Aged MH - Polymyositis/blood/*immunology/*pathology MH - Scleroderma, Systemic/blood/*immunology/pathology EDAT- 2007/09/06 09:00 MHDA- 2007/10/03 09:00 CRDT- 2007/09/06 09:00 PHST- 2007/09/06 09:00 [pubmed] PHST- 2007/10/03 09:00 [medline] PHST- 2007/09/06 09:00 [entrez] AID - 1109/1/311 [pii] AID - 10.1196/annals.1398.037 [doi] PST - ppublish SO - Ann N Y Acad Sci. 2007 Aug;1109:311-21. doi: 10.1196/annals.1398.037. PMID- 18215315 OWN - NLM STAT- MEDLINE DCOM- 20080327 LR - 20211020 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 3 DP - 2008 Jan 23 TI - Primary biliary cirrhosis. PG - 1 LID - 10.1186/1750-1172-3-1 [doi] AB - Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC. FAU - Kumagi, Teru AU - Kumagi T AD - Department of Medicine, Toronto Western Hospital (University Health Network/University of Toronto), Toronto, Ontario, Canada. masato_teru@yahoo.co.jp FAU - Heathcote, E Jenny AU - Heathcote EJ LA - eng PT - Journal Article PT - Review DEP - 20080123 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (Antibodies, Antinuclear) RN - 0 (Cholagogues and Choleretics) RN - 724L30Y2QR (Ursodeoxycholic Acid) SB - IM MH - Aged MH - Antibodies, Antinuclear MH - Autoimmune Diseases/*physiopathology MH - Cholagogues and Choleretics/therapeutic use MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/diagnosis/drug therapy/*immunology/*physiopathology MH - Liver Transplantation MH - Male MH - Middle Aged MH - Sex Factors MH - Ursodeoxycholic Acid/therapeutic use PMC - PMC2266722 EDAT- 2008/01/25 09:00 MHDA- 2008/03/28 09:00 PMCR- 2008/01/23 CRDT- 2008/01/25 09:00 PHST- 2007/07/26 00:00 [received] PHST- 2008/01/23 00:00 [accepted] PHST- 2008/01/25 09:00 [pubmed] PHST- 2008/03/28 09:00 [medline] PHST- 2008/01/25 09:00 [entrez] PHST- 2008/01/23 00:00 [pmc-release] AID - 1750-1172-3-1 [pii] AID - 10.1186/1750-1172-3-1 [doi] PST - epublish SO - Orphanet J Rare Dis. 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID- 41072301 OWN - NLM STAT- MEDLINE DCOM- 20251124 LR - 20251124 IS - 2590-0412 (Electronic) IS - 2590-0412 (Linking) VI - 88 DP - 2025 Nov TI - When to test for myositis antibodies in usual interstitial pneumonia on chest CT? PG - 101211 LID - S2590-0412(25)00058-3 [pii] LID - 10.1016/j.resmer.2025.101211 [doi] AB - INTRODUCTION: Usual interstitial pneumonia (UIP) is a key pattern of interstitial lung disease (ILD), most commonly linked to idiopathic pulmonary fibrosis (IPF). Identifying underlying autoimmune conditions such as myositis is clinically relevant, yet guidelines provide limited recommendations regarding myositis antibody (MSA) screening. METHODS: We retrospectively analyzed patients with UIP who underwent systematic MSA screening at our center. Clinical and serological characteristics were compared between MSA-positive (MSA+) and MSA-negative (MSA-) groups. Logistic regression was used to identify predictive factors. RESULTS: Among 134 patients, 15 (11 %) were MSA+. Compared with MSA- patients, MSA+ cases were more likely to present with autoimmune disease (p = 0.03), ANA ≥ 1:320 (p < 0.001), and positive rheumatoid factor (p = 0.04). A four-parameter profile combining ANA < 1:320, absence of rheumatoid factor, no hypergammaglobulinemia, and no Raynaud's phenomenon strongly predicted MSA negativity with excellent specificity (100 %), modest sensitivity (35 %), and good overall discriminative ability (AUC = 0.82). DISCUSSION: In patients with UIP, a simple four-parameter profile (ANA < 1:320, absence of rheumatoid factor, absence of hypergammaglobulinemia, and absence of Raynaud's phenomenon) strongly predicts negative MSA status. These findings support a more targeted approach to MSA screening, potentially improving diagnostic accuracy, guiding treatment decisions, and reducing unnecessary testing in UIP. CI - Copyright © 2025. Published by Elsevier Masson SAS. FAU - Guillois, Eliott AU - Guillois E AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Bertin, Daniel AU - Bertin D AD - Biogénopôle, CHU La Timone, Assistance Publique-Hôpitaux de Marseille (AP-HM), Marseille, France. FAU - Milesi, Jules AU - Milesi J AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Habert, Paul AU - Habert P AD - Department of Radiology, Hôpital Nord, Assistance Publique - Hôpitaux de Marseille (AP-HM), Marseille, France. FAU - Di Bisceglie, Mathieu AU - Di Bisceglie M AD - Department of Radiology, Hôpital Nord, Assistance Publique - Hôpitaux de Marseille (AP-HM), Marseille, France. FAU - Heim, Xavier AU - Heim X AD - Biogénopôle, CHU La Timone, Assistance Publique-Hôpitaux de Marseille (AP-HM), Marseille, France. FAU - Coiffard, Benjamin AU - Coiffard B AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Naud, Romain AU - Naud R AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Nieves, Ana AU - Nieves A AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Nguyen, Ngoc Anh Thu AU - Nguyen NAT AD - Department of Epidemiology and Health Economics, Faculty of Medicine, Aix-Marseille University, Marseille, France. FAU - Reynaud-Gaubert, Martine AU - Reynaud-Gaubert M AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France. FAU - Bardin, Nathalie AU - Bardin N AD - Biogénopôle, CHU La Timone, Assistance Publique-Hôpitaux de Marseille (AP-HM), Marseille, France; Center for Cardiovascular and Nutrition Research, Aix Marseille Univ, INSERM 1263, INRA 1260, Marseille, France. FAU - Bermudez, Julien AU - Bermudez J AD - Department of Respiratory Medicine and Lung Transplantation, Centre de compétences des maladies pulmonaires rares, RespiFil, Aix Marseille Univ, APHM, North Hospital, Marseille, France; Center for Cardiovascular and Nutrition Research, Aix Marseille Univ, INSERM 1263, INRA 1260, Marseille, France. Electronic address: julien.bermudez@ap-hm.fr. LA - eng PT - Journal Article DEP - 20250926 PL - France TA - Respir Med Res JT - Respiratory medicine and research JID - 101746324 RN - 0 (Autoantibodies) RN - 9009-79-4 (Rheumatoid Factor) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Male MH - Female MH - Retrospective Studies MH - Aged MH - *Myositis/immunology/diagnosis/blood MH - Middle Aged MH - *Tomography, X-Ray Computed/methods MH - *Autoantibodies/blood MH - *Idiopathic Pulmonary Fibrosis/diagnostic imaging/immunology/blood MH - *Lung Diseases, Interstitial/diagnostic imaging MH - Sensitivity and Specificity MH - Rheumatoid Factor/blood MH - Antibodies, Antinuclear/blood OTO - NOTNLM OT - Antibodies OT - Idiopathic pulmonary fibrosis OT - Immunoblotting OT - Myositis OT - Usual interstitial pneumonia COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/10/11 00:32 MHDA- 2025/11/25 00:27 CRDT- 2025/10/10 18:09 PHST- 2025/03/08 00:00 [received] PHST- 2025/09/14 00:00 [revised] PHST- 2025/09/21 00:00 [accepted] PHST- 2025/11/25 00:27 [medline] PHST- 2025/10/11 00:32 [pubmed] PHST- 2025/10/10 18:09 [entrez] AID - S2590-0412(25)00058-3 [pii] AID - 10.1016/j.resmer.2025.101211 [doi] PST - ppublish SO - Respir Med Res. 2025 Nov;88:101211. doi: 10.1016/j.resmer.2025.101211. Epub 2025 Sep 26. PMID- 28794554 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 62 IP - 4 DP - 2017 Jul-Aug TI - Mucocutaneous Manifestations in Patients with Rheumatoid Arthritis: A Cross-sectional Study from Eastern India. PG - 411-417 LID - 10.4103/ijd.IJD_260_17 [doi] AB - BACKGROUND: Cutaneous manifestations are fairly common in rheumatoid arthritis (RA) and they can help in early diagnosis, prompt treatment, and hence reduced morbidity from the disease. AIMS: The objective of the present study was to find out the different patterns of dermatoses in a group of patients with RA from Eastern India. METHODOLOGY: Consecutive patients fulfilling the American Rheumatism Association 1987 revised criteria for the classification of RA and who had different dermatoses were included in this cross-sectional study done over a period of 8 years in a tertiary care hospital in Eastern India. Thorough clinical examination and appropriate laboratory investigations were performed as needed. Data were recorded in a predesigned schedule, and appropriate statistical analysis was done. RESULTS: We studied 111 evaluable patients with an age range of 19-71 years and a female to male ratio of 7:1. The mean disease duration of RA was 6.5 years. Cutaneous infections as a group was the most common mucocutaneous manifestation (34.2%) followed by xerosis including ichthyotic skin changes (27%), pigmented purpuric dermatoses (14.4%), leg ulcer (9.9%), periungual telangiectasia (9.9%), rheumatoid nodules (RNs) (8.1%), purpura and ecchymoses (7.2%), small vessel vasculitis in (7.2%), corn and callosities (6.3%), palmar erythema (4.5%), and neutrophilic dermatosis (4.5%). Raynaud's phenomenon was found in 3.6% patients and panniculitis in (3.6%) patients. Rheumatoid factor (RF) and anti-cyclic citrullinated peptides antibody were positive in 74.8% and 88.3% patients, respectively. No statistically significant difference of incidence of leg ulcer, small vessel vasculitis, RN, or Raynaud's phenomenon could be noted between RF positive and negative groups. LIMITATIONS: Being an institution-based study, the study findings may not reflect the true situation in the community which remained a limitation of this study. CONCLUSION: While some of the features of this study were analogous to Western data, other features showed discordance which may be due to ethnic variations among the patients with RA. FAU - Ghosh, Sudip Kumar AU - Ghosh SK AD - Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India. FAU - Bandyopadhyay, Debabrata AU - Bandyopadhyay D AD - Department of Dermatology, Venereology, and Leprosy, Medical College, Kolkata, West Bengal, India. FAU - Biswas, Surajit Kumar AU - Biswas SK AD - Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India. FAU - Darung, Ivoreen AU - Darung I AD - Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India. LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC5527724 OTO - NOTNLM OT - Mucocutaneous OT - rheumatoid arthritis OT - rheumatoid nodule COIS- There are no conflicts of interest. What is new? The present study highlighted the patterns of mucocutaneous manifestations in a group of patients from Eastern IndiaWhereas some of the features of this study are comparable to Western data, other features showed discordance to the existing data which may be due ethnic variations of the patients with rheumatoid arthritis. EDAT- 2017/08/11 06:00 MHDA- 2017/08/11 06:01 PMCR- 2017/07/01 CRDT- 2017/08/11 06:00 PHST- 2017/08/11 06:00 [entrez] PHST- 2017/08/11 06:00 [pubmed] PHST- 2017/08/11 06:01 [medline] PHST- 2017/07/01 00:00 [pmc-release] AID - IJD-62-411 [pii] AID - 10.4103/ijd.IJD_260_17 [doi] PST - ppublish SO - Indian J Dermatol. 2017 Jul-Aug;62(4):411-417. doi: 10.4103/ijd.IJD_260_17. PMID- 40557171 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250626 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 36 IP - 1 DP - 2025 Mar TI - Prevalence of Anti-Synthetase Syndrome in Patients of Interstitial Lung Disease with Connective Tissue Diseases and Autoimmune Features: A Cross-Sectional Study. PG - 107-115 LID - 10.31138/mjr.180324.dtc [doi] AB - OBJECTIVE: Anti-synthetase syndrome (ASS) is a rare autoimmune disease with heterogenous manifestations. Interstitial lung disease (ILD) is one among its common manifestations. The aim of this study was to evaluate the prevalence of ASS in cases of ILD associated with autoimmune features and describe the clinical, serological, and radiological profile in them. METHODS: This cross-sectional study included a total of 100 patients: 50 cases each of connective tissue disease-related ILD (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF). RESULTS: Four cases of CTD-ILD and 7 cases of IPAF had anti-ARS auto-antibodies. All eleven of them fulfilled Connor's criteria for ASS. The classic triad of arthritis, myositis, and ILD was present only in two cases. Anti-nuclear antibody (ANA) was positive in 63.6%. Anti-Jo1 (54.56%), Anti-PL12(27.3%), anti-PL7(18.2%), and anti-EJ(18.2%) were the anti ARS autoantibodies. Though generally considered to be mutually exclusive, anti-PL12 and anti-EJ antibodies were found together in two cases. Myalgia was associated with all four ARS antibodies. Anti-Jo1 antibody was associated with Raynaud's phenomenon, polyarthralgia, polyarthritis, and myopathy. Anti-PL7 antibody was associated with myopathy and mechanic's hands. Anti-PL12 and anti-EJ antibodies were associated with inflammatory poly-arthritis, polyarthralgia, and unexplained fever. Non-specific interstitial pneumonia (NSIP) was the most common radiologic pattern of ILD (81.8%). The remaining two had Usual interstitial pneumonia (UIP) pattern and were positive for anti-Jo1 antibody. CONCLUSION: ASS can present in many ways, often incomplete at the onset without the classic clinical triad. Anti-ARS autoantibodies can be found in established CTDs. Anti-cytoplasmic antibodies (not ANA) must be used to screen for ASS in suspected cases. CI - © 2025 The Author(s). FAU - Mb, Indu AU - Mb I AD - Department of General Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, New Delhi, India. FAU - Deepak, Desh AU - Deepak D AD - Department of Pulmonary Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia, New Delhi, India. FAU - Bhatta, Ajay AU - Bhatta A AD - Department of General Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, New Delhi, India. FAU - Lalwani, Gunjan AU - Lalwani G AD - Department of General Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, New Delhi, India. FAU - Sharma, Brijesh AU - Sharma B AD - Department of General Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, New Delhi, India. FAU - Somayya, Vardhini AU - Somayya V AD - Department of General Medicine, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, New Delhi, India. FAU - Chhabra, Mala AU - Chhabra M AD - Department of Microbiology, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia, New Delhi, India. FAU - Duggal, Nandini AU - Duggal N AD - Department of Microbiology, Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia, New Delhi, India. LA - eng PT - Journal Article DEP - 20250331 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC12183454 OTO - NOTNLM OT - anti-aminoacyl tRNA synthetase antibodies OT - anti-synthetase syndrome OT - connective tissue diseases OT - interstitial lung disease OT - interstitial pneumonia with autoimmune features COIS- The authors declare no conflicts of interest. EDAT- 2025/06/25 06:25 MHDA- 2025/06/25 06:26 PMCR- 2025/03/31 CRDT- 2025/06/25 04:31 PHST- 2024/03/18 00:00 [received] PHST- 2024/04/20 00:00 [revised] PHST- 2024/05/15 00:00 [accepted] PHST- 2025/06/25 06:26 [medline] PHST- 2025/06/25 06:25 [pubmed] PHST- 2025/06/25 04:31 [entrez] PHST- 2025/03/31 00:00 [pmc-release] AID - MJR-36-1-107 [pii] AID - 10.31138/mjr.180324.dtc [doi] PST - epublish SO - Mediterr J Rheumatol. 2025 Mar 31;36(1):107-115. doi: 10.31138/mjr.180324.dtc. eCollection 2025 Mar. PMID- 31750929 OWN - NLM STAT- MEDLINE DCOM- 20200828 LR - 20200828 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 59 IP - 5 DP - 2020 May 1 TI - Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study. PG - 1051-1058 LID - 10.1093/rheumatology/kez374 [doi] AB - OBJECTIVE: The aim of this observational study was to investigate the evolution of scleroderma microangiopathy throughout different nailfold videocapillaroscopy (NVC) patterns ('early', 'active', 'late') as well as the prevalence of organ involvement in SSc patients during a 12-year follow-up. METHODS: Thirty-four SSc patients showing at baseline (first capillaroscopic analysis) the 'early' NVC pattern of microangiopathy were enrolled and followed for 12 years (s.d. 2). Complete NVC analysis and clinical and serological findings were collected. Patients were in a standard therapeutic care setting. Statistical analysis was carried out by non-parametric tests. RESULTS: After a 12-year follow-up, the 'early' NVC pattern changed from baseline in 76% of the patients. The NVC pattern was found to be 'active' in 9 patients (26%), 'late' in 13 (38%) and characterized by non-specific capillary abnormalities in 4 (12%). In the subgroup whose microangiopathy progressed from the 'early' to the 'late' NVC pattern, the median time of progression from the 'early' to the 'active' pattern was significantly shorter (11 months) when compared with patients who progressed from the 'early' to the 'active' NVC pattern (55 months) (P = 0.002). The median time of progression between NVC patterns was significantly shorter in SSc patients showing either a nucleolar ANA pattern or Scl70 autoantibodies (P = 0.048). Organ involvement was progressively greater in SSc patients with 'early', 'active' and 'late' NVC patterns, respectively. CONCLUSIONS: This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting. CI - © The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Sulli, A AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. FAU - Paolino, S AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. FAU - Pizzorni, C AU - Pizzorni C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. FAU - Ferrari, G AU - Ferrari G AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. FAU - Pacini, G AU - Pacini G AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. FAU - Pesce, G AU - Pesce G AD - Laboratory for Autoimmunity, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genova. FAU - Carmisciano, L AU - Carmisciano L AD - Biostatistics Section, Department of Health Sciences, University of Genova, Genova, Italy. FAU - Smith, V AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, VIB Inflammation Research Centre - Ghent University, Ghent, Belgium. FAU - Cutolo, M AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine. LA - eng PT - Journal Article PT - Observational Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) SB - IM EIN - Rheumatology (Oxford). 2020 May 1;59(5):1192. doi: 10.1093/rheumatology/keaa226. PMID: 32344434 MH - Autoantibodies/immunology MH - *Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Longitudinal Studies MH - Male MH - Microscopic Angioscopy/*methods MH - Nail Diseases/*diagnostic imaging/etiology MH - Nails/*blood supply MH - Retrospective Studies MH - Risk Assessment MH - Scleroderma, Systemic/*complications/diagnosis/*immunology MH - Severity of Illness Index MH - Time Factors OTO - NOTNLM OT - Raynaud’s phenomenon OT - autoantibodies OT - connective tissue diseases OT - microangiopathy OT - nailfold videocapillaroscopy OT - patterns OT - systemic sclerosis EDAT- 2019/11/22 06:00 MHDA- 2020/08/29 06:00 CRDT- 2019/11/22 06:00 PHST- 2019/04/17 00:00 [received] PHST- 2019/07/18 00:00 [revised] PHST- 2019/11/22 06:00 [pubmed] PHST- 2020/08/29 06:00 [medline] PHST- 2019/11/22 06:00 [entrez] AID - 5571129 [pii] AID - 10.1093/rheumatology/kez374 [doi] PST - ppublish SO - Rheumatology (Oxford). 2020 May 1;59(5):1051-1058. doi: 10.1093/rheumatology/kez374. PMID- 30840212 OWN - NLM STAT- MEDLINE DCOM- 20191217 LR - 20210210 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 7 DP - 2019 Jul TI - Validation of Turkish version of the Scleroderma Health Assessment Questionnaire. PG - 1917-1923 LID - 10.1007/s10067-019-04494-5 [doi] AB - OBJECTIVES: The Scleroderma Health Assessment Questionnaire (SHAQ) is a functional scale which consists of five scleroderma-specific items (overall disease severity, Raynaud's phenomenon, digital ulcers, respiratory and intestinal involvement) in addition to Health Assessment Questionnaire Disability Index (HAQ-DI). The objective of this study was to perform an adaptation and validation of a Turkish version of the SHAQ. METHOD: We validated psychometric properties of the scale with 70 consecutive systemic sclerosis (SSc) patients, who fulfilled the 2013 ACR/EULAR classification criteria for SSc. We evaluated test-retest reliability with the intraclass correlation coefficient (ICC), discriminant validity by stratifying patients according to organ involvements and disease subtypes, and convergent validity by testing the correlation between SHAQ and related components of Short Form 36 version 2 (SF-36v2). Internal consistency of the questionnaire was evaluated by Cronbach's alpha coefficient. RESULTS: The SHAQ-global, visual analogue scales (VAS) of pulmonary, digital ulcer, and Raynaud's phenomenon were significantly correlated with the physical component score of the SF-36v2 (r = - 0.274, r = - 0.295, r = - 0.326, r = - 0.308, p < 0.05, respectively) for the convergent validity. The instruments could not discriminate between disease subtypes, except the digital ulcer VAS which was significantly higher in patients with dcSSc (1.00 ± 0.93 vs 0.55 ± 0.88, p = 0.026) for the discriminant validity. The HAQ-DI, SHAQ-global, digital ulcer VAS, and pulmonary VAS showed moderate correlation with an increase in the number of the organs involved (r = 0.319, r = 0.329, r = 0.341, r = 0.278, p < 0.05, respectively). We demonstrated high reproducibility for HAQ-DI (ICC = 0.962, 95% confidence interval = 0.934-0.978) and the other items of SHAQ. The overall internal consistency of the SHAQ was satisfactory (Cronbach's alpha = 0.953). CONCLUSIONS: The Turkish version of the SHAQ met the requirements of validity and reproducibility. FAU - Temiz Karadag, Duygu AU - Temiz Karadag D AUID- ORCID: 0000-0002-5891-2032 AD - Department of Rheumatology, Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. dr_dtemiz@hotmail.com. FAU - Karakas, Fatih AU - Karakas F AD - Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. FAU - Tekeoglu, Senem AU - Tekeoglu S AD - Department of Rheumatology, Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. FAU - Yazici, Ayten AU - Yazici A AD - Department of Rheumatology, Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. FAU - Isik, Ozlem Ozdemir AU - Isik OO AD - Department of Rheumatology, Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. FAU - Cefle, Ayse AU - Cefle A AD - Department of Rheumatology, Kocaeli University School of Medicine, 41380, İzmit, Kocaeli, Turkey. LA - eng PT - Journal Article PT - Validation Study DEP - 20190306 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Cultural Characteristics MH - Disability Evaluation MH - Female MH - Humans MH - Language MH - Male MH - Middle Aged MH - Pain Measurement MH - Psychometrics MH - Quality of Life MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis/psychology MH - Severity of Illness Index MH - *Surveys and Questionnaires MH - Turkey MH - Visual Analog Scale OTO - NOTNLM OT - Scleroderma Health Assessment Questionnaire Disability Index OT - Systemic sclerosis OT - Turkish OT - Validation EDAT- 2019/03/07 06:00 MHDA- 2019/12/18 06:00 CRDT- 2019/03/07 06:00 PHST- 2018/11/14 00:00 [received] PHST- 2019/02/24 00:00 [accepted] PHST- 2019/02/10 00:00 [revised] PHST- 2019/03/07 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2019/03/07 06:00 [entrez] AID - 10.1007/s10067-019-04494-5 [pii] AID - 10.1007/s10067-019-04494-5 [doi] PST - ppublish SO - Clin Rheumatol. 2019 Jul;38(7):1917-1923. doi: 10.1007/s10067-019-04494-5. Epub 2019 Mar 6. PMID- 20360189 OWN - NLM STAT- MEDLINE DCOM- 20100708 LR - 20110302 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 6 DP - 2010 Jun TI - Secondary Sjogren's syndrome in systemic lupus erythematosus defines a distinct disease subset. PG - 1143-9 LID - 10.3899/jrheum.090804 [doi] AB - OBJECTIVE: Sjögren's syndrome (SS) may occur in patients with systemic lupus erythematosus (SLE). We sought to determine whether the presence of SS in a large cohort of patients with SLE defines a subset with distinctive sociodemographic, clinical, and laboratory features. METHODS: The Johns Hopkins Lupus Cohort was divided into 2 groups, based on the presence or absence of SS, defined by the presence of an objective measure of sicca or an abnormal minor salivary gland biopsy in a patient with sicca symptoms. These groups were compared with regard to sociodemographic, clinical, and laboratory features. Multivariable logistic regression was then performed to adjust the findings for potential sociodemographic, clinical, and laboratory confounders. RESULTS: The 259 patients with SS (14% of the cohort), when compared with the 1531 patients without SS, were older at the time of SLE diagnosis and were more commonly women and white. Photosensitivity, oral ulcers, Raynaud's phenomenon, anti-Ro antibodies, and anti-La antibodies had a significant positive association while renal disease, anti-ribonucleoprotein (RNP) antibodies, and anti-dsDNA antibodies had a negative association with the presence of SS after adjustment for age (at last cohort visit), gender, ethnicity, and anti-Ro antibodies. The older age at diagnosis of SLE among the patients with SS did not remain a significant finding after adjustment for the age of the patient at last cohort visit. CONCLUSION: The subset of patients with SLE and SS has a distinct clinical and laboratory phenotype, with a higher frequency of older white women with photosensitivity, oral ulcers, Raynaud's phenomenon, anti-Ro antibodies, and anti-La antibodies and a lower frequency of renal disease, anti-dsDNA antibodies, and anti-RNP antibodies. FAU - Baer, Alan N AU - Baer AN AD - Division of Rheumatology, Good Samaritan Hospital, Russell Morgan Building, Suite 508, 5601 Loch Raven Blvd., Baltimore, MD 21239, USA. alanbaer@jhmi.edu FAU - Maynard, Janet W AU - Maynard JW FAU - Shaikh, Fasil AU - Shaikh F FAU - Magder, Laurence S AU - Magder LS FAU - Petri, Michelle AU - Petri M LA - eng GR - AR 43727/AR/NIAMS NIH HHS/United States GR - MO1-RR00052/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20100401 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2011 Feb;38(2):393. doi: 10.3899/jrheum.100720. PMID: 21285180 MH - Adult MH - Cohort Studies MH - Comorbidity MH - Female MH - Humans MH - Logistic Models MH - Lupus Erythematosus, Systemic/*epidemiology/pathology/physiopathology MH - Male MH - Maryland/epidemiology MH - Multivariate Analysis MH - Sjogren's Syndrome/*epidemiology/pathology/physiopathology EDAT- 2010/04/03 06:00 MHDA- 2010/07/09 06:00 CRDT- 2010/04/03 06:00 PHST- 2010/04/03 06:00 [entrez] PHST- 2010/04/03 06:00 [pubmed] PHST- 2010/07/09 06:00 [medline] AID - jrheum.090804 [pii] AID - 10.3899/jrheum.090804 [doi] PST - ppublish SO - J Rheumatol. 2010 Jun;37(6):1143-9. doi: 10.3899/jrheum.090804. Epub 2010 Apr 1. PMID- 28049963 OWN - NLM STAT- MEDLINE DCOM- 20170316 LR - 20171106 IS - 1349-7413 (Electronic) IS - 0911-4300 (Linking) VI - 39 IP - 6 DP - 2016 TI - [A case of anti-aminoacyl tRNA synthetase antibody syndrome complicated by hemophagocytic syndrome]. PG - 538-544 LID - 10.2177/jsci.39.538 [doi] AB - A 48-year-old woman had suffered from a fever and general fatigue, and visited the other hospital for fever elevation in November 2013, at which time interstitial lung disease was revealed. In January 2014, she experienced an eruption in the hand and developed peripheral blood flow damage. Under a diagnosis of adult Still's disease, the patient was administered 0.5 mg of betamethasone as well as cyclosporin at 75 mg/day. In November 2014, general fatigue, fever, and headache were noted, while MRI revealed an enlarged hypophysis and laboratory findings were positive for the anti-pituitary cell antibody, thus a diagnosis of autoimmune hypophysitis was made. Although disease activity was low, she requested hospitalization and was admitted by the Division of Endocrinology and Metabolism at our hospital in May 2015, though only observed. Fever developed again, along with interstitial lung disease, Raynaud's phenomenon, and pain in the crural area again, and we considered the possibility of another disease. After stopping administration of betamethasone and cyclosporin, we made a diagnosis of anti-aminoacyl tRNA synthetase antibody syndrome, and administered methylprednisolone at 500 mg for 3 days as well as prednisolone at 35 mg/day following steroid pulse therapy. Although her condition soon improved, fever, muscle pain, and pancytopenia returned after 3 days. Bone marrow findings revealed the existence of hemophagocytosis, for which we again gave methylprednisolone at 500 mg for 3 days and cyclosporin at 125 mg/day. Thereafter, the patient recovered and was discharged from the hospital. FAU - Azuma, Kota AU - Azuma K AD - Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine. FAU - Tamura, Masao AU - Tamura M FAU - Kurajoh, Masafumi AU - Kurajoh M FAU - Hosono, Yuji AU - Hosono Y FAU - Nakajima, Ran AU - Nakajima R FAU - Tsuboi, Kazuyuki AU - Tsuboi K FAU - Abe, Takeo AU - Abe T FAU - Ogita, Chie AU - Ogita C FAU - Yokoyama, Yuichi AU - Yokoyama Y FAU - Furukawa, Tetsuya AU - Furukawa T FAU - Yoshikawa, Takahiro AU - Yoshikawa T FAU - Saito, Atsushi AU - Saito A FAU - Nishioka, Aki AU - Nishioka A FAU - Sekiguchi, Masahiro AU - Sekiguchi M FAU - Azuma, Naoto AU - Azuma N FAU - Kitano, Masayasu AU - Kitano M FAU - Tsunoda, Shinichiro AU - Tsunoda S FAU - Omura, Koichiro AU - Omura K FAU - Koyama, Hidenori AU - Koyama H FAU - Matsui, Kiyoshi AU - Matsui K FAU - Mimori, Tsuneyo AU - Mimori T FAU - Sano, Hajime AU - Sano H LA - jpn PT - Case Reports PT - Journal Article PT - Review PL - Japan TA - Nihon Rinsho Meneki Gakkai Kaishi JT - Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology JID - 9505992 RN - 83HN0GTJ6D (Cyclosporine) RN - 9PHQ9Y1OLM (Prednisolone) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoimmune Diseases/*complications/*diagnosis/drug therapy MH - Cyclosporine/administration & dosage MH - Diagnosis, Differential MH - Drug Therapy, Combination MH - Female MH - Humans MH - Lymphohistiocytosis, Hemophagocytic/*complications/*diagnosis/drug therapy MH - Methylprednisolone/administration & dosage MH - Middle Aged MH - Prednisolone/administration & dosage MH - Pulse Therapy, Drug MH - Syndrome MH - Treatment Outcome EDAT- 2017/01/05 06:00 MHDA- 2017/03/17 06:00 CRDT- 2017/01/05 06:00 PHST- 2017/01/05 06:00 [entrez] PHST- 2017/01/05 06:00 [pubmed] PHST- 2017/03/17 06:00 [medline] AID - 10.2177/jsci.39.538 [doi] PST - ppublish SO - Nihon Rinsho Meneki Gakkai Kaishi. 2016;39(6):538-544. doi: 10.2177/jsci.39.538. PMID- 22674907 OWN - NLM STAT- MEDLINE DCOM- 20130107 LR - 20260518 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 64 IP - 11 DP - 2012 Nov TI - Comparison of children with onset of juvenile dermatomyositis symptoms before or after their fifth birthday in a UK and Ireland juvenile dermatomyositis cohort study. PG - 1665-72 LID - 10.1002/acr.21753 [doi] AB - OBJECTIVE: To compare 2 groups of children with juvenile dermatomyositis (DM), those with onset of symptoms before their fifth birthday versus those whose disease begins either on or after their fifth birthday, and to assess whether age at onset is associated with differences in disease presentation, treatments received, or outcomes 2 years after diagnosis. METHODS: Data were analyzed on children recruited to a UK juvenile DM cohort study with a diagnosis of probable or definite juvenile DM and less than 12 months between diagnosis and recruitment. RESULTS: Fifty-five (35%) of 157 children had onset of symptoms before their fifth birthday. At diagnosis, cutaneous ulceration was found in 32.7% of the younger group versus 11.8% of the older group (P = 0.003). Facial or body swelling was reported more often in the younger group, whereas headaches, alopecia, and Raynaud's phenomenon were all more frequently reported in the older group. At followup 2 years later, there were no important differences in outcomes between the groups. More than 90% of patients in both groups received both methotrexate and steroids. Twenty-three percent of both groups remained on steroids 2 years after diagnosis. CONCLUSION: Our study showed that children with juvenile DM with disease onset at age <5 years are more likely to present with ulcerative skin disease and edema. There were no clinically significant differences in outcomes between the 2 groups. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Martin, N AU - Martin N AD - Great Ormond Street Hospital, London, UK. neil.martin3@nhs.net FAU - Krol, P AU - Krol P FAU - Smith, S AU - Smith S FAU - Beard, L AU - Beard L FAU - Pilkington, C A AU - Pilkington CA FAU - Davidson, J AU - Davidson J FAU - Wedderburn, L R AU - Wedderburn LR CN - Juvenile Dermatomyositis Research Group (JDRG) LA - eng GR - 18474/VAC_/Versus Arthritis/United Kingdom GR - 085860/WT_/Wellcome Trust/United Kingdom PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - Amyopathic dermatomyositis SB - IM MH - Adolescent MH - Age Distribution MH - Age of Onset MH - Alopecia/epidemiology MH - Child MH - Child, Preschool MH - Cohort Studies MH - Dermatomyositis/*epidemiology/*pathology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Headache/epidemiology MH - Humans MH - Incidence MH - Infant MH - Ireland/epidemiology MH - Male MH - *Severity of Illness Index MH - Skin Ulcer/epidemiology/pathology MH - United Kingdom/epidemiology PMC - PMC3533762 FIR - McCann, Liza IR - McCann L FIR - Roberts, Ian IR - Roberts I FIR - Riley, Phil IR - Riley P FIR - Baildam, Eileen IR - Baildam E FIR - Ryder, Clive IR - Ryder C FIR - Scott, Janis IR - Scott J FIR - Wyatt, Sue IR - Wyatt S FIR - Jackson, Gillian IR - Jackson G FIR - Davidson, Joyce IR - Davidson J FIR - Gardner-Medwin, Janet IR - Gardner-Medwin J FIR - Ferguson, Sue IR - Ferguson S FIR - Friswell, Mark IR - Friswell M FIR - Foster, Helen IR - Foster H FIR - Swift, Alison IR - Swift A FIR - Venning, Helen IR - Venning H FIR - Hutchinson, Elizabeth IR - Hutchinson E FIR - Wedderburn, Lucy R IR - Wedderburn LR FIR - Pilkington, Clarissa A IR - Pilkington CA FIR - Hasson, N IR - Hasson N FIR - Maillard, Sue IR - Maillard S FIR - Halkon, Elizabeth IR - Halkon E FIR - Brown, Virginia IR - Brown V FIR - Juggins, Audrey IR - Juggins A FIR - Smith, Sally IR - Smith S FIR - Beard, Laura IR - Beard L FIR - Evans, Sian IR - Evans S FIR - Enayat, Elli IR - Enayat E FIR - Murray, Kevin IR - Murray K EDAT- 2012/06/08 06:00 MHDA- 2013/01/08 06:00 CRDT- 2012/06/08 06:00 PHST- 2012/06/08 06:00 [entrez] PHST- 2012/06/08 06:00 [pubmed] PHST- 2013/01/08 06:00 [medline] AID - 10.1002/acr.21753 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 Nov;64(11):1665-72. doi: 10.1002/acr.21753. PMID- 20620090 OWN - NLM STAT- MEDLINE DCOM- 20110707 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 78 IP - 2 DP - 2011 Mar TI - Elderly onset of primary Sjögren's syndrome: clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients. PG - 171-4 LID - 10.1016/j.jbspin.2010.05.008 [doi] AB - OBJECTIVES: To study and compare the clinical and serological features of patients with elderly versus adult and younger onset of primary Sjögren's syndrome (pSS). METHODS: We analyzed retrospectively 336 consecutive pSS patients followed at our unit. They were subdivided into three groups according to the age at disease onset: elderly (>65 years), adult (>40 and ≤65 years), and young (≤40 years). Clinical and immunological features of the disease, labial salivary glands biopsy, ocular and oral tests were collected at time of diagnosis and then compared among the three groups. RESULTS: In 21 (6%) patients, disease onset occurred after the age of 65 years. At the time of diagnosis, 15 (71.4%) of these patients reported symptoms of dry mouth and 16 (76.1%) of dry eye. The most common extraglandular manifestation were arthralgias in 14 (66.7%), Raynaud's phenomenon in five (23.8%) and purpura in three (14.2%) cases. Ocular diagnostic tests (Schirmer's I and Rose-Bengal staining) were positive respectively in 17 (80%) and nine (44.4%) patients. In eight (38%) cases, unstimulated whole salivary flow showed normal values, while 12 patients (57.1%) showed positivity for salivary sialography. A focus score greater or equal to 1 per 4mm(2) was demonstrated in 11 (53.3%) of the 21 cases. CONCLUSION: Elderly onset of pSS was associated with similar incidence of the diagnostic tests positivity (parotid sialography, ocular tests, minor salivary gland biopsy) in comparison with adult and younger onset. Moreover, no statistical differences were found among the three groups concerning sex, disease duration, as well as ocular and oral symptoms. CI - Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Botsios, Costantino AU - Botsios C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, via Giustiniani 2, 35128 Padova, Italy. constantin.botsios@unipd.it FAU - Furlan, Antonio AU - Furlan A FAU - Ostuni, Pierantonio AU - Ostuni P FAU - Sfriso, Paolo AU - Sfriso P FAU - Andretta, Marilisa AU - Andretta M FAU - Ometto, Francesca AU - Ometto F FAU - Raffeiner, Bernd AU - Raffeiner B FAU - Todesco, Silvano AU - Todesco S FAU - Punzi, Leonardo AU - Punzi L LA - eng PT - Comparative Study PT - Journal Article PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Age Factors MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Autoantibodies/blood MH - Biopsy MH - Cohort Studies MH - *Diagnostic Tests, Routine MH - Eye/pathology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Mouth/pathology MH - Retrospective Studies MH - Salivary Glands/pathology MH - Sjogren's Syndrome/*diagnosis/*epidemiology MH - Young Adult EDAT- 2010/07/14 06:00 MHDA- 2011/07/08 06:00 CRDT- 2010/07/13 06:00 PHST- 2009/12/23 00:00 [received] PHST- 2010/05/20 00:00 [accepted] PHST- 2010/07/13 06:00 [entrez] PHST- 2010/07/14 06:00 [pubmed] PHST- 2011/07/08 06:00 [medline] AID - S1297-319X(10)00163-6 [pii] AID - 10.1016/j.jbspin.2010.05.008 [doi] PST - ppublish SO - Joint Bone Spine. 2011 Mar;78(2):171-4. doi: 10.1016/j.jbspin.2010.05.008. PMID- 19958295 OWN - NLM STAT- MEDLINE DCOM- 20100527 LR - 20151119 IS - 1755-3768 (Electronic) IS - 1755-375X (Linking) VI - 88 IP - 1 DP - 2010 Feb TI - Systemic disease associations of familial and sporadic glaucoma: the Glaucoma Inheritance Study in Tasmania. PG - 70-4 LID - 10.1111/j.1755-3768.2009.01786.x [doi] AB - BACKGROUND: This aim of this study was to compare the prevalence of various disease-associated and potentially modifiable risk factors between people with familial and sporadic forms of primary open angle glaucoma (OAG). METHODS: A cross-sectional, retrospective study design was utilized. A detailed questionnaire enquiring about knowledge of family history, demographic data, current medications, and medical history of systemic disorders was administered. Where possible, living relatives were examined for signs of OAG. RESULTS: A total of 3,800 potential patients with OAG were identified, of whom 2062 were examined. One thousand twelve (59.5%) subjects were found to have familial OAG, and 688 (40.5%) subjects had no known or identified relative with OAG (sporadic glaucoma). One thousand forty-two unaffected family members examined. A past history of migraine was found more often with familial OAG (OR: 1.67 95% CI: 1.15-2.42). This effect was primarily driven by patients who had a first-degree relative also affected by OAG. Following adjustment for male gender and the age at review, the presence of atherosclerosis was also found to be more common in patients with familial glaucoma than in people with sporadic disease (OR: 1.42 95% CI: 1.05-1.92). No significant difference in the prevalence of hypertension, Raynaud's phenomenon, diabetes mellitus or thyroid disease was identified. CONCLUSIONS: Patients with a known relative affected by OAG were statistically significantly more likely to have a past history for migraine or presence of atherosclerosis compared to people with no known affected relative. An understanding of such differences and systemic comorbidities will be useful for further work investigating the underlying molecular mechanisms of this disease. FAU - Hewitt, Alex W AU - Hewitt AW AD - Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, Melbourne, Australia. hewitt.alex@gmail.com FAU - Wu, Johnny AU - Wu J FAU - Green, Catherine M AU - Green CM FAU - Lai, Tze AU - Lai T FAU - Kearns, Lisa S AU - Kearns LS FAU - Craig, Jamie E AU - Craig JE FAU - Mackey, David A AU - Mackey DA LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20091216 PL - England TA - Acta Ophthalmol JT - Acta ophthalmologica JID - 101468102 SB - IM MH - Aged MH - Aged, 80 and over MH - Atherosclerosis/*complications MH - Cross-Sectional Studies MH - Female MH - Glaucoma, Open-Angle/*complications/*genetics MH - Humans MH - Male MH - Medical Records MH - Middle Aged MH - Migraine Disorders/*complications MH - Retrospective Studies MH - Surveys and Questionnaires MH - Tasmania EDAT- 2009/12/05 06:00 MHDA- 2010/05/28 06:00 CRDT- 2009/12/05 06:00 PHST- 2009/12/05 06:00 [entrez] PHST- 2009/12/05 06:00 [pubmed] PHST- 2010/05/28 06:00 [medline] AID - AOS1786 [pii] AID - 10.1111/j.1755-3768.2009.01786.x [doi] PST - ppublish SO - Acta Ophthalmol. 2010 Feb;88(1):70-4. doi: 10.1111/j.1755-3768.2009.01786.x. Epub 2009 Dec 16. PMID- 16458168 OWN - NLM STAT- MEDLINE DCOM- 20060222 LR - 20220321 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 47 IP - 3 Suppl DP - 2006 Feb 7 TI - Some thoughts on the vasculopathy of women with ischemic heart disease. PG - S30-5 AB - Considerable experimental and clinical data indicate that sex has an important influence on cardiovascular physiology and pathology. This report integrates selected literature with new data from the Women's Ischemia Syndrome Evaluation (WISE) on vascular findings in women with ischemic heart disease (IHD) and how these findings differ from those in men. A number of common vascular disease-related conditions are either unique to (e.g., hypertensive disorders of pregnancy, gestational diabetes, peripartum dissection, polycystic ovarian syndrome, etc.) or more frequent (e.g., migraine, coronary spasm, lupus, vasculitis, Raynaud's phenomenon, etc.) in women than men. Post-menopausal women more frequently have many traditional vascular disease risk conditions (e.g., hypertension, diabetes, obesity, inactivity, and so on), and these conditions cluster more frequently in them than men. Considerable evidence supports the notion that, with these requisite conditions, women develop a more severe or somewhat different form of vascular disease than men. Structurally, women's coronary vessels are smaller in size and appear to contain more diffuse atherosclerosis, their aortas are stiffer (fibrosis, remodeling, and so on), and their microvessels appear to be more frequently dysfunctional compared with men. Functionally, women's vessels frequently show impaired vasodilator responses. Limitations of existing data and higher risks in women with acute myocardial infarction, need for revascularization, or heart failure create uncertainty about management. A better understanding of these findings should provide direction for new algorithms to improve management of the vasculopathy underlying IHD in women. FAU - Pepine, Carl J AU - Pepine CJ AD - Division of Cardiovascular Medicine, Department of Medicine, University of Florida College of Medicine, Gainesville, Florida, USA. pepincj@medicine.ufl.edu FAU - Kerensky, Richard A AU - Kerensky RA FAU - Lambert, Charles R AU - Lambert CR FAU - Smith, Karen M AU - Smith KM FAU - von Mering, Gregory O AU - von Mering GO FAU - Sopko, George AU - Sopko G FAU - Bairey Merz, C Noel AU - Bairey Merz CN LA - eng GR - M01-RR00425/RR/NCRR NIH HHS/United States GR - N01-HV-68161/HV/NHLBI NIH HHS/United States GR - N01-HV-68162/HV/NHLBI NIH HHS/United States GR - N01-HV-68163/HV/NHLBI NIH HHS/United States GR - N01-HV-68164/HV/NHLBI NIH HHS/United States GR - U01 HL649141/HL/NHLBI NIH HHS/United States GR - U01 HL649241/HL/NHLBI NIH HHS/United States GR - U0164829/PHS HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 SB - IM MH - Cardiovascular System MH - Endothelium, Vascular/physiology MH - Female MH - Humans MH - Muscle, Smooth, Vascular/physiology MH - Myocardial Ischemia/*physiopathology MH - Risk Factors MH - Sex Factors RF - 64 EDAT- 2006/02/07 09:00 MHDA- 2006/02/24 09:00 CRDT- 2006/02/07 09:00 PHST- 2005/09/21 00:00 [received] PHST- 2005/09/29 00:00 [accepted] PHST- 2006/02/07 09:00 [pubmed] PHST- 2006/02/24 09:00 [medline] PHST- 2006/02/07 09:00 [entrez] AID - S0735-1097(05)02508-8 [pii] AID - 10.1016/j.jacc.2005.09.023 [doi] PST - ppublish SO - J Am Coll Cardiol. 2006 Feb 7;47(3 Suppl):S30-5. doi: 10.1016/j.jacc.2005.09.023. PMID- 27487743 OWN - NLM STAT- MEDLINE DCOM- 20170327 LR - 20260127 IS - 1349-3329 (Electronic) IS - 0040-8727 (Linking) VI - 239 IP - 4 DP - 2016 Aug TI - High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis. PG - 297-305 LID - 10.1620/tjem.239.297 [doi] AB - Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrosis and autoantibodies. Its clinical manifestations are diverse and include Raynaud's phenomenon, gastrointestinal dysmotility, interstitial lung disease (ILD), pulmonary hypertension, and renal crisis. Among these, ILD is the primary cause of SSc-related death. It has been considered that acute exacerbation of ILD (AE-ILD) is not common in patients with SSc; however, little is known about the prevalence of AE-ILD in Japanese patients with SSc. In this study, we aimed to clarify the prevalence, clinical characteristics, and prognosis of patients with SSc who developed AE-ILD and to identify predictive factors for AE-ILD in our Japanese cohorts. Clinical data of patients who visited our department from 1990 to 2014 and fulfilled the 2013 classification criteria for SSc were retrospectively reviewed. A total of 139 patients were enrolled. The mean age of onset was 49.1 years, and 113 (81.3%) patients were female; 116 (83.5%) had limited cutaneous involvement, and the overall 10-year survival rate was 92.0%. Among 66 (47.5%) patients with ILD, 13 (9.4%) developed AE-ILD. Patients with AE-ILD had a significantly higher incidence of overlap with polymyositis (PM) or dermatomyositis (DM) and lower prevalence of anticentromere antibodies with higher mortality rate compared with those without AE-ILD. Multivariate Cox regression analysis identified that an overlap with PM or DM was the most significant predictive factor for AE-ILD. Our study results suggest that Japanese patients with SSc, particularly patients overlapped with PM or DM, have a high risk of AE-ILD. FAU - Tomiyama, Fumiko AU - Tomiyama F AD - Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine. FAU - Watanabe, Ryu AU - Watanabe R FAU - Ishii, Tomonori AU - Ishii T FAU - Kamogawa, Yukiko AU - Kamogawa Y FAU - Fujita, Yoko AU - Fujita Y FAU - Shirota, Yuko AU - Shirota Y FAU - Sugimura, Koichiro AU - Sugimura K FAU - Fujii, Hiroshi AU - Fujii H FAU - Harigae, Hideo AU - Harigae H LA - eng PT - Journal Article PL - Japan TA - Tohoku J Exp Med JT - The Tohoku journal of experimental medicine JID - 0417355 SB - IM MH - Acute Disease MH - Adult MH - Demography MH - *Disease Progression MH - Female MH - Humans MH - Japan/epidemiology MH - Lung Diseases, Interstitial/*complications/diagnostic imaging/*epidemiology/mortality MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Prevalence MH - Proportional Hazards Models MH - Scleroderma, Systemic/*complications/diagnostic imaging/*epidemiology/mortality MH - Survival Rate MH - Treatment Outcome EDAT- 2016/08/05 06:00 MHDA- 2017/03/28 06:00 CRDT- 2016/08/05 06:00 PHST- 2016/08/05 06:00 [entrez] PHST- 2016/08/05 06:00 [pubmed] PHST- 2017/03/28 06:00 [medline] AID - 10.1620/tjem.239.297 [doi] PST - ppublish SO - Tohoku J Exp Med. 2016 Aug;239(4):297-305. doi: 10.1620/tjem.239.297. PMID- 40649115 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250714 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 13 DP - 2025 Jul 4 TI - Insights into Pulmonary Arterial Hypertension in Connective Tissue Diseases. LID - 10.3390/jcm14134742 [doi] LID - 4742 AB - Pulmonary arterial hypertension (PAH) is a severe complication associated with connective tissue diseases (CTDs), which is characterized by a significant influence on the patient's prognosis and mortality. The prevalence of PAH varies depending on the type of CTD. Still, it is highly prevalent in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), and primary Sjögren's syndrome (pSS). Identifying rheumatic disease-specific risk factors is crucial for early diagnosis and intervention. Risk factors for PAH development include specific sociological factors (related to race, gender, and age), clinical features (particularly severe Raynaud's phenomenon and multiple telangiectasias), cardiological factors (pericarditis and left heart disease), biochemical factors (elevated NT-proBNP and decreased HDL-cholesterol), serological factors (presence of ANA, e.g., anti-U1-RNP or SSA, and antiphospholipid antibodies), and pulmonary factors (interstitial lung disease and decreased DLCO or DLCO/alveolar volume ratio < 70%, FVC/DLCO > 1.6). The analysis of risk factors can be the most useful during the selection of patients at high risk of PAH development. The initial diagnosis of PAH is usually based on transthoracic echocardiography (TTE) and is finally confirmed by right heart catheterization (RHC). Targeted therapies can improve outcomes and include endothelin receptor antagonists, prostacyclin analogs, phosphodiesterase inhibitors, and tailored immunosuppressive treatments. Effective management strategies require a multidisciplinary approach involving rheumatologists, cardiologists, and pulmonologists. The risk stratification and individualized treatment strategies can enhance survival and quality of life in patients with PAH-CTD. FAU - Grygiel-Górniak, Bogna AU - Grygiel-Górniak B AUID- ORCID: 0000-0002-3438-0764 AD - Department of Rheumatology, Rehabilitation and Internal Diseases, Poznan University of Medical Sciences, 61-701 Poznań, Poland. FAU - Lucki, Mateusz AU - Lucki M AD - Department and Clinic of Cardiology, University of Medical Sciences, 60-545 Poznań, Poland. FAU - Daroszewski, Przemysław AU - Daroszewski P AD - Department of Organization and Management in Healthcare, Poznań University of Medical Sciences, 60-545 Poznań, Poland. FAU - Lucka, Ewa AU - Lucka E AUID- ORCID: 0000-0002-8248-2247 AD - Clinical Rehabilitation Laboratory, Department of Rehabilitation and Physiotherapy, University of Medical Sciences, 60-545 Poznań, Poland. LA - eng PT - Journal Article PT - Review DEP - 20250704 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC12250367 OTO - NOTNLM OT - connective tissue diseases OT - pulmonary arterial hypertension OT - risk factors OT - treatment strategies COIS- The authors declare that they have no financial or non-financial interests that could influence the outcomes of this study. There are no competing interests to report. None of the authors has any conflicts of interest to disclose. EDAT- 2025/07/13 03:32 MHDA- 2025/07/13 03:33 PMCR- 2025/07/04 CRDT- 2025/07/12 01:16 PHST- 2025/04/25 00:00 [received] PHST- 2025/06/12 00:00 [revised] PHST- 2025/07/02 00:00 [accepted] PHST- 2025/07/13 03:33 [medline] PHST- 2025/07/13 03:32 [pubmed] PHST- 2025/07/12 01:16 [entrez] PHST- 2025/07/04 00:00 [pmc-release] AID - jcm14134742 [pii] AID - jcm-14-04742 [pii] AID - 10.3390/jcm14134742 [doi] PST - epublish SO - J Clin Med. 2025 Jul 4;14(13):4742. doi: 10.3390/jcm14134742. PMID- 41799671 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260309 LR - 20260309 IS - 2589-5141 (Electronic) IS - 2589-5141 (Linking) VI - 8 IP - 3 DP - 2026 May TI - Enhancing Hand Vascular Assessment: The Role of Hyperspectral Imaging in Perfusion Monitoring. PG - 100980 LID - 10.1016/j.jhsg.2026.100980 [doi] LID - 100980 AB - PURPOSE: Accurate assessment and monitoring of tissue perfusion are critical in diagnosing and managing vascular diseases of the hand. Traditional methods, including clinical examination, pulse oximetry, and Doppler ultrasound, have limitations in detecting early or subtle perfusion deficits. Hyperspectral imaging (HSI) is a noninvasive, real-time technology that generates quantitative perfusion maps by measuring oxygen saturation, oxyhemoglobin, and deoxyhemoglobin levels. To the best of our knowledge, this is the first study of its kind to evaluate the clinical use of HSI in assessing perfusion abnormalities in vascular conditions of the hand and its emerging applications in hand surgery. METHODS: A single center study was conducted from 2021 to 2025, involving 5 patients with various upper-extremity vascular conditions, each followed to early 2025. Perfusion images were obtained using the HyperView (HyperMed, Memphis, TN) system before and after surgical or procedural interventions. Results were compared against the contralateral hand or historical normal values from the prior-generation HyperView (OxyVu-1) system. RESULTS: Hyperspectral imaging effectively identified perfusion deficits, guided urgent clinical decision-making, and monitored postoperative improvements. In one case, HSI demonstrated a 5% increase in tissue oxygen saturation after ulnar artery reconstruction, correlating with successful wound healing. Another patient with Raynaud's phenomenon showed a 23% perfusion increase following botulinum toxin injection. Hyperspectral imaging also detected thrombosis and monitored perioperative oxygen saturation changes in real-time. CONCLUSIONS: Hyperspectral imaging offers a novel, objective approach to perfusion assessment in hand and upper-extremity vascular disease, addressing key limitations of traditional methods. Its integration into clinical workflows holds promise for enhancing real-time, data-driven decision-making in hand surgery and vascular medicine. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV. CI - © 2026 The Authors. FAU - Ganesan, Ovya AU - Ganesan O AD - Department of Plastic Surgery, Brigham and Women's Hospital, Boston, MA. AD - Geisel School of Medicine at Dartmouth, Hanover, NH. FAU - Trost, Jeffrey AU - Trost J AD - Department of Plastic Surgery, McGovern Medical School at UTHealth Houston, Houston, TX. AD - Department of Plastic Surgery, Memorial Hermann-Texas Medical Center, Houston, TX. FAU - Pike, Michael Drake AU - Pike MD AD - Harvard Medical School, Boston, MA. FAU - Sampson, Robert D AU - Sampson RD AD - Department of Plastic Surgery, Brigham and Women's Hospital, Boston, MA. AD - Department of Plastic Surgery, Renaissance School of Medicine at Stony Brook University, Stony Brook, NY. FAU - Holzmer, Stephanie AU - Holzmer S AD - Department of Plastic Surgery, Brigham and Women's Hospital, Boston, MA. AD - Harvard Medical School, Boston, MA. FAU - Talbot, Simon AU - Talbot S AD - Department of Plastic Surgery, Brigham and Women's Hospital, Boston, MA. AD - Harvard Medical School, Boston, MA. FAU - Sampson, Christian E AU - Sampson CE AD - Department of Plastic Surgery, Brigham and Women's Hospital, Boston, MA. AD - Harvard Medical School, Boston, MA. LA - eng PT - Journal Article DEP - 20260228 PL - United States TA - J Hand Surg Glob Online JT - Journal of hand surgery global online JID - 101759126 PMC - PMC12966670 OTO - NOTNLM OT - Hand disorders OT - HyperView OT - Hyperspectral imaging OT - Tissue perfusion OT - Vascular disease COIS- No benefits in any form have been received or will be received related directly to this article. EDAT- 2026/03/09 17:37 MHDA- 2026/03/09 17:38 PMCR- 2026/02/28 CRDT- 2026/03/09 06:20 PHST- 2025/12/01 00:00 [received] PHST- 2026/01/28 00:00 [accepted] PHST- 2026/03/09 17:38 [medline] PHST- 2026/03/09 17:37 [pubmed] PHST- 2026/03/09 06:20 [entrez] PHST- 2026/02/28 00:00 [pmc-release] AID - S2589-5141(26)00035-6 [pii] AID - 100980 [pii] AID - 10.1016/j.jhsg.2026.100980 [doi] PST - epublish SO - J Hand Surg Glob Online. 2026 Feb 28;8(3):100980. doi: 10.1016/j.jhsg.2026.100980. eCollection 2026 May. PMID- 41552126 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260119 LR - 20260121 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 12 DP - 2025 Dec TI - Pediatric Essential Thrombocythemia: A Case of a JAK2-Mutated Adolescent With Microvascular Symptoms. PG - e99370 LID - 10.7759/cureus.99370 [doi] LID - e99370 AB - Thrombocytosis, defined as platelet counts >450 × 10⁹/L, is frequent in the pediatric population and usually secondary to inflammatory conditions or iron deficiency. Essential thrombocythemia (ET), a Philadelphia chromosome-negative myeloproliferative neoplasm, is exceptionally rare in childhood. Pediatric ET often follows an indolent course but carries risks of thrombotic and hemorrhagic events, as well as late progression to myelofibrosis or leukemia. We report the case of a 14-year-old girl presenting with recurrent acral edema, erythema alternating with cyanosis, burning pain, paresthesia, and headaches. Physical examination was unremarkable. Initial suspicion of Raynaud's phenomenon was excluded by nailfold capillaroscopy. Laboratory studies revealed persistent thrombocytosis with platelets over 1,092 × 10⁹/L. Secondary causes were excluded. Bone marrow biopsy revealed megakaryocytic hyperplasia with hyperlobulated megakaryocytes, abdominal ultrasound revealed hepatosplenomegaly, and molecular testing identified a JAK2 V617F mutation, confirming ET. She was initially treated with low-dose acetylsalicylic acid, with partial improvement, but microvascular symptoms persisted, and platelet counts remained >1,000 × 10⁹/L. Hydroxyurea was initiated, leading to progressive platelet reduction and marked clinical benefit. Over three years of follow-up, the patient remained clinically stable, without adverse effects or leukemic transformation. This case illustrates the rarity and diagnostic complexity of pediatric ET, which requires exclusion of reactive causes, bone marrow evaluation, and molecular testing. Management remains particularly challenging due to the absence of pediatric-specific guidelines, with current approaches being largely derived from adult protocols. Cytoreductive therapy may be indicated in cases with extreme thrombocytosis or refractory symptoms, and long-term follow-up is crucial to monitor disease evolution and treatment outcomes. This case highlights the need for multicenter studies and international registries to have pediatric-specific evidence that can better inform diagnostic and therapeutic strategies. CI - Copyright © 2025, Fonseca et al. FAU - Fonseca, Madalena AU - Fonseca M AD - Pediatrics, Hospital Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, PRT. FAU - Cristóvão Ferreira, Ana AU - Cristóvão Ferreira A AD - Pediatric Hematology Unit, Hospital Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, PRT. FAU - Amaro Gonçalves, Carolina AU - Amaro Gonçalves C AD - Pediatric Hematology Unit, Hospital Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, PRT. FAU - Ferrão, Anabela AU - Ferrão A AD - Pediatric Hematology Unit, Hospital Santa Maria, Unidade Local de Saúde Santa Maria, Lisbon, PRT. LA - eng PT - Case Reports PT - Journal Article DEP - 20251216 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12805859 OTO - NOTNLM OT - acquired von willebrand disease OT - erythromelalgia OT - essential thrombocythemia OT - hydroxyurea OT - jak2 v617f mutation COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/01/19 12:51 MHDA- 2026/01/19 12:52 PMCR- 2025/12/16 CRDT- 2026/01/19 06:23 PHST- 2025/12/16 00:00 [accepted] PHST- 2026/01/19 12:52 [medline] PHST- 2026/01/19 12:51 [pubmed] PHST- 2026/01/19 06:23 [entrez] PHST- 2025/12/16 00:00 [pmc-release] AID - 10.7759/cureus.99370 [doi] PST - epublish SO - Cureus. 2025 Dec 16;17(12):e99370. doi: 10.7759/cureus.99370. eCollection 2025 Dec. PMID- 41502238 OWN - NLM STAT- MEDLINE DCOM- 20260406 LR - 20260609 IS - 1462-3994 (Electronic) IS - 1462-3994 (Linking) VI - 28 DP - 2026 Jan 8 TI - A Systematic Review of Autoimmunity in 22q11.2 Deletion Syndrome. PG - e12 LID - 10.1017/erm.2026.10031 [doi] LID - e12 AB - BACKGROUND: The 22q11.2 Deletion Syndrome (22q11DS) is the most common chromosomal microdeletion disorder, characterised by a heterogeneous clinical spectrum including immunodeficiency, autoimmunity, and neuropsychiatric comorbidities. This systematic review critically appraises current evidence on autoimmunity in 22q11DS, fulfilling the need for an unbiased and comprehensive synthesis of the current literature. METHODS: An extensive search was conducted through PubMed, Web of Science, EMBASE, CINAHL, and the Cochrane Library using Boolean combinations of relevant keywords. Qualitative studies, abstracts, conference proceedings and non-English studies were excluded. RESULTS: A total of 82 peer-reviewed studies published since 1968 were identified. We identified a total of 40 distinct autoimmune conditions involving multiple organ systems. Haematological disorders were most frequently cited, followed by autoimmune thyroid diseases and systemic autoimmune diseases. Less common conditions included coeliac disease, psoriasis, vitiligo, alopecia areata, Raynaud's phenomenon, and vasculitis, while 19 diseases appeared only as single-case reports. Neuropsychiatric manifestations were addressed in 24 studies. CONCLUSION: Our review confirms that autoimmunity is a complication of 22q11DS and highlights the need for epidemiological studies across organ-systems and inclusion of ethnically diverse populations. There was substantial variation in study designs, underscoring the need for more standardised approaches and larger sample sizes. FAU - Ogunsola, Hidayat Y AU - Ogunsola HY AUID- ORCID: 0009-0006-4357-2211 AD - Rollins School of Public Health, https://ror.org/03czfpz43Emory University, Atlanta, USA. FAU - Malik, Sana AU - Malik S AUID- ORCID: 0009-0007-7440-5632 AD - School of Medicine, Medical Sciences and Nutrition, https://ror.org/016476m91University of Aberdeen, UK. FAU - Rogers, Hannah AU - Rogers H AD - Library (Head of Information Services), Woodruff Health Sciences Center, https://ror.org/03czfpz43Emory University, Atlanta, USA. FAU - Pearce, Brad D AU - Pearce BD AUID- ORCID: 0000-0002-3259-4275 AD - Epidemiology, Rollins School of Public Health, https://ror.org/03czfpz43Emory University, Atlanta, USA. LA - eng GR - 1-R21-MH083138-01A1/NH/NIH HHS/United States PT - Journal Article PT - Review PT - Systematic Review DEP - 20260108 PL - England TA - Expert Rev Mol Med JT - Expert reviews in molecular medicine JID - 100939725 SB - IM MH - Humans MH - *DiGeorge Syndrome/immunology/complications/genetics/epidemiology MH - *Autoimmunity/genetics MH - *Autoimmune Diseases/etiology/genetics/immunology PMC - PMC13148428 OTO - NOTNLM OT - 22q.11DS OT - 22q11.2DS OT - DiGeorge OT - DiGeorge syndrome OT - autoimmune OT - autoimmune thyroid disease OT - autoimmunity OT - systematic review COIS- The authors declare that there are no competing interests relevant to this work. EDAT- 2026/01/08 07:12 MHDA- 2026/04/06 06:31 PMCR- 2026/05/06 CRDT- 2026/01/08 03:02 PHST- 2026/04/06 06:31 [medline] PHST- 2026/01/08 07:12 [pubmed] PHST- 2026/01/08 03:02 [entrez] PHST- 2026/05/06 00:00 [pmc-release] AID - S1462399426100313 [pii] AID - 10.1017/erm.2026.10031 [doi] PST - epublish SO - Expert Rev Mol Med. 2026 Jan 8;28:e12. doi: 10.1017/erm.2026.10031. PMID- 23745455 OWN - NLM STAT- MEDLINE DCOM- 20131217 LR - 20130610 IS - 0374-1338 (Print) IS - 0374-1338 (Linking) VI - 59 IP - 2 DP - 2012 TI - [Polymyositis/dermatomyositis--clinical picture and treatment]. PG - 44-50 AB - The clinical presentation ofmyositis ranges from a painless muscle weakness to significant myalgia with muscle weakness and constitutional symptoms. Along with muscle and skin affection and constitutional symptoms, the disease can affect lungs, joints, heart and gastrointestinal system. It is important to note that the clinical presentation ofmyositis syndrome may overlap with symptoms of other connective tissue disease in overlap syndromes (SLE, SSCL, RA, SSjö). Common manifestations of the disease are weakness and muscle fatigue, which is the result of skeletal muscles inflammation (usually the proximal group of muscles, bilaterally and symmetrical). Severe forms of the disease with affection of the throat and respiratory muscles can vitally endan- ger patients. Among constitutional (general) symptoms, fever, malaise and weight loss are usually expressed. Skin affection in dermatomyositis can be localized or generalized like vesiculobullous erythroderma. Pathognomonic cutaneous manifestations of dermatomyositis are Gottron's papules and heliotrope erythema. Lungs are most commonly affected organs (with exception of muscles and skin) in polymyositis and dermatomyositis. The affection of lung can sometimes result in fatal outcome (interstitial lung disease, secondary pulmonary hypertension). Cardiac affection is usually subclinical, but can also be expressed as heart failure, acute coronary syndrome or conduction disturbances. Infrequent manifestations of the disease are gastroesophageal reflux, malabsorption, gastrointestinal mucosal ulceration, soft tissue calcification, Raynaud's syndrome, arthralgia/arthritis and some other less common clinical manifestations of the disease. Treatment of polymyositis/dermatomyositis includes immunosuppressive/immunomodulatory therapy and supportive, symptomatic treatment. The basis for myositis treatment are glucocorticoids, which are applied orally in a daily dosage regimen of 0.75 to 1 mg/kg/day, and in severe forms of the disease in the i.v. pulse doses of 1 g/day. Immunosuppressants/immunomodulators are added in the therapy along with glucocorticoids for better control of the disease and to reduce the required dose of glucocorticoids (side effects of longterm high doses glucocorticoide use). The most commonly used immunosuppressive drug is methotrexate at a dose of up to 25 mg/week. Hydroxychloroquine has a good effect on the cutaneous manifestations of the disease. Among other immunosuppressants which are used in the treatment of myositis are azathioprine, cyclosporine (in patients with pulmonary affection), mycophenolate mofetil and tacrolimus. Intravenous immunoglobulins applied parenterally in a dose of 2 g/kg divided into multiple doses showed an excellent clinical effect in patients with affection of the esophagus and throat muscles, in patients with pulmonary affection and in patients with resistant disease. The experience with the biologics is limited to a small number of patients. Physiotherapy is a necessary form of treatment for the recovery of muscle strength in the remission phase of the disease. A prompt treatment of infections and heart failure is sometimes life-saving in patients with myositis. Symptomatic treatment of pain with analgesics and NSAIDs reduces pain, speeds up recovery and improves the quality of life in patients with myositis. FAU - Anić, Branimir AU - Anić B AD - Zavod za klinicku imunologiju i reumatologiju, Klinika za unutarnje bolesti, Klinicki bolnicki centar Zagreb, Kispatićeva 12, 10000 Zagreb. FAU - Cerovec, Mislav AU - Cerovec M LA - hrv PT - English Abstract PT - Journal Article TT - Polimiozitis/dermatomiozitis--klinicka slika i terapija. PL - Croatia TA - Reumatizam JT - Reumatizam JID - 0216650 SB - IM MH - Dermatomyositis/*diagnosis/*therapy MH - Humans MH - Polymyositis/*diagnosis/*therapy EDAT- 2012/01/01 00:00 MHDA- 2013/12/18 06:00 CRDT- 2013/06/11 06:00 PHST- 2013/06/11 06:00 [entrez] PHST- 2012/01/01 00:00 [pubmed] PHST- 2013/12/18 06:00 [medline] PST - ppublish SO - Reumatizam. 2012;59(2):44-50. PMID- 38530791 OWN - NLM STAT- MEDLINE DCOM- 20250422 LR - 20260310 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 63 IP - 12 DP - 2024 Dec 1 TI - Capi-score: a quantitative algorithm for identifying disease patterns in nailfold videocapillaroscopy. PG - 3315-3321 LID - 10.1093/rheumatology/keae197 [doi] AB - OBJECTIVES: EULAR supports the use of nailfold videocapillaroscopy (NVC) for identifying disease patterns (DPs) associated with SSc and RP. Recently, EULAR proposed an easy-to-manage procedure, a so-called Fast Track algorithm, for differentiating SSc patterns from non-SSc patterns in NVC specimens. However, subjectivity among capillaroscopists remains a limitation. Our aim was to perform a software-based analysis of NVC peculiarities in a cohort of samples from SSc and RP patients and, subsequently, build a Fast Track-inspired algorithm for identifying DPs without the constraint of interobserver variability. METHODS: NVCs were examined by 9 capillaroscopists. Those NVCs whose DPs were consensually agreed upon (by ≥2 out of 3 interobservers) were subsequently analysed using in-house-developed software. The results for each variable were grouped according to the consensually agreed-upon DPs in order to identify useful hallmarks for categorizing them. RESULTS: A total of 851 NVCs (21 957 images) whose DPs had been consensually agreed upon were software-analysed. Appropriate cut-offs set for capillary density and percentage of abnormal and giant capillaries, tortuosities and haemorrhages allowed DP categorization and the development of the CAPI-score algorithm. This consisted of four rules: Rule 1, SSc vs non-SSc, accuracy 0.88; Rules 2 and 3, SSc-early vs SSc-active vs SSc-late, accuracy 0.82; Rule 4, non-SSc normal vs non-SSc non-specific, accuracy 0.73. Accuracy improved when the analysis was limited to NVCs whose DPs had achieved full consensus between the interobservers. CONCLUSION: The CAPI-score algorithm may become a tool that is useful in assigning DPs by overcoming the limitations of subjectivity. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Gracia Tello, Borja Del Carmelo AU - Gracia Tello BDC AUID- ORCID: 0000-0003-3248-2908 AD - Internal Medicine Department, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. AD - Aragon Health Research Institute, Zaragoza, Spain. FAU - Sáez Comet, Luis AU - Sáez Comet L AD - Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain. FAU - Lledó, Gema AU - Lledó G AD - Department of Autoimmune Diseases, Hospital Clinic de Barcelona, Barcelona, Spain. FAU - Freire Dapena, Mayka AU - Freire Dapena M AD - Thrombosis and Vasculitis Unit, Complejo Hospitalario de Vigo, Pontevedra, Spain. FAU - Mesa, Miguel Antonio AU - Mesa MA AUID- ORCID: 0000-0001-5625-6791 AD - Rheumatology Section, Clinica El Rosario, Medellin, Colombia. FAU - Martín-Cascón, Miguel AU - Martín-Cascón M AD - Internal Medicine, Hospital General Universitario José M Morales Meseguer, Murcia, Spain. FAU - Guillén Del Castillo, Alfredo AU - Guillén Del Castillo A AD - Autoimmune Unit, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Martínez Robles, Elena AU - Martínez Robles E AD - Department of Internal Medicine, Hospital General Universitario La Paz, Madrid, Spain. FAU - Simeón-Aznar, Carmen Pilar AU - Simeón-Aznar CP AD - Autoimmune Unit, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Todolí Parra, Jose Antonio AU - Todolí Parra JA AD - Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain. FAU - Varela, Diana Cristina AU - Varela DC AD - Rheumatology Department, Hospital General de Medellín Luz Castro de Gutiérrez, Medellin, Colombia. FAU - Maldonado Vélez, Genessis AU - Maldonado Vélez G AUID- ORCID: 0000-0003-3978-0799 AD - Rheumatology Department, Vanderbilt University Medical Center, Nashville, TN, United States. FAU - Marín Ballvé, Adela AU - Marín Ballvé A AD - Internal Medicine Department, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. AD - Aragon Health Research Institute, Zaragoza, Spain. FAU - Aramburu Llorente, Jimena AU - Aramburu Llorente J AD - Internal Medicine Department, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Pérez Abad, Laura AU - Pérez Abad L AD - Internal Medicine Department, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Ramos Ibáñez, Eduardo AU - Ramos Ibáñez E AUID- ORCID: 0000-0002-4931-2190 AD - Computer Engineer, University of Zaragoza, Zaragoza, Spain. LA - eng GR - Fundación Instituto de Investigación Sanitaria/ GR - Boehringer Ingelheim Spain/ GR - Spanish Society of Internal Medicine/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - doi: 10.1093/rheumatology/keae330 MH - Humans MH - *Microscopic Angioscopy/methods MH - *Algorithms MH - *Nails/blood supply MH - *Scleroderma, Systemic/diagnosis/diagnostic imaging MH - Female MH - Male MH - Capillaries/pathology/diagnostic imaging MH - Middle Aged MH - Adult MH - Aged MH - Observer Variation PMC - PMC11637479 OTO - NOTNLM OT - Fast Track algorithm OT - Raynaud’s phenomenon OT - nailfold videocapillaroscopy OT - quantitative OT - software-based algorithm OT - systemic sclerosis EDAT- 2024/03/26 18:44 MHDA- 2025/02/08 19:53 PMCR- 2024/03/26 CRDT- 2024/03/26 13:24 PHST- 2023/11/29 00:00 [received] PHST- 2024/03/17 00:00 [accepted] PHST- 2025/02/08 19:53 [medline] PHST- 2024/03/26 18:44 [pubmed] PHST- 2024/03/26 13:24 [entrez] PHST- 2024/03/26 00:00 [pmc-release] AID - 7635170 [pii] AID - keae197 [pii] AID - 10.1093/rheumatology/keae197 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Dec 1;63(12):3315-3321. doi: 10.1093/rheumatology/keae197. PMID- 19644851 OWN - NLM STAT- MEDLINE DCOM- 20090925 LR - 20250529 IS - 0004-3591 (Print) IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 60 IP - 8 DP - 2009 Aug TI - Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: analysis of three large multicenter, double-blind, randomized controlled trials. PG - 2490-8 LID - 10.1002/art.24681 [doi] AB - OBJECTIVE: To assess the course of the modified Rodnan skin thickness score (MRSS) in 3 large, multicenter, double-blind, randomized controlled trials (RCTs) of patients with diffuse cutaneous systemic sclerosis (dcSSc) with different baseline disease durations, as defined from the date of onset of the first dcSSc symptom (excluding Raynaud's phenomenon) or from the date of onset of the first dcSSc-related symptom (including Raynaud's phenomenon). METHODS: Data from 3 RCTs examining high-dose versus low-dose D-penicillamine (D-Pen Trial), recombinant human relaxin versus placebo (Relaxin Trial), and oral bovine type I collagen versus placebo (Collagen Trial) treatment in patients with dcSSc were pooled and analyzed. Patients were divided into 5 groups according to their disease duration at baseline. The linear mixed model for correlated data was used to model the 2 predictors of MRSS: time in study (expressed in months after baseline) and baseline disease duration (expressed in months, calculated from the date of onset of the first symptom characteristic of dcSSc with and without Raynaud's phenomenon). RESULTS: At study entry, the mean MRSS value was 21.0 in the D-Pen Trial cohort, 27.3 in the Relaxin Trial cohort, and 26.1 in the Collagen Trial cohort. Time in study was a significant predictor of improvement in MRSS regardless of the disease duration at baseline (P<0.0001). Patients with a disease duration of >or=24 months showed a greater rate of decline as compared with patients with a disease duration of <24 months (P<0.05). Similar results were obtained when disease duration was reclassified by including the time of the first Raynaud's phenomenon symptom in the definition. CONCLUSION: Our study confirms recent findings that in patients entered into these 3 RCTs, skin thickening did not follow the same trend in natural history as that seen in the dcSSc populations entered into early, open longitudinal studies previously reported. These findings have important implications for study design, in which "prevention of worsening" is the main objective. FAU - Amjadi, Sogol AU - Amjadi S AD - Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA. FAU - Maranian, Paul AU - Maranian P FAU - Furst, Daniel E AU - Furst DE FAU - Clements, Philip J AU - Clements PJ FAU - Wong, Weng Kee AU - Wong WK FAU - Postlethwaite, Arnold E AU - Postlethwaite AE FAU - Khanna, Puja P AU - Khanna PP FAU - Khanna, Dinesh AU - Khanna D CN - Investigators of the D-Penicillamine, Human Recombinant Relaxin, and Oral Bovine Type I Collagen Clinical Trials LA - eng GR - N01-AR-902242/AR/NIAMS NIH HHS/United States GR - 1 T32-AR-053463/AR/NIAMS NIH HHS/United States GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - K23-AR-053858-01A1/AR/NIAMS NIH HHS/United States GR - T32 AR053463/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antirheumatic Agents) RN - 0 (Collagen Type I) RN - 0 (Recombinant Proteins) RN - 9002-69-1 (Relaxin) RN - GNN1DV99GX (Penicillamine) SB - IM MH - Adult MH - Age of Onset MH - Animals MH - Antirheumatic Agents/therapeutic use MH - Cattle MH - Collagen Type I/therapeutic use MH - Disease Progression MH - Female MH - Health Status MH - Humans MH - Male MH - Middle Aged MH - Penicillamine/therapeutic use MH - Recombinant Proteins/therapeutic use MH - Relaxin/therapeutic use MH - Scleroderma, Systemic/drug therapy/*pathology MH - Severity of Illness Index MH - Skin/*pathology PMC - PMC2725229 MID - NIHMS117401 EDAT- 2009/08/01 09:00 MHDA- 2009/09/26 06:00 PMCR- 2010/08/01 CRDT- 2009/08/01 09:00 PHST- 2009/08/01 09:00 [entrez] PHST- 2009/08/01 09:00 [pubmed] PHST- 2009/09/26 06:00 [medline] PHST- 2010/08/01 00:00 [pmc-release] AID - 10.1002/art.24681 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Aug;60(8):2490-8. doi: 10.1002/art.24681. PMID- 37157177 OWN - NLM STAT- MEDLINE DCOM- 20231102 LR - 20231102 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 26 IP - 11 DP - 2023 Nov TI - Autoimmune diseases post-COVID 19 infection in children in intensive care unit: A case series. PG - 2288-2293 LID - 10.1111/1756-185X.14724 [doi] AB - SARS-CoV2 primarily affects the respiratory system but a hyperinflammatory response leading to multisystem inflammatory syndrome - children (MIS-C), immune dysfunction and various autoimmune manifestations has also been noted. Autoimmunity depends on various factors, including genetic predisposition, environmental factors, immune dysregulation and infections acting as triggers like Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus, hepatitis B. Molecular mimicry, bystander T-cell activation and persistence of viral infection are the main mechanisms behind these manifestations. We present here 3 cases of newly diagnosed connective tissue disease with high titers of COVID19 immunoglobulin G antibody in children. A 9-year-old girl with fever, oliguria and malar rash (prior history of sore throat) and a 10-year-old girl with fever for 2 weeks and choreoathetoid movements were diagnosed as systemic lupus erythematosus (SLE) nephritis (stage 4) and neuropsychiatric SLE, respectively as per European League Against Rheumatism / American College of Rheumatology 2019 criteria. An 8-year-old girl with fever, joint pain and respiratory distress (a recent contact with a positive COVID19 patient) presented with altered sensorium, Raynaud's phenomenon noted, and eventually diagnosed as mixed connective tissue disease as per Kusukawa criteria. The immune-mediated manifestations post-COVID infection are a de-novo phenomenon which necessitates further workup as not many studies exist in the pediatric population. CI - © 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Das, Subhadipa AU - Das S AUID- ORCID: 0000-0002-6468-2413 AD - Department of Paediatrics, Nilrantan Sircar Medical College, Kolkata, West Bengal, India. FAU - Parul AU - Parul AUID- ORCID: 0000-0002-3906-2271 AD - Department of Paediatrics, Nilrantan Sircar Medical College, Kolkata, West Bengal, India. FAU - Samanta, Moumita AU - Samanta M AUID- ORCID: 0000-0001-7961-2672 AD - Department of Paediatrics, Nilrantan Sircar Medical College, Kolkata, West Bengal, India. LA - eng PT - Case Reports PT - Journal Article DEP - 20230508 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (RNA, Viral) RN - malar rash RN - pediatric multisystem inflammatory disease, COVID-19 related SB - IM MH - *COVID-19/complications MH - SARS-CoV-2 MH - Systemic Inflammatory Response Syndrome MH - Child MH - Female MH - *Epstein-Barr Virus Infections MH - Humans MH - Exanthema MH - RNA, Viral MH - *Lupus Erythematosus, Systemic/diagnosis MH - Herpesvirus 4, Human MH - *Lupus Nephritis OTO - NOTNLM OT - SLE OT - case report OT - mixed connective tissue disease OT - post-COVID autoimmune manifestations EDAT- 2023/05/09 06:42 MHDA- 2023/11/02 12:44 CRDT- 2023/05/09 01:03 PHST- 2023/04/16 00:00 [revised] PHST- 2022/05/30 00:00 [received] PHST- 2023/04/24 00:00 [accepted] PHST- 2023/11/02 12:44 [medline] PHST- 2023/05/09 06:42 [pubmed] PHST- 2023/05/09 01:03 [entrez] AID - 10.1111/1756-185X.14724 [doi] PST - ppublish SO - Int J Rheum Dis. 2023 Nov;26(11):2288-2293. doi: 10.1111/1756-185X.14724. Epub 2023 May 8. PMID- 33226736 OWN - NLM STAT- MEDLINE DCOM- 20211011 LR - 20211011 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 23 IP - 12 DP - 2020 Dec TI - Risk factors for progression and prognosis of primary Sjögren's syndrome-associated interstitial lung disease in a Chinese population. PG - 1734-1740 LID - 10.1111/1756-185X.14023 [doi] AB - OBJECTIVE: This study explored differences between primary Sjögren's syndrome-associated interstitial lung disease (pSS-ILD) patients with and without ILD progression, and analyzed the factors affecting the progression and prognosis of pSS-ILD. METHODS: This study is a retrospective cohort study which enrolled 113 pSS-ILD patients hospitalized between 2011 and 2017. RESULTS: The 3-year survival rate of the pSS-ILD patients was 91.15%, and the 5-year survival rate was 84.07%. Univariate analysis showed that Raynaud's syndrome, hypoproteinemia, extensive lung involvement, possible usual interstitial pneumonia pattern were risk factors for the progression of ILD in patients with pSS-ILD, and cyclophosphamide was a protective factor for the progression of ILD in patients with pSS-ILD. Multiple logistic regression analysis showed that extensive lung involvement (odds ratio 4.143, 95% CI: 1.203-14.267, P < .05) was an independent risk factor for the progression of pSS-ILD. Cox hazard analysis showed that pSS-ILD with hypoproteinemia (hazard ratio [HR] 17.758, 95% CI: 4.753-66.340, P <- .05) and extensive lung involvement (HR 3.450, 95% CI: 1.419-8.390, P < .05) were associated with worse survival of patients. CONCLUSION: Extensive lung involvement is an independent risk factor for the progression of ILD in patients with pSS-ILD. Hypoproteinemia and extensive lung involvement are independent risk factors for mortality in patients with pSS-ILD, after controlling for potentially influential variables. CI - © 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Xu, Yuetong AU - Xu Y AUID- ORCID: 0000-0001-8595-0950 AD - Department of Rheumatology and Immunology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China. FAU - Zhou, Junfei AU - Zhou J AD - Department of Rheumatology and immunology, Henan Province People's Hospital, Zhengzhou City, China. FAU - Dong, Xin AU - Dong X AD - Department of Rheumatology and Immunology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China. FAU - Guo, Xiaojuan AU - Guo X AD - Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China. FAU - Lu, Yuewu AU - Lu Y AD - Department of Rheumatology and Immunology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China. FAU - Zheng, Yi AU - Zheng Y AD - Department of Rheumatology and Immunology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China. LA - eng PT - Journal Article DEP - 20201123 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - China/epidemiology MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Incidence MH - Lung Diseases, Interstitial/*epidemiology/etiology MH - Male MH - Middle Aged MH - Prognosis MH - Retrospective Studies MH - Risk Factors MH - Sjogren's Syndrome/*complications/diagnosis/epidemiology MH - Survival Rate/trends OTO - NOTNLM OT - interstitial lung disease OT - primary Sjögren's syndrome OT - prognostic factors OT - progression EDAT- 2020/11/24 06:00 MHDA- 2021/10/12 06:00 CRDT- 2020/11/23 12:13 PHST- 2020/06/01 00:00 [received] PHST- 2020/09/12 00:00 [revised] PHST- 2020/10/15 00:00 [accepted] PHST- 2020/11/24 06:00 [pubmed] PHST- 2021/10/12 06:00 [medline] PHST- 2020/11/23 12:13 [entrez] AID - 10.1111/1756-185X.14023 [doi] PST - ppublish SO - Int J Rheum Dis. 2020 Dec;23(12):1734-1740. doi: 10.1111/1756-185X.14023. Epub 2020 Nov 23. PMID- 41179010 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251103 LR - 20251105 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 9 DP - 2025 Sep TI - Anti-PL-12 and Interstitial Lung Disease as the First Signs of Antisynthetase Syndrome in Patients With Previously Diagnosed Systemic Lupus Erythematosus. PG - e93472 LID - 10.7759/cureus.93472 [doi] LID - e93472 AB - Antisynthetase syndrome (ASTS) is an infrequent autoimmune condition marked by chronic inflammation and the presence of autoantibodies targeting aminoacyl-tRNA synthetases. Clinically, it often manifests through a constellation of features, including inflammatory arthritis, myositis, Raynaud's phenomenon, hyperkeratotic skin changes (mechanic's hands), and interstitial lung disease (ILD). Among these, ILD is the most significant in terms of prognosis, as it contributes to elevated rates of morbidity and mortality, surpassing those seen in other idiopathic inflammatory myopathies. We report the clinical course of a 56-year-old woman with a prior diagnosis of systemic lupus erythematosus (SLE) established in 2019, based on the 2017 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria. She was admitted to the Institute for Pulmonary Diseases of Vojvodina (IPBV) in Sremska Kamenica, Serbia, due to respiratory symptoms suggestive of ILD. Although ILD is an uncommon manifestation in SLE, the patient was assessed for the potential coexistence of other connective tissue diseases, such as systemic sclerosis (SSc) and inflammatory myopathies. Laboratory testing demonstrated a strong antinuclear antibody (ANA) reactivity on Hep-2 cells and low anti-double-stranded DNA (anti-dsDNA) levels (<10 IU/ml). Extended myositis panel testing revealed marked positivity for anti-PL-12 and anti-Ro-52 antibodies. Given the presence of pre-existing ILD and clinical signs of arthritis and proximal muscle weakness, alongside the identification of anti-tRNA synthetases antibodies, a diagnosis of ASTS was established. Immunosuppressive treatment was intensified by increasing the glucocorticoid (GC) dosage and introducing intravenous cyclophosphamide (CYC, 1 g). The patient demonstrated a favorable clinical and biochemical response to this regimen. CI - Copyright © 2025, Djuran et al. FAU - Djuran, Ivana AU - Djuran I AD - Nephrology and Clinical Immunology, Clinic for Nephrology and Clinical Immunology, University Clinical Centre of Vojvodina, Novi Sad, SRB. FAU - Ljubicic, Bojana AU - Ljubicic B AD - Emergency Internal Medicine, Emergency Centre, University Clinical Centre of Vojvodina, Novi Sad, SRB. FAU - Lazarevic, Ana AU - Lazarevic A AD - Emergency Internal Medicine, Emergency Centre, University Clinical Centre of Vojvodina, Novi Sad, SRB. FAU - Popovic, Milica AU - Popovic M AD - Allergy and Immunology, Clinic for Nephrology and Clinical Immunology, University Clinical Centre of Vojvodina, Novi Sad, SRB. FAU - Knezevic, Violeta AU - Knezevic V AD - Nephrology, Clinic for Nephrology and Clinical Immunology, University Clinical Centre of Vojvodina, Novi Sad, SRB. LA - eng PT - Case Reports PT - Journal Article DEP - 20250929 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12571698 OTO - NOTNLM OT - anti-pl-12 antibody OT - antisynthetase syndrome OT - interstitial lung disease OT - juvenile idiopathic inflammatory myositis OT - overlap disease COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/11/03 13:42 MHDA- 2025/11/03 13:43 PMCR- 2025/09/29 CRDT- 2025/11/03 06:55 PHST- 2025/09/29 00:00 [accepted] PHST- 2025/11/03 13:43 [medline] PHST- 2025/11/03 13:42 [pubmed] PHST- 2025/11/03 06:55 [entrez] PHST- 2025/09/29 00:00 [pmc-release] AID - 10.7759/cureus.93472 [doi] PST - epublish SO - Cureus. 2025 Sep 29;17(9):e93472. doi: 10.7759/cureus.93472. eCollection 2025 Sep. PMID- 41898254 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260328 LR - 20260330 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 14 IP - 3 DP - 2026 Mar 9 TI - Micro- and Macro-Vascular Disease in Systemic Sclerosis and Very Early SSc (VEDOSS): Results from a Monocentric Observational Study. LID - 10.3390/biomedicines14030607 [doi] LID - 607 AB - Background: Systemic sclerosis (SSc) is characterized by endothelial dysfunction leading to progressive vascular injury and fibrosis. While microvascular involvement is well established as an early disease feature, macrovascular disease has been historically underrecognized and poorly investigated in very early disease stages. Integrated assessments across the SSc spectrum, including very early diagnosis of systemic sclerosis (VEDOSS), remain limited. Methods: In this cross-sectional observational study, patients with established SSc, VEDOSS, and primary Raynaud's phenomenon (PRP) were prospectively enrolled between October 2023 and April 2025. Participants underwent comprehensive microvascular and macrovascular evaluation, including nailfold videocapillaroscopy, multisegmental arterial Doppler ultrasound (carotid, aortic, and lower limb districts), flow-mediated dilation, and measurement of endothelial biomarkers (vascular cell adhesion molecule 1 (VCAM-1), intercellular adhesion molecule 1 (ICAM-1), and circulating endothelial cells (CECs)). Traditional cardiovascular risk was estimated using Systematic Coronary Risk Estimation 2 (SCORE2). Results: Sixty-two female subjects were included (34 SSc, 14 VEDOSS, and 14 PRP). Microvascular abnormalities followed the expected disease continuum, with capillaroscopic changes present in 57% of VEDOSS and 91% of SSc patients. Although SCORE2 estimates and carotid intima-media thickness were comparable across groups, macrovascular abnormalities were more frequent in SSc (52.9%) and VEDOSS (50%) compared with PRP (21.4%). VCAM-1, ICAM-1, and CEC levels were significantly increased in SSc compared with PRP, whereas no significant differences were observed between VEDOSS and PRP. Conclusions: These findings support a unified micro- and macro-vascular disease model in SSc and demonstrate that macrovascular involvement is detectable already in the VEDOSS phase. Conventional cardiovascular risk scores underestimate the true vascular burden, highlighting the need for disease-specific risk stratification tools integrating vascular imaging and endothelial biomarkers. FAU - Zaccone, Vincenzo AU - Zaccone V AUID- ORCID: 0000-0002-5285-859X AD - PhD Program in Human Health, Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Contegiacomo, Silvia AU - Contegiacomo S AD - Internal Medicine Residency Program, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Agarbati, Silvia AU - Agarbati S AUID- ORCID: 0000-0003-2823-6325 AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Paolini, Chiara AU - Paolini C AUID- ORCID: 0000-0001-6364-7408 AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Clementi, Carolina AU - Clementi C AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Mozzicafreddo, Matteo AU - Mozzicafreddo M AUID- ORCID: 0000-0001-9835-8446 AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Svegliati, Silvia AU - Svegliati S AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. FAU - Falsetti, Lorenzo AU - Falsetti L AUID- ORCID: 0000-0003-3411-2388 AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. AD - Clinica Medica, Department of Internal Medicine, Marche University Hospital, Via Conca 71, 60126 Ancona, Italy. FAU - Benfaremo, Devis AU - Benfaremo D AUID- ORCID: 0000-0002-9867-2360 AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. AD - Clinica Medica, Department of Internal Medicine, Marche University Hospital, Via Conca 71, 60126 Ancona, Italy. FAU - Moroncini, Gianluca AU - Moroncini G AD - Department of Clinical and Molecular Sciences, Marche Polytechnic University, Via Tronto 10/A, 60126 Ancona, Italy. AD - Clinica Medica, Department of Internal Medicine, Marche University Hospital, Via Conca 71, 60126 Ancona, Italy. LA - eng GR - Project Code PE00000019, CUP I33C22006900006/Ministero dell'università e della ricerca/ PT - Journal Article DEP - 20260309 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC13024536 OTO - NOTNLM OT - VEDOSS OT - arterial disease OT - atherosclerosis OT - endothelial dysfunction OT - macrovascular disease OT - microvascular involvement OT - systemic sclerosis COIS- The authors declare no conflicts of interest. EDAT- 2026/03/28 06:39 MHDA- 2026/03/28 06:40 PMCR- 2026/03/09 CRDT- 2026/03/28 01:21 PHST- 2026/02/02 00:00 [received] PHST- 2026/02/27 00:00 [revised] PHST- 2026/03/05 00:00 [accepted] PHST- 2026/03/28 06:40 [medline] PHST- 2026/03/28 06:39 [pubmed] PHST- 2026/03/28 01:21 [entrez] PHST- 2026/03/09 00:00 [pmc-release] AID - biomedicines14030607 [pii] AID - biomedicines-14-00607 [pii] AID - 10.3390/biomedicines14030607 [doi] PST - epublish SO - Biomedicines. 2026 Mar 9;14(3):607. doi: 10.3390/biomedicines14030607. PMID- 31621191 OWN - NLM STAT- MEDLINE DCOM- 20200413 LR - 20200413 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 22 IP - 11 DP - 2019 Nov TI - Prevalence of undifferentiated inflammatory arthropathy in patients with Hashimoto's thyroiditis in an endocrinology clinic. PG - 1985-1989 LID - 10.1111/1756-185X.13722 [doi] AB - AIM: Rheumatic manifestations are common in patients with Hashimoto's thyroiditis (HT). Since previous reports on the prevalence of arthritis in this disease may have a rheumatology referral bias, we sought to establish the prevalence of undifferentiated inflammatory arthropathy (UIA) in unselected HT patients as seen in an endocrinology clinic. METHODS: Cross-sectional study of 92 consecutive HT patients and no definite rheumatic disease from the Endocrinology Division, Hospital Universitario de Caracas diagnosed by the presence of anti-thyroid peroxidase antibodies (n = 68) or typical ultrasonographic findings (n = 24). Undifferentiated inflammatory arthropathy was defined as combination of morning stiffness and joint pain with ≥2 characteristics of inflammatory joint pain. The study was revised and approved by the Ethics Committee of our hospital and all patients signed an informed consent form. RESULTS: Twenty-three patients (25%; 95% CI 16-34) met the criteria for UIA. Joints most commonly affected were the knees, hands and ankles and the most common pattern was oligoarticular (82.6%). In the multivariate analysis, variables associated to the presence of UIA were the presence of myalgia (odds ratio [OR] = 19.41; 95% CI = 2.38-158.38) and Raynaud's phenomenon (OR = 4.32; 95% CI = 1.01-18.60). No association was found with demographics, duration of disease, comorbidities or thyroid function status. CONCLUSIONS: Almost 1 in 4 patients with HT had no definite rheumatic disease present with UIA. An early identification of concurrent arthritis in HT patients is necessary for thorough differential diagnosis and prompt treatment initiation to halt potential joint damage and disability. CI - © 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Valderrama-Hinds, Luis M AU - Valderrama-Hinds LM AD - Division of Rheumatology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - García-Carrión, Eliangel AU - García-Carrión E AD - Division of Rheumatology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - Hernández, Evelyn AU - Hernández E AD - Division of Endocrinology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - Agostini, María I AU - Agostini MI AD - Division of Endocrinology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - Reyes-Morales, Omar R AU - Reyes-Morales OR AD - Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - Fung, Liliana AU - Fung L AD - Division of Endocrinology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. FAU - Al Snih, Soham AU - Al Snih S AD - Division of Rehabilitation Sciences, School of Health Professions, University of Texas Medical Branch, Galveston, TX, USA. FAU - Rodríguez, Martin A AU - Rodríguez MA AUID- ORCID: 0000-0001-6949-9012 AD - Division of Rheumatology, Department of Internal Medicine, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela. AD - Sealy Center on Aging, University of Texas Medical Branch, Galveston, TX, USA. LA - eng PT - Journal Article DEP - 20191016 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM CIN - Int J Rheum Dis. 2020 Apr;23(4):597-598. doi: 10.1111/1756-185X.13820. PMID: 32134196 MH - Adult MH - Arthritis/diagnosis/*epidemiology/immunology/therapy MH - Cross-Sectional Studies MH - *Endocrinology MH - Female MH - Hashimoto Disease/diagnosis/*epidemiology/immunology/therapy MH - Humans MH - Male MH - Middle Aged MH - *Outpatient Clinics, Hospital MH - Prevalence MH - Prognosis MH - Risk Assessment MH - Risk Factors MH - Venezuela/epidemiology OTO - NOTNLM OT - Hashimoto's disease OT - arthritis OT - autoimmune thyroiditis OT - prevalence OT - undifferentiated inflammatory arthropathy EDAT- 2019/10/18 06:00 MHDA- 2020/04/14 06:00 CRDT- 2019/10/18 06:00 PHST- 2019/07/02 00:00 [received] PHST- 2019/09/10 00:00 [revised] PHST- 2019/09/16 00:00 [accepted] PHST- 2019/10/18 06:00 [pubmed] PHST- 2020/04/14 06:00 [medline] PHST- 2019/10/18 06:00 [entrez] AID - 10.1111/1756-185X.13722 [doi] PST - ppublish SO - Int J Rheum Dis. 2019 Nov;22(11):1985-1989. doi: 10.1111/1756-185X.13722. Epub 2019 Oct 16. PMID- 34177936 OWN - NLM STAT- MEDLINE DCOM- 20211028 LR - 20211028 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 12 DP - 2021 TI - Association Between Minor Salivary Gland Biopsy During Sjӧgren's Syndrome and Serologic Biomarkers: A Systematic Review and Meta-Analysis. PG - 686457 LID - 10.3389/fimmu.2021.686457 [doi] LID - 686457 AB - OBJECTIVE: Patients with primary Sjögren's syndrome (pSS) may develop a potentially severe disease with extra-glandular involvement and lymphoma insurgence. Minor salivary gland biopsy is routinely used in the disease diagnosis, but its potential role as a biomarker for clinical disease presentation and prognosis is still poorly understood. METHODS: We performed a systematic review and meta-analysis on clinical presentation and prognosis in pSS patients who underwent minor salivary gland biopsy at diagnosis according to the PRISMA guidelines. RESULTS: We included five retrospective studies and 589 pSS patients. Ectopic GCs presence was not associated with a significant increase in the odds ratio for the clinical variables explored such as salivary gland swelling, arthritis, and Raynaud's phenomenon. As far as serological features are concerned, ectopic GCs presence accounted for an increased ratio of antibodies anti-SSA (OR = 3.13, 95% CI: 1.25-7.85, p = 0.02, I(2) = 79%), anti-SSB (OR = 3.94, 95% CI: 1.50-10.37, p = 0.0005, I(2) = 80%), and RFs presence (OR = 3.12, 95% CI: 1.94-5.00, p < 0.00001, I(2) = 0%). CONCLUSIONS: This study showed that the association between ectopic GC in salivary glands identifies a clinical subset characterized by autoantibodies presence, and probably pSS patients affected from a more severe disease. CI - Copyright © 2021 Berardicurti, Ruscitti, Di Benedetto, D’Andrea, Navarini, Marino, Cipriani and Giacomelli. FAU - Berardicurti, Onorina AU - Berardicurti O AD - Department of Biotechnological and Applied Clinical Sciences, Rheumatology Unit, University of L'Aquila, L'Aquila, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Department of Biotechnological and Applied Clinical Sciences, Rheumatology Unit, University of L'Aquila, L'Aquila, Italy. FAU - Di Benedetto, Paola AU - Di Benedetto P AD - Department of Biotechnological and Applied Clinical Sciences, Rheumatology Unit, University of L'Aquila, L'Aquila, Italy. FAU - D'Andrea, Settimio AU - D'Andrea S AD - Department of Life, Health and Environment Sciences, Andrology Unit, University of L'Aquila, L'Aquila, Italy. FAU - Navarini, Luca AU - Navarini L AD - Rheumatology and Immunology Unit, Department of Medicine, University of Rome 'Campus Biomedico', Rome, Italy. FAU - Marino, Annalisa AU - Marino A AD - Rheumatology and Immunology Unit, Department of Medicine, University of Rome 'Campus Biomedico', Rome, Italy. FAU - Cipriani, Paola AU - Cipriani P AD - Department of Biotechnological and Applied Clinical Sciences, Rheumatology Unit, University of L'Aquila, L'Aquila, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Rheumatology and Immunology Unit, Department of Medicine, University of Rome 'Campus Biomedico', Rome, Italy. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20210611 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Biopsy MH - Germinal Center/pathology MH - Humans MH - Prognosis MH - Salivary Glands, Minor/*pathology MH - Sjogren's Syndrome/blood/*diagnosis/*pathology PMC - PMC8226119 OTO - NOTNLM OT - Sjӧgren’s syndrome OT - autoantibodies OT - clinical features OT - focus score OT - germinal center OT - minor salivary gland biopsy OT - serological biomarkers COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/06/29 06:00 MHDA- 2021/10/29 06:00 PMCR- 2021/01/01 CRDT- 2021/06/28 05:56 PHST- 2021/03/26 00:00 [received] PHST- 2021/04/20 00:00 [accepted] PHST- 2021/06/28 05:56 [entrez] PHST- 2021/06/29 06:00 [pubmed] PHST- 2021/10/29 06:00 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2021.686457 [doi] PST - epublish SO - Front Immunol. 2021 Jun 11;12:686457. doi: 10.3389/fimmu.2021.686457. eCollection 2021. PMID- 30020876 OWN - NLM STAT- MEDLINE DCOM- 20190410 LR - 20190410 IS - 1646-0758 (Electronic) IS - 0870-399X (Linking) VI - 31 IP - 6 DP - 2018 Jun 29 TI - Clinical Presentation and Long-Term Outcomes of Systemic Sclerosis Portuguese Patients from a Single Centre Cohort: A EUSTAR Registration Initiative. PG - 312-320 LID - 10.20344/amp.10658 [doi] AB - INTRODUCTION: Systemic sclerosis is a complex disorder that requires systematic screening. Our objective is to report the European Scleroderma Trials and Research group centre affiliation and its impact in our clinical practice. MATERIAL AND METHODS: The European Scleroderma Trials and Research group affiliation process, database update and current patient evaluation, with respect to demographic and clinical features. Cumulative mortality was analysed. RESULTS: We identified 19 female patients (which met all the American College of Rheumatology/ European League Against Rheumatism 2013 criteria for systemic sclerosis) under current follow-up, divided according to the LeRoy classification into diffuse cutaneous (n = 5), limited cutaneous (n = 11) and limited (n = 3) types, followed for a median period of 5, 12 and 6 years, respectively. Raynaud´s phenomenon and abnormal nailfold capillaries were universally present. Interstitial lung disease was absent in the limited cutaneous form but present in 100% of the diffuse subtype. Pitting scars were more common in the diffuse form. Active disease was also more frequent in the diffuse form, and most patients with active disease were treated with anti-endothelin receptor antagonists. Over 21 years (from 1994 to 2015) the mortality rate was 55% (n = 23/42). Age at time of death was significantly lower in the diffuse subtype. DISCUSSION: Our single centre cohort shares many features with larger and international reports and more specifically is in accordance with patient characteristics described in the European Scleroderma Trials and Research group registries. CONCLUSION: The European Scleroderma Trials and Research group registration motivated our systematic patient characterization and may be used as a tool for homogenous disease registries. FAU - Vidal, Carolina AU - Vidal C AD - Serviço de Medicina Interna. Hospital do Divino Espírito Santo de Ponta Delgada. São Miguel. FAU - Ruano, Carina AU - Ruano C AD - Serviço de Radiologia. Hospital de Santa Marta. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Bernardino, Vera AU - Bernardino V AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Lavado Carreira, Pedro AU - Lavado Carreira P AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Lladó, Ana AU - Lladó A AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Santos, Maria Céu AU - Santos MC AD - Laboratório de Imunologia. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Gruner, Heidi AU - Gruner H AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Panarra, António AU - Panarra A AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Riso, Nuno AU - Riso N AD - Unidade de Doenças Auto-Imunes/Serviço Medicina 7.2. Hospital de Curry Cabral. Centro Hospitalar de Lisboa Central. Lisboa. Portugal. FAU - Moraes-Fontes, Maria Francisca AU - Moraes-Fontes MF AD - Serviço de Medicina Interna. Hospital do Divino Espírito Santo de Ponta Delgada. São Miguel. Portugal. LA - eng PT - Journal Article DEP - 20180629 PL - Portugal TA - Acta Med Port JT - Acta medica portuguesa JID - 7906803 SB - IM EIN - Acta Med Port. 2018 Sep 28;31(9):520. doi: 10.20344/amp.11238. PMID: 30332383 MH - Aged MH - Europe MH - Female MH - Humans MH - Middle Aged MH - Registries MH - *Scleroderma, Systemic/diagnosis/mortality/therapy MH - Survival Rate MH - Time Factors MH - Treatment Outcome OTO - NOTNLM OT - Databases, Factual OT - Scleroderma, Systemic OT - Severity of Illness Index EDAT- 2018/07/19 06:00 MHDA- 2019/04/11 06:00 CRDT- 2018/07/19 06:00 PHST- 2018/04/13 00:00 [received] PHST- 2018/05/14 00:00 [accepted] PHST- 2018/07/19 06:00 [entrez] PHST- 2018/07/19 06:00 [pubmed] PHST- 2019/04/11 06:00 [medline] AID - 10.20344/amp.10658 [doi] PST - ppublish SO - Acta Med Port. 2018 Jun 29;31(6):312-320. doi: 10.20344/amp.10658. Epub 2018 Jun 29. PMID- 25645801 OWN - NLM STAT- MEDLINE DCOM- 20151027 LR - 20181202 IS - 1873-2623 (Electronic) IS - 0041-1345 (Linking) VI - 47 IP - 1 DP - 2015 Jan-Feb TI - A case report of living-donor lobar lung transplantation for scleroderma-associated usual interstitial pneumonia: eight years and counting. PG - 190-3 LID - S0041-1345(14)01196-8 [pii] LID - 10.1016/j.transproceed.2014.10.038 [doi] AB - INTRODUCTION: Scleroderma-associated interstitial lung disease is a life-limiting complication of scleroderma, often requiring lung transplantation. Living-donor lobar lung transplantation (LDLLT) is a viable alternative to deceased-donor lung transplantation in specialized centers under select circumstances. CLINICAL CASE: A 47-year-old female underwent LDLLT after nine years of symptomatic scleroderma-associated usual interstitial pneumonia and three years awaiting deceased-donor lung transplantation. Her manifestations of scleroderma included mild sclerodactyly, periungual erythema, Raynaud's phenomenon, and gastroesophageal reflux, with positive antinuclear autoantibodies. Several years post-transplantation, manometry revealed feeble lower esophageal sphincteric pressure with ineffective esophageal motility. Bronchiolitis obliterans syndrome developed 64 months post-transplantation without evidence of aspiration or reflux on transbronchial biopsy. Currently, she has normal renal function and good allograft function [FEV1 1.52 L (73% predicted) and FVC 2.50 L (99% predicted)]. RELEVANCE: This is the second reported case of LDLLT in scleroderma, and the first reporting long-term pulmonary, renal, and esophageal function post-transplantation. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Laratta, C AU - Laratta C AD - Department of Medicine, University of Alberta, Edmonton, Alberta, Canada. FAU - Lien, D AU - Lien D AD - Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada. FAU - Puttagunta, L AU - Puttagunta L AD - Department of Pathology, University of Alberta, Edmonton, Alberta, Canada. FAU - Jackson, K AU - Jackson K AD - Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada. FAU - Mullen, J AU - Mullen J AD - Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Department of Cardiothoracic Surgery, University of Alberta, Edmonton, Alberta, Canada. FAU - Kapasi, A AU - Kapasi A AD - Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada. FAU - Weinkauf, J AU - Weinkauf J AD - Lung Transplant Program, University of Alberta, Edmonton, Alberta, Canada; Division of Respirology, University of Alberta, Edmonton, Alberta, Canada. Electronic address: justin.weinkauf@ualberta.ca. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Transplant Proc JT - Transplantation proceedings JID - 0243532 SB - IM MH - Bronchiolitis Obliterans/etiology MH - Female MH - Gastroesophageal Reflux/etiology MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnosis/etiology/*surgery MH - Living Donors MH - *Lung Transplantation MH - Middle Aged MH - Scleroderma, Systemic/complications/diagnosis/*surgery MH - Time Factors MH - Treatment Outcome EDAT- 2015/02/04 06:00 MHDA- 2015/10/28 06:00 CRDT- 2015/02/04 06:00 PHST- 2014/10/05 00:00 [received] PHST- 2014/10/29 00:00 [accepted] PHST- 2015/02/04 06:00 [entrez] PHST- 2015/02/04 06:00 [pubmed] PHST- 2015/10/28 06:00 [medline] AID - S0041-1345(14)01196-8 [pii] AID - 10.1016/j.transproceed.2014.10.038 [doi] PST - ppublish SO - Transplant Proc. 2015 Jan-Feb;47(1):190-3. doi: 10.1016/j.transproceed.2014.10.038. PMID- 40556620 OWN - NLM STAT- MEDLINE DCOM- 20250804 LR - 20250807 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 8 DP - 2025 Aug TI - Clinical and serological features of systemic sclerosis patients, according to different geographic areas: insights from an observational, cross-sectional study of two independent cohorts. PG - 1472-1480 LID - 10.55563/clinexprheumatol/9bhd5d [doi] AB - OBJECTIVES: To evaluate the clinical, and serological features and treatments in two independent cohorts of Italian and Egyptian systemic sclerosis (SSc) patients, according to geographic areas. METHODS: 3 Italian and 5 Egyptian centres participated in patient recruitment in 2017. The demographic, clinical, and serological data were collected and defined according to the previously developed severity score and activity index. The database included 261 consecutive Italian patients (242 women/19 men) and 197 Egyptian patients (177 women/20 men), all of whom fulfilled the classification criteria of ACR/EULAR 2013 and criteria proposed by LeRoy and Medsger. RESULTS: Egyptians were younger, had an earlier onset of both the first non-Raynaud's and Raynaud's phenomenon and a more severe modified skin score. A greater percentage of Egyptians had the active form of the disease, a pulmonary arterial pressure estimated by echocardiography>35mmHg than Italians and interstitial lung disease. The severity score was higher in Egyptians, the frequency of anti-topoisomerase I (ATA) was higher in Italians, and Egyptians were more likely to be negative for both anticentromere and ATA antibodies than Italians. Egyptians had higher rates of synthetic disease-modifying antirheumatic drugs use than Italians; Italians but not Egyptians were under treatment with vasoactive therapy. Notably, Egyptians affected by the limited form of the disease exhibited a more severe clinical course when matched with their Italian counterparts, characterised by higher modified Rodnan skin score (mRSS), more frequent pulmonary involvement, increased frequency of ischaemic digital ulcers, earlier onset of symptoms, and higher severity scores. CONCLUSIONS: Clinical differences may be shown between Italian and Egyptian SSc patients. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. vasiliki.liakouli@unicampania.it, vasiliki_liakouli@yahoo.it. FAU - Hamoud, Hesham AU - Hamoud H AD - Department of Rheumatology, University of Al-Azhar, Cairo, Egypt. FAU - Risha, Mahmoud Ibrahim AU - Risha MI AD - Department of Rheumatology, University of Al-Azhar, Cairo, Egypt. FAU - Hanafy, Mohamed Elsayed AU - Hanafy ME AD - Department of Rheumatology, University of Al-Azhar, Cairo, Egypt. FAU - Moshrif, Abdelhafeez AU - Moshrif A AD - Department of Rheumatology, University of Al-Azhar, Cairo, Egypt. FAU - Abdel-Magied, Rasha A AU - Abdel-Magied RA AD - Department of Rheumatology and Rehabilitation, University of Minia, Cairo, Egypt. FAU - Omar, Gihan M AU - Omar GM AD - Department of Rheumatology and Rehabilitation, University of Minia, Cairo, Egypt. FAU - Elsayed, Adel M AU - Elsayed AM AD - Department of Rheumatology, University of Ain Shams, Cairo, Egypt. FAU - Elhefny, Abdelazeim AU - Elhefny A AD - Department of Rheumatology, University of Ain Shams, Cairo, Egypt. FAU - Mobasher, Sameh AU - Mobasher S AD - Department of Rheumatology, University of Ain Shams, Cairo, Egypt. FAU - Abo Gabal, Mervat AU - Abo Gabal M AD - Department of Rheumatology, University of Ain Shams, Cairo, Egypt. FAU - Hassanien, Manal AU - Hassanien M AD - Department of Physical Medicine, Rheumatology and Rehabilitation, University of Assuit, Egypt. FAU - El Nouby, Fatma H AU - El Nouby FH AD - Department of Physical Medicine, Rheumatology and Rehabilitation, University of Assuit, Egypt. FAU - Abdelgalil, Khaled AU - Abdelgalil K AD - Department of Rheumatology and Physical Medicine, University of Aswan, Egypt. FAU - Forte, Giulio AU - Forte G AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Navarini, Luca AU - Navarini L AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; and Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, Italy. FAU - Ciccia, Francesco AU - Ciccia F AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; and Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20250623 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - 0 (Antirheumatic Agents) RN - 0 (Biomarkers) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Age of Onset MH - Antibodies, Antinuclear/blood MH - Antirheumatic Agents/therapeutic use MH - Biomarkers/blood MH - Cross-Sectional Studies MH - DNA Topoisomerases, Type I/immunology MH - Egypt MH - Italy MH - *Scleroderma, Systemic/blood/diagnosis/drug therapy/immunology MH - Severity of Illness Index EDAT- 2025/06/25 06:25 MHDA- 2025/08/04 12:27 CRDT- 2025/06/25 04:23 PHST- 2025/02/15 00:00 [received] PHST- 2025/04/07 00:00 [accepted] PHST- 2025/08/04 12:27 [medline] PHST- 2025/06/25 06:25 [pubmed] PHST- 2025/06/25 04:23 [entrez] AID - 22241 [pii] AID - 10.55563/clinexprheumatol/9bhd5d [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Aug;43(8):1472-1480. doi: 10.55563/clinexprheumatol/9bhd5d. Epub 2025 Jun 23. PMID- 26028634 OWN - NLM STAT- MEDLINE DCOM- 20160309 LR - 20221115 IS - 1600-0617 (Electronic) IS - 0905-9180 (Print) IS - 0905-9180 (Linking) VI - 24 IP - 136 DP - 2015 Jun TI - Idiopathic inflammatory myopathies and the lung. PG - 216-38 LID - 10.1183/16000617.00002015 [doi] AB - Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. CI - Copyright ©ERS 2015. FAU - Lega, Jean-Christophe AU - Lega JC AD - Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, University of Lyon, Lyon, France jean-christophe.lega@chu-lyon.fr. FAU - Reynaud, Quitterie AU - Reynaud Q AD - Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France. FAU - Belot, Alexandre AU - Belot A AD - Dept of Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France. FAU - Fabien, Nicole AU - Fabien N AD - Dept of Immunology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France. FAU - Durieu, Isabelle AU - Durieu I AD - Dept of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France. FAU - Cottin, Vincent AU - Cottin V AD - National Reference Centre for Rare Pulmonary Diseases, Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, University of Lyon, Lyon, France. LA - eng PT - Journal Article PT - Review PL - England TA - Eur Respir Rev JT - European respiratory review : an official journal of the European Respiratory Society JID - 9111391 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) SB - IM EIN - Eur Respir Rev. 2015 Sep;24(137):545. doi: 10.1183/16000617.50002015.. Dosage error in article text. PMID: 26324818 MH - Animals MH - Autoantibodies/blood MH - Biomarkers/blood MH - Comorbidity MH - Disease Progression MH - Drug Therapy, Combination MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - *Lung/drug effects/immunology/physiopathology MH - Lung Diseases, Interstitial/diagnosis/epidemiology/immunology/physiopathology/*therapy MH - *Myositis/diagnosis/epidemiology/immunology/physiopathology/therapy MH - Predictive Value of Tests MH - Prognosis MH - Risk Factors MH - Time Factors PMC - PMC9487811 COIS- Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com EDAT- 2015/06/02 06:00 MHDA- 2016/03/10 06:00 PMCR- 2015/06/01 CRDT- 2015/06/02 06:00 PHST- 2015/06/02 06:00 [entrez] PHST- 2015/06/02 06:00 [pubmed] PHST- 2016/03/10 06:00 [medline] PHST- 2015/06/01 00:00 [pmc-release] AID - 24/136/216 [pii] AID - ERR-0020-2015 [pii] AID - 10.1183/16000617.00002015 [doi] PST - ppublish SO - Eur Respir Rev. 2015 Jun;24(136):216-38. doi: 10.1183/16000617.00002015. PMID- 22134565 OWN - NLM STAT- MEDLINE DCOM- 20120406 LR - 20151119 IS - 1545-9616 (Print) IS - 1545-9616 (Linking) VI - 10 IP - 12 DP - 2011 Dec TI - Is chronic cutaneous discoid lupus protective against severe renal disease in patients with systemic lupus erythematosus? PG - 1413-20 AB - OBJECTIVE: The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies. METHODS: Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation. RESULTS: Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal. CONCLUSION: Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation. FAU - Merola, Joseph F AU - Merola JF AD - Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA. jfmerola@partners.org FAU - Chang, Caroline A AU - Chang CA FAU - Sanchez, Miguel R AU - Sanchez MR FAU - Prystowsky, Stephen D AU - Prystowsky SD LA - eng PT - Journal Article PL - United States TA - J Drugs Dermatol JT - Journal of drugs in dermatology : JDD JID - 101160020 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/blood MH - Biomarkers/blood MH - Female MH - Humans MH - Lupus Erythematosus, Discoid/complications/*immunology MH - Lupus Erythematosus, Systemic/*complications MH - Lupus Nephritis/etiology MH - Male MH - Middle Aged MH - Renal Insufficiency/*etiology MH - Severity of Illness Index EDAT- 2011/12/03 06:00 MHDA- 2012/04/07 06:00 CRDT- 2011/12/03 06:00 PHST- 2011/12/03 06:00 [entrez] PHST- 2011/12/03 06:00 [pubmed] PHST- 2012/04/07 06:00 [medline] PST - ppublish SO - J Drugs Dermatol. 2011 Dec;10(12):1413-20. PMID- 29145709 OWN - NLM STAT- MEDLINE DCOM- 20190111 LR - 20220408 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 70 IP - 2 DP - 2018 Feb TI - Review: Defining a Unified Vascular Phenotype in Systemic Sclerosis. PG - 162-170 LID - 10.1002/art.40377 [doi] AB - Microcirculation impairment and related vasculopathy are hallmarks of systemic sclerosis (SSc). Digital ulceration is second only to Raynaud's phenomenon as a vascular complication occurring in patients with SSc. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers, and it is reasonable to question whether the outcomes of such patients might be different from those of patients who are not affected. Recently, several registries have provided relevant information about digital ulcers. Male sex and severe skin disease appear to be the main associated factors observed in several registries. However, limitations of those studies are the differences in the definitions of digital ulcers and organ involvement. Few longitudinal studies are available, and the more robust data from the European League Against Rheumatism Scleroderma Trial and Research cohort suggested worse outcomes in patients with a history of digital ulcers but could not demonstrate that a history of digital ulcers can predict additional vascular complications such as pulmonary arterial hypertension, heart failure, or renal crisis. Nevertheless, the autopsy studies published many years ago and the more recent longitudinal biomarker studies support the concept of generalized vasculopathy and a potential association between various cardiovascular complications. It is expected that with the availability of several structured registries, identification of a vascular profile or vascular phenotype will be addressed using more robust data in the near future. CI - © 2017, American College of Rheumatology. FAU - Allanore, Yannick AU - Allanore Y AD - Cochin Hospital, INSERM U1016, Paris Descartes University, Paris, France. FAU - Distler, Oliver AU - Distler O AD - University Hospital Zurich, Zurich, Switzerland. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy. FAU - Denton, Christopher P AU - Denton CP AD - University College London and Royal Free Hospital, London, UK. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20180122 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Biomarkers) SB - IM MH - Biomarkers MH - Female MH - Fingers/blood supply/pathology MH - Humans MH - Male MH - Microcirculation MH - Phenotype MH - Risk Factors MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/etiology MH - Vascular Diseases/*etiology EDAT- 2017/11/18 06:00 MHDA- 2019/01/12 06:00 CRDT- 2017/11/18 06:00 PHST- 2017/09/14 00:00 [received] PHST- 2017/11/10 00:00 [accepted] PHST- 2017/11/18 06:00 [pubmed] PHST- 2019/01/12 06:00 [medline] PHST- 2017/11/18 06:00 [entrez] AID - 10.1002/art.40377 [doi] PST - ppublish SO - Arthritis Rheumatol. 2018 Feb;70(2):162-170. doi: 10.1002/art.40377. Epub 2018 Jan 22. PMID- 24517557 OWN - NLM STAT- MEDLINE DCOM- 20150511 LR - 20161020 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 24 IP - 5 DP - 2014 Sep TI - Anti-Jo-1 myositis and the antiphospholipid syndrome showing right ventricular thrombus: a novel overlap syndrome with atypical presentation. PG - 865-8 LID - 10.3109/14397595.2013.874741 [doi] AB - It has long been recognized that patients with myositis and positive anti-Jo1 antibody tend to be associated with interstitial lung disease. Recent studies revealed that such patients may also have fever, Raynaud's phenomenon, mechanic's hand, polyarthralgia, or usually mild, self-limiting, non-erosive or erosive polyarthritis known as antisynthetase syndrome. The hallmark of this disorder is the presence of the autoantibodies that recognize the aminoacyl-tRNA synthetases, which play a critical role in protein synthesis. The most well recognized of the autoantibodies is anti-histidyl (Jo-1). Antisynthetase syndrome cases associated with other autoimmune diseases are rarely reported. We here present a case of antisynthetase syndrome presented with right ventricle thrombus and deep vein thrombosis in the lower limbs. Secondary antiphospholipid syndrome was then diagnosed after a series of examinations. The patient was successfully treated with anticoagulant alone without surgical thrombectomy. Our case revealed that clinical physicians should watch for thrombotic complications when facing patients with antisynthetase syndrome. Medical therapy with anticoagulants alone may be an alternative treatment option in patients with right ventricle thrombus who cannot tolerate surgical thrombectomy. FAU - Wang, Ching-Hsun AU - Wang CH AD - Division of Infectious Diseases and Tropical Medicine, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center , Taipei , Taiwan. FAU - Wang, Ning-Chi AU - Wang NC FAU - Lin, Te-Yu AU - Lin TY FAU - Chen, Chen-Hung AU - Chen CH LA - eng PT - Case Reports PT - Journal Article DEP - 20140211 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Antibodies, Antinuclear/*blood MH - Antiphospholipid Syndrome/blood/*complications/immunology MH - Heart Diseases/blood/*complications/immunology MH - Humans MH - Male MH - Middle Aged MH - Myositis/blood/*complications/immunology MH - Thrombosis/blood/*complications/immunology OTO - NOTNLM OT - Antiphospholipid syndrome OT - Antisynthetase syndrome OT - Polymyositis EDAT- 2014/02/13 06:00 MHDA- 2015/05/12 06:00 CRDT- 2014/02/13 06:00 PHST- 2014/02/13 06:00 [entrez] PHST- 2014/02/13 06:00 [pubmed] PHST- 2015/05/12 06:00 [medline] AID - 10.3109/14397595.2013.874741 [doi] PST - ppublish SO - Mod Rheumatol. 2014 Sep;24(5):865-8. doi: 10.3109/14397595.2013.874741. Epub 2014 Feb 11. PMID- 40630797 OWN - NLM STAT- MEDLINE DCOM- 20250709 LR - 20260430 IS - 1916-7245 (Electronic) IS - 1198-2241 (Print) IS - 1198-2241 (Linking) VI - 2025 DP - 2025 TI - The Clinical Efficacy and Safety of Nintedanib in the Treatment of Interstitial Lung Disease Among Patients With Systemic Sclerosis: Systematic Review. PG - 1682546 LID - 10.1155/carj/1682546 [doi] LID - 1682546 AB - Systemic sclerosis (SSc) is predominantly characterized by an array of cutaneous manifestations including Raynaud's phenomenon, calcinosis, telangiectasias, and skin fibrosis contributing toward substantial morbidity and diminished quality of life. The monumental impact of the disease regarding mortality is due to its pulmonary involvement known as SSc-associated interstitial lung disease (SSc-ILD). Currently, treatment is chiefly directed toward impeding disease progression with the mainstay treatment approaches involving the utilization of cyclophosphamide, mycophenolate mofetil, rituximab, and tocilizumab. Recently, a tyrosine kinase inhibitor, nintedanib, has been approved for the treatment of SSc-ILD and thus became the first medication to be fully licensed for SSc-ILD. A systematic review based on the Preferred Reporting Items of Systematic Review with Meta-analysis (PRISMA) was conducted after successful registration in PROSPERO to evaluate the efficacy and safety of nintedanib in SSc-ILD. We searched PubMed, Scopus, and CENTRAL up to the first of September 2023 utilizing the following keywords: ((Diffuse Parenchymal Lung Disease) OR (Diffuse Parenchymal Lung Diseases) OR (Interstitial Lung Disease) OR (Interstitial Lung Diseases) OR (Interstitial Pneumonia) OR (Interstitial Pneumonitis) OR (Pulmonary Fibrosis)) AND ((Systemic Scleroderma) OR (Systemic Scleroderma)) AND ((BIBF 1120) OR (BIBF-1120) OR (BIBF1120) OR (Nintedanib esylate) OR (Ofev) OR (Vargatef)). The clinical safety profile of nintedanib was deemed more favorable than other therapeutic regimens currently utilized, in addition to adequate clinical efficacy toward SSc-ILD. CI - Copyright © 2025 Khaled S. Al Oweidat et al. Canadian Respiratory Journal published by John Wiley & Sons Ltd. FAU - Al Oweidat, Khaled S AU - Al Oweidat KS AD - Department of Respiratory and Sleep Medicine, Department of Internal Medicine, The University of Jordan, Amman, Jordan. FAU - Abdulelah, Ahmed A AU - Abdulelah AA AUID- ORCID: 0000-0001-9474-1106 AD - School of Medicine, The University of Jordan, Amman, Jordan. FAU - Toubasi, Ahmad A AU - Toubasi AA AUID- ORCID: 0000-0002-4688-9728 AD - Faculty of Medicine, The University of Jordan, Amman, Jordan. FAU - Abdulelah, Mohammad AU - Abdulelah M AD - Department of Internal Medicine, University of Massachusetts Chan Medical School-Baystate Campus, Springfield, Massachusetts, USA. FAU - Alatteili, Nour Z AU - Alatteili NZ AD - Faculty of Medicine, The University of Jordan, Amman, Jordan. FAU - Abdulelah, Zaid A AU - Abdulelah ZA AUID- ORCID: 0000-0002-1952-4627 AD - Department of Cardiology, Royal Papworth Hospital, Cambridge, UK. LA - eng PT - Journal Article PT - Systematic Review DEP - 20250701 PL - United States TA - Can Respir J JT - Canadian respiratory journal JID - 9433332 RN - G6HRD2P839 (nintedanib) RN - 0 (Indoles) RN - 0 (Protein Kinase Inhibitors) SB - IM MH - Humans MH - *Lung Diseases, Interstitial/drug therapy/etiology MH - *Scleroderma, Systemic/complications MH - *Indoles/therapeutic use/adverse effects MH - Treatment Outcome MH - *Protein Kinase Inhibitors/therapeutic use/adverse effects PMC - PMC12237565 OTO - NOTNLM OT - autoimmune lung disease OT - scleroderma OT - systemic sclerosis OT - tyrosine kinase inhibitor COIS- The authors declare no conflicts of interest. EDAT- 2025/07/09 06:27 MHDA- 2025/07/09 06:28 PMCR- 2025/07/01 CRDT- 2025/07/09 05:03 PHST- 2023/10/25 00:00 [received] PHST- 2025/05/11 00:00 [revised] PHST- 2025/05/30 00:00 [accepted] PHST- 2025/07/09 06:28 [medline] PHST- 2025/07/09 06:27 [pubmed] PHST- 2025/07/09 05:03 [entrez] PHST- 2025/07/01 00:00 [pmc-release] AID - 10.1155/carj/1682546 [doi] PST - epublish SO - Can Respir J. 2025 Jul 1;2025:1682546. doi: 10.1155/carj/1682546. eCollection 2025. PMID- 32697139 OWN - NLM STAT- MEDLINE DCOM- 20210920 LR - 20210920 IS - 2472-5625 (Electronic) IS - 2472-5625 (Linking) VI - 5 IP - 1 DP - 2021 Jan TI - Antisynthetase syndrome and pulmonary hypertension: report of two cases and review of the literature. PG - 152-155 LID - 10.1080/24725625.2020.1794521 [doi] AB - Antisynthetase Syndrome (ASS) is a subset of idiopathic inflammatory myopathies characterised by specific clinical features such as interstitial lung disease (ILD), fever, myositis, Raynaud's phenomenon, cutaneous involvement and arthritis related to the presence of anti-aminoacyl-tRNA-synthetase (anti-ARS) autoantibodies. Moreover, Pulmonary arterial hypertension (PAH) is a life-threatening complication associated with connective tissue diseases mainly systemic sclerosis (SSc-PAH). It has been suggested that PAH can complicate ASS patients but little is known about the prevalence and risk factors to develop this complication. Here we report on two patients with ASS and PH. The first one represents a complete picture of ASS anti-Jo-1 positive, the second an amyophatic ASS anti-PL-12 positive. In one of our ASS-PAH patients, specific treatment lead to improvement of PAH. There are no specific recommendations on current guidelines regarding either PAH screening or treatment in ASS, but performing echocardiogram, ECG, pulmonary function test and prompt initiation of specific therapies seems to improve right heart catheterisation (RHC) parameters and survival. FAU - García-Fernández, Antía AU - García-Fernández A AD - Rheumatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Quezada-Loaiza, Carlos Andrés AU - Quezada-Loaiza CA AD - Pneumology Department, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain. FAU - de la Puente-Bujidos, Carlos AU - de la Puente-Bujidos C AD - Rheumatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20200722 PL - England TA - Mod Rheumatol Case Rep JT - Modern rheumatology case reports JID - 101761026 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/immunology MH - Autoantibodies/blood MH - Female MH - Humans MH - Middle Aged MH - Myositis/*complications/immunology MH - Pulmonary Arterial Hypertension/*complications/immunology MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Antisynthetase syndrome OT - anti-ARS antibodies OT - connective tissue diseases OT - myositis OT - pulmonary hypertension EDAT- 2020/07/23 06:00 MHDA- 2021/09/21 06:00 CRDT- 2020/07/23 06:00 PHST- 2020/07/23 06:00 [pubmed] PHST- 2021/09/21 06:00 [medline] PHST- 2020/07/23 06:00 [entrez] AID - 10.1080/24725625.2020.1794521 [doi] PST - ppublish SO - Mod Rheumatol Case Rep. 2021 Jan;5(1):152-155. doi: 10.1080/24725625.2020.1794521. Epub 2020 Jul 22. PMID- 31334060 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220409 IS - 2229-5178 (Print) IS - 2249-5673 (Electronic) IS - 2229-5178 (Linking) VI - 10 IP - 4 DP - 2019 Jul-Aug TI - An Observational Cross-Sectional Study of Varied Clinical Manifestations of Connective Tissue Disorders and their Association with Antinuclear Antibodies in a Tertiary Care Center. PG - 413-417 LID - 10.4103/idoj.IDOJ_398_18 [doi] AB - CONTEXT: Connective tissue disorders (CTD) occur in 3-5% of the population. The advent of antibodies to extractable nuclear antigens (ENA) has become a reliable predictor to establish the diagnosis of CTD, subclassify patients into prognostic groups, and monitor disease activity. AIMS: The aim of this study was to (a) study the frequency of cutaneous manifestations, systemic manifestations, and anti-ENA antibodies in CTD; (b) determine the association between systemic manifestations and ENAs; and (c) determine the association between cutaneous and systemic manifestations of CTD. SUBJECTS AND MATERIALS: An observational cross- sectional study was conducted on 50 patients diagnosed to have CTD. The clinical profile and antibodies to ENA (ANA Profile) reports were retrieved and studied. RESULTS: The major dermatological manifestations were skin tightness (36%), salt and pepper pigmentation (30%), Raynaud's phenomenon (28%), and malar rash (28%). The common antibodies seen were anti SS-A (36%), anti-UI-ribonucleoprotein (U1-RNP) (34%), anti-dsDNA (32%), and anti-Sm (24%). Patients with anti-Sm and anti-dsDNA antibodies had increased frequency of renal manifestations. A strong association with significant P values was seen between neurological manifestations and anti-Sm antibody, and cardiovascular manifestations and anti-RNP antibody. An association between gastrointestinal manifestations and malar rash as well as neurological manifestations and photosensitivity was also seen. CONCLUSIONS: ENA panel predicts systemic involvement, thus helping in the multidisciplinary management. Cutaneous manifestations of CTD can be an early predictor in giving a clue to impending systemic manifestations. FAU - Kadiru, Rana Abdul AU - Kadiru RA AD - Department of Dermatology, Yenepoya Medical College and University, Mangalore, Karnataka, India. FAU - Hegde, Spandana P AU - Hegde SP AD - Department of Dermatology, Yenepoya Medical College and University, Mangalore, Karnataka, India. FAU - Shenoy, Manjunath M AU - Shenoy MM AD - Department of Dermatology, Yenepoya Medical College and University, Mangalore, Karnataka, India. LA - eng PT - Journal Article PL - India TA - Indian Dermatol Online J JT - Indian dermatology online journal JID - 101586880 PMC - PMC6615393 OTO - NOTNLM OT - Connective tissue disorders OT - cutaneous manifestations OT - extractable nuclear antigens OT - systemic manifestations COIS- There are no conflicts of interest. EDAT- 2019/07/25 06:00 MHDA- 2019/07/25 06:01 PMCR- 2019/07/01 CRDT- 2019/07/24 06:00 PHST- 2019/07/24 06:00 [entrez] PHST- 2019/07/25 06:00 [pubmed] PHST- 2019/07/25 06:01 [medline] PHST- 2019/07/01 00:00 [pmc-release] AID - IDOJ-10-413 [pii] AID - 10.4103/idoj.IDOJ_398_18 [doi] PST - ppublish SO - Indian Dermatol Online J. 2019 Jul-Aug;10(4):413-417. doi: 10.4103/idoj.IDOJ_398_18. PMID- 21955007 OWN - NLM STAT- MEDLINE DCOM- 20120504 LR - 20181201 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 39 IP - 1 DP - 2012 Jan TI - Bosentan for digital ulcers in patients with systemic sclerosis. PG - 48-51 LID - 10.1111/j.1346-8138.2011.01299.x [doi] AB - Recurrent digital ulcers are manifestations of vascular disease in patients with systemic sclerosis (SSc). We report six patients with severe digital ulcers who were treated with bosentan administered p.o., 62.5-125 mg daily. The mean duration from the diagnosis of SSc to the initiation of bosentan was 9.5 years, and the observation period after bosentan administration was from 7 months to 4.5 years. In case 1, neither new digital ulcers nor Raynaud's phenomenon developed for 4.5 years. In case 2, digital ulcers recurred after the discontinuation of bosentan; however, re-administration of bosentan lead to the improvement. In cases 3-5 with recurrent digital ulcers, no new lesions have developed. In these five patients, pain evaluated by visual analog scale was significantly reduced. In three patients, bosentan was discontinued because of severe liver dysfunction. These results suggest that bosentan is an effective treatment for refractory digital ulcers associated with SSc; however, liver function should be carefully monitored. Compared to the doses of bosentan used to treat pulmonary hypertension, relatively lower doses may effectively control painful digital ulcer/gangrene in patients with SSc. CI - © 2011 Japanese Dermatological Association. FAU - Nagai, Yayoi AU - Nagai Y AD - Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan. yanagai@showa.gunma-u.ac.jp FAU - Hasegawa, Michiko AU - Hasegawa M FAU - Hattori, Tomoyasu AU - Hattori T FAU - Okada, Etsuko AU - Okada E FAU - Tago, Osamu AU - Tago O FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article DEP - 20110928 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Antihypertensive Agents/*therapeutic use MH - Bosentan MH - Chemical and Drug Induced Liver Injury/etiology MH - Female MH - Hand Dermatoses/*drug therapy/etiology MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology MH - Sulfonamides/*therapeutic use MH - Treatment Outcome MH - Young Adult EDAT- 2011/10/01 06:00 MHDA- 2012/05/05 06:00 CRDT- 2011/09/30 06:00 PHST- 2011/09/30 06:00 [entrez] PHST- 2011/10/01 06:00 [pubmed] PHST- 2012/05/05 06:00 [medline] AID - 10.1111/j.1346-8138.2011.01299.x [doi] PST - ppublish SO - J Dermatol. 2012 Jan;39(1):48-51. doi: 10.1111/j.1346-8138.2011.01299.x. Epub 2011 Sep 28. PMID- 18476458 OWN - NLM STAT- MEDLINE DCOM- 20080603 LR - 20170306 VI - 118 IP - 3 DP - 2008 Mar TI - Coexistence of systemic sclerosis, scleroderma-like syndromes and neoplastic diseases. PG - 119-26 AB - Coexistence of rheumatic and neoplastic diseases may take different forms. Rheumatic paraneoplastic syndromes, including systemic sclerosis, scleroderma-like changes and Raynaud's phenomenon are induced by substances secreted by neoplastic cells and immunological disturbances connected are associated with malignancy. They may precede the clinical manifestation of neoplasm, occur simultaneously or after its diagnosis. In turn, chronic course of rheumatic diseases (Sjögren's syndrome, systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis) by immunologic stimulation may promote carcinogenesis. Genetic, environmental factors (viruses, chemical substances, radiation) and alterations of immunological surveillance may be the cause of both rheumatic and paraneoplastic disorders. Anticancer therapy may cause rheumatic diseases and immunosuppressive agents used in patients with rheumatic syndromes may have carcinogenic effect. Patients with long-standing or atypical course of rheumatic disorders, positive family or personal history of neoplastic disease, positive cancer markers, monoclonal antibodies or presence of other paraneoplastic syndromes should be diagnosed as possibly having occult neoplasm. In this paper we reviewed available literature on coexistence of rheumatic processes and malignancies to attract particular attention to practical aspects of this vital issue. FAU - Ciołkiewicz, Mariusz AU - Ciołkiewicz M AD - Department of General and Experimental Pathology, Medical University of Białystok, NZOZ Vitamed Hospice House, Białystok, Poland. FAU - Domysławska, Izabela AU - Domysławska I FAU - Ciołkiewicz, Agata AU - Ciołkiewicz A FAU - Klimiuk, Piotr Adrian AU - Klimiuk PA FAU - Kuryliszyn-Moskal, Anna AU - Kuryliszyn-Moskal A LA - eng PT - Journal Article PT - Review PL - Poland TA - Pol Arch Med Wewn JT - Polskie Archiwum Medycyny Wewnetrznej JID - 0401225 SB - IM MH - Comorbidity MH - Dermatomyositis/epidemiology MH - Environmental Exposure MH - Humans MH - Immune System Diseases/epidemiology MH - Lupus Erythematosus, Systemic/epidemiology MH - Neoplasms, Unknown Primary/diagnosis/*epidemiology MH - Paraneoplastic Syndromes/diagnosis/*epidemiology MH - Precancerous Conditions/diagnosis/*epidemiology MH - Rheumatic Diseases/diagnosis/*epidemiology MH - Risk Factors MH - Scleroderma, Systemic/epidemiology MH - Sjogren's Syndrome/epidemiology RF - 81 EDAT- 2008/05/15 09:00 MHDA- 2008/06/05 09:00 CRDT- 2008/05/15 09:00 PHST- 2008/05/15 09:00 [pubmed] PHST- 2008/06/05 09:00 [medline] PHST- 2008/05/15 09:00 [entrez] PST - ppublish SO - Pol Arch Med Wewn. 2008 Mar;118(3):119-26. PMID- 38474309 OWN - NLM STAT- MEDLINE DCOM- 20240314 LR - 20240315 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 25 IP - 5 DP - 2024 Mar 6 TI - The Potential of Twendee X(®) as a Safe Antioxidant Treatment for Systemic Sclerosis. LID - 10.3390/ijms25053064 [doi] LID - 3064 AB - Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic skin hardening, which combines Raynaud's phenomenon and other vascular disorders, skin and internal organ fibrosis, immune disorders, and a variety of other abnormalities. Symptoms vary widely among individuals, and personalized treatment is sought for each patient. Since there is no fundamental cure for SSc, it is designated as an intractable disease with patients receiving government subsidies for medical expenses in Japan. Oxidative stress (OS) has been reported to play an important role in the cause and symptoms of SSc. HOCl-induced SSc mouse models are known to exhibit skin and visceral fibrosis, vascular damage, and autoimmune-like symptoms observed in human SSc. The antioxidant combination Twendee X(®) (TwX) is a dietary supplement consisting of vitamins, amino acids, and CoQ10. TwX has been proven to prevent dementia in humans with mild cognitive impairment and significantly improve cognitive impairment in an Alzheimer's disease mouse model by regulating OS through a strong antioxidant capacity that cannot be achieved with a single antioxidant ingredient. We evaluated the effectiveness of TwX on various symptoms of HOCl-induced SSc mice. TwX-treated HOCl-induced SSc mice showed significantly reduced lung and skin fibrosis compared to untreated HOCl-induced SSc mice. TwX also significantly reduced highly oxidized protein products (AOPP) in serum and suppressed Col-1 gene expression and activation of B cells involved in autoimmunity. These findings suggest that TwX has the potential to be a new antioxidant treatment for SSc without side effects. FAU - You, Fukka AU - You F AUID- ORCID: 0009-0003-2561-0796 AD - Division of Anti-Oxidant Research, Life Science Research Center, Gifu University, Yanagito 1-1, Gifu 501-1194, Japan. AD - Anti-Oxidant Research Laboratory, Louis Pasteur Center for Medical Research, Tanakamonzen-cho 103-5, Sa-kyo-ku, Kyoto 606-8225, Japan. FAU - Nicco, Carole AU - Nicco C AUID- ORCID: 0000-0001-8211-2556 AD - Université Paris Cité, 45 Rue des Saints-Pères, 75006 Paris, France. FAU - Harakawa, Yoshiaki AU - Harakawa Y AUID- ORCID: 0009-0000-0612-7078 AD - Division of Anti-Oxidant Research, Life Science Research Center, Gifu University, Yanagito 1-1, Gifu 501-1194, Japan. FAU - Yoshikawa, Toshikazu AU - Yoshikawa T AD - Louis Pasteur Center for Medical Research, Tanakamonzen-cho 103-5, Sakyo-ku, Kyoto 606-8225, Japan. AD - School of Medicine, Kyoto Prefectural University of Medicine, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan. FAU - Inufusa, Haruhiko AU - Inufusa H AUID- ORCID: 0009-0004-3355-201X AD - Division of Anti-Oxidant Research, Life Science Research Center, Gifu University, Yanagito 1-1, Gifu 501-1194, Japan. AD - Anti-Oxidant Research Laboratory, Louis Pasteur Center for Medical Research, Tanakamonzen-cho 103-5, Sa-kyo-ku, Kyoto 606-8225, Japan. LA - eng GR - 20170101/TIMA Establishment (Liechtenstein)/ PT - Journal Article DEP - 20240306 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 RN - 0 (Antioxidants) RN - 0 (Twendee X) RN - PQ6CK8PD0R (Ascorbic Acid) RN - 48TCX9A1VT (Cystine) RN - 0RH81L854J (Glutamine) SB - IM MH - Humans MH - Mice MH - Animals MH - *Antioxidants/pharmacology MH - *Scleroderma, Systemic/metabolism MH - Dietary Supplements MH - Fibrosis MH - Skin/metabolism MH - Disease Models, Animal MH - *Ascorbic Acid MH - *Cystine MH - *Glutamine PMC - PMC10932212 OTO - NOTNLM OT - Twendee X® OT - antioxidant OT - autoimmunity OT - oxidative stress OT - systemic sclerosis COIS- F.Y., Y.H., and H.I. are employees of Gifu University. The Division of Antioxidant Research is a laboratory that has been established at the Life Science Research Center at Gifu University, based on a research fund from the TIMA Establishment (Liechtenstein). T.Y. is an advisor to TIMA Establishment (Liechtenstein). The sponsor had no control over the interpretation, writing, or publication of this work. EDAT- 2024/03/13 06:46 MHDA- 2024/03/14 06:47 PMCR- 2024/03/06 CRDT- 2024/03/13 01:25 PHST- 2024/01/29 00:00 [received] PHST- 2024/03/02 00:00 [revised] PHST- 2024/03/04 00:00 [accepted] PHST- 2024/03/14 06:47 [medline] PHST- 2024/03/13 06:46 [pubmed] PHST- 2024/03/13 01:25 [entrez] PHST- 2024/03/06 00:00 [pmc-release] AID - ijms25053064 [pii] AID - ijms-25-03064 [pii] AID - 10.3390/ijms25053064 [doi] PST - epublish SO - Int J Mol Sci. 2024 Mar 6;25(5):3064. doi: 10.3390/ijms25053064. PMID- 28420047 OWN - NLM STAT- MEDLINE DCOM- 20180521 LR - 20260127 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 26 IP - 11 DP - 2017 Oct TI - Organ damage accrual and distribution in systemic lupus erythematosus patients followed-up for more than 10 years. PG - 1197-1204 LID - 10.1177/0961203317693096 [doi] AB - Objective The aim of this study was to determine the prevalence, predictors and progression of organ damage in a monocentric cohort of systemic lupus erythematosus patients with a long follow-up. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index one year after diagnosis and every five years. Disease activity was measured by the systemic lupus erythematosus disease activity index (SLEDAI)-2K at the beginning of the follow-up. Univariate and multivariable analyses were used to detect items associated with damage. A total of 511 systemic lupus erythematosus patients (92% females, 95% Caucasian), prospectively followed from 1972 to 2014, were included. Results After a mean disease duration of 16 years (SD: 9.5) and a mean follow-up of 12.9 years (SD: 8.8), 354 patients (69.3%) had accrued some damage: 49.7% developed mild/moderate damage, while 19.5% showed severe damage. Damage was evident in 40% of 511 patients one year after diagnosis, and its prevalence linearly increased over time. Longer disease duration, higher SLEDAI, severe Raynaud's, chronic alopecia and cerebral ischaemia were significantly associated with organ damage. No associations between damage and autoantibodies, including anti-dsDNA, anti-Sm or antiphospholipid antibodies, were observed. Anyway, antiphospholipid syndrome and anticardiolipin antibodies predicted the development of neuropsychiatric damage. The ocular, musculoskeletal and neuropsychiatric systems were the most frequently damaged organs, with a linear increase during follow-up. Conclusion A high rate of moderate and severe damage has been detected early in a wide cohort of young lupus patients, with a linear trend of increase over time. Disease activity and long duration of disease predict damage, while antiphospholipid antibodies play a role in determining neuropsychiatric damage. FAU - Taraborelli, M AU - Taraborelli M AD - 1 Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, Brescia, Italy. FAU - Cavazzana, I AU - Cavazzana I AD - 1 Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, Brescia, Italy. FAU - Martinazzi, N AU - Martinazzi N AD - 2 Rheumatology and Clinical Immunology Department, University of Brescia, Brescia, Italy. FAU - Lazzaroni, M Grazia AU - Lazzaroni MG AD - 2 Rheumatology and Clinical Immunology Department, University of Brescia, Brescia, Italy. FAU - Fredi, M AU - Fredi M AD - 1 Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, Brescia, Italy. FAU - Andreoli, L AU - Andreoli L AD - 2 Rheumatology and Clinical Immunology Department, University of Brescia, Brescia, Italy. FAU - Franceschini, F AU - Franceschini F AD - 1 Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, Brescia, Italy. FAU - Tincani, A AU - Tincani A AD - 1 Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, Brescia, Italy. AD - 2 Rheumatology and Clinical Immunology Department, University of Brescia, Brescia, Italy. LA - eng PT - Journal Article DEP - 20170222 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Biomarkers) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/blood MH - Biomarkers/blood MH - Chi-Square Distribution MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Italy/epidemiology MH - Linear Models MH - Logistic Models MH - Lupus Erythematosus, Systemic/blood/diagnosis/*epidemiology/therapy MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Prevalence MH - Prognosis MH - Retrospective Studies MH - Risk Factors MH - Severity of Illness Index MH - Time Factors MH - Young Adult OTO - NOTNLM OT - Systemic lupus erythematosus OT - antiphospholipid antibodies OT - damage OT - disease activity OT - neuropsychiatric damage EDAT- 2017/04/20 06:00 MHDA- 2018/05/22 06:00 CRDT- 2017/04/20 06:00 PHST- 2017/04/20 06:00 [pubmed] PHST- 2018/05/22 06:00 [medline] PHST- 2017/04/20 06:00 [entrez] AID - 10.1177/0961203317693096 [doi] PST - ppublish SO - Lupus. 2017 Oct;26(11):1197-1204. doi: 10.1177/0961203317693096. Epub 2017 Feb 22. PMID- 34246388 OWN - NLM STAT- MEDLINE DCOM- 20211125 LR - 20211204 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 160 IP - 1 DP - 2021 Jul TI - A 12-Year-Old Girl Presenting With Recurrent Dyspnea and Pulmonary Ground-Glass Opacities. PG - e45-e50 LID - S0012-3692(21)00258-0 [pii] LID - 10.1016/j.chest.2021.02.002 [doi] AB - A 12-year-old girl presented with shortness of breath with exercise for 2 weeks. Her oxygen saturation was 85% during exercise. Birth and family histories were unremarkable. The girl was healthy until 7.1 years of age, when she suffered a "pneumonia" with fever, dyspnea, and hypoxemia, which diminished after a 19-day treatment with antibiotics and methylprednisolone. These symptoms relapsed 8 months later, and she was diagnosed with rapidly progressive interstitial lung disease (ILD) and a Mycoplasma pneumoniae infection. At that time, her symptoms failed to respond to a course of antibiotic therapy but resolved with IV methylprednisolone at 2.7 mg/kg/day. She remained on a tapering dose of methylprednisolone plus methotrexate for the next 18 months until withdrawal of these medications because of return of almost normal lung imaging. She had never had myalgia, muscle weakness, arthritis, rashes, mechanic's hands, Raynaud's phenomenon, dry mouth, or dry eyes. CI - Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. FAU - Qin, Xu AU - Qin X AD - Department of Pediatric Pulmonology, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Chen, Xiaobo AU - Chen X AD - Department of Critical Care and Respiratory Medicine, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Deng, Yu AU - Deng Y AD - Department of Radiology, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Gu, Ying-Ying AU - Gu YY AD - Department of Pathology, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Zeng, Lijun AU - Zeng L AD - Department of Pediatric Pulmonology, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Huang, Shunkai AU - Huang S AD - Department of Pediatric Pulmonology, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Sun, Lihong AU - Sun L AD - Department of Pediatric Pulmonology, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. FAU - Li, Shiyue AU - Li S AD - Department of Critical Care and Respiratory Medicine, Guangzhou Institute of Respiratory Health, First Affiliated Hospital of Guangzhou Medical University, Guangdong, China. Electronic address: sunlihong@gird.cn. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - EC 6.- (Ligases) SB - IM MH - Antibodies, Antinuclear/*blood MH - Child MH - Dyspnea/diagnosis/*etiology MH - Female MH - Humans MH - Ligases/*metabolism MH - Lung/*diagnostic imaging/metabolism MH - Lung Diseases, Interstitial/*diagnosis/enzymology MH - Magnetic Resonance Imaging MH - Syndrome MH - Tomography, X-Ray Computed EDAT- 2021/07/12 06:00 MHDA- 2021/11/26 06:00 CRDT- 2021/07/11 20:24 PHST- 2020/09/03 00:00 [received] PHST- 2021/01/20 00:00 [revised] PHST- 2021/02/03 00:00 [accepted] PHST- 2021/07/11 20:24 [entrez] PHST- 2021/07/12 06:00 [pubmed] PHST- 2021/11/26 06:00 [medline] AID - S0012-3692(21)00258-0 [pii] AID - 10.1016/j.chest.2021.02.002 [doi] PST - ppublish SO - Chest. 2021 Jul;160(1):e45-e50. doi: 10.1016/j.chest.2021.02.002. PMID- 32149834 OWN - NLM STAT- MEDLINE DCOM- 20210726 LR - 20210726 IS - 1533-0311 (Electronic) IS - 0193-1091 (Linking) VI - 42 IP - 10 DP - 2020 Oct TI - Morphea With Keloidal Features: A Case Report and Review of the Literature. PG - 766-768 LID - 10.1097/DAD.0000000000001629 [doi] AB - Keloidal morphea is a rare variant of scleroderma, which often can be clinically confused with keloid or scar formation. We report a 34-year-old woman with a medical history of asthma and Raynaud's phenomenon, presented for the evaluation and management of multiple erythematous hyperpigmented annular plaques reportedly developed after taking trimethoprim/sulfamethoxazole. An initial skin biopsy showed findings supportive of a drug eruption. She was treated with oral prednisone and achieved some improvement. She presented 1 year later with enlargement of the plaques and emergence of new lesions. Skin biopsies revealed an unremarkable epidermis with marked fibrosis of the mid-to-deep dermis with sparing of the papillary dermis, and superficial and deep perivascular and perieccrine lymphoplasmacytic inflammation. Verhoeff-Van Gieson staining demonstrated the loss of elastin fibers within the fibrotic areas of the biopsy specimens, which supported the diagnosis of keloidal morphea. Her laboratory tests were positive for antinuclear antibody (greater than 1:1280). She continued treatment with oral prednisone and topical steroids, and she showed improvement. This case highlights the importance of differentiating keloidal scleroderma from a hypertrophic scar or keloid to reveal an underlying systemic process. A correlation of clinical and histopathological findings is paramount to reach a correct diagnosis, ensure appropriate treatment, and monitor for comorbid disease. FAU - Yu, Dongfang AU - Yu D AD - Departments of Pathology, and. FAU - Ibarra, Barbara S AU - Ibarra BS AD - Departments of Pathology, and. FAU - Akkurt, Zeynep M AU - Akkurt ZM AD - Dermatology, Wake Forest University School of Medicine, Winston Salem, NC. FAU - Ahn, Christine AU - Ahn C AD - Departments of Pathology, and. AD - Dermatology, Wake Forest University School of Medicine, Winston Salem, NC. FAU - Sangüeza, Omar P AU - Sangüeza OP AD - Departments of Pathology, and. AD - Dermatology, Wake Forest University School of Medicine, Winston Salem, NC. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - Am J Dermatopathol JT - The American Journal of dermatopathology JID - 7911005 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Biopsy MH - Diagnosis, Differential MH - Erythema/pathology MH - Female MH - Humans MH - Hyperpigmentation/pathology MH - Keloid/diagnosis/pathology MH - Scleroderma, Localized/*diagnosis/drug therapy/*pathology MH - Skin/*pathology EDAT- 2020/03/10 06:00 MHDA- 2021/07/27 06:00 CRDT- 2020/03/10 06:00 PHST- 2020/03/10 06:00 [pubmed] PHST- 2021/07/27 06:00 [medline] PHST- 2020/03/10 06:00 [entrez] AID - 00000372-202010000-00008 [pii] AID - 10.1097/DAD.0000000000001629 [doi] PST - ppublish SO - Am J Dermatopathol. 2020 Oct;42(10):766-768. doi: 10.1097/DAD.0000000000001629. PMID- 28429690 OWN - NLM STAT- MEDLINE DCOM- 20170911 LR - 20181023 IS - 1308-4488 (Electronic) IS - 1016-5169 (Linking) VI - 45 IP - 3 DP - 2017 Apr TI - [Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children]. PG - 227-234 LID - 10.5543/tkda.2017.36517 [doi] AB - OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded. Tilt table test, 24-hour Holter rhythm monitoring, and exercise test were performed, as required. Patients were diagnosed as vasovagal syncope, postural orthostatic tachycardia syndrome (POTS), or inappropriate sinus tachycardia based on these findings. Treatment of the patients was evaluated. RESULTS: Thirty patients were diagnosed as vasovagal syncope, 7 patients as POTS, and 2 as inappropriate sinus tachycardia. POTS accompanied Raynaud's phenomenon in 1 patient, hypertrophic cardiomyopathy in 1 patient, and homocystinuria in another patient. Complaints of patients with vasovagal syncope improved with non-medical therapy. Medical treatment was administered to the patients with diagnosis of POTS and inappropriate sinus tachycardia. CONCLUSION: In patients with complaints of syncope, pre-syncope, dizziness, and palpitations without structural heart disease or non-rhythm problems, cardiovascular autonomic disorders, such as POTS and inappropriate sinus tachycardia should be kept in mind, as well as vasovagal syncope. FAU - Ugan Atik, Sezen AU - Ugan Atik S AD - Department of Pediatric Cardiology, Istanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey. sezenugan@hotmail.com. FAU - Dedeoğlu, Reyhan AU - Dedeoğlu R FAU - Koka, Aida AU - Koka A FAU - Öztunç, Funda AU - Öztunç F LA - tur PT - Journal Article TT - Çocuklarda postüral ortostatik taşikardi sendromu, uygunsuz sinüs taşikardisi ve vazovagal senkop tanı ve tedavisindeki deneyimlerimiz. PL - Turkey TA - Turk Kardiyol Dern Ars JT - Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir JID - 9426239 SB - IM CIN - Turk Kardiyol Dern Ars. 2017 Apr;45(3):214-216. doi: 10.5543/tkda.2017.77943. PMID: 28429687 MH - Child MH - Cohort Studies MH - Electrocardiography, Ambulatory MH - Humans MH - *Postural Orthostatic Tachycardia Syndrome/diagnosis/therapy MH - *Syncope, Vasovagal/diagnosis/therapy MH - *Tachycardia, Sinus/diagnosis/therapy MH - Tilt-Table Test EDAT- 2017/04/22 06:00 MHDA- 2017/09/12 06:00 CRDT- 2017/04/22 06:00 PHST- 2017/04/22 06:00 [entrez] PHST- 2017/04/22 06:00 [pubmed] PHST- 2017/09/12 06:00 [medline] AID - 10.5543/tkda.2017.36517 [doi] PST - ppublish SO - Turk Kardiyol Dern Ars. 2017 Apr;45(3):227-234. doi: 10.5543/tkda.2017.36517. PMID- 30459985 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2055-7159 (Electronic) IS - 2055-7159 (Linking) VI - 2 IP - 3 DP - 2016 TI - Tumoral calcinosis in the lumbar spine secondary to systemic sclerosis: a rare cause of radiculopathy in an adult with advanced disease. PG - 20150435 LID - 10.1259/bjrcr.20150435 [doi] LID - 20150435 AB - Calcinosis is frequently associated with systemic sclerosis (SSc) and can be located at various sites, although it is most commonly seen in the hands. When it presents around the synovial joints and is associated with a mass-like appearance, it is classically called tumoral calcinosis. Few cases of tumoral calcinosis have been reported in the paraspinal region. They are usually located in the cervical segment and rarely in the lumbar region. Occasionally, they have been associated with nerve root compression and intraspinal extension. We report the case of a 47-year-old female with advanced SSc who presented to our hospital's radiology department with chronic low back pain and right L5 radiculopathy due to tumoral calcinosis. An initial lumbar spine MRI showed multifocal, low signal, mass-like lesions involving the right paraspinal soft tissues. At the L5-S1 level, one lesion compressed the right L5 exiting nerve root. A CT scan of the lumbar spine performed later demonstrated the calcified nature of the lesions depicted by MRI and evidenced signs of pulmonary fibrosis at the base of the lungs. Further clinical work-up also showed that the patient had Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, dyspnoea, facial telangiectasias, generalized weakness and arthralgia. The diagnosis of a subtype of SSc, called limited cutaneous SSc, was made. Our case describes the CT and MRI findings of tumoral calcinosis in an unusual location secondary to limited cutaneous SSc. Knowledge of the imaging features of this uncommon manifestation of SSc could potentially increase its prospective diagnosis and hence improve patient management. FAU - Liberato, Afonso Celso Pedrotti AU - Liberato ACP AD - Neuroradiology, Beneficencia Portuguesa Hospital, Med Imagem, São Paulo, Brazil. FAU - Amaral, Lazaro Luiz Faria Do AU - Amaral LLFD AD - Neuroradiology, Beneficencia Portuguesa Hospital, Med Imagem, São Paulo, Brazil. FAU - Marussi, Victor Hugo Rocha AU - Marussi VHR AD - Neuroradiology, Beneficencia Portuguesa Hospital, Med Imagem, São Paulo, Brazil. LA - eng PT - Case Reports PT - Journal Article DEP - 20160728 PL - England TA - BJR Case Rep JT - BJR case reports JID - 101684132 PMC - PMC6243371 EDAT- 2016/07/28 00:00 MHDA- 2016/07/28 00:01 PMCR- 2016/07/28 CRDT- 2018/11/22 06:00 PHST- 2015/11/07 00:00 [received] PHST- 2016/02/24 00:00 [revised] PHST- 2016/03/10 00:00 [accepted] PHST- 2018/11/22 06:00 [entrez] PHST- 2016/07/28 00:00 [pubmed] PHST- 2016/07/28 00:01 [medline] PHST- 2016/07/28 00:00 [pmc-release] AID - 10.1259/bjrcr.20150435 [doi] PST - epublish SO - BJR Case Rep. 2016 Jul 28;2(3):20150435. doi: 10.1259/bjrcr.20150435. eCollection 2016. PMID- 23428662 OWN - NLM STAT- MEDLINE DCOM- 20130819 LR - 20161209 IS - 2213-0276 (Electronic) IS - 0755-4982 (Linking) VI - 42 IP - 6 Pt 1 DP - 2013 Jun TI - Outcome of anti-PL12 positive patients with antisynthetase syndrome. PG - e153-8 LID - S0755-4982(13)00020-1 [pii] LID - 10.1016/j.lpm.2012.12.003 [doi] AB - OBJECTIVES: The aim of the present study was to assess the outcome in anti-PL12 patients with antisynthetase syndrome (ASS). METHODS: The medical records of anti-PL12 (n=5) patients with ASS were retrospectively analyzed without prior selection. To exclude false-positive patients, we included patients who were successively tested positive for anti-PL12 antibody at least twice by immunodot and/or Western blot. RESULTS: Anti-PL12 patients experienced: myositis (n=2), Raynaud's phenomenon (n=2), mechanic's hands (n=1), joint impairment (n=4), digestive involvement (n=2), and interstitial lung disease (ILD) (n=4). The two patients with myositis exhibited deterioration of muscle manifestations despite therapy. As regards outcome of ILD, patients developed resolution (n=1), stabilization (n=1) or deterioration (n=2) of pulmonary status. One patient died of pyogenic pneumonia. CONCLUSION: Our series underscores that the presence of anti-PL12 antibody is associated with a particular phenotype of ASS characterized by: (1) less frequent although severe/steroid refractory myositis; (2) less common mechanic's hands and calcinosis cutis; (3) both frequent and severe ILD. Taken together, our findings suggest that PM/DM patients should routinely undergo the search for anti-PL12 antibody as this autoantibody appears to impact patients' prognosis. Furthermore, ILD patients with anti-PL12 antibody should routinely undergo clinical screening for underlying ASS. CI - Copyright © 2013 Elsevier Masson SAS. All rights reserved. FAU - Marie, Isabelle AU - Marie I AD - CHU Rouen, Institute for Biochemical Research, University of Rouen IFRMP, Department of Internal medicine, Inserm U 905, 76031 Rouen cedex, France. isabelle.marie@chu-rouen.fr FAU - Josse, Séverine AU - Josse S FAU - Decaux, Olivier AU - Decaux O FAU - Dominique, Stéphane AU - Dominique S FAU - Landron, Cédric AU - Landron C FAU - Roblot, Pascal AU - Roblot P FAU - Jouneau, Stéphane AU - Jouneau S FAU - Vittecoq, Olivier AU - Vittecoq O FAU - Jouen, Fabienne AU - Jouen F LA - eng PT - Journal Article DEP - 20130218 PL - France TA - Presse Med JT - Presse medicale (Paris, France : 1983) JID - 8302490 RN - 0 (Autoantibodies) RN - EC 6.1.1.7 (Alanine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Alanine-tRNA Ligase/*immunology MH - Autoantibodies/*blood MH - Female MH - Humans MH - Male MH - Middle Aged MH - Myositis/*blood/complications/diagnosis MH - Prognosis MH - Retrospective Studies EDAT- 2013/02/23 06:00 MHDA- 2013/08/21 06:00 CRDT- 2013/02/23 06:00 PHST- 2012/10/10 00:00 [received] PHST- 2012/12/15 00:00 [revised] PHST- 2012/12/20 00:00 [accepted] PHST- 2013/02/23 06:00 [entrez] PHST- 2013/02/23 06:00 [pubmed] PHST- 2013/08/21 06:00 [medline] AID - S0755-4982(13)00020-1 [pii] AID - 10.1016/j.lpm.2012.12.003 [doi] PST - ppublish SO - Presse Med. 2013 Jun;42(6 Pt 1):e153-8. doi: 10.1016/j.lpm.2012.12.003. Epub 2013 Feb 18. PMID- 18701013 OWN - NLM STAT- MEDLINE DCOM- 20090204 LR - 20211020 IS - 1478-7083 (Electronic) IS - 0035-8843 (Print) IS - 0035-8843 (Linking) VI - 90 IP - 7 DP - 2008 Oct TI - The acute blue finger: management and outcome. PG - 557-60 LID - 10.1308/003588408X318237 [doi] AB - INTRODUCTION: The objective was to assess the management, and short- and longer-term outcome of patients presenting with an acute blue finger. PATIENTS AND METHODS: This was a retrospective, case-note review and prospective follow-up by telephone and general practitioner enquiry. All patients who presented with sudden onset blue discolouration of a finger within the previous 72 h, with normal radial and ulnar pulses, were included. RESULTS: From 2000 to 2006, 22 patients, 15 female, 7 male, were reviewed. Median age was 56 years (range, 19-88 years). Median time from onset of blue finger was 6 days (range 1 day to 3 months). In most cases (17), no underlying cause was identified. Five patients had an underlying cause; two had symptoms compatible with Raynaud's phenomenon, one patient had signs (later confirmed on MRA) of arterial thoracic outlet syndrome and two had polycythaemia (haemoglobin > 17 g/dl). Otherwise, all laboratory investigations were normal. Upper limb duplex, echocardiogram and 24-h cardiac tapes were normal in all cases. Median follow-up was 19 months. Three patients had recurrent symptoms in the finger. No patient suffered tissue loss or loss of digit(s), and none had stroke or arterial embolisation. CONCLUSIONS: The acute blue finger is a benign condition not suggestive of arterial embolisation. Tissue or digit loss is not a threat and, in the longer term, there is no threat of embolisation to other vascular sites. FAU - Cowen, R AU - Cowen R AD - Department of Vascular Surgery, The John Radcliffe, Oxford, UK. FAU - Richards, T AU - Richards T FAU - Dharmadasa, A AU - Dharmadasa A FAU - Handa, A AU - Handa A FAU - Perkins, J M T AU - Perkins JM LA - eng PT - Journal Article DEP - 20080812 PL - England TA - Ann R Coll Surg Engl JT - Annals of the Royal College of Surgeons of England JID - 7506860 SB - IM CIN - Ann R Coll Surg Engl. 2009 May;91(4):354. doi: 10.1308/003588409X428423. PMID: 19416593 MH - Acute Disease MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Fingers/*blood supply MH - Follow-Up Studies MH - Humans MH - Ischemia/etiology/*therapy MH - Male MH - Middle Aged MH - Treatment Outcome MH - Young Adult PMC - PMC2728302 EDAT- 2008/08/15 09:00 MHDA- 2009/02/05 09:00 PMCR- 2009/10/01 CRDT- 2008/08/15 09:00 PHST- 2008/08/15 09:00 [pubmed] PHST- 2009/02/05 09:00 [medline] PHST- 2008/08/15 09:00 [entrez] PHST- 2009/10/01 00:00 [pmc-release] AID - 9716 [pii] AID - 10.1308/003588408X318237 [doi] PST - ppublish SO - Ann R Coll Surg Engl. 2008 Oct;90(7):557-60. doi: 10.1308/003588408X318237. Epub 2008 Aug 12. PMID- 42220959 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260601 LR - 20260601 IS - 2219-2808 (Print) IS - 2219-2808 (Electronic) IS - 2219-2808 (Linking) VI - 15 IP - 2 DP - 2026 Jun 9 TI - Use of nailfold capillaroscopy for evaluation of disease activity in juvenile dermatomyositis: Results of a two-center retrospective study. PG - 114270 LID - 10.5409/wjcp.v15.i2.114270 [doi] LID - 114270 AB - BACKGROUND: Juvenile dermatomyositis (JDM) is a rare, immune-mediated children disease that affects both skeletal muscles and skin. Inflammatory vasculopathy is a key part of the JDM pathogenesis. Nailfold capillaroscopy (NFC) is a non-invasive method for microvasculature assessment. Despite the pathogenic relevance of microangiopathy in JDM, the diagnostic utility of NFC in JDM remains insufficiently defined. AIM: To quantitatively assess capillaroscopic dynamics and their association with clinical and laboratory activity markers in JDM. METHODS: This retrospective double-center cohort study included 52 children with confirmed JDM, who were followed at the clinics of Sechenov University and Saint Petersburg State Pediatric Medical University. Patients were divided into active and inactive JDM groups based on the modified disease activity score. Demographic, clinical, laboratory, and instrumental data were analyzed. NFC evaluation was performed using standardized criteria developed initially for adult patients with systemic sclerosis and Raynaud's phenomenon. Statistical analysis was conducted using Windows v.13 (StatSoft). RESULTS: Active and inactive JDM groups were demographically comparable (predominantly female, median onset age 6 years). NFC revealed marked differences: Active patients showed higher rates of reduced capillary density (96.2% vs 60.9%, P = 0.003), giant capillaries (73.1% vs 13.0%, P < 0.001), perivascular edema (73.1% vs 17.4%, P < 0.001), and greater maximal apical loop width. Disease activity positively correlated with giant capillaries (r = 0.65), perivascular edema (r = 0.60), and reduced capillary density (r = 0.46). These findings confirm that specific NFC features are strongly associated with clinical disease activity, as measured by the disease activity score. CONCLUSION: Children with active JDM demonstrated more frequent NFC changes, indicating that NFC may play a potential role in monitoring disease activity. Further studies are needed to determine the diagnostic accuracy and prognostic relevance of this approach. CI - ©Author(s) 2026. FAU - Podzolkova, Vera AU - Podzolkova V AD - Department of Children's Diseases, N.F. Filatov Clinical Institute of Children's Health, Sechenovskiy University, Moscow 119991, Moskva, Russia. FAU - Avrusin, Ilia S AU - Avrusin IS AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Nikolaeva, Maria AU - Nikolaeva M AD - Department of Pediatric Rheumatology No. 1, Clinic of Children's Diseases, Sechenov's Center of Maternity and Childhood, Sechenovskiy University, Moscow 119991, Moskva, Russia. FAU - Afonina, Elena AU - Afonina E AD - Department of Pediatric Rheumatology No. 1, Clinic of Children's Diseases, Sechenov's Center of Maternity and Childhood, Sechenovskiy University, Moscow 119991, Moskva, Russia. FAU - Davtian, Siuzanna AU - Davtian S AD - Internal Diseases Faculty, N.V. Sklifosovskiy Institute of Clinical Medicine, Sechenovskiy University, Moscow 119991, Moskva, Russia. FAU - Nurseitova, Asel AU - Nurseitova A AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Kravtsova, Kira AU - Kravtsova K AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Malahova, Aleksandra AU - Malahova A AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Yakovlev, Alexandr A AU - Yakovlev AA AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Avrusin, Sergey L AU - Avrusin SL AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Kalashnikova, Olga V AU - Kalashnikova OV AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Chasnyk, Vyacheslav G AU - Chasnyk VG AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. FAU - Kostik, Mikhail M AU - Kostik MM AD - Hospital Pediatry, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia. kost-mikhail@yandex.ru. LA - eng PT - Journal Article DEP - 20260609 PL - United States TA - World J Clin Pediatr JT - World journal of clinical pediatrics JID - 101627548 PMC - PMC13217200 OTO - NOTNLM OT - Children OT - Disease activity OT - Inflammatory myopathy OT - Juvenile dermatomyositis OT - Nailfold capillaroscopy COIS- Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article. EDAT- 2026/06/01 08:04 MHDA- 2026/06/01 08:05 PMCR- 2026/06/09 CRDT- 2026/06/01 05:59 PHST- 2025/09/16 00:00 [received] PHST- 2025/11/06 00:00 [revised] PHST- 2026/01/14 00:00 [accepted] PHST- 2026/06/09 00:00 [pmc-release] PHST- 2026/06/01 08:05 [medline] PHST- 2026/06/01 08:04 [pubmed] PHST- 2026/06/01 05:59 [entrez] AID - 10.5409/wjcp.v15.i2.114270 [doi] PST - epublish SO - World J Clin Pediatr. 2026 Jun 9;15(2):114270. doi: 10.5409/wjcp.v15.i2.114270. eCollection 2026 Jun 9. PMID- 36460049 OWN - NLM STAT- MEDLINE DCOM- 20230222 LR - 20230222 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 32 IP - 2 DP - 2023 Feb TI - Pulmonary involvement: A potential independent factor for quality of life in systemic lupus erythematosus. PG - 198-206 LID - 10.1177/09612033221143934 [doi] AB - BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic multi-systemic autoimmune disease. SLE patients may experience a wide range of physical, psychological, and social perception of well-being influenced by the patient illness that are not always fully captured by descriptions of the disease's physiological consequences alone. Nowadays, patients with SLE have a better survival than decades ago, nevertheless still experience a low health related quality of life (HRQoL). Assessing disease activity in SLE is crucial to the physician as it forms the basis for treatment decisions, moreover careful evaluation for respiratory involvement should be routinely considered. More chronic lung disease related to SLE can have a significant negative effect on patient well-being and physical performance status and are detrimental to quality of life. OBJECTIVE: The aim of this study was to evaluate quality of life changes in SLE patients using Lupus QoL scale, assessing their correlation with different disease aspects particularly pulmonary manifestations and predictors for worse QoL. MATERIALS AND METHODS: Total of 60 SLE patients, who fulfilled the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria, were enrolled in this study. Disease activity was measured by systemic lupus erythematosus disease activity index (SLEDAI) and quality of life was assessed by Lupus QoL. Pulmonary evaluation included pulmonary function tests parameters (PFTs), mMRC dyspnea scale, HRCT score, and pulmonary damage index. RESULTS: Lupus QoL had a strong significant correlations with PFTs FEV1, FVC, and DLCO (r = 0.79, 0.78, 0.76, p < .001), respectively}, while Lupus QoL had strong negative correlations with both mMRC dyspnea scale and HRCT score (r = -0.96, -0.85, p < .001), respectively, and moderate negative correlation with neuropsychiatric lupus (NPSLE) (r = -0.61, p < .001). Weak negative correlations were found between Lupus QoL, photosensitivity, alopecia, Raynaud's and renal affection (r = -0.29, -0.30, -0.30, 0.38, p = .03, .02, .02, .002), respectively. NPSLE and pulmonary involvement were the most consistent predictors of low HRQoL [contributing 36% and 18% of the variance of Lupus QoL], respectively. CONCLUSION: Lupus QoL is negatively correlated with different SLE clinical parameters particularly pulmonary manifestations. Neuropsychiatric, pulmonary, renal affection, and SLEDAI are the best determinants for worse Lupus QoL. FAU - Osman, Haidy M AU - Osman HM AUID- ORCID: 0000-0003-4982-6797 AD - Department of Rheumatology and Rehabilitation, 68877Minia University, Minya, Egypt. FAU - Abdel-Nasser, Ahmed M AU - Abdel-Nasser AM AD - Department of Rheumatology and Rehabilitation, 68877Minia University, Minya, Egypt. FAU - Kasem, Ahmed H AU - Kasem AH AD - Department of Chest Diseases, 68877Minia University, Minya, Egypt. FAU - Elameen, Nadia F AU - Elameen NF AD - Department of Radiodiagnosis, 68877Minia University, Minya, Egypt. FAU - Omar, Gihan M AU - Omar GM AD - Department of Rheumatology and Rehabilitation, 68877Minia University, Minya, Egypt. LA - eng PT - Journal Article DEP - 20221202 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic MH - Quality of Life MH - Severity of Illness Index MH - *Lung Diseases MH - Dyspnea OTO - NOTNLM OT - SLEDAI OT - Systemic lupus erythematosus OT - lupus QoL OT - neuropsychiatric lupus OT - pulmonary affection EDAT- 2022/12/03 06:00 MHDA- 2023/02/22 06:00 CRDT- 2022/12/02 19:13 PHST- 2022/12/03 06:00 [pubmed] PHST- 2023/02/22 06:00 [medline] PHST- 2022/12/02 19:13 [entrez] AID - 10.1177/09612033221143934 [doi] PST - ppublish SO - Lupus. 2023 Feb;32(2):198-206. doi: 10.1177/09612033221143934. Epub 2022 Dec 2. PMID- 35382370 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 36 IP - 4 DP - 2021 Dec TI - Lupus-related vasculitis in a cohort of systemic lupus erythematosus patients. PG - 595-692 LID - 10.46497/ArchRheumatol.2021.8804 [doi] AB - OBJECTIVES: This study aims to examine the frequency and clinical association of lupus-related vasculitis in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We retrospectively analyzed medical records of a total of 565 SLE patients (42 males, 523 females; mean age: 32.7±9.5 years; range, 13 to 63 years) between January 2017 and February 2020. Demographic, clinical data, and laboratory data and treatment modalities applied were recorded. Lupus-related vasculitis and its different types were documented, and the patients with vasculitis were compared with those without vasculitis. RESULTS: The mean disease duration was 8.9±6.3 years. Vasculitis associated with lupus was found in 191 (33.45%) patients. Cutaneous vasculitis was found in 59.2%, visceral vasculitis in 34.0%, and both in 6.8% of total vasculitis patients. The patients with vasculitis had a longer disease duration (p=0.01), were more likely to have juvenile onset (p=0.002), livedo reticularis (p<0.001), Raynaud's phenomenon (RP) (p<0.001), digital gangrene (p<0.001), thrombosis (p=0.003), and cranial neuropathy (p=0.004). The patients with vasculitis showed a higher prevalence of hypercholesterolemia (p=0.045), diabetes mellitus (p=0.026), higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) at disease onset (p<0.001), and Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (p=0.003) scores. They had more prevalent hematological manifestations (p<0.001), hypocomplementemia (p=0.007), received a higher cumulative dose of intravenous methylprednisolone (p<0.001), and had also more frequent cyclophosphamide (p=0.016) and azathioprine intake (p<0.001). In the logistic regression analysis, SLE vasculitis was independently associated with juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher scores of SLEDAI at disease onset (p<0.05). CONCLUSION: Juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher SLEDAI scores at disease onset may be associated with the development of vasculitis in SLE patients. CI - Copyright © 2021, Turkish League Against Rheumatism. FAU - Gamal, Sherif M AU - Gamal SM AUID- ORCID: 0000-0002-2663-4111 AD - Rheumatology, Cairo University, Faculty of Medicine, Cairo, Egypt. FAU - Mohamed, Sally S AU - Mohamed SS AUID- ORCID: 0000-0003-1868-9443 AD - Rheumatology, Cairo University, Faculty of Medicine, Cairo, Egypt. FAU - Tantawy, Marwa AU - Tantawy M AUID- ORCID: 0000-0001-5057-0202 AD - Rheumatology, Beni Suif University, Beni Suif, Egypt. FAU - Siam, Ibrahem AU - Siam I AUID- ORCID: 0000-0003-4176-2389 AD - Department of Internal Medicine, National Research Centre, Cairo, Egypt. FAU - Soliman, Ahmed AU - Soliman A AUID- ORCID: 0000-0001-6835-3548 AD - Department of Dermatology, National Research Centre, Cairo, Egypt. FAU - Niazy, Marwa H AU - Niazy MH AUID- ORCID: 0000-0003-1107-3240 AD - Rheumatology, Cairo University, Faculty of Medicine, Cairo, Egypt. LA - eng PT - Journal Article DEP - 20211012 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC8957770 OTO - NOTNLM OT - Clinical association OT - frequency OT - lupus vasculitis COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2022/04/07 06:00 MHDA- 2022/04/07 06:01 PMCR- 2021/10/12 CRDT- 2022/04/06 05:14 PHST- 2021/02/22 00:00 [received] PHST- 2021/04/19 00:00 [accepted] PHST- 2022/04/06 05:14 [entrez] PHST- 2022/04/07 06:00 [pubmed] PHST- 2022/04/07 06:01 [medline] PHST- 2021/10/12 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2021.8804 [doi] PST - epublish SO - Arch Rheumatol. 2021 Oct 12;36(4):595-692. doi: 10.46497/ArchRheumatol.2021.8804. eCollection 2021 Dec. PMID- 19219480 OWN - NLM STAT- MEDLINE DCOM- 20090825 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 28 IP - 5 DP - 2009 May TI - Nailfold capillaroscopy in Behçet's disease, analysis of 128 patients. PG - 603-5 LID - 10.1007/s10067-009-1106-2 [doi] AB - The aims of this study were to find the characteristics and prevalence of nailfold capillary changes in a large series of patients with Behçet's disease (BD) and to analyze their possible relation to other clinical characteristics of the disease. We performed nailfold capillaroscopy in 128 randomly selected patients fulfilling the international classification criteria for BD. Capillaroscopy was done in eight fingers with a x3.2 microscopy. All patients were questioned for history of Raynaud's phenomenon, ischemic ulcers, smoking, and hypertension. A computerized form including demographic, clinical, and para-clinical features was used to collect data. Univariate and multivariate logistic regressions were used to analyze the relation between capillaroscopic findings and disease characteristics. Odds ratio and a confidence interval at 95% (CI) were calculated for each item. The mean age of the patients was 37 +/- 10 years, and the male to female ratio was 1.56:1. Capillaroscopy was abnormal in 51 patients (40%, CI 8.5). Enlarged capillaries were seen in 33 patients (26%, CI 7.6), hemorrhages in 21 (16%, CI 6.4), and capillary loss only in one patient. In univariate logistic regression analysis, the presence of enlarged capillaries was associated with lower age at disease onset (OR = 0.9, CI 0.9-1; p = 0.04), hypertension (OR = 4.2, CI 1.5-11.4; p = 0.006), superficial phlebitis (OR = 5.5, CI 1.2-24.4; p = 0.03), and negative pathergy test (OR = 0.4, CI 0.2-0.9; p = 0.04). The presence of hemorrhages tended to be associated with articular symptoms (p = 0.05). Multivariate analysis also confirmed the association of enlarged capillaries with lower age at disease onset (p = 0.01), hypertension (p = 0.001), and superficial phlebitis (p = 0.03). Nailfold abnormalities, mainly enlarged capillaries, are frequent in patients with BD. Our results suggest that these abnormalities may be related to other vascular features of the disease such as superficial phlebitis, but it does not seem to confer special risk for any other specific clinical symptom of the disease. FAU - Movasat, Atusa AU - Movasat A AD - Department of Rheumatology, Hospital 12 de Octubre, Madrid, Spain. FAU - Shahram, Farhad AU - Shahram F FAU - Carreira, Patricia E AU - Carreira PE FAU - Nadji, Abdolhadi AU - Nadji A FAU - Akhlaghi, Maassoomeh AU - Akhlaghi M FAU - Naderi, Nassim AU - Naderi N FAU - Davatchi, Fereydoun AU - Davatchi F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090214 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Behcet Syndrome/*diagnosis MH - Capillaries/*pathology MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Multivariate Analysis MH - Nails/*blood supply MH - Odds Ratio MH - Phlebitis/*diagnosis/pathology MH - Prospective Studies MH - Risk EDAT- 2009/02/17 09:00 MHDA- 2009/08/26 09:00 CRDT- 2009/02/17 09:00 PHST- 2008/12/15 00:00 [received] PHST- 2009/01/27 00:00 [accepted] PHST- 2009/01/06 00:00 [revised] PHST- 2009/02/17 09:00 [entrez] PHST- 2009/02/17 09:00 [pubmed] PHST- 2009/08/26 09:00 [medline] AID - 10.1007/s10067-009-1106-2 [doi] PST - ppublish SO - Clin Rheumatol. 2009 May;28(5):603-5. doi: 10.1007/s10067-009-1106-2. Epub 2009 Feb 14. PMID- 27132536 OWN - NLM STAT- MEDLINE DCOM- 20170901 LR - 20180210 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 46 IP - 1 DP - 2016 Aug TI - Impaired quality of life in systemic sclerosis and patient perception of the disease: A large international survey. PG - 115-23 LID - S0049-0172(16)00061-5 [pii] LID - 10.1016/j.semarthrit.2016.02.005 [doi] AB - OBJECTIVES: The purpose of this study was to assess health-related quality of life (HRQoL) and disease perception in a large, international group of patients with systemic sclerosis (SSc). METHODS: We placed a standardized questionnaire on a website for patient access. Socio-demographic information, disease characteristics, and self-assessment questionnaires-the Short Form 36 (SF-36) and the Revised Illness Perception Questionnaire (IPQ-R)-were collected. RESULTS: A total of 1902 patients from 60 countries were included. HRQoL appeared to be impaired in SSc, particularly for physical health (PCS, mean ± SD = 43.4 ± 23.4). SSc patients also had strong perceptions about the chronic nature and negative consequence of the disease, and experienced negative emotions due to SSc. Patients with diffuse cutaneous SSc had a poorer HRQoL than those with limited cutaneous SSc, for both physical (PCS, mean ± SD = 46.6 ± 23.7 vs. 39.8 ± 22.3; p < 0.0001) and mental components (MCS, mean ± SD = 53.8 ± 23.0 vs. 50.3 ± 23.2; p = 0.003). Late-stage SSc patients were more likely to perceive their disease chronic (p < 0.0001), less controllable (p = 0.03) and with more consequences (p = 0.008), but they had a better understanding of their disease and experienced fewer negative emotions. Raynaud's phenomenon and gastrointestinal complications were the organ involvements with the greatest impact on QoL, they were the two variables associated with the most negative perception of illness severity. CONCLUSION: This study, performed on the largest group ever set up for this purpose, confirms the major impact on QoL and the negative perceptions of their disease expressed by SSc patients. However, the perception of this illness tended to improve with disease duration, suggesting that patients find effective coping strategies. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Frantz, Camelia AU - Frantz C AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. FAU - Avouac, Jérôme AU - Avouac J AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. FAU - Distler, Oliver AU - Distler O AD - Division of Rheumatology, University Hospital, Zurich, Switzerland; European Scleroderma Trials And Research (EUSTAR) Board, Paris, France. FAU - Amrouche, Fazia AU - Amrouche F AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. FAU - Godard, Dominique AU - Godard D AD - Association des Sclérodermie de France, Auxerre, France. FAU - Kennedy, Ann Tyrrell AU - Kennedy AT AD - Federation of European Scleroderma Associations (FESCA), Tournai, Belgium. FAU - Connolly, Kerry AU - Connolly K AD - Scleroderma Foundation, Danvers, MA. FAU - Varga, John AU - Varga J AD - Scleroderma Foundation, Danvers, MA; Rheumatology, Northwestern University, Chicago, IL. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Medicine & Rheumatology, University of Florence, Florence, Italy; World Scleroderma Foundation, BASEL, Switzerland. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A Department, Cochin Hospital, Paris Descartes University, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France; European Scleroderma Trials And Research (EUSTAR) Board, Paris, France. Electronic address: yannick.allanore@cch.aphp.fr. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20160226 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - *Disability Evaluation MH - Female MH - Health Status MH - Health Surveys MH - Humans MH - Male MH - Middle Aged MH - *Quality of Life MH - Scleroderma, Systemic/diagnosis/*psychology MH - Severity of Illness Index OTO - NOTNLM OT - Illness perception OT - Quality of life OT - Systemic sclerosis EDAT- 2016/05/03 06:00 MHDA- 2017/09/02 06:00 CRDT- 2016/05/03 06:00 PHST- 2015/09/13 00:00 [received] PHST- 2015/12/22 00:00 [revised] PHST- 2016/02/20 00:00 [accepted] PHST- 2016/05/03 06:00 [entrez] PHST- 2016/05/03 06:00 [pubmed] PHST- 2017/09/02 06:00 [medline] AID - S0049-0172(16)00061-5 [pii] AID - 10.1016/j.semarthrit.2016.02.005 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Aug;46(1):115-23. doi: 10.1016/j.semarthrit.2016.02.005. Epub 2016 Feb 26. PMID- 39544895 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260129 LR - 20260129 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 2 DP - 2025 Jun TI - Efficacy and safety of digital nerve block for pain management during sharp debridement of digital ulcers in systemic sclerosis: A prospective observational study. PG - 156-162 LID - 10.1177/23971983241285206 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and vascular abnormalities, often leading to the development of digital ulcers (DUs). DUs are painful and debilitating, significantly impacting patients' quality of life. Effective pain management during debridement is crucial, yet there is no consensus on the optimal approach. This study evaluates the efficacy and safety of digital nerve block (DNB) using lidocaine and mepivacaine for pain control during sharp debridement of DUs in SSc patients. METHODS: This prospective observational study was conducted from September 2023 to May 2024 at the Rheumatology Operating Unit, University of Verona. Patients were randomized to receive either 1 mL of 2% lidocaine or 2% mepivacaine for DNB. Pain levels were assessed using a categorical grading scale during the injection and debridement. The outcomes were pain control and performance of lidocaine versus mepivacaine. RESULTS: The cohort developed 46 ulcers. The pain was abolished in almost all patients. Lidocaine achieved faster anaesthesia (127.92 ± 34.32 s) compared with mepivacaine (252.65 ± 49.89 s, p < 0.001). Mepivacaine resulted in less pain during injection (p = 0.006). No significant difference in pain levels during debridement was observed between the two anaesthetics. Three mild adverse effects (Raynaud's phenomenon) were reported. All procedures were completed successfully, and 35 ulcers healed with a mean time of 6.1 ± 7.77 weeks. CONCLUSIONS: DNB with mepivacaine provides effective pain control during DU debridement in SSc patients, with lower injection site pain and comparable efficacy to lidocaine. The procedure is safe, well-tolerated and facilitates successful ulcer healing. Further studies with larger cohorts are warranted to confirm these findings. CI - © The Author(s) 2024. FAU - Bixio, Riccardo AU - Bixio R AUID- ORCID: 0000-0001-9335-3371 AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Mastropaolo, Francesca AU - Mastropaolo F AUID- ORCID: 0000-0002-3225-2152 AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Nava, Francesca AU - Nava F AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Veliaj, Olta AU - Veliaj O AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Fracassi, Elena AU - Fracassi E AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Viapiana, Ombretta AU - Viapiana O AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Rossini, Maurizio AU - Rossini M AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. FAU - Idolazzi, Luca AU - Idolazzi L AUID- ORCID: 0000-0002-7254-4686 AD - Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy. LA - eng PT - Journal Article DEP - 20241009 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC11559528 OTO - NOTNLM OT - Systemic sclerosis OT - anaesthetic OT - debridement OT - digital nerve block OT - digital ulcer COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2024/11/15 11:00 MHDA- 2024/11/15 11:01 PMCR- 2025/10/09 CRDT- 2024/11/15 04:34 PHST- 2024/07/09 00:00 [received] PHST- 2024/09/04 00:00 [accepted] PHST- 2024/11/15 11:01 [medline] PHST- 2024/11/15 11:00 [pubmed] PHST- 2024/11/15 04:34 [entrez] PHST- 2025/10/09 00:00 [pmc-release] AID - 10.1177_23971983241285206 [pii] AID - 10.1177/23971983241285206 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2024 Oct 9;10(2):156-162. doi: 10.1177/23971983241285206. eCollection 2025 Jun. PMID- 36873897 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230307 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 10 DP - 2023 TI - Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy. PG - 1057643 LID - 10.3389/fmed.2023.1057643 [doi] LID - 1057643 AB - OBJECTIVES: To assess performance of interstitial pneumonia (IP) with autoimmune features (IPAF) criteria in clinical practice and describe the utility of additional workup in identifying patients with underlying connective tissue diseases (CTD). METHODS: We set a retrospective study of our patients with autoimmune IP, who were allocated to CTD-IP, IPAF or undifferentiated autoimmune IP (uAIP) subgroups according to the updated classification criteria. Presence of the process-related variables comprising IPAF defining domains was scrutinized in all patients, and, when available, the results of nailfold videocapillaroscopy (NVC) were recorded. RESULTS: Thirty nine out of 118 patients, accounting for 71% of former undifferentiated cases, fulfilled IPAF criteria. Arthritis and Raynaud's phenomenon were prevalent in this subgroup. While systemic sclerosis-specific autoantibodies were restricted to CTD-IP patients, anti-tRNA synthetase antibodies were also present in IPAF. In contrast, rheumatoid factor, anti-Ro antibodies and ANA nucleolar patterns could be found in all subgroups. Usual interstitial pneumonia (UIP) / possible UIP were the most frequently observed radiographic patterns Therefore, the presence of thoracic multicompartimental findings as also performance of open lung biopsies were useful in characterizing as IPAF those UIP cases lacking a clinical domain. Interestingly, we could observe NVC abnormalities in 54% of IPAF and 36% of uAIP tested patients, even though many of them did not report Raynaud's phenomenon. CONCLUSION: Besides application of IPAF criteria, distribution of IPAF defining variables along with NVC exams help identify more homogeneous phenotypic subgroups of autoimmune IP of potential relevance beyond clinical diagnosis. CI - Copyright © 2023 Romero-Bueno, Rodríguez-Nieto, Palacios Miras, Martínez Estupiñán, Martínez-Becerra, Vegas Sánchez, Cedeño Díaz, Sánchez-Pernaute and The NEREA Autoimmune ILD Study Group. FAU - Romero-Bueno, Fredeswinda Isabel AU - Romero-Bueno FI AD - Rheumatology Department, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Rodríguez-Nieto, Maria Jesús AU - Rodríguez-Nieto MJ AD - Department of Pulmonology, IIS-HU Fundación Jiménez Díaz, Autonoma University and CIBERES, Madrid, Spain. FAU - Palacios Miras, Carmelo AU - Palacios Miras C AD - Department of Imaging, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Martínez Estupiñán, Lina AU - Martínez Estupiñán L AD - Rheumatology Department, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Martínez-Becerra, Maria José AU - Martínez-Becerra MJ AD - Department of Immunology, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Vegas Sánchez, Maria Carmen AU - Vegas Sánchez MC AD - Department of Immunology, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Cedeño Díaz, Oderay Mabel AU - Cedeño Díaz OM AD - Department of Pathology, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. FAU - Sánchez-Pernaute, Olga AU - Sánchez-Pernaute O AD - Rheumatology Department, IIS-HU Fundación Jiménez Díaz, Autonoma University, Madrid, Spain. CN - NEREA Autoimmune ILD Study Group LA - eng PT - Journal Article DEP - 20230215 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC9975591 OTO - NOTNLM OT - anti synthetase syndrome OT - connective tissue diseases (CTD) OT - interstitial pneumonia (iP) OT - interstitial pneumonia with autoimmune features (IPAF) OT - nailfold videocapillaroscopy COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/03/07 06:00 MHDA- 2023/03/07 06:01 PMCR- 2023/02/15 CRDT- 2023/03/06 03:47 PHST- 2022/09/29 00:00 [received] PHST- 2023/01/24 00:00 [accepted] PHST- 2023/03/06 03:47 [entrez] PHST- 2023/03/07 06:00 [pubmed] PHST- 2023/03/07 06:01 [medline] PHST- 2023/02/15 00:00 [pmc-release] AID - 10.3389/fmed.2023.1057643 [doi] PST - epublish SO - Front Med (Lausanne). 2023 Feb 15;10:1057643. doi: 10.3389/fmed.2023.1057643. eCollection 2023. PMID- 19479119 OWN - NLM STAT- MEDLINE DCOM- 20090901 LR - 20220321 IS - 0001-5555 (Print) IS - 0001-5555 (Linking) VI - 89 IP - 3 DP - 2009 TI - Prostacyclin analogue iloprost influences endothelial cell-associated soluble adhesion molecules and growth factors in patients with systemic sclerosis: a time course study of serum concentrations. PG - 245-9 LID - 10.2340/00015555-0632 [doi] AB - Systemic sclerosis is a connective tissue disorder with unclear aetiology and pathogenesis. However, there is evidence that microvascular changes belong to the early symptoms of the disease. These are associated with increased serum levels of markers of endothelium activation, such as adhesion molecules and growth factors. The stable prostacyclin analogue iloprost is licensed for vascular symptoms (Raynaud's phenomenon) and was recently shown to exert short-term effects on these markers. In this study, serum samples (n = 13) from patients with systemic sclerosis were examined for serum levels of vascular cell adhesion molecule-1 (VCAM-1), intercellular cell adhesion molecule-1, E-selectin, endothelin-1 and vascular endothelial growth factor over 6 months after iloprost infusions in order to detect possible long-term effects. Iloprost significantly reduced initially elevated levels of these markers, partly until the end of the observation period (E-selectin, VCAM-1, endothelin-1). These effects provide serological evidence for the benefits of iloprost infusions that are seen clinically in patients with systemic sclerosis. FAU - Rehberger, Philipp AU - Rehberger P AD - Department of Dermatology, Technical University of Dresden, Fetscherstr. 74. Philipp.Rehberger@uniklinikum-dresden.de FAU - Beckheinrich-Mrowka, Petra AU - Beckheinrich-Mrowka P FAU - Haustein, Uwe-Frithjof AU - Haustein UF FAU - Sticherling, Michael AU - Sticherling M LA - eng PT - Journal Article PL - Sweden TA - Acta Derm Venereol JT - Acta dermato-venereologica JID - 0370310 RN - 0 (Biomarkers) RN - 0 (E-Selectin) RN - 0 (Endothelin-1) RN - 0 (Vascular Cell Adhesion Molecule-1) RN - 0 (Vascular Endothelial Growth Factor A) RN - 0 (Vasodilator Agents) RN - 126547-89-5 (Intercellular Adhesion Molecule-1) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Biomarkers/*blood MH - E-Selectin/blood MH - Endothelin-1/blood MH - Female MH - Humans MH - Iloprost/administration & dosage/*pharmacology MH - Infusions, Intravenous MH - Intercellular Adhesion Molecule-1/blood MH - Middle Aged MH - Scleroderma, Systemic/*blood/*drug therapy MH - Statistics, Nonparametric MH - Time Factors MH - Vascular Cell Adhesion Molecule-1/blood MH - Vascular Endothelial Growth Factor A/blood MH - Vasodilator Agents/administration & dosage/*pharmacology EDAT- 2009/05/30 09:00 MHDA- 2009/09/02 06:00 CRDT- 2009/05/30 09:00 PHST- 2009/05/30 09:00 [entrez] PHST- 2009/05/30 09:00 [pubmed] PHST- 2009/09/02 06:00 [medline] AID - 10.2340/00015555-0632 [doi] PST - ppublish SO - Acta Derm Venereol. 2009;89(3):245-9. doi: 10.2340/00015555-0632. PMID- 35463901 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220429 IS - 2296-2360 (Print) IS - 2296-2360 (Electronic) IS - 2296-2360 (Linking) VI - 10 DP - 2022 TI - Case Report, Practices Survey and Literature Review of an Under-Recognized Pediatric Vascular Disorder: The BASCULE Syndrome. PG - 849914 LID - 10.3389/fped.2022.849914 [doi] LID - 849914 AB - INTRODUCTION: Bier anemic spots, cyanosis, and urticaria-like eruption (BASCULE) syndrome is an underreported pediatric vascular disorder from the group of acrosyndromes. In children, these include paroxysmal acrosyndromes (Raynaud's phenomenon and chilblain-like lesions), permanent acrosyndromes (acrocyanosis), and transient acrosyndromes, in which their pathogeneses are associated with virus infections, Epstein-Barr virus, and, more recently, SARS-CoV-2, respectively. METHODS: We reported a case of BASCULE syndrome associated with postural orthostatic tachycardia syndrome (POTS) and provided a narrative review of case reports describing the BASCULE syndrome in children. Moreover, we presented the results of a prospective practice survey that we performed in the French medical community. RESULTS: A 14-years-old boy reported pruritic erythrocyanic lesions on the lower limbs, which occurred whenever he was in a standing position and fully resolved when he laid down. He reported asthenia and cramps. He presented a typical BASCULE syndrome associated with POTS confirmed by a tilt-test. Physical and vascular examinations were within the normal range. We identified 12 case reports, describing 21 pediatric cases since 2016. Most patients were adolescents between 12 and 19 years of age or were newborns. Furthermore, 20% of cases in the literature have presented POTS or orthostatic intolerance. Our survey among 95 French physicians confirmed that BASCULE syndrome is an underdiagnosed and under recognized disease in the general pediatric practice, at least in France. Among these physicians, 65% had already encountered patients with similar symptoms, but only 30% declared that they had knowledge of the BASCULE syndrome. CONCLUSION: The under-recognition of the clinical manifestations leads the patients to consult emergency rooms, with multiple unnecessary investigations performed. Therefore, we suggest that the diagnosis of BASCULE syndrome is based on clinical observations, without the need for laboratory tests, to avoid unnecessary health costs. We suggest physicians to perform a tilt-test when POTS is suspected. CI - Copyright © 2022 Baurens, Briand, Giovannini-Chami, De Guillebon De Resnes, Hubiche, Chiaverini, Giordana, Leftheriotis and Bernardor. FAU - Baurens, Natacha AU - Baurens N AD - CHU Lenval, Hôpitaux Pédiatriques de Nice, Nice, France. FAU - Briand, Clémence AU - Briand C AD - Unité de Dermatologie, Hôpital Archet-2, CHU de Nice, Nice, France. FAU - Giovannini-Chami, Lisa AU - Giovannini-Chami L AD - CHU Lenval, Hôpitaux Pédiatriques de Nice, Nice, France. AD - Université Côte d'Azur, Faculté de Médecine, Nice, France. FAU - De Guillebon De Resnes, Jean-Marie AU - De Guillebon De Resnes JM AD - Unité de Néphrologie et de Rhumatologie Pédiatrique, Hôpital L'Archet, CHU de Nice, Nice, France. FAU - Hubiche, Thomas AU - Hubiche T AD - Unité de Dermatologie, Hôpital Archet-2, CHU de Nice, Nice, France. FAU - Chiaverini, Christine AU - Chiaverini C AD - Unité de Dermatologie, Hôpital Archet-2, CHU de Nice, Nice, France. FAU - Giordana, Pascal AU - Giordana P AD - Unité de Médecine Vasculaire, Université Côte d'Azur, CHU Nice, Nice, France. FAU - Leftheriotis, Georges AU - Leftheriotis G AD - Unité de Médecine et d'Explorations Vasculaires, CHU Pasteur et CHU Pédiatrique Lenval, Nice, France. FAU - Bernardor, Julie AU - Bernardor J AD - CHU Lenval, Hôpitaux Pédiatriques de Nice, Nice, France. AD - Université Côte d'Azur, Faculté de Médecine, Nice, France. AD - Unité de Néphrologie et de Rhumatologie Pédiatrique, Hôpital L'Archet, CHU de Nice, Nice, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20220407 PL - Switzerland TA - Front Pediatr JT - Frontiers in pediatrics JID - 101615492 PMC - PMC9021422 OTO - NOTNLM OT - BASCULE syndrome OT - POTS OT - acrosyndrome OT - children OT - orthostatic intolerance COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/04/26 06:00 MHDA- 2022/04/26 06:01 PMCR- 2022/04/07 CRDT- 2022/04/25 05:25 PHST- 2022/01/06 00:00 [received] PHST- 2022/03/07 00:00 [accepted] PHST- 2022/04/25 05:25 [entrez] PHST- 2022/04/26 06:00 [pubmed] PHST- 2022/04/26 06:01 [medline] PHST- 2022/04/07 00:00 [pmc-release] AID - 10.3389/fped.2022.849914 [doi] PST - epublish SO - Front Pediatr. 2022 Apr 7;10:849914. doi: 10.3389/fped.2022.849914. eCollection 2022. PMID- 28203114 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1179-271X (Print) IS - 1179-271X (Electronic) IS - 1179-271X (Linking) VI - 8 DP - 2017 TI - Longitudinal analysis of quality of life in patients with undifferentiated connective tissue diseases. PG - 7-13 LID - 10.2147/PROM.S117767 [doi] AB - INTRODUCTION/OBJECTIVES: To prospectively assess the quality of life (QoL) of patients affected by undifferentiated connective tissue diseases (UCTDs) and to identify factors associated with changes over time. PATIENTS AND METHODS: A total of 46 consecutive UCTD patients completed the Short-Form 36 (SF-36) questionnaire at presentation and then yearly. At each 6-month visit, all patients underwent a detailed history taking and a laboratory and physical assessment, in order to follow the evolution of the disease over time and to assess the the co-existence of fibromyalgia. RESULTS: At presentation, scores lower than the average of the general population were detected in 34 (74%) and 41 (89%) patients in the physical and mental domains, respectively. No difference between patients with and without Raynaud's phenomenon was detected. Fibromyalgia was the only independent variable associated with an impaired physical component summary score (p = 0.0009). No patient feature was found to be associated with the basal mental component summary score. During 24 months of follow-up, a significant improvement (ie, a change ≥5 from baseline) in physical component summary and mental component summary scores was observed in 14 (33.3%) and 20 (43.4%) patients, respectively. Patients who significantly improved in the physical domain more frequently had a history of glucocorticoids intake (p<0.001), while those who improved in the mental component more frequently had a history of either glucocorticoids (p = 0.043) or immunosuppressors (p = 0.037) intake during follow-up. CONCLUSION: UCTD patients perceive a worse QoL, regardless of Raynaud's phenomenon Fibromyalgia is one of the major contributors of physical QoL, whereas no factor influencing mental component has been identified. An improvement in QoL can be observed in less than half of patients over a 2-year follow-up. Larger studies are needed to identify factors influencing QoL and to define the role of pharmacological treatments. FAU - Iudici, Michele AU - Iudici M AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. FAU - Irace, Rosaria AU - Irace R AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. FAU - Riccardi, Antonella AU - Riccardi A AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. FAU - Cuomo, Giovanna AU - Cuomo G AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. FAU - Vettori, Serena AU - Vettori S AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. FAU - Valentini, Gabriele AU - Valentini G AD - Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy. LA - eng PT - Journal Article DEP - 20170202 PL - New Zealand TA - Patient Relat Outcome Meas JT - Patient related outcome measures JID - 101551170 PMC - PMC5295807 OTO - NOTNLM OT - fibromyalgia OT - glucocorticoids OT - quality of life OT - undifferentiated connective tissue diseases COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2017/02/17 06:00 MHDA- 2017/02/17 06:01 PMCR- 2017/02/02 CRDT- 2017/02/17 06:00 PHST- 2017/02/17 06:00 [entrez] PHST- 2017/02/17 06:00 [pubmed] PHST- 2017/02/17 06:01 [medline] PHST- 2017/02/02 00:00 [pmc-release] AID - prom-8-007 [pii] AID - 10.2147/PROM.S117767 [doi] PST - epublish SO - Patient Relat Outcome Meas. 2017 Feb 2;8:7-13. doi: 10.2147/PROM.S117767. eCollection 2017. PMID- 41088497 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251015 LR - 20251017 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 9 IP - 1 DP - 2025 Oct 14 TI - The impact of familial autoimmunity and familial lupus on the clinical presentations and disease outcomes of SLE patients in the United Arab Emirates. PG - 118 LID - 10.1186/s41927-025-00574-z [doi] LID - 118 AB - OBJECTIVE: The presentation and outcomes of Systemic lupus erythematosus (SLE) are influenced by ethnicity and genetic background. The United Arab Emirates (UAE) is one of the leading countries of SLE per recent reports. In this study, we evaluated the effect of positive family history (FHx) of SLE and autoimmunity on clinical presentations and disease outcomes. METHODS: A retrospective observational study of patients seen between 2011 till 2023 was conducted. Included patients were those fulfilling the 2019 EULAR/ACR classification criteria. Comparative analyses were conducted between those with familial history of autoimmunity and SLE and those without. RESULTS: Out of 279 SLE patients, a total of 241 patients fulfilled the 2019 EULAR/ACR classification criteria and were included in the study. There was positive FHx of autoimmunity in 27% of the study population, and positive FHx of SLE (in first-degree relatives, "familial SLE") in 14.5% of the study population. Comparisons between positive and negative FHx of autoimmunity/SLE showed younger age at diagnosis in those with positive FHx of autoimmunity (p-value = < 0.001) and higher frequency of Raynaud's phenomonen (p-value = 0.022). Patients with familial SLE were also younger at diagnosis (p- value = 0.004) and had more mucocutaneous features (p-value = 0.042). CONCLUSION: The percentage of familial SLE in our UAE study population is 14.5% which is higher than reported in non-Arab study populations. In our study population, patients with familial SLE and familial autoimmunity tend to present earlier, while patients with familial SLE tend to have more mucocutaneous features than those without familial SLE. CLINICAL TRIAL NUMBER: Not applicable. CI - © 2025. The Author(s). FAU - Al Dhaheri, Afra AU - Al Dhaheri A AD - Rheumatology Department, Tawam Hospital, Al Ain, UAE. FAU - Alblooshi, Hiba AU - Alblooshi H AD - Department of Genetics and Genomics, College of Medicine & Health Sciences, UAE University, Al Ain, UAE. FAU - Bharathan, Anjali AU - Bharathan A AD - Department of Genetics and Genomics, College of Medicine & Health Sciences, UAE University, Al Ain, UAE. FAU - Alneyadi, Asma AU - Alneyadi A AD - Department of Academic Affairs, Tawam Hospital, Al Ain, UAE. FAU - Al Ali, Maryam AU - Al Ali M AD - Department of Academic Affairs, Tawam Hospital, Al Ain, UAE. FAU - Alzaabi, Amna AU - Alzaabi A AD - Department of Academic Affairs, Tawam Hospital, Al Ain, UAE. FAU - Trad, Jalal AU - Trad J AD - Department of Academic Affairs, Tawam Hospital, Al Ain, UAE. FAU - Chandrasekhar Nair, Satish AU - Chandrasekhar Nair S AD - Department of Academic Affairs, Tawam Hospital, Al Ain, UAE. FAU - Aljaberi, Najla AU - Aljaberi N AUID- ORCID: 0000-0002-1390-7934 AD - Department of Pediatrics, College of Medicine & Health Sciences, UAE University, Al Ain, UAE. najla.aljaberi@uaeu.ac.ae. AD - Department of Pediatrics, Tawam Hospital, Al Ain, UAE. najla.aljaberi@uaeu.ac.ae. LA - eng PT - Journal Article DEP - 20251014 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC12522406 OTO - NOTNLM OT - Autoimmunity OT - Childhood-onset OT - Familial OT - Genetics OT - Systemic lupus erythematous (SLE) OT - UAE COIS- Declarations. Human ethics and consent to participate: The study was performed in accordance with the Declaration of Helsinki and was approved by Tawam Hospital’s Institutional Review Board, Al Ain, UAE with the reference number (MF2058-2021-781). Informed consent was waived as per the regulation for retrospective studies. Competing interests: The authors declare no competing interests. EDAT- 2025/10/15 06:29 MHDA- 2025/10/15 06:30 PMCR- 2025/10/14 CRDT- 2025/10/15 00:28 PHST- 2024/11/09 00:00 [received] PHST- 2025/09/16 00:00 [accepted] PHST- 2025/10/15 06:30 [medline] PHST- 2025/10/15 06:29 [pubmed] PHST- 2025/10/15 00:28 [entrez] PHST- 2025/10/14 00:00 [pmc-release] AID - 10.1186/s41927-025-00574-z [pii] AID - 574 [pii] AID - 10.1186/s41927-025-00574-z [doi] PST - epublish SO - BMC Rheumatol. 2025 Oct 14;9(1):118. doi: 10.1186/s41927-025-00574-z. PMID- 37214354 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230523 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 15 DP - 2023 TI - Incidence and Predictors of an Abnormal Liver Function Test Among 674 Systemic Sclerosis Patients: A Cohort Study. PG - 81-92 LID - 10.2147/OARRR.S410165 [doi] AB - BACKGROUND: Abnormal liver function tests (LFTs) can indicate cirrhosis or liver cancer leading to mortality among systemic sclerosis (SSc) patients. No recent studies have investigated the clinical predictors of an abnormal LFT in SSc. We aimed to determine the incidence of abnormal LFT (including from hepatitis and cholestasis) and to identify its clinical predictors in SSc patients. METHODS: An historical cohort was conducted on 674 adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between January 2012 and November 2019 and who underwent routine screening for LFT. A Cox regression was used to analyze the clinical predictors of abnormal LFT. RESULTS: Four hundred and thirty cases, representing 4190 person-years, had abnormal LFTs (viz, from hepatitis, cholestasis, and cholestatic hepatitis) for an incidence rate of 10.2 per 100 person-years. The respective incidence of hepatitis, cholestasis, and cholestatic hepatitis was 20.5, 12.9, and 20.4 per 100 person-years. The respective median first-time detection of hepatitis, cholestasis, and cholestatic hepatitis was 3.0, 5.9, and 2.8 years, and none had signs or symptoms suggestive of liver disease. According to the Cox regression analysis, the predictors of an abnormal LFT in SSc were elderly onset of SSc (hazard ratio (HR) 1.02), alcoholic drinking (HR 1.74), high modified Rodnan Skin Score (mRSS) (HR 1.03), edematous skin (HR 2.94), Raynaud's phenomenon (HR 1.39), hyperCKaemia (HR 1.88), and methotrexate use (HR 1.55). In contrast, current sildenafil treatment (HR 0.63) and high serum albumin (HR 0.70) were protective factors. CONCLUSION: Occult hepatitis, cholestasis, and cholestatic hepatitis can be detected in SSc patients using LFT screening, especially in cases of early disease onset. The long-term outcome is uncertain, and more longitudinal research is required. CI - © 2023 Sawadpanich et al. FAU - Sawadpanich, Kookwan AU - Sawadpanich K AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Promasen, Palinee AU - Promasen P AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Mairiang, Pisaln AU - Mairiang P AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Sukeepaisarnjareon, Wattana AU - Sukeepaisarnjareon W AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Sangchan, Apichat AU - Sangchan A AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Suttichaimongkol, Tanita AU - Suttichaimongkol T AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Tangvoraphonkchai, Kawin AU - Tangvoraphonkchai K AD - Division of Gastroenterology and Hepatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AUID- ORCID: 0000-0002-1964-4389 AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. LA - eng PT - Journal Article DEP - 20230516 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC10199701 OTO - NOTNLM OT - abnormal liver function test OT - cholestasis OT - cholestatic hepatitis OT - cohort study OT - hepatitis OT - liver OT - scleroderma OT - scleroderma and related disorders OT - systemic sclerosis COIS- All authors declare that they do not have any competing interests. EDAT- 2023/05/22 19:11 MHDA- 2023/05/22 19:12 PMCR- 2023/05/16 CRDT- 2023/05/22 12:11 PHST- 2023/03/09 00:00 [received] PHST- 2023/05/02 00:00 [accepted] PHST- 2023/05/22 19:12 [medline] PHST- 2023/05/22 19:11 [pubmed] PHST- 2023/05/22 12:11 [entrez] PHST- 2023/05/16 00:00 [pmc-release] AID - 410165 [pii] AID - 10.2147/OARRR.S410165 [doi] PST - epublish SO - Open Access Rheumatol. 2023 May 16;15:81-92. doi: 10.2147/OARRR.S410165. eCollection 2023. PMID- 37131295 OWN - NLM STAT- MEDLINE DCOM- 20230504 LR - 20230523 IS - 1557-9891 (Electronic) IS - 1557-9883 (Print) IS - 1557-9883 (Linking) VI - 17 IP - 3 DP - 2023 May-Jun TI - The First Case Report of 47,XXY/46,XX/46,XY Mosaic Klinefelter Syndrome Patient With Mixed Connective Tissue Disorder. PG - 15579883231165173 LID - 10.1177/15579883231165173 [doi] LID - 15579883231165173 AB - Klinefelter syndrome (KS) mosaicism 47,XXY/46,XX/46,XY is an extremely rare disorder. Mixed connective tissue disorder (MCTD) is a systemic rheumatological disease with overlapping characteristic features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM)/dermatomyositis (DM), and rheumatoid arthritis (RA). It contains a higher titer level of U1-RNP and anti-RNP antibodies. A 50-year-old man was referred to our clinic with gynecomastia, lower extremity rash, persistent fever, arthralgia, muscle weakness, dry eye and mouth, Raynaud's phenomenon abnormal, and hormone levels. He was a follow-up patient for MCTD. Chromosome analysis of the patient revealed an abnormal karyotype of mos47,XXY/46,XX/46,XY. Fluorescence in situ hybridization (FISH) analysis indicated ish(SRYx1),(DZYx1)(DZX1x2)/ish (SRYx0),(DYZ1x0)(DZX1x2)/ish(SRYx1), (DZYx1)(DZX1x1). Although the prevalence of autoimmune diseases in Klinefelter syndrome is unknown, it is thought that the estimated frequency is higher than men, close levels to that of women. This might be explained by several genes that regulate the function of the immune system located on the X chromosome and the gene dosage mechanism that is the escape of X-inactivation in early embryogenesis for KS development. To the best of our knowledge, this is the first case to report a 47,XXY/46,XX/46,XY Klinefelter syndrome patient with MCTD. FAU - Kalayci Yigin, Aysel AU - Kalayci Yigin A AD - Department of Medical Genetics, Cerrahpaşa Medical School, Istanbul University-Cerrahpasa, Fatih/Istanbul, Türkiye. FAU - Alay, Mustafa Tarık AU - Alay MT AD - Department of Medical Genetics, Cerrahpaşa Medical School, Istanbul University-Cerrahpasa, Fatih/Istanbul, Türkiye. FAU - Uğurlu, Serdal AU - Uğurlu S AD - Division of Rheumatology, Department of Internal Medicine, Cerrahpaşa Medical School, Istanbul University-Cerrahpasa, Fatih/Istanbul, Türkiye. FAU - Seven, Mehmet AU - Seven M AUID- ORCID: 0000-0001-7878-2039 AD - Department of Medical Genetics, Cerrahpaşa Medical School, Istanbul University-Cerrahpasa, Fatih/Istanbul, Türkiye. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Mens Health JT - American journal of men's health JID - 101287723 SB - IM MH - Male MH - Humans MH - Female MH - *Klinefelter Syndrome/complications/genetics MH - *Mixed Connective Tissue Disease MH - In Situ Hybridization, Fluorescence MH - *Lupus Erythematosus, Systemic MH - Connective Tissue PMC - PMC10159258 OTO - NOTNLM OT - Klinefelter syndrome OT - MCTD OT - cytogenetic analyses OT - mosaicism COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/05/03 06:42 MHDA- 2023/05/04 12:42 PMCR- 2023/05/02 CRDT- 2023/05/03 01:03 PHST- 2023/05/04 12:42 [medline] PHST- 2023/05/03 06:42 [pubmed] PHST- 2023/05/03 01:03 [entrez] PHST- 2023/05/02 00:00 [pmc-release] AID - 10.1177_15579883231165173 [pii] AID - 10.1177/15579883231165173 [doi] PST - ppublish SO - Am J Mens Health. 2023 May-Jun;17(3):15579883231165173. doi: 10.1177/15579883231165173. PMID- 36140581 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220928 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 12 IP - 9 DP - 2022 Sep 9 TI - Analysis of Ana/Dfs70 Pattern in a Large Cohort of Autoimmune/Autoinflammatory Diseases Compared with First Degree Relatives and Healthy Controls Evaluated from Colombia. LID - 10.3390/diagnostics12092181 [doi] LID - 2181 AB - BACKGROUND: The presence of Antinuclear antibodies/Dense Fine Speckled 70 (ANA/DFS70) has been proposed as a negative biomarker in the process of exclusion of systemic autoimmune/autoinflammatory rheumatic diseases (SARD). The purpose was to evaluate and characterize ANA/DFS70 patients in a large Colombian population with SARD; rheumatoid arthritis (RA), Psoriasis (PsO), Undifferentiated connective tissue disease (UCTD), first-degree relatives of (FDR), and healthy controls (HC). METHODS: ANA determination was performed using indirect immunofluorescence. Samples with positive dense fine granular staining in the nucleoplasm of the interphase cell (AC2) fluorescence were confirmed with CytoBead/ANA and ANA/modified (Knocked out for the PSPI1 gen). RESULTS: 530 mestizo Colombian participants were included. ANA/DFS70 antibody positivity in the whole group was 2.3%, and 0.8% in SARD; no RA patients were positive. ANA/DFS70 positives in UCTD were three women; the average time of evolution of the disease was 9.4 years. The most frequent clinical findings were arthralgias, non-erosive arthritis, and Raynaud's phenomenon. The PsO positive was a woman with C-reactive protein (CRP) positivity and a negative erythrocyte sedimentation rate (ESR) without any other positive autoantibody or extracutaneous manifestation. FDR and HC positives were 7/8 women. All were negative for other autoantibodies. CONCLUSIONS: ANA/DFS70 autoantibodies were present in Colombian patients with SARD at a shallow frequency, they were more prevalent in healthy individuals. FAU - Romero-Sánchez, Consuelo AU - Romero-Sánchez C AUID- ORCID: 0000-0002-6973-7639 AD - Rheumatology and Immunology Department, Hospital Militar Central, Bogota 110111, Colombia. AD - Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogota 110111, Colombia. AD - Cellular and Immunology Group/InmuBo, Universidad El Bosque, Bogota 110111, Colombia. FAU - Calixto, Omar-Javier AU - Calixto OJ AD - Rheumatology and Immunology Department, Hospital Militar Central, Bogota 110111, Colombia. AD - Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogota 110111, Colombia. FAU - Romero-Alvarez, Veronica AU - Romero-Alvarez V AD - Rheumatology and Immunology Department, Hospital Militar Central, Bogota 110111, Colombia. AD - Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogota 110111, Colombia. FAU - Vargas-Martin, Alejandra AU - Vargas-Martin A AD - Faculty of Basic Science, Universidad Colegio Mayor de Cundinamarca, Bogota 111311, Colombia. FAU - Castro, Luis AU - Castro L AD - Hospital Militar Central, Dermatology Department, Universidad Militar Nueva Granada, Bogota 110111, Colombia. FAU - Amador, Julio AU - Amador J AD - Hospital Militar Central, Dermatology Department, Universidad Militar Nueva Granada, Bogota 110111, Colombia. FAU - Marín-Acevedo, Daniela AU - Marín-Acevedo D AD - Hospital Militar Central, Dermatology Department, Universidad Militar Nueva Granada, Bogota 110111, Colombia. FAU - Acevedo-Godoy, Mónica AU - Acevedo-Godoy M AD - Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogota 110111, Colombia. AD - Immunology Laboratory, Hospital Militar Central, Bogota 110111, Colombia. FAU - Rincón-Riaño, Diana AU - Rincón-Riaño D AD - Rheumatology and Immunology Department, Hospital Militar Central, Bogota 110111, Colombia. FAU - Bello-Gualtero, Juan Manuel AU - Bello-Gualtero JM AD - Rheumatology and Immunology Department, Hospital Militar Central, Bogota 110111, Colombia. AD - Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogota 110111, Colombia. LA - eng GR - No.130865740792-2014/Colciencias/ PT - Journal Article DEP - 20220909 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC9498280 OTO - NOTNLM OT - ANA/DFS70 OT - antinuclear antibodies OT - healthy individuals OT - systemic autoimmune/autoinflammatory rheumatic diseases COIS- The authors declare no conflict of interest. EDAT- 2022/09/24 06:00 MHDA- 2022/09/24 06:01 PMCR- 2022/09/09 CRDT- 2022/09/23 01:12 PHST- 2022/08/10 00:00 [received] PHST- 2022/08/24 00:00 [revised] PHST- 2022/08/25 00:00 [accepted] PHST- 2022/09/23 01:12 [entrez] PHST- 2022/09/24 06:00 [pubmed] PHST- 2022/09/24 06:01 [medline] PHST- 2022/09/09 00:00 [pmc-release] AID - diagnostics12092181 [pii] AID - diagnostics-12-02181 [pii] AID - 10.3390/diagnostics12092181 [doi] PST - epublish SO - Diagnostics (Basel). 2022 Sep 9;12(9):2181. doi: 10.3390/diagnostics12092181. PMID- 17852993 OWN - NLM STAT- MEDLINE DCOM- 20071206 LR - 20071204 IS - 1403-4948 (Print) IS - 1403-4948 (Linking) VI - 35 IP - 5 DP - 2007 TI - Hospital contacts for chronic diseases among danish seafarers and fishermen: a population-based cohort study. PG - 481-9 AB - OBJECTIVES: Seafarers' and fishermen's working conditions may impact on their lifestyle and health. Standardized hospital contact ratios (SHCRs) were compared in two time periods and the relative risks of hospital contact as a function of employment time were estimated. METHODS: Cohorts of all Danish seafarers (officers and non-officers) registered by the Danish Maritime Authority (DMA) 1989-98 and fishermen retrieved from a pension registry 1989-98 were linked to the nationwide Occupational Hospitalization Registry (OHR) and followed up for incident diseases in two five-year time periods, from 1 January 1994 and 1 January 1999, respectively, using rates specific for age and calendar time for the entire Danish workforce as a reference. RESULTS: The SHCRs for lung and cardiovascular diseases were high for non-officers. Among male officers, the SHCR for diabetes was high in the 1999 cohort and the SHCR for chronic heart diseases was statistically significantly higher in the 1999 than in the 1994 cohort. For both time periods high SHCR values were found for bronchitis, emphysema, cancer of the lung, alcohol-related liver diseases, and diabetes among male non-officers, and lung cancer among male officers. Among female non-officers, a high SHCR for skin melanomas was seen. Among fishermen high SHCRs for bronchitis, emphysema, lung cancer, and Raynaud's syndrome were found in both cohorts. No duration-response pattern was observed in any of the analyses, which may reflect health- and lifestyle-related selection into the trades or a healthy worker effect. CONCLUSIONS: Danish seafarers, especially short-term employees, had an elevated risk of hospitalization for lifestyle-related diseases. FAU - Kaerlev, Linda AU - Kaerlev L AD - Research Unit of Maritime Medicine, University of Southern Denmark, Esbjerg, Denmark. L.kaerlev@dadlnet.dk FAU - Dahl, Søren AU - Dahl S FAU - Nielsen, Per Sabro AU - Nielsen PS FAU - Olsen, Jørn AU - Olsen J FAU - Hannerz, Harald AU - Hannerz H FAU - Jensen, Anker AU - Jensen A FAU - Tüchsen, Finn AU - Tüchsen F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Sweden TA - Scand J Public Health JT - Scandinavian journal of public health JID - 100883503 SB - IM MH - Adult MH - Chronic Disease/*epidemiology/mortality MH - Cohort Studies MH - Denmark/epidemiology MH - Employment MH - Environmental Exposure/*adverse effects MH - Female MH - Follow-Up Studies MH - Hospitalization/*statistics & numerical data MH - Humans MH - Life Style MH - Male MH - Middle Aged MH - Naval Medicine MH - Registries MH - Risk Factors MH - *Ships MH - Workplace EDAT- 2007/09/14 09:00 MHDA- 2007/12/07 09:00 CRDT- 2007/09/14 09:00 PHST- 2007/09/14 09:00 [pubmed] PHST- 2007/12/07 09:00 [medline] PHST- 2007/09/14 09:00 [entrez] AID - 778146350 [pii] AID - 10.1080/14034940701267385 [doi] PST - ppublish SO - Scand J Public Health. 2007;35(5):481-9. doi: 10.1080/14034940701267385. PMID- 30079243 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230926 IS - 2046-1402 (Print) IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 7 DP - 2018 TI - What does it mean if a patient is positive for anti-Jo-1 in routine hospital practice? A retrospective nested case-control study. PG - 698 LID - 10.12688/f1000research.14834.1 [doi] LID - 698 AB - Background:  It is widely believed that patients bearing auto-antibodies to histidyl tRNA synthetase (anti-Jo-1) very likely have a connective tissue disease including myositis and interstitial lung disease.  The value of positive tests in low disease prevalence settings such as those tested in routine care is unknown.  We sought to determine the value of anti-Jo-1 auto-antibodies in routine practice. Methods: Our study was a nested case control study within a retrospective cohort of all patients tested for anti-ENA our hospital, from any hospital department, between January 2013 and December 2014.  Data was extracted from electronic records of anti-Jo-1 positive patients and randomly selected ENA negative patients (ratio of 1:2), allowing for a minimum follow up of at least 12 months after first testing. Results: 4009 samples (3581 patients) were tested.  Anti-ENA was positive in 616 (17.2%) patients, 40 (1.1%) were anti-Jo-1 positive. Repeat ENA testing was done for 350/3581 (9.8%) patients (428 of 4009 (10.7%) samples) and in 7/40 (17.5%) of anti-Jo-1 positive patients. The median interval between the first and second request was 124 days (inter-quartile range 233 days).  The frequencies of interstitial lung disease (ILD), myositis and Raynaud's were comparable for anti-Jo-1 positive patients (n=40) and 80 randomly selected ENA negative controls.  Positive tests led to additional diagnostic testing in the absence of clinical disease.  Sensitivity of Jo-1 for ILD was 50% (CI 19-81%), specificity 68% (CI 59-77%), positive predictive value 12.5% (CI 4 to 27%) and negative predictive value 93.8% (CI 86-98%). Of 10 (25%) patients with high anti-Jo1 levels, 3 had ILD, one myositis and two a malignancy (disseminated melanoma and CML).  Conclusion: Anti-Jo-1 is uncommon in a heterogenous hospital population and is only weakly predictive for ILD.  Repeated test requests were common and potentially unnecessary indicating that controls over repeat requests could yield significant cost savings. FAU - Jobanputra, Paresh AU - Jobanputra P AUID- ORCID: 0000-0002-2712-667X AD - Department of Rheumatology, Queen Elizabeth Hospital, Birmingham, Birmingham, B15 2TH, UK. FAU - Malick, Feryal AU - Malick F AD - Department of Rheumatology, Queen Elizabeth Hospital, Birmingham, Birmingham, B15 2TH, UK. AD - Department of Rheumatology, Epsom and St Helier University Hospitals NHS Trust, Carshalton, Surrey, SM15 1AA, UK. FAU - Derrett-Smith, Emma AU - Derrett-Smith E AD - Department of Rheumatology, Queen Elizabeth Hospital, Birmingham, Birmingham, B15 2TH, UK. FAU - Plant, Tim AU - Plant T AD - Department of Clinical Immunology, School of Medicine, University of Birmingham, Birmingham, B15 2TT, UK. FAU - Richter, Alex AU - Richter A AD - Department of Clinical Immunology, School of Medicine, University of Birmingham, Birmingham, B15 2TT, UK. LA - eng PT - Journal Article DEP - 20180604 PL - England TA - F1000Res JT - F1000Research JID - 101594320 PMC - PMC6058461 OTO - NOTNLM OT - Jo-1 OT - anti-histidyl-tRNA synthetase OT - connective tissue disease OT - interstitial lung disease OT - myositis OT - over diagnosis OT - synthetase syndrome COIS- No competing interests were disclosed. EDAT- 2018/08/07 06:00 MHDA- 2018/08/07 06:01 PMCR- 2018/06/04 CRDT- 2018/08/07 06:00 PHST- 2018/05/21 00:00 [accepted] PHST- 2018/08/07 06:00 [entrez] PHST- 2018/08/07 06:00 [pubmed] PHST- 2018/08/07 06:01 [medline] PHST- 2018/06/04 00:00 [pmc-release] AID - 10.12688/f1000research.14834.1 [doi] PST - epublish SO - F1000Res. 2018 Jun 4;7:698. doi: 10.12688/f1000research.14834.1. eCollection 2018. PMID- 42199401 OWN - NLM STAT- MEDLINE DCOM- 20260527 LR - 20260527 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Recurrent aseptic meningitis in adults: a potential indicator of undetermined autoinflammatory disease. PG - 1842256 LID - 10.3389/fimmu.2026.1842256 [doi] LID - 1842256 AB - BACKGROUND: Autoinflammatory diseases (AIDs) are characterized by recurrent episodes of inflammation caused by primary immune system dysregulation. Central nervous manifestations, including aseptic meningitis (AM), are rare in AIDs. In this study, we identified six patients with undetermined AIDs characterized by recurrent aseptic meningitis (RAM). METHODS: Patients referred to Centre National de Référence des Maladies Auto-Inflammatoires for AM with an undetermined diagnosis were included. AIDs with RAM were defined by two or more episodes of aseptic meningitis (>10 leukocytes) with a predominance of neutrophils (>50%) and an association with autoinflammatory elements (i.e., fever, C-reactive protein [CRP] elevation) and the absence of an alternative diagnosis. RESULTS: Six patients (four men, two women) were included, all of whom presented with fever and elevated CRP levels (≥30 mg/L), and five patients had clinical meningitis syndrome. The median patient age at disease onset was 41 years (range, 28-49). The frequency of AM was 1-24 episodes/year, and the duration of episodes was ≤8 days. Associated clinical symptoms included cutaneous manifestations (rash [n = 3], Raynaud's syndrome [n = 1]), arthromyalgia (n = 2), and central neurological or ophthalmologic manifestations (n = 2). All patients underwent genetic analysis focusing on AIDs, and variants of undetermined significance within four autoinflammatory genes were noted in three patients. All patients benefited from classical therapeutic regimens of AIDs including corticosteroids, colchicine, and anti-interleukin (IL)-1 receptor or anti-IL6 antibodies. CONCLUSION: RAM was identified as a potential indicator for undetermined AIDs. Physicians should be aware of this condition and provide adequate analysis and treatment. CI - Copyright © 2026 Martinot, Ardois, Chauvet, Cavestro, Blanche, Papo and Murarasu. FAU - Martinot, Martin AU - Martinot M AD - Service de Maladies Infectieuse, Hôpitaux Civils de Colmar, Colmar, France. FAU - Ardois, Samuel AU - Ardois S AD - Service de Médecine Interne, CHRU Rennes, Rennes, France. FAU - Chauvet, Elodie AU - Chauvet E AD - Service de Médecine Interne, CHRU Montpellier, Montpellier, France. FAU - Cavestro, David AU - Cavestro D AD - Service de Maladies Infectieuse, Hôpitaux Civils de Colmar, Colmar, France. FAU - Blanche, Philippe AU - Blanche P AD - Département de Médecine Interne, Hôpital Cochin, APHP, Paris, France. FAU - Papo, Thomas AU - Papo T AD - Service de Médecine Interne, Hôpital Bichat, Université Paris Cité, Paris, France. FAU - Murarasu, Anne AU - Murarasu A AD - Service de Médecine Interne, CHRU Tours, Tours, France. LA - eng PT - Journal Article DEP - 20260511 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Biomarkers) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Humans MH - *Meningitis, Aseptic/diagnosis/etiology/drug therapy/immunology MH - Male MH - Female MH - Adult MH - Middle Aged MH - Recurrence MH - Biomarkers MH - *Autoimmune Diseases/diagnosis MH - C-Reactive Protein PMC - PMC13199347 OTO - NOTNLM OT - USAID OT - aseptic meningitis OT - autoinflammation OT - autoinflammatory diseases OT - recurrent meningitis COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/05/27 12:20 MHDA- 2026/05/27 12:36 PMCR- 2026/05/11 CRDT- 2026/05/27 04:26 PHST- 2026/03/29 00:00 [received] PHST- 2026/04/22 00:00 [revised] PHST- 2026/04/29 00:00 [accepted] PHST- 2026/05/27 12:36 [medline] PHST- 2026/05/27 12:20 [pubmed] PHST- 2026/05/27 04:26 [entrez] PHST- 2026/05/11 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1842256 [doi] PST - epublish SO - Front Immunol. 2026 May 11;17:1842256. doi: 10.3389/fimmu.2026.1842256. eCollection 2026. PMID- 35128327 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220501 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 32 IP - 4 DP - 2021 Dec TI - Prognostic Role of Measurement of Renal Resistive Index in Systemic Sclerosis. PG - 345-349 LID - 10.31138/mjr.32.4.345 [doi] AB - OBJECTIVE: The spectrum of vascular involvement in systemic sclerosis (SSc) includes digital ulcers, gangrene, Raynaud's phenomenon, renovascular disease, and pulmonary hypertension (PH). Recognition of markers of subclinical vascular disease in SSc is an area of active research, but such studies are limited. This study assesses the role of measurement of the renal resistive index (RRI) as an early marker of renal and systemic vasculopathy. It is a step forward towards examining the possibility of a "unified vascular phenotype' in SSc. METHODS: In this single-centre prospective study, RRI was calculated for SSc patients >18 years age. Elevated RRI (>0.7) was correlated with renal function (eGFR and proteinuria) and systemic vasculopathy manifestations like digital ulcers, digital infarcts, and PH. RESULTS: A total of 73 patients with mean (SD) age 41.8(10.9) years were included. Mean (SD) RRI in the right and left renal artery was 0.65(0.08) and 0.66(0.07), respectively. 16 (21.9%) patients had elevated RRI (>0.7). A strong negative correlation was noted between elevated RRI and eGFR (r= -0.96, p=0.03). The percentage of patients with overt proteinuria was higher in the group with elevated RRI (20% versus 7%) (p=0.16). Similarly, digital ulcers (56% vs 33%) and digital pitting (50% vs 35%.) were numerically higher in the group with raised RRI, although statistical significance was not reached because of small numbers (p=0.09 and 0.28, respectively). No correlation of RRI with PH was identified. CONCLUSION: RRI correlates well with asymptomatic renal dysfunction and holds promise in the assessment of systemic vasculopathy. However, validation in studies with a larger sample size is needed. CI - © 2021 The Mediterranean Journal of Rheumatology (MJR). FAU - Sharma, Shefali Khanna AU - Sharma SK AD - Clinical Immunology and Rheumatology Division. FAU - Chattopadhyay, Arghya AU - Chattopadhyay A AD - Clinical Immunology and Rheumatology Division. FAU - Jain, Siddharth AU - Jain S AD - Clinical Immunology and Rheumatology Division. FAU - Sharma, Chitra Raj AU - Sharma CR AD - Department of Internal Medicine. FAU - Mishra, Debashish AU - Mishra D AD - Clinical Immunology and Rheumatology Division. FAU - Rathi, Manish AU - Rathi M AD - Department of Nephrology. FAU - Prakash, Mahesh AU - Prakash M AD - Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Jain, Sanjay AU - Jain S AD - Clinical Immunology and Rheumatology Division. LA - eng PT - Journal Article DEP - 20211227 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC8802198 OTO - NOTNLM OT - digital ulcers OT - proteinuria OT - pulmonary hypertension OT - renal doppler indices OT - renal resistive index OT - systemic sclerosis OT - vasculopathy EDAT- 2022/02/08 06:00 MHDA- 2022/02/08 06:01 PMCR- 2021/12/27 CRDT- 2022/02/07 05:37 PHST- 2020/09/29 00:00 [received] PHST- 2021/06/06 00:00 [revised] PHST- 2021/09/03 00:00 [accepted] PHST- 2022/02/07 05:37 [entrez] PHST- 2022/02/08 06:00 [pubmed] PHST- 2022/02/08 06:01 [medline] PHST- 2021/12/27 00:00 [pmc-release] AID - mjr-32-4-345 [pii] AID - 10.31138/mjr.32.4.345 [doi] PST - epublish SO - Mediterr J Rheumatol. 2021 Dec 27;32(4):345-349. doi: 10.31138/mjr.32.4.345. eCollection 2021 Dec. PMID- 30066757 OWN - NLM STAT- MEDLINE DCOM- 20180910 LR - 20240229 IS - 1806-4841 (Electronic) IS - 0365-0596 (Print) IS - 0365-0596 (Linking) VI - 93 IP - 4 DP - 2018 Jul-Aug TI - Prevalence and reactivity of anti-melanoma differentiation-associated gene 5 (anti-MDA-5) autoantibody in Brazilian patients with dermatomyositis. PG - 517-523 LID - S0365-0596(20)30473-6 [pii] LID - 10.1590/abd1806-4841.20186803 [doi] AB - BACKGROUND: There have been no studies to date on the frequency and reactivity of aanti-melanoma differentiation-associated gene 5 (anti-MDA-5) in samples from the Brazilian population with dermatomyositis. OBJECTIVES: To analyze this autoantibody in the Brazilian population. METHODS: This was a single-center cross-sectional study in which 131 consecutive adult patients (109 dermatomyositis and 22 clinically amyopathic dermatomyositis) with active disease were evaluated from 2000 to 2016. Analysis of the anti-MDA-5 autoantibody was performed by ELISA. RESULTS: The presence of this autoantibody was observed in 14.7% and 22.7% of patients with dermatomyositis and clinically amyopathic dermatomyositis, respectively. In the case of dermatomyositis, the autoantibody was associated less frequently with Raynaud's phenomenon and periungual hyperemia (P<0.05). In clinically amyopathic dermatomyositis, the presence of this autoantibody was not associated statistically with any demographic, clinical, laboratory, or imaging characteristics. STUDY LIMITATIONS: The cross-sectional study design did not allow establishing a temporal correlation between anti-MDA-5 autoantibody and various study variables. In addition, pulmonary function tests were not performed in the patients. CONCLUSIONS: The frequency of anti-MDA-5 autoantibody was comparable to that of other populations with dermatomyositis, but with a different reactivity than described in the literature. In addition, there was a phenotypic variability between our patients with clinically amyopathic dermatomyositis and those described in the literature. Further studies are needed to confirm the current study's findings and elucidate this autoantibody's reactivity in Brazilians with idiopathic inflammatory myopathies. FAU - Borges, Isabela Bruna Pires AU - Borges IBP AUID- ORCID: 0000-0002-5207-7603 AD - Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP), Brasil. FAU - Silva, Marilda Guimarães AU - Silva MG AUID- ORCID: 0000-0001-5668-1130 AD - Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP), Brasil. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AUID- ORCID: 0000-0002-3682-4517 AD - Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP), Brasil. LA - eng PT - Journal Article PL - Spain TA - An Bras Dermatol JT - Anais brasileiros de dermatologia JID - 0067662 RN - 0 (Autoantibodies) RN - EC 3.6.1.- (IFIH1 protein, human) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - Amyopathic dermatomyositis SB - IM MH - Adult MH - Autoantibodies/*blood/immunology MH - Cross-Sectional Studies MH - Dermatomyositis/blood/complications/*immunology MH - Disease Progression MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Interferon-Induced Helicase, IFIH1/blood/*immunology MH - Male MH - Severity of Illness Index MH - Statistics, Nonparametric PMC - PMC6063131 COIS- Conflict of interest: None. EDAT- 2018/08/02 06:00 MHDA- 2018/09/11 06:00 PMCR- 2018/07/01 CRDT- 2018/08/02 06:00 PHST- 2016/12/20 00:00 [received] PHST- 2017/06/23 00:00 [accepted] PHST- 2018/08/02 06:00 [entrez] PHST- 2018/08/02 06:00 [pubmed] PHST- 2018/09/11 06:00 [medline] PHST- 2018/07/01 00:00 [pmc-release] AID - S0365-0596(20)30473-6 [pii] AID - 10.1590/abd1806-4841.20186803 [doi] PST - ppublish SO - An Bras Dermatol. 2018 Jul-Aug;93(4):517-523. doi: 10.1590/abd1806-4841.20186803. PMID- 25797524 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 33 IP - 4 Suppl 91 DP - 2015 Jul-Aug TI - Prospective evaluation of frequency of signs of systemic sclerosis in 76 patients with morphea. PG - S23-5 AB - OBJECTIVES: Some authors consider that morphoea and systemic sclerosis (SSc) could be part of the same disease spectrum. The aim of this study was to analyse the prevalence of signs indicative of SSc in a cohort of patients with morphoea. METHODS: This is a prospective multi-centre study performed in four French academic dermatology departments: 76 patients with morphoea and 101 age- and sex-matched controls, who underwent complete clinical examination, were enrolled. A systemic search for signs indicative of SSc (e.g. Raynaud's phenomenon, reflux) was performed with the help of a standardised questionnaire. RESULTS: There were 58 women and 18 men (ration=3/1) with a median age of 59 years. Mean age at diagnosis was 54 years (extremes, 13-87). 49 subjects had plaque morphoea, 9 had generalised morphoea and 18 had linear morphoea. Mean duration of morphoea was 7.9 years. Signs possibly indicative of SSc were noted in four patients of the control group and in 8 patients with morphoea. This difference was not statistically significant (p=0.129). Further investigations ruled out SSc in all patients. CONCLUSIONS: Signs indicative of SSc are statistically not more frequently present in patients with morphoea than in controls and this study does not support the view that those 2 entities are part of a common disease spectrum. FAU - Lipsker, Dan AU - Lipsker D AD - Université de Strasbourg, Faculté de Médecine, Clinique Dermatologique, Strasbourg, France. dan.lipsker@chru-strasbourg.fr. FAU - Bessis, Didier AU - Bessis D AD - Université Montpellier I, Service de Dermatologie, CHU de Montpellier, Montpellier, France. FAU - Cosnes, Anne AU - Cosnes A AD - Service de Dermatologie, Hôpital Henri Mondor, Créteil, France. FAU - Kluger, Nicolas AU - Kluger N AD - Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki, Skin and Allergy Hospital, Helsinki University Central Hospital, Finland. FAU - Lutz, Virginie AU - Lutz V AD - Université de Strasbourg, Faculté de Médecine, Clinique Dermatologique, Strasbourg, France. FAU - Sauleau, Erik AU - Sauleau E AD - Service de Santé Publique, Groupe méthode en recherche clinique, Biostatistiques et Méthodologies, Université de Strasbourg, CHU Strasbourg, Strasbourg, France. FAU - Francès, Camille AU - Francès C AD - Service de Dermatologie, Hôpital Tenon, Université Pierre et Marie Curie-Paris 6, Paris, France. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20150310 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Female MH - France/epidemiology MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Prospective Studies MH - Scleroderma, Localized/diagnosis/*epidemiology MH - Scleroderma, Systemic/diagnosis/*epidemiology MH - Surveys and Questionnaires MH - Time Factors MH - Young Adult EDAT- 2015/03/24 06:00 MHDA- 2015/10/27 06:00 CRDT- 2015/03/24 06:00 PHST- 2014/09/08 00:00 [received] PHST- 2014/11/24 00:00 [accepted] PHST- 2015/03/24 06:00 [entrez] PHST- 2015/03/24 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 8631 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S23-5. Epub 2015 Mar 10. PMID- 21951943 OWN - NLM STAT- MEDLINE DCOM- 20120309 LR - 20220409 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 20 IP - 13 DP - 2011 Nov TI - Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis. PG - 1345-55 LID - 10.1177/0961203311416694 [doi] AB - OBJECTIVE: It is known that age at disease onset has an impact on the clinical course and outcome of systemic lupus erythematosus (SLE); however, the precise differences in the prevalence of SLE manifestations are debated. Our objective was to conduct a systematic literature review and meta-analysis of all studies that directly compare childhood-onset lupus with adult-onset lupus to determine which clinical manifestations vary with age at disease onset. METHODS: A comprehensive literature search of the MEDLINE/PubMed,EMBASE, CINAHL, and SCOPUS databases was conducted to identify relevant articles. Study quality was assessed using the STROBE checklist. Study sample characteristics and clinical manifestation event rates were extracted from each study. Pooled odds ratios (ORs) were calculated using the random effects method, and between-study heterogeneity was quantified using the I (2) statistic. RESULTS: Of the 484 studies identified by the search strategy, 16 were included in this review. The total number of patients was 5993 adults and 905 children with SLE. Study quality was on average 16/32, ranging from 8 to 29. Several statistically significant differences were found: malar rash, ulcers/mucocutaneous involvement, renal involvement, proteinuria, urinary cellular casts, seizures, thrombocytopenia, hemolytic anemia, fever, and lymphadenopathy were more common in childhood-onset SLE with ORs ranging from 1.3 to 3.7; however, Raynaud's, pleuritis, and sicca were more common in adult-onset SLE (twice as common). CONCLUSIONS: The results of this meta-analysis suggest that some clinical manifestations of lupus are different in childhood-onset SLE and adult-onset SLE. FAU - Livingston, B AU - Livingston B AD - Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada. FAU - Bonner, A AU - Bonner A FAU - Pope, J AU - Pope J LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20110927 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Child MH - Child, Preschool MH - Databases, Factual MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*epidemiology/pathology/*physiopathology MH - Odds Ratio MH - Young Adult EDAT- 2011/09/29 06:00 MHDA- 2012/03/10 06:00 CRDT- 2011/09/29 06:00 PHST- 2011/09/29 06:00 [entrez] PHST- 2011/09/29 06:00 [pubmed] PHST- 2012/03/10 06:00 [medline] AID - 0961203311416694 [pii] AID - 10.1177/0961203311416694 [doi] PST - ppublish SO - Lupus. 2011 Nov;20(13):1345-55. doi: 10.1177/0961203311416694. Epub 2011 Sep 27. PMID- 19417117 OWN - NLM STAT- MEDLINE DCOM- 20090715 LR - 20250529 IS - 1542-6270 (Electronic) IS - 1060-0280 (Print) IS - 1060-0280 (Linking) VI - 43 IP - 5 DP - 2009 May TI - Anaphylactic reaction to anakinra in a rheumatoid arthritis patient intolerant to multiple nonbiologic and biologic disease-modifying antirheumatic drugs. PG - 967-72 LID - 10.1345/aph.1L573 [doi] AB - OBJECTIVE: To report a case of probable anaphylaxis due to anakinra in a patient with rheumatoid arthritis and multiple drug allergies. CASE SUMMARY: A 46-year-old Indian female with rheumatoid arthritis demonstrated distinct adverse reactions to all commercially available anti-tumor necrosis factor therapies, sulfasalazine, and hydroxychloroquine. Over a 4-year period her disease remained active during therapy with methotrexate and prednisone. Biologics were added sequentially, with development of intolerable reactions, first to infliximab (urticarial rash, infusion reactions) after 3 doses, and then to etanercept (autoantibodies, worsening Raynaud's phenomenon, digital microinfarcts) after 1 year. Following 2 months of daily injections of anakinra, she experienced an immediate immunoglobulin E-mediated anaphylactic reaction within 20 minutes of an injection, as evidenced by positive testing to both anakinra and histamine with the skin prick method. The patient subsequently started adalimumab therapy, which was discontinued after the fourth dose due to the development of generalized hives. DISCUSSION: The Naranjo probability scale demonstrated a probable relationship between anaphylaxis and anakinra in this patient. Although cases of anakinra-related hypersensitivity have been reported in patients in which therapy was interrupted and then reintroduced, to our knowledge, this is the first report of anaphylaxis with continuous therapy. CONCLUSIONS: This unusual case of a patient with multiple drug allergies presents a difficult clinical scenario, which was unsuccessfully managed with multiple biologic therapies on a trial-and-error basis. In the future, pharmacogenetics may help to better identify individuals at risk for multiple drug reactions and preclude unnecessary exposure to potentially harmful therapeutic options in similar patients. FAU - Desai, Ditina AU - Desai D AD - Care Improvement Plus of Maryland, Inc., XLHealth Corporation, The Warehouse at Camden Yards, Baltimore, MD, USA. FAU - Goldbach-Mansky, Raphaela AU - Goldbach-Mansky R FAU - Milner, Joshua D AU - Milner JD FAU - Rabin, Ronald L AU - Rabin RL FAU - Hull, Keith AU - Hull K FAU - Pucino, Frank AU - Pucino F FAU - Colburn, Nona AU - Colburn N LA - eng GR - ZIA AR041138/ImNIH/Intramural NIH HHS/United States PT - Case Reports PT - Journal Article PL - United States TA - Ann Pharmacother JT - The Annals of pharmacotherapy JID - 9203131 RN - 0 (Antirheumatic Agents) RN - 0 (Interleukin 1 Receptor Antagonist Protein) SB - IM MH - Anaphylaxis/*chemically induced MH - Antirheumatic Agents/*adverse effects MH - Arthritis, Rheumatoid/*drug therapy/immunology MH - Drug Hypersensitivity/*etiology MH - Female MH - Humans MH - Interleukin 1 Receptor Antagonist Protein/*adverse effects MH - Middle Aged PMC - PMC2938799 MID - NIHMS228605 EDAT- 2009/05/07 09:00 MHDA- 2009/07/16 09:00 PMCR- 2010/09/14 CRDT- 2009/05/07 09:00 PHST- 2009/05/07 09:00 [entrez] PHST- 2009/05/07 09:00 [pubmed] PHST- 2009/07/16 09:00 [medline] PHST- 2010/09/14 00:00 [pmc-release] AID - 43/5/967 [pii] AID - 10.1345/aph.1L573 [doi] PST - ppublish SO - Ann Pharmacother. 2009 May;43(5):967-72. doi: 10.1345/aph.1L573. PMID- 17477486 OWN - NLM STAT- MEDLINE DCOM- 20070622 LR - 20191210 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 34 IP - 5 DP - 2007 May TI - Systemic sclerosis - continuing progress in developing clinical measures of response. PG - 1194-200 AB - Few randomized controlled trials (RCT) have shown a demonstrable treatment effect in systemic sclerosis (SSc), making it difficult to evaluate outcome measures in this disease indication. Results from recent RCT, including those evaluating cyclophosphamide for SSc interstitial lung disease and endothelin receptor antagonists for pulmonary hypertension, have allowed analysis of certain organ-specific endpoints using the OMERACT filter. An earlier metaanalysis established that skin score, measures of Raynaud's, pulmonary function tests, blood pressure, pain, Health Assessment Questionnaire, and Medical Outcomes Survey Short-Form 36 are validated outcome measures in SSc. At OMERACT 8, data regarding validation of high-resolution computed tomography of the lungs, 6-minute walk test, and patient reported outcomes in SSc were presented. A Delphi exercise to develop consensus regarding a combined set of noninvasive measures for pulmonary arterial hypertension (PAH) is under way. Given the protean nature of this illness and its multiorgan system involvement, a composite responder index may be preferable. Another Delphi exercise is designed to develop consensus regarding a combined SSc response index to be validated in future RCT. FAU - Furst, Daniel AU - Furst D AD - University of California at Los Angeles, Los Angeles, California 90025, USA. defurst@mednet.ucla.edu FAU - Khanna, Dinesh AU - Khanna D FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M FAU - Clements, Philip AU - Clements P FAU - Steen, Virginia AU - Steen V FAU - Pope, Janet AU - Pope J FAU - Merkel, Peter AU - Merkel P FAU - Foeldvari, Ivan AU - Foeldvari I FAU - Seibold, James AU - Seibold J FAU - Pittrow, David AU - Pittrow D FAU - Polisson, Richard AU - Polisson R FAU - Strand, Vibeke AU - Strand V LA - eng GR - HD051953/HD/NICHD NIH HHS/United States GR - K24 AR2224-01A1/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Consensus MH - Delphi Technique MH - Endpoint Determination/*methods MH - Health Status MH - Humans MH - Meta-Analysis as Topic MH - *Outcome Assessment, Health Care MH - Randomized Controlled Trials as Topic MH - Scleroderma, Systemic/*diagnosis/physiopathology/*therapy MH - Severity of Illness Index MH - Treatment Outcome RF - 47 EDAT- 2007/05/05 09:00 MHDA- 2007/06/23 09:00 CRDT- 2007/05/05 09:00 PHST- 2007/05/05 09:00 [pubmed] PHST- 2007/06/23 09:00 [medline] PHST- 2007/05/05 09:00 [entrez] AID - 0315162X-34-1194 [pii] PST - ppublish SO - J Rheumatol. 2007 May;34(5):1194-200. PMID- 40843865 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250914 LR - 20250914 IS - 2571-841X (Electronic) IS - 2571-841X (Linking) VI - 8 IP - 3 DP - 2025 Jul 26 TI - Reclassification of Seronegative Rheumatoid Arthritis as Anti-PL-12 Antisynthetase Syndrome with Interstitial Lung Disease and Joint Involvement-Case Report. LID - 10.3390/reports8030123 [doi] LID - 123 AB - Background and Clinical Significance: Antisynthetase syndrome (ASyS) is a rare autoimmune entity defined by the presence of anti-aminoacyl-t ribonucleic acid (RNA) synthetase autoantibodies and classically associated with a triad of interstitial lung disease (ILD), inflammatory myopathy, and arthritis. Additional clinical features may include Raynaud's phenomenon and "mechanic's hands". Among antisynthetase antibodies, anti-PL-12 is notably associated with predominant or isolated ILD and may occur in the absence of clinically evident myositis, thereby complicating timely diagnosis. Case Presentation: We are presenting a 45-year-old non-smoking female patient with a prior diagnosis of seronegative rheumatoid arthritis (RA) who developed progressive dyspnea, dry cough, and sicca symptoms. High-resolution computed tomography revealed a nonspecific interstitial pneumonia (NSIP) pattern. Despite normal creatine kinase and lactate dehydrogenase levels, serological work-up revealed positive anti-PL-12 and anti-Ro52 antibodies, supporting a diagnosis of antisynthetase syndrome without myositis, fulfilling the diagnostic criteria for ASyS per Connors and Solomon. Treatment with corticosteroids and cyclophosphamide induced clinical and functional respiratory improvement, while azathioprine was initiated for maintenance. Conclusions: This case underscores the clinical heterogeneity of antisynthetase syndrome and highlights the diagnostic challenge posed by anti-PL-12-associated ILD in the absence of myositis. Importantly, it demonstrates that in patients with pre-existing rheumatologic diagnoses, the emergence of atypical pulmonary manifestations warrants repeat serologic evaluation to assess ASyS and other autoimmune conditions. Early diagnosis and immunosuppressive treatment are essential to optimize outcomes. FAU - Cosău, Diana Elena AU - Cosău DE AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Costache, Alexandru Dan AU - Costache AD AUID- ORCID: 0000-0001-8544-9904 AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Costache Enache, Irina Iuliana AU - Costache Enache II AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - "St. Spiridon" Emergency County Hospital, 700111 Iasi, Romania. FAU - Șerban, Ionela Lăcrămioara AU - Șerban IL AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. FAU - Petrariu, Luiza Andreea AU - Petrariu LA AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Pomîrleanu, Cristina AU - Pomîrleanu C AUID- ORCID: 0009-0001-2828-7758 AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Russu, Mara AU - Russu M AUID- ORCID: 0009-0009-8395-1808 AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Lăpuște, Vladia AU - Lăpuște V AUID- ORCID: 0009-0009-2516-1058 AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. FAU - Ancuța, Codrina AU - Ancuța C AUID- ORCID: 0000-0002-1777-8121 AD - Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania. AD - Clinical Rehabilitation Hospital, 700661 Iasi, Romania. LA - eng PT - Case Reports PT - Journal Article DEP - 20250726 PL - Switzerland TA - Reports (MDPI) JT - Reports (MDPI) JID - 101757880 PMC - PMC12372094 OTO - NOTNLM OT - PL-12 antibody OT - Sjögren’s syndrome OT - amyopathic myositis OT - antisynthetase syndrome OT - interstitial lung disease OT - overlap syndrome OT - rheumatoid arthritis COIS- The authors declare no conflicts of interest. EDAT- 2025/08/26 04:44 MHDA- 2025/08/26 04:45 PMCR- 2025/07/26 CRDT- 2025/08/22 06:46 PHST- 2025/06/15 00:00 [received] PHST- 2025/07/20 00:00 [revised] PHST- 2025/07/24 00:00 [accepted] PHST- 2025/08/26 04:45 [medline] PHST- 2025/08/26 04:44 [pubmed] PHST- 2025/08/22 06:46 [entrez] PHST- 2025/07/26 00:00 [pmc-release] AID - reports8030123 [pii] AID - reports-08-00123 [pii] AID - 10.3390/reports8030123 [doi] PST - epublish SO - Reports (MDPI). 2025 Jul 26;8(3):123. doi: 10.3390/reports8030123. PMID- 41800544 OWN - NLM STAT- Publisher LR - 20260309 IS - 1744-5086 (Electronic) IS - 0928-6586 (Linking) DP - 2026 Mar 9 TI - Systemic Conditions Associated with Primary Open-Angle Glaucoma and Normal Tension Glaucoma in a Large US Adult Cohort. PG - 1-10 LID - 10.1080/09286586.2026.2633607 [doi] AB - PURPOSE: To compare the patterns of association between two subtypes of open-angle glaucoma, primary open-angle glaucoma (POAG) and normal tension glaucoma (NTG), and systemic cardiovascular, neurologic, and autoimmune conditions in a large sample. MATERIALS AND METHODS: This cross-sectional study generated propensity score-matched cohorts of patients with and without the above systemic conditions, based on International Classification of Diseases (ICD) encounter diagnosis codes, using the TriNetX platform, containing data from >120 million patients. Patients were matched across age, sex, race, and ethnicity. Cohorts for autoimmune conditions were additionally matched for long-term corticosteroid use. Primary outcomes were odds of either POAG or NTG. OR threshold of significance was defined as >1.1 or <0.9 to improve signal-to-noise ratio. RESULTS: The only systemic conditions associated with POAG were autoimmune disorders, namely Raynaud's syndrome (OR = 1.15, 95% CI 1.08-1.23), giant cell arteritis (OR = 1.15, 95% CI 1.06-1.24), and Graves' disease (OR = 1.26, 95% CI 1.18-1.34). POAG was not associated with cardiovascular or neurologic conditions. NTG was positively associated with conditions across all three categories of systemic conditions, with stronger positive associations with each condition compared to POAG. CONCLUSION: POAG was not associated with cardiovascular or neurological conditions in a very large, propensity score-matched sample, but was positively associated with certain autoimmune conditions. NTG was positively associated with conditions across multiple organ systems. These findings indicate that both glaucoma subtypes likely have multifactorial etiologies of disease, one component of which may be auto-inflammatory. FAU - Joo, Julia H AU - Joo JH AD - Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA. FAU - Talcott, Katherine E AU - Talcott KE AD - Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA. AD - Department of Ophthalmology, Cleveland Clinic Cole Eye Institute, Cleveland, OH, USA. FAU - Singh, Rishi P AU - Singh RP AD - Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA. AD - Department of Ophthalmology, Cleveland Clinic Cole Eye Institute, Cleveland, OH, USA. AD - Cleveland Clinic Martin Hospitals, Cleveland Clinic Florida, Stuart, FL, USA. FAU - Kaelber, David C AU - Kaelber DC AD - School of Medicine, Case Western Reserve University, Cleveland, OH, USA. AD - Departments of Internal Medicine, Pediatrics, and Population and Quantitative Health Sciences, Case Western Reserve University, Cleveland, OH, USA. AD - The Center for Clinical Informatics Research and Education, The MetroHealth System, Cleveland, OH, USA. FAU - Li, Ang AU - Li A AD - Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA. AD - Department of Ophthalmology, Cleveland Clinic Cole Eye Institute, Cleveland, OH, USA. LA - eng PT - Journal Article DEP - 20260309 PL - England TA - Ophthalmic Epidemiol JT - Ophthalmic epidemiology JID - 9435674 SB - IM OTO - NOTNLM OT - Autoimmune OT - TriNetX OT - cardiovascular OT - neurologic OT - normal tension glaucoma OT - primary open-angle glaucoma EDAT- 2026/03/09 13:42 MHDA- 2026/03/09 13:42 CRDT- 2026/03/09 06:43 PHST- 2026/03/09 13:42 [medline] PHST- 2026/03/09 13:42 [pubmed] PHST- 2026/03/09 06:43 [entrez] AID - 10.1080/09286586.2026.2633607 [doi] PST - aheadofprint SO - Ophthalmic Epidemiol. 2026 Mar 9:1-10. doi: 10.1080/09286586.2026.2633607. PMID- 36210140 OWN - NLM STAT- MEDLINE DCOM- 20221011 LR - 20221011 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 18 IP - 8 DP - 2022 Oct TI - Correlation Between Quantitative Anti-dsDNA Levels with Severity of Proteinuria in Systemic Lupus Erythematosus Patients. PG - 464-468 LID - S2173-5743(22)00140-X [pii] LID - 10.1016/j.reumae.2021.06.005 [doi] AB - OBJECTIVE: To evaluate the correlation of quantitative anti-dsDNA level with proteinuria levels in patients with lupus nephritis in a tertiary care hospital. STUDY DESIGN: In this prospective cross-sectional study, 76 patients of newly diagnosed SLE coming to Fatima Memorial Hospital were included in the study period between January 2020 to June 2020. Demographic data such as age, gender, lupus manifestations such as serositis, arthritis, mucocutaneous disease, and neuropsychiatric manifestations were recorded. Quantitative anti-dsDNA was measured by enzyme-linked immunosorbent assay and proteinuria was estimated by 24h urinary protein collection. Data was analyzed by SPSS 23. Association between categorical variables was assessed using chi-square test. For comparison of categorical independent and continuous dependent variable t-test or Mann-Whitney U test was applied. RESULTS: The median age of the cohort was 29 (with inter quartile range - IQR - of 13) years. The female gender comprised of 68 (89.4%) of the cohort population. The median anti-dsDNA level was 54.9 (183.6 IQR) IU, and baseline proteinuria of the cohort was 520mg/dL (1.49 IQR). There was a significant association of anti-dsDNA level with systemic features such as arthritis (p=<0.01), serositis (p=<0.01) and, Raynaud's phenomenon (p=<0.01). NPSLE and mucocutaneous features did not show statistically significant association (p=0.91 and 0.14 respectively). Baseline anti-dsDNA showed a statistically significant correlation with baseline proteinuria levels (p=<0.01). CONCLUSION: Quantitative anti-dsDNA is directly correlated with nephritis measured as proteinuria, and can be detected even before organ involvement. Hence, it can determine disease course and guide early treatment. CI - Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Asif, Sadia AU - Asif S AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. FAU - Khan, Asadullah AU - Khan A AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. FAU - Zahoor, Sarmad AU - Zahoor S AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. Electronic address: drsarmadzahoor@gmail.com. FAU - Lashari, Naveed AU - Lashari N AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. FAU - Haroon, Muhammad AU - Haroon M AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. FAU - Khanum, Afshan AU - Khanum A AD - Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan; Department of Internal Medicine, KEMU, Mayo Hospital, Lahore, Pakistan. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 RN - 0 (Antibodies, Antinuclear) RN - 9007-49-2 (DNA) SB - IM MH - Adolescent MH - Antibodies, Antinuclear MH - *Arthritis MH - Cross-Sectional Studies MH - DNA MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - Prospective Studies MH - Proteinuria/etiology MH - *Serositis OTO - NOTNLM OT - Anti-dsDNA OT - Lupus eritematoso sistémico OT - Nefritis OT - Nephritis OT - Systemic lupus erythematosus EDAT- 2022/10/10 06:00 MHDA- 2022/10/12 06:00 CRDT- 2022/10/09 21:04 PHST- 2020/10/02 00:00 [received] PHST- 2021/06/24 00:00 [accepted] PHST- 2022/10/09 21:04 [entrez] PHST- 2022/10/10 06:00 [pubmed] PHST- 2022/10/12 06:00 [medline] AID - S2173-5743(22)00140-X [pii] AID - 10.1016/j.reumae.2021.06.005 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2022 Oct;18(8):464-468. doi: 10.1016/j.reumae.2021.06.005. PMID- 38813136 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240531 IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 11 DP - 2022 TI - Case Report: Extensive digital gangrene as a primary manifestation of late-onset systemic lupus erythematosus. PG - 922 LID - 10.12688/f1000research.124225.2 [doi] LID - 922 AB - Background: Digital gangrene is a rare but serious complication of systemic lupus erythematosus (SLE). It occurs usually in middle-aged patients with longer disease duration. Case: Herein we report the case of a 56-year-old man (with no history suggestive of Raynaud's phenomenon, diabetes mellitus, smoking, trauma, infection, or chemical exposure), who presented with SLE and digital gangrene was among the first signs. He presented with a one-month history of joint pain, hair loss, photosensitivity, mouth ulcers, malar rash, dyspnea, and digital pain. Physical examination revealed painful and diffuse erythematous skin lesions in the extremities and back, as well as cyanosis in the fingers. We noted lymphocytopenia (600 cells/mm (3)), and an elevated C-reactive protein (15.1 mg/l) on laboratory tests. Immunological tests were positive for antinuclear antibodies (ANA) with Title 1:400. Pulmonary computed tomography revealed pulmonary fibrosis, and pulmonary function tests revealed the restrictive pulmonary disease. Diagnosis of SLE with lung involvement was retained. The immunological assessment in search of elements in favor of a vascular origin of the patient's skin lesions was negative. Treatment was initiated with 200 mg/day hydroxychloroquine. For dermal and pulmonary involvement, intravenous (IV) pulse therapy was used with methylprednisolone (1,000 mg/d for three consecutive days monthly) and cyclophosphamide (1 g/month). Calcium blocking agents were also prescribed. However, the lesions did not improve. The patient was given two infusions of rituximab (1 g) at a 14-day interval with a marked improvement ofthe majority of vasculitis lesions, and a partial improvement of dyspnea. Conclusions: Digital gangrene is a rare complication of late-onset SLE, especially as a primary manifestation. CI - Copyright: © 2022 Boussaid S et al. FAU - Boussaid, Soumaya AU - Boussaid S AUID- ORCID: 0000-0002-9185-1710 AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Ben Majdouba, Marouene AU - Ben Majdouba M AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Rekik, Sonia AU - Rekik S AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Jemmali, Samia AU - Jemmali S AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Zouaoui, Khaoula AU - Zouaoui K AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Rahmouni, Safa AU - Rahmouni S AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Sahli, Hela AU - Sahli H AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Research unit LR 05 SP 01, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. FAU - Elleuch, Mohamed AU - Elleuch M AD - Rheumatology Department, La Rabta Hospital, La Rabta Jebbari, Tunis, 1007, Tunisia. AD - Faculty of Medicine of Tunis, University Tunis el Manar, La Rabta Jebbari, Tunis, 1007, Tunisia. LA - eng PT - Case Reports PT - Journal Article DEP - 20220907 PL - England TA - F1000Res JT - F1000Research JID - 101594320 SB - IM PMC - PMC11134139 OTO - NOTNLM OT - Systemic lupus erythematosus OT - digital gangrene OT - vasculitis COIS- No competing interests were disclosed. EDAT- 2022/09/07 00:00 MHDA- 2022/09/07 00:01 PMCR- 2022/09/07 CRDT- 2024/05/30 03:59 PHST- 2022/09/05 00:00 [accepted] PHST- 2022/09/07 00:01 [medline] PHST- 2022/09/07 00:00 [pubmed] PHST- 2024/05/30 03:59 [entrez] PHST- 2022/09/07 00:00 [pmc-release] AID - 10.12688/f1000research.124225.2 [doi] PST - epublish SO - F1000Res. 2022 Sep 7;11:922. doi: 10.12688/f1000research.124225.2. eCollection 2022. PMID- 34251311 OWN - NLM STAT- MEDLINE DCOM- 20220301 LR - 20220405 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 40 IP - 2 DP - 2022 Feb TI - A retrospective cohort study in Chinese patients with adult polymyositis and dermatomyositis: risk of comorbidities and subclassification using machine learning. PG - 224-236 LID - 10.55563/clinexprheumatol/i2xeao [doi] AB - OBJECTIVES: To identify the risk factors in Chinese patients with adult polymyositis and dermatomyositis for their comorbidities and explore a subclassification system. METHODS: Clinical records of 397 patients with idiopathic inflammatory myopathies were retrospectively reviewed. Logistic regression was used to identify potential risk factors for interstitial lung disease (ILD), other rheumatic diseases, and malignancy after bivariate analysis. Hierarchical clustering and decisional tree were utilised to identify subgroups and explore a subclassification system. RESULTS: A total of 119 polymyositis and 191 dermatomyositis patients were included. Anti-PM/Scl, anti-Ro52, anti-aminoacyl-tRNA synthetase and anti-MDA5 (adjusted odds ratios (AOR)=4.779, 1.917, 5.092 and 7.714 respectively) antibodies were risks (p<0.05), whereas overlapping malignancy was protective (AOR=0.107; p=0.002) for ILD across polymyositis, dermatomyositis and the total group. In subgroup models, Raynaud's phenomenon, arthralgia and semi-quantitative anti-nuclear antibody (AOR=51.233, 4.261, 3.047 respectively) were risks for other overlapping rheumatic diseases (p<0.05). For overlapping malignancy, male and anti-TIF1γ antibodies (AOR=2.533, 16.949) were risks (p<0.05), whereas disease duration and combination of ILD (AOR=0.954, 0.106) were protective in the total group (p<0.05); while anti-NXP2 antibodies were identified as risk factors (AOR=73.152; p=0.038) in polymyositis. Hierarchical clustering suggested a subclassification with 6 subgroups: malignancy overlapping dermatomyositis, classical dermatomyositis, polymyositis with severe muscle involvement, dermatomyositis with ILD, polymyositis with ILD, and overlapping of myositis with other rheumatic diseases. CONCLUSIONS: Accompanying ILD, other rheumatic diseases and malignancy are strongly associated with clinical manifestation and myositis-specific or myositis-associated autoantibodies among Chinese polymyositis and dermatomyositis patients. The subclassification system proposed a more precise phenotype defining toward stratified treatments. FAU - Zhu, Junqing AU - Zhu J AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Wu, Lisheng AU - Wu L AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou; and Department of Internal Medicine of Traditional Chinese Medicine, School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, China. FAU - Zhou, Yi AU - Zhou Y AD - Department of Obstetrics, Guangdong Women and Children Hospital, Guangzhou, China. FAU - Wang, Ran AU - Wang R AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Chen, Shixian AU - Chen S AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Zhao, Jinjun AU - Zhao J AD - Department of Rheumatology, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Yu, Shenyi AU - Yu S AD - Department of Rheumatology, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Zheng, Songyuan AU - Zheng S AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Xiao, Fei AU - Xiao F AD - Department of Rheumatic & TCM Medical Center, Nanfang Hospital, Southern Medical University, Guangzhou; and Department of Internal Medicine of Traditional Chinese Medicine, School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, China. FAU - Ren, Hao AU - Ren H AD - Department of Rheumatology, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Yang, Min AU - Yang M AD - Department of Rheumatology, Nanfang Hospital, Southern Medical University, Guangzhou, China. FAU - Li, Juan AU - Li J AD - Department of Internal Medicine of Traditional Chinese Medicine, School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, China. lijuan@smu.edu.cn. LA - eng PT - Journal Article DEP - 20210626 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - China/epidemiology MH - *Dermatomyositis/complications/diagnosis/epidemiology MH - Humans MH - Machine Learning MH - Male MH - *Polymyositis MH - Retrospective Studies EDAT- 2021/07/13 06:00 MHDA- 2022/03/03 06:00 CRDT- 2021/07/12 12:22 PHST- 2020/11/17 00:00 [received] PHST- 2021/01/25 00:00 [accepted] PHST- 2021/07/13 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2021/07/12 12:22 [entrez] AID - 16689 [pii] AID - 10.55563/clinexprheumatol/i2xeao [doi] PST - ppublish SO - Clin Exp Rheumatol. 2022 Feb;40(2):224-236. doi: 10.55563/clinexprheumatol/i2xeao. Epub 2021 Jun 26. PMID- 30088450 OWN - NLM STAT- MEDLINE DCOM- 20190806 LR - 20190806 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 15 IP - 2 DP - 2019 TI - An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes? PG - 172-175 LID - 10.2174/1573397114666180808091621 [doi] AB - BACKGROUND: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmune disease. Many conditions mimic the presentation of SSc, especially skin thickening and fibrosis. One of these conditions is scleredema, a rare collagen and mucin deposition disorder which was found to be associated with diabetes mellitus, streptococcal infection or monoclonal gammopathy. CASE PRESENTATION: A 55 years old female presented with insidious onset and progressive course of diffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both hands and feet of 6 years' duration associated with arthralgia, dysphagia and dyspnea on exertion of 1- year duration. There was no history of Raynaud's phenomenon. Erythrocyte sedimentation rate was 100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonary function tests showed restrictive pattern and high-resolution computed tomography showed interstitial lung fibrosis. Patient was not fulfilling the American collage of rheumatology/European League Against Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histological appearance of scleredema. Investigations were done for disease association with scleredema. The patient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixation and bone marrow biopsy were done, and the patient was diagnosed as scleredema associated with immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib, cyclophosphamide, and dexamethasone was started with marked clinical and hematological improvement. CONCLUSION: Many conditions mimic SSc including scleredema, which may be the initial presentation of multiple myeloma. Rheumatologists and dermatologists should be able to recognize these conditions to provide the suitable management and follow-up for these patients. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Ibrahim, Rehab Ali AU - Ibrahim RA AD - Physical Medicine, Rheumatology and Rehabilitation Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt. FAU - Abdalla, Nour El-Hoda Hussien AU - Abdalla NEH AD - Internal Medicine, Hematology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt. FAU - Shabaan, Engy Amr Husssein AU - Shabaan EAH AD - Dermatology, Venereology and Andrology Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt. FAU - Mostafa, Noha Bassiouny Hassan AU - Mostafa NBH AD - Department of Clinical Pathology, Faculty of Medicine, Ain Shams University, El-Abaseya, Egypt. LA - eng PT - Case Reports PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Multiple Myeloma/*complications/diagnosis MH - Scleredema Adultorum/*diagnosis/*etiology/*pathology MH - Scleroderma, Systemic/diagnosis/pathology OTO - NOTNLM OT - Scleroderma OT - fibrosis OT - monoclonal gammopathy OT - multiple myeloma OT - scleredema OT - scleroderma mimics. EDAT- 2018/08/09 06:00 MHDA- 2019/08/07 06:00 CRDT- 2018/08/09 06:00 PHST- 2018/04/18 00:00 [received] PHST- 2018/08/03 00:00 [revised] PHST- 2018/08/06 00:00 [accepted] PHST- 2018/08/09 06:00 [pubmed] PHST- 2019/08/07 06:00 [medline] PHST- 2018/08/09 06:00 [entrez] AID - CRR-EPUB-92244 [pii] AID - 10.2174/1573397114666180808091621 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2019;15(2):172-175. doi: 10.2174/1573397114666180808091621. PMID- 23375620 OWN - NLM STAT- MEDLINE DCOM- 20140211 LR - 20130617 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 24 IP - 5 DP - 2013 Jul TI - Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome. PG - 474-9 LID - S0953-6205(13)00007-1 [pii] LID - 10.1016/j.ejim.2013.01.002 [doi] AB - BACKGROUND: The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). METHODS: The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. RESULTS: Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). CONCLUSION: Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan. CI - Copyright © 2013 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. FAU - Marie, I AU - Marie I AD - Department of Internal Medicine, CHU Rouen, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France. isabelle.marie@chu-rouen.fr FAU - Josse, S AU - Josse S FAU - Decaux, O AU - Decaux O FAU - Diot, E AU - Diot E FAU - Landron, C AU - Landron C FAU - Roblot, P AU - Roblot P FAU - Jouneau, S AU - Jouneau S FAU - Hatron, P Y AU - Hatron PY FAU - Hachulla, E AU - Hachulla E FAU - Vittecoq, O AU - Vittecoq O FAU - Menard, J-F AU - Menard JF FAU - Jouen, F AU - Jouen F FAU - Dominique, S AU - Dominique S LA - eng PT - Journal Article DEP - 20130201 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Autoantibodies) RN - EC 6.1.1.3 (Threonine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/*blood MH - Dermatomyositis/immunology/mortality MH - Female MH - Humans MH - Lung Diseases, Interstitial/*immunology/mortality MH - Male MH - Middle Aged MH - Myositis/*immunology/mortality MH - Polymyositis/immunology/mortality MH - Retrospective Studies MH - Threonine-tRNA Ligase/*immunology EDAT- 2013/02/05 06:00 MHDA- 2014/02/12 06:00 CRDT- 2013/02/05 06:00 PHST- 2012/09/17 00:00 [received] PHST- 2012/12/31 00:00 [revised] PHST- 2013/01/03 00:00 [accepted] PHST- 2013/02/05 06:00 [entrez] PHST- 2013/02/05 06:00 [pubmed] PHST- 2014/02/12 06:00 [medline] AID - S0953-6205(13)00007-1 [pii] AID - 10.1016/j.ejim.2013.01.002 [doi] PST - ppublish SO - Eur J Intern Med. 2013 Jul;24(5):474-9. doi: 10.1016/j.ejim.2013.01.002. Epub 2013 Feb 1. PMID- 21593063 OWN - NLM STAT- MEDLINE DCOM- 20111228 LR - 20151119 IS - 1753-4666 (Electronic) IS - 1753-4658 (Linking) VI - 5 IP - 5 DP - 2011 Oct TI - Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids. PG - 299-304 LID - 10.1177/1753465811407786 [doi] AB - We would like to report our experience of using rituximab in cyclophosphamide refractory, rapidly progressive interstitial lung disease (ILD) in a patient with limited scleroderma. A 40-year-old man presented with 10-week history of inflammatory polyarthritis, which responded to a short course of oral corticosteroids. However, 3 weeks later, he developed new onset of exertional dyspnoea. High-resolution CT of the thorax was suggestive of early ILD. Surgical lung biopsy showed features of fibrotic non-specific interstitial pneumonia. He was diagnosed with scleroderma on the basis of: presence of anticentromere antibodies, Raynaud's phenomenon, pulmonary fibrosis, digital oedema and hypomotility along with a dilated oesophagus. He was treated aggressively with pulse doses of corticosteroids and cyclophosphamide; however, his ILD continued to deteriorate. At this stage, he received rituximab (two pulses of 1 g each), which led to a gradual clinical improvement. Now, 12 months since his rituximab infusion, he walks 2 miles daily without any exertional dyspnoea. FAU - Haroon, Muhammad AU - Haroon M AD - Department of Rheumatology, Cork University Hospital, Cork, Ireland. mharoon301@hotmail.com FAU - McLaughlin, Patrick AU - McLaughlin P FAU - Henry, Michael AU - Henry M FAU - Harney, Sinead AU - Harney S LA - eng PT - Case Reports PT - Journal Article DEP - 20110518 PL - England TA - Ther Adv Respir Dis JT - Therapeutic advances in respiratory disease JID - 101316317 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (Glucocorticoids) RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - 8N3DW7272P (Cyclophosphamide) SB - IM MH - Adult MH - Antibodies, Monoclonal, Murine-Derived/administration & dosage/*therapeutic use MH - Cyclophosphamide/therapeutic use MH - Dose-Response Relationship, Drug MH - Drug Therapy, Combination MH - Follow-Up Studies MH - Glucocorticoids/administration & dosage/*therapeutic use MH - Humans MH - Immunologic Factors/administration & dosage/therapeutic use MH - Lung Diseases, Interstitial/diagnosis/*drug therapy/physiopathology MH - Male MH - Pulse Therapy, Drug MH - Rituximab MH - Scleroderma, Limited/diagnosis/*drug therapy/physiopathology MH - Treatment Outcome EDAT- 2011/05/20 06:00 MHDA- 2011/12/29 06:00 CRDT- 2011/05/20 06:00 PHST- 2011/05/20 06:00 [entrez] PHST- 2011/05/20 06:00 [pubmed] PHST- 2011/12/29 06:00 [medline] AID - 1753465811407786 [pii] AID - 10.1177/1753465811407786 [doi] PST - ppublish SO - Ther Adv Respir Dis. 2011 Oct;5(5):299-304. doi: 10.1177/1753465811407786. Epub 2011 May 18. PMID- 18203317 OWN - NLM STAT- MEDLINE DCOM- 20080506 LR - 20111201 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 2 DP - 2008 Feb TI - Polymorphisms in COL15 gene are not associated with systemic sclerosis. PG - 251-3 AB - OBJECTIVE: Systemic sclerosis (SSc) is marked by microvascular abnormalities leading to ischemic features such as Raynaud's phenomenon and fingertip ulcers. Digital ischemia in turn results in hypoxia, which is expected to drive compensatory angiogenesis; however, this phenomenon is deregulated in SSc. Vascular basement membrane (VBM) that consists of type IV, XV, and XVIII collagens supports the growth and survival of vascular endothelial cells and plays a key role in regulating angiogenesis. Recent gene expression analyses of skin tissue and dermal fibroblasts from patients with SSc revealed COL15 to be one of the significantly differentially regulated genes. We undertook an association study to explore the role of COL15 single-nucleotide polymorphisms (SNP) in SSc disease development. METHODS: Eleven SNP across COL15 were genotyped in a cohort of 175 UK Caucasian patients with SSc and 190 population-matched unrelated healthy subjects using 2 methods: TaqMan and SNaPshot. Statistical analysis was performed by Pearson's chi-square test and HelixTree software was utilized for haplotype analysis. RESULTS: No difference in genotype or allele frequencies were detected between patients with SSc and controls. None of the haplotype frequencies were found to differ between patients and controls. CONCLUSION: Failure to detect an association may reflect a true lack of association or could be a false-negative result arising as a result of low power of the study. Our study had sufficient power to detect an effect size of 2.1 (p = 0.05); however, larger patient cohorts may be needed for exclusion of COL15 from a possible candidacy in SSc. FAU - Pushpakom, Sudeep P AU - Pushpakom SP AD - National Genetics Reference Laboratory, Department of Medical Genetics, St. Mary's Hospital, Hathersage Road, Manchester M13 0JH, UK. sudeep.parameshwar@cmmc.nhs.uk FAU - Herrick, Ariane L AU - Herrick AL FAU - Kumar, Shant AU - Kumar S FAU - Worthington, Jane AU - Worthington J LA - eng GR - Arthritis Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (COL15A1 protein, human) RN - 9007-34-5 (Collagen) SB - IM MH - Case-Control Studies MH - Collagen/*genetics MH - Exons/genetics MH - Gene Frequency/genetics MH - Genetic Predisposition to Disease/genetics MH - Humans MH - Polymorphism, Single Nucleotide/*genetics MH - Promoter Regions, Genetic/genetics MH - Scleroderma, Systemic/*genetics EDAT- 2008/01/19 09:00 MHDA- 2008/05/07 09:00 CRDT- 2008/01/19 09:00 PHST- 2008/01/19 09:00 [pubmed] PHST- 2008/05/07 09:00 [medline] PHST- 2008/01/19 09:00 [entrez] AID - 08/13/0113 [pii] PST - ppublish SO - J Rheumatol. 2008 Feb;35(2):251-3. Epub 2008 Jan 15. PMID- 38880552 OWN - NLM STAT- MEDLINE DCOM- 20240616 LR - 20240616 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 20 IP - 5 DP - 2024 May TI - Cardiopulmonary phenotype in systemic sclerosis associated pulmonary hypertension. PG - 243-248 LID - S2173-5743(24)00064-9 [pii] LID - 10.1016/j.reumae.2024.01.006 [doi] AB - INTRODUCTION: Pulmonary hypertension (PH) associated with systemic sclerosis (SSc) increases morbidity and mortality. Cardiopulmonary comorbidities, as per the 2021 PH consensus, play a role in the choice of therapy between monotherapy and combination therapy. METHODS: A cross-sectional study was conducted in patients with SSc based on the 2013 ACR/EULAR criteria or very early disease (VEDOSS 2011). PH was considered if they met the following criteria: pulmonary artery systolic pressure (PASP)>39mmHg or peak tricuspid regurgitation velocity (PTRV)>3.4m/s, PASP between 33 and 39mmHg or PTRV between 2.9 and 3.4m/s plus two additional findings suggestive of PH. PH was classified as type 2 if LVEF<50% or moderate to severe diastolic dysfunction was present; type 3 if extensive interstitial disease on tomography>20% or forced vital capacity (FVC)<75%; type 4 if abnormalities related to embolism were detected on scintigraphy or tomography. If patients did not meet these criteria, they were classified as type 1 PH. Complete data on cardiopulmonary risk factors and other factors were required. The frequency of these factors in the population and differences between groups based on risk factors were estimated. RESULTS: A total of 228 patients were selected. Three had type 2 PH, 24 had type 3, and 40 had type 1 PH, with the majority (75%) having at least one cardiopulmonary risk factor, and 47.5% having more than one. Mild diastolic dysfunction (25%) and hypertension (35%) were the most prevalent. In the type 1 PH group, those with risk factors experienced an increase in the number of years with Raynaud's phenomenon, anticentromere antibodies, and gastrointestinal symptoms (p<0.05). CONCLUSION: In patients with PH, 75% have one, and 45% have two or more risk factors. CI - Copyright © 2024 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Cajas Santana, Luis Javier AU - Cajas Santana LJ AD - Hospital Universitario Nacional de Colombia, Colombia; Universidad Nacional de Colombia, Colombia. Electronic address: ljaviercs.77@hotmail.com. FAU - Correa Giraldo, Alejandro AU - Correa Giraldo A AD - Hospital Universitario Nacional de Colombia, Colombia; Universidad Nacional de Colombia, Colombia. FAU - Torres, Maria Carolina AU - Torres MC AD - Hospital Universitario Nacional de Colombia, Colombia; Universidad Nacional de Colombia, Colombia. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications MH - Cross-Sectional Studies MH - Female MH - *Hypertension, Pulmonary/etiology MH - Male MH - Middle Aged MH - *Phenotype MH - Adult MH - Aged MH - Risk Factors OTO - NOTNLM OT - Eclerosis sistémica OT - Factores de riesgo cardiovasculares OT - Heart disease risk factors OT - Hipertensión pulmonar OT - Pulmonary hypertension OT - Systemic sclerosis EDAT- 2024/06/17 00:42 MHDA- 2024/06/17 00:43 CRDT- 2024/06/16 20:58 PHST- 2023/09/08 00:00 [received] PHST- 2024/01/23 00:00 [revised] PHST- 2024/01/25 00:00 [accepted] PHST- 2024/06/17 00:43 [medline] PHST- 2024/06/17 00:42 [pubmed] PHST- 2024/06/16 20:58 [entrez] AID - S2173-5743(24)00064-9 [pii] AID - 10.1016/j.reumae.2024.01.006 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2024 May;20(5):243-248. doi: 10.1016/j.reumae.2024.01.006. PMID- 35203643 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220401 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 10 IP - 2 DP - 2022 Feb 13 TI - Treatment and Systemic Sclerosis Interstitial Lung Disease Outcome: The Overweight Paradox. LID - 10.3390/biomedicines10020434 [doi] LID - 434 AB - (1) Background: Systemic sclerosis (SSc) is frequently associated with interstitial lung diseases (ILDs). The progressive form of SSc-ILD often limits patient survival. The aim of our study is to evaluate the clinical characteristics and predictors of lung function changes in SSc-ILD patients treated in a real-world setting. (2) Methods: All SSc-ILD cases previously confirmed by rheumatologists and a multidisciplinary ILD team between January 2017 and June 2019 were included (n = 54). The detailed medical history, clinical parameters and HRCT were analyzed. The longitudinal follow-up for pulmonary symptoms, functional parameters and treatment were performed for at least 2 years in no treatment, immunosuppression and biological treatment subgroups. (3) Results: In SSc-ILD patients (age 58.7 ± 13.3 years, 87.0% women), the main symptoms included dyspnea, cough, crackles and the Raynaud's phenomenon. The functional decline was most prominent in untreated patients, and a normal body mass index (BMI < 25 kg/m(2)) was associated with a significant risk of deterioration. The majority of patients improved or were stable during follow-up. The progressive fibrosing-ILD criteria were met by 15 patients, the highest proportion being in the untreated subgroup. (4) Conclusions: SSc-ILD patients who are overweight are at a lower risk of the functional decline and progressive phenotype especially affecting untreated patients. The close monitoring of lung involvement and a regular BMI measurement are advised and early treatment interventions are encouraged. FAU - Nagy, Alexandra AU - Nagy A AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Palmer, Erik AU - Palmer E AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Polivka, Lorinc AU - Polivka L AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Eszes, Noemi AU - Eszes N AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Vincze, Krisztina AU - Vincze K AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Barczi, Eniko AU - Barczi E AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Bohacs, Aniko AU - Bohacs A AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. FAU - Tarnoki, Adam Domonkos AU - Tarnoki AD AUID- ORCID: 0000-0001-5909-3780 AD - Medical Imaging Centre, Semmelweis University, 1082 Budapest, Hungary. FAU - Tarnoki, David Laszlo AU - Tarnoki DL AUID- ORCID: 0000-0002-7001-7647 AD - Medical Imaging Centre, Semmelweis University, 1082 Budapest, Hungary. FAU - Nagy, György AU - Nagy G AD - Department of Genetics, Cell- and Immunobiology, Semmelweis University, 1082 Budapest, Hungary. AD - Department of Rheumatology and Clinical Immunology, Department of Internal Medicine and Oncology, Semmelweis University, 1083 Budapest, Hungary. FAU - Kiss, Emese AU - Kiss E AD - Department of Clinical Immunology, Adult and Pediatric Rheumatology, National Institute of National Institute of Locomotor Diseases and Disabilities, 1023 Budapest, Hungary. AD - 3rd Department of Internal Medicine and Haematology, Semmelweis University, 1088 Budapest, Hungary. FAU - Maurovich-Horvat, Pal AU - Maurovich-Horvat P AD - Medical Imaging Centre, Semmelweis University, 1082 Budapest, Hungary. FAU - Müller, Veronika AU - Müller V AUID- ORCID: 0000-0002-1398-3187 AD - Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary. LA - eng PT - Journal Article DEP - 20220213 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC8962393 OTO - NOTNLM OT - body mass index OT - interstitial lung disease OT - lung function OT - progressive fibrosing interstitial lung disease OT - systemic sclerosis OT - treatment COIS- The authors declare no conflict of interest. EDAT- 2022/02/26 06:00 MHDA- 2022/02/26 06:01 PMCR- 2022/02/13 CRDT- 2022/02/25 01:02 PHST- 2021/12/31 00:00 [received] PHST- 2022/02/04 00:00 [revised] PHST- 2022/02/11 00:00 [accepted] PHST- 2022/02/25 01:02 [entrez] PHST- 2022/02/26 06:00 [pubmed] PHST- 2022/02/26 06:01 [medline] PHST- 2022/02/13 00:00 [pmc-release] AID - biomedicines10020434 [pii] AID - biomedicines-10-00434 [pii] AID - 10.3390/biomedicines10020434 [doi] PST - epublish SO - Biomedicines. 2022 Feb 13;10(2):434. doi: 10.3390/biomedicines10020434. PMID- 40126224 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250326 IS - 2038-8322 (Print) IS - 2038-8330 (Electronic) IS - 2038-8322 (Linking) VI - 17 IP - 2 DP - 2025 Mar 21 TI - Avascular Necrosis of the Femoral Head in Patients with Antiphospholipid Syndrome: A Case Series. LID - 10.3390/hematolrep17020015 [doi] LID - 15 AB - Background/Objectives: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis or obstetric complications and the laboratory detection of antiphospholipid antibodies. Although vascular thrombosis is the main manifestation of the disease, other rarer complications have also been described. Avascular necrosis (AN) is considered a rare manifestation of APS. The aim of our case series is to study patients with APS and AN. Methods: A retrospective study was performed on 80 patients diagnosed with APS. Results: AN was observed in 3 patients out of 80 diagnosed with APS. AN of the femoral head was observed in all cases. Case (1): A 54-year-old woman presented due to multiple ischemic infarctions in the brain, as detected in magnetic resonance imaging of the brain, Raynaud's phenomenon, and AN of the femoral head. In laboratory testing, a prolongation of activated partial thromboplastin time was recorded. A heterozygous mutation was also found in the gene MTHFR C677T, and the patients was positive for lupus anticoagulant (LA). The patient was given clopidogrel and acenocoumarol. Case (2): A 52-year-old man was diagnosed with APS, based on the clinical presentation (stroke) and positivity for LA and anti-β2GPI (anti-β2 glycoprotein I antibody). In his medical history, episodes of vertigo and an episode of AN of the femoral head 2 years ago were described. Case (3): A woman aged 43 years presented due to AN of the femoral head. Due to suspected APS, immunological testing was performed, and positivity for LA and IgM anticardiolipin antibodies was detected. She was treated with acenocoumarol. Conclusions: AN is a rare clinical manifestation of APS, which may precede the diagnosis of APS for many years. FAU - Evangelidis, Paschalis AU - Evangelidis P AUID- ORCID: 0000-0003-1783-5111 AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Gavriilaki, Eleni AU - Gavriilaki E AUID- ORCID: 0000-0002-8883-8208 AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Kotsiou, Nikolaos AU - Kotsiou N AUID- ORCID: 0009-0004-2919-5179 AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Ntova, Zacharo AU - Ntova Z AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Kalmoukos, Panagiotis AU - Kalmoukos P AUID- ORCID: 0009-0000-7124-2785 AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Papadopoulou, Theodosia AU - Papadopoulou T AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Chissan, Sofia AU - Chissan S AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. FAU - Vakalopoulou, Sofia AU - Vakalopoulou S AD - Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocration Hospital, 54642 Thessaloniki, Greece. LA - eng PT - Journal Article DEP - 20250321 PL - Switzerland TA - Hematol Rep JT - Hematology reports JID - 101556723 PMC - PMC11932201 OTO - NOTNLM OT - antiphospholipid syndrome OT - avascular necrosis OT - stroke OT - thrombophilia OT - thrombosis COIS- E.G. has consulted for Alexion, Sobi, and Omeros Pharmaceuticals, Inc. The remaining authors declare no conflicts of interest. EDAT- 2025/03/24 12:45 MHDA- 2025/03/24 12:46 PMCR- 2025/03/21 CRDT- 2025/03/24 10:33 PHST- 2024/12/03 00:00 [received] PHST- 2025/03/18 00:00 [revised] PHST- 2025/03/19 00:00 [accepted] PHST- 2025/03/24 12:46 [medline] PHST- 2025/03/24 12:45 [pubmed] PHST- 2025/03/24 10:33 [entrez] PHST- 2025/03/21 00:00 [pmc-release] AID - hematolrep17020015 [pii] AID - hematolrep-17-00015 [pii] AID - 10.3390/hematolrep17020015 [doi] PST - epublish SO - Hematol Rep. 2025 Mar 21;17(2):15. doi: 10.3390/hematolrep17020015. PMID- 36200933 OWN - NLM STAT- MEDLINE DCOM- 20221222 LR - 20221222 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 40 IP - 12 DP - 2022 Dec TI - Clinical and laboratory findings of primary Sjögren's syndrome patients without sicca symptoms. PG - 2298-2302 LID - 10.55563/clinexprheumatol/gqvyus [doi] AB - OBJECTIVES: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by oral and eye dryness. A minority of patients can present without dryness but studies on their clinico-laboratory manifestations are scarce. Our purpose was to describe the clinical phenotype of pSS patients lacking sicca symptoms. METHODS: From a total of 1738 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those who presented without sicca symptoms were identified (non-dryness group). Their medical data was collected and compared with 2 control groups: a) the remaining unmatched sicca pSS patients with both oral and eye dryness (unmatched dryness group) and b) matched sicca pSS patients according to age, sex, and disease duration, in 1:2 ratio (matched dryness group). RESULTS: Thirty-eight (2.19%) patients lacked sicca manifestations presenting mainly with arthralgias (47%), parotid enlargement (24%), Raynaud's phenomenon (11%) and persistent lymphadenopathy (11%) that led them to be evaluated for pSS. Non-dryness pSS patients were younger than the unmatched sicca controls, displaying a higher frequency of anti-Ro/SSA antibodies (100% vs. 79.7%, p<0.001), ANA positivity (100% vs. 90.4%, p<0.001), neutropenia (20.8% vs. 7.5%, p=0.04) and thrombocytopenia (13.8% vs. 4.2%, p=0.04). They also had lower frequency of positive ocular tests compared to both unmatched and matched dryness patients. No differences were found between non-dryness pSS patients and both control groups regarding focus score or any other extraglandular manifestation. CONCLUSIONS: pSS patients without sicca complaints constitute a distinct phenotype involving younger patients, sharing common immunopathologic mechanisms with typical sicca patients. FAU - Chatzis, Loukas G AU - Chatzis LG AD - Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece. lukechatzis@gmail.com. FAU - Koulouri, Vasiliki AU - Koulouri V AD - Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Baldini, Chiara AU - Baldini C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Pezoulas, Vasilis C AU - Pezoulas VC AD - Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Greece. FAU - Voulgari, Paraskevi V AU - Voulgari PV AD - Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Greece. FAU - Skopouli, Fotini N AU - Skopouli FN AD - Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Greece. FAU - Fotiadis, Dimitrios I AU - Fotiadis DI AD - Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, and Department of Biomedical Research, Institute of Molecular Biology and Biotechnology, FORTH, Ioannina, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece. FAU - Goules, Andreas V AU - Goules AV AD - Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece. LA - eng PT - Journal Article DEP - 20221005 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - *Sjogren's Syndrome/complications/diagnosis MH - *Dry Eye Syndromes EDAT- 2022/10/07 06:00 MHDA- 2022/12/23 06:00 CRDT- 2022/10/06 10:22 PHST- 2022/07/04 00:00 [received] PHST- 2022/08/26 00:00 [accepted] PHST- 2022/10/07 06:00 [pubmed] PHST- 2022/12/23 06:00 [medline] PHST- 2022/10/06 10:22 [entrez] AID - 18945 [pii] AID - 10.55563/clinexprheumatol/gqvyus [doi] PST - ppublish SO - Clin Exp Rheumatol. 2022 Dec;40(12):2298-2302. doi: 10.55563/clinexprheumatol/gqvyus. Epub 2022 Oct 5. PMID- 35444873 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220514 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 3 DP - 2022 Mar TI - Systemic Sclerosis (SSc) After COVID-19: A Case Report. PG - e23179 LID - 10.7759/cureus.23179 [doi] LID - e23179 AB - Since the start of the global pandemic caused by coronavirus disease 2019 (COVID-19), there have been numerous reports of autoimmune and rheumatological disorders developing after infection with SARS-CoV-2. To date, there has been only one reported case of systemic sclerosis (SSc) developing after SARS-CoV-2 infection. Here, we present another case of SSc developing after infection with SARS-CoV-2. A 48-year-old female with past medical history of anxiety and depression presented to the rheumatology clinic after being referred for further evaluation of abnormal labs, Raynaud's phenomenon, and other concerning symptoms. Shortly after hospitalization for COVID-19 pneumonia, she began experiencing symptoms that included fatigue, xerostomia, dysphagia, bilateral lower extremity weakness, dyspnea with exertion, unintentional weight loss, and diffuse skin hyperpigmentation. Labs ordered shortly before presentation were significant for antinuclear antibody (ANA) titer > 1:1280. Physical exam was remarkable for puffy fingers, sclerodactyly of the fingers, diffuse skin hyperpigmentation, and abnormal nailfold capillaries. Anti-RNA polymerase III, anti-Scl-70, anti-centromere, anti-SSA, anti-SSB, anti-Smith, and anti-Smith/RNP antibodies were all negative. BNP, aldolase, and serum myoglobin levels were within normal limits while creatine phosphokinase level was slightly decreased. Pulmonary function testing showed reduced diffusion capacity with normal lung mechanics and volumes. High-resolution CT scan of the chest showed interstitial lung disease, with findings suggestive of nonspecific interstitial pneumonia. Transthoracic echocardiogram showed mild elevation of right ventricular systolic pressure, but pulmonary hypertension was not found on right heart catheterization. Esophagogastroduodenoscopy (EGD) with biopsy performed for evaluation of esophageal dysphagia showed sliding hiatal hernia, irregular Z-line, and gastric hyperemia. Biopsy of the distal esophagus was consistent with Barrett's esophagus. The patient was diagnosed with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for SSc. She is currently being treated with mycophenolate mofetil, amlodipine, methotrexate, and prednisone. CI - Copyright © 2022, Chandra et al. FAU - Chandra, Arjun AU - Chandra A AD - Internal Medicine, University of Toledo, Toledo, USA. FAU - Kahaleh, Bashar AU - Kahaleh B AD - Rheumatology, University of Toledo, Toledo, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20220315 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9009972 OTO - NOTNLM OT - autoimmunity OT - covid-19 OT - molecular mimicry OT - netosis OT - nets OT - sars-cov-2 OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2022/04/22 06:00 MHDA- 2022/04/22 06:01 PMCR- 2022/03/15 CRDT- 2022/04/21 05:44 PHST- 2022/03/14 00:00 [accepted] PHST- 2022/04/21 05:44 [entrez] PHST- 2022/04/22 06:00 [pubmed] PHST- 2022/04/22 06:01 [medline] PHST- 2022/03/15 00:00 [pmc-release] AID - 10.7759/cureus.23179 [doi] PST - epublish SO - Cureus. 2022 Mar 15;14(3):e23179. doi: 10.7759/cureus.23179. eCollection 2022 Mar. PMID- 34306488 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210727 IS - 1943-8141 (Print) IS - 1943-8141 (Electronic) IS - 1943-8141 (Linking) VI - 13 IP - 6 DP - 2021 TI - The combined detection of autoantibody characteristics in systemic lupus erythematosus. PG - 7242-7248 AB - OBJECTIVE: To investigate the combined detection of the autoantibody characteristics in systemic lupus erythematosus (SLE). METHODS: 105 SLE patients admitted to our hospital from May 2019 to September 2020 were placed in the SLE examination group (the SE group), 110 patients with rheumatic diseases admitted to our hospital during the same period were placed in the disease control group (the DC group), and 100 healthy people who came to our hospital for physical examinations during the same time period were placed in the healthy control group (the HC group). The SLE patients' clinical data were recorded. The patients' antinuclear antibody (ANA), anti-dsDNA, anti-SM, anti-SSA, and anti-rRnp levels were measured. RESULTS: There were no significant differences in the occurrences of fever, alopecia, photosensitivity, interstitial lung disease, Raynaud's phenomenon, pulmonary arterial hypertension, or osteoporosis. (P > 0.05), but there were higher incidences of arthralgia and butterfly erythema in the female patients (P < 0.05), and higher incidences of renal damage, oral ulcers, nervous system damage, and dysopsia in the male patients (P < 0.05). The positive autoantibody rate in the SE group was significantly higher than it was in the DC and HC groups (P < 0.05). A logistic regression analysis showed that the anti-SM, anti-dsDNA, AnuA, and anti-SSA levels entered the regression equation with statistical differences when P < 0.05. Among the four autoantibodies, the anti-SSA sensitivity was the highest, but its specificity was the lowest, and the specificities of the other three autoantibodies were all more than 97%. The positive rate of anti-dsDNA in its active stage was higher than it was in its inactive stage (P < 0.05). The positive rates of AnuA and anti-SM in their active stages was higher than they were in their inactive stages (P > 0.05). The sensitivities of the combined measurements were higher than the single autoantibody measurements (P < 0.05). CONCLUSION: Single autoantibody detection has the disadvantage of low sensitivity or specificity. The combined detection of autoantibodies can effectively improve the sensitivity and specificity of SLE detection, so it is worthy of clinical promotion. CI - AJTR Copyright © 2021. FAU - Wang, Weichen AU - Wang W AD - Medical Research & Laboratory Diagnostic Center, Jinan Central Hospital Affiliated to Shandong First Medical University Jinan 250013, Shandong Province, China. FAU - Gao, Hongmei AU - Gao H AD - Medical Research & Laboratory Diagnostic Center, Jinan Central Hospital Affiliated to Shandong First Medical University Jinan 250013, Shandong Province, China. LA - eng PT - Journal Article DEP - 20210615 PL - United States TA - Am J Transl Res JT - American journal of translational research JID - 101493030 PMC - PMC8290698 OTO - NOTNLM OT - Systemic lupus erythematosus (SLE) OT - autoantibody characteristics OT - combined detection of autoantibodies OT - diagnostic value COIS- None. EDAT- 2021/07/27 06:00 MHDA- 2021/07/27 06:01 PMCR- 2021/06/15 CRDT- 2021/07/26 06:28 PHST- 2021/02/07 00:00 [received] PHST- 2021/03/10 00:00 [accepted] PHST- 2021/07/26 06:28 [entrez] PHST- 2021/07/27 06:00 [pubmed] PHST- 2021/07/27 06:01 [medline] PHST- 2021/06/15 00:00 [pmc-release] PST - epublish SO - Am J Transl Res. 2021 Jun 15;13(6):7242-7248. eCollection 2021. PMID- 31010573 OWN - NLM STAT- MEDLINE DCOM- 20200120 LR - 20260127 IS - 2173-5778 (Electronic) IS - 2173-5778 (Linking) VI - 110 IP - 6 DP - 2019 Jul-Aug TI - Clinical Management of Cutaneous Adverse Events in Patients on Chemotherapy: A National Consensus Statement by the Spanish Academy of Dermatology and Venereology and the Spanish Society of Medical Oncology. PG - 448-459 LID - S0001-7310(19)30112-7 [pii] LID - 10.1016/j.ad.2019.01.011 [doi] AB - Although the arrival of new chemotherapy drugs and combinations has brought progress in terms of cancer patient survival, they entail many adverse effects that can compromise treatment, and hence prognosis, of the disease. Cytostatic agents can cause dermatological toxicity, among other side effects. The most familiar adverse effect of chemotherapy is alopecia. Although not serious, this changes the outward appearance of cancer patients. Other adverse effects include hypersensitivity and photosensitivity reactions, hand-foot syndrome, epidermal necrolysis, recall reactions, scleroderma-like reactions, Raynaud's phenomenon, eccrine squamous syringometaplasia, neutrophilic eccrine hidradenitis, nail abnormalities, pigmentation changes and extravasation injuries. Onset of these adverse effects often causes dose reduction and/or delayed treatment, which can affect patient survival and quality of life. It is therefore important to prevent their occurrence and treat them promptly, which requires cooperation between medical oncologists and dermatologists. This article reviews chemotherapy-associated dermatological toxicity, along with its diagnosis and therapeutic management. CI - Copyright © 2019 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved. FAU - Sanmartín, O AU - Sanmartín O AD - Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España. FAU - Beato, C AU - Beato C AD - Departamento de Oncología Médica, Hospital Universitario Virgen Macarena, Sevilla, España. FAU - Suh-Oh, H Jin AU - Suh-Oh HJ AD - Servicio de Dermatología, Complejo Hospitalario Universitario de Pontevedra, Pontevedra, España. FAU - Aragón, I AU - Aragón I AD - Departamento de Oncología Médica, Complejo Hospitalario Universitario de Huelva, Huelva, España. FAU - España, A AU - España A AD - Servicio de Dermatología, Clínica Universitaria de Navarra, Pamplona, España. FAU - Majem, M AU - Majem M AD - Departamento de Oncología Médica, Hospital de la Santa Creu i Sant Pau, Barcelona, España. FAU - Segura, S AU - Segura S AD - Servicio de Dermatología, Hospital del Mar, Barcelona, España. FAU - Gúrpide, A AU - Gúrpide A AD - Departamento de Oncología Médica, Clínica Universitaria de Navarra, Pamplona, España. FAU - Botella, R AU - Botella R AD - Servicio de Dermatología, Hospital Universitario La Fe, Facultad de Medicina, Universidad de Valencia, Valencia, España. Electronic address: rbotellaes@gmail.com. FAU - Grávalos, C AU - Grávalos C AD - Servicio de Oncología Médica, Hospital Universitario Doce de Octubre, Madrid, España. LA - eng LA - spa PT - Consensus Statement PT - Journal Article PT - Practice Guideline TT - Manejo clínico de los eventos adversos cutáneos en pacientes tratados con quimioterapia: consenso nacional de la Academia Española de Dermatología y Venereología y de la Sociedad Española de Oncología Médica. DEP - 20190419 PL - Spain TA - Actas Dermosifiliogr (Engl Ed) JT - Actas dermo-sifiliograficas JID - 101777537 RN - 0 (Antineoplastic Agents) SB - IM MH - Alopecia/chemically induced MH - Antineoplastic Agents/*adverse effects/classification MH - Disease Management MH - Drug Eruptions/*etiology/therapy MH - Drug Hypersensitivity/etiology MH - Humans MH - Nail Diseases/chemically induced MH - Neoplasms/complications/drug therapy MH - Photosensitivity Disorders/chemically induced MH - Pigmentation Disorders/chemically induced MH - Referral and Consultation MH - Severity of Illness Index MH - Spain OTO - NOTNLM OT - Chemotherapy OT - Cutaneous toxicity OT - Dermatological toxicity OT - Erupción OT - Fotosensibilidad OT - Hiperpigmentación OT - Hyperpigmentation OT - Photosensitivity OT - Quimioterapia OT - Rash OT - Toxicidad cutánea OT - Toxicidad dermatológica EDAT- 2019/04/24 06:00 MHDA- 2020/01/21 06:00 CRDT- 2019/04/24 06:00 PHST- 2018/09/04 00:00 [received] PHST- 2019/01/24 00:00 [revised] PHST- 2019/01/26 00:00 [accepted] PHST- 2019/04/24 06:00 [pubmed] PHST- 2020/01/21 06:00 [medline] PHST- 2019/04/24 06:00 [entrez] AID - S0001-7310(19)30112-7 [pii] AID - 10.1016/j.ad.2019.01.011 [doi] PST - ppublish SO - Actas Dermosifiliogr (Engl Ed). 2019 Jul-Aug;110(6):448-459. doi: 10.1016/j.ad.2019.01.011. Epub 2019 Apr 19. PMID- 41278053 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251124 LR - 20251126 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 11 DP - 2025 Nov TI - Anti-synthetase Syndrome Presenting Primarily as Interstitial Lung Disease in a Young Adult: A Diagnostic Challenge in Acute Medicine. PG - e97344 LID - 10.7759/cureus.97344 [doi] LID - e97344 AB - Anti-synthetase syndrome (ASyS) is a rare autoimmune connective tissue disorder (CTD) characterized by the presence of autoantibodies targeting tRNA synthetase, most notably the anti-Jo1 antibody. Clinically, it typically manifests with interstitial lung disease (ILD), inflammatory myopathy, arthritis, Raynaud's phenomenon, and the so-called mechanic's hands. Diagnosis can be challenging due to clinical overlap with other pulmonary and rheumatological disorders. We report a case of a young woman in her early 20s who presented at our same day emergency care (SDEC) with a four-week history of exertional dyspnea, low-grade fever, and productive cough, initially managed as a lower respiratory tract infection in the community. Persistent symptoms prompted further evaluation, and CT imaging of the chest, abdomen, and pelvis revealed bilateral ground-glass opacities suggestive of ILD. Five days later, she re-presented with hemoptysis, worsening breathlessness, new-onset myalgia, arthralgia, facial erythema, and hyperkeratosis of the fingertips. Autoimmune screening revealed strongly positive anti-Jo-1 and anti-Ro52 antibodies, confirming the diagnosis of anti-synthetase syndrome. She was treated with intravenous (IV) pulse methylprednisolone, mycophenolate mofetil, and rituximab, with partial clinical improvement and ongoing follow-up for advanced ILD management in a tertiary center. This case highlights how an initial diagnosis of lower respiratory tract infection (LRTI) may mask an underlying autoimmune syndrome (anti-synthetase syndrome), which later manifests as progressive respiratory failure requiring lung transplant. Therefore, as acute medicine physicians, it is imperative to maintain a broad differential diagnosis, particularly in young females newly diagnosed with possible ILD. There may be underlying conditions that require careful examination by acute care physicians, especially in the presence of systemic symptoms, notably rheumatological symptoms. This vigilance is crucial to avoid delayed diagnosis of anti-synthetase syndrome, which necessitates early multidisciplinary involvement to achieve better outcomes. CI - Copyright © 2025, Ahmed et al. FAU - Ahmed, Kawser AU - Ahmed K AD - Acute Internal Medicine, Northampton General Hospital NHS Trust, Northampton, GBR. FAU - Bangash, Faaraan AU - Bangash F AD - Acute Medicine, Northampton General Hospital NHS Trust, Northampton, GBR. FAU - Bangash, Arsalan AU - Bangash A AD - General Medicine, Northampton General Hospital NHS Trust, Northampton, GBR. FAU - Hussain, Yaseen AU - Hussain Y AD - Acute Medicine, Northampton General Hospital NHS Trust, Northampton, GBR. LA - eng PT - Case Reports PT - Journal Article DEP - 20251120 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12633841 OTO - NOTNLM OT - anti-synthetase (as) syndrome OT - auto immune OT - diffuse interstitial lung disease OT - home oxygen therapy OT - lung transplant COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/11/24 18:21 MHDA- 2025/11/24 18:22 PMCR- 2025/11/20 CRDT- 2025/11/24 08:16 PHST- 2025/11/20 00:00 [accepted] PHST- 2025/11/24 18:22 [medline] PHST- 2025/11/24 18:21 [pubmed] PHST- 2025/11/24 08:16 [entrez] PHST- 2025/11/20 00:00 [pmc-release] AID - 10.7759/cureus.97344 [doi] PST - epublish SO - Cureus. 2025 Nov 20;17(11):e97344. doi: 10.7759/cureus.97344. eCollection 2025 Nov. PMID- 41659860 OWN - NLM STAT- MEDLINE DCOM- 20260608 LR - 20260608 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Anti-CENP-B polarity divides SLE: divergent clinical-immune phenotypes and distinct treatment responses. PG - 1762899 LID - 10.3389/fimmu.2026.1762899 [doi] LID - 1762899 AB - BACKGROUND: Anti-CENP-B antibodies (anti-CENP-B), directed against centromere protein B, are a serological hallmark of limited cutaneous systemic sclerosis (lcSSc) but are only occasionally encountered in systemic lupus erythematosus (SLE). When detected in SLE they may create diagnostic ambiguity. Since autoantibody-defined SLE subsets exhibit distinct phenotypes, delineating the clinical and immunological features of anti-CENP-B-positive disease is essential for precise management. METHODS: We retrospectively collected demographic, clinical, laboratory and therapeutic data from 310 SLE patients including 73 anti-CENP-B-positive patients and 237 anti-CENP-B-negative patients. Inter-group differences, correlations, and multivariable logistic regression were performed. RESULTS: Compared with the anti-CENP-B-negative patients, the anti-CENP-B-positive patients were older, less frequently had lupus nephritis (LN), but more often exhibited Raynaud's phenomenon, cardiac, or pleuropulmonary involvement. Serologically, they displayed lower anti-dsDNA, anti-nucleosome and anti-histone antibody levels, reduced C4, yet higher IgA, IgM, and IgG concentrations and expanded CD19(+) B-cell numbers; accordingly, SLEDAI-2K scores and 24-h urinary protein (24h-UTP) were lower. C4 inversely correlated with disease activity indices and IgG in the positive group, whereas in the negative group it also correlated with B-cell counts and IgM levels. Multivariate logistic regression identified age, Raynaud's phenomenon, CD19(+) B-cell count, and IgG level as factors independently associated with anti-CENP-B positivity. Compared with their anti-CENP-B-negative patients, anti-CENP-B-positive SLE patients displayed a markedly divergent therapeutic response. CONCLUSIONS: Anti-CENP-B positivity defines a distinct SLE subset characterized by older age at onset, milder renal involvement, Raynaud's phenomenon, and specific humoral alterations; importantly, these patients also show a treatment response that differs significantly from that of the anti-CENP-B-negative group, underscoring the imperative for personalized, precision therapy. CI - Copyright © 2026 Liu, Xiao, Yao, Yuan, Zhang, Li, Zhang, Tian and An. FAU - Liu, Xiaoli AU - Liu X AD - Department of Clinical Laboratory, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan, China. FAU - Xiao, Zhefeng AU - Xiao Z AD - Department of Pathology, NHC Key Laboratory of Cancer Proteomics, National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, China. FAU - Yao, Yuxing AU - Yao Y AD - College of Letters and Science, University of California, Berkeley, Berkeley, CA, United States. FAU - Yuan, Youhua AU - Yuan Y AD - Department of Clinical Laboratory, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan, China. FAU - Zhang, Xia AU - Zhang X AD - Department of Pathology, Henan Medical College, Zhengzhou, China. FAU - Li, Jianfeng AU - Li J AD - Department of Pathology, Henan Medical College, Zhengzhou, China. FAU - Zhang, Xiuzhi AU - Zhang X AD - Department of Pathology, Henan Medical College, Zhengzhou, China. FAU - Tian, Xiaohui AU - Tian X AD - Department of Clinical Laboratory, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan, China. FAU - An, Lemei AU - An L AD - Department of Rheumatology and Immunology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, China. LA - eng PT - Journal Article DEP - 20260122 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Female MH - *Lupus Erythematosus, Systemic/immunology/diagnosis/therapy MH - Male MH - Adult MH - Phenotype MH - *Autoantibodies/immunology/blood MH - Retrospective Studies MH - Middle Aged MH - Treatment Outcome PMC - PMC12872543 OTO - NOTNLM OT - anti-CENP OT - immune cell OT - limited cutaneous systemic sclerosis OT - multivariate regression OT - systemic lupus erythematosus (SLE) OT - therapeutic response COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/02/09 12:54 MHDA- 2026/02/09 12:55 PMCR- 2026/01/22 CRDT- 2026/02/09 06:56 PHST- 2025/12/08 00:00 [received] PHST- 2026/01/03 00:00 [revised] PHST- 2026/01/07 00:00 [accepted] PHST- 2026/02/09 12:55 [medline] PHST- 2026/02/09 12:54 [pubmed] PHST- 2026/02/09 06:56 [entrez] PHST- 2026/01/22 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1762899 [doi] PST - epublish SO - Front Immunol. 2026 Jan 22;17:1762899. doi: 10.3389/fimmu.2026.1762899. eCollection 2026. PMID- 33788398 OWN - NLM STAT- MEDLINE DCOM- 20211130 LR - 20221207 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 24 IP - 5 DP - 2021 May TI - Clinical characteristics of Vietnamese patients with idiopathic inflammatory myopathies and autoantibodies to aminoacyl-transfer RNA synthetases. PG - 663-670 LID - 10.1111/1756-185X.14105 [doi] AB - OBJECTIVE: To assess clinical phenotypes of anti-aminoacyl-transfer RNA synthetases (aaRS) autoantibodies in Vietnamese patients of Kinh ethnicity with idiopathic inflammatory myopathies (IIM). METHODS: In a cross-sectional study 23 patients with anti-aaRS autoantibodies were compared to 36 patients with other myositis-specific antibodies and to 69 seronegative patients with IIM. Assessments included muscle performance, extra-muscular involvement, and disease activity according to the International Myositis Assessment and Clinical Studies (IMACS). Sera were tested by a line immunoassay (Euroline Myositis Profile 4). RESULTS: The frequency of anti-Jo-1 antibodies was 56.5%, anti-EJ antibodies 26.1%, and anti-PL-7 antibodies 17.4%, while anti-PL-12 and anti-OJ antibodies were not present in any case. All patients with anti-aaRS autoantibodies had signs of myositis. At time of investigation 22/23 patients had muscle weakness, 52.2% arthritis, 34.8% Raynaud's phenomenon, 73.9% fever, 14.3% mechanic's hands and 56.5% dysphagia. Interstitial lung disease was present in 52.2%, and pulmonary hypertension in 56.5%. The anti-aaRS autoantibody positive group had higher disease activity in the domains of skin and pulmonary disease compared to the seronegative group and had lower disease activity in skeletal disease compared to the anti-melanoma differentiation-associated protein 5-positive patients. The clinical presentation of antisynthetase syndrome was similar between the aaRS autoantibody specificities with the exception of more frequent pulmonary hypertension in anti-Jo-1 positive patients. CONCLUSIONS: Different aaRS autoantibody specificities may vary between different ethnic populations for reasons that still need to be clarified. Furthermore, the high frequency of pulmonary hypertension is noteworthy but otherwise clinical manifestations associated with aaRS autoantibodies did not differ from other ethnic populations. CI - © 2021 The Authors. International Journal of Rheumatic Diseases published by Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Phuong, Thuy Nguyen Thi AU - Phuong TNT AD - Rheumatology Department, Bach Mai Hospital, Hanoi, Vietnam. AD - Internal Medicine Department, Hanoi Medical University, Hanoi, Vietnam. FAU - Ngoc, Lan Nguyen Thi AU - Ngoc LNT AD - Internal Medicine Department, Hanoi Medical University, Hanoi, Vietnam. FAU - Rönnelid, Johan AU - Rönnelid J AD - Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden. FAU - Padyukov, Leonid AU - Padyukov L AD - Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden. AD - Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden. FAU - Lundberg, Ingrid E AU - Lundberg IE AUID- ORCID: 0000-0002-6068-9212 AD - Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden. AD - Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden. AD - Rheumatology clinic, Karolinska University Hospital, Stockholm, Sweden. LA - eng GR - The Swedish Research council/ GR - Grants provided by Region Stockholm (ALF project)/ GR - Asia Pacific League of Associations for Rheumatology/ PT - Journal Article DEP - 20210331 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Adult MH - Aged MH - Amino Acyl-tRNA Synthetases/*immunology MH - Antibodies, Antinuclear/blood MH - Asian People MH - Autoantibodies/blood/*immunology MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Myositis/*diagnosis/epidemiology MH - Vietnam/epidemiology OTO - NOTNLM OT - Kinh ethnicity OT - antisynthetase autoantibodies OT - dermatomyositis OT - myositis OT - polymyositis EDAT- 2021/04/01 06:00 MHDA- 2021/12/01 06:00 CRDT- 2021/03/31 13:42 PHST- 2021/03/02 00:00 [revised] PHST- 2020/11/03 00:00 [received] PHST- 2021/03/06 00:00 [accepted] PHST- 2021/04/01 06:00 [pubmed] PHST- 2021/12/01 06:00 [medline] PHST- 2021/03/31 13:42 [entrez] AID - 10.1111/1756-185X.14105 [doi] PST - ppublish SO - Int J Rheum Dis. 2021 May;24(5):663-670. doi: 10.1111/1756-185X.14105. Epub 2021 Mar 31. PMID- 35387216 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221003 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 6 IP - 3 DP - 2021 Oct TI - Mycophenolate in scleroderma-associated interstitial lung disease: Real-world data from rheumatology and pulmonology clinics in South Asia. PG - 271-276 LID - 10.1177/23971983211024410 [doi] AB - INTRODUCTION: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease. AIM: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease. METHODS: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients. RESULTS: Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud's symptoms of 36 months (13.5-60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0-1.3) and 1 (0-1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5-49), the percentage of forced vital capacity improved by 1.8% (-3.82 to 9.07) as compared with baseline visit (p = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively. CONCLUSION: Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period. CI - © The Author(s) 2021. FAU - Janardana, Ramya AU - Janardana R AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. AD - Clinical Immunology & Rheumatology, St. John's Medical College, Bengaluru, India. FAU - Irodi, Aparna AU - Irodi A AD - Department of Radiology, Christian Medical College, Vellore, India. FAU - Chebbi, Pramod P AU - Chebbi PP AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. AD - SDM College of Medical Sciences and Hospital, Dharwad, India. FAU - Goel, Ruchika AU - Goel R AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Vimala, Leena R AU - Vimala LR AD - Department of Radiology, Christian Medical College, Vellore, India. FAU - Padiyar, Shivraj AU - Padiyar S AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Peediyakal, Anoof AU - Peediyakal A AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. AD - Dr Anoof's RheumaCare, Calicut, India. FAU - Mathew, John AU - Mathew J AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Nair, Aswin AU - Nair A AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. FAU - Christopher, Devasahayam J AU - Christopher DJ AD - Department of Pulmonary Medicine, Christian Medical College, Vellore, India. FAU - Danda, Debashish AU - Danda D AUID- ORCID: 0000-0002-2121-0942 AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India. LA - eng PT - Journal Article DEP - 20210630 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922665 OTO - NOTNLM OT - Scleroderma-ILD OT - mycophenolate OT - outcomes COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2022/04/08 06:00 MHDA- 2022/04/08 06:01 PMCR- 2022/10/01 CRDT- 2022/04/07 05:22 PHST- 2021/04/06 00:00 [received] PHST- 2021/05/15 00:00 [accepted] PHST- 2022/04/07 05:22 [entrez] PHST- 2022/04/08 06:00 [pubmed] PHST- 2022/04/08 06:01 [medline] PHST- 2022/10/01 00:00 [pmc-release] AID - 10.1177_23971983211024410 [pii] AID - 10.1177/23971983211024410 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2021 Oct;6(3):271-276. doi: 10.1177/23971983211024410. Epub 2021 Jun 30. PMID- 18852227 OWN - NLM STAT- MEDLINE DCOM- 20090305 LR - 20081014 IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 17 IP - 11 DP - 2008 Nov TI - Greater accrual damage in late-onset systemic lupus erythematosus: a long-term follow-up study. PG - 1023-8 LID - 10.1177/0961203308089695 [doi] AB - The main objective of this study was to evaluate the clinical differences and the pattern and extent of organ damage in late-onset systemic lupus erythematosus (SLE). A nested case-control study was performed from patients with SLE followed in the Rheumatology Unit of the State University of Campinas between 1974 and 2005. Patients who developed SLE after the age of 49 were considered late-onset SLE. SLE patients with age <49 years, matched for sex, ethnicity, disease duration and organ damage at study entry were randomly chosen to compose the control group. Baseline and cumulative clinical manifestations, laboratory data, SLE disease activity index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology-damage index (SDI) and mortality were compared between groups. At diagnosis and follow-up, late-onset group had lower SLEDAI scores when compared with younger age onset. Clinically, they presented less frequently arthritis (P = 0.0002) and malar rash (P = 0.02) and more frequently Raynaud's phenomenon (P = 0.002) and arterial hypertension (P = 0.02) when compared with young onset at diagnosis. Late-onset SLE received lower total corticosteroid dose (P < 0.001) and less frequently cyclophosphamide (P = 0.01). During the study period, late-onset SLE had always lower SLEDAI scores (P = 0.001). At study endpoint, late-onset SLE patients had significantly higher SDI scores (P = 0.001) and a higher mortality rate when compared with younger onset group (P < 0.01). In conclusion, late-onset SLE is milder on presentation and during course of disease, but patients have more organ damage and a higher rate of mortality than young onset SLE. Patients with late onset should be followed with close monitoring and early identification of complications is mandatory in this subgroup of patients with SLE. FAU - Appenzeller, S AU - Appenzeller S AD - Department of Internal Medicine, Rheumatology Unit, State University of Campinas, Campinas, Brazil. appenzel@unicamp.br FAU - Pereira, D A AU - Pereira DA FAU - Costallat, L T L AU - Costallat LT LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Age of Onset MH - Aged MH - Case-Control Studies MH - Female MH - Follow-Up Studies MH - Humans MH - Lupus Erythematosus, Systemic/*diagnosis MH - Male MH - Middle Aged MH - Severity of Illness Index MH - Time Factors EDAT- 2008/10/15 09:00 MHDA- 2009/03/06 09:00 CRDT- 2008/10/15 09:00 PHST- 2008/10/15 09:00 [pubmed] PHST- 2009/03/06 09:00 [medline] PHST- 2008/10/15 09:00 [entrez] AID - 17/11/1023 [pii] AID - 10.1177/0961203308089695 [doi] PST - ppublish SO - Lupus. 2008 Nov;17(11):1023-8. doi: 10.1177/0961203308089695. PMID- 36589603 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230111 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 37 IP - 3 DP - 2022 Sep TI - Demographic, clinical, laboratory data, prognostic, and treatment features of patients with antisynthetase syndrome: An international, two-center cohort study. PG - 424-434 LID - 10.46497/ArchRheumatol.2022.9108 [doi] AB - OBJECTIVES: To compare clinical, demographic, laboratory data, prognostic and treatment characteristics of patients with antisynthetase syndrome (ASSD) treated in two different centers of India and Brazil. PATIENTS AND METHODS: This international, two-center, retro-prospective cohort study which was conducted at two tertiary rheumatology centers (one in Brazil and one in India) between January 2000 to January 2020 included a total of 115 patients with ASSD (21 males, 94 females; mean age; at disease diagnosis at 40.3; range, 18 to 80 years). Demographic, clinical and laboratory data of the patients were recorded. Clinical involvement was evaluated. RESULTS: Of the patients, 81 were Brazilians and 34 were of Indian origin. The Indian group exhibited a greater delay in diagnosis after the onset of symptoms compared to Brazilian patients (12 vs. 6 months, respectively; p=0.026). Brazilian patients exhibited a significantly higher prevalence of joint and lung involvement, mechanic's hands, and Raynaud's phenomenon. Anti-Jo-1 was the most common autoantibodies in both groups. Systemic arterial hypertension, followed by diabetes mellitus were the most prevalent comorbidities. Concerning previously used drugs, the Indian patients had a larger group of patients treated with antimalarials, whereas the Brazilian group used more azathioprine and intravenous immunoglobulin. A higher proportion of Indian patients was treated with one immunosuppressive drug (70.6%), while the Brazilian group were often treated using two immunosuppressive drugs (33%). Comparison between the severity and prognosis showed that Brazilian group had a higher number of relapses, and during follow-up, the global mortality rates were similar in both groups (6.2% for Brazilian vs. 8.8% for Indian). CONCLUSION: Brazilian and Indian patients with ASSD have comparable epidemiological characteristics such as age at the time of disease diagnosis, and sex distribution, and autoantibodies. Diagnostic delay is seen in Indian patients, and Brazilians exhibit a higher prevalence of joint and lung involvement, mechanic's hands, Raynaud's phenomenon with a higher number of relapses, although the mortality rate seems to be similar in both groups. CI - Copyright © 2022, Turkish League Against Rheumatism. FAU - Da Silva, Lila Morena Bueno AU - Da Silva LMB AUID- ORCID: 0000-0003-0792-5789 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil. FAU - Rathore, Upendra AU - Rathore U AUID- ORCID: 0000-0003-1658-8155 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. FAU - Agarwal, Vikas AU - Agarwal V AUID- ORCID: 0000-0002-4508-1233 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. FAU - Gupta, Latika AU - Gupta L AUID- ORCID: 0000-0003-2753-2990 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AUID- ORCID: 0000-0002-3682-4517 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil. LA - eng PT - Journal Article DEP - 20220303 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC9791548 OTO - NOTNLM OT - Antisynthetase syndrome OT - dermatomyositis OT - myositis OT - outcomes COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2023/01/03 06:00 MHDA- 2023/01/03 06:01 PMCR- 2022/03/03 CRDT- 2023/01/02 04:02 PHST- 2021/07/19 00:00 [received] PHST- 2021/11/11 00:00 [accepted] PHST- 2023/01/02 04:02 [entrez] PHST- 2023/01/03 06:00 [pubmed] PHST- 2023/01/03 06:01 [medline] PHST- 2022/03/03 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2022.9108 [doi] PST - epublish SO - Arch Rheumatol. 2022 Mar 3;37(3):424-434. doi: 10.46497/ArchRheumatol.2022.9108. eCollection 2022 Sep. PMID- 25233870 OWN - NLM STAT- MEDLINE DCOM- 20150615 LR - 20150326 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 67 IP - 4 DP - 2015 Apr TI - 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group. PG - 582-7 LID - 10.1002/acr.22451 [doi] AB - OBJECTIVE: The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc; scleroderma) in an independent cohort of SSc subjects and to assess the contribution of individual items of the criteria to the overall sensitivity. METHODS: SSc subjects from the Canadian Scleroderma Research Group cohort were assessed. Sensitivity was determined in several subgroups of patients. In patients without the criterion of skin thickening proximal to the metacarpophalangeal (MCP) joints, we recalculated sensitivity after removing the individual criterion. RESULTS: A total of 724 SSc patients were included. Most were women (86%), mean age was 55.8 years, mean disease duration was 10.9 years, and 59% had limited cutaneous SSc (lcSSc). Overall, the sensitivity of the 2013 criteria was 98.3% compared to 88.3% for the 1980 criteria. This pattern was consistent among those with lcSSc (98.8% versus 85.6%), anticentromere antibodies (98.9% versus 79.8%), disease duration ≤3 years (98.7% versus 84.7%), and no skin involvement proximal to the MCP joints (97% versus 60%). In the latter subgroup, removing Raynaud's phenomenon and sclerodactyly from the criteria reduced the sensitivity to 77% and 79%, respectively. Removing both sclerodactyly and puffy fingers reduced the sensitivity to 62%. CONCLUSION: The 2013 SSc classification criteria classify more SSc patients than the 1980 criteria. The improvement in sensitivity is most striking in those with lcSSc, especially those without skin involvement proximal to the MCP joints. The addition of Raynaud's phenomenon and puffy fingers to the 2013 criteria accounts for important gains in sensitivity. CI - Copyright © 2015 by the American College of Rheumatology. FAU - Alhajeri, Hebah AU - Alhajeri H AD - McGill University and Jewish General Hospital, Montreal, Quebec, Canada. FAU - Hudson, Marie AU - Hudson M FAU - Fritzler, Marvin AU - Fritzler M FAU - Pope, Janet AU - Pope J FAU - Tatibouet, Solène AU - Tatibouet S FAU - Markland, Janet AU - Markland J FAU - Robinson, David AU - Robinson D FAU - Jones, Niall AU - Jones N FAU - Khalidi, Nader AU - Khalidi N FAU - Docherty, Peter AU - Docherty P FAU - Kaminska, Elzbieta AU - Kaminska E FAU - Masetto, Ariel AU - Masetto A FAU - Sutton, Evelyn AU - Sutton E FAU - Mathieu, Jean-Pierre AU - Mathieu JP FAU - Ligier, Sophie AU - Ligier S FAU - Grodzicky, Tamara AU - Grodzicky T FAU - LeClercq, Sharon AU - LeClercq S FAU - Thorne, Carter AU - Thorne C FAU - Gyger, Geneviève AU - Gyger G FAU - Smith, Douglas AU - Smith D FAU - Fortin, Paul R AU - Fortin PR FAU - Larché, Maggie AU - Larché M FAU - Baron, Murray AU - Baron M LA - eng GR - FRN83518/Canadian Institutes of Health Research/Canada PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Aged MH - Canada/epidemiology MH - Cohort Studies MH - Cross-Sectional Studies MH - Europe/epidemiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Rheumatic Diseases/*classification/diagnosis/epidemiology MH - Rheumatology/*classification/standards MH - Scleroderma, Localized/*classification/diagnosis/epidemiology MH - Scleroderma, Systemic/*classification/diagnosis/epidemiology MH - Societies, Medical/*standards MH - United States/epidemiology MH - Young Adult EDAT- 2014/09/23 06:00 MHDA- 2015/06/16 06:00 CRDT- 2014/09/20 06:00 PHST- 2014/01/13 00:00 [received] PHST- 2014/08/12 00:00 [accepted] PHST- 2014/09/20 06:00 [entrez] PHST- 2014/09/23 06:00 [pubmed] PHST- 2015/06/16 06:00 [medline] AID - 10.1002/acr.22451 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2015 Apr;67(4):582-7. doi: 10.1002/acr.22451. PMID- 35585952 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230602 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 7 IP - 2 DP - 2022 Jun TI - Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest. PG - 135-143 LID - 10.1177/23971983221086214 [doi] AB - OBJECTIVE: Certain Hispanic/Latino (Hispanic) populations have been reported to have higher rates and severity of systemic sclerosis; however, little is known of systemic sclerosis in the American Southwest. This study compared manifestations of systemic sclerosis in Hispanics with non-Hispanics of New Mexico. METHODS: This cross-sectional longitudinal study included 109 systemic sclerosis patients followed over a mean of 12.6 ± 8.9 years. Subjects were repetitively evaluated including physical examination, echocardiography, chest imaging, and serologic testing and observed for complications. Disease characteristics and long-term outcomes were statistically compared between self-identified Hispanic and non-Hispanic subjects. RESULTS: A total of 73 (67%) systemic sclerosis subjects were Hispanic and 36 (33%) were non-Hispanic. The cohorts were similar in mean age, age of systemic sclerosis onset, limited versus diffuse cutaneous systemic sclerosis, telangiectases, gastroesophageal reflux disease, Raynaud's phenomenon, autoantibody profile, interstitial lung disease, pulmonary hypertension, scleroderma renal crisis, mortality, and comorbid malignancy (all p > 0.05). However, the standardized mortality ratio was increased in both cohorts relative to age-adjusted mortality: Hispanic: 2.08, confidence interval (1.94-2.24); non-Hispanic: 1.56, confidence interval (1.46-1.68). Furthermore, the standardized incidence ratio for malignancy was increased in both cohorts: Hispanic: 1.45, confidence interval (1.35-1.56); non-Hispanic: 1.24, confidence interval (1.16-1.34). The mean age of cancer diagnosis occurred at a significantly younger age in Hispanics (Hispanics: 53.1 ± 9.7 years; non-Hispanics 63.7 ± 7.9 years; 95% confidence interval: -19 ⩽ 10.6 ⩽ 2.2; p = 0.016). CONCLUSION: Systemic sclerosis phenotype, autoantibodies, complications, outcomes, malignancy rates, and mortality are generally similar between Hispanics and non-Hispanics with systemic sclerosis in the American Southwest. However, age-adjusted comorbid malignancy and mortality rates are significantly increased in both groups. CI - © The Author(s) 2022. FAU - Nunez, Sharon E AU - Nunez SE AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Ariza-Hutchinson, Angie AU - Ariza-Hutchinson A AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Fields, Roderick A AU - Fields RA AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Vondenberg, Jaime A AU - Vondenberg JA AD - Department of Medicine, Rheumatology/Immunology, Cleveland Clinic, Cleveland, OH, USA. FAU - Patel, Rosemina A AU - Patel RA AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Emil, N Suzanne AU - Emil NS AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Muruganandam, Maheswari AU - Muruganandam M AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Gibb, James I AU - Gibb JI AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Poole, Janet L AU - Poole JL AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. FAU - Sibbitt, Wilmer L Jr AU - Sibbitt WL Jr AUID- ORCID: 0000-0001-5872-160X AD - Department of Internal Medicine, Division of Rheumatology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA. LA - eng PT - Journal Article DEP - 20220410 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC9109509 OTO - NOTNLM OT - Hispanic OT - Latino OT - Scleroderma OT - cancer OT - outcome OT - systemic sclerosis COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2022/05/20 06:00 MHDA- 2022/05/20 06:01 PMCR- 2023/06/01 CRDT- 2022/05/19 02:15 PHST- 2021/11/10 00:00 [received] PHST- 2022/02/16 00:00 [accepted] PHST- 2022/05/19 02:15 [entrez] PHST- 2022/05/20 06:00 [pubmed] PHST- 2022/05/20 06:01 [medline] PHST- 2023/06/01 00:00 [pmc-release] AID - 10.1177_23971983221086214 [pii] AID - 10.1177/23971983221086214 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2022 Jun;7(2):135-143. doi: 10.1177/23971983221086214. Epub 2022 Apr 10. PMID- 34251299 OWN - NLM STAT- MEDLINE DCOM- 20210802 LR - 20220407 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 39 Suppl 131 IP - 4 DP - 2021 Jul-Aug TI - Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India. PG - 149-156 LID - 10.55563/clinexprheumatol/7ubxxi [doi] AB - OBJECTIVES: To describe the clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma (JSSc) from a tertiary care referral hospital in North-West India. METHODS: A review of case records was performed and children with JSSc (disease onset <14 years of age) were analysed. Diagnosis was based on the Paediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc. RESULTS: Forty patients (28 girls and 12 boys; F:M ratio= 2.3:1) were diagnosed with JSSc (including 22 children with overlap) in the last 25 years. Mean age at symptom onset was 7.75±3.19 years with a mean delay in diagnosis of 2.275±2.09 years. Raynaud's phenomenon was seen in 26/40 (65%) patients at presentation. Lung involvement was noted in 40% patients. Methotrexate was the most commonly used therapy, followed by oral prednisolone. Patients without overlap had higher incidence of cutaneous ulcers as compared to patients with overlap (55% vs. 18%; p-value: 0.01). Patients with overlap required significantly higher oral prednisolone (81% vs. 22%), methotrexate (72% vs. 38%) and hydroxychloroquine (54% vs. 5%) while cyclophosphamide (13% vs. 44%) and azathioprine (9% vs. 44%) were used relatively less in this group. Mortality was 15% at a mean follow-up of 51.75 months. Infections were noted to be the most common cause of death. There was no significant difference in the mortality between patients with and without lung disease or patients with or without overlap. CONCLUSIONS: We describe the largest single-centre cohort with longest follow-up of juvenile systemic scleroderma from India. FAU - Jindal, Ankur Kumar AU - Jindal AK AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. ankurjindal11@gmail.com. FAU - Patra, Pratap AU - Patra P AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Guleria, Sandesh AU - Guleria S AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Gupta, Anju AU - Gupta A AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Bhattad, Sagar AU - Bhattad S AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Rawat, Amit AU - Rawat A AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Suri, Deepti AU - Suri D AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Pandiarajan, Vignesh AU - Pandiarajan V AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Bishnoi, Anuradha AU - Bishnoi A AD - Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Dogra, Sunil AU - Dogra S AD - Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Singh, Surjit AU - Singh S AD - Allergy Immunology Unit, Department of Paediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. LA - eng PT - Journal Article PT - Review DEP - 20210706 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - MRK240IY2L (Azathioprine) RN - Juvenile systemic scleroderma SB - IM MH - Azathioprine MH - Child MH - Female MH - Follow-Up Studies MH - Humans MH - India/epidemiology MH - Male MH - *Scleroderma, Systemic/diagnosis/drug therapy/epidemiology EDAT- 2021/07/13 06:00 MHDA- 2021/08/03 06:00 CRDT- 2021/07/12 12:22 PHST- 2020/05/18 00:00 [received] PHST- 2020/10/21 00:00 [accepted] PHST- 2021/07/13 06:00 [pubmed] PHST- 2021/08/03 06:00 [medline] PHST- 2021/07/12 12:22 [entrez] AID - 15842 [pii] AID - 10.55563/clinexprheumatol/7ubxxi [doi] PST - ppublish SO - Clin Exp Rheumatol. 2021 Jul-Aug;39 Suppl 131(4):149-156. doi: 10.55563/clinexprheumatol/7ubxxi. Epub 2021 Jul 6. PMID- 31498073 OWN - NLM STAT- MEDLINE DCOM- 20191021 LR - 20191022 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 37 Suppl 119 IP - 4 DP - 2019 Jul-Aug TI - Reliability, construct validity and responsiveness to change of the PROMIS-29 in systemic sclerosis-associated interstitial lung disease. PG - 49-56 AB - OBJECTIVES: PROMIS-29 is a generic health-related quality of life instrument. Our objective was to assess the reliability, construct validity, and responsiveness to change of PROMIS-29 in systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Seventy-three participants with SSc-ILD were administered patient reported outcomes (PROs) at baseline and follow-up visits which included PROMIS-29 and other measures of generic health, dyspnea, and cough instruments. We assessed internal consistency reliability using Cronbach's α, an alpha of ≥ 0.70 was considered satisfactory. We assessed the responsiveness to change using linear regression models. RESULTS: Mean age of the participants was 51.9 years and the mean disease duration was 7.9 years after first non-Raynaud's symptom. Of the 73 participants, 56.2% were classified as diffuse SSc and 26% limited SSc. The baseline (mean ± SD) FVC % predicted was 73.9±15.5 with a DLCO % predicted of 57.7±21.1; 95.9% had fibrotic NSIP pattern on HRCT. PROMIS-29 scores were 0.2 to 0.9 SD below the US population. Cronbach's α reliability was acceptable for all domains (ranged from 0.77 to 0.98). All scales showed statistically significant correlations with hypothesised PROMIS-29 domains (p≤0.05 for all comparisons). PROMIS-29 showed none-to-small discriminatory ability in comparison with physiologic measures (FVC and DLCO). There was no significant relationship between the change in FVC versus the change in PROMIS-29 measures over time. CONCLUSIONS: PROMIS-29 has adequate reliability and construct validity for evaluation in SSc-ILD. It has moderate-to-large correlations with other PROs. The PROMIS-29 domains were not found to change over time in this cohort, likely due to stable nature of the observational cohort. FAU - Fisher, Caitlyn J AU - Fisher CJ AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Namas, Rajaie AU - Namas R AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Seelman, Daniela AU - Seelman D AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Jaafar, Sara AU - Jaafar S AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Homer, Kate AU - Homer K AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Wilhalme, Holly AU - Wilhalme H AD - Statistics Core, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA. FAU - Young, Amber AU - Young A AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Nagaraja, Vivek AU - Nagaraja V AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - White, Eric S AU - White ES AD - Division of Pulmonary Medicine and Critical Care, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Schiopu, Elena AU - Schiopu E AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Flaherty, Kevin AU - Flaherty K AD - Division of Pulmonary Medicine and Critical Care, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan Scleroderma Program, Division of Rheumatology Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. khannad@umich.edu. LA - eng PT - Journal Article DEP - 20190904 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Dyspnea MH - Female MH - Humans MH - *Lung Diseases, Interstitial/psychology MH - Male MH - Middle Aged MH - *Quality of Life MH - Reproducibility of Results MH - *Scleroderma, Systemic/psychology MH - Surveys and Questionnaires/*standards EDAT- 2019/09/10 06:00 MHDA- 2019/10/23 06:00 CRDT- 2019/09/10 06:00 PHST- 2018/10/16 00:00 [received] PHST- 2019/01/28 00:00 [accepted] PHST- 2019/09/10 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] PHST- 2019/09/10 06:00 [entrez] AID - 13541 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2019 Jul-Aug;37 Suppl 119(4):49-56. Epub 2019 Sep 4. PMID- 26179502 OWN - NLM STAT- MEDLINE DCOM- 20160608 LR - 20150904 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 18 IP - 7 DP - 2015 Sep TI - Anti-centromere antibody-positive Sjögren's syndrome: A distinct clinical subgroup? PG - 776-82 LID - 10.1111/1756-185X.12684 [doi] AB - AIM: To investigate whether patients with Sjögren's syndrome (SS) can be distinguished based on the positivity of anti-centromere antibody (ACA), and if so, whether the subgroups differ in their clinical and laboratory features. METHODS: Eleven patients with ACA-positive and 71 patients ACA-negative SS were examined. All patients had minor salivary gland biopsy; sociodemographic data, glandular and extraglandular manifestations, and laboratory findings, including autoantibodies, complement and immunoglobulin levels, were analyzed. European League Against Rheumatism SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. RESULTS: The prevalence of ACA among SS patients was 13.4%. ACA-positive SS patients had a higher prevalence of Raynaud's phenomenon, sclerodactyly and autoimmune thyroiditis and a lower prevalence of anti-SSA/Ro and anti-SSB/La antibodies compared to ACA-negative patients. Disease activity was higher in ACA-positive patients than in ACA-negative patients, but the damage index did not differ between the two groups. None of the patients who originally had ACA evolved to having full-blown systemic sclerosis. CONCLUSION: Patients with SS who have ACA differ from classic SS patients in several clinical and laboratory parameters. ACA should be considered one of the pathogenically relevant autoantibodies for SS. CI - © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd. FAU - Lee, Kyung-Eun AU - Lee KE AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Kang, Ji-Hyoun AU - Kang JH AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Lee, Jeong-Won AU - Lee JW AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Wen, Lihui AU - Wen L AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Park, Dong-Jin AU - Park DJ AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Kim, Tae-Jong AU - Kim TJ AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Park, Yong-Wook AU - Park YW AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. FAU - Lee, Shin-Seok AU - Lee SS AD - Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150714 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (anticentromere antibody) SB - IM MH - Adult MH - Antibodies, Antinuclear/*analysis MH - Biomarkers/blood MH - Biopsy MH - Female MH - Fluorescent Antibody Technique MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prevalence MH - Prognosis MH - Republic of Korea/epidemiology MH - Retrospective Studies MH - Salivary Glands/*immunology/pathology MH - Severity of Illness Index MH - Sjogren's Syndrome/blood/classification/diagnosis/epidemiology/*immunology OTO - NOTNLM OT - Sjögren's syndrome OT - anti-centromere antibody OT - autoantibodies EDAT- 2015/07/17 06:00 MHDA- 2016/06/09 06:00 CRDT- 2015/07/17 06:00 PHST- 2015/07/17 06:00 [entrez] PHST- 2015/07/17 06:00 [pubmed] PHST- 2016/06/09 06:00 [medline] AID - 10.1111/1756-185X.12684 [doi] PST - ppublish SO - Int J Rheum Dis. 2015 Sep;18(7):776-82. doi: 10.1111/1756-185X.12684. Epub 2015 Jul 14. PMID- 24217667 OWN - NLM STAT- MEDLINE DCOM- 20140929 LR - 20191112 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 53 IP - 4 DP - 2013 Aug TI - [Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ]. PG - 352-7 AB - OBJECTIVES: Due to the scarcity of studies in the literature, we conducted an analysis of a series of patients with the anti-PL-7, PL-12 and EJ types of antisynthetase syndrome (ASS). METHODS: We conducted a retrospective cohort study of 20 patients with ASS (8 with anti-PL-7, 6 with PL-12, 6 with EJ) monitored in our department between 1982 and 2012. RESULTS: The mean patient age at disease onset was 38.5 ± 12.9 years, and the disease duration was 4.5 ± 6.4 years. Of all the patients, 70% were white and 85% were female. Constitutional symptoms occurred in 90% of cases. All patients presented objective muscle weakness in the limbs; in addition, 30% were bedridden and 65% demonstrated high dysphagia at diagnosis. Joint and pulmonary involvement and Raynaud's phenomenon occurred in 50%, 40% and 65% of cases, respectively, with more than half of the patients presenting incipient pneumopathy, ground-glass opacity and/or pulmonary fibrosis. There were no cases of neurological and/or cardiac involvement. All patients received prednisone or other immunosuppressants depending on tolerance, side effects and/or disease refractoriness. Importantly, patients with the anti-EJ type of ASS demonstrated higher rates of recurrence. Two patients died during follow-up, and 1 patient had breast cancer at the time of diagnosis. CONCLUSIONS: ASS (anti-PL-7, PL-12 and EJ) was found to predominantly affect white women. Although the autoantibodies described in the present study are more related to pulmonary than joint involvement, our patients showed a significant percentage of both types of involvement and a high percentage of myopathy. We also observed a low mortality rate. FAU - Souza, Fernando Henrique Carlos de AU - Souza FH FAU - Cruellas, Marcela Gran Pina AU - Cruellas MG FAU - Levy-Neto, Mauricio AU - Levy-Neto M FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK LA - por PT - English Abstract PT - Journal Article PT - Research Support, Non-U.S. Gov't TT - Síndrome antissintetase: anti-PL-7, anti-PL-12 e anti-EJ. PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 RN - 0 (Antibodies) RN - EC 6.1.1.14 (Glycine-tRNA Ligase) RN - EC 6.1.1.3 (Threonine-tRNA Ligase) RN - EC 6.1.1.7 (Alanine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Adolescent MH - Adult MH - Alanine-tRNA Ligase/*immunology MH - Antibodies/*blood MH - Cohort Studies MH - Female MH - Glycine-tRNA Ligase/*immunology MH - Humans MH - Male MH - Middle Aged MH - Myositis/*blood/*immunology MH - Retrospective Studies MH - Threonine-tRNA Ligase/*immunology MH - Young Adult EDAT- 2013/11/13 06:00 MHDA- 2014/09/30 06:00 CRDT- 2013/11/13 06:00 PHST- 2012/07/20 00:00 [received] PHST- 2013/02/19 00:00 [accepted] PHST- 2013/11/13 06:00 [entrez] PHST- 2013/11/13 06:00 [pubmed] PHST- 2014/09/30 06:00 [medline] AID - S0482-5004(13)70100-1 [pii] AID - 10.1590/s0482-50042013000400007 [doi] PST - ppublish SO - Rev Bras Reumatol. 2013 Aug;53(4):352-7. doi: 10.1590/s0482-50042013000400007. PMID- 34822051 OWN - NLM STAT- MEDLINE DCOM- 20220524 LR - 20220524 IS - 1573-2630 (Electronic) IS - 0165-5701 (Linking) VI - 42 IP - 5 DP - 2022 May TI - Structural abnormalities associated with glaucoma using swept-source optical coherence tomography in patients with systemic sclerosis. PG - 1369-1380 LID - 10.1007/s10792-021-02124-1 [doi] AB - PURPOSE: Vasospasm represents an early event in systemic sclerosis (SSc). Ocular vasospasm may induce optic nerve head (ONH) damage and has been involved in the pathogenesis of glaucoma, especially normal-tension glaucoma (NTG). We aimed to investigate the presence of structural abnormalities associated with NTG using swept-source optical coherence tomography (SS-OCT) and to correlate the OCT parameters with clinical, capillaroscopy and digital blood flow measures in patients with SSc. METHODS: In this cross-sectional study, 40 patients with SSc and 23 age-matched controls were included. The following parameters were measured using SS-OCT: mean and sectoral retinal nerve fibre layer (RNFL) thickness, macular ganglion cell layer complex (GCC) thickness and ONH morphology. Nailfold capillaroscopy (NFC) and digital blood flow measurements using laser Doppler imaging (LDI) were performed in all subjects. RESULTS: Patients with SSc showed a thinner temporal RNFL than the controls (69.23 ± 11.74 versus 83.35 ± 20.19 µm, p = 0.001). The other parameters were similar between the two groups. In SSc patients, there was an inverse correlation between the disease duration and the average, superior and inferior RNFL thickness and the GCC thickness and between Raynaud's phenomenon duration and the average RNFL and GCC thickness (p < 0.05). NFC and LDI measurements did not show correlations with OCT parameters. CONCLUSION: A thinner temporal RNFL and the correlation between Raynaud's phenomenon and disease duration and structural abnormalities on OCT suggest the presence of early ganglion cell damage in patients with SSc. Although mild, these findings indicate the need to monitor ocular abnormalities in SSc. CI - © 2021. The Author(s), under exclusive licence to Springer Nature B.V. FAU - Agapito Tito, Cecilia Victoria AU - Agapito Tito CV AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Rua Botucatu 740, 3° andar, São Paulo, SP, 04023-062, Brazil. FAU - Silvatti, Juliana AU - Silvatti J AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Rua Botucatu 740, 3° andar, São Paulo, SP, 04023-062, Brazil. FAU - de Almeida, Izabela N F AU - de Almeida INF AD - Department of Ophthalmology and Visual Science, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Taniguchi, Elise V AU - Taniguchi EV AD - Department of Ophthalmology and Visual Science, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Prata, Tiago S AU - Prata TS AD - Department of Ophthalmology and Visual Science, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Paranhos, Augusto Jr AU - Paranhos A Jr AD - Department of Ophthalmology and Visual Science, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AUID- ORCID: 0000-0003-0543-5305 AD - Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Rua Botucatu 740, 3° andar, São Paulo, SP, 04023-062, Brazil. cristiane.kayser@unifesp.br. LA - eng PT - Journal Article DEP - 20211125 PL - Netherlands TA - Int Ophthalmol JT - International ophthalmology JID - 7904294 SB - IM MH - Cross-Sectional Studies MH - *Glaucoma/diagnosis/etiology/pathology MH - Humans MH - Intraocular Pressure MH - *Low Tension Glaucoma/diagnosis MH - Nerve Fibers/pathology MH - Retinal Ganglion Cells/pathology MH - *Scleroderma, Systemic/complications/diagnosis MH - Tomography, Optical Coherence/methods MH - Visual Fields OTO - NOTNLM OT - Normal-tension glaucoma OT - Optical coherence tomography OT - Retinal nerve fibre layer thickness OT - Systemic sclerosis EDAT- 2021/11/26 06:00 MHDA- 2022/05/25 06:00 CRDT- 2021/11/25 12:21 PHST- 2021/01/13 00:00 [received] PHST- 2021/11/12 00:00 [accepted] PHST- 2021/11/26 06:00 [pubmed] PHST- 2022/05/25 06:00 [medline] PHST- 2021/11/25 12:21 [entrez] AID - 10.1007/s10792-021-02124-1 [pii] AID - 10.1007/s10792-021-02124-1 [doi] PST - ppublish SO - Int Ophthalmol. 2022 May;42(5):1369-1380. doi: 10.1007/s10792-021-02124-1. Epub 2021 Nov 25. PMID- 19950328 OWN - NLM STAT- MEDLINE DCOM- 20100121 LR - 20260518 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 61 IP - 12 DP - 2009 Dec 15 TI - Prevalence and clinical correlates of pruritus in patients with systemic sclerosis. PG - 1765-70 LID - 10.1002/art.25010 [doi] AB - OBJECTIVE: There are no studies of pruritus prevalence or clinical correlates in systemic sclerosis (SSc). The objectives of this study were to document the proportion of SSc patients with pruritus on most days, to determine when in the course of the disease pruritus is most prevalent, and to identify clinical correlates. METHODS: We performed a cross-sectional, multicenter study of 400 SSc patients from the Canadian Scleroderma Research Group Registry > or =1 year after Registry enrollment. Patients indicated whether they experienced pruritus during the past month on most days and underwent clinical histories and medical examinations. Multiple logistic regression was used to assess the association between sociodemographic and clinical variables and pruritus. RESULTS: A total of 179 patients (45%) reported pruritus on most days, including 69% (11 of 16) among patients 1.0-1.9 years from onset of non-Raynaud's symptoms, 41% (38 of 93) for 2.0-4.9 years, 47% (44 of 94) for 5.0-9.9 years, 43% (60 of 140) for 10.0-19.9 years, and 46% (26 of 57) for > or =20 years. In post hoc analysis, patients 1.0-1.9 years from disease onset were significantly more likely to report pruritus (P = 0.049). Patients with pruritus had significantly more skin involvement (P = 0.029), more gastrointestinal (GI) symptoms (P < 0.001), worse breathing problems (P = 0.001), worse Raynaud's symptoms (P = 0.002), and more severe finger ulcers (P = 0.009). Only the number of GI symptoms predicted pruritus in multiple logistic regression analysis (odds ratio 1.25, 95% confidence interval 1.13-1.37; P < 0.001). CONCLUSION: Pruritus is common in SSc and is independently associated with GI symptoms. Focused research on sources of pruritus and its management in SSc is needed. FAU - Razykov, Ilya AU - Razykov I AD - McGill University and Jewish General Hospital, Montreal, Quebec, Canada. FAU - Thombs, Brett D AU - Thombs BD FAU - Hudson, Marie AU - Hudson M FAU - Bassel, Marielle AU - Bassel M FAU - Baron, Murray AU - Baron M CN - Canadian Scleroderma Research Group LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM MH - Canada/epidemiology MH - Comorbidity MH - Cross-Sectional Studies MH - Female MH - Gastrointestinal Diseases/complications/*epidemiology/physiopathology MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Prevalence MH - Pruritus/complications/*epidemiology/physiopathology MH - Scleroderma, Systemic/complications/*epidemiology/physiopathology MH - Skin Diseases/complications/*epidemiology/physiopathology FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Khalidi, N IR - Khalidi N FIR - Kaminska, E IR - Kaminska E FIR - Robinson, D IR - Robinson D FIR - Mittoo, S IR - Mittoo S FIR - Jones, N IR - Jones N FIR - Masetto, A IR - Masetto A FIR - Sutton, E IR - Sutton E FIR - Docherty, P IR - Docherty P FIR - Mathieu, J -P IR - Mathieu J- FIR - Ligier, S IR - Ligier S FIR - Abu-Hakima, M IR - Abu-Hakima M FIR - LeClercq, S IR - LeClercq S FIR - Smith, D IR - Smith D EDAT- 2009/12/02 06:00 MHDA- 2010/01/22 06:00 CRDT- 2009/12/02 06:00 PHST- 2009/12/02 06:00 [entrez] PHST- 2009/12/02 06:00 [pubmed] PHST- 2010/01/22 06:00 [medline] AID - 10.1002/art.25010 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Dec 15;61(12):1765-70. doi: 10.1002/art.25010. PMID- 41858696 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260320 LR - 20260320 IS - 2514-2119 (Electronic) IS - 2514-2119 (Linking) VI - 10 IP - 3 DP - 2026 Mar TI - When atrioventricular block paves the way to a more severe diagnosis: a case report. PG - ytag152 LID - 10.1093/ehjcr/ytag152 [doi] LID - ytag152 AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by widespread fibrosis of skin and internal organs. Cardiac involvement is common but often subclinical, and advanced conduction abnormalities such as complete atrioventricular block are rarely the initial manifestation. CASE SUMMARY: A 42-year-old man presented with fatigue and episodes of syncope. Electrocardiography confirmed complete AV block. Physical examination revealed diffuse skin tightening and Raynaud's phenomenon. Immunological testing detected high-titre antinuclear antibodies and anti-topoisomerase I, confirming diffuse cutaneous SSc. Further evaluation disclosed multiorgan involvement: high-resolution CT showed pulmonary fibrosis and right heart catheterization confirmed severe precapillary pulmonary hypertension. The patient underwent permanent dual-chamber pacemaker implantation and was started on immunosuppressive therapy and pulmonary vasodilators. On follow-up, his bradycardia resolved and exercise tolerance improved, indicating partial clinical improvement. DISCUSSION: This case highlights an atypical cardiac onset of diffuse SSc with high-grade conduction disease. It underscores the importance of considering systemic autoimmune disorders in patients with unexplained high-degree AV block, especially in the presence of characteristic skin findings. Comprehensive assessment revealed concurrent pulmonary and gastrointestinal involvement, reflecting the multisystem nature of SSc. Early recognition allowed a coordinated multidisciplinary treatment strategy, including device implantation and disease-modifying therapy, which ultimately stabilized the patient's condition. This case emphasizes the need for early diagnosis and integrated management of cardiac, pulmonary, and gastrointestinal complications in SSc. Although SSc is generally progressive, prompt recognition and treatment of organ involvement may significantly improve patient outcomes and quality of life. CI - © The Author(s) 2026. Published by Oxford University Press on behalf of the European Society of Cardiology. FAU - Argint, Ecaterina AU - Argint E AUID- ORCID: 0000-0002-8222-5507 AD - Department of Cardiology, Nicolae Testemițanu State University of Medicine and Pharmacy, 165 Ștefan cel Mare și Sfânt Boulevard, Chișinău, Republic of Moldova. FAU - Cheibaș, Dorina AU - Cheibaș D AUID- ORCID: 0009-0004-8792-3808 AD - Republican Clinical Hospital 'Timofei Moșneaga', 29 Nicolae Testemițanu Street, Chișinău, Republic of Moldova. FAU - Ochișor, Viorica AU - Ochișor V AUID- ORCID: 0000-0002-9185-7960 AD - Department of Cardiology, Nicolae Testemițanu State University of Medicine and Pharmacy, 165 Ștefan cel Mare și Sfânt Boulevard, Chișinău, Republic of Moldova. FAU - Revenco, Valeriu AU - Revenco V AUID- ORCID: 0000-0002-9419-025X AD - Department of Cardiology, Nicolae Testemițanu State University of Medicine and Pharmacy, 165 Ștefan cel Mare și Sfânt Boulevard, Chișinău, Republic of Moldova. FAU - Agapii, Mihaela AU - Agapii M AUID- ORCID: 0009-0003-9598-3002 AD - Department of Cardiology, Nicolae Testemițanu State University of Medicine and Pharmacy, 165 Ștefan cel Mare și Sfânt Boulevard, Chișinău, Republic of Moldova. LA - eng PT - Case Reports PT - Journal Article DEP - 20260309 PL - England TA - Eur Heart J Case Rep JT - European heart journal. Case reports JID - 101730741 PMC - PMC12998533 OTO - NOTNLM OT - Case report OT - Complete atrioventricular block OT - Gastrointestinal involvement OT - Pulmonary arterial hypertension OT - Pulmonary fibrosis OT - Systemic sclerosis COIS- Conflict of interest: The authors declare no conflicts of interest in relation to the content of this article. EDAT- 2026/03/20 07:10 MHDA- 2026/03/20 07:11 PMCR- 2026/03/09 CRDT- 2026/03/20 04:47 PHST- 2025/10/17 00:00 [received] PHST- 2026/01/20 00:00 [revised] PHST- 2026/03/02 00:00 [accepted] PHST- 2026/03/20 07:11 [medline] PHST- 2026/03/20 07:10 [pubmed] PHST- 2026/03/20 04:47 [entrez] PHST- 2026/03/09 00:00 [pmc-release] AID - ytag152 [pii] AID - 10.1093/ehjcr/ytag152 [doi] PST - epublish SO - Eur Heart J Case Rep. 2026 Mar 9;10(3):ytag152. doi: 10.1093/ehjcr/ytag152. eCollection 2026 Mar. PMID- 35911366 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220802 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 6 DP - 2022 Jun TI - Looks Like Neurosyphilis, Feels Like Guillain-Barre: At the Confluence of Infection and Immunology. PG - e26318 LID - 10.7759/cureus.26318 [doi] LID - e26318 AB - We present a 51-year-old male, with a past medical history of type 2 insulin-dependent diabetes mellitus (T2IDDM) without neuropathy, coronavirus disease 2019 (COVID-19) in April 2020 without residual symptoms, Raynaud's, and recent occupational outdoor exposure to insects as a construction manager who came to the emergency room complaining of a three-week history of bilateral progressive numbness and weakness beginning in his lower extremities and ascending toward his pelvis. Notably, he received the second dose of his Moderna COVID-19 vaccine one week prior to symptom onset and four weeks prior to admission. He also reported a recent appearance of a maculopapular rash on his upper extremities and flanks. Physical exam was remarkable for bilateral distal motor weakness in the upper and lower extremities with associated paresthesia and decreased reflexes in the lower extremities. The patient had slight ataxia and difficulty with heel walk and toe walk. Notably, the cranial nerve exam was normal, and the patient was afebrile. Intravenous immune globulin (IVIG) was started empirically for the treatment of Guillain-Barre syndrome (GBS), and doxycycline 100mg intravenous twice a day and ceftriaxone 2g intravenous daily were started for possible tick-borne disease. Subsequently, rapid plasma reagin (RPR) returned reactive at 1:64, and cerebral spinal fluid (CSF) venereal disease research laboratory (VDRL) test was reactive at 1:2 with markedly elevated protein and pleocytosis. Human immunodeficiency virus (HIV) testing was negative. Lyme disease testing was negative. Nerve conduction studies (NCS) and electromyography (EMG) showed a sensorimotor polyneuropathy with mixed demyelinating and axonal features. IVIG was continued for a total of five days, and antibiotics were changed to penicillin G (PCN G) for a total of 14 days for definitive treatment of early neurosyphilis (NS). While both clinical and laboratory findings confirm a positive diagnosis of NS, the patient's CSF composition showed very elevated total protein levels and pleocytosis. Additionally, his early peripheral neuropathy and EMG findings are not characteristics of a single disease and, instead, suggested a mixed pathology. We postulate that this patient had confirmed secondary syphilis with early NS associated with, and possibly correlated with, a simultaneous episode of acute inflammatory demyelinating polyneuropathy (AIDP) and/or a vaccine-related phenomenon. CI - Copyright © 2022, Berger et al. FAU - Berger, Joseph I AU - Berger JI AD - Internal Medicine, St. John's Riverside Hospital, Yonkers, USA. FAU - Vernon, Kasun AU - Vernon K AD - Internal Medicine, St. John's Riverside Hospital, Yonkers, USA. FAU - Abdo, Farid AU - Abdo F AD - Radiology, St. John's Riverside Hospital, Yonkers, USA. FAU - Gulati, Sandeep AU - Gulati S AD - Neurology, St. John's Riverside Hospital, Yonkers, USA. FAU - Hariharan, Radhika AU - Hariharan R AD - Infectious Disease, St. John's Riverside Hospital, Yonkers, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20220625 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9314239 OTO - NOTNLM OT - covid-19 OT - csf OT - csf pleocytosis OT - emg OT - guillain-barre syndrome OT - neurosyphilis OT - rpr OT - syphilis OT - vaccine OT - vdrl COIS- The authors have declared that no competing interests exist. EDAT- 2022/08/02 06:00 MHDA- 2022/08/02 06:01 PMCR- 2022/06/25 CRDT- 2022/08/01 03:45 PHST- 2022/06/24 00:00 [accepted] PHST- 2022/08/01 03:45 [entrez] PHST- 2022/08/02 06:00 [pubmed] PHST- 2022/08/02 06:01 [medline] PHST- 2022/06/25 00:00 [pmc-release] AID - 10.7759/cureus.26318 [doi] PST - epublish SO - Cureus. 2022 Jun 25;14(6):e26318. doi: 10.7759/cureus.26318. eCollection 2022 Jun. PMID- 35896805 OWN - NLM STAT- MEDLINE DCOM- 20230228 LR - 20230228 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 43 IP - 3 DP - 2023 Mar TI - The diagnostic challenge of patients with anti-U1-RNP antibodies. PG - 509-521 LID - 10.1007/s00296-022-05161-w [doi] AB - Anti-U1-RNP antibodies are necessary for the diagnosis of mixed connective tissue disease (MCTD), but they are also prevalent in other connective tissue diseases, especially systemic lupus erythematosus (SLE), from which distinction remains challenging. We aimed to describe the presentation and outcome of patients with anti-U1-RNP antibodies and to identify factors to distinguish MCTD from SLE. We retrospectively applied the criteria sets for MCTD, SLE, systemic sclerosis (SSc) and rheumatoid arthritis (RA) to all patients displaying anti-U1-RNP antibodies in the hospital of Caen from 2000 to 2020. Thirty-six patients were included in the analysis. Eighteen patients (50%) satisfied at least one of the MCTD classifications, 11 of whom (61%) also met 2019 ACR/EULAR criteria for SLE. Twelve other patients only met SLE without MCTD criteria, and a total of 23 patients (64%) met SLE criteria. The most frequent manifestations included Raynaud's phenomenon (RP, 91%) and arthralgia (67%). We compared the characteristics of patients meeting only the MCTD (n = 7), SLE (n = 12), or both (n = 11) criteria. Patients meeting the MCTD criteria were more likely to display SSc features, including sclerodactyly (p < 0.01), swollen hands (p < 0.01), RP (p = 0.04) and esophageal reflux (p < 0.01). The presence of scleroderma features (swollen hands, sclerodactyly, gastro-oesophageal reflux), was significantly associated with the diagnosis of MCTD. Conversely, the absence of those manifestations suggested the diagnosis of another definite connective tissue disease, especially SLE. CI - © 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Elhani, Ines AU - Elhani I AUID- ORCID: 0000-0002-9767-2262 AD - Department of Internal Medicine, Caen University Hospital, Caen, France. FAU - Khoy, Kathy AU - Khoy K AUID- ORCID: 0000-0002-7021-1415 AD - Laboratory of Immunology, Department of Biology, Caen University Hospital, Caen, France. FAU - Mariotte, Delphine AU - Mariotte D AUID- ORCID: 0000-0002-9989-4272 AD - Laboratory of Immunology, Department of Biology, Caen University Hospital, Caen, France. FAU - Comby, Elisabeth AU - Comby E AD - Laboratory of Immunology, Department of Biology, Caen University Hospital, Caen, France. FAU - Marcelli, Christian AU - Marcelli C AUID- ORCID: 0000-0002-9699-2467 AD - Department of Rheumatology, Caen University Hospital, Caen, France. FAU - Le Mauff, Brigitte AU - Le Mauff B AUID- ORCID: 0000-0001-9242-4895 AD - Laboratory of Immunology, Department of Biology, Caen University Hospital, Caen, France. AD - UMR-S1237, Physiopathology and Imaging of Neurological Disorders, INSERM, Caen, France. AD - Normandie Université, UNICAEN, Caen, France. FAU - Audemard-Verger, Alexandra AU - Audemard-Verger A AUID- ORCID: 0000-0002-0226-5016 AD - Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France. AD - University of Tours, Tours, France. FAU - Boutemy, Jonathan AU - Boutemy J AD - Department of Internal Medicine, Caen University Hospital, Caen, France. FAU - Maigné, Gwénola AU - Maigné G AUID- ORCID: 0000-0002-3023-361X AD - Department of Internal Medicine, Caen University Hospital, Caen, France. FAU - Martin Silva, Nicolas AU - Martin Silva N AUID- ORCID: 0000-0002-1536-1373 AD - Department of Internal Medicine, Caen University Hospital, Caen, France. FAU - Aouba, Achille AU - Aouba A AUID- ORCID: 0000-0003-1726-3811 AD - Department of Internal Medicine, Caen University Hospital, Caen, France. AD - Normandie Université, UNICAEN, Caen, France. FAU - de Boysson, Hubert AU - de Boysson H AUID- ORCID: 0000-0001-9083-8365 AD - Department of Internal Medicine, Caen University Hospital, Caen, France. deboysson-h@chu-caen.fr. AD - Normandie Université, UNICAEN, Caen, France. deboysson-h@chu-caen.fr. LA - eng PT - Journal Article DEP - 20220727 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Retrospective Studies MH - *Mixed Connective Tissue Disease/diagnosis MH - Antibodies, Antinuclear MH - *Lupus Erythematosus, Systemic/diagnosis MH - *Scleroderma, Systemic MH - *Scleroderma, Localized MH - *Gastroesophageal Reflux OTO - NOTNLM OT - Anti-U1-RNP antibodies OT - Connective tissue disease OT - Mixed connective tissue disease OT - Systemic lupus erythematosus EDAT- 2022/07/28 06:00 MHDA- 2023/03/03 06:00 CRDT- 2022/07/27 23:36 PHST- 2021/11/21 00:00 [received] PHST- 2022/06/02 00:00 [accepted] PHST- 2022/07/28 06:00 [pubmed] PHST- 2023/03/03 06:00 [medline] PHST- 2022/07/27 23:36 [entrez] AID - 10.1007/s00296-022-05161-w [pii] AID - 10.1007/s00296-022-05161-w [doi] PST - ppublish SO - Rheumatol Int. 2023 Mar;43(3):509-521. doi: 10.1007/s00296-022-05161-w. Epub 2022 Jul 27. PMID- 40765531 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250808 IS - 1661-7649 (Print) IS - 1661-7657 (Electronic) IS - 1661-7649 (Linking) VI - 19 IP - 4 DP - 2025 Jul TI - Do the incidence and severity of lower urinary tract symptoms measured by the International Consultation on Incontinence Questionnaires correlate with urodynamic findings in patients with systemic sclerosis? PG - 274-279 LID - 10.1097/CU9.0000000000000208 [doi] AB - BACKGROUND: Systemic sclerosis (SSC) affects the urinary bladder and many other body organs. Systemic sclerosis commonly manifests as lower urinary tract symptoms (LUTS), which are usually reported using validated questionnaires. This study aimed to correlate questionnaire-reported LUTS with urodynamic findings in patients with SSC. MATERIALS AND METHODS: This cross-sectional study was performed at our center between August 2018 and July 2021. Lower urinary tract symptoms were assessed using the International Consultation on Incontinence Questionnaire (ICIQ) Female Lower Urinary Tract Symptoms Modules and ICIQ Male Lower Urinary Tract Symptoms Module and urodynamic studies. Univariate analyses were then performed on variables affecting detrusor activity. RESULTS: This study included 22 patients (5 men, 17 women). The mean ± standard deviation age, body mass index, and disease duration were 37.2 ± 11.1 years, 25.5 ± 4.2 kg/m(2), and 6.2 ± 5.3 years, respectively. Lower urinary tract symptoms occurred in 19 (86.4%) patients, of whom 18 (94.7%) had mild to moderate symptoms. The ICIQ scores for voiding and filling symptoms were higher than those for incontinence. The most frequent organ involvements included Raynaud's phenomenon in 22 (100%), skin conditions in 20 (90.9%), gastrointestinal tract maladies in 17 (77.3%), joint disorders in 16 (72.7%), and lung diseases in 14 (63.6%) patients. Abnormal detrusor contractions occurred in only 5 patients (22.7%). Univariate analyses showed that disease duration <5 years (p = 0.010), nonobstructive uroflowmetry findings (p = 0.024), absence of incontinence (p = 0.024) and telangiectasia (p = 0.010), and negative rheumatoid factors (p = 0.043) were significantly associated with normal detrusor contractions. CONCLUSIONS: Mild to moderate severity of ICIQ-measured LUTS affected most patients with SSC but was not correlated with urodynamic findings. Normal detrusor contractions were significantly associated with shorter disease duration, nonobstructive uroflowmetry findings, absence of incontinence and telangiectasia, and negative rheumatoid factors. CI - Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. FAU - Faddan, Amr Abou AU - Faddan AA AD - Assiut Urology and Nephrology Hospital, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Hassanien, Manal AU - Hassanien M AD - Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Talaat, Esraa Ahmed AU - Talaat EA AD - Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assiut University, Assiut, Egypt. FAU - Gadelkareem, Rabea Ahmed AU - Gadelkareem RA AD - Assiut Urology and Nephrology Hospital, Faculty of Medicine, Assiut University, Assiut, Egypt. LA - eng PT - Journal Article DEP - 20230531 PL - United States TA - Curr Urol JT - Current urology JID - 101471188 PMC - PMC12321482 OTO - NOTNLM OT - Detrusor contractility OT - Filling symptoms OT - Lower urinary tract symptoms OT - Systemic sclerosis OT - Urodynamics COIS- The authors declare that they have no conflicts of interest. EDAT- 2025/08/06 06:28 MHDA- 2025/08/06 06:29 PMCR- 2025/07/01 CRDT- 2025/08/06 05:07 PHST- 2022/10/15 00:00 [received] PHST- 2023/03/01 00:00 [accepted] PHST- 2025/08/06 06:29 [medline] PHST- 2025/08/06 06:28 [pubmed] PHST- 2025/08/06 05:07 [entrez] PHST- 2025/07/01 00:00 [pmc-release] AID - CURR-UROL_230124 [pii] AID - 10.1097/CU9.0000000000000208 [doi] PST - ppublish SO - Curr Urol. 2025 Jul;19(4):274-279. doi: 10.1097/CU9.0000000000000208. Epub 2023 May 31. PMID- 29072596 OWN - NLM STAT- MEDLINE DCOM- 20180416 LR - 20260127 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 14 IP - 11 DP - 2017 Oct 26 TI - Environmental Pollution by Benzene and PM(10) and Clinical Manifestations of Systemic Sclerosis: A Correlation Study. LID - 10.3390/ijerph14111297 [doi] LID - 1297 AB - Atmospheric air pollution has been associated with a range of adverse health effects. The environment plays a causative role in the development of Systemic Sclerosis (SSc). The aim of the present study is to explore the association between particulate (PM(10)) and benzene (B) exposure in Italian patients with systemic sclerosis and their clinical characteristics of the disease. A correlation study was conducted by enrolling 88 patients who suffer from SSc at the Fondazione Policlinico "A. Gemelli" in Rome (Italy) in the period from January 2013 to January 2014. The average mean concentrations of B (in 11 monitoring sites) and PM(10) (in 14 sites) were calculated using data from the Regional Environmental Protection Agency's monitoring stations located throughout the Lazio region (Italy) and then correlated with the clinical characteristics of the SSc patients. Of the study sample, 92.5% were female. The mean age was 55 ± 12.9 years old and the mean disease duration from the onset of Raynaud's phenomenon was 13.0 ± 9.4 years. The Spearman's correlation showed that concentrations of B correlate directly with the skin score (R = 0.3; p ≤ 0.05) and inversely with Diffusing Lung Carbon Monoxide (DLCO) results (R = -0.36; p = 0.04). This study suggests a possible role of B in the development of diffuse skin disease and in a worse progression of the lung manifestations of SSc. FAU - Borghini, Alice AU - Borghini A AD - Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy. alice.borghini01@icatt.it. FAU - Poscia, Andrea AU - Poscia A AD - Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy. andrea.poscia@unicatt.it. FAU - Bosello, Silvia AU - Bosello S AD - Department of Rheumatology, Fondazione Policlinico Gemelli, 00168 Rome, Italy. silvia.bosello@libero.it. FAU - Teleman, Adele Anna AU - Teleman AA AD - Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy. ateleman@hotmail.com. FAU - Bocci, Mario AU - Bocci M AD - Department of Rheumatology, Fondazione Policlinico Gemelli, 00168 Rome, Italy. mariobocci@gmail.com. FAU - Iodice, Lanfranco AU - Iodice L AD - Vice Medical Director, San Raffaele Cassino Hospital, 03043 Cassino (FR), Italy. lanfranco.iodice@sanraffaele.it. FAU - Ferraccioli, Gianfranco AU - Ferraccioli G AD - Department of Rheumatology, Fondazione Policlinico Gemelli, 00168 Rome, Italy. gff1990@gmail.com. FAU - La Milìa, Daniele Ignazio AU - La Milìa DI AD - Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy. daniele.lamilia@gmail.com. FAU - Moscato, Umberto AU - Moscato U AD - Institute of Public Health-Hygiene Section, Università Cattolica del Sacro Cuore, 00168 Roma, Italy. umberto.moscato@unicatt.it. LA - eng PT - Journal Article DEP - 20171026 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 RN - 0 (Air Pollutants) RN - 0 (Particulate Matter) RN - 7U1EE4V452 (Carbon Monoxide) RN - J64922108F (Benzene) SB - IM MH - Adult MH - Aged MH - Air Pollutants/*analysis MH - Air Pollution/analysis MH - Benzene/*analysis MH - Carbon Monoxide/metabolism MH - Environmental Monitoring/methods MH - Female MH - Humans MH - Lung/metabolism MH - Male MH - Middle Aged MH - Particulate Matter/*analysis MH - Rome/epidemiology MH - Scleroderma, Systemic/*epidemiology PMC - PMC5707936 OTO - NOTNLM OT - benzene OT - environmental exposure OT - particulate matter (PM10) OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2017/10/27 06:00 MHDA- 2018/04/17 06:00 PMCR- 2017/11/01 CRDT- 2017/10/27 06:00 PHST- 2017/09/22 00:00 [received] PHST- 2017/10/13 00:00 [revised] PHST- 2017/10/17 00:00 [accepted] PHST- 2017/10/27 06:00 [entrez] PHST- 2017/10/27 06:00 [pubmed] PHST- 2018/04/17 06:00 [medline] PHST- 2017/11/01 00:00 [pmc-release] AID - ijerph14111297 [pii] AID - ijerph-14-01297 [pii] AID - 10.3390/ijerph14111297 [doi] PST - epublish SO - Int J Environ Res Public Health. 2017 Oct 26;14(11):1297. doi: 10.3390/ijerph14111297. PMID- 18032543 OWN - NLM STAT- MEDLINE DCOM- 20080111 LR - 20071122 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 46 IP - 12 DP - 2007 Dec TI - Sjögren's Systemic Clinical Activity Index (SCAI)--a systemic disease activity measure for use in clinical trials in primary Sjögren's syndrome. PG - 1845-51 AB - OBJECTIVE: This article describes the development of the Sjögren's Systemic Clinical Activity Index (SCAI) for the measurement of systemic disease activity in patients with primary Sjögren's syndrome (PSS). METHODS: A pilot tool was developed based on expert consensus and previous published data. One hundred and four patients with PSS were evaluated in a cross-sectional analysis, of whom 65 were reviewed at 3-monthly intervals, using this index, over a 12-month period. Factor analysis was used to evaluate the proposed domain structure. External validation was assessed by comparison with relevant domains of the Profile of Fatigue and Discomfort (PROFAD), Medical Outcomes Study Short Form-36 (SF-36) and The World Health Organization Quality of Life-Bref (WHOQOL-BREF). Sensitivity to change was assessed by comparing SCAI-derived flares with physician-designated disease flare and intention-to-treat analysis. A reliability and repeatability workshop was also held. RESULTS: Factor analysis supported the proposed domain structure. There were strong correlations between the SCAI fatigue, musculoskeletal and Raynaud's components and the PROFAD fatigue, arthralgia and vascular domains. There was a significant correlation between change in therapy and SCAI-defined flares (P = 0.01). The mean kappa-test results both for reliability of the SCAI and for physician repeatability were 0.71. CONCLUSION: This initial evaluation supports the potential for the SCAI as a tool for systemic activity assessment in patients with PSS but additional work is required to assess sensitivity to change in clinical therapeutic trials. FAU - Bowman, S J AU - Bowman SJ AD - Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Selly Oak, Birmingham B29 6JD, UK. simon.bowman@uhb.nhs.uk FAU - Sutcliffe, N AU - Sutcliffe N FAU - Isenberg, D A AU - Isenberg DA FAU - Goldblatt, F AU - Goldblatt F FAU - Adler, M AU - Adler M FAU - Price, E AU - Price E FAU - Canavan, A AU - Canavan A FAU - Hamburger, J AU - Hamburger J FAU - Richards, A AU - Richards A FAU - Rauz, S AU - Rauz S FAU - Regan, M AU - Regan M FAU - Gadsby, K AU - Gadsby K FAU - Rigby, S AU - Rigby S FAU - Jones, A AU - Jones A FAU - Mathew, R AU - Mathew R FAU - Mulherin, D AU - Mulherin D FAU - Stevenson, A AU - Stevenson A FAU - Nightingale, P AU - Nightingale P LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Aged MH - Clinical Trials as Topic MH - Cross-Sectional Studies MH - Fatigue/*diagnosis MH - Female MH - Humans MH - Middle Aged MH - Pilot Projects MH - Probability MH - Reproducibility of Results MH - Sensitivity and Specificity MH - Severity of Illness Index MH - *Sickness Impact Profile MH - Sjogren's Syndrome/classification/*diagnosis MH - Time Factors EDAT- 2007/11/23 09:00 MHDA- 2008/01/12 09:00 CRDT- 2007/11/23 09:00 PHST- 2007/11/23 09:00 [pubmed] PHST- 2008/01/12 09:00 [medline] PHST- 2007/11/23 09:00 [entrez] AID - 46/12/1845 [pii] AID - 10.1093/rheumatology/kem280 [doi] PST - ppublish SO - Rheumatology (Oxford). 2007 Dec;46(12):1845-51. doi: 10.1093/rheumatology/kem280. PMID- 41929488 OWN - NLM STAT- MEDLINE DCOM- 20260403 LR - 20260403 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Deciphering the interferon gene signature spectrum: association with the clinical heterogeneity of Sjögren's disease. PG - 1762625 LID - 10.3389/fimmu.2026.1762625 [doi] LID - 1762625 AB - PURPOSE: This study aimed to identify interferon (IFN)-related key genes in patients with Sjögren's Disease (SjD) and to elucidate their specific associations with the heterogeneous clinical phenotypes and laboratory parameters of the disease. METHODS: Bioinformatics analyses, including differentially expressed gene (DEG) screening, weighted gene co-expression network analysis (WGCNA), and machine learning, were conducted on dataset GSE84844 to identify IFN-related key genes. Based on the EULAR Sjögren's syndrome disease activity index (ESSDAI), SjD patients with low, moderate, and high disease activity were enrolled, with 20 in each subgroup. Quantitative real-time polymerase chain reaction (qRT-PCR) was performed on peripheral blood mononuclear cells (PBMCs) from the 60 SjD patients and 15 healthy controls (HCs). Expression levels were compared between SjD patients and HCs, across disease activity subgroups, and correlated with clinical phenotypes and laboratory indicators. RESULTS: DEGs in SjD were significantly enriched in IFN-related signaling pathways. Five IFN-related hub genes were identified: CXCL10, DDX60L, IFIH1, JAK2, and NMI. qRT-PCR validation confirmed that all five genes were significantly upregulated in SjD patients compared to HCs (P < 0.05). However, their expression did not significantly differ among SjD subgroups with varying levels of overall disease activity (P > 0.05). Importantly, these genes were differentially expressed in distinct clinical manifestations. For instance, elevated CXCL10 expression was observed in patients with interstitial lung disease (ILD), leukopenia, and anemia; JAK2 expression differed in rheumatoid arthritis comorbidity; IFIH1 expression also showed differences in those with Raynaud's phenomenon and ILD. Furthermore, certain genes were highly expressed in specific laboratory abnormalities: elevated erythrocyte sedimentation rate with CXCL10 and JAK2; hyperglobulinemia with CXCL10, DDX60L, IFIH1, and JAK2; and elevated immunoglobulin G with CXCL10, DDX60L, and IFIH1 (all P < 0.05). CONCLUSION: This study identifies five IFN-related key genes (CXCL10, DDX60L, IFIH1, JAK2, and NMI) that are upregulated in SjD. Their expression patterns are not generalized markers of disease activity but are specifically linked to distinct clinical phenotypes and serological abnormalities. These findings provide novel mechanistic insights into the clinical heterogeneity of SjD and highlight CXCL10, JAK2, and IFIH1 as potential biomarkers for specific disease complications and promising candidates for targeted therapeutic strategies. CI - Copyright © 2026 Luo, Chen, Huang, Zhang, Chen and Wang. FAU - Luo, Ziyue AU - Luo Z AD - School of Basic Medical Sciences, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Chen, Ai AU - Chen A AD - Second Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Huang, Yue AU - Huang Y AD - Second Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Zhang, Kaiyuan AU - Zhang K AD - School of Basic Medical Sciences, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Chen, Muzhi AU - Chen M AD - Department of Rheumatology, The Second Affiliated Hospital, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Wang, Xinchang AU - Wang X AD - Department of Rheumatology, The Second Affiliated Hospital, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. LA - eng PT - Journal Article DEP - 20260318 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 9008-11-1 (Interferons) RN - 0 (Chemokine CXCL10) RN - 0 (CXCL10 protein, human) SB - IM MH - Humans MH - *Sjogren's Syndrome/genetics/immunology/diagnosis MH - *Interferons/genetics MH - Gene Expression Profiling MH - Female MH - *Transcriptome MH - Male MH - Middle Aged MH - Computational Biology/methods MH - Gene Regulatory Networks MH - Chemokine CXCL10/genetics MH - Adult MH - Signal Transduction MH - Leukocytes, Mononuclear/immunology/metabolism PMC - PMC13038951 OTO - NOTNLM OT - Sjögren’s disease OT - disease activity OT - heterogeneous OT - interferon OT - transcriptomics COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/04/03 06:30 MHDA- 2026/04/03 06:31 PMCR- 2026/03/18 CRDT- 2026/04/03 05:00 PHST- 2025/12/07 00:00 [received] PHST- 2026/02/24 00:00 [revised] PHST- 2026/03/03 00:00 [accepted] PHST- 2026/04/03 06:31 [medline] PHST- 2026/04/03 06:30 [pubmed] PHST- 2026/04/03 05:00 [entrez] PHST- 2026/03/18 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1762625 [doi] PST - epublish SO - Front Immunol. 2026 Mar 18;17:1762625. doi: 10.3389/fimmu.2026.1762625. eCollection 2026. PMID- 36017206 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220827 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 37 IP - 2 DP - 2022 Jun TI - Disease characteristics in patients with juvenile- and adult-onset systemic lupus erythematosus: A multi-center comparative study. PG - 280-287 LID - 10.46497/ArchRheumatol.2022.8888 [doi] AB - OBJECTIVES: This study aims to compare disease characteristics in patients with juvenile-onset systemic lupus erythematosus (JSLE) and adult-onset systemic lupus erythematosus (ASLE). PATIENTS AND METHODS: Between June 2010 and March 2020, a total of 186 patients with JSLE (23 males, 163 females; median age: 25 years; range, 20 to 30.3 years) and 236 patients with ASLE (23 males, 213 females; median age: 35 years; range, 29 to 40 years) were retrospectively analyzed. Clinical and laboratory data, treatment received, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index (SDI) scores, comorbidities and deaths were compared between the groups. RESULTS: The JSLE patients showed statistically significant higher constitutional manifestations, cardiac manifestations, serositis, nephritis, end-stage renal disease, neurological manifestations, gastrointestinal manifestations, secondary vasculitis, Raynaud's, livedo-reticularis, dry mouth, dry eye, ocular manifestations, avascular necrosis, hematological manifestations, and hypocomplementemia (p<0.001, p=0.016, p=0.005, p=0.001, p=0.04, p<0.001, p<0.001, p<0.001, p=0.002, p=0.043, p=0.004, p=0.03, p<0.001, p=0.01, p<0.001, and p=0.001, respectively). Median SLEDAI scores were statistically significant higher in the JSLE group, both at onset (p<0.001) and in the final follow-up visit (p<0.001). Median SLICC scores were also higher in the JSLE group (p<0.001). Mycophenolate mofetil and intravenous pulse steroids were more frequently used in the juvenile group (p<0.001 and p=0.03, respectively). Hypertension, dyslipidemia, and avascular necrosis were found to be statistically significantly higher in the JSLE group (p<0.001, p=0.006, and p=0.01, respectively). The mortality rate was statistically significantly higher in the JSLE group than the ASLE group (p<0.001). CONCLUSION: The JSLE patients showed more serious manifestations, higher disease activity, higher damage index, and mortality rate compared to ASLE patients. These results suggest the need of a regular follow-up and close surveillance of JSLE patients. CI - Copyright © 2022, Turkish League Against Rheumatism. FAU - Gamal, Sherif M AU - Gamal SM AUID- ORCID: 0000-0002-2663-4111 AD - Rheumatology, Faculty of Medicine, Cairo University, Cairo, Egypt. FAU - Fouad, Nermeen AU - Fouad N AUID- ORCID: 0000-0002-6461-4939 AD - Rheumatology, Faculty of Medicine, Fayoum University, Fayoum, Egypt. FAU - Yosry, Nora AU - Yosry N AUID- ORCID: 0000-0001-9432-2403 AD - Rheumatology, Faculty of Medicine, Cairo University, Cairo, Egypt. FAU - Badr, Wael AU - Badr W AUID- ORCID: 0000-0002-9864-8758 AD - Department of Pediatric, Faculty of Medicine, Fayoum University, Fayoum, Egypt. FAU - Sobhy, Nesreen AU - Sobhy N AD - Rheumatology, Faculty of Medicine, Cairo University, Cairo, Egypt. LA - eng PT - Journal Article DEP - 20211224 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC9377179 OTO - NOTNLM OT - Adult-onset OT - juvenile-onset OT - surveillance OT - systemic lupus erythematosus COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2022/08/27 06:00 MHDA- 2022/08/27 06:01 PMCR- 2021/12/24 CRDT- 2022/08/26 02:28 PHST- 2021/04/12 00:00 [received] PHST- 2021/06/27 00:00 [accepted] PHST- 2022/08/26 02:28 [entrez] PHST- 2022/08/27 06:00 [pubmed] PHST- 2022/08/27 06:01 [medline] PHST- 2021/12/24 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2022.8888 [doi] PST - epublish SO - Arch Rheumatol. 2021 Dec 24;37(2):280-287. doi: 10.46497/ArchRheumatol.2022.8888. eCollection 2022 Jun. PMID- 33331330 OWN - NLM STAT- MEDLINE DCOM- 20201218 LR - 20201219 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 52 IP - 6 DP - 2020 Dec 18 TI - [Angioimmunoblastic T-cell lymphoma with fever, arthritis and skin pigmentation: A case report]. PG - 1150-1152 AB - Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoma. The clinical manifestations are not specific. In addition to the common clinical manifestations of lymphomas such as fever, weight loss, night sweats and lymphadenopathy, it may also have skin rashes, arthritis, multiple serous effusions, eosinophilia and other systemic inflammatory or immune symptoms. The lymphoma cells of angioimmunoblastic T-cell lymphoma originates from follicular helper T cells, and the follicular structure of lymph nodes disappears. In the tumor microenvironment, in addition to tumor cells, there are a large number of over-activated immune cells, such as abnormally activated B cells, which produce a series of systemic inflammation or immune-related symptoms. This disease is rare and difficult to diagnose. This article reports a 36-year-old female. She got fever, joint swelling and pain, skin pigmentation, accompanied by hepatomegaly, splenomegaly, lymphadenopathy, anemia and other multiple-systems manifestations. The clinical manifestations of this patient were similar to autoimmune diseases such as adult onset Still's disease, rheumatoid arthritis, and systemic sclerosis, which made the diagnosis difficult. At the beginning of the disease course, the patient got arthritis and fever. And her white blood cells were significantly increased. Adult onset Still's disease should be considered, but her multiple-systems manifestations could not be explained by adult onset Still's disease. And her arthritis of hands should be distinguished with rheumatoid arthritis. However, the patient's joint swelling could get better within 3-7 days, and there was no synovitis and bone erosion on joint imaging examination. The rheumatoid factor and anti-CCP antibody were negative. The diagnostic evidence for rheumatoid arthritis was insufficient. The patient's skin pigmentation and punctate depigmentation were similar to those of systemic sclerosis. But the patient had no Raynaud's phenomenon, and her sclerosis-related antibody was negative. The diagnostic evidence for systemic sclerosis was also insufficient. After 3 years, she was finally diagnosed with angioimmunoblastic T-cell lymphoma by lymph node biopsy aspiration. This case suggests that the clinical manifestations of angioimmunoblastic T-cell lymphoma are diverse, and some symptoms similar to immune diseases may appear. When the patient's clinical symptoms are atypical and immune diseases cannot explain the patient's condition, and further evidence should be sought to confirm the diagnosis. FAU - Cheng, G AU - Cheng G AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhang, X AU - Zhang X AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Yang, F AU - Yang F AD - Department of Department of Pathology, Peking University People's Hospital, Beijing 100044, China. FAU - Cheng, J Y AU - Cheng JY AD - Department of Endocrinology, Peking University People's Hospital, Beijing 100044, China. FAU - Liu, Y Y AU - Liu YY AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Case Reports PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Adult MH - Diagnosis, Differential MH - Female MH - Humans MH - *Immunoblastic Lymphadenopathy/diagnosis MH - *Lymphoma, T-Cell/complications/diagnosis MH - Skin Pigmentation MH - Tomography, X-Ray Computed MH - Tumor Microenvironment PMC - PMC7745261 OTO - NOTNLM OT - Angioimmunoblastic T-cell lymphoma OT - Clinical manifestation OT - Diagnosis EDAT- 2020/12/18 06:00 MHDA- 2020/12/19 06:00 PMCR- 2020/12/18 CRDT- 2020/12/17 08:42 PHST- 2020/12/17 08:42 [entrez] PHST- 2020/12/18 06:00 [pubmed] PHST- 2020/12/19 06:00 [medline] PHST- 2020/12/18 00:00 [pmc-release] AID - bjdxxbyxb-52-6-1150 [pii] AID - 10.19723/j.issn.1671-167X.2020.06.028 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1150-1152. doi: 10.19723/j.issn.1671-167X.2020.06.028. PMID- 21163125 OWN - NLM STAT- MEDLINE DCOM- 20110408 LR - 20220419 IS - 2542-5641 (Electronic) IS - 0366-6999 (Linking) VI - 123 IP - 22 DP - 2010 Nov TI - Clinical and prognostic characteristics of 573 cases of primary Sjögren's syndrome. PG - 3252-7 AB - BACKGROUND: Primary Sjögren's syndrome (pSS) is one of the autoimmune diseases with high incidence. There were several clinical investigations in Caucasian but seldom in Chinese. The aim of this study was to compare the difference of clinical manifestations, immunological features and prognosis of pSS between Caucasian and Chinese pSS patients. METHODS: Five hundred and seventy-three patients who fulfilled the 2002 international classification (criteria) for pSS from Peking Union Medical College Hospital between 1985 and 2006 were screened retrospectively and compared with other populations. RESULTS: (1) The study consisted of 524 (91%) female and 49 (9%) male patients (female: male = 10.7:1). Mean age at the onset of the disease was (39.0 ± 13.7) years and in 169 (29.5%) patients the disease onset occurred before the age of 30 years. The average duration from disease onset to pSS diagnosis was 48 months (range, 1 - 552 months). It had been shortened during the recent five years. (2) Dry mouth (84.5%) and dry eyes (70.0%) were the most common symptoms, significantly lower than foreign patients (P = 0.000). Two hundred and seventy-two (47.5%) patients presented with rampant caries, 160 (27.9%) with parotidomegaly. The positivity of xerostomia, xerophthalmia and salivary gland biopsy were 91.9%, 94.8% and 90.7%, respectively. (3) Systemic involvement occurred in 91.4% patients. Compared with studies done outside China, higher prevalence of fever 41.0%, myositis 4.9%, pericardial effusion 14.8%, pulmonary involvement 42.3%, renal involvement 33.5%, thyroid involvement 32.7%, pancrease involvement 5.6% (P < 0.01) and lower prevalence of fatigue, lymphadenectasis and Raynaud's phenomenon (P < 0.01) were seen. (4) Risk factors of death include pulmonary artery hypertension, liver damage and interstitial lung disease. CONCLUSIONS: Chinese pSS differs significantly from the non-Chinese cases in terms of the age of onset, systemic involvement, autoantibodies and proportional mortality rate. Lung and liver damage were found to be the highest risk factors of the disease prognosis. FAU - Lin, Dong-Fang AU - Lin DF AD - Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Yan, Shu-Min AU - Yan SM FAU - Zhao, Yan AU - Zhao Y FAU - Zhang, Wen AU - Zhang W FAU - Li, Meng-Tao AU - Li MT FAU - Zeng, Xiao-Feng AU - Zeng XF FAU - Zhang, Feng-Chun AU - Zhang FC FAU - Dong, Yi AU - Dong Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - China TA - Chin Med J (Engl) JT - Chinese medical journal JID - 7513795 SB - IM MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - Sjogren's Syndrome/diagnosis/*pathology/*physiopathology EDAT- 2010/12/18 06:00 MHDA- 2011/04/09 06:00 CRDT- 2010/12/18 06:00 PHST- 2010/12/18 06:00 [entrez] PHST- 2010/12/18 06:00 [pubmed] PHST- 2011/04/09 06:00 [medline] PST - ppublish SO - Chin Med J (Engl). 2010 Nov;123(22):3252-7. PMID- 18648520 OWN - NLM STAT- MEDLINE DCOM- 20080924 LR - 20240607 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 3 IP - 7 DP - 2008 Jul 16 TI - Molecular subsets in the gene expression signatures of scleroderma skin. PG - e2696 LID - 10.1371/journal.pone.0002696 [doi] LID - e2696 AB - BACKGROUND: Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production. METHODOLOGY AND FINDINGS: We analyzed the genome-wide patterns of gene expression with DNA microarrays in skin biopsies from distinct scleroderma subsets including 17 patients with systemic sclerosis (SSc) with diffuse scleroderma (dSSc), 7 patients with SSc with limited scleroderma (lSSc), 3 patients with morphea, and 6 healthy controls. 61 skin biopsies were analyzed in a total of 75 microarray hybridizations. Analysis by hierarchical clustering demonstrates nearly identical patterns of gene expression in 17 out of 22 of the forearm and back skin pairs of SSc patients. Using this property of the gene expression, we selected a set of 'intrinsic' genes and analyzed the inherent data-driven groupings. Distinct patterns of gene expression separate patients with dSSc from those with lSSc and both are easily distinguished from normal controls. Our data show three distinct patient groups among the patients with dSSc and two groups among patients with lSSc. Each group can be distinguished by unique gene expression signatures indicative of proliferating cells, immune infiltrates and a fibrotic program. The intrinsic groups are statistically significant (p<0.001) and each has been mapped to clinical covariates of modified Rodnan skin score, interstitial lung disease, gastrointestinal involvement, digital ulcers, Raynaud's phenomenon and disease duration. We report a 177-gene signature that is associated with severity of skin disease in dSSc. CONCLUSIONS AND SIGNIFICANCE: Genome-wide gene expression profiling of skin biopsies demonstrates that the heterogeneity in scleroderma can be measured quantitatively with DNA microarrays. The diversity in gene expression demonstrates multiple distinct gene expression programs in the skin of patients with scleroderma. FAU - Milano, Ausra AU - Milano A AD - Department of Genetics, Dartmouth Medical School, Hanover, New Hampshire, United States of America. FAU - Pendergrass, Sarah A AU - Pendergrass SA FAU - Sargent, Jennifer L AU - Sargent JL FAU - George, Lacy K AU - George LK FAU - McCalmont, Timothy H AU - McCalmont TH FAU - Connolly, M Kari AU - Connolly MK FAU - Whitfield, Michael L AU - Whitfield ML LA - eng GR - T32 AR007576/AR/NIAMS NIH HHS/United States GR - 76200-560801/HHMI/Howard Hughes Medical Institute/United States GR - AR007576/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20080716 PL - United States TA - PLoS One JT - PloS one JID - 101285081 SB - IM EIN - PLoS ONE. 2008;3(10). doi: 10.1371/annotation/05bed72c-c6f6-4685-a732-02c78e5f66c2 MH - Adult MH - Aged MH - Biopsy MH - Case-Control Studies MH - Female MH - Gene Expression Profiling MH - *Gene Expression Regulation MH - Humans MH - Male MH - Middle Aged MH - Oligonucleotide Array Sequence Analysis MH - Phenotype MH - Scleroderma, Diffuse/*genetics/*metabolism MH - Scleroderma, Limited/*genetics/*metabolism MH - Scleroderma, Systemic/*genetics/*metabolism PMC - PMC2481301 COIS- Competing Interests: The authors have declared that no competing interests exist. EDAT- 2008/07/24 09:00 MHDA- 2008/09/25 09:00 PMCR- 2008/07/16 CRDT- 2008/07/24 09:00 PHST- 2008/02/13 00:00 [received] PHST- 2008/06/17 00:00 [accepted] PHST- 2008/07/24 09:00 [pubmed] PHST- 2008/09/25 09:00 [medline] PHST- 2008/07/24 09:00 [entrez] PHST- 2008/07/16 00:00 [pmc-release] AID - 08-PONE-RA-03626R1 [pii] AID - 10.1371/journal.pone.0002696 [doi] PST - epublish SO - PLoS One. 2008 Jul 16;3(7):e2696. doi: 10.1371/journal.pone.0002696. PMID- 40799490 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250816 IS - 2693-7204 (Electronic) IS - 2693-7204 (Linking) VI - 5 IP - 1 DP - 2025 TI - Dense fine speckled antinuclear antibody patterns: Clinical correlations and implications. PG - 1-8 LID - 10.46439/rheumatology.5.027 [doi] AB - BACKGROUND/PURPOSE: The significance of Dense Fine Speckled (DFS) pattern Anti-nuclear antibodies (ANA) by indirect mmunofluorescence (IIF) is unclear in the existing research. We aimed to investigate associations between positive ANA with DFS pattern and multiple autoimmune and rheumatic conditions. METHODS: This retrospective study analyzed datasets from patients tested for ANA between August 2017 and August 2019. Comparisons were made between diagnostic categories and diseases for patients with negative ANA, positive ANA (any pattern), and positive ANA with DFS pattern. Relative risk (RR) was calculated for diagnostic categories and individual diseases. RESULTS: Of 13,845 ANA results, 65.8% were negative and 34.2% were positive, including 4.6% with DFS pattern. Among ANA positive DFS pattern patients, 10.6% had inflammatory arthritis, 20.6% had fibromyalgia/chronic pain syndrome/chronic fatigue, 13.3% had SARD and only 2.2% had atopic disorder. Comparing ANA positive other patterns and DFS pattern, specific diagnoses like seropositive RA, SLE, SSc, and UCTD were lower among ANA positive DFS pattern. Similarly, diagnoses of Raynaud's with SSc or UCTD were lower in DFS pattern patients compared to ANA positive with other patterns. The rate of other diagnoses including seronegative RA, IIM, SS, autoimmune thyroid disorder, and autoimmune hepatitis did not differ between other patterns and DFS pattern. CONCLUSION: The presence of DFS pattern cannot indiscriminately exclude the presence of SARD or rheumatic disease, as diagnoses including seronegative RA, IIM, SS, autoimmune thyroid disorder, or autoimmune hepatitis did not differ among ANA positive with other patterns and DFS pattern. FAU - Sapkota, Smarika AU - Sapkota S AD - Division of Rheumatology, Department of Medicine, National Jewish Health, Denver, CO 80206, USA. FAU - Crosson, John AU - Crosson J AD - Department of Lab Medicine and Pathology, University of Minnesota, Minneapolis, MN 55455, USA. FAU - Evans, Michael D AU - Evans MD AD - Biostatistical Design and Analysis Center, Clinical and Translational Science Institute, University of Minnesota, Minneapolis, MN 55414, USA. FAU - Robinson, Londyn AU - Robinson L AD - Division of Rheumatology, University of Washington, Seattle, WA 98195, USA. FAU - Lord, Adam AU - Lord A AD - Department of Family Medicine and Community Health, University of Minnesota, Minneapolis, MN 55455, USA. FAU - Kofoed, Benjamin AU - Kofoed B AD - Primary Care Track, Department of Internal Medicine, Emory University, Atlanta, GA 30322, USA. FAU - Molitor, Jerry A AU - Molitor JA AD - Division of Rheumatology, Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA. LA - eng GR - UM1 TR004405/TR/NCATS NIH HHS/United States PT - Journal Article PL - United States TA - Curr Rheumatol Res JT - Current rheumatology research JID - 9919014594806676 PMC - PMC12341086 MID - NIHMS2077901 OTO - NOTNLM OT - Anti-DFS70 antibodies OT - Anti-nuclear antibodies (ANA) OT - Dense fine speckled (DFS) pattern OT - Systemic Autoimmune Rheumatic Diseases (SARD) COIS- Conflicts of Interest There are no conflicts of interest by any of the authors. EDAT- 2025/08/13 06:30 MHDA- 2025/08/13 06:31 PMCR- 2025/08/12 CRDT- 2025/08/13 05:50 PHST- 2025/08/13 06:31 [medline] PHST- 2025/08/13 06:30 [pubmed] PHST- 2025/08/13 05:50 [entrez] PHST- 2025/08/12 00:00 [pmc-release] AID - 10.46439/rheumatology.5.027 [doi] PST - ppublish SO - Curr Rheumatol Res. 2025;5(1):1-8. doi: 10.46439/rheumatology.5.027. PMID- 38646215 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240426 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 3 DP - 2024 Mar TI - A Case of Palmoplantar Keratoderma in the Constellation of Connective Tissue Diseases. PG - e56531 LID - 10.7759/cureus.56531 [doi] LID - e56531 AB - Overlap syndrome is a clinical challenge and brings together a wide range of treatment options for the treating physician. Addressing each and every complaint of the patient is crucial. A 50-year-old female patient presented with skin thickening, blackening, and hyperkeratosis; dysphagia; joint pain; features of myopathy; Raynaud's phenomenon; and dry mouth. Inflammatory markers were raised along with a positive antinuclear antibody (ANA) with Golgi apparatus pattern, anti-Sjögren's-syndrome-related antigen A (anti-SSA)/Ro60 3+, anti-SSA/Ro52 3+, and anti-PM/Scl 2+ antibodies that suggested overlap syndrome. Although the patient had no respiratory complaints, a unique interstitial lung disease (ILD) pattern was noted during the evaluation. Skin manifestations were puzzling, but the histopathology analyses of skin biopsies taken from two different sites revealed distinguishing features of cutaneous lupus and dermatomyositis. Treatment with hydroxychloroquine, pilocarpine, nifedipine, methotrexate, and topical tacrolimus produced a dramatic improvement in the clinical features. This case highlights subtle and florid features of different autoimmune diseases. The hyperkeratotic skin changes were the most striking feature, but the whole evaluation process unveiled many rare presentations of known autoimmune conditions that can open doors to new areas of our understanding toward connective tissue diseases (CTDs). Our case report demonstrates significant heterogeneity in the ANA patterns, ILD patterns, clinical manifestations, and treatment approaches. CI - Copyright © 2024, Verma et al. FAU - Verma, Ishan AU - Verma I AD - General Medicine, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Dube, Amol H AU - Dube AH AD - General Medicine, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Kumbhalkar, Sunita AU - Kumbhalkar S AD - General Medicine, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Nagpure, Keshao AU - Nagpure K AD - General Medicine, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Sawatkar, Gitesh AU - Sawatkar G AD - Dermatology, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Chuadhari, Sachin R AU - Chuadhari SR AD - Pathology, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. FAU - Umredkar, Ashwini AU - Umredkar A AD - Radiodiagnosis, All India Institute of Medical Sciences, Nagpur, Nagpur, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20240320 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11026992 OTO - NOTNLM OT - anti-golgi apparatus ab OT - anti-pm/scl ab OT - hypomyopathic dermatomyositis OT - low-dose methotrexate OT - normal cpk OT - overlap syndromes OT - palmoplantar keratoderma OT - topical tacrolimus 0.1% COIS- The authors have declared that no competing interests exist. EDAT- 2024/04/22 06:45 MHDA- 2024/04/22 06:46 PMCR- 2024/03/20 CRDT- 2024/04/22 04:17 PHST- 2024/03/20 00:00 [accepted] PHST- 2024/04/22 06:46 [medline] PHST- 2024/04/22 06:45 [pubmed] PHST- 2024/04/22 04:17 [entrez] PHST- 2024/03/20 00:00 [pmc-release] AID - 10.7759/cureus.56531 [doi] PST - epublish SO - Cureus. 2024 Mar 20;16(3):e56531. doi: 10.7759/cureus.56531. eCollection 2024 Mar. PMID- 23918994 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130806 LR - 20220318 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 58 IP - 4 DP - 2013 Jul TI - Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations. PG - 255-68 LID - 10.4103/0019-5154.113930 [doi] AB - Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud's phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient's quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature. FAU - Viswanath, Vishalakshi AU - Viswanath V AD - Department of Dermatology, Rajiv Gandhi Medical College and CSM Hospital, Kalwa, Thane Municipal Corporation, India. FAU - Phiske, Meghana M AU - Phiske MM FAU - Gopalani, Vinay V AU - Gopalani VV LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3726870 OTO - NOTNLM OT - Endothelin receptor antagonists OT - PDE-5 inhibitors OT - fibrosis OT - immune activation OT - immunosuppressives OT - systemic sclerosis OT - vascular injury COIS- Conflict of Interest: Nil. EDAT- 2013/08/07 06:00 MHDA- 2013/08/07 06:01 PMCR- 2013/07/01 CRDT- 2013/08/07 06:00 PHST- 2013/08/07 06:00 [entrez] PHST- 2013/08/07 06:00 [pubmed] PHST- 2013/08/07 06:01 [medline] PHST- 2013/07/01 00:00 [pmc-release] AID - IJD-58-255 [pii] AID - 10.4103/0019-5154.113930 [doi] PST - ppublish SO - Indian J Dermatol. 2013 Jul;58(4):255-68. doi: 10.4103/0019-5154.113930. PMID- 22382348 OWN - NLM STAT- MEDLINE DCOM- 20130122 LR - 20220408 IS - 1499-2752 (Electronic) IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 39 IP - 4 DP - 2012 Apr TI - Differences between male and female systemic lupus erythematosus in a multiethnic population. PG - 759-69 LID - 10.3899/jrheum.111061 [doi] AB - OBJECTIVE: Male patients with systemic lupus erythematosus (SLE) are thought to be similar to female patients with SLE, but key clinical characteristics may differ. Comparisons were made between male and female patients with SLE in the Hopkins Lupus Cohort. METHODS: A total of 1979 patients in the Hopkins Lupus Cohort were included in the analysis. RESULTS: The cohort consisted of 157 men (66.2% white, 33.8% African American) and 1822 women (59.8% white, 40.2% African American). The mean followup was 6.02 years (range 0-23.73). Men were more likely than women to have disability, hypertension, thrombosis, and renal, hematological, and serological manifestations. Men were more likely to be diagnosed at an older age and to have a lower education level. Women were more likely to have malar rash, photosensitivity, oral ulcers, alopecia, Raynaud's phenomenon, or arthralgia. Men were more likely than women to have experienced end organ damage including neuropsychiatric, renal, cardiovascular, peripheral vascular disease, and myocardial infarction, and to have died. In general, differences between males and females were more numerous and striking in whites, especially with respect to lupus nephritis, abnormal serologies, and thrombosis. CONCLUSION: Our study suggests that there are major clinical differences between male and female patients with SLE. Differences between male and female patients also depend on ethnicity. Future SLE studies will need to consider both ethnicity and gender to understand these differences. FAU - Tan, Tze Chin AU - Tan TC AD - Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 7500, Baltimore, MD 21205, USA. FAU - Fang, Hong AU - Fang H FAU - Magder, Laurence S AU - Magder LS FAU - Petri, Michelle A AU - Petri MA LA - eng GR - R01 AR043727/AR/NIAMS NIH HHS/United States GR - R01AR043727/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20120301 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Cohort Studies MH - Comorbidity/trends MH - Female MH - Follow-Up Studies MH - Humans MH - Lupus Erythematosus, Systemic/*ethnology/mortality/*physiopathology MH - Male MH - Middle Aged MH - Racial Groups/*ethnology MH - *Sex Characteristics MH - Young Adult PMC - PMC3605704 MID - NIHMS450970 EDAT- 2012/03/03 06:00 MHDA- 2013/01/23 06:00 PMCR- 2013/03/22 CRDT- 2012/03/03 06:00 PHST- 2012/03/03 06:00 [entrez] PHST- 2012/03/03 06:00 [pubmed] PHST- 2013/01/23 06:00 [medline] PHST- 2013/03/22 00:00 [pmc-release] AID - jrheum.111061 [pii] AID - 10.3899/jrheum.111061 [doi] PST - ppublish SO - J Rheumatol. 2012 Apr;39(4):759-69. doi: 10.3899/jrheum.111061. Epub 2012 Mar 1. PMID- 33549777 OWN - NLM STAT- MEDLINE DCOM- 20211231 LR - 20221207 IS - 1615-5947 (Electronic) IS - 0890-5096 (Linking) VI - 74 DP - 2021 Jul TI - Objective Improvements in Peripheral Arterial Disease from Dorsal Root Ganglion Stimulation: A Case Series. PG - 519.e7-519.e16 LID - S0890-5096(21)00103-5 [pii] LID - 10.1016/j.avsg.2021.01.069 [doi] AB - BACKGROUND: The sympathetic nervous system (SNS) is important in the regulation of perfusion. Dorsal root ganglion stimulation (DRG-S) modulates sympathetic tone and is approved to treat complex regional pain syndrome, a disorder related to SNS dysfunction. We herein present 3 cases of DRG-S therapy to improve blood flow and symptoms of ischemia in peripheral arterial disease (PAD). METHODS: Patient 1 is a 44-year-old female with dry gangrene of the third and fourth digits of her right hand due to Raynaud's syndrome who was scheduled for amputation of the affected digits. DRG-S leads were placed at the right C6, 7, and 8 DRG. Pulse volume recordings (PVR) were measured at baseline and after DRG-S. Patient 2 is a 55-year-old female with a non-healing ulcer of her left foot secondary to PAD scheduled for a below the knee amputation who underwent a DRG-S trial with leads placed at the left L4 and L5 DRG followed by a spinal cord stimulation trial with leads placed at the T9-T10 spinal levels for comparison. Transcutaneous oximetry (TcPO2) was measured at baseline and after 3 days of each therapy. Patient 3 is a 69-year-old female with persistent left foot pain at rest secondary to PAD with DRG-S leads placed at the left L4 and S1 levels. RESULTS: All 3 patients experienced a significant reduction in pain with DRG-S, along with improvements in blood flow of the involved extremities, avoiding or limiting amputation. PVR improved dramatically with DRG-S in patient 1. A greater improvement in TcPO2 was seen with the DRG-S trial compared to spinal cord stimulation trial in patient 2. Patient 3 experienced an increase in walking distance and demonstrated long term efficacy and limb salvage at 32 months postimplantation. CONCLUSIONS: Modulation of SNS output from DRG-S through orthodromic and antidromic autonomic pathways is likely responsible for improving blood flow. DRG-S may be a treatment option for PAD. CI - Copyright © 2021 Elsevier Inc. All rights reserved. FAU - Chapman, Kenneth B AU - Chapman KB AD - Spine & Pain Institute of New York, New York City, NY; Department of Anesthesiology, New York University Langone Medical Center, New York City, NY; Zucker School of Medicine at Hofstra/Northwell, New York City, NY. Electronic address: chapmanken@spinepainny.com. FAU - Kloosterman, Jaap AU - Kloosterman J AD - College of Medicine, Radboud University, Nijmegen, The Netherlands. FAU - Schor, Jonathan A AU - Schor JA AD - Department of Surgery, Division of Vascular Surgery, Staten Island University Hospital, New York, NY. FAU - Girardi, George E AU - Girardi GE AD - Front Range Pain Medicine, Fort Collins, CO. FAU - van Helmond, Noud AU - van Helmond N AD - Spine & Pain Institute of New York, New York City, NY; Department of Anesthesiology, Cooper Medical School of Rowan University, Cooper University Hospital, Camden, NJ. FAU - Yousef, Tariq A AU - Yousef TA AD - Spine & Pain Institute of New York, New York City, NY. LA - eng PT - Case Reports PT - Journal Article DEP - 20210204 PL - Netherlands TA - Ann Vasc Surg JT - Annals of vascular surgery JID - 8703941 SB - IM MH - Adult MH - Aged MH - Amputation, Surgical MH - *Electric Stimulation Therapy MH - Female MH - *Ganglia, Spinal MH - *Hemodynamics MH - Humans MH - Ischemia/diagnosis/physiopathology/*therapy MH - Middle Aged MH - Peripheral Arterial Disease/diagnosis/physiopathology/*therapy MH - Recovery of Function MH - Regional Blood Flow MH - Treatment Outcome EDAT- 2021/02/08 06:00 MHDA- 2022/01/01 06:00 CRDT- 2021/02/07 20:34 PHST- 2020/11/25 00:00 [received] PHST- 2021/01/02 00:00 [revised] PHST- 2021/01/05 00:00 [accepted] PHST- 2021/02/08 06:00 [pubmed] PHST- 2022/01/01 06:00 [medline] PHST- 2021/02/07 20:34 [entrez] AID - S0890-5096(21)00103-5 [pii] AID - 10.1016/j.avsg.2021.01.069 [doi] PST - ppublish SO - Ann Vasc Surg. 2021 Jul;74:519.e7-519.e16. doi: 10.1016/j.avsg.2021.01.069. Epub 2021 Feb 4. PMID- 41409661 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260519 LR - 20260604 DP - 2026 Feb 10 TI - Early gastrointestinal manifestations predict disease progression and mortality in patients with systemic sclerosis. LID - 2025.12.01.25341383 [pii] LID - 10.64898/2025.12.01.25341383 [doi] AB - OBJECTIVE: Gastrointestinal (GI) complications are common in systemic sclerosis (SSc), yet the value of early symptoms as predictors of progression remains poorly defined. We aimed to determine whether baseline GI symptoms in early SSc predict progression to significant GI outcomes. METHODS: We studied 450 participants from the GENISOS cohort with early SSc (≤5 years from first non-Raynaud's symptom). Baseline assessments included demographics and clinical variables, including individual GI symptoms (GERD, dysphagia, bloating, constipation, diarrhea, peptic ulcer). Significant GI involvement was defined as Medsger GI severity score ≥2. Cox models evaluated associations between baseline symptoms and progression to significant GI disease and all-cause mortality. Additional models examined total baseline GI symptom burden and outcomes. RESULTS: Participants were predominantly female (84%) with a mean age of 47.7 ± 13.3 years. Baseline GI symptoms were frequent, most commonly GERD (76%) and dysphagia (41%). 13% of the patients had significant GI involvement, with 4% presenting with significant GI disease at baseline. Baseline GERD (HR 4.15, P=0.019) and diarrhea (HR 2.25, P=0.021) were strong predictors of GI progression. When symptom burden was analyzed, each additional baseline GI symptom increased the hazard of significant GI progression by 53% (P=0.003). On the other hand, peptic ulcer disease (HR 2.21, P=0.007) and diarrhea (HR 1.48, P=0.045) predicted higher mortality, with each additional symptom raising mortality risk by 29% (P<0.001). CONCLUSIONS: Early GI symptoms and overall symptom burden predict GI progression and mortality in SSc. Symptom-based profiling may offer a practical strategy for clinical risk stratification and trial enrichment. KEY MESSAGES: Key message 1: Baseline GERD and diarrhea strongly predict progression to significant gastrointestinal involvement in systemic sclerosis.Key message 2: Greater baseline gastrointestinal symptom burden increases risk of severe GI disease progression and all-cause mortality.Key message 3: Simple symptom profiles may guide early risk stratification and patient selection for GI-focused clinical trials. FAU - Ayla, Ali Y AU - Ayla AY AUID- ORCID: 0000-0003-2581-7992 FAU - Balar, Ashish P AU - Balar AP AUID- ORCID: 0000-0001-6241-926X FAU - Martinez, Ernesto Calderon AU - Martinez EC FAU - Chen, Bingrui AU - Chen B FAU - Pedroza, Claudia AU - Pedroza C AUID- ORCID: 0000-0003-4235-1282 FAU - Bonomi, Francesco AU - Bonomi F FAU - Bellando-Randone, Silvia AU - Bellando-Randone S FAU - Mayes, Maureen D AU - Mayes MD FAU - Skaug, Brian AU - Skaug B FAU - Hughes, Michael AU - Hughes M FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 FAU - McMahan, Zsuzsanna H AU - McMahan ZH AUID- ORCID: 0000-0001-6461-8940 LA - eng PT - Journal Article PT - Preprint DEP - 20260210 PL - United States TA - medRxiv JT - medRxiv : the preprint server for health sciences JID - 101767986 UIN - Rheumatology (Oxford). 2026 Jun 3;65(6):keag257. doi: 10.1093/rheumatology/keag257. PMID: 42143732 PMC - PMC12706611 EDAT- 2025/12/18 07:20 MHDA- 2025/12/18 07:21 PMCR- 2026/02/16 CRDT- 2025/12/18 04:38 PHST- 2025/12/18 07:20 [pubmed] PHST- 2025/12/18 07:21 [medline] PHST- 2025/12/18 04:38 [entrez] PHST- 2026/02/16 00:00 [pmc-release] AID - 2025.12.01.25341383 [pii] AID - 10.64898/2025.12.01.25341383 [doi] PST - epublish SO - medRxiv [Preprint]. 2026 Feb 10:2025.12.01.25341383. doi: 10.64898/2025.12.01.25341383. PMID- 31585842 OWN - NLM STAT- MEDLINE DCOM- 20200217 LR - 20200217 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 33 IP - 4 DP - 2019 Aug TI - Suspected inflammatory rheumatic diseases in patients presenting with skin rashes. PG - 101440 LID - S1521-6942(19)30125-1 [pii] LID - 10.1016/j.berh.2019.101440 [doi] AB - Skin lesions occur, often at very early stages, in many of the most frequent inflammatory rheumatic diseases such as in systemic lupus erythematosus (SLE), dermatomyositis (DM), systemic sclerosis (SSc), Sjögren's syndrome, rheumatoid arthritis (RA), and psoriatic arthritis. It is important to recognize the different specific cutaneous lesions in SLE (e.g., "butterfly" rash in acute, annular or psoriasiform photosensitive lesions in the subacute form, and discoid lesions in the chronic form) for an early diagnosis and to estimate the associated risks of internal disease, whereas nonspecific lesions (exanthema, vasculitis, and alopecia) can be part of SLE flares. Cutaneous lesions in DM (Gottron's papules and sign, heliotrope rash, dystrophic cuticles, and nailfold capillary abnormalities) may occur before any clinically evident muscular or systemic organ involvement and are of utmost importance for early diagnosis. The pattern of cutaneous lesions and associated autoantibodies also allow the distinction of different phenotypes, either more prone to life-threatening interstitial lung disease (MDA-5) or with higher risk for neoplasia (TIF1-γ). Many other skin lesions, although not specific, require further investigation to look for a possible underlying inflammatory rheumatic disease: non-pruritic urticarial lesions in anti-C1q-associated urticarial vasculitis, Still's disease or hereditary auto-inflammatory syndromes, transient macular purpura of vasculitis in Sjögren's syndrome, Behçet's disease, or RA, Raynaud's phenomenon in SSc and mixed connective tissue disease, erythema nodosum or other panniculitis in RA, Behçet's disease and SLE, pustular eruptions in Behçet's disease, psoriasis, and hereditary auto-inflammatory syndromes. After reviewing in detail the cutaneous manifestations of the most frequent inflammatory rheumatic diseases, we describe a topographic and morphological approach to skin rashes, calling attention to facial rashes, hand involvement, scalp, nail, or leg lesions or to some morphological aspects of skin lesions (annular, pustular, urticarial, or exanthematous) that may be the initial manifestations of inflammatory rheumatic diseases. The importance of skin lesions is confirmed by their presence as part of the classification criteria of many inflammatory rheumatic diseases. They also contribute to early diagnosis, to characterize disease phenotypes, to aid in effective patient management, and, ultimately, to impact on disease prognosis. CI - Copyright © 2019 Elsevier Ltd. All rights reserved. FAU - Alves, Francisca AU - Alves F AD - Clinic of Dermatology, University Hospital of Coimbra, Portugal. FAU - Gonçalo, Margarida AU - Gonçalo M AD - Clinic of Dermatology, University Hospital of Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal. Electronic address: mgoncalo@fmed.uc.pt. LA - eng PT - Journal Article PT - Review DEP - 20191001 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - *Arthritis, Rheumatoid/complications/diagnosis MH - *Dermatomyositis/complications/diagnosis MH - *Exanthema/etiology MH - Humans MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - *Rheumatic Diseases/complications/diagnosis MH - *Sjogren's Syndrome/complications/diagnosis MH - Skin Diseases OTO - NOTNLM OT - Autoantibodies OT - Dermatology OT - Dermatomyositis OT - Psoriasis OT - Rheumatology OT - Sjögren's syndrome OT - Skin OT - Still's disease OT - Systemic lupus erythematosus OT - Systemic sclerosis EDAT- 2019/10/06 06:00 MHDA- 2020/02/18 06:00 CRDT- 2019/10/06 06:00 PHST- 2019/10/06 06:00 [pubmed] PHST- 2020/02/18 06:00 [medline] PHST- 2019/10/06 06:00 [entrez] AID - S1521-6942(19)30125-1 [pii] AID - 10.1016/j.berh.2019.101440 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2019 Aug;33(4):101440. doi: 10.1016/j.berh.2019.101440. Epub 2019 Oct 1. PMID- 35778430 OWN - NLM STAT- MEDLINE DCOM- 20220707 LR - 20250728 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 12 IP - 1 DP - 2022 Jul 1 TI - Clinical characteristics of idiopathic interstitial pneumonias with anti-Ro52/tripartite motif-containing 21 antibodies. PG - 11122 LID - 10.1038/s41598-022-15321-4 [doi] LID - 11122 AB - Antibodies to Ro52/tripartite motif-containing 21 (TRIM21), referred to as anti-Ro52, are found in patients diagnosed with diverse systemic autoimmune rheumatic disease and associated with interstitial lung diseases. However, little is known about the clinical characteristics of anti-Ro52 in patients with idiopathic interstitial pneumonias (IIPs). We aimed to analyze the prevalence, co-existent autoantibodies, and clinical characteristics of anti-Ro52 in patients with IIP. The study enrolled 288 patients diagnosed with IIP. Clinical, laboratory and radiographic findings of IIP patients were compared between anti-Ro52 positives and negatives. Anti-Ro52 (20/288; 6.9%), anti-ARS (18/288; 6.3%), and anti-Ro60/SS-A (16/288; 5.6%) were the most common autoantibodies detected in IIP patients. Among 20 IIP patients who had anti-Ro52, anti-ARS was present in 8 (40%) patients. The criteria for interstitial pneumonia with autoimmune features (IPAF) were significantly better fulfilled by patients with anti-Ro52 than those without (P = 0.001). Meeting serological domain (P < 0.001) and Raynaud's phenomenon (P = 0.009) were significantly more common in the anti-Ro52-positive patients. Anti-Ro52-positive IIP patients have clinical features consistent with IPAF. Anti-Ro52 may have an important role in detecting the autoimmune phenotype in IIP patients. CI - © 2022. The Author(s). FAU - Tahara, Masahiro AU - Tahara M AD - Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan. FAU - Sakamoto, Noriho AU - Sakamoto N AD - Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Satoh, Minoru AU - Satoh M AD - Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. FAU - Ishimoto, Hiroshi AU - Ishimoto H AD - Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Yura, Hirokazu AU - Yura H AD - Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Yamasaki, Kei AU - Yamasaki K AD - Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan. FAU - Kido, Takashi AU - Kido T AD - Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan. AD - Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Fujino, Yoshihisa AU - Fujino Y AD - Department of Environmental Epidemiology, Institute of Industrial Ecological Sciences, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. FAU - Hasegawa, Tomoko AU - Hasegawa T AD - Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. FAU - Tanaka, Shin AU - Tanaka S AD - Department of Human, Information and Life Sciences, School of Health Sciences, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan. FAU - Yatera, Kazuhiro AU - Yatera K AD - Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan. yatera@med.uoeh-u.ac.jp. FAU - Mukae, Hiroshi AU - Mukae H AD - Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220701 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies MH - *Clinical Laboratory Services MH - Humans MH - *Idiopathic Interstitial Pneumonias MH - Laboratories, Clinical MH - Protein Domains PMC - PMC9249750 COIS- The authors declare no competing interests. EDAT- 2022/07/02 06:00 MHDA- 2022/07/08 06:00 PMCR- 2022/07/01 CRDT- 2022/07/01 23:18 PHST- 2021/11/22 00:00 [received] PHST- 2022/06/22 00:00 [accepted] PHST- 2022/07/01 23:18 [entrez] PHST- 2022/07/02 06:00 [pubmed] PHST- 2022/07/08 06:00 [medline] PHST- 2022/07/01 00:00 [pmc-release] AID - 10.1038/s41598-022-15321-4 [pii] AID - 15321 [pii] AID - 10.1038/s41598-022-15321-4 [doi] PST - epublish SO - Sci Rep. 2022 Jul 1;12(1):11122. doi: 10.1038/s41598-022-15321-4. PMID- 33863937 OWN - NLM STAT- MEDLINE DCOM- 20211105 LR - 20211105 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 11 IP - 1 DP - 2021 Apr 16 TI - Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea. PG - 8393 LID - 10.1038/s41598-021-87718-6 [doi] LID - 8393 AB - Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis. FAU - Lee, Jeong-Seon AU - Lee JS AD - Department of Paediatrics, Seoul National University Children's Hospital, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. FAU - Kim, Joong-Gon AU - Kim JG AD - Department of Paediatrics, Seoul National University Children's Hospital, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. FAU - Lee, Soyoung AU - Lee S AUID- ORCID: 0000-0001-9627-6607 AD - Department of Paediatrics, Seoul National University Children's Hospital, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. leadethme@naver.com. LA - eng PT - Clinical Trial PT - Journal Article DEP - 20210416 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 SB - IM MH - Adolescent MH - Child MH - Child, Preschool MH - Disease Progression MH - Female MH - Humans MH - Male MH - Polyarteritis Nodosa/epidemiology/*pathology/surgery MH - Prognosis MH - Republic of Korea/epidemiology MH - Retrospective Studies MH - Vasculitis/epidemiology/*pathology/surgery PMC - PMC8052421 COIS- The authors declare no competing interests. EDAT- 2021/04/18 06:00 MHDA- 2021/11/06 06:00 PMCR- 2021/04/16 CRDT- 2021/04/17 05:48 PHST- 2020/12/09 00:00 [received] PHST- 2021/03/31 00:00 [accepted] PHST- 2021/04/17 05:48 [entrez] PHST- 2021/04/18 06:00 [pubmed] PHST- 2021/11/06 06:00 [medline] PHST- 2021/04/16 00:00 [pmc-release] AID - 10.1038/s41598-021-87718-6 [pii] AID - 87718 [pii] AID - 10.1038/s41598-021-87718-6 [doi] PST - epublish SO - Sci Rep. 2021 Apr 16;11(1):8393. doi: 10.1038/s41598-021-87718-6. PMID- 30534480 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240406 IS - 2169-7574 (Print) IS - 2169-7574 (Electronic) IS - 2169-7574 (Linking) VI - 6 IP - 10 DP - 2018 Oct TI - Botulinum Toxin A: A Novel Therapeutic Modality for Upper Extremity Chronic Regional Pain Syndrome. PG - e1847 LID - 10.1097/GOX.0000000000001847 [doi] LID - e1847 AB - BACKGROUND: Complex regional pain syndromes (CRPS) is a disease that is poorly understood. It is a chronic pain syndrome characterized by sympathetic disruptions as well as CNS sensitization. Botulinum Toxin-A has been shown to have efficacy in Raynaud's as well as other neuropathic pain disorders. Perhaps BTX-A warrants experimentation in the treatment of CRPS. METHODS: Patients with CRPS refractory to 2 or more regional sympathetic nerve blocks in 2007 were included in the study. Patient's were asked to rank their initial pain on a visual analog scale of 0 to 10 (10 being the worst pain). "Tenderness maps" were marked on patient's areas of most pain in 1 by 1 centimeter grids. Each box on the grid was injected with 10 IU of BTX-A after nerve blocks with 1% lidocaine. Treatment sessions occured on a monthly basis with VAS pain scores being re-assessed immediately before the new treatment. t Test, linear regression, and Cohen's D-test were used to analyze the correlation of the data. RESULTS: Study sample was 20 patients. Etiology of CRPS was 6 amputations, 4 crush injuries, 4 penetrating injuries, and 2 lacerations. Average pain reduction on VAS scale achieved was 2.05 points. Average percentage pain reduction was 22.94%. Cohen's D Test also showed a meaningful difference with a score of 1.01. Linear regression R2 = 0.491. Maximum pain reduction, on average, was achieved by treatment 9. CONCLUSION: Despite the esoteric etiology of CRPS, BTX-A has a well-demonstrated mechanism of effect. BTX-A should be further explored as a treatment modality for CRPS. FAU - Lessard, Lucie AU - Lessard L AD - Division of Plastic and Reconstructive Surgery, Department of Surgery, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Bartow, Matthew J AU - Bartow MJ AD - Division of Plastic and Reconstructive Surgery, Department of Surgery, Louisiana State University Health Sciences Center, New Orleans, La. FAU - Lee, James AU - Lee J AD - Division of Plastic and Reconstructive Surgery, Department of Surgery, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Diaz-Abel, Julian AU - Diaz-Abel J AD - Division of Plastic and Reconstructive Surgery, Department of Surgery, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Tessler, Oren E S AU - Tessler OES AD - Elite Plastic Surgery, Phoenix, Arizona. LA - eng PT - Journal Article DEP - 20181016 PL - United States TA - Plast Reconstr Surg Glob Open JT - Plastic and reconstructive surgery. Global open JID - 101622231 PMC - PMC6250468 EDAT- 2018/12/12 06:00 MHDA- 2018/12/12 06:01 PMCR- 2018/10/16 CRDT- 2018/12/12 06:00 PHST- 2018/03/04 00:00 [received] PHST- 2018/05/07 00:00 [accepted] PHST- 2018/12/12 06:00 [entrez] PHST- 2018/12/12 06:00 [pubmed] PHST- 2018/12/12 06:01 [medline] PHST- 2018/10/16 00:00 [pmc-release] AID - 10.1097/GOX.0000000000001847 [doi] PST - epublish SO - Plast Reconstr Surg Glob Open. 2018 Oct 16;6(10):e1847. doi: 10.1097/GOX.0000000000001847. eCollection 2018 Oct. PMID- 24942495 OWN - NLM STAT- MEDLINE DCOM- 20150916 LR - 20221207 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 41 IP - 7 DP - 2014 Jul TI - No association of atherosclerosis with digital ulcers in Japanese patients with systemic sclerosis: evaluation of carotid intima-media thickness and plaque characteristics. PG - 604-8 LID - 10.1111/1346-8138.12532 [doi] AB - Patients with systemic sclerosis (SSc) usually develop Raynaud's phenomenon, persistent digital ischemia and sometimes develop digital ulcers (DU). Several studies have reported an association of carotid artery atherosclerosis with SSc by evaluating carotid intima-media thickness (IMT) in SSc patients. However, none of those studies analyzed the association between DU and carotid artery atherosclerosis in SSc patients. We examined the association of carotid artery atherosclerosis with digital ulcers by comparing SSc patients with (n = 48, 29.5%) and without (n = 206, 70.5%) DU. The demographic and clinical features of the SSc patients showed that young age, male sex, anti-topoisomerase I antibody positivity, severe skin sclerosis, interstitial lung disease complication and cardiac involvements were significantly prevalent in patients with DU. In addition, diffuse cutaneous type, anti-RNA polymerase III antibody positivity and severe skin sclerosis are more frequent in SSc patients with DU at the extensor surface of joints than SSc patients with DU at the digital tip. There were no differences in serum lipid level, carotid IMT or plaque score between SSc patients with and without DU, suggesting that atherosclerotic changes are not primarily involved in the development of DU. CI - © 2014 Japanese Dermatological Association. FAU - Motegi, Sei-Ichiro AU - Motegi S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan. FAU - Toki, Sayaka AU - Toki S FAU - Hattori, Tomoyasu AU - Hattori T FAU - Yamada, Kazuya AU - Yamada K FAU - Uchiyama, Akihiko AU - Uchiyama A FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article DEP - 20140618 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 SB - IM MH - Aged MH - Asian People MH - Carotid Artery Diseases/*complications/pathology MH - Carotid Intima-Media Thickness MH - Carotid Stenosis/complications/pathology MH - Female MH - Fingers MH - Heart Diseases/complications MH - Humans MH - Japan MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications/pathology MH - Skin Ulcer/*complications OTO - NOTNLM OT - atherosclerosis OT - carotid intima-media thickness OT - digital ulcers OT - systemic sclerosis EDAT- 2014/06/20 06:00 MHDA- 2015/09/17 06:00 CRDT- 2014/06/20 06:00 PHST- 2014/04/08 00:00 [received] PHST- 2014/05/06 00:00 [accepted] PHST- 2014/06/20 06:00 [entrez] PHST- 2014/06/20 06:00 [pubmed] PHST- 2015/09/17 06:00 [medline] AID - 10.1111/1346-8138.12532 [doi] PST - ppublish SO - J Dermatol. 2014 Jul;41(7):604-8. doi: 10.1111/1346-8138.12532. Epub 2014 Jun 18. PMID- 23211636 OWN - NLM STAT- MEDLINE DCOM- 20130801 LR - 20180417 IS - 1549-4713 (Electronic) IS - 0161-6420 (Linking) VI - 120 IP - 3 DP - 2013 Mar TI - An evidence-based review of prognostic factors for glaucomatous visual field progression. PG - 512-519 LID - S0161-6420(12)00864-0 [pii] LID - 10.1016/j.ophtha.2012.09.005 [doi] AB - PURPOSE: To examine which prognostic factors are associated with glaucomatous visual field progression. DESIGN: Knowledge of prognostic factors helps clinicians to select patients at risk of glaucomatous visual field progression and intensify their treatment. METHODS: By consulting relevant databases, we identified 2733 articles published up to September 2010, of which 85 articles investigating prognostic factors for visual field progression in patients with open-angle glaucoma (OAG) were eligible. We summarized results for each factor in tables, noting the direction of the association between the prognostic factor and progression, and the accompanying P value. Four authors, working blind to the factors, independently judged the extent to which a prognostic factor was associated with glaucomatous visual field progression. If there were different associations for normal-tension glaucoma (NTG) studies, they were judged separately. Consensus was reached during group meetings. MAIN OUTCOME MEASURES: A ranking of all studied prognostic factors for glaucomatous visual field progression according to their likelihood of being prognostic. RESULTS: A total of 103 different prognostic factors were investigated in 85 articles. The following factors were clearly associated with glaucomatous visual field progression: age, disc hemorrhages (for NTG), baseline visual field loss, baseline intraocular pressure (IOP), and exfoliation syndrome. An association was unlikely for family history of glaucoma, atherosclerosis, systemic hypertension, visual acuity, sex (for NTG), systolic blood pressure, myopic refractive error (for NTG), and Raynaud's phenomenon. CONCLUSIONS: The factors we found clearly associated with progression could be used in clinical practice and for developing clinical prediction models. For many other factors, further research is necessary. CI - Copyright © 2013 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved. FAU - Ernest, Paul J AU - Ernest PJ AD - University Eye Clinic Maastricht, Maastricht, The Netherlands; Department of Epidemiology, Maastricht University, Maastricht, The Netherlands. Electronic address: p.j.g.ernest@gmail.com. FAU - Schouten, Jan S AU - Schouten JS AD - University Eye Clinic Maastricht, Maastricht, The Netherlands. FAU - Beckers, Henny J AU - Beckers HJ AD - University Eye Clinic Maastricht, Maastricht, The Netherlands. FAU - Hendrikse, Fred AU - Hendrikse F AD - University Eye Clinic Maastricht, Maastricht, The Netherlands. FAU - Prins, Martin H AU - Prins MH AD - Department of Epidemiology, Maastricht University, Maastricht, The Netherlands. FAU - Webers, Carroll A AU - Webers CA AD - University Eye Clinic Maastricht, Maastricht, The Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20121201 PL - United States TA - Ophthalmology JT - Ophthalmology JID - 7802443 SB - IM MH - Disease Progression MH - Exfoliation Syndrome/diagnosis MH - Glaucoma, Open-Angle/*diagnosis/physiopathology MH - Humans MH - Intraocular Pressure/physiology MH - Prognosis MH - Retinal Hemorrhage/diagnosis MH - Risk Factors MH - Vision Disorders/*diagnosis/physiopathology MH - Visual Acuity/physiology MH - *Visual Fields EDAT- 2012/12/06 06:00 MHDA- 2013/08/02 06:00 CRDT- 2012/12/06 06:00 PHST- 2011/05/27 00:00 [received] PHST- 2012/08/28 00:00 [revised] PHST- 2012/09/04 00:00 [accepted] PHST- 2012/12/06 06:00 [entrez] PHST- 2012/12/06 06:00 [pubmed] PHST- 2013/08/02 06:00 [medline] AID - S0161-6420(12)00864-0 [pii] AID - 10.1016/j.ophtha.2012.09.005 [doi] PST - ppublish SO - Ophthalmology. 2013 Mar;120(3):512-519. doi: 10.1016/j.ophtha.2012.09.005. Epub 2012 Dec 1. PMID- 22004238 OWN - NLM STAT- MEDLINE DCOM- 20120209 LR - 20151119 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 14 IP - 4 DP - 2011 Oct TI - The prevalence of fibromyalgia among textile workers in the city of Denizli in Turkey. PG - 390-4 LID - 10.1111/j.1756-185X.2011.01620.x [doi] AB - AIM: Fibromyalgia (FM) is characterized by chronic widespread pain, fatigue, reduced sleep quality and multiple tender points. A recent population study from Turkey found the prevalence of FM as 3.6%. A prevalence study among workers has not yet been performed. We performed a prevalence survey among working population in the city of Denizli in Turkey. METHODS: The field survey was done in two stages. In the first stage 655 (523 female, 132 male) textile workers from four factories were asked to fill a screening questionnaire. In the next stage, participants who had widespread pain were examined by an experienced rheumatologist. Patients who had 11 tender points according to ACR 1990 FM classification criteria were diagnosed as FM syndrome and later a detailed clinical and laboratory evaluation was done. RESULTS: Forty-eight patients (7.3%) (one male [0.76% of males], 47 females [9.0% of females]) among 655 textile workers were diagnosed as FM. The clinical features were as follows: all patients had widespread pain, 12.5% had arthralgia, 14.6% had Raynaud's phenomenon, 41.6% had sleep disturbance, 87.5% had headache, 52% had irritable bowel disease. Age, gender, marital status, income level, education level, smoking status, and body mass index level of workers were evaluated by logistic regression analysis; multiple analysis. Only three variables (age, gender and annual income level) were significantly affected FM prevalence. CONCLUSION: This is the first study investigating FM prevalence among workers from Turkey. The prevalence of FM appears higher among females, older workers, and workers with low annual money income. CI - © 2011 The Authors. International Journal of Rheumatic Diseases © 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd. FAU - Cobankara, Veli AU - Cobankara V AD - Rheumatology Clinic, Internal Medicine Department, Pamukkale University Faculty of Medicine, Denizli, Turkey. FAU - Unal, U Olcun AU - Unal UO FAU - Kaya, Arif AU - Kaya A FAU - Bozkurt, Ali Ihsan AU - Bozkurt AI FAU - Ozturk, Mehmet Akif AU - Ozturk MA LA - eng PT - Journal Article DEP - 20110629 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Adolescent MH - Adult MH - Comorbidity MH - Female MH - Fibromyalgia/diagnosis/*epidemiology/physiopathology MH - Humans MH - Male MH - Middle Aged MH - Occupational Diseases/diagnosis/*epidemiology/physiopathology MH - Occupational Exposure/*analysis MH - Pain/diagnosis/epidemiology/physiopathology MH - Population Surveillance MH - Prevalence MH - Surveys and Questionnaires MH - *Textile Industry MH - Turkey/epidemiology MH - Young Adult EDAT- 2011/10/19 06:00 MHDA- 2012/02/10 06:00 CRDT- 2011/10/19 06:00 PHST- 2011/10/19 06:00 [entrez] PHST- 2011/10/19 06:00 [pubmed] PHST- 2012/02/10 06:00 [medline] AID - 10.1111/j.1756-185X.2011.01620.x [doi] PST - ppublish SO - Int J Rheum Dis. 2011 Oct;14(4):390-4. doi: 10.1111/j.1756-185X.2011.01620.x. Epub 2011 Jun 29. PMID- 21392097 OWN - NLM STAT- MEDLINE DCOM- 20130201 LR - 20120423 IS - 1472-8206 (Electronic) IS - 0767-3981 (Linking) VI - 26 IP - 3 DP - 2012 Jun TI - Calcitonin ameliorates enhanced arterial contractility after chronic constriction injury of the sciatic nerve in rats. PG - 315-21 LID - 10.1111/j.1472-8206.2011.00934.x [doi] AB - In addition to its regulatory effect on bone mass, calcitonin has been shown to relieve pain and alleviate peripheral circulatory disturbance in patients with Raynaud's syndrome and complex regional pain syndrome. In the present study, we investigated whether calcitonin ameliorates diminished blood flow and enhanced arterial contraction in response to noradrenaline in chronic constriction injury (CCI) of the sciatic nerve in rats. Following surgically induced CCI, laser Doppler flowmetry studies showed a significant decrease in plantar skin blood flow of the ipsilateral hind paw compared to the contralateral side. A subcutaneous bolus injection of elcatonin (20 U/kg), a synthetic derivative of eel calcitonin, significantly improved decreased skin blood flow in the ipsilateral side. In vitro analysis of plantar arteries isolated from the ipsilateral hind paw 7-13 days after the CCI procedure showed higher sensitivity to noradrenaline than the plantar arteries from the contralateral side. Elcatonin (0.1-10 nm) significantly reduced noradrenaline-induced contraction in the arteries of the ipsilateral side, whereas it had little effect on those of the contralateral side. These results suggest that calcitonin selectively ameliorates enhanced arterial contractility in CCI neuropathic rats, thus leading to its alleviating effect on peripheral circulatory disturbance. CI - © 2011 The Authors Fundamental and Clinical Pharmacology © 2011 Société Française de Pharmacologie et de Thérapeutique. FAU - Yoshimura, Takeshi AU - Yoshimura T AD - Laboratory for Development Pharmacology, Pharmaceuticals Research Center, Asahi Kasei Pharma Corporation, 632-1 Mifuku, Izunokuni City, Shizuoka 410-2321, Japan. yoshimura.th@om.asahi-kasei.co.jp FAU - Ito, Akitoshi AU - Ito A FAU - Saito, Shin-ya AU - Saito SY FAU - Takeda, Mineko AU - Takeda M FAU - Kuriyama, Hiroshi AU - Kuriyama H FAU - Ishikawa, Tomohisa AU - Ishikawa T LA - eng PT - Journal Article DEP - 20110311 PL - England TA - Fundam Clin Pharmacol JT - Fundamental & clinical pharmacology JID - 8710411 RN - 0 (Fish Proteins) RN - 9007-12-9 (Calcitonin) SB - IM MH - Animals MH - Arteries/*physiology MH - Blood Flow Velocity/physiology MH - Calcitonin/*physiology MH - Chronic Disease MH - Constriction, Pathologic/physiopathology/therapy MH - Eels MH - Fish Proteins/*physiology MH - Hindlimb/blood supply MH - Male MH - Organ Culture Techniques MH - Rats MH - Rats, Sprague-Dawley MH - Sciatic Nerve/*injuries/*physiology MH - Skin/blood supply MH - Vasoconstriction/*physiology EDAT- 2011/03/12 06:00 MHDA- 2013/02/05 06:00 CRDT- 2011/03/12 06:00 PHST- 2011/03/12 06:00 [entrez] PHST- 2011/03/12 06:00 [pubmed] PHST- 2013/02/05 06:00 [medline] AID - 10.1111/j.1472-8206.2011.00934.x [doi] PST - ppublish SO - Fundam Clin Pharmacol. 2012 Jun;26(3):315-21. doi: 10.1111/j.1472-8206.2011.00934.x. Epub 2011 Mar 11. PMID- 18496697 OWN - NLM STAT- MEDLINE DCOM- 20090319 LR - 20220330 IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 28 IP - 11 DP - 2008 Sep TI - The relationship between serum trace element levels and clinical parameters in patients with fibromyalgia. PG - 1117-21 LID - 10.1007/s00296-008-0593-9 [doi] AB - We examined the association between serum trace elements and clinical findings such as number of sensitive tender points, severity of fatigue and functional status in patients with fibromyalgia (FM). Thirty-two patients diagnosed as having FM according to the ACR 1990 criteria and 32 normal healthy controls (NHC) were included in this study. The demographic data, disease duration, number of tender points and accompanying symptoms (fatigue, sleep disorders, headache, paresthesia, irritable bowel syndrome, sicca symptoms, Raynaud's phenomena) of the patients were noted. Visual analog scale (10 cm) was implemented to estimate daily severity of pain and fatigue. Fibromyalgia impact questionnaire was used for functional assessment. Serum selenium (microg/dL) and serum zinc (microg/dL) levels were measured by atomic absorption spectrometer. Serum magnesium (mmol/L) level was measured by the original kits of Abbott Aeroset auto-analyzer. The mean age of patients in FM group and NHC were calculated as 42.9 (SD = 7.7) years and 41.3 (SD = 9.7) years, respectively. Serum levels of zinc (P = 0.001) and magnesium (P = 0.002) were significantly decreased by FM groups, whereas there was no considerable difference with selenium levels of both groups (P > 0.05). Association between serum zinc level and number of tender points (P = 0.008) and that between fatigue and magnesium level (P = 0.003) was found as meaningful. According to the results of this study, it was asserted that serum magnesium and zinc levels may play an important role in the pathophysiology of FM. FAU - Sendur, Omer Faruk AU - Sendur OF AD - Department of Physical Medicine and Rehabilitation, Adnan Menderes University Medicine School Hospital, Aydin, Turkey. FAU - Tastaban, Engin AU - Tastaban E FAU - Turan, Yasemin AU - Turan Y FAU - Ulman, Cevval AU - Ulman C LA - eng PT - Journal Article DEP - 20080522 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Trace Elements) RN - H6241UJ22B (Selenium) RN - I38ZP9992A (Magnesium) RN - J41CSQ7QDS (Zinc) SB - IM MH - Adult MH - Case-Control Studies MH - Female MH - Fibromyalgia/*blood/physiopathology MH - Humans MH - Magnesium/blood MH - Middle Aged MH - Selenium/blood MH - Severity of Illness Index MH - Trace Elements/*blood MH - Zinc/blood/*deficiency EDAT- 2008/05/23 09:00 MHDA- 2009/03/20 09:00 CRDT- 2008/05/23 09:00 PHST- 2007/12/31 00:00 [received] PHST- 2008/04/29 00:00 [accepted] PHST- 2008/05/23 09:00 [pubmed] PHST- 2009/03/20 09:00 [medline] PHST- 2008/05/23 09:00 [entrez] AID - 10.1007/s00296-008-0593-9 [doi] PST - ppublish SO - Rheumatol Int. 2008 Sep;28(11):1117-21. doi: 10.1007/s00296-008-0593-9. Epub 2008 May 22. PMID- 16681582 OWN - NLM STAT- MEDLINE DCOM- 20060914 LR - 20131121 IS - 0307-6938 (Print) IS - 0307-6938 (Linking) VI - 31 IP - 3 DP - 2006 May TI - Pseudoscleroderma associated with cancer. PG - 381-3 AB - In the period 1994-2002, we saw 71 patients with sclerotic skin changes; 66 were diagnosed with systemic sclerosis (SSc) while five (7%) were diagnosed with pseudoscleroderma associated with various malignancies. The mean duration of disease in these five patients was significantly shorter than that of patients with SSc. The incidence of positive antinuclear antibodies, Raynaud's phenomenon or oesophageal involvement in patients with pseudoscleroderma was significantly lower than that in patients with SSc. The distributions of skin sclerosis varied in each case. Serum basic fibroblast growth factor (bFGF) levels in the five patients with pseudoscleroderma were very elevated compared with levels in controls. Elevated expression of bFGF was detected on fibroblasts of affected skin and in one lung-cancer tissue sample obtained by excision. FAU - Kikuchi, K AU - Kikuchi K AD - Department of Dermatology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan. kikuchi-der@h.u-tokyo.ac.jp FAU - Hoashi, T AU - Hoashi T FAU - Yazawa, N AU - Yazawa N FAU - Tamaki, K AU - Tamaki K LA - eng PT - Case Reports PT - Journal Article PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 RN - 0 (Antineoplastic Agents) RN - 0 (Immunosuppressive Agents) RN - 103107-01-3 (Fibroblast Growth Factor 2) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adenocarcinoma/blood/complications/immunology MH - Aged, 80 and over MH - Antineoplastic Agents/therapeutic use MH - Breast Neoplasms/blood/complications/therapy MH - Carcinoma, Squamous Cell/blood/complications/immunology MH - Case-Control Studies MH - Fatal Outcome MH - Female MH - Fibroblast Growth Factor 2/analysis/blood MH - Hand Dermatoses/blood/*etiology/therapy MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lung Neoplasms/blood/complications/secondary MH - Male MH - Middle Aged MH - Nasopharyngeal Neoplasms/blood/complications/immunology/therapy MH - Neoplasms/blood/*complications/therapy MH - Prednisolone/therapeutic use MH - Scleroderma, Localized/blood/*etiology/therapy MH - Scleroderma, Systemic/metabolism MH - Skin/metabolism EDAT- 2006/05/10 09:00 MHDA- 2006/09/15 09:00 CRDT- 2006/05/10 09:00 PHST- 2006/05/10 09:00 [pubmed] PHST- 2006/09/15 09:00 [medline] PHST- 2006/05/10 09:00 [entrez] AID - CED2092 [pii] AID - 10.1111/j.1365-2230.2006.02092.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2006 May;31(3):381-3. doi: 10.1111/j.1365-2230.2006.02092.x. PMID- 18260175 OWN - NLM STAT- MEDLINE DCOM- 20080610 LR - 20250529 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 3 DP - 2008 Mar TI - Clinical and immunologic manifestations of mixed connective tissue disease in a Miami population compared to a Midwestern US Caucasian population. PG - 429-37 AB - OBJECTIVE: A cross-sectional study of mixed connective tissue disease (MCTD) was performed to determine if there were identifiable differences in the clinical expression of MCTD associated with race or ethnicity. METHODS: Miami, Florida, and Midwestern US (Missouri) Caucasian MCTD cohorts were studied. Clinical and laboratory features of the 2 MCTD cohorts were compared. A concurrently collected cohort of Sm-positive patients with systemic lupus erythematosus (SLE) was studied as a control. Disease activity and severity and functional status were measured. CD4+CD25(high)-expressing T-regulatory cells were enumerated and serum soluble L selectin was measured as biomarkers of disease activity. RESULTS: The Miami and Missouri Caucasian MCTD groups, while differing from the SLE group, were largely similar; however, gastroesophageal reflux, sclerodactyly, and malar rash were significantly more frequent in the Missouri MCTD group and alopecia was more frequent in the Miami MCTD group. Significant clinical and laboratory differences were found between the Miami MCTD and Miami SLE groups despite similar disease duration, activity, severity and functional status. Raynaud's phenomenon (RP), hand swelling, synovitis, myositis, and sclerodactyly were all significantly more common in RNP-positive MCTD versus Sm-positive SLE subjects. CONCLUSION Ethnic differences were observed in the frequency of end-organ involvement in the Miami MCTD versus the Missouri Caucasian MCTD groups. Clinical and laboratory features of all MCTD groups were clearly different from the SLE group, despite similar disease activity, disease severity, and functional status. Disease activity measures appeared to behave similarly as valid measures of disease activity in SLE and MCTD. FAU - Maldonado, Marcos E AU - Maldonado ME AD - Division of Rheumatology and Immunology, Department of Medicine, University of Miami Miller School of Medicine; and the Miami VA Medical Center, Miama, Florida 33136, USA. FAU - Perez, Magdalena AU - Perez M FAU - Pignac-Kobinger, Judith AU - Pignac-Kobinger J FAU - Marx, Emily Triana AU - Marx ET FAU - Tozman, Elaine M AU - Tozman EM FAU - Greidinger, Eric L AU - Greidinger EL FAU - Hoffman, Robert W AU - Hoffman RW LA - eng GR - R01 AR043308/AR/NIAMS NIH HHS/United States GR - AR43308/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20080201 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 126880-86-2 (L-Selectin) SB - IM MH - Adolescent MH - Adult MH - Black or African American/ethnology MH - Aged MH - Case-Control Studies MH - Cross-Sectional Studies MH - Florida/epidemiology MH - Hispanic or Latino/ethnology MH - Humans MH - L-Selectin/*blood MH - Lupus Erythematosus, Systemic/ethnology/*immunology MH - Middle Aged MH - Missouri/epidemiology MH - Mixed Connective Tissue Disease/*ethnology/*immunology MH - Severity of Illness Index MH - *T-Lymphocytes, Regulatory MH - White People/ethnology PMC - PMC2919224 MID - NIHMS217671 EDAT- 2008/02/09 09:00 MHDA- 2008/06/11 09:00 PMCR- 2010/08/10 CRDT- 2008/02/09 09:00 PHST- 2008/02/09 09:00 [pubmed] PHST- 2008/06/11 09:00 [medline] PHST- 2008/02/09 09:00 [entrez] PHST- 2010/08/10 00:00 [pmc-release] AID - 08/13/024 [pii] PST - ppublish SO - J Rheumatol. 2008 Mar;35(3):429-37. Epub 2008 Feb 1. PMID- 42037288 OWN - NLM STAT- MEDLINE DCOM- 20260427 LR - 20260427 IS - 1724-5990 (Electronic) IS - 0393-5590 (Linking) VI - 43 IP - 1 DP - 2026 Feb 28 TI - Membranous Nephropathy Preceding Systemic Sclerosis: An Unusual Presentation of Systemic Sclerosis sine Scleroderma. LID - 2026-vol1 [pii] LID - 10.69097/43-01-2026-10 [doi] AB - Background. Membranous nephropathy (MN) is generally primary, but it can also occur as a secondary form in association with infections, neoplasms or autoimmune diseases. Systemic Sclerosis (SSc), especially in its sine scleroderma forms or in its early stages, rarely manifests itself as MN. Case report. A 60-year-old woman with onset of nephrotic syndrome and histological picture of MN, in the absence of systemic manifestations. The patient subsequently developed episodes of acute renal failure, recurrent proteinuria and clinical-serological signs suggestive of autoimmune connective tissue disease, including increasing ANA titre with anticentromere pattern and onset of Raynaud's phenomenon. The second renal biopsy showed an evolving picture with extensive interstitial fibrosis and severe arteriosclerosis, consistent with a secondary form of MN. The patient was treated with the Ponticelli regimen and subsequently with rituximab, achieving significant clinical remission. In light of the capillaroscopy and autoantibody profile, a diagnosis of very early systemic SSc (sine scleroderma) was made. Discussion. This case highlights how MN can represent an early and atypical manifestation of SSc sine scleroderma, preceding systemic manifestations by years. The negative anti-PLA2R test, the presence of antinuclear autoantibodies and rapid histological progression pointed towards a secondary autoimmune aetiology. Repeated renal biopsy and immunological monitoring proved to be key tools for diagnosis and therapeutic management. Conclusion. MN secondary to SSc sine scleroderma is a rare but important condition that requires attention and a multidisciplinary approach. Early classification as a secondary form allows for targeted therapy and potentially prevents progression to end-stage renal failure. CI - Copyright by Società Italiana di Nefrologia SIN, Rome,Italy. FAU - Melideo, Héctor Matías AU - Melideo HM AD - Nephrology Unit Kidney Trasnplant, Castro Rendon Hospital, Neuquen, Argentina. FAU - Cristiano, Fabrizio AU - Cristiano F AD - Nephrology and Dialysis Unit, Ortona Hospital, Asl 2 Lanciano Vasto Chieti, 66026 Ortona, Italy. FAU - Ramírez, Maximiliano AU - Ramírez M AD - Nephrology Unit Kidney Trasnplant, Castro Rendon Hospital, Neuquen, Argentina. FAU - Maisano, Cecilia AU - Maisano C AD - Nephrology Unit Kidney Trasnplant, Castro Rendon Hospital, Neuquen, Argentina. FAU - Picin, Romina AU - Picin R AD - Anatomic Pathology Department, Castro Rendon Hospital. LA - eng PT - Case Reports PT - Journal Article DEP - 20260228 PL - Italy TA - G Ital Nefrol JT - Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia JID - 9426434 RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Humans MH - Female MH - *Glomerulonephritis, Membranous/etiology/diagnosis/drug therapy MH - *Scleroderma, Systemic/complications/diagnosis MH - Middle Aged MH - Rituximab/therapeutic use OTO - NOTNLM OT - membranous nephropathy OT - scleroderma OT - systemic sclerosis EDAT- 2026/04/27 06:31 MHDA- 2026/04/27 06:32 CRDT- 2026/04/27 04:53 PHST- 2026/04/27 06:32 [medline] PHST- 2026/04/27 06:31 [pubmed] PHST- 2026/04/27 04:53 [entrez] AID - 43-01-2026-10 [pii] AID - 10.69097/43-01-2026-10 [doi] PST - epublish SO - G Ital Nefrol. 2026 Feb 28;43(1):2026-vol1. doi: 10.69097/43-01-2026-10. PMID- 40643579 OWN - NLM STAT- MEDLINE DCOM- 20251002 LR - 20260417 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 37 IP - 6 DP - 2025 Nov 1 TI - Patient-reported outcomes in systemic sclerosis: insights into quality of life and disease burden. PG - 335-342 LID - 10.1097/BOR.0000000000001111 [doi] AB - PURPOSE OF REVIEW: Assessing the impact of active therapy on how patients 'feel and function' is considered a necessary requirement by regulatory agencies for the approval of future treatments for SSc. In this context, patient-reported outcome measures (PROMs) have become a cornerstone of therapeutic assessment in randomized controlled trials (RCTs). RECENT FINDINGS: This narrative review will discuss a selection of main available PROMs used in SSc RCTs, with a specific focus on recently developed PROMs, highlight ongoing initiatives related to SSc-PROMs, and provide points to consider for future use of SSc-PROMs. SUMMARY: Several recent initiatives include a patient-centered approach [such as the Systemic Sclerosis-Associated Raynaud's Phenomenon (ASRAP), the MCQ (Mawdsley Calcinosis Questionnaire], the COAST (Clinical Outcome Assessments for Systemic Sclerosis Clinical Trials), and the CRISTAL (Combined Response index for scleroderma trials assessing limited systemic sclerosis) initiatives] to develop new PROMs and actively involve patient partners in each step of the process. Using a combined response index incorporating PROMs as the primary outcome measure in future SSc trials, such as the CRISS index for diffuse cutaneous SSc, could ensure that the perspectives of both physicians and patients would be incorporated to assess the efficacy of future interventions. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, Rennes University Hospital. AD - Université de Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France. AD - Division of Rheumatology, Scleroderma Program. FAU - Chen, Yen T AU - Chen YT AD - Division of Rheumatology, Scleroderma Program. AD - Department of Physical Medicine & Rehabilitation, University of Michigan, Ann Arbor, MI, USA. FAU - Khanna, Dinesh AU - Khanna D AD - Division of Rheumatology, Scleroderma Program. LA - eng PT - Journal Article PT - Review DEP - 20250711 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Humans MH - *Patient Reported Outcome Measures MH - *Scleroderma, Systemic/therapy/psychology MH - *Quality of Life MH - *Cost of Illness MH - Randomized Controlled Trials as Topic OTO - NOTNLM OT - patient partners OT - patient-reported outcome measures OT - randomized controlled trials OT - systemic sclerosis EDAT- 2025/07/11 12:31 MHDA- 2025/10/02 12:31 CRDT- 2025/07/11 10:53 PHST- 2025/10/02 12:31 [medline] PHST- 2025/07/11 12:31 [pubmed] PHST- 2025/07/11 10:53 [entrez] AID - 00002281-202511000-00002 [pii] AID - 10.1097/BOR.0000000000001111 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2025 Nov 1;37(6):335-342. doi: 10.1097/BOR.0000000000001111. Epub 2025 Jul 11. PMID- 38564673 OWN - NLM STAT- MEDLINE DCOM- 20241024 LR - 20241029 IS - 1744-5078 (Electronic) IS - 0927-3948 (Linking) VI - 32 IP - 9 DP - 2024 Nov TI - Autoimmune Liver Disease Associated Uveitis: An Extrahepatic Manifestation or a Polyautoimmunity Phenomenon? Case Reports. PG - 2268-2272 LID - 10.1080/09273948.2024.2333391 [doi] AB - PURPOSE: To report two cases of non-granulomatous unilateral anterior uveitis in two female patients associated with autoimmune liver diseases (ALD), emphasizing the possibility of this rare coexistence as a polyautoimmunity phenomenon. CASE DESCRIPTIONS: Case 1: An 18-year-old female with a history of congenital renal hypoplasia and metabolic syndrome presented with anterior uveitis in OS and a history of jaundice, blood elevated hepatic enzymes, and cholangioresonance compatible with primary sclerosing cholangitis (PSC). Laboratory work-up for additional autoimmune and infective causes were within normal limits. Case 2: An 58-year-old female presented an episode of anterior uveitis in OD and a history of Sjögren syndrome diagnosed at the age of 53, primary biliary cholangitis (PBC), systemic sclerosis, Raynaud's phenomenon, bilateral sacroiliitis, and vitiligo, consistent with polyautoimmunity and multiple autoimmune syndrome. CONCLUSIONS: Uveitis rarely coexists with ALD. However, it is essential to recognize the possibility of polyautoimmunity in patients presenting with ophthalmic manifestations and a previous diagnosis of ALD, such as PSC or PBC. FAU - Zarate-Pinzón, Laura AU - Zarate-Pinzón L AUID- ORCID: 0000-0003-0295-4986 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - Flórez-Esparza, Gabriela AU - Flórez-Esparza G AUID- ORCID: 0009-0004-6442-0204 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - Rodríguez-Rodríguez, Camilo Andrés AU - Rodríguez-Rodríguez CA AUID- ORCID: 0000-0002-8361-2584 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - Diez-Bahamón, Luis A AU - Diez-Bahamón LA AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - Mejía-Salgado, Germán AU - Mejía-Salgado G AUID- ORCID: 0000-0002-1241-0715 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. AD - Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - Cifuentes-González, Carlos AU - Cifuentes-González C AUID- ORCID: 0000-0002-2703-0977 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. AD - Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. FAU - de-la-Torre, Alejandra AU - de-la-Torre A AUID- ORCID: 0000-0003-0684-1989 AD - Ophthalmology Interest Group, Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. AD - Neuroscience (NEUROS) Research Group, Neurovitae Research Center, Institute of Translational Medicine (IMT), Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia. LA - eng PT - Case Reports PT - Journal Article DEP - 20240402 PL - England TA - Ocul Immunol Inflamm JT - Ocular immunology and inflammation JID - 9312169 SB - IM MH - Humans MH - Female MH - Adolescent MH - Middle Aged MH - *Autoimmune Diseases/diagnosis/immunology/complications MH - *Uveitis, Anterior/diagnosis/immunology OTO - NOTNLM OT - Autoimmune uveitis OT - multiple autoimmune syndrome OT - polyautoimmunity OT - primary biliary cholangitis OT - primary sclerosing cholangitis EDAT- 2024/04/02 18:44 MHDA- 2024/10/25 00:21 CRDT- 2024/04/02 15:43 PHST- 2024/10/25 00:21 [medline] PHST- 2024/04/02 18:44 [pubmed] PHST- 2024/04/02 15:43 [entrez] AID - 10.1080/09273948.2024.2333391 [doi] PST - ppublish SO - Ocul Immunol Inflamm. 2024 Nov;32(9):2268-2272. doi: 10.1080/09273948.2024.2333391. Epub 2024 Apr 2. PMID- 20164033 OWN - NLM STAT- MEDLINE DCOM- 20100524 LR - 20171116 IS - 1897-5631 (Electronic) IS - 0239-8508 (Linking) VI - 47 IP - 3 DP - 2009 Jan TI - Diminished production of TWEAK by the peripheral blood mononuclear cells is associated with vascular involvement in patients with systemic sclerosis. PG - 465-9 LID - 10.2478/v10042-009-0103-2 [doi] AB - Widespread vasculopathy and profound fibrosis are key features of the pathogenesis of systemic sclerosis (SSc). We hypothesized that the TNF-like weak inducer of apoptosis (TWEAK), a recently recognized multifunctional cytokine which regulates angiogenesis and tissue remodeling, may play a role in the development of SSc. The production of TWEAK by the peripheral blood mononuclear cells (PBMC) was investigated, by means of ELISA, in 24 SSc patients and 14 healthy subjects. Moreover, production of TWEAK was correlated with clinical features of SSc. PBMC were isolated using density gradient centrifugation on Histopaque and were cultured in FCS supplemented RPMI medium at 37 degrees C under 5% CO2. Production of TWEAK by PBMC was significantly diminished in patients with more severe microvascular damage, as indicated by the presence of "active" capillaroscopic pattern, compared with SSc patients with less pronounced microangiopathy ("slow" pattern), and healthy subjects. Moreover production of TWEAK correlated inversely with duration of Raynaud's phenomenon. PBMC from patients with scleroderma-related interstitial lung disease tended to produce lower amounts of TWEAK compared with SSc patients without lung involvement but the difference was not significant. The results of our study suggest that diminished production of TWEAK might play a role in the pathogenesis of vascular injury in SSc patients. Whether TWEAK may represent a new therapeutic target in SSc requires further studies. FAU - Bielecki, Marek AU - Bielecki M AD - Department of Orthopedics, Medical University of Bialystok, Bialystok, Poland. FAU - Kowal, Krzysztof AU - Kowal K FAU - Lapinska, Anna AU - Lapinska A FAU - Chwiecko, Justyna AU - Chwiecko J FAU - Skowronski, Jan AU - Skowronski J FAU - Sierakowski, Stanislaw AU - Sierakowski S FAU - Chyczewski, Lech AU - Chyczewski L FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O LA - eng PT - Journal Article PL - Poland TA - Folia Histochem Cytobiol JT - Folia histochemica et cytobiologica JID - 8502651 RN - 0 (Cytokine TWEAK) RN - 0 (TNFSF12 protein, human) RN - 0 (Tumor Necrosis Factors) SB - IM MH - Adult MH - Cells, Cultured MH - Cytokine TWEAK MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Leukocytes, Mononuclear/*metabolism MH - Male MH - Microvessels/*metabolism/pathology MH - Middle Aged MH - Scleroderma, Systemic/*metabolism/pathology MH - Tumor Necrosis Factors/*biosynthesis EDAT- 2010/02/19 06:00 MHDA- 2010/05/25 06:00 CRDT- 2010/02/19 06:00 PHST- 2010/02/19 06:00 [entrez] PHST- 2010/02/19 06:00 [pubmed] PHST- 2010/05/25 06:00 [medline] AID - 1279345524000051 [pii] AID - 10.2478/v10042-009-0103-2 [doi] PST - ppublish SO - Folia Histochem Cytobiol. 2009 Jan;47(3):465-9. doi: 10.2478/v10042-009-0103-2. PMID- 19040522 OWN - NLM STAT- MEDLINE DCOM- 20090423 LR - 20191210 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 34 IP - 3 DP - 2009 Apr TI - Clinicopathological evaluation of in vivo epidermal nuclear fluorescence. PG - 314-8 LID - 10.1111/j.1365-2230.2008.02947.x [doi] AB - BACKGROUND: Nuclear fluorescence in keratinocytes is an occasional phenomenon, often present in autoimmune diseases, especially in connective-tissue disease (CTD); however, its clinical significance remains unclear. AIM: To investigate the profile of patients with positive nuclear staining on direct immunofluorescence (DIF) of skin samples. METHODS: A retrospective analysis of 28 patient records from our immunodermatology laboratory was performed between May 2003 and June 2006. Inclusion criteria were the presence of autoantibodies (IgG, IgA or IgM) or complement (C3) binding keratinocyte nuclei on DIF. RESULTS: The most prevalent diseases related to the nuclear keratinocyte DIF staining were systemic lupus erythematosus (n = 9), mixed CTD (n = 3), overlap syndrome (n = 3), Sjögren's syndrome (n = 1), and CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) syndrome (n = 1). Serum antinuclear antibody (ANA) was positive in 20 of 28 patients, with titres varying from 1 : 160 to 1 : 1280. Of the 20 patients with positive anti-nuclear antibodies (ANA), 17 were positive for anti-extractable nuclear antigen antibodies, 12 had anti-SSA/Ro, 11 had anti-SSB/La and 8 had anti-ribonucleoprotein. Eight patients were negative for ANA. Positive predictive value of in vivo ANA for systemic CTDs was 75%. CONCLUSION: The present data suggest that in vivo ANA evaluation is an additional and feasible auxiliary tool for diagnosing CTDs. FAU - Sousa, J X Jr AU - Sousa JX Jr AD - Department of Dermatology, University of São Paulo School of Medicine, São Paulo, Brazil. FAU - Miyamoto, D AU - Miyamoto D FAU - Zimbres, J M AU - Zimbres JM FAU - Costa, D V AU - Costa DV FAU - Aoki, V AU - Aoki V LA - eng PT - Evaluation Study PT - Journal Article DEP - 20081124 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Antinuclear/*analysis MH - Biomarkers/analysis MH - Cell Nucleus/immunology MH - Connective Tissue Diseases/*diagnosis/immunology MH - Epidermis/*immunology MH - Feasibility Studies MH - Female MH - Fluorescent Antibody Technique, Direct/methods MH - Humans MH - Keratinocytes/immunology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Young Adult EDAT- 2008/12/02 09:00 MHDA- 2009/04/25 09:00 CRDT- 2008/12/02 09:00 PHST- 2008/12/02 09:00 [pubmed] PHST- 2009/04/25 09:00 [medline] PHST- 2008/12/02 09:00 [entrez] AID - CED2947 [pii] AID - 10.1111/j.1365-2230.2008.02947.x [doi] PST - ppublish SO - Clin Exp Dermatol. 2009 Apr;34(3):314-8. doi: 10.1111/j.1365-2230.2008.02947.x. Epub 2008 Nov 24. PMID- 18719326 OWN - NLM STAT- MEDLINE DCOM- 20081126 LR - 20190606 IS - 1349-8029 (Electronic) IS - 0470-8105 (Linking) VI - 48 IP - 8 DP - 2008 Aug TI - Acute basilar artery occlusion in a patient with left subclavian artery occlusion due to first rib anomaly: case report. PG - 355-8 AB - A previously healthy 22-year-old man presented with thoracic outlet syndrome manifesting as Raynaud's phenomenon in the left hand and embolic occlusion of the basilar artery. Three-dimensional computed tomography angiography showed that the left subclavian artery was occluded as it passed over the abnormal first rib. Retrograde propagation of the thrombus from the site of arterial occlusion and/or reflux of embolic material was suspected. Medical therapy was started. The patient underwent resection of the anomalous rib. Postoperative angiography demonstrated that the subclavian artery was recanalized with almost normal distal flow. The basilar artery was also recanalized. Thoracic outlet syndrome due to a first rib anomaly may cause stroke. FAU - Yamaguchi, Rei AU - Yamaguchi R AD - Department of Neurosurgery, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. ryamaguc@showa.gunma-u.ac.jp FAU - Kohga, Hideaki AU - Kohga H FAU - Kurosaki, Minori AU - Kurosaki M FAU - Tamura, Masaru AU - Tamura M FAU - Tanaka, Soukichi AU - Tanaka S FAU - Tosaka, Masahiko AU - Tosaka M FAU - Yoshimoto, Yuhei AU - Yoshimoto Y LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Neurol Med Chir (Tokyo) JT - Neurologia medico-chirurgica JID - 0400775 RN - 0 (Anticoagulants) SB - IM MH - Acute Disease/therapy MH - Anticoagulants/therapeutic use MH - Cerebellum/blood supply/pathology MH - Cerebral Angiography MH - Functional Laterality/physiology MH - Humans MH - Intracranial Embolism/diagnostic imaging/*etiology/*pathology MH - Magnetic Resonance Imaging MH - Male MH - Regional Blood Flow/physiology MH - Ribs/*abnormalities/diagnostic imaging/surgery MH - Subclavian Steal Syndrome/*complications/etiology/pathology MH - Thoracic Outlet Syndrome/complications/diagnostic imaging/etiology MH - Thoracic Surgical Procedures MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Vertebral Artery/diagnostic imaging/physiopathology MH - Vertebrobasilar Insufficiency/diagnostic imaging/*etiology/*pathology MH - Young Adult EDAT- 2008/08/23 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/08/23 09:00 PHST- 2008/08/23 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/08/23 09:00 [entrez] AID - JST.JSTAGE/nmc/48.355 [pii] AID - 10.2176/nmc.48.355 [doi] PST - ppublish SO - Neurol Med Chir (Tokyo). 2008 Aug;48(8):355-8. doi: 10.2176/nmc.48.355. PMID- 17215624 OWN - NLM STAT- MEDLINE DCOM- 20070524 LR - 20191110 IS - 1540-9740 (Print) IS - 1540-9740 (Linking) VI - 6 IP - 1 DP - 2007 Jan-Feb TI - Dermatomyositis with panniculitis. PG - 46-7 AB - Case 1. A 23-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and limbs, particularly over the dorsum of the hands and fingers; diffuse alopecia; and an inability to climb stairs and get up from a low seat. The clinical examination showed red to violaceous well-demarcated plaques on sun-exposed areas on the dorsum of the fingers and hands, with periungual erythema and telangiectasia; facial erythema; and heliotrope rash. There was also symmetric involvement of proximal muscles of the limbs. Laboratory examination showed hypergammaglobulinemia, elevated serum aspartate aminotransferase, and serum alanine aminotransferase; normal activities of creatinokinase, lactate dehydrogenase, and aldolase; an antinuclear antibody titer of 1:40 with a speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Skin biopsy histopathology showed hyperkeratosis, edema of the upper epidermis, scattered inflammatory infiltrate, and focal accumulation of mucin in the form of acid mucopolysaccharides. Deep asymptomatic nodules on the inner upper limbs appeared later. Histopathology of these lesions showed focal areas of lobular panniculitis in the subcutaneous tissue, with lymphoplasmocytic inflammatory infiltrate without vasculitis (Figure 1 and Figure 2). Case 2. A 29-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and lower extremities. Clinical examination showed red to violaceous well-demarcated aching plaques on the internal surface of the thighs and tips of the fingers; periungual erythema and digital petechiae; Raynaud's phenomenon; and bilateral ulnar and cervical enlarged lymph nodes. Laboratory examination showed elevated serum aspartate aminotransferase, alanine aminotransferase, creatinokinase, lactate dehydrogenase, and aldolase; negative venereal disease research test results; an antinuclear antibody titer of 1:1024 with speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Histopathology of the deep asymptomatic nodule on the inner left thigh showed lobular panniculitis with a scattered inflammatory infiltrate and diffuse fat necrosis, in addition to calcium deposition between the lipocytes and microcysts without vasculitis (Figure 3). FAU - Carneiro, Sueli AU - Carneiro S AD - Section of Dermatology, Federal University of Rio de Janeiro, School of Medicine, HUCFF, Rio de Janeiro, Brazil. sueli@hucff.ufrj.br FAU - Alvim, Giselle AU - Alvim G FAU - Resende, Patricia AU - Resende P FAU - Auxiliadora Jeunon Sousa, Maria AU - Auxiliadora Jeunon Sousa M FAU - Cuzzi, Tullia AU - Cuzzi T FAU - Ramos-e-Silva, Marcia AU - Ramos-e-Silva M LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Skinmed JT - Skinmed JID - 101168327 SB - IM MH - Adult MH - Dermatomyositis/*complications/*pathology MH - Female MH - Humans MH - Panniculitis/*complications/*pathology EDAT- 2007/01/12 09:00 MHDA- 2007/05/26 09:00 CRDT- 2007/01/12 09:00 PHST- 2007/01/12 09:00 [pubmed] PHST- 2007/05/26 09:00 [medline] PHST- 2007/01/12 09:00 [entrez] AID - 10.1111/j.1540-9740.2007.05844.x [doi] PST - ppublish SO - Skinmed. 2007 Jan-Feb;6(1):46-7. doi: 10.1111/j.1540-9740.2007.05844.x. PMID- 20101308 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20100623 LR - 20240322 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 54 IP - 2 DP - 2009 TI - Cutaneous manifestations of systemic lupus erythematosus in a tertiary referral center. PG - 132-6 LID - 10.4103/0019-5154.53189 [doi] AB - BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management. AIMS: The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement. MATERIALS AND METHODS: At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007. RESULTS: Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud's phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1.34%), erythema multiforme in 10 patients (6.67%), and nail fold infarcts in 2 patients (1.34%); however, mucosal discoid lupus, lichenoid discoid lupus, livedo reticularis, sclerodactyly, etc. were not detected. Patients having lupus-specific skin lesions e.g., malar rash were associated with systemic involvement, whereas those having lupus non-specific skin lesions were associated with disease flare. CONCLUSIONS: Skin lesions in patients with SLE are important disease manifestations and proper understanding is essential for diagnosis and efficient management. FAU - Kole, Alakes Kumar AU - Kole AK AD - Institute of Post Graduate Medical Education and Research, Department of Rheumatology and Clinical Immunology. FAU - Ghosh, Alakendu AU - Ghosh A LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC2807152 OTO - NOTNLM OT - Cutaneous manifestations OT - organ involvement OT - systemic lupus erythematosus COIS- Conflict of Interest: Nil. EDAT- 2010/01/27 06:00 MHDA- 2010/01/27 06:01 PMCR- 2009/04/01 CRDT- 2010/01/27 06:00 PHST- 2010/01/27 06:00 [entrez] PHST- 2010/01/27 06:00 [pubmed] PHST- 2010/01/27 06:01 [medline] PHST- 2009/04/01 00:00 [pmc-release] AID - IJD-54-132 [pii] AID - 10.4103/0019-5154.53189 [doi] PST - ppublish SO - Indian J Dermatol. 2009;54(2):132-6. doi: 10.4103/0019-5154.53189. PMID- 42180815 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260525 LR - 20260525 IS - 2160-200X (Print) IS - 2160-200X (Electronic) IS - 2160-200X (Linking) VI - 16 IP - 2 DP - 2026 TI - Association between inflammatory diseases and connective tissue disorders with atrial fibrillation/flutter. PG - 92-100 LID - 10.62347/HKSK4068 [doi] AB - BACKGROUND: The role of inflammatory disease in the occurrence of atrial fibrillation/flutter (AF/AFL) is not well studied. OBJECTIVE: The goal of this study was to evaluate any association between inflammatory and autoimmune disorders with the occurrences of AF/AFL using a large database. METHODS: Using the Nationwide Inpatient Sample (NIS) database and ICD-10 codes for AF/AFL and several inflammatory diseases for the years 2016-2020, we evaluated the above association. RESULTS: A total of 23,037,013 patients were identified with a diagnosis of AF/AFL. The following diseases were independently associated with the presence of AF/AFL despite adjustment for age, demographics, and traditional risk factors: rheumatoid arthritis: OR: 1.05, CI 1.04-1.06, P<0.001, systemic sclerosis OR: 1.31, CI 1.26-1.36, P<0.001, systemic connective tissue disorders: OR: 1.07, CI 1.05-1.08, P<0.001, antiphospholipid syndrome: OR: 1.36, CI: 1.31-1.42, P<001, systemic lupus erythematosus: OR: 1.15, CI: 1.13-1.17, P<0.001 and Raynaud's syndrome: OR: 1.1, CI: 1.07-1.13, P<0.001. Ankylosing spondylitis was not found to be associated with AF/AFL. CONCLUSION: Using a large inpatient database, we found that some common inflammatory diseases and connective tissue disorders are independently associated with the presence of AF/AFL. Our findings are hypothesis-generating, requiring confirmation in prospective controlled trials. CI - AJCD Copyright © 2026. FAU - Araghi, Tala AU - Araghi T AD - University of Arizona College of Medicine Phoenix, AZ 85724, USA. FAU - Hashemzadeh, Mehrtash AU - Hashemzadeh M AD - University of Arizona College of Medicine Phoenix, AZ 85724, USA. FAU - Movahed, Mohammad Reza AU - Movahed MR AD - University of Arizona College of Medicine Phoenix, AZ 85724, USA. AD - University of Arizona Sarver Heart Center Tucson, AZ 85724, USA. LA - eng PT - Journal Article DEP - 20260415 PL - United States TA - Am J Cardiovasc Dis JT - American journal of cardiovascular disease JID - 101569582 PMC - PMC13195259 OTO - NOTNLM OT - Atrial fibrillation OT - antiphospholipid syndrome OT - arrhythmia OT - atrial flutter OT - autoimmune disorders OT - inflammation OT - risk factors OT - systemic OT - systemic connective tissue disorders OT - systemic lupus erythematosus COIS- None. EDAT- 2026/05/25 12:35 MHDA- 2026/05/25 12:36 PMCR- 2026/04/15 CRDT- 2026/05/25 07:56 PHST- 2025/11/18 00:00 [received] PHST- 2026/03/06 00:00 [accepted] PHST- 2026/05/25 12:36 [medline] PHST- 2026/05/25 12:35 [pubmed] PHST- 2026/05/25 07:56 [entrez] PHST- 2026/04/15 00:00 [pmc-release] AID - 10.62347/HKSK4068 [doi] PST - epublish SO - Am J Cardiovasc Dis. 2026 Apr 15;16(2):92-100. doi: 10.62347/HKSK4068. eCollection 2026. PMID- 40873289 OWN - NLM STAT- Publisher LR - 20250828 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) DP - 2025 Aug 26 TI - Bibliometric Analysis of Capillaroscopy Use in Systemic Sclerosis: Current State and Advances from 2000 to 2024. LID - 10.2174/0115733971388040250815185341 [doi] AB - INTRODUCTION: Systemic sclerosis is a complex autoimmune disease characterized by diverse clinical manifestations that vary among individuals. It is associated with significant morbidity and mortality. This study aimed to evaluate the existing literature on the use of capillaroscopy in systemic sclerosis and to perform a bibliometric analysis. METHODS: Data were downloaded from Scopus on 2nd October 2024, and analyzed using VOSviewer and the Biblioshiny package programs. RESULTS: A total of 819 articles from 246 sources were analyzed. Italy led in the number of publications (n=209) and the frequency of international cooperation. The article, titled "Nailfold Videocapillaroscopy Assessment of Microvascular Damage in Systemic Sclerosis," published in The Journal of Rheumatology, has garnered 606 citations, making it the most cited article. Cutolo M. was the most influential author, with 1302 citations and 107 publications, while the University of Genova was the most prolific institution. The investigation identified significant issue clusters in the literature related to keywords, such as "systemic sclerosis," "Raynaud's phenomenon," "capillaroscopy," and "digital ulcers." DISCUSSION: This study presents the first comprehensive bibliometric analysis of capillaroscopy use in systemic sclerosis. The findings suggest that scientific interest in the subject is increasing and that research leadership is primarily located in European countries, with Italy at the forefront. The most frequently used keywords in the thematic analysis indicated that capillaroscopy can be used not only for diagnosis but also for monitoring complications. The use of only the Scopus database and the inclusion of only original articles in the study constitute limitations. This methodological choice was made to ensure data integrity. CONCLUSION: Understanding the research on capillaroscopy applications in systemic sclerosis can benefit rheumatologists and provide ideas for future investigations. This bibliometric analysis highlights key authors, citation patterns, collaboration structures, and emerging research issues over the past two decades, providing a comprehensive overview of the discipline. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Doğan, Sevil Ceyhan AU - Doğan SC AUID- ORCID: 0000-0003-1570-8848 AD - Sivas Cumhuriyet University Faculty of Medicine, Sivas, Turkey. FAU - Atas, Mert AU - Atas M AUID- ORCID: 0000-0002-6713-5888 AD - Sivas Cumhuriyet University Faculty of Medicine, Sivas, Turkey. LA - eng PT - Journal Article DEP - 20250826 PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 SB - IM OTO - NOTNLM OT - Systemic sclerosis OT - VOSviewer OT - bibliometric analysis OT - biblioshiny. OT - capillaroscopy EDAT- 2025/08/28 08:45 MHDA- 2025/08/28 08:45 CRDT- 2025/08/28 03:23 PHST- 2025/02/23 00:00 [received] PHST- 2025/05/27 00:00 [revised] PHST- 2025/06/10 00:00 [accepted] PHST- 2025/08/28 08:45 [medline] PHST- 2025/08/28 08:45 [pubmed] PHST- 2025/08/28 03:23 [entrez] AID - CRR-EPUB-150258 [pii] AID - 10.2174/0115733971388040250815185341 [doi] PST - aheadofprint SO - Curr Rheumatol Rev. 2025 Aug 26. doi: 10.2174/0115733971388040250815185341. PMID- 36362943 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221129 IS - 2075-1729 (Print) IS - 2075-1729 (Electronic) IS - 2075-1729 (Linking) VI - 12 IP - 11 DP - 2022 Nov 4 TI - Increased Circulating Soluble Junctional Adhesion Molecules in Systemic Sclerosis: Association with Peripheral Microvascular Impairment. LID - 10.3390/life12111790 [doi] LID - 1790 AB - Systemic sclerosis (SSc, scleroderma) is a severe disease characterized by peripheral microcirculation abnormalities manifesting with Raynaud's phenomenon, nailfold videocapillaroscopic (NVC) changes, and even ischemic digital ulcers (DUs) that are often refractory to treatments. In the wake of previously described associations between the circulating levels of soluble junctional adhesion molecules (sJAMs) and SSc clinical features, here, we measured sJAM-A and sJAM-C levels by enzyme-linked immunosorbent assay in serum samples from a large case series of 110 SSc patients and 85 healthy controls, focusing on their possible association with peripheral vascular clinical features and their potential as biomarkers that are either diagnostic or mirror SSc-related microvasculopathy severity. Our data demonstrated that serum sJAM-A and sJAM-C are significantly increased in patients with SSc vs. healthy controls, especially in those featuring early/active NVC patterns and the presence of ischemic DUs. Moreover, circulating sJAM-C levels showed good diagnostic accuracy in discriminating between patients and controls, as assessed by receiver operator characteristics curve analysis. Finally, logistic regression revealed that, when comparing sJAM-A to sJAM-C, the latter might be better suited as a biomarker for SSc-related DUs. Our promising findings provide the necessary groundwork for longitudinal follow-up analyses of SSc patients aiming to assess whether circulating sJAM-C levels might be predictive for the development of new DUs, as well as DU recurrence and/or refractoriness to targeted therapies. FAU - Romano, Eloisa AU - Romano E AUID- ORCID: 0000-0003-2744-8625 AD - Section of Internal Medicine, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. FAU - Rosa, Irene AU - Rosa I AUID- ORCID: 0000-0001-6352-0175 AD - Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. FAU - Fioretto, Bianca Saveria AU - Fioretto BS AUID- ORCID: 0000-0001-9275-7593 AD - Section of Internal Medicine, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. AD - Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Section of Internal Medicine, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy. FAU - Manetti, Mirko AU - Manetti M AUID- ORCID: 0000-0003-3956-8480 AD - Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy. LA - eng PT - Journal Article DEP - 20221104 PL - Switzerland TA - Life (Basel) JT - Life (Basel, Switzerland) JID - 101580444 PMC - PMC9695837 OTO - NOTNLM OT - digital ulcers OT - enzyme-linked immunosorbent assay OT - junctional adhesion molecules OT - peripheral microvascular damage OT - sJAM-A OT - sJAM-C OT - scleroderma OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2022/11/12 06:00 MHDA- 2022/11/12 06:01 PMCR- 2022/11/04 CRDT- 2022/11/11 01:33 PHST- 2022/10/04 00:00 [received] PHST- 2022/10/25 00:00 [revised] PHST- 2022/11/03 00:00 [accepted] PHST- 2022/11/11 01:33 [entrez] PHST- 2022/11/12 06:00 [pubmed] PHST- 2022/11/12 06:01 [medline] PHST- 2022/11/04 00:00 [pmc-release] AID - life12111790 [pii] AID - life-12-01790 [pii] AID - 10.3390/life12111790 [doi] PST - epublish SO - Life (Basel). 2022 Nov 4;12(11):1790. doi: 10.3390/life12111790. PMID- 31808306 OWN - NLM STAT- MEDLINE DCOM- 20201216 LR - 20201216 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 23 IP - 2 DP - 2020 Feb TI - Inhomogeneity of capillaroscopic findings in systemic sclerosis. PG - 207-215 LID - 10.1111/1756-185X.13760 [doi] AB - BACKGROUND: Despite the great interest in capillaroscopy in systemic sclerosis (SSc), research on the possible combinations of different microvascular phenomena at different fingers in SSc patients have not been performed until now. OBJECTIVE: To assess the diversity of capillaroscopic findings in SSc. METHODS: The study includes analysis of the capillaroscopic findings in 40 SSc patients who were divided into the following categories: "scleroderma", type pattern - "early", "active", "late" phase, normal and/or nonspecific findings. The data were analyzed using descriptive statistics and t test. RESULTS: In 77% of the patients, inhomogeneity of the capillaroscopic findings of the fingers was detected. The most frequent combinations of capillaroscopic patterns were "early + active" (n = 7), "active + late" phase (n = 7), "active" phase + normal and/or nonspecific findings (n = 6), "early + active" phase + normal and/or nonspecific findings (n = 4). Concomitant presence of normal and/or nonspecific findings and "late" phase was detected in only 3 cases. CONCLUSION: Inhomogeneity of the capillaroscopic findings in SSc is a frequent phenomenon. The results indicate that combinations of "scleroderma" type capillaroscopic findings from different phases could be observed as well as concomitant appearance of pathological and normal/or nonspecific findings of different digits. This phenomenon could be a result of the complex action of different factors eg, disease duration, severity of Raynaud's phenomenon, presence of digital ulcers, local action of different angiogenic and angiostatic factors, gradual transition from one phase to another due to the extensive capillary area, therapeutic interventions. CI - © 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AUID- ORCID: 0000-0002-1321-6770 AD - Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University, Plovdiv, Bulgaria. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Internal Medicine and Rheumatology, Justus-Liebig University Giessen, Giessen, Germany. AD - Department of Rheumatology and Clinical Immunology, Campus Kerckhoff, Bad Nauheim, Germany. LA - eng PT - Journal Article DEP - 20191205 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Capillaries/diagnostic imaging/*physiopathology MH - Female MH - Fingers/*blood supply MH - Humans MH - Male MH - Microcirculation/*physiology MH - Microscopic Angioscopy/*methods MH - Middle Aged MH - Scleroderma, Systemic/*diagnosis/physiopathology OTO - NOTNLM OT - "scleroderma" type capillaroscopic pattern OT - Inhomogeneity OT - systemic sclerosis EDAT- 2019/12/07 06:00 MHDA- 2020/12/17 06:00 CRDT- 2019/12/07 06:00 PHST- 2019/06/28 00:00 [received] PHST- 2019/10/09 00:00 [revised] PHST- 2019/11/13 00:00 [accepted] PHST- 2019/12/07 06:00 [pubmed] PHST- 2020/12/17 06:00 [medline] PHST- 2019/12/07 06:00 [entrez] AID - 10.1111/1756-185X.13760 [doi] PST - ppublish SO - Int J Rheum Dis. 2020 Feb;23(2):207-215. doi: 10.1111/1756-185X.13760. Epub 2019 Dec 5. PMID- 35382237 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240824 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 3 IP - 2 DP - 2018 Jun TI - Therapeutic plasma exchange for the treatment of systemic sclerosis: A comprehensive review and analysis. PG - 132-152 LID - 10.1177/2397198318758606 [doi] AB - BACKGROUND: Therapeutic plasma exchange has been tried as a treatment approach for systemic sclerosis since 1978 based on the rationale that some circulating factor is involved in disease pathogenesis, for example, autoantibodies or immune complexes, and that removing the potential pathogenic factors could lead to symptom improvement. Based on our impression that clinicians and researchers are largely unaware that a large volume of research has been published about the use of therapeutic plasma exchange as a treatment for systemic sclerosis, we conducted a comprehensive review and analysis of all published research on this topic. RESULTS: We identified 46 relevant articles that met our search criteria, involving a total of 572 patients. Of these, 19 were case studies; the rest ranged from small observational studies to prospective randomized clinical trials. In all but two studies, most patients receiving therapeutic plasma exchange showed improvements in both clinical symptoms and laboratory markers, including significant improvement in Raynaud's symptoms and healing of digital ulceration after three to four weekly treatments. The beneficial effects from even a short course of therapeutic plasma exchange treatments were long-lasting, typically 6 months or longer. Therapeutic plasma exchange was very well tolerated. Adverse events were rare and, in almost all cases, mild and transitory. CONCLUSION: These results suggest that long-term therapeutic plasma exchange may offer a low-risk way to control and in some cases reverse systemic sclerosis symptoms. The mechanism for the clinical improvements seen from therapeutic plasma exchange in systemic sclerosis patients is unclear. Therefore, additional studies of therapeutic plasma exchange effects in systemic sclerosis appear to be highly desirable. CI - © The Author(s) 2018. FAU - Harris, Edward S AU - Harris ES AD - Department of Medicine, University of Wisconsin, Madison, WI, USA. FAU - Meiselman, Herbert J AU - Meiselman HJ AD - Department of Physiology & Biophysics, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. FAU - Moriarty, Patrick M AU - Moriarty PM AD - Division of Clinical Pharmacology, University of Kansas Medical Center, Lawrence, KS, USA. FAU - Metzger, Allan AU - Metzger A AD - RDL Reference Laboratory, Los Angeles, CA, USA. FAU - Malkovsky, Miroslav AU - Malkovsky M AD - Department of Medical Microbiology and Immunology, University of Wisconsin, Madison, WI, USA. LA - eng PT - Journal Article PT - Review DEP - 20180309 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8892860 OTO - NOTNLM OT - Therapeutic plasma exchange OT - hyperviscosity OT - mixed connective tissue disorder OT - plasmapheresis OT - systemic sclerosis OT - therapeutic apheresis COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2018/06/01 00:00 MHDA- 2018/06/01 00:01 PMCR- 2018/03/09 CRDT- 2022/04/06 05:13 PHST- 2017/08/21 00:00 [received] PHST- 2018/01/03 00:00 [accepted] PHST- 2022/04/06 05:13 [entrez] PHST- 2018/06/01 00:00 [pubmed] PHST- 2018/06/01 00:01 [medline] PHST- 2018/03/09 00:00 [pmc-release] AID - 10.1177_2397198318758606 [pii] AID - 10.1177/2397198318758606 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2018 Jun;3(2):132-152. doi: 10.1177/2397198318758606. Epub 2018 Mar 9. PMID- 29686439 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220318 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 56 IP - 1 DP - 2018 TI - Clinical pattern of systemic sclerosis in Central Ukraine. Association between clinical manifestations of systemic sclerosis and hypertension. PG - 24-30 LID - 10.5114/reum.2018.74745 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is a rare disease of connective tissue, manifestations of which may vary in different geographical areas. We aimed to describe the clinical portrait of patients with SSc in Dnipropetrovsk region and to investigate how initial clinical and laboratory characteristics are connected with the presence of hypertension in SSc onset. MATERIAL AND METHODS: Patients were enrolled to this study from the registry of SSc patients, established in the Rheumatology Department, Mechnikov Dnipropetrovsk Regional Clinic, Dnipro. This registry contains histories of new cases of SSc from 1993 to 2014. Patients are followed-up and receive treatment according to EULAR and local standards. Diagnosis of SSc was based on ACR and EULAR Criteria for systemic Sclerosis. Two patients developed scleroderma renal crisis during follow-up. This report is a cross-sectional study. We analysed only data of the first visit to a rheumatologist. RESULTS: In total 148 patients (median age [IQR] - 47 [40; 52] years) fulfilled the inclusion criteria. Male/female ratio was 1 : 20.1. The most frequent clinical signs were Raynaud's phenomenon and arthritis. The prevalence of skin lesion in dcSSc patients was twice as high as in lcSSc patients. Pulmonary fibrosis occurred significantly more commonly in dcSSc patients. Hypertension occurred in 26-33% in both groups. Patients with hypertension at the SSc onset were seven years older than normotensive patients. More hypertensive patients were classified as lcSSc. Mean GFR was dramatically lower in hypertensive patients. CONCLUSIONS: The most common clinical form in our study was diffuse cutaneous subset of SSc. Hypertension in patients with SSc may be associated with local cutaneous subset of SSc and renal impairment. The strongest predictors of clinical form of SSc are signs of fibrosis (skin lesion and pulmonary fibrosis) and inflammation (arthritis and elevated CRP). FAU - Semenov, Viktor AU - Semenov V AD - Internal Medicine 2, State Establishment "Dnipropetrovsk Medical Academy of Health Ministry of Ukraine". FAU - Kuryata, Olexandr AU - Kuryata O AD - Internal Medicine 2, State Establishment "Dnipropetrovsk Medical Academy of Health Ministry of Ukraine". FAU - Lysunets, Tatiana AU - Lysunets T AD - Rheumatology Department, Municipal institution "Mechnikov Dnipropetrovsk regional clinic", Dnipro, Ukraine. LA - eng PT - Journal Article DEP - 20180228 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC5911654 OTO - NOTNLM OT - arterial hypertension OT - registry OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2018/04/25 06:00 MHDA- 2018/04/25 06:01 PMCR- 2018/01/01 CRDT- 2018/04/25 06:00 PHST- 2018/01/24 00:00 [received] PHST- 2018/02/23 00:00 [accepted] PHST- 2018/04/25 06:00 [entrez] PHST- 2018/04/25 06:00 [pubmed] PHST- 2018/04/25 06:01 [medline] PHST- 2018/01/01 00:00 [pmc-release] AID - 32403 [pii] AID - 10.5114/reum.2018.74745 [doi] PST - ppublish SO - Reumatologia. 2018;56(1):24-30. doi: 10.5114/reum.2018.74745. Epub 2018 Feb 28. PMID- 26150271 OWN - NLM STAT- MEDLINE DCOM- 20170320 LR - 20170320 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 67 IP - 1 DP - 2015 Jun 30 TI - Systemic sclerosis sine scleroderma: a case report of anterior uveitis. PG - 21-5 LID - 10.4081/reumatismo.2015.818 [doi] AB - Systemic sclerosis (SSc) sine scleroderma (ssSSc) is characterized by the absence of skin involvement, despite other manifestations of systemic sclerosis are present. It is not known whether sSSc represents a forme fruste of limited cutaneous SSc or a distinct entity, but the 2013 American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc have considered SSc without skin involvement to be a distinct subset. The authors present the case of a 70-year old female that was referred for a consultation for Raynaud's phenomenon and a chronic anterior uveitis (CAU). She had a history of dysphagia, diffuse pulmonary emphysema and a biopsy-documented fibrosis of the upper lobes, and an idiopathic non-ischemic dilated cardiomyopathy with severe left ventricle systolic dysfunction and left bundle branch block. Anti-nuclear and anti-centromere antibodies were positive, while manometry revealed distal esophageal hypomotility. After establishing the diagnosis of ssSSc and starting immunosuppression, the ocular disease improved, while the lung and heart diseases remained stable. This case underlines that it is very important to suspect SSc when CAU is present and/or skin thickening is absent. To our knowledge, this is the first report of CAU in a patient with ssSSc. FAU - Borges, T AU - Borges T AD - Internal Medicine Department, Centro Hospitalar São João, Al Prof Hernâni Monteiro, Porto. mtiago.b@hotmail.com. FAU - Vilaça, J AU - Vilaça J FAU - Ferreira, S AU - Ferreira S FAU - Chora, I AU - Chora I FAU - Silva, S AU - Silva S FAU - Dias, C AU - Dias C LA - eng PT - Case Reports PT - Journal Article DEP - 20150630 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) SB - IM MH - Aged MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Risk Factors MH - Scleroderma, Diffuse/immunology MH - Scleroderma, Limited/immunology MH - Scleroderma, Systemic/blood/complications/drug therapy/*immunology MH - Treatment Outcome MH - Uveitis, Anterior/blood/drug therapy/etiology/*immunology EDAT- 2015/07/08 06:00 MHDA- 2017/03/21 06:00 CRDT- 2015/07/08 06:00 PHST- 2015/01/17 00:00 [received] PHST- 2015/03/19 00:00 [accepted] PHST- 2015/07/08 06:00 [entrez] PHST- 2015/07/08 06:00 [pubmed] PHST- 2017/03/21 06:00 [medline] AID - 10.4081/reumatismo.2015.818 [doi] PST - epublish SO - Reumatismo. 2015 Jun 30;67(1):21-5. doi: 10.4081/reumatismo.2015.818. PMID- 19860698 OWN - NLM STAT- MEDLINE DCOM- 20100105 LR - 20220321 IS - 1873-4286 (Electronic) IS - 1381-6128 (Linking) VI - 15 IP - 30 DP - 2009 TI - PDE5 inhibitors in non-urological conditions. PG - 3521-39 AB - Phosphodiesterase type-5 (PDE5) inhibitors are widely used as first-line therapy for erectile dysfunction (ED). Their efficacy and safety combined with an increasing understanding of cyclic guanosine monophosphate (cGMP)-regulated mechanisms, have triggered a number of attempts to determine their effects and potential benefits in non-urological conditions. In recent years, extensive and diverse preclinical and clinical evidence has been made available. PDE5 inhibition has shown collateral benefits for a multitude of risk factors or diseases associated with, or accompanying ED. To date, PDE5 inhibition has been shown to be effective for the treatment of idiopathic pulmonary arterial hypertension and both sildenafil and tadalafil are approved for this indication. However, PDE5 inhibitors appear to have the potential of further expanding their indications. Importantly, accumulating data show that the therapeutic potential extends to the cardiovascular, gastrointestinal, cutaneous and nervous system and that these agents may be beneficial in a multitude of conditions such as Raynaud's phenomenon, heart failure, essential hypertension and stroke. PDE5 inhibitors are a conceptually attractive therapeutic class of agents with pleiotropic effects. The present review discusses recent findings regarding the effects of PDE5 inhibitors on non-urological conditions and highlights current and future clinical applications beyond ED. FAU - Vlachopoulos, C AU - Vlachopoulos C AD - Cardiovascular Diseases and Sexual Health Unit, 1st Department of Cardiology, Athens Medical School, Hippokration Hospital, Athens, Greece. cvlachop@otenet.gr FAU - Terentes-Printzios, D AU - Terentes-Printzios D FAU - Ioakeimidis, N AU - Ioakeimidis N FAU - Rokkas, K AU - Rokkas K FAU - Stefanadis, C AU - Stefanadis C LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Pharm Des JT - Current pharmaceutical design JID - 9602487 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Phosphodiesterase Inhibitors) SB - IM MH - Alzheimer Disease/drug therapy MH - Animals MH - Cardiovascular Diseases/drug therapy MH - Endocrine System Diseases/drug therapy MH - Eye Diseases/drug therapy MH - Gastrointestinal Diseases/drug therapy MH - Hematologic Diseases/drug therapy MH - Humans MH - Lung Diseases/drug therapy MH - Neoplasms/drug therapy MH - Nervous System Diseases/drug therapy MH - Organ Transplantation MH - *Phosphodiesterase 5 Inhibitors MH - Phosphodiesterase Inhibitors/*therapeutic use MH - Skin Diseases/drug therapy MH - Stroke/drug therapy RF - 236 EDAT- 2009/10/29 06:00 MHDA- 2010/01/06 06:00 CRDT- 2009/10/29 06:00 PHST- 2009/10/29 06:00 [entrez] PHST- 2009/10/29 06:00 [pubmed] PHST- 2010/01/06 06:00 [medline] AID - 10.2174/138161209789206980 [doi] PST - ppublish SO - Curr Pharm Des. 2009;15(30):3521-39. doi: 10.2174/138161209789206980. PMID- 19850657 OWN - NLM STAT- MEDLINE DCOM- 20100114 LR - 20250513 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 18 IP - 14 DP - 2009 Dec TI - An analysis of joint replacement in patients with systemic lupus erythematosus. PG - 1298-302 LID - 10.1177/0961203309345795 [doi] AB - A small but important group of patients in our lupus cohort has needed total joint replacement (TJR). Arthritis was identified in 94% of our lupus patients. We have determined how many of our patients needed TJR, explored the risk factors for this procedure in patients with SLE and reviewed the outcome for these patients. Records of the cohort of patients with SLE who have attended our lupus clinic at University College of London Hospital/Middlesex from 1978 to 2008 were reviewed and patients who underwent TJR were identified. We recorded demographic data, other major systemic manifestations of SLE, autoantibody profile, previous use of steroids, other major systemic illnesses, smoking and alcohol habits. Nineteen patients with SLE from our cohort of 500 were found to have at least one TJR. Avascular necrosis (AVN) or concomitant rheumatoid arthritis (RA) was present in the majority of these patients. In contrast, age at disease onset, the presence of anti-cardiolipin antibodies, Raynaud's phenomenon and smoking habits were not found to be contributing factors for the need to replace joints. Four of our 19 patients (21.1%) had complications of the joint replacement: two of them had infections of the replaced joint, one had a large haematoma immediately after the surgery requiring surgical evacuation and the other had a deep vein thrombosis. None of the patients so far has required joint re-replacement. In conclusion, 4% of SLE patients in our cohort have one or more joints replaced, the majority because of AVN or RA. FAU - Mourão, A F AU - Mourão AF AD - Centre For Rheumatology, Department of Medicine, University College of London Hospital, London, UK. filipamourao@yahoo.com FAU - Amaral, M AU - Amaral M FAU - Caetano-Lopes, J AU - Caetano-Lopes J FAU - Isenberg, D AU - Isenberg D LA - eng PT - Journal Article DEP - 20091022 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM CIN - Acta Orthop. 2016 Jul;87 Suppl 1:9-23. doi: 10.1080/17453674.2016.1181816. PMID: 27228230 MH - Adolescent MH - Adult MH - Arthritis, Rheumatoid/*epidemiology/surgery MH - Arthroplasty, Replacement/*statistics & numerical data MH - Female MH - Humans MH - London/epidemiology MH - Lupus Erythematosus, Systemic/*epidemiology MH - Middle Aged MH - Osteonecrosis/*epidemiology/surgery MH - Postoperative Complications/*epidemiology MH - Prevalence MH - Retrospective Studies MH - Risk Factors MH - Young Adult EDAT- 2009/10/24 06:00 MHDA- 2010/01/15 06:00 CRDT- 2009/10/24 06:00 PHST- 2009/10/24 06:00 [entrez] PHST- 2009/10/24 06:00 [pubmed] PHST- 2010/01/15 06:00 [medline] AID - 0961203309345795 [pii] AID - 10.1177/0961203309345795 [doi] PST - ppublish SO - Lupus. 2009 Dec;18(14):1298-302. doi: 10.1177/0961203309345795. Epub 2009 Oct 22. PMID- 18821172 OWN - NLM STAT- MEDLINE DCOM- 20090623 LR - 20090123 IS - 1502-7708 (Electronic) IS - 0036-5521 (Linking) VI - 44 IP - 2 DP - 2009 TI - Pulmonary hypertension in primary biliary cirrhosis: a prospective study in 178 patients. PG - 219-23 LID - 10.1080/00365520802400883 [doi] AB - OBJECTIVE. To analyze the incidence, clinical features, and prognosis of patients with primary biliary cirrhosis (PBC) complicated by pulmonary hypertension (PH). MATERIAL AND METHODS: A total of 178 consecutive PBC patients, who were admitted to Peking Union Medical College Hospital from January 2001 to March 2007, were included in this prospective study. A structured interview, systemic rheumatological examination, laboratory tests (including autoantibodies), and Doppler echocardiography were conducted for each patient and compared between patients with and without PH. RESULTS: Twenty-one PBC patients (11.8%) had PH. Among them, four patients (19.0%) had moderate to severe PH, and one patient died of right heart failure instead of liver failure. The incidences of Raynaud's phenomenon, interstitial lung disease, Sjögren's syndrome, and portal hypertension, the proportion of patients with a positive anti-SSA, the level of serum IgA, as well as the Mayo risk score in the PH-PBC patients were significantly higher than in the non-PH-PBC group (p = 0.02, 0.001, 0.02, 0.03, 0.006, 0.04 and 0.02, respectively). CONCLUSIONS: PH, including moderate to severe PH, is not a rare complication of PBC. This complication is closely associated with portal hypertension and immunological dysregulation and indicates a poor prognosis. FAU - Shen, Min AU - Shen M AD - Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China. FAU - Zhang, Fengchun AU - Zhang F FAU - Zhang, Xuan AU - Zhang X LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Scand J Gastroenterol JT - Scandinavian journal of gastroenterology JID - 0060105 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Hypertension, Pulmonary/*complications/*epidemiology MH - Incidence MH - Liver Cirrhosis, Biliary/*complications/*epidemiology MH - Male MH - Middle Aged MH - Prospective Studies MH - Severity of Illness Index EDAT- 2008/09/30 09:00 MHDA- 2009/06/24 09:00 CRDT- 2008/09/30 09:00 PHST- 2008/09/30 09:00 [pubmed] PHST- 2009/06/24 09:00 [medline] PHST- 2008/09/30 09:00 [entrez] AID - 903068204 [pii] AID - 10.1080/00365520802400883 [doi] PST - ppublish SO - Scand J Gastroenterol. 2009;44(2):219-23. doi: 10.1080/00365520802400883. PMID- 37959314 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231117 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 12 IP - 21 DP - 2023 Oct 30 TI - Clinical Characteristics of Anti-Synthetase Syndrome and Variables Associated with Interstitial Lung Disease and Mortality: A Retrospective Cohort Study. LID - 10.3390/jcm12216849 [doi] LID - 6849 AB - Anti-synthetase syndrome (ASS) is a rare autoimmune disease. Since the knowledge of ASS remains limited, we conducted the retrospective study aiming to describe clinical characteristics and identify variables associated with interstitial lung disease (ILD) and mortality among patients with ASS. Patients diagnosed with ASS from January 2013 to October 2022 were included. Patient demographics, clinical manifestations, myositis auto-antibody profiles, HRCT findings, and laboratory tests were collected. Variables associated with mortality risk and ILD were evaluated using the Cox proportional hazards model and the logistic regression model, respectively. A total of 82 patients with ASS were included. Clinical manifestations included arthritis (57%), Raynaud's phenomenon (32%), mechanic's hands (29%), fever (26%), and myositis (17%). The myositis auto-antibody profiles included anti-PL-7 (29%), anti-Jo-1 (27%), anti-EJ (17%), anti-PL-12 (16%), and anti-OJ (11%). ILD was observed in 64 patients (78%). Among patients with ILD, 21 initially presented with ILD before developing other ASS clinical manifestations, 29 simultaneously presented with ILD and other symptoms, and 14 had isolated ILD throughout follow-up. Overall, 6 patients presented with rapid-progressive ILD. With a median follow-up time of 2.5 years, mortality was observed in 10 patients (12.2%). Factors associated with mortality included increased lymphocyte counts (adjusted HR, 0.74; 95% CI, 0.61-0.91; p < 0.01), isolated ILD (adjusted HR, 9.59; 95% CI, 1.52-60.61; p = 0.02) and the presence of anti-Ro52 antibodies (adjusted HR, 0.14; 95% CI, 0.02-0.93; p = 0.04). Factors associated with ILD included age (adjusted OR, 1.10; 95% CI, 1.03-1.18; p = 0.01), presence of anti-Ro52 antibodies (adjusted OR, 17.92; 95% CI, 2.13-138.68; p = 0.01), and presence of arthritis (adjusted OR, 0.09; 95% CI, 0.01-0.75; p = 0.03). Our study demonstrated a favorable overall mortality rate among ASS patients. FAU - Sodsri, Tulaton AU - Sodsri T AUID- ORCID: 0000-0002-8999-5133 AD - Chakri Naruebodindra Medical Institute, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Samut Prakan 10540, Thailand. FAU - Petnak, Tananchai AU - Petnak T AUID- ORCID: 0000-0002-7633-4029 AD - Division of Pulmonary and Pulmonary Critical Care Medicine, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand. FAU - Ngamjanyaporn, Pintip AU - Ngamjanyaporn P AUID- ORCID: 0000-0001-7948-1748 AD - Division of Allergy, Immunology and Rheumatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand. LA - eng PT - Journal Article DEP - 20231030 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC10648265 OTO - NOTNLM OT - anti-synthetase syndrome OT - autoantibodies OT - idiopathic inflammatory myositis OT - interstitial lung disease OT - mortality COIS- The authors declare no conflict of interest. EDAT- 2023/11/14 06:42 MHDA- 2023/11/14 06:43 PMCR- 2023/10/30 CRDT- 2023/11/14 02:13 PHST- 2023/09/30 00:00 [received] PHST- 2023/10/22 00:00 [revised] PHST- 2023/10/23 00:00 [accepted] PHST- 2023/11/14 06:43 [medline] PHST- 2023/11/14 06:42 [pubmed] PHST- 2023/11/14 02:13 [entrez] PHST- 2023/10/30 00:00 [pmc-release] AID - jcm12216849 [pii] AID - jcm-12-06849 [pii] AID - 10.3390/jcm12216849 [doi] PST - epublish SO - J Clin Med. 2023 Oct 30;12(21):6849. doi: 10.3390/jcm12216849. PMID- 24609069 OWN - NLM STAT- MEDLINE DCOM- 20150209 LR - 20220409 IS - 1464-3685 (Electronic) IS - 0300-5771 (Linking) VI - 43 IP - 3 DP - 2014 Jun TI - Body mass index and risk of autoimmune diseases: a study within the Danish National Birth Cohort. PG - 843-55 LID - 10.1093/ije/dyu045 [doi] AB - BACKGROUND: A possible aetiological link between obesity and certain autoimmune diseases (ADs) has been suggested. We investigated the associations between body mass index (BMI, kg/m2) and 43 ADs. METHODS: 75,008 women participating in the Danish National Birth Cohort were followed during a median time of 11 years. Diagnoses on ADs were retrieved from the Danish National Patient Register. Cox proportional hazard ratios (HRs) with 95% confidence intervals (CIs) were calculated adjusting for potential confounders (smoking, alcohol, parity and socio-occupational status). RESULTS: During follow-up, 2430 women (3.2%) developed a total of 2607 new-onset ADs. Risk of any autoimmune disease was increased in obese women (HR, 1.27; 95% CI, 1.11 to 1.46) compared with normal weight women (18.5-≤25 kg/m2). Obese women (BMI≥30 kg/m2) were at increased risk of sarcoidosis (HR 3.59; 95% CI, 2.31 to 5.57) and type 1 diabetes mellitus (HR 2.67; 95% CI, 1.71 to 4.17). Risk of dermatitis herpetiformis increased by 14% (95% CI, 1% to 30%) per BMI unit. Conversely, risk of celiac disease and Raynaud's phenomenon decreased by 7% (95% CI, 1% to 13%) and 12% (95% CI, 4% to 19%) per BMI unit, respectively. Further associations between BMI and risk of psoriasis, rheumatoid arthritis and Crohn's disease were suggested. CONCLUSIONS: BMI was found to be associated with several Ads. This was most pronounced between obesity and risk of sarcoidosis and and risk of type 1 diabetes mellitus. These novel findings need confirmation and the possible role of adipose tissue-derived immunological changes in the development of autoimmune reactions needs consideration. CI - © The Author 2014; all rights reserved. Published by Oxford University Press on behalf of the International Epidemiological Association. FAU - Harpsøe, Maria C AU - Harpsøe MC AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark rrh@ssi.dk. FAU - Basit, Saima AU - Basit S AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Andersson, Mikael AU - Andersson M AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Nielsen, Nete M AU - Nielsen NM AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Frisch, Morten AU - Frisch M AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Wohlfahrt, Jan AU - Wohlfahrt J AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Nohr, Ellen A AU - Nohr EA AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Linneberg, Allan AU - Linneberg A AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. FAU - Jess, Tine AU - Jess T AD - Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark, Section for Epidemiology, Department of Public Health, Aarhus University, Aarhus, and Department of Obstetrics and Gynaecology, Odense University Hospital, Odense, Denmark and Research Centre for Prevention and Health, Glostrup University Hospital, Glostrup, Denmark. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140307 PL - England TA - Int J Epidemiol JT - International journal of epidemiology JID - 7802871 SB - IM CIN - Int J Epidemiol. 2015 Feb;44(1):363-4. doi: 10.1093/ije/dyu133. PMID: 24997209 MH - Adult MH - Autoimmune Diseases/*epidemiology MH - *Body Mass Index MH - Cohort Studies MH - Denmark/epidemiology MH - Female MH - Health Behavior MH - Humans MH - Incidence MH - Longitudinal Studies MH - Obesity/*epidemiology MH - Risk Factors MH - Socioeconomic Factors OTO - NOTNLM OT - Body mass index OT - autoimmune disease OT - inflammation OT - leptin OT - obesity EDAT- 2014/03/13 06:00 MHDA- 2015/02/11 06:00 CRDT- 2014/03/11 06:00 PHST- 2014/03/11 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2015/02/11 06:00 [medline] AID - dyu045 [pii] AID - 10.1093/ije/dyu045 [doi] PST - ppublish SO - Int J Epidemiol. 2014 Jun;43(3):843-55. doi: 10.1093/ije/dyu045. Epub 2014 Mar 7. PMID- 38556034 OWN - NLM STAT- MEDLINE DCOM- 20240521 LR - 20250115 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 23 IP - 5 DP - 2024 May TI - The 'whole landscape' of research on systemic sclerosis over the past 73 years. PG - 103538 LID - S1568-9972(24)00029-6 [pii] LID - 10.1016/j.autrev.2024.103538 [doi] AB - OBJECTIVE: This study aimed to analyse existing research on systemic sclerosis (SSc) conducted over the past 73 years to develop an essential reference for a comprehensive and objective understanding of this field of inquiry. METHODS: Using the Web of Science Core Collection, PubMed, and Scopus databases as data sources for the bibliometric analysis, we searched for published literature related to SSc over the past 73 years. The Bibliometrix package was used to analyse key bibliometric indicators, such as annual publication volume, countries, journals, author contributions, and research hotspots. RESULTS: From 1970 to 2022, the number of SSc articles steadily increased, reaching its peak in 2020-2022, with approximately 1200 papers published in each of these three years. Matucci-Cerinic et al.'s team published the most articles (425). The United States (11,282), Italy (7027), and France (5226) were the most predominant contexts. The most influential scholars in the field were Denton, Leroy, Steen, and Khanna, with H-indices of 86, 84, and 83, respectively. Arthritis and Rheumatism was the most influential journal in this field (H-index 142). High-frequency keywords in the SSc field included fibrosis (738), inflammation (242), vasculopathy (145), fibroblasts (120), and autoantibodies (118) with respect to pathogenesis, and interstitial lung disease (ILD, 708), pulmonary arterial hypertension (PAH, 696), and Raynaud's phenomenon (326) with regards to clinical manifestations. CONCLUSION: In the past three years, SSc research has entered a period of rapid development, mainly driven by research institutions in Europe and the United States. The most influential journal has been Arthritis and Rheumatism, and autoimmune aspects, vasculopathy, fibrogenesis, PAH, and ILD remain the focus of current research and indicate trends in future research. CI - Copyright © 2024 Elsevier B.V. All rights reserved. FAU - Zhang, Meng-Di AU - Zhang MD AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Huang, Wan-Ying AU - Huang WY AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Luo, Jia-Yuan AU - Luo JY AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - He, Rong-Quan AU - He RQ AD - Department of Medical Oncology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Huang, Zhi-Guang AU - Huang ZG AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Li, Jian-Di AU - Li JD AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Qin, Fang AU - Qin F AD - Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. FAU - Chen, Gang AU - Chen G AD - Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. Electronic address: chengang@gxmu.edu.cn. FAU - Lei, Ling AU - Lei L AD - Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, No. 6, Shuangyong Road, 530021 Nanning, PR China. Electronic address: leiling1972@aliyun.com. LA - eng PT - Historical Article PT - Journal Article DEP - 20240329 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Humans MH - *Bibliometrics MH - Biomedical Research/trends/history MH - History, 21st Century MH - *Scleroderma, Systemic/history/immunology/physiopathology MH - History, 20th Century OTO - NOTNLM OT - Bibliometrics OT - Interstitial lung disease OT - Pulmonary arterial hypertension OT - Research hotspots OT - Systemic sclerosis COIS- Declaration of competing interest Meng-Di Zhang, Wan-Ying Huang, Jia-Yuan Luo, Rong-Quan He, Zhi-Guang Huang, Jian-Di Li, Fang Qin, Gang Chen and Ling Lei declare that they have no conflict of interest. EDAT- 2024/04/01 00:42 MHDA- 2024/05/22 01:52 CRDT- 2024/03/31 20:23 PHST- 2024/01/08 00:00 [received] PHST- 2024/03/28 00:00 [revised] PHST- 2024/03/28 00:00 [accepted] PHST- 2024/05/22 01:52 [medline] PHST- 2024/04/01 00:42 [pubmed] PHST- 2024/03/31 20:23 [entrez] AID - S1568-9972(24)00029-6 [pii] AID - 10.1016/j.autrev.2024.103538 [doi] PST - ppublish SO - Autoimmun Rev. 2024 May;23(5):103538. doi: 10.1016/j.autrev.2024.103538. Epub 2024 Mar 29. PMID- 30886958 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 2 DP - 2018 TI - High prevalence of protein tyrosine phosphatase non-receptor N22 gene functional variant R620W in systemic lupus erythematosus patients from Kuwait: implications for disease susceptibility. PG - 7 LID - 10.1186/s41927-018-0015-x [doi] LID - 7 AB - BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease which involves the loss of self-tolerance with hyperactivation of autoreactive T- and B-cells. Protein tyrosine phosphatase non-receptor type 22 (PTPN22) encodes for lymphoid specific phosphatase (LYP) which is a key negative regulator of T lymphocyte activation. The aim of this study was to investigate the association between PTPN22 gene functional variant R620W and systemic lupus erythematosus (SLE) by comparing its prevalence in Kuwaiti SLE patients and controls. METHODS: The study included 134 SLE patients and 214 controls from Kuwait. The genotypes of PTPN22 gene functional variant R620W were determined by PCR-RFLP and confirmed by DNA sequence analysis in both SLE patients and the controls. RESULTS: A relatively high prevalence of the variant 620 W (T-allele) of the PTPN22 gene was detected in the SLE patients from Kuwait. 35.7% of the SLE patients had at least one variant allele (T-allele) compared to 15.9% in the controls. A statistically significant difference was detected in the frequency of variant genotypes, TT and CT between SLE patients and the controls (p < 0.0001). No association was detected between the PTPN22 gene variant and the Raynaud's phenomenon, renal involvement and severity of the SLE. CONCLUSIONS: The frequency of PTPN22 gene functional variant R620W reported in this study is amongst the highest compared to other world populations. A high prevalence of this variant in SLE patients in comparison to the healthy controls suggests its significant contribution in conferring susceptibility to SLE together with other factors. FAU - Al-Awadhi, Adel M AU - Al-Awadhi AM AD - 1Department of Medicine, Faculty of Medicine, Kuwait University, Jabriya, Kuwait. ISNI: 0000 0001 1240 3921. GRID: grid.411196.a AD - 2Rheumatic Disease Unit, Al-Amiri Hospital, Dasman, Kuwait. GRID: grid.413513.1 FAU - Haider, Mohammad Z AU - Haider MZ AUID- ORCID: 0000-0002-8187-0061 AD - 3Department of Pediatrics, Faculty of Medicine, Kuwait University, P. O. Box 24923, 13110 Safat, Kuwait. ISNI: 0000 0001 1240 3921. GRID: grid.411196.a FAU - Sukumaran, Jalaja AU - Sukumaran J AD - 3Department of Pediatrics, Faculty of Medicine, Kuwait University, P. O. Box 24923, 13110 Safat, Kuwait. ISNI: 0000 0001 1240 3921. GRID: grid.411196.a FAU - Balakrishnan, Sowmya AU - Balakrishnan S AD - 3Department of Pediatrics, Faculty of Medicine, Kuwait University, P. O. Box 24923, 13110 Safat, Kuwait. ISNI: 0000 0001 1240 3921. GRID: grid.411196.a LA - eng PT - Journal Article DEP - 20180316 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC6390595 OTO - NOTNLM OT - Functional variant OT - Gene OT - Kuwait OT - Protein tyrosine phosphatase non receptor-22 OT - Systemic lupus erythematosus COIS- This project was approved by the Committee for Protection of Human Subjects in Research by the Kuwait University, Faculty of Medicine (Ref. FOM/213–02). The study has been carried out strictly according to the guidelines of the Committee and as per Helsinki Declaration. Written informed consent was obtained from the study subjects for participation in this study.Not applicable.The authors declared that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/03/20 06:00 MHDA- 2019/03/20 06:01 PMCR- 2018/03/16 CRDT- 2019/03/20 06:00 PHST- 2017/09/10 00:00 [received] PHST- 2018/03/01 00:00 [accepted] PHST- 2019/03/20 06:00 [entrez] PHST- 2019/03/20 06:00 [pubmed] PHST- 2019/03/20 06:01 [medline] PHST- 2018/03/16 00:00 [pmc-release] AID - 15 [pii] AID - 10.1186/s41927-018-0015-x [doi] PST - epublish SO - BMC Rheumatol. 2018 Mar 16;2:7. doi: 10.1186/s41927-018-0015-x. eCollection 2018. PMID- 39986125 OWN - NLM STAT- MEDLINE DCOM- 20250509 LR - 20250509 IS - 1872-8243 (Electronic) IS - 1386-5056 (Linking) VI - 197 DP - 2025 May TI - Predicting and validating the risk of interstitial lung disease in systemic lupus erythematosus. PG - 105839 LID - S1386-5056(25)00056-5 [pii] LID - 10.1016/j.ijmedinf.2025.105839 [doi] AB - OBJECTIVE: Our study aimed toconstruct a web-based calculator to predict high risk patients of interstitial lung disease (ILD) in systemic lupus erythematosus (SLE). METHODS: This retrospective study comprised training and test cohorts, including 581 and 86 patients, respectively. Univariate, least absolute shrinkage and selection operator (LASSO), random forest (RF), eXtreme Gradient Boosting (XGBoost), and logistic regression (LR) analyses were performed. A Venn diagram was used to investigate critical features. Receiver operating characteristic (ROC) analysis and decision curve analysis were used to evaluate the model's performance. Risk stratification was performed using the best ROC cut-off value. The web-based calculator was established using Streamlit software. RESULTS: Characteristics such as Raynaud's phenomenon, pulmonary artery systolic pressure, serositis, anti-U1RNP antibodies, anti-Ro52 antibodies, C-reactive protein, age, and disease course were associated with SLE complicated by ILD (SLE-ILD). LR-Venn, RF-Venn, XGBoost-Venn, LASSO-logic, RF, and XGBoost models were constructed. In training cohort, the XGBoost model demonstrated the highest area under the ROC curve (AUC, 0.890; cut-off value, 0.197; sensitivity, 0.793; specificity, 0.836) and provideda netbenefitin decision curve analysis (odds ratio [OR] for SLE-ILD [high- vs. low-risk], 19.6). The model was validated in the test cohort (AUC, 0.866; sensitivity, 0.722; specificity, 0.897; OR, 22.7). Furthermore, an XGBoost model-based web calculator was developed. CONCLUSION: Our web calculator (https://st-xgboost-app-kcv9qm.streamlit.app/) greatly improved risk prediction for SLE-ILD and was implemented effectively. CI - Copyright © 2025. Published by Elsevier B.V. FAU - Guo, Aoyang AU - Guo A AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China; Department of Rheumatology and Immunology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China; Department of Standardized Training of Residents, Shenzhen People's Hospital, Shenzhen, China. FAU - Chen, Yanran AU - Chen Y AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China. FAU - Liu, Hongyang AU - Liu H AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China. FAU - Gao, Shujun AU - Gao S AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China. FAU - Huang, Xinyi AU - Huang X AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China; Department of Rheumatology and Immunology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China. FAU - Liu, Dongzhou AU - Liu D AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China; Department of Rheumatology and Immunology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China. FAU - Zhao, Qianqian AU - Zhao Q AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China; Department of Rheumatology and Immunology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China. Electronic address: zhaoqianqian@fudan.edu.cn. FAU - Hong, Xiaoping AU - Hong X AD - The Second Clinical Medical College of Jinan University, Department of Rheumatology and Immunology, Shenzhen People's Hospital, Shenzhen, China; Department of Rheumatology and Immunology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China. Electronic address: hong_xiaoping@hotmail.com. LA - eng PT - Journal Article DEP - 20250214 PL - Ireland TA - Int J Med Inform JT - International journal of medical informatics JID - 9711057 SB - IM MH - Humans MH - *Lung Diseases, Interstitial/etiology/diagnosis MH - *Lupus Erythematosus, Systemic/complications MH - Female MH - Retrospective Studies MH - Male MH - Adult MH - Middle Aged MH - ROC Curve MH - Risk Assessment/methods MH - Risk Factors MH - Logistic Models OTO - NOTNLM OT - Interstitial lung disease OT - Risk prediction OT - Systemic lupus erythematosus OT - Web-based calculator COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/02/23 00:42 MHDA- 2025/03/11 00:25 CRDT- 2025/02/22 18:09 PHST- 2025/01/29 00:00 [received] PHST- 2025/02/10 00:00 [revised] PHST- 2025/02/13 00:00 [accepted] PHST- 2025/03/11 00:25 [medline] PHST- 2025/02/23 00:42 [pubmed] PHST- 2025/02/22 18:09 [entrez] AID - S1386-5056(25)00056-5 [pii] AID - 10.1016/j.ijmedinf.2025.105839 [doi] PST - ppublish SO - Int J Med Inform. 2025 May;197:105839. doi: 10.1016/j.ijmedinf.2025.105839. Epub 2025 Feb 14. PMID- 39675699 OWN - NLM STAT- MEDLINE DCOM- 20250426 LR - 20250426 IS - 1898-4002 (Electronic) IS - 1896-1126 (Linking) VI - 70 IP - 1 DP - 2025 Mar TI - Systemic immune-inflammation index in the evaluation of Sjogren's syndrome associated with interstitial lung disease, interstitial pneumonia with autoimmune features, and idiopathic pulmonary fibrosis. PG - 57-61 LID - S1896-1126(24)00064-6 [pii] LID - 10.1016/j.advms.2024.12.001 [doi] AB - PURPOSE: Interstitial lung disease (ILD) damages the lungs and can be caused by environmental exposures and collagen-vascular diseases. The systemic immune-inflammation index (SII) is investigated to diagnose and manage ILDs in different etiological diseases. The study aims to examine the usefulness of SII in diagnosing specific ILDs like Sjogren's syndrome (SjS)-ILD, interstitial pneumonia with autoimmune features (IPAF), and idiopathic pulmonary fibrosis (IPF). MATERIALS AND METHODS: In this cross-sectional study, we included 109 patients with IPAF, IPF, and SjS-ILD. Demographic characteristics, symptoms, lung patterns, autoantibodies, and SII were assessed. Morphologic, serologic, and clinical factors determined the classification of IPAF. Student's t-test, Mann-Whitney U test, Pearson-Spearman's method, and receiver operating characteristic (ROC) curves were used to analyze data. RESULTS: Male patients were more common in IPF and IPAF, while SjS-ILD had mostly female patients. Raynaud's phenomenon and dry mouth/eyes were more common in SjS-ILD compared to IPF and IPAF. The groups had significant differences in patterns, antinuclear antibody positivity, and SII levels. SII levels differed significantly between IPAF, SjS-ILD, and IPF patients, and were correlated with CRP in IPAF and SjS-ILD. The cut-off value of the SII between IPAF and IPF in patients with ILD was 576.1 with 76.0 ​% sensitivity and 76.0 ​% specificity. CONCLUSIONS: Evaluation of SII provides valuable information for understanding and identifying different disease groups with ILDs. CI - Crown Copyright © 2025. Published by Elsevier B.V. All rights reserved. FAU - Sargin, Gokhan AU - Sargin G AD - Department of Rheumatology, Aydin Adnan Menderes University Medical Faculty, Aydin, Turkey. Electronic address: gokhan_sargin@hotmail.com. FAU - Baris, Kursad AU - Baris K AD - Department of Rheumatology, Aydin Adnan Menderes University Medical Faculty, Aydin, Turkey. FAU - Gulen, Sule Tas AU - Gulen ST AD - Department of Chest Diseases, Aydin Adnan Menderes University Medical Faculty, Aydin, Turkey. LA - eng PT - Journal Article DEP - 20241214 PL - Netherlands TA - Adv Med Sci JT - Advances in medical sciences JID - 101276222 SB - IM MH - Humans MH - *Sjogren's Syndrome/immunology/complications/diagnosis MH - Female MH - Male MH - *Lung Diseases, Interstitial/immunology/diagnosis/complications MH - Cross-Sectional Studies MH - Middle Aged MH - *Idiopathic Pulmonary Fibrosis/immunology/diagnosis/complications MH - Aged MH - *Inflammation/immunology MH - ROC Curve MH - *Autoimmune Diseases/immunology/diagnosis OTO - NOTNLM OT - Idiopathic pulmonary fibrosis OT - Interstitial lung disease OT - Interstitial pneumonia with autoimmune features OT - Sjogren's syndrome OT - Systemic immune-inflammation index COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/12/16 11:30 MHDA- 2025/04/26 16:44 CRDT- 2024/12/15 19:20 PHST- 2024/07/13 00:00 [received] PHST- 2024/08/10 00:00 [revised] PHST- 2024/12/13 00:00 [accepted] PHST- 2025/04/26 16:44 [medline] PHST- 2024/12/16 11:30 [pubmed] PHST- 2024/12/15 19:20 [entrez] AID - S1896-1126(24)00064-6 [pii] AID - 10.1016/j.advms.2024.12.001 [doi] PST - ppublish SO - Adv Med Sci. 2025 Mar;70(1):57-61. doi: 10.1016/j.advms.2024.12.001. Epub 2024 Dec 14. PMID- 30092725 OWN - NLM STAT- MEDLINE DCOM- 20190128 LR - 20190128 IS - 1938-9116 (Electronic) IS - 1538-5744 (Linking) VI - 53 IP - 1 DP - 2019 Jan TI - Successful Endovascular Treatment for Aortic Thrombosis Due to Primary Antiphospholipid Syndrome: A Case Report and Literature Review. PG - 51-57 LID - 10.1177/1538574418791355 [doi] AB - A 60-year-old man with a history of Raynaud's phenomenon presented with bilateral intermittent claudication and an ulcer on his right toe. The ankle-brachial index of the right and left legs was 0.77 and 0.75, respectively. Laboratory data showed prolongation of the activated partial thromboplastin time and a positive result on the lupus anticoagulant test. Computed tomography angiography revealed isolated infrarenal aortic stenosis with irregular surface and noncalcified plaques. Intravascular ultrasonography examination demonstrated a noncalcified, irregular, and mobile plaque, suggestive of abdominal aortic thrombosis. In addition to anticoagulant and dual antiplatelet therapy, endovascular treatment was performed. A total of three 40-mm-long balloon-expandable stents were successfully implanted on a 15-mm balloon. The final angiography showed good results except for minimal plaque shifting in the terminal aorta. Three months later, the ulcer resolved and a final diagnosis of primary antiphospholipid syndrome (APS) was made. Clinicians should recognize that APS can affect the abdominal aorta, leading to aortic thrombosis. Endovascular treatment may be the one good treatment option for this rare condition. FAU - Kadoya, Yoshito AU - Kadoya Y AUID- ORCID: 0000-0001-8333-2219 AD - 1 Department of Cardiovascular Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan. AD - 2 Department of Cardiovascular Medicine, Kyotango City Yasaka Hospital, Kyoto, Japan. FAU - Zen, Kan AU - Zen K AD - 1 Department of Cardiovascular Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan. FAU - Oda, Yohei AU - Oda Y AD - 2 Department of Cardiovascular Medicine, Kyotango City Yasaka Hospital, Kyoto, Japan. FAU - Matoba, Satoaki AU - Matoba S AD - 1 Department of Cardiovascular Medicine, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20180809 PL - United States TA - Vasc Endovascular Surg JT - Vascular and endovascular surgery JID - 101136421 SB - IM MH - *Angioplasty, Balloon/instrumentation MH - Antiphospholipid Syndrome/blood/*complications/diagnosis MH - Aorta, Abdominal/diagnostic imaging/*surgery MH - Aortic Diseases/diagnostic imaging/etiology/*surgery MH - Aortography/methods MH - Arterial Occlusive Diseases/diagnostic imaging/etiology/*surgery MH - Computed Tomography Angiography MH - Constriction, Pathologic MH - Humans MH - Male MH - Middle Aged MH - Stents MH - Thrombosis/diagnostic imaging/etiology/*surgery MH - Treatment Outcome MH - Ultrasonography, Interventional OTO - NOTNLM OT - case reports OT - endovascular treatment OT - peripheral artery disease OT - thrombosis EDAT- 2018/08/11 06:00 MHDA- 2019/01/29 06:00 CRDT- 2018/08/11 06:00 PHST- 2018/08/11 06:00 [pubmed] PHST- 2019/01/29 06:00 [medline] PHST- 2018/08/11 06:00 [entrez] AID - 10.1177/1538574418791355 [doi] PST - ppublish SO - Vasc Endovascular Surg. 2019 Jan;53(1):51-57. doi: 10.1177/1538574418791355. Epub 2018 Aug 9. PMID- 29269694 OWN - NLM STAT- MEDLINE DCOM- 20180227 LR - 20180227 IS - 1882-0654 (Electronic) IS - 0009-918X (Linking) VI - 58 IP - 1 DP - 2018 Jan 26 TI - [An anti-RNP antibody-positive case of aseptic meningitis induced by non-steroidal anti-inflammatory drugs in a young woman]. PG - 25-29 LID - 10.5692/clinicalneurol.cn-001085 [doi] AB - A 19-year-old woman developed high fever, headache, and nausea after taking Loxoprofen for pharyngitis, followed by disturbed consciousness and nuchal stiffness. The patient and her mother had a history of Raynaud's phenomenon. Cerebrospinal fluid (CSF) examination indicated a diagnosis of aseptic meningitis and revealed high levels of Q albumin and IgG index. Anti-RNP antibodies were positive in serum and CSF. Her symptoms disappeared immediately after cessation of Loxoprofen and a drug lymphocyte stimulation test was negative, confirming a diagnosis of non-steroidal anti-inflammatory drugs (NSAIDs)-induced aseptic meningitis. It should be kept in mind that an immune abnormality such as serum and CSF anti-RNP antibodies may play a role in development of NSAIDs-induced aseptic meningitis. A history of usage of NSAIDs and a thorough examination of collagen diseases are useful for identification of the origin of aseptic meningitis in a young woman. FAU - Matsui, Taro AU - Matsui T AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. FAU - Nakagawa, Keiichi AU - Nakagawa K AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. FAU - Yamazaki, Keishi AU - Yamazaki K AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. FAU - Wada, Taishi AU - Wada T AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. FAU - Kadoya, Masato AU - Kadoya M AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. FAU - Kaida, Kenichi AU - Kaida K AD - Department of Neurology, Anti-aging and Vascular Medicine, National Defense Medical College. LA - jpn PT - Case Reports PT - Journal Article DEP - 20171222 PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Phenylpropionates) RN - 0 (Ribonucleoproteins) RN - 3583H0GZAP (loxoprofen) RN - 362O9ITL9D (Acetaminophen) SB - IM MH - Acetaminophen/administration & dosage MH - Adult MH - Anti-Inflammatory Agents, Non-Steroidal/administration & dosage/*adverse effects MH - Antibodies, Antinuclear/*blood/*cerebrospinal fluid MH - Autoimmune Diseases/complications/diagnosis MH - Autoimmunity MH - Biomarkers/blood/cerebrospinal fluid MH - Diagnosis, Differential MH - Drug Substitution MH - Female MH - Humans MH - Meningitis, Aseptic/*diagnosis/*etiology MH - Phenylpropionates/*adverse effects MH - Ribonucleoproteins/*immunology MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - Loxoprofen OT - anti-RNP antibody OT - aseptic meningitis OT - collagen disease OT - non-steroidal anti-inflammatory drugs (NSAIDs) EDAT- 2017/12/23 06:00 MHDA- 2018/02/28 06:00 CRDT- 2017/12/23 06:00 PHST- 2017/12/23 06:00 [pubmed] PHST- 2018/02/28 06:00 [medline] PHST- 2017/12/23 06:00 [entrez] AID - 10.5692/clinicalneurol.cn-001085 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2018 Jan 26;58(1):25-29. doi: 10.5692/clinicalneurol.cn-001085. Epub 2017 Dec 22. PMID- 33447331 OWN - NLM STAT- MEDLINE DCOM- 20210127 LR - 20210127 IS - 1937-8688 (Electronic) VI - 37 DP - 2020 TI - Systemic sclerosis in sub-Saharan Africa: a systematic review. PG - 176 LID - 10.11604/pamj.2020.37.176.22557 [doi] LID - 176 AB - Systematic studies on connective tissue disorders are scarce in sub-Saharan Africa. Our aim was to analyse the published clinical data on systemic sclerosis (SSc) in sub-Saharan Africa. A systematic review was carried out in accordance with the PRISMA guidelines. We screened the Embase, PubMed and African Health Sciences databases for literature published until March 2018. Searches produced 1210 publications. After abstract and full-text screenings, 91 publications were analysed, and epidemiological information and clinical features extracted. Publications were mostly publications case reports (36%), cross-sectional studies (26%) and case series (23%) and came predominantly from South Africa (45%), Nigeria (15%) and Senegal (14%). A total of 1884 patients were reported, 66% of patients came from South Africa. The patients were between 4 and 77 years old; 83% of patients were female. Overall, 72% had diffuse SSc. Raynaud´s phenomenon was reported in 78% and skin ulcerations in 42% of patients. Focal skin hypopigmentation was common and telangiectasia not frequent. Interstitial lung involvement was reported in 50%, pulmonary hypertension in 30%, heart involvement in 28% of patients. Oesophageal reflux was observed in 70% and dysphagia in 37% of patients. Antinuclear antibodies were positive in 65% of patients. Anti-centromere autoantibodies (9.2%) and RNA polymerase 3 antibodies (7.1%) were rare and anti-fibrillarin most frequent (16.5%). SSc presentations in sub-Saharan Africa differ from those reported in Europe and America by a frequent diffuse skin involvement, focal skin hypopigmentation and a high prevalence of anti-fibrillarin autoantibodies. CI - Copyright: Julian Nicolas Erzer et al. FAU - Erzer, Julian Nicolas AU - Erzer JN AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. FAU - Jaeger, Veronika Katharina AU - Jaeger VK AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. AD - Institute of Epidemiology and Social Medicine, University of Münster, Münster, Germany. FAU - Tikly, Mohammed AU - Tikly M AD - Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa. FAU - Walker, Ulrich Andreas AU - Walker UA AD - Department of Rheumatology, University Hospital Basel, Basel, Switzerland. LA - eng PT - Journal Article PT - Systematic Review DEP - 20201022 PL - Uganda TA - Pan Afr Med J JT - The Pan African medical journal JID - 101517926 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Africa South of the Sahara/epidemiology MH - Aged MH - Antibodies, Antinuclear/*immunology MH - Autoantibodies/*immunology MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Scleroderma, Systemic/*epidemiology/immunology/physiopathology MH - Young Adult PMC - PMC7778190 OTO - NOTNLM OT - Systemic sclerosis OT - connective tissue disease OT - sub-Saharan Africa OT - systematic review COIS- The authors declare no competing interests. EDAT- 2021/01/16 06:00 MHDA- 2021/01/28 06:00 PMCR- 2020/10/22 CRDT- 2021/01/15 06:00 PHST- 2020/03/29 00:00 [received] PHST- 2020/04/04 00:00 [accepted] PHST- 2021/01/15 06:00 [entrez] PHST- 2021/01/16 06:00 [pubmed] PHST- 2021/01/28 06:00 [medline] PHST- 2020/10/22 00:00 [pmc-release] AID - PAMJ-37-176 [pii] AID - 10.11604/pamj.2020.37.176.22557 [doi] PST - epublish SO - Pan Afr Med J. 2020 Oct 22;37:176. doi: 10.11604/pamj.2020.37.176.22557. eCollection 2020. PMID- 34595860 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211008 IS - 1738-6586 (Print) IS - 2005-5013 (Electronic) IS - 1738-6586 (Linking) VI - 17 IP - 4 DP - 2021 Oct TI - Incidence and Reappraisal of Known Risk Factors Associated with Carpal Tunnel Syndrome: A Nationwide, 11-Year, Population-Based Study in South Korea. PG - 524-533 LID - 10.3988/jcn.2021.17.4.524 [doi] AB - BACKGROUND AND PURPOSE: Previous studies have revealed various risk factors for carpal tunnel syndrome (CTS), but few large-scale studies have been conducted. We used data from the 11-year, longitudinal, nationwide population-based National Health Insurance Service-National Health Screening cohort to identify the actual risk factors for CTS. METHODS: We collected patients with CTS newly diagnosed using electrodiagnostic studies while excluding radiculopathy, plexopathy, or polyneuropathy, which can be confused with CTS. The crude and standardized incidence rates of CTS were calculated. Univariate and multivariate Cox analyses and the incidence of CTS were used to identify the risk factors for newly diagnosed CTS. RESULTS: The standardized incidence was 130.8/100,000 person-years based on the World Health Organization World Standard Population as a reference. Multivariate Cox analysis identified that the risk factors for CTS were being middle-aged, female, and obese, and having rheumatoid arthritis and Raynaud's syndrome, whereas gout and hypothyroidism were not risk factors. Diabetes and end-stage renal disease did not show a significant hazard ratio, although it is implicit that the durations of these diseases affect the development of CTS. CONCLUSIONS: This study calculated the incidence of CTS and reappraised the associated risk factors found in previous studies. This information will be helpful for determining the pathophysiology of CTS, and hence aid the establishment of effective new public health policies. CI - Copyright © 2021 Korean Neurological Association. FAU - Rhee, Seung Yeon AU - Rhee SY AUID- ORCID: 0000-0001-9717-3650 AD - Department of Physical Medicine and Rehabilitation, National Health Insurance Service Ilsan Hospital, Goyang, Korea. FAU - Cho, Han Eol AU - Cho HE AUID- ORCID: 0000-0001-5625-3013 AD - Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea. FAU - Kim, Jong Hun AU - Kim JH AUID- ORCID: 0000-0002-2594-1048 AD - Department of Neurology, National Health Insurance Service Ilsan Hospital, Goyang, Korea. jh7521@naver.com. FAU - Kim, Hyoung Seop AU - Kim HS AUID- ORCID: 0000-0002-5310-4802 AD - Department of Physical Medicine and Rehabilitation, National Health Insurance Service Ilsan Hospital, Goyang, Korea. rehappydoc@gmail.com. LA - eng GR - National Health Insurance Service Ilsan Hospital/Korea PT - Journal Article PL - Korea (South) TA - J Clin Neurol JT - Journal of clinical neurology (Seoul, Korea) JID - 101252374 PMC - PMC8490900 OTO - NOTNLM OT - big data OT - carpal tunnel syndrome OT - cohort studies OT - epidemiology OT - risk factors COIS- The authors have no potential conflicts of interest to disclose. EDAT- 2021/10/02 06:00 MHDA- 2021/10/02 06:01 PMCR- 2021/10/01 CRDT- 2021/10/01 07:57 PHST- 2021/03/15 00:00 [received] PHST- 2021/05/12 00:00 [revised] PHST- 2021/05/12 00:00 [accepted] PHST- 2021/10/01 07:57 [entrez] PHST- 2021/10/02 06:00 [pubmed] PHST- 2021/10/02 06:01 [medline] PHST- 2021/10/01 00:00 [pmc-release] AID - 17.524 [pii] AID - 10.3988/jcn.2021.17.4.524 [doi] PST - ppublish SO - J Clin Neurol. 2021 Oct;17(4):524-533. doi: 10.3988/jcn.2021.17.4.524. PMID- 36653758 OWN - NLM STAT- MEDLINE DCOM- 20230120 LR - 20230122 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 23 IP - 1 DP - 2023 Jan 18 TI - Pulmonary veno-occlusive disease in Sjogren's syndrome: a case report. PG - 26 LID - 10.1186/s12890-023-02322-w [doi] LID - 26 AB - BACKGROUND: Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) belongs to Group 1 pulmonary hypertension. Pulmonary veno-occlusive disease (PVOD), which is characterized by venous system aberrations, has been previously reported in CTD-PAH; however, it has rarely been observed in Sjogren's syndrome (SS). CASE PRESENTATION: Our 28-year-old female patient was admitted to the hospital with recurrent shortness of breath even after minimal physical activity. Her chest high-resolution CT scan demonstrated pulmonary artery dilatation and bilateral ground-glass nodules. A subsequent right heart catheterization confirmed pulmonary hypertension because her mean pulmonary arterial pressure was 62 mmHg. Our inquisitive genomic assessment identified a novel EIF2AK4 mutation at c.1021 C > T (p. Gln341*), the dominant causal gene of PVOD. Histological examination demonstrated stenosis and occlusions in the pulmonary veins. Because she presented with features such as dry eyes and Raynaud's phenomenon, we performed a biopsy on the labial salivary gland, which confirmed SS. Her treatment regimen included PAH-targeted therapies (tadalafil and macitentan) in combination with hydroxychloroquine. Although she was hospitalized several times due to acute exacerbation of PAH, her disease progression was under control, and she did not demonstrate any signs of pulmonary edema even after a three-year treatment period. CONCLUSION: Here, we report the case of an SS-PAH patient with PVOD who carried a novel biallelic EIF2AK4 mutation, and PAH-targeted therapies were well tolerated by our patient. CI - © 2023. The Author(s). FAU - Zeng, Xiaofang AU - Zeng X AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. AD - National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan, China. FAU - Liu, Qiong AU - Liu Q AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. FAU - Rathinasabapathy, Anandharajan AU - Rathinasabapathy A AD - Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Zha, Lihuang AU - Zha L AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. FAU - Liu, Dongliang AU - Liu D AD - Department of Emergency Medicine, Xiangya Hospital, Central South University, Changsha, Hunan, China. FAU - Tang, Yiyang AU - Tang Y AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. AD - National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan, China. FAU - Sun, Jing AU - Sun J AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. AD - National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan, China. FAU - Luo, Hui AU - Luo H AD - Department of Cardiology, The First Hospital of Changsha, Changsha, Hunan, China. FAU - Yu, Zaixin AU - Yu Z AD - Department of Cardiology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China. yuzaixin@csu.edu.cn. AD - National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan, China. yuzaixin@csu.edu.cn. LA - eng GR - 81873416/National Natural Science Foundation of China/ GR - 82070055/National Natural Science Foundation of China/ GR - 8210012237/National Natural Science Foundation of China/ GR - 2020SK2065/Key Research and development program of Hunan Province/ GR - 1053320191678/Fundamental Research Funds for the Central Universities of Central South University/ GR - 2020JJ4634/Natural Science Foundation project of Hunan province/ PT - Case Reports PT - Journal Article DEP - 20230118 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 RN - EC 2.7.11.1 (EIF2AK4 protein, human) RN - EC 2.7.11.1 (Protein Serine-Threonine Kinases) SB - IM MH - Humans MH - Female MH - Adult MH - *Hypertension, Pulmonary MH - *Pulmonary Veno-Occlusive Disease/complications/diagnosis/genetics MH - *Sjogren's Syndrome/complications/genetics MH - Lung MH - *Pulmonary Arterial Hypertension MH - Familial Primary Pulmonary Hypertension MH - Protein Serine-Threonine Kinases/genetics PMC - PMC9847112 OTO - NOTNLM OT - EIF2AK4 OT - Sjogren’s syndrome OT - pulmonary arterial hypertension OT - pulmonary veno-occlusive disease COIS- The authors declare that they have no competing interests. EDAT- 2023/01/19 06:00 MHDA- 2023/01/21 06:00 PMCR- 2023/01/18 CRDT- 2023/01/18 23:34 PHST- 2022/05/10 00:00 [received] PHST- 2023/01/10 00:00 [accepted] PHST- 2023/01/18 23:34 [entrez] PHST- 2023/01/19 06:00 [pubmed] PHST- 2023/01/21 06:00 [medline] PHST- 2023/01/18 00:00 [pmc-release] AID - 10.1186/s12890-023-02322-w [pii] AID - 2322 [pii] AID - 10.1186/s12890-023-02322-w [doi] PST - epublish SO - BMC Pulm Med. 2023 Jan 18;23(1):26. doi: 10.1186/s12890-023-02322-w. PMID- 24618222 OWN - NLM STAT- MEDLINE DCOM- 20160422 LR - 20221207 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 18 IP - 4 DP - 2015 May TI - Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort. PG - 459-65 LID - 10.1111/1756-185X.12322 [doi] AB - AIM: To evaluate the clinical and antibody profile of systemic sclerosis (SSc) in a Malaysian cohort. METHODS: Consecutive patients with SSc in University Malaya Medical Centre from March to November 2012 were included in this study. In addition to clinical characterization, all subjects underwent autoantibody testing using Euroline immunoblot assay. The association between clinical features and autoantibody profile was evaluated. RESULTS: There were 31, predominantly Chinese (45.2%), subjects. Limited cutaneous disease was the most common subtype (71%). Raynaud's phenomenon was the most commonly observed feature (83.9%). Nine (29%) had esophageal dysmotility symptoms and 23 (74.2%), including all patients with diffuse SSc, had symptoms of gastro-esophageal reflux disease (GERD). Restrictive pattern on pulmonary function test and evidence of lung fibrosis were seen in more than 70% of patients. Echocardiographic evidence of pulmonary arterial hypertension was seen in 58.1%. Telangiectasia, calcinosis, digital ulcers, digital pulp loss or pitting were seen more commonly in the diffuse subtype. The two most prevalent autoantibodies were anti-Scl-70 and anti-Ro-52. The presence of anti-Scl-70 was significantly associated with restrictive lung disease (P = 0.05). Anti-Ro-52 was associated with control subjects with other autoimmune diseases (P = 0.043). The presence of anti-PM-Scl-75 was associated with overlap syndrome (P = 0.032). Patients with anticentromere antibodies were more likely to have vasculitic rash (P = 0.012). CONCLUSION: In Malaysia, SSc most commonly affects the Chinese. Limited cutaneous is more common than diffuse subtype. Features of CREST (calcinosis, Reynaud disease, esophageal dysmotility, sclerodactyly, telangiectasia) are more commonly observed in the diffuse cutaneous subgroup. Anti-Scl-70 and anti-Ro-52 antibodies are promising biomarkers for pulmonary involvement in SSc. CI - © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd. FAU - Sujau, Ibrahim AU - Sujau I AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. FAU - Ng, Chin Teck AU - Ng CT AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. FAU - Sthaneshwar, Pavai AU - Sthaneshwar P AD - Division of Laboratory Medicine, Department of Pathology, University of Malaya, Kuala Lumpur, Malaysia. FAU - Sockalingam, Sargunan AU - Sockalingam S AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. FAU - Cheah, Tien Eang AU - Cheah TE AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. FAU - Yahya, Fariz AU - Yahya F AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. FAU - Jasmin, Raja AU - Jasmin R AD - Division of Rheumatology, Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140223 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Autoantibodies) SB - IM MH - Academic Medical Centers MH - Adult MH - Age Factors MH - Asian People/statistics & numerical data MH - Autoantibodies/*immunology MH - Case-Control Studies MH - Female MH - Hospitals, Teaching MH - Humans MH - Malaysia MH - Male MH - Middle Aged MH - Prospective Studies MH - Rare Diseases MH - Risk Assessment MH - Scleroderma, Systemic/ethnology/*immunology/*physiopathology MH - Severity of Illness Index MH - Sex Factors MH - Statistics, Nonparametric OTO - NOTNLM OT - Malaysia OT - autoantibodies OT - clinical profile OT - systemic sclerosis EDAT- 2014/03/13 06:00 MHDA- 2016/04/23 06:00 CRDT- 2014/03/13 06:00 PHST- 2014/03/13 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2016/04/23 06:00 [medline] AID - 10.1111/1756-185X.12322 [doi] PST - ppublish SO - Int J Rheum Dis. 2015 May;18(4):459-65. doi: 10.1111/1756-185X.12322. Epub 2014 Feb 23. PMID- 33039312 OWN - NLM STAT- Publisher LR - 20240222 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) DP - 2020 Oct 7 TI - Characterization of Venezuelan Patients With Systemic Sclerosis: A Study in a Tertiary Hospital in Caracas. LID - S1699-258X(20)30159-5 [pii] LID - 10.1016/j.reuma.2020.06.006 [doi] AB - OBJECTIVE: The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. METHODS: Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. RESULTS: Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1±13.7 (mean±SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4±11.7 (mean±SD) years, 16.74±12.99 years for limited SSc and 7.52±5.25 years for diffuse SSc (p=0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p=0.0211 and p=0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p=0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p=0.0443) and with calcinosis (p=0.0020). Anti-topoisomerase antibodies were associated with ILD (p=0.0077). CONCLUSIONS: Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement. CI - Copyright © 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved. FAU - Rivas-Vargas, Daniel AU - Rivas-Vargas D AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Caracas, Venezuela. Electronic address: rivasvargas4@gmail.com. FAU - Snih, Soham Al AU - Snih SA AD - Division of Rehabilitation Sciences/School of Health Professions, Division of Geriatrics/Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, United States. FAU - Rodríguez, Martín A AU - Rodríguez MA AD - Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Caracas, Venezuela; Sealy Center on Aging, University of Texas Medical Branch, Galveston, TX, United States. LA - eng LA - spa PT - Journal Article DEP - 20201007 PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 SB - IM OTO - NOTNLM OT - Demografía OT - Demography OT - Epidemiology OT - Epidemiología OT - Esclerosis sistémica OT - Hispanic OT - Hispanos OT - Systemic sclerosis EDAT- 2020/10/12 06:00 MHDA- 2020/10/12 06:00 CRDT- 2020/10/11 20:22 PHST- 2019/09/12 00:00 [received] PHST- 2020/04/16 00:00 [revised] PHST- 2020/06/24 00:00 [accepted] PHST- 2020/10/11 20:22 [entrez] PHST- 2020/10/12 06:00 [pubmed] PHST- 2020/10/12 06:00 [medline] AID - S1699-258X(20)30159-5 [pii] AID - 10.1016/j.reuma.2020.06.006 [doi] PST - aheadofprint SO - Reumatol Clin (Engl Ed). 2020 Oct 7:S1699-258X(20)30159-5. doi: 10.1016/j.reuma.2020.06.006. PMID- 19684145 OWN - NLM STAT- MEDLINE DCOM- 20091113 LR - 20151119 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 9 DP - 2009 Sep TI - Sensorineural hearing loss in patients with mixed connective tissue disease: immunological markers and cytokine levels. PG - 1930-6 LID - 10.3899/jrheum.081314 [doi] AB - OBJECTIVE: To investigate the frequency of sensorineural hearing loss (SNHL) in patients with mixed connective tissue disease (MCTD). METHODS: The study population consisted of 71 patients with MCTD (69 female; 2 male), with a mean age of 57.1 +/- 7.9 years and a mean disease duration of 14.5 +/- 8.0 years. All patients underwent audiological evaluation that included pure tone and speech audiometry. In addition, the systemic manifestations of the disease and drug therapy were recorded. All patients were tested for presence of autoantibodies. Fifty-one age-matched healthy subjects served as controls. RESULTS: SNHL was found in 33 (46.4%) of the 71 patients with MCTD. There was no correlation between SNHL and age and disease duration. An association was found between Raynaud's phenomenon (p < 0.03), secondary antiphospholipid syndrome (APS) (p < 0.05), and SNHL. MCTD patients with SNHL had higher serum levels of anti-U1RNP (p < 0.05), antiendothelial cell antibodies (p < 0.001), and IgG type anticardiolipin antibodies (p < 0.0001) than patients without SNHL. Serum levels of interferon-gamma and tumor necrosis factor-alpha were increased in MCTD patients with SNHL compared to patients without SNHL. The absolute number of natural (CD4+CD25(high)FoxP+) regulatory T cells (Treg) was lower compared to patients without SNHL. CONCLUSION: In MCTD, SNHL is a specific organ manifestation and appears frequently. We have found that pathogenic autoantibodies, decreased levels of regulatory T cells, and overexpression of proinflammatory cytokines may play a role in the pathogenesis of immune mediated inner ear disorders in MCTD. FAU - Hajas, Agota AU - Hajas A AD - Division of Clinical Immunology, 3rd Department of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. FAU - Szodoray, Peter AU - Szodoray P FAU - Barath, Sandor AU - Barath S FAU - Sipka, Sandor AU - Sipka S FAU - Rezes, Szilard AU - Rezes S FAU - Zeher, Margit AU - Zeher M FAU - Sziklai, Istvan AU - Sziklai I FAU - Szegedi, Gyula AU - Szegedi G FAU - Bodolay, Edit AU - Bodolay E LA - eng PT - Journal Article DEP - 20090814 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antibodies, Anti-Idiotypic) RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Cytokines) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 0 (Tumor Necrosis Factor-alpha) RN - 0 (anti-endothelial cell antibody) RN - 82115-62-6 (Interferon-gamma) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Anti-Idiotypic/blood MH - Antibodies, Anticardiolipin/blood MH - Auditory Pathways/physiopathology MH - Autoantibodies/blood MH - Biomarkers/blood MH - Case-Control Studies MH - Cytokines/*blood MH - Female MH - Hearing Loss, Sensorineural/blood/*epidemiology/*immunology MH - Humans MH - Incidence MH - Interferon-gamma/blood MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/blood/*complications/*immunology MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - T-Lymphocytes, Regulatory/pathology MH - Tumor Necrosis Factor-alpha/blood EDAT- 2009/08/18 09:00 MHDA- 2009/11/17 06:00 CRDT- 2009/08/18 09:00 PHST- 2009/08/18 09:00 [entrez] PHST- 2009/08/18 09:00 [pubmed] PHST- 2009/11/17 06:00 [medline] AID - jrheum.081314 [pii] AID - 10.3899/jrheum.081314 [doi] PST - ppublish SO - J Rheumatol. 2009 Sep;36(9):1930-6. doi: 10.3899/jrheum.081314. Epub 2009 Aug 14. PMID- 22859355 OWN - NLM STAT- MEDLINE DCOM- 20130305 LR - 20161125 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 39 IP - 9 DP - 2012 Sep TI - Antisynthetase syndrome with anti-Jo1 antibodies in 48 patients: pulmonary involvement predicts disease-modifying antirheumatic drug use. PG - 1835-9 LID - 10.3899/jrheum.111604 [doi] AB - OBJECTIVE: To analyze the characteristics, outcomes, and predictive factors of disease-modifying antirheumatic drug (DMARD) use in 48 patients with antisynthetase syndrome [characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon (RP), and/or mechanic's hands] and the presence of anti-histidyl-transfer RNA synthetase (anti-Jo1) autoantibodies. METHODS: Forty-eight patients (33 women, 15 men) who were anti-Jo1-positive referred to one center between 1998 and 2008 were analyzed retrospectively. RESULTS: The median age of disease onset was 43 years [interquartile range (IQR) 33-53 yrs]. The median followup was 5 years (IQR 2-8 yrs). At diagnosis, 81% of patients presented with myositis, 80% ILD, 77% arthralgia, 48% RP, and 21% mechanic's hands. During the followup, 14 patients (29%) had no need for DMARD, while 34 (71%) required DMARD. Patients with mechanic's hands (p=0.02) and higher creatine phosphokinase at diagnosis (median 6070 IU/l vs 1121 IU/l; p=0.002) were more likely to need DMARD. ILD, noted on computed tomography scan by a nonspecific interstitial pneumonia score, was lower in the group of patients with no DMARD need (4 vs 7; p=0.04). Twenty patients (44%) presented with a pulmonary aggravation (worsening of radiologic score of ILD and/or pulmonary function test results) leading to DMARD use. Nonspecific interstitial pneumonia score (7 vs 5; p=0.05) and total lung volume (57.5% vs 70%; p=0.02) values predicted pulmonary aggravation. CONCLUSION: Our study outlines the burden of chest involvement for the prognosis of antisynthetase syndrome in terms of patients' requirement for DMARD therapy. FAU - Stanciu, Raluca AU - Stanciu R AD - Department of Internal Medicine, Institute of Myology, Université Pierre et Marie Curie, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 1, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75651 Paris Cedex 13, France. FAU - Guiguet, Marguerite AU - Guiguet M FAU - Musset, Lucile AU - Musset L FAU - Touitou, Diane AU - Touitou D FAU - Beigelman, Catherine AU - Beigelman C FAU - Rigolet, Aude AU - Rigolet A FAU - Costedoat-Chalumeau, Nathalie AU - Costedoat-Chalumeau N FAU - Allenbach, Yves AU - Allenbach Y FAU - Hervier, Baptiste AU - Hervier B FAU - Dubourg, Odile AU - Dubourg O FAU - Maisonobe, Thierry AU - Maisonobe T FAU - Charuel, Jean-Luc AU - Charuel JL FAU - Behin, Anthony AU - Behin A FAU - Herson, Serge AU - Herson S FAU - Amoura, Zahir AU - Amoura Z FAU - Grenier, Philippe AU - Grenier P FAU - Benveniste, Olivier AU - Benveniste O LA - eng PT - Journal Article DEP - 20120801 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antirheumatic Agents) RN - 0 (Autoantibodies) RN - 0 (Jo-1 antibody) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Antibodies, Antinuclear/*immunology MH - Antirheumatic Agents/*therapeutic use MH - Arthritis/diagnostic imaging/drug therapy/immunology MH - Autoantibodies MH - Female MH - Humans MH - Lung/*diagnostic imaging MH - Lung Diseases, Interstitial/*diagnostic imaging/immunology MH - Male MH - Middle Aged MH - Myositis/*diagnostic imaging/drug therapy/immunology MH - Radiography MH - Respiratory Function Tests MH - Retrospective Studies EDAT- 2012/08/04 06:00 MHDA- 2013/03/06 06:00 CRDT- 2012/08/04 06:00 PHST- 2012/08/04 06:00 [entrez] PHST- 2012/08/04 06:00 [pubmed] PHST- 2013/03/06 06:00 [medline] AID - jrheum.111604 [pii] AID - 10.3899/jrheum.111604 [doi] PST - ppublish SO - J Rheumatol. 2012 Sep;39(9):1835-9. doi: 10.3899/jrheum.111604. Epub 2012 Aug 1. PMID- 22238253 OWN - NLM STAT- MEDLINE DCOM- 20120517 LR - 20250529 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 64 IP - 5 DP - 2012 May TI - Inactive disease and remission in childhood-onset systemic lupus erythematosus. PG - 683-93 LID - 10.1002/acr.21612 [doi] AB - OBJECTIVE: To define inactive disease (ID) and clinical remission (CR) and to delineate variables that can be used to measure ID/CR in childhood-onset systemic lupus erythematosus (cSLE). METHODS: Delphi questionnaires were sent to an international group of pediatric rheumatologists. Respondents provided information about variables to be used in future algorithms to measure ID/CR. The usefulness of these variables was assessed in 35 children with ID and 31 children with minimally active lupus (MAL). RESULTS: While ID reflects cSLE status at a specific point in time, CR requires the presence of ID for >6 months and considers treatment. There was consensus that patients in ID/CR can have <2 mild nonlimiting symptoms (i.e., fatigue, arthralgia, headaches, or myalgia) but not Raynaud's phenomenon, chest pain, or objective physical signs of cSLE; antinuclear antibody positivity and erythrocyte sedimentation rate elevation can be present. Complete blood count, renal function testing, and complement C3 all must be within the normal range. Based on consensus, only damage-related laboratory or clinical findings of cSLE are permissible with ID. The above parameters were suitable to differentiate children with ID/CR from those with MAL (area under the receiver operating characteristic curve >0.85). Disease activity scores with or without the physician global assessment of disease activity and patient symptoms were well suited to differentiate children with ID from those with MAL. CONCLUSION: Consensus has been reached on common definitions of ID/CR with cSLE and relevant patient characteristics with ID/CR. Further studies must assess the usefulness of the data-driven candidate criteria for ID in cSLE. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Mina, Rina AU - Mina R AD - Cincinnati Children's Hospital Medical Center, University of Cincinnati, William S. Rowe Division of Rheumatology, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA. FAU - Klein-Gitelman, Marisa S AU - Klein-Gitelman MS FAU - Ravelli, Angelo AU - Ravelli A FAU - Beresford, Michael W AU - Beresford MW FAU - Avcin, Tadej AU - Avcin T FAU - Espada, Graciela AU - Espada G FAU - Eberhard, B Anne AU - Eberhard BA FAU - Schanberg, Laura E AU - Schanberg LE FAU - O'Neil, Kathleen M AU - O'Neil KM FAU - Silva, Clovis A AU - Silva CA FAU - Higgins, Gloria C AU - Higgins GC FAU - Onel, Karen AU - Onel K FAU - Singer, Nora G AU - Singer NG FAU - von Scheven, Emily AU - von Scheven E FAU - Imundo, Lisa F AU - Imundo LF FAU - Nelson, Shannen AU - Nelson S FAU - Giannini, Edward H AU - Giannini EH FAU - Brunner, Hermine I AU - Brunner HI LA - eng GR - P60-AR047884/AR/NIAMS NIH HHS/United States GR - U01 AR051868/AR/NIAMS NIH HHS/United States GR - T32100291/PHS HHS/United States GR - U01 AR059509/AR/NIAMS NIH HHS/United States GR - P60 AR047784/AR/NIAMS NIH HHS/United States GR - P30 AR047363/AR/NIAMS NIH HHS/United States GR - P30-AR AR47363/AR/NIAMS NIH HHS/United States GR - R01 AR051868/AR/NIAMS NIH HHS/United States GR - U01 AR055054/AR/NIAMS NIH HHS/United States GR - U01-AR51868/AR/NIAMS NIH HHS/United States GR - UL1 RR026314/RR/NCRR NIH HHS/United States GR - UL1RR026314/RR/NCRR NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adolescent MH - Age Factors MH - Child MH - Female MH - Health Surveys/methods MH - Humans MH - Lupus Erythematosus, Systemic/*pathology/*therapy MH - Male MH - Remission Induction/methods MH - *Severity of Illness Index PMC - PMC3336032 MID - NIHMS349088 EDAT- 2012/01/13 06:00 MHDA- 2012/05/18 06:00 PMCR- 2013/05/01 CRDT- 2012/01/13 06:00 PHST- 2012/01/13 06:00 [entrez] PHST- 2012/01/13 06:00 [pubmed] PHST- 2012/05/18 06:00 [medline] PHST- 2013/05/01 00:00 [pmc-release] AID - 10.1002/acr.21612 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 May;64(5):683-93. doi: 10.1002/acr.21612. PMID- 16650177 OWN - NLM STAT- MEDLINE DCOM- 20061020 LR - 20061030 IS - 0011-9059 (Print) IS - 0011-9059 (Linking) VI - 45 IP - 4 DP - 2006 Apr TI - Is Kikuchi-Fujimoto disease a manifestation of systemic lupus erythematosus? PG - 454-6 AB - A 32-year-old woman presented with fever and swelling in the axillae of 2 months' duration, and erythema of the face, fluid-filled lesions on the trunk, oral ulcers, crusting of the lips, and redness and watering of the eyes for 3 days. The patient was initially diagnosed with tuberculous lymphadenitis and was given antituberculous treatment. One month later, she developed the present complaints (see below). The patient was photosensitive. There was no joint pain or Raynaud's phenomenon. She had experienced three intrauterine deaths in the past with no live births. On examination, the patient was pale. Bilateral axillary lymphadenopathy was present. The lymph nodes were mobile, non-tender, and not matted. Mucocutaneous examination revealed a malar eruption, flaccid bullae on the back (Fig. 1), crusting of the lips (Fig. 2), oral ulcers, and redness and discharge from the eyes. On investigation, immunoglobulin G (IgG), IgM, and IgA for tuberculosis were negative. There was anemia and leukopenia, the erythrocyte sedimentation rate (ESR) was raised, albumin in urine was positive, enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was negative, and venereal disease research laboratory (VDRL) test was nonreactive. Antinuclear antibody (ANA), dsDNA, and antiphospholipid antibody were positive. Fine needle aspiration cytology (FNAC) of the axillary lymph node showed loss of nodal architecture. The extensive infiltrate consisted of lymphocytes, histiocytes, immunoblasts, and necrosis of cortical and paracortical areas. There was histiocytic necrotizing lymphadenitis without granulocytic infiltration. These features were suggestive of Kikuchi-Fujimoto's disease (Fig. 3). Skin biopsy showed epidermal atrophy, basal cell vacuolation, focal hyperkeratosis, pilosebaceous atrophy, and follicular plugging. The dermis showed edema and a lymphocytic infiltrate in the upper dermis and around the blood vessels. These features were suggestive of systemic lupus erythematosus (SLE). Direct immunofluorescence of lesional skin showed a strong continuous basement membrane zone (BMZ) band of C3 and fibrinogen and a strong discontinuous granular BMZ band of IgG. IgA was negative. Covered skin showed moderate and strong positivity for IgM and IgG, respectively. C3, IgA, and fibrinogen were negative. These findings were suggestive of SLE (Fig. 4). Based on the clinical findings and investigations, a diagnosis of Kikuchi-Fujimoto's disease with SLE was made. FAU - Rao, Gatha S AU - Rao GS AD - Department of Skin and STD, Kasturba Medical College Hospital, Attavar, Mangalore, Karnataka, India. drupady@hotmail.com FAU - Vohra, Deepak AU - Vohra D FAU - Kuruvilla, Maria AU - Kuruvilla M LA - eng PT - Case Reports PT - Journal Article PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Complement C3) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) RN - 9007-49-2 (DNA) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Antibodies, Antiphospholipid/blood MH - Biopsy, Fine-Needle MH - Complement C3/analysis MH - DNA/immunology MH - Female MH - Histiocytic Necrotizing Lymphadenitis/*diagnosis MH - Humans MH - Immunoglobulin G/analysis MH - Immunoglobulin M/analysis MH - Lupus Erythematosus, Systemic/*diagnosis MH - Lymph Nodes/pathology MH - Skin/pathology EDAT- 2006/05/03 09:00 MHDA- 2006/10/21 09:00 CRDT- 2006/05/03 09:00 PHST- 2006/05/03 09:00 [pubmed] PHST- 2006/10/21 09:00 [medline] PHST- 2006/05/03 09:00 [entrez] AID - IJD2379 [pii] AID - 10.1111/j.1365-4632.2004.02379.x [doi] PST - ppublish SO - Int J Dermatol. 2006 Apr;45(4):454-6. doi: 10.1111/j.1365-4632.2004.02379.x. PMID- 38101787 OWN - NLM STAT- MEDLINE DCOM- 20231218 LR - 20231219 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 55 IP - 6 DP - 2023 Dec 18 TI - [Analysis of pregnancy outcomes, disease progression, and risk factors in patients with undifferentiated connective tissue disease]. PG - 1045-1052 AB - OBJECTIVE: To investigate the fetal and maternal outcomes, risk factors of disease progression and adverse pregnancy outcomes (APOs) in patients with undifferentiated connective tissue disease (UCTD). METHODS: This retrospective study described the outcomes of 106 pregnancies in patients with UCTD. The patients were divided into APOs group (n=53) and non-APOs group (n=53). The APOs were defined as miscarriage, premature birth, pre-eclampsia, premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), postpartum hemorrhage (PPH), and stillbirth, small for gestational age infant (SGA), low birth weight infant (LBW) and birth defects. The differences in clinical manifestations, laboratory data and pregnancy outcomes between the two groups were compared. Logistic regression analysis was performed to analyze the risk factors for APOs and the progression of UCTD to definitive CTD. RESULTS: There were 99 (93.39%) live births, 4 (3.77%) stillbirths and 3 (2.83%) miscarriage, 20 (18.86%) preterm delivery, 6 (5.66%) SGA, 17 (16.03%) LBW, 11 (10.37%) pre-eclampsia, 7 (6.60%) cases IUGR, 19 (17.92%) cases PROM, 10 (9.43%) cases PPH. Compared with the patients without APOs, the patients with APOs had a higher positive rate of anti-SSA antibodies (73.58% vs. 54.71%, P=0.036), higher rate of leukopenia (15.09% vs. 3.77%, P=0.046), lower haemoglobin level [109.00 (99.50, 118.00) g/L vs. 124.00 (111.50, 132.00) g/L, P < 0.001].Multivariate Logistic regression analysis showed that leucopenia (OR=0.82, 95%CI: 0.688-0.994) was an independent risk factors for APOs in UCTD (P=0.042). Within a mean follow-up time of 5.00 (3.00, 7.00) years, the rate of disease progression to a definite CTD was 14.15%, including 8 (7.54%) Sjögren's syndrome, 4 (3.77%) systemic lupus erythematosus (SLE), 4 (3.77%) rheumatoid arthritis and 1 (0.94%) mixed connective tissue disease. Multivariate Cox proportional risk regression analysis showed that Raynaud phenomenon (HR=40.157, 95%CI: 3.172-508.326) was an independent risk factor for progression to SLE. CONCLUSION: Leukopenia is an independent risk factor for the development of APOs in patients with UCTD. Raynaud's phenmon is a risk factor for the progression of SLE. Tight disease monitoring and regular follow-up are the key measures to prevent adverse pregnancy outcomes and predict disease progression in UCTD patients with pregnancy. FAU - You, Fang Ning AU - You FN AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. AD - Department of Nephropathy and Rheumatology, Chongqing Hospital of Traditional Chinese Medicine, Chongqing 400011, China. FAU - Luo, Liang AU - Luo L AD - Department of Chinese Medicine, the People's Hospital of Yubei District of Chongqing City, Chongqing 401120, China. FAU - Liu, Xiang Jun AU - Liu XJ AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhang, Xue Wu AU - Zhang XW AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Chun AU - Li C AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Pregnancy MH - Infant, Newborn MH - Female MH - Humans MH - Pregnancy Outcome MH - Retrospective Studies MH - *Abortion, Spontaneous/epidemiology/etiology MH - *Undifferentiated Connective Tissue Diseases MH - *Pre-Eclampsia/epidemiology MH - *Lupus Erythematosus, Systemic MH - Risk Factors MH - *Leukopenia MH - *Pregnancy Complications/epidemiology MH - Disease Progression MH - *Connective Tissue Diseases/complications/epidemiology PMC - PMC10724001 OTO - NOTNLM OT - Adverse pregnancy outcomes OT - Risk factor OT - Undifferentiated connective tissue disease EDAT- 2023/12/16 11:43 MHDA- 2023/12/18 06:41 PMCR- 2023/12/18 CRDT- 2023/12/15 19:53 PHST- 2023/12/18 06:41 [medline] PHST- 2023/12/16 11:43 [pubmed] PHST- 2023/12/15 19:53 [entrez] PHST- 2023/12/18 00:00 [pmc-release] AID - bjdxxbyxb-55-6-1045 [pii] AID - 10.19723/j.issn.1671-167X.2023.06.014 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2023 Dec 18;55(6):1045-1052. doi: 10.19723/j.issn.1671-167X.2023.06.014. PMID- 31421031 OWN - NLM STAT- MEDLINE DCOM- 20201127 LR - 20201127 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 72 IP - 10 DP - 2020 Oct TI - Can Patient-Reported Symptoms Be Used to Measure Disease Activity in Systemic Sclerosis? PG - 1459-1465 LID - 10.1002/acr.24053 [doi] AB - OBJECTIVE: To evaluate the association between patient-reported symptoms and changes in disease activity over time in systemic sclerosis (SSc). METHODS: Using data from 1,636 patients enrolled in the Australian Scleroderma Cohort Study, we used generalized estimating equations to determine the relationship between patient-reported worsening of Raynaud's phenomenon (RP), skin involvement, and breathlessness in the month preceding each study visit and features of disease activity in the corresponding organ systems. The associations between the following parameters were analyzed: patient-reported worsening RP and the presence of new-onset digital pitting and digital ulcers; patient-reported worsening skin involvement and increasing modified Rodnan skin thickness score (MRSS); new areas of skin involvement and new-onset joint contractures; patient-reported worsening breathlessness and deteriorating respiratory functions test (RFT) results, indicated by a 10% decrease in forced vital capacity (FVC) and a 15% decrease in diffusing capacity for carbon monoxide (DLco), new-onset interstitial lung disease (ILD), and new-onset pulmonary arterial hypertension (PAH). RESULTS: We found a significant association between patient-reported worsening RP and the presence of digital ulcers (odds ratio [OR] 1.53 [95% confidence interval (95% CI) 0.60-0.93]), patient-reported worsening skin involvement and increasing MRSS (OR 2.10 [95% CI 1.54-2.86]), and worsening patient breathlessness and deteriorating RFTs (FVC OR 2.12 [95% CI 1.70-2.65]; DLco OR 1.97 [95% CI 1.34-2.02]), new-onset ILD (OR 1.91 [95% CI 1.40-2.61]), and new-onset PAH (OR 5.08 [95% CI 3.59-7.19]). CONCLUSION: These results demonstrate that patient-reported symptoms are associated with clinically meaningful changes in disease activity in patients with SSc. This suggests that when objective measures of change in disease status are unavailable, patient-reported symptoms could be used to indicate a change in SSc disease activity. CI - © 2019, American College of Rheumatology. FAU - Ross, Laura AU - Ross L AUID- ORCID: 0000-0003-4636-729X AD - St. Vincent's Hospital Melbourne and the University of Melbourne, Fitzroy, Victoria, Australia. FAU - Stevens, Wendy AU - Stevens W AD - St. Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia. FAU - Wilson, Michelle AU - Wilson M AD - St. Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia. FAU - Strickland, Gemma AU - Strickland G AD - St. Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia. FAU - Walker, Jennifer AU - Walker J AD - Flinders Medical Centre, Bedford Park, South Australia, Australia, and Royal Adelaide Hospital and Flinders University, Adelaide, South Australia, Australia. FAU - Sahhar, Joanne AU - Sahhar J AD - Monash Health and Monash University, Clayton, Victoria, Australia. FAU - Ngian, Gene-Siew AU - Ngian GS AD - Monash Health and Monash University, Clayton, Victoria, Australia. FAU - Roddy, Janet AU - Roddy J AD - Fiona Stanley Hospital, Murdoch, Western Australia, Australia. FAU - Major, Gabor AU - Major G AUID- ORCID: 0000-0003-3464-7438 AD - Royal Newcastle Centre, New Lambton Heights, New South Wales, Australia. FAU - Proudman, Susanna AU - Proudman S AD - Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia. FAU - Baron, Murray AU - Baron M AD - Sir Mortimer B. Davis Jewish General Hospital, Montreal, Quebec, Canada. FAU - Nikpour, Mandana AU - Nikpour M AD - St. Vincent's Hospital Melbourne and the University of Melbourne, Fitzroy, Victoria, Australia. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Aged MH - Cohort Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Respiratory Function Tests MH - *Scleroderma, Systemic MH - *Self Report MH - *Severity of Illness Index EDAT- 2019/08/20 06:00 MHDA- 2020/11/28 06:00 CRDT- 2019/08/18 06:00 PHST- 2019/02/21 00:00 [received] PHST- 2019/08/13 00:00 [accepted] PHST- 2019/08/20 06:00 [pubmed] PHST- 2020/11/28 06:00 [medline] PHST- 2019/08/18 06:00 [entrez] AID - 10.1002/acr.24053 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2020 Oct;72(10):1459-1465. doi: 10.1002/acr.24053. PMID- 19538721 OWN - NLM STAT- MEDLINE DCOM- 20100322 LR - 20211020 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 11 IP - 3 DP - 2009 TI - Negative association of the chemokine receptor CCR5 d32 polymorphism with systemic inflammatory response, extra-articular symptoms and joint erosion in rheumatoid arthritis. PG - R91 LID - 10.1186/ar2733 [doi] AB - INTRODUCTION: Chemokines and their receptors control immune cell migration during infections as well as in autoimmune responses. A 32 bp deletion in the gene of the chemokine receptor CCR5 confers protection against HIV infection, but has also been reported to decrease susceptibility to rheumatoid arthritis (RA). The influence of this deletion variant on the clinical course of this autoimmune disease was investigated. METHODS: Genotyping for CCR5d32 was performed by PCR and subsequent electrophoretic fragment length determination. For the clinical analysis, the following extra-articular manifestations of RA were documented by the rheumatologist following the patient: presence of rheumatoid nodules, major organ vasculitis, pulmonary fibrosis, serositis or a Raynaud's syndrome. All documented CRP levels were analyzed retrospectively, and the last available hand and feet radiographs were analyzed with regards to the presence or absence of erosive disease. RESULTS: Analysis of the CCR5 polymorphism in 503 RA patients and in 459 age-matched healthy controls revealed a significantly decreased disease susceptibility for carriers of the CCR5d32 deletion (Odds ratio 0.67, P = 0.0437). Within the RA patient cohort, CCR5d32 was significantly less frequent in patients with extra-articular manifestations compared with those with limited, articular disease (13.2% versus 22.8%, P = 0.0374). In addition, the deletion was associated with significantly lower average CRP levels over time (median 8.85 vs. median 14.1, P = 0.0041) and had a protective effect against the development of erosive disease (OR = 0.40, P = 0.0047). Intriguingly, homozygosity for the RA associated DNASE2 -1066 G allele had an additive effect on the disease susceptibility conferred by the wt allele of CCR5 (OR = 2.24, P = 0.0051 for carrier of both RA associated alleles) CONCLUSIONS: The presence of CCR5d32 significantly influenced disease susceptibility to and clinical course of RA in a German study population. The protective effect of this deletion, which has been described to lead to a decreased receptor expression in heterozygous patients, underlines the importance of chemokines in the pathogenesis of RA. FAU - Rossol, Manuela AU - Rossol M AD - Division of Rheumatology, Department of Internal Medicine II, University of Leipzig, Johannisallee 30, 04103 Leipzig, Germany. manuela.rossol@medizin.uni-leipzig.de FAU - Pierer, Matthias AU - Pierer M FAU - Arnold, Sybille AU - Arnold S FAU - Keysser, Gernot AU - Keysser G FAU - Burkhardt, Harald AU - Burkhardt H FAU - Baerwald, Christoph AU - Baerwald C FAU - Wagner, Ulf AU - Wagner U LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090618 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Receptors, CCR5) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Arthritis, Rheumatoid/*genetics/immunology/*pathology MH - Cartilage, Articular/immunology/*pathology MH - Case-Control Studies MH - Cohort Studies MH - Female MH - Gene Deletion MH - Genetic Carrier Screening MH - Humans MH - Male MH - Middle Aged MH - Polymorphism, Genetic/*immunology MH - Receptors, CCR5/biosynthesis/*genetics MH - Retrospective Studies MH - Systemic Inflammatory Response Syndrome/*genetics/pathology MH - Young Adult PMC - PMC2714147 EDAT- 2009/06/23 09:00 MHDA- 2010/03/23 06:00 PMCR- 2009/06/18 CRDT- 2009/06/23 09:00 PHST- 2008/12/05 00:00 [received] PHST- 2009/05/08 00:00 [revised] PHST- 2009/06/18 00:00 [accepted] PHST- 2009/06/23 09:00 [entrez] PHST- 2009/06/23 09:00 [pubmed] PHST- 2010/03/23 06:00 [medline] PHST- 2009/06/18 00:00 [pmc-release] AID - ar2733 [pii] AID - 10.1186/ar2733 [doi] PST - ppublish SO - Arthritis Res Ther. 2009;11(3):R91. doi: 10.1186/ar2733. Epub 2009 Jun 18. PMID- 36809134 OWN - NLM STAT- MEDLINE DCOM- 20230224 LR - 20230224 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 28 IP - 5 DP - 2022 Oct 15 TI - Questions raised by a case of adult-onset linear nodular scleroderma. LID - 10.5070/D328559245 [doi] AB - Morphea presenting clinically with nodular or keloidal skin changes is extremely rare. Nodular scleroderma or keloidal morphea presenting in a linear distribution is even more uncommon. We present an otherwise healthy young woman with unilateral, linear, nodular scleroderma and review the somewhat confounding earlier literature in this area. To date, this young woman's skin changes have proven refractory to oral hydroxychloroquine and ultraviolet A1 phototherapy. Several aspects of this case including the patient's family history of Raynaud disease, her nodular sclerodermatous skin lesions, and the presence of U1RNP autoantibodies raised concern about her management with respect to future risk of developing systemic sclerosis. FAU - Marcelus, Christina AU - Marcelus C FAU - Jimenez, Amber AU - Jimenez A FAU - Zussman, Jamie AU - Zussman J FAU - Hansen, Christopher B AU - Hansen CB FAU - Sontheimer, Richard D AU - Sontheimer RD AD - Department of Dermatology, Spencer Fox Eccles School of Medicine, University of Utah, Salt Lake City, Utah, USA. richard.sontheimer@hsc.utah.edu. LA - eng PT - Case Reports PT - Journal Article DEP - 20221015 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Humans MH - Adult MH - Female MH - *Scleroderma, Localized/pathology MH - *Scleroderma, Systemic/pathology MH - Skin/pathology MH - *Keloid/pathology MH - Hydroxychloroquine EDAT- 2023/02/23 06:00 MHDA- 2023/02/25 06:00 CRDT- 2023/02/22 13:33 PHST- 2022/10/19 00:00 [received] PHST- 2022/10/19 00:00 [accepted] PHST- 2023/02/22 13:33 [entrez] PHST- 2023/02/23 06:00 [pubmed] PHST- 2023/02/25 06:00 [medline] AID - 10.5070/D328559245 [doi] PST - epublish SO - Dermatol Online J. 2022 Oct 15;28(5). doi: 10.5070/D328559245. PMID- 41715181 OWN - NLM STAT- In-Process LR - 20260330 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 28 IP - 1 DP - 2026 Feb 20 TI - Regional differences in clinical manifestations of antisynthetase syndrome: a comparison between Asian and European cohorts. LID - 10.1186/s13075-026-03770-5 [doi] LID - 78 AB - BACKGROUND: Antisynthetase syndrome (ASyS) is an idiopathic inflammatory myopathy characterized by interstitial lung disease (ILD), mechanic’s hands, fever, arthritis, and Raynaud’s phenomenon. Although several domestic and international reports have described the clinical manifestations of ASyS, regional differences in clinical characteristics, including skin involvement, remain poorly understood. This study aimed to compare the regional differences in clinical features of ASyS by comparing data of Japanese patients with ASyS with those of previously published cohorts. METHODS: We conducted a retrospective chart review of 48 patients with ASyS at Yokohama City University Hospital between 2010 and 2024 and compared their data with those of previously published cohorts using univariate analysis. A literature review was performed using the PubMed database, with the search results limited to articles published between January 2010 and December 2024. RESULTS: The mean age at onset was 56.5 ± 14.0 years, with a male-to-female ratio of 13:35. The prevalence of anti-Jo1 antibody was 35%. ILD and muscle weakness were observed in 97% and 52% of the patients, respectively. The most frequent skin involvement was Gottron’s sign (73%), followed by mechanic’s hands (65%) and periungual erythema (40%). A literature review using the same ASyS diagnostic criteria revealed that Asian cohorts, including ours, tend to have higher rates of ILD complications and a lower prevalence of anti-Jo1 antibodies than European cohorts. Furthermore, in the comparison of clinical manifestations with other Japanese and Asian cohorts, the rates of Raynaud’s phenomenon, muscle weakness, and malignant complications were nearly identical, although slight differences were observed in skin manifestations. By contrast, the European cohort was characterized by a high frequency of Raynaud’s phenomenon (39%), muscle weakness (85%), and anti-Jo1 antibody positivity (83%); meanwhile, ILD and mechanic’s hands were observed in 68% and 31%, respectively. CONCLUSIONS: These results suggest that regional differences may exist in the clinical manifestations of ASyS, particularly in ILD prevalence, which may be linked to the underlying distribution of anti-aminoacyl transfer RNA synthetase antibody subtypes. A more detailed profiling of each antisynthetase antibody in a larger population is warranted to clarify these differences. FAU - Watanabe, Tomoya AU - Watanabe T AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. nabetomo@yokohama-cu.ac.jp. FAU - Ototake, Yasushi AU - Ototake Y AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. FAU - Tanaka, Masako AU - Tanaka M AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. FAU - Kawamura, Hisho AU - Kawamura H AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. FAU - Moteki, Izumi AU - Moteki I AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. FAU - Kanaoka, Miwa AU - Kanaoka M AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. FAU - Yamaguchi, Yukie AU - Yamaguchi Y AUID- ORCID: 0000-0002-0297-6627 AD - Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. yui1783@yokohama-cu.ac.jp. LA - eng PT - Journal Article DEP - 20260220 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM PMC - PMC13032421 OTO - NOTNLM OT - Antisynthetase antibody OT - Antisynthetase syndrome OT - Clinical manifestation OT - Cohort study OT - Regional difference COIS- Declarations. Ethics approval and consent to participate: This study was approved by the Institutional Review Board of Yokohama City University (approval no. B190800045). Consent to participate was not applicable. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2026/02/20 07:05 MHDA- 2026/02/20 07:05 PMCR- 2026/02/20 CRDT- 2026/02/20 00:25 PHST- 2025/10/05 00:00 [received] PHST- 2026/02/11 00:00 [accepted] PHST- 2026/02/20 07:05 [pubmed] PHST- 2026/02/20 07:05 [medline] PHST- 2026/02/20 00:25 [entrez] PHST- 2026/02/20 00:00 [pmc-release] AID - 10.1186/s13075-026-03770-5 [pii] AID - 3770 [pii] AID - 10.1186/s13075-026-03770-5 [doi] PST - epublish SO - Arthritis Res Ther. 2026 Feb 20;28(1):78. doi: 10.1186/s13075-026-03770-5. PMID- 41994431 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260417 LR - 20260417 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 13 DP - 2026 TI - Cluster analysis and risk prediction model construction for antisynthase syndrome-associated interstitial lung disease based on clinical, imaging, and antibody characteristics. PG - 1798039 LID - 10.3389/fmed.2026.1798039 [doi] LID - 1798039 AB - OBJECTIVE: Interstitial lung disease (ILD) is a common and serious complication of anti-synthase syndrome (ASS), exhibiting high heterogeneity. This study aimed to stratify patients with ASS-associated ILD (ASS-ILD) based on their clinical features, high-resolution computed tomography (HRCT) findings, and specific antibody profiles. We also aimed to build a predictive model for severe ASS-ILD to help identify it more accurately. METHODS: We retrospectively analyzed the clinical and laboratory data of 100 patients with ASS-ILD. Unsupervised clustering of clinical characteristics was done using multiple correspondence analysis (MCA) and hierarchical cluster analysis. Univariate and multivariate logistic regression were used to find independent risk factors for severe ASS-ILD, and a risk prediction model was built. RESULTS: We identified three patient groups: Cluster 1 (n = 46), the joint-predominant group, featured joint pain as the primary clinical manifestation. HRCT typically showed a non-specific interstitial pneumonia (NSIP) pattern, anti-Jo-1 antibodies were most common, and lung damage was relatively mild. Cluster 2 (n = 41), the fever and Raynaud's phenomenon group, had significantly higher anti-EJ antibody levels than the other groups. Cluster 3 (n = 13), the severe lung group, had much higher Warrick scores than the others. This group also had a higher frequency of a usual interstitial pneumonia (UIP) pattern on HRCT, more cases positive for anti-PL-7 or anti-PL-12 antibodies, more severe lung diffusion problems, and more heart involvement. We created and tested a combined biomarker index based on complement C3 level, rash, and age. This index demonstrated superior performance in distinguishing severe from non-severe ILD in ASS patients, independent of clinical variables such as gender, age, and smoking history. CONCLUSION: Identifying ASS-ILD heterogeneity requires comprehensive consideration of HRCT features, antibody profiles, and clinical manifestations. The combined biomarker index (based on complement C3, rash, and age) offers an objective tool for ASS-ILD diagnosis and risk stratification. CI - Copyright © 2026 Zhang, Gao, Chao, Huang, Yang, Zhang, Xia, Lin, Gao, Xu and Yang. FAU - Zhang, Jin AU - Zhang J AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Gao, Wenfeng AU - Gao W AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, China. FAU - Chao, Baoting AU - Chao B AD - Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Huang, Minghua AU - Huang M AD - Department of Respiratory and Critical Care Medicine, Shandong Provincial Third Hospital, Shandong University, Jinan, China. FAU - Yang, Yuyu AU - Yang Y AD - School of Pharmacy, University College London, London, United Kingdom. FAU - Zhang, Ying AU - Zhang Y AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Xia, Rongzhen AU - Xia R AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Lin, Yiting AU - Lin Y AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Gao, Kaining AU - Gao K AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Xu, Wei AU - Xu W AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. FAU - Yang, Qingrui AU - Yang Q AD - Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China. LA - eng PT - Journal Article DEP - 20260401 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC13079685 OTO - NOTNLM OT - Warrick score OT - antisynthetase syndrome OT - cluster analysis OT - high-resolution computed tomography OT - interstitial lung disease COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/04/17 13:26 MHDA- 2026/04/17 13:27 PMCR- 2026/04/01 CRDT- 2026/04/17 05:32 PHST- 2026/01/28 00:00 [received] PHST- 2026/03/12 00:00 [revised] PHST- 2026/03/17 00:00 [accepted] PHST- 2026/04/17 13:27 [medline] PHST- 2026/04/17 13:26 [pubmed] PHST- 2026/04/17 05:32 [entrez] PHST- 2026/04/01 00:00 [pmc-release] AID - 10.3389/fmed.2026.1798039 [doi] PST - epublish SO - Front Med (Lausanne). 2026 Apr 1;13:1798039. doi: 10.3389/fmed.2026.1798039. eCollection 2026. PMID- 37005523 OWN - NLM STAT- MEDLINE DCOM- 20230828 LR - 20230828 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 19 IP - 4 DP - 2023 TI - Clinical Characteristics and Risk Factors of Polymyositis and Dermatomyositis Combined with Interstitial Lung Disease in Patients Residing in the Northeast Sichuan Province of China. PG - 455-462 LID - 10.2174/1573397119666230330082452 [doi] AB - BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are non-suppurative and autoimmune inflammatory diseases of striated muscle. Interstitial lung disease (ILD) is a group of heterogeneous diseases that mainly involve the pulmonary interstitium, alveoli, and/or bronchioles, also known as diffuse parenchymal lung disease (DPLD). A significant cause of death in persons with polymyositis (PM) and dermatomyositis (DM) is concurrent interstitial lung disease (ILD). However, research on the clinical characteristics and associated influencing factors of PM/DM combined with ILD (PM/DM-ILD) is currently scarce in China. OBJECTIVE: The study aimed to probe the clinical features and risk factors of PM/DM-ILD. METHODS: The data of 130 patients with PM/DM were gathered. General medical status, clinical symptoms, laboratory parameters, high-resolution CT, therapeutic outcomes, and prognoses were retrospectively reviewed in patients with PM/DM with (ILD group) and without (NILD) ILD. RESULTS: The age of the ILD group (n=65) was more than the NILD group (n=65), and the difference was statistically significant; there were no significant between-group variations in the PM/DM ratio, sex, or duration of the disease. The initial symptoms were arthritis and respiratory symptoms in the ILD group, and myasthenia symptoms in the NILD group. Incidences of Raynaud's phenomenon, dry cough, expectoration, dyspnea on exertion, arthritis, fever, total globulin (GLOB), erythrocyte sedimentation rate (ESR), and anti-Jo-1 antibody rate were higher for ILD; however, albumin (ALB), creatine kinase aspartate aminotransferase activity ratio (CK/AST) and CK levels were significantly lower in the ILD group. Bivariate logistic regression analysis showed age, dry cough, arthritis, dyspnea on exertion, anti-Jo-1 antibody, and elevated GLOB to be independent risk factors for ILD among patients with PM/DM. CONCLUSION: Advanced age, dry cough, arthritis, dyspnea on exertion, anti-Jo-1 antibody positivity, and elevated GLOB level are risk factors for PM/DM-ILD. This information could be utilized to carefully monitor changing lung function in these patients. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Li, Tao AU - Li T AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Tang, Zi-Yi AU - Tang ZY AD - Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Zhang, Quan-Bo AU - Zhang QB AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Xiao, Fan-Ni AU - Xiao FN AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Jian, Gui-Lin AU - Jian GL AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Xie, Yan AU - Xie Y AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Guo, Jian-Wei AU - Guo JW AD - Department of Geriatrics, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. FAU - Qing, Yu-Feng AU - Qing YF AD - Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, 637000, China. LA - eng GR - 2018HXBH017/Science Foundation for Excellent Youth Scholars of Sichuan University/ GR - 81974250/National Natural Science Foundation of China (NSFC)/ PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 RN - Antisynthetase syndrome SB - IM MH - Cough/complications MH - Myositis MH - *Autoimmune Diseases MH - Risk Factors MH - *Polymyositis/complications/epidemiology MH - Humans MH - Dyspnea/complications MH - Prognosis MH - Retrospective Studies MH - *Dermatomyositis/complications/epidemiology MH - *Lung Diseases, Interstitial/complications/epidemiology/diagnosis OTO - NOTNLM OT - DPLD OT - Polymyositis OT - clinical characteristics OT - dermatomyositis OT - interstitial lung disease OT - risk factors EDAT- 2023/04/04 06:00 MHDA- 2023/08/28 06:42 CRDT- 2023/04/03 00:53 PHST- 2022/07/19 00:00 [received] PHST- 2022/12/17 00:00 [revised] PHST- 2023/01/25 00:00 [accepted] PHST- 2023/08/28 06:42 [medline] PHST- 2023/04/04 06:00 [pubmed] PHST- 2023/04/03 00:53 [entrez] AID - CRR-EPUB-130477 [pii] AID - 10.2174/1573397119666230330082452 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2023;19(4):455-462. doi: 10.2174/1573397119666230330082452. PMID- 39372157 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241008 IS - 2284-2594 (Electronic) IS - 2284-2594 (Linking) VI - 11 IP - 10 DP - 2024 TI - Dacryoadenitis as a Rare Initial Presentation in a Patient with Suspected Crest Syndrome. PG - 004801 LID - 10.12890/2024_004801 [doi] LID - 004801 AB - Systemic sclerosis (SSc) is a rare, chronic disease with diverse clinical presentations, and only a few cases with ocular manifestations have been reported in the literature. In this case report, we describe the case of a 51-year-old South Asian woman who initially complained of painless swelling in her left upper eyelid. An ultrasound examination of the left eye revealed an enlarged lacrimal gland with increased vascularity. The presence of dacryoadenitis, which was unresponsive to initial conservative management with oral antibiotics and warm compresses, along with positive antinuclear antibodies, prompted further investigation. Dacryoadenitis or orbital inflammation is a common presentation in systemic conditions such as Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, or granulomatosis with polyangiitis. However, it can also be a rare initial symptom in a patient with CREST syndrome. To our knowledge, this is only the second case in the literature of dacryoadenitis in the context of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome. This case highlights the significance of serological markers and peripheral clinical presentations in individuals with chronic orbital inflammation, emphasizing the importance of considering further systemic associations. LEARNING POINTS: Broad spectrum of clinical features: Recognize that although CREST syndrome is primarily characterized by its hallmark features - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia - it can also present with additional clinical features that may indicate the onset or presence of the syndrome.Diagnostic challenges and differentiation: Diagnostic challenges and differentiation: Bilateral dacryoadenitis can be challenging to diagnose due to its overlapping symptoms with other orbital or systemic conditions. The case report may highlight the importance of differentiating it from conditions like orbital cellulitis, sarcoidosis, or lymphoproliferative disorders. Advanced imaging techniques (like magnetic resonance imaging or computed tomography scans) and careful assessment of clinical history (including systemic symptoms like fever or autoimmune conditions) are crucial for accurate diagnosis.Awareness of atypical presentations and follow-up: The case report underscores the need for tailored management strategies for bilateral dacryoadenitis, from conservative treatments in mild cases to more aggressive therapies in severe ones, while also highlighting the importance of monitoring for complications and being vigilant about atypical presentations, such as those seen in patients with CREST syndrome. CI - © EFIM 2024. FAU - Singh, Arkaja AU - Singh A AD - Department of Medicine, Mahatma Gandhi Medical College, Jaipur, India. FAU - Rao, Sameer AU - Rao S AD - Department of Medicine, Rutgers New Jersey Medical School, Newark, USA. FAU - Shah, Riya AU - Shah R AD - Department of Medicine, NHL Municipal Medical College, Ahmedabad, Gujarat, India. FAU - Maheshwari, Mashal AU - Maheshwari M AD - Department of Medicine, Sawai Man Singh Medical College, Jaipur, India. FAU - Dhillon, Aarish AU - Dhillon A AD - Department of Neurology, Tufts University, Boston, USA. FAU - Gupta, Silka AU - Gupta S AD - Department of Ophthalmology, Sawai Man Singh Medical College, Jaipur, India. LA - eng PT - Journal Article DEP - 20240919 PL - Italy TA - Eur J Case Rep Intern Med JT - European journal of case reports in internal medicine JID - 101648453 PMC - PMC11451854 OTO - NOTNLM OT - CREST syndrome OT - Systemic sclerosis OT - dacryoadenitis OT - ocular manifestations OT - orbital inflammation COIS- Conflicts of Interests: The Authors declare that there are no competing interests. EDAT- 2024/10/07 11:22 MHDA- 2024/10/07 11:23 PMCR- 2024/09/19 CRDT- 2024/10/07 05:45 PHST- 2024/07/31 00:00 [received] PHST- 2024/09/09 00:00 [accepted] PHST- 2024/10/07 11:23 [medline] PHST- 2024/10/07 11:22 [pubmed] PHST- 2024/10/07 05:45 [entrez] PHST- 2024/09/19 00:00 [pmc-release] AID - 4801 [pii] AID - 10.12890/2024_004801 [doi] PST - epublish SO - Eur J Case Rep Intern Med. 2024 Sep 19;11(10):004801. doi: 10.12890/2024_004801. eCollection 2024. PMID- 27709074 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220311 IS - 2223-4292 (Print) IS - 2223-4306 (Electronic) IS - 2223-4306 (Linking) VI - 6 IP - 4 DP - 2016 Aug TI - Correlation of delta high-resolution computed tomography (HRCT) score with delta clinical variables in early systemic sclerosis (SSc) patients. PG - 381-390 AB - BACKGROUND: The correlation of changes (delta: Δ) of high-resolution computed tomography (HRCT) score with the Δ of other clinical variables has not been well studied. The purpose of this study was to determine the correlation of Δ HRCT score with Δ percent predicted forced vital capacity (%pFVC), Δ modified Rodnan Skin Score (mRSS), Δ erythrocyte sedimentation rate (ESR), and Δ percent of oxygen saturation at room air (%SpO(2)) in patients with early systemic sclerosis (SSc). METHODS: We used an inception cohort of early-SSc patients seen at the Rheumatology Clinic, Chiang Mai University, Thailand, between January 2010 and June 2014. All patients underwent HRCT at study entry and every 12 months thereafter. Thirty-one SSc patients who underwent pulmonary function test (PFT) within 12 weeks of their corresponding HRCT at baseline and last visit were identified. The extent of ground glass (GG), lung fibrosis (Fib), bronchiectasis (B), and honeycombing (HC) was scored, and then aggregated to produce a total (t) HRCT score. RESULTS: Mean ± SD age and disease duration from non-Raynaud's phenomenon (NRP) to undergo HRCT at baseline were 52.2±8.8 years and 11.7±7.1 months, respectively. Seventeen (54.8%) patients were female and 20 (64.5%) were classified as dcSSc. The mean ± SD interval between the two HRCT tests was 16.0±7.2 months. The Δ HRCT scores [total fibrosis scores (t-Fib), total bronchiectasis scores (t-B), and total HRCT score (t-HRCT) scores] and Δ mRSS, but not Δ %pFVC, showed significant change over the observation period. We found significant correlation of Δ total honeycombing scores (t-HC) with Δ ESR (r=-0.44, P<0.05), and Δ t-Fib with Δ %SpO(2) (r=-0.38, P<0.05). However, no significant correlation of any Δ HRCT scores with Δ %pFVC and Δ mRSS were observed. CONCLUSIONS: In this study, the changes in the HRCT scores were greater than %pFVC; this, along with their correlations with the changes in ESR and %SpO(2), suggest that HRCT scores are a useful and sensitive method for monitoring disease progression in early SSc-related ILD (SSc-ILD). FAU - Wangkaew, Suparaporn AU - Wangkaew S AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Euathrongchit, Juntima AU - Euathrongchit J AD - Division of Diagnostic Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Wattanawittawas, Pittaporn AU - Wattanawittawas P AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Kasitanon, Nuntana AU - Kasitanon N AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. LA - eng PT - Journal Article PL - China TA - Quant Imaging Med Surg JT - Quantitative imaging in medicine and surgery JID - 101577942 PMC - PMC5009111 OTO - NOTNLM OT - Correlation OT - high-resolution computed tomography (HRCT) OT - interstitial lung disease (ILD) OT - systemic sclerosis (SSc) COIS- The authors have no conflicts of interest to declare. EDAT- 2016/10/07 06:00 MHDA- 2016/10/07 06:01 PMCR- 2016/08/01 CRDT- 2016/10/07 06:00 PHST- 2016/10/07 06:00 [entrez] PHST- 2016/10/07 06:00 [pubmed] PHST- 2016/10/07 06:01 [medline] PHST- 2016/08/01 00:00 [pmc-release] AID - qims-06-04-381 [pii] AID - 10.21037/qims.2016.08.08 [doi] PST - ppublish SO - Quant Imaging Med Surg. 2016 Aug;6(4):381-390. doi: 10.21037/qims.2016.08.08. PMID- 39860561 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250130 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 2 DP - 2025 Jan 16 TI - Identification of Key Predictors of Cryoglobulinemia Severity at Diagnosis: Threshold, Type, and Severity Score at Diagnosis. LID - 10.3390/jcm14020556 [doi] LID - 556 AB - Background: Cryoglobulinemia (CG) syndrome is a heterogeneous condition characterized by the presence of cryoglobulins in serum, often leading to vasculitis with protean clinical manifestations. Understanding the presentation of cryoglobulinemia-related symptoms based on cryoprecipitate levels, GC type, and severity at diagnosis is essential for effective management. Hence, this study aimed to provide a comprehensive analysis of patients with positive cryoglobulin detection to investigate these aspects. Methods: We conducted a retrospective review of clinical charts from patients with positive cryoglobulin detection at Colmar Hospital between May 2015 and April 2019. Results: Among 166 patients with positive cryoglobulins, the median cryoprecipitate value was 37 mg/L [IQR: 25-70], with 62% of patients below the 50 mg/L threshold. High cryoprecipitate levels were associated with C-virus hepatitis (p = 0.0007), increased fatigue (p = 0.001), fever (p = 0.0013), weight loss (p = 0.028), and musculoskeletal symptoms (p = 0.002). These patients also exhibited decreases in complement fractions (p-values 0.017 to 0.006). At the end of the one-year follow-up, they required frequent renal replacement therapy (p < 0.0001) and had a higher mortality rate (p = 0.02). Based on the CG type, patients with type I GC had splenomegaly (p = 0.039) and hemopathy (p = 0.001). According to severity at initial presentation, the severe patients had more purpura (p < 0.001), Raynaud's phenomenon (p = 0.039), and leukocytoclastic vasculitis on skin biopsy (p < 0.001), along with higher cryoprecipitate levels (p = 0.011). Multivariate analysis identified purpura (OR: 10.25), hematological malignancy (OR: 7.06), Raynaud's phenomenon (OR: 6.41), and cryoprecipitate levels (OR: 1.02) as significant markers of disease severity serving for the development of a severity score for clinical practice. Conclusions: This study identifies severity markers in patients with positive cryoprecipitate and proposes a score related to severity at diagnosis. FAU - Razanamahery, Jerome AU - Razanamahery J AUID- ORCID: 0000-0001-8453-7224 AD - Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, 21000 Dijon, France. FAU - Aubertin, Nils AU - Aubertin N AD - Department of Geriatric Medicine, Centre Départementale de Repos et de Soins, 68026 Colmar, France. FAU - Bach Bunner, Maxime AU - Bach Bunner M AD - Department of Internal Medicine and Clinical Immunology, Hopitaux Civils de Colmar, 68026 Colmar, France. FAU - Blaison, Gilles AU - Blaison G AD - Department of Internal Medicine and Clinical Immunology, Hopitaux Civils de Colmar, 68026 Colmar, France. FAU - Bouldoires, Bastien AU - Bouldoires B AD - Department of Internal Medicine and Clinical Immunology, Hopital de Macon, 71000 Macon, France. FAU - Soumagne, Thibaud AU - Soumagne T AD - Department of Pulmonary Medicine, European Hospital Georges Pompidou, 75015 Paris, France. LA - eng PT - Journal Article DEP - 20250116 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC11765962 OTO - NOTNLM OT - cryoglobulinemia OT - diagnosis methods OT - score OT - severity OT - vasculitis COIS- The authors declare no competing financial interests for this study. EDAT- 2025/01/25 13:52 MHDA- 2025/01/25 13:53 PMCR- 2025/01/16 CRDT- 2025/01/25 01:23 PHST- 2024/12/09 00:00 [received] PHST- 2025/01/03 00:00 [revised] PHST- 2025/01/14 00:00 [accepted] PHST- 2025/01/25 13:53 [medline] PHST- 2025/01/25 13:52 [pubmed] PHST- 2025/01/25 01:23 [entrez] PHST- 2025/01/16 00:00 [pmc-release] AID - jcm14020556 [pii] AID - jcm-14-00556 [pii] AID - 10.3390/jcm14020556 [doi] PST - epublish SO - J Clin Med. 2025 Jan 16;14(2):556. doi: 10.3390/jcm14020556. PMID- 41929493 OWN - NLM STAT- MEDLINE DCOM- 20260403 LR - 20260403 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Risk factors for pulmonary complications in systemic lupus erythematosus: a meta-analysis of infectious pneumonia and interstitial lung disease. PG - 1758316 LID - 10.3389/fimmu.2026.1758316 [doi] LID - 1758316 AB - BACKGROUND: Pulmonary complications (PC), including infectious pneumonia (IP) and non-infectious interstitial lung disease (ILD), are major contributors of morbidity and mortality in systemic lupus erythematosus (SLE). As they are fundamentally different with respect to their respective etiologies and pathophysiology, we aimed to comprehensively identify and compare their risk factors. METHODS: We conducted a comprehensive literature search in seven electronic databases from database inception to September 2025. Pooled effect sizes were computed using appropriate model-based methods and thoroughly examined for heterogeneity. Our analytical approach advanced an evidence stratification framework that integrated both univariate associations and multivariate-adjusted results, which classified factors into four strata of findings: robust independent risk factors, preliminary independent risk factors, potential risk factors, and protective factors. RESULTS: In total, 16 studies comprising 6,978 participants yielded fundamentally distinct risk architectures. IP was predominantly driven by immunosuppression and systemic inflammation, with robust independent risk factors including advanced age, pulmonary involvement, high CRP/WBC, immunosuppressant use, and antibacterial drug use. In contrast, ILD was strongly driven by autoimmunity and vascular pathology, with preliminary independent risk factors including Raynaud's phenomenon, anti-Sm antibody positivity, high IgG and C4 levels. Most strikingly, serum IgG emerged as two strongly associated factors: low levels of serum IgG protected against IP, whereas high levels of serum IgG increased ILD risk. CONCLUSION: This is the first study to systematically stratify PC risk factors in SLE, demonstrating their distinct pathogenesis. The hierarchic framework allows a shift from a uniform management mindset to individualized risk evaluation for the respective complications, supportimg targeted prevention and early detection strategies. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/PROSPERO/view, identifier CRD420251020965. CI - Copyright © 2026 Yang, Wu, Wu, Huang, Fan and Bao. FAU - Yang, Ze AU - Yang Z AD - The First School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Wu, Yanzuo AU - Wu Y AD - The First School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Wu, Zexuan AU - Wu Z AD - School of Basic Medical Sciences, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Huang, Shuo AU - Huang S AD - School of Basic Medical Sciences, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Fan, Yongsheng AU - Fan Y AD - Department of Rheumatology, The Second Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. FAU - Bao, Jie AU - Bao J AD - School of Basic Medical Sciences, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20260318 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 SB - IM MH - Humans MH - *Lung Diseases, Interstitial/etiology/epidemiology/immunology MH - *Lupus Erythematosus, Systemic/complications/immunology MH - Risk Factors MH - *Pneumonia/etiology PMC - PMC13038906 OTO - NOTNLM OT - infectious pneumonia OT - interstitial lung disease OT - meta-analysis OT - pulmonary complication OT - risk factors OT - systemic lupus erythematosus COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/04/03 06:30 MHDA- 2026/04/03 06:31 PMCR- 2026/03/18 CRDT- 2026/04/03 05:00 PHST- 2025/12/01 00:00 [received] PHST- 2026/02/16 00:00 [revised] PHST- 2026/02/20 00:00 [accepted] PHST- 2026/04/03 06:31 [medline] PHST- 2026/04/03 06:30 [pubmed] PHST- 2026/04/03 05:00 [entrez] PHST- 2026/03/18 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1758316 [doi] PST - epublish SO - Front Immunol. 2026 Mar 18;17:1758316. doi: 10.3389/fimmu.2026.1758316. eCollection 2026. PMID- 41388317 OWN - NLM STAT- MEDLINE DCOM- 20251213 LR - 20251216 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 27 IP - 1 DP - 2025 Dec 12 TI - Clinical significance of inflammation-coagulation-autoantibody triad in systemic sclerosis-associated interstitial lung disease: a retrospective study. PG - 225 LID - 10.1186/s13075-025-03685-7 [doi] LID - 225 AB - BACKGROUND: Interstitial lung disease (ILD) is highly prevalent among systemic sclerosis (SSc) patients and is the leading cause of mortality. This study aims to evaluate the independent and combined value of inflammatory markers, coagulation parameters, and autoantibodies for the presence of SSc-ILD. METHODS: A total of 177 patients with SSc were enrolled in this study, including 116 patients with SSc-ILD and 61 without ILD. Multivariate logistic regression was performed to identify independently associated factors for SSc-ILD. The identification efficiency of individual and combined biomarkers was assessed using receiver operating characteristic curves, with the DeLong test applied to compare differences in area under the curve. RESULTS: The incidence of dcSSc, Raynaud's phenomenon, and digestive system involvement was significantly higher in the SSc-ILD group. Four stepwise models were constructed, and the results of the full-factor model indicated that elevated NLR (neutrophil to lymphocyte ratio), prolonged PT (prothrombin time), and anti-SCL-70 antibody positivity were independent associated factors for SSc-ILD. The combined model of NLR, PT, and anti-SCL-70 yielded an AUC of 0.879 (95% CI: 0.828-0.930), which was significantly greater than any individual marker (P < 0.001), with a sensitivity of 82.6% and a specificity of 85.0%. CONCLUSION: NLR, PT, and anti-SCL-70 antibody together form a triad of independent associated factors for SSc-ILD, demonstrating substantial discriminatory potential when considered as a composite indicator. CI - © 2025. The Author(s). FAU - Guo, Ronghong AU - Guo R AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. FAU - Zhou, Xianzhe AU - Zhou X AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. FAU - Chen, Jiaxin AU - Chen J AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. FAU - Gao, Jinfang AU - Gao J AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. FAU - Yang, Yanli AU - Yang Y AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. FAU - Xu, Ke AU - Xu K AD - Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, 030032, Taiyuan, China. xuke@sxbqeh.com.cn. LA - eng GR - 81871292/National Natural Science Foundation of China/ GR - 2023XM002/Four "batches" innovation project of invigorating medical through science and technology of Shanxi province/ GR - 202303021211218/Fundamental Research Program of Shanxi Province/ GR - 201803D31136/Key Research and Development (R&D) Projects of Shanxi Province/ PT - Journal Article DEP - 20251212 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/blood/immunology MH - Female MH - Male MH - *Lung Diseases, Interstitial/blood/immunology/etiology/diagnosis MH - Middle Aged MH - Retrospective Studies MH - *Autoantibodies/blood/immunology MH - Biomarkers/blood MH - Aged MH - Adult MH - *Inflammation/blood/immunology MH - *Blood Coagulation/physiology MH - Clinical Relevance PMC - PMC12699806 OTO - NOTNLM OT - Autoantibody OT - Coagulation OT - Inflammation OT - Interstitial lung disease OT - Systemic sclerosis COIS- Declarations. Competing interests: The authors declare no competing interests. EDAT- 2025/12/13 06:29 MHDA- 2025/12/13 06:30 PMCR- 2025/12/12 CRDT- 2025/12/13 00:39 PHST- 2025/08/21 00:00 [received] PHST- 2025/10/30 00:00 [accepted] PHST- 2025/12/13 06:30 [medline] PHST- 2025/12/13 06:29 [pubmed] PHST- 2025/12/13 00:39 [entrez] PHST- 2025/12/12 00:00 [pmc-release] AID - 10.1186/s13075-025-03685-7 [pii] AID - 3685 [pii] AID - 10.1186/s13075-025-03685-7 [doi] PST - epublish SO - Arthritis Res Ther. 2025 Dec 12;27(1):225. doi: 10.1186/s13075-025-03685-7. PMID- 24557777 OWN - NLM STAT- MEDLINE DCOM- 20160324 LR - 20220410 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 23 IP - 7 DP - 2014 Jun TI - Rare autoantibodies to cellular antigens in systemic lupus erythematosus. PG - 672-7 LID - 10.1177/0961203314524850 [doi] AB - OBJECTIVE: A high number of antinuclear antibody specificities can be detected in systemic lupus erythematosus (SLE). Some of them are related to a distinct clinical subset of disease, independently of their frequency. The aim of our study was to investigate, in a cohort of SLE patients, the prevalence and the clinical relevance of autoantibodies to cellular antigens less frequently found in SLE. METHODS: Antinuclear antibodies were detected by indirect immunofluorescence on HEp-2 cells while counterimmunoelectrophoresis was used to detect anti-ENA antibodies in 540 patients with SLE, classified according to ACR and SLICC criteria. Clinical and serological features were collected from clinical charts. RESULTS: A total of 319 (58.9%) out of 540 sera were positive for anti-ENA antibodies. Anti-Ro/SSA was found in 235 sera, 50 of which also contained anti-La/SSB. Anti-U1RNP were detected in 67, anti-Sm in 46 and anti-ribosomal P protein in 13 sera. In a multivariate analysis anti-Sm was associated with discoid lupus (p = 0.045) and photosensitivity (p = 0.037), anti-U1RNP with malar rash and Raynaud's phenomenon (p = 0.01 and p = 0.0004, respectively) and anti-Ro/SSA with malar rash, oral ulcers, xerostomia, xerophthalmia and rheumatoid factor (p = 0.029, p = 0.01, p = 0.031, p = 0.002 and p = 0.028, respectively). Other anti-ENA antibodies were found in 50 positive sera (15.6%). Anti-Ki antibodies were detected in 31, anti-Ku in 8, anti-centromere in 5, isolated anti-La/SSB, anti-PCNA and anti-Topo I in 3 each and anti-Jo-1 in 2 sera. About half of these antibodies (27 out of 50) were detected as the single anti-ENA specificity in serum. At multivariate analysis anti-Ki was significantly associated with male gender while anti-Ku with African ethnicity (p = 0.017 and p < 0.0001, respectively). No sign of muscular or pulmonary involvement was found in anti-Jo-1-positive patients whilst features of systemic sclerosis were detectable in two out of three anti-Topo I. CONCLUSIONS: Our study shows that antibodies to cellular antigens more rarely found in SLE are detectable in more than 15% of patients with anti-ENA antibodies. Most of them are found as single anti-ENA specificity. Anti-Ki and anti-Ku are found in a subset of disease, characterized by male gender and African origin, respectively. Clinical features of scleroderma were found only in patients with anti-Topo I. CI - © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Fredi, M AU - Fredi M AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy Dipartimento di Scienze Cliniche e Sperimentali, Università degli Studi di Brescia, Brescia, Italy Rheumatology Chair, Università degli Studi di Pavia, Pavia, Italy elmic83@libero.it. FAU - Cavazzana, I AU - Cavazzana I AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy. FAU - Quinzanini, M AU - Quinzanini M AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy. FAU - Taraborelli, M AU - Taraborelli M AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy Dipartimento di Scienze Cliniche e Sperimentali, Università degli Studi di Brescia, Brescia, Italy Rheumatology Chair, Università degli Studi di Pavia, Pavia, Italy. FAU - Cartella, S AU - Cartella S AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy. FAU - Tincani, A AU - Tincani A AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy Dipartimento di Scienze Cliniche e Sperimentali, Università degli Studi di Brescia, Brescia, Italy. FAU - Franceschini, F AU - Franceschini F AD - Rheumatology and Clinical Immunology Unit, Spedali Civili, Brescia, Italy. LA - eng PT - Journal Article DEP - 20140220 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antigens) RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Antigens MH - Autoantibodies/*blood MH - Cells/immunology MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*blood/*immunology MH - Male MH - Retrospective Studies OTO - NOTNLM OT - Rare antibodies OT - anti-ENA OT - anti-Ki OT - anti-Ku OT - subset disease OT - systemic lupus erythematosus EDAT- 2014/02/22 06:00 MHDA- 2016/03/25 06:00 CRDT- 2014/02/22 06:00 PHST- 2013/09/30 00:00 [received] PHST- 2014/01/23 00:00 [accepted] PHST- 2014/02/22 06:00 [entrez] PHST- 2014/02/22 06:00 [pubmed] PHST- 2016/03/25 06:00 [medline] AID - 0961203314524850 [pii] AID - 10.1177/0961203314524850 [doi] PST - ppublish SO - Lupus. 2014 Jun;23(7):672-7. doi: 10.1177/0961203314524850. Epub 2014 Feb 20. PMID- 42112350 OWN - NLM STAT- MEDLINE DCOM- 20260511 LR - 20260511 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 17 DP - 2026 TI - Anti-centromere protein B antibody positivity in primary Sjögren's disease: clinical features and prognostic implications. PG - 1665077 LID - 10.3389/fimmu.2026.1665077 [doi] LID - 1665077 AB - OBJECTIVE: To investigate the clinical and immunological features of primary Sjögren's disease (pSjD) patients with anti-centromere protein B (CENP-B) antibody positivity and to evaluate its prognostic significance. METHODS: This ambispective cohort study included 1,222 patients with pSjD from the China-Japan Friendship Hospital between February 2014 and February 2023, with follow-up through February 2024. Patients were categorized into anti-CENP-B positive and negative groups based on serum testing. Clinical characteristics, immunological features, and outcomes were compared between groups. A subgroup analysis compared patients with isolated CENP-B positivity to those with additional autoantibodies. Statistical analyses were conducted using SPSS 26.0 and R 4.2.3, including descriptive statistics, univariate tests, and Kaplan-Meier survival analysis. RESULTS: In this study, 100 patients (8.2%) were positive for anti-CENP-B antibody, while 1,122 patients (91.8%) were negative. Compared with the negative group, positive patients were older, more often female, and have higher rates of xerostomia and Raynaud's phenomenon, but lower frequency of dyspnea. They showed lower platelet counts, higher aspartate aminotransferase, gamma glutamyl transferase, lactate dehydrogenase, total bilirubin, and lower estimated glomerular filtration rate, with reduced frequencies of elevated Immunoglobulin (Ig) G, but increased IgM levels and slightly lower EULAR Sjögren's Syndrome Disease Activity Index scores. Among anti-CENP-B positive patients, those with isolated anti-CENP-B positivity had milder immunologic abnormalities than those with concomitant autoantibodies. Among patients with available LSGB data, focal lymphocytic infiltration counts were higher in the anti-CENP-B-positive group. Anti-CENP-B positivity was not significantly associated with mortality, cancer, or interstitial lung disease. CONCLUSION: Anti-CENP-B positivity in pSjD may identify a different baseline serological and clinical profile, characterized by relatively milder immunologic abnormalities and lower disease activity. However, its prognostic and clinical significance remains uncertain and requires further validation. CI - Copyright © 2026 Lei, Zhang, Chen, Zhang, Liu, Zhou, Tang, Luo, Song and Tao. FAU - Lei, Chunxin AU - Lei C AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Zhang, Yan AU - Zhang Y AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Chen, Jiaqi AU - Chen J AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Zhang, Xiya AU - Zhang X AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Liu, Zihan AU - Liu Z AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Zhou, Xuanyi AU - Zhou X AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Tang, Bojie AU - Tang B AD - Graduate School, Beijing University of Chinese Medicine, Beijing, China. AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Luo, Jing AU - Luo J AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. FAU - Song, Weijiang AU - Song W AD - Traditional Chinese Medicine Department, Peking University Third Hospital, Beijing, China. FAU - Tao, Qingwen AU - Tao Q AD - National Center for Integrative Medicine, Department of Traditional Chinese Medicine Rheumatism, China-Japan Friendship Hospital, Beijing, China. LA - eng PT - Journal Article DEP - 20260423 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Sjogren's Syndrome/immunology/diagnosis/mortality/blood MH - Female MH - Male MH - Middle Aged MH - Prognosis MH - *Autoantibodies/blood/immunology MH - Adult MH - Aged MH - Biomarkers PMC - PMC13149392 OTO - NOTNLM OT - Sjögren’s disease OT - anti-centromere protein B OT - clinical features OT - prognosis OT - real-world cohort study COIS- The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2026/05/11 13:00 MHDA- 2026/05/11 13:01 PMCR- 2026/04/23 CRDT- 2026/05/11 07:01 PHST- 2025/07/13 00:00 [received] PHST- 2026/03/24 00:00 [revised] PHST- 2026/04/10 00:00 [accepted] PHST- 2026/05/11 13:01 [medline] PHST- 2026/05/11 13:00 [pubmed] PHST- 2026/05/11 07:01 [entrez] PHST- 2026/04/23 00:00 [pmc-release] AID - 10.3389/fimmu.2026.1665077 [doi] PST - epublish SO - Front Immunol. 2026 Apr 23;17:1665077. doi: 10.3389/fimmu.2026.1665077. eCollection 2026. PMID- 19004039 OWN - NLM STAT- MEDLINE DCOM- 20090323 LR - 20250529 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 1 DP - 2009 Jan TI - Fibromyalgia, systemic lupus erythematosus (SLE), and evaluation of SLE activity. PG - 82-8 LID - 10.3899/jrheum.080212 [doi] AB - OBJECTIVE: To determine if fibromyalgia (FM) or fibromyalgia-ness (the tendency to respond to illness and psychosocial stress with fatigue, widespread pain, general increase in symptoms, and similar factors) is increased in patients with compared to those without systemic lupus erythematosus (SLE); to determine whether FM or fibromyalgia-ness biases the SLE Activity Questionnaire (SLAQ); and to determine if the SLAQ is overly sensitive to FM symptoms. METHODS: We developed a 16-item SLE Symptom Scale (SLESS) modeled on the SLAQ and used that scale to investigate the relation between SLE symptoms and fibromyalgia-ness in 23,321 patients with rheumatic disease. FM was diagnosed by survey FM criteria, and fibromyalgia-ness was measured using the Symptom Intensity (SI) Scale. As comparison groups, we combined patients with rheumatoid arthritis and noninflammatory rheumatic disorders into an "arthritis" group and also utilized a physician-diagnosed group of patients with FM. RESULTS: FM was identified in 22.1% of SLE and 17.0% of those with arthritis. The SI scale was minimally increased in SLE. The correlation between SLAQ and SLESS was 0.738. SLESS/SLAQ scale items (Raynaud's phenomenon, rash, fever, easy bruising, hair loss) were significantly more associated with SLE than FM, while the reverse was true for headache, abdominal pain, paresthesias/stroke, fatigue, cognitive problems, and muscle pain or weakness. There was no evidence of disproportionate symptom-reporting associated with fibromyalgia-ness. Self-reported SLE was associated with an increased prevalence of FM that was unconfirmed by physicians, compared to SLE confirmed by physicians. CONCLUSION: The prevalence of FM in SLE is minimally increased compared with its prevalence in patients with arthritis. Fibromyalgia-ness does not bias the SLESS and should not bias SLE assessments, including the SLAQ. FAU - Wolfe, Frederick AU - Wolfe F AD - National Data Bank for Rheumatic Diseases, 1035 N. Emporia, Suite 288, Wichita, KS 67214, USA. fwolfe@arthritis-research.org FAU - Petri, Michelle AU - Petri M FAU - Alarcón, Graciela S AU - Alarcón GS FAU - Goldman, John AU - Goldman J FAU - Chakravarty, Eliza F AU - Chakravarty EF FAU - Katz, Robert S AU - Katz RS FAU - Karlson, Elizabeth W AU - Karlson EW LA - eng GR - K24 AR052403/AR/NIAMS NIH HHS/United States GR - P60 AR047782/AR/NIAMS NIH HHS/United States GR - R01 AR049880/AR/NIAMS NIH HHS/United States PT - Journal Article PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Databases, Factual MH - Fatigue/epidemiology/physiopathology MH - Female MH - Fibromyalgia/drug therapy/*epidemiology/*physiopathology MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/drug therapy/*epidemiology/*physiopathology MH - Male MH - Middle Aged MH - Pain/epidemiology/physiopathology MH - Prevalence MH - *Severity of Illness Index MH - Surveys and Questionnaires PMC - PMC2944223 MID - NIHMS233996 COIS- The authors declare no conflict of interest. EDAT- 2008/11/13 09:00 MHDA- 2009/03/24 09:00 PMCR- 2010/09/23 CRDT- 2008/11/13 09:00 PHST- 2008/11/13 09:00 [pubmed] PHST- 2009/03/24 09:00 [medline] PHST- 2008/11/13 09:00 [entrez] PHST- 2010/09/23 00:00 [pmc-release] AID - 36/1/82 [pii] AID - 10.3899/jrheum.080212 [doi] PST - ppublish SO - J Rheumatol. 2009 Jan;36(1):82-8. doi: 10.3899/jrheum.080212. PMID- 38233944 OWN - NLM STAT- MEDLINE DCOM- 20240119 LR - 20240226 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 26 IP - 1 DP - 2024 Jan 17 TI - Children systemic lupus erythematosus-associated pancreatitis. PG - 28 LID - 10.1186/s13075-024-03265-1 [doi] LID - 28 AB - OBJECTIVE: To early recognise and improve the prognosis of children systemic lupus erythematosus (cSLE)-associated pancreatitis by summarising and analysing clinical features and prognosis data from 12 cases. METHODS: Retrospective analysis of clinical data from 12 cases of cSLE-associated pancreatitis diagnosed and treated from January 2016 to December 2021 at hospitals such as Children's Hospital of Capital Institute of Paediatrics. RESULTS: The median SLEDAI-2K score for disease activity was 18.00 (range 12.25-21.00) in the case group and 10.00 (range 7.00-18.00) in the control group, with a statistically significant difference (P < 0.05) between the two groups. The case group had a higher proportion of abdominal pain, vomiting, abdominal distension, pleural effusion, Raynaud's phenomenon (RP), splenic infarction, and concurrent macrophage activation syndrome (MAS) than the control group, with a statistically significant difference (P < 0.05). Serum ferritin (SF), alanine transaminase (ALT), aspartate transaminase (AST), lactate dehydrogenase (LDH), amylase, and increased 24-h urine protein levels were statistically different between the two groups (P < 0.05); platelet counts (PLT) reduction was also statistically different (P < 0.05). The case group had a higher proportion of methylprednisolone pulse therapy, cyclophosphamide pulse therapy during remission induction, and therapeutic plasma exchange than the control group, with a statistically significant difference (P < 0.05) between the two. CONCLUSION: CSLE-associated pancreatitis has a high fatality rate. The presence of RP, splenic infarction, pleural effusion, and MAS warrants attention from clinicians regarding the possibility of pancreatitis. Once pancreatitis is detected, the primary disease needs active treatment for better prognosis. CI - © 2024. The Author(s). FAU - Zhang, Dan AU - Zhang D AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Lai, Jianming AU - Lai J AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. laijm99@sina.com. FAU - Su, Gaixiu AU - Su G AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Zhu, Jia AU - Zhu J AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Min AU - Min FAU - Kang AU - Kang AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Li, Ming AU - Li M AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Xu, Yingjie AU - Xu Y AD - Department of Rheumatology and Immunology, Children's Hospital of Capital Institute of Paediatrics, No.2 Yabao Road, Chaoyang District, Beijing, 100020, China. FAU - Meng, Li AU - Meng L AD - Department of Internal Medicine, Children's Hospital of Capital Institute of Paediatrics, Beijing, China. LA - eng GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ GR - LCYJ-2023-12/Clinical Cultivation Project of the Capital Institute of Pediatrics/ PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20240117 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Humans MH - Child MH - Retrospective Studies MH - *Splenic Infarction MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - *Pancreatitis/etiology/therapy MH - *Pleural Effusion PMC - PMC10792798 COIS- The authors declare no competing interests. EDAT- 2024/01/18 00:42 MHDA- 2024/01/19 06:42 PMCR- 2024/01/17 CRDT- 2024/01/17 23:43 PHST- 2023/11/13 00:00 [received] PHST- 2024/01/09 00:00 [accepted] PHST- 2024/01/19 06:42 [medline] PHST- 2024/01/18 00:42 [pubmed] PHST- 2024/01/17 23:43 [entrez] PHST- 2024/01/17 00:00 [pmc-release] AID - 10.1186/s13075-024-03265-1 [pii] AID - 3265 [pii] AID - 10.1186/s13075-024-03265-1 [doi] PST - epublish SO - Arthritis Res Ther. 2024 Jan 17;26(1):28. doi: 10.1186/s13075-024-03265-1. PMID- 34064667 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210615 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 9 IP - 5 DP - 2021 May 11 TI - Endocan and Circulating Progenitor Cells in Women with Systemic Sclerosis: Association with Inflammation and Pulmonary Hypertension. LID - 10.3390/biomedicines9050533 [doi] LID - 533 AB - BACKGROUND: Systemic sclerosis (SSc) is characterized by early vasculopathy and fibrosis in the skin, lungs, and other tissues. Vascular manifestations of SSc include Raynaud's phenomenon, digital ulcers, and pulmonary artery hypertension (PAH). PAH is the second most common cause of mortality in SSc. Circulating CD34+ cells associated with cardiovascular health status in several conditions, including chronic immune-inflammatory disease. CD34+ cell numbers have been found inconstantly reduced in SSc. Endocan, a proteoglycan expressed by endothelial cells, was recently suggested as a marker of vascular stress. We tested the relationships among CD34+ cells, endocan, inflammatory markers, vitamin D levels, and clinical parameters in SSc patients with PAH. METHODS: Standard echocardiography was performed. Vitamin D levels, CD34+ cells, inflammatory markers, endocan plasma levels were determined in 36 female SSc patients (24 diffuse/12 limited) and 36 matched controls (HC). RESULTS: We found no difference in CD34+ and vitamin D levels in SSc as compared to controls; ESR, CRP, fibrinogen, endocan, sPAP were higher in SSc with respect to controls. We found a correlation between endocan and: CD34+ cells (r: -0.540, p = 0.002), pulmonary arterial pressure (sPAP) (r: 0.565, p < 0.001), tricuspid annular plane excursion (TAPSE) (r: -0.311, p < 0.01), and E/A ratio (r: -0.487, p < 0.001), but not with ejection fraction (r: -0.057, p = 0.785) in SSc. CD34+ cells correlate with fibrinogen (r: -0.619, p < 0.001), sPAP (r: -0.404, p = 0.011), E/A (r: 0.470, p < 0.005 in SSc. CONCLUSION: CD34+ cell number was significantly correlated with endocan levels and with sPAP in SSc; endocan and CD34+ progenitor cells might be suggested as a potential marker of disease status. FAU - Lo Gullo, Alberto AU - Lo Gullo A AD - Medicine and Urgency Unit, Piemonte Hospital, IRCCS Neurolesi Bonino Pulejo, 98121 Messina, Italy. FAU - Mandraffino, Giuseppe AU - Mandraffino G AUID- ORCID: 0000-0003-0272-2237 AD - Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Rodríguez-Carrio, Javier AU - Rodríguez-Carrio J AUID- ORCID: 0000-0002-0011-5102 AD - Area of Immunology, Department of Functional Biology, Faculty of Medicine, University of Oviedo, 33006 Oviedo, Spain. AD - Instituto de Investigación Sanitaria Del Principado de Asturias (ISPA), 33011 Oviedo, Spain. AD - Bone and Mineral Research Unit, Instituto Reina Sofía de Investigación Nefrológica, REDinREN Del ISCIII, Hospital Universitario Central de Asturias, 33011 Oviedo, Spain. FAU - Scuruchi, Michele AU - Scuruchi M AD - Molecular Biology Lab, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Sinicropi, Davide AU - Sinicropi D AD - Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Postorino, Maria AU - Postorino M AD - Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Morace, Carmela AU - Morace C AD - Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Giuffrida, Clemente AU - Giuffrida C AD - Medicine and Urgency Unit, Piemonte Hospital, IRCCS Neurolesi Bonino Pulejo, 98121 Messina, Italy. FAU - Sciortino, Davide AU - Sciortino D AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Gallizzi, Romina AU - Gallizzi R AD - Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood, University of Messina, 98125 Messina, Italy. AD - Pediatric Unit, Department of Medical of Health Sciences, Magna Graecia University, 88100 Catanzaro, Italy. FAU - Loddo, Saverio AU - Loddo S AD - Laboratory Medicine, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Zito, Concetta AU - Zito C AUID- ORCID: 0000-0002-6908-4316 AD - Cardiology Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. FAU - Squadrito, Giovanni AU - Squadrito G AD - Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy. LA - eng PT - Journal Article DEP - 20210511 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC8150353 OTO - NOTNLM OT - circulating progenitor cells OT - endocan OT - pulmonary hypertension OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2021/06/03 06:00 MHDA- 2021/06/03 06:01 PMCR- 2021/05/11 CRDT- 2021/06/02 01:10 PHST- 2021/04/07 00:00 [received] PHST- 2021/04/28 00:00 [revised] PHST- 2021/04/29 00:00 [accepted] PHST- 2021/06/02 01:10 [entrez] PHST- 2021/06/03 06:00 [pubmed] PHST- 2021/06/03 06:01 [medline] PHST- 2021/05/11 00:00 [pmc-release] AID - biomedicines9050533 [pii] AID - biomedicines-09-00533 [pii] AID - 10.3390/biomedicines9050533 [doi] PST - epublish SO - Biomedicines. 2021 May 11;9(5):533. doi: 10.3390/biomedicines9050533. PMID- 33476815 OWN - NLM STAT- MEDLINE DCOM- 20210302 LR - 20220531 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 20 IP - 3 DP - 2021 Mar TI - Real life picture of the use of intravenous immunoglobulins in idiopathic inflammatory myopathies: Results of a multicentric study. PG - 102757 LID - S1568-9972(21)00016-1 [pii] LID - 10.1016/j.autrev.2021.102757 [doi] AB - BACKGROUND: despite the absence of specific guidelines, the treatment with intravenous immunoglobulins (IvIg) is considered effective in patients with refractory idiopathic inflammatory myopathies (IIM). The aim of our study is to evaluate the effectiveness and the safety of IvIg and define the possible profile of IIM patients candidate to IvIg treatment. METHODS: we performed a retrospective study of IIM pts. treated with IvIg (2 g/kg/month). We collected demographic, epidemiological, laboratory and clinical data. Additionally, to evaluate the toxicity, the adverse events occurred during the treatment were collected. RESULTS: 123 patients with IIM were included in the study. The main indications for the prescription of IvIg were muscle (83.7% of patients) and esophageal involvement (45.5% of patients). IvIg were started mainly for refractory disease. At the end of treatment (mean duration 14 months), muscular necrosis enzymes decreased significantly and dysphagia VAS decreased significantly (p < 0.001), while MMT value increased (104.6 ± 24.2 vs. 127.0 ± 22.2 p < 0.001). Ninety-six pts. (78%) responded to IvIg. They had a shorter disease duration (p < 0.001), higher creatine kinase levels (p < 0.001), and higher prevalence of myalgias at the baseline (p = 0.023) compared to non-responders. The presence of Raynaud's phenomenon (p = 0.023-odds ratio 0.28 [0.11-0.72]) and skin involvement (p = 0.004, odds ratio 0.18 [0.06-0.55]), were associated to a worse response. Adverse events were mostly mild and transitory. CONCLUSIONS: Despite their high cost, IvIg confirmed their effectiveness in refractory IIM pts., particularly in muscular and esophageal manifestations. Specific clinical characteristics at the baseline may identify the patients with higher probability of response to the treatment. CI - Copyright © 2021. Published by Elsevier B.V. FAU - Barsotti, Simone AU - Barsotti S AD - Rheumatology Unit, Pisa University Hospital, Italy. Electronic address: simone.barsotti.pisa@gmail.com. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology Unit, University of Brescia, ASST SpedaliCivili Brescia, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Neri, Rossella AU - Neri R AD - Rheumatology Unit, Pisa University Hospital, Italy. FAU - Taraborelli, Mara AU - Taraborelli M AD - Internal Medicine Unit, ASST Franciacorta, Chiari Hospital, Brescia, Italy. FAU - Cioffi, Elisa AU - Cioffi E AD - Rheumatology Unit, Pisa University Hospital, Italy. FAU - Cardelli, Chiara AU - Cardelli C AD - Rheumatology Unit, Pisa University Hospital, Italy. FAU - Tripoli, Alessandra AU - Tripoli A AD - Rheumatology Unit, Pisa University Hospital, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Tincani, Angela AU - Tincani A AD - Rheumatology and Clinical Immunology Unit, University of Brescia, ASST SpedaliCivili Brescia, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology and Clinical Immunology Unit, University of Brescia, ASST SpedaliCivili Brescia, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Pisa University Hospital, Italy. LA - eng PT - Journal Article PT - Review DEP - 20210118 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Humans MH - *Immunoglobulins, Intravenous/adverse effects/therapeutic use MH - Multicenter Studies as Topic MH - *Myositis/drug therapy MH - Retrospective Studies OTO - NOTNLM OT - Immunoglobulins OT - Inflammatory myopathies OT - Myositis OT - Prognosis OT - Response OT - Treatment EDAT- 2021/01/22 06:00 MHDA- 2021/03/03 06:00 CRDT- 2021/01/21 20:11 PHST- 2020/11/21 00:00 [received] PHST- 2020/11/30 00:00 [accepted] PHST- 2021/01/22 06:00 [pubmed] PHST- 2021/03/03 06:00 [medline] PHST- 2021/01/21 20:11 [entrez] AID - S1568-9972(21)00016-1 [pii] AID - 10.1016/j.autrev.2021.102757 [doi] PST - ppublish SO - Autoimmun Rev. 2021 Mar;20(3):102757. doi: 10.1016/j.autrev.2021.102757. Epub 2021 Jan 18. PMID- 31903322 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 0253-8253 (Print) IS - 2227-0426 (Electronic) IS - 0253-8253 (Linking) VI - 2019 IP - 3 DP - 2019 TI - Characteristics of patients with systemic sclerosis living in Qatar. PG - 16 LID - 10.5339/qmj.2019.16 [doi] LID - 16 AB - Objective: The aim of this study was to determine the demographic, clinical, and immunological characteristics of patients with systemic sclerosis living in Qatar. Method: This retrospective study included 42 patients with systemic sclerosis who attended Rheumatology Clinics at Hamad General Hospital in Doha, Qatar, between January 2000 and December 2014. All patients fulfilled the 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis. Results: The 42 consecutively recruited patients of mixed ethnicities consisted of 37 (88.1%) females and 5 (11.9%) males. Of the total 42 patients, 22 (52.4%) had diffuse cutaneous systemic sclerosis (dcSSc) and 20 (47.6%) had limited cutaneous systemic sclerosis (lcSSc). Mean age at onset of first symptoms was 34.5 ± 12 years, and mean age at diagnosis was 36.1 ± 11.5 years. During follow-up, Raynaud's phenomenon occurred in 36 (85.7%) patients, sclerodactyly in 39 (92.9%) patients, digital ulcers in 16 (38.1%) patients, calcinosis in 6 (14.3%) patients, telangiectasia in 16 (38.1%) patients, and arthritis in 13 (31%) patients. The gastrointestinal and respiratory systems were the most frequently affected internal organs. Gastrointestinal involvement was present in 36 (85.7%) patients, and respiratory involvement was found in 30 (71.4%) patients. The majority of patients had positive antinuclear antibodies (ANA; 97.6%). Anti-Scl-70 antibody was found in 66.7% and anti-centromere antibody (ACA) was detected in 14.3% of the patients. Conclusion: To our knowledge, this is the first study that describes the clinical and immunological profile of patients with systemic sclerosis living in Qatar. This study cohort showed an earlier age of disease onset and diagnosis than that reported in other international studies. Furthermore, in contrast to several other studies, the diffuse type of scleroderma was more commonly observed than the limited type, which resulted in a high frequency of anti-Scl-70 antibody and interstitial lung disease. CI - © 2019 Alam, Abdulaziz, Ul Haq, Mahdy, Mohammed Siam, Chandra, Al Emadi, licensee HBKU Press. FAU - Alam, Fiaz AU - Alam F AD - Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Abdulaziz, Hani Malallah AU - Abdulaziz HM AD - Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Ul Haq, Irfan AU - Ul Haq I AD - Pulmonary Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Mahdy, Salah Mohamed AU - Mahdy SM AD - Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Mohammed Siam, Abdul Rahim AU - Mohammed Siam AR AD - Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Chandra, Prem AU - Chandra P AD - Medical Research Centre, Hamad Medical Corporation, Doha, Qatar. FAU - Al Emadi, Samar AU - Al Emadi S AD - Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. LA - eng PT - Journal Article DEP - 20191224 PL - Qatar TA - Qatar Med J JT - Qatar medical journal JID - 8101648 PMC - PMC6929649 OTO - NOTNLM OT - anti-Scl-70 antibody OT - anti-centromere antibody OT - antinuclear antibodies OT - diffuse cutaneous systemic sclerosis OT - limited cutaneous systemic sclerosis EDAT- 2020/01/07 06:00 MHDA- 2020/01/07 06:01 PMCR- 2019/12/24 CRDT- 2020/01/07 06:00 PHST- 2019/01/20 00:00 [received] PHST- 2019/07/18 00:00 [accepted] PHST- 2020/01/07 06:00 [entrez] PHST- 2020/01/07 06:00 [pubmed] PHST- 2020/01/07 06:01 [medline] PHST- 2019/12/24 00:00 [pmc-release] AID - qmj.2019.16 [pii] AID - 10.5339/qmj.2019.16 [doi] PST - epublish SO - Qatar Med J. 2019 Dec 24;2019(3):16. doi: 10.5339/qmj.2019.16. eCollection 2019. PMID- 22077618 OWN - NLM STAT- MEDLINE DCOM- 20120906 LR - 20121115 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 39 IP - 6 DP - 2012 Jun TI - Effects of sarpogrelate hydrochloride on skin ulcers and quality of life in patients with systemic sclerosis. PG - 536-40 LID - 10.1111/j.1346-8138.2011.01432.x [doi] AB - 5-Hydroxytryptamine 2A serotonin receptor (5-HT(2A) ) is associated with the contraction of vascular smooth muscle, platelet aggregation and thrombus formation and coronary artery spasms. Sarpogrelate hydrochloride (sarpogrelate) is a selective 5-HT(2A) antagonist and was supposed to be effective for Raynaud's phenomenon with collagen disease. Sarpogrelate has not been investigated regarding the effects, safety and quality of life (QOL) in patient with skin ulcers of collagen disease. Eleven patients with skin ulcers and systemic sclerosis (SSc) were administrated sarpogrelate p.o. three times a day for 3-6 months. The area (mean ± standard error) of skin ulcer at the pretreatment, and after 3 and 6 months of sarpogrelate intake was 2.1 ± 0.8, 0.2 ± 0.1 and 0.1 ± 0.1 mm(2), respectively. The reduction of skin ulcer area was significant after 3 months of sarpogrelate intake. In assessment of QOL, scores of symptoms and emotions but not of functioning were significantly improved after sarpogrelate intake. The global score (mean ± SE) of Skindex-16 at pretreatment, and after 3 and 6 months of sarpogrelate intake was 31.8 ± 8.7, 23.7 ± 8.3 and 10.9 ± 4.6, respectively. The score was significantly improved after 6 months of sarpogrelate intake. There were no obvious side-effects during this study. Sarpogrelate was considered to be a useful drug to improve skin ulcers and QOL in patients with SSc. CI - © 2011 Japanese Dermatological Association. FAU - Yoshimasu, Takashi AU - Yoshimasu T AD - Department of Dermatology, Arida Municipal Hospital, Arida, Japan. yosshii8@jg8.so-net.ne.jp FAU - Ikeda, Takaharu AU - Ikeda T FAU - Uede, Koji AU - Uede K FAU - Kanazawa, Nobuo AU - Kanazawa N FAU - Furukawa, Fukumi AU - Furukawa F LA - eng PT - Journal Article DEP - 20111114 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Serotonin 5-HT2 Receptor Antagonists) RN - 0 (Succinates) RN - 19P708E787 (sarpogrelate) SB - IM MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Quality of Life MH - Scleroderma, Diffuse/complications/drug therapy MH - Scleroderma, Limited/complications/drug therapy MH - Scleroderma, Systemic/complications/*drug therapy MH - Serotonin 5-HT2 Receptor Antagonists/therapeutic use MH - Skin Ulcer/*drug therapy/etiology/pathology MH - Succinates/*therapeutic use EDAT- 2011/11/15 06:00 MHDA- 2012/09/07 06:00 CRDT- 2011/11/15 06:00 PHST- 2011/11/15 06:00 [entrez] PHST- 2011/11/15 06:00 [pubmed] PHST- 2012/09/07 06:00 [medline] AID - 10.1111/j.1346-8138.2011.01432.x [doi] PST - ppublish SO - J Dermatol. 2012 Jun;39(6):536-40. doi: 10.1111/j.1346-8138.2011.01432.x. Epub 2011 Nov 14. PMID- 25972968 OWN - NLM STAT- MEDLINE DCOM- 20151022 LR - 20181113 IS - 1806-3756 (Electronic) IS - 1806-3713 (Print) IS - 1806-3713 (Linking) VI - 41 IP - 2 DP - 2015 Mar-Apr TI - Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features. PG - 151-60 LID - 10.1590/S1806-37132015000004443 [doi] AB - OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria. FAU - Pereira, Daniel Antunes Silva AU - Pereira DA AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Dias, Olívia Meira AU - Dias OM AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Almeida, Guilherme Eler de AU - Almeida GE AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Araujo, Mariana Sponholz AU - Araujo MS AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Kawano-Dourado, Letícia Barbosa AU - Kawano-Dourado LB AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Baldi, Bruno Guedes AU - Baldi BG AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Kairalla, Ronaldo Adib AU - Kairalla RA AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. FAU - Carvalho, Carlos Roberto Ribeiro AU - Carvalho CR AD - University of São Paulo, School of Medicine, Hospital das Clínicas, São Paulo, Brazil, Department of Cardiorespiratory Diseases, University of São Paulo School of Medicine, São Paulo, Brazil; and Director. Department of Pulmonology, Instituto do Coração - InCor, Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas, São Paulo, Brazil. LA - eng PT - Journal Article PL - Brazil TA - J Bras Pneumol JT - Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia JID - 101222274 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Antibodies, Antinuclear/analysis MH - Autoantibodies/*analysis MH - Brazil/epidemiology MH - Connective Tissue Diseases/*diagnosis/epidemiology/immunology MH - Female MH - Humans MH - Lung Diseases, Interstitial/*diagnosis/epidemiology/immunology MH - Male MH - Middle Aged MH - Prevalence MH - Respiratory Function Tests MH - Retrospective Studies MH - Spirometry MH - Tomography, X-Ray Computed PMC - PMC4428852 OTO - NOTNLM OT - Autoantibodies OT - Autoimmunity OT - Connective tissue diseases OT - Idiopathic interstitial pneumonias EDAT- 2015/05/15 06:00 MHDA- 2015/10/23 06:00 PMCR- 2015/03/01 CRDT- 2015/05/15 06:00 PHST- 2014/09/29 00:00 [received] PHST- 2014/02/27 00:00 [accepted] PHST- 2015/05/15 06:00 [entrez] PHST- 2015/05/15 06:00 [pubmed] PHST- 2015/10/23 06:00 [medline] PHST- 2015/03/01 00:00 [pmc-release] AID - 10.1590/S1806-37132015000004443 [doi] PST - ppublish SO - J Bras Pneumol. 2015 Mar-Apr;41(2):151-60. doi: 10.1590/S1806-37132015000004443. PMID- 38497858 OWN - NLM STAT- MEDLINE DCOM- 20240319 LR - 20240319 IS - 2284-0729 (Electronic) IS - 1128-3602 (Linking) VI - 28 IP - 5 DP - 2024 Mar TI - Clinical and serological characteristics of anti-Ro/SS-A and anti-La/SS-B negative primary Sjögren's syndrome: a comparative study. PG - 1760-1767 LID - 35589 [pii] LID - 10.26355/eurrev_202403_35589 [doi] AB - OBJECTIVE: This study aimed to describe the clinical spectrum of primary Sjögren's syndrome (pSS) patients with anti-Ro/SS-A and anti-La/SS-B negativity. PATIENTS AND METHODS: From a single-center study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, those with triple seronegativity anti-Ro/SS-A (anti-Sjögren's-syndrome-related antigen A autoantibody), anti-La/SS-B (anti-Sjögren's-syndrome-related antigen B autoantibody), rheumatoid factor (RF) (-) and antinuclear antibody (ANA)(+)] or [anti-Ro/SS-A(-), anti-La/ SS-B(-), RF(+) and ANA(-)] and quad¬ruple seronegativity [anti-Ro/SS-A(-), anti-La/SS-B(-), RF(-) and ANA(-)] were identified retrospectively. Clinical, serological, and laboratory features were compared. A comparison between triple and quadruple seronegative pSS patients was also performed. RESULTS: We included 184 patients (168 women, 16 men) with a mean age at diagnosis of 50.1±13.1 years. The most common subjective presenting features at the time of the diagnosis were dry mouth (94.5%) and dry eye (91.3 %). ANA positivity was 57.0%, and RF positivity was 30.4%. Salivary gland enlargement, arthritis, Raynaud's phenomenon, vasculitis, interstitial lung disease (ILD), neurological involvement, primary biliary cholangitis (PBC), lymphopenia, and thrombocytopenia were observed in ANA+ and RF+ patients but not in seronegative patients (p<0.0001). Arthritis was observed most frequently in RF-positive patients and secondly in ANA-positive patients, whereas arthritis was not observed in seronegative patients (p<0.0001). Autoimmune thyroiditis was present in 65 patients (35.0%), 84.6% of these patients were ANA positive while 12.3% were ANA negative (p=0.0014), RF positivity was 30.7% while RF negativity was 6.15% (p=0.001), 23.0% were both ANA and RF positive while 12.3% were seronegative (p<0.002). Cryoglobulinemia, renal disease, and lymphoma were not observed in any of the patients. CONCLUSIONS: We confirm the strong influence of immunological markers on the phenotype of primary SS at diagnosis. FAU - Bodakçi, E AU - Bodakçi E AD - Department of Internal Medicine, Division of Rheumatology, Eskisehir City Hospital, Eskisehir, Turkey. erdal.bodakci@saglik.gov.tr. LA - eng PT - Journal Article PL - Italy TA - Eur Rev Med Pharmacol Sci JT - European review for medical and pharmacological sciences JID - 9717360 RN - 9009-79-4 (Rheumatoid Factor) RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Male MH - Humans MH - Female MH - Adult MH - Middle Aged MH - *Sjogren's Syndrome/diagnosis MH - Retrospective Studies MH - Rheumatoid Factor MH - Antibodies, Antinuclear MH - Autoantibodies MH - *Arthritis EDAT- 2024/03/18 12:46 MHDA- 2024/03/19 06:44 CRDT- 2024/03/18 11:14 PHST- 2024/03/19 06:44 [medline] PHST- 2024/03/18 12:46 [pubmed] PHST- 2024/03/18 11:14 [entrez] AID - 35589 [pii] AID - 10.26355/eurrev_202403_35589 [doi] PST - ppublish SO - Eur Rev Med Pharmacol Sci. 2024 Mar;28(5):1760-1767. doi: 10.26355/eurrev_202403_35589. PMID- 17727302 OWN - NLM STAT- MEDLINE DCOM- 20071109 LR - 20181113 IS - 1170-229X (Print) IS - 1170-229X (Linking) VI - 24 IP - 9 DP - 2007 TI - Elderly-onset systemic lupus erythematosus: prevalence, clinical course and treatment. PG - 701-15 AB - Systemic lupus erythematosus is an autoimmune multi-system disease of uncertain aetiology with highly variable clinical manifestations. Women of child-bearing age are most often affected; however, approximately 10-20% of cases occur in older patients. Elderly-onset lupus has been defined in various studies as onset of lupus after age 50-65 years. Menopause and changes in cellular immunity with aging may contribute to development of lupus in older adults. Many studies suggest that the clinical and serological features of elderly-onset lupus differ from those of lupus in younger patients. Arthritis, fever, serositis, sicca symptoms, Raynaud's syndrome, lung disease and neuropsychiatric symptoms are more common in patients with elderly-onset lupus, while malar rash, discoid lupus and glomerulonephritis are less common in elderly-onset patients compared with younger lupus patients. Most elderly-onset lupus patients have a positive anti-nuclear antibody test, but the prevalence of anti-double-stranded DNA and hypocomplementaemia is lower in elderly-onset patients than in younger patients. Rheumatoid factor, anti-Ro/Sjögren's syndrome (SS) A and anti-La/SSB are more often positive in elderly-onset patients. The diagnosis of elderly-onset lupus may be delayed for many months: insidious onset, low prevalence and similarity to other more common disorders make the diagnosis of lupus challenging in this population. Treatment of lupus in the elderly may be complicated by co-morbidities and increased risk of toxicities from usual treatments. Optimal management of elderly-onset lupus is empiric because of a lack of randomised controlled studies. However, the approach to treatment is similar regardless of the age of the patient. This article discusses the prevalence, clinical course, serological features, prognosis and treatment of elderly-onset systemic lupus erythematosus. FAU - Lazaro, Deana AU - Lazaro D AD - Department of Medicine, Division of Rheumatology, SUNY Downstate Medical Center, Brooklyn, New York, USA. deana.lazaro@downstate.edu LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Drugs Aging JT - Drugs & aging JID - 9102074 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Analgesics) RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Antimalarials) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Analgesics/therapeutic use MH - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use MH - Antimalarials/therapeutic use MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/*drug therapy/epidemiology/physiopathology MH - Male MH - Middle Aged MH - Prevalence RF - 99 EDAT- 2007/08/31 09:00 MHDA- 2007/11/10 09:00 CRDT- 2007/08/31 09:00 PHST- 2007/08/31 09:00 [pubmed] PHST- 2007/11/10 09:00 [medline] PHST- 2007/08/31 09:00 [entrez] AID - 2491 [pii] AID - 10.2165/00002512-200724090-00001 [doi] PST - ppublish SO - Drugs Aging. 2007;24(9):701-15. doi: 10.2165/00002512-200724090-00001. PMID- 32106788 OWN - NLM STAT- MEDLINE DCOM- 20210128 LR - 20221207 IS - 1477-0962 (Electronic) IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 29 IP - 5 DP - 2020 Apr TI - Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the 'Attikon' cohort. PG - 514-522 LID - 10.1177/0961203320908932 [doi] AB - OBJECTIVE: This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at 'Attikon' University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece. METHODS: This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation. RESULTS: The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common 'classification' manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud's phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide. CONCLUSION: In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management. FAU - Nikolopoulos, D AU - Nikolopoulos D AUID- ORCID: 0000-0002-9894-6966 AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. AD - Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece. FAU - Kostopoulou, M AU - Kostopoulou M AD - Department of Nephrology, 'Georgios Gennimatas' Hospital, Athens, Greece. FAU - Pieta, A AU - Pieta A AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Karageorgas, T AU - Karageorgas T AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Tseronis, D AU - Tseronis D AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Chavatza, K AU - Chavatza K AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Flouda, S AU - Flouda S AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Rapsomaniki, P AU - Rapsomaniki P AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Banos, A AU - Banos A AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. AD - Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece. FAU - Kremasmenou, E AU - Kremasmenou E AD - 3rd Department of Internal Medicine, 'Red Cross' Hospital, Athens, Greece. FAU - Tzavara, V AU - Tzavara V AD - Laboratory of Immunology, 'Red Cross' Hospital, Athens, Greece. FAU - Katsimbri, P AU - Katsimbri P AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Fanouriakis, A AU - Fanouriakis A AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. AD - Department of Rheumatology, 'Asklepieion' General Hospital, Athens, Greece. FAU - Boumpas, D T AU - Boumpas DT AD - 4th Department of Internal Medicine, Attikon University Hospital, Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. AD - Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece. AD - Medical School, University of Cyprus, Nicosia, Cyprus. LA - eng PT - Journal Article DEP - 20200227 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adult MH - Comorbidity MH - Female MH - Greece/epidemiology MH - Humans MH - Incidence MH - Lupus Erythematosus, Systemic/*classification/*epidemiology MH - Lupus Nephritis/*epidemiology MH - Lupus Vasculitis, Central Nervous System/*epidemiology MH - Male MH - Middle Aged MH - Phenotype MH - Retrospective Studies MH - Rheumatology/*standards MH - Severity of Illness Index MH - White People MH - Young Adult PMC - PMC7168806 OTO - NOTNLM OT - Prevalent cohort OT - damage OT - incident cohort OT - lupus criteria OT - non-lupus criteria EDAT- 2020/02/29 06:00 MHDA- 2021/01/29 06:00 PMCR- 2020/04/20 CRDT- 2020/02/29 06:00 PHST- 2020/02/29 06:00 [pubmed] PHST- 2021/01/29 06:00 [medline] PHST- 2020/02/29 06:00 [entrez] PHST- 2020/04/20 00:00 [pmc-release] AID - 10.1177_0961203320908932 [pii] AID - 10.1177/0961203320908932 [doi] PST - ppublish SO - Lupus. 2020 Apr;29(5):514-522. doi: 10.1177/0961203320908932. Epub 2020 Feb 27. PMID- 37597472 OWN - NLM STAT- MEDLINE DCOM- 20230824 LR - 20231212 IS - 1980-5322 (Electronic) IS - 1807-5932 (Print) IS - 1807-5932 (Linking) VI - 78 DP - 2023 TI - Screening tool development for hand surgery referrals in systemic sclerosis. PG - 100270 LID - S1807-5932(23)00106-0 [pii] LID - 10.1016/j.clinsp.2023.100270 [doi] LID - 100270 AB - BACKGROUND: Systemic Sclerosis (SSc) patients may need hand surgery. OBJECTIVE: To develop a screening tool for rheumatologists to identify potential candidates with systemic sclerosis for hand surgery, optimizing referrals. METHODS: A pilot cross-sectional study from January 2015 to December 2016. SAMPLE SIZE: 51 participants. INCLUSION CRITERIA: ≥ 18 years old, meeting the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc and hand impairment. DATA COLLECTED: age, sex, race, disease duration, SSc subtypes, vasodilator use, skin thickness, finger stiffness, presence of Digital Ulcers (DU) and/or calcinosis, presence of Raynaud's Phenomenon (RP) attacks, health status and disability, disease status, pain intensity and functional status of the hands. Data were analyzed by a multivariate logistic regression model. RESULTS: Fulfillment of surgical criteria: 68.8%. The surgical group had higher scores on the HAQ-DI (1.39 vs. 0.96, p = 0.032) and CHFS (25.0 vs. 12.0, p = 0.005) questionnaires, and a higher frequency of DU (91.43% vs. 18.75%, p < 0.0010), calcinosis (60.0% vs. 0.0%, p < 0.001), use of vasodilators (100.0% vs. 75.0%, p = 0.007) and digital stiffness (28.57% vs. 0.0%, p = 0.017). The presence of DU increased the chance of surgical indication by 46.2 times (ORIC 95% = 8.23 to 259.49). The statistical model showed good accuracy (86.3%, p < 0.001), sensitivity (91.4%), and specificity (81.2%). CONCLUSION: The presence of DU in SSc could be used as a screening feature for early identification and referral of potential candidates for hand surgery. CI - Copyright © 2023. Published by Elsevier España, S.L.U. FAU - Marcatto de Abreu, Marcos Felipe AU - Marcatto de Abreu MF AD - Department of Orthopedics, Rheumatology and Traumatology, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, Brazil. Electronic address: marcosmarcatto@hc.unicamp.br. FAU - Landim, Síbila AU - Landim S AD - Occupational Therapy School, Faculty of Psychology, Universidad de Talca, Talca, Chile. FAU - Yuamoto, Fernanda Yuri AU - Yuamoto FY AD - Department of Orthopedics, Rheumatology and Traumatology, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, Brazil. FAU - Lins, Carolina AU - Lins C AD - Department of Orthopedics, Rheumatology and Traumatology, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, Brazil. FAU - Magalhães, Eduardo Paiva AU - Magalhães EP AD - Department of Orthopedics, Rheumatology and Traumatology, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, Brazil. FAU - Etchebehere, Maurício AU - Etchebehere M AD - Department of Orthopedics, Rheumatology and Traumatology, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, São Paulo, Brazil. LA - eng PT - Journal Article DEP - 20230817 PL - United States TA - Clinics (Sao Paulo) JT - Clinics (Sao Paulo, Brazil) JID - 101244734 RN - digital ulcers SB - IM MH - Cross-Sectional Studies MH - Adolescent MH - *Scleroderma, Systemic/diagnosis MH - Skin Ulcer MH - Humans MH - Hand/surgery MH - Referral and Consultation MH - *Calcinosis PMC - PMC10460938 OTO - NOTNLM OT - Cross-sectional studies OT - Orthopedic procedures OT - Referral and consultation OT - Scleroderma, systemic OT - Skin ulcer COIS- Conflicts of interest The authors M.F. Marcatto de Abreu, S. Landin, F.Y. Yuamoto, C. Lins, E. Paiva Magalhães, and M. Etchebehere declare that they have no conflict of interest. EDAT- 2023/08/20 00:41 MHDA- 2023/08/24 06:42 PMCR- 2023/08/17 CRDT- 2023/08/19 18:09 PHST- 2023/02/01 00:00 [received] PHST- 2023/07/03 00:00 [revised] PHST- 2023/07/26 00:00 [accepted] PHST- 2023/08/24 06:42 [medline] PHST- 2023/08/20 00:41 [pubmed] PHST- 2023/08/19 18:09 [entrez] PHST- 2023/08/17 00:00 [pmc-release] AID - S1807-5932(23)00106-0 [pii] AID - 100270 [pii] AID - 10.1016/j.clinsp.2023.100270 [doi] PST - epublish SO - Clinics (Sao Paulo). 2023 Aug 17;78:100270. doi: 10.1016/j.clinsp.2023.100270. eCollection 2023. PMID- 33573268 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210214 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 10 IP - 3 DP - 2021 Jan 30 TI - Implication of miR-126 and miR-139-5p in Plasmacytoid Dendritic Cell Dysregulation in Systemic Sclerosis. LID - 10.3390/jcm10030491 [doi] LID - 491 AB - Compelling evidence shows the involvement of plasmacytoid dendritic cells (pDCs) in systemic sclerosis (SSc) pathogenesis. This study investigated whether microRNAs (miRNAs) are involved in the dysregulation of pDCs in SSc patients already at early stages. RNA from circulating pDCs was isolated from two independent cohorts of SSc patients with different disease phenotypes, and individuals with Raynaud's phenomenon, for microRNA profiling and RNA-sequencing analysis. Proteomic analysis was exploited to identify novel direct miRNA targets at the protein level. Twelve and fifteen miRNAs were differentially expressed in at least one group of patients compared to healthy controls in discovery cohort I and II, respectively. Of note, miR-126 and miR-139-5p were upregulated in both preclinical and definite SSc patients and correlated with the expression of type I interferon (IFN)-responsive genes. Toll-like receptor 9 (TLR9) stimulation of healthy pDCs upregulated the expression of both miRNAs, similarly to what was observed in patients. The proteomic analysis identified USP24 as a novel target of miR-139-5p. The expression level of USP24 was inversely correlated with miR-139-5p expression in SSc patients and induced by TLR9 stimulation in healthy pDCs. These findings demonstrated that the miRNA profile is altered in pDCs of SSc patients already at early stages of the disease and indicate their potential contribution to pDC activation observed in patients. FAU - Chouri, Eleni AU - Chouri E AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Wang, Maojie AU - Wang M AD - Department of Molecular Cancer Research, Center Molecular Medicine, Oncode Institute, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Hillen, Maarten R AU - Hillen MR AUID- ORCID: 0000-0002-9505-3468 AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Angiolilli, Chiara AU - Angiolilli C AUID- ORCID: 0000-0003-1404-4429 AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Silva-Cardoso, Sandra C AU - Silva-Cardoso SC AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Wichers, Catharina G K AU - Wichers CGK AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - van der Kroef, Maarten AU - van der Kroef M AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Bekker, Cornelis P J AU - Bekker CPJ AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Cossu, Marta AU - Cossu M AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - van Bon, Lenny AU - van Bon L AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Affandi, Alsya J AU - Affandi AJ AUID- ORCID: 0000-0003-0859-2322 AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Carvalheiro, Tiago AU - Carvalheiro T AUID- ORCID: 0000-0002-3187-2288 AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Pandit, Aridaman AU - Pandit A AUID- ORCID: 0000-0003-2057-9737 AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - van Roon, Joel A G AU - van Roon JAG AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Beretta, Lorenzo AU - Beretta L AUID- ORCID: 0000-0002-6529-6258 AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, 20122 Milan, Italy. FAU - Burgering, Boudewijn M T AU - Burgering BMT AUID- ORCID: 0000-0002-4044-9596 AD - Department of Molecular Cancer Research, Center Molecular Medicine, Oncode Institute, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Radstake, Timothy R D J AU - Radstake TRDJ AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. FAU - Rossato, Marzia AU - Rossato M AD - Center of Translational Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, 3584 CX Utrecht, The Netherlands. AD - Department of Biotechnology, University of Verona, 37134 Verona, Italy. LA - eng GR - ERC-2011-StG, Circumvent/ERC_/European Research Council/International GR - 622811, MicroSCAP/MCCC_/Marie Curie/United Kingdom PT - Journal Article DEP - 20210130 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC7866506 OTO - NOTNLM OT - microRNAs (miRNAs) OT - plasmacytoid dendritic cells OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2021/02/13 06:00 MHDA- 2021/02/13 06:01 PMCR- 2021/01/30 CRDT- 2021/02/12 01:04 PHST- 2020/12/09 00:00 [received] PHST- 2021/01/19 00:00 [revised] PHST- 2021/01/26 00:00 [accepted] PHST- 2021/02/12 01:04 [entrez] PHST- 2021/02/13 06:00 [pubmed] PHST- 2021/02/13 06:01 [medline] PHST- 2021/01/30 00:00 [pmc-release] AID - jcm10030491 [pii] AID - jcm-10-00491 [pii] AID - 10.3390/jcm10030491 [doi] PST - epublish SO - J Clin Med. 2021 Jan 30;10(3):491. doi: 10.3390/jcm10030491. PMID- 24755203 OWN - NLM STAT- MEDLINE DCOM- 20150908 LR - 20181202 IS - 1876-4738 (Electronic) IS - 0914-5087 (Linking) VI - 64 IP - 6 DP - 2014 Dec TI - Arterial stiffness, antiphospholipid antibodies, and pulmonary arterial hypertension in systemic lupus erythematosus. PG - 450-5 LID - S0914-5087(14)00096-3 [pii] LID - 10.1016/j.jjcc.2014.02.030 [doi] AB - BACKGROUND: The aim of this study is to evaluate the role of arterial stiffness in pulmonary arterial hypertension (PAH) in systemic lupus erythematosus (SLE) patients and its relationship with antiphospholipid antibody (aPL). METHODS: Measurement of brachial ankle pulse wave velocity (baPWV), carotid arterial stiffness, and pulmonary vascular resistance (PVR) was performed in 51 patients with SLE. PAH was diagnosed if the pulmonary artery systolic pressure was >40mmHg. Information concerning SLE duration, medication, and serum autoantibodies was recorded. SLE activity was assessed by the SLE disease activity index (SLEDAI). RESULTS: aPL was present in 10 patients (20%), and PAH was detected in 6 patients (12%). The prevalence of Raynaud's phenomenon, baPWV, positive aPL, and titers of IgG anticardiolipin antibody (aCL) were increased in SLE with PAH; however, no difference was found in inflammatory markers, disease duration, and SLEDAI compared to SLE without PAH. Carotid artery deformation and right ventricular function were reduced in patients with PAH (all p<0.05). Carotid artery circumferential strain (r=0.34, p=0.021), radial strain (r=-0.30, p=0.045), and baPWV (r=0.46, p=0.001) showed significant correlation between IgG aCL. Univariate and multiple regression analysis revealed that the only significant independent predictors of the presence of PAH were baPWV, carotid artery stiffness, and IgG aCL. CONCLUSION: Arterial stiffness might contribute to the pathogenesis of PAH related to SLE as well as aPLs. Furthermore, the significant association of aPL with arterial stiffness suggests its important role in PAH with SLE. CI - Copyright © 2014 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. FAU - Lee, Ji Hyun AU - Lee JH AD - Division of Rheumatology, Department of Internal Medicine, Maryknoll Medical Center, Busan, Republic of Korea. FAU - Im Cho, Kyoung AU - Im Cho K AD - Division of Cardiology, Department of Internal Medicine, Kosin University School of Medicine, Busan, Republic of Korea. Electronic address: kyoungim74@gmail.com. LA - eng PT - Journal Article DEP - 20140420 PL - Netherlands TA - J Cardiol JT - Journal of cardiology JID - 8804703 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Ankle Brachial Index MH - Antibodies, Antiphospholipid/blood MH - Blood Flow Velocity MH - Brachial Artery/*physiopathology MH - Female MH - Humans MH - Hypertension, Pulmonary/physiopathology MH - Lupus Erythematosus, Systemic/blood/diagnostic imaging/*physiopathology MH - Male MH - Middle Aged MH - Pulsatile Flow MH - Tibial Arteries/*physiopathology MH - Ultrasonography MH - Vascular Stiffness OTO - NOTNLM OT - Arterial stiffness OT - Pulmonary hypertension OT - Systemic lupus erythematosus EDAT- 2014/04/24 06:00 MHDA- 2015/09/09 06:00 CRDT- 2014/04/24 06:00 PHST- 2013/08/10 00:00 [received] PHST- 2014/02/03 00:00 [revised] PHST- 2014/02/20 00:00 [accepted] PHST- 2014/04/24 06:00 [entrez] PHST- 2014/04/24 06:00 [pubmed] PHST- 2015/09/09 06:00 [medline] AID - S0914-5087(14)00096-3 [pii] AID - 10.1016/j.jjcc.2014.02.030 [doi] PST - ppublish SO - J Cardiol. 2014 Dec;64(6):450-5. doi: 10.1016/j.jjcc.2014.02.030. Epub 2014 Apr 20. PMID- 16228107 OWN - NLM STAT- MEDLINE DCOM- 20060818 LR - 20181113 IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 25 IP - 2 DP - 2006 Mar TI - A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. PG - 205-12 AB - Current therapeutic modalities for the treatment of systemic sclerosis (SSc) have significant limitations. The window of opportunity to prevent tissue fibrosis and irreversible damage occurs during the early inflammatory phase of this condition. A drug that we believe has promise to exert a desired effect in early SSc is cyclophosphamide (CYC). However, there are only a few published reports regarding the use of cytotoxic immunosuppressive medications at the onset of the illness. The goal of this study was to test the efficacy and toxicity of CYC in patients with early diffuse SSc. The study design was a randomized, unblinded, 18 months per patient trial with a comparison group that received azathioprine (AZ). Thirty patients were assigned to receive oral CYC (2 mg/kg daily for 12 months and then maintained on 1 mg/kg daily) and 30 patients were assigned to receive oral AZ (2.5 mg/kg daily for 12 months and then maintained on 2 mg/kg daily). During first 6 months of the trial, the patients also received prednisolone, which was started at a dosage of 15 mg daily and tapered to zero by the end of the sixth month. After treatment there was a statistically significant improvement in the modified Rodnan skin score (MRSS), attack frequency of Raynaud's phenomenon (RP), and erythrocyte sedimentation rate (ESR) in the CYC-group, but not in the AZ-group. The forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) did not change after treatment in the CYC-group, but statistically significantly worsened in the AZ-group. No life-threatening or irreversible adverse reactions were observed in either group. This study showed that CYC is a promising disease modifying medication for SSc as it exhibited a positive influence on the evolution of disease. FAU - Nadashkevich, O AU - Nadashkevich O AD - Department of Internal Medicine/Rheumatology, L'viv Medical University, L'viv, Ukraine. FAU - Davis, P AU - Davis P FAU - Fritzler, M AU - Fritzler M FAU - Kovalenko, W AU - Kovalenko W LA - eng PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial DEP - 20051014 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - 9PHQ9Y1OLM (Prednisolone) RN - MRK240IY2L (Azathioprine) SB - IM MH - Administration, Oral MH - Adult MH - Aged MH - Azathioprine/administration & dosage/*therapeutic use MH - Cyclophosphamide/administration & dosage/*therapeutic use MH - Female MH - Humans MH - Immunosuppressive Agents/administration & dosage/*therapeutic use MH - Male MH - Middle Aged MH - Prednisolone/administration & dosage MH - Scleroderma, Systemic/*drug therapy/physiopathology EDAT- 2005/10/18 09:00 MHDA- 2006/08/19 09:00 CRDT- 2005/10/18 09:00 PHST- 2004/09/16 00:00 [received] PHST- 2005/04/20 00:00 [accepted] PHST- 2005/04/20 00:00 [revised] PHST- 2005/10/18 09:00 [pubmed] PHST- 2006/08/19 09:00 [medline] PHST- 2005/10/18 09:00 [entrez] AID - 10.1007/s10067-005-1157-y [doi] PST - ppublish SO - Clin Rheumatol. 2006 Mar;25(2):205-12. doi: 10.1007/s10067-005-1157-y. Epub 2005 Oct 14. PMID- 34105109 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240402 IS - 2198-6576 (Print) IS - 2198-6584 (Electronic) IS - 2198-6576 (Linking) VI - 8 IP - 2 DP - 2021 Jun TI - Primary Sjögren's Syndrome: A Retrospective Cohort Study of Burden of Illness in Sweden. PG - 955-971 LID - 10.1007/s40744-021-00314-y [doi] AB - INTRODUCTION: Primary Sjögren's syndrome (pSS) is an autoimmune disease that affects salivary and lachrymal glands and is associated with complex extraglandular manifestations. This study investigates the clinical and economic burden and disease course of pSS in Sweden. METHODS: This retrospective cohort study utilizes data from Swedish national registries and consists of patients at least 18 years of age diagnosed in secondary care with pSS, and matched members of the Swedish general population. Healthcare resource utilization (HRU) and costs were compared. The clinical burden of pSS during follow-up was explored via year-on-year prevalence of and time-to-first extraglandular manifestation. Employment status and retirement rates were used to investigate the impact of pSS on productivity. RESULTS: A total of 8884 patients with pSS and 88,233 general population comparators were included. Patients with pSS had significantly higher rates of HRU and higher healthcare costs than matched comparators, including twice as many outpatient visits. Costs were highest in year 1 post index before reducing in years 2 and 3 and stabilizing thereafter. Almost two-thirds of patients received their index diagnosis during an outpatient visit, and < 30% of diagnoses were from rheumatology departments. Overall, 41% of patients experienced a healthcare encounter that included a relevant extraglandular manifestation code during follow-up. Patients with pSS had significantly higher odds of early retirement than the general population at 5 years post index. CONCLUSIONS: Patients with pSS experience a high clinical and economic disease burden in Sweden. FAU - Westerlund, Anna AU - Westerlund A AD - Global Database Studies, IQVIA, Stockholm, Sweden. FAU - Kejs, Anne Mette Tranberg AU - Kejs AMT AD - Global Database Studies, IQVIA, Copenhagen, Denmark. FAU - Beydogan, Heval AU - Beydogan H AD - Global Database Studies, IQVIA, Stockholm, Sweden. AD - The National Board of Health and Welfare, Stockholm, Sweden. FAU - Gairy, Kerry AU - Gairy K AUID- ORCID: 0000-0001-8591-1396 AD - GlaxoSmithKline, Value Evidence and Outcomes, Brentford, Middlesex, UK. kerry.x.gairy@gsk.com. LA - eng PT - Journal Article DEP - 20210609 PL - England TA - Rheumatol Ther JT - Rheumatology and therapy JID - 101674543 PMC - PMC8217380 OAB - Primary Sjögren’s syndrome (pSS) is an autoimmune disease that typically affects the secretory glands that produce tears, saliva, and other secretions. Patients can experience debilitating fatigue and can also develop conditions in other parts of the body, commonly including arthritis and Raynaud’s phenomenon. This study investigated the burden and disease course of pSS in Sweden. Swedish national registry data were used to match adult patients diagnosed with pSS, with members of the Swedish general population. The use of healthcare resources and their associated costs were compared. The clinical burden of pSS during patient follow-up was explored via year-on-year occurrence and cumulative incidence of extraglandular symptoms. Employment and retirement rates were used to investigate the effect of pSS on patient productivity. A total of 8884 patients with pSS and 88,233 members of the general population were included in the study. Patients with pSS had higher use of healthcare resources and higher associated costs than members of the general population, including twice as many outpatient visits. Costs were highest in the first year after diagnosis, reducing and stabilizing over the following 7 years. Overall, 41% of patients had a healthcare encounter that included a relevant extraglandular symptom code during follow-up. Almost two-thirds of patients were diagnosed during an outpatient visit, and < 30% of diagnoses were from rheumatology departments. Patients with pSS were at a greater risk of early retirement than the general population at 5 years post-diagnosis. These results indicate that patients with pSS experience a high disease burden in Sweden. OABL- eng OTO - NOTNLM OT - Burden of illness OT - Healthcare cost/productivity OT - Healthcare resource utilization OT - Primary Sjögren’s syndrome OT - Treatment pathways EDAT- 2021/06/10 06:00 MHDA- 2021/06/10 06:01 PMCR- 2021/06/09 CRDT- 2021/06/09 06:59 PHST- 2020/12/22 00:00 [received] PHST- 2021/04/22 00:00 [accepted] PHST- 2021/06/10 06:00 [pubmed] PHST- 2021/06/10 06:01 [medline] PHST- 2021/06/09 06:59 [entrez] PHST- 2021/06/09 00:00 [pmc-release] AID - 10.1007/s40744-021-00314-y [pii] AID - 314 [pii] AID - 10.1007/s40744-021-00314-y [doi] PST - ppublish SO - Rheumatol Ther. 2021 Jun;8(2):955-971. doi: 10.1007/s40744-021-00314-y. Epub 2021 Jun 9. PMID- 23370858 OWN - NLM STAT- MEDLINE DCOM- 20140306 LR - 20220317 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 33 IP - 8 DP - 2013 Aug TI - Sleep quality in patients with systemic sclerosis: relationship between the clinical variables, depressive symptoms, functional status, and the quality of life. PG - 1973-9 LID - 10.1007/s00296-013-2680-9 [doi] AB - The aim of this study is to evaluate the relationship between the sleep quality and the disease-related variables, functional status, quality of life, and depressive symptoms in patients with systemic sclerosis (SSc). Forty-eight patients diagnosed with SSc and 42 healthy control subjects were enrolled in the study. The demographic and clinical characteristics of the patients such as the Raynaud's phenomenon, SSc subtype, digital ulcers, gastrointestinal and lung involvement, and disease activity were recorded. All patients were assessed using the short form 36 (SF-36) quality of life scale, the health assessment questionnaire and the beck depression inventory. Generalized pain and fatigue were assessed with the Visual Analoge Scale. For the evaluation of the sleep disturbance, the SSc and control groups were assessed with the help of the Pittsburgh Sleep Quality Index (PSQI). The patients with SSc had significantly higher scores in the subjective sleep quality, sleep latency, habitual sleep efficiency, sleep disturbance, daytime dysfunction domains, and in terms of the total PSQI score compared to the healthy control group (p < 0.05). According to the results of spearman's analysis, there was a significantly higher correlation between the generalised pain, fatigue, depressive symptoms, functional status, and physical score of the SF-36 and the sleep disturbance (p < 0.01). There was also a significantly lower correlation between the menopause status, dyspnoea, gastroesophageal reflux, dysphagia, the mental score of the SF-36, and the sleep disturbance (p < 0.05). The sleep quality is disturbed in patients with SSc. The lower quality of sleep is especially associated with the pain, fatigue, depressive symptoms, and functional status. FAU - Sariyildiz, Mustafa Akif AU - Sariyildiz MA AD - Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Dicle University, Diyarbakır, Turkey. makifsariyildiz@hotmail.com FAU - Batmaz, Ibrahim AU - Batmaz I FAU - Budulgan, Mahmut AU - Budulgan M FAU - Bozkurt, Mehtap AU - Bozkurt M FAU - Yazmalar, Levent AU - Yazmalar L FAU - Inanir, Ahmet AU - Inanir A FAU - Celepkolu, Tahsin AU - Celepkolu T FAU - Çevik, Remzi AU - Çevik R LA - eng PT - Journal Article DEP - 20130131 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Depression/*psychology MH - Disability Evaluation MH - Fatigue/complications/psychology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain/complications/psychology MH - Quality of Life/*psychology MH - Scleroderma, Systemic/*complications/psychology MH - Severity of Illness Index MH - Sleep/*physiology MH - Sleep Wake Disorders/*complications/diagnosis/psychology EDAT- 2013/02/02 06:00 MHDA- 2014/03/07 06:00 CRDT- 2013/02/02 06:00 PHST- 2012/06/04 00:00 [received] PHST- 2013/01/19 00:00 [accepted] PHST- 2013/02/02 06:00 [entrez] PHST- 2013/02/02 06:00 [pubmed] PHST- 2014/03/07 06:00 [medline] AID - 10.1007/s00296-013-2680-9 [doi] PST - ppublish SO - Rheumatol Int. 2013 Aug;33(8):1973-9. doi: 10.1007/s00296-013-2680-9. Epub 2013 Jan 31. PMID- 22192662 OWN - NLM STAT- MEDLINE DCOM- 20120207 LR - 20151119 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 59 IP - 1 DP - 2012 Jan 3 TI - Cardiac uses of phosphodiesterase-5 inhibitors. PG - 9-15 LID - 10.1016/j.jacc.2011.07.051 [doi] AB - Phosphodiesterase-5 inhibitors (PDE5Is) improve erectile function by enhancing nitric oxide availability in the penis and its supplying vasculature, resulting in vasodilation and increased blood flow. PDE5Is might benefit cardiovascular diseases because phosphodiesterase-5 is also located elsewhere in the body, including the pulmonary and systemic vasculature and in hypertrophied myocardium. PDE5Is are approved for pulmonary arterial hypertension, given that they improved several hemodynamic and clinical parameters in large randomized trials. Initial evidence suggests that PDE5Is benefit patients with congestive heart failure and secondary pulmonary hypertension. PDE5Is seem to improve hemodynamic and clinical parameters in patients with high-altitude pulmonary edema (HAPE) and high-altitude pulmonary hypertension. In climbers with prior episodes of HAPE, PDE5Is prevented HAPE in 2 small randomized trials. In small randomized trials of PDE5Is, patients with Raynaud's phenomenon demonstrated improved blood flow, fewer symptoms and frequency of attacks, and resolution of digital ulcers. In addition to enhancing vasodilation, PDE5Is seem to protect the myocardium through complex pathways that involve nitric oxide, cyclic guanosine monophosphate, protein kinase G, extracellular-signal-regulated kinase, B-cell lymphoma protein-2, and Rho kinase inhibition. In animal models of acute myocardial infarction, PDE5Is consistently reduced infarct size indicating cardioprotection and PDE5Is also promote reverse remodeling and reduce myocardial apoptosis, fibrosis, and hypertrophy. PDE5Is might also benefit patients with treatment-resistant hypertension, preeclampsia, or peripheral arterial disease. This review presents the pathophysiology and trial data with regard to the use of PDE5Is for cardiac diseases. CI - Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. FAU - Schwartz, Bryan G AU - Schwartz BG AD - Heart Institute, Good Samaritan Hospital, 1225 Wilshire Boulevard, Los Angeles, CA 90017-2395, USA. rkloner@goodsam.org FAU - Levine, Laurence A AU - Levine LA FAU - Comstock, Gary AU - Comstock G FAU - Stecher, Vera J AU - Stecher VJ FAU - Kloner, Robert A AU - Kloner RA LA - eng PT - Journal Article PT - Review PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM MH - Animals MH - Coronary Circulation/drug effects MH - Cross-Over Studies MH - Disease Models, Animal MH - Female MH - Heart Failure/diagnosis/*drug therapy/mortality MH - Hemodynamics/drug effects MH - Humans MH - Hypertension, Pulmonary/diagnosis/*drug therapy/mortality MH - Male MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Piperazines/*therapeutic use MH - Prognosis MH - Purines/therapeutic use MH - Randomized Controlled Trials as Topic MH - Risk Assessment MH - Severity of Illness Index MH - Sildenafil Citrate MH - Sulfones/*therapeutic use MH - Survival Analysis MH - Treatment Outcome EDAT- 2011/12/24 06:00 MHDA- 2012/02/09 06:00 CRDT- 2011/12/24 06:00 PHST- 2011/03/02 00:00 [received] PHST- 2011/06/28 00:00 [revised] PHST- 2011/07/25 00:00 [accepted] PHST- 2011/12/24 06:00 [entrez] PHST- 2011/12/24 06:00 [pubmed] PHST- 2012/02/09 06:00 [medline] AID - S0735-1097(11)04527-X [pii] AID - 10.1016/j.jacc.2011.07.051 [doi] PST - ppublish SO - J Am Coll Cardiol. 2012 Jan 3;59(1):9-15. doi: 10.1016/j.jacc.2011.07.051. PMID- 17376860 OWN - NLM STAT- MEDLINE DCOM- 20070614 LR - 20181113 IS - 1556-6811 (Print) IS - 1556-679X (Electronic) IS - 1556-679X (Linking) VI - 14 IP - 5 DP - 2007 May TI - Clinical value of multiplexed bead-based immunoassays for detection of autoantibodies to nuclear antigens. PG - 505-9 AB - The advent of multiplexed bead assays in recent years has introduced a new dimension of testing for complex diseases such as lupus, which can involve multiple autoantibodies. The ability to rapidly identify multiple autoantibodies, with high sensitivity and specificity in an automated fashion, is highly attractive. The aim of this study was to assess the performance and clinical value of multiplexed bead-based (AtheNA Multi-Lyte ANA-II test system) immunoassays both by comparing the results with those achieved by indirect fluorescent-antibody assay (IFA) or conventional enzyme immunoassays (EIAs) and by independent identification of autoantibodies in well-characterized samples. To achieve this goal, 984 samples were tested for seven analytes (SS/A, SS/B, Sm, RNP, Scl-70, double-stranded DNA [dsDNA], and centromere B) in both traditional and bead-based assays. The average concordance for the different analytes was 91%, ranging from 81% (dsDNA) to 97% (centromere B). The average relative specificity and sensitivity for the analytes were also high, 92% and 81%, respectively. An examination of 93 "normal controls" demonstrated a 7% false-positive rate, which was comparable to IFA. Percentages of different autoantibodies found in patients with a variety of disease conditions (34 with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia; 41 with mixed connective tissue disease; 24 with scleroderma; and 35 with Sjogren's syndrome) were well within the range expected from each group. A scrutiny of results from AtheNA and EIA and Farr results for 185 systemic lupus erythematosus samples revealed comparable results by both methods, with the exception of SS/A and dsDNA, where AtheNA had a higher percentage of SS/A-positive results compared to EIA (51% versus 29%) and a lower percentage of dsDNA-positive results (18% versus 28% at a cutoff of 5 IU/ml). FAU - Avaniss-Aghajani, Erik AU - Avaniss-Aghajani E AD - Primex Clinical Laboratories, 16742 Stagg Street #120, Van Nuys, CA 91406, USA. erik@primexlab.com FAU - Berzon, Sophia AU - Berzon S FAU - Sarkissian, Arlen AU - Sarkissian A LA - eng PT - Clinical Trial PT - Comparative Study PT - Journal Article DEP - 20070321 PL - United States TA - Clin Vaccine Immunol JT - Clinical and vaccine immunology : CVI JID - 101252125 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) SB - IM MH - Antibodies, Antinuclear/*immunology MH - Antigens, Nuclear/*immunology MH - Autoantibodies/*immunology MH - Case-Control Studies MH - Fluorescent Antibody Technique, Indirect/methods MH - Humans MH - Immune System Diseases/*diagnosis/immunology MH - Immunoenzyme Techniques/methods MH - Sensitivity and Specificity MH - Vasculitis/*immunology PMC - PMC1865627 EDAT- 2007/03/23 09:00 MHDA- 2007/06/15 09:00 PMCR- 2007/09/01 CRDT- 2007/03/23 09:00 PHST- 2007/03/23 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/03/23 09:00 [entrez] PHST- 2007/09/01 00:00 [pmc-release] AID - CVI.00034-07 [pii] AID - 0034-07 [pii] AID - 10.1128/CVI.00034-07 [doi] PST - ppublish SO - Clin Vaccine Immunol. 2007 May;14(5):505-9. doi: 10.1128/CVI.00034-07. Epub 2007 Mar 21. PMID- 28954092 OWN - NLM STAT- MEDLINE DCOM- 20171010 LR - 20220310 IS - 1806-4841 (Electronic) IS - 0365-0596 (Print) IS - 0365-0596 (Linking) VI - 92 IP - 4 DP - 2017 Jul-Aug TI - Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus. PG - 466-469 LID - S0365-05962017000400466 [pii] LID - 10.1590/abd1806-4841.20175476 [doi] AB - BACKGROUND: The clinical significance of anti-neutrophil cytoplasmic antibodies in patients with new-onset systemic lupus erythematosus, especially in systemic disease accompanied by interstitial lung disease remains to be elucidated. OBJECTIVES: This study was designed to investigate the role of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients. METHODS: A hundred and seven patients with new-onset SLE were enrolled. Presence of anti-neutrophil cytoplasmic antibodies in the sera was assessed by indirect immunofluorescence as well as enzyme linked immunosorbent assay against proteinase-3 and myeloperoxidase. Clinical features and laboratory parameters of patients were also recorded. All patients were subjected to chest X-ray, chest high-resolution computed tomography and pulmonary function test. RESULTS: Forty-five systemic lupus erythematosus patients (45/107, 42%) were seropositive for anti-neutrophil cytoplasmic antibodies. Compared with anti-neutrophil cytoplasmic antibodies-negative patients, the anti-neutrophil cytoplasmic antibodies-positive patients had significantly higher incidence of renal involvement, anemia, and Raynaud's phenomenon as well as decreased serum level of complement 3/complement 4 and elevated erythrocyte sedimentation rate. In addition, there was a positive correlation between serum anti-neutrophil cytoplasmic antibodies level and disease activity of systemic lupus erythematosus. Furthermore, prevalence of interstitial lung disease in the anti-neutrophil cytoplasmic antibodies -positive patients (25/45, 55.6%) was obviously higher than that in the anti-neutrophil cytoplasmic antibodies-negative patients (15/62, 24.2%). STUDY LIMITATIONS: The sample size was limited and the criteria for screening new-onset systemic lupus erythematosus patients might produce bias. CONCLUSIONS: The level of anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus patients correlates positively with the disease activity and the prevalence of interstitial lung disease. FAU - Su, Fang AU - Su F AD - Department of Dermatology, the Seventh People's Hospital of Shenyang - Shenyang, China. FAU - Xiao, Weiguo AU - Xiao W AD - Department of Rheumatology and Immunology, the First Affiliated Hospital of China Medical University - Shenyang, China. FAU - Yang, Pingting AU - Yang P AD - Department of Rheumatology and Immunology, the First Affiliated Hospital of China Medical University - Shenyang, China. FAU - Chen, Qingyan AU - Chen Q AD - Department of Dermatology, the Seventh People's Hospital of Shenyang - Shenyang, China. FAU - Sun, Xiaojie AU - Sun X AD - Department of Dermatology, the Seventh People's Hospital of Shenyang - Shenyang, China. FAU - Li, Tienan AU - Li T AD - Department of Dermatology, the Seventh People's Hospital of Shenyang - Shenyang, China. LA - eng PT - Journal Article PL - Spain TA - An Bras Dermatol JT - Anais brasileiros de dermatologia JID - 0067662 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) SB - IM MH - Adolescent MH - Adult MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications/*immunology MH - Antibodies, Antineutrophil Cytoplasmic/*blood MH - Cross-Sectional Studies MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fluorescent Antibody Technique, Indirect MH - Humans MH - Lung Diseases, Interstitial/etiology/*immunology MH - Lupus Erythematosus, Systemic/diagnostic imaging/*immunology MH - Male MH - Middle Aged MH - Neutrophils/enzymology MH - Prognosis MH - Tomography, X-Ray Computed/methods MH - Young Adult PMC - PMC5595590 COIS- Conflict of interest: None EDAT- 2017/09/28 06:00 MHDA- 2017/10/11 06:00 PMCR- 2017/07/01 CRDT- 2017/09/28 06:00 PHST- 2015/12/06 00:00 [received] PHST- 2016/05/28 00:00 [accepted] PHST- 2017/09/28 06:00 [entrez] PHST- 2017/09/28 06:00 [pubmed] PHST- 2017/10/11 06:00 [medline] PHST- 2017/07/01 00:00 [pmc-release] AID - S0365-05962017000400466 [pii] AID - 10.1590/abd1806-4841.20175476 [doi] PST - ppublish SO - An Bras Dermatol. 2017 Jul-Aug;92(4):466-469. doi: 10.1590/abd1806-4841.20175476. PMID- 17269983 OWN - NLM STAT- MEDLINE DCOM- 20070614 LR - 20131121 IS - 0011-9059 (Print) IS - 0011-9059 (Linking) VI - 46 IP - 2 DP - 2007 Feb TI - Evaluation of oral methotrexate in the treatment of systemic sclerosis. PG - 218-23 AB - BACKGROUND: Treatment of scleroderma is difficult and currently no treatment can induce complete remission of the disease. OBJECTIVE: To evaluate weekly oral methotrexate in the treatment of Indian patients with systemic sclerosis. METHODS: Thirty-three patients with systemic sclerosis presenting to the department of dermatology (outpatients) who satisfied the inclusion criteria were enrolled into the study. All cases were admitted into the dermatology ward for detailed evaluation. A detailed history and physical examination, including assessment of disease severity by Rodnan skin scoring, was carried out. Baseline investigations included complete blood counts, blood glucose, serum electrolytes, renal function test, liver function tests, urine examination (albumin, sugar, microscopic examination, 24-h protein), ANA, chest X-ray, Barium swallow, pulmonary function test, electrocardiogram (ECG), HRCT of chest, and 4-mm punch skin biopsy from dorsum of the hand. All the patients were treated with oral methotrexate (15 mg/week) for 6 months, following standard guidelines. RESULTS: The patients included 29 (87.9%) females and four (12.1%) males with a mean age of 31.45 +/- 8.76 years. The mean duration of disease was 5.6 +/- 4.5 years (range 2 months to 15 years). All the patients had binding down of skin, 31 (93.9%) had Raynaud's phenomenon, 31 (93.9%) had pigmentary change, 21 (63.6%) had hand contractures, 17 (51.5%) had fingertip ulcers, 15 (45.5%) had dyspnoea, 14 (42.4%) had restricted mouth opening, 13 (39.4%) had telangiectasia, 11 (33.3%) had fingertip resorption, eight (24.2%) had joint complaints, six (18.2%) had dysphagia, and one (3.03%) had gangrene. On laboratory investigation ANA was positive in 29 (87.9%) patients, dsDNA was raised in only four (12.1%), baseline chest X-ray was abnormal in 18 (54.5%), HRCT was abnormal in 27 (81.8%), abnormal PFT in 32 (96.9%), abnormal ECG in five (15.2%), and barium swallow abnormality in 19 (57.5%) patients. Twenty-five patients completed the 6-month follow up. There was subjective improvement in binding down (80%), Raynaud's phenomenon (96%), fingertip ulceration (88.8%), hyperpigmentation (77.2%) and dyspnoea (45.5%). The objective parameters showed statistically significant improvement in mouth openingm, but improvement of skin score, lung function (chest radiograph, PFT, HRCT), and dysphagia was not significant at the 6-month follow up. In eight patients, treatment was continued for 1 year of methotrexate, which showed statistically significant improvement in skin score. CONCLUSION: It was concluded that methotrexate for 6 months only provides subjective improvement, and further studies after 1 year of treatment with methotrexate are recommended. FAU - Krishna Sumanth, M AU - Krishna Sumanth M AD - Department of Dermatology and Venereology and the Department of Radiology, All India Institute of Medical Sciences, New Delhi, India. FAU - Sharma, Vinod K AU - Sharma VK FAU - Khaitan, Binod K AU - Khaitan BK FAU - Kapoor, Anu AU - Kapoor A FAU - Tejasvi, Trilokraj AU - Tejasvi T LA - eng PT - Clinical Trial PT - Journal Article PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - 0 (Immunosuppressive Agents) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Administration, Oral MH - Adolescent MH - Adult MH - Female MH - Humans MH - Immunosuppressive Agents/administration & dosage/*therapeutic use MH - India MH - Male MH - Methotrexate/administration & dosage/*therapeutic use MH - Middle Aged MH - Scleroderma, Systemic/*drug therapy MH - Treatment Outcome EDAT- 2007/02/03 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/02/03 09:00 PHST- 2007/02/03 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/02/03 09:00 [entrez] AID - IJD2887 [pii] AID - 10.1111/j.1365-4632.2007.02887.x [doi] PST - ppublish SO - Int J Dermatol. 2007 Feb;46(2):218-23. doi: 10.1111/j.1365-4632.2007.02887.x. PMID- 26022697 OWN - NLM STAT- MEDLINE DCOM- 20160922 LR - 20220316 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 24 IP - 12 DP - 2015 Oct TI - Clinical, serologic, and immunogenetic characterization (HLA-DRB1) of late-onset lupus erythematosus in a Colombian population. PG - 1293-9 LID - 10.1177/0961203315588576 [doi] AB - INTRODUCTION: Late-onset systemic lupus erythematosus (SLE) represents a specific subgroup that is defined as onset after 50 years of age. Late-onset lupus may have a different clinical course and serological findings, which may delay diagnosis and timely treatment. OBJECTIVES: The objective of this paper is to determine the clinical, serologic, and immunogenetic differences among Colombian patients with late-onset SLE versus conventional SLE patients. METHODOLOGY: This was a cross-sectional study in a Colombian population. Patients and their medical records were analyzed from the services of Rheumatology in Bogotá and met the criteria for SLE, according to the American College of Rheumatology (ACR) revised criteria for the classification of SLE.In a reference group of late-onset SLE patients (98 participants, with an onset after 50 years of age) and a group of conventional SLE patients (72 participants, with an onset of age of 49 years or less), multiple clinical variables (age, clinical criteria for lupus, alopecia, weight loss, fever, Raynaud's phenomenon) and multiple serological variables (blood count, blood chemistry profile, autoantibodies) were analyzed. Additionally, the HLA class II (DRB1) of all the patients was genotyped, including an additional group of patients without the autoimmune disease. Statistical analysis was performed using the STATA 10.0 package. RESULTS: In the group of late-onset lupus, there was a higher frequency of pleurisy (p = 0.002), pericarditis (p = 0.026), dry symptoms (p = 0.029), lymphopenia (p = 0.007), and higher titers of rheumatoid factor (p = 0.001) compared with the group of conventional SLE. Late-onset SLE patients had a lower seizure frequency (p = 0.019), weight loss (p = 0.009), alopecia (p < 0.001), and Raynaud's phenomenon (p = 0.013) compared to the conventional SLE group. In late-onset SLE, HLA DR17 (DR3) was found more frequently compared with individuals without autoimmune disease (OR 3.81, 95% CI 1.47 to 10.59) (p = 0.0016). CONCLUSION: In the Colombian SLE population analyzed, there may be a probable association of several clinical and serologic variants, which would allow the differentiation of variables in the presentation of the disease among patients with late-onset SLE vs. conventional SLE. CI - © The Author(s) 2015. FAU - Peñaranda-Parada, E AU - Peñaranda-Parada E AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - Quintana, G AU - Quintana G AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - Yunis, J J AU - Yunis JJ AD - Unit of Genetics, National University of Colombia, Bogotá, Colombia. FAU - Mantilla, R AU - Mantilla R AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - Rojas, W AU - Rojas W AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - Panqueva, U AU - Panqueva U AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - Caminos, J E AU - Caminos JE AD - Unit of Biochemistry, National University of Colombia, Bogotá, Colombia. FAU - Garces, M F AU - Garces MF AD - Unit of Biochemistry, National University of Colombia, Bogotá, Colombia. FAU - Sanchez, E AU - Sanchez E AD - Unit of Biochemistry, National University of Colombia, Bogotá, Colombia. FAU - Rondón-Herrera, F AU - Rondón-Herrera F AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia. FAU - de Jesús Iglesias-Gamarra, A AU - de Jesús Iglesias-Gamarra A AD - Rheumatology Unit, National University of Colombia, Bogotá, Colombia iglesias.antonio1@gmail.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150528 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Autoantibodies) RN - 0 (HLA-DRB1 Chains) SB - IM MH - Adolescent MH - Adult MH - *Age of Onset MH - Aged MH - Aged, 80 and over MH - Autoantibodies/blood MH - Colombia MH - Cross-Sectional Studies MH - Female MH - Genotype MH - HLA-DRB1 Chains/*genetics MH - Humans MH - Immunogenetics MH - Lupus Erythematosus, Systemic/*epidemiology/*genetics MH - Male MH - Middle Aged MH - Young Adult OTO - NOTNLM OT - Systemic lupus erythematosus (SLE) OT - human leukocyte antigen DRB1 (HLA-DRB1) OT - late-onset lupus EDAT- 2015/05/30 06:00 MHDA- 2016/09/23 06:00 CRDT- 2015/05/30 06:00 PHST- 2014/09/24 00:00 [received] PHST- 2015/04/23 00:00 [accepted] PHST- 2015/05/30 06:00 [entrez] PHST- 2015/05/30 06:00 [pubmed] PHST- 2016/09/23 06:00 [medline] AID - 0961203315588576 [pii] AID - 10.1177/0961203315588576 [doi] PST - ppublish SO - Lupus. 2015 Oct;24(12):1293-9. doi: 10.1177/0961203315588576. Epub 2015 May 28. PMID- 16831323 OWN - NLM STAT- MEDLINE DCOM- 20070306 LR - 20140226 IS - 0578-1426 (Print) IS - 0578-1426 (Linking) VI - 45 IP - 6 DP - 2006 Jun TI - [The clinical analysis of pulmonary arterial hypertension in connective tissue disease]. PG - 467-71 AB - OBJECTIVE: To investigate the clinical features and prognosis of pulmonary arterial hypertension (PAH) secondary to connective tissue disease (CTD). METHODS: Eighty-two patients diagnosed as having CTD associated PAH (CTDaPAH) were retrospectively analyzed among 2189 patients with CTD in Peking Union Medical College Hospital between January 1997 and September 2004. RESULTS: Eighty-two (3.7%) patients (75 females and 7 males), aged 41 years, were found to have CTDaPAH. The underlying diseases included mixed CTD (MCTD), systemic scleroderma, primary Sjogren's syndrome (pSS), systemic lupus erythematosus (SLE), undifferentiated CTD, dermatomyositis and Behcet disease. PAH was an earlier complication in patients with SLE or MCTD (median onset, 3 and 2 years, respectively), compared to those with pSS (6 years). The most common clinical manifestations of CTDaPAH were dyspnea on exertion (84.1%) and Raynaud's phenomenon (56.1%). The mean pulmonary artery systolic pressure (PASP) level of these patients was (65.71 +/- 20.44) mm Hg, with the decrease of PaO2 and PaCO2 level [(70.37 +/- 15.02) mm Hg, (27.88 +/- 6.46) mm Hg, respectively]. The diffusing capacity of the lungs measured using carbon monoxide was decreased [D(L)CO pred% (51 +/- 14)%]. 13%, 32%, 29% and 8% of these patients were grouped to class I, II, III and IV, respectively, on NYHA functional classification. Although the patients received treatment for underlying CTD and traditional vasodilators, the condition of elevated PASP had not been improved with the exception of a marginal decrease of PASP in SLE patients [(76.47 +/- 18.20) mm Hg --> (69.08 +/- 20.77) mm Hg]. Thirteen (15.85%) patients died during an average of 4.33 years follow-up, compared to the mortality of those with non-CTDaPAH (2.75%, P < 0.01). Lower level of PaO2 [(58.51 +/- 16.16) mm Hg] and higher proportion of NYHA class III, IV [38.46% (5/13), 30.77% (4/13), respectively] were observed in death group compared to survivors [PaO2 (73.25 +/- 14.32) mm Hg; NYHA class III, IV 34.78% (24/69), 5.80% (4/69), respectively] (P < 0.01; P < 0.05; respectively). CONCLUSIONS: PAH is a common complication of CTDs, which occurs often in the forth year after initial CTD manifestations and is earlier in patients with SLE or MCTD, compared to those with pSS. The most common manifestations of CTDaPAH are dyspnea on exertion and Raynaud's phenomenon. CTDaPAH could be very severe, which will lead to lower level of PaO2 and more advanced NYHA classification, and therefore reduce the survival rate of the patients. Doppler echocardiography and lung function test are necessary to detect PAH early. FAU - Ji, Ying-qun AU - Ji YQ AD - Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing 100730, China. FAU - Zhang, Zhuo-li AU - Zhang ZL FAU - Lu, Wei-xuan AU - Lu WX LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Nei Ke Za Zhi JT - Zhonghua nei ke za zhi JID - 16210490R SB - IM MH - Adolescent MH - Adult MH - Aged MH - Child MH - Connective Tissue Diseases/*complications MH - Echocardiography MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/*complications/diagnosis MH - Male MH - Middle Aged MH - Prognosis MH - Respiratory Function Tests MH - Retrospective Studies EDAT- 2006/07/13 09:00 MHDA- 2007/03/07 09:00 CRDT- 2006/07/13 09:00 PHST- 2006/07/13 09:00 [pubmed] PHST- 2007/03/07 09:00 [medline] PHST- 2006/07/13 09:00 [entrez] PST - ppublish SO - Zhonghua Nei Ke Za Zhi. 2006 Jun;45(6):467-71. PMID- 29320807 OWN - NLM STAT- MEDLINE DCOM- 20181114 LR - 20181114 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 23 IP - 6 DP - 2018 Jun TI - Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality. PG - 600-605 LID - 10.1111/resp.13254 [doi] AB - BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome. METHODS: Patients with prior UCTD-interstitial lung disease (ILD) were screened by ERS/ATS criteria for IPAF. Clinical data along with all-cause mortality were collated and compared with selected idiopathic pulmonary fibrosis (IPF) patients from the same study period. Survival was compared between IPAF subgroups with and without UIP features. RESULTS: One hundred and one UCTD-ILD subjects (91%) evaluated from 2005 to 2012 also met strict criteria for IPAF. Frequent clinical findings included Raynaud's phenomenon, positive anti-nuclear antibody (ANA) and non-specific interstitial pneumonia (NSIP) pattern on chest computed tomography (CT). Nineteen had features of UIP either on histopathology or CT imaging. As compared with IPF, IPAF patients had overall better survival except in those with UIP features. CONCLUSION: Current IPAF criteria encompassed the majority of broadly defined UCTD-ILD and included those with UIP findings. Survival compared with IPF in those with UIP was similar. Further studies are necessary to refine IPAF definitions for clinical use and guide directed management strategies. CI - © 2018 Asian Pacific Society of Respirology. FAU - Kelly, Bryan T AU - Kelly BT AD - Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA. FAU - Moua, Teng AU - Moua T AUID- ORCID: 0000-0003-3329-5717 AD - Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. LA - eng PT - Journal Article DEP - 20180110 PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 SB - IM CIN - Respirology. 2018 Oct;23(10):959. doi: 10.1111/resp.13379. PMID: 30070408 CIN - Respirology. 2018 Oct;23(10):958. doi: 10.1111/resp.13380. PMID: 30070755 MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoimmune Diseases/*classification/diagnostic imaging/mortality/pathology MH - Cause of Death MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/*classification/diagnostic imaging/mortality/pathology MH - Lung/diagnostic imaging/pathology MH - Lung Diseases, Interstitial/*classification/diagnostic imaging/mortality/pathology MH - Male MH - Middle Aged MH - Mortality MH - Tomography, X-Ray Computed/methods MH - Undifferentiated Connective Tissue Diseases/*classification/diagnostic imaging/mortality/pathology MH - Young Adult OTO - NOTNLM OT - autoimmune disease OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - interstitial pneumonia OT - usual interstitial pneumonia EDAT- 2018/01/11 06:00 MHDA- 2018/11/15 06:00 CRDT- 2018/01/11 06:00 PHST- 2017/02/17 00:00 [received] PHST- 2017/11/22 00:00 [revised] PHST- 2017/12/13 00:00 [accepted] PHST- 2018/01/11 06:00 [pubmed] PHST- 2018/11/15 06:00 [medline] PHST- 2018/01/11 06:00 [entrez] AID - 10.1111/resp.13254 [doi] PST - ppublish SO - Respirology. 2018 Jun;23(6):600-605. doi: 10.1111/resp.13254. Epub 2018 Jan 10. PMID- 19099898 OWN - NLM STAT- MEDLINE DCOM- 20101202 LR - 20161124 IS - 0578-1310 (Print) IS - 0578-1310 (Linking) VI - 46 IP - 11 DP - 2008 Nov TI - [Clinical analysis of pulmonary arterial hypertension secondary to connective tissue disease in children]. PG - 822-6 AB - OBJECTIVE: To study the epidemiology, clinical characteristics and prognosis of children with pulmonary arterial hypertension (PAH) secondary to connective tissue disease (CTD). METHODS: The clinical record files of the pediatric inpatient with PAH in the population of CTD in the hospital treated between January 2000 and January 2007 were analyzed retrospectively. RESULTS: (1) In 299 patients with CTD and complete Doppler echocardiography files, 31 (31/299, 10.4%) patients (28 females and 3 males), aged from 7 to 18 years (average: 12.5 years), were found to have PAH, in whom, 5(62.5%)in 8 patients with overlapped CTD (OCTD), 2 (50.0%) in 4 patients with antiphospholipid syndrome (APS), 4(28.6%) in 14 patients with systematic vasculitis (SV), 3 (10.7%) in 28 patients with dermatomyositis (DM), 17(7.6%) in 223 systemic lupus erythematosus (SLE) had PAH. The CTD-associated PAH occurred in the 3rd week to 5th year after initial CTD manifestations (median onset: 1.5 years). (2) The onset of CTD-associated PAH was obscure and children with severe CTD-associated PAH presented with dyspnea (18/31, 58.1%) and heart failure (9/31, 29.0%). The children with Raynaud's phenomenon or positive anticardiophospholipid antibody (ACL) or positive lupus anticoagulant (LA) were prone to have more severe PAH. (3) Doppler echocardiography and pulmonary function test, especially the test of pulmonary diffusion function of CO (D(LCO)) were necessary to detect PAH early. (4) After treatment, the pulmonary arterial pressure in mild and moderate PAH cases could be normalized and in severe PAH cases could be decreased to mild or moderate levels. There was a lower PaO(2) level (P < 0.01), a higher pulmonary arterial systolic pressure (PASP) level (P < 0.05) in the cases of CTD-PAH who died as compared with the live patients. CONCLUSIONS: PAH is a common complication of CTDs, which occurs often 1.5 years after initial CTD manifestations. The early associated symptom is obscure and the severe cases manifest with dyspnea and heart failure. Those with Raynaud's phenomenon and positive ACL and LA are prone to develop more severe PAH. Early and regular Doppler echocardiography and pulmonary function test are necessary to detect PAH early. Severe CTD associated PAH in children could lead to poor outcome. PASP classification and PaO(2) level are important factors affecting prognosis. FAU - Xing, Yan AU - Xing Y AD - Department of Pediatrics, Peking Union Medical College Hospital, Beijing 100730, China. FAU - Song, Hong-mei AU - Song HM FAU - Wu, Xiao-yan AU - Wu XY FAU - He, Yan-yan AU - He YY FAU - Wei, Min AU - Wei M LA - chi PT - Journal Article PL - China TA - Zhonghua Er Ke Za Zhi JT - Zhonghua er ke za zhi = Chinese journal of pediatrics JID - 0417427 SB - IM MH - Adolescent MH - Child MH - Connective Tissue Diseases/*complications/*diagnostic imaging/physiopathology MH - Echocardiography, Doppler MH - Female MH - Humans MH - Hypertension, Pulmonary/*diagnostic imaging/*etiology MH - Male MH - Prognosis MH - Respiratory Function Tests MH - Retrospective Studies EDAT- 2008/12/23 09:00 MHDA- 2010/12/14 06:00 CRDT- 2008/12/23 09:00 PHST- 2008/12/23 09:00 [entrez] PHST- 2008/12/23 09:00 [pubmed] PHST- 2010/12/14 06:00 [medline] PST - ppublish SO - Zhonghua Er Ke Za Zhi. 2008 Nov;46(11):822-6. PMID- 26121083 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20220317 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 33 IP - 4 Suppl 91 DP - 2015 Jul-Aug TI - Increased messenger RNA levels of the mesenchymal cadherin-11 in the peripheral blood of systemic sclerosis patients correlate with diffuse skin involvement. PG - S36-9 AB - OBJECTIVES: Cadherin-11 is a cell-cell adhesion molecule also involved in cellular migration and invasion. Experimental studies implicated this molecule in inflammatory arthritis and fibrosing conditions. Moreover, cadherin-11 protein is hyper-expressed on fibroblasts and macrophages in the skin of systemic sclerosis (SSc) patients, whereas the respective mRNA levels correlate with skin thickness. Herein, we searched for possible cadherin-11 expression also in cells that circulate in SSc peripheral blood. METHODS: Cadherin-11 mRNA was quantified by real-time reverse transcription-polymerase chain reaction in 3 ml blood samples obtained from 71 SSc patients (aged 53±2 years, 65 women) and 35 control non-SSc patients with Raynaud's phenomenon. RESULTS: Cadherin-11 mRNA transcripts were detected in blood samples from 39% of patients with diffuse SSc, versus 16% of those with limited SSc, versus 6% and 16% of patients with idiopathic or associated with other connective tissue diseases Raynaud's phenomenon, respectively (p=0.049). Cadherin-11 mRNA levels in SSc patients were increased by 3.74-fold comparing to controls (p=0.036). By multivariate logistic regression analysis we found that diffuse skin involvement correlated, independently of age, gender, disease duration, lung involvement, digital ulcers, inflammatory indices or anti-Scl-70 autoantibody presence, with cadherin-11 mRNA positivity (p=0.028), but also with increased cadherin-11 mRNA levels (≥3-fold of non-SSc levels, p=0.011). CONCLUSIONS: Cadherin-11 may be hyper-expressed in the peripheral blood of diffuse SSc patients. Studies on the origin and possible pathogenic function of these circulating cells may shed light into the complex disease pathogenesis and further support the notion that cadherin-11 is a potential therapeutic target in SSc. FAU - Christopoulos, Panagiotis F AU - Christopoulos PF AD - Rheumatology Unit, First Department of Propaedeutic and Internal Medicine; and Department of Experimental Physiology, Athens University Medical School, Greece. FAU - Bournia, Vasiliki-Kalliopi AU - Bournia VK AD - Rheumatology Unit, First Department of Propaedeutic and Internal Medicine, Athens University Medical School, Greece. FAU - Panopoulos, Stylianos AU - Panopoulos S AD - Rheumatology Unit, First Department of Propaedeutic and Internal Medicine, Athens University Medical School, Greece. FAU - Vaiopoulos, Aristeides AU - Vaiopoulos A AD - Department of Experimental Physiology, Athens University Medical School, Greece. FAU - Koutsilieris, Michael AU - Koutsilieris M AD - Department of Experimental Physiology, Athens University Medical School, Greece. FAU - Sfikakis, Petros P AU - Sfikakis PP AD - Rheumatology Unit, First Department of Propaedeutic and Internal Medicine, Athens University Medical School, Greece. psfikakis@med.uoa.gr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150629 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Cadherins) RN - 0 (Genetic Markers) RN - 0 (RNA, Messenger) RN - 156621-71-5 (osteoblast cadherin) SB - IM MH - Adult MH - Aged MH - Cadherins/*genetics MH - Case-Control Studies MH - Chi-Square Distribution MH - Female MH - Genetic Markers MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - RNA, Messenger/*blood MH - Real-Time Polymerase Chain Reaction MH - Reverse Transcriptase Polymerase Chain Reaction MH - Risk Factors MH - Scleroderma, Diffuse/blood/diagnosis/*genetics MH - Scleroderma, Limited/blood/diagnosis/*genetics MH - Severity of Illness Index MH - Up-Regulation MH - Young Adult EDAT- 2015/06/30 06:00 MHDA- 2015/10/27 06:00 CRDT- 2015/06/30 06:00 PHST- 2015/01/22 00:00 [received] PHST- 2015/04/17 00:00 [accepted] PHST- 2015/06/30 06:00 [entrez] PHST- 2015/06/30 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 9121 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S36-9. Epub 2015 Jun 29. PMID- 41464713 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251230 LR - 20260102 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 24 DP - 2025 Dec 12 TI - Association of Inflammation-Based Ratios with Endothelial Dysfunction Markers and Clinical Parameters in Limited Cutaneous Systemic Sclerosis. LID - 10.3390/jcm14248806 [doi] LID - 8806 AB - Background: Limited cutaneous systemic sclerosis (lcSSc) is an autoimmune disease with a wide range of different biomarkers, while inflammation-based ratios have been less extensively investigated. This study aimed to evaluate the associations between inflammation-based ratios, disease-specific parameters, and endothelial dysfunction, as well as to assess the predictive role of inflammation-based ratios in lcSSc. Methods: A total of 38 lcSSc patients and 38 matched controls with primary Raynaud's phenomenon were analyzed at baseline regarding inflammation-based ratios, lcSSc-specific parameters, and parameters of endothelial dysfunction. LcSSc patients were prospectively observed during a 3-year follow-up period in which lcSSc complications were recorded annually. Results: LcSSc patients had a significantly higher neutrophil-to-lymphocyte ratio, monocyte-to-lymphocyte ratio (MLR), fibrinogen-to-albumin ratio, monocyte/high-density lipoprotein (HDL) ratio, and neutrophil/HDL ratio versus controls (all p < 0.05). During follow-up, the MLR, C-reactive protein (CRP)/albumin ratio, monocyte/HDL ratio, and neutrophil/HDL ratio increased significantly (all p < 0.05) in lcSSc patients. The monocyte/HDL ratio correlated positively with the DETECT score step 2 (r = 0.453, p = 0.032) and negatively with the UCLA SCTC GIT total score (r = -0.469, p = 0.024). The CRP/albumin ratio correlated significantly with the EUSTAR index (r = 0.473, p = 0.024) and the fibrinogen-to-albumin ratio correlated with asymmetric dimethylarginine (r = 0.452, p = 0.044). The MLR and CRP/albumin ratio were associated with development of pulmonary arterial hypertension (p = 0.036, p = 0.006), and the lymphocyte/HDL ratio was associated with newly developed interstitial lung disease (p = 0.004). Conclusions: Readily available inflammation-based ratios may reflect vascular and inflammatory activity and could contribute to risk stratification for pulmonary complications in lcSSc; however, these exploratory findings require confirmation in larger cohorts. FAU - Schweiger, Leyla AU - Schweiger L AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Meinitzer, Andreas AU - Meinitzer A AUID- ORCID: 0000-0002-7883-7973 AD - Institute of Medical and Chemical Laboratory Diagnostics, Medical University of Graz, 8010 Graz, Austria. FAU - Strohmaier, Heimo AU - Strohmaier H AUID- ORCID: 0000-0002-4037-3608 AD - Center of Medical Research (ZMF), Medical University of Graz, 8010 Graz, Austria. FAU - Moazedi-Fürst, Florentine AU - Moazedi-Fürst F AD - Division of Rheumatology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Nemecz, Viktoria AU - Nemecz V AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Kurzmann-Gütl, Katharina AU - Kurzmann-Gütl K AUID- ORCID: 0000-0002-1659-7185 AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Brodmann, Marianne AU - Brodmann M AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Hafner, Franz AU - Hafner F AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. FAU - Jud, Philipp AU - Jud P AUID- ORCID: 0000-0002-0832-1322 AD - Division of Angiology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria. LA - eng PT - Journal Article DEP - 20251212 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC12733724 OTO - NOTNLM OT - endothelial dysfunction OT - inflammation-based ratios OT - limited cutaneous systemic sclerosis OT - pulmonary arterial hypertension COIS- The authors declare no conflicts of interest. EDAT- 2025/12/30 07:41 MHDA- 2025/12/30 07:42 PMCR- 2025/12/12 CRDT- 2025/12/30 02:01 PHST- 2025/10/17 00:00 [received] PHST- 2025/12/03 00:00 [revised] PHST- 2025/12/10 00:00 [accepted] PHST- 2025/12/30 07:42 [medline] PHST- 2025/12/30 07:41 [pubmed] PHST- 2025/12/30 02:01 [entrez] PHST- 2025/12/12 00:00 [pmc-release] AID - jcm14248806 [pii] AID - jcm-14-08806 [pii] AID - 10.3390/jcm14248806 [doi] PST - epublish SO - J Clin Med. 2025 Dec 12;14(24):8806. doi: 10.3390/jcm14248806. PMID- 38938909 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240629 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 6 DP - 2024 Jun TI - The Autoimmune Gender Gap: A Rare Case of a Male Patient With Overlapping Autoimmune Hepatitis and Primary Biliary Cholangitis. PG - e63312 LID - 10.7759/cureus.63312 [doi] LID - e63312 AB - Autoimmune hepatitis (AIH) is a condition resulting in chronic, inflammatory changes to the liver. Primary biliary cholangitis (PBC) is an autoimmune condition that destroys intrahepatic bile ducts. Overlap syndrome with concomitant AIH and PBC comprises a rare subgroup of patients with immune-mediated liver disease, with incidence rates of male patients being exceedingly uncommon in a predominantly female patient population. Our case report investigates a rare case of a 41-year-old male patient diagnosed with overlapping AIH and PBC. He initially presented with symptoms of fatigue, pruritus, and episodes of Raynaud's phenomenon, in addition to findings of persistently elevated liver enzymes despite lifestyle modifications. He had no past medical history, no history of alcohol use disorder, and no family medical history of chronic liver disease. Imaging did not reveal evidence of cirrhosis. Further diagnostic workup was significant for elevated immunologic markers for antinuclear antibodies (ANA) with positive centromere and cytoplasmic patterns, antimitochondrial antibodies (AMA) with F-actin antibodies, anti-smooth muscle antibodies (ASMA), and cytoplasmic antinuclear cytoplasmic antibodies (ANCA C). Liver biopsy showed prominent plasma cells and rare granulomas, consistent with the diagnosis of AIH with a component of PBC, respectively. He was started on ursodeoxycholic acid (UDCA), demonstrating a near-complete clinical response with resolution of symptoms and normalization of liver enzymes. Studies investigating the low incidence of male patients with overlap syndrome are limited, as current research is overwhelmingly based on studies with predominantly female subjects. However, most studies generally recommend treatment with both UDCA and corticosteroids to reduce symptoms and biochemical markers. Our case report highlights a rare case of a male patient documenting excellent biochemical and clinical responses to monotherapy with UDCA. A possible theory is that our patient's early treatment (prior to advanced disease progression) is associated with his near-complete biochemical response and symptomatic resolution on UDCA alone. Further research is needed to fully understand the clinical course and long-term prognosis of male patients with overlap syndrome. Our patient remains in life-long follow-up to monitor if or when he requires treatment with corticosteroids in addition to current monotherapy with UDCA.​. CI - Copyright © 2024, Chwa et al. FAU - Chwa, Katherine AU - Chwa K AD - Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA. FAU - Aung, Sammy AU - Aung S AD - Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA. FAU - Reyes Yparraguirre, Armando AU - Reyes Yparraguirre A AD - Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA. FAU - Wayman, Connor AU - Wayman C AD - Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA. FAU - Canaday, Omar AU - Canaday O AD - Internal Medicine, University of Nevada Reno School of Medicine, Reno, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20240627 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11210994 OTO - NOTNLM OT - autoimmune hepatitis OT - male overlap syndrome OT - overlap syndrome OT - pbc-aih overlap syndrome OT - primary biliary cholangitis COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/06/28 06:42 MHDA- 2024/06/28 06:43 PMCR- 2024/06/27 CRDT- 2024/06/28 04:52 PHST- 2024/06/27 00:00 [accepted] PHST- 2024/06/28 06:43 [medline] PHST- 2024/06/28 06:42 [pubmed] PHST- 2024/06/28 04:52 [entrez] PHST- 2024/06/27 00:00 [pmc-release] AID - 10.7759/cureus.63312 [doi] PST - epublish SO - Cureus. 2024 Jun 27;16(6):e63312. doi: 10.7759/cureus.63312. eCollection 2024 Jun. PMID- 35443665 OWN - NLM STAT- MEDLINE DCOM- 20220422 LR - 20220716 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Apr 20 TI - Nodular Regenerative Hyperplasia of the liver in Juvenile Dermatomyositis. PG - 30 LID - 10.1186/s12969-022-00690-x [doi] LID - 30 AB - BACKGROUND: We present two cases of Nodular Regenerative Hyperplasia (NRH) associated with Juvenile Dermatomyositis (JDM). CASE PRESENTATION: Case 1: A nine-year-old Caucasian male with refractory JDM and anti-NXP2 autoantibodies was diagnosed at age two. Over seven years, he developed arthritis, dysphagia, dysphonia, severe calcinosis, and colitis. Complications included recurrent cellulitis, infections, and hepatosplenomegaly. Multiple medications were chronically used, including prednisone, methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil, rituximab, tacrolimus, etanercept, abatacept, infliximab, and tocilizumab. Case 2: A 19-year-old Asian female with chronically active JDM and anti-MDA5 autoantibodies was diagnosed at age 15. Symptomatology included ulcerative skin lesions, Raynaud's phenomenon with digital ulcers, arthritis, interstitial lung disease with pulmonary hypertension, and calcinosis. Medications included chronic use of prednisone, methotrexate, abatacept, cyclophosphamide, mycophenolate mofetil, rituximab, tofacitinib, and sildenafil. In both patients, clinical symptomatology was not suggestive of liver disease or portal hypertension, but laboratory studies revealed elevated serum transaminases with progressive thrombocytopenia and no active liver-associated infections. The first patient's liver ultrasound showed coarse hepatic texture with mild echogenicity, splenomegaly, and portal hypertension. The second patient's liver ultrasound was normal, but elastography indicated increased stiffness. Liver biopsy confirmed NRH in both patients. CONCLUSIONS: It is difficult to recognize NRH in JDM, as it often presents with elevated transaminases which may be mistaken for JDM muscle flare, corticosteroid-related fatty liver, or medication-related transaminitis. NRH has been associated with several medications used to treat JDM, including methotrexate, azathioprine, and cyclophosphamide, which should be discontinued if NRH develops. Providers should consider NRH in JDM patients with severe, refractory disease who have persistently elevated transaminases and persistent thrombocytopenia. CI - © 2022. The Author(s). FAU - Lanis, Aviya AU - Lanis A AUID- ORCID: 0000-0002-1309-6481 AD - Seattle Children's Hospital and Research Center, 4800 Sand Point Way NE, PO Box 5371, Seattle, WA, 98105, USA. Aviya.Lanis@seattlechildrens.org. FAU - Volochayev, Rita AU - Volochayev R AD - Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Kleiner, David E AU - Kleiner DE AD - Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Vittal, Anusha AU - Vittal A AD - Translational Hepatology Section, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA. FAU - Heller, Theo AU - Heller T AD - Translational Hepatology Section, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA. FAU - Rider, Lisa G AU - Rider LG AD - Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Shenoi, Susan AU - Shenoi S AD - Seattle Children's Hospital and Research Center, 4800 Sand Point Way NE, PO Box 5371, Seattle, WA, 98105, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20220420 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - 0 (Autoantibodies) RN - 4F4X42SYQ6 (Rituximab) RN - 7D0YB67S97 (Abatacept) RN - 8N3DW7272P (Cyclophosphamide) RN - EC 2.6.1.- (Transaminases) RN - HU9DX48N0T (Mycophenolic Acid) RN - MRK240IY2L (Azathioprine) RN - VB0R961HZT (Prednisone) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Abatacept/therapeutic use MH - Adolescent MH - *Arthritis MH - Autoantibodies MH - Azathioprine/therapeutic use MH - *Calcinosis/pathology MH - Child MH - Cyclophosphamide/therapeutic use MH - *Dermatomyositis/complications/diagnosis/drug therapy MH - Female MH - Humans MH - Hyperplasia/complications/pathology MH - *Hypertension, Portal/complications/pathology MH - Liver/pathology MH - Male MH - Methotrexate/therapeutic use MH - Mycophenolic Acid/therapeutic use MH - Prednisone/therapeutic use MH - Rituximab/therapeutic use MH - Splenomegaly MH - *Thrombocytopenia/complications MH - Transaminases/therapeutic use MH - Young Adult PMC - PMC9022230 OTO - NOTNLM OT - Juvenile Dermatomyositis OT - Nodular Regenerative Hyperplasia COIS- The authors declare that they have no competing interests. EDAT- 2022/04/22 06:00 MHDA- 2022/04/23 06:00 PMCR- 2022/04/20 CRDT- 2022/04/21 05:07 PHST- 2021/11/05 00:00 [received] PHST- 2022/04/09 00:00 [accepted] PHST- 2022/04/21 05:07 [entrez] PHST- 2022/04/22 06:00 [pubmed] PHST- 2022/04/23 06:00 [medline] PHST- 2022/04/20 00:00 [pmc-release] AID - 10.1186/s12969-022-00690-x [pii] AID - 690 [pii] AID - 10.1186/s12969-022-00690-x [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Apr 20;20(1):30. doi: 10.1186/s12969-022-00690-x. PMID- 37451812 OWN - NLM STAT- MEDLINE DCOM- 20230717 LR - 20240922 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 3 DP - 2023 Jul TI - Microvascular damage in autoimmune connective tissue diseases: a capillaroscopic analysis from 20 years of experience in a EULAR training and research referral centre for imaging. LID - 10.1136/rmdopen-2023-003071 [doi] LID - e003071 AB - OBJECTIVE: Nailfold videocapillaroscopy (NVC) allows the detection of microvascular damage in autoimmune connective tissue diseases (CTDs). The prevalence of the morphological capillary findings was retrospectively evaluated in a wide cohort of patients with Raynaud's phenomenon secondary to a CTD at the time of the first single NVC, independently from their current treatment, autoantibody profile and comorbidities. METHODS: One-thousand-one-hundred-eighty-one patients affected by CTDs were included from 2001 to 2021. The considered CTDs were systemic sclerosis (SSc), undifferentiated connective tissue disease (UCTD), mixed connective tissue disease (MCTD), dermatomyositis (DM), systemic lupus erythematosus, Sjögren's syndrome and primary antiphospholipid syndrome (aPS). The capillaroscopic parameters were distinguished between scleroderma patterns and non-scleroderma patterns. RESULTS: Giant capillaries were significantly more frequent in SSc, DM and MCTD than in other CTDs (respectively, in 73%, 73% and 61% of patients, p<0.001 when comparing each rate vs the other CTDs). The mean capillary count was significantly lower in SSc, DM and MCTD (respectively, 7.04±0.18 vs 6.5±0.75 vs 7.7±2 capillaries/linear mm) compared with the other CTDs (p<0.001 for each rate vs the other CTDs). The non-specific abnormalities of capillary morphology were significantly more frequent in SSc, MCTD and aPS (respectively, in 48%, 41% and 36% of cases, all p<0.001 vs each other CTDs). CONCLUSION: This large size sample of patients with CTDs, collected over 20 years of analysis, confirms the highest prevalence of specific capillaroscopic alterations in patients with SSc, DM and MCTD, when compared with other CTDs. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Hysa, Elvis AU - Hysa E AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Pizzorni, Carmen AU - Pizzorni C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Sammorì, Silvia AU - Sammorì S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Gotelli, Emanuele AU - Gotelli E AUID- ORCID: 0000-0002-4732-0306 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Cere, Andrea AU - Cere A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Schenone, Carlotta AU - Schenone C AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Ferrari, Giorgia AU - Ferrari G AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Campitiello, Rosanna AU - Campitiello R AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Gerli, Veronica AU - Gerli V AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Paolino, Sabrina AU - Paolino S AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Internal Medicine, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), University Hospital Ghent, Gent, Belgium. AD - Department of Rheumatology, University Hospital Ghent, Gent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AUID- ORCID: 0000-0002-5396-0932 AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties, Postgraduate School of Rheumatology, IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy mcutolo@unige.it. LA - eng PT - Journal Article PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM MH - Humans MH - *Mixed Connective Tissue Disease/complications MH - Microscopic Angioscopy/methods MH - Retrospective Studies MH - *Connective Tissue Diseases/complications/diagnosis MH - *Autoimmune Diseases/diagnosis/epidemiology/complications MH - *Scleroderma, Systemic/complications/diagnosis PMC - PMC10351269 OTO - NOTNLM OT - Sjogren's syndrome OT - antiphospholipid syndrome OT - dermatomyositis OT - lupus erythematosus, systemic OT - scleroderma, systemic COIS- Competing interests: None declared. EDAT- 2023/07/15 10:42 MHDA- 2023/07/17 06:42 PMCR- 2023/07/14 CRDT- 2023/07/14 21:03 PHST- 2023/02/14 00:00 [received] PHST- 2023/06/23 00:00 [accepted] PHST- 2023/07/17 06:42 [medline] PHST- 2023/07/15 10:42 [pubmed] PHST- 2023/07/14 21:03 [entrez] PHST- 2023/07/14 00:00 [pmc-release] AID - rmdopen-2023-003071 [pii] AID - 10.1136/rmdopen-2023-003071 [doi] PST - ppublish SO - RMD Open. 2023 Jul;9(3):e003071. doi: 10.1136/rmdopen-2023-003071. PMID- 31431317 OWN - NLM STAT- MEDLINE DCOM- 20200806 LR - 20200806 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 104 DP - 2019 Nov TI - Predictive markers of lymphomagenesis in Sjögren's syndrome: From clinical data to molecular stratification. PG - 102316 LID - S0896-8411(19)30511-6 [pii] LID - 10.1016/j.jaut.2019.102316 [doi] AB - Sjögren's syndrome (SS) is a chronic systemic autoimmune disease, affecting predominantly the exocrine glands, a large array of systemic manifestations and high risk of lymphoma development. The latter constitutes the major adverse outcome of SS contributing in the increased morbidity and mortality of the disease. The vast majority of lymphomas in SS are B-cell non-Hodgkin's lymphomas (NHL), primarily indolent mucosa-associated lymphoid tissue (MALT) lymphomas, followed by nodal marginal zone lymphomas (NMZL) and diffuse large B cell lymphomas (DLBCL). In the last 3 decades and due to the adverse impact of NHL in disease outcome, an effort has been undertaken to identify markers and models predicting patients with SS at high risk for lymphoma development. Several epidemiological, clinical, laboratory and histological parameters, some of which are evident at the time of SS diagnosis, were proved to independently predict the development of NHL. These include salivary gland enlargement, skin vasculitis/purpura, glomerulonephritis, peripheral neuropathy, Raynaud's phenomenon, lymphadenopathy, splenomegaly, cytopenias, hypocomplementemia, cryoglobulinemia, rheumatoid factor, anti-Ro/La autoantibodies, hypergammaglobulinemia, serum monoclonal gammopathy, biopsy focus score and organization of lymphocytic infiltrates in the salivary glands into ectopic germinal centers. Prediction models combining some of the afore-mentioned predictors have also been described. However, the identification of specific and sensitive molecular biomarkers, related to the process of lymphomagenesis is still pending. Recently, we described a novel biomarker the miR200b-5p micro-RNA. Low levels of this miRNA in the minor salivary glands, appears to discriminate with high specificity and sensitivity the SS patients who have from those who do not have NHL. miR200b-5p, being expressed years before the clinical onset of NHL, independently predicts NHL development with a predictive value higher than the previously published multifactorial models and has a possible role in the monitoring of therapeutic response. Thus, it is a strong candidate for the identification and follow-up of patients at risk. CI - Copyright © 2019 Elsevier Ltd. All rights reserved. FAU - Kapsogeorgou, Efstathia K AU - Kapsogeorgou EK AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece; Academic Joint Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Greece. Electronic address: ekapso@med.uoa.gr. FAU - Voulgarelis, Michael AU - Voulgarelis M AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece; Academic Joint Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Greece. Electronic address: mvoulgar@med.uoa.gr. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Greece; Academic Joint Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Greece. Electronic address: agtzi@med.uoa.gr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20190817 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 SB - IM MH - Carcinogenesis MH - Humans MH - *Lymphoma/diagnosis/epidemiology/immunology MH - Risk Factors MH - *Sjogren's Syndrome/diagnosis/epidemiology/immunology OTO - NOTNLM OT - Non-hodgkin's lymphoma OT - Predictive biomarkers OT - Sjögren's syndrome OT - miR200b-5p EDAT- 2019/08/23 06:00 MHDA- 2020/08/07 06:00 CRDT- 2019/08/22 06:00 PHST- 2019/08/02 00:00 [received] PHST- 2019/08/03 00:00 [accepted] PHST- 2019/08/23 06:00 [pubmed] PHST- 2020/08/07 06:00 [medline] PHST- 2019/08/22 06:00 [entrez] AID - S0896-8411(19)30511-6 [pii] AID - 10.1016/j.jaut.2019.102316 [doi] PST - ppublish SO - J Autoimmun. 2019 Nov;104:102316. doi: 10.1016/j.jaut.2019.102316. Epub 2019 Aug 17. PMID- 39313554 OWN - NLM STAT- MEDLINE DCOM- 20250530 LR - 20250530 IS - 1530-0447 (Electronic) IS - 0031-3998 (Linking) VI - 97 IP - 6 DP - 2025 May TI - Profile of patients with Juvenile Dermatomyositis and Anti-MDA5 autoantibodies. PG - 2020-2028 LID - 10.1038/s41390-024-03551-3 [doi] AB - BACKGROUND: Anti-MDA5 autoantibody-positive dermatomyositis (MDA5-DM) is associated with clinically amyopathic forms and rapidly progressive interstitial lung disease (ILD); however, data in children are limited. In this study, we described our cohort of anti-MDA5-positive juvenile DM (MDA5-JDM) from a tertiary care center in North India. METHODS: We performed a retrospective analysis of children with MDA5-JDM who were diagnosed and followed up at our center and compared them with our anti-MDA5-negative cohort. We also compared the published literature on MDA5-DM with the juvenile cohort. RESULTS: Of 66 children with JDM who underwent testing for MSA, 10(15.5%) had anti-MDA5 positivity. The mean age at onset of clinical manifestations was 8.4 years; male: female ratio was 7:3. Five of nine patients who underwent screening HRCT chest had ILD; one amongst them had a fatal rapidly progressive disease. Children with MDA5-JDM had significantly more arthralgia/arthritis (p = 0.006) and ILD (p = 0.0005) compared to anti-MDA5 negative JDM in our cohort. While MDA5-DM had high rates of Raynaud's phenomenon (p = 0.04) and pulmonary involvement (p = 0.001), juvenile patients had a higher prevalence of constitutional symptoms (p = 0.01), skin manifestations (p = 0.003), arthritis (p = 0.001), and muscle weakness (p = 0.001). CONCLUSIONS: Arthritis and ILD are commonly seen with MDA5-JDM; however, the frequency of ILD and clinically amyopathic forms are less common compared to adult counterparts. IMPACT: The frequency of anti-MDA5 antibodies in a North Indian cohort of JDM is much lower (15.5%) compared to adult studies in dermatomyositis from Southeast Asia (~25%). Incidence of interstitial lung disease (ILD) and arthritis is high in anti-MDA5 autoantibody-positive JDM. Rates of a rapidly progressive form of ILD and clinically amyopathic dermatomyositis are much lower in children compared to adults with anti-MDA5-associated dermatomyositis. CI - © 2024. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc. FAU - Vignesh, Pandiarajan AU - Vignesh P AUID- ORCID: 0000-0001-8490-5584 AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. vigimmc@gmail.com. FAU - Nadig, Pallavi L AU - Nadig PL AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Basu, Suprit AU - Basu S AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Reddy, Shravani AU - Reddy S AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Tyagi, Reva AU - Tyagi R AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Dod, Aditya AU - Dod A AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Kumrah, Rajni AU - Kumrah R AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Garg, Ravinder AU - Garg R AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Sharma, Saniya AU - Sharma S AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Dhaliwal, Manpreet AU - Dhaliwal M AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Pilania, Rakesh Kumar AU - Pilania RK AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Jindal, Ankur AU - Jindal A AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Suri, Deepti AU - Suri D AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Rawat, Amit AU - Rawat A AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Singh, Surjit AU - Singh S AD - Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. LA - eng PT - Journal Article DEP - 20240923 PL - United States TA - Pediatr Res JT - Pediatric research JID - 0100714 RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - 0 (Autoantibodies) RN - EC 3.6.1.- (IFIH1 protein, human) SB - IM MH - Humans MH - *Interferon-Induced Helicase, IFIH1/immunology MH - *Dermatomyositis/immunology/blood/complications/diagnosis MH - *Autoantibodies/blood/immunology MH - Male MH - Female MH - Child MH - Retrospective Studies MH - Adolescent MH - Lung Diseases, Interstitial/immunology MH - Child, Preschool MH - India MH - Age of Onset COIS- Competing interests: The authors declare no competing interests. Ethical approval: This study was performed in line with the principles of the Declaration of Helsinki. The Departmental Review Board of the Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh approved the manuscript (DRB-102-22, dt. 09 January 2023). Informed consent: Informed consent was obtained from the parents of the children included in this study. A written informed consent was obtained from the parent of the child depicted in Fig. 2. EDAT- 2024/09/24 00:42 MHDA- 2025/05/30 08:31 CRDT- 2024/09/23 23:17 PHST- 2024/01/31 00:00 [received] PHST- 2024/08/07 00:00 [accepted] PHST- 2024/07/27 00:00 [revised] PHST- 2025/05/30 08:31 [medline] PHST- 2024/09/24 00:42 [pubmed] PHST- 2024/09/23 23:17 [entrez] AID - 10.1038/s41390-024-03551-3 [pii] AID - 10.1038/s41390-024-03551-3 [doi] PST - ppublish SO - Pediatr Res. 2025 May;97(6):2020-2028. doi: 10.1038/s41390-024-03551-3. Epub 2024 Sep 23. PMID- 32963114 OWN - NLM STAT- MEDLINE DCOM- 20210922 LR - 20210922 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 7 IP - 1 DP - 2020 Sep TI - Cutaneous vasculitis in SLE. LID - 10.1136/lupus-2020-000411 [doi] LID - e000411 AB - OBJECTIVES: We determined the temporal association between clinical and serological disease manifestations and development of cutaneous small vessel vasculitis in a large prospective multiethnic cohort. METHODS: Patients with SLE diagnosed according to the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria or the revised classification criteria as defined by the American College of Rheumatology (ACR) were enrolled in the Hopkins Lupus Cohort. Cutaneous small vessel vasculitis was determined as a component of the Systemic Lupus Erythematosus Disease Activity Index. SLE-associated cutaneous small vessel vasculitis lesions were reported clinically. They presented as punctate lesions, palpable purpura, tender erythematous plaques or macules with or without necrosis. No histopathological diagnosis was pursued to confirm the diagnosis of vasculitis or to differentiate it from other causes of digital lesions in patients with SLE. Disease manifestations that preceded the first occurrence of cutaneous small vessel vasculitis lesions were analysed using Kaplan-Meier. Cox regression analysis was used to assess the relationship between baseline clinical and immunological manifestations and the development of cutaneous small vessel vasculitis. We adjusted for gender, race and age at SLE diagnosis. RESULTS: A total of 2580 patients were studied: 52.4% were Caucasian and 39.4% were African-American. The mean age of the cohort was 45.5±14.5 years. The mean years of cohort follow-up was 7.9±7.6. Cutaneous small vessel vasculitis was observed in 449 (17.3%). The mean time to cutaneous vasculitis after SLE diagnosis was 4.78 years (95% CI 3.96 to 5.60). At least 159 (35%) patients had recurrences of cutaneous vasculitis lesions. Discoid rash, Raynaud's phenomenon, myositis, anaemia, Coombs' positivity, leucopenia, anti-Smith and anti-RNP (Ribonucleoprotein) were significantly associated with the development of cutaneous vasculitis. The SLICC/ACR Damage Index score was higher in patients with cutaneous vasculitis compared with those without cutaneous vasculitis. CONCLUSIONS: Cutaneous vasculitis is frequent (17.3%) and often recurrent (35%). African-Americans are at higher risk of developing cutaneous small vessel vasculitis than Caucasians. Clinical presentations such as myositis and haematological manifestations are predictors of cutaneous vasculitis development. The presence of cutaneous vasculitis is associated with increased organ damage. CI - © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Kallas, Romy AU - Kallas R AUID- ORCID: 0000-0001-6571-5662 AD - Division of Rheumatology, Johns Hopkins Medicine, Baltimore, Maryland, USA kallasr@mlhs.org. FAU - Goldman, Daniel AU - Goldman D AD - Department of Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA. FAU - Petri, Michelle A AU - Petri MA AUID- ORCID: 0000-0003-1441-5373 AD - Division of Rheumatology, Johns Hopkins Medicine, Baltimore, Maryland, USA. LA - eng GR - R01 AR069572/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/complications MH - Male MH - Middle Aged MH - Neoplasm Recurrence, Local MH - Prospective Studies MH - United States MH - *Vasculitis/etiology PMC - PMC7509964 OTO - NOTNLM OT - autoantibodies OT - inflammation OT - lupus erythematosus OT - systemic COIS- Competing interests: None declared. EDAT- 2020/09/24 06:00 MHDA- 2021/09/23 06:00 PMCR- 2020/09/22 CRDT- 2020/09/23 06:28 PHST- 2020/04/24 00:00 [received] PHST- 2020/08/17 00:00 [revised] PHST- 2020/08/24 00:00 [accepted] PHST- 2020/09/23 06:28 [entrez] PHST- 2020/09/24 06:00 [pubmed] PHST- 2021/09/23 06:00 [medline] PHST- 2020/09/22 00:00 [pmc-release] AID - 7/1/e000411 [pii] AID - lupus-2020-000411 [pii] AID - 10.1136/lupus-2020-000411 [doi] PST - ppublish SO - Lupus Sci Med. 2020 Sep;7(1):e000411. doi: 10.1136/lupus-2020-000411. PMID- 31746507 OWN - NLM STAT- MEDLINE DCOM- 20210104 LR - 20210104 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 34 IP - 3 DP - 2020 Mar TI - Significance of the angiotensin I/angiotensin II/angiotensin-(1-7) axis in the pathogenesis of systemic sclerosis. PG - 558-564 LID - 10.1111/jdv.16103 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is a multisystemic disease with an extensive microvasculopathy. Previously, disturbances in plasma levels of angiotensin II (Ang II) and its antagonistic angiotensin-(1-7) (Ang-(1-7)) were found in patients with SSc. Their significance in a pathogenesis of SSc stays unclear due to discrepancies of earlier studies. OBJECTIVES: To evaluate a significance of disturbances in production pathway of angiotensins in a development of SSc. METHODS: There were enrolled 27 patients with established SSc, 23 subjects with very early SSc and 23 healthy controls. The diagnosis of SSc was established in patients who met EULAR/ACR 2013 classification criteria. Very early SSc described patients with Raynaud's phenomenon having SSc-specific antinuclear antibodies and SSc-like abnormalities in nailfold videocapillaroscopy. Patients were submitted to evaluation of internal organ involvement and blood sampling to assay plasma levels of angiotensin I, angiotensin II and angiotensin-(1-7) with ELISA technique. RESULTS: Plasma level of angiotensin-(1-7) was significantly reduced in both SSc group (median = 47.2 pg/mL; P < 0.001) and ones with very early SSc (median = 102.7 pg/mL; P = 0.002) when compared to healthy controls (median = 176.1 pg/mL). A tendency to higher than in control group (median = 214 pg/mL) plasma level of angiotensin I was seen in SSc group (median = 392 pg/mL; P = 0.059). Differences in plasma level of angiotensin II were insignificant between all study groups. Those disturbances produced unfavourable angiotensin-(1-7)/angiotensin II (%) ratio in both groups of patients, which achieved statistical significance in subjects with established SSc (P < 0.001). Production pathway of angiotensins showed a dependence on a subtype of SSc, immune profile and a presence of interstitial lung disease. CONCLUSIONS: Production of angiotensin-(1-7) was significantly reduced in both SSc patients and those ones with very early SSc, although a significant imbalance between angiotensin II and angiotensin-(1-7) occurred only in subjects with established disease. CI - © 2019 European Academy of Dermatology and Venereology. FAU - Miziołek, B AU - Miziołek B AUID- ORCID: 0000-0001-8388-4419 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Bergler-Czop, B AU - Bergler-Czop B AUID- ORCID: 0000-0003-3788-5984 AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Kucharz, E AU - Kucharz E AD - Department of Internal Medicine Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Kotyla, P AU - Kotyla P AD - Department of Internal Medicine Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Kopeć-Mędrek, M AU - Kopeć-Mędrek M AD - Department of Internal Medicine Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Widuchowska, M AU - Widuchowska M AD - Department of Internal Medicine Rheumatology and Clinical Immunology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. FAU - Sieńczyk, M AU - Sieńczyk M AUID- ORCID: 0000-0002-5528-5264 AD - Faculty of Chemistry, Division of Medicinal Chemistry and Microbiology, Wroclaw University of Science and Technology, Wrocław, Poland. FAU - Brzezińska-Wcisło, L AU - Brzezińska-Wcisło L AD - Department of Dermatology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland. LA - eng PT - Journal Article DEP - 20191218 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 RN - 0 (Peptide Fragments) RN - 11128-99-7 (Angiotensin II) RN - 9041-90-1 (Angiotensin I) RN - IJ3FUK8MOF (angiotensin I (1-7)) SB - IM MH - Adult MH - Aged MH - Angiotensin I/blood/*physiology MH - Angiotensin II/blood/*physiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Peptide Fragments/blood/*physiology MH - Scleroderma, Systemic/blood/*etiology MH - Young Adult EDAT- 2019/11/21 06:00 MHDA- 2021/01/05 06:00 CRDT- 2019/11/21 06:00 PHST- 2019/08/07 00:00 [received] PHST- 2019/11/13 00:00 [accepted] PHST- 2019/11/21 06:00 [pubmed] PHST- 2021/01/05 06:00 [medline] PHST- 2019/11/21 06:00 [entrez] AID - 10.1111/jdv.16103 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2020 Mar;34(3):558-564. doi: 10.1111/jdv.16103. Epub 2019 Dec 18. PMID- 26161156 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150710 LR - 20220311 IS - 1874-3129 (Print) IS - 1874-3129 (Electronic) IS - 1874-3129 (Linking) VI - 9 DP - 2015 TI - Clinical Characteristics and Outcome of Primary Sjogren's Syndrome: A Large Asian Indian Cohort. PG - 36-45 LID - 10.2174/1874312901409010036 [doi] AB - OBJECTIVE: To characterise the clinical features, immunological profile and outcome in a cohort of Asian Indian patients with primary Sjögren's syndrome (SS). METHODS: Electronic medical records from a tertiary care teaching hospital in south India were screened for SS between 2004 and 2011. Patients fulfilling American European Consensus group (AECG) 2002 or American College of Rheumatology (ACR) 2012 classification criteria were included. Agglomerative hierarchical cluster analysis to identify patterns of associations between clinical and immunological features was done. Multivariate logistic regression to identify predictors of major systemic involvement was performed. Data on treatment and outcome were retrieved from electronic records. RESULTS: Of 423 patients suspected to have SS, 332 fulfilled inclusion criteria. Only 8.3% of patients complained of sicca symptoms on their own at initial presentation. Younger age of onset, higher female to male ratio, paucity of cryoglobulinemia, Raynaud's phenomenon and hyperglobulinemia were unique to this cohort. Cluster analysis revealed two subsets: The first cluster comprised of patients having a major systemic illness with high antibody titers and the second comprised of seronegative patients with mild disease. Over a third of SS cases had severe systemic manifestations necessitating treatment with immunosuppressants. In multivariate logistic regression analysis, anti-Ro and anti-La antibody positivity was associated with higher odds for systemic disease features (OR=2.67, P=0.03 and OR=3.25, P=0.003, respectively) whereas chronic pain was associated with lower odds (OR=0.4, p=0.032). Clinical improvement including symptomatic benefit in sicca and musculoskeletal features was noted with immunomodulators in the majority. CONCLUSION: Our cohort of patients with SS has characteristic clinical features; some of them are in contrast with previous observations reported in European patients. This cohort consisted of two distinct patient clusters. The first cluster was associated with major systemic illness and high antibody titers, where as the second cluster comprised of seronegative patients with mild disease. Association of antibody positivity with systemic features was further confirmed on logistic regression analysis. FAU - Sandhya, Pulukool AU - Sandhya P AD - Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore-632004, Tamil Nadu, India. FAU - Jeyaseelan, Lakshmanan AU - Jeyaseelan L AD - Department of Biostatistics, Christian Medical College and Hospital, Vellore-632004, Tamil Nadu, India. FAU - Scofield, Robert Hal AU - Scofield RH AD - Arthritis & Clinical Immunology Program, Oklahoma Medical Research Foundation, Endocrinology, Metabolism and Diabetes, Department of Medicine, University of Oklahoma Health Sciences Center and Department of Veterans Affairs Medical Center, Oklahoma City, OK, USA. FAU - Danda, Debashish AU - Danda D AD - Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore-632004, Tamil Nadu, India. LA - eng PT - Journal Article DEP - 20150626 PL - United Arab Emirates TA - Open Rheumatol J JT - The open rheumatology journal JID - 101480507 PMC - PMC4493630 OTO - NOTNLM OT - Antibodies OT - India OT - Sj gren s syndrome OT - cluster analysis OT - immunosuppressive agents OT - logistic regression analysis EDAT- 2015/07/15 06:00 MHDA- 2015/07/15 06:01 PMCR- 2015/01/01 CRDT- 2015/07/11 06:00 PHST- 2014/12/22 00:00 [received] PHST- 2015/02/23 00:00 [revised] PHST- 2015/02/27 00:00 [accepted] PHST- 2015/07/11 06:00 [entrez] PHST- 2015/07/15 06:00 [pubmed] PHST- 2015/07/15 06:01 [medline] PHST- 2015/01/01 00:00 [pmc-release] AID - TORJ-9-36 [pii] AID - 10.2174/1874312901409010036 [doi] PST - epublish SO - Open Rheumatol J. 2015 Jun 26;9:36-45. doi: 10.2174/1874312901409010036. eCollection 2015. PMID- 19355827 OWN - NLM STAT- MEDLINE DCOM- 20090730 LR - 20161125 IS - 1557-9077 (Electronic) IS - 1050-7256 (Linking) VI - 19 IP - 4 DP - 2009 Apr TI - Retrobulbar 99mTc-diethylenetriamine-pentaacetic-acid uptake may predict the effectiveness of immunosuppressive therapy in Graves' ophthalmopathy. PG - 375-80 LID - 10.1089/thy.2008.0298 [doi] AB - BACKGROUND: In Graves' ophthalmopathy (GO), only patients with immunologically active disease respond to immunosuppressive therapy. Previous studies and theoretical considerations suggest that elevated orbital (99m)Tc-diethylenetriamine-pentaacetic-acid (DTPA) single photon emission computed tomography (SPECT) reflects inflammatory disease activity. We studied whether corticosteroid treatment causes a substantial decrease in DTPA uptake in GO, a result consistent with successful immunosuppressive treatment of GO and referred to as a favorable treatment outcome. METHODS: One hundred fourteen orbits in 57 patients with active GO (CAS >or= 4) were entered into the study. All patients received corticosteroid treatment. Orbital DTPA uptakes were numerically quantified for the entire orbit as well as the anterior and posterior segments separately. DTPA SPECT was performed before, and 2 to 9 months after the initiation of immunosuppressive treatment. The normal range for DTPA uptake was established in 34 orbits of 17 patients who were being worked up for Raynaud's phenomenon and had no thyroid disease. RESULTS: The mean DTPA uptake of the 114 orbits of GO patients was higher prior to corticosteroid therapy than after this treatment (11.03 +/- 4.26 MBq/cm(3) and 9.84 +/- 3.51 MBq/cm(3), respectively, p < 0.001) but a substantial decline in DTPA uptake was seen in only 39.5% of GO patients. The positive predictive value of an initial DTPA >12.28 MBq/cm(3) for a substantial decline in DTPA uptake (favorable treatment outcome) was 76%, while a negative predictive value of a pretreatment DTPA 50 years). RESULTS: Four hundred and forty-five patients were included. Renal disease and cutaneous manifestations were more frequent in the juvenile-onset group at disease onset. During follow-up, juvenile-onset group presented a higher incidence of renal disease, malar rash, Raynaud's phenomenon, cutaneous vasculitis, and neuropsychiatric manifestations than the other two groups. Arthritis and lymphopoenia were more frequent in the adult-onset group. Arterial hypertension and neoplasm were more frequent in the late-onset group. Low serum complement, anti-dsDNA, anti-U1RNP and anti-Sm antibodies were more common in the juvenile-onset group. Patients with late-onset SLE had more damage accrual. Thirty-seven patients (8.3%) died during the study. All-cause mortality was significantly higher in the late-onset group. Age at disease onset >50 years was an independent risk factor for damage accrual (OR, 2.2; 95%CI, 1.1-4.6; p=0.029) and mortality (OR, 2.6; 95%CI, 1.1-6.3; p=0.03). CONCLUSIONS: We found significant differences in clinical and serological profiles between juvenile, adult and late-onset SLE. The most significant of which was a higher prevalence of neuropsychiatric and renal complications as well as different autoantibody signatures for the juvenile-onset group. FAU - Martínez-Barrio, J AU - Martínez-Barrio J AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. juliamartinezbarrio@gmail.com. FAU - Ovalles-Bonilla, J G AU - Ovalles-Bonilla JG AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - López-Longo, F J AU - López-Longo FJ AD - Department of Rheumatology, Gregorio Marañón General Hospital; and Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain. FAU - González, C M AU - González CM AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Montoro, M AU - Montoro M AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Valor, L AU - Valor L AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Martínez, L P AU - Martínez LP AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Nieto, J C AU - Nieto JC AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Hinojosa-Dávila, M C AU - Hinojosa-Dávila MC AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Bello, N AU - Bello N AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Monteagudo, I AU - Monteagudo I AD - Unit of Paediatric Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Naredo, E AU - Naredo E AD - Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain. FAU - Carreño, L AU - Carreño L AD - Department of Rheumatology, Gregorio Marañón General Hospital; and Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain. LA - eng PT - Journal Article DEP - 20151117 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Age Distribution MH - Age of Onset MH - Aged MH - *Autoantibodies/blood/classification MH - Child MH - Female MH - Follow-Up Studies MH - Humans MH - *Hypertension/epidemiology/etiology MH - *Lupus Erythematosus, Systemic/classification/diagnosis/ethnology/immunology/mortality MH - Male MH - Monitoring, Immunologic/methods MH - *Neoplasms/epidemiology/etiology MH - Prevalence MH - Risk Factors MH - Spain/epidemiology MH - Survival Analysis EDAT- 2015/11/18 06:00 MHDA- 2016/03/25 06:00 CRDT- 2015/11/18 06:00 PHST- 2014/08/29 00:00 [received] PHST- 2015/03/23 00:00 [accepted] PHST- 2015/11/18 06:00 [entrez] PHST- 2015/11/18 06:00 [pubmed] PHST- 2016/03/25 06:00 [medline] AID - 8596 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2015 Nov-Dec;33(6):788-94. Epub 2015 Nov 17. PMID- 41594275 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260128 LR - 20260131 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 16 IP - 2 DP - 2026 Jan 17 TI - Interstitial Pneumonia with Autoimmune Features from the Rheumatologists' Perspective; Single Center Experience. LID - 10.3390/diagnostics16020299 [doi] LID - 299 AB - Background/Objectives: Interstitial pneumonia with autoimmune features (IPAF) is a recently defined entity characterized by interstitial lung disease (ILD) with clinical, serological, and radiological features suggestive of autoimmunity that do not fulfil the criteria for a defined connective tissue disease (CTD). This study aimed to evaluate the clinical characteristics, treatment modalities, and outcomes of patients with IPAF in a tertiary referral center. Methods: We retrospectively analyzed 72 patients who fulfilled the IPAF classification criteria. Demographic, clinical, serological, radiological, pulmonary function, treatment, and survival data were collected and evaluated. Logistic regression analysis was performed to identify factors associated with mortality. Results: The cohort consisted of 62.5% female patients, with a mean age of 62.7 (SD, 10.4) years at diagnosis. The most frequent radiological pattern was nonspecific interstitial pneumonia (83.3%). Raynaud's phenomenon (6.9%) and arthritis (2.8%) were the most common rheumatological manifestations. Antinuclear antibodies positivity at titers ≥1:320 was observed in 27.8% of patients. Azathioprine was the most frequently prescribed agent (20.8%), followed by mycophenolate mofetil (11.1%). After a median follow-up of 30.1 months (IQR, 52.8), 16 patients (22.22%) died, with a 5-year survival rate of 70%. Glucocorticoid therapy at doses ≥20 mg/day was independently associated with increased mortality (OR 6.13 (95% CI 1.17-32.21). Conclusions: IPAF predominantly affects middle-aged females. Glucocorticoid use at doses ≥20 mg/day was associated with mortality; however, this observational association may reflect underlying disease severity rather than a causal effect of high-dose treatment. Further prospective studies are needed to optimize management strategies in patients with IPAF. FAU - Uslu, Emine AU - Uslu E AUID- ORCID: 0000-0002-4717-3000 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Sahin, Didem AU - Sahin D AUID- ORCID: 0000-0003-3558-5400 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Ilbay, Ahmet AU - Ilbay A AUID- ORCID: 0000-0002-7645-9462 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Yilmaz, Recep AU - Yilmaz R AUID- ORCID: 0000-0001-8806-8621 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Gaydan, Abdulbaki AU - Gaydan A AUID- ORCID: 0009-0002-4917-6106 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Govec Giynas, Nilgun AU - Govec Giynas N AUID- ORCID: 0009-0001-9636-2406 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Usta, Ahmet AU - Usta A AUID- ORCID: 0000-0003-3149-9505 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Mahmutoglu, Yeter AU - Mahmutoglu Y AUID- ORCID: 0009-0004-3691-5031 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Aksoy, Rahime AU - Aksoy R AUID- ORCID: 0000-0001-8433-3627 AD - Department of Hematology, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Sezer, Serdar AU - Sezer S AUID- ORCID: 0000-0001-5401-5599 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Yayla, Mucteba Enes AU - Yayla ME AUID- ORCID: 0000-0002-5998-6703 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Kul, Melahat AU - Kul M AUID- ORCID: 0000-0001-9843-5114 AD - Department of Radiology, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Gursoy Coruh, Aysegul AU - Gursoy Coruh A AUID- ORCID: 0000-0002-8638-8688 AD - Department of Radiology, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Uzun, Caglar AU - Uzun C AUID- ORCID: 0000-0002-8441-2912 AD - Department of Radiology, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Us, Ebru AU - Us E AUID- ORCID: 0000-0001-9705-1792 AD - Department of Medical Microbiology, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Ozdemir Kumbasar, Ozlem AU - Ozdemir Kumbasar O AUID- ORCID: 0000-0003-4599-1461 AD - Department of Pulmonology, Faculty of Medicine, Ankara University, Ankara 06620, Turkey. FAU - Ates, Askin AU - Ates A AUID- ORCID: 0000-0003-1966-3333 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. FAU - Turgay, Tahsin Murat AU - Turgay TM AUID- ORCID: 0000-0001-5302-4485 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Ankara University, Ankara 06230, Turkey. LA - eng PT - Journal Article DEP - 20260117 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC12840511 OTO - NOTNLM OT - autoimmunity OT - cyclophosphamide OT - glucocorticoids OT - interstitial lung disease COIS- The authors declare no conflicts of interest. EDAT- 2026/01/28 07:47 MHDA- 2026/01/28 07:48 PMCR- 2026/01/17 CRDT- 2026/01/28 01:01 PHST- 2025/12/04 00:00 [received] PHST- 2025/12/28 00:00 [revised] PHST- 2026/01/09 00:00 [accepted] PHST- 2026/01/28 07:48 [medline] PHST- 2026/01/28 07:47 [pubmed] PHST- 2026/01/28 01:01 [entrez] PHST- 2026/01/17 00:00 [pmc-release] AID - diagnostics16020299 [pii] AID - diagnostics-16-00299 [pii] AID - 10.3390/diagnostics16020299 [doi] PST - epublish SO - Diagnostics (Basel). 2026 Jan 17;16(2):299. doi: 10.3390/diagnostics16020299. PMID- 37338524 OWN - NLM STAT- MEDLINE DCOM- 20230711 LR - 20230718 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 32 IP - 8 DP - 2023 Jul TI - Investigating the potential associated factors and developing a risk model for interstitial lung disease in Chinese patients with systemic lupus erythematosus. PG - 993-1000 LID - 10.1177/09612033231182747 [doi] AB - OBJECTIVE: This study aimed to explore the risk factors for interstitial lung disease (ILD) in Chinese patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: This study recruited 40 SLE patients with ILD (SLE-ILD) and 40 SLE patients without ILD (SLE-non-ILD). Clinical data were collected from all patients, including basic clinical characteristics, affected organ systems, biochemical indexes, autoantibodies, and immunocytes. RESULTS: Compared with the SLE-non-ILD patients, SLE-ILD patients presented older age (p < 0.001), dry cough (p = 0.006), "velcro-like" crackles (p = 0.021), Raynaud's phenomenon (p = 0.040), elevated complement 3 (C3) level (p = 0.044), and lower SLE disease activity index score (p = 0.013) and cluster of difference-3 cell count (p = 0.043). Multivariate logistic regression analysis showed that older age (p < 0.001, odds ratio [OR]: 1.212), female sex (p = 0.022, OR: 37.075), renal involvement (p = 0.011, OR: 20.039), C3 level (p = 0.037, OR: 63.126), immunoglobulin (Ig) M level (p = 0.005, OR: 5.082), and positive anti-U1 small ribonucleoprotein antibody (anti-nRNP) result (p = 0.003, OR: 19.886) were independent ILD risk factors in SLE patients. Consequently, the ILD risk model in patients with SLE was constructed based on statistically significant variables from the multivariate logistic regression analysis, which significantly correlated with ILD risk, with an area under the curve of 0.887 (95% confidence interval: 0.815-0.960) using receiver operating characteristic curve analysis. CONCLUSION: Age, female sex, renal involvement, C3 level, IgM level, and a positive anti-nRNP result are independent risk factors for ILD. Furthermore, their combination model is closely associated with an increased ILD risk in Chinese patients with SLE. FAU - Liu, Yuan AU - Liu Y AD - Department of Rheumatology, Liuzhou People's Hospital, Guangxi Medical University, Liuzhou, China. AD - Department of Rheumatology, First Affiliated Hospital of Baotou Medical College, Baotou, China. RINGGOLD: 540473 FAU - Lu, Fuai AU - Lu F AD - Department of Rheumatology, First Affiliated Hospital of Baotou Medical College, Baotou, China. RINGGOLD: 540473 FAU - Liao, Hongli AU - Liao H AD - Department of Rheumatology, Liuzhou People's Hospital, Guangxi Medical University, Liuzhou, China. FAU - Wang, Le AU - Wang L AD - Department of Rheumatology, Liuzhou People's Hospital, Guangxi Medical University, Liuzhou, China. FAU - Lu, Pei AU - Lu P AUID- ORCID: 0000-0003-4922-135X AD - Department of Oncology, Liuzhou People's Hospital, Guangxi Medical University, Liuzhou, China. LA - eng PT - Journal Article DEP - 20230620 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Autoantibodies) RN - 0 (Complement C3) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Female MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - East Asian People MH - *Lung Diseases, Interstitial/etiology/complications MH - Autoantibodies MH - Risk Factors MH - Complement C3 MH - Antibodies, Antinuclear OTO - NOTNLM OT - Systemic lupus erythematosus OT - biochemical index OT - clinical features OT - interstitial lung disease OT - risk model EDAT- 2023/06/20 13:10 MHDA- 2023/07/11 06:42 CRDT- 2023/06/20 10:53 PHST- 2023/07/11 06:42 [medline] PHST- 2023/06/20 13:10 [pubmed] PHST- 2023/06/20 10:53 [entrez] AID - 10.1177/09612033231182747 [doi] PST - ppublish SO - Lupus. 2023 Jul;32(8):993-1000. doi: 10.1177/09612033231182747. Epub 2023 Jun 20. PMID- 27312381 OWN - NLM STAT- MEDLINE DCOM- 20170901 LR - 20260127 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 46 IP - 2 DP - 2016 Oct TI - Digital ulcers and cutaneous subsets of systemic sclerosis: Clinical, immunological, nailfold capillaroscopy, and survival differences in the Spanish RESCLE Registry. PG - 200-208 LID - S0049-0172(16)30041-5 [pii] LID - 10.1016/j.semarthrit.2016.04.007 [doi] AB - OBJECTIVE: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. METHODS: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE). Demographics, organ involvement, autoimmunity features, nailfold capillary pattern, survival time, and causes of death were analyzed to identify DU related characteristics and survival of the entire series and according to the following cutaneous subsets-diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc). RESULTS: Out of 1326, 552 patients enrolled in the RESCLE registry had prior or current DU, 88% were women, the mean age was 50 ± 16 years, and the mean disease duration from first SSc symptom was 7.6 ± 9.6 years. Many significant differences were observed in the univariate analysis between patients with and without prior/current DU. Multivariate analysis identified that history of prior/current DU in patients with SSc was independently associated to younger age at SSc diagnosis, diffuse cutaneous SSc, peripheral vascular manifestations such Raynaud's phenomenon, telangiectasia, and acro-osteolysis but no other vascular features such as pulmonary arterial hypertension or scleroderma renal crisis. DU was also associated to calcinosis cutis, interstitial lung disease, as well as worse survival. Multivariate analysis performed in the cutaneous subsets showed that prior/current DU were independently associated: (1) in dcSSc, to younger age at SSc diagnosis, presence of telangiectasia and calcinosis and rarely a non-SSc pattern on nailfold capillaroscopy; (2) in lcSSc, to younger age at SSc diagnosis, presence of Raynaud's phenomenon as well as calcinosis cutis, interstitial lung disease, and higher incidence of death from all causes; and (3) in ssSSc, to younger age at first SSc symptom and greater incidence of death from all causes. CONCLUSIONS: Digital ulcers develop in patients with SSc younger at diagnosis, mainly in patients with dcSSc and lcSSc, and they are associated to other peripheral vascular manifestations such as Raynaud's phenomenon, telangiectasia, and acro-osteolysis but also to calcinosis, and interstitial lung disease. History of DU in SSc leads to worse survival, also noticeable for lcSSc and ssSSc subsets but not for dcSSc patients. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Tolosa-Vilella, Carles AU - Tolosa-Vilella C AD - Department of Internal Medicine, Corporació Sanitària Universitària Parc Taulí, Universidad Autónoma de Barcelona, Sabadell, Barcelona, Spain. Electronic address: ctolosa@tauli.cat. FAU - Morera-Morales, Maria Lluisa AU - Morera-Morales ML AD - Department of Internal Medicine, Corporació Sanitària Universitària Parc Taulí, Universidad Autónoma de Barcelona, Sabadell, Barcelona, Spain. FAU - Simeón-Aznar, Carmen Pilar AU - Simeón-Aznar CP AD - Department of Internal Medicine, Hospital Valld'Hebron, Barcelona, Spain. FAU - Marí-Alfonso, Begoña AU - Marí-Alfonso B AD - Department of Internal Medicine, Corporació Sanitària Universitària Parc Taulí, Universidad Autónoma de Barcelona, Sabadell, Barcelona, Spain. FAU - Colunga-Arguelles, Dolores AU - Colunga-Arguelles D AD - Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. FAU - Callejas Rubio, José Luis AU - Callejas Rubio JL AD - Unit of Autoimmune Systemic Diseases, Department of Internal Medicine, Hospital Clínico San Cecilio, Granada, Spain. FAU - Rubio-Rivas, Manuel AU - Rubio-Rivas M AD - Department of Internal Medicine, Hospital Universitario de Bellvitge, Barcelona, Spain. FAU - Freire-Dapena, Maika AU - Freire-Dapena M AD - Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Pontevedra, Spain. FAU - Guillén-Del Castillo, Alfredo AU - Guillén-Del Castillo A AD - Department of Internal Medicine, Hospital Valld'Hebron, Barcelona, Spain. FAU - Iniesta-Arandia, Nerea AU - Iniesta-Arandia N AD - Department of Autoimmune Systemic Diseases, Hospital Clinic, Barcelona, Spain. FAU - Castillo-Palma, Maria Jesús AU - Castillo-Palma MJ AD - Unit of Connective Tissue Diseases, Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Spain. FAU - Egurbide-Arberas, Marivi AU - Egurbide-Arberas M AD - Department of Internal Medicine, Hospital de Cruces, Barakaldo, Vizcaya, Spain. FAU - Trapiellla-Martínez, Luis AU - Trapiellla-Martínez L AD - Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. FAU - Vargas-Hitos, José A AU - Vargas-Hitos JA AD - Department of Internal Medicine, Hospital Virgen de las Nieves, Granada, Spain. FAU - Todolí-Parra, José Antonio AU - Todolí-Parra JA AD - Department of Internal Medicine, Hospital La Fe, Valencia, Spain. FAU - Rodriguez-Carballeira, Mónica AU - Rodriguez-Carballeira M AD - Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain. FAU - Marin-Ballvé, Adela AU - Marin-Ballvé A AD - Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. FAU - Pla-Salas, Xavier AU - Pla-Salas X AD - Consorci Hospitalari de Vic, Barcelona, Spain. FAU - Rios-Blanco, Juan José AU - Rios-Blanco JJ AD - Department of Internal Medicine, Hospital La Paz, Madrid, Spain. FAU - Fonollosa-Pla, Vicent AU - Fonollosa-Pla V AD - Department of Internal Medicine, Hospital Valld'Hebron, Barcelona, Spain. CN - RESCLE Investigators, Autoimmune Diseases Study Group (GEAS) LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160518 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Registries MH - Scleroderma, Systemic/*complications/mortality/physiopathology MH - Skin Ulcer/*etiology/mortality/physiopathology MH - Spain/epidemiology MH - Survival Rate OTO - NOTNLM OT - Anti-centromere antibodies OT - Anti-topoisomerase I antibodies OT - Diffuse cutaneous SSc OT - Digital ulcers OT - Limited cutaneous SSc OT - Nailfold capillaroscopy OT - SSc sine scleroderma OT - Survival OT - Systemic sclerosis EDAT- 2016/06/18 06:00 MHDA- 2017/09/02 06:00 CRDT- 2016/06/18 06:00 PHST- 2015/12/23 00:00 [received] PHST- 2016/04/12 00:00 [revised] PHST- 2016/04/28 00:00 [accepted] PHST- 2016/06/18 06:00 [entrez] PHST- 2016/06/18 06:00 [pubmed] PHST- 2017/09/02 06:00 [medline] AID - S0049-0172(16)30041-5 [pii] AID - 10.1016/j.semarthrit.2016.04.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Oct;46(2):200-208. doi: 10.1016/j.semarthrit.2016.04.007. Epub 2016 May 18. PMID- 40806794 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250817 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 15 DP - 2025 Jul 22 TI - Placental Pathology in Obstetric Antiphospholipid Syndrome Beyond Thrombosis: A Case Report and Literature Review. LID - 10.3390/jcm14155172 [doi] LID - 5172 AB - Background: Antiphospholipid syndrome (APS) is one of the highest risk factors for obstetric complications. This article contains a case report of a patient with obstetric APS who experienced fetal loss during their first pregnancy and experienced a successful second pregnancy upon treatment with acetylsalicylic acid (ASA), low-molecular-weight heparin (LMWH), and hydroxychloroquine (HCQ). We compare placental pathology in these two pregnancies and discuss the impact of antiphospholipid antibodies and clinical management on pregnancy outcomes. We also propose methods to monitor obstetric antiphospholipid syndrome (OAPS) patients during pregnancy. Methods: A 26-year-old woman presented with a history of stillbirth at 25 weeks of pregnancy due to placental insufficiency. Before pregnancy, she experienced symptoms suggestive of autoimmune disease (thrombocytopenia, recurrent mouth aphthous ulcers, and Raynaud's phenomenon) but had no diagnosis. Placental dysfunction correlated with the high ratio of sFlt-1/PIGF (soluble fms-like tyrosine kinase 1 and the placental growth factors index). Laboratory tests revealed the presence of antinuclear antibodies (ANAs) and triple positivity for antiphospholipid antibodies (aPLs). Results: Following the initiation of treatment for OAPS and regular monitoring consistent with current guidelines, the patient conceived and successfully delivered a healthy child. Conclusions: Adequate therapy and close monitoring during pregnancy, including clinical observation, placental biomarkers and regular ultrasonography, may help to reduce the risks and increase chances for optimal pregnancy outcomes. Additionally, pathological examination and clinical collaboration are essential components in future pregnancy counseling and should be a part of multidisciplinary management. FAU - Dzirba, Dagmara AU - Dzirba D AUID- ORCID: 0009-0005-1454-9947 AD - Lower Silesian Oncology Center, 53-413 Wroclaw, Poland. FAU - Glinko, Malwina AU - Glinko M AUID- ORCID: 0009-0006-7380-743X AD - Lower Silesian Oncology Center, 53-413 Wroclaw, Poland. FAU - Skoczyńska, Marta AU - Skoczyńska M AD - Department of Rheumatology and Internal Medicine, Lower Silesian Specialist Hospital, 54-049 Wroclaw, Poland. FAU - Gruszecka, Katarzyna AU - Gruszecka K AUID- ORCID: 0009-0007-2330-1729 AD - Department of Rheumatology and Internal Medicine, Lower Silesian Specialist Hospital, 54-049 Wroclaw, Poland. AD - Department of Non-Procedural Clinical Sciences, Faculty of Medicine, University of Technology and Science, 58-376 Wroclaw, Poland. FAU - Trzeszcz, Martyna AU - Trzeszcz M AUID- ORCID: 0000-0002-9971-1093 AD - Department of Pathology and Clinical Cytology, University Clinical Hospital, 50-556 Wroclaw, Poland. FAU - Benedyczak, Adam AU - Benedyczak A AD - 4th Military Clinical Hospital, 50-981 Wroclaw, Poland. FAU - Szmyrka, Magdalena AU - Szmyrka M AD - Department of Rheumatology and Internal Diseases, Faculty of Medicine, Wroclaw Medical University, 50-556 Wroclaw, Poland. LA - eng PT - Case Reports PT - Journal Article DEP - 20250722 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC12347586 OTO - NOTNLM OT - obstetric antiphospholipid syndrome OT - placental insufficiency OT - placental pathology OT - pregnancy monitoring COIS- The authors declare no conflicts of interest. EDAT- 2025/08/14 06:28 MHDA- 2025/08/14 06:29 PMCR- 2025/07/22 CRDT- 2025/08/14 01:14 PHST- 2025/04/05 00:00 [received] PHST- 2025/06/30 00:00 [revised] PHST- 2025/07/09 00:00 [accepted] PHST- 2025/08/14 06:29 [medline] PHST- 2025/08/14 06:28 [pubmed] PHST- 2025/08/14 01:14 [entrez] PHST- 2025/07/22 00:00 [pmc-release] AID - jcm14155172 [pii] AID - jcm-14-05172 [pii] AID - 10.3390/jcm14155172 [doi] PST - epublish SO - J Clin Med. 2025 Jul 22;14(15):5172. doi: 10.3390/jcm14155172. PMID- 40416150 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250527 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 4 DP - 2025 Apr TI - Effect of UV-A1 Phototherapy Treatment on Scleroderma: A Systematic Review. PG - e82899 LID - 10.7759/cureus.82899 [doi] LID - e82899 AB - Scleroderma is an autoimmune disease characterized by thickened and hardened skin, Raynaud's phenomenon, calcinosis, telangiectasias, joint and muscle problems, as well as respiratory, cardiac, renal, and gastrointestinal disturbances. Scleroderma can be classified as either localized or systemic. Localized scleroderma refers to sclerosis of isolated areas of the body confined to the skin and subcutaneous layer, but it does not involve the distal extremities. However, an exception to this in some cases is morphea, which may progress to organs. In comparison, systemic scleroderma affects both cutaneous and visceral organs. Diagnosis of the condition is made by clinical presentation, medical history, and diagnostic tests. Current treatment options for scleroderma include cyclophosphamide, methotrexate, azathioprine, mycophenolate mofetil, and hematopoietic stem cell transplant. However, immunosuppressive agents used to treat scleroderma are associated with adverse effects. An unmet need exists for alternative therapies that are tied to a lower risk of adverse events. Ultraviolet (UV)-A1 phototherapy has increasingly been analyzed for use within autoimmune conditions as both a potential first-line or adjuvant treatment option. As such, we conducted a systematic literature review of a total of 293 articles using Ovid, Web of Science, and Cumulative Index to Nursing and Allied Health Literature (CINAHL). Based on our inclusion and exclusion criteria, we included 11 articles in this review, which consisted of a total of 166 patients, the majority being female at 140 (84.3%) patients and only 26 males (15.7%). Overall, patients who received UV-A1 phototherapy saw beneficial effects, including improvements in skin elasticity, mobility of extremities, reduction of skin thickness within sclerotic areas, ulcer improvement, skin softening, reduction of skin tightness, and reduction in collagen bundle size and thickness. UV-A1 phototherapy has the potential to become an integral component of scleroderma management, offering a non-invasive and effective option for patients. CI - Copyright © 2025, Nagy et al. FAU - Nagy, Stephanie AU - Nagy S AD - Rheumatology, Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Davie, USA. FAU - Tehrani, Lily AU - Tehrani L AD - Rheumatology, Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Davie, USA. FAU - Kesselman, Marc M AU - Kesselman MM AD - Rheumatology, Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Davie, USA. LA - eng PT - Journal Article PT - Review DEP - 20250424 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12103090 OTO - NOTNLM OT - limited systemic sclerosis OT - morphea OT - phototherapy OT - scleroderma OT - systemic sclerosis OT - ultraviolet-a1 OT - uv-a1 OT - uv-a1 phototherapy COIS- Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/05/26 06:26 MHDA- 2025/05/26 06:27 PMCR- 2025/04/24 CRDT- 2025/05/26 05:58 PHST- 2025/03/18 00:00 [received] PHST- 2025/04/23 00:00 [accepted] PHST- 2025/05/26 06:27 [medline] PHST- 2025/05/26 06:26 [pubmed] PHST- 2025/05/26 05:58 [entrez] PHST- 2025/04/24 00:00 [pmc-release] AID - 10.7759/cureus.82899 [doi] PST - epublish SO - Cureus. 2025 Apr 24;17(4):e82899. doi: 10.7759/cureus.82899. eCollection 2025 Apr. PMID- 36535846 OWN - NLM STAT- MEDLINE DCOM- 20230306 LR - 20230306 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 44 IP - 3 DP - 2023 Mar TI - [Profiles of autoimmune myositis with or without pulmonary involvement: A retrospective single-center study of 40 patients]. PG - 105-111 LID - S0248-8663(22)01153-5 [pii] LID - 10.1016/j.revmed.2022.11.010 [doi] AB - INTRODUCTION: Idiopathic inflammatory myopathies (IMM) are rare diseases with clinico-biological heterogeneity. Pulmonary involvement is frequent and associated with some distinctive manifestations. The aim of this study was to describe the clinico-biological profile of patients with autoimmune myositis with and without pulmonary involvement. METHODS: This retrospective descriptive study included patients with idiopathic inflammatory myopathies and a positive antibody test performed at Grenoble Alpes University Hospital between 2010 and 2020. RESULTS: Forty patients were included, the majority were women. The anti-Jo1 autoantibody was the most frequently found (37.5%). The prevalence of pulmonary involvement was 70%. Mechanics' hands and Raynaud's syndrome were the extra-respiratory signs significantly more present in the group with lung involvement (P <0.05), in contrast to creatine kinase levels which were lower in this group (P <0.05). Glucocorticoids and rituximab were significantly more often used in the group with lung involvement (P <0.05). The 5-year survival rate was 76.2% in patients with lung involvement and 100% in patients without lung involvement (P=0.50). CONCLUSION: We report a high prevalence of lung involvement probably explained by the presence of many patients with anti-synthetase syndrome. Our study highlights a lower severity of muscle involvement in myositis patients with lung disease, which deserves to be confirmed in a larger study. CI - Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Chol, O AU - Chol O AD - Service de médecine interne, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France; Faculté de médecine, université Grenoble Alpes, 38700 La Tronche, France. Electronic address: ochol@chu-grenoble.fr. FAU - Deroux, A AU - Deroux A AD - Service de médecine interne, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France. FAU - Bosseray, A AU - Bosseray A AD - Service de médecine interne, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France. FAU - Dumestre-Perard, C AU - Dumestre-Perard C AD - Laboratoire d'immunologie, institut de biologie et pathologie, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France; Institut de biologie structurale (IBS), université Grenoble Alpes, CEA, CNRS, Grenoble, France; Faculté de médecine, université Grenoble Alpes, 38700 La Tronche, France. FAU - Quetant, S AU - Quetant S AD - Service de pneumologie, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France. FAU - Bocquet, A AU - Bocquet A AD - Service de médecine interne, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France; Faculté de médecine, université Grenoble Alpes, 38700 La Tronche, France. FAU - Bouillet, L AU - Bouillet L AD - Service de médecine interne, CHU de Grenoble Alpes, CS 10271, 38043 Grenoble cedex 9, France; Faculté de médecine, université Grenoble Alpes, 38700 La Tronche, France. LA - fre PT - English Abstract PT - Journal Article TT - Profils des myosites auto-immunes avec ou sans atteinte pulmonaire : une étude monocentrique rétrospective de 40 patients. DEP - 20221217 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Autoantibodies) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Humans MH - Male MH - Female MH - Retrospective Studies MH - *Myositis/complications/diagnosis/epidemiology MH - Autoantibodies MH - *Autoimmune Diseases/complications/epidemiology MH - Rituximab OTO - NOTNLM OT - Anti-Jo1 antibody OT - Anticorps anti-Jo1 OT - Antisynthetase syndrome OT - Interstitial pneumonia OT - Myosite auto-immune OT - Myositis OT - Pneumopathie interstitielle OT - Syndrome des antisynthétases EDAT- 2022/12/20 06:00 MHDA- 2023/03/07 06:00 CRDT- 2022/12/19 21:57 PHST- 2022/03/12 00:00 [received] PHST- 2022/11/16 00:00 [revised] PHST- 2022/11/30 00:00 [accepted] PHST- 2022/12/20 06:00 [pubmed] PHST- 2023/03/07 06:00 [medline] PHST- 2022/12/19 21:57 [entrez] AID - S0248-8663(22)01153-5 [pii] AID - 10.1016/j.revmed.2022.11.010 [doi] PST - ppublish SO - Rev Med Interne. 2023 Mar;44(3):105-111. doi: 10.1016/j.revmed.2022.11.010. Epub 2022 Dec 17. PMID- 22707772 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120824 LR - 20211021 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 57 IP - 3 DP - 2012 May TI - Mucocutaneous and demographic features of systemic sclerosis: a profile of 46 patients from eastern India. PG - 201-5 LID - 10.4103/0019-5154.96193 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is a multisystem connective tissue disorder of uncertain etiology. The clinical picture is frequently dominated by prominent cutaneous manifestations that have diagnostic and prognostic significance. The objective of the present study was to find out the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. In addition, we sought to compare the frequency and pattern of the findings in the limited versus the diffuse variety of the disease. MATERIALS AND METHODS: This was a cross-sectional, clinical observational study. Consecutive patients of SSc attending the dermatology O.P.D. of a tertiary care hospital of eastern India over 3 years were enrolled to the present study. RESULTS: A total of 46 patients (41 females and 5 males; mean age 29.6±12.3 years) of SSc were evaluated. Among mucocutaneous manifestations Raynaud's phenomenon was present in 39 (84.8%) patients. Other cutaneous features included dyspigmentation (40, 86.9%), sclerodactyly (38, 82.6%), inability to open the mouth (38,82.6%), mat-like telangiectasia (11,23.1%), fingertip ulceration and scarring (29,63%), cutaneous calcinosis (1,2.2%), digital gangrene in (2,4.3%), generalized pruritus (4,8.7%), cutaneous small vessel vasculitis (2,4.3%), chronic urticaria (2,4.3%), flexion contractures of the fingers (13,28.3%), and amputation of the digits (3,6.5%). Mucosal changes were observed in 10 (21.7%) patients and nail changes were seen in 13 (28.2%) patients. Diffuse cutaneous SSc was noted in 27 (58.7%) patients and limited cutaneous SSc was seen in the remainder. Thirty-six (78.2%) patients tested positive for ANA. CONCLUSION: The present study provides a snapshot of the spectrum of the demographic and mucocutaneous manifestations of SSc in the eastern Indian population. We have not observed any statistically significant differences between dcSSc and lcSSc in terms of mucocutaneous manifestations in the studied population. FAU - Ghosh, Sudip Kumar AU - Ghosh SK AD - Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, India. FAU - Bandyopadhyay, Debabrata AU - Bandyopadhyay D FAU - Saha, Indranil AU - Saha I FAU - Barua, Jayanta Kumar AU - Barua JK LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3371524 OTO - NOTNLM OT - Demographic OT - eastern India OT - mucocutaneous OT - systemic sclerosis COIS- Conflict of Interest: Nil. EDAT- 2012/06/19 06:00 MHDA- 2012/06/19 06:01 PMCR- 2012/05/01 CRDT- 2012/06/19 06:00 PHST- 2012/06/19 06:00 [entrez] PHST- 2012/06/19 06:00 [pubmed] PHST- 2012/06/19 06:01 [medline] PHST- 2012/05/01 00:00 [pmc-release] AID - IJD-57-201 [pii] AID - 10.4103/0019-5154.96193 [doi] PST - ppublish SO - Indian J Dermatol. 2012 May;57(3):201-5. doi: 10.4103/0019-5154.96193. PMID- 20407818 OWN - NLM STAT- MEDLINE DCOM- 20110217 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 29 IP - 12 DP - 2010 Dec TI - Clinical characteristics and outcomes of patients with idiopathic inflammatory myopathies from Jordan 1996-2009. PG - 1381-5 LID - 10.1007/s10067-010-1465-8 [doi] AB - To describe demographic characteristics, clinical features and outcome of Jordanian patients with idiopathic inflammatory myopathies (IIM), a retrospective chart review of all patients diagnosed with IIM at Jordan University Hospital between 1996 and 2009 was carried out. Thirty patients with IIM were identified. Female to male ratio was 1.7:1, with mean age at diagnosis 34.3 ± 9.2 (10-72) years with bimodal presentation at 21 and 49 years and a mean follow-up of 6.5 ± 5.7 years. Eleven patients had polymyositis (PM); 19 patients had dermatomyositis (DM); 1 patient had DM with malignancy; 2 patients had juvenile DM; and 2 patients had DM/PM with other rheumatologic diseases. Raynaud's phenomenon was present in 26% of patients, dysphagia in 40%, fever in 16%, arthralgia/arthritis in 26%, and dyspnea was present in 26% patients. Positive muscle biopsy and EMG were present in 81% and 92% of patients, respectively. Elevated serum creatinine kinase (CK), AST/ALT and LDH were found in 90%, 72%, and 88% of patients at presentation, respectively. Interstitial fibrosis identified on high-resolution computed tomography (HRCT) was found in 7/14 (50%) patients. Restrictive lung disease was present in 16/21 (76%), low diffusion capacity of lung of carbon monoxide (DLCO) in 10/17 (59%) and pulmonary hypertension in only 3/19 (16%) patients tested. Arab Jordanian patients with IIM showed very low prevalence of malignancy, lower mean age than previous reports, and similar other clinical, laboratory and serologic markers, and survival rate to previous reports. Of interest, we found that extra-muscular manifestations were mainly associated with dermatomyositis. FAU - Mustafa, Khader N AU - Mustafa KN AD - Section of Rheumatology, Department of Internal Medicine, Jordan University Hospital, Queen Rania St, PO Box 13046, 11942 Amman, Jordan. kmustafa@ju.edu.jo FAU - Dahbour, Said S AU - Dahbour SS LA - eng PT - Journal Article DEP - 20100421 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Glucocorticoids) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Child MH - Female MH - Glucocorticoids/therapeutic use MH - Humans MH - Jordan/epidemiology MH - Male MH - Middle Aged MH - Myositis/diagnosis/drug therapy/*mortality MH - Prednisolone/therapeutic use MH - Retrospective Studies MH - Treatment Outcome MH - Young Adult EDAT- 2010/04/22 06:00 MHDA- 2011/02/18 06:00 CRDT- 2010/04/22 06:00 PHST- 2010/01/31 00:00 [received] PHST- 2010/04/07 00:00 [accepted] PHST- 2010/04/01 00:00 [revised] PHST- 2010/04/22 06:00 [entrez] PHST- 2010/04/22 06:00 [pubmed] PHST- 2011/02/18 06:00 [medline] AID - 10.1007/s10067-010-1465-8 [doi] PST - ppublish SO - Clin Rheumatol. 2010 Dec;29(12):1381-5. doi: 10.1007/s10067-010-1465-8. Epub 2010 Apr 21. PMID- 39176361 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240824 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 7 DP - 2024 Jul TI - A Rare Lung Malignancy in a Case of Systemic Sclerosis. PG - e65146 LID - 10.7759/cureus.65146 [doi] LID - e65146 AB - Systemic sclerosis (SSc) is one of the chronic autoimmune diseases characterized by the infiltration of excess collagen in various organs, especially the skin. It is found to be associated with a higher prevalence of internal malignancies, particularly lung carcinoma. Herein, we report a case of adenosquamous carcinoma confining within the lung in a patient who had long-standing SSc. She was a 55-year-old female patient presenting with progressive dry cough and breathlessness for six months. She had been a known case of diffuse cutaneous SSc for over a decade, based on 2013 American College of Rheumatology (ACR) criteria. The diagnosis is made based on her findings of bilateral thickening of the fingers on both hands, extending up to the metacarpophalangeal joints. Furthermore, she had telangiectasia at the upper chest wall and neck, multiple pitting scars at the toes, Raynaud's esophageal dilatation, and interstitial lung disease (ILD). She had been treated on Mycophenolate Mofetil 500 mg twice daily and low-dose prednisolone 5 mg once daily for 10 years. The patient's high-resolution computed tomography (HRCT) of the chest revealed a subpleural nodule in the posterior basal segment of the left lower lobe with areas of reticular opacities and interlobular septal thickening on bilateral lung fields six months earlier. The current computed tomography of the lung revealed a new 2.6 x 2.5 cm ill-defined lesion with irregular margins at the left lower lobe. A CT-guided biopsy was done for the lesion, which revealed adenosquamous carcinoma. Immunohistochemistry was consistent with a diagnosis of primary pulmonary adenosquamous carcinoma. The patient did not accept any further investigations and/or treatment. Herein, we present a rare lung malignancy, adenosquamous carcinoma of the lung with an underlying long-term diffuse cutaneous SSc in a nonsmoking female, which highlights the importance of lung cancer screening in individuals with SSc complicated with ILD and supports the fact that there is an increased prevalence of lung cancer among SSc-ILD patients than that of the regular population. CI - Copyright © 2024, C et al. FAU - C, Syed Akram AU - C SA AD - Department of Respiratory Medicine, SRM Medical College Hospital and Research Centre, Chennai, IND. FAU - P, Harshavardhini AU - P H AD - Department of Respiratory Medicine, SRM Medical College Hospital and Research Centre, Chennai, IND. FAU - Jayanthi, Nalini AU - Jayanthi N AD - Department of Respiratory Medicine, SRM Medical College Hospital and Research Centre, Chennai, IND. LA - eng PT - Case Reports PT - Journal Article DEP - 20240722 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11341069 OTO - NOTNLM OT - adenosquamous carcinoma OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - progressive pulmonary fibrosis OT - pulmonary hypertension OT - systemic sclerosis OT - usual interstitial pneumonia (uip) COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/08/23 06:41 MHDA- 2024/08/23 06:42 PMCR- 2024/07/22 CRDT- 2024/08/23 04:27 PHST- 2024/06/26 00:00 [received] PHST- 2024/07/22 00:00 [accepted] PHST- 2024/08/23 06:42 [medline] PHST- 2024/08/23 06:41 [pubmed] PHST- 2024/08/23 04:27 [entrez] PHST- 2024/07/22 00:00 [pmc-release] AID - 10.7759/cureus.65146 [doi] PST - epublish SO - Cureus. 2024 Jul 22;16(7):e65146. doi: 10.7759/cureus.65146. eCollection 2024 Jul. PMID- 34046688 OWN - NLM STAT- MEDLINE DCOM- 20220719 LR - 20220721 IS - 1437-160X (Electronic) IS - 0172-8172 (Print) IS - 0172-8172 (Linking) VI - 42 IP - 8 DP - 2022 Aug TI - Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study. PG - 1431-1441 LID - 10.1007/s00296-021-04883-7 [doi] AB - The current study aimed to characterize patients from a rheumatology referral center in north India, who satisfied the definition of interstitial pneumonia with autoimmune features (IPAF) as given by the American Thoracic Society and European Respiratory Society (ATS/ERS) consensus committee in 2015. Thirty-five adult patients aged 18 years and above, fulfilling the 2015 ATS/ERS criteria for IPAF were included in the study. The clinical and immunological profile, and radiologic findings on high-resolution computerized tomography thorax were noted. Antinuclear antibody (ANA) by indirect immunofluorescence at 1:320 titer and myositis-specific antibody (MSA) assays were performed. Non-parametric tests were used to compare variables between groups. The study cohort included predominantly female patients with a mean age of 50.6 ± 13 years and mean duration of disease of 38.8 ± 28.4 months. Majority of patients (49%) fulfilled the morphologic and serologic domains as per the IPAF consensus criteria and 31% patients had features in all three domains. Non-specific interstitial pneumonia was the most common pattern observed in 77% patients. Raynaud's phenomenon and inflammatory arthritis were the predominant autoimmune features. Pulmonary arterial hypertension was documented in 60% of patients on echocardiography. Positive ANA at 1:320 dilution was present in all 26 patients tested, whereas extractable nuclear antigen and MSA assays detected autoantibodies in 49% and 51% of patients respectively. IPAF predominantly affected females in the age group of 50 years and above, with varied autoimmune manifestations and autoantibody profile. CI - © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Hazarika, Kasturi AU - Hazarika K AUID- ORCID: 0000-0001-7579-136X AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. FAU - Sahoo, Rasmi Ranjan AU - Sahoo RR AUID- ORCID: 0000-0001-6616-4989 AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. FAU - Mohindra, Namita AU - Mohindra N AUID- ORCID: 0000-0003-3043-1218 AD - Department of Radiodiagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Wakhlu, Archana AU - Wakhlu A AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. FAU - Manoj, Manesh AU - Manoj M AUID- ORCID: 0000-0002-4031-136X AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. FAU - Bafna, Prashant AU - Bafna P AUID- ORCID: 0000-0001-9424-6412 AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. FAU - Garg, Rajiv AU - Garg R AUID- ORCID: 0000-0002-5564-8037 AD - Department of Respiratory Medicine, King George's Medical University, Lucknow, 226003, India. FAU - Misra, Durga Prasanna AU - Misra DP AUID- ORCID: 0000-0002-5035-7396 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Agarwal, Vikas AU - Agarwal V AUID- ORCID: 0000-0002-4508-1233 AD - Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India. FAU - Wakhlu, Anupam AU - Wakhlu A AUID- ORCID: 0000-0003-4342-9547 AD - Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, 226003, India. anupamwakhlu@gmail.com. LA - eng PT - Journal Article DEP - 20210527 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - Autoantibodies MH - *Autoimmune Diseases/diagnostic imaging MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Lung Diseases, Interstitial/diagnostic imaging MH - Male MH - Middle Aged MH - *Myositis PMC - PMC8158082 OTO - NOTNLM OT - Autoantibodies OT - Connective tissue diseases OT - Idiopathic interstitial pneumonias OT - Interstitial lung disease EDAT- 2021/05/29 06:00 MHDA- 2022/07/20 06:00 PMCR- 2021/05/27 CRDT- 2021/05/28 07:34 PHST- 2021/03/11 00:00 [received] PHST- 2021/04/30 00:00 [accepted] PHST- 2021/05/29 06:00 [pubmed] PHST- 2022/07/20 06:00 [medline] PHST- 2021/05/28 07:34 [entrez] PHST- 2021/05/27 00:00 [pmc-release] AID - 10.1007/s00296-021-04883-7 [pii] AID - 4883 [pii] AID - 10.1007/s00296-021-04883-7 [doi] PST - ppublish SO - Rheumatol Int. 2022 Aug;42(8):1431-1441. doi: 10.1007/s00296-021-04883-7. Epub 2021 May 27. PMID- 37127039 OWN - NLM STAT- MEDLINE DCOM- 20231216 LR - 20231216 IS - 2567-5761 (Electronic) IS - 0720-9355 (Linking) VI - 43 IP - 6 DP - 2023 Dec TI - Factor XIII and Endothelial Dysfunction in Patients with Systemic Sclerosis. PG - 411-417 LID - 10.1055/a-2018-7014 [doi] AB - Systemic sclerosis (SSc, scleroderma) is a severe autoimmune connective tissue disease which affects the skin and internal organs. There has been evidence that coagulation factor XIII (FXIII) has a positive impact on clinical results in patients with SSc. In a single-center cohort study, we investigated the relationship between coagulation FXIII, endothelial dysfunction, and skin infection in SSc. Fifty-six patients could be included and were divided into two groups (with and without scleroderma). Markers of inflammation, coagulation, and endothelial dysfunction like C-reactive protein, leucocytes, fibrinogen, FVIII, VWF-Ag (von Willebrand factor antigen), D-dimers, and vascular endothelial growth factor were analyzed as well as MRSS (modified Rodnan skin scores) data were evaluated. Reduced daily activities were evaluated by the Scleroderma Health Assessment Questionnaire (SHAQ). There were no significant correlations between FXIII activity, MRSS, and SHAQ score. There were correlations between FXIII activity and Raynaud's phenomenon-related symptoms and a weak but not significant positive correlation with the level of pain. A significant correlation between VWF-Ag and lung-associated complaints (n = 56; p = 0.41, p < 0.0001) was found. Moreover, the study showed a correlation between VWF-Ag and MRSS (r [N = 48] = 0.4, p = 0.01), which means that higher VWF-Ag levels come along with more severe skin involvement. A trend toward a negative correlation between FXIII activity and VWF-Ag as marker of endothelial dysfunction was found (r [N = 56] = - 0.20, p = 0.15). In our cohort, there is no FXIII deficiency in patients with SSc. FXIII might have a role in improving cutaneous manifestations indirectly by means of a moderating influence on endothelial dysfunction. Further clinical evaluation is needed. CI - Thieme. All rights reserved. FAU - Alesci, Sonja AU - Alesci S AD - Frankfurt Haemophilia Centre, Goethe University Frankfurt, Frankfurt am Main, Hessen, Germany. AD - MVZ IMD GmbH Gerinnungszentrum Hochtaunus, Bad Homburg, Hessen, Germany. FAU - Wahle, Matthias AU - Wahle M AD - Department of Rheumatology, Medical Clinic III, University Hospital Augsburg, Augsburg, Bayern, Germany. FAU - Himsel, Andrea AU - Himsel A AD - Department of Rheumatology, GPR MVZ Rüsselsheim, Medical Clinic I, Rüsselsheim, Hessen, Germany. FAU - Miesbach, Wolfgang AU - Miesbach W AD - Frankfurt Haemophilia Centre, Goethe University Frankfurt, Frankfurt am Main, Hessen, Germany. LA - eng PT - Journal Article DEP - 20230501 PL - Germany TA - Hamostaseologie JT - Hamostaseologie JID - 8204531 RN - 9013-56-3 (Factor XIII) RN - 0 (von Willebrand Factor) RN - 0 (Vascular Endothelial Growth Factor A) SB - IM MH - Humans MH - *Factor XIII MH - von Willebrand Factor/metabolism MH - Cohort Studies MH - Vascular Endothelial Growth Factor A MH - *Scleroderma, Systemic/complications/diagnosis COIS- The authors declare that they have no conflict of interest. EDAT- 2023/05/02 00:42 MHDA- 2023/12/17 09:46 CRDT- 2023/05/01 18:43 PHST- 2023/12/17 09:46 [medline] PHST- 2023/05/02 00:42 [pubmed] PHST- 2023/05/01 18:43 [entrez] AID - 10.1055/a-2018-7014 [doi] PST - ppublish SO - Hamostaseologie. 2023 Dec;43(6):411-417. doi: 10.1055/a-2018-7014. Epub 2023 May 1. PMID- 35812543 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 14 IP - 6 DP - 2022 Jun TI - Systemic Lupus Erythematosus in India: A Clinico-Serological Correlation. PG - e25763 LID - 10.7759/cureus.25763 [doi] LID - e25763 AB - Background and objectives Systemic lupus erythematosus (SLE) is a chronic multisystem disorder exhibiting a wide spectrum of clinical and immunological abnormalities. Skin is the second most affected organ; lesions may precede systemic manifestations and foretell systemic involvement. Correlation between systemic manifestations and immunological profile is known but the interplay between antibodies and cutaneous findings is an area of recent interest. The present study aims to evaluate the demographic differences, pattern and prevalence of skin lesions, and correlation between cutaneous, systemic manifestations, and serological profile in SLE. Methods A total of 40 patients diagnosed with SLE, fulfilling Systemic Lupus International Collaborating Clinics (SLICC) criteria (2012), who visited the Dermatology outpatient department between April 2019 to April 2020 were recruited. Demographic details, evaluation of cutaneous lesions as lupus erythematosus (LE) specific and LE non-specific, systemic examination, hematological tests, and serological profile findings were noted. Results The mean age of onset was 23.3 years with a female to male ratio of 19:1. Common LE-specific lesions were malar rash (77.5%), photosensitivity (70%), and generalized maculopapular rash (20%). Frequently occurring LE non-specific lesions were non-scarring alopecia (60%), oral ulcers (45%), and vasculitis (12.5%). Arthritis (77.5%) and nephritis (30%) were common systemic findings. Among 14 patients with cutaneous manifestations alone, 12 (85%) had antinuclear antibody (ANA), eight (57%) had anti-double-stranded DNA (anti-dsDNA), four (28%) had anti-Smith (anti-Sm) and anti-RO/Sjögren's syndrome antigen A (Anti-RO/SSA), three (21%) had anti-histone, and one (7%) had anti-ribonucleoprotein (anti-RNP) antibodies in serum. Conclusions Lower age at onset, high prevalence of photosensitivity, anemia, and alopecia with a low prevalence of Raynaud's phenomenon suggest environmental influence in the context of the Indian population. A positive immunological profile in patients with cutaneous involvement alone gives an opportunity to the caregiver to identify the disease process much before systemic manifestations are expressed. CI - Copyright © 2022, Mathur et al. FAU - Mathur, Rachita AU - Mathur R AD - Dermatology, Sawai Man Singh (SMS) Medical College, Jaipur, IND. FAU - Deo, Kirti AU - Deo K AD - Dermatology, Dr. D.Y. (Dnyandeo Yashwantrao) Patil Medical College, Hospital & Research Centre, Dr. D.Y. Patil Vidyapeeth, Pune, IND. FAU - Raheja, Aishwarya AU - Raheja A AD - Dermatology, Dr. D.Y. (Dnyandeo Yashwantrao) Patil Medical College, Hospital & Research Centre, Dr. D.Y. Patil Vidyapeeth, Pune, IND. LA - eng PT - Journal Article DEP - 20220608 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC9270079 OTO - NOTNLM OT - autoantibodies OT - india OT - lupus OT - malar rash OT - photosensitivity OT - serology OT - sle COIS- The authors have declared that no competing interests exist. EDAT- 2022/07/12 06:00 MHDA- 2022/07/12 06:01 PMCR- 2022/06/08 CRDT- 2022/07/11 03:48 PHST- 2022/06/07 00:00 [accepted] PHST- 2022/07/11 03:48 [entrez] PHST- 2022/07/12 06:00 [pubmed] PHST- 2022/07/12 06:01 [medline] PHST- 2022/06/08 00:00 [pmc-release] AID - 10.7759/cureus.25763 [doi] PST - epublish SO - Cureus. 2022 Jun 8;14(6):e25763. doi: 10.7759/cureus.25763. eCollection 2022 Jun. PMID- 35387213 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221003 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 6 IP - 3 DP - 2021 Oct TI - Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants. PG - 299-305 LID - 10.1177/23971983211004362 [doi] AB - INTRODUCTION: Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw. CASE DESCRIPTION: A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up. CONCLUSION: The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis. CI - © The Author(s) 2021. FAU - Garcés Villalá, Miguel Angel AU - Garcés Villalá MA AUID- ORCID: 0000-0002-6920-623X AD - Universidad Nacional de Córdoba, Córdoba, Argentina. FAU - Zorrilla Albert, Carolina AU - Zorrilla Albert C AD - Universidad Nacional de Córdoba, Córdoba, Argentina. LA - eng PT - Case Reports PT - Journal Article DEP - 20210401 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922661 OTO - NOTNLM OT - Limited cutaneous systemic sclerosis OT - dental implants OT - fixed prostheses OT - microstomy OT - total rehabilitation COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2022/04/08 06:00 MHDA- 2022/04/08 06:01 PMCR- 2022/10/01 CRDT- 2022/04/07 05:22 PHST- 2021/02/07 00:00 [received] PHST- 2021/02/16 00:00 [accepted] PHST- 2022/04/07 05:22 [entrez] PHST- 2022/04/08 06:00 [pubmed] PHST- 2022/04/08 06:01 [medline] PHST- 2022/10/01 00:00 [pmc-release] AID - 10.1177_23971983211004362 [pii] AID - 10.1177/23971983211004362 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2021 Oct;6(3):299-305. doi: 10.1177/23971983211004362. Epub 2021 Apr 1. PMID- 35296408 OWN - NLM STAT- MEDLINE DCOM- 20220517 LR - 20220530 IS - 1538-2990 (Electronic) IS - 0002-9629 (Linking) VI - 363 IP - 6 DP - 2022 Jun TI - Prevalence of peripheral eosinophilia and clinical associations in systemic sclerosis patients. PG - 519-525 LID - S0002-9629(22)00112-4 [pii] LID - 10.1016/j.amjms.2021.10.031 [doi] AB - BACKGROUND: Peripheral eosinophilia (eosinophilia) is observed among systemic sclerosis (SSc) patients. The association between eosinophilia and SSc in terms of pathogenesis remains uncertain. We aimed to determine the prevalence of the clinical, serological, and cytokine associations with eosinophilia in SSc patients. METHODS: A cross-sectional study was conducted among adult SSc patients. We excluded patients having overlap syndrome and other conditions that cause eosinophilia. Investigations into the etiology of eosinophilia were performed on the same study date, including clinical parameters, blood tests for tissue parasites, IgE, interleukin-5, and transforming growth factor-beta. Eosinophilia is defined when the total eosinophil count is > 500 cells/mm(3). RESULTS: According to the sample size calculation, 185 patients were enrolled, of whom 57 (30.8%) had eosinophilia. The causes of eosinophilia were based on laboratory indicators without clinical symptoms in 21 cases (10 had a parasitic infection, 9 adrenal insufficiency, and 2 tuberculosis). After excluding suspected causes of eosinophilia, the total prevalence of eosinophilia was 21.9% (95%CI 15.9-29.1). Most of patients (164 cases; 70.6%) had diffuse cutaneous SSc. According to the logistic regression analysis, the factors associated with eosinophilia were being male (OR 3.46), duration of disease increasing every year (OR 1.16), and Raynaud's phenomenon (OR 0.27), while SSc subset, serology (i.e., anti-topoisomerase I, anti-neutrophilic cytoplasmic antibody), inflammatory markers, and cytokine levels were not. CONCLUSIONS: Eosinophilia of unknown causes was detected in 1 in 5 SSc patients, particularly in males with no vasculopathy. Eosinophilia has a nonspecific role vis-à-vis clinical relevance in SSc. CI - Copyright © 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. Electronic address: fching@kku.ac.th. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Maleewong, Pewpan AU - Maleewong P AD - Department of Parasitology, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Maleewong, Wanchai AU - Maleewong W AD - Department of Parasitology, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Jumnainsong, Amonrat AU - Jumnainsong A AD - Faculty of Associated Medical Sciences, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Pongkulkiat, Patnarin AU - Pongkulkiat P AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Teawtrakul, Nattiya AU - Teawtrakul N AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Nanagara, Ratanavadee AU - Nanagara R AD - Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220313 PL - United States TA - Am J Med Sci JT - The American journal of the medical sciences JID - 0370506 RN - 0 (Cytokines) SB - IM MH - Adult MH - Cross-Sectional Studies MH - Cytokines MH - *Eosinophilia/epidemiology MH - Female MH - Humans MH - Male MH - Prevalence MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology OTO - NOTNLM OT - Eosinophilia OT - Fibrosis OT - Prevalence estimation OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of Competing Interest The authors declare no conflicts of interest. EDAT- 2022/03/18 06:00 MHDA- 2022/05/18 06:00 CRDT- 2022/03/17 05:30 PHST- 2021/02/18 00:00 [received] PHST- 2021/06/12 00:00 [revised] PHST- 2021/10/21 00:00 [accepted] PHST- 2022/03/18 06:00 [pubmed] PHST- 2022/05/18 06:00 [medline] PHST- 2022/03/17 05:30 [entrez] AID - S0002-9629(22)00112-4 [pii] AID - 10.1016/j.amjms.2021.10.031 [doi] PST - ppublish SO - Am J Med Sci. 2022 Jun;363(6):519-525. doi: 10.1016/j.amjms.2021.10.031. Epub 2022 Mar 13. PMID- 30032128 OWN - NLM STAT- MEDLINE DCOM- 20190909 LR - 20190909 IS - 1437-4331 (Electronic) IS - 1434-6621 (Linking) VI - 56 IP - 12 DP - 2018 Nov 27 TI - The clinical significance of borderline results of the Elia CTD Screen assay. PG - 2088-2092 LID - 10.1515/cclm-2018-0576 [doi] AB - Background Data on the clinical relevance of borderline results of solid-phase assays in the screening for antinuclear antibodies (ANA) are sparse. This study aimed to determine the clinical significance of borderline results of the Elia CTD Screen (ECS; Phadia/Thermo Fisher Scientific, Freiburg, Germany), a fluoroenzymeimmunoassay incorporating 17 recombinant human nuclear antigens. Methods We retrospectively examined the medical records of 143 subjects with borderline ECS results for ANA-associated autoimmune disorders (AASARD) and the association with the results of indirect immunofluorescence (IIF) and confirmatory assays for ANA. Results AASARD were diagnosed in 10 patients (7%) with systemic lupus erythematosus (n=5; four patients were prediagnosed and in clinical remission), polymyositis overlap syndromes (n=2), scleroderma, Raynaud's syndrome and undetermined connective tissue disease (each n=1). Most frequently, homogeneous and nucleolar IIF patterns were found. Positive ANA subsets were observed in three patients. Furthermore, four patients were diagnosed with autoimmune liver diseases and yielded positive IIF in three and positive confirmatory assays in all cases. Taken together, 129 subjects had no AASARD. Within this group, 43 patients were IIF positive and most frequently showed speckled, unspecific nucleolar and only rarely homogeneous patterns. Positive ANA subsets were found in low concentrations near to the upper reference range in 18 subjects. Conclusions AASARD were observed in 7% of the subjects with borderline ECS and showed homogeneous or nucleolar IIF patterns in the majority of these cases. Our findings suggest that borderline results of the ECS can be clinically relevant and support the concept of a parallel or sequential screening for ANA by both ECS and IIF. FAU - Robier, Christoph AU - Robier C AD - Institute of Laboratory Diagnostics, Hospital of the Brothers of St. John of God, Bergstr. 27, 8020 Graz, Austria. AD - Clinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University of Graz, Graz, Austria. FAU - Amouzadeh-Ghadikolai, Omid AU - Amouzadeh-Ghadikolai O AD - Department of Psychiatry, General Hospital Southwest, Graz, Austria. LA - eng PT - Journal Article PL - Germany TA - Clin Chem Lab Med JT - Clinical chemistry and laboratory medicine JID - 9806306 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/immunology MH - Antigens, Nuclear/*analysis/immunology MH - Autoimmune Diseases/*diagnosis/immunology MH - Connective Tissue Diseases/*diagnosis/immunology MH - Female MH - *Fluorescent Antibody Technique, Indirect MH - Humans MH - Male MH - Middle Aged MH - Retrospective Studies MH - Young Adult OTO - NOTNLM OT - ANA OT - Elia CTD Screen OT - antinuclear antibodies EDAT- 2018/07/23 06:00 MHDA- 2019/09/10 06:00 CRDT- 2018/07/23 06:00 PHST- 2018/06/04 00:00 [received] PHST- 2018/06/25 00:00 [accepted] PHST- 2018/07/23 06:00 [pubmed] PHST- 2019/09/10 06:00 [medline] PHST- 2018/07/23 06:00 [entrez] AID - /j/cclm.ahead-of-print/cclm-2018-0576/cclm-2018-0576.xml [pii] AID - 10.1515/cclm-2018-0576 [doi] PST - ppublish SO - Clin Chem Lab Med. 2018 Nov 27;56(12):2088-2092. doi: 10.1515/cclm-2018-0576. PMID- 29052562 OWN - NLM STAT- MEDLINE DCOM- 20180625 LR - 20181113 IS - 2542-5641 (Electronic) IS - 0366-6999 (Print) IS - 0366-6999 (Linking) VI - 130 IP - 20 DP - 2017 Oct 20 TI - Clinical Profile and Significance of Mucocutaneous Lesions of Primary Sjögren's Syndrome: A Large Cross-sectional Study with 874 Patients. PG - 2423-2428 LID - 10.4103/0366-6999.216403 [doi] AB - BACKGROUND: Mucocutaneous lesions are common features of primary Sjögren's syndrome (pSS), but only a few studies have focused on them. To demonstrate the profile of mucocutaneous lesions of pSS and further explore their potential clinical significance, we performed a cross-sectional study on 874 patients. METHODS: Demographic data, clinical manifestations, and laboratory results of 874 pSS patients were collected. Patients were divided into two groups according to the presence of mucocutaneous lesions. Differences in primary symptoms and systemic impairments between the two groups were analyzed. Results of laboratory tests were also compared after excluding those who had taken corticosteroid from both groups. One-year follow-up was done, and occurrences of various new complications were compared. RESULTS: Among the 874 pSS patients, 181 patients had mucocutaneous lesions, accounting for 20.7%. Multiple mucocutaneous manifestations were displayed, and the top four most common types of lesions were purpuric eruptions (39.8%), urticaria (23.8%), Raynaud's phenomenon (14.9%), and angular stomatitis (9.9%). Incidences of pulmonary interstitial fibrosis, pulmonary bullae, leukopenia, and anemia were significantly higher among patients with mucocutaneous lesions (P < 0.05). Increase in IgG and decrease in C4 among patients with mucocutaneous lesions displayed statistical significance after excluding patients from both groups who had taken corticosteroid (P < 0.05). After one-year follow-up, patients with mucocutaneous lesions presented a slightly higher incidence of new complications compared to those without. CONCLUSIONS: Mucocutaneous manifestations of pSS patients were common and diverse. Patients with mucocutaneous manifestations had more systemic damages, higher level of IgG, and lower level of serum C4, suggesting a higher activity of the primary disease. FAU - Xuan, Lei AU - Xuan L AD - Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. FAU - Zhang, Yi-Dong AU - Zhang YD AD - Department of Traditional Chinese Medicine, Peking Union Medical College, Beijing 100730, China. FAU - Li, Li AU - Li L AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. FAU - Zeng, Yue-Ping AU - Zeng YP AD - Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. FAU - Zhang, Hao-Ze AU - Zhang HZ AD - Department of Rheumatology, Peking University First Hospital, Beijing 100034, China. FAU - Wang, Jing AU - Wang J AD - Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. FAU - Dong, Zhen-Hua AU - Dong ZH AD - Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. LA - eng PT - Journal Article PL - China TA - Chin Med J (Engl) JT - Chinese medical journal JID - 7513795 SB - IM MH - Adult MH - Cross-Sectional Studies MH - Female MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Risk Factors MH - Sjogren's Syndrome/epidemiology/*pathology PMC - PMC5684622 COIS- There are no conflicts of interest. EDAT- 2017/10/21 06:00 MHDA- 2018/06/26 06:00 PMCR- 2017/10/20 CRDT- 2017/10/21 06:00 PHST- 2017/10/21 06:00 [entrez] PHST- 2017/10/21 06:00 [pubmed] PHST- 2018/06/26 06:00 [medline] PHST- 2017/10/20 00:00 [pmc-release] AID - ChinMedJ_2017_130_20_2423_216403 [pii] AID - CMJ-130-2423 [pii] AID - 10.4103/0366-6999.216403 [doi] PST - ppublish SO - Chin Med J (Engl). 2017 Oct 20;130(20):2423-2428. doi: 10.4103/0366-6999.216403. PMID- 25412573 OWN - NLM STAT- MEDLINE DCOM- 20150508 LR - 20181113 IS - 1365-2249 (Electronic) IS - 0009-9104 (Print) IS - 0009-9104 (Linking) VI - 180 IP - 1 DP - 2015 Apr TI - How should a district general hospital immunology service screen for anti-nuclear antibodies? An 'in-the-field' audit. PG - 52-7 LID - 10.1111/cei.12556 [doi] AB - Anti-nuclear antibody (ANA) testing assists in the diagnosis of several immune-mediated disorders. The gold standard method for detection of these antibodies is by indirect immunofluorescence testing on human epidermoid laryngeal carcinoma (HEp-2) cells. However, many laboratories test for these antibodies using solid-phase assays such as enzyme-linked immunosorbent assay (ELISA), which allows for higher throughput testing at reduced cost. In this study, we have audited the performance of a previously established ELISA assay to screen for ANA, making comparison with the gold standard HEp-2 immunofluorescence test. A prospective and unselected sample of 89 consecutive ANA test requests by consultant rheumatologists were evaluated in parallel over a period of 10 months using both tests. ELISA and HEp-2 screening assays yielded 40 (45%) and 72 (81%) positive test results, respectively, demonstrating lack of concordance between test methods. Using standard and clinical samples, it was demonstrated that the ELISA method did not detect several ANA with nucleolar, homogeneous and speckled immunofluorescence patterns. None of these ELISA(NEG) HEp-2(POS) ANA were reactive with a panel of six extractable nuclear antigens or with double-stranded DNA. Nonetheless, 13 of these samples (15%) originated from patients with recognized ANA-associated disease (n = 7) or Raynaud's phenomenon (n = 6). We conclude that ELISA screening may fail to detect clinically relevant ANA that lack defined specificity for antigen. CI - © 2014 British Society for Immunology. FAU - Hira-Kazal, R AU - Hira-Kazal R AD - Department of Immunology, Royal Free London NHS Foundation Trust, Barnet Hospital, Barnet, UK. FAU - Shea-Simonds, P AU - Shea-Simonds P FAU - Peacock, J L AU - Peacock JL FAU - Maher, J AU - Maher J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear/*blood/immunology MH - Autoimmune Diseases/*blood/diagnosis/immunology MH - Biological Assay/methods MH - Cell Line, Tumor MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - *Laboratories, Hospital MH - Male MH - *Medical Audit PMC - PMC4367093 OTO - NOTNLM OT - anti-nuclear antibody OT - audit OT - autoantibody OT - autoimmune testing OT - autoimmunity EDAT- 2014/11/22 06:00 MHDA- 2015/05/09 06:00 PMCR- 2016/04/01 CRDT- 2014/11/22 06:00 PHST- 2014/11/18 00:00 [accepted] PHST- 2014/11/22 06:00 [entrez] PHST- 2014/11/22 06:00 [pubmed] PHST- 2015/05/09 06:00 [medline] PHST- 2016/04/01 00:00 [pmc-release] AID - 10.1111/cei.12556 [doi] PST - ppublish SO - Clin Exp Immunol. 2015 Apr;180(1):52-7. doi: 10.1111/cei.12556. PMID- 21081568 OWN - NLM STAT- MEDLINE DCOM- 20110822 LR - 20211020 IS - 1748-880X (Electronic) IS - 0007-1285 (Print) IS - 0007-1285 (Linking) VI - 84 IP - 1003 DP - 2011 Jul TI - The use of joint-specific and whole-body MRI in osteonecrosis: a study in patients with juvenile systemic lupus erythematosus. PG - 621-8 LID - 10.1259/bjr/34972239 [doi] AB - OBJECTIVE: This study aimed to estimate the prevalence of osteonecrosis (ON) in juvenile systemic lupus erythematosus (SLE) patients using joint-specific and whole-body MRI; to explore risk factors that are associated with the development of ON; and to evaluate prospectively patients 1 year after initial imaging. METHOD: Within a 2 year period, we studied 40 juvenile SLE patients (aged 8-18 years) with a history of steroid use of more than 3 months duration. Risk factors including disease activity, corticosteroid use, vasculitis, Raynaud's phenomenon and lipid profile were evaluated. All patients underwent MRI of the hips, knees and ankles using joint-specific MRI. Whole-body STIR (short tau inversion recovery) MRI was performed in all patients with ON lesions. RESULTS: Osteonecrosis was identified in 7 patients (17.5 %) upon joint-specific MRI. Whole-body STIR MRI detected ON in 6 of these 7 patients. There was no significant difference between the ON and non-ON groups in the risk factors studied. One patient had pre-existing symptomatic ON. At 1 year follow-up, the ON lesions had resolved in one patient, remained stable in four and decreased in size in two. No asymptomatic patients with ON developed clinical manifestations. CONCLUSION: Whole-body STIR MRI may be useful in detecting ON lesions in juvenile SLE patients but larger studies are needed to define its role. FAU - Castro, T C M AU - Castro TC AD - Department of Pediatrics, Division of Allergy, Clinical Immunology and Rheumatology, Universidade Federal de São Paulo, Brazil. taniacaroline@uol.com.br FAU - Lederman, H AU - Lederman H FAU - Terreri, M T A AU - Terreri MT FAU - Caldana, W I AU - Caldana WI FAU - Kaste, S C AU - Kaste SC FAU - Hilário, M O AU - Hilário MO LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20101116 PL - England TA - Br J Radiol JT - The British journal of radiology JID - 0373125 SB - IM MH - Adolescent MH - *Ankle Joint MH - Child MH - Female MH - Humans MH - *Knee Joint MH - Lupus Erythematosus, Systemic/*complications MH - Magnetic Resonance Imaging/*methods MH - Male MH - Osteonecrosis/*diagnosis MH - Prevalence MH - Prospective Studies MH - Risk Factors MH - Whole Body Imaging/*methods PMC - PMC3473496 EDAT- 2010/11/18 06:00 MHDA- 2011/08/23 06:00 PMCR- 2012/07/01 CRDT- 2010/11/18 06:00 PHST- 2010/11/18 06:00 [entrez] PHST- 2010/11/18 06:00 [pubmed] PHST- 2011/08/23 06:00 [medline] PHST- 2012/07/01 00:00 [pmc-release] AID - 34972239 [pii] AID - D9731 [pii] AID - 10.1259/bjr/34972239 [doi] PST - ppublish SO - Br J Radiol. 2011 Jul;84(1003):621-8. doi: 10.1259/bjr/34972239. Epub 2010 Nov 16. PMID- 18302303 OWN - NLM STAT- MEDLINE DCOM- 20080828 LR - 20121115 IS - 1521-2254 (Electronic) IS - 1099-498X (Linking) VI - 10 IP - 5 DP - 2008 May TI - Plasmid growth hormone releasing hormone therapy in healthy and laminitis-afflicted horses-evaluation and pilot study. PG - 564-74 LID - 10.1002/jgm.1170 [doi] AB - BACKGROUND: In vivo electroporation dramatically improves the potency of plasmid-mediated therapies, including in large animal models. Laminitis and arthritis are common and debilitating diseases in the horse, as well as humans. METHODS: The effects of growth hormone releasing hormone (GHRH) on healthy horses and on horses with laminitis that were followed for 6 months after a single intramuscular injection and electroporation of 2.5 mg of an optimized myogenic GHRH-expressing plasmid were examined. RESULTS: In the first study on six healthy horses, we observed a significant increase in body mass by day 180 compared to baseline (P < 0.003), and an increase in erythrocyte production (hematocrit, red blood cells, hemoglobin, P = 0.03). IGF-I levels were increased by 7% by day 120 (P = 0.02). A pilot study was performed on two horses with chronic laminitis, a vascular condition often associated with arthritis, with two horses with similar clinical disease serving as non-treated controls. Treated horses experienced an increase in weight compared to control horses that received standard care (P = 0.007). By 6 months post-treatment, treated subjects were rated pasture sound. Physical and radiographic evaluation demonstrated significant improvement with reduced inflammation and decreased lameness. CONCLUSIONS: These results demonstrate that a plasmid therapy delivered by electroporation can potentially be used to treat chronic conditions in horses, and possibly other very large mammals. While further studies are needed, overall this proof-of-concept work presents encouraging data for studying gene therapeutic treatments for Raynaud's syndrome and arthritis in humans. FAU - Brown, Patricia A AU - Brown PA AD - VGX Animal Health, The Woodlands, Texas 77381, USA. FAU - Bodles-Brakhop, Angela AU - Bodles-Brakhop A FAU - Draghia-Akli, Ruxandra AU - Draghia-Akli R LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - J Gene Med JT - The journal of gene medicine JID - 9815764 RN - 9034-39-3 (Growth Hormone-Releasing Hormone) SB - IM MH - Animals MH - Arthritis/*therapy MH - Electroporation MH - Genetic Therapy/*methods MH - Growth Hormone-Releasing Hormone/*administration & dosage MH - Horse Diseases/therapy MH - Horses MH - Injections, Intramuscular MH - Models, Animal MH - Pilot Projects MH - Plasmids/administration & dosage MH - Treatment Outcome EDAT- 2008/02/28 09:00 MHDA- 2008/08/30 09:00 CRDT- 2008/02/28 09:00 PHST- 2008/02/28 09:00 [pubmed] PHST- 2008/08/30 09:00 [medline] PHST- 2008/02/28 09:00 [entrez] AID - 10.1002/jgm.1170 [doi] PST - ppublish SO - J Gene Med. 2008 May;10(5):564-74. doi: 10.1002/jgm.1170. PMID- 17937465 OWN - NLM STAT- MEDLINE DCOM- 20080201 LR - 20071106 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 34 IP - 11 DP - 2007 Nov TI - Anticentromere antibodies identify patients with Sjögren's syndrome and autoimmune overlap syndrome. PG - 2253-8 AB - OBJECTIVE: To assess the prevalence and clinical and immunological significance of anticentromere antibodies (ACA) in patients with primary Sjögren's syndrome (pSS). METHODS: We retrospectively investigated the prevalence of ACA in patients with SS. We compared ACA-positive SS patients with ACA-negative pSS patients. RESULTS: The prevalence of ACA among patients with pSS was 4.7% (10/212). Among the patients with SS and an associated autoimmune disease, 10 patients had ACA and limited cutaneous sclerosis (SSc). Clinical and immunological patterns did not differ between the 10 pSS patients with ACA alone and the 10 SS patients with ACA and SSc, except for presence of limited cutaneous SSc (lcSSc). Moreover, all ACA-positive sera recognized centromere protein-B on ELISA, regardless of the presence of SSc. The entire SS-ACA group (n = 20) showed greater frequency of Raynaud's phenomenon, objective xerophthalmia, peripheral neuropathy, and additional autoimmune disorders, especially primary biliary cirrhosis, compared to pSS patients without ACA (p = 0.005, p = 0.04, p = 0.001, p = 0.05, p < 0.0001, respectively). SS patients with ACA less frequently showed anti-SSA or anti-SSB antibodies than those without ACA (p = 0.0002, p = 0.01, respectively) but greater prevalence of autoantibodies other than anti-SSA/SSB or ACA (p = 0.001). CONCLUSION: Clinical and immunological features of SS were largely similar among SS patients with ACA with and without SSc. However, the presence of ACA among patients with SS allows identification of a subset of patients with "SS overlap syndrome," who show a wide diversity of autoimmunity, encompassing but not limited to SSc. FAU - Salliot, Carine AU - Salliot C AD - Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Service de Rhumatologie, Université Paris-Sud 11, 78 rue de Général Leclerc, Le Kremlin-Bicêtre, France. FAU - Gottenberg, Jacques-Eric AU - Gottenberg JE FAU - Bengoufa, Djaouida AU - Bengoufa D FAU - Desmoulins, Frédéric AU - Desmoulins F FAU - Miceli-Richard, Corinne AU - Miceli-Richard C FAU - Mariette, Xavier AU - Mariette X LA - eng PT - Journal Article DEP - 20071015 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Antibodies, Antinuclear) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood MH - Autoimmune Diseases/complications/diagnosis/*immunology MH - Centromere/*immunology MH - Diagnosis, Differential MH - Female MH - Humans MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Localized/complications/diagnosis/immunology MH - Sjogren's Syndrome/complications/diagnosis/*immunology MH - Syndrome EDAT- 2007/10/17 09:00 MHDA- 2008/02/02 09:00 CRDT- 2007/10/17 09:00 PHST- 2007/10/17 09:00 [pubmed] PHST- 2008/02/02 09:00 [medline] PHST- 2007/10/17 09:00 [entrez] AID - 07/13/1019 [pii] PST - ppublish SO - J Rheumatol. 2007 Nov;34(11):2253-8. Epub 2007 Oct 15. PMID- 39305614 OWN - NLM STAT- MEDLINE DCOM- 20241217 LR - 20241217 IS - 1525-3171 (Electronic) IS - 0032-5791 (Print) IS - 0032-5791 (Linking) VI - 103 IP - 12 DP - 2024 Dec TI - Research Note: Dynamics of black grouse (Tetrao tetrix) infestation with various Eimeria species. PG - 104317 LID - S0032-5791(24)00896-4 [pii] LID - 10.1016/j.psj.2024.104317 [doi] LID - 104317 AB - The black grouse (Tetrao tetrix) is an endangered species facing challenges in breeding and reintroduction programs, including parasitic infestations. This study aimed to assess natural infestations by various Eimeria species and infestation dynamics in female, male, and young black grouse kept in a stationary aviary. Faecal samples were collected from adult grouses between April and the time of chicks' hatching and rearing (September). Faecal samples from young birds were collected from the hatching for a period of 1 year. The prevalence of Eimeria spp. was determined by a qualitative method (Fulleborn's flotation) and a quantitative method (McMaster's method with Raynaud's modification). The following Eimeria species were identified: E. lyruri, E. nadsoni and E. nonbrumpti. The average percentages of Eimeria spp. in the cock were 80.52%, 9.27%, and 10.21%, respectively; in the hen, they were 86.19%, 9.28%, and 4.53%, respectively; in the young black grouses they were 84.60%, 9.34% and 6.06%, respectively. The highest E. lyruri infestation was observed in the cock in June (144227 OPG) and July (129365 OPG). In the hen, the infestation intensity increased in May (304302 OPG) and then decreased in June (39583 OPG). Furthermore, an additional increase was observed in July (216533 OPG). Two increases in infestation intensity were also observed in young birds, with peaks in January (91387 OPG) and July (126178 OPG). A positive strong correlation was identified between Eimeria spp. in the cock and the young birds. A statistically significant positive correlation was identified in the hen between E. lyruri and E. nadsoni. No correlation was demonstrated between the infestation intensity and the age of the birds or season of the year in all the grouses under study. Despite some attempts, a comprehensive approach to the issue of coccidiosis in the black grouse as a disease that may affect the success of reintroduction has yet to be established. It seems crucial to monitor the level of Eimeria spp. invasion, and the proposed faecal sampling scheme is an important tool for achieving this goal. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Sokół, Rajmund AU - Sokół R AD - Department of Parasitology and Invasive Diseases, Faculty of Veterinary Medicine, University of Warmia and Mazury in Olsztyn, M. Oczapowskiego 13, 10-719 Olsztyn, Poland. FAU - Koziatek-Sadłowska, Sylwia AU - Koziatek-Sadłowska S AD - Department of Parasitology and Invasive Diseases, Faculty of Veterinary Medicine, University of Warmia and Mazury in Olsztyn, M. Oczapowskiego 13, 10-719 Olsztyn, Poland. Electronic address: sylwia.koziatek@uwm.edu.pl. LA - eng PT - Journal Article DEP - 20240910 PL - England TA - Poult Sci JT - Poultry science JID - 0401150 SB - IM MH - Animals MH - *Eimeria/physiology MH - *Coccidiosis/veterinary/epidemiology/parasitology MH - *Galliformes MH - Male MH - Female MH - *Bird Diseases/parasitology/epidemiology MH - *Feces/parasitology MH - Prevalence PMC - PMC11458979 OTO - NOTNLM OT - Eimeria species OT - Tetrao tetrix OT - black grouse OT - invasion dynamic COIS- DISCLOSURES The authors declare that they have no known competing finanscial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/09/22 00:42 MHDA- 2024/12/17 11:49 PMCR- 2024/09/10 CRDT- 2024/09/21 18:04 PHST- 2024/06/12 00:00 [received] PHST- 2024/09/04 00:00 [revised] PHST- 2024/09/05 00:00 [accepted] PHST- 2024/12/17 11:49 [medline] PHST- 2024/09/22 00:42 [pubmed] PHST- 2024/09/21 18:04 [entrez] PHST- 2024/09/10 00:00 [pmc-release] AID - S0032-5791(24)00896-4 [pii] AID - 104317 [pii] AID - 10.1016/j.psj.2024.104317 [doi] PST - ppublish SO - Poult Sci. 2024 Dec;103(12):104317. doi: 10.1016/j.psj.2024.104317. Epub 2024 Sep 10. PMID- 38652711 OWN - NLM STAT- MEDLINE DCOM- 20240423 LR - 20240507 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 25 DP - 2024 Apr 23 TI - Eosinophilic Pleural Effusion Secondary to Trichinella spiralis Infection in a Patient with Systemic Sclerosis: A Case Report. PG - e943420 LID - 10.12659/AJCR.943420 [doi] AB - BACKGROUND Scleroderma is a chronic autoimmune disease characterized by angiopathy, autoimmunity, and fibrosis. One form of scleroderma, systemic sclerosis, is characterized by diffuse skin lesions and visceral involvement. Eosinophilic pleural effusion is a rare complication attributed to a large array of diseases. We present a case of a man with underlying systemic sclerosis who developed eosinophilic pleural effusion as a complication of associated Trichinella spiralis infection. CASE REPORT A 49-year-old man presented for bilateral inflammatory radio-ulnar-carpal joint pain, paresthesia of the hands and forearms and a 2-week history of right posterior aching thoracic pain and night sweats. The physical examination revealed sclerodermatous skin involvement of the hands, forearms, and forehead, sclerodactyly, Raynaud's phenomenon, and telangiectasias, together with muffled cardiac sounds and right basal abolishment of the vesicular breath sounds. Imagistic evaluation showed the presence of pleuro-pericardial fluid. A thoracocentesis highlighted the presence of an exudative eosinophilic pleural effusion. Laboratory findings showed leukocytosis, with elevated neutrophil and eosinophil counts. The patient was tested for a parasitic infection, but initially the results were negative. He started anti-inflammatory treatment, but no reduction of the pleural fluid was observed. Subsequent evaluation revealed specific anti-trichinella IgG antibodies. Albendazole and corticosteroid therapy were initiated, which resulted in remission of the symptoms. CONCLUSIONS This report highlights the possibility of developing rare or even not-until-now seen complications when 2 etiologically different diseases are associated. The physician should carefully assess the situation to find and resolve the underlying causes. FAU - Vulcan, Mădălina Ștefania AU - Vulcan MȘ AUID- ORCID: 0009-0007-4185-7499 AD - Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. AD - Department of Internal Medicine, Colentina Clinical Hospital, Bucharest, Romania. FAU - Dragne, Andrei-Daniel AU - Dragne AD AUID- ORCID: 0009-0000-5834-8630 AD - Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Badea, Camelia Georgeta AU - Badea CG AD - Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. AD - Department of Internal Medicine, Colentina Clinical Hospital, Bucharest, Romania. LA - eng PT - Case Reports PT - Journal Article DEP - 20240423 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 SB - IM MH - Humans MH - Male MH - Middle Aged MH - *Scleroderma, Systemic/complications/diagnosis MH - *Trichinella spiralis MH - *Trichinellosis/complications/diagnosis MH - *Pleural Effusion/etiology/parasitology MH - *Eosinophilia/parasitology/complications MH - Animals PMC - PMC11056214 COIS- Conflict of interest: None declared EDAT- 2024/04/23 18:51 MHDA- 2024/04/23 18:52 PMCR- 2024/04/23 CRDT- 2024/04/23 13:23 PHST- 2024/04/23 18:52 [medline] PHST- 2024/04/23 18:51 [pubmed] PHST- 2024/04/23 13:23 [entrez] PHST- 2024/04/23 00:00 [pmc-release] AID - 943420 [pii] AID - 10.12659/AJCR.943420 [doi] PST - epublish SO - Am J Case Rep. 2024 Apr 23;25:e943420. doi: 10.12659/AJCR.943420. PMID- 31526595 OWN - NLM STAT- MEDLINE DCOM- 20210421 LR - 20210421 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 50 IP - 2 DP - 2020 Apr TI - The preliminary validation of laser Doppler flowmetry in systemic sclerosis in accordance with the OMERACT filter: A systematic review. PG - 321-328 LID - S0049-0172(19)30201-X [pii] LID - 10.1016/j.semarthrit.2019.08.007 [doi] AB - OBJECTIVES: To investigate the validation status of laser Doppler flowmetry (LDF) in systemic sclerosis (SSc) according to the 'Outcome Measures in Rheumatologic Clinical Trials' (OMERACT) filter. METHODS: The literature was systematically reviewed to identify all reports assessing the microcirculatory flow in SSc patients. The OMERACT filter -including the domains of truth, discrimination and feasibility- was applied and a quality assessment was done by the 'Good Methods Checklist'. To ease the comparison between studies the results were grouped per dynamic test situation: basal, cold/heat challenge and occlusion. RESULTS: The literature search resulted in 4332 hits. Based on title and abstract screening 243 hits were retained and of these, 52 full texts described an assessment by LDF in SSc patients. Finally, 18 studies passed the quality assessment and form the object of this review. The review reveals that expert consensus is lacking on the face and content validity of LDF in SSc. The construct validity of LDF, on the other hand is partially validated. Conflicting results exist on the discriminant capacity of LDF in distinguishing healthy from diseased patients, primary from secondary Raynaud's phenomenon and in differentiating between disease subsets. Yet, complementing an LDF-measurement with a heat challenge, as well as the evaluation of the post-occlusive hyperaemic response, has the potential to elicit a difference between healthy and diseased patients. Lastly, data on the feasibility of LDF in SSc is lacking in the identified literature. CONCLUSION: This systematic review emphasizes the very preliminary validation status of LDF in the assessment of the microcirculatory flow in SSc. CI - Copyright © 2019 Elsevier Inc. All rights reserved. FAU - Melsens, Karin AU - Melsens K AD - Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: karin.melsens@ugent.be. FAU - Van Impe, Sarah AU - Van Impe S AD - Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: sarah.vanimpe@ugent.be. FAU - Paolino, Sabrina AU - Paolino S AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Via Balbi 5, Genoa, Italy. Electronic address: sabrina.paolino@unige.it. FAU - Vanhaecke, Amber AU - Vanhaecke A AD - Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Via Balbi 5, Genoa, Italy. Electronic address: maurizio.cutolo@unige.it. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: vanessa.smith@ugent.be. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20190906 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - Laser-Doppler Flowmetry/*methods MH - Microcirculation MH - Scleroderma, Systemic/*blood/pathology MH - Validation Studies as Topic OTO - NOTNLM OT - Laser Doppler flowmetry OT - Microcirculation OT - OMERACT OT - Outcome measure OT - Systemic scleroderma OT - Validity (epidemiology) COIS- Declaration of Competing Interest None. EDAT- 2019/09/19 06:00 MHDA- 2021/04/22 06:00 CRDT- 2019/09/19 06:00 PHST- 2019/03/21 00:00 [received] PHST- 2019/07/09 00:00 [revised] PHST- 2019/08/21 00:00 [accepted] PHST- 2019/09/19 06:00 [pubmed] PHST- 2021/04/22 06:00 [medline] PHST- 2019/09/19 06:00 [entrez] AID - S0049-0172(19)30201-X [pii] AID - 10.1016/j.semarthrit.2019.08.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2020 Apr;50(2):321-328. doi: 10.1016/j.semarthrit.2019.08.007. Epub 2019 Sep 6. PMID- 30145635 OWN - NLM STAT- MEDLINE DCOM- 20190513 LR - 20200225 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 1 DP - 2019 Jan TI - Myositis autoantibody profiles and their clinical associations in Greek patients with inflammatory myopathies. PG - 125-132 LID - 10.1007/s10067-018-4267-z [doi] AB - Myositis-specific (MSAs) or-associated autoantibodies (MAAs) have been linked to particular clinical phenotypes of idiopathic inflammatory myopathies (IIM) and appear to aid diagnosis. The objective of this study was to analyze the prevalence of MSAs and MAAs and their possible clinical associations in Greek IIM patients. This study comprised 95 IIM patients classified based on the 2017 EULAR/ACR classification criteria. All patients had MSAs and MAAs measured in their sera by line immunoblot assay. Dermatomyositis was the most prevalent IIM clinical subtype. MSAs were found in 44% of the patients, whereas MAAs in 23%. The most frequently detected MSA was anti-Jo-1 (22%), while the most frequently detected MAA was anti-Ro-52 (30%). The distributions of MSAs/MAAs did not differ between the five IIM subgroups, except for anti-Mi-2 which was only detected in dermatomyositis patients. Patients with at least one MSA and/or MAA positivity showed more frequently IIM characteristic skin rashes, while those presenting solely MAA positivity had more often puffy hands and Raynaud's phenomenon. Anti-Jo1-positive patients presented more frequently lung disease, while anti-Ro52 positivity related to mechanic's hands. Anti-Ro-52 and anti-Jo-1 strongly associated with one another. Prevalence of IIM subtypes and of MSAs/MAAs in our patients is in line with published reports in populations of similar geographic distribution. While MSA and/or MAA positivity did associate with particular clinical manifestations, it did not predict in our cohort specific IIM subgroup as defined by the latest EULAR/ACR classification criteria. Future studies are warranted to conclusively decide if these autoantibodies, measured with a standardized method, should or not be incorporated in every day clinical practice to aid IIM diagnosis. FAU - Zampeli, Evangelia AU - Zampeli E AUID- ORCID: 0000-0002-3668-4454 AD - Institute for Systemic Autoimmune and Neurological Diseases, Athens, Greece. zampelieva@gmail.com. FAU - Venetsanopoulou, Aliki AU - Venetsanopoulou A AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Argyropoulou, Ourania D AU - Argyropoulou OD AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Mavragani, Clio P AU - Mavragani CP AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. AD - Department of Physiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Tektonidou, Maria G AU - Tektonidou MG AD - First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Vlachoyiannopoulos, Panayiotis G AU - Vlachoyiannopoulos PG AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Skopouli, Fotini N AU - Skopouli FN AD - Department of Internal Medicine and Clinical Immunology, Euroclinic Hospital, Athens, Greece. FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM AD - Institute for Systemic Autoimmune and Neurological Diseases, Athens, Greece. AD - Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. AD - Academy of Athens, Athens, Greece. LA - eng PT - Journal Article DEP - 20180825 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Autoantibodies/*blood/classification MH - Autoantigens/immunology MH - Biomarkers/blood MH - Cohort Studies MH - Female MH - Greece MH - Humans MH - Male MH - Middle Aged MH - Myositis/*immunology MH - Phenotype MH - Young Adult OTO - NOTNLM OT - Dermatomyositis OT - Idiopathic inflammatory myopathies OT - Myositis-associated autoantibodies OT - Myositis-specific autoantibodies OT - Polymyositis EDAT- 2018/08/27 06:00 MHDA- 2019/05/14 06:00 CRDT- 2018/08/27 06:00 PHST- 2018/06/14 00:00 [received] PHST- 2018/08/15 00:00 [accepted] PHST- 2018/07/29 00:00 [revised] PHST- 2018/08/27 06:00 [pubmed] PHST- 2019/05/14 06:00 [medline] PHST- 2018/08/27 06:00 [entrez] AID - 10.1007/s10067-018-4267-z [pii] AID - 10.1007/s10067-018-4267-z [doi] PST - ppublish SO - Clin Rheumatol. 2019 Jan;38(1):125-132. doi: 10.1007/s10067-018-4267-z. Epub 2018 Aug 25. PMID- 27851803 OWN - NLM STAT- MEDLINE DCOM- 20170626 LR - 20221207 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 11 IP - 11 DP - 2016 TI - The 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis Could Classify Systemic Sclerosis Patients at Earlier Stage: Data from a Chinese EUSTAR Center. PG - e0166629 LID - 10.1371/journal.pone.0166629 [doi] LID - e0166629 AB - OBJECTIVES: To evaluate the performance of the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in clinical practice in a Chinese patient cohort, and to compare outcomes with the 1980 ACR criteria. METHODS: Patients clinically diagnosed with SSc between September 2013 and May 2015 were prospectively recruited from the EUSTAR database of the Peking Union Medical College Hospital. Diagnosis of SSc was based on the evaluation of three experienced rheumatologists. Patients diagnosed with other connective tissue diseases were recruited as disease controls. The 1980 ACR and 2013 ACR/EULAR criteria were applied to the cohort, and patients who fulfilled the criteria were classified as definite SSc patients. Sensitivity and specificity were analyzed for the 2013 and 1980 criteria. RESULTS: A total of 143 SSc patients and 87 patients with other connective diseases were recruited. 41 (28.7%) and 102 (71.3%) cases were diffuse cutaneous SSc and limited cutaneous SSc, respectively. Although the sensitivity of the 2013 criteria (94.4%) exceeded the 1980 criteria (72.7%) (P<0.001), the 1980 and 2013 criteria sets showed no significant difference in specificity (97.7% and 93.1%, respectively, P = 0.278). The sensitivity of the 2013 criteria was significantly higher than the 1980 criteria in some SSc subgroups (e.g., lcSSc, abnormal pattern of nailfold videocapillaroscopy [NVC] and presence of Raynaud's phenomenon [RP]) compared to others. CONCLUSIONS: Relative to the 1980 ACR criteria, in Chinese SSc patients the new 2013 ACR/EULAR criteria had similar specificity and higher sensitivity, especially for patients with mild skin thickening or prominent microvascular diseases. FAU - Xu, Dong AU - Xu D AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. FAU - Hou, Yong AU - Hou Y AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. FAU - Zheng, Yuanfang AU - Zheng Y AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. FAU - Zheng, Yue AU - Zheng Y AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. FAU - Li, Mengtao AU - Li M AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. FAU - Zeng, Xiaofeng AU - Zeng X AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China. LA - eng PT - Journal Article DEP - 20161116 PL - United States TA - PLoS One JT - PloS one JID - 101285081 SB - IM MH - Adult MH - *Asian People MH - Case-Control Studies MH - Demography MH - Europe MH - Female MH - Humans MH - Male MH - Middle Aged MH - Rheumatic Diseases/*classification MH - *Rheumatology MH - Scleroderma, Systemic/*classification MH - Sensitivity and Specificity MH - United States PMC - PMC5112860 COIS- The authors have declared that no competing interests exist. EDAT- 2016/11/17 06:00 MHDA- 2017/06/27 06:00 PMCR- 2016/11/16 CRDT- 2016/11/17 06:00 PHST- 2016/06/22 00:00 [received] PHST- 2016/11/01 00:00 [accepted] PHST- 2016/11/17 06:00 [entrez] PHST- 2016/11/17 06:00 [pubmed] PHST- 2017/06/27 06:00 [medline] PHST- 2016/11/16 00:00 [pmc-release] AID - PONE-D-16-25067 [pii] AID - 10.1371/journal.pone.0166629 [doi] PST - epublish SO - PLoS One. 2016 Nov 16;11(11):e0166629. doi: 10.1371/journal.pone.0166629. eCollection 2016. PMID- 20364700 OWN - NLM STAT- MEDLINE DCOM- 20100706 LR - 20161125 IS - 0040-3660 (Print) IS - 0040-3660 (Linking) VI - 82 IP - 1 DP - 2010 TI - [Brachial artery responsiveness in patients with systemic lupus erythematosus and scleroderma systematica]. PG - 43-6 AB - AIM: to evaluate brachial artery (BA) endothelial vasomotor function in patients with systemic lupus erythematosus (SLE) and scleroderma systematica (SDS), by using noninvasive studies. Subjects and methods. Sixty-five patients, including 25 with SLE, 20 with SDS, and 20 with atherosclerosis (AS) obliterans of lower extremity peripheral arteries, were examined. A control group consisted of 30 apparently healthy individuals matched with the study groups for gender and age. The ultrasound technique described by D. Celermajer, et al. was employed to evaluate endothelium-dependent vasodilation (EIVD). Endothelium-independent vasodilation (EIVD) was assessed by the nitroglycerin test. The coefficient of BA susceptibility (CS) to reactive hyperemia was calculated. RESULTS: In all study patient groups EDVD values were significantly lower (7.3 +/- 1.35% in SLE, 6.91 +/- 0.9% in SDS, and Z7.64 +/- 1.9% in AS; p < 0.05) than in the controls (11.23 +/- 1.1%). An adequate vascular bed response was found in 5 (20%) patients with SLE and in 2 (10%) patients with SDS. A paradoxical vasoconstrictor response to reactive hyperemia was encountered in 9 (36%) patients with SLE, 11 (55%) with SDS. In all the study groups, the patients had normal EIVD with lower CS. In SLE and SDS, CS was decreased than that in the controls. The impaired BA responsiveness in SLE and SDS significantly correlated with the duration and activity of the disease, Raynaud's syndrome, capillaritides, mean blood pressure, renal lesions, as well as with the laboratory values of the activity of inflammation and blood lipid composition. CONCLUSION: In SLE and SDS, there was a reduction in EDVD and EIVD, as well as paradoxical vasoconstriction. CS is an independent indicator of endothelial dysfunction with the normal values of EIVD. Impaired BA responsiveness was associated with the course of systemic inflammation and severe lesion of organs. FAU - Vinogradov, A A AU - Vinogradov AA FAU - Shilkina, N P AU - Shilkina NP FAU - Kostyreva, N A AU - Kostyreva NA LA - rus PT - Journal Article PL - Russia (Federation) TA - Ter Arkh JT - Terapevticheskii arkhiv JID - 2984818R RN - 0 (Vasodilator Agents) RN - G59M7S0WS3 (Nitroglycerin) SB - IM MH - Adult MH - Brachial Artery/diagnostic imaging/drug effects/*physiopathology MH - Endothelium, Vascular/physiopathology MH - Humans MH - Lupus Erythematosus, Systemic/*physiopathology MH - Middle Aged MH - Nitroglycerin MH - Scleroderma, Systemic/*physiopathology MH - Severity of Illness Index MH - Ultrasonography, Doppler MH - Vasodilation/drug effects/*physiology MH - Vasodilator Agents MH - Young Adult EDAT- 2010/04/07 06:00 MHDA- 2010/07/07 06:00 CRDT- 2010/04/07 06:00 PHST- 2010/04/07 06:00 [entrez] PHST- 2010/04/07 06:00 [pubmed] PHST- 2010/07/07 06:00 [medline] PST - ppublish SO - Ter Arkh. 2010;82(1):43-6. PMID- 18843953 OWN - NLM STAT- MEDLINE DCOM- 20090303 LR - 20140226 IS - 0578-1426 (Print) IS - 0578-1426 (Linking) VI - 47 IP - 4 DP - 2008 Apr TI - [A clinical analysis of primary Sjögren's syndrome with anticentromere antibodies]. PG - 296-9 AB - OBJECTIVE: To investigate the clinical manifestations, immunological features and prognosis of primary Sjögren's syndrome (pSS) with anticentromere antibodies (ACA). METHODS: Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. RESULTS: The mean age at the onset of pSS with ACA was higher than that of those without ACA [(48 +/- 11) yr vs (41 +/- 12) yr, P =0.000]. There was no difference in sex ratio, dry mouth, dry eyes and positive salivary gland biopsy between the two groups (P > 0.05). Compared with those without ACA, patients with ACA presented a higher prevalence of liver involvement (68.3% vs 37.0%, P = 0.000), while a lower prevalence of renal involvement (13.3% vs 30.9%, P = 0.009), neuropathy (1.7% vs 11.5%, P = 0.025) and hypergammaglobulinemia (20.8% vs 45.7%, P = 0.002). The difference was not significant between the two groups in Raynaud's phenomenon, articular involvement, myositis, hematologic involvement, lung involvement, and thyroiditis. While both groups showed the same prevalence of antinuclear antibody (ANA), the patterns of ANA-IF were different and the discrete speckled pattern was the most frequent in patients with ACA and occurred in 61.7%. Different from those without ACA, patients with ACA presented a lower prevalence of anti-SSA, anti-SSB, rheumatoid factor, and anti-U1RNP, while showed a higher prevalence of antimitochondrial antibodies (AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis, notably bleeding varices (3/5 cases). CONCLUSION: Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS. FAU - Yan, Shu-Min AU - Yan SM AD - Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Zeng, Xiao-Feng AU - Zeng XF FAU - Zhao, Yan AU - Zhao Y FAU - Dong, Yi AU - Dong Y LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Nei Ke Za Zhi JT - Zhonghua nei ke za zhi JID - 16210490R RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/*immunology MH - Centromere/*immunology MH - Female MH - Humans MH - Liver/pathology MH - Middle Aged MH - Prognosis MH - Retrospective Studies MH - Sjogren's Syndrome/*immunology/pathology EDAT- 2008/10/11 09:00 MHDA- 2009/03/04 09:00 CRDT- 2008/10/11 09:00 PHST- 2008/10/11 09:00 [pubmed] PHST- 2009/03/04 09:00 [medline] PHST- 2008/10/11 09:00 [entrez] PST - ppublish SO - Zhonghua Nei Ke Za Zhi. 2008 Apr;47(4):296-9. PMID- 29665800 OWN - NLM STAT- MEDLINE DCOM- 20190422 LR - 20190422 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 18 IP - 1 DP - 2018 Apr 17 TI - MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report. PG - 59 LID - 10.1186/s12890-018-0622-8 [doi] LID - 59 AB - BACKGROUND: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum. CASE PRESENTATION: A 49-year-old previously healthy lady, presented with a 6 week history of skin rash, photosensitivity, mouth ulcers, fatiguability, arthralgia and myalgia. She denied subjective weakness, respiratory symptoms or dysphagia. She had Raynaud's phenomenon affecting her fingers only. Initial examination showed synovitis in her hands with skin rash. Autoimmune screen was negative. She was started on hydroxychloroquine. 4 weeks later on follow-up, she developed proximal muscle pain, dysphagia, dyspnea and dry cough. Examination showed mild proximal muscle weakness and bi-basal crackles. She was admitted and extended myositis screen was sent. She had mild anemia, lymphopenia and neutropenia, normal inflammatory markers, liver and renal panels. Capillaroscopy showed pattern of systemic sclerosis. CT chest showed early ILD. Electromyography and MRI showed features of mild myositis. PFT showed muscle weakness with low DLCO. She was given intravenous steroid and Rituximab. As she continued to deteriorate, intravenous immunoglobulins and cyclophosphamide were given. There was a brief clinical response that was short-lived with increasing oxygen dependency necessitating transfer to the ICU. At this point, the extended myositis screen confirmed the presence of anti-MDA-5 antibodies. She commenced plasmapharesis and required intubation. Unfortunately, she developed multiple pneumothoraces, and was transferred urgently for ECMO. Subsequent immunosuppression included rituximab and tacrolimus. There was progression of her ILD and recurrent pneumothoraces and pneumomediastinum. Unfortunately, she passed away as a consequence of her disease. CONCLUSION: This case highlights a number of considerations in approaching patients with inflammatory myositis, particularly to pulmonary involvement. It is important to highlight the utility of extended myositis antibody testing in predicting disease phenotypes and its impact on therapeutic decisions. From a management perspective, aggressive immunosuppression should be considered with potential need of earlier utilization of ECMO. FAU - Alqatari, Safi AU - Alqatari S AUID- ORCID: 0000-0002-3994-8216 AD - Royal College of Physicians of Ireland, Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland, T12 DC4A. alqatarisafi@hotmail.com. FAU - Riddell, Peter AU - Riddell P AD - Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland. FAU - Harney, Sinead AU - Harney S AD - Internal Medicine and Rheumatology Consultant Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland. FAU - Henry, Michael AU - Henry M AD - Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland. FAU - Murphy, Grainne AU - Murphy G AD - Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland. LA - eng PT - Case Reports PT - Journal Article DEP - 20180417 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 RN - 0 (Antibodies, Anti-Idiotypic) RN - 4F4X42SYQ6 (Rituximab) RN - 8N3DW7272P (Cyclophosphamide) RN - EC 3.6.1.- (IFIH1 protein, human) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) SB - IM MH - Antibodies, Anti-Idiotypic/blood MH - Cyclophosphamide/therapeutic use MH - Dermatomyositis/complications/*diagnosis/drug therapy MH - *Disease Progression MH - Fatal Outcome MH - Female MH - Humans MH - Interferon-Induced Helicase, IFIH1/metabolism MH - Lung Diseases, Interstitial/*diagnosis/drug therapy MH - Middle Aged MH - Pneumothorax/complications MH - Rituximab/therapeutic use MH - Tomography, X-Ray Computed PMC - PMC5905122 OTO - NOTNLM OT - Interstitial lung disease OT - MDA-5 OT - Myositis COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Not applicable. CONSENT FOR PUBLICATION: Consent was obtained from the patient’s husband. He signed a consent form indicating that he is aware of this case report and the possibility of it being published (the patient unfortunately passed away). COMPETING INTERESTS: There is no financial or non-financial conflict of interest with any person or organization. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2018/04/19 06:00 MHDA- 2019/04/23 06:00 PMCR- 2018/04/17 CRDT- 2018/04/19 06:00 PHST- 2017/11/30 00:00 [received] PHST- 2018/04/09 00:00 [accepted] PHST- 2018/04/19 06:00 [entrez] PHST- 2018/04/19 06:00 [pubmed] PHST- 2019/04/23 06:00 [medline] PHST- 2018/04/17 00:00 [pmc-release] AID - 10.1186/s12890-018-0622-8 [pii] AID - 622 [pii] AID - 10.1186/s12890-018-0622-8 [doi] PST - epublish SO - BMC Pulm Med. 2018 Apr 17;18(1):59. doi: 10.1186/s12890-018-0622-8. PMID- 31215844 OWN - NLM STAT- MEDLINE DCOM- 20200107 LR - 20221207 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 28 IP - 8 DP - 2019 Jul TI - Spectrum of cutaneous lupus erythematosus in South Africans with systemic lupus erythematosus. PG - 1021-1026 LID - 10.1177/0961203319856091 [doi] AB - BACKGROUND: Cutaneous involvement is very common in systemic lupus erythematosus. We describe the prevalence and spectrum of lupus-specific (cutaneous lupus erythematosus) and non-specific cutaneous features amongst mostly black South Africans with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective record review of 298 South Africans (262 blacks and 36 non-blacks) with systemic lupus erythematosus was carried out. Cutaneous features were classified according to the Gilliam and Sontheimer classification of cutaneous lupus. RESULTS: Most (81.5%) patients were black African females. The mean (SD) age at diagnosis and follow-up duration were 35.0 (11.8) and 8.0 (5.9) years, respectively. Cutaneous lupus erythematosus was seen in 76.1% of patients, mainly chronic cutaneous lupus erythematosus with the discoid lupus erythematosus subtype seen in 52.1% of patients. Acute cutaneous lupus erythematosus was seen in 30.2% of patients and was more common in non-blacks than blacks (odds ratio = 3.8 (1.9-7.9)); localized acute cutaneous lupus erythematosus was more common than generalized acute cutaneous lupus erythematosus (odds ratio = 2.6 (1.6-4.4)). Non-specific cutaneous features occurred in 77.2%, with oral/nasal ulcers and Raynaud's phenomenon each occurring in approximately 40% of patients. Diffuse melanonychia at initial diagnosis was present in 37.4% of patients and was more common in blacks than non-blacks (odds ratio = 3.1 (1.3-7.3)). Acute cutaneous lupus erythematosus was associated with renal disease (odds ratio = 2.8 (1.6-4.7)) and chronic cutaneous lupus erythematosus with arthritis (odds ratio = 2.02 (1.24-3.29)). Diffuse melanonychia was associated with less renal disease and anti-dsDNA antibody positivity (odds ratio = 0.4 (0.3-0.7) and 0.4 (0.2-0.6), respectively) and significantly lower lupus severity index scores (mean (SD) = 5.99 (1.11) vs 6.56 (1.36) in patients with no melanonychia, p < 0.05)). CONCLUSION: In this study of South Africans with systemic lupus erythematosus, the skin was the most commonly affected organ. In general, cutaneous lupus erythematosus was associated with less severe systemic disease. Acute cutaneous lupus erythematosus was less common in blacks, whereas discoid lupus erythematosus was more common than reported in Caucasians. Diffuse melanonychia was a distinctive finding and was associated with milder systemic disease. FAU - Koch, K AU - Koch K AD - 1 Department of Dermatology, University of Witwatersrand, South Africa. FAU - Tikly, M AU - Tikly M AUID- ORCID: 0000-0001-7850-3538 AD - 2 Division of Rheumatology, University of the Witwatersrand, South Africa. LA - eng PT - Journal Article DEP - 20190619 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Black People MH - Female MH - Humans MH - Lupus Erythematosus, Cutaneous/*epidemiology/ethnology MH - Lupus Erythematosus, Discoid/*epidemiology/ethnology MH - Lupus Erythematosus, Systemic/*complications MH - Male MH - Middle Aged MH - Nail Diseases/etiology MH - Retrospective Studies MH - Severity of Illness Index MH - South Africa/epidemiology MH - Young Adult OTO - NOTNLM OT - Africa OT - Cutaneous lupus OT - blacks OT - melanonychia OT - systemic lupus erythematosus EDAT- 2019/06/20 06:00 MHDA- 2020/01/08 06:00 CRDT- 2019/06/20 06:00 PHST- 2019/06/20 06:00 [pubmed] PHST- 2020/01/08 06:00 [medline] PHST- 2019/06/20 06:00 [entrez] AID - 10.1177/0961203319856091 [doi] PST - ppublish SO - Lupus. 2019 Jul;28(8):1021-1026. doi: 10.1177/0961203319856091. Epub 2019 Jun 19. PMID- 39668722 OWN - NLM STAT- MEDLINE DCOM- 20241213 LR - 20250331 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 27 IP - 12 DP - 2024 Dec TI - Case Report: A Young Woman Diagnosed With Overlap Syndrome of Systemic Sclerosis and Ankylosing Spondylitis. PG - e15427 LID - 10.1111/1756-185X.15427 [doi] AB - This case report describes a rare occurrence of a 25-year-old female diagnosed with both systemic sclerosis (SSc) and ankylosing spondylitis (AS), two distinct autoimmune diseases. The patient presented with a combination of symptoms, including progressive skin tightening, lumbosacral pain, and Raynaud's phenomenon, which complicated the diagnosis. Despite the challenges posed by the coexistence of SSc and AS, a multidisciplinary treatment approach involving corticosteroids, immunosuppressants, and supportive therapies led to significant clinical improvement. Over the course of 3 weeks, the patient's Rodnan Skin Score improved from 18 to 11, and her pain and overall disease activity were markedly reduced. Although skin sclerosis showed substantial improvement, pulmonary involvement remains a concern that requires long-term monitoring. This case highlights the complexities in diagnosing and managing overlap syndromes, emphasizing the need for personalized treatment strategies and further investigation into the underlying genetic and immunological mechanisms. CI - © 2024 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Li, Chen AU - Li C AUID- ORCID: 0000-0002-8527-1680 AD - Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China. AD - Department of Rheumatology, Fangshan Hospital, Beijing University of Chinese Medicine, Beijing, China. FAU - Jin, Di AU - Jin D AD - Department of Rheumatology, Weifang People's Hospital, Weifang, Shandong, China. FAU - Gong, Yu-Fang AU - Gong YF AD - Department of Rheumatology, Weifang People's Hospital, Weifang, Shandong, China. FAU - Liu, Hong-Xu AU - Liu HX AD - School of Traditional Chinese Medicine, Beijing University of Chinese Medicine, Beijing, China. FAU - Guan, Yi-Wei AU - Guan YW AD - School of Chinese Materia Medica, Beijing University of Chinese Medicine, Beijing, China. FAU - Li, Sheng-Guang AU - Li SG AD - Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China. LA - eng GR - WFWSJK-2023-222/Weifang Health Commission's scientific research program/ GR - WFWSJK-2023-240/Weifang Health Commission's scientific research program/ GR - WFWSJK-2022-106/Weifang Health Commission's scientific research program/ GR - No.82074246/National Natural Science Foundation of China/ GR - 82374272/National Natural Science Foundation of China/ GR - Weifang Young Medical Talent Support Project/ PT - Case Reports PT - Letter PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Immunosuppressive Agents) RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Humans MH - Female MH - Adult MH - *Spondylitis, Ankylosing/diagnosis/complications/drug therapy MH - *Scleroderma, Systemic/diagnosis/complications/drug therapy MH - Treatment Outcome MH - *Immunosuppressive Agents/therapeutic use MH - Syndrome MH - Adrenal Cortex Hormones/therapeutic use OTO - NOTNLM OT - HLA‐B27 OT - ankylosing spondylitis (AS) OT - autoimmune overlap syndrome OT - case report OT - systemic sclerosis (SSc) EDAT- 2024/12/13 11:32 MHDA- 2024/12/13 11:33 CRDT- 2024/12/13 03:33 PHST- 2024/10/26 00:00 [revised] PHST- 2024/09/18 00:00 [received] PHST- 2024/11/12 00:00 [accepted] PHST- 2024/12/13 11:33 [medline] PHST- 2024/12/13 11:32 [pubmed] PHST- 2024/12/13 03:33 [entrez] AID - 10.1111/1756-185X.15427 [doi] PST - ppublish SO - Int J Rheum Dis. 2024 Dec;27(12):e15427. doi: 10.1111/1756-185X.15427. PMID- 36090729 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240904 IS - 1642-395X (Print) IS - 2299-0046 (Electronic) IS - 1642-395X (Linking) VI - 39 IP - 4 DP - 2022 Aug TI - Clinicopathological characteristics of cutaneous lupus erythematosus patients in Bangladesh. PG - 782-787 LID - 10.5114/ada.2021.110254 [doi] AB - INTRODUCTION: Nearly all epidemiologic studies have involved patients with systemic lupus erythematosus (SLE). Few authors have investigated the characteristics of patients with cutaneous lupus erythematosus (CLE). AIM: To describe the clinical and pathologic characteristics of a series of patients diagnosed with CLE. MATERIAL AND METHODS: This is a descriptive retrospective cross-sectional study carried out using the consecutive registered records of 218 patients attending the 'Lupus Clinic' in Chittagong Medical College Hospital during the period between 2010 and 2020. The activity and damage of CLE were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). RESULTS: There were 187 (85.8%) females and 31 (14.2%) males, with the female:male ratio being 6 : 1. The mean age was 30.0 ±11.7 years. The chronic cutaneous lupus erythematosus (CCLE) patients numbered 154 (70.6%), followed by acute cutaneous lupus erythematosus (ACLE) n = 46 (21.1%), and subacute cutaneous lupus erythematosus (SCLE) n = 18 (8.3%). In LE-specific skin lesions, the most common manifestation was photosensitivity, 198 (90.8%), followed by discoid rash, 155 (71.1%) and maculo-papular lupus rash, 55 (25.2%). Among LE-nonspecific skin lesions, the most common manifestation was non-scarring alopecia, 123 (56.4%), followed by livedo reticularis, 18 (8.3%), Raynaud's phenomenon, 17 (7.8%), vasculitis, 15 (6.9%), periungual telangiectasia, 7 (3.2%), erythema multiforme, 6 (2.7%) and leg ulcers, 5 (2.3%). Antinuclear antibodies (ANA) were the most common type of autoantibody (n = 132, 60.5%) followed by anti-ds DNA (n = 91, 41.7%) and anti-phospholipid antibodies (n = 9, 4.1%). CONCLUSIONS: CCLE was the most common subtypes of CLE. Photosensitivity was the most common clinical manifestation, whereas ANA were the most frequent autoantibodies of the LE patients of this region. Patients with different subtypes of CLE have distinct clinical and pathological characteristics. CI - Copyright: © 2022 Termedia Sp. z o. o. FAU - Mowla, Mohammad Rafiqul AU - Mowla MR AD - Department of Dermatology and Venereology, Chittagong Medical College, Chittagong, Bangladesh. FAU - Barua, Deva Pratim AU - Barua DP AD - Department of Dermatology and Venereology, Chittagong Medical College, Chittagong, Bangladesh. FAU - Zaman, Shakila AU - Zaman S AD - Department of Dermatology and Venereology, Chittagong Medical College, Chittagong, Bangladesh. FAU - Chowdhury, Mohammad Ismail Hossain AU - Chowdhury MIH AD - Department of Dermatology and Venereology, Chittagong Medical College, Chittagong, Bangladesh. FAU - Ara, Shamim AU - Ara S AD - Chittagong International Medical College, Chittagong, Bangladesh. FAU - Reich, Adam AU - Reich A AD - Department of Dermatology, University of Rzeszow, Rzeszow, Poland. LA - eng PT - Journal Article DEP - 20211022 PL - Poland TA - Postepy Dermatol Alergol JT - Postepy dermatologii i alergologii JID - 101168357 PMC - PMC9454348 OTO - NOTNLM OT - Bangladesh OT - Chittagong OT - cutaneous lupus erythematosus COIS- The authors declare no conflict interest. EDAT- 2022/09/13 06:00 MHDA- 2022/09/13 06:01 PMCR- 2022/08/01 CRDT- 2022/09/12 03:48 PHST- 2021/07/09 00:00 [received] PHST- 2021/07/21 00:00 [accepted] PHST- 2022/09/12 03:48 [entrez] PHST- 2022/09/13 06:00 [pubmed] PHST- 2022/09/13 06:01 [medline] PHST- 2022/08/01 00:00 [pmc-release] AID - 45494 [pii] AID - 10.5114/ada.2021.110254 [doi] PST - ppublish SO - Postepy Dermatol Alergol. 2022 Aug;39(4):782-787. doi: 10.5114/ada.2021.110254. Epub 2021 Oct 22. PMID- 31646426 OWN - NLM STAT- MEDLINE DCOM- 20210406 LR - 20231111 IS - 1532-2807 (Electronic) IS - 1219-4956 (Print) IS - 1219-4956 (Linking) VI - 26 IP - 3 DP - 2020 Jul TI - Retrospective Analysis of Cancer-Associated Myositis Patients over the Past 3 Decades in a Hungarian Myositis Cohort. PG - 1749-1755 LID - 10.1007/s12253-019-00756-4 [doi] AB - Association between cancer and myositis has been extensively reported and malignancy is a potentially life-threating complication in myositis. In this retrospective study authors give an overview of Hungarian cancer-associated myositis (CAM) patients treated at a single centre managing 450 myositis patients. All patients were diagnosed according to Bohan and Peter. Statistical analysis of disease onset, age, sex, muscle, skin and extramuscular symptoms, muscle enzymes, presence of antibodies, treatment and prognosis was performed. 43 patients could be considered as having CAM. 83.72% had cancer within one year of diagnosis of myositis. Most common localizations were ductal carcinoma of breast and adenocarcinoma of lung. Significant differences were observed between CAM and the non-CAM control patients: DM:PM ratio was 2.31:1 vs. 0.87:1, respectively (p = 0.029), age at diagnosis was 56.60 ± 12.79 vs. 38.88 ± 10.88 years, respectively (p < 0.001). Tumour-treatment was the following: surgical removal in 55.81%, chemotherapy in 51.1%, radiotherapy in 39.53%, hormone treatment in 18.6%, combination therapy in 51.16% of patients. Muscle enzyme levels of patients undergoing surgery were significantly reduced after intervention. 36 patients died (83.72%); 25 DM (83.33%) and 11 PM patients (84.62%); 5 years survival was 15.4% for PM and 27.5% for DM. This study demonstrates that DM, distal muscle weakness, asymmetric Raynaud's phenomenon, older age, ANA-negativity are risk factors for developing malignancy and polymyositis patients have less chance of long-lasting survival. It is very important to think about cancer and follow every single myositis patient in the clinical routine because survival rate of CAM is very poor. FAU - András, Csilla AU - András C AD - Faculty of Medicine, Department of Medicine, Division of Oncology, University of Debrecen, Debrecen, Hungary. FAU - Bodoki, Levente AU - Bodoki L AD - Faculty of Medicine, Department of Medicine, Division of Rheumatology, University of Debrecen, Debrecen, Hungary. bodoki.levente@gmail.com. FAU - Nagy-Vincze, Melinda AU - Nagy-Vincze M AD - Faculty of Medicine, Department of Medicine, Division of Clinical Immunology, University of Debrecen, Debrecen, Hungary. FAU - Griger, Zoltán AU - Griger Z AD - Faculty of Medicine, Department of Medicine, Division of Clinical Immunology, University of Debrecen, Debrecen, Hungary. FAU - Csiki, Emese AU - Csiki E AD - Faculty of Medicine, Department of Medicine, Division of Oncology, University of Debrecen, Debrecen, Hungary. FAU - Dankó, Katalin AU - Dankó K AD - Faculty of Medicine, Department of Medicine, Division of Clinical Immunology, University of Debrecen, Debrecen, Hungary. LA - eng PT - Journal Article DEP - 20191023 PL - Switzerland TA - Pathol Oncol Res JT - Pathology oncology research : POR JID - 9706087 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Hungary/epidemiology MH - Male MH - Middle Aged MH - Myositis/epidemiology/*etiology MH - Neoplasms/*complications MH - Retrospective Studies PMC - PMC7297838 OTO - NOTNLM OT - Cancer OT - Cancer-associated myositis (CAM) OT - Dermatomyositis (DM) OT - Muscle weakness OT - Polymyositis (PM) COIS- The authors declare that they have no conflict of interest. EDAT- 2019/10/28 06:00 MHDA- 2021/04/07 06:00 PMCR- 2019/10/23 CRDT- 2019/10/25 06:00 PHST- 2019/05/17 00:00 [received] PHST- 2019/10/01 00:00 [accepted] PHST- 2019/10/28 06:00 [pubmed] PHST- 2021/04/07 06:00 [medline] PHST- 2019/10/25 06:00 [entrez] PHST- 2019/10/23 00:00 [pmc-release] AID - 10.1007/s12253-019-00756-4 [pii] AID - 756 [pii] AID - 10.1007/s12253-019-00756-4 [doi] PST - ppublish SO - Pathol Oncol Res. 2020 Jul;26(3):1749-1755. doi: 10.1007/s12253-019-00756-4. Epub 2019 Oct 23. PMID- 25958415 OWN - NLM STAT- MEDLINE DCOM- 20160107 LR - 20181113 IS - 1941-5923 (Electronic) IS - 1941-5923 (Linking) VI - 16 DP - 2015 May 10 TI - A case of radiation-induced generalized morphea with prominent mucin deposition and tenderness. PG - 279-82 LID - 10.12659/AJCR.893481 [doi] AB - BACKGROUND: Radiation-induced morphea is a rare complication of radiation therapy. The affected areas are generally restricted to the radiation field or to the nearby surrounding area. CASE REPORT: A 67-year-old Japanese woman with a history of right breast cancer followed by adjuvant radiotherapy was referred our hospital because of 7-year history of symmetrical indurated erythematous plaques on her trunk. Three months after completion of irradiation, erythematous plaques developed on her right chest and gradually spread accompanied tenderness. She did not have a history of trauma to her right chest. Laboratory testing was positive for antinuclear antibody test at 1: 640 but negative for anti-SS-A/B, anti-U1-RNP, anti-DNA, anti-Sm, anticentromere, anti-topoisomerase I antibodies, and Borrelia and cytomegalovirus infection. She had no Raynaud's phenomenon, sclerodactyly, or nail-fold bleeding. She did not have interstitial lung disease or other internal organ involvement. A biopsy specimen revealed reticular dermal fibrosis with thickened collagen bundles with superficial and deep perivascular infiltration of mononuclear cells. These findings were consistent with morphea. Furthermore, mucin deposition was present in the papillary dermis upon Alcian blue staining, which has been reported to be observed in generalized morphea. Consequently, a diagnosis of generalized morphea induced by radiotherapy was made. She had been treated with oral hydroxychloroquine sulfate, resulting in the resolution of tenderness but the erythematous plaques remained. CONCLUSIONS: To the best of our knowledge, this is the first report of radiation-induced generalized morphea with prominent mucin deposition. Hydroxychloroquine sulfate may be efficacious for radiation-induced morphea-associated tenderness. FAU - Yanaba, Koichi AU - Yanaba K AD - Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan. FAU - Umezawa, Yoshinori AU - Umezawa Y AD - Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan. FAU - Nakagawa, Hidemi AU - Nakagawa H AD - Department of Dermatology, The Jikei University School of Medicine, Tokyo, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20150510 PL - United States TA - Am J Case Rep JT - The American journal of case reports JID - 101489566 RN - 0 (Mucins) SB - IM MH - Aged MH - Breast Neoplasms/radiotherapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Mucins/*metabolism MH - Radiation Injuries/*complications/diagnosis MH - Radiotherapy, Adjuvant/adverse effects MH - Scleroderma, Localized/diagnosis/*etiology/metabolism MH - Skin/metabolism/pathology/*radiation effects PMC - PMC4432618 EDAT- 2015/05/11 06:00 MHDA- 2016/01/08 06:00 PMCR- 2015/05/10 CRDT- 2015/05/11 06:00 PHST- 2015/05/11 06:00 [entrez] PHST- 2015/05/11 06:00 [pubmed] PHST- 2016/01/08 06:00 [medline] PHST- 2015/05/10 00:00 [pmc-release] AID - 893481 [pii] AID - 10.12659/AJCR.893481 [doi] PST - epublish SO - Am J Case Rep. 2015 May 10;16:279-82. doi: 10.12659/AJCR.893481. PMID- 25830071 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150401 LR - 20220316 IS - 2093-7911 (Print) IS - 2093-7997 (Electronic) IS - 2093-7911 (Linking) VI - 6 IP - 1 DP - 2015 Mar TI - Cold exposure and health effects among frozen food processing workers in eastern Thailand. PG - 56-61 LID - 10.1016/j.shaw.2014.10.004 [doi] AB - Frozen food processing workers work under a cold environment which can cause several adverse health effects.This study explored factors affecting workers' health in the frozen food industry in Thailand. Participants comprised 497 workers exposed to a cold working environment and 255 office workers who served as the controls. Data were collected by a survey on the work environment, and the interview of workers for abnormal symptoms. The exposed group had the following characteristics: 52.7% male, overall average age of 27 (SD 6.6) years old, attained elementary education (Grade 4 and Grade 6) (54.1%), married (48.9%), smokers (21.3%), alcohol consumption (31.0%), duration of work was between 1 and 5 years (65.2%), working 6 days a week (82.7%), 1-5 hours of overtime per week (33.8%), office workers (33.9%); work category: sizing (6.9%), peeling (28.3%) dissecting (22.2%), and in the warehouse (8.6%). The temperature in the work environment ranged from 17.2°C to 19.2°C in most sections, -18.0°C in the warehouse, and 25°C in the office areas. Warehouse workers had more abnormal symptoms than controls including repeated pain in the musculoskeletal system (OR 11.9; 95% CI 6.12-23.45), disturbance throughout the body (OR 4.60; 95% CI 2.00-10.56), respiratory symptoms (OR 9.73; 95% CI 3.53-26.80), episodic finger symptoms (OR 13.51; 95% CI 5.17-35.33). The study results suggest that workers' health should be monitored especially with regard to back and muscle pain, respiratory symptoms, episodic finger symptoms, and cardiovascular symptoms. Health promotion campaigns such as antismoking and reduction of alcohol consumption should be established because smoking and alcohol consumption are contributing factors to the pathogenesis of Raynaud's phenomenon and peripheral vascular disorders such as hypertension and heart disease. FAU - Thetkathuek, Anamai AU - Thetkathuek A AD - Department of Industrial Hygiene and Safety, Faculty of Public Health, Burapha University, Chonburi, Thailand. FAU - Yingratanasuk, Tanongsak AU - Yingratanasuk T AD - Department of Industrial Hygiene and Safety, Faculty of Public Health, Burapha University, Chonburi, Thailand. FAU - Jaidee, Wanlop AU - Jaidee W AD - Department of Public Health Foundations, Faculty of Public Health, Burapha University, Chonburi, Thailand. FAU - Ekburanawat, Wiwat AU - Ekburanawat W AD - Occupational Medicine Center, Samitivej Sriracha Hospital, Chonburi, Thailand. LA - eng PT - Journal Article DEP - 20141018 PL - Korea (South) TA - Saf Health Work JT - Safety and health at work JID - 101542940 PMC - PMC4371894 OTO - NOTNLM OT - cold OT - health surveillance OT - illness EDAT- 2015/04/02 06:00 MHDA- 2015/04/02 06:01 PMCR- 2014/10/18 CRDT- 2015/04/02 06:00 PHST- 2014/08/01 00:00 [received] PHST- 2014/09/08 00:00 [revised] PHST- 2014/10/03 00:00 [accepted] PHST- 2015/04/02 06:00 [entrez] PHST- 2015/04/02 06:00 [pubmed] PHST- 2015/04/02 06:01 [medline] PHST- 2014/10/18 00:00 [pmc-release] AID - S2093-7911(14)00079-1 [pii] AID - 10.1016/j.shaw.2014.10.004 [doi] PST - ppublish SO - Saf Health Work. 2015 Mar;6(1):56-61. doi: 10.1016/j.shaw.2014.10.004. Epub 2014 Oct 18. PMID- 24569396 OWN - NLM STAT- MEDLINE DCOM- 20160324 LR - 20220408 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 23 IP - 7 DP - 2014 Jun TI - Postural tachycardia syndrome (POTS) and other autonomic disorders in antiphospholipid (Hughes) syndrome (APS). PG - 697-702 LID - 10.1177/0961203314524468 [doi] AB - BACKGROUND: Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder that has been shown to cause a large number of cardiac and neurological manifestations. Two recent studies have demonstrated abnormalities in cardiovascular autonomic function testing in APS patients without other cardiovascular or autoimmune disease. However, an association between autonomic disorders such as postural tachycardia syndrome and APS has not previously been described. METHODS AND RESULTS: Data were obtained by retrospective chart review. We identified 15 patients who have been diagnosed with APS and an autonomic disorder. The median age of the patients at the time of data analysis was 39 years. The autonomic disorders seen in these patients included postural tachycardia syndrome, neurocardiogenic syncope and orthostatic hypotension. The majority of patients (14/15) were female and the majority (14/15) had non-thrombotic neurological manifestations of APS, most commonly migraine, memory loss and balance disorder. Many also had livedo reticularis (11/15) and Raynaud's phenomenon (nine of 15). In some patients, the autonomic manifestations improved with anticoagulation and/or anti-platelet therapy; in others they did not. Two patients with postural tachycardia syndrome who failed to improve with the usual treatment of APS have been treated with intravenous immunoglobulin with significant improvement in their autonomic symptoms. CONCLUSION: We believe that autonomic disorders in APS may represent an important clinical association with significant implications for treatment. CI - © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav. FAU - Schofield, J R AU - Schofield JR AD - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. FAU - Blitshteyn, S AU - Blitshteyn S AD - Department of Neurology, State University at Buffalo School of Medicine and Biomedical Sciences, Buffalo, NY, USA. FAU - Shoenfeld, Y AU - Shoenfeld Y AD - Sackler Faculty in Medicine, Laura Schwarz-Kipp Chair for Research of Autoimmune Diseases, Tel-Aviv University, Israel Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel. FAU - Hughes, G R V AU - Hughes GR AD - The London Lupus Centre, London Bridge Hospital, London, UK Graham.Hughes@HCAConsultant.co.uk. LA - eng PT - Journal Article DEP - 20140225 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antiphospholipid Syndrome/*complications MH - Autonomic Nervous System Diseases/etiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Postural Orthostatic Tachycardia Syndrome/*etiology MH - Retrospective Studies MH - Young Adult OTO - NOTNLM OT - Hughes syndrome OT - antiphospholipid syndrome (APS) OT - autonomic disorders OT - intravenous immunoglobulin (IVIG) OT - neurocardiogenic syncope (NCS) OT - postural tachycardia syndrome (POTS) EDAT- 2014/02/27 06:00 MHDA- 2016/03/25 06:00 CRDT- 2014/02/27 06:00 PHST- 2013/11/12 00:00 [received] PHST- 2014/01/28 00:00 [accepted] PHST- 2014/02/27 06:00 [entrez] PHST- 2014/02/27 06:00 [pubmed] PHST- 2016/03/25 06:00 [medline] AID - 0961203314524468 [pii] AID - 10.1177/0961203314524468 [doi] PST - ppublish SO - Lupus. 2014 Jun;23(7):697-702. doi: 10.1177/0961203314524468. Epub 2014 Feb 25. PMID- 21700658 OWN - NLM STAT- MEDLINE DCOM- 20120116 LR - 20110915 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 20 IP - 10 DP - 2011 Oct TI - Clinical expression and morbidity of systemic lupus erythematosus during a post-diagnostic 5-year follow-up: a male:female comparison. PG - 1090-4 LID - 10.1177/0961203311403640 [doi] AB - The aim of this study was to analyse the prevalence of the most relevant clinical features of the diagnosis of systemic lupus erythematosus (SLE) in a sample of male patients with lupus as well as the incidence of the main causes of morbidity in a 5-year period after the diagnosis. A further aim of this study was to investigate the impact of gender on expression and morbidity of SLE. Data were collected from the medical records of 59 male and 535 female patients with SLE who were diagnosed at the hospitals in the region of Thessaloniki. Several differences in the expression and morbidity of the disease were found in relation to the gender of the patient. Male patients had a higher prevalence of thromboses, nephropathy, strokes, gastrointestinal tract symptoms and antiphospholipid syndrome when compared with female patients, but tended to present less often with arthralgia, hair loss, Raynaud's phenomenon and photosensitivity as the initial clinical manifestations. During the 5-year follow-up, positive associations have been found between male gender and the incidence of tendonitis, myositis, nephropathy and infections, particularly of the respiratory tract. In conclusion, this study has provided information regarding the features of clinical expression and morbidity in male patients, and has shown that gender is a possible factor that can influence the clinical expression of SLE. FAU - Stefanidou, S AU - Stefanidou S AD - 4th Department of Internal Medicine, Aristotle University of Thessaloniki, Hippocratio Hospital, Thessaloniki, Greece. FAU - Benos, A AU - Benos A FAU - Galanopoulou, V AU - Galanopoulou V FAU - Chatziyannis, I AU - Chatziyannis I FAU - Kanakoudi, F AU - Kanakoudi F FAU - Aslanidis, S AU - Aslanidis S FAU - Boura, P AU - Boura P FAU - Sfetsios, T AU - Sfetsios T FAU - Settas, L AU - Settas L FAU - Katsounaros, M AU - Katsounaros M FAU - Papadopoulou, D AU - Papadopoulou D FAU - Giamalis, P AU - Giamalis P FAU - Dombros, N AU - Dombros N FAU - Chatzistilianou, M AU - Chatzistilianou M FAU - Garyfallos, A AU - Garyfallos A LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20110623 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Female MH - Follow-Up Studies MH - Gastrointestinal Diseases/etiology MH - Greece/epidemiology MH - Humans MH - Lung Diseases/etiology MH - Lupus Erythematosus, Systemic/complications/*diagnosis/*epidemiology MH - Lupus Vasculitis, Central Nervous System/etiology MH - Lymphatic Diseases/etiology MH - Male MH - Middle Aged MH - Morbidity MH - Prospective Studies MH - Retrospective Studies MH - Sex Factors MH - Thrombosis/etiology MH - Young Adult EDAT- 2011/06/28 06:00 MHDA- 2012/01/17 06:00 CRDT- 2011/06/25 06:00 PHST- 2011/06/25 06:00 [entrez] PHST- 2011/06/28 06:00 [pubmed] PHST- 2012/01/17 06:00 [medline] AID - 0961203311403640 [pii] AID - 10.1177/0961203311403640 [doi] PST - ppublish SO - Lupus. 2011 Oct;20(10):1090-4. doi: 10.1177/0961203311403640. Epub 2011 Jun 23. PMID- 18612928 OWN - NLM STAT- MEDLINE DCOM- 20081016 LR - 20080709 IS - 0300-9742 (Print) IS - 0300-9742 (Linking) VI - 37 IP - 4 DP - 2008 Jul-Aug TI - The presence of anti-centromere antibodies may predict progression of estimated pulmonary arterial systolic pressure in systemic sclerosis. PG - 278-83 LID - 10.1080/03009740801978871 [doi] AB - OBJECTIVE: To define the risk factors associated with a relatively rapid increase in estimated pulmonary arterial systolic pressure (PASP) in patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for pulmonary arterial hypertension (PAH) by echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded PASP, pulmonary function, and clinical and laboratory data. PAH was defined as an estimated PASP> or =40 mmHg. In each patient, the PASP values with their corresponding time intervals were fitted to a linear function and the slope of the line was calculated. RESULTS: Seventy-one patients with at least two echocardiographic studies each were analysed. In 16 (23%) patients, the rate of PASP progression was > or =2.5 mmHg/year whereas in the remaining 55 (77%) patients the rate of progression was <2.5 mmHg/year. In multiple logistic regression analysis, anti-centromere antibodies (ACA) (OR 8.75, CI 1.12-68.38, p = 0.039) and age > or =50 years at diagnosis (OR 8.76, CI 1.28-60.14, p = 0.027) were independently associated with a rise of PASP by > or =2.5 mmHg/year. Baseline forced vital capacity (FVC) <70% (predicted), Raynaud's duration preceding skin manifestations by > or =5 years, and fibrosis on lung computed tomography (CT) were not associated with a rapid rise of PASP (p>0.05). CONCLUSIONS: Old age at diagnosis and ACA are associated with a relatively rapid rise of PASP estimated by echocardiography in SSc. Screening for PAH in these patients may, if followed by right heart catheterization, detect PAH at an earlier stage and guide therapeutic decisions. FAU - Kampolis, C AU - Kampolis C AD - Department of Pathophysiology, University of Athens School of Medicine, Athens, Greece. FAU - Plastiras, Sc AU - Plastiras S FAU - Vlachoyiannopoulos, Pg AU - Vlachoyiannopoulos P FAU - Moyssakis, I AU - Moyssakis I FAU - Tzelepis, Ge AU - Tzelepis G LA - eng PT - Journal Article PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear/*blood MH - Centromere/*immunology MH - Disease Progression MH - Echocardiography MH - Female MH - Humans MH - Hypertension, Pulmonary/*etiology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications/*immunology EDAT- 2008/07/10 09:00 MHDA- 2008/10/17 09:00 CRDT- 2008/07/10 09:00 PHST- 2008/07/10 09:00 [pubmed] PHST- 2008/10/17 09:00 [medline] PHST- 2008/07/10 09:00 [entrez] AID - 794851745 [pii] AID - 10.1080/03009740801978871 [doi] PST - ppublish SO - Scand J Rheumatol. 2008 Jul-Aug;37(4):278-83. doi: 10.1080/03009740801978871. PMID- 18553114 OWN - NLM STAT- MEDLINE DCOM- 20090413 LR - 20211020 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 27 IP - 12 DP - 2008 Dec TI - Primary Sjögren's syndrome in men: clinical and immunological characteristic based on a large cohort of Hungarian patients. PG - 1479-83 LID - 10.1007/s10067-008-0944-7 [doi] AB - The aim of the study was to define main symptoms of clinical appearance and immunoserological profile of male patients with primary Sjögren's syndrome (pSS). Four hundred and ninety-two patients fulfilling the European-American Consensus Criteria for pSS were involved in this study. The mean age of the patients was 55.93 years (55.67 years in women and 56.18 years in men). The female-male ratio was 7:1 (432 and 60 patients, respectively). At the time of the diagnosis of pSS, glandular, extraglandular manifestations (EGMs), and immunoserological parameters were assessed. The major EGMs differ between genders. Arthritis was frequently presented as EGM in both genders, but the ratio was higher in men (68% vs. 42%). Various vasculitis symptoms and lymphadenopathy were more frequent in men than in women, in contrast to Raynaud's phenomenon or autoimmune thyroiditis. Anti-SS-A and anti-SS-B were the most frequent autoantibodies in both genders, although autoantibodies against anti-nuclear factor and extractable nuclear antigens also presented in some patients. In a few cases, there were other specific autoantibodies correlated with EGMs, such as double-stranded DNA, anti-neutrophilic-cytoplasmic antibody, cyclic-citrullinated peptide, anti-thyreoglobuline antibodies, and anti-thyreoid-peroxidase antibodies. Based upon our large cohort of patients with pSS, we conclude that, although the disease is more frequent in women usually about climax, it develops also in men with the predominant symptoms of vasculitis or arthritis besides keratoconjunctivitis sicca or xerostomy. FAU - Horvath, Ildiko Fanny AU - Horvath IF AD - Division of Clinical Immunology, 3rd Department of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary. fanny.horvath@gmail.com FAU - Szodoray, Peter AU - Szodoray P FAU - Zeher, Margit AU - Zeher M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080614 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Antibodies, Antinuclear MH - Cohort Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - Keratoconjunctivitis Sicca MH - Lymphatic Diseases/immunology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Sex Factors MH - Sjogren's Syndrome/*immunology/*physiopathology MH - Vasculitis/immunology MH - Xerostomia/immunology EDAT- 2008/06/17 09:00 MHDA- 2009/04/14 09:00 CRDT- 2008/06/17 09:00 PHST- 2008/03/03 00:00 [received] PHST- 2008/05/23 00:00 [accepted] PHST- 2008/05/07 00:00 [revised] PHST- 2008/06/17 09:00 [pubmed] PHST- 2009/04/14 09:00 [medline] PHST- 2008/06/17 09:00 [entrez] AID - 10.1007/s10067-008-0944-7 [doi] PST - ppublish SO - Clin Rheumatol. 2008 Dec;27(12):1479-83. doi: 10.1007/s10067-008-0944-7. Epub 2008 Jun 14. PMID- 40122582 OWN - NLM STAT- MEDLINE DCOM- 20250323 LR - 20250529 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 12 IP - 1 DP - 2025 Mar 23 TI - Using patient-reported measures to predict hospitalisation in a population-based lupus cohort. LID - 10.1136/lupus-2024-001406 [doi] LID - e001406 AB - OBJECTIVE: SLE is a multisystem autoimmune disease where periods of disease activity, often difficult to predict, can cause irreversible disease damage. This study aimed to develop a patient-centric predictive model using real-world data that can identify patients with SLE at a higher risk of hospitalisation compared with the general SLE population. METHODS: This observational, retrospective analysis used data from the Georgians Organized Against Lupus (GOAL) cohort from 2011 to 2013. The GOAL cohort is a population-based SLE cohort that collects yearly self-report surveys covering participants' sociodemographic characteristics, clinical characteristics and perceived SLE symptoms (using the Systemic Lupus Activity Questionnaire (SLAQ)). GOAL data were linked to the Georgia Hospital Discharge Database to collect participants' all-cause hospitalisation events in the 6 months following survey completion. A two-step approach was used to predict all-cause hospitalisations-logistic regressions selected a list of GOAL predictors that were subsequently included in the classification and regression tree (CART) models to generate patient subsets based on estimated hospitalisation rates. RESULTS: There were 846 participants who completed 1486 surveys. Participants who were hospitalised within 6 months after survey completion were more likely to be younger, living in poverty and have more reported SLE symptoms than participants without a hospitalisation. CART modelling identified participants who reported any weight loss without trying, severe fatigue and Raynaud's symptoms as most likely to have an all-cause hospitalisation: one in three (34%) patients in this subset were hospitalised in the 6 months following survey completion, 2.6-fold the hospitalisation rates of the overall GOAL cohort (13%) and 6.8-fold the rate in the subset with the lowest hospitalisation rate (5%). CONCLUSIONS: This study suggests that patient-reported SLE symptoms and disease activity, specifically certain components of the SLAQ, may be of value in SLE risk management when considering hospitalisation reduction as a treatment goal. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Lim, Sung Sam AU - Lim SS AUID- ORCID: 0000-0003-2361-0787 AD - Department of Medicine, Division of Rheumatology, Emory University School of Medicine, Atlanta, Georgia, USA sslim@emory.edu. FAU - Wu, Sandra Sze-Jung AU - Wu SS AD - Respiratory and Immunology, BioPharmaceuticals Medical, AstraZeneca, Wilmington, Delaware, USA. FAU - Ross, Ryan AU - Ross R AD - Merative LP, Ann Arbor, Michigan, USA. FAU - Bao, Gaobin AU - Bao G AD - Department of Medicine, Division of Rheumatology, Emory University School of Medicine, Atlanta, Georgia, USA. FAU - Richards, Megan AU - Richards M AUID- ORCID: 0000-0003-0737-389X AD - Merative LP, Ann Arbor, Michigan, USA. FAU - Palmer, Liisa AU - Palmer L AD - Merative LP, Ann Arbor, Michigan, USA. FAU - Bryant, Gary AU - Bryant G AD - Respiratory and Immunology, BioPharmaceuticals Medical, AstraZeneca, Wilmington, Delaware, USA. LA - eng PT - Journal Article PT - Observational Study DEP - 20250323 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/therapy/epidemiology/diagnosis/physiopathology MH - Female MH - *Hospitalization/statistics & numerical data MH - Male MH - Adult MH - Middle Aged MH - Retrospective Studies MH - *Patient Reported Outcome Measures MH - Self Report MH - Severity of Illness Index MH - Georgia/epidemiology MH - Surveys and Questionnaires MH - Young Adult PMC - PMC11931938 OTO - NOTNLM OT - Outcome Assessment, Health Care OT - Risk Factors OT - Systemic Lupus Erythematosus COIS- Competing interests: SS-jW is an employee of AstraZeneca. RR, MR and LP are employed by Merative which received funding from AstraZeneca to conduct this study. GBr was an employee of AstraZeneca at the time of study. EDAT- 2025/03/24 00:29 MHDA- 2025/03/24 00:30 PMCR- 2025/03/23 CRDT- 2025/03/23 21:02 PHST- 2024/09/30 00:00 [received] PHST- 2025/02/21 00:00 [accepted] PHST- 2025/03/24 00:30 [medline] PHST- 2025/03/24 00:29 [pubmed] PHST- 2025/03/23 21:02 [entrez] PHST- 2025/03/23 00:00 [pmc-release] AID - 12/1/e001406 [pii] AID - lupus-2024-001406 [pii] AID - 10.1136/lupus-2024-001406 [doi] PST - epublish SO - Lupus Sci Med. 2025 Mar 23;12(1):e001406. doi: 10.1136/lupus-2024-001406. PMID- 30464652 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 1179-142X (Print) IS - 1179-142X (Electronic) IS - 1179-142X (Linking) VI - 11 DP - 2018 TI - Mixed connective tissue disease complicated by heart failure in Ile-Ife, Nigeria: management challenges in a resource-limited economy. PG - 307-312 LID - 10.2147/IMCRJ.S151693 [doi] AB - BACKGROUND: Mixed connective tissue disease (MCTD; also known as Sharp's syndrome) is a rare autoimmune inflammatory disorder characterized by high titer of U1 ribonucleoprotein (U1RNP) antibody and clinical and serological overlap of systemic lupus erythematosus, systemic sclerosis, and polymyositis. The diagnosis is based on clinical and serological factors in criteria such as Alarcon-Segovia, Khan, Kusakawa, and Sharps. Cardiac disease can be a complication of connective tissue disease (CTD). There are few reports in Africa. AIMS: To present MCTD as underlying cause of heart failure with reduced ejection fraction and highlight challenges of investigations and treatment. OBJECTIVES: To highlight the first case in our center and discuss the cardiac, respiratory, and rheumatologic management. PATIENT AND METHODS: We present a 52-year-old woman with 3 weeks history of productive cough with whitish sputum, severe dyspnea, orthopnea, paroxysmal nocturnal dyspnea, right sided abdominal pain, leg swellings, a one year history of recurrent fever, Raynaud's phenomenon, small joint swellings and deformities with pain in both hands. RESULTS: On examination there was microstomia, tethered forehead and lower eyelid skin, tender swelling of the interphalangeal joints and arthritis mutilans. Laboratory findings showed estimated glomerular filtration rate <60 mL/kg/min/1.73 m(2), U1RNP antibody levels were eight times upper limit of normal, elevated rheumatoid factor, speckled antinuclear antibody pattern, negative anticentromere antibody, anti Scl-70 and anticyclic citrullinated peptide. Chest X-ray/CT revealed pulmonary fibrosis. Echocardiography findings showed reduced ejection fraction of 40%, elevated pulmonary arterial pressure at rest of 60.16 mmHg. The patient showed improvement on antifailure drugs, but prednisolone was stopped for sudden reversal of previously controlled stage 2 hypertension (HTN), and the patient was discharged in a stable condition. Difficulties ensued in obtaining prompt definite results due to the unavailability of serologic tests in the hospital, and the tests were done outside the state and country. CONCLUSION: Identifying MCTD is critical, especially in patients requiring steroids that may worsen systemic HTN and heart failure. There is a need to have definitive investigative facilities for such patients in hospitals. FAU - Adewuya, Oladapo A AU - Adewuya OA AD - Department of Cardiology, dapo3000@gmail.com. FAU - Adebayo, Rasaaq A AU - Adebayo RA AD - Department of Cardiology, dapo3000@gmail.com. FAU - Ajibade, Adeola I AU - Ajibade AI AD - Department of Rheumatology. FAU - Odunlami, Gbenga J AU - Odunlami GJ AD - Department of Rheumatology. FAU - Akintomide, Anthony O AU - Akintomide AO AD - Department of Cardiology, dapo3000@gmail.com. FAU - Ogunyemi, Suraj A AU - Ogunyemi SA AD - Department of Cardiology, dapo3000@gmail.com. FAU - Ajayi, Olufemi E AU - Ajayi OE AD - Department of Cardiology, dapo3000@gmail.com. FAU - Adetiloye, Adebola O AU - Adetiloye AO AD - Department of Pulmonology. FAU - Omisore, Adeleye D AU - Omisore AD AD - Department of Radiology, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun, Nigeria. FAU - Olanipekun, Oladipo A AU - Olanipekun OA AD - Department of Cardiology, dapo3000@gmail.com. FAU - Owolabi, Adeyinka O AU - Owolabi AO AD - Department of Cardiology, dapo3000@gmail.com. FAU - Amjo, Ifeoluwa AU - Amjo I AD - Department of Cardiology, dapo3000@gmail.com. FAU - Akinyele, Olumide A AU - Akinyele OA AD - Department of Cardiology, dapo3000@gmail.com. FAU - Bamgboje, Abayomi O AU - Bamgboje AO AD - Department of Cardiology, dapo3000@gmail.com. FAU - Balogun, Michael O AU - Balogun MO AD - Department of Cardiology, dapo3000@gmail.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20181102 PL - New Zealand TA - Int Med Case Rep J JT - International medical case reports journal JID - 101566269 PMC - PMC6219426 OTO - NOTNLM OT - HFrEF OT - U1RNP OT - arthritis mutilans PAH OT - mixed connective tissue disorder OT - prednisolone COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2018/11/23 06:00 MHDA- 2018/11/23 06:01 PMCR- 2018/11/02 CRDT- 2018/11/23 06:00 PHST- 2018/11/23 06:00 [entrez] PHST- 2018/11/23 06:00 [pubmed] PHST- 2018/11/23 06:01 [medline] PHST- 2018/11/02 00:00 [pmc-release] AID - imcrj-11-307 [pii] AID - 10.2147/IMCRJ.S151693 [doi] PST - epublish SO - Int Med Case Rep J. 2018 Nov 2;11:307-312. doi: 10.2147/IMCRJ.S151693. eCollection 2018. PMID- 24989017 OWN - NLM STAT- MEDLINE DCOM- 20150402 LR - 20220318 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 33 IP - 8 DP - 2014 Aug TI - Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. PG - 1093-8 LID - 10.1007/s10067-014-2730-z [doi] AB - Because overlap syndromes (OSs) are rarely described, we analyzed retrospectively their frequencies and correlations in Brazilian series of 31 patients with dermatomyositis (DM)/polymyositis (PM) associated with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or rheumatoid arthritis (RA) attended at a referral single center. Myositis-specific autoantibodies (MSAs: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-SRP, anti-Mi-2) and myositis-associated autoantibodies (MAAs: anti-PM-Scl75, anti-PM-Scl100, anti-Ku) as well as specific autoantibodies related to SLE, SSc, and RA were investigated. The mean age of the OS patients (9 DM and 22 PM) was 44.6 ± 15.4 years, with a predominance of women (83.9 %) and white ethnicity (58.1 %). PM was the most frequent inflammatory myopathy, and the clinical presentation of DM/PM was significantly different among the OS groups. Overlap was found with SSc (48.4 %), SLE (29.0 %), and RA (22.6 %). The clinical manifestations of DM/PM were identified simultaneously with SSc and RA in the majority of cases, in contrast to identification in the SLE group (p < 0.05). All patients were positive for antinuclear antibodies, and the prevalence of MSA and MAA was 38.8 % in all OS groups, mutually exclusive, and more frequent in the SSc group. Comparing the clinical and laboratory features, there was a higher frequency of vascular (skin ulcers, Raynaud's phenomenon) and pulmonary (interstitial lung disease) involvement in the SSc group (p < 0.05). Moreover, there were no differences among the groups in relation to disease relapse and deaths. Concluding, this is the first study to show the different characteristics of a series of patients with connective tissue disease (CTD)-OS in the heterogeneous Brazilian population. FAU - Aguila, Lisbeth Aranbicia AU - Aguila LA AD - Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455 - 3 andar - sala 3190, São Paulo, 01246-903, Brazil. FAU - Lopes, Michelle Remião Ugolini AU - Lopes MR FAU - Pretti, Flavia Zon AU - Pretti FZ FAU - Sampaio-Barros, Percival Degrava AU - Sampaio-Barros PD FAU - Carlos de Souza, Fernando Henrique AU - Carlos de Souza FH FAU - Borba, Eduardo Ferreira AU - Borba EF FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140704 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Arthritis, Rheumatoid/*complications/immunology MH - *Autoantibodies MH - Brazil MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/immunology MH - Male MH - Middle Aged MH - Myositis/complications/*diagnosis/immunology MH - Retrospective Studies MH - Scleroderma, Systemic/*complications/immunology MH - Severity of Illness Index MH - Syndrome EDAT- 2014/07/06 06:00 MHDA- 2015/04/04 06:00 CRDT- 2014/07/04 06:00 PHST- 2013/10/18 00:00 [received] PHST- 2014/06/04 00:00 [accepted] PHST- 2014/05/29 00:00 [revised] PHST- 2014/07/04 06:00 [entrez] PHST- 2014/07/06 06:00 [pubmed] PHST- 2015/04/04 06:00 [medline] AID - 10.1007/s10067-014-2730-z [doi] PST - ppublish SO - Clin Rheumatol. 2014 Aug;33(8):1093-8. doi: 10.1007/s10067-014-2730-z. Epub 2014 Jul 4. PMID- 41056197 OWN - NLM STAT- MEDLINE DCOM- 20260306 LR - 20260306 IS - 2578-5826 (Electronic) IS - 2578-5826 (Linking) VI - 49 IP - 1 DP - 2026 Mar TI - Clinical improvement of salt-and-pepper skin changes in juvenile systemic sclerosis using mycophenolate mofetil after intravenous methylprednisolone: a 3-year follow-up. PG - 125-129 LID - 10.1080/25785826.2025.2570899 [doi] AB - In adult systemic sclerosis (SSc), salt-and-pepper skin changes can be used to diagnose diffuse cutaneous SSc during the early stages. However, reports of juvenile SSc (JSSc) with salt-and-pepper skin changes are unavailable. A 12-year-old Japanese girl presented with JSSc, showing scleroderma, Raynaud's phenomenon, digital ulcers, and telangiectasia. She developed scleroderma at 10 years of age and later experienced salt-and-pepper skin changes. Laboratory findings revealed positive antinuclear and anti-U3-RNP antibodies. After being diagnosed with JSSc based on established criteria, she received two courses of intravenous methylprednisolone (IVMP) followed by mycophenolate mofetil (MMF). Her modified Rodnan skin score improved from 25 to 0, and the salt-and-pepper changes resolved. This case represents the first report of the presence and subsequent improvement of salt-and-pepper skin changes in JSSc treated with MMF following IVMP. Recognition of salt-and-pepper changes may serve as an early clinical clue, prompting further diagnostic evaluation for JSSc and supporting early diagnosis of adult SSc. Moreover, MMF after IVMP may exert beneficial anti-fibrotic effects, potentially improving pigment changes by controlling scleroderma. FAU - Hironaka, Daisuke AU - Hironaka D AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Wakiguchi, Hiroyuki AU - Wakiguchi H AUID- ORCID: 0000-0003-1534-2583 AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. AD - Division of General Pediatrics and Emergency Medicine, Department of Pediatrics, Oita University Faculty of Medicine, Yufu, Japan. FAU - Okazaki, Fumiko AU - Okazaki F AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Korenaga, Yuno AU - Korenaga Y AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Azuma, Yoshihiro AU - Azuma Y AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Tanaka, Akira AU - Tanaka A AD - Department of Pathology, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Hirano, Reiji AU - Hirano R AD - Division of Pediatrics, Yamaguchi-ken Saiseikai Shimonoseki General Hospital, Shimonoseki, Japan. FAU - Shimomura, Yutaka AU - Shimomura Y AD - Department of Dermatology, Yamaguchi University Graduate School of Medicine, Ube, Japan. FAU - Hasegawa, Shunji AU - Hasegawa S AD - Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20251007 PL - England TA - Immunol Med JT - Immunological medicine JID - 101736847 RN - HU9DX48N0T (Mycophenolic Acid) RN - X4W7ZR7023 (Methylprednisolone) RN - 0 (Immunosuppressive Agents) RN - Juvenile systemic scleroderma SB - IM MH - Humans MH - Female MH - *Mycophenolic Acid/therapeutic use/administration & dosage MH - *Methylprednisolone/therapeutic use/administration & dosage MH - Child MH - *Scleroderma, Systemic/drug therapy/diagnosis/pathology MH - *Skin/pathology/drug effects MH - Follow-Up Studies MH - Treatment Outcome MH - *Immunosuppressive Agents/therapeutic use MH - Administration, Intravenous OTO - NOTNLM OT - Anti-U3-RNP antibody OT - autoimmune skin disease OT - japanese OT - modified Rodnan skin score EDAT- 2025/10/07 18:29 MHDA- 2026/03/06 09:06 CRDT- 2025/10/07 13:03 PHST- 2026/03/06 09:06 [medline] PHST- 2025/10/07 18:29 [pubmed] PHST- 2025/10/07 13:03 [entrez] AID - 10.1080/25785826.2025.2570899 [doi] PST - ppublish SO - Immunol Med. 2026 Mar;49(1):125-129. doi: 10.1080/25785826.2025.2570899. Epub 2025 Oct 7. PMID- 41324656 OWN - NLM STAT- MEDLINE DCOM- 20260113 LR - 20260116 IS - 2731-7099 (Electronic) IS - 2731-7080 (Print) IS - 2731-7080 (Linking) VI - 67 IP - 1 DP - 2026 Jan TI - [Systemic sclerosis : A disease with multiple faces]. PG - 63-71 LID - 10.1007/s00108-025-02024-x [doi] AB - Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease within the spectrum of rheumatologic disorders. It is characterized by a combination of vasculopathy and inflammatory fibrosis affecting the skin and various internal organs. The disease presents with heterogeneous manifestations, which can complicate diagnosis and must be carefully considered during both monitoring and treatment. In recent decades, advances in research have expanded the range of therapeutic options available for SSc. Treatment strategies need to be tailored individually, depending on the specific clinical manifestations in each patient. The aim of this article is to provide an overview of the diverse disease manifestations and current treatment approaches in SSc. For optimal patient care, close collaboration between rheumatologists, pneumologists, gastroenterologists, cardiologists, nephrologists and general practitioners is essential. CI - © 2025. The Author(s). FAU - Evers, Caroline AU - Evers C AD - Klinik für Rheumatologie, Universitätsspital Zürich, Rämistr. 100, 8091, Zürich, Schweiz. FAU - Distler, Jörg AU - Distler J AD - Klinik für Rheumatologie und Hiller-Forschungszentrum, Universitätsklinikum Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland. FAU - Distler, Oliver AU - Distler O AD - Klinik für Rheumatologie, Universitätsspital Zürich, Rämistr. 100, 8091, Zürich, Schweiz. oliver.distler@usz.ch. LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Systemische Sklerose : Eine Erkrankung mit vielen Gesichtern. DEP - 20251201 PL - Germany TA - Inn Med (Heidelb) JT - Innere Medizin (Heidelberg, Germany) JID - 9918384885306676 SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/therapy PMC - PMC12799631 OTO - NOTNLM OT - Antifibrotic therapy OT - Connective tissue diseases OT - Immunosuppression therapy OT - Raynaud disease OT - Skin fibrosis COIS- Einhaltung ethischer Richtlinien. Interessenkonflikt: Gemäß den Richtlinien des Springer Medizin Verlags werden Autoren und Wissenschaftliche Leitung im Rahmen der Manuskripterstellung und Manuskriptfreigabe aufgefordert, eine vollständige Erklärung zu ihren finanziellen und nichtfinanziellen Interessen abzugeben. Autoren: O. Distler: A. Finanzielle Interessen: Diverse nicht kommerzielle Stiftungen: im Stiftungsrat oder chair executive committee; nationale und internationale Wissenschaftsorganisationen: diverse Funktionen, z.B. grant reviewer, abstract reviewer, Projektmitarbeiter, Mitglied wissenschaftliches Komitee; Boehringer Ingelheim, Kymera, Mitsubishi: Unterstützung für wissenschaftliche Projekte. – Referentenhonorar: Boehringer, Medscape, Novartis, Sanofi, Bayer, Janssen. – Im Bereich der Sklerodermie und assoziierter Erkrankungen: 4P-Pharma, AbbVie, Acepodia, Aera, AnaMar, Anaveon, Argenx, AstraZeneca, Boehringer Ingelheim, BMS, Calluna, Cantargia, CSL Behring, EMD Serono, Galderma, Galapagos, Gossamer, Hemetron, Innovaderm, Janssen, Kali, Lilly, MSD Merck, Nkarta, Novartis, Oorja Bio, Orion, Pliant, Prometheus, Quell, Redx Pharma, Scleroderma Research Foundation, Sumitomo, Tandem, Topadur, UCB und Umlaut.bio. – Patent issued „mir-29 for the treatment of systemic sclerosis“ (US8247389, EP2331143). – B. Nichtfinanzielle Interessen: Professor, Klinikdirektor, Klinik für Rheumatologie, Universitätsspital Zürich; Direktor Forschung, Zentrum für Experimentelle Rheumatologie, Schlieren; Fakultätsmitglied, Medizinische Fakultät, Universität Zürich | Mitgliedschaften: Schweizerische Gesellschaft für Rheumatologie, Chairman European Scleroderma Trials and Research Group (EUSTAR), Walter-Siegenthaler-Gesellschaft, Chefärztegesellschaft der Universitätskliniken des Kantons Zürich (CUK), Stiftungsrat Swiss Clinical Quality Management in Rheumatic Diseases, Zürich, Stiftungsrat Hartmann Müller-Stiftung, Schweizerische Akademie der Medizinischen Wissenschaften (SAMW), Vorsitzender beim Ausschuss der FOREUM Foundation. C. Evers: A. Finanzielle Interessen: C. Evers gibt an, dass kein finanzieller Interessenkonflikt besteht. – B. Nichtfinanzielle Interessen: Angestellte Oberärztin, Klinik für Rheumatologie, Universitätsspital Zürich, Zürich, Schweiz | Mitgliedschaften: Foederatio Medicorum Helveticorum (FMH), Verband Schweizerischer Assistenz- und Oberärztinnen und -ärzte (VSAO). J. Distler: A. Finanzielle Interessen: Forschungsförderung von Anamar, AstraZeneca, ARXX, BMS, Boehringer Ingelheim, Cantargia, Celgene, CSL Behring, Exo Therapeutics, Galapagos, GSK, Incyte, Inventiva, Kiniksa, Kyverna, Lassen Therapeutics, Mestag, Sanofi-Aventis, Spica Therapeutics, RedX, UCB und Zenas BioPharma. – Unterstützung bei wissenschaftlichen Publikationen von Boehringer Ingelheim und Endeavor BioMedicines. – Beratungstätigkeit für Active Biotech, Anamar, ARXX, AstraZeneca, Bayer Pharma, Boehringer Ingelheim, Calliditas Therapeutics, Calluna, Galapagos, GSK, Janssen, Kyverna, Ono Pharmaceutical, Merck, Novartis, Quell Therapeutics, Tyra und UCB. – J. Distler ist CEO von 4D Science und wissenschaftlicher Leiter bei FibroCure. – B. Nichtfinanzielle Interessen: Klinikdirektor Universitätsklinikum. Wissenschaftliche Leitung: Die vollständige Erklärung zum Interessenkonflikt der Wissenschaftlichen Leitung finden Sie am Kurs der zertifizierten Fortbildung auf www.springermedizin.de/cme . Der Verlag: erklärt, dass für die Publikation dieser CME-Fortbildung keine Sponsorengelder an den Verlag fließen. Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien. EDAT- 2025/12/01 12:29 MHDA- 2026/01/13 13:15 PMCR- 2025/12/01 CRDT- 2025/12/01 11:14 PHST- 2025/10/10 00:00 [received] PHST- 2025/11/05 00:00 [accepted] PHST- 2026/01/13 13:15 [medline] PHST- 2025/12/01 12:29 [pubmed] PHST- 2025/12/01 11:14 [entrez] PHST- 2025/12/01 00:00 [pmc-release] AID - 10.1007/s00108-025-02024-x [pii] AID - 2024 [pii] AID - 10.1007/s00108-025-02024-x [doi] PST - ppublish SO - Inn Med (Heidelb). 2026 Jan;67(1):63-71. doi: 10.1007/s00108-025-02024-x. Epub 2025 Dec 1. PMID- 27604306 OWN - NLM STAT- MEDLINE DCOM- 20180814 LR - 20181113 IS - 1460-2156 (Electronic) IS - 0006-8950 (Print) IS - 0006-8950 (Linking) VI - 139 IP - 11 DP - 2016 Nov 1 TI - Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations. PG - 2909-2922 LID - 10.1093/brain/aww217 [doi] AB - See Charidimou (doi:10.1093/aww253) for a scientific commentary on this article. Cerebroretinal vasculopathy, hereditary vascular retinopathy, and hereditary endotheliopathy, retinopathy, nephropathy and stroke are neurovascular syndromes initially described as distinct entities. Recently they were shown to be one disease caused by C-terminal frame-shift mutations in TREX1, which was termed ‘retinal vasculopathy with cerebral leukodystrophy’. Here we defined the genetic and clinicopathologic spectrum of this clinically and pathophysiologically poorly characterized and frequently misdiagnosed fatal neurovascular disorder. We identified five different TREX1 mutations in 78 members from 11 unrelated families and by using a standardized study protocol we retrospectively reviewed and aggregated the associated clinical, neuroimaging, and pathology data. Findings were similar across mutations and families. Sixty-four mutation carriers had vascular retinopathy. Neuroimaging revealed (i) punctate, hyperintense, white matter lesions with or without nodular enhancement in 97% of them; (ii) rim-enhancing mass lesions in 84%; and (iii) calcifications in the white matter in 52%. Ninety per cent had clinical manifestations of brain disease, including focal neurological deficits (68%), migraine (59%), cognitive impairment (56%), psychiatric disturbances (42%), and seizures (17%). One mutation carrier had enhancing brain lesions and neurological features but unknown retinopathy status. Additional systemic features included liver disease (78%), anaemia (74%), nephropathy (61%), hypertension (60%), mild Raynaud’s phenomenon (40%), and gastro-intestinal bleeding (27%). Mean (± standard deviation) age at diagnosis was 42.9 ± 8.3 years and at death 53.1 ± 9.6 years. Pathological examination revealed systemic vasculopathy with luminal narrowing and multi-laminated basement membranes. The 13 mutation carriers without retinopathy or brain lesions were on average 8 years younger (mean age: 35.1 ± 10.6 years). Of them, 54% had mild Raynaud’s phenomenon, 42% had migraine, and 23% had psychiatric disturbances. Retinal vasculopathy with cerebral leukodystrophy is an autosomal dominant systemic small-vessel disease due to specific TREX1 mutations and clinically primarily characterized by (i) visual impairment from vascular retinopathy; and (ii) neurological decline and premature death due to progressive enhancing cerebral white matter lesions. Impaired liver and kidney function, anaemia sometimes associated with gastrointestinal bleeding, hypertension, migraine, and Raynaud’s phenomenon appear to be part of the clinical spectrum as well. Penetrance seems high. Because of the pathogenetic basis and the emerging clinical picture with systemic manifestations and conspicuous absence of leukodystrophy, we renamed the disease ‘retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations’. We propose diagnostic criteria to facilitate clinical recognition and future studies. FAU - Stam, Anine H AU - Stam AH AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Kothari, Parul H AU - Kothari PH AD - Department of Medicine, Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri 63110, USA. FAU - Shaikh, Aisha AU - Shaikh A AD - Department of Medicine, Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri 63110, USA. FAU - Gschwendter, Andreas AU - Gschwendter A AD - Institute for Stroke and Dementia Research, Klinikum der Universität München, Ludwig-Maximilians Universität, D-81377 München, Germany. FAU - Jen, Joanna C AU - Jen JC AD - Department of Neurology, University of California at Los Angeles, Los Angeles, California 90095, USA. FAU - Hodgkinson, Suzanne AU - Hodgkinson S AD - Department of Neurology, Liverpool Hospital, Liverpool, New South Wales 2170, Australia. FAU - Hardy, Todd A AU - Hardy TA AD - Department of Neurology, Concord Repatriation General Hospital, Concord, New South Wales 2139, Australia. AD - Brain and Mind Centre, University of Sydney, Australia. FAU - Hayes, Michael AU - Hayes M AD - Department of Neurology, Concord Repatriation General Hospital, Concord, New South Wales 2139, Australia. FAU - Kempster, Peter A AU - Kempster PA AD - Neurosciences Department, Monash Medical Centre, Clayton, Victoria 3168, Australia. FAU - Kotschet, Katya E AU - Kotschet KE AD - Neurosciences Department, Monash Medical Centre, Clayton, Victoria 3168, Australia. FAU - Bajema, Ingeborg M AU - Bajema IM AD - Department of Pathology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - van Duinen, Sjoerd G AU - van Duinen SG AD - Department of Pathology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Maat-Schieman, Marion L C AU - Maat-Schieman MLC AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - de Jong, Paulus T V M AU - de Jong PTVM AD - Department of Ophthalmology, Academic Medical Centre, 1100 DD Amsterdam, The Netherlands. AD - Department of Retinal Signaling, Netherlands Institute for Neuroscience, Royal Netherlands Academy of Arts and Sciences, 1000 GC Amsterdam, The Netherlands. AD - Department of Ophthalmology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - de Smet, Marc D AU - de Smet MD AD - Department of Ophthalmology, Academic Medical Centre, 1100 DD Amsterdam, The Netherlands. FAU - de Wolff-Rouendaal, Didi AU - de Wolff-Rouendaal D AD - Department of Ophthalmology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Dijkman, Greet AU - Dijkman G AD - Department of Ophthalmology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Pelzer, Nadine AU - Pelzer N AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Kolar, Grant R AU - Kolar GR AD - Department of Medicine, Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri 63110, USA. AD - Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Schmidt, Robert E AU - Schmidt RE AD - Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Lacey, JoAnne AU - Lacey J AD - West County Radiology Group, Mercy Hospital in St Louis, MO 63141, USA. FAU - Joseph, Daniel AU - Joseph D AD - The Retina Institute, Department of Ophthalmology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Fintak, David R AU - Fintak DR AD - The Retina Institute, Department of Ophthalmology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Grand, M Gilbert AU - Grand MG AD - The Retina Institute, Department of Ophthalmology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Brunt, Elizabeth M AU - Brunt EM AD - Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Liapis, Helen AU - Liapis H AD - Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, 63110 USA. FAU - Hajj-Ali, Rula A AU - Hajj-Ali RA AD - Department of Rheumatic and Immunologic Disease, Cleveland Clinic, Cleveland, Ohio, 44195 USA. FAU - Kruit, Mark C AU - Kruit MC AD - Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - van Buchem, Mark A AU - van Buchem MA AD - Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Dichgans, Martin AU - Dichgans M AD - Institute for Stroke and Dementia Research, Klinikum der Universität München, Ludwig-Maximilians Universität, D-81377 München, Germany. AD - Munich Cluster for Systems Neurology (SyNergy), Munich, Germany. FAU - Frants, Rune R AU - Frants RR AD - Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands. FAU - van den Maagdenberg, Arn M J M AU - van den Maagdenberg AMJM AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. AD - Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Haan, Joost AU - Haan J AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. AD - Department of Neurology, Alrijne Hospital, Leiderdorp, The Netherlands. FAU - Baloh, Robert W AU - Baloh RW AD - Department of Neurology, University of California at Los Angeles, Los Angeles, California 90095, USA. FAU - Atkinson, John P AU - Atkinson JP AD - Department of Medicine, Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri 63110, USA. FAU - Terwindt, Gisela M AU - Terwindt GM AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Ferrari, Michel D AU - Ferrari MD AD - Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. LA - eng GR - R01 NS062069/NH/NIH HHS/United States GR - HL083822/NH/NIH HHS/United States PT - Journal Article PL - England TA - Brain JT - Brain : a journal of neurology JID - 0372537 SB - IM CIN - Brain. 2016 Nov 1;139(11):2819-2821. doi: 10.1093/brain/aww253. PMID: 29106489 MH - Adult MH - CADASIL/*diagnostic imaging/*epidemiology/genetics MH - Female MH - Humans MH - Leukoencephalopathies/diagnostic imaging/epidemiology/genetics MH - Male MH - Middle Aged MH - Retinal Vasculitis/*diagnostic imaging/*epidemiology/genetics PMC - PMC5091044 OAB - See Charidimou (doi:10.1093/aww253) for a scientific commentary on this article. Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations is a newly recognised and often misdiagnosed neurovascular syndrome caused by mutations in TREX1. Stam et al. provide the first comprehensive clinical, pathological and radiological characterization of RVCL-S, and highlight cerebral mass lesions, impaired liver and kidney function, and premature death. OABL- eng OTO - NOTNLM OT - migraine OT - molecular genetics OT - neuro-ophthalmology OT - neuropathology OT - small vessel disease EDAT- 2016/09/09 06:00 MHDA- 2018/08/15 06:00 PMCR- 2017/11/01 CRDT- 2016/09/09 06:00 PHST- 2015/12/25 00:00 [received] PHST- 2016/07/11 00:00 [accepted] PHST- 2016/09/09 06:00 [pubmed] PHST- 2018/08/15 06:00 [medline] PHST- 2016/09/09 06:00 [entrez] PHST- 2017/11/01 00:00 [pmc-release] AID - aww217 [pii] AID - 10.1093/brain/aww217 [doi] PST - ppublish SO - Brain. 2016 Nov 1;139(11):2909-2922. doi: 10.1093/brain/aww217. PMID- 32185995 OWN - NLM STAT- MEDLINE DCOM- 20200327 LR - 20200327 IS - 2589-451X (Electronic) IS - 0255-2922 (Linking) VI - 38 IP - 5 DP - 2018 Oct TI - Effect of the herbal medicine Danggui Sini plus Wuzhuyu Shengjiang Tang on erythrocyte deformability in normal subjects: a cross-over trial. PG - 769-772 AB - OBJECTIVE: To show whether Danggui Sini plus Wuzhuyu Shengjiang Tang (DSWST) has any transient effect on erythrocyte deformability in normal subjects. METHODS: A total of 25 subjects [mean age (27.8 ¡À 1.8) years] was enrolled in this study. The study was designed as a cross-over trial in which the subjects took part for 2 d. On the first day, blood samples were collected at baseline and 1-2 h after administration of water, whereas, on the second day, instead of water, the subjects were administered DSWST after the baseline blood sampling. The blood samples collected at baseline and after the administration water or DSWST, were examined for erythrocyte deformability. RESULTS: The elongation index increased significantly after 2 h (P = 0.009) compared to the baseline after DSWST intake. However, after water intake, there was no significant difference observed. When comparing the percent change of erythrocyte deformability between DSWST and water, we found that after 2 h of administration, DSWST improved erythrocyte deformability significantly compared to water (P < 0.001). CONCLUSION: DSWST has a transient effect on erythrocyte deformability in normal subjects. FAU - Ji, Eun Lee AU - Ji EL AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Seung-Yeon, Cho AU - Seung-Yeon C AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Seong-Uk, Park AU - Seong-Uk P AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Woo-Sang, Jung AU - Woo-Sang J AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Sang-Kwan, Moon AU - Sang-Kwan M AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Jung-Mi, Park AU - Jung-Mi P AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Chang-Nam, Ko AU - Chang-Nam K AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Ki-Ho, Cho AU - Ki-Ho C AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. FAU - Seungwon, Kwon AU - Seungwon K AD - Department of Cardiology and Neurology, College of Korean Medicine, Kyung Hee University, Seoul 02447, Korea. LA - eng PT - Journal Article PT - Randomized Controlled Trial PL - China TA - J Tradit Chin Med JT - Journal of traditional Chinese medicine = Chung i tsa chih ying wen pan JID - 8211546 RN - 0 (Drugs, Chinese Herbal) SB - IM MH - Adult MH - Cross-Over Studies MH - Drugs, Chinese Herbal/*administration & dosage MH - Erythrocyte Deformability/*drug effects MH - Female MH - Humans MH - Male OTO - NOTNLM OT - Coldness of hands and feet OT - Cross-over studies OT - Danggui Sini plus Wuzhuyu Shengjiang Tang OT - Erythrocyte deformability OT - Peptide elongation factors OT - Raynaud disease EDAT- 2018/10/01 00:00 MHDA- 2020/03/28 06:00 CRDT- 2020/03/19 06:00 PHST- 2020/03/19 06:00 [entrez] PHST- 2018/10/01 00:00 [pubmed] PHST- 2020/03/28 06:00 [medline] AID - 2830 [pii] PST - ppublish SO - J Tradit Chin Med. 2018 Oct;38(5):769-772. PMID- 41721344 OWN - NLM STAT- In-Process LR - 20260330 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 24 IP - 1 DP - 2026 Feb 20 TI - Juvenile Sjögren disease: time for age-specific classification criteria. LID - 10.1186/s12969-026-01196-6 [doi] LID - 15 AB - OBJECTIVE: This study aims to report the clinical, laboratory, and imaging manifestations of juvenile Sjögren’s disease (SjD) and highlight the role of large salivary gland ultrasound and small salivary gland biopsy for the purpose of diagnosis and classification in this age group. BACKGROUND: Currently, there are no unified classification criteria for juvenile SjD, and pediatric-specific literature is scarce. The current literature lacks validated pediatric-specific classification and diagnostic tools. Many tests used in adult classification criteria are not routinely assessed in children because they can be difficult to perform on younger patients and lack age-specific thresholds for abnormal results. This study aims to help build the foundation for future proposals to specifically classify SjD in the pediatric age group. METHODS: This was a single-center exploratory pilot retrospective study of medical records of patients diagnosed with juvenile SjD over the past five years. RESULTS: Fifteen patients with juvenile SjD were included. The median age of disease onset was 11.5 years (range 8.5–17.5 years), and the median age at diagnosis was 12.5 years (range 8.5–17.5 years). Ten patients had primary SjD, and five patients had SjD secondary to systemic lupus erythematosus and limited scleroderma. The clinical manifestations at onset included musculoskeletal manifestations (n = 9), recurrent parotitis (n = 7), mucocutaneous manifestations (n = 6), cervical lymphadenopathy (n = 6), Raynaud’s phenomenon or acrocyanosis (n = 3), fever (n = 2), dysphagia (n = 2), respiratory symptoms (n = 2), and sicca manifestations (n = 2). One patient with primary SjD presented with life-threatening macrophage activation syndrome. Sarcoidosis, IgG-4-related disease, and relevant infections were ruled out in all patients. Anti-SSA antibodies were positive in 13/15 patients. All patients had abnormal large salivary gland ultrasound findings, including heterogeneous echogenicity (14/15), gland enlargement (14/15), hyperemia (11/14), cystic changes (10/15), cervical lymphadenopathy (8/15), and intraductal mucus plugging or calcifications (3/15). Small salivary gland biopsies from 13/13 patients were negative for granulomas and malignancies and revealed lymphocytic sialoadenitis with a focus score of ≥1. CONCLUSION: Recurrent parotitis and scarce sicca manifestations are two distinct clinical features of juvenile versus adult SjD. Large salivary gland ultrasound study is a promising practical tool that should be considered in suspected juvenile SjD patients. These clinical and imaging features should be considered in any proposed classification criteria for juvenile SjD. FAU - Davis, Michael AU - Davis M AD - Authority Health GME Consortium Pediatrics Residency Program, Children's Hospital of Michigan, Detroit, MI, USA. FAU - Altinok, Deniz AU - Altinok D AD - Department of Pediatric Imaging, Wayne State University, Children's Hospital of Michigan, Detroit, MI, USA. FAU - Poulik, Janet AU - Poulik J AD - Department of Pathology, Wayne State University, Children's Hospital of Michigan, Detroit, MI, USA. FAU - Thomas, Ronald AU - Thomas R AD - Department of Pediatrics, Children's Research Institute, Central Michigan University School of Medicine, Detroit, MI, USA. FAU - Fathalla, Basil M AU - Fathalla BM AUID- ORCID: 0000-0002-5964-9088 AD - Department of Allergy, Immunology and Rheumatology, Central Michigan University, Children's Hospital of Michigan, Detroit, MI, USA. bfathall@dmc.org. LA - eng PT - Journal Article DEP - 20260220 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM PMC - PMC13032440 OTO - NOTNLM OT - Juvenile Sjögren disease OT - Recurrent Parotitis OT - Sicca symptoms OT - Sjögren disease classification COIS- Declarations. Ethics approval and consent to participate: Our study received exemption from the Central Michigan University Institutional Review Board (part of study number 2022–619) according to the U.S. Department of Health and Human Services’ regulations. In addition, the need for consent to participate was waived by the review committee. Consent for publication: The need for consent to participate was waived by the Institutional Review Board. Competing interests: The authors declare no competing interests. EDAT- 2026/02/21 00:40 MHDA- 2026/02/21 00:40 PMCR- 2026/02/20 CRDT- 2026/02/20 23:52 PHST- 2025/11/12 00:00 [received] PHST- 2026/02/16 00:00 [accepted] PHST- 2026/02/21 00:40 [pubmed] PHST- 2026/02/21 00:40 [medline] PHST- 2026/02/20 23:52 [entrez] PHST- 2026/02/20 00:00 [pmc-release] AID - 10.1186/s12969-026-01196-6 [pii] AID - 1196 [pii] AID - 10.1186/s12969-026-01196-6 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2026 Feb 20;24(1):15. doi: 10.1186/s12969-026-01196-6. PMID- 27999501 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220410 IS - 1524-5012 (Print) IS - 1524-5012 (Linking) VI - 16 IP - 4 DP - 2016 Winter TI - Axillary Block-Induced Chemical Sympathectomy in the Setting of Digital Ischemia. PG - 450-456 AB - BACKGROUND: Digital ischemia is associated with several rheumatologic disorders and is often difficult to treat. Symptoms and sequelae can include pain, disability, need for amputation, and poor quality of life. METHODS: Patients diagnosed with digital ischemia were referred for an ultrasound-guided axillary nerve block using liposomal bupivacaine (Exparel, Pacira Pharmaceuticals). The primary outcome measure was radial and ulnar artery diameter preprocedure and postprocedure. Doppler waveform analyses were performed to measure arterial diameter and blood flow velocity. The QuickDASH questionnaire was administered to evaluate upper extremity function and perceived disability. RESULTS: Mean radial and ulnar artery diameters increased from 0.19 cm and 0.16 cm to 0.23 cm and 0.20 cm, respectively, 1 hour postprocedure. Concomitant increases in blood flow velocities and hand temperature and lower pain scores were also noted. Although pain generally returned to baseline after 1 week, QuickDASH symptom/disability scores improved at 30 days, and 2 patients' ischemic symptoms resolved spontaneously during the study period in the absence of other interventions. CONCLUSION: Data regarding chemical sympathectomy using regional anesthesia are limited at this time. Our experience suggests a potential role in the treatment and evaluation of digital ischemia. Patients with digital ischemia from rheumatologic conditions appeared to have a greater benefit from a chemical sympathectomy than patients whose conditions had an atherosclerotic or anatomic etiology. Even when the vasodilatory effects are transient, such an intervention may be useful when a more permanent option such as surgical sympathectomy is being considered. Liposomal bupivacaine is only approved for surgical infiltration at this time. FAU - Soberón, José R Jr AU - Soberón JR Jr AD - Department of Anesthesiology, Ochsner Clinic Foundation, New Orleans, LA. FAU - Truxillo, T Michael AU - Truxillo TM AD - Department of Anesthesiology, Ochsner Clinic Foundation, New Orleans, LA ; The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA. FAU - Gethers, Christin C AU - Gethers CC AD - Department of Anesthesiology, Ochsner Clinic Foundation, New Orleans, LA. FAU - Smith, Taylor A AU - Smith TA AD - The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA ; Department of Surgery, Ochsner Clinic Foundation, New Orleans, LA. FAU - Davis, William E AU - Davis WE AD - The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA ; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA. LA - eng PT - Journal Article PL - United States TA - Ochsner J JT - Ochsner journal JID - 101125795 PMC - PMC5158149 OTO - NOTNLM OT - Anesthesia–conduction OT - CREST syndrome OT - Raynaud disease OT - bupivacaine OT - nerve block OT - scleroderma–systemic OT - sympathectomy EDAT- 2016/12/22 06:00 MHDA- 2016/12/22 06:01 PMCR- 2016/12/01 CRDT- 2016/12/22 06:00 PHST- 2016/12/22 06:00 [entrez] PHST- 2016/12/22 06:00 [pubmed] PHST- 2016/12/22 06:01 [medline] PHST- 2016/12/01 00:00 [pmc-release] AID - TOJ-16-0048 [pii] PST - ppublish SO - Ochsner J. 2016 Winter;16(4):450-456. PMID- 34972595 OWN - NLM STAT- MEDLINE DCOM- 20220318 LR - 20220318 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 52 DP - 2022 Feb TI - A prospective observational cohort study and systematic review of 40 patients with mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome. PG - 151924 LID - S0049-0172(21)00197-9 [pii] LID - 10.1016/j.semarthrit.2021.10.007 [doi] AB - OBJECTIVE: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To date, no studies have defined the clinical spectrum of disease in a cohort of patients with MAGIC syndrome. METHODS: Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome by searching four databases: PubMed (US National Library of Medicine), Embase (Elsevier), Scopus (Elsevier) and Web of Science: Core Collection (Clarivate Analytics). The inclusion criteria used were: [1] patients of any age or gender who were diagnosed with MAGIC syndrome, or both RP and BD; [2] case report or case series study; [3] published from 1985 - July 2020; and [4] in English language. Risk of bias was assessed using a checklist developed by the authors and based on the Consensus-based Clinical Case Reporting (CARE) Guidelines. Search results screening, article inclusion, data extraction and risk of bais assessment was performed independently by two investigators. Clinical characteristics, particularly BD-related features, were compared between patients with MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of different criteria to classify MAGIC syndrome were also evaluated. RESULTS: Out of 96 patients with RP, 13 (14%) patients were diagnosed with MAGIC syndrome. For the systematic review, 380 articles were retrieved of which 90 were screened at title and abstract levels. Of these screened, 60 were excluded and 30 proceeded to full text review where an additional 8 were excluded. Twenty-two articles were included in our review and from which 27 additional cases of MAGIC syndrome were identified. Pooling all 40 cases together and comparing them with non-MAGIC RP, there was a significantly higher prevalence of ocular involvement (28% vs 4%, p<0.01), cutaneous involvement (35% vs 1%, p<0.01), GI involvement (23% vs 4%, p<0.01), and CNS involvement (8% vs 0, p = 0.04) in MAGIC syndrome. A higher prevalence of aortitis (23% vs 1%, p<0.01), Raynaud's phenomenon (54% vs 11%, p<0.01), and elevated anti-collagen II antibodies (50% vs 9%, p = 0.04) were observed in MAGIC syndrome. Fulfillment of either McAdam's or Damiani's Criteria for RP plus the International Criteria for Behçet's Disease had excellent sensitivity (98%) to classify cases of MAGIC syndrome. CONCLUSION: A substantial proportion of patients with RP can be clinically diagnosed with MAGIC syndrome. These patients have features of RP, BD, and other unique features including aortitis, Raynaud's phenomenon and elevated anti-collagen II antibodies. CI - Copyright © 2021. Published by Elsevier Inc. FAU - Luo, Yiming AU - Luo Y AD - Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States; Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Bolek, Ertugrul Cargi AU - Bolek EC AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States; Hacettepe University Vasculitis Research Centre, Ankara, Turkey. FAU - Quinn, Kaitlin A AU - Quinn KA AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Wells, Kristina AU - Wells K AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Rose, Emily AU - Rose E AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Sikora, Keith AU - Sikora K AD - Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Goodspeed, Wendy AU - Goodspeed W AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Rominger, Emily AU - Rominger E AD - Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Kilic, Levent AU - Kilic L AD - Hacettepe University Vasculitis Research Centre, Ankara, Turkey; Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey. FAU - Karadag, Omer AU - Karadag O AD - Hacettepe University Vasculitis Research Centre, Ankara, Turkey; Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey. FAU - Clark, Cindy AU - Clark C AD - National Institutes of Health Library, Office of Research Services, National Institutes of Health, Bethesda, United States. FAU - Livinski, Alicia A AU - Livinski AA AD - National Institutes of Health Library, Office of Research Services, National Institutes of Health, Bethesda, United States. FAU - Grayson, Peter C AU - Grayson PC AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. FAU - Ferrada, Marcela A AU - Ferrada MA AD - Vasculitis Translational Research Program, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, United States. Electronic address: marcela.ferrada@nih.gov. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Intramural PT - Systematic Review DEP - 20211114 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adult MH - *Behcet Syndrome/complications MH - Cartilage MH - Genitalia MH - Humans MH - Mouth MH - Observational Studies as Topic MH - Prospective Studies MH - *Ulcer MH - United States OTO - NOTNLM OT - Behcet's disease OT - Magic syndrome OT - Mouth and genital ulcers with inflamed cartilage syndrome OT - Relapsing polychondritis COIS- Declaration of Competing Interest None. EDAT- 2022/01/02 06:00 MHDA- 2022/03/19 06:00 CRDT- 2022/01/01 05:15 PHST- 2021/08/12 00:00 [received] PHST- 2021/10/19 00:00 [revised] PHST- 2021/10/27 00:00 [accepted] PHST- 2022/01/02 06:00 [pubmed] PHST- 2022/03/19 06:00 [medline] PHST- 2022/01/01 05:15 [entrez] AID - S0049-0172(21)00197-9 [pii] AID - 10.1016/j.semarthrit.2021.10.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Feb;52:151924. doi: 10.1016/j.semarthrit.2021.10.007. Epub 2021 Nov 14. PMID- 40083480 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250318 IS - 2072-1439 (Print) IS - 2077-6624 (Electronic) IS - 2072-1439 (Linking) VI - 17 IP - 2 DP - 2025 Feb 28 TI - Thoracic autonomic nervous system surgery current application-a survey among members of the European Society of Thoracic Surgeons. PG - 979-990 LID - 10.21037/jtd-24-1167 [doi] AB - BACKGROUND: Thoracic autonomic nervous system surgery is mainly used for hyperhidrosis/facial flushing, whereas cardiac and vascular indications are limited. The literature remains controversial regarding the correct indications and surgical technique, with the lack of homogeneous data being a major limitation. We designed a survey to investigate current practice among members of the European Society of Thoracic Surgeons (ESTS). METHODS: A 29-question ad hoc questionnaire was available to all ESTS members from December 2022 to February 2023. It included questions on demographics, indications, preoperative evaluation, technique, complications and follow-up. A descriptive analysis of the data is presented. RESULTS: The response rate was 7% and 121 of 123 valid responses were analysed. Sympathetic surgery was performed for hyperhidrosis/facial flushing, cardiac and vascular disease in 99%, 29% and 29% of respondents respectively. Palmar hyperhidrosis was the most common, followed by axillary, facial flushing and craniofacial hyperhidrosis. Catecholaminergic ventricular tachycardia was more common than long QT syndrome and Raynaud's over Buerger's disease. Data analysis showed that members preferred nerve cutting to clipping (66%, 64% and 58% for hyperhidrosis/facial flushing, cardiac and vascular disease respectively). Preference for the target level of nerve block varied significantly depending on the condition addressed. For most responders (65%), severe compensatory sweating was an adverse event, occurring in less than 10% of treated cases. Only 52% used a database for follow-up. CONCLUSIONS: Current practice in sympathetic surgery in ESTS responders is consistent with the available evidence, although it is characterized by great heterogeneity in almost all aspects. A database could help to standardize patient selection, surgical techniques and follow-up, and provide the basis for future multi-institutional trials. CI - Copyright © 2025 AME Publishing Company. All rights reserved. FAU - Raveglia, Federico AU - Raveglia F AD - Thoracic Surgery, IRCCS San Gerardo dei Tintori, Monza, Italy. FAU - Lugaresi, Marialuisa AU - Lugaresi M AD - Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy. FAU - Furak, Jozsef AU - Furak J AD - Department of Surgery, University of Szeged, Szeged, Hungary. FAU - Batirel, Hasan Fevzi AU - Batirel HF AD - Department of Thoracic Surgery, Biruni University School of Medicine Istanbul, Istanbul, Turkey. FAU - Bolukbas, Servet AU - Bolukbas S AD - Department of Thoracic Surgery and Thoracic Endoscopy, University Medical Center Essen - Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany. FAU - Falcoz, Pierre Emmanuel AU - Falcoz PE AD - Department of Thoracic Surgery, Nouvel Hôpital Civil, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. FAU - Agrafiotis, Apostolos C AU - Agrafiotis AC AD - Thoracic and Vascular Surgery, Wallonie Picarde Hospital Center, Tournai, Belgium. FAU - Aigner, Clemens AU - Aigner C AD - Department of Thoracic Surgery, Comprehensive Center of Chest Disease, Medical University of Vienna, Vienna, Austria. FAU - Depypere, Lieven AU - Depypere L AD - Department of Thoracic Surgery, University Hospital Leuven, Leuven, Belgium. FAU - Silva, Joao Santos AU - Silva JS AD - Cardiothoracic Surgery Department, Hospital de Santa Marta, University Hospital Centre Lisboa Central, Lisboa, Portugal. FAU - Novoa, Nuria Maria AU - Novoa NM AD - Department of Thoracic Surgery, University Hospital Puerta de Hierro-Majadahonda, Madrid, Spain. FAU - Daddi, Niccolò AU - Daddi N AD - Department of Thoracic Surgery, IRCCS Azienda Ospedaliero Universitaria di Bologna Policlinico S. Orsola-Malpighi, University of Bologna, Bologna, Italy. LA - eng PT - Journal Article DEP - 20250227 PL - China TA - J Thorac Dis JT - Journal of thoracic disease JID - 101533916 PMC - PMC11898363 OTO - NOTNLM OT - European Society of Thoracic Surgeons (ESTS) OT - Survey OT - autonomic nervous system OT - hyperhidrosis OT - sympathetic surgery COIS- Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-24-1167/coif). H.F.B. reports that he receives consulting fees from Medtronic and Johnson & Johnson. N.M.N. reports that she had leadership roles within the ESTS and advisory board participation for 2 companies J&J and AZ, and had received payment from Medela about Educational event 2022. The other authors have no conflicts of interest to declare. EDAT- 2025/03/14 11:23 MHDA- 2025/03/14 11:24 PMCR- 2025/02/28 CRDT- 2025/03/14 04:46 PHST- 2024/08/06 00:00 [received] PHST- 2024/10/17 00:00 [accepted] PHST- 2025/03/14 11:24 [medline] PHST- 2025/03/14 11:23 [pubmed] PHST- 2025/03/14 04:46 [entrez] PHST- 2025/02/28 00:00 [pmc-release] AID - jtd-17-02-979 [pii] AID - 10.21037/jtd-24-1167 [doi] PST - ppublish SO - J Thorac Dis. 2025 Feb 28;17(2):979-990. doi: 10.21037/jtd-24-1167. Epub 2025 Feb 27. PMID- 42123049 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260513 LR - 20260515 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 15 IP - 9 DP - 2026 Apr 27 TI - Anatomy of a Cohort: 40-Year Follow-Up of a Sjögren's Cohort. LID - 10.3390/jcm15093316 [doi] LID - 3316 AB - Background: Sjögren's disease (SjD) is a chronic autoimmune rheumatic disorder primarily affecting exocrine glands, leading to dryness and systemic involvement. B-cell hyperactivity and autoantibody production drive its pathogenesis and contribute to increased lymphoma risk. Although several long-term studies exist, we present a review of a closely monitored cohort assessed over 40 years. Methods: Retrospective observational study at University College London Hospital included patients fulfilling the 2016 ACR/EULAR criteria for SjD between 1986-2025. Patients with associated SjD were excluded. Associations between serological markers and clinical features were analysed using chi-square or Fisher's exact tests (p < 0.05). Differences between ethnic groups were also assessed. Results: 283 patients were included, 93.3% female, with mean age at diagnosis of 50.1 ± 15.2 years and mean follow-up of 12.5 ± 8.6 years. Common manifestations were fatigue (61.5%), parotid swelling (30.5%), arthritis (25.8%), and Raynaud's phenomenon (27.6%). Anti-Ro and anti-La antibodies were present in 75.7% and 45.2%, respectively; rheumatoid factor in 57.3%. Lymphoma developed in 9.9% (mostly non-Hodgkin MALT) and was associated with hypergammaglobulinemia (p = 0.03; RR = 2.56) and parotid swelling (p < 0.001; RR = 5.53). Serological markers correlated with systemic features including lymphadenopathy, vasculitis, and pulmonary involvement. Caucasian patients showed higher mortality (p < 0.001; RR = 3.89) and peripheral nervous system involvement (p = 0.02; RR = 2.18), and less ANA positivity (p = 0.004; RR = 0.88), anti-Ro (p = <0.001; RR = 0.77) and RF (p = 0.04; RR = 0.81) and hypergammaglobulinemia (p = <0.001; RR = 0.63) when compared with non-Caucasian patients. Conclusions: This long-term cohort confirms the strong association between B-cell activation markers and adverse outcomes in Sjögren's disease. Hypergammaglobulinemia and parotid swelling emerged as key predictors of lymphoma, supporting their role in risk stratification. These findings reinforce the importance of long-term monitoring and may help guide personalized clinical management and surveillance strategies. FAU - Viejo-Sosa, Blanca AU - Viejo-Sosa B AUID- ORCID: 0009-0002-0732-0931 AD - Servicio de Reumatología, Hospital Universitario Jerez de la Frontera, 11407 Cádiz, Spain. FAU - Couto-Lareo, Uxía AU - Couto-Lareo U AUID- ORCID: 0009-0004-7794-551X AD - Servicio de Reumatología, Hospital Universitario A Coruña, 15006 A Coruña, Spain. FAU - Angerri-Nadal, Mònica AU - Angerri-Nadal M AUID- ORCID: 0000-0002-4007-6320 AD - Servei Medicina Interna, Hospital Universitari Dr. Josep Trueta, 17007 Girona, Spain. FAU - Isenberg, David A AU - Isenberg DA AUID- ORCID: 0000-0001-9514-2455 AD - Department of Ageing, Rheumatology and Regenerative Medicine, University College London, London WC1E 6BT, UK. LA - eng PT - Journal Article DEP - 20260427 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC13164210 OTO - NOTNLM OT - Sjogren’s Disease OT - ethnic groups OT - long-term follow-up OT - lymphoma OT - serological pattern COIS- The authors declare no conflict of interest. EDAT- 2026/05/13 06:31 MHDA- 2026/05/13 06:32 PMCR- 2026/04/27 CRDT- 2026/05/13 01:09 PHST- 2026/03/31 00:00 [received] PHST- 2026/04/19 00:00 [revised] PHST- 2026/04/22 00:00 [accepted] PHST- 2026/05/13 06:32 [medline] PHST- 2026/05/13 06:31 [pubmed] PHST- 2026/05/13 01:09 [entrez] PHST- 2026/04/27 00:00 [pmc-release] AID - jcm15093316 [pii] AID - jcm-15-03316 [pii] AID - 10.3390/jcm15093316 [doi] PST - epublish SO - J Clin Med. 2026 Apr 27;15(9):3316. doi: 10.3390/jcm15093316. PMID- 37805891 OWN - NLM STAT- MEDLINE DCOM- 20231101 LR - 20231101 IS - 1512-0112 (Print) IS - 1512-0112 (Linking) IP - 340-341 DP - 2023 Jul-Aug TI - DIAGNOSTIC VALUE OF LABORATORY MARKERS OF SYNTROPIC LESIONS OF THE CIRCULATORY SYSTEM ORGANS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS. PG - 159-164 AB - Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects almost all internal organs, among which circulatory system organs (CSO) lesions are not only among the most common but also at the top of the list of causes of mortality. The tactics of treatment of patients with SLE without and in combination with CSO lesions are fundamentally different, and therefore, improving diagnostic methods will help to enhance the effectiveness of the management of this category of patients. The aim of the study - to determine the diagnostic value of laboratory markers of syntropic lesions of the circulatory system organs in patients with systemic lupus erythematosus. The research included 125 patients with SLE with CSO lesions, among whom the vast majority were young women. Patients were stratified according to syntropy. Syntropic lesions were those whose frequency significantly increased with increasing severity of SLE: retinal angiopathy, capillaritis, Raynaud's syndrome, livedo reticularis, atherosclerosis, mitral valve insufficiency, mitral valve thickening, pericardial effusion, pulmonary hypertension, myocarditis, endocarditis, symptomatic arterial hypertension, and vein thrombosis. During the study, the diagnostic value of individual laboratory markers and their constellations in terms of sensitivity, specificity, and accuracy in patients with SLE with syntropic lesions of CSO was determined step by step, and the one with the highest diagnostic value for the diagnosis of these lesions was chosen. The difference was considered statistically significant if p<0.050. The association coefficient and the contingent coefficient were used to determine the closeness of the relationship between the marker and the syntropic lesion. The relationship was considered confirmed if the association coefficient was ≥ 0.50 or the contingent coefficient was ≥ 0.30. We studied the diagnostic value of individual laboratory markers and their constellations in terms of sensitivity, specificity, and accuracy in patients with SLE with syntropic CSO lesions. It was found that the best diagnostic value for the diagnosis of retinal angiopathy is the constellation of ↑ LDL + ↑ IA + ↑ anti-ds DNA + ↑ ANA; capillaritis - ↑ β-globulins + ↑ IA + ↑ anti-ds DNA + ↑ antiphospholipid antibodies Ig M + ↑ anti-Sm + ↓ C4; Raynaud's syndrome - a separate marker ↓ C3; livedo reticularis - ↑ ESR + ↑ small CIC + ↑ anti-ds DNA + ↑ anti-Sm; atherosclerosis - ↓ hemoglobin + ↑ LDL + ↑ ANA + ↓ C4; mitral valve insufficiency - ↑ ESR + ↑ anti-ds DNA + ↑ ANA + ↑ antiphospholipid antibodies Ig M; mitral valve stenosis - ↑ ESR+↑ LDL + ↑ small CK + ↑ ANA; pericardial effusion - erythropenia + ↑ C-RP + ↑ lupus anticoagulant; pulmonary hypertension - hypercholesterolemia + ↑ LDL + ↑ anti-ds DNA + ↑ ANA; myocarditis - an individual marker ↓ C4; endocarditis - ↑ ESR + ↑ total fibrinogen + ↑ γ-globulins + hypercholesterolemia + ↑ anti-Sm; symptomatic arterial hypertension - ↑ LDL + ↑ anti-ds DNA + ↑ ANA + ↑ anti-SSA (Ro); vein thrombosis - erythropenia + ↓ hemoglobin + ↑ LDL + ↑ ANA. For each syntropic lesion in patients with systemic lupus erythematosus, an individual laboratory marker or constellations have been identified that having the best diagnostic value for the diagnosis of these lesions. FAU - Kobak, L AU - Kobak L AD - 1Danylo Halytsky Lviv National Medical University, Ukraine. FAU - Abrahamovych, O AU - Abrahamovych O AD - 1Danylo Halytsky Lviv National Medical University, Ukraine. FAU - Abrahamovych, U AU - Abrahamovych U AD - 1Danylo Halytsky Lviv National Medical University, Ukraine. FAU - Maksymuk, A AU - Maksymuk A AD - 2Ivan Franko National University of Lviv, Ukraine. FAU - Ivanochko, R AU - Ivanochko R AD - 1Danylo Halytsky Lviv National Medical University, Ukraine. LA - eng PT - Journal Article PL - Georgia (Republic) TA - Georgian Med News JT - Georgian medical news JID - 101218222 RN - 0 (Biomarkers) RN - 0 (Antibodies, Antiphospholipid) RN - 9007-49-2 (DNA) RN - 0 (Hemoglobins) SB - IM MH - Humans MH - Female MH - *Myocarditis MH - *Livedo Reticularis MH - *Hypertension, Pulmonary MH - *Pericardial Effusion MH - *Hypercholesterolemia MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - Biomarkers MH - *Hypertension MH - *Endocarditis MH - Antibodies, Antiphospholipid MH - *Cardiovascular System MH - *Atherosclerosis MH - DNA MH - Hemoglobins EDAT- 2023/10/08 12:41 MHDA- 2023/11/01 12:44 CRDT- 2023/10/08 07:23 PHST- 2023/11/01 12:44 [medline] PHST- 2023/10/08 12:41 [pubmed] PHST- 2023/10/08 07:23 [entrez] PST - ppublish SO - Georgian Med News. 2023 Jul-Aug;(340-341):159-164. PMID- 34393235 OWN - NLM STAT- MEDLINE DCOM- 20210817 LR - 20220716 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 53 IP - 4 DP - 2021 Aug 18 TI - [Application of lymphocytes test in peripheral blood of patients with systemic sclerosis during the treatment]. PG - 721-727 AB - OBJECTIVE: To explore the significance of lymphocytes in systemic sclerosis (SSc), by detecting the levels of T lymphocytes, B lymphocytes and natural killer (NK) cells, and analyzing the correlation between the lymphocytes and clinical laboratory indexes. METHODS: The numbers and proportion of T, CD4(+)T, CD8(+)T, B, and NK cells were detected by flow cytometry in peripheral blood of 32 SSc patients who had taken immunosuppressive drugs and 30 healthy controls (HC). The comparison of the lymphocyte subsets in SSc with them in the HC groups, and the correlation between the lymphocytes and other clinical and laboratory indicators were analyzed by the relevant statistical analysis. RESULTS: Compared with the HC group, the numbers of T, CD4(+)T, CD8(+)T, and NK cells in peripheral blood of SSc group, who had taken immunosuppressive drugs, were significantly decreased (P < 0.05). More-over, the proportion of NK cells in peripheral blood of the SSc group was also significantly lower than that in the HC group (P=0.004). In addition, all the lymphocyte subsets were decreased in peripheral blood of more than 65% of the SSc patients who had taken immunosuppressive drugs. Compared with CD4(+)T normal group, the positivity of Raynaud's phenomenon, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) was significantly increased in CD4(+)T reduction group, respectively (P＝0.024, P < 0.001, P=0.018). ESR was higher in CD8(+)T reduction group than CD8(+)T normal group (P＝0.022). The prevalence of fingertip ulcer was significantly increased in B cell decrease group (P=0.019). Compared with NK cell normal group, the prevalence of fingertip ulcer was significantly increased in NK cell lower group (P=0.033), IgM was remarkablely decreased yet (P=0.049). The correlation analysis showed that ESR was negatively correlated with the counts of T lymphocytes (r＝－0.455, P＝0.009), CD4(+)T lymphocytes (r＝－0.416, P＝0.018), CD8(+)T lymphocytes (r＝－0.430, P=0.014), B cells (r＝－0.366, P＝0.039). CONCLUSION: The number of CD4(+)T, CD8(+)T, B, and NK cells significantly decreased in peripheral blood of SSc patients who had used immunosuppressive drugs, some lymphocyte subsets might be related with Raynaud's phenomenon and fingertip ulcer, and reflected the disease activity by negatively correlated with ESR and CRP; the numbers of lymphocyte subsets in peripheral blood should be detected regularly in SSc patients who had taken immunosuppressive drugs. FAU - Ma, X B AU - Ma XB AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. AD - Department of Rheumatology and Immunology, Handan First Hospital, Handan 056002, Hebei, China. FAU - Zhang, X W AU - Zhang XW AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Jia, R L AU - Jia RL AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Gao, Y AU - Gao Y AD - Department of Endocrinology, Peking University People's Hospital, Beijing 100044, China. FAU - Liu, H J AU - Liu HJ AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Liu, Y F AU - Liu YF AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. AD - Department of Rheumatology and Immunology, Second Hospital of Shanxi Medical University, Taiyuan 030000, China. FAU - Li, Y N AU - Li YN AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - B-Lymphocytes MH - Flow Cytometry MH - Humans MH - Killer Cells, Natural MH - *Lymphocyte Subsets MH - *Scleroderma, Systemic MH - T-Lymphocyte Subsets MH - T-Lymphocytes PMC - PMC8365082 OTO - NOTNLM OT - B lymphocytes OT - Natural killer cells OT - Systemic sclerosis OT - T lymphocytes EDAT- 2021/08/17 06:00 MHDA- 2021/08/18 06:00 PMCR- 2021/08/18 CRDT- 2021/08/16 05:38 PHST- 2021/08/16 05:38 [entrez] PHST- 2021/08/17 06:00 [pubmed] PHST- 2021/08/18 06:00 [medline] PHST- 2021/08/18 00:00 [pmc-release] AID - bjdxxbyxb-53-4-721 [pii] AID - 10.19723/j.issn.1671-167X.2021.04.017 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Aug 18;53(4):721-727. doi: 10.19723/j.issn.1671-167X.2021.04.017. PMID- 36274012 OWN - NLM STAT- MEDLINE DCOM- 20221116 LR - 20230301 IS - 1878-0539 (Electronic) IS - 1748-6815 (Linking) VI - 75 IP - 12 DP - 2022 Dec TI - Long-term survival analysis of free flap reconstruction in patients with collagen vascular disorders. PG - 4371-4378 LID - S1748-6815(22)00493-4 [pii] LID - 10.1016/j.bjps.2022.08.052 [doi] AB - BACKGROUND: Collagen vascular disorders (CVD) are inflammatory diseases that can affect the blood vessels and soft tissues. Patients with CVD are often immunosuppressed, prone to hyper-coagulation, and represent a challenging patient cohort for free tissue transfer. METHODS: A retrospective review of patients with CVD who underwent free flap reconstructions from 2000-2020 was performed at our institution. Inclusion criteria were patients 18 years old or older with the clinical diagnosis of CVD, including rheumatoid arthritis, Raynaud phenomenon, systemic lupus erythematosus, scleroderma, and sarcoidosis. A time-to-event analysis was performed to identify predictors of surgical complications. RESULTS: A total of 78 patients and 96 free flaps were included. The most common CVD were rheumatoid arthritis (n=36) and Raynaud's phenomenon (n=9). Type of flap included abdominal-based flap (26%), trunk-based flaps (32.3%), and extremity-based flaps (19.8%). The mean age was 56.7±14.6 years, and the mean BMI was 27.5±5.9 kg/m2. Antibody positivity was present in 25.6% of patients; 59% were on chronic steroids, 6.4% were on chronic anticoagulation, 35.9% had radiation therapy, and 29.5% had chemotherapy. Nine percent of patients had a history of prior flap loss, and 11.5% had a history of DVT or arterial thrombosis. The flap loss rate was 3.8%. Steroid treatment was associated with an increased risk of major complications after adjusting for the type of flap HR 2.5(1.3-4.9), p= 0.01. Specifically associated with a higher risk of cellulitis, OR 5.1 (1.1-24.5), p=0.02, and abscess, OR 5.7 (1.2-27.1), p=0.01. CONCLUSION: Free flap reconstruction can be safely performed in patients with CVD. Perioperative optimization of steroids is important to promote wound healing and stabilize disease activity. CI - Copyright © 2022 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. FAU - Yan, Maria AU - Yan M AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. FAU - Rajput, Sanjna AU - Rajput S AD - Mayo Clinic Alix School of Medicine, Rochester, MN. FAU - Singh, Kuldeep AU - Singh K AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. FAU - Carlsen, Brian T AU - Carlsen BT AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. FAU - Moran, Steven L AU - Moran SL AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. FAU - Martinez-Jorge, Jorys AU - Martinez-Jorge J AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. FAU - Vijayasekaran, Aparna AU - Vijayasekaran A AD - Division of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN. Electronic address: Vijayasekaran.Aparna@mayo.edu. LA - eng PT - Journal Article DEP - 20220824 PL - Netherlands TA - J Plast Reconstr Aesthet Surg JT - Journal of plastic, reconstructive & aesthetic surgery : JPRAS JID - 101264239 RN - 9007-34-5 (Collagen) SB - IM MH - Humans MH - Adolescent MH - Adult MH - Middle Aged MH - Aged MH - *Free Tissue Flaps/blood supply MH - Treatment Outcome MH - *Plastic Surgery Procedures/adverse effects MH - Retrospective Studies MH - Postoperative Complications/etiology MH - *Thrombosis/etiology MH - *Connective Tissue Diseases/complications MH - Collagen MH - *Arthritis, Rheumatoid/surgery MH - Survival Analysis OTO - NOTNLM OT - Collagen vascular disorder OT - Flap OT - Microsurgery OT - Reconstruction COIS- Declaration of Competing Interest None declared. EDAT- 2022/10/24 06:00 MHDA- 2022/11/18 06:00 CRDT- 2022/10/23 22:07 PHST- 2022/01/12 00:00 [received] PHST- 2022/07/17 00:00 [revised] PHST- 2022/08/16 00:00 [accepted] PHST- 2022/10/24 06:00 [pubmed] PHST- 2022/11/18 06:00 [medline] PHST- 2022/10/23 22:07 [entrez] AID - S1748-6815(22)00493-4 [pii] AID - 10.1016/j.bjps.2022.08.052 [doi] PST - ppublish SO - J Plast Reconstr Aesthet Surg. 2022 Dec;75(12):4371-4378. doi: 10.1016/j.bjps.2022.08.052. Epub 2022 Aug 24. PMID- 32632623 OWN - NLM STAT- MEDLINE DCOM- 20210106 LR - 20210530 IS - 1432-5241 (Electronic) IS - 0364-216X (Linking) VI - 44 IP - 5 DP - 2020 Oct TI - Autologous Fat Grafting for the Oral and Digital Complications of Systemic Sclerosis: Results of a Prospective Study. PG - 1820-1832 LID - 10.1007/s00266-020-01848-2 [doi] AB - BACKGROUND: Systemic sclerosis is a connective tissue disease. Skin involvement of the mouth and hand may compromise function and quality of life. Autologous fat grafting has been described as a specific treatment of these clinical features. We report the results of our prospective study designed to treat and prevent skin complications in systemic sclerosis. MATERIALS AND METHODS: We treated 25 patients with mouth and/or hand involvement (microstomia, xerostomia, skin sclerosis, Raynaud's phenomenon and long-lasting digital ulcers) with autologous fat grafting, according to the Coleman's technique, around the mouth and/or at the base of each finger. The surgical procedures were repeated in each patient every 6 months for a total of two or three times. Clinical data were collected before the first surgery and again 6 months after each surgical procedure. Pain, skin thickness, saliva production and disability were assessed with validated tests. RESULTS: Overall we performed 63 autologous fat grafting sessions (either on the mouth, on the hands or on both anatomical areas). Results at 6 moths after the last session included improvement of xerostomia evaluated with a sialogram, reduction of the skin tension around the mouth and, in the hands, reduction of the Raynaud phenomenon as well as skin thickness. Pain was reduced while the perception of disability improved. Digital ulcers healed completely in 8/9 patients. CONCLUSIONS: Our results confirm the efficacy and safety of autologous fat grafting for the treatment of skin complications and digital ulcers due to systemic sclerosis. In addition, the patients' subjective well-being improved. Level of evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 . FAU - Pignatti, Marco AU - Pignatti M AUID- ORCID: 0000-0002-8259-496X AD - Plastic Surgery, Policlinico di Sant'Orsola, DIMES, University of Bologna, Bologna, Italy. mrpignatti@gmail.com. FAU - Spinella, Amelia AU - Spinella A AUID- ORCID: 0000-0003-4941-8507 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Cocchiara, Emanuele AU - Cocchiara E AUID- ORCID: 0000-0003-0409-7236 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Boscaini, Giulia AU - Boscaini G AD - Plastic Surgery, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Lusetti, Irene Laura AU - Lusetti IL AD - Plastic Surgery, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Citriniti, Giorgia AU - Citriniti G AUID- ORCID: 0000-0003-1990-2891 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Lumetti, Federica AU - Lumetti F AUID- ORCID: 0000-0003-4671-7982 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Setti, Giacomo AU - Setti G AUID- ORCID: 0000-0002-2933-8174 AD - Unit of Dentistry and Oral-Maxillofacial Surgery, University of Modena and Reggio Emilia, Modena, Italy. FAU - Dominici, Massimo AU - Dominici M AD - Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - De Santis, Giorgio AU - De Santis G AD - Plastic Surgery, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AUID- ORCID: 0000-0002-0041-3695 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy. LA - eng PT - Journal Article DEP - 20200706 PL - United States TA - Aesthetic Plast Surg JT - Aesthetic plastic surgery JID - 7701756 SB - IM CIN - Aesthetic Plast Surg. 2021 Jun;45(3):1344-1345. doi: 10.1007/s00266-020-02024-2. PMID: 33145615 CIN - Aesthetic Plast Surg. 2021 Jun;45(3):1342-1343. doi: 10.1007/s00266-020-02014-4. PMID: 33403420 MH - Adipose Tissue MH - Humans MH - Prospective Studies MH - *Quality of Life MH - *Scleroderma, Systemic/complications/surgery MH - Transplantation, Autologous MH - Treatment Outcome OTO - NOTNLM OT - Autologous fat grafting OT - Digital ulcers OT - Microstomia OT - Stem cell transplantation OT - Systemic sclerosis OT - Xerostomia EDAT- 2020/07/08 06:00 MHDA- 2021/01/07 06:00 CRDT- 2020/07/08 06:00 PHST- 2020/03/31 00:00 [received] PHST- 2020/06/14 00:00 [accepted] PHST- 2020/07/08 06:00 [pubmed] PHST- 2021/01/07 06:00 [medline] PHST- 2020/07/08 06:00 [entrez] AID - 10.1007/s00266-020-01848-2 [pii] AID - 10.1007/s00266-020-01848-2 [doi] PST - ppublish SO - Aesthetic Plast Surg. 2020 Oct;44(5):1820-1832. doi: 10.1007/s00266-020-01848-2. Epub 2020 Jul 6. PMID- 37916477 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240117 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 6 IP - 1 DP - 2024 Jan TI - Adherence to the Mediterranean Diet in Italian Patients With Systemic Sclerosis: An Epidemiologic Survey. PG - 14-20 LID - 10.1002/acr2.11627 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) is an orphan disease that can lead to severe involvement of the gastrointestinal tract with a significant impact on patients' quality of life (QoL). The Mediterranean diet (MD) was consistently demonstrated to have beneficial effects on chronic diseases based on biological bases. We aimed to evaluate the adherence to the MD of Italian patients with SSc to preliminarily assess its association with gastrointestinal symptoms and other disease features, mood, and QoL. METHODS: In this cross-sectional study, adherence to the MD was measured in 387 patients from four SSc Italian referral centers through the 14-item Mediterranean Diet Adherence Screener (14-MEDAS) questionnaire. We also registered patients' reported outcomes related to the QoL and mood. RESULTS: Overall, an optimal adherence to MD was observed in 14.7% of patients with SSc, a moderate adherence in 71.3%, and a low adherence in 14.0%. In univariate analysis, poor adherence to the MD was associated with a more prominent depressive mood, time missed at work, and perception of more severe Raynaud's phenomenon and digital ulcers, whereas the 14-MEDAS score inversely correlated with depression score and reflux. CONCLUSION: In our cohort of patients with SSc, overall adherence to MD was moderate. Patients with lower adherence to MD also reported worse outcomes related to QoL and mood. Administration of the 14-MEDAS could be a reasonable choice to assess adherence to the MD in patients with SSc. Future initiatives to study the role of MD in the management of patients with SSc are warranted. CI - © 2023 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Natalello, Gerlando AU - Natalello G AUID- ORCID: 0000-0001-7333-371X AD - Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AUID- ORCID: 0000-0001-6806-3794 AD - IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milan, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - San Carlo Hospital, Potenza, Italy, and University of Leeds and NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - De Santis, Maria AU - De Santis M AD - IRCCS-Humanitas Clinical and Research Center and Humanitas University, Milan, Italy. FAU - Ferlito, Arianna AU - Ferlito A AD - IRCCS San Raffaele Hospital, Milan, Italy. FAU - Karadağ, Duygu Temiz AU - Karadağ DT AUID- ORCID: 0000-0002-5891-2032 AD - San Carlo Hospital, Potenza, Italy, and Canakkale State Hospital, Canakkale, Turkey. FAU - Padula, Angela Anna AU - Padula AA AD - San Carlo Hospital, Potenza, Italy. FAU - Cavalli, Giulio AU - Cavalli G AD - IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milan, Italy. FAU - D'Agostino, Maria Antonietta AU - D'Agostino MA AD - Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. FAU - Selmi, Carlo AU - Selmi C AUID- ORCID: 0000-0002-0323-0376 AD - IRCCS-Humanitas Clinical and Research Center and Humanitas University, Milan, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Vita-Salute San Raffaele University, Milan, Italy, and University of Florence and AOUC, Florence, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milan, Italy. FAU - De Luca, Giacomo AU - De Luca G AUID- ORCID: 0000-0002-5306-7714 AD - IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milan, Italy. LA - eng GR - 104/2018, 272/2018/Gruppo Italiano Lotta alla Sclerodermia (GILS)/ PT - Journal Article DEP - 20231102 PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC10789299 EDAT- 2023/11/02 06:42 MHDA- 2023/11/02 06:43 PMCR- 2023/11/02 CRDT- 2023/11/02 05:53 PHST- 2023/09/11 00:00 [revised] PHST- 2023/03/01 00:00 [received] PHST- 2023/10/09 00:00 [accepted] PHST- 2023/11/02 06:43 [medline] PHST- 2023/11/02 06:42 [pubmed] PHST- 2023/11/02 05:53 [entrez] PHST- 2023/11/02 00:00 [pmc-release] AID - ACR211627 [pii] AID - 10.1002/acr2.11627 [doi] PST - ppublish SO - ACR Open Rheumatol. 2024 Jan;6(1):14-20. doi: 10.1002/acr2.11627. Epub 2023 Nov 2. PMID- 19565553 OWN - NLM STAT- MEDLINE DCOM- 20090917 LR - 20250529 IS - 0004-3591 (Print) IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 61 IP - 7 DP - 2009 Jul 15 TI - Anti-U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. PG - 958-65 LID - 10.1002/art.24586 [doi] AB - OBJECTIVE: To characterize a new serum autoantibody in patients with systemic sclerosis (SSc) directed against U11/U12 RNP and to identify the clinical features associated with this autoantibody. METHODS: We identified autoantibodies directed against the U11/U12 RNP complex in sera of patients with SSc and confirmed antibody specificity by immunoprecipitation, reverse transcriptase-polymerase chain reaction, and Southern blotting. We determined the prevalence of these antibodies in SSc and their specificity for SSc. We compared anti-U11/U12 RNP autoantibody-positive and negative SSc patients on demographic, disease classification, clinical variables, and survival. RESULTS: We identified 33 patients with anti-U11/U12 RNP antibodies. In 2 consecutive series of SSc patients first seen at 10-year intervals (1994-1995 and 2004-2005), the prevalence of anti-U11/U12 RNP antibody-positive patients was 15 of 462 (3.2%). Seventeen (52%) of these 33 patients had limited cutaneous involvement. All patients had Raynaud's phenomenon and 82% had gastrointestinal (GI) involvement. None had "intrinsic" pulmonary arterial hypertension. The most significant clinical difference between anti-U11/U12 antibody-positive and negative cohorts was the prevalence of lung fibrosis, which occurred in 79% of the anti-U11/U12 RNP antibody-positive patients versus 37% of the anti-U11/U12 RNP antibody-negative patients (P < 0.0001). GI involvement was also significantly increased in the anti-U11/U12 RNP antibody-positive group. Patients with anti-U11/U12 RNP antibodies and pulmonary fibrosis had a 2.25-fold greater risk of death than anti-U11/U12 RNP negative patients with pulmonary fibrosis. CONCLUSION: Anti-U11/U12 RNP antibodies are present in the sera of approximately 3% of patients with SSc and are a marker for lung fibrosis, which is often severe. FAU - Fertig, Noreen AU - Fertig N AD - University of Pittsburgh, Pittsburgh, PA 15213, USA. FAU - Domsic, Robyn T AU - Domsic RT FAU - Rodriguez-Reyna, Tatiana AU - Rodriguez-Reyna T FAU - Kuwana, Masataka AU - Kuwana M FAU - Lucas, Mary AU - Lucas M FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Feghali-Bostwick, Carol A AU - Feghali-Bostwick CA LA - eng GR - R01 AR050840/AR/NIAMS NIH HHS/United States GR - AR-050840/AR/NIAMS NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Ribonucleoproteins, Small Nuclear) RN - 0 (U11-U12 small nuclear ribonucleoprotein) SB - IM MH - Adult MH - Antibody Specificity/immunology MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Cohort Studies MH - Disease Progression MH - Female MH - Humans MH - Immunoprecipitation MH - Male MH - Middle Aged MH - Pulmonary Fibrosis/*blood/diagnosis/mortality MH - Ribonucleoproteins, Small Nuclear/*immunology MH - Scleroderma, Systemic/*blood/complications/*immunology MH - Survival Rate PMC - PMC2739404 MID - NIHMS117355 EDAT- 2009/07/01 09:00 MHDA- 2009/09/18 06:00 PMCR- 2010/07/15 CRDT- 2009/07/01 09:00 PHST- 2009/07/01 09:00 [entrez] PHST- 2009/07/01 09:00 [pubmed] PHST- 2009/09/18 06:00 [medline] PHST- 2010/07/15 00:00 [pmc-release] AID - 10.1002/art.24586 [doi] PST - ppublish SO - Arthritis Rheum. 2009 Jul 15;61(7):958-65. doi: 10.1002/art.24586. PMID- 35319814 OWN - NLM STAT- MEDLINE DCOM- 20220518 LR - 20220518 IS - 1440-1827 (Electronic) IS - 1320-5463 (Linking) VI - 72 IP - 5 DP - 2022 May TI - Antinuclear antibody pattern and autoantibody profiling of systemic sclerosis patients in a tertiary referral center in North India. PG - 283-292 LID - 10.1111/pin.13221 [doi] AB - Antinuclear antibody (ANA) pattern and autoantibody (autoAb) profiling of 150 adult systemic sclerosis (SSc) patients concerning their clinical association and diagnostic significance were analyzed by indirect immunofluorescence (IIF), immunoblot, and fluorescence enzyme immunoassay. One hundred and forty-three (95.3%) patients had positive ANA: DNA topoisomerase I (topo I)-like pattern-84(56%); speckled pattern-44(29.3%);centromere pattern-7(4.6%); and nucleolar pattern-4(2.6%). Three distinct topo I-like immunofluorescence patterns were detected at 1:40 dilution. Topo I-like pattern (32/75-limited cutaneous systemic sclerosis (lcSSc) vs. 52/75-diffuse cutaneous systemic sclerosis (dcSSc); p < 0.001) was found to be associated with dcSSc subset and speckled pattern (lcSSc 28/75 vs. dcSSc 16/75; p < 0.03) with lcSSc subset. One hundred and thirty-eight (92%) patients were positive for SSc-associated autoAbs. The frequency distribution of autoAbs to topo I, centromere A (CENP A) and centromereB (CENP B), RNA polymerase III (RP11, RP155), fibrillarin (U3RNP), nucleolus organizer region (NOR)-90, Th/To, PM-Scl75, PM-Scl100, Ku, platelet-derived growth factor receptor (PDGFR) and Ro-52, were 87(58%), 9(6%), 8(5.3%), 6(4%), 9(6%), 0, 6(4%), 6(4%), 8(5.3%), 5(3.3%), 11(7.3%),0 and 46(30.6%), respectively. Topo I autoAb was strongly associated with dcSSc (35/75 lcSSc vs. 52/75 dcSSc; p < 0.004), Raynaud's (p < 0.003), interstitial lung disease (ILD) (p < 0.001) and pulmonary arterial hypertension (PAH) (p < 0.04). This study helps in defining SSc clinical subset, prognostic markers of disease severity, characterization of the topo I-like ANA pattern, and provides a definite association between the ANA patterns and corresponding autoAb. CI - © 2022 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd. FAU - Machhua, Sanghamitra AU - Machhua S AD - Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Sharma, Shefali K AU - Sharma SK AD - Department of Internal Medicine, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Kumar, Yashwant AU - Kumar Y AD - Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Anand, Shashi AU - Anand S AD - Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Handa, Sanjeev AU - Handa S AD - Department of Dermatology Venereology Leprology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. FAU - Minz, Ranjana W AU - Minz RW AUID- ORCID: 0000-0003-1304-542X AD - Department of Immunopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. LA - eng GR - 3/2/2/22/2018/Online Onco Fship/NCD-III/Indian Council of Medical Research/ PT - Journal Article DEP - 20220323 PL - Australia TA - Pathol Int JT - Pathology international JID - 9431380 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Adult MH - *Antibodies, Antinuclear MH - Autoantibodies MH - Humans MH - RNA Polymerase III MH - *Scleroderma, Systemic/complications/diagnosis MH - Tertiary Care Centers OTO - NOTNLM OT - ANA OT - SSc OT - topo I autoantibody OT - topo I-like pattern EDAT- 2022/03/24 06:00 MHDA- 2022/05/19 06:00 CRDT- 2022/03/23 12:16 PHST- 2021/03/09 00:00 [received] PHST- 2022/02/21 00:00 [accepted] PHST- 2022/03/24 06:00 [pubmed] PHST- 2022/05/19 06:00 [medline] PHST- 2022/03/23 12:16 [entrez] AID - 10.1111/pin.13221 [doi] PST - ppublish SO - Pathol Int. 2022 May;72(5):283-292. doi: 10.1111/pin.13221. Epub 2022 Mar 23. PMID- 29409385 OWN - NLM STAT- MEDLINE DCOM- 20180717 LR - 20180717 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 47 IP - 4 DP - 2018 Jul TI - Evidence for increase in finger blood flow, evaluated by laser Doppler flowmetry, following iloprost infusion in patients with systemic sclerosis: a week-long observational longitudinal study. PG - 311-318 LID - 10.1080/03009742.2017.1397187 [doi] AB - OBJECTIVES: Iloprost plays an important role in the treatment of Raynaud's phenomenon (RP), but has transient vasodilatory effects owing to its very short half-time. We aimed to evaluate short- and medium-term haemodynamic effects of iloprost by measuring dorsal finger microvessel blood flow using laser Doppler flowmetry (LDF), in patients with RP associated with systemic sclerosis (SSc). METHOD: In 24 consecutive SSc patients with RP (disease duration 10.5 ± 1.3 years), LDF with heating probes was used to measure blood flow in four fingers by occlusive and heating tests, at baseline, after 3 consecutive days of iloprost infusion, and at 24 h and 7 days after last iloprost infusion. Nailfold videocapillaroscopy (NVC) patterns of microvascular damage were investigated. Sixteen healthy controls were studied to compare baseline flows. RESULTS: Compared to controls, SSc patients showed significantly impaired axon reflex vasoregulation and nitric oxide responses at baseline (p = 0.001 and p = 0.03, respectively). After iloprost, a prompt but transient significant improvement in endothelial-dependent vasodilation (occlusive test) was seen only in SSc patients with an 'active' NVC pattern (p ≤ 0.05). The iloprost effects vanished within 7 days after the last infusion. No significant differences were found, in the whole study, between patients with and without digital ulcers. CONCLUSIONS: Microcirculatory blood flow increases following 3 days of iloprost infusion but fades shortly after treatment. Although iloprost is effective in reducing the severity of RP in SSc, the most suitable regimen and timing to obtain longer lasting vasodilatory benefits remain to be established. FAU - Rotondo, C AU - Rotondo C AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. FAU - Nivuori, M AU - Nivuori M AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. FAU - Chialà, A AU - Chialà A AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. FAU - Praino, E AU - Praino E AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. AD - b Department of Experimental and Clinical Medicine , University of Florence , Florence , Italy. AD - c Department of Geriatric Medicine, Division of Rheumatology AOUC , University of Florence , Florence , Italy. FAU - Matucci Cerinic, M AU - Matucci Cerinic M AD - b Department of Experimental and Clinical Medicine , University of Florence , Florence , Italy. AD - c Department of Geriatric Medicine, Division of Rheumatology AOUC , University of Florence , Florence , Italy. FAU - Cutolo, M AU - Cutolo M AD - d Research Laboratory and Division of Clinical Rheumatology, Department of Emergency and Transplantation , University of Genoa, IRCCS AOU San Martino , Genoa , Italy. FAU - Lapadula, G AU - Lapadula G AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. FAU - Iannone, F AU - Iannone F AD - a Rheumatology Unit, Department of Emergency and Transplantation , University of Bari , Bari , Italy. LA - eng PT - Journal Article PT - Observational Study DEP - 20180207 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Vasodilator Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Case-Control Studies MH - Female MH - Fingers/*blood supply/diagnostic imaging MH - Humans MH - Iloprost/*pharmacology/therapeutic use MH - Infusions, Intravenous MH - Laser-Doppler Flowmetry MH - Longitudinal Studies MH - Male MH - Microcirculation/*drug effects MH - Microscopic Angioscopy MH - Middle Aged MH - Regional Blood Flow/*drug effects MH - Scleroderma, Systemic/*drug therapy/physiopathology MH - Vasodilation/*drug effects MH - Vasodilator Agents/*pharmacology/therapeutic use EDAT- 2018/02/08 06:00 MHDA- 2018/07/18 06:00 CRDT- 2018/02/08 06:00 PHST- 2018/02/08 06:00 [pubmed] PHST- 2018/07/18 06:00 [medline] PHST- 2018/02/08 06:00 [entrez] AID - 10.1080/03009742.2017.1397187 [doi] PST - ppublish SO - Scand J Rheumatol. 2018 Jul;47(4):311-318. doi: 10.1080/03009742.2017.1397187. Epub 2018 Feb 7. PMID- 16869018 OWN - NLM STAT- MEDLINE DCOM- 20060919 LR - 20111117 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 54 IP - 8 DP - 2006 Aug TI - Influence of CTLA4 haplotypes on susceptibility and some extraglandular manifestations in primary Sjögren's syndrome. PG - 2434-40 AB - OBJECTIVE: Cytotoxic T lymphocyte-associated antigen 4 (CTLA-4) is a key negative regulator of the T cell immune response, and the CTLA4 gene is highly polymorphic. Many positive associations between CTLA4 single-nucleotide polymorphisms (SNPs) and various autoimmune diseases have been identified. Two CTLA4 SNPs that are important relative to genetic susceptibility in human autoimmune diseases are the +49GA polymorphism in exon 1 and the CT60A/G polymorphism in the 3'-untranslated region. Using these 2 polymorphisms as markers, we investigated possible genetic associations of CTLA4 in Australian patients with primary Sjögren's syndrome. METHODS: One hundred eleven Australian Caucasian patients with primary SS and 156 population-based controls were genotyped for CTLA4 by polymerase chain reaction-restriction fragment length polymorphism methods, using the restriction enzymes BseXI (+49G/A) and HpyCh4 IV (CT60). RESULTS: The CT60 and +49G/A SNPs were in strong linkage disequilibrium, and only 3 haplotypes were observed. Significant differences in the haplotype frequencies between patients with primary SS and controls (P = 0.032) were observed, with susceptibility to primary SS associated with both the +49A;CT60A haplotype and the +49A;CT60G haplotype, whereas the +49G;CT60G haplotype was protective against primary SS. The +49A;CT60G haplotype association was predominantly with Ro/La autoantibody-positive primary SS, and the dose of this haplotype influenced the severity of daytime sleepiness (P = 0.036). The +49A;CT60A haplotype appeared to be protective against the development of Raynaud's phenomenon in patients with primary SS (odds ratio 0.49, 95% confidence interval 0.27-0.91). CONCLUSION: The CTLA4 +49G/A and CT60 haplotypes are associated with susceptibility to primary SS and with some extraglandular manifestations of the disease. FAU - Downie-Doyle, Sarah AU - Downie-Doyle S AD - Hanson Institute and The Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - Bayat, Noushin AU - Bayat N FAU - Rischmueller, Maureen AU - Rischmueller M FAU - Lester, Susan AU - Lester S LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antigens, CD) RN - 0 (Antigens, Differentiation) RN - 0 (CTLA-4 Antigen) RN - 0 (CTLA4 protein, human) SB - IM MH - Adult MH - Antigens, CD MH - Antigens, Differentiation/*genetics/immunology MH - CTLA-4 Antigen MH - DNA Fingerprinting MH - Disorders of Excessive Somnolence/*etiology MH - Female MH - *Genetic Predisposition to Disease MH - Haplotypes MH - Humans MH - Male MH - Middle Aged MH - Polymorphism, Restriction Fragment Length MH - Polymorphism, Single Nucleotide MH - Restriction Mapping MH - *Sjogren's Syndrome/complications/genetics/immunology MH - Urologic Diseases/*etiology EDAT- 2006/07/27 09:00 MHDA- 2006/09/20 09:00 CRDT- 2006/07/27 09:00 PHST- 2006/07/27 09:00 [pubmed] PHST- 2006/09/20 09:00 [medline] PHST- 2006/07/27 09:00 [entrez] AID - 10.1002/art.22004 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Aug;54(8):2434-40. doi: 10.1002/art.22004. PMID- 35164787 OWN - NLM STAT- MEDLINE DCOM- 20220222 LR - 20220222 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Feb 14 TI - Primary antiphospholipid syndrome in pediatrics: beyond thrombosis. Report of 32 cases and review of the evidence. PG - 13 LID - 10.1186/s12969-022-00673-y [doi] LID - 13 AB - OBJECTIVE: Describe the frequency of thrombotic and non-thrombotic clinical manifestations, laboratory, treatment and prognosis in patients with pediatric primary antiphospholipid syndrome. MATERIAL AND METHODS: A retrospective study was carried out in patients with a diagnosis of primary antiphospholipid antibody syndrome, under 16 years of age, under follow-up by the pediatric rheumatology service of the General Hospital, National Medical Center, La Raza, from January 2013 to December 2020. The antiphospholipid syndrome was defined when it met the laboratory criteria of the Sidney criteria and the presence of thrombosis or non-criteria manifestations of the disease (hematological, neurological, cutaneous, renal, cardiac or pulmonary). Demographic, clinical, laboratory, treatment, and prognosis data were collected. RESULTS: We report 32 patients, 21 female (65%) and 11 male (35%), mean age 11.75 years, evolution time 16 weeks. Thrombosis 9 patients (28%), 1 arterial and 8 venous. Non-thrombotic manifestations; Hematologic: thrombocytopenia 22 patients (69%), autoimmune hemolytic anemia 13 (40%), Fisher-Evans syndrome 6 (19%), lupus anticoagulant with hypoprothrombinemia syndrome 2 (6%). Dermatological: livedo reticularis 20 (62%), skin ulcers 2 (6%), Raynaud's phenomenon 8 (25%). Neurological: epilepsy 1 (3%), migraine 3 (9%), chorea 1 (3%) and cognitive impairment 3 (9%). Renal in 4 (13%). Laboratory: prolonged aPTT 30 (93%), lupus anticoagulant 32 (100%), positive IgG anticardiolipin 20 (62%), positive IgM anticardiolipin 19 (60%). AntiB2GPI was performed in only 3 patients, being positive in all. TREATMENT: anticoagulation in patients with thrombosis, antiplatelet in 23 (72%), steroid 30 (94%), immunosuppressant 30 (94%) and rituximab 4 (12.5%). No deaths were reported. CONCLUSIONS: The clinical characteristics of patients with pediatric primary antiphospholipid syndrome differ from those presented in adults, since non-thrombotic manifestations are more frequent in children, for which classification criteria that include these manifestations are necessary for a better characterization of the disease in pediatric population. CI - © 2022. The Author(s). FAU - Torres-Jimenez, Alfonso-Ragnar AU - Torres-Jimenez AR AUID- ORCID: 0000-0003-1070-5875 AD - Department of Pediatric Rheumatology, National Medical Center La Raza, IMSS, Vallejo y Jacarandas, colonia La Raza, Azcapotzalco, D.F. México CP, 02990, México City, México. tojadr@gmail.com. FAU - Ramirez-Nova, Virginia AU - Ramirez-Nova V AD - Department of Pediatric Rheumatology, National Medical Center La Raza, IMSS, Vallejo y Jacarandas, colonia La Raza, Azcapotzalco, D.F. México CP, 02990, México City, México. FAU - Cespedes-Cruz, Adriana Ivonne AU - Cespedes-Cruz AI AD - Department of Pediatric Rheumatology, National Medical Center La Raza, IMSS, Vallejo y Jacarandas, colonia La Raza, Azcapotzalco, D.F. México CP, 02990, México City, México. FAU - Sanchez-Jara, Berenice AU - Sanchez-Jara B AD - Department of Pediatric Hematology, National Medical Center La Raza, IMSS, Mexico City, México. FAU - Velazquez-Cruz, Alejandra AU - Velazquez-Cruz A AD - Department of Pediatric Rheumatology, National Medical Center La Raza, IMSS, Vallejo y Jacarandas, colonia La Raza, Azcapotzalco, D.F. México CP, 02990, México City, México. FAU - Bekker-Méndez, Vilma Carolina AU - Bekker-Méndez VC AD - Research Unit in Immunology and Infectology, National Medical Center La Raza, IMSS, Mexico City, México. FAU - Guerra-Castillo, Francisco Xavier AU - Guerra-Castillo FX AD - Research Unit in Immunology and Infectology, National Medical Center La Raza, IMSS, Mexico City, México. LA - eng PT - Journal Article PT - Review DEP - 20220214 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM MH - Adolescent MH - Antiphospholipid Syndrome/*complications/*pathology/therapy MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Infant MH - Male MH - Retrospective Studies PMC - PMC8842521 COIS- The authors declare that they have no competing interests. EDAT- 2022/02/16 06:00 MHDA- 2022/02/23 06:00 PMCR- 2022/02/14 CRDT- 2022/02/15 05:31 PHST- 2021/10/21 00:00 [received] PHST- 2022/01/30 00:00 [accepted] PHST- 2022/02/15 05:31 [entrez] PHST- 2022/02/16 06:00 [pubmed] PHST- 2022/02/23 06:00 [medline] PHST- 2022/02/14 00:00 [pmc-release] AID - 10.1186/s12969-022-00673-y [pii] AID - 673 [pii] AID - 10.1186/s12969-022-00673-y [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Feb 14;20(1):13. doi: 10.1186/s12969-022-00673-y. PMID- 30997150 OWN - NLM STAT- MEDLINE DCOM- 20200420 LR - 20260304 IS - 2056-5933 (Print) IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 5 IP - 1 DP - 2019 TI - Setting the international standard for longitudinal follow-up of patients with systemic sclerosis: a Delphi-based expert consensus on core clinical features. PG - e000826 LID - 10.1136/rmdopen-2018-000826 [doi] LID - e000826 AB - BACKGROUND: Systemic sclerosis (SSc) is a severe, progressive multiorgan disease but to date, there are no established standardised international guidelines for follow-up of patients with SSc. The goal of this project was to develop an expert consensus for annual systematic investigations in patients with SSc to enhance their standard-of-care. MATERIAL AND METHODS: The Delphi method was applied. All SSc experts from the European Scleroderma Trials and Research group network and the Scleroderma Clinical Trial Consortium were invited to participate. All experts were asked to answer questionnaires in five Delphi steps to determine the domains of interest and tools for each domain for an annual systematic assessment of patients with SSc. Each item was rated on a scale between 0% and 100% (not and very important), and parameters rated >80% by more than 75% of the experts were regarded as acceptable. RESULTS: In total, 157 experts worldwide participated with 71.3% experts seeing >50 patients with SSc annually. In the first round, 23 domains and 204 tools were suggested. After five Delphi steps, experts agreed on 10 domains including (1) Raynaud's phenomenon; (2) Digital ulcers; (3) Skin and mucosa; (4) Lung; (5); Heart; (6) GI domain, (7) Renal; (8) Musculoskeletal; (9) Laboratory and (10) Treatment. Overall, 55 tools were identified including clinical assessments, laboratory measurements and imaging or functional investigations. CONCLUSION: Through five Delphi steps with world leading experts, a consensus was established on strongly suggested tools for a minimum annual systemic assessment of organ involvement in SSc. This work should enhance the standardisation and homogenisation of the practices. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University of Zurich, Zurich, Switzerland. FAU - Murray, Baron AU - Murray B AD - Rheumatology, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Khanna, Dinesh AU - Khanna D AD - Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A Department, Universite Paris Descartes, Paris, France. AD - INSERM U1016, Immunology, Cochin Institute, Paris, France. CN - EUSTAR and SCTC collaborators LA - eng PT - Consensus Statement PT - Journal Article DEP - 20190304 PL - England TA - RMD Open JT - RMD open JID - 101662038 MH - Delphi Technique MH - Disease Management MH - *Expert Testimony MH - Follow-Up Studies MH - Humans MH - Qualitative Research MH - Scleroderma, Systemic/*diagnosis/therapy MH - *Standard of Care PMC - PMC6446182 OTO - NOTNLM OT - autoimmune diseases OT - multidisciplinary team-care OT - qualitative research OT - systemic sclerosis COIS- Competing interests: A-MH-V has obtained research support from Boehringer Ingelheim. She is a scientific consultant for Actelion and Boehringer Ingelheim in the field of systemic sclerosis and related diseases. She has received travel expenses from GSK, Actelion and Boehringer Ingelheim. OD has obtained research support from Bayer, Sanofi, Ergonex, Boehringer Ingelheim, Actelion and Pfizer. He is a scientific consultant for 4 D Science, Actelion, Active Biotec, Bayer, BiogenIdec, BMS, Boehringer Ingelheim, ChemoAb, EpiPharm, Ergonex, espeRare foundation, Genentech/Roche, GSK, Inventiva, Lilly, medac, MedImmune, Pharmacyclics, Pfizer, Serodapharm and Sinoxa in the field of systemic sclerosis and related diseases and has a patent licensed on mir-29 for the treatment of systemic sclerosis. OK-B received consultancies, honoraria and/or speaker fees from Abbvie, Actelion, Bayer, Roche. DK has received consulting fees from Actelion, Bayer, Bristol-Myers Squibb, Cytori, CSL Behring, Corbus, Genentech/Roche, GlaxoSmithKline, Inventiva, Regeneron, Sanofi-Aventis and UCB and has stock options with Eicos Sciences, Inc. YA has/had consultancy relationship and/or has received research funding in relationship with the treatment of systemic sclerosis from Actelion, Bayer, Biogen Idec, Bristol-Myers Squibb, Genentech/ Roche, Inventiva, Medac, Pfizer, Sanofi/Genzyme, Servier and UCB. EDAT- 2019/04/19 06:00 MHDA- 2019/04/19 06:01 PMCR- 2019/03/04 CRDT- 2019/04/19 06:00 PHST- 2018/09/25 00:00 [received] PHST- 2018/11/28 00:00 [revised] PHST- 2018/12/17 00:00 [accepted] PHST- 2019/04/19 06:00 [entrez] PHST- 2019/04/19 06:00 [pubmed] PHST- 2019/04/19 06:01 [medline] PHST- 2019/03/04 00:00 [pmc-release] AID - rmdopen-2018-000826 [pii] AID - 10.1136/rmdopen-2018-000826 [doi] PST - epublish SO - RMD Open. 2019 Mar 4;5(1):e000826. doi: 10.1136/rmdopen-2018-000826. eCollection 2019. PMID- 22253028 OWN - NLM STAT- MEDLINE DCOM- 20120622 LR - 20151119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 51 IP - 5 DP - 2012 May TI - Increased serum COMP predicts mortality in SSc: results from a longitudinal study of interstitial lung disease. PG - 915-20 LID - 10.1093/rheumatology/ker442 [doi] AB - OBJECTIVES: COMP is a regulator of assembly and maintenance of the fibrillar collagen I and II networks. Serum COMP reflects skin fibrosis in SSc. The purpose of this study was to examine whether serum COMP reflects fibrotic lung involvement in SSc patients and to study if serum COMP predicts mortality. METHODS: Three overlapping cohorts of 244 SSc patients were studied. Two hundred and eighteen patients were included to study survival, 80 patients to study longitudinal changes of pulmonary function tests and 64 to study pulmonary involvement assessed by high-resolution CT (HRCT). Serum COMP was measured by ELISA. Skin involvement was assessed with the modified Rodnan skin score (mRSS). Data about survival were obtained from the central population registry. RESULTS: Serum COMP measured within 5 years after the first non-Raynaud's manifestation was a predictor of death, and crude mortality increased by 6% for each COMP unit elevation. Serum COMP levels >15 U/l were associated with a 3.13-fold (95% CI 1.73, 5.64; P < 0.001) increased risk of death. During the first year of follow-up serum COMP and vital capacity (VC) changed inversely (r(s) = -0.32; P = 0.005), but there were no correlations between baseline serum COMP and concurrent findings by spirometry or HRCT. CONCLUSION: Serum COMP early in disease is a predictor of mortality in SSc patients. Serum COMP changes in parallel with lung fibrosis as measured by VC, but the release from fibrotic skin possibly obscures the influx from the lungs and therefore serum COMP seems to have little utility as a marker of lung fibrosis. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Skåne University Hospital, S-221 85 Lund, Sweden. roger.hesselstrand@med.lu.se FAU - Andréasson, Kristofer AU - Andréasson K FAU - Wuttge, Dirk M AU - Wuttge DM FAU - Bozovic, Gracijela AU - Bozovic G FAU - Scheja, Agneta AU - Scheja A FAU - Saxne, Tore AU - Saxne T LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120116 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Cartilage Oligomeric Matrix Protein) RN - 0 (Extracellular Matrix Proteins) RN - 0 (Glycoproteins) RN - 0 (Matrilin Proteins) RN - 0 (TSP5 protein, human) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - Biomarkers/blood MH - Cartilage Oligomeric Matrix Protein MH - Cross-Sectional Studies MH - Extracellular Matrix Proteins/*blood MH - Female MH - Glycoproteins/*blood MH - Humans MH - Longitudinal Studies MH - Lung Diseases, Interstitial/blood/complications/*mortality MH - Male MH - Matrilin Proteins MH - Middle Aged MH - Predictive Value of Tests MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/blood/complications/*mortality MH - Severity of Illness Index EDAT- 2012/01/19 06:00 MHDA- 2012/06/23 06:00 CRDT- 2012/01/19 06:00 PHST- 2012/01/19 06:00 [entrez] PHST- 2012/01/19 06:00 [pubmed] PHST- 2012/06/23 06:00 [medline] AID - ker442 [pii] AID - 10.1093/rheumatology/ker442 [doi] PST - ppublish SO - Rheumatology (Oxford). 2012 May;51(5):915-20. doi: 10.1093/rheumatology/ker442. Epub 2012 Jan 16. PMID- 20506531 OWN - NLM STAT- MEDLINE DCOM- 20101026 LR - 20260518 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 62 IP - 10 DP - 2010 Oct TI - Association of pruritus with quality of life and disability in systemic sclerosis. PG - 1489-95 LID - 10.1002/acr.20257 [doi] AB - OBJECTIVE: To our knowledge, no studies have investigated the association of pruritus, which is present in almost half of patients with systemic sclerosis (SSc; scleroderma), with quality of life (QOL) and disability. The objective of this study was to investigate the association of pruritus with QOL and disability in SSc. METHODS: We performed a cross-sectional, multicenter study of 578 SSc patients ≥1 year post-enrollment in the Canadian Scleroderma Research Group Registry. Patients reported whether they experienced pruritus during the past month on most days and underwent clinical histories and medical examinations. QOL was measured using the mental and physical component summary scores of the Short Form 36, and disability was measured with the Health Assessment Questionnaire disability index. The association of pruritus with QOL and disability was estimated using linear regression, controlling for sociodemographic and disease variables. RESULTS: A total of 248 patients (43%) reported pruritus on most days. Patients with pruritus had significantly worse mental (Hedges's g = -0.43; 95% confidence interval [95% CI] -0.59, -0.26) and physical function (Hedges's g = -0.51; 95% CI -0.68, -0.34) and greater disability (Hedges's g = 0.46; 95% CI 0.29, 0.63) than patients without pruritus. In multivariate analyses, controlling for age, sex, marital status, education, disease duration, skin score, number of tender joints, gastrointestinal symptoms, breathing problems, Raynaud's phenomenon, and finger ulcers, pruritus was independently associated with mental (P = 0.017) and physical function (P = 0.003), but not disability (P = 0.112). CONCLUSION: Pruritus is common and associated with QOL in SSc. More attention to pruritus in SSc is needed, including its measurement, etiology, trajectory, and potential methods for intervention. FAU - El-Baalbaki, Ghassan AU - El-Baalbaki G AD - McGill University and Jewish General Hospital, Montreal, Quebec, Canada. FAU - Razykov, Ilya AU - Razykov I FAU - Hudson, Marie AU - Hudson M FAU - Bassel, Marielle AU - Bassel M FAU - Baron, Murray AU - Baron M FAU - Thombs, Brett D AU - Thombs BD CN - Canadian Scleroderma Research Group LA - eng GR - Canadian Institutes of Health Research/Canada PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - *Disability Evaluation MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pruritus/complications/diagnosis/*psychology MH - Quality of Life/*psychology MH - Registries/statistics & numerical data MH - Reproducibility of Results MH - Scleroderma, Systemic/complications/diagnosis/*psychology FIR - Baron, M IR - Baron M FIR - Mathieu, J-P IR - Mathieu JP FIR - Hudson, M IR - Hudson M FIR - Ligier, S IR - Ligier S FIR - Grodzicky, T IR - Grodzicky T FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Masetto, A IR - Masetto A FIR - Sutton, E IR - Sutton E FIR - Khalidi, N A IR - Khalidi NA FIR - Kaminska, E IR - Kaminska E FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Docherty, P IR - Docherty P FIR - Smith, C D IR - Smith CD FIR - Mittoo, S IR - Mittoo S FIR - Fritzler, M IR - Fritzler M EDAT- 2010/05/28 06:00 MHDA- 2010/10/27 06:00 CRDT- 2010/05/28 06:00 PHST- 2010/05/28 06:00 [entrez] PHST- 2010/05/28 06:00 [pubmed] PHST- 2010/10/27 06:00 [medline] AID - 10.1002/acr.20257 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2010 Oct;62(10):1489-95. doi: 10.1002/acr.20257. PMID- 24314389 OWN - NLM STAT- MEDLINE DCOM- 20140801 LR - 20131209 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 32 IP - 1 DP - 2014 Jan-Feb TI - The red face revisited: connective tissue disorders. PG - 153-8 LID - S0738-081X(13)00104-1 [pii] LID - 10.1016/j.clindermatol.2013.05.037 [doi] AB - Red face is not a rare finding in patients with connective tissue disorders. The malar eruption is the most frequent cutaneous manifestation of systemic lupus erythematosus (LE). This condition is more apparent among fair-skinned individuals, and it usually appears after sun exposure. A very important clinical sign is that nasolabial folds remain free of any erythematous or other changes. With subacute cutaneous LE, sun exposure can provoke a red face that resembles the malar eruption of systemic LE. The typical clinical findings of chronic cutaneous LE are the discoid lesions. There is a clinical form of chronic cutaneous LE called erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. Other rare "red face" forms of chronic cutaneous LE are LE tumidus and LE telangiectaticus. Red face is not typical of systemic sclerosis, but facial telangiectasias are frequent, especially with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. The differential diagnoses of other red face manifestations are easy due to the additional findings. Telangiectasias are accompanied by calcinosis, sclerodactyly, digital ischemia, and Raynaud disease. Many studies mention telangiectasias as markers of the severity of the systemic sclerosis, the disease duration, any pulmonary arterial hypertension, and any esophageal involvement. Purple- or violet-colored upper eyelids are the hallmark and one of the first clinical signs that is helpful for the diagnosis of dermatomyositis. This violaceous to dusky erythema can extend over the whole face and the upper aspects of the trunk. Erythematous changes on the face that are different from those of the heliotrope sign which occurs with dermatomyositis may be observed in both sun-exposed skin and non-sun-exposed skin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributed eruptions can also appear. CI - © 2013 Elsevier Inc. All rights reserved. FAU - Kazandjieva, Jana AU - Kazandjieva J AD - Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria. Electronic address: janaderm@abv.bg. FAU - Tsankov, Nikolai AU - Tsankov N FAU - Pramatarov, Kyrill AU - Pramatarov K LA - eng PT - Journal Article PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 SB - IM MH - Dermatomyositis/*complications MH - Erythema/etiology MH - Facial Dermatoses/*etiology MH - Humans MH - Lupus Erythematosus, Discoid/*complications MH - Lupus Erythematosus, Systemic/*complications MH - Mixed Connective Tissue Disease/complications MH - Scleroderma, Systemic/*complications EDAT- 2013/12/10 06:00 MHDA- 2014/08/02 06:00 CRDT- 2013/12/10 06:00 PHST- 2013/12/10 06:00 [entrez] PHST- 2013/12/10 06:00 [pubmed] PHST- 2014/08/02 06:00 [medline] AID - S0738-081X(13)00104-1 [pii] AID - 10.1016/j.clindermatol.2013.05.037 [doi] PST - ppublish SO - Clin Dermatol. 2014 Jan-Feb;32(1):153-8. doi: 10.1016/j.clindermatol.2013.05.037. PMID- 40316394 OWN - NLM STAT- MEDLINE DCOM- 20250503 LR - 20250502 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 21 IP - 3 DP - 2025 Mar TI - Role of the anti-RO/SSA antibody in patients with systemic lupus erythematosus. PG - 501816 LID - S2173-5743(25)00036-X [pii] LID - 10.1016/j.reumae.2025.501816 [doi] AB - BACKGROUND: Patients with systemic lupus erythematosus (SLE) and anti-Ro+ antibody frequently pose a diagnostic and therapeutic challenge for the specialist, as they frequently present sicca syndrome, overlapping with Sjögren's syndrome (SS). To date, the clinical and prognostic variability that this antibody confers on SLE patients is not well characterized. OBJECTIVES: To investigate the possible clinical, analytical, therapeutic and prognostic implications of anti-Ro antibody in SLE. Furthermore, we analyzed the possible implications of the expressed anti-Ro profile (subunit 52, 60 or both) on the disease phenotype. METHODS: The medical records of patients with anti-Ro+ and - SLE, primary SS and SLE/SS overlap have been reviewed. RESULTS: Anti-Ro+ SLE presents less arthritis, low C4, expression of DNA Crithidia and need for bolus corticosteroids than anti-Ro- SLE, but more xerophthalmia, xerostomia, expression of anti-La, anti-cyclic citrullinated peptide and overlap with other rheumatological entities. Anti-Ro+ SLE and the overlap group behave similarly for multiple variables. SS group shows a higher expression of β2-microglobulin compared to the overlap group. Anti-Ro52+ patients associate more Raynaud's phenomenon than anti-Ro60+ patients. The latter express more lupus anticoagulant and antiphospholipid antibodies than the group with both subunits. CONCLUSIONS: The presence of anti-Ro+ in patients with SLE provides clinical and analytical differences compared to patients with anti-Ro- SLE and SLE/SS. anti-Ro+ SLE and the overlap group behave similarly, but present differential characteristics that postulate them as separate phenotypes of the disease. The different serological profiles of anti-Ro confer specific clinical and analytical characteristics in patients with SLE and SS. CI - Copyright © 2025 Sociedad Española de Reumatología (SER), Colegio Mexicano de Reumatología (CMR) and Elsevier España, S.L.U. All rights reserved. FAU - Jiménez, Paula Pérez AU - Jiménez PP AD - Faculty of Medicine, Autonomous University of Madrid, Madrid, Spain. FAU - Barrera, Laura Tío AU - Barrera LT AD - Hospital del Mar Medical Research Institute, IMIM, Barcelona, Spain. FAU - Sánchez, José Luis Andréu AU - Sánchez JLA AD - Department of Rheumatology, Hospital Universitario Puerta de Hierro de Majadahonda, Madrid, Spain. FAU - Salman-Monte, Tarek Carlos AU - Salman-Monte TC AD - Department of Rheumatology, Hospital del Mar, Barcelona, Spain; Hospital del Mar Clinical Expertise Unit in Systemic Autoimmune Diseases and Vasculitis, Spain. Electronic address: tareto4@gmail.com. FAU - Carrión-Barberà, Irene AU - Carrión-Barberà I AD - Department of Rheumatology, Hospital del Mar, Barcelona, Spain; Hospital del Mar Clinical Expertise Unit in Systemic Autoimmune Diseases and Vasculitis, Spain. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 RN - 0 (Antibodies, Antinuclear) RN - 0 (SS-A antibodies) SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/immunology/blood/diagnosis/complications MH - *Antibodies, Antinuclear/blood MH - Female MH - Male MH - Adult MH - Sjogren's Syndrome/immunology/blood/diagnosis/complications MH - Middle Aged MH - Retrospective Studies MH - Prognosis OTO - NOTNLM OT - Anti-Ro OT - Autoantibodies OT - Autoanticuerpos OT - Lupus eritematoso sistémico OT - Overlap syndrome OT - Sjögren syndrome OT - Systemic lupus erythematosus OT - Síndrome de Sjögren OT - Síndrome de superposición EDAT- 2025/05/03 00:37 MHDA- 2025/05/03 22:23 CRDT- 2025/05/02 21:00 PHST- 2024/10/22 00:00 [received] PHST- 2024/12/05 00:00 [revised] PHST- 2025/01/08 00:00 [accepted] PHST- 2025/05/03 22:23 [medline] PHST- 2025/05/03 00:37 [pubmed] PHST- 2025/05/02 21:00 [entrez] AID - S2173-5743(25)00036-X [pii] AID - 10.1016/j.reumae.2025.501816 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2025 Mar;21(3):501816. doi: 10.1016/j.reumae.2025.501816. PMID- 39907594 OWN - NLM STAT- MEDLINE DCOM- 20250228 LR - 20250609 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 43 IP - 2 DP - 2025 Feb TI - Different phenotypic manifestations between Brazilian and Japanese anti-MDA5 antibody-positive dermatomyositis: an international tricentric longitudinal study. PG - 193-201 LID - 10.55563/clinexprheumatol/9s7djz [doi] AB - OBJECTIVES: Anti-MDA5 autoantibodies are strongly associated with interstitial lung disease (ILD) and rapidly progressive ILD (RP-ILD) in Asian patients with dermatomyositis (DM) or amyopathic DM (ADM). However, this association has not yet been established in Brazilian patients with anti-MDA5(+) DM/ADM. This study aimed to investigate the phenotypic differences between Brazilian and Japanese patients with anti-MDA5(+) DM/ADM, with a particular focus on ILD. METHODS: This was an international, tricentric, retrospective cohort study conducted in one Brazilian and two Japanese tertiary centres. Patients diagnosed with anti-MDA5(+) DM/ADM at the three centres were enrolled. Clinical characteristics and outcomes were collected using a pre-standardised protocol and compared between Brazilian and Japanese patients. RESULTS: Thirty-four Brazilian and 65 Japanese patients were analysed. Brazilian patients were younger at the time of diagnosis than Japanese patients. The prevalence of muscle weakness, myalgia, dysphagia, heliotrope rash, V-sign, calcinosis, Raynaud's phenomenon, and digital ulcers was higher in Brazilian patients, whereas mechanic's hands were more prevalent in Japanese patients. The prevalence of ILD was significantly lower in Brazilian patients than in Japanese patients (50.0% vs. 98.5%, p<0.001). RP-ILD was observed in 34 (52.3%) Japanese patients and in only one (3.3%) Brazilian patient (p<0.001). Outcomes including overall survival and the frequency of relapses and complications, such as severe infection and malignancy, were comparable between the two populations. CONCLUSIONS: Brazilian patients with anti-MDA5(+) DM/ADM had a higher prevalence of skin and muscle involvement, whereas the prevalence of ILD and RP-ILD was significantly lower than in Japanese patients. FAU - Faria, Marlise S M S AU - Faria MSMS AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, Brazil. FAU - Yoshida, Akira AU - Yoshida A AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Mugii, Naoki AU - Mugii N AD - Department of Rehabilitation, Kanazawa University Hospital, Kanazawa, Japan. FAU - Inaoka, Pleiades T AU - Inaoka PT AD - Division of Rehabilitation Science, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Matsushita, Takashi AU - Matsushita T AD - Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. FAU - Gono, Takahisa AU - Gono T AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Shinjo, Samuel K AU - Shinjo SK AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, Brazil. samuel.shinjo@usp.br. LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20250205 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - EC 3.6.1.- (IFIH1 protein, human) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - Amyopathic dermatomyositis RN - Japanese people RN - Brazilian people SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Autoantibodies/blood MH - Biomarkers/blood MH - Brazil/epidemiology MH - *Dermatomyositis/immunology/diagnosis/ethnology/epidemiology/blood MH - Disease Progression MH - *Interferon-Induced Helicase, IFIH1/immunology MH - Japan/epidemiology MH - Longitudinal Studies MH - *Lung Diseases, Interstitial/immunology/ethnology/diagnosis/epidemiology MH - Phenotype MH - Prevalence MH - Retrospective Studies MH - South American People MH - East Asian People EDAT- 2025/02/05 12:24 MHDA- 2025/02/28 06:21 CRDT- 2025/02/05 10:03 PHST- 2024/08/13 00:00 [received] PHST- 2024/11/20 00:00 [accepted] PHST- 2025/02/28 06:21 [medline] PHST- 2025/02/05 12:24 [pubmed] PHST- 2025/02/05 10:03 [entrez] AID - 21544 [pii] AID - 10.55563/clinexprheumatol/9s7djz [doi] PST - ppublish SO - Clin Exp Rheumatol. 2025 Feb;43(2):193-201. doi: 10.55563/clinexprheumatol/9s7djz. Epub 2025 Feb 5. PMID- 21402907 OWN - NLM STAT- MEDLINE DCOM- 20110610 LR - 20211203 IS - 1091-6490 (Electronic) IS - 0027-8424 (Print) IS - 0027-8424 (Linking) VI - 108 IP - 13 DP - 2011 Mar 29 TI - Homozygous mutation in SAMHD1 gene causes cerebral vasculopathy and early onset stroke. PG - 5372-7 LID - 10.1073/pnas.1014265108 [doi] AB - We describe an autosomal recessive condition characterized with cerebral vasculopathy and early onset of stroke in 14 individuals in Old Order Amish. The phenotype of the condition was highly heterogeneous, ranging from severe developmental disability to normal schooling. Cerebral vasculopathy was a major hallmark of the condition with a common theme of multifocal stenoses and aneurysms in large arteries, accompanied by chronic ischemic changes, moyamoya morphology, and evidence of prior acute infarction and hemorrhage. Early signs of the disease included mild intrauterine growth restriction, infantile hypotonia, and irritability, followed by failure to thrive and short stature. Acrocyanosis, Raynaud's phenomenon, chilblain lesions, low-pitch hoarse voice, glaucoma, migraine headache, and arthritis were frequently observed. The early onset or recurrence of strokes secondary to cerebral vasculopathy seems to always be associated with poor outcomes. The elevated erythrocyte sedimentation rate (ESR), IgG, neopterin, and TNF-α found in these patients suggested an immune disorder. Through genomewide homozygosity mapping, we localized the disease gene to chromosome (Chr) 20q11.22-q12. Candidate gene sequencing identified a homozygous mutation, c.1411-2A > G, in the SAMHD1 gene, being associated with this condition. The mutation appeared at the splice-acceptor site of intron 12, resulted in the skipping of exon 13, and gave rise to an aberrant protein with in-frame deletion of 31 amino acids. Immunoblotting analysis showed lack of mutant SAMHD1 protein expression in affected cell lines. The function of SAMHD1 remains unclear, but the inflammatory vasculopathies of the brain found in the patients with SAMHD1 mutation indicate its important roles in immunoregulation and cerebral vascular hemeostasis. FAU - Xin, Baozhong AU - Xin B AD - DDC Clinic for Special Needs Children, Middlefield, OH 44062, USA. FAU - Jones, Stephen AU - Jones S FAU - Puffenberger, Erik G AU - Puffenberger EG FAU - Hinze, Claas AU - Hinze C FAU - Bright, Alicia AU - Bright A FAU - Tan, Haiyan AU - Tan H FAU - Zhou, Aimin AU - Zhou A FAU - Wu, Guiyun AU - Wu G FAU - Vargus-Adams, Jilda AU - Vargus-Adams J FAU - Agamanolis, Dimitris AU - Agamanolis D FAU - Wang, Heng AU - Wang H LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110314 PL - United States TA - Proc Natl Acad Sci U S A JT - Proceedings of the National Academy of Sciences of the United States of America JID - 7505876 RN - EC 3.1.5.- (SAM Domain and HD Domain-Containing Protein 1) RN - EC 3.1.5.- (SAMHD1 protein, human) RN - EC 3.6.5.2 (Monomeric GTP-Binding Proteins) SB - IM CIN - Proc Natl Acad Sci U S A. 2011 Jun 28;108(26):E232; author reply E233. doi: 10.1073/pnas.1104699108. PMID: 21633013 MH - Adolescent MH - Adult MH - Age of Onset MH - Base Sequence MH - *Cerebrovascular Circulation MH - Cerebrovascular Disorders/*genetics/pathology MH - Child MH - Child, Preschool MH - DNA Mutational Analysis MH - Ethnicity/genetics MH - Female MH - *Homozygote MH - Humans MH - Infant MH - Infant, Newborn MH - Male MH - Monomeric GTP-Binding Proteins/*genetics MH - *Mutation MH - Pedigree MH - SAM Domain and HD Domain-Containing Protein 1 MH - Stroke/*genetics/pathology MH - Young Adult PMC - PMC3069167 COIS- The authors declare no conflict of interest. EDAT- 2011/03/16 06:00 MHDA- 2011/06/11 06:00 PMCR- 2011/09/29 CRDT- 2011/03/16 06:00 PHST- 2011/03/16 06:00 [entrez] PHST- 2011/03/16 06:00 [pubmed] PHST- 2011/06/11 06:00 [medline] PHST- 2011/09/29 00:00 [pmc-release] AID - 1014265108 [pii] AID - 201014265 [pii] AID - 10.1073/pnas.1014265108 [doi] PST - ppublish SO - Proc Natl Acad Sci U S A. 2011 Mar 29;108(13):5372-7. doi: 10.1073/pnas.1014265108. Epub 2011 Mar 14. PMID- 34725185 OWN - NLM STAT- MEDLINE DCOM- 20220301 LR - 20220301 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 8 IP - 1 DP - 2021 Oct TI - Smoking associates with increased BAFF and decreased interferon-γ levels in patients with systemic lupus erythematosus. LID - 10.1136/lupus-2021-000537 [doi] LID - e000537 AB - OBJECTIVE: In SLE, smoking increases the burden of cutaneous disease and organ damage, and leads to premature mortality. However, the effect of smoking on disease manifestations and cytokine levels of patients with SLE is unclear. This study compared characteristics of patients with SLE across smoking status, and determined the association of smoking with serum cytokine levels. METHOD: A cross-sectional study of patients with SLE (n=99) during a research visit in which smoking status was ascertained. Smoking status was compared across classification criteria (American College of Rheumatology Classification Criteria for SLE (ACR97)), disease activity (SLE Disease Activity Index), autoantibody levels, accrued damage (Systemic Lupus International Collaborating Clinics/ACR Damage Index), and circulating concentrations of serum interferon-gamma (IFN-γ), interleukin (IL)-1β, IL-4, IL-6, IL-10, IL-12, IL-17, B cell-activating factor (BAFF), tumour necrosis factor-alpha, transforming growth factor beta 1 (TGF-β1), macrophage inflammatory protein 1 alpha (MIP-1α), MIP-1β and monocyte chemoattractant protein 1. Linear regression models determined the association between smoking and cytokine levels, adjusting for age and sex, clinical characteristics (model 1), and anti-inflammatory (IL-4, IL-10 and TGF- β1) and regulatory (IL-1β) cytokines (model 2). RESULTS: Among patients with SLE (97.9% ANA+; mean 48.48 years old; 86.9% female; mean 10 years of disease duration), 35.4% (n=35 of 99) were smoking (an average of 7 cigarettes/day for 24 years). Smokers had increased odds of prevalent ACR97 malar rash (OR 3.40, 95% CI 1.23 to 9.34) and mucosal ulcers (OR 3.31, 95% CI 1.36 to 8.05). Smokers had more arthritis (OR 3.19, 95% CI 1.19 to 8.60), migraine (OR 2.82, 95% CI 1.07 to 7.44), Raynaud's phenomenon (OR 5.15, 95% CI 1.95 to 13.56) and increased non-steroidal anti-inflammatory drug use (OR 6.88, 95% CI 1.99 to 23.72). Smoking associated with 27% increased BAFF levels (95% CI 6% to 48%) and 42% decreased IFN-γ levels (95% CI -79% to -5%) in model 2. CONCLUSION: In patients with SLE, smoking independently associated with increased BAFF and decreased IFN-γ levels, and an increased frequency of arthritis, migraine and Raynaud's phenomenon. Smoking cessation is advisable to reduce systemic inflammation, reduce disease activity and improve host defence. CI - © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Raymond, Warren David AU - Raymond WD AUID- ORCID: 0000-0002-2537-0070 AD - Rheumatology Section, Medical School, University of Western Australia Faculty of Medicine, Dentistry and Health Sciences, Crawley, Western Australia, Australia warren.raymond@uwa.edu.au. FAU - Hamdorf, Matthew AU - Hamdorf M AUID- ORCID: 0000-0003-0516-8702 AD - Rheumatology Section, Medical School, University of Western Australia Faculty of Medicine, Dentistry and Health Sciences, Crawley, Western Australia, Australia. FAU - Furfaro, Michael AU - Furfaro M AD - Rheumatology Section, Medical School, University of Western Australia Faculty of Medicine, Dentistry and Health Sciences, Crawley, Western Australia, Australia. FAU - Eilertsen, Gro Ostli AU - Eilertsen GO AD - Clinical Medicine, UiT The Arctic University of Norway, Tromso, Norway. FAU - Nossent, Johannes Cornelis AU - Nossent JC AUID- ORCID: 0000-0002-2833-7997 AD - Rheumatology Section, Medical School, University of Western Australia Faculty of Medicine, Dentistry and Health Sciences, Crawley, Western Australia, Australia. AD - Clinical Medicine, UiT The Arctic University of Norway, Tromso, Norway. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 RN - 0 (Cytokines) RN - 82115-62-6 (Interferon-gamma) SB - IM MH - Cross-Sectional Studies MH - Cytokines MH - Female MH - Humans MH - *Interferon-gamma MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - Male MH - Middle Aged MH - Smoking/adverse effects/epidemiology PMC - PMC8562512 OTO - NOTNLM OT - cytokines OT - lupus erythematosus OT - smoking OT - systemic COIS- Competing interests: None declared. EDAT- 2021/11/03 06:00 MHDA- 2022/03/03 06:00 PMCR- 2021/01/01 CRDT- 2021/11/02 05:37 PHST- 2021/07/14 00:00 [received] PHST- 2021/10/09 00:00 [accepted] PHST- 2021/11/02 05:37 [entrez] PHST- 2021/11/03 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 8/1/e000537 [pii] AID - lupus-2021-000537 [pii] AID - 10.1136/lupus-2021-000537 [doi] PST - ppublish SO - Lupus Sci Med. 2021 Oct;8(1):e000537. doi: 10.1136/lupus-2021-000537. PMID- 39562303 OWN - NLM STAT- MEDLINE DCOM- 20250409 LR - 20250414 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 35 IP - 3 DP - 2025 Apr 8 TI - Effectiveness and safety of low-energy shock wave therapy for digital ulcers associated with systemic sclerosis: A Phase 3 pivotal clinical trial. PG - 484-495 LID - 10.1093/mr/roae104 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is characterised by ischaemic skin ulcers on the fingertips, and low-energy shock wave therapy is suggested as a novel treatment for ischaemic lesions with angiogenic effects. We aimed to investigate the efficacy and safety of shock wave therapy for skin ulcers in patients with SSc. METHODS: In this Phase 3 pivotal study, we analysed 60 SSc patients with digital ulcers that did not disappear after >4 weeks of existing treatment: 30 patients were treated with extracorporeal shock wave therapy and 30 with conventional treatment. The ulcer count reduction observed after an 8-week treatment period was compared between the shock wave therapy and conventional treatment groups. RESULTS: After an 8-week treatment period, the mean reduction in the number of ulcers was 0.83 (SD 2.79) in the conventional treatment group compared to a more pronounced reduction of 4.47 (SD 2.65) in the shock wave therapy group. CONCLUSIONS: The study findings indicate the efficacy of extracorporeal shock wave therapy for refractory digital ulcers associated with SSc, which has limited therapeutic options. This therapy is non-invasive and safe and can be used without restriction in combination with other therapies, thus serving as a novel therapeutic method. CI - © Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com. FAU - Ishii, Tomonori AU - Ishii T AUID- ORCID: 0000-0001-5361-5824 AD - Clinical Research, Innovation and Education Center, Tohoku University Hospital, Sendai, Miyagi, Japan. AD - Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Miyagi, Japan. FAU - Kawaguchi, Yasushi AU - Kawaguchi Y AD - Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University, Tokyo, Japan. FAU - Ishikawa, Osamu AU - Ishikawa O AD - Department of Dermatology, Gunma University Graduate School of Medicine, Gunma, Japan. FAU - Takemori, Hiromitsu AU - Takemori H AD - Department of Rheumatology, Aomori Jikeikai Hospital, Aomori, Japan. FAU - Takasawa, Naruhiko AU - Takasawa N AD - Department of Rheumatology, Tohoku Medical and Pharmaceutical University Wakabayashi Hospital, Sendai, Japan. FAU - Kobayashi, Hitoshi AU - Kobayashi H AD - Suginoko-kai Marios Kobayashi Internal Medicine Clinic, Iwate, Japan. FAU - Takahashi, Yuichi AU - Takahashi Y AD - Yu Family Clinic, Miyagi, Japan. FAU - Yasuoka, Hidekata AU - Yasuoka H AD - Division of Rheumatology, Department of Internal Medicine, Fujita Health University School of Medicine, Aichi, Japan. FAU - Kodera, Takao AU - Kodera T AD - Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Miyagi, Japan. FAU - Takai, Osamu AU - Takai O AD - Ishibashi Hospital, Sendai, Japan. FAU - Nakaya, Izaya AU - Nakaya I AD - Department of Nephrology and Rheumatology, Iwate Prefectural Central Hospital, Morioka, Iwate, Japan. FAU - Sato, Yukio AU - Sato Y AD - Kaiyama Central Hospital, Sendai, Miyagi, Japan. FAU - Izumiyama, Tomomasa AU - Izumiyama T AD - Higashisendai Rheumatic Disease Clinic, Sendai, Miyagi, Japan. FAU - Fujii, Hiroshi AU - Fujii H AD - Department of Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Kamogawa, Yukiko AU - Kamogawa Y AD - Ichibancho Internal Medicine & Rheumatology Clinic, Sendai, Miyagi, Japan. FAU - Shirota, Yuko AU - Shirota Y AD - Department of Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Shirai, Tsuyoshi AU - Shirai T AD - Department of Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Fujita, Yoko AU - Fujita Y AD - Kouzenkai (Kouzen Association), Sakuragaoka Aozora Internal Medicine Clinic, Miyagi, Japan. FAU - Saito, Shinichiro AU - Saito S AD - IMS Meirikai Sendai General Hospital, Sendai, Miyagi, Japan. FAU - Chiu, Shih-Wei AU - Chiu SW AD - Division of Biostatistics, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Yamaguchi, Takuhiro AU - Yamaguchi T AD - Division of Biostatistics, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Shimokawa, Hiroaki AU - Shimokawa H AD - Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. FAU - Harigae, Hideo AU - Harigae H AD - Department of Hematology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. LA - eng PT - Clinical Trial, Phase III PT - Journal Article PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 RN - digital ulcers SB - IM MH - Humans MH - *Scleroderma, Systemic/complications/therapy MH - *Skin Ulcer/therapy/etiology MH - Female MH - Male MH - *Extracorporeal Shockwave Therapy/methods/adverse effects MH - Middle Aged MH - *Fingers MH - Treatment Outcome MH - Aged MH - Adult OTO - NOTNLM OT - Digital ulcer OT - Raynaud’s phenomenon OT - extracorporeal shock wave therapy OT - neovascularisation OT - systemic sclerosis EDAT- 2024/11/20 04:19 MHDA- 2025/04/08 13:54 CRDT- 2024/11/19 22:52 PHST- 2024/04/09 00:00 [received] PHST- 2024/11/04 00:00 [accepted] PHST- 2025/04/08 13:54 [medline] PHST- 2024/11/20 04:19 [pubmed] PHST- 2024/11/19 22:52 [entrez] AID - 7905071 [pii] AID - 10.1093/mr/roae104 [doi] PST - ppublish SO - Mod Rheumatol. 2025 Apr 8;35(3):484-495. doi: 10.1093/mr/roae104. PMID- 39953567 OWN - NLM STAT- MEDLINE DCOM- 20250507 LR - 20260127 IS - 2523-3106 (Electronic) IS - 2523-3106 (Linking) VI - 65 IP - 1 DP - 2025 Feb 14 TI - Patients with anti-PM/Scl-positive and idiopathic inflammatory myopathy resemble anti-synthetase syndrome. PG - 9 LID - 10.1186/s42358-025-00441-y [doi] AB - BACKGROUND: Anti-PM/Scl autoantibody has been associated with an overlap between polymyositis (PM) and systemic sclerosis (SSc). However, due to limited studies, the relevance of this autoantibody in patients with idiopathic inflammatory myopathies (IIMs) without SSc was analyzed. METHODS: This single-center retrospective cohort study was conducted between 2004 and 2024. A total of 93 adult patients with IIMs (66 with dermatomyositis and 27 with PM - EULAR/ACR 2017) without SSc were included: 16 anti-PM/Scl(+) and 77 anti-PM/Scl(-). Patients with other types of IIMs, cancer-associated myositis, or overlap myositis, including SSc, as well as those with other myositis-specific and/or myositis-associated autoantibodies were excluded. RESULTS: The median age, sex distribution, and median follow-up duration were comparable between the anti-PM/Scl(+) and anti-PM/Scl(-) groups. There were no differences in clinical and laboratory characteristics, except for a higher frequency of lung involvement, joint involvement, "mechanics' hand," "hiker's feet," and Raynaud's phenomenon, in contrast to a lower frequency of facial rash and "V"-neck sign in patients with anti-PM/Scl(+) than in those with anti-PM/Scl(-) (all P < 0.05). Furthermore, patients with anti-PM/Scl(+) exhibited a higher frequency of disease relapse (68.8% vs. 33.8%), disease activity (50.0% vs. 24.7%), and immunosuppressant use (methotrexate or azathioprine) at the last medical evaluation (all P < 0.05). Severe infection and death rates were comparable between the groups. CONCLUSIONS: Anti-PM/Scl positivity was observed in 17.2% of the sample analyzed in the present study. Patients with this autoantibody present clinical manifestations resembling anti-synthetase syndrome, with increased disease relapse and activity rates. CI - © 2025. The Author(s). FAU - Barbosa, Rafaella do Amaral AU - Barbosa RDA AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AUID- ORCID: 0000-0002-3682-4517 AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil. samuel.shinjo@usp.br. AD - Division of Rheumatology, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3º andar, sala 3184 - Cerqueira César, Sao Paulo, SP, CEP 01246-903, Brazil. samuel.shinjo@usp.br. LA - eng GR - 139748/2024-5/Conselho Nacional de Desenvolvimento Científico e Tecnológico/ GR - 301500/2022-3/Conselho Nacional de Desenvolvimento Científico e Tecnológico/ PT - Journal Article DEP - 20250214 PL - England TA - Adv Rheumatol JT - Advances in rheumatology (London, England) JID - 101734172 RN - 0 (Autoantibodies) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - EC 3.1.13.- (EXOSC10 protein, human) RN - EC 3.1.- (Exoribonucleases) RN - Antisynthetase syndrome SB - IM MH - Humans MH - Female MH - Male MH - Retrospective Studies MH - *Myositis/immunology/diagnosis/drug therapy MH - Middle Aged MH - *Autoantibodies/blood/immunology MH - Adult MH - *Polymyositis/immunology/diagnosis MH - Aged MH - Scleroderma, Systemic/immunology/diagnosis MH - Exosome Multienzyme Ribonuclease Complex/immunology MH - Dermatomyositis/immunology MH - Exoribonucleases OTO - NOTNLM OT - Anti-PM/Scl OT - Autoantibodies OT - Inflammatory myopathies OT - Myositis OT - Phenotypic OT - Systemic autoimmune myopathies COIS- Declarations. Ethics approval and consent to participate: The study was approved by the local ethics committee (CAAE 34954620.5.0000.0068). Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. EDAT- 2025/02/15 15:12 MHDA- 2025/02/15 15:13 CRDT- 2025/02/14 23:40 PHST- 2024/11/04 00:00 [received] PHST- 2025/02/10 00:00 [accepted] PHST- 2025/02/15 15:13 [medline] PHST- 2025/02/15 15:12 [pubmed] PHST- 2025/02/14 23:40 [entrez] AID - 10.1186/s42358-025-00441-y [pii] AID - 10.1186/s42358-025-00441-y [doi] PST - epublish SO - Adv Rheumatol. 2025 Feb 14;65(1):9. doi: 10.1186/s42358-025-00441-y. PMID- 37255624 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230601 IS - 1178-7015 (Print) IS - 1178-7015 (Electronic) IS - 1178-7015 (Linking) VI - 16 DP - 2023 TI - Copeptin as a Biomarker of Microcirculation Alterations in Systemic Sclerosis. PG - 1351-1361 LID - 10.2147/CCID.S409490 [doi] AB - BACKGROUND: Systemic sclerosis is a connective tissue disease characterized by vasculopathy and progressive fibrosis, leading to multiorgan dysfunction. Given the complex and not fully elucidated pathogenesis, biomarkers of rapid disease progression and therapeutic response are lacking. Copeptin, which reflects vasopressin activity in serum, is used in diagnosing or prognosing different cardiometabolic conditions. OBJECTIVE: The aim of study was to investigate the concentration of copeptin in patients with systemic sclerosis and correlate it with specific clinical symptoms. PATIENTS AND METHODS: Serum copeptin was measured in patients with systemic sclerosis (34 women and 3 men; mean age 57.6 years) and in healthy individuals (n=30) using commercially available ELISA kits. According to the criteria of LeRoy our systemic sclerosis cohort consisted of 17 patients with limited cutaneous systemic sclerosis (45.9%) and 20 diffuse cutaneous systemic sclerosis patients (54.1%). According to the criteria of LeRoy our systemic sclerosis cohort consisted of 17 patients with limited cutaneous systemic sclerosis (45.9%) and 20 diffuse cutaneous systemic sclerosis patients (54.1%). The median duration of the disease was 10 [4-14] years. RESULTS: We found significantly higher copeptin concentration in patients with systemic sclerosis (4.21 pmol/L [3.04-5.42]) in comparison to control group (3.40 pmol/L [2.38-3.76], p<0.01). Copeptin significantly correlated with Raynaud's condition score (r=0.801, p<0.05). Patients with "late" capillaroscopic patterns had higher copeptin concentrations (5.37 pmol/L [4.29-8.06]) than patients with "early" (2.43 pmol/L [2.25-3.20], p<0.05) and "active" patterns (3.93 pmol/L [2.92-5.16], p<0.05]). Copeptin was found to be significantly higher in SSc patients with DUs (5.71 pmol/L [IQR 4.85-8.06]) when compared to SSc patients without DUs (3.31 pmol/L, [2.28-4.30], p<0.05). Additionally, copeptin concentration had good diagnostic accuracy in discriminating between patients with and without digital ulcers (AUC=0.863). Alprostadil decreased copeptin concentration from 4.96 [4.02-6.01] to 3.86 pmol/L [3.17-4.63] (p<0.01) after 4-6 cycles of administration. CONCLUSION: Our findings suggest that copeptin may be a promising biomarker of microcirculation alterations in systemic sclerosis. CI - © 2023 Maciejewska et al. FAU - Maciejewska, Magdalena AU - Maciejewska M AUID- ORCID: 0000-0003-1009-6395 AD - Department of Dermatology, Doctoral School of Medical University of Warsaw, Warsaw, Poland. FAU - Stec, Albert AU - Stec A AUID- ORCID: 0000-0002-9719-8185 AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Zaremba, Michał AU - Zaremba M AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Maciejewski, Cezary AU - Maciejewski C AD - 1st Department of Cardiology, Medical University of Warsaw, Warsaw, Poland. FAU - Rudnicka, Lidia AU - Rudnicka L AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Sikora, Mariusz AU - Sikora M AUID- ORCID: 0000-0002-6162-9916 AD - National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. LA - eng PT - Journal Article DEP - 20230525 PL - New Zealand TA - Clin Cosmet Investig Dermatol JT - Clinical, cosmetic and investigational dermatology JID - 101543449 PMC - PMC10226486 OTO - NOTNLM OT - digital ulcers OT - endothelial dysfunction OT - serum biomarkers OT - systemic sclerosis OT - vasculopathy COIS- The authors declare no conflict of interest. EDAT- 2023/05/31 13:12 MHDA- 2023/05/31 13:13 PMCR- 2023/05/25 CRDT- 2023/05/31 10:26 PHST- 2023/02/27 00:00 [received] PHST- 2023/05/03 00:00 [accepted] PHST- 2023/05/31 13:13 [medline] PHST- 2023/05/31 13:12 [pubmed] PHST- 2023/05/31 10:26 [entrez] PHST- 2023/05/25 00:00 [pmc-release] AID - 409490 [pii] AID - 10.2147/CCID.S409490 [doi] PST - epublish SO - Clin Cosmet Investig Dermatol. 2023 May 25;16:1351-1361. doi: 10.2147/CCID.S409490. eCollection 2023. PMID- 31088420 OWN - NLM STAT- MEDLINE DCOM- 20190910 LR - 20200225 IS - 1471-2407 (Electronic) IS - 1471-2407 (Linking) VI - 19 IP - 1 DP - 2019 May 14 TI - Acral vascular necrosis associated with immune-check point inhibitors: case report with literature review. PG - 449 LID - 10.1186/s12885-019-5661-x [doi] LID - 449 AB - BACKGROUND: Treatment of solid malignancies has been revolutionized with the introduction of immune checkpoint inhibitors (ICIs) and their use is being expanded in therapy of different cancers. However, immune related adverse events (IRAEs) can occur during treatment. These side effects occur due to stimulation of the innate and adaptive immune system and can lead to serious complications. Recently, acral ischemia has been reported in some cases during treatment with programmed death-1 (PD-1) and cytotoxic T lymphocyte associated antigen-4 (CTLA-4) inhibitors. Here, we discuss a case in which acral necrosis developed after initiation of a PD-1 inhibitor. We offer a review of the existing literature on the pathophysiology, clinical course and treatment outcomes. CASE PRESENTATION: A 68-year-old female was diagnosed with stage IV non-small cell lung adenocarcinoma and was started on pembrolizumab. The patient developed sudden onset numbness and discoloration of fingertips bilaterally at week 25 after initiation of ICI treatment. Extensive workup to rule out hypercoagulable, autoimmune and vascular disease was unremarkable except for mild elevation of ANA and ESR. The symptoms quickly progressed into dry gangrene within four weeks and did not respond to medical or surgical treatment. Pembrolizumab was subsequently discontinued due to progression of metastatic disease. The patient refused further interventions and transitioned to hospice care where she expired after two months. CONCLUSION: Acral ischemia can develop during treatment of malignancies. This complication, although uncommon, canresult in digital amputation. Physicians should be aware of the possible progression of acral vascular necrosis when Raynaud's like symptoms develop. Larger studies are needed to confirm the role of ICIs in the pathogenesis of acral vascular necrosis. FAU - Khaddour, Karam AU - Khaddour K AUID- ORCID: 0000-0001-6697-3516 AD - Rosalind Franklin University of Medicine and Science, 915 Armistead Lane, McHenry, Chicago, IL, 60050, USA. Karam.khaddour@gmail.com. FAU - Singh, Veerpal AU - Singh V AD - North Western Medicine Centegra Healthcare System, Chicago, USA. FAU - Shayuk, Maryna AU - Shayuk M AD - Rosalind Franklin University of Medicine and Science, 915 Armistead Lane, McHenry, Chicago, IL, 60050, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20190514 PL - England TA - BMC Cancer JT - BMC cancer JID - 100967800 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Antineoplastic Agents, Immunological) RN - DPT0O3T46P (pembrolizumab) SB - IM MH - Aged MH - Antibodies, Monoclonal, Humanized/administration & dosage/*adverse effects MH - Antineoplastic Agents, Immunological/administration & dosage/*adverse effects MH - Carcinoma, Non-Small-Cell Lung/drug therapy MH - Disease Progression MH - Fatal Outcome MH - Female MH - Fingers/*pathology/surgery MH - Gangrene MH - Humans MH - Lung Neoplasms/drug therapy PMC - PMC6518656 OTO - NOTNLM OT - Acral ischemia OT - Case reports OT - Immune related adverse events (IRAEs) OT - Immunotherapy OT - Necrosis OT - Small vessel vasculitis COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Not applicable. CONSENT FOR PUBLICATION: Written informed consent for publication of the clinical details and clinical images was obtained from the patient’s medical power of attorney. A copy of the written consent is available for review by the editor of the journal. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/05/16 06:00 MHDA- 2019/09/11 06:00 PMCR- 2019/05/14 CRDT- 2019/05/16 06:00 PHST- 2018/12/03 00:00 [received] PHST- 2019/04/30 00:00 [accepted] PHST- 2019/05/16 06:00 [entrez] PHST- 2019/05/16 06:00 [pubmed] PHST- 2019/09/11 06:00 [medline] PHST- 2019/05/14 00:00 [pmc-release] AID - 10.1186/s12885-019-5661-x [pii] AID - 5661 [pii] AID - 10.1186/s12885-019-5661-x [doi] PST - epublish SO - BMC Cancer. 2019 May 14;19(1):449. doi: 10.1186/s12885-019-5661-x. PMID- 23812032 OWN - NLM STAT- MEDLINE DCOM- 20141013 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 34 IP - 1 DP - 2014 Jan TI - Sex differences in patients with systemic lupus erythematosus from Northwest Spain. PG - 11-24 LID - 10.1007/s00296-013-2798-9 [doi] AB - To further establish potential differences according to sex in systemic lupus erythematosus (SLE) patients from Southern Europe. We assessed clinical and epidemiological data of patients diagnosed with SLE according to the 1982 American College of Rheumatology classification criteria at the single hospital for a well-defined population of Northwest Spain, between 1987 and 2006. Prevalence in December 2006 and age-standardized incidence rates in the whole period were estimated. Kaplan-Meier method was used in order to estimate the probability of survivorship. Women outnumbered men [127 (84.7%) vs. 23 (15.3%)]. The median age at the time of disease diagnosis in men was 54 years versus 43 in women (p < 0.001). Annual incidence rates were higher in women [5.9 (95% confidence interval--CI 4.9-7.0) per 100,000 population] than in men [1.1 (95% CI 0.7-1.7) per 100,000 population; p < 0.001]. Raynaud's phenomenon was more common in women (40.9 vs. 3.0%; p = 0.01). While the frequency of secondary Sjögren's syndrome was increased in women (p = 0.02), renal disease at the time of diagnosis (39.1 vs. 15.0%; p < 0.01) and over the course of the disease was more common in men (43.5 vs. 24.4%; p = 0.06). Higher frequency of thrombocytopenia (39.1 vs. 16.5%; p = 0.01) and lower frequency of anti-SSA (13.0 vs. 31.5%; p = 0.08) and anti-SSB (0 vs. 17.7%; p = 0.03) were observed in men. The 5- and 10-year survival probabilities were nonsignificantly reduced in men (91.3 and 78.3 3% vs. 94.6 and 89.2% in women). The frequency of some clinical manifestations is different in men and women with SLE. Higher awareness of these peculiarities may help to establish appropriate diagnosis and management of SLE in men. FAU - Alonso, Maria D AU - Alonso MD AD - Division of Internal Medicine, Hospital Xeral-Calde, Lugo, Spain. FAU - Martínez-Vázquez, Francisco AU - Martínez-Vázquez F FAU - Riancho-Zarrabeitia, Leyre AU - Riancho-Zarrabeitia L FAU - Díaz de Terán, Teresa AU - Díaz de Terán T FAU - Miranda-Filloy, Jose A AU - Miranda-Filloy JA FAU - Blanco, Ricardo AU - Blanco R FAU - González-Juanatey, Carlos AU - González-Juanatey C FAU - Llorca, Javier AU - Llorca J FAU - González-Gay, Miguel A AU - González-Gay MA LA - eng PT - Comparative Study PT - Journal Article DEP - 20130628 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (SS-A antibodies) RN - 0 (SS-B antibodies) SB - IM CIN - Rheumatol Int. 2014 Aug;34(8):1179-80. doi: 10.1007/s00296-013-2899-5. PMID: 24248805 CIN - Rheumatol Int. 2015 May;35(5):939-40. doi: 10.1007/s00296-014-3124-x. PMID: 25213024 MH - Adult MH - Age Distribution MH - Age of Onset MH - Aged MH - Antibodies, Antinuclear/blood MH - Biomarkers/blood MH - Female MH - Humans MH - Incidence MH - Kaplan-Meier Estimate MH - Logistic Models MH - Lupus Erythematosus, Systemic/blood/diagnosis/*epidemiology/mortality MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Prevalence MH - Prognosis MH - Retrospective Studies MH - Risk Factors MH - Sex Distribution MH - Sex Factors MH - Sjogren's Syndrome/epidemiology MH - Spain/epidemiology MH - Thrombocytopenia/epidemiology MH - Time Factors EDAT- 2013/07/03 06:00 MHDA- 2014/10/14 06:00 CRDT- 2013/07/02 06:00 PHST- 2013/04/04 00:00 [received] PHST- 2013/06/04 00:00 [accepted] PHST- 2013/07/02 06:00 [entrez] PHST- 2013/07/03 06:00 [pubmed] PHST- 2014/10/14 06:00 [medline] AID - 10.1007/s00296-013-2798-9 [doi] PST - ppublish SO - Rheumatol Int. 2014 Jan;34(1):11-24. doi: 10.1007/s00296-013-2798-9. Epub 2013 Jun 28. PMID- 25896533 OWN - NLM STAT- MEDLINE DCOM- 20160418 LR - 20240610 IS - 1434-9949 (Electronic) IS - 0770-3198 (Print) IS - 0770-3198 (Linking) VI - 34 IP - 7 DP - 2015 Jul TI - Clinical associations of anti-Smith antibodies in PROFILE: a multi-ethnic lupus cohort. PG - 1217-23 LID - 10.1007/s10067-015-2941-y [doi] AB - The aim of this study was to determine the association of anti-Sm antibodies with clinical manifestations, comorbidities, and disease damage in a large multi-ethnic SLE cohort. SLE patients (per American College of Rheumatology criteria), age ≥16 years, disease duration ≤10 years at enrollment, and defined ethnicity (African American, Hispanic or Caucasian), from a longitudinal US cohort were studied. Socioeconomic-demographic features, cumulative clinical manifestations, comorbidities, and disease damage (as per the Systemic Lupus International Collaborating Clinics Damage Index [SDI]) were determined. The association of anti-Sm antibodies with clinical features was examined using multivariable logistic regression analyses adjusting for age, gender, ethnicity, disease duration, level of education, health insurance, and smoking. A total of 2322 SLE patients were studied. The mean (standard deviation, SD) age at diagnosis was 34.4 (12.8) years and the mean (SD) disease duration was 9.0 (7.9) years; 2127 (91.6%) were women. Anti-Sm antibodies were present in 579 (24.9%) patients. In the multivariable analysis, anti-Sm antibodies were significantly associated with serositis, renal involvement, psychosis, vasculitis, Raynaud's phenomenon, hemolytic anemia, leukopenia, lymphopenia, and arterial hypertension. No significant association was found for damage accrual. In this cohort of SLE patients, anti-Sm antibodies were associated with several clinical features including serious manifestations such as renal, neurologic, and hematologic disorders as well as vasculitis. FAU - Arroyo-Ávila, Mariangelí AU - Arroyo-Ávila M AD - Division of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, PO Box 365067, San Juan, PR, 00936-5067, USA. FAU - Santiago-Casas, Yesenia AU - Santiago-Casas Y FAU - McGwin, Gerald Jr AU - McGwin G Jr FAU - Cantor, Ryan S AU - Cantor RS FAU - Petri, Michelle AU - Petri M FAU - Ramsey-Goldman, Rosalind AU - Ramsey-Goldman R FAU - Reveille, John D AU - Reveille JD FAU - Kimberly, Robert P AU - Kimberly RP FAU - Alarcón, Graciela S AU - Alarcón GS FAU - Vilá, Luis M AU - Vilá LM FAU - Brown, Elizabeth E AU - Brown EE LA - eng GR - K24-AR002138/AR/NIAMS NIH HHS/United States GR - UL1 TR000150/TR/NCATS NIH HHS/United States GR - R01 AR064820/AR/NIAMS NIH HHS/United States GR - P60AR064464/AR/NIAMS NIH HHS/United States GR - U54 MD007587/MD/NIMHD NIH HHS/United States GR - M01 RR000048/RR/NCRR NIH HHS/United States GR - UL1 TR001422/TR/NCATS NIH HHS/United States GR - M01 RR000052/RR/NCRR NIH HHS/United States GR - M01 RR002558/RR/NCRR NIH HHS/United States GR - P01 AR049084/AR/NIAMS NIH HHS/United States GR - P01 AR49084/AR/NIAMS NIH HHS/United States GR - M01-RR02558/RR/NCRR NIH HHS/United States GR - R01 AR42503/AR/NIAMS NIH HHS/United States GR - UL1 TR000165/TR/NCATS NIH HHS/United States GR - M01-RR00048/RR/NCRR NIH HHS/United States GR - R01-AR42503/AR/NIAMS NIH HHS/United States GR - P60 AR064464/AR/NIAMS NIH HHS/United States GR - P60 AR048098/AR/NIAMS NIH HHS/United States GR - 8UL1TR000150/TR/NCATS NIH HHS/United States GR - M01-RR00052/RR/NCRR NIH HHS/United States GR - P60AR048098/AR/NIAMS NIH HHS/United States GR - R01 AR043727/AR/NIAMS NIH HHS/United States GR - K24 AR002138/AR/NIAMS NIH HHS/United States GR - R01 AR042503/AR/NIAMS NIH HHS/United States GR - R 8U54MD00758/MD/NIMHD NIH HHS/United States GR - AR43727/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150422 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Antibodies) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adolescent MH - Adult MH - Black or African American MH - Antibodies/*immunology MH - Antibodies, Antinuclear/chemistry MH - Cohort Studies MH - Comorbidity MH - Cross-Sectional Studies MH - Ethnicity MH - Female MH - Hispanic or Latino MH - Humans MH - Longitudinal Studies MH - Lupus Erythematosus, Systemic/*ethnology/*immunology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - United States MH - White People MH - Young Adult PMC - PMC4475431 MID - NIHMS685332 EDAT- 2015/04/22 06:00 MHDA- 2016/04/19 06:00 PMCR- 2016/07/01 CRDT- 2015/04/22 06:00 PHST- 2015/02/13 00:00 [received] PHST- 2015/04/12 00:00 [accepted] PHST- 2015/03/24 00:00 [revised] PHST- 2015/04/22 06:00 [entrez] PHST- 2015/04/22 06:00 [pubmed] PHST- 2016/04/19 06:00 [medline] PHST- 2016/07/01 00:00 [pmc-release] AID - 10.1007/s10067-015-2941-y [doi] PST - ppublish SO - Clin Rheumatol. 2015 Jul;34(7):1217-23. doi: 10.1007/s10067-015-2941-y. Epub 2015 Apr 22. PMID- 35059476 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220122 IS - 2352-1872 (Print) IS - 2352-1872 (Electronic) IS - 2352-1872 (Linking) VI - 16 DP - 2022 Jun TI - Cancellous bone-like tissue replacement from calcinosis in patients with systemic sclerosis with multiple external root resorption. PG - 101165 LID - 10.1016/j.bonr.2021.101165 [doi] LID - 101165 AB - Calcinosis is frequently observed in patients with systemic sclerosis (SSc). The fundamental treatment of calcinosis has not yet been established. During follow-up, calcinosis in the subcutaneous surface is often spontaneously extracted or remains confined by fibrous tissues. We previously identified a new symptom in SSc patients, multiple external root resorption (MERR), and these patients had calcifications in the nasal spine. Here, we report for the first time that calcinosis at the nasal spine in patients with MERR can be replaced by cancellous bone-like tissue. Patients 1 and 2 were a 62-year-old Japanese female and a 45-year-old Japanese female (respectively) with MERR who had been previously treated for SSc (Patient 1: limited type, positive for anti-centromere antibody; Patient 2: diffuse type, positive for anti-Scl70 and anti-SS-A antibodies). Patient 3 was a 57-year-old female with MERR who had been previously treated for SSc (diffuse type, positive anti-Scl-70 antibody) and underwent denosumab injection for osteoporosis. Cone-beam computed tomography (CBCT) and CT images in the calcifications at the nasal spine in Patient 1 and 2 were replaced with cancellous bone-like tissue, but not in Patient 3. Serum laboratory examination was performed to assess the systemic bone disease. All three patients had normal clinical data within the references, apart from slightly higher 1,25-dihydroxyvitamin D levels in Patient 1. SSc patients with calcinosis in the maxillofacial area need to be examined carefully for bone replacement using CBCT or CT. CI - © 2022 The Authors. Published by Elsevier Inc. FAU - Memida, Takumi AU - Memida T AD - Department of Periodontal Medicine, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Matsuda, Shinji AU - Matsuda S AD - Department of Periodontal Medicine, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Nakamoto, Takashi AU - Nakamoto T AD - Department of Oral and Maxillofacial Radiology, Institute of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Ouhara, Kazuhisa AU - Ouhara K AD - Department of Periodontal Medicine, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Kajiya, Mikihito AU - Kajiya M AD - Department of Periodontal Medicine, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Hirata, Shintaro AU - Hirata S AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Sugiyama, Eiji AU - Sugiyama E AD - Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. AD - Department of Internal Medicine, JA Yoshida General Hospital, 3666 Yoshida, Yoshida-cho, Akitakata, Hiroshima 731-0501, Japan. FAU - Kakimoto, Naoya AU - Kakimoto N AD - Department of Oral and Maxillofacial Radiology, Institute of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. FAU - Mizuno, Noriyoshi AU - Mizuno N AD - Department of Periodontal Medicine, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8553, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20220108 PL - United States TA - Bone Rep JT - Bone reports JID - 101646176 PMC - PMC8760497 OTO - NOTNLM OT - BAP, bone alkaline phosphatase OT - CBCT, cone-beam computed tomography OT - CREST, calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia OT - Calcinosis OT - FOP, fibrodysplasia ossificans progressive OT - HO, heterotopic ossification OT - Heterotopic ossification OT - MERR, multiple external root resorption OT - Multiple external root resorption OT - NSAID, non-steroidal anti-inflammatory drugs OT - POH, progressive osseous heteroplasia OT - PTH, parathyroid hormone OT - RANK, receptor activator of nuclear factor-kappa B OT - SSc, systemic sclerosis OT - Systemic sclerosis COIS- The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2022/01/22 06:00 MHDA- 2022/01/22 06:01 PMCR- 2022/01/08 CRDT- 2022/01/21 06:23 PHST- 2021/10/07 00:00 [received] PHST- 2021/12/27 00:00 [revised] PHST- 2021/12/31 00:00 [accepted] PHST- 2022/01/21 06:23 [entrez] PHST- 2022/01/22 06:00 [pubmed] PHST- 2022/01/22 06:01 [medline] PHST- 2022/01/08 00:00 [pmc-release] AID - S2352-1872(21)00422-8 [pii] AID - 101165 [pii] AID - 10.1016/j.bonr.2021.101165 [doi] PST - epublish SO - Bone Rep. 2022 Jan 8;16:101165. doi: 10.1016/j.bonr.2021.101165. eCollection 2022 Jun. PMID- 37047117 OWN - NLM STAT- MEDLINE DCOM- 20230414 LR - 20260127 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 24 IP - 7 DP - 2023 Mar 24 TI - Low P-Selectin Glycoprotein Ligand-1 Expression in Neutrophils Associates with Disease Activity and Deregulated NET Formation in Systemic Lupus Erythematosus. LID - 10.3390/ijms24076144 [doi] LID - 6144 AB - Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by the generation of anti-DNA autoantibodies due to exposure of immune cells to excessive amounts of extracellular DNA. Lack of P-selectin in mice induces the development of a lupus-like syndrome and patients with cutaneous lupus have reduced P-selectin expression in skin vessels. Using flow cytometry we analyzed in healthy donors and patients the expression of P-selectin Glycoprotein Ligand-1 (PSGL-1) in circulating neutrophils and the implication of PSGL-1/P-selectin interaction in neutrophil extracellular traps (NETs) generation. We found a statistical significance that neutrophils from active SLE patients have a reduced expression of PSGL-1 and low levels of PSGL-1 in neutrophils from SLE patients associated with the presence of anti-dsDNA antibodies, clinical lung involvement, Raynaud's phenomenon, and positive lupus anticoagulant. PSGL-1 is present along the DNA in the NET. In healthy donors, neutrophil interaction with immobilized P-selectin triggers Syk activation, increases the NETs percentage and reduces the amount of DNA extruded in the NETs. In active SLE patients, neutrophil interaction with P-selectin does not activate Syk or reduce the amount of DNA extruded in the NETs, that might contribute to increase the extracellular level of DNA and hence, to disease pathogenesis. FAU - Muñoz-Callejas, Antonio AU - Muñoz-Callejas A AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - González-Sánchez, Elena AU - González-Sánchez E AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Silván, Javier AU - Silván J AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - San Antonio, Esther AU - San Antonio E AUID- ORCID: 0000-0001-8667-7247 AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - González-Tajuelo, Rafael AU - González-Tajuelo R AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Ramos-Manzano, Alejandra AU - Ramos-Manzano A AUID- ORCID: 0000-0002-6376-058X AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Sánchez-Abad, Inés AU - Sánchez-Abad I AUID- ORCID: 0000-0003-2661-6367 AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - González-Alvaro, Isidoro AU - González-Alvaro I AUID- ORCID: 0000-0001-9614-5199 AD - Rheumatology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - García-Pérez, Javier AU - García-Pérez J AUID- ORCID: 0000-0003-0026-1043 AD - Pulmonology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Tomero, Eva G AU - Tomero EG AD - Rheumatology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - de Vicuña, Rosario García AU - de Vicuña RG AUID- ORCID: 0000-0001-9096-3780 AD - Rheumatology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Vicente-Rabaneda, Esther F AU - Vicente-Rabaneda EF AD - Rheumatology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. FAU - Castañeda, Santos AU - Castañeda S AUID- ORCID: 0000-0002-7748-853X AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. AD - Rheumatology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. AD - Catedra UAM-Roche, EPID-Future, Department of Medicine, Universidad Autónoma de Madrid, 28049 Madrid, Spain. FAU - Urzainqui, Ana AU - Urzainqui A AUID- ORCID: 0000-0001-9326-6112 AD - Immunology Department, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Hospital de la Princesa, 28006 Madrid, Spain. LA - eng GR - FIS-PI17-01819/Spanish Ministry of Health and ISCIII (co-financed by Fondos FEDER)/ GR - FIS-PI20-01690/Spanish Ministry of Health and ISCIII (co-financed by Fondos FEDER)/ GR - EuroNanoMed III AC17-0027/Spanish Ministry of Health and ISCIII (co-financed by Fondos FEDER)/ GR - "Molecular stratification of patients with giant cell arteritis to tailor glucocorticoid and tocili-zumab therapy (START Project)"/FOREUM, Foundation for Research in Rheumatology/ PT - Journal Article DEP - 20230324 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 RN - 9007-49-2 (DNA) RN - 0 (P-Selectin) RN - 0 (P-selectin ligand protein) RN - 0 (Membrane Glycoproteins) SB - IM MH - Animals MH - Mice MH - *Autoimmune Diseases/metabolism MH - DNA/metabolism MH - *Extracellular Traps/metabolism MH - *Lupus Erythematosus, Systemic MH - Neutrophils/metabolism MH - P-Selectin/metabolism MH - Humans MH - Membrane Glycoproteins PMC - PMC10093849 OTO - NOTNLM OT - NETs OT - P-selectin OT - PSGL-1 OT - SLE pathogenesis OT - neutrophils COIS- The authors declare no conflict of interest. EDAT- 2023/04/14 06:00 MHDA- 2023/04/14 06:42 PMCR- 2023/03/24 CRDT- 2023/04/13 01:10 PHST- 2023/01/19 00:00 [received] PHST- 2023/03/14 00:00 [revised] PHST- 2023/03/20 00:00 [accepted] PHST- 2023/04/14 06:42 [medline] PHST- 2023/04/13 01:10 [entrez] PHST- 2023/04/14 06:00 [pubmed] PHST- 2023/03/24 00:00 [pmc-release] AID - ijms24076144 [pii] AID - ijms-24-06144 [pii] AID - 10.3390/ijms24076144 [doi] PST - epublish SO - Int J Mol Sci. 2023 Mar 24;24(7):6144. doi: 10.3390/ijms24076144. PMID- 28426895 OWN - NLM STAT- MEDLINE DCOM- 20170821 LR - 20260127 IS - 2326-5205 (Electronic) IS - 2326-5191 (Linking) VI - 69 IP - 8 DP - 2017 Aug TI - Short-Term Pulmonary Function Trends Are Predictive of Mortality in Interstitial Lung Disease Associated With Systemic Sclerosis. PG - 1670-1678 LID - 10.1002/art.40130 [doi] AB - OBJECTIVE: To determine the prognostic value of pulmonary function test (PFT) trends at 1 and 2 years in interstitial lung disease (ILD) associated with systemic sclerosis (SSc). METHODS: The prognostic significance of PFT trends at 1 year (n = 162) and 2 years (n = 140) was examined against 15-year survival in patients with SSc-associated ILD. PFT trends, expressed as continuous change and as categorical change in separate analyses, were examined against mortality in univariate and multivariate models. SSc-associated ILD was defined at presentation as either limited lung fibrosis or extensive lung fibrosis, using the United Kingdom Raynaud's and Scleroderma Association severity staging system. RESULTS: One-year PFT trends were predictive of mortality only in patients with extensive lung fibrosis: categorical change in the forced vital capacity (FVC), alone or in combination with categorical change in the diffusing capacity for carbon monoxide (DLco), had greater prognostic significance than continuous change in the FVC or trends in other PFT variables. Taking into account both prognostic value and sensitivity to change, the optimal definition of progression for trial purposes was an FVC and DLco composite end point, consisting of either an FVC decline from baseline of ≥10% or an FVC decline of 5-9% in association with a DLco decline of ≥15%. At 2 years, gas transfer trends had the greatest prognostic significance, in the whole cohort and in those with limited lung fibrosis. However, in patients with extensive lung fibrosis, the above-defined FVC and DLco composite end point was the strongest prognostic determinant. Larger changes in the FVC:DLco ratio than in the carbon monoxide transfer coefficient were required to achieve prognostic significance. CONCLUSION: Based on linkages to long-term outcomes, these findings provide support for use of routine spirometry and gas transfer monitoring in patients with SSc-associated ILD, with further evaluation of a composite FVC and DLco end point warranted for trial purposes. CI - © 2017, American College of Rheumatology. FAU - Goh, Nicole S AU - Goh NS AD - Austin Hospital, Melbourne, Victoria, Australia, and Royal Brompton and Harefield NHS Foundation Trust, London, UK. FAU - Hoyles, Rachel K AU - Hoyles RK AD - Oxford University Hospitals, NHS Foundation Trust, Oxford, UK, and Royal Free Hospital, London, UK. FAU - Denton, Christopher P AU - Denton CP AD - Royal Free Hospital, London, UK. FAU - Hansell, David M AU - Hansell DM AD - Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK. FAU - Maher, Toby M AU - Maher TM AD - Imperial College and Royal Brompton Hospital, London, UK. FAU - Nicholson, Andrew G AU - Nicholson AG AD - Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK. FAU - Wells, Athol U AU - Wells AU AD - Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK. LA - eng GR - 20719/ARC_/Arthritis Research UK/United Kingdom PT - Journal Article DEP - 20170718 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 SB - IM CIN - Nat Rev Rheumatol. 2017 Aug;13(8):455-456. doi: 10.1038/nrrheum.2017.103. PMID: 28680133 MH - Adult MH - Disease Progression MH - Female MH - Humans MH - Lung/*physiopathology MH - Lung Diseases, Interstitial/etiology/mortality/*physiopathology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Prognosis MH - Pulmonary Diffusing Capacity MH - Pulmonary Fibrosis/etiology/mortality/*physiopathology MH - Respiratory Function Tests MH - Scleroderma, Systemic/complications/mortality/*physiopathology MH - Severity of Illness Index MH - United Kingdom MH - Vital Capacity EDAT- 2017/04/21 06:00 MHDA- 2017/08/22 06:00 CRDT- 2017/04/21 06:00 PHST- 2016/08/15 00:00 [received] PHST- 2017/04/13 00:00 [accepted] PHST- 2017/04/21 06:00 [pubmed] PHST- 2017/08/22 06:00 [medline] PHST- 2017/04/21 06:00 [entrez] AID - 10.1002/art.40130 [doi] PST - ppublish SO - Arthritis Rheumatol. 2017 Aug;69(8):1670-1678. doi: 10.1002/art.40130. Epub 2017 Jul 18. PMID- 38337770 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240212 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 14 IP - 3 DP - 2024 Jan 24 TI - Nailfold Capillaroscopy Analysis Can Add a New Perspective to Biomarker Research in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. LID - 10.3390/diagnostics14030254 [doi] LID - 254 AB - BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), all of which are characterised by inflammation of small-medium-sized vessels. Progressive understanding of these diseases has allowed researchers and clinicians to start discussing nailfold video capillaroscopy (NVC) as a future tool for many applications in daily practice. Today, NVC plays a well-established and validated role in differentiating primary from secondary Raynaud's phenomenon correlated with scleroderma. Nevertheless, there has not been sufficient attention paid to its real potential in the ANCA-associated vasculitis. In fact, the role of NVC in vasculitis has never been defined and studied in a multicentre and multinational study. In this review, we carried out a literature analysis to identify and synthesise the possible role of capillaroscopy for patients with ANCA-associated vasculitis. METHODS: Critical research was performed in the electronic archive (PUBMED, UpToDate, Google Scholar, ResearchGate), supplemented with manual research. We searched in these databases for articles published until November 2023. The following search words were searched in the databases in all possible combinations: capillaroscopy, video capillaroscopy, nailfold-video capillaroscopy, ANCA-associated vasculitis, vasculitis, granulomatosis with polyangiitis, EGPA, and microscopic polyangiitis. RESULTS: The search identified 102 unique search results. After the evaluation, eight articles were selected for further study. The literature reported that capillaroscopy investigations documented non-specific abnormalities in 70-80% of AAV patients. Several patients showed neoangiogenesis, capillary loss, microhaemorrhages, and bushy and enlarged capillaries as the most frequent findings. Furthermore, the difference between active phase and non-active phase in AAV patients was clearly discernible. The non-active phase showed similar rates of capillaroscopy alterations compared to the healthy subjects, but the active phase had higher rates in almost all common abnormalities instead. CONCLUSIONS: Microvascular nailfold changes, observed in patients affected by vasculitis, may correlate with the outcome of these patients. However, these non-specific abnormalities may help in the diagnosis of vasculitis. As such, new analysis analyses are necessary to confirm our results. FAU - Screm, Gianluca AU - Screm G AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Mondini, Lucrezia AU - Mondini L AUID- ORCID: 0000-0002-7043-9639 AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Confalonieri, Paola AU - Confalonieri P AUID- ORCID: 0000-0002-7284-5016 AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Salton, Francesco AU - Salton F AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Trotta, Liliana AU - Trotta L AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Barbieri, Mariangela AU - Barbieri M AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Mari, Marco AU - Mari M AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Reccardini, Nicolò AU - Reccardini N AUID- ORCID: 0009-0004-5792-3655 AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Della Porta, Rossana AU - Della Porta R AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Kodric, Metka AU - Kodric M AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Bandini, Giulia AU - Bandini G AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine, University of Florence, Azienda Ospedaliero-Universitaria Careggi (AOUC), 50134 Florence, Italy. FAU - Hughes, Michael AU - Hughes M AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK. FAU - Bellan, Mattia AU - Bellan M AUID- ORCID: 0000-0003-1488-8736 AD - Department of Translational Medicine, Università del Piemonte Orientale (UPO), 28100 Novara, Italy. AD - Center for Autoimmune and Allergic Disease (CAAD), Università del Piemonte Orientale (UPO), 28100 Novara, Italy. AD - Azienda Ospedaliero-Universitaria, Maggiore della Carità, 28100 Novara, Italy. FAU - Lerda, Selene AU - Lerda S AD - Graduate School, University of Milan, 20149 Milan, Italy. FAU - Confalonieri, Marco AU - Confalonieri M AUID- ORCID: 0000-0002-4791-768X AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Pulmonology Unit, Department of Medical Surgical and Healt Sciencies, University of Trieste, Hospital of Cattinara, 34149 Trieste, Italy. LA - eng PT - Journal Article PT - Review DEP - 20240124 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10854947 OTO - NOTNLM OT - ANCA-associated vasculitis OT - autoimmune disease OT - disease severity OT - microvascular abnormality OT - nailfold video capillaroscopy OT - vasculitis COIS- The authors declare no conflicts of interest. EDAT- 2024/02/10 10:53 MHDA- 2024/02/10 10:54 PMCR- 2024/01/24 CRDT- 2024/02/10 01:05 PHST- 2023/12/12 00:00 [received] PHST- 2024/01/12 00:00 [revised] PHST- 2024/01/23 00:00 [accepted] PHST- 2024/02/10 10:54 [medline] PHST- 2024/02/10 10:53 [pubmed] PHST- 2024/02/10 01:05 [entrez] PHST- 2024/01/24 00:00 [pmc-release] AID - diagnostics14030254 [pii] AID - diagnostics-14-00254 [pii] AID - 10.3390/diagnostics14030254 [doi] PST - epublish SO - Diagnostics (Basel). 2024 Jan 24;14(3):254. doi: 10.3390/diagnostics14030254. PMID- 34042326 OWN - NLM STAT- MEDLINE DCOM- 20220118 LR - 20220914 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 73 IP - 12 DP - 2021 Dec TI - Anticentromere Antibody Levels and Isotypes and the Development of Systemic Sclerosis. PG - 2338-2347 LID - 10.1002/art.41814 [doi] AB - OBJECTIVE: Little is known on the disease course of very early systemic sclerosis (SSc). Among the information yet to be elucidated is whether anticentromere antibody (ACA) isotype levels can serve as biomarkers for future SSc development and for organ involvement. This study was undertaken to evaluate whether IgG, IgM, and IgA ACA levels in IgG ACA-positive patients are associated with disease severity and/or progression from very early SSc to definite SSc. METHODS: IgG ACA-positive patients from 5 different cohorts who had very early SSc or SSc fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria were included. A diagnosis of very early SSc was based on the presence of IgG ACAs and Raynaud's phenomenon, and/or puffy fingers and/or abnormal nailfold capillaroscopy, but not fulfilling the ACR/EULAR 2013 criteria for SSc. Multivariable regression analyses were performed to determine the association between baseline ACA isotype levels and progression to definite SSc with organ involvement. RESULTS: Six hundred twenty-five IgG ACA-positive patients were included, of whom 138 (22%) fulfilled the criteria for very early SSc and 487 (78%) had definite SSc. Levels of IgG ACAs (odds ratio 2.5 [95% confidence interval 1.8-3.7]) and IgM ACAs (odds ratio 1.8 [95% confidence interval 1.3-2.3]) were significantly higher in patients with definite SSc. Of 115 patients with very early SSc with follow-up, progression to definite SSc occurred within 5 years in 48 (42%). Progression to definite SSc was associated with higher IgG ACA levels at baseline (odds ratio 4.3 [95% confidence interval 1.7-10.7]). CONCLUSION: ACA isotype levels may serve as biomarkers to identify patients with very early SSc who are at risk for disease progression to definite SSc. CI - © 2021 The Authors. Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - van Leeuwen, Nina M AU - van Leeuwen NM AUID- ORCID: 0000-0003-4228-012X AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Boonstra, Maaike AU - Boonstra M AUID- ORCID: 0000-0002-3322-0160 AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Bakker, Jaap A AU - Bakker JA AUID- ORCID: 0000-0002-2918-7361 AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Grummels, Annette AU - Grummels A AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Jordan, Suzana AU - Jordan S AD - University Hospital Zurich, Zurich, Switzerland. FAU - Liem, Sophie AU - Liem S AUID- ORCID: 0000-0003-0328-7062 AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-2413-1959 AD - University Hospital Zurich, Zurich, Switzerland. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AD - Oslo University Hospital and Rikshospitalet, Oslo, Norway. FAU - Melsens, Karin AU - Melsens K AUID- ORCID: 0000-0003-4125-7163 AD - Ghent University, Ghent University Hospital, and VIB-UGent Center for Inflammation Research, Ghent, Belgium. FAU - Smith, Vanessa AU - Smith V AD - Ghent University, Ghent University Hospital, and VIB-UGent Center for Inflammation Research, Ghent, Belgium. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Bordeaux University Hospital, Bordeaux, France. FAU - Scherer, Hans U AU - Scherer HU AUID- ORCID: 0000-0002-5700-5617 AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Toes, René AU - Toes R AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - Huizinga, Tom W J AU - Huizinga TWJ AD - Leiden University Medical Center, Leiden, The Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Leiden University Medical Center, Leiden, The Netherlands. LA - eng PT - Journal Article DEP - 20211102 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (anticentromere antibody) SB - IM CIN - Arthritis Rheumatol. 2022 Sep;74(9):1606-1607. doi: 10.1002/art.42159. PMID: 35536755 MH - Adult MH - Aged MH - Antibodies, Antinuclear/*blood MH - Biomarkers/blood MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Scleroderma, Systemic/blood/diagnostic imaging/*immunology MH - Severity of Illness Index PMC - PMC9297867 EDAT- 2021/05/28 06:00 MHDA- 2022/01/19 06:00 PMCR- 2022/07/20 CRDT- 2021/05/27 09:04 PHST- 2020/08/13 00:00 [received] PHST- 2021/05/11 00:00 [accepted] PHST- 2021/05/28 06:00 [pubmed] PHST- 2022/01/19 06:00 [medline] PHST- 2021/05/27 09:04 [entrez] PHST- 2022/07/20 00:00 [pmc-release] AID - ART41814 [pii] AID - 10.1002/art.41814 [doi] PST - ppublish SO - Arthritis Rheumatol. 2021 Dec;73(12):2338-2347. doi: 10.1002/art.41814. Epub 2021 Nov 2. PMID- 27389713 OWN - NLM STAT- MEDLINE DCOM- 20170816 LR - 20240610 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 69 IP - 6 DP - 2017 Jun TI - Risk of Digital Vascular Events in Scleroderma Patients Who Have Both Anticentromere and Anti-Interferon-Inducible Protein 16 Antibodies. PG - 922-926 LID - 10.1002/acr.22978 [doi] AB - OBJECTIVE: To evaluate whether scleroderma patients who are double-positive for anti-interferon-inducible protein 16 (anti-IFI-16) antibodies and anticentromere (anti-CENP) antibodies are at increased risk for significant digital vascular events relative to patients positive for anti-CENP antibodies alone. METHODS: Sera from 165 scleroderma patients who tested positive for anti-CENP antibodies upon clinical evaluation were reassayed for both anti-CENP and anti-IFI-16 antibodies using enzyme-linked immunosorbent assay testing. Patients who were positive for anti-CENP antibodies alone were then compared to patients who were double-positive for both anti-IFI-16 and anti-CENP antibodies. The association between a history of significant digital vascular events (digital pits, ischemic digital ulcers, and/or gangrene) and double-positive antibody status was examined using chi-square tests. After completion of univariate analysis, multivariable analyses were done to adjust for clinically relevant covariates. RESULTS: Of the 165 anti-CENP antibody positive patients, 21 (12.7%) also had anti-IFI-16 antibodies. Patients who were double-positive for anti-CENP and anti-IFI-16 antibodies were more likely to have had digital pits, ischemic digital ulcers, and/or gangrene (P = 0.03). After adjustment for clinically relevant covariates (age, cutaneous subtype, disease duration, and smoking), double-positive patients remained at significantly higher odds of having severe Raynaud's phenomenon (odds ratio 3.5 [95% confidence interval 1.1-11.1]; P = 0.03). CONCLUSION: Scleroderma patients who are double-positive for antibodies recognizing CENP and IFI-16 are significantly more likely to have significant digital vascular events during the course of their disease. This study provides further evidence that anti-CENP and anti-IFI-16 antibodies are disease biomarkers that may be used for risk stratification of vascular events in scleroderma. CI - © 2016, American College of Rheumatology. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Wigley, Frederick M AU - Wigley FM AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. LA - eng GR - R01 AR044684/AR/NIAMS NIH HHS/United States GR - R56 AR062615/AR/NIAMS NIH HHS/United States GR - P30 AR053503/AR/NIAMS NIH HHS/United States GR - R01 DE012354/DE/NIDCR NIH HHS/United States GR - L30 AR065225/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Nuclear Proteins) RN - 0 (Phosphoproteins) RN - 0 (anticentromere antibody) RN - 148998-64-5 (IFI16 protein, human) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/*blood MH - Autoantibodies/*blood MH - Biomarkers/blood MH - Cohort Studies MH - Female MH - Fingers/*blood supply/pathology MH - Humans MH - Male MH - Middle Aged MH - Nuclear Proteins/*blood MH - Phosphoproteins/*blood MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Localized/*blood/*diagnosis MH - Young Adult PMC - PMC5219877 MID - NIHMS800736 COIS- Conflicts of interest: None of the authors has received any financial support or other benefits from commercial sources for the work reported in this manuscript, nor do any of the other authors have any financial interests which could create a potential conflict of interest, or the appearance thereof. EDAT- 2016/07/09 06:00 MHDA- 2017/08/17 06:00 PMCR- 2018/06/01 CRDT- 2016/07/09 06:00 PHST- 2016/03/04 00:00 [received] PHST- 2016/05/31 00:00 [revised] PHST- 2016/06/28 00:00 [accepted] PHST- 2016/07/09 06:00 [pubmed] PHST- 2017/08/17 06:00 [medline] PHST- 2016/07/09 06:00 [entrez] PHST- 2018/06/01 00:00 [pmc-release] AID - 10.1002/acr.22978 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2017 Jun;69(6):922-926. doi: 10.1002/acr.22978. PMID- 24692361 OWN - NLM STAT- MEDLINE DCOM- 20141223 LR - 20211021 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 66 IP - 11 DP - 2014 Nov TI - Correlates and responsiveness to change of measures of skin and musculoskeletal disease in early diffuse systemic sclerosis. PG - 1731-9 LID - 10.1002/acr.22339 [doi] AB - OBJECTIVE: Skin and musculoskeletal involvement are frequently present early in diffuse cutaneous systemic sclerosis (dcSSc). The current study examined the correlates for skin and musculoskeletal measures in a 1-year longitudinal observational study. METHODS: Patients with dcSSc were recruited at 4 US centers and enrolled in a 1-year study. Prespecified and standardized measures included physician and patient assessments of skin involvement, modified Rodnan skin score (MRSS), durometer score, Health Assessment Questionnaire disability index, serum creatine phosphokinase, tender joint counts, and presence/absence of tendon friction rubs, small joint contractures, and large joint contractures. Additionally, physician and patient global health assessments and health-related quality of life assessments were recorded. Correlations were computed among the baseline global assessments, skin variables, and musculoskeletal variables. Using the followup physician and patient anchors, effect sizes were calculated. RESULTS: A total of 200 patients were studied: 75% were women, mean ± SD age was 50.0 ± 11.9 years, and mean ± SD disease duration from first non-Raynaud's phenomenon symptom was 1.6 ± 1.4 years. Physician global health assessment had large correlations with MRSS (r = 0.60) and physician-reported skin involvement visual analog scale in the last month (r = 0.74), whereas patient global assessment had large correlations with MRSS, the Short Form 36 health survey physical component scale, skin interference, and skin involvement in the last month (r = 0.37-0.72). Four of 9 skin variables had moderate to large effect sizes (0.51-1.09). CONCLUSION: Physician and patient global assessments have larger correlations with skin measures compared to musculoskeletal measures. From a clinical trial perspective, skin variables were more responsive to change than musculoskeletal variables over a 1-year period, although both provide complementary information. CI - Copyright © 2014 by the American College of Rheumatology. FAU - Wiese, Alexandra B AU - Wiese AB AD - University of Michigan, Ann Arbor. FAU - Berrocal, Veronica J AU - Berrocal VJ FAU - Furst, Daniel E AU - Furst DE FAU - Seibold, James R AU - Seibold JR FAU - Merkel, Peter A AU - Merkel PA FAU - Mayes, Maureen D AU - Mayes MD FAU - Khanna, Dinesh AU - Khanna D LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - U01 AR055057/AR/NIAMS NIH HHS/United States GR - U01-AR-055057/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Observational Study PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - EC 2.7.3.2 (Creatine Kinase) SB - IM MH - Adult MH - Creatine Kinase/blood MH - Disability Evaluation MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Joints/physiopathology MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Musculoskeletal Diseases/*etiology/*physiopathology MH - Outcome Assessment, Health Care/*methods MH - Quality of Life MH - Scleroderma, Diffuse/*complications/*physiopathology MH - Skin/physiopathology MH - Skin Diseases/*etiology/*physiopathology MH - Surveys and Questionnaires MH - Tendons/physiopathology MH - Visual Analog Scale PMC - PMC4511097 MID - NIHMS703962 EDAT- 2014/04/03 06:00 MHDA- 2014/12/24 06:00 PMCR- 2015/07/22 CRDT- 2014/04/03 06:00 PHST- 2013/09/26 00:00 [received] PHST- 2014/03/25 00:00 [accepted] PHST- 2014/04/03 06:00 [entrez] PHST- 2014/04/03 06:00 [pubmed] PHST- 2014/12/24 06:00 [medline] PHST- 2015/07/22 00:00 [pmc-release] AID - 10.1002/acr.22339 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2014 Nov;66(11):1731-9. doi: 10.1002/acr.22339. PMID- 41036232 OWN - NLM STAT- Publisher LR - 20251004 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) DP - 2025 Sep 29 TI - Factors associated with eHealth literacy among people with systemic sclerosis: A Scleroderma Patient-centred Intervention Network (SPIN) Cohort cross-sectional study. PG - 23971983251376428 LID - 10.1177/23971983251376428 [doi] LID - 23971983251376428 AB - INTRODUCTION/OBJECTIVE: eHealth literacy reflects the ability to obtain, understand, and evaluate health information from electronic sources and apply this information to health problems. Our objective was to evaluate sociodemographic (age, sex, race or ethnicity, education, marital status, country, residence location) and disease factors (duration, subtype) associations with eHealth literacy among individuals with systemic sclerosis (SSc). METHODS: Scleroderma Patient-centred Intervention Network (SPIN) Cohort participants completed the 8-item eHealth Literacy Scale (eHEALS) from January 17 to February 18, 2025. Multivariable linear regression was used to assess associations of sociodemographic and disease characteristics with eHealth literacy. RESULTS: The 333 participants were from France (N = 116, 35%), Canada (N = 90, 27%), the United States (N = 85, 26%), the United Kingdom (N = 32, 10%), and Australia, Mexico, or Spain (N = 10, 3%). Most participants were female (N = 295, 89%), White (N = 268, 80%), and had limited SSc (N = 206, 62%). Compared to the United States, participants from Canada (-2.2 points, 95% CI -4.2 to -0.1; standardized mean difference (SMD) = -0.33) and France (-4.2 points, 95% CI -6.2 to -2.3; SMD = -0.64) had significantly lower eHEALS scores. Age, sex, race or ethnicity, marital status, education level, time since first non-Raynaud's symptom onset, and disease subtype were not associated with eHEALS scores. CONCLUSION: eHealth literacy in SSc was not associated with age and education level as in some other studies but was associated with country. Future research should examine country-level differences in eHealth literacy for individuals with SSc. CI - © The Author(s) 2025. FAU - Co, Natalie AU - Co N AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychology, McGill University, Montreal, QC, Canada. FAU - Adams, Claire AU - Adams C AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychiatry, McGill University, Montreal, QC, Canada. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Golberg, Meira AU - Golberg M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Nassar, Elsa-Lynn AU - Nassar EL AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychology, McGill University, Montreal, QC, Canada. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, The Netherlands. AD - Department of IQ Health, Radboud University Medical Center, Nijmegen, The Netherlands. AD - Radboudumc Center for Mindfulness, Department of Psychiatry, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Bartlett, Susan J AU - Bartlett SJ AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Research Institute of the McGill University Health Centre, Montreal, QC, Canada. FAU - Fortuné, Catherine AU - Fortuné C AD - Ottawa Scleroderma Support Group, Ottawa, ON, Canada. FAU - Gietzen, Amy AU - Gietzen A AD - Steffens Scleroderma Foundation, Albany, NY, USA. FAU - Guillot, Geneviève AU - Guillot G AD - Sclérodermie Québec, Longueuil, QC, Canada. FAU - Lawrie-Jones, Amanda AU - Lawrie-Jones A AD - Scleroderma Australia, Melbourne, VIC, Australia. AD - Scleroderma Victoria, Melbourne, VIC, Australia. FAU - Malcarne, Vanessa L AU - Malcarne VL AD - Department of Psychology, San Diego State University, San Diego, CA, USA. AD - San Diego State University/University of California, San Diego Joint Doctoral Program in Clinical Psychology, San Diego, CA, USA. FAU - Mayes, Maureen D AU - Mayes MD AD - University of Texas McGovern School of Medicine, Houston, TX, USA. FAU - Richard, Michelle AU - Richard M AD - Scleroderma Atlantic, Halifax, NS, Canada. FAU - Mouthon, Luc AU - Mouthon L AD - Service de Médecine Interne, Centre de Référence Maladies Autoimmunes et Autoinflammatoires Systémiques Rares d'Ile de France, de l'Est et de l'Ouest, Hôpital Cochin, Paris, France. AD - Assistance Publique Hôpitaux de Paris-Centre, Hôpital Cochin, Université Paris Cité, Paris, France. FAU - Benedetti, Andrea AU - Benedetti A AD - Research Institute of the McGill University Health Centre, Montreal, QC, Canada. AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada. AD - Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, QC, Canada. FAU - Thombs, Brett D AU - Thombs BD AUID- ORCID: 0000-0002-5644-8432 AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychiatry, McGill University, Montreal, QC, Canada. AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada. LA - eng PT - Journal Article DEP - 20250929 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC12479466 OTO - NOTNLM OT - Cross-sectional study OT - eHealth literacy OT - health literacy OT - systemic sclerosis COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/10/02 06:28 MHDA- 2025/10/02 06:28 PMCR- 2025/09/29 CRDT- 2025/10/02 04:56 PHST- 2025/06/03 00:00 [received] PHST- 2025/08/21 00:00 [accepted] PHST- 2025/10/02 06:28 [medline] PHST- 2025/10/02 06:28 [pubmed] PHST- 2025/10/02 04:56 [entrez] PHST- 2025/09/29 00:00 [pmc-release] AID - 10.1177_23971983251376428 [pii] AID - 10.1177/23971983251376428 [doi] PST - aheadofprint SO - J Scleroderma Relat Disord. 2025 Sep 29:23971983251376428. doi: 10.1177/23971983251376428. PMID- 38622599 OWN - NLM STAT- MEDLINE DCOM- 20240417 LR - 20240501 IS - 1471-2466 (Electronic) IS - 1471-2466 (Linking) VI - 24 IP - 1 DP - 2024 Apr 15 TI - Anti-synthase syndrome associated with SARS-Cov-2 infection. PG - 179 LID - 10.1186/s12890-024-02966-2 [doi] LID - 179 AB - BACKGROUND: Anti-synthetase syndrome (AS) is a rare autoimmune idiopathic inflammatory myopathy (IIM) with diverse manifestations, including arthritis, interstitial lung disease (ILD), Raynaud's phenomenon, unexplained persistent fever, and mechanic's hands. CASE PRESENTATION: We present the case of a 72-year-old woman, previously healthy, who was admitted to our hospital for treatment of cough and rapid breathing. The patient had elevated white blood cells and C-reactive protein, and tested negative for severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2). She was initially diagnosed with community-acquired pneumonia and received tamoxifen for anti-infection treatment, but her dystonia worsened. She eventually required non-invasive ventilator support, tested positive for SARS-Cov-2 again, and started antiviral therapy, corticosteroids to reduce alveolar effusion, anticoagulation, and other treatments. However, her condition continued to deteriorate, with the lowest oxygenation index reaching only 80mmHg. Ultimately, she underwent tracheal intubation and mechanical ventilation. Chest CT revealed rapid progressive interstitial changes in her lungs, and her hands showed noticeable fraternization changes. At this point, we suspected that the novel coronavirus infection might be associated with autoimmune diseases. The patient's autoimmune antibody spectrum showed positive results for anti-recombinant RO-52 antibody and myositis-specific antibody anti-alanyl tRNA synthetase (anti-PL-12). The patient was treated with dexamethasone sodium phosphate for anti-inflammatory and anti-fibrotic effects. After successful extubation, the patient was discharged with only oral prednisone tablets at a dose of 30 mg. CONCLUSIONS: This case presents an early diagnosis and successful treatment of anti-synthetase syndrome combined with SARS-Cov-2 infection, emphasizing the importance of comprehensive physical examination. Additionally, it highlights the rapid progression of interstitial lung disease under SARS-Cov-2 infection, which is often difficult to distinguish on imaging. In cases where treatment for SARS-Cov-2 infection is ineffective, early screening for autoimmune diseases is recommended. As there is currently no standardized method for treating AS-ILD, the successful treatment of this case provides a reference for clinical research on anti-synthetase syndrome in the later stage. CI - © 2024. The Author(s). FAU - Chen, Xing-Yue AU - Chen XY AD - Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610,032, China. FAU - Chen, Jun AU - Chen J AD - Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610,032, China. FAU - Zhi, Li-Jia AU - Zhi LJ AD - Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610,032, China. FAU - Long, Kun-Lan AU - Long KL AD - Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610,032, China. FAU - Gao, Pei-Yang AU - Gao PY AD - Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610,032, China. gaopy930@126.com. LA - eng PT - Case Reports PT - Journal Article DEP - 20240415 PL - England TA - BMC Pulm Med JT - BMC pulmonary medicine JID - 100968563 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Female MH - Aged MH - *COVID-19/complications MH - SARS-CoV-2 MH - *Myositis/complications/diagnosis/drug therapy MH - *Lung Diseases, Interstitial/complications/diagnosis/drug therapy MH - *Autoimmune Diseases/complications MH - Autoantibodies PMC - PMC11020169 OTO - NOTNLM OT - Anti-alanyl tRNA synthetase OT - Anti-synthase syndrome OT - Autoimmune disease OT - Interstitial lung disease OT - SARS-Cov-2 infection COIS- The authors declare no competing interests. EDAT- 2024/04/16 00:42 MHDA- 2024/04/17 06:42 PMCR- 2024/04/15 CRDT- 2024/04/15 23:49 PHST- 2023/12/07 00:00 [received] PHST- 2024/03/13 00:00 [accepted] PHST- 2024/04/17 06:42 [medline] PHST- 2024/04/16 00:42 [pubmed] PHST- 2024/04/15 23:49 [entrez] PHST- 2024/04/15 00:00 [pmc-release] AID - 10.1186/s12890-024-02966-2 [pii] AID - 2966 [pii] AID - 10.1186/s12890-024-02966-2 [doi] PST - epublish SO - BMC Pulm Med. 2024 Apr 15;24(1):179. doi: 10.1186/s12890-024-02966-2. PMID- 41670330 OWN - NLM STAT- Publisher LR - 20260211 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) DP - 2026 Feb 11 TI - Can Baseline Features Predict Progression to Defined CTD? Insights from a Minimum 5-Year Follow-Up Study of 504 Patients with UCTD. LID - roag006 [pii] LID - 10.1093/mr/roag006 [doi] AB - OBJECTIVES: Undifferentiated connective tissue disease (UCTD) represents a systemic autoimmune condition characterized by clinical and serological features suggestive of defined connective tissue diseases (CTDs), yet insufficient to fulfill existing classification criteria. Despite increasing interest, long-term outcomes-particularly progression to defined CTDs-remain incompletely understood. This study aimed to evaluate the clinical outcomes of a UCTD cohort followed for at least 5 years and to identify baseline clinical and serological factors associated with an increased risk of evolution to a defined CTD. METHODS: A total of 658 patients were screened for this retrospective cohort study. After applying predefined eligibility criteria, 504 patients who met established UCTD definitions were included. Baseline clinical, laboratory, and serological characteristics were analyzed. Two patient outcome groups were defined at 5-year follow-up: those who evolved to a defined CTD and those who remained stable. Given the limited number of cases evolving to defined CTDs, group comparisons were performed using Student's t-test or Mann-Whitney U test rather than logistic regression. Autoantibodies were assessed using indirect immunofluorescence for ANA and validated immunoassays for anti-dsDNA, anti-Sm, anti-Ro/SS-A, anti-La/SS-B, anti-Scl-70, anti-centromere, anti-U1RNP, anti-Jo1, rheumatoid factor, anti-cyclic citrullinated peptide antibodies (ACPA), antiphospholipid antibodies (anti-β2 glycoprotein I IgM/IgG, anti-cardiolipin IgM/IgG), and lupus anticoagulant. RESULTS: After a mean follow-up of 82 months, 102 of the 504 UCTD patients (20.2%) developed a defined CTD (37 Sjögren's disease (SjD), 35 systemic lupus erythematosus (SLE), 12 systemic sclerosis (SSc), 14 rheumatoid arthritis, and 4 mixed connective tissue disease) within a follow-up period of at least 5 years. The most common clinical manifestations of UCTD included arthralgias, sicca symptoms, photosensitivity, oral aphthae, and Raynaud's phenomenon. Differentiation was significantly associated with features such as a Schirmer test <5 mm (p=0.007), anti-dsDNA (p=0.001), anti-Ro/SS-A (p<0.001), and hypocomplementemia (p=0.004). Multivariate analysis identified several disease-specific predictors, including anti-Ro/SS-A for SjD and anti-dsDNA and hypocomplementemia for SLE. CONCLUSIONS: Approximately one-fifth of UCTD patients progressed to a defined connective tissue disease during long-term follow-up, most frequently to SjD or SLE. Anti-Ro/SS-A, anti-dsDNA, and a Schirmer test <5 mm were significant predictors of progression. CI - © Japan College of Rheumatology 2026. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Apaydin, Hakan AU - Apaydin H AUID- ORCID: 0000-0001-7219-1457 AD - Ankara Etlik City Hospital, Clinical of Rheumatology, Ankara, Turkey. FAU - Çoşkun Sağirkaya, Şerife AU - Çoşkun Sağirkaya Ş AUID- ORCID: 0000-0001-7244-5488 AD - Ankara Bilkent City Hospital, Clinical of Rheumatology, Ankara, Turkey. FAU - Polat, Bünyamin AU - Polat B AUID- ORCID: 0000-0003-4972-8142 AD - Ankara Bilkent City Hospital, Clinical of Rheumatology, Ankara, Turkey. FAU - Armağan, Berkan AU - Armağan B AUID- ORCID: 0000-0003-4409-059X AD - Ankara Bilkent City Hospital, Clinical of Rheumatology, Ankara, Turkey. FAU - Omma, Ahmet AU - Omma A AUID- ORCID: 0000-0003-2582-7445 AD - University of Health Sciences, Clinical of Rheumatology, Ankara, Turkey. FAU - Erten, Şükran AU - Erten Ş AUID- ORCID: 0000-0003-0717-8365 AD - Yıldırım Beyazıt University Medical School, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Küçükşahin, Orhan AU - Küçükşahin O AUID- ORCID: 0000-0002-4109-0642 AD - Yıldırım Beyazıt University Medical School, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. FAU - Güven, Serdar Can AU - Güven SC AUID- ORCID: 0000-0003-1965-9756 AD - Ankara Bilkent City Hospital, Clinical of Rheumatology, Ankara, Turkey. LA - eng PT - Journal Article DEP - 20260211 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM OTO - NOTNLM OT - antinuclear antibodies OT - autoantibodies OT - evolution OT - undifferentiated connective tissue disease EDAT- 2026/02/11 13:07 MHDA- 2026/02/11 13:07 CRDT- 2026/02/11 08:53 PHST- 2025/10/06 00:00 [received] PHST- 2025/11/22 00:00 [revised] PHST- 2026/01/09 00:00 [accepted] PHST- 2026/02/11 13:07 [medline] PHST- 2026/02/11 13:07 [pubmed] PHST- 2026/02/11 08:53 [entrez] AID - 8472599 [pii] AID - 10.1093/mr/roag006 [doi] PST - aheadofprint SO - Mod Rheumatol. 2026 Feb 11:roag006. doi: 10.1093/mr/roag006. PMID- 40072585 OWN - NLM STAT- MEDLINE DCOM- 20250312 LR - 20250529 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 45 IP - 4 DP - 2025 Mar 12 TI - Characteristics and factors associated with treatment response among patients with eosinophilic fasciitis: a systematic review and meta-analysis. PG - 71 LID - 10.1007/s00296-025-05826-2 [doi] AB - BACKGROUND/OBJECTIVES: Eosinophilic Fasciitis (EF) is a rare connective tissue disorder characterized by skin thickening. Few studies explored its characteristics and factors associated with treatment response. METHODS: PubMed, CENTRAL, Web of Sciences, and Scopus databases were searched in July 2023 to identify all published case reports of EF without restrictions on publication year or language. We extracted patients' demographics, clinical symptoms, laboratory findings, biopsy results and treatment outcomes. RESULTS: We analyzed 476 published case reports with an aggregate number of 597 patients. The mean age of the patients was 44.52, with a 1:1 male-to-female ratio. Comorbid autoimmune diseases were present in 13.3% of patients. The most common reported skin manifestation was induration (80.7%), with the most commonly affected body part being the legs (77.1%). Only 1.6% of patients had Raynaud's phenomena and 3.1% of patients had Dysphagia. Hypereosinophilia was present in 90.2% and hypergammaglobulinemia was present in 65.9%. Corticosteroids were received by 89.7% of patients. The majority of patients (82.2%) exhibited a clinical response to treatment, but 24.5% of patients who initially responded to treatment relapsed. Patients who received oral corticosteroid monotherapy were significantly more likely to respond to treatment compared to those who received other treatments (56.2% Vs. 39.4%). Patients who had forearm skin involvement (OR = 3.459; 95% CI: 1.334-8.966) had significantly higher odds of clinical response to treatment. CONCLUSIONS: EF is a complex disease with non-specific symptoms. Our study offers comprehensive insights into patient characteristics and treatment outcomes, aiding clinicians in enhancing their approach. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Hamdan, Omar AU - Hamdan O AUID- ORCID: 0009-0008-0442-0578 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Alshajrawi, Roa'a AU - Alshajrawi R AUID- ORCID: 0000-0003-3619-7645 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Mussa, Qais AU - Mussa Q AUID- ORCID: 0009-0006-7350-6143 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Alajlouni, Yazeed AU - Alajlouni Y AUID- ORCID: 0009-0004-1079-5633 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Dabbah, Yazan AU - Dabbah Y AUID- ORCID: 0009-0000-1958-4796 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Fratekh, Rawan AU - Fratekh R AUID- ORCID: 0009-0005-6371-4750 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Al-Mabrouk, Yousef AU - Al-Mabrouk Y AUID- ORCID: 0009-0007-8045-2048 AD - School of Medicine, Mansoura University, Almansoura, 35516, Egypt. FAU - Al-Mabrok, Shatha AU - Al-Mabrok S AUID- ORCID: 0009-0009-6231-368X AD - School of Medicine, Mansoura University, Almansoura, 35516, Egypt. FAU - Toubasi, Ahmad A AU - Toubasi AA AUID- ORCID: 0000-0002-4688-9728 AD - School of Medicine, University of Jordan, Amman, 19942, Jordan. FAU - Alnaimat, Fatima AU - Alnaimat F AUID- ORCID: 0000-0002-5574-2939 AD - Department of Internal Medicine, Division of Rheumatology, School of Medicine, University of Jordan, Amman, 11942, Jordan. f.naimat@ju.edu.jo. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20250312 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Adrenal Cortex Hormones) RN - Eosinophilic Fasciitis SB - IM MH - Humans MH - *Fasciitis/drug therapy MH - *Eosinophilia/drug therapy MH - *Adrenal Cortex Hormones/therapeutic use MH - Treatment Outcome MH - Female MH - Male OTO - NOTNLM OT - Eosinophilic fasciitis OT - Fasciitis OT - Fasciitis with diffuse eosinophilia OT - Shulman syndrome OT - Shulman’s disease COIS- Declarations. Conflict of interest: All authors declare that there are no conflicts of interest. Patient consent for publication: Not required. Disclaimer: No part of this manuscript, including the text and graphics, is copied or published elsewhere in whole or in part. Grammarly ( https://app.grammarly.com ) was utilized to enhance the grammar and clarity of this manuscript. Open-access publishing funding: None. EDAT- 2025/03/12 18:17 MHDA- 2025/03/12 18:18 CRDT- 2025/03/12 12:16 PHST- 2024/12/30 00:00 [received] PHST- 2025/02/28 00:00 [accepted] PHST- 2025/03/12 18:18 [medline] PHST- 2025/03/12 18:17 [pubmed] PHST- 2025/03/12 12:16 [entrez] AID - 10.1007/s00296-025-05826-2 [pii] AID - 10.1007/s00296-025-05826-2 [doi] PST - epublish SO - Rheumatol Int. 2025 Mar 12;45(4):71. doi: 10.1007/s00296-025-05826-2. PMID- 40806935 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250817 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 15 DP - 2025 Jul 28 TI - Evaluation of Nailfold Capillaroscopy as a Novel Tool in the Assessment of Eosinophilic Granulomatosis with Polyangiitis. LID - 10.3390/jcm14155311 [doi] LID - 5311 AB - Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), represent a spectrum of systemic disorders characterized by necrotizing inflammation of small- to medium-sized vessels. Nailfold videocapillaroscopy (NVC) is a validated, non-invasive technique routinely employed in the assessment of microvascular involvement in systemic sclerosis and in the differential diagnosis of Raynaud's phenomenon; its application in the context of AAV, particularly EGPA, has not been investigated yet. The present study aims to assess the presence and the possible pattern of microcirculatory abnormalities detected by NVC in EGPA patients, and to explore potential correlations between capillaroscopic findings and disease activity status. Methods: A total of 29 patients with EGPA (19 women and 10 men), aged between 51 and 73 years, and 29 age- and sex-matched healthy controls were retrospectively enrolled between October 2023 and April 2025, after providing informed consent and meeting the inclusion and exclusion criteria. NVC was conducted in both groups to assess various morphological parameters, and mean capillary density was also calculated. Results: This study observed the presence of capillaroscopic alterations in the EGPA group, including decreased capillary density (38%), neoangiogenesis (72%), rolling (100%), pericapillary stippling (66%), and inverted capillary apex (52%). Overall, when comparing healthy controls with EGPA patients, microcirculatory abnormalities were significantly more prevalent in the latter. Specifically, scores for neoangiogenesis, capillary rolling, pericapillary stippling, and inverted capillary apex showed p-values < 0.001. Conclusions: Our study demonstrates a higher prevalence of four nailfold videocapillaroscopic abnormalities in patients with EGPA compared to healthy controls. However, the identification of these capillaroscopic alterations as specific to EGPA requires further confirmation. Ongoing studies aim to explore the potential role of NVC as a diagnostic marker and to investigate its correlation with the clinical manifestations of EGPA. FAU - Screm, Gianluca AU - Screm G AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Gandin, Ilaria AU - Gandin I AD - Biostatistics Unit, Department of Medical Sciences, University of Trieste, 34149 Trieste, Italy. FAU - Mondini, Lucrezia AU - Mondini L AUID- ORCID: 0000-0002-7043-9639 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Cifaldi, Rossella AU - Cifaldi R AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Confalonieri, Paola AU - Confalonieri P AUID- ORCID: 0000-0002-7284-5016 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Bozzi, Chiara AU - Bozzi C AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Salton, Francesco AU - Salton F AUID- ORCID: 0000-0002-0864-9766 AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Bandini, Giulia AU - Bandini G AUID- ORCID: 0000-0002-2076-7319 AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine, Azienda Ospedaliero Universitaria Careggi, University of Florence, 50134 Florence, Italy. FAU - Monteleone, Giorgio AU - Monteleone G AD - Department of Cardiovascular and Pulmonary Sciences, Catholic University of Sacred Heart, 00168 Rome, Italy. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK. FAU - Cameli, Paolo AU - Cameli P AUID- ORCID: 0000-0001-8639-2882 AD - Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Novello, Marileda AU - Novello M AD - Pulmonology Unit, Ospedale di Gorizia e Mondalcone-SC Patologie Respiratorie, 34170 Gorizia, Italy. FAU - Della Porta, Rossana AU - Della Porta R AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Pietro, Geri AU - Pietro G AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Confalonieri, Marco AU - Confalonieri M AUID- ORCID: 0000-0002-4791-768X AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Pulmonology Unit, Department of Medical Surgical and Health Sciences, Hospital of Cattinara, University of Trieste, 34149 Trieste, Italy. LA - eng PT - Journal Article DEP - 20250728 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC12347172 OTO - NOTNLM OT - ANCA-associated vasculitis OT - EGPA OT - autoimmune disease OT - microvascular abnormality OT - nailfold video capillaroscopy COIS- The authors declare no conflicts of interest. EDAT- 2025/08/14 06:27 MHDA- 2025/08/14 06:28 PMCR- 2025/07/28 CRDT- 2025/08/14 01:15 PHST- 2025/06/29 00:00 [received] PHST- 2025/07/23 00:00 [revised] PHST- 2025/07/25 00:00 [accepted] PHST- 2025/08/14 06:28 [medline] PHST- 2025/08/14 06:27 [pubmed] PHST- 2025/08/14 01:15 [entrez] PHST- 2025/07/28 00:00 [pmc-release] AID - jcm14155311 [pii] AID - jcm-14-05311 [pii] AID - 10.3390/jcm14155311 [doi] PST - epublish SO - J Clin Med. 2025 Jul 28;14(15):5311. doi: 10.3390/jcm14155311. PMID- 42143732 OWN - NLM STAT- MEDLINE DCOM- 20260608 LR - 20260608 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 65 IP - 6 DP - 2026 Jun 3 TI - Early gastrointestinal manifestations predict disease progression and mortality in patients with systemic sclerosis. LID - keag257 [pii] LID - 10.1093/rheumatology/keag257 [doi] AB - OBJECTIVE: Gastrointestinal (GI) complications are common in systemic sclerosis (SSc), yet the value of early symptoms as predictors of progression remains poorly defined. We aimed to determine whether baseline GI symptoms in early SSc predict progression to significant GI outcomes. METHODS: We studied 450 participants from the GENISOS cohort with early SSc (≤5 years from first non-Raynaud's symptom). Baseline assessments included demographics and clinical variables, including individual GI symptoms (gastroesophageal reflux disease [GERD], dysphagia, bloating, constipation, diarrhoea, peptic ulcer). Significant GI involvement was defined as Medsger GI severity score ≥2. Cox models evaluated associations between baseline symptoms and progression to significant GI disease and all-cause mortality. Additional models examined total baseline GI symptom burden and outcomes. RESULTS: Participants were predominantly female (84%) with a mean age of 47.7 (13.3) years. Baseline GI symptoms were frequent, most commonly GERD (76%) and dysphagia (41%). Thirteen per cent of the patients had significant GI involvement, with 4% presenting with significant GI disease at baseline. Baseline GERD (hazard ratio [HR] 4.15, P = 0.019) and diarrhoea (HR 2.25, P = 0.021) were strong predictors of GI progression. When symptom burden was analysed, each additional baseline GI symptom increased the hazard of significant GI progression by 53% (P = 0.003). On the other hand, peptic ulcer disease (HR 2.21, P = 0.007) and diarrhoea (HR 1.48, P = 0.045) predicted higher mortality, with each additional symptom raising mortality risk by 29% (P < 0.001). CONCLUSION: Early GI symptoms and overall symptom burden predict GI progression and mortality in SSc. Symptom-based profiling may offer a practical strategy for clinical risk stratification and trial enrichment. CI - Published by Oxford University Press on behalf of the British Society for Rheumatology 2026. FAU - Ayla, Ali Y AU - Ayla AY AUID- ORCID: 0000-0003-2581-7992 AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Balar, Ashish P AU - Balar AP AUID- ORCID: 0000-0001-6241-926X AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Calderon Martinez, Ernesto AU - Calderon Martinez E AD - Department of Internal Medicine, Division of Medical Genetics, UTHealth Houston, Houston, TX, USA. FAU - Chen, Bingrui AU - Chen B AD - Department of Pediatrics, The Institute for Clinical Research and Learning Health Care, McGovern Medical School, UTHealth Houston, Houston, TX, USA. FAU - Pedroza, Claudia AU - Pedroza C AD - Department of Pediatrics, The Institute for Clinical Research and Learning Health Care, McGovern Medical School, UTHealth Houston, Houston, TX, USA. FAU - Bonomi, Francesco AU - Bonomi F AUID- ORCID: 0000-0001-7744-8549 AD - Department of Internal Medicine, Careggi University Hospital, Florence, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Division of Rheumatology, University of Florence, Florence, Italy. FAU - Mayes, Maureen D AU - Mayes MD AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Skaug, Brian AU - Skaug B AUID- ORCID: 0000-0002-6689-4655 AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - Hughes, Michael AU - Hughes M AD - Division of Musculoskeletal and Dermatological Science, School of Biological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organization, Salford, UK. AD - NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester, UK. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AUID- ORCID: 0000-0001-6461-8940 AD - Department of Internal Medicine, Division of Rheumatology, UTHealth Houston, Houston, TX, USA. LA - eng GR - R01 AR081382/AR/NIAMS NIH HHS/United States GR - K23 AR071473/AR/NIAMS NIH HHS/United States GR - K08AR081402/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) of NIH/ GR - R01AR081280/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) of NIH/ GR - 892808/Rheumatology Research Foundation/ GR - W81XWH-22-1-0162/U.S. Department of Defence (DoD)/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM UOF - medRxiv. 2026 Feb 10:2025.12.01.25341383. doi: 10.64898/2025.12.01.25341383. PMID: 41409661 MH - Humans MH - *Scleroderma, Systemic/complications/mortality MH - Female MH - Disease Progression MH - Middle Aged MH - *Gastrointestinal Diseases/etiology/mortality/diagnosis MH - Male MH - Adult MH - Severity of Illness Index MH - Gastroesophageal Reflux/etiology MH - Prognosis MH - Deglutition Disorders/etiology MH - Symptom Burden OTO - NOTNLM OT - gastrointestinal symptoms OT - prediction OT - risk stratification OT - systemic sclerosis EDAT- 2026/05/17 18:37 MHDA- 2026/06/09 00:34 CRDT- 2026/05/17 12:43 PHST- 2026/01/17 00:00 [received] PHST- 2026/04/16 00:00 [accepted] PHST- 2026/06/09 00:34 [medline] PHST- 2026/05/17 18:37 [pubmed] PHST- 2026/05/17 12:43 [entrez] AID - 8681596 [pii] AID - 10.1093/rheumatology/keag257 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 Jun 3;65(6):keag257. doi: 10.1093/rheumatology/keag257. PMID- 35386942 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240825 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 7 IP - 1 DP - 2022 Feb TI - Quality of life in SSc-ILD patients: Understanding the impact of the ILD and the needs of the SSc-ILD patients and their need for caregivers in France. PG - 49-56 LID - 10.1177/23971983211013979 [doi] AB - OBJECTIVES: The objectives of this study were to describe the impact of systemic sclerosis associated interstitial lung disease, on quality of life, to estimate the correlation between quality of life and severity of lung disease and to assess the impact of interstitial lung disease on caregivers. METHODS: Seven investigators included systemic sclerosis associated interstitial lung disease patients from December 2019 to April 2020. Sociodemographics and clinical data were collected. Patients reported outcomes and questionnaires were used with 1 generic patients reported outcome (EQ-5D-5L), 1 specific PRO (Brief Interstitial Lung Disease) and 2 self-reported questionnaires on impact of SSc complications and impact on caregivers. The correlation between forced vital capacity and EQ-5D-5L score was estimated with a multivariate linear regression model adjusted on several covariates. RESULTS: In all, 89 patients were included. 26.4% were males, mean age was 58.2 ± 14.5 years. Mean EQ-5D-5L score = 0.79 ± 0.22 (median = 0.85). Mean EQ-5D-5L visual analog scale score = 60.8 ± 20.4 (median = 61.5). Mean King's Brief Interstitial Lung Disease score = 58.4 ± 12.7 (median = 58.0). After adjustment on covariates, a significant correlation between forced vital capacity and EQ-5D-5L score was found with an increase of 0.003 of the EQ-5D-5L score for a 1% increase of FVC (p = 0.0096). No significant correlation between forced vital capacity and the EQ-VAS and King's Brief Interstitial Lung Disease score were found. The impact of SSc on other organs was significantly correlated with EQ- 5D-5L score, respectively, for the impact scores on the lung system (p = 0.0003), heart system (p = 0.0182), Raynaud's syndrome (p = 0.0015), digestive system (p = 0.0032), joints/muscles (p = 0.0003), skin (p < 0.0001), kidney (p = 0.0052) and gastro-oesophageal reflux (p = 0.0063). Significant correlations between King's Brief Interstitial Lung Disease score and lung system (p < 0.0001), heart system (p < 0.0001), digital ulcers (p = 0.058), digestive system (p < 0.0001), kidney (p = 0.0004), skin (p = 0.0499) and gastro-oesophageal reflux (p = 0.0033) scores were found 68.5% of patients reported their need for a caregiver to help them in their daily life activities. CONCLUSION: Our study highlighted the strong burden of systemic sclerosis associated interstitial lung disease` for patients, especially with an impact on quality of life, on other organs manifestations and need for caregivers in their daily life. CI - © The Author(s) 2021. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology, Cochin Hospital, Université de Paris, Paris, France. FAU - Constans, Joel AU - Constans J AD - Vascular Medicine, CHU Bordeaux, Bordeaux, France. FAU - Godard, Dominique AU - Godard D AD - Association des Sclerodermiques de France, Baccon, France. FAU - de Pouvourville, Gerard AU - de Pouvourville G AD - ESSEC Business School, Cergy Pontoise, France. FAU - Bouee, Stephane AU - Bouee S AUID- ORCID: 0000-0001-6838-2680 AD - Real World Evidence, CEMKA, Bourg La Reine, France. FAU - Jeanbat, Viviane AU - Jeanbat V AD - Real World Evidence, CEMKA, Bourg La Reine, France. FAU - Teissier, Clement AU - Teissier C AD - Real World Evidence, CEMKA, Bourg La Reine, France. FAU - Le Lay, Katell AU - Le Lay K AD - Boehringer Ingelheim, Paris, France. FAU - Chollet, Julien AU - Chollet J AD - Boehringer Ingelheim, Paris, France. FAU - Hachulla, Eric AU - Hachulla E AD - Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne et Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France. LA - eng PT - Journal Article DEP - 20210621 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922678 OTO - NOTNLM OT - Systemic sclerosis OT - interstitial lung disease OT - quality of life COIS- Declaration of conflicting interests: The Editor/ Editorial Board Member of JSRD is an author of this paper, therefore, the peer review process was managed by alternative members of the Board and the submitting Editor/Board member had no involvement in the decision-making process. Disclosure: The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE). The authors did not receive payment for the development of the manuscript. Writing, editorial support, and formatting assistance was provided by Stéphane Bouée, MD, of CEMKA, which was contracted and funded by BI. BI was given the opportunity to review the manuscript for medical and scientific accuracy. EDAT- 2022/04/08 06:00 MHDA- 2022/04/08 06:01 PMCR- 2023/02/01 CRDT- 2022/04/07 05:19 PHST- 2021/01/07 00:00 [received] PHST- 2021/04/03 00:00 [accepted] PHST- 2022/04/07 05:19 [entrez] PHST- 2022/04/08 06:00 [pubmed] PHST- 2022/04/08 06:01 [medline] PHST- 2023/02/01 00:00 [pmc-release] AID - 10.1177_23971983211013979 [pii] AID - 10.1177/23971983211013979 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2022 Feb;7(1):49-56. doi: 10.1177/23971983211013979. Epub 2021 Jun 21. PMID- 35382144 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240825 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 4 IP - 1 DP - 2019 Feb TI - Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? Clinical characteristics of a juvenile systemic sclerosis cohort. PG - 49-61 LID - 10.1177/2397198318790494 [doi] AB - INTRODUCTION: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. METHODS: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. Clinical characteristics and patient-related outcomes were assessed. RESULTS: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. The mean age at the onset of Raynaud's and first non-Raynaud's symptoms was similar in both groups, approximately 9 and 10.5 years. Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). DISCUSSION: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis. CI - © The Author(s) 2018. FAU - Foeldvari, Ivan AU - Foeldvari I AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Klotsche, Jens AU - Klotsche J AD - German Rheumatism Research Center, Berlin, Germany. FAU - Torok, Kathryn S AU - Torok KS AD - Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. FAU - Kasapcopur, Ozgur AU - Kasapcopur O AD - Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. FAU - Adrovic, Amra AU - Adrovic A AD - Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey. FAU - Stanevicha, Valda AU - Stanevicha V AD - University Children's Hospital, Riga, Latvia. FAU - Terreri, Maria Teresa AU - Terreri MT AD - Universidade Federal de São Paulo, Sao Paulo, Brazil. FAU - Alexeeva, Ekaterina AU - Alexeeva E AD - Scientific Center of Children's Health, Moskva, Russia. FAU - Katsicas, Maria AU - Katsicas M AD - Hospital de Pediatria, Buenos Aires, Argentine. FAU - Cimaz, Rolando AU - Cimaz R AD - Meyer Children's Hospital, Florence, Italy. FAU - Kostik, Mikhail AU - Kostik M AD - Federal State Autonomous Institution "National Medical Research Center of Children's Health" of the Ministry of Health of the Russian Federation, Moscow, Russia. FAU - Lehman, Thomas AU - Lehman T AD - Hospital for Special Surgery, New York, NY, USA. FAU - Sifuentes-Giraldo, Walter-Alberto AU - Sifuentes-Giraldo WA AD - University Hospital Ramón y Cajal, Madrid, Spain. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Sztajnbok, Flavio AU - Sztajnbok F AD - Universidade do Estado, Rio de Janeiro, Brazil. FAU - Avcin, Tadej AU - Avcin T AD - University Children's Hospital Ljubljana, Ljubljana, Slovenia. FAU - Jose Santos, Maria AU - Jose Santos M AD - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal. FAU - Moll, Monika AU - Moll M AD - Pediatric Rheumatology, University Tübingen, Tübingen, Germany. FAU - Nemcova, Dana AU - Nemcova D AD - Department of Pediatrics and Adolescent Medicine, University Childrens Hospital, Prague, Czech Republic. FAU - Battagliotti, Cristina AU - Battagliotti C AD - Hospital de Niños Dr. Orlando Alassia, Santa Fee, Argentine. FAU - Eleftheriou, Despina AU - Eleftheriou D AD - Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. FAU - Janarthanan, Mahesh AU - Janarthanan M AD - Pediatric Rheumatology, Sri Ramachandra University, Chennai, India. FAU - Kallinich, Tilmann AU - Kallinich T AD - Division of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany. FAU - Anton, Jordi AU - Anton J AD - Hospital Sant Joan de Déu Barcelona, Barcelona, Spain. FAU - Minden, Kirsten AU - Minden K AD - German Rheumatism Research Center, Berlin, Germany. AD - Division of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany. FAU - Nielsen, Susan AU - Nielsen S AD - Rigshospitalet, Copenhagen, Denmark. FAU - Uziel, Yosef AU - Uziel Y AD - Meir Medical Center, Tel Aviv University, Kfar Saba, Israel. FAU - Helmus, Nicola AU - Helmus N AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany. LA - eng PT - Journal Article DEP - 20180807 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC8922583 OTO - NOTNLM OT - Juvenile scleroderma OT - diffuse cutaneous subset OT - juvenile systemic sclerosis OT - limited cutaneous subset OT - organ involvement OT - patient-related outcomes COIS- Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2019/02/01 00:00 MHDA- 2019/02/01 00:01 PMCR- 2020/02/01 CRDT- 2022/04/06 05:12 PHST- 2018/03/16 00:00 [received] PHST- 2018/07/01 00:00 [accepted] PHST- 2022/04/06 05:12 [entrez] PHST- 2019/02/01 00:00 [pubmed] PHST- 2019/02/01 00:01 [medline] PHST- 2020/02/01 00:00 [pmc-release] AID - 10.1177_2397198318790494 [pii] AID - 10.1177/2397198318790494 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2019 Feb;4(1):49-61. doi: 10.1177/2397198318790494. Epub 2018 Aug 7. PMID- 40326264 OWN - NLM STAT- MEDLINE DCOM- 20250506 LR - 20260127 IS - 2772-4336 (Electronic) IS - 2772-4328 (Linking) VI - 20 IP - 2 DP - 2025 TI - Interferon Causes Endothelial Injury in Humans. PG - 122-139 LID - 10.2174/0127724328322183240922153629 [doi] AB - Therapy with exogenous interferon and human conditions that feature endogenous interferon upregulation may be associated with endothelial damage that primarily involves small blood vessels. Endothelial injury associated with interferon may display different clinical expression, including thrombotic microangiopathy, Raynaud's phenomenon, vasculopathy of dermatomyositis and atrophic papulosis, interferon-associated skin angiopathy, systemic capillary leak syndrome, collapsing glomerulopathy, interstitial lung disease, pulmonary hypertension, and retinopathy. Interferon- induced endothelial damage involves complement-mediated injury, although pathogenic mechanisms by which interferon promote abnormal complement activation on endothelial cells are not fully understood. Human interferon-γ (type II interferon) binds to heparan sulfate on the endothelial surface, suggesting that overproduction of interferon-γ may hinder factor H attachment to the same location. Absence of factor H on self surfaces promotes activation of the alternative pathway of complement and complement-mediated endothelial damage. Type I interferon typically induces the generation of antibodies. Type I interferon upregulation may elicit the formation of autoantibodies against factor H. These autoantibodies block factor H binding to endothelial surfaces, abolishing the protective effect of factor H on complement-mediated damage. In addition, interferon induces insulin resistance which is associated with reduced heparan sulfate in the extracellular matrix, including the endothelial surface. Decreased amount of heparan sulfate suppresses factor H attachment, promoting activation of the alternative pathway of complement. Complement blockade with eculizumab (a monoclonal antibody against C5) improves endothelial damage in patients with thrombotic microangiopathy and other situations associated with interferon upregulation and interferon-induced endothelial injury, suggesting that complement-mediated injury is clinically relevant under conditions that feature interferon overproduction. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Adeva-Andany, Maria M AU - Adeva-Andany MM AUID- ORCID: 0000-0002-9997-2568 AD - Department of Internal Medicine, Hospital General Juan Cardona, c/ Pardo Bazán, s/n, Ferrol 15406, Spain. FAU - Adeva-Contreras, Lucía AU - Adeva-Contreras L AD - School of Medicine, Santiago de Compostela University, Santiago de Compostela, Spain. FAU - Fernandez-Fernandez, Carlos AU - Fernandez-Fernandez C AD - Department of Internal Medicine, Hospital General Juan Cardona, c/ Pardo Bazán, s/n, Ferrol 15406, Spain. FAU - Vila-Altesor, Matilde AU - Vila-Altesor M AD - Department of Internal Medicine, Hospital General Juan Cardona, c/ Pardo Bazán, s/n, Ferrol 15406, Spain. FAU - Castro-Quintela, Elvira AU - Castro-Quintela E AD - Department of Internal Medicine, Hospital General Juan Cardona, c/ Pardo Bazán, s/n, Ferrol 15406, Spain. FAU - Funcasta-Calderon, Raquel AU - Funcasta-Calderon R AD - Department of Internal Medicine, Hospital General Juan Cardona, c/ Pardo Bazán, s/n, Ferrol 15406, Spain. LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Curr Rev Clin Exp Pharmacol JT - Current reviews in clinical and experimental pharmacology JID - 9918227368306676 RN - 9008-11-1 (Interferons) RN - 9050-30-0 (Heparan Sulfate) RN - 80295-65-4 (Complement Factor H) SB - IM MH - Humans MH - *Interferons/adverse effects MH - *Endothelium, Vascular/drug effects/pathology/immunology/metabolism MH - Animals MH - Heparan Sulfate/metabolism MH - Complement Activation/drug effects MH - Complement Factor H/immunology OTO - NOTNLM OT - Thrombotic microangiopathy OT - atrophic papulosis OT - complement. OT - dermatomyositis OT - lupus erythematosus OT - monoclonal gammopathy OT - systemic capillary leak syndrome EDAT- 2025/05/06 06:29 MHDA- 2025/05/06 12:42 CRDT- 2025/05/06 04:23 PHST- 2024/05/07 00:00 [received] PHST- 2024/08/04 00:00 [revised] PHST- 2024/08/08 00:00 [accepted] PHST- 2025/05/06 12:42 [medline] PHST- 2025/05/06 06:29 [pubmed] PHST- 2025/05/06 04:23 [entrez] AID - CRCEP-EPUB-143644 [pii] AID - 10.2174/0127724328322183240922153629 [doi] PST - ppublish SO - Curr Rev Clin Exp Pharmacol. 2025;20(2):122-139. doi: 10.2174/0127724328322183240922153629. PMID- 40046231 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250307 IS - 2296-634X (Print) IS - 2296-634X (Electronic) IS - 2296-634X (Linking) VI - 13 DP - 2025 TI - Cell-free regenerative medicine: identifying the best source of mesenchymal stem cells for skin therapy in Systemic Sclerosis. PG - 1518412 LID - 10.3389/fcell.2025.1518412 [doi] LID - 1518412 AB - INTRODUCTION: Systemic Sclerosis (SSc) is a rare chronic systemic autoimmune disease characterized by fibrosis of the skin and internal organs and vasculopathy. Raynaud's phenomenon is typically the earliest clinical manifestation accompanied by skin inflammation, finger ulcers, and organ manifestations, including pulmonary fibrosis. There is an urgent need for the development of effective targeted therapeutic intervention for SSc patients. A greater focus has been placed on bioactive factors secreted by Mesenchymal Stem Cells (MSCs), with immunomodulatory and regenerative potentials. Current data report a different secretion profile of MSCs, depending on the tissue of origin. Understanding of the secretion profile of different MSCs is necessary to identify the most efficient and useful source for SSc treatment. METHODS: We analyzed the content of MSC-conditioned media (MSC-CM) obtained from MSCs isolated from adipose tissue (AT), bone marrow (BM), Wharton's jelly (WJ), and cord blood (CB) by ELISA method, and their effects on the wound healing process by fibroblast proliferation, migration, and ECM deposition assays, to compare regenerative potential of different MSC populations. RESULTS: WJ-MSC-conditioned medium (CM) and BM-MSC-CM show a greater regenerative profile, compared to CB-MSC-CM and AT-MSC-CM, due to the abundance of growth factors and immunomodulatory cytokines and the effects on fibroblast functions. In SSc fibroblasts, WJ-MSC-CM significantly promotes fibroblast-mediated wound healing processes and VEGF expression, compared to BM-MSC-CM. DISCUSSION: Our data indicate that WJ-MSC-CM could be considered an appealing strategy to both topical and systemic administrations in SSc patients. CI - Copyright © 2025 Napolitano, Giudice, D’Esposito, Prevete, Scala, de Paulis, Selleri, Formisano, Rossi and Montuori. FAU - Napolitano, Filomena AU - Napolitano F AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Giudice, Valentina AU - Giudice V AD - Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy. AD - Hematology and Transplant Center, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", Salerno, Italy. FAU - D'Esposito, Vittoria AU - D'Esposito V AD - Institute for Experimental Endocrinology and Oncology "G. Salvatore" - National Research Council (IEOS - CNR), Naples, Italy. FAU - Prevete, Nella AU - Prevete N AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Scala, Pasqualina AU - Scala P AD - Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy. FAU - de Paulis, Amato AU - de Paulis A AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. FAU - Selleri, Carmine AU - Selleri C AD - Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy. AD - Hematology and Transplant Center, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", Salerno, Italy. FAU - Formisano, Pietro AU - Formisano P AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. FAU - Rossi, Francesca Wanda AU - Rossi FW AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. FAU - Montuori, Nunzia AU - Montuori N AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. LA - eng PT - Journal Article DEP - 20250219 PL - Switzerland TA - Front Cell Dev Biol JT - Frontiers in cell and developmental biology JID - 101630250 PMC - PMC11880212 OTO - NOTNLM OT - SSc fibroblasts OT - Wharton’s jelly OT - adipose tissue OT - bone marrow OT - mesenchymal stem cells OT - secretome OT - umbilical cord COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2025/03/06 06:21 MHDA- 2025/03/06 06:22 PMCR- 2025/01/01 CRDT- 2025/03/06 04:38 PHST- 2024/10/28 00:00 [received] PHST- 2025/01/23 00:00 [accepted] PHST- 2025/03/06 06:22 [medline] PHST- 2025/03/06 06:21 [pubmed] PHST- 2025/03/06 04:38 [entrez] PHST- 2025/01/01 00:00 [pmc-release] AID - 1518412 [pii] AID - 10.3389/fcell.2025.1518412 [doi] PST - epublish SO - Front Cell Dev Biol. 2025 Feb 19;13:1518412. doi: 10.3389/fcell.2025.1518412. eCollection 2025. PMID- 38282933 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241023 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 34 IP - 4 DP - 2023 Dec TI - Late Onset Systemic Lupus Erythematosus - Clinical and Autoantibody Profile and its Comparison with Young Onset Systemic Lupus Erythematosus. PG - 454-459 LID - 10.31138/mjr.290723.los [doi] AB - OBJECTIVES: The aim of the study was to determine the clinical features & autoantibody profile of patients having late onset Systemic Lupus Erythematosus (SLE) and to compare with young onset SLE due to its scarce data from India. METHODS: All patients who fulfilled the 1997 ACR criteria for SLE were included. Late onset patients were >50 years of age and young onset were <50 years >18 years at the time of first SLE-related symptom. Clinical, laboratory, and autoantibody (ENA 25 & APLA) profiles were compared between the two groups using descriptive statistics and chi square test. RESULTS: Of the 305 patients, 69 had late onset (75.4% females). Mean age was 59.42±6.7 years (Late onset lupus) and 33.13±8.44 years (young onset lupus). The most common symptom was arthritis (60%) followed by oral ulceration (50%), fever (43%), and serositis (37.68%). Most common antibody was SSA/Ro60 (50%) and anti-SSA/Ro52 (46%). Interstitial lung disease (ILD) (14.5%), pancytopenia (13%) and diffuse alveolar haemorrhage (4.3%) were more frequent in late onset group. Statistically significant differences were found between two groups in terms of photosensitivity (p=0.009), malar rash (p=0.005), excessive hair loss (p=0.0006), Raynaud's phenomenon (p=0.001), lymphadenopathy (p=0.01), nephritis (p=0.0007), ILD (p=0.01), anti-dsDNA (p=0.005), anti-nucleosome (p=0.01), anti-Sm (p=0.007), Ribosomes P0 (p=0.0004). CONCLUSION: This study suggests that late onset SLE has distinct clinical and serological manifestations when compared with young onset SLE patients. CI - © 2023 The Mediterranean Journal of Rheumatology (MJR). FAU - Bindroo, Muzaffar Ahmad AU - Bindroo MA AD - Department of Rheumatology, SKIMS Soura Srinagar, India. FAU - Majid, Nahida AU - Majid N AD - Department of Immunology and Molecular Medicine, SKIMS Soura Srinagar, India. FAU - Ekbote, Gayatri AU - Ekbote G AD - Deenanath Mangeshkar Hospital, Sassoon Gen Hospital Pune, India. FAU - Raval, Dhiren AU - Raval D AD - Kd Hospital, Ahmedabad Gujrat, India. FAU - Negalur, Natasha Vijay AU - Negalur NV AD - Synergy Rheumatology and Arthritis Clinic, India. FAU - Mendiratta, Naval AU - Mendiratta N AD - Fortis Hospital Gurgaon, India. FAU - Bajad, Shruti AU - Bajad S AD - Moolchand Hospital Delhi, India. FAU - Gupta, Rajiva AU - Gupta R AD - Medanta-The Medicity Gurgaon, India. LA - eng PT - Journal Article DEP - 20230729 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC10815541 OTO - NOTNLM OT - Systemic Lupus Erythematosus OT - autoantibodies profile OT - late onset lupus OT - profile of SLE OT - young onset lupus COIS- The authors declare no conflict of interest. EDAT- 2024/01/29 06:44 MHDA- 2024/01/29 06:45 PMCR- 2023/07/29 CRDT- 2024/01/29 04:10 PHST- 2022/11/14 00:00 [received] PHST- 2023/01/14 00:00 [revised] PHST- 2023/01/26 00:00 [accepted] PHST- 2024/01/29 06:45 [medline] PHST- 2024/01/29 06:44 [pubmed] PHST- 2024/01/29 04:10 [entrez] PHST- 2023/07/29 00:00 [pmc-release] AID - MJR-34-4-454 [pii] AID - 10.31138/mjr.290723.los [doi] PST - epublish SO - Mediterr J Rheumatol. 2023 Jul 29;34(4):454-459. doi: 10.31138/mjr.290723.los. eCollection 2023 Dec. PMID- 32442284 OWN - NLM STAT- MEDLINE DCOM- 20210126 LR - 20210126 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 59 IP - 12 DP - 2020 Dec 1 TI - Rituximab for rapidly progressive juvenile systemic sclerosis. PG - 3793-3797 LID - 10.1093/rheumatology/keaa193 [doi] AB - OBJECTIVE: Juvenile systemic sclerosis (JSSc) with rapidly progressive course is a life-threatening condition associated with a poor prognosis. Recently, rituximab (RTX) has been shown to be a promising treatment for adult patients with SSc. We present a series of four patients with rapidly progressive JSSc successfully treated with RTX. METHODS: Clinical, laboratory and functional parameters were collected from four patients with rapidly progressive JSSc treated with RTX for at least 1 year. All patients underwent four yearly courses of i.v. RTX 375 mg/m2 on day 0 and 14, at 3-month intervals. Low dose oral prednisone and MMF were also administered. Data were recorded at baseline and every 6 months and included pulmonary and myocardial function parameters, muscular, vascular and skin changes. The Juvenile Systemic Sclerosis Severity Score (J4S) estimated the overall disease severity over time. RESULTS: Four patients (three males, one female), aged 8-17 years, entered the study. Three patients presented with prevalent cardiac involvement, one with severe pulmonary involvement. After 1 year of RTX treatment, all patients showed significant improvement of J4S, Raynaud's phenomenon and cutaneous involvement. Among those with prevalent cardiac involvement, two showed an improvement of the myocardial function (left ventricular ejection fraction [EF] +37% and +19%, respectively) and in the third arrhythmias disappeared. The patient with severe pulmonary involvement showed a significant improvement of the respiratory function (forced vital capacity +46%, forced expiratory volume in 1 s +33%, diffusing capacity of the lung for carbon monoxide [DLCO] +30%). No major side effects were reported. CONCLUSIONS: Our data suggest that a combination of RTX and MMF is effective in arresting the rapid progression of JSSc. CI - © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Zulian, Francesco AU - Zulian F AD - Department of Woman's and Child's Health. FAU - Dal Pozzolo, Roberto AU - Dal Pozzolo R AD - Department of Woman's and Child's Health. FAU - Meneghel, Alessandra AU - Meneghel A AD - Department of Woman's and Child's Health. FAU - Castaldi, Biagio AU - Castaldi B AD - Department of Woman's and Child's Health. FAU - Marcolongo, Renzo AU - Marcolongo R AD - Clinical Immunology, Department of Medicine. FAU - Caforio, Alida Linda Patrizia AU - Caforio ALP AD - Department of Cardiological, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy. FAU - Martini, Giorgia AU - Martini G AD - Department of Woman's and Child's Health. LA - eng PT - Journal Article PT - Observational Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antirheumatic Agents) RN - 4F4X42SYQ6 (Rituximab) RN - Juvenile systemic scleroderma SB - IM MH - Adolescent MH - Antirheumatic Agents/*therapeutic use MH - Child MH - Female MH - Humans MH - Male MH - Rituximab/*therapeutic use MH - Scleroderma, Systemic/*drug therapy OTO - NOTNLM OT - juvenile systemic sclerosis OT - rituximab OT - scleroderma EDAT- 2020/05/23 06:00 MHDA- 2021/01/27 06:00 CRDT- 2020/05/23 06:00 PHST- 2020/02/18 00:00 [received] PHST- 2020/03/26 00:00 [revised] PHST- 2020/05/23 06:00 [pubmed] PHST- 2021/01/27 06:00 [medline] PHST- 2020/05/23 06:00 [entrez] AID - 5842179 [pii] AID - 10.1093/rheumatology/keaa193 [doi] PST - ppublish SO - Rheumatology (Oxford). 2020 Dec 1;59(12):3793-3797. doi: 10.1093/rheumatology/keaa193. PMID- 19578107 OWN - NLM STAT- MEDLINE DCOM- 20091022 LR - 20221207 IS - 0961-2033 (Print) IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 18 IP - 9 DP - 2009 Aug TI - Time to neuropsychiatric damage occurrence in LUMINA (LXVI): a multi-ethnic lupus cohort. PG - 822-30 LID - 10.1177/0961203309104392 [doi] AB - The aims of this study were to examine the predictors of time to neuropsychiatric (NP) damage and its impact on mortality in 632 systemic lupus erythematosus African-American, Hispanic and Caucasian LUpus in MInorities: NAture versus Nurture (LUMINA) patients, age >or= 16 years and disease duration  18 were associated with contracture of fingers. Furthermore, hand MRSS > 4 was associated with digital ulcers. CONCLUSION: Our results confirm that dcSSc patients had more severe clinical hand complications than lcSSc. However, radiographic findings were similar among subgroups, except that more finger contractures were seen in dcSSc. Finally, the presence of rheumatoid factor is associated with arthritis, and high MRSS is associated with finger contractures and digital ulcers. CI - © 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Wangkaew, Suparaporn AU - Wangkaew S AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Sivasomboon, Chate AU - Sivasomboon C AD - Division of Diagnostic Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Leungwatthananon, Wit AU - Leungwatthananon W AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Kasitanon, Nuntana AU - Kasitanon N AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. FAU - Louthrenoo, Worawit AU - Louthrenoo W AD - Division of Rheumatology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand. LA - eng PT - Journal Article PT - Observational Study DEP - 20160726 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Arthritis/diagnostic imaging/*epidemiology MH - Chi-Square Distribution MH - Contracture/diagnostic imaging/*epidemiology MH - Cross-Sectional Studies MH - Female MH - Hand/*diagnostic imaging MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Prevalence MH - Prospective Studies MH - Scleroderma, Diffuse/diagnostic imaging/*epidemiology MH - Scleroderma, Limited/diagnostic imaging/*epidemiology MH - Severity of Illness Index MH - Thailand/epidemiology MH - Ulcer/diagnostic imaging/*epidemiology OTO - NOTNLM OT - hand OT - predictors OT - prevalence OT - radiography OT - systemic sclerosis EDAT- 2016/07/28 06:00 MHDA- 2018/08/28 06:00 CRDT- 2016/07/27 06:00 PHST- 2016/07/28 06:00 [pubmed] PHST- 2018/08/28 06:00 [medline] PHST- 2016/07/27 06:00 [entrez] AID - 10.1111/1756-185X.12919 [doi] PST - ppublish SO - Int J Rheum Dis. 2018 Jan;21(1):240-248. doi: 10.1111/1756-185X.12919. Epub 2016 Jul 26. PMID- 22781510 OWN - NLM STAT- MEDLINE DCOM- 20140203 LR - 20120711 IS - 2184-8777 (Electronic) IS - 0303-464X (Linking) VI - 37 IP - 1 DP - 2012 Jan-Mar TI - [Clinical features of systemic sclerosis and association with antitopoisomerase-1 antibody and centromere pattern of antinuclear antibody]. PG - 9-17 AB - OBJECTIVE: To describe clinical features of patients with systemic sclerosis (SSc) and associate them with antitopoisomerase-1 antibody and centromere pattern of antinuclear antibody. METHODS: We evaluated 50 patients who met SSc screening criteria of the American College of Rheumatology, and classified as limited cutaneous SSc and diffuse cutaneous SSc. Clinical history, physical examination and laboratory tests were evaluated: cutaneous, vascular, gastrointestinal, renal, pulmonary and cardiac involvement, in addition to autoantibodies. The continuous quantitative variables, discrete quantitative and categorical variables were evaluated employing the relevant statistical tests. A P value ≤ 0.05 was considered statistically significant. RESULTS: The authors found that 88% of the patients were women, and 70% of the cases corresponded to the limited cutaneous form. The skin thickening assessed by the modified Rodnan skin score found as minimum of 3, and a maximum of 32, and median of 14. Raynaud's phenomenon was found in 100% of patients, skin ulcers in 56%, esophageal symptoms in 80%, interstitial lung disease in 44%, and increase in right ventricular systolic pressure in18% of the patients. Antinuclear antibodies were found in 80%, the centromere pattern in 34% and antitopoisomerase-1 in 22% of the patients. We found that 88.2% of patients with centromere pattern antinuclear antibody had limited cutaneous SSc, while 72.7% of patients with topoisomerase-1 antibodies had diffuse cutaneous SSc. CONCLUSION: The clinical features of this population of patients with SSc are similar to results in the literature, as well as the association with the autoantibodies evaluated. FAU - Bertazzi, Glausse Rejane Leonardi AU - Bertazzi GR AD - Serviço de Reumatologia da Faculdade de Medicina de São José do Rio Preto - FAMERP, SP-Brasil. glbertazzi@netsite.com.br FAU - de Toledo, Roberto Acayaba AU - de Toledo RA FAU - de Godoy, Moacir Fernandes AU - de Godoy MF FAU - Geraldino, Geise Cristina AU - Geraldino GC FAU - Polizelli, Daniela Vichiato AU - Polizelli DV FAU - Fernandes, Gisele Cristine Dyonisio AU - Fernandes GC FAU - Pedroso, Camila Lobo AU - Pedroso CL FAU - dos Santos, Fernando Gonçalves AU - dos Santos FG FAU - de Assis, Tássia Moraes AU - de Assis TM LA - por PT - English Abstract PT - Journal Article TT - Características clínicas da esclerose sistêmica e associação ao anticorpo antitopoisomerase-1 e padrão centrómero do anticorpo antinuclear. PL - Portugal TA - Acta Reumatol Port JT - Acta reumatologica portuguesa JID - 0431702 RN - 0 (Antibodies, Antinuclear) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 5.99.1.2 (TOP1 protein, human) SB - IM MH - Adult MH - Aged MH - *Antibodies, Antinuclear MH - *Centromere MH - DNA Topoisomerases, Type I/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*diagnosis/*immunology MH - Young Adult EDAT- 2012/07/12 06:00 MHDA- 2014/02/04 06:00 CRDT- 2012/07/12 06:00 PHST- 2012/07/12 06:00 [entrez] PHST- 2012/07/12 06:00 [pubmed] PHST- 2014/02/04 06:00 [medline] PST - ppublish SO - Acta Reumatol Port. 2012 Jan-Mar;37(1):9-17. PMID- 19116537 OWN - NLM STAT- MEDLINE DCOM- 20090223 LR - 20210106 IS - 1529-4242 (Electronic) IS - 0032-1052 (Linking) VI - 123 IP - 1 DP - 2009 Jan TI - Prevention of anastomotic thrombosis by botulinum toxin a in an animal model. PG - 64-70 LID - 10.1097/PRS.0b013e3181904c31 [doi] AB - BACKGROUND: Free tissue transfer is used widely for reconstruction of complex defects throughout the body. The most common cause for free flap failure remains vascular thrombosis. Currently, there exists no animal model for anastomotic vasospasm. Botulinum toxin type A has been successfully used to treat vasospasm in Raynaud's phenomenon. A blinded, vasospasm animal model was designed to determine its ability to prevent anastomotic thrombosis. METHODS: Ten Sprague-Dawley-derived rats were pretreated with botulinum toxin type A subcutaneously to a randomly determined femoral vessel. Animals acted as their own controls, receiving saline to the contralateral limb. Five days postoperatively, femoral vessels were measured to determine the effect of neuromuscular blockade on diameter. Vessels were then divided and reanastomosed. Animals were subjected to a systemic treatment with a peripheral vasoconstrictor, phenylephrine, and a lower extremity thermic challenge in an ice bath. Vessel patency was recorded before cold challenge and 1 hour after. RESULTS: Vessel diameter was consistently larger in all neuromuscularly blocked vessels. The botulinum toxin type A-treated arterial average was significantly larger than the matched control average, and the venous average was significantly larger than the matched control average. Difficulty of anastomosis and time of suturing were significantly less in the pretreated botulinum toxin type A group. Patency was maintained in 100 percent of vessels treated with botulinum toxin type A and in 44 percent of saline-treated vessels at 1 hour after vasospastic challenge. CONCLUSIONS: Botulinum toxin type A was successful in preventing thrombosis within this model. Its ability to decrease vasospasm and thrombosis may have applications for improving free flap survival in select patients. FAU - Clemens, Mark W AU - Clemens MW AD - Washington, D.C.; and Baltimore, Md. From the Department of Plastic Surgery, Georgetown University Hospital, and the Curtis National Hand Center, Union Memorial Hospital. FAU - Higgins, James P AU - Higgins JP FAU - Wilgis, E F Shaw AU - Wilgis EFS LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Plast Reconstr Surg JT - Plastic and reconstructive surgery JID - 1306050 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - Anastomosis, Surgical MH - Animals MH - Botulinum Toxins, Type A/administration & dosage/*pharmacology MH - Disease Models, Animal MH - Neuromuscular Agents/administration & dosage/*pharmacology MH - Postoperative Complications/*prevention & control MH - Rats MH - Rats, Sprague-Dawley MH - Thrombosis/*etiology/*prevention & control EDAT- 2009/01/01 09:00 MHDA- 2009/02/24 09:00 CRDT- 2009/01/01 09:00 PHST- 2009/01/01 09:00 [entrez] PHST- 2009/01/01 09:00 [pubmed] PHST- 2009/02/24 09:00 [medline] AID - 00006534-200901000-00009 [pii] AID - 10.1097/PRS.0b013e3181904c31 [doi] PST - ppublish SO - Plast Reconstr Surg. 2009 Jan;123(1):64-70. doi: 10.1097/PRS.0b013e3181904c31. PMID- 37349709 OWN - NLM STAT- MEDLINE DCOM- 20230626 LR - 20231120 IS - 1471-2261 (Electronic) IS - 1471-2261 (Linking) VI - 23 IP - 1 DP - 2023 Jun 22 TI - Epipericardial fat necrosis in chest CT and MRI: a case report of an unusual cause of chest pain associated with the initial diagnosis of undifferentiated connective tissue disease. PG - 314 LID - 10.1186/s12872-023-03349-x [doi] LID - 314 AB - BACKGROUND: Epipericardial fat necrosis (EFN) is a benign and self-limited condition of unknown cause with a good prognosis, usually affecting otherwise healthy patients. Clinically, it presents with severe acute left pleuritic chest pain, often leading the patient to the Emergency Room (ER). CASE PRESENTATION: A 23-year-old male, smoker (5 pack-years), was evaluated in the ER due to left pleuritic chest pain, worsening with deep breathing and Valsalva maneuver. It was not associated with trauma and did not present other symptoms. The physical examination was unremarkable. The arterial blood gases while breathing room air and the laboratory tests, including D-dimers and high-sensitivity cardiac Troponin T, were normal. The chest radiograph, electrocardiogram, and transthoracic echocardiogram showed no abnormalities. A computed tomography (CT) pulmonary angiogram showed no signs of pulmonary embolism but depicted at the left cardiophrenic angle a focal 3 cm ovoid-shaped fat lesion with stranding and thin soft tissue margins, consistent with necrosis of the epicardial fat, which was confirmed by magnetic resonance (MRI) of the chest. The patient was medicated with ibuprofen and pantoprazole, with clinical improvement in four weeks. At a two-month follow-up, he was asymptomatic and presented radiologic resolution of the inflammatory changes of the epicardial fat of the left cardiophrenic angle on chest CT. Laboratory tests revealed positive antinuclear antibodies, positive anti-RNP antibody, and positive lupus anticoagulant. The patient complained of biphasic Raynaud's phenomenon initiated five years ago, and a diagnosis of undifferentiated connective tissue disease (UCTD) was made. CONCLUSIONS: This case report highlights the diagnosis of EFN as a rare and frequently unknown clinical condition, which should be considered in the differential diagnosis of acute chest pain. It can mimic emergent conditions such as pulmonary embolism, acute coronary syndrome, or acute pericarditis. The diagnosis is confirmed by CT of the thorax or MRI. The treatment is supportive and usually includes non-steroidal anti-inflammatory drugs. The association of EFN with UCTD has not been previously described in the medical literature. CI - © 2023. The Author(s). FAU - Barreto, Inês AU - Barreto I AUID- ORCID: 0000-0002-1127-4811 AD - Pulmonology Department, North Lisbon University Hospital Centre (CHULN), Lisbon Medical Academic Centre (CAML), Avenida Professor Egas Moniz 1649-035, Lisbon, Portugal. inesscbarreto@gmail.com. FAU - Oliveira, Francisca Godinho AU - Oliveira FG AD - Pulmonology Department, North Lisbon University Hospital Centre (CHULN), Lisbon Medical Academic Centre (CAML), Avenida Professor Egas Moniz 1649-035, Lisbon, Portugal. FAU - Barreira, Sofia Carvalho AU - Barreira SC AD - Rheumatology Department, North Lisbon University Hospital Centre (CHULN), Lisbon Medical Academic Centre (CAML), Lisbon, Portugal. AD - Rheumatology Research Unit, Institute of Molecular Medicine (IMM), Faculty of Medicine of the University of Lisbon, Lisbon Medical Academic Centre (CAML), Lisbon, Portugal. FAU - Inácio, João Rodrigues AU - Inácio JR AD - Radiology Department, North Lisbon University Hospital Centre (CHUNL), Lisbon Medical Academic Centre (CAML), Lisbon, Portugal. AD - Radiology Clinic, Faculty of Medicine of the University of Lisbon, Lisbon Medical Academic Centre (CAML), Lisbon, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20230622 PL - England TA - BMC Cardiovasc Disord JT - BMC cardiovascular disorders JID - 100968539 SB - IM MH - Male MH - Humans MH - Young Adult MH - Adult MH - *Fat Necrosis/complications/diagnosis MH - *Undifferentiated Connective Tissue Diseases/complications MH - Chest Pain/diagnostic imaging/etiology MH - Tomography, X-Ray Computed/adverse effects MH - Magnetic Resonance Imaging/adverse effects MH - Thorax MH - *Pulmonary Embolism/complications PMC - PMC10286368 OTO - NOTNLM OT - CT OT - Chest pain OT - Epipericardial fat necrosis OT - MRI OT - Undifferentiated connective tissue disease COIS- The authors declare that they have no competing interests. EDAT- 2023/06/23 01:10 MHDA- 2023/06/26 06:41 PMCR- 2023/06/22 CRDT- 2023/06/22 23:36 PHST- 2022/08/17 00:00 [received] PHST- 2023/06/13 00:00 [accepted] PHST- 2023/06/26 06:41 [medline] PHST- 2023/06/23 01:10 [pubmed] PHST- 2023/06/22 23:36 [entrez] PHST- 2023/06/22 00:00 [pmc-release] AID - 10.1186/s12872-023-03349-x [pii] AID - 3349 [pii] AID - 10.1186/s12872-023-03349-x [doi] PST - epublish SO - BMC Cardiovasc Disord. 2023 Jun 22;23(1):314. doi: 10.1186/s12872-023-03349-x. PMID- 36408259 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221122 IS - 1663-9812 (Print) IS - 1663-9812 (Electronic) IS - 1663-9812 (Linking) VI - 13 DP - 2022 TI - Changes in peripheral T-lymphocyte subsets and serum cytokines in patients with systemic sclerosis. PG - 986199 LID - 10.3389/fphar.2022.986199 [doi] LID - 986199 AB - Objective: T cells represent a predominant cell type in autoimmune disease. However, their exact roles are not fully clear in systemic sclerosis (SSc). This study aimed to mainly investigate the alteration in the absolute numbers of T-lymphocyte subsets and the serum levels of cytokines in SSc patients. Methods: A total of 76 patients with SSc and 76 age- and sex-matched healthy controls (HCs) were enrolled. The levels of circulating T cell subsets and serum cytokines were measured by flow cytometry. T cell subsets or serum cytokines correlations with disease activity and organ involvement were analyzed. Results: The absolute numbers of Th2 and Treg cells in SSc patients were lower than those in HCs (p < 0.05), resulting in the ratios of Th1/Th2 [25.01 (12.24, 38.61) vs. 11.64 (6.38, 20.34)] and Th17/Treg [0.42 (0.17, 0.66) vs. 0.17 (0.13, 0.29)] were increased significantly (p < 0.001). The absolute numbers of total T, Th, and Treg cells were negatively correlated with CRP (r = -0.406, p = 0.002; r = -0.263, p < 0.05; r = -0.367 p < 0.01). The serum levels of IL-2, SIL-2R, IL-6, IL-10, INF-γ, and TNF-α were significantly higher than those in HCs (p < 0.001). Increasing IL-2 in the wake of the augment of ESR (r = 0.671, p = 0.004), so did IL-6 (r = 0.378, p < 0.05). The ratio of Th17/Treg in SSc-ILD patients had lower levels than that in other patients [0.35 (0.14, 0.53) vs. 0.64 (0.26, 0.93) p = 0.028]; Treg cells were lessened in patients with Raynaud's phenomenon relative to controls [3.00 (2.41, 4.28) vs. 3.55 (2.86, 4.53) p < 0.05]. The levels of IL-2, IL-10 and INF-γ [3.32 (1.05,11.73) vs. 2.32 (0.44,6.45), p = 0.045], [8.08 (3.63, 355,77) vs. 4.89 (0.78, 21.44), p = 0.02], [6.31 (2.66, 44.03) vs. 4.03 (0.22, 16.96), p = 0.009] were elevated in patients with arthralgia, while the level of Th17 was decreased [0.62 (0.20,2.16) vs. 1.26 (0.22,10.93), p = 0.026]. ROC curve analysis yielded an optimal cut-off IL-2, IL-10, and INF-γ levels of 2.67, 5.93, and 5.32 pg/ml for the presence of arthralgia. Conclusion: We exhibited abnormalities in T subsets and the production of their cytokines in SSc, as compared with those in HCs. This may allow the pathogenesis of SSc and the development of novel therapeutic interventions aimed at targeting these cells and the cytokines they produce. CI - Copyright © 2022 Guo, Cheng, Zhang, Yu, Wang, Hao, Gao and Wen. FAU - Guo, Rong-Hong AU - Guo RH AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Cheng, Hao AU - Cheng H AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Zhang, Xiao-Ying AU - Zhang XY AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Yu, Zhen AU - Yu Z AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Wang, Guang-Hui AU - Wang GH AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Hao, Shu-Ya AU - Hao SY AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Gao, Xiao-Peng AU - Gao XP AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. FAU - Wen, Hong-Yan AU - Wen HY AD - Department of Rheumatology, Shanxi Medical University, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China. LA - eng PT - Journal Article DEP - 20221103 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC9669295 OTO - NOTNLM OT - T-lymphocyte subsets OT - arthralgia OT - cytokines OT - interstitial lung disease (ILD) OT - systemic sclerosis COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/11/22 06:00 MHDA- 2022/11/22 06:01 PMCR- 2022/11/03 CRDT- 2022/11/21 04:50 PHST- 2022/07/04 00:00 [received] PHST- 2022/10/21 00:00 [accepted] PHST- 2022/11/21 04:50 [entrez] PHST- 2022/11/22 06:00 [pubmed] PHST- 2022/11/22 06:01 [medline] PHST- 2022/11/03 00:00 [pmc-release] AID - 986199 [pii] AID - 10.3389/fphar.2022.986199 [doi] PST - epublish SO - Front Pharmacol. 2022 Nov 3;13:986199. doi: 10.3389/fphar.2022.986199. eCollection 2022. PMID- 29667101 OWN - NLM STAT- MEDLINE DCOM- 20190110 LR - 20190110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 8 DP - 2018 Aug TI - Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study. PG - 2125-2132 LID - 10.1007/s10067-018-4111-5 [doi] AB - To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint. Patients with IPAF were much common in young female and had lower percentage of ever smoking and a significantly shorter survival than those with non-IPAF (P < 0.001). Subgroup analysis revealed that IPAF cohort survival was worse than that in non-IPF (P < 0.001), but better than that in IPF (P < 0.001). In IPAF cohort, the most common systemic symptom and serological abnormality were Raynaud's phenomenon (12.9%) and ANA ≥ 1:320 (49.2%); the most frequent high-resolution computed tomography (HRCT) pattern was nonspecific interstitial pneumonia (NSIP) (61.6%). Multivariate analysis indicated that several factors including age, smoking history, organizing pneumonia (OP) pattern in HRCT, and anti-RNP positivity were independently associated with significantly worse survival. IPAF had the distinct clinical features and outcomes compared with other groups of ILD. Additional studies should be needed to explore the underlying autoimmune mechanism and to determine risk stratification in future clinical research. FAU - Dai, Jinghong AU - Dai J AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Wang, Lei AU - Wang L AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Yan, Xin AU - Yan X AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Li, Hui AU - Li H AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Zhou, Kefeng AU - Zhou K AD - Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - He, Jian AU - He J AD - Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - Meng, Fanqing AU - Meng F AD - Department of Pathology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - Xu, Siyi AU - Xu S AD - Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, 210009, Jiangsu, China. FAU - Liang, Geyu AU - Liang G AD - Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, 210009, Jiangsu, China. FAU - Cai, Hourong AU - Cai H AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. caihourong2013@163.com. LA - eng GR - 81570058/the National Natural Science Foundation of China/ GR - ZDRCA2016058/Jiangsu Provincial Medical Talent/ GR - BE2017604/Jiangsu Social Development Project/ PT - Journal Article DEP - 20180418 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Age Factors MH - Aged MH - *Autoimmune Diseases/blood/complications/drug therapy/pathology MH - Connective Tissue Diseases/pathology MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/blood/complications/drug therapy/pathology MH - Immunosuppressive Agents/therapeutic use MH - *Lung Diseases, Interstitial/blood/complications/drug therapy/pathology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Risk Factors MH - Sex Factors MH - Smoking/epidemiology MH - Tomography, X-Ray Computed MH - Treatment Outcome OTO - NOTNLM OT - Idiopathic interstitial pneumonia OT - Interstitial pneumonia with autoimmune features OT - Outcome OT - Risk factor OT - Undifferentiated connective tissue disease EDAT- 2018/04/19 06:00 MHDA- 2019/01/11 06:00 CRDT- 2018/04/19 06:00 PHST- 2018/01/19 00:00 [received] PHST- 2018/04/10 00:00 [accepted] PHST- 2018/03/21 00:00 [revised] PHST- 2018/04/19 06:00 [pubmed] PHST- 2019/01/11 06:00 [medline] PHST- 2018/04/19 06:00 [entrez] AID - 10.1007/s10067-018-4111-5 [pii] AID - 10.1007/s10067-018-4111-5 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Aug;37(8):2125-2132. doi: 10.1007/s10067-018-4111-5. Epub 2018 Apr 18. PMID- 35140136 OWN - NLM STAT- MEDLINE DCOM- 20220301 LR - 20220301 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 9 IP - 1 DP - 2022 Feb TI - Nailfold capillary scleroderma pattern may be associated with disease damage in childhood-onset systemic lupus erythematosus: important lessons from longitudinal follow-up. LID - 10.1136/lupus-2021-000572 [doi] LID - e000572 AB - OBJECTIVES: To observe if capillary patterns in childhood-onset SLE (cSLE) change over time and find associations between a capillary scleroderma pattern with disease activity, damage or scleroderma-like features. METHODS: Clinical and (yearly) capillaroscopy data from a longitudinal cohort of patients with cSLE (minimum of four Systemic Lupus International Collaborating Clinics (SLICC) criteria, onset <18 years) were analysed. Disease activity was measured by Systemic Lupus Erythematosus Activity Index (SLEDAI) and disease damage by SLICC Damage Index. A scleroderma pattern was defined according to the 'fast track algorithm' from the European League Against Rheumatism Study Group on Microcirculation in Rheumatic Diseases. An abnormal capillary pattern, not matching a scleroderma pattern, was defined as 'microangiopathy'. RESULTS: Our cohort consisted of 53 patients with cSLE with a median disease onset of 14 years (IQR 12.5-15.5 years), median SLEDAI score at diagnosis was 11 (IQR 8-16), median SLEDAI at follow-up was 2 (IQR 1-6). A scleroderma pattern (ever) was seen in 18.9%, while only 13.2% of patients had a normal capillary pattern. Thirty-three patients had follow-up capillaroscopy of which 21.2% showed changes in type of capillary pattern over time. Type of capillary pattern was not associated with disease activity. Raynaud's phenomenon (ever) was equally distributed among patients with different capillaroscopy patterns (p=0.26). Anti-ribonucleoprotein antibodies (ever) were significantly more detected (Χ(2), p=0.016) in the scleroderma pattern subgroup (n=7 of 10, 70%). Already 5 years after disease onset, more than 50% of patients with a scleroderma pattern had SLE-related disease damage (HR 4.5, 95% CI 1.1 to 18.8, p=0.034), but they did not develop clinical features of systemic sclerosis at follow-up. Number of detected fingers with a scleroderma pattern was similar between cSLE, juvenile systemic sclerosis and juvenile undifferentiated connective tissue disease. CONCLUSION: This longitudinal study shows that the majority of capillary patterns in cSLE are abnormal and they can change over time. Irrespective of disease activity, a capillary scleroderma pattern in cSLE may be associated with higher risk of SLE-related disease damage. CI - © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Schonenberg-Meinema, Dieneke AU - Schonenberg-Meinema D AUID- ORCID: 0000-0001-9416-6631 AD - Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands d.schonenberg@amsterdamumc.nl. FAU - Bergkamp, Sandy C AU - Bergkamp SC AD - Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands. FAU - Nassar-Sheikh Rashid, Amara AU - Nassar-Sheikh Rashid A AD - Department of Pediatrics, Zaans Medisch Centrum, Zaandam, The Netherlands. FAU - Gruppen, Mariken P AU - Gruppen MP AD - Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands. FAU - Middelkamp-Hup, Maritza A AU - Middelkamp-Hup MA AD - Department of Dermatology, University of Amsterdam, Amsterdam Universitair Medische Centra, Amsterdam, The Netherlands. FAU - Armbrust, Wineke AU - Armbrust W AD - University Medical Centre Groningen, University of Groningen, Department of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, Groningen, The Netherlands. FAU - Dolman, Koert AU - Dolman K AD - Department of Pediatrics, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands. AD - Department of Pediatric Rheumatology, Reade, Amsterdam, The Netherlands. FAU - Hak, A Elisabeth AU - Hak AE AD - Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam Rheumatology and Immunology Center, Amsterdam, The Netherlands. FAU - Hissink Muller, Petra C E AU - Hissink Muller PCE AD - Department of Paediatric Rheumatology, Willem Alexander Children's Hospital, Leiden University Medical Center, Leiden, The Netherlands. FAU - van Onna, Marieke AU - van Onna M AUID- ORCID: 0000-0002-9858-2429 AD - Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam Rheumatology and Immunology Center, Amsterdam, The Netherlands. FAU - Swart, Joost F AU - Swart JF AD - Department of Paediatric Immunology, Wilhelmina Children's Hospital, University of Utrecht, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Kuijpers, Taco W AU - Kuijpers TW AD - Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands. FAU - Kamphuis, Sylvia S M AU - Kamphuis SSM AD - Department of Paediatric Rheumatology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Faculty of Internal Medicine, Ghent University, Ghent, Belgium. FAU - van den Berg, J Merlijn AU - van den Berg JM AD - Department of Pediatric Immunology, Rheumatology and Infectious diseases, Emma Children's Hospital, Amsterdam University Medical Centres, University of Amsterdam, Amsterdam, The Netherlands. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 SB - IM MH - Follow-Up Studies MH - Humans MH - Longitudinal Studies MH - *Lupus Erythematosus, Systemic/complications/diagnosis/epidemiology MH - Microscopic Angioscopy MH - *Scleroderma, Systemic/complications/epidemiology PMC - PMC8830289 OTO - NOTNLM OT - cardiovascular diseases OT - lupus erythematosus OT - scleroderma OT - systemic COIS- Competing interests: VS is a Senior Clinical Investigator of the Research Foundation–Flanders (Belgium) (FWO) (1.8.029.20N). VS is supported by an unrestricted educational chair on systemic sclerosis of Janssen-Cilag NV. EDAT- 2022/02/11 06:00 MHDA- 2022/03/03 06:00 PMCR- 2022/02/09 CRDT- 2022/02/10 05:30 PHST- 2021/09/02 00:00 [received] PHST- 2022/01/24 00:00 [accepted] PHST- 2022/02/10 05:30 [entrez] PHST- 2022/02/11 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PHST- 2022/02/09 00:00 [pmc-release] AID - 9/1/e000572 [pii] AID - lupus-2021-000572 [pii] AID - 10.1136/lupus-2021-000572 [doi] PST - ppublish SO - Lupus Sci Med. 2022 Feb;9(1):e000572. doi: 10.1136/lupus-2021-000572. PMID- 38163807 OWN - NLM STAT- MEDLINE DCOM- 20240103 LR - 20240106 IS - 2408-8757 (Electronic) IS - 1022-4742 (Linking) VI - 33 IP - 1 DP - 2024 Jan TI - A Rare Case of Primary Sjogren's Syndrome with Idiopathic Pulmonary Fibrosis with Variable Presentations: A case report. PG - 298-302 AB - We diagnosed and treated a case of Primary SjoGren's Syndrome with Idiopathic Pulmonary Fibrosis (IPF) in a 65 years old woman who presented with dyspnoea and multiple joint pains for 5 years and remained undiagnosed. She had variable presentation and was initially established as a case of mixed connective tissue disease which consists of Systemic Lupus Erythematosus (SLE), Systemic Sclerosis and Dermatomyositis. She complained of xerostomia, xerophthalmia, difficulty in opening mouth, progressive dysphagia with solid foods and raynaud's phenomenon. In addition to this she noticed photosensitive rash, oral ulcers and difficulty in raising arms above head especially while combing hair. Examination revealed bi basal fine end inspiratory crepitations unaltered while coughing, bed side 6 minutes walking test showed exertional desaturation of SpO2 from 92.0% to 84.0%. Grade 2 tenderness was noted in wrists, knees, elbows and small joints of hands and feet except DIP. However, no oral lesions or dental carries were found. Unstimulated salivary flow rate was 1.0 ml in 15 minutes and sublingual salivary pool was significantly reduced. Schirmer's test was positive. HRCT lung revealed reticulonodular shadowing, honey combing and traction bronchiectasis in basal segments of both lobes, suggestive of usual interstitial pneumonia in both lungs. Auto antibody tests revealed ANA, RA, anti CCP and anti ds DNA negative, CPK was 63U/L. ENA (Extractable Nuclear Antigen) profile demonstrated positive Anti SS- A antibody while it remained insignificant for anti SS-B, anti RNP, anti Sm antibody, anti Scl-7o, anti Jo-1. According to the American-European Consensus Criteria for SjoGren's Syndrome, it meets all the criteria to be diagnosed as Primary Sjogren's Syndrome. We finally diagnosed a case of Primary SjoGren's syndrome with IPF and the patient was treated with pirfenidone, prednisolone, artificial tears and vaccination against Haemophilus influenzae and Streptococcal pneumoniae. The 10 year survival rate for such patients is nearly 80.0%. FAU - Yadav, A AU - Yadav A AD - Dr Ashmita Yadav, Doctoral Intern, Department of Medicine, Mymensingh Medical College (MMC), Mymensingh, Bangladesh; E-mail: ashmitayadav84@gmail.com. FAU - Nepali, R B AU - Nepali RB FAU - Alam, A M J AU - Alam AMJ LA - eng PT - Case Reports PT - Journal Article PL - Bangladesh TA - Mymensingh Med J JT - Mymensingh medical journal : MMJ JID - 9601799 SB - IM MH - Female MH - Humans MH - Aged MH - *Sjogren's Syndrome/complications/diagnosis MH - *Idiopathic Pulmonary Fibrosis/complications/diagnosis MH - Lung MH - *Lupus Erythematosus, Systemic EDAT- 2024/01/02 11:42 MHDA- 2024/01/03 09:41 CRDT- 2024/01/01 22:52 PHST- 2024/01/03 09:41 [medline] PHST- 2024/01/02 11:42 [pubmed] PHST- 2024/01/01 22:52 [entrez] PST - ppublish SO - Mymensingh Med J. 2024 Jan;33(1):298-302. PMID- 34534690 OWN - NLM STAT- MEDLINE DCOM- 20220406 LR - 20220406 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 89 IP - 1 DP - 2022 Jan TI - Joint involvement in Noonan syndrome. A retrospective paediatric descriptive study. PG - 105270 LID - S1297-319X(21)00143-3 [pii] LID - 10.1016/j.jbspin.2021.105270 [doi] AB - OBJECTIVES: Noonan syndrome is a rare genetic disorder characterized mainly by congenital heart disease, occasional intellectual disability, and varied orthopaedic, rheumatological and haematologic anomalies. Despite potentially serious functional consequences, joint involvement has been rarely studied in the literature. Our objective was to perform a retrospective study evaluating the prevalence and characteristics of joint involvement in Noonan syndrome. METHODS: We recorded articular symptoms, including their type and frequency, in patients with Noonan syndrome followed up in French hospitals. Patients were included if the diagnosis was confirmed before the age of 20 based on the van der Burgt criteria or genetic analysis. Data are presented as frequencies or medians (ranges), and patient groups were compared using chi-square or Fisher tests. RESULTS: Seventy-one patients were included from 4 centres. The average age was 12.5 years (range: 2-36). Musculoskeletal pain was found in 18 patients (25%) and joint stiffness in 10 (14%) located in the wrists, elbows, ankles, knees and hips, which was usually bilateral. Only one destructive form was described (multiple villonodular synovitis and a giant cell lesion of the jaw). There were no cases of systemic lupus erythaematosus (SLE) or other autoimmune arthritis. Raynaud's phenomenon was observed in 3 patients. Only 50% of joint complaints led to additional exploration. SOS1 mutations (P<0.05) and treatment with growth hormone (GH) (P<0.05) were the only factors significantly related to musculoskeletal pain. Patients treated with GH did not have more SOS1 mutations. Patients experiencing pain were not more likely to experience stiffness, joint hypermobility, or coagulation abnormalities. CONCLUSION: Joint manifestations were frequent in Noonan syndrome, predominant in large joints, and rarely explored. Multiple villonodular synovitis is characteristic but rare. Auto-immune disorders were not described in this cohort. A more multidisciplinary approach could be recommended for the early detection of possibly disabling rheumatologic manifestations. CI - Copyright © 2021. Published by Elsevier Masson SAS. FAU - Le Quellec, Aurore AU - Le Quellec A AD - Rheumatology department, Center of Autoimmune Rare Diseases (CERAINO), CHU Cavale Blanche, Brest, France. FAU - Edouard, Thomas AU - Edouard T AD - Endocrinology, bone diseases and genetics unit, hôpital des Enfants, CHU de Toulouse, France. FAU - Audebert-Bellanger, Séverine AU - Audebert-Bellanger S AD - Pediatric and genetic department, CHU Morvan, Brest, France. FAU - Pouzet, Antoine AU - Pouzet A AD - Pediatric and genetic department, CHU Morvan, Brest, France. FAU - Bourdet, Karine AU - Bourdet K AD - Pediatric department, CHU Morvan, Brest, France. FAU - Colson, Cindy AU - Colson C AD - Genetic department, CHU de Caen, France. FAU - Oriot, Charlotte AU - Oriot C AD - Pediatric and genetic department, CHU de Nantes, France. FAU - Poignant, Sylvaine AU - Poignant S AD - Pediatric and genetic department, CHU de Nantes, France. FAU - Saraux, Alain AU - Saraux A AD - Rheumatology department, Center of Autoimmune Rare Diseases (CERAINO), CHU Cavale Blanche, Brest, France; Endocrinology, bone diseases and genetics unit, hôpital des Enfants, CHU de Toulouse, France; Pediatric and genetic department, CHU Morvan, Brest, France; Pediatric department, CHU Morvan, Brest, France; Genetic department, CHU de Caen, France; Pediatric and genetic department, CHU de Nantes, France; UMR1227, Inserm, université de Bretagne Occidentale, Brest, France. FAU - Devauchelle-Pensec, Valérie AU - Devauchelle-Pensec V AD - Rheumatology department, Center of Autoimmune Rare Diseases (CERAINO), CHU Cavale Blanche, Brest, France; Endocrinology, bone diseases and genetics unit, hôpital des Enfants, CHU de Toulouse, France; Pediatric and genetic department, CHU Morvan, Brest, France; Pediatric department, CHU Morvan, Brest, France; Genetic department, CHU de Caen, France; Pediatric and genetic department, CHU de Nantes, France; UMR1227, Inserm, université de Bretagne Occidentale, Brest, France. Electronic address: valerie.devauchelle-pensec@chu-brest.fr. LA - eng PT - Journal Article DEP - 20210914 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Child MH - Humans MH - *Lupus Erythematosus, Systemic MH - *Noonan Syndrome/diagnosis/epidemiology/genetics MH - Retrospective Studies MH - *Synovitis MH - *Synovitis, Pigmented Villonodular/pathology OTO - NOTNLM OT - Central giant cell granuloma OT - Joint involvement OT - Noonan Syndrome OT - Pigmented villonodular synovitis OT - RASopathy EDAT- 2021/09/18 06:00 MHDA- 2022/04/07 06:00 CRDT- 2021/09/17 20:14 PHST- 2021/01/20 00:00 [received] PHST- 2021/08/26 00:00 [accepted] PHST- 2021/09/18 06:00 [pubmed] PHST- 2022/04/07 06:00 [medline] PHST- 2021/09/17 20:14 [entrez] AID - S1297-319X(21)00143-3 [pii] AID - 10.1016/j.jbspin.2021.105270 [doi] PST - ppublish SO - Joint Bone Spine. 2022 Jan;89(1):105270. doi: 10.1016/j.jbspin.2021.105270. Epub 2021 Sep 14. PMID- 25015229 OWN - NLM STAT- MEDLINE DCOM- 20150407 LR - 20211021 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 8 DP - 2014 Jul 11 TI - Combined use of ursodeoxycholic acid and bosentan prevents liver toxicity caused by endothelin receptor antagonist bosentan monotherapy: two case reports. PG - 250 LID - 10.1186/1752-1947-8-250 [doi] AB - INTRODUCTION: Pulmonary arterial hypertension is a fatal disease characterized by progressive remodeling of the pulmonary arteries and an increase in pulmonary vascular resistance. Up to 50% of patients with systemic sclerosis have pulmonary arterial hypertension, which significantly affects the prognosis. The endothelin receptor antagonist bosentan is used for the treatment of pulmonary arterial hypertension and shows a great beneficial effect. However, the most frequent side effect of bosentan is liver toxicity, which often requires dose reduction and discontinuation. CASE PRESENTATION: We report two cases (a 64-year-old Japanese woman and a 69-year old Japanese woman) of systemic sclerosis, both with severe Raynaud's phenomenon and pulmonary arterial hypertension. Both patients had initially received bosentan monotherapy, which caused liver toxicity as indicated by increased levels of alanine aminotransferase, alkaline phosphatase, and gamma-glutamyltransferase. After dose reduction or discontinuation of bosentan, these liver function abnormalities were normalized and the patients subsequently received retreatment with a combination of bosentan and ursodeoxycholic acid. The results of liver function tests did not show any abnormalities after this combination therapy. CONCLUSIONS: These reports suggest the usefulness of ursodeoxycholic acid for preventing liver toxicity caused by bosentan. Thus, the addition of ursodeoxycholic acid to the treatment protocol is expected to be useful when liver toxicity emerges as a side effect of bosentan. FAU - Ito, Tomoki AU - Ito T AD - First Department of Internal Medicine, Kansai Medical University, 2-5-1 Shin-machi, Hirakata City, Osaka 573-1010, Japan. itot@hirakata.kmu.ac.jp. FAU - Ozaki, Yoshio AU - Ozaki Y FAU - Son, Yonsu AU - Son Y FAU - Nishizawa, Tohru AU - Nishizawa T FAU - Amuro, Hideki AU - Amuro H FAU - Tanaka, Akihiro AU - Tanaka A FAU - Tamaki, Takeshi AU - Tamaki T FAU - Nomura, Shosaku AU - Nomura S LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140711 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 RN - 0 (Cholagogues and Choleretics) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - 724L30Y2QR (Ursodeoxycholic Acid) RN - Q326023R30 (Bosentan) SB - IM MH - Aged MH - Bosentan MH - Chemical and Drug Induced Liver Injury/*etiology/*prevention & control MH - Cholagogues and Choleretics/*therapeutic use MH - Drug Therapy, Combination MH - Endothelin Receptor Antagonists/*adverse effects/therapeutic use MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy MH - Liver Function Tests MH - Middle Aged MH - Sulfonamides/*adverse effects/therapeutic use MH - Ursodeoxycholic Acid/*therapeutic use PMC - PMC4107936 EDAT- 2014/07/13 06:00 MHDA- 2015/04/08 06:00 PMCR- 2014/07/11 CRDT- 2014/07/13 06:00 PHST- 2014/03/14 00:00 [received] PHST- 2014/06/11 00:00 [accepted] PHST- 2014/07/13 06:00 [entrez] PHST- 2014/07/13 06:00 [pubmed] PHST- 2015/04/08 06:00 [medline] PHST- 2014/07/11 00:00 [pmc-release] AID - 1752-1947-8-250 [pii] AID - 10.1186/1752-1947-8-250 [doi] PST - epublish SO - J Med Case Rep. 2014 Jul 11;8:250. doi: 10.1186/1752-1947-8-250. PMID- 24809534 OWN - NLM STAT- MEDLINE DCOM- 20160101 LR - 20211021 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 48 IP - 2-3 DP - 2015 Jun TI - Extrahepatic autoimmune conditions associated with primary biliary cirrhosis. PG - 192-7 LID - 10.1007/s12016-014-8427-x [doi] AB - There is a paucity of information on extrahepatic autoimmune (EHA) conditions associated with primary biliary cirrhosis (PBC) and on the impact of EHA conditions on PBC patients' survival. Our goal was to assess the association between PBC and other autoimmune diseases and the impact of EHA conditions on the natural history of PBC. We took advantage of 361 consecutive PBC patients enrolled between 1975 and 2012 (22 males, 339 females; mean follow-up 8 ± 6.9 years). Any associated EHA conditions, PBC histological stage at diagnosis, biochemical data, physiological history, and extrahepatic malignancies developing during the follow-up were recorded. Survival was analyzed by means of Kaplan-Meier curves. Importantly, 221 patients (61.2 %) had at least one EHA conditions: 45 patients (20.4 %) had Hashimoto thyroiditis; 7 (3.2 %) had Graves' thyroiditis; 65 (29.4 %) had Raynaud's phenomenon; 124 (56.1 %) had Sjogren's syndrome; 8 (3.6 %) had systemic lupus erythematosus; 22 (9.9 %) had scleroderma; 22 (9.9 %) had rheumatoid arthritis; 18 (8.1 %) had cutaneous autoimmune diseases; 8 (3.6 %) had vasculitis; 5 (1.4 %) had celiac disease; and 25 (13.1 %) had other EHA conditions. The proportion of patients with associated EHA conditions enrolled during representative periods (1975-1980, 1981-1990, 1991-2000, 2001-2010, 2011-2012) remained stable. No differences emerged between patients with versus without EHA conditions in terms of mean age at PBC diagnosis, antimitochondrial antibody (AMA), or antinuclear antibody (ANA) positivity, histological stage at diagnosis, smoking habits, alcohol consumption, or BMI >25. Multiple logistic regression analysis showed that only female gender was significantly associated with positivity for EHA conditions (OR 4.8; 95 % CI 1.6-13.7, p = 0.004). The mean survival after the diagnosis of PBC was much the same in patients with and without EHA conditions. In conclusion, EHA conditions are often associated with PBC, especially in female patients, but they do not reduce patient survival. FAU - Floreani, Annarosa AU - Floreani A AD - Department of Surgery, Oncology and Gastroenterology-DiSCOG, University of Padova, Via Giustiniani, 2, 35128, Padova, Italy, annarosa.floreani@unipd.it. FAU - Franceschet, Irene AU - Franceschet I FAU - Cazzagon, Nora AU - Cazzagon N FAU - Spinazzè, Alice AU - Spinazzè A FAU - Buja, Alessandra AU - Buja A FAU - Furlan, Patrizia AU - Furlan P FAU - Baldo, Vincenzo AU - Baldo V FAU - Gershwin, M Eric AU - Gershwin ME LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 SB - IM MH - Adult MH - Aged MH - Animals MH - Autoimmune Diseases/diagnosis/*immunology/mortality MH - *Autoimmunity MH - Female MH - Humans MH - Liver Cirrhosis, Biliary/diagnosis/*immunology/mortality MH - Male MH - Middle Aged EDAT- 2014/05/09 06:00 MHDA- 2016/01/02 06:00 CRDT- 2014/05/10 06:00 PHST- 2014/05/10 06:00 [entrez] PHST- 2014/05/09 06:00 [pubmed] PHST- 2016/01/02 06:00 [medline] AID - 10.1007/s12016-014-8427-x [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2015 Jun;48(2-3):192-7. doi: 10.1007/s12016-014-8427-x. PMID- 21125149 OWN - NLM STAT- MEDLINE DCOM- 20110616 LR - 20220330 IS - 1809-4570 (Electronic) IS - 0482-5004 (Linking) VI - 50 IP - 2 DP - 2010 Mar-Apr TI - Quantification of basal digital blood flow and after cold stimulus by laser doppler imaging in patients with systemic sclerosis. PG - 128-40 LID - S0482-50042010000200003 [pii] AB - OBJECTIVES: The objective of this study was to investigate the dynamic behavior of the blood flow of the microvascular circulation of the fingertips before and after two cold stimuli (CS), using Laser Doppler Imaging with different intensities in patients with systemic sclerosis (SSc) and in healthy individuals. PATIENTS AND METHODS: Fourteen SSc patients (51.2 ± 5.5 years) with Raynaud's phenomenon and 12 healthy controls (44.8 ± 9.0 years) were included in this study. Two CS protocols (submersion of the hands in water at 10 ºC or 15 ºC for 1 minute) were performed on the same day. Mean fingertip blood flow (FBF) of four digits of the left hand was measured using LDI (Moor LDI-VR, Moor Instruments) at baseline and at 1, 4, 10, 25, and 40 minutes after CS. RESULTS: Baseline blood flow was significantly lower in both CS protocols in SSc patients when compared to controls (312.9 ± 102.7 vs 465.4 ± 135.4 PU, P = 0.006 at 15 ºC; 305.2 ± 121.0 vs 437.9 ± 119.8 PU; P = 0.01 at 10 ºC). In the control group, a significant decrease in FBF after CS, when compared to baseline, was observed 1 minute (P = 0.001) after CS at 15 ºC and at 1 (P = 0.005) and 25 minutes (P = 0.001) after CS at 10 ºC. In SSc patients, a significant decrease in FBF was observed in both CS protocols at 1, 4, and 10 minutes (P < 0.000; P = 0.002; P = 0.014, after CS at 15 ºC; P < 0.000; P = 0.004; P = 0.001, after CS at 10 ºC). CONCLUSIONS: Laser Doppler Imaging showed lower baseline fingertip perfusion and further reduction after CS in SSc patients compared to controls. Quantification of fingertip blood flow by LDI may be useful in the longitudinal monitoring of the disease status and therapeutic interventions in SSc. FAU - Corrêa, Marcelo José Uchoa AU - Corrêa MJ AD - Disciplina de Reumatologia, UNIFESP. FAU - Perazzio, Sandro F AU - Perazzio SF FAU - Andrade, Luís Eduardo Coelho AU - Andrade LE FAU - Kayser, Cristiane AU - Kayser C LA - eng LA - por PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Brazil TA - Rev Bras Reumatol JT - Revista brasileira de reumatologia JID - 0404256 SB - IM MH - Adult MH - Cold Temperature MH - Female MH - Fingers/*blood supply MH - Humans MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation MH - Middle Aged MH - Physical Stimulation MH - Regional Blood Flow MH - Scleroderma, Systemic/*physiopathology EDAT- 2010/12/03 06:00 MHDA- 2011/06/17 06:00 CRDT- 2010/12/03 06:00 PHST- 2009/03/17 00:00 [received] PHST- 2010/02/24 00:00 [accepted] PHST- 2010/12/03 06:00 [entrez] PHST- 2010/12/03 06:00 [pubmed] PHST- 2011/06/17 06:00 [medline] AID - S0482-50042010000200003 [pii] PST - ppublish SO - Rev Bras Reumatol. 2010 Mar-Apr;50(2):128-40. PMID- 19296402 OWN - NLM STAT- MEDLINE DCOM- 20100302 LR - 20100205 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 38 IP - 4 DP - 2009 TI - A longitudinal follow-up of hand involvement and activities of daily living in early systemic sclerosis. PG - 304-10 LID - 10.1080/03009740802695466 [doi] AB - OBJECTIVE: To investigate the development of hand involvement and activities of daily life (ADL) in early systemic sclerosis (SSc), and to examine the usefulness of the Hand Mobility in Scleroderma (HAMIS) test in a longitudinal study. METHODS: Forty-three patients with disease duration no longer than 3 years and at least 4 years of follow-up formed the study group. Based on skin involvement, 31 patients were classified as limited cutaneous SSc (lcSSc) and 12 as diffuse cutaneous SSc (dcSSc). The patients were assessed regarding hand function [mobility (HAMIS), grip force (Grippit), and self-assessed hand function visual analogue scale (VAS)], vascular involvement [Raynaud's phenomenon (RP)], skin involvement [modified Rodnan skin score (mRss)], and ADL capacity [the scleroderma Functional Score (FS)]. RESULTS: Hand mobility and ADL capacity were in general good and did not change significantly. However, at the individual level 72% of the patients showed a change in HAMIS score. Grip force and perceived hand function were moderately impaired at baseline and during the follow-up. Skin involvement in the hand/arm improved significantly (p<0.001). During the observation period, the hand/arm mRss and HAMIS score changed in parallel (r(s) = 0.58, p<0.001). ADL capacity correlated significantly with grip force, self-assessed hand function, and RP at baseline, and also with HAMIS at follow-up. CONCLUSIONS: In this study hand mobility and ADL capacity were maintained during the first years of SSc. HAMIS was found to be a feasible test for longitudinal assessment of hand mobility in SSc. FAU - Sandqvist, G AU - Sandqvist G AD - Department of Rheumatology, Lund University Hospital, SE-22185 Lund, Sweden. gunnel.sandqvist@skane.se FAU - Hesselstrand, R AU - Hesselstrand R FAU - Eberhardt, K AU - Eberhardt K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 SB - IM MH - *Activities of Daily Living MH - Disability Evaluation MH - Female MH - Finger Joint/physiopathology MH - Follow-Up Studies MH - Hand Deformities, Acquired/*epidemiology/etiology MH - Hand Strength MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Pain Measurement MH - Probability MH - Quality of Life MH - Range of Motion, Articular/*physiology MH - Risk Assessment MH - Scleroderma, Systemic/*complications/*diagnosis MH - Severity of Illness Index MH - Statistics, Nonparametric MH - Sweden EDAT- 2009/03/20 09:00 MHDA- 2010/03/03 06:00 CRDT- 2009/03/20 09:00 PHST- 2009/03/20 09:00 [entrez] PHST- 2009/03/20 09:00 [pubmed] PHST- 2010/03/03 06:00 [medline] AID - 909673081 [pii] AID - 10.1080/03009740802695466 [doi] PST - ppublish SO - Scand J Rheumatol. 2009;38(4):304-10. doi: 10.1080/03009740802695466. PMID- 40826379 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250914 LR - 20250914 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 9 IP - 1 DP - 2025 Aug 18 TI - Clinical characteristics and outcomes of anti-MDA5 dermatomyositis: a retrospective study. PG - 102 LID - 10.1186/s41927-025-00556-1 [doi] LID - 102 AB - BACKGROUND: Anti-MDA5 dermatomyositis (anti-MDA5 DM) is a rare but severe subtype of dermatomyositis associated with high mortality. This study aimed to describe clinical characteristics and identify variables associated with rapidly progressive interstitial lung disease (RP-ILD) and mortality among affected patients. METHODS: Patients diagnosed with anti-MDA5 DM between January 2013 and October 2022 were included. Demographics, clinical manifestations, and laboratory data were collected. Univariable Cox proportional hazard regression was used to assess mortality predictors, while logistic regression models identified factors associated with RP-ILD. RESULTS: Among 26 patients (mean age, 56.6 ± 15.1 years, 7 men), the most common clinical manifestation was Gottrón’s papules (80.8%), followed by ILD (73.1%), periungual erythema (69.2%), heliotrope rash (61.5%), V-sign (42.3%), arthritis (42.3%), weakness (38.5%), shawl sign (34.6%), mechanic’s hand (34.6%), and Raynaud’s phenomenon (19.2%). Among 19 ILD patients, 9 (47.4%) developed RP-ILD, and seven patients died over a median follow-up of 1.5 years. The overall mortality rate in this cohort was 26.9%, with 77.8% having RP-ILD. Mortality was associated with male sex (HR, 7.7; 95% CI, 1.49–39.9; P = 0.02) and RP-ILD, which had a risk difference of 77.8 (95% CI 50.6-104.9%; P = 0.001). Arthritis (P = 0.01) and ANA positivity (P = 0.03) were associated with reduced risk of RP-ILD. Patients with RP-ILD tended to receive aggressive treatments, including triple therapy regimens (55.6%), which consisted of a combination of intravenous methylprednisolone and intravenous cyclophosphamide with either rituximab or a calcineurin inhibitor. Additional treatments included intravenous immunoglobulin (22.2%) and plasma exchange (33.3%). CONCLUSION: Anti-MDA5 DM was associated with a high prevalence of ILD, particularly RP-ILD, contributing to increased mortality, underscoring the need for early identification of high-risk patients and personalized management. TAKE-HOME MESSAGE: Male sex was a key predictor of RP-ILD and poor prognosis in anti-MDA5 dermatomyositis patients. Conversely, arthritis and ANA positivity might be associated with a lower risk of RP-ILD. CLINICAL TRIAL NUMBER: Not applicable. FAU - Sodsri, Tulaton AU - Sodsri T AD - Faculty of Medicine Ramathibodi Hospital, Chakri Naruebodindra Medical Institute, Mahidol University, Samut Prakan, Thailand. FAU - Petnak, Tananchai AU - Petnak T AD - Division of Pulmonary and Pulmonary Critical Care Medicine, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. FAU - Suwatanapongched, Thitiporn AU - Suwatanapongched T AD - Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. FAU - Nitiwarangkul, Chayanin AU - Nitiwarangkul C AD - Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. FAU - Pongtarakulpanit, Nantakarn AU - Pongtarakulpanit N AD - Division of Allergy, Immunology and Rheumatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama 6 Road, Thoong Paya Thai Subdistrict, Ratchathewi District, Bangkok, 10400, Thailand. FAU - Ngamjanyaporn, Pintip AU - Ngamjanyaporn P AD - Division of Allergy, Immunology and Rheumatology, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama 6 Road, Thoong Paya Thai Subdistrict, Ratchathewi District, Bangkok, 10400, Thailand. pintip.nga@mahidol.ac.th. LA - eng PT - Journal Article DEP - 20250818 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC12359913 OTO - NOTNLM OT - Dermatomyositis OT - Interstitial lung disease OT - MDA5 autoantibody OT - Mortality OT - Rapidly progressive interstitial lung disease COIS- Declarations. Ethics approval and consent to participate: The study was carried out in accordance with the Declaration of Helsinki. Ethical approval was granted by the Research Ethics Committee of the Faculty of Medicine Ramathibodi Hospital, Mahidol University (COA no. MURA2023/109). The committee approved the use of patient samples and data for publishing this study. Individual subject informed consent was waived due to the retrospective nature of the study and no prospective engagement with subjects or their legal guardians. Consent for publication: Not applicable. Competing interests: All authors have completed the ICMJE Uniform Disclosure Form. T. Petnak, T. Suwatanapongched, C. Nitiwarangkul and P. Ngamjanyaporn received honoraria from Boehringer Ingelheim for past lectures on idiopathic pulmonary fibrosis, connective tissue-related ILD, or progressive pulmonary fibrosis. However, these activities are unrelated to the content of the submitted manuscript and have been disclosed. The other authors have no conflicts of interest to declare. EDAT- 2025/08/19 06:28 MHDA- 2025/08/19 06:29 PMCR- 2025/08/18 CRDT- 2025/08/19 00:15 PHST- 2025/03/02 00:00 [received] PHST- 2025/08/08 00:00 [accepted] PHST- 2025/08/19 06:29 [medline] PHST- 2025/08/19 06:28 [pubmed] PHST- 2025/08/19 00:15 [entrez] PHST- 2025/08/18 00:00 [pmc-release] AID - 10.1186/s41927-025-00556-1 [pii] AID - 556 [pii] AID - 10.1186/s41927-025-00556-1 [doi] PST - epublish SO - BMC Rheumatol. 2025 Aug 18;9(1):102. doi: 10.1186/s41927-025-00556-1. PMID- 38018600 OWN - NLM STAT- MEDLINE DCOM- 20231130 LR - 20250110 IS - 2050-4527 (Electronic) IS - 2050-4527 (Linking) VI - 11 IP - 11 DP - 2023 Nov TI - Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti-aminoacyl-tRNA synthetase antibodies: A single center experience. PG - e1085 LID - 10.1002/iid3.1085 [doi] LID - e1085 AB - OBJECTIVES: To identify the differences of clinical characteristics, laboratory findings, and the long-term outcomes in patients with anti-synthetase syndrome (ASS) of different anti-aminoacyl-transfer RNA synthetase antibodies. METHODS: We retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS. RESULTS: The frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)-ILD. The highest incidence of RP-ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non-ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti-Jo1 antibody-positive group were significantly higher than anti-Jo1 antibody-negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti-nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP-ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti-Ro52 antibody-positive group than the anti-Ro52 antibody-negative patients with ASS (all p < .05). CONCLUSIONS: Patients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti-Ro52 antibody provides valuable insights for managing and predicting disease progression and long-term outcomes. CI - © 2023 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd. FAU - Zhang, Di AU - Zhang D AUID- ORCID: 0000-0001-7752-4804 AD - Department of Rheumatology, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Wang, Huijing AU - Wang H AD - Department of Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China. FAU - Zhou, Xinpeng AU - Zhou X AD - Department of Rheumatology, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Yang, Jianguo AU - Yang J AD - College of Traditional Chinese Medicine, Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Liu, Yuan AU - Liu Y AD - College of Traditional Chinese Medicine, Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Wang, Wenjing AU - Wang W AD - College of Traditional Chinese Medicine, Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Jiang, Ping AU - Jiang P AD - Department of Rheumatology, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. FAU - Fan, Bing AU - Fan B AD - Department of Rheumatology, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China. LA - eng GR - 82274481/National Natural Science Foundation of China/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Immun Inflamm Dis JT - Immunity, inflammation and disease JID - 101635460 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Prognosis MH - *Amino Acyl-tRNA Synthetases MH - Retrospective Studies MH - Autoantibodies MH - *Myositis/epidemiology/diagnosis MH - *Lung Diseases, Interstitial/epidemiology/diagnosis MH - *Arthritis PMC - PMC10655634 OTO - NOTNLM OT - anti-aminoacyl-tRNA synthetase antibodies OT - anti-synthetase syndrome OT - interstitial lung disease OT - prognostic factor COIS- The authors declare no conflicts of interest. EDAT- 2023/11/29 12:44 MHDA- 2023/11/30 06:43 PMCR- 2023/11/17 CRDT- 2023/11/29 06:38 PHST- 2023/10/30 00:00 [revised] PHST- 2023/07/10 00:00 [received] PHST- 2023/11/02 00:00 [accepted] PHST- 2023/11/30 06:43 [medline] PHST- 2023/11/29 12:44 [pubmed] PHST- 2023/11/29 06:38 [entrez] PHST- 2023/11/17 00:00 [pmc-release] AID - IID31085 [pii] AID - 10.1002/iid3.1085 [doi] PST - ppublish SO - Immun Inflamm Dis. 2023 Nov;11(11):e1085. doi: 10.1002/iid3.1085. PMID- 40044501 OWN - NLM STAT- MEDLINE DCOM- 20250305 LR - 20250511 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 12 IP - 1 DP - 2025 Mar 5 TI - Systematic review of the reporting of extrarenal manifestations in observational studies of Saudi patients with systemic lupus erythematosus. LID - 10.1136/lupus-2024-001469 [doi] LID - e001469 AB - BACKGROUND: SLE is prevalent in Saudi Arabia, with numerous studies focusing on SLE in adult patients. However, there is a lack of comprehensive studies summarising the extrarenal manifestations of SLE in this population. This study aims to assess the variability in the prevalence rates of extrarenal manifestations of SLE across different cities in Saudi Arabia and to emphasise the need for a national registry to better understand the overall disease burden in the region. METHODS: We conducted a systematic review of articles with no time restrictions, including studies from databases such as Medline, ScienceDirect, EBSCO and PubMed up to July 2024. The review process involved screening, data extraction and quality assessment in duplicate. Only observational or experimental studies focusing on extrarenal manifestations in adult patients with SLE in Saudi Arabia were included. The review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) checklist for systematic reviews to ensure a rigorous and comprehensive evaluation. RESULTS: A total of 35 studies were included, primarily retrospective cohort studies. Riyadh showed the highest number of publications over time. Musculoskeletal involvement in SLE ranged from 2% to 100%, with most studies reporting 46%-85%. Mucocutaneous manifestations, including discoid rash (5%-100%), malar rash (up to 79%) and photosensitivity (6.12%-29.3%), varied widely. Raynaud's phenomenon was noted at 4.5%-15.2%. Constitutional symptoms were more common in early-onset SLE, while serositis and cardiopulmonary issues showed variability. Neuropsychiatric symptoms, especially depression, reached up to 67.6%. CONCLUSION: This study explores the prevalence of extrarenal manifestations of SLE among adult Saudi patients, highlighting significant regional variability in musculoskeletal, dermatological, cardiovascular and neurological symptoms. It addresses a gap in the literature for a region where autoimmune diseases are a growing public health concern. The findings emphasise the need for population-based studies to investigate environmental, genetic and lifestyle factors influencing SLE progression. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Alenzi, Fahidah AU - Alenzi F AUID- ORCID: 0000-0001-8495-9064 AD - Department of Internal Medicine, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia fmalenzi@pnu.edu.sa. FAU - Aljohani, Roaa AU - Aljohani R AD - Department of Medicine, College of Medicine, Taibah University, Medinah, Saudi Arabia. FAU - Aboabat, Aos AU - Aboabat A AD - Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia. FAU - Alanazi, Fehaid AU - Alanazi F AD - Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia. FAU - Almalag, Haya M AU - Almalag HM AUID- ORCID: 0000-0001-8531-556X AD - Department of Clinical Pharmacy, College of Pharmacy, King Saud University, Riyadh, Saudi Arabia. FAU - Omair, Mohammed A AU - Omair MA AD - Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia. LA - eng PT - Journal Article PT - Systematic Review DEP - 20250305 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - Saudi Arabia/epidemiology MH - Observational Studies as Topic MH - Prevalence MH - Adult PMC - PMC11883561 OTO - NOTNLM OT - Autoimmune Diseases OT - Lupus Erythematosus, Systemic OT - Systemic Lupus Erythematosus COIS- Competing interests: None declared. EDAT- 2025/03/06 05:04 MHDA- 2025/03/06 05:05 PMCR- 2025/03/05 CRDT- 2025/03/05 21:53 PHST- 2024/12/01 00:00 [received] PHST- 2025/02/18 00:00 [accepted] PHST- 2025/03/06 05:05 [medline] PHST- 2025/03/06 05:04 [pubmed] PHST- 2025/03/05 21:53 [entrez] PHST- 2025/03/05 00:00 [pmc-release] AID - 12/1/e001469 [pii] AID - lupus-2024-001469 [pii] AID - 10.1136/lupus-2024-001469 [doi] PST - epublish SO - Lupus Sci Med. 2025 Mar 5;12(1):e001469. doi: 10.1136/lupus-2024-001469. PMID- 38711827 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240508 IS - 1178-7074 (Print) IS - 1178-7074 (Electronic) IS - 1178-7074 (Linking) VI - 17 DP - 2024 TI - Journey into the Esophageal Complications: Decoding Systemic Sclerosis with Cutting-Edge Endoscopy, Manometry, and Ambulatory pH-Study. PG - 1823-1831 LID - 10.2147/IJGM.S448421 [doi] AB - PURPOSE: Systemic Sclerosis (SSc) is a rare connective tissue disorder characterized by autoimmunity, fibrosis, and vasculopathy that affects the skin and internal organs, including the gastrointestinal tract, particularly the esophagus. This article highlights the characteristics and clinical symptoms of esophageal involvement in patients with SSc. PATIENTS AND METHODS: This study was conducted between November 2022 to August 2023, including 26 already diagnosed cases of SSc in the Department of Rheumatology and Rehabilitation and Kurdistan Center for Gastroenterology and Hepatology-Sulaymaniyah, Iraq. Esophageal involvement was investigated using esophageal manometry, esophagogastroduodenoscopy (EGD), and 24-hour impedance-pH monitoring. RESULTS: Females were significantly predominant (P = 0.019) regarding the symptoms; 76.9% of the patients had heart burn, 76.9% dysphagia, 73.1% water brush, and 69.2% regurgitation. In total, 69.2% of the patients showed erosive gastrointestinal reflux disease (GERD) on EGD, 76.9% had decreased lower esophageal sphincter pressure (DLESP) and decreased distal esophageal peristaltic contractions (DDEPC) on esophageal manometry, and 84.6% had reflux on pH monitoring. Raynaud's phenomenon is the most common and typically the earliest clinical manifestation of SSc. The presence of erosive GERD was found to significantly increase the risk of developing dysphagia (B = 4.725, P = 0.014, OR = 3.482) and regurgitation (B = 3.521, P = 0.006, OR = 4.030). CONCLUSION: It is crucial to take gender-specific considerations into account when diagnosing and managing esophageal complications in patients with systemic sclerosis (SSc). Additionally, employing various diagnostic assessments to detect esophageal involvement during SSc is essential. Erosive GERD has been identified as a risk factor that contributes to the development of dysphagia and regurgitation in individuals with SSc. CI - © 2024 Amin et al. FAU - Amin, Omer Ahmed Hamad AU - Amin OAH AD - Department of Rheumatology, Ranya Teaching Hospital, Ministry of Health, Ranya, Kurdistan Region, Iraq. FAU - Mirza, Raouf Rahim AU - Mirza RR AD - College of Medicine, University of Sulaimani, Sulaimani, Kurdistan Region, Iraq. FAU - Hussein, Hiwa Abubakr AU - Hussein HA AD - College of Medicine, University of Sulaimani, Sulaimani, Kurdistan Region, Iraq. FAU - Khudhur, Zhikal Omar AU - Khudhur ZO AD - Biology Education Department, Tishk International University, Erbil, Iraq. FAU - Awla, Harem Khdir AU - Awla HK AUID- ORCID: 0000-0003-1725-1730 AD - Department of Biology, College of Science, Salahaddin University, Erbil, Kurdistan Region, Iraq. FAU - Smail, Shukur Wasman AU - Smail SW AUID- ORCID: 0000-0001-8188-2540 AD - Department of Biology, College of Science, Salahaddin University, Erbil, Kurdistan Region, Iraq. AD - Department of Medical Microbiology, College of Science, Cihan University-Erbil, Kurdistan Region, Iraq. LA - eng PT - Journal Article DEP - 20240502 PL - New Zealand TA - Int J Gen Med JT - International journal of general medicine JID - 101515487 PMC - PMC11073525 OTO - NOTNLM OT - 24-hour Impedance-pH Monitoring OT - esophageal involvement OT - esophageal manometry OT - esophagogastroduodenoscopy OT - systemic sclerosis COIS- The authors declare that there is no specific funding received for conducting this research and that there is no conflict of interest in influencing or bias the work. EDAT- 2024/05/07 06:42 MHDA- 2024/05/07 06:43 PMCR- 2024/05/02 CRDT- 2024/05/07 03:40 PHST- 2024/01/09 00:00 [received] PHST- 2024/04/18 00:00 [accepted] PHST- 2024/05/07 06:43 [medline] PHST- 2024/05/07 06:42 [pubmed] PHST- 2024/05/07 03:40 [entrez] PHST- 2024/05/02 00:00 [pmc-release] AID - 448421 [pii] AID - 10.2147/IJGM.S448421 [doi] PST - epublish SO - Int J Gen Med. 2024 May 2;17:1823-1831. doi: 10.2147/IJGM.S448421. eCollection 2024. PMID- 27051100 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160407 LR - 20200930 IS - 0970-2113 (Print) IS - 0974-598X (Electronic) IS - 0970-2113 (Linking) VI - 33 IP - 2 DP - 2016 Mar-Apr TI - Pulmonary involvement in systemic sclerosis: A clinical profile. PG - 144-7 LID - 10.4103/0970-2113.177439 [doi] AB - BACKGROUND: Systemic sclerosis is a generalized disorder of connective tissue affecting skin and internal organs. Lung involvement accounts for significant morbidity and is a leading cause of mortality in patients. OBJECTIVES: This study intends to study the frequency of occurrence of pulmonary involvement in progressive systemic sclerosis (PSS) and to describe the clinical and radiological picture of pulmonary involvement in PSS. MATERIALS AND METHODS: This was a descriptive cross-sectional study. A detailed history, modified Rodnan score, clinical examination, routine investigation, antinuclear antibody, immuno biot, chest X-ray (CXR), pulmonary function test (PFT), and 6 min walk test (6MWT) were performed on all patients. High resolution computed tomography was done on those who consented. RESULTS: Hundred subjects with PSS were included in the study; 90 were females and 10 were males. Common presenting complaints were skin thickening in 98% and Raynaud's phenomenon in 98%. Skin thickening of digits beyond metacarpo phalangeal was seen in 98%, face and neck in 92%, and hands in 92%. Chest wall thickening was seen in 40 subjects (40%). 90 (90%) of the studied subjects had pulmonary involvement, longer duration of disease was significantly associated with pulmonary involvement (P < 0.05). Dyspnea, cough, bilateral crepitations, CXR, Borg score, and Rodnan score was found to be significantly associated with severe pulmonary involvement (P < 0.05). CONCLUSION: The prevalence of pulmonary involvement in this cohort study was 90%. Almost 1/3(rd) of patients, that is 29 (29%) were detected to have pulmonary involvement despite being asymptomatic for respiratory complaints, hence early screening and evaluation is recommended. PFT and 6MWT are noninvasive, cost-effective, and easily available screening tests which can be used in resource-limited settings. FAU - Deepa, A S AU - Deepa AS AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - Rachel, Roopa Premanand AU - Rachel RP AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - Ramchandran, Priya AU - Ramchandran P AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - Devaraj, Uma AU - Devaraj U AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - Arnold, S Abhilash AU - Arnold SA AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - Shobha, Vineeta AU - Shobha V AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. FAU - D'souza, George AU - D'souza G AD - Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India. LA - eng PT - Journal Article PL - India TA - Lung India JT - Lung India : official organ of Indian Chest Society JID - 8405380 PMC - PMC4797431 OTO - NOTNLM OT - Diffusing capacity of the lungs for carbon monoxide (DLCO) OT - forced expiratory volume at 1 s OT - forced vital capacity OT - metacarpo phalangeal OT - mixed connective tissue disorder OT - systemic sclerosis EDAT- 2016/04/07 06:00 MHDA- 2016/04/07 06:01 PMCR- 2016/03/01 CRDT- 2016/04/07 06:00 PHST- 2016/04/07 06:00 [entrez] PHST- 2016/04/07 06:00 [pubmed] PHST- 2016/04/07 06:01 [medline] PHST- 2016/03/01 00:00 [pmc-release] AID - LI-33-144 [pii] AID - 10.4103/0970-2113.177439 [doi] PST - ppublish SO - Lung India. 2016 Mar-Apr;33(2):144-7. doi: 10.4103/0970-2113.177439. PMID- 26759224 OWN - NLM STAT- MEDLINE DCOM- 20170106 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 36 IP - 5 DP - 2016 May TI - Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort. PG - 697-702 LID - 10.1007/s00296-015-3412-0 [doi] AB - The objective of this study is to describe the characteristics of patients with Erasmus syndrome (ES) in a large SSc Brazilian cohort. Nine hundred and forty-seven SSc patients attended at the Scleroderma Outpatient Clinic at two academic medical centers in Brazil and classified as SSc according to the ACR/EULAR criteria were retrospectively studied. Information on demographics, clinical, and laboratory features was obtained by chart review. ES patients had their HLA class II characterized by PCR-SSO method as available. Among the 947 SSc patients studied, nine (0.9 %) had ES. These ES patients were predominantly male (78 %) and smokers (68 %) and presented diffuse SSc (67 %). Mean time of occupational exposure to silica was 13.7 years, with mean age at onset of 47 years. Previous history of tuberculosis was referred by 33 % of the ES patients. All the ES patients presented Raynaud's phenomenon, esophageal involvement, and interstitial lung disease (ILD). Antinuclear antibodies were present in all the ES patients, while anti-topoisomerase I was positive in 44 % and no patient had anticentromere antibody. Three different HLA-DQB alleles (0506, 0305, and 0303) were observed. Compared to non-ES cases, patients with ES were associated with male gender (p < 0.001), diffuse SSc (p < 0.05), ILD (p < 0.05), positive anti-topoisomerase I antibodies (p < 0.05), and death (p < 0.05). Multivariate analysis did not confirm that silicosis is an independent risk factor for SSc. To conclude, ES was rare in this large SSc cohort, although associated with a bad prognosis. FAU - Rocha, Luiza F AU - Rocha LF AD - Lusíada Foundation School of Medicine, Santos, Brazil. FAU - Luppino Assad, Ana Paula AU - Luppino Assad AP AD - Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Marangoni, Roberta G AU - Marangoni RG AD - Division of Rheumatology, Northwestern University, Chicago, IL, USA. FAU - Del Rio, Ana Paula Toledo AU - Del Rio AP AD - Unit of Rheumatology, University of Campinas, Campinas, Brazil. FAU - Marques-Neto, João Francisco AU - Marques-Neto JF AD - Unit of Rheumatology, University of Campinas, Campinas, Brazil. FAU - Sampaio-Barros, Percival D AU - Sampaio-Barros PD AD - Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. pdsampaiobarros@uol.com.br. AD - Disciplina de Reumatologia, Universidade de São Paulo, Avenida Dr. Arnaldo 455, sala 3142, Cerqueira César, São Paulo, SP, CEP 01246-903, Brazil. pdsampaiobarros@uol.com.br. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160113 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 7631-86-9 (Silicon Dioxide) SB - IM MH - Adult MH - Brazil MH - Female MH - Humans MH - Male MH - Middle Aged MH - Occupational Exposure/*adverse effects MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*etiology MH - Silicon Dioxide/*toxicity MH - Silicosis/*etiology OTO - NOTNLM OT - Erasmus syndrome OT - Occupational exposure OT - Silicosis OT - Systemic sclerosis EDAT- 2016/01/14 06:00 MHDA- 2017/01/07 06:00 CRDT- 2016/01/14 06:00 PHST- 2015/06/16 00:00 [received] PHST- 2015/12/18 00:00 [accepted] PHST- 2016/01/14 06:00 [entrez] PHST- 2016/01/14 06:00 [pubmed] PHST- 2017/01/07 06:00 [medline] AID - 10.1007/s00296-015-3412-0 [pii] AID - 10.1007/s00296-015-3412-0 [doi] PST - ppublish SO - Rheumatol Int. 2016 May;36(5):697-702. doi: 10.1007/s00296-015-3412-0. Epub 2016 Jan 13. PMID- 19022831 OWN - NLM STAT- MEDLINE DCOM- 20090225 LR - 20081205 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 48 IP - 1 DP - 2009 Jan TI - Autologous progenitor cell implantation as a novel therapeutic intervention for ischaemic digits in systemic sclerosis. PG - 61-4 LID - 10.1093/rheumatology/ken407 [doi] AB - OBJECTIVES: The effects of local stem cell implantation on clinical and functional characteristics of peripheral vascular disease were studied in two SSc patients with non-healing ischaemic ulcers. METHODS: The local injections of CD34(+) cells from peripheral blood (PB) after mobilization by G-CSF (Case 1) and bone marrow (BM) (Case 2) were used for ischaemic skin ulcers in hands, while mononuclear cells (MNCs) were implanted in lower extremities of the same patients. Ischaemic status was evaluated by measuring ulcer healing, Raynaud's condition score (RCS), visual analogue pain, RP and ulcer scales. To evaluate vasculoprotective action of the implanted cells, we studied weekly the changes in endothelial function, using measurement of flow-mediated brachial artery reactivity by high-resolution ultrasonography, circulating endothelial precursors (CD34(+)VEGFR2(+), CD133(+)VEGFR2(+) CEP) by FACS analysis, cutaneous blood flow (laser Doppler flowmetry), skin surface temperature (thermograph), peripheral arterial diameter and blood flow characteristics by Duplex ultrasonography. RESULTS: CD34(+) cells and MNCs both from BM and PB showed rapid and evident beneficial effect on vascular symptoms resulting in ulcer healing, remarkably decreased daily frequency and duration of RP attacks, RCS, visual analogue scale for RP, ulcers and pain. Physical function and disability measured with HAQ and SHAQ improved. Therapeutic efficacy of stem cell therapy was associated with restoration of endothelial function, augmentation of microcirculatory blood flow and significant increase in circulating CD133(+)VEGFR2(+) progenitors, known as cell effectors of angiogenesis. CONCLUSION: This first open-label pilot study demonstrates the feasibility and short-term safety of local CD34(+) cell therapy for SSc ischaemic complications. FAU - Nevskaya, T AU - Nevskaya T AD - Department of Microcirculation and Inflammation, State Institute of Rheumatology of Russian Academy of Medical Sciences, Moscow, Russia. tatiananevsk@mail.ru FAU - Ananieva, L AU - Ananieva L FAU - Bykovskaia, S AU - Bykovskaia S FAU - Eremin, I AU - Eremin I FAU - Karandashov, E AU - Karandashov E FAU - Khrennikov, J AU - Khrennikov J FAU - Mach, E AU - Mach E FAU - Zaprjagaeva, M AU - Zaprjagaeva M FAU - Guseva, N AU - Guseva N FAU - Nassonov, E AU - Nassonov E LA - eng PT - Case Reports PT - Journal Article DEP - 20081120 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Antigens, CD34) SB - IM MH - Adult MH - Antigens, CD34/blood MH - Feasibility Studies MH - Female MH - Fingers/*blood supply/pathology MH - Hematopoietic Stem Cell Mobilization/methods MH - Humans MH - Ischemia/etiology/*therapy MH - Microcirculation MH - Middle Aged MH - Peripheral Blood Stem Cell Transplantation/*methods MH - Scleroderma, Systemic/complications/pathology/*therapy MH - Severity of Illness Index MH - Skin/blood supply MH - Skin Ulcer/etiology/pathology/therapy MH - Treatment Outcome EDAT- 2008/11/22 09:00 MHDA- 2009/02/26 09:00 CRDT- 2008/11/22 09:00 PHST- 2008/11/22 09:00 [pubmed] PHST- 2009/02/26 09:00 [medline] PHST- 2008/11/22 09:00 [entrez] AID - ken407 [pii] AID - 10.1093/rheumatology/ken407 [doi] PST - ppublish SO - Rheumatology (Oxford). 2009 Jan;48(1):61-4. doi: 10.1093/rheumatology/ken407. Epub 2008 Nov 20. PMID- 19845665 OWN - NLM STAT- MEDLINE DCOM- 20100526 LR - 20151119 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 162 IP - 2 DP - 2010 Feb 1 TI - Long-term outcome of patients with polymyositis/ dermatomyositis and anti-PM-Scl antibody. PG - 337-44 LID - 10.1111/j.1365-2133.2009.09484.x [doi] AB - BACKGROUND: To date, no series has analysed long-term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti-PM-Scl antibody. OBJECTIVES: The aims of the present study were: (i) to assess clinical features and long-term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti-PM-Scl antibody; and (ii) to evaluate prevalence, characteristics and long-term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti-PM-Scl antibody. METHODS: The medical records of 20 consecutive patients with isolated PM/DM with anti-PM-Scl antibody were reviewed. RESULTS: Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short-term recurrences (during tapering of therapy) occurred in nine patients and long-term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti-PM-Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti-PM-Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud's phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. CONCLUSIONS: Our series suggests that the presence of anti-PM-Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti-PM-Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti-PM-Scl antibody-positive patients with PM/DM often exhibit 'mechanic's hands', Raynaud's phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA-DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti-PM-Scl antibodies. FAU - Marie, I AU - Marie I AD - Department of Internal Medicine, CHU Rouen, 76031 Rouen Cedex, France. isabelle.marie@chu-rouen.fr FAU - Lahaxe, L AU - Lahaxe L FAU - Benveniste, O AU - Benveniste O FAU - Delavigne, K AU - Delavigne K FAU - Adoue, D AU - Adoue D FAU - Mouthon, L AU - Mouthon L FAU - Hachulla, E AU - Hachulla E FAU - Constans, J AU - Constans J FAU - Tiev, K AU - Tiev K FAU - Diot, E AU - Diot E FAU - Levesque, H AU - Levesque H FAU - Boyer, O AU - Boyer O FAU - Jouen, F AU - Jouen F LA - eng PT - Journal Article DEP - 20090829 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Antibodies, Anti-Idiotypic) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - 0 (Nuclear Proteins) RN - 0 (Steroids) RN - EC 3.1.- (Exoribonucleases) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - EC 3.1.13.- (EXOSC10 protein, human) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Anti-Idiotypic/blood/*immunology MH - Biomarkers/blood MH - Dermatomyositis/complications/drug therapy/*immunology MH - Drug Therapy, Combination MH - Exoribonucleases/blood/*immunology MH - Exosome Multienzyme Ribonuclease Complex MH - Female MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Lung Diseases, Interstitial/etiology/immunology MH - Male MH - Middle Aged MH - Nuclear Proteins/blood/*immunology MH - Prognosis MH - Steroids/therapeutic use MH - Time Factors MH - Young Adult EDAT- 2009/10/23 06:00 MHDA- 2010/05/27 06:00 CRDT- 2009/10/23 06:00 PHST- 2009/10/23 06:00 [entrez] PHST- 2009/10/23 06:00 [pubmed] PHST- 2010/05/27 06:00 [medline] AID - BJD9484 [pii] AID - 10.1111/j.1365-2133.2009.09484.x [doi] PST - ppublish SO - Br J Dermatol. 2010 Feb 1;162(2):337-44. doi: 10.1111/j.1365-2133.2009.09484.x. Epub 2009 Aug 29. PMID- 37485232 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230918 IS - 2040-6223 (Print) IS - 2040-6231 (Electronic) IS - 2040-6223 (Linking) VI - 14 DP - 2023 TI - Predicting cardiovascular risk in a Chinese primary Sjögren's syndrome population: development and assessment of a predictive nomogram. PG - 20406223231181490 LID - 10.1177/20406223231181490 [doi] LID - 20406223231181490 AB - BACKGROUND: Patients with primary Sjögren's syndrome (pSS) are at increased risk of cardiovascular morbidity as compared with the general population. OBJECTIVES: A retrospective study on 349 Chinese patients with pSS was conducted to identify potential risk factors for cardiovascular events and develop a cardiovascular risk nomogram. DESIGN: This is a retrospective observational study. METHODS: The study included 349 patients who were diagnosed with pSS at Tongji Hospital, School of Medicine, Tongji University, China from January 2010 to March 2022. The least absolute shrinkage and selection operator (LASSO) was used to select features for the cardiovascular risk model. The features selected in LASSO were used to build the cardiovascular risk model in a multivariate logistic regression analysis. C-index, receiver operating characteristic (ROC) curve, calibration plot, and decision curve analysis were used to assess the predictive model. Internal validation was performed by bootstrapping. RESULTS: Sex, joint pain as an initial symptom, dry mouth, oral ulcers, dental caries, Raynaud's phenomenon, fatigue, diabetes, elevated thyroid-stimulating hormone (TSH) level, and elevated systolic blood pressure were included in the nomogram for the prediction of cardiovascular risk. Our model had good discrimination (C-index: 0.824, 95% confidence interval: 0.712-0.936) and good calibration (C-index in the interval validation: 0.8). Decision curve analysis indicated that our nomogram demonstrated clinical usefulness for intervention in a cardiovascular disease possibility threshold of 3%. CONCLUSION: The cardiovascular risk nomogram incorporating sex, initial joint pain, dry mouth, oral ulcer, dental caries, Raynaud's phenomenon, fatigue, diabetes, elevated TSH, and systolic blood pressure could be used in the prediction of cardiovascular risk in patients with pSS and the guidance of further treatment. CI - © The Author(s), 2023. FAU - Pu, Jincheng AU - Pu J AUID- ORCID: 0000-0001-9242-3067 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Song, Jiamin AU - Song J AUID- ORCID: 0000-0001-9815-8508 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Pan, Shengnan AU - Pan S AUID- ORCID: 0000-0002-4520-0239 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Zhuang, Shuqi AU - Zhuang S AUID- ORCID: 0000-0002-3506-0631 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Gao, Ronglin AU - Gao R AUID- ORCID: 0000-0002-4507-480X AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Liang, Yuanyuan AU - Liang Y AUID- ORCID: 0000-0003-0360-286X AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Wu, Zhenzhen AU - Wu Z AUID- ORCID: 0000-0003-4384-3473 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Wang, Yanqing AU - Wang Y AUID- ORCID: 0000-0002-9946-159X AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Zhang, Youwei AU - Zhang Y AUID- ORCID: 0000-0001-5765-7618 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Yang, Lufei AU - Yang L AUID- ORCID: 0000-0002-6756-7293 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Han, Fang AU - Han F AUID- ORCID: 0000-0001-5584-8971 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Wu, Huihong AU - Wu H AUID- ORCID: 0000-0002-2842-4812 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Tang, Jianping AU - Tang J AUID- ORCID: 0000-0003-4629-7631 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, No.389 Xincun Road, Shanghai 200065, China. FAU - Wang, Xuan AU - Wang X AUID- ORCID: 0000-0002-6588-2951 AD - Department of Rheumatology and Immunology, Tongji Hospital, School of Medicine, Tongji University, No.389 Xincun Road, Shanghai 200065, China. LA - eng PT - Journal Article DEP - 20230718 PL - United States TA - Ther Adv Chronic Dis JT - Therapeutic advances in chronic disease JID - 101532140 PMC - PMC10357044 OTO - NOTNLM OT - cardiovascular events OT - nomogram OT - predictive model OT - primary Sjögren’s syndrome OT - risk COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/07/24 06:42 MHDA- 2023/07/24 06:43 PMCR- 2023/07/18 CRDT- 2023/07/24 04:54 PHST- 2022/10/18 00:00 [received] PHST- 2023/05/24 00:00 [accepted] PHST- 2023/07/24 06:42 [pubmed] PHST- 2023/07/24 06:43 [medline] PHST- 2023/07/24 04:54 [entrez] PHST- 2023/07/18 00:00 [pmc-release] AID - 10.1177_20406223231181490 [pii] AID - 10.1177/20406223231181490 [doi] PST - epublish SO - Ther Adv Chronic Dis. 2023 Jul 18;14:20406223231181490. doi: 10.1177/20406223231181490. eCollection 2023. PMID- 39185579 OWN - NLM STAT- MEDLINE DCOM- 20250423 LR - 20250514 IS - 2376-9130 (Electronic) IS - 1080-3548 (Linking) VI - 30 IP - 4 DP - 2024 Dec TI - Hand cold stress testing among Arctic open-pit miners: a clinical study. PG - 1188-1195 LID - 10.1080/10803548.2024.2383051 [doi] AB - Objectives. This study aimed to evaluate the influence of individual characteristics (sex, age, body mass index [BMI] and smoking habits) on the tolerance time, pain ratings and rewarming time of hand cold stress testing (CST). Methods. We included 153 subjects (63% men) working in a Swedish open-pit mine (participation rate 41%). The right hand was immersed in 3 °C circulating water for up to 45 s. Pain ratings were registered every fifth second using a visual analog scale. Results. The tolerance time (mean ± standard deviation) was 35 ± 12 s for men and 29 ± 14 s for women (p = 0.007). The youngest age group (18-29 years) had the longest tolerance time, while the oldest group (54-65 years) had the shortest (p = 0.005). Women had significantly higher pain ratings than men after 5, 10 and 25 s. The group with the highest BMI had the shortest rewarming time (p < 0.001). Conclusions. Age and sex influenced the tolerance time of hand CST, while only sex affected the pain ratings and BMI the rewarming time. When performing CST in future studies, these parameters should be considered. FAU - Stjernbrandt, Albin AU - Stjernbrandt A AUID- ORCID: 0000-0001-6082-8465 AD - Department of Public Health and Clinical Medicine, Umeå University, Sweden. FAU - Pettersson, Hans AU - Pettersson H AUID- ORCID: 0000-0001-7077-2389 AD - Department of Public Health and Clinical Medicine, Umeå University, Sweden. FAU - Wahlström, Jens AU - Wahlström J AUID- ORCID: 0000-0002-2359-509X AD - Department of Public Health and Clinical Medicine, Umeå University, Sweden. FAU - Rödin, Ingemar AU - Rödin I AD - Department of Occupational and Environmental Medicine, University Hospital of North Norway, Norway. FAU - Nilsson, Tohr AU - Nilsson T AUID- ORCID: 0000-0003-2789-6321 AD - Department of Public Health and Clinical Medicine, Umeå University, Sweden. FAU - Burström, Lage AU - Burström L AUID- ORCID: 0000-0001-6534-2026 AD - Department of Public Health and Clinical Medicine, Umeå University, Sweden. LA - eng PT - Journal Article DEP - 20240826 PL - England TA - Int J Occup Saf Ergon JT - International journal of occupational safety and ergonomics : JOSE JID - 9507598 SB - IM MH - Humans MH - Male MH - Female MH - Middle Aged MH - Adult MH - Young Adult MH - *Hand/physiology MH - Aged MH - Pain Measurement MH - Body Mass Index MH - Age Factors MH - Adolescent MH - Sex Factors MH - Sweden MH - *Cold Temperature/adverse effects MH - Time Factors MH - *Miners MH - Arctic Regions MH - *Mining MH - Smoking OTO - NOTNLM OT - Raynaud disease OT - Sweden OT - cold climate OT - cold sensitivity OT - cold stress testing OT - mining OT - peripheral nervous system diseases OT - vibration EDAT- 2024/08/26 12:38 MHDA- 2025/01/29 06:20 CRDT- 2024/08/26 05:33 PHST- 2025/01/29 06:20 [medline] PHST- 2024/08/26 12:38 [pubmed] PHST- 2024/08/26 05:33 [entrez] AID - 10.1080/10803548.2024.2383051 [doi] PST - ppublish SO - Int J Occup Saf Ergon. 2024 Dec;30(4):1188-1195. doi: 10.1080/10803548.2024.2383051. Epub 2024 Aug 26. PMID- 31758424 OWN - NLM STAT- MEDLINE DCOM- 20201119 LR - 20220307 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 39 IP - 2 DP - 2020 Feb TI - Comparative analysis of connective tissue disease-associated interstitial lung disease and interstitial pneumonia with autoimmune features. PG - 575-583 LID - 10.1007/s10067-019-04836-3 [doi] AB - OBJECTIVE: This retrospective clinical study aimed to examine the similarities and differences between connective tissue disease-associated interstitial lung disease (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF) and to identify the influencing factors of CTD-ILD, with a goal of early detection and active treatment of the disease. METHODS: We conducted a retrospective study of 480 patients: 412 with CTD-ILD and 68 with IPAF. Demographic features, clinical characteristics, laboratory indicators, and chest high-resolution computed tomography (HRCT) imaging data were analyzed. RESULTS: Compared with the IPAF group, the CTD-ILD group contained more women, and the incidences of joint pain, dry mouth/dry eyes, and Raynaud's phenomenon were higher; erythrocyte sedimentation rate (ESR) and D-dimer levels were higher; red blood cell (RBC) and hemoglobin (Hb) levels were lower; a high rheumatoid factor (RF) titer (> 2 times the normal upper limit) was observed, and anti-cyclic citrullinated peptide antibody (anti-CCP), anti-keratin antibody (AKA), antinuclear antibody (ANA), and anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) levels were higher. Compared with CTD-ILD patients, IPAF patients were more likely to present initially with respiratory symptoms, with higher rates of fever, cough and expectoration, dyspnea, and Velcro crackles; anti-Ro52 titers were higher; incidences of honeycombing opacity, reticulate opacity, patchy opacity, and pleural thickening were greater. Female sex, a high RF titer (> 2 times the normal upper limit), anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD when the odds ratios were adjusted. CONCLUSION: CTD-ILD and IPAF patients differed in demographic features, clinical characteristics, laboratory indicators, and chest HRCT imaging data. Female sex, a high RF titer (> 2 times the normal upper limit), anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD.Key Points• This retrospective clinical study comprehensively compared the demographic features, clinical characteristics, laboratory indicators, and chest HRCT imaging data of CTD-ILD and IPAF patients.• The evidence suggested that female sex, a high RF titer, anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD. FAU - Tian, Mengxue AU - Tian M AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Huang, Wenhan AU - Huang W AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Ren, Feifeng AU - Ren F AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Luo, Lei AU - Luo L AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Zhou, Jun AU - Zhou J AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Huang, Dongmei AU - Huang D AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. FAU - Tang, Lin AU - Tang L AUID- ORCID: 0000-0002-6406-9563 AD - Department of Rheumatology and Immunology, The Second Affiliated Hospital of Chongqing Medical University, 76 Linjiang Road, Chongqing, 400010, China. tanglin1217@163.com. LA - eng GR - grant No. 81771738/National Natural Science Foundation of China/ PT - Comparative Study PT - Journal Article DEP - 20191122 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Autoantibodies/blood MH - Connective Tissue Diseases/blood/*complications/diagnostic imaging MH - Diabetes Mellitus, Type 1/blood/*complications/diagnostic imaging MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung Diseases, Interstitial/blood/diagnostic imaging/*etiology MH - Male MH - Middle Aged MH - Radiography, Thoracic MH - Retrospective Studies MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Autoantibodies OT - Connective tissue disease OT - Interstitial lung disease OT - Interstitial pneumonia with autoimmune features OT - Rheumatic immune disease EDAT- 2019/11/24 06:00 MHDA- 2020/11/20 06:00 CRDT- 2019/11/24 06:00 PHST- 2019/07/09 00:00 [received] PHST- 2019/10/31 00:00 [accepted] PHST- 2019/09/29 00:00 [revised] PHST- 2019/11/24 06:00 [pubmed] PHST- 2020/11/20 06:00 [medline] PHST- 2019/11/24 06:00 [entrez] AID - 10.1007/s10067-019-04836-3 [pii] AID - 10.1007/s10067-019-04836-3 [doi] PST - ppublish SO - Clin Rheumatol. 2020 Feb;39(2):575-583. doi: 10.1007/s10067-019-04836-3. Epub 2019 Nov 22. PMID- 28580766 OWN - NLM STAT- MEDLINE DCOM- 20180611 LR - 20180611 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 20 IP - 9 DP - 2017 Sep TI - Pharmacological stress, rest perfusion and delayed enhancement cardiac magnetic resonance identifies very early cardiac involvement in systemic sclerosis patients of recent onset. PG - 1247-1260 LID - 10.1111/1756-185X.13107 [doi] AB - OBJECTIVE: To evaluate occult cardiac involvement in asymptomatic systemic sclerosis (SSc) patients by pharmacological stress, rest perfusion and delayed enhancement cardiac magnetic resonance (CMR), for a very early identification of patients at higher risk of cardiac-related mortality. METHODS: Sixteen consecutive patients with definite SSc, fulfilling the American College of Rheumatology/European League Against Rheumatism 2013 classification criteria in less than 1 year from the onset of Raynaud's phenomenon, underwent pharmacological stress, rest perfusion and delayed enhancement CMR. At enrollment, no patient showed signs and/or symptoms suggestive for cardiac involvement. No patient showed traditional cardiovascular risk factors. Both the 12-lead electrocardiogram examination and echocardiographic evaluation did not show any alterations in our cohort. RESULTS: Stress perfusion defects of left ventricle were detected in six out of 16 (37.5%) patients and these defects did not match with the coronary flow distribution. The results showed the presence of two different patterns of stress perfusion defects: sub-endocardial and/or a midmyocardial. The presence of stress perfusion defects did not correlate with any clinical feature of enrolled patients. CONCLUSION: Myocardial stress perfusion defects may be detected early by pharmacological stress perfusion CMR, a reliable and sensitive technique for the noninvasive evaluation of SSc heart disease, in patients with SSc of recent onset. These defects seem to be independent from traditional risk factors and associated comorbidities, suggesting they are a specific hallmark of the disease. CI - © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Giacomelli, Roberto AU - Giacomelli R AUID- ORCID: 0000-0003-0670-9638 AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Di Cesare, Ernesto AU - Di Cesare E AD - Department of Biotechnological and Applied Clinical Sciences, Division of Cardiac Radiology, Laboratory of Radiobiology, University of L'Aquila, L'Aquila, Italy. FAU - Cipriani, Paola AU - Cipriani P AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Di Sibio, Alessandra AU - Di Sibio A AD - Department of Biotechnological and Applied Clinical Sciences, Division of Radiology, Laboratory of Radiobiology, University of L'Aquila, L'Aquila, Italy. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Gennarelli, Antonio AU - Gennarelli A AD - Department of Biotechnological and Applied Clinical Sciences, Division of Radiology, Laboratory of Radiobiology, University of L'Aquila, L'Aquila, Italy. FAU - Carubbi, Francesco AU - Carubbi F AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Splendiani, Alessandra AU - Splendiani A AD - Department of Biotechnological and Applied Clinical Sciences, Division of Radiology, Laboratory of Radiobiology, University of L'Aquila, L'Aquila, Italy. FAU - Berardicurti, Onorina AU - Berardicurti O AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Di Benedetto, Paola AU - Di Benedetto P AD - Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. FAU - Ciccia, Francesco AU - Ciccia F AD - Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy. FAU - Guggino, Giuliana AU - Guggino G AD - Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy. FAU - Radchenko, Ganna AU - Radchenko G AD - Institute of Cardiology of Ukrainian National Academy of Medical Science, Kyiv, Ukraine. FAU - Triolo, Giovanni AU - Triolo G AD - Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy. FAU - Masciocchi, Carlo AU - Masciocchi C AD - Department of Biotechnological and Applied Clinical Sciences, Division of Radiology, Laboratory of Radiobiology, University of L'Aquila, L'Aquila, Italy. LA - eng PT - Journal Article DEP - 20170604 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Contrast Media) RN - 0 (Vasodilator Agents) RN - K72T3FS567 (Adenosine) SB - IM MH - Adenosine/*administration & dosage MH - Adult MH - Asymptomatic Diseases MH - Contrast Media/administration & dosage MH - *Coronary Circulation MH - Early Diagnosis MH - Female MH - Heart Diseases/*diagnostic imaging/etiology/physiopathology MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Male MH - *Microcirculation MH - *Multidetector Computed Tomography MH - Myocardial Perfusion Imaging/*methods MH - Predictive Value of Tests MH - Scleroderma, Systemic/*complications/diagnosis MH - Vasodilator Agents/*administration & dosage OTO - NOTNLM OT - cardiac magnetic resonance imaging with pharmacological stress OT - myocardial perfusion defect OT - systemic sclerosis OT - systemic sclerosis heart involvement EDAT- 2017/06/06 06:00 MHDA- 2018/06/12 06:00 CRDT- 2017/06/06 06:00 PHST- 2017/06/06 06:00 [pubmed] PHST- 2018/06/12 06:00 [medline] PHST- 2017/06/06 06:00 [entrez] AID - 10.1111/1756-185X.13107 [doi] PST - ppublish SO - Int J Rheum Dis. 2017 Sep;20(9):1247-1260. doi: 10.1111/1756-185X.13107. Epub 2017 Jun 4. PMID- 36576023 OWN - NLM STAT- MEDLINE DCOM- 20230728 LR - 20240425 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 75 IP - 8 DP - 2023 Aug TI - Associations Between Patterns of Esophageal Dysmotility and Extra-Intestinal Features in Patients With Systemic Sclerosis. PG - 1715-1724 LID - 10.1002/acr.25080 [doi] AB - OBJECTIVE: The gastrointestinal tract is commonly involved in patients with systemic sclerosis (SSc) with varied manifestations. As our understanding of SSc gastrointestinal disease pathogenesis and risk stratification is limited, we sought to investigate whether patterns of esophageal dysfunction associate with specific clinical phenotypes in SSc. METHODS: Patients enrolled in the Johns Hopkins Scleroderma Center Research Registry who completed high-resolution esophageal manometry (HREM) studies as part of their clinical care between 2011 and 2020 were identified. Associations between esophageal abnormalities on HREM (absent contractility [AC], ineffective esophageal motility [IEM], hypotensive lower esophageal sphincter [hypoLES]) and patient demographic information, clinical characteristics, and autoantibody profiles were examined. RESULTS: Ninety-five patients with SSc had HREM data. Sixty-five patients (68.4%) had AC (37 patients with only AC, 28 patients with AC and a hypoLES), 9 patients (9.5%) had IEM, and 11 patients (11.6%) had normal studies. AC was significantly associated with diffuse cutaneous disease (38.5% versus 10.0%; P < 0.01), more severe Raynaud's phenomenon, including digital pits, ulcers, or gangrene (56.9% versus 30.0%; P = 0.02), and reduced median diffusing capacity of lung for carbon monoxide (50.6% versus 72.2%; P = 0.03). AC was observed in most of the patients who died (13 of 14; P = 0.06). These findings were not seen in patients with IEM. CONCLUSION: Among patients with SSc, AC is associated with a significantly more severe clinical phenotype. IEM may associate with a milder phenotype. Further studies are needed to evaluate AC, IEM, and their clinical impact relative to the timing of other end-organ complications in SSc. CI - © 2022 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Tucker, Ana E AU - Tucker AE AUID- ORCID: 0000-0003-3774-6087 AD - Medical University of South Carolina, Charleston. FAU - Perin, Jamie AU - Perin J AD - Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland. FAU - Volkmann, Elizabeth R AU - Volkmann ER AUID- ORCID: 0000-0003-3750-6569 AD - University of California Los Angeles. FAU - Abdi, Tsion AU - Abdi T AD - Johns Hopkins University, Baltimore, Maryland. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University, Baltimore, Maryland. FAU - Pandolfino, John AU - Pandolfino J AD - Northwestern University, Chicago, Illinois. FAU - Silver, Richard M AU - Silver RM AUID- ORCID: 0000-0002-2038-3278 AD - Medical University of South Carolina, Charleston. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AUID- ORCID: 0000-0001-6461-8940 AD - Johns Hopkins University, Baltimore, Maryland. LA - eng GR - K23 AR071473/AR/NIAMS NIH HHS/United States GR - P30 AR070254/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20230217 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Esophageal Motility Disorders/etiology/complications MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology MH - *Skin Diseases/complications MH - Autoantibodies MH - *Scleroderma, Localized PMC - PMC11019887 MID - NIHMS1980805 EDAT- 2022/12/29 06:00 MHDA- 2023/07/28 06:42 PMCR- 2024/04/16 CRDT- 2022/12/28 05:33 PHST- 2022/11/29 00:00 [revised] PHST- 2022/07/11 00:00 [received] PHST- 2022/12/20 00:00 [accepted] PHST- 2023/07/28 06:42 [medline] PHST- 2022/12/29 06:00 [pubmed] PHST- 2022/12/28 05:33 [entrez] PHST- 2024/04/16 00:00 [pmc-release] AID - 10.1002/acr.25080 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2023 Aug;75(8):1715-1724. doi: 10.1002/acr.25080. Epub 2023 Feb 17. PMID- 40151102 OWN - NLM STAT- MEDLINE DCOM- 20250421 LR - 20250421 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 34 IP - 6 DP - 2025 May TI - Skin and beyond: Demographics, clinical features and systemic involvement of cutaneous lupus erythematosus in Pakistani population. PG - 640-647 LID - 10.1177/09612033251330115 [doi] AB - BackgroundLupus erythematosus (LE) is an autoimmune disease with skin being the most important organ involved and presents as LE specific and LE nonspecific lesions.ObjectiveTo determine the clinical demographics, systemic involvement and disease impact on daily lives of patients of cutaneous lupus erythematosus (CLE).MethodologyA cross sectional study of a total of 135 patients who presented with cutaneous symptoms were enrolled. Informed consent was taken. Detailed History, examination, clinical features, laboratory and radiograph investigations were conducted to assess the systemic involvement of internal organs.ResultsOf the 135 patients, 73.3% patients were female and 26.7% were male. Mean age was 28.1 years and duration of CLE was 8.4 months. 56.3% of patients belonged to the rural area. Isolated Chronic CLE (CCLE) was observed in 35.5%, CCLE with overlap Acute CLE (ACLE) in 30.3% and Isolated ACLE in 33.3%. Only two patients had Sub acute CLE (SCLE). In LE specific lesions, limited and generalized discoid rash was noted in 33.3% and 17.7% respectively. 46.6% patients had Malar rash. Oral and nasal ulcers were observed in 51.1 %. Non scarring alopecia was seen in 53.3% of patients. Scarring alopecia was observed in 35.5%. 34% patients had Raynaud's phenomenon, 23.7% had Periungual telangiectasia, and 14.8% had Vasculitis. In systemic involvement, joint involvement was observed in 49 patients (36.3%), renal in 48 patients (35.6%), haematological in 42 patients (31.1%), serositis, 23 patients (17%) and neurological in 14 patients (10.4%). No difference in sexes was observed. ANA positivity was seen in 61 patients, (45.1%), most of whom were females. (56.5%) Anti DSDNA was positive in 42 patients (31.1%) of which 40 were women. Mean DLQI was 11.01 ± 8.2. 45 patients had no or slight effect on their lives. 22.9%, 27.4% and 16.3% had moderate, large and very large effects of CLE on their lifestyle.ConclusionThe clinical characteristics of cutaneous lupus is similar to other studies, but the renal involvement is higher with cutaneous involvement in this study, while Serositis is less prevalent. Localized discoid lupus did not show extra cutaneous involvement, but showed significant impact on daily life. FAU - Abowath, Misbah Zakir AU - Abowath MZ AUID- ORCID: 0009-0005-3670-9585 AD - Dermatology, Institute of Skin Diseases, Sindh Karachi, Pakistan. FAU - Ashraf, Erum AU - Ashraf E AD - Dermatology, Institute of Skin Diseases, Sindh Karachi, Pakistan. FAU - Abowath, Umair Zakir AU - Abowath UZ AUID- ORCID: 0009-0000-3509-5239 AD - Graduate Student, Georgia Institute of Technology, Atlanta, GA, USA. RINGGOLD: 1372 LA - eng PT - Journal Article DEP - 20250328 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Humans MH - Female MH - Male MH - Adult MH - Cross-Sectional Studies MH - *Lupus Erythematosus, Cutaneous/epidemiology/pathology/complications/diagnosis MH - Pakistan/epidemiology MH - Young Adult MH - Adolescent MH - Middle Aged MH - *Skin/pathology OTO - NOTNLM OT - cutaneous lupus erythematosus OT - discoid lupus erythematosus OT - systemic lupus erythematosus COIS- Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2025/03/28 06:25 MHDA- 2025/04/21 06:25 CRDT- 2025/03/28 03:53 PHST- 2025/04/21 06:25 [medline] PHST- 2025/03/28 06:25 [pubmed] PHST- 2025/03/28 03:53 [entrez] AID - 10.1177/09612033251330115 [doi] PST - ppublish SO - Lupus. 2025 May;34(6):640-647. doi: 10.1177/09612033251330115. Epub 2025 Mar 28. PMID- 31084620 OWN - NLM STAT- MEDLINE DCOM- 20191216 LR - 20200225 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 13 IP - 1 DP - 2019 May 14 TI - Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature. PG - 145 LID - 10.1186/s13256-019-2072-1 [doi] LID - 145 AB - BACKGROUND: Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date. CASE PRESENTATION: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud's phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient's diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition. CONCLUSIONS: Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association. FAU - Keragala, B S D P AU - Keragala BSDP AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Herath, H M M T B AU - Herath HMMTB AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. tharukaherath111@gmail.com. FAU - Janappriya, G H D C AU - Janappriya GHDC AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Dissanayaka, B S AU - Dissanayaka BS AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Shyamini, S C AU - Shyamini SC AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Liyanagama, D P AU - Liyanagama DP AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Balendran, Thanushah AU - Balendran T AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Constantine, S R AU - Constantine SR AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. FAU - Gunasekera, C N AU - Gunasekera CN AD - National Hospital of Sri Lanka, Colombo, Sri Lanka. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20190514 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 RN - 0 (Antineoplastic Agents) RN - 0 (Immunoglobulins) RN - 0 (Immunologic Factors) RN - 69G8BD63PP (Bortezomib) SB - IM MH - Aged MH - Antineoplastic Agents/administration & dosage MH - Bortezomib/administration & dosage MH - Disease Progression MH - Female MH - Humans MH - Immunoglobulins/administration & dosage MH - Immunologic Factors/administration & dosage MH - *Magnetic Resonance Imaging MH - Scleredema Adultorum/*diagnostic imaging/drug therapy/physiopathology MH - Scleroderma, Systemic/complications/drug therapy/*physiopathology MH - Smoldering Multiple Myeloma/*diagnostic imaging/drug therapy/physiopathology MH - Treatment Outcome MH - *Whole Body Imaging PMC - PMC6515649 OTO - NOTNLM OT - Monoclonal gammopathy OT - Scleredema OT - Smoldering myeloma COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Not applicable. CONSENT FOR PUBLICATION: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2019/05/16 06:00 MHDA- 2019/12/18 06:00 PMCR- 2019/05/14 CRDT- 2019/05/16 06:00 PHST- 2018/10/23 00:00 [received] PHST- 2019/04/04 00:00 [accepted] PHST- 2019/05/16 06:00 [entrez] PHST- 2019/05/16 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2019/05/14 00:00 [pmc-release] AID - 10.1186/s13256-019-2072-1 [pii] AID - 2072 [pii] AID - 10.1186/s13256-019-2072-1 [doi] PST - epublish SO - J Med Case Rep. 2019 May 14;13(1):145. doi: 10.1186/s13256-019-2072-1. PMID- 30745940 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1817-1737 (Print) IS - 1998-3557 (Electronic) IS - 1998-3557 (Linking) VI - 14 IP - 1 DP - 2019 Jan-Mar TI - Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality. PG - 83-89 LID - 10.4103/atm.ATM_33_18 [doi] AB - BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients. METHODS: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics. RESULTS: Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m(2). The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73-633.10) and 6MWD (OR 1.02: 95% CI; 1.01-1.03) were associated with the development of PAH. CONCLUSION: Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH. FAU - Al Otair, Hadil Ak AU - Al Otair HA AD - Department of Critical Care, King Saud University, Riyadh, Saudi Arabia. FAU - Idrees, Majdy M AU - Idrees MM AD - Department of Medicine, Division of Pulmonology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia. FAU - Saleemi, Sarfraz A AU - Saleemi SA AD - Department of Medicine, Division of Pulmonology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. FAU - Eltoukhy, Ahmed M AU - Eltoukhy AM AD - Department of Critical Care, King Saud University, Riyadh, Saudi Arabia. FAU - Alhijji, Ali A AU - Alhijji AA AD - Department of Medicine, Division of Rheumatology, King Saud University, Riyadh, Saudi Arabia. FAU - Al Habeeb, Waleed A AU - Al Habeeb WA AD - King Fahad Cardiac Centre, King Saud University, Riyadh, Saudi Arabia. FAU - Omair, Mohammed A AU - Omair MA AD - Department of Medicine, Division of Rheumatology, King Saud University, Riyadh, Saudi Arabia. LA - eng PT - Journal Article PL - India TA - Ann Thorac Med JT - Annals of thoracic medicine JID - 101280721 PMC - PMC6341865 OTO - NOTNLM OT - Mortality OT - Saudi Arabia OT - pulmonary arterial hypertension OT - systemic sclerosis COIS- There are no conflicts of interest. EDAT- 2019/02/13 06:00 MHDA- 2019/02/13 06:01 PMCR- 2019/01/01 CRDT- 2019/02/13 06:00 PHST- 2019/02/13 06:00 [entrez] PHST- 2019/02/13 06:00 [pubmed] PHST- 2019/02/13 06:01 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - ATM-14-83 [pii] AID - 10.4103/atm.ATM_33_18 [doi] PST - ppublish SO - Ann Thorac Med. 2019 Jan-Mar;14(1):83-89. doi: 10.4103/atm.ATM_33_18. PMID- 22348608 OWN - NLM STAT- MEDLINE DCOM- 20121217 LR - 20211021 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 14 IP - 1 DP - 2012 Feb 20 TI - Erectile dysfunction is frequent in systemic sclerosis and associated with severe disease: a study of the EULAR Scleroderma Trial and Research group. PG - R37 LID - 10.1186/ar3748 [doi] AB - INTRODUCTION: Erectile dysfunction (ED) is common in men with systemic sclerosis (SSc) but the demographics, risk factors and treatment coverage for ED are not well known. METHOD: This study was carried out prospectively in the multinational EULAR Scleroderma Trial and Research database by amending the electronic data-entry system with the International Index of Erectile Function-5 and items related to ED risk factors and treatment. Centres participating in this EULAR Scleroderma Trial and Research substudy were asked to recruit patients consecutively. RESULTS: Of the 130 men studied, only 23 (17.7%) had a normal International Index of Erectile Function-5 score. Thirty-eight per cent of all participants had severe ED (International Index of Erectile Function-5 score ≤ 7). Men with ED were significantly older than subjects without ED (54.8 years vs. 43.3 years, P < 0.001) and more frequently had simultaneous non-SSc-related risk factors such as alcohol consumption. In 82% of SSc patients, the onset of ED was after the manifestation of the first non-Raynaud's symptom (median delay 4.1 years). ED was associated with severe cutaneous, muscular or renal involvement of SSc, elevated pulmonary pressures and restrictive lung disease. ED was treated in only 27.8% of men. The most common treatment was sildenafil, whose efficacy is not established in ED of SSc patients. CONCLUSIONS: Severe ED is a common and early problem in men with SSc. Physicians should address modifiable risk factors actively. More research into the pathophysiology, longitudinal development, treatment and psychosocial impact of ED is needed. FAU - Foocharoen, Chingching AU - Foocharoen C AD - Department of Rheumatology, Basel University, Burgfelderstrasse 101, Basel 4012, Switzerland. FAU - Tyndall, Alan AU - Tyndall A FAU - Hachulla, Eric AU - Hachulla E FAU - Rosato, Edoardo AU - Rosato E FAU - Allanore, Yannick AU - Allanore Y FAU - Farge-Bancel, Dominique AU - Farge-Bancel D FAU - Caramaschi, Paola AU - Caramaschi P FAU - Airó, Paolo AU - Airó P FAU - Nikolaevna, Starovojtova M AU - Nikolaevna SM FAU - Pereira da Silva, José António AU - Pereira da Silva JA FAU - Stamenkovic, Bojana AU - Stamenkovic B FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Rednic, Simona AU - Rednic S FAU - Sibilia, Jean AU - Sibilia J FAU - Wiland, Piotr AU - Wiland P FAU - Tarner, Ingo AU - Tarner I FAU - Smith, Vanessa AU - Smith V FAU - Onken, Anna T AU - Onken AT FAU - Abdel Atty Mohamed, Walid Ahmed AU - Abdel Atty Mohamed WA FAU - Distler, Oliver AU - Distler O FAU - Morović-Vergles, Jadranka AU - Morović-Vergles J FAU - Himsel, Andrea AU - Himsel A FAU - de la Peña Lefebvre, Paloma Garcia AU - de la Peña Lefebvre PG FAU - Hügle, Thomas AU - Hügle T FAU - Walker, Ulrich A AU - Walker UA LA - eng PT - Journal Article PT - Multicenter Study DEP - 20120220 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) SB - IM CIN - J Urol. 2012 Aug;188(2):549-50. doi: 10.1016/j.juro.2012.04.091. PMID: 22784749 MH - Adult MH - Databases, Factual/statistics & numerical data MH - Erectile Dysfunction/complications/drug therapy/*physiopathology MH - Humans MH - Kidney Diseases/complications/*physiopathology MH - Male MH - Middle Aged MH - Muscular Diseases/complications/*physiopathology MH - Piperazines/therapeutic use MH - Prospective Studies MH - Purines/therapeutic use MH - Scleroderma, Systemic/complications/*physiopathology MH - Severity of Illness Index MH - Sildenafil Citrate MH - Skin Diseases/complications/*physiopathology MH - Sulfones/therapeutic use MH - Surveys and Questionnaires MH - Treatment Outcome MH - Vasodilator Agents/therapeutic use PMC - PMC3392836 EDAT- 2012/02/22 06:00 MHDA- 2012/12/18 06:00 PMCR- 2012/02/20 CRDT- 2012/02/22 06:00 PHST- 2011/09/18 00:00 [received] PHST- 2011/12/11 00:00 [revised] PHST- 2012/02/20 00:00 [accepted] PHST- 2012/02/22 06:00 [entrez] PHST- 2012/02/22 06:00 [pubmed] PHST- 2012/12/18 06:00 [medline] PHST- 2012/02/20 00:00 [pmc-release] AID - ar3748 [pii] AID - 10.1186/ar3748 [doi] PST - epublish SO - Arthritis Res Ther. 2012 Feb 20;14(1):R37. doi: 10.1186/ar3748. PMID- 37291935 OWN - NLM STAT- MEDLINE DCOM- 20230612 LR - 20240919 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 55 IP - 3 DP - 2023 Jun 18 TI - [Anti-HMGCR immune-mediated necrotizing myopathy: A case report]. PG - 558-562 AB - The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin. FAU - Zhang, Y J AU - Zhang YJ AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. FAU - Ma, J Y AU - Ma JY AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. FAU - Liu, X Y AU - Liu XY AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. FAU - Zheng, D F AU - Zheng DF AD - Department of Pathology, Peking University School of Basic Medical Sciences, Beijing 100191, China. FAU - Zhang, Y S AU - Zhang YS AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. FAU - Li, X G AU - Li XG AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. FAU - Fan, D S AU - Fan DS AD - Department of Neurology, Peking University Third Hospital, Beijing 100191, China. LA - chi PT - Case Reports PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) RN - 0 (Hydroxymethylglutaryl-CoA Reductase Inhibitors) SB - IM MH - Male MH - Humans MH - Middle Aged MH - Autoantibodies MH - *Myositis/diagnosis MH - *Autoimmune Diseases MH - Muscle, Skeletal/pathology MH - *Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use MH - Necrosis/pathology MH - *Muscular Diseases/diagnosis/drug therapy PMC - PMC10258068 OTO - NOTNLM OT - 3-hydroxy-3-methylglutaryl-coenzyme areductase (HMGCR) OT - Antibody OT - Autoimmune-mediated necrotizing myopathy EDAT- 2023/06/09 06:42 MHDA- 2023/06/12 06:42 PMCR- 2023/06/18 CRDT- 2023/06/09 03:58 PHST- 2023/06/12 06:42 [medline] PHST- 2023/06/09 06:42 [pubmed] PHST- 2023/06/09 03:58 [entrez] PHST- 2023/06/18 00:00 [pmc-release] AID - bjdxxbyxb-55-3-558 [pii] AID - 10.19723/j.issn.1671-167X.2023.03.025 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2023 Jun 18;55(3):558-562. doi: 10.19723/j.issn.1671-167X.2023.03.025. PMID- 33743728 OWN - NLM STAT- MEDLINE DCOM- 20211103 LR - 20240331 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 19 IP - 1 DP - 2021 Mar 20 TI - Joint contractures responsive to immunosuppressive therapy in a girl with childhood-onset systemic sclerosis double-seropositive for rare anti-nucleolar autoantibodies: a case report. PG - 37 LID - 10.1186/s12969-021-00525-1 [doi] LID - 37 AB - BACKGROUND: Systemic sclerosis (SSc; scleroderma) is an autoimmune connective tissue disease that affects the skin and subcutaneous tissue, in addition to the internal organs of the whole body. Onset in childhood is uncommon; however, both patients with childhood-onset and adult-onset SSc are positive for anti-nuclear antibodies (ANAs).Detection of SSc-related anti-nuclear antibodies is often useful for predicting clinical features, disease course, and outcomes. CASE PRESENTATION: A 5-year-old Japanese female manifested gradually progressive abnormal gait disturbance, regression of motor development, Raynaud's phenomenon, and the shiny appearance of the skin of the face and extremities at age 2. On admission, she presented a mask-like appearance, loss of wrinkles and skin folds, puffy fingers, moderate diffuse scleroderma (18/51 of the modified Rodnan total skin thickness score), and contracture in the ankle and proximal interphalangeal joints. Grossly visible capillary hemorrhage on nail fold and severe abnormal capillaroscopy findings including bleeding, giant loop and disappearance of capillaryconsistent with the late phase in SSc. A skin biopsy showed fibrous thickening of the dermis, entrapment of an eccrine sweat glands, and thickened fiber. Chest high-resolution computed tomographic scanning demonstrated patchy areas of ill-defined air-space opacity and consolidation predominantly involving the posterior basilar aspects of the lower lobes presenting withinterstitial lung disease. Positive ANA (1:160 nucleolar and homogeneous nuclear staining by indirect fluorescent antibody technique) and double-seropositive for anti-Th/To and anti-PM-Scl antibodies were identified. She was diagnosed with diffuse cutaneous SSc based on the Pediatric Rheumatology European Society/American College of Rheumatology/European League Against Rheumatism Provisional Classification Criteria for Juvenile Systemic Sclerosis and was successfully treated with immunosuppressive agents, including methylprednisolone pulses and intravenous cyclophosphamide. CONCLUSIONS: We experienced the first case of juvenile SSc with anti-PM-Scl and anti-Th/To antibodies. ILD was identified as a typical feature of patients with these autoantibodies; however, diffuse cutaneous SSc and joint contraction were uncharacteristically associated. The case showed unexpected clinical findings though the existence of SSc-related autoantibodies aids in determining possible organ involvement and to estimate the children's outcome. FAU - Tanaka, Riki AU - Tanaka R AUID- ORCID: 0000-0001-7419-6286 AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Tani, Yumi AU - Tani Y AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. AD - Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Kaburaki, Yoichiro AU - Kaburaki Y AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Kinoshita, Manao AU - Kinoshita M AD - Department of Dermatology, Faculty of Medicine, University of Yamanashi, Yamanashi, Japan, Shimokato, Chuo, 1110, 409-3898, Yamanashi, Japan. FAU - Kawaguchi, Yasushi AU - Kawaguchi Y AD - Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Okazaki, Yuka AU - Okazaki Y AD - Department of Allergy and Rheumatology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-Ku, 113- 8602, Tokyo, Japan. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-Ku, 113- 8602, Tokyo, Japan. FAU - Harigai, Masayoshi AU - Harigai M AD - Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Nagata, Satoru AU - Nagata S AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. FAU - Miyamae, Takako AU - Miyamae T AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. tmiyamae@twmu.ac.jp. AD - Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, 8-1 Kawada-Cho, Shinjuku- Ku, 162-8666, Tokyo, Japan. tmiyamae@twmu.ac.jp. LA - eng PT - Case Reports PT - Journal Article DEP - 20210320 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) SB - IM MH - Autoantibodies/*blood MH - Cell Nucleolus/*immunology MH - Child, Preschool MH - Contracture/*blood/*drug therapy/etiology MH - Female MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Scleroderma, Systemic/*blood/complications MH - Treatment Outcome PMC - PMC7981830 OTO - NOTNLM OT - Anti Th/To OT - Anti-PM-Scl OT - Autoantibody OT - Children OT - Joint contracture OT - Lung interstitial lesion OT - Systemic sclerosis COIS- The authors declare that they have no competing interests. EDAT- 2021/03/22 06:00 MHDA- 2021/11/04 06:00 PMCR- 2021/03/20 CRDT- 2021/03/21 20:32 PHST- 2020/07/01 00:00 [received] PHST- 2021/03/05 00:00 [accepted] PHST- 2021/03/21 20:32 [entrez] PHST- 2021/03/22 06:00 [pubmed] PHST- 2021/11/04 06:00 [medline] PHST- 2021/03/20 00:00 [pmc-release] AID - 10.1186/s12969-021-00525-1 [pii] AID - 525 [pii] AID - 10.1186/s12969-021-00525-1 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2021 Mar 20;19(1):37. doi: 10.1186/s12969-021-00525-1. PMID- 42018656 OWN - NLM STAT- MEDLINE DCOM- 20260422 LR - 20260504 IS - 2379-3708 (Electronic) IS - 2379-3708 (Linking) VI - 11 IP - 8 DP - 2026 Apr 22 TI - FOSL2 regulates endothelial cell state and chromatin accessibility in systemic sclerosis pulmonary vascular remodeling. LID - 10.1172/jci.insight.189107 [doi] LID - e189107 AB - Systemic sclerosis (SSc) is characterized by fibrosis and vasculopathy affecting the skin and internal organs, leading to multiorgan dysfunction. Injury of microvascular endothelial cells (ECs) in SSc impairs blood flow and causes tissue ischemia, leading to vascular complications such as Raynaud's, digital ulcers, and pulmonary hypertension (PH). PH in SSc presents as group 1 pulmonary arterial hypertension or as group 3 PH related to hypoxia and interstitial lung disease (ILD), both major causes of mortality. Analysis of multiome data from SSc ILD-PH lungs inferred transcription factors regulating EC phenotype, including FOSL2. Overexpression of FOSL2 in transgenic mice (Fosl2tg) leads to vascular changes mirroring human SSc-PH, such as intimal thickening and fibrosis. scRNA-Seq analysis of altered EC gene expression in Fosl2tg mice showed strong overlap with altered EC gene expression in SSc-ILD-PH. Overlapping as well as discrete EC gene expression in Sugen/hypoxia- and hypoxia-treated mice suggested that FOSL2 regulates both hypoxia-dependent and -independent pathways in Fosl2tg mice and SSc-ILD-PH. A deep learning model, ChromBPNet, inferred increased AP-1 binding at base pair resolution in SSc-ILD-PH ECs, and binding to the same motifs was found upon FOSL2 overexpression in primary vascular ECs, highlighting FOSL2's key role in driving the pathological changes seen in SSc-ILD-PH. FAU - Behera, Rithika AU - Behera R AD - Division of Rheumatology and Clinical Immunology and. FAU - Zhou, Yuechen AU - Zhou Y AD - Division of Rheumatology and Clinical Immunology and. FAU - Gerges, Peter H AU - Gerges PH AD - Center for Systems Immunology and Department of Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Fan, Jingyu AU - Fan J AD - Center for Systems Immunology and Department of Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Tabib, Tracy AU - Tabib T AD - Division of Rheumatology and Clinical Immunology and. FAU - Gaydosik, Alyxzandria M AU - Gaydosik AM AD - Division of Rheumatology and Clinical Immunology and. FAU - Huang, Mengqi AU - Huang M AD - Division of Rheumatology and Clinical Immunology and. FAU - Das, Jishnu AU - Das J AD - Center for Systems Immunology and Department of Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Pachera, Elena AU - Pachera E AD - Center for Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland. FAU - Hukara, Amela AU - Hukara A AD - Center for Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland. FAU - Tang, Ying AU - Tang Y AD - Division of Cardiology, Cardiovascular Institute, and. FAU - Renoux, Florian AU - Renoux F AD - Center for Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland. FAU - Tai, Miranda AU - Tai M AD - Division of Cardiology, Cardiovascular Institute, and. FAU - Distler, Oliver AU - Distler O AD - Center for Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland. FAU - Kania, Gabriela AU - Kania G AD - Center for Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland. FAU - Chan, Stephen Y AU - Chan SY AD - Division of Cardiology, Cardiovascular Institute, and. FAU - Singh, Harinder AU - Singh H AD - Center for Systems Immunology and Department of Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Valenzi, Eleanor AU - Valenzi E AD - Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Lafyatis, Robert AU - Lafyatis R AD - Division of Rheumatology and Clinical Immunology and. LA - eng PT - Journal Article DEP - 20260422 PL - United States TA - JCI Insight JT - JCI insight JID - 101676073 RN - 0 (Fos-Related Antigen-2) RN - 0 (Fosl2 protein, mouse) RN - 0 (FOSL2 protein, human) RN - 0 (Chromatin) SB - IM MH - *Fos-Related Antigen-2/metabolism/genetics MH - *Scleroderma, Systemic/genetics/pathology/complications/metabolism MH - Animals MH - Mice MH - *Endothelial Cells/metabolism/pathology MH - Mice, Transgenic MH - Humans MH - *Vascular Remodeling/genetics MH - Hypertension, Pulmonary/genetics/pathology/etiology MH - *Chromatin/metabolism MH - Lung/pathology/blood supply/metabolism MH - Disease Models, Animal MH - Lung Diseases, Interstitial/pathology/genetics PMC - PMC13135392 OTO - NOTNLM OT - Endothelial cells OT - Pulmonology OT - Vascular biology COIS- RL reports grants from Bristol-Myers Squibb, Formation, Moderna, Regeneron, and Pfizer. RL served or serves as a consultant with AbbVie, Mediar Therapeutics, Bristol-Myers Squibb, Formation, Thirona Bio, Sanofi, Boehringer-Ingelheim, Merck, Genentech/Roche, EMD Serono, Morphic, Third Rock Ventures, Bain Capital, and Zag Bio. RL sits on an independent data safety monitoring committees for Advarra/GSK and Genentech. RL holds stock in and is president of Modumac Therapeutics Inc. SYC has served as a consultant for Merck, Janssen, and United Therapeutics. SYC is a director, officer, and shareholder in Synhale Therapeutics and Amlysion Therapeutics. SYC holds research grants from United Therapeutics, Bayer, and WoodNext Foundation. SYC has filed patent applications regarding the targeting of metabolism and inflammation in pulmonary hypertension (US 10,925,869 B2; US20250295657 A1; PCT/US2015/029286; PCT/US2018/062013; PCT/US2020/059969; PCT/US2021/058511). EDAT- 2026/04/22 18:32 MHDA- 2026/04/22 18:33 PMCR- 2026/04/22 CRDT- 2026/04/22 14:04 PHST- 2024/11/11 00:00 [received] PHST- 2026/02/26 00:00 [accepted] PHST- 2026/04/22 18:33 [medline] PHST- 2026/04/22 18:32 [pubmed] PHST- 2026/04/22 14:04 [entrez] PHST- 2026/04/22 00:00 [pmc-release] AID - 189107 [pii] AID - 10.1172/jci.insight.189107 [doi] PST - epublish SO - JCI Insight. 2026 Apr 22;11(8):e189107. doi: 10.1172/jci.insight.189107. eCollection 2026 Apr 22. PMID- 37330316 OWN - NLM STAT- MEDLINE DCOM- 20230905 LR - 20231017 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 115 DP - 2023 Sep TI - Phenotypic Profiles Among 72 Caucasian and Afro-Caribbean Patients with Antisynthetase Syndrome Involving Anti-PL7 or Anti-PL12 Autoantibodies. PG - 104-113 LID - S0953-6205(23)00206-6 [pii] LID - 10.1016/j.ejim.2023.06.012 [doi] AB - OBJECTIVES: Antisynthetase syndrome (ASyS) is a rare autoimmune disease. We aimed to determine clinical, biological, radiological, and evolutive profiles of ASyS patients with anti-PL7 or anti-PL12 autoantibodies. METHODS: We performed a retrospective study that included adults with overt positivity for anti-PL7/anti-PL12 autoantibodies and at least one Connors' criterion. RESULTS: Among 72 patients, 69% were women, 29 had anti-PL7 and 43 anti-PL12 autoantibodies, median age was 60.3 years, and median follow-up period was 52.2 months. At diagnosis, 76% of patients had interstitial lung disease, 61% had arthritis, 39% myositis, 25% Raynaud's phenomenon, 18% mechanic's hands, and 17% had fever. The most frequent pattern on initial chest computed tomography was non-specific interstitial pneumonia and 67% had fibrosis at last follow-up. During follow-up, 12 patients had pericardial effusion (18%), 19 had pulmonary hypertension (29%), 9 (12.5%) had neoplasms, and 14 (19%) died. Sixty-seven patients (93%) received at least one steroid or immunosuppressive drug. Patients with anti-PL12 autoantibodies were younger (p=0.01) and more frequently exhibited anti-SSA autoantibodies (p=0.01); patients with anti-PL7 autoantibodies had more severe weakness and higher maximum creatine kinase levels (p=0.03 and 0.04, respectively). Initial severe dyspnoea was more common in patients from the West Indies (p=0.009), with lower predicted values of forced vital capacity, forced expiratory volume in 1s, and total lung capacity (p=0.01, p=0.02, p=0.01, respectively) contributing to a more severe 'respiratory' initial presentation. CONCLUSIONS: The high mortality and significant numbers of cardiovascular events, neoplasms and lung fibrosis in anti-PL7/12 patients justify close monitoring and question addition of antifibrotic drugs. CI - Copyright © 2023 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. FAU - Abel, Aurore AU - Abel A AD - Department of Internal Medicine, Martinique University Hospital, CEDEX CS, 90632 Fort-de-France, Martinique, France; Department of internal Medicine and Infectious Diseases Department, Haut Leveque Hospital, University Hospital Centre of Bordeaux, F33604 Pessac, France. FAU - Lazaro, Estibaliz AU - Lazaro E AD - Department of internal Medicine and Infectious Diseases Department, Haut Leveque Hospital, University Hospital Centre of Bordeaux, F33604 Pessac, France; Université de Bordeaux, UMR CNRS 5164 Immunoconcept, F33000 Bordeaux, France. FAU - Ralazamahaleo, Mamy AU - Ralazamahaleo M AD - Université de Bordeaux, UMR CNRS 5164 Immunoconcept, F33000 Bordeaux, France; CHU de Bordeaux, Laboratoire d'Immunologie et Immunogénétique, Hôpital Pellegrin, F33000 Bordeaux, France. FAU - Pierrisnard, Emma AU - Pierrisnard E AD - Laboratoire d'immunologie, Cité Hospitalière de Mangot-Vulcin, CHU de Martinique. FAU - Suzon, Benoit AU - Suzon B AD - Department of Internal Medicine, Martinique University Hospital, CEDEX CS, 90632 Fort-de-France, Martinique, France. FAU - Bonnet, Fabrice AU - Bonnet F AD - CHU de Bordeaux, Service de médecine interne et maladies infectieuses, Hôpital Saint-André, F33000 Bordeaux, France; University Bordeaux, ISPED, Inserm U1219, Bordeaux Population Health Research Center, teamGHIGS. F33000 Bordeaux, France. FAU - Mercié, Patrick AU - Mercié P AD - Department of Internal Medicine and Clinical Immunology, Saint Andre Hospital, University Hospital Centre of Bordeaux, F33000 Bordeaux, France; Univ. Bordeaux, INSERM, BRIC, U1312, F-33000 Bordeaux,France. FAU - Macey, Julie AU - Macey J AD - CHU de Bordeaux, Service d'Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d'Exploration Fonctionnelle Respiratoire, Unité de Pneumologie Pédiatrique, CIC 1401, Pessac, France. FAU - Agossou, Moustapha AU - Agossou M AD - Departement of respiratory care, Martinique University Hospital, CEDEX CS, 90632 Fort-de-France, Martinique, France. FAU - Viallard, Jean-Francois AU - Viallard JF AD - Department of internal Medicine and Infectious Diseases Department, Haut Leveque Hospital, University Hospital Centre of Bordeaux, F33604 Pessac, France; INSERM, Biology of Cardiovascular Diseases, U1034, University of Bordeaux, F33604 Pessac, France. FAU - Deligny, Christophe AU - Deligny C AD - Department of Internal Medicine, Martinique University Hospital, CEDEX CS, 90632 Fort-de-France, Martinique, France. FAU - Rivière, Etienne AU - Rivière E AD - Department of internal Medicine and Infectious Diseases Department, Haut Leveque Hospital, University Hospital Centre of Bordeaux, F33604 Pessac, France; INSERM, Biology of Cardiovascular Diseases, U1034, University of Bordeaux, F33604 Pessac, France. Electronic address: etienne.riviere@u-bordeaux.fr. LA - eng PT - Journal Article DEP - 20230615 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Humans MH - Female MH - Middle Aged MH - Male MH - *Autoantibodies MH - Retrospective Studies MH - *Myositis/diagnosis MH - Caribbean Region OTO - NOTNLM OT - Anti-PL12 OT - Anti-PL7 autoantibody OT - Antisynthetase syndrome OT - Idiopathic inflammatory myopathy OT - tRNA synthetase autoantibody COIS- Declaration of Competing Interest The authors have no conflicts of interest to disclose. EDAT- 2023/06/18 01:07 MHDA- 2023/09/05 06:41 CRDT- 2023/06/17 21:53 PHST- 2023/04/20 00:00 [received] PHST- 2023/06/12 00:00 [revised] PHST- 2023/06/13 00:00 [accepted] PHST- 2023/09/05 06:41 [medline] PHST- 2023/06/18 01:07 [pubmed] PHST- 2023/06/17 21:53 [entrez] AID - S0953-6205(23)00206-6 [pii] AID - 10.1016/j.ejim.2023.06.012 [doi] PST - ppublish SO - Eur J Intern Med. 2023 Sep;115:104-113. doi: 10.1016/j.ejim.2023.06.012. Epub 2023 Jun 15. PMID- 34725073 OWN - NLM STAT- MEDLINE DCOM- 20211206 LR - 20211214 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 11 IP - 11 DP - 2021 Nov 1 TI - Treatment patterns and control of hypertension in systemic lupus erythematosus (SLE): a cross-sectional study. PG - e048384 LID - 10.1136/bmjopen-2020-048384 [doi] LID - e048384 AB - OBJECTIVE: Hypertension (HTN) is common in systemic lupus erythematosus (SLE), representing a key risk factor for cardiovascular and renal disease. We described HTN treatment patterns in SLE, evaluated uncontrolled HTN according to Canadian and American guidelines and identified factors associated with uncontrolled HTN. METHODS: We performed a cross-sectional study, identifying all McGill Lupus Clinic registry patients with an annual visit between January 2017 and May 2019 who were taking HTN medications. We excluded those taking medications only for another indication (eg, Raynaud's). We determined the frequency of uncontrolled HTN according to Canadian and American College of Cardiology/American Heart Association guidelines. Multivariate logistic regression (adjusted for age, sex and race/ethnicity) evaluated if uncontrolled HTN was more common with high body mass index (BMI), longer SLE duration, high disease activity, renal damage, multiple concomitant antihypertensives, prednisone and non-steroidal anti-inflammatory drugs. RESULTS: Of 442 patients with SLE, 108 were taking medications to treat HTN, and 38 took multiple medications concurrently. Angiotensin-receptor blockers were most common, followed by calcium channel blockers, diuretics, angiotensin-converting enzyme inhibitors and beta blockers. Among the 108 patients, 39.8% (n=43) had blood pressure (BP) >140/90 mm Hg, while 66.7% (n=72) had BP >130/80 mm Hg. In multivariate analyses, uncontrolled HTN (>130/80 mm Hg) was more likely in Caucasians (OR 2.72, 95% CI 1.12 to 6.78) and patients with higher BMI (OR 1.08, 95% CI 1.00 to 1.19). Patients with renal damage had better HTN control (OR 0.39, 95% CI 0.16 to 0.97). We could not draw definitive conclusions regarding other variables. CONCLUSION: Caucasians and patients with higher BMI had more uncontrolled HTN. The negative association with renal damage is reassuring, as controlled BP is key for renal protection. CI - © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Liu, Jia Li AU - Liu JL AD - Department of Medicine, McGill University, Montreal, Quebec, Canada. FAU - Pineau, Christian A AU - Pineau CA AD - Department of Medicine, McGill University, Montreal, Quebec, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Quebec, Canada. AD - Centre for Innovative Medicine, Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. FAU - Grenier, Louis-Pierre AU - Grenier LP AD - Department of Medicine, McGill University, Montreal, Quebec, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Vinet, Evelyne AU - Vinet E AUID- ORCID: 0000-0001-7727-5879 AD - Department of Medicine, McGill University, Montreal, Quebec, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Quebec, Canada. AD - Centre for Outcomes Research and Evaluation (CORE), Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. FAU - Kalache, Fares AU - Kalache F AD - Department of Medicine, McGill University, Montreal, Quebec, Canada. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Lukusa, Luck AU - Lukusa L AD - Centre for Outcomes Research and Evaluation (CORE), Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. FAU - Bernatsky, Sasha AU - Bernatsky S AUID- ORCID: 0000-0002-9515-2802 AD - Department of Medicine, McGill University, Montreal, Quebec, Canada sasha.bernatsky@mcgill.ca. AD - Division of Rheumatology, McGill University Health Centre, Montreal, Quebec, Canada. AD - Centre for Outcomes Research and Evaluation (CORE), Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211101 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 RN - 0 (Antihypertensive Agents) SB - IM MH - Antihypertensive Agents/therapeutic use MH - Canada/epidemiology MH - Cross-Sectional Studies MH - Humans MH - *Hypertension/drug therapy/epidemiology MH - *Lupus Erythematosus, Systemic/complications/drug therapy PMC - PMC8562504 OTO - NOTNLM OT - epidemiology OT - hypertension OT - protocols & guidelines OT - rheumatology COIS- Competing interests: None declared. EDAT- 2021/11/03 06:00 MHDA- 2021/12/15 06:00 PMCR- 2021/11/01 CRDT- 2021/11/02 05:35 PHST- 2021/11/02 05:35 [entrez] PHST- 2021/11/03 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/11/01 00:00 [pmc-release] AID - bmjopen-2020-048384 [pii] AID - 10.1136/bmjopen-2020-048384 [doi] PST - epublish SO - BMJ Open. 2021 Nov 1;11(11):e048384. doi: 10.1136/bmjopen-2020-048384. PMID- 28197938 OWN - NLM STAT- MEDLINE DCOM- 20180924 LR - 20191210 IS - 1993-0402 (Electronic) IS - 1672-0415 (Linking) VI - 24 IP - 3 DP - 2018 Mar TI - Clinical Efficacy and Safety of Bathing with Chinese Medicine Taohong Siwu Decoction () for Treatment of Diffuse Cutaneous Systemic Sclerosis: A Randomized Placebo-Controlled Trial. PG - 185-192 LID - 10.1007/s11655-017-2954-2 [doi] AB - OBJECTIVE: To examine the efficacy and safety of bathing therapy with Taohong Siwu Decoction (, TSD) in the treatment of early-stage, mild-moderate diffuse cutaneous systemic sclerosis (dcSSc). METHODS: This randomized, placebo-controlled trial enrolled 148 men and women (18-60 years) with dcSSc (disease duration 12 months) and baseline modified Rodnan skin score (MRSS) 10. Patients were randomized into a TSD group (71 cases bathing with TSD plus oral prednisone) or control group (71 cases bathing with placebo plus oral prednisone). Bathing (40 °C, 30 min) of the upper and lower limbs was carried out once daily for 12 consecutive weeks. The primary outcome measure was MRSS; secondary outcomes were Raynaud's phenomenon (RP) score, quality of life (QOL), physician visual analogue scale (VAS), patient VAS, percent predicted diffusing capacity for carbon monoxide (DLCO), percent predicted forced vital capacity (FVC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level and overall treatment effect. RESULTS: The final analysis included 135 patients (control group, 68 cases; TSD group, 67 cases). Primary and secondary outcome measures after 2 weeks of treatment showed no improvement (versus baseline) in both groups, with no differences between groups. At 12 weeks, QOL, physician VAS, patient VAS, ESR and CRP were improved in both groups, but MRSS and RP score were improved only in the TSD group (all P<0.05). MRSS, RP score, QOL, physician VAS, patient VAS, ESR and CRP differed significantly between groups (all P<0.05). Meanwhile, the overall treatment effect was significantly higher in the TSD group than in the control group (P<0.05). Adverse events in the two groups were similar (P>0.05). CONCLUSIONS: Bathing with TSD plus oral prednisone achieves better outcomes than oral prednisone alone in patients with dcSSc and is not associated with serious adverse events. FAU - Zhou, Jing AU - Zhou J AD - Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China. FAU - Yang, Dong AU - Yang D AD - Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China. FAU - Zhou, Shu-Hong AU - Zhou SH AD - Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China. zshlz1973@126.com. FAU - Wang, Jin-Ping AU - Wang JP AD - Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China. FAU - Liu, Yue-Shu AU - Liu YS AD - Department of Traditional Chinese Medicine, Gansu Provincial Hospital, Lanzhou, 730000, China. FAU - Wang, Shu-Lan AU - Wang SL AD - Department of Immunology and Rheumatology, Gansu Provincial Hospital, Lanzhou, 730000, China. LA - eng PT - Journal Article PT - Randomized Controlled Trial DEP - 20170215 PL - China TA - Chin J Integr Med JT - Chinese journal of integrative medicine JID - 101181180 RN - 0 (Drugs, Chinese Herbal) RN - 0 (Placebos) RN - 0 (Taohong Siwu decoction II) SB - IM MH - Adult MH - Drugs, Chinese Herbal/*adverse effects/*therapeutic use MH - Female MH - Humans MH - *Hygiene MH - Intention to Treat Analysis MH - Male MH - Middle Aged MH - Outcome Assessment, Health Care MH - Placebos MH - Scleroderma, Diffuse/*drug therapy MH - Treatment Outcome OTO - NOTNLM OT - Chinese medicine OT - adverse effects OT - bathing therapy OT - systemic sclerosis OT - treatment efficacy EDAT- 2017/02/16 06:00 MHDA- 2018/09/25 06:00 CRDT- 2017/02/16 06:00 PHST- 2016/09/13 00:00 [received] PHST- 2017/02/16 06:00 [pubmed] PHST- 2018/09/25 06:00 [medline] PHST- 2017/02/16 06:00 [entrez] AID - 10.1007/s11655-017-2954-2 [pii] AID - 10.1007/s11655-017-2954-2 [doi] PST - ppublish SO - Chin J Integr Med. 2018 Mar;24(3):185-192. doi: 10.1007/s11655-017-2954-2. Epub 2017 Feb 15. PMID- 19820225 OWN - NLM STAT- MEDLINE DCOM- 20100211 LR - 20211020 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 36 IP - 10 DP - 2009 Oct TI - Measures of response in clinical trials of systemic sclerosis: the Combined Response Index for Systemic Sclerosis (CRISS) and Outcome Measures in Pulmonary Arterial Hypertension related to Systemic Sclerosis (EPOSS). PG - 2356-61 LID - 10.3899/jrheum.090372 [doi] AB - There have been steady efforts to develop a combined response index for systemic sclerosis (CRISS). A parallel and equally successful effort has been made by an Expert Panel on Outcome Measures in PAH related to Systemic Sclerosis (EPOSS) to measure effect in treatment of pulmonary arterial hypertension of systemic sclerosis (PAH-SSc). CRISS conducted a Delphi process combined with expert review to identify 11 candidate domains for inclusion in a core set of outcomes for SSc clinical trials: soluble biomarkers, cardiac, digital ulcers, gastrointestinal, global health, health related quality of life (HRQOL) and function, musculoskeletal, pulmonary, Raynaud's, renal, and skin. Tools within domains were also agreed upon. Concentrating on one aspect of disease, PAH, EPOSS also conducted a Delphi process and judged the following domains as the most appropriate for randomized controlled trials in PAH-SSc: lung vascular/pulmonary arterial pressure, cardiac function, exercise testing; severity of dyspnea, discontinuation of treatment; quality of life/activities of daily living; global state; and survival. Possible useful tools within each domain were also agreed on. Patient derived, physician derived, and objective measures of response will be included and combined with the idea that each reflects different aspects of PAH (EPOSS) and overall disease (CRISS) although this assumption may not prove true and can be separated if statistically and clinically valid to do so. In either case, prospective studies will require measurement of all domains, and tools are required and will be developed to define appropriate combined measures of response. CRISS and EPOSS are being developed through the OMERACT process. Through Delphi process and literature review significant progress has been made for both indices, and prospective data are being collected. FAU - Khanna, Dinesh AU - Khanna D AD - David Geffen School at University of California, Los Angeles, CA, USA. FAU - Distler, Oliver AU - Distler O FAU - Avouac, Jerome AU - Avouac J FAU - Behrens, Frank AU - Behrens F FAU - Clements, Philip J AU - Clements PJ FAU - Denton, Christopher AU - Denton C FAU - Foeldvari, Ivan AU - Foeldvari I FAU - Giannini, Edward AU - Giannini E FAU - Huscher, Doerte AU - Huscher D FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O FAU - Lovell, Daniel AU - Lovell D FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M FAU - Mayes, Maureen AU - Mayes M FAU - Merkel, Peter A AU - Merkel PA FAU - Nash, Peter AU - Nash P FAU - Opitz, Christian F AU - Opitz CF FAU - Pittrow, David AU - Pittrow D FAU - Rubin, Lewis AU - Rubin L FAU - Seibold, James R AU - Seibold JR FAU - Steen, Virginia AU - Steen V FAU - Strand, C Vibeke AU - Strand CV FAU - Tugwell, Peter S AU - Tugwell PS FAU - Varga, John AU - Varga J FAU - Zink, Angela AU - Zink A FAU - Furst, Daniel E AU - Furst DE CN - CRISS CN - EPOSS LA - eng GR - U01 AR055057/AR/NIAMS NIH HHS/United States GR - K23 AR053858-01A1/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Clinical Trials as Topic/*standards MH - Consensus MH - Delphi Technique MH - Disability Evaluation MH - Humans MH - Hypertension, Pulmonary/physiopathology/psychology/*therapy MH - Outcome Assessment, Health Care/*standards MH - Quality of Life MH - Scleroderma, Systemic/physiopathology/psychology/*therapy MH - *Severity of Illness Index MH - Treatment Outcome EDAT- 2009/10/13 06:00 MHDA- 2010/02/12 06:00 CRDT- 2009/10/13 06:00 PHST- 2009/10/13 06:00 [entrez] PHST- 2009/10/13 06:00 [pubmed] PHST- 2010/02/12 06:00 [medline] AID - 36/10/2356 [pii] AID - 10.3899/jrheum.090372 [doi] PST - ppublish SO - J Rheumatol. 2009 Oct;36(10):2356-61. doi: 10.3899/jrheum.090372. PMID- 34824049 OWN - NLM STAT- MEDLINE DCOM- 20220428 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 81 IP - 4 DP - 2022 Apr TI - Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire. PG - 507-515 LID - 10.1136/annrheumdis-2021-220702 [doi] AB - OBJECTIVES: Patient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts. METHODS: This European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres. Relevant health dimensions were chosen and prioritised by patients. The resulting Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was subsequently weighted and validated by Outcome Measures in Rheumatology criteria in an observational cohort study, cross-sectionally and longitudinally. As comparators, SSc-Health Assessment Questionnaire (HAQ), EuroQol Five Dimensional (EQ-5D), Short Form-36 (SF-36) were included. RESULTS: Initially, 17 health dimensions were selected and prioritised. The top 10 health dimensions were selected for the ScleroID questionnaire. Importantly, Raynaud's phenomenon, impaired hand function, pain and fatigue had the highest patient-reported disease impact. The validation cohort study included 472 patients with a baseline visit, from which 109 had a test-retest reliability visit and 113 had a follow-up visit (85% female, 38% diffuse SSc, mean age 58 years, mean disease duration 9 years). The total ScleroID score showed strong Pearson correlation coefficients with comparators (SSc-HAQ, 0.73; Patient's global assessment, Visual Analogue Scale 0.77; HAQ-Disability Index, 0.62; SF-36 physical score, -0.62; each p<0.001). The internal consistency was strong: Cronbach's alpha was 0.87, similar to SSc-HAQ (0.88) and higher than EQ-5D (0.77). The ScleroID had excellent reliability and good sensitivity to change, superior to all comparators (intraclass correlation coefficient 0.84; standardised response mean 0.57). CONCLUSIONS: We have developed and validated the EULAR ScleroID, which is a novel, brief, disease-specific, patient-derived, disease impact PROM, suitable for research and clinical use in SSc. CI - © Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Becker, Mike O AU - Becker MO AUID- ORCID: 0000-0001-9102-3088 AD - Department of Rheumatology, University Hospital of Zurich, Zurich, Switzerland. FAU - Dobrota, Rucsandra AU - Dobrota R AUID- ORCID: 0000-0001-9819-7574 AD - Department of Rheumatology, University Hospital of Zurich, Zurich, Switzerland. FAU - Garaiman, Alexandru AU - Garaiman A AD - Department of Rheumatology, University Hospital of Zurich, Zurich, Switzerland. FAU - Debelak, Rudolf AU - Debelak R AUID- ORCID: 0000-0001-8900-2106 AD - Department of Psychology, Psychological Methods, Evaluation and Statistics, University of Zurich, Zurich, Switzerland. AD - Department of Psychology, Psychological Methodology, University of Leipzig, Leipzig, Germany. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma and Raynaud's UK, London, UK. FAU - Tyrrell Kennedy, Ann AU - Tyrrell Kennedy A AD - Federation of the European Scleroderma Associations (FESCA) aisbl, Tournai, Belgium. FAU - Roennow, Annelise AU - Roennow A AD - Federation of European Scleroderma Associations (FESCA), Saint Maur, Belgium. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France. FAU - Carreira, Patricia E AU - Carreira PE AD - Department of Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Czirják, László AU - Czirják L AD - Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, University College London, Royal Free Campus, London, UK. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Sandqvist, Gunnel AU - Sandqvist G AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Bruni, Cosimo AU - Bruni C AUID- ORCID: 0000-0003-2813-2083 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy. AD - IRCCS San Raffaele Hospital, Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), Milan, Italy. FAU - Mihai, Carina AU - Mihai C AUID- ORCID: 0000-0002-8627-8817 AD - Department of Rheumatology, University Hospital of Zurich, Zurich, Switzerland. AD - Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Gheorghiu, Ana Maria AU - Gheorghiu AM AD - Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Mueller-Ladner, Ulf AU - Mueller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. FAU - Sexton, Joseph AU - Sexton J AD - Division of Rheumatology and Research, Diakonhjemmet Hospital, Oslo, Norway. FAU - Kvien, Tore K AU - Kvien TK AD - Division of Rheumatology and Research, Diakonhjemmet Hospital, Oslo, Norway. FAU - Heiberg, Turid AU - Heiberg T AD - Regional Research Support, Oslo University Hospital, Oslo, Norway. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital of Zurich, Zurich, Switzerland oliver.distler@usz.ch. LA - eng PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20211125 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Cohort Studies MH - Disability Evaluation MH - Female MH - Humans MH - Male MH - Middle Aged MH - Patient Reported Outcome Measures MH - Quality of Life MH - Reproducibility of Results MH - *Rheumatology MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/complications MH - Severity of Illness Index MH - Surveys and Questionnaires OTO - NOTNLM OT - health care OT - immune system diseases OT - patient reported outcome measures OT - quality indicators OT - scleroderma OT - systemic COIS- Competing interests: MB personal fees from Amgen and Bayer, outside the submitted work. RDo reports grants from Articulum Fellowship, sponsored by Pfizer (2013-2014), grants from Actelion, personal fees from Actelion and Boehringer-Ingelheim, outside the submitted work. AG none with respect to the study. RDe none with respect to the study.KF none with respect to the study. ATK none with respect to the study. AR none with respect to the study. YA reports grants and personal fees from Bayer, Boehringer-Ingelheim, Bristol Myers Squibb, Curzion, Inventiva, Roche and Sanofi during the conduct of the study. PEC reports consultancy relationship and/or research grants from: Abbvie, Actelion, Bayer, Boehringer Ingelheim, Corbus Pharma, Emerald Health Pharmaceuticals, Galapagos, Gesynta Pharma, Inventiva, iQvia, Mitsubishi Tanabe Pharma, MSD, Roche and Sanofi Genzyme. LC none with respect to the study. CPD reports grants and personal fees from GSK, Mitsubishi Tanabe Pharma, Boehringer-Ingelheim, Servier, Arxx Therapeutics, Bayer, Inventiva, Galapagos, Horizon, Roche, CSL Behring, Sanofi during the conduct of the study.RH reports personal lecture fees from Actelion Pharmaceuticals Sweden AB, Boehringer-Ingelheim Sweden AB, and Roche Sweden AB, and has been co-investigator in clinical trials for United Therapeutics and Actelion Pharmaceuticals Sweden AB, and has been involved in research advisory boards for Actelion Pharmaceuticals Sweden AB and Boehringer-Ingelheim Sweden AB. GS none with respect to the study. OK-B reports personal fees and/or congress support from Bayer, Boehringer Ingelheim, CSL Behring, Gilead, Inventiva, Medac, MSD, Novartis, Pfizer, Roche, Sandoz, outside the submitted work. CB reports personal fees from Actelion, personal fees from Boehringer-Ingelheim, personal fees from Eli Lilly, grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), grants from New Horizon Fellowship, grants from European Scleroderma Trials and Research (EUSTAR), grants from Foundation for Research in Rheumatology (FOREUM), grants from Fondazione Italiana per la Ricerca sull’Artrite (FIRA), outside the submitted work. MM-C reports grants and personal fees from Actelion, personal fees from Biogen, personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from CSL Behring, personal fees from Eli-Lilly, outside the submitted work. CM reports personal fees from Boehringer Ingelheim, Eli-Lilly, Mepha, MEDTalks Switzerland and congress support from Actelion and Roche, outside the submitted work. AMG reports consultancy fees from Boehringer-Ingelheim and congress support from Abbvie and Novartis, outside the submitted work. UM-L none with respect to the study. JS none with respect to the study. TKK none with respect to the study. TH reports no conflicts of interest.OD has/had consultancy relationship and/or has received research funding in the area of potential treatments of scleroderma and its complications from (last three years): Abbvie, Acceleron Pharma, Alexion, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Bayer, Boehringer Ingelheim, Catenion, Drug Development International, CSL Behring, ChemomAb, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, Italfarmaco, iQvia, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Target Bio Science and UCB In addition, he has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143). EDAT- 2021/11/27 06:00 MHDA- 2022/04/29 06:00 CRDT- 2021/11/26 06:01 PHST- 2021/05/05 00:00 [received] PHST- 2021/11/09 00:00 [accepted] PHST- 2021/11/27 06:00 [pubmed] PHST- 2022/04/29 06:00 [medline] PHST- 2021/11/26 06:01 [entrez] AID - S0003-4967(24)18486-5 [pii] AID - 10.1136/annrheumdis-2021-220702 [doi] PST - ppublish SO - Ann Rheum Dis. 2022 Apr;81(4):507-515. doi: 10.1136/annrheumdis-2021-220702. Epub 2021 Nov 25. PMID- 31309339 OWN - NLM STAT- MEDLINE DCOM- 20200330 LR - 20200330 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 38 IP - 11 DP - 2019 Nov TI - Unique clinical and autoantibody profile of a large Asian Indian cohort of scleroderma-do South Asians have a more aggressive disease? PG - 3179-3187 LID - 10.1007/s10067-019-04659-2 [doi] AB - AIM AND METHODS: A single-centre retrospective study was conducted using electronic medical records (EMR) of inpatients and outpatients with the diagnosis of "scleroderma" or "systemic sclerosis" visiting our clinic over the preceding 5 years. RESULTS: A total of 327 patients' charts met our selection criteria; 301 were females. The median (IQR (inter quartile range)) age at onset of first non-Raynaud's symptom was 34.67 (27-43) years and median (IQR) disease duration prior to presentation to our department was 2.5 (1-5) years. Of these, 310 (94.8%) belonged to diffuse systemic sclerosis variety, 13 (4%) had limited systemic sclerosis, and 4 (1.2%) were of sine scleroderma type. A total of 289/302 (95.7%) patients were positive for ANA; of them, 245/327 (74.9%) were Scl-70 antibody-positive and 4% were CENP antibody-positive. Interstitial lung disease (ILD) was present in 288/327 (88.1%) patients. Among patients with available baseline forced vital capacity (FVC) data, 20% had a normal lung function and 28.4% had severe restriction. Pulmonary hypertension as assessed by echocardiography was present in 8.1% of patients. A significant association of Scl-70 antibody positivity with the presence of interstitial lung disease (ILD) (p = 0.000) and pulmonary hypertension (p = 0.035) was seen. On the other hand, presence of CENP antibody showed a protective trend against muscle weakness and/or muscle enzyme elevation (p = 0.052). Presence of arthritis was protective against development of digital ulceration (p = 0.021) and PAH (0.004). Patients younger than 40 years of age had significantly higher frequency of Scl-70 positivity (p = 0.038), whereas CENP antibody positivity was more likely in those aged > 40 years (p = 0.002). CONCLUSION: Younger age of onset and high prevalence of Scl-70 antibody are unique South Asian features common with large Indian, Thai, and Chinese series. High prevalence of ILD is a feature common to Indian and Chinese series. Strong correlation of Scl-70 antibody with younger age and pulmonary hypertension were unique features of our cohort. KEY POINTS: • Asian Indian scleroderma patients are younger by 2 decades compared to Caucasian series. • Higher prevalence of Scl-70 antibody, its association with young age, interstitial lung disease and pulmonary hypertension are features of our cohort. • High prevalence of interstitial lung disease (88.1%) was noted ; among those with baseline spirometry data (141/327), two thirds(66%) had moderate to severe restriction. • Younger age at onset, higher prevalence of Scl-70 antibody are features common to other Indian, Thai and Chinese series. FAU - Janardana, Ramya AU - Janardana R AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, 632004, India. FAU - Nair, Aswin M AU - Nair AM AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, 632004, India. FAU - Surin, Ajit K AU - Surin AK AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, 632004, India. FAU - Prakash, John Anthony Jude AU - Prakash JAJ AD - Department of Microbiology, Christian Medical College, Vellore, India. FAU - Gowri, Mahasampath AU - Gowri M AD - Department of Biostatistics, Christian Medical College, Vellore, India. FAU - Danda, Debashish AU - Danda D AUID- ORCID: 0000-0002-4904-1511 AD - Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, 632004, India. debashisdandacmc@hotmail.com. LA - eng PT - Journal Article DEP - 20190715 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - *Autoantibodies MH - Female MH - Humans MH - India/epidemiology MH - Lung Diseases, Interstitial/diagnostic imaging/epidemiology/etiology MH - Male MH - Middle Aged MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/complications/epidemiology/*immunology/therapy MH - Tomography, X-Ray Computed MH - Vascular Diseases/epidemiology/etiology MH - Young Adult OTO - NOTNLM OT - Anti-Scl-70 antibody OT - Interstitial lung disease OT - Pulmonary arterial hypertension OT - Scleroderma OT - Younger EDAT- 2019/07/17 06:00 MHDA- 2020/03/31 06:00 CRDT- 2019/07/17 06:00 PHST- 2019/04/22 00:00 [received] PHST- 2019/06/24 00:00 [accepted] PHST- 2019/06/11 00:00 [revised] PHST- 2019/07/17 06:00 [pubmed] PHST- 2020/03/31 06:00 [medline] PHST- 2019/07/17 06:00 [entrez] AID - 10.1007/s10067-019-04659-2 [pii] AID - 10.1007/s10067-019-04659-2 [doi] PST - ppublish SO - Clin Rheumatol. 2019 Nov;38(11):3179-3187. doi: 10.1007/s10067-019-04659-2. Epub 2019 Jul 15. PMID- 31482431 OWN - NLM STAT- MEDLINE DCOM- 20200203 LR - 20231019 IS - 1573-2649 (Electronic) IS - 0962-9343 (Print) IS - 0962-9343 (Linking) VI - 28 IP - 12 DP - 2019 Dec TI - Factors associated with quality of life in systemic sclerosis: a cross-sectional study. PG - 3347-3354 LID - 10.1007/s11136-019-02284-9 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs, leading to their failure and disturbances in the morphology and function of blood vessels. The disease affects people in different ways, and identifying how the difficulties and limitations are related to quality of life may contribute to designing helpful interventions. The aim of this study was to identify factors associated with quality of life in people with SSc. METHODS: This was a cross-sectional study conducted in 11 rheumatic centres in Poland. Patients diagnosed with SSc were included. Quality of life was measured using the SSc Quality of Life Questionnaire (SScQoL). The following candidate factors were entered in preliminary multivariable analysis: age, place of residence, marital status, occupational status, disease type, disease duration, pain, fatigue, intestinal problems, breathing problems, Raynaud's symptoms, finger ulcerations, disease severity, functional disability, anxiety and depression. Factors that achieved statistical significance at the 10% level were then entered into a final multivariable model. Factors achieving statistical significance at the 5% level in the final model were considered to be associated with quality of life in SSc. RESULTS: In total, 231 participants were included. Mean age (SD) was 55.82 (12.55) years, disease duration 8.39 (8.18) years and 198 (85.7%) were women. Factors associated with quality of life in SSc were functional disability (β = 2.854, p < 0.001) and anxiety (β = 0.404, p < 0.001). This model with two factors (functional disability and anxiety) explained 56.7% of the variance in patients with diffuse SSc and 73.2% in those with localized SSc. CONCLUSIONS: Functional disability and anxiety are significantly associated with quality of life in SSc. Interventions aimed at improving either of these factors may contribute towards improving the quality of life of people with SSc. FAU - Sierakowska, Matylda AU - Sierakowska M AUID- ORCID: 0000-0003-2816-8588 AD - Department of Integrated Medical Care, Medical University of Bialystok, 7a Maria Sklodowska-Curie Street, 15-096, Bialystok, Poland. matyldasierakowska@gmail.com. FAU - Doroszkiewicz, Halina AU - Doroszkiewicz H AUID- ORCID: 0000-0002-5949-758X AD - Department of Geriatrics, Medical University of Bialystok, Bialystok, Poland. FAU - Sierakowska, Justyna AU - Sierakowska J AUID- ORCID: 0000-0003-0446-7188 AD - Department of Foreign Languages, Medical University of Bialystok, Bialystok, Poland. FAU - Olesińska, Marzena AU - Olesińska M AD - Department of Connective Tissue Disease, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. FAU - Grabowska-Jodkowska, Agnieszka AU - Grabowska-Jodkowska A AD - Department of Connective Tissue Disease, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. FAU - Brzosko, Marek AU - Brzosko M AD - Department of Rheumatology, Internal Diseases and Geriatrics, Pomeranian Medical University in Szczecin, Szczecin, Poland. FAU - Leszczyński, Piotr AU - Leszczyński P AUID- ORCID: 0000-0003-3096-5375 AD - Department of Rheumatology and Rehabilitation, Medical University in Poznań, Poznań, Poland. FAU - Pawlak-Buś, Katarzyna AU - Pawlak-Buś K AD - Department of Rheumatology and Rehabilitation, Medical University in Poznań, Poznań, Poland. FAU - Batko, Bogdan AU - Batko B AD - Center of Rheumatology, J. Dietl Hospital in Krakow, Krakow, Poland. FAU - Wiland, Piotr AU - Wiland P AD - Department of Rheumatology and Internal Diseases and Geriatrics, Medical University in Wroclaw, Wroclaw, Poland. FAU - Majdan, Maria AU - Majdan M AUID- ORCID: 0000-0002-4345-1675 AD - Department of Rheumatology and Connective Tissue Diseases, Medical University in Lublin, Lublin, Poland. FAU - Bykowska-Sochacka, Małgorzata AU - Bykowska-Sochacka M AD - Dr J. Titz-Kosko Regional Hospital for Rheumatic Diseases, Sopot, Poland. FAU - Romanowski, Wojciech AU - Romanowski W AD - Poznań Centre of Rheumatology, Śrem, Poland. FAU - Zon-Giebel, Aleksandra AU - Zon-Giebel A AD - Silesian Center of Rheumatology, Rehabilitation and Prevention of Disability, Ustroń, Poland. FAU - Jeka, Sławomir AU - Jeka S AD - Department of Rheumatology and Connective Tissue Diseases, 2nd University Hospital in Bydgoszcz, Bydgoszcz, Poland. FAU - Ndosi, Mwidimi AU - Ndosi M AUID- ORCID: 0000-0002-7764-3173 AD - Department of Nursing and Midwifery, University of the West of England, Bristol, UK. LA - eng GR - UMB (N/ST/ZB/16/002/3310)/Medical University of Bialystok, Poland/ PT - Journal Article PT - Multicenter Study DEP - 20190903 PL - Netherlands TA - Qual Life Res JT - Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation JID - 9210257 SB - IM MH - Anxiety/diagnosis MH - Anxiety Disorders/diagnosis MH - Cross-Sectional Studies MH - Depression/diagnosis MH - Depressive Disorder/diagnosis MH - *Disability Evaluation MH - Fatigue/diagnosis MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pain/diagnosis MH - Poland MH - Quality of Life/*psychology MH - Scleroderma, Systemic/*psychology MH - Surveys and Questionnaires PMC - PMC6863937 OTO - NOTNLM OT - Anxiety OT - Physical disability OT - Quality of life OT - Systemic sclerosis COIS- Author M.S. declares that she has no conflict of interest. Author H.D. declares that she has no conflict of interest. Author J.S. declares that she has no conflict of interest. Author M.O. declares that she has no conflict of interest. Author A.G-J. declares that she has no conflict of interest. Author M.B. declares that he has no conflict of interest. Author P.L. declares that he has no conflict of interest. Author K.P-B. declares that she has no conflict of interest. Author B.B. declares that he has no conflict of interest. Author P.W. declares that he has no conflict of interest. Author M.M. declares that she has no conflict of interest. Author M.B-S. declares that she has no conflict of interest. Author W.R. declares that he has no conflict of interest. Author A.Z.-G. declares that she has no conflict of interest. Author S.J. declares that he has no conflict of interest. Author M.N. declares that he has no conflict of interest. EDAT- 2019/09/05 06:00 MHDA- 2020/02/06 06:00 PMCR- 2019/09/03 CRDT- 2019/09/05 06:00 PHST- 2019/08/23 00:00 [accepted] PHST- 2019/09/05 06:00 [pubmed] PHST- 2020/02/06 06:00 [medline] PHST- 2019/09/05 06:00 [entrez] PHST- 2019/09/03 00:00 [pmc-release] AID - 10.1007/s11136-019-02284-9 [pii] AID - 2284 [pii] AID - 10.1007/s11136-019-02284-9 [doi] PST - ppublish SO - Qual Life Res. 2019 Dec;28(12):3347-3354. doi: 10.1007/s11136-019-02284-9. Epub 2019 Sep 3. PMID- 33193443 OWN - NLM STAT- MEDLINE DCOM- 20210623 LR - 20210623 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 11 DP - 2020 TI - Primary Sjögren's Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development. PG - 594096 LID - 10.3389/fimmu.2020.594096 [doi] LID - 594096 AB - OBJECTIVES: To study the clinical, serological and histologic features of primary Sjögren's syndrome (pSS) patients with early (young ≤35 years) or late (old ≥65 years) onset and to explore the differential effect on lymphoma development. METHODS: From a multicentre study population of 1997 consecutive pSS patients, those with early or late disease onset, were matched and compared with pSS control patients of middle age onset. Data driven analysis was applied to identify the independent variables associated with lymphoma in both age groups. RESULTS: Young pSS patients (19%, n = 379) had higher frequency of salivary gland enlargement (SGE, lymphadenopathy, Raynaud's phenomenon, autoantibodies, C4 hypocomplementemia, hypergammaglobulinemia, leukopenia, and lymphoma (10.3% vs. 5.7%, p = 0.030, OR = 1.91, 95% CI: 1.11-3.27), while old pSS patients (15%, n = 293) had more frequently dry mouth, interstitial lung disease, and lymphoma (6.8% vs. 2.1%, p = 0.011, OR = 3.40, 95% CI: 1.34-8.17) compared to their middle-aged pSS controls, respectively. In young pSS patients, cryoglobulinemia, C4 hypocomplementemia, lymphadenopathy, and SGE were identified as independent lymphoma associated factors, as opposed to old pSS patients in whom SGE, C4 hypocomplementemia and male gender were the independent lymphoma associated factors. Early onset pSS patients displayed two incidence peaks of lymphoma within 3 years of onset and after 10 years, while in late onset pSS patients, lymphoma occurred within the first 6 years. CONCLUSION: Patients with early and late disease onset constitute a significant proportion of pSS population with distinct clinical phenotypes. They possess a higher prevalence of lymphoma, with different predisposing factors and lymphoma distribution across time. CI - Copyright © 2020 Goules, Argyropoulou, Pezoulas, Chatzis, Critselis, Gandolfo, Ferro, Binutti, Donati, Zandonella Callegher, Venetsanopoulou, Zampeli, Mavrommati, Voulgari, Exarchos, Mavragani, Baldini, Skopouli, Fotiadis, De Vita, Moutsopoulos and Tzioufas. FAU - Goules, Andreas V AU - Goules AV AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Argyropoulou, Ourania D AU - Argyropoulou OD AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Pezoulas, Vasileios C AU - Pezoulas VC AD - Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Ioannina, Greece. FAU - Chatzis, Loukas AU - Chatzis L AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Critselis, Elena AU - Critselis E AD - Proteomics Facility, Center for Systems Biology, Biomedical Research Foundation of the Academy of Athens, Athens, Greece. AD - Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece. FAU - Gandolfo, Saviana AU - Gandolfo S AD - Rheumatology Clinic, Department of Medical Area, University of Udine, Udine, Italy. FAU - Ferro, Francesco AU - Ferro F AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Binutti, Marco AU - Binutti M AD - Rheumatology Clinic, Department of Medical Area, University of Udine, Udine, Italy. FAU - Donati, Valentina AU - Donati V AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Zandonella Callegher, Sara AU - Zandonella Callegher S AD - Rheumatology Clinic, Department of Medical Area, University of Udine, Udine, Italy. FAU - Venetsanopoulou, Aliki AU - Venetsanopoulou A AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Zampeli, Evangelia AU - Zampeli E AD - Institute for Autoimmune Systemic and Neurological Diseases, Athens, Greece. FAU - Mavrommati, Maria AU - Mavrommati M AD - Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece. FAU - Voulgari, Paraskevi V AU - Voulgari PV AD - Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, Greece. FAU - Exarchos, Themis AU - Exarchos T AD - Department of Informatics, Ionian University, Corfu, Greece. FAU - Mavragani, Clio P AU - Mavragani CP AD - Department of Physiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Baldini, Chiara AU - Baldini C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Skopouli, Fotini N AU - Skopouli FN AD - Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece. FAU - Fotiadis, Dimitrios I AU - Fotiadis DI AD - Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Ioannina, Greece. AD - Department of Biomedical Research, Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology - Hellas, Ioannina, Greece. FAU - De Vita, Salvatore AU - De Vita S AD - Rheumatology Clinic, Department of Medical Area, University of Udine, Udine, Italy. FAU - Moutsopoulos, Haralampos M AU - Moutsopoulos HM AD - Institute for Autoimmune Systemic and Neurological Diseases, Athens, Greece. AD - Chair Medical Sciences/Immunology, Academy of Athens, Athens, Greece. FAU - Tzioufas, Athanasios G AU - Tzioufas AG AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. AD - Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20201019 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 SB - IM MH - Adult MH - Age Factors MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Case-Control Studies MH - Disease Susceptibility MH - Female MH - Humans MH - Lymphoma/epidemiology/etiology MH - Male MH - Middle Aged MH - Odds Ratio MH - Phenotype MH - Prevalence MH - Retrospective Studies MH - Sjogren's Syndrome/complications/diagnosis/*epidemiology/*etiology MH - Young Adult PMC - PMC7604905 OTO - NOTNLM OT - age group OT - clinical phenotype characteristics OT - data driven analysis OT - lymphoma OT - primary Sjögren’s syndrome EDAT- 2020/11/17 06:00 MHDA- 2021/06/24 06:00 PMCR- 2020/01/01 CRDT- 2020/11/16 08:48 PHST- 2020/08/12 00:00 [received] PHST- 2020/09/28 00:00 [accepted] PHST- 2020/11/16 08:48 [entrez] PHST- 2020/11/17 06:00 [pubmed] PHST- 2021/06/24 06:00 [medline] PHST- 2020/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2020.594096 [doi] PST - epublish SO - Front Immunol. 2020 Oct 19;11:594096. doi: 10.3389/fimmu.2020.594096. eCollection 2020. PMID- 28303481 OWN - NLM STAT- MEDLINE DCOM- 20180129 LR - 20260127 IS - 1179-1888 (Electronic) IS - 1175-0561 (Print) IS - 1175-0561 (Linking) VI - 18 IP - 4 DP - 2017 Aug TI - Morphea and Eosinophilic Fasciitis: An Update. PG - 491-512 LID - 10.1007/s40257-017-0269-x [doi] AB - Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF. FAU - Mertens, Jorre S AU - Mertens JS AD - Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands. Jorre.Mertens@radboudumc.nl. AD - Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. Jorre.Mertens@radboudumc.nl. FAU - Seyger, Marieke M B AU - Seyger MMB AD - Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands. FAU - Thurlings, Rogier M AU - Thurlings RM AD - Department of Rheumatology, Radboud University Medical Centre, Nijmegen, The Netherlands. FAU - Radstake, Timothy R D J AU - Radstake TRDJ AD - Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. AD - Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - de Jong, Elke M G J AU - de Jong EMGJ AD - Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands. AD - Radboud University, Nijmegen, The Netherlands. LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Am J Clin Dermatol JT - American journal of clinical dermatology JID - 100895290 RN - 0 (Dermatologic Agents) RN - 0 (Glucocorticoids) RN - 143NQ3779B (calcipotriene) RN - FXC9231JVH (Calcitriol) RN - WM0HAQ4WNM (Tacrolimus) RN - YL5FZ2Y5U1 (Methotrexate) RN - Eosinophilic Fasciitis SB - IM MH - Administration, Cutaneous MH - Administration, Oral MH - Algorithms MH - Biopsy MH - Calcitriol/administration & dosage/*analogs & derivatives MH - Dermatologic Agents/*administration & dosage MH - Diagnosis, Differential MH - Disease Progression MH - Drug Therapy, Combination MH - *Eosinophilia/diagnosis/drug therapy/epidemiology MH - Evidence-Based Medicine MH - *Fasciitis/diagnosis/drug therapy/epidemiology MH - Glucocorticoids/*administration & dosage MH - Humans MH - Methotrexate/*administration & dosage MH - Phototherapy/methods MH - Practice Guidelines as Topic MH - Randomized Controlled Trials as Topic MH - *Scleroderma, Localized/classification/diagnosis/drug therapy/epidemiology MH - Skin/pathology MH - Tacrolimus/*administration & dosage MH - Treatment Outcome MH - United States/epidemiology PMC - PMC5506513 COIS- FUNDING: No funding was received for the preparation of this review. CONFLICT OF INTEREST: Jorre S. Mertens, Marieke M.B. Seyger, Rogier M. Thurlings, Timothy R.D.J. Radstake, and Elke M.G.J. de Jong have no conflicts of interest. EDAT- 2017/03/18 06:00 MHDA- 2018/01/30 06:00 PMCR- 2017/03/16 CRDT- 2017/03/18 06:00 PHST- 2017/03/18 06:00 [pubmed] PHST- 2018/01/30 06:00 [medline] PHST- 2017/03/18 06:00 [entrez] PHST- 2017/03/16 00:00 [pmc-release] AID - 10.1007/s40257-017-0269-x [pii] AID - 269 [pii] AID - 10.1007/s40257-017-0269-x [doi] PST - ppublish SO - Am J Clin Dermatol. 2017 Aug;18(4):491-512. doi: 10.1007/s40257-017-0269-x. PMID- 20391486 OWN - NLM STAT- MEDLINE DCOM- 20100519 LR - 20240324 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 62 IP - 3 DP - 2010 Mar TI - Possible protective effect of hydroxychloroquine on delaying the occurrence of integument damage in lupus: LXXI, data from a multiethnic cohort. PG - 393-400 LID - 10.1002/acr.20097 [doi] AB - OBJECTIVE: To determine the features predictive of time to integument damage in patients with systemic lupus erythematosus (SLE) from a multiethnic cohort (LUpus in MInorities, NAture versus nurture [LUMINA]). METHODS: LUMINA SLE patients (n = 580) age > or =16 years, with a disease duration of < or =5 years at baseline (T0), of African American, Hispanic, and Caucasian ethnicity were studied. Integument damage was defined per the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (scarring alopecia, extensive skin scarring, and skin ulcers lasting at least 6 months); factors associated with time to its occurrence were examined by Cox proportional univariable and multivariable (main model) hazards regression analyses. Two alternative models were also examined: in model 1, all patients, regardless of when integument damage occurred (n = 94), were included; in model 2, a time-varying approach (generalized estimating equation) was employed. RESULTS: Thirty-nine (6.7%) of 580 patients developed integument damage over a mean +/- SD total disease duration of 5.9 +/- 3.7 years, and were included in the main multivariable regression model. After adjusting for discoid rash, nailfold infarcts, photosensitivity, and Raynaud's phenomenon (significant in the univariable analyses), disease activity over time (hazard ratio [HR] 1.17, 95% confidence interval [95% CI] 1.09-1.26) was associated with a shorter time to integument damage, whereas hydroxychloroquine use (HR 0.23, 95% CI 0.12-0.47) and Texan-Hispanic (HR 0.35, 95% CI 0.14-0.87) and Caucasian ethnicities (HR 0.37, 95% CI 0.14-0.99) were associated with a longer time. Results of the alternative models were consistent with those of the main model, although in model 2, the association with hydroxychloroquine was not significant. CONCLUSION: Our data indicate that hydroxychloroquine use is possibly associated with a delay in integument damage development in patients with SLE. FAU - Pons-Estel, Guillermo J AU - Pons-Estel GJ AD - University of Alabama at Birmingham, USA. FAU - Alarcón, Graciela S AU - Alarcón GS FAU - González, Luis A AU - González LA FAU - Zhang, Jie AU - Zhang J FAU - Vilá, Luis M AU - Vilá LM FAU - Reveille, John D AU - Reveille JD FAU - McGwin, Gerald Jr AU - McGwin G Jr CN - Lumina Study Group LA - eng GR - M01-RR02558/RR/NCRR NIH HHS/United States GR - P20 RR011126/RR/NCRR NIH HHS/United States GR - P01-AR49084/AR/NIAMS NIH HHS/United States GR - P01 AR049084/AR/NIAMS NIH HHS/United States GR - M01-RR00032/RR/NCRR NIH HHS/United States GR - M01 RR002558/RR/NCRR NIH HHS/United States GR - 1P20RR11126/RR/NCRR NIH HHS/United States GR - M01 RR000032/RR/NCRR NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Antirheumatic Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Adult MH - Black or African American MH - Antirheumatic Agents/*therapeutic use MH - Disease Progression MH - Female MH - Hispanic or Latino MH - Humans MH - Hydroxychloroquine/*therapeutic use MH - Kaplan-Meier Estimate MH - Lupus Erythematosus, Systemic/complications/*drug therapy/ethnology MH - Male MH - Skin Diseases/complications/ethnology/*prevention & control MH - White People MH - Young Adult PMC - PMC3202433 MID - NIHMS162662 EDAT- 2010/04/15 06:00 MHDA- 2010/05/21 06:00 PMCR- 2011/10/26 CRDT- 2010/04/15 06:00 PHST- 2010/04/15 06:00 [entrez] PHST- 2010/04/15 06:00 [pubmed] PHST- 2010/05/21 06:00 [medline] PHST- 2011/10/26 00:00 [pmc-release] AID - 10.1002/acr.20097 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2010 Mar;62(3):393-400. doi: 10.1002/acr.20097. PMID- 19636015 OWN - NLM STAT- MEDLINE DCOM- 20090915 LR - 20260128 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 27 IP - 25 DP - 2009 Sep 1 TI - Chronic physical effects and health care utilization in long-term ovarian germ cell tumor survivors: a Gynecologic Oncology Group study. PG - 4142-9 LID - 10.1200/JCO.2008.20.9189 [doi] AB - PURPOSE: This study compares late effects of treatment on physical well-being and utilization of health care resources between ovarian germ cell tumor (OGCT) survivors and age/race/education-matched controls. PATIENTS AND METHODS: Eligible patients had OGCT treated with surgery and chemotherapy and were disease-free for at least 2 years at time of enrollment. The matched control group was selected from acquaintances recommended by survivors. Symptoms and function were measured using previously validated scales. Health care utilization was assessed by questions regarding health insurance coverage and health services utilization. RESULTS: One hundred thirty-two survivors and 137 controls completed the study. Survivors were significantly more likely to report a diagnosis of hypertension (17% v 8%, P = .02), and marginally hypercholesterolemia (9.8% v 4.4%, P = .09), and hearing loss (5.3% v 1.5%, P = .09) compared with controls. There were no significant differences in the rates of self-reported arthritis, heart, pulmonary or kidney disease, diabetes, non-OGCT malignancies, anxiety, hearing loss, or eating disorders between groups. Among chronic functional problems, numbness, tinnitus, nausea elicited by reminders of chemotherapy (v general nausea triggers for controls), and Raynaud's symptoms were reported more frequently by survivors. Patients who received vincristine, dactinomycin, and cyclophosphamide in addition to cisplatin therapy had increased functional complaints, particularly numbness and nausea. Health care utilization was similar, but 15.9% of survivors reported being denied health insurance versus 4.4% of controls (P < .001). CONCLUSION: Although a few sequelae of treatment persist, in general, OGCT survivors enjoy a healthy life comparable to that of controls. FAU - Matei, Daniela AU - Matei D AD - Indiana University Melvin and Bren Simon Cancer Center, 535 Barnhill Drive RT-473, Indianapolis, IN 46202, USA. dmatei@iupui.edu FAU - Miller, Anna M AU - Miller AM FAU - Monahan, Patrick AU - Monahan P FAU - Gershenson, David AU - Gershenson D FAU - Zhao, Qianqian AU - Zhao Q FAU - Cella, David AU - Cella D FAU - Champion, Victoria L AU - Champion VL FAU - Williams, Stephen D AU - Williams SD LA - eng GR - R01 CA077470/CA/NCI NIH HHS/United States GR - CA 37517/CA/NCI NIH HHS/United States GR - CA 27469/CA/NCI NIH HHS/United States GR - U10 CA027469/CA/NCI NIH HHS/United States GR - R01 CA 77470/CA/NCI NIH HHS/United States GR - U10 CA037517/CA/NCI NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural DEP - 20090727 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 SB - IM MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use MH - Case-Control Studies MH - Chemotherapy, Adjuvant MH - Chronic Disease MH - Delivery of Health Care/*statistics & numerical data MH - Disease-Free Survival MH - Female MH - Health Status MH - Humans MH - Insurance Coverage MH - Insurance, Health MH - Middle Aged MH - Neoplasms, Germ Cell and Embryonal/*therapy MH - Ovarian Neoplasms/complications/psychology/*therapy MH - *Ovariectomy/adverse effects MH - *Quality of Life MH - Surveys and Questionnaires MH - *Survivors/psychology MH - Time Factors MH - Treatment Outcome MH - United States MH - Young Adult PMC - PMC2734426 COIS- Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article. EDAT- 2009/07/29 09:00 MHDA- 2009/09/16 06:00 PMCR- 2010/09/01 CRDT- 2009/07/29 09:00 PHST- 2009/07/29 09:00 [entrez] PHST- 2009/07/29 09:00 [pubmed] PHST- 2009/09/16 06:00 [medline] PHST- 2010/09/01 00:00 [pmc-release] AID - JCO.2008.20.9189 [pii] AID - 09189 [pii] AID - 10.1200/JCO.2008.20.9189 [doi] PST - ppublish SO - J Clin Oncol. 2009 Sep 1;27(25):4142-9. doi: 10.1200/JCO.2008.20.9189. Epub 2009 Jul 27. PMID- 34290696 OWN - NLM STAT- MEDLINE DCOM- 20210928 LR - 20210928 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 12 DP - 2021 TI - Common Variable Immunodeficiency and Autoimmune Diseases: A Retrospective Study of 95 Adult Patients in a Single Tertiary Care Center. PG - 652487 LID - 10.3389/fimmu.2021.652487 [doi] LID - 652487 AB - Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency in adulthood, which presents a broad spectrum of clinical manifestations, often including non-infectious complications in addition to heightened susceptibility to infections. These protean manifestations may significantly complicate the differential diagnosis resulting in diagnostic delay and under-treatment with increased mortality and morbidity. Autoimmunity occurs in up to 30% of CVID patients, and it is an emerging cause of morbidity and mortality in this type of patients. 95 patients (42 males and 53 females) diagnosed with CVID, basing on ESID diagnostic criteria, were enrolled in this retrospective cohort study. Clinical phenotypes were established according to Chapel 2012: i) no other disease-related complications, ii) cytopenias (thrombocytopenia/autoimmune hemolytic anemia/neutropenia), iii) polyclonal lymphoproliferation (granuloma/lymphoid interstitial pneumonitis/persistent unexplained lymphadenopathy), and iv) unexplained persistent enteropathy. Clinical items in the analysis were age, gender, and clinical features. Laboratory data included immunoglobulin (Ig)G, IgM and IgA levels at diagnosis, flow-cytometric analysis of peripheral lymphocytes (CD3+, CD3+CD4+, CD3+CD8+, CD19+, CD4+CD25highCD127low, CD19hiCD21loCD38lo, and follicular T helper cell counts). Comparisons of continuous variables between groups were performed with unpaired t-test, when applicable. 39 patients (41%) showed autoimmune complications. Among them, there were 21 females (53.8%) and 18 males (46.2%). The most prevalent autoimmune manifestations were cytopenias (17.8%), followed by arthritis (11.5%), psoriasis (9.4%), and vitiligo (6.3%). The most common cytopenia was immune thrombocytopenia, reported in 10 out of 95 patients (10.5%), followed by autoimmune hemolytic anemia (n=3, 3.1%) and autoimmune neutropenia (n=3, 3.1%). Other autoimmune complications included thyroiditis, coeliac disease, erythema nodosum, Raynaud's phenomenon, alopecia, recurring oral ulcers, autoimmune gastritis, and primary biliary cholangitis. There were no statistically significant differences comparing immunoglobulin levels between CVID patients with or without autoimmune manifestations. There was no statistical difference in CD3+, CD8+, CD4+CD25highCD127low T, CD19, CD19hiCD21loCD38lo, and follicular T helper cell counts in CVID patients with or without autoimmune disorders. In conclusion, autoimmune manifestations often affect patients with CVID. Early recognition and tailored treatment of these conditions are pivotal to ensure a better quality of life and the reduction of CVID associated complications. CI - Copyright © 2021 Mormile, Punziano, Riolo, Granata, Williams, de Paulis, Spadaro and Rossi. FAU - Mormile, Ilaria AU - Mormile I AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Punziano, Alessandra AU - Punziano A AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Riolo, Carlo Alberto AU - Riolo CA AD - Post-Graduate Program in Clinical Immunology and Allergy, University of Naples Federico II, Naples, Italy. FAU - Granata, Francescopaolo AU - Granata F AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - Williams, Michela AU - Williams M AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. FAU - de Paulis, Amato AU - de Paulis A AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. FAU - Spadaro, Giuseppe AU - Spadaro G AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. FAU - Rossi, Francesca Wanda AU - Rossi FW AD - Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy. AD - Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy. LA - eng PT - Journal Article DEP - 20210705 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - 0 (Biomarkers) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoimmune Diseases/diagnosis/*epidemiology/immunology/therapy MH - Autoimmunity MH - Biomarkers MH - Common Variable Immunodeficiency/diagnosis/*epidemiology/immunology/therapy MH - Disease Management MH - Disease Susceptibility/immunology MH - Female MH - Humans MH - Italy/epidemiology MH - Male MH - Middle Aged MH - Phenotype MH - Prevalence MH - Retrospective Studies MH - Skin/pathology MH - Symptom Assessment MH - Tertiary Care Centers MH - Young Adult PMC - PMC8287325 OTO - NOTNLM OT - arthritis OT - autoimmunity OT - common variable immunodeficiency OT - cytopenia OT - psoriasis COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/07/23 06:00 MHDA- 2021/09/29 06:00 PMCR- 2021/01/01 CRDT- 2021/07/22 06:32 PHST- 2021/01/12 00:00 [received] PHST- 2021/06/15 00:00 [accepted] PHST- 2021/07/22 06:32 [entrez] PHST- 2021/07/23 06:00 [pubmed] PHST- 2021/09/29 06:00 [medline] PHST- 2021/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2021.652487 [doi] PST - epublish SO - Front Immunol. 2021 Jul 5;12:652487. doi: 10.3389/fimmu.2021.652487. eCollection 2021. PMID- 22176734 OWN - NLM STAT- MEDLINE DCOM- 20120924 LR - 20131121 IS - 1464-410X (Electronic) IS - 1464-4096 (Linking) VI - 110 IP - 1 DP - 2012 Jul TI - Safety and tolerability of local treatment with iloprost, a prostacyclin analogue, in patients with Peyronie's disease: a phase I study. PG - 117-21 LID - 10.1111/j.1464-410X.2011.10733.x [doi] AB - Study Type - Therapy (case series) Level of Evidence 4 What's known on the subject? and What does the study add? Peyronie's disease (PD) is an acquired curvature of the penis attributable to progressive fibromatosis of the tunica albuginea (TA). It is frequently associated with Dupuytren's contracture and those of Ledderhose. More recently it was found that patients suffering from PD also often suffer from diabetes mellitus and gout. Cigarette smoking and the intake of large amounts of alcohol are considered risk factors for PD.The exact aetiology of the disease is unknown, however, the trauma hypothesis is shared by most authors. According to this theory, repeated sexual microtrauma in people genetically predisposed could cause PD. The inflammatory process leads to the formation of fibrosis and plaques. Plaque can lead to penile curvature and may reduce its functionality. Pain is the most common symptom of early-stage disease. In the late stages the pain disappears, but erectile dysfunction may occur. Surgical treatment is available, but this exposes the patient to a greater risk of erectile dysfunction and it is most frequently associated with a reduction in the length of the penis. The rationale for local medical therapy is to use a treatment that acts on the initial phase of the disease by reducing and stopping the processes that lead to fibrosis, thus stabilizing the disease. Systemic medical therapy is usually accompanied by high rates of recurrence. Many authors consider local drug therapy more appropriate. Local treatment consists of several types of medication, but results are often sub-optimal. Anti-inflammatory or immunoregulatory therapy, either systemic or topical, has shown some efficacy when administered early in the disease by modulating the inflammatory response and attenuating the alteration of tissue repair. Unfortunately, in most cases, patients are first seen when the plaque is chronically inflamed, stabilized and sometimes already calcified. We have tested a biological drug for intralesional administration for the first time. We chose iloprost, an analogue of prostacyclin I2, for its theoretically favourable properties. If used i.v., it has been shown to be effective in treating vascular ischaemic disease such as thromboangioitis obliterans, peripheral arterial occlusive disease, Raynaud's phenomenon and systemic sclerosis. The rationale for the therapeutic use of iloprost in the late stages of PD is based on the assumption that activation of fibrinolysis induced by the drug would be able to determine a regression of the plaque with a consequent reduction of the curvature on erection. The main purpose of this phase I study was to evaluate the safety and tolerability of this drug injected in the context of the fibrous plaque on a small number of patients before designing a large-scale randomized trial. According to the results,therapy with intralesional iloprost in Peyronie's disease seems to be safe and tolerated and is a possible alternative to surgery. OBJECTIVE: To assess the safety and tolerability of intralesional injections of iloprost (an I2 prostacyclin analogue) for its ability to suppress the production of connective tissue growth factor in fibroblasts, for the treatment of Peyronie's disease (PD). PATIENTS AND METHODS: A total of 38 patients with PD were preliminarily assessed according to symptoms, the degree of penile curvature and the size and number of plaques. Each patient received weekly intralesional injections of iloprost 200 ng in 1 mL normal saline for 5 weeks. If tolerated, the single dose was increased weekly to the maximum of 400 ng (2 mL). All the patients were preliminarily evaluated using at-home photography and were re-evaluated 2 months after the end of the treatment regimen. There was no placebo control group in this phase I study. RESULTS: Almost all patients showed mild side effects (burning or pain) during the treatment at the site of injection. All patients tolerated well a iloprost dose of 200 ng; 19 patients reached a 300 ng dose and 14 tolerated a 400 ng dose without showing side effects. A total of 29% of the patients showed an improvement in curvature. CONCLUSION: The results show that therapy with intralesional iloprost is a possible alternative to surgery for Peyronie's disease. CI - 2011 BJU INTERNATIONAL. FAU - Pavone, Carlo AU - Pavone C AD - Urology Unit, University of Palermo 'P. Giaccone' Policlinic Hospital, Palermo, Italy. uropa@unipa.it FAU - Napoli, Giancarlo AU - Napoli G FAU - Caruana, Giovanni AU - Caruana G FAU - Alonge, Vincenza AU - Alonge V FAU - Usala, Manuela AU - Usala M FAU - Abbadessa, Daniela AU - Abbadessa D LA - eng PT - Clinical Trial, Phase I PT - Journal Article DEP - 20111216 PL - England TA - BJU Int JT - BJU international JID - 100886721 RN - DCR9Z582X0 (Epoprostenol) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Epoprostenol/*analogs & derivatives MH - Fibrinolysis/drug effects MH - Humans MH - Iloprost/administration & dosage/adverse effects/*therapeutic use MH - Injections, Intralesional MH - Male MH - Middle Aged MH - Penile Induration/*drug therapy EDAT- 2011/12/20 06:00 MHDA- 2012/09/25 06:00 CRDT- 2011/12/20 06:00 PHST- 2011/12/20 06:00 [entrez] PHST- 2011/12/20 06:00 [pubmed] PHST- 2012/09/25 06:00 [medline] AID - 10.1111/j.1464-410X.2011.10733.x [doi] PST - ppublish SO - BJU Int. 2012 Jul;110(1):117-21. doi: 10.1111/j.1464-410X.2011.10733.x. Epub 2011 Dec 16. PMID- 37385917 OWN - NLM STAT- MEDLINE DCOM- 20260310 LR - 20260310 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 116 DP - 2023 Oct TI - Multiple autoimmune syndrome: Clinical, immunological and genotypic characterization. PG - 119-130 LID - S0953-6205(23)00215-7 [pii] LID - 10.1016/j.ejim.2023.06.020 [doi] AB - INTRODUCTION: The existence of subphenotypes common to several autoimmune diseases (AIDs) suggests a shared physiopathology - autoimmune tautology. Multiple Autoimmune Syndrome (MAS) - the coexistence of three or more AIDs in one person-, best illustrates that polyautoimmunity is more than a coincidence. OBJECTIVES: Characterize and compare the monoautoimmune and MAS patients. Understand if clustering of AIDs leads to differences in disease severity, autoantibodies expression or genetic polymorphisms that could be markers for polyautoimmunity. METHODS: Currently adult patients were selected from unit cohort. MAS was assumed when ≥3 AIDs were present. 343 patients were included after exclusion criteria: having two AIDs or undetermined diagnosis. Clinical and immunological data were collected from medical files. HLA-DRB1 was genotyped by PCR-SSP methodology and PTPN22(rs2476601) polymorphisms by TaqMan Real Time PCR. Data were analysed using Chi-Square, Fisher's exact tests and logistic regression. Odds ratios (OR) and 95% confidence intervals were calculated. RESULTS: In comparison with control population: ELEVATED FREQUENCIES: HLA-DRB1*03 in study cohort (OR=3.68,p<0.001) and in monoautoimmune SLE (OR=2.79,p<0.001) and SjS (OR=8.27,p<0.001); HLA-DRB1*15 in monoautoimmune SjS (OR=2.39,p = 0.011); HLA-DRB1*16 in MAS SLE (OR=2.67,p = 0.031); PTPN22_T in all groups except monoautoimmune SjS and triple positive systemic MAS. DIMINISHED FREQUENCIES: HLA-DRB1*11 in study cohort (OR=0.57,p = 0.013), in MAS SLE (OR=0.39,p = 0.031) and monoautoimmune SjS (OR=0.10,p = 0.005); HLA-DRB1*13 in study cohort (OR=0.52,p = 0.001) and in monoautoimmune SLE (OR=0.53,p = 0.009) and SjS (OR=0.38,p = 0.031); HLA-DRB1*14 in study cohort (OR=0.32,p = 0.013) and monoautoimmune SLE (OR=0.21,p = 0.021); SLE group: HLA-DRB1*07 frequency was higher in monoautoimmune patients (OR=0.43,p = 0.023). MAS patients had significantly more NPSLE (OR=2.99,p<0.001), subacute cutaneous lesions (OR=2.30,p = 0.037), muscle&tendon (OR=2.00,p = 0.045), and haematological (OR=3.18,p = 0.006) involvement and Raynaud's (OR=2.94,p<0.001). SjS group: MAS patients had more frequently cryoglobulins (OR=2.96,p = 0.030), low complement (OR=2.43,p = 0.030) and Raynaud's (OR=4.38,p<0.001); monoautoimmune patients had more parotid enlargement (OR=0.12,p<0.001). APS group: MAS patients had more non-thrombotic manifestations (OR=4.69,p = 0.020) and Raynaud's (OR=9.12,p<0.001). Triple positive systemic MAS (SLE+SjS+APS) had more frequently severe kidney involvement (OR=11.67,p = 0.021) and CNS thrombosis (OR=4.44,p = 0.009). Anti-U1RNP increased frequency was transversally attributable to MAS. CONCLUSIONS: The coexistence of AIDs contributes to a more severe disease course. We confirmed previously established genetic risk and protection factors and suggest a new protective one - HLA-DRB1*14. HLA-DRB1*07 and anti-U1RNP could be markers for mono and polyautoimmunity, respectively; HLA-DRB1*13 could be a predictor for vascular risk in patients with multiple AIDs. PTPN22(rs2476601) polymorphism could be associated with less severe disease. CI - Copyright © 2023. Published by Elsevier B.V. FAU - Fidalgo, Mariana AU - Fidalgo M AD - Internal Medicine Resident, Clinical Internship at Unidade de Imunologia Clínica (2), Portugal. Electronic address: marianafidalgo@gmail.com. FAU - Faria, Raquel AU - Faria R AD - Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine, Portugal. FAU - Carvalho, Cláudia AU - Carvalho C AD - Unit for Multidisciplinary Research in Biomedicine, Portugal; Laboratório de Imunogenética, Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Portugal. FAU - Carvalheiras, Graziela AU - Carvalheiras G AD - Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Portugal. FAU - Mendonça, Denisa AU - Mendonça D AD - Departamento de Estudos de Populações, Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Portugal; EpiUnit, Instituto de Saúde Pública, Universidade do Porto, Portugal. FAU - Farinha, Fátima AU - Farinha F AD - Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine, Portugal. FAU - da Silva, Berta Martins AU - da Silva BM AD - Unit for Multidisciplinary Research in Biomedicine, Portugal; Laboratório de Imunogenética, Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Portugal. FAU - Vasconcelos, Carlos AU - Vasconcelos C AD - Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine, Portugal. LA - eng PT - Journal Article DEP - 20230628 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (HLA-DRB1 Chains) RN - EC 3.1.3.48 (Protein Tyrosine Phosphatase, Non-Receptor Type 22) RN - EC 3.1.3.48 (PTPN22 protein, human) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Male MH - *HLA-DRB1 Chains/genetics MH - Female MH - Adult MH - Middle Aged MH - Genotype MH - *Protein Tyrosine Phosphatase, Non-Receptor Type 22/genetics MH - *Autoimmune Diseases/genetics/immunology MH - Lupus Erythematosus, Systemic/genetics/immunology MH - Autoantibodies/blood/immunology MH - Logistic Models MH - Case-Control Studies MH - Aged MH - Young Adult OTO - NOTNLM OT - Antiphospholipid syndrome OT - HLA-DRB1 OT - Multiple autoimmune syndrome OT - PTPN22 OT - Polyautoimmunity OT - Sjögren's syndrome OT - Systemic lupus erythematosus OT - Triple positive systemic MAS COIS- Declaration of Competing Interest The authors declare no conflicting interests. EDAT- 2023/06/30 01:06 MHDA- 2026/03/11 01:12 CRDT- 2023/06/29 21:57 PHST- 2023/02/27 00:00 [received] PHST- 2023/06/17 00:00 [revised] PHST- 2023/06/20 00:00 [accepted] PHST- 2026/03/11 01:12 [medline] PHST- 2023/06/30 01:06 [pubmed] PHST- 2023/06/29 21:57 [entrez] AID - S0953-6205(23)00215-7 [pii] AID - 10.1016/j.ejim.2023.06.020 [doi] PST - ppublish SO - Eur J Intern Med. 2023 Oct;116:119-130. doi: 10.1016/j.ejim.2023.06.020. Epub 2023 Jun 28. PMID- 28761166 OWN - NLM STAT- MEDLINE DCOM- 20190219 LR - 20190320 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 7 IP - 1 DP - 2017 Jul 31 TI - Anti-centromere antibody exhibits specific distribution levels among anti-nuclear antibodies and may characterize a distinct subset in rheumatoid arthritis. PG - 6911 LID - 10.1038/s41598-017-07137-4 [doi] LID - 6911 AB - Anti-centromere antibody (ACA) is one of the classical anti-nuclear antibody (ANA) staining patterns. However, characteristics of ACA in comparison with the other ANA patterns and clinical features of ACA-positive subjects have not been elucidated. Here, we examined all ANA patterns by indirect immunofluorescence for 859 rheumatoid arthritis (RA) patients. Together with the ANA data of 9,575 healthy volunteers, we compared distributions of the ANA levels. ACA was the only ANA that demonstrated a definite bimodal distribution of levels. ACA showed significantly higher levels than the other ANA staining patterns in both RA and healthy population (p < 0.0001). ACA-positivity was associated with old age and was observed more in females. We further recruited another cohort of 3,353 RA patients and confirmed the findings. ACA was also associated with Raynaud's phenomenon (p = 6.8 × 10(-11)) in RA. As a conclusion, ACA displays a specific ANA staining pattern with a bimodal distribution, and ACA-positive RA may constitute a distinct subset with specific clinical features. FAU - Kuramoto, Nobuo AU - Kuramoto N AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Ohmura, Koichiro AU - Ohmura K AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Ikari, Katsunori AU - Ikari K AD - Tokyo Woman's Medical University, Tokyo, Japan. FAU - Yano, Koichiro AU - Yano K AD - Tokyo Woman's Medical University, Tokyo, Japan. FAU - Furu, Moritoshi AU - Furu M AD - Department of the Control for Rheumatic Disease, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Yamakawa, Noriyuki AU - Yamakawa N AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Hashimoto, Motomu AU - Hashimoto M AD - Department of the Control for Rheumatic Disease, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Ito, Hiromu AU - Ito H AD - Department of the Control for Rheumatic Disease, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Fujii, Takao AU - Fujii T AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Murakami, Kosaku AU - Murakami K AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Nakashima, Ran AU - Nakashima R AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Imura, Yoshitaka AU - Imura Y AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Yukawa, Naoichiro AU - Yukawa N AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Yoshifuji, Hajime AU - Yoshifuji H AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Taniguchi, Atsuo AU - Taniguchi A AD - Tokyo Woman's Medical University, Tokyo, Japan. FAU - Momohara, Shigeki AU - Momohara S AD - Tokyo Woman's Medical University, Tokyo, Japan. FAU - Yamanaka, Hisashi AU - Yamanaka H AD - Tokyo Woman's Medical University, Tokyo, Japan. FAU - Matsuda, Fumihiko AU - Matsuda F AD - Department of Center for Genomic Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Mimori, Tsuneyo AU - Mimori T AD - Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan. AD - Department of the Control for Rheumatic Disease, Kyoto University Graduate School of Medicine, Kyoto, Japan. FAU - Terao, Chikashi AU - Terao C AD - Department of Center for Genomic Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan. a0001101@kuhp.kyoto-u.ac.jp. AD - Center for the Promotion of Interdisciplinary Education and Research, Kyoto University Graduate School of Medicine, Kyoto, Japan. a0001101@kuhp.kyoto-u.ac.jp. AD - Division of Rheumatology, Immunology, and Allergy, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, 02115, USA. a0001101@kuhp.kyoto-u.ac.jp. AD - Division of Genetics, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, 02115, USA. a0001101@kuhp.kyoto-u.ac.jp. AD - Program in Medical and Population Genetics, Broad Institute, Cambridge, MA, 02142, USA. a0001101@kuhp.kyoto-u.ac.jp. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20170731 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Age Factors MH - Age of Onset MH - Aged MH - Aged, 80 and over MH - Antibodies, Antinuclear/*metabolism MH - Arthritis, Rheumatoid/*classification/*immunology MH - Autoantibodies MH - Case-Control Studies MH - Centromere/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Sex Factors PMC - PMC5537247 COIS- The authors declare that they have no competing interests. EDAT- 2017/08/02 06:00 MHDA- 2019/03/21 06:00 PMCR- 2017/07/31 CRDT- 2017/08/02 06:00 PHST- 2016/12/20 00:00 [received] PHST- 2017/06/22 00:00 [accepted] PHST- 2017/08/02 06:00 [entrez] PHST- 2017/08/02 06:00 [pubmed] PHST- 2019/03/21 06:00 [medline] PHST- 2017/07/31 00:00 [pmc-release] AID - 10.1038/s41598-017-07137-4 [pii] AID - 7137 [pii] AID - 10.1038/s41598-017-07137-4 [doi] PST - epublish SO - Sci Rep. 2017 Jul 31;7(1):6911. doi: 10.1038/s41598-017-07137-4. PMID- 24704867 OWN - NLM STAT- MEDLINE DCOM- 20150323 LR - 20220409 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 13 IP - 9 DP - 2014 Sep TI - The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome. PG - 883-91 LID - S1568-9972(14)00101-3 [pii] LID - 10.1016/j.autrev.2014.03.004 [doi] AB - OBJECTIVE: To describe the clinical spectrum associated with aminoacyl-transfer RNA synthetase (ARS) autoantibodies in patients with idiopathic inflammatory myositis defined according to Peter and Bohan's criteria. METHODS: Cohort studies were selected from MEDLINE and Embase up to August 2013. Two investigators independently extracted data on study design, patient characteristics, and clinical features (interstitial lung disease [ILD], fever, mechanic's hands [MH], Raynaud's phenomenon [RPh], arthralgia, sclerodactyly, cancer and dermatomyositis-specific rash) according to the presence of myositis-specific (anti-aminoacyl-transfer RNA synthetase [ARS], anti-signal recognition particle [anti-SRP] and anti-Mi2) and myositis-associated (anti-PM/Scl, anti-U1-RNP and anti-Ku) autoantibodies. RESULTS: 27 studies (3487 patients) were included in the meta-analysis. Arthralgia (75%, CI 67-81) and ILD (69%, CI 63-74) were the most prevalent clinical signs associated with anti-ARS autoantibodies. Anti-Mi2 and anti-SRP autoantibodies were associated with few extramuscular signs. ARS autoantibodies were identified in 13% of patients with cancer-associated myositis (5-25). Patients with non-anti-Jo1 ARS had greater odds of presenting fever (RR 0.63, CI 0.52-0.90) and ILD (RR 0.87, CI 0.81-0.93) compared to those with anti-Jo1 autoantibodies. The frequencies of myositis (RR 1.60, CI 1.38-1.85), arthralgia (RR 1.52, CI 1.32-1.76) and MH (RR 1.47, CI 1.11-1.94) were almost 50% higher in patients with anti-Jo1 compared to non-anti-Jo1 ARS autoantibodies. Patients with anti-PM/Scl differed from those with anti-ARS autoantibodies by a greater prevalence of RPh (RR 0.70, CI 0.53-0.94) and sclerodactyly (RR 0.47, CI 0.25-0.89). ILD was less frequent in patients with anti-U1-RNP autoantibodies (RR 3.35, CI 1.07-10.43). No difference was observed between anti-ARS and myositis-associated autoantibodies for other outcomes. CONCLUSIONS: The presence of anti-ARS autoantibodies delimits a heterogeneous subset of patients with a high prevalence of myositis, MH, arthralgia in anti-Jo1 patients, and RPh and fever in non-anti-Jo1 patients. The clinical signs of populations positive for anti-PM/Scl and anti-ARS autoantibodies largely overlap, especially with regard to ILD, challenging the clinical delimitation of the antisynthetase syndrome. CI - Copyright © 2014 Elsevier B.V. All rights reserved. FAU - Lega, Jean-Christophe AU - Lega JC AD - Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France; UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Fabien, Nicole AU - Fabien N AD - Department of Immunology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Reynaud, Quitterie AU - Reynaud Q AD - Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Durieu, Isabelle AU - Durieu I AD - Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Durupt, Stéphane AU - Durupt S AD - Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Dutertre, Marine AU - Dutertre M AD - Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Cordier, Jean-François AU - Cordier JF AD - National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, University of Lyon, France. FAU - Cottin, Vincent AU - Cottin V AD - National Reference Centre for Rare Pulmonary Diseases, Department of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, University of Lyon, France. LA - eng PT - Journal Article PT - Meta-Analysis PT - Review DEP - 20140403 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/immunology MH - Autoantibodies/*immunology MH - Cohort Studies MH - Humans MH - Myositis/*immunology/mortality MH - Phenotype OTO - NOTNLM OT - Anti-PM/Scl autoantibodies OT - Anti-aminoacyl-tRNA synthetase autoantibodies OT - Interstitial lung disease OT - Myositis OT - Systemic sclerosis EDAT- 2014/04/08 06:00 MHDA- 2015/03/24 06:00 CRDT- 2014/04/08 06:00 PHST- 2014/03/06 00:00 [received] PHST- 2014/03/30 00:00 [accepted] PHST- 2014/04/08 06:00 [entrez] PHST- 2014/04/08 06:00 [pubmed] PHST- 2015/03/24 06:00 [medline] AID - S1568-9972(14)00101-3 [pii] AID - 10.1016/j.autrev.2014.03.004 [doi] PST - ppublish SO - Autoimmun Rev. 2014 Sep;13(9):883-91. doi: 10.1016/j.autrev.2014.03.004. Epub 2014 Apr 3. PMID- 29044628 OWN - NLM STAT- MEDLINE DCOM- 20180828 LR - 20180828 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 45 IP - 2 DP - 2018 Feb TI - Possible pro-inflammatory role of heparin-binding epidermal growth factor-like growth factor in the active phase of systemic sclerosis. PG - 182-188 LID - 10.1111/1346-8138.14088 [doi] AB - Heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) is a member of the EGF family growth factors, which affects multiple aspects of the wound healing process such as epithelialization, wound contraction and angiogenesis. In our study, we measured the serum HB-EGF levels of 51 systemic sclerosis (SSc) patients, which showed a significant increase compared with those of 20 normal subjects. Further analysis revealed a positive correlation between the HB-EGF level and pulmonary ground-glass score but no correlation between the former and pulmonary fibrosis score. Other findings include: a significant increase of serum sialylated carbohydrate antigen KL-6 levels and significant shortness of disease duration in the diffuse cutaneous SSc patients with elevated HB-EGF levels; and significantly higher HB-EGF levels in the presence of Raynaud's phenomenon, in that of telangiectasia, and in the absence of contracture of phalanges in all SSc patients. We then evaluated HB-EGF mRNA levels of fibroblasts harvested from skin samples of the SSc patients and those of foreskin-derived fibroblasts treated with transforming growth factor-β, both of which were significantly higher than each control. In conclusion, we speculate that HB-EGF plays a pro-inflammatory role in the active skin and lung lesions of SSc. CI - © 2017 Japanese Dermatological Association. FAU - Hirabayashi, Megumi AU - Hirabayashi M AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Asano, Yoshihide AU - Asano Y AUID- ORCID: 0000-0001-5560-9778 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yamashita, Takashi AU - Yamashita T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miura, Shunsuke AU - Miura S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Nakamura, Kouki AU - Nakamura K AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Taniguchi, Takashi AU - Taniguchi T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Saigusa, Ryosuke AU - Saigusa R AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Takahashi, Takehiro AU - Takahashi T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Ichimura, Yohei AU - Ichimura Y AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miyagawa, Takuya AU - Miyagawa T AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Yoshizaki, Ayumi AU - Yoshizaki A AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Miyagaki, Tomomitsu AU - Miyagaki T AUID- ORCID: 0000-0003-1879-3128 AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Sugaya, Makoto AU - Sugaya M AUID- ORCID: 0000-0002-1618-329X AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. FAU - Sato, Shinichi AU - Sato S AD - Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan. LA - eng PT - Journal Article DEP - 20171017 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (HBEGF protein, human) RN - 0 (Heparin-binding EGF-like Growth Factor) RN - 0 (MUC1 protein, human) RN - 0 (Mucin-1) RN - 0 (RNA, Messenger) RN - 0 (Transforming Growth Factor beta) SB - IM MH - Adult MH - Aged MH - Biopsy MH - Cells, Cultured MH - Female MH - Fibroblasts MH - Fibrosis MH - Heparin-binding EGF-like Growth Factor/*blood/genetics/metabolism MH - Humans MH - Lung/diagnostic imaging/*pathology MH - Male MH - Middle Aged MH - Mucin-1/blood MH - Pulmonary Fibrosis/blood/diagnostic imaging/*pathology MH - RNA, Messenger/metabolism MH - Respiratory Function Tests MH - Scleroderma, Systemic/blood/diagnostic imaging/*pathology MH - Skin/cytology/*pathology MH - Transforming Growth Factor beta/metabolism OTO - NOTNLM OT - heparin-binding epidermal growth factor-like growth factor OT - inflammation OT - interstitial lung disease OT - systemic sclerosis OT - wound healing EDAT- 2017/10/19 06:00 MHDA- 2018/08/29 06:00 CRDT- 2017/10/19 06:00 PHST- 2017/07/30 00:00 [received] PHST- 2017/09/06 00:00 [accepted] PHST- 2017/10/19 06:00 [pubmed] PHST- 2018/08/29 06:00 [medline] PHST- 2017/10/19 06:00 [entrez] AID - 10.1111/1346-8138.14088 [doi] PST - ppublish SO - J Dermatol. 2018 Feb;45(2):182-188. doi: 10.1111/1346-8138.14088. Epub 2017 Oct 17. PMID- 34146098 OWN - NLM STAT- MEDLINE DCOM- 20220314 LR - 20260518 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 61 IP - 3 DP - 2022 Mar 2 TI - Digital pitting scars are associated with a severe disease course and death in systemic sclerosis: a study from the EUSTAR cohort. PG - 1141-1147 LID - 10.1093/rheumatology/keab510 [doi] AB - OBJECTIVE: Digital pitting scars (DPS) are frequent, but little studied in SSc to date. METHODS: An analysis of SSc patients enrolled in the EUSTAR database. Primary objectives were to (i) examine DPS prevalence; (ii) examine whether DPS are associated with digital ulcers (DUs) and active digital ischaemia (DUs or gangrene); and (iii) describe other associations with DPS including internal organ complications. Secondary objectives were whether DPS are associated with (i) functional impairment; (ii) structural microvascular disease; and (iii) mortality. Descriptive statistics and parametric/non-parametric tests were used. Binary logistic regression was used to examine the association between DPS and DUs, active digital ischaemia and mortality. RESULTS: A total of 9671 patients were included with reported DPS at any time point (n = 4924) or 'never' DPS (n = 4747). The majority (86.9%) were female and mean age was 55.7 years. DPS were associated with longer disease and Raynaud's duration (both P ≤ 0.001). DPS were associated with interstitial lung disease, pulmonary hypertension, conduction blocks, telangiectases, calcinosis (all P ≤ 0.001) and joint synovitis (P = 0.021). Patients were more likely to have more severe capillaroscopic abnormality and greater hand functional impairment. Multivariable logistic regression analyses showed that DPS were associated (odds ratio) with DUs: 22.03 (19.51-24.87), active digital ischaemia: 6.30 (5.34-7.42) and death: 1.86 (1.48-2.36). CONCLUSION: DPS are associated with a severe disease course including death. The impact of DPS on hand function and ischaemia is significant. The presence of DPS should alert the clinician to a poor prognosis and need to optimize the therapeutic strategy. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield. FAU - Heal, Calvin AU - Heal C AD - Centre for Biostatistics, University of Manchester, Manchester, UK. FAU - Henes, Jörg AU - Henes J AUID- ORCID: 0000-0001-5940-3195 AD - Centre for Interdisciplinary Clinical Immunology, Rheumatology and Autoinflammatory Diseases and Department of Internal Medicine II (Hematology, Oncology, Immunology and Rheumatology), University Hospital Tuebingen, Tuebingen, Germany. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B. Shine Rheumatology Institute, Rambam Health Care Campus and Rappaport Faculty of Medicine - Technion, Haifa, Israel. FAU - Distler, Jörg H W AU - Distler JHW AD - Department of Internal Medicine 3 and Institute for Clinical Immunology, University Hospital Erlangen-Nuremberg, Erlangen, Germany. FAU - Airò, Paolo AU - Airò P AD - Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology, University of Cologne, Cologne, Germany. FAU - Kerzberg, Eduardo AU - Kerzberg E AD - Servicio de Reumatología, Hospital J. M. Ramos Mejía, Urquiza 609, Buenos Aires, Argentina. FAU - Rudnicka, Lidia AU - Rudnicka L AD - Department of Dermatology, Medical University of Warsaw, Warsaw, Poland. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Department of Rheumatology, Bordeaux University Hospital, Bordeaux, France. FAU - Stebbings, Simon AU - Stebbings S AD - Department of Medicine, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand. FAU - Tanaka, Yoshiya AU - Tanaka Y AUID- ORCID: 0000-0002-0807-7139 AD - Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Hoffman-Vold, Anna Maria AU - Hoffman-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. FAU - Gabrielli, Armando AU - Gabrielli A AD - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. CN - EUSTAR Collaborators LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Aged MH - Cicatrix/*etiology MH - Disease Progression MH - Female MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications/*mortality MH - Skin Ulcer/*etiology OTO - NOTNLM OT - SSc OT - death OT - digital ischaemia OT - digital pitting scars OT - scleroderma OT - vasculopathy FIR - Ananieva, Lidia P IR - Ananieva LP FIR - de Vries-Bouwstra, Jeska IR - de Vries-Bouwstra J FIR - Bruni, Cosimo IR - Bruni C FIR - Cantatore, Francesco Paolo IR - Cantatore FP FIR - Castellví, Ivan IR - Castellví I FIR - Cutolo, Maurizio IR - Cutolo M FIR - Damjanov, Nemanja IR - Damjanov N FIR - Farge-Bancel, Dominique IR - Farge-Bancel D FIR - Gheorghiu, Ana Maria IR - Gheorghiu AM FIR - Hachulla, Eric IR - Hachulla E FIR - Hsu, Vivien M IR - Hsu VM FIR - Iannone, Florenzo IR - Iannone F FIR - Ingegnoli, Francesca IR - Ingegnoli F FIR - Ionescu, Ruxandra IR - Ionescu R FIR - de la Peña Lefebvre, Paloma García IR - de la Peña Lefebvre PG FIR - Li, Mengtao IR - Li M FIR - Mohamed, Walid Ahmed Abdel Atty IR - Mohamed WAAA FIR - Montecucco, Carlomaurizio IR - Montecucco C FIR - Mouthon, Luc IR - Mouthon L FIR - Pellerito, Raffaele IR - Pellerito R FIR - Randone, Silvia Bellando IR - Randone SB FIR - Riccieri, Valeria IR - Riccieri V FIR - Riemekasten, Gabriela IR - Riemekasten G FIR - Siegert, Elise IR - Siegert E FIR - Spertini, François IR - Spertini F FIR - Smith, Vanessa IR - Smith V FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - Tikly, Mohammed IR - Tikly M FIR - Ullman, Susanne IR - Ullman S FIR - Vanthuyne, Marie IR - Vanthuyne M FIR - Walker, Ulrich IR - Walker U EDAT- 2021/06/20 06:00 MHDA- 2022/03/15 06:00 CRDT- 2021/06/19 12:06 PHST- 2021/02/11 00:00 [received] PHST- 2021/06/14 00:00 [revised] PHST- 2021/06/20 06:00 [pubmed] PHST- 2022/03/15 06:00 [medline] PHST- 2021/06/19 12:06 [entrez] AID - 6306424 [pii] AID - 10.1093/rheumatology/keab510 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 Mar 2;61(3):1141-1147. doi: 10.1093/rheumatology/keab510. PMID- 37541742 OWN - NLM STAT- MEDLINE DCOM- 20230807 LR - 20230810 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 3 DP - 2023 Aug TI - Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort. LID - 10.1136/rmdopen-2023-003121 [doi] LID - e003121 AB - OBJECTIVES: Patients with connective tissue diseases can develop interstitial lung disease (ILD), leading to a progressive fibrosing ILD (PF-ILD) phenotype in some cases. We aimed to investigate the occurrence of PF-ILD in idiopathic inflammatory myopathies (IIMs), and factors potentially predicting this phenotype. Secondary aims were to assess the radiological pattern and factors associated with IIMs-ILD. METHODS: Patients with IIMs from our multicentric prospective cohort were retrospectively evaluated. Data were recorded at IIMs and ILD diagnosis, and during follow-up. Patients with ILD were classified according to the predominant high-resolution CT (HRCT) pattern: non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP) and organising pneumonia (OP). PF-ILD was defined according to the 2022 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) guidelines. Univariate and multivariate analyses were performed to identify factors associated to ILD and to PF-ILD. RESULTS: Of 253 patients with IIMs, 125 (49%) had ILD: 99 (78%) at IIMs diagnosis and 26 (22%) during follow-up (21/26 within 5 years). Multivariate analysis identified anti-Jo-1, anti-MDA5, anti-Ro52, high score on manual muscle test, mechanic's hands and Raynaud's phenomenon as independently associated with ILD. The predominant HRCT pattern was NSIP (50% of patients), followed by UIP (28%) and OP (22%). At 1-year follow-up, PF-ILD occurred in 18% of IIMs-ILD. PF-ILD was predicted by anti-MDA5, heliotropic rash, xerostomia and xerophthalmia at univariate but not at multivariate analysis. CONCLUSION: Patients with IIM should be carefully screened for ILD at IIMs diagnosis and yearly during follow-up. All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Zanatta, Elisabetta AU - Zanatta E AUID- ORCID: 0000-0002-4845-5413 AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. FAU - Cocconcelli, Elisabetta AU - Cocconcelli E AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Castelli, Gioele AU - Castelli G AUID- ORCID: 0000-0002-5925-4035 AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Giraudo, Chiara AU - Giraudo C AD - Unit of Advanced Clinical and Translational Imaging, Department of Medicine - DIMED, Padova University Hospital, Padova, Italy. FAU - Fraia, Anna Sara AU - Fraia AS AD - Unit of Advanced Clinical and Translational Imaging, Department of Medicine - DIMED, Padova University Hospital, Padova, Italy. FAU - De Zorzi, Elena AU - De Zorzi E AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Gatto, Mariele AU - Gatto M AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. FAU - Ienna, Luana AU - Ienna L AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. FAU - Treppo, Elena AU - Treppo E AD - Division of Rheumatology, Department of Medicine (DAME), Academic Hospital "Santa Maria della Misericordia", ASUFC, University of Udine, Udine, Italy. FAU - Malandrino, Danilo AU - Malandrino D AD - Department of Experimental and Clinical Medicine, University of Firenze, and Interdisciplinary Internal Medicine Unit, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Firenze, Italy. FAU - Cereser, Lorenzo AU - Cereser L AD - Institute of Radiology, Department of Medicine (DAME), Academic Hospital "Santa Maria della Misericordia", ASUFC, University of Udine, Udine, Italy. FAU - Emmi, Giacomo AU - Emmi G AUID- ORCID: 0000-0001-9575-8321 AD - Department of Experimental and Clinical Medicine, University of Firenze, and Interdisciplinary Internal Medicine Unit, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Firenze, Italy. AD - Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, Victoria, Australia. FAU - Giannelli, Federico AU - Giannelli F AD - Department of Radiology, Azienda USL Toscana Centro, Mugello Hospital, Borgo San Lorenzo, Italy. FAU - Bellani, Serena AU - Bellani S AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Martini, Andrea AU - Martini A AD - Unit of Internal Medicine and Hepatology, Department of Medicine, University-Teaching Hospital of Padova, Padova, Italy. FAU - Moccaldi, Beatrice AU - Moccaldi B AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. FAU - Ghirardello, Anna AU - Ghirardello A AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. FAU - Avouac, Jérôme AU - Avouac J AD - Service de Rhumatologie, Hôpital Cochin, APHP, Université Paris Cité Faculté de Santé, Paris, Île-de-France, France. FAU - Quartuccio, Luca AU - Quartuccio L AUID- ORCID: 0000-0002-0134-6439 AD - Division of Rheumatology, Department of Medicine (DAME), Academic Hospital "Santa Maria della Misericordia", ASUFC, University of Udine, Udine, Italy. FAU - Allanore, Yannick AU - Allanore Y AD - Service de Rhumatologie, Hôpital Cochin, APHP, Université Paris Cité Faculté de Santé, Paris, Île-de-France, France. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy adoria@unipd.it. FAU - Spagnolo, Paolo AU - Spagnolo P AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Balestro, Elisabetta AU - Balestro E AD - Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Iaccarino, Luca AU - Iaccarino L AD - Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy. LA - eng PT - Journal Article PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM MH - Humans MH - Retrospective Studies MH - Prospective Studies MH - *Lung Diseases, Interstitial/complications/diagnosis/epidemiology MH - *Idiopathic Pulmonary Fibrosis MH - *Myositis/complications/diagnosis/epidemiology PMC - PMC10407351 OTO - NOTNLM OT - autoimmune diseases OT - dermatomyositis OT - polymyositis OT - pulmonary fibrosis COIS- Competing interests: EZ and EB report personal and consulting fees from Boehringer Ingelheim. JA and YA report consulting fees from Boehringer Ingelheim. PS reports institutional grants and personal fees from PPM Services, Boehringer Ingelheim and Roche; consulting fees from Boehringer Ingelheim; non-financial support from PPM Services and Boehringer Ingelheim, and personal fees from Galapagos, Chiesi, Novartis, Lupin, Pieris and Santhera Pharmaceuticals. EDAT- 2023/08/05 05:42 MHDA- 2023/08/07 06:41 PMCR- 2023/08/04 CRDT- 2023/08/04 21:02 PHST- 2023/03/02 00:00 [received] PHST- 2023/07/17 00:00 [accepted] PHST- 2023/08/07 06:41 [medline] PHST- 2023/08/05 05:42 [pubmed] PHST- 2023/08/04 21:02 [entrez] PHST- 2023/08/04 00:00 [pmc-release] AID - rmdopen-2023-003121 [pii] AID - 10.1136/rmdopen-2023-003121 [doi] PST - ppublish SO - RMD Open. 2023 Aug;9(3):e003121. doi: 10.1136/rmdopen-2023-003121. PMID- 42256631 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260608 LR - 20260608 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 11 IP - 1 DP - 2026 TI - Scleroderma Clinical Trials Consortium (SCTC) outcome instruments for evaluation of systemic sclerosis. PG - e000009 LID - 10.1136/jsrd-2026-000009 [doi] LID - e000009 AB - The Scleroderma Clinical Trials Consortium (SCTC) is committed to advancing the understanding and treatment of systemic sclerosis (SSc). This review focuses on the development and validation of various outcome instruments by the SCTC, which are crucial for evaluating the effectiveness of interventions in clinical trials and observational studies. Key instruments discussed include the Assessment of Systemic Sclerosis-Associated RAynaud's Phenomenon Questionnaire, the SCTC Radiologic Scoring System for calcinosis, the Mawdsley Questionnaire for calcinosis, the University of California Los Angeles SCTC Gastrointestinal Tract Instrument (GIT) 2.0, the SCTC Activity Index and the SCTC Damage Index. Additionally, the SCTC and World Scleroderma Foundation (WSF) Skin Scoring Certification is highlighted as an important resource for researchers. Each instrument's purpose, description and validation process are examined, underscoring their pivotal role in advancing SSc research and improving patient outcomes. CI - Copyright © Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Valenzuela, Antonia AU - Valenzuela A AUID- ORCID: 0000-0003-3357-9402 AD - Rheumatology and Clinical Immunology, Pontificia Universidad Catolica de Chile, Santiago de Chile, Santiago Metropolitan Region, Chile. AD - Red de Salud UC Christus, Santiago, Santiago Metropolitan Region, Chile. FAU - Chung, Lorinda AU - Chung L AD - Stanford University School of Medicine, Stanford, California, USA. AD - VA Palo Alto Health Care System, Palo Alto, California, USA. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, NHS North Bristol NHS Trust, Bristol, UK. AD - Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, Bristol, UK. FAU - Domsic, Robyn AU - Domsic R AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Saketkoo, Lesley AU - Saketkoo L AUID- ORCID: 0000-0002-1878-8591 AD - Medicine, New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, Louisiana, USA. AD - University Medical Center-Comprehensive Pulmonary Hypertension Center and Interstitiatial Lung Disease Clinic Programs, New Orleans, Louisiana, USA. FAU - Fligelstone, Kim AU - Fligelstone K AD - Patient Research Partner, London, UK. AD - Scleroderma & Raynaud UK (SRUK), London, UK. FAU - Russell, Anne-Marie AU - Russell AM AD - Birmingham City University School of Health Sciences, Birmingham, UK. AD - University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0001-6822-3401 AD - University of Michigan, Ann Arbor, Michigan, USA. FAU - Frech, Tracy AU - Frech T AUID- ORCID: 0000-0002-5472-3840 AD - Division of Rheumatology and Immunology, Vanderbilt University, Nashville, Tennessee, USA. FAU - Ross, Laura AU - Ross L AD - University of Melbourne VCCC, Parkville, Victoria, Australia. FAU - Nikpour, Mandana AU - Nikpour M AD - The University of Sydney School of Public Health, Sydney, New South Wales, Australia. AD - Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia. FAU - Denton, Christopher AU - Denton C AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, University College London Medical School, London, UK. FAU - Baron, Murray AU - Baron M AD - Sir Mortimer B Davis Jewish General Hospital, Montreal, Quebec, Canada. LA - eng PT - Journal Article PT - Review DEP - 20260526 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC13231724 OTO - NOTNLM OT - Outcome Assessment, Health Care OT - Patient Reported Outcome Measures OT - Randomized Controlled Trials OT - Severity of Illness Index EDAT- 2026/06/08 12:40 MHDA- 2026/06/08 12:41 PMCR- 2026/05/26 CRDT- 2026/06/08 07:42 PHST- 2026/04/10 00:00 [received] PHST- 2026/04/20 00:00 [accepted] PHST- 2026/06/08 12:41 [medline] PHST- 2026/06/08 12:40 [pubmed] PHST- 2026/06/08 07:42 [entrez] PHST- 2026/05/26 00:00 [pmc-release] AID - jsrd-2026-000009 [pii] AID - 10.1136/jsrd-2026-000009 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2026 May 26;11(1):e000009. doi: 10.1136/jsrd-2026-000009. eCollection 2026. PMID- 41029620 OWN - NLM STAT- MEDLINE DCOM- 20251001 LR - 20251003 IS - 1471-2415 (Electronic) IS - 1471-2415 (Linking) VI - 25 IP - 1 DP - 2025 Sep 30 TI - Permanent visual impairment following a Behçet's disease flare while on calcitonin gene-related peptide receptor antagonist therapy: a case report. PG - 518 LID - 10.1186/s12886-025-04322-2 [doi] LID - 518 AB - BACKGROUND: Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis that can involve both arteries and veins. Ocular involvement, including non-granulomatous panuveitis and occlusive retinal vasculitis, is common and a significant cause of morbidity. Erenumab is a monoclonal antibody targeting the calcitonin gene-related peptide (CGRP) receptor approved for migraine prevention Although it is generally well tolerated, recent concerns have emerged regarding its vasoconstrictive potential in patients with underlying vascular disorders. CASE PRESENTATION: We report a case of a 44-year-old woman with a history of BD, well-managed with azathioprine and methotrexate, who developed painless, bilateral subacute visual loss eleven days after receiving her second monthly dose of erenumab. This occurred during a BD flare marked by oral ulcers and severe migraine. Despite corticosteroid treatment, visual acuity did not improve. Erenumab was discontinued, but at her following visit four weeks later, vision remained impaired and visual fields showed blind spot enlargement and nonspecific defects. CONCLUSION: We hypothesize that the patient's visual loss was due to secondary ischemia from BD-associated small-vessel vasculitis, potentially exacerbated by CGRP receptor blockade from erenumab, which may have impaired compensatory vasodilation in the optic nerve or retinal circulation. This case represents the first report of permanent bilateral visual impairment associated with CGRP antagonist use in a patient with BD. Recent FDA labeling updates for CGRP-targeting agents now caution against use in patients with preexisting vascular disease, including risk of hypertension and Raynaud's phenomenon. This case underscores the need for heightened caution when prescribing CGRP inhibitors to patients with systemic vasculitic disorders and highlights the importance of further investigation to better define the safety profile of these agents in individuals with underlying vascular disease. CI - © 2025. The Author(s). FAU - Khan, Fawad A AU - Khan FA AUID- ORCID: 0000-0003-0486-5004 AD - The McCasland Family Comprehensive Headache Center, Department of Neurology, Ochsner Neuroscience Institute, Ochsner Health, 1514 Jefferson Highway, New Orleans, LA, USA. fakhan@ochsner.org. AD - The University of Queensland Medical School, Brisbane, Australia. fakhan@ochsner.org. FAU - Malik, Alaa AU - Malik A AD - Louisiana State University Health Sciences Center School of Medicine, New Orleans, LA, USA. FAU - Nelson, Evan AU - Nelson E AD - The McCasland Family Comprehensive Headache Center, Department of Neurology, Ochsner Neuroscience Institute, Ochsner Health, 1514 Jefferson Highway, New Orleans, LA, USA. FAU - Fahimdanesh, Kian AU - Fahimdanesh K AD - The University of Queensland Medical School, Brisbane, Australia. FAU - Kaur, Karmveer AU - Kaur K AD - The University of Queensland Medical School, Brisbane, Australia. FAU - Elison, Jasmine AU - Elison J AD - Retina and Vitreous Specialists New Orleans, New Orleans, LA, USA. FAU - Sayed, Mohamed AU - Sayed M AD - Department of Cell Biology & Anatomy, Louisiana State University Health Sciences Center, New Orleans, LA, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20250930 PL - England TA - BMC Ophthalmol JT - BMC ophthalmology JID - 100967802 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) RN - I5I8VB78VT (erenumab) SB - IM MH - Humans MH - Female MH - Adult MH - *Behcet Syndrome/drug therapy/complications MH - *Antibodies, Monoclonal, Humanized/adverse effects MH - *Calcitonin Gene-Related Peptide Receptor Antagonists/adverse effects/therapeutic use MH - Visual Acuity MH - *Vision Disorders/chemically induced PMC - PMC12487509 OTO - NOTNLM OT - Behçet’s disease OT - CGRP antagonist OT - Calcitonin Gene-Related peptide receptor OT - Erenumab OT - Migraine OT - Vasodilation OT - Vision loss COIS- Declarations. Ethics approval and consent to participate: Ethics approval – This case report was approved by Ochsner Neuroscience Research Review Committee. Consent for publication: Written informed consent for publication, including the use of clinical details and clinical images, was obtained from the patient discussed in the manuscript. Competing interest: Fawad A. Khan reported receiving institutional research support from Biohaven, Teva, Marinus, Lundbeck, Supernus, Neurona, and UCB; advisory fees from Eisai, Neurelis, Marinus, Epitel, SK Life Science, AbbVie, Pfizer; honorarium for speaker engagements from Amgen, UCB, SK Life Science, Lundbeck, Abbvie, Lilly USA, Pfizer. He serves on the Louisiana State Medical Advisory Board in a non-paid position. In non-compensated roles, he is a board member of the Deccan Alumni Association of North America. Alaa Malik, Evan Nelson, Kian Fahimdanesh, Karmveer Kaur, Jasmine Elison, Mohamed Sayed report no conflicts of interest. EDAT- 2025/10/01 06:29 MHDA- 2025/10/01 06:30 PMCR- 2025/09/30 CRDT- 2025/10/01 01:31 PHST- 2025/06/24 00:00 [received] PHST- 2025/08/05 00:00 [accepted] PHST- 2025/10/01 06:30 [medline] PHST- 2025/10/01 06:29 [pubmed] PHST- 2025/10/01 01:31 [entrez] PHST- 2025/09/30 00:00 [pmc-release] AID - 10.1186/s12886-025-04322-2 [pii] AID - 4322 [pii] AID - 10.1186/s12886-025-04322-2 [doi] PST - epublish SO - BMC Ophthalmol. 2025 Sep 30;25(1):518. doi: 10.1186/s12886-025-04322-2. PMID- 28456914 OWN - NLM STAT- MEDLINE DCOM- 20180705 LR - 20231112 IS - 1559-0267 (Electronic) IS - 1080-0549 (Print) IS - 1080-0549 (Linking) VI - 53 IP - 2 DP - 2017 Oct TI - Intracellular B Lymphocyte Signalling and the Regulation of Humoral Immunity and Autoimmunity. PG - 237-264 LID - 10.1007/s12016-017-8609-4 [doi] AB - B lymphocytes are critical for effective immunity; they produce antibodies and cytokines, present antigens to T lymphocytes and regulate immune responses. However, because of the inherent randomness in the process of generating their vast repertoire of antigen-specific receptors, B cells can also cause diseases through recognizing and reacting to self. Therefore, B lymphocyte selection and responses require tight regulation at multiple levels and at all stages of their development and activation to avoid diseases. Indeed, newly generated B lymphocytes undergo rigorous tolerance mechanisms in the bone marrow and, subsequently, in the periphery after their migration. Furthermore, activation of mature B cells is regulated through controlled expression of co-stimulatory receptors and intracellular signalling thresholds. All these regulatory events determine whether and how B lymphocytes respond to antigens, by undergoing apoptosis or proliferation. However, defects that alter regulated co-stimulatory receptor expression or intracellular signalling thresholds can lead to diseases. For example, autoimmune diseases can result from altered regulation of B cell responses leading to the emergence of high-affinity autoreactive B cells, autoantibody production and tissue damage. The exact cause(s) of defective B cell responses in autoimmune diseases remains unknown. However, there is evidence that defects or mutations in genes that encode individual intracellular signalling proteins lead to autoimmune diseases, thus confirming that defects in intracellular pathways mediate autoimmune diseases. This review provides a synopsis of current knowledge of signalling proteins and pathways that regulate B lymphocyte responses and how defects in these could promote autoimmune diseases. Most of the evidence comes from studies of mouse models of disease and from genetically engineered mice. Some, however, also come from studying B lymphocytes from patients and from genome-wide association studies. Defining proteins and signalling pathways that underpin atypical B cell response in diseases will help in understanding disease mechanisms and provide new therapeutic avenues for precision therapy. FAU - Taher, Taher E AU - Taher TE AD - Centre for Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University of London, Charterhouse Square, London, EC1M 6BQ, UK. FAU - Bystrom, Jonas AU - Bystrom J AD - Centre for Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University of London, Charterhouse Square, London, EC1M 6BQ, UK. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, University College London, London, UK. FAU - Isenberg, David A AU - Isenberg DA AD - Centre for Rheumatology, University College London, London, UK. FAU - Renaudineau, Yves AU - Renaudineau Y AD - Immunology Laboratory, University of Brest Medical School, Brest, France. FAU - Abraham, David J AU - Abraham DJ AD - Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, University College London, London, UK. FAU - Mageed, Rizgar A AU - Mageed RA AUID- ORCID: 0000-0003-1561-784X AD - Centre for Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University of London, Charterhouse Square, London, EC1M 6BQ, UK. r.a.mageed@qmul.ac.uk. LA - eng GR - UC5/Scleroderma and Raynaud's-UK/ PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Intracellular Signaling Peptides and Proteins) SB - IM MH - Animals MH - *Autoimmunity MH - B-Lymphocytes/*immunology MH - Humans MH - *Immunity, Humoral MH - *Immunomodulation MH - Intracellular Signaling Peptides and Proteins/metabolism MH - Signal Transduction/*immunology PMC - PMC5597704 OTO - NOTNLM OT - Autoimmune diseases OT - B lymphocytes OT - Intracellular signalling COIS- CONFLICT OF INTEREST: The authors declare that they have no conflict of interest. FUNDING: Dr. Taher E Taher was funded by grant UC5 from Scleroderma and Raynaud’s-UK (SR-UK) awarded to Professors Rizgar Mageed and David Abraham. ETHICAL APPROVAL: This article does not contain any studies with human participants or animals performed by any of the authors. INFORMED CONSENT: Informed consent was obtained from all individual participants included in the study. EDAT- 2017/05/01 06:00 MHDA- 2018/07/06 06:00 PMCR- 2017/04/29 CRDT- 2017/05/01 06:00 PHST- 2017/05/01 06:00 [pubmed] PHST- 2018/07/06 06:00 [medline] PHST- 2017/05/01 06:00 [entrez] PHST- 2017/04/29 00:00 [pmc-release] AID - 10.1007/s12016-017-8609-4 [pii] AID - 8609 [pii] AID - 10.1007/s12016-017-8609-4 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2017 Oct;53(2):237-264. doi: 10.1007/s12016-017-8609-4. PMID- 34841681 OWN - NLM STAT- MEDLINE DCOM- 20220314 LR - 20260514 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 25 IP - 2 DP - 2022 Feb TI - Baseline characteristics of systemic sclerosis patients with restrictive lung disease in a multi-center US-based longitudinal registry. PG - 163-174 LID - 10.1111/1756-185X.14253 [doi] AB - AIM: Interstitial lung disease (ILD) is the leading cause of disease-related death in systemic sclerosis (SSc). Here, we assess baseline characteristics of SSc subjects with and without restrictive lung disease (RLD) in a multi-center, US-based registry. METHODS: SSc patients within 5 years of disease onset were enrolled in the Collaborative National Quality and Efficacy Registry (CONQUER), a multi-center US-based registry of SSc study participants (age ≥ 18 years) enrolled at 13 expert centers. All subjects met 2013 American College of Rheumatology / European League Against Rheumatism criteria. Subjects with a pulmonary function test (PFT) at baseline before April 1, 2020 were included. High-resolution computed tomography scan of the chest was not available to characterize ILD for all subjects. RLD was defined as forced vital capacity (FVC) <80% or total lung capacity (TLC) <80% predicted. RESULTS: There were 160 (45%) SSc subjects characterized as having RLD. There was no significant difference in age, gender or disease duration. RLD subjects had a mean disease duration from date of first non-Raynaud's symptom of 2.6 years and a mean FVC% predicted of 67% at baseline. In multivariable analysis, non-White race, higher physician global health assessment and modified Medical Research Council (mMRC) dyspnea scores, were independently associated with RLD. In the subgroup of RLD subjects with ILD, ILD had a negative correlation with RNA polymerase III antibody. CONCLUSION: CONQUER is the largest, multi-center, prospective cohort of early SSc patients in the US. Non-White race was independently associated with RLD. In addition, 45% of CONQUER subjects already had RLD, highlighting the importance of screening for SSc-ILD at initial diagnosis. CI - © 2021 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Castelino, Flavia V AU - Castelino FV AUID- ORCID: 0000-0001-9862-0299 AD - Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA. FAU - VanBuren, John M AU - VanBuren JM AD - Department or Pediatrics, University of Utah, Salt Lake City, Utah, USA. FAU - Startup, Emily AU - Startup E AD - Department or Pediatrics, University of Utah, Salt Lake City, Utah, USA. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - The University of Texas Health Science Center at Houston, Houston, Texas, USA. FAU - Bernstein, Elana J AU - Bernstein EJ AUID- ORCID: 0000-0001-5560-6390 AD - Division of Rheumatology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, New York, USA. FAU - Chung, Lorinda AU - Chung L AUID- ORCID: 0000-0003-0072-6939 AD - Division of Rheumatology, Department of Medicine and Dermatology, Stanford University and Palo Alto Veterans Affairs Health Care System, Stanford, California, USA. FAU - Correia, Chase AU - Correia C AUID- ORCID: 0000-0001-9906-7093 AD - Division of Rheumatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA. FAU - Evnin, Luke B AU - Evnin LB AD - Scleroderma Research Foundation, San Francisco, California, USA. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - Division of Rheumatology, Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Salt Lake City, Utah, USA. FAU - Gordon, Jessica K AU - Gordon JK AD - Hospital for Special Surgery, New York, New York, USA. FAU - Hant, Faye N AU - Hant FN AD - Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, South Carolina, USA. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - University of Michigan Scleroderma Program, Ann Arbor, Michigan, USA. FAU - Sandorfi, Nora AU - Sandorfi N AD - Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA. FAU - Shah, Ami A AU - Shah AA AUID- ORCID: 0000-0002-1139-2009 AD - Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Shanmugam, Victoria K AU - Shanmugam VK AUID- ORCID: 0000-0002-5882-4884 AD - Division of Rheumatology, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA. FAU - Steen, Virginia AU - Steen V AUID- ORCID: 0000-0002-6333-0133 AD - Georgetown University School of Medicine, Washington, District of Columbia, USA. LA - eng GR - Scleroderma Research Foundation/ PT - Journal Article PT - Multicenter Study DEP - 20211128 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Adult MH - Female MH - Humans MH - Longitudinal Studies MH - Lung Diseases, Interstitial/diagnosis/*epidemiology/etiology MH - Male MH - Middle Aged MH - Registries MH - Scleroderma, Systemic/*epidemiology/physiopathology MH - Severity of Illness Index MH - United States/epidemiology MH - Vital Capacity OTO - NOTNLM OT - interstitial lung disease OT - registry OT - restrictive lung disease OT - scleroderma OT - systemic sclerosis EDAT- 2021/11/30 06:00 MHDA- 2022/03/15 06:00 CRDT- 2021/11/29 06:55 PHST- 2021/11/12 00:00 [revised] PHST- 2021/06/30 00:00 [received] PHST- 2021/11/12 00:00 [accepted] PHST- 2021/11/30 06:00 [pubmed] PHST- 2022/03/15 06:00 [medline] PHST- 2021/11/29 06:55 [entrez] AID - 10.1111/1756-185X.14253 [doi] PST - ppublish SO - Int J Rheum Dis. 2022 Feb;25(2):163-174. doi: 10.1111/1756-185X.14253. Epub 2021 Nov 28. PMID- 33331306 OWN - NLM STAT- MEDLINE DCOM- 20201218 LR - 20201219 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 52 IP - 6 DP - 2020 Dec 18 TI - [Significance of anti-tubulin-α-1C autoantibody in systemic sclerosis]. PG - 1009-1013 AB - OBJECTIVE: To detect the serum level of a novel autoantibody, anti-tubulin-α-1C, in patients with systemic sclerosis (SSc) and to investigate its clinical significance. METHODS: Anti-tubulin-α-1C antibody levels were determined by enzyme-linked immunosorbent assay (ELISA) in 62 patients with SSc, 38 systemic lupus erythematosus (SLE), 24 primary Sjögren's syndrome (pSS) patients, and 30 healthy controls (HCs). Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), immunoglobulin A(IgA), immunoglobulin M (IgM), immunoglobulin G (IgG), C3, C4, rheumatoid factor (RF), antinuclear antibody(ANA), anti-centromere antibodies(ACA), anticardiolipin (aCL), anti-dsDNA antibody, anti-Sm antibody, anti-RNP antibody, anti-Scl-70 antibody, anti-Ro52 antibody, anti-SSA antibody, anti-SSB antibody, centromere protein A(CENP-A), centromere protein B (CENP-B) were measured by standard laboratory techniques. Raynaud's phenomenon and modified Rodnan skin score(MRSS) were recorded to evaluate the disease status of SSc. Independent sample t test, Chi square test, Mann-Whitney U test, Spearman rank correlation were used for statistical analyses. RESULTS: The serum anti-tubulin-α-1C antibody concentration in SSc group was 81.24±34.38, the serum anti-tubulin-α-1C antibody concentration in SLE group was 87.84±38.52, the serum anti-tubulin-α-1C antibody concentration in pSS group was 59.79±25.24, and the serum anti-tubulin-α-1C antibody concentration in healthy group was 39.37±18.7. Multivariate analysis revealed that anti-tubulin-α-1C antibody levels were significantly increased in the SSc and SLE patients. The expression level of anti-tubulin-α-1C antibody in SSc was higher compared with the pSS group and the health control group (P < 0.01). Further analysis demonstrated that the elevated anti-tubulin-α-1C antibody were correlated with the SSc inflammation and disease activity markers ESR(r=0.313, P=0.019), The levels of anti-tubulin-α-1C antibody were also significantly correlated with MRSS(r=0.636, P < 0.01). The best cut-off value for the diagnose of SSc was 76.77 as mean+2SD value. The proportion of Raynaud's phenomenon was higher in the group of anti-tubulin-α-1C autoantibody-postive SSc patients than that in anti-tubulin-α-1C autoantibody negative group(71.4% vs. 37.5%, P=0.039). The proportions of anti-Scl-70 antibody, anti-CENP antibody and anti-cardiolipin antibody were higher in the group of anti-tubulin-α-1C autoantibody-postive SSc patients than in the anti-tubulin-α-1C autoantibody negative group (37.9% vs. 15.2%, 34.5% vs. 12.1%, 13.8 vs. 0, respectively, all P < 0.05). CONCLUSION: Based on this explorative stu-dy, the level of anti-tubulin-α-1C antibody increased in the serum of the patients with SSc. There were correlations between anti-tubulin-α-1C autoantibody and clinical and laboratory indicators of the SSc patients. It may become a novel biomarker indicative of active SSc and could be applied in future clinical practice. FAU - Zhao, J AU - Zhao J FAU - Sun, F AU - Sun F FAU - Li, Y AU - Li Y FAU - Zhao, X Z AU - Zhao XZ FAU - Xu, D AU - Xu D FAU - Li, Y N AU - Li YN FAU - Li, Y H AU - Li YH FAU - Sun, X L AU - Sun XL LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Antibodies, Antinuclear MH - Autoantibodies MH - Humans MH - *Lupus Erythematosus, Systemic MH - *Scleroderma, Systemic MH - *Sjogren's Syndrome PMC - PMC7745286 OTO - NOTNLM OT - Anti-tubulin-α-1C antibody OT - Autoantibodies OT - Systemic sclerosis EDAT- 2020/12/18 06:00 MHDA- 2020/12/19 06:00 PMCR- 2020/12/18 CRDT- 2020/12/17 08:42 PHST- 2020/12/17 08:42 [entrez] PHST- 2020/12/18 06:00 [pubmed] PHST- 2020/12/19 06:00 [medline] PHST- 2020/12/18 00:00 [pmc-release] AID - bjdxxbyxb-52-6-1009 [pii] AID - 10.19723/j.issn.1671-167X.2020.06.004 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1009-1013. doi: 10.19723/j.issn.1671-167X.2020.06.004. PMID- 35382633 OWN - NLM STAT- MEDLINE DCOM- 20220407 LR - 20220407 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 31 IP - 5 DP - 2022 Apr TI - Multicenter lupus register from Argentina, the RELESSAR database: Influence of ethnicity on disease phenotype. PG - 637-645 LID - 10.1177/09612033221083267 [doi] AB - OBJECTIVE: The objective is to describe the main characteristics of patients with systemic lupus erythematosus (SLE) in Argentina and to examine the influence of ethnicity on the expression of the disease. PATIENTS AND METHODS: RELESSAR is a multicentre register carried out by 106 researchers from 67 rheumatologic Argentine centres. It is a cross-sectional study of SLE (1982/1997 ACR) patients. RELESSAR electronic database includes demographic, cumulative SLE manifestations, SELENA-SLEDAI, SLICC-SDI, Katz's severity and Charlson's comorbidity indexes and treatment patterns. RESULTS: We included 1,610 patients, 91.7% were female with a median age at diagnosis of 28.1 ± 12.8; 96.2% met ≥4 ACR 1982/97 criteria. Frequent manifestations were arthritis (83.5%), malar rash (79.5%), photosensitivity (75.3%), haematological (63.8%) and renal disease (47.4%), antinuclear antibodies (96%), anti-dsDNA (66.5%) and anti-Smith antibodies (29%). The mean Selena-SLEDAI score at last visit was 3.18 (SD 4.3) and mean SDI was 1 (SD 1.3). The accumulated treatments most frequently used were antimalarials (90.4%), corticosteroids (90%), azathioprine (31.8%), intravenous cyclophosphamide (30.2%), mycophenolate mofetil or mycophenolic acid (24.5%), methotrexate (19.3%), belimumab 5.3% and rituximab 5.1%. Refractory lupus was diagnosed in 9.3% of the cases. The main causes of death were lupus activity (25.0%), activity and concomitant infections (25.0%), infections (18.2%), vascular disease (13.6%) and cancer (4.5%). Mortality was associated with higher SLEDAI, Katz, damage indexes and comorbidities. Of the 1610 patients included, 44.6% were Caucasian, 44.5% Mestizo, 8.1% Amerindian and 1.2% Afro-Latin American. Mestizo patients had higher male representation, low socioeconomic status, more inadequate medical coverage, fewer formal years of education and shorter disease duration. Polyadenopathies and Raynaud's phenomenon were more frequent in Caucasians. In the logistic regression analysis higher damage index (OR 1.28, CI 95% 1.02-1.61, p = 0.03) remained associated to mestizo ethnicity. CONCLUSIONS: This study represents the largest number of adult patients with SLE studied in Argentina. Caucasian patients were differentiated by having Raynaud's phenomenon and polyadenopathy more frequently, while patients of Mestizo origin had higher damage indexes. FAU - García, Mercedes A AU - García MA AUID- ORCID: 0000-0003-4773-9112 AD - 198367HIGA General San Martin, La Plata, Argentina. FAU - Alba, Paula AU - Alba P AD - Hospital Córdoba, Córdoba, Argentina. FAU - Del Campo-Perez, Victor AU - Del Campo-Perez V AD - Servicio de Medicina Preventiva y Epidemiología, 96682Complexo Hospitalario Universitario de Vigo, Vigo, España. FAU - Roverano, Susana AU - Roverano S AD - Hospital J.M. Cullen, Santa Fe, Argentina. FAU - Quintana, Rosana M AU - Quintana RM AUID- ORCID: 0000-0003-0643-2755 AD - Centro Regional de Enfermedades Autoinmunes y Reumáticas (CREAR), Rosario, Argentina. FAU - Alvarez, Analia P AU - Alvarez AP AD - Hospital Penna603552, Buenos Aires, Argentina. FAU - Graf, Cesar E AU - Graf CE AD - Centro Médico Mitre, Paraná, Argentina. FAU - Pisoni, Cecilia AU - Pisoni C AUID- ORCID: 0000-0001-6473-9857 AD - CEMIC62883, Buenos Aires, Argentina. FAU - Spindler, Alberto AU - Spindler A AD - Centro de Investigaciones Médicas Tucumán, Tucumán, Argentina. FAU - Gomez, Catalina AU - Gomez C AD - Hospital Central de Formosa, Formosa, Argentina. FAU - Figueredo, Heber M AU - Figueredo HM AD - Hospital de Alta Complejidad Pte. Juan Domingo Perón, Formosa, Argentina. FAU - Papasidero, Silvia AU - Papasidero S AD - Hospital General de Agudos Dr. Enrique Tornú580023, Buenos Aires, Argentina. FAU - Paniego, Raul AU - Paniego R AD - CePReR, Santa Rosa, Argentina. FAU - de la Vega, Maria C AU - de la Vega MC AD - CEIM Investigaciones Médicas, Buenos Aires, Argentina. FAU - Civit, Emma AU - Civit E AD - Hospital del Carmen, Godoy Cruz, Argentina. FAU - Gonzalez Lucero, Luciana AU - Gonzalez Lucero L AUID- ORCID: 0000-0003-4878-1603 AD - Hospital Angel C. Padilla, Tucumán, Argentina. FAU - Martire, Maria V AU - Martire MV AD - Hospital Italiano de La Plata, La Plata, Argentina. FAU - Aguila Maldonado, Rodrigo AU - Aguila Maldonado R AD - 198367HIGA General San Martin, La Plata, Argentina. FAU - Gordon, Sergio AU - Gordon S AD - HIGA Dr. Alende, Mar del Plata, Argentina. FAU - Gobbi, Carla AU - Gobbi C AD - Sanatorio Allende, Cordoba, Argentina. FAU - Micelli, Marina AU - Micelli M AD - 541318Hospital General de Agudos José María Ramos Mejía, Buenos Aires, Argentina. FAU - Nieto, Romina AU - Nieto R AD - Hospital Provincial de Rosario, Rosario, Argentina. FAU - Rausch, Gretel AU - Rausch G AD - Consultorio Privado, Rio Grande, Argentina. FAU - Gongora, Vanina AU - Gongora V AD - Hospital San Martin, Parana, Argentina. FAU - Damico, Agustina AU - Damico A AD - Hospital Interzonal Dr. José Penna, Bahía Blanca, Argentina. FAU - Dubinsky, Diana AU - Dubinsky D AD - Hospital Clínicas, Buenos Aires, Argentina. FAU - Orden, Alberto AU - Orden A AD - Hospital Aeronáutico Central, 433287 Buenos Aires, Argentina. FAU - Zacariaz, Johana AU - Zacariaz J AD - Hospital Italiano de Buenos Aires, 37533 Buenos Aires, Argentina. FAU - Romero, Julia AU - Romero J AD - Clínica Alvear San Juan, San Juan, Argentina. FAU - Pera, Mariana AU - Pera M AD - 198367HIGA General San Martin, La Plata, Argentina. FAU - Goñi, Mario AU - Goñi M AD - Centro de Especialidades Médicas Ambulatorias de Rosario, Rosario, Argentina. FAU - Rillo, Oscar AU - Rillo O AD - 58783Hospital General de Agudos Dr Ignacio Pirovano, Buenos Aires, Argentina. FAU - Baez, Roberto AU - Baez R AD - Hospital De General Roca, General Roca, Argentina. FAU - Arturi, Valeria AU - Arturi V AD - 198367HIGA General San Martin, La Plata, Argentina. FAU - Gonzalez, Andrea AU - Gonzalez A AD - Hospital Eva Perón, San Martín, Argentina. FAU - Vivero, Florencia AU - Vivero F AD - Hospital Privado de la Comunidad, Mar del Plata, Argentina. FAU - Bedoya, Maria E AU - Bedoya ME AD - Hospital San Martin, Paraná, Argentina. FAU - Shmid, Maria M AU - Shmid MM AD - Hospital Angela Iglesia de Llano, Corrientes, Argentina. FAU - Caputo, Victor AU - Caputo V AD - Sanatorio Juncal, Temperley, Argentina. FAU - Larroude, Maria S AU - Larroude MS AD - Consultorio Privado, Avellaneda, Argentina. FAU - Dominguez, Nadia AU - Dominguez N AD - 541318Hospital General de Agudos José María Ramos Mejía, Buenos Aires, Argentina. FAU - Gómez, Graciela N AU - Gómez GN AUID- ORCID: 0000-0003-4389-3083 AD - Instituto de Investigaciones Médicas Alfredo Lanari, 207909 Buenos Aires, Argentina. FAU - Rodriguez, Graciela N AU - Rodriguez GN AD - 198367HIGA General San Martin, La Plata, Argentina. FAU - Marin, Josefina AU - Marin J AD - Hospital Italiano de Buenos Aires, 37533 Buenos Aires, Argentina. FAU - Collado, Victoria AU - Collado V AD - Instituto de Investigaciones Médicas Alfredo Lanari, 207909 Buenos Aires, Argentina. FAU - Jorfen, Marisa AU - Jorfen M AD - Centro de Especialidades Médicas Ambulatorias, Rosario, Argentina. FAU - Bedran, Zaida AU - Bedran Z AD - Hospital Escuela de Agudos Dr. Ramón Madariaga, Posadas, Argentina. FAU - Curti, Ana AU - Curti A AD - Hospital Luis Carlos Lagomaggiore, 297830 Mendoza, Argentina. FAU - Gazzoni, Maria V AU - Gazzoni MV AD - Hospital Nacional de Clinicas, 63049 Córdoba, Argentina. FAU - Sarano, Judith AU - Sarano J AD - Instituto de Investigaciones Médicas Alfredo Lanari, 207909 Buenos Aires, Argentina. FAU - Zelaya, Marcos AU - Zelaya M AD - 541318Hospital General de Agudos José María Ramos Mejía, Buenos Aires, Argentina. FAU - Sacnun, Monica AU - Sacnun M AD - Hospital Provincial del Centenario, 541448 Rosario, Argentina. FAU - Finucci Curi, Pablo AU - Finucci Curi P AD - Hospital San Martin, Parana, Argentina. FAU - Rojas Tessel, Romina AU - Rojas Tessel R AD - 218812Hospital Señor del Milagro, Salta, Argentina. FAU - Arias Saavedra, Maira AU - Arias Saavedra M AD - 541318Hospital General de Agudos José María Ramos Mejía, Buenos Aires, Argentina. FAU - Sattler, Maria E AU - Sattler ME AD - Hospital Escuela Eva Peron, Granadero Baigorria, Argentina. FAU - Machado Escobar, Maximiliano A AU - Machado Escobar MA AD - Hospital Eva Perón, Tucumán, Argentina. FAU - Astesana, Pablo AU - Astesana P AD - Sanatorio Allende, Cordoba, Argentina. FAU - Paris, Ursula AU - Paris U AD - Hospital Escuela de Agudos Dr. Ramón Madariaga, Posadas, Argentina. FAU - Virasoro, Belen Maria AU - Virasoro BM AUID- ORCID: 0000-0002-2401-607X AD - CEMIC62883, Buenos Aires, Argentina. FAU - Santa Cruz, Maria J AU - Santa Cruz MJ AD - Hospital General de Agudos Dr. Enrique Tornú580023, Buenos Aires, Argentina. FAU - Allievi, Alberto AU - Allievi A AD - 28213Universidad del Salvador, Autoinmunidad, Buenos Aires, Argentina. FAU - Vandale, Juan M AU - Vandale JM AD - Hospital Privado de la Comunidad, Mar del Plata, Argentina. FAU - Hojberg, Noelia G AU - Hojberg NG AD - 541318Hospital General de Agudos José María Ramos Mejía, Buenos Aires, Argentina. FAU - Pons-Estel, Bernardo AU - Pons-Estel B AD - 198365Sanatorio Parque, Rosario, Argentina. LA - eng PT - Journal Article PT - Multicenter Study PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Argentina/epidemiology MH - Cross-Sectional Studies MH - *Ethnicity MH - Female MH - Humans MH - *Lupus Erythematosus, Systemic/complications MH - Male MH - Phenotype MH - Severity of Illness Index OTO - NOTNLM OT - Systemic lupus erythematosus OT - ethnicity OT - systemic lupus erythematosus in Argentina EDAT- 2022/04/07 06:00 MHDA- 2022/04/08 06:00 CRDT- 2022/04/06 05:20 PHST- 2022/04/06 05:20 [entrez] PHST- 2022/04/07 06:00 [pubmed] PHST- 2022/04/08 06:00 [medline] AID - 10.1177/09612033221083267 [doi] PST - ppublish SO - Lupus. 2022 Apr;31(5):637-645. doi: 10.1177/09612033221083267. PMID- 32814509 OWN - NLM STAT- MEDLINE DCOM- 20210722 LR - 20240229 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 29 IP - 12 DP - 2020 Oct TI - Ophthalmologic manifestations in primary antiphospholipid syndrome patients: A cross-sectional analysis of a primary antiphospholipid syndrome cohort (APS-Rio) and systematic review of the literature. PG - 1528-1543 LID - 10.1177/0961203320949667 [doi] AB - OBJECTIVE: There is a broad spectrum of eye involvement in antiphospholipid syndrome (APS). The majority of descriptions are presented as case reports that include mostly APS patients secondary to systemic lupus erythematosus (SLE), with no compelling evidence in primary APS (PAPS). This study aimed to describe ocular manifestations in our well-defined PAPS cohort (APS-Rio) and then perform a systematic literature review (SLR) of ocular manifestations in patients with APS or positivity to aPL without SLE. METHODS: We retrospectively analyzed PAPS patients followed at our outpatient clinics. All patients fulfilled Sydney APS classification criteria (2006). We evaluated them for ocular symptoms and previous ocular diagnoses. Antiphospholipid antibodies and clinical APS manifestations were compared between patients with and without ocular manifestations. For the SLR, electronic databases were searched up to November 2019. RESULTS: We studied 105 PAPS patients; 90.5% were female and 56.2% were Caucasian. We found ocular manifestations in 37.1% of our cohort. Thrombosis was the main criteria manifestation (95.2%) and lupus anticoagulant was the most prevalent antibody. Ophthalmologic diagnoses were present in 7 patients, with 5 having retinal vessels thromboses. Amaurosis fugax was the leading complaint, present in 30 patients. In the univariate analysis, amaurosis fugax was related to livedo (p = 0.005), Raynaud's phenomenon (p = 0.048) and the presence of anticardiolipin antibody (≥40 GPL/MPL) (p = 0.041). Hemianopia was associated with arterial hypertension (p = 0.049). In the multivariate analysis, the only association found was between livedo and amaurosis fugax (OR 4.09, 95%CI 1.5-11.11, p = 0.006). Our SLR incorporated 96 articles of ocular manifestations in patients with PAPS or positivity to aPL without SLE. Ocular findings varied from 5 to 88%, including anterior and posterior segments, orbital and neuro-ophthalmologic changes. CONCLUSION: There is little evidence on ocular manifestations in PAPS. We described an association between livedo and amaurosis fugax. Prospective studies are needed to promote the best treatment and avoid blindness in PAPS patients. FAU - Franco, Adriana M de M AU - Franco AMM AD - Department of Rheumatology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil. AD - Department of Ophthalmology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Brazil. FAU - Medina, Flavio Mac Cord AU - Medina FMC AD - Department of Ophthalmology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Brazil. FAU - Balbi, Gustavo Guimarães Moreira AU - Balbi GGM AUID- ORCID: 0000-0003-0235-8834 AD - Department of Rheumatology, Hospital Universitário, Universidade Federal de Juiz de Fora, Juiz de Fora, Brazil. FAU - Levy, Roger Abramino AU - Levy RA AD - Department of Rheumatology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil. FAU - Signorelli, Flavio AU - Signorelli F AD - Department of Rheumatology, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil. AD - Department of Internal Medicine, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. LA - eng PT - Journal Article PT - Systematic Review DEP - 20200819 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Adult MH - Antibodies, Antiphospholipid/*immunology MH - Antiphospholipid Syndrome/*complications/diagnosis/*immunology/therapy MH - Cross-Sectional Studies MH - Eye Diseases/*etiology/pathology MH - Female MH - Humans MH - Hypertension MH - Lupus Erythematosus, Systemic/complications/immunology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Retrospective Studies MH - Thrombosis OTO - NOTNLM OT - Antiphospholipid syndrome OT - antiphospholipid antibodies OT - eye disease OT - eye disorder OT - retinal diseases OT - thrombosis EDAT- 2020/08/21 06:00 MHDA- 2021/07/22 06:00 CRDT- 2020/08/21 06:00 PHST- 2020/08/21 06:00 [pubmed] PHST- 2021/07/22 06:00 [medline] PHST- 2020/08/21 06:00 [entrez] AID - 10.1177/0961203320949667 [doi] PST - ppublish SO - Lupus. 2020 Oct;29(12):1528-1543. doi: 10.1177/0961203320949667. Epub 2020 Aug 19. PMID- 40290703 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260422 LR - 20260423 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 3 DP - 2025 Oct TI - Evaluation and prescription trends in systemic sclerosis: Report of a survey among Indian rheumatologists. PG - 322-331 LID - 10.1177/23971983251328797 [doi] AB - OBJECTIVE: This study evaluates assessment and prescription trends in systemic sclerosis across different Indian healthcare settings, with a focus on diagnostic practices such as screening for interstitial lung disease, pulmonary hypertension, and adherence to recommended treatment protocols. The goal is to identify disparities and areas for improvement in the management of systemic sclerosis. METHODS: A cross-sectional questionnaire-based survey was conducted among rheumatologists from teaching and non-teaching hospitals across India. Data collection focused on key diagnostic practices such as the modified Rodnan skin score, chest imaging, pulmonary function tests, and echocardiography. Organ-specific prescription trends were collected and compared between teaching and non-teaching centres. RESULTS: The response rate for the survey was 70.5%. Teaching centres demonstrated higher adherence to performing modified Rodnan skin score at baseline (72.2%) compared to non-teaching hospitals (38.4%). For interstitial lung disease screening, overall, 93.7% performed chest imaging, with only 31.4% utilizing a High-Resolution CT thorax as the screening tool. Teaching centres performed 6MWT (79.5%) more often than non-teaching centres (64.7%). Echocardiography was commonly used for screening pulmonary hypertension (96.4%), while 16.5% reported using right heart catheterization. Steroids were used by 79.9% of participants at low doses (<10 mg) for a duration of less than 3 months, commonly for myositis(68%). Methotrexate(49.8%) and mycophenolate (38.3%) were the most prescribed first-line agents for systemic sclerosis-skin involvement. For systemic sclerosis-interstitial lung disease, mycophenolate (95%) was the most commonly used immunosuppression. Sequential addition of antifibrotic(62.4%) to immunosuppression was preferred over an upfront combination in systemic sclerosis-interstitial lung disease. The majority treated uncomplicated Raynaud's phenomenon with calcium channel blockers, followed by PDE5 inhibitors (61.4%). An upfront combination of endothelin receptor antagonists and PDE5i for systemic sclerosis-pulmonary hypertension was reported by 42.2%. CONCLUSION: The study highlights differences in systemic sclerosis management trends among Indian rheumatologists. Despite variations in disease-encounter and practice settings, adherence to international recommendations in key domains and areas for further improvement are brought to light. CI - © The Author(s) 2025. FAU - Das, Anna C AU - Das AC AUID- ORCID: 0000-0002-6433-550X AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India. FAU - Sakthivel, Yogananth AU - Sakthivel Y AUID- ORCID: 0009-0004-8510-1245 AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India. FAU - Shenoy, Padmanabha AU - Shenoy P AUID- ORCID: 0000-0002-7666-1361 AD - Department of Rheumatology, Centre for Arthritis and Rheumatism Excellence, Cochin, India. FAU - Sircar, Geetabali AU - Sircar G AD - Department of Rheumatology & Clinical Immunology, Seth Sukhlal Karnani Memorial Hospital, Institute of Post Graduate Medical Education & Research, Kolkata, India. FAU - Mamadapur, Mahabaleshwar AU - Mamadapur M AUID- ORCID: 0000-0001-7421-2418 AD - Department of Clinical Immunology & Rheumatology, JSS Medical College, Mysuru, India. FAU - Buche, Avinash Suresh AU - Buche AS AUID- ORCID: 0009-0008-7879-2884 AD - Department of Medicine & Rheumatology, Dr. Hedgewar Hospital, Aurangabad, India. FAU - Shukla, Dhaiwat AU - Shukla D AUID- ORCID: 0000-0002-2314-9241 AD - Division of Rheumatology, Department of Medicine, Sheth VS General Hospital, Ahmedabad, India. FAU - Bhojani, Kaushik S AU - Bhojani KS AD - Rheumatology Services, Fortis Hospital, Mumbai, India. FAU - Nalawade, Ajit AU - Nalawade A AUID- ORCID: 0000-0001-8603-126X AD - Department of Rheumatology, Sancheti Institute of Orthopedics and Rehabilitation, Pune, India. FAU - Goel, Ruchika AU - Goel R AD - Department of Clinical Immunology & Rheumatology, Christian Medical College, Vellore, India. FAU - Singh, Yogesh Preet AU - Singh YP AUID- ORCID: 0000-0003-1258-0041 AD - Department of Clinical Immunology & Rheumatology, AIIMS Bilaspur, Himachal Pradesh, India. FAU - Parimi, Vijaya Prasanna AU - Parimi VP AUID- ORCID: 0000-0002-7834-4845 AD - Department of Clinical Immunology & Rheumatology, ESIC Medical College & Hospital, Hyderabad, India. FAU - Sharma, Aman AU - Sharma A AD - Clinical Immunology & Rheumatology Wing, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India. FAU - Agarwal, Vikas AU - Agarwal V AUID- ORCID: 0000-0002-4508-1233 AD - Department of Clinical Immunology & Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0001-6822-3401 AD - Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Kodali, Ramya Sri AU - Kodali RS AUID- ORCID: 0000-0002-6054-6166 AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India. FAU - Janardana, Ramya AU - Janardana R AUID- ORCID: 0000-0002-4904-1511 AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India. FAU - Shobha, Vineeta AU - Shobha V AUID- ORCID: 0000-0001-5271-0766 AD - Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, India. LA - eng PT - Journal Article DEP - 20250422 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC12018362 OTO - NOTNLM OT - Indian rheumatologists OT - Systemic sclerosis OT - physician practice patterns OT - questionnaire OT - survey COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2025/04/28 17:04 MHDA- 2025/04/28 17:05 PMCR- 2026/04/22 CRDT- 2025/04/28 06:23 PHST- 2024/12/28 00:00 [received] PHST- 2025/03/04 00:00 [accepted] PHST- 2025/04/28 17:05 [medline] PHST- 2025/04/28 17:04 [pubmed] PHST- 2025/04/28 06:23 [entrez] PHST- 2026/04/22 00:00 [pmc-release] AID - 10.1177_23971983251328797 [pii] AID - 10.1177/23971983251328797 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2025 Apr 22;10(3):322-331. doi: 10.1177/23971983251328797. eCollection 2025 Oct. PMID- 41678148 OWN - NLM STAT- In-Process LR - 20260608 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 44 IP - 2 DP - 2026 Feb TI - Assessment of myositis specific antibodies in primary chronic arthritis: single centre prospective study. PG - 268-272 LID - 10.55563/clinexprheumatol/z438d5 [doi] AB - OBJECTIVES: Among the myositis specific antibodies (MSAs), the antisynthetase (anti-ARS) and the anti-MDA5 antibodies are those more frequently characterised by the occurrence of joint involvement. We aim to define the prevalence of MSAs in patients with established rheumatoid arthritis (RA), psoriatic arthritis (PsA), or undifferentiated polyarthritis (UPA). METHODS: From January 2021 to December 2024, all RA, PsA and UPA patients prospectively followed in our Early Arthritis Clinic (EAC), were evaluated. Changes in diagnosis, clinical/laboratory signs of muscle/lung/skin involvement at onset or during the follow-up, overlap syndromes, anti-ENA or cytoplasmic ANA positivity, Raynaud's phenomenon, and less than 24 months of follow-up were exclusion criteria. Baseline serum samples were tested for MSAs (line-blot). Positivity was defined according to manufacturers' instructions. RESULTS: 143 patients were enrolled (93 females, 65%; 67 AR, 47%; 50 UPA, 35%, 26 PsA, 18%). Line-blot resulted positive in 10 (7%), weak-positive in 12 (8%), and borderline in 26 cases (18%). The remaining 95 patients (67%) were negative. MSAs positivity was anti-cN1A in 3 cases and anti-ARS and anti-MDA in 4 cases each. Weak positivity was found for anti-ARS (4), and anti-PM-Scl75 (3). Borderline results showed a high number of anti-ARS and aMDA5 (12, 46%). No variables were associated with MSA positivity. CONCLUSIONS: MSAs positivity may be observed in one third of patients with primary isolated arthritis. About half of these cases displayed full or weak positivity for MSAs, whereas the remaining half displayed borderline results. Clinicians should be aware that MSA should be assessed only in case of effective clinical need. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Bianchessi, Lorenzo M AU - Bianchessi LM AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. lorenzomattia.bianchessi01@universitadipavia.it. FAU - Codullo, Veronica AU - Codullo V AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Bottazzi, Francesca AU - Bottazzi F AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Guerini, Miriam AU - Guerini M AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - De Stefano, Ludovico AU - De Stefano L AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Niedda, Flora AU - Niedda F AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and IRCCS Fondazione Policlinico Gemelli, Università Cattolica del Sacro Cuore, Rome, Italy. FAU - Alpini, Claudia AU - Alpini C AD - Laboratory of Biochemical-Clinical Analyses, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Caporali, Roberto AU - Caporali R AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO, Milan; and Department of Clinical Sciences and Community Health, University of Milan, Italy. FAU - Bugatti, Serena AU - Bugatti S AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Dipartimento di Medicina Interna e Terapia Medica, Università di Pavia; and UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy. LA - eng PT - Journal Article DEP - 20260211 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - 0 (Biomarkers) RN - EC 3.6.1.- (IFIH1 protein, human) SB - IM MH - Humans MH - Female MH - Male MH - Prospective Studies MH - Middle Aged MH - *Myositis/immunology/blood/diagnosis/epidemiology MH - *Autoantibodies/blood MH - Adult MH - Aged MH - *Arthritis/immunology/blood MH - *Arthritis, Psoriatic/immunology/blood MH - Interferon-Induced Helicase, IFIH1/immunology MH - Biomarkers/blood MH - *Arthritis, Rheumatoid/immunology/blood MH - Chronic Disease EDAT- 2026/02/12 12:50 MHDA- 2026/02/25 13:37 CRDT- 2026/02/12 11:33 PHST- 2025/05/18 00:00 [received] PHST- 2025/09/24 00:00 [accepted] PHST- 2026/02/25 13:37 [medline] PHST- 2026/02/12 12:50 [pubmed] PHST- 2026/02/12 11:33 [entrez] AID - 22600 [pii] AID - 10.55563/clinexprheumatol/z438d5 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2026 Feb;44(2):268-272. doi: 10.55563/clinexprheumatol/z438d5. Epub 2026 Feb 11. PMID- 40658239 OWN - NLM STAT- MEDLINE DCOM- 20250714 LR - 20260323 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 45 IP - 8 DP - 2025 Jul 14 TI - Rheumatoid vasculitis presenting in the absence of rheumatoid arthritis: a case-based review. PG - 168 LID - 10.1007/s00296-025-05926-z [doi] AB - Rheumatoid vasculitis (RV) is an extra-articular manifestation (EAM) of rheumatoid arthritis (RA), which is typically observed in long-standing, severe disease. The skin and peripheral nerves are most commonly affected, although the clinical presentation can be highly variable. We report an unusual case of a 58-year-old male who presented with peripheral neuropathy, constitutional symptoms, and Raynaud's phenomenon and was subsequently diagnosed with RV despite having no history of RA. Rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies were strongly positive, and a sural nerve biopsy demonstrated vasculitis. The patient also developed nodular lesions over his forearms consistent with rheumatoid nodules that regressed following treatment. Currently, the patient has not developed any clinical synovitis. In addition, a search of Pubmed/Medline, Embase, Web of Science, ScienceDirect, the Directory of Open Access Journals (DOAJ), and Google Scholar identified 18 full-text case reports describing patients who developed RV in the absence of a diagnosis of RA at the time of onset. Further analysis of these cases revealed a wide range of clinical manifestations. RV preceded RA onset in 5 of these patients, with durations ranging from 6 months to 6 years. In addition to our patient, 3 patients with RV who did not develop evidence of articular involvement were identified. The development of subcutaneous nodules in our patient appeared to be a unique finding among the identified patients. We highlight that RV can precede RA and suggest that this presentation be considered in any future diagnostic criteria for RV. CI - © 2025. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Cromie, David A J AU - Cromie DAJ AUID- ORCID: 0009-0005-0901-6345 AD - Department of Intensive Care Medicine, Antrim Area Hospital, Northern Health and Social Care Trust, Antrim, Northern Ireland, UK. dcromie04@qub.ac.uk. FAU - Stewart, Sarah K AU - Stewart SK AUID- ORCID: 0009-0007-1863-9178 AD - Department of Rheumatology, Craigavon Area Hospital, Southern Health and Social Care Trust, Craigavon, Northern Ireland, UK. FAU - Wright, Gary D AU - Wright GD AD - Department of Rheumatology, Musgrave Park Hospital, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK. FAU - Healy, Estelle G AU - Healy EG AUID- ORCID: 0000-0002-3463-1068 AD - Department of Cell Pathology, The Institute of Pathology, Royal Victoria Hospital, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20250714 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 9009-79-4 (Rheumatoid Factor) RN - 0 (Anti-Citrullinated Protein Antibodies) SB - IM EIN - Rheumatol Int. 2026 Mar 23;46(4):64. doi: 10.1007/s00296-026-06092-6. PMID: 41870571 MH - Humans MH - Middle Aged MH - Male MH - *Rheumatoid Vasculitis/diagnosis/drug therapy/immunology MH - Rheumatoid Factor/blood MH - Anti-Citrullinated Protein Antibodies/blood MH - Arthritis, Rheumatoid/diagnosis MH - Sural Nerve/pathology MH - Biopsy MH - Treatment Outcome OTO - NOTNLM OT - Biopsy OT - Rheumatoid arthritis OT - Rheumatoid nodules OT - Rheumatoid vasculitis OT - Vasculitic neuropathy OT - Vasculitis COIS- Declarations. Conflict of interest: The authors have no relevant financial or nonfinancial interests to disclose. Ethical approval: No part of this manuscript, including the text and graphics, has been copied or published elsewhere in whole or in part. No artificial intelligence (AI) tools were used in the writing or editing of this manuscript. All content was generated and reviewed by the authors. Ethical approval was not required for this type of study. Informed consent: Written informed consent was obtained from the patient for publication of this case and any accompanying images. Consent to participate: Written informed consent was obtained from the patient. Consent to publish: The participant has consented to the submission of this case-based review to the journal, including publication of included images. EDAT- 2025/07/14 12:28 MHDA- 2025/07/14 12:29 CRDT- 2025/07/14 11:14 PHST- 2025/02/02 00:00 [received] PHST- 2025/07/01 00:00 [accepted] PHST- 2025/07/14 12:29 [medline] PHST- 2025/07/14 12:28 [pubmed] PHST- 2025/07/14 11:14 [entrez] AID - 10.1007/s00296-025-05926-z [pii] AID - 10.1007/s00296-025-05926-z [doi] PST - epublish SO - Rheumatol Int. 2025 Jul 14;45(8):168. doi: 10.1007/s00296-025-05926-z. PMID- 39539989 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241116 IS - 2036-7392 (Print) IS - 2036-7406 (Electronic) IS - 2036-7392 (Linking) VI - 16 IP - 3 DP - 2024 Sep 2 TI - Platelet-rich plasma for the treatment of scleroderma-associated ulcers: a single-center experience and literature review. PG - 9878 LID - 10.4081/dr.2024.9878 [doi] LID - 9878 AB - Systemic sclerosis (SS) is a complex connective tissue disease characterized by vasculopathy and progressive fibrosis, primarily considered an autoimmune disorder. SS can affect multiple organs and tissues, including the skin, respiratory, gastrointestinal, genitourinary, cardiovascular, and musculoskeletal systems. Skin involvement is common, and SS-related ulcers, especially digital ulcers, occur in roughly 50% of patients. These ulcers not only cause pain but also significantly impact patients' quality of life, and in severe cases, they can lead to infection, gangrene, and amputation. The search for novel therapies for scleroderma-related ulcers remains an ongoing research area. Platelet-rich plasma (PRP) has been investigated as a potential treatment for difficult-to-heal ulcers, including diabetic, pressure, and vascular ulcers. In this study, we share our experience in treating scleroderma ulcers with PRP. Ten patients with confirmed SS and chronic skin ulcers lasting at least six weeks, which had not responded to conventional treatments, were selected for the study. Homologous PRP gel was prepared and applied once a week for up to eight weeks. The ulcers were documented photographically before and after PRP treatment, and pain levels were assessed using a visual analog scale (VAS). We also conducted a systematic review of the literature focusing on the use of PRP in the setting of SS. The results from our casuistry showed that the ten patients, including eight females and two males with a median age of 52.5 years, had ulcer sizes ranging from 0.78 cm(2) to 28.26 cm(2). The ulcers were located on fingers, legs, and heels, and they were associated with various forms of SS, including limited and diffuse cutaneous involvement. Raynaud's phenomenon was prevalent, and two patients exhibited organ involvement. The average ulcer size at the end of PRP treatment decreased significantly, with a 78% reduction in ulcered area. Pain levels also markedly improved, as indicated by a reduction in VAS scores. With regards to systematic revision of literature, we retrieved 45 cases of SS treated with PRP-based therapeutic regimes. However, only a minority of them (n=16) underwent PRP treatment for the treatment of SS-related ulcers. An improvement in wound size and pain has been documented in all cases. Taken together, these data highlight the potential benefits of using homologous PRP in the treatment of scleroderma ulcers, emphasizing its positive impact on ulcer size reduction and pain relief. CI - Copyright © 2024, the Author(s). FAU - Condorelli, Alessandra Grazia AU - Condorelli AG AD - Dermatology Unit, Reggio Emilia AUSL-IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia. FAU - Paganelli, Alessia AU - Paganelli A AD - Dermatology Unit, Reggio Emilia AUSL-IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia. FAU - Marraccini, Chiara AU - Marraccini C AD - Transfusion Medicine Unit, Reggio Emilia AUSL-IRCCS. FAU - Ficarelli, Elena AU - Ficarelli E AD - Dermatology Unit, Reggio Emilia AUSL-IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia. FAU - Motolese, Alfonso AU - Motolese A AD - Section of Dermatology, Department of Clinical and Experimental Medicine, University of Messina, Italy. FAU - Contu, Luca AU - Contu L AD - Dermatology Unit, Reggio Emilia AUSL-IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia. FAU - Motolese, Alberico AU - Motolese A AD - Dermatology Unit, Reggio Emilia AUSL-IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia. LA - eng PT - Journal Article DEP - 20240221 PL - Italy TA - Dermatol Reports JT - Dermatology reports JID - 101566470 PMC - PMC11558311 OTO - NOTNLM OT - PRP OT - homologous PRP OT - scleroderma ulcers OT - systemic sclerosis COIS- Conflict of interest: the authors declare no potential conflict of interest. EDAT- 2024/11/14 17:37 MHDA- 2024/11/14 17:38 PMCR- 2024/02/21 CRDT- 2024/11/14 04:26 PHST- 2023/10/20 00:00 [received] PHST- 2024/02/04 00:00 [accepted] PHST- 2024/11/14 17:38 [medline] PHST- 2024/11/14 17:37 [pubmed] PHST- 2024/11/14 04:26 [entrez] PHST- 2024/02/21 00:00 [pmc-release] AID - 10.4081/dr.2024.9878 [doi] PST - epublish SO - Dermatol Reports. 2024 Feb 21;16(3):9878. doi: 10.4081/dr.2024.9878. eCollection 2024 Sep 2. PMID- 39287841 OWN - NLM STAT- MEDLINE DCOM- 20240917 LR - 20250416 IS - 1591-9528 (Electronic) IS - 1591-8890 (Print) IS - 1591-8890 (Linking) VI - 24 IP - 1 DP - 2024 Sep 17 TI - Ultrasound shear wave elastography for assessing minor salivary gland involvement in anti-centromere antibody-positive primary Sjögren's syndrome: a retrospective study. PG - 221 LID - 10.1007/s10238-024-01486-x [doi] LID - 221 AB - The aim of this study is to investigate salivary gland involvement in patients with anti-centromere antibody (ACA)-positive primary Sjögren's syndrome (pSS). We retrospectively evaluated 134 patients with pSS. Patients were divided into four groups based on the results of ACA and SSA antibodies. We compared clinical manifestations, laboratory findings, salivary gland shear wave elastography, minor salivary gland biopsy results, and EULAR Sjögren's syndrome disease activity index (ESSDAI) scores among the four groups. A total of 134 patients were classified as having pSS and divided into three groups based on serum ACA and anti-SSA antibody status: ACA + SSA + , ACA + SSA-, ACA-SSA + , and seronegative. The primary analysis focused on comparing the clinical and SWE findings between the ACA + SSA + and ACA + SSA- groups. In the double-positive group, SWE revealed fewer minor salivary glands along with higher mean (Emean) and maximum (Emax) values of Young's moduli than those in the ACA-negative group. Patients in the positive group had increased occurrence of Raynaud's phenomenon, liver involvement, and a higher incidence of malignancy (P < 0.05). ACA-positive pSS patients are a subgroup with different clinical manifestations and more pronounced involvement of the minor salivary glands. SWE findings revealed that ACA-positive patients exhibit significantly higher mean and maximum stiffness values compared to ACA-negative patients, indicating more extensive glandular fibrosis and involvement. These results underscore the utility of SWE as a valuable method for evaluating salivary gland pathology and supporting the stratification of pSS patients. CI - © 2024. The Author(s). FAU - Wang, Xinyu AU - Wang X AD - Departments of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. FAU - Wang, Xujie AU - Wang X AD - Departments of Ultrasound, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. FAU - Wu, Jian AU - Wu J AD - Departments of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. FAU - Dong, Fenglin AU - Dong F AD - Departments of Ultrasound, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. FAU - Chang, Xin AU - Chang X AD - Departments of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. xinchang@suda.edu.cn. FAU - Wang, Aju AU - Wang A AD - Departments of Ultrasound, The First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China. 13814875143@163.com. LA - eng PT - Journal Article PT - Review DEP - 20240917 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 RN - 0 (Antibodies, Antinuclear) SB - IM EIN - Clin Exp Med. 2025 Apr 16;25(1):120. doi: 10.1007/s10238-025-01594-2. PMID: 40240616 MH - Humans MH - *Sjogren's Syndrome/diagnostic imaging/immunology/pathology MH - Retrospective Studies MH - Female MH - *Elasticity Imaging Techniques/methods MH - Middle Aged MH - Male MH - *Salivary Glands, Minor/pathology/diagnostic imaging MH - *Antibodies, Antinuclear/blood MH - Adult MH - Aged MH - Centromere/immunology MH - Biopsy PMC - PMC11408540 OTO - NOTNLM OT - Anti-centromere antibody OT - Salivary gland OT - Shear wave elastography OT - Sjogren’s syndrome COIS- The authors declare no competing interests. EDAT- 2024/09/17 21:47 MHDA- 2024/09/18 00:51 PMCR- 2024/09/17 CRDT- 2024/09/17 11:15 PHST- 2024/07/18 00:00 [received] PHST- 2024/09/04 00:00 [accepted] PHST- 2024/09/18 00:51 [medline] PHST- 2024/09/17 21:47 [pubmed] PHST- 2024/09/17 11:15 [entrez] PHST- 2024/09/17 00:00 [pmc-release] AID - 10.1007/s10238-024-01486-x [pii] AID - 1486 [pii] AID - 10.1007/s10238-024-01486-x [doi] PST - epublish SO - Clin Exp Med. 2024 Sep 17;24(1):221. doi: 10.1007/s10238-024-01486-x. PMID- 37427219 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230718 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 85 IP - 7 DP - 2023 Jul TI - Neurological injury in primary Sjogren's syndrome. PG - 3381-3385 LID - 10.1097/MS9.0000000000000937 [doi] AB - The incidence of neurological injury in primary Sjogren's syndrome varies between 2.5 and 60%. The authors aimed to evaluate its prevalence and characteristics in patients with primary Sjogren's syndrome in a sample of the Syrian population. PATIENTS AND METHODS: Forty-eight patients with primary Sjogren's syndrome, attending outpatient clinics at Damascus Hospital between January 2020 and January 2022 in this cross-sectional study at the outpatient clinics, were interviewed and examined, and the necessary laboratory and radiological examinations were demanded. Information was collected on disease duration, onset time, and patterns of neurological symptoms. RESULTS: Forty-eight patients, including 42 females, aged 56.1±10.3 years were enroled.Central nervous system involvement was found in 34 patients. 85% of patients had generalized nerve manifestations, while local nerve manifestations were found in 77,5% of patients. The common neurological manifestation was headaches, then cognitive disorders, and the most common pattern of headache was migraine. Beck Depression Index showed a significant increase in the apathy evaluation scale.The study of cognitive changes showed a significant increase in the Mini-Mental State Examination (MMSE) index.Carotid Doppler showed the presence of injury in 42.4% of patients. The magnetic resonance imaging showed positive findings in 21 patients and positive evoked potentials in 52% of patients. DISCUSSION: Studies showing the prevalence of Sjogren's neurological injury patterns are insufficient, but this was changed when the criteria for diagnosing Sjogren's syndrome was modified, and the definition of neurological traits in the context of the syndrome was expanded.The presence of a high rate of headaches, cognitive changes, and fatigue confirms that generalized nervous system injuries are more common than local injuries. Migraine was the most common pattern of headache found in patients with the syndrome compared with other patterns such as tension headaches and headaches due to medications, especially analgesics.This was associated with the presence of anti-SSA antibodies and Raynaud's phenomenon, which suggest that the headache mechanism may be due to vascular endothelial dysfunction or an immune-mediated inflammation injury of the neurovascular system.The changes that appeared on the MRI images suggested premotor cortex involvement rather than mesolimbic cortical impairment, and its presence was also associated with SSA antibody positivity, and it is caused by inflammation. CONCLUSION: Primary Sjogren's syndrome should be considered as having any unspecified or specific neurological disorder. CI - Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. FAU - Khalayli, Naram AU - Khalayli N AD - Resident in Internal Medicine. FAU - Bouri, Mhd Fares AU - Bouri MF AUID- ORCID: 0009-0005-5986-7826 AD - Syrian Private University, Daraa, Syria. FAU - Wahbeh, Molham AU - Wahbeh M AD - Syrian Private University, Daraa, Syria. FAU - Drie, Tasneem AU - Drie T AD - Resident in Internal Medicine. FAU - Kudsi, Maysoun AU - Kudsi M AD - Professor in Rheumatology, Paculty of Medicine, Damascus University, Damascus. LA - eng PT - Journal Article DEP - 20230605 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC10328669 OTO - NOTNLM OT - PSS OT - central nervous system involvement OT - neurological injury OT - primary Sjogren’s syndrome COIS- NA. EDAT- 2023/07/10 06:42 MHDA- 2023/07/10 06:43 PMCR- 2023/06/05 CRDT- 2023/07/10 05:21 PHST- 2023/04/11 00:00 [received] PHST- 2023/05/29 00:00 [accepted] PHST- 2023/07/10 06:43 [medline] PHST- 2023/07/10 06:42 [pubmed] PHST- 2023/07/10 05:21 [entrez] PHST- 2023/06/05 00:00 [pmc-release] AID - AMSU-D-23-00836 [pii] AID - 10.1097/MS9.0000000000000937 [doi] PST - epublish SO - Ann Med Surg (Lond). 2023 Jun 5;85(7):3381-3385. doi: 10.1097/MS9.0000000000000937. eCollection 2023 Jul. PMID- 27837194 OWN - NLM STAT- MEDLINE DCOM- 20170915 LR - 20240529 IS - 1477-0962 (Electronic) IS - 0961-2033 (Print) IS - 0961-2033 (Linking) VI - 26 IP - 6 DP - 2017 May TI - Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus. PG - 646-649 LID - 10.1177/0961203316676377 [doi] AB - Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus. FAU - Wu, E Y AU - Wu EY AD - 1 Department of Pediatrics, The University of North Carolina at Chapel Hill, Chapel Hill, USA. FAU - Schanberg, L E AU - Schanberg LE AD - 2 Department of Pediatrics, Duke University Medical Center, Durham, USA. FAU - Wershba, E C AU - Wershba EC AD - 3 Phoenix Children's Medical Group, Phoenix, USA. FAU - Rabinovich, C E AU - Rabinovich CE AD - 2 Department of Pediatrics, Duke University Medical Center, Durham, USA. LA - eng GR - T32 AI007062/AI/NIAID NIH HHS/United States GR - T32 AI007217/AI/NIAID NIH HHS/United States GR - T32 HD060558/HD/NICHD NIH HHS/United States PT - Journal Article DEP - 20161112 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Immunologic Factors) RN - 4Z8R6ORS6L (Thalidomide) RN - 9PHQ9Y1OLM (Prednisolone) RN - F0P408N6V4 (Lenalidomide) SB - IM MH - Adolescent MH - Female MH - Humans MH - Immunologic Factors/*administration & dosage/therapeutic use MH - Lenalidomide MH - Lupus Erythematosus, Cutaneous/*drug therapy/ethnology MH - Lupus Erythematosus, Systemic/*complications/ethnology MH - Male MH - Prednisolone/administration & dosage/therapeutic use MH - Retrospective Studies MH - Thalidomide/administration & dosage/*analogs & derivatives/therapeutic use MH - Treatment Outcome MH - Young Adult PMC - PMC5388573 MID - NIHMS821978 OTO - NOTNLM OT - Cutaneous lupus OT - pediatric OT - systemic lupus erythematosus EDAT- 2016/11/12 06:00 MHDA- 2017/09/16 06:00 PMCR- 2018/05/01 CRDT- 2016/11/13 06:00 PHST- 2016/11/12 06:00 [pubmed] PHST- 2017/09/16 06:00 [medline] PHST- 2016/11/13 06:00 [entrez] PHST- 2018/05/01 00:00 [pmc-release] AID - 0961203316676377 [pii] AID - 10.1177/0961203316676377 [doi] PST - ppublish SO - Lupus. 2017 May;26(6):646-649. doi: 10.1177/0961203316676377. Epub 2016 Nov 12. PMID- 24720440 OWN - NLM STAT- MEDLINE DCOM- 20141121 LR - 20260128 IS - 1502-7732 (Electronic) IS - 0300-9742 (Linking) VI - 43 IP - 5 DP - 2014 TI - Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval. PG - 398-402 LID - 10.3109/03009742.2014.887768 [doi] AB - OBJECTIVES: Ischaemic digital ulcers (DUs) are a common complication of systemic sclerosis (SSc). This study aimed to characterize patients with SSc and ongoing DUs treated with the endothelin receptor antagonist bosentan in clinical practice in France. METHOD: An observational, retrospective, longitudinal study was conducted in 10 French expert centres. Medical records from randomly selected adult SSc patients who received treatment with bosentan for DU prevention from March 2007 to December 2010 were analysed. The primary objective was to determine the profile of patients at treatment initiation. Secondary objectives were to monitor bosentan dosing, treatment schedule, and reasons for treatment termination. RESULTS: The study included 89 patients (mean age 52 years, 69% female, 44% diffuse cutaneous SSc). At bosentan treatment initiation, the mean duration of Raynaud's phenomenon was 15 ± 12 years, and the mean time since first episode with DU was 6.5 ± 7 years. Most patients had a history of at least two episodes with DUs, separated by < 12 months (61%), and had received intravenous iloprost (63%). Previous DU complications included auto-amputation (8%), surgical amputation (6%), osteitis (6%), and gangrene (4.5%). Active smokers (25%) had a history of significantly more surgical amputation (p = 0.004) and osteitis (p = 0.004) than non-smokers. At least one active DU at bosentan initiation was detected in 82% of patients. Bosentan was used according to prescription guidelines and was well tolerated; six patients (7%) withdrew from treatment because of raised liver enzymes. CONCLUSIONS: Patients treated with bosentan for DU prevention in France have severe, refractory, ongoing ulcerative disease. Active smoking was correlated to a history of DU complications. Tolerance of bosentan was comparable to previous studies. FAU - Agard, C AU - Agard C AD - Department of Internal Medicine, Hôtel-Dieu University Hospital , Nantes , France. FAU - Carpentier, P H AU - Carpentier PH FAU - Mouthon, L AU - Mouthon L FAU - Clerson, P AU - Clerson P FAU - Gressin, V AU - Gressin V FAU - Bérezné, A AU - Bérezné A FAU - Diot, E AU - Diot E FAU - Jego, P AU - Jego P FAU - Lok, C AU - Lok C FAU - Sparsa, A AU - Sparsa A FAU - Chatelus, E AU - Chatelus E FAU - Van Kien, A Khau AU - Van Kien AK FAU - Quéré, I AU - Quéré I FAU - Sibilia, J AU - Sibilia J FAU - Hachulla, E AU - Hachulla E LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20140411 PL - England TA - Scand J Rheumatol JT - Scandinavian journal of rheumatology JID - 0321213 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Aged MH - Bosentan MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Endothelin Receptor Antagonists/administration & dosage/*therapeutic use MH - Female MH - *Fingers MH - France MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/*complications MH - Smoking/adverse effects MH - Sulfonamides/administration & dosage/*therapeutic use MH - Treatment Outcome MH - Ulcer/*prevention & control EDAT- 2014/04/12 06:00 MHDA- 2014/12/15 06:00 CRDT- 2014/04/12 06:00 PHST- 2014/04/12 06:00 [entrez] PHST- 2014/04/12 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] AID - 10.3109/03009742.2014.887768 [doi] PST - ppublish SO - Scand J Rheumatol. 2014;43(5):398-402. doi: 10.3109/03009742.2014.887768. Epub 2014 Apr 11. PMID- 23656799 OWN - NLM STAT- MEDLINE DCOM- 20140425 LR - 20211021 IS - 1554-6179 (Electronic) IS - 1539-4182 (Print) IS - 1539-4182 (Linking) VI - 11 IP - 3 DP - 2013 Sep TI - Limited scleroderma with pauci-immune glomerulonephritis in the presence of renal cell carcinoma. PG - 117-9 LID - 10.3121/cmr.2013.1141 [doi] AB - Connective tissue disorders increase the risk of malignancy; conversely, they may manifest as rheumatological paraneoplastic syndromes due to an underlying malignancy. We describe the case of a patient with limited scleroderma whose rapid disease progression coincided with the discovery of a renal tumor. A woman, age 75 years, presented with a 3-month history of progressive difficulty grasping objects, unsteadiness, dyspnea, xerostomia, xerophthalmia, and significant weight loss. She had a 10-year history of gastroesophageal reflux and Raynaud's phenomenon. Pertinent physical examination findings included facial telangiectasias, bibasilar inspiratory rales, sclerodactyly, and absent pinprick and vibratory sensation in her toes. She also had swelling and tenderness in several metacarpophalangeal and interphalangeal joints and in both ankles. A renal mass was demonstrated on abdominal computed tomography. A left partial nephrectomy was performed, confirming an unclassified type of renal cell carcinoma, along with a focal proliferative crescentic pauci-immune glomerulonephritis. Medical therapy with rituximab, pulse methylprednisolone, and prednisone led to improvement in her symptoms. The patient's presentation is consistent with a rapid progression of pre-existing limited scleroderma with the development of new rheumatological symptoms, including vasculitis. We propose that this progression was secondary to paraneoplastic stimulation by the renal cell carcinoma. Clinicians should consider looking for a malignancy in patients with connective tissue disorders who present with a myriad of new symptoms. FAU - Abrich, Victor AU - Abrich V AD - Corresponding Author: Victor Abrich, Marshfield Clinic, Department of Internal Medicine, 1000 North Oak Avenue, Marshfield, WI 54449 USA. abrich.victor@marshfieldclinic.org. FAU - Duvuru, Sudhir AU - Duvuru S FAU - Swanson, Howard J AU - Swanson HJ LA - eng PT - Case Reports PT - Journal Article DEP - 20130508 PL - United States TA - Clin Med Res JT - Clinical medicine & research JID - 101175887 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 4F4X42SYQ6 (Rituximab) RN - VB0R961HZT (Prednisone) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Aged MH - Antibodies, Monoclonal, Murine-Derived/administration & dosage MH - Antineoplastic Combined Chemotherapy Protocols/*administration & dosage MH - *Carcinoma, Renal Cell/diagnostic imaging/drug therapy MH - Female MH - *Glomerulonephritis/complications/diagnostic imaging/drug therapy MH - Humans MH - *Kidney Neoplasms/diagnostic imaging/drug therapy MH - Methylprednisolone/administration & dosage MH - Prednisone/administration & dosage MH - Rituximab MH - *Scleroderma, Limited/complications/diagnostic imaging/drug therapy MH - Tomography, X-Ray Computed PMC - PMC3788437 OTO - NOTNLM OT - Complications OT - Connective Tissue Diseases OT - Etiology OT - Neoplasms OT - Pauci-immune crescentic glomerulonephritis OT - Renal Cell Carcinoma OT - Scleroderma EDAT- 2013/05/10 06:00 MHDA- 2014/04/26 06:00 PMCR- 2013/09/01 CRDT- 2013/05/10 06:00 PHST- 2013/05/10 06:00 [entrez] PHST- 2013/05/10 06:00 [pubmed] PHST- 2014/04/26 06:00 [medline] PHST- 2013/09/01 00:00 [pmc-release] AID - cmr.2013.1141 [pii] AID - 0110117 [pii] AID - 10.3121/cmr.2013.1141 [doi] PST - ppublish SO - Clin Med Res. 2013 Sep;11(3):117-9. doi: 10.3121/cmr.2013.1141. Epub 2013 May 8. PMID- 18634147 OWN - NLM STAT- MEDLINE DCOM- 20081211 LR - 20080911 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 35 IP - 9 DP - 2008 Sep TI - Prevalence of exercise pulmonary arterial hypertension in scleroderma. PG - 1812-6 AB - OBJECTIVE: Pulmonary arterial hypertension (PAH) is a complication of scleroderma (systemic sclerosis, SSc); as soon as PAH develops, the patient's prognosis deteriorates rapidly. Early detection of PAH ensures timely treatment. We investigated the prevalence of exercise-induced PAH in a cohort of patients with SSc, and examined the relation between exercise-induced PAH and clinical characteristics and biochemical markers. METHODS: Patients with SSc and normal resting systolic pulmonary arterial pressure (sPAP) were studied. Eligible patients were asked to perform cycloergometer exercise until exhaustion, and exercise sPAP was measured. All patients had their pulmonary function tested and underwent echocardiography at rest. Brain natriuretic peptide (BNP) was also determined. RESULTS: Forty-one patients with SSc were studied. Mean sPAP at rest was 29.7 mm Hg, rising to a mean of 41.4 mm Hg on exercise. Eleven of 41 patients (26.8%) had sPAP post-exercise > 50 mm Hg and 8/41 (19.5%) > 55 mm Hg. A significant correlation was found between exercise sPAP and DLCO (p = 0.008) and between sPAP and BNP levels (p = 0.04). Pre-existing severe Raynaud's phenomenon was more prevalent (50% vs 20%), DLCO levels lower (78.9 vs 92.7 % predicted), and BNP levels higher (72.6 vs 42.1 pmol/ml) in patients with exercise sPAP > 55 mm Hg. CONCLUSION: The prevalence of exercise-induced PAH in patients with scleroderma is high. Patients with lower DLCO and higher levels of BNP are at higher risk of developing higher sPAP. Studies with longterm followup are required to evaluate the risk of developing resting PAH in these patients. FAU - Callejas-Rubio, Jose Luis AU - Callejas-Rubio JL AD - Unidad de Enfermedades Autoinmunes Sistémicas, Granada, Spain. JLCALLEJA@telefonica.net FAU - Moreno-Escobar, Eduardo AU - Moreno-Escobar E FAU - de la Fuente, Pilar Martín AU - de la Fuente PM FAU - Pérez, Lourdes López AU - Pérez LL FAU - Fernández, Raquel Rios AU - Fernández RR FAU - Sánchez-Cano, Daniel AU - Sánchez-Cano D FAU - Mora, José Pomares AU - Mora JP FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080715 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 114471-18-0 (Natriuretic Peptide, Brain) SB - IM MH - Blood Pressure Determination MH - Cross-Sectional Studies MH - Disease Progression MH - Echocardiography, Doppler MH - *Exercise MH - Exercise Test MH - *Exercise Tolerance MH - Female MH - Humans MH - Hypertension, Pulmonary/blood/*etiology/physiopathology MH - Male MH - Middle Aged MH - Natriuretic Peptide, Brain/blood MH - Prevalence MH - Respiratory Function Tests MH - Scleroderma, Systemic/blood/*complications/physiopathology MH - Spain EDAT- 2008/07/18 09:00 MHDA- 2008/12/17 09:00 CRDT- 2008/07/18 09:00 PHST- 2008/07/18 09:00 [pubmed] PHST- 2008/12/17 09:00 [medline] PHST- 2008/07/18 09:00 [entrez] AID - 08/13/0723 [pii] PST - ppublish SO - J Rheumatol. 2008 Sep;35(9):1812-6. Epub 2008 Jul 15. PMID- 18270914 OWN - NLM STAT- MEDLINE DCOM- 20080215 LR - 20131121 IS - 1439-4413 (Electronic) IS - 0012-0472 (Linking) VI - 133 IP - 8 DP - 2008 Feb TI - [Therapy-resistant tuberculous skin abscesses after methylprednisolone therapy]. PG - 346-9 LID - 10.1055/s-2008-1046716 [doi] AB - HISTORY AND CLINICAL FINDINGS: Systemic lupus erythematodes was suspected in a 55-year-old patient with arthralgias, secondary Raynaud's syndrome with acral necroses and increased antinuclear antibodies. Treatment with methylprednisolone was started but despite initial improvement of symptoms, the fever, weight loss and nocturnal sweating continued and the patient developed a progressive erythema on his left thigh. Skin biopsy revealed Mycobacterium tuberculosis. INVESTIGATIONS: Drug sensitivity tests of the initial culture isolate from the skin biopsy indicated response to all tested first-line drugs. The initial chest-x-ray showed only posttubercular lesions in both upper lobes. Computed tomography revealed a progressive infiltration in the right upper lobe and multiple disseminated lesions in both lungs. Mycobacteria were cultured from sputum. A fluorescence-activated cell analysis of peripheral leucocytes showed a decreased total T-cell-count of 173/microl. TREATMENT AND CLINICAL COURSE: Tuberculostatic treatment with five first-line drugs led to a temporary improvement of the patient's general condition and of the inflammatory signs. But the initially small cutaneous erythema expanded and developed into a large abscess, which could not be controlled even with surgery. Multiple necrotic lesions then developed rapidly in different areas of the skin without any response to various tuberculostatic drugs. Despite of several months of treatment the patient died of septic multi-organ-failure. CONCLUSION: A possible explanation for the treatment-failure may have been the state of low immunity, either as a result of an underlying disease or after the methylprednisolone therapy. An alternative reason could be the insufficient penetration of the antibiotics into the necrotic abscesses. FAU - Daxecker, M AU - Daxecker M AD - Universität-Klinik für Innere Medizin, Abteilung für Infektiologie und Immunologie, Medizinische Universität Innsbruck. FAU - Weiss, G AU - Weiss G LA - ger PT - Case Reports PT - English Abstract PT - Journal Article TT - Therapierefraktäre Hauttuberkulose nach Methylprednisolon-Therapie. PL - Germany TA - Dtsch Med Wochenschr JT - Deutsche medizinische Wochenschrift (1946) JID - 0006723 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antitubercular Agents) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Abscess/drug therapy/microbiology/surgery MH - Anti-Inflammatory Agents/adverse effects/*therapeutic use MH - Antitubercular Agents/*pharmacology/therapeutic use MH - Diagnosis, Differential MH - Drug Resistance, Multiple, Bacterial MH - Fatal Outcome MH - Humans MH - Lupus Erythematosus, Systemic/diagnosis/drug therapy MH - Male MH - Methylprednisolone/adverse effects/*therapeutic use MH - Middle Aged MH - Multiple Organ Failure/etiology MH - Mycobacterium tuberculosis/*drug effects/isolation & purification MH - Skin/microbiology MH - Sputum/microbiology MH - Treatment Failure MH - Tuberculosis, Cutaneous/*diagnosis/*drug therapy EDAT- 2008/02/14 09:00 MHDA- 2008/02/19 09:00 CRDT- 2008/02/14 09:00 PHST- 2008/02/14 09:00 [pubmed] PHST- 2008/02/19 09:00 [medline] PHST- 2008/02/14 09:00 [entrez] AID - 10.1055/s-2008-1046716 [doi] PST - ppublish SO - Dtsch Med Wochenschr. 2008 Feb;133(8):346-9. doi: 10.1055/s-2008-1046716. PMID- 33879896 OWN - NLM STAT- MEDLINE DCOM- 20210423 LR - 20231111 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 53 IP - 2 DP - 2021 Mar 4 TI - [Clinical characteristics and related factors of systemic lupus erythematosus with interstitial pneumonia]. PG - 266-272 AB - OBJECTIVE: To investigate the clinical features, radiologic scores and clinically relevant risk factors prognosis of secondary interstitial lung disease (ILD) in patients with systemic lupus erythematosus (SLE). METHODS: In this study, 60 SLE patients in Department of Rheumatology of the First Affiliated Hospital of Baotou Medical College and Taizhou First People's Hospital from January 2015 to March 2019 were retrospectively analyzed. All of those 60 patients with SLE underwent lung high resolution computed tomography (HRCT) examination. We used a 1 ∶1 case-control study. There was a matching of age and gender between the two groups. Thirty patients with SLE related ILD (SLE-ILD) were in the case group, and 30 patients with SLE without ILE (SLE non-ILD) were in the control group. The clinical features, pulmonary function test, radiologic characteristic of SLE patients were collected and were used to analyze SLE-ILD. RESULTS: In this study, we reached the following conclusions: First, there were statistically significant differences in chest tightness/shortness of breath, Raynaud's phenomenon, and Velcro rale between SLE-ILD and SLE non-ILD patients (both P < 0.05); Second, hemoglobin (Hb) and albumin (ALB) in the patients of SLE-ILD had a significant decrease compared with the patients of SLE non-ILD. Blood urea nitrogen (BUN), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) increased in SLE-ILD patients compared with SLE non-ILD patients, the difference had statistical significance (P < 0.05); Third, for SLE-ILD patients, the most common type was non-specific interstitial pneumonia (NSIP), followed by usual interstitial pneumonia and lymphocytic interstitial pneumonia; Fourth, there was no significant difference in clinical-radiology-physiology scores between the different ILD types (P>0.05), similarly, the lung HRCT score and lung function between different ILD types had no significant difference (P>0.05); Fifth, multivariate Logistic regression analysis showed that decreased albumin and chest tightness/shortness of breath might be the risk factor for SLE-ILD. CONCLUSION: There are statistically significant differences between the SLE-ILD group and SLE non-ILD group in terms of chest tightness/shortness of breath, Velcro rale and Raynaud's phenomenon. Decreased albumin and chest tightness/shortness of breath in SLE patients should be alerted to the occurrence of ILD. NSIP is the most common manifestation of SLE-ILD. FAU - Xia, F F AU - Xia FF AD - Department of Rheumatology, First Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou 014010, Inner Mongolia, China. FAU - Lu, F A AU - Lu FA AD - Department of Rheumatology, First Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou 014010, Inner Mongolia, China. FAU - Lv, H M AU - Lv HM AD - Department of Rheumatology, First Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou 014010, Inner Mongolia, China. FAU - Yang, G A AU - Yang GA AD - Central Laboratory, First Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou 014010, Inner Mongolia, China. FAU - Liu, Y AU - Liu Y AD - Department of Rheumatology, Taizhou First People's Hospital, Taizhou 318000, Zhejiang, China. LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Case-Control Studies MH - Humans MH - Lung/diagnostic imaging MH - *Lung Diseases, Interstitial/etiology MH - *Lupus Erythematosus, Systemic/complications MH - Retrospective Studies PMC - PMC8072446 OTO - NOTNLM OT - Clinical features OT - Interstitial lung diseases OT - Risk factors OT - Systemic lupus erythematosus EDAT- 2021/04/22 06:00 MHDA- 2021/04/24 06:00 PMCR- 2021/04/18 CRDT- 2021/04/21 06:38 PHST- 2021/04/21 06:38 [entrez] PHST- 2021/04/22 06:00 [pubmed] PHST- 2021/04/24 06:00 [medline] PHST- 2021/04/18 00:00 [pmc-release] AID - bjdxxbyxb-53-2-266 [pii] AID - 10.19723/j.issn.1671-167X.2021.02.006 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Mar 4;53(2):266-272. doi: 10.19723/j.issn.1671-167X.2021.02.006. PMID- 37998293 OWN - NLM STAT- MEDLINE DCOM- 20231127 LR - 20250911 IS - 1660-4601 (Electronic) IS - 1661-7827 (Print) IS - 1660-4601 (Linking) VI - 20 IP - 22 DP - 2023 Nov 14 TI - Validation of the Portuguese Version of the Scleroderma Health Assessment Questionnaire. LID - 10.3390/ijerph20227062 [doi] LID - 7062 AB - The Health Assessment Questionnaire Disability Index (HAQ-DI) was completed with five visual analog scales to assess systemic sclerosis (SSc) called Scleroderma HAQ (SHAQ). We performed a validation of the European Portuguese version of SHAQ for patients with SSc. Patients with different forms of SSc from five Hospital Centers were invited. The reliability of the Portuguese SHAQ was evaluated by internal consistency and by test-retest reliability. Content validity was checked by two rheumatologists and by a panel of patients. Construct validity was assessed by structural validity and by known-groups hypothesis tests. Criterion validity was addressed with selected dimensions from the UCLA GIT 2.0, the SF-36v2, and the EuroQoL EQ-5D-5L. A total of 102 SSc patients agreed to participate, 31 of which answered to the retest. HAQ-DI demonstrated high internal consistency reliability (α = 0.866) and SHAQ also showed high test-retest reliability (ICC 0.61-0.95). We evidenced the unidimensionality of all VASs. HAQ-DI scores were worse in males, patients older than 65 years, and individuals with a diffuse form of SSc. Criterion validity was mainly evidenced through the correlation between the HAQ-DI and SF-36v2 physical summary measure (r = -0.688) and EQ-5D-5L index score (r = -0.723). Likewise, the SHAQ overall disease severity VAS was also correlated with SF-36v2 physical summary measure (r = -0.628). Mental score correlations were smaller. With the exception of the Raynaud's VAS, all the other VASs correlated well with similar clinical variables. This paper provides evidence to demonstrate how reliable and valid the European Portuguese version of SHAQ is, to be used in SSc patients to assess the clinical severity under the perspective of patients. FAU - Genrinho, Inês AU - Genrinho I AD - Rheumatology Department, Tondela Viseu Hospital Centre, 3504-509 Viseu, Portugal. AD - Rheumatology Department, Baixo Vouga Hospital Centre, 3810-164 Aveiro, Portugal. FAU - Ferreira, Pedro L AU - Ferreira PL AUID- ORCID: 0000-0002-9448-9542 AD - Centre for Health Studies and Research, Faculty of Economics, University of Coimbra, 3004-512 Coimbra, Portugal. FAU - Santiago, Tânia AU - Santiago T AD - Rheumatology Department, Hospital and University Centre of Coimbra, 3004-512 Coimbra, Portugal. FAU - Carones, Adriana AU - Carones A AUID- ORCID: 0000-0001-6853-7670 AD - Rheumatology Department, Hospital and University Centre of Coimbra, 3004-512 Coimbra, Portugal. FAU - Mazeda, Carolina AU - Mazeda C AD - Rheumatology Department, Baixo Vouga Hospital Centre, 3810-164 Aveiro, Portugal. AD - EpiDoC Unit, CEDOC, NOVA Medical School, NOVA University of Lisbon, 1169-056 Lisbon, Portugal. FAU - Barcelos, Anabela AU - Barcelos A AUID- ORCID: 0000-0002-4116-8964 AD - Rheumatology Department, Baixo Vouga Hospital Centre, 3810-164 Aveiro, Portugal. AD - EpiDoC Unit, CEDOC, NOVA Medical School, NOVA University of Lisbon, 1169-056 Lisbon, Portugal. AD - Comprehensive Health Research Center (CHRC), NOVA University of Lisbon, 1169-056 Lisbon, Portugal. FAU - Beirão, Tiago AU - Beirão T AD - Rheumatology Department, Vila Nova de Gaia Espinho Hospital Center, 4434-502 Vila Nova de Gaia, Portugal. FAU - Costa, Flávio AU - Costa F AD - Rheumatology Department, Vila Nova de Gaia Espinho Hospital Center, 4434-502 Vila Nova de Gaia, Portugal. FAU - Santos, Inês AU - Santos I AD - Rheumatology Department, Tondela Viseu Hospital Centre, 3504-509 Viseu, Portugal. FAU - Couto, Maura AU - Couto M AD - Rheumatology Department, Tondela Viseu Hospital Centre, 3504-509 Viseu, Portugal. FAU - Rato, Maria AU - Rato M AD - Rheumatology Department, Hospital Centre of São João, 4200-319 Porto, Portugal. FAU - Terroso, Georgina AU - Terroso G AD - Rheumatology Department, Hospital Centre of São João, 4200-319 Porto, Portugal. FAU - Monteiro, Paulo AU - Monteiro P AD - Rheumatology Department, Tondela Viseu Hospital Centre, 3504-509 Viseu, Portugal. LA - eng PT - Journal Article DEP - 20231114 PL - Switzerland TA - Int J Environ Res Public Health JT - International journal of environmental research and public health JID - 101238455 SB - IM MH - Male MH - Humans MH - Reproducibility of Results MH - Portugal MH - Surveys and Questionnaires MH - Severity of Illness Index MH - *Scleroderma, Systemic/diagnosis MH - Quality of Life MH - Disability Evaluation PMC - PMC10671070 OTO - NOTNLM OT - SHAQ OT - outcome assessment OT - quality of life OT - systemic sclerosis COIS- The authors have no competing interests to declare that are relevant to the content of this article. EDAT- 2023/11/24 12:42 MHDA- 2023/11/27 16:18 PMCR- 2023/11/14 CRDT- 2023/11/24 09:35 PHST- 2023/10/05 00:00 [received] PHST- 2023/11/09 00:00 [revised] PHST- 2023/11/11 00:00 [accepted] PHST- 2023/11/27 16:18 [medline] PHST- 2023/11/24 12:42 [pubmed] PHST- 2023/11/24 09:35 [entrez] PHST- 2023/11/14 00:00 [pmc-release] AID - ijerph20227062 [pii] AID - ijerph-20-07062 [pii] AID - 10.3390/ijerph20227062 [doi] PST - epublish SO - Int J Environ Res Public Health. 2023 Nov 14;20(22):7062. doi: 10.3390/ijerph20227062. PMID- 21618208 OWN - NLM STAT- MEDLINE DCOM- 20130125 LR - 20181201 IS - 1529-0131 (Electronic) IS - 0004-3591 (Linking) VI - 63 IP - 10 DP - 2011 Oct TI - The specific free radical scavenger edaravone suppresses fibrosis in the bleomycin-induced and tight skin mouse models of systemic sclerosis. PG - 3086-97 LID - 10.1002/art.30470 [doi] AB - OBJECTIVE: Patients with systemic sclerosis (SSc) exhibit enhanced production of free radicals due to ischemia and reperfusion injury following Raynaud's phenomenon, an initial clinical manifestation. Oxidative stress induces cytokine production, inflammatory cell recruitment, and tissue injury in several inflammatory diseases. The aim of this study was to examine the effect of edaravone, a free radical scavenger, on the development of fibrosis and autoimmunity in two different mouse models of SSc. METHODS: The bleomycin-induced SSc model in mice and the tight skin mouse model were used to evaluate the effect of edaravone on fibrosis and immunologic abnormalities. To assess the reaction of fibroblasts to stimulation with free radicals, fibroblasts from these mice were cultured with NONOate, a nitric oxide-releasing agent, and hydrogen peroxide. RESULTS: Treatment with edaravone reduced fibrosis in mice with bleomycin-induced SSc and in TSK/+ mice. The production of free radicals was also attenuated by edaravone in both models. In addition, production of fibrogenic cytokines such as interleukin-6 and transforming growth factor β1, production of anti-topoisomerase I antibody, and the degree of hypergammaglobulinemia were reduced by edaravone. Furthermore, bleomycin induced the production of H2O2 and nitric oxide from inflammatory cells, and collagen production was increased in fibroblasts cultured with H2O2 and NONOate. CONCLUSION: This study is the first to show that edaravone has a significant inhibitory effect on fibrosis both in the bleomycin-induced SSc model and in TSK/+ mice. These results indicate that edaravone should be further evaluated for potential use as an antifibrotic agent in SSc. CI - Copyright © 2011 by the American College of Rheumatology. FAU - Yoshizaki, Ayumi AU - Yoshizaki A AD - Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Yanaba, Koichi AU - Yanaba K FAU - Ogawa, Asako AU - Ogawa A FAU - Iwata, Yohei AU - Iwata Y FAU - Ogawa, Fumihide AU - Ogawa F FAU - Takenaka, Motoi AU - Takenaka M FAU - Shimizu, Kazuhiro AU - Shimizu K FAU - Asano, Yoshihide AU - Asano Y FAU - Kadono, Takafumi AU - Kadono T FAU - Sato, Shinichi AU - Sato S LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Free Radical Scavengers) RN - 11056-06-7 (Bleomycin) RN - S798V6YJRP (Edaravone) RN - T3CHA1B51H (Antipyrine) SB - IM MH - Animals MH - Antipyrine/*analogs & derivatives/pharmacology/therapeutic use MH - Bleomycin MH - Disease Models, Animal MH - Edaravone MH - Fibrosis/chemically induced/drug therapy/pathology MH - Free Radical Scavengers/pharmacology/*therapeutic use MH - Mice MH - Pulmonary Fibrosis/chemically induced/*drug therapy/pathology MH - Scleroderma, Systemic/chemically induced/*drug therapy/pathology MH - Skin/*drug effects/pathology MH - Treatment Outcome EDAT- 2011/05/28 06:00 MHDA- 2013/01/26 06:00 CRDT- 2011/05/28 06:00 PHST- 2011/05/28 06:00 [entrez] PHST- 2011/05/28 06:00 [pubmed] PHST- 2013/01/26 06:00 [medline] AID - 10.1002/art.30470 [doi] PST - ppublish SO - Arthritis Rheum. 2011 Oct;63(10):3086-97. doi: 10.1002/art.30470. PMID- 40385096 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250929 LR - 20260129 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 2 DP - 2025 Jun TI - Isolated scleroderma of the lower extremities misdiagnosed as lymphedema and presenting with scleroderma renal crisis. PG - NP1-NP4 LID - 10.1177/23971983251334505 [doi] AB - Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis, vasculopathy, and immune dysregulation. The disease commonly presents with Raynaud's phenomenon and skin thickening, commonly of the upper limb. Isolated lower extremity presentation is uncommon and often misdiagnosed. This diagnostic uncertainty may lead to delayed recognition and increased morbidity, particularly when SSc mimics lymphedema in the early stages. We report a case of a 61-year-old female who initially presented with lower extremity swelling and was misdiagnosed with primary lymphedema. Despite treatment with diuretics and compression therapy, her symptoms progressed to involve her upper extremities, prompting further evaluation. Physical examination revealed non-pitting lower extremity scleroderma, sclerodactyly, puffy hands, and nailfold capillary abnormalities. Laboratory workup was positive for anti-RNA polymerase III antibodies, a marker associated with an increased risk of scleroderma renal crisis. The patient developed scleroderma renal crisis after a delayed diagnosis, necessitating hospital admission and initiation of angiotensin-converting enzyme inhibitors. This case highlights the challenges in distinguishing early lower extremity SSc from lymphedema. Early identification of atypical SSc presentations is critical to be cognizant of life-threatening complications such as scleroderma renal crisis. Clinicians should maintain a high index of suspicion for SSc in patients with persistent non-pitting lower extremity swelling, skin thickening, and abnormal capillaroscopy findings, even in the absence of initial upper limb involvement. CI - © The Author(s) 2025. FAU - Ali, Hammad AU - Ali H AUID- ORCID: 0000-0003-1328-480X AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - Gomes, Lais Lopes Almeida AU - Gomes LLA AUID- ORCID: 0000-0001-5396-4362 AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - Khosravi-Hafshejani, Touraj AU - Khosravi-Hafshejani T AUID- ORCID: 0000-0001-5395-8480 AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - On, Aretha AU - On A AUID- ORCID: 0009-0002-3221-5391 AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - Yang, Xiwei AU - Yang X AUID- ORCID: 0009-0007-0325-8989 AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - Chambers, Shae AU - Chambers S AUID- ORCID: 0009-0005-1893-157X AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. FAU - Werth, Victoria P AU - Werth VP AUID- ORCID: 0000-0003-3030-5369 AD - Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, USA. AD - Department of Dermatology, Perelman Center for Advanced Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. LA - eng GR - I01 BX005921/BX/BLRD VA/United States GR - R01 AR071653/AR/NIAMS NIH HHS/United States PT - Case Reports PT - Journal Article DEP - 20250515 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 EIN - J Scleroderma Relat Disord. 2025 Jul 3;10(3):NP12. doi: 10.1177/23971983251355659. PMID: 40620826 PMC - PMC12081387 OTO - NOTNLM OT - Systemic sclerosis OT - lower extremity swelling OT - lymphedema OT - nailfold capillaroscopy OT - scleroderma OT - scleroderma renal crisis COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: V.P.W. has grants from Celgene, Janssen, Pfizer, Biogen, Gilead, Corbus Pharmaceuticals, Genentech, AstraZeneca, Viela, Syntimmune, Amgen, Regeneron, Argenx, CSL Behring, Ventus, q32 Bio, BMS, and Horizon and has consulted for Celgene, Genentech, Janssen, Lilly, Pfizer, Biogen, BMS, Gilead, Amgen, Medscape, Nektar, Incyte, EMD Sorona, CSL Behring, Principia, Crisalis, Viela Bio, Argenx, Kyowa Kirin, Regeneron, Principia, AstraZeneca, Abbvie, Octapharma, GSK, AstraZeneca, Cugene, UCB, Corcept, Beacon Bioscience, Rome Pharmaceuticals, Horizon, Gilead, Merck, Kezar, Sanofi, Bayer, Akari, Calyx, and Cabaletta Bio. The University of Pennsylvania owns the copyright for the CLASI. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. EDAT- 2025/05/19 17:01 MHDA- 2025/05/19 17:02 PMCR- 2026/05/15 CRDT- 2025/05/19 06:08 PHST- 2025/02/21 00:00 [received] PHST- 2025/03/27 00:00 [accepted] PHST- 2026/05/15 00:00 [pmc-release] PHST- 2025/05/19 17:02 [medline] PHST- 2025/05/19 17:01 [pubmed] PHST- 2025/05/19 06:08 [entrez] AID - 10.1177_23971983251334505 [pii] AID - 10.1177/23971983251334505 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2025 May 15;10(2):NP1-NP4. doi: 10.1177/23971983251334505. eCollection 2025 Jun. PMID- 38674921 OWN - NLM STAT- MEDLINE DCOM- 20240427 LR - 20240513 IS - 2072-6643 (Electronic) IS - 2072-6643 (Linking) VI - 16 IP - 8 DP - 2024 Apr 20 TI - L-Carnitine in the Treatment of Psychiatric and Neurological Manifestations: A Systematic Review. LID - 10.3390/nu16081232 [doi] LID - 1232 AB - OBJECTIVE: L-carnitine (LC), a vital nutritional supplement, plays a crucial role in myocardial health and exhibits significant cardioprotective effects. LC, being the principal constituent of clinical-grade supplements, finds extensive application in the recovery and treatment of diverse cardiovascular and cerebrovascular disorders. However, controversies persist regarding the utilization of LC in nervous system diseases, with varying effects observed across numerous mental and neurological disorders. This article primarily aims to gather and analyze database information to comprehensively summarize the therapeutic potential of LC in patients suffering from nervous system diseases while providing valuable references for further research. METHODS: A comprehensive search was conducted in PubMed, Web Of Science, Embase, Ovid Medline, Cochrane Library and Clinicaltrials.gov databases. The literature pertaining to the impact of LC supplementation on neurological or psychiatric disorders in patients was reviewed up until November 2023. No language or temporal restrictions were imposed on the search. RESULTS: A total of 1479 articles were retrieved, and after the removal of duplicates through both automated and manual exclusion processes, 962 articles remained. Subsequently, a meticulous re-screening led to the identification of 60 relevant articles. Among these, there were 12 publications focusing on hepatic encephalopathy (HE), while neurodegenerative diseases (NDs) and peripheral nervous system diseases (PNSDs) were represented by 9 and 6 articles, respectively. Additionally, stroke was addressed in five publications, whereas Raynaud's syndrome (RS) and cognitive disorder (CD) each had three dedicated studies. Furthermore, migraine, depression, and amyotrophic lateral sclerosis (ALS) each accounted for two publications. Lastly, one article was found for other symptoms under investigation. CONCLUSION: In summary, LC has demonstrated favorable therapeutic effects in the management of HE, Alzheimer's disease (AD), carpal tunnel syndrome (CTS), CD, migraine, neurofibromatosis (NF), PNSDs, RS, and stroke. However, its efficacy appears to be relatively limited in conditions such as ALS, ataxia, attention deficit hyperactivity disorder (ADHD), depression, chronic fatigue syndrome (CFS), Down syndrome (DS), and sciatica. FAU - Wang, Wenbo AU - Wang W AUID- ORCID: 0009-0000-4345-1088 AD - Key Laboratory of Environmental Medicine and Engineering of Ministry of Education, and Department of Nutrition and Food Hygiene, School of Public Health, Southeast University, Nanjing 210009, China. FAU - Pan, Da AU - Pan D AD - Key Laboratory of Environmental Medicine and Engineering of Ministry of Education, and Department of Nutrition and Food Hygiene, School of Public Health, Southeast University, Nanjing 210009, China. FAU - Liu, Qi AU - Liu Q AD - Department of Public Health, School of Medicine, Xizang Minzu University, Xianyang 712082, China. FAU - Chen, Xiangjun AU - Chen X AD - Key Laboratory of Environmental Medicine and Engineering of Ministry of Education, and Department of Nutrition and Food Hygiene, School of Public Health, Southeast University, Nanjing 210009, China. AD - Department of Public Health, School of Medicine, Xizang Minzu University, Xianyang 712082, China. FAU - Wang, Shaokang AU - Wang S AUID- ORCID: 0000-0002-7503-7655 AD - Key Laboratory of Environmental Medicine and Engineering of Ministry of Education, and Department of Nutrition and Food Hygiene, School of Public Health, Southeast University, Nanjing 210009, China. AD - Department of Public Health, School of Medicine, Xizang Minzu University, Xianyang 712082, China. LA - eng PT - Journal Article PT - Systematic Review DEP - 20240420 PL - Switzerland TA - Nutrients JT - Nutrients JID - 101521595 RN - S7UI8SM58A (Carnitine) SB - IM MH - Humans MH - *Carnitine/therapeutic use MH - Dietary Supplements MH - *Mental Disorders/drug therapy MH - *Nervous System Diseases/drug therapy PMC - PMC11055039 OTO - NOTNLM OT - L-carnitine OT - mechanism of action OT - neurology OT - psychiatry COIS- The authors have no conflicts of interest to declare. EDAT- 2024/04/27 09:57 MHDA- 2024/04/27 09:58 PMCR- 2024/04/20 CRDT- 2024/04/27 01:19 PHST- 2024/03/21 00:00 [received] PHST- 2024/04/13 00:00 [revised] PHST- 2024/04/19 00:00 [accepted] PHST- 2024/04/27 09:58 [medline] PHST- 2024/04/27 09:57 [pubmed] PHST- 2024/04/27 01:19 [entrez] PHST- 2024/04/20 00:00 [pmc-release] AID - nu16081232 [pii] AID - nutrients-16-01232 [pii] AID - 10.3390/nu16081232 [doi] PST - epublish SO - Nutrients. 2024 Apr 20;16(8):1232. doi: 10.3390/nu16081232. PMID- 36278776 OWN - NLM STAT- MEDLINE DCOM- 20251103 LR - 20251205 IS - 0125-877X (Print) IS - 0125-877X (Linking) VI - 43 IP - 3 DP - 2025 Sep TI - Bosentan versus nifedipine in the treatment of vasculopathy in systemic sclerosis patients: A randomized control trial. PG - 706-711 LID - 10.12932/AP-070722-1406 [doi] AB - BACKGROUND: Bosentan is effective agent in scleroderma vasculopathy. However, there are no studies evaluating effectiveness of bosentan in Vietnamese patients, where nifedipine is still the common treatment. OBJECTIVE: To compare the efficacy of bosentan versus nifedipine in scleroderma vasculopathy in Vietnamese patients. METHODS: We randomly assigned 70 patients in a 2:1 ratio to receive oral bosentan or oral nifedipine for 16 weeks, respectively. The primary outcomes were the change in Raynaud's Condition Score (RCS), appearance of new digital ulcers (DUs) and change in World Health Organization (WHO) functional class. Secondary outcomes were the change in the nailfold capillaries disease stage and systolic pulmonary arterial pressure (sPAP) value. RESULTS: At week 16, patients in bosentan group had no RCS imprvement, the mean difference was 0.8 ± 0.2 (95% CI, 0.4 to 1.1, p < 0.001) and improved WHO functional class, a mean treatment effect of 35.6% in favor of bosentan (95% CI, 13.4 to 57.7%, p < 0.05). Bosentan treatment was associated with a 58% reduction in the number of new DUs compared with nifedipine (mean ± standard error: 0.22 ± 0.42 vs 0.52 ± 0.59 new DUs, p < 0.05). sPAP was decreased by 4.1 ± 3.8 mmHg (95% CI, 3.0 to 5.3, p < 0.001) in bosentan group, versus 1.0 ± 2.9 mmHg (95% CI, -0.2 to 2.1, p > 0.05) in nifedipine group. Headache was the most common adverse event in both groups. CONCLUSIONS: Bosentan significantly limited the occurrence of new DUs, reduced symptoms of pulmonary arterial hypertension and sPAP value and all were better than nifedipine. FAU - Phat, Trinh Ngoc AU - Phat TN AD - Hanoi Medical University, Hanoi, Vietnam. FAU - Luong, Vu Huy AU - Luong VH AD - Hanoi Medical University, Hanoi, Vietnam. AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. FAU - Minh, Vu Nguyet AU - Minh VN AD - Hanoi Medical University, Hanoi, Vietnam. AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. FAU - My, Le Huyen AU - My LH AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. FAU - Phuong, Hoang Thi AU - Phuong HT AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. FAU - Vinh, Nguyen Thi Ha AU - Vinh NTH AD - Hanoi Medical University, Hanoi, Vietnam. AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. FAU - Doanh, Le Huu AU - Doanh LH AD - Hanoi Medical University, Hanoi, Vietnam. AD - National Hospital of Dermatology and Venereology, Hanoi, Vietnam. LA - eng PT - Comparative Study PT - Journal Article PT - Randomized Controlled Trial PL - Thailand TA - Asian Pac J Allergy Immunol JT - Asian Pacific journal of allergy and immunology JID - 8402034 RN - Q326023R30 (Bosentan) RN - I9ZF7L6G2L (Nifedipine) RN - 0 (Sulfonamides) RN - 0 (Antihypertensive Agents) SB - IM MH - Humans MH - Bosentan MH - *Nifedipine/therapeutic use/adverse effects/administration & dosage MH - Female MH - Male MH - *Scleroderma, Systemic/drug therapy/complications MH - Middle Aged MH - *Sulfonamides/therapeutic use/adverse effects/administration & dosage MH - Adult MH - Treatment Outcome MH - *Antihypertensive Agents/therapeutic use MH - Aged EDAT- 2022/10/25 06:00 MHDA- 2025/11/03 18:30 CRDT- 2022/10/24 06:43 PHST- 2025/11/03 18:30 [medline] PHST- 2022/10/25 06:00 [pubmed] PHST- 2022/10/24 06:43 [entrez] AID - 10.12932/AP-070722-1406 [doi] PST - ppublish SO - Asian Pac J Allergy Immunol. 2025 Sep;43(3):706-711. doi: 10.12932/AP-070722-1406. PMID- 35313985 OWN - NLM STAT- MEDLINE DCOM- 20220324 LR - 20220325 IS - 1757-6512 (Electronic) IS - 1757-6512 (Linking) VI - 13 IP - 1 DP - 2022 Mar 21 TI - Efficacy and safety of mesenchymal stem cells in the treatment of systemic sclerosis: a systematic review and meta-analysis. PG - 118 LID - 10.1186/s13287-022-02786-3 [doi] LID - 118 AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease with high morbidity and mortality characterized by fibrosis of the skin and internal organs. Some studies have investigated the use of stem cells to treat SSc. Herein, a systematic review and meta-analysis was conducted to determine the efficacy and safety of mesenchymal stem cells (MSCs) in the treatment of SSc. METHODS: PubMed, Embase, Cochrane Library, Web of Science, OVID, China National Knowledge Infrastructure and Wanfang databases were searched up to February 1, 2021. Literature screening, data extraction and quality assessment were conducted independently by two researchers in according to the inclusion and exclusion criteria. The discrepancies were resolved by a third researcher. RESULTS: A total of 9 studies encompassing 133 SSc patients were included in the study. Compared to the baseline after treatment with MSCs: 1. The modified Rodnan skin score (mRSS) was significantly reduced in patients with SSc (P < 0.00001). 2. MSCs decreased the number of digital ulcer, mouth handicap scale, and visual analog scale of hand pain in SSc patients (P = 0.0007 and P = 0.03, respectively). 3. No statistical differences were detected in Raynaud's condition score and Cochin hand function scale score at 6 months of MSCs therapy (P = 0.5 and P = 0.62). 4. After 12 months of follow-up, MSCs improve carbon monoxide diffusing capacity and forced vital capacity of SSc patients (P < 0.05). 5. Overall, MSCs application was safe; a few cases exhibited swelling at the injection site, diarrhea and arthralgia, which had self-recovery, and no severe adverse events occurred in the included trials. CONCLUSIONS: MSC therapy improves the degree of skin thickening, lung function, and mouth opening and relieves finger ulcers and pain in patients with SSc without severe adverse events. Thus, MSCs or MSCs combined with plasma and traditional medicine might be an effective and promising treatment of SSc patients. PROSPERO registration number: CRD42020200350. CI - © 2022. The Author(s). FAU - Cui, Jiehan AU - Cui J AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Jin, Lu AU - Jin L AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Ding, Meng AU - Ding M AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - He, Jingjing AU - He J AD - Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Yang, Lin AU - Yang L AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Cui, Shaoxin AU - Cui S AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Wang, Xiaoping AU - Wang X AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Ma, Jun AU - Ma J AD - Hebei Research Center for Stem Cell Medical Translational Engineering, Shijiazhuang, 050000, Hebei Province, China. AD - Department of Anatomy, Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. FAU - Liu, Aijing AU - Liu A AUID- ORCID: 0000-0002-5762-5592 AD - Department of Rheumatology and Immunology, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. laj111@126.com. AD - Hebei Research Center for Stem Cell Medical Translational Engineering, Shijiazhuang, 050000, Hebei Province, China. laj111@126.com. AD - Hebei Key Laboratory of Laboratory Medicine, the Second Hospital of Hebei Medical University, Shijiazhuang, 050000, Hebei Province, China. laj111@126.com. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20220321 PL - England TA - Stem Cell Res Ther JT - Stem cell research & therapy JID - 101527581 SB - IM MH - *Autoimmune Diseases MH - China MH - Humans MH - *Mesenchymal Stem Cells MH - *Scleroderma, Systemic/therapy MH - Skin PMC - PMC8935249 OTO - NOTNLM OT - Mesenchymal stem cells OT - Meta-analysis OT - Systemic sclerosis OT - Treatment COIS- The authors declare that they have no competing interests. EDAT- 2022/03/23 06:00 MHDA- 2022/03/25 06:00 PMCR- 2022/03/21 CRDT- 2022/03/22 05:31 PHST- 2021/11/09 00:00 [received] PHST- 2022/02/17 00:00 [accepted] PHST- 2022/03/22 05:31 [entrez] PHST- 2022/03/23 06:00 [pubmed] PHST- 2022/03/25 06:00 [medline] PHST- 2022/03/21 00:00 [pmc-release] AID - 10.1186/s13287-022-02786-3 [pii] AID - 2786 [pii] AID - 10.1186/s13287-022-02786-3 [doi] PST - epublish SO - Stem Cell Res Ther. 2022 Mar 21;13(1):118. doi: 10.1186/s13287-022-02786-3. PMID- 32760879 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240329 IS - 2520-1026 (Electronic) IS - 2520-1026 (Linking) VI - 4 DP - 2020 TI - Room for improvement in non-pharmacological systemic sclerosis care? - a cross-sectional online survey of 650 patients. PG - 43 LID - 10.1186/s41927-020-00142-7 [doi] LID - 43 AB - BACKGROUND/ OBJECTIVE: To gain insight in the use of current systemic sclerosis (SSc) care provided by health professionals from the patient perspective. We focused on referral reasons, treatment goals, the alignment with unmet care needs, and outcome satisfaction. METHODS: Dutch SSc patients from 13 participating rheumatology departments were invited to complete an online survey. Descriptive statistics were used to describe current use of non-pharmacological care and outcome satisfaction. Reasons for referral and treatment goals were encoded in International Classification of Function and Disability (ICF) terms. RESULTS: We included 650 patients (mean (standard deviation [SD]) age, 59.4 (11.4) years. 50% had contact with a health professional in the past year; 76.3% since disease onset. Physiotherapists were the most frequently visited in the past year (40.0%), followed by dental hygienists (11.4%) and podiatrists (9.2%). The three most common referral reasons were pain, joint mobility and cardiovascular functions. Fatigue, Raynaud's phenomenon, physical limitations, reduced hand function and joint problems were mentioned by more than 25% of all respondents as unmet needs. The proportion of patients treated in the past year by a health professional who were satisfied with knowledge and expertise of their health professionals was 74.4%; 73% reported improved daily activities and better coping with complaints. However, 48.9% perceived that the collaboration between rheumatologist and health professional was never or only sometimes sufficient. CONCLUSION: Despite the high outcome satisfaction and good accessibility of health professionals, there are obstacles in the access to non-pharmacological care and communication barriers between health professionals and rheumatologists. CI - © The Author(s) 2020. FAU - Stöcker, Juliane K AU - Stöcker JK AUID- ORCID: 0000-0002-3852-1157 AD - Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands. GRID: grid.452818.2. ISNI: 0000 0004 0444 9307 AD - Musculoskeletal Rehabilitation Research Group, HAN University of Applied Sciences, Nijmegen, The Netherlands. GRID: grid.450078.e. ISNI: 0000 0000 8809 2093 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 FAU - van den Hoogen, Frank H J AU - van den Hoogen FHJ AD - Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands. GRID: grid.452818.2. ISNI: 0000 0004 0444 9307 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 FAU - Nijhuis-van der Sanden, Maria W G AU - Nijhuis-van der Sanden MWG AD - Radboud Institute for Health Sciences, IQ Healthcare, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 FAU - Spierings, Julia AU - Spierings J AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. GRID: grid.7692.a. ISNI: 0000000090126352 FAU - Staal, J Bart AU - Staal JB AD - Musculoskeletal Rehabilitation Research Group, HAN University of Applied Sciences, Nijmegen, The Netherlands. GRID: grid.450078.e. ISNI: 0000 0000 8809 2093 AD - Radboud Institute for Health Sciences, IQ Healthcare, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 FAU - Satink, Ton AU - Satink T AD - Research Group Neuro Rehabilitation, HAN University of Applied Sciences, Nijmegen, The Netherlands. GRID: grid.450078.e. ISNI: 0000 0000 8809 2093 AD - European Masters of Science in Occupational Therapy, HvA University of Applied Sciences, Amsterdam, The Netherlands. GRID: grid.5477.1. ISNI: 0000000120346234 FAU - van den Ende, Cornelia H M AU - van den Ende CHM AD - Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands. GRID: grid.452818.2. ISNI: 0000 0004 0444 9307 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. GRID: grid.10417.33. ISNI: 0000 0004 0444 9382 CN - ARCH study group LA - eng PT - Journal Article DEP - 20200731 PL - England TA - BMC Rheumatol JT - BMC rheumatology JID - 101738571 PMC - PMC7393838 OTO - NOTNLM OT - Cross sectional survey OT - Health care use OT - Health professionals OT - Systemic sclerosis COIS- Competing interestsThe authors declare: There are no benefits from commercial sources, no other financial interests and no conflicts of interest. EDAT- 2020/08/08 06:00 MHDA- 2020/08/08 06:01 PMCR- 2020/07/31 CRDT- 2020/08/08 06:00 PHST- 2020/03/08 00:00 [received] PHST- 2020/05/28 00:00 [accepted] PHST- 2020/08/08 06:00 [entrez] PHST- 2020/08/08 06:00 [pubmed] PHST- 2020/08/08 06:01 [medline] PHST- 2020/07/31 00:00 [pmc-release] AID - 142 [pii] AID - 10.1186/s41927-020-00142-7 [doi] PST - epublish SO - BMC Rheumatol. 2020 Jul 31;4:43. doi: 10.1186/s41927-020-00142-7. eCollection 2020. PMID- 30093237 OWN - NLM STAT- MEDLINE DCOM- 20190603 LR - 20190603 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 48 IP - 5 DP - 2019 Apr TI - Foot and ankle characteristics in systemic lupus erythematosus: A systematic review and meta-analysis. PG - 847-859 LID - S0049-0172(18)30234-8 [pii] LID - 10.1016/j.semarthrit.2018.07.002 [doi] AB - OBJECTIVE: To determine characteristics of the foot and ankle in people with systemic lupus erythematosus (SLE). METHODS: Medline, CINAHL, Sports-Discus, Scopus and Cochrane Library databases were searched up to January 2018. Studies reporting foot- and ankle-related outcomes in the following domains were included: vascular, neurological, musculoskeletal, cutaneous (skin and nail) or pain/function. The Quality Index tool was used to assess methodological quality. Where appropriate, odds ratio (OR) and mean difference meta-analyses were conducted for case-control studies; and pooled mean prevalence meta-analyses for studies assessing characteristics in SLE. RESULTS: Forty-nine studies were included with mean (range) quality scores of 75% (38-100%). Twenty-three studies assessed vascular characteristics, followed by musculoskeletal (n = 16), neurological (n = 11), cutaneous (n = 5) and pain/function (n = 4). Foot and ankle characteristics in people with SLE included impaired vascular supply, abnormal nerve function, musculoskeletal pathology, skin and nail pathology, and pain and functional disability. Twenty-four studies were included in meta-analyses. Pooled OR for abnormal ankle brachial index was 3.08 for SLE compared with controls. Pooled mean difference in brachial-ankle pulse-wave velocity between SLE and controls was significant (161.39 cm/s, P = 0.004). Pooled prevalence was 0.54 for intermittent claudication, 0.50 for Raynaud's phenomenon, 0.28 for chilblains, 0.00 for gangrene, 0.30 for hallux valgus, 0.15 for onychomycosis, 0.76 for history of foot pain, and 0.36 for current foot pain. CONCLUSION: People with SLE experience a wide range of foot and ankle manifestations. Published research highlights the impact of peripheral arterial disease, peripheral neuropathy, musculoskeletal deformity, skin and nail pathology and patient-reported foot pain and disability. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Stewart, Sarah AU - Stewart S AD - Health and Rehabilitation Research Institute, Auckland University of Technology, Private Bag 92006, Auckland 1142, New Zealand. Electronic address: sarah.stewart@aut.ac.nz. FAU - Brenton-Rule, Angela AU - Brenton-Rule A AD - Health and Rehabilitation Research Institute, Auckland University of Technology, Private Bag 92006, Auckland 1142, New Zealand. FAU - Dalbeth, Nicola AU - Dalbeth N AD - Faculty of Medical and Health Sciences, The University of Auckland, Private Bag 92019, Auckland 1142, New Zealand; Department of Rheumatology, Auckland District Health Board, New Zealand. FAU - Aiyer, Ashok AU - Aiyer A AD - Health and Rehabilitation Research Institute, Auckland University of Technology, Private Bag 92006, Auckland 1142, New Zealand. FAU - Frampton, Christopher AU - Frampton C AD - Biostatistics Department, University of Otago, PO Box 7343, Wellington South, New Zealand. FAU - Rome, Keith AU - Rome K AD - Health and Rehabilitation Research Institute, Auckland University of Technology, Private Bag 92006, Auckland 1142, New Zealand. LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20180806 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Case-Control Studies MH - Female MH - Foot Diseases/*etiology MH - Foot Joints/blood supply/innervation/*physiopathology MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Male MH - Pain/etiology OTO - NOTNLM OT - Ankle OT - Foot OT - Systematic review OT - Systemic lupus erythematosus EDAT- 2018/08/11 06:00 MHDA- 2019/06/04 06:00 CRDT- 2018/08/11 06:00 PHST- 2018/04/26 00:00 [received] PHST- 2018/06/14 00:00 [revised] PHST- 2018/07/06 00:00 [accepted] PHST- 2018/08/11 06:00 [pubmed] PHST- 2019/06/04 06:00 [medline] PHST- 2018/08/11 06:00 [entrez] AID - S0049-0172(18)30234-8 [pii] AID - 10.1016/j.semarthrit.2018.07.002 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2019 Apr;48(5):847-859. doi: 10.1016/j.semarthrit.2018.07.002. Epub 2018 Aug 6. PMID- 27704312 OWN - NLM STAT- MEDLINE DCOM- 20170411 LR - 20181113 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 35 IP - 12 DP - 2016 Dec TI - Prediction of Antiphospholipid syndrome using Annexin A5 competition assay in patients with SLE. PG - 2933-2938 AB - A significantly high correlation between reduced activity of Annexin A5 by the flow cytometric assay (FCA) and the diagnosis of antiphospholipid syndrome (APS) has been reported. The aim of this study was to assess the clinical and laboratory significance of the Annexin A5 competition assay among patients with systemic lupus erythematosus (SLE). The FCA competition assay was performed on blood samples from 57 consecutive SLE patients. The FCA was performed according to a previously validated method. Forty-seven patients (82.5 %) had SLE without APS and ten (17.5 %) had SLE with APS. Twenty-four (42 %) of the patients had mean levels of AnxA5 fluorescence below the mean and standard deviation of the controls and were considered positive. SLE patients with a positive FCA were found to have an increased risk for a hypercoagulable or vascular state (86 % of the patients had cerebrovascular disease, 89 % had Raynaud's phenomenon, and 80 % had deep vein thrombosis). The risk for any hypercoagulable or vascular state was significantly increased (P = 0.012, RR-2.3, 95 % CI 1.4-3.8). A positive FCA assay was found in 90 % of the patients with APS (P < 0.001), with a sensitivity of 90 % and a specificity of 68 % for this diagnosis. The positive and negative predictive values were 0.4 and 0.97, respectively. Correlations were found between positive FCA and positive Anti-Cardiolipin antibody (P < 0.001), and Anti-β2 glycoprotein I levels (P = 0.013). Our findings suggest that the FCA is a practical assay for the detection of clinically relevant APS among patients with SLE. FAU - Avriel, Avital AU - Avriel A AD - Department of Medicine, Soroka Medical Center, Faculty of Health Sciences Ben-Gurion University, Beer-Sheva, Israel. FAU - Fleischer, Stela AU - Fleischer S AD - Blood Bank and Transfusion Medicine, Soroka University Medical Centre, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. FAU - Friger, Michael AU - Friger M AD - Department of Public Health, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. FAU - Shovman, Ora AU - Shovman O AD - Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Sackler Faculty of Medicine in Tel-Aviv University, Tel-Aviv, Israel. FAU - Neuman, Gal AU - Neuman G AD - Rambam Medical Center and Technion Faculty of Medicine, Haifa, Israel. FAU - Shoenfeld, Yehuda AU - Shoenfeld Y AD - Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Sackler Faculty of Medicine in Tel-Aviv University, Tel-Aviv, Israel. FAU - Abu-Shakra, Mahmoud AU - Abu-Shakra M AD - Department of Medicine, Soroka Medical Center, Faculty of Health Sciences Ben-Gurion University, Beer-Sheva, Israel. Mahmoud@bgu.ac.il. AD - Rheumatic Diseases Unit, Soroka University Medical Centre, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. Mahmoud@bgu.ac.il. LA - eng PT - Journal Article DEP - 20161004 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Annexin A5) RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Adult MH - Annexin A5/*chemistry MH - Antibodies, Anticardiolipin/blood MH - Antiphospholipid Syndrome/*blood/immunology MH - Binding, Competitive MH - Blood Coagulation MH - Blood Platelets/cytology MH - Female MH - Flow Cytometry MH - Fluorescence MH - Humans MH - Lupus Coagulation Inhibitor/immunology MH - Lupus Erythematosus, Systemic/*blood/immunology MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Rheumatology/methods MH - Sensitivity and Specificity OTO - NOTNLM OT - Annexin A5 OT - Antiphospholipid syndrome OT - Flow cytometric assay OT - Systemic lupus erythematosus EDAT- 2016/10/06 06:00 MHDA- 2017/04/12 06:00 CRDT- 2016/10/06 06:00 PHST- 2016/07/19 00:00 [received] PHST- 2016/09/20 00:00 [accepted] PHST- 2016/09/17 00:00 [revised] PHST- 2016/10/06 06:00 [pubmed] PHST- 2017/04/12 06:00 [medline] PHST- 2016/10/06 06:00 [entrez] AID - 10.1007/s10067-016-3428-1 [pii] AID - 10.1007/s10067-016-3428-1 [doi] PST - ppublish SO - Clin Rheumatol. 2016 Dec;35(12):2933-2938. doi: 10.1007/s10067-016-3428-1. Epub 2016 Oct 4. PMID- 24057058 OWN - NLM STAT- MEDLINE DCOM- 20140530 LR - 20151119 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 22 IP - 11 DP - 2013 Oct TI - Clinical outcomes and safety of rituximab treatment for patients with systemic lupus erythematosus (SLE) - results from a nationwide cohort in Germany (GRAID). PG - 1142-9 LID - 10.1177/0961203313503912 [doi] AB - OBJECTIVE: The objective of this article is to evaluate the safety and clinical outcome of rituximab treatment in systemic lupus erythematosus (SLE) patients refractory to standard of care therapy in a real-life setting in Germany. METHODS: The GRAID registry included patients with different autoimmune diseases who were given off-label treatment with rituximab. Data on safety and clinical response were collected retrospectively. In SLE patients, clinical parameters included tender and swollen joint counts, fatigue, myalgia, general wellbeing, Raynaud's and the SLEDAI index. Laboratory tests included dsDNA antibody titres, complement factors, hematologic parameters and proteinuria. Finally, the investigators rated their patients as non-, partial or complete responders based on clinical grounds. RESULTS: Data from 85 SLE patients were collected, 69 female and 16 male, with a mean disease duration of 9.8 years. The mean follow-up period was 9.6 ± 7.4 months, resulting in 66.8 patient years of observation. A complete response was reported in 37 patients (46.8%), partial response in 27 (34.2%), no response in 15 (19.0%). On average, major clinical as well as laboratory efficacy parameters improved substantially, with the SLEDAI decreasing significantly from 12.2 to 3.3 points. Concerning safety, one infusion reaction leading to discontinuation of treatment occurred. Infections were reported with a rate of 19.5 (including six severe infections) per 100 patient years. CONCLUSION: With the restrictions of a retrospective data collection, the results of this study confirm data of other registries, which suggest a favourable benefit-risk ratio of rituximab in patients with treatment-refractory SLE. FAU - Witt, M AU - Witt M AD - 1Division of Rheumatology, Medizinische Klinik und Poliklinik IV, University Hospital Munich, Germany. FAU - Grunke, M AU - Grunke M FAU - Proft, F AU - Proft F FAU - Baeuerle, M AU - Baeuerle M FAU - Aringer, M AU - Aringer M FAU - Burmester, G AU - Burmester G FAU - Chehab, G AU - Chehab G FAU - Fiehn, C AU - Fiehn C FAU - Fischer-Betz, R AU - Fischer-Betz R FAU - Fleck, M AU - Fleck M FAU - Freivogel, K AU - Freivogel K FAU - Haubitz, M AU - Haubitz M FAU - Kötter, I AU - Kötter I FAU - Lovric, S AU - Lovric S FAU - Metzler, C AU - Metzler C FAU - Rubberth-Roth, A AU - Rubberth-Roth A FAU - Schwarting, A AU - Schwarting A FAU - Specker, C AU - Specker C FAU - Tony, H-P AU - Tony HP FAU - Unger, L AU - Unger L FAU - Wassenberg, S AU - Wassenberg S FAU - Dörner, T AU - Dörner T FAU - Schulze-Koops, H AU - Schulze-Koops H CN - German Registry of Autoimmune Diseases (GRAID) Investigators LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus JT - Lupus JID - 9204265 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Adult MH - Antibodies, Monoclonal, Murine-Derived/adverse effects/*therapeutic use MH - Cohort Studies MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*drug therapy MH - Male MH - Off-Label Use MH - Retrospective Studies MH - Rituximab OTO - NOTNLM OT - B lymphocytes OT - Systemic lupus erythematosus OT - cohort studies OT - efficacy OT - rituximab OT - safety EDAT- 2013/09/24 06:00 MHDA- 2014/05/31 06:00 CRDT- 2013/09/24 06:00 PHST- 2013/09/24 06:00 [entrez] PHST- 2013/09/24 06:00 [pubmed] PHST- 2014/05/31 06:00 [medline] AID - 22/11/1142 [pii] AID - 10.1177/0961203313503912 [doi] PST - ppublish SO - Lupus. 2013 Oct;22(11):1142-9. doi: 10.1177/0961203313503912. PMID- 22691207 OWN - NLM STAT- MEDLINE DCOM- 20120813 LR - 20260518 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 30 IP - 2 Suppl 71 DP - 2012 Mar-Apr TI - Best practices in scleroderma: an analysis of practice variability in SSc centres within the Canadian Scleroderma Research Group (CSRG). PG - S38-43 AB - OBJECTIVES: There is currently no consensus on best practice in systemic sclerosis (SSc). To determine if variability in treatment and investigations exists, practices among Canadian Sclerodermia Research Group (CSRG) centres were compared. METHODS: Prospective clinical and demographic data from adult SSc patients are collected annually from 15 CSRG treatment centres. Laboratory parameters, self-reported socio-demographic questionnaires, current and past medications and disease outcome measures are recorded. For centres with >50 patients enrolled, treatment practices were analysed to determine practice variability. RESULTS: Data from 640 of 938 patients within the CSRG database met inclusion criteria, where 87.3% were female, the mean ± SEM age was 55.3±0.5, 48.9% had limited SSc and 47.8% had diffuse SSc (and 3.3% uncharacterised). Some investigation and treatment practices were inconsistent among 6 centres including proportion receiving: PDE5 (phosphodiesterase type 5) inhibitors for Raynaud's phenomenon (p=0.036); cyclophosphamide (p=0.037) and azathioprine (p=0.037) for treatment of ILD; and current use of D-penicillamine, although uncommon, varied among sites. Annual echocardiograms and PFTs were frequently done and did not vary among sites but the rate of pulmonary arterial hypertension (PAH) was directly related to site size and this was not the case for other organ involvement. CONCLUSIONS: Despite routine tests within a database, site variation in SSc with respect to investigations and management among CSRG centres exists suggesting a need for a standardised approach to the investigation and treatment of SSc. One can speculate that larger centres are more export in detecting PAH. FAU - Harding, Sarah AU - Harding S AD - University of Western Ontario, London, ON, Canada. sarahharding@rcsi.ie FAU - Khimdas, Sarit AU - Khimdas S FAU - Bonner, Ashley AU - Bonner A FAU - Baron, Murray AU - Baron M FAU - Pope, Janet AU - Pope J CN - Canadian Scleroderma Research Group LA - eng GR - Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120529 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Benchmarking/*standards MH - Canada MH - Consensus MH - Databases, Factual MH - Diagnostic Tests, Routine/standards MH - Evidence-Based Medicine/standards MH - Female MH - Guideline Adherence/standards MH - Humans MH - Male MH - Middle Aged MH - Outcome and Process Assessment, Health Care/*standards MH - Practice Guidelines as Topic/standards MH - Practice Patterns, Physicians'/*standards MH - Predictive Value of Tests MH - Prospective Studies MH - Quality Indicators, Health Care/*standards MH - Rheumatology/*standards MH - Scleroderma, Systemic/complications/diagnosis/*therapy MH - Severity of Illness Index MH - Time Factors MH - Treatment Outcome FIR - Baron, M IR - Baron M FIR - Hudson, M IR - Hudson M FIR - Markland, J IR - Markland J FIR - Docherty, P IR - Docherty P FIR - Fritzler, M IR - Fritzler M FIR - Jones, N IR - Jones N FIR - Kaminska, E IR - Kaminska E FIR - Khalidi, N IR - Khalidi N FIR - Ligier, S IR - Ligier S FIR - Masetto, A IR - Masetto A FIR - Mathieu, J-P IR - Mathieu JP FIR - Pope, J IR - Pope J FIR - Robinson, D IR - Robinson D FIR - Smith, D IR - Smith D FIR - Sutton, E IR - Sutton E EDAT- 2012/07/26 06:00 MHDA- 2012/08/14 06:00 CRDT- 2012/06/14 06:00 PHST- 2011/02/23 00:00 [received] PHST- 2011/11/22 00:00 [accepted] PHST- 2012/06/14 06:00 [entrez] PHST- 2012/07/26 06:00 [pubmed] PHST- 2012/08/14 06:00 [medline] AID - 4779 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2012 Mar-Apr;30(2 Suppl 71):S38-43. Epub 2012 May 29. PMID- 22147109 OWN - NLM STAT- MEDLINE DCOM- 20130507 LR - 20211021 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 12 DP - 2012 Dec TI - Comparison of qualitative and quantitative analysis of capillaroscopic findings in patients with rheumatic diseases. PG - 3729-35 LID - 10.1007/s00296-011-2222-2 [doi] AB - No guidelines for the application of qualitative and quantitative analysis of the capillaroscopic examination in the rheumatologic practice exist. The aims of the study were to compare qualitative and quantitative analysis of key capillaroscopic parameters in patients with common rheumatic diseases and to assess the reproducibility of the qualitative evaluation of the capillaroscopic parameters, performed by two different investigators. Two hundred capillaroscopic images from 93 patients with different rheumatic diseases were analysed quantitatively and qualitatively by two different investigators. The distribution of the images according to the diagnosis and the microvascular abnormalities was as follows-group 1: 73 images from systemic sclerosis patients ("scleroderma" type pattern), group 2: 10 images from dermatomyositis ("scleroderma-like" pattern), group 3: 25 images from undifferentiated connective tissue disease and different forms of overlap (24 "scleroderma-like"), group 4: 26 images from systemic lupus erythematosus patients, group 5: 46 images from rheumatoid arthritis and group 6: 20 images from primary Raynaud's phenomenon patients. All the images were mixed and blindly presented to both investigators. For comparison of the quantitative and qualitative method, investigator 1 assessed presence of dilated, giant capillaries and avascular areas quantitatively by the available software programme and his estimates were compared with the results of investigator 2, who assessed the parameters qualitatively. In addition, the capillaroscopic images were evaluated qualitatively by the investigator 1 and 2 for presence of dilated, giant capillaries, avascular areas and haemorrhages. The comparison of the quantitative and qualitative assessment of the two investigators demonstrated statistically significant difference between the two methods for the detection of dilated and giant capillaries (P < 0.05) but no significant difference regarding the detection of avascular areas (P > 0.05). As we further analysed the results for the capillaroscopic images, demonstrating a "scleroderma" and a "scleroderma-like" pattern (170/200), analogous results were found for the evaluated parameters. Among the 20 capillaroscopic images from patients with primary RP, the estimates for the absence of giant capillaries and avascular areas were equal in 100% (P > 0.05). Comparing the qualitative assessment of the two investigators, a statistically significant difference between estimates of the two investigators was found for the presence of dilated capillaries (P < 0.05), while for giant capillaries, avascular areas and haemorrhages the difference was not statistically significant (P > 0.05). The results of the study have shown that qualitative assessment of capillaroscopic parameters in patients with rheumatic diseases is an adequate method for the everyday rheumatologic practice, especially in cases with primary RP for exclusion presence of microangiopathy. No significant difference between qualitative and quantitative methods of assessment was found for the detection of avascular areas. However, the quantitative analysis is more precise especially for the detection of capillary dilation. A good reproducibility of the qualitative evaluation, performed by two different investigators was also found. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Medical University, Plovdiv, Bulgaria. sevdalina_n@abv.bg FAU - Hermann, Walter AU - Hermann W FAU - Müller-Ladner, Ulf AU - Müller-Ladner U LA - eng PT - Comparative Study PT - Journal Article DEP - 20111207 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Capillaries/*physiopathology MH - Humans MH - Microscopic Angioscopy/*methods MH - Observer Variation MH - Reproducibility of Results MH - Rheumatic Diseases/*diagnosis/physiopathology EDAT- 2011/12/08 06:00 MHDA- 2013/05/08 06:00 CRDT- 2011/12/08 06:00 PHST- 2011/07/10 00:00 [received] PHST- 2011/10/22 00:00 [accepted] PHST- 2011/12/08 06:00 [entrez] PHST- 2011/12/08 06:00 [pubmed] PHST- 2013/05/08 06:00 [medline] AID - 10.1007/s00296-011-2222-2 [doi] PST - ppublish SO - Rheumatol Int. 2012 Dec;32(12):3729-35. doi: 10.1007/s00296-011-2222-2. Epub 2011 Dec 7. PMID- 20194453 OWN - NLM STAT- MEDLINE DCOM- 20100622 LR - 20151119 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 4 DP - 2010 Apr TI - Similarities and differences between primary and secondary Sjögren's syndrome. PG - 800-8 LID - 10.3899/jrheum.090866 [doi] AB - OBJECTIVE: To define the clinical, serological, and histopathological characteristics of primary (pSS) and secondary Sjögren's syndrome (SS). METHODS: Fifty subjects with pSS and 300 with connective tissue diseases (CTD; systemic lupus erythematosus 100, rheumatoid arthritis 100, scleroderma 100) were selected randomly from our patient registry. Selected patients were assessed for fulfillment of the American-European Consensus Group criteria for SS using a 3-phase approach: screening (European questionnaire, Schirmer-I test, wafer test), confirmatory (fluorescein staining test, nonstimulated whole salivary flow, anti-Ro/La antibodies), and lip biopsy (H&E and immunohistochemical staining for anti-CD20 and anti-CD45RO scored by morphometry). RESULTS: All patients with pSS and 65 with CTD met criteria for SS. Oral symptoms (pSS = 92% and secondary SS = 84%; p = 0.02), parotid enlargement (pSS 56%, secondary SS 9.2%; p < 0.001), and higher prevalence (pSS 82%, secondary SS 41%; p < 0.001) and titers of anti-Ro/La antibodies were more common in pSS. Extraglandular manifestations were similar in both groups, except for Raynaud's phenomenon, which was more common in those with secondary SS (pSS 16% vs secondary SS 41%; p = 0.001). These results remained after 3 different sensitivity analyses. The prevalence of focal infiltration was also similar in both SS varieties; however, a higher B:T cell ratio and higher expression of CD20 cells (2922 vs 607.5 positive cells; p < 0.001) were observed in pSS. CONCLUSION: A higher frequency of oral symptoms and parotid enlargement and stronger B cell activity (autoantibody production and lymphocyte infiltration) were observed in pSS. Whether these results reflect a true difference between the 2 disease entities or derive from underlying variables remains uncertain. FAU - Hernández-Molina, Gabriela AU - Hernández-Molina G AD - Department of Immunology and Rheumatology, Ophthalmology Service, and Dental Service, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, México. FAU - Avila-Casado, Carmen AU - Avila-Casado C FAU - Cárdenas-Velázquez, Francisco AU - Cárdenas-Velázquez F FAU - Hernández-Hernández, Carlos AU - Hernández-Hernández C FAU - Calderillo, María Luisa AU - Calderillo ML FAU - Marroquín, Verónica AU - Marroquín V FAU - Soto-Abraham, Virgilia AU - Soto-Abraham V FAU - Recillas-Gispert, Claudia AU - Recillas-Gispert C FAU - Sánchez-Guerrero, Jorge AU - Sánchez-Guerrero J LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100301 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Autoantibodies/immunology MH - Connective Tissue Diseases/diagnosis/immunology MH - Diagnosis, Differential MH - Female MH - Humans MH - Male MH - Middle Aged MH - Patient Selection MH - Registries MH - Severity of Illness Index MH - Sjogren's Syndrome/*classification/*diagnosis/immunology MH - Statistics, Nonparametric MH - Surveys and Questionnaires EDAT- 2010/03/03 06:00 MHDA- 2010/06/23 06:00 CRDT- 2010/03/03 06:00 PHST- 2010/03/03 06:00 [entrez] PHST- 2010/03/03 06:00 [pubmed] PHST- 2010/06/23 06:00 [medline] AID - jrheum.090866 [pii] AID - 10.3899/jrheum.090866 [doi] PST - ppublish SO - J Rheumatol. 2010 Apr;37(4):800-8. doi: 10.3899/jrheum.090866. Epub 2010 Mar 1. PMID- 34610217 OWN - NLM STAT- MEDLINE DCOM- 20211221 LR - 20211221 IS - 1899-5276 (Print) IS - 1899-5276 (Linking) VI - 30 IP - 12 DP - 2021 Dec TI - Characteristics of idiopathic inflammatory myopathies with novel myositis-specific autoantibodies. PG - 1239-1248 LID - 10.17219/acem/141181 [doi] AB - BACKGROUND: In recent years, many novel myositis-specific autoantibodies (MSAs) have been identified. However, their links with the pathogenesis and clinical manifestations of inflammatory myopathies remain uncertain. OBJECTIVES: To characterize the population of adult dermatomyositis (DM) and polymyositis (PM) patients treated at our center for autoimmune diseases using clinical and laboratory measures. MATERIAL AND METHODS: According to the Bohan and Peter criteria, we retrospectively analyzed patients who fulfilled diagnostic criteria for DM or PM. Myositis-specific autoantibodies and myositis-associated autoantibodies (MAAs) were identified using immunoblot assays. RESULTS: Fifty-one PM (71% women) and 36 DM (67% women) Caucasian patients with a median age of 58 (range: 21-88) years who met the definite or probable diagnostic criteria for myositis were included in the study. Myositis-specific autoantibodies were identified in 63 (72%) patients, whereas MAAs were observed in 43 (49%) of them. Interstitial lung disease (ILD) was characteristic of PM patients (67%, χ2 with Yates's correction (χc2) = 13.8078, df = 1, p = 0.0002), being associated with anti-Jo-1 or anti-PL-12 antibodies (fraction comparison test (FCT) 6.4878, p < 0.0001, 6.8354, p = 0.0003, respectively). Interestingly, among patients with anti-MDA5 antibodies (n = 8, 9.2%), all but one had an amyopathic form, with more frequent ILD, skin changes and arthralgias than observed in other patients (FCT 4.7029, p = 0.0228 and p = 7.7986, p = 0.0357, p = 4.7029 and p = 0.0228, respectively). Anti-signal recognition particle (SRP) was strongly associated with the Raynaud's phenomenon (FCT 4.1144, p = 0.0289) and the highest muscle injury markers (Mann-Whitney U test, z = 2.5293, p = 0.0114). Malignancy was recorded in 14 (16%) patients and was equally common in those with PM and DM. The anti-TIF-1γ was the most frequently related to cancer χ2 = 14.7691, df = 1, p < 0.0001). The anti-Mi-2α, similarly prevalent in DM and PM, was typically accompanied by skin changes (FCT 7.7986, p = 0.0357) but not ILD (FCT 8.7339, p = 0.0026). CONCLUSIONS: Identification of MSAs might help to predict the clinical course of the autoimmune myopathy and malignancy risk. However, these antibodies were absent in about 30% of patients with typical PM or DM manifestations, which encourages further research in this area. FAU - Rams, Anna AU - Rams A AD - Department of Pulmonology and Allergology, University Hospital, Kraków, Poland. FAU - Kosałka-Węgiel, Joanna AU - Kosałka-Węgiel J AD - Department of Rheumatology and Immunology, University Hospital, Kraków, Poland. FAU - Kuszmiersz, Piotr AU - Kuszmiersz P AD - Department of Rheumatology and Immunology, University Hospital, Kraków, Poland. FAU - Matyja-Bednarczyk, Aleksandra AU - Matyja-Bednarczyk A AD - Faculty of Medicine, Jagiellonian University, Medical College, Kraków, Poland. FAU - Polański, Stanisław AU - Polański S AD - Faculty of Medicine, Jagiellonian University, Medical College, Kraków, Poland. FAU - Zaręba, Lech AU - Zaręba L AD - Faculty of Medicine, Jagiellonian University, Medical College, Kraków, Poland. FAU - Bazan-Socha, Stanisława AU - Bazan-Socha S AD - Faculty of Medicine, Jagiellonian University, Medical College, Kraków, Poland. LA - eng PT - Journal Article PL - Poland TA - Adv Clin Exp Med JT - Advances in clinical and experimental medicine : official organ Wroclaw Medical University JID - 101138582 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - *Autoantibodies MH - Humans MH - Middle Aged MH - *Myositis/diagnosis MH - Retrospective Studies MH - Young Adult OTO - NOTNLM OT - dermatomyositis OT - idiopathic inflammatory myopathies OT - myositis-specific antibodies OT - polymyositis EDAT- 2021/10/06 06:00 MHDA- 2021/12/22 06:00 CRDT- 2021/10/05 17:29 PHST- 2021/10/06 06:00 [pubmed] PHST- 2021/12/22 06:00 [medline] PHST- 2021/10/05 17:29 [entrez] AID - 10.17219/acem/141181 [doi] PST - ppublish SO - Adv Clin Exp Med. 2021 Dec;30(12):1239-1248. doi: 10.17219/acem/141181. PMID- 33331305 OWN - NLM STAT- MEDLINE DCOM- 20201218 LR - 20201219 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 52 IP - 6 DP - 2020 Dec 18 TI - [Comparison of clinical and immunological features between clinically amyopathic dermatomyositis and typical dermatomyositis]. PG - 1001-1008 AB - OBJECTIVE: To study the differences between clinically amyopathic dermatomyositis (CADM) and typical dermatomyositis (DM) on clinical and immunological features. METHODS: By collecting clinical data of 106 CADM patients and 158 DM patients from January 2010 to June 2019 in the department of Rheumatology and Immunology, Peking University People's Hospital, the clinical characteristics and immunological features in the two groups were compared, and the distribution characters and the clinical meanings of myositis autoantibodies were discussed in the two groups respectively. Myositis autoantibodies were measured by immunoblotting according to the manufacturers' instructions. RESULTS: In the aspects of clinical manifestations, CADM presented more with onset of interstial lung diseases (ILD) compared with DM (20.7% vs. 7.6%, P=0.002), and CADM-ILD was more likely to be acute ILD (58.3% vs. 26%, P < 0.001), and there were no differences between CADM and DM in cutaneous manifestations, accompanied with connective tissue disease (CTD) and malignancy. In CADM, the positive rate of rheumatoid factors and antinuclear antibodies was lower in DM. The most common myositis specific autoantibodies (MSAs) in CADM were anti-MDA5 (36%), anti-PL-7 (11.2%) and anti-TIF-1γ (10.1%). The most common MSAs in DM were anti-Jo-1 (19.2%), anti-TIF-1γ (11.5%) and anti-MDA5 (11.5%). Anti-MDA5 was correlated with acute ILD and skin ulceration both in CADM and DM; in CADM, skin ulceration was not associated with the titer of anti-MDA5; while in DM, skin ulceration was associated with high titer of anti-MDA5. In DM, anti-TIF-1γ was correlated with heliotrope eruption, V/shawl neck sign, perionychia erythma and malignancy, and higher rate of malignancy was seen in all titers of the anti-TIF-1γ positive patients. In CADM, anti-TIF1-γ showed no correlation with clinical manifestations. The most common myositis associated autoantibody was anti-Ro-52 both in CADM and DM. In CADM, anti-Ro-52 was associated with Raynaud's phenomenon and chronic ILD, while in DM, anti-Ro-52 was associated with mechanic's hands, noninfectious fever and accompanied CTD. CONCLUSION: Compared with DM, ILD is more likely to be acute in CADM. It is different between CADM and DM about the distribution of myositis autoantibodies and the clinical significance of the same myositis antibody, and the clinical significance of some myositis antibodies is related to titers. FAU - Gan, Y Z AU - Gan YZ AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Y H AU - Li YH AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Zhang, L H AU - Zhang LH AD - Department of Rheumatology, Hulunbeier People's Hospital, Hulunbeier 021008, Inner Mongolia, China. FAU - Ma, L AU - Ma L AD - Department of Rheumatology, Hebei Hospital of Traditional Chinese Medicine, Shijiazhuang 050200, China. FAU - He, W W AU - He WW AD - Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China. FAU - Jin, Y B AU - Jin YB AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - An, Y AU - An Y AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Z G AU - Li ZG AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Ye, H AU - Ye H AD - Department of Rheumatology & Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) RN - Amyopathic dermatomyositis SB - IM MH - Autoantibodies MH - *Dermatomyositis/complications MH - Humans MH - *Lung Diseases, Interstitial MH - *Neoplasms PMC - PMC7745270 OTO - NOTNLM OT - Clinically amyopathic dermatomyositis OT - Dermatomyositis OT - Myositis autoantibody EDAT- 2020/12/18 06:00 MHDA- 2020/12/19 06:00 PMCR- 2020/12/18 CRDT- 2020/12/17 08:42 PHST- 2020/12/17 08:42 [entrez] PHST- 2020/12/18 06:00 [pubmed] PHST- 2020/12/19 06:00 [medline] PHST- 2020/12/18 00:00 [pmc-release] AID - bjdxxbyxb-52-6-1001 [pii] AID - 10.19723/j.issn.1671-167X.2020.06.003 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1001-1008. doi: 10.19723/j.issn.1671-167X.2020.06.003. PMID- 28240590 OWN - NLM STAT- MEDLINE DCOM- 20170831 LR - 20220408 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 IP - 4 DP - 2017 Jul-Aug TI - A meta-analysis of avascular necrosis in systemic lupus erythematosus: prevalence and risk factors. PG - 700-710 AB - OBJECTIVES: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE). METHODS: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE. RESULTS: 2,041 citations identified 62 articles. Many results had high heterogeneity. The prevalence of symptomatic AVN was 9% (range 0.8%-33%) in SLE and 29% for asymptomatic AVN; femoral head was the most common location (8.0%). High-dose corticosteroids (CS) any CS use, maximum and cumulative dose, pulse therapy, and CS side-effects (hypertension, Cushings, but not diabetes mellitus or hyperlipidaemia) were associated with AVN, as was active SLE (cutaneous vasculitis, renal and neuropsychiatric manifestations, serositis, cytopenias) and Sjögren's, Raynaud's phenomenon, arthritis, cyclophosphamide (but not azathioprine mycophenolate mofetil, or methotrexate) and more damage (excluding musculoskeletal system). Antimalarial drugs were not protective. Rashes and oral ulcers were not associated with AVN. Mean daily dose of CS and duration of CS use had no impact on AVN occurence. Autoantibodies and other immunological markers did not predispose to AVN, except IgM anticardiolipin antibodies which doubled the risk. African Americans experienced more AVN (OR 1.8, p=0.04). CONCLUSIONS: AVN may occur in 1/3 of patients with SLE and 9% with symptoms. Features of active organ SLE (CNS, renal, cutaneous vasculitis, serositis, cytopenias) are associated with AVN as are CS, especially early in disease and at high doses. Those with early CS side-effects seem to have the highest risk of AVN. FAU - Nevskaya, Tatiana AU - Nevskaya T AD - Division of Rheumatology, Department of Medicine, Joseph's Health Care, London, Canada. FAU - Gamble, Maeve P AU - Gamble MP AD - Division of Rheumatology, Department of Medicine, Joseph's Health Care, London, Canada. FAU - Pope, Janet E AU - Pope JE AD - Division of Rheumatology, Department of Medicine, Joseph's Health Care, London, Canada. janet.pope@sjhc.london.on.ca. LA - eng PT - Journal Article PT - Meta-Analysis PT - Review DEP - 20170224 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/administration & dosage/adverse effects/therapeutic use MH - Cushing Syndrome/chemically induced/epidemiology MH - Femur Head Necrosis/epidemiology MH - Humans MH - Hypertension/chemically induced/epidemiology MH - Lupus Erythematosus, Systemic/drug therapy/*epidemiology MH - Lupus Nephritis/epidemiology MH - Lupus Vasculitis, Central Nervous System/epidemiology MH - Osteonecrosis/*epidemiology MH - Prevalence MH - Risk Factors MH - Serositis/epidemiology MH - Skin Diseases/epidemiology MH - Vasculitis/epidemiology EDAT- 2017/02/28 06:00 MHDA- 2017/09/01 06:00 CRDT- 2017/02/28 06:00 PHST- 2016/08/19 00:00 [received] PHST- 2017/01/23 00:00 [accepted] PHST- 2017/02/28 06:00 [pubmed] PHST- 2017/09/01 06:00 [medline] PHST- 2017/02/28 06:00 [entrez] AID - 11045 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Jul-Aug;35(4):700-710. Epub 2017 Feb 24. PMID- 26424665 OWN - NLM STAT- MEDLINE DCOM- 20170202 LR - 20220317 IS - 1559-0267 (Electronic) IS - 1080-0549 (Print) IS - 1080-0549 (Linking) VI - 52 IP - 1 DP - 2017 Feb TI - A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. PG - 1-19 LID - 10.1007/s12016-015-8510-y [doi] AB - Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP). Anti-Jo-1 is the first autoantibodies to ARS detected in 15-25 % of patients. In addition to anti-Jo-1, antibodies to seven other aminoacyl tRNA synthetases (ARS) have been reported with prevalence, usually 1-5 % or lower. Patients with any anti-ARS antibodies are associated with anti-synthetase syndrome characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and others. Several recent studies suggested heterogeneity in clinical features among different anti-ARS antibody-positive patients and anti-ARS may also be found in idiopathic ILD without myositis. Anti-Mi-2 is a classic marker for DM and associated with good response to steroid treatment and good prognosis. Anti-SRP is specific for PM and associated with treatment-resistant myopathy histologically characterized as necrotizing myopathy. In addition to classic MSAs, several new autoantibodies with strong clinical significance have been described in DM. Antibodies to transcription intermediary factor 1γ/α (TIF1γ/α, p155/140) are frequently found in DM associated with malignancy while anti-melanoma differentiation-associated gene 5 (MDA5; CADM140) are associated with clinically amyopathic DM (CADM) complicated by rapidly progressive ILD. Also, anti-MJ/nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modifier-1 (SUMO-1) activating enzyme (SAE) are recognized as new DM-specific autoantibodies. Addition of these new antibodies to clinical practice in the future will help in making earlier and more accurate diagnoses and better management for patients. FAU - Satoh, Minoru AU - Satoh M AD - Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Japan, 1-1 Isei-ga-oka, Yahata-nishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan. satohm@health.uoeh-u.ac.jp. FAU - Tanaka, Shin AU - Tanaka S AD - Department of Human Information and Sciences, School of Health Sciences, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Ceribelli, Angela AU - Ceribelli A AD - Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Via A. Manzoni 56, 20089, Rozzano (Milan), Italy. AD - BIOMETRA Department, University of Milan, Via Vanvitelli 32, 20129, Milan, Italy. FAU - Calise, S John AU - Calise SJ AD - Department of Oral Biology, University of Florida, Gainesville, FL, USA. FAU - Chan, Edward K L AU - Chan EK AD - Department of Oral Biology, University of Florida, Gainesville, FL, USA. LA - eng GR - R01 DE019783/DE/NIDCR NIH HHS/United States GR - T90 DE021990/DE/NIDCR NIH HHS/United States PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Biomarkers) SB - IM MH - Autoantibodies/*immunology MH - Autoantigens/immunology MH - Biomarkers/analysis MH - Humans MH - Myositis/*immunology PMC - PMC5828023 MID - NIHMS943588 OTO - NOTNLM OT - Anti-nuclear antibodies OT - Autoantibodies OT - Dermatomyositis OT - Inflammatory myopathy OT - Polymyositis EDAT- 2015/10/02 06:00 MHDA- 2017/02/06 06:00 PMCR- 2018/02/27 CRDT- 2015/10/02 06:00 PHST- 2015/10/02 06:00 [pubmed] PHST- 2017/02/06 06:00 [medline] PHST- 2015/10/02 06:00 [entrez] PHST- 2018/02/27 00:00 [pmc-release] AID - 10.1007/s12016-015-8510-y [pii] AID - 10.1007/s12016-015-8510-y [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2017 Feb;52(1):1-19. doi: 10.1007/s12016-015-8510-y. PMID- 25483258 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20161230 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 42 IP - 2 DP - 2015 Feb TI - Demographic and clinical features of systemic sclerosis patients with anti-RNA polymerase III antibodies. PG - 189-92 LID - 10.1111/1346-8138.12722 [doi] AB - Anti-RNA polymerase III antibody (RNAP) is primarily detected in diffuse cutaneous type systemic sclerosis (dcSSc) patients and strongly associated with renal crisis. Additionally, there has been increasing evidence that cancer in SSc patients is associated with RNAP. The aim of this study was to examine the demographic and clinical features of SSc patients with RNAP. Among 246 SSc patients, 5.7% were positive for RNAP, 20.7% were positive for anti-topoisomerase I antibody (Topo I) alone and 39.4% were positive for anticentromere antibody (ACA) alone. The modified Rodnan total skin score (mRTSS) in SSc patients with RNAP (19.1 ± 2.6) was significantly higher than those in SSc patients with Topo I (11.5 ± 1.1) and patients with ACA (4.4 ± 0.4). Furthermore, among SSc patients with RNAP, the levels of RNAP were positively correlated with mRTSS. Renal crisis is also significantly more prevalent in SSc patients with RNAP than patients without RNAP. Male sex, dcSSc subtype, digital vasculopathy, including digital ulcers and acro-osteolysis, interstitial lung disease and rheumatoid arthritis complications were prevalent in SSc patients with RNAP and patients with Topo-I. Primary biliary cirrhosis and Sjögren's syndrome were more in SSc patients with RNAP and patients with ACA compared with patients with Topo 1. No significant difference in the frequency of complications, including Raynaud's phenomenon, pulmonary artery hypertension and malignancy was observed between the three groups. Thus, measurement of RNAP in SSc patients is useful for the diagnosis and risk stratification of severe manifestation, such as renal crisis and severe skin sclerosis. CI - © 2014 Japanese Dermatological Association. FAU - Motegi, Sei-Ichiro AU - Motegi S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. FAU - Toki, Sayaka AU - Toki S FAU - Yamada, Kazuya AU - Yamada K FAU - Uchiyama, Akihiko AU - Uchiyama A FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article DEP - 20141206 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) RN - EC 2.7.7.6 (RNA Polymerase III) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Acute Kidney Injury/blood/complications MH - Aged MH - Antibodies, Antinuclear/*blood MH - Arthritis, Rheumatoid/blood/complications MH - DNA Topoisomerases, Type I/*immunology MH - Female MH - Humans MH - Lung Diseases, Interstitial/blood/complications MH - Male MH - Middle Aged MH - Osteolysis/blood/complications MH - RNA Polymerase III/*immunology MH - Scleroderma, Systemic/complications/*immunology MH - Severity of Illness Index MH - Sex Factors MH - Sjogren's Syndrome/blood/complications MH - Vascular Diseases/blood/complications OTO - NOTNLM OT - anti-RNA polymerase III antibody OT - autoantibody OT - malignancy OT - renal crisis OT - systemic sclerosis EDAT- 2014/12/09 06:00 MHDA- 2016/12/15 06:00 CRDT- 2014/12/09 06:00 PHST- 2014/09/11 00:00 [received] PHST- 2014/10/16 00:00 [accepted] PHST- 2014/12/09 06:00 [entrez] PHST- 2014/12/09 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - 10.1111/1346-8138.12722 [doi] PST - ppublish SO - J Dermatol. 2015 Feb;42(2):189-92. doi: 10.1111/1346-8138.12722. Epub 2014 Dec 6. PMID- 25449678 OWN - NLM STAT- MEDLINE DCOM- 20150518 LR - 20250711 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 14 IP - 2 DP - 2015 Feb TI - A PRISMA-driven systematic review for predictive risk factors of digital ulcers in systemic sclerosis patients. PG - 140-52 LID - S1568-9972(14)00221-3 [pii] LID - 10.1016/j.autrev.2014.10.009 [doi] AB - Vasculopathy has a major role in the pathogenesis and tissue injury in systemic sclerosis (SSc). Raynaud's phenomenon (RP) is frequently the first clinical manifestation of SSc preceding by years other clinical manifestations. RP in SSc patients is frequent, often very severe and long lasting. The repeated bouts of RP lead to prolonged digital ischemia that may progress to digital ulceration or in extreme to critical digital ischemia with gangrene. Digital ulcers (DU) are a true burden for all patients. They are very painful, with a long and slow healing course, have high risk of infection and are extremely disabling. In adults, up to 40-50% of patients will experience at least one DU in the course of the disease and of these 31-71% will have recurrent ulcers. In order to try to identify predictive risk factors for DU in SSc patients, an extensive literature review was conducted, according to the guidelines proposed at the PRISMA statement. MEDLINE database (PubMed) and Thomson Reuters Web of Knowledge platform were searched for articles published in peer-reviewed journals since 1990 with the last search run on June 2014 and published in English language. The keyword search terms included: digital ulcer/s, systemic sclerosis, scleroderma, digital scars, ischemic complications, autoantibodies, biomarkers, endothelium dysfunction, endothelin-1, vascular endothelial growth factor (VEGF), endostatin, ADMA, endoglin, angiostatin, and capillaroscopy. The following criteria were included: (1) cohorts of SSc patients including patients with DU, (2) endothelium dysfunction and angiogenesis biomarkers compared with a healthy control group, (3) autoantibodies, capillary morphology and distribution, endothelium dysfunction and angiogenesis biomarkers compared between patients with and without digital ulcers, (4) detailed description of the statistical methods used to conclude for predictive factors, and (5) English language. Our search provided a total of 376 citations. Of these, 297 studies were discarded for not meeting the criteria proposed. CI - Copyright © 2014 Elsevier B.V. All rights reserved. FAU - Silva, I AU - Silva I AD - Angiology and Vascular Surgery and Clinical Imunology Unit, Hospital Santo António, Centro Hospitalar do Porto, Portugal. Electronic address: heitor.ivone@gmail.com. FAU - Almeida, J AU - Almeida J AD - Internal Medicine Service, Centro Hospitalar de Vila Nova de Gaia, Portugal. FAU - Vasconcelos, C AU - Vasconcelos C AD - Clinical Imunology Unit, Hospital Santo António, Centro Hospitalar do Porto, Portugal; UMIB, ICBAS, Universidade do Porto, Portugal. LA - eng PT - Journal Article PT - Systematic Review DEP - 20141014 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - 0 (Vascular Endothelial Growth Factor A) SB - IM MH - Autoantibodies/immunology MH - Humans MH - Practice Guidelines as Topic MH - Risk Factors MH - Scleroderma, Systemic/*complications/immunology MH - Ulcer/*etiology MH - Vascular Endothelial Growth Factor A/immunology OTO - NOTNLM OT - Autoantibodies OT - Capillaroscopy OT - Digital ulcer/s OT - Endothelin-1 OT - Systemic sclerosis OT - Vascular endothelial growth factor (VEGF) EDAT- 2014/12/03 06:00 MHDA- 2015/05/20 06:00 CRDT- 2014/12/03 06:00 PHST- 2014/09/16 00:00 [received] PHST- 2014/10/04 00:00 [accepted] PHST- 2014/12/03 06:00 [entrez] PHST- 2014/12/03 06:00 [pubmed] PHST- 2015/05/20 06:00 [medline] AID - S1568-9972(14)00221-3 [pii] AID - 10.1016/j.autrev.2014.10.009 [doi] PST - ppublish SO - Autoimmun Rev. 2015 Feb;14(2):140-52. doi: 10.1016/j.autrev.2014.10.009. Epub 2014 Oct 14. PMID- 20144926 OWN - NLM STAT- MEDLINE DCOM- 20110311 LR - 20250529 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 49 IP - 5 DP - 2010 May TI - Tendon friction rubs in early diffuse systemic sclerosis: prevalence, characteristics and longitudinal changes in a randomized controlled trial. PG - 955-9 LID - 10.1093/rheumatology/kep464 [doi] AB - OBJECTIVES: To characterize the baseline tendon friction rubs (TFRs) in early dcSSc and to evaluate the association of change in TFR over 6 and 12 months with changes in modified Rodnan skin score (MRSS) and HAQ-Disability Index (HAQ-DI) over 12 and 24 months, respectively. METHODS: We analysed data from the d-Pen study, a 2-year study in early dcSSc (< or =18 months from first non-Raynaud's symptom). TFR was scored as present/absent at seven anatomical sites at baseline and every 6 months thereafter. Multivariable linear regression models assessed associations between TFR and change in MRSS, and change in the HAQ-DI, over 12 and 24 months, respectively. Covariates included baseline TFR, change in the TFR over 6 and 12 months, age, sex, duration of SSc, MRSS, and tender joint count and swollen joint count (SJC). RESULTS: Forty-nine (37%) of 134 patients had TFR at baseline, 50% had resolution of their TFR, whereas 21% developed new TFRs. Patients with baseline TFRs were likely to be Caucasian (86 vs 58%) and had a higher HAQ-DI score (P = 0.008). In regression analyses, change in TFR (P = 0.04) and baseline MRSS (P = 0.03) predicted change in MRSS over a 12-month period (Model R(2 )= 0.14). For the HAQ-DI model, independent predictors were change in TFR at 6 months (P = 0.008) and baseline SJC (P = 0.04, Model R(2 )= 0.19). Results were similar for 24-month models. CONCLUSIONS: We document the presence of TFR very early in the course of dcSSc. Changes in TFR over 6 and 12 months predict changes in MRSS and HAQ-DI over 12 and 24 months, respectively. FAU - Khanna, Puja P AU - Khanna PP AD - Department of Medicine, Division of Rheumatology, David Geffen School of Medicine, UCLA, Los Angeles, CA 90095, USA. dkhanna@mednet.ucla.edu FAU - Furst, Daniel E AU - Furst DE FAU - Clements, Philip J AU - Clements PJ FAU - Maranian, Paul AU - Maranian P FAU - Indulkar, Lilavati AU - Indulkar L FAU - Khanna, Dinesh AU - Khanna D CN - D-Penicillamine Investigators LA - eng GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - T32 AR053463/AR/NIAMS NIH HHS/United States GR - 1 T32 AR053463/AR/NIAMS NIH HHS/United States GR - K23 AR053858-03/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20100209 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Disability Evaluation MH - Disease Progression MH - Female MH - Friction/*physiology MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prevalence MH - Regression Analysis MH - Scleroderma, Diffuse/physiopathology MH - Scleroderma, Systemic/*physiopathology MH - Tendons/*physiology MH - Time Factors PMC - PMC2909791 EDAT- 2010/02/11 06:00 MHDA- 2011/03/12 06:00 PMCR- 2011/05/01 CRDT- 2010/02/11 06:00 PHST- 2010/02/11 06:00 [entrez] PHST- 2010/02/11 06:00 [pubmed] PHST- 2011/03/12 06:00 [medline] PHST- 2011/05/01 00:00 [pmc-release] AID - kep464 [pii] AID - 10.1093/rheumatology/kep464 [doi] PST - ppublish SO - Rheumatology (Oxford). 2010 May;49(5):955-9. doi: 10.1093/rheumatology/kep464. Epub 2010 Feb 9. PMID- 40593370 OWN - NLM STAT- MEDLINE DCOM- 20250724 LR - 20250801 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 44 IP - 8 DP - 2025 Aug TI - Validation and cross-cultural adaptation of the Thai version of the Systemic Sclerosis Health Assessment Questionnaire. PG - 3235-3243 LID - 10.1007/s10067-025-07553-2 [doi] AB - BACKGROUND: The Scleroderma Health Assessment Questionnaire (S-HAQ), including the Disability Index (HAQ-DI) and six-symptom visual analog scales (VAS), assesses functional ability in systemic sclerosis (SSc). The Thai HAQ-DI has been validated, but not the S-HAQ VAS. OBJECTIVE: We aim to translate and culturally adapt the VAS for symptoms in S-HAQ and assess its validity and reliability. METHOD: The six VAS items, which assess pain, gastrointestinal symptoms, respiratory symptoms, Raynaud's phenomenon (RP), digital ulcers (DU), and overall disease activity interfering with daily activities, were translated into Thai and validated. Thirty SSc patients completed the translated questionnaire twice, with a one-week interval. Intraclass correlation (ICC) was used for test-retest reliability, and Spearman correlation was used to test the reliability of the associated clinical parameters and the S-HAQ VAS. RESULT: For the validity testing, the VAS for pain, respiratory symptoms, RP, DU, and overall disease activity showed significant correlations with the modified Rodnan skin score (mRSS) (Rho = 0.508, 0.550, 0.474, 0.492, and 0.557, respectively) and with the EuroQol visual analog scale (EQ VAS) (Rho =  - 0.652, - 0.697, - 0.568, - 0.581, and - 0.668, respectively). Additionally, the World Health Organization functional class demonstrated significant correlations with both the VAS overall disease activity and the HAQ-DI (Rho = 0.473 and 0.460, respectively). The HAQ-DI showed significant correlations with all the S-HAQ VAS questions. The reliability testing indicated a strong ICC between two testing sessions for each VAS item in the S-HAQ, with coefficient values ranging from 0.777 to 0.940. CONCLUSION: This study substantiates the utility of the Thai S-HAQ VAS as a valid measure for assessing function and disability in patients with SSc. The translated Thai version of the S-HAQ VAS exhibits high reliability, demonstrated by strong ICC between two testing sessions. Key Points • The Thai version of the Scleroderma Health Assessment Questionnaire (S-HAQ) visual analog scale (VAS) was successfully translated and culturally adapted. It provides a reliable and valid tool for assessing systemic sclerosis (SSc) symptoms and disability in native Thai patients. • Strong test-retest reliability was demonstrated for all six VAS items, with intraclass correlation (ICC) values ranging from 0.777 to 0.940 and the validity testing showed significant correlations between S-HAQ VAS scores and clinical parameters, including mRSS, EQ-VAS, and WHO functional class. • The availability of this validated tool enables Thai researchers to participate in international SSc clinical trials and comparative studies using standardized outcome measures, enhancing global research collaboration. CI - © 2025. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Ruangnopparut, Ratthanin AU - Ruangnopparut R AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Osiri, Manathip AU - Osiri M AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, 10330, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Pongkulkiat, Patnarin AU - Pongkulkiat P AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Onchan, Tippawan AU - Onchan T AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AUID- ORCID: 0000-0002-1964-4389 AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand. fching@kku.ac.th. LA - eng PT - Journal Article PT - Validation Study DEP - 20250701 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Humans MH - *Scleroderma, Systemic/physiopathology/diagnosis MH - Female MH - Male MH - Thailand MH - Middle Aged MH - Reproducibility of Results MH - Surveys and Questionnaires MH - Adult MH - Disability Evaluation MH - Severity of Illness Index MH - Aged MH - Pain Measurement MH - Cross-Cultural Comparison MH - Visual Analog Scale MH - Translations MH - Activities of Daily Living MH - Quality of Life MH - Southeast Asian People OTO - NOTNLM OT - Cultural adaption OT - Functional assessment OT - Health assessment OT - Outcome systemic sclerosis OT - Questionnaire OT - Scleroderma-related disorder COIS- Declarations. Ethical approval: The Human Research Ethics Committee of Khon Kaen University approved the study protocol and the written informed consent per the Helsinki Declaration and the Good Clinical Practice Guidelines (HE661578). All methods were performed according to the Declaration of Helsinki, relevant guidelines, and regulations. Written informed consent was obtained from all eligible patients before enrolment in the study. Consent for publication: The authors consent to publication and grant the Publisher’s exclusive license for full copyright. Disclosures: None. EDAT- 2025/07/02 06:28 MHDA- 2025/07/24 12:28 CRDT- 2025/07/02 00:05 PHST- 2025/05/23 00:00 [received] PHST- 2025/06/20 00:00 [accepted] PHST- 2025/06/15 00:00 [revised] PHST- 2025/07/24 12:28 [medline] PHST- 2025/07/02 06:28 [pubmed] PHST- 2025/07/02 00:05 [entrez] AID - 10.1007/s10067-025-07553-2 [pii] AID - 10.1007/s10067-025-07553-2 [doi] PST - ppublish SO - Clin Rheumatol. 2025 Aug;44(8):3235-3243. doi: 10.1007/s10067-025-07553-2. Epub 2025 Jul 1. PMID- 38302817 OWN - NLM STAT- MEDLINE DCOM- 20240220 LR - 20240220 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 43 IP - 3 DP - 2024 Mar TI - Digital gangrene in systemic sclerosis patients: not only due to the microvascular disease. PG - 1083-1092 LID - 10.1007/s10067-024-06886-8 [doi] AB - OBJECTIVE: This study aims to investigate the characteristics, risk factors, and outcomes of digital gangrenes in SSc patients, and to identify whether vasculitis is one of the causes for digital gangrene. METHODS: A retrospective case-control study was performed from February 2003 to April 2021. Forty-three SSc patients with digital gangrene admitted to Peking Union Medical College Hospital were included. One-hundred forty-six age- and sex-matched SSc patients without gangrene were selected as controls during the same period. Univariate and multivariate logistic regression analysis was used to determine risk factors. RESULTS: Among 43 SSc patients with gangrene, 93.0% had Raynaud's phenomenon (RP) and 32.6% had current or previous digital ulcers (DU). SSc patients with digital gangrene had more ESR elevation (54.8% vs. 34.9%, p = 0.020) and higher level of high-sensitive C reactive protein (median 7.2 mg/L vs. 1.8 mg/L, p = 0.045) compared with controls. In the multivariable logistic regression analysis, smoking history (OR 4.119, p = 0.037), anti-centromere antibody positivity (OR 3.542, p = 0.016), anti-neutrophil cytoplasmic antibody positivity (OR 22.605, p = 0.037), and anti-phospholipid antibody positivity (OR 16.563, p = 0.001), as well as elevated ESR (OR 2.524, p = 0.038) were identified as independent risk factors for gangrenes. Most (79.1%) cases were treated with combination of immunosuppressive and vasodilating therapy, and four cases also got remised after treatment of only glucocorticoid and immunosuppressive agent. CONCLUSION: Smoking history; positive-ACA, ANCA, and anti-phospholipid antibodies; and increased ESR were independent risk factors for digital gangrenes in SSc. Vasculitis and macrovascular disease may contribute to the progression of digital gangrenes. Key Points •18.6% of SSc patients with digital gangrene had macrovascular stenosis. •Smoking, positive-ACA, ANCA, aPL, and increased ESR were indicators for digital gangrenes in SSc. •Vasculitis and macrovascular disease may involve in the pathogenesis. CI - © 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Hui, Min AU - Hui M AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Zhou, Jiaxin AU - Zhou J AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Li, Mengtao AU - Li M AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Wang, Qian AU - Wang Q AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Zhao, Jiuliang AU - Zhao J AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Hou, Yong AU - Hou Y AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. FAU - Xu, Dong AU - Xu D AUID- ORCID: 0000-0002-6413-3043 AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. xudong74@hotmail.com. FAU - Zeng, Xiaofeng AU - Zeng X AD - Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Sciences & Peking Union Medical College, Peking Union Medical College Hospital, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. LA - eng GR - 2021-I2M-1-005/CAMS Innovation Fund for Medical Sciences (CIFMS)/ PT - Journal Article DEP - 20240202 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) SB - IM CIN - Clin Rheumatol. 2024 Apr;43(4):1413. doi: 10.1007/s10067-024-06908-5. PMID: 38376770 MH - Humans MH - Autoantibodies MH - Gangrene/complications MH - Antibodies, Antineutrophil Cytoplasmic MH - Retrospective Studies MH - Case-Control Studies MH - *Scleroderma, Systemic/complications MH - *Vasculitis OTO - NOTNLM OT - Autoantibodies OT - Case–control study OT - Risk factors OT - Systemic sclerosis OT - Vasculitis EDAT- 2024/02/02 00:42 MHDA- 2024/02/20 11:50 CRDT- 2024/02/01 23:56 PHST- 2023/07/26 00:00 [received] PHST- 2024/01/22 00:00 [accepted] PHST- 2024/01/02 00:00 [revised] PHST- 2024/02/20 11:50 [medline] PHST- 2024/02/02 00:42 [pubmed] PHST- 2024/02/01 23:56 [entrez] AID - 10.1007/s10067-024-06886-8 [pii] AID - 10.1007/s10067-024-06886-8 [doi] PST - ppublish SO - Clin Rheumatol. 2024 Mar;43(3):1083-1092. doi: 10.1007/s10067-024-06886-8. Epub 2024 Feb 2. PMID- 37071834 OWN - NLM STAT- MEDLINE DCOM- 20230630 LR - 20231030 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 41 IP - 19 DP - 2023 Jul 1 TI - Cardiovascular Disease in Testicular Cancer Survivors: Identification of Risk Factors and Impact on Quality of Life. PG - 3512-3522 LID - 10.1200/JCO.22.01016 [doi] AB - PURPOSE: Testicular cancer (TC) treatment is clearly associated with cardiovascular morbidity and mortality. To enable development of preventive strategies for cardiovascular disease (CVD), we assessed cardiometabolic risk factors and quality of life (QoL) in TC survivors. METHODS: Incidence of coronary artery disease, myocardial infarction, and heart failure after TC treatment was assessed in a multicenter cohort comprising 4,748 patients treated at the age of 12-50 years between 1976 and 2007. Patients who had developed CVD and a random sample from the cohort (subcohort) received a questionnaire on cardiometabolic risk factors and QoL. A subgroup of responders in the subcohort additionally underwent clinical evaluation of cardiovascular risk factors. RESULTS: After a median follow-up of 16 years, 272 patients had developed CVD. Compared with orchidectomy only, cisplatin combination chemotherapy was associated with an increased CVD risk (hazard ratio [HR], 1.9; 95% CI, 1.1 to 3.1). Patients who were obese or a smoker at diagnosis (HR, 4.6; 95% CI, 2.0 to 10.0 and HR, 1.7; 95% CI, 1.1 to 2.4, respectively), developed Raynaud's phenomenon (HR, 1.9; 95% CI, 1.1 to 3.6) or dyslipidemia (HR, 2.8; 95% CI, 1.6 to 4.7) or had a positive family history for CVD (HR, 2.9; 95% CI, 1.7 to 4.9) had higher CVD risk. More TC survivors with CVD reported inferior QoL on physical domains than survivors who did not develop CVD. Of 304 TC survivors who underwent clinical evaluation for cardiovascular risk factors (median age at assessment: 51 years), 86% had dyslipidemia, 50% had hypertension, and 35% had metabolic syndrome, irrespective of treatment. CONCLUSION: Cardiovascular events in TC survivors impair QoL. Many TC survivors have undetected cardiovascular risk factors. We advocate early lifestyle adjustments and lifelong follow-up with low-threshold treatment of cardiovascular risk factors, especially in obese and smoking patients treated with platinum-based chemotherapy. FAU - Lubberts, Sjoukje AU - Lubberts S AD - Department of Medical Oncology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. FAU - Groot, Harmke J AU - Groot HJ AUID- ORCID: 0000-0002-5793-3106 AD - Department of Epidemiology, Netherlands Cancer Institute, Amsterdam, the Netherlands. FAU - de Wit, Ronald AU - de Wit R AD - Department of Medical Oncology, Erasmus Medical Center, Rotterdam, the Netherlands. FAU - Mulder, Sasja AU - Mulder S AUID- ORCID: 0000-0002-8068-4240 AD - Department of Medical Oncology, Radboud University Medical Center, Nijmegen, the Netherlands. FAU - Witjes, Johannes A AU - Witjes JA AD - Department of Urology, Radboud University Medical Center, Nijmegen, the Netherlands. FAU - Kerst, J Martijn AU - Kerst JM AD - Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands. FAU - Groenewegen, Gerard AU - Groenewegen G AUID- ORCID: 0000-0002-0752-1744 AD - Department of Medical Oncology, University Medical Center Utrecht, Utrecht, the Netherlands. FAU - Lefrandt, Joop D AU - Lefrandt JD AD - Department of Vascular Medicine, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. FAU - van Leeuwen, Flora E AU - van Leeuwen FE AUID- ORCID: 0000-0002-5871-1484 AD - Department of Epidemiology, Netherlands Cancer Institute, Amsterdam, the Netherlands. FAU - Nuver, Janine AU - Nuver J AD - Department of Medical Oncology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. FAU - Schaapveld, Michael AU - Schaapveld M AUID- ORCID: 0000-0003-4390-7182 AD - Department of Epidemiology, Netherlands Cancer Institute, Amsterdam, the Netherlands. FAU - Gietema, Jourik A AU - Gietema JA AUID- ORCID: 0000-0003-0629-7354 AD - Department of Medical Oncology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. LA - eng SI - ClinicalTrials.gov/NCT02276430 PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20230418 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - Testicular Germ Cell Tumor SB - IM CIN - J Clin Oncol. 2023 Nov 1;41(31):4938. doi: 10.1200/JCO.23.01122. PMID: 37579254 MH - Adult MH - Quality of Life MH - Neoplasms, Germ Cell and Embryonal MH - *Cardiovascular Diseases/epidemiology/etiology MH - *Testicular Neoplasms/drug therapy MH - Adolescent MH - Child MH - Risk Factors MH - Humans MH - Survivors MH - Middle Aged MH - Male MH - Young Adult MH - *Dyslipidemias MH - Obesity/complications PMC - PMC10306438 COIS- The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated unless otherwise noted. Relationships are self-held unless noted. I = Immediate Family Member, Inst = My Institution. Relationships may not relate to the subject matter of this manuscript. For more information about ASCO's conflict of interest policy, please refer to www.asco.org/rwc or ascopubs.org/jco/authors/author-center. Open Payments is a public database containing information reported by companies about payments made to US-licensed physicians (Open Payments). EDAT- 2023/04/18 18:41 MHDA- 2023/06/30 06:42 PMCR- 2023/04/18 CRDT- 2023/04/18 16:02 PHST- 2023/06/30 06:42 [medline] PHST- 2023/04/18 18:41 [pubmed] PHST- 2023/04/18 16:02 [entrez] PHST- 2023/04/18 00:00 [pmc-release] AID - JCO.22.01016 [pii] AID - 10.1200/JCO.22.01016 [doi] PST - ppublish SO - J Clin Oncol. 2023 Jul 1;41(19):3512-3522. doi: 10.1200/JCO.22.01016. Epub 2023 Apr 18. PMID- 35454989 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230308 IS - 2075-1729 (Print) IS - 2075-1729 (Electronic) IS - 2075-1729 (Linking) VI - 12 IP - 4 DP - 2022 Mar 29 TI - Capillaroscopy and Immunological Profile in Systemic Sclerosis. LID - 10.3390/life12040498 [doi] LID - 498 AB - Introduction: Data on the associations between capillaroscopic changes and diagnostic systemic-sclerosis (SSc)-related antibodies are scarce. Presence of such correlation would improve current knowledge about the disease’s pathogenesis by revealing the mechanisms of microangiopathy. The microvascular pathology of SSc is a hallmark of the disease, and immunological abnormalities probably contribute to its development. Patients and methods: 19 patients with definite diagnosis of SSc were included in the current pilot study; 16 had limited and 3 had diffuse cutaneous involvement; their mean age was 51.56 ± 15.07 years. All patients exhibited symptoms of Raynaud’s phenomenon of the fingers. A “scleroderma” type capillaroscopic pattern was classified according to the staging suggested by Cutolo et al. (2000): “early”, “active” or ”late” phase. In the presence of different degrees of capillaroscopic changes in different fingers, the most-advanced microvascular pathology was chosen for classification. In cases without capillaroscopic features of microangiopathy, the findings were categorized as normal or nonspecific (dilated, tortuous capillaries, and/or hemorrhages). Indirect immunofluorescence on HEp-2 cells was performed as the gold-standard screening method for the detection of antinuclear autoantibodies (ANA), and determination of the immunofluorescent staining pattern (anti-cell pattern) was in accordance with the International Consensus on ANA Patterns. Scleroderma-associated autoantibodies in the patients’ serum were assessed using line immunoblot assay for detection of autoantibodies to 13 scleroderma-associated autoantigens: Scl-70, CENP A, CENP B, RP11/RNAP-III, RP155/RNAP-III, fibrillarin, NOR-90, Th/To, PM-Scl100, PM-Scl75, Ku, PDGFR, and Ro-52. Results: In 73.7% (n = 14) of the examined patients, “scleroderma” type capillaroscopic changes were found, and in 26.3% (n = 5), capillaroscopic features of microangiopathy were absent (nonspecific changes, n = 3; normal findings, n = 2). In SSc patients with positive anti-Scl-70 (n = 7) antibodies, significantly lower mean capillary density was observed along with a higher frequency of “active” and “late” phase capillaroscopic changes as compared to the anti-Scl-70-negative patients (p < 0.05). Anti-RNAP III−155 positive patients (n = 4) had significantly higher mean capillary density than anti-RNAP III−155 negative patients (n = 15). In three of the anti-RNAP III−155-positive cases, capillaroscopic features of microangiopathy were not detected, and in one case there was an “early” phase “scleroderma” pattern. Conclusion: In the current pilot study, the association between more advanced capillaroscopic changes and the presence of anti-Scl-70 autoantibodies was confirmed. As a novel observation, positive anti-RNAP III−155 antibodies were found in SSc patients with or without early microangiopathy. The question of associations between microvascular changes in SSc and other SSc-related autoantibodies requires further research. FAU - Lambova, Sevdalina Nikolova AU - Lambova SN AD - Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University of Plovdiv, 4002 Plovdiv, Bulgaria. AD - Department of Rheumatology, MHAT "Sveti Mina", 4000 Plovdiv, Bulgaria. FAU - Kurteva, Ekaterina Krasimirova AU - Kurteva EK AD - Laboratory of Clinical Immunology, University Hospital "St. Ivan Rilski", 1431 Sofia, Bulgaria. AD - Department of Clinical Immunology, Faculty of Medicine, Medical University of Sofia, 1431 Sofia, Bulgaria. FAU - Dzhambazova, Sanie Syuleymanova AU - Dzhambazova SS AUID- ORCID: 0000-0002-6567-3279 AD - Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University of Plovdiv, 4002 Plovdiv, Bulgaria. AD - Clinic in Rheumatology, UMHAT "Sveti Georgi", 4000 Plovdiv, Bulgaria. FAU - Vasilev, Georgi Hristov AU - Vasilev GH AD - Laboratory of Clinical Immunology, University Hospital "St. Ivan Rilski", 1431 Sofia, Bulgaria. FAU - Kyurkchiev, Dobroslav Stanimirov AU - Kyurkchiev DS AD - Laboratory of Clinical Immunology, University Hospital "St. Ivan Rilski", 1431 Sofia, Bulgaria. AD - Department of Clinical Immunology, Faculty of Medicine, Medical University of Sofia, 1431 Sofia, Bulgaria. FAU - Geneva-Popova, Mariela Gencheva AU - Geneva-Popova MG AUID- ORCID: 0000-0002-7655-3846 AD - Department of Propaedeutics of Internal Diseases, Faculty of Medicine, Medical University of Plovdiv, 4002 Plovdiv, Bulgaria. AD - Clinic in Rheumatology, UMHAT "Sveti Georgi", 4000 Plovdiv, Bulgaria. LA - eng GR - Project of Medical University - Plovdiv №SD-04/2020/Medical University Plovdiv/ PT - Journal Article DEP - 20220329 PL - Switzerland TA - Life (Basel) JT - Life (Basel, Switzerland) JID - 101580444 PMC - PMC9024594 OTO - NOTNLM OT - autoantibodies OT - capillaroscopy OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2022/04/24 06:00 MHDA- 2022/04/24 06:01 PMCR- 2022/03/29 CRDT- 2022/04/23 01:05 PHST- 2022/02/14 00:00 [received] PHST- 2022/03/15 00:00 [revised] PHST- 2022/03/26 00:00 [accepted] PHST- 2022/04/23 01:05 [entrez] PHST- 2022/04/24 06:00 [pubmed] PHST- 2022/04/24 06:01 [medline] PHST- 2022/03/29 00:00 [pmc-release] AID - life12040498 [pii] AID - life-12-00498 [pii] AID - 10.3390/life12040498 [doi] PST - epublish SO - Life (Basel). 2022 Mar 29;12(4):498. doi: 10.3390/life12040498. PMID- 35340187 OWN - NLM STAT- MEDLINE DCOM- 20220331 LR - 20220401 IS - 0578-1426 (Print) IS - 0578-1426 (Linking) VI - 61 IP - 4 DP - 2022 Apr 1 TI - [Sex disparities in clinical characteristics of Chinese patients with systemic sclerosis]. PG - 403-408 LID - 10.3760/cma.j.cn112138-20210825-00586 [doi] AB - Objective: To evaluate the differences in clinical characteristics between different genders of Chinese patients with systemic sclerosis(SSc). Methods: The data of SSc patients registered in Chinese Rheumatism Data Center between August 2008 and June 2020 were retrospectively analyzed. Results: A total of 1 844 patients with SSc were enrolled in the study. The ratio of males to females was 289 to 1 555. The onset age was (48.6±13.7) years in males and (45.5±13.1) years in females(P<0.001). Male patients represented shorter disease duration [2.0(0.0, 4.0)years vs.3.0(1.0, 7.0) years, P<0.001],higher proportion of diffuse cutaneous SSc (dcSSc) [63.0% (182/289)vs.44.2%(688/1 555), P<0.001]. Although more man patients experienced smoking [47.4%(137/289) vs. 1.7%(27/1 555), P<0.001] and exposure to harmful environments [7.6%(22/289) vs. 2.1%(33/1 555), P<0.001], there was no statistically significant difference in interstitial lung disease between male and female patients [69.3%(181/261) vs. 74.5%(1 085/1 457), P=0.084].Otherwise, Raynaud's phenomenon [87.7% (1 364/1 555) vs.75.4%(218/289), P<0.001], arthritis [11.1%(173/1 555) vs.6.9%(20/289), P=0.032], gastroesophageal reflux disease [22.0%(342/ 1 555) vs.13.1%(38/289), P=0.001], and leucopoenia [10.7(161/1 511)% vs. 6.1%(17/279), P=0.019] were more common in female patients, but finger ulcer was less common [22.5%(350/1 555) vs. 30.4%(88/289), P=0.004]. Antinuclear antibody(ANA) positivity rate [85.6%(1 310/1 531) vs. 78.6%(221/281), P=0.003], anti-RNP antibody positivity rate [23.1%(342/1 479) vs.14.0%(38/271), P=0.001], anti-SSA antibody positivity rate [28.2%(419/1 487) vs.13.9%(38/274), P<0.001] were higher in female patients. Physician's global assessment(PGA) scores [1.4 (1.0, 2.0) vs. 1.0 (0.3, 1.6), P<0.001] and modified Rodnan Skin Score(mRSS) [18.0 (9.5, 28.0) vs. 14.0 (5.0, 28.0), P=0.003] were higher in males. Conclusion: Even though male SSc patients account for a small proportion, more extensive skin involvement, finger ulcers and higher PGA are manifested in males. Physicians need pay attention to these clinical disparities between different genders in SSc. FAU - Hou, Y Y AU - Hou YY AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Duan, X W AU - Duan XW AD - Department of Rheumatology and Immunology, the Second Affiliated Hospital of Nanchang University, Nanchang 330006, China. FAU - Li, Q AU - Li Q AD - Department of Rheumatology and Immunology, the First People's Hospital of Yunnan Province, Kunming 650032, China. FAU - Li, H B AU - Li HB AD - Affiliated Hospital of Inner Mongolia Medical University, Hohhot 028000, China. FAU - Zhou, J X AU - Zhou JX AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Hou, Y AU - Hou Y AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Li, M T AU - Li MT AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Zhao, J L AU - Zhao JL AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Wang, Q AU - Wang Q AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Xu, D AU - Xu D AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. FAU - Zeng, Xiaofeng AU - Zeng X AD - Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China. LA - chi PT - Journal Article PL - China TA - Zhonghua Nei Ke Za Zhi JT - Zhonghua nei ke za zhi JID - 16210490R RN - 0 (Antibodies, Antinuclear) SB - IM MH - Adult MH - Antibodies, Antinuclear MH - China/epidemiology MH - Female MH - Humans MH - *Lung Diseases, Interstitial MH - Male MH - Middle Aged MH - Retrospective Studies MH - *Scleroderma, Systemic EDAT- 2022/03/29 06:00 MHDA- 2022/04/01 06:00 CRDT- 2022/03/28 01:14 PHST- 2022/03/28 01:14 [entrez] PHST- 2022/03/29 06:00 [pubmed] PHST- 2022/04/01 06:00 [medline] AID - 10.3760/cma.j.cn112138-20210825-00586 [doi] PST - ppublish SO - Zhonghua Nei Ke Za Zhi. 2022 Apr 1;61(4):403-408. doi: 10.3760/cma.j.cn112138-20210825-00586. PMID- 17393393 OWN - NLM STAT- MEDLINE DCOM- 20070510 LR - 20220317 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 56 IP - 4 DP - 2007 Apr TI - Clinical and immunogenetic features of patients with autoantibodies to asparaginyl-transfer RNA synthetase. PG - 1295-303 AB - OBJECTIVE: We have previously described anti-KS autoantibodies and provided evidence that they are directed against asparaginyl-transfer RNA (tRNA) synthetase (AsnRS). The aim of the present study was to identify patients with anti-AsnRS autoantibodies and elucidate the clinical significance of this sixth antisynthetase antibody. In particular, we studied whether it was associated with the syndrome of myositis (polymyositis or dermatomyositis [DM]), interstitial lung disease (ILD), arthritis, and other features that had been previously associated with the 5 other anti-aminoacyl-tRNA synthetase autoantibodies. METHODS: More than 2,500 sera from patients with connective tissue disease (including myositis and ILD) and controls were examined for anti-AsnRS autoantibodies by immunoprecipitation (IP). Positive and control sera were tested for the ability to inhibit AsnRS by preincubation of the enzyme source with the serum. The HLA class II (DRB1, DQA1, DQB1, DPB1) alleles were identified from restriction fragment length polymorphism of polymerase chain reaction-amplified genomic DNA. RESULTS: Anti-AsnRS antibodies were identified in the sera of 8 patients (5 Japanese, 1 American, 1 German, and 1 Korean) by IP of the same distinctive set of tRNA and protein that differed from those precipitated by the other 5 antisynthetases, and these antibodies showed specific inhibition of AsnRS activity. Two of these patients had DM, but 7 of 8 (88%) had ILD. Four patients (50%) had arthritis, and 1 had Raynaud's phenomenon. This antisynthetase was very rare among myositis patients (present in 0% of Japanese myositis patients), but it was found in 3% of Japanese ILD patients. Thus, most patients with anti-AsnRS had chronic ILD with or without features of connective tissue disease. Interestingly, all 4 Japanese patients tested had DR2 (DRB1*1501/1502), compared with 33% of healthy controls. CONCLUSION: These results indicate that anti-AsnRS autoantibodies, like anti-alanyl-tRNA synthetase autoantibodies, have a stronger association with ILD than with myositis and may be associated with the DR2 phenotype. FAU - Hirakata, Michito AU - Hirakata M AD - Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, and Seoul National University Hospital, Korea. mhirakat@sc.itc.keio.ac.jp FAU - Suwa, Akira AU - Suwa A FAU - Takada, Tetsuya AU - Takada T FAU - Sato, Shinji AU - Sato S FAU - Nagai, Sonoko AU - Nagai S FAU - Genth, Ekkehard AU - Genth E FAU - Song, Yeong W AU - Song YW FAU - Mimori, Tsuneyo AU - Mimori T FAU - Targoff, Ira N AU - Targoff IN LA - eng GR - P30 AR053483/AR/NIAMS NIH HHS/United States PT - Case Reports PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (HLA-DR2 Antigen) RN - 0 (RNA, Transfer, Amino Acyl) RN - EC 6.1.1.12 (Aspartate-tRNA Ligase) RN - EC 6.1.1.22 (asparaginyl-tRNA synthetase) SB - IM MH - Aged MH - Arthritis/genetics/*immunology/pathology MH - Aspartate-tRNA Ligase/*immunology MH - Autoantibodies/*blood MH - Autoantigens/*immunology MH - Dermatomyositis/genetics/*immunology/pathology MH - Fatal Outcome MH - Female MH - HLA-DR2 Antigen/*genetics MH - Haplotypes MH - HeLa Cells MH - Histocompatibility Testing MH - Humans MH - Lung Diseases, Interstitial/genetics/*immunology/pathology MH - Male MH - Middle Aged MH - Polymorphism, Restriction Fragment Length MH - Polymyositis/genetics/*immunology/pathology MH - RNA, Transfer, Amino Acyl/*immunology MH - Syndrome EDAT- 2007/03/30 09:00 MHDA- 2007/05/11 09:00 CRDT- 2007/03/30 09:00 PHST- 2007/03/30 09:00 [pubmed] PHST- 2007/05/11 09:00 [medline] PHST- 2007/03/30 09:00 [entrez] AID - 10.1002/art.22506 [doi] PST - ppublish SO - Arthritis Rheum. 2007 Apr;56(4):1295-303. doi: 10.1002/art.22506. PMID- 37053291 OWN - NLM STAT- MEDLINE DCOM- 20230417 LR - 20230418 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 18 IP - 4 DP - 2023 TI - Development and validation of algorithms to build an electronic health record based cohort of patients with systemic sclerosis. PG - e0283775 LID - 10.1371/journal.pone.0283775 [doi] LID - e0283775 AB - OBJECTIVES: To evaluate methods of identifying patients with systemic sclerosis (SSc) using International Classification of Diseases, Tenth Revision (ICD-10) codes (M34*), electronic health record (EHR) databases and organ involvement keywords, that result in a validated cohort comprised of true cases with high disease burden. METHODS: We retrospectively studied patients in a healthcare system likely to have SSc. Using structured EHR data from January 2016 to June 2021, we identified 955 adult patients with M34* documented 2 or more times during the study period. A random subset of 100 patients was selected to validate the ICD-10 code for its positive predictive value (PPV). The dataset was then divided into a training and validation sets for unstructured text processing (UTP) search algorithms, two of which were created using keywords for Raynaud's syndrome, and esophageal involvement/symptoms. RESULTS: Among 955 patients, the average age was 60. Most patients (84%) were female; 75% of patients were White, and 5.2% were Black. There were approximately 175 patients per year with the code newly documented, overall 24% had an ICD-10 code for esophageal disease, and 13.4% for pulmonary hypertension. The baseline PPV was 78%, which improved to 84% with UTP, identifying 788 patients likely to have SSc. After the ICD-10 code was placed, 63% of patients had a rheumatology office visit. Patients identified by the UTP search algorithm were more likely to have increased healthcare utilization (ICD-10 codes 4 or more times 84.1% vs 61.7%, p < .001), organ involvement (pulmonary hypertension 12.7% vs 6% p = .011) and medication use (mycophenolate use 28.7% vs 11.4%, p < .001) than those identified by the ICD codes alone. CONCLUSION: EHRs can be used to identify patients with SSc. Using unstructured text processing keyword searches for SSc clinical manifestations improved the PPV of ICD-10 codes alone and identified a group of patients most likely to have SSc and increased healthcare needs. CI - Copyright: © 2023 Tukpah et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. FAU - Tukpah, Ann-Marcia C AU - Tukpah AC AUID- ORCID: 0000-0002-2877-6535 AD - Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. FAU - Rose, Jonathan A AU - Rose JA AD - Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. FAU - Seger, Diane L AU - Seger DL AD - Division of General Internal Medicine and Primary Care, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. FAU - Dellaripa, Paul F AU - Dellaripa PF AD - Division of Rheumatology, Inflammation and Immunity, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. FAU - Hunninghake, Gary M AU - Hunninghake GM AD - Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. FAU - Bates, David W AU - Bates DW AD - Division of General Internal Medicine and Primary Care, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, United States of America. LA - eng GR - T32 HL007633/HL/NHLBI NIH HHS/United States GR - R01 HL111024/HL/NHLBI NIH HHS/United States GR - R01 HL130974/HL/NHLBI NIH HHS/United States GR - R01 HL135142/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20230413 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - UT0S826Z60 (Uridine Triphosphate) SB - IM MH - Adult MH - Humans MH - Female MH - Middle Aged MH - Male MH - Electronic Health Records MH - Retrospective Studies MH - *Hypertension, Pulmonary MH - Uridine Triphosphate MH - Reproducibility of Results MH - Algorithms MH - International Classification of Diseases MH - *Scleroderma, Systemic/epidemiology MH - Databases, Factual PMC - PMC10101630 COIS- Dr. Bates reports grants and personal fees from EarlySense, personal fees from CDI Negev, equity from ValeraHealth, equity from Clew, equity from MDClone, personal fees and equity from AESOP, personal fees and equity from Feelbetter, equity from Guided Clinical Solutions, and grants from IBM Watson Health, outside the submitted work. Dr. Bates has a patent pending (PHC-028564 US PCT), on intraoperative clinical decision support. This does not alter our adherence to PLOS ONE policies on sharing data and materials. Dr. Hunninghake has performed consulting work for the Gerson Lehrman Group, Boehringer-Ingelheim, and Chugai Pharmaceuticals in the last 3 years. In addition, he receive some funding support with Bayer Pulmonary Research Lab at the Brigham and Women’s Hospital. EDAT- 2023/04/14 06:00 MHDA- 2023/04/17 06:41 PMCR- 2023/04/13 CRDT- 2023/04/13 14:04 PHST- 2022/10/25 00:00 [received] PHST- 2023/03/16 00:00 [accepted] PHST- 2023/04/17 06:41 [medline] PHST- 2023/04/13 14:04 [entrez] PHST- 2023/04/14 06:00 [pubmed] PHST- 2023/04/13 00:00 [pmc-release] AID - PONE-D-22-29470 [pii] AID - 10.1371/journal.pone.0283775 [doi] PST - epublish SO - PLoS One. 2023 Apr 13;18(4):e0283775. doi: 10.1371/journal.pone.0283775. eCollection 2023. PMID- 28664832 OWN - NLM STAT- MEDLINE DCOM- 20171221 LR - 20171221 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 Suppl 106 IP - 4 DP - 2017 Sep-Oct TI - Clinical determinants of elevated systolic pulmonary artery pressure measured by transthoracic Doppler echocardiography in early systemic sclerosis. PG - 114-121 AB - OBJECTIVES: To explore the prevalence and clinical associations of elevated systolic pulmonary artery pressure (sPAP), measured by Transthoracic Doppler-echocardiography (TTE) in patients with early systemic sclerosis (SSc). METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research (EUSTAR) database was performed. SSc patients with <3 years from the first non-Raynaud's phenomenon (RP) symptom at baseline EUSTAR visit, were selected. Elevated sPAP was defined as sPAP>40 mmHg on baseline TTE. First visit SSc related variables, including disease subsets, antibodies and visceral involvement, were examined. RESULTS: From 1,188 patients, 81% were women. Mean (SD) age at first non-RP symptom was 50 (14) years, 55% had limited cutaneous SSc (lcSSc) and 42% active disease. Elevated sPAP was found in 17% of patients, both lcSSc and diffuse cutaneous SSc (dcSSc). In lcSSc, older age at first non-RP symptom, ACA positivity, joint contractures, restrictive defect and lower DLCO, were independently associated with elevated sPAP. In dcSSc, older age at first non-RP symptom, longer time between RP onset and first non-RP symptom, digital ulcers, cardiac blocks, and proteinuria were associated with elevated sPAP. CONCLUSIONS: The prevalence of elevated sPAP on TTE in early SSc patients is considerable. Association with cardiac, lung and renal involvement suggests that, although some patients might have pulmonary arterial hypertension, others may present pulmonary hypertension secondary to lung or heart involvement. Our findings emphasize the need to consider right heart catheterisation in selected early SSc patients with PH suspicion, to clearly determine the cause of PH. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario12 de Octubre, Madrid, Spain. carreira@h12o.es. FAU - Carmona, Loreto AU - Carmona L AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Joven, Beatriz E AU - Joven BE AD - Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Loza, Estibaliz AU - Loza E AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - Andreu, Jose Luis AU - Andreu JL AD - Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology, University of Lübeck, Germany. FAU - Vettori, Serena AU - Vettori S AD - UOC di Reumatologia, Dipartimento di Internistica Clinica e Sperimentale "F-Magrassi-A-Lanzara", Seconda Università di Napoli, Italy. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A Department, Cochin Hospital, APHP, Paris Descartes University, France. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B.Shine Rheumatology Unit, Rambam Health Care Campus and Rappaport Faculty of Medicine-Technion, Haifa, Israel. FAU - Airò, Paolo AU - Airò P AD - UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy. FAU - Walker, Ulrich A AU - Walker UA AD - Department of Rheumatology, Unispital Basel, Switzerland. FAU - Damjanov, Nemanja AU - Damjanov N AD - University of Belgrade School of Medicine, Institute of Rheumatology, Belgrade, Serbia. FAU - Ananieva, Lidia P AU - Ananieva LP AD - Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia. FAU - Rednic, Simona AU - Rednic S AD - Clinica Reumatologie, University of Medicine & Pharmacy "Iuliu Hatieganu", Cluj-Napoca, Romania. FAU - Czirják, László AU - Czirják L AD - Department of Immunology and Rheumatology, Faculty of Medicine, University of Pécs, Hungary. FAU - Distler, Oliver AU - Distler O AD - Division of Rheumatology, University Hospital Zurich, Switzerland. FAU - Farge, Dominique AU - Farge D AD - Department of Internal Medicine, Hopital Saint-Louis, Paris, France. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University, Sweden. FAU - Corrado, Ada AU - Corrado A AD - UO Reumatologia-Università degli Studi di Foggia, Ospedale "Col. D'Avanzo", Foggia, Italy. FAU - Caramaschi, Paola AU - Caramaschi P AD - Rheumatology Unit, AOUI, Verona, Italy. FAU - Tikly, Mohammed AU - Tikly M AD - Rheumatology Unit, Department of Medicine Chris Hani Baragwanath, Hospital and University of the Witwatersrand, Johannesburg, South Africa. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Florence, Italy. CN - and EUSTAR co-authors LA - eng PT - Journal Article DEP - 20170620 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Echocardiography/*methods MH - Echocardiography, Doppler/*methods MH - Female MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Pulmonary Artery/*physiopathology MH - Scleroderma, Systemic/diagnostic imaging/*physiopathology MH - Systole/*physiology EDAT- 2017/07/01 06:00 MHDA- 2017/12/22 06:00 CRDT- 2017/07/01 06:00 PHST- 2016/06/24 00:00 [received] PHST- 2016/10/26 00:00 [accepted] PHST- 2017/07/01 06:00 [pubmed] PHST- 2017/12/22 06:00 [medline] PHST- 2017/07/01 06:00 [entrez] AID - 10824 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):114-121. Epub 2017 Jun 20. PMID- 24122180 OWN - NLM STAT- MEDLINE DCOM- 20140102 LR - 20260304 IS - 1529-0131 (Electronic) IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 65 IP - 11 DP - 2013 Nov TI - 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. PG - 2737-47 LID - 10.1002/art.38098 [doi] AB - OBJECTIVE: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. METHODS: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. RESULTS: It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. CONCLUSION: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease. CI - Copyright © 2013 by the American College of Rheumatology. FAU - van den Hoogen, Frank AU - van den Hoogen F AD - St. Maartenskliniek and Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. FAU - Khanna, Dinesh AU - Khanna D FAU - Fransen, Jaap AU - Fransen J FAU - Johnson, Sindhu R AU - Johnson SR FAU - Baron, Murray AU - Baron M FAU - Tyndall, Alan AU - Tyndall A FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M FAU - Naden, Raymond P AU - Naden RP FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Carreira, Patricia E AU - Carreira PE FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Clements, Philip J AU - Clements PJ FAU - Denton, Christopher P AU - Denton CP FAU - Distler, Oliver AU - Distler O FAU - Allanore, Yannick AU - Allanore Y FAU - Furst, Daniel E AU - Furst DE FAU - Gabrielli, Armando AU - Gabrielli A FAU - Mayes, Maureen D AU - Mayes MD FAU - van Laar, Jacob M AU - van Laar JM FAU - Seibold, James R AU - Seibold JR FAU - Czirjak, Laszlo AU - Czirjak L FAU - Steen, Virginia D AU - Steen VD FAU - Inanc, Murat AU - Inanc M FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Valentini, Gabriele AU - Valentini G FAU - Veale, Douglas J AU - Veale DJ FAU - Vonk, Madelon C AU - Vonk MC FAU - Walker, Ulrich A AU - Walker UA FAU - Chung, Lorinda AU - Chung L FAU - Collier, David H AU - Collier DH FAU - Csuka, Mary Ellen AU - Csuka ME FAU - Fessler, Barri J AU - Fessler BJ FAU - Guiducci, Serena AU - Guiducci S FAU - Herrick, Ariane AU - Herrick A FAU - Hsu, Vivien M AU - Hsu VM FAU - Jimenez, Sergio AU - Jimenez S FAU - Kahaleh, Bashar AU - Kahaleh B FAU - Merkel, Peter A AU - Merkel PA FAU - Sierakowski, Stanislav AU - Sierakowski S FAU - Silver, Richard M AU - Silver RM FAU - Simms, Robert W AU - Simms RW FAU - Varga, John AU - Varga J FAU - Pope, Janet E AU - Pope JE LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - UL1 TR000371/TR/NCATS NIH HHS/United States GR - K24-AR-063120/AR/NIAMS NIH HHS/United States GR - CAPMC/CIHR/Canada PT - Consensus Statement PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20131003 PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 SB - IM CIN - Ann Rheum Dis. 2014 May;73(5):e29. doi: 10.1136/annrheumdis-2014-205193. PMID: 24464963 CIN - Ann Rheum Dis. 2014 May;73(5):e30. doi: 10.1136/annrheumdis-2014-205200. PMID: 24492752 MH - *Diagnosis-Related Groups MH - Humans MH - *Rheumatology MH - Scleroderma, Systemic/*classification/*diagnosis/immunology MH - Sensitivity and Specificity PMC - PMC3930146 MID - NIHMS541263 EDAT- 2013/10/15 06:00 MHDA- 2014/01/03 06:00 PMCR- 2014/11/01 CRDT- 2013/10/15 06:00 PHST- 2013/01/26 00:00 [received] PHST- 2013/07/16 00:00 [accepted] PHST- 2013/10/15 06:00 [entrez] PHST- 2013/10/15 06:00 [pubmed] PHST- 2014/01/03 06:00 [medline] PHST- 2014/11/01 00:00 [pmc-release] AID - 10.1002/art.38098 [doi] PST - ppublish SO - Arthritis Rheum. 2013 Nov;65(11):2737-47. doi: 10.1002/art.38098. Epub 2013 Oct 3. PMID- 38216288 OWN - NLM STAT- MEDLINE DCOM- 20240115 LR - 20241209 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 10 IP - 1 DP - 2024 Jan 12 TI - Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets. LID - 10.1136/rmdopen-2023-003216 [doi] LID - e003216 AB - BACKGROUND: Hand involvement is an early manifestation of systemic sclerosis (SSc), culprit of diagnosis and classification, and recognised major driver of disability. Impairment of hand function burdens both limited and diffuse cutaneous subsets and therefore could be targeted as 'basket' endpoint in SSc. Nevertheless, its natural history in current standard of care is not well characterised, limiting the design of targeted trials. The aim of this study is to describe prevalence, natural history and clinical factors associated with hand function deterioration in a longitudinal, multicentre, observational SSc cohort. METHODS: Hand function was captured through the validated Cochin Hand Function Scale in patients consecutively enrolled in a multicentre observational study and observed over 24 months. Minimal clinically important differences and patient acceptable symptom state were analysed as previously described. RESULTS: Three hundred and ninety-six consecutive patients were enrolled from 10 centres; 201 with complete follow-up data were included in the analysis. Median (IQR) disease duration was 5 (2-11) years. One hundred and five (52.2%) patients reported clinically significant worsening. Accordingly, the proportion of patients reporting unacceptable hand function increased over 2 years from 27.8% to 35.8% (p<0.001). Least absolute shrinkage and selection operator analysis identified male gender, disease subset, Raynaud's Condition Score, tenosynovitis and pain, as some of the key factors associated with worsening hand involvement. CONCLUSIONS: Hand function deteriorates over time in more than 50% of SSc patients despite available therapies. The analysis of factors associated with hand function worsening supports the involvement of both inflammation, vascular and fibrotic processes in hand involvement, making it a hallmark clinical manifestation of SSc. Our data are poised to inform the design of intervention studies to target this major driver of disability in SSc. CI - © Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ. FAU - De Lorenzis, Enrico AU - De Lorenzis E AUID- ORCID: 0000-0001-9819-105X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. FAU - Kakkar, Vishal AU - Kakkar V AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Wilson, Michelle AU - Wilson M AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Barnes, Theresa AU - Barnes T AD - Department of Rheumatology, Countess of Chester Hospital NHS Foundation Trust, Liverpool, UK. FAU - Denton, Chris AU - Denton C AD - Department of Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Derrett-Smith, Emma AU - Derrett-Smith E AD - Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK. FAU - Douglas, Karen AU - Douglas K AD - Department of Rheumatology, The Dudley Group NHS Foundation Trust, Dudley, UK. FAU - Helliwell, Philip AU - Helliwell P AUID- ORCID: 0000-0002-4155-9105 AD - Department of Rheumatology, St. Luke's Hospital, Bradford Teaching Hospitals NHS Foundation Trust, Bradford, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust,Manchester Academic Health Science Centre, Manchester, UK. FAU - Saleem, Benazir AU - Saleem B AUID- ORCID: 0000-0001-8719-7800 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - Department of Rheumatology, York Teaching Hospital NHS Foundation Trust, York, UK. FAU - Nisar, Muhammad AU - Nisar M AD - Department of Rheumatology, Luton & Dunstable University Hospital NHS Foundation Trust, Luton, UK. FAU - Morley, Catherine AU - Morley C AD - Department of Rheumatology, South Tyneside and Sunderland NHS Foundation Trust, Sunderland, UK. FAU - Green, Lorraine AU - Green L AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Alcacer-Pitarch, Begonya AU - Alcacer-Pitarch B AUID- ORCID: 0000-0002-2208-444X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK f.delgaldo@leeds.ac.uk. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. LA - eng PT - Journal Article PT - Observational Study DEP - 20240112 PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM MH - Humans MH - Male MH - *Scleroderma, Systemic/complications/epidemiology/diagnosis MH - Hand PMC - PMC10806527 OTO - NOTNLM OT - Outcome Assessment, Health Care OT - Patient Reported Outcome Measures OT - Systemic Sclerosis COIS- Competing interests: Direct or indirect interests that might affect or be perceived to affect the conduct or reporting of this work. EDL: nothing to declare, VK; nothing to declare, SDD: nothing to declare, MW: nothing to declare, TB: nothing to declare, CPD: nothing to declare, EDS: nothing to declare, KD: nothing to declare, PW: nothing to declare, AH: nothing to declare, BS: nothing to declare, MN: nothing to declare, CM: nothing to declare, LG: nothing to declare, BAP: nothing to declare, FDG: nothing to declare. EDAT- 2024/01/13 00:42 MHDA- 2024/01/15 12:42 PMCR- 2024/01/12 CRDT- 2024/01/12 21:02 PHST- 2023/04/09 00:00 [received] PHST- 2023/07/15 00:00 [accepted] PHST- 2024/01/15 12:42 [medline] PHST- 2024/01/13 00:42 [pubmed] PHST- 2024/01/12 21:02 [entrez] PHST- 2024/01/12 00:00 [pmc-release] AID - rmdopen-2023-003216 [pii] AID - 10.1136/rmdopen-2023-003216 [doi] PST - epublish SO - RMD Open. 2024 Jan 12;10(1):e003216. doi: 10.1136/rmdopen-2023-003216. PMID- 28217959 OWN - NLM STAT- MEDLINE DCOM- 20170817 LR - 20191210 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 69 IP - 6 DP - 2017 Jun TI - Brief Report: Anti-RNPC-3 Antibodies As a Marker of Cancer-Associated Scleroderma. PG - 1306-1312 LID - 10.1002/art.40065 [doi] AB - OBJECTIVE: Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with anti-RNA polymerase III (anti-RNAP III) autoantibodies as well as in patients who are triple-negative for anticentromere (anti-CENP), anti-topoisomerase I (anti-topo I), and anti-RNAP III (also known as anti-POL) autoantibodies (referred to as CTP negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, 25% of the patients were found to have autoantibodies to RNPC-3, a member of the minor spliceosome complex. This investigation was undertaken to validate the relationship between anti-RNPC-3 antibodies and cancer and examine the associated clinical phenotype in a large sample of scleroderma patients. METHODS: Scleroderma patients with cancer were assayed for anti-CENP, anti-topo I, anti-RNAP III, and anti-RNPC-3 autoantibodies. Disease characteristics and the cancer-scleroderma interval were compared across autoantibody groups. The relationship between autoantibody status and cancer-associated scleroderma was assessed by logistic regression. RESULTS: Of 318 patients with scleroderma and cancer, 70 (22.0%) were positive for anti-RNAP III, 54 (17.0%) were positive for anti-topo I, and 96 (30.2%) were positive for anti-CENP. Twelve patients (3.8% of the overall group or 12.2% of CTP-negative patients) were positive for anti-RNPC-3. Patients with anti-RNPC-3 had a short cancer-scleroderma interval (median 0.9 years). Relative to patients with anti-CENP, patients with anti-RNPC-3 and those with anti-RNAP III had a >4-fold increased risk of cancer within 2 years of scleroderma onset (for anti-RNPC-3-positive patients, odds ratio [OR] 4.3, 95% confidence interval [95% CI] 1.10-16.9 [P = 0.037]; for anti-RNAP III-positive patients, OR 4.49, 95% CI 1.98-10.2 [P < 0.001]). Patients with anti-RNPC-3 had severe restrictive lung disease, gastrointestinal disease, Raynaud's phenomenon, and myopathy. CONCLUSION: Anti-RNPC-3 autoantibodies, similar to anti-RNAP III autoantibodies, are associated with an increased risk of cancer at the onset of scleroderma. These data suggest the possibility of cancer-induced autoimmunity in this subset of patients with scleroderma. CI - © 2017, American College of Rheumatology. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Xu, George AU - Xu G AD - Harvard University and Harvard-Massachusetts Institute of Technology Division of Health Sciences and Technology, Cambridge, Massachusetts, and Howard Hughes Medical Institute and Brigham and Women's Hospital, Boston, Massachusetts. FAU - Rosen, Antony AU - Rosen A AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Hummers, Laura K AU - Hummers LK AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Wigley, Fredrick M AU - Wigley FM AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Elledge, Stephen J AU - Elledge SJ AD - Howard Hughes Medical Institute, Brigham and Women's Hospital, and Harvard University Medical School, Boston, Massachusetts. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Johns Hopkins University School of Medicine, Baltimore, Maryland. LA - eng GR - K23 AR061439/AR/NIAMS NIH HHS/United States GR - P30 AR053503/AR/NIAMS NIH HHS/United States GR - R01 DE012354/DE/NIDCR NIH HHS/United States PT - Journal Article PT - Validation Study PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Nuclear Proteins) RN - 0 (RNA-Binding Proteins) RN - 0 (RNPC3 protein, human) SB - IM CIN - Arthritis Rheumatol. 2017 Sep;69(9):1914-1915. doi: 10.1002/art.40129. PMID: 28426925 CIN - Arthritis Rheumatol. 2017 Sep;69(9):1915-1916. doi: 10.1002/art.40132. PMID: 28454209 MH - Adult MH - Autoantibodies/*blood/immunology MH - Biomarkers/blood MH - Female MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Neoplasms/*blood/complications/immunology MH - Nuclear Proteins/*immunology MH - RNA-Binding Proteins/*immunology MH - Scleroderma, Systemic/*blood/immunology PMC - PMC5449218 MID - NIHMS849641 COIS- Conflicts of interest: None EDAT- 2017/02/22 06:00 MHDA- 2017/08/18 06:00 PMCR- 2018/06/01 CRDT- 2017/02/21 06:00 PHST- 2016/10/12 00:00 [received] PHST- 2017/02/02 00:00 [accepted] PHST- 2017/02/22 06:00 [pubmed] PHST- 2017/08/18 06:00 [medline] PHST- 2017/02/21 06:00 [entrez] PHST- 2018/06/01 00:00 [pmc-release] AID - 10.1002/art.40065 [doi] PST - ppublish SO - Arthritis Rheumatol. 2017 Jun;69(6):1306-1312. doi: 10.1002/art.40065. PMID- 22556030 OWN - NLM STAT- MEDLINE DCOM- 20121101 LR - 20260518 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 64 IP - 9 DP - 2012 Sep TI - Association of C-reactive protein with high disease activity in systemic sclerosis: results from the Canadian Scleroderma Research Group. PG - 1405-14 LID - 10.1002/acr.21716 [doi] AB - OBJECTIVE: This study was performed to determine the prevalence of elevated C-reactive protein (CRP) levels and the significance of CRP in clinical parameters in systemic sclerosis (SSc; scleroderma) patients. METHODS: Canadian Scleroderma Research Group data were used. Statistical comparisons were made for CRP levels ≤8 mg/liter versus >8 mg/liter, early (≤3 years from first non-Raynaud's phenomenon symptom) versus late SSc, and diffuse cutaneous SSc (dcSSc) versus limited cutaneous SSc (lcSSc). A survival analysis was analyzed between patients with normal versus elevated CRP levels. RESULTS: A total of 1,043 patients (mean ± SD age 55.4 ± 12.1 years, mean ± SD disease duration of 11.0 ± 9.5 years) were analyzed; elevation of CRP level and erythrocyte sedimentation rate (ESR; >20 mm/hour) occurred in 25.7% and 38.2%, respectively. Mean ± SD baseline CRP level in dcSSc (11.98 ± 25.41 mg/liter) was higher than in lcSSc (8.15 ± 16.09 mg/liter; P = 0.016). SSc patients with an early disease duration had a higher mean ± SD CRP level (12.89 ± 28.13 mg/liter) than those with a late disease duration (8.60 ± 17.06 mg/liter; P = 0.041). Although not consistent in all subsets, CRP was significantly associated (P < 0.01) with ESR, modified Rodnan skin score (MRSS), worse pulmonary function parameters, disease activity, damage, and Health Assessment Questionnaire. CRP level seemed to normalize in many SSc patients over time. Total lung capacity <80% predicted, MRSS, and serum creatinine were predictors of elevated CRP levels in SSc (odds ratio [OR] 2.76 [95% confidence interval (95% CI) 1.73-4.40], P = 0.0001; OR 1.03 [95% CI 1.01-1.05], P = 0.005; and OR 1.005 [95% CI 1.001-1.010], P = 0.02, respectively). Survival for patients with elevated CRP levels was less than for patients with normal CRP levels (P = 0.001). CONCLUSION: CRP level is elevated in one-quarter of SSc patients, especially early disease. It is correlated with disease activity, severity, poor pulmonary function, and shorter survival. CI - Copyright © 2012 by the American College of Rheumatology. FAU - Muangchan, Chayawee AU - Muangchan C AD - University of Western Ontario, London, Ontario, Canada. FAU - Harding, Sarah AU - Harding S FAU - Khimdas, Sarit AU - Khimdas S FAU - Bonner, Ashley AU - Bonner A CN - Canadian Scleroderma Research group FAU - Baron, Murray AU - Baron M FAU - Pope, Janet AU - Pope J LA - eng GR - Canadian Institutes of Health Research/Canada PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Biomarkers) RN - 9007-41-4 (C-Reactive Protein) RN - AYI8EX34EU (Creatinine) SB - IM MH - Adult MH - Aged MH - Biomarkers/blood MH - Blood Sedimentation MH - C-Reactive Protein/*analysis MH - Canada MH - Creatinine/blood MH - Female MH - Humans MH - Kaplan-Meier Estimate MH - Logistic Models MH - Lung/physiopathology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Prognosis MH - Prospective Studies MH - Registries MH - Respiratory Function Tests MH - Risk Assessment MH - Risk Factors MH - Scleroderma, Diffuse/blood/diagnosis/immunology MH - Scleroderma, Limited/blood/diagnosis/immunology MH - Scleroderma, Systemic/blood/*diagnosis/immunology/mortality/physiopathology MH - Severity of Illness Index MH - Skin/pathology MH - Surveys and Questionnaires MH - Time Factors MH - Total Lung Capacity MH - Up-Regulation FIR - Baron, M IR - Baron M FIR - Mathieu, J -P IR - Mathieu J- FIR - Hudson, M IR - Hudson M FIR - Ligier, S IR - Ligier S FIR - Grodzicky, T IR - Grodzicky T FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Khalidi, N IR - Khalidi N FIR - Kaminska, E IR - Kaminska E FIR - Docherty, P IR - Docherty P FIR - Masetto, A IR - Masetto A FIR - Sutton, E IR - Sutton E FIR - LeClercq, S IR - LeClercq S FIR - Thorne, C IR - Thorne C FIR - Fritzler, M IR - Fritzler M EDAT- 2012/05/05 06:00 MHDA- 2012/11/02 06:00 CRDT- 2012/05/05 06:00 PHST- 2012/05/05 06:00 [entrez] PHST- 2012/05/05 06:00 [pubmed] PHST- 2012/11/02 06:00 [medline] AID - 10.1002/acr.21716 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2012 Sep;64(9):1405-14. doi: 10.1002/acr.21716. PMID- 19896197 OWN - NLM STAT- MEDLINE DCOM- 20100218 LR - 20220317 IS - 1549-4713 (Electronic) IS - 0161-6420 (Linking) VI - 117 IP - 1 DP - 2010 Jan TI - Factors affecting rates of visual field progression in glaucoma patients with optic disc hemorrhage. PG - 24-9 LID - 10.1016/j.ophtha.2009.06.028 [doi] AB - PURPOSE: Optic disc hemorrhage (DH) is an important risk factor for glaucoma progression. We sought to investigate factors affecting the rate of visual field (VF) progression after DH in glaucomatous eyes. DESIGN: Retrospective cohort study. PARTICIPANTS: Consecutive glaucoma patients from our Glaucoma Progression Study with > or =5 Swedish interactive threshold algorithm standard 24-2 VFs from 1999 through 2008. METHODS: Disc photographs of all patients were evaluated for the presence of DH. Exclusion criteria were conditions other than glaucoma likely to affect the VF and insufficient number of VFs to create a slope after DH detection. Automated pointwise linear regression was used to determine the rate of VF loss after DH detection. Fast progression was defined as a global VF loss of > or =1.5 dB/year. Factors associated with a fast rate of VF loss after the detection of the DH were evaluated. MAIN OUTCOME MEASURES: Assessed variables included baseline (age, gender, intraocular pressure [IOP], central corneal thickness, VF mean deviation [MD], presence of migraine, Raynaud's phenomenon, low blood pressure, and exfoliation syndrome) and intercurrent data (DH recurrence, fellow eye involvement, glaucoma surgery, and IOP reduction). Odds ratios (ORs) and 95% confidence intervals (CIs) were determined for each variable. RESULTS: Seventy-six eyes (76 patients; mean age, 68.3+/-10.9 years) were enrolled. Mean IOP and VF MD at the time of the DH detection were 16.6+/-3.8 mmHg and -5.6+/-5.7 dB, respectively. The mean global progression rate after DH was -1.1+/-1.3 dB/year (mean follow-up, 3.8+/-2.8 years). A rate of progression of > or =1.5 dB/year was found in 20 (26%) eyes. Multivariate logistic regression analysis revealed larger baseline MD (OR, 1.11; 95% CI, 1.01-1.20; P = 0.03) and older age (OR, 1.06; 95% CI, 1.01-1.13; P = 0.04) to be significant risk factors for fast progression after DH. Eyes with a baseline MD worse than -4.0 dB had a 270% increased risk of fast progression compared with those with an MD better than -4.0 dB. CONCLUSIONS: The presence of a DH in older subjects with a worse VF predicted further VF global MD deterioration by more than 5 dB within 4 years. These eyes should undergo careful and frequent disease surveillance and consideration should be given to more aggressive treatment. CI - Copyright 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved. FAU - Prata, Tiago S AU - Prata TS AD - Department of Ophthalmology, Einhorn Clinical Research Center, New York Eye and Ear Infirmary, New York, New York, USA. FAU - De Moraes, Carlos Gustavo V AU - De Moraes CG FAU - Teng, Christopher C AU - Teng CC FAU - Tello, Celso AU - Tello C FAU - Ritch, Robert AU - Ritch R FAU - Liebmann, Jeffrey M AU - Liebmann JM LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20091106 PL - United States TA - Ophthalmology JT - Ophthalmology JID - 7802443 SB - IM MH - Aged MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Glaucoma/*physiopathology MH - Humans MH - Intraocular Pressure MH - Male MH - Optic Disk/*physiopathology MH - Optic Nerve Diseases/*physiopathology MH - Photography MH - Retinal Hemorrhage/*physiopathology MH - Retrospective Studies MH - Risk Factors MH - Vision Disorders/*physiopathology MH - Visual Field Tests MH - *Visual Fields EDAT- 2009/11/10 06:00 MHDA- 2010/02/19 06:00 CRDT- 2009/11/10 06:00 PHST- 2009/02/18 00:00 [received] PHST- 2009/05/17 00:00 [revised] PHST- 2009/06/12 00:00 [accepted] PHST- 2009/11/10 06:00 [entrez] PHST- 2009/11/10 06:00 [pubmed] PHST- 2010/02/19 06:00 [medline] AID - S0161-6420(09)00656-3 [pii] AID - 10.1016/j.ophtha.2009.06.028 [doi] PST - ppublish SO - Ophthalmology. 2010 Jan;117(1):24-9. doi: 10.1016/j.ophtha.2009.06.028. Epub 2009 Nov 6. PMID- 26005882 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 33 IP - 4 Suppl 91 DP - 2015 Jul-Aug TI - Telangiectasia as a potential clinical marker of microvascular lesions in systemic sclerosis patients from EUSTAR data in China. PG - S106-10 AB - OBJECTIVES: To investigate the prevalence and clinical relevance of telangiectasia in Chinese patients with systemic sclerosis (SSc). METHODS: Data from 230 SSc EUSTAR patients from Peking Union Medical College Hospital (2009-2011) that fulfilled the 1980 American College of Rheumatology SSc classification criteria were prospectively collected. Demographic, clinical, and laboratory data were calculated between groups with and without telangiectasia, and a six-minute walk test, pulmonary function test (PFT), transthoracic echocardiography (TTE), right heart catheterisation (RHC) and modified Rodnan skin score (mRSS) were performed. RESULTS: 96 patients (41.7%) were diagnosed with telangiectasia. There were no significant differences between patients with and without telangiectasia based on gender, age at onset, Raynaud's phenomenon (RP) duration, or SSc classification. Disease duration both from RP onset of patients and from first non-RP manifestation of patients with telangiectasia was significantly longer than patients without (p<0.05). RP (97.9% vs. 90.3%), finger/toe sclerosis (96.9% vs. 88.1%), facial sclerosis (68.8% vs. 53.7%), digital ulcers (DUs; 40.6% vs. 23.1%), digital pitting (49.0% vs. 33.8%), joint contracture (20.8% vs. 10.4%) and erythrocyte sedimentation rate elevation (26.7% vs. 14.8%) were significantly greater in telangiectasia patients (p<0.05). There were no differences in autoantibody development between patients with and without telangiectasia (p>0.05). PFT showed that forced vital capacity (77.0±17.26 vs. 83.05±16.53, p=0.005) and diffusion capacity for CO of the lung (58.9±19.4 vs. 65.7±19.7, p=0.030) were lower, while forced expiratory volume ratio (87.02±7.8 vs. 84.33±7.1, p=0.029) was higher in SSc with telangiectasia. Pulmonary artery hypertension (PAH) prevalence (25.0% vs. 14.2%) was significantly greater in patients with telangiectasia. CONCLUSIONS: Telangiectasia are common in Chinese SSc patients and usually associated with DUs, RP, and PAH. Telangiectasia could be a clinical marker of microvascular disease in SSc. FAU - Zhang, Shang-zhu AU - Zhang SZ AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Xu, Dong AU - Xu D AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Li, Meng-tao AU - Li MT AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Hou, Yong AU - Hou Y AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Wang, Qian AU - Wang Q AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Tian, Zhuang AU - Tian Z AD - Department of Cardiology, Peking Union Medical College Hospital, Beijing, China. FAU - Liu, Yong-tai AU - Liu YT AD - Department of Cardiology, Peking Union Medical College Hospital, Beijing, China. FAU - Guo, Xiao-xiao AU - Guo XX AD - Department of Cardiology, Peking Union Medical College Hospital, Beijing, China. FAU - Lai, Jin-zhi AU - Lai JZ AD - Department of Cardiology, Peking Union Medical College Hospital, Beijing, China. FAU - Zhao, Jiu-liang AU - Zhao JL AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Hu, Chao-jun AU - Hu CJ AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Song, Ning AU - Song N AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Sun, Qiu-ning AU - Sun QN AD - Department of Dermatology, Peking Union Medical College Hospital, Beijing, China. FAU - Zhang, Feng-chun AU - Zhang FC AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Zhao, Yan AU - Zhao Y AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. FAU - Zeng, Xiao-feng AU - Zeng XF AD - Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. zengxfpumc@gmail.com. LA - eng PT - Journal Article DEP - 20150525 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - China/epidemiology MH - Databases, Factual MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/epidemiology MH - Male MH - Microvessels/*pathology/physiopathology MH - Middle Aged MH - Predictive Value of Tests MH - Prevalence MH - Prognosis MH - Prospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*diagnosis/epidemiology/pathology/physiopathology MH - Skin/*blood supply MH - Skin Ulcer/diagnosis/epidemiology/pathology MH - Telangiectasis/*diagnosis/epidemiology/pathology/physiopathology MH - Vasodilation EDAT- 2015/05/26 06:00 MHDA- 2015/10/27 06:00 CRDT- 2015/05/26 06:00 PHST- 2014/07/20 00:00 [received] PHST- 2015/01/26 00:00 [accepted] PHST- 2015/05/26 06:00 [entrez] PHST- 2015/05/26 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 8478 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S106-10. Epub 2015 May 25. PMID- 38910602 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250602 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 9 IP - 2 DP - 2024 Jun TI - Assessing hand and global disability in a cohort of Algerian patients with systemic sclerosis: Construct validities of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire. PG - 134-142 LID - 10.1177/23971983241231082 [doi] AB - OBJECTIVE: The study aimed to assess the construct validity of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire in Algerian patients with systemic sclerosis. METHODS: Consecutive Algerian patients who fulfilled the American College of Rheumatology/European League Against Rheumatism criteria for systemic sclerosis were included. In addition to disease characteristics, global disability and hand disability were assessed using the Arabic Health Assessment Questionnaire and the Arab Hand Function Index, respectively. Construct validity was assessed by convergent and divergent validity (Spearman's rank correlation coefficient) and factor analysis. The scale reliability was assessed using the Cronbach's alpha. RESULTS: We evaluated 100 systemic sclerosis patients (83 females) of mean ± standard deviation age 46.7 ± 12.3 years, including 59 limited cutaneous systemic sclerosis and 41 diffuse cutaneous systemic sclerosis. Raynaud's phenomenon was detected in 99 patients and digital ulcers in 25. Gastrointestinal tract involvement and interstitial lung disease were detected in 86/100 (86%) and 46/72 (63.9%) patients, respectively. Anti-topoisomerase I and anti-centromere antibodies were detected in 33/76 (43.4%) and 23/76 (30.3%) patients, respectively. The Arab Hand Function Index had a good construct validity with a total score explaining 61% of the variance of the Arabic Health Assessment Questionnaire which also had a good construct validity. Factor analysis of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire items extracted two factors explaining 64% of the variance for the Arab Hand Function Index and one factor explaining 55% of the variance for the Arabic Health Assessment Questionnaire. The Arab Hand Function Index and the Arabic Health Assessment Questionnaire were reliable questionnaires with a Cronbach's alpha >0.8. CONCLUSION: In Algerian patients with systemic sclerosis, Arab Hand Function Index and Arabic Health Assessment Questionnaire have a good construct validity and reliability. CI - © The Author(s) 2024. FAU - Benmostefa, Nouria AU - Benmostefa N AUID- ORCID: 0000-0002-5787-4986 AD - Department of Internal Medicine, Setif University Hospital, Setif, Algeria. AD - Faculty of Medicine, Ferhat Abbas Setif University 1, Setif, Algeria. AD - Department of Internal Medicine, Centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares d'Ile de France, de l'Est et de l'Ouest, Assistance Publique-Hôpitaux de Paris-Centre (APHP-CUP), Université Paris Cité, Paris, France. FAU - Malek, Rachid AU - Malek R AD - Department of Internal Medicine, Setif University Hospital, Setif, Algeria. AD - Faculty of Medicine, Ferhat Abbas Setif University 1, Setif, Algeria. FAU - Robert, Marie AU - Robert M AD - Department of Internal Medicine, Centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares d'Ile de France, de l'Est et de l'Ouest, Assistance Publique-Hôpitaux de Paris-Centre (APHP-CUP), Université Paris Cité, Paris, France. FAU - Chaigne, Benjamin AU - Chaigne B AD - Department of Internal Medicine, Centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares d'Ile de France, de l'Est et de l'Ouest, Assistance Publique-Hôpitaux de Paris-Centre (APHP-CUP), Université Paris Cité, Paris, France. FAU - Slimani, Samy AU - Slimani S AD - Department of Orthopedics, Batna University Hospital, Batna, Algeria. AD - Faculty of Medicine, Batna University 2, Batna, Algeria. FAU - Rouabhia, Samir AU - Rouabhia S AD - Faculty of Medicine, Batna University 2, Batna, Algeria. AD - Department of Internal Medicine, Batna University Hospital, Batna, Algeria. FAU - Roula, Daoud AU - Roula D AD - Department of Internal Medicine, Constantine University Hospital, Constantine, Algeria. AD - Faculty of Medicine, Salah Boubnider Constantine University 3, Constantine, Algeria. FAU - Djamel, Mallem AU - Djamel M AD - Faculty of Medicine, Batna University 2, Batna, Algeria. AD - Department of Internal Medicine, Batna University Hospital, Batna, Algeria. FAU - Mouthon, Luc AU - Mouthon L AD - Department of Internal Medicine, Centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares d'Ile de France, de l'Est et de l'Ouest, Assistance Publique-Hôpitaux de Paris-Centre (APHP-CUP), Université Paris Cité, Paris, France. LA - eng PT - Journal Article DEP - 20240228 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC11188850 OTO - NOTNLM OT - Arab Hand Function Index OT - Systemic sclerosis OT - construct validity OT - disability OT - hand OT - health assessment OT - outcome measures COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: N.B. received fees from El Kendi. L.M. has received grants and consultancy fees from Boehringer Ingelheim. R.M., M.R., B.C., S.S., S.R., M.D., and D.R. declare that there are no competing interests. EDAT- 2024/06/24 06:41 MHDA- 2024/06/24 06:42 PMCR- 2025/06/01 CRDT- 2024/06/24 05:14 PHST- 2023/11/02 00:00 [received] PHST- 2024/01/21 00:00 [accepted] PHST- 2024/06/24 06:42 [medline] PHST- 2024/06/24 06:41 [pubmed] PHST- 2024/06/24 05:14 [entrez] PHST- 2025/06/01 00:00 [pmc-release] AID - 10.1177_23971983241231082 [pii] AID - 10.1177/23971983241231082 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2024 Jun;9(2):134-142. doi: 10.1177/23971983241231082. Epub 2024 Feb 28. PMID- 41412094 OWN - NLM STAT- MEDLINE DCOM- 20260110 LR - 20260114 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 76 DP - 2026 Feb TI - Juvenile-onset mixed connective tissue disease: A multicenter retrospective cohort study. PG - 152889 LID - S0049-0172(25)00260-4 [pii] LID - 10.1016/j.semarthrit.2025.152889 [doi] AB - OBJECTIVES: Juvenile-onset mixed connective tissue disease (jMCTD) accounts for 7-23 % of MCTD cases but remains poorly described. We aimed to characterize clinical features, treatments, and outcomes of patients with jMCTD, and compare them to adult-onset MCTD (aMCTD) patients. METHODS: We conducted a multicenter, retrospective, case-control study within the French MCTD cohort. Each jMCTD patient was compared to 3 matched aMCTD patients. RESULTS: Forty-seven jMCTD patients (93.6 % girls; median age at onset 14 [11-16] years) were included. Forty-four (93.6 %) jMCTD patients fulfilled either Sharp or Kasukawa diagnostic criteria. None of them met other diagnostic criteria without fulfilling Sharp or Kasukawa criteria. At diagnosis, jMCTD patients' main manifestations were Raynaud's phenomenon, arthralgia, and myalgia. jMCTD patients had less frequently puffy fingers than aMCTD (p < 0.0001). Cumulatively, jMCTD patients mainly received glucocorticoids (80.9 %), hydroxychloroquine (95.7 %) and immunosuppressants (93.6 %). They received a higher initial dose of glucocorticoids (30 [20-60] mg/day vs. 15 [10-35] mg/day, p = 0.02), and significantly more frequently methotrexate (Methotrexate) and rituximab (p = 0.01) over time compared to aMCTD. After a median follow-up of 9.8 [6.6-16.2] years, 29 (61.7 %) jMCTD patients were in remission (vs. 62 (44.0 %) aMCTD; p < 0.05), 36 % had progressed to another CTD (vs. 30.5 % aMCTD; p = 0.5), mainly systemic lupus erythematosus, 11 (23.4 %) had developed interstitial lung disease, 2 (4.3 %) pulmonary arterial hypertension, and 1 (2.1 %) died. CONCLUSIONS: jMCTD share the same clinical characteristics as aMCTD patients, but less frequently have puffy fingers. Outcomes appear more favorable in jMCTD than aMCTD, with higher remission rates, albeit at the cost of more intensive treatment. CI - Copyright © 2025. Published by Elsevier Inc. FAU - Chevalier, Kevin AU - Chevalier K AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Bader-Meunier, Brigitte AU - Bader-Meunier B AD - Department for Immunology, Hematology and Pediatric Rheumatology, Necker Hospital, APHP, Institut IMAGINE, Paris, France. FAU - Kone-Paut, Isabelle AU - Kone-Paut I AD - Department of Paediatric Rheumatology, and CEREMAIA, Bicêtre University Hospital, APHP, university of Paris Saclay Le Kremlin-Bicêtre. FAU - Thoreau, Benjamin AU - Thoreau B AD - Department of Internal Medicine and Clinical immunology, CHRU de Tours, Tours, France. FAU - Michel, Marc AU - Michel M AD - Department of Internal Medicine, Henri-Mondor University Hospital, APHP, Université Paris Est Créteil (UPEC), Créteil, France. FAU - Godeau, Bertrand AU - Godeau B AD - Department of Internal Medicine, Henri-Mondor University Hospital, APHP, Université Paris Est Créteil (UPEC), Créteil, France. FAU - Agard, Christian AU - Agard C AD - Department of Internal Medicine, Nantes Université, CHU Nantes, Service de médecine interne, F-44000 Nantes, France. FAU - Papo, Thomas AU - Papo T AD - Department of Internal Medicine, Hôpital Bichat-Claude Bernard, APHP, Université Paris Cité. FAU - Sacre, Karim AU - Sacre K AD - Department of Internal Medicine, Hôpital Bichat-Claude Bernard, APHP, Université Paris Cité. FAU - Seror, Raphaèle AU - Seror R AD - Department of Rheumatology, National Reference Center for Rare Systemic Autoimmune, AP-HP, Hôpital Bicêtre, Université Paris Saclay, Le Kremlin-Bicêtre, France; Centre d'etude en santé des population (CESP) clinical epidemiology team, INSERM UMR1018, Le Kremlin-Bicêtre. FAU - Mariette, Xavier AU - Mariette X AD - Department of Rheumatology, National Reference Center for Rare Systemic Autoimmune, AP-HP, Hôpital Bicêtre, Université Paris Saclay, Le Kremlin-Bicêtre, France; Center for Immunology of Viral Infections and Auto-immune Diseases (IMVA), INSERM UMR1184, Le Kremlin-Bicêtre. FAU - Cacoub, Patrice AU - Cacoub P AD - Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Université Paris Sorbonne, Paris. FAU - Benhamou, Ygal AU - Benhamou Y AD - Department of Internal Medicine, CHU de Rouen, UniRouen, Rouen, France. FAU - Leclercq, Mathilde AU - Leclercq M AD - Department of Internal Medicine, CHU de Rouen, UniRouen, Rouen, France. FAU - Goujard, Cécile AU - Goujard C AD - Université Paris Saclay, Department of Internal Medicine and clinical immunology, Bicêtre hospital, APHP, Le Kremlin Bicêtre. FAU - Lambotte, Olivier AU - Lambotte O AD - Université Paris Saclay, Department of Internal Medicine and clinical immunology, Bicêtre hospital, APHP, Le Kremlin Bicêtre; Université Paris-Saclay, Inserm, CEA, Center for Immunology of Viral, Auto-immune, Hematological, Bacterial Diseases (IMVA-HB/IDMIT/UMRS1184), Le Kremlin Bicêtre, France. FAU - Bonnotte, Bernard AU - Bonnotte B AD - Université Paris-Saclay, Inserm, CEA, Center for Immunology of Viral, Auto-immune, Hematological, Bacterial Diseases (IMVA-HB/IDMIT/UMRS1184), Le Kremlin Bicêtre, France. FAU - Samson, Maxime AU - Samson M AD - Université Paris-Saclay, Inserm, CEA, Center for Immunology of Viral, Auto-immune, Hematological, Bacterial Diseases (IMVA-HB/IDMIT/UMRS1184), Le Kremlin Bicêtre, France. FAU - Ackermann, Félix AU - Ackermann F AD - Department of Internal Medicine and Clinical Immunology, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Dijon University Hospital, Dijon, France. FAU - Schmidt, Jean AU - Schmidt J AD - Department of Internal Medicine, Foch Hospital, Suresnes, France. FAU - Duhaut, Pierre AU - Duhaut P AD - Department of Internal Medicine, Foch Hospital, Suresnes, France. FAU - Kahn, Jean-Emmanuel AU - Kahn JE AD - Department of Internal Medicine, Ambroise Paré Hospital, APHP, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France. FAU - Hanslik, Thomas AU - Hanslik T AD - Department of Internal Medicine, Ambroise Paré Hospital, APHP, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France. FAU - Costedoat-Chalumeau, Nathalie AU - Costedoat-Chalumeau N AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Terrier, Benjamin AU - Terrier B AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Regent, Alexis AU - Regent A AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Dunogue, Bertrand AU - Dunogue B AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Cohen, Pascal AU - Cohen P AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France; Department of Internal Medicine and RECIF, Amiens University Hospital, Université Picardie Jules Verne, Amiens, France. FAU - Guern, Véronique LE AU - Guern VL AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Hachulla, Eric AU - Hachulla E AD - Department of Internal Medicine and Clinical immunology, Referral Centre for Rare Systemic Auto-immune and auto-inflammatory Diseases North-West Mediterranean and Guadeloupe (CeRAINOM), Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE, University of Lille, F-59037 Lille, France. FAU - Mouthon, Luc AU - Mouthon L AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. FAU - Chaigne, Benjamin AU - Chaigne B AD - Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases and Autoinflammatory Diseases of Ile de France, East and West, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Cité, Paris, France. Electronic address: benjamin.chaigne@aphp.fr. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20251211 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - 0 (Antirheumatic Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Humans MH - *Mixed Connective Tissue Disease/drug therapy/diagnosis MH - Female MH - Retrospective Studies MH - Male MH - Adolescent MH - Child MH - Case-Control Studies MH - Glucocorticoids/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Age of Onset MH - Antirheumatic Agents/therapeutic use MH - Hydroxychloroquine/therapeutic use MH - Adult MH - Treatment Outcome OTO - NOTNLM OT - Adult onset OT - Classification OT - Juvenile onset OT - Mixed connective tissue disease OT - Outcome OT - Prognosis OT - Sharp syndrome OT - Treatments COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/12/19 01:45 MHDA- 2026/01/11 00:34 CRDT- 2025/12/18 18:19 PHST- 2025/08/26 00:00 [received] PHST- 2025/11/08 00:00 [revised] PHST- 2025/12/03 00:00 [accepted] PHST- 2026/01/11 00:34 [medline] PHST- 2025/12/19 01:45 [pubmed] PHST- 2025/12/18 18:19 [entrez] AID - S0049-0172(25)00260-4 [pii] AID - 10.1016/j.semarthrit.2025.152889 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2026 Feb;76:152889. doi: 10.1016/j.semarthrit.2025.152889. Epub 2025 Dec 11. PMID- 39019570 OWN - NLM STAT- MEDLINE DCOM- 20241021 LR - 20250305 IS - 1468-2060 (Electronic) IS - 0003-4967 (Print) IS - 0003-4967 (Linking) VI - 83 IP - 11 DP - 2024 Oct 21 TI - Racial variability in immune responses only partially explains differential systemic sclerosis disease severity. PG - 1513-1521 LID - 10.1136/ard-2023-225458 [doi] AB - OBJECTIVE: To understand if autoantibodies account for racial variation in disease severity, we compared autoantibody distribution and associated phenotype between self-identified black and white systemic sclerosis (SSc) patients. METHODS: 803 black and 2178 white SSc patients had systematic testing for autoantibodies using Euroimmun (centromere (ACA), RNA-polymerase III (POLR3), Scl70, PM/Scl, NOR90, Th/To, Ku, U3RNP and Ro52) and commercial ELISA (U1RNP). In this observational study, logistic regression was performed to assess the association between self-identified race and outcomes, adjusting for autoantibodies. To estimate whether the effect of race was mediated by autoantibody status, race coefficients from multivariate models including and excluding autoantibodies were compared. RESULTS: Anti-Scl70, anti-U1RNP, anti-U3RNP, anti-Th/To, anti-Ku and anti-NOR90 were more common in the black cohort than in the white cohort, which was enriched for ACA, anti-POLR3 and anti-PM/Scl. Black individuals had a higher prevalence of severe Raynaud's, skin, lung, gastrointestinal and renal disease whereas white individuals had a higher prevalence of severe heart and muscle disease. Adjusting for autoantibodies decreased the effect of race on outcome for telangiectasias, forced vital capacity <70%, pulmonary hypertension and severe lung, heart, muscle and gastrointestinal disease by 11%-44% and increased the association between race and renal crisis and severe kidney disease by 37%-52%. CONCLUSIONS: This study is the largest systematic analysis of autoantibody responses in a geographically diverse population of black SSc patients. Black and white individuals with SSc have distinct autoantibody profiles. Autoantibodies explain only a fraction of the effect of race on clinical outcomes, suggesting other factors contribute to disparate outcomes between these groups. CI - © Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ on behalf of EULAR. FAU - Kuchinad, Kamini E AU - Kuchinad KE AUID- ORCID: 0000-0001-8384-7634 AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Kim, Ji Soo AU - Kim JS AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Woods, Adrianne AU - Woods A AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Leatherman, Gwen AU - Leatherman G AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Gutierrez-Alamillo, Laura AU - Gutierrez-Alamillo L AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Mayes, Maureen D AU - Mayes MD AD - Division of Rheumatology and Clinical Immunogenetics, The University of Texas Health Science Center John P and Katherine G McGovern Medical School, Houston, Texas, USA. FAU - Domsic, Robyn AU - Domsic R AUID- ORCID: 0000-0002-2765-0922 AD - Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. FAU - Ramos, Paula S AU - Ramos PS AUID- ORCID: 0000-0002-9433-6634 AD - Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina, USA. FAU - Silver, Richard M AU - Silver RM AUID- ORCID: 0000-0002-2038-3278 AD - Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, South Carolina, USA. FAU - Varga, John AU - Varga J AD - Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Tulane University School of Medicine, New Orleans, Louisiana, USA. AD - Section of Pulmonary Medicine, University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, Louisiana State University School of Medicine, New Orleans, Louisiana, USA. FAU - Kafaja, Suzanne AU - Kafaja S AD - Internal Medicine/Division of Rheumatology, University of California Los Angeles, Los Angeles, California, USA. FAU - Shanmugan, Victoria K AU - Shanmugan VK AD - Office of Autoimmune Disease Research, National Institute of Health, Bethesda, Maryland, USA. FAU - Gordon, Jessica AU - Gordon J AD - Department of Rheumatology, Department of Medicine, Hospital for Special Surgery, New York, New York, USA. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, California, USA. FAU - Bernstein, Elana J AU - Bernstein EJ AUID- ORCID: 0000-0001-5560-6390 AD - Division of Rheumatology, Columbia University Vagelos College of Physicians and Surgeons, New York, New York, USA. FAU - Gourh, Pravitt AU - Gourh P AD - NIAMS, National Institutes of Health, Bethesda, Maryland, USA. FAU - Boin, Francesco AU - Boin F AD - Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA. FAU - Kastner, Daniel L AU - Kastner DL AUID- ORCID: 0000-0001-7188-4550 AD - National Human Genome Research Institute Division of Intramural Research, Bethesda, Maryland, USA. FAU - Zeger, Scott L AU - Zeger SL AD - Biostatistics, Johns Hopkins University, Baltimore, Maryland, USA. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AUID- ORCID: 0000-0002-4172-1539 AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Wigley, Fredrick M AU - Wigley FM AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Shah, Ami A AU - Shah AA AUID- ORCID: 0000-0002-1139-2009 AD - Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA Ami.Shah@jhmi.edu. LA - eng GR - K24 AR080217/AR/NIAMS NIH HHS/United States GR - R01 AR073208/AR/NIAMS NIH HHS/United States GR - T32 AR048522/AR/NIAMS NIH HHS/United States GR - P30 AR072582/AR/NIAMS NIH HHS/United States GR - K23 AR075112/AR/NIAMS NIH HHS/United States GR - P30 AR070254/AR/NIAMS NIH HHS/United States GR - R01 HL164758/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Observational Study DEP - 20241021 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Autoantibodies/blood/immunology MH - Black or African American MH - *Scleroderma, Systemic/immunology/ethnology MH - *Severity of Illness Index MH - White PMC - PMC11875133 MID - NIHMS2013119 OTO - NOTNLM OT - Autoantibodies OT - Epidemiology OT - Risk Factors OT - Systemic Sclerosis COIS- Competing interests: RD received consulting fees from CSL Behring and Astra-Zeneca. LAS received grant funding from Horizon Pharmaceuticals, aTyr Pharmaceuticals and Kinevant Pharmaceuticals and honoraria from Janssen Pharmaceuticals. She served on the data safety monitoring board for Argenx Pharmaceuticals. LC received grant funding from Boehringer Ingelheim and consulting fees from Kyverna, Eicos Sciences, Genentech, IgM Biosciences, Lilly. She participated in an advisory capacity for Mitsubishi Tanabe and Janssen. FB received honoraria from Janssen. RMS received grant funding from Boehringer Ingelheim, Merck and Amgen; he is the CEO of FibroBiologics. JV received consulting fees from TeneoBio, and Conquest; he serves on the data safety monitoring board for Conquest. MDM received grant funding from Prometheus Biosciences, Mitsubishi Tanabe, Boehringer Ingelheiem, Eicos, Corbus and Horizon Pharmaceuticals. She received consulting fees from Cabaletta Pharmaceuticals; she served on the advisory board for Mitsubishi Tanabe and Eicos Sciences. AS has grant support from Kadmon, Arena Pharmaceuticals, Medpace and Eicos Sciences. EDAT- 2024/07/18 00:42 MHDA- 2024/10/22 00:21 PMCR- 2025/03/03 CRDT- 2024/07/17 21:13 PHST- 2024/01/02 00:00 [received] PHST- 2024/06/19 00:00 [accepted] PHST- 2024/10/22 00:21 [medline] PHST- 2024/07/18 00:42 [pubmed] PHST- 2024/07/17 21:13 [entrez] PHST- 2025/03/03 00:00 [pmc-release] AID - S0003-4967(24)66544-1 [pii] AID - 10.1136/ard-2023-225458 [doi] PST - epublish SO - Ann Rheum Dis. 2024 Oct 21;83(11):1513-1521. doi: 10.1136/ard-2023-225458. PMID- 38265968 OWN - NLM STAT- MEDLINE DCOM- 20240126 LR - 20240206 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 4 IP - 10 DP - 2022 Oct TI - Sex-specific risk of anti-topoisomerase antibodies on mortality and disease severity in systemic sclerosis: 10-year analysis of the Leiden CCISS and EUSTAR cohorts. PG - e699-e709 LID - S2665-9913(22)00224-7 [pii] LID - 10.1016/S2665-9913(22)00224-7 [doi] AB - BACKGROUND: We aimed to evaluate sex-specific risk of anti-topoisomerase I antibodies (ATA) on mortality, diffuse cutaneous systemic sclerosis, interstitial lung disease, and pulmonary hypertension in two cohorts of people with systemic sclerosis. METHODS: This study was a 10-year analysis of the prospective Leiden Combined Care in Systemic Sclerosis (CCISS) cohort in the Netherlands and the international European Scleroderma Trials and Research (EUSTAR) cohort. We included participants with systemic sclerosis according to the 2013 American College of Rheumatology-European League Against Rheumatism (ACR-EULAR) classification criteria; available autoantibody status; available skin subtyping; at least one available radiographic assessment of interstitial lung disease; and with a known date of disease onset. People with systemic sclerosis were categorised in six risk groups by sex and autoantibody status (anti-centromere antibody [ACA]-positive female, ACA-positive male, ACA and ATA-negative female, ACA and ATA-negative male, ATA-positive female, and ATA-positive male). We constructed Kaplan-Meier curves and Cox proportional hazard models, accounting for left-truncated survival to prevent bias because the date of disease onset (first non-Raynaud's symptom) preceded the date of cohort entry for all patients. The primary outcome was all-cause mortality and the secondary outcomes were diffuse cutaneous systemic sclerosis, interstitial lung disease, and pulmonary hypertension. FINDINGS: 445 (63%) of 708 participants between April 1, 2009, and Jan 1, 2022, in CCISS (101 [23%] male and 344 [77%] female) and 4263 (50%) of 8590 between June 1, 2004, and March 28, 2018, in EUSTAR (783 [18%] male and 3480 [82%] female) were eligible for this study. In both cohorts, ATA expression occurred significantly more often in males than in females (39 [39%] of 101 males vs 67 [19%] of 344 females in CCISS; p<0·0001 and 381 [49%] of 783 males vs 1323 [38%] of 3480 females in EUSTAR; p<0·0001). According to estimated survival rates, 30% of ATA-positive males versus 12% of ATA-positive females died in the CCISS cohort and 33% versus 15% died in the EUSTAR cohort within 10 years. After adjustment for age, race, and autoantibody status, male sex remained the most important risk factor for all-cause mortality (HR 2·9 [95% CI 1·5-5·5] in CCISS, p=0·0018; and HR 2·6 [2·0-3·4] in EUSTAR, p<0·0001). INTERPRETATION: We show that the association between male sex and increased mortality in systemic sclerosis cannot be explained by higher ATA prevalence. However, additional research on the effect of sex-specific characteristics on people with systemic sclerosis is required. FUNDING: None. CI - Copyright © 2022 Elsevier Ltd. All rights reserved. FAU - Liem, Sophie I E AU - Liem SIE AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. Electronic address: s.i.e.liem@lumc.nl. FAU - Boonstra, Maaike AU - Boonstra M AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. FAU - le Cessie, Saskia AU - le Cessie S AD - Department of Clinical Epidemiology, University Medical Center, Leiden, Netherlands; Department of Biomedical Data Sciences, University Medical Center, Leiden, Netherlands. FAU - Riccardi, Antonella AU - Riccardi A AD - Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Airo, Paolo AU - Airo P AD - Division of Rheumatology and Clinical Immunology, Civil Hospital Brescia, Brescia, Italy. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. FAU - Caimmi, Cristian AU - Caimmi C AD - Rheumatology Unit, University Verona, Verona, Italy. FAU - Siegert, Elise AU - Siegert E AD - Department of Rheumatology, Berlin Institute of Health, Berlin, Germany. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Cochin Hospital, Paris, France. FAU - Huizinga, Tom W J AU - Huizinga TWJ AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. FAU - Toes, René E M AU - Toes REM AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. FAU - Scherer, Hans U AU - Scherer HU AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Department of Rheumatology, University Medical Center, Leiden, Netherlands. CN - EUSTAR collaborators LA - eng PT - Journal Article PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 RN - 0 (Autoantibodies) RN - EC 5.- (Isomerases) SB - IM MH - Humans MH - Female MH - Male MH - *Scleroderma, Diffuse MH - *Hypertension, Pulmonary MH - Prospective Studies MH - *Scleroderma, Systemic MH - *Scleroderma, Localized MH - Autoantibodies MH - Patient Acuity MH - *Basidiomycota MH - Isomerases MH - *Lung Diseases, Interstitial COIS- Declaration of interests SIEL received a grant from ZonMw and Nationale vereniging voor mensen met lupus, APS, sclerodermie en MCTD to study physical therapy in primary care in systemic sclerosis. PA received consulting fees from Bristol Myers Squibb; payments or honoraria from Bristol Myers Squibb, Bohringer Ingelheim, and Novartis; and support for attending meetings and travel from CSL Behring, Janssen, Roche, and Bristol Myers Squibb. OD was a consultant, received research funding, or speaker fees, or both, from 4P-Pharma, 4P-Science, Abbvie, Acceleron, Alcimed, Altavant Sciences, Amgen, AnaMar, Arxx, AstraZeneca, Blade Therapeutics, Bayer, Boehringer Ingelheim Pharma, Corbus Pharmaceuticals, CSL Behring, Galapagos, Glenmark, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Miltenyi Biotec, Mitsubishi Tanabe Pharma, MSD, Novartis, Prometheus Biosciences, Pfizer, Redxpharma, Roivant, Sanofi, and Topadur. JKdV-B received research grants from Roche, Galapagos, and Janssen; and consulting fees from Abbvie, Janssen, and Boehringer Ingelheim. All other authors declare no competing interests. EDAT- 2022/10/01 00:00 MHDA- 2024/01/26 06:43 CRDT- 2024/01/24 13:03 PHST- 2022/04/29 00:00 [received] PHST- 2022/07/22 00:00 [revised] PHST- 2022/07/25 00:00 [accepted] PHST- 2024/01/26 06:43 [medline] PHST- 2022/10/01 00:00 [pubmed] PHST- 2024/01/24 13:03 [entrez] AID - S2665-9913(22)00224-7 [pii] AID - 10.1016/S2665-9913(22)00224-7 [doi] PST - ppublish SO - Lancet Rheumatol. 2022 Oct;4(10):e699-e709. doi: 10.1016/S2665-9913(22)00224-7. PMID- 29599028 OWN - NLM STAT- MEDLINE DCOM- 20181221 LR - 20220408 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 48 IP - 2 DP - 2018 Oct TI - Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency. PG - 318-326 LID - S0049-0172(17)30654-6 [pii] LID - 10.1016/j.semarthrit.2018.02.013 [doi] AB - Patients with common variable immunodeficiency (CVID) have a higher incidence of rheumatologic disorders. To delineate this clinical association, we investigated the phenotypic features of patients with CVID affected by these conditions. METHODS: We conducted a retrospective analysis of 870 pediatric and adult patients with CVID included in the United States Immunodeficiency Network (USIDNET) registry. Outcomes included clinical characteristics (age, gender, ethnicity, rheumatologic diagnosis, and comorbidities), infectious history and basic immunophenotype (serum immunoglobulin levels, CD19+ B cells, and CD4/CD8 ratio) in patients with CVID and rheumatologic disorders compared to those with non-inflammatory CVID. Demographic and clinical data were compared using chi-square, Fisher's exact or Wilcoxon-Mann-Whitney tests. Non-parametric tests, single and multiple logistic regression models were used to evaluate the relationship between CVID-associated rheumatologic disorders and basic immunophenotypic parameters (IgA, IgM, CD19+ B-cell counts, and CD4/CD8 ratios). RESULTS: Physician-reported rheumatic diseases were present in 5.9% of patients with CVID (n = 51) included in the registry. Although CVID affects both sexes equally, and patients are of predominantly White-Caucasian ethnicity, there were more females (3.3:1 female to male ratio) and increased proportion of non-white patients in the rheumatologic disease group (p < 0.05). Specific disorders included: inflammatory arthritis (n = 18), Sjogren's syndrome (n = 11), SLE (n = 8), Raynaud's syndrome (n = 8), vasculitis (n = 9), MCTD (n = 3), and other (n = 5). In about one-third of patients, a rheumatologic condition was associated with an additional inflammatory complication or malignancy. In regards to the immunophenotype parameters compared (CD19+ B-cell counts, CD4/CD8 ratio, IgA, and IgM), no significant differences were demonstrated between the two groups. CONCLUSION: Our findings highlight the coexistence of primary antibody immunodeficiencies and systemic rheumatologic disorders, describe the spectrum of rheumatologic manifestations, and contrast differences in relevant demographic, clinical and immunophenotype parameters in the largest registry of CVID patients in the U.S. In spite of its limitations, our study details the intersection of systemic autoimmunity and CVID and provides valuable insights into these two groups of disorders. Further delineating the link between systemic autoimmunity and humoral immunodeficiencies can provide novel insights into the immune abnormalities underlying these related conditions. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Gutierrez, Maria J AU - Gutierrez MJ AD - Division of Pediatric Allergy and Immunology, Johns Hopkins University, Baltimore, PA. Electronic address: mgutie10@jhmi.edu. FAU - Sullivan, Kathleen E AU - Sullivan KE AD - Division of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, PA. FAU - Fuleihan, Ramsay AU - Fuleihan R AD - Division of Allergy and Immunology, Ann & Robert H. Lurie Children's Hospital, Chicago, IL. CN - USIDNET Consortium FAU - Bingham, Clifton O 3rd AU - Bingham CO 3rd AD - Divisions of Rheumatology and Allergy and Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD. LA - eng GR - T32 AI007007/AI/NIAID NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20180223 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - *Autoimmunity MH - Child MH - Child, Preschool MH - Common Variable Immunodeficiency/*diagnosis/immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Phenotype MH - Registries MH - Retrospective Studies MH - Sex Factors MH - Symptom Assessment MH - Young Adult PMC - PMC6107427 MID - NIHMS954932 OTO - NOTNLM OT - Autoimmunity OT - CVID OT - Primary Antibody Deficiency OT - Rheumatologic Disease EDAT- 2018/03/31 06:00 MHDA- 2018/12/24 06:00 PMCR- 2019/10/01 CRDT- 2018/03/31 06:00 PHST- 2017/11/04 00:00 [received] PHST- 2018/01/31 00:00 [revised] PHST- 2018/02/20 00:00 [accepted] PHST- 2018/03/31 06:00 [pubmed] PHST- 2018/12/24 06:00 [medline] PHST- 2018/03/31 06:00 [entrez] PHST- 2019/10/01 00:00 [pmc-release] AID - S0049-0172(17)30654-6 [pii] AID - 10.1016/j.semarthrit.2018.02.013 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2018 Oct;48(2):318-326. doi: 10.1016/j.semarthrit.2018.02.013. Epub 2018 Feb 23. PMID- 27225012 OWN - NLM STAT- MEDLINE DCOM- 20170331 LR - 20191114 IS - 1098-2825 (Electronic) IS - 0887-8013 (Print) IS - 0887-8013 (Linking) VI - 30 IP - 5 DP - 2016 Sep TI - Comparison of Screening Dilution and Automated Reading for Antinuclear Antibody Detection on HEP2 Cells in the Monitoring of Connective Tissue Diseases. PG - 471-8 LID - 10.1002/jcla.21881 [doi] AB - BACKGROUND: Indirect immunofluorescence plays a major role in the detection of antinuclear antibodies (ANAs) and follow-up of their titers in the context of connective tissue diseases. Given the numerous unfavorable features of the conventional manual reading of HEP2 slides (need of time and expert morphologists for the reading, lack of standardization, subjectivity of the interpretation), the biomedical industry has developed automated techniques of slide preparation and microscope reading. METHODS: We collected 49 sera beforehand analyzed by the conventional reading of slides. They were prepared again by QUANTA-Lyser(®) and reanalyzed in four different conditions: two dilutions of screening (1/40 and 1/80), two different systems of analysis, NOVA View(®) automated reading (INOVA Diagnostics), then confirmation by the operator, and conventional manual reading by two different qualified operators. The analysis was realized in blind of the first interpretation and clinical diagnosis. The sera were classified in four groups, on the basis of the results of the first analysis: negative sera (titer < 1/160; 11 patients), low positives (titer at 1/160; 18 patients), moderated positives (titers between 1/320 and 1/640; 10 patients), and strong positives (titers between 1/1,280 and 1/2,560; 10 patients). RESULTS: Among the 49 patients, 13 presented a connective tissue disease including 4 systemic scleroderma (SS), 3 rheumatoid arthritis (RA), 2 Goujerot-Sjogren (GS), 2 systemic lupus erythematosus (SLE), 1 polymyositis (PM), 1 Raynaud's syndrome (RS), and 1 CREST syndrome. One patient presented both an SLE and an SS. Regarding the screening dilution, the 1/40 dilution is less specific than the 1/80 dilution for both the systems of analysis (5.6% vs. 16.7% for the manual reading, and 27.8% vs. 50% for the automated reading). It also generates statistically more false positives (P = 0.037 for the conventional analysis and P = 0.003 for the automated system). The automated NOVA View(®) reading of slides allows a gain in specificity for both dilutions, and also statistically less false positives (P = 0.002 at the 1/40 and P = 0.0006 at the 1/80), and detriment of the sensitivity at the highest dilution (84.6% vs. 92.3% with manual reading). Thus, according to our analysis of 49 sera, the automated NOVA View(®) system of reading of slides at the dilution 1/80 seems to be a successful condition for the detection of ANAs on HEP2 cells, close to the significance (P = 0.067). CONCLUSION: The automated NOVA View(®) reading of slides allows saving time, and an improvement in the standardization. Nevertheless, it requires a confirmation by a qualified operator, to interpret mixed patterns in particular. CI - © 2016 Wiley Periodicals, Inc. FAU - Depincé-Berger, Anne E AU - Depincé-Berger AE AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. FAU - Moreau, Amelie AU - Moreau A AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. FAU - Bossy, Virginie AU - Bossy V AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. FAU - Genin, Christian AU - Genin C AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. FAU - Rinaudo, Melanie AU - Rinaudo M AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. FAU - Paul, Stephane AU - Paul S AD - Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France. stephane.paul@chu-st-etienne.fr. LA - eng PT - Journal Article DEP - 20160526 PL - United States TA - J Clin Lab Anal JT - Journal of clinical laboratory analysis JID - 8801384 RN - 0 (Alkaloids) RN - 0 (Antibodies, Antinuclear) RN - 0 (Wilcox compound) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Alkaloids MH - Analysis of Variance MH - Antibodies, Antinuclear/*blood MH - Carcinoma/pathology MH - Cell Line, Tumor MH - Child MH - Connective Tissue Diseases/blood/*diagnosis MH - Electronic Data Processing/*methods MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - *Mass Screening MH - Middle Aged MH - ROC Curve MH - Retrospective Studies MH - Sensitivity and Specificity MH - Young Adult PMC - PMC6806706 OTO - NOTNLM OT - ANA OT - Connective tissue disease OT - HEP2 cells OT - IIF OT - NOVA View® automated reading of slides OT - conventional manual reading COIS- Mr. Boutin is employed at INOVA Diagnostics, the society that manufactures and markets the QUANTA‐Lyser(®) and NOVA View(®) EDAT- 2016/05/27 06:00 MHDA- 2017/04/01 06:00 PMCR- 2016/05/26 CRDT- 2016/05/27 06:00 PHST- 2015/02/06 00:00 [received] PHST- 2015/07/17 00:00 [accepted] PHST- 2016/05/27 06:00 [entrez] PHST- 2016/05/27 06:00 [pubmed] PHST- 2017/04/01 06:00 [medline] PHST- 2016/05/26 00:00 [pmc-release] AID - JCLA21881 [pii] AID - 10.1002/jcla.21881 [doi] PST - ppublish SO - J Clin Lab Anal. 2016 Sep;30(5):471-8. doi: 10.1002/jcla.21881. Epub 2016 May 26. PMID- 38733668 OWN - NLM STAT- MEDLINE DCOM- 20240627 LR - 20260327 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 67 DP - 2024 Aug TI - Immune checkpoint inhibitor therapy in patients with cancer and pre-existing systemic sclerosis. PG - 152460 LID - S0049-0172(24)00100-8 [pii] LID - 10.1016/j.semarthrit.2024.152460 [doi] AB - OBJECTIVE: Immune checkpoint inhibitor (ICI) therapies have dramatically improved outcomes in multiple cancers. ICI's mechanism of action involves immune system activation to augment anti-tumor immunity. Patients with pre-existing autoimmune diseases, such as systemic sclerosis (SSc), were excluded from initial ICI clinical trials due to concern that such immune system activation could precipitate an autoimmune disease flare or new, severe immune related adverse events (irAE). In the present study, we report our experience with ICIs in patients with pre-existing SSc. METHODS: Patients with SSc who received ICI therapy for cancer were identified from the Johns Hopkins Scleroderma Center Research Registry. Through chart review and prespecified definitions, we identified whether patients experienced worsening SSc activity or new irAEs. SSc disease activity worsening was pre-defined as an increase in modified Rodnan skin score (mRSS), new scleroderma renal crisis, progression of interstitial lung disease (ILD) on CT scan, increased Raynaud's phenomenon frequency or severity, new pulmonary hypertension, or myositis flare. IrAEs also included active inflammatory arthritis and dermatitis. RESULTS: Eight patients with SSc who received ICI therapy for cancer were included. Overall, SSc symptoms remained stable during and after ICI therapy. None of the patients with long-standing sine or limited cutaneous SSc (lcSSc) had progressive skin thickening after ICI therapy. One patient, who was early in his diffuse cutaneous SSc (dcSSc) disease course, experienced worsening skin thickening and renal crisis. Three patients (38 %) experienced a total of five irAEs (grade 2: diarrhea, mucositis and dermatitis; grade 3: pneumonitis, and grade 4: nephritis). The patient with grade 4 nephritis developed scleroderma renal crisis and immune checkpoint related nephritis simultaneously. There were no deaths due to irAEs. CONCLUSION: In this study, ICI therapy was well tolerated in patients with longstanding, sine or lcSSc. IrAE were common but generally manageable. Patients with early, active SSc may be at greater risk from ICI therapy, but more research is needed. CI - Copyright © 2024. Published by Elsevier Inc. FAU - Wallwork, Rachel S AU - Wallwork RS AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. FAU - Kotzin, Jonathan J AU - Kotzin JJ AD - Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, United States. FAU - Cappelli, Laura C AU - Cappelli LC AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. FAU - Mecoli, Christopher AU - Mecoli C AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. FAU - Bingham, Clifton O 3rd AU - Bingham CO 3rd AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. FAU - Wigley, Fredrick M AU - Wigley FM AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. FAU - Wilson, Parker C AU - Wilson PC AD - Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, United States. FAU - DiRenzo, Dana D AU - DiRenzo DD AD - Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, United States. FAU - Shah, Ami A AU - Shah AA AD - Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, United States. Electronic address: ami.shah@jhmi.edu. LA - eng GR - K24 AR080217/AR/NIAMS NIH HHS/United States GR - T32 AR076951/AR/NIAMS NIH HHS/United States GR - R01 AR073208/AR/NIAMS NIH HHS/United States GR - T32 AR048522/AR/NIAMS NIH HHS/United States GR - K23 AR075898/AR/NIAMS NIH HHS/United States GR - K23 AR075872/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20240504 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Immune Checkpoint Inhibitors) SB - IM MH - Humans MH - *Immune Checkpoint Inhibitors/adverse effects/therapeutic use MH - *Scleroderma, Systemic/drug therapy/complications/immunology MH - Female MH - Male MH - Middle Aged MH - *Neoplasms/drug therapy/complications MH - Aged MH - Adult MH - Treatment Outcome MH - Disease Progression PMC - PMC11211049 MID - NIHMS1993682 OTO - NOTNLM OT - Cancer OT - Immune checkpoint inhibitors OT - Immune related adverse events OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Ami Shah reports financial support was provided by National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health. Christopher Mecoli reports financial support was provided by National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health. Laura Cappelli reports was provided by National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health. Rachel Wallwork reports financial support was provided by National Institute of Arthritis and Musculoskeletal and Skin Diseases at the National Institutes of Health. Rachel Wallwork reports financial support was provided by The Jerome L Greene Foundation. Ami Shah reports financial support was provided by The Donald B. and Dorothy L. Stabler Foundation. Rachel Wallwork reports financial support was provided by The Rheumatology Research Foundation. Clifton Bingham reports a relationship with Bristol Myers Squibb that includes: consulting or advisory and funding grants. Laura Cappelli reports a relationship with Bristol Myers Squibb that includes: consulting or advisory and funding grants. Laura Cappelli reports a relationship with Amgen that includes: consulting or advisory. Ami Shah reports a relationship with Kadmon Corporation that includes: funding grants. Ami Shah reports a relationship with Arena Pharmaceuticals that includes: funding grants. Ami Shah reports a relationship with Medpace LLC that includes: funding grants. Ami Shah reports a relationship with Eicos Sciences that includes: funding grants. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/05/12 00:44 MHDA- 2024/06/28 00:42 PMCR- 2025/08/01 CRDT- 2024/05/11 18:03 PHST- 2023/12/20 00:00 [received] PHST- 2024/04/02 00:00 [revised] PHST- 2024/04/11 00:00 [accepted] PHST- 2024/06/28 00:42 [medline] PHST- 2024/05/12 00:44 [pubmed] PHST- 2024/05/11 18:03 [entrez] PHST- 2025/08/01 00:00 [pmc-release] AID - S0049-0172(24)00100-8 [pii] AID - 10.1016/j.semarthrit.2024.152460 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2024 Aug;67:152460. doi: 10.1016/j.semarthrit.2024.152460. Epub 2024 May 4. PMID- 36254548 OWN - NLM STAT- MEDLINE DCOM- 20221019 LR - 20221019 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 30 IP - 2 DP - 2022 Sep TI - Atypical Scleroderma-like Chronic GVHD in a Liver Transplant Patient, Clinical and MRI Correlations. PG - 123-125 AB - Dear Editor, cutaneous chronic graft versus host disease (cGVHD) is a pathological process consisting of donor-derived T-cells aimed at the antigens of the recipient. It exhibits a large range of clinical presentations resembling morphea and deep sclerosis/fasciitis, all characterized by both inflammation and progressive dermal and hypodermic fibrosis (1). Although classic scleroderma-like lesions in cGVHD are nummular or irregular plaques and linear bundles associated with hypo- or hyperpigmentation (2), we report an atypical case with ulcerative presentation. No other case-reports of morphea-like or scleroderma-like cGVHD with an ulcerated appearance after liver transplantation (LT) and magnetic resonance imaging (MRI) correlation have been found in the literature. CASE REPORT Ten months after LT due to an end-stage cirrhosis associated with multifocal hepatocarcinoma (HCC), a 61-year-old man on immunosuppressive therapy with Tacrolimus (1 mg) and Everolimus (10 mg) presented to our clinic for a skin lesion in the right scapular region. We observed a flat ulcerated plaque with areas of sclerosis, minimal necrosis, and well-defined slightly erythematous margins (Figure 1, a). On palpation, the plaque had a hard consistency and was slightly painful. The skin lesion had been preceded by subjective discomfort with stinging sensation for seven months before its onset. Gradually lesion developed starting from a small, flat, oval purplish plaque associated with a progressive increase in pain. Patient denied dysphagia, retrosternal heartburn, Raynaud's phenomenon, arthralgia, and dyspnea. A previous MRI (Figure 2, a,b) showed subcutaneous and muscle edema. Blood tests showed abnormal liver function indexes due to extrahepatic cholestasis, while C-reactive protein, erythrocyte sedimentation rate, and leukocytes were within normal ranges. Self-reactive antibodies were negative. Histological examination (Figure 1, b) identified rare dyskeratotic keratinocytes and basal lymphocyte infiltrate, a dermal dense fibrosis with the disappearance of the skin appendages, and large fibrous septa in the adipose panniculus. It led to the diagnosis of scleroderma/morphea, based on the patient's clinical history. The diagnosis of graft versus host disease scleroderma-like post liver transplant was established. The lesion was treated by topical application of 0.05% clobetasol once a day. We did not use systemic immunosuppressive therapy in order to prevent HCC recurrence. The patient is currently in clinical follow-up to identify worsening or neoplastic degeneration. CASE DISCUSSION Cutaneous cGVHD often presents clinically as an ulcerative evolution in the context of fibrosis and diffuse skin atrophy (2), but very rarely initially appears as a well-delimited ulcerated plaque. Only few cases of ulcer have been found in literature, all in patients undergoing hematopoietic stem cell transplantation (HSCT), which is associated with the highest risk of developing GVHD, 20-50% (3,4), while LT has quite low incidence, at 0.5-2% (5). To our knowledge, this is the first case report of a scleroderma-like cGVHD lesion with ulcer appearance in LT. Our patient underwent two MRIs during post-transplant follow-up, which allowed us to evaluate the deep disease evolution. The T2-weighted MRI (Figure 2, c,d) performed approximately 1 year after transplantation, demonstrated fibrous septa in the subcutaneous fat and fascial thickening, with associated muscle hypotrophy and edema. The previous MRI, performed seven months after transplantation, already showed subcutaneous tissues and fascial edema, highlighting active inflammation. This evidence suggests that MRI could identify the lesion location before clinical manifestations, providing an opportunity to intervene promptly. To the best of our knowledge, this is the first reported case of cGVHD with atypical scleroderma-like presentation in a liver transplant patient whose clinical and MRI correlations have been traced. Our suggestions are supported by the results of other previous studies (6,7) evaluating MRI performance for assessing disease extent and activity, as well as therapeutic response in HSCT. FAU - Giannoni, Melania AU - Giannoni M FAU - Rizzetto, Giulio AU - Rizzetto G FAU - Diotallevi, Federico AU - Diotallevi F FAU - Molinelli, Elisa AU - Molinelli E FAU - Radi, Giulia AU - Radi G FAU - Campanati, Anna AU - Campanati A AD - Prof. A. Campanati, Dermatology Unit, Department of Clinical and Molecular Sciences,, Polytechnic Marche University, Ancona, Italy; anna.campanati@gmail.com; a.campanati@staff.univpm.it. FAU - Brancorsni, Donatella AU - Brancorsni D FAU - Offidani, Annamaria AU - Offidani A LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 RN - 9007-41-4 (C-Reactive Protein) RN - 9HW64Q8G6G (Everolimus) RN - ADN79D536H (Clobetasol) RN - WM0HAQ4WNM (Tacrolimus) SB - IM MH - C-Reactive Protein MH - Clobetasol MH - Everolimus MH - Fibrosis MH - *Graft vs Host Disease/complications MH - Humans MH - Inflammation/complications MH - *Liver Transplantation/adverse effects MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - *Scleroderma, Localized/diagnosis MH - Sclerosis MH - *Skin Diseases/complications MH - Tacrolimus/therapeutic use MH - Ulcer/complications EDAT- 2022/10/19 06:00 MHDA- 2022/10/20 06:00 CRDT- 2022/10/18 03:22 PHST- 2022/10/18 03:22 [entrez] PHST- 2022/10/19 06:00 [pubmed] PHST- 2022/10/20 06:00 [medline] PST - ppublish SO - Acta Dermatovenerol Croat. 2022 Sep;30(2):123-125. PMID- 39243744 OWN - NLM STAT- MEDLINE DCOM- 20241104 LR - 20260406 IS - 1873-5835 (Electronic) IS - 0145-2126 (Linking) VI - 146 DP - 2024 Nov TI - Association between myeloid disorders and adult onset-inflammatory syndromes, successful treatment with JAK-inhibitors: Case series and literature review. PG - 107584 LID - S0145-2126(24)00150-4 [pii] LID - 10.1016/j.leukres.2024.107584 [doi] AB - Approximately one-third of patients with myeloid disorders like myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) exhibit inflammatory and autoimmune disorders (IADs). These IADs often include atypical and incomplete forms of common autoimmune conditions, and exhibit resistance to conventional immunosuppressive therapies. There is growing interest in molecular relationships between IADs and MDS/CMML to find potential targeted therapies. Recently, patients with somatic mutations in the UBA1 gene were identified as having VEXAS syndrome. Herein, we present a concise case-series illustrating concurrent elderly-onset inflammatory manifestations and myeloid disorders (MDS, CMML, and idiopathic cytopenia of undetermined significance). These patients manifested inflammatory or autoimmune symptoms, including erythema nodosum, Raynaud's phenomenon, Sjogren syndrome, and refractory pruritus, having onset after 60-years of age. The inflammatory manifestations were largely refractory to traditional immunosuppressive regimens. Remarkably, treatment with a JAK-1 inhibitor, upadacitinib, in two cases yielded marked resolution of inflammatory symptoms, facilitating the gradual tapering of corticosteroids, improvement of hemoglobin levels, and reduction in serum C-reactive protein levels. Upon loss of response to upadacitinib, JAK-2 inhibitor ruxolitinib provided clinical benefit in one of the cases, facilitating further tapering of glucocorticoids. This arena warrants further exploration through prospective studies of larger cohorts to delineate optimal management strategies. CI - Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Mishra, Rahul AU - Mishra R AD - Department of Internal Medicine, Anne Arundel Medical Center, Annapolis, MD, United States. FAU - Calabrese, Cassandra AU - Calabrese C AD - Department of Rheumatologic and Immunologic Disease, Cleveland Clinic, Cleveland, OH, United States. FAU - Jain, Akriti G AU - Jain AG AD - Leukemia and Myeloid Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, United States. FAU - Singh, Abhay AU - Singh A AD - Leukemia and Myeloid Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, United States. Electronic address: Singha21@ccf.org. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20240904 PL - England TA - Leuk Res JT - Leukemia research JID - 7706787 RN - 0 (Janus Kinase Inhibitors) RN - 0 (UBA1 protein, human) RN - EC 6.2.1.45 (Ubiquitin-Activating Enzymes) SB - IM MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Male MH - Middle Aged MH - Autoimmune Diseases/drug therapy MH - Inflammation/drug therapy/pathology MH - *Janus Kinase Inhibitors/therapeutic use MH - Leukemia, Myelomonocytic, Chronic/drug therapy/complications/genetics MH - *Myelodysplastic Syndromes/drug therapy MH - Ubiquitin-Activating Enzymes/genetics/antagonists & inhibitors OTO - NOTNLM OT - Hemato-inflammatory syndromes OT - JAK inhibitors OT - Myeloid neoplasm OT - Upadacitinib OT - Vexas phenotypes COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/09/08 13:51 MHDA- 2024/11/04 06:23 CRDT- 2024/09/07 18:10 PHST- 2024/07/09 00:00 [received] PHST- 2024/08/29 00:00 [revised] PHST- 2024/09/03 00:00 [accepted] PHST- 2024/11/04 06:23 [medline] PHST- 2024/09/08 13:51 [pubmed] PHST- 2024/09/07 18:10 [entrez] AID - S0145-2126(24)00150-4 [pii] AID - 10.1016/j.leukres.2024.107584 [doi] PST - ppublish SO - Leuk Res. 2024 Nov;146:107584. doi: 10.1016/j.leukres.2024.107584. Epub 2024 Sep 4. PMID- 29844404 OWN - NLM STAT- MEDLINE DCOM- 20191210 LR - 20200306 IS - 2041-4889 (Electronic) VI - 9 IP - 6 DP - 2018 May 29 TI - DSGOST regulates resistance via activation of autophagy in gastric cancer. PG - 649 LID - 10.1038/s41419-018-0658-y [doi] LID - 649 AB - Danggui-Sayuk-Ga-Osuyu-Saenggang-Tang (DSGOST in Korean, Danggui-Sini-Jia-Wuzhuyu-Shengian-Tang in Chinese, and Tokishigyakukagoshuyushokyoto (TJ-38) in Japanese), a well-known traditional Korean/Chinese/Japanese medicine, has long been used to treat vascular diseases such as Raynaud's phenomenon (RP). However, anticancer effect of DSGOST remains elusive. In this study, we checked if DSGOST has an anticancer effect against gastric cancer cells, and investigated the mechanisms underlying DSGOST resistance. Moreover, DSGOST regulates chemoresistance in cisplatin-treated gastric cancer cells. Interestingly, DSGOST treatment induced the accumulation of GFP-LC3 puncta and increased the level of autophagy markers, such as LC3-II, ATG5, and Beclin-1, indicating activated autophagy. Furthermore, DSGOST could activate epithelial-to-mesenchymal transition (EMT) and exosomes via induction of autophagy. DSGOST in combination with TGFβ also induced autophagy and EMT. However, autophagy inhibition induces DSGOST-mediated cell death in gastric cancer cells. In addition, autophagy inhibition blocks the activation of DSGOST-mediated EMT markers including N-cadherin, Snail, Slug, vimentin, β-catenin, p-Smad2, and p-Smad3. Taken together, these findings indicated that prosurvival autophagy was one of the mechanisms involved in the resistance of gastric cancer to DSGOST. Targeting the inhibition of autophagy could be an effective therapeutic approach to overcome resistance to DSGOST in gastric cancer. FAU - Kim, Tae Woo AU - Kim TW AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Lee, Seon Young AU - Lee SY AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Kim, Mia AU - Kim M AD - Department of Cardiovascular and Neurologic Disease (Stroke center), College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Cheon, Chunhoo AU - Cheon C AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Jang, Bo-Hyoung AU - Jang BH AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Shin, Yong Cheol AU - Shin YC AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. FAU - Ko, Seong-Gyu AU - Ko SG AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul, Korea. epiko@khu.ac.kr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180529 PL - England TA - Cell Death Dis JT - Cell death & disease JID - 101524092 RN - 0 (Beclin-1) RN - 0 (DSGOST) RN - 0 (Drugs, Chinese Herbal) RN - 0 (Proto-Oncogene Proteins c-bcl-2) RN - Q20Q21Q62J (Cisplatin) SB - IM MH - Autophagy/*drug effects MH - Beclin-1/metabolism MH - Cell Line, Tumor MH - Cisplatin/pharmacology MH - Drug Resistance, Neoplasm/*drug effects MH - Drugs, Chinese Herbal/*pharmacology MH - Epithelial-Mesenchymal Transition/drug effects MH - Humans MH - Proto-Oncogene Proteins c-bcl-2/metabolism MH - Signal Transduction/drug effects MH - Stomach Neoplasms/*pathology PMC - PMC5974125 COIS- The authors declare that they have no conflict of interest. EDAT- 2018/05/31 06:00 MHDA- 2019/12/18 06:00 PMCR- 2018/05/29 CRDT- 2018/05/31 06:00 PHST- 2017/12/12 00:00 [received] PHST- 2018/04/23 00:00 [accepted] PHST- 2018/04/20 00:00 [revised] PHST- 2018/05/31 06:00 [entrez] PHST- 2018/05/31 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2018/05/29 00:00 [pmc-release] AID - 10.1038/s41419-018-0658-y [pii] AID - 658 [pii] AID - 10.1038/s41419-018-0658-y [doi] PST - epublish SO - Cell Death Dis. 2018 May 29;9(6):649. doi: 10.1038/s41419-018-0658-y. PMID- 35658736 OWN - NLM STAT- MEDLINE DCOM- 20220907 LR - 20250728 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 31 IP - 10 DP - 2022 Sep TI - Long-term outcome and predictors of long-term outcome in patients with lupus nephritis managed at a tertiary hospital in Mumbai. PG - 1191-1201 LID - 10.1177/09612033221106607 [doi] AB - AIM: Study the long-term outcome of the patients with LN and identify the baseline factors that can predict the long-term outcome of these patients. METHODS: All biopsy-proven LN patients who attended our regular 'lupus nephritis' clinic from 2013 to 2021 were studied. Data of these patients were collected from the hospital patient records. Standard therapy was given as per the KDIGO guidelines, and the renal response was evaluated according to KDIGO outcome criteria. Cox' regression analysis was used to determine predictors of chronic kidney disease (persistent doubling of serum creatinine with creatinine ≥1.5 mg%). Kaplan-Meier analysis was used for renal survival. RESULTS: Eighty patients with at least 1 year of follow-up were included. Median age of onset was 24 years (IQR18-35). Median follow up was 6.5 years (IQR 3-10). World Health Organisation renal biopsy profile was Class I 1(1.2 %), Class II 6(7.5 %), Class III 9(11.2 %), Class IV 36(45 %), Class V 18(22.5 %) and Mixed Class IV + V 10 (12%). Complete remission was achieved in 63.75%, 70 % and 66.6% patients at 1, 2 and 5 years, respectively. Survival with normal renal function was 88.5 %, 85.8% and 60 % at 5, 10 and 15 years, respectively. Risk factors for poor outcome on univariate analysis were presence of Raynaud's phenomena-hazard ratio(HR) 7.78 (CI 1.944-31.207; p < .004), baseline hypertension-HR 5.356 (CI 1.479-19.403; p < .011), tubulointerstitial involvement-HR 1.076 (CI 1.032-1.222; p < .001), time to complete response-HR 1.036 (CI 1.036-1.067; p < .02 ), serum creatinine at 6 months HR 10.51 (CI 2.19-50.39; p < .003), failure to achieve complete response at 2 years HR 6.271 (CI 1.567-25.092; p < .009) and the number of nephritic flares HR 1.868(CI 1.103-3.164 ; p < .02). Renal relapses were quite common, with 1.8 flares per 10 patient-years of follow up. Infection was the most common cause of death, with bacterial lower respiratory infections and pulmonary tuberculosis being the most common. CONCLUSIONS: Apart from conventional risk factors, other predictive factors like the presence of Raynaud's phenomenon, tubulointerstitial fibrosis and tubular atrophy on kidney biopsy, and initial response to induction therapy by 6 months have a significant impact on the long-term outcome in patients with LN. FAU - Yadav, Sandeep AU - Yadav S AUID- ORCID: 0000-0001-6460-1743 AD - Department of Rheumatology, 29537P.D. Hinduja National Hospital and Medical Research Centre, Mumbai, India. FAU - Balakrishnan, C AU - Balakrishnan C AD - Department of Rheumatology, 29537P.D. Hinduja National Hospital and Medical Research Centre, Mumbai, India. FAU - Kothari, Jatin AU - Kothari J AD - Department of Nephrology, 29537PD Hinduja National Hospital and Medical Research Centre, Khar Mumbai, India. AD - Apex Kidney Foundation, Mumbai, India. AD - Apex Kidney Care - Dialysis Networks, Mumbai, India. AD - Nanavati Max Hospital, Mumbai, India. LA - eng PT - Journal Article DEP - 20220604 PL - England TA - Lupus JT - Lupus JID - 9204265 RN - AYI8EX34EU (Creatinine) SB - IM MH - Adolescent MH - Adult MH - Biopsy MH - Creatinine MH - Humans MH - Kidney/pathology MH - *Lupus Erythematosus, Systemic/pathology MH - *Lupus Nephritis/pathology MH - Retrospective Studies MH - Tertiary Care Centers MH - Treatment Outcome MH - Young Adult OTO - NOTNLM OT - complete remission OT - long term outcome OT - long term predictive factors OT - lupus nephritis OT - renal histopathology OT - renal survival EDAT- 2022/06/07 06:00 MHDA- 2022/09/08 06:00 CRDT- 2022/06/06 09:48 PHST- 2022/06/07 06:00 [pubmed] PHST- 2022/09/08 06:00 [medline] PHST- 2022/06/06 09:48 [entrez] AID - 10.1177/09612033221106607 [doi] PST - ppublish SO - Lupus. 2022 Sep;31(10):1191-1201. doi: 10.1177/09612033221106607. Epub 2022 Jun 4. PMID- 18555939 OWN - NLM STAT- MEDLINE DCOM- 20081006 LR - 20220408 IS - 0149-2918 (Print) IS - 0149-2918 (Linking) VI - 30 IP - 5 DP - 2008 May TI - Effects of aminaftone 75 mg TID on soluble adhesion molecules: a 12-week, randomized, open-label pilot study in patients with systemic sclerosis. PG - 924-9 LID - 10.1016/j.clinthera.2008.05.009 [doi] AB - BACKGROUND: Vasculopathy is one of the hallmarks of systemic sclerosis (SSc), characterized by endothelial activation and over expression of adhesion molecules. A preliminary in vitro study has suggested that aminaftone, a naphtohydrochinone used in the treatment of capillary disorders, may downregulate the expression of adhesion molecules in endothelial cells. OBJECTIVE: This study investigated the ex vivo effects of aminaftone on soluble adhesion molecule concentrations in patients with SSc. METHODS: This randomized, open-label pilot study was conducted in patients with SSc. Patients received baseline treatment for Raynaud's phenomenon (eg, calcium channel blockers and IV cyclic iloprost) with (test) or without (control) aminaftone 75 mg or placebo TID for 12 weeks. Standard treatment for Raynaud's phenomenon was allowed as long as the dose was stable for >or=3 months prior to randomization. Concentrations of soluble E-selectin adhesion molecule 1 (sELAM-1), soluble vascular cell adhesion molecule 1 (sVCAM-1), and soluble intracellular adhesion molecule 1 (sICAM-1) were measured at baseline and 12 weeks, and their variation was tested using the analysis of variance for repeated measures with statistical correction. Laboratory analyses were performed by experienced personnel blinded to treatment assignment. RESULTS: A total of 24 patients were enrolled (21 women, 3 men; mean age, 53.4 years; aminaftone, 12 patients; control, 12 patients). Decreases in mean (SD) sELAM-1 and sVCAM-1 concentrations were significantly greater in treated patients (sELAM-1, from 17.0 [7.8] to 11.9 [9.0] pg/mL; sVCAM-1, from 51.2 [12.9] to 40.8 [13.8] ng/mL) compared with controls (sELAM-1, from 20.3 [9.9] to 20.4 [10.5] pg/mL; sVCAM-1, from 56.8 [49.6] to 62.7 [40.6] ng/mL) (both, P < 0.05 [analysis of variance or repeated measures after Bonferroni correction]). No significant changes in sICAM-1 concentrations versus controls were observed. CONCLUSIONS: In this small pilot study in this select group of patients with SSc, aminaftone was associated with downregulation of sELAM-1 and sVCAM-1 concentrations. Studies evaluating the potential role of aminaftone in the treatment of vascular sclerodermal disease and SSc are warranted. FAU - Scorza, Raffaella AU - Scorza R AD - Referral Center for Systemic Autoimmune Diseases, University of Milan, Milan, Italy. raffaella.scorza@unimi.it FAU - Santaniello, Alessandro AU - Santaniello A FAU - Salazar, Giulia AU - Salazar G FAU - Lenna, Stefania AU - Lenna S FAU - Della Bella, Silvia AU - Della Bella S FAU - Antonioli, Rita AU - Antonioli R FAU - Toussoun, Karen AU - Toussoun K FAU - Beretta, Lorenzo AU - Beretta L LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin Ther JT - Clinical therapeutics JID - 7706726 RN - 0 (Cell Adhesion Molecules) RN - 0 (E-Selectin) RN - 0 (Vascular Cell Adhesion Molecule-1) RN - 0 (para-Aminobenzoates) RN - 03JLX11PE9 (aminaftone) RN - 126547-89-5 (Intercellular Adhesion Molecule-1) RN - TL2TJE8QTX (4-Aminobenzoic Acid) SB - IM MH - 4-Aminobenzoic Acid/administration & dosage/pharmacology MH - Cell Adhesion Molecules/*biosynthesis MH - E-Selectin/biosynthesis MH - Female MH - Humans MH - Intercellular Adhesion Molecule-1/biosynthesis MH - Male MH - Middle Aged MH - Pilot Projects MH - Prospective Studies MH - Scleroderma, Systemic/*metabolism MH - Solubility MH - Vascular Cell Adhesion Molecule-1/biosynthesis MH - *para-Aminobenzoates EDAT- 2008/06/17 09:00 MHDA- 2008/10/07 09:00 CRDT- 2008/06/17 09:00 PHST- 2008/05/14 00:00 [accepted] PHST- 2008/06/17 09:00 [pubmed] PHST- 2008/10/07 09:00 [medline] PHST- 2008/06/17 09:00 [entrez] AID - S0149-2918(08)00179-3 [pii] AID - 10.1016/j.clinthera.2008.05.009 [doi] PST - ppublish SO - Clin Ther. 2008 May;30(5):924-9. doi: 10.1016/j.clinthera.2008.05.009. PMID- 42001358 OWN - NLM STAT- Publisher LR - 20260419 IS - 1437-7799 (Electronic) IS - 1342-1751 (Linking) DP - 2026 Apr 19 TI - Characteristics of newly diagnosed systemic lupus erythematosus patients with or without kidney involvement: analysis of the National Database of Designated Intractable Diseases of Japan. LID - 10.1007/s10157-026-02870-5 [doi] AB - BACKGROUND: The purpose of this study was to clarify the characteristics of newly diagnosed systemic lupus erythematosus (SLE) patients with or without kidney involvement in Japan. METHODS: We used electronic data of SLE patients in the National Database of Designated Intractable Diseases of Japan who newly registered between 2015 and 2017. We analyzed patients within one year of disease onset. Kidney involvement was defined as any of the following: urinary protein ≥ 0.5 g/day, granular casts, a clinical diagnosis of nephrotic syndrome, acute or chronic renal failure, or rapidly progressive glomerulonephritis, an estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m(2), lupus nephritis confirmed by renal biopsy, or hemodialysis. RESULTS: Among 2315 SLE patients, 1088 (47.0%) had kidney involvement. Patients with kidney involvement more frequently exhibited symptoms such as pulmonary hemorrhage, pulmonary infarction and disturbance of consciousness, pericarditis, and hemolytic anemia whereas manifestations such as arthritis, aseptic meningitis, discoid rash, photosensitivity, and Raynaud's phenomenon were less common compared with those without kidney involvement. Anti-DNA antibody positivity was higher and complement levels (C3, C4, and CH50) were lower in patients with kidney involvement. In addition, concomitant glucocorticoid pulse therapy and immunosuppressive drugs were more frequently used in patients with kidney involvement. CONCLUSION: In this nationwide cohort, nearly half of newly diagnosed Japanese patients with SLE had kidney involvement and showed a distinct pattern of systemic manifestations, autoantibody profiles, and treatment intensity. These findings provide important insights into the epidemiology and pathophysiology of kidney involvement in SLE in Japan. CI - © 2026. The Author(s), under exclusive licence to Japanese Society of Nephrology. FAU - Ikeuchi, Hidekazu AU - Ikeuchi H AD - Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan. FAU - Kimura, Tomonori AU - Kimura T AD - Department of Nephrology, The University of Osaka Graduate School of Medicine, Suita, Japan. FAU - Sakate, Ryuichi AU - Sakate R AD - Laboratory of Rare Disease Information and Resource Library, Center for Intractable Diseases and ImmunoGenomics (CiDIG), Health and Nutrition (NIBN), National Institutes of Biomedical Innovation, Ibaraki, Japan. FAU - Maruyama, Shoichi AU - Maruyama S AD - Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan. FAU - Isaka, Yoshitaka AU - Isaka Y AD - Department of Nephrology, The University of Osaka Graduate School of Medicine, Suita, Japan. FAU - Narita, Ichiei AU - Narita I AD - Division of Clinical Nephrology and Rheumatology, Kidney Research Center, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. FAU - Hiromura, Keiju AU - Hiromura K AUID- ORCID: 0000-0002-9490-8364 AD - Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan. hiromura@gunma-u.ac.jp. LA - eng GR - 20FC1045/Ministry of Health, Labour and Welfare/ GR - 23FC1048/Ministry of Health, Labour and Welfare/ PT - Journal Article DEP - 20260419 PL - Japan TA - Clin Exp Nephrol JT - Clinical and experimental nephrology JID - 9709923 SB - IM OTO - NOTNLM OT - Anti-DNA antibody OT - Glucocorticoid OT - Kidney involvement OT - Systemic lupus erythematosus COIS- Declarations. Conflict of interest: S. Maruyama has received grants/research support from Rohto Pharmaceutical Co., Ltd., Tanabe Pharma Corporation, Zenyaku Kogyo, Nipro Corporation, and honoraria from AstraZeneca, Alexion Pharma, Tanabe Pharma Corporation, Ono Pharmaceutical, Kyowa Kirin, Torii Pharmaceutical, Nippon Boehringer Ingelheim, Otsuka Pharmaceutical, and scholarship donations from Otsuka Pharmaceutical and Torii Pharmaceutical. I. Narita has received speaker's fees from Otsuka Pharmaceutical, Sanofi, Bayer Yakuhin, Kyowa Kirin, and research grants from Bayer Yakuhin, Chugai Pharmaceutical, Kyowa Kirin, Terumo, Kaneka, Otsuka Pharmaceutical, Daiichi Sankyo, Astellas Pharma, Sumitomo Pharma, Ono Pharmaceutical, and Amicus Therapeutics. K. Hiromura has received consultancy fees from Otsuka Pharmaceutical and GlaxoSmithKline, grants/research support from Chugai Pharmaceutical, and honoraria from Astellas Pharma, AstraZeneca, Asahi Kasei Pharma, GlaxoSmithKline, Kissei Pharmaceutical, and Otsuka Pharmaceutical. Ethical approval: This retrospective cohort study followed the guidelines of the Declaration of Helsinki. It was approved by the Ethics Committee of the Japanese Society of Nephrology (approval number 70) and the Ethics Committee of Gunma University Graduate School of Medicine (approval number HS2019-280). Informed consent: Written informed consent from patients was obtained to register Medical Certificates of Designated Intractable Diseases in the NDDID-J and to use the data for research and policy-making. EDAT- 2026/04/20 07:10 MHDA- 2026/04/20 07:10 CRDT- 2026/04/19 11:04 PHST- 2025/12/27 00:00 [received] PHST- 2026/04/07 00:00 [accepted] PHST- 2026/04/20 07:10 [medline] PHST- 2026/04/20 07:10 [pubmed] PHST- 2026/04/19 11:04 [entrez] AID - 10.1007/s10157-026-02870-5 [pii] AID - 10.1007/s10157-026-02870-5 [doi] PST - aheadofprint SO - Clin Exp Nephrol. 2026 Apr 19. doi: 10.1007/s10157-026-02870-5. PMID- 39557603 OWN - NLM STAT- MEDLINE DCOM- 20250426 LR - 20250517 IS - 1750-3639 (Electronic) IS - 1015-6305 (Print) IS - 1015-6305 (Linking) VI - 35 IP - 3 DP - 2025 May TI - Clinico-sero-pathological characteristics of anti-Ha antisynthetase syndrome. PG - e13319 LID - 10.1111/bpa.13319 [doi] LID - e13319 AB - To define the clinical, serological, and muscle histopathological characteristics, as well as treatment outcomes, of patients with anti-Ha antibody. We performed a retrospective analysis of clinical, serological, and pathological data and long-term treatment outcomes of anti-Ha patients between January 2005 and July 2023 at our center. Anti-Ha antibody was identified by immunoblot and reconfirmed by immunoprecipitation. Of the 570 patients with idiopathic inflammatory myopathies, 17 (3.0%) were found to be anti-Ha positive, of whom 5 (29.4%) were also positive for another myositis-specific antibody (MSA). All patients with anti-Ha antibody as the single MSA (12/17, 70.6%) had clinical and histopathological evidence of muscle damage. Skin lesions were identified in nine of them (75%), while both interstitial lung disease and Raynaud's phenomenon were only seen in four patients. A necrotizing myopathy without a perifascicular pattern was the most common pathological manifestation (50%). Perifascicular necrosis (PFN) and myofiber major histocompatibility complex class-II expression were observed only in one and four patients, respectively. Muscle weakness relapse was reported in five patients, and skin rashes worsening were observed in one patient. Most of the anti-Ha patients (66.7%) finally achieved a favorable outcome at last follow-up. Anti-Ha antibody might not be as rare as previously thought and may coexist with other MSAs. Muscle damage is the most common manifestation in anti-Ha patients, while extra-muscular symptoms except for the cutaneous manifestations are unusual. The histopathological features varied with a predominance of necrotizing myopathy without PFN. These patients often finally had favorable outcomes, although relapses often occur. CI - © 2024 The Author(s). Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology. FAU - Zhao, Bing AU - Zhao B AD - Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. FAU - Hou, Ying AU - Hou Y AD - Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. FAU - Shao, Kai AU - Shao K AD - Department of Medicine Experimental Center, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. FAU - Ma, XiaoTian AU - Ma X AD - Department of Medicine Experimental Center, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. FAU - Yan, YaPing AU - Yan Y AD - Key Laboratory of the Ministry of Education for Medicinal Resources and Natural Pharmaceutical Chemistry, College of Life Sciences, Shanxi Normal University, Xi'an, China. FAU - Lu, Jian-Qiang AU - Lu JQ AUID- ORCID: 0000-0003-1945-5569 AD - Department of Pathology and Molecular Medicine, Division of Neuropathology, McMaster University, Hamilton, Ontario, Canada. FAU - Li, Wei AU - Li W AD - Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. FAU - Yan, ChuanZhu AU - Yan C AD - Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. AD - Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. AD - Department of Medicine Experimental Center, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. FAU - Zhang, LiNing AU - Zhang L AUID- ORCID: 0000-0002-7563-6957 AD - Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. AD - Department of Rheumatology, Shandong Key Laboratory of Medicine and Prevention Integration in Rheumatism and Immunity Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. FAU - Dai, TingJun AU - Dai T AUID- ORCID: 0000-0003-4928-0421 AD - Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China. AD - Department of Medicine Experimental Center, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China. LA - eng GR - ZR2023QH364/Natural Science Foundation of Shandong Province/ GR - 82071412/National Natural Science Foundation of China/ GR - 82171395/National Natural Science Foundation of China/ GR - 22-3-7-lczx-3-nsh/Qingdao Key Health Discipline Development Fund and Qingdao Clinical Research Center for Rare Diseases of Nervous System/ GR - 24-4-4zrjj-104-jch/Qingdao Natural Science Foundation/ PT - Journal Article DEP - 20241118 PL - Switzerland TA - Brain Pathol JT - Brain pathology (Zurich, Switzerland) JID - 9216781 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Humans MH - *Myositis/pathology/immunology/blood MH - Male MH - Female MH - Middle Aged MH - Retrospective Studies MH - *Autoantibodies/blood/immunology MH - Adult MH - Aged MH - Muscle, Skeletal/pathology PMC - PMC11961205 OTO - NOTNLM OT - anti‐Ha antibody OT - favorable outcomes OT - myositis‐specific antibodies OT - necrotizing myopathy without perifascicular pattern OT - relapse COIS- The authors have declared no conflicts of interest. EDAT- 2024/11/19 00:22 MHDA- 2025/04/02 04:57 PMCR- 2024/11/18 CRDT- 2024/11/18 22:02 PHST- 2024/06/27 00:00 [received] PHST- 2024/10/30 00:00 [accepted] PHST- 2025/04/02 04:57 [medline] PHST- 2024/11/19 00:22 [pubmed] PHST- 2024/11/18 22:02 [entrez] PHST- 2024/11/18 00:00 [pmc-release] AID - BPA13319 [pii] AID - 10.1111/bpa.13319 [doi] PST - ppublish SO - Brain Pathol. 2025 May;35(3):e13319. doi: 10.1111/bpa.13319. Epub 2024 Nov 18. PMID- 39036171 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240723 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 16 IP - 6 DP - 2024 Jun TI - Excessive Dynamic Airway Collapse After Entecavir Use in a Patient With Pegylated Interferon-Induced Undifferentiated Connective Tissue Disease and Entecavir Use to Prevent Hepatitis B Virus Reactivation Upon Giving Rituximab. PG - e62835 LID - 10.7759/cureus.62835 [doi] LID - e62835 AB - Pegylated interferon-alpha (PEG-IFN-α) is an antiviral medication used to treat chronic hepatitis C virus (HCV) and hepatitis B virus (HBV) infections. It may result in rare but severe side effects, such as undifferentiated connective tissue disease (UCTD) and excessive dynamic airway collapse (EDAC), which can occur as delayed complications of PEG-IFN-α-induced UCTD. In cases where these complications arise, entecavir, employed for treating HBV infection, may be considered. A 49-year-old female patient, monitored for nine years with HCV and a viral load of 1.5 million, genotype 3, and normal liver function tests (LFTs), possibly acquired the infection from her HCV-positive husband. The patient was initially treated with PEG-IFN-α (IFN-α-2b, 100 µg/week subcutaneously) and ribavirin (RBV, 500 mg/twice daily). Following the sixth injection, the patient exhibited symptoms, including shortness of breath and cough, leading to limited daily activities. Subsequent high-resolution computed tomography (HRCT) showed interstitial pneumonitis (IP) signs. She was given a high dose of steroids. Over the next two to four weeks, the patient experienced Raynaud's phenomenon, skin tightening, joint pains, and dryness of the eyes and mouth. The antinuclear antibody (ANA) test was negative, while the extractable nuclear antigen (ENA) test showed equivocal anti-Smith antibodies (6.38). Rheumatoid factor (RA) factors were mildly positive, and pulmonary function tests (PFTs) indicated a restrictive pattern. The patient was intolerant to hydroxychloroquine (HCQ) and azathioprine (Imuran) 500 mg, subsequently receiving mycophenolate mofetil 500 mg/thrice daily. Despite four years of treatment, UCTD due to PEG-IFN-α remained difficult to control; however, IP responded well to steroids. Rituximab pulse therapy was planned before the initiation; serological tests showed positive anti-HBs with a titer of 17.02, positive anti-HBc, but negative HBsAg and undetectable HBV viral load, indicating immunity to HBV due to natural infection. Given the potential for rituximab to cause immunosuppression and HBV reactivation, entecavir treatment was started and continued for 18 months. The patient was followed for another five years, during which her LFTs and viral markers showed stability. However, after nine years of PEG-IFN-α-induced UCTD disorder, she experienced a reoccurring cough but was unresponsive to steroids that were against her suspicion of a flare of IP. A subsequent dynamic CT scan detected a 75% trachea collapse while in a supine position, indicating a potential complication termed EDAC. This EDAC could not be linked to PEG-IFN-α-induced UCTD disorder or EDAC after the use of entecavir in a patient with PEG-IFN-α-induced UCTD disorder. Treatment of such complex patients requires flexible, specific treatment plans and continuous monitoring. This case emphasizes the need for caution in patients with a history of IFN-induced disease and the possibility of late effects and possible effects of the use of entecavir in a patient with PEG-IFN-α-induced UCTD. To the best of our knowledge, this is the first case reported as EDAC, a possible delayed complication of PEG-IFN-α plus ribavirin or entecavir in a patient with PEG-IFN-α-induced UCTD. CI - Copyright © 2024, Akhtar et al. FAU - Akhtar, Muhammad Zain AU - Akhtar MZ AD - Department of Medicine, Mayo Hospital, Lahore, PAK. AD - Department of Medicine, Royal Preston Hospital, Preston, GBR. FAU - Tayab, Ghias Un Nabi AU - Tayab GUN AD - Department of Gastroenterology and Hepatology, Lahore General Hospital, Lahore, PAK. FAU - Nawaz, Muhammad Imran AU - Nawaz MI AD - Department of Medicine, Lahore General Hospital, Lahore, PAK. FAU - Seher, Iqra AU - Seher I AD - Department of Medicine, Lahore General Hospital, Lahore, PAK. LA - eng PT - Case Reports PT - Journal Article DEP - 20240621 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC11260405 OTO - NOTNLM OT - delayed complications OT - entecavir OT - hepatitis c virus treatment OT - pegylated interferon OT - undifferentiated connective tissue disease COIS- Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2024/07/22 06:43 MHDA- 2024/07/22 06:44 PMCR- 2024/06/21 CRDT- 2024/07/22 05:29 PHST- 2024/06/21 00:00 [accepted] PHST- 2024/07/22 06:44 [medline] PHST- 2024/07/22 06:43 [pubmed] PHST- 2024/07/22 05:29 [entrez] PHST- 2024/06/21 00:00 [pmc-release] AID - 10.7759/cureus.62835 [doi] PST - epublish SO - Cureus. 2024 Jun 21;16(6):e62835. doi: 10.7759/cureus.62835. eCollection 2024 Jun. PMID- 29531773 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240922 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 5 IP - 1 DP - 2018 TI - Unique clinical characteristics, autoantibodies and medication use in Native American patients with systemic lupus erythematosus. PG - e000247 LID - 10.1136/lupus-2017-000247 [doi] LID - e000247 AB - OBJECTIVE: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with varied morbidity and mortality. We assessed clinical presentations, autoantibody specificities and therapeutic interventions in Native American (NA) patients with SLE. METHODS: Patients with SLE meeting 1997 American College of Rheumatology classification criteria (n=3148) were enrolled between 1992 and 2010 in the multiethnic, cross-sectional Lupus Family Registry and Repository. Clinical, demographic and therapeutic information were extracted from medical records using a standardised form and formalised training. Autoantibodies were assessed by indirect immunofluorescence (antinuclear antibodies (ANA) and antidouble-stranded DNA), precipitin (ENA) and ELISA (IgG and IgM anticardiolipins). RESULTS: NA patients met SLE classification at a younger age (29.89±12.3 years) than European Americans (EA; 32.02±12.87, P=0.0157) and a similar age to African-Americans (AAs) and Hispanics (HIS). More NA patients had concurrent rheumatic diseases or symptoms, such as Raynaud's phenomenon, interstitial lung disease, Sjӧgren's syndrome and systemic sclerosis. Compared with EAs, NAs were more likely to have high-titre ANA (≥1:3240; P<0.0001) and had more SLE-associated autoantibodies. Autoantibodies with unknown specificities were more common in NAs (41%) compared with other racial/ethnic groups in this collection (AA: 24%, P=0.0006; EA: 17%, P<0.0001; HIS: 23%, P=0.0050). Fewer NA patients used hydroxychloroquine (68%) compared with others (AA: 74%, P=0.0308; EA: 79%, P=0.0001, HIS: 77%, P=0.0173); this was influenced by lower hydroxychloroquine use in NA patients from Latin America (32%). NA patients had higher rates of methotrexate use (28%) compared with AA (18%, P=0.0006) and HIS patients (14%, P=0.0003), higher azathioprine use (38%) compared with EA patients (30%, P=0.0105) and higher mycophenolate mofetil use (26%) compared with EA (17%, P=0.0012) and HIS patients (11%, P<0.0001). CONCLUSIONS: NA patients are diagnosed with SLE earlier in life and present worse concurrent rheumatic disease symptoms than EA patients. NA patients also are more likely to have expanded autoantibody profiles and precipitins of unknown specificities. FAU - Kheir, Joseph M AU - Kheir JM AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Guthridge, Carla J AU - Guthridge CJ AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Johnston, Jonathon R AU - Johnston JR AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. AD - Oklahoma State University Health Sciences Center, Tulsa, Oklahoma, USA. FAU - Adams, Lucas J AU - Adams LJ AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Rasmussen, Astrid AU - Rasmussen A AUID- ORCID: 0000-0001-7744-2948 AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Gross, Timothy F AU - Gross TF AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Munroe, Melissa E AU - Munroe ME AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Bourn, Rebecka L AU - Bourn RL AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Sivils, Kathy L AU - Sivils KL AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Guthridge, Joel M AU - Guthridge JM AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. FAU - Weisman, Michael H AU - Weisman MH AD - Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA. FAU - Wallace, Daniel J AU - Wallace DJ AD - Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA. FAU - Anaya, Juan-Manuel AU - Anaya JM AD - Center for Autoimmune Diseases Research (CREA), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia. FAU - Rojas Villarraga, Adriana AU - Rojas Villarraga A AD - Artmedica IPS, Bogotá, Colombia. FAU - Jarvis, James N AU - Jarvis JN AD - Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA. FAU - Harley, John B AU - Harley JB AD - Center for Autoimmune Genomics and Etiology (CAGE), Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. AD - US Department of Veterans Affairs Medical Center, Cincinnati, Ohio, USA. FAU - James, Judith A AU - James JA AD - Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA. AD - Department of Medicine and Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA. LA - eng GR - U54 GM104938/GM/NIGMS NIH HHS/United States GR - P30 AR053483/AR/NIAMS NIH HHS/United States GR - I01 BX001834/BX/BLRD VA/United States GR - P30 GM103510/GM/NIGMS NIH HHS/United States GR - U19 AI082714/AI/NIAID NIH HHS/United States GR - U01 AI130830/AI/NIAID NIH HHS/United States GR - U01 AI101934/AI/NIAID NIH HHS/United States GR - F32 HS000088/HS/AHRQ HHS/United States GR - R01 AI024717/AI/NIAID NIH HHS/United States GR - U01 HG008666/HG/NHGRI NIH HHS/United States PT - Journal Article DEP - 20180227 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 PMC - PMC5844376 OTO - NOTNLM OT - antinuclear antibodies (ANA) OT - autoantibodies OT - systemic lupus erythematosus (SLE) COIS- Competing interests: AR reports personal fees from ThermoFisher for talks regarding diagnosis and classification of Sjögren’s syndrome and the different autoantibody testing platforms. EDAT- 2018/03/14 06:00 MHDA- 2018/03/14 06:01 PMCR- 2018/02/27 CRDT- 2018/03/14 06:00 PHST- 2017/10/31 00:00 [received] PHST- 2018/01/16 00:00 [revised] PHST- 2018/02/02 00:00 [accepted] PHST- 2018/03/14 06:00 [entrez] PHST- 2018/03/14 06:00 [pubmed] PHST- 2018/03/14 06:01 [medline] PHST- 2018/02/27 00:00 [pmc-release] AID - lupus-2017-000247 [pii] AID - 10.1136/lupus-2017-000247 [doi] PST - epublish SO - Lupus Sci Med. 2018 Feb 27;5(1):e000247. doi: 10.1136/lupus-2017-000247. eCollection 2018. PMID- 39700423 OWN - NLM STAT- MEDLINE DCOM- 20250528 LR - 20260518 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 6 DP - 2025 Jun 1 TI - Biological hallmarks of systemic sclerosis are present in the skin and serum of patients with Very Early Diagnosis of Systemic Sclerosis (VEDOSS). PG - 3606-3617 LID - 10.1093/rheumatology/keae698 [doi] AB - OBJECTIVE: The Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR study showed that, despite not showing any clinical sign of disease, patients with Raynaud's and ANA and/or capillaroscopy abnormalities often progress to SSc within 5 years. We aimed to determine whether VEDOSS biosamples show biological SSc activity pre-clinically. METHODS: Skin biopsies were histologically analysed. Dermal fibroblasts analysed by RT-qPCR and gel contraction assays. Sera were assayed by Luminex (CXCL10) or ELISA (ELF score). Healthy controls (HC) and SSc biosamples were used for controls. RESULTS: Overall, 114 consecutive VEDOSS patients were enrolled, of which 36 consented to have skin biopsies. Skin biopsies showed a variable but overall increased collagen staining and skin thickness, increased perivascular infiltrate of CD45-positive cells and CXCL10 expression. In vitro, VEDOSS dermal fibroblasts showed increased profibrotic gene expression and contractibility compared with HC. Increased serological CXCL10 [mean (s.d.) 75.90 (107.80) vs HC 39.90 (26.27) pg/ml, P = 0.02] and ELF score was evident in VEDOSS compared with HC [8.19 (0.78) vs 8.55 (0.79), P = 0.04]. In longitudinal analysis of a median of 27.5 (interquartile range 44.5) months, 14.9% of VEDOSS patients progressed to SSc. Baseline CXCL10 serum concentration was significantly higher in the VEDOSS patients that progressed (2-fold increase, P = 0.0071) and correlated with ELF score (R = 0.3096, P = 0.0065). CONCLUSIONS: Despite not fulfilling classification criteria, VEDOSS patients show SSc-linked fibrosis and immunity dysregulation both within the tissue and sera, supporting a biological diagnosis of disease and a window of opportunity to detect the biological pathways amenable for preventive intervention. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Ross, Rebecca L AU - Ross RL AUID- ORCID: 0000-0001-7750-4280 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals, NHS Trust, Chapel Allerton Hospital, Leeds, UK. FAU - Caballero-Ruiz, Begoña AU - Caballero-Ruiz B AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Clarke, Emily L AU - Clarke EL AD - Section of Pathology and Tumour Biology, University of Leeds, Leeds, UK. AD - Leeds Teaching Hospitals, NHS Trust, St James's University Hospital, Leeds, UK. FAU - Kakkar, Vishal AU - Kakkar V AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Wasson, Christopher W AU - Wasson CW AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Mulipa, Panji AU - Mulipa P AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - De Lorenzis, Enrico AU - De Lorenzis E AUID- ORCID: 0000-0001-9819-105X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy. FAU - Merchant, Will AU - Merchant W AD - Leeds Teaching Hospitals, NHS Trust, St James's University Hospital, Leeds, UK. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Rindone, Andrea AU - Rindone A AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - Department of Rheumatology and Medical Science, University of Milan, ASST Gaetano Pini-CTO Institute, Milan, Italy. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - Riobo-Del Galdo, Natalia A AU - Riobo-Del Galdo NA AD - School of Molecular and Cellular Biology, Faculty of Biological Sciences, University of Leeds, Leeds, UK. AD - Leeds Institute of Medical Research, University of Leeds, Leeds, UK. AD - Astbury Centre for Structural Molecular Biology, University of Leeds, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals, NHS Trust, Chapel Allerton Hospital, Leeds, UK. LA - eng GR - SRUK/ GR - Kennedy Trust Program Foundation/ GR - National Institute for Health/ GR - Care Research Biomedical Research Centre/ GR - NIHR213331/NIHR/ GR - Susan Cheney Scleroderma Research Foundation/ GR - MR/S001530/1/MRC_/Medical Research Council/United Kingdom GR - Alan Turing Institute/ GR - National Institute for Health and Care Research/ GR - NIHR203331/Leeds Biomedical Research Centre/ GR - NIHR203308/Manchester Biomedical Research Centre/ GR - Department of Health and Social Care/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Chemokine CXCL10) RN - 0 (Biomarkers) RN - 0 (CXCL10 protein, human) SB - IM MH - Humans MH - *Scleroderma, Systemic/diagnosis/pathology/blood/metabolism MH - Female MH - Male MH - Early Diagnosis MH - Middle Aged MH - *Skin/pathology/metabolism MH - Adult MH - *Chemokine CXCL10/blood/metabolism MH - Fibroblasts/metabolism/pathology MH - Aged MH - Disease Progression MH - Biopsy MH - Biomarkers/blood/metabolism MH - Case-Control Studies PMC - PMC12107078 OTO - NOTNLM OT - CXCL10 OT - VEDOSS OT - autoimmune diseases OT - collagen OT - connective tissue diseases OT - dermal fibroblasts OT - extracellular matrix OT - fibrosis OT - interferon OT - systemic sclerosis EDAT- 2024/12/19 18:21 MHDA- 2025/05/28 06:29 PMCR- 2024/12/19 CRDT- 2024/12/19 15:43 PHST- 2024/08/01 00:00 [received] PHST- 2024/12/04 00:00 [accepted] PHST- 2025/05/28 06:29 [medline] PHST- 2024/12/19 18:21 [pubmed] PHST- 2024/12/19 15:43 [entrez] PHST- 2024/12/19 00:00 [pmc-release] AID - 7928850 [pii] AID - keae698 [pii] AID - 10.1093/rheumatology/keae698 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Jun 1;64(6):3606-3617. doi: 10.1093/rheumatology/keae698. PMID- 36470210 OWN - NLM STAT- MEDLINE DCOM- 20230210 LR - 20230224 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 134 DP - 2023 Jan TI - Autoantigenic properties of the aminoacyl tRNA synthetase family in idiopathic inflammatory myopathies. PG - 102951 LID - S0896-8411(22)00159-7 [pii] LID - 10.1016/j.jaut.2022.102951 [doi] AB - OBJECTIVES: Autoantibodies are thought to play a key role in the pathogenesis of idiopathic inflammatory myopathies (IIM). However, up to 40% of IIM patients, even those with clinical manifestations of anti-synthetase syndrome (ASSD), test seronegative to known myositis-specific autoantibodies. We hypothesized the existence of new potential autoantigens among human cytoplasmic aminoacyl tRNA synthetases (aaRS) in patients with IIM. METHODS: Plasma samples from 217 patients with IIM according to 2017 EULAR/ACR criteria, including 50 patients with ASSD, 165 without, and two with unknown ASSD status were identified retrospectively, as well as age and gender-matched sera from 156 population controls, and 219 disease controls. Patients with previously documented ASSD had to test positive for at least one of the five most common anti-aaRS autoantibodies (anti-Jo1, -PL7, -PL12, -EJ, and -OJ) and present with one or more of the following clinical manifestations: interstitial lung disease, myositis, arthritis, Raynaud's phenomenon, fever, or mechanic's hands. Demographics, laboratory, and clinical data of the IIM cohort (ASSD and non-ASSD) were compared. Samples were screened using a multiplex bead array assay for presence of autoantibodies against a panel of 117 recombinant protein variants, representing 33 myositis-related proteins, including all nineteen cytoplasmic aaRS. Prospectively collected clinical data for the IIM cohort were retrieved and compared between groups within the IIM cohort and correlated with the results of the autoantibody screening. Principal component analysis was used to analyze clinical manifestations between ASSD, non-ASSD groups, and individuals with novel anti-aaRS autoantibodies. RESULTS: We identified reactivity towards 16 aaRS in 72 of the 217 IIM patients. Twelve patients displayed reactivity against nine novel aaRS. The novel autoantibody specificities were detected in four previously seronegative patients for myositis-specific autoantibodies and eight with previously detected myositis-specific autoantibodies. IIM individuals with novel anti-aaRS autoantibodies (n = 12) all had signs of myositis, and they had either muscle weakness and/or muscle enzyme elevation, 2/12 had mechanic's hands, 3/12 had interstitial lung disease, and 2/12 had arthritis. The individuals with novel anti-aaRS and a pathological muscle biopsy all presented widespread up-regulation of major histocompatibility complex class I. The reactivities against novel aaRS could be confirmed in ELISA and western blot. Using the multiplex bead array assay, we could confirm previously known reactivities to four of the most common aaRS (Jo1, PL12, PL7, and EJ (n = 45)) and identified patients positive for anti-Zo, -KS, and -HA (n = 10) that were not previously tested. A low frequency of anti-aaRS autoantibodies was also detected in controls. CONCLUSION: Our results suggest that most, if not all, cytoplasmic aaRS may become autoantigenic. Autoantibodies against new aaRS may be found in plasma of patients previously classified as seronegative with potential high clinical relevance. CI - Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Preger, Charlotta AU - Preger C AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden; Structural Genomics Consortium, Karolinska Institutet, Stockholm, Sweden. FAU - Notarnicola, Antonella AU - Notarnicola A AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden. FAU - Hellström, Cecilia AU - Hellström C AD - KTH Royal Institute of Technology, Department of Protein Science, SciLifeLab, Stockholm, Sweden. FAU - Wigren, Edvard AU - Wigren E AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden; Structural Genomics Consortium, Karolinska Institutet, Stockholm, Sweden. FAU - Fernandes-Cerqueira, Cátia AU - Fernandes-Cerqueira C AD - IsoPlexis, Branford, USA. FAU - Kvarnström, Marika AU - Kvarnström M AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden; Academic Specialist Center, Center for Rheumatology, Stockholm Health Services, Stockholm, Sweden. FAU - Wahren-Herlenius, Marie AU - Wahren-Herlenius M AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden; Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Bergen, Norway. FAU - Idborg, Helena AU - Idborg H AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden. FAU - Lundberg, Ingrid E AU - Lundberg IE AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden. FAU - Persson, Helena AU - Persson H AD - KTH Royal Institute of Technology, Department of Protein Science, SciLifeLab, Stockholm, Sweden. FAU - Gräslund, Susanne AU - Gräslund S AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden; Structural Genomics Consortium, Karolinska Institutet, Stockholm, Sweden. FAU - Jakobsson, Per-Johan AU - Jakobsson PJ AD - Karolinska Institutet, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden; Karolinska University Hospital, Stockholm, Sweden. Electronic address: per-johan.jakobsson@ki.se. LA - eng GR - 106169/ZZ14/Z/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20221202 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - 0 (Autoantigens) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Amino Acyl-tRNA Synthetases MH - Retrospective Studies MH - Autoantigens MH - *Myositis MH - Autoantibodies MH - *Lung Diseases, Interstitial MH - *Arthritis MH - Syndrome OTO - NOTNLM OT - Aminoacyl tRNA synthetases OT - Anti-synthetase syndrome OT - Autoantibodies OT - Idiopathic inflammatory myopathies OT - anti-aaRS EDAT- 2022/12/06 06:00 MHDA- 2023/02/11 06:00 CRDT- 2022/12/05 18:29 PHST- 2022/06/13 00:00 [received] PHST- 2022/11/01 00:00 [revised] PHST- 2022/11/02 00:00 [accepted] PHST- 2022/12/06 06:00 [pubmed] PHST- 2023/02/11 06:00 [medline] PHST- 2022/12/05 18:29 [entrez] AID - S0896-8411(22)00159-7 [pii] AID - 10.1016/j.jaut.2022.102951 [doi] PST - ppublish SO - J Autoimmun. 2023 Jan;134:102951. doi: 10.1016/j.jaut.2022.102951. Epub 2022 Dec 2. PMID- 40731758 OWN - NLM STAT- MEDLINE DCOM- 20250730 LR - 20250801 IS - 1648-9144 (Electronic) IS - 1010-660X (Print) IS - 1010-660X (Linking) VI - 61 IP - 7 DP - 2025 Jun 23 TI - Pediatric Sjögren's Syndrome: Focus on Ocular Involvement and Diagnostic Challenges. LID - 10.3390/medicina61071128 [doi] LID - 1128 AB - Background and Objectives: Pediatric Sjögren's syndrome is a rare autoimmune disease with a heterogeneous clinical expression and limited pediatric-specific diagnostic criteria. Ocular involvement often represents an early manifestation, yet it may go unrecognized in children due to poor symptom reporting and the underuse of objective diagnostic tools. This retrospective study evaluated six pediatric patients with Sjögren's syndrome, integrating systemic and ocular findings with a focus on early immunological and clinical markers. Materials and Methods: All patients underwent ophthalmological assessments, including tear break-up time, Schirmer's test, and slit-lamp examination. Results: Tear break-up time values consistently indicated tear film instability (mean RE 7.4 ± 2.5 s; LE 7.7 ± 2.3 s), while Schirmer's test showed greater variability. Slit-lamp examination revealed inhomogeneous tear films in all patients and blepharitis in 66.7%, consistent with Meibomian gland dysfunction. Systemic features included arthralgia, Raynaud's phenomenon, fatigue, and frequent seropositivity for ANA and anti-SSA/Ro antibodies. Minor salivary gland biopsy confirmed lymphoepithelial sialadenitis in all cases. Conclusions: These findings highlight the importance of combining laboratory and clinical markers with ophthalmological parameters to support an early diagnosis of Sjögren's syndrome in pediatric patients. Integrating TBUT and slit-lamp evaluation with serological and histopathological data may enhance diagnostic accuracy and guide timely, targeted intervention to prevent long-term complications. FAU - Del Giudice, Emanuela AU - Del Giudice E AUID- ORCID: 0000-0002-3334-4884 AD - Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy. FAU - Saturno, Maria Carmela AU - Saturno MC AUID- ORCID: 0000-0003-3081-4788 AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. FAU - Fiorino, Maria Grazia AU - Fiorino MG AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. FAU - Iannetta, Danilo AU - Iannetta D AUID- ORCID: 0000-0002-8532-4578 AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. FAU - Spadea, Luca AU - Spadea L AUID- ORCID: 0000-0002-2229-6995 AD - Post Graduate School of Public Health, University of Siena, 53100 Siena, Italy. FAU - Martucci, Vanessa AU - Martucci V AD - Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy. FAU - Marcellino, Alessia AU - Marcellino A AUID- ORCID: 0000-0001-9277-9231 AD - Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy. FAU - Sanseviero, Mariateresa AU - Sanseviero M AUID- ORCID: 0000-0001-7935-6648 AD - Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy. FAU - Mauro, Angela AU - Mauro A AUID- ORCID: 0000-0002-7008-3409 AD - Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, 20121 Milan, Italy. FAU - Carlesimo, Sandra Cinzia AU - Carlesimo SC AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. FAU - Nante, Nicola AU - Nante N AUID- ORCID: 0000-0002-2040-9562 AD - Post Graduate School of Public Health, University of Siena, 53100 Siena, Italy. AD - Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy. FAU - Guarducci, Giovanni AU - Guarducci G AUID- ORCID: 0000-0003-1469-7294 AD - Post Graduate School of Public Health, University of Siena, 53100 Siena, Italy. AD - Healthcare Management, Local Health Authority of Ferrara, 44100 Ferrara, Italy. FAU - Spadea, Leopoldo AU - Spadea L AUID- ORCID: 0000-0002-1190-3956 AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. FAU - Lubrano, Riccardo AU - Lubrano R AUID- ORCID: 0000-0001-8237-955X AD - Pediatrics and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Santa Maria Goretti Hospital, Sapienza University of Rome, Polo Pontino, 04100 Latina, Italy. FAU - Paroli, Maria Pia AU - Paroli MP AUID- ORCID: 0000-0002-4395-4774 AD - Department of Sense Organs, Sapienza University of Rome, 00161 Rome, Italy. LA - eng PT - Journal Article DEP - 20250623 PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 SB - IM MH - Humans MH - *Sjogren's Syndrome/diagnosis/complications/physiopathology MH - Retrospective Studies MH - Female MH - Male MH - Child MH - Adolescent MH - Tears PMC - PMC12300881 OTO - NOTNLM OT - ocular involvement OT - pediatric sjögren’s syndrome OT - tear break-up time COIS- The authors declare no conflicts of interest. EDAT- 2025/07/30 06:30 MHDA- 2025/07/30 06:31 PMCR- 2025/06/23 CRDT- 2025/07/30 01:03 PHST- 2025/05/05 00:00 [received] PHST- 2025/06/16 00:00 [revised] PHST- 2025/06/22 00:00 [accepted] PHST- 2025/07/30 06:31 [medline] PHST- 2025/07/30 06:30 [pubmed] PHST- 2025/07/30 01:03 [entrez] PHST- 2025/06/23 00:00 [pmc-release] AID - medicina61071128 [pii] AID - medicina-61-01128 [pii] AID - 10.3390/medicina61071128 [doi] PST - epublish SO - Medicina (Kaunas). 2025 Jun 23;61(7):1128. doi: 10.3390/medicina61071128. PMID- 28770006 OWN - NLM STAT- MEDLINE DCOM- 20180207 LR - 20181113 IS - 1757-1146 (Electronic) IS - 1757-1146 (Linking) VI - 10 DP - 2017 TI - Categorisation of foot complaints in systemic lupus erythematosus (SLE) from a New Zealand cohort. PG - 33 LID - 10.1186/s13047-017-0217-2 [doi] LID - 33 AB - BACKGROUND: Foot complaints have been shown to be common in systemic lupus erythematosus (SLE) and heterogeneous in nature. We aimed to categorize self-reported foot complaints in people with SLE and foot symptoms. METHODS: A self-administered validated questionnaire was posted to 406 people with SLE attending adult rheumatology clinics across three health boards in Auckland, New Zealand. In addition to foot pain, vascular complaints, dermatological lesions and neurological symptoms were included in the analysis. Pairwise correlations among the variables were undertaken followed by factor analysis to identify and categorise associations between reported foot complaints. RESULTS: From the questionnaires returned, 93 full datasets were analysed. Participants' were predominantly female (n = 87, 93.7%), with mean (SD) age of 50.4 (14.3) years and a mean (SD) disease duration of 13.1 (11) years. Three categories of foot complaint were determined: 'foot pain', 'skin disorders' and 'vascular insufficiency'. These three groups provided the best fit (0.91) to describe the wide range of foot complaints reported by those with SLE. Factor analysis for foot pain demonstrated a high positive loading for the inter-correlation of foot pain in past month (0.83), foot pain today (0.71), intermittent claudication (0.71), numbness (0.62), loss of balance (0.81), swelling (0.59), foot joint pain (0.77), arch pain (0.68) and tendon pain (0.77). Skin disorders demonstrated a very high positive loading for 3 factors skin rash (0.82), blistering skin rash (0.95) and foot ulceration (0.88). In vascular insufficiency a high positive loading for cold feet (0.83), chilblains (0.76) and Raynaud's phenomenon (0.70). CONCLUSIONS: This work suggests people with SLE report three independent categories of foot complaints; foot pain, skin disorders or vascular insufficiency. FAU - Otter, Simon J AU - Otter SJ AD - Health and Research Rehabilitation Institute and School of Podiatry, AUT University, Auckland, New Zealand. ISNI: 0000 0001 0705 7067. GRID: grid.252547.3 AD - School of Health Sciences, University of Brighton, 49 Darley Rd, Eastbourne, BN20 7UR UK. ISNI: 0000000121073784. GRID: grid.12477.37 FAU - Rohan, Maheswaran AU - Rohan M AD - Biostatistics and Epidemiology, AUT University, Auckland, New Zealand. ISNI: 0000 0001 0705 7067. GRID: grid.252547.3 FAU - Davies, Kevin A AU - Davies KA AD - Rheumatology Department of Brighton and Sussex Medical School, Brighton, UK. ISNI: 0000 0000 8853 076X. GRID: grid.414601.6 FAU - Kumar, Sunil AU - Kumar S AD - Rheumatology Department of Counties Manukau District Health Board New Zealand, Auckland, New Zealand. FAU - Gow, Peter AU - Gow P AD - Rheumatology Department of Counties Manukau District Health Board New Zealand, Auckland, New Zealand. FAU - Dalbeth, Nicola AU - Dalbeth N AD - Faculty of Medical and Health Sciences The University of Auckland, New Zealand and Department of Rheumatology, Auckland District Health Board, Auckland, New Zealand. ISNI: 0000 0001 0042 379X. GRID: grid.414057.3 FAU - Corkill, Michael AU - Corkill M AD - Rheumatology Department of Waitemata District Health Board New Zealand, Auckland, New Zealand. FAU - Panthakalam, Sam AU - Panthakalam S AD - Rheumatology Department of East Sussex Healthcare Trust, East Sussex, UK. FAU - Rome, Keith AU - Rome K AD - Health and Research Rehabilitation Institute and School of Podiatry, AUT University, Auckland, New Zealand. ISNI: 0000 0001 0705 7067. GRID: grid.252547.3 LA - eng PT - Journal Article DEP - 20170726 PL - United States TA - J Foot Ankle Res JT - Journal of foot and ankle research JID - 101471610 SB - IM MH - Adult MH - Aged MH - Cohort Studies MH - Female MH - Foot Diseases/*etiology MH - Humans MH - Lupus Erythematosus, Systemic/*complications MH - Male MH - Middle Aged PMC - PMC5530459 OTO - NOTNLM OT - Dermal OT - Foot complaints OT - Foot pain OT - Systemic lupus Erythematosus OT - Vascular COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Ethical approval was granted by Auckland University of Technology Ethics Committee (AUTEC 14/56). CONSENT FOR PUBLICATION: Not applicable. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2017/08/05 06:00 MHDA- 2018/02/08 06:00 PMCR- 2017/07/26 CRDT- 2017/08/04 06:00 PHST- 2017/06/11 00:00 [received] PHST- 2017/07/11 00:00 [accepted] PHST- 2017/08/04 06:00 [entrez] PHST- 2017/08/05 06:00 [pubmed] PHST- 2018/02/08 06:00 [medline] PHST- 2017/07/26 00:00 [pmc-release] AID - 217 [pii] AID - 10.1186/s13047-017-0217-2 [doi] PST - epublish SO - J Foot Ankle Res. 2017 Jul 26;10:33. doi: 10.1186/s13047-017-0217-2. eCollection 2017. PMID- 41967572 OWN - NLM STAT- In-Process LR - 20260609 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 166 DP - 2026 Jul TI - Nailfold videocapillaroscopy reveals early microvascular changes in pediatric epilepsy. PG - 104952 LID - S0026-2862(26)00052-X [pii] LID - 10.1016/j.mvr.2026.104952 [doi] AB - Nailfold videocapillaroscopy (NVC) provides a non-invasive and reproducible approach to visualize and quantify microvascular morphology. Originally standardized for evaluating Raynaud's phenomenon and systemic sclerosis, NVC has recently been applied to a broader range of non-rheumatologic conditions. In epilepsy, both the disease process and long-term exposure to anti-seizure medications (ASMs) may alter endothelial function and microvascular integrity. The present study was conducted to examine the relationship between ASM monotherapy and nailfold capillary morphology in children with epilepsy. METHODS: This cross-sectional study included 36 children with epilepsy and 38 age- and sex-matched controls. All patients received monotherapy with valproic acid (VPA), carbamazepine (CBZ), levetiracetam (LEV), oxcarbazepine (OXC), lamotrigine (LTG), or topiramate (TPM) for ≥6 months. NVC (×200 magnification) was used to assess capillary density, apical loop diameter, tortuosity, hemorrhages, and abnormal morphology. RESULTS: Compared with controls, patients exhibited significantly wider apical loop width (15.6 [13.1-17.4] μm vs. 14 [13-15] μm; p = 0.007) and a higher frequency of reduced capillary density (<7 cap/mm: 25% vs. 5.3%; p = 0.023). Dilated capillaries were also more frequent among patients (41.7% vs. 15.8%; p = 0.020). Correlation analysis demonstrated positive associations between treatment duration and apical loop width (r = 0.32, p = 0.055), dilated capillary count (r = 0.36, p = 0.043), and abnormal capillary count (r = 0.40, p = 0.025). In subgroup analyses, patients treated with VPA and CBZ had significantly lower capillary density than those receiving newer-generation ASMs (p = 0.023). In contrast, crossed capillaries were more prominent in the newer-drug group (p = 0.012). CONCLUSION: Chronic ASMs exposure, particularly valproic acid, may lead to subtle but measurable alterations in microcirculation. NVC may serve as a practical, non-invasive method for the early detection of endothelial dysfunction in pediatric epilepsy and could aid long-term vascular monitoring in this population. CI - Copyright © 2026 Elsevier Inc. All rights reserved. FAU - Perk, Peren AU - Perk P AD - Department of Pediatric Neurology, Ministry of Health University Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey; Department of Medical Physiology, Trakya University, Faculty of Medicine, Edirne, Turkey. Electronic address: perkperen@gmail.com. FAU - Çakmak, Figen AU - Çakmak F AD - Department of Pediatric Rheumatology, Ministry of Health University Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey. FAU - Koç, Rahime AU - Koç R AD - Department of Pediatric Rheumatology, Ministry of Health University Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey. FAU - Dağdelen, Zeynep Öz AU - Dağdelen ZÖ AD - Department of Pediatric Neurology, Sancaktepe Sehit Prof. Dr. Ilhan Varank Research and Training Hospital, Istanbul, Turkey. FAU - Yüksel, Arzu AU - Yüksel A AD - Department of Medical Biochemistry, Ministry of Health University Basaksehır Cam and Sakura City Hospital, Istanbul, Turkey. FAU - Demirkan, Fatma Gül AU - Demirkan FG AD - Department of Pediatric Rheumatology, University of Health Sciences Kanuni Sultan Suleyman Research and Training Hospital, Istanbul, Turkey. LA - eng PT - Journal Article DEP - 20260410 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 SB - IM OTO - NOTNLM OT - Antiseizure medication OT - Epilepsy OT - Nailfold videocapillaroscopy OT - Pediatrics COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/04/13 00:31 MHDA- 2026/04/13 00:31 CRDT- 2026/04/12 19:34 PHST- 2025/12/17 00:00 [received] PHST- 2026/03/17 00:00 [revised] PHST- 2026/04/06 00:00 [accepted] PHST- 2026/04/13 00:31 [pubmed] PHST- 2026/04/13 00:31 [medline] PHST- 2026/04/12 19:34 [entrez] AID - S0026-2862(26)00052-X [pii] AID - 10.1016/j.mvr.2026.104952 [doi] PST - ppublish SO - Microvasc Res. 2026 Jul;166:104952. doi: 10.1016/j.mvr.2026.104952. Epub 2026 Apr 10. PMID- 31464664 OWN - NLM STAT- MEDLINE DCOM- 20191007 LR - 20211204 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 37 Suppl 118 IP - 3 DP - 2019 May-Jun TI - Systemic manifestations of primary Sjögren's syndrome out of the ESSDAI classification: prevalence and clinical relevance in a large international, multi-ethnic cohort of patients. PG - 97-106 AB - OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon. FAU - Retamozo, Soledad AU - Retamozo S AD - Inst. De Investigaciones En Ciencias De La Salud (INICSA), Universidad Nacional de Córdoba (UNC), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Córdoba, and Inst. Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Argentina. FAU - Acar-Denizli, Nihan AU - Acar-Denizli N AD - Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey. FAU - Rasmussen, Astrid AU - Rasmussen A AD - Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA. FAU - Horváth, Ildikó Fanny AU - Horváth IF AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary. FAU - Baldini, Chiara AU - Baldini C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Priori, Roberta AU - Priori R AD - Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy. FAU - Sandhya, Pulukool AU - Sandhya P AD - Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India. FAU - Hernandez-Molina, Gabriela AU - Hernandez-Molina G AD - Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Subirán, México City, Mexico. FAU - Armagan, Berkan AU - Armagan B AD - Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey. FAU - Praprotnik, Sonja AU - Praprotnik S AD - Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia. FAU - Kvarnstrom, Marika AU - Kvarnstrom M AD - Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden. FAU - Gerli, Roberto AU - Gerli R AD - Rheumatology Unit, Department of Medicine, University of Perugia, Italy. FAU - Sebastian, Agata AU - Sebastian A AD - Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland. FAU - Solans, Roser AU - Solans R AD - Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Rischmueller, Maureen AU - Rischmueller M AD - Department of Rheumatology, The Queen Elizabeth Hospital and University of Adelaide, South Australia, Australia. FAU - Pasoto, Sandra G AU - Pasoto SG AD - Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil. FAU - Valim, Valeria AU - Valim V AD - Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil. FAU - Nordmark, Gunnel AU - Nordmark G AD - Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden. FAU - Kruize, Aike AU - Kruize A AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Nakamura, Hideki AU - Nakamura H AD - Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. FAU - Hofauer, Benedikt AU - Hofauer B AD - Otorhinolaryngology, Head and Neck Surgery, Technical University Munich, Munich, Germany. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila, Italy. FAU - Fernandes Moça Trevisani, Virginia AU - Fernandes Moça Trevisani V AD - Federal University of São Paulo, Brazil. FAU - Devauchelle-Pensec, Valerie AU - Devauchelle-Pensec V AD - Rheumatology Department, Brest University Hospital, France. FAU - Atzeni, Fabiola AU - Atzeni F AD - IRCCS Galeazzi Orthopedic Institute, Milan and Rheumatology Unit, University of Messina, Italy. FAU - Gheita, Tamer A AU - Gheita TA AD - Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Egypt. FAU - Consani-Fernández, Sandra AU - Consani-Fernández S AD - Internal Medicine, Hospital Maciel, and Universidad de la República (UdelaR), Montevideo, Uruguay. FAU - Szántó, Antonia AU - Szántó A AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary. FAU - Sivils, Kathy AU - Sivils K AD - Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA. FAU - Gattamelata, Angelina AU - Gattamelata A AD - Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy. FAU - Danda, Debashish AU - Danda D AD - Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India. FAU - Kilic, Levent AU - Kilic L AD - Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey. FAU - Bartoloni, Elena AU - Bartoloni E AD - Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. FAU - Bombardieri, Stefano AU - Bombardieri S AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Sánchez-Guerrero, Jorge AU - Sánchez-Guerrero J AD - Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Subirán, México City, Mexico. FAU - Wahren-Herlenius, Marie AU - Wahren-Herlenius M AD - Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden. FAU - Mariette, Xavier AU - Mariette X AD - Centre for Immunology of Viral Infections and Autoimmune Diseases, Université Paris-Sud, INSERM UMR1184, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, France. FAU - Ramos-Casals, Manuel AU - Ramos-Casals M AD - Sjögren's Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Spain. mramos@clinic.cat. FAU - Brito-Zerón, Pilar AU - Brito-Zerón P AD - Sjögren's Syndrome Res. Group (AGAUR), Lab. of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Dept. of Autoimmune Diseases, ICMiD, Univ. of Barcelona, Hospital Clínic, and Autoimmune Diseases Unit, Dept. of Medicine, Hosp. CIMA-Sanitas, Barcelona, Spain. CN - Sjögren Big Data Consortium LA - eng PT - Journal Article DEP - 20190828 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Cohort Studies MH - Ethnicity MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Registries MH - Retrospective Studies MH - Severity of Illness Index MH - *Sjogren's Syndrome/diagnosis/physiopathology EDAT- 2019/08/30 06:00 MHDA- 2019/10/08 06:00 CRDT- 2019/08/30 06:00 PHST- 2019/04/15 00:00 [received] PHST- 2019/07/01 00:00 [accepted] PHST- 2019/08/30 06:00 [entrez] PHST- 2019/08/30 06:00 [pubmed] PHST- 2019/10/08 06:00 [medline] AID - 14220 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2019 May-Jun;37 Suppl 118(3):97-106. Epub 2019 Aug 28. PMID- 35547355 OWN - NLM STAT- MEDLINE DCOM- 20220517 LR - 20220716 IS - 2314-6141 (Electronic) IS - 2314-6133 (Print) VI - 2022 DP - 2022 TI - Clinical, Serological, and Genetic Characteristics of a Hungarian Myositis-Scleroderma Overlap Cohort. PG - 6251232 LID - 10.1155/2022/6251232 [doi] LID - 6251232 AB - Overlap myositis is a distinct subgroup of idiopathic inflammatory myositis (IIM) with various clinical phenotypes. The aim of this study was to determine the clinical, serological, and genetic features of systemic sclerosis (SSc)-IIM overlap patients. It was a retrospective study using clinical database of 39 patients, fulfilling both the criteria of SSc and IIM. 56.4% of the patients had limited cutaneous, 43.6% had diffuse cutaneous SSc, whereas 7.7% of the patients had dermatomyositis and 92.3% polymyositis. The two diseases occurred simultaneously in 58.97%, while 10.26% in myositis and 30.77% in scleroderma were initially diagnosed. The frequencies of organ involvement were interstitial lung disease 71.8%, dysphagia 66.7%, cardiac involvement 41%, pulmonary arterial hypertension (PAH) 30.8%, and renal involvement 12.8%, respectively. The presence of human leukocyte antigen (HLA) - DRB1∗03 and DQA1∗051∗01 alleles were significantly higher in the overlap patients than in healthy controls (82.35% vs. 27.54%; p < 0.0001 and 88.24% vs. 30.16; p < 0.0001). Certain clinical parameters, such as fever at diagnosis (41.67% vs. 7.41%, p = 0.0046), cardiac involvement (83.33% vs. 22.22%, p = 0.0008), subcutaneous calcinosis (41.66 vs. 11.11, p = 0.01146), and claw hand deformity (25% vs. 11.11%, p = 0.00016) were significantly associated with the presence of PAH. Upon comparison, the overlap patients and anti-Jo-1 positive antisynthetase patients showed similarities in terms of genetic results and major clinical features; however, SSc-IIM overlap patients could be distinguished with higher erythrocyte sedimentation rate (ESR) level, more frequent presence of Raynaud's phenomenon (p < 0.0001; OR: 20.00), dysphagia (p < 0.0001; OR: 15.63), and infrequent livedo reticularis (p < 0.01; OR: 0.11). SSc-IIM overlap myositis is a unique group within IIM-s possessing characteristic clinical features. CI - Copyright © 2022 Katalin Szabó et al. FAU - Szabó, Katalin AU - Szabó K AUID- ORCID: 0000-0002-0617-1703 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Bodoki, Levente AU - Bodoki L AUID- ORCID: 0000-0002-4934-6032 AD - Division of Rheumatology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Nagy-Vincze, Melinda AU - Nagy-Vincze M AUID- ORCID: 0000-0003-0316-3828 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Béldi, Tibor AU - Béldi T AUID- ORCID: 0000-0003-4972-1370 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Vincze, Anett AU - Vincze A AUID- ORCID: 0000-0003-3654-9268 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Zilahi, Erika AU - Zilahi E AUID- ORCID: 0000-0002-7376-8921 AD - Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Nagyerdei krt. 98, 4032 Debrecen, Hungary. FAU - Varga, József AU - Varga J AUID- ORCID: 0000-0001-5797-9625 AD - Department of Medical Imaging, Division of Nuclear Medicine, University of Debrecen, Debrecen, Hungary. FAU - Szűcs, Gabriella AU - Szűcs G AUID- ORCID: 0000-0002-0958-9531 AD - Division of Rheumatology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Dankó, Katalin AU - Dankó K AUID- ORCID: 0000-0002-8729-3947 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. FAU - Griger, Zoltán AU - Griger Z AUID- ORCID: 0000-0002-1371-7911 AD - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zs. krt. 22, 4032 Debrecen, Hungary. LA - eng PT - Journal Article DEP - 20220502 PL - United States TA - Biomed Res Int JT - BioMed research international JID - 101600173 RN - 0 (HLA-DRB1 Chains) SB - IM MH - *Deglutition Disorders MH - HLA-DRB1 Chains MH - Humans MH - Hungary MH - *Myositis/genetics MH - Retrospective Studies MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/complications/genetics PMC - PMC9085307 COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/05/14 06:00 MHDA- 2022/05/18 06:00 PMCR- 2022/05/02 CRDT- 2022/05/13 11:12 PHST- 2022/02/23 00:00 [received] PHST- 2022/04/12 00:00 [accepted] PHST- 2022/05/13 11:12 [entrez] PHST- 2022/05/14 06:00 [pubmed] PHST- 2022/05/18 06:00 [medline] PHST- 2022/05/02 00:00 [pmc-release] AID - 10.1155/2022/6251232 [doi] PST - epublish SO - Biomed Res Int. 2022 May 2;2022:6251232. doi: 10.1155/2022/6251232. eCollection 2022. PMID- 32988710 OWN - NLM STAT- MEDLINE DCOM- 20210908 LR - 20210908 IS - 1873-2364 (Electronic) IS - 0960-8966 (Linking) VI - 30 IP - 10 DP - 2020 Oct TI - Biallelic mutations in Tenascin-X cause classical-like Ehlers-Danlos syndrome with slowly progressive muscular weakness. PG - 833-838 LID - S0960-8966(20)30567-8 [pii] LID - 10.1016/j.nmd.2020.09.002 [doi] AB - Tenascin-X, is an extracellular matrix glycoprotein expressed in skin, muscle, tendons, and blood vessels with an anti-adhesive function. Biallelic Tenascin-X mutations cause classical-like Ehlers-Danlos syndrome. We report a 46-year-old woman with slowly progressive weakness of the lower limbs and myalgia from age 28 years. In the past she had Raynaud's phenomenon, multiple sprains and joint dislocations, conjunctival haemorrhages and a colonic perforation during colonoscopy. Neurologic examination showed moderate asymmetric proximal and axial muscular weakness, distal amyotrophy of 4 limbs, moderate skin hyperextensibility, and hypermobility of distal joints of fingers. Whole body Magnetic Resonance Imaging showed symmetric fatty infiltration of thigh and leg muscles, with predominant atrophy of thighs. Next Generation Sequencing revealed two pathogenic TNXB variants, g.32024681C>G, c.7826-1G>C, and g.32016181dup, c.9998dupA, p.(Asn3333Lysfs*35). Western Blot and immunofluorescence studies confirmed a marked Tenascin-X reduction in both patient's serum and muscle. Here we further detail the clinical and genetic spectrum of a patient with classical-like Ehlers-Danlos syndrome and prominent muscle involvement. CI - Copyright © 2020 Elsevier B.V. All rights reserved. FAU - Brisset, Marion AU - Brisset M AD - APHP, Department of Neurology, Raymond Poincaré Hospital, Centre de Référence de Pathologie Neuromusculaire Nord-Est-Ile-de-France, 104 Bld Raymond Poincaré, 92380 Garches, France. FAU - Metay, Corinne AU - Metay C AD - APHP, Centre de Génétique Moléculaire et Chromosomique, Service de Biochimie Métabolique, U.F de Cardiogénétique et Myogénétique Moléculaire et Cellulaire, Groupe Hospitalier La Pitié-Salpêtrière Charles Foix, Paris, France. FAU - Carlier, Robert-Yves AU - Carlier RY AD - APHP, Medical imaging Department, Raymond Poincaré teaching Hospital, Centre de Référence de Pathologie Neuromusculaire Nord-Est-Ile-de-France, GHU PIFO, 104 Bld Raymond Poincaré, 92380 Garches, France. FAU - Badosa, Carmen AU - Badosa C AD - Department of Dermatology, Radboud University Medical Center, Nijmegen, the Netherlands. FAU - Marques, Caterina AU - Marques C AD - Department of Dermatology, Radboud University Medical Center, Nijmegen, the Netherlands. FAU - Schalkwijk, Joost AU - Schalkwijk J AD - Neuromuscular Unit, Neuropaediatrics Department, Institut de Recerca Sant Joan de Déu Hospital Sant Joan de Deu, Barcelona Spain. FAU - vanVlijmen-Willems, Ivonne AU - vanVlijmen-Willems I AD - Neuromuscular Unit, Neuropaediatrics Department, Institut de Recerca Sant Joan de Déu Hospital Sant Joan de Deu, Barcelona Spain. FAU - Jimenez-Mallebrera, Cecilia AU - Jimenez-Mallebrera C AD - Neuromuscular Unit, Neuropaediatrics Department, Institut de Recerca Sant Joan de Déu Hospital Sant Joan de Deu, Barcelona Spain; U703 Center for Biomedical Research on Rare Diseases (CIBERER), Instituto de Salud Carlos III, Spain; Department of Genetics, Microbiology and Statistics, University of Barcelona, Spain. FAU - Keren, Boris AU - Keren B AD - AP-HP, Hôpital Pitié-Salpêtrière, Département de Génétique et de Cytogénétique, Unité fonctionnelle de cytogénétique, Paris, France. FAU - Jobic, Valérie AU - Jobic V AD - APHP, Centre de Génétique Moléculaire et Chromosomique, Service de Biochimie Métabolique, U.F de Cardiogénétique et Myogénétique Moléculaire et Cellulaire, Groupe Hospitalier La Pitié-Salpêtrière Charles Foix, Paris, France. FAU - Laforêt, Pascal AU - Laforêt P AD - APHP, Department of Neurology, Raymond Poincaré Hospital, Centre de Référence de Pathologie Neuromusculaire Nord-Est-Ile-de-France, 104 Bld Raymond Poincaré, 92380 Garches, France; Service de Neurologie, U1179 UVSQ-INSERM Handicap Neuromusculaire : Physiologie, Biothérapie et Pharmacologie appliquées, UFR Simone Veil-Santé, Université Versailles Saint Quentin en Yvelines, Pôle neuro-locomoteur, Hôpital Raymond Poincaré, 104 boulevard Raymond Poincaré, 92380 Paris-Saclay, France. FAU - Malfatti, Edoardo AU - Malfatti E AD - APHP, Department of Neurology, Raymond Poincaré Hospital, Centre de Référence de Pathologie Neuromusculaire Nord-Est-Ile-de-France, 104 Bld Raymond Poincaré, 92380 Garches, France; Service de Neurologie, U1179 UVSQ-INSERM Handicap Neuromusculaire : Physiologie, Biothérapie et Pharmacologie appliquées, UFR Simone Veil-Santé, Université Versailles Saint Quentin en Yvelines, Pôle neuro-locomoteur, Hôpital Raymond Poincaré, 104 boulevard Raymond Poincaré, 92380 Paris-Saclay, France. Electronic address: edoardo.malfatti@aphp.fr. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200907 PL - England TA - Neuromuscul Disord JT - Neuromuscular disorders : NMD JID - 9111470 RN - 0 (Tenascin) RN - 0 (tenascin X) SB - IM MH - *Disease Progression MH - Ehlers-Danlos Syndrome/complications/diagnosis/*genetics/*physiopathology MH - Female MH - Humans MH - Magnetic Resonance Imaging MH - Middle Aged MH - Muscle Weakness/diagnosis/etiology/*physiopathology MH - Pedigree MH - Tenascin/*genetics OTO - NOTNLM OT - Myopathy OT - TNXB gene OT - Tenascin X OT - Whole body MRI EDAT- 2020/09/30 06:00 MHDA- 2021/09/09 06:00 CRDT- 2020/09/29 05:29 PHST- 2020/01/16 00:00 [received] PHST- 2020/09/01 00:00 [revised] PHST- 2020/09/02 00:00 [accepted] PHST- 2020/09/30 06:00 [pubmed] PHST- 2021/09/09 06:00 [medline] PHST- 2020/09/29 05:29 [entrez] AID - S0960-8966(20)30567-8 [pii] AID - 10.1016/j.nmd.2020.09.002 [doi] PST - ppublish SO - Neuromuscul Disord. 2020 Oct;30(10):833-838. doi: 10.1016/j.nmd.2020.09.002. Epub 2020 Sep 7. PMID- 31623944 OWN - NLM STAT- MEDLINE DCOM- 20200928 LR - 20200928 IS - 1578-1267 (Electronic) IS - 0301-0546 (Linking) VI - 48 IP - 1 DP - 2020 Jan-Feb TI - Staining of antinuclear antibodies and antibodies against removable nuclear antigens in connective tissue diseases. PG - 18-25 LID - S0301-0546(19)30105-3 [pii] LID - 10.1016/j.aller.2019.07.002 [doi] AB - INTRODUCTION AND OBJECTIVES: Connective tissue diseases are inflammatory, autoimmune diseases and threaten quality of life. To determine the relationship between staining patterns of antinuclear antibodies and antibodies against extractable nuclear antigens in patients with connective tissue disease. MATERIALS AND METHODS: Observational, basic, analytical and transversal study. Study conducted in the Immunology Service of the Arzobispo Loayza National Hospital between January 2017 and June 2017. We analyzed 291 samples of patients with CTD and for the detection of anti-nuclear antibody staining patterns, the immunological kit and observation with microscope of at 40X Immunofluorescence and for the detection of the antibodies against extractable nuclear antigens. The Immunoblot method was employed. Statistical analyses were carried out with the statistical package SPSS version 21 for Windows. We used the Pearson Chi-square test for the categorical variables, a value of p<0.05 was considered significant. RESULTS: There was a significant relationship p<0.05 of the homogeneous pattern, the mottled pattern with Anti-histones (p=0.000), Anti-nucleosomes (p=0.000), Anti-Ro 52 (p=0.000), Anti-SSA (p=0.001), Anti-SSB (p=0.003), Anti-dsDNA (p=0.000) with the Pearson Chi-square test. There was a significant relationship of p<0.05 of the centromeric pattern with Anti-Cenp B (p=0.000) with Fisher⿿s exact statistic. CONCLUSIONS: There was a significant relationship between the anti-nuclear antibody staining patterns and the antibodies to the core extractable antigens in patients with systemic lupus erythematosus, Sjögren⿿s syndrome, Calcinosis, Raynaud⿿s phenomenon, esophageal Dysmotility, sclerodactyly and Telangiectasia (CREST), Scleroderma and Polymyositis. CI - Copyright © 2019 SEICAP. Published by Elsevier España, S.L.U. All rights reserved. FAU - Oliva Menacho, José Enrique AU - Oliva Menacho JE AD - Faculty of Medicine, Cayetano Heredia Peruvian University, Lima, Peru; Postgraduate unit of the Faculty of Medicine of the National Major University of San Marcos, Arzobispo Loayza National Hospital, Lima, Peru. Electronic address: jose.enrique.oliva@hotmail.com. FAU - Arroyo-Acevedo, Jorge Luis AU - Arroyo-Acevedo JL AD - Institute of Clinical Research, Faculty of Medicine, National University of San Marcos, Lima, Peru; Laboratory of Experimental Pharmacology, Faculty of Medicine, National University of San Marcos, Lima, Peru. FAU - Oliva-Candela, José Arturo AU - Oliva-Candela JA AD - Arzobispo Loayza National Hospital, Lima, Peru. FAU - García-Hjarles, Marco Antonio AU - García-Hjarles MA AD - Faculty of Sciences and philosophy, Faculty of Medicine, Cayetano Heredia Peruvian University, Lima, Peru. FAU - Domínguez-Huarcaya, Lester AU - Domínguez-Huarcaya L AD - Institute of Clinical Research, Faculty of Medicine, National University of San Marcos. Faculty of Medicine of the University Norbert Wiener, Lima, Peru. LA - eng PT - Journal Article PT - Observational Study DEP - 20191014 PL - Singapore TA - Allergol Immunopathol (Madr) JT - Allergologia et immunopathologia JID - 0370073 RN - 0 (Antibodies, Antinuclear) RN - 0 (Antigens, Nuclear) RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Antibodies, Antinuclear/*blood MH - Antigens, Nuclear/immunology MH - Autoantibodies/*blood MH - Autoimmune Diseases/immunology MH - Connective Tissue Diseases/*immunology MH - Cross-Sectional Studies MH - Female MH - Fluorescent Antibody Technique, Indirect MH - Humans MH - Immunoblotting MH - Male MH - Middle Aged MH - Peru/epidemiology OTO - NOTNLM OT - Anti-Nuclear antibody staining pattern OT - Antibodies against extractable nuclear antigens OT - Connective tissue diseases OT - Immunoblot OT - Indirect immunofluorescence EDAT- 2019/10/19 06:00 MHDA- 2020/09/29 06:00 CRDT- 2019/10/19 06:00 PHST- 2019/05/24 00:00 [received] PHST- 2019/07/26 00:00 [accepted] PHST- 2019/10/19 06:00 [pubmed] PHST- 2020/09/29 06:00 [medline] PHST- 2019/10/19 06:00 [entrez] AID - S0301-0546(19)30105-3 [pii] AID - 10.1016/j.aller.2019.07.002 [doi] PST - ppublish SO - Allergol Immunopathol (Madr). 2020 Jan-Feb;48(1):18-25. doi: 10.1016/j.aller.2019.07.002. Epub 2019 Oct 14. PMID- 30872612 OWN - NLM STAT- MEDLINE DCOM- 20200930 LR - 20211204 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 9 IP - 1 DP - 2019 Mar 14 TI - Epidermal Growth Factor Like-domain 7 and miR-126 are abnormally expressed in diffuse Systemic Sclerosis fibroblasts. PG - 4589 LID - 10.1038/s41598-019-39485-8 [doi] LID - 4589 AB - Systemic sclerosis (SSc) is characterized by microangiopathy with impaired reparative angiogenesis and fibrosis. Epidermal Growth Factor Like-domain 7 (EGFL7), firstly described in endothelial cells plays a pivotal role in angiogenesis. Fibroblasts (FBs) are involved in vascular remodeling, under physiological and pathological conditions. In this study, we investigated: (i) the expression of EGFL7 and its miR-126 in patients affected by diffuse cutaneous SSc (dcSSc); (ii) the ability of Transforming Growth Factor-beta (TGF-β) to modulate EGFL7 expression; (iii) the ability of EGFL7 to modulate COL1A1 expression and proliferation/migration, and (iv) the functional role of EGFL7 on angiogenesis. Patients were divided in 2 subsets: patients fulfilling the classification criteria in less than one year from Raynaud's Phenomenon onset (Early Onset Subset-EOS), and all the others (Long Standing Subset-LSS). We show that EGFL7 expression is increased in EOS dcSSc skin and cultured FBs. EGFL7 is inducible by TGF-β on Healthy Controls (HC) FBs but not in SSc-FBs. EGFL7 decreases COL1A1 expression in EOS SSc-FBs while EGFL7 silencing up-regulates COL1A1 expression. EGFL7 promotes migration/invasion of EOS SSc-FBs but not proliferation. Finally, SSc-FBs, partially inhibit angiogenesis in organotypic coculture assays, and this is reversed by treatment with human recombinant (rh)EGFL7. We conclude that EGFL7 and its specific microRNA miR-126 may be involved in the pathogenesis of SSc vasculopathy and fibrosis. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. vasiliki.liakouli@univaq.it. FAU - Cipriani, Paola AU - Cipriani P AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Di Benedetto, Paola AU - Di Benedetto P AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Panzera, Noemi AU - Panzera N AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Pantano, Ilenia AU - Pantano I AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Berardicurti, Onorina AU - Berardicurti O AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Carubbi, Francesco AU - Carubbi F AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. FAU - Esteves, Filomena AU - Esteves F AD - Leeds Institute of Cancer and Pathology, University of Leeds, Leeds, UK. FAU - Mavria, Georgia AU - Mavria G AD - Signal Transduction and Tumor Microenvironment Group, Leeds Institute of Cancer and Pathology, University of Leeds, Leeds, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Division of Rheumatic and Musculoskeletal Diseases, Leeds Institute of Molecular Medicine, University of Leeds, Leeds, UK. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190314 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Biomarkers) RN - 0 (Calcium-Binding Proteins) RN - 0 (Collagen Type I) RN - 0 (Collagen Type I, alpha 1 Chain) RN - 0 (EGF Family of Proteins) RN - 0 (EGFL7 protein, human) RN - 0 (MIRN126 microRNA, human) RN - 0 (MicroRNAs) RN - 0 (RNA, Messenger) SB - IM MH - Biomarkers MH - Biopsy MH - Calcium-Binding Proteins/*genetics/metabolism MH - Collagen Type I/genetics/metabolism MH - Collagen Type I, alpha 1 Chain MH - Disease Susceptibility MH - EGF Family of Proteins/*genetics/metabolism MH - Fibroblasts/*metabolism/pathology MH - *Gene Expression Regulation MH - Humans MH - Immunohistochemistry MH - MicroRNAs/*genetics MH - Neovascularization, Pathologic/genetics/metabolism MH - RNA, Messenger MH - Scleroderma, Systemic/*etiology/*pathology MH - Skin/metabolism/pathology PMC - PMC6418261 COIS- The authors declare no competing interests. EDAT- 2019/03/16 06:00 MHDA- 2020/10/02 06:00 PMCR- 2019/03/14 CRDT- 2019/03/16 06:00 PHST- 2017/11/03 00:00 [received] PHST- 2018/12/12 00:00 [accepted] PHST- 2019/03/16 06:00 [entrez] PHST- 2019/03/16 06:00 [pubmed] PHST- 2020/10/02 06:00 [medline] PHST- 2019/03/14 00:00 [pmc-release] AID - 10.1038/s41598-019-39485-8 [pii] AID - 39485 [pii] AID - 10.1038/s41598-019-39485-8 [doi] PST - epublish SO - Sci Rep. 2019 Mar 14;9(1):4589. doi: 10.1038/s41598-019-39485-8. PMID- 28966213 OWN - NLM STAT- MEDLINE DCOM- 20180528 LR - 20180528 IS - 1349-3329 (Electronic) IS - 0040-8727 (Linking) VI - 243 IP - 1 DP - 2017 Sep TI - Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis. PG - 77-83 LID - 10.1620/tjem.243.77 [doi] AB - Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea. We diagnosed her as having systemic sclerosis complicated with sarcoidosis. Chest computed tomography (CT) and transbronchial lung biopsy showed the findings of pulmonary fibrosis without PAP. We treated her with corticosteroid and intravenous cyclophosphamide therapy, followed by tacrolimus therapy. Thereafter, her symptoms improved except for exertional dyspnea, and she began to complain of productive cough thirteen months after corticosteroid and immunosuppressant therapy. On the second admission, a chest CT scan detected the emergence of crazy-paving pattern in bilateral upper lobes. Bronchoalveolar lavage (BAL) fluid with milky appearance and a lung biopsy specimen revealed acellular periodic acid-schiff stain-positive bodies. The serum titer of anti-granulocyte macrophage colony stimulating factor (GM-CSF) antibodies was elevated on first admission and remained high on second admission. We thus diagnosed her as having autoimmune PAP. Reducing the dose of immunosuppressive agents and repeating the segmental BAL resulted in the improvement of her symptoms and radiological findings. Immunosuppressant therapy may trigger the onset of autoimmune PAP in a subset of patients with systemic sclerosis and/or sarcoidosis. FAU - Yamasue, Mari AU - Yamasue M AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Nureki, Shin-Ichi AU - Nureki SI AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Usagawa, Yuko AU - Usagawa Y AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Ono, Tomoko AU - Ono T AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Matsumoto, Hiroyuki AU - Matsumoto H AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Kan, Takamasa AU - Kan T AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. FAU - Kadota, Jun-Ichi AU - Kadota JI AD - Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine. LA - eng PT - Case Reports PT - Journal Article PL - Japan TA - Tohoku J Exp Med JT - The Tohoku journal of experimental medicine JID - 0417355 RN - 0 (Autoantibodies) RN - 83869-56-1 (Granulocyte-Macrophage Colony-Stimulating Factor) RN - Pulmonary Alveolar Proteinosis, Acquired SB - IM MH - Autoantibodies/*blood MH - Autoimmune Diseases/*blood/*complications/diagnostic imaging/pathology MH - Female MH - Granulocyte-Macrophage Colony-Stimulating Factor/*immunology MH - Humans MH - Middle Aged MH - Pulmonary Alveolar Proteinosis/*blood/*complications/diagnostic imaging/pathology MH - Radiography, Thoracic MH - Sarcoidosis/*blood/*complications/diagnostic imaging/pathology MH - Scleroderma, Systemic/*blood/*complications/diagnostic imaging/pathology MH - Tomography, X-Ray Computed OTO - NOTNLM OT - anti-granulocyte macrophage colony stimulating factor antibodies OT - autoimmune pulmonary alveolar proteinosis OT - immunosuppressant therapy OT - sarcoidosis OT - systemic sclerosis EDAT- 2017/10/03 06:00 MHDA- 2018/05/29 06:00 CRDT- 2017/10/03 06:00 PHST- 2017/10/03 06:00 [entrez] PHST- 2017/10/03 06:00 [pubmed] PHST- 2018/05/29 06:00 [medline] AID - 10.1620/tjem.243.77 [doi] PST - ppublish SO - Tohoku J Exp Med. 2017 Sep;243(1):77-83. doi: 10.1620/tjem.243.77. PMID- 19116719 OWN - NLM STAT- MEDLINE DCOM- 20090924 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 29 IP - 9 DP - 2009 Jul TI - Potential biomarkers for detecting pulmonary arterial hypertension in patients with systemic sclerosis. PG - 1017-24 LID - 10.1007/s00296-008-0829-8 [doi] AB - Pulmonary arterial hypertension (PAH) is the major complication of systemic sclerosis (SSc) and the main cause of morbi-mortality. It is important to find predictors for this vascular problem. The objective of this study was to determine the serum levels of different biomarkers in patients with SSc and secondary PAH and to compare them with those of healthy control subjects to define their potential role as predictors of PAH. Cross-section study in which 20 patients with SSc were included. PAH was diagnosed by echocardiogram. The optical densities of endoglin (Eng), endothelin-1 (ET-1), platelet-derived growth factor (PDGF), tumoral necrosis factor alpha (TNF-alpha), Transforming growth factor beta 2 (TGF-beta2) and Interleukin 8 (IL-8) were measured in 20 patients with SSc and 20 healthy controls matched by sex. The differences found between the group of patients with PAH and the control group were (mean or median and range): ET-1 (0.20; 0.10-0.35 vs. 0.16; 0.10-0.24; P = 0.0276), IL-8 (195.7; 45.5-504 vs. 118.9; 23-299.5; P = 0.0364), TNF-alpha (0.70; 0.50-0.96 vs. 0.48; 0.38-0.65; P = 1 x 10(-8)) and Eng (0.95; 0.57-1.72 vs. 0.75; 0.57-0.89; P = 0.0028). A correlation was found between the progression of the disease and the development of Raynaud's phenomenon (Rho: 0.67 and P = 0.0011), ET-1 and Eng (Rho: 0.53 and P = 0.0196), and between IL-8 and Eng (Rho: 0.68 and P = 0.0019). In conclusions, the elevation of the serum levels of Eng and ET-1 could represent a useful tool as PAH biomarkers. Nevertheless, the diagnostic value of these markers needs to be determined by prospective studies. FAU - Coral-Alvarado, Paola AU - Coral-Alvarado P AD - Rheumatology Unit, Universidad Nacional de Colombia, Carrera 30 calle 45, Bogotá, Colombia. pxcoral@yahoo.com FAU - Quintana, Gerardo AU - Quintana G FAU - Garces, Maria Fernanda AU - Garces MF FAU - Cepeda, Libia Alexandra AU - Cepeda LA FAU - Caminos, Jorge Eduardo AU - Caminos JE FAU - Rondon, Federico AU - Rondon F FAU - Iglesias-Gamarra, Antonio AU - Iglesias-Gamarra A FAU - Restrepo, Jose Felix AU - Restrepo JF LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20081231 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antigens, CD) RN - 0 (Biomarkers) RN - 0 (ENG protein, human) RN - 0 (Endoglin) RN - 0 (Endothelin-1) RN - 0 (Receptors, Cell Surface) SB - IM MH - Adult MH - Age of Onset MH - Aged MH - *Antigens, CD MH - Biomarkers/blood MH - Case-Control Studies MH - Cross-Sectional Studies MH - Echocardiography/adverse effects MH - Endoglin MH - *Endothelin-1 MH - Female MH - Humans MH - Hypertension, Pulmonary/*diagnosis/etiology/physiopathology MH - Male MH - Receptors, Cell Surface MH - Scleroderma, Systemic/*complications/*diagnosis/physiopathology EDAT- 2009/01/01 09:00 MHDA- 2009/09/25 06:00 CRDT- 2009/01/01 09:00 PHST- 2008/06/05 00:00 [received] PHST- 2008/12/12 00:00 [accepted] PHST- 2009/01/01 09:00 [entrez] PHST- 2009/01/01 09:00 [pubmed] PHST- 2009/09/25 06:00 [medline] AID - 10.1007/s00296-008-0829-8 [doi] PST - ppublish SO - Rheumatol Int. 2009 Jul;29(9):1017-24. doi: 10.1007/s00296-008-0829-8. Epub 2008 Dec 31. PMID- 33025900 OWN - NLM STAT- MEDLINE DCOM- 20201027 LR - 20220531 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 38 Suppl 126 IP - 4 DP - 2020 Jul-Aug TI - Digestive involvement in primary Sjögren's syndrome: analysis from the Sjögrenser registry. PG - 110-115 AB - OBJECTIVES: Digestive involvement (DI) has been reported in 10-30% of primary Sjögren's syndrome (pSS) patients, and few studies have systematically analysed the prevalence of DI in pSS patients. The aim of this study was to describe DI prevalence in pSS patients from the Sjögrenser Study, and to analyse its clinical associations. METHODS: All patients included in the Sjögrenser study, a Spanish multicentre randomised cohort, containing demographic, clinical and histologic data, have been analysed retrospectively. Patients were classified according to the presence of DI (oesophageal, gastric, intestinal, hepatic and pancreatic), and we have performed DI clinical associations, descriptive statistics, Student t or χ2 test, and uni and multivariate logistic regression. RESULTS: From 437 included patients, 95% were women, with a median age of 58 years, 71 (16.2%) presented DI: 21 (29.5%) chronic atrophic gastritis, 12 (16.9%) oesophageal motility dysfunction, 3 (4.2%) lymphocytic colitis, 18 (25.3%) primary biliary cholangitis, 15 (21.1%) autoimmune hepatitis, 7 (9.8%) pancreatic involvement and 5 (7%) coeliac disease. Half of them developed DI at the same time or after pSS diagnosis. Patients with DI were significantly older at pSS diagnosis (p=0.032), more frequently women (p=0.009), presented more autoimmune hypothyroidism and C3 hypocomplementaemia (p=0.040), and were treated more frequently with glucocorticoids, immunosuppressant and biologic therapies. Patients with pancreatic involvement presented more central nervous system and renal involvement, Raynaud's phenomenon, lymphoma and C3/C4 hypocomplementaemia. CONCLUSIONS: DI is frequent in Sjögrenser patients, mainly in the form of autoimmune disorders, and seem to be associated with a more severe phenotype. Our results suggest that DI should be evaluated in pSS patients, especially those with more severe disease. FAU - Melchor, Sheila AU - Melchor S AD - Rheumatology Department, Hospital Universitario Doce de Octubre, Madrid, Spain. sheila.melchor@salud.madrid.org. FAU - Sánchez-Piedra, Carlos AU - Sánchez-Piedra C AD - Unidad de Investigación de la SER, Madrid, Spain. FAU - Fernández Castro, Mónica AU - Fernández Castro M AD - Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain. FAU - Andreu, Jose Luis AU - Andreu JL AD - Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain. FAU - Martínez Taboada, Víctor AU - Martínez Taboada V AD - Hospital Universitario Marqués de Valdecilla, Facultad de Medicina Universidad de Cantabria, Santander, Spain. FAU - Olivé, Alejandro AU - Olivé A AD - Hospital Germans Trias i Pujol, Barcelona, Spain. FAU - Rosas, José AU - Rosas J AD - Hospital Marina Baixa, Alicante, Spain. FAU - Menor, Raúl AU - Menor R AD - Hospital General Jerez de la Frontera, Cádiz, Spain. FAU - García-Aparicio, Ángel AU - García-Aparicio Á AD - Hospital Virgen de la Salud, Toledo, Spain. FAU - López Longo, Francisco Javier AU - López Longo FJ AD - Hospital Universitario Gregorio Marañón, Madrid, Spain. FAU - Manrique-Arija, Sara AU - Manrique-Arija S AD - Hospital Universitario Carlos Haya, Málaga, Spain. FAU - García Vadillo, Jesus Alberto AU - García Vadillo JA AD - Hospital Universitario de la Princesa, Madrid, Spain. FAU - López González, Ruth AU - López González R AD - Hospital Virgen de la Concha, Zamora, Spain. FAU - Narváez, Javier AU - Narváez J AD - Hospital Universitario de Bellvitge, Barcelona, Spain. FAU - Galisteo, Carlos AU - Galisteo C AD - Hospital Universitario Parc-Taulí, Barcelona, Spain. FAU - González Martín, Jorge AU - González Martín J AD - Hospital Universitario Madrid Norte Sanchinarro, Madrid, Spain. FAU - Naranjo, Antonio AU - Naranjo A AD - Hospital Universitario Doctor Negrín, Las Palmas, Spain. FAU - Illera, Óscar AU - Illera Ó AD - Hospital Universitario Infanta Sofía, Madrid, Spain. FAU - Moreira, Begoña AU - Moreira B AD - Complejo Hospitalario de Pontevedra, Spain. FAU - Raya, Enrique AU - Raya E AD - Hospital Clínico Universitario San Cecilio, Granada, Spain. FAU - Rodríguez López, Marina AU - Rodríguez López M AD - Hospital do Meixoeiro, Vigo, Spain. FAU - Júdez, Enrique AU - Júdez E AD - Hospital Universitario de Albacete, Spain. FAU - Moriano, Clara AU - Moriano C AD - Hospital Universitario de León, Spain. FAU - Torrente-Segarra, Vicenc AU - Torrente-Segarra V AD - Hospital General de L'Hospitalet, Barcelona, Spain. FAU - García Magallón, Blanca AU - García Magallón B AD - Hospital Universitario Miguel Servet, Zaragoza, Spain. FAU - Guillén Astete, Carlos AU - Guillén Astete C AD - Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Castellvi, Iván AU - Castellvi I AD - Hospital Universitario Santa Creu y Sant Pau, Barcelona, Spain. FAU - Bohórquez, Cristina AU - Bohórquez C AD - Hospital Universitario Príncipe de Asturias, Madrid, Spain. FAU - Loricera, Javier AU - Loricera J AD - Hospital Universitario Marqués de Valdecilla, Facultad de Medicina Universidad de Cantabria, Santander, Spain. FAU - Belzunegui, Joaquin AU - Belzunegui J AD - Hospital Universitario de Donostia, Guipúzcoa, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Rheumatology Department, Hospital Universitario Doce de Octubre, Madrid, Spain. CN - Sjögrenser group, part of the Spanish Society of Rheumatology Systemic Autoimmune Diseases Study Group (EASSER) LA - eng PT - Journal Article PT - Multicenter Study DEP - 20201001 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Cohort Studies MH - Female MH - *Hepatitis, Autoimmune MH - Humans MH - Male MH - Middle Aged MH - Randomized Controlled Trials as Topic MH - Registries MH - Retrospective Studies MH - *Sjogren's Syndrome/complications/diagnosis/epidemiology EDAT- 2020/10/08 06:00 MHDA- 2020/10/28 06:00 CRDT- 2020/10/07 08:40 PHST- 2020/07/08 00:00 [received] PHST- 2020/09/14 00:00 [accepted] PHST- 2020/10/08 06:00 [pubmed] PHST- 2020/10/28 06:00 [medline] PHST- 2020/10/07 08:40 [entrez] AID - 16119 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2020 Jul-Aug;38 Suppl 126(4):110-115. Epub 2020 Oct 1. PMID- 26433044 OWN - NLM STAT- MEDLINE DCOM- 20160817 LR - 20250529 IS - 1872-6240 (Electronic) IS - 0006-8993 (Print) IS - 0006-8993 (Linking) VI - 1627 DP - 2015 Nov 19 TI - The effects of repetitive vibration on sensorineural function: biomarkers of sensorineural injury in an animal model of metabolic syndrome. PG - 216-24 LID - S0006-8993(15)00724-6 [pii] LID - 10.1016/j.brainres.2015.09.026 [doi] AB - Exposure to hand-transmitted vibration in the work-place can result in the loss of sensation and pain in workers. These effects may be exacerbated by pre-existing conditions such as diabetes or the presence of primary Raynaud's phenomena. The goal of these studies was to use an established model of vibration-induced injury in Zucker rats. Lean Zucker rats have a normal metabolic profile, while obese Zucker rats display symptoms of metabolic disorder or Type II diabetes. This study examined the effects of vibration in obese and lean rats. Zucker rats were exposed to 4h of vibration for 10 consecutive days at a frequency of 125 Hz and acceleration of 49 m/s(2) for 10 consecutive days. Sensory function was checked using transcutaneous electrical stimulation on days 1, 5 and 9 of the exposure. Once the study was complete the ventral tail nerves, dorsal root ganglia and spinal cord were dissected, and levels of various transcripts involved in sensorineural dysfunction were measured. Sensorineural dysfunction was assessed using transcutaneous electrical stimulation. Obese Zucker rats displayed very few changes in sensorineural function. However they did display significant changes in transcript levels for factors involved in synapse formation, peripheral nerve remodeling, and inflammation. The changes in transcript levels suggested that obese Zucker rats had some level of sensory nerve injury prior to exposure, and that exposure to vibration activated pathways involved in injury and re-innervation. CI - Published by Elsevier B.V. FAU - Kiedrowski, Megan AU - Kiedrowski M AD - National Institute for Occupational Safety and Health, 1095 Willowdale Road, Morgantown, WV 26505, USA. FAU - Waugh, Stacey AU - Waugh S AD - National Institute for Occupational Safety and Health, 1095 Willowdale Road, Morgantown, WV 26505, USA. FAU - Miller, Roger AU - Miller R AD - National Institute for Occupational Safety and Health, 1095 Willowdale Road, Morgantown, WV 26505, USA. FAU - Johnson, Claud AU - Johnson C AD - National Institute for Occupational Safety and Health, 1095 Willowdale Road, Morgantown, WV 26505, USA. FAU - Krajnak, Kristine AU - Krajnak K AD - National Institute for Occupational Safety and Health, 1095 Willowdale Road, Morgantown, WV 26505, USA. Electronic address: ksk1@cdc.gov. LA - eng GR - CC999999/ImCDC/Intramural CDC HHS/United States PT - Journal Article PT - Research Support, U.S. Gov't, P.H.S. DEP - 20151001 PL - Netherlands TA - Brain Res JT - Brain research JID - 0045503 RN - 0 (Adra2a protein, rat) RN - 0 (Chemokine CCL2) RN - 0 (Interleukin-1beta) RN - 0 (RNA, Messenger) RN - 0 (Receptors, Adrenergic, alpha-2) RN - 3604-79-3 (3-nitrotyrosine) RN - 42HK56048U (Tyrosine) RN - EC 1.14.13.39 (Nitric Oxide Synthase Type I) SB - IM MH - Animals MH - Biophysics MH - Chemokine CCL2/genetics/metabolism MH - Disease Models, Animal MH - Ganglia, Spinal/metabolism MH - Gene Expression Regulation/physiology MH - Hyperalgesia/*physiopathology MH - Interleukin-1beta/genetics/metabolism MH - Male MH - Metabolic Syndrome/*complications MH - Neuralgia/*etiology MH - Nitric Oxide Synthase Type I/genetics/metabolism MH - RNA, Messenger MH - Rats MH - Rats, Zucker MH - Receptors, Adrenergic, alpha-2/genetics/metabolism MH - Time Factors MH - Transcutaneous Electric Nerve Stimulation/adverse effects MH - Tyrosine/analogs & derivatives/metabolism MH - Vibration/*adverse effects PMC - PMC4725191 MID - NIHMS749724 OID - NLM: HHSPA749724 OTO - NOTNLM OT - Hyperalgesia OT - Metabolic disorder OT - Neuropathic pain OT - Zucker rat EDAT- 2015/10/04 06:00 MHDA- 2016/08/18 06:00 PMCR- 2016/01/25 CRDT- 2015/10/04 06:00 PHST- 2015/04/22 00:00 [received] PHST- 2015/09/14 00:00 [revised] PHST- 2015/09/22 00:00 [accepted] PHST- 2015/10/04 06:00 [entrez] PHST- 2015/10/04 06:00 [pubmed] PHST- 2016/08/18 06:00 [medline] PHST- 2016/01/25 00:00 [pmc-release] AID - S0006-8993(15)00724-6 [pii] AID - 10.1016/j.brainres.2015.09.026 [doi] PST - ppublish SO - Brain Res. 2015 Nov 19;1627:216-24. doi: 10.1016/j.brainres.2015.09.026. Epub 2015 Oct 1. PMID- 23404236 OWN - NLM STAT- MEDLINE DCOM- 20140205 LR - 20211021 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 32 IP - 6 DP - 2013 Jun TI - Peripheral neuropathy: a complication of systemic sclerosis. PG - 885-8 LID - 10.1007/s10067-013-2206-6 [doi] AB - We performed bedside testing for peripheral neuropathy in our systemic sclerosis (SSc) population to determine whether foot care guidelines should be developed for SSc. Twenty consecutive SSc patients and 20 healthy control (HC) patients were evaluated for peripheral neuropathy in both feet using the 10-g Semmes-Weinstein monofilament examination (SWME) and 128 Hz vibration sensation using the on-off method. Independent, blinded, vibratory sensation, and SWME evaluations were performed on each subject by two investigators who had completed a training session to standardize each exam. An additional consecutive 20 patients with type 2 diabetes mellitus (DM) were examined by a diabetologist to compare with peripheral neuropathy prevalence in SSc patients. We examined the inter-rater variability using Cohen's kappa. We compared SWME and vibratory sensation in SSc to HC using Fisher's exact. The t test was used to compare duration of disease and modified Rodnan skin score (mRSS) for those with abnormal SWME or vibratory sensation. Two of 20 SSc patients reported sensory foot symptoms consistent with peripheral neuropathy prior to the examination. Inter-rater agreement for both SWME and vibratory sensation was strong (kappa: 0.72 and 0.83, respectively). Two HC and 12 SSc patients demonstrated abnormal vibratory sense (one-sided Fishers' exact, p < 0.002). No HC and four SSc patients had abnormal monofilament exams (one-sided Fisher's exact, p = 0.053). Neither mRSS (p = 0.28) nor duration of non-Raynauds (p = 0.07) symptoms differed between those with peripheral neuropathy and those without. Duration of Raynaud's symptoms were clinically significantly associated with presence of peripheral neuropathy (p = 0.04). The prevalence of sensory loss to monofilament in SSc was identical to DM patients (4/20). SSc patients have a considerable prevalence of pedal peripheral neuropathy as detected by loss of vibratory sensation or inability to sense the 10-g SWME. Further studies are indicated to determine if routine screening for neuropathy and subsequent podiatric care for SSc patients with abnormalities can reduce pedal complications. FAU - Frech, Tracy M AU - Frech TM AD - Division of Rheumatology, Department of Internal Medicine, University of Utah, 4B200 SOM 30 N 1900 E, Salt Lake City, UT 84132, USA. Tracy.Frech@hsc.utah.edu FAU - Smith, Gordon AU - Smith G FAU - Reily, Melissa AU - Reily M FAU - Chamberlain, James AU - Chamberlain J FAU - Murtaugh, Maureen A AU - Murtaugh MA FAU - Penrod, Jason AU - Penrod J FAU - Battistone, Michael J AU - Battistone MJ FAU - Stults, Barry M AU - Stults BM LA - eng GR - 8UL1TR000104/TR/NCATS NIH HHS/United States GR - R01DK064814/DK/NIDDK NIH HHS/United States GR - UL1RR025763/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20130213 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 SB - IM MH - Adult MH - Aged MH - Diabetes Mellitus, Type 2/blood/complications MH - Female MH - Humans MH - Male MH - Mass Screening MH - Middle Aged MH - Observer Variation MH - Peripheral Nervous System Diseases/complications/*diagnosis MH - Reproducibility of Results MH - Risk Factors MH - Scleroderma, Systemic/*complications MH - Ulcer/physiopathology MH - Vibration EDAT- 2013/02/14 06:00 MHDA- 2014/02/06 06:00 CRDT- 2013/02/14 06:00 PHST- 2012/10/02 00:00 [received] PHST- 2013/01/29 00:00 [accepted] PHST- 2013/01/22 00:00 [revised] PHST- 2013/02/14 06:00 [entrez] PHST- 2013/02/14 06:00 [pubmed] PHST- 2014/02/06 06:00 [medline] AID - 10.1007/s10067-013-2206-6 [doi] PST - ppublish SO - Clin Rheumatol. 2013 Jun;32(6):885-8. doi: 10.1007/s10067-013-2206-6. Epub 2013 Feb 13. PMID- 20551155 OWN - NLM STAT- MEDLINE DCOM- 20101015 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 69 IP - 10 DP - 2010 Oct TI - Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. PG - 1809-15 LID - 10.1136/ard.2009.114264 [doi] AB - OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc. FAU - Tyndall, Anthony J AU - Tyndall AJ AD - Department of Rheumatology, University of Basel, Basel, Switzerland. FAU - Bannert, Bettina AU - Bannert B FAU - Vonk, Madelon AU - Vonk M FAU - Airò, Paolo AU - Airò P FAU - Cozzi, Franco AU - Cozzi F FAU - Carreira, Patricia E AU - Carreira PE FAU - Bancel, Dominique Farge AU - Bancel DF FAU - Allanore, Yannick AU - Allanore Y FAU - Müller-Ladner, Ulf AU - Müller-Ladner U FAU - Distler, Oliver AU - Distler O FAU - Iannone, Florenzo AU - Iannone F FAU - Pellerito, Raffaele AU - Pellerito R FAU - Pileckyte, Margarita AU - Pileckyte M FAU - Miniati, Irene AU - Miniati I FAU - Ananieva, Lidia AU - Ananieva L FAU - Gurman, Alexandra Balbir AU - Gurman AB FAU - Damjanov, Nemanja AU - Damjanov N FAU - Mueller, Adelheid AU - Mueller A FAU - Valentini, Gabriele AU - Valentini G FAU - Riemekasten, Gabriela AU - Riemekasten G FAU - Tikly, Mohammed AU - Tikly M FAU - Hummers, Laura AU - Hummers L FAU - Henriques, Maria J S AU - Henriques MJ FAU - Caramaschi, Paola AU - Caramaschi P FAU - Scheja, Agneta AU - Scheja A FAU - Rozman, Blaz AU - Rozman B FAU - Ton, Evelien AU - Ton E FAU - Kumánovics, Gábor AU - Kumánovics G FAU - Coleiro, Bernard AU - Coleiro B FAU - Feierl, Eva AU - Feierl E FAU - Szucs, Gabriella AU - Szucs G FAU - Von Mühlen, Carlos Alberto AU - Von Mühlen CA FAU - Riccieri, Valeria AU - Riccieri V FAU - Novak, Srdan AU - Novak S FAU - Chizzolini, Carlo AU - Chizzolini C FAU - Kotulska, Anna AU - Kotulska A FAU - Denton, Christopher AU - Denton C FAU - Coelho, Paulo C AU - Coelho PC FAU - Kötter, Ina AU - Kötter I FAU - Simsek, Ismail AU - Simsek I FAU - de la Pena Lefebvre, Paloma García AU - de la Pena Lefebvre PG FAU - Hachulla, Eric AU - Hachulla E FAU - Seibold, James R AU - Seibold JR FAU - Rednic, Simona AU - Rednic S FAU - Stork, Jirí AU - Stork J FAU - Morovic-Vergles, Jadranka AU - Morovic-Vergles J FAU - Walker, Ulrich A AU - Walker UA LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20100615 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Adult MH - Aged MH - Comorbidity MH - Epidemiologic Methods MH - Female MH - Gastrointestinal Hemorrhage/mortality MH - Heart Diseases/mortality MH - Humans MH - Lung Diseases/mortality MH - Male MH - Middle Aged MH - Neoplasms/mortality MH - Pneumonia/mortality MH - Prognosis MH - Scleroderma, Systemic/*mortality MH - Sepsis/mortality EDAT- 2010/06/17 06:00 MHDA- 2010/10/16 06:00 CRDT- 2010/06/17 06:00 PHST- 2010/06/17 06:00 [entrez] PHST- 2010/06/17 06:00 [pubmed] PHST- 2010/10/16 06:00 [medline] AID - S0003-4967(24)14822-4 [pii] AID - 10.1136/ard.2009.114264 [doi] PST - ppublish SO - Ann Rheum Dis. 2010 Oct;69(10):1809-15. doi: 10.1136/ard.2009.114264. Epub 2010 Jun 15. PMID- 17133599 OWN - NLM STAT- MEDLINE DCOM- 20070206 LR - 20220311 IS - 0004-3591 (Print) IS - 0004-3591 (Linking) VI - 54 IP - 12 DP - 2006 Dec TI - Relationship of antiphospholipid antibodies to cardiovascular manifestations of systemic lupus erythematosus. PG - 3918-25 AB - OBJECTIVE: Although antiphospholipid antibodies (aPL) are associated with arterial and venous thrombosis in systemic lupus erythematosus (SLE), the extent to which they influence other cardiovascular manifestations is either controversial or uncertain. We undertook this study to examine the relationships of aPL with valvular, myocardial, and arterial disease in SLE. METHODS: Two hundred patients in an SLE registry, recruited at the time of outpatient visits, underwent comprehensive interviews, physical examinations, laboratory assessments, echocardiography to assess left ventricular (LV) and valvular status, carotid ultrasonography to detect atherosclerosis (discrete plaque), and radial applanation tonometry to measure arterial stiffness. RESULTS: Antiphospholipid antibodies were present(defined as IgG or IgM anticardiolipin > or =40 IU/ml or the presence of lupus anticoagulant) in 42 patients (21%). Mitral valve nodules and moderate-to-severe mitral regurgitation were more common in aPL-positive patients (both 14.3% versus 4.4%; P = 0.02). Thirty-one percent of patients with high titers of IgG aPL (>80 IU/ml) had mitral valve nodules, compared with 20% of patients with mildly to moderately elevated levels of IgG aPL (16-80 IU/ml) and 4% of patients without IgG aPL (overall P < 0.001). Levels of soluble tumor necrosis factor receptors were higher in the presence of both aPL and mitral valve nodules. LV dimensions, systolic function, and carotid artery stiffness as well as prevalences of Raynaud's phenomenon, pulmonary hypertension, and atherosclerosis were similar in aPL-positive and aPL-negative patients. CONCLUSION: Antiphospholipid antibodies in SLE are associated with mitral valve nodules and significant mitral regurgitation, possibly due to valvular endothelial cell activation. However, in this population, they are not associated with evidence of myocardial hypertrophy, systolic dysfunction, coronary or carotid atherosclerosis, or other vascular abnormalities. FAU - Farzaneh-Far, Afshin AU - Farzaneh-Far A AD - Hospital for Special Surgery, Weill Medical College of Cornell University, New York, New York 10021, USA. FAU - Roman, Mary J AU - Roman MJ FAU - Lockshin, Michael D AU - Lockshin MD FAU - Devereux, Richard B AU - Devereux RB FAU - Paget, Stephen A AU - Paget SA FAU - Crow, Mary K AU - Crow MK FAU - Davis, Adrienne AU - Davis A FAU - Sammaritano, Lisa AU - Sammaritano L FAU - Levine, Daniel M AU - Levine DM FAU - Salmon, Jane E AU - Salmon JE LA - eng GR - AR-45591/AR/NIAMS NIH HHS/United States GR - M10-RR-0047/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PL - United States TA - Arthritis Rheum JT - Arthritis and rheumatism JID - 0370605 RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin M) SB - IM MH - Adult MH - Antibodies, Anticardiolipin/*blood MH - Cardiovascular Diseases/complications/*immunology/physiopathology MH - Echocardiography MH - Female MH - Health Status MH - Humans MH - Immunoglobulin G/blood MH - Immunoglobulin M/blood MH - Lupus Erythematosus, Systemic/complications/*immunology/physiopathology MH - Male MH - Mitral Valve/pathology/physiopathology MH - Mitral Valve Insufficiency/complications/immunology/physiopathology MH - Severity of Illness Index EDAT- 2006/11/30 09:00 MHDA- 2007/02/07 09:00 CRDT- 2006/11/30 09:00 PHST- 2006/11/30 09:00 [pubmed] PHST- 2007/02/07 09:00 [medline] PHST- 2006/11/30 09:00 [entrez] AID - 10.1002/art.22265 [doi] PST - ppublish SO - Arthritis Rheum. 2006 Dec;54(12):3918-25. doi: 10.1002/art.22265. PMID- 38660003 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240426 IS - 2398-8835 (Electronic) IS - 2398-8835 (Linking) VI - 7 IP - 4 DP - 2024 Apr TI - Neurological, cardiac, musculoskeletal, and renal manifestations of scleroderma along with insights into its genetics, pathophysiology, diagnostic, and therapeutic updates. PG - e2072 LID - 10.1002/hsr2.2072 [doi] LID - e2072 AB - BACKGROUND: Scleroderma, also referred to as systemic sclerosis, is a multifaceted autoimmune condition characterized by abnormal fibrosis and impaired vascular function. Pathologically, it encompasses the persistent presence of inflammation, abnormal collagen buildup, and restructuring of blood vessels in various organs, resulting in a wide range of clinical symptoms. This review incorporates the most recent scientific literature on scleroderma, with a particular emphasis on its pathophysiology, clinical manifestations, diagnostic approaches, and treatment options. METHODOLOGY: A comprehensive investigation was carried out on numerous databases, such as PubMed, MEDLINE, Scopus, Web of Science, and Google Scholar, to collect pertinent studies covering diverse facets of scleroderma research. RESULTS: Scleroderma presents with a range of systemic manifestations, such as interstitial lung disease, gastrointestinal dysmotility, Raynaud's phenomenon, pulmonary arterial hypertension, renal complications, neurological symptoms, and cardiac abnormalities. Serological markers, such as antinuclear antibodies, anti-centromere antibodies, and anti-topoisomerase antibodies, are important for classifying diseases and predicting their outcomes. DISCUSSION: The precise identification of scleroderma is crucial for promptly and correctly implementing effective treatment plans. Treatment approaches aim to improve symptoms, reduce complications, and slow down the progression of the disease. An integrated approach that combines pharmacological agents, including immunosuppressants, endothelin receptor antagonists, and prostanoids, with nonpharmacological interventions such as physical and occupational therapy is essential for maximizing patient care. CONCLUSION: Through the clarification of existing gaps in knowledge and identification of emerging trends, our goal is to improve the accuracy of diagnosis, enhance the effectiveness of therapeutic interventions, and ultimately enhance the overall quality of life for individuals suffering from scleroderma. Ongoing cooperation and creative research are necessary to advance the field and achieve improved patient outcomes and new therapeutic discoveries. CI - © 2024 The Authors. Health Science Reports published by Wiley Periodicals LLC. FAU - Prajjwal, Priyadarshi AU - Prajjwal P AUID- ORCID: 0000-0001-7894-1829 AD - Department of Neurology Bharati Vidyapeeth Deemed University Pune India. FAU - Marsool, Mohammed Dheyaa Marsool AU - Marsool MDM AUID- ORCID: 0000-0002-3481-4534 AD - Department of Neurology, Al-Kindy College of Medicine University of Baghdad Baghdad Iraq. FAU - Yadav, Vikas AU - Yadav V AD - Department of Internal Medicine Pt. B. D. S. Postgraduate Institute of Medical Sciences Rohtak India. FAU - Kanagala, Ramya S D AU - Kanagala RSD AD - Department of Internal Medicine Mamata Medical College Khammam Telangana India. FAU - Reddy, Yeruva Bheemeswara AU - Reddy YB AD - Department of Internal Medicine Zaporozhyea State Medical University Zaporozhyea Ukraine. FAU - John, Jobby AU - John J AD - Department of Internal Medicine Dr. Somervell Memorial CSI Medical College and Hospital Neyyāttinkara India. FAU - Lam, Justin Riley AU - Lam JR AD - Department of Internal Medicine Cebu Institute of Medicine Cebu Philippines. FAU - Karra, Nanditha AU - Karra N AD - Department of Internal Medicine Osmania Medical College Hyderabad Telangana India. FAU - Amiri, Bita AU - Amiri B AD - Cardiovascular Research Center Tabriz University of Medical Sciences Tabriz Iran. FAU - Islam, Moiz Ul AU - Islam MU AD - Department of Internal Medicine Punjab Medical College Faisalabad Pakistan. FAU - Nithya, Venkatesh AU - Nithya V AD - Department of Internal Medicine S. D. Asfendiyarov Kazakh National Medical University Almaty Kazakhstan. FAU - Marsool, Ali Dheyaa Marsool AU - Marsool ADM AD - Department of Neurology, Al-Kindy College of Medicine University of Baghdad Baghdad Iraq. FAU - Gadam, Srikanth AU - Gadam S AUID- ORCID: 0000-0003-1800-1207 AD - Mayo Clinic Rochester Minnesota USA. FAU - Vora, Neel AU - Vora N AD - B. J. Medical College Ahmedabad India. FAU - Hussin, Omniat Amir AU - Hussin OA AUID- ORCID: 0000-0002-5021-263X AD - Department of Medicine Almanhal University Academy of Science Khartoum Sudan. LA - eng PT - Journal Article DEP - 20240424 PL - United States TA - Health Sci Rep JT - Health science reports JID - 101728855 PMC - PMC11040569 OTO - NOTNLM OT - cardiac abnormalities OT - genetics OT - neurological manifestations OT - renal complications OT - scleroderma OT - systemic sclerosis OT - therapeutic updates COIS- The authors declare no conflict of interest. EDAT- 2024/04/25 06:49 MHDA- 2024/04/25 06:50 PMCR- 2024/04/24 CRDT- 2024/04/25 04:02 PHST- 2023/07/24 00:00 [received] PHST- 2024/04/08 00:00 [revised] PHST- 2024/04/09 00:00 [accepted] PHST- 2024/04/25 06:50 [medline] PHST- 2024/04/25 06:49 [pubmed] PHST- 2024/04/25 04:02 [entrez] PHST- 2024/04/24 00:00 [pmc-release] AID - HSR22072 [pii] AID - 10.1002/hsr2.2072 [doi] PST - epublish SO - Health Sci Rep. 2024 Apr 24;7(4):e2072. doi: 10.1002/hsr2.2072. eCollection 2024 Apr. PMID- 23316984 OWN - NLM STAT- MEDLINE DCOM- 20130312 LR - 20130115 IS - 1532-0650 (Electronic) IS - 0002-838X (Linking) VI - 86 IP - 12 DP - 2012 Dec 15 TI - Cutaneous cryosurgery. PG - 1118-24 AB - Cutaneous cryosurgery refers to localized application of freezing temperatures to achieve destruction of skin lesions. It can be used to treat a broad range of benign and premalignant skin conditions, and certain malignant skin conditions, with high cure rates. Cellular destruction is accomplished by delivery of the cryogen via dipstick, probe, or spray techniques. It is widely used in primary care because of its safety, effectiveness, low cost, ease of use, good cosmetic results, and lack of need for anesthesia. Cryosurgery is as effective as alternative therapies for most cases of molluscum contagiosum, dermatofibromas, keloids, and plantar or genital warts. It is a more effective cure for common warts than salicylic acid or observation. Cryosurgery is generally the treatment of choice for actinic keratosis. Contraindications to cryosurgery include cryofibrinogenemia, cryoglobulinemia, Raynaud disease, agammaglobulinemia, and multiple myeloma. Complications from cryosurgery include hypopigmentation and alopecia, and can be avoided by limiting freeze times to less than 30 seconds. Referral to a dermatologist should be considered in cases of diagnostic uncertainty or for treatment of skin cancer, which requires larger amounts of tissue destruction, resulting in higher complication rates. FAU - Zimmerman, Ethan E AU - Zimmerman EE AD - Nellis Family Medicine Residency, Nellis Air Force Base, NV, USA. ethan.zimmerman@nellis.af.mil FAU - Crawford, Paul AU - Crawford P LA - eng PT - Journal Article PL - United States TA - Am Fam Physician JT - American family physician JID - 1272646 SB - IM MH - Condylomata Acuminata/surgery MH - Cryosurgery/adverse effects/economics/instrumentation/*methods MH - Histiocytoma, Benign Fibrous/surgery MH - Humans MH - Keloid/surgery MH - Keratosis, Actinic/surgery MH - Molluscum Contagiosum/surgery MH - Practice Guidelines as Topic MH - Skin Diseases/pathology/*surgery MH - Skin Neoplasms/surgery MH - Treatment Outcome MH - United States MH - Warts/surgery MH - Wound Healing EDAT- 2013/01/16 06:00 MHDA- 2013/03/13 06:00 CRDT- 2013/01/16 06:00 PHST- 2013/01/16 06:00 [entrez] PHST- 2013/01/16 06:00 [pubmed] PHST- 2013/03/13 06:00 [medline] AID - d10610 [pii] PST - ppublish SO - Am Fam Physician. 2012 Dec 15;86(12):1118-24. PMID- 33431193 OWN - NLM STAT- MEDLINE DCOM- 20210806 LR - 20220531 IS - 1531-6564 (Electronic) IS - 0363-5023 (Linking) VI - 46 IP - 6 DP - 2021 Jun TI - The Effects of Botulinum Toxin A on Pain in Ischemic Vasospasm. PG - 513.e1-513.e12 LID - S0363-5023(20)30671-7 [pii] LID - 10.1016/j.jhsa.2020.11.005 [doi] AB - PURPOSE: The purpose was to describe the impact of botulinum toxin A (BTX-A) administration in patients with ischemic vasospasm on the magnitude and timing of pain relief and subsequent effect on opioid use. The secondary purposes were to determine the role of photoplethysomgraph (PPG) testing on treatment decisions, effect on patient-reported outcomes, and additional procedures. METHODS: A retrospective analysis of patients who received BTX-A injections was performed. Botulinum toxin type A was injected subcutaneously in symptom-specific 2-level patterns. Pain, shortened version of the Disabilities of the Arm, Shoulder, and Hand (QuickDASH), and opioid use (quantified by median morphine equivalents) were recorded and the need for repeat injections or unplanned surgeries was assessed. RESULTS: All patients (n = 20 patients; 31 hands) had ischemic pain from vasospasm and failed multiple pharmacological options. Average follow-up was 10.5 months. All patients had abnormal PPG amplitude (mean, 6.43 mm) at room temperature and increased amplitude (mean, 19.55 mm) after immersion in warm water. All patients (n = 12) with a PPG amplitude increase of 4 mm or greater had clinical success. Eleven of 13 patients had a clinically relevant decrease in pain at 20 minutes after injection. Clinically significant pain relief was sustained for 3 months (visual analog scale decreased by a mean of 4). Median morphine equivalent usage view decreased from 82.5 to 0 after injection. Patient-reported disability (QuickDASH) improved from 49 before treatment to 29 and 26 at 6 weeks and 6 months after BTX-A injection, respectively. Three patients were retreated for recurrent symptoms. Four patients required unplanned secondary procedures. CONCLUSIONS: Botulinum toxin type A administration can result in rapid (within 20 minutes) and sustained pain relief for several months with a reduction in opioid prescriptions. Botulinum toxin type A administration also improved patient-reported disability for 6 months. Use of PPG testing to determine baseline perfusion deficit and capacity to improve after warm water immersion was helpful in consideration of BTX-A use. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV. CI - Copyright © 2021 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved. FAU - Goldberg, Steven H AU - Goldberg SH AD - Muscuoskeletal Institute, Geisinger Medical Center, Danville, PA. Electronic address: Steven.Goldberg@bellin.org. FAU - Akoon, Anil AU - Akoon A AD - Muscuoskeletal Institute, Geisinger Medical Center, Danville, PA. FAU - Kirchner, H Lester AU - Kirchner HL AD - Department of Biomedical and Translational Informatics, Geisinger Medical Center, Danville, PA. FAU - Deegan, John AU - Deegan J AD - Muscuoskeletal Institute, Geisinger Medical Center, Danville, PA. LA - eng PT - Journal Article DEP - 20210109 PL - United States TA - J Hand Surg Am JT - The Journal of hand surgery JID - 7609631 RN - 0 (Neuromuscular Agents) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM MH - *Botulinum Toxins, Type A/therapeutic use MH - Humans MH - *Neuromuscular Agents/therapeutic use MH - Pain/*drug therapy MH - Pain Measurement MH - Retrospective Studies MH - Treatment Outcome MH - Vascular Diseases/*drug therapy MH - Visual Analog Scale OTO - NOTNLM OT - Botulinum toxin type A OT - Raynaud disease OT - opioid analgesics OT - vascular diseases OT - vasoconstriction EDAT- 2021/01/13 06:00 MHDA- 2021/08/07 06:00 CRDT- 2021/01/12 05:48 PHST- 2019/05/20 00:00 [received] PHST- 2020/09/03 00:00 [revised] PHST- 2020/11/03 00:00 [accepted] PHST- 2021/01/13 06:00 [pubmed] PHST- 2021/08/07 06:00 [medline] PHST- 2021/01/12 05:48 [entrez] AID - S0363-5023(20)30671-7 [pii] AID - 10.1016/j.jhsa.2020.11.005 [doi] PST - ppublish SO - J Hand Surg Am. 2021 Jun;46(6):513.e1-513.e12. doi: 10.1016/j.jhsa.2020.11.005. Epub 2021 Jan 9. PMID- 26782036 OWN - NLM STAT- MEDLINE DCOM- 20170202 LR - 20220318 IS - 1559-0267 (Electronic) IS - 1080-0549 (Linking) VI - 52 IP - 1 DP - 2017 Feb TI - Serum Jo-1 Autoantibody and Isolated Arthritis in the Antisynthetase Syndrome: Review of the Literature and Report of the Experience of AENEAS Collaborative Group. PG - 71-80 LID - 10.1007/s12016-016-8528-9 [doi] AB - Anti-Jo-1 is the most frequently detectable antibody in the antisynthetase syndrome (ASSD), an autoimmune disease characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). Recently, we organized an international collaborative group called American and European NEtwork of Antisynthetase Syndrome (AENEAS) for the study of this rare and fascinating disease. The group collected and published one of the largest series of ASSD patients ever described and with one of the longer follow-up ever reported. The number of participating centers is steadily increasing, as well as the available cohort. In the first paper, we showed that arthritis, myositis, and ILD may be frequently the only feature at disease onset, raising problems to reach a correct diagnosis of this syndrome. Nevertheless, we first observed that the ex novo appearance of further manifestations is common during the follow-up, strengthening the importance of a correct diagnosis. In our cohort, the 24 % of the 243 patients up to now collected had isolated arthritis as a presenting feature. These patients represent the most intriguing group in terms of differential diagnosis and clinical time course. Furthermore, data on this aspect are scanty, the reason that lead us to evaluate these aspects in our cohort of patients, reviewing also available literature. In fact, the most relevant aspect is that ASSD is rarely suspected in this setting of patients, in particular in case of poliarticular involvement, positive rheumatoid factor (RF), or anti-cyclic citrullinated peptide antibodies (ACPA) or evidence of joint erosions at plain radiographs. These findings were not rare in our cohort, and they have been also described in other series. Furthermore, manifestations such as Raynaud's phenomenon, mechanic's hands, and fever that may lead to the suspect of ASSD are observed only in a third of cases. If we consider the high rate of clinical picture progression in these patients, we feel that ASSD should be carefully considered in all patients presenting with isolated arthritis, even in those with erosive, RF, and ACPA-positive arthritis. FAU - Cavagna, Lorenzo AU - Cavagna L AUID- ORCID: 0000-0003-3292-1528 AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. cavagna@unipv.it. FAU - Nuño, Laura AU - Nuño L AD - Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. FAU - Scirè, Carlo Alberto AU - Scirè CA AD - Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy. FAU - Govoni, Marcello AU - Govoni M AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Longo, Francisco Javier Lopez AU - Longo FJ AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Neri, Rossella AU - Neri R AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Castañeda, Santos AU - Castañeda S AD - Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa, Madrid, Spain. FAU - Sifuentes Giraldo, Walter Alberto AU - Sifuentes Giraldo WA AD - Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. FAU - Caporali, Roberto AU - Caporali R AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. FAU - Fusaro, Enrico AU - Fusaro E AD - Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. FAU - Paolazzi, Giuseppe AU - Paolazzi G AD - Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. FAU - Pellerito, Raffaele AU - Pellerito R AD - Division of Rheumatology, Mauriziano Hospital, Turin, Italy. FAU - Schwarting, Andreas AU - Schwarting A AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center New Orleans, New Orleans, LA, USA. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. FAU - Quartuccio, Luca AU - Quartuccio L AD - Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, Udine, Italy. FAU - Bartoloni, Elena AU - Bartoloni E AD - Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. FAU - Specker, Christof AU - Specker C AD - Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. FAU - Pina Murcia, Trinitario AU - Pina Murcia T AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - La Corte, Renato AU - La Corte R AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Furini, Federica AU - Furini F AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Foschi, Valentina AU - Foschi V AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. FAU - Bachiller Corral, Javier AU - Bachiller Corral J AD - Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. FAU - Airò, Paolo AU - Airò P AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Martínez-Barrio, Julia AU - Martínez-Barrio J AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Hinojosa, Michelle AU - Hinojosa M AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Giannini, Margherita AU - Giannini M AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Menke, Julia AU - Menke J AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. FAU - Triantafyllias, Kostantinos AU - Triantafyllias K AD - ACURA Rheumatology Center, Bad Kreuznach, Germany. FAU - Vitetta, Rosetta AU - Vitetta R AD - Division of Rheumatology, Mauriziano Hospital, Turin, Italy. FAU - Russo, Alessandra AU - Russo A AD - Division of Rheumatology, Mauriziano Hospital, Turin, Italy. FAU - Bogliolo, Laura AU - Bogliolo L AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - Bajocchi, Gianluigi AU - Bajocchi G AD - Rheumatology Unit, Department of Internal Medicine, S.Maria Hospital-IRCCS, Reggio Emilia, Italy. FAU - Bravi, Elena AU - Bravi E AD - Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. FAU - Barausse, Giovanni AU - Barausse G AD - Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. FAU - Bortolotti, Roberto AU - Bortolotti R AD - Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. FAU - Selmi, Carlo AU - Selmi C AD - Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy. FAU - Parisi, Simone AU - Parisi S AD - Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. FAU - Salaffi, Fausto AU - Salaffi F AD - Rheumatology Department, Polytechnic University of Marche, C. Urbani Hospital, Jesi, Ancona, Italy. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. FAU - González-Gay, Miguel Angel AU - González-Gay MA AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. CN - AENEAS (American and European NEtwork of Antisynthetase Syndrome) Collaborative Group LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Antibodies, Antinuclear/*blood/immunology MH - Arthritis/*immunology MH - Autoantibodies/blood/*immunology MH - Histidine-tRNA Ligase/immunology MH - Humans MH - Myositis/blood/complications/*immunology OTO - NOTNLM OT - Anti-Jo-1 OT - Anti-cyclic citrullinated peptide OT - Antisynthetase syndrome OT - Clinical time course OT - Isolated polyarthritis OT - Rheumatoid factor EDAT- 2016/01/20 06:00 MHDA- 2017/02/06 06:00 CRDT- 2016/01/20 06:00 PHST- 2016/01/20 06:00 [pubmed] PHST- 2017/02/06 06:00 [medline] PHST- 2016/01/20 06:00 [entrez] AID - 10.1007/s12016-016-8528-9 [pii] AID - 10.1007/s12016-016-8528-9 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2017 Feb;52(1):71-80. doi: 10.1007/s12016-016-8528-9. PMID- 22034123 OWN - NLM STAT- MEDLINE DCOM- 20111215 LR - 20250529 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 63 IP - 11 DP - 2011 Nov TI - Validity of two new patient-reported outcome measures in systemic sclerosis: Patient-Reported Outcomes Measurement Information System 29-item Health Profile and Functional Assessment of Chronic Illness Therapy-Dyspnea short form. PG - 1620-8 LID - 10.1002/acr.20591 [doi] AB - OBJECTIVE: Many patient-reported outcome (PRO) instruments used in systemic sclerosis (SSc) trials are limited by lack of validation, licensing fees, and complicated scoring systems. We assessed the construct validity for discriminative purposes of 2 new PRO instruments, the Patient-Reported Outcomes Measurement Information System 29-item Health Profile (PROMIS-29) and the Functional Assessment of Chronic Illness Therapy-Dyspnea short form (FACIT-Dyspnea), measuring health status and dyspnea in SSc patients. METHODS: Seventy-three patients participated in a cross-sectional study at a tertiary SSc program. PROMIS-29, FACIT-Dyspnea, and legacy PRO instruments used in clinical trials (Medical Research Council Dyspnea Score, St. George's Respiratory Questionnaire, Health Assessment Questionnaire disability index, and Short Form 36) were administered. Composite severity scores using an adaptation of the Medsger Disease Severity Index were generated using clinical, diagnostic, and laboratory information. PROMIS-29 and FACIT-Dyspnea scores were compared with legacy PRO measures and composite severity scores. RESULTS: The mean patient age (84% women) was 51 years (range 22-72 years). The mean SSc disease duration from the onset of the first non-Raynaud's phenomenon symptom was 7.2 years (range 0-45 years). Spearman's correlation coefficients across FACIT-Dyspnea and PROMIS physical functioning scores with legacy PRO instruments were generally high (range 0.50-0.86); those between PROMIS and FACIT-Dyspnea with composite disease severity scores were more modest, but statistically significant (range 0.33-0.48, P < 0.01). CONCLUSION: PROMIS-29 and FACIT-Dyspnea are valid instruments to measure the health status of SSc patients. PROMIS-29 and FACIT-Dyspnea may be preferable to legacy instruments because they are freely available in multiple languages and simple to administer, score, and interpret. CI - Copyright © 2011 by the American College of Rheumatology. FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA. m-hinchcliff@northwestern.edu FAU - Beaumont, Jennifer L AU - Beaumont JL FAU - Thavarajah, Krishna AU - Thavarajah K FAU - Varga, John AU - Varga J FAU - Chung, Anh AU - Chung A FAU - Podlusky, Sofia AU - Podlusky S FAU - Carns, Mary AU - Carns M FAU - Chang, Rowland W AU - Chang RW FAU - Cella, David AU - Cella D LA - eng GR - UL1RR025741/RR/NCRR NIH HHS/United States GR - P60-AR48098/AR/NIAMS NIH HHS/United States GR - U01 AR052177/AR/NIAMS NIH HHS/United States GR - UL1 RR025741/RR/NCRR NIH HHS/United States GR - P60 AR048098/AR/NIAMS NIH HHS/United States GR - HD055884/HD/NICHD NIH HHS/United States GR - K12 HD055884/HD/NICHD NIH HHS/United States GR - U5AR057951-01/AR/NIAMS NIH HHS/United States GR - U54 AR057951/AR/NIAMS NIH HHS/United States GR - U01AR052177-06S1/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Validation Study PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM CIN - Arthritis Care Res (Hoboken). 2012 Jun;64(6):942-3; author reply 943-4. doi: 10.1002/acr.21578. PMID: 22183973 MH - Adult MH - Age of Onset MH - Aged MH - Chronic Disease MH - Cross-Sectional Studies MH - Dyspnea/*diagnosis/etiology/physiopathology/psychology MH - Female MH - *Health Status MH - *Health Status Indicators MH - Humans MH - Male MH - Michigan/epidemiology MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Registries MH - Reproducibility of Results MH - Scleroderma, Systemic/complications/*diagnosis/physiopathology/psychology MH - *Self Report MH - Severity of Illness Index MH - Time Factors MH - Young Adult PMC - PMC3205420 MID - NIHMS317606 COIS- Conflict of Interest: None of the authors have received any financial support or other benefits from commercial sources for the work reported on in the manuscript, and none have any other financial interests that could create a potential conflict of interest or the appearance of a conflict of interest with regard to the work. EDAT- 2011/10/29 06:00 MHDA- 2011/12/16 06:00 PMCR- 2012/11/01 CRDT- 2011/10/29 06:00 PHST- 2011/10/29 06:00 [entrez] PHST- 2011/10/29 06:00 [pubmed] PHST- 2011/12/16 06:00 [medline] PHST- 2012/11/01 00:00 [pmc-release] AID - 10.1002/acr.20591 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2011 Nov;63(11):1620-8. doi: 10.1002/acr.20591. PMID- 38494831 OWN - NLM STAT- MEDLINE DCOM- 20240717 LR - 20260518 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 29 IP - 8 DP - 2024 Aug TI - Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases. PG - 713-721 LID - 10.1111/resp.14703 [doi] AB - BACKGROUND AND OBJECTIVE: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis. METHODS: We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE. RESULTS: Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression. CONCLUSION: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease. CI - © 2024 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology. FAU - Deneuville, Lou AU - Deneuville L AD - Université Paris Cité, Inserm, PHERE, F-75018 Paris, et Hôpital Bichat, APHP, Service de Pneumologie A, Centre constitutif du centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Paris, France. FAU - Mageau, Arthur AU - Mageau A AD - Département de Médecine Interne, Hôpital Bichat, Assistance Publique Hôpitaux de Paris, Paris, France. FAU - Debray, Marie Pierre AU - Debray MP AD - Service de Radiologie, Hôpital Bichat Paris, Paris, France. FAU - Sacre, Karim AU - Sacre K AUID- ORCID: 0000-0002-6544-234X AD - Département de Médecine Interne, Hôpital Bichat, Assistance Publique Hôpitaux de Paris, Paris, France. FAU - Costedoat-Chalumeau, Nathalie AU - Costedoat-Chalumeau N AD - Département de Médecine Interne, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France. FAU - Hachulla, Eric AU - Hachulla E AD - Department of Internal Medicine and Clinical Immunology, Referral Centre for Centre for Rare Systemic Autoimmune Diseases North and North-West of France (CeRAINO), CHU Lille, Univ. Lille, Inserm, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France. FAU - Uzunhan, Yurdagul AU - Uzunhan Y AD - Service de Pneumologie, Centre constitutif du centre de référence des maladies pulmonaires rares, Hôpital Avicenne, INSERM U1272, Université Sorbonne Paris Nord, Bobigny, France. FAU - Le Tallec, Erwan AU - Le Tallec E AUID- ORCID: 0009-0002-5768-3159 AD - Service de Médecine Interne et Immunologie Clinique, CHU de Rennes, Rennes, France. FAU - Cadranel, Jacques AU - Cadranel J AD - Service de Pneumologie, Centre de référence des maladies pulmonaires rares (site constitutif), Assistance Publique Hôpitaux de Paris-Hôpital Tenon et Sorbonne Université, Paris, France. FAU - Marchand Adam, Sylvain AU - Marchand Adam S AD - Service de Pneumologie et explorations fonctionnelles respiratoires, CHRU de Tours et université de Tours, Inserm 1100, Tours, France. FAU - Montani, David AU - Montani D AD - Université Paris-Saclay, AP-HP, INSERM UMR_S 999, Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital de Bicêtre, Le Kremlin Bicêtre, France. FAU - Rémi-Jardin, Martine AU - Rémi-Jardin M AD - Department of Thoracic Imaging, Heart & Lung Institute, University Hospital Center of Lille, Lille, France. FAU - Reynaud-Gaubert, Martine AU - Reynaud-Gaubert M AD - Service de Pneumologie, Centre de compétences des maladies pulmonaires rares, CHU Nord, AP-HM, Marseille; Aix- Marseille Université, IHU Méditerranée Infection, MEPHI, Marseille, France. FAU - Prevot, Gregoire AU - Prevot G AD - Service de Pneumologie, CHU Toulouse, Toulouse, France. FAU - Beltramo, Guillaume AU - Beltramo G AD - Service de Pneumologie et Soins Intensifs Respiratoires, Centre constitutif de référence des maladies pulmonaires rares, CHU Dijon-Bourgogne, Université de Bourgogne, UMR 1231-LNC-HSP-pathies, Dijon, France. FAU - Crestani, Bruno AU - Crestani B AD - Université Paris Cité, Inserm, PHERE, F-75018 Paris, et Hôpital Bichat, APHP, Service de Pneumologie A, Centre constitutif du centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Paris, France. FAU - Cottin, Vincent AU - Cottin V AUID- ORCID: 0000-0002-5591-0955 AD - Service de Pneumologie, Centre coordonnateur national de référence des maladies pulmonaires rares, Hôpital Louis Pradel, Université Claude Bernard Lyon 1, Université de Lyon; INRAE; ERN-LUNG, Lyon, France. FAU - Borie, Raphael AU - Borie R AUID- ORCID: 0000-0002-9906-0024 AD - Université Paris Cité, Inserm, PHERE, F-75018 Paris, et Hôpital Bichat, APHP, Service de Pneumologie A, Centre constitutif du centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Paris, France. CN - OrphaLung Network LA - eng PT - Journal Article PT - Multicenter Study DEP - 20240317 PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - Female MH - *Lung Diseases, Interstitial/diagnosis/mortality/etiology/complications MH - *Lupus Erythematosus, Systemic/complications MH - Male MH - Adult MH - Retrospective Studies MH - Middle Aged MH - Prognosis MH - Chronic Disease MH - Antibodies, Antinuclear/blood OTO - NOTNLM OT - connective‐tissue disease OT - disease progression OT - interstitial lung disease OT - lupus OT - non‐specific interstitial pneumonia OT - pulmonary fibrosis OT - survival FIR - Ahmad, Kais IR - Ahmad K FIR - Traclet, Julie IR - Traclet J FIR - Leguen, Pierre IR - Leguen P FIR - Bravais, Juliette IR - Bravais J FIR - Nieves, Ana IR - Nieves A FIR - Audia, Sylvain IR - Audia S FIR - Nunes, Hilario IR - Nunes H EDAT- 2024/03/18 06:44 MHDA- 2024/07/18 00:42 CRDT- 2024/03/18 02:44 PHST- 2023/08/10 00:00 [received] PHST- 2024/03/03 00:00 [accepted] PHST- 2024/07/18 00:42 [medline] PHST- 2024/03/18 06:44 [pubmed] PHST- 2024/03/18 02:44 [entrez] AID - 10.1111/resp.14703 [doi] PST - ppublish SO - Respirology. 2024 Aug;29(8):713-721. doi: 10.1111/resp.14703. Epub 2024 Mar 17. PMID- 27692137 OWN - NLM STAT- MEDLINE DCOM- 20180116 LR - 20220330 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 119 DP - 2016 Oct TI - Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience. PG - 150-154 LID - S0954-6111(16)30225-6 [pii] LID - 10.1016/j.rmed.2016.09.002 [doi] AB - OBJECTIVE: To describe the clinical phenotype and natural history of a cohort of patients with interstitial pneumonia with autoimmune features (IPAF). METHODS: A retrospective, single center study of 56 patients with IPAF evaluated between February 2008 and August 2014. All clinical data were extracted from the electronic medical record and longitudinal changes in forced vital capacity (FVC) were analyzed with mixed-effects, piecewise linear regression models that considered time as a continuous factor. RESULTS: All patients fulfilled classification criteria for IPAF. The majority were women (71%) and never smokers (68%). The most frequently identified clinical features were Raynaud's phenomenon (39%), distal digital fissuring (29%), Gottron's sign (18%) and inflammatory arthropathy (16%). The most frequently identified serologies were antinuclear antibody (ANA) (48%), anti-Ro (SSA) (43%) and anti-tRNA-synthetase antibodies (36%). Nonspecific interstitial pneumonia (NSIP) (57.1%) followed by NSIP with organizing pneumonia (18%) were the most common radiologic patterns, while usual interstitial pneumonia was identified in only 9%. All but one patient was treated with immunosuppression: prednisone (82%) and mycophenolate mofetil (76%) were the most frequently used agents. During a follow-up period of 284.9 ± 141.3 days, modeled longitudinal FVC% was stable (slope = 0.69/year) and no deaths were observed in the cohort. CONCLUSIONS: In this single center study, patients with IPAF were predominately non-smoking women with high-resolution computed tomography scans that suggested NSIP. Their pulmonary physiology was stable, and during limited follow-up, no deaths were observed. Prospective and multi-center studies are needed to better inform our understanding of IPAF. CI - Copyright © 2016 Elsevier Ltd. All rights reserved. FAU - Chartrand, Sandra AU - Chartrand S AD - Department of Medicine, Hôpital Maisonneuve-Rosemont affiliated to Université de Montréal, Montréal, Québec, Canada. FAU - Swigris, Jeffrey J AU - Swigris JJ AD - Department of Medicine, National Jewish Health, Denver, CO, USA; Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. FAU - Stanchev, Lina AU - Stanchev L AD - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. FAU - Lee, Joyce S AU - Lee JS AD - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. FAU - Brown, Kevin K AU - Brown KK AD - Department of Medicine, National Jewish Health, Denver, CO, USA; Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. FAU - Fischer, Aryeh AU - Fischer A AD - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. Electronic address: aryeh.fischer@ucdenver.edu. LA - eng PT - Journal Article DEP - 20160903 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antinuclear) RN - 0 (Enzyme Inhibitors) RN - 0 (Immunosuppressive Agents) RN - 7U1EE4V452 (Carbon Monoxide) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - HU9DX48N0T (Mycophenolic Acid) SB - IM CIN - Respir Med. 2017 Nov;132:265-266. doi: 10.1016/j.rmed.2017.03.028. PMID: 28385573 CIN - Respir Med. 2017 Nov;132:267. doi: 10.1016/j.rmed.2017.07.015. PMID: 28760577 MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Amino Acyl-tRNA Synthetases/*antagonists & inhibitors/immunology MH - Antibodies, Antinuclear MH - Autoimmune Diseases/diagnosis/immunology MH - Biopsy MH - Carbon Monoxide/metabolism MH - Connective Tissue Diseases/diagnosis/pathology MH - Diagnosis, Differential MH - Enzyme Inhibitors/therapeutic use MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/diagnosis/pathology MH - Immunosuppressive Agents/therapeutic use MH - Lung/*diagnostic imaging/pathology/physiopathology MH - Lung Diseases, Interstitial/*diagnosis/drug therapy/pathology/physiopathology MH - Male MH - Middle Aged MH - Mycophenolic Acid/therapeutic use MH - Phenotype MH - Pulmonary Diffusing Capacity/physiology MH - Respiratory Function Tests/methods MH - Retrospective Studies MH - Tomography, X-Ray Computed MH - Vital Capacity/physiology OTO - NOTNLM OT - Connective tissue disease OT - Idiopathic interstitial pneumonia OT - Interstitial lung disease OT - Interstitial pneumonia with autoimmune features EDAT- 2016/10/04 06:00 MHDA- 2018/01/18 06:00 CRDT- 2016/10/04 06:00 PHST- 2016/05/24 00:00 [received] PHST- 2016/09/01 00:00 [accepted] PHST- 2016/10/04 06:00 [entrez] PHST- 2016/10/04 06:00 [pubmed] PHST- 2018/01/18 06:00 [medline] AID - S0954-6111(16)30225-6 [pii] AID - 10.1016/j.rmed.2016.09.002 [doi] PST - ppublish SO - Respir Med. 2016 Oct;119:150-154. doi: 10.1016/j.rmed.2016.09.002. Epub 2016 Sep 3. PMID- 34127049 OWN - NLM STAT- MEDLINE DCOM- 20210629 LR - 20251007 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 23 IP - 1 DP - 2021 Jun 14 TI - Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort. PG - 170 LID - 10.1186/s13075-021-02548-1 [doi] LID - 170 AB - BACKGROUND: Early diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. We report baseline characteristics, current immunosuppressive therapies, progression of skin and internal organ involvement, and mortality in a multicenter prospective cohort from the United States (US) of America. METHODS: We performed a longitudinal analysis of participants from 12 US centers, from April 2012 to July 2020. All participants had early dcSSc or were at-risk for dcSSc, with ≤2 years since the first non-Raynaud's phenomenon (RP) symptom. RESULTS: Three hundred one patients were included with a baseline median disease duration of 1.2 years since RP and a mean modified skin score of 21.1 units. At baseline, 263 (87.3%) had definite dcSSc and 38 (12.7%) were classified as at-risk; 112 (49.6%) patients were positive for anti-RNA polymerase III antibodies. The median follow-up duration was 24.5 months (IQR = 10.3-40.7 months). One hundred ninety (63.1%) participants were treated with an immunosuppressive therapy, of which mycophenolate mofetil was most used at baseline and follow-up. Of 38 who were classified as at-risk at baseline, 27 (71%) went on to develop dcSSc; these patients were characterized by higher baseline mean HAQ-DI (0.8 versus 0.4, p = 0.05) and higher baseline mRSS (8.8 versus 4.4, p < 0.01) in comparison with those who remained as limited cutaneous SSc. In the overall cohort, 48 participants (21.1%) had clinically significant worsening of skin fibrosis, mainly occurring in the first year of follow-up; 41 (23.3%) had an absolute forced vital capacity decline of ≥10%. Twenty participants (6.6%) died, of which 18 died in the first 3 years of follow-up. Cardiac involvement (33.3%), gastrointestinal dysmotility (22.2%), and progressive interstitial lung disease (ILD) (16.7%) were the main causes of death. CONCLUSION: This US cohort highlights the management of early SSc in the current era, demonstrating progression of skin and lung involvement despite immunosuppressive therapy and high mortality due to cardiac involvement. FAU - Jaafar, Sara AU - Jaafar S AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. FAU - Lescoat, Alain AU - Lescoat A AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. AD - Department of Internal Medicine and Clinical Immunology, CHU Rennes, Univ Rennes, Rennes, France. AD - University of Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail, Rennes, France. FAU - Huang, Suiyuan AU - Huang S AD - School of Public Health, University of Michigan, Ann Arbor, USA. FAU - Gordon, Jessica AU - Gordon J AD - Department of Medicine, Hospital for Special Surgery, New York, NY, USA. FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Department of Medicine, Northwestern University, Chicago, IL, USA. FAU - Shah, Ami A AU - Shah AA AD - Department of Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Assassi, Shervin AU - Assassi S AD - Department of Medicine, University of Texas Health Science Center, Houston, TX, USA. FAU - Domsic, Robyn AU - Domsic R AD - Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. FAU - Bernstein, Elana J AU - Bernstein EJ AD - Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA. FAU - Steen, Virginia AU - Steen V AD - Department of Medicine, Georgetown University, Washington, DC, USA. FAU - Elliott, Sabrina AU - Elliott S AD - Department of Medicine, Georgetown University, Washington, DC, USA. FAU - Hant, Faye AU - Hant F AD - Department of Medicine, Medical University of South Carolina, Charleston, SC, USA. FAU - Castelino, Flavia V AU - Castelino FV AD - Department of Medicine, Harvard University, Boston, MA, USA. FAU - Shanmugam, Victoria K AU - Shanmugam VK AD - Department of Medicine, George Washington University, Washington, DC, USA. FAU - Correia, Chase AU - Correia C AD - Department of Medicine, Northwestern University, Chicago, IL, USA. FAU - Varga, John AU - Varga J AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. FAU - Nagaraja, Vivek AU - Nagaraja V AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. FAU - Roofeh, David AU - Roofeh D AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. FAU - Frech, Tracy AU - Frech T AD - Department of Medicine, University of Utah, Salt Lake City, UT, USA. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Division of Rheumatology and Scleroderma Program, Department of Internal Medicine, University of Michigan, Suite 7C27 300 North Ingalls Street, SPC 5422, Ann Arbor, MI, 48109, USA. khannad@umich.edu. LA - eng GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - R01 AR070470/AR/NIAMS NIH HHS/United States GR - UL1 TR001863/TR/NCATS NIH HHS/United States GR - K24-AR-063120/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Observational Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20210614 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - HU9DX48N0T (Mycophenolic Acid) SB - IM MH - Cohort Studies MH - Humans MH - *Lung Diseases, Interstitial MH - Mycophenolic Acid MH - Prospective Studies MH - *Scleroderma, Diffuse MH - *Scleroderma, Systemic MH - United States PMC - PMC8201684 OTO - NOTNLM OT - Diffuse cutaneous systemic sclerosis OT - Interstitial lung disease OT - Mortality OT - Scleroderma OT - Survival OT - Systemic sclerosis COIS- SJ, AL, SH, MH, AS, SA, SE, FH, FC, VKS, CC, JV, VN, DR, and TF: nothing to disclose. JG reports grants from EICOS Sciences, grants from Cumberland Pharmaceuticals. RD reports personal fees from Formation Biologics, personal fees from Eicos Sciences, Inc, and personal fees from Corbus Pharmaceuticals. EB reports grants from NIH (K23 AR075112), grants and personal fees from Boehringer Ingleheim, grants and personal fees from Eicos Sciences, Inc, and grants from Pfizer. VS reports grants and others from Corbus; grants and others from Bayer; grants and others from CSL Behring; others from Forbius, during the conduct of the study; grants and others from Boehringer Ingelheim; grants and others from Eisocs; and grants from Reata. DK reports grants from NIH, grants from Immune Tolerance Network, grants and personal fees from Bayer, grants from Bristol Myers Squibb, grants from Horizon, grants from Pfizer, personal fees from Acceleron, personal fees from Actelion, personal fees from Amgen, personal fees from Blade Therapeutics, personal fees from Boehringer Ingelheim, personal fees from CSL Behring, personal fees from Corbus, personal fees from Cytori, personal fees from Galapagos, personal fees from Genentech/Roche, personal fees from GSK, personal fees from Horizon, personal fees from Merck, personal fees from Mitsubishi Tanabe Pharma, personal fees from Regeneron, personal fees from Sanofi-Aventis, personal fees from United Therapeutics, others from Impact PH, personal fees from Eicos Sciences, Inc, and personal fees and others from CiviBioPharma/Eicos Sciences, Inc. EDAT- 2021/06/16 06:00 MHDA- 2021/06/30 06:00 PMCR- 2021/06/14 CRDT- 2021/06/15 05:53 PHST- 2021/01/08 00:00 [received] PHST- 2021/05/24 00:00 [accepted] PHST- 2021/06/15 05:53 [entrez] PHST- 2021/06/16 06:00 [pubmed] PHST- 2021/06/30 06:00 [medline] PHST- 2021/06/14 00:00 [pmc-release] AID - 10.1186/s13075-021-02548-1 [pii] AID - 2548 [pii] AID - 10.1186/s13075-021-02548-1 [doi] PST - epublish SO - Arthritis Res Ther. 2021 Jun 14;23(1):170. doi: 10.1186/s13075-021-02548-1. PMID- 42234171 OWN - NLM STAT- MEDLINE DCOM- 20260605 LR - 20260605 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 46 IP - 6 DP - 2026 Jun 3 TI - Myocarditis in idiopathic inflammatory myopathies: serologic and clinical correlates in a single-center registry. LID - 124 [pii] LID - 10.1007/s00296-026-06159-4 [doi] AB - To characterize the clinical and serological correlates of cardiovascular magnetic resonance (CMR)-confirmed myocarditis in idiopathic inflammatory myopathies (IIM), evaluate the diagnostic performance of high-sensitivity cardiac troponin I (hs-TnI), and quantify the independent prognostic impact of myocarditis relative to interstitial lung disease (ILD). Single-center retrospective cohort study (STROBE-compliant) of 142 consecutive adults with IIM (2019-2025). Myocarditis was confirmed by CMR according to the 2018 Lake Louise Criteria, performed both at diagnosis and during follow-up (with or without immunosuppression) upon clinical suspicion and/or elevated hs-TnI. Myositis-specific antibodies (MSA), myositis-associated antibodies (MAA), anti-Ro52 and antiphospholipid antibodies (aPL, Sydney criteria: ≥40 GPL/MPL units confirmed at ≥ 12 weeks) were systematically recorded. Associations were assessed with Fisher's exact test and logistic regression; multivariable models were pre-specified as parsimonious (two predictors) given the limited event count. Overall survival was analyzed with Kaplan-Meier curves, the log-rank test and Cox proportional-hazards regression. Myocarditis was confirmed in 9/142 patients (6.3%), without significant association with IIM class (P = 0.303) or with the antisynthetase antibody subgroup (anti-Jo-1 + PL-12 + PL-7 + EJ; 5.8% vs. 6.7%, P = 1.000). In univariable analyses, myocarditis was associated with Raynaud phenomenon (OR 13.6, 95% CI 2.3-80.0), aPL positivity (OR 10.7, 95% CI 2.6-43.9), anti-Ro52 coexisting with MSA/MAA (OR 8.7, 95% CI 2.2-34.8), anti-PM/Scl (OR 7.1, 95% CI 1.6-30.3), fever (OR 7.1, 95% CI 1.6-30.3) and ILD (OR 4.7, 95% CI 1.1-20.3). hs-TnI was markedly higher in myocarditis (median 366 vs. 3.5 ng/L; P < 0.001) with an area under the receiver-operating characteristic curve (AUROC) of 0.91 (95% CI 0.83-0.98). In the event-constrained multivariable model, Raynaud phenomenon (adjusted OR 13.1, 95% CI 1.5-112.7) and aPL (adjusted OR 7.2, 95% CI 1.4-36.0) remained independently associated. All-cause mortality was higher in myocarditis (44% vs. 9%; OR 7.95, 95% CI 2.0-31.5; log-rank P = 0.030; univariable Cox HR 3.77, 95% CI 1.03-13.78; P = 0.044), and persisted after adjustment for ILD in the logistic model (adjusted OR 5.9, 95% CI 1.3-26.1; P = 0.020), whereas ILD attenuated to a non-significant trend (adjusted OR 2.9, 95% CI 0.9-9.2; P = 0.073). In this single-center retrospective cohort, IIM-associated myocarditis was consistently associated with Raynaud phenomenon, aPL positivity and anti-Ro52 coexistence, and was associated with higher mortality than ILD. These findings support hs-TnI-guided triage to CMR as part of systematic cardiac surveillance in IIM, pending multicenter confirmation. CI - © 2026. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Álvarez-Troncoso, Jorge AU - Álvarez-Troncoso J AUID- ORCID: 0000-0003-1108-9461 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. jorge.alvarez.troncoso@gmail.com. FAU - Refoyo-Salicio, Elena AU - Refoyo-Salicio E AUID- ORCID: 0000-0003-4910-9827 AD - Cardiology Department, Hospital Universitario La Paz, IDIPAZ, Madrid, Spain. FAU - Díaz-Planellas, Samuel AU - Díaz-Planellas S AUID- ORCID: 0009-0000-7160-4483 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Carrasco-Molina, Sergio AU - Carrasco-Molina S AUID- ORCID: 0000-0002-5379-1665 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Soto-Abánades, Clara AU - Soto-Abánades C AUID- ORCID: 0009-0001-6544-0969 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Martínez-Robles, Elena AU - Martínez-Robles E AUID- ORCID: 0009-0002-6506-8746 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Gutiérrez-Sancerni, Belén AU - Gutiérrez-Sancerni B AUID- ORCID: 0009-0002-7119-6808 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Robles-Marhuenda, Ángel AU - Robles-Marhuenda Á AUID- ORCID: 0000-0003-1156-2570 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Noblejas-Mozo, Ana AU - Noblejas-Mozo A AUID- ORCID: 0000-0003-1282-6515 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. FAU - Ríos-Blanco, Juan José AU - Ríos-Blanco JJ AUID- ORCID: 0000-0001-5257-6042 AD - Systemic Immune-Mediated Diseases Unit, Internal Medicine Department, Hospital Universitario La Paz, IDIPAZ, Paseo de la Castellana, 261, 28046, Madrid, Spain. LA - eng PT - Journal Article DEP - 20260603 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Troponin I) RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - 0 (Antibodies, Antiphospholipid) SB - IM MH - Humans MH - *Myocarditis/blood/diagnosis/diagnostic imaging/immunology/etiology MH - Male MH - Female MH - *Myositis/complications/blood/immunology MH - Retrospective Studies MH - Middle Aged MH - Adult MH - *Troponin I/blood MH - Registries MH - Prognosis MH - Autoantibodies/blood MH - Lung Diseases, Interstitial/blood MH - Magnetic Resonance Imaging MH - Biomarkers/blood MH - Antibodies, Antiphospholipid/blood MH - Aged OTO - NOTNLM OT - Antiphospholipid syndrome OT - Autoantibodies OT - Magnetic resonance imaging, cine OT - Mortality OT - Myocarditis OT - Myositis OT - Raynaud disease OT - Troponin I COIS- Declarations. Conflict of interest: All authors declare no conflicts of interest relevant to this work, as defined by the ICMJE disclosure criteria. Use of artificial intelligence: No artificial intelligence tool was used in the conception, design, drafting, editing, analysis, or any other aspect of the preparation of this manuscript. Disclaimer: No part of this work has been copied or published, or posted online, in whole or in part. EDAT- 2026/06/03 12:38 MHDA- 2026/06/06 00:35 CRDT- 2026/06/03 11:15 PHST- 2026/03/20 00:00 [received] PHST- 2026/05/14 00:00 [accepted] PHST- 2026/06/06 00:35 [medline] PHST- 2026/06/03 12:38 [pubmed] PHST- 2026/06/03 11:15 [entrez] AID - 10.1007/s00296-026-06159-4 [pii] AID - 10.1007/s00296-026-06159-4 [doi] PST - epublish SO - Rheumatol Int. 2026 Jun 3;46(6):124. doi: 10.1007/s00296-026-06159-4. PMID- 21509800 OWN - NLM STAT- MEDLINE DCOM- 20110822 LR - 20131121 IS - 1096-8652 (Electronic) IS - 0361-8609 (Linking) VI - 86 IP - 7 DP - 2011 Jul TI - New sequential treatment with chemotherapy and reduced-intensity conditioning for allogeneic stem-cell transplantation in very high-risk acute myeloid leukemia. PG - 619-21 LID - 10.1002/ajh.22029 [doi] FAU - Cluzeau, Thomas AU - Cluzeau T AD - Department of Hematology, CHU Nice, France. cluzeau.thomas@gmail.com FAU - De Matteis, Muriel AU - De Matteis M FAU - Mounier, Nicolas AU - Mounier N FAU - Mannone, Lionel AU - Mannone L FAU - Gratecos, Nicole AU - Gratecos N FAU - Ticchioni, Michel AU - Ticchioni M FAU - Thyss, Antoine AU - Thyss A FAU - Raynaud, Sophie AU - Raynaud S FAU - Cassuto, Jill-Patrice AU - Cassuto JP FAU - Sirvent, Anne AU - Sirvent A LA - eng PT - Journal Article DEP - 20110420 PL - United States TA - Am J Hematol JT - American journal of hematology JID - 7610369 RN - 0 (Antilymphocyte Serum) RN - 6PLQ3CP4P3 (Etoposide) RN - 905Z5W3GKH (Thiotepa) RN - FA2DM6879K (Vidarabine) RN - P2K93U8740 (fludarabine) SB - IM MH - Adult MH - Antilymphocyte Serum/administration & dosage MH - Antineoplastic Combined Chemotherapy Protocols/*administration & dosage MH - Disease-Free Survival MH - Etoposide/administration & dosage MH - Female MH - Graft vs Host Disease/mortality/therapy MH - Humans MH - Leukemia, Myeloid, Acute/*mortality/*therapy MH - Male MH - Middle Aged MH - Retrospective Studies MH - *Stem Cell Transplantation MH - Survival Rate MH - Thiotepa/administration & dosage MH - Time Factors MH - *Transplantation Conditioning MH - Transplantation, Homologous MH - Vidarabine/administration & dosage/analogs & derivatives EDAT- 2011/04/22 06:00 MHDA- 2011/08/23 06:00 CRDT- 2011/04/22 06:00 PHST- 2011/04/22 06:00 [entrez] PHST- 2011/04/22 06:00 [pubmed] PHST- 2011/08/23 06:00 [medline] AID - 10.1002/ajh.22029 [doi] PST - ppublish SO - Am J Hematol. 2011 Jul;86(7):619-21. doi: 10.1002/ajh.22029. Epub 2011 Apr 20. PMID- 36439640 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240907 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 14 DP - 2022 TI - Higher body mass index is associated with a lower iloprost infusion rate tolerance and higher iloprost-related adverse events in patients with systemic sclerosis. PG - 1759720X221137125 LID - 10.1177/1759720X221137125 [doi] LID - 1759720X221137125 AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease characterized by vasospasm and microvascular involvement. Iloprost (ILO), a prostaglandin analogous, is used for the treatment of SSc-related Raynaud's phenomenon and digital ulcers. The suggested dose is 0.5-2 ng/kg/min for 6-8 h, and the maximum dose is decided upon the patient's tolerance. OBJECTIVES: This study aims to analyze ILO infusion tolerance and possible predictive factors in patients with SSc. DESIGN: This is a retrospective observational study. METHOD: We evaluated 113 patients with SSc beginning ILO intravenous (IV) infusion treatment between 2004 and 2021. We assessed the maximum tolerated ILO IV infusion rate, the incidence of adverse events (AEs), and the need for symptomatic therapy during the dose-finding sessions. We collected relevant demographic and medical and employed generalized linear models to assess possible predictors of maximum tolerated ILO infusion rate and AEs and logistic regression to assess predictors of AEs. RESULTS: The median ILO infusion rate at the end of the dose-finding process was 0.88 ng/kg/min [interquartile range (IQR) = 0.37]. We found a significant inverse correlation between ILO infusion rate and body mass index (BMI) at the beginning of treatment. BMI was negatively associated with ILO infusion rate (β = -0.21, p = 0.02) after correction for relevant confounding factors. Overweight patients (BMI >26) presented a 13-fold increased risk of developing AEs during ILO titration [adjusted odds ratio = 13.979, 95% confidence interval (CI) = 2.359-82.845]. AEs during ILO titration occurred in 47.8% of patients, of whom 22.2% presented hypotension. Other AEs were headache, nausea, vomiting, diarrhea, and edema. Symptomatic therapy was needed in half of the patients at least once. CONCLUSION: This study showed that higher BMI was statistically associated with lower ILO infusion rate tolerance and higher AEs rate, underlying a possible BMI-dependent endothelial dysfunction. Individual ILO regimens still need to be tailored to the patient. PLAIN LANGUAGE SUMMARY: Introduction: Systemic sclerosis is a rare a rheumatic disease characterized by skin thickening, vasospasm, and digital ulcers (DUs), as well as other organs involvement. Iloprost, which is administered as intravenous infusion, is one of the main treatments for this disease, and it is effective in reducing vasospasm and the frequency of DUs. Even if there is a suggested dose range, the exact dose must be tailored on each patient, because the tolerance to the drug is variable. Tolerance is limited by dose-dependent unwanted effects, as headache, low blood pressure, dizziness, and sickness. This study aimed to identify possible predictors of such tolerance.Materials and Methods: We collected data from our patients with systemic sclerosis beginning the treatment with iloprost between January 2004 and November 2021 at our hospital facility in Verona, Italy, and analyzed different factors that could be associated with a better tolerance, as age, sex, disease duration, smoking habit, body mass index (a measure of body fatness), blood pressure, concomitant medications, and different patterns of the disease.Results: We found that a higher body mass index was associated with lower iloprost tolerance and higher adverse events rate in patients with systemic sclerosis, while we did not find a correlation with other factors. We believe overweight and obese patients (who have a higher body mass index) have a defect in the vasodilatation mechanism and can therefore be more susceptible to the effect of this medication.Conclusions: While preliminary, our results could provide a good starting point to develop a predictive tool to limit adverse events during this therapy. CI - © The Author(s), 2022. FAU - Bixio, Riccardo AU - Bixio R AUID- ORCID: 0000-0001-9335-3371 AD - Rheumatology Section, Department of Medicine, University of Verona, Policlinico G.B. Rossi 10, 37134 Verona, Italy. FAU - Adami, Giovanni AU - Adami G AUID- ORCID: 0000-0002-8915-0755 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Bertoldo, Eugenia AU - Bertoldo E AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Giollo, Alessandro AU - Giollo A AUID- ORCID: 0000-0001-9355-7673 AD - Division of Rheumatology, University of Padova, Padova, Italy. FAU - Morciano, Andrea AU - Morciano A AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Bertelle, Davide AU - Bertelle D AUID- ORCID: 0000-0002-5901-7742 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Orsolini, Giovanni AU - Orsolini G AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Idolazzi, Luca AU - Idolazzi L AUID- ORCID: 0000-0002-7254-4686 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Rossini, Maurizio AU - Rossini M AUID- ORCID: 0000-0001-9692-2293 AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Viapiana, Ombretta AU - Viapiana O AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. LA - eng PT - Journal Article DEP - 20221122 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC9685102 OTO - NOTNLM OT - body mass index OT - iloprost OT - prostanoids OT - systemic sclerosis COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. The Associate Editor of Therapeutic Advances in Musculoskeletal Disease (Giovanni A is an author of this paper, therefore, the peer review process was managed by alternative members of the Board and the submitting Editor was not involved in the decision-making process. EDAT- 2022/11/29 06:00 MHDA- 2022/11/29 06:01 PMCR- 2022/11/22 CRDT- 2022/11/28 04:41 PHST- 2022/05/09 00:00 [received] PHST- 2022/10/12 00:00 [accepted] PHST- 2022/11/28 04:41 [entrez] PHST- 2022/11/29 06:00 [pubmed] PHST- 2022/11/29 06:01 [medline] PHST- 2022/11/22 00:00 [pmc-release] AID - 10.1177_1759720X221137125 [pii] AID - 10.1177/1759720X221137125 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2022 Nov 22;14:1759720X221137125. doi: 10.1177/1759720X221137125. eCollection 2022. PMID- 26071192 OWN - NLM STAT- MEDLINE DCOM- 20160325 LR - 20181113 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 17 IP - 1 DP - 2015 Jun 13 TI - Gene expression changes reflect clinical response in a placebo-controlled randomized trial of abatacept in patients with diffuse cutaneous systemic sclerosis. PG - 159 LID - 10.1186/s13075-015-0669-3 [doi] LID - 159 AB - INTRODUCTION: Systemic sclerosis is an autoimmune disease characterized by inflammation and fibrosis of the skin and internal organs. We sought to assess the clinical and molecular effects associated with response to intravenous abatacept in patients with diffuse cutaneous systemic. METHODS: Adult diffuse cutaneous systemic sclerosis patients were randomized in a 2:1 double-blinded fashion to receive abatacept or placebo over 24 weeks. Primary outcomes were safety and the change in modified Rodnan Skin Score (mRSS) at week 24 compared with baseline. Improvers were defined as patients with a decrease in mRSS of ≥30% post-treatment compared to baseline. Skin biopsies were obtained for differential gene expression and pathway enrichment analyses and intrinsic gene expression subset assignment. RESULTS: Ten subjects were randomized to abatacept (n = 7) or placebo (n = 3). Disease duration from first non-Raynaud's symptom was significantly longer (8.8 ± 3.8 years vs. 2.4 ± 1.6 years, p = 0.004) and median mRSS was higher (30 vs. 22, p = 0.05) in the placebo compared to abatacept group. Adverse events were similar in the two groups. Five out of seven patients (71%) randomized to abatacept and one out of three patients (33%) randomized to placebo experienced ≥30% improvement in skin score. Subjects receiving abatacept showed a trend toward improvement in mRSS at week 24 (-8.6 ± 7.5, p = 0.0625) while those in the placebo group did not (-2.3 ± 15, p = 0.75). After adjusting for disease duration, mRSS significantly improved in the abatacept compared with the placebo group (abatacept vs. placebo mRSS decrease estimate -9.8, 95% confidence interval -16.7 to -3.0, p = 0.0114). In the abatacept group, the patients in the inflammatory intrinsic subset showed a trend toward greater improvement in skin score at 24 weeks compared with the patients in the normal-like intrinsic subset (-13.5 ± 3.1 vs. -4.5 ± 6.4, p = 0.067). Abatacept resulted in decreased CD28 co-stimulatory gene expression in improvers consistent with its mechanism of action. Improvers mapped to the inflammatory intrinsic subset and showed decreased gene expression in inflammatory pathways, while non-improver and placebos showed stable or reverse gene expression over 24 weeks. CONCLUSIONS: Clinical improvement following abatacept therapy was associated with modulation of inflammatory pathways in skin. TRIAL REGISTRATION: ClinicalTrials.gov NCT00442611. Registered 1 March 2007. FAU - Chakravarty, Eliza F AU - Chakravarty EF AD - Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA. Eliza-Chakravarty@omrf.org. FAU - Martyanov, Viktor AU - Martyanov V AD - Department of Genetics, Geisel School of Medicine at Dartmouth, Hanover, NH, USA. Viktor.Martyanov@dartmouth.edu. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Dermatology, Stanford University School of Medicine, Stanford, CA, USA. fiorentino@stanford.edu. FAU - Wood, Tammara A AU - Wood TA AD - Department of Genetics, Geisel School of Medicine at Dartmouth, Hanover, NH, USA. Tammara.A.Wood@dartmouth.edu. FAU - Haddon, David James AU - Haddon DJ AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. djhaddon@stanford.edu. FAU - Jarrell, Justin Ansel AU - Jarrell JA AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. justin.ansel.jarrell@gmail.com. FAU - Utz, Paul J AU - Utz PJ AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. pjutz@stanford.edu. FAU - Genovese, Mark C AU - Genovese MC AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. genovese@stanford.edu. FAU - Whitfield, Michael L AU - Whitfield ML AD - Department of Genetics, Geisel School of Medicine at Dartmouth, Hanover, NH, USA. Michael.L.Whitfield@dartmouth.edu. FAU - Chung, Lorinda AU - Chung L AD - Department of Dermatology, Stanford University School of Medicine, Stanford, CA, USA. shauwei@stanford.edu. AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. shauwei@stanford.edu. AD - Palo Alto VA Health Care System, Palo Alto, CA, USA. shauwei@stanford.edu. LA - eng SI - ClinicalTrials.gov/NCT00442611 GR - P50AR060780/AR/NIAMS NIH HHS/United States GR - 1 U19-AI1110491/AI/NIAID NIH HHS/United States GR - Canadian Institutes of Health Research/Canada GR - U19 AI110491/AI/NIAID NIH HHS/United States GR - P50 AR060780/AR/NIAMS NIH HHS/United States GR - P30 AR061271/AR/NIAMS NIH HHS/United States GR - P30AR061271/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150613 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (CD28 Antigens) RN - 0 (Immunosuppressive Agents) RN - 0 (TMIGD2 protein, human) RN - 7D0YB67S97 (Abatacept) SB - IM MH - Abatacept/*therapeutic use MH - Adult MH - CD28 Antigens/*biosynthesis/drug effects MH - Double-Blind Method MH - Female MH - Gene Expression/*drug effects MH - Humans MH - Immunosuppressive Agents/*therapeutic use MH - Male MH - Middle Aged MH - Oligonucleotide Array Sequence Analysis MH - Scleroderma, Diffuse/*drug therapy PMC - PMC4487200 EDAT- 2015/06/14 06:00 MHDA- 2016/03/26 06:00 PMCR- 2015/06/13 CRDT- 2015/06/14 06:00 PHST- 2014/12/23 00:00 [received] PHST- 2015/06/01 00:00 [accepted] PHST- 2015/06/14 06:00 [entrez] PHST- 2015/06/14 06:00 [pubmed] PHST- 2016/03/26 06:00 [medline] PHST- 2015/06/13 00:00 [pmc-release] AID - 10.1186/s13075-015-0669-3 [pii] AID - 669 [pii] AID - 10.1186/s13075-015-0669-3 [doi] PST - epublish SO - Arthritis Res Ther. 2015 Jun 13;17(1):159. doi: 10.1186/s13075-015-0669-3. PMID- 22464314 OWN - NLM STAT- MEDLINE DCOM- 20121129 LR - 20260518 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 42 IP - 1 DP - 2012 Aug TI - Treatment of systemic sclerosis complications: what to use when first-line treatment fails--a consensus of systemic sclerosis experts. PG - 42-55 LID - 10.1016/j.semarthrit.2012.01.003 [doi] AB - OBJECTIVES: There is a need for standardization in systemic sclerosis (SSc) management. METHODS: SSc experts (n = 117) were sent 3 surveys to gain consensus for SSc management. RESULTS: First-line therapy for scleroderma renal crisis (SRC) was an angiotensin-converting enzyme inhibitor (ACEi). For SRC there were not many differences between treating mild or severe SRC. In general, Second-line was to add either a calcium channel blocker (CCB) or angiotensin receptor blocker (ARB) and then an alpha-blocker (66% agreed). Endothelin receptor agonists (ERAs) were the first treatment in mild pulmonary arterial hypertension (PAH) (72%), followed by adding a phosphodiesterase-5 inhibitor (PDE5i) (77%) and then a prostanoid (73%). For severe PAH, initial treatment was 1 of the following: a prostanoid (49%), combination of a ERA and a PDE5i (18%), or combination of a ERA and a prostanoid (16%) (71% agreed). For mild Raynaud's phenomenon (RF), after a CCB and adding a PDE5i (35%), trying an ARB (32%) and finally a prostanoid (23%) was suggested. For more severe RF, 54% agreed on adding a PDE5i (45%) or prostanoid (32%) to a CCB. In the prevention of digital ulcers (DU), initial treatment was a CCB (73%), then adding a PDE5i, then use of a ERA, and then a prostanoid (44% agreed). In interstitial lung disease/pulmonary fibrosis, for induction, usually intravenous cyclophosphamide and mycophenolate mofetil (MMF) or azathioprine were chosen. For maintenance, MMF was chosen by three-fourths (56% agreed). For gastroesophageal reflux disease, >50% would exceed the maximum recommended proton pump inhibitor dose if required (72% agreed). For skin involvement after methotrexate, MMF was usually chosen (37% agreement). For SSC-related inflammatory arthritis, methotrexate therapy (60%) was followed by adding corticosteroids (37%) or hydroxychloroquine (31%) (62% agreed). CONCLUSIONS: Discrepancies in drug choices occurred in treatment after first line in SSc. Not all algorithms had good agreement. This study provides some guidance for SSc management. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Walker, Kyle M AU - Walker KM AD - Trinity College, Dublin, Dublin, Ireland. FAU - Pope, Janet AU - Pope J CN - participating members of the Scleroderma Clinical Trials Consortium (SCTC) CN - Canadian Scleroderma Research Group (CSRG) LA - eng PT - Consensus Statement PT - Journal Article PT - Practice Guideline PT - Research Support, Non-U.S. Gov't DEP - 20120329 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Adrenergic alpha-Antagonists) RN - 0 (Angiotensin Receptor Antagonists) RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - 0 (Calcium Channel Blockers) SB - IM MH - Adrenergic alpha-Antagonists/therapeutic use MH - Algorithms MH - Angiotensin Receptor Antagonists/therapeutic use MH - Angiotensin-Converting Enzyme Inhibitors/therapeutic use MH - Calcium Channel Blockers/therapeutic use MH - *Disease Management MH - Drug Therapy, Combination MH - *Health Care Surveys/statistics & numerical data MH - Humans MH - Scleroderma, Systemic/complications/*drug therapy MH - Treatment Failure FIR - Alkassab, Firas IR - Alkassab F FIR - Catoggio Marco, Luis J IR - Catoggio Marco LJ FIR - Matucci-Cerinic IR - Matucci-Cerinic FIR - Chatterjee, Soumya IR - Chatterjee S FIR - Chung, Lorinda IR - Chung L FIR - Clements, Philip J IR - Clements PJ FIR - Connolly, M Kari IR - Connolly M FIR - Csuka, M E IR - Csuka ME FIR - Denton, Christopher P IR - Denton CP FIR - Distler, Oliver IR - Distler O FIR - Emery, Paul IR - Emery P FIR - Farge, Dominique IR - Farge D FIR - Fessler, Barri J IR - Fessler BJ FIR - Frech, Tracy M IR - Frech TM FIR - Goldberg, Avram IR - Goldberg A FIR - Griffing, Leroy IR - Griffing L FIR - Hachulla, Eric IR - Hachulla E FIR - Hayat, Samina IR - Hayat S FIR - Hsu, Vivien IR - Hsu V FIR - Inanc, Murat IR - Inanc M FIR - Johnson, Sindhu R IR - Johnson SR FIR - Kahaleh, Bashar IR - Kahaleh B FIR - Katsumoto, Tamiko R IR - Katsumoto TR FIR - Khanna, Dinesh IR - Khanna D FIR - Krieg, Thomas IR - Krieg T FIR - Lafyatis, Robert IR - Lafyatis R FIR - Lally, Edward V IR - Lally EV FIR - Lawrence, Able IR - Lawrence A FIR - Mahmud, Tafazzul H IR - Mahmud TH FIR - Martin, Richard W IR - Martin RW FIR - Mayes, Maureen D IR - Mayes MD FIR - McKown, Kevin IR - McKown K FIR - Medsger, Thomas A Jr IR - Medsger TA Jr FIR - Merkel, Peter A IR - Merkel PA FIR - Molitor, Jerry A IR - Molitor JA FIR - Mouthon, Luc IR - Mouthon L FIR - Proudman, Susanna IR - Proudman S FIR - Riemekasten, Gabriela IR - Riemekasten G FIR - Rodriguez-Reyna, Tatiana S IR - Rodriguez-Reyna TS FIR - Rudnicka, Lidia IR - Rudnicka L FIR - Seibold, James R IR - Seibold JR FIR - Senécal, Jean-Luc IR - Senécal JL FIR - Shapiro, Lee S IR - Shapiro LS FIR - Steen, Virginia IR - Steen V FIR - Stevens, Wendy IR - Stevens W FIR - Toloza, Sergio M A IR - Toloza SM FIR - Vacca, Alessandra IR - Vacca A FIR - Valentini, Gabriele IR - Valentini G FIR - Volkov, Suncica IR - Volkov S FIR - Voskuyl, Alexandre E IR - Voskuyl AE FIR - Wigley, Fredrick M IR - Wigley FM FIR - Wollheim, Frank A IR - Wollheim FA FIR - Baron, Murray IR - Baron M FIR - Docherty, Peter IR - Docherty P FIR - Hudson, Marie IR - Hudson M FIR - Kaminska, Elzbieta A IR - Kaminska EA FIR - Khalidi, Nader A IR - Khalidi NA FIR - Masetto, Ariel IR - Masetto A FIR - Smith, C Douglas IR - Smith C EDAT- 2012/04/03 06:00 MHDA- 2012/12/10 06:00 CRDT- 2012/04/03 06:00 PHST- 2011/07/12 00:00 [received] PHST- 2012/01/11 00:00 [revised] PHST- 2012/01/16 00:00 [accepted] PHST- 2012/04/03 06:00 [entrez] PHST- 2012/04/03 06:00 [pubmed] PHST- 2012/12/10 06:00 [medline] AID - S0049-0172(12)00017-0 [pii] AID - 10.1016/j.semarthrit.2012.01.003 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2012 Aug;42(1):42-55. doi: 10.1016/j.semarthrit.2012.01.003. Epub 2012 Mar 29. PMID- 38857012 OWN - NLM STAT- MEDLINE DCOM- 20240610 LR - 20250324 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 92 IP - 6S Suppl 4 DP - 2024 Jun 1 TI - Vasoactive and Antifibrotic Properties of Cannabinoids and Applications to Vasospastic/Vaso-Occlusive Disorders: A Systematic Review. PG - S445-S452 LID - 10.1097/SAP.0000000000003985 [doi] AB - BACKGROUND: Management of vasospastic and vaso-occlusive disorders is a complex challenge, with current treatments showing varied success. Cannabinoids have demonstrated both vasodilatory and antifibrotic properties, which present potential mechanisms for therapeutic relief. No existing review examines these effects in peripheral circulation in relation to vasospastic and vaso-occlusive disorders. This study aims to investigate vasodilatory and antifibrotic properties of cannabinoids in peripheral vasculature for application in vasospastic and vaso-occlusive disorders affecting the hand. METHODS: A systematic search was conducted by 2 independent reviewers across PubMed, Cochrane, Ovid MEDLINE, and CINAHL to identify studies in accordance with the determined inclusion/exclusion criteria. Information regarding study design, medication, dosage, and hemodynamic or antifibrotic effects were extracted. Descriptive statistics were used to summarize study findings as appropriate. RESULTS: A total of 584 articles were identified, and 32 were selected for inclusion. Studies were grouped by effect type: hemodynamic (n = 17, 53%) and antifibrotic (n = 15, 47%). Vasodilatory effects including reduced perfusion pressure, increased functional capillary density, inhibition of vessel contraction, and increased blood flow were reported in 82% of studies. Antifibrotic effects including reduced dermal thickening, reduced collagen synthesis, and reduced fibroblast migration were reported in 100% of studies. CONCLUSION: Overall, cannabinoids were found to have vasodilatory and antifibrotic effects on peripheral circulation via both endothelium-dependent and independent mechanisms. Our review suggests the applicability of cannabis-based medicines for vasospastic and vaso-occlusive disorders affecting the hand (eg, Raynaud disease, Buerger disease). Future research should aim to assess the effectiveness of cannabis-based medicines for these conditions. CI - Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved. FAU - Li, Gabrielle AU - Li G AD - From the University of Virginia School of Medicine, Charlottesville, VA. FAU - Choi, Janice AU - Choi J AD - From the University of Virginia School of Medicine, Charlottesville, VA. FAU - Stephens, Kristen L AU - Stephens KL AUID- ORCID: 0000-0002-5508-4593 AD - Department of Plastic Surgery, University of Virginia Health System, Charlottesville, VA. FAU - DeGeorge, Brent R Jr AU - DeGeorge BR Jr AD - Department of Plastic Surgery, University of Virginia Health System, Charlottesville, VA. LA - eng PT - Journal Article PT - Systematic Review PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 RN - 0 (Cannabinoids) RN - 0 (Vasodilator Agents) RN - 0 (Antifibrotic Agents) SB - IM MH - Humans MH - *Cannabinoids/pharmacology/therapeutic use MH - Vasodilator Agents/therapeutic use/pharmacology MH - Antifibrotic Agents/pharmacology/therapeutic use MH - Fibrosis/drug therapy COIS- Conflict of interest and sources of funding: none declared. EDAT- 2024/06/10 13:50 MHDA- 2024/06/10 13:51 CRDT- 2024/06/10 11:33 PHST- 2024/06/10 13:51 [medline] PHST- 2024/06/10 13:50 [pubmed] PHST- 2024/06/10 11:33 [entrez] AID - 00000637-202406004-00019 [pii] AID - 10.1097/SAP.0000000000003985 [doi] PST - ppublish SO - Ann Plast Surg. 2024 Jun 1;92(6S Suppl 4):S445-S452. doi: 10.1097/SAP.0000000000003985. PMID- 35891592 OWN - NLM STAT- MEDLINE DCOM- 20221118 LR - 20221118 IS - 2795-4552 (Electronic) IS - 2795-4552 (Linking) VI - 1 IP - ARP Rheumatology, nº3 2022 DP - 2022 Nov 1 TI - Clinical characterisation of a multicentre nationwide cohort of patients with antisynthetase syndrome. PG - 190-196 AB - BACKGROUND: Antisynthetase syndrome (ASyS) is characterised by the association of inflammatory myopathy, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon (RP) or mechanic's hands (MH), with the presence of anti-aminoacyl-tRNA-synthetase antibodies (anti-ARS). It has been suggested that different anti-ARS may be associated with distinct clinical pictures. OBJECTIVE: To characterise the clinical and immunological features of a multicentric nationwide cohort of ASyS patients. METHODS: This is a multicentre retrospective cohort study including patients with ASyS from nine Portuguese rheumatology centres. Data on patients' demographics, signs and symptoms, laboratory results, pulmonary imaging findings and treatment with immunomodulators were collected. Comparison between patients with different anti-ARS antibodies was made using the Chi-square test for categorical variables and Student's t-test or Man-Whitney test for continuous variables, considering anti-Jo1 positive patients as the reference group. RESULTS: Seventy patients were included (70% female) with a median age in years at disease onset of 52 (15-75) years and median follow-up time of 3 years (range 0-32). The three most common clinical manifestations were ILD (n=53, 75.7%), followed by arthritis (n=43, 61.4%) and myositis (n=37, 52.9%). Forty-three patients were positive for anti-Jo1 (61.4%), 11 for anti-PL12 (15.7%), 10 for anti-PL7 (14.3%), 4 for anti-EJ (5.7%), and 2 for anti-OJ (2.9%) antibodies. Antibody co-positivity with anti-Ro52 antibodies was found in 15 patients (21.4%) and was more prevalent in anti-Jo1 patients. ILD prevalence was similar in the different anti-ARS subgroups, without statistically significant differences. Patients positive for anti-PL7 antibodies had significantly lower risk of presenting arthritis (p =< 0.05) and those positive for anti-PL-12 antibodies had a significantly lower risk of presenting myositis than the reference group of anti-Jo1 positive patients (p =< 0.05). RP was more frequently found in patients positive for anti-PL-12 than in anti-Jo1-positive patients (p =< 0.05). Malignancies were reported in four (5.7%) patients, none of whom were anti-Ro52-positive, and one of such patients had a double malignancy. Only three deaths were reported. Corticosteroids were the most frequently prescribed therapy and the use of immunosuppressive drugs was decided according to the type of predominant clinical manifestation. CONCLUSION: The three most common clinical manifestations were ILD, followed by arthritis and myositis. Patients positive for anti-PL7 antibodies had significantly lower risk of presenting arthritis and those positive for anti-PL-12 antibodies had a significantly lower risk of presenting myositis than the reference group of anti-Jo1 positive patients. RP was more frequently found in patients positive for anti-PL-12 than in anti-Jo1-positive patients. Corticosteroids were the most frequently prescribed therapy. These results are generally concordant with data retrieved from international cohorts. FAU - Martins, Patrícia AU - Martins P AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Dourado, Eduardo AU - Dourado E AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Melo, Ana Teresa AU - Melo AT AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Samões, Beatriz AU - Samões B AD - Serviço de Reumatologia, Centro Hospitalar Vila Nova de Gaia/Espinho. FAU - Sousa, Marlene AU - Sousa M AD - Serviço de Reumatologia, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Freitas, Raquel AU - Freitas R AD - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal. FAU - Lourenço, Maria Helena AU - Lourenço MH AD - Serviço de Reumatologia, Centro Hospitalar Lisboa Ocidental, Hospital Egas Moniz, Lisboa, Portugal. FAU - Fernandes, Bruno AU - Fernandes B AD - Serviço de Reumatologia, Centro Hospitalar e Universitário São João, Porto, Portugal. FAU - Costa, Emanuel AU - Costa E AD - Serviço de Reumatologia, Hospital de Braga, Braga, Portugal. FAU - Parente, Hugo AU - Parente H AD - Serviço de Reumatologia, Unidade Local de Saúde Do Alto Minho, Ponte de Lima, Portugal. FAU - Martins, Frederico AU - Martins F AD - Serviço de Reumatologia, Centro Hospitalar Universitário do Algarve, Faro, Portugal. FAU - Fonseca, João Eurico AU - Fonseca JE AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Cordeiro, Inês AU - Cordeiro I AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Romão, Vasco C AU - Romão VC AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Khmelinskii, Nikita AU - Khmelinskii N AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. FAU - Campanilho-Marques, Raquel AU - Campanilho-Marques R AD - Serviço de Reumatologia e Doenças Ósseas Metabólicas, Centro Hospitalar Universitário Lisboa Norte; Unidade de Investigação em Reumatologia, IMM, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa. LA - eng PT - Journal Article PT - Multicenter Study TT - Clinical characterisation of a multicentre nationwide cohort of patients with antisynthetase syndrome. DEP - 20221101 PL - Portugal TA - ARP Rheumatol JT - ARP rheumatology JID - 9918402287906676 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) RN - Antisynthetase syndrome SB - IM MH - Humans MH - Female MH - Male MH - Retrospective Studies MH - Autoantibodies MH - *Myositis/drug therapy MH - *Lung Diseases, Interstitial/diagnosis MH - Cohort Studies MH - Antibodies, Antinuclear/therapeutic use MH - *Arthritis/diagnosis EDAT- 2022/07/28 06:00 MHDA- 2022/11/19 06:00 CRDT- 2022/07/27 02:05 PHST- 2022/07/27 02:05 [entrez] PHST- 2022/07/28 06:00 [pubmed] PHST- 2022/11/19 06:00 [medline] AID - AO220020 [pii] PST - epublish SO - ARP Rheumatol. 2022 Nov 1;1(ARP Rheumatology, nº3 2022):190-196. PMID- 28628467 OWN - NLM STAT- MEDLINE DCOM- 20180301 LR - 20220330 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 35 IP - 6 DP - 2017 Nov-Dec TI - Juvenile- and adult-onset systemic lupus erythematosus: a comparative study in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER). PG - 1047-1055 AB - OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years. RESULTS: We reviewed 3,428 aSLE patients (89.6% women) and 484 jSLE patients (89.8% girls), 93% Caucasian (both groups). Mean age at diagnosis was 38.1±14 and 16.6±6.3 years (p<0.001) and mean age at the end of follow-up was 48.8±14.3 and 31.5±30 years (p<0.001), respectively. jSLE showed significantly more clinical (including lymphadenopathy, fever, malar rash, mucosal ulcers, pericarditis, pleuritis, Raynaud's phenomenon, lupus nephritis, recurrent nephritis, histologic nephritis changes, thrombocytopenia, haemolytic anaemia, thrombotic thrombocytopenic purpura, seizures, lupus headache and organic brain syndrome) and immunological (a-dsDNA and a-Sm antibodies, hypocomplementaemia) involvement than did aSLE, except for secondary Sjögren's syndrome, a-Ro antibodies, fibromyalgia and osteoporosis. jSLE also showed more SLE family history, longer diagnosis delay, higher SLEDAI and Katz scores, but lower Charlson scores than aSLE. Several specific domains were more frequently involved in SLICC/ACR DI in jSLE. jSLE patients more frequently underwent all SLE-related treatment and procedures, as well as dialysis and kidney transplantations. CONCLUSIONS: jSLE shares many clinical and serological features with aSLE. However, jSLE patients typically manifested more activity, severity, cumulative damage in certain areas, than their aSLE counterparts. FAU - Torrente-Segarra, Vicenç AU - Torrente-Segarra V AD - Department of Rheumatology, Hospitalet-Sant Joan Despí Moisès Broggi University General Hospital, Hospitalet Llobregat, Spain. vtorrente@hsjdbcn.org. FAU - Salman Monte, Tarek Carlos AU - Salman Monte TC AD - Department of Rheumatology, Parc de Salut Mar-IMIM, Barcelona, Spain. FAU - Rúa-Figueroa, Iñigo AU - Rúa-Figueroa I AD - Department of Rheumatology, Doctor Negrín University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain. FAU - Sánchez-Alonso, Fernando AU - Sánchez-Alonso F AD - Research Unit, Spanish Society of Rheumatology (SER), Madrid, Spain. FAU - López-Longo, Francisco Javier AU - López-Longo FJ AD - Rheumatology Department, Gregorio Marañón University Hospital, Madrid, Spain. FAU - Galindo-Izquierdo, María AU - Galindo-Izquierdo M AD - Rheumatology Department, Doce de Octubre University Hospital, Madrid, Spain. FAU - Calvo-Alén, Jaime AU - Calvo-Alén J AD - Rheumatology Department, Sierrallana Hospital, Torrelavega, Spain. FAU - Olivé-Marqués, Alejandro AU - Olivé-Marqués A AD - Rheumatology Department, Germans Trías i Pujol University Hospital, Badalona, Spain. FAU - Ibañez-Ruán, Jesús AU - Ibañez-Ruán J AD - Rheumatology Unit, Clinica POVISA, Vigo, Spain. FAU - Horcada, Loreto AU - Horcada L AD - Department of Rheumatology, Navarra Hospital, Navarra, Pamplona, Spain. FAU - Sánchez-Atrio, Ana AU - Sánchez-Atrio A AD - Department of Rheumatology, Príncipe de Asturias University Hospital, Madrid, Spain. FAU - Montilla, Carlos AU - Montilla C AD - Department of Rheumatology, Salamanca Clinic University Hospital, Salamanca, Spain. FAU - Melero González, Rafael Benito AU - Melero González RB AD - Department of Rheumatology Department, Complexo Hospitalario Universitario de Ourense, Spain. FAU - Díez-Álvarez, Elvira AU - Díez-Álvarez E AD - Department of Rheumatology, León Hospital, Spain. FAU - Martinez-Taboada, Victor AU - Martinez-Taboada V AD - Department of Rheumatology, Marqués de Valdecilla University Hospital, Santander, Spain. FAU - Andreu, José Luis AU - Andreu JL AD - Rheumatology Department, Puerta de Hierro-Majadahonda Hospital, Madrid, Spain. FAU - Fernández-Berrizbeitia, Olaia AU - Fernández-Berrizbeitia O AD - Department of Rheumatology, Basurto Hospital, Spain. FAU - Hernández-Beriain, José Ángel AU - Hernández-Beriain JÁ AD - Rheumatology Department, Hospital Insular of Gran Canaria, Gran Canaria, Las Palmas de Gran Canaria, Spain. FAU - Gantes, Marian AU - Gantes M AD - Rheumatology Department, Tenerife Clinic Hospital, Santa Cruz de Tenerife, Spain. FAU - Hernández-Cruz, Blanca AU - Hernández-Cruz B AD - Department of Rheumatology, Virgen Macarena Hospital, Sevilla, Spain. FAU - Pecondón-Español, Ángela AU - Pecondón-Español Á AD - Department of Rheumatology, Miguel Servet University Hospital, Zaragoza, Spain. FAU - Marras, Carlos AU - Marras C AD - Department of Rheumatology, Virgen de la Arrixaca University Hospital, Murcia, Spain. FAU - Bonilla, Gema AU - Bonilla G AD - Department of Rheumatology, La Paz University Hospital, Madrid, Spain. FAU - Pego-Reigosa, José M AU - Pego-Reigosa JM AD - Department of Rheumatology, Complexo Hospitalario Universitario de Vigo, Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain. CN - RELESSER Study Group of the Spanish Society of Rheumatology (SER) and the Study Group of Systemic Autoimmune Diseases of the SER (EAS-SER). LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20170612 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Child MH - Cohort Studies MH - Cross-Sectional Studies MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*complications/immunology MH - Male MH - Middle Aged MH - Registries MH - Severity of Illness Index MH - Young Adult EDAT- 2017/06/20 06:00 MHDA- 2018/03/02 06:00 CRDT- 2017/06/20 06:00 PHST- 2017/01/24 00:00 [received] PHST- 2017/04/03 00:00 [accepted] PHST- 2017/06/20 06:00 [pubmed] PHST- 2018/03/02 06:00 [medline] PHST- 2017/06/20 06:00 [entrez] AID - 11549 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2017 Nov-Dec;35(6):1047-1055. Epub 2017 Jun 12. PMID- 34856663 OWN - NLM STAT- MEDLINE DCOM- 20211206 LR - 20211214 IS - 0578-1310 (Print) IS - 0578-1310 (Linking) VI - 59 IP - 12 DP - 2021 Dec 2 TI - [Clinical characteristics of 25 patients with type Ⅰ interferonopathies]. PG - 1043-1047 LID - 10.3760/cma.j.cn112140-20211004-00843 [doi] AB - Objective: To summarize the clinical characteristics of type I interferonopathies and provide clues for early identification and diagnosis. Methods: Clinical data of 20 patients admitted to Department of Pediatrics, Peking Union Medical College Hospital and 5 patients admitted to Department of Rheumatology and Immunology, Shenzhen Children's Hospital from January 2016 to September 2021 were retrospectively analyzed. The data included gene results, clinical manifestations and auxiliary examination results. Results: Of the 25 cases, 12 were males and 13 were females. Age of onset ranged from 1 day to 11 years. And 84% of them had the onset before the age of 3 years. The cases consisted of 14 cases of Aicardi-Goutières syndrome (AGS), 6 cases of adenosine deaminase 2 deficiency (DADA2), 3 cases of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), and 2 cases of Spondyloenchondrodysplasia with immune dysregulation (SPENCDI). Eighteen patients (72%) experienced neurologic disorder, among whom 16 (64%) showed intracranial calcification, 11 (44%) had dystonia, 10 (40%) had leukodystrophy, 6 (24%) had epilepsy, 5 (20%) had brain atrophy and 5 (20%) had early-onset cerebrovascular events. Skin involvement occurred in 15 cases (60%), among whom 8 cases (32%) had chilblain-like rash, 4 cases (16%) had livedo reticularis, 3 cases (12%) had erythema, 2 cases (8%) had erythema nodosum and 2 cases (8%) had Raynaud's phenomenon. In addition, 12 cases (48%) had positive autoimmune antibodies, 10 cases (40%) manifested as developmental retardation, 8 cases (32%) experienced lung interstitial lesions, and 7 cases (28%) demonstrated thyroid dysfunction. And 1 died (4%) at 11 years of age. Conclusions: Type Ⅰinterferonopathies can involve multiple organs, and share the characteristics of systemic inflammatory and autoimmune diseases. The early-onset neurological symptoms (early-onset cerebrovascular events, intracranial calcification, leukodystrophy and cerebral atrophy), rashes (chilblain-like rash, livedo reticularis and erythema), positive autoimmune antibodies, developmental delay, interstitial lung disease and thyroid dysfunction may indicate type Ⅰ interferonopathies. FAU - Wang, W AU - Wang W AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Wang, W AU - Wang W AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Zou, L P AU - Zou LP AD - Department of Pediatrics, Chinese People's Liberation Army General Hospital, Beijing 100039, China. FAU - He, T Y AU - He TY AD - Department of Rheumatology and Immunology, Shenzhen Children's Hospital, Shenzhen 518034, China. FAU - Ma, M S AU - Ma MS AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Li, W D AU - Li WD AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Yu, Z X AU - Yu ZX AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. FAU - Yang, J AU - Yang J AD - Department of Rheumatology and Immunology, Shenzhen Children's Hospital, Shenzhen 518034, China. FAU - Song, H M AU - Song HM AD - Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China. LA - chi GR - 2021-12M-C&T-B-008/CAMS Innovation Fund for Medical Sciences/ GR - 2016YFC0901500/National Key Research and Development Program of China/ GR - 2016-2-40114/Captial Health Research and Development of Special/ GR - 201402012/Public Welfare Scientific Research Project of China/ GR - L202050/Beijing Natural Science Foundation/ PT - Journal Article PL - China TA - Zhonghua Er Ke Za Zhi JT - Zhonghua er ke za zhi = Chinese journal of pediatrics JID - 0417427 RN - 0 (Intercellular Signaling Peptides and Proteins) RN - EC 3.5.4.4 (Adenosine Deaminase) SB - IM MH - Adenosine Deaminase/genetics MH - *Autoimmune Diseases of the Nervous System/genetics MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Infant MH - Intercellular Signaling Peptides and Proteins MH - Male MH - *Nervous System Malformations MH - Retrospective Studies EDAT- 2021/12/03 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/12/02 21:10 PHST- 2021/12/02 21:10 [entrez] PHST- 2021/12/03 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] AID - 10.3760/cma.j.cn112140-20211004-00843 [doi] PST - ppublish SO - Zhonghua Er Ke Za Zhi. 2021 Dec 2;59(12):1043-1047. doi: 10.3760/cma.j.cn112140-20211004-00843. PMID- 41730565 OWN - NLM STAT- In-Process DCOM- 20260223 LR - 20260608 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 13 IP - 1 DP - 2026 Feb 23 TI - Sex-based clinical and immunological differences across lupus erythematosus subtypes: a cross-sectional multicentre study from China. LID - 10.1136/lupus-2025-001783 [doi] LID - e001783 AB - OBJECTIVE: To investigate sex-related differences in clinical and immunological features across lupus erythematosus (LE) subtypes. METHODS: This cross-sectional analysis, based on the Lupus Erythematosus Multicenter Case-Control Study in Chinese populations (ChiCTR2100048939), included patients with SLE and major cutaneous LE (CLE) subtypes. Sex-specific comparisons were performed using R V.4.4.2. RESULTS: In 2097 patients (1865 SLE, 1648 CLE), female predominance was observed in all subtypes, with female-to-male ratios ranging from 11.3:1 (acute CLE, ACLE) to 2.1:1 (isolated CLE, iCLE). Except for ACLE, females had earlier or similar onset than males in all other subtypes. ACLE lesions were most common in females (67%). In male patients with LE, the proportion of discoid LE (DLE) lesions was higher than female patients (31% vs 12%). Compared with males, females exhibited higher frequencies of arthritis in SLE, ACLE, DLE and chilblain LE (CHLE). In DLE, renal involvement, haematological abnormalities and serositis were more frequently observed in females. In subacute CLE (SCLE), haematological abnormalities were significantly more common in females. Additionally, non-scarring alopecia was more common in females than in males. Females had higher autoantibody positivity in iCLE and chronic CLE, with significant differences in anti-double-stranded DNA, anti-Smith, anti-U1-nuclear ribonucleoprotein and anti-ribosomal P antibodies. CONCLUSIONS: Across the subtypes, several clinical manifestations show a consistent sex distribution: ACLE lesions, arthritis, non-scarring alopecia, Raynaud's phenomenon and autoantibodies occur more frequently in women with LE, whereas the proportions of DLE and SCLE lesions are higher in men with LE. In addition, certain features exhibit subtype-specific sex differences: among patients with SCLE, DLE and CHLE, women show a greater propensity for systemic involvement, whereas in those with SLE and ACLE, men demonstrate a higher tendency toward systemic disease. TRIAL REGISTRATION NUMBER: ChiCTR2100048939. CI - © Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY. Published by BMJ Group. FAU - Pan, Yu AU - Pan Y AD - Department of Epidemiology and Biostatistics, School of Public Health, Nanjing Medical University, Nanjing, Jiangsu, China. AD - Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, Jiangsu, China. FAU - Jin, Hui AU - Jin H AD - Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, Jiangsu, China. AD - Key Laboratory of Basic and Translational Research on Immune-Mediated Skin Diseases, Chinese Academy of Medical Sciences, Nanjing, Jiangsu, China. FAU - Zhou, Shihang AU - Zhou S AD - Department of Dermatology and Cosmetic Medicine Center, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China. FAU - Ying, Zhang AU - Ying Z AD - Department of Infection Control and Public Health, The Second Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang, China. FAU - Bai, Leilei AU - Bai L AD - Department of Epidemiology and Biostatistics, School of Public Health, Nanjing Medical University, Nanjing, Jiangsu, China. AD - Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, Jiangsu, China. FAU - Lu, Qianjin AU - Lu Q AUID- ORCID: 0000-0002-4192-4897 AD - Department of Epidemiology and Biostatistics, School of Public Health, Nanjing Medical University, Nanjing, Jiangsu, China qianlu5860@pumcderm.cams.cn. AD - Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, Jiangsu, China. AD - Key Laboratory of Basic and Translational Research on Immune-Mediated Skin Diseases, Chinese Academy of Medical Sciences, Nanjing, Jiangsu, China. LA - eng PT - Journal Article DEP - 20260223 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Male MH - Female MH - Cross-Sectional Studies MH - China/epidemiology MH - Adult MH - *Lupus Erythematosus, Systemic/immunology/epidemiology MH - Case-Control Studies MH - Sex Factors MH - *Lupus Erythematosus, Cutaneous/immunology/epidemiology MH - Middle Aged MH - Young Adult MH - Adolescent MH - Autoantibodies/blood MH - Lupus Erythematosus, Discoid/immunology/epidemiology PMC - PMC12931554 OTO - NOTNLM OT - Antibodies OT - Autoimmune Diseases OT - Autoimmunity OT - Lupus Erythematosus, Systemic COIS- Competing interests: None declared. EDAT- 2026/02/24 00:30 MHDA- 2026/02/24 00:31 PMCR- 2026/02/23 CRDT- 2026/02/23 20:43 PHST- 2025/08/28 00:00 [received] PHST- 2026/01/23 00:00 [accepted] PHST- 2026/02/24 00:31 [medline] PHST- 2026/02/24 00:30 [pubmed] PHST- 2026/02/23 20:43 [entrez] PHST- 2026/02/23 00:00 [pmc-release] AID - 13/1/e001783 [pii] AID - lupus-2025-001783 [pii] AID - 10.1136/lupus-2025-001783 [doi] PST - epublish SO - Lupus Sci Med. 2026 Feb 23;13(1):e001783. doi: 10.1136/lupus-2025-001783. PMID- 39832094 OWN - NLM STAT- MEDLINE DCOM- 20250502 LR - 20250522 IS - 1724-6059 (Electronic) IS - 1121-8428 (Linking) VI - 38 IP - 2 DP - 2025 Mar TI - Clinicopathological characteristics and prognosis of lupus nephritis patients with positive anti-ribonucleoprotein antibodies. PG - 633-641 LID - 10.1007/s40620-024-02177-2 [doi] AB - BACKGROUND: Positive anti-ribonucleoprotein antibodies may characterize a subgroup of patients affected by lupus nephritis with mild kidney damage, but little is known about their clinical features and long-term prognosis. METHODS: Patients were retrospectively selected from the lupus nephritis database ( http://ln.medidata.cn ) of the First Affiliated Hospital of Sun Yat-sen University between 2006 and 2011. Logistic regression analysis identified the clinicopathological indicators related to positive anti-ribonucleoprotein antibodies. Additionally, the Cox proportional hazard regression model was used to assess the association of baseline variables with clinical outcomes. RESULTS: Of the 485 enrolled patients, 184 (37.9%) tested positive for anti-ribonucleoprotein antibodies. The group with positive anti-ribonucleoprotein antibodies exhibited a higher prevalence of rash, photosensitivity, and Raynaud's phenomenon, and lower scores on the systemic lupus erythematosus disease activity index (SLEDAI) and the Activity Index scores in kidney biopsies. It is important to note that, although proteinuria did not differ, patients with anti-ribonucleoprotein positivity had a lower prevalence of hematuria and cylindruria, and a higher estimated glomerular filtration rate than patients without anti-ribonucleoprotein antibodies. After a median follow-up of approximately 170 months, no significant differences were observed in kidney or patient survival between groups. CONCLUSIONS: Lupus nephritis patients with anti-ribonucleoprotein antibodies present milder kidney damage and more dermatological manifestations. Despite the negative correlation between anti-ribonucleoprotein antibodies and both SLEDAI and activity index scores, these antibodies may not be predictive of better kidney outcomes. CI - © 2025. The Author(s) under exclusive licence to Italian Society of Nephrology. FAU - Kang, Di AU - Kang D AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. FAU - Zhang, Manhuai AU - Zhang M AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. FAU - Chen, Zhiqing AU - Chen Z AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. FAU - Zheng, Zhihua AU - Zheng Z AD - Department of Nephrology, Center of Kidney and Urology, The Seventh Affiliated Hospital, Sun Yat-sen University, Shenzhen, People's Republic of China. FAU - Tang, Ruihan AU - Tang R AUID- ORCID: 0000-0001-5250-6239 AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. tangrh8@mail.sysu.edu.cn. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. tangrh8@mail.sysu.edu.cn. FAU - Xia, Xi AU - Xia X AUID- ORCID: 0000-0003-3146-5567 AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. xiax29@mail.sysu.edu.cn. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. xiax29@mail.sysu.edu.cn. FAU - Chen, Wei AU - Chen W AUID- ORCID: 0000-0001-7194-8040 AD - Department of Nephrology, The First Affiliated Hospital of Sun Yat-sen University, 58Th, Zhongshan Road II, Guangzhou, 510080, People's Republic of China. chenwei99@mail.sysu.edu.cn. AD - Key Laboratory of Nephrology, Ministry of Health and Guangdong Province, Guangzhou, People's Republic of China. chenwei99@mail.sysu.edu.cn. LA - eng GR - 81970599/National Natural Science Foundation of China/ GR - 82170737/National Natural Science Foundation of China/ GR - 82370707/National Natural Science Foundation of China/ GR - SL2023A04J02091/Natural Science Foundation of Guangzhou Municipality/ PT - Journal Article DEP - 20250120 PL - Italy TA - J Nephrol JT - Journal of nephrology JID - 9012268 RN - 0 (Ribonucleoproteins) RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Lupus Nephritis/immunology/blood/physiopathology/diagnosis/mortality/pathology MH - Female MH - Male MH - Retrospective Studies MH - Adult MH - Prognosis MH - Middle Aged MH - Young Adult MH - Proportional Hazards Models MH - *Ribonucleoproteins/immunology MH - Glomerular Filtration Rate MH - *Antibodies, Antinuclear/blood MH - Biomarkers/blood MH - Logistic Models MH - Risk Factors MH - Time Factors MH - *Kidney/pathology/physiopathology/immunology MH - Proteinuria/epidemiology MH - Kaplan-Meier Estimate MH - Databases, Factual MH - Severity of Illness Index MH - Hematuria/epidemiology OTO - NOTNLM OT - Anti-RNP antibodies OT - Kidney outcome OT - Lupus nephritis OT - Mortality COIS- Declarations. Conflict of interest: All the authors declared no competing interests. Ethical approval and consent to participate: The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013). All study participants provided signed informed consents. Statement of human and animal rights: All procedures were approved by the Human Ethics Committees of Sun Yat-sen University. Consent for publication: Not applicable. EDAT- 2025/01/20 22:54 MHDA- 2025/04/02 00:25 CRDT- 2025/01/20 11:22 PHST- 2024/06/07 00:00 [received] PHST- 2024/11/27 00:00 [accepted] PHST- 2025/04/02 00:25 [medline] PHST- 2025/01/20 22:54 [pubmed] PHST- 2025/01/20 11:22 [entrez] AID - 10.1007/s40620-024-02177-2 [pii] AID - 10.1007/s40620-024-02177-2 [doi] PST - ppublish SO - J Nephrol. 2025 Mar;38(2):633-641. doi: 10.1007/s40620-024-02177-2. Epub 2025 Jan 20. PMID- 37465567 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230918 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 15 DP - 2023 TI - Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy. PG - 1759720X231181336 LID - 10.1177/1759720X231181336 [doi] LID - 1759720X231181336 AB - BACKGROUND: Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied. OBJECTIVE: We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis. DESIGN: This is a retrospective cohort study. METHODS: We reviewed the clinical data of myositis patients with anti-U1RNP antibodies and compared them to those with anti-signal recognition particle (SRP) and hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-associated immune-mediated necrotizing myopathy (IMNM). RESULTS: A total of 30 adult cases were identified; median age was 47.5 years and 24 (80%) were females, and 12 patients did not coexist with myositis-specific antibodies (MSAs) (isolated anti-U1RNP). The serum creatine kinase (CK) was significantly higher in patients with isolated anti-U1RNP (2182.5 U/L versus 289 U/L, p = 0.01), and the number of patients with CK > 2000 U/L was higher compared to that in anti-U1RNP antibody patients coexisting with MSAs (66.7% versus 16.7%, p = 0.009). The prevalence of IMNM in patients' muscle pathology with isolated anti-U1RNP was significantly higher (75%, p = 0.003). Skin rashes were less common in isolated anti-U1RNP group (p < 0.05). Of the 25 individuals with available pulmonary high-resolution CT (HRCT), 14 (56%) were diagnosed with interstitial lung disease (ILD). The incidence of muscular weakness, dysphagia, or levels of CK was not different between the isolated anti-U1RNP antibody individuals and the anti-HMGCR or SRP-IMNM groups (p > 0.05). But the frequency of Raynaud's phenomenon, arthritis, and membrane attack complex (MAC) deposits in myositis patients with isolated anti-U1RNP antibodies were higher than in anti-HMGCR and SRP-IMNM (all p < 0.005). There was no difference between anti-U1RNP patients with and without Ro-52 (p > 0.05). Isolated anti-U1RNP individuals showed marked improvements in muscle strength, and the remission rate in 1 and 2 years was significantly higher than that in anti-HMGCR and SRP-IMNM (p < 0.05). CONCLUSIONS: The clinical and pathological features of myositis patients with isolated anti-U1RNP antibodies were similar to IMNM. Arthritis and ILD are the most common extramuscular clinical features. Most respond well to treatment and have a good prognosis. CI - © The Author(s), 2023. FAU - Ge, Yongpeng AU - Ge Y AUID- ORCID: 0000-0003-3674-9307 AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China. FAU - Yang, Hongxia AU - Yang H AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China. AD - Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China. FAU - Jiang, Wei AU - Jiang W AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China. FAU - Tian, Xiaolan AU - Tian X AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China. FAU - Lu, Xin AU - Lu X AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China. FAU - Wang, Guochun AU - Wang G AD - Department of Rheumatology, The Key Laboratory of Myositis, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, Beijing 100029, China. AD - Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China. LA - eng PT - Journal Article DEP - 20230714 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC10350785 OTO - NOTNLM OT - anti-U1RNP antibody OT - anti-hydroxy-3-methylglutaryl-CoA reductase antibody OT - anti-signal recognition particle antibody OT - immune-mediated necrotizing myopathy OT - myositis COIS- The authors declare that there is no conflict of interest. EDAT- 2023/07/19 06:43 MHDA- 2023/07/19 06:44 PMCR- 2023/07/14 CRDT- 2023/07/19 04:03 PHST- 2023/01/21 00:00 [received] PHST- 2023/05/24 00:00 [accepted] PHST- 2023/07/19 06:43 [pubmed] PHST- 2023/07/19 06:44 [medline] PHST- 2023/07/19 04:03 [entrez] PHST- 2023/07/14 00:00 [pmc-release] AID - 10.1177_1759720X231181336 [pii] AID - 10.1177/1759720X231181336 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2023 Jul 14;15:1759720X231181336. doi: 10.1177/1759720X231181336. eCollection 2023. PMID- 40725689 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250801 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 14 DP - 2025 Jul 15 TI - Rheumatologists' Adherence to EULAR Recommendations for Systemic Sclerosis Treatment: Experience of a Single Center in Serbia. LID - 10.3390/jcm14144994 [doi] LID - 4994 AB - Background: The European League Against Rheumatism (EULAR), in collaboration with the European Scleroderma Trial and Research group (EUSTAR), published the first set of treatment recommendations for systemic sclerosis (SSc) in 2009, with subsequent updates in 2016 and 2023. Objectives: This study aimed to evaluate how rheumatologists' clinical approaches to the treatment of SSc evolved following the 2016 update of the clinical management guidelines. Methods: Medication use for SSc was analyzed in a cohort of 378 patients. The patients were stratified based on enrollment before (233 patients) and after (145 patients) the guideline update, and medication usage was compared between the two groups. Results: Although all patients presented with Raynaud's phenomenon (RP), only 35% received calcium channel blockers. Medications such as iloprost, phosphodiesterase type 5 (PDE-5) inhibitors, fluoxetine, and bosentan, recommended for the treatment of RP and digital ulcers, were not approved for SSc by the Republic Health Insurance Fund. Treatment for pulmonary arterial hypertension (PAH) was administered to only 16 patients (4.2%), including 2 who received bosentan, 10 who received PDE-5 inhibitors, and 4 who were treated with riociguat. The use of PDE-5 inhibitors increased following the 2016 update of the guidelines. Cyclophosphamide was consistently prescribed for interstitial lung disease (ILD), with an increased frequency observed after the guideline update. No significant differences were observed in the use of methotrexate for skin involvement, ACE inhibitors for scleroderma renal crisis, or antibiotics for gastrointestinal symptoms. Proton pump inhibitors (PPIs) were prescribed to 87.3% of patients with gastrointestinal involvement, with an increase in use of both PPIs and prokinetic agents following the guideline update. Conclusions: Rheumatologists' adherence to the EULAR/EUSTAR guidelines varied considerably, with 25% to 100% of eligible patients receiving the recommended treatments. Concordance improved in the management of PAH, ILD, and gastrointestinal involvement after the 2016 guideline update. FAU - Pavlov-Dolijanovic, Slavica AU - Pavlov-Dolijanovic S AUID- ORCID: 0009-0005-5683-2193 AD - Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia. FAU - Jeremic, Ivan AU - Jeremic I AUID- ORCID: 0000-0001-8269-8526 AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Bogojevic, Milan AU - Bogojevic M AUID- ORCID: 0000-0003-1037-1342 AD - Clinical Center of Montenegro, Department of Rheumatology, 81110 Podgorica, Montenegro. FAU - Velickovic, Zoran AU - Velickovic Z AUID- ORCID: 0000-0002-0590-8590 AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Zlatkovic-Svenda, Mirjana AU - Zlatkovic-Svenda M AUID- ORCID: 0000-0002-7123-140X AD - Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia. AD - Faculty of Medicine Foca, University of East Sarajevo,73300 Foca, Bosnia and Herzegovina. FAU - Kojic, Tijana AU - Kojic T AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Janjic, Sasa AU - Janjic S AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Dimic, Tatjana AU - Dimic T AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Stojic, Biljana AU - Stojic B AUID- ORCID: 0009-0001-9885-1772 AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Markovic, Ana AU - Markovic A AUID- ORCID: 0009-0004-9828-1839 AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Perunicic, Andjela AU - Perunicic A AUID- ORCID: 0009-0009-4645-6074 AD - Institute of Rheumatology, 11000 Belgrade, Serbia. FAU - Djokovic, Aleksandra AU - Djokovic A AUID- ORCID: 0000-0002-6094-7306 AD - Faculty of Medicine, University Hospital Medical Center Bezanijska kosa, Department of Cardiology, University of Belgrade, 11000 Belgrade, Serbia. FAU - Petrovic, Jelena AU - Petrovic J AUID- ORCID: 0000-0002-3322-4831 AD - Clinic for Cardiology, University Clinical Center of Serbia, 11000 Belgrade, Serbia. FAU - Baljosevic, Nevena AU - Baljosevic N AUID- ORCID: 0009-0005-5571-6211 AD - Emergency Center, University Clinical Center of Serbia, 11000 Belgrade, Serbia. FAU - Jankovic, Aleksandar AU - Jankovic A AUID- ORCID: 0000-0002-7931-973X AD - Faculty of Medicine, University Medical Center Zvezdara, Nephrology Department, University of Belgrade, 11000 Belgrade, Serbia. FAU - Omcikus, Maja AU - Omcikus M AUID- ORCID: 0000-0002-6489-5187 AD - Faculty of Medicine, University Clinical Center of Serbia, Clinic for Pulmonary Diseases, University of Belgrade, 11000 Belgrade, Serbia. FAU - Supic, Zorica Terzic AU - Supic ZT AD - Faculty of Medicine, Institute of Social Medicine, University of Belgrade, 11000 Belgrade, Serbia. FAU - Milosavljevic, Natasa AU - Milosavljevic N AUID- ORCID: 0000-0003-4056-089X AD - Department of Mathematics and Physics, Faculty of Agriculture, University of Belgrade, 11000 Belgrade, Serbia. FAU - Radunovic, Goran AU - Radunovic G AUID- ORCID: 0000-0002-4449-8969 AD - Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia. LA - eng PT - Journal Article DEP - 20250715 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC12295422 OTO - NOTNLM OT - EULAR/EUSTAR OT - adherence to guidelines OT - recommendations OT - systemic sclerosis treatment COIS- All authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2025/07/29 06:29 MHDA- 2025/07/29 06:30 PMCR- 2025/07/15 CRDT- 2025/07/29 01:17 PHST- 2025/05/11 00:00 [received] PHST- 2025/06/09 00:00 [revised] PHST- 2025/07/11 00:00 [accepted] PHST- 2025/07/29 06:30 [medline] PHST- 2025/07/29 06:29 [pubmed] PHST- 2025/07/29 01:17 [entrez] PHST- 2025/07/15 00:00 [pmc-release] AID - jcm14144994 [pii] AID - jcm-14-04994 [pii] AID - 10.3390/jcm14144994 [doi] PST - epublish SO - J Clin Med. 2025 Jul 15;14(14):4994. doi: 10.3390/jcm14144994. PMID- 36581379 OWN - NLM STAT- MEDLINE DCOM- 20230102 LR - 20230105 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 9 IP - 1 DP - 2022 Dec TI - Autoantibodies associated with systemic sclerosis in three autoimmune diseases imprinted by type I interferon gene dysregulation: a comparison across SLE, primary Sjögren's syndrome and systemic sclerosis. LID - 10.1136/lupus-2022-000732 [doi] LID - e000732 AB - OBJECTIVE: SLE, primary Sjögren's syndrome (pSS) and systemic sclerosis (SSc) are heterogeneous autoimmune diseases with a dysregulated type I interferon (IFN) system. The diseases often show overlapping clinical manifestations, which may result in diagnostic challenges. We asked to which extent SSc-associated autoantibodies are present in SLE and pSS, and whether these link to serum IFN-α, clinical phenotypes and sex. Samples with clinical data from patients with SSc and healthy blood donors (HBDs) served as controls. Finally, the diagnostic performance of SSc-associated autoantibodies was evaluated. METHODS: Samples from well-characterised subjects with SLE (n=510), pSS (n=116), SSc (n=57) and HBDs (n=236) were analysed using a commercially available immunoassay (EuroLine Systemic Sclerosis Profile (IgG)). IFN-α was quantified by ELISA. Self-reported data on Raynaud's phenomenon (RP) were available. RESULTS: With exceptions for anti-Ro52/SSA and anti-Th/To, SSc-associated autoantibodies were more frequent in SSc than in SLE, pSS and HBDs regardless of sex. IFN-α levels correlated with the number of positive SSc-associated autoantibodies (r=0.29, p<0.0001) and associated with Ro52/SSA positivity (p<0.0001). By using data from SLE, SSc and HBDs, RP was significantly associated with topoisomerase I, centromere protein (CENP)-B, RNA polymerase III 11 kDa, RNA polymerase III 155 kDa and PM-Scl100 whereas Ro52/SSA associated inversely with RP. In SLE, CENP-A was associated with immunological disorder, CENP-B with serositis and Ku with lupus nephritis. By combining analysis of ANA (immunofluorescence) with SSc-associated autoantibodies, the diagnostic sensitivity reached 98% and the specificity 33%. CONCLUSIONS: The 13 specificities included in the EuroLine immunoassay are commonly detected in SSc, but they are also frequent among individuals with other diseases imprinted by type I IFNs. These findings are valuable when interpreting serological data on patients with suspected SSc, especially as patients may present with disease manifestations overlapping different rheumatological diseases. In SLE, we observed associations between manifestations and SSc-associated autoantibodies which have not previously been reported. CI - © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Andraos, Rama AU - Andraos R AUID- ORCID: 0000-0002-5433-3885 AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Rheumatology, Linköping University, Linköping, Sweden. FAU - Ahmad, Awais AU - Ahmad A AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Clinical Immunology, Linköping University, Linköping, Sweden. FAU - Eriksson, Per AU - Eriksson P AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Rheumatology, Linköping University, Linköping, Sweden. FAU - Dahlström, Örjan AU - Dahlström Ö AD - Department of Behavioural Sciences and Learning, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden. FAU - Wirestam, Lina AU - Wirestam L AUID- ORCID: 0000-0003-3687-8344 AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Rheumatology, Linköping University, Linköping, Sweden. FAU - Dahle, Charlotte AU - Dahle C AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Clinical Immunology, Linköping University, Linköping, Sweden. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Clinical Sciences, Rheumatology, Lund University, Skåne University Hospital, Lund, Sweden. FAU - Bengtsson, Anders A AU - Bengtsson AA AD - Department of Clinical Sciences, Rheumatology, Lund University, Skåne University Hospital, Lund, Sweden. FAU - Jönsen, Andreas AU - Jönsen A AD - Department of Clinical Sciences, Rheumatology, Lund University, Skåne University Hospital, Lund, Sweden. FAU - Andréasson, Kristofer AU - Andréasson K AD - Department of Clinical Sciences, Rheumatology, Lund University, Skåne University Hospital, Lund, Sweden. FAU - Sjöwall, Christopher AU - Sjöwall C AUID- ORCID: 0000-0003-0900-2048 AD - Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Rheumatology, Linköping University, Linköping, Sweden Christopher.sjowall@liu.se. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 RN - 0 (Autoantibodies) RN - 0 (Interferon Type I) RN - EC 2.7.7.6 (RNA Polymerase III) SB - IM MH - Humans MH - Autoantibodies MH - *Sjogren's Syndrome/complications/diagnosis MH - *Interferon Type I MH - RNA Polymerase III MH - *Lupus Erythematosus, Systemic/complications/diagnosis MH - *Scleroderma, Systemic/complications/diagnosis PMC - PMC9806068 OTO - NOTNLM OT - Autoantibodies OT - Interferon Type I OT - Lupus Erythematosus, Systemic OT - Scleroderma, Systemic OT - Sjogren's Syndrome COIS- Competing interests: RH is employed by Boehringer Ingelheim. The other authors declare no potential conflicts of interest with respect to the research, authorship and/or publication of this article. EDAT- 2022/12/30 06:00 MHDA- 2023/01/03 06:00 PMCR- 2022/12/29 CRDT- 2022/12/29 20:52 PHST- 2022/05/12 00:00 [received] PHST- 2022/12/22 00:00 [accepted] PHST- 2022/12/29 20:52 [entrez] PHST- 2022/12/30 06:00 [pubmed] PHST- 2023/01/03 06:00 [medline] PHST- 2022/12/29 00:00 [pmc-release] AID - 9/1/e000732 [pii] AID - lupus-2022-000732 [pii] AID - 10.1136/lupus-2022-000732 [doi] PST - ppublish SO - Lupus Sci Med. 2022 Dec;9(1):e000732. doi: 10.1136/lupus-2022-000732. PMID- 16616154 OWN - NLM STAT- MEDLINE DCOM- 20060927 LR - 20081121 IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 35 IP - 5 DP - 2006 Apr TI - Atypical autoantibodies in patients with primary Sjögren syndrome: clinical characteristics and follow-up of 82 cases. PG - 312-21 AB - OBJECTIVES: To analyze the clinical characteristics, follow-up, and fulfillment of classification criteria for other systemic autoimmune diseases (SAD) in patients with primary Sjögren syndrome (SS) and atypical autoantibodies. METHODS: We studied 402 patients diagnosed with primary SS seen consecutively in our Department since 1994. We considered anti-DNA, anti-Sm, anti-RNP, anti-topoisomerase1/Scl70, anticentromere (ACA), anti-Jo1, anti-neutrophil cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aPL), and lupus anticoagulant as atypical autoantibodies. The patients were prospectively followed after inclusion into the protocol, focusing on the development of features that might lead to the fulfillment of classification criteria for additional SAD. As a control group, we selected an age-sex-matched subset of patients with primary SS without atypical autoantibodies. RESULTS: Eighty-two (20%) patients showed atypical autoantibodies (36 had aPL, 21 anti-DNA, 13 ANCA, 10 anti-RNP, 8 ACA, 6 anti-Sm, 2 anti-Scl70, and 1 anti-Jo-1 antibodies). There were 77 (94%) women and 5 (6%) men, with a mean age of 57 years. Patients with atypical autoantibodies had no statistical differences in the prevalence of the main sicca features, extraglandular manifestations (except for a higher prevalence of Raynaud's phenomenon, 28% versus 7%, P=0.001), immunological markers, and in the fulfillment of the 2002 classification criteria, compared with the control group. After a follow-up of 534 patient-years, 13 (16%) of the 82 patients with atypical autoantibodies developed an additional SAD (systemic lupus erythematosus in 5 cases, antiphospholipid syndrome in 4, limited scleroderma in 3, and microscopic polyangiitis in 1) compared with none in the control group (P<0.001). CONCLUSIONS: This study shows an immunological overlap (defined by the presence of autoantibodies considered typical of other SAD) in 20% of our patients with primary SS. However, the clinical significance of these atypical autoantibodies varies widely depending on the autoantibodies detected, with a broad spectrum of prevalence and clinical patterns of disease expression, and a specific predilection for association with some SAD in preference to others. FAU - Ramos-Casals, Manuel AU - Ramos-Casals M AD - Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), School of Medicine, University of Barcelona, Barcelona, Spain. mramos@clinic.ub.es FAU - Nardi, Norma AU - Nardi N FAU - Brito-Zerón, Pilar AU - Brito-Zerón P FAU - Aguiló, Sira AU - Aguiló S FAU - Gil, Victor AU - Gil V FAU - Delgado, German AU - Delgado G FAU - Bové, Albert AU - Bové A FAU - Font, Josep AU - Font J LA - eng PT - Journal Article PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Anticardiolipin) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantigens) RN - 0 (Lupus Coagulation Inhibitor) RN - 0 (Ribonucleoproteins, Small Nuclear) RN - 0 (snRNP Core Proteins) RN - 9007-49-2 (DNA) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Anticardiolipin/immunology MH - Antibodies, Antineutrophil Cytoplasmic/*immunology MH - Antibodies, Antinuclear/*immunology MH - Autoantigens/immunology MH - Centromere/immunology MH - DNA/immunology MH - DNA Topoisomerases, Type I/immunology MH - Female MH - Follow-Up Studies MH - Histidine-tRNA Ligase/immunology MH - Humans MH - Lupus Coagulation Inhibitor/immunology MH - Male MH - Middle Aged MH - Ribonucleoproteins, Small Nuclear/immunology MH - Seroepidemiologic Studies MH - Sjogren's Syndrome/epidemiology/*immunology MH - snRNP Core Proteins EDAT- 2006/04/18 09:00 MHDA- 2006/09/28 09:00 CRDT- 2006/04/18 09:00 PHST- 2006/04/18 09:00 [pubmed] PHST- 2006/09/28 09:00 [medline] PHST- 2006/04/18 09:00 [entrez] AID - S0049-0172(05)00243-X [pii] AID - 10.1016/j.semarthrit.2005.12.004 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2006 Apr;35(5):312-21. doi: 10.1016/j.semarthrit.2005.12.004. PMID- 30508197 OWN - NLM STAT- MEDLINE DCOM- 20200109 LR - 20200109 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 58 IP - 3 DP - 2019 Mar 1 TI - Increased expression of the TNF superfamily member LIGHT/TNFSF14 and its receptors (HVEM and LTßR) in patients with systemic sclerosis. PG - 502-510 LID - 10.1093/rheumatology/key348 [doi] AB - OBJECTIVES: This study aimed to assess the potential role of the TNF superfamily member lymphocyte T-related inducible ligand that competes for glycoprotein D binding to herpesvirus entry mediator on T cells (LIGHT) in SSc through evaluation of: skin expression of LIGHT and its receptors, herpesvirus entry mediator and lymphotoxin ß-related receptor, and serum concentration of LIGHT in SSc patients. METHODS: Expression of LIGHT and its receptors was investigated by immunohistochemistry and evaluated semi-quantitatively in skin biopsies from 19 SSc patients and 9 healthy controls. Serum levels of LIGHT were measured using ELISA in 329 patients with SSc and 50 control subjects. RESULTS: Expression of LIGHT and both receptors was higher in SSc patients compared with controls (P < 0.05 for all comparisons). Patients with early SSc (⩽ 3 years from the first non-Raynaud's phenomenon symptom) showed higher expression of LIGHT and herpesvirus entry mediator compared with patients with longer disease duration (P < 0.05 for both comparisons). The mean serum concentration of LIGHT was significantly higher in SSc patients compared with the controls (P < 0.05). High serum concentration of LIGHT was associated with male sex, presence of digital ulcers, muscle involvement (defined by elevated serum creatine kinase levels), steroid treatment and lack of ACA. However, in multivariate regression analysis only presence of digital ulcers and creatine kinase elevation were independently associated with serum concentration of LIGHT. CONCLUSION: These data provide the first evidence of overexpression of LIGHT and its receptors in SSc and suggest that the LIGHT axis might contribute to the pathogenesis of SSc. Increased serum concentrations of LIGHT seem to reflect vascular injury in SSc. CI - © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Gindzienska-Sieskiewicz, Ewa AU - Gindzienska-Sieskiewicz E AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Reszec, Joanna AU - Reszec J AD - Department of Medical Pathomorphology, Medical University of Bialystok, Bialystok, Poland. FAU - Jordan, Suzana AU - Jordan S AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Bielecki, Pawel AU - Bielecki P AD - Department of Otolaryngology, Medical University of Bialystok, Bialystok, Poland. FAU - Sieskiewicz, Andrzej AU - Sieskiewicz A AD - Department of Otolaryngology, Medical University of Bialystok, Bialystok, Poland. FAU - Sulik, Agnieszka AU - Sulik A AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Lukasik, Malgorzata AU - Lukasik M AD - Department of Medical Pathomorphology, Medical University of Bialystok, Bialystok, Poland. FAU - Bielecki, Marek AU - Bielecki M AD - Department of Orthopedics and Traumatology, Medical University of Bialystok, Bialystok, Poland. FAU - Kowal, Krzysztof AU - Kowal K AD - Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. AD - Department of Experimental Allergology and Immunology, Medical University of Bialystok, Bialystok, Poland. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (LTBR protein, human) RN - 0 (Lymphotoxin beta Receptor) RN - 0 (Receptors, Tumor Necrosis Factor, Member 14) RN - 0 (Tumor Necrosis Factor Ligand Superfamily Member 14) SB - IM MH - Adult MH - Female MH - Humans MH - Lymphotoxin beta Receptor/genetics/*metabolism MH - Male MH - Middle Aged MH - Receptors, Tumor Necrosis Factor, Member 14/genetics/*metabolism MH - Scleroderma, Systemic/genetics/*metabolism/pathology MH - Skin/*metabolism/pathology MH - Tumor Necrosis Factor Ligand Superfamily Member 14/blood/genetics/*metabolism OTO - NOTNLM OT - TNFRSF14 OT - pathogenesis OT - systemic sclerosis EDAT- 2018/12/07 06:00 MHDA- 2020/01/10 06:00 CRDT- 2018/12/04 06:00 PHST- 2018/03/27 00:00 [received] PHST- 2018/10/18 00:00 [accepted] PHST- 2018/12/07 06:00 [pubmed] PHST- 2020/01/10 06:00 [medline] PHST- 2018/12/04 06:00 [entrez] AID - 5224786 [pii] AID - 10.1093/rheumatology/key348 [doi] PST - ppublish SO - Rheumatology (Oxford). 2019 Mar 1;58(3):502-510. doi: 10.1093/rheumatology/key348. PMID- 29427827 OWN - NLM STAT- MEDLINE DCOM- 20180529 LR - 20250623 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 17 IP - 4 DP - 2018 Apr TI - Nailfold capillaroscopy in systemic lupus erythematosus: A systematic review and critical appraisal. PG - 344-352 LID - S1568-9972(18)30031-4 [pii] LID - 10.1016/j.autrev.2017.11.025 [doi] AB - Nailfold capillaroscopy is an easy, non-invasive technique to assess microvascular involvement in rheumatic diseases. Multiple studies describe capillaroscopic changes in systemic lupus erythematosus (SLE), including a wide range of non-specific findings. On behalf of the European League Against Rheumatism (EULAR) study group on microcirculation in rheumatic diseases, a systematic review was done to obtain all original research studies (in English) in which SLE patients had capillaroscopy. Forty such studies are identified. This article firstly provides a résumé of the results of these studies according to capillaroscopic parameters (density, dimensions, morphology, haemorrhages), semi-quantitative assessment and qualitative assessment of capillaroscopy in SLE patients. Secondly, the correlations between capillaroscopic parameters in SLE patients and clinical and laboratory parameters (including auto-immune parameters) are outlined. The following capillaroscopic parameters are found to be significantly more prevalent in SLE patients compared to healthy controls: tortuous capillaries, abnormal morphology and haemorrhages. Hairpin-shaped capillaries are significantly less prevalent than in healthy persons. The semi-quantitatively determined nailfold capillaroscopic score (NFC score) in SLE patients is also higher than in healthy controls. Several correlations between clinical and laboratory parameters and capillaroscopic parameters are identified in the review. Disease activity is correlated with NFC score in seven studies, with abnormal morphology (i.e. "meandering") in one study and with haemorrhages in one study. Frequent attacks of Raynaud's phenomenon (RP) and gangrene are significantly correlated with dilated capillaries. In two studies a possible correlation between anti-SSA antibodies and lower density of capillaries is withheld. About other immune parameters conflicting results are found. In one study a significant negative correlation is found between 24-hour proteinuria and abnormal morphology (i.e. "meandering"). For the first time, an overview of the nailfold capillaroscopic changes that have been described in SLE and their correlations with clinical and laboratory findings is given. Further large-scale research on the identification of capillaroscopic changes in SLE and their correlations with standardised clinical and laboratory parameters, is ongoing at the EULAR study group on microcirculation in rheumatic diseases. CI - Copyright © 2018 Elsevier B.V. All rights reserved. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Via Balbi 5, Genoa, Italy. Electronic address: mcutolo@unige.it. FAU - Melsens, Karin AU - Melsens K AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: karin.melsens@ugent.be. FAU - Wijnant, Sara AU - Wijnant S AD - Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: sara.wijnant@ugent.be. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Rheumatology, ASST Gaetano Pini, Department of Clinical Sciences and Community Health, University of Milan, Piazza Cardinal Andrea Ferrari 1, Milan, Italy. Electronic address: francesca.ingegnoli@unimi.it. FAU - Thevissen, Kristof AU - Thevissen K AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium. Electronic address: kristof.thevissen@uzgent.be. FAU - De Keyser, Filip AU - De Keyser F AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: filip.dekeyser@ugent.be. FAU - Decuman, Saskia AU - Decuman S AD - Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: Saskia.Decuman@riziv.fgov.be. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Rheumatology and Clinical Immunology, University of Giessen/Kerckhoff-Klinik, Benekestraße 2-8, 61231 Bad Nauheim, Germany. Electronic address: U.Mueller-Ladner@kerckhoff-klinik.de. FAU - Piette, Yves AU - Piette Y AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium. Electronic address: yves.piette@ugent.be. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Internal Medicine and Clinical Specialities, Sapienza University, Piazzale Aldo Moro 5, Rome, Italy. Electronic address: valeria.riccieri@uniroma1.it. FAU - Ughi, Nicola AU - Ughi N AD - Division of Rheumatology, ASST Gaetano Pini, Department of Clinical Sciences and Community Health, University of Milan, Piazza Cardinal Andrea Ferrari 1, Milan, Italy. FAU - Vandecasteele, Els AU - Vandecasteele E AD - Department of Cardiology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: els.vandecasteele@ugent.be. FAU - Vanhaecke, Amber AU - Vanhaecke A AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: amber.vanhaecke@ugent.be. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent, Belgium; Department of Internal Medicine, Ghent University, C. Heymanslaan 10, Ghent, Belgium. Electronic address: vanessa.smith@ugent.be. LA - eng PT - Journal Article PT - Systematic Review DEP - 20180208 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 SB - IM MH - Capillaries MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/*immunology MH - Male MH - Microscopic Angioscopy/*methods MH - Nails/*blood supply OTO - NOTNLM OT - Capillaroscopy OT - EULAR study group on microcirculation in rheumatic diseases OT - Microcirculation OT - Systematic literature review OT - Systemic lupus erythematosus OT - Vasculopathy EDAT- 2018/02/11 06:00 MHDA- 2018/05/31 06:00 CRDT- 2018/02/11 06:00 PHST- 2017/10/27 00:00 [received] PHST- 2017/11/02 00:00 [accepted] PHST- 2018/02/11 06:00 [pubmed] PHST- 2018/05/31 06:00 [medline] PHST- 2018/02/11 06:00 [entrez] AID - S1568-9972(18)30031-4 [pii] AID - 10.1016/j.autrev.2017.11.025 [doi] PST - ppublish SO - Autoimmun Rev. 2018 Apr;17(4):344-352. doi: 10.1016/j.autrev.2017.11.025. Epub 2018 Feb 8. PMID- 28920529 OWN - NLM STAT- MEDLINE DCOM- 20171113 LR - 20171113 IS - 1938-9116 (Electronic) IS - 1538-5744 (Linking) VI - 51 IP - 8 DP - 2017 Nov TI - Surgical Interventions for Organ and Limb Ischemia Associated With Primary and Secondary Antiphospholipid Antibody Syndrome With Arterial Involvement. PG - 550-554 LID - 10.1177/1538574417729273 [doi] AB - OBJECTIVE: The association of antiphospholipid antibody syndrome (APS) and hypercoagulability is well known. Arterial compromise leading to ischemia of organs and/or limbs in patients with APS is uncommon, frequently unrecognized, and rarely described. We evaluated our institutional experience. METHODS: Retrospective review was conducted. From August 2007 to September 2016, 807 patients with diagnosis of APS were managed in our Institution. Patients with primary and secondary APS who required interventions were examined. Demographics, comorbidities, manifestations, procedures, complications, and other factors affecting outcomes were recorded. RESULTS: Fourteen patients (mean age 35 years old, standard deviation ±14) were evaluated and treated by our service. Six (43%) of them had primary APS and 8 (57%) had secondary APS; 11 (79%) were female. Two (14%) experienced distal aorta and iliac arteries involvement, 3 (21%) visceral vessels disease, 2 (14%) in upper and 7 (50%) in the lower extremity vasculatures. Thirteen (93%) patients underwent direct open revascularization and 1 with hand ischemia (Raynaud disease) underwent sympathectomy. During the mean follow-up period of 48 months, reinterventions included a revision of the proximal anastomosis of an aortobifemoral bypass graft, 1 (7%) abdominal exploration for bleeding, 1 (7%) graft thrombectomy, and 4 (29%) amputations (2 below the knee, 1 above the knee, and 1 transmetatarsal). One (7%) death occurred secondary to sepsis in a patient who had acute mesenteric ischemia. Significant differences in clinical manifestations and outcomes were not observed among patients with primary and secondary APS. All patients remained on systemic anticoagulation. CONCLUSION: APS is a prothrombotic disorder that may lead to arterial involvement with less frequency than the venous circulation but has significant morbidity and limb loss rate. Arterial reconstruction seems feasible in an attempt to salvage organs and limbs; however, research is necessary to establish the optimal anticoagulation regime and long-term management following surgical interventions. FAU - Hinojosa, Carlos A AU - Hinojosa CA AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - Anaya-Ayala, Javier E AU - Anaya-Ayala JE AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - Bermudez-Serrato, Karla AU - Bermudez-Serrato K AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - García-Alva, Ramón AU - García-Alva R AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - Laparra-Escareno, Hugo AU - Laparra-Escareno H AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - Torres-Machorro, Adriana AU - Torres-Machorro A AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. FAU - Lizola, Rene AU - Lizola R AD - 1 Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico. LA - eng PT - Journal Article DEP - 20170918 PL - United States TA - Vasc Endovascular Surg JT - Vascular and endovascular surgery JID - 101136421 RN - 0 (Anticoagulants) SB - IM MH - Adult MH - Anticoagulants/therapeutic use MH - Antiphospholipid Syndrome/*complications/diagnosis/drug therapy/mortality MH - Aortography/methods MH - Computed Tomography Angiography MH - Female MH - Humans MH - Ischemia/diagnostic imaging/etiology/mortality/*surgery MH - Male MH - Middle Aged MH - Peripheral Arterial Disease/diagnostic imaging/etiology/mortality/*surgery MH - Postoperative Complications/mortality/surgery MH - Reoperation MH - Retrospective Studies MH - Risk Factors MH - Thrombophilia/diagnosis/drug therapy/*etiology/mortality MH - Time Factors MH - Treatment Outcome MH - *Vascular Surgical Procedures/adverse effects/mortality MH - Young Adult OTO - NOTNLM OT - antiphospholipid antibody syndrome OT - clinical outcomes OT - interventions OT - ischemia EDAT- 2017/09/19 06:00 MHDA- 2017/11/14 06:00 CRDT- 2017/09/19 06:00 PHST- 2017/09/19 06:00 [pubmed] PHST- 2017/11/14 06:00 [medline] PHST- 2017/09/19 06:00 [entrez] AID - 10.1177/1538574417729273 [doi] PST - ppublish SO - Vasc Endovascular Surg. 2017 Nov;51(8):550-554. doi: 10.1177/1538574417729273. Epub 2017 Sep 18. PMID- 30567600 OWN - NLM STAT- MEDLINE DCOM- 20191009 LR - 20260518 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 20 IP - 1 DP - 2018 Dec 19 TI - Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort. PG - 280 LID - 10.1186/s13075-018-1776-8 [doi] LID - 280 AB - BACKGROUND: The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort. METHODS: All adult patients in the RELESSER-TRANS (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology [SER], cross-sectional phase) registry were retrospectively investigated for the presence of primary pleuropulmonary manifestations. RESULTS: In total 3215 patients were included. At least one pleuropulmonary manifestation was present in 31% of patients. The most common manifestation was pleural disease (21%), followed by lupus pneumonitis (3.6%), pulmonary thromboembolism (2.9%), primary pulmonary hypertension (2.4%), diffuse interstitial lung disease (2%), alveolar hemorrhage (0.8%), and shrinking lung syndrome (0.8%). In the multivariable analysis, the variables associated with the development of pleuropulmonary manifestation were older age at disease onset (odds ratio (OR) 1.03, 95% confidence interval (CI) 1.02-1.04), higher SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores (OR 1.03, 95% CI 1.00-1.07), the presence of Raynaud's phenomenon (OR 1.41, 95% CI 1.09-1.84), secondary antiphospholipid syndrome (OR 2.20, 95% CI 1.63-2.97), and the previous or concomitant occurrence of severe lupus nephritis, (OR 1.48, 95% CI 1.12-1.95) neuropsychiatric manifestations (OR 1.49, 95% CI 1.11-2.02), non-ischemic cardiac disease (OR 2.91, 95% CI 1.90-4.15), vasculitis (OR 1.81, 95% CI 1.25-2.62), hematological manifestations (OR 1.31, 95% CI 1.00-1.71), and gastrointestinal manifestations, excluding hepatitis (OR 2.05, 95% CI 1.14-3.66). Anti-RNP positivity had a clear tendency to significance (OR 1.32, 95% CI 1.00-1.75; P = 0.054). The development of pleuropulmonary manifestations independently contributes to a diminished survival (hazard ratio of 3.13). However, not all complications will influence the prognosis in the same way. Whereas the occurrence of pleural disease or pulmonary thromboembolism has a minimal impact on the survival of these patients, the remaining manifestations have a major impact on mortality. CONCLUSION: Except for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients. FAU - Narváez, Javier AU - Narváez J AUID- ORCID: 0000-0002-1614-8064 AD - Department of Rheumatology (Planta 10-2), Servicio de Reumatología, Hospital Universitario de Bellvitge, Feixa Llarga, s/n, Hospitalet de Llobregat, 08907, Barcelona, Spain. fjnarvaez@bellvitgehospital.cat. FAU - Borrell, Helena AU - Borrell H AD - Department of Rheumatology (Planta 10-2), Servicio de Reumatología, Hospital Universitario de Bellvitge, Feixa Llarga, s/n, Hospitalet de Llobregat, 08907, Barcelona, Spain. FAU - Sánchez-Alonso, Fernando AU - Sánchez-Alonso F AD - Unidad de Investigación, Sociedad Española de Reumatología, Madrid, Spain. FAU - Rúa-Figueroa, Iñigo AU - Rúa-Figueroa I AD - Hospital Universitario Doctor Negrín, Las Palmas de, Gran Canaria, Spain. FAU - López-Longo, Francisco Javier AU - López-Longo FJ AD - Hospital Universitario Gregorio Marañón, Madrid, Spain. FAU - Galindo-Izquierdo, María AU - Galindo-Izquierdo M AD - Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Calvo-Alén, Jaime AU - Calvo-Alén J AD - Hospital Universitario Araba, Vitoria, Spain. FAU - Fernández-Nebro, Antonio AU - Fernández-Nebro A AD - Hospital Universitario de Málaga, Málaga, Spain. FAU - Olivé, Alejandro AU - Olivé A AD - Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain. FAU - Andreu, José Luis AU - Andreu JL AD - Hospital Universitario Puerta de Hierro, Madrid, Spain. FAU - Martínez-Taboada, Víctor AU - Martínez-Taboada V AD - Hospital Universitario Marqués de Valdecilla, Santander, Spain. FAU - Nolla, Joan Miquel AU - Nolla JM AD - Department of Rheumatology (Planta 10-2), Servicio de Reumatología, Hospital Universitario de Bellvitge, Feixa Llarga, s/n, Hospitalet de Llobregat, 08907, Barcelona, Spain. FAU - Pego-Reigosa, José María AU - Pego-Reigosa JM AD - Complexo Hospitalario Universitario de Vigo, Vigo, Spain. CN - RELESSER Study Group LA - eng PT - Journal Article DEP - 20181219 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Comorbidity MH - Cross-Sectional Studies MH - Female MH - Humans MH - Kaplan-Meier Estimate MH - Lung Diseases/diagnosis/*epidemiology MH - Lupus Erythematosus, Systemic/*epidemiology MH - Lupus Nephritis/epidemiology MH - Male MH - Middle Aged MH - Prevalence MH - Proportional Hazards Models MH - Registries/*statistics & numerical data MH - Retrospective Studies MH - Spain/epidemiology MH - Young Adult PMC - PMC6299951 OTO - NOTNLM OT - Pleuropulmonary involvement OT - Systemic lupus erythematosus COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: The study protocol was approved by the institutional ethics committee of the Hospital Universitario Doctor Negrín (Las Palmas de Gran Canaria) and subsequently by the local ethics committee of all participating centers. Informed consent was obtained from the patients, and their clinical records and information were anonymized prior to analysis. Confidential information of the patients was protected in accordance with national norms. This study was conducted in accordance with the principles of the Declaration of Helsinki and the International Conference for Harmonization. CONSENT FOR PUBLICATION: Informed consent was obtained from the patients, and their clinical records and information were anonymized prior to analysis. Confidential information of the patients was protected in accordance with national norms. COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. FIR - Vela, Paloma IR - Vela P FIR - Vals, Elia IR - Vals E FIR - Cobo-Ibáñez, Tatiana IR - Cobo-Ibáñez T FIR - Bonilla, Gema IR - Bonilla G FIR - García-Villanueva, María Jesús IR - García-Villanueva MJ FIR - Diez-Álvarez, Elvira IR - Diez-Álvarez E FIR - Freire, Mercedes IR - Freire M FIR - Gantes, Marian IR - Gantes M FIR - de la Peña, Paloma García IR - de la Peña PG FIR - García-Vicuña, Rosario IR - García-Vicuña R FIR - Hernández-Beiraín, José Ángel IR - Hernández-Beiraín JÁ FIR - Horcada, Loreto IR - Horcada L FIR - Ibañez-Ruán, Jesús IR - Ibañez-Ruán J FIR - Ibañez, Mónica IR - Ibañez M FIR - Marras, Carlos IR - Marras C FIR - Marenco, José Luis IR - Marenco JL FIR - Castellví, Ivan IR - Castellví I FIR - Montilla-Morales, Carlos IR - Montilla-Morales C FIR - Moreno, Mireia IR - Moreno M FIR - Pecondon-Español, Ángela IR - Pecondon-Español Á FIR - Lucea, Esther Ruiz IR - Lucea ER FIR - Sánchez-Atrio, Ana IR - Sánchez-Atrio A FIR - Santos-Soler, Gregorio IR - Santos-Soler G FIR - Toyos, Francisco IR - Toyos F FIR - Isacelaya, Esther Uriarte IR - Isacelaya EU FIR - Vazquez-Rodríguez, Tomas IR - Vazquez-Rodríguez T EDAT- 2018/12/21 06:00 MHDA- 2019/10/11 06:00 PMCR- 2018/12/19 CRDT- 2018/12/21 06:00 PHST- 2018/08/10 00:00 [received] PHST- 2018/11/25 00:00 [accepted] PHST- 2018/12/21 06:00 [entrez] PHST- 2018/12/21 06:00 [pubmed] PHST- 2019/10/11 06:00 [medline] PHST- 2018/12/19 00:00 [pmc-release] AID - 10.1186/s13075-018-1776-8 [pii] AID - 1776 [pii] AID - 10.1186/s13075-018-1776-8 [doi] PST - epublish SO - Arthritis Res Ther. 2018 Dec 19;20(1):280. doi: 10.1186/s13075-018-1776-8. PMID- 33787070 OWN - NLM STAT- MEDLINE DCOM- 20220930 LR - 20221107 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 74 IP - 10 DP - 2022 Oct TI - Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. PG - 1575-1584 LID - 10.1002/acr.24609 [doi] AB - OBJECTIVE: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. RESULTS: At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease. CI - © 2021 American College of Rheumatology. FAU - Foeldvari, Ivan AU - Foeldvari I AUID- ORCID: 0000-0003-0659-5298 AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Klotsche, Jens AU - Klotsche J AUID- ORCID: 0000-0002-2954-5755 AD - German Rheumatism Research Center, Berlin, Germany. FAU - Kasapcopur, Ozgur AU - Kasapcopur O AUID- ORCID: 0000-0002-1125-7720 AD - Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey. FAU - Adrovic, Amra AU - Adrovic A AD - Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey. FAU - Terreri, Maria Teresa AU - Terreri MT AD - Universidade Federal de São Paulo, Sao Paulo, Brazil. FAU - Sakamoto, Ana Paula AU - Sakamoto AP AD - Universidade Federal de São Paulo, Sao Paulo, Brazil. FAU - Stanevicha, Valda AU - Stanevicha V AD - Riga Stradins University, University Children Hospital, Riga, Latvia. FAU - Sztajnbok, Flavio AU - Sztajnbok F AD - Universidade do Estado, Rio de Janeiro, Brazil. FAU - Anton, Jordi AU - Anton J AUID- ORCID: 0000-0002-8792-4219 AD - Hospital Sant Joan de Déu, Esplugues, Universitat de Barcelona, Barcelona, Spain. FAU - Feldman, Brian AU - Feldman B AD - SickKids, The Hospital for Sick Children, Toronto, Ontario, Canada. FAU - Alexeeva, Ekaterina AU - Alexeeva E AD - National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia. FAU - Katsicas, Maria AU - Katsicas M AD - Hospital de Pediatria J. P. Garrahan, Buenos Aires, Argentina. FAU - Smith, Vanessa AU - Smith V AD - Ghent University and Ghent University Hospital, Ghent, Belgium. FAU - Avcin, Tadej AU - Avcin T AD - University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia. FAU - Marrani, Edoardo AU - Marrani E AUID- ORCID: 0000-0002-0346-3268 AD - Meyer Children's Hospital, Florence, Italy. FAU - Kostik, Mikhail AU - Kostik M AD - Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia. FAU - Lehman, Thomas AU - Lehman T AD - Hospital for Special Surgery, New York, New York. FAU - Sifuentes-Giraldo, Walter-Alberto AU - Sifuentes-Giraldo WA AD - University Hospital Ramón y Cajal, Madrid, Spain. FAU - Vasquez-Canizares, Natalia AU - Vasquez-Canizares N AD - Children's Hospital at Montefiore, Bronx, New York. FAU - Appenzeller, Simone AU - Appenzeller S AUID- ORCID: 0000-0001-5075-4474 AD - School of Medical Science University of Campinas, Sao Paulo, Brazil. FAU - Janarthanan, Mahesh AU - Janarthanan M AD - Sri Ramachandra University, Chennai, India. FAU - Moll, Monika AU - Moll M AD - University Tuebingen, Tuebingen, Germany. FAU - Nemcova, Dana AU - Nemcova D AD - Charles University, Prague, Czech Republic. FAU - Patwardhan, Anjali AU - Patwardhan A AD - University of Missouri, Columbia. FAU - Santos, Maria Jose AU - Santos MJ AUID- ORCID: 0000-0002-7946-1365 AD - Hospital Garcia de Orta, Almada, Portugal. FAU - Sawhney, Sujata AU - Sawhney S AD - Sir Ganga Ram Hospital, Delhi, India. FAU - Schonenberg-Meinema, Dieneke AU - Schonenberg-Meinema D AUID- ORCID: 0000-0001-9416-6631 AD - Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands. FAU - Battagliotti, Cristina AU - Battagliotti C AD - Hospital de Niños Dr. Orlando Alassia, Santa Fe, Argentina. FAU - Berntson, Lillemor AU - Berntson L AD - Uppsala University, Uppsala, Sweden. FAU - Bica, Blanca AU - Bica B AD - Hospital Universitário Clementino Fraga Filho and Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. FAU - Brunner, Jürgen AU - Brunner J AD - Medical University Innsbruck, Innsbruck, Austria. FAU - Costa-Reis, Patricia AU - Costa-Reis P AD - Hospital de Santa Maria, Universidade de Lisboa, Lisbon, Portugal. FAU - Eleftheriou, Despina AU - Eleftheriou D AD - Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. FAU - Harel, Liora AU - Harel L AD - Schneider Children's Medical Center of Israel Sackler Faculty of Medicine, Tel Aviv University, Petah-Tikva, Israel. FAU - Horneff, Gerd AU - Horneff G AD - Asklepios Klnik Sankt Augustin, Sankt Augustin, Germany. FAU - Kaiser, Daniela AU - Kaiser D AD - Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland. FAU - Kallinich, Tilmann AU - Kallinich T AD - Charité University Medicine and German Rheumatism Research Center Berlin, Berlin, Germany. FAU - Lazarevic, Dragana AU - Lazarevic D AD - Clinical Center Nis, University of Niš, Nis, Serbia. FAU - Minden, Kirsten AU - Minden K AUID- ORCID: 0000-0003-2775-0111 AD - Charité University Medicine and German Rheumatism Research Center Berlin, Berlin, Germany. FAU - Nielsen, Susan AU - Nielsen S AD - Rigshospitalet, Copenhagen, Denmark. FAU - Nuruzzaman, Farzana AU - Nuruzzaman F AD - Stony Brook Children's Hospital, Stony Brook, New York. FAU - Uziel, Yosef AU - Uziel Y AD - Meir Medical Center, Kfar Saba, Tel Aviv University, Tel Aviv, Israel. FAU - Helmus, Nicola AU - Helmus N AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Torok, Kathryn S AU - Torok KS AUID- ORCID: 0000-0002-1662-143X AD - University of Pittsburgh, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220608 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Lung Diseases, Interstitial MH - Male MH - *Scleroderma, Diffuse/diagnosis MH - Scleroderma, Localized MH - *Scleroderma, Systemic/diagnosis/epidemiology MH - *Skin Ulcer EDAT- 2021/04/01 06:00 MHDA- 2022/10/01 06:00 CRDT- 2021/03/31 12:30 PHST- 2021/03/01 00:00 [revised] PHST- 2020/07/01 00:00 [received] PHST- 2021/03/25 00:00 [accepted] PHST- 2021/04/01 06:00 [pubmed] PHST- 2022/10/01 06:00 [medline] PHST- 2021/03/31 12:30 [entrez] AID - 10.1002/acr.24609 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584. doi: 10.1002/acr.24609. Epub 2022 Jun 8. PMID- 41383230 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251212 LR - 20251214 IS - 1664-2295 (Print) IS - 1664-2295 (Electronic) IS - 1664-2295 (Linking) VI - 16 DP - 2025 TI - SAFE-CGRP study: multicenter retrospective evaluation of the safety of CGRP pathway-targeting monoclonal antibodies in migraine with relevant comorbidities or conditions excluded from trials. PG - 1703876 LID - 10.3389/fneur.2025.1703876 [doi] LID - 1703876 AB - INTRODUCTION: Monoclonal antibodies (mAbs) targeting calcitonin gene-related peptide (CGRP) have shown efficacy in the treatment of migraine. However, certain comorbidities have been excluded from clinical trials or may pose potential risks, despite not being formal contraindications. This study aimed to assess the safety of anti-CGRP or anti-CGRP receptor mAbs in real-world patients with such conditions. METHODS: A retrospective multicenter study was conducted across 11 headache units in Spain. Patients with relevant or trial-excluded comorbidities who received at least one anti-CGRP or anti-CGRP receptor mAb were included. RESULTS: Of 2,042 evaluated patients, 353 had at least one comorbidity. The mean treatment duration was 12.7 months [standard deviation (SD) = 8 months]. A total of 53 conditions were included: 202 had autoimmune diseases, 163 presented vascular risk factors or diseases [body mass index (BMI) > 30: 15.2%, diabetes: 5.38%, stroke/transient ischemic attack (TIA): 3.08%, cardiac ischaemic disease: 1.44%], of which 23 had moderate-to-high cardiovascular risk (Framingham scale); 23 had pulmonary diseases, 71 had a history of cancer, 12 were immunosuppressed, and 16 had other conditions. In 12% of cases, disease control was suboptimal after treatment initiation, without a causal relationship to the mAb. A possible treatment-related worsening was observed in 14 cases: four with arterial hypertension worsening, seven with Raynaud's syndrome, two with arthritis flares, and one hereditary angioedema; all improved upon treatment discontinuation. No severe adverse events were reported. DISCUSSION: In this study, treatment with monoclonal antibodies acting on the CGRP pathway showed a favorable safety profile in patients with complex clinical conditions not represented in clinical trials, with no serious adverse events observed during extended follow-up. These findings support their use in real-world clinical settings, although further studies are needed to confirm their long-term safety. CI - Copyright © 2025 Sanabria-Gago, Lázaro, Heredia, Sánchez-Soblechero, Ros, Buzo, Osorio, Guerrero-Peral, Gonzalez-Martinez, Azorín, Latorre, Calle, Toledo-Alfocea, Limón, Valle, Salaices, Ávila, Carpio, Pascual, González-Quintanilla, Madera, Polanco, Rodríguez-Vico, Jaimes, García, Cuadrado and Gago-Veiga. FAU - Sanabria-Gago, Cristina AU - Sanabria-Gago C AD - Headache Unit, Department of Neurology, Hospital Universitario de la Princesa, Madrid, Spain. FAU - Lázaro, Iris Fernández AU - Lázaro IF AD - Headache Unit, Department of Neurology, Hospital Universitario de la Princesa, Madrid, Spain. FAU - Heredia, Patricia AU - Heredia P AD - Headache Unit, Department of Neurology, Hospital Universitario de la Princesa, Madrid, Spain. FAU - Sánchez-Soblechero, Antonio AU - Sánchez-Soblechero A AD - Headache Unit, Department of Neurology, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Ros, Alberto Lozano AU - Ros AL AD - Headache Unit, Department of Neurology, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Buzo, Elisa Luque AU - Buzo EL AD - Headache Unit, Department of Neurology, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Osorio, Yesica González AU - Osorio YG AD - Headache Unit, Department of Neurology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. FAU - Guerrero-Peral, Ángel AU - Guerrero-Peral Á AD - Headache Unit, Department of Neurology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. FAU - Gonzalez-Martinez, Alicia AU - Gonzalez-Martinez A AD - Departments of Neurology and Immunology, Hospital Universitario de la Princesa and Instituto de Investigación Sanitaria Princesa (IIS-Princesa), Madrid, Spain. FAU - Azorín, David García AU - Azorín DG AD - Headache Unit, Department of Neurology, Hospital Universitario Río Hortega de Valladolid, Valladolid, Spain. FAU - Latorre, Germán AU - Latorre G AD - Headache Unit, Department of Neurology, Hospital Universitario de Fuenlabrada, Fuenlabrada, Spain. FAU - Calle, Carlos AU - Calle C AD - Headache Unit, Department of Neurology, Hospital Universitario de Fuenlabrada, Fuenlabrada, Spain. FAU - Toledo-Alfocea, Daniel AU - Toledo-Alfocea D AD - Headache Unit, Department of Neurology, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Limón, Javier Casas AU - Limón JC AD - Headache Unit, Department of Neurology, Hospital Universitario Fundación Alcorcón, Alcorcón, Spain. FAU - Valle, Sarai Urtiaga AU - Valle SU AD - Headache Unit, Department of Neurology, Hospital de Torrejón, Torrejón, Spain. FAU - Salaices, Marta González AU - Salaices MG AD - Headache Unit, Department of Neurology, Hospital de Torrejón, Torrejón, Spain. FAU - Ávila, Guillermo Martín AU - Ávila GM AD - Headache Unit, Department of Neurology, Hospital Universitario de Getafe, Getafe, Spain. FAU - Carpio, Rodrigo Terrero AU - Carpio RT AD - Headache Unit, Department of Neurology, Hospital Universitario de Getafe, Getafe, Spain. FAU - Pascual, Julio AU - Pascual J AD - Headache Unit, Department of Neurology, Hospital Universitario Marqués de Valdecilla, Santander, Spain. AD - Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. AD - Headache and Other Non-Degenerative Neurological Diseases Research Group, IDIVAL, Santander, Spain. FAU - González-Quintanilla, Vicente AU - González-Quintanilla V AD - Headache Unit, Department of Neurology, Hospital Universitario Marqués de Valdecilla, Santander, Spain. AD - Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. AD - Headache and Other Non-Degenerative Neurological Diseases Research Group, IDIVAL, Santander, Spain. FAU - Madera, Jorge AU - Madera J AD - Headache Unit, Department of Neurology, Hospital Universitario Marqués de Valdecilla, Santander, Spain. AD - Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. AD - Headache and Other Non-Degenerative Neurological Diseases Research Group, IDIVAL, Santander, Spain. FAU - Polanco, Marcos AU - Polanco M AD - Headache Unit, Department of Neurology, Hospital Universitario Marqués de Valdecilla, Santander, Spain. AD - Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. AD - Headache and Other Non-Degenerative Neurological Diseases Research Group, IDIVAL, Santander, Spain. FAU - Rodríguez-Vico, Jaime AU - Rodríguez-Vico J AD - Headache Unit, Department of Neurology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain. FAU - Jaimes, Alex AU - Jaimes A AD - Headache Unit, Department of Neurology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain. FAU - García, Andrea Gómez AU - García AG AD - Headache Unit, Department of Neurology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain. FAU - Cuadrado, María-Luz AU - Cuadrado ML AD - Headache Unit, Department of Neurology, Hospital Universitario Clínico San Carlos, Madrid, Spain. AD - Complutense University of Madrid, Madrid, Spain. FAU - Gago-Veiga, Ana Beatriz AU - Gago-Veiga AB AD - Headache Unit, Department of Neurology, Hospital Universitario de la Princesa, Madrid, Spain. LA - eng PT - Journal Article DEP - 20251126 PL - Switzerland TA - Front Neurol JT - Frontiers in neurology JID - 101546899 PMC - PMC12689294 OTO - NOTNLM OT - autoimmune diseases OT - calcitonin gene-related peptide OT - comorbidity OT - migraine OT - monoclonal antibodies OT - risk factors OT - safety COIS- AS-S has received honoraria from Organon, TEVA, Lundbeck. AR has received speaker honoraria from Teva, Lundbeck, Novartis, Pfizer, Dr. Reddis and Abbvie. CC has received honoraria from TEVA, Abbvie, Lundbeck and Lilly. AG-M has received speaker honoraria from TEVA, Lilly and Altermedica. DA received honoraria for lectures/presentations from Abbvie/Allergan, Eli Lilly, Teva, Lundbeck, and Novartis. DT-A. has received speaker honoraria from TEVA, Lilly, Organon and Lundbeck. JL has received speaker honoraria from Lundbeck, Novartis, Organon and Teva. MS has received honoraria from Lundbeck. JP has participated in Symposia or Advisory Boards from Abbvie, Almirall, Organon, Pfizer and Teva. JR-V has received honoraria from Lilly, TEVA, Novartis, Allergan-Abbvie and Exeltis, as well as research support from Allergan-Abbvie. AJ has received honoraria from Lilly, TEVA, Organon, Allergan-Abbvie and Lundbeck. AG has received honoraria from Organon. Olga Pajares, Ignacio Eguilior, Lena Nystrom. M-LC has been involved as a consultant or lecturer for Novartis, Lundbeck and Teva. AG-V has received speaker honoraria and/or served as a clinical advisor for Novartis, Lilly, TEVA, Exeltis, Chiesi, Abbvie, Pfizer, Dr. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer LI declared a past co-authorship with the author DA to the handling editor. EDAT- 2025/12/12 07:20 MHDA- 2025/12/12 07:21 PMCR- 2025/11/26 CRDT- 2025/12/12 04:23 PHST- 2025/09/12 00:00 [received] PHST- 2025/10/27 00:00 [revised] PHST- 2025/11/04 00:00 [accepted] PHST- 2025/12/12 07:21 [medline] PHST- 2025/12/12 07:20 [pubmed] PHST- 2025/12/12 04:23 [entrez] PHST- 2025/11/26 00:00 [pmc-release] AID - 10.3389/fneur.2025.1703876 [doi] PST - epublish SO - Front Neurol. 2025 Nov 26;16:1703876. doi: 10.3389/fneur.2025.1703876. eCollection 2025. PMID- 37708984 OWN - NLM STAT- MEDLINE DCOM- 20231103 LR - 20231103 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 22 IP - 11 DP - 2023 Nov TI - Immunoglobulins in systemic sclerosis management. A large multicenter experience. PG - 103441 LID - S1568-9972(23)00175-1 [pii] LID - 10.1016/j.autrev.2023.103441 [doi] AB - OBJECTIVE: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc). METHODS: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography. RESULTS: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect. CONCLUSIONS: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile. CI - Copyright © 2023 Elsevier B.V. All rights reserved. FAU - Tandaipan, J AU - Tandaipan J AD - Department of Rheumatology and Systemic Autoinmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Guillén-Del-Castillo, A AU - Guillén-Del-Castillo A AD - Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, Barcelona, Spain. FAU - Simeón-Aznar, C P AU - Simeón-Aznar CP AD - Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d'Hebrón, Barcelona, Spain. FAU - Carreira, P E AU - Carreira PE AD - Department of Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - De la Puente, C AU - De la Puente C AD - Department of Rheumatology, Hospital Universitario Ramon y Cajal, Madrid, Spain. FAU - Narváez, J AU - Narváez J AD - Department of Rheumatology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Spain. FAU - Lluch, J AU - Lluch J AD - Department of Rheumatology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Spain. FAU - Rubio-Rivas, M AU - Rubio-Rivas M AD - Department of Internal Medicine, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Spain. FAU - Alegre-Sancho, J J AU - Alegre-Sancho JJ AD - Department of Rheumatology, Hospital Universitario Doctor Peset, Valencia, Spain. FAU - Bonilla, G AU - Bonilla G AD - Department of Rheumatology, Hospital Universitario La Paz, Madrid, Spain. FAU - Moriano, C AU - Moriano C AD - Department of Rheumatology, Complejo Asistencial Universitario de León, León, Spain. FAU - Casafont-Sole, I AU - Casafont-Sole I AD - Department of Rheumatology, Hospital Germans Trias i Pujol, Badalona, Spain. FAU - García-Vicuña, R AU - García-Vicuña R AD - Department of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain. FAU - Ortiz-Santamaría, V AU - Ortiz-Santamaría V AD - Department of Rheumatology, Hospital General de Granollers, Granollers, Spain. FAU - Riera, E AU - Riera E AD - Department of Rheumatology, Hospital Universitari Mútua de Terrassa, Terrassa, Spain. FAU - Atienza-Mateo, B AU - Atienza-Mateo B AD - Department of Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain. FAU - Blanco, R AU - Blanco R AD - Department of Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain. FAU - Galisteo, C AU - Galisteo C AD - Department of Rheumatology, Hospital Universitari Parc Taulí, Sabadell, Spain. FAU - Gonzalez-Martin, J J AU - Gonzalez-Martin JJ AD - Department of Rheumatology, Hospital Universitario HM San Chinarro, Madrid, Spain. FAU - Pego-Reigosa, J M AU - Pego-Reigosa JM AD - Department of Rheumatology, Complejo Hospitalario Universitario de Vigo, IRIDIS (Investigation in Rheumatology and Immune-Mediated Diseases)-VIGO Group, Galicia Sur Health Research Institute (IISGS), Vigo, Spain. FAU - Pros, A AU - Pros A AD - Department of Rheumatology, Hospital del Mar, Barcelona, Spain. FAU - Heredia, S AU - Heredia S AD - Department of Rheumatology, Hospital Sant Joan Despí Moisès Broggi, Sant Joan Despí, Spain. FAU - Castellví, I AU - Castellví I AD - Department of Rheumatology and Systemic Autoinmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain; Department of Medicine, Universitat Autonoma de Barcelona, Spain. Electronic address: icastellvi@santpau.cat. LA - eng PT - Journal Article PT - Review DEP - 20230912 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Female MH - Humans MH - Male MH - Immunoglobulins, Intravenous/therapeutic use MH - *Scleroderma, Systemic/complications/drug therapy MH - Retrospective Studies MH - Skin MH - *Myositis/drug therapy MH - Multicenter Studies as Topic MH - Observational Studies as Topic OTO - NOTNLM OT - Immunoglobulins intravenous OT - Myositis OT - Systemic sclerosis OT - Treatment COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2023/09/15 00:42 MHDA- 2023/11/03 06:44 CRDT- 2023/09/14 19:14 PHST- 2023/08/08 00:00 [received] PHST- 2023/09/03 00:00 [accepted] PHST- 2023/11/03 06:44 [medline] PHST- 2023/09/15 00:42 [pubmed] PHST- 2023/09/14 19:14 [entrez] AID - S1568-9972(23)00175-1 [pii] AID - 10.1016/j.autrev.2023.103441 [doi] PST - ppublish SO - Autoimmun Rev. 2023 Nov;22(11):103441. doi: 10.1016/j.autrev.2023.103441. Epub 2023 Sep 12. PMID- 27692000 OWN - NLM STAT- MEDLINE DCOM- 20180220 LR - 20260127 IS - 1601-5215 (Electronic) IS - 0924-2708 (Linking) VI - 29 IP - 3 DP - 2017 Jun TI - Frequency of autoimmune disorders and autoantibodies in patients with neuromyelitis optica. PG - 170-178 LID - 10.1017/neu.2016.49 [doi] AB - OBJECTIVE: The aim of this study was to report the frequency of autoimmune disorders and autoantibodies in 22 patients with neuromyelitis optica (NMO), as well as whether the seropositivity for autoantibodies differs between anti-aquaporin 4 (AQP4) positive and AQP4 negative NMO patients. METHODS: Demographic, medical records, and a profile of autoantibodies were evaluated in 22 NMO patients, including AQP4, anti-thyroid-stimulating hormone receptor, antinuclear antibodies (ANA), anti-thyroperoxidase (anti-TPO), anti-thyroglobulin (anti-Tg), anti-double-stranded DNA, anti-neutrophil cytoplasmic, anti-cyclic citrullinate peptide, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, anti-Smith antibodies (anti-Sm), anti-ribonucleoprotein, anti-nucleosome, and anti-Scl70. Thyroid-stimulating hormone and free thyroxin were measured. RESULTS: The frequency of women was higher than men (95.5% vs. 4.5%) and 68.2% were Afro-Brazilians. Six (27.3%) patients presented other autoimmune disorders, such as Hashimoto thyroiditis (n=2), Graves' disease (n=1), juvenile idiopathic arthritis (n=1), systemic lupus erythematosus and systemic sclerosis (n=1), and Raynaud's phenomenon (n=1). The most frequent autoantibodies were anti-AQP4 (54.5%), anti-nucleosome (31.8%), ANA (27.3%), anti-TPO (22.7%), and anti-Tg (22.7%). Difference was not observed in the frequency of autoimmune disorders when the patients were compared according to their anti-AQP4 status. CONCLUSION: The results of the present study underscored that the NMO patients present high frequency of autoantibodies against cellular antigens and the presence of autoimmune disorders. Further studies with large number of NMO patients may contribute to advances in the understanding of NMO disease mechanisms. FAU - Pereira, Wildéa Lice de Carvalho Jennings AU - Pereira WLCJ AD - 1Health Sciences Postgraduate Program,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Reiche, Edna Maria Vissoci AU - Reiche EMV AD - 2Department of Pathology, Clinical Analysis and Toxicology,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Kallaur, Ana Paula AU - Kallaur AP AD - 1Health Sciences Postgraduate Program,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Oliveira, Sayonara Rangel AU - Oliveira SR AD - 1Health Sciences Postgraduate Program,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Simão, Andréa Name Colado AU - Simão ANC AD - 2Department of Pathology, Clinical Analysis and Toxicology,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Lozovoy, Marcell Alysson Batisti AU - Lozovoy MAB AD - 2Department of Pathology, Clinical Analysis and Toxicology,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Schiavão, Lucas José Vaz AU - Schiavão LJV AD - 3Neurology Outpatient Department of the Outpatient Specialties of University Hospital,State University of Londrina,Londrina,Paraná,Brazil. FAU - Rodrigues, Paula Raquel do Vale Pascoal AU - Rodrigues PRDVP AD - 3Neurology Outpatient Department of the Outpatient Specialties of University Hospital,State University of Londrina,Londrina,Paraná,Brazil. FAU - Alfieri, Daniela Frizon AU - Alfieri DF AD - 1Health Sciences Postgraduate Program,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Flauzino, Tamires AU - Flauzino T AD - 1Health Sciences Postgraduate Program,Health Sciences Center,State University of Londrina,Londrina,Paraná,Brazil. FAU - Kaimen-Maciel, Damacio Ramón AU - Kaimen-Maciel DR AD - 3Neurology Outpatient Department of the Outpatient Specialties of University Hospital,State University of Londrina,Londrina,Paraná,Brazil. LA - eng PT - Journal Article DEP - 20161003 PL - England TA - Acta Neuropsychiatr JT - Acta neuropsychiatrica JID - 9612501 RN - 0 (Aquaporin 4) RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Iron-Binding Proteins) RN - 0 (anti-thyroglobulin) RN - EC 1.11.1.7 (TPO protein, human) RN - EC 1.11.1.8 (Iodide Peroxidase) SB - IM MH - Adult MH - Aquaporin 4/immunology MH - Arthritis, Juvenile/complications/epidemiology/*immunology/pathology MH - Autoantibodies/*immunology MH - Autoantigens MH - Autoimmune Diseases/*epidemiology/*immunology/pathology MH - Brazil/epidemiology MH - Female MH - Humans MH - Iodide Peroxidase MH - Iron-Binding Proteins MH - Lupus Erythematosus, Systemic/complications/epidemiology/*immunology/pathology MH - Male MH - Middle Aged MH - Neuromyelitis Optica/complications/epidemiology/*immunology/pathology MH - Scleroderma, Systemic/complications/epidemiology/*immunology/pathology MH - Thyroiditis, Autoimmune/complications/epidemiology/*immunology/pathology OTO - NOTNLM OT - anti-aquaporin 4 OT - autoimmune thyroiditis OT - autoimmunity OT - juvenile idiopathic arthritis OT - neuromyelitis optica EDAT- 2016/10/04 06:00 MHDA- 2018/02/21 06:00 CRDT- 2016/10/04 06:00 PHST- 2016/10/04 06:00 [pubmed] PHST- 2018/02/21 06:00 [medline] PHST- 2016/10/04 06:00 [entrez] AID - S0924270816000491 [pii] AID - 10.1017/neu.2016.49 [doi] PST - ppublish SO - Acta Neuropsychiatr. 2017 Jun;29(3):170-178. doi: 10.1017/neu.2016.49. Epub 2016 Oct 3. PMID- 27156934 OWN - NLM STAT- MEDLINE DCOM- 20160713 LR - 20260127 IS - 1474-547X (Electronic) IS - 0140-6736 (Linking) VI - 387 IP - 10038 DP - 2016 Jun 25 TI - Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. PG - 2630-2640 LID - S0140-6736(16)00232-4 [pii] LID - 10.1016/S0140-6736(16)00232-4 [doi] AB - BACKGROUND: Systemic sclerosis is a rare disabling autoimmune disease with few treatment options. The efficacy and safety of tocilizumab, an interleukin 6 receptor-α inhibitor, was assessed in the faSScinate phase 2 trial in patients with systemic sclerosis. METHODS: We did this double-blind, placebo-controlled study at 35 hospitals in Canada, France, Germany, the UK, and the USA. We enrolled adults with progressive systemic sclerosis of 5 or fewer years' duration from first non-Raynaud's sign or symptom. Patients were randomly assigned (1:1) to weekly subcutaneous tocilizumab 162 mg or placebo. The primary endpoint was the difference in mean change from baseline in modified Rodnan skin score at 24 weeks. This study is registered with ClinicalTrials.gov, number NCT01532869. FINDINGS: We enrolled 87 patients: 43 assigned to tocilizumab and 44 assigned to placebo. The least squares mean change in modified Rodnan skin score at 24 weeks was -3·92 in the tocilizumab group and -1·22 in the placebo group (difference -2·70, 95% CI -5·85 to 0·45; p=0·0915). The least squares mean change at 48 weeks was -6·33 in the tocilizumab group and -2·77 in the placebo group (treatment difference -3·55, 95% CI -7·23 to 0·12; p=0·0579). In one of several exploratory analyses, fewer patients in the tocilizumab group than in the placebo group had a decline in percent predicted forced vital capacity at 48 weeks (p=0·0373). However, we detected no significant difference in disability, fatigue, itching, or patient or clinician global disease severity. 42 (98%) of 43 patients in the tocilizumab group versus 40 (91%) of 44 in the placebo group had adverse events. 14 (33%) versus 15 (34%) had serious adverse events. Serious infections were more common in the tocilizumab group (seven [16%] of 43 patients) than in the placebo group (two [5%] of 44). One patient died in relation to tocilizumab treatment. INTERPRETATION: Tocilizumab was not associated with a significant reduction in skin thickening. However, the difference was greater in the tocilizumab group than in the placebo group and we found some evidence of less decline in forced vital capacity. The efficacy and safety of tocilizumab should be investigated in a phase 3 trial before definitive conclusions can be made about its risks and benefits. FUNDING: F Hoffmann-La Roche, Genentech. CI - Copyright © 2016 Elsevier Ltd. All rights reserved. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan Scleroderma Program, Ann Arbor, MI, USA. Electronic address: khannad@med.umich.edu. FAU - Denton, Christopher P AU - Denton CP AD - University College London, London, UK. FAU - Jahreis, Angelika AU - Jahreis A AD - Genentech, South San Francisco, CA, USA. FAU - van Laar, Jacob M AU - van Laar JM AD - University Medical Center Utrecht, Utrecht, Netherlands. FAU - Frech, Tracy M AU - Frech TM AD - University of Utah, Veterans Affairs Medical Center, Salt Lake City, UT, USA. FAU - Anderson, Marina E AU - Anderson ME AD - University of Liverpool and Aintree University Hospital, Liverpool, UK. FAU - Baron, Murray AU - Baron M AD - Jewish General Hospital, Montreal, QC, Canada. FAU - Chung, Lorinda AU - Chung L AD - Stanford University School of Medicine and Palo Alto VA Health Care System, Palo Alto, CA, USA. FAU - Fierlbeck, Gerhard AU - Fierlbeck G AD - University of Tübingen, Tübingen, Germany. FAU - Lakshminarayanan, Santhanam AU - Lakshminarayanan S AD - University of Connecticut Health Center, Farmington, CT, USA. FAU - Allanore, Yannick AU - Allanore Y AD - Paris Descartes University and Cochin Hospital, Paris, France. FAU - Pope, Janet E AU - Pope JE AD - Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Charité University Hospital, German Rheumatism Research Centre, Berlin, Germany. FAU - Steen, Virginia AU - Steen V AD - Georgetown University, Washington, DC, USA. FAU - Müller-Ladner, Ulf AU - Müller-Ladner U AD - Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany. FAU - Lafyatis, Robert AU - Lafyatis R AD - Boston University School of Medicine, Boston, MA, USA. FAU - Stifano, Giuseppina AU - Stifano G AD - Boston University School of Medicine, Boston, MA, USA. FAU - Spotswood, Helen AU - Spotswood H AD - Roche Products, Welwyn Garden City, UK. FAU - Chen-Harris, Haiyin AU - Chen-Harris H AD - Genentech, South San Francisco, CA, USA. FAU - Dziadek, Sebastian AU - Dziadek S AD - Roche, Basel, Switzerland. FAU - Morimoto, Alyssa AU - Morimoto A AD - Genentech, South San Francisco, CA, USA. FAU - Sornasse, Thierry AU - Sornasse T AD - Genentech, South San Francisco, CA, USA. FAU - Siegel, Jeffrey AU - Siegel J AD - Genentech, South San Francisco, CA, USA. FAU - Furst, Daniel E AU - Furst DE AD - University of California, Los Angeles, CA, USA. LA - eng SI - ClinicalTrials.gov/NCT01532869 PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20160505 PL - England TA - Lancet JT - Lancet (London, England) JID - 2985213R RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Biomarkers) RN - 0 (Interleukin-6) RN - I031V2H011 (tocilizumab) SB - IM CIN - Lancet. 2016 Jun 25;387(10038):2580-2581. doi: 10.1016/S0140-6736(16)00622-X. PMID: 27156931 EIN - Lancet. 2018 Apr 7;391(10128):1356. doi: 10.1016/S0140-6736(18)30807-9. PMID: 29636271 MH - Adult MH - Aged MH - Antibodies, Monoclonal, Humanized/administration & dosage/*therapeutic use MH - Biomarkers/metabolism MH - Canada MH - Double-Blind Method MH - Europe MH - Female MH - Humans MH - Injections, Subcutaneous MH - Interleukin-6/physiology MH - Male MH - Middle Aged MH - Scleroderma, Systemic/complications/*drug therapy/metabolism MH - Treatment Outcome MH - United Kingdom MH - Vital Capacity EDAT- 2016/05/10 06:00 MHDA- 2016/07/14 06:00 CRDT- 2016/05/10 06:00 PHST- 2016/05/10 06:00 [entrez] PHST- 2016/05/10 06:00 [pubmed] PHST- 2016/07/14 06:00 [medline] AID - S0140-6736(16)00232-4 [pii] AID - 10.1016/S0140-6736(16)00232-4 [doi] PST - ppublish SO - Lancet. 2016 Jun 25;387(10038):2630-2640. doi: 10.1016/S0140-6736(16)00232-4. Epub 2016 May 5. PMID- 30402270 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240607 IS - 2056-5933 (Print) IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 4 IP - Suppl 1 DP - 2018 TI - Systemic sclerosis: state of the art on clinical practice guidelines. PG - e000782 LID - 10.1136/rmdopen-2018-000782 [doi] LID - e000782 AB - Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Scirè, Carlo Alberto AU - Scirè CA AUID- ORCID: 0000-0001-7451-0271 AD - Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy. AD - Epidemiology Unit, Italian Society for Rheumatology (SIR), Milan, Italy. FAU - Talarico, Rosaria AU - Talarico R AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Airo, Paolo AU - Airo P AD - Rheumatology and Clinical Immunology Unit, Azienda Ospedaliera Spedali Civili di Brescia, Brescia, Italy. FAU - Alexander, Tobias AU - Alexander T AUID- ORCID: 0000-0003-1193-0097 AD - Department of Rheumatology and Clinical Immunology Unit, Charité University Hospital Berlin, Berlin, Germany. FAU - Allanore, Yannick AU - Allanore Y AD - Service de Médicine Interne, Université Paris Descartes, Paris, France. AD - Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Paris, France. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Department of Rheumatology, University of Pavia, Pavia, Italy. AD - Department of Rheumatology, IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Dalm, Virgil AU - Dalm V AD - Department of Internal Medicine and Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - De Vries-Bouwstra, Jeska AU - De Vries-Bouwstra J AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Galetti, Ilaria AU - Galetti I AD - Federation of European Scleroderma Associations (FESCA), Brussels, Belgium. FAU - Launay, David AU - Launay D AD - Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France. AD - Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, CHU Lille, Lille, France. FAU - Lepri, Gemma AU - Lepri G AD - Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy. FAU - Mathian, Alexis AU - Mathian A AD - Department of Internal Medicine, Hospital Pitié-Salpêtrière, Paris, France. FAU - Mouthon, Luc AU - Mouthon L AD - Service de Médicine Interne, Université Paris Descartes, Paris, France. AD - Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Cochin, Paris, France. FAU - Ruaro, Barbara AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy. FAU - Tincani, Angela AU - Tincani A AD - Rheumatology and Clinical Immunology Unit, Azienda Ospedaliera Spedali Civili di Brescia, Brescia, Italy. FAU - Vandecasteele, Els AU - Vandecasteele E AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Department of Cardiology, Ghent University Hospital, Ghent, Belgium. FAU - Vanhaecke, Amber AU - Vanhaecke A AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Vanthuyne, Marie AU - Vanthuyne M AD - Department of Rheumatology, Université Catholique de Louvain, Louvain-la-Neuve, Belgium. AD - Department of Rheumatology, Cliniques Universitaires Saint-Luc, Louvain-la-Neuve, Belgium. FAU - Van den Hoogen, Frank AU - Van den Hoogen F AD - Department of Rheumatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands. AD - Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands. FAU - Van Vollenhoven, Ronald AU - Van Vollenhoven R AD - Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands. FAU - Voskuyl, Alexandre E AU - Voskuyl AE AD - Department of Rheumatology, Amsterdam UMC, Amsterdam, The Netherlands. AD - Amsterdam Infection & Immunity Institute, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Division of Rheumatology, Department of Medicine, University of Padova, Padova, Italy. FAU - Bombardieri, Stefano AU - Bombardieri S AD - Rheumatology Unit, Department of Clinical & Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Burmester, Gerd AU - Burmester G AD - Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany. FAU - Eurico, Fonseca João AU - Eurico FJ AD - Department of Rheumatology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal. AD - Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal. FAU - Frank, Charissa AU - Frank C AD - Flemish Patient Organization of Hereditary Collagen Disorders in Belgium, Koersel, Belgium. FAU - Hachulla, Eric AU - Hachulla E AD - Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France. AD - Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, CHU Lille, Lille, France. FAU - Houssiau, Frederic AU - Houssiau F AD - Department of Rheumatology, Université Catholique de Louvain, Louvain-la-Neuve, Belgium. AD - Department of Rheumatology, Cliniques Universitaires Saint-Luc, Louvain-la-Neuve, Belgium. FAU - Mueller-Ladner, Ulf AU - Mueller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Kerckhoff Klinik, Justus-Liebig University of Giessen, Giessen, Germany. FAU - Schneider, Matthias AU - Schneider M AD - Institute for Rheumatology, Hiller Research Unit for Rheumatology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany. FAU - van Laar, Jacob M AU - van Laar JM AD - Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Vieira, Ana AU - Vieira A AD - Núcleo Síndrome de Sjögren of Liga Portuguesa Contra as Doenças Reumáticas (LPCDR, Portuguese League Against Rheumatic Diseases), Lisbon, Portugal. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. AD - Division of Rheumatology and Scleroderma Unit, AOU Careggi, Florence, Italy. LA - eng PT - Journal Article DEP - 20181018 PL - England TA - RMD Open JT - RMD open JID - 101662038 PMC - PMC6203100 OTO - NOTNLM OT - ERN ReCONNET OT - European reference networks OT - clinical practice guidelines OT - nailfold videocapillaroscopy OT - systemic sclerosis OT - unmet needs COIS- Conflicts of interest: VS, None to declare. CAS, None to declare. RT, None to declare. PA, None to declare. TA, None to declare. YA, consulted for Actelion, Bayer, Roche/Genentech, Inventiva, Medac, Pfizer, Sanofi, Servier, and UCB; and has received research grants from Bristol-Myers Squibb, Roche/Genentech, Inventiva, Pfizer, Sanofi. CB, None to declare. VC, None to declare. VD, None to declare. JVB, None to declare. ADS, None to declare. OD, had consultancy relationship and/or has received research funding from Actelion, AnaMar, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, ChemomAb, Roche, GSK, Inventiva, Italfarmaco, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Sanofi, and UCB in the area of potential treatments ofscleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of systemic sclerosis licensed. The real or perceived potential conflicts listed above are accurately stated. IG, None to declare. DL, None to declare. GL, None to declare. AM, None to declare. LM, None to declare. BR, None to declare. AS, None to declare. AT, None to declare. EV, None to declare. AV, None to declare. MV, None to declare. FVH, None to declare. RVV, consulted for AbbVie, AstraZeneca, Biogen, Biotest, BMS, Celgene, Gilead, GSK, Janssen, Lilly, Novartis, Pfizer, UCB; and received research support and grants from AbbVie, BMS, GSK, Pfizer, UCB. AV None to declare. EZ, None to declare. SB, None to declare. GB, None to declare. FJE, None to declare. CF, None to declare. EH, None to declare. FH, None to declare. UML, None to declare. MS, None to declare. JML, None to declare. AV, None to declare. MC, None to declare. MM, None to declare. MMC, has consultancy relationship and/or has received research funding for Actelion, BMS, Celgene, Chemomab, CSL Behring, Eli Lilly and Pfizer; and is a member of the college of emeritus presidents of the Italian Society of Rheumatology (SIR). EDAT- 2018/11/08 06:00 MHDA- 2018/11/08 06:01 PMCR- 2018/10/18 CRDT- 2018/11/08 06:00 PHST- 2018/07/31 00:00 [received] PHST- 2018/09/21 00:00 [revised] PHST- 2018/09/22 00:00 [accepted] PHST- 2018/11/08 06:00 [entrez] PHST- 2018/11/08 06:00 [pubmed] PHST- 2018/11/08 06:01 [medline] PHST- 2018/10/18 00:00 [pmc-release] AID - rmdopen-2018-000782 [pii] AID - 10.1136/rmdopen-2018-000782 [doi] PST - epublish SO - RMD Open. 2018 Oct 18;4(Suppl 1):e000782. doi: 10.1136/rmdopen-2018-000782. eCollection 2018. PMID- 38383267 OWN - NLM STAT- MEDLINE DCOM- 20240523 LR - 20240531 IS - 1578-8989 (Electronic) IS - 0025-7753 (Linking) VI - 162 IP - 10 DP - 2024 May 31 TI - Immune thrombocytopenia in systemic lupus erythematosus: Prevalence, risk factors, and a novel predictive model for risk assessment. PG - 461-469 LID - S0025-7753(24)00029-0 [pii] LID - 10.1016/j.medcli.2023.12.012 [doi] AB - INTRODUCTION: Immune thrombocytopenia (ITP) is a potentially severe manifestation of systemic lupus erythematosus (SLE) reported in 7-40% of SLE patients. ITP has been associated with a higher risk of organ damage and mortality. OBJECTIVES: To describe which factors are associated with the presence of ITP in SLE patients. METHODS: Retrospective case-control study. Cases were defined as SLE patients who had ever developed ITP and were sex- and age-matched with two controls. A predictive model was constructed to identify SLE patients who were at risk of developing ITP. RESULTS: ITP prevalence in our SLE cohort was 8.35%. Cases had a higher frequency of hemolytic anemia, while controls had a higher prevalence of arthritis at SLE diagnosis. During SLE progression, cases tested positive for anticardiolipin, anti-β2-glycoprotein 1, and lupus anticoagulant antibodies more frequently. Cases received mycophenolic acid and azathioprine more often than controls and had a higher SLICC/ACR score. The model demonstrated a sensitivity of 87.53%, a positive predictive value of 81.92%, a specificity of 80.50%, area under the curve of 83.92%, a F1 of 83% and an overall accuracy of 83.68%. The variables that best explain the model were hemolytic anemia, arthritis, oral ulcers, Raynaud's phenomenon, low C4, low CH50, anticardiolipin and anti-β2GP1 antibodies. CONCLUSION: SLE patients who develop ITP have a distinct phenotype characterized by more hemolytic anemia and less arthritis at SLE onset, and higher prevalence of antiphospholipid syndrome antibodies during SLE progression. This phenotype is associated with heightened organ damage and the need for more intensive therapies and stricter follow-up. Our predictive model has demonstrated an impressive ability to identify SLE patients at risk of developing ITP. CI - Copyright © 2024 Elsevier España, S.L.U. All rights reserved. FAU - Cornudella Lema, Jesús AU - Cornudella Lema J AD - Universitat Pompeu Fabra - Universitat Autònoma de Barcelona, Spain. FAU - Sánchez-González, Blanca AU - Sánchez-González B AD - Department of Hematology, Hospital del Mar, Barcelona, Spain; Hospital del Mar Research Institute, Barcelona, Spain; Hospital del Mar Autoimmune Diseases and Vasculitis Unit of Excellence Unit Disease, Spain. FAU - Carrión-Barberà, Irene AU - Carrión-Barberà I AD - Hospital del Mar Research Institute, Barcelona, Spain; Hospital del Mar Autoimmune Diseases and Vasculitis Unit of Excellence Unit Disease, Spain; Department of Rheumatology, Hospital del Mar, Barcelona, Spain. Electronic address: ipirenaica@gmail.com. FAU - Vázquez Montes de Oca, Sergio AU - Vázquez Montes de Oca S AD - Biomedical Informatics Research Group, Hospital de Mar Research Institute, Barcelona, Spain. FAU - García Pallarols, Francesc AU - García Pallarols F AD - Department of Hematology, Hospital del Mar, Barcelona, Spain; Hospital del Mar Research Institute, Barcelona, Spain. FAU - Salman-Monte, Tarek Carlos AU - Salman-Monte TC AD - Hospital del Mar Research Institute, Barcelona, Spain; Hospital del Mar Autoimmune Diseases and Vasculitis Unit of Excellence Unit Disease, Spain; Department of Rheumatology, Hospital del Mar, Barcelona, Spain. LA - eng LA - spa PT - Journal Article DEP - 20240220 PL - Spain TA - Med Clin (Barc) JT - Medicina clinica JID - 0376377 SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - Female MH - Retrospective Studies MH - Male MH - Adult MH - Prevalence MH - Case-Control Studies MH - Risk Assessment MH - Risk Factors MH - *Purpura, Thrombocytopenic, Idiopathic/epidemiology/etiology MH - Middle Aged MH - Young Adult OTO - NOTNLM OT - Antiphospholipid syndrome OT - Case–control studies OT - Estudios de casos y controles OT - Immune thrombocytopenia OT - Lupus eritematoso sistémico OT - Random forest OT - Systemic lupus erythematosus OT - Síndrome antifosfolípido OT - Trombocitopenia inmune EDAT- 2024/02/22 00:43 MHDA- 2024/05/24 00:42 CRDT- 2024/02/21 21:57 PHST- 2023/09/28 00:00 [received] PHST- 2023/12/05 00:00 [revised] PHST- 2023/12/08 00:00 [accepted] PHST- 2024/05/24 00:42 [medline] PHST- 2024/02/22 00:43 [pubmed] PHST- 2024/02/21 21:57 [entrez] AID - S0025-7753(24)00029-0 [pii] AID - 10.1016/j.medcli.2023.12.012 [doi] PST - ppublish SO - Med Clin (Barc). 2024 May 31;162(10):461-469. doi: 10.1016/j.medcli.2023.12.012. Epub 2024 Feb 20. PMID- 33242341 OWN - NLM STAT- MEDLINE DCOM- 20210920 LR - 20240928 IS - 1365-2125 (Electronic) IS - 0306-5251 (Print) IS - 0306-5251 (Linking) VI - 87 IP - 7 DP - 2021 Jul TI - Riociguat: Clinical research and evolving role in therapy. PG - 2645-2662 LID - 10.1111/bcp.14676 [doi] AB - Riociguat is a first-in-class soluble guanylate cyclase stimulator, approved for the treatment of adults with pulmonary arterial hypertension (PAH), inoperable chronic thromboembolic pulmonary hypertension (CTEPH), or persistent or recurrent CTEPH after pulmonary endarterectomy. Approval was based on the results of the phase III PATENT-1 (PAH) and CHEST-1 (CTEPH) studies, with significant improvements in the primary endpoint of 6-minute walk distance vs placebo of +36 m and +46 m, respectively, as well as improvements in secondary endpoints such as pulmonary vascular resistance and World Health Organization functional class. Riociguat acts as a stimulator of cyclic guanosine monophosphate synthesis rather than as an inhibitor of cGMP metabolism. As with other approved therapies for PAH, riociguat has antifibrotic, antiproliferative and anti-inflammatory effects, in addition to vasodilatory properties. This has led to further clinical studies in patients who do not achieve a satisfactory clinical response with phosphodiesterase type-5 inhibitors. Riociguat has also been evaluated in patients with World Health Organization group 2 and 3 pulmonary hypertension, and other conditions including diffuse cutaneous systemic sclerosis, Raynaud's phenomenon and cystic fibrosis. This review evaluates the results of the original clinical trials of riociguat for the treatment of PAH and CTEPH, and summarises the body of work that has examined the safety and efficacy of riociguat for the treatment of other types of pulmonary hypertension. CI - © 2020 The Authors. British Journal of Clinical Pharmacology published by John Wiley & Sons Ltd on behalf of British Pharmacological Society.. FAU - Klinger, James R AU - Klinger JR AUID- ORCID: 0000-0003-2997-5955 AD - Division of Pulmonary, Sleep, and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA. FAU - Chakinala, Murali M AU - Chakinala MM AD - Division of Pulmonary and Critical Care Medicine, Washington University School of Medicine, St Louis, Missouri, USA. FAU - Langleben, David AU - Langleben D AD - Center for Pulmonary Vascular Disease and Lady Davis Institute, Jewish General Hospital, McGill University, Montreal, Canada. FAU - Rosenkranz, Stephan AU - Rosenkranz S AD - Clinic III for Internal Medicine (Cardiology), and Center for Molecular Medicine Cologne (CMMC), University of Cologne, Cologne, Germany. AD - Cologne Cardiovascular Research Center (CCRC), University of Cologne, Cologne, Germany. FAU - Sitbon, Olivier AU - Sitbon O AD - Universite Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France. AD - AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. AD - INSERM UMR_S 999, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20201230 PL - England TA - Br J Clin Pharmacol JT - British journal of clinical pharmacology JID - 7503323 RN - 0 (Pyrazoles) RN - 0 (Pyrimidines) RN - RU3FE2Y4XI (riociguat) SB - IM MH - Adult MH - Humans MH - *Hypertension, Pulmonary/drug therapy MH - *Pulmonary Arterial Hypertension MH - Pyrazoles/therapeutic use MH - Pyrimidines/therapeutic use PMC - PMC8359233 OTO - NOTNLM OT - drug information OT - pharmacotherapy OT - therapeutics COIS- J.R.K. reports research support to his institution from Actelion, Bayer AG, Lung Biotechnology and United Therapeutics. M.M.C. has received research support from Actelion, Eiger BioPharmaceuticals, GeNO LLC, Gilead, GlaxoSmithKline, Medtronic and Reata Pharmaceuticals; consulting fees from Actelion, Express Scripts Holding Company, Gilead, SteadyMed Therapeutics, United Therapeutics and WebMD LLC (Medscape); and honoraria for speaking for Bayer AG and Gilead. D.L. reports honoraria, consultation fees, research support and/or travel expenses from Actelion, Arena, Bayer AG, Northern Therapeutics, PhaseBio and United Therapeutics. S.R. reports grants and personal fees from Abbott, Actelion, Arena, Bayer AG, Ferrer, Gilead, GlaxoSmithKline, MSD, Novartis, Pfizer and United Therapeutics, and research support from Actelion, Bayer, Novartis, Pfizer and United Therapeutics. O.S. reports grants, personal fees and nonfinancial support from Actelion, Bayer AG, GlaxoSmithKline and Merck, and personal fees from Arena. EDAT- 2020/11/27 06:00 MHDA- 2021/09/21 06:00 PMCR- 2020/12/30 CRDT- 2020/11/26 17:06 PHST- 2020/10/30 00:00 [revised] PHST- 2020/05/07 00:00 [received] PHST- 2020/11/13 00:00 [accepted] PHST- 2020/11/27 06:00 [pubmed] PHST- 2021/09/21 06:00 [medline] PHST- 2020/11/26 17:06 [entrez] PHST- 2020/12/30 00:00 [pmc-release] AID - BCP14676 [pii] AID - 10.1111/bcp.14676 [doi] PST - ppublish SO - Br J Clin Pharmacol. 2021 Jul;87(7):2645-2662. doi: 10.1111/bcp.14676. Epub 2020 Dec 30. PMID- 37699157 OWN - NLM STAT- MEDLINE DCOM- 20231009 LR - 20231009 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 32 IP - 11 DP - 2023 Oct TI - Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus: A meta-analysis. PG - 1310-1319 LID - 10.1177/09612033231202398 [doi] AB - OBJECTIVE: To determine the risk factors of pulmonary arterial hypertension (PAH) related to systemic lupus erythematosus (SLE) through systematic reviews and meta-analyses. METHODS: We undertook electronic search strategies using Medline via PubMed, Embase, Web of Science, and Cochrane Library up to April 11, 2023. Study selection and data extraction were performed by 2 authors independently. We made risk of bias judgments based on the Newcastle-Ottawa Scale (NOS). Pooled risk ratios (RRs) and 95% confidence intervals (CIs) were calculated to estimate the overall effect sizes of potential risk factors for PAH in SLE patients. Univariate and multivariate meta-regression models were used to assess the independent effects of each risk factor on PAH. Sensitivity analyses were also conducted to explore potential sources of heterogeneity. RESULTS: A total of 19 articles were included in this meta-analysis, and the results showed that gender (female) [RR = 1.04, 95% CI (1.02, 1.06), p = .0001], interstitial lung disease [RR = 4.36, 95% CI (2.42, 7.85), p = .0001], alopecia [RR = 1.39, 95% CI (1.06, 1.83), p = .017], Raynaud's phenomenon [RR = 1.83, 95% CI (1.41, 2.37), p = .0001], systemic hypertension [RR = 1.30, 95% CI (1.07, 1.58), p = .007], serositis [RR = 2.29, 95% CI (1.89, 2.77), p = .0001], pericardial effusion [RR = 3.33, 95% CI (2.20, 5.05), p = .0001], anti-RNP [RR = 1.86, 95% CI (1.19, 2.91), p = .006], anti-SSA [RR = 1.28, 95% CI (1.01, 1.62), p = .041], anti-SSB [RR = 1.38, 95% CI (1.19, 1.60), p = .0001], anti-U1RNP [RR = 1.58, 95% CI (1.07, 2.34), p = .023], thrombocytopenia [RR = 1.38, 95% CI (1.14, 1.68), p = .001], and current smokers [RR = 2.20, 95% CI (1.19, 4.06), p = .012] were all risk factors for PAH related to SLE. CONCLUSION: PAH is a serious complication of SLE. Since prognosis of SLE patients after the occurrence of PAH is poor, routine examination should be conducted for SLE patients with PAH risk factors. FAU - Liu, Yuqi AU - Liu Y AUID- ORCID: 0009-0008-0649-0414 AD - Beijing Luhe Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Cheng, Zhen AU - Cheng Z AD - Beijing Tiantan Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Zha, Bowen AU - Zha B AD - Beijing Anzhen Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Chen, Xiaodong AU - Chen X AD - Beijing Luhe Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Gong, Zhiyu AU - Gong Z AD - Beijing Luhe Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Ji, Lang AU - Ji L AD - Central Laboratory, Beijing Luhe Clinical Institute, Capital Medical University, Beijing, China. RINGGOLD: 12517 FAU - Wei, Lingling AU - Wei L AUID- ORCID: 0000-0001-5270-9853 AD - Beijing Key Laboratory of Diabetic Prevention and Research, Center for Endocrine Metabolism and Immune Diseases, Beijing Luhe Hospital, Capital Medical University, Beijing, China. RINGGOLD: 12517 LA - eng PT - Journal Article PT - Meta-Analysis DEP - 20230912 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Humans MH - Female MH - *Pulmonary Arterial Hypertension/epidemiology/etiology MH - *Hypertension, Pulmonary/epidemiology/etiology/diagnosis MH - *Lupus Erythematosus, Systemic/complications/epidemiology/diagnosis MH - Risk Factors MH - Prognosis OTO - NOTNLM OT - Pulmonary arterial hypertension OT - meta-analysis OT - risk factors OT - systemic lupus erythematosus COIS- Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/09/12 18:41 MHDA- 2023/10/09 06:42 CRDT- 2023/09/12 15:53 PHST- 2023/10/09 06:42 [medline] PHST- 2023/09/12 18:41 [pubmed] PHST- 2023/09/12 15:53 [entrez] AID - 10.1177/09612033231202398 [doi] PST - ppublish SO - Lupus. 2023 Oct;32(11):1310-1319. doi: 10.1177/09612033231202398. Epub 2023 Sep 12. PMID- 18799052 OWN - NLM STAT- MEDLINE DCOM- 20090205 LR - 20221207 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 3 Suppl 49 DP - 2008 May-Jun TI - Unclassified vasculitis with acral ischemic lesions: "forme fruste" or idiopathic vasculitis? PG - S41-6 AB - BACKGROUND AND OBJECTIVES: While acral ischemia and necrosis represent a common problem in connective tissue diseases and other disorders, acral ischemic lesions are also occasionally encountered in primary and secondary systemic vasculitides. Here we report on the course of 4 patients with acral ischemic lesions as a hallmark of unclassified vasculitis. We compare these cases with 4 additional cases of acral ischemia complicating classified vasculitis. OBJECTIVES: To report on our experience with cases of unclassified vasculitis and acral ischemic lesions during the past 5 years and review the literature on vasculitis and acral ischemic lesions. METHODS: The case history of one of the patients with unclassified vasculitis and acral ischemic lesions is reported in detail. The Medical history of another 3 patients presenting with vasculitic acral ischemic lesions and unclassified vasculitis during the past 5 years in our department (Lübeck/Bad Bramstedt) is summarized and compared to the course of patients with acral ischemic lesions complicating classified vasculitides. A PubMed database review of reports on acral ischemic lesions and vasculitis from 1985 to August 2006 was performed using the following combination of keywords: "Vasculitis" [MeSH] AND ("Necrosis" [MeSH] OR "Ischemia" [MeSH] OR "Infarction" [MeSH]) AND ("Extremities" [MeSH] OR "Fingers [MeSH] OR "Toes" [MeSH] OR "limb"), yielding 1328 entries. This search was subsequently limited to "Humans, All Adult (19+ years)", yielding 904 entries. Only three (0.7%) of these entries described one (one paper) or more (n=28) patients (two papers) with idiopathic vasculitis characterized by digit necrosis in the absence of systemic manifestations (except in some cases for arthralgia) or laboratory parameters pointing to a diagnosis of an established type of vasculitis. RESULTS: A 37-year-old female presented with acral ischemic lesions of the left forefoot, fingers and toes, and Raynaud's phenomenon. Angiography showed multiple stenoses of ulnar and digital arteries, anterior and posterior tibialis arteries, and occlusions of radial artery and occlusion of the plantar artery in the absence of large vessel abnormalities. Histological analysis of an amputation disclosed giant cell arteritis of small vessels. The patient achieved remission with immunosuppressive treatment (cyclophosphamide and prednisolone). Three other patients with acral ischemic lesions and unclassified vasculitis also lacking other manifestations and defining laboratory and technical features during initial presentation and follow-up of 4 month to 5 years are presented. Necrotizing and leukocytoclastic vasculitis were present in two other patients, respectively. In contrast, acral ischemic lesions could be attributed to rheumatoid vasculitis and essential cryoglobulinemic vasculitis in two other cases each based on the patient's history and laboratory findings at the time of presentation of acral ischemic lesions. CONCLUSIONS: While acral ischemic lesions could represent initial or isolated (forme fruste) manifestations of a defined vasculitis, acral ischemic lesions may rarely be encountered as the predominant manifestation of an as yet unclassified vasculitis. the histological findings seem to differ. Our report includes a peculiar case of giant cell arteritis of small arteries not classifiable as giant cell arteritis of large arteries or Takayasu disease. FAU - Pipitone, N AU - Pipitone N AD - Rheumatology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy. pipitone.nicolo@asmn.re.it FAU - Holl-Ulrich, K AU - Holl-Ulrich K FAU - Gross, W L AU - Gross WL FAU - Lamprecht, P AU - Lamprecht P LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Antirheumatic Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - 9PHQ9Y1OLM (Prednisolone) RN - MRK240IY2L (Azathioprine) SB - IM MH - Adult MH - Amputation, Surgical MH - Antirheumatic Agents/therapeutic use MH - Arteritis/*diagnosis/drug therapy/*pathology MH - Azathioprine/therapeutic use MH - Cyclophosphamide/therapeutic use MH - Female MH - Fingers/blood supply/*pathology MH - Forefoot, Human/blood supply/*pathology/surgery MH - Humans MH - Male MH - Middle Aged MH - Necrosis MH - Prednisolone/therapeutic use EDAT- 2008/10/24 09:00 MHDA- 2009/02/06 09:00 CRDT- 2008/10/24 09:00 PHST- 2008/10/24 09:00 [pubmed] PHST- 2009/02/06 09:00 [medline] PHST- 2008/10/24 09:00 [entrez] AID - 2356 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 May-Jun;26(3 Suppl 49):S41-6. PMID- 23597147 OWN - NLM STAT- MEDLINE DCOM- 20150603 LR - 20220410 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 15 IP - 2 DP - 2013 Apr 18 TI - Open label study of escalating doses of oral treprostinil diethanolamine in patients with systemic sclerosis and digital ischemia: pharmacokinetics and correlation with digital perfusion. PG - R54 LID - 10.1186/ar4216 [doi] LID - R54 AB - INTRODUCTION: Treprostinil diethanolamine is an innovative salt form of the prostacyclin analogue, treprostinil sodium, developed as an oral sustained release (SR) osmotic tablet. The availability of a formulation permitting convenient systemic delivery might have applicability to scleroderma vascular complications. We evaluated pharmacokinetics and perfusion in scleroderma patients with digital ischemia following escalating twice-daily doses of treprostinil diethanolamine SR. METHODS: Scleroderma patients with digital ulcers were enrolled in this dual-center, open-label, phase I pharmacokinetic study. Drug concentrations and perfusion, quantified by laser Doppler imaging, were measured over 12 hours at the 2 mg and 4 mg (or maximally tolerated) doses. Pharmacokinetic parameters were determined from individual plasma concentration versus time profiles using non-compartmental analysis methods. Digital perfusion and skin temperature were modeled as a function of log-transformed drug concentration and other covariates by performing repeated measures analyses using random effects models. RESULTS: Nineteen scleroderma patients (84% female, 53% limited scleroderma) received treprostinil diethanolamine SR with dose titration up to 4 mg twice daily as tolerated. Peak concentrations (mean maximum plasma concentration (Cmax) = 1,176 and 2,107 pg/mL) occurred approximately 3.6 hours after dose administration, and overall exposure (under the plasma concentration-time curve from time 0 to 12 hours post dose (AUC0-12) = 7,187 and 12,992 hr*pg/mL) was linear between the 2 mg and 4 mg doses. Perfusion and digital skin temperature were positively associated with log-transformed plasma concentration at the 4 mg dose (P = 0.015 and P = 0.013, respectively). The most frequent adverse events were similar to those seen with prostacyclin analogues. CONCLUSIONS: Oral treprostinil diethanolamine was effectively absorbed in patients with scleroderma. Drug administration was temporally associated with improved cutaneous perfusion and temperature. Treprostinil diethanolamine may provide a new therapeutic option for Raynaud's phenomenon and the peripheral vascular disease of scleroderma. TRIAL REGISTRATION: ClinicalTrials.gov NCT00848939. FAU - Shah, Ami A AU - Shah AA FAU - Schiopu, Elena AU - Schiopu E FAU - Hummers, Laura K AU - Hummers LK FAU - Wade, Michael AU - Wade M FAU - Phillips, Kristine AU - Phillips K FAU - Anderson, Cynthia AU - Anderson C FAU - Wise, Robert AU - Wise R FAU - Boin, Francesco AU - Boin F FAU - Seibold, James R AU - Seibold JR FAU - Wigley, Fredrick AU - Wigley F FAU - Rollins, Kristan D AU - Rollins KD LA - eng SI - ClinicalTrials.gov/NCT00848939 GR - 1K23AR060241/AR/NIAMS NIH HHS/United States GR - 1K23AR052742/AR/NIAMS NIH HHS/United States GR - UL1 RR 025005/RR/NCRR NIH HHS/United States GR - K23 AR060241/AR/NIAMS NIH HHS/United States GR - K23 AR052742/AR/NIAMS NIH HHS/United States GR - 1K23AR055667/AR/NIAMS NIH HHS/United States GR - 1K23AR061439/AR/NIAMS NIH HHS/United States GR - K23 AR055667/AR/NIAMS NIH HHS/United States GR - 1P50 HL084946-01/HL/NHLBI NIH HHS/United States GR - UL1 RR024986/RR/NCRR NIH HHS/United States GR - UL1 RR 024986/RR/NCRR NIH HHS/United States GR - P50 HL084946/HL/NHLBI NIH HHS/United States GR - UL1 RR025005/RR/NCRR NIH HHS/United States GR - K23 AR061439/AR/NIAMS NIH HHS/United States PT - Clinical Trial, Phase I PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20130418 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Antihypertensive Agents) RN - DCR9Z582X0 (Epoprostenol) RN - RUM6K67ESG (treprostinil) SB - IM MH - Administration, Oral MH - Adult MH - Aged MH - Antihypertensive Agents/administration & dosage/*pharmacokinetics MH - Area Under Curve MH - Epoprostenol/administration & dosage/*analogs & derivatives/pharmacokinetics MH - Female MH - Fingers/blood supply MH - Half-Life MH - Humans MH - Ischemia/*drug therapy/etiology MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications PMC - PMC5011881 EDAT- 2013/04/20 06:00 MHDA- 2015/06/04 06:00 PMCR- 2013/04/18 CRDT- 2013/04/20 06:00 PHST- 2012/10/29 00:00 [received] PHST- 2013/04/18 00:00 [accepted] PHST- 2013/04/20 06:00 [entrez] PHST- 2013/04/20 06:00 [pubmed] PHST- 2015/06/04 06:00 [medline] PHST- 2013/04/18 00:00 [pmc-release] AID - ar4216 [pii] AID - 4361 [pii] AID - 10.1186/ar4216 [doi] PST - epublish SO - Arthritis Res Ther. 2013 Apr 18;15(2):R54. doi: 10.1186/ar4216. PMID- 38326675 OWN - NLM STAT- MEDLINE DCOM- 20240220 LR - 20240220 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 43 IP - 3 DP - 2024 Mar TI - Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study. PG - 1145-1154 LID - 10.1007/s10067-024-06873-z [doi] AB - OBJECTIVES: Myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinctive dermatomyositis (DM) clinical phenotypes. The aim of this study is to explicate the clinical and immunological features of MSAs-negative DM patients. METHODS: A total of 515 individuals diagnosed with DM was screened from 2013 to 2022 and 220 DM patients were enrolled in this retrospective cohort. Clinical and laboratory data of these patients were analyzed. RESULTS: MSAs-negative DM patients were categorized into two groups: MAAs-negative (MSAs (-)/MAAs (-)) group and MAAs-positive (MSAs (-)/MAAs (+)) group. The percentage of Raynaud's phenomenon (P=0.026) was higher in the MSAs (-)/MAAs (+) DM patients than the MSAs-positive DM patients and MSAs (-)/MAAs (-) DM patients. The proportion of rapidly progressive interstitial lung disease (RP-ILD) in the MSAs-negative DM patients was lower than that in the MSAs-positive group. The MSAs (-)/MAAs (+) group had a higher proportion of organizing pneumonia and usual interstitial pneumonia (P=0.011), and elevated eosinophils in their bronchoalveolar lavage fluid (P=0.008). Counts of lymphocytes (P=0.001) and CD16(+)CD56(+) natural killer (NK) cells (P=0.012) were higher in the MSAs-negative group. Additionally, the percentage of CD4(+)TNFα(+) (P=0.040), CD4(+)IFNγ(+) (P=0.037), and CD4(+)IL-2(+) (P=0.018) cells among total CD4(+) T cells were higher in the MSA-negative DM patients compared with the MSAs-positive DM patients. Besides, MSAs-negative patients demonstrated a more favorable prognosis than MSAs-positive patients. Multivariable regression analysis identified advanced onset age, higher level of carcinoembryonic antigen (CEA), and RP-ILD as risk factors for mortality in DM patients. CONCLUSIONS: Compared with MSAs-positive group, MSAs-negative DM patients suffered less from organ involvement compared with MSAs-positive group and tend to have better prognosis. Key Points MSAs-negative DM patients exhibited distinct characteristics in comparison with MSAs-positive DM patients:   • The MSAs (-)/MAAs (+) DM patients demonstrated a higher prevalence of organizing pneumonia (OP) and usual interstitial pneumonia (UIP), and elevated eosinophil counts in bronchoalveolar lavage fluid.   • CEA levels were lower in MSAs-negative patients compared with MSAs-positive patients.   • Elevated counts of lymphocytes and CD16(+)CD56(+) NK cells were identified in the MSAs-negative patients. Additionally, proportions of CD4(+)TNFα(+), CD4(+)IFNγ(+), and CD4(+)IL-2(+) cells among total CD4(+) T cells were higher in the MSAs-negative DM patients compared with DM MSAs-positive DM patients.   • MSAs-negative DM patients had a more favorable prognosis than MSAs-positive DM patients. A multivariable regression analysis revealed the advanced onset age, high CEA levels, and RP-ILD were risk factors for mortality in DM patients. CI - © 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR). FAU - Xing, Xiaoyan AU - Xing X AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Gan, Yuzhou AU - Gan Y AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Mo, Wanxing AU - Mo W AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Zhang, Jian AU - Zhang J AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Wang, Naidi AU - Wang N AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Zhang, Kai AU - Zhang K AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. FAU - Ma, Ke AU - Ma K AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Qingdao, 266111, Shandong Province, China. FAU - Zhang, Lihua AU - Zhang L AD - Department of Rheumatology, Hulunbeier People's Hospital, Hulunbuir, 021008, Inner Mongolia, China. FAU - Ma, Lin AU - Ma L AD - Department of Rheumatology, Hebei Hospital of Traditional Chinese Medicine, Shijiazhuang, 050200, Hebei Province, China. FAU - Lu, Dan AU - Lu D AD - Institute of Systems Biomedicine, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, 100191, China. FAU - Li, Yuhui AU - Li Y AUID- ORCID: 0000-0002-5794-8172 AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. liyuhui84@163.com. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. liyuhui84@163.com. FAU - He, Jing AU - He J AD - Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, 100044, China. hejing1105@126.com. AD - Center of Clinical Immunology, Peking University, Beijing, 100044, China. hejing1105@126.com. LA - eng PT - Journal Article DEP - 20240207 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Autoantibodies) RN - 0 (Carcinoembryonic Antigen) RN - 0 (Interleukin-2) RN - 0 (Tumor Necrosis Factor-alpha) SB - IM MH - Humans MH - *Dermatomyositis MH - Autoantibodies MH - Carcinoembryonic Antigen MH - Case-Control Studies MH - Retrospective Studies MH - Interleukin-2 MH - Tumor Necrosis Factor-alpha MH - *Lung Diseases, Interstitial/etiology MH - *Myositis MH - Prognosis MH - *Idiopathic Pulmonary Fibrosis/complications MH - *Organizing Pneumonia OTO - NOTNLM OT - Clinical characteristics OT - Immune cell subsets OT - Myositis autoantibodies OT - Prognosis EDAT- 2024/02/08 00:41 MHDA- 2024/02/20 11:50 CRDT- 2024/02/07 23:31 PHST- 2023/08/09 00:00 [received] PHST- 2024/01/13 00:00 [accepted] PHST- 2023/11/21 00:00 [revised] PHST- 2024/02/20 11:50 [medline] PHST- 2024/02/08 00:41 [pubmed] PHST- 2024/02/07 23:31 [entrez] AID - 10.1007/s10067-024-06873-z [pii] AID - 10.1007/s10067-024-06873-z [doi] PST - ppublish SO - Clin Rheumatol. 2024 Mar;43(3):1145-1154. doi: 10.1007/s10067-024-06873-z. Epub 2024 Feb 7. PMID- 39152753 OWN - NLM STAT- MEDLINE DCOM- 20240817 LR - 20250204 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 42 IP - 8 DP - 2024 Aug TI - Clinical and subclinical atherosclerosis in patients with systemic sclerosis: an observational, multicentre study of GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale). PG - 1645-1655 LID - 10.55563/clinexprheumatol/zr8j5p [doi] AB - OBJECTIVES: Conflicting results about clinical and subclinical atherosclerosis in systemic sclerosis (SSc) and the associated risk factors have been reported. Hence, we aimed to determine the prevalence of clinical and subclinical atherosclerosis in a large number of Italian SSc patients and the associated risk factors. METHODS: This study included 613 SSc patients from 11 Italian tertiary Rheumatologic Units. All patients underwent full history taking, clinical examination, and relevant laboratory and radiological investigations. Doppler ultrasonography (US) of the common carotid and upper and lower limbs was performed to measure carotid and femoral intima-media thickness (cIMT and fIMT), and carotid and peripheral atheroma plaques. Doppler US of the brachial artery was performed to measure flow-mediated dilatation (FMD). RESULTS: Patients were mostly women (91.4%) with a median age of 61 years (range, 20-100); a median disease duration of 14 years (range, 0-77) from the onset of the first non-Raynaud's phenomenon (RP); 9.3% had a history of clinical atherosclerosis (9 stable/unstable angina, 21 myocardial infarctions, 24 heart failure, 3 strokes, 8 transient ischaemic attack, 6 intermittent claudication, 10 atrial thrombo-embolism). In 37.1% of patients, subclinical atherosclerosis was detected, after excluding those with a history of clinical atherosclerosis. The prevalence of clinical and subclinical atherosclerosis was higher than that reported by the European Society of Cardiology and observational studies that enrolled Italian healthy individuals as a control group, respectively. CONCLUSIONS: A higher prevalence of clinical and subclinical atherosclerosis was detected in SSc Italian patients and correlated with traditional and SSc-related risk factors. FAU - Liakouli, Vasiliki AU - Liakouli V AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. vasiliki.liakouli@unicampania.it, vasiliki_liakouli@yahoo.it. FAU - Verde, Ignazio AU - Verde I AD - Unit of General, Mininvasive, Oncological and Bariatric Surgery Teaching Hospital, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Ruscitti, Piero AU - Ruscitti P AD - Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, Italy. FAU - Di Vico, Claudio AU - Di Vico C AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Ruggiero, Annarita AU - Ruggiero A AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Mauro, Daniele AU - Mauro D AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Forte, Giulio AU - Forte G AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Navarini, Luca AU - Navarini L AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Di Donato, Stefano AU - Di Donato S AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Bearzi, Pietro AU - Bearzi P AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Minerba, Marco AU - Minerba M AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Bertolini, Nicoletta AU - Bertolini N AD - Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II of Naples, Italy. FAU - Favoino, Elvira AU - Favoino E AD - Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM), University of Bari Medical School, Bari, Italy. FAU - Destro Castaniti, Giulia Maria AU - Destro Castaniti GM AD - Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Rheumatology section, University of Palermo, Italy. FAU - D'Alessandro, Roberto AU - D'Alessandro R AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy. FAU - Berlengiero, Virginia AU - Berlengiero V AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy. FAU - Italiano, Noemi AU - Italiano N AD - Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, Italy. FAU - Bellisai, Francesca AU - Bellisai F AD - Rheumatology Unit, Azienda Ospedaliero Universitaria Senese, Siena, Italy. FAU - Caso, Francesco AU - Caso F AD - Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II of Naples, Italy. FAU - Guggino, Giuliana AU - Guggino G AD - Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Rheumatology section, University of Palermo, Italy. FAU - Corrado, Ada AU - Corrado A AD - Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Italy. FAU - Triggianese, Paola AU - Triggianese P AD - Rheumatology, Allergology and Clinical Immunology, Department of Medicina dei Sistemi, University of Rome Tor Vergata, Rome, Italy. FAU - Lo Gullo, Alberto AU - Lo Gullo A AD - Rheumatology Unit, Department of Medicine, ARNAS Garibaldi Hospital, Catania, Italy. FAU - Mandraffino, Giuseppe AU - Mandraffino G AD - Internal Medicine Unit, University Hospital G. Martino, University of Messina, Italy. FAU - Cantarini, Luca AU - Cantarini L AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy. FAU - Cipriani, Paola AU - Cipriani P AD - Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, Italy. FAU - Cantatore, Francesco Paolo AU - Cantatore FP AD - Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Italy. FAU - Chimenti, Maria Sole AU - Chimenti MS AD - Rheumatology, Allergology and Clinical Immunology, Department of Medicina dei Sistemi, University of Rome Tor Vergata, Rome, Italy. FAU - Perosa, Federico AU - Perosa F AD - Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM), University of Bari Medical School, Bari, Italy. FAU - Iagnocco, Annamaria AU - Iagnocco A AD - Academic Rheumatology Centre, Hospital Mauriziano, Department of Clinical and Biological Science, University of Turin, Italy. FAU - Docimo, Ludovico AU - Docimo L AD - Unit of General, Mininvasive, Oncological and Bariatric Surgery Teaching Hospital, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Giacomelli, Roberto AU - Giacomelli R AD - Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Biomedico, Rome; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome Campus Biomedico, School of Medicine, Rome, Italy. FAU - Ciccia, Francesco AU - Ciccia F AD - Rheumatology Unit, Department of Precision Medicine, University of Campania Luigi Vanvitelli, Naples, Italy. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20240814 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Female MH - *Scleroderma, Systemic/epidemiology/complications MH - Male MH - Middle Aged MH - Italy/epidemiology MH - Aged MH - Adult MH - Prevalence MH - *Atherosclerosis/epidemiology/diagnostic imaging/etiology MH - *Carotid Intima-Media Thickness MH - Risk Factors MH - Aged, 80 and over MH - Young Adult MH - Ultrasonography, Doppler MH - Asymptomatic Diseases MH - Carotid Artery Diseases/epidemiology/diagnostic imaging MH - Plaque, Atherosclerotic/diagnostic imaging/epidemiology EDAT- 2024/08/17 15:43 MHDA- 2024/08/17 15:44 CRDT- 2024/08/17 05:33 PHST- 2024/03/19 00:00 [received] PHST- 2024/06/24 00:00 [accepted] PHST- 2024/08/17 15:44 [medline] PHST- 2024/08/17 15:43 [pubmed] PHST- 2024/08/17 05:33 [entrez] AID - 21079 [pii] AID - 10.55563/clinexprheumatol/zr8j5p [doi] PST - ppublish SO - Clin Exp Rheumatol. 2024 Aug;42(8):1645-1655. doi: 10.55563/clinexprheumatol/zr8j5p. Epub 2024 Aug 14. PMID- 31888720 OWN - NLM STAT- MEDLINE DCOM- 20201020 LR - 20260314 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 21 IP - 1 DP - 2019 Dec 30 TI - Rule-based and machine learning algorithms identify patients with systemic sclerosis accurately in the electronic health record. PG - 305 LID - 10.1186/s13075-019-2092-7 [doi] LID - 305 AB - BACKGROUND: Systemic sclerosis (SSc) is a rare disease with studies limited by small sample sizes. Electronic health records (EHRs) represent a powerful tool to study patients with rare diseases such as SSc, but validated methods are needed. We developed and validated EHR-based algorithms that incorporate billing codes and clinical data to identify SSc patients in the EHR. METHODS: We used a de-identified EHR with over 3 million subjects and identified 1899 potential SSc subjects with at least 1 count of the SSc ICD-9 (710.1) or ICD-10-CM (M34*) codes. We randomly selected 200 as a training set for chart review. A subject was a case if diagnosed with SSc by a rheumatologist, dermatologist, or pulmonologist. We selected the following algorithm components based on clinical knowledge and available data: SSc ICD-9 and ICD-10-CM codes, positive antinuclear antibody (ANA) (titer ≥ 1:80), and a keyword of Raynaud's phenomenon (RP). We performed both rule-based and machine learning techniques for algorithm development. Positive predictive values (PPVs), sensitivities, and F-scores (which account for PPVs and sensitivities) were calculated for the algorithms. RESULTS: PPVs were low for algorithms using only 1 count of the SSc ICD-9 code. As code counts increased, the PPVs increased. PPVs were higher for algorithms using ICD-10-CM codes versus the ICD-9 code. Adding a positive ANA and RP keyword increased the PPVs of algorithms only using ICD billing codes. Algorithms using ≥ 3 or ≥ 4 counts of the SSc ICD-9 or ICD-10-CM codes and ANA positivity had the highest PPV at 100% but a low sensitivity at 50%. The algorithm with the highest F-score of 91% was ≥ 4 counts of the ICD-9 or ICD-10-CM codes with an internally validated PPV of 90%. A machine learning method using random forests yielded an algorithm with a PPV of 84%, sensitivity of 92%, and F-score of 88%. The most important feature was RP keyword. CONCLUSIONS: Algorithms using only ICD-9 codes did not perform well to identify SSc patients. The highest performing algorithms incorporated clinical data with billing codes. EHR-based algorithms can identify SSc patients across a healthcare system, enabling researchers to examine important outcomes. FAU - Jamian, Lia AU - Jamian L AD - Hartford HealthCare Medical Group, Hartford, CT, USA. FAU - Wheless, Lee AU - Wheless L AD - Department of Dermatology, Vanderbilt University Medical Center, Nashville, TN, USA. AD - Data Science Institute, Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Crofford, Leslie J AU - Crofford LJ AD - Department of Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South T3113 MCN, Nashville, TN, 37232, USA. FAU - Barnado, April AU - Barnado A AUID- ORCID: 0000-0002-9573-4335 AD - Department of Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South T3113 MCN, Nashville, TN, 37232, USA. april.barnado@vumc.org. LA - eng GR - NICHD 5K12HD043483-12/National Institute of Child Health and Human Development/International GR - K08 AR072757/AR/NIAMS NIH HHS/United States GR - K12 HD043483/HD/NICHD NIH HHS/United States GR - 1 K08AR072757-01/AR/NIAMS NIH HHS/United States GR - ULTR000445/TR/NCATS NIH HHS/United States GR - UL1 RR024975/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20191230 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - *Algorithms MH - Databases, Factual/standards/statistics & numerical data MH - Electronic Health Records/*standards/statistics & numerical data MH - Female MH - Humans MH - International Classification of Diseases/*standards MH - *Machine Learning MH - Male MH - Middle Aged MH - Reproducibility of Results MH - Scleroderma, Systemic/*diagnosis MH - Sensitivity and Specificity PMC - PMC6937803 OTO - NOTNLM OT - Algorithms OT - Bioinformatics OT - Electronic health records OT - Systemic sclerosis COIS- The authors declare that they have no competing interests. EDAT- 2020/01/01 06:00 MHDA- 2020/10/21 06:00 PMCR- 2019/12/30 CRDT- 2020/01/01 06:00 PHST- 2019/08/17 00:00 [received] PHST- 2019/12/18 00:00 [accepted] PHST- 2020/01/01 06:00 [entrez] PHST- 2020/01/01 06:00 [pubmed] PHST- 2020/10/21 06:00 [medline] PHST- 2019/12/30 00:00 [pmc-release] AID - 10.1186/s13075-019-2092-7 [pii] AID - 2092 [pii] AID - 10.1186/s13075-019-2092-7 [doi] PST - epublish SO - Arthritis Res Ther. 2019 Dec 30;21(1):305. doi: 10.1186/s13075-019-2092-7. PMID- 20661740 OWN - NLM STAT- MEDLINE DCOM- 20120702 LR - 20220311 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 32 IP - 1 DP - 2012 Jan TI - Serologic response to Epstein-Barr virus antigens in patients with systemic lupus erythematosus: a controlled study. PG - 79-83 LID - 10.1007/s00296-010-1573-4 [doi] AB - Previous studies showed a link between systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection. We sought to determine the features of serologic response to EBV in SLE patients and whether this response differs from those of systemic sclerosis (SSc) and primary antiphospholipid syndrome (PAPS) patients as well as healthy individuals. Sera from 198 consecutive SLE patients have been tested to detect IgG antibodies to EA/D, EBNA-1, VCA P18 and for comparison, cytomegalovirus (CMV) using commercially available ELISA kits (Trinity Biotech, USA). Forty-six SSc patients and 38 PAPS patients were enrolled as diseased control groups and sixty-five individuals as healthy controls. Significantly more SLE (54%, P = 0.001, OR 5.77, 95% CI 2.8-11.6), SSc (41.3%, P = 0.005, OR 3.4, 95% CI 1.4-8.2) and PAPS sera (36.8%, P = 0.023, OR 2.86, 95% CI 1.14-7.22) reacted against EA/D than healthy controls (16.9%). The mean age of anti-EA/D-positive SLE patients was significantly higher, and their disease duration was longer compared to anti-EA/D-negative SLE patients (41 ± 14 vs. 33.8 ± 10.8 years, P < 0.001 and 100 ± 73 vs. 71 ± 62 months, P = 0.003). In SLE patients, EA/D reactivity was associated with Raynaud's phenomenon and the presence of any anti-ENA antibodies. Although it did not reach a statistical significance, anti-EBNA-1 reactivity was slightly lower in patients with SLE. The frequency of anti-CMV Ig G positivity was found significantly higher in SLE patients (100%) when compared to patients with SSc (95.7%), PAPS (94.7%) and healthy controls (95.4%) (P = 0.035, P = 0.025 and P = 0.015 respectively). Our results support the proposed link between EBV and SLE. The finding that SSc and PAPS patients also have increased frequency of anti-EA/D response has revealed that this immune interaction may not be unique to patients with SLE, and there may be a common mechanism involving EBV in these autoimmune diseases. FAU - Esen, Bahar Artım AU - Esen BA AD - Rheumatology Division, Internal Medicine Department, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. bahartimesen@gmail.com FAU - Yılmaz, Gülden AU - Yılmaz G FAU - Uzun, Sami AU - Uzun S FAU - Ozdamar, Melda AU - Ozdamar M FAU - Aksözek, Alper AU - Aksözek A FAU - Kamalı, Sevil AU - Kamalı S FAU - Türkoğlu, Salih AU - Türkoğlu S FAU - Gül, Ahmet AU - Gül A FAU - Ocal, Lale AU - Ocal L FAU - Aral, Orhan AU - Aral O FAU - Inanç, Murat AU - Inanç M LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100727 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Viral) RN - 0 (Antigens, Viral) RN - 0 (Capsid Proteins) RN - 0 (Epstein-Barr Virus Nuclear Antigens) RN - 0 (Epstein-Barr viral capsid antigen) RN - 0 (Epstein-Barr virus early antigen) RN - O5GA75RST7 (EBV-encoded nuclear antigen 1) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antibodies, Viral/*blood MH - Antigens, Viral/*immunology MH - Antiphospholipid Syndrome/blood/*immunology MH - Capsid Proteins/immunology MH - Case-Control Studies MH - Cytomegalovirus/immunology MH - Epstein-Barr Virus Nuclear Antigens/immunology MH - Female MH - Herpesvirus 4, Human/*immunology MH - Humans MH - Lupus Erythematosus, Systemic/blood/*immunology MH - Male MH - Middle Aged MH - Scleroderma, Systemic/blood/*immunology MH - Seroepidemiologic Studies MH - Young Adult EDAT- 2010/07/28 06:00 MHDA- 2012/07/03 06:00 CRDT- 2010/07/28 06:00 PHST- 2010/03/04 00:00 [received] PHST- 2010/07/11 00:00 [accepted] PHST- 2010/07/28 06:00 [entrez] PHST- 2010/07/28 06:00 [pubmed] PHST- 2012/07/03 06:00 [medline] AID - 10.1007/s00296-010-1573-4 [doi] PST - ppublish SO - Rheumatol Int. 2012 Jan;32(1):79-83. doi: 10.1007/s00296-010-1573-4. Epub 2010 Jul 27. PMID- 40802523 OWN - NLM STAT- MEDLINE DCOM- 20251202 LR - 20260502 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 12 DP - 2025 Dec 1 TI - Anxiety symptoms and associated factors in the Scleroderma Patient-centered Intervention Network (SPIN) cohort: a cross-sectional study. PG - 6268-6277 LID - 10.1093/rheumatology/keaf374 [doi] AB - OBJECTIVE: We (1) compared anxiety symptom levels in a multinational SSc cohort to a general population normative sample and (2) evaluated sociodemographic, lifestyle and SSc disease factors associated with symptoms. METHODS: Scleroderma Patient-centered Intervention Network Cohort participants completed the Patient-Reported Outcomes Measurement Information System (PROMIS) Version 2 4a Anxiety domain upon enrolment. PROMIS domain scores use T-scores (mean = 50, S.D. = 10) calibrated to a United States normative sample. We compared T-scores to the PROMIS United States normative sample and, in SSc, assessed associations of sociodemographic, lifestyle and physician-reported disease-related variables with multivariable linear regression. RESULTS: Among 2463 participants with SSc, mean anxiety symptom T-score (52.6, S.D. = 10.0, 95% CI 52.2, 53.0) was ∼1/3 S.D. higher than the United States general population mean of 50 (S.D. = 10), though within normal limits. Higher T-scores were associated with younger age (1.07 T-score points per 10 years, 95% CI 0.74, 1.40), female sex (1.81, 95% CI 0.63, 3.00), non-married status (0.99, 95% CI 0.14, 1.84), race or ethnicity other than White (1.79, 95% CI 0.72, 2.85), living in Canada (1.70, 95% CI 0.61, 2.79), the United Kingdom (1.53, 95% CI 0.06, 2.99) or France (2.01, 95% CI 0.98, 3.03) (vs the United States), higher BMI (0.11, 95% CI 0.03, 0.17), less time since non-Raynaud's symptom onset (0.82 per 10 years, 95% CI 0.40, 1.30), gastrointestinal involvement (2.70, 95% CI 1.52, 3.88), moderate small joint contractures (1.24, 95% CI 0.10, 2.38), the absence of interstitial lung disease (0.93, 95% CI -1.79, -0.07) and Sjögren disease (1.67, 95% CI 0.17, 3.17). Interstitial lung disease was not statistically significant when accounting for an interaction with country. Anxiety was also associated with pruritus and pain intensity in a sensitivity analysis that included variables with possible bi-directional pathways with anxiety. CONCLUSION: Anxiety symptoms were somewhat elevated among individuals with SSc and associated with multiple sociodemographic and disease factors. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Provencher, Sabrina AU - Provencher S AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychiatry, McGill University, Montreal, QC, Canada. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Virgili-Gervais, Gabrielle AU - Virgili-Gervais G AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Golberg, Meira AU - Golberg M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Henry, Richard S AU - Henry RS AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychiatry, McGill University, Montreal, QC, Canada. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, The Netherlands. AD - Department of IQ Healthcare, Radboud University Medical Center, Nijmegen, The Netherlands. AD - Centre for Mindfulness, Department of Psychiatry, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Fortuné, Catherine AU - Fortuné C AD - Ottawa Scleroderma Support Group, Ottawa, ON, Canada. FAU - Gietzen, Amy AU - Gietzen A AD - National Scleroderma Foundation, Tri-State Chapter, Buffalo, NY, USA. FAU - Gottesman, Karen AU - Gottesman K AD - National Scleroderma Foundation, Los Angeles, CA, USA. FAU - Guillot, Geneviève AU - Guillot G AD - Sclérodermie Québec, Longueuil, QC, Canada. FAU - Lawrie-Jones, Amanda AU - Lawrie-Jones A AD - Scleroderma Australia and Scleroderma Victoria, Melbourne, Victoria, Australia. FAU - Sauvé, Maureen AU - Sauvé M AD - Scleroderma Society of Ontario and Scleroderma Canada, Hamilton, ON, Canada. FAU - Bartlett, Susan J AU - Bartlett SJ AUID- ORCID: 0000-0001-9755-2490 AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Research Institute of the McGill University Health Centre, Montreal, QC, Canada. FAU - Hummers, Laura K AU - Hummers LK AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Malcarne, Vanessa AU - Malcarne V AD - Department of Psychology, San Diego State University, San Diego, CA, USA. AD - San Diego Joint Doctoral Program in Clinical Psychology, San Diego State University and University of California, San Diego, CA, USA. FAU - Mayes, Maureen D AU - Mayes MD AD - Division of Rheumatology, University of Texas McGovern School of Medicine, Houston, TX, USA. FAU - Richard, Michelle AU - Richard M AD - Scleroderma Atlantic, Halifax, NS, Canada. FAU - Stempel, James AU - Stempel J AD - Scleroderma Chicago, Chicago, IL, USA. FAU - Wojeck, Robyn K AU - Wojeck RK AD - Amgen Inc, Thousand Oaks, CA, USA. FAU - Mouthon, Luc AU - Mouthon L AUID- ORCID: 0000-0002-9838-246X AD - Service de Médecine Interne, Centre de Référence Maladies Autoimmunes et Autoinflammatoires Systémiques Rares d'Ile de France, de l'Est et de l'Ouest, Hôpital Cochin, Paris, France. AD - Assistance Publique Hôpitaux de Paris-Centre, Hôpital Cochin, Université Paris Cité, Paris, France. FAU - Benedetti, Andrea AU - Benedetti A AUID- ORCID: 0000-0002-8314-9497 AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada. AD - Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, QC, Canada. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. AD - Department of Psychiatry, McGill University, Montreal, QC, Canada. AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada. AD - Department of Psychology, McGill University, Montreal, QC, Canada. CN - Scleroderma Patient-centered Intervention Network Investigators LA - eng GR - The Scleroderma Patient-centered Intervention Network/ GR - CIHR/Canada GR - Arthritis Society/ GR - Lady Davis Institute for Medical Research/ GR - Jewish General Hospital, Montreal, Quebec/ GR - Jewish General Hospital Foundation/ GR - McGill University/ GR - Scleroderma Society of Ontario/ GR - Scleroderma Canada; Sclérodermie Québec/ GR - Scleroderma Manitoba/ GR - Scleroderma Atlantic/ GR - Scleroderma Association of BC/ GR - Scleroderma SASK/ GR - Scleroderma New South Wales/ GR - Scleroderma Victoria/ GR - Scleroderma Foundation of California/ GR - Fonds de Recherche du Québec-Santé/ GR - Master's Research Award/ GR - Tier 1 Canada Research Chair/ PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Female MH - *Scleroderma, Systemic/psychology/complications MH - Male MH - Cross-Sectional Studies MH - Middle Aged MH - *Anxiety/etiology/epidemiology/psychology MH - Adult MH - Patient Reported Outcome Measures MH - United States/epidemiology MH - Aged MH - Cohort Studies MH - Life Style MH - Patient-Centered Care PMC - PMC12671864 OTO - NOTNLM OT - PROMIS-29 OT - SSc OT - anxiety OT - scleroderma EDAT- 2025/08/13 18:27 MHDA- 2025/12/02 19:16 PMCR- 2025/08/13 CRDT- 2025/08/13 12:42 PHST- 2025/01/25 00:00 [received] PHST- 2025/06/27 00:00 [accepted] PHST- 2025/12/02 19:16 [medline] PHST- 2025/08/13 18:27 [pubmed] PHST- 2025/08/13 12:42 [entrez] PHST- 2025/08/13 00:00 [pmc-release] AID - 8233714 [pii] AID - keaf374 [pii] AID - 10.1093/rheumatology/keaf374 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Dec 1;64(12):6268-6277. doi: 10.1093/rheumatology/keaf374. PMID- 38251485 OWN - NLM STAT- MEDLINE DCOM- 20240123 LR - 20251209 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 5 IP - 10 DP - 2023 Oct TI - MRI Digital Artery Volume Index (DAVIX) as a surrogate outcome measure of digital ulcer disease in patients with systemic sclerosis: a prospective cohort study. PG - e611-e621 LID - S2665-9913(23)00189-3 [pii] LID - 10.1016/S2665-9913(23)00189-3 [doi] AB - BACKGROUND: Vascular fibrosis is a key manifestation of systemic sclerosis that leads to the narrowing of small and medium arteries, causing vascular clinical manifestations including digital ulcers and pulmonary arterial hypertension. We investigated the potential of the MRI-based Digital Artery Volume Index (DAVIX) as a surrogate outcome measure of vascular fibrosis by using it to quantify and predict the burden of digital ulcer disease in patients with systemic sclerosis. METHODS: Two independent cohorts of patients participating in the prospective observational study STRIKE were consecutively enrolled from the Scleroderma Clinic of the Leeds Teaching Hospitals Trust, Leeds, UK. Eligible patients were aged 18 years or older and fulfilled the very early diagnosis of systemic sclerosis (VEDOSS) or the 2013 American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) systemic sclerosis classification criteria. DAVIX was calculated as the percentage mean of the ratio of digital artery volume to finger volume in the four fingers of the dominant hand. Data were collected at baseline and 12-month follow-up, and the primary outcome was the presence of digital ulcers at 12-month follow-up. FINDINGS: Between Feb 7, 2018, and April 11, 2022, we included 85 patients in the exploratory cohort and 150 in the validation cohort. In the exploratory cohort, the mean age was 54·5 years (SD 11·6), 75 (88%) of 85 patients were women, ten (12%) were men, and 69 (82%) were White. In the validation cohort, the mean age was 53·5 years (SD 13·8), 136 (91%) of 150 patients were women, 14 (9%) were men, and 127 (85%) were White. In the exploratory cohort, DAVIX was significantly lower in patients with previous or active digital ulcers (0·34% [IQR 0·16-0·69]) than in those without digital ulcer disease (0·65% [0·42-0·88]; p=0·015); this finding was substantiated in the validation cohort (0·43% [0·20-0·73] vs 0·73% [0·53-0·97]; p<0·0001). Patients who developed new digital ulcers during 12-month follow-up had a lower DAVIX (0·23% [0·10-0·66]) than those who did not (0·65% [0·45-0·91]; p=0·0039). DAVIX was negatively correlated with disease duration (r=-0·415; p<0·0001), the ratio of forced vital capacity to the diffusing capacity of the lungs for carbon monoxide (r=-0·334; p=0·0091), nailfold capillaroscopy pattern (r=-0·447; p<0·0001), and baseline modified Rodnan skin score (r=-0·305; p=0·014) and was positively correlated with the diffusing capacity of carbon monoxide (r=0·368; p=0·0041). DAVIX was negatively correlated with change in score on the Scleroderma Health Assessment Questionnaire-Disability Index (r=-0·308; p=0·024), Visual Analogue Scale (VAS) Raynaud's (r=-0·271; p=0·044), and VAS digital ulcers (r=-0·291; p=0·044). INTERPRETATION: DAVIX is a promising surrogate outcome measure of digital ulcer disease in patients with systemic sclerosis. The ability of DAVIX to non-invasively predict future digital ulcers and worsening of patient-reported outcomes could aid patient enrichment and stratification in clinical trials. Clinically, DAVIX could offer insights into the assessment of vascular activity. The sensitivity of DAVIX to change over time and with treatment will establish its value as an imaging outcome measure of vascular disease. FUNDING: National Institute for Health Research Biomedical Research Centre and University of Leeds Industry Engagement Accelerator Fund. CI - Copyright © 2023 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY-NC-ND 4.0 license. Published by Elsevier Ltd.. All rights reserved. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, UK; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. FAU - Di Donato, Stefano AU - Di Donato S AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Gjeloshi, Klodian AU - Gjeloshi K AD - Department of Advanced Medical and Surgical Sciences, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - Istituto Reumatologico Lucano, Azienda Ospedaliera Regionale San Carlo, Potenza, Italy. FAU - Danzo, Fiammetta AU - Danzo F AD - Division of Respiratory Diseases, Ospedale L Sacco, ASST Fatebenefratelli-Sacco, Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. FAU - Lettieri, Giovanni AU - Lettieri G AD - Istituto Reumatologico Lucano, Azienda Ospedaliera Regionale San Carlo, Potenza, Italy. FAU - De Lorenzis, Enrico AU - De Lorenzis E AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A Gemelli IRCCS, Rome, Italy. FAU - Bertham, Dominic AU - Bertham D AD - NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - O'Connor, Philip AU - O'Connor P AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Kubassova, Olga AU - Kubassova O AD - Image Analysis Group, London, UK. FAU - Dehmeshki, Jamshid AU - Dehmeshki J AD - Image Analysis Group, London, UK; Department of Computer Science, Kingston University, London, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK. Electronic address: f.delgaldo@leeds.ac.uk. LA - eng PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't DEP - 20230925 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 RN - 7U1EE4V452 (Carbon Monoxide) RN - digital ulcers SB - IM CIN - Lancet Rheumatol. 2023 Oct;5(10):e569-e570. doi: 10.1016/S2665-9913(23)00239-4. PMID: 38251477 MH - Male MH - Humans MH - Female MH - Middle Aged MH - Carbon Monoxide MH - Prospective Studies MH - *Scleroderma, Systemic/complications MH - Ulnar Artery MH - Magnetic Resonance Imaging MH - *Scleroderma, Localized MH - Outcome Assessment, Health Care MH - Fibrosis MH - *Skin Ulcer COIS- Declaration of interests MH reports speaking fees from Actelion Pharmaceuticals, Eli Lilly, and Pfizer, outside of the submitted work and he is a member of a data and safety monitoring board for Certa Therapeutics. OK and JD are employees and shareholders of Image Analysis Group and co-developers of DAVIX. OK reports speaking fees from Takeda Pharmaceuticals and is on scientific advisory boards for Werfen and MiMedx. FDG reports consulting fees from Argenx, Arxx Therapeutics, AstraZeneca, Ergomed, GSK, Image Analysis Group, Janssen Pharmaceuticals, and Novartis and research support from AbbVie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab Therapeutics, and Mitsubishi Tanabe Pharma. All other authors declare no competing interests. EDAT- 2024/01/22 12:43 MHDA- 2024/01/23 06:43 CRDT- 2024/01/22 07:23 PHST- 2022/10/07 00:00 [received] PHST- 2023/06/05 00:00 [revised] PHST- 2023/07/05 00:00 [accepted] PHST- 2024/01/23 06:43 [medline] PHST- 2024/01/22 12:43 [pubmed] PHST- 2024/01/22 07:23 [entrez] AID - S2665-9913(23)00189-3 [pii] AID - 10.1016/S2665-9913(23)00189-3 [doi] PST - ppublish SO - Lancet Rheumatol. 2023 Oct;5(10):e611-e621. doi: 10.1016/S2665-9913(23)00189-3. Epub 2023 Sep 25. PMID- 28770709 OWN - NLM STAT- MEDLINE DCOM- 20180424 LR - 20260127 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 36 IP - 1 DP - 2018 Jan-Feb TI - Timing of onset affects arthritis presentation pattern in antisyntethase syndrome. PG - 44-49 AB - OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility. FAU - González-Gay, Miguel A AU - González-Gay MA AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AD - Internal Medicine Department, Vall d'Hebron General Hospital, Barcelona, Spain. FAU - Trallero-Araguas, Ernesto AU - Trallero-Araguas E AD - Internal Medicine Department, Vall d'Hebron General Hospital, Barcelona, Spain. FAU - Molberg, Ovynd AU - Molberg O AD - Department of Rheumatology, Oslo University Hospital (OUH), Oslo, Norway. FAU - Andersson, Helena AU - Andersson H AD - Department of Rheumatology, Oslo University Hospital (OUH), Oslo, Norway. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AD - Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México. FAU - Perez-Roman, Diana Isabel AU - Perez-Roman DI AD - Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México. FAU - Bauhammer, Jutta AU - Bauhammer J AD - ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany. FAU - Fiehn, Christoph AU - Fiehn C AD - ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany. FAU - Neri, Rossella AU - Neri R AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Italy. FAU - Lorenz, Hannes M AU - Lorenz HM AD - Department of Internal Medicine V, Division of Rheumatology, University of Heidelberg, Germany. FAU - Doria, Andrea AU - Doria A AD - Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Italy. FAU - Ghirardello, Anna AU - Ghirardello A AD - Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Italy. FAU - Giannini, Margherita AU - Giannini M AD - Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. FAU - Triantafyllias, Konstantinos AU - Triantafyllias K AD - ACURA Rheumatology Center, Bad Kreuznach, Germany. FAU - Benucci, Maurizio AU - Benucci M AD - Rheumatology Unit, Azienda Sanitaria di Firenze, S. Giovanni di Dio Hospital, Florence, Italy. FAU - Infantino, Maria AU - Infantino M AD - Immunology and Allergy Laboratory, S.Giovanni di Dio Hospital, Florence, Italy. FAU - Manfredi, Mariangela AU - Manfredi M AD - Immunology and Allergy Laboratory, S.Giovanni di Dio Hospital, Florence, Italy. FAU - Conti, Fabrizio AU - Conti F AD - Department of Internal Medicine and Medical Specialties-Rheumatology, Sapienza University of Rome, Italy. FAU - Schwarting, Andreas AU - Schwarting A AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. FAU - Sebastiani, Giandomenico AU - Sebastiani G AD - U.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Roma, Italy. FAU - Iuliano, Annamaria AU - Iuliano A AD - U.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Roma, Italy. FAU - Emmi, Giacomo AU - Emmi G AD - Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Silvestri, Elena AU - Silvestri E AD - Department of Experimental and Clinical Medicine, University of Florence, Italy. FAU - Govoni, Marcello AU - Govoni M AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy. FAU - Scirè, Carlo Alberto AU - Scirè CA AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy. FAU - Furini, Federica AU - Furini F AD - UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy. FAU - Lopez-Longo, Francisco Javier AU - Lopez-Longo FJ AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Martínez-Barrio, Julia AU - Martínez-Barrio J AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. FAU - Sebastiani, Marco AU - Sebastiani M AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Italy. FAU - Manfredi, Andreina AU - Manfredi A AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Italy. FAU - Bachiller-Corral, Javier AU - Bachiller-Corral J AD - Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. FAU - Sifuentes Giraldo, Walter Alberto AU - Sifuentes Giraldo WA AD - Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. FAU - Cimmino, Marco A AU - Cimmino MA AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy. FAU - Cosso, Claudio AU - Cosso C AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy. FAU - Belotti Masserini, Alessandro AU - Belotti Masserini A AD - ASST Bergamo Ovest, UOC Medicina, Ospedale di Treviglio, Italy. FAU - Cagnotto, Giovanni AU - Cagnotto G AD - Lund University, Skane University Hospital, Department of Clinical Sciences, Rheumatology, Lund, Sweden. FAU - Codullo, Veronica AU - Codullo V AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Romano, Mariaeva AU - Romano M AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. FAU - Paolazzi, Giuseppe AU - Paolazzi G AD - Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. FAU - Pellerito, Raffaele AU - Pellerito R AD - Division of Rheumatology, Mauriziano Hospital , Turin, Italy. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - Tulane University Lung Center Tulane, UMC Scleroderma and Sarcoidosis Patient Care and Research Center New Orleans, LA, USA. FAU - Ortego-Centeno, Norberto AU - Ortego-Centeno N AD - Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. FAU - Quartuccio, Luca AU - Quartuccio L AD - Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, Udine, Italy. FAU - Batticciotto, Alberto AU - Batticciotto A AD - Rheumatology Unit, Luigi Sacco University Hospital, Milan, Italy. FAU - Bartoloni Bocci, Elena AU - Bartoloni Bocci E AD - Rheumatology Unit, Department of Medicine, University of Perugia, Italy. FAU - Gerli, Roberto AU - Gerli R AD - Rheumatology Unit, Department of Medicine, University of Perugia, Italy. FAU - Specker, Christof AU - Specker C AD - Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. FAU - Bravi, Elena AU - Bravi E AD - Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. FAU - Selmi, Carlo AU - Selmi C AD - Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy. FAU - Parisi, Simone AU - Parisi S AD - Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. FAU - Salaffi, Fausto AU - Salaffi F AD - Rheumatology Department, Polytechnic University of Marche, C. Urbani Hospital, Jesi, Ancona, Italy. FAU - Meloni, Federica AU - Meloni F AD - Pneumology Unit, Cardiothoracic and Vascular Department, University and IRCCS Policlinico San Matteo Foundation, Pavia, Italy. FAU - Marchioni, Enrico AU - Marchioni E AD - C. Mondino National Neurological Institute, Pavia, Italy. FAU - Pesci, Alberto AU - Pesci A AD - School of Medicine and Surgery, University of Milan-Bicocca, Respiratory Unit, ASST Monza, Italy. FAU - Dei, Giulia AU - Dei G AD - School of Medicine and Surgery, University of Milan-Bicocca, Respiratory Unit, ASST Monza, Italy. FAU - Confalonieri, Marco AU - Confalonieri M AD - Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste, Italy. FAU - Tomietto, Paola AU - Tomietto P AD - Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Italy. FAU - Nuno, Laura AU - Nuno L AD - Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. FAU - Bonella, Francesco AU - Bonella F AD - Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany. FAU - Pipitone, Nicolò AU - Pipitone N AD - Rheumatology Unit, Department of Internal Medicine, S.Maria Hospital, IRCCS, Reggio Emilia, Italy. FAU - Mera-Valera, Antonio AU - Mera-Valera A AD - Division of Rheumatology, Instituto de Investigación Sanitaria, Hospital Clinico Universitario de Santiago, Santiago de Compostela, Spain. FAU - Perez-Gomez, Nair AU - Perez-Gomez N AD - Division of Rheumatology, Instituto de Investigación Sanitaria, Hospital Clinico Universitario de Santiago, Santiago de Compostela, Spain. FAU - Gerzeli, Simone AU - Gerzeli S AD - Department of Political and Social Sciences, Social Statistic studies, University of Pavia, Italy. FAU - Lopez-Mejias, Raquel AU - Lopez-Mejias R AD - Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - Matos-Costa, Carlo Jorge AU - Matos-Costa CJ AD - Rheumatology Unit, Centro Hospitalar e Universitário de Coimbra, Portugal. FAU - Pereira da Silva, Jose Antonio AU - Pereira da Silva JA AD - Rheumatology Unit, Centro Hospitalar e Universitário de Coimbra, Portugal. FAU - Cifrian, José AU - Cifrian J AD - Division of Pneumology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. FAU - Alpini, Claudia AU - Alpini C AD - Laboratory of Biochemical-Clinical Analyses, IRCCS Policlinico San Matteo Foundation, Pavia, Italy. FAU - Olivieri, Ignazio AU - Olivieri I AD - Rheumatology Institute of Lucania (IRel), Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, and Basilicata Biomedica (BRB) Foundation, Italy. FAU - Blázquez Cañamero, María Ángeles AU - Blázquez Cañamero MÁ AD - Rheumatology Unit, Severo Ochoa Hospital, Madrid, Spain. FAU - Rodriguez Cambrón, Ana Belén AU - Rodriguez Cambrón AB AD - Rheumatology Unit, Severo Ochoa Hospital, Madrid, Spain. FAU - Castañeda, Santos AU - Castañeda S AD - Rheumatology Department, Hospital Universitario de la Princesa, IIS-IP, Madrid, Spain. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. lorenzo.cavagna@unipv.it. CN - AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group. LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study DEP - 20170726 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Autoantibodies) RN - 0 (Biomarkers) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Arthritis/diagnosis/*epidemiology/immunology MH - Autoantibodies/blood MH - Biomarkers/blood MH - Europe/epidemiology MH - Female MH - Humans MH - Male MH - Mexico/epidemiology MH - Middle Aged MH - Myositis/diagnosis/*epidemiology/immunology MH - Phenotype MH - Prevalence MH - Prognosis MH - Retrospective Studies MH - Risk Factors MH - Time Factors EDAT- 2017/08/05 06:00 MHDA- 2018/04/25 06:00 CRDT- 2017/08/04 06:00 PHST- 2017/01/08 00:00 [received] PHST- 2017/04/05 00:00 [accepted] PHST- 2017/08/05 06:00 [pubmed] PHST- 2018/04/25 06:00 [medline] PHST- 2017/08/04 06:00 [entrez] AID - 11506 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2018 Jan-Feb;36(1):44-49. Epub 2017 Jul 26. PMID- 39089858 OWN - NLM STAT- MEDLINE DCOM- 20241016 LR - 20251102 IS - 1469-9001 (Electronic) IS - 1355-8382 (Print) IS - 1355-8382 (Linking) VI - 30 IP - 11 DP - 2024 Oct 16 TI - The antivirulent Staphylococcal sRNA SprC regulates CzrB efflux pump to adapt its response to zinc toxicity. PG - 1451-1464 LID - 10.1261/rna.080122.124 [doi] AB - Bacterial regulatory RNAs (sRNAs) are important players to control gene expression. In Staphylococcus aureus, SprC is an antivirulent trans-acting sRNA known to base-pair with the major autolysin atl mRNA, preventing its translation. Using MS2-affinity purification coupled with RNA sequencing, we looked for its sRNA-RNA interactome and identified 14 novel mRNA targets. In vitro biochemical investigations revealed that SprC binds two of them, czrB and deoD, and uses a single accessible region to regulate its targets, including Atl translation. Unlike Atl regulation, the characterization of the SprC-czrB interaction pinpointed a destabilization of the czrAB cotranscript, leading to a decrease of the mRNA level that impaired CzrB zinc efflux pump expression. On a physiological standpoint, we showed that SprC expression is detrimental to combat against zinc toxicity. In addition, phagocyctosis assays revealed a significant, but moderate, increase of czrB mRNA levels in a sprC-deleted mutant, indicating a functional link between SprC and czrB upon internalization in macrophages, and suggesting a role in resistance to both oxidative and zinc bursts. Altogether, our data uncover a novel pathway in which SprC is implicated, highlighting the multiple strategies used by S. aureus to balance virulence using an RNA regulator. CI - © 2024 Raynaud et al.; Published by Cold Spring Harbor Laboratory Press for the RNA Society. FAU - Raynaud, Simon AU - Raynaud S AD - Inserm, BRM (Bacterial RNAs and Medicine)-UMR_S 1230, Université de Rennes, 35000 Rennes, France. FAU - Hallier, Marc AU - Hallier M AD - Inserm, BRM (Bacterial RNAs and Medicine)-UMR_S 1230, Université de Rennes, 35000 Rennes, France. AD - Université de Rennes, QCPS (Quality Control in Protein Synthesis), IGDR UMR CNRS 6290, F-35042 Rennes, France. FAU - Dréano, Stéphane AU - Dréano S AD - Université de Rennes, CNRS UMR 6290 IGDR, BIOSIT, Molecular Bases of Tumorigenesis: VHL Disease Team, 35043 Rennes, France. FAU - Felden, Brice AU - Felden B AD - Inserm, BRM (Bacterial RNAs and Medicine)-UMR_S 1230, Université de Rennes, 35000 Rennes, France. FAU - Augagneur, Yoann AU - Augagneur Y AUID- ORCID: 0000-0002-6816-5931 AD - Inserm, BRM (Bacterial RNAs and Medicine)-UMR_S 1230, Université de Rennes, 35000 Rennes, France yoann.augagneur@univ-rennes.fr helene.lepabic@univ-rennes.fr. FAU - Le Pabic, Hélène AU - Le Pabic H AD - Inserm, BRM (Bacterial RNAs and Medicine)-UMR_S 1230, Université de Rennes, 35000 Rennes, France yoann.augagneur@univ-rennes.fr helene.lepabic@univ-rennes.fr. LA - eng PT - Journal Article DEP - 20241016 PL - United States TA - RNA JT - RNA (New York, N.Y.) JID - 9509184 RN - J41CSQ7QDS (Zinc) RN - 0 (Bacterial Proteins) RN - 0 (RNA, Bacterial) RN - 0 (RNA, Messenger) RN - 0 (RNA, Small Untranslated) SB - IM MH - *Zinc/metabolism/toxicity MH - *Staphylococcus aureus/genetics/metabolism/pathogenicity MH - *Bacterial Proteins/metabolism/genetics MH - *Gene Expression Regulation, Bacterial MH - *RNA, Bacterial/genetics/metabolism MH - Virulence/genetics MH - RNA, Messenger/genetics/metabolism MH - RNA, Small Untranslated/genetics/metabolism MH - Macrophages/microbiology/metabolism/drug effects PMC - PMC11482605 OTO - NOTNLM OT - SprC OT - Staphylococcus aureus OT - czrB OT - sRNA OT - zinc EDAT- 2024/08/02 00:46 MHDA- 2024/10/17 10:03 PMCR- 2025/11/01 CRDT- 2024/08/01 21:14 PHST- 2024/06/07 00:00 [received] PHST- 2024/07/24 00:00 [accepted] PHST- 2024/10/17 10:03 [medline] PHST- 2024/08/02 00:46 [pubmed] PHST- 2024/08/01 21:14 [entrez] PHST- 2025/11/01 00:00 [pmc-release] AID - rna.080122.124 [pii] AID - RA [pii] AID - 10.1261/rna.080122.124 [doi] PST - epublish SO - RNA. 2024 Oct 16;30(11):1451-1464. doi: 10.1261/rna.080122.124. PMID- 25900032 OWN - NLM STAT- MEDLINE DCOM- 20170717 LR - 20170817 IS - 1479-666X (Print) IS - 1479-666X (Linking) VI - 14 IP - 5 DP - 2016 Oct TI - Endoscopic thoracic sympathectomy for upper limb ischemia. A 16 year follow-up in a single center. PG - 265-9 LID - S1479-666X(15)00034-7 [pii] LID - 10.1016/j.surge.2015.03.002 [doi] AB - INTRODUCTION: The aim of our study was to evaluate the long term results of Endoscopic Thoracic Sympathectomy (ETS) in the management of upper limb ischemia (ULI). METHODS: We retrospectively reviewed the records of all consecutive patients who underwent ETS for ULI between January 1994 and May 2009. A standardized questionnaire was used to evaluate the long term success, morbidity and overall patient satisfaction. RESULTS: Thirty-five patients (20 female, mean age 49 years (range 23-79)) underwent bilateral (n = 9) and unilateral (n = 27) ETS procedures, respectively. Six patients had Primary (idiopathic) Raynaud Disease. Twenty-nine patients had upper limb ischemia secondary to systemic disorders (n = 12), embolic disease (n = 10), occlusion of the arteries of the arm (n = 5) or hypothenar hammer syndrome (n = 2). Tissue loss at time of surgery was present in nineteen patients. Short term beneficial effects were reported by 12 patients (63%). Eleven of the 35 patients experienced a total of 13 complications or adverse events, whereof 11 were minor or transient. Limb salvage was unsuccessful in three patients because of major amputations (n = 2) or severe functional impairment (n = 1). Necrotectomies or minor amputations without functional impairment were performed in 9 patients. Medium or long term follow up (mean 98 months (range 18-198) was available in 19 out of 22 living patients(86%). Long term beneficial effects were reported by 10 (53%). Overall patient satisfaction was 56%. Compensatory sweating was experienced by 11 patients (58%). CONCLUSION: Although the long term efficacy of ETS in our study was moderate (53%), due to its low invasiveness ETS is a valuable option in the management of ULI. CI - Copyright © 2016. Published by Elsevier Ltd. FAU - Coveliers, Hans AU - Coveliers H AD - Department of Vascular Surgery, VU Medical Center, Amsterdam, The Netherlands. Electronic address: h.coveliers@vumc.nl. FAU - Hoexum, Frank AU - Hoexum F AD - Department of Vascular Surgery, VU Medical Center, Amsterdam, The Netherlands. FAU - Rauwerda, Jan A AU - Rauwerda JA AD - Department of Vascular Surgery, VU Medical Center, Amsterdam, The Netherlands. FAU - Wisselink, Willem AU - Wisselink W AD - Department of Vascular Surgery, VU Medical Center, Amsterdam, The Netherlands. LA - eng PT - Journal Article DEP - 20150418 PL - Scotland TA - Surgeon JT - The surgeon : journal of the Royal Colleges of Surgeons of Edinburgh and Ireland JID - 101168329 SB - IM MH - Adult MH - Aged MH - Female MH - Follow-Up Studies MH - Humans MH - *Ischemia MH - Male MH - Middle Aged MH - Patient Satisfaction MH - Quality of Life MH - Retrospective Studies MH - Surveys and Questionnaires MH - *Sympathectomy/methods MH - Thoracic Nerves/*surgery MH - *Thoracoscopy/methods MH - Treatment Outcome MH - Upper Extremity/*blood supply/*surgery OTO - NOTNLM OT - Endoscopic OT - Ischemia OT - Sympathectomy OT - Thoracic OT - Upper limb EDAT- 2015/04/23 06:00 MHDA- 2017/07/18 06:00 CRDT- 2015/04/23 06:00 PHST- 2014/05/16 00:00 [received] PHST- 2015/03/24 00:00 [accepted] PHST- 2015/04/23 06:00 [entrez] PHST- 2015/04/23 06:00 [pubmed] PHST- 2017/07/18 06:00 [medline] AID - S1479-666X(15)00034-7 [pii] AID - 10.1016/j.surge.2015.03.002 [doi] PST - ppublish SO - Surgeon. 2016 Oct;14(5):265-9. doi: 10.1016/j.surge.2015.03.002. Epub 2015 Apr 18. PMID- 19590382 OWN - NLM STAT- MEDLINE DCOM- 20091009 LR - 20141105 IS - 2331-2637 (Electronic) IS - 1074-7931 (Linking) VI - 15 IP - 4 DP - 2009 Jul TI - Digital neuropathy of the median and ulnar nerves caused by Dupuytren's contracture: Case report. PG - 217-9 LID - 10.1097/NRL.0b013e3181a8c983 [doi] AB - INTRODUCTION: Digital neuropathy is a pure sensory neuropathy of a digital nerve. It may be caused by acute or chronic local trauma or pressure, or accompany systemic illnesses such as rheumatoid disease, leprosy, Raynaud disease, dysproteinemia, or diabetes mellitus. We describe an extraordinary case of digital neuropathy of the median and ulnar nerves caused by Dupuytren contracture. CASE REPORT: A 56-year-old right-handed man was presented with numbness and tingling of the little finger of the right and ring finger of the left hand. The clinical and EMG findings in this patient were consistent with a lesion of the median and ulnar palmar digital nerves of the right and left ring and little fingers. CONCLUSION: Dupuytren tissue usually affects the palmar fascia, superficial to the digital nerves, and it may rarely affect the spiral cord in the digits. A spiral cord may cause sensory loss due to impingement of digital nerves or Dupuytren tissue may have been compressing the palmar digital nerves against the relatively inelastic deep transverse metacarpal ligament. As a result, digital neuropathy can develop in those with Dupuytren's contracture, and nerve conduction studies should also be performed to determine the condition. New studies are needed to provide better diagnostic criteria for the condition. FAU - Guney, Figen AU - Guney F AD - Department of Neurology, University of Selcuk, Konya, Turkey. guneyfigen@yahoo.com.tr FAU - Yuruten, Betigul AU - Yuruten B FAU - Karalezli, Nazim AU - Karalezli N LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Neurologist JT - The neurologist JID - 9503763 RN - 0 (Anticonvulsants) RN - 0 (Glucocorticoids) RN - 1ZK20VI6TY (Triamcinolone) RN - 33CM23913M (Carbamazepine) SB - IM MH - Anticonvulsants/pharmacology/therapeutic use MH - Carbamazepine/pharmacology/therapeutic use MH - Dupuytren Contracture/etiology/pathology/*physiopathology MH - Electrodiagnosis MH - Fascia/pathology/physiopathology MH - Fingers/innervation/*physiopathology MH - Glucocorticoids/pharmacology/therapeutic use MH - Hand/innervation/pathology/physiopathology MH - Humans MH - Male MH - Median Nerve/pathology/physiopathology/surgery MH - Median Neuropathy/etiology/pathology/*physiopathology MH - Middle Aged MH - Neural Conduction/physiology MH - Patient Compliance MH - Treatment Outcome MH - Triamcinolone/pharmacology/therapeutic use MH - Ulnar Nerve/pathology/physiopathology/surgery MH - Ulnar Neuropathies/etiology/pathology/*physiopathology EDAT- 2009/07/11 09:00 MHDA- 2009/10/10 06:00 CRDT- 2009/07/11 09:00 PHST- 2009/07/11 09:00 [entrez] PHST- 2009/07/11 09:00 [pubmed] PHST- 2009/10/10 06:00 [medline] AID - 00127893-200907000-00007 [pii] AID - 10.1097/NRL.0b013e3181a8c983 [doi] PST - ppublish SO - Neurologist. 2009 Jul;15(4):217-9. doi: 10.1097/NRL.0b013e3181a8c983. PMID- 26371289 OWN - NLM STAT- MEDLINE DCOM- 20170606 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 75 IP - 8 DP - 2016 Aug TI - The interferon type I signature is present in systemic sclerosis before overt fibrosis and might contribute to its pathogenesis through high BAFF gene expression and high collagen synthesis. PG - 1567-73 LID - 10.1136/annrheumdis-2015-207392 [doi] AB - BACKGROUND: Interferon (IFN) signature has been reported in definite systemic sclerosis (SSc) but it has not been characterised in early SSc (EaSSc). We aim at characterising IFN type I signature in SSc before overt skin fibrosis develops. METHODS: The expression of 11 IFN type I inducible genes was tested in whole-blood samples from 30 healthy controls (HCs), 12 subjects with primary Raynaud's phenomenon (RP), 19 patients with EaSSc, 7 patients with definite SSc without cutaneous fibrosis, 21 limited cutaneous SSc and 10 diffuse cutaneous SSc subjects. The correlation between IFN activity in monocytes, B cell activating factor (BAFF) mRNA expression and type III procollagen N-terminal propeptide (PIIINP) serum levels was tested. RESULTS: In all the SSc groups, higher IFN scores were observed compared with HC. An IFN score ≥7.09 discriminated HCs from patients with SSc (sensitivity=0.7, specificity=0.88, area under receiving operating characteristic (AUROC)=0.82); the prevalence of an elevated IFN score was: HC=3.3%; RP=33.3%, EaSSc=78.9%, definite SSc=100%, limited cutaneous SSc=42.9%, diffuse cutaneous SSc=70.0%. In monocytes an IFN score ≥4.12 distinguished HCs from patients with fibrotic SSc (sensitivity=0.62, specificity=0.85, AUROC=0.76). Compared with IFN-negative subjects, IFN-positive subjects had higher monocyte BAFF mRNA levels (19.7±5.2 vs 15.20±4.0, p=2.1×10(-5)) and serum PIIINP levels (median=6.0 (IQR 5.4-8.9) vs median=3.9 (IQR 3.3-4.7), p=0.0004). CONCLUSIONS: An IFN type I signature is observed in patients with SSc from the earliest phases of the disease, even before overt skin fibrosis. The presence of IFN type I signature in monocytes is correlated with BAFF mRNA expression and serum PIIINP levels, supporting a contribution in the pathogenesis and progression of SSc. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/ FAU - Brkic, Zana AU - Brkic Z AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - van Bon, Lenny AU - van Bon L AD - Department of Rheumatology, Clinical Immunology and Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. FAU - Cossu, Marta AU - Cossu M AD - Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - van Helden-Meeuwsen, Cornelia G AU - van Helden-Meeuwsen CG AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. FAU - Knaapen, Hanneke AU - Knaapen H AD - Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. FAU - van den Berg, Wim AU - van den Berg W AD - Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. FAU - Dalm, Virgil A AU - Dalm VA AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Van Daele, Paul L AU - Van Daele PL AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Severino, Adriana AU - Severino A AD - Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Maria, Naomi I AU - Maria NI AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Guillen, Samara AU - Guillen S AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Dik, Willem A AU - Dik WA AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Beretta, Lorenzo AU - Beretta L AD - Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Versnel, Marjan A AU - Versnel MA AD - Department of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Radstake, Timothy AU - Radstake T AD - Department of Rheumatology, Clinical Immunology and Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150914 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (B-Cell Activating Factor) RN - 0 (Interferon Type I) RN - 0 (Peptide Fragments) RN - 0 (Procollagen) RN - 0 (RNA, Messenger) RN - 0 (TNFSF13B protein, human) RN - 0 (procollagen Type III-N-terminal peptide) SB - IM MH - Adult MH - Aged MH - B-Cell Activating Factor/*biosynthesis/genetics MH - Case-Control Studies MH - Female MH - Fibrosis MH - Gene Expression Regulation MH - Humans MH - Interferon Type I/biosynthesis/*genetics MH - Male MH - Middle Aged MH - Monocytes/metabolism MH - Peptide Fragments/biosynthesis/blood MH - Procollagen/biosynthesis/blood MH - RNA, Messenger/genetics MH - Scleroderma, Systemic/*genetics/metabolism MH - Skin/pathology MH - Transcriptome OTO - NOTNLM OT - Autoimmune Diseases OT - Cytokines OT - Inflammation OT - Systemic Sclerosis EDAT- 2015/09/16 06:00 MHDA- 2017/06/07 06:00 CRDT- 2015/09/16 06:00 PHST- 2015/02/01 00:00 [received] PHST- 2015/08/28 00:00 [accepted] PHST- 2015/09/16 06:00 [entrez] PHST- 2015/09/16 06:00 [pubmed] PHST- 2017/06/07 06:00 [medline] AID - S0003-4967(24)01763-1 [pii] AID - 10.1136/annrheumdis-2015-207392 [doi] PST - ppublish SO - Ann Rheum Dis. 2016 Aug;75(8):1567-73. doi: 10.1136/annrheumdis-2015-207392. Epub 2015 Sep 14. PMID- 20175840 OWN - NLM STAT- MEDLINE DCOM- 20100607 LR - 20260128 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 37 IP - 1 DP - 2010 Jan TI - Future treatments in systemic sclerosis. PG - 54-70 LID - 10.1111/j.1346-8138.2009.00758.x [doi] AB - Systemic sclerosis (SSc) is an autoimmune disorder with clinical manifestations resulting from immune activation, fibrosis development and damage of small blood vessels. Although there have been no established treatments for SSc, lots of new treatments targeting organ and pathogenesis are in the process of development. Transforming growth factor (TGF)-beta is a major cytokine involved in the pathogenesis of fibrosis in SSc. The blockade of cell surface molecules capable of activating latent TGF-beta, blockade of ligand by the pan-isoform-specific antibody, soluble TGF-beta receptors and a recombinant latency associated peptide, as well as inhibitors for ALK5 and Smad3 are the potential strategies to abolish the pathological activation of TGF-beta signaling in SSc fibroblasts. Besides TGF-beta, connective tissue growth factor (CTGF)/CCN2, platelet-derived growth factor (PDGF) and endothelin-1 are the candidates for the new therapeutic targets. As for immune dysfunction in SSc, i.v. immunoglobulin infusion, stem cell transplantation and B-cell depletion are potential new therapies under or awaiting a randomized, double-blind, placebo-controlled trial, although their efficacies are still controversial. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and inhibitors for serotonin signaling are the new therapeutic targets for Raynaud's phenomenon, digital ulceration and pulmonary arterial hypertension in SSc. Imatinib mesylate may be a novel new therapy for fibrosis and vasculopathy in SSc because it reverses the expression levels of Fli1, which is a transcription factor downregulated in SSc through an epigenetic mechanism and is likely to be involved in the development of fibrosis and vasculopathy in this disease. Potential therapeutic targets other than those described above are also reviewed. FAU - Asano, Yoshihide AU - Asano Y AD - Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan. yasano-tky@umin.ac.jp LA - eng PT - Journal Article PT - Review PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0 (Benzamides) RN - 139568-91-5 (Connective Tissue Growth Factor) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Endothelin-1) RN - 8A1O1M485B (Imatinib Mesylate) RN - 0 (Immunoglobulins) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Piperazines) RN - 0 (Platelet-Derived Growth Factor) RN - 0 (Pyrimidines) RN - 0 (Receptors, Transforming Growth Factor beta) RN - 0 (Selective Serotonin Reuptake Inhibitors) RN - 0 (Smad3 Protein) RN - 0 (Transforming Growth Factor beta) SB - IM MH - Animals MH - Autoimmune Diseases/*drug therapy/*immunology/physiopathology MH - B-Lymphocytes/immunology MH - Benzamides MH - Connective Tissue Growth Factor/antagonists & inhibitors/immunology MH - Endothelin Receptor Antagonists MH - Endothelin-1/antagonists & inhibitors/immunology MH - Humans MH - Imatinib Mesylate MH - Immunoglobulins/therapeutic use MH - Mice MH - Phosphodiesterase 5 Inhibitors MH - Piperazines/therapeutic use MH - Platelet-Derived Growth Factor/antagonists & inhibitors/immunology MH - Pyrimidines/therapeutic use MH - Randomized Controlled Trials as Topic MH - Receptors, Transforming Growth Factor beta/antagonists & inhibitors/immunology MH - Scleroderma, Systemic/*drug therapy/*immunology/physiopathology MH - Selective Serotonin Reuptake Inhibitors/therapeutic use MH - Smad3 Protein/antagonists & inhibitors/immunology MH - Transforming Growth Factor beta/antagonists & inhibitors/immunology RF - 160 EDAT- 2010/02/24 06:00 MHDA- 2010/06/09 06:00 CRDT- 2010/02/24 06:00 PHST- 2010/02/24 06:00 [entrez] PHST- 2010/02/24 06:00 [pubmed] PHST- 2010/06/09 06:00 [medline] AID - JDE758 [pii] AID - 10.1111/j.1346-8138.2009.00758.x [doi] PST - ppublish SO - J Dermatol. 2010 Jan;37(1):54-70. doi: 10.1111/j.1346-8138.2009.00758.x. PMID- 35940960 OWN - NLM STAT- MEDLINE DCOM- 20221216 LR - 20221221 IS - 1096-3618 (Electronic) IS - 1044-5323 (Linking) VI - 58 DP - 2021 Dec TI - Cellular and Molecular Diversity in Scleroderma. PG - 101648 LID - S1044-5323(22)00065-3 [pii] LID - 10.1016/j.smim.2022.101648 [doi] AB - With the increasing armamentarium of high-throughput tools available at manageable cost, it is attractive and informative to determine the molecular underpinnings of patient heterogeneity in systemic sclerosis (SSc). Given the highly variable clinical outcomes of patients labelled with the same diagnosis, unravelling the cellular and molecular basis of disease heterogeneity will be crucial to predicting disease risk, stratifying management and ultimately informing a patient-centered precision medicine approach. Herein, we summarise the findings of the past several years in the fields of genomics, transcriptomics, and proteomics that contribute to unraveling the cellular and molecular heterogeneity of SSc. Expansion of these findings and their routine integration with quantitative analysis of histopathology and imaging studies into clinical care promise to inform a scientifically driven patient-centred personalized medicine approach to SSc in the near future. CI - Copyright © 2022. Published by Elsevier Ltd. FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Yale School of Medicine, Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, USA. Electronic address: monique.hinchcliff@yale.edu. FAU - Garcia-Milian, Rolando AU - Garcia-Milian R AD - Bioinformatics Support Program, Cushing/Whitney Medical Library, New Haven, CT, USA. FAU - Di Donato, Stefano AU - Di Donato S AD - Raynaud's and Scleroderma Programme, Leeds Institute of Rheumatic and Musculoskeletal Medicine and NIHR Biomedical Research Centre, University of Leeds, UK. FAU - Dill, Karin AU - Dill K AD - Emory Healthcare, USA. FAU - Bundschuh, Elizabeth AU - Bundschuh E AD - Yale School of Medicine, Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, USA. FAU - Galdo, Francesco Del AU - Galdo FD AD - Raynaud's and Scleroderma Programme, Leeds Institute of Rheumatic and Musculoskeletal Medicine and NIHR Biomedical Research Centre, University of Leeds, UK. Electronic address: F.delgaldo@leeds.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20220806 PL - England TA - Semin Immunol JT - Seminars in immunology JID - 9009458 SB - IM MH - Humans MH - *Scleroderma, Systemic/genetics/therapy/diagnosis MH - Proteomics/methods MH - Gene Expression Profiling EDAT- 2022/08/09 06:00 MHDA- 2022/12/15 06:00 CRDT- 2022/08/08 22:04 PHST- 2022/08/09 06:00 [pubmed] PHST- 2022/12/15 06:00 [medline] PHST- 2022/08/08 22:04 [entrez] AID - S1044-5323(22)00065-3 [pii] AID - 10.1016/j.smim.2022.101648 [doi] PST - ppublish SO - Semin Immunol. 2021 Dec;58:101648. doi: 10.1016/j.smim.2022.101648. Epub 2022 Aug 6. PMID- 37028847 OWN - NLM STAT- MEDLINE DCOM- 20230411 LR - 20230411 IS - 1558-3163 (Electronic) IS - 0889-857X (Linking) VI - 49 IP - 2 DP - 2023 May TI - Patient Experience of Systemic Sclerosis-Related Calcinosis: An International Study Informing Clinical Trials, Practice, and the Development of the Mawdsley Calcinosis Questionnaire. PG - 463-481 LID - S0889-857X(23)00017-0 [pii] LID - 10.1016/j.rdc.2023.01.017 [doi] AB - Systemic sclerosis (SSc) -related calcinosis can be a debilitating, constantly painful, poorly understood vascular complication of calcium hydroxyapatite deposition in soft tissue structures that affects approximately 40% of both limited and diffuse cutaneous SSc subtypes. This publication describes the iterative and multitiered international qualitative investigations that yielded remarkable insights into natural history, daily experience, and complications of SSc-calcinosis providing pivotal information for health management. Patient-driven question development and field testing, according to Food and Drug Administration guidance, propelled the development of a patient-reported outcome measure for SSc-calcinosis, the Mawdsley Calcinosis Questionnaire. CI - Copyright © 2023 Elsevier Inc. All rights reserved. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; University Medical Center-Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, LA, USA; Section of Pulmonary Medicine, Louisiana State University School of Medicine, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA. Electronic address: lsaketk@tulane.edu. FAU - Gordon, Jessica K AU - Gordon JK AD - Department of Rheumatology, Hospital for Special Surgery, New York, NY, USA. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma & Raynaud Society, UK (SRUK); Federation of European Scleroderma Associations, UK. FAU - Mawdsley, Anne AU - Mawdsley A AD - Raynaud's & Scleroderma Association-Care and Support, London, UK. FAU - Chaudhry, Humza A AU - Chaudhry HA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA. FAU - Valenzuela, Antonia AU - Valenzuela A AD - Department of Rheumatology and Clinical Immunology, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Christensen, Angela AU - Christensen A AD - Doctors Hospital, Renaissance, TX, USA. FAU - Khalique, Samara M AU - Khalique SM AD - Department of Rheumatology, Virginia Tech Carilion Clinic School of Medicine, Roanoke, VA, USA. FAU - Jensen, Kelly AU - Jensen K AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA; University of Colorado School of Medicine, Denver, CO, USA. FAU - Weinmann, Sophia C AU - Weinmann SC AD - Department of Medicine, Division of Rheumatology and Immunology, Duke University Hospital, Durham, NC, USA. FAU - Busman, Evan AU - Busman E AD - Healthcare Patient Advocate, Atlanta, GA, USA. FAU - Chung, Lorinda AU - Chung L AD - Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine and Palo Alto VA Healthcare System, Palo Alto, CA, USA; Department of Dermatology, Division of Immunology and Rheumatology, Stanford University School of Medicine and Palo Alto VA Healthcare System, Palo Alto, CA, USA. FAU - Hsu, Vivien M AU - Hsu VM AD - RWJ-Scleroderma Program, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA. FAU - Russell, Anne-Marie AU - Russell AM AD - Respiratory Institute, University of Exeter, Exeter, UK; Respiratory Medicine, Royal Devon University Healthcare NHS Foundation Trust, UK. FAU - Steen, Virginia D AU - Steen VD AD - Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA. LA - eng PT - Journal Article PT - Review PL - United States TA - Rheum Dis Clin North Am JT - Rheumatic diseases clinics of North America JID - 8708093 SB - IM MH - Humans MH - *Scleroderma, Systemic/complications MH - *Calcinosis/complications MH - Surveys and Questionnaires MH - Patient Outcome Assessment OTO - NOTNLM OT - Calcinosis OT - Digital ulcers OT - Patient-reported outcomes OT - Qualitative research OT - Qualtiy of LIfe OT - Raynaud phenomenon OT - Scleroderma OT - Self-Management EDAT- 2023/04/08 06:00 MHDA- 2023/04/11 06:42 CRDT- 2023/04/07 20:57 PHST- 2023/04/11 06:42 [medline] PHST- 2023/04/07 20:57 [entrez] PHST- 2023/04/08 06:00 [pubmed] AID - S0889-857X(23)00017-0 [pii] AID - 10.1016/j.rdc.2023.01.017 [doi] PST - ppublish SO - Rheum Dis Clin North Am. 2023 May;49(2):463-481. doi: 10.1016/j.rdc.2023.01.017. PMID- 36996276 OWN - NLM STAT- MEDLINE DCOM- 20230831 LR - 20260518 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 75 IP - 9 DP - 2023 Sep TI - Autoantibodies Recognizing Specificity Protein 4 Co-occur With Anti-Transcription Intermediary Factor 1 and Are Associated With Distinct Clinical Features and Immunogenetic Risk Factors in Juvenile Myositis. PG - 1668-1677 LID - 10.1002/art.42512 [doi] AB - OBJECTIVE: Autoantibodies recognizing specificity protein 4 (Sp4) were recently discovered in adults with idiopathic inflammatory myopathies (IIM). Anti-Sp4 autoantibodies co-occurred in patients with anti-transcription intermediary factor 1 (anti-TIF1) autoantibody-positive dermatomyositis (DM) and were associated with a reduced risk of cancer. In the present study, the prevalence and clinical features associated with anti-Sp4 autoantibodies in juvenile-onset IIM were investigated. METHODS: Serum samples from 336 patients with juvenile myositis in a cross-sectional cohort and 91 healthy controls were screened for anti-Sp4 autoantibodies using enzyme-linked immunosorbent assay. Clinical characteristics, outcomes, and HLA alleles of those with and those without anti-Sp4 autoantibodies were compared. RESULTS: Anti-Sp4 autoantibodies were present in 23 patients (7%) with juvenile myositis and were not present in any of the controls. Anti-Sp4 autoantibodies were found among each clinical myositis subgroup. The frequency of TIF1 autoantibody positivity was significantly higher among those with anti-Sp4 autoantibodies (21 [91%] versus 92 [30%], P < 0.001). In the anti-TIF1 autoantibody-positive subgroup, Raynaud's phenomenon (8 [38%] versus 2 [2%], P < 0.001) was more common and peak aspartate aminotransferase was significantly lower in those with anti-Sp4 autoantibodies. None of the patients with anti-Sp4 autoantibodies required a wheelchair. Among White patients, DQA1*04 and DRB1*08 were associated with anti-Sp4 autoantibodies. CONCLUSION: Anti-Sp4 autoantibodies were found in patients with juvenile-onset IIM, predominantly those with coexisting anti-TIF1 autoantibodies. Patients with anti-Sp4 autoantibodies represent a phenotypic subset of anti-TIF1 autoantibody-positive myositis characterized by frequent Raynaud's phenomenon and less pronounced muscle involvement, similar to adults with these autoantibodies. Novel immunogenetic risk factors for White patients with IIM were identified among juveniles with anti-Sp4 autoantibodies. CI - © 2023 American College of Rheumatology. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA. FAU - Sherman, Matthew A AU - Sherman MA AUID- ORCID: 0000-0002-5448-1538 AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. FAU - Pak, Katherine AU - Pak K AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. FAU - Pinal-Fernandez, Iago AU - Pinal-Fernandez I AUID- ORCID: 0000-0001-6338-9218 AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland, and Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Flegel, Willy A AU - Flegel WA AUID- ORCID: 0000-0002-1631-7198 AD - Department of Transfusion Medicine, NIH Clinical Center, NIH, Bethesda, Maryland. FAU - Targoff, Ira N AU - Targoff IN AD - Veteran's Affairs Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma. FAU - Miller, Frederick W AU - Miller FW AUID- ORCID: 0000-0003-2831-9593 AD - Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Rider, Lisa G AU - Rider LG AUID- ORCID: 0000-0002-6912-2458 AD - Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Mammen, Andrew L AU - Mammen AL AUID- ORCID: 0000-0003-3732-3252 AD - Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, and Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland. CN - Childhood Myositis Heterogeneity Collaborative Study Group LA - eng GR - ZIA ES101074/ImNIH/Intramural NIH HHS/United States GR - Z01 ES101081/ImNIH/Intramural NIH HHS/United States GR - Z99 AR999999/ImNIH/Intramural NIH HHS/United States GR - ZIC CL002128/ImNIH/Intramural NIH HHS/United States GR - ZIA AR041203/ImNIH/Intramural NIH HHS/United States GR - ZIA ES101081/ImNIH/Intramural NIH HHS/United States GR - Z01 ES101074/ImNIH/Intramural NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Intramural DEP - 20230719 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Humans MH - *Dermatomyositis MH - Autoantibodies MH - Cross-Sectional Studies MH - Immunogenetics MH - Mediation Analysis MH - *Myositis MH - Risk Factors PMC - PMC10524257 MID - NIHMS1888121 COIS- Disclosures: MAS, KP, IPF, WAF, FWM, LGR, and ALM report no competing interests. INT is a consultant to the Oklahoma Medical Research Foundation Clinical Immunology Laboratory with regard to myositis autoantibody testing and a myositis section editor for UpToDate. FIR - Albert, Daniel A IR - Albert DA FIR - Arabshahi, Bita IR - Arabshahi B FIR - Balboni, Imelda M IR - Balboni IM FIR - Ballinger, Susan IR - Ballinger S FIR - Bayat, Nastaran IR - Bayat N FIR - Bingham, C April IR - Bingham CA FIR - Bohnsack, John F IR - Bohnsack JF FIR - Cartwright, Victoria W IR - Cartwright VW FIR - Cron, Randy Q IR - Cron RQ FIR - Curiel, Rodolfo IR - Curiel R FIR - de la Pena, Wendy IR - de la Pena W FIR - de Guzman, Marietta M IR - de Guzman MM FIR - Eberhardt, Barbara Anne IR - Eberhardt BA FIR - Edelheit, Barbara S IR - Edelheit BS FIR - Farhadi, Payam Noroozi IR - Farhadi PN FIR - Finkel, Terri H IR - Finkel TH FIR - Fuhlbrigge, Robert C IR - Fuhlbrigge RC FIR - Gewanter, Harry L IR - Gewanter HL FIR - Goldmuntz, Ellen A IR - Goldmuntz EA FIR - Gottlieb, Beth S IR - Gottlieb BS FIR - Griffin, Thomas A IR - Griffin TA FIR - Groh, Brandt P IR - Groh BP FIR - Hannan, William P IR - Hannan WP FIR - Hawkins-Holt, Melissa IR - Hawkins-Holt M FIR - Henrickson, Michael IR - Henrickson M FIR - Higgins, Gloria C IR - Higgins GC FIR - Imundo, Lisa IR - Imundo L FIR - Jansen, Anna IR - Jansen A FIR - Jarvis, James IR - Jarvis J FIR - Jones, Olcay Y IR - Jones OY FIR - Kamdar, Ankur IR - Kamdar A FIR - Kim, Hanna IR - Kim H FIR - Kingsbury, Daniel J IR - Kingsbury DJ FIR - Kishi, Takayuki IR - Kishi T FIR - Lindsley, Carol B IR - Lindsley CB FIR - Mamyrova, Gulnara IR - Mamyrova G FIR - McCarthy, Paul L IR - McCarthy PL FIR - Mitchell, Stephen R IR - Mitchell SR FIR - Nanda, Kabita IR - Nanda K FIR - Nativ, Simona IR - Nativ S FIR - Oral, Elif A IR - Oral EA FIR - Pachman, Lauren M IR - Pachman LM FIR - Perez, Maria D IR - Perez MD FIR - Person, Donald A IR - Person DA FIR - Rabinovich, Egla C IR - Rabinovich EC FIR - Ronis, Tova IR - Ronis T FIR - Sabbagh, Sara E IR - Sabbagh SE FIR - Sarkar, Kakali IR - Sarkar K FIR - Schiffenbauer, Adam IR - Schiffenbauer A FIR - Shaham, Bracha IR - Shaham B FIR - Soep, Jennifer IR - Soep J FIR - Stoll, Matthew L IR - Stoll ML FIR - Sule, Sangeeta IR - Sule S FIR - Tarvin, Stacey E IR - Tarvin SE FIR - Taylor-Albert, Elizabeth IR - Taylor-Albert E FIR - Vogelgesang, Scott A IR - Vogelgesang SA FIR - Volochayev, Rita IR - Volochayev R FIR - White, Patience H IR - White PH EDAT- 2023/03/31 06:00 MHDA- 2023/08/31 06:41 PMCR- 2024/09/01 CRDT- 2023/03/30 14:22 PHST- 2023/03/17 00:00 [revised] PHST- 2022/11/21 00:00 [received] PHST- 2023/03/28 00:00 [accepted] PHST- 2023/08/31 06:41 [medline] PHST- 2023/03/31 06:00 [pubmed] PHST- 2023/03/30 14:22 [entrez] PHST- 2024/09/01 00:00 [pmc-release] AID - 10.1002/art.42512 [doi] PST - ppublish SO - Arthritis Rheumatol. 2023 Sep;75(9):1668-1677. doi: 10.1002/art.42512. Epub 2023 Jul 19. PMID- 29390428 OWN - NLM STAT- MEDLINE DCOM- 20180213 LR - 20260127 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 96 IP - 51 DP - 2017 Dec TI - Clinical and serological features of systemic sclerosis in a multicenter African American cohort: Analysis of the genome research in African American scleroderma patients clinical database. PG - e8980 LID - 10.1097/MD.0000000000008980 [doi] LID - e8980 AB - Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research in African American Scleroderma Patients (GRASP) cohort).African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987-2016), from 18 academic centers throughout the United States. The cross-sectional prevalence of sociodemographic, clinical, and serological features was evaluated. Factors associated with clinically significant manifestations of SSc were assessed using multivariate logistic regression analyses.The study population included a total of 1009 African American SSc patients, comprised of 84% women. In total, 945 (94%) patients met the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc, with the remaining 64 (6%) meeting the 1980 ACR or CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) criteria. While 43% were actively employed, 33% required disability support. The majority (57%) had the more severe diffuse subtype and a young age at symptom onset (39.1 ± 13.7 years), in marked contrast to that reported in cohorts of predominantly European ancestry. Also, 1 in 10 patients had a severe Medsger cardiac score of 4. Pulmonary fibrosis evident on computed tomography (CT) chest was present in 43% of patients and was significantly associated with anti-topoisomerase I positivity. 38% of patients with CT evidence of pulmonary fibrosis had a severe restrictive ventilator defect, forced vital capacity (FVC) ≤50% predicted. A significant association was noted between longer disease duration and higher odds of pulmonary hypertension, telangiectasia, and calcinosis. The prevalence of potentially fatal scleroderma renal crisis was 7%, 3.5 times higher than the 2% prevalence reported in the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) cohort.Our study emphasizes the unique and severe disease burden of SSc in African Americans compared to those of European ancestry. CI - Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved. FAU - Morgan, Nadia D AU - Morgan ND AD - Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD Division of Rheumatology, University of Texas-McGovern Medical School, Houston, TX Division of Rheumatology, University of Pittsburgh, PA Division of Rheumatology, Georgetown University School of Medicine, Washington, DC Division of Rheumatology, Northwestern University, Feinberg School of Medicine, Chicago, IL Division of Rheumatology, Medical University of South Carolina, Charleston, SC Division of Rheumatology, University of Michigan, Ann Arbor, MI Division of Rheumatology, Robert Wood Johnson University, New Brunswick, NJ Division of Rheumatology, Hospital for Special Surgery, New York, NY Department of Rheumatology, Arthritis and Osteoporosis Consultants of the Carolinas, Charlotte, NC Division of Rheumatology, Tulane University School of Medicine, New Orleans, LA Division of Rheumatology, University of California San Francisco, CA Division of Rheumatology, University of Pennsylvania, Philadelphia, PA Division of Rheumatology, Boston University School of Medicine, Boston, MA Division of Rheumatology, George Washington University, Washington, DC Division of Rheumatology, Stanford University School of Medicine, Stanford, CA Division of Rheumatology, University of Chicago Pritzker School of Medicine, Chicago, IL Division of Rheumatology, New York University Langone Medical Center, New York, NY National Human Genome Research Institute National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA. FAU - Shah, Ami A AU - Shah AA FAU - Mayes, Maureen D AU - Mayes MD FAU - Domsic, Robyn T AU - Domsic RT FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Steen, Virginia D AU - Steen VD FAU - Varga, John AU - Varga J FAU - Carns, Mary AU - Carns M FAU - Ramos, Paula S AU - Ramos PS FAU - Silver, Richard M AU - Silver RM FAU - Schiopu, Elena AU - Schiopu E FAU - Khanna, Dinesh AU - Khanna D FAU - Hsu, Vivien AU - Hsu V FAU - Gordon, Jessica K AU - Gordon JK FAU - Gladue, Heather AU - Gladue H FAU - Saketkoo, Lesley A AU - Saketkoo LA FAU - Criswell, Lindsey A AU - Criswell LA FAU - Derk, Chris T AU - Derk CT FAU - Trojanowski, Marcin A AU - Trojanowski MA FAU - Shanmugam, Victoria K AU - Shanmugam VK FAU - Chung, Lorinda AU - Chung L FAU - Valenzuela, Antonia AU - Valenzuela A FAU - Jan, Reem AU - Jan R FAU - Goldberg, Avram AU - Goldberg A FAU - Remmers, Elaine F AU - Remmers EF FAU - Kastner, Daniel L AU - Kastner DL FAU - Wigley, Fredrick M AU - Wigley FM FAU - Gourh, Pravitt AU - Gourh P FAU - Boin, Francesco AU - Boin F LA - eng GR - K23 AR061439/AR/NIAMS NIH HHS/United States GR - P50 AR054144/AR/NIAMS NIH HHS/United States GR - T32 AR048522/AR/NIAMS NIH HHS/United States GR - N01 AR002251/AR/NIAMS NIH HHS/United States GR - R03 AR065801/AR/NIAMS NIH HHS/United States GR - K01 AR067280/AR/NIAMS NIH HHS/United States GR - UL1 TR000062/TR/NCATS NIH HHS/United States GR - K24 AR063120/AR/NIAMS NIH HHS/United States GR - UL1 RR029882/RR/NCRR NIH HHS/United States GR - R01 AR055258/AR/NIAMS NIH HHS/United States GR - UL1 TR001450/TR/NCATS NIH HHS/United States GR - P60 AR062755/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Adult MH - Black or African American MH - Chromosome Mapping MH - Cohort Studies MH - Cross-Sectional Studies MH - Databases, Factual MH - Female MH - Humans MH - Male MH - Prevalence MH - Prospective Studies MH - Retrospective Studies MH - Scleroderma, Systemic/blood/*epidemiology/ethnology/physiopathology MH - Severity of Illness Index MH - Socioeconomic Factors MH - United States/epidemiology PMC - PMC5758130 COIS- The authors have no conflicts of interest to disclose. EDAT- 2018/02/03 06:00 MHDA- 2018/02/14 06:00 PMCR- 2017/12/22 CRDT- 2018/02/03 06:00 PHST- 2018/02/03 06:00 [entrez] PHST- 2018/02/03 06:00 [pubmed] PHST- 2018/02/14 06:00 [medline] PHST- 2017/12/22 00:00 [pmc-release] AID - 00005792-201712220-00015 [pii] AID - MD-D-17-05555 [pii] AID - 10.1097/MD.0000000000008980 [doi] PST - ppublish SO - Medicine (Baltimore). 2017 Dec;96(51):e8980. doi: 10.1097/MD.0000000000008980. PMID- 24151254 OWN - NLM STAT- MEDLINE DCOM- 20140612 LR - 20260518 IS - 2151-4658 (Electronic) IS - 2151-464X (Print) IS - 2151-464X (Linking) VI - 66 IP - 5 DP - 2014 May TI - Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies. PG - 732-40 LID - 10.1002/acr.22212 [doi] AB - OBJECTIVE: Because juvenile idiopathic inflammatory myopathies (IIMs) are potentially life-threatening systemic autoimmune diseases, we examined risk factors for juvenile IIM mortality. METHODS: Mortality status was available for 405 patients (329 with juvenile dermatomyositis [DM], 30 with juvenile polymyositis [PM], and 46 with juvenile connective tissue disease-associated myositis [CTM]) enrolled in nationwide protocols. Standardized mortality ratios (SMRs) were calculated using US population statistics. Cox regression analysis was used to assess univariable associations with mortality, and random survival forest (RSF) classification and Cox regression analysis were used for multivariable associations. RESULTS: Of 17 deaths (4.2% overall mortality), 8 (2.4%) were in juvenile DM patients. Death was related to the pulmonary system (primarily interstitial lung disease [ILD]) in 7 patients, gastrointestinal system in 3, and multisystem in 3, and of unknown etiology in 4 patients. The SMR for juvenile IIMs overall was 14.4 (95% confidence interval [95% CI] 12.2-16.5) and was 8.3 (95% CI 6.4-10.3) for juvenile DM. The top mortality risk factors in the univariable analysis included clinical subgroup (juvenile CTM, juvenile PM), antisynthetase autoantibodies, older age at diagnosis, ILD, and Raynaud's phenomenon at diagnosis. In multivariable analyses, clinical subgroup, illness severity at onset, age at diagnosis, weight loss, and delay to diagnosis were the most important predictors from RSF; clinical subgroup and illness severity at onset were confirmed by multivariable Cox regression analysis. CONCLUSION: Overall mortality was higher in juvenile IIM patients, and several early illness features were identified as risk factors. Clinical subgroup, antisynthetase autoantibodies, older age at diagnosis, and ILD are also recognized as mortality risk factors in adult myositis. CI - Copyright © 2014 by the American College of Rheumatology. FAU - Huber, Adam M AU - Huber AM AD - IWK Health Centre and Dalhousie University, Halifax, Nova Scotia, Canada. FAU - Mamyrova, Gulnara AU - Mamyrova G FAU - Lachenbruch, Peter A AU - Lachenbruch PA FAU - Lee, Julia A AU - Lee JA FAU - Katz, James D AU - Katz JD FAU - Targoff, Ira N AU - Targoff IN FAU - Miller, Frederick W AU - Miller FW FAU - Rider, Lisa G AU - Rider LG CN - Childhood Myositis Heterogeneity Collaborative Study Group LA - eng GR - R01 DK088114/DK/NIDDK NIH HHS/United States GR - Z01 ES101074/ImNIH/Intramural NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Intramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM MH - Adolescent MH - Adult MH - Child MH - Early Diagnosis MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Myositis/*diagnosis/*mortality MH - Risk Factors MH - Young Adult PMC - PMC4646219 MID - NIHMS735728 COIS- Conflicts of Interest: The authors do not have any conflicts of interest related to this work. FIR - Abramson, Leslie S IR - Abramson LS FIR - Albert, Daniel A IR - Albert DA FIR - Arabshahi, Bita IR - Arabshahi B FIR - Baer, Alan N IR - Baer AN FIR - Balboni, Imelda M IR - Balboni IM FIR - Blocker, William P IR - Blocker WP FIR - Bohnsack, John F IR - Bohnsack JF FIR - Borzy, Michael S IR - Borzy MS FIR - Botstein, Gary R IR - Botstein GR FIR - Bowyer, Suzanne IR - Bowyer S FIR - Burnham, Jon M IR - Burnham JM FIR - Carrasco, Ruy IR - Carrasco R FIR - Cartwright, Victoria W IR - Cartwright VW FIR - Chao, Chun Peng T IR - Chao CP FIR - Cron, Randy Q IR - Cron RQ FIR - De Guzman, Marietta M IR - De Guzman MM FIR - Eberhard, B Anne IR - Eberhard BA FIR - Edelheit, Barbara S IR - Edelheit BS FIR - Eggert, John F IR - Eggert JF FIR - Eichenfield, Andrew H IR - Eichenfield AH FIR - Elder, Melissa E IR - Elder ME FIR - Finkel, Terri H IR - Finkel TH FIR - Flatau, Irene IR - Flatau I FIR - Fuhlbrigge, Robert C IR - Fuhlbrigge RC FIR - Gabriel, Christos A IR - Gabriel CA FIR - Garwood, Vernon F IR - Garwood VF FIR - Gewanter, Harry L IR - Gewanter HL FIR - Goldmunz, Ellen A IR - Goldmunz EA FIR - Goldsmith, Donald P IR - Goldsmith DP FIR - Gordon, Gary V IR - Gordon GV FIR - Gospodinoff, Alexia C IR - Gospodinoff AC FIR - Gottlieb, Beth S IR - Gottlieb BS FIR - Griffin, Thomas A IR - Griffin TA FIR - Groh, Brandt P IR - Groh BP FIR - Haftel, Hillary M IR - Haftel HM FIR - Henrickson, Michael IR - Henrickson M FIR - Higgins, Gloria C IR - Higgins GC FIR - Hoeltzel, Mark F IR - Hoeltzel MF FIR - Hollister, J Roger IR - Hollister JR FIR - Hopp, Russell J IR - Hopp RJ FIR - Ilowite, Norman T IR - Ilowite NT FIR - James-Newton, Laura IR - James-Newton L FIR - Jansen, Anna IR - Jansen A FIR - Jarvis, James IR - Jarvis J FIR - Jerath, Rita S IR - Jerath RS FIR - Johnson, Courtney R IR - Johnson CR FIR - Kantor, Thomas V IR - Kantor TV FIR - Katona, Ildy M IR - Katona IM FIR - Kimura, Yukiko IR - Kimura Y FIR - Kingsbury, Daniel J IR - Kingsbury DJ FIR - Klein, Steven J IR - Klein SJ FIR - Knee, C Michael IR - Knee CM FIR - Knibbe, W Patrick IR - Knibbe WP FIR - Lasky, Andrew IR - Lasky A FIR - Levine, Johanan IR - Levine J FIR - Lindsley, Carol B IR - Lindsley CB FIR - Madson, Katherine L IR - Madson KL FIR - McCarthy, Paul L IR - McCarthy PL FIR - Mitchell, Stephen R IR - Mitchell SR FIR - Moallem, Hamid Jack IR - Moallem HJ FIR - Murphy, Frederick T IR - Murphy FT FIR - O'Hanlon, Terrance IR - O'Hanlon T FIR - Oral, Elif A IR - Oral EA FIR - Ostrov, Barbara E IR - Ostrov BE FIR - Pachman, Lauren M IR - Pachman LM FIR - Pappu, Ramesh IR - Pappu R FIR - Passo, Murray H IR - Passo MH FIR - Perez, Maria D IR - Perez MD FIR - Person, Donald A IR - Person DA FIR - Peterson, Karin S IR - Peterson KS FIR - Punaro, Marilynn G IR - Punaro MG FIR - Rabinovich, C Elga IR - Rabinovich CE FIR - Radis, Charles D IR - Radis CD FIR - Rennebohm, Robert M IR - Rennebohm RM FIR - Reuman, Peter D IR - Reuman PD FIR - Rivas-Chacon, Rafael F IR - Rivas-Chacon RF FIR - Rothman, Deborah IR - Rothman D FIR - Schikler, Kenneth N IR - Schikler KN FIR - Shaham, Bracha IR - Shaham B FIR - Sheets, Robert M IR - Sheets RM FIR - Sherry, David D IR - Sherry DD FIR - Sills, Edward M IR - Sills EM FIR - Sinal, Sara H IR - Sinal SH FIR - Sule, Sangeeta D IR - Sule SD FIR - Sundel, Robert P IR - Sundel RP FIR - Szer, Ilona IR - Szer I FIR - Taylor, Simeon I IR - Taylor SI FIR - Vehe, Richard K IR - Vehe RK FIR - Vogelgesang, Scott A IR - Vogelgesang SA FIR - Vogler, Larry B IR - Vogler LB FIR - Wallace, Carol A IR - Wallace CA FIR - Wargula, Jennifer C IR - Wargula JC FIR - White, Patience H IR - White PH FIR - Wilkenfeld, M Jack IR - Wilkenfeld MJ FIR - Wu, Lan IR - Wu L FIR - Zemel, Lawrence S IR - Zemel LS EDAT- 2013/10/24 06:00 MHDA- 2014/06/13 06:00 PMCR- 2015/11/16 CRDT- 2013/10/24 06:00 PHST- 2013/07/04 00:00 [received] PHST- 2013/10/15 00:00 [accepted] PHST- 2013/10/24 06:00 [entrez] PHST- 2013/10/24 06:00 [pubmed] PHST- 2014/06/13 06:00 [medline] PHST- 2015/11/16 00:00 [pmc-release] AID - 10.1002/acr.22212 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2014 May;66(5):732-40. doi: 10.1002/acr.22212. PMID- 41034340 OWN - NLM STAT- MEDLINE DCOM- 20251023 LR - 20251023 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 21 IP - 11 DP - 2025 Nov TI - The role of the patient in rheumatology. PG - 651-656 LID - 10.1038/s41584-025-01303-z [doi] AB - In this Viewpoint article, six patients and patient advocates discuss the role of the patient in rheumatology, the current unmet needs of patients and promising advances. By reflecting on their own lived experiences, the authors emphasize the integral role of patients for progress in the field. CI - © 2025. Springer Nature Limited. FAU - Andersen, Jeanette AU - Andersen J AUID- ORCID: 0000-0001-8848-9039 AD - Lupus Europe, Knebel, Denmark. jeanette@lupus-europe.org. FAU - Church, Janet AU - Church J AD - The Sjögren's Foundation, Reston, VA, USA. jchurch@sjogrens.org. FAU - Durrant, Seth AU - Durrant S AUID- ORCID: 0009-0004-5271-7596 AD - Autoinflammatory Alliance, San Francisco, CA, USA. srd923@yahoo.com. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK. sue.farrington@sruk.co.uk. FAU - Okochi, Noriko AU - Okochi N AD - Rheumatic Disease and Vasculitis Support Network Japan, Tokyo, Japan. noriko.okochi@kosapo.org. AD - Graduate School of Psychology, Tokyo Seitoku University, Tokyo, Japan. noriko.okochi@kosapo.org. FAU - Trehan, Natasha AU - Trehan N AD - Take a Pain Check Foundation, Toronto, Ontario, Canada. natasha@takeapaincheck.com. LA - eng PT - Journal Article DEP - 20251001 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - Humans MH - *Rheumatology MH - *Rheumatic Diseases/therapy/psychology MH - *Patient Participation MH - *Patient Advocacy MH - Female COIS- Competing interests: N.T. is the executive director of Take a Pain Check Foundation, which has received grant support from Pfizer, Janssen, AbbVie, McMaster University, the Ontario Brain Institute, the Hospital of the University of Pennsylvania, Accord, UCB, Takeda, Augurex, Amgen, AstraZeneca and Organon. N.T. was a consultant for Janssen and AbbVie. EDAT- 2025/10/02 00:28 MHDA- 2025/10/23 06:27 CRDT- 2025/10/01 23:30 PHST- 2025/09/04 00:00 [accepted] PHST- 2025/10/23 06:27 [medline] PHST- 2025/10/02 00:28 [pubmed] PHST- 2025/10/01 23:30 [entrez] AID - 10.1038/s41584-025-01303-z [pii] AID - 10.1038/s41584-025-01303-z [doi] PST - ppublish SO - Nat Rev Rheumatol. 2025 Nov;21(11):651-656. doi: 10.1038/s41584-025-01303-z. Epub 2025 Oct 1. PMID- 40764937 OWN - NLM STAT- MEDLINE DCOM- 20250806 LR - 20250808 IS - 1752-1947 (Electronic) IS - 1752-1947 (Linking) VI - 19 IP - 1 DP - 2025 Aug 5 TI - Anti-centromere antibody positivity with coexisting idiopathic portal hypertension and primary biliary cholangitis progressing to limited cutaneous systemic sclerosis: a case report. PG - 392 LID - 10.1186/s13256-025-05426-5 [doi] LID - 392 AB - BACKGROUND: Anti-centromere antibodies are autoantibodies that selectively bind to the centromere region of chromosomes. Studies have indicated that anti-centromere antibodies can induce microvascular alterations and tissue remodeling, ultimately leading to fibrosis. They have been implicated in limited cutaneous systemic sclerosis, including calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome, and primary biliary cholangitis, where anti-centromere antibody positivity can be associated with rapid progression of portal hypertension, although the underlying mechanisms remain unclear. Idiopathic portal hypertension, despite being termed "idiopathic," has distinctive pathological, angiographic, and ultrasound findings, and autoimmune processes have been proposed to mediate its intrahepatic microcirculatory disruptions. Interestingly, idiopathic portal hypertension-related small portal vein and scleroderma skin findings share certain similarities. However, no documented cases have linked anti-centromere antibody-induced intrahepatic vascular endothelial dysfunction to idiopathic portal hypertension and subsequent progression to limited cutaneous systemic sclerosis. CASE PRESENTATION: A 57-year-old Japanese woman was referred to our hospital with suspected anti-centromere antibody-positive primary biliary cholangitis. Further examination revealed the coexistence of idiopathic portal hypertension, and the patient progressed to limited cutaneous systemic sclerosis over 3 years. On the basis of this case, we suspected that anti-centromere antibodies might cause microvascular endothelial dysfunction, leading to the development of idiopathic portal hypertension and other systemic abnormalities. Supplementary tests were performed to verify this hypothesis, including flow-mediated vasodilation, brachial-ankle pulse wave velocity, nailfold video capillaroscopy, upper gastrointestinal endoscopy, pathological CD34 and indoleamine 2,3-dioxygenase 1 staining, and measurements of soluble lectin-like oxidized low-density lipoprotein receptor-1 and its ligand containing apolipoprotein B. The results indicated vascular abnormalities in the liver, skin, and gastrointestinal tract, highlighting the universal effects of anti-centromere antibodies in vascular and autoimmune pathologies. CONCLUSION: This is the first documented case of hepatic and systemic microvascular impairment observed in an anti-centromere antibody-positive patient. The pathological evidence of endothelial damage in the liver suggests that the "idiopathic" label of idiopathic portal hypertension may need reconsideration in the context of anti-centromere antibody-related pathophysiology, potentially warranting a unifying concept such as "anti-centromere antibody-related systemic microangiopathy syndrome." While our case may provide novel insights into anti-centromere antibody-driven microvascular dysfunction across multiple organ systems, the findings are preliminary. Future studies involving larger cohorts and detailed mechanistic analyses are necessary to confirm the systemic and hepatic effects of anti-centromere antibodies. CI - © 2025. The Author(s). FAU - Ando, Yuya AU - Ando Y AUID- ORCID: 0000-0002-1449-0464 AD - Department of Internal Medicine, Self-Defense Forces Central Hospital, Setagaya-Ku, Tokyo, 154-0001, Japan. AD - Department of Gastroenterology, Mishuku Hospital, Meguro-Ku, Tokyo, 153-0051, Japan. AD - Department of General Medicine, Institute of Science Tokyo, 1-5-45 Yushima, Bunkyo-Ku, Tokyo, 113-8510, Japan. FAU - Mabuchi, Suguru AU - Mabuchi S AUID- ORCID: 0000-0002-8620-5191 AD - Department of General Medicine, Institute of Science Tokyo, 1-5-45 Yushima, Bunkyo-Ku, Tokyo, 113-8510, Japan. mabuvasc@tmd.ac.jp. FAU - Mataki, Norikazu AU - Mataki N AD - Department of Internal Medicine, Self-Defense Forces Central Hospital, Setagaya-Ku, Tokyo, 154-0001, Japan. AD - Department of Gastroenterology, Mishuku Hospital, Meguro-Ku, Tokyo, 153-0051, Japan. FAU - Nakayama, Satoshi AU - Nakayama S AUID- ORCID: 0000-0003-1861-1961 AD - Department of Gastroenterology, Mishuku Hospital, Meguro-Ku, Tokyo, 153-0051, Japan. FAU - Honda, Arata AU - Honda A AD - Department of Gastroenterology, Mishuku Hospital, Meguro-Ku, Tokyo, 153-0051, Japan. FAU - Kamiya, Mari AU - Kamiya M AUID- ORCID: 0000-0002-2093-3823 AD - Department of Rheumatology, Institute of Science Tokyo, Bunkyo-Ku, Tokyo, 113-8510, Japan. FAU - Yasuda, Shinsuke AU - Yasuda S AUID- ORCID: 0000-0001-6171-2077 AD - Department of Rheumatology, Institute of Science Tokyo, Bunkyo-Ku, Tokyo, 113-8510, Japan. FAU - Takeo, Hiroaki AU - Takeo H AD - Department of Pathology, Self-Defense Forces Central Hospital, Setagaya-Ku, Tokyo, 154-0001, Japan. FAU - Aono, Shigeaki AU - Aono S AD - Department of Internal Medicine, Self-Defense Forces Central Hospital, Setagaya-Ku, Tokyo, 154-0001, Japan. FAU - Hashimoto, Masayoshi AU - Hashimoto M AD - Department of General Medicine, Institute of Science Tokyo, 1-5-45 Yushima, Bunkyo-Ku, Tokyo, 113-8510, Japan. FAU - Harada, Kenichi AU - Harada K AUID- ORCID: 0000-0002-6451-0638 AD - Department of Human Pathology, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Ishikawa, 920-1192, Japan. FAU - Murashima, Naoya AU - Murashima N AD - Department of Gastroenterology, Mishuku Hospital, Meguro-Ku, Tokyo, 153-0051, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20250805 PL - England TA - J Med Case Rep JT - Journal of medical case reports JID - 101293382 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) RN - 0 (anticentromere antibody) SB - IM MH - Humans MH - Female MH - Middle Aged MH - *Hypertension, Portal/complications/immunology MH - *Liver Cirrhosis, Biliary/complications/immunology MH - *Centromere/immunology MH - *Autoantibodies/blood/immunology MH - Disease Progression MH - *Antibodies, Antinuclear/blood MH - *Scleroderma, Systemic MH - *Scleroderma, Limited/immunology MH - Idiopathic Noncirrhotic Portal Hypertension PMC - PMC12326789 OTO - NOTNLM OT - Anti-centromere antibody OT - CREST syndrome OT - Idiopathic portal hypertension OT - Intrapapillary capillary loop OT - Liver cirrhosis OT - Nail capillary microscopy OT - Primary biliary cholangitis OT - Vascular endothelial dysfunction COIS- Declarations. Ethics approval and consent to participate: Not applicable. Consent for publication: Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare that they have no competing interests. EDAT- 2025/08/06 06:28 MHDA- 2025/08/06 06:29 PMCR- 2025/08/05 CRDT- 2025/08/06 00:55 PHST- 2024/12/26 00:00 [received] PHST- 2025/07/10 00:00 [accepted] PHST- 2025/08/06 06:29 [medline] PHST- 2025/08/06 06:28 [pubmed] PHST- 2025/08/06 00:55 [entrez] PHST- 2025/08/05 00:00 [pmc-release] AID - 10.1186/s13256-025-05426-5 [pii] AID - 5426 [pii] AID - 10.1186/s13256-025-05426-5 [doi] PST - epublish SO - J Med Case Rep. 2025 Aug 5;19(1):392. doi: 10.1186/s13256-025-05426-5. PMID- 40514051 OWN - NLM STAT- MEDLINE DCOM- 20250613 LR - 20250730 IS - 2053-8790 (Print) IS - 2053-8790 (Electronic) IS - 2053-8790 (Linking) VI - 12 IP - 1 DP - 2025 Jun 12 TI - Pulmonary arterial hypertension in systemic lupus erythematosus: identification of risk factors and haemodynamics characteristics in a multicentre retrospective cohort. LID - 10.1136/lupus-2024-001471 [doi] LID - e001471 AB - OBJECTIVES: The aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE). METHODS: We identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC). RESULTS: All patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud's phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9-14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries. CONCLUSIONS: Our data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Marasco, Emiliano AU - Marasco E AUID- ORCID: 0000-0001-5346-3357 AD - Division of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, Italy. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Düsing, Christina AU - Düsing C AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany. AD - Rheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, Germany. FAU - Keymel, Stefanie AU - Keymel S AD - Department of Cardiology, Pneumology and Angiology, Heinrich-Heine-Universitat Dusseldorf, Dusseldorf, Germany. FAU - Bortoluzzi, Alessandra AU - Bortoluzzi A AD - Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy. FAU - Bracaglia, Claudia AU - Bracaglia C AD - Division of Rheumatology, Bambino Gesu Pediatric Hospital IRCCS, Roma, Italy. FAU - Canuet, Matthieu AU - Canuet M AD - Service de Pneumologie Centre de Compétence de l'Hypertension Artérielle Pulmonaire, Les Hôpitaux Universitaires de Strasbourg, Strasbourg, France. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, Italy. FAU - Chehab, Gamal AU - Chehab G AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany. AD - Rheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, Germany. FAU - Codullo, Veronica AU - Codullo V AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Fischer, Rebecca AU - Fischer R AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, Italy. FAU - Fredi, Micaela AU - Fredi M AUID- ORCID: 0000-0002-6511-4936 AD - Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and Clinical and Experimental Science Department, University of Brescia, Brescia, Italy. FAU - Ghio, Stefano AU - Ghio S AD - Division of Cardiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Keller, Lisa AU - Keller L AD - Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany. FAU - Meyer, Alain AU - Meyer A AD - Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Strasbourg University Hospitals, Strasbourg, France. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Division of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, Italy. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Richter, Jutta AU - Richter J AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany. AD - Rheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, Germany. FAU - Riou, Marianne AU - Riou M AD - Service de Pneumologie Centre de Compétence de l'Hypertension Artérielle Pulmonaire, Les Hôpitaux Universitaires de Strasbourg, Strasbourg, France. FAU - Sahin, Sezgin AU - Sahin S AD - Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey. FAU - Sander, Oliver AU - Sander O AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany. AD - Rheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, Germany. FAU - Schwarting, Andreas AU - Schwarting A AD - Rheumatology, Rheumatology Center Rhineland-Palatinate, Bad Kreuznach, Germany. FAU - Scirè, Carlo Alberto AU - Scirè CA AD - Division of Rheumatology, University of Milan-Bicocca, Milano, Italy. FAU - Silvagni, Ettore AU - Silvagni E AUID- ORCID: 0000-0001-7654-8222 AD - Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy. FAU - Triantafyllias, Konstantinos AU - Triantafyllias K AD - Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany. AD - Rheumatology, Rheumatology Center Rhineland-Palatinate, Bad Kreuznach, Germany. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Division of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, Italy. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Division of Rheumatology, Department of Internal Medicine and Therapeutic, University of Pavia, Pavia, Italy lorenzo.cavagna@unipv.it karl.schneider@hhu.de. AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Schneider, Matthias AU - Schneider M AD - Department for Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine-University, Düsseldorf, Germany lorenzo.cavagna@unipv.it karl.schneider@hhu.de. AD - Rheumatology, Heinrich Heine University Düsseldorf, Dusseldorf, Germany. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20250612 PL - England TA - Lupus Sci Med JT - Lupus science & medicine JID - 101633705 RN - 0 (Lupus Coagulation Inhibitor) SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/complications/physiopathology MH - Female MH - Adult MH - Retrospective Studies MH - Risk Factors MH - Middle Aged MH - Microscopic Angioscopy MH - *Pulmonary Arterial Hypertension/physiopathology/etiology/diagnosis MH - Male MH - Lupus Coagulation Inhibitor/blood MH - Hemodynamics MH - *Hypertension, Pulmonary/etiology/physiopathology/diagnosis MH - Case-Control Studies MH - Nails/blood supply PMC - PMC12164622 OTO - NOTNLM OT - Lupus Erythematosus, Systemic OT - Pulmonary Arterial Hypertension OT - Risk Factors COIS- Competing interests: None declared. EDAT- 2025/06/14 16:45 MHDA- 2025/06/14 16:46 PMCR- 2025/06/12 CRDT- 2025/06/13 20:43 PHST- 2024/12/03 00:00 [received] PHST- 2025/04/25 00:00 [accepted] PHST- 2025/06/14 16:46 [medline] PHST- 2025/06/14 16:45 [pubmed] PHST- 2025/06/13 20:43 [entrez] PHST- 2025/06/12 00:00 [pmc-release] AID - 12/1/e001471 [pii] AID - lupus-2024-001471 [pii] AID - 10.1136/lupus-2024-001471 [doi] PST - epublish SO - Lupus Sci Med. 2025 Jun 12;12(1):e001471. doi: 10.1136/lupus-2024-001471. PMID- 40923484 OWN - NLM STAT- MEDLINE DCOM- 20250909 LR - 20250909 IS - 2146-3077 (Electronic) IS - 1300-6320 (Linking) VI - 49 IP - 3 DP - 2025 Sep 8 TI - Efficacy of Medicinal Leech Therapy in Diverse Clinical Applications: A Comprehensive Study from Azerbaijan. PG - 120-128 LID - 10.4274/tpd.galenos.2025.32559 [doi] AB - OBJECTIVE: Hirudotherapy (HT), the therapeutic use of medicinal leeches, has been practised for centuries, and the interest in modern medicine has recently been renewed. This study evaluates the clinical outcomes of HT at Herba Medical Center in Azerbaijan between 2020 and 2024, focusing on its efficacy across 11 medical conditions. METHODS: A total of 181 patients were treated using disposable medicinal leeches (Hirudo orientalis) sourced from hygienic farms approved by Azerbaijan's Ministry of Ecology and Natural Resources. Treatment protocols were tailored to disease severity, with sessions scheduled daily, every 3 days, or weekly, depending on the condition. Success rates were calculated based on post-treatment examinations, patient feedback, and physician evaluations. Statistical analyses, including Pearson correlation analysis and paired t-test, were used to compare treatment success rates between conditions. RESULTS: The overall success rate of HT was found to be 82.68±29.25%. 100% success was achieved in the treatment of osteoarthritis pain (n=50), lipoma (n=8), Raynaud disease (n=3) and scleroderma (n=2). High success rates were also observed in thyroiditis (94.44%, n=18), Baker's cyst (80%, n=25), ear diseases (80%, n=10) and diabetic foot ulcers (80%, n=5). Moderate success was achieved in eye diseases (75%, n=20), and the lowest efficacy was observed in the treatment of varicose veins (33.33%, n=30). HT effectively relieved pain and improved symptoms. However, it was limited in reversing structural deformities (e.g., hallux valgus) or tissue loss (e.g., diabetic foot ulcers). CONCLUSION: These findings suggest that HT may have broader indications. We propose that HT can effectively relieve pain, regulate blood circulation, and treat some chronic diseases with fewer side effects. Further and more detailed research is needed to understand the mechanism of this treatment method better. FAU - Farzali, Shabnam AU - Farzali S AUID- ORCID: 0000-0002-8118-9516 AD - Fırat University Faculty of Fisheries, Department of Aquaculture and Fish Diseases, Elazığ, Türkiye. FAU - Yaraliyeva, Sudaba AU - Yaraliyeva S AUID- ORCID: 0000-0002-9886-8697 AD - Herba Medical Center, Baku, Azerbaijan. FAU - Huseynov, Fizuli AU - Huseynov F AUID- ORCID: 0000-0002-4301-9604 AD - Herba Medical Center, Baku, Azerbaijan. FAU - Manafov, Asif AU - Manafov A AUID- ORCID: 0000-0001-7392-6136 AD - National Academy of Sciences of Azerbaijan, Institute of Zoology, Baku, Azerbaijan. FAU - Sağlam, Naim AU - Sağlam N AUID- ORCID: 0000-0002-3163-8432 AD - Fırat University Faculty of Fisheries, Department of Aquaculture and Fish Diseases, Elazığ, Türkiye. LA - eng PT - Journal Article PL - Turkey TA - Turkiye Parazitol Derg JT - Turkiye parazitolojii dergisi JID - 9425544 SB - IM MH - Humans MH - *Leeching/methods MH - Male MH - Azerbaijan MH - Female MH - Treatment Outcome MH - Middle Aged MH - Animals MH - Adult MH - Aged MH - Young Adult MH - Adolescent MH - *Hirudo medicinalis OTO - NOTNLM OT - Hirudotherapy OT - chronic diseases OT - complementary treatment OT - leech therapy OT - pain management OT - traditional medicine COIS- Conflict of Interest: No conflict of interest was declared by the authors. EDAT- 2025/09/09 12:31 MHDA- 2025/09/09 12:32 CRDT- 2025/09/09 07:01 PHST- 2025/09/09 12:32 [medline] PHST- 2025/09/09 12:31 [pubmed] PHST- 2025/09/09 07:01 [entrez] AID - 10.4274/tpd.galenos.2025.32559 [doi] PST - ppublish SO - Turkiye Parazitol Derg. 2025 Sep 8;49(3):120-128. doi: 10.4274/tpd.galenos.2025.32559. PMID- 40685839 OWN - NLM STAT- MEDLINE DCOM- 20260316 LR - 20260319 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 78 IP - 3 DP - 2026 Mar TI - Case-Based Immunology: B Cells and Systemic Sclerosis Interstitial Lung Disease. PG - 566-581 LID - 10.1002/art.43326 [doi] AB - Interstitial lung disease (ILD) is an important complication of systemic sclerosis (SSc), with high mortality and morbidity. Recent clinical studies in SSc-ILD have led to US Food and Drug Administration-approved therapies in SSc-ILD. Importantly, evidence from these studies has been extrapolated to guide management of ILDs of other systemic autoimmune rheumatic diseases. Pathogenesis of SSc-ILD involves interplay between fibroblasts and the innate and adaptive immune system. A central role for the B cell compartment is supported by clinical and translational studies. We use a case from our center as a basis to discuss the pathogenesis of SSc-ILD, autoantibodies in SSc-ILD, and the role of B cells in the disease. We go on to consider treatment options for the case, the decision-making algorithm for treatment, and risks associated with treatment. CI - © 2025 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Goldman, Nina AU - Goldman N AUID- ORCID: 0000-0002-6800-4230 AD - Centre for Rheumatology, Division of Medicine, University College London, London, United Kingdom. FAU - Ong, Voon AU - Ong V AD - Centre for Rheumatology, Division of Medicine, University College London, London, United Kingdom. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Division of Medicine, University College London, London, United Kingdom. LA - eng GR - Scleroderma and Raynaud's UK/ GR - MR/V030108/1/MRC_/Medical Research Council/United Kingdom PT - Case Reports PT - Journal Article PT - Review DEP - 20251202 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Scleroderma, Systemic/immunology/complications MH - *Lung Diseases, Interstitial/immunology/etiology/drug therapy MH - *B-Lymphocytes/immunology MH - Autoantibodies/immunology MH - Middle Aged MH - Female MH - Male PMC - PMC12991924 EDAT- 2025/07/21 06:30 MHDA- 2026/03/17 00:33 PMCR- 2026/03/17 CRDT- 2025/07/21 03:03 PHST- 2025/07/01 00:00 [revised] PHST- 2024/09/26 00:00 [received] PHST- 2025/07/10 00:00 [accepted] PHST- 2026/03/17 00:33 [medline] PHST- 2025/07/21 06:30 [pubmed] PHST- 2025/07/21 03:03 [entrez] PHST- 2026/03/17 00:00 [pmc-release] AID - ART43326 [pii] AID - 10.1002/art.43326 [doi] PST - ppublish SO - Arthritis Rheumatol. 2026 Mar;78(3):566-581. doi: 10.1002/art.43326. Epub 2025 Dec 2. PMID- 37676716 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230924 IS - 2562-0959 (Electronic) IS - 2562-0959 (Linking) VI - 6 DP - 2023 Sep 7 TI - Public Health Risks, Dermatological Manifestations, and Environmental Justice Associated With Vinyl Chloride Exposure: Narrative Review. PG - e48998 LID - 10.2196/48998 [doi] LID - e48998 AB - BACKGROUND: Environmental vinyl chloride (VC) exposure may result in serious acute and chronic dermatological conditions. Because existing literature largely focuses on exposures in occupational settings, a gap persists in our understanding of the medical consequences of large-scale chemical spills. OBJECTIVE: This study aims to examine the potential dermatological manifestations of VC exposure in the context of industrial spills and other environmental disasters and to highlight the public health and justice implications of such releases. METHODS: In this narrative review, relevant evidence-based, peer-reviewed scientific sources, gray literature, and media reports were identified via searches of search PubMed and Google using predetermined keyword search terms related to VC, VC spills and releases, train derailment, cutaneous disease, public health, and vulnerable and marginalized populations. RESULTS: Contact dermatitis and frostbite may arise acutely, highlighting the importance of swift decontamination. Long-term manifestations from chronic VC exposure due to persistence in environmental reservoirs include Raynaud disease, sclerodermatous skin changes, acro-osteolysis, and cutaneous malignancies. The clinical severity of cutaneous manifestations is influenced by individual susceptibility as well as duration, intensity, and route of exposure. Additionally, chemical releases of VC more frequently impact Communities of Color and those of lower socioeconomic status, resulting in greater rates of exposure-related disease. CONCLUSIONS: With environmental release events of hazardous chemicals becoming increasingly common and because the skin has increased contact with environmental toxins relative to other organs, an urgent need exists for a greater understanding of the overall short- and long-term health impacts of large-scale, toxic exposures, underscoring the need for ongoing clinical vigilance. Dermatologists and public health officials should also aim to better understand the ways in which the disproportionate impacts of hazardous chemical exposures on lower-income and minority populations may exacerbate existing health disparities. Herein, we describe the health implications of toxic releases with particular consideration paid to marginalized and vulnerable populations. In addition to legal and regulatory frameworks, we advocate for improved public health measures, to not only mitigate the risk of environmental catastrophes in the future, but also ensure timely and effective responses to them. CI - ©Rachel S Goodman, Lavanya Mittal, Eva Rawlings Parker. Originally published in JMIR Dermatology (http://derma.jmir.org), 07.09.2023. FAU - Goodman, Rachel S AU - Goodman RS AUID- ORCID: 0000-0001-7992-8108 AD - School of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States. FAU - Mittal, Lavanya AU - Mittal L AUID- ORCID: 0009-0004-6473-5468 AD - The Ronald O Perelman Department of Dermatology, Grossman School of Medicine, New York University, New York, NY, United States. FAU - Parker, Eva Rawlings AU - Parker ER AUID- ORCID: 0000-0002-2462-5051 AD - Department of Dermatology, Vanderbilt University Medical Center, Nashville, TN, United States. AD - Center for Biomedical Ethics and Society, Vanderbilt University Medical Center, Nashville, TN, United States. LA - eng PT - Journal Article PT - Review DEP - 20230907 PL - Canada TA - JMIR Dermatol JT - JMIR dermatology JID - 101770607 PMC - PMC10514763 OTO - NOTNLM OT - acute OT - chronic OT - climate advocacy OT - community OT - cutaneous manifestations OT - dermatology OT - environmental health OT - environmental toxins OT - hazardous chemicals OT - industrial accident OT - malignancy OT - public health OT - socioeconomic OT - toxicology OT - utilization OT - vinyl chloride COIS- Conflicts of Interest: RSG receives funding from the Supporting Careers in Research for Interventional Physicians and Surgeons (SCRIPS) Grant and Burroughs Wellcome Fund. EDAT- 2023/09/07 12:42 MHDA- 2023/09/07 12:43 PMCR- 2023/09/07 CRDT- 2023/09/07 11:53 PHST- 2023/05/14 00:00 [received] PHST- 2023/08/15 00:00 [accepted] PHST- 2023/08/06 00:00 [revised] PHST- 2023/09/07 12:43 [medline] PHST- 2023/09/07 12:42 [pubmed] PHST- 2023/09/07 11:53 [entrez] PHST- 2023/09/07 00:00 [pmc-release] AID - v6i1e48998 [pii] AID - 10.2196/48998 [doi] PST - epublish SO - JMIR Dermatol. 2023 Sep 7;6:e48998. doi: 10.2196/48998. PMID- 33183531 OWN - NLM STAT- MEDLINE DCOM- 20210924 LR - 20210924 IS - 2405-4577 (Electronic) IS - 2405-4577 (Linking) VI - 40 DP - 2020 Dec TI - Nutritional status and predictors of weight loss in patients with systemic sclerosis. PG - 164-170 LID - S2405-4577(20)30217-5 [pii] LID - 10.1016/j.clnesp.2020.09.030 [doi] AB - BACKGROUND & AIMS: Systemic sclerosis (SSc) commonly affects the gastrointestinal (GI) tract and predisposes to malnutrition. Few studies assessed body composition in outpatients with SSc or used more than one method for comparison over time. The aim of this study was to describe markers of nutrition and body composition in patients with SSc and to identify predictors of unintentional weight loss. METHODS: We consecutively included outpatients with SSc and performed a one-year follow-up. Gastrointestinal (GI) involvement was evaluated from clinical investigations. Patients completed questionnaires for organ involvement and functional status. Clinical assessment included body mass index (BMI), the malnutrition universal screening tool (MUST), inter-incisor distance, anthropometry, and bio-electrical impedance analysis (BIA). RESULTS: In total, 168 consecutive patients with SSc were included, and 127 (76%) completed one-year follow-up. Thirteen (8%) died before follow-up. Based on MUST scores, 12% of patients were at high and 14% at medium risk of malnutrition. A low BMI was associated with small intestinal involvement (p < 0.0001). Percentage body fat correlated with BMI, both when using four-site anthropometry (r = 0.65, p < 0.01) and BIA (r = 0.49, p < 0.01). Nine (7%) patients had >5% unintentional weight loss at follow-up. Independent baseline predictors of unintentional weight loss included upper GI involvement and disease severity estimated by Health Assessment Questionnaire Disability Index score. CONCLUSIONS: Nutritional risk and GI involvement are frequent and closely correlated in patients with SSc. BIA and four-site anthropometry are comparable in the clinical assessment of patients with SSc. Unintentional weight loss is discrete and related to disease-specific characteristics. CI - Copyright © 2020 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved. FAU - Hvas, Christian L AU - Hvas CL AD - Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark. Electronic address: christian.hvas@auh.rm.dk. FAU - Harrison, Elizabeth AU - Harrison E AD - Department of Gastroenterology, Shrewsbury and Telford Hospitals NHS Trust, Shrewsbury, United Kingdom. FAU - Eriksen, Marcel K AU - Eriksen MK AD - Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark. FAU - Herrick, Ariane L AU - Herrick AL AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, M13 93, UK. FAU - McLaughlin, John T AU - McLaughlin JT AD - Division of Diabetes, Endocrinology and Gastroenterology, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, M13 9NT, UK. FAU - Lal, Simon AU - Lal S AD - Division of Diabetes, Endocrinology and Gastroenterology, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, M13 9NT, UK; Intestinal Failure Unit, Salford Royal NHS Foundation Trust, Salford, UK. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20201006 PL - England TA - Clin Nutr ESPEN JT - Clinical nutrition ESPEN JID - 101654592 SB - IM MH - Body Composition MH - Humans MH - *Malnutrition/diagnosis/epidemiology MH - Nutritional Status MH - *Scleroderma, Systemic/diagnosis MH - Weight Loss OTO - NOTNLM OT - Body composition OT - Malnutrition OT - Nutritional assessment OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of competing interest EH was funded by the Raynaud's and Scleroderma Association. All other authors declare no conflicts of interest. EDAT- 2020/11/14 06:00 MHDA- 2021/09/25 06:00 CRDT- 2020/11/13 05:34 PHST- 2020/09/19 00:00 [received] PHST- 2020/09/24 00:00 [accepted] PHST- 2020/11/13 05:34 [entrez] PHST- 2020/11/14 06:00 [pubmed] PHST- 2021/09/25 06:00 [medline] AID - S2405-4577(20)30217-5 [pii] AID - 10.1016/j.clnesp.2020.09.030 [doi] PST - ppublish SO - Clin Nutr ESPEN. 2020 Dec;40:164-170. doi: 10.1016/j.clnesp.2020.09.030. Epub 2020 Oct 6. PMID- 39544898 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260129 LR - 20260129 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 1 DP - 2025 Feb TI - An evaluation of autonomic and gastrointestinal symptoms, and gastric emptying, in patients with systemic sclerosis. PG - 42-49 LID - 10.1177/23971983241288039 [doi] AB - OBJECTIVE: Assessment of gastrointestinal and autonomic symptoms in patients with systemic sclerosis, and possible associations with gastric emptying rate. METHODS: Participant and patient disease-related characteristics were collected. Gastrointestinal and autonomic symptoms were assessed by the UCLA-SCTC GIT 2.0 and COMPASS-31 questionnaires, respectively. Potentially confounding gastrointestinal medications were discontinued where possible. Gastric emptying was assessed using a non-radioactive (13)C sodium acetate isotope, end-expiratory breath samples collected at baseline and then serial timepoints up to 120 min. RESULTS: In total, 49 participants were studied: 17 with systemic sclerosis with variable gastrointestinal involvement, and healthy matched (n = 17) and non-matched controls (n = 15), the last to control for the impact of age rather than disease on gastric emptying and autonomic function. The total mean (range) UCLA GIT 2.0 questionnaire for patients with systemic sclerosis was 0.63 (0.0-1.5) and for both healthy matched and non-matched controls was 0.04 (0.0-0.2), and was higher in patients with systemic sclerosis across all domains. The total mean (range) COMPASS-31 score for patients with systemic sclerosis patients was 32.2 (0.0-54.9) and for healthy matched- and non-matched controls: 7.45 (0.0-24.9) and 4.25 (0.0-2.1), respectively, again higher for patients with systemic sclerosis across all domains. No association was observed between patients' UCLA GIT 2.0 total score (s = -0.039, p = 0.38), total COMPASS 31 score (s = -0.108, p = 0.68), or COMPASS-31 GI domain (s = -0.051, p = 0.85) and gastric emptying rates. CONCLUSION: Gastrointestinal and autonomic symptoms are overrepresented in patients with systemic sclerosis but did not associate with gastric emptying rates. CI - © The Author(s) 2024. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Harrison, Elizabeth AU - Harrison E AD - Shrewsbury and Telford Hospitals NHS Trust, Shrewsbury, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Lal, Simon AU - Lal S AD - Division of Diabetes, Endocrinology and Gastroenterology, Faculty of Biology, Medicine and Health, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Manchester, UK. AD - Intestinal Failure Unit, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK. FAU - McLaughlin, John T AU - McLaughlin JT AD - Division of Diabetes, Endocrinology and Gastroenterology, Faculty of Biology, Medicine and Health, The University of Manchester, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Manchester, UK. LA - eng PT - Journal Article DEP - 20241024 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC11559523 OTO - NOTNLM OT - Systemic sclerosis OT - autonomic dysfunction OT - gastric emptying OT - gastrointestinal OT - scleroderma COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: M.H. received none. E.H. was funded by the Raynaud’s and Scleroderma Association. A.L.H. received consultancy fees from Arena, Boehringer Ingelheim, Camurus, Galderma and Gesynta Pharma, speaker fees from Janssen and research funding from Gesynta Pharma. S.L. and J.T.M. received none. EDAT- 2024/11/15 06:25 MHDA- 2024/11/15 06:26 PMCR- 2025/10/24 CRDT- 2024/11/15 04:34 PHST- 2024/08/02 00:00 [received] PHST- 2024/09/13 00:00 [accepted] PHST- 2024/11/15 06:26 [medline] PHST- 2024/11/15 06:25 [pubmed] PHST- 2024/11/15 04:34 [entrez] PHST- 2025/10/24 00:00 [pmc-release] AID - 10.1177_23971983241288039 [pii] AID - 10.1177/23971983241288039 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2024 Oct 24;10(1):42-49. doi: 10.1177/23971983241288039. eCollection 2025 Feb. PMID- 21821511 OWN - NLM STAT- MEDLINE DCOM- 20120716 LR - 20220408 IS - 1526-7598 (Electronic) IS - 0003-2999 (Linking) VI - 113 IP - 4 DP - 2011 Oct TI - Continuous peripheral nerve blocks: a review of the published evidence. PG - 904-25 LID - 10.1213/ANE.0b013e3182285e01 [doi] AB - A continuous peripheral nerve block, also termed "perineural local anesthetic infusion," involves the percutaneous insertion of a catheter adjacent to a peripheral nerve, followed by local anesthetic administration via the catheter, providing anesthesia/analgesia for multiple days or even months. Continuous peripheral nerve blocks may be provided in the hospital setting, but the use of lightweight, portable pumps permits ambulatory infusion as well. This technique's most common application is providing analgesia after surgical procedures. However, additional indications include treating intractable hiccups; inducing a sympathectomy and vasodilation to increase blood flow after a vascular accident, digit transfer/replantation, or limb salvage; alleviating vasospasm of Raynaud disease; and treating peripheral embolism and chronic pain such as complex regional pain syndrome, phantom limb pain, trigeminal neuralgia, and cancer-induced pain. After trauma, perineural infusion can provide analgesia during transportation to a distant treatment center, or while simply awaiting surgical repair. Catheter insertion may be accomplished using many possible modalities, including nerve stimulation, ultrasound guidance, paresthesia induction, fluoroscopic imaging, and simple tactile perceptions ("facial click"). Either a nonstimulating epidural-type catheter may be used, or a "stimulating catheter" that delivers electrical current to its tip. Administered infusate generally includes exclusively long-acting, dilute, local anesthetic delivered as a bolus only, basal only, or basal-bolus combination. Documented benefits appear to be dependent on successfully improving analgesia, and include decreasing baseline/breakthrough/dynamic pain, supplemental analgesic requirements, opioid-related side effects, and sleep disturbances. In some cases, patient satisfaction and ambulation/functioning may be improved; an accelerated resumption of passive joint range-of-motion realized; and the time until discharge readiness as well as actual discharge from the hospital or rehabilitation center achieved. Lastly, postoperative joint inflammation and inflammatory markers may be decreased. Nearly all benefits occur during the infusion itself, but several randomized controlled trials suggest that in some situations there are prolonged benefits after catheter removal as well. Easily rectified minor complications occur somewhat frequently, but major risks including clinically relevant infection and nerve injury are relatively rare. This article is an evidence-based review of the published literature involving continuous peripheral nerve blocks. FAU - Ilfeld, Brian M AU - Ilfeld BM AD - Clinical Investigation, University of California San Diego, 200 West Arbor Dr., MC 8770, San Diego, CA 92103-8770, USA. bilfeld@ucsd.edu LA - eng PT - Journal Article PT - Review DEP - 20110804 PL - United States TA - Anesth Analg JT - Anesthesia and analgesia JID - 1310650 RN - 0 (Anesthetics, Local) SB - IM CIN - Anesth Analg. 2011 Oct;113(4):689-91. doi: 10.1213/ANE.0b013e31822dad0a. PMID: 21948278 CIN - Reg Anesth Pain Med. 2014 Nov-Dec;39(6):556-7. doi: 10.1097/AAP.0000000000000139. PMID: 25340485 MH - Anesthetics, Local/*administration & dosage/adverse effects MH - Catheterization MH - Evidence-Based Medicine MH - Humans MH - Infusions, Parenteral MH - Nerve Block/adverse effects/*methods MH - *Peripheral Nerves MH - Risk Assessment MH - Risk Factors MH - Time Factors MH - Treatment Outcome EDAT- 2011/08/09 06:00 MHDA- 2012/07/17 06:00 CRDT- 2011/08/09 06:00 PHST- 2011/08/09 06:00 [entrez] PHST- 2011/08/09 06:00 [pubmed] PHST- 2012/07/17 06:00 [medline] AID - ANE.0b013e3182285e01 [pii] AID - 10.1213/ANE.0b013e3182285e01 [doi] PST - ppublish SO - Anesth Analg. 2011 Oct;113(4):904-25. doi: 10.1213/ANE.0b013e3182285e01. Epub 2011 Aug 4. PMID- 36753129 OWN - NLM STAT- MEDLINE DCOM- 20230320 LR - 20240210 IS - 2168-6084 (Electronic) IS - 2168-6068 (Print) IS - 2168-6068 (Linking) VI - 159 IP - 3 DP - 2023 Mar 1 TI - Clinical Associations of Degos-Like Lesions in Patients With Systemic Sclerosis. PG - 308-313 LID - 10.1001/jamadermatol.2022.6330 [doi] AB - IMPORTANCE: Degos-like lesions are cutaneous manifestations of a small-vessel vasculopathy that appear as atrophic, porcelain-white papules with red, telangiectatic borders. No study has adequately examined Degos-like lesions in patients with systemic sclerosis (SSc). OBJECTIVE: To characterize the serologic, cutaneous, and internal organ manifestations associated with Degos-like lesions in a large cohort of patients with SSc. DESIGN, SETTINGS, AND PARTICIPANTS: This retrospective cohort study involved adult patients with SSc who were seen at Stanford Rheumatologic Dermatology Clinic between January 1, 1998, and December 31, 2018. Participants fulfilled the 2013 classification criteria for SSc. Data analysis was conducted from February 1 to June 1, 2019. MAIN OUTCOMES AND MEASURES: Data on demographic characteristics; autoantibody status; clinical characteristics, including cutaneous and systemic manifestations of SSc; and presence of Degos-like lesions were collected. RESULTS: The cohort comprised 506 patients with SSc (447 females [88.3%]; mean [SD] age at first non-Raynaud disease symptoms, 46.1 [15.2] years). Twenty-seven patients (5.3%) had Degos-like lesions, of whom 24 (89.0%) had lesions affecting the fingers. Patients with Degos-like lesions were more likely to have diffuse cutaneous SSc compared with patients without lesions (15 [55.6%] vs 181 [37.8%]; P = .04). Degos-like lesions were also associated with acro-osteolysis (10 [37.0%] vs 62 [12.9%]; P < .01), digital ulcers (15 [55.6%] vs 173 [36.1%]; P = .04), and calcinosis (15 [55.6%] vs 115 [24.0%]; P < .01). While Degos-like lesions were not associated with internal organ manifestations, such as scleroderma renal crisis, interstitial lung disease, or pulmonary arterial hypertension, there was P < .10 for the association with gastric antral vascular ectasia. CONCLUSIONS AND RELEVANCE: Results of this study suggest an association of Degos-like lesions with diffuse cutaneous SSc and other cutaneous manifestations of vasculopathy, including acro-osteolysis, calcinosis, and digital ulcers. A prospective longitudinal study is warranted to examine the onset of Degos-like lesions and to elucidate whether these lesions play a role in SSc. FAU - Song, Paula AU - Song P AD - Division of Rheumatology, Columbia University School of Medicine, New York, New York. FAU - Li, Shufeng AU - Li S AD - Department of Dermatology, Stanford University School of Medicine, Palo Alto, California. AD - Department of Urology, Stanford University School of Medicine, Palo Alto, California. FAU - Lewis, Matthew A AU - Lewis MA AD - Department of Dermatology, Stanford University School of Medicine, Palo Alto, California. FAU - Fiorentino, David F AU - Fiorentino DF AD - Department of Dermatology, Stanford University School of Medicine, Palo Alto, California. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, California. AD - Division of Immunology and Rheumatology, Palo Alto Veterans Affairs Healthcare System, Palo Alto, California. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - JAMA Dermatol JT - JAMA dermatology JID - 101589530 RN - digital ulcers SB - IM MH - Retrospective Studies MH - Adolescent MH - *Acro-Osteolysis/complications MH - Female MH - Skin Ulcer MH - Humans MH - *Scleroderma, Systemic/complications/diagnosis MH - *Calcinosis MH - Adult MH - *Vascular Diseases MH - Prospective Studies MH - Longitudinal Studies PMC - PMC9909573 COIS- Conflict of Interest Disclosures: Dr Lewis is part of the speaker’s bureau for AbbVie and Regeneron/Sanofi and reported receiving consulting fees for UCB, Pfizer, and Gilead. Dr Fiorentino reported receiving grants from Serono and personal fees from Bristol Myers Squibb, Janssen, UCB, Acelyrin, Amgen, Priovant, and Gilead outside the submitted work. Dr Chung reported receiving grants from Boehringer Ingelheim and other funding from Eicos Sciences, Genentech, Mitsubishi Tanabe, Kyverna, and Jasper as well as personal fees from Janssen outside the submitted work. No other disclosures were reported. EDAT- 2023/02/09 06:00 MHDA- 2023/03/21 06:00 PMCR- 2024/02/08 CRDT- 2023/02/08 11:33 PHST- 2023/02/09 06:00 [pubmed] PHST- 2023/03/21 06:00 [medline] PHST- 2023/02/08 11:33 [entrez] PHST- 2024/02/08 00:00 [pmc-release] AID - 2800961 [pii] AID - dbr220026 [pii] AID - 10.1001/jamadermatol.2022.6330 [doi] PST - ppublish SO - JAMA Dermatol. 2023 Mar 1;159(3):308-313. doi: 10.1001/jamadermatol.2022.6330. PMID- 39544899 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260129 LR - 20260129 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 1 DP - 2025 Feb TI - A Phase II study of avenciguat, a novel soluble guanylate cyclase activator, in patients with systemic sclerosis: Study design and rationale of the VITALISScE™ study. PG - 27-35 LID - 10.1177/23971983241291923 [doi] AB - INTRODUCTION: Systemic sclerosis is a rare autoimmune connective tissue disease characterised by (1) microvasculopathy; (2) immune dysregulation; and (3) progressive fibrosis of the skin and internal organs. Soluble guanylate cyclase plays an important role in maintaining vascular and immunological homeostasis and preventing organ fibrosis. Pharmacological modulation of soluble guanylate cyclase with soluble guanylate cyclase stimulators has shown anti-inflammatory and antifibrotic effects in animal models of systemic sclerosis, with a trend towards clinical efficacy in a Phase II study (RISE-SSc). However, the efficacy of soluble guanylate cyclase stimulators may be reduced under conditions of hypoxia and oxidative stress. Soluble guanylate cyclase activators have the potential to overcome this limitation. This paper describes the study design of VITALISScE™, a Phase II clinical trial assessing the efficacy, safety and tolerability of avenciguat, a novel soluble guanylate cyclase activator in patients with active systemic sclerosis at risk of progression. METHODS: The VITALISScE™ study (NCT05559580) is evaluating the action of avenciguat on all three aspects of systemic sclerosis pathophysiology. The primary endpoint is the rate of decline in forced vital capacity (mL) over 48 weeks. Secondary endpoints include absolute change from baseline at Week 48 in modified Rodnan skin score, Health Assessment Questionnaire Disability Index score and the proportion of responders based on the revised Composite Response Index in Systemic Sclerosis. Additional endpoints include a composite assessment of Raynaud's phenomenon, digital ulcer burden, functional outcomes and quality of life, safety, pharmacokinetics, and biomarkers associated with systemic sclerosis and the mechanism of action of avenciguat. RESULTS: VITALISScE™ is an ongoing, multicentre (180 sites; 38 countries), placebo-controlled, double-blind, parallel-group, Phase II clinical study. Recruitment is currently ongoing. CONCLUSIONS: The VITALISScE™ study is assessing the efficacy, safety and tolerability of avenciguat in patients with active systemic sclerosis at risk of progression. Results will inform further development of avenciguat. TRIAL REGISTRATION: VITALISScE™; EU CT No. 2022-500332-11-00; Clinicaltrials.gov: NCT05559580 (https://www.clinicaltrials.gov/study/NCT05559580). CI - © The Author(s) 2024. FAU - Khanna, Dinesh AU - Khanna D AD - Department of Internal Medicine, University of Michigan Scleroderma Clinic, Ann Arbor, MI, USA. FAU - de Vries-Bouwstra, Jeska AU - de Vries-Bouwstra J AD - Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Low, Andrea Hsiu Ling AU - Low AHL AD - Department of Rheumatology and Immunology, Singapore General Hospital, Singapore. AD - Duke-National University of Singapore Medical School, Singapore. FAU - Proudman, Susanna AU - Proudman S AD - Discipline of Medicine, University of Adelaide and Rheumatology Unit, Royal Adelaide Hospital, Adelaide, SA, Australia. FAU - Flack, Mary AU - Flack M AD - TA Inflammation Medicine, Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA. FAU - Kukreja, Anjli AU - Kukreja A AD - Translational Medicine & Clinical Pharmacology, Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA. FAU - Fagan, Nora AU - Fagan N AD - Global Biostatistics & Data Sciences, Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. LA - eng SI - ClinicalTrials.gov/NCT05559580 PT - Journal Article DEP - 20241107 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC11559521 OAB - Systemic sclerosis, or SSc, is a rare autoimmune disease where a person’s immune system attacks their body and causes thickening or scarring, known as fibrosis. Fibrosis can happen in the skin and in other tissues, such as the lungs, and may cause bothersome symptoms throughout the body. Alongside fibrosis, patients with SSc may also have damage to their blood vessels and problems with their immune system. Some treatments for SSc work by targeting an enzyme in the blood called soluble guanylate cyclase, or sGC for short. These treatments either stimulate or activate sGC, which helps to maintain normal blood vessel and immune system activity. Effects of the sGC pathway can also help to prevent fibrosis. The activity of sGC stimulators may decrease when there are low oxygen levels in tissues affected by fibrosis. sGC activators have been suggested to work well in low-oxygen environments and may be useful treatments for patients with SSc. The VITALISScE™ study is looking at an sGC activator called avenciguat in patients with SSc. This study is currently ongoing across multiple countries. It includes patients with SSc at risk of their disease getting worse. During the study, patients will randomly receive either avenciguat or a placebo (non-active drug). Patients can take some of their usual medications for SSc, with some restrictions. The impact on lung function will be measured over 48 weeks. Through the study, the investigators will look at changes in participants’ lung function, skin thickness, ability to carry out daily activities, quality of life and any other effects of the medicine. Researchers want to understand if it is possible to reduce fibrosis by targeting the sGC pathway. OABL- eng OTO - NOTNLM OT - SSc OT - clinical trial OT - fibrosis OT - microvasculopathy OT - sGC activator COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: D.K. is currently or in the recent past has been consultant to Amgen, Argenx, AstraZeneca, Boehringer Ingelheim, Chemomab, Cabaletta Bio, CSL Behring, GSK, Janssen, Merck, Novartis, Prometheus and Zura Bio. He has received grants from Amgen, Bayer, Bristol Myers Squibb, Boehringer Ingelheim and Pfizer. J.d.V.-B. has received grant/research support to her institution from ReumaNederland (Dutch patient Society for Rheumatology), Nationale Vereniging voor mensen met lupus, APS, sclerodermie en MCTD (Dutch patient society), ARCH (Autoimmune Research and Collaboration Hub; Dutch interdisciplinary society for patients and caregivers), Roche, Galapagos, Janssen-Cilag and Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Janssen-Cilag and AbbVie (payments made to institution); speaker fees from Dutch Society of Rheumatology (payments made to institution), Boehringer Ingelheim (payments made to institution) and Janssen-Cilag (payments made to institution). A.-M.H.-V. has received research funding and/or consulting fees and/or other remuneration from Arxx, Bristol Myers Squibb, Boehringer Ingelheim, Genentech, Janssen, Medscape, Merck Sharp & Dohme, Novartis, Pliant Therapeutics and Roche. She is also group leader for the EULAR study group on the lung in rheumatic and musculoskeletal diseases. M.K. has received consulting fees, speaking fees and/or research grants from Argenx, Asahi Kasei Parma, AstraZeneca, Boehringer Ingelheim, Chugai, GSK, Janssen, Kissei, MBL, Mochida, Ono Pharmaceuticals and Tanabe-Mitsubishi. A.H.L.L. has received consultancy funds from Boehringer Ingelheim and Janssen. She has received research grant support from Boehringer Ingelheim, National Medical Research Council, Asia-Pacific League of Associations for Rheumatology and Reverie Rheumatology Research Fund. She is medical advisor to Neuquin Biopharma Pte Ltd. S.P. has received research funding, consulting fees or speaker’s fees from Boehringer Ingelheim and Janssen. M.F., A.K. and N.F. are employees of Boehringer Ingelheim International GmbH. O.D. has/had a consultancy relationship with and/or has received research funding from and/or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years: 4P-Pharma, AbbVie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Argenx, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Cantargia AB, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta Inc., Novartis, Orion, Prometheus, Redxpharma, Roivant, EMD Serono, Topadur and UCB. He has a patent issued: ‘mir-29 for the treatment of systemic sclerosis’ (US8247389, EP2331143) and is co-founder of CITUS AG. EDAT- 2024/11/15 06:25 MHDA- 2024/11/15 06:26 PMCR- 2025/11/07 CRDT- 2024/11/15 04:34 PHST- 2024/09/16 00:00 [received] PHST- 2024/09/19 00:00 [accepted] PHST- 2024/11/15 06:26 [medline] PHST- 2024/11/15 06:25 [pubmed] PHST- 2024/11/15 04:34 [entrez] PHST- 2025/11/07 00:00 [pmc-release] AID - 10.1177_23971983241291923 [pii] AID - 10.1177/23971983241291923 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2024 Nov 7;10(1):27-35. doi: 10.1177/23971983241291923. eCollection 2025 Feb. PMID- 32299845 OWN - NLM STAT- MEDLINE DCOM- 20200707 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Print) IS - 0003-4967 (Linking) VI - 79 IP - 5 DP - 2020 May TI - Riociguat in patients with early diffuse cutaneous systemic sclerosis (RISE-SSc): randomised, double-blind, placebo-controlled multicentre trial. PG - 618-625 LID - 10.1136/annrheumdis-2019-216823 [doi] AB - OBJECTIVES: Riociguat is approved for pulmonary arterial hypertension and has antiproliferative, anti-inflammatory and antifibrotic effects in animal models of tissue fibrosis. We evaluated the efficacy and safety of riociguat in patients with early diffuse cutaneous systemic sclerosis (dcSSc) at high risk of skin fibrosis progression. METHODS: In this randomised, double-blind, placebo-controlled, phase IIb trial, adults with dcSSc of <18 months' duration and a modified Rodnan skin score (mRSS) 10-22 units received riociguat 0.5 mg to 2.5 mg orally three times daily (n=60) or placebo (n=61). The primary endpoint was change in mRSS from baseline to week 52. RESULTS: At week 52, change from baseline in mRSS units was -2.09±5.66 (n=57) with riociguat and -0.77±8.24 (n=52) with placebo (difference of least squares means -2.34 (95% CI -4.99 to 0.30; p=0.08)). In patients with interstitial lung disease, forced vital capacity declined by 2.7% with riociguat and 7.6% with placebo. At week 14, average Raynaud's condition score had improved ≥50% in 19 (41.3%)/46 patients with riociguat and 13 (26.0%)/50 patients with placebo. Safety assessments showed no new signals with riociguat and no treatment-related deaths. CONCLUSIONS: Riociguat did not significantly benefit mRSS versus placebo at the predefined p<0.05. Secondary and exploratory analyses showed potential efficacy signals that should be tested in further trials. Riociguat was well tolerated. CI - © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA khannad@med.umich.edu oliver.distler@usz.ch. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology A department, Cochin Hospital, APHP, Paris Descartes University, Paris, France. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy. FAU - Pope, Janet E AU - Pope JE AUID- ORCID: 0000-0003-1479-5302 AD - Schulich School of Medicine, Division of Rheumatology, The University of Western Ontario, London, Ontario, Canada. FAU - Atsumi, Tatsuya AU - Atsumi T AD - Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan. FAU - Bečvář, Radim AU - Bečvář R AD - Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic. FAU - Czirják, László AU - Czirják L AD - Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary. FAU - Hachulla, Eric AU - Hachulla E AD - Department of Internal Medicine and Clinical Immunology, Claude Huriez Hospital, Lille University School of Medicine, Lille, France. FAU - Ishii, Tomonori AU - Ishii T AD - Clinical Research, Innovation and Education Center, Tohoku University Hospital, Sendai, Japan. FAU - Ishikawa, Osamu AU - Ishikawa O AD - Department of Dermatology, Gunma University Postgraduate School of Medicine, Maebashi, Japan. FAU - Johnson, Sindhu R AU - Johnson SR AUID- ORCID: 0000-0003-0591-2976 AD - Division of Rheumatology, Department of Medicine, Toronto Western Hospital, University Health Network, Mount Sinai Hospital, University of Toronto, Toronto Scleroderma Research Program, Toronto, Ontario, Canada. FAU - De Langhe, Ellen AU - De Langhe E AD - Laboratory of Tissue Homeostasis and Disease, Skeletal Biology and Engineering Research Center, Department of Development and Regeneration, KU Leuven, Leuven, Belgium. FAU - Stagnaro, Chiara AU - Stagnaro C AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Clinical Medicine and Therapy, University of Rome La Sapienza, Rome, Italy. FAU - Schiopu, Elena AU - Schiopu E AD - Division of Rheumatology, Department of Internal Medicine, Michigan Medicine University Hospitals, Ann Arbor, Michigan, USA. FAU - Silver, Richard M AU - Silver RM AUID- ORCID: 0000-0002-2038-3278 AD - Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, South Carolina, USA. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium. FAU - Steen, Virginia AU - Steen V AD - Division of Rheumatology, Georgetown University Medical Center, Washington, DC, USA. FAU - Stevens, Wendy AU - Stevens W AD - Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia. FAU - Szücs, Gabriella AU - Szücs G AD - Division of Rheumatology, Department of Internal Medicine, University of Debrecen, Debrecen, Hungary. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Department of Rheumatology, CHU Bordeaux, Bordeaux, France. FAU - Wosnitza, Melanie AU - Wosnitza M AD - Research & Development, Bayer AG, Wuppertal, Germany. FAU - Laapas, Kaisa AU - Laapas K AD - StatFinn Oy, Espoo, Finland. FAU - de Oliveira Pena, Janethe AU - de Oliveira Pena J AD - Bayer HealthCare Pharmaceuticals Inc, Whippany, New Jersey, USA. FAU - Yao, Zhen AU - Yao Z AD - Bayer Healthcare, Beijing, China. FAU - Kramer, Frank AU - Kramer F AD - Research & Development, Bayer AG, Wuppertal, Germany. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital, Zurich, Switzerland khannad@med.umich.edu oliver.distler@usz.ch. LA - eng PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Enzyme Activators) RN - 0 (Pyrazoles) RN - 0 (Pyrimidines) RN - RU3FE2Y4XI (riociguat) SB - IM CIN - Ann Rheum Dis. 2022 Jul;81(7):e116. doi: 10.1136/annrheumdis-2020-218180. PMID: 32669302 CIN - Ann Rheum Dis. 2022 Jul;81(7):e117. doi: 10.1136/annrheumdis-2020-218194. PMID: 32669303 MH - Adult MH - Biopsy, Needle MH - Dose-Response Relationship, Drug MH - Double-Blind Method MH - Drug Administration Schedule MH - Enzyme Activators/*administration & dosage MH - Female MH - Follow-Up Studies MH - Humans MH - Immunohistochemistry MH - Internationality MH - Male MH - Middle Aged MH - Pyrazoles/*administration & dosage MH - Pyrimidines/*administration & dosage MH - Respiratory Function Tests MH - Risk Assessment MH - Scleroderma, Diffuse/*drug therapy/pathology MH - Severity of Illness Index MH - Treatment Failure PMC - PMC7213318 OTO - NOTNLM OT - disease activity OT - systemic sclerosis OT - treatment COIS- Competing interests: DK reports personal fees from Actelion, grants and personal fees from Bayer AG, grants and personal fees from BMS, grants from Pfizer, personal fees from Arena, personal fees from Eclos Sciences, Inc, personal fees from BI, personal fees from Arena, personal fees from CSL Behring, personal fees from GSK, personal fees from Galapagos, personal fees from Genentech/Roche, personal fees from Corbus, personal fees from Cytori, grants from Horizon, outside the submitted work. OD reports other from Actelion, other from Bayer, other from Boehringer Ingelheim, other from Mitsubishi Tanabe, other from AnaMar, other from ChemonAb, other from espeRare Foundation, other from Genentech/Roche, other from GSK, other from Inventiva, other from Italfarmaco, other from iQvia, other from Lilly, other from Medac, other from MedImmune, other from Pharmacyclics, other from Novartis, other from Pfizer, other from Sanofi, other from Serodapharm, other from UCB, other from Amgen, other from AbbVie, other from Mepha, other from MSD, outside the submitted work. YA reports personal fees from Actelion, personal fees from Bayer AG, grants and personal fees from BMS, grants from Inventiva, personal fees from BI, grants from Roche, grants from Sanofi, outside the submitted work. CPD reports personal fees from Actelion, personal fees from Bayer AG, grants and personal fees from Inventiva, personal fees from BI, personal fees from Roche-Genentech, personal fees from Sanofi Aventis, grants and personal fees from CSL Behring, grants and personal fees from GlaxoSmithKline, outside the submitted work. MK reports grants and personal fees from Actelion, personal fees from Bayer AG, personal fees from Chugai, personal fees from BI, personal fees from Reata, personal fees from Corpus, personal fees from CSL Behring, personal fees from GlaxoSmithKline, personal fees from Mochida, personal fees from Pfizer, personal fees from Nippon Shinyaku, outside the submitted work. MMC reports grants and personal fees from Actelion, grants from Chemomab, grants and personal fees from BMS, grants from Pfizer, grants and personal fees from MSD, grants from Sanipedia, personal fees from Janssen, outside the submitted work. JEP reports personal fees from Actelion, personal fees from Bayer AG, personal fees from BMS, personal fees from AbbVie, personal fees from Lilly, personal fees from Roche, personal fees from Sanofi, personal fees from Merck, personal fees from Novartis, personal fees from Pfizer, personal fees from Sandoz, personal fees from UCB, personal fees from Amgen, outside the submitted work. TA reports grants and personal fees from Astellas, grants and personal fees from Takeda, grants and personal fees from Mitsubishi Tanabe, grants and personal fees from Chugai, grants and personal fees from Daiichi-Sankyo, grants from Otuska, grants from Takeda, grants and personal fees from Pfizer, grants from Alexion, grants from Bayer, grants from Eisai, grants from Bristol-Myers Squibb, grants from Asahi Kasei, personal fees from Ono, personal fees from Sanofi, personal fees from Eli Lilly, outside the submitted work. LC reports personal fees from Actelion, personal fees from Bayer AG, personal fees from BI, personal fees from Roche-Genentech, personal fees from Lilly, personal fees from Medac, personal fees from Novartis, personal fees from Pfizer, outside the submitted work. EH reports grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer AG, grants, personal fees and non-financial support from GlaxoSmithKline, grants, personal fees and non-financial support from MSD, grants and personal fees from Pfizer, outside the submitted work. TI reports personal fees from Astellas, personal fees from Daiichi-Sankyo, personal fees from Chugai, personal fees from Sanofi, personal fees from AbbVie, personal fees from Bristol-Myers, personal fees from Mitsubishi Tanabe, personal fees from Eisai, personal fees from Janssen, personal fees from Asahi Kasei, personal fees from Ono Pharmaceutical, personal fees from Ayumi Pharmaceutical, personal fees from Pfizer, outside the submitted work. Virginia Steen reports participation in advisory boards, consultancy for economics of scleroderma management, and clinical trials for Bayer; investigator-initiated grant, advisory board, and steering committee (consulting) for CSL Behring; advisory board and site primary investigator (PI) of clinical trial for Roche; site PI of clinical trial for Sanofi; site PI of clinical trial for Immune Tolerance Network; and DSMB for open labs phase 2 trial and site PI for the phase 3 trial for Corbus. ME-T reports consulting fees, speaking fees and honoraria from AbbVie, BMS, Lilly, Medac, MSD, Pfizer, Roche, UCB, outside the submitted work. MW reports employment from Bayer AG, outside the submitted work. KL reports other from StatFinn Oy, outside the submitted work. JdOP reports other from Bayer AG, outside the submitted work. ZY reports other from Bayer HealthCare, outside the submitted work. FK reports other from Bayer AG, outside the submitted work. EDAT- 2020/04/18 06:00 MHDA- 2020/07/08 06:00 PMCR- 2020/05/11 CRDT- 2020/04/18 06:00 PHST- 2019/12/13 00:00 [received] PHST- 2020/02/19 00:00 [revised] PHST- 2020/03/09 00:00 [accepted] PHST- 2020/04/18 06:00 [entrez] PHST- 2020/04/18 06:00 [pubmed] PHST- 2020/07/08 06:00 [medline] PHST- 2020/05/11 00:00 [pmc-release] AID - S0003-4967(24)01408-0 [pii] AID - annrheumdis-2019-216823 [pii] AID - 10.1136/annrheumdis-2019-216823 [doi] PST - ppublish SO - Ann Rheum Dis. 2020 May;79(5):618-625. doi: 10.1136/annrheumdis-2019-216823. PMID- 37402671 OWN - NLM STAT- MEDLINE DCOM- 20230706 LR - 20230706 IS - 0376-2491 (Print) IS - 0376-2491 (Linking) VI - 103 IP - 25 DP - 2023 Jul 4 TI - [Risk factors analysis and prediction model establishment of malignant tumor in patients with polymyositis/dermatomyositis]. PG - 1903-1910 LID - 10.3760/cma.j.cn112137-20230329-00505 [doi] AB - Objective: To analyze the risk factors of polymyositis/dermatomyositis (PM/DM) complicated with malignant tumor and to construct clinical prediction model. Methods: A total of 427 PM/DM patients, who were admitted to Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University from January 1, 2015 to January 1, 2021, were enrolled in the study, including 129 males and 298 females. The mean age was (51.4±12.2) years. The patients were divided into control group (without malignant tumor, n=379) and case group (with malignant tumor, n=48) according to whether they were complicated with malignant tumors. In the two groups, 70% of the patients' clinical data were randomly selected as the training set data, and the remaining 30% were used as the validation set data. The clinical parameters were retrospectively collected, and risk factors of PM/DM complicated with malignant tumor were analyzed by binary logistic regression. R software was used to construct a clinical prediction model for malignant tumors in PM/DM patients using training set data. Validation set data were used to assess the feasibility of the model. The area under the receiver operating characteristic (ROC) curve (AUC), calibration curve and decision curve analysis (DCA) were used to evaluate the predictive ability, accuracy and clinical applicability of the nomogram model. Results: The age of the control group was (50.4±11.8) years, and males accounted for 26.9%(102/379); the age of the case group was (59.1±12.7) years, and the proportion of males was 56.3% (27/48). The proportion of male, age, the positive rate of anti-transcription mediator 1-γ (TIF1-γ) antibody, glucocorticoid therapy resistance, and levels of creatine kinase (CK), carbohydrate antigen 125(CA125) and carbohydrate antigen 199 (CA199) were all higher in the case group than those in control group, while incidence of interstitial lung disease (ILD), arthralgia, Raynaud's phenomenon, serum albumin (ALB) level and lymphocyte (LYM) count were all lower than those in control group (all P<0.05). Binary logistic regression analysis showed that male (OR=2.931, 95%CI: 1.356-6.335), glucocorticoid therapy resistance (OR=5.261, 95%CI: 2.212-12.513), older age (OR=1.056, 95%CI: 1.022-1.091), elevated CA125 (OR=8.327, 95%CI: 2.448-28.319) and positive anti-TIF1-γ antibody (OR=7.529, 95%CI: 2.436-23.270) were risk factors of malignancy in PM/DM patients (all P<0.05); and complicated with ILD (OR=0.261, 95%CI: 0.099-0.689), complicated with arthralgia (OR=0.238,95%CI:0.073-0.779), elevated LYM count (OR=0.267, 95%CI: 0.103-0.691) were protective factors of malignancy in PM/DM patients (all P<0.05). The AUC of ROC curve predicting malignancy in PM/DM patients with the training concentrated prediction model was 0.887 (95%CI: 0.852-0.922), with a sensitivity of 77.9% and a specificity of 86.3%; it was 0.925 (95%CI: 0.890-0.960), 86.5% and 88.0% in the validated centralized prediction model, respectively. The correction curves of the training set and the validation set indicated that the predictive model had good calibration ability. Both the DCA curves of the training set and the validation set showed that the proposed predictive model had good clinical applicability. Conclusions: Older age, male, glucocorticoid therapy resistance, not complicated with ILD and arthralgia, elevated CA125, positive anti-TIF1-γ antibody, decreased LYM count are risk factors for malignancy in PM/DM patients, and the established nomogram model shows good predictive ability. FAU - Wang, Y F AU - Wang YF AD - Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University, Xi'an 710038, China. FAU - Li, H X AU - Li HX AD - Department of Rheumatology and Immunology, Air Force Medical Center, Beijing 100142, China. FAU - Feng, Y AU - Feng Y AD - Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University, Xi'an 710038, China. FAU - Zhang, Y AU - Zhang Y AD - Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University, Xi'an 710038, China. FAU - Wu, Z B AU - Wu ZB AD - Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University, Xi'an 710038, China. LA - chi GR - 2021ZTXM-030/Natural Science Foundation Boost Program of Tangdu Hospital 2021-2023/ PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Yi Xue Za Zhi JT - Zhonghua yi xue za zhi JID - 7511141 RN - 0 (Glucocorticoids) RN - 0 (CA-125 Antigen) RN - 0 (Carbohydrates) SB - IM MH - Female MH - Humans MH - Male MH - Adult MH - Middle Aged MH - Aged MH - *Dermatomyositis/complications MH - *Polymyositis/complications MH - Retrospective Studies MH - Glucocorticoids MH - Models, Statistical MH - Prognosis MH - Risk Factors MH - *Neoplasms/complications MH - *Lung Diseases, Interstitial/complications MH - CA-125 Antigen MH - Carbohydrates EDAT- 2023/07/05 01:06 MHDA- 2023/07/06 06:42 CRDT- 2023/07/04 22:51 PHST- 2023/07/06 06:42 [medline] PHST- 2023/07/05 01:06 [pubmed] PHST- 2023/07/04 22:51 [entrez] AID - 10.3760/cma.j.cn112137-20230329-00505 [doi] PST - ppublish SO - Zhonghua Yi Xue Za Zhi. 2023 Jul 4;103(25):1903-1910. doi: 10.3760/cma.j.cn112137-20230329-00505. PMID- 40902212 OWN - NLM STAT- MEDLINE DCOM- 20250918 LR - 20251118 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 74 DP - 2025 Oct TI - Item selection for the development and validation of a revised classification criteria for adult and juvenile idiopathic inflammatory myopathies: MyoROC project. PG - 152822 LID - S0049-0172(25)00193-3 [pii] LID - 10.1016/j.semarthrit.2025.152822 [doi] AB - OBJECTIVE: A revision of the 2017 EULAR-ACR myositis classification criteria, namely EULAR-ACR funded Myositis Revision of Classification (MyoROC) project, is currently underway involving a large international group of experts. In the first phase of this project, we identified additional items to be tested in the criteria. METHODS: We distributed an electronic survey to International Myositis Assessment and Clinical Studies (IMACS) members to identify new items. The identified items were discussed within the Steering Committee and a multi-step Delphi consensus process consisting of an open discussion and three rounds of e-voting were conducted to reach the final item list. RESULTS: The IMACS survey results revealed 24 new items. After an open discussion with Steering Committee members, 14 items were dropped and five new items were added, resulting in a total of 15 items. After three rounds of e-voting, the following variables were agreed to be tested in addition to the original items: finger flexion, knee extension ≥ hip flexion weakness, myonecrosis pattern on biopsy, magnetic resonance imaging and electromyography findings of myositis, additional rashes, skin biopsy, capillaroscopy, interstitial lung disease, arthritis, Raynaud's phenomenon, myositis-specific (MSA) and -associated autoantibodies, enzyme elevation at ≥2 time points, and aldolase. CONCLUSION: The new items that will be tested in the revised criteria were generated with input from a wide range of stakeholders and included, most importantly, MSA, pattern of weakness, skin changes, and additional diagnostic modalities. The next steps of the project are data collection followed by statistical analysis for development and validation of the revised criteria. CI - Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Saygin, Didem AU - Saygin D AD - Division of Rheumatology, Department of Medicine, Rush University Medical Center, Chicago, IL, USA. FAU - Zeng, Rachel AU - Zeng R AD - Department of Neurology, University Medical Center Göttingen, Göttingen, Germany; Department of Neurology, Faculty of Medicine and University Hospital Carl Gustav Carus, TUD Dresden University of Technology, Dresden, Germany. FAU - Glaubitz, Stefanie AU - Glaubitz S AD - Department of Neurology, University Medical Center Göttingen, Göttingen, Germany. FAU - Bottai, Matteo AU - Bottai M AD - Division of Biostatistics, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden. FAU - Amato, Anthony A AU - Amato AA AD - Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Department of Internal Medicine and Therapeutics, University of Pavia, Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Danoff, Sonye K AU - Danoff SK AD - Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, MD, USA. FAU - de Visser, Marianne AU - de Visser M AD - Department of Neurology, Amsterdam University Medical Center, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, the Netherlands. FAU - Dimachkie, Mazen M AU - Dimachkie MM AD - Department of Neurology, The University of Kansas Medical Center, Kansas City, KS, USA. FAU - Fujimoto, Manabu AU - Fujimoto M AD - Department of Dermatology, Osaka University, Osaka, Japan. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Machado, Pedro M AU - Machado PM AD - Department of Neuromuscular Diseases, University College London, London, UK; Department of Rheumatology, Northwick Park Hospital, London North West University Healthcare NHS Trust, London, UK; NIHR University College London Hospitals Biomedical Research Centre, University College London Hospitals NHS Foundation Trust, London, UK. FAU - Needham, Merrilee AU - Needham M AD - Department of Neurology Fiona Stanley Hospital, University of Notre Dame, Murdoch University and the Perron Institute, Perth, Western Australia, Australia. FAU - Pilkington, Clarissa AU - Pilkington C AD - Great Ormond Street Hospital, London, UK. FAU - Rajasekhar, Liza AU - Rajasekhar L AD - Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India. FAU - Rider, Lisa G AU - Rider LG AD - Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Salem, Yasser AU - Salem Y AD - Physical Therapy Program, Hofstra University Hempstead, NY, USA. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AD - Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil. FAU - Singh, Jasvinder A AU - Singh JA AD - Medicine Service, Michael E. DeBakey VA Medical Center, Houston, TX 77030, USA; Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA; Department of Medicine at the School of Medicine, University of Alabama at Birmingham (UAB), Birmingham, AL 35233, USA. FAU - Tikly, Mohammed AU - Tikly M AD - Division of Rheumatology, Department of Internal Medicine, University of the Witwatersrand, Johannesburg, South Africa. FAU - Wang, Guochun AU - Wang G AD - Department of Rheumatology, Key Myositis Laboratories, China-Japan Friendship Hospital, Beijing, China. FAU - Werth, Victoria P AU - Werth VP AD - Department of Dermatology, University of Pennsylvania Perelman School of Medicine and CMCVAMC, Philadelphia, PA, USA. FAU - Aggarwal, Rohit AU - Aggarwal R AD - Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA. FAU - Lundberg, Ingrid E AU - Lundberg IE AD - Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden; Department of Gastroenterology, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University Hospital, Stockholm, Sweden. Electronic address: ingrid.lundberg@ki.se. LA - eng GR - P50 AR060772/AR/NIAMS NIH HHS/United States GR - R01 AR082839/AR/NIAMS NIH HHS/United States GR - ZIA ES101081/ImNIH/Intramural NIH HHS/United States PT - Journal Article PT - Validation Study DEP - 20250828 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Myositis/classification/diagnosis MH - Adult MH - Delphi Technique MH - Consensus MH - Child MH - Surveys and Questionnaires PMC - PMC12619961 MID - NIHMS2109900 OTO - NOTNLM OT - Anti-synthetase syndrome OT - Classification OT - Myopathy OT - Myositis COIS- Declaration of competing interest Didem Saygin served as consultant for CSI Pharmacy and ArgenX (unpaid). Dr. Rohit Aggarwal has received research grants from Boehringer Ingelheim (BI), EMD Serono, Janssen, PRoivant, and Pfizer. Dr. Rohit Aggarwal has received consulting fees from Abcuro, Alexion, ANI Pharmaceutical, Argenx, Artasome, AstraZeneca, BeiGene (Beijing) Co. Ltd, Boehringer Ingelheim, Bristol Myers-Squibb, CabalettaBio, Capstanx, CSL Behring, Dren Bio, EMD Serono, Galapagos, Horizontal Therapeutics, I-Cell, Immunovant, Janssen, Janux Therapeutics, Kiniksa Pharmaceuticals, Kyverna, Lilly, Meiji Pharma, Novartis, Nakarta, Octapharma, Ouro Medicines, Pfizer, PRoivant, Sun Pharmaceutical Industries, Tourmaline Bio, UCB and Verismo Therapeutics. IE Lundberg has received speaker fees from Janssen PHARMACEUTICA NV and has received a research grant from JANSSEN PHARMACEUTICA NV. IEL has been serving on the advisory board for EMD Serono. Research & Development Institute, Astra-Zeneca, Pfizer, Galapagos, Chugai Pharmaceutical Co. Ltd, Novartis and PHARMACEUTICA NV and has stock shares in Roche and Novartis. Rachel Zeng has received honoraria from Amicus Therapeutics, Sanofi and Argenx, as well as travel expenses and congress fees from Argenx, Biogen, LFB and Octapharma. Stefanie Glaubitz has received honoraria from Alexion, Argenx and UCB as well as travel expenses and congress fees from Grifols, UCB, Octapharma, Bayer and Alexion. Anthony Amato has received support serving on Medical Advisory Boards and as a Consultant for Abcuro, Argenx, UCB, Horizon Therapeutics, Medpace, OnoPharma, Alexion, EMD Serono, Takeda, Johnson & Johnson, Viridian Therapeutics. Sonye K. Danoff has received research funding from Boehringer Ingelheim; clinical trial funding from Boehringer Ingelheim and United Therapeutics; consulting fees from Boehringer Ingelheim, AbbVie, Avalyn, Bristol Myers -Squib, and Pfizer; and serves on clinical trial oversight committees for Bristol Myers-Squib and Avalyn. Marianne de Visser has received support as a Consultant for Novartis and Argenx. Merrilee Needham has received honoraria from Sanofi-Genzyme and Abcuro. Masataka Kuwana has received research grants from Boehringer Ingelheim, consulting fees from Argenx, Astrazeneca, Boehringer Ingelheim, Bristol Myers-Squib, and Novartis. Lisa Rider has received research grant support from NIH Office of Autoimmune Disease Research and NIH Office of Data Science Strategies; Hope Pharmaceuticals, Cure JM Foundation, and Briston Myers Squibb. She has been an unpaid consultant (advisory board) to Cabaletta Bio, Horizon Therapeutics, and Pfizer. JAS has received consultant fees from ROMTech, Atheneum, Clearview healthcare partners, American College of Rheumatology, Yale, Hulio, Horizon Pharmaceuticals/DINORA, ANI/Exeltis, USA Inc., Frictionless Solutions, Schipher, Crealta/Horizon, Medisys, Fidia, PK Med, Two labs Inc., Adept Field Solutions, Clinical Care options, Putnam associates, Focus forward, Navigant consulting, Spherix, MedIQ, Jupiter Life Science, UBM LLC, Trio Health, Medscape, WebMD, and Practice Point communications; the National Institutes of Health; and the American College of Rheumatology. JAS has received institutional research support from Zimmer Biomet Holdings. JAS owns stock options in Atai life sciences, Kintara therapeutics, Intelligent Biosolutions, Acumen pharmaceutical, TPT Global Tech, Vaxart pharmaceuticals, Atyu biopharma, Adaptimmune Therapeutics, GeoVax Labs, Pieris Pharmaceuticals, Enzolytics Inc., Seres Therapeutics, Tonix Pharmaceuticals Holding Corp., Aebona Pharmaceuticals, and Charlotte’s Web Holdings, Inc. JAS previously owned stock options in Amarin, Viking and Moderna pharmaceuticals. Victoria Werth has received honoraria from Janssen, Lilly, Pfizer, Biogen, BMS, Gilead, Amgen, Nektar, EMD Sorona, CSL Behring, Crisalis, Viela Bio, Argenx, Kwoya Kirin, Regeneron, AstraZeneca, Abbvie, GSK, Cugene, UCB, Rome Pharmaceuticals, Horizon, Merck, Sanofi, Calyx, Cabaletta Bio, Nuvig Pharmaceuticals, Takeda, Immunovant, Anaptysbio, Evommune, Innovaderm, Alpine Immune Sciences, Caribou, Xencor, Ventus, Novartis, Merck, Novartis, Merck. She has received grants from Pfizer, Biogen, Gilead, Corbus Pharmaceuticals, AstraZeneca, Amgen, Regeneron, CSL Behring, BMS, Horizon, Rome Pharmaceuticals, Priovant, Ventus, Viela PMM has received honoraria from Abbvie, BMS, Celgene, Eli Lilly, Galapagos/Alfasigma, Janssen, MSD, Novartis, Orphazyme, Pfizer, Roche and UCB. EDAT- 2025/09/03 18:35 MHDA- 2025/09/19 00:36 PMCR- 2025/11/16 CRDT- 2025/09/03 18:00 PHST- 2025/06/24 00:00 [received] PHST- 2025/08/10 00:00 [revised] PHST- 2025/08/18 00:00 [accepted] PHST- 2025/09/19 00:36 [medline] PHST- 2025/09/03 18:35 [pubmed] PHST- 2025/09/03 18:00 [entrez] PHST- 2025/11/16 00:00 [pmc-release] AID - S0049-0172(25)00193-3 [pii] AID - 10.1016/j.semarthrit.2025.152822 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2025 Oct;74:152822. doi: 10.1016/j.semarthrit.2025.152822. Epub 2025 Aug 28. PMID- 36923262 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250530 IS - 2514-1775 (Electronic) IS - 2514-1775 (Linking) VI - 7 IP - 1 DP - 2023 TI - Management of systemic sclerosis: British Society for Rheumatology guideline scope. PG - rkad022 LID - 10.1093/rap/rkad022 [doi] LID - rkad022 AB - This guideline will provide a practical roadmap for management of SSc that builds upon the previous treatment guideline to incorporate advances in evidence-based treatment and increased knowledge about assessment, classification and management. General approaches to management as well as treatment of specific complications will be covered, including lung, cardiac, renal and gastrointestinal tract disease, as well as RP, digital vasculopathy, skin manifestations, calcinosis and impact on quality of life. It will include guidance related to emerging approved therapies for interstitial lung disease and account for National Health Service England prescribing policies and national guidance relevant to SSc. The guideline will be developed using the methods and processes outlined in Creating Clinical Guidelines: Our Protocol. This development process to produce guidance, advice and recommendations for practice has National Institute for Health and Care Excellence accreditation. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - De Lorenzis, Enrico AU - De Lorenzis E AD - Department of Rheumatology, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Roblin, Elen AU - Roblin E AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Goldman, Nina AU - Goldman N AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - Alcacer-Pitarch, Begonya AU - Alcacer-Pitarch B AD - Department of Rheumatology, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Blamont, Emma AU - Blamont E AD - Scleroderma and Raynaud's UK, London, UK. FAU - Buch, Maya AU - Buch M AUID- ORCID: 0000-0002-8962-5642 AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Carulli, Maresa AU - Carulli M AD - Department of Rheumatology, Hammersmith Hospitals NHS Foundation Trust, London, UK. FAU - Cotton, Caroline AU - Cotton C AD - Department of Rheumatology, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Department of Rheumatology, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Derrett-Smith, Emma AU - Derrett-Smith E AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - Douglas, Karen AU - Douglas K AD - Department of Rheumatology, Dudley Group NHS Foundation Trust, Birmingham, UK. FAU - Farrington, Sue AU - Farrington S AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Gompels, Luke AU - Gompels L AD - Department of Rheumatology, Somerset NHS Foundation Trust, Taunton, UK. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle, UK. FAU - Herrick, Ariane AU - Herrick A AUID- ORCID: 0000-0003-4941-7926 AD - Department of Rheumatology, Hammersmith Hospitals NHS Foundation Trust, London, UK. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Hammersmith Hospitals NHS Foundation Trust, London, UK. FAU - Pain, Clare AU - Pain C AUID- ORCID: 0000-0003-4965-3259 AD - Department of Rheumatology, Alder Hey Children's Hospital, Liverpool, UK. FAU - Pantano, Georgina AU - Pantano G AD - Patient representative, London, UK. FAU - Pauling, John AU - Pauling J AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Foundation Trust, Bristol, UK. FAU - Prabu, Athiveeraramapandian AU - Prabu A AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - O'Donoghue, Nuala AU - O'Donoghue N AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Renzoni, Elisabetta AU - Renzoni E AD - Interstitial Lung Disease Unit, Royal Brompton NHS Foundation Trust, London, UK. FAU - Royle, Jeremy AU - Royle J AD - Department of Rheumatology, University Hospitals NHS Foundation Trust, Leicester, UK. FAU - Samaranayaka, Muditha AU - Samaranayaka M AD - Department of Rheumatology, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Spierings, Julia AU - Spierings J AD - Department of Rheumatology, University of Utrecht, Utrecht, The Netherlands. FAU - Tynan, Aoife AU - Tynan A AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Warburton, Louise AU - Warburton L AD - Primary Care Sciences, Keele University, Keele, UK. FAU - Ong, Voon AU - Ong V AUID- ORCID: 0000-0001-5474-0053 AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. LA - eng GR - 20719/VAC_/Versus Arthritis/United Kingdom GR - 22398/VAC_/Versus Arthritis/United Kingdom PT - Journal Article PT - Review DEP - 20230314 PL - England TA - Rheumatol Adv Pract JT - Rheumatology advances in practice JID - 101736676 PMC - PMC10010890 OTO - NOTNLM OT - SSc OT - guideline OT - management OT - pulmonary fibrosis OT - scleroderma EDAT- 2023/03/17 06:00 MHDA- 2023/03/17 06:01 PMCR- 2023/03/14 CRDT- 2023/03/16 02:19 PHST- 2023/01/06 00:00 [received] PHST- 2023/02/03 00:00 [accepted] PHST- 2023/03/16 02:19 [entrez] PHST- 2023/03/17 06:00 [pubmed] PHST- 2023/03/17 06:01 [medline] PHST- 2023/03/14 00:00 [pmc-release] AID - rkad022 [pii] AID - 10.1093/rap/rkad022 [doi] PST - epublish SO - Rheumatol Adv Pract. 2023 Mar 14;7(1):rkad022. doi: 10.1093/rap/rkad022. eCollection 2023. PMID- 24052748 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130920 LR - 20211021 IS - 1311-0160 (Print) IS - 1311-0160 (Linking) VI - 15 IP - Suppl DP - 2012 Dec TI - Integrated genomic analysis of breast cancers. PG - 71-4 LID - 10.2478/v10034-012-0023-x [doi] AB - Breast cancer is the most frequent and the most deadly cancer in women in Western countries. Different classifications of disease (anatomoclinical, pathological, prognostic, genetic) are used for guiding the management of patients. Unfortunately, they fail to reflect the whole clinical heterogeneity of the disease. Consequently, molecularly distinct diseases are grouped in similar clinical classes, likely explaining the different clinical outcome between patients in a given class, and the fact that selection of the most appropriate diagnostic or therapeutic strategy for each patient is not done accurately. Today, treatment is efficient in only 70.0-75.0% of cases overall. Our repertoire of efficient drugs is limited but is being expanded with the discovery of new molecular targets for new drugs, based on the identification of candidate oncogenes and tumor suppressor genes (TSG) functionally relevant in disease. Development of new drugs makes therapeutical decisions even more demanding of reliable classifiers and prognostic/predictive tests. Breast cancer is a complex, heterogeneous disease at the molecular level. The combinatorial molecular origin and the heterogeneity of malignant cells, and the variability of the host background, create distinct subgroups of tumors endowed with different phenotypic features such as response to therapy and clinical outcome. Cellular and molecular analyses can identify new classes biologically and clinically relevant, as well as provide new clinically relevant markers and targets. The various stages of mammary tumorigenesis are not clearly defined and the genetic and epigenetic events critical to the development and aggressiveness of breast cancer are not precisely known. Because the phenotype of tumors is dependent on many genes, a large-scale and integrated molecular characterization of the genetic and epigenetic alterations and gene expression deregulation should allow the identification of new molecular classes clinically relevant, as well as among the altered genes and/or pathways, the identification of more accurate molecular diagnostic, prognostic/predictive factors, and for some of them, after functional validation, the identification of new therapeutic targets. FAU - Addou-Klouche, L AU - Addou-Klouche L AD - Marseille Cancer Research Center, Department of Molecular Oncology, UMR891 Inserm; Institut Paoli-Calmettes, Marseille, France ; Biotoxicology Laboratory, Djillali Liabes University, Sidi-Bel-Abbès, Algeria. FAU - Adélaïde, J AU - Adélaïde J FAU - Cornen, S AU - Cornen S FAU - Bekhouche, I AU - Bekhouche I FAU - Finetti, P AU - Finetti P FAU - Guille, A AU - Guille A FAU - Sircoulomb, F AU - Sircoulomb F FAU - Raynaud, S AU - Raynaud S FAU - Bertucci, F AU - Bertucci F FAU - Birnbaum, D AU - Birnbaum D FAU - Chaffanet, M AU - Chaffanet M LA - eng PT - Journal Article PL - Poland TA - Balkan J Med Genet JT - Balkan journal of medical genetics : BJMG JID - 9806959 PMC - PMC3776676 OTO - NOTNLM OT - Breast cancers OT - Epigenome OT - Genome OT - Oncogenes OT - Transcriptome OT - Tumor suppressor genes EDAT- 2013/09/21 06:00 MHDA- 2013/09/21 06:01 PMCR- 2012/12/01 CRDT- 2013/09/21 06:00 PHST- 2013/09/21 06:00 [entrez] PHST- 2013/09/21 06:00 [pubmed] PHST- 2013/09/21 06:01 [medline] PHST- 2012/12/01 00:00 [pmc-release] AID - bjmg-15-02a-71 [pii] AID - 10.2478/v10034-012-0023-x [doi] PST - ppublish SO - Balkan J Med Genet. 2012 Dec;15(Suppl):71-4. doi: 10.2478/v10034-012-0023-x. PMID- 41240880 OWN - NLM STAT- MEDLINE DCOM- 20251130 LR - 20251130 IS - 1095-9157 (Electronic) IS - 0896-8411 (Linking) VI - 157 DP - 2025 Dec TI - Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology. PG - 103501 LID - S0896-8411(25)00146-5 [pii] LID - 10.1016/j.jaut.2025.103501 [doi] AB - OBJECTIVE: Elderly-onset systemic sclerosis (SSc) is relatively uncommon, and its clinical phenotype and prognostic implications remain poorly characterized, with conflicting evidence regarding disease course and outcomes. METHODS: Within the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry, we compared demographic and clinical characteristics of patients with elderly-onset SSc (≥70 years at the time of the first non-Raynaud's manifestation) to those with younger onset. Cross-sectional analyses, multivariable logistic regression, and unsupervised cluster analysis were conducted to identify features associated with elderly-onset SSc. Longitudinal analysis was performed to assess mortality risk within SSc patients and compared to the general Italian population. RESULTS: Elderly-onset accounted for 8.5 % (160/1893) SSc cases in SPRING. These patients exhibited fewer peripheral vascular complications (digital ulcers: 13 % vs. 23 %; p = 0.016), higher prevalence of anticentromere antibodies (60 % vs. 39 %; p = 0.007), a lower prevalence and likelihood of diffuse skin subset (OR 0.40; 95 % CI 0.19-0.83) but an increased risk of pulmonary arterial hypertension confirmed on right-heart catheterization (OR 14.1; 95 % CI 3.68-54.5) at multivariate analysis. As expected, patients with elderly onset SSc had an increased risk of death compared to younger-onset individuals. Compared with the age-, sex-, and calendar year-matched general Italian population, patients with SSc showed a fivefold increased mortality, with a trend toward a higher risk in young-onset (SMR 6.3; 95 %CI 4.1-9.1) compared with elderly-onset (SMR 4.5; 95 %CI 2.4-7.7) cases. CONCLUSIONS: Elderly-onset identifies a distinct clinical subset of SSc, mainly characterized by mild cutaneous and peripheral vascular involvement, but showing a greater burden of pulmonary vascular disease and increased mortality compared to the age-matched general population. CI - Copyright © 2025 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Tonutti, Antonio AU - Tonutti A AD - Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, 20072, Milan, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, 20089, Milan, Italy. FAU - Motta, Francesca AU - Motta F AD - Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, 20072, Milan, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, 20089, Milan, Italy. FAU - Bajocchi, Gianluigi AU - Bajocchi G AD - Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Careggi Hospital, Florence, Italy. FAU - Bruni, Cosimo AU - Bruni C AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Careggi Hospital, Florence, Italy. FAU - Orlandi, Martina AU - Orlandi M AD - Rheumatology Unit, School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Foti, Roberta AU - Foti R AD - Rheumatology Unit, AOU Policlinico San Marco, Catania, Italy. FAU - Cuomo, Giovanna AU - Cuomo G AD - Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Ariani, Alarico AU - Ariani A AD - Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma, Italy. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Lepri, Gemma AU - Lepri G AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Careggi Hospital, Florence, Italy. FAU - Girelli, Francesco AU - Girelli F AD - Rheumatology Unit, Ospedale GB Morgagni, AUSL Romagna, Forlì, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Department of Medicine, University of Padua, Padova, Italy; Department of Cardiac, Thoracic, and Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Division of Rheumatology and Clinical Immunology, Fondazione Policlinico Universitario A. Gemelli-IRCCS and Catholic University of the Sacred Heart, Rome, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Rheumatology Clinic, Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO, Department of Clinical Sciences & Community Health, Dipartimento di Eccellenza, 2023-2027, Università Degli Studi di Milano, Milan, Italy. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AD - Rheumatology Service "F. Miulli" General Hospital Acquaviva Delle Fonti - Department of Medicine and Surgery, LUM University "Giuseppe De Gennaro" Casamassima, Bari, Italy. FAU - Murdaca, Giuseppe AU - Murdaca G AD - Department of Internal Medicine, University of Genoa, Genoa and IRCCS Ospedale Policlinico San Martino, Genoa, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - Rheumatology Institute of Lucania (IReL), San Carlo Hospital, Potenza and University of Basilicata, Department of Health of Science, Potenza, Italy. FAU - Pettiti, Giorgio AU - Pettiti G AD - Rheumatology Unit, S. Croce e Carle Hospital, Cuneo, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Department of Rheumatology, University of Pisa, Pisa, Italy. FAU - Caminiti, Maurizio AU - Caminiti M AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy. FAU - Iuliano, Annamaria AU - Iuliano A AD - Rheumatology Unit, San Camillo-Forlanini Hospital, Rome, Italy. FAU - Ciano, Giovanni AU - Ciano G AD - Local Health Department, Ariano Irpino Hospital, Avellino, Italy. FAU - Beretta, Lorenzo AU - Beretta L AD - Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Bagnato, Gianluca AU - Bagnato G AD - Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy. FAU - Lubrano, Ennio AU - Lubrano E AD - Department of Rheumatology, University of Molise, Campobasso, Italy. FAU - De Andres, Ilenia AU - De Andres I AD - Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione Garibaldi, Catania, Italy. FAU - Idolazzi, Luca AU - Idolazzi L AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Saracco, Marta AU - Saracco M AD - Rheumatology Unit, Mauriziano-Umberto I Hospital, Turin, Italy. FAU - Agnes, Cecilia AU - Agnes C AD - Department of Medicine, Division of Rehabilitation, Turin, ASL TO5, Carmagnola, TO, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR)IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - Nivuori, Mariangela AU - Nivuori M AD - Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J) University of Bari, Italy. FAU - Cipolletta, Edoardo AU - Cipolletta E AD - Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy. FAU - Lumetti, Federica AU - Lumetti F AD - Rheumatology Unit, School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy. FAU - Spinella, Amelia AU - Spinella A AD - Rheumatology Unit, School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy. FAU - Cocchiara, Emanuele AU - Cocchiara E AD - Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. FAU - De Luca, Giacomo AU - De Luca G AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR)IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy. FAU - Codullo, Veronica AU - Codullo V AD - Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Visalli, Elisa AU - Visalli E AD - Rheumatology Unit, AOU Policlinico San Marco, Catania, Italy. FAU - Iandoli, Carlo AU - Iandoli C AD - Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy. FAU - Gigante, Antonietta AU - Gigante A AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Pellegrino, Greta AU - Pellegrino G AD - IRCCS Ospedale Galeazzi Sant'Ambrogio, Milan, Italy; Dipartimento di Scienze Biomediche e Cliniche, Università Degli Studi di Milano, Milan, Italy. FAU - Pigatto, Erika AU - Pigatto E AD - UOC Medicina Generale, Azienda Pedemontana 7, San Bassiano Hospital, Bassano Del Grappa, Italy. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - De Lorenzis, Enrico AU - De Lorenzis E AD - Division of Rheumatology and Clinical Immunology, Fondazione Policlinico Universitario A. Gemelli-IRCCS and Catholic University of the Sacred Heart, Rome, Italy. FAU - Mennillo, Gianna AU - Mennillo G AD - Rheumatology Institute of Lucania (IReL), San Carlo Hospital, Potenza and University of Basilicata, Department of Health of Science, Potenza, Italy. FAU - Di Battista, Marco AU - Di Battista M AD - Department of Rheumatology, University of Pisa, Pisa, Italy. FAU - Pagano-Mariano, Giuseppa AU - Pagano-Mariano G AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy. FAU - Furini, Federica AU - Furini F AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - Vultaggio, Licia AU - Vultaggio L AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - Parisi, Simone AU - Parisi S AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e Della Scienza, Turin, Italy. FAU - Ditto, Maria Chiara AU - Ditto MC AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e Della Scienza, Turin, Italy. FAU - Bianchi, Gerolamo AU - Bianchi G AD - Rheumatology Unit, Department of Medical Specialties, Local Health Trust 3, Genoa, Italy. FAU - Fusaro, Enrico AU - Fusaro E AD - Rheumatology Unit, Azienda Ospedaliera Universitaria Città Della Salute e Della Scienza, Turin, Italy. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AD - Rheumatology Unit, San Camillo-Forlanini Hospital, Rome, Italy. FAU - Govoni, Marcello AU - Govoni M AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - D'Angelo, Salvatore AU - D'Angelo S AD - Rheumatology Institute of Lucania (IReL), San Carlo Hospital, Potenza and University of Basilicata, Department of Health of Science, Potenza, Italy; Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy. FAU - Cozzi, Franco AU - Cozzi F AD - Department of Medicine, Villa Salus Hospital, Venice, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Careggi Hospital, Florence, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR)IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy. FAU - Doria, Andrea AU - Doria A AD - Department of Medicine, University of Padua, Padova, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Rheumatology Unit, School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Medical and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Azienda USL-IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Unit of Rheumatology, Department of Precision and Regenerative Medicine, Area Jonica (DiMePRe-J) University of Bari, Italy. FAU - Ferri, Clodoveo AU - Ferri C AD - Rheumatology Unit, School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Modena, Italy; Rheumatology Clinic 'Madonna Dello Scoglio', Cotronei, Crotone, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR)IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy; Inflammation Fibrosis and Ageing Initiative (INFLAGE), Division of Genetics and Cell Biology, IRCCS San Raffaele Scientific Institute, Milan, Italy. FAU - De Santis, Maria AU - De Santis M AD - Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, 20072, Milan, Italy; Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, 20089, Milan, Italy. FAU - De Angelis, Rossella AU - De Angelis R AD - Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy; IRCSS INRCA, Ancona, Italy. Electronic address: r.deangelis@staff.univpm.it. LA - eng PT - Journal Article DEP - 20251114 PL - England TA - J Autoimmun JT - Journal of autoimmunity JID - 8812164 SB - IM MH - Humans MH - *Scleroderma, Systemic/epidemiology/diagnosis/mortality MH - Male MH - Female MH - Italy/epidemiology MH - Aged MH - Registries MH - Age of Onset MH - Middle Aged MH - Cross-Sectional Studies MH - Adult MH - Aged, 80 and over MH - Rheumatology MH - Prognosis MH - Disease Progression MH - Age Factors OTO - NOTNLM OT - Clinical phenotype OT - Elderly OT - Geriatric autoimmunity OT - Systemic sclerosis EDAT- 2025/11/16 00:27 MHDA- 2025/12/01 00:30 CRDT- 2025/11/15 18:13 PHST- 2025/10/12 00:00 [received] PHST- 2025/11/07 00:00 [revised] PHST- 2025/11/08 00:00 [accepted] PHST- 2025/12/01 00:30 [medline] PHST- 2025/11/16 00:27 [pubmed] PHST- 2025/11/15 18:13 [entrez] AID - S0896-8411(25)00146-5 [pii] AID - 10.1016/j.jaut.2025.103501 [doi] PST - ppublish SO - J Autoimmun. 2025 Dec;157:103501. doi: 10.1016/j.jaut.2025.103501. Epub 2025 Nov 14. PMID- 36211201 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231002 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 7 IP - 3 DP - 2022 Oct TI - The need to accurately measure energy intake and expenditure in patients with systemic sclerosis. PG - 217-223 LID - 10.1177/23971983221095763 [doi] AB - BACKGROUND: Malnutrition is common in systemic sclerosis and patients are frequently underweight. However, the balance between assessed dietary energy intake versus expenditure has been neglected to date. This study aimed to assess energy (dietary) intakes and expenditures and to compare discrepancies in systemic sclerosis. METHODS: Thirty-six outpatients with systemic sclerosis completed the study. Demographics and clinical data were recorded. Functional questionnaires were completed. Predicted energy requirements were calculated. Over a consecutive 3-day period, patients completed an estimated food diary and wore a specialist energy expenditure monitor (SenseWear(®) Armband). Assessments of intake and expenditure were compared for individual patients, and the impact according to patient demographics, clinical manifestations and disease severity evaluated. RESULTS: Energy intake did not correlate with predicted (s = 0.117; p = 0.511) or measured (s = -0.039; p = 0.825) expenditures. Predicted and measured energy expenditures correlated, but actual values differed for individuals (intraclass correlation = 0.62; 95% limits of agreement = -459 to 751 kcal). Respiratory involvement was negatively correlated with number of steps (s = -0.350; p = 0.04) and time spent lying (s = 0.333; p = 0.05). There was a significant correlation between body mass index and predicted versus measured energy discrepancy (s = 0.41; p = 0.02), and this discrepancy was greater with higher body mass indices. CONCLUSION: There was no correlation between intake and either predicted or measured energy expenditure. Predicted and measured energy expenditures were strongly correlated yet differed for the individual patient. In patients with systemic sclerosis, where energy expenditure must be accurately assessed, it should be directly measured. CI - © The Author(s) 2022. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Department of Rheumatology, Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, UK. AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. FAU - Harrison, Elizabeth AU - Harrison E AD - Shrewsbury and Telford Hospitals NHS Trust, Shrewsbury, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. FAU - McLaughlin, John T AU - McLaughlin JT AD - Division of Diabetes, Endocrinology and Gastroenterology, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. FAU - Lal, Simon AU - Lal S AD - Division of Diabetes, Endocrinology and Gastroenterology, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester, UK. AD - Intestinal Failure Unit, Salford Care Organisation, Salford Royal NHS Foundation Trust, Salford, UK. LA - eng PT - Journal Article DEP - 20220530 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC9537710 OTO - NOTNLM OT - SenseWear® Armband OT - Systemic sclerosis OT - energy expenditure OT - energy intake OT - physical activity COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: M.H.: speaking fees from Actelion pharmaceuticals, Eli Lilly and Pfizer, outside of the submitted work. E.H.: was funded by the Raynaud’s and Scleroderma Association. A.H.: speaker’s fees from Actelion and Janssen. J.M.: none. S.L.: grants or contracts from any entity: Takeda and Baxter (not linked to this work); consulting fees: Takeda, Vectiv Bio, Fresenius and Zealand (not linked to this work); payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: Takeda, Baxter and Fresenius (not linked to this work); support for attending meetings and/or travel (not linked to this work). EDAT- 2022/10/11 06:00 MHDA- 2022/10/11 06:01 PMCR- 2023/10/01 CRDT- 2022/10/10 03:49 PHST- 2022/01/09 00:00 [received] PHST- 2022/04/01 00:00 [accepted] PHST- 2022/10/10 03:49 [entrez] PHST- 2022/10/11 06:00 [pubmed] PHST- 2022/10/11 06:01 [medline] PHST- 2023/10/01 00:00 [pmc-release] AID - 10.1177_23971983221095763 [pii] AID - 10.1177/23971983221095763 [doi] PST - ppublish SO - J Scleroderma Relat Disord. 2022 Oct;7(3):217-223. doi: 10.1177/23971983221095763. Epub 2022 May 30. PMID- 22939912 OWN - NLM STAT- MEDLINE DCOM- 20130925 LR - 20220317 IS - 1879-3460 (Electronic) IS - 0168-1605 (Linking) VI - 162 IP - 2 DP - 2013 Mar 15 TI - Review of Shiga-toxin-producing Escherichia coli (STEC) and their significance in dairy production. PG - 190-212 LID - S0168-1605(12)00433-3 [pii] LID - 10.1016/j.ijfoodmicro.2012.08.008 [doi] AB - The involvement of the pathogenic Shiga-toxin-producing Escherichia coli (STEC; also called verocytotoxic-producing E. coli or VTEC) in sporadic cases and disease outbreaks is presently increasing. Infrequent cases are due to ingestion of milk and dairy products. As ruminants are healthy carriers of STEC and most dairy products may provide these bacteria with favourable conditions for their growth, milk and dairy products are a potential source of STEC. But not all STEC serotypes are pathogens; only relatively small numbers in the entire family of STEC are pathogenic. This review focuses on the recent advances in understanding of STEC and their significance in milk and dairy products. It is intended to gather the information that is needed to understand how these bacteria are described, detected and characterised, how they contaminate milk and grow in dairy products, and how the dairy industry can prevent them from affecting the consumer. CI - Copyright © 2012 Elsevier B.V. All rights reserved. FAU - Farrokh, Choreh AU - Farrokh C AD - CNIEL- French Dairy Inter-branch Organisation, 42 rue de Chateaudun, FR-75009 Paris, France. cfarrokh@cniel.com FAU - Jordan, Kieran AU - Jordan K FAU - Auvray, Frederic AU - Auvray F FAU - Glass, Kathleen AU - Glass K FAU - Oppegaard, Hanne AU - Oppegaard H FAU - Raynaud, Sabrina AU - Raynaud S FAU - Thevenot, Delphine AU - Thevenot D FAU - Condron, Robin AU - Condron R FAU - De Reu, Koen AU - De Reu K FAU - Govaris, Alexander AU - Govaris A FAU - Heggum, Klaus AU - Heggum K FAU - Heyndrickx, Marc AU - Heyndrickx M FAU - Hummerjohann, Joerg AU - Hummerjohann J FAU - Lindsay, Denise AU - Lindsay D FAU - Miszczycha, Stephane AU - Miszczycha S FAU - Moussiegt, Sylvie AU - Moussiegt S FAU - Verstraete, Karen AU - Verstraete K FAU - Cerf, Olivier AU - Cerf O LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20120814 PL - Netherlands TA - Int J Food Microbiol JT - International journal of food microbiology JID - 8412849 RN - 0 (Virulence Factors) SB - IM MH - Animals MH - Bacterial Typing Techniques MH - Bacteriological Techniques MH - Dairy Products/*microbiology MH - Escherichia coli Infections/epidemiology/microbiology MH - *Food Microbiology MH - Humans MH - Microbial Viability MH - Milk/microbiology MH - Shiga-Toxigenic Escherichia coli/classification/genetics/*physiology MH - Virulence Factors/genetics EDAT- 2012/09/04 06:00 MHDA- 2013/09/26 06:00 CRDT- 2012/09/04 06:00 PHST- 2012/02/13 00:00 [received] PHST- 2012/07/31 00:00 [revised] PHST- 2012/08/01 00:00 [accepted] PHST- 2012/09/04 06:00 [entrez] PHST- 2012/09/04 06:00 [pubmed] PHST- 2013/09/26 06:00 [medline] AID - S0168-1605(12)00433-3 [pii] AID - 10.1016/j.ijfoodmicro.2012.08.008 [doi] PST - ppublish SO - Int J Food Microbiol. 2013 Mar 15;162(2):190-212. doi: 10.1016/j.ijfoodmicro.2012.08.008. Epub 2012 Aug 14. PMID- 33033851 OWN - NLM STAT- MEDLINE DCOM- 20210325 LR - 20250530 IS - 1432-0851 (Electronic) IS - 0340-7004 (Print) IS - 0340-7004 (Linking) VI - 70 IP - 4 DP - 2021 Apr TI - Immune reconstitution and clinical recovery following anti-CD28 antibody (TGN1412)-induced cytokine storm. PG - 1127-1142 LID - 10.1007/s00262-020-02725-2 [doi] AB - Cytokine storm can result from cancer immunotherapy or certain infections, including COVID-19. Though short-term immune-related adverse events are routinely described, longer-term immune consequences and sequential immune monitoring are not as well defined. In 2006, six healthy volunteers received TGN1412, a CD28 superagonist antibody, in a first-in-man clinical trial and suffered from cytokine storm. After the initial cytokine release, antibody effect-specific immune monitoring started on Day + 10 and consisted mainly of evaluation of dendritic cell and T-cell subsets and 15 serum cytokines at 21 time-points over 2 years. All patients developed problems with concentration and memory; three patients were diagnosed with mild-to-moderate depression. Mild neutropenia and autoantibody production was observed intermittently. One patient suffered from peripheral dry gangrene, required amputations, and had persistent Raynaud's phenomenon. Gastrointestinal irritability was noted in three patients and coincided with elevated γδT-cells. One had pruritus associated with elevated IgE levels, also found in three other asymptomatic patients. Dendritic cells, initially undetectable, rose to normal within a month. Naïve CD8(+) T-cells were maintained at high levels, whereas naïve CD4(+) and memory CD4(+) and CD8(+) T-cells started high but declined over 2 years. T-regulatory cells cycled circannually and were normal in number. Cytokine dysregulation was especially noted in one patient with systemic symptoms. Over a 2-year follow-up, cognitive deficits were observed in all patients following TGN1412 infusion. Some also had signs or symptoms of psychological, mucosal or immune dysregulation. These observations may discern immunopathology, treatment targets, and long-term monitoring strategies for other patients undergoing immunotherapy or with cytokine storm. FAU - Panoskaltsis, Nicki AU - Panoskaltsis N AUID- ORCID: 0000-0001-8972-5697 AD - Department of Haematology, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. nicki.panoskaltsis@emory.edu. AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. nicki.panoskaltsis@emory.edu. AD - Biological Systems Engineering Laboratory, Centre for Process Systems Engineering, Department of Chemical Engineering, Imperial College London, London, UK. nicki.panoskaltsis@emory.edu. AD - Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University School of Medicine, Atlanta, USA. nicki.panoskaltsis@emory.edu. AD - BioMedical Systems Engineering Laboratory, Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, USA. nicki.panoskaltsis@emory.edu. FAU - McCarthy, Neil E AU - McCarthy NE AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. AD - Centre for Immunobiology, The Blizard Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK. FAU - Stagg, Andrew J AU - Stagg AJ AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. AD - Centre for Immunobiology, The Blizard Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK. FAU - Mummery, Catherine J AU - Mummery CJ AD - Dementia Research Centre, National Hospital for Neurology and Neurosurgery, Queen Square, Department of Neurology, Northwick Park Hospital, London, UK. AD - National Hospital for Neurology and Neurosurgery, University College London Hospital, University College London, London, UK. FAU - Husni, Mariwan AU - Husni M AD - Central and North West London Mental Health NHS Foundation Trust, Northwick Park Hospital, London, UK. AD - Psychiatry Department, Arabian Gulf University, Manama, Kingdom of Bahrain. FAU - Arebi, Naila AU - Arebi N AD - Department of Gastroenterology and Intestinal Physiology, St. Mark's Hospital, London, UK. AD - Inflammatory Bowel Disease Clinical Service, St Mark's Hospital, London, UK. FAU - Greenstein, David AU - Greenstein D AD - Department of Vascular Surgery, North West London Hospitals NHS Trust, Northwick Park & St. Mark's Hospitals Site, London, UK. AD - Department of Vascular Surgery, Northwick Park Hospital and Imperial College London, London, UK. FAU - Price, Claire L AU - Price CL AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. AD - Lucid Group Communications, Buckinghamshire, UK. FAU - Al-Hassi, Hafid O AU - Al-Hassi HO AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. AD - Research Institute in Healthcare Science, Faculty of Science and Engineering, University of Wolverhampton, Wolverhampton, UK. FAU - Koutinas, Michalis AU - Koutinas M AD - Biological Systems Engineering Laboratory, Centre for Process Systems Engineering, Department of Chemical Engineering, Imperial College London, London, UK. AD - Department of Chemical Engineering, Cyprus University of Technology, Limassol, Cyprus. FAU - Mantalaris, Athanasios AU - Mantalaris A AD - Biological Systems Engineering Laboratory, Centre for Process Systems Engineering, Department of Chemical Engineering, Imperial College London, London, UK. AD - BioMedical Systems Engineering Laboratory, Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, USA. FAU - Knight, Stella C AU - Knight SC AD - Antigen Presentation Research Group, Imperial College London, Northwick Park & St. Mark's Campus, London, UK. LA - eng GR - BBS/E/F/000PR10353/BB_/Biotechnology and Biological Sciences Research Council/United Kingdom GR - BBS/E/F/000PR10356/BB_/Biotechnology and Biological Sciences Research Council/United Kingdom GR - MR/R008302/1/MRC_/Medical Research Council/United Kingdom GR - C18928/A8548/CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial PT - Journal Article DEP - 20201008 PL - Germany TA - Cancer Immunol Immunother JT - Cancer immunology, immunotherapy : CII JID - 8605732 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (CD28 Antigens) RN - POO0DOD3AS (TGN-1412) SB - IM MH - Adult MH - Antibodies, Monoclonal, Humanized/*adverse effects/pharmacology MH - CD28 Antigens/*agonists MH - COVID-19/*immunology MH - Cognitive Dysfunction/etiology/*immunology MH - Cohort Studies MH - Cytokine Release Syndrome/etiology/*immunology MH - Drug-Related Side Effects and Adverse Reactions/*immunology MH - Follow-Up Studies MH - Humans MH - Immunotherapy/*adverse effects MH - Male MH - SARS-CoV-2/*physiology MH - T-Lymphocytes/*immunology MH - Young Adult PMC - PMC7543968 OTO - NOTNLM OT - Cytokine release syndrome OT - Cytokine storm OT - Immune monitoring OT - Immune-related adverse events (irAEs) OT - Immunotherapy OT - TGN1412 COIS- None of the authors declare a financial conflict of interest. NP, SCK, MH, DG, CLP, HOA, MK, and AM declare no conflicts of interest. NEM is supported by a Career Development Award from The Medical Research Council (Grant Ref: MR/R008302/1) and is in receipt of a project grant from Bart’s and The London Charity (MGU0465). He has also received consultancy fees and funding for research from ImCheck Therapeutics SAS. AJS research is supported by grants from Gilead Sciences AbbVie, The Medical College of St Bartholomew's Hospital Trust, Bowel & Cancer Research and Bart's Charity. CLM has received consultancy fees from Roche and Biogen for clinical trials in Alzheimer's disease, not relevant to the work presented here. NA has received honoraria from Janssen and Pfizer and also consulting for Janssen. She also has an affiliation with Imperial College London. SCK, NEM and AJS have done contract work for Parexel pre-dating the work described in this report. At the time of this work and report, Parexel Clinical Trials Unit had a short-term contract with the Antigen Presentation Research Group (APRG) to use a Class II cabinet within the laboratory. The APRG has also been contracted to perform immunological studies by a pharmaceutical company, the tissue specimens for which were supplied on behalf of that company via Parexel which is located adjacent to the APRG department. There is no conflict of interest involved. EDAT- 2020/10/10 06:00 MHDA- 2021/03/26 06:00 PMCR- 2020/10/08 CRDT- 2020/10/09 05:40 PHST- 2020/05/05 00:00 [received] PHST- 2020/09/11 00:00 [accepted] PHST- 2020/10/10 06:00 [pubmed] PHST- 2021/03/26 06:00 [medline] PHST- 2020/10/09 05:40 [entrez] PHST- 2020/10/08 00:00 [pmc-release] AID - 10.1007/s00262-020-02725-2 [pii] AID - 2725 [pii] AID - 10.1007/s00262-020-02725-2 [doi] PST - ppublish SO - Cancer Immunol Immunother. 2021 Apr;70(4):1127-1142. doi: 10.1007/s00262-020-02725-2. Epub 2020 Oct 8. PMID- 41370133 OWN - NLM STAT- MEDLINE DCOM- 20251210 LR - 20260524 IS - 1469-493X (Electronic) IS - 1361-6137 (Linking) VI - 12 IP - 12 DP - 2025 Dec 10 TI - Prognostic factors associated with progression of open-angle glaucoma in adults. PG - CD015436 LID - 10.1002/14651858.CD015436.pub2 [doi] LID - CD015436 AB - BACKGROUND: Glaucoma is the leading cause of irreversible blindness globally, and the second leading cause of blindness in high-income countries. It is a chronic optic nerve disease that can lead to loss of vision, although it is usually asymptomatic until it progresses (worsens) to an advanced stage. Primary open angle glaucoma (POAG) is the most common type of glaucoma, and it is recognized as a multifactorial disorder. Pseudoexfoliative glaucoma (PXFG) is a common form of secondary open-angle glaucoma and has a higher rate of progression. The understanding of prognostic factors is useful for clinicians to estimate the risk of disease progression and to identify those who are at risk of losing sight early. OBJECTIVES: To identify risk factors associated with disease progression, defined as worsening or deterioration of functional visual outcomes and/or structural outcomes, amongst adults with POAG and PXFG. SEARCH METHODS: We searched CENTRAL, Ovid MEDLINE, Ovid Embase, and two trial registries on 15 August 2024. The search was supplemented by checking reference lists of eligible articles. We did not apply any restrictions on language or year of publication. SELECTION CRITERIA: We included randomized controlled trials, cohort, and case-control study designs. We excluded any study with less than two years of follow-up and a sample size of < 200. The targeted population consisted of adults ≥ 18 years of age of any sex with glaucoma type restricted to POAG, normal-tension glaucoma (NTG), and PXFG and no previous glaucoma surgery. Two review authors independently screened titles and abstracts, and full-text articles, to determine eligibility. The discrepancies were resolved through discussion with a third reviewer. The time points for the evaluation of outcomes were at a minimum of two years of follow-up. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data from included studies using pre-piloted data extraction forms in Covidence, SRDR+ and Microsoft Excel. We used the Quality in Prognosis Studies (QUIPS) tool to assess the risk of bias in Covidence. We conducted meta-analyses where homogeneous outcomes were reported, using a random-effects, generic inverse variance model. We reported hazard ratios (HR), odds ratio (OR), and risk ratios (RR) separately for each available prognostic factor and outcome, stratified by different time points and multivariable or univariable prognostic estimates, where possible. We evaluated and reported the certainty of evidence using the GRADE guidelines. MAIN RESULTS: We screened 16,188 titles and abstracts and retrieved 487 full-text reports from 239 studies for assessment. After full-text screening, we included 123 reports of 22 studies in this review. The 22 studies included 6082 participants. The mean ages of participants ranged from 50 to 78 years across studies. Sixteen of the included studies used visual field (VF) deterioration alone to detect and measure glaucoma progression. In six studies, disease progression was assessed by both functional and structural outcomes (e.g. retinal nerve fiber layer thickness changes by spectral-domain optical coherence tomography). We judged 19 of the 22 (86%) included studies to be at a high risk of bias overall. We found some prognostic factors had consistent evidence of a relationship with progression. Specifically, presence of disc hemorrhage (adjusted HR 2.03, 95% CI 1.55 to 2.67, 1068 participants, 3 studies; unadjusted HR 1.51, 95% CI 1.12 to 2.02, 961 participants, 3 studies; low certainty), presence of bilateral disease (adjusted HR 1.77, 95% CI 1.35 to 2.32, 771 participants, 2 studies; moderate certainty), and treatment for glaucoma (adjusted HR 0.44, 95% CI 0.31 to 0.61, 961 participants, 3 studies; unadjusted HR 0.56, 95% CI 0.44 to 0.72, 771, 2 studies; low certainty). The remaining factors had mixed evidence as to their prognostic associations with glaucoma progression, including a few factors that were expected to be important based on other literature. With regard to intraocular pressure (IOP) at baseline, our meta-analysis of the HR had sufficient evidence for an effect (adjusted HR 1.08, 95% CI 1.03 to 1.13, 913 participants, 3 studies, low certainty) while the OR did not (adjusted OR 0.96, 95% CI 0.84 to 1.10, 458 participants, 2 studies, low certainty) and more than half the studies reporting IOP found no evidence of an effect. Similarly, the pooled adjusted HR per 1-year increase in age at baseline was 1.01 (95% CI 0.97 to 1.05; 865 participants, 4 studies) with very low certainty of evidence, and studies had mixed results for its association with progression. Regarding sex, the combined adjusted analyses of two studies suggest females may have a 64% greater hazard of progression than males (HR 1.64, 95% CI 1.15 to 2.34; 961, 3 studies; low certainty). However, other study estimates for sexes that could not be combined were mixed in their directions of effect. We did not find consistent evidence to suggest that central corneal thickness (adjusted HR 1.13, 95% CI 0.85 to 1.51, 425 participants, 2 studies; unadjusted HR 1.00, 95% CI 1.00 to 1.00, 706 participants, 2 studies, very low certainty), systemic hypertension (adjusted HR 1.33, 95% CI 0.68 to 2.60, 731 participants, 3 studies; unadjusted HR 0.89, 95% CI 0.67 to 1.17, 771 participants, 2 studies; very low certainty), cardiovascular disease (adjusted HR 1.06, 95% CI 0.75 to 1.49, 771 participants, 2 studies; low certainty), migraine (unadjusted HR 1.06, 95% CI 0.75 to 1.49, 961 participants, 3 studies; very low certainty), or Raynaud's syndrome (unadjusted HR 1.21, 95% CI 0.85 to 1.73, 961 participants, 3 studies; very low certainty), have an effect on visual field progression. AUTHORS' CONCLUSIONS: There is moderate-certainty evidence to support the finding that bilateral disease is a prognostic factor associated with VF progression in people with glaucoma. There is low-certainty evidence that female sex and the presence of disc hemorrhage are associated with progression, while treatment with pharmacologics is protective of progression. The evidence is uncertain about the associations between progression and all other prognostic factors that we identified. Properly designed prognostic factor research studies are required in the future. FUNDING: NIH (NEI: UG1EY020522), USA; HRB, Ireland; HSC PHA (CBES-2018-001), Ireland REGISTRATION: Protocol available at doi.org/10.1002/14651858.CD015436. CI - Copyright © 2025 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. FAU - Piyasena, Mapa Prabhath AU - Piyasena MP AUID- ORCID: 0000-0002-0236-0101 AD - Vision and Eye Research Institute, Faculty of Health, Medicine and Social Care, School of Medicine, Anglia Ruskin University, Cambridge, UK. FAU - Daka, Qëndresë AU - Daka Q AD - Department of Pathophysiology, Medical Faculty, University of Prishtina "Hasan Prishtina", Prishtinë, Kosovo. AD - Eye Clinic, University Clinical Centre of Kosova, Prishtinë, Kosovo. FAU - Qureshi, Riaz AU - Qureshi R AD - Department of Ophthalmology, University of Colorado Anschutz, Aurora, Colorado, USA. AD - Department of Epidemiology, Colorado School of Public Health, Aurora, Colorado, USA. FAU - Roberti, Gloria AU - Roberti G AD - IRCCS-Fondazione Bietti, Rome, Italy. FAU - Michelessi, Manuele AU - Michelessi M AD - IRCCS-Fondazione Bietti, Rome, Italy. FAU - Liu, Su-Hsun AU - Liu SH AD - Department of Ophthalmology, University of Colorado Anschutz, Aurora, Colorado, USA. AD - Department of Epidemiology, Colorado School of Public Health, Aurora, Colorado, USA. FAU - Li, Tianjing AU - Li T AD - Department of Ophthalmology, University of Colorado Anschutz, Aurora, Colorado, USA. AD - Department of Epidemiology, Colorado School of Public Health, Aurora, Colorado, USA. FAU - Takwoingi, Yemisi AU - Takwoingi Y AD - Department of Applied Health Sciences, University of Birmingham, Birmingham, UK. FAU - Azuara-Blanco, Augusto AU - Azuara-Blanco A AD - Centre for Public Health, Institute of Clinical Sciences Block A, Queen's University Belfast, Belfast - Northern Ireland, UK. FAU - Virgili, Gianni AU - Virgili G AD - IRCCS-Fondazione Bietti, Rome, Italy. AD - NEUROFARBA, University of Florence, Florence, Italy. CN - supported by the Cochrane Eyes and Vision Group LA - eng GR - UG1 EY020522/EY/NEI NIH HHS/United States PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20251210 PL - England TA - Cochrane Database Syst Rev JT - The Cochrane database of systematic reviews JID - 100909747 SB - IM UOF - doi: 10.1002/14651858.CD015436 MH - Humans MH - *Glaucoma, Open-Angle/pathology MH - *Disease Progression MH - Prognosis MH - Adult MH - Randomized Controlled Trials as Topic MH - Risk Factors MH - *Exfoliation Syndrome MH - Intraocular Pressure MH - Bias MH - Female MH - Male PMC - PMC12694756 COIS- Riaz Qureshi, Su‐Hsun Liu, and Tianjing Li reported a grant from the National Eye Institute, National Institutes of Health, USA; payment to the institution. Riaz Qureshi, Su‐Hsun Liu, and Tianjing Li are part of Cochrane Eyes and Vision (CEV). Tianjing Li is Coordinating Editor for Cochrane Eyes and Vision (CEV) and Su‐Hsun Liu is the Managing Editor for CEV. Su‐Hsun Liu and Tianjing Li have no role in the editorial process for this review. Manuele Michelessi received payment from SIFI to attend meetings. Gloria Roberti received payment from NTC Pharma to participate in an Advisory Board; received payment from Zeiss to prepare a presentation at the World Glaucoma Congress; received payment from Omikron SRL to organise three meetings. Mapa Piyasena, Qëndresë Daka, Yemisi Takwoingi, Augusto Azuara‐Blanco, and Gianni Virgilli declared no conflicts of interest. EDAT- 2025/12/10 19:21 MHDA- 2025/12/10 19:22 PMCR- 2026/12/10 CRDT- 2025/12/10 12:43 PHST- 2026/12/10 00:00 [pmc-release] PHST- 2025/12/10 19:22 [medline] PHST- 2025/12/10 19:21 [pubmed] PHST- 2025/12/10 12:43 [entrez] AID - CD015436.pub2 [pii] AID - 10.1002/14651858.CD015436.pub2 [doi] PST - epublish SO - Cochrane Database Syst Rev. 2025 Dec 10;12(12):CD015436. doi: 10.1002/14651858.CD015436.pub2. PMID- 39255970 OWN - NLM STAT- MEDLINE DCOM- 20241104 LR - 20241119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 63 IP - 11 DP - 2024 Nov 1 TI - The 2024 British Society for Rheumatology guideline for management of systemic sclerosis-executive summary. PG - 2948-2955 LID - 10.1093/rheumatology/keae390 [doi] AB - This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, University College London, London, UK. FAU - De Lorenzis, Enrico AU - De Lorenzis E AUID- ORCID: 0000-0001-9819-105X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Roblin, Elen AU - Roblin E AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Goldman, Nina AU - Goldman N AD - Division of Medicine, University College London, London, UK. FAU - Alcacer-Pitarch, Begonya AU - Alcacer-Pitarch B AUID- ORCID: 0000-0002-2208-444X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Blamont, Emma AU - Blamont E AD - Scleroderma and Raynaud's UK (SRUK), London, UK. FAU - Buch, Maya H AU - Buch MH AUID- ORCID: 0000-0002-8962-5642 AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Carulli, Maresa AU - Carulli M AD - Department of Rheumatology, Hammersmith Hospitals NHS Foundation Trust, London, UK. FAU - Cotton, Caroline AU - Cotton C AD - Department of Rheumatology, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Derrett-Smith, Emma AU - Derrett-Smith E AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - Douglas, Karen AU - Douglas K AD - Department of Rheumatology, The Dudley Group NHS Foundation Trust, Dudley, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK (SRUK), London, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Gompels, Luke AU - Gompels L AD - Department of Rheumatology, Somerset NHS Foundation Trust, Taunton, UK. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle, UK. FAU - Herrick, Ariane AU - Herrick A AUID- ORCID: 0000-0003-4941-7926 AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Pain, Clare AU - Pain C AUID- ORCID: 0000-0003-4965-3259 AD - Department of Rheumatology, Alder Hey Children's Hospital, Liverpool, UK. FAU - Pantano, Georgina AU - Pantano G AD - Lived Experience, London, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Foundation Trust, Bristol, UK. FAU - Prabu, Athiveeraramapandian AU - Prabu A AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - O'Donoghue, Nuala AU - O'Donoghue N AD - Department of Dermatology, Salford Royal, Northern Care Alliance, Salford, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Interstitial Lung Disease Unit, Royal Brompton NHS Foundation Trust, London, UK. FAU - Royle, Jeremy AU - Royle J AD - Department of Rheumatology, University Hospitals NHS Foundation Trust, Leicester, UK. FAU - Samaranayaka, Muditha AU - Samaranayaka M AD - Department of Rheumatology, Salford Royal, Northern Care Alliance, Salford, UK. FAU - Spierings, Julia AU - Spierings J AD - Department of Rheumatology, University of Utrecht, Utrecht, Netherlands. FAU - Tynan, Aoife AU - Tynan A AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Warburton, Louise AU - Warburton L AD - Primary Care Sciences, Keele University, Keele, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Division of Medicine, University College London, London, UK. LA - eng GR - British Society for Rheumatology/ GR - Boehringer Ingelheim/ GR - AstraZeneca/ GR - AstraZeneca/ PT - Journal Article PT - Practice Guideline PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - doi: 10.1093/rheumatology/keae394 MH - Humans MH - *Scleroderma, Systemic/therapy/complications MH - *Rheumatology/standards MH - United Kingdom MH - Societies, Medical MH - Adult PMC - PMC11534113 OTO - NOTNLM OT - guideline OT - interstitial lung disease OT - management OT - scleroderma OT - systemic sclerosis EDAT- 2024/09/11 00:43 MHDA- 2024/11/04 18:24 PMCR- 2024/09/11 CRDT- 2024/09/10 20:02 PHST- 2024/02/22 00:00 [received] PHST- 2024/04/08 00:00 [accepted] PHST- 2024/11/04 18:24 [medline] PHST- 2024/09/11 00:43 [pubmed] PHST- 2024/09/10 20:02 [entrez] PHST- 2024/09/11 00:00 [pmc-release] AID - 7750185 [pii] AID - keae390 [pii] AID - 10.1093/rheumatology/keae390 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Nov 1;63(11):2948-2955. doi: 10.1093/rheumatology/keae390. PMID- 39255973 OWN - NLM STAT- MEDLINE DCOM- 20241104 LR - 20250530 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 63 IP - 11 DP - 2024 Nov 1 TI - The 2024 British Society for Rheumatology guideline for management of systemic sclerosis. PG - 2956-2975 LID - 10.1093/rheumatology/keae394 [doi] AB - This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, University College London, London, UK. FAU - De Lorenzis, Enrico AU - De Lorenzis E AUID- ORCID: 0000-0001-9819-105X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Roblin, Elen AU - Roblin E AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Goldman, Nina AU - Goldman N AD - Division of Medicine, University College London, London, UK. FAU - Alcacer-Pitarch, Begonya AU - Alcacer-Pitarch B AUID- ORCID: 0000-0002-2208-444X AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Blamont, Emma AU - Blamont E AD - Scleroderma and Raynaud's UK (SRUK), London, UK. FAU - Buch, Maya H AU - Buch MH AUID- ORCID: 0000-0002-8962-5642 AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Carulli, Maresa AU - Carulli M AD - Department of Rheumatology, Hammersmith Hospitals NHS Foundation Trust, London, UK. FAU - Cotton, Caroline AU - Cotton C AD - Department of Rheumatology, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Derrett-Smith, Emma AU - Derrett-Smith E AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - Douglas, Karen AU - Douglas K AD - Department of Rheumatology, The Dudley Group NHS Foundation Trust, Dudley, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK (SRUK), London, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Gompels, Luke AU - Gompels L AD - Department of Rheumatology, Somerset NHS Foundation Trust, Taunton, UK. FAU - Griffiths, Bridget AU - Griffiths B AD - Department of Rheumatology, Freeman Hospital, Newcastle, UK. FAU - Herrick, Ariane AU - Herrick A AUID- ORCID: 0000-0003-4941-7926 AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, University of Manchester, Manchester, UK. FAU - Pain, Clare AU - Pain C AUID- ORCID: 0000-0003-4965-3259 AD - Department of Rheumatology, Alder Hey Childrens Hospital, Liverpool, UK. FAU - Pantano, Georgina AU - Pantano G AD - Lived Experience, London, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Foundation Trust, Bristol, UK. FAU - Prabu, Athiveeraramapandian AU - Prabu A AD - Department of Rheumatology, University of Birmingham, Birmingham, UK. FAU - O'Donoghue, Nuala AU - O'Donoghue N AD - Department of Dermatology, Northern Care Alliance, Salford Royal, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Interstitial Lung Disease Unit, Royal Brompton NHS Foundation Trust, London, UK. FAU - Royle, Jeremy AU - Royle J AD - Department of Rheumatology, University Hospitals NHS Foundation Trust, Leicester, UK. FAU - Samaranayaka, Muditha AU - Samaranayaka M AD - Department of Rheumatology, Northern Care Alliance, Salford Royal, UK. FAU - Spierings, Julia AU - Spierings J AD - Department of Rheumatology, University of Utrecht, Utrecht, Netherlands. FAU - Tynan, Aoife AU - Tynan A AD - Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. FAU - Warburton, Louise AU - Warburton L AD - Primary Care Sciences, Keele University, Keele, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Division of Medicine, University College London, London, UK. LA - eng GR - 22398/VAC_/Versus Arthritis/United Kingdom GR - Astra Zeneca/ GR - British Society for Rheumatology/ GR - Boehringer Ingelheim/ PT - Journal Article PT - Practice Guideline PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM CIN - doi: 10.1093/rheumatology/keae390 MH - Humans MH - *Scleroderma, Systemic/therapy/complications MH - *Rheumatology/standards MH - United Kingdom MH - Societies, Medical MH - Adult PMC - PMC11534099 OTO - NOTNLM OT - guideline OT - interstitial lung disease OT - management OT - scleroderma OT - systemic sclerosis EDAT- 2024/09/11 00:43 MHDA- 2024/11/04 18:24 PMCR- 2024/09/11 CRDT- 2024/09/10 20:02 PHST- 2024/06/06 00:00 [received] PHST- 2024/06/06 00:00 [accepted] PHST- 2024/11/04 18:24 [medline] PHST- 2024/09/11 00:43 [pubmed] PHST- 2024/09/10 20:02 [entrez] PHST- 2024/09/11 00:00 [pmc-release] AID - 7750184 [pii] AID - keae394 [pii] AID - 10.1093/rheumatology/keae394 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Nov 1;63(11):2956-2975. doi: 10.1093/rheumatology/keae394. PMID- 39200224 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240901 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 12 IP - 8 DP - 2024 Aug 5 TI - The Potential Role of Butyrate in the Pathogenesis and Treatment of Autoimmune Rheumatic Diseases. LID - 10.3390/biomedicines12081760 [doi] LID - 1760 AB - The gut microbiota is a complex ecosystem of microorganisms residing in the human gastrointestinal tract, playing a crucial role in various biological processes and overall health maintenance. Dysbiosis, an imbalance in the composition and function of the gut microbiota, is linked to systemic autoimmune diseases (SAD). Short-chain fatty acids (SCFAs), especially butyrate, produced by the gut microbiota through the fermentation of dietary fibers, play a significant role in immunomodulation and maintaining intestinal homeostasis. Butyrate is essential for colonocyte energy, anti-inflammatory responses, and maintaining intestinal barrier integrity. Studies show reduced butyrate-producing bacteria in SAD patients, suggesting that increasing butyrate levels could have therapeutic benefits. Butyrate's anti-inflammatory effects and its potential therapeutic role have been studied in rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, systemic sclerosis, and Behçet's disease. Despite promising in vitro and animal model results, human studies are limited, and the optimal strategies for modulating dysbiosis in SADs remain elusive. This review explores the current evidence on the immunoregulatory role of butyrate and its potential therapeutic effects in SAD. FAU - Coccia, Carmela AU - Coccia C AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Bonomi, Francesco AU - Bonomi F AUID- ORCID: 0000-0001-7744-8549 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Lo Cricchio, Anna AU - Lo Cricchio A AUID- ORCID: 0009-0001-5933-9776 AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine AOUC, University of Florence, 50134 Florence, Italy. FAU - Russo, Edda AU - Russo E AUID- ORCID: 0000-0003-3141-1091 AD - Department of Experimental and Clinical Medicine, University of Florence, 50139 Florence, Italy. FAU - Peretti, Silvia AU - Peretti S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Bandini, Giulia AU - Bandini G AUID- ORCID: 0000-0002-2076-7319 AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine AOUC, University of Florence, 50134 Florence, Italy. FAU - Lepri, Gemma AU - Lepri G AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Bartoli, Francesca AU - Bartoli F AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Moggi-Pignone, Alberto AU - Moggi-Pignone A AD - Department of Experimental and Clinical Medicine, Division of Internal Medicine AOUC, University of Florence, 50134 Florence, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS9 7JT, UK. FAU - Furst, Daniel E AU - Furst DE AD - Department of Rheumatology, University of California Los Angeles, Los Angeles, CA 90095, USA. FAU - Matucci Cerinic, Marco AU - Matucci Cerinic M AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, 20132 Milan, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Scleroderma Unit, AOU Careggi, University of Florence, 50139 Florence, Italy. LA - eng PT - Journal Article PT - Review DEP - 20240805 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC11351188 OTO - NOTNLM OT - Behçet’s disease OT - Sjogren’s syndrome OT - butyrate OT - microbiota OT - rheumatoid arthritis OT - short-chain fatty acids OT - systemic autoimmune diseases OT - systemic lupus erythematosus OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2024/08/31 09:44 MHDA- 2024/08/31 09:45 PMCR- 2024/08/05 CRDT- 2024/08/29 01:06 PHST- 2024/06/26 00:00 [received] PHST- 2024/07/30 00:00 [revised] PHST- 2024/07/31 00:00 [accepted] PHST- 2024/08/31 09:45 [medline] PHST- 2024/08/31 09:44 [pubmed] PHST- 2024/08/29 01:06 [entrez] PHST- 2024/08/05 00:00 [pmc-release] AID - biomedicines12081760 [pii] AID - biomedicines-12-01760 [pii] AID - 10.3390/biomedicines12081760 [doi] PST - epublish SO - Biomedicines. 2024 Aug 5;12(8):1760. doi: 10.3390/biomedicines12081760. PMID- 37857429 OWN - NLM STAT- MEDLINE DCOM- 20231024 LR - 20231024 IS - 1399-3003 (Electronic) IS - 0903-1936 (Print) IS - 0903-1936 (Linking) VI - 62 IP - 4 DP - 2023 Oct TI - Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial. LID - 10.1183/13993003.00262-2023 [doi] LID - 2300262 AB - Individuals with IPF who follow a MUFA diet report a lower incidence of pirfenidone gastrointestinal adverse events than those that follow a SFA diet, which could explain the different prevalence in GI pirfenidone AEs reported by countries in IPF cohorts https://bit.ly/3LuzAUJ FAU - Molina-Molina, Maria AU - Molina-Molina M AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain mariamolinamolina@hotmail.com. AD - Spanish Research Network in Respiratory Diseases (CIBERES), Barcelona, Spain. FAU - Shull, Jessica Germaine AU - Shull JG AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. FAU - Vicens-Zygmunt, Vanesa AU - Vicens-Zygmunt V AUID- ORCID: 0000-0002-0940-3215 AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. AD - Spanish Research Network in Respiratory Diseases (CIBERES), Barcelona, Spain. FAU - Rivera-Ortega, Pilar AU - Rivera-Ortega P AD - NIHR Manchester Clinical Research Facility, Interstitial Lung Disease Unit, Respiratory Medicine Department, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK. FAU - Antoniou, Katerina AU - Antoniou K AUID- ORCID: 0000-0002-0283-5293 AD - Laboratory of Cellular and Molecular Pneumonology, Dept of Respiratory Medicine, School of Medicine, University of Crete, Heraklion, Greece. FAU - Bonella, Francesco AU - Bonella F AUID- ORCID: 0000-0001-7579-9767 AD - Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik, University Hospital, University of Duisburg Essen, Essen, Germany. FAU - Renzoni, Elisabetta AU - Renzoni E AD - NIHR Respiratory Clinical Research Facility, Royal Brompton Hospital, and Fibrosis Research Group, National Heart and Lung Institute, Imperial College, London, UK. FAU - Russell, Anne-Marie AU - Russell AM AUID- ORCID: 0000-0002-0468-3537 AD - Exeter Respiratory Institute, University of Exeter and Royal Devon University Hospitals NHS Foundation Trust, Exeter, UK. FAU - Maher, Toby M AU - Maher TM AD - Keck Medicine of University of Southern California, Los Angeles, CA, USA. AD - National Heart and Lung Institute, Imperial College, London, UK. FAU - Vancheri, Ada AU - Vancheri A AD - Dept of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, University Hospital "Policlinico G. Rodolico - San Marco", University of Catania, Catania, Italy. FAU - Bachs, Anna AU - Bachs A AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. FAU - Avilés, Victoria AU - Avilés V AD - Unit of Nutritional Support, University Hospital of Vall d'Hebron, Barcelona, Spain. FAU - Palma, Josep AU - Palma J AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. FAU - Bermudo, Guadalupe AU - Bermudo G AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. AD - Spanish Research Network in Respiratory Diseases (CIBERES), Barcelona, Spain. FAU - Suarez-Cuartin, Guillermo AU - Suarez-Cuartin G AUID- ORCID: 0000-0003-2320-6047 AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. AD - Spanish Research Network in Respiratory Diseases (CIBERES), Barcelona, Spain. FAU - Tebé, Cristian AU - Tebé C AD - Dept of Biostatistics, IDIBELL, Hospitalet de Llobregat, Barcelona, Spain. FAU - Rigo-Bonnin, Raul AU - Rigo-Bonnin R AD - Dept of Clinical Laboratory, Bellvitge University Hospital, IDIBELL, Barcelona, Spain. FAU - Montes-Worboys, Ana AU - Montes-Worboys A AD - Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, Research Lab 4126, IDIBELL, UB, Barcelona, Spain. AD - Spanish Research Network in Respiratory Diseases (CIBERES), Barcelona, Spain. FAU - Wijsenbeek, Marlies AU - Wijsenbeek M AD - Dept of Respiratory Medicine, Erasmus University Medical Centre, Rotterdam, The Netherlands. FAU - Vancheri, Carlo AU - Vancheri C AD - Dept of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, University Hospital "Policlinico G. Rodolico - San Marco", University of Catania, Catania, Italy. LA - eng SI - ClinicalTrials.gov/NCT03539289 PT - Clinical Trial PT - Letter PT - Research Support, Non-U.S. Gov't DEP - 20231019 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - D7NLD2JX7U (pirfenidone) RN - 0 (Pyridones) SB - IM MH - Humans MH - Anti-Inflammatory Agents, Non-Steroidal/adverse effects MH - Diet MH - *Idiopathic Pulmonary Fibrosis/drug therapy MH - *Pyridones/adverse effects MH - Treatment Outcome MH - Vital Capacity PMC - PMC10586233 OAB - Idiopathic pulmonary fibrosis (IPF) is a chronic and lethal interstitial lung disease (ILD) [1, 2]. Antifibrotic medications such as pirfenidone have been a turning point in the management of IPF, slowing of disease progression and improving survival [1–5]. OABL- eng COIS- Conflict of interest: M. Molina-Molina declares grants and fees received for research projects or scientific advice from Esteve-Teijin, Roche, Boehringer Ingelheim and Chiesi. V. Vicens-Zygmunt received fees for scientific advice from Boehringer Ingelheim. P. Rivera-Ortega declares speaker and consultation fees from Boehringer Ingelheim and Hoffmann-La Roche, and fees received for research projects from Boehringer Ingelheim, Hoffmann-La Roche, CSL Behring, FibroGen, Vicore Pharma AB, Gilead Sciences and Galecto; all research fees were paid to her institution. F. Bonella declares speaker and consultation fees from Boehringer Ingelheim, Hoffman La Roche and Fibrogene, outside the submitted work. E. Renzoni reports grants, lecture fees and advisory board fees from Boehringer Ingelheim, lecture fees from Roche and Chiesi, research grants from Raynaud's and Scleroderma, and support for attending meetings from Boehringer Ingelheim, outside the submitted work; all grants and fees were paid to her institution. A-M. Russell declares speaker and consultation fees from Boehringer Ingelheim and Hoffman-La Roche. T.M. Maher reports consultancy fees from AstraZeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, Galapagos, Galecto, GlaxoSmithKline, IQVIA, Pliant, Respivant Sciences, Roche/Genentech, Theravance Biopharma and Veracyte, and fees for presentations from Boehringer Ingelheim and Roche/Genentech. G. Suarez-Cuartin has received grants from Grifols, travel grants from Chiesi, Menarini and Boehringer Ingelheim, a speaker fee from Insmed, and advisory board fees from Insmed Inc. and Zambon. M. Wijsenbeek has received grants from Boehringer Ingelheim, The Netherlands Organisation for Health Research and Development, The Dutch Lung Foundation, Sarcoidose.nl and The Dutch Pulmonary Society, consulting fees from Boehringer Ingelheim, Galapagos, Bristol Myers Squibb, Galecto, Respivant, NeRRe Therapeutics, Horizon Therapeutics, PureTech health, Kinevant Sciences, Molecure and CLS Behring, speaker fees from Boehringer Ingelheim, Hoffman-La Roche, Novartis and CLS Behring, support for attending meetings from Boehringer Ingelheim, Galapagos and Hoffman-La Roche, and has participated in advisory boards of different patient associations (unpaid); all grants and fees were paid to her institution. C. Vancheri served on advisory committees of InterMune, Roche, AstraZeneca, Sanofi, Insmed, Zambon and Boehringer Ingelheim, and received lecture fees and nongovernmental research support from InterMune, Roche, Boehringer Ingelheim, Novartis, Chiesi, Menarini, AstraZeneca, GSK, Sanofi and Insmed. The rest of the authors have no relevant relationships to disclose. EDAT- 2023/10/20 00:43 MHDA- 2023/10/23 01:18 PMCR- 2023/10/19 CRDT- 2023/10/19 20:43 PHST- 2023/02/07 00:00 [received] PHST- 2023/08/18 00:00 [accepted] PHST- 2023/10/23 01:18 [medline] PHST- 2023/10/20 00:43 [pubmed] PHST- 2023/10/19 20:43 [entrez] PHST- 2023/10/19 00:00 [pmc-release] AID - 13993003.00262-2023 [pii] AID - ERJ-00262-2023 [pii] AID - 10.1183/13993003.00262-2023 [doi] PST - epublish SO - Eur Respir J. 2023 Oct 19;62(4):2300262. doi: 10.1183/13993003.00262-2023. Print 2023 Oct. PMID- 40445198 OWN - NLM STAT- MEDLINE DCOM- 20251003 LR - 20251003 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - 10 DP - 2025 Oct 1 TI - Cardiovascular and venous thromboembolic events in systemic sclerosis: epidemiological analysis of the Clinical Practice Research Datalink. PG - 5330-5337 LID - 10.1093/rheumatology/keaf285 [doi] AB - OBJECTIVES: Cardiovascular disease is a leading cause of mortality in systemic sclerosis (SSc). We investigated the association between SSc and the occurrence of both cardiovascular and thromboembolic events using the Clinical Practice Research Datalink (CPRD). METHODS: A validated case-ascertainment strategy identified SSc patients in the CPRD. A cohort study design examined rates of coronary arterial disease (CAD), cerebrovascular disease, peripheral artery disease (PAD) and venous thromboembolism (VTE) in SSc patients matched to non-SSc comparators by age, sex and location in a 1:6 ratio. Prevalent and incident cases of SSc were analysed separately. Cox regression analysis was used to determine hazard ratios for event occurrence, adjusted for traditional cardiovascular risk factors. RESULTS: We identified 877 eligible incident cases of SSc who were matched to 5262 patients without SSc (83.7% female), with a mean follow-up of 8 years. We identified a higher background prevalence of CAD, PAD and VTE at baseline (pre-dating diagnosis) in the SSc cohort. There was a significantly increased risk of CAD, PAD, peripheral venous thromboses and pulmonary embolism during follow-up in SSc compared with the non-SSc group (adjusted hazard ratios between 1.84-3.01), particularly amongst males. There was no increased risk of cerebrovascular disease. CONCLUSIONS: We have identified an increased risk of cardiovascular events and VTE, both prior to a diagnosis of SSc and within 8 years of SSc diagnosis. Work is needed to establish the mechanism of arterial/venous thrombotic events in SSc. This insight will facilitate more targeted and effective preventative strategies and improve outcomes in SSc. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. AD - Department of Life Sciences, University of Bath, Bath, UK. AD - Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK. FAU - Charlton, Rachel AU - Charlton R AD - Department of Life Sciences, University of Bath, Bath, UK. FAU - Ross, Laura AU - Ross L AD - Department of Medicine, University of Melbourne, Melbourne, Australia. AD - Department of Rheumatology, St Vincent's Hospital, Melbourne, Australia. FAU - McHugh, Neil J AU - McHugh NJ AD - Department of Life Sciences, University of Bath, Bath, UK. FAU - McGrogan, Anita AU - McGrogan A AD - Department of Life Sciences, University of Bath, Bath, UK. LA - eng GR - Scleroderma Raynaud's UK/ GR - Bath Institute for Rheumatic Diseases/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Male MH - Female MH - *Scleroderma, Systemic/complications/epidemiology MH - Middle Aged MH - *Venous Thromboembolism/epidemiology/etiology MH - Aged MH - Peripheral Arterial Disease/epidemiology/etiology MH - United Kingdom/epidemiology MH - Prevalence MH - Risk Factors MH - Incidence MH - Adult MH - *Coronary Artery Disease/epidemiology/etiology MH - *Cardiovascular Diseases/epidemiology/etiology MH - Cerebrovascular Disorders/epidemiology MH - Pulmonary Embolism/epidemiology MH - Proportional Hazards Models MH - Cohort Studies OTO - NOTNLM OT - cardiovascular disease OT - epidemiology OT - incidence OT - mortality OT - prevalence OT - smoking OT - systemic sclerosis OT - venous thromboembolism EDAT- 2025/05/30 18:15 MHDA- 2025/10/03 18:28 CRDT- 2025/05/30 10:53 PHST- 2025/02/03 00:00 [received] PHST- 2025/05/13 00:00 [accepted] PHST- 2025/10/03 18:28 [medline] PHST- 2025/05/30 18:15 [pubmed] PHST- 2025/05/30 10:53 [entrez] AID - 8153915 [pii] AID - 10.1093/rheumatology/keaf285 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Oct 1;64(10):5330-5337. doi: 10.1093/rheumatology/keaf285. PMID- 38287631 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20260518 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 3 IP - 12 DP - 2021 Dec TI - Pain levels and associated factors in the Scleroderma Patient-centered Intervention Network (SPIN) cohort: a multicentre cross-sectional study. PG - e844-e854 LID - S2665-9913(21)00318-0 [pii] LID - 10.1016/S2665-9913(21)00318-0 [doi] AB - BACKGROUND: Pain is an important and detrimental feature of systemic sclerosis but is often overlooked or deprioritised in research and clinical care. Raynaud's phenomenon, arthritis, and cutaneous ulcers are among the commonly reported disease manifestations of systemic sclerosis that could be associated with pain. We aimed to assess levels of pain intensity and interference and to evaluate disease factors associated with pain intensity and interference. METHODS: In this multicentre cross-sectional study, participants from the Scleroderma Patient-centered Intervention Network cohort who completed pain intensity and interference measures (Patient Reported Outcomes Information System-29 profile, version 2·0) as part of baseline assessments were included. Patients were recruited from 46 centres in Australia, Canada, France, Mexico, Spain, the UK, and the USA between April 15, 2014, and Jan 7, 2020. Eligible patients included those aged 18 years or older who met the criteria for systemic sclerosis devised by the American College of Rheumatology and the European League Against Rheumatism. Associations of pain intensity and pain interference with systemic sclerosis-related variables and overlap syndromes, controlling for sociodemographic variables, were assessed with multiple linear regression. Continuous independent variables were standardised. FINDINGS: Among 2157 participants with systemic sclerosis (268 [12%] males and 1889 [88%] females), 1870 (87%) reported mild, moderate, or severe pain (defined as ≥1 on a 0 to 10 scale), and 815 (38%) reported moderate or severe pain (defined as ≥5). Moreover, 757 (35%) participants reported moderate or severe pain interference. Greater pain intensity was independently associated with female sex (0·58 points [95% CI 0·26-0·90]), non-White race or ethnicity (0·50 points [0·21-0·79]), fewer years in formal education (0·30 points per SD [0·19-0·41]), country (reference: USA; Canada: 0·29 points [0·01-0·57] and UK: 0·58 points [0·21-0·95]), greater body-mass index (0·35 points per SD [0·24-0·45]); joint contractures (0·67 points [0·39-0·94]), digital ulcers (0·33 points [0·10-0·55]), gastrointestinal involvement (0·66 points [0·33-0·98]), skin involvement (measured using modified Rodnan skin score; 0·22 points per SD [0·10-0·35]), rheumatoid arthritis (0·96 points [0·50-1·43]), and Sjögren's syndrome (0·42 points [0·01-0·83]). Pain interference results were similar. INTERPRETATION: Pain is common among people with systemic sclerosis. Controlling for sociodemographic variables, greater pain was associated with multiple systemic sclerosis-related manifestations, including joint contractures, digital ulcers, gastrointestinal involvement, skin involvement, and the presence of overlap syndromes. Health-care providers should work with patients to address pain, including identifying and addressing systemic sclerosis manifestations associated with their pain, and supporting behavioural approaches to minimise impact on function and quality of life. FUNDING: Canadian Institutes of Health Research, Arthritis Society, The Lady Davis Institute for Medical Research of the Jewish General Hospital, Jewish General Hospital Foundation, McGill University, Scleroderma Society of Ontario, Scleroderma Canada, Sclérodermie Québec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, and Scleroderma Queensland. CI - Copyright © 2021 Elsevier Ltd. All rights reserved. FAU - Lee, Yvonne C AU - Lee YC AD - Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. Electronic address: yvonne.lee@northwestern.edu. FAU - Fox, Rina S AU - Fox RS AD - Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, Netherlands; Department of Medical Psychology, Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Levis, Brooke AU - Levis B AD - Centre for Prognosis Research, School of Medicine, Keele University, Staffordshire, UK. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Welling, Joep AU - Welling J AD - Dutch patient organization for systemic autoimmune diseases, Utrecht, Netherlands; Federation of European Scleroderma Associations, Brussels, Belgium. FAU - Sauvé, Maureen AU - Sauvé M AD - Scleroderma Society of Ontario and Scleroderma Canada, Hamilton, ON, Canada. FAU - Mouthon, Luc AU - Mouthon L AD - Service de Medecine Interne, Centre de Reference Maladies Autoimmunes et Systemiques Rares d'Ile de France, Hopital Cochin, Assistance-Publique Hopitaux de Paris, Centre, Universite de Paris, Paris, France. FAU - Benedetti, Andrea AU - Benedetti A AD - Department of Medicine, McGill University, Montreal, QC, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada; Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, QC, Canada. FAU - Bartlett, Susan J AU - Bartlett SJ AD - Department of Medicine, McGill University, Montreal, QC, Canada; Research Institute of the McGill University Health Centre, McGill University Health Centre, Montreal, QC, Canada. FAU - Varga, John AU - Varga J AD - University of Michigan, Ann Arbor, MI, USA. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada; Department of Medicine, McGill University, Montreal, QC, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada; Department of Psychiatry, McGill University, Montreal, QC, Canada; Department of Psychology, McGill University, Montreal, QC, Canada; Department of Educational and Counselling Psychology, McGill University, Montreal, QC, Canada; Biomedical Ethics Unit, McGill University, Montreal, QC, Canada. CN - Scleroderma Patient-centered Intervention Network Investigators LA - eng PT - Journal Article PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 SB - IM COIS- Declaration of interests YCL reports grant support from Pfizer, participation on a data safety monitoring board for Highland Instruments, stock ownership in Cigna-Express Scripts, and medical writing support from Sanofi and Eli Lilly; all outside of the submitted work. LM reports personal fees from Actelion (Johnson & Johnson), grants from LFB, non-financial support from Octapharma, and non-financial support from Grifols, all outside of the submitted work. All other authors declare no competing interests. FIR - Henry, Richard S IR - Henry RS FIR - Gottesman, Karen IR - Gottesman K FIR - Hudson, Marie IR - Hudson M FIR - Hummers, Laura K IR - Hummers LK FIR - Malcarne, Vanessa L IR - Malcarne VL FIR - Mayes, Maureen D IR - Mayes MD FIR - Nielson, Warren R IR - Nielson WR FIR - Riggs, Robert IR - Riggs R FIR - Assassi, Shervin IR - Assassi S FIR - El-Baalbaki, Ghassan IR - El-Baalbaki G FIR - Ells, Carolyn IR - Ells C FIR - Fligelstone, Kim IR - Fligelstone K FIR - Fortuné, Catherine IR - Fortuné C FIR - Frech, Tracy IR - Frech T FIR - Gietzen, Amy IR - Gietzen A FIR - Guillot, Geneviève IR - Guillot G FIR - Harel, Daphna IR - Harel D FIR - Hinchcliff, Monique IR - Hinchcliff M FIR - Johnson, Sindhu R IR - Johnson SR FIR - Larche, Maggie IR - Larche M FIR - Leite, Catarina IR - Leite C FIR - Nguyen, Christelle IR - Nguyen C FIR - Nielsen, Karen IR - Nielsen K FIR - Pope, Janet IR - Pope J FIR - Rannou, François IR - Rannou F FIR - Richard, Michelle IR - Richard M FIR - Rodriguez-Reyna, Tatiana Sofia IR - Rodriguez-Reyna TS FIR - Schouffoer, Anne A IR - Schouffoer AA FIR - Suarez-Almazor, Maria E IR - Suarez-Almazor ME FIR - Agard, Christian IR - Agard C FIR - Ait Abdallah, Nassim IR - Ait Abdallah N FIR - Albert, Alexandra IR - Albert A FIR - André, Marc IR - André M FIR - Bernstein, Elana J IR - Bernstein EJ FIR - Berthier, Sabine IR - Berthier S FIR - Bissonnette, Lyne IR - Bissonnette L FIR - Bruns, Alessandra IR - Bruns A FIR - Carreira, Patricia IR - Carreira P FIR - Casadevall, Marion IR - Casadevall M FIR - Chaigne, Benjamin IR - Chaigne B FIR - Chung, Lorinda IR - Chung L FIR - Correia, Chase IR - Correia C FIR - Crichi, Benjamin IR - Crichi B FIR - Denton, Christopher IR - Denton C FIR - Domsic, Robyn IR - Domsic R FIR - Dunne, James V IR - Dunne JV FIR - Dunogue, Bertrand IR - Dunogue B FIR - Fare, Regina IR - Fare R FIR - Farge-Bancel, Dominique IR - Farge-Bancel D FIR - Fortin, Paul R IR - Fortin PR FIR - Gordon, Jessica IR - Gordon J FIR - Granel-Rey, Brigitte IR - Granel-Rey B FIR - Gyger, Genevieve IR - Gyger G FIR - Hachulla, Eric IR - Hachulla E FIR - Herrick, Ariane L IR - Herrick AL FIR - Hoa, Sabrina IR - Hoa S FIR - Ikic, Alena IR - Ikic A FIR - Jones, Niall IR - Jones N FIR - Kafaja, Suzanne IR - Kafaja S FIR - Khalidi, Nader IR - Khalidi N FIR - Lambert, Marc IR - Lambert M FIR - Launay, David IR - Launay D FIR - Maillard, Hélène IR - Maillard H FIR - Maltez, Nancy IR - Maltez N FIR - Manning, Joanne IR - Manning J FIR - Marie, Isabelle IR - Marie I FIR - Martin, Maria IR - Martin M FIR - Martin, Thierry IR - Martin T FIR - Masetto, Ariel IR - Masetto A FIR - Maurier, François IR - Maurier F FIR - Mekinian, Arsene IR - Mekinian A FIR - Melchor, Sheila IR - Melchor S FIR - Nikpour, Mandana IR - Nikpour M FIR - Olagne, Louis IR - Olagne L FIR - Poindron, Vincent IR - Poindron V FIR - Proudman, Susanna IR - Proudman S FIR - Régent, Alexis IR - Régent A FIR - Rivière, Sébastien IR - Rivière S FIR - Robinson, David IR - Robinson D FIR - Rodriguez, Esther IR - Rodriguez E FIR - Roux, Sophie IR - Roux S FIR - Smets, Perrine IR - Smets P FIR - Sobanski, Vincent IR - Sobanski V FIR - Spiera, Robert IR - Spiera R FIR - Steen, Virginia IR - Steen V FIR - Sutton, Evelyn IR - Sutton E FIR - Thorne, Carter IR - Thorne C FIR - Wilcox, Pearce IR - Wilcox P FIR - Bourgeault, Angelica IR - Bourgeault A FIR - Cañedo Ayala, Mara IR - Cañedo Ayala M FIR - Carboni Jiménez, Andrea IR - Carboni Jiménez A FIR - Discepola, Marie-Nicole IR - Discepola MN FIR - Gagarine, Maria IR - Gagarine M FIR - Nordlund, Julia IR - Nordlund J FIR - Østbø, Nora IR - Østbø N EDAT- 2021/12/01 00:00 MHDA- 2021/12/01 00:01 CRDT- 2024/01/30 01:02 PHST- 2021/06/14 00:00 [received] PHST- 2021/09/14 00:00 [revised] PHST- 2021/09/15 00:00 [accepted] PHST- 2021/12/01 00:01 [medline] PHST- 2021/12/01 00:00 [pubmed] PHST- 2024/01/30 01:02 [entrez] AID - S2665-9913(21)00318-0 [pii] AID - 10.1016/S2665-9913(21)00318-0 [doi] PST - ppublish SO - Lancet Rheumatol. 2021 Dec;3(12):e844-e854. doi: 10.1016/S2665-9913(21)00318-0. PMID- 31112379 OWN - NLM STAT- MEDLINE DCOM- 20190709 LR - 20260518 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 380 IP - 26 DP - 2019 Jun 27 TI - Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. PG - 2518-2528 LID - 10.1056/NEJMoa1903076 [doi] AB - BACKGROUND: Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis and a leading cause of systemic sclerosis-related death. Nintedanib, a tyrosine kinase inhibitor, has been shown to have antifibrotic and antiinflammatory effects in preclinical models of systemic sclerosis and ILD. METHODS: We conducted a randomized, double-blind, placebo-controlled trial to investigate the efficacy and safety of nintedanib in patients with ILD associated with systemic sclerosis. Patients who had systemic sclerosis with an onset of the first non-Raynaud's symptom within the past 7 years and a high-resolution computed tomographic scan that showed fibrosis affecting at least 10% of the lungs were randomly assigned, in a 1:1 ratio, to receive 150 mg of nintedanib, administered orally twice daily, or placebo. The primary end point was the annual rate of decline in forced vital capacity (FVC), assessed over a 52-week period. Key secondary end points were absolute changes from baseline in the modified Rodnan skin score and in the total score on the St. George's Respiratory Questionnaire (SGRQ) at week 52. RESULTS: A total of 576 patients received at least one dose of nintedanib or placebo; 51.9% had diffuse cutaneous systemic sclerosis, and 48.4% were receiving mycophenolate at baseline. In the primary end-point analysis, the adjusted annual rate of change in FVC was -52.4 ml per year in the nintedanib group and -93.3 ml per year in the placebo group (difference, 41.0 ml per year; 95% confidence interval [CI], 2.9 to 79.0; P = 0.04). Sensitivity analyses based on multiple imputation for missing data yielded P values for the primary end point ranging from 0.06 to 0.10. The change from baseline in the modified Rodnan skin score and the total score on the SGRQ at week 52 did not differ significantly between the trial groups, with differences of -0.21 (95% CI, -0.94 to 0.53; P = 0.58) and 1.69 (95% CI, -0.73 to 4.12 [not adjusted for multiple comparisons]), respectively. Diarrhea, the most common adverse event, was reported in 75.7% of the patients in the nintedanib group and in 31.6% of those in the placebo group. CONCLUSIONS: Among patients with ILD associated with systemic sclerosis, the annual rate of decline in FVC was lower with nintedanib than with placebo; no clinical benefit of nintedanib was observed for other manifestations of systemic sclerosis. The adverse-event profile of nintedanib observed in this trial was similar to that observed in patients with idiopathic pulmonary fibrosis; gastrointestinal adverse events, including diarrhea, were more common with nintedanib than with placebo. (Funded by Boehringer Ingelheim; SENSCIS ClinicalTrials.gov number, NCT02597933.). CI - Copyright © 2019 Massachusetts Medical Society. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Highland, Kristin B AU - Highland KB AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Gahlemann, Martina AU - Gahlemann M AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Azuma, Arata AU - Azuma A AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Fischer, Aryeh AU - Fischer A AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Mayes, Maureen D AU - Mayes MD AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Raghu, Ganesh AU - Raghu G AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Sauter, Wiebke AU - Sauter W AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Girard, Mannaig AU - Girard M AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Alves, Margarida AU - Alves M AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Clerisme-Beaty, Emmanuelle AU - Clerisme-Beaty E AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Stowasser, Susanne AU - Stowasser S AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Tetzlaff, Kay AU - Tetzlaff K AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Kuwana, Masataka AU - Kuwana M AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). FAU - Maher, Toby M AU - Maher TM AD - From the Department of Rheumatology, University Hospital Zurich, Zurich (O.D.), and Boehringer Ingelheim (Schweiz), Basel (M. Gahlemann) - both in Switzerland; the Respiratory Institute, Cleveland Clinic, Cleveland (K.B.H.); the Departments of Pulmonary Medicine and Oncology (A.A.) and Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine, Tokyo; University of Colorado School of Medicine, Aurora (A.F.); the Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston (M.D.M.); University of Washington, Seattle (G.R.); Boehringer Ingelheim Pharma, Biberach an der Riss (W.S.), Boehringer Ingelheim International, Ingelheim am Rhein (M.A., E.C.-B., S.S., K.T.), and the Department of Sports Medicine, University of Tübingen, Tübingen (K.T.) - all in Germany; Boehringer Ingelheim France, Reims, France (M. Girard); and the National Heart and Lung Institute, Imperial College London, and the National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London (T.M.M.). CN - SENSCIS Trial Investigators LA - eng SI - ClinicalTrials.gov/NCT02597933 GR - CS-2013-13-017/DH_/Department of Health/United Kingdom PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20190520 PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 RN - 0 (Enzyme Inhibitors) RN - 0 (Indoles) RN - EC 2.7.10.1 (Protein-Tyrosine Kinases) RN - G6HRD2P839 (nintedanib) SB - IM CIN - Rev Med Interne. 2019 Dec;40(12):775-777. doi: 10.1016/j.revmed.2019.08.009. PMID: 31477468 CIN - N Engl J Med. 2019 Oct 17;381(16):1595. doi: 10.1056/NEJMc1910735. PMID: 31618554 CIN - N Engl J Med. 2019 Oct 17;381(16):1595-1596. doi: 10.1056/NEJMc1910735. PMID: 31618555 MH - Administration, Oral MH - Adult MH - Diarrhea/chemically induced MH - Disease Progression MH - Double-Blind Method MH - Enzyme Inhibitors/adverse effects/*therapeutic use MH - Female MH - Humans MH - Indoles/adverse effects/*therapeutic use MH - Lung Diseases, Interstitial/*drug therapy/etiology/physiopathology MH - Male MH - Middle Aged MH - Protein-Tyrosine Kinases/*antagonists & inhibitors MH - Scleroderma, Systemic/*complications/drug therapy MH - Vital Capacity FIR - Bergna, M IR - Bergna M FIR - Casado, G IR - Casado G FIR - Mannucci Walter, P IR - Mannucci Walter P FIR - Proudman, S IR - Proudman S FIR - Stevens, W IR - Stevens W FIR - Thakkar, V IR - Thakkar V FIR - Troy, L IR - Troy L FIR - Loeffler-Ragg, J IR - Loeffler-Ragg J FIR - Olschewski, H IR - Olschewski H FIR - Bondue, B IR - Bondue B FIR - Houssiau, F IR - Houssiau F FIR - Smith, V IR - Smith V FIR - Wuyts, W IR - Wuyts W FIR - Johnson, S IR - Johnson S FIR - Keystone, E IR - Keystone E FIR - Khalidi, N IR - Khalidi N FIR - Levesque, M IR - Levesque M FIR - Maturana Rozas, R IR - Maturana Rozas R FIR - Silva Orellana, A IR - Silva Orellana A FIR - Huang, C IR - Huang C FIR - Li, J IR - Li J FIR - Jiang, Z IR - Jiang Z FIR - Liu, Y IR - Liu Y FIR - Xiao, W IR - Xiao W FIR - Xu, J IR - Xu J FIR - Zeng, X IR - Zeng X FIR - Zheng, Y IR - Zheng Y FIR - Zou, H IR - Zou H FIR - Becvar, R IR - Becvar R FIR - Madsen, H IR - Madsen H FIR - Søndergaard, K IR - Søndergaard K FIR - Kilpeläinen, M IR - Kilpeläinen M FIR - Myllärniemi, M IR - Myllärniemi M FIR - Agard, C IR - Agard C FIR - Allanore, Y IR - Allanore Y FIR - Bourdin, A IR - Bourdin A FIR - Cottin, V IR - Cottin V FIR - Crestani, B IR - Crestani B FIR - Diot, E IR - Diot E FIR - Dominique, S IR - Dominique S FIR - Hachulla, E IR - Hachulla E FIR - Jouneau, S IR - Jouneau S FIR - Leroy, S IR - Leroy S FIR - Nunes, H IR - Nunes H FIR - Prevot, G IR - Prevot G FIR - Wallaert, B IR - Wallaert B FIR - Wemeau, L IR - Wemeau L FIR - Aringer, M IR - Aringer M FIR - Bewig, B IR - Bewig B FIR - Blaas, S IR - Blaas S FIR - Distler, J IR - Distler J FIR - Ehrchen, J IR - Ehrchen J FIR - Ewert, R IR - Ewert R FIR - Gläser, S IR - Gläser S FIR - Henes, J IR - Henes J FIR - Hunzelmann, N IR - Hunzelmann N FIR - König, R IR - König R FIR - Kötter, I IR - Kötter I FIR - Kreuter, M IR - Kreuter M FIR - Prasse, A IR - Prasse A FIR - Schulze-Koops, H IR - Schulze-Koops H FIR - Sfikakis, P IR - Sfikakis P FIR - Vlachoyiannopoulos, P IR - Vlachoyiannopoulos P FIR - Losonczy, G IR - Losonczy G FIR - Behera, D IR - Behera D FIR - Gayathri Devi, H J IR - Gayathri Devi HJ FIR - Kadel, J IR - Kadel J FIR - Kawedia, M IR - Kawedia M FIR - Kumar, D IR - Kumar D FIR - Kumar, U IR - Kumar U FIR - Lokhande, R IR - Lokhande R FIR - Malpani, A IR - Malpani A FIR - Mohan, M IR - Mohan M FIR - Nalawade, A IR - Nalawade A FIR - Parakh, U IR - Parakh U FIR - Swarnakar, R IR - Swarnakar R FIR - Shobha, V IR - Shobha V FIR - Thangakunam, B IR - Thangakunam B FIR - Udwadia, Z IR - Udwadia Z FIR - Henry, M IR - Henry M FIR - O'Reilly, K IR - O'Reilly K FIR - Balbir-Gurman, A IR - Balbir-Gurman A FIR - Kramer, M IR - Kramer M FIR - Litinsky, I IR - Litinsky I FIR - Rosner, I IR - Rosner I FIR - Cutolo, M IR - Cutolo M FIR - Gabrielli, A IR - Gabrielli A FIR - Iaccarino, L IR - Iaccarino L FIR - Pesci, A IR - Pesci A FIR - Riccieri, V IR - Riccieri V FIR - Vettori, S IR - Vettori S FIR - Funakubo, Y IR - Funakubo Y FIR - Inoue, Y IR - Inoue Y FIR - Kawakami, A IR - Kawakami A FIR - Kawaguchi, Y IR - Kawaguchi Y FIR - Kawamura, T IR - Kawamura T FIR - Kondoh, Y IR - Kondoh Y FIR - Kuwana, M IR - Kuwana M FIR - Nanki, T IR - Nanki T FIR - Nishioka, Y IR - Nishioka Y FIR - Nozawa, K IR - Nozawa K FIR - Oguragawa, T IR - Oguragawa T FIR - Okamoto, M IR - Okamoto M FIR - Sano, H IR - Sano H FIR - Sasai, R IR - Sasai R FIR - Sasaki, N IR - Sasaki N FIR - Suda, T IR - Suda T FIR - Takahashi, H IR - Takahashi H FIR - Takeuchi, T IR - Takeuchi T FIR - Tanaka, S IR - Tanaka S FIR - Yamasaki, Y IR - Yamasaki Y FIR - Ch'ng, S S IR - Ch'ng SS FIR - Cheah, C IR - Cheah C FIR - Kan, S IR - Kan S FIR - Raja Mohamed, R B IR - Raja Mohamed RB FIR - Selman, M IR - Selman M FIR - de Vries-Bouwstra, J K IR - de Vries-Bouwstra JK FIR - van den Toorn, L IR - van den Toorn L FIR - Vonken, M IR - Vonken M FIR - Voskuyl, A E IR - Voskuyl AE FIR - Hoffmann-Vold, A M IR - Hoffmann-Vold AM FIR - Seip, M IR - Seip M FIR - Dankiewicz-Fares, I IR - Dankiewicz-Fares I FIR - Olesiejuk, R IR - Olesiejuk R FIR - Pulka, G IR - Pulka G FIR - Szepietowski, J IR - Szepietowski J FIR - Alves, J IR - Alves J FIR - Bernardes, M IR - Bernardes M FIR - Cordeiro, A IR - Cordeiro A FIR - Costa, J IR - Costa J FIR - Neves, S IR - Neves S FIR - Salvador, M J IR - Salvador MJ FIR - Alegre Sancho, J IR - Alegre Sancho J FIR - Carreira Delgado, P IR - Carreira Delgado P FIR - Castellví Barranco, I IR - Castellví Barranco I FIR - Cifrián Martínez, J IR - Cifrián Martínez J FIR - Guillén Del Castillo, A IR - Guillén Del Castillo A FIR - Ovalles, J G IR - Ovalles JG FIR - López-Longo, F J IR - López-Longo FJ FIR - Rivera Gallego, A IR - Rivera Gallego A FIR - Freire Dapena, M C IR - Freire Dapena MC FIR - Román Ivorra, J A IR - Román Ivorra JA FIR - Ekwall, A-K H IR - Ekwall AH FIR - Maurer, B IR - Maurer B FIR - Mihai, C M IR - Mihai CM FIR - Müller, R IR - Müller R FIR - Mahakkanukrauh, A IR - Mahakkanukrauh A FIR - Nantiruj, K IR - Nantiruj K FIR - Siripaitoon, B IR - Siripaitoon B FIR - Denton, C P IR - Denton CP FIR - Herrick, A IR - Herrick A FIR - Madhok, R IR - Madhok R FIR - Maher, T M IR - Maher TM FIR - West, A IR - West A FIR - Bascom, R IR - Bascom R FIR - Criner, G IR - Criner G FIR - Csuka, M E IR - Csuka ME FIR - Dematte D'Amico, J IR - Dematte D'Amico J FIR - Ettinger, N IR - Ettinger N FIR - Fischer, A IR - Fischer A FIR - Gerbino, A IR - Gerbino A FIR - Gerke, A IR - Gerke A FIR - Glassberg, M IR - Glassberg M FIR - Glazer, C IR - Glazer C FIR - Golden, J IR - Golden J FIR - Gripaldo, R IR - Gripaldo R FIR - Gupta, N IR - Gupta N FIR - Hamblin, M IR - Hamblin M FIR - Highland, K IR - Highland K FIR - Ho, L IR - Ho L FIR - Huggins, J T IR - Huggins JT FIR - Hummers, L IR - Hummers L FIR - Jones, L IR - Jones L FIR - Kahaleh, M IR - Kahaleh M FIR - Khanna, D IR - Khanna D FIR - Kim, H IR - Kim H FIR - Lancaster, L H IR - Lancaster LH FIR - Luckhardt, T IR - Luckhardt T FIR - Mayes, M IR - Mayes M FIR - Mendoza Ballesteros, F IR - Mendoza Ballesteros F FIR - Mooney, J IR - Mooney J FIR - Mohabir, P IR - Mohabir P FIR - Morrissey, B IR - Morrissey B FIR - Moua, T IR - Moua T FIR - Padilla, M IR - Padilla M FIR - Patel, N IR - Patel N FIR - Perez, R IR - Perez R FIR - Roman, J IR - Roman J FIR - Rossman, M IR - Rossman M FIR - Russell, T IR - Russell T FIR - Saketkoo, L IR - Saketkoo L FIR - Shah, A IR - Shah A FIR - Shlobin, O IR - Shlobin O FIR - Scholand, M B IR - Scholand MB FIR - Simmssetts, R IR - Simmssetts R FIR - Spiera, R IR - Spiera R FIR - Steen, V IR - Steen V FIR - Veeraraghavan, S IR - Veeraraghavan S FIR - Weigt, S IR - Weigt S EDAT- 2019/05/22 06:00 MHDA- 2019/07/10 06:00 CRDT- 2019/05/22 06:00 PHST- 2019/05/22 06:00 [pubmed] PHST- 2019/07/10 06:00 [medline] PHST- 2019/05/22 06:00 [entrez] AID - 10.1056/NEJMoa1903076 [doi] PST - ppublish SO - N Engl J Med. 2019 Jun 27;380(26):2518-2528. doi: 10.1056/NEJMoa1903076. Epub 2019 May 20. PMID- 40965427 OWN - NLM STAT- MEDLINE DCOM- 20251204 LR - 20251204 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 38 IP - 1 DP - 2026 Jan 1 TI - What have we learned about systemic sclerosis from the EUSTAR database? PG - 53-59 LID - 10.1097/BOR.0000000000001128 [doi] AB - PURPOSE OF REVIEW: This review provides a timely synthesis of key findings derived from the EUSTAR (European Scleroderma Trials and Research) database, the largest international registry dedicated to systemic sclerosis (SSc), now including over 27 000 patients worldwide. As interest grows in real-world data and precision medicine in rare diseases, EUSTAR offers a uniquely rich, longitudinal dataset built over two decades of global collaboration. With sustained growth, more than 1000 new patients enrolled annually, this registry continues to inform clinical practice and research with contemporary, diverse patient data. RECENT FINDINGS: Analyses from EUSTAR have clarified disease phenotypes and trajectories, identified predictors of organ involvement and mortality, and validated outcome measures including the EUSTAR Activity Index. Studies have also revealed heterogeneity in treatment patterns, supported the refinement of classification criteria, and highlighted regional disparities in care. The registry has been a foundation for innovative research approaches such as emulated clinical trials, comparative effectiveness analyses, and external control arms for interventional studies. SUMMARY: EUSTAR has become a reference model for collaborative research in rare diseases. Its findings have directly informed guidelines and routine management of SSc. Future directions include integrating digital tools, artificial intelligence, and expanding the registry's role in clinical trial design and personalized medicine. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases. San Raffaele Hospital. and Vita-Salute San Raffaele University, Milano, Italy. FAU - Vonk, Madelon C AU - Vonk MC AD - Radboud University Medical Center, Department of Rheumatology; Nijmegen, The Netherlands. FAU - Osborne, Thomas AU - Osborne T AD - Leeds Raynaud's and Scleroderma Program, NIHR Biomedical Research Centre Leeds, Leeds, UK. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Hughes, Michael AU - Hughes M AD - Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Santiago, Tânia AU - Santiago T AD - Centro Hospital e Universitário de Coimbra, University of Coimbra, Coimbra, Portugal. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Raynaud's and Scleroderma Program, NIHR Biomedical Research Centre Leeds, Leeds, UK. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - CHU de Bordeaux, Rheumatology Department, Bordeaux, France. LA - eng PT - Journal Article PT - Review DEP - 20250918 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 SB - IM MH - Humans MH - *Scleroderma, Systemic/therapy/diagnosis/epidemiology MH - *Registries MH - *Databases, Factual MH - Europe OTO - NOTNLM OT - EUSTAR database OT - cohort OT - systemic sclerosis EDAT- 2025/09/18 13:14 MHDA- 2025/12/04 13:39 CRDT- 2025/09/18 10:33 PHST- 2025/12/04 13:39 [medline] PHST- 2025/09/18 13:14 [pubmed] PHST- 2025/09/18 10:33 [entrez] AID - 00002281-990000000-00196 [pii] AID - 10.1097/BOR.0000000000001128 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2026 Jan 1;38(1):53-59. doi: 10.1097/BOR.0000000000001128. Epub 2025 Sep 18. PMID- 23708256 OWN - NLM STAT- MEDLINE DCOM- 20140320 LR - 20221207 IS - 1791-2423 (Electronic) IS - 1019-6439 (Print) IS - 1019-6439 (Linking) VI - 43 IP - 2 DP - 2013 Aug TI - Genetic differences between Asian and Caucasian chronic lymphocytic leukemia. PG - 561-5 LID - 10.3892/ijo.2013.1966 [doi] AB - Chronic lymphocytic leukemia (CLL) is a common hematological malignancy in Western countries. However, this disease is very rare in Asian countries. It is not clear whether the mechanisms of development of CLL in Caucasians and Asians are the same. We compared genetic abnormalities in Asian and Caucasian CLL using 250k GeneChip arrays. Both Asian and Caucasian CLL had four common genetic abnormalities: deletion of 13q14.3, trisomy 12, abnormalities of ATM (11q) and abnormalities of 17p. Interestingly, trisomy 12 and deletion of 13q14.3 were mutually exclusive in both groups. We also found that deletions of miR 34b/34c (11q), caspase 1/4/5 (11q), Rb1 (13q) and DLC1 (8p) are common in both ethnic groups. Asian CLL more frequently had gain of 3q and 18q. These suggest that classic genomic changes in the Asian and Caucasina CLL are same. Further, we found amplification of IRF4 and deletion of the SP140/SP100 genes; these genes have been reported as CLL-associated genes by previous genome-wide-association study. We have found classic genomic abnormalities in Asian CLL as well as novel genomic alteration in CLL. FAU - Kawamata, Norihiko AU - Kawamata N AD - Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA. kawamatan@cshs.org FAU - Moreilhon, Chimene AU - Moreilhon C FAU - Saitoh, Takayuki AU - Saitoh T FAU - Karasawa, Masamitsu AU - Karasawa M FAU - Bernstein, Brian K AU - Bernstein BK FAU - Sato-Otsubo, Aiko AU - Sato-Otsubo A FAU - Ogawa, Seishi AU - Ogawa S FAU - Raynaud, Sophie AU - Raynaud S FAU - Koeffler, H Phillip AU - Koeffler HP LA - eng GR - GM008243/GM/NIGMS NIH HHS/United States GR - R01CA02603831/CA/NCI NIH HHS/United States GR - U54CA143930/CA/NCI NIH HHS/United States GR - T32 GM008243/GM/NIGMS NIH HHS/United States GR - U54 CA143930/CA/NCI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20130527 PL - Greece TA - Int J Oncol JT - International journal of oncology JID - 9306042 RN - 0 (Biomarkers, Tumor) RN - 0 (Genetic Markers) SB - IM MH - Asian People/*genetics MH - Biomarkers, Tumor/genetics MH - Chromosome Aberrations MH - Genetic Markers/genetics MH - Humans MH - Leukemia, Lymphocytic, Chronic, B-Cell/*epidemiology/*genetics MH - Sequence Deletion/genetics MH - Trisomy/genetics MH - White People/*genetics PMC - PMC3775563 EDAT- 2013/05/28 06:00 MHDA- 2014/03/22 06:00 PMCR- 2013/05/27 CRDT- 2013/05/28 06:00 PHST- 2012/12/27 00:00 [received] PHST- 2013/02/22 00:00 [accepted] PHST- 2013/05/28 06:00 [entrez] PHST- 2013/05/28 06:00 [pubmed] PHST- 2014/03/22 06:00 [medline] PHST- 2013/05/27 00:00 [pmc-release] AID - ijo-43-02-0561 [pii] AID - 10.3892/ijo.2013.1966 [doi] PST - ppublish SO - Int J Oncol. 2013 Aug;43(2):561-5. doi: 10.3892/ijo.2013.1966. Epub 2013 May 27. PMID- 40124984 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260320 LR - 20260320 IS - 2397-1991 (Electronic) IS - 2397-1983 (Print) IS - 2397-1983 (Linking) VI - 10 IP - 3 DP - 2025 Oct TI - Physician-patient communication in the treatment of systemic sclerosis-associated interstitial lung disease: A narrative review and recommendations. PG - 224-236 LID - 10.1177/23971983251324803 [doi] AB - Interstitial lung disease is a common complication and cause of mortality in patients with systemic sclerosis. Pharmacotherapy for systemic sclerosis-associated interstitial lung disease was mostly limited to off-label use of immunosuppressive drugs until recently, when two drugs became licenced for this condition: nintedanib, an antifibrotic agent, and tocilizumab, a targeted anti-inflammatory/immunomodulatory therapy licenced in the United States. In chronic diseases, communication between physicians and patients is associated with treatment adherence, patient satisfaction, and clinical outcomes. This review of physician-patient communication during systemic sclerosis-associated interstitial lung disease treatment covers key issues identified by studies in Japan, the United States and Europe, as well as the clinical experience, opinion, and recommendations of the physician and patient advocate authors. As discussed, recent surveys in Japan found low usage of guideline-recommended immunosuppressive drugs for systemic sclerosis-associated interstitial lung disease and physician dissatisfaction with them. Physicians and patients in Japan also had differing perceptions about what had been said during consultations, suggesting the need to improve physician-patient communication. Other studies in Japan, the United States and Europe made several key findings. Notably, most patients feel uneasy at the diagnosis of systemic sclerosis-associated interstitial lung disease, and both physicians and patients avoid discussing prognosis and mortality. Furthermore, a white-coat barrier hinders patients raising topics important to them. For physicians, listening and empathy may be key for building rapport with patients. Importantly, physicians and patients have different cognitive models of systemic sclerosis-associated interstitial lung disease, creating communication challenges. There are also similarities and differences in clinical practice and physician-patient communication between countries that are important to consider. From the patient's perspective, key factors include the quality of the first consultation, physician empathy and active listening, and space to ask questions. Efforts to improve physician-patient communication include peer mentoring, patient self-education (such as the 'Self-Manage Scleroderma' website from the University of Michigan), and shared decision-making - although not all activities will necessarily be appropriate everywhere. CI - © The Author(s) 2025. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Saito, Aiko AU - Saito A AD - Nippon Boehringer Ingelheim Co., Ltd, Tokyo, Japan. FAU - Farrington, Sue AU - Farrington S AD - Patient advocate. AD - Scleroderma and Raynaud's UK, London, UK. AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. FAU - Galetti, Ilaria AU - Galetti I AD - Patient advocate. AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. AD - Gruppo Italiano per la Lotta alla Sclerodermia, Milan, Italy. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Scleroderma Program and Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. LA - eng PT - Journal Article PT - Review DEP - 20250320 PL - England TA - J Scleroderma Relat Disord JT - Journal of scleroderma and related disorders JID - 101685427 PMC - PMC11926815 OTO - NOTNLM OT - Interstitial lung disease OT - physician–patient communication OT - shared decision-making OT - systemic sclerosis COIS- The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: The authors received no direct compensation related to the development of the manuscript. M.K. has received consulting fees, speaking fees and research grants from argenx, Asahi Kasei, AstraZeneca, Boehringer Ingelheim, Chugai, GlaxoSmithKline, Janssen, Kissei, MBL, Mitsubishi Tanabe, Mochida, Nippon Shinyaku, Ono Pharmaceuticals and Taisho. A.S. is an employee of Nippon Boehringer Ingelheim Co. Ltd. S.F. and I.G. have nothing to disclose. C.P.D. reports consulting fees from Roche, Boehringer Ingelheim, GlaxoSmithKline, Horizon Therapeutics, CSL Behring; honoraria from Boehringer Ingelheim and Janssen; and grants from Horizon Therapeutics, GlaxoSmithKline and Servier. D.K. reports consultancies with Amgen, argenx, Astra Zeneca BMS, Boehringer Ingelheim, Genentech/Roche, GSK, Mitsubishi Tanabi, Merck, Novartis and Zura Bio. EDAT- 2025/03/24 12:45 MHDA- 2025/03/24 12:46 PMCR- 2026/03/20 CRDT- 2025/03/24 06:13 PHST- 2024/09/13 00:00 [received] PHST- 2025/01/28 00:00 [accepted] PHST- 2025/03/24 12:46 [medline] PHST- 2025/03/24 12:45 [pubmed] PHST- 2025/03/24 06:13 [entrez] PHST- 2026/03/20 00:00 [pmc-release] AID - 10.1177_23971983251324803 [pii] AID - 10.1177/23971983251324803 [doi] PST - epublish SO - J Scleroderma Relat Disord. 2025 Mar 20;10(3):224-236. doi: 10.1177/23971983251324803. eCollection 2025 Oct. PMID- 30289986 OWN - NLM STAT- MEDLINE DCOM- 20190722 LR - 20250530 IS - 1365-2362 (Electronic) IS - 0014-2972 (Linking) VI - 49 IP - 1 DP - 2019 Jan TI - Functional and phenotypic heterogeneity of Th17 cells in health and disease. PG - e13032 LID - 10.1111/eci.13032 [doi] AB - BACKGROUND: Th17 cells have nonredundant roles in maintaining immunity, particularly at mucosal surfaces. These roles are achieved principally through the production of cytokines and the recruitment of other immune cells to maintain the integrity of mucosal barriers and prevent the dissemination of microorganisms. Th17 cells are heterogeneous and exhibit a considerable degree of plasticity. This allows these cells to respond to changing environmental challenges. However, Th17 cells also play pro-inflammatory roles in chronic autoimmune diseases. The trigger(s) that initiate these Th17 responses in chronic autoimmune diseases remain unclear. DESIGN: In this report, we provide an overview of studies involving animal models, patient data, genome wide association studies and clinical trials targeting IL-17 for treatment of patients to gain a better understanding of the pathogenic roles of Th17 cells play in a range of autoimmune diseases. RESULTS: The report sheds light on likely triggers that initiate or perpetuate Th17 responses that promote chronic inflammation and autoimmunity. The divergent effects of tumour necrosis factor alpha blockade on Th17 cells in patients, is explored. Furthermore, we highlight the role of Th17 cells in inducing autoreactive B cells, leading to autoantibody production. Pathogenic bacterial species can change Th17 cell phenotype and responses. These findings provide insights into how Th17 cells could be induced to promoting autoimmune disease pathogenesis. CONCLUSION: This article provides an overview of the distinct roles Th17 cells play in maintaining immunity at mucosal surfaces and in skin mucosa and how their functional flexibility could be linked with chronic inflammation in autoimmune rheumatic diseases. CI - © 2018 Stichting European Society for Clinical Investigation Journal Foundation. FAU - Bystrom, Jonas AU - Bystrom J AUID- ORCID: 0000-0002-6458-3028 AD - William Harvey Research Institute, Queen Mary University of London, London, UK. FAU - Clanchy, Felix I L AU - Clanchy FIL AD - Kennedy Institute of Rheumatology, University of Oxford, Oxford, UK. FAU - Taher, Taher E AU - Taher TE AD - Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK. FAU - Al-Bogami, Mohammed AU - Al-Bogami M AD - Radiology Department, Alnakheel Medical Centre, Riyadh, Kingdom of Saudi Arabia. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology and Connective Tissue Diseases, University College London, Royal Free Hospital, London, UK. FAU - Abraham, David J AU - Abraham DJ AD - Centre for Rheumatology and Connective Tissue Diseases, University College London, Royal Free Hospital, London, UK. FAU - Williams, Richard O AU - Williams RO AD - Kennedy Institute of Rheumatology, University of Oxford, Oxford, UK. FAU - Mageed, Rizgar A AU - Mageed RA AD - William Harvey Research Institute, Queen Mary University of London, London, UK. LA - eng GR - 19427/VAC_/Versus Arthritis/United Kingdom GR - 21810/VAC_/Versus Arthritis/United Kingdom GR - G0802513/MRC_/Medical Research Council/United Kingdom GR - Raynaud's and Scleroderma Association/ PT - Journal Article PT - Review DEP - 20181101 PL - England TA - Eur J Clin Invest JT - European journal of clinical investigation JID - 0245331 SB - IM MH - Arthritis, Rheumatoid/etiology/immunology MH - Autoimmune Diseases/*immunology MH - Autoimmunity/physiology MH - Cell Differentiation/immunology MH - Genome-Wide Association Study MH - Humans MH - Intestines/immunology MH - Lupus Erythematosus, Systemic/etiology/immunology MH - Phenotype MH - Psoriasis/etiology/immunology MH - Scleroderma, Systemic/etiology/immunology MH - Signal Transduction/immunology MH - Skin/immunology MH - Th17 Cells/*physiology EDAT- 2018/10/06 06:00 MHDA- 2019/07/23 06:00 CRDT- 2018/10/06 06:00 PHST- 2018/02/02 00:00 [received] PHST- 2018/09/14 00:00 [revised] PHST- 2018/10/01 00:00 [accepted] PHST- 2018/10/06 06:00 [pubmed] PHST- 2019/07/23 06:00 [medline] PHST- 2018/10/06 06:00 [entrez] AID - 10.1111/eci.13032 [doi] PST - ppublish SO - Eur J Clin Invest. 2019 Jan;49(1):e13032. doi: 10.1111/eci.13032. Epub 2018 Nov 1. PMID- 41369954 OWN - NLM STAT- MEDLINE DCOM- 20260121 LR - 20260318 IS - 2168-6084 (Electronic) IS - 2168-6068 (Print) IS - 2168-6068 (Linking) VI - 162 IP - 1 DP - 2026 Jan 1 TI - Botulinum Toxin for Refractory Digital Ischemia and Ulcers in Systemic Sclerosis: A Systematic Review and Meta-Analysis. PG - 47-54 LID - 10.1001/jamadermatol.2025.4929 [doi] AB - IMPORTANCE: Acute digital ischemia, digital ulcers, and gangrene are debilitating complications of systemic sclerosis and other vasculopathies and are often refractory to standard vasodilator and immunosuppressive therapies. Botulinum toxin (BTX) has emerged as a potential rescue therapy, but its clinical effectiveness and safety remain unclear. OBJECTIVE: To evaluate the effectiveness and safety of BTX injections for ischemic digital complications and identify predictors of treatment response using individual participant data (IPD). DATA SOURCES: MEDLINE (PubMed), Embase (Ovid), and Scopus were searched from inception through April 20, 2024. STUDY SELECTION: Eligible studies included patients who presented with acute digital ischemia, ischemic digital ulcers, or gangrene. Studies were limited to Raynaud disease without digital ulcers or gangrene were excluded. Two reviewers independently screened articles using Covidence, with discrepancies resolved by consensus with the senior author. Of 116 studies screened, 31 (27%) met inclusion criteria. DATA EXTRACTION AND SYNTHESIS: Data were extracted in duplicate and study quality was assessed using the Joanna Briggs Institute checklist. Descriptive statistics were used to summarize baseline characteristics, treatment regimens, and outcomes. MAIN OUTCOMES AND MEASURES: The primary outcome was complete response (CR), which was defined as resolution of ischemia or ulcer healing. Secondary outcomes included adverse events and time to response. Cox regression was used to identify factors associated with CR. RESULTS: This systematic review and IPD meta-analysis included 119 patients (72 female individuals [75.0%]; mean [SD] age, 49.0 [15.1] years). BTX was associated with high CR rates for ischemia (93.1%), ulcers (90.1%), and gangrene (87.5%). Adverse events were infrequent, with transient muscle weakness (7.6%) and injection site pain (5.9%) being most common. No associated factors reached statistical significance in multivariable models, but autoimmune etiology and younger age were associated with faster response in Kaplan-Meier analyses. CONCLUSIONS AND RELEVANCE: The results of this systematic review and IPD meta-analysis suggest that BTX injections appear to be a safe and effective adjunct for refractory digital ischemia in systemic sclerosis. Prospective trials are needed to confirm long-term effectiveness and standardize administration protocols. FAU - Zhu, Catherine AU - Zhu C AD - Faculty of Medicine, McGill University, Montreal, Quebec, Canada. FAU - Peri, Katya AU - Peri K AD - Faculty of Medicine, McGill University, Montreal, Quebec, Canada. FAU - Silotch, Catherine AU - Silotch C AD - Division of Clinical and Translational Research, McGill University, Montreal, Quebec, Canada. FAU - Prosty, Connor AU - Prosty C AD - Faculty of Medicine, McGill University, Montreal, Quebec, Canada. FAU - BinJadeed, Hessah AU - BinJadeed H AD - Division of Dermatology, McGill University, Montreal, Quebec, Canada. FAU - Muntyanu, Anastasiya AU - Muntyanu A AD - Division of Dermatology, University of Toronto, Toronto, Ontario, Canada. FAU - Nagarajan, Mahalakshmi AU - Nagarajan M AD - Department of Plastic and Reconstructive Surgery, Saint Helens and Knowsley National Health Service Trust, Whiston Hospital, Prescot, England. FAU - McArthur, Paul AU - McArthur P AD - Department of Plastic and Reconstructive Surgery, Saint Helens and Knowsley National Health Service Trust, Whiston Hospital, Prescot, England. FAU - Benedetti, Andrea AU - Benedetti A AD - Faculty of Medicine, McGill University, Montreal, Quebec, Canada. AD - Departments of Medicine and of Epidemiology, Biostatistics and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Netchiporouk, Elena AU - Netchiporouk E AD - Division of Dermatology, McGill University, Montreal, Quebec, Canada. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review PL - United States TA - JAMA Dermatol JT - JAMA dermatology JID - 101589530 RN - EC 3.4.24.69 (Botulinum Toxins) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) SB - IM CIN - JAMA Dermatol. 2026 Jan 1;162(1):54. doi: 10.1001/jamadermatol.2025.4879. PMID: 41369945 EIN - JAMA Dermatol. 2026 Jan 1;162(1):103. doi: 10.1001/jamadermatol.2025.5957. PMID: 41563735 MH - Female MH - Humans MH - *Botulinum Toxins/administration & dosage/adverse effects MH - *Botulinum Toxins, Type A/administration & dosage/adverse effects MH - *Fingers/blood supply MH - Gangrene/etiology/drug therapy MH - *Ischemia/drug therapy/etiology MH - *Scleroderma, Systemic/complications MH - *Skin Ulcer/drug therapy/etiology MH - Treatment Outcome PMC - PMC12696660 COIS- Conflict of Interest Disclosures: Dr Netchiporouk reported personal fees from Galderma, AbbVie, Beiersdorft, Apogee, Arcutis, Bausch Health, BioJamp, Boehringer Ingelheim International, Bristol Myers Squibb, Celltrion, Eli Lilly, Galderma, Innovaderm, Janssen, LEO Pharma, Medexus, Novartis Pharmaceuticals, Organon, Pfizer, Sanofi Genzyme, Searchlight Pharma, Sun Pharmaceuticals, and UCB outside the submitted work. No other disclosures were reported. EDAT- 2025/12/10 13:51 MHDA- 2026/01/21 12:58 PMCR- 2026/12/10 CRDT- 2025/12/10 11:33 PHST- 2026/12/10 00:00 [pmc-release] PHST- 2026/01/21 12:58 [medline] PHST- 2025/12/10 13:51 [pubmed] PHST- 2025/12/10 11:33 [entrez] AID - 2842387 [pii] AID - doi250059 [pii] AID - 10.1001/jamadermatol.2025.4929 [doi] PST - ppublish SO - JAMA Dermatol. 2026 Jan 1;162(1):47-54. doi: 10.1001/jamadermatol.2025.4929. PMID- 20697523 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110713 LR - 20211020 IS - 1718-7729 (Electronic) IS - 1198-0052 (Print) IS - 1198-0052 (Linking) VI - 17 IP - 4 DP - 2010 Aug TI - Severe Raynaud syndrome induced by adjuvant interferon alfa in metastatic melanoma. PG - 122-3 AB - Melanoma is the most lethal form of skin malignancy because of its aggressive behaviour. In advanced disease, interferon alfa can be used as adjuvant therapy. However, this therapy is not free of side effects. We present a case of severe Raynaud syndrome and digital necrosis induced by interferon alfa in a patient with melanoma. Pathogenic mechanisms are discussed. FAU - Husein-Elahmed, H AU - Husein-Elahmed H AD - Department of Dermatology, San Cecilio University Hospital, Granada, Spain. huseinelahmed@hotmail.com FAU - Callejas-Rubio, J L AU - Callejas-Rubio JL FAU - Ortega Del Olmo, R AU - Ortega Del Olmo R FAU - Ríos-Fernandez, R AU - Ríos-Fernandez R FAU - Ortego-Centeno, N AU - Ortego-Centeno N LA - eng PT - Journal Article PL - Switzerland TA - Curr Oncol JT - Current oncology (Toronto, Ont.) JID - 9502503 PMC - PMC2913821 OTO - NOTNLM OT - Melanoma OT - Raynaud syndrome OT - adjuvant OT - digital necrosis OT - interferon OT - treatment OT - vascular toxicity EDAT- 2010/08/11 06:00 MHDA- 2010/08/11 06:01 PMCR- 2010/08/01 CRDT- 2010/08/11 06:00 PHST- 2010/08/11 06:00 [entrez] PHST- 2010/08/11 06:00 [pubmed] PHST- 2010/08/11 06:01 [medline] PHST- 2010/08/01 00:00 [pmc-release] AID - conc-17-4-122 [pii] AID - 10.3747/co.v17i4.519 [doi] PST - ppublish SO - Curr Oncol. 2010 Aug;17(4):122-3. doi: 10.3747/co.v17i4.519. PMID- 36660849 OWN - NLM STAT- MEDLINE DCOM- 20230605 LR - 20230605 IS - 1475-097X (Electronic) IS - 1475-0961 (Linking) VI - 43 IP - 4 DP - 2023 Jul TI - Responses to exercise in systemic sclerosis-associated interstitial lung disease. PG - 253-262 LID - 10.1111/cpf.12813 [doi] AB - INTRODUCTION: Pulmonary complications in systemic sclerosis (SSc) significantly increase morbidity and mortality. Our aim was to determine the factors limiting exercise capacity in SSc patients with and without interstitial lung disease (ILD), and to identify and quantify abnormalities during exercise that might assist in clinical assessment of this complication. METHODS: Fifteen patients with SSc and ILD (SSc-ILD) were compared with 10 patients with SSc without ILD and 9 age- and sex-matched normal volunteers. Subjects performed symptom-limited incremental treadmill exercise with online measurement of respiratory gas exchange, arterial blood gas sampling and measurement of neurohormones in venous blood. RESULTS: Patients with SSc-ILD had lower exercise capacity than SSc patients without ILD or normal subjects (peak oxygen consumption (PV̇O(2) ) (17.1 [4.2] vs. 22.0 [4.7] and 23.0 [5.4] ml kg(-1)  min(-1) , respectively, mean [SD], p < 0.01 ANOVA), but PV̇O(2) did not correlate with static pulmonary function measurements. Ventilatory equivalent for CO(2) (V̇E/V̇CO(2) ; nadir) was higher in SSc-ILD patients than the other two groups (36.6 [8.0] vs. 29.9 [4.4] and 30.0 [2.5], p < 0.005) as were peak exercise dead-space tidal volume ratio (0.44 [0.06] vs. 0.26 [0.09] and 0.26 [0.05], p < 0.001) and peak exercise alveolar-arterial difference (28.9 [16.9] vs. 18.8 [14.0] and 11.5 [6.9] mmHg, p < 0.05). Atrial natriuretic peptide was elevated in both SSc patient groups. CONCLUSIONS: SSc-ILD results in lower exercise capacity than SSc without ILD, and abnormalities of gas exchange are seen. The possible use of cardiopulmonary exercise testing to identify disease and quantify impairment in SSc-ILD merits further study. CI - © 2023 Scandinavian Society of Clinical Physiology and Nuclear Medicine. Published by John Wiley & Sons Ltd. FAU - Donnelly, Ronan P AU - Donnelly RP AD - Royal Victoria Hospital, Belfast, Northern Ireland. FAU - Smyth, Anita E AU - Smyth AE AD - Department of Rheumatology, Ulster Hospital, Dundonald, Northern Ireland. FAU - Mullan, Charles AU - Mullan C AD - City Hospital, Belfast, Northern Ireland. FAU - Riley, Marshall S AU - Riley MS AUID- ORCID: 0000-0001-8652-6617 AD - City Hospital, Belfast, Northern Ireland. FAU - Nicholls, D Paul AU - Nicholls DP AD - Royal Victoria Hospital, Belfast, Northern Ireland. LA - eng GR - Scleroderma and Raynaud's UK/ PT - Journal Article DEP - 20230214 PL - England TA - Clin Physiol Funct Imaging JT - Clinical physiology and functional imaging JID - 101137604 SB - IM MH - Humans MH - *Hypertension, Pulmonary MH - Lung MH - *Lung Diseases, Interstitial/etiology/complications MH - *Scleroderma, Systemic/complications/diagnosis MH - Exercise Test/methods OTO - NOTNLM OT - exercise test OT - neurohumoral responses OT - pulmonary fibrosis OT - pulmonary gas exchange OT - scleroderma EDAT- 2023/01/21 06:00 MHDA- 2023/06/05 06:42 CRDT- 2023/01/20 03:13 PHST- 2022/12/14 00:00 [revised] PHST- 2022/06/20 00:00 [received] PHST- 2023/01/16 00:00 [accepted] PHST- 2023/06/05 06:42 [medline] PHST- 2023/01/21 06:00 [pubmed] PHST- 2023/01/20 03:13 [entrez] AID - 10.1111/cpf.12813 [doi] PST - ppublish SO - Clin Physiol Funct Imaging. 2023 Jul;43(4):253-262. doi: 10.1111/cpf.12813. Epub 2023 Feb 14. PMID- 30915191 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1941-8744 (Print) IS - 1941-8752 (Electronic) IS - 1941-8744 (Linking) VI - 9 IP - 2 DP - 2019 Apr TI - DHE-Induced Peripheral Arterial Vasospasm in Primary Raynaud Phenomenon: Case Report. PG - 113-115 LID - 10.1177/1941874418797764 [doi] AB - Dihydroergotamine (DHE) is primarily a serotonin 5HT1B and 5HT1D receptor agonist used for acute migraine treatment. It is associated with acute vasoconstriction mediated through the 5HT1B receptor and is contraindicated in patients with history of cardiac disease and peripheral vascular disease. We present a case of acute peripheral arterial vasospasm in a patient with primary Raynaud phenomenon while receiving inpatient treatment for status migrainosus with intravenous (IV) DHE. The patient is a 35-year-old female with a history of chronic migraine and primary Raynaud phenomenon. After 15 doses of IV DHE, the patient reported paresthesias of the right hand and was noted to have absent right radial and ulnar pulses to palpation. Portable arterial Doppler study demonstrated abnormal flat line pulse volume recordings (PVRs) in the right second, third, and fourth digits, with markedly dampened PVR in the right thumb and fifth finger along with no ulnar PVR detectable at the wrist. Duplex revealed bilateral severely diminished flow in the right ulnar and radial arteries without acute occlusions. Computed tomography angiogram of right upper extremity visualized arteries through the mid-forearm but not distally. Dihydroergotamine was discontinued, and the patient was started on oral amlodipine and aspirin. Repeat Doppler ultrasound 3 days later revealed normal arm and digital waveforms bilaterally consistent with resolution of vasospasm. This case highlights a potential complication of IV DHE therapy. Risk may be increased in patients with primary Raynaud phenomenon. We suggest cautious use of IV DHE in this population. FAU - Khalife, Jane AU - Khalife J AD - Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Lauritsen, Clinton G AU - Lauritsen CG AD - Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Liang, John AU - Liang J AD - Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Shah, Syed O AU - Shah SO AD - Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20180911 PL - United States TA - Neurohospitalist JT - The Neurohospitalist JID - 101558199 PMC - PMC6429678 OTO - NOTNLM OT - Raynaud OT - dihydroergotamine OT - migraine OT - status migrainosus OT - vasospam COIS- Declaration of Conflicting Interests: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Clinton Lauritsen has received honoraria from Cefaly technologies. EDAT- 2019/03/28 06:00 MHDA- 2019/03/28 06:01 PMCR- 2020/04/01 CRDT- 2019/03/28 06:00 PHST- 2019/03/28 06:00 [entrez] PHST- 2019/03/28 06:00 [pubmed] PHST- 2019/03/28 06:01 [medline] PHST- 2020/04/01 00:00 [pmc-release] AID - 10.1177_1941874418797764 [pii] AID - 10.1177/1941874418797764 [doi] PST - ppublish SO - Neurohospitalist. 2019 Apr;9(2):113-115. doi: 10.1177/1941874418797764. Epub 2018 Sep 11. PMID- 37698987 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20260518 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 63 IP - 8 DP - 2024 Aug 1 TI - Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry. PG - 2093-2100 LID - 10.1093/rheumatology/kead481 [doi] AB - OBJECTIVES: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. METHODS: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). RESULTS: In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes-heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)-differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). CONCLUSION: DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Hum, Ryan Malcolm AU - Hum RM AUID- ORCID: 0000-0003-0801-6974 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal & Dermatological Sciences, The University of Manchester Faculty of Biology Medicine and Health, Manchester, UK. AD - The University of Manchester, National Institute for Health Research Manchester Biomedical Research Centre, Manchester, UK. FAU - Lilleker, James B AU - Lilleker JB AUID- ORCID: 0000-0002-9230-4137 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal & Dermatological Sciences, The University of Manchester Faculty of Biology Medicine and Health, Manchester, UK. AD - Northern Care Alliance NHS Foundation Trust, Manchester Centre for Clinical Neuroscience, Salford Royal Hospital, Salford, UK. FAU - Lamb, Janine A AU - Lamb JA AUID- ORCID: 0000-0002-7248-0539 AD - Division of Population Health, Health Services Research and Primary Care, The University of Manchester Faculty of Biology Medicine and Health, Epidemiology and Public Health Group, Manchester, UK. FAU - Oldroyd, Alexander G S AU - Oldroyd AGS AUID- ORCID: 0000-0001-5701-6490 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal & Dermatological Sciences, The University of Manchester Faculty of Biology Medicine and Health, Manchester, UK. AD - The University of Manchester, National Institute for Health Research Manchester Biomedical Research Centre, Manchester, UK. FAU - Wang, Guochun AU - Wang G AD - Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China. FAU - Wedderburn, Lucy R AU - Wedderburn LR AUID- ORCID: 0000-0003-2956-6676 AD - Great Ormond Street Hospital for Children NHS Foundation Trust, Infection, Immunity and Inflammation, London, UK. FAU - Diederichsen, Louise P AU - Diederichsen LP AD - Center for Rheumatology and Spine Diseases, Copenhagen University Hospital, Copenhagen, Denmark. FAU - Schmidt, Jens AU - Schmidt J AD - Department of Neurology, University Medical Center Göttingen, Göttingen, Germany. AD - Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel Klinik Rüdersdorf, University Hospital of the Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany. AD - Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany. FAU - Danieli, Maria Giovanna AU - Danieli MG AD - Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Universita Politecnica delle Marche, Ancona, Italy. FAU - Oakley, Paula AU - Oakley P AD - Myositis UK, Southampton, UK. FAU - Griger, Zoltan AU - Griger Z AUID- ORCID: 0000-0002-1371-7911 AD - Department of Immunology, University of Debrecen, Debrecen, Hajdú-Bihar, Hungary. FAU - Nguyen Thi Phuong, Thuy AU - Nguyen Thi Phuong T AUID- ORCID: 0000-0003-0702-3320 AD - Internal Medicine Department, Hanoi Medical University, Hanoi, Vietnam. FAU - Kodishala, Chanakya AU - Kodishala C AUID- ORCID: 0000-0003-3553-4304 AD - Clinical Immunology and Rheumatology, St John's National Academy of Health Sciences, Bangalore, Karnataka, India. AD - Department of Rheumatology, Mayo Clinic, Rochester, MN, USA. FAU - Vazquez-Del Mercado, Monica AU - Vazquez-Del Mercado M AUID- ORCID: 0000-0002-3823-4676 AD - Division de Medicina Interna, Servicio de Reumatologia, Hospital Civil Dr. Juan I. Menchaca, Universidad de Guadalajara, Guadalajara, Jalisco, Mexico. FAU - Andersson, Helena AU - Andersson H AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. FAU - De Paepe, Boel AU - De Paepe B AUID- ORCID: 0000-0001-9403-4401 AD - Department of Neurology, Universitair Ziekenhuis Gent, Ghent, Belgium. FAU - De Bleecker, Jan L AU - De Bleecker JL AD - Department of Neurology, Universitair Ziekenhuis Gent, Ghent, Belgium. FAU - Maurer, Britta AU - Maurer B AD - Department of Rheumatology and Immunology, Inselspital University Hospital Bern, Bern, Switzerland. FAU - McCann, Liza AU - McCann L AD - Department of Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK. FAU - Pipitone, Nicolo AU - Pipitone N AD - Department of Rheumatology, Arcispedale Santa Maria Nuova di Reggio Emilia, Reggio Emilia, Emilia-Romagna, Italy. FAU - McHugh, Neil AU - McHugh N AUID- ORCID: 0000-0003-2765-658X AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - New, Robert Paul AU - New RP AD - MRC/ARUK Institute of Ageing and Chronic Disease, University of Liverpool, Liverpool, UK. FAU - Ollier, William E AU - Ollier WE AD - Faculty of Science and Engineering, Manchester Metropolitan University, Manchester, UK. FAU - Krogh, Niels Steen AU - Krogh NS AD - Zitelab Aps, Copenhagen, Denmark. FAU - Vencovsky, Jiri AU - Vencovsky J AUID- ORCID: 0000-0002-0851-0713 AD - Institute of Rheumatology and Department of Rheumatology, Charles University, Praha, Czech Republic. FAU - Lundberg, Ingrid E AU - Lundberg IE AUID- ORCID: 0000-0002-6068-9212 AD - Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden. AD - Department of Gastroenterology, Dermatology, and Rheumatology, Karolinska University Hospital, Stockholm, Sweden. FAU - Chinoy, Hector AU - Chinoy H AUID- ORCID: 0000-0001-6492-1288 AD - Centre for Musculoskeletal Research, Division of Musculoskeletal & Dermatological Sciences, The University of Manchester Faculty of Biology Medicine and Health, Manchester, UK. AD - Northern Care Alliance NHS Foundation Trust, Department of Rheumatology, Salford Royal Hospital, Salford, UK. CN - MYONET Registry LA - eng GR - Association Francaise Contre Les Myopathies/ GR - LSH-018661/he European Union Sixth Framework Programme/ GR - European Science Foundation/ GR - Research Networking Programme European Myositis Network/ GR - K2014-52X-14045-14-3/Swedish Research Council/ GR - Stockholm County Council and Karolinska Institutet/ GR - MR/N003322/1/MRC_/Medical Research Council/United Kingdom GR - 18474/ARC_/Arthritis Research UK/United Kingdom GR - 00023728/Project for Conceptual Development of Research Organization/ GR - Ministry of Health in the Czech Republic/ GR - National Institute for Health Research/ GR - NIHR203308/Manchester Biomedical Research Centre/ GR - National Institute for Health Research/ GR - GOSH Biomedical Research Centre/ GR - Department of Health and Social Care/ PT - Comparative Study PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM EIN - Rheumatology (Oxford). 2024 Aug 1;63(8):2316. doi: 10.1093/rheumatology/keae186. PMID: 38531671 MH - Humans MH - *Dermatomyositis/immunology/complications MH - Male MH - Female MH - Middle Aged MH - *Registries MH - *Myositis/immunology/complications MH - *Autoantibodies/blood/immunology MH - Adult MH - Aged MH - Exanthema/etiology MH - Lung Diseases, Interstitial/immunology/etiology MH - Amino Acyl-tRNA Synthetases/immunology MH - Neoplasms/complications PMC - PMC11292049 OTO - NOTNLM OT - Anti-synthetase syndrome OT - Cutaneous OT - Dermatomyositis OT - Epidemiology OT - Extramuscular OT - MYONET OT - Malignancy OT - Rashes OT - Skin FIR - D'Hose, Sophie IR - D'Hose S FIR - Lu, Xin IR - Lu X FIR - Tian, Xiaolan IR - Tian X FIR - Mann, Heřman IR - Mann H FIR - Kryštůfková, Olga IR - Kryštůfková O FIR - Pleštilová, Lenka IR - Pleštilová L FIR - Klein, Martin IR - Klein M FIR - Barochová, Tereza IR - Barochová T FIR - Kubínová, Kateřina IR - Kubínová K FIR - Gelardi, Chiara IR - Gelardi C FIR - Paladini, Alberto IR - Paladini A FIR - Piga, Mario Andrea IR - Piga MA FIR - Jara, Luis J IR - Jara LJ FIR - Saavedra, Miguel A IR - Saavedra MA FIR - Cruz-Reyes, Claudia V IR - Cruz-Reyes CV FIR - Vera-Lastra, Olga IR - Vera-Lastra O FIR - Andrade-Ortega, Lilia IR - Andrade-Ortega L FIR - Medrano-Ramírez, Gabriel IR - Medrano-Ramírez G FIR - Satoh, Minoru IR - Satoh M FIR - Salazar-Páramo, Mario IR - Salazar-Páramo M FIR - Chavarría-Ávila, Efrain IR - Chavarría-Ávila E FIR - Aguilar-Vazquez, Andrea IR - Aguilar-Vazquez A FIR - Anda, Jesus-Aureliano Robles-de IR - Anda JR FIR - Petri, Marcelo H IR - Petri MH FIR - Molberg, Øyvind IR - Molberg Ø FIR - Dastmalchi, Maryam IR - Dastmalchi M FIR - Notarnicola, Antonella IR - Notarnicola A FIR - Gheorghe, Karina IR - Gheorghe K FIR - Rönnelid, Johan IR - Rönnelid J FIR - Liden, Maria IR - Liden M FIR - Hanna, Balsam IR - Hanna B FIR - Jalal, Awat IR - Jalal A FIR - Hellström, Helena IR - Hellström H FIR - Martineus, Jehns Christian IR - Martineus JC FIR - Lan, Nguyen Thi Ngoc IR - Lan NTN FIR - Padyukov, Leonid IR - Padyukov L FIR - New, Paul IR - New P FIR - Platt, Hazel IR - Platt H FIR - Rothwell, Simon IR - Rothwell S FIR - Ahmed, Yasmeen IR - Ahmed Y FIR - Armstrong, Raymond IR - Armstrong R FIR - Bernstein, Robert IR - Bernstein R FIR - Black, Carol IR - Black C FIR - Bowman, Simon IR - Bowman S FIR - Bruce, Ian IR - Bruce I FIR - Butler, Robin IR - Butler R FIR - Carty, John IR - Carty J FIR - Chattopadhyay, Chandra IR - Chattopadhyay C FIR - Chelliah, Easwaradhas IR - Chelliah E FIR - Clarke, Fiona IR - Clarke F FIR - Dawes, Peter IR - Dawes P FIR - Denton, Christopher IR - Denton C FIR - Devlin, Joseph IR - Devlin J FIR - Edwards, Christopher IR - Edwards C FIR - Emery, Paul IR - Emery P FIR - Fordham, John IR - Fordham J FIR - Fraser, Alexander IR - Fraser A FIR - Gaston, Hill IR - Gaston H FIR - Gordon, Patrick IR - Gordon P FIR - Griffiths, Bridget IR - Griffiths B FIR - Gunawardena, Harsha IR - Gunawardena H FIR - Hall, Frances IR - Hall F FIR - Hanna, Michael IR - Hanna M FIR - Harrison, Beverley IR - Harrison B FIR - Hay, Elaine IR - Hay E FIR - Hilton-Jones, David IR - Hilton-Jones D FIR - Horden, Lesley IR - Horden L FIR - Isaacs, John IR - Isaacs J FIR - Isenberg, David IR - Isenberg D FIR - Jones, Adrian IR - Jones A FIR - Kamath, Sanjeet IR - Kamath S FIR - Kennedy, Thomas IR - Kennedy T FIR - Kitas, George IR - Kitas G FIR - Klimiuk, Peter IR - Klimiuk P FIR - Knights, Sally IR - Knights S FIR - Lambert, John IR - Lambert J FIR - Lanyon, Peter IR - Lanyon P FIR - Laxminarayan, Ramasharan IR - Laxminarayan R FIR - Lecky, Bryan IR - Lecky B FIR - Luqmani, Raashid IR - Luqmani R FIR - Machado, Pedro IR - Machado P FIR - Marks, Jeffrey IR - Marks J FIR - Martin, Michael IR - Martin M FIR - McGonagle, Dennis IR - McGonagle D FIR - McHugh, Neil IR - McHugh N FIR - McKenna, Francis IR - McKenna F FIR - McLaren, John IR - McLaren J FIR - McMahon, Michael IR - McMahon M FIR - McRorie, Euan IR - McRorie E FIR - Merry, Peter IR - Merry P FIR - Miles, Sarah IR - Miles S FIR - Miller, James IR - Miller J FIR - Nicholls, Anne IR - Nicholls A FIR - Nixon, Jennifer IR - Nixon J FIR - Ong, Voon IR - Ong V FIR - Over, Katherine IR - Over K FIR - Packham, John IR - Packham J FIR - Pipitone, Nicolo IR - Pipitone N FIR - Plant, Michael IR - Plant M FIR - Pountain, Gillian IR - Pountain G FIR - Pullar, Thomas IR - Pullar T FIR - Roberts, Mark IR - Roberts M FIR - Sanders, Paul IR - Sanders P FIR - Scott, David IR - Scott D FIR - Scott, David IR - Scott D FIR - Shadforth, Michael IR - Shadforth M FIR - Sheeran, Thomas IR - Sheeran T FIR - Srinivasan, Arul IR - Srinivasan A FIR - Swinson, David IR - Swinson D FIR - Teh, Lee-Suan IR - Teh LS FIR - Webley, Michael IR - Webley M FIR - Williams, Brian IR - Williams B FIR - Winer, Jonathan IR - Winer J EDAT- 2023/09/12 18:41 MHDA- 2024/08/01 12:43 PMCR- 2023/09/12 CRDT- 2023/09/12 13:23 PHST- 2023/04/19 00:00 [received] PHST- 2023/08/16 00:00 [accepted] PHST- 2024/08/01 12:43 [medline] PHST- 2023/09/12 18:41 [pubmed] PHST- 2023/09/12 13:23 [entrez] PHST- 2023/09/12 00:00 [pmc-release] AID - 7271197 [pii] AID - kead481 [pii] AID - 10.1093/rheumatology/kead481 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Aug 1;63(8):2093-2100. doi: 10.1093/rheumatology/kead481. PMID- 22423244 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20211021 IS - 1662-0631 (Electronic) IS - 1662-0631 (Linking) VI - 6 IP - 1 DP - 2012 Jan TI - Calcium channel blockers and esophageal sclerosis: should we expect exacerbation of interstitial lung disease? PG - 82-7 LID - 10.1159/000336584 [doi] AB - Esophageal sclerosis is the most common visceral manifestation of systemic sclerosis, resulting in impaired esophageal clearance and retention of ingested food; in addition, co-existence of lung fibrosis with esophageal scleroderma is not uncommon. Both the progression of generalized connective tissue disorders and the damaging effect of chronic aspiration due to esophageal dysmotility appear to be involved in this procedure of interstitial fibrosis. Nifedipine is a widely prescribed calcium antagonist in a significant percentage of rheumatologic patients suffering from Raynaud syndrome, in order to inhibit peripheral vasospasm. Nevertheless, blocking calcium channels has proven to contribute to exacerbation of gastroesophageal reflux, which consequently can lead to chronic aspiration. We describe the case of severe exacerbation of interstitial lung disease in a 76-year-old female with esophageal sclerosis who was treated with oral nifedipine for Raynaud syndrome. FAU - Seretis, Charalampos AU - Seretis C AD - 2nd Department of Surgery, 401 General Army Hospital of Athens, Athens, Argos, Greece. FAU - Seretis, Fotios AU - Seretis F FAU - Gemenetzis, George AU - Gemenetzis G FAU - Liakos, Nikolaos AU - Liakos N FAU - Pappas, Apostolos AU - Pappas A FAU - Gourgiotis, Stavros AU - Gourgiotis S FAU - Lagoudianakis, Emmanuel AU - Lagoudianakis E FAU - Keramidaris, Dimitrios AU - Keramidaris D FAU - Salemis, Nikolaos AU - Salemis N LA - eng PT - Case Reports PT - Journal Article DEP - 20120131 PL - Switzerland TA - Case Rep Gastroenterol JT - Case reports in gastroenterology JID - 101474819 PMC - PMC3304075 OTO - NOTNLM OT - Aspiration OT - Emergency OT - Esophagus OT - Hypoxia OT - Nifedipine OT - Raynaud syndrome OT - Sclerosis EDAT- 2012/03/17 06:00 MHDA- 2012/03/17 06:01 PMCR- 2012/01/31 CRDT- 2012/03/17 06:00 PHST- 2012/03/17 06:00 [entrez] PHST- 2012/03/17 06:00 [pubmed] PHST- 2012/03/17 06:01 [medline] PHST- 2012/01/31 00:00 [pmc-release] AID - crg-0006-0082 [pii] AID - 10.1159/000336584 [doi] PST - ppublish SO - Case Rep Gastroenterol. 2012 Jan;6(1):82-7. doi: 10.1159/000336584. Epub 2012 Jan 31. PMID- 34674982 OWN - NLM STAT- MEDLINE DCOM- 20220407 LR - 20220531 IS - 2152-2669 (Electronic) IS - 2152-2669 (Linking) VI - 22 IP - 3 DP - 2022 Mar TI - Prognostic of Core Binding Factor (CBF) Acute Myeloid Leukemia With Complex Karyotype. PG - e199-e205 LID - S2152-2650(21)02038-3 [pii] LID - 10.1016/j.clml.2021.09.007 [doi] AB - BACKGROUND: Core-binding factor acute myeloid leukemia (CBF AML) with recurrent genetic abnormalities inv(16)(p13.1q22) or t(16;16)(p13.1;q22)/ CBFB-MYH11 are usually prognostically favorable but heterogeneous group and additional abnormalities change their prognosis. MATERIALS AND METHODS: To evaluate the impact of a complex karyotype on CBF AML prognosis, we included 24 patients with a median age of 56.4 years (23.2-83.3) and a median number of abnormalities of 5 (4-13). RESULTS: Median follow-up was 110.4 months. Among patients with a primary clone, complete remission (CR) was reached in 66.7% of patients. 31.3% of patients experienced AML relapse with a median of 8.5 months. Median OS for transplanted patients was 80.7 versus 40.5 months for non-transplanted patients, excluding the 4 patients with early death. Among patients harboring AML with clonal evolution, CR was reached in 62.5% of patients. 50% of patients underwent allogeneic stem cell transplantation (ASCT). In these, median RFS was 19.3 versus 0 months in non-transplanted patients. Median OS seemed also longer in transplanted patients with 23.5 versus 2.95 months in non-transplanted. CONCLUSION: Use of new treatment and tailored strategy based on measurable residual disease monitoring may now improve these results. However, these data allow us to reconsider the good prognosis historically associated with CBF patients despite of karyotype and the place of ASCT in the strategy. CI - Copyright © 2021. Published by Elsevier Inc. FAU - Marcault, Clemence AU - Marcault C AD - CHU of Nice, Hematology department, Cote D'Azur University, Nice Sophia Antipolis University, Nice, France. FAU - Boissel, Nicolas AU - Boissel N AD - Saint-Louis Hospital, Hematology Department, Institut de Recherche Saint-Louis, Paris University, Paris, France. FAU - Haferlach, Claudia AU - Haferlach C AD - MLL Munich Leukemia Laboratory, Munich, Germany. FAU - Loschi, Michael AU - Loschi M AD - CHU of Nice, Hematology department, Cote D'Azur University, Nice Sophia Antipolis University, Nice, France; INSERM U1065, Mediterranean Center of Molecular Medecine, Cote D'Azur University, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - CHU of Nice, Onco-hematology Laboratory, Cote D'Azur University, Nice Sophia Antipolis University, Nice, France. FAU - Cluzeau, Thomas AU - Cluzeau T AD - CHU of Nice, Hematology department, Cote D'Azur University, Nice Sophia Antipolis University, Nice, France; INSERM U1065, Mediterranean Center of Molecular Medecine, Cote D'Azur University, Nice, France. Electronic address: cluzeau.t@chu-nice.fr. LA - eng PT - Journal Article DEP - 20210911 PL - United States TA - Clin Lymphoma Myeloma Leuk JT - Clinical lymphoma, myeloma & leukemia JID - 101525386 RN - 0 (Core Binding Factors) SB - IM MH - Abnormal Karyotype MH - *Core Binding Factors/genetics MH - Humans MH - Karyotyping MH - *Leukemia, Myeloid, Acute/diagnosis/genetics/therapy MH - Middle Aged MH - Prognosis OTO - NOTNLM OT - AML OT - Allograft OT - CBF OT - Karyotype OT - Prognosis EDAT- 2021/10/23 06:00 MHDA- 2022/04/08 06:00 CRDT- 2021/10/22 05:45 PHST- 2021/08/11 00:00 [received] PHST- 2021/09/03 00:00 [revised] PHST- 2021/09/08 00:00 [accepted] PHST- 2021/10/23 06:00 [pubmed] PHST- 2022/04/08 06:00 [medline] PHST- 2021/10/22 05:45 [entrez] AID - S2152-2650(21)02038-3 [pii] AID - 10.1016/j.clml.2021.09.007 [doi] PST - ppublish SO - Clin Lymphoma Myeloma Leuk. 2022 Mar;22(3):e199-e205. doi: 10.1016/j.clml.2021.09.007. Epub 2021 Sep 11. PMID- 26252834 OWN - NLM STAT- MEDLINE DCOM- 20160509 LR - 20150815 IS - 1098-2264 (Electronic) IS - 1045-2257 (Linking) VI - 54 IP - 10 DP - 2015 Oct TI - Molecular characterization and follow-up of five CML patients with new BCR-ABL1 fusion transcripts. PG - 595-605 LID - 10.1002/gcc.22263 [doi] AB - We report five chronic myeloid leukaemia (CML) patients in whom we identified and characterized undescribed BCR-ABL1 fusion transcripts. We investigated the precise features of the molecular rearrangements and the minimal residual disease follow-up for these five patients. Three resulted from new rearrangements between the BCR and ABL1 sequences (the breakpoints being located within BCR exon 13 in two cases and within BCR exon 18 in one case). The other two cases revealed a complex e8-[ins]-a2 fusion transcript involving a third partner gene, PRDM12 and SPECC1L, respectively. Moreover, single nucleotide polymorphism-array analysis performed in the latter two cases showed copy number alterations shared by the two patients, thus identifying genes that were deleted during rearrangement and suggesting their potential role in CML pathogenesis. Interestingly, we highlight that the prognosis of alterations, such as the presence of an e8a2 transcript or the deletion of various genes, which have been controversial, may be definitively erased by the introduction of tyrosine kinase inhibitors (TKIs). CI - © 2015 Wiley Periodicals, Inc. FAU - Huet, Sarah AU - Huet S AD - Laboratoire D'hématologie, Centre De Biologie Sud, Hospices Civils De Lyon, Pierre-Bénite, France. FAU - Dulucq, Stéphanie AU - Dulucq S AD - Laboratoire D'hématologie, Centre Hospitalo-Universitaire De Bordeaux, Bordeaux, France. FAU - Chauveau, Aurélie AU - Chauveau A AD - Laboratoire D'hématologie, Centre Hospitalo-Universitaire Régional De Brest, Brest, France. FAU - Ménard, Audrey AU - Ménard A AD - Laboratoire D'hématologie, Institut De Biologie, Centre Hospitalo-Universitaire De Nantes, Nantes, France. FAU - Chomel, Jean-Claude AU - Chomel JC AD - Service De Cancérologie Biologique, Centre Hospitalo-Universitaire De Poitiers, Poitiers, France. FAU - Maisonneuve, Hervé AU - Maisonneuve H AD - Service Clinique d'onco-Hématologie, Centre Hospitalier Départemental Vendée, La-Roche-sur-Yon, France. FAU - Legros, Laurence AU - Legros L AD - Service D'hématologie Clinique, Groupe Hospitalier L'archet, Centre Hospitalo-Universitaire De Nice, Nice, France. FAU - Perrin, Marie-Claire AU - Perrin MC AD - Service D'hématologie Clinique, Hôpital Fleyriat, Bourg-en-Bresse, France. FAU - Ferrant, Emmanuelle AU - Ferrant E AD - Service D'hématologie Clinique, Centre Hospitalo-Universitaire De Dijon, Dijon, France. FAU - Moreilhon, Chimène AU - Moreilhon C AD - Laboratoire D'hématologie, Hôpital Pasteur, Centre Hospitalo-Universitaire De Nice, Nice, France. FAU - Couturier, Marie-Anne AU - Couturier MA AD - Service D'hématologie Stérile, Institut Cancérologie-Hématologie, Centre Hospitalo-Universitaire Régional De Brest, Brest, France. FAU - Sujobert, Pierre AU - Sujobert P AD - Laboratoire D'hématologie, Centre De Biologie Sud, Hospices Civils De Lyon, Pierre-Bénite, France. FAU - Magaud, Jean-Pierre AU - Magaud JP AD - Laboratoire D'hématologie, Centre De Biologie Sud, Hospices Civils De Lyon, Pierre-Bénite, France. FAU - Ugo, Valérie AU - Ugo V AD - Laboratoire D'hématologie, Centre Hospitalo-Universitaire Régional De Brest, Brest, France. FAU - Chabane, Kaddour AU - Chabane K AD - Laboratoire D'hématologie, Centre De Biologie Sud, Hospices Civils De Lyon, Pierre-Bénite, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Laboratoire D'hématologie, Hôpital Pasteur, Centre Hospitalo-Universitaire De Nice, Nice, France. FAU - Hayette, Sandrine AU - Hayette S AD - Laboratoire D'hématologie, Centre De Biologie Sud, Hospices Civils De Lyon, Pierre-Bénite, France. CN - GBMHM (Groupe des Biologistes Moléculaires des Hémopathies Malignes, French Molecular Biology Group in Hematology) LA - eng PT - Journal Article DEP - 20150807 PL - United States TA - Genes Chromosomes Cancer JT - Genes, chromosomes & cancer JID - 9007329 RN - EC 2.7.10.2 (Fusion Proteins, bcr-abl) SB - IM MH - Adult MH - Aged MH - Cohort Studies MH - Female MH - Fusion Proteins, bcr-abl/*genetics MH - Humans MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*genetics MH - Male MH - Middle Aged EDAT- 2015/08/08 06:00 MHDA- 2016/05/10 06:00 CRDT- 2015/08/08 06:00 PHST- 2014/12/23 00:00 [received] PHST- 2015/04/13 00:00 [revised] PHST- 2015/04/13 00:00 [accepted] PHST- 2015/08/08 06:00 [entrez] PHST- 2015/08/08 06:00 [pubmed] PHST- 2016/05/10 06:00 [medline] AID - 10.1002/gcc.22263 [doi] PST - ppublish SO - Genes Chromosomes Cancer. 2015 Oct;54(10):595-605. doi: 10.1002/gcc.22263. Epub 2015 Aug 7. PMID- 39418195 OWN - NLM STAT- MEDLINE DCOM- 20251210 LR - 20251211 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - SI DP - 2025 Dec 1 TI - Deep-learning CT imaging algorithm to detect usual interstitial pneumonia pattern in patients with systemic sclerosis-associated interstitial lung disease: association with disease progression and survival. PG - SI21-SI27 LID - 10.1093/rheumatology/keae571 [doi] AB - OBJECTIVES: Interstitial lung disease (ILD) is the most common cause of death in patients with systemic sclerosis (SSc), although disease behaviour is highly heterogeneous. While a usual interstitial pneumonia (UIP) pattern is associated with worse survival in other ILDs, its significance in SSc-ILD is unclear. We sought to assess the prognostic utility of a deep-learning high resolution CT (HRCT) algorithm of UIP probability in SSc-ILD. METHODS: Patients with SSc-ILD were included if HRCT images, concomitant lung function tests and follow-up data were available. We used the Systematic Objective Fibrotic Imaging analysis Algorithm (SOFIA), a convolution neural network algorithm that provides probabilities of a UIP pattern on HRCT images. These were converted into the Prospective Investigation of Pulmonary Embolism Diagnosis (PIOPED)-based UIP probability categories. Decline in lung function was assessed by mixed-effect model analysis and relationship with survival by Cox proportional hazards analysis. RESULTS: Five hundred and twenty-two patients were included in the study; 19.5% were classified as UIP not in the differential, 53.5% as low probability of UIP, 25.7% as intermediate probability of UIP, and 1.3% as high probability of UIP. A higher likelihood of UIP probability expressed as PIOPED categories was associated with worse baseline forced vital capacity (FVC), as well as with decline in FVC (P = 0.008), and worse 15-year survival (P = 0.001), both independently of age, gender, ethnicity, smoking history and baseline FVC or Goh et al. staging system. CONCLUSION: A higher probability of a SOFIA-determined UIP pattern is associated with more advanced ILD, disease progression and worse survival, suggesting that it may be a useful prognostic marker in SSc-ILD. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Stock, Carmel J W AU - Stock CJW AUID- ORCID: 0000-0003-4090-8761 AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Nan, Yang AU - Nan Y AD - National Heart and Lung Institute, Imperial College London, London, UK. FAU - Fang, Yingying AU - Fang Y AD - National Heart and Lung Institute, Imperial College London, London, UK. FAU - Kokosi, Maria AU - Kokosi M AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Kouranos, Vasilios AU - Kouranos V AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - George, Peter M AU - George PM AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Chua, Felix AU - Chua F AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Jenkins, Gisli R AU - Jenkins GR AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Devaraj, Anand AU - Devaraj A AD - National Heart and Lung Institute, Imperial College London, London, UK. AD - Department of Radiology, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Desai, Sujal R AU - Desai SR AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. AD - Department of Radiology, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology, Division of Medicine, UCL, London, UK. FAU - Wells, Athol U AU - Wells AU AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Walsh, Simon L F AU - Walsh SLF AD - National Heart and Lung Institute, Imperial College London, London, UK. AD - Department of Radiology, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Hospitals, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. LA - eng GR - RBH1/Scleroderma and Raynaud's UK/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - Female MH - Male MH - *Scleroderma, Systemic/complications/mortality MH - Middle Aged MH - *Tomography, X-Ray Computed/methods MH - Disease Progression MH - *Deep Learning MH - *Lung Diseases, Interstitial/diagnostic imaging/mortality/etiology MH - Algorithms MH - Aged MH - Prognosis MH - Idiopathic Pulmonary Fibrosis/diagnostic imaging MH - Respiratory Function Tests MH - Prospective Studies MH - Adult OTO - NOTNLM OT - HRCT OT - ILD OT - SSc OT - UIP OT - prognosis EDAT- 2024/10/18 07:19 MHDA- 2025/12/11 01:23 CRDT- 2024/10/17 13:03 PHST- 2024/05/31 00:00 [received] PHST- 2024/10/05 00:00 [accepted] PHST- 2025/12/11 01:23 [medline] PHST- 2024/10/18 07:19 [pubmed] PHST- 2024/10/17 13:03 [entrez] AID - 7825351 [pii] AID - 10.1093/rheumatology/keae571 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Dec 1;64(SI):SI21-SI27. doi: 10.1093/rheumatology/keae571. PMID- 42247287 OWN - NLM STAT- Publisher LR - 20260605 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) DP - 2026 Jun 5 TI - Measuring disease activity in juvenile systemic sclerosis: a multidisciplinary consensus from the Hamburg 2024 symposium. LID - 10.1080/1744666X.2026.2685050 [doi] AB - INTRODUCTION: Assessment of disease activity in juvenile systemic sclerosis (jSSc) is essential for clinical care and trial readiness, yet no validated pediatric activity measures exist. Adult systemic sclerosis activity tools, including the Scleroderma Clinical Trials Consortium Activity Index, revised CRISS, and revised EUSTAR, provide conceptual frameworks but require adaptation for developmental, physiologic, and feasibility considerations for children. At the 17th Hamburg Symposium on JSSc, an international multidisciplinary panel reviewed adult indices, evaluated corresponding variables in the jSSc Inception Cohort and the NRCOS registry, and conducted a structured Delphi process to define organ-specific indicators of clinically meaningful activity in jSSc. AREAS COVERED: Across skin, pulmonary, cardiac, vascular, musculoskeletal, gastrointestinal, renal, and global domains, the panel reached broad and often unanimous consensus on variables reflecting active, potentially reversible disease. Key endorsed measures included mRSS progression, new ILD on HRCT, ≥10% declines in FVC or DLCO, new cardiac abnormalities, active digital ulcers, synovitis, myositis, nutritional decline, and physician global assessment. Patient-reported outcomes were strongly supported across domains. EXPERT OPINION: These consensus-derived indicators provide the first comprehensive pediatric-specific foundation for defining disease activity in jSSc and represent a critical step toward developing and validating a unified pediatric activity index suitable for future clinical trials. FAU - Foeldvari, Ivan AU - Foeldvari I AUID- ORCID: 0000-0003-0659-5298 AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schon Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Torok, Kathryn S AU - Torok KS AD - Division of Pediatric Rheumatology, UPMC & University of Pittsburgh Scleroderma Center, University of Pittsburgh School of Medicine, Pittsburgh, PA , USA. FAU - Klotsche, Jens AU - Klotsche J AD - German Rheumatism Research Center, Berlin, Germany. FAU - Li, Jonathan C AU - Li JC AD - Division of Pediatric Rheumatology, UPMC & University of Pittsburgh Scleroderma Center, University of Pittsburgh School of Medicine, Pittsburgh, PA , USA. FAU - Bruni, Cosimo AU - Bruni C AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich. FAU - Costa-Reis, Patricia AU - Costa-Reis P AD - Pediatrics Department, Hospital de Santa Maria, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology-Department of Internal Medicine and Specialties, University of Genoa and IRCCS San Martino Polyclinic Hospital, Genoa, Italy. FAU - Denton, Christopher P AU - Denton CP AD - UCL Division of Medicine, Royal Free London NHS Foundation Trust, London, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma & Raynaud's United Kindgom (SRUK) (Research Subcommittee, Patient Research Partner, London, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, and NIHR Biomedical Research Centre, Leeds Teaching Hospital Trust, Leeds, UK. FAU - Gimenez-Roca, Clara AU - Gimenez-Roca C AD - Department of Pediatric Rheumatology. Hospital Sant Joan de Déu, Esplugues de Llobregat (Barcelona), Spain. FAU - Henes, Jörg AU - Henes J AD - Centre for Interdisciplinary Clinical Immunology, Rheumatology and Autoinflammatory Diseases and Department for Internal Medicine II, University Hospital Tübingen, Germany. FAU - Kasapcopur, Ozgur AU - Kasapcopur O AD - Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University - Cerrahpasa, Istanbul, Turkey. FAU - Li, Suzanne C AU - Li SC AD - Joseph M Sanzari Children's Hospital, Hackensack Meridian School of Medicine, Hackensack, NJ , USA. FAU - Marrani, Edoardo AU - Marrani E AD - Pediatric Rheumatology, Meyer Children's Hospital IRCCS, Firenze, Italy. FAU - Orteu, Catherine H AU - Orteu CH AD - UCL Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK. FAU - Pauling, John D AU - Pauling JD AD - North Bristol NHS Trust, Bristol, UK. FAU - Pilkington, Clarissa AU - Pilkington C AD - Great Ormond Street Hospital London, UK. FAU - Ross, Laura AU - Ross L AD - Department of Medicine, Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, Australia& Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia. AD - University of Washington, Seattle. FAU - Schonenberg-Meinema, Dieneke AU - Schonenberg-Meinema D AD - Department of Internal Medicine, Ghent University, Ghent, BelgiumDepartment of Rheumatology, Ghent University Hospital, Ghent, Belgium, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium and ERN ReCONNET. FAU - Furst, Daniel E AU - Furst DE AD - Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia. AD - Department of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands. AD - University of California, Los Angeles. AD - , and University of Florence, Florence, Italy. FAU - Smith, Vanessa AU - Smith V AD - Department of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20260605 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 SB - IM OTO - NOTNLM OT - Clinical trials OT - consensus OT - disease activity OT - disease activity index OT - juvenile systemic sclerosis OT - outcome measures OT - pediatric rheumatology EDAT- 2026/06/05 18:36 MHDA- 2026/06/05 18:36 CRDT- 2026/06/05 12:33 PHST- 2026/06/05 18:36 [medline] PHST- 2026/06/05 18:36 [pubmed] PHST- 2026/06/05 12:33 [entrez] AID - 10.1080/1744666X.2026.2685050 [doi] PST - aheadofprint SO - Expert Rev Clin Immunol. 2026 Jun 5. doi: 10.1080/1744666X.2026.2685050. PMID- 21494260 OWN - NLM STAT- MEDLINE DCOM- 20110916 LR - 20220311 IS - 1476-5551 (Electronic) IS - 0887-6924 (Linking) VI - 25 IP - 7 DP - 2011 Jul TI - Impact of TET2 mutations on response rate to azacitidine in myelodysplastic syndromes and low blast count acute myeloid leukemias. PG - 1147-52 LID - 10.1038/leu.2011.71 [doi] AB - The impact of ten-eleven-translocation 2 (TET2) mutations on response to azacitidine (AZA) in MDS has not been reported. We sequenced the TET2 gene in 86 MDS and acute myeloid leukemia (AML) with 20-30% blasts treated by AZA, that is disease categories wherein this drug is approved by Food and Drug Administration (FDA). Thirteen patients (15%) carried TET2 mutations. Patients with mutated and wild-type (WT) TET2 had mostly comparable pretreatment characteristics, except for lower hemoglobin, better cytogenetic risk and longer MDS duration before AZA in TET2 mutated patients (P=0.03, P=0.047 and P=0.048, respectively). The response rate (including hematological improvement) was 82% in MUT versus 45% in WT patients (P=0.007). Mutated TET2 (P=0.04) and favorable cytogenetic risk (intermediate risk: P=0.04, poor risk: P=0.048 compared with good risk) independently predicted a higher response rate. Response duration and overall survival were, however, comparable in the MUT and WT groups. In higher risk MDS and AML with low blast count, TET2 status may be a genetic predictor of response to AZA, independently of karyotype. FAU - Itzykson, R AU - Itzykson R AD - Service d'Hématologie Clinique Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris 13, Bobigny, France. FAU - Kosmider, O AU - Kosmider O FAU - Cluzeau, T AU - Cluzeau T FAU - Mansat-De Mas, V AU - Mansat-De Mas V FAU - Dreyfus, F AU - Dreyfus F FAU - Beyne-Rauzy, O AU - Beyne-Rauzy O FAU - Quesnel, B AU - Quesnel B FAU - Vey, N AU - Vey N FAU - Gelsi-Boyer, V AU - Gelsi-Boyer V FAU - Raynaud, S AU - Raynaud S FAU - Preudhomme, C AU - Preudhomme C FAU - Adès, L AU - Adès L FAU - Fenaux, P AU - Fenaux P FAU - Fontenay, M AU - Fontenay M CN - Groupe Francophone des Myelodysplasies (GFM) LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110415 PL - England TA - Leukemia JT - Leukemia JID - 8704895 RN - 0 (Antimetabolites, Antineoplastic) RN - 0 (DNA, Neoplasm) RN - 0 (DNA-Binding Proteins) RN - 0 (Hemoglobins) RN - 0 (Neoplasm Proteins) RN - 0 (Proto-Oncogene Proteins) RN - EC 1.13.11.- (Dioxygenases) RN - EC 1.13.11.- (TET2 protein, human) RN - M801H13NRU (Azacitidine) SB - IM MH - Aged MH - Aged, 80 and over MH - Antimetabolites, Antineoplastic/*therapeutic use MH - Azacitidine/*therapeutic use MH - DNA Methylation/drug effects MH - DNA, Neoplasm/genetics MH - DNA-Binding Proteins/*genetics/physiology MH - Dioxygenases MH - Disease-Free Survival MH - Female MH - Hemoglobins/analysis MH - Humans MH - Kaplan-Meier Estimate MH - Karyotyping MH - Leukemia, Myeloid, Acute/blood/drug therapy/*genetics/pathology MH - Leukocyte Count MH - Male MH - Middle Aged MH - *Mutation MH - Myelodysplastic Syndromes/blood/drug therapy/*genetics/pathology MH - Neoplasm Proteins/*genetics/physiology MH - Proto-Oncogene Proteins/*genetics/physiology MH - Sequence Analysis, DNA MH - Treatment Outcome EDAT- 2011/04/16 06:00 MHDA- 2011/09/17 06:00 CRDT- 2011/04/16 06:00 PHST- 2011/04/16 06:00 [entrez] PHST- 2011/04/16 06:00 [pubmed] PHST- 2011/09/17 06:00 [medline] AID - leu201171 [pii] AID - 10.1038/leu.2011.71 [doi] PST - ppublish SO - Leukemia. 2011 Jul;25(7):1147-52. doi: 10.1038/leu.2011.71. Epub 2011 Apr 15. PMID- 39673658 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250130 IS - 2193-8253 (Print) IS - 2193-6536 (Electronic) IS - 2193-6536 (Linking) VI - 14 IP - 1 DP - 2025 Feb TI - Long-Term Safety and Effectiveness of Dimethyl Fumarate in Patients with Multiple Sclerosis Treated in Routine Medical Practice: Final Analysis of the ESTEEM Study. PG - 243-260 LID - 10.1007/s40120-024-00680-z [doi] AB - INTRODUCTION: Dimethyl fumarate (DMF) has demonstrated a favorable benefit-risk profile in patients with relapsing-remitting multiple sclerosis (RRMS) in clinical and real-world studies. The ESTEEM study (NCT02047097) was conducted to assess the long-term safety and effectiveness of delayed-release DMF in patients with relapsing forms of MS in routine clinical practice. We report final outcomes from ESTEEM with up to 6.5 years of follow-up. METHODS: Patients newly prescribed DMF were recruited from 393 sites globally. The primary objective was to assess the incidence and type of serious adverse events (SAEs) and adverse events (AEs) leading to discontinuation of DMF. Secondary objectives included assessment of DMF effectiveness on annualized relapse rate (ARR) and patient-reported outcomes (PROs). RESULTS: Overall, 5124 patients received ≥ 1 dose of DMF. The mean (standard deviation [SD]) age at enrollment was 40.0 (11.2) years; 74% of patients were female. Patients received DMF for a mean (SD) duration of 31.0 (22.7) months. Primary reasons for discontinuation were AEs (n = 1237; 24%); the most common were gastrointestinal AEs (n = 469; 9%), blood and lymphatic disorders (n = 218; 4%), and vascular disorders (n = 200; 4%). SAEs occurred in 391 (8%) patients, most commonly infections and infestations (n = 102; 2%). Adjusted ARR declined by 90% (95% confidence interval [CI]: 90-91%; p < 0.0001), from 0.81 (95% CI 0.79-0.84) 12 months before enrollment to 0.08 (95% CI 0.08-0.09) 6 years after enrollment. The estimated proportion of patients free from confirmed disability progression sustained over 48 weeks was 87.0% at month 60. Mean scores for physical and psychological impact, fatigue, health, and productivity remained stable over 5 years. CONCLUSION: DMF demonstrated a safety profile in real-world clinical practice consistent with the known profile of DMF. Relapse rates were low and both ARR and PROs remained stable over time. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT02047097. CI - © 2024. The Author(s). FAU - Pandey, Krupa Shah AU - Pandey KS AD - Hackensack University Medical Center, Hackensack Meridian School of Medicine, Hackensack, NJ, USA. FAU - Giles, Kathryn AU - Giles K AD - Cambridge Memorial Hospital, Cambridge, ON, Canada. FAU - Balashov, Konstantin AU - Balashov K AD - Chobanian & Avedisian School of Medicine and Boston Medical Center, Boston University, Boston, MA, USA. FAU - Macdonell, Richard AU - Macdonell R AUID- ORCID: 0000-0001-6604-3968 AD - Department of Neurology, Austin Health, Univ. of Melbourne, Melbourne, Australia. FAU - Windsheimer, Jörg AU - Windsheimer J AD - Praxis für Neurologie und Psychiatrie, Nuremberg, Germany. FAU - Martinez, Mikel AU - Martinez M AD - Centre Hospitalier Dax, Dax, France. FAU - Božin, Ivan AU - Božin I AUID- ORCID: 0009-0008-2205-4233 AD - Biogen, Baar, Switzerland. Ivan.Bozin@Biogen.com. FAU - Raynaud, Stephanie AU - Raynaud S AD - Formerly: Biogen, Cambridge, MA, USA. FAU - Scaramozza, Matthew AU - Scaramozza M AUID- ORCID: 0000-0003-3738-459X AD - Biogen, Cambridge, MA, USA. FAU - Mokliatchouk, Oksana AU - Mokliatchouk O AD - Biogen, Cambridge, MA, USA. FAU - Sun, Zhaonan AU - Sun Z AUID- ORCID: 0000-0001-5333-4387 AD - Biogen, Cambridge, MA, USA. FAU - Belviso, Nicholas AU - Belviso N AD - Biogen, Cambridge, MA, USA. FAU - Sato, Yayoi AU - Sato Y AUID- ORCID: 0009-0005-8747-2450 AD - Biogen, Tokyo, Japan. FAU - Lin, Xiaochen AU - Lin X AD - Biogen, Cambridge, MA, USA. FAU - Okai, Annette AU - Okai A AD - North Texas Institute of Neurology and Headache, Plano, TX, USA. LA - eng SI - ClinicalTrials.gov/NCT02047097 PT - Journal Article DEP - 20241214 PL - New Zealand TA - Neurol Ther JT - Neurology and therapy JID - 101637818 PMC - PMC11762038 OTO - NOTNLM OT - Dimethyl fumarate OT - Disease-modifying treatment OT - Effectiveness OT - Multiple sclerosis OT - Real world COIS- Declarations. Conflict of Interest: Krupa Shah Pandey: consulting fees from Bristol Myers Squibb; fees for non-CME services and/or speaker bureaus from Alexion, Biogen, Bristol Myers Squibb, Genentech, and Sanofi; research support from Genentech. Kathryn Giles: consulting fees from Biogen, EMD Serono, and Genzyme. Konstantin Balashov: consulting fees from Genentech; research support from Biogen. Richard Macdonell: consulting fees and research support from Biogen, Celgene, Genentech, Genzyme, and Teva; speaker bureau for Biogen, Celgene, Genentech, Genzyme, and Teva. Jörg Windsheimer: consulting fees from Genzyme, Merck, Novartis, Roche, and Teva. Mikel Martinez: consulting fees from Genzyme, Novartis, and Pfizer. Ivan Božin, Matthew Scaramozza, Oksana Mokliatchouk, Zhaonan Sun, Nicholas Belviso, Yayoi Sato, Xiaochen Lin: employees of Biogen and may hold stock in the company. Stephanie Raynaud: employee of and held stock/stock options in Biogen at the time this work was conducted. Annette Okai: consulting fees from Biogen, Bristol Myers Squibb, EMD Serono, Novartis, Roche-Genentech, and Sanofi-Genzyme; research support from Biogen, Bristol Myers Squibb, Roche-Genentech, and Sanofi Genzyme; speaker bureau for Biogen, Bristol Myers Squibb, EMD Serono, Roche-Genentech, and Sanofi-Genzyme.​ Ethical Approval: The study was conducted in accordance with protocol, the International Council for Harmonization Guideline E6, all applicable local regulatory requirements, and ethical principles based on the Declaration of Helsinki. EDAT- 2024/12/14 20:03 MHDA- 2024/12/14 20:04 PMCR- 2024/12/14 CRDT- 2024/12/14 11:18 PHST- 2024/08/12 00:00 [received] PHST- 2024/10/22 00:00 [accepted] PHST- 2024/12/14 20:04 [medline] PHST- 2024/12/14 20:03 [pubmed] PHST- 2024/12/14 11:18 [entrez] PHST- 2024/12/14 00:00 [pmc-release] AID - 10.1007/s40120-024-00680-z [pii] AID - 680 [pii] AID - 10.1007/s40120-024-00680-z [doi] PST - ppublish SO - Neurol Ther. 2025 Feb;14(1):243-260. doi: 10.1007/s40120-024-00680-z. Epub 2024 Dec 14. PMID- 33328257 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 10 IP - 12 DP - 2020 Dec 16 TI - Collaboration between patient organisations and a clinical research sponsor in a rare disease condition: learnings from a community advisory board and best practice for future collaborations. PG - e039473 LID - 10.1136/bmjopen-2020-039473 [doi] LID - e039473 AB - Introduction Transparent collaborations between patient organisations (POs) and clinical research sponsors (CRS) can identify and address the unmet needs of patients and caregivers. These insights can improve clinical trial participant experience and delivery of medical innovations necessary to advance health outcomes and standards of care. We share our experiences from such a collaboration undertaken surrounding the SENSCIS(®) clinical trial (NCT02597933), and discuss its impact during, and legacy beyond, the trial.Summary We describe the establishment of a community advisory board (CAB): a transparent, multiyear collaboration between the scleroderma patient community and a CRS. We present shared learnings from the collaboration, which is split into three main areas: (1) the implementation and conduct of the clinical trial; (2) analysis and dissemination of the results; and (3) aspects of the collaboration not related to the trial.1. The scleroderma CAB reviewed and provided advice on trial conduct and reporting. This led to the improvement and optimisation of trial procedures; meaningful, patient-focused adaptations were made to address challenges relevant to scleroderma-associated interstitial lung disease patients.2. To ensure that results of the trial were accessible to lay audiences and patients, written lay summaries were developed by the trial sponsor with valuable input from the CAB to ensure that language and figures were understandable.3. The CAB and the CRS also collaborated to co-develop opening tools for medication blister packs and bottles. In addition, to raise disease awareness among physicians, patients and caregivers, educational materials to improve diagnosis and management of scleroderma were co-created and delivered by the CAB and CRS.Conclusions This collaboration between POs and a CRS, in a rare disease condition, led to meaningful improvements in patient safety, comfort and self-management and addressed information needs. This collaboration may serve as a template of best practice for future collaborations between POs, research sponsors and other healthcare stakeholders. CI - © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Roennow, Annelise AU - Roennow A AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. AD - Sklerodermiforening, Rødovre, Denmark. FAU - Sauvé, Maureen AU - Sauvé M AD - Scleroderma Canada, Hamilton, Ontario, Canada. FAU - Welling, Joep AU - Welling J AUID- ORCID: 0000-0002-2179-2110 AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium joep.at.fesca@gmail.com. FAU - Riggs, Robert J AU - Riggs RJ AD - Scleroderma Foundation, Inc, Danvers, Massachusetts, USA. FAU - Kennedy, Ann Tyrrell AU - Kennedy AT AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. FAU - Galetti, Ilaria AU - Galetti I AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. AD - Gruppo Italiano per la Lotta alla Sclerodermia, Milan, Italy. FAU - Brown, Edith AU - Brown E AD - Scleroderma and Raynaud's UK, London, UK. FAU - Leite, Catarina AU - Leite C AD - Portuguese Association of Patients with Scleroderma, Monção, Portugal. FAU - Gonzalez, Alex AU - Gonzalez A AD - Scleroderma Research Foundation, San Francisco, California, USA. FAU - Portales Guiraud, Alexandra Paula AU - Portales Guiraud AP AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. AD - Asociación Española de Esclerodermia, Madrid, Spain. FAU - Houÿez, François AU - Houÿez F AD - EURORDIS-Rare Diseases Europe, Barcelona, Spain. FAU - Camp, Rob AU - Camp R AD - EURORDIS-Rare Diseases Europe, Barcelona, Spain. FAU - Gilbert, Annie AU - Gilbert A AD - AK Gilbert Ltd, Brighton, UK. FAU - Gahlemann, Martina AU - Gahlemann M AD - Boehringer Ingelheim Schweiz GmbH, Basel, BS, Switzerland. FAU - Moros, Lizette AU - Moros L AD - Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Rheinland-Pfalz, Germany. FAU - Luna Flores, Jose Luis AU - Luna Flores JL AD - Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Rheinland-Pfalz, Germany. FAU - Schmidt, Friedrich AU - Schmidt F AD - Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Rheinland-Pfalz, Germany. FAU - Sauter, Wiebke AU - Sauter W AD - Boehringer Ingelheim Pharma GmbH and Co KG Biberach, Biberach, Baden-Württemberg, Germany. FAU - Finnern, Henrik AU - Finnern H AD - Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Rheinland-Pfalz, Germany. LA - eng SI - ClinicalTrials.gov/NCT02597933 PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20201216 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - *Delivery of Health Care MH - Humans MH - *Rare Diseases/therapy PMC - PMC7745690 OTO - NOTNLM OT - clinical trials OT - interstitial lung disease OT - medical education & training COIS- Competing interests: The authors have read and understood the BMJ policy on declaration of interests and have the following interests to declare:AR, MS, JW, RJR, ATK, IG, EB, CL, AGo, APPG, FH and RC did not receive any personal payments for their contributions to this article.AR, MS, JW, RJR, ATK, IG, EB, CL and APPG declare payments from Boehringer Ingelheim International GmbH to their respective POs (FESCA aisbl., Scleroderma Canada and Scleroderma Foundation, USA) for participation in community advisory board meetings and payment of travel expenses. AGo declares that Boehringer Ingelheim International GmbH paid for their travel expenses for participation in community advisory board meetings. AR, MS, JW and RJR declare that their POs (FESCA aisbl., Scleroderma Canada and Scleroderma Foundation, USA) received additional payments from Boehringer Ingelheim International GmbH for speaker and advisory services including payment of travel expenses. FH and RC declare that Boehringer Ingelheim International GmbH paid for their travel expenses for speaker and advisory services including one BI internal meeting and community advisory board meetings.RJR declares that the Scleroderma Foundation (USA) has received sponsorships in support of the annual scleroderma awareness raising campaign and the Scleroderma National Patient Education Conference from both Boehringer Ingelheim International GmbH and Boehringer Ingelheim Pharmaceuticals. FH and RC declare that EURORDIS-Rare Diseases Europe has received sponsorships from Boehringer Ingelheim International GmbH for the EURORDIS Round Table of Companies and the European Conference on Rare Diseases and Orphan Products. AR and ATK declare that FESCA aisbl. has received sponsorships in support of their annual scleroderma awareness raising campaign and World Scleroderma Conference from Boehringer Ingelheim International GmbH. MS declares that Scleroderma Canada has received sponsorships in support of their Scleroderma Canada Patient Conference from Boehringer Ingelheim International GmbH. These sponsorships are not related to this article. AGi has worked with the research sponsor (BI) as a paid consultant since September 2016. MG, LM, JLLF, FS, WS and HF are paid employees of the research sponsor (BI). EDAT- 2020/12/18 06:00 MHDA- 2021/05/15 06:00 PMCR- 2020/12/16 CRDT- 2020/12/17 05:38 PHST- 2020/12/17 05:38 [entrez] PHST- 2020/12/18 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/12/16 00:00 [pmc-release] AID - bmjopen-2020-039473 [pii] AID - 10.1136/bmjopen-2020-039473 [doi] PST - epublish SO - BMJ Open. 2020 Dec 16;10(12):e039473. doi: 10.1136/bmjopen-2020-039473. PMID- 16837144 OWN - NLM STAT- MEDLINE DCOM- 20070927 LR - 20220321 IS - 0378-1135 (Print) IS - 0378-1135 (Linking) VI - 117 IP - 2-4 DP - 2006 Oct 31 TI - Dissemination and persistence of Shiga toxin-producing Escherichia coli (STEC) strains on French dairy farms. PG - 180-91 AB - Some Shiga toxin-producing Escherichia coli strains (STEC), and in particular E. coli O157:H7, are known to cause severe illness in humans. STEC have been responsible for large foodborne outbreaks and some of these have been linked to dairy products. The aim of the present study was to determine the dissemination and persistence of STEC on 13 dairy farms in France, which were selected out of 151 randomized dairy farms. A total of 1309 samples were collected, including 415 faecal samples from cattle and 894 samples from the farm environment. Bacteria from samples were cultured and screened for Shiga toxin (stx) genes by polymerase chain reaction (PCR). STEC isolates were recovered from stx-positive samples after colony blotting, and characterized for their virulence genes, serotypes and XbaI digestion patterns of total DNA separated by pulsed-field gel electrophoresis (PFGE). Stx genes were detected in 145 faecal samples (35%) and 179 (20%) environmental samples, and a total of 118 STEC isolates were recovered. Forty-six percent of the STEC isolates were positive for stx1, 86% for stx2, 29% for intimin (eae-gene) and 92% for enterohemolysin (ehx), of which 16% of the STEC strains carried these four virulence factors in combination. Furthermore, we found that some faecal STEC strains belonged to serotypes involved in human disease (O26:H11 and O157:H7). PFGE profiles indicated genetic diversity of the STEC strains and some of these persisted in the farm environment for up to 12 months. A large range of contaminated samples were collected, in particular from udders and teats. These organs are potential sources for contamination and re-contamination of dairy cattle and constitute an important risk for milk contamination. FAU - Fremaux, B AU - Fremaux B AD - Unité de Microbiologie Alimentaire et Prévisionnelle, Ecole Nationale Vétérinaire de Lyon, Marcy l'Etoile, France. b.fremaux@vet-lyon.fr FAU - Raynaud, S AU - Raynaud S FAU - Beutin, L AU - Beutin L FAU - Rozand, C Vernozy AU - Rozand CV LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060519 PL - Netherlands TA - Vet Microbiol JT - Veterinary microbiology JID - 7705469 RN - 0 (Shiga Toxins) SB - IM MH - Animals MH - Cattle MH - Consumer Product Safety MH - Dairying MH - Environmental Microbiology MH - Escherichia coli Infections/epidemiology/microbiology/*veterinary MH - Escherichia coli O157/genetics/isolation & purification/*pathogenicity MH - Feces/microbiology MH - Female MH - Food Contamination MH - *Food Microbiology MH - *Genetic Variation MH - Mastitis, Bovine/*epidemiology/*microbiology MH - Milk/microbiology MH - Serotyping/veterinary MH - Shiga Toxins/*metabolism EDAT- 2006/07/14 09:00 MHDA- 2007/09/28 09:00 CRDT- 2006/07/14 09:00 PHST- 2005/06/14 00:00 [received] PHST- 2006/04/21 00:00 [revised] PHST- 2006/04/21 00:00 [accepted] PHST- 2006/07/14 09:00 [pubmed] PHST- 2007/09/28 09:00 [medline] PHST- 2006/07/14 09:00 [entrez] AID - S0378-1135(06)00185-4 [pii] AID - 10.1016/j.vetmic.2006.04.030 [doi] PST - ppublish SO - Vet Microbiol. 2006 Oct 31;117(2-4):180-91. doi: 10.1016/j.vetmic.2006.04.030. Epub 2006 May 19. PMID- 21777462 OWN - NLM STAT- MEDLINE DCOM- 20111209 LR - 20211020 IS - 1476-4598 (Electronic) IS - 1476-4598 (Linking) VI - 10 DP - 2011 Jul 21 TI - Kinome expression profiling and prognosis of basal breast cancers. PG - 86 LID - 10.1186/1476-4598-10-86 [doi] AB - BACKGROUND: Basal breast cancers (BCs) represent ~15% of BCs. Although overall poor, prognosis is heterogeneous. Identification of good- versus poor-prognosis patients is difficult or impossible using the standard histoclinical features and the recently defined prognostic gene expression signatures (GES). Kinases are often activated or overexpressed in cancers, and constitute targets for successful therapies. We sought to define a prognostic model of basal BCs based on kinome expression profiling. METHODS: DNA microarray-based gene expression and histoclinical data of 2515 early BCs from thirteen datasets were collected. We searched for a kinome-based GES associated with disease-free survival (DFS) in basal BCs of the learning set using a metagene-based approach. The signature was then tested in basal tumors of the independent validation set. RESULTS: A total of 591 samples were basal. We identified a 28-kinase metagene associated with DFS in the learning set (N = 73). This metagene was associated with immune response and particularly cytotoxic T-cell response. On multivariate analysis, a metagene-based predictor outperformed the classical prognostic factors, both in the learning and the validation (N = 518) sets, independently of the lymphocyte infiltrate. In the validation set, patients whose tumors overexpressed the metagene had a 78% 5-year DFS versus 54% for other patients (p = 1.62E-4, log-rank test). CONCLUSIONS: Based on kinome expression, we identified a predictor that separated basal BCs into two subgroups of different prognosis. Tumors associated with higher activation of cytotoxic tumor-infiltrative lymphocytes harbored a better prognosis. Such classification should help tailor the treatment and develop new therapies based on immune response manipulation. FAU - Sabatier, Renaud AU - Sabatier R AD - Department of Molecular Oncology, Centre de Recherche en Cancérologie de Marseille, UMR891 Inserm, Institut Paoli-Calmettes, 27 bd Leï Roure, 13009 Marseille, France. FAU - Finetti, Pascal AU - Finetti P FAU - Mamessier, Emilie AU - Mamessier E FAU - Raynaud, Stéphane AU - Raynaud S FAU - Cervera, Nathalie AU - Cervera N FAU - Lambaudie, Eric AU - Lambaudie E FAU - Jacquemier, Jocelyne AU - Jacquemier J FAU - Viens, Patrice AU - Viens P FAU - Birnbaum, Daniel AU - Birnbaum D FAU - Bertucci, François AU - Bertucci F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110721 PL - England TA - Mol Cancer JT - Molecular cancer JID - 101147698 RN - EC 2.7.- (Phosphotransferases) SB - IM MH - Adenocarcinoma/*diagnosis/enzymology/*genetics/metabolism MH - Adult MH - Aged MH - Aged, 80 and over MH - Breast Neoplasms/*diagnosis/enzymology/*genetics/metabolism MH - Female MH - Gene Expression Profiling MH - Gene Expression Regulation, Enzymologic MH - Gene Expression Regulation, Neoplastic MH - Humans MH - Middle Aged MH - Oligonucleotide Array Sequence Analysis MH - Phosphotransferases/*genetics/metabolism MH - Prognosis MH - Retrospective Studies MH - Validation Studies as Topic PMC - PMC3156788 EDAT- 2011/07/23 06:00 MHDA- 2011/12/14 06:00 PMCR- 2011/07/21 CRDT- 2011/07/23 06:00 PHST- 2011/04/28 00:00 [received] PHST- 2011/07/21 00:00 [accepted] PHST- 2011/07/23 06:00 [entrez] PHST- 2011/07/23 06:00 [pubmed] PHST- 2011/12/14 06:00 [medline] PHST- 2011/07/21 00:00 [pmc-release] AID - 1476-4598-10-86 [pii] AID - 10.1186/1476-4598-10-86 [doi] PST - epublish SO - Mol Cancer. 2011 Jul 21;10:86. doi: 10.1186/1476-4598-10-86. PMID- 35382372 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2618-6500 (Electronic) IS - 2148-5046 (Print) IS - 2148-5046 (Linking) VI - 36 IP - 4 DP - 2021 Dec TI - Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan. PG - 587-594 LID - 10.46497/ArchRheumatol.2021.8701 [doi] AB - OBJECTIVES: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. PATIENTS AND METHODS: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. RESULTS: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). CONCLUSION: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity. CI - Copyright © 2021, Turkish League Against Rheumatism. FAU - Asif, Sadia AU - Asif S AUID- ORCID: 0000-0001-9670-6654 AD - Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan. FAU - Khan, Asadullah AU - Khan A AUID- ORCID: 0000-0001-8067-0124 AD - Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan. FAU - Faiq, Muhammad AU - Faiq M AD - Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan. FAU - Din, Zia Ud AU - Din ZU AD - Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan. FAU - Zahoor, Sarmad AU - Zahoor S AUID- ORCID: 0000-0003-1964-2655 AD - Department of Internal Medicine, King Edward Medical University, Mayo Hospital, Lahore, Pakistan. FAU - Haroon, Muhammad AU - Haroon M AD - Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan. LA - eng PT - Journal Article DEP - 20211024 PL - Turkey TA - Arch Rheumatol JT - Archives of rheumatology JID - 101639000 PMC - PMC8957765 OTO - NOTNLM OT - Interstitial lung disease OT - Raynaud phenomenon OT - scleroderma OT - systemic sclerosis COIS- Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article. EDAT- 2022/04/07 06:00 MHDA- 2022/04/07 06:01 PMCR- 2021/10/24 CRDT- 2022/04/06 05:14 PHST- 2021/01/05 00:00 [received] PHST- 2021/04/26 00:00 [accepted] PHST- 2022/04/06 05:14 [entrez] PHST- 2022/04/07 06:00 [pubmed] PHST- 2022/04/07 06:01 [medline] PHST- 2021/10/24 00:00 [pmc-release] AID - 10.46497/ArchRheumatol.2021.8701 [doi] PST - epublish SO - Arch Rheumatol. 2021 Oct 24;36(4):587-594. doi: 10.46497/ArchRheumatol.2021.8701. eCollection 2021 Dec. PMID- 34053315 OWN - NLM STAT- MEDLINE DCOM- 20221227 LR - 20240102 IS - 1558-9455 (Electronic) IS - 1558-9447 (Print) IS - 1558-9447 (Linking) VI - 18 IP - 1 DP - 2023 Jan TI - Considerations for Hand Surgery in Patients With Scleroderma. PG - 32-39 LID - 10.1177/15589447211017211 [doi] AB - Systemic sclerosis (scleroderma, SSc) is an autoimmune disease that causes significant dysfunction to multiple organ systems, including the musculoskeletal system. It poses significant challenges to the hand surgeon, including calcinosis, ischemic changes, Raynaud phenomenon, tendinopathies, synovitis, and joint contractures. Patients with SSc also suffer from multiorgan dysfunction, which makes them high-risk surgical patients. The hand surgeon must understand the pathophysiology, treatment strategies, and special operative considerations required in this population to avoid complications and help maintain or improve hand function. FAU - Brown, Chelsea R AU - Brown CR AD - The Ohio State University Wexner Medical Center, Columbus, USA. FAU - Crouser, Nisha J AU - Crouser NJ AD - The Ohio State University Wexner Medical Center, Columbus, USA. FAU - Speeckaert, Amy L AU - Speeckaert AL AUID- ORCID: 0000-0002-1013-2045 AD - The Ohio State University Wexner Medical Center, Columbus, USA. LA - eng PT - Journal Article PT - Review DEP - 20210531 PL - United States TA - Hand (N Y) JT - Hand (New York, N.Y.) JID - 101264149 SB - IM MH - Humans MH - *Hand/surgery MH - *Scleroderma, Systemic/complications/surgery PMC - PMC9806536 OTO - NOTNLM OT - Raynaud phenomenon OT - calcinosis OT - hand surgery OT - joint contracture OT - scleroderma OT - systemic sclerosis COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2021/06/01 06:00 MHDA- 2022/12/28 06:00 PMCR- 2024/01/01 CRDT- 2021/05/31 05:25 PHST- 2021/06/01 06:00 [pubmed] PHST- 2022/12/28 06:00 [medline] PHST- 2021/05/31 05:25 [entrez] PHST- 2024/01/01 00:00 [pmc-release] AID - 10.1177_15589447211017211 [pii] AID - 10.1177/15589447211017211 [doi] PST - ppublish SO - Hand (N Y). 2023 Jan;18(1):32-39. doi: 10.1177/15589447211017211. Epub 2021 May 31. PMID- 31194355 STAT- Publisher CTDT- 20240727 PB - StatPearls Publishing DP - 2026 Jan TI - Mixed Connective Tissue Disease. BTI - StatPearls AB - Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disease with the main features of at least 2 overlapping connective tissue diseases, including systemic lupus erythematosus, systemic sclerosis, polymyositis, dermatomyositis, and rheumatoid arthritis. The disease is also defined by the presence of anti-U1-ribonucleoprotein (RNP) antibodies and Raynaud phenomenon. The condition was initially described by Sharp in 1972 through a case series of 25 patients with features of systemic lupus erythematosus, systemic sclerosis, and inflammatory muscle disease associated with anti-U1-RNP antibodies. However, at that time, MCTD was not described as a separate entity from undifferentiated connective tissue disease, and its characteristics have evolved since then. Although most authors describe MCTD as an independent entity, some believe it may represent an early stage of a definite connective tissue disease, such as systemic lupus erythematosus, systemic sclerosis, or overlap syndrome. MCTD has no unique clinical features, and considerable interindividual variation in clinical manifestations exists. Multiple attempts have been made to develop classification criteria, but there are currently no internationally agreed-upon diagnostic criteria. Most clinicians agree on a diagnosis if the following criteria are met: : The presence of a high titer of positive anti-U1-RNP, and Raynaud phenomenon, puffy digits, or hand edema. And at least 2 of the following: Synovitis. Myositis. Leukopenia. Esophageal dysmotility. Pleuritis. Pericarditis. Interstitial lung disease. In 2019, a consensus panel in Japan proposed another revised set of diagnostic criteria for MCTD, which divides the disease features into 4 categories. : The presence of a high titer of positive anti-U1-RNP, and Raynaud phenomenon, puffy digits, or hand edema. And either 1 of the following organ involvements or at least 2 overlapping manifestations: Organ involvement includes: Pulmonary arterial hypertension. Aseptic meningitis. Trigeminal neuropathy. Overlapping manifestations include: Systemic lupus erythematosus: polyarthritis, lymphadenopathy, malar rash, pericarditis or pleuritis, leukopenia or thrombocytopenia. Systemic sclerosis: sclerodactyly, interstitial lung disease, esophageal dysmotility or dilatation. Inflammatory myositis: muscle weakness, elevated levels of myogenic enzymes, myogenic abnormalities on electromyogram. Diagnosis is based on at least 1 common manifestation, immunological manifestation, and either 1 characteristic organ involvement or at least 1 feature in 2 or more overlapping manifestations. These criteria have a sensitivity of 90.6% and a specificity of 98.4%, although they have not been formally adopted by the international community. CI - Copyright © 2026, StatPearls Publishing LLC. FAU - Sapkota, Binita AU - Sapkota B FAU - Al Khalili, Yasir AU - Al Khalili Y AD - Virginia Commonwealth University LA - eng PT - Study Guide PT - Book Chapter PL - Treasure Island (FL) COIS- Disclosure: Binita Sapkota declares no relevant financial relationships with ineligible companies. Disclosure: Yasir Al Khalili declares no relevant financial relationships with ineligible companies. EDAT- 2024/07/27 00:00 CRDT- 2024/07/27 00:00 AID - NBK542198 [bookaccession] PMID- 22216066 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20120823 LR - 20211021 IS - 1874-3129 (Electronic) IS - 1874-3129 (Linking) VI - 5 DP - 2011 TI - Plasma Homocysteine is Not Related to the Severity of Microangiopathy in Secondary Raynaud Phenomenon. PG - 64-8 LID - 10.2174/1874312901105010064 [doi] AB - INTRODUCTION: The role of elevated homocysteine in primary and secondary Raynaud phenomenon (RP) and in patients with atherosclerosis has been reported controversially. In secondary RP due to connective tissue disease specific alterations of nailfold capillaries might be present. An association between these microvascular changes and homocysteine has been suggested. AIM: The aim of this study was to determine whether homocysteine level differs between patients with primary and secondary RP and to test the hypothesis that homocysteine or other cardiovascular risk factors are associated with specific features of microangiopathy in secondary RP. PATIENTS AND METHODS: Eighty-one consecutive patients with RP referred for vascular assessment were studied by nailfold capillaroscopy. Homocysteine, C-reactive protein and cholesterol were measured and other cardiovascular risk factors and comorbidities assessed. RESULTS: Homocysteine, C-reactive-protein and cholesterol levels did not differ between patients with primary (n=60) and secondary RP (n=21). Likewise, no differences in the prevalence of cardiovascular risk factors and comorbidities were found. In secondary RP no correlation was found between microvascular involvement and homocysteine or C-reactive protein. CONCLUSION: Plasma homocysteine is not different in patients with either primary or secondary RP and is therefore not a marker for the distinction of these diseases. The extent of microvascular involvement in secondary RP does not correlate with plasma homocysteine. FAU - Jacomella, Vincenzo AU - Jacomella V AD - Clinic for Angiology, University Hospital, Zurich, Switzerland. FAU - Wasila, Monika AU - Wasila M FAU - Husmann, Marc AU - Husmann M FAU - Gitzelmann, Gabriela AU - Gitzelmann G FAU - Meier, Thomas AU - Meier T FAU - Amann-Vesti, Beatrice AU - Amann-Vesti B LA - eng PT - Journal Article DEP - 20111129 PL - United Arab Emirates TA - Open Rheumatol J JT - The open rheumatology journal JID - 101480507 PMC - PMC3245410 OTO - NOTNLM OT - Raynaud phenomenon OT - homocysteine OT - microangiopathy. OT - nailfold capillaroscopy EDAT- 2012/01/05 06:00 MHDA- 2012/01/05 06:01 PMCR- 2011/01/01 CRDT- 2012/01/05 06:00 PHST- 2011/09/28 00:00 [received] PHST- 2011/09/28 00:00 [revised] PHST- 2011/10/10 00:00 [accepted] PHST- 2012/01/05 06:00 [entrez] PHST- 2012/01/05 06:00 [pubmed] PHST- 2012/01/05 06:01 [medline] PHST- 2011/01/01 00:00 [pmc-release] AID - TORJ-5-64 [pii] AID - 10.2174/1874312901105010064 [doi] PST - ppublish SO - Open Rheumatol J. 2011;5:64-8. doi: 10.2174/1874312901105010064. Epub 2011 Nov 29. PMID- 38149621 OWN - NLM STAT- MEDLINE DCOM- 20240318 LR - 20260301 IS - 1744-8409 (Electronic) IS - 1744-666X (Linking) VI - 20 IP - 4 DP - 2024 Apr TI - Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022. PG - 387-404 LID - 10.1080/1744666X.2023.2298354 [doi] AB - INTRODUCTION: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed. AREAS COVERED: Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology. EXPERT OPINION: We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable. FAU - Foeldvari, Ivan AU - Foeldvari I AUID- ORCID: 0000-0003-0659-5298 AD - Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany. FAU - Torok, Kathryn S AU - Torok KS AUID- ORCID: 0000-0002-1662-143X AD - University of Pittsburgh and University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. FAU - Antón, Jordi AU - Antón J AD - Department of Pediatric Rheumatology. Hospital Sant Joan de Déu and Universitat de Barcelona, Barcelona, Spain. FAU - Blakley, Michael AU - Blakley M AD - Riley Hospital for Children at IU Health, Indiana University School of Medicine, Indianapolis, IN, USA. FAU - Constantin, Tamás AU - Constantin T AD - Unit of Pediatric Rheumatology, Tűzoltó Street Department, Pediatric Centre, Semmelweis University, Budapest, Hungary. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology-Department of Internal Medicine and Specialties, University of Genoa and IRCCS San Martino Polyclinic Hospital, Genoa, Italy. FAU - Denton, Christopher P AU - Denton CP AD - UCL Division of Medicine, Royal Free London NHS Foundation Trust, London, UK. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma & Raynaud's United Kindgom (SRUK) (Research Subcommittee, Patient Research Partner), FESCA, London, UK. FAU - Hinrichs, Bernd AU - Hinrichs B AD - Children's pulmonology, Asklepios Klinik Nord - Heidberg, Hamburg, Germany. FAU - Li, Suzanne C AU - Li SC AD - Hackensack University Medical Center, Hackensack, NJ, USA. FAU - Maillard, Susan AU - Maillard S AD - Great Ormond Street Hospital, London, UK. FAU - Marrani, Edoardo AU - Marrani E AD - Pediatric Rheumatology Unit, Meyer Children's Hospital IRCCS, Florence, Italy. FAU - Moinzadeh, Pia AU - Moinzadeh P AD - Department of Dermatology and Venereology, University Hospital Cologne, Cologne, Germany. FAU - Orteu, Catherine H AU - Orteu CH AD - UCL Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK. FAU - Pain, Clare E AU - Pain CE AD - Alder Hey Children's Foundation NHS Trust, Liverpool, UK. FAU - Pauling, John D AU - Pauling JD AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium and ERN ReCONNET. FAU - Pilkington, Clarissa AU - Pilkington C AD - Great Ormond Street Hospital, London, UK. FAU - Rosser, Franziska AU - Rosser F AD - University of Pittsburgh and University of Pittsburgh Medical Center Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. FAU - Smith, Vanessa AU - Smith V AD - University of California, Los Angeles, CA, USA. AD - University of Washington, Seattle, WA, USA. AD - University of Florence, Florence, Italy. FAU - Furst, Daniel F AU - Furst DF AD - Division of Rheumatology Fellow, Geffen School of Medicine at the University of California in Los Angeles, Los Angeles, CA, USA. LA - eng PT - Consensus Statement PT - Journal Article PT - Review DEP - 20240102 PL - England TA - Expert Rev Clin Immunol JT - Expert review of clinical immunology JID - 101271248 RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Child MH - Humans MH - *Scleroderma, Localized MH - *Scleroderma, Systemic/drug therapy OTO - NOTNLM OT - gastrointestinal involvement OT - juvenile systemic sclerosis OT - musculoskeletal involvement OT - physical therapy OT - pulmonary involvement OT - skin involvement OT - treatment recommendations OT - vascular involvement EDAT- 2023/12/27 12:41 MHDA- 2024/03/18 06:43 CRDT- 2023/12/27 07:50 PHST- 2024/03/18 06:43 [medline] PHST- 2023/12/27 12:41 [pubmed] PHST- 2023/12/27 07:50 [entrez] AID - 10.1080/1744666X.2023.2298354 [doi] PST - ppublish SO - Expert Rev Clin Immunol. 2024 Apr;20(4):387-404. doi: 10.1080/1744666X.2023.2298354. Epub 2024 Jan 2. PMID- 20068225 OWN - NLM STAT- MEDLINE DCOM- 20100427 LR - 20250529 IS - 1528-0020 (Electronic) IS - 0006-4971 (Print) IS - 0006-4971 (Linking) VI - 115 IP - 14 DP - 2010 Apr 8 TI - Prevalence and prognostic impact of allelic imbalances associated with leukemic transformation of Philadelphia chromosome-negative myeloproliferative neoplasms. PG - 2882-90 LID - 10.1182/blood-2009-07-235119 [doi] AB - Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia, and primary myelofibrosis show an inherent tendency for transformation into leukemia (MPN-blast phase), which is hypothesized to be accompanied by acquisition of additional genomic lesions. We, therefore, examined chromosomal abnormalities by high-resolution single nucleotide polymorphism (SNP) array in 88 MPN patients, as well as 71 cases with MPN-blast phase, and correlated these findings with their clinical parameters. Frequent genomic alterations were found in MPN after leukemic transformation with up to 3-fold more genomic changes per sample compared with samples in chronic phase (P < .001). We identified commonly altered regions involved in disease progression including not only established targets (ETV6, TP53, and RUNX1) but also new candidate genes on 7q, 16q, 19p, and 21q. Moreover, trisomy 8 or amplification of 8q24 (MYC) was almost exclusively detected in JAK2V617F(-) cases with MPN-blast phase. Remarkably, copy number-neutral loss of heterozygosity (CNN-LOH) on either 7q or 9p including homozygous JAK2V617F was related to decreased survival after leukemic transformation (P = .01 and P = .016, respectively). Our high-density SNP-array analysis of MPN genomes in the chronic compared with leukemic stage identified novel target genes and provided prognostic insights associated with the evolution to leukemia. FAU - Thoennissen, Nils H AU - Thoennissen NH AD - Division of Hematology and Oncology, Cedars-Sinai Medical Center, University of California Los Angeles School of Medicine, Los Angeles, CA 90048, USA. nils.thoennissen@cshs.org FAU - Krug, Utz O AU - Krug UO FAU - Lee, Dhong Hyun Tony AU - Lee DH FAU - Kawamata, Norihiko AU - Kawamata N FAU - Iwanski, Gabriela B AU - Iwanski GB FAU - Lasho, Terra AU - Lasho T FAU - Weiss, Tamara AU - Weiss T FAU - Nowak, Daniel AU - Nowak D FAU - Koren-Michowitz, Maya AU - Koren-Michowitz M FAU - Kato, Motohiro AU - Kato M FAU - Sanada, Masashi AU - Sanada M FAU - Shih, Lee-Yung AU - Shih LY FAU - Nagler, Arnon AU - Nagler A FAU - Raynaud, Sophie D AU - Raynaud SD FAU - Müller-Tidow, Carsten AU - Müller-Tidow C FAU - Mesa, Ruben AU - Mesa R FAU - Haferlach, Torsten AU - Haferlach T FAU - Gilliland, D Gary AU - Gilliland DG FAU - Tefferi, Ayalew AU - Tefferi A FAU - Ogawa, Seishi AU - Ogawa S FAU - Koeffler, H Phillip AU - Koeffler HP LA - eng GR - R01 CA026038/CA/NCI NIH HHS/United States GR - 5R01CA026038-31/CA/NCI NIH HHS/United States PT - Clinical Trial PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20100112 PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (Neoplasm Proteins) RN - EC 2.7.10.2 (JAK2 protein, human) RN - EC 2.7.10.2 (Janus Kinase 2) SB - IM CIN - Blood. 2010 Apr 8;115(14):2727-8. doi: 10.1182/blood-2010-01-261412. PMID: 20378759 MH - Aged MH - Blast Crisis/*genetics/metabolism/mortality MH - Chromosomes, Human/*genetics/metabolism MH - Disease-Free Survival MH - Female MH - Hematologic Neoplasms/*genetics/metabolism/mortality MH - Humans MH - Janus Kinase 2/*genetics/metabolism MH - *Loss of Heterozygosity MH - Male MH - Middle Aged MH - Neoplasm Proteins/*genetics/metabolism MH - *Philadelphia Chromosome MH - Prevalence MH - Survival Rate PMC - PMC2854432 EDAT- 2010/01/14 06:00 MHDA- 2010/04/28 06:00 PMCR- 2011/04/08 CRDT- 2010/01/14 06:00 PHST- 2010/01/14 06:00 [entrez] PHST- 2010/01/14 06:00 [pubmed] PHST- 2010/04/28 06:00 [medline] PHST- 2011/04/08 00:00 [pmc-release] AID - S0006-4971(20)56533-6 [pii] AID - 2009/235119 [pii] AID - 10.1182/blood-2009-07-235119 [doi] PST - ppublish SO - Blood. 2010 Apr 8;115(14):2882-90. doi: 10.1182/blood-2009-07-235119. Epub 2010 Jan 12. PMID- 26834221 OWN - NLM STAT- MEDLINE DCOM- 20161226 LR - 20220408 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 43 IP - 3 DP - 2016 Mar TI - Quantitative Alterations of Capillary Diameter Have a Predictive Value for Development of the Capillaroscopic Systemic Sclerosis Pattern. PG - 599-606 LID - 10.3899/jrheum.150900 [doi] AB - OBJECTIVE: To quantify earlier capillary diameter abnormalities, observed by nailfold videocapillaroscopy (NVC), in primary Raynaud phenomenon (PRP) subjects compared with RP subjects later evolved to systemic sclerosis (SSc)-associated secondary Raynaud phenomenon (SRP). METHODS: There were 6112 NVC images of 191 subjects analyzed at baseline and after a mean followup of 42.77 ± 35.80 months. We selected 48 patients affected by SRP and 143 matched controls confirmed with PRP. The diameter of the most dilated limbs (arterial, venous, and apical) was measured in 16 images per subject. Statistical analysis was performed using nonparametric tests. The threshold values for capillary diameters associated with the development of SSc-associated SRP were determined through receiver-operating characteristic curves. RESULTS: Mean capillary diameter values were significantly different for arterial, venous, and average diameters (mean value of arterial, venous, and apical) between patients with PRP and SRP (p < 0.0001). These alterations were found to be independent predictors for disease development (p = 0.015). Threshold values of 30 µm (area under the curve = 0.802, sensitivity/specificity = 0.85/0.63) to 31 µm were identified for average, arterial, and venous diameters, with a shortening effect on time to disease development. CONCLUSION: The study showed that capillary diameter is an independent predictor for development of SSc-associated SRP. Progression to SRP is unlikely for subjects affected by RP when average capillary diameter is under 30 μm. Subsequently, the execution of the qualitative/quantitative integrated analysis should be part of the NVC followup of RP subjects. FAU - Trombetta, Amelia Chiara AU - Trombetta AC AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Smith, Vanessa AU - Smith V AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Pizzorni, Carmen AU - Pizzorni C AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Meroni, Marianna AU - Meroni M AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Paolino, Sabrina AU - Paolino S AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Cariti, Caterina AU - Cariti C AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Ruaro, Barbara AU - Ruaro B AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Sulli, Alberto AU - Sulli A AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. FAU - Cutolo, Maurizio AU - Cutolo M AD - From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium.A.C. Trombetta, MD, PhD, Trainee in Clinical and Experimental Immunology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; V. Smith, MD, Associate Professor of Rheumatology, Department of Rheumatology, Ghent University Hospital, Ghent University; C. Pizzorni, MD, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Meroni, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; S. Paolino, PhD, Assistant Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; C. Cariti, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; B. Ruaro, MD, Trainee in Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; A. Sulli, MD, Associate Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa; M. Cutolo, MD, Full Professor of Rheumatology, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa. mcutolo@unige.it. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160201 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Aged MH - Capillaries/*diagnostic imaging MH - Case-Control Studies MH - Disease Progression MH - Female MH - Humans MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Predictive Value of Tests MH - Scleroderma, Systemic/*diagnostic imaging MH - Sensitivity and Specificity OTO - NOTNLM OT - EARLY DIAGNOSIS OT - EARLY SCLERODERMA PATTERN OT - MICROVASCULAR DISEASE OT - NAILFOLD VIDEOCAPILLAROSCOPY OT - RAYNAUD PHENOMENON OT - SYSTEMIC SCLEROSIS EDAT- 2016/02/03 06:00 MHDA- 2016/12/27 06:00 CRDT- 2016/02/03 06:00 PHST- 2015/11/25 00:00 [accepted] PHST- 2016/02/03 06:00 [entrez] PHST- 2016/02/03 06:00 [pubmed] PHST- 2016/12/27 06:00 [medline] AID - jrheum.150900 [pii] AID - 10.3899/jrheum.150900 [doi] PST - ppublish SO - J Rheumatol. 2016 Mar;43(3):599-606. doi: 10.3899/jrheum.150900. Epub 2016 Feb 1. PMID- 29759118 OWN - NLM STAT- MEDLINE DCOM- 20181022 LR - 20220330 IS - 1558-299X (Electronic) IS - 0095-4543 (Linking) VI - 45 IP - 2 DP - 2018 Jun TI - Approach to Patients with Suspected Rheumatic Disease. PG - 169-180 LID - S0095-4543(18)30006-X [pii] LID - 10.1016/j.pop.2018.02.001 [doi] AB - Patients with rheumatic disease may present with a myriad of symptoms, from joint pain and rashes to more subtle findings, such as dry eyes and dry mouth. In this article, the authors review in detail the common presenting symptoms of rheumatic disease along with key features in the history and physical examination to help distinguish these from other disease processes. CI - Copyright © 2018 Elsevier Inc. All rights reserved. FAU - Ventura, Iaszmin AU - Ventura I AD - Section of Rheumatology, University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA. FAU - Reid, Pankti AU - Reid P AD - Section of Rheumatology, University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA. FAU - Jan, Reem AU - Jan R AD - Section of Rheumatology, University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA. Electronic address: rjan@medicine.bsd.uchicago.edu. LA - eng PT - Journal Article PT - Review PL - United States TA - Prim Care JT - Primary care JID - 0430463 SB - IM MH - Connective Tissue Diseases/diagnosis/*etiology MH - Fatigue/etiology MH - Humans MH - Rheumatic Diseases/complications/*diagnosis MH - Sjogren's Syndrome/diagnosis MH - Skin Diseases/diagnosis/*etiology OTO - NOTNLM OT - Alopecia OT - Arthralgia OT - Malar rash OT - Raynaud OT - Sicca EDAT- 2018/05/16 06:00 MHDA- 2018/10/23 06:00 CRDT- 2018/05/16 06:00 PHST- 2018/05/16 06:00 [entrez] PHST- 2018/05/16 06:00 [pubmed] PHST- 2018/10/23 06:00 [medline] AID - S0095-4543(18)30006-X [pii] AID - 10.1016/j.pop.2018.02.001 [doi] PST - ppublish SO - Prim Care. 2018 Jun;45(2):169-180. doi: 10.1016/j.pop.2018.02.001. PMID- 39752322 OWN - NLM STAT- MEDLINE DCOM- 20251210 LR - 20251212 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - SI DP - 2025 Dec 1 TI - Tocilizumab and rituximab for systemic sclerosis interstitial lung disease: a real-world cohort analysis. PG - SI184-SI189 LID - 10.1093/rheumatology/keaf006 [doi] AB - OBJECTIVES: SSc-interstitial lung disease (ILD) is one of the leading causes of mortality in SSc. Data from randomized controlled trials (RCTs) support rituximab and tocilizumab monotherapy but there are limited data regarding their use for those who fail standard immunomodulatory therapies. METHODS: SSc patients treated with rituximab or tocilizumab were retrospectively identified in a single centre cohort. Linear mixed effect models were used to analyse before and after treatment lung function trajectory, and identify patient characteristics associated with treatment response. RESULTS: A total of 127 patients were included for analysis. Fifty-one of 94 (54.2%) and 13 of 33 (39.4%) of the rituximab and tocilizumab cohorts, respectively, were receiving concurrent MMF. Pre-treatment decline in absolute change % forced vital capacity (%FVC)/year and % diffusion capacity for carbon monoxide (%DLCO)/year, respectively, was similar in both cohorts (-3.2% and -4.0% rituximab, and -3.2% and -3.6% tocilizumab). Both treatments resulted in lung function stabilization (%FVC/year and %DLCO/year: 1.2% and +0.2% rituximab cohort, 1.0% and 1.0% tocilizumab cohort). Anti-topoisomerase antibody (ATA)-positive patients had a significant response on %FVC/year to tocilizumab compared with ATA-negative patients. Gender had a significant impact on %FVC/year response to rituximab, with males responding to a greater degree than females. Age, ILD extent and skin subset had no impact on treatment response. CONCLUSION: Combination rituximab or tocilizumab with background immunosuppressive therapy is associated with stabilization in lung function trajectory among those who remain refractory to standard immunosuppressives. Specific patient characteristics have an impact on lung function response. Improved FVC response among ATA patients receiving tocilizumab validate data from RCTs. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Goldman, Nina R AU - Goldman NR AUID- ORCID: 0000-0002-6800-4230 AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. FAU - Nihtyanova, Svetlana I AU - Nihtyanova SI AUID- ORCID: 0000-0001-5178-9038 AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. AD - Clinical Research, Immunology, GSK, London, UK. FAU - Beesley, Claire F AU - Beesley CF AUID- ORCID: 0000-0003-3254-729X AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. FAU - Wells, Athol U AU - Wells AU AUID- ORCID: 0000-0003-2108-6248 AD - Interstitial Lung Disease Unit, Royal Brompton Hospital, Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AUID- ORCID: 0000-0002-1118-797X AD - Interstitial Lung Disease Unit, Royal Brompton Hospital, Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK. AD - Margaret Turner-Warwick Centre for Fibrosing Lung Disease, Imperial College London, London, UK. FAU - Mageed, Rizgar AU - Mageed R AUID- ORCID: 0000-0003-1561-784X AD - Centre for Translation Medicine and Therapeutics, Queen Mary University, London, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Division of Medicine, Centre for Rheumatology, University College London, London, UK. LA - eng GR - MR/V030108/1/Medical Research Council and Scleroderma & Raynaud's UK/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - I031V2H011 (tocilizumab) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 4F4X42SYQ6 (Rituximab) RN - 0 (Immunologic Factors) SB - IM MH - Humans MH - *Antibodies, Monoclonal, Humanized/therapeutic use MH - Male MH - Female MH - *Lung Diseases, Interstitial/drug therapy/etiology/physiopathology MH - *Rituximab/therapeutic use MH - Middle Aged MH - Retrospective Studies MH - *Scleroderma, Systemic/complications/drug therapy MH - Aged MH - Treatment Outcome MH - Vital Capacity MH - Adult MH - *Immunologic Factors/therapeutic use PMC - PMC12695047 OTO - NOTNLM OT - anti-topoisomerase antibody OT - interstitial lung disease OT - rituximab OT - systemic sclerosis OT - tocilizumab EDAT- 2025/01/03 18:20 MHDA- 2025/12/11 06:15 PMCR- 2025/01/03 CRDT- 2025/01/03 13:22 PHST- 2024/06/14 00:00 [received] PHST- 2024/12/06 00:00 [accepted] PHST- 2025/12/11 06:15 [medline] PHST- 2025/01/03 18:20 [pubmed] PHST- 2025/01/03 13:22 [entrez] PHST- 2025/01/03 00:00 [pmc-release] AID - 7942510 [pii] AID - keaf006 [pii] AID - 10.1093/rheumatology/keaf006 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Dec 1;64(SI):SI184-SI189. doi: 10.1093/rheumatology/keaf006. PMID- 36868782 OWN - NLM STAT- MEDLINE DCOM- 20230307 LR - 20260518 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 1 DP - 2023 Mar TI - Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology. LID - 10.1136/rmdopen-2022-002890 [doi] LID - e002890 AB - OBJECTIVE: To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. METHODS: Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. RESULTS: Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). CONCLUSION: The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - De Angelis, Rossella AU - De Angelis R AUID- ORCID: 0000-0001-5169-3511 AD - Rheumatology Unit - Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy r.deangelis@staff.univpm.it. FAU - Ferri, Clodoveo AU - Ferri C AD - Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AD - Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy. FAU - Bajocchi, Gianluigi AU - Bajocchi G AD - Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy. FAU - Dagna, Lorenzo AU - Dagna L AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Zanframundo, Giovanni AU - Zanframundo G AUID- ORCID: 0000-0001-5042-1282 AD - Department of Rheumatology, Policlinico San Matteo, Pavia, Italy, Pavia, Italy. FAU - Foti, Rosario AU - Foti R AD - Rheumatology Unit, A.O.U Policlinico S. Marco, Catania, Italy, Catania, Italy. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AUID- ORCID: 0000-0001-7479-4462 AD - Rheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari, Bari, Italy. FAU - Cuomo, Giovanna AU - Cuomo G AD - Department of Precision Medicine - University of Campania "Luigi Vanvitelli", Naples, Italy, Caserta, Italy. FAU - Ariani, Alarico AU - Ariani A AUID- ORCID: 0000-0003-1428-6102 AD - Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma, Italy, Parma, Italy. FAU - Rosato, Edoardo AU - Rosato E AUID- ORCID: 0000-0002-7417-8093 AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Italy, Roma, Italy. FAU - Lepri, Gemma AU - Lepri G AUID- ORCID: 0000-0003-4141-6937 AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Girelli, Francesco AU - Girelli F AD - Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni - L Pierantoni, Forlì, Italy, Forlì, Italy. FAU - Riccieri, Valeria AU - Riccieri V AD - Department of Rheumatology, Sapienza University of Rome, Rome, Italy, Rome, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Department of Rheumatology, University of Padua, Padua, Italy. FAU - Bosello, Silvia Laura AU - Bosello SL AD - Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, Rome, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy. FAU - Ingegnoli, Francesca AU - Ingegnoli F AD - Division of Clinical Rheumatology, ASST Pini, Dept. of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan, Italy, Milano, Italy. FAU - De Santis, Maria AU - De Santis M AUID- ORCID: 0000-0002-3196-1336 AD - Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, and Humanitas University, Pieve Emanuele, Milan, Italy, Rozzano, Italy. FAU - Murdaca, Giuseppe AU - Murdaca G AD - Research Hospital San Martino, University of Genoa, Genoa, Italy, Genova, Italy. FAU - Abignano, Giuseppina AU - Abignano G AD - Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy. FAU - Romeo, Nicoletta AU - Romeo N AD - Azienda Ospedaliera S Croce e Carle, Cuneo, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Department of Rheumatology, University of Pisa, Pisa, Italy, Italy. FAU - Caminiti, Maurizio AU - Caminiti M AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy, Italy. FAU - Iuliano, Anna Maria AU - Iuliano AM AD - Rheumatology Unit, San Camillo - Forlanini Hospital, Rome, Italy, Italy. FAU - Ciano, Giovanni AU - Ciano G AD - Hospital of Ariano Irpino, Local Health Department, Ariano Irpino, Avellino, Italy. FAU - Beretta, Lorenzo AU - Beretta L AUID- ORCID: 0000-0002-6529-6258 AD - Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy. FAU - Bagnato, Gianluca AU - Bagnato G AD - Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy. FAU - Lubrano, Ennio AU - Lubrano E AUID- ORCID: 0000-0003-1471-6467 AD - Department of Rheumatology, University of Molise, Campobasso, Italy. FAU - De Andres, Ilenia AU - De Andres I AD - Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione "Garibaldi", Catania, Italy. FAU - Giollo, Alessandro AU - Giollo A AD - Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. FAU - Saracco, Marta AU - Saracco M AD - Rheumatology Unit, Mauriziano-Umberto I Hospital, Torino, Italy. FAU - Agnes, Cecilia AU - Agnes C AD - San Lorenzo Hospital, Carmagnola, Turin, Italy. FAU - Cipolletta, Edoardo AU - Cipolletta E AD - Rheumatology Unit - Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy. FAU - Lumetti, Federica AU - Lumetti F AD - Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy. FAU - Spinella, Amelia AU - Spinella A AD - Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy. FAU - Magnani, Luca AU - Magnani L AD - Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy. FAU - De Luca, Giacomo AU - De Luca G AUID- ORCID: 0000-0002-5306-7714 AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy. FAU - Codullo, Veronica AU - Codullo V AUID- ORCID: 0000-0003-2557-8514 AD - Department of Rheumatology, Policlinico San Matteo, Pavia, Italy, Pavia, Italy. FAU - Visalli, Elisa AU - Visalli E AD - Rheumatology Unit, A.O.U Policlinico S. Marco, Catania, Italy, Catania, Italy. FAU - Di Vico, Claudio AU - Di Vico C AD - Department of Precision Medicine - University of Campania "Luigi Vanvitelli", Naples, Italy, Caserta, Italy. FAU - Gigante, Antonietta AU - Gigante A AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Italy, Roma, Italy. FAU - Pellagrino, Greta AU - Pellagrino G AD - Department of Rheumatology, Sapienza University of Rome, Rome, Italy, Rome, Italy. FAU - Pigatto, Erika AU - Pigatto E AD - Department of Medicine, Villa Salus Hospital, Venice, Italy. FAU - Lazzaroni, Maria-Grazia AU - Lazzaroni MG AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy. FAU - Franceschini, Franco AU - Franceschini F AD - Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy. FAU - Generali, Elena AU - Generali E AD - Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, and Humanitas University, Pieve Emanuele, Milan, Italy, Rozzano, Italy. FAU - Mennillo, Gianna AU - Mennillo G AD - Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy. FAU - Barsotti, Simone AU - Barsotti S AD - Department of Rheumatology, University of Pisa, Pisa, Italy, Italy. FAU - Mariano, Giuseppa Pagano AU - Mariano GP AD - Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy, Italy. FAU - Furini, Federica AU - Furini F AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - Vultaggio, Licia AU - Vultaggio L AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - Parisi, Simone AU - Parisi S AUID- ORCID: 0000-0003-4496-8315 AD - Rheumatology Unit, Città della Salute e della Scienza, Turin, Italy. FAU - Peroni, Clara Lisa AU - Peroni CL AD - Rheumatology Unit, Città della Salute e della Scienza, Turin, Italy. FAU - Rozza, Davide AU - Rozza D AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Zanetti, Anna AU - Zanetti A AUID- ORCID: 0000-0001-8408-451X AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Carrara, Greta AU - Carrara G AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Landolfi, Gianpiero AU - Landolfi G AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Scirè, Carlo Alberto AU - Scirè CA AUID- ORCID: 0000-0001-7451-0271 AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. AD - Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy. FAU - Bianchi, Gerolamo AU - Bianchi G AD - Rheumatology Unit, Department of Musculoskeletal Sciences, Local Health Trust 3, La Colletta Hospital, Genoa, Italy. FAU - Fusaro, Enrico AU - Fusaro E AD - Rheumatology Unit, Città della Salute e della Scienza, Turin, Italy. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AD - Rheumatology Unit, San Camillo - Forlanini Hospital, Rome, Italy, Italy. FAU - Govoni, Marcello AU - Govoni M AD - Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy. FAU - D'Angelo, Salvatore AU - D'Angelo S AUID- ORCID: 0000-0002-7442-1110 AD - Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy, Potenza, Italy. FAU - Cozzi, Franco AU - Cozzi F AUID- ORCID: 0000-0003-3627-3927 AD - Department of Medicine, Villa Salus Hospital, Venice, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - Department of Rheumatology, University of Padua, Padua, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy, Modena, Italy. FAU - Iannone, Florenzo AU - Iannone F AD - Rheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari, Bari, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, Milano, Italy. AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy. CN - SPRING-SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society of Rheumatology) LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Carbon-19) RN - 0 (Carbon Radioisotopes) RN - digital ulcers SB - IM EIN - RMD Open. 2023 Jun;9(2):e002890corr1. doi: 10.1136/rmdopen-2022-002890corr1. PMID: 37290927 MH - Seasons MH - *Scleroderma, Systemic MH - *Autoimmune Diseases MH - *Rheumatology MH - Humans MH - Carbon Radioisotopes MH - Skin Ulcer PMC - PMC9990652 OTO - NOTNLM OT - Autoimmune Diseases OT - Autoimmunity OT - Epidemiology OT - Scleroderma, Systemic COIS- Competing interests: None declared. FIR - Ferri, Clodoveo IR - Ferri C FIR - Matucci-Cerinic, Marco IR - Matucci-Cerinic M FIR - Giuseppina, Abignano IR - Giuseppina A FIR - Cecilia, Agnes IR - Cecilia A FIR - Giorgio, Amato IR - Giorgio A FIR - Alarico, Ariani IR - Alarico A FIR - Gianluca, Bagnato IR - Gianluca B FIR - Gianluigi, Bajoicchi IR - Gianluigi B FIR - Simone, Barsotti IR - Simone B FIR - Silvia, Bellando-Randone IR - Silvia BR FIR - Alessia, Benenati IR - Alessia B FIR - Lorenzo, Beretta IR - Lorenzo B FIR - Gerolamo, Bianchi IR - Gerolamo B FIR - Silvia, Bosello IR - Silvia B FIR - Fabio, Cacciapaglia IR - Fabio C FIR - Francesca, Calabrese IR - Francesca C FIR - Maurizio, Caminiti IR - Maurizio C FIR - Corrado, Campochiaro IR - Corrado C FIR - Renato, Carignola IR - Renato C FIR - Giovanni, Ciano IR - Giovanni C FIR - Edoardo, Cipolletta IR - Edoardo C FIR - Veronica, Codullo IR - Veronica C FIR - Franco, Cozzi IR - Franco C FIR - Giovanna, Cuomo IR - Giovanna C FIR - Salvatore, D'Angelo IR - Salvatore D FIR - Lorenzo, Dagna IR - Lorenzo D FIR - Francesca, Dall'Ara IR - Francesca D FIR - Ilenia, De Andres IR - Ilenia A FIR - Rossella, De Angelis IR - Rossella A FIR - Angelo, De Cata IR - Angelo C FIR - Giacomo, De Luca IR - Giacomo L FIR - Maria, De Santis IR - Maria S FIR - Alessandra, Della Rossa IR - Alessandra DR FIR - Claudio, Di Vico IR - Claudio DV FIR - Andrea, Doria IR - Andrea D FIR - Marica, Doveri IR - Marica D FIR - Rosario, Foti IR - Rosario F FIR - Federica, Furini IR - Federica F FIR - Enrico, Fusaro IR - Enrico F FIR - Elena, Generali IR - Elena G FIR - Antonietta, Gigante IR - Antonietta G FIR - Alessandro, Giollo IR - Alessandro G FIR - Francesco, Girelli IR - Francesco G FIR - Dilia, Giuggioli IR - Dilia G FIR - Marcello, Govoni IR - Marcello G FIR - Serena, Guiducci IR - Serena G FIR - Florenzo, Iannone IR - Florenzo I FIR - Francesca, Ingegnoli IR - Francesca I FIR - Maria, Iuliano Anna IR - Maria IA FIR - Grazia, Lazzaroni Maria IR - Grazia LM FIR - Gemma, Lepri IR - Gemma L FIR - Ennio, Lubrano IR - Ennio L FIR - Federica, Lumetti IR - Federica L FIR - Luca, Magnani IR - Luca M FIR - Gianna, Mennillo IR - Gianna M FIR - Ospedale, Murdaca Giuseppe IR - Ospedale MG FIR - Giuseppa, Pagano Mariano IR - Giuseppa PM FIR - Simone, Parisi IR - Simone P FIR - Greta, Pellegrino IR - Greta P FIR - Lisa, Peroni Clara IR - Lisa PC FIR - Erika, Pigatto IR - Erika P FIR - Valeria, Riccieri IR - Valeria R FIR - Nicoletta, Romeo IR - Nicoletta R FIR - Edoardo, Rosato IR - Edoardo R FIR - Gianluca, Sambataro IR - Gianluca S FIR - Marta, Saracco IR - Marta S FIR - Giandomenico, Sebastiani IR - Giandomenico S FIR - Amelia, Spinella IR - Amelia S FIR - Rossella, Talotta IR - Rossella T FIR - Elisa, Visalli IR - Elisa V FIR - Licia, Vultaggio IR - Licia V FIR - Elisabetta, Zanatta IR - Elisabetta Z FIR - Giovanni, Zanframundo IR - Giovanni Z FIR - Scirè, Carlo IR - Scirè C FIR - Carrara, Greta IR - Carrara G FIR - Landolfi, Gianpiero IR - Landolfi G FIR - Rozza, Davide IR - Rozza D FIR - Zanetti, Anna IR - Zanetti A EDAT- 2023/03/04 06:00 MHDA- 2023/03/08 06:00 PMCR- 2023/03/03 CRDT- 2023/03/03 21:03 PHST- 2022/11/24 00:00 [received] PHST- 2023/02/08 00:00 [accepted] PHST- 2023/03/03 21:03 [entrez] PHST- 2023/03/04 06:00 [pubmed] PHST- 2023/03/08 06:00 [medline] PHST- 2023/03/03 00:00 [pmc-release] AID - rmdopen-2022-002890 [pii] AID - 10.1136/rmdopen-2022-002890 [doi] PST - ppublish SO - RMD Open. 2023 Mar;9(1):e002890. doi: 10.1136/rmdopen-2022-002890. PMID- 39270208 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241215 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 6 IP - 12 DP - 2024 Dec TI - Association of Combined Autoreactivity to Sm/RNP Common Motif and U1 RNP With Mixed Connective Tissue Disease and Systemic Lupus Erythematosus. PG - 856-862 LID - 10.1002/acr2.11739 [doi] AB - OBJECTIVE: This study aimed to evaluate the clinical features in patients with suspected connective tissue disease who tested positive for anti-Sm/RNP common motif antibody with or without associated anti-RNP antibody. METHODS: The titers of anti-Sm/RNP and anti-RNP antibodies were measured by the multiplex solid-phase bioassays (Bio-Rad). Clinical manifestations were compared among the three subgroups (RNP only, Sm/RNP only, and double positive for RNP and Sm/RNP). Patients were further evaluated for the diagnosis of mixed connective tissue disease (MCTD) and/or systemic lupus erythematosus (SLE) using accepted classification criteria. RESULTS: A total of 133 patients were included in this study. The rates of inflammatory arthritis and Raynaud phenomenon were significantly higher in patients testing positive for both anti-RNP and anti-Sm/RNP antibodies compared to anti-RNP only or anti-Sm/RNP only (69.1% vs 28.8% vs 25.0%, P < 0.0001 for arthritis and 59.5% vs 23.3% vs 37.5%, P = 0.0005 for Raynaud phenomenon). Area under the curve (AUC) values were 0.68 (95% confidence interval [CI] 0.59-0.77, P < 0.0001) for anti-Sm/RNP titers and 0.65 (95% CI 0.55-0.74, P = 0.0039) for anti-RNP titers with inflammatory arthritis. AUC values were 0.67 (95% CI 0.58-0.77, P = 0.0002) for anti-Sm/RNP titers and 0.59 (95% CI 0.49-0.69, P = 0.0352) for anti-RNP titers with Raynaud phenomenon. The odds ratios for the diagnosis of MCTD and SLE were significantly higher in patients with double positivity compared to those testing solely positive for anti-RNP antibody. CONCLUSION: Anti-Sm/RNP common motif autoreactivity when combined with anti-RNP antibody positivity identifies those patients who are closely related with certain clinical manifestations and who are associated with well-defined connective tissue disease such as MCTD or SLE. CI - © 2024 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Ni, Ruoning AU - Ni R AUID- ORCID: 0000-0002-6463-4796 AD - The University of Iowa, Iowa City. FAU - Lenert, Aleksander AU - Lenert A AD - The University of Iowa, Iowa City. FAU - Lenert, Petar AU - Lenert P AD - The University of Iowa, Iowa City. LA - eng GR - K23 AR082966/AR/NIAMS NIH HHS/United States GR - Kelting Foundation/ GR - 1K23AR082966-01/CL/CLC NIH HHS/United States PT - Journal Article DEP - 20240913 PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC11638137 EDAT- 2024/09/13 18:44 MHDA- 2024/09/13 18:45 PMCR- 2024/09/13 CRDT- 2024/09/13 16:43 PHST- 2024/08/08 00:00 [revised] PHST- 2024/03/15 00:00 [received] PHST- 2024/08/13 00:00 [accepted] PHST- 2024/09/13 18:45 [medline] PHST- 2024/09/13 18:44 [pubmed] PHST- 2024/09/13 16:43 [entrez] PHST- 2024/09/13 00:00 [pmc-release] AID - ACR211739 [pii] AID - 10.1002/acr2.11739 [doi] PST - ppublish SO - ACR Open Rheumatol. 2024 Dec;6(12):856-862. doi: 10.1002/acr2.11739. Epub 2024 Sep 13. PMID- 37018139 OWN - NLM STAT- MEDLINE DCOM- 20231204 LR - 20231217 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 62 IP - 12 DP - 2023 Dec 1 TI - COVID-19 infection, admission and death and the impact of corticosteroids among people with rare autoimmune rheumatic disease during the second wave of COVID-19 in England: results from the RECORDER Project. PG - 3828-3837 LID - 10.1093/rheumatology/kead150 [doi] AB - OBJECTIVES: To calculate the rates of COVID-19 infection and COVID-19-related death among people with rare autoimmune rheumatic diseases (RAIRD) during the second wave of the COVID-19 pandemic in England, and describe the impact of corticosteroids on outcomes. METHODS: Hospital Episode Statistics data were used to identify people alive on 1 August 2020 with ICD-10 codes for RAIRD from the whole population of England. Linked national health records were used to calculate rates and rate ratios of COVID-19 infection and death up to 30 April 2021. Primary definition of COVID-19-related death was mention of COVID-19 on the death certificate. NHS Digital and Office for National Statistics general population data were used for comparison. The association between 30-day corticosteroid usage and COVID-19-related death, COVID-19-related hospital admissions and all-cause deaths was also described. RESULTS: Of 168 330 people with RAIRD, 9961 (5.92%) had a positive COVID-19 PCR test. The age-standardized infection rate ratio between RAIRD and the general population was 0.99 (95% CI: 0.97, 1.00). 1342 (0.80%) people with RAIRD died with COVID-19 on their death certificate and the age-sex-standardized mortality rate for COVID-19-related death was 2.76 (95% CI: 2.63, 2.89) times higher than in the general population. There was a dose-dependent relationship between 30-day corticosteroid usage and COVID-19-related death. There was no increase in deaths due to other causes. CONCLUSIONS: During the second wave of COVID-19 in England, people with RAIRD had the same risk of COVID-19 infection but a 2.76-fold increased risk of COVID-19-related death compared with the general population, with corticosteroids associated with increased risk. CI - © The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Rutter, Megan AU - Rutter M AD - Department of Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - Lanyon, Peter C AU - Lanyon PC AD - Department of Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. AD - National Institute for Health Research (NIHR), Nottingham Biomedical Research Centre, Nottingham, UK. FAU - Grainge, Matthew J AU - Grainge MJ AUID- ORCID: 0000-0001-7181-4042 AD - Department of Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. FAU - Hubbard, Richard AU - Hubbard R AD - Department of Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - National Institute for Health Research (NIHR), Nottingham Biomedical Research Centre, Nottingham, UK. FAU - Bythell, Mary AU - Bythell M AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - Stilwell, Peter AU - Stilwell P AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - Aston, Jeanette AU - Aston J AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - McPhail, Sean AU - McPhail S AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - Stevens, Sarah AU - Stevens S AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. FAU - Pearce, Fiona A AU - Pearce FA AUID- ORCID: 0000-0003-2884-1998 AD - Department of Lifespan and Population Health, School of Medicine, University of Nottingham, Nottingham, UK. AD - Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. AD - National Congenital Anomaly and Rare Disease Registration Service, National Disease Registration Service, NHS Digital, Leeds, UK. AD - National Institute for Health Research (NIHR), Nottingham Biomedical Research Centre, Nottingham, UK. LA - eng GR - 22727/Versus Arthritis Clinical Research/ GR - NIHR300863/NIHR/ GR - Lupus UK/ GR - Scleroderma and Raynaud's UK/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Humans MH - *COVID-19/epidemiology MH - SARS-CoV-2 MH - Pandemics MH - England/epidemiology MH - *Rheumatic Diseases/drug therapy/epidemiology MH - Adrenal Cortex Hormones/therapeutic use PMC - PMC10691923 OTO - NOTNLM OT - COVID-19 OT - coronavirus OT - epidemiology OT - infection OT - mortality OT - rare autoimmune rheumatic diseases OT - shielding EDAT- 2023/04/06 06:00 MHDA- 2023/12/04 12:42 PMCR- 2023/04/05 CRDT- 2023/04/05 13:12 PHST- 2023/01/30 00:00 [received] PHST- 2023/03/20 00:00 [accepted] PHST- 2023/12/04 12:42 [medline] PHST- 2023/04/06 06:00 [pubmed] PHST- 2023/04/05 13:12 [entrez] PHST- 2023/04/05 00:00 [pmc-release] AID - 7108765 [pii] AID - kead150 [pii] AID - 10.1093/rheumatology/kead150 [doi] PST - ppublish SO - Rheumatology (Oxford). 2023 Dec 1;62(12):3828-3837. doi: 10.1093/rheumatology/kead150. PMID- 39400748 OWN - NLM STAT- MEDLINE DCOM- 20250506 LR - 20260514 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 5 DP - 2025 May 1 TI - Self-reported skin severity and quality of life in systemic sclerosis: multicentre validation of PASTUL. PG - 2802-2809 LID - 10.1093/rheumatology/keae561 [doi] AB - OBJECTIVES: The aim of this study was to validate the Patient self-Assessment of Skin Thickness in Upper Limb (PASTUL) questionnaire in SSc and assess impact of skin involvement on health-related quality of life (HRQoL). METHODS: Participants were included in four UK centres. PASTUL specifies a grading of skin at eight sites corresponding to the modified Rodnan Skin Score (mRSS). Construct validity was assessed by comparing PASTUL scores with mRSS. HRQoL was evaluated with EuroQoL 5 dimension 5 levels (EQ5D5L) and Leeds SSc QoL questionnaires. Additionally, correlation between PASTUL and Scleroderma Skin Patient-Reported Outcome (SSPRO) was explored. Follow-up was 12 months. RESULTS: In total, 196 participants were included, mean age was 56.4 years (s.d. 13.9), 80.6% female (n = 158), mean disease duration 11.9 years (s.d. 9.9), 110 (56.1%) had lcSSc and 81 (41.3%) dcSSc. PASTUL and upper limb mRSS were well correlated at baseline, 6 and 12 months [intraclass correlation coefficients (ICC) = 0.67, 0.78 and 0.62, P < 0.001]. Test-retest reliability was good (ICC = 0.83, P < 0.001). There was a stronger correlation between PASTUL and upper limb mRSS in dcSSc compared with lcSSc (0.69 vs 0.51, P < 0.001). In participants with early disease (<4 years) PASTUL was moderately correlated with HRQoL (r = 0.53, P < 0.001); correlations were weaker in the whole group. Mean time to do the PASTUL self-assessment was 5.0 min (s.d. 3.7). CONCLUSION: PASTUL is a feasible outcome tool that adds to assessments such as SSPRO. Skin thickening is correlated with HRQoL, particularly in early disease. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Spierings, Julia AU - Spierings J AUID- ORCID: 0000-0002-2546-312X AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School Royal Free Campus, London, UK. AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - Welsing, Paco M J AU - Welsing PMJ AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - Colak, Seda AU - Colak S AUID- ORCID: 0000-0002-5703-6739 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Quah, Helen AU - Quah H AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School Royal Free Campus, London, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Herrick, Ariane L AU - Herrick AL AUID- ORCID: 0000-0003-4941-7926 AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester & Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Hughes, Michael AU - Hughes M AUID- ORCID: 0000-0003-3361-4909 AD - Division of Musculoskeletal & Dermatological Sciences, The University of Manchester & Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - Department of Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School Royal Free Campus, London, UK. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School Royal Free Campus, London, UK. LA - eng GR - Scleroderma & Raynaud's United Kingdom/ PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Validation Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Quality of Life MH - Female MH - Middle Aged MH - Male MH - Severity of Illness Index MH - *Scleroderma, Systemic/pathology/psychology MH - *Self Report MH - *Skin/pathology MH - Aged MH - Patient Reported Outcome Measures MH - Adult MH - Surveys and Questionnaires MH - Reproducibility of Results MH - Upper Extremity/pathology PMC - PMC12048044 OTO - NOTNLM OT - patient reported outcome OT - quality of life OT - scleroderma OT - skin OT - systemic sclerosis EDAT- 2024/10/14 12:22 MHDA- 2025/05/06 06:29 PMCR- 2024/10/14 CRDT- 2024/10/14 11:16 PHST- 2024/06/24 00:00 [received] PHST- 2024/09/26 00:00 [accepted] PHST- 2025/05/06 06:29 [medline] PHST- 2024/10/14 12:22 [pubmed] PHST- 2024/10/14 11:16 [entrez] PHST- 2024/10/14 00:00 [pmc-release] AID - 7821206 [pii] AID - keae561 [pii] AID - 10.1093/rheumatology/keae561 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 May 1;64(5):2802-2809. doi: 10.1093/rheumatology/keae561. PMID- 34178517 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210629 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 5 DP - 2021 May 23 TI - Intraductal Papillary Mucinous Neoplasm of the Pancreas Arising in a Patient With Limited Cutaneous Systemic Sclerosis. PG - e15197 LID - 10.7759/cureus.15197 [doi] LID - e15197 AB - Systemic sclerosis (SS) is a heterogenous autoimmune disease that manifests itself with skin and internal organ involvement. The association of SS and malignancy is an emerging field of study with limited data in the literature. This report highlights the unique case of a patient with limited cutaneous SS (lcSS) found to have an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. In this report, we review the clinical manifestations, serologic findings, and phenotypes of SS. Furthermore, an evaluation of the risk of pancreatic neoplasms in patients with SS will be discussed, as well as the correlation of cancers among SS phenotypes and auto-antibodies. As part of our research, a PubMed search of the following terms was performed: "systemic sclerosis, scleroderma, limited cutaneous systemic sclerosis, CREST syndrome, Raynaud syndrome, cancer, malignancy, pancreas, and intraductal papillary mucinous neoplasm". CI - Copyright © 2021, Azzam et al. FAU - Azzam, Alex AU - Azzam A AD - Internal Medicine, University of Arizona College of Medicine - Tucson, Tucson, USA. FAU - Azzam, Martin AU - Azzam M AD - Dermatology, University of Missouri School of Medicine - Columbia, Columbia, USA. AD - Transitional Year, Southern Hills Hospital and Medical Center, Las Vegas, USA. FAU - Arif, Salman AU - Arif S AD - Internal Medicine, Southern Hills Hospital and Medical Center, Las Vegas, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210523 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8219343 OTO - NOTNLM OT - cancer OT - crest syndrome OT - intraductal papillary mucinous neoplasm OT - limited cutaneous OT - malignancy OT - pancreas OT - raynaud OT - scleroderma OT - systemic sclerosis COIS- The authors have declared that no competing interests exist. EDAT- 2021/06/29 06:00 MHDA- 2021/06/29 06:01 PMCR- 2021/05/23 CRDT- 2021/06/28 06:02 PHST- 2021/06/28 06:02 [entrez] PHST- 2021/06/29 06:00 [pubmed] PHST- 2021/06/29 06:01 [medline] PHST- 2021/05/23 00:00 [pmc-release] AID - 10.7759/cureus.15197 [doi] PST - epublish SO - Cureus. 2021 May 23;13(5):e15197. doi: 10.7759/cureus.15197. PMID- 32266587 OWN - NLM STAT- MEDLINE DCOM- 20210816 LR - 20210816 IS - 1573-742X (Electronic) IS - 0929-5305 (Linking) VI - 50 IP - 4 DP - 2020 Nov TI - Low prevalence of JAK2 V617F mutation in patients with thrombosis and normal blood counts: a retrospective impact study. PG - 995-1003 LID - 10.1007/s11239-020-02100-z [doi] AB - To determine the prevalence of the V617F Janus Kinase 2 (JAK2) mutation in patients with thrombosis without other biological signs of underlying myeloproliferative neoplasm (MPN) and identify associated risk factors for thrombosis. Over a 10-year period, data were collected from patients with thrombotic events and who had also been screened for the V617F JAK2 mutation. Patients with signs of underlying MPN, such as haematocrit levels ≥ 50% and/or platelet counts ≥ 450 × 10(9)/L and/or splanchnic thrombosis were excluded from the study. Of 340 patients fulfilling inclusion criteria, JAK2 mutation was found in 9 (2.65%), the allele burden being at least 2% in 4 (1.1%). Upon follow-up, MPN was diagnosed in the latter 4. Univariate analysis of the whole cohort showed that age (54 ± 15 vs. 64 ± 13, p = 0.027), platelet count (317 ± 111 vs. 255 ± 75, p = 0.017), C-reactive protein level > 5 mg/L (OR 7.29, p = 0.014), and splenomegaly (OR 54.5, p = 0.0002) were significantly associated with JAK2 mutation. There was also a trend for an increased risk of cerebral venous thrombosis (OR 6.54, p = 0.064). Logistic regression confirmed a significant association between splenomegaly and JAK2 mutation (OR 43.15 [95%CI, 3.05-610.95], p = 0.0054). The V617F JAK2 mutation is rarely found in patients with thrombotic events without overt MPN. Splenomegaly, however, is a statistically and clinically relevant indicator of a potential JAK2 mutation in patients with non-splanchnic thrombotic events. Such patients should require further assessment and a close follow-up. FAU - Levraut, Michaël AU - Levraut M AD - Université Côte D'Azur, Nice, France. AD - Department of Internal Medicine, Nice University Hospital, Nice, France. AD - Institut de Pharmacologie Moléculaire Et Cellulaire, Team Non Coding Genome & Lung Disorders, CNRS UMR7275, Valbonne, France. FAU - Legros, Laurence AU - Legros L AD - Université Côte D'Azur, Nice, France. AD - Department of Clinical Haematology, Nice University Hospital, Nice, France. FAU - Drappier, Charles AU - Drappier C AD - Université Côte D'Azur, Nice, France. AD - Department of Internal Medicine, Nice University Hospital, Nice, France. FAU - Béné, Marie C AU - Béné MC AD - Haematology Biology, Nantes University Hospital, Nantes, France. FAU - Queyrel, Viviane AU - Queyrel V AD - Université Côte D'Azur, Nice, France. AD - Department of Internal Medicine, Nice University Hospital, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Université Côte D'Azur, Nice, France. AD - Department of Haematology, Nice University Hospital, Nice, France. FAU - Martis, Nihal AU - Martis N AUID- ORCID: 0000-0002-8932-3169 AD - Université Côte D'Azur, Nice, France. nihal.martis@gmail.com. AD - Department of Internal Medicine, Nice University Hospital, Nice, France. nihal.martis@gmail.com. AD - Institut de Pharmacologie Moléculaire Et Cellulaire, Team Non Coding Genome & Lung Disorders, CNRS UMR7275, Valbonne, France. nihal.martis@gmail.com. LA - eng PT - Journal Article PL - Netherlands TA - J Thromb Thrombolysis JT - Journal of thrombosis and thrombolysis JID - 9502018 RN - 9007-41-4 (C-Reactive Protein) RN - EC 2.7.10.2 (JAK2 protein, human) RN - EC 2.7.10.2 (Janus Kinase 2) SB - IM MH - C-Reactive Protein/analysis MH - Cohort Studies MH - Female MH - France/epidemiology MH - Genetic Predisposition to Disease MH - Humans MH - *Intracranial Thrombosis/etiology/prevention & control MH - Janus Kinase 2/*genetics MH - Male MH - Middle Aged MH - Mutation MH - *Myeloproliferative Disorders/classification/epidemiology/genetics MH - Platelet Count/methods MH - Prevalence MH - Risk Assessment MH - Risk Factors MH - Splenomegaly/*diagnostic imaging MH - *Thrombosis/blood/epidemiology/genetics/physiopathology MH - Venous Thrombosis OTO - NOTNLM OT - Janus kinase 2 OT - Myeloproliferative neoplasia OT - Splenomegaly OT - Thrombophilia OT - Thrombosis EDAT- 2020/04/09 06:00 MHDA- 2021/08/17 06:00 CRDT- 2020/04/09 06:00 PHST- 2020/04/09 06:00 [pubmed] PHST- 2021/08/17 06:00 [medline] PHST- 2020/04/09 06:00 [entrez] AID - 10.1007/s11239-020-02100-z [pii] AID - 10.1007/s11239-020-02100-z [doi] PST - ppublish SO - J Thromb Thrombolysis. 2020 Nov;50(4):995-1003. doi: 10.1007/s11239-020-02100-z. PMID- 17625608 OWN - NLM STAT- MEDLINE DCOM- 20071011 LR - 20130304 IS - 0887-6924 (Print) IS - 0887-6924 (Linking) VI - 21 IP - 9 DP - 2007 Sep TI - Common deleted genes in the 5q- syndrome: thrombocytopenia and reduced erythroid colony formation in SPARC null mice. PG - 1931-6 AB - The commonly deleted region (CDR) for the 5q- syndrome has been identified as a 1.5-megabase interval on human chromosome 5q32. We studied, by real-time reverse-transcription (RT)-PCR, the expression of 33 genes within the CDR that are known to be expressed in CD34+ hematopoietic stem cells. Genes in the 5q- samples that showed the most pronounced decrease in expression compared to non-5q- samples were: solute carrier family 36, member 1 (SLC36A1; 89% downregulated), Ras-GTPase-activating protein SH3 domain-binding (G3BP; 79%), antioxidant protein 1 (ATOX1; 76%), colony-stimulating factor-1 receptor precursor (CSF1R; 76%), ribosomal protein S14 (RPS14; 74%), platelet-derived growth factor receptor-beta (PDGFRB; 73%), Nef-associated factor 1 (TNIP1; 72%), secreted protein, acidic and rich in cysteine (SPARC; 71%), annexin VI (ANAX6; 69%), NSDT (66%) and TIGD (60%). We further studied the hematopoietic system in SPARC-null mice. These mice showed significantly lower platelet counts compared to wild-type animals (P=0.008). Although hemoglobin, hematocrit and mean corpuscular volume (MCV) were lower in mice lacking SPARC, differences were not statistically significant. SPARC-null mice showed a significantly impaired ability to form erythroid burst-forming units (BFU-E). However, no significant differences were found in the formation of erythroid colony-forming units (CFU-E), granulocyte/monocyte colony-forming units (CFU-GM) or megakaryocyte colony-forming units (CFU-Mk) in these animals. We conclude that many of the genes within the CDR associated with the 5q- syndrome exhibit significantly decreased expression and that SPARC, as a potential tumor suppressor gene, may play a role in the pathogenesis of this disease. FAU - Lehmann, S AU - Lehmann S AD - Division of Hematology/Oncology, Cedars-Sinai Medical Center, School of Medicine, University of California-Los Angeles, Los Angeles, CA, USA. Soren.Lehmann@ki.se FAU - O'Kelly, J AU - O'Kelly J FAU - Raynaud, S AU - Raynaud S FAU - Funk, S E AU - Funk SE FAU - Sage, E H AU - Sage EH FAU - Koeffler, H P AU - Koeffler HP LA - eng PT - Journal Article DEP - 20070712 PL - England TA - Leukemia JT - Leukemia JID - 8704895 RN - 0 (Osteonectin) SB - IM MH - Animals MH - Bone Marrow Cells/cytology MH - Chromosome Deletion MH - Erythrocyte Count MH - Erythroid Cells/cytology MH - Flow Cytometry MH - Gene Expression Profiling MH - Genes, Tumor Suppressor MH - HL-60 Cells MH - Hematopoiesis/genetics MH - Humans MH - Megakaryocytes/cytology MH - Mice MH - Mice, Inbred C57BL MH - Mice, Mutant Strains MH - Myelodysplastic Syndromes/*genetics/*pathology MH - Osteonectin/*genetics MH - Platelet Count MH - Reverse Transcriptase Polymerase Chain Reaction MH - Stem Cells MH - Thrombocytopenia/*genetics/*pathology EDAT- 2007/07/13 09:00 MHDA- 2007/10/12 09:00 CRDT- 2007/07/13 09:00 PHST- 2007/07/13 09:00 [pubmed] PHST- 2007/10/12 09:00 [medline] PHST- 2007/07/13 09:00 [entrez] AID - 2404852 [pii] AID - 10.1038/sj.leu.2404852 [doi] PST - ppublish SO - Leukemia. 2007 Sep;21(9):1931-6. doi: 10.1038/sj.leu.2404852. Epub 2007 Jul 12. PMID- 35864917 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220723 IS - 0253-8253 (Print) IS - 2227-0426 (Electronic) IS - 0253-8253 (Linking) VI - 2022 IP - 3 DP - 2022 TI - Seropositive Neuromyelitis Optica in a Case of Undiagnosed Ankylosing Spondylitis: A Neuro-Rheumatological Conundrum. PG - 29 LID - 10.5339/qmj.2022.29 [doi] LID - 29 AB - Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels. Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases. However, AQP-4-antibody-positive NMOSD coexisting with ankylosing spondylitis (AS) is rare. AS is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27. In this study, a 35-year-old Indian man with an undiagnosed progressive axial spondyloarthropathy (i.e., AS) is reported presenting with acute-onset longitudinally extensive transverse myelitis, a clinical subset of NMOSD. Neuromyelitis optica spectrum disorder (NMOSD), a primary demyelinating disorder of the central nervous system (CNS), is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels, which manifests with optic neuritis, longitudinally extensive transverse myelitis (LETM), area-postrema syndrome, brainstem syndrome diencephalic syndrome, and cerebral syndrome.(1-4) Ankylosing spondylitis (AS) is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27.(5,6) AS characteristically targets the axial skeleton, peripheral joints, entheses (connective tissues between tendons/ligaments and bones), and gut.(5,6) Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases.(7) For example, cases with NMOSD and multiple sclerosis, which are other autoimmune primary demyelinating disorders of the CNS, have been reported.(8,9) However, concurrent existence of AS and NMOSD in the same patient even over years of disease course is rare.(10,11) In addition, studies describing neurological manifestations of AS are limited,(12) and they focus on joint inflammation and long-standing bony pathology (ankylosis) related to compressive myelopathy, myelo-radiculopathy, and cauda equina syndromes.(12,13) The authors present a case of a young Indian man with an undiagnosed progressive AS (misdiagnosed and mismanaged by an indigenous medical practitioner) presenting with acute-onset LETM variant of AQP4-positive NMOSD. A 35-year-old healthy, non-comorbid man from rural India came to the outpatient department with complaints of persistent tingling, numbness, and weakness of both lower limbs (right more than left) for 10 days. The clinical picture showed acute-onset urinary retention, which was relieved by urinary catheterization. An indigenous medical practitioner had prescribed drugs to treat a urinary tract infection. His weakness gradually progressed over the following week, causing him to become bedridden. During the removal of the catheter, he felt urgency, increased frequency of micturition, and overt urinary incontinence. He gave no history suggestive of any girdle-like sensations, root/radicular/tract pain, vertebral pain, trauma, recent vaccination, and diarrheal or febrile illness. For the last 8 months, he had a complaint of an insidious-onset, persistent, bilateral, dull aching pain in the gluteal region accompanied by low-back pain and morning stiffness up to 1 h, which markedly improved with activity and reoccurred following long periods of inactivity. He sometimes had to rise in the middle of the night because of excruciating pain, which could be relieved after moving around the room and corridors for half an hour. He was taking over-the-counter diclofenac tablets for pain relief prescribed by some indigenous medical practitioners who told him that it was due to overwork in agricultural fields, that is, mechanical back pain. He also had a normal X-ray of the lumbosacral spine. He had no addiction liabilities, and none of the family members had ever suffered from a similar kind of illness. He had never consulted any trained medical practitioner, as his previous back-pain-related symptoms responded well to the tablets prescribed by the indigenous medical practitioner(s). During examination, he was found to have recent-onset, asymmetric spastic paraparesis (right more than left) with upper motor neuron-type urinary bladder symptoms. Cognitive assessment (assessed by the Montreal cognitive assessment test) was normal, and posterior column sensations were preserved. Sensory system examination revealed no definite sensory level. Except for the paretic lower limbs, cerebellar functions were normal in other regions. Neuro-ophthalmological examinations were also normal, and no signs of meningeal irritation were observed. The history and course of the disease and clinical examinations were analyzed. Selective tractopathy (early and predominant motor and autonomic tract affection) was suggested for an intramedullary demyelinating pathology affecting the anterior central cord. This case was initially classified as acute-onset non-compressive myelopathy at the lower cervical/upper dorsal region level in a patient with a pre-existing axial spondyloarthropathy. Complete blood cell count; liver, kidney, and thyroid function tests; and plasma glucose and electrolytes were normal, except for an increased erythrocyte sedimentation rate (66 mm in the first hour). Magnetic resonance imaging (MRI) of the spinal cord revealed a demyelinating LETM from C5 to D4 level (Figure 1). Meanwhile, an MRI of the sacroiliac joints revealed bilateral sacroiliitis. Brain and orbital MRIs were devoid of any lesions. Anti-aquaporin 4 (AQP-4) antibodies were tested by cell-based assay in serum and cerebrospinal fluid (CSF), and both were positive. CSF further revealed lymphocytic pleocytosis and increased intrathecal protein production. Visually evoked potential recordings were also normal. In addition, anti-myelin oligodendrocyte glycoprotein antibodies were negative. Anti-nuclear antibody (ANA), ANA-profile, autoimmune vasculitis profile (c-ANCA, p-ANCA), neurovirus panel (i.e., polymerase chain reaction for adenovirus, Epstein-Barr virus, herpes simplex viruses 1 and 2, human herpesviruses 6 and 7, cytomegalovirus, enteroviruses, varicella-zoster virus, Japanese encephalitis, and dengue virus), CSF-polymerase chain reaction for Mycobacterium tuberculosis, angiotensin-converting enzyme, anti-phospholipid, and anti-thyroid antibodies were negative. Anti-CCP-antibody and rheumatoid factor were also negative, including creatine phosphokinase level and serum vitamin B12. Moreover, serologies for hepatitis B, C, human immunodeficiency virus, and scrub typhus were negative. However, HLA-B27 assay was positive. The final diagnosis was AQP4-positive NMOSD associated with AS. He was placed on pulse intravenous methylprednisolone (1 g/day for 5 days). Consequently, his lower limb power improved remarkably. Cyclical rituximab therapy was initiated to prevent relapses. At 3-month follow-up, he had no residual neurological deficit except for persistence of paresthesias. Neuroimaging and visually evoked potential studies revealed no active or new lesions. After 6 months of therapy, a subjective and objective improvement was observed in disease severity based on the Ankylosing Spondylitis Disease Activity Score. Our patient satisfied the new Assessment of SpondyloArthritis International Society diagnostic/classification criteria for AS and the Wingerchuk criteria for NMOSD,(4,14) an association that has been rarely reported.(10,11) Amid the extra-articular complications of long-standing AS, neurological manifestations are considered infrequent.(15) However, subclinical neurological complications may be frequent in AS.(12) Common neurological manifestations result from bony (vertebral) ankylosis, subluxation of joints, ossification of anterior and posterior longitudinal ligaments, secondary spinal canal stenosis, bony (vertebral) fractures, and subsequent compressions over nerve radicles/roots/cauda equina, and inflammation-related (entrapment) peripheral neuropathies.(12,16,17) Acute transverse myelitis can occur as a subset of several primary demyelinating disorders of the CNS (i.e., multiple sclerosis, NMOSD, myelin oligodendrocyte glycoprotein antibody disease, and acute disseminated encephalomyelitis) and various systemic autoimmune connective tissue disorders (i.e., systemic lupus erythematosus, mixed connective tissue disease, Sjögren syndrome, inflammatory bowel disease, and neurosarcoidosis).(18) Acute transverse myelitis (short or long segment) is an infrequent extra-articular complication of AS.(18) It has been reported to evolve either as a distinct neurological complication of AS, or it may develop secondary to TNF-alpha-inhibitor therapy for the treatment of AS.(18,19) AS is a heritable inflammatory spondyloarthropathy that primarily affects the axial skeleton, which is mediated by T-cells; B-cells only play a minor role.(5) On the contrary, the key for the pathogenesis of NMOSD is the production of autoantibodies against AQP-4 channels expressed on astrocytes, leading to complement-mediated damage, with ensuing demyelination. Myelitis usually shows high signal intensity on the tbl2-weighted image and contrast enhancement in the spinal cord.(1-4) Despite the difference in molecular mechanisms, the diagnosis of these diseases in the same individual may not be coincidental. Recent evidence has shown T-cell-mediated inflammatory responses in cases of NMOSD.(20) In particular, Th17 and Th2-related cytokines are elevated in the CSF of NMO patients.(20) Environmental factors such as Escherichia coli have also been proven to aggravate autoimmunity in AS and NMOSD (however, body fluid cultures for Escherichia coli, performed in our patient, showed similar association, and they were found negative two times).(21,22) Although large-scale epidemiological studies investigating the underlying pathogenesis related to these diseases are lacking, studies have demonstrated an increased incidence of optic neuritis among patients with AS.(23) Systemic sclerosis and mixed and undifferentiated connective tissue diseases were excluded after expert opinions (from two board-certified rheumatologists and two dermatologists) because of the lack of suggestive clinical findings (e.g., absence of skin thickening, salt-and-pepper appearance, nail changes, Mauskopf facies, sclerodactyly, calcinosis cutis, Raynaud's phenomenon, other cutaneous manifestations, pulmonary arterial hypertension/interstitial lung disease, dysphagia, muscular pain/weakness renal impairments, absence of ANA, anti-centromere antibodies, anti-Scl-70, PM-Scl antibodies, anti-ds DNA, PCNA, CENP-B, anti-nucleosomes, anti-Smith, anti-U1-RNP, anti-Jo1, anti-Mi2, anti-Ro52, anti-La antibodies, and normal C3 and C4 complement levels) (The European League Against Rheumatism and the American College of Rheumatology classification criteria 2019).(24) Finally, our patient was treated with intravenous steroids followed by rituximab infusions, a monoclonal anti-CD20 antibody directed against B-cells. In particular, this patient clinically and radiologically responded to immunomodulatory drugs, which might support a possible common pathogenic basis of the two processes. TNF-alpha inhibitors are commonly used as novel therapeutics in AS; however, they can potentially result in serious complications, that is, secondary demyelinating disorders.(25) However, such inhibitors in this patient were not used. When used in cases of AS, they show satisfactory results.(25,26) Therefore, it was decided to treat him with rituximab only without adding any second immunomodulatory. Other possible therapeutic options include cyclophosphamide and mycophenolate mofetil, but they were not used because of their low efficacy-safety balance. Moreover, plasmapheresis was not available in our specific setting, despite solid evidence that early treatment with therapeutic strategy (5-7 courses) provides good long-term outcomes in patients with NMOSD.(27) Therefore, when dealing with a case of acute non-compressive myelopathy, history and clinical examination are important to determine the potential underlying etiology and identify an undermined systemic disorder with apparently unrelated non-specific features. Connective tissue disorders should always be considered as a differential diagnosis and be ruled out in all cases of either seropositive or seronegative NMOSD. A diagnosis of AS should be considered in relevant circumstances when dealing with a case of isolated seronegative LETM. Moreover, early diagnosis and treatment of AS are quintessential to prevent lifelong distressing disabilities. However, whether patients with AS have any extra predilection to develop NMOSD throughout their life requires further studies. CI - © 2022 Ghosh, Roy, León-Ruiz; Das; Dubey; Benito-León, licensee HBKU Press. FAU - Ghosh Md, Ritwik AU - Ghosh Md R AD - Department of General Medicine, Burdwan Medical College & Hospital, Burdwan, West Bengal, India E-mail: jbenitol67@gmail.com. FAU - Roy, Devlina AU - Roy D AD - Department of Neurology, Calcutta National Medical College & Hospital, Kolkata, West Bengal India. FAU - León-Ruiz, Moisés AU - León-Ruiz M AD - Section of Clinical Neurophysiology, Department of Neurology, University Hospital "La Paz," Madrid, Spain. FAU - Das, Shambaditya AU - Das S AD - Department of Neuromedicine, Bangur Institute of Neurosciences, Institute of Post Graduate Medical Education and Research & SSKM Hospital, Kolkata, West Bengal, India. FAU - Dubey, Souvik AU - Dubey S AD - Department of Neuromedicine, Bangur Institute of Neurosciences, Institute of Post Graduate Medical Education and Research & SSKM Hospital, Kolkata, West Bengal, India. FAU - Benito-León, Julián AU - Benito-León J LA - eng PT - Case Reports PT - Journal Article DEP - 20220707 PL - Qatar TA - Qatar Med J JT - Qatar medical journal JID - 8101648 PMC - PMC9272764 EDAT- 2022/07/23 06:00 MHDA- 2022/07/23 06:01 PMCR- 2022/07/07 CRDT- 2022/07/22 02:16 PHST- 2022/01/16 00:00 [received] PHST- 2022/03/03 00:00 [accepted] PHST- 2022/07/22 02:16 [entrez] PHST- 2022/07/23 06:00 [pubmed] PHST- 2022/07/23 06:01 [medline] PHST- 2022/07/07 00:00 [pmc-release] AID - qmj.2022.29 [pii] AID - 10.5339/qmj.2022.29 [doi] PST - epublish SO - Qatar Med J. 2022 Jul 7;2022(3):29. doi: 10.5339/qmj.2022.29. eCollection 2022. PMID- 39222296 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250325 IS - 2193-8253 (Print) IS - 2193-6536 (Electronic) IS - 2193-6536 (Linking) VI - 13 IP - 5 DP - 2024 Oct TI - Effectiveness of Nusinersen in Adolescents and Adults with Spinal Muscular Atrophy: Systematic Review and Meta-analysis. PG - 1483-1504 LID - 10.1007/s40120-024-00653-2 [doi] AB - INTRODUCTION: Nusinersen clinical trials have limited data on adolescents and adults with 5q-associated spinal muscular atrophy (SMA). We conducted a systematic literature review (SLR) and meta-analysis to assess effectiveness of nusinersen in adolescents and adults with SMA in clinical practice. METHODS: Our search included papers published 12/23/2016 through 07/01/2022 with ≥ 5 individuals ≥ 13 years of age and with ≥ 6 months' data on ≥ 1 selected motor function outcomes [Hammersmith Functional Motor Scale-Expanded (HFMSE), Revised Upper Limb Module (RULM), and Six-Minute Walk Test (6MWT)]. For meta-analysis, effect sizes were pooled using random-effects models. To understand treatment effects by disease severity, subgroup meta-analysis by SMA type and ambulatory status was conducted. RESULTS: Fourteen publications including 539 patients followed up to 24 months met inclusion criteria for the SLR. Patients were age 13-72 years and most (99%) had SMA Type II or III. Modest improvement or stability in motor function was consistently observed at the group level. Significant mean increases from baseline were observed in HFMSE [2.3 points (95% CI 1.3-3.3)] with 32.1% (21.7-44.6) of patients demonstrating a clinically meaningful increase (≥ 3 points) at 18 months. Significant increases in RULM were consistently found, with a mean increase of 1.1 points (0.7-1.4) and 38.3% (30.3-47.1) showing a clinically meaningful improvement (≥ 2 points) at 14 months. Among ambulatory patients, there was a significant increase in mean 6MWT distance of 25.0 m (8.9-41.2) with 50.9% (33.4-68.2) demonstrating a clinically meaningful improvement (≥ 30 m) at 14 months. The increases in HFMSE were greater for less severely affected patients, whereas more severely affected patients showed greater improvement in RULM. CONCLUSIONS: Findings provide consolidated evidence that nusinersen is effective in improving or stabilizing motor function in many adolescents and adults with a broad spectrum of SMA. CI - © 2024. The Author(s). FAU - Hagenacker, Tim AU - Hagenacker T AUID- ORCID: 0000-0002-3631-3450 AD - Department of Neurology Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Essen, Germany. tim.hagenacker@uk-essen.de. FAU - Maggi, Lorenzo AU - Maggi L AD - Neuroimmunology and Neuromuscular Diseases, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. FAU - Coratti, Giorgia AU - Coratti G AD - Department of Pediatric Neurology, Università Cattolica del Sacro Cuore, Rome, Italy. FAU - Youn, Bora AU - Youn B AD - Biogen, Cambridge, MA, USA. FAU - Raynaud, Stephanie AU - Raynaud S AD - Biogen, Cambridge, MA, USA. FAU - Paradis, Angela D AU - Paradis AD AUID- ORCID: 0000-0001-5698-8802 AD - Biogen, Cambridge, MA, USA. angela.paradis@biogen.com. AD - , 225 Binney Street, Cambridge, MA, 02142, USA. angela.paradis@biogen.com. FAU - Mercuri, Eugenio AU - Mercuri E AD - Department of Pediatric Neurology, Università Cattolica del Sacro Cuore, Rome, Italy. LA - eng PT - Journal Article DEP - 20240902 PL - New Zealand TA - Neurol Ther JT - Neurology and therapy JID - 101637818 PMC - PMC11393259 OAB - Motor neurons are specialized cells in the brain and spinal cord that control the function of muscles. People with spinal muscular atrophy (SMA) do not make enough survival motor neuron (SMN) protein, which motor neurons need to function. As a result, people with SMA experience decreased muscle function that gets worse over time. Nusinersen is a drug that increases the amount of SMN protein made in the brain and spinal cord. However, most clinical trials of nusinersen have been in infants and children with SMA. Less is known about the effects of nusinersen in teenagers and adults with SMA who may have less severe but still progressive forms of the disease. In this manuscript, we first conducted a thorough review and analysis of research published by investigators who treated teenagers and adults with nusinersen for up to 24 months. We then used an additional analysis, called a meta-analysis, that allowed us to combine the information from several articles, so that we could better understand whether nusinersen helped these patients. We looked at 3 tests that investigators used to see how nusinersen affected patients’ motor function. The Hammersmith Functional Motor Scale–Expanded (HFMSE) assesses upper and lower limb motor function; the Revised Upper Limb Module (RULM) evaluates upper limb function; and the Six-Minute Walk Test (6MWT) measures the maximum distance a person can walk in 6 minutes. Our study showed that nusinersen can improve motor function or prevent motor function from getting worse in many teenagers and adults with SMA. OABL- eng OTO - NOTNLM OT - Adolescents OT - Adults OT - Hammersmith Functional Motor Scale–Expanded OT - Motor function OT - Nusinersen OT - Revised Upper Limb Module OT - Six-Minute Walk Test OT - Spinal muscular atrophy COIS- Tim Hagenacker received grants/research support from Biogen, Novartis, and Roche, and honoraria or consulting fees from Biogen, Novartis, and Roche. Lorenzo Maggi received a grant from Biogen and honoraria for speaking, and consulting fees or compensation for congress participations from Amicus Therapeutics, Biogen, Janssen Pharmaceutics, Roche, and Sanofi Genzyme. Giorgia Coratti has received honoraria for speaking and compensation for congress participation from AveXis, Biogen, BioLogix, Novartis, and Roche. Bora Youn and Angela D. Paradis are employees of Biogen and may hold stock in the company. Stephanie Raynaud is a former employee of Biogen and may have held stock in the company at the time of the study. Eugenio Mercuri has served on advisory boards for SMA studies for AveXis, Biogen, Ionis, Novartis, and Roche; as Principal Investigator for ongoing Biogen/Ionis and Roche clinical trials; and has received funding from Famiglie SMA Italy, Italian Telethon, and SMA Europe. EDAT- 2024/09/02 12:42 MHDA- 2024/09/02 12:43 PMCR- 2024/09/02 CRDT- 2024/09/02 11:14 PHST- 2023/12/21 00:00 [received] PHST- 2024/07/29 00:00 [accepted] PHST- 2024/09/02 12:43 [medline] PHST- 2024/09/02 12:42 [pubmed] PHST- 2024/09/02 11:14 [entrez] PHST- 2024/09/02 00:00 [pmc-release] AID - 10.1007/s40120-024-00653-2 [pii] AID - 653 [pii] AID - 10.1007/s40120-024-00653-2 [doi] PST - ppublish SO - Neurol Ther. 2024 Oct;13(5):1483-1504. doi: 10.1007/s40120-024-00653-2. Epub 2024 Sep 2. PMID- 18617285 OWN - NLM STAT- MEDLINE DCOM- 20090501 LR - 20260518 IS - 0168-1605 (Print) IS - 0168-1605 (Linking) VI - 128 IP - 1 DP - 2008 Nov 30 TI - Quantitative risk assessment for Escherichia coli O157:H7 in frozen ground beef patties consumed by young children in French households. PG - 158-64 LID - 10.1016/j.ijfoodmicro.2008.05.040 [doi] AB - A quantitative risk assessment for Escherichia coli O157:H7 in frozen ground beef patties consumed by children under 10 years of age in French households was conducted by a national study group describing an outbreak which occurred in France in 2005. Our exposure assessment model incorporates results from French surveys on consumption frequency of ground beef patties, serving size and consumption preference, microbial destruction experiments and microbial counts on patties sampled from the industrial batch which were responsible for the outbreak. Two different exposure models were proposed, respectively for children under the age of 5 and for children between 5 and 10 years. For each of these two age groups, a single-hit dose-response model was proposed to describe the probability of hemolytic and uremic syndrome (HUS) as a function of the ingested dose. For each group, the single parameter of this model was estimated by Bayesian inference, using the results of the exposure assessment and the epidemiological data collected during the outbreak. Results show that children under 5 years of age are roughly 5 times more susceptible to the pathogen than children over 5 years. Exposure and dose-response models were used in a scenario analysis in order to validate the use of the model and to propose appropriate guidelines in order to prevent new outbreaks. The impact of the cooking preference was evaluated, showing that only a well-done cooking notably reduces the HUS risk, without annulling it. For each age group, a relation between the mean individual HUS risk per serving and the contamination level in a ground beef batch was proposed, as a tool to help French risk managers. FAU - Delignette-Muller, M L AU - Delignette-Muller ML AD - Laboratoire de Microbiologie Alimentaire et Prévisionnelle, Université de Lyon, Ecole Nationale Vétérinaire, 1 Avenue Bourgelat, F-69280, France. ml.delignette@vet-lyon.fr FAU - Cornu, M AU - Cornu M CN - AFSSA STEC study group LA - eng PT - Journal Article DEP - 20080605 PL - Netherlands TA - Int J Food Microbiol JT - International journal of food microbiology JID - 8412849 SB - IM MH - Age Factors MH - Animals MH - Bayes Theorem MH - Cattle MH - Child MH - Child, Preschool MH - Colony Count, Microbial MH - Cooking MH - Disease Outbreaks MH - Escherichia coli Infections/epidemiology/etiology/microbiology MH - Escherichia coli O157/*growth & development MH - Female MH - Food Contamination/*analysis MH - Food Handling/*methods MH - Foodborne Diseases/epidemiology MH - France/epidemiology MH - Frozen Foods/*microbiology MH - Hemolytic-Uremic Syndrome/epidemiology/etiology/microbiology MH - Humans MH - Male MH - Meat Products/*microbiology MH - *Risk Assessment FIR - Bemrah, N IR - Bemrah N FIR - Brugère, H IR - Brugère H FIR - Cerf, O IR - Cerf O FIR - Espié, E IR - Espié E FIR - Leclerc, V IR - Leclerc V FIR - Liverelli, V IR - Liverelli V FIR - Mahé, A IR - Mahé A FIR - Mariani-Kurkdjian, P IR - Mariani-Kurkdjian P FIR - Saunier, R IR - Saunier R FIR - Raynaud, S IR - Raynaud S FIR - Vernozy-Rozand, C IR - Vernozy-Rozand C EDAT- 2008/07/12 09:00 MHDA- 2009/05/02 09:00 CRDT- 2008/07/12 09:00 PHST- 2007/12/17 00:00 [received] PHST- 2008/05/28 00:00 [revised] PHST- 2008/05/30 00:00 [accepted] PHST- 2008/07/12 09:00 [pubmed] PHST- 2009/05/02 09:00 [medline] PHST- 2008/07/12 09:00 [entrez] AID - S0168-1605(08)00310-3 [pii] AID - 10.1016/j.ijfoodmicro.2008.05.040 [doi] PST - ppublish SO - Int J Food Microbiol. 2008 Nov 30;128(1):158-64. doi: 10.1016/j.ijfoodmicro.2008.05.040. Epub 2008 Jun 5. PMID- 36823505 OWN - NLM STAT- MEDLINE DCOM- 20230227 LR - 20230812 IS - 1600-0846 (Electronic) IS - 0909-752X (Print) IS - 0909-752X (Linking) VI - 29 IP - 2 DP - 2023 Feb TI - An evaluation of mechanical and biophysical skin parameters at different body locations. PG - e13292 LID - 10.1111/srt.13292 [doi] LID - e13292 AB - BACKGROUND: Skin is the largest organ in the body, representing an important interface to monitor health and disease. However, there is significant variation in skin properties for different ages, genders and body regions due to the differences in the structure and morphology of the skin tissues. This study aimed to evaluate the use of non-invasive tools to discriminate a range of mechanical and functional skin parameters from different skin sites. MATERIALS AND METHODS: A cohort of 15 healthy volunteers was recruited following appropriate informed consent. Four well-established CE-marked non-invasive techniques were used to measure four anatomical regions: palm, forearm, sole and lower lumbar L3, using a repeated measures design. Skin parameters included trans-epidermal water loss (TEWL), pH (acidity), erythema, stratum corneum hydration and stiffness and elasticity using Myoton Pro (skin and muscle probe). Differences between body locations for each parameter and the intra-rater reliability between days were evaluated by the same operator. RESULTS: The results indicate that parameters differed significantly between skin sites. For the Myoton skin probe, the sole recorded the highest stiffness value of 1006 N/m (SD ± 179), while the lower lumbar recorded the least value of 484 N/m (SD ± 160). The muscle indenter Myoton probe revealed the palm's highest value of 754 N/m (± 108), and the lower lumbar recorded the least value of 208 N/m (SD ± 44). TEWL values were lowest on the forearm, averaging 11 g/m2/h, and highest on the palm, averaging 41 g/m2/h. Similar skin hydration levels were recorded in three of the four sites, with the main difference being observed in the sole averaging 13 arbitrary units. Erythema values were characterised by a high degree of inter-subject variation, and no significant differences between sites or sides were observed. The Myoton Pro Skin showed excellent reliability (intra-class correlation coefficients > 0.70) for all sites with exception of one site right lower back; the Myoton pro muscle probes showed good to poor reliability (0.90-017), the corneometer showed excellent reliability (>0.75) among all the sites tested, and the TEWL showed Good to poor reliability (0.74-0.4) among sites. CONCLUSION: The study revealed that using non-invasive methods, the biophysical properties of skin can be mapped, and significant differences in the mechanical and functional properties of skin were observed. These parameters were reliably recorded between days, providing a basis for their use in assessing and monitoring changes in the skin during health and disease. CI - © 2023 The Authors. Skin Research and Technology published by John Wiley & Sons Ltd. FAU - John, Anto J U K AU - John AJUK AUID- ORCID: 0000-0003-3080-7393 AD - School of Health Sciences, University of Southampton, Southampton, UK. FAU - Galdo, Francesco Del AU - Galdo FD AUID- ORCID: 0000-0002-8528-2283 AD - Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Gush, Rodney AU - Gush R AUID- ORCID: 0000-0002-8624-3606 AD - Moor Instruments, Axminster, UK. FAU - Worsley, Peter R AU - Worsley PR AUID- ORCID: 0000-0003-0145-5042 AD - School of Health Sciences, University of Southampton, Southampton, UK. LA - eng GR - SRUK- SP1/ PT - Journal Article PL - England TA - Skin Res Technol JT - Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) JID - 9504453 RN - 059QF0KO0R (Water) SB - IM MH - Humans MH - Female MH - Male MH - *Skin Physiological Phenomena MH - Reproducibility of Results MH - *Skin/metabolism MH - Epidermis MH - Erythema MH - Water/metabolism MH - Water Loss, Insensible/physiology PMC - PMC10155800 OTO - NOTNLM OT - biophysical parameters OT - functional properties OT - hydration OT - mechanical loading OT - mechanical properties OT - sensitivity analysis COIS- The authors wish to confirm that there is no known conflict of interest associated with this publication, and there has been no significant financial support for this work that could have influenced its outcome. EDAT- 2023/02/25 06:00 MHDA- 2023/03/03 06:00 PMCR- 2023/02/17 CRDT- 2023/02/24 00:37 PHST- 2022/12/12 00:00 [received] PHST- 2023/01/31 00:00 [accepted] PHST- 2023/02/24 00:37 [entrez] PHST- 2023/02/25 06:00 [pubmed] PHST- 2023/03/03 06:00 [medline] PHST- 2023/02/17 00:00 [pmc-release] AID - SRT13292 [pii] AID - 10.1111/srt.13292 [doi] PST - ppublish SO - Skin Res Technol. 2023 Feb;29(2):e13292. doi: 10.1111/srt.13292. PMID- 33562846 OWN - NLM STAT- MEDLINE DCOM- 20210630 LR - 20210630 IS - 2072-6651 (Electronic) IS - 2072-6651 (Linking) VI - 13 IP - 2 DP - 2021 Feb 5 TI - Therapeutic Use of Botulinum Neurotoxins in Dermatology: Systematic Review. LID - 10.3390/toxins13020120 [doi] LID - 120 AB - Botulinum toxin is a superfamily of neurotoxins produced by the bacterium Clostridium Botulinum with well-established efficacy and safety profile in focal idiopathic hyperhidrosis. Recently, botulinum toxins have also been used in many other skin diseases, in off label regimen. The objective of this manuscript is to review and analyze the main therapeutic applications of botulinum toxins in skin diseases. A systematic review of the published data was conducted, following Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Botulinum toxins present several label and off-label indications of interest for dermatologists. The best-reported evidence concerns focal idiopathic hyperhidrosis, Raynaud phenomenon, suppurative hidradenitis, Hailey-Hailey disease, epidermolysis bullosa simplex Weber-Cockayne type, Darier's disease, pachyonychia congenita, aquagenic keratoderma, alopecia, psoriasis, notalgia paresthetica, facial erythema and flushing, and oily skin. Further clinical trials are still needed to better understand the real efficacy and safety of these applications and to standardize injection and doses protocols for off label applications. FAU - Martina, Emanuela AU - Martina E AD - Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60020 Ancona, Italy. FAU - Diotallevi, Federico AU - Diotallevi F AD - Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60020 Ancona, Italy. FAU - Radi, Giulia AU - Radi G AD - Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60020 Ancona, Italy. FAU - Campanati, Anna AU - Campanati A AD - Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60020 Ancona, Italy. FAU - Offidani, Annamaria AU - Offidani A AD - Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60020 Ancona, Italy. LA - eng PT - Journal Article PT - Systematic Review DEP - 20210205 PL - Switzerland TA - Toxins (Basel) JT - Toxins JID - 101530765 RN - 0 (Dermatologic Agents) RN - EC 3.4.24.69 (Botulinum Toxins) SB - IM MH - Botulinum Toxins/*administration & dosage/adverse effects MH - Dermatologic Agents/*administration & dosage/adverse effects MH - *Dermatology MH - Female MH - Humans MH - Male MH - Off-Label Use MH - Patient Safety MH - Risk Assessment MH - Risk Factors MH - Skin Diseases/*drug therapy MH - Treatment Outcome PMC - PMC7915854 OTO - NOTNLM OT - Darier’s disease OT - Hailey–Hailey disease OT - Raynaud phenomenon OT - alopecia OT - aquagenic keratoderma OT - botulinum toxin OT - dermatology OT - epidermolysis bullosa simplex Weber–Cockayne type OT - facial erythema and flushing OT - focal idiopathic hyperhidrosis OT - notalgia paresthetica OT - oily skin OT - pachyonychia congenita OT - psoriasis OT - skin diseases OT - suppurative hidradenitis COIS- The authors declare no conflict of interest. EDAT- 2021/02/11 06:00 MHDA- 2021/07/01 06:00 PMCR- 2021/02/05 CRDT- 2021/02/10 01:03 PHST- 2020/12/28 00:00 [received] PHST- 2021/01/19 00:00 [revised] PHST- 2021/01/22 00:00 [accepted] PHST- 2021/02/10 01:03 [entrez] PHST- 2021/02/11 06:00 [pubmed] PHST- 2021/07/01 06:00 [medline] PHST- 2021/02/05 00:00 [pmc-release] AID - toxins13020120 [pii] AID - toxins-13-00120 [pii] AID - 10.3390/toxins13020120 [doi] PST - epublish SO - Toxins (Basel). 2021 Feb 5;13(2):120. doi: 10.3390/toxins13020120. PMID- 26677987 OWN - NLM STAT- MEDLINE DCOM- 20170717 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 83 IP - 3 DP - 2016 May TI - Initial presentation and outcome of pediatric-onset mixed connective tissue disease: A French multicenter retrospective study. PG - 369-71 LID - S1297-319X(15)00252-3 [pii] LID - 10.1016/j.jbspin.2015.05.013 [doi] FAU - Tellier, Stéphanie AU - Tellier S AD - Department of Pediatric Nephrology-Internal Medicine, hôpital Purpan, 330, avenue Grande-Bretagne, 31400 Toulouse, France; Centre de référence des maladies rénales rares du Sud-Ouest (SORARE), 31400 Toulouse, France; University Toulouse III - Paul-Sabatier, 31400 Toulouse, France. Electronic address: tellier.s@chu-toulouse.fr. FAU - Bader-Meunier, Brigitte AU - Bader-Meunier B AD - Department of Pediatric Immunology, Hematology and Rheumatology, hôpital Necker, 75015 Paris, France. FAU - Quartier, Pierre AU - Quartier P AD - Department of Pediatric Immunology, Hematology and Rheumatology, hôpital Necker, 75015 Paris, France. FAU - Belot, Alexandre AU - Belot A AD - Department of Pediatric Nephrology and Rheumatology, hôpitaux Est, 69000 Lyon, France. FAU - Deslandre, Chantal AU - Deslandre C AD - Department of Rheumatology, hôpital Cochin, 75014 Paris, France. FAU - Koné-Paut, Isabelle AU - Koné-Paut I AD - Department of Pediatrics, hôpital de Bicêtre, 92270 Le Kremlin-Bicêtre, France. FAU - Tiriau, Soizic AU - Tiriau S AD - Department of Pediatrics, hôpital Mère-Enfants, 44000 Nantes, France. FAU - Jurquet, Anne-Laure AU - Jurquet AL AD - Department of Pediatrics, hôpital Nord, 13000 Marseille, France. FAU - Rosellini, David AU - Rosellini D AD - Department of Pediatrics, centre hospitalier, 66000 Perpignan, France. FAU - Dheu-Bentz, Céline AU - Dheu-Bentz C AD - Department of Pediatrics, hôpital Le Parc, 68000 Colmar, France. FAU - Mestrallet, Guillaume AU - Mestrallet G AD - Department of Pediatrics, hôpital Nord-Ouest, 69655 Villefranche-sur-Saône, France. FAU - Decramer, Stéphane AU - Decramer S AD - Department of Pediatric Nephrology-Internal Medicine, hôpital Purpan, 330, avenue Grande-Bretagne, 31400 Toulouse, France; Centre de référence des maladies rénales rares du Sud-Ouest (SORARE), 31400 Toulouse, France; University Toulouse III - Paul-Sabatier, 31400 Toulouse, France. LA - eng PT - Letter PT - Multicenter Study DEP - 20151208 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Adolescent MH - Age Factors MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Male MH - Mixed Connective Tissue Disease/complications/*diagnosis/epidemiology MH - Retrospective Studies OTO - NOTNLM OT - Anti-RNP antibodies OT - Child OT - Connective tissue disease OT - Raynaud phenomena OT - Sharp syndrome EDAT- 2015/12/19 06:00 MHDA- 2017/07/18 06:00 CRDT- 2015/12/19 06:00 PHST- 2015/02/04 00:00 [received] PHST- 2015/05/05 00:00 [accepted] PHST- 2015/12/19 06:00 [entrez] PHST- 2015/12/19 06:00 [pubmed] PHST- 2017/07/18 06:00 [medline] AID - S1297-319X(15)00252-3 [pii] AID - 10.1016/j.jbspin.2015.05.013 [doi] PST - ppublish SO - Joint Bone Spine. 2016 May;83(3):369-71. doi: 10.1016/j.jbspin.2015.05.013. Epub 2015 Dec 8. PMID- 41015908 OWN - NLM STAT- MEDLINE DCOM- 20250928 LR - 20251001 IS - 1753-4666 (Electronic) IS - 1753-4658 (Print) IS - 1753-4658 (Linking) VI - 19 DP - 2025 Jan-Dec TI - How do doctors and patients communicate about the treatment of systemic sclerosis-associated interstitial lung disease? A plain language summary of publication. PG - 17534666251371814 LID - 10.1177/17534666251371814 [doi] LID - 17534666251371814 AB - SummaryWhat is this summary about?Systemic sclerosis (SSc) is a condition that affects the immune system (the body's natural defence system) and causes the skin to harden and thicken in large patches. Research shows that 30% to 90% of people with SSc also have interstitial lung disease (ILD), a condition that causes inflammation and scarring of the lungs. When people have SSc and ILD, it is known as SSc-associated ILD or SSc-ILD. The authors of this plain language summary of publication (PLS-P) reviewed different articles to find out what the key issues were in the way doctors and patients with SSc-ILD communicate with each other.What were the results?The key messages from the studies were:Most patients felt uneasy when they were diagnosed with SSc-ILDGood communication between doctors and patients at the first visit is crucial as it sets the tone for future relationshipsBoth doctors and patients avoid talking about how SSc-ILD symptoms may get worse (prognosis) or the subject of death. Patients should be encouraged to ask questions to address important and personal topics that would not be talked about otherwisePatients may feel intimidated by a doctor, which could interfere with communicationDoctors must be able to listen and show empathy to build a relationship with patients and be aware that different communication styles may suit a patient during different stages in their journeyDoctors should avoid using a lot of technical terms. Patients felt metaphors helped them understand their condition betterPatients have different awareness, thoughts, and feelings about SSc-ILD than doctors. If doctors understand this, it may improve the communication between doctors and patientsWays to close the gap between the way doctors and patients communicate include patients having the opportunity to access:Self-learning and patient organizationsPeer-mentoring (patients mentoring other patients)Information technologyShared decision-making, where the doctor and patient work together to come to a decision about treatment and careWhat do the results mean?The best way to improve the feelings patients have when they are diagnosed with SSc, including SSc-ILD, is to improve the quality of the communication between doctors and patients. The quality of the first meeting between a doctor and patient sets the tone for future checkups, especially if the doctor can listen, show empathy, and allow the patient to ask questions. Improving the patient's knowledge about SSc-ILD, for example by using websites, reading printed materials, or taking part in peer-mentoring schemes, may also contribute to a better conversation. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Saito, Aiko AU - Saito A AD - Nippon Boehringer Ingelheim Co. Ltd., Tokyo, Japan. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma and Raynaud's UK, London, UK. AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. FAU - Galetti, Ilaria AU - Galetti I AD - Federation of European Scleroderma Associations aisbl, Saint-Maur, Belgium. AD - Gruppo Italiano per la Lotta alla Sclerodermia, Milan, Italy. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Division of Medicine, University College London, London, UK. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Scleroderma Research Program and Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA. LA - eng PT - Journal Article PT - Review DEP - 20250928 PL - England TA - Ther Adv Respir Dis JT - Therapeutic advances in respiratory disease JID - 101316317 SB - IM MH - Humans MH - *Lung Diseases, Interstitial/therapy/etiology/diagnosis/physiopathology/psychology MH - *Scleroderma, Systemic/complications/therapy/diagnosis/psychology/physiopathology MH - *Physician-Patient Relations MH - *Communication MH - Health Knowledge, Attitudes, Practice MH - Plain Language Summaries PMC - PMC12477389 OTO - NOTNLM OT - interstitial lung disease OT - physician–patient communication OT - plain language summary of publications OT - shared decision-making OT - systemic sclerosis COIS- The authors received no direct compensation related to the development of the manuscript. M.K. has received consulting fees, speaking fees, and research grants from Argenx, Asahi Kasei, AstraZeneca, Boehringer Ingelheim, Chugai, GlaxoSmithKline, Janssen, Kissei, MBL, Mitsubishi Tanabe, Mochida, Nippon Shinyaku, Ono Pharmaceuticals, and Taisho. A.S. is an employee of Nippon Boehringer Ingelheim Co. Ltd. S.F. and I.G. have nothing to disclose. C.P.D. reports consulting fees from Roche, Boehringer Ingelheim, GlaxoSmithKline, Horizon Therapeutics, CSL Behring; honoraria from Boehringer Ingelheim and Janssen; and grants from Horizon Therapeutics, GlaxoSmithKline, and Servier. D.K. reports consultancies with Amgen, Argnx, Astra Zeneca BMS, Boehringer Ingelheim, Genentech/Roche, GSK, Mitsubishi Tanabi, Merck, Novartis, and Zura Bio. EDAT- 2025/09/28 15:30 MHDA- 2025/09/28 15:31 PMCR- 2025/09/28 CRDT- 2025/09/28 07:33 PHST- 2025/09/28 15:31 [medline] PHST- 2025/09/28 15:30 [pubmed] PHST- 2025/09/28 07:33 [entrez] PHST- 2025/09/28 00:00 [pmc-release] AID - 10.1177_17534666251371814 [pii] AID - 10.1177/17534666251371814 [doi] PST - ppublish SO - Ther Adv Respir Dis. 2025 Jan-Dec;19:17534666251371814. doi: 10.1177/17534666251371814. Epub 2025 Sep 28. PMID- 33448231 OWN - NLM STAT- MEDLINE DCOM- 20210928 LR - 20240812 IS - 1941-7632 (Electronic) IS - 1941-7640 (Print) IS - 1941-7640 (Linking) VI - 14 IP - 2 DP - 2021 Feb TI - 2021 ACC/AHA/SVM/ACP Advanced Training Statement on Vascular Medicine (Revision of the 2004 ACC/ACP/SCAI/SVMB/SVS Clinical Competence Statement on Vascular Medicine and Catheter-Based Peripheral Vascular Interventions). PG - e000079 LID - 10.1161/HCV.0000000000000079 [doi] AB - Supplemental Digital Content is available in the text. FAU - Creager, Mark A AU - Creager MA FAU - Hamburg, Naomi M AU - Hamburg NM FAU - Calligaro, Keith D AU - Calligaro KD AD - Society for Vascular Surgery representative. FAU - Casanegra, Ana I AU - Casanegra AI AD - Society for Vascular Medicine representative. FAU - Freeman, Rosario AU - Freeman R FAU - Gordon, Phyllis A AU - Gordon PA AD - Society for Vascular Nursing representative. FAU - Gornik, Heather L AU - Gornik HL AD - Society for Vascular Medicine representative. FAU - Kim, Esther S H AU - Kim ESH AD - American Heart Association representative. FAU - Leeper, Nicholas J AU - Leeper NJ AD - American Heart Association representative. FAU - Merli, Geno J AU - Merli GJ AD - American College of Physicians representative. FAU - Niazi, Khusrow AU - Niazi K FAU - Olin, Jeffrey W AU - Olin JW FAU - Quiroz, Rene AU - Quiroz R FAU - Rrapo Kaso, Elona AU - Rrapo Kaso E FAU - Wasan, Suman AU - Wasan S AD - Society for Vascular Medicine representative. FAU - Waxler, Andrew R AU - Waxler AR FAU - White, Christopher J AU - White CJ AD - Society for Cardiovascular Angiography and Interventions representative. FAU - White Solaru, Khendi AU - White Solaru K AD - American Heart Association representative. FAU - Williams, Marlene S AU - Williams MS AD - Association of Black Cardiologists representative. LA - eng PT - Journal Article DEP - 20210115 PL - United States TA - Circ Cardiovasc Interv JT - Circulation. Cardiovascular interventions JID - 101499602 SB - IM EIN - Circ Cardiovasc Interv. 2021 Feb;14(2):e000082. doi: 10.1161/HCV.0000000000000082. PMID: 33591823 MH - *Cardiology/education MH - Catheters MH - *Clinical Competence MH - Humans MH - Societies, Medical MH - Support Vector Machine PMC - PMC8221116 OTO - NOTNLM OT - AHA Scientific Statements OT - Raynaud phenomenon OT - aortic diseases OT - cerebrovascular disease OT - clinical competence OT - fellowship training OT - lymphedema OT - peripheral artery disease OT - vascular medicine OT - venous insufficiency OT - venous thromboembolism EDAT- 2021/01/16 06:00 MHDA- 2021/09/29 06:00 PMCR- 2021/06/23 CRDT- 2021/01/15 08:42 PHST- 2021/01/16 06:00 [pubmed] PHST- 2021/09/29 06:00 [medline] PHST- 2021/01/15 08:42 [entrez] PHST- 2021/06/23 00:00 [pmc-release] AID - 10.1161/HCV.0000000000000079 [doi] PST - ppublish SO - Circ Cardiovasc Interv. 2021 Feb;14(2):e000079. doi: 10.1161/HCV.0000000000000079. Epub 2021 Jan 15. PMID- 40592552 OWN - NLM STAT- In-Process LR - 20251001 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 52 IP - 10 DP - 2025 Oct 1 TI - The Risk and Time Course for Autoimmune Rheumatic Disease Development in Patients With Raynaud Phenomenon. PG - 1056-1058 LID - 10.3899/jrheum.2024-0954 [doi] FAU - Teaw, Shannon AU - Teaw S AUID- ORCID: 0000-0001-7409-8731 AD - Department of Internal Medicine, University of Washington, Seattle, Washington. FAU - Shojaee, Abbas AU - Shojaee A AUID- ORCID: 0000-0003-1803-3284 AD - Independent researcher, New Haven, Connecticut. FAU - Carnaru, Miruna AU - Carnaru M AUID- ORCID: 0000-0002-2973-7247 AD - University of Connecticut School of Medicine, Farmington, Connecticut. AD - Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, Yale School of Medicine, New Haven, Connecticut, USA. FAU - Hinchcliff, Monique AU - Hinchcliff M AUID- ORCID: 0000-0002-8652-9890 AD - Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, Yale School of Medicine, New Haven, Connecticut, USA. Monique.hinchcliff@yale.edu. LA - eng PT - Letter DEP - 20251001 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM EDAT- 2025/07/02 00:27 MHDA- 2025/07/02 00:27 CRDT- 2025/07/01 21:02 PHST- 2025/07/02 00:27 [pubmed] PHST- 2025/07/02 00:27 [medline] PHST- 2025/07/01 21:02 [entrez] AID - jrheum.2024-0954 [pii] AID - 10.3899/jrheum.2024-0954 [doi] PST - epublish SO - J Rheumatol. 2025 Oct 1;52(10):1056-1058. doi: 10.3899/jrheum.2024-0954. PMID- 40878837 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20250915 LR - 20250915 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 7 IP - 8 DP - 2025 Aug TI - Effects of Audio-Guided Imagery on Raynaud Phenomenon in Connective Tissue Disease. PG - e70074 LID - 10.1002/acr2.70074 [doi] LID - e70074 AB - OBJECTIVE: To assess the physical, emotional, and mental impact of regular audio-guided imagery (AGI) in patients with connective tissue disease (CTD)-associated Raynaud phenomenon (RP). METHODS: Sixteen adult patients with CTD-associated RP were enrolled in an open-label, single-arm, eight-week intervention with daily AGI at a single center. Patients completed surveys, including assessment of RP severity, depression/anxiety, and quality of life at baseline and the end of eight weeks. RESULTS: Study participants demonstrated significant improvement in all patient reported outcomes, except Generalized Anxiety Disorder 7, which showed improvement but was not statistically significant. This eight-week intervention shows that patients with CTD-associated RP had less severe RP episodes, as well as improved quality of life and depressive symptoms. CONCLUSION: AGI significantly improved several domains of health, including physical, emotional, and mental, in patients with CTD-associated RP who otherwise more commonly struggle with more RP attacks, lower quality of life, and depression. AGI is a low-cost, low-risk, and convenient therapy for CTD-associated RP. A larger study studying the effects of this intervention is indicated. CI - © 2025 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Padilla, Cristina AU - Padilla C AUID- ORCID: 0000-0001-9548-5263 AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Katikineni, Veena AU - Katikineni V AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Park, Yongseok AU - Park Y AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Freno, Leigh AU - Freno L AD - University of Pittsburgh Medical Center Arthritis and Autoimmunity Center, Pittsburgh, Pennsylvania. FAU - Laffoon, Maureen AU - Laffoon M AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Reichbaum, Lee AU - Reichbaum L AD - University of Pittsburgh and University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, Pennsylvania. FAU - Lafyatis, Robert AU - Lafyatis R AD - University of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Domsic, Robyn AU - Domsic R AUID- ORCID: 0000-0002-2765-0922 AD - University of Pittsburgh, Pittsburgh, Pennsylvania. LA - eng GR - P50 AR060780/AR/NIAMS NIH HHS/United States PT - Journal Article PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC12305345 EDAT- 2025/09/01 16:45 MHDA- 2025/09/01 16:46 PMCR- 2025/07/29 CRDT- 2025/08/29 06:33 PHST- 2025/05/02 00:00 [revised] PHST- 2025/01/06 00:00 [received] PHST- 2025/05/12 00:00 [accepted] PHST- 2025/09/01 16:46 [medline] PHST- 2025/09/01 16:45 [pubmed] PHST- 2025/08/29 06:33 [entrez] PHST- 2025/07/29 00:00 [pmc-release] AID - ACR270074 [pii] AID - 10.1002/acr2.70074 [doi] PST - ppublish SO - ACR Open Rheumatol. 2025 Aug;7(8):e70074. doi: 10.1002/acr2.70074. PMID- 29484025 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 1857-9655 (Print) IS - 1857-9655 (Electronic) IS - 1857-9655 (Linking) VI - 6 IP - 1 DP - 2018 Jan 25 TI - Acrocyanosis - A Symptom with Many Facettes. PG - 208-212 LID - 10.3889/oamjms.2018.035 [doi] AB - Acrocyanosis is an uncommon complaint belonging to the acro-syndromes. It typically presents with coolness and bluish discolourations of hands, feet, ears, nose, lips and nipple. The most frequently affected parts of the body are the hands. This review discusses physical factors, vascular disorders, infectious diseases, haematological disorders, solid tumours genetic disorders, drugs, eating disorders, and spinal disease presenting as or leading to acrocyanosis. FAU - Wollina, Uwe AU - Wollina U AD - Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany. FAU - Koch, André AU - Koch A AD - Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany. FAU - Langner, Dana AU - Langner D AD - Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany. FAU - Hansel, Gesina AU - Hansel G AD - Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany. FAU - Heinig, Birgit AU - Heinig B AD - Städtisches Klinikum Dresden - Center of Physical and Rehabilitative Medicine, Dresden, Germany. FAU - Lotti, Torello AU - Lotti T AD - University G. Marconi of Rome - Dermatology and Venereology, Rome, Italy. FAU - Tchernev, Georgi AU - Tchernev G AD - Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior, Sofia, Bulgaria. AD - Onkoderma, Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria. LA - eng PT - Journal Article PT - Review DEP - 20180110 PL - North Macedonia TA - Open Access Maced J Med Sci JT - Open access Macedonian journal of medical sciences JID - 101662294 PMC - PMC5816301 OTO - NOTNLM OT - Acrocyanosis OT - Adverse drug reactions OT - Connective tissue diseases OT - Raynaud syndrome OT - Tumors OT - hands and feet OT - vascular disorders EDAT- 2018/02/28 06:00 MHDA- 2018/02/28 06:01 PMCR- 2018/01/10 CRDT- 2018/02/28 06:00 PHST- 2017/07/28 00:00 [received] PHST- 2017/10/27 00:00 [revised] PHST- 2017/10/30 00:00 [accepted] PHST- 2018/02/28 06:00 [entrez] PHST- 2018/02/28 06:00 [pubmed] PHST- 2018/02/28 06:01 [medline] PHST- 2018/01/10 00:00 [pmc-release] AID - OAMJMS-6-208 [pii] AID - 10.3889/oamjms.2018.035 [doi] PST - epublish SO - Open Access Maced J Med Sci. 2018 Jan 10;6(1):208-212. doi: 10.3889/oamjms.2018.035. eCollection 2018 Jan 25. PMID- 39941292 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250215 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 15 IP - 3 DP - 2025 Feb 4 TI - The Role of Nailfold Videocapillaroscopy in the Diagnosis and Monitoring of Interstitial Lung Disease Associated with Rheumatic Autoimmune Diseases. LID - 10.3390/diagnostics15030362 [doi] LID - 362 AB - Interstitial lung disease (ILD) is a severe complication of certain connective tissue diseases (CTDs) such as systemic sclerosis (SSc), mixed connective tissue disease (MCTD), idiopathic inflammatory myopathies (IIM), rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), and it is associated with nailfold videocapillaroscopy (NVC) changes and increased morbidity and mortality rates. Early diagnosis is crucial in order to prevent the progression of ILD, prevent respiratory failure and enhance the patient's overall quality of life. The most common paraclinical investigations are high-resolution computed tomography (HRCT) and functional respiratory tests such as forced vital capacity (FVC) and the diffusing capacity of the lungs for carbon monoxide (DLCO). The most frequent CTD associated with both ILD and NVC changes is systemic sclerosis. The "late" scleroderma pattern was the most common abnormality identified in NVC results in SSc patients. Other autoimmune diseases were also correlated with ILD and NVC changes, especially when the Raynaud phenomenon was present. Low capillary density was associated with the presence and severity of ILD and a reduction in FVC and DLCO. NVC can also differentiate the capillaroscopic changes in some particular types of ILD, such as the usual interstitial pneumonia (UIP) pattern from the non-specific interstitial pneumonia (NSIP) pattern. Nevertheless, further extensive research is necessary in order to establish the diagnostic value of NVC in CTD-ILD in clinical practice. FAU - Anghel, Daniela AU - Anghel D AD - Department of Internal Medicine 2, Central Military Emergency University Hospital 'Dr. Carol Davila', 010825 Bucharest, Romania. AD - Department of Medico-Surgical and Prophylactic Disciplines, 'Titu Maiorescu' University, 031593 Bucharest, Romania. FAU - Prioteasă, Oana-Georgiana AU - Prioteasă OG AUID- ORCID: 0000-0001-8070-8495 AD - Department of Internal Medicine 2, Central Military Emergency University Hospital 'Dr. Carol Davila', 010825 Bucharest, Romania. AD - Department of Internal Medicine and Rheumatology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. FAU - Nicolau, Iulia-Nadine AU - Nicolau IN AD - Department of Internal Medicine 2, Central Military Emergency University Hospital 'Dr. Carol Davila', 010825 Bucharest, Romania. FAU - Bucurică, Săndica AU - Bucurică S AUID- ORCID: 0000-0002-8787-3829 AD - Department of Gastroenterology, Central Military Emergency University Hospital 'Dr. Carol Davila', 010825 Bucharest, Romania. AD - Department of Gastroenterology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. FAU - Belinski, Daniela-Opriș AU - Belinski DO AUID- ORCID: 0000-0003-3883-9672 AD - Department of Internal Medicine and Rheumatology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. AD - Department of Rheumatology, 'Sf. Maria' Clinical Hospital, 011172 Bucharest, Romania. FAU - Popescu, Gilda-Georgeta AU - Popescu GG AD - Department of Medico-Surgical and Prophylactic Disciplines, 'Titu Maiorescu' University, 031593 Bucharest, Romania. AD - Tuberculosis Control Subcomission, Romanian Ministry of Health, 030167 Bucharest, Romania. FAU - Ghinescu, Minerva Claudia AU - Ghinescu MC AD - Department of Medico-Surgical and Prophylactic Disciplines, 'Titu Maiorescu' University, 031593 Bucharest, Romania. FAU - Bobircă, Anca AU - Bobircă A AUID- ORCID: 0000-0002-6662-7354 AD - Department of Internal Medicine and Rheumatology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. AD - Department of Internal Medicine and Rheumatology, "Dr. Ion Cantacuzino" Clinical Hospital, 011437 Bucharest, Romania. FAU - Groșeanu, Maria-Laura AU - Groșeanu ML AD - Department of Internal Medicine and Rheumatology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. AD - Department of Rheumatology, 'Sf. Maria' Clinical Hospital, 011172 Bucharest, Romania. FAU - Bojincă, Violeta-Claudia AU - Bojincă VC AD - Department of Internal Medicine and Rheumatology, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania. AD - Department of Internal Medicine, 'Sf. Maria' Clinical Hospital, 011172 Bucharest, Romania. LA - eng PT - Journal Article PT - Review DEP - 20250204 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC11816486 OTO - NOTNLM OT - Raynaud phenomenon OT - autoimmune diseases OT - interstitial lung disease OT - nailfold capillaroscopy COIS- The authors declare no conflicts of interest. EDAT- 2025/02/13 11:00 MHDA- 2025/02/13 11:01 PMCR- 2025/02/04 CRDT- 2025/02/13 01:07 PHST- 2024/11/18 00:00 [received] PHST- 2025/01/19 00:00 [revised] PHST- 2025/01/21 00:00 [accepted] PHST- 2025/02/13 11:01 [medline] PHST- 2025/02/13 11:00 [pubmed] PHST- 2025/02/13 01:07 [entrez] PHST- 2025/02/04 00:00 [pmc-release] AID - diagnostics15030362 [pii] AID - diagnostics-15-00362 [pii] AID - 10.3390/diagnostics15030362 [doi] PST - epublish SO - Diagnostics (Basel). 2025 Feb 4;15(3):362. doi: 10.3390/diagnostics15030362. PMID- 33250266 OWN - NLM STAT- MEDLINE DCOM- 20210920 LR - 20210920 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 77 IP - 7 DP - 2021 Feb 23 TI - 2021 ACC/AHA/SVM/ACP Advanced Training Statement on Vascular Medicine (Revision of the 2004 ACC/ACP/SCAI/SVMB/SVS Clinical Competence Statement on Vascular Medicine and Catheter-Based Peripheral Vascular Interventions): A Report of the ACC Competency Management Committee. PG - 998-1020 LID - S0735-1097(20)37310-1 [pii] LID - 10.1016/j.jacc.2020.09.579 [doi] CN - Writing Committee Members FAU - Creager, Mark A AU - Creager MA FAU - Hamburg, Naomi M AU - Hamburg NM FAU - Calligaro, Keith D AU - Calligaro KD FAU - Casanegra, Ana I AU - Casanegra AI FAU - Freeman, Rosario AU - Freeman R FAU - Gordon, Phyllis A AU - Gordon PA FAU - Gornik, Heather L AU - Gornik HL FAU - Kim, Esther S H AU - Kim ESH FAU - Leeper, Nicholas J AU - Leeper NJ FAU - Merli, Geno J AU - Merli GJ FAU - Niazi, Khusrow AU - Niazi K FAU - Olin, Jeffrey W AU - Olin JW FAU - Quiroz, Rene AU - Quiroz R FAU - Rrapo Kaso, Elona AU - Rrapo Kaso E FAU - Wasan, Suman AU - Wasan S FAU - Waxler, Andrew R AU - Waxler AR FAU - White, Christopher J AU - White CJ FAU - White Solaru, Khendi AU - White Solaru K FAU - Williams, Marlene S AU - Williams MS LA - eng PT - Journal Article DEP - 20201126 PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 SB - IM MH - Advisory Committees MH - Cardiology/standards MH - Clinical Competence/*standards MH - Humans MH - *Peripheral Vascular Diseases/diagnosis/therapy MH - Societies, Medical MH - United States OTO - NOTNLM OT - ACC/AHA/SVM/ACP Training Statement OT - Raynaud phenomenon OT - aortic diseases OT - cerebrovascular disease OT - clinical competence OT - fellowship training OT - lymphedema OT - peripheral artery disease OT - vascular medicine OT - venous insufficiency OT - venous thromboembolism EDAT- 2020/12/01 06:00 MHDA- 2021/09/21 06:00 CRDT- 2020/11/30 05:32 PHST- 2020/12/01 06:00 [pubmed] PHST- 2021/09/21 06:00 [medline] PHST- 2020/11/30 05:32 [entrez] AID - S0735-1097(20)37310-1 [pii] AID - 10.1016/j.jacc.2020.09.579 [doi] PST - ppublish SO - J Am Coll Cardiol. 2021 Feb 23;77(7):998-1020. doi: 10.1016/j.jacc.2020.09.579. Epub 2020 Nov 26. PMID- 25065694 OWN - NLM STAT- MEDLINE DCOM- 20150707 LR - 20141121 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 171 IP - 5 DP - 2014 Nov TI - IgG4-related skin disease. PG - 959-67 LID - 10.1111/bjd.13296 [doi] AB - IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4(+) plasma cells. IgG4-RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1-3 are induced by direct infiltration of IgG4(+) plasma cells, while the other types (4-7) are caused by secondary mechanisms. IgG4-related skin disease is defined as IgG4(+) plasma-cell-infiltrating skin lesions that form plaques, nodules or tumours (types 1-3), but may manifest secondary lesions caused by IgG4(+) plasma cells and/or IgG4 (types 4-7). CI - © 2014 British Association of Dermatologists. FAU - Tokura, Y AU - Tokura Y AD - Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192, Japan. FAU - Yagi, H AU - Yagi H FAU - Yanaguchi, H AU - Yanaguchi H FAU - Majima, Y AU - Majima Y FAU - Kasuya, A AU - Kasuya A FAU - Ito, T AU - Ito T FAU - Maekawa, M AU - Maekawa M FAU - Hashizume, H AU - Hashizume H LA - eng PT - Journal Article PT - Review DEP - 20141020 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Immunoglobulin G) SB - IM CIN - Br J Dermatol. 2014 Nov;171(5):929. doi: 10.1111/bjd.13394. PMID: 25409991 MH - Angiolymphoid Hyperplasia with Eosinophilia/immunology MH - Autoimmune Diseases/classification/*immunology MH - Erythema/immunology MH - Fingers/blood supply MH - Humans MH - Immunoglobulin G/*immunology/metabolism MH - Ischemia/immunology MH - Mikulicz' Disease/immunology MH - Plasma Cells/immunology MH - Plasmacytoma/immunology MH - Pseudolymphoma/immunology MH - Psoriasis/immunology MH - Purpura, Hyperglobulinemic/immunology MH - Skin Diseases/classification/*immunology MH - Skin Diseases, Papulosquamous/immunology MH - Urticaria/immunology MH - Vasculitis/immunology EDAT- 2014/07/30 06:00 MHDA- 2015/07/08 06:00 CRDT- 2014/07/29 06:00 PHST- 2014/07/14 00:00 [accepted] PHST- 2014/07/29 06:00 [entrez] PHST- 2014/07/30 06:00 [pubmed] PHST- 2015/07/08 06:00 [medline] AID - 10.1111/bjd.13296 [doi] PST - ppublish SO - Br J Dermatol. 2014 Nov;171(5):959-67. doi: 10.1111/bjd.13296. Epub 2014 Oct 20. PMID- 34731871 OWN - NLM STAT- MEDLINE DCOM- 20220126 LR - 20230912 IS - 1536-481X (Electronic) IS - 1057-0829 (Print) IS - 1057-0829 (Linking) VI - 31 IP - 1 DP - 2022 Jan 1 TI - Multiple Systemic Vascular Risk Factors Are Associated With Low-Tension Glaucoma. PG - 15-22 LID - 10.1097/IJG.0000000000001964 [doi] AB - PRCIS: Multiple systemic vascular-associated conditions including systemic hypertension and hypotension, diabetes mellitus, migraine headache, peripheral vascular disease, Raynaud syndrome, and anemia were associated with low-tension glaucoma. PURPOSE: The purpose of this study was to identify systemic risk factors associated with low-tension glaucoma. PATIENTS AND METHODS: A retrospective case-control study design was employed to identify patients seen at the Mayo Clinic Department of Ophthalmology between 2005 and 2015 with low-tension glaucoma and an age-matched and sex-matched control group, each containing 277 patients. RESULTS: The low-tension glaucoma group had more myopic refractive errors (-1.6 vs. -1.0 D, P<0.001), lower intraocular pressure (14.2 vs. 15.2 mm Hg, P<0.001), and a higher cup-to-disc ratio (0.7 vs. 0.3, P<0.001). The low-tension glaucoma group was significantly less likely to be obese (body mass index >30, P=0.03). This group had a significantly higher prevalence of systemic hypertension [odds ratio (OR): 1.64, P=0.004], diabetes mellitus (OR: 3.01, P<0.001), peripheral vascular disease (OR: 2.61, P=0.009), migraine headache (OR: 2.12, P=0.02), anemia (OR: 2.18, P=0.003), systemic hypotension (OR: 4.43, P<0.001), Raynaud syndrome (OR: 3.09, P=0.05), and angiotensin-converting enzyme inhibitor (OR: 1.64, P=0.01) or calcium channel blocker use (OR: 1.98, P=0.004). After adjusting for systemic hypertension, calcium channel blocker use remained significant (OR: 1.70, P=0.03). No significant difference was found between groups with respect to hyperlipidemia, obstructive sleep apnea, coronary artery disease, carotid stenosis, stroke, or statin, angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, beta-blocker, or metformin use. CONCLUSIONS: Multiple vascular-associated conditions were associated with low-tension glaucoma including systemic hypertension, diabetes mellitus, peripheral vascular disease, migraine headache, Raynaud syndrome, anemia, systemic hypotension, and calcium channel blocker use. This study strengthens the evidence for the vascular hypothesis of low-tension glaucoma. CI - Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved. FAU - Funk, Robert O AU - Funk RO AD - Department of Ophthalmology, Mayo Clinic, Rochester, MN. FAU - Hodge, David O AU - Hodge DO AD - Department of Quantitative Health Sciences, Mayo Clinic, Jacksonville, FL. FAU - Kohli, Darrell AU - Kohli D AD - Department of Ophthalmology, Mayo Clinic, Rochester, MN. FAU - Roddy, Gavin W AU - Roddy GW AD - Department of Ophthalmology, Mayo Clinic, Rochester, MN. LA - eng GR - K08 EY031758/EY/NEI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - J Glaucoma JT - Journal of glaucoma JID - 9300903 SB - IM MH - Case-Control Studies MH - Humans MH - *Intraocular Pressure MH - *Low Tension Glaucoma/epidemiology MH - Retrospective Studies MH - Risk Factors PMC - PMC9337264 MID - NIHMS1823702 COIS- Disclosure: The authors declare no conflict of interest. EDAT- 2021/11/04 06:00 MHDA- 2022/01/27 06:00 PMCR- 2022/07/29 CRDT- 2021/11/03 20:11 PHST- 2021/07/12 00:00 [received] PHST- 2021/10/20 00:00 [accepted] PHST- 2021/11/04 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] PHST- 2021/11/03 20:11 [entrez] PHST- 2022/07/29 00:00 [pmc-release] AID - 00061198-202201000-00003 [pii] AID - 10.1097/IJG.0000000000001964 [doi] PST - ppublish SO - J Glaucoma. 2022 Jan 1;31(1):15-22. doi: 10.1097/IJG.0000000000001964. PMID- 34295197 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240402 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 13 DP - 2021 TI - Autoantibody Profile of Egyptian Juvenile Systemic Lupus Erythematosus Patients and Its Association with Clinical Characteristics and Disease Activity. PG - 201-212 LID - 10.2147/OARRR.S317315 [doi] AB - OBJECTIVE: This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile systemic lupus erythematosus (JSLE) patients and their association with different clinical manifestations and disease activity. PATIENTS AND METHODS: A cross-sectional study that includes 100 JSLE patients from Ain Shams University Hospital was conducted. All subjects underwent history taking, clinical examination, assessment of disease activity based on the SLE disease activity index (SLEDAI), laboratory investigations, and tests for autoantibodies, namely ANA, anti-dsDNA, and anti-ENA antibodies, including anti-Ro (SSA), anti-La (SSB), anti-Smith (Sm), and anti-U1-ribonucleoprotein (U1-RNP). RESULTS: The most common clinical features were polyarthralgia (71%), haematological manifestations (65%), malar rash (54%), and nephritis (51%), respectively. All patients had positive ANA (100%), while anti-dsDNA frequency was 83%. The most common anti-ENA antibodies were anti-RNP (41%), anti-Sm (31%), anti-SSA (27%), and anti-SSB (20%), respectively. Anti-RNP had a clinical association with oral ulcer, Raynaud' phenomena, haematological, neuropsychiatric and thromboembolic manifestations. Meanwhile, anti-Sm had a significant association with serositis, mucocutaneous, constitutional, and neuropsychiatric manifestations. Anti-SSA was associated with mucocutaneous, musculoskeletal, Raynaud' phenomena, renal, haematological and cardiac manifestations, while anti-SSB was significantly associated with malar rash, serositis, thromboembolic, musculoskeletal, and neuropsychiatric manifestations. Concerning SLEADI score, anti-dsDNA antibody was significantly associated with moderate disease activity score (p=0.032) while anti-SSA significantly associated with high disease activity (p=0.045). Both anti-SSB and anti-Sm were significantly associated with both moderate and high disease activities, meanwhile anti-U1-RNP was associated with moderate disease activity (p=0.014). CONCLUSION: Anti-dsDNA and anti-ENAs antibodies were frequently found in JSLE patients (83%, 63%), respectively. They were significantly associated with variable clinical manifestations and could be used as predictors for assessment of disease activity. CI - © 2021 Abd El Monem Teama et al. FAU - Abd El Monem Teama, Mohammed AU - Abd El Monem Teama M AUID- ORCID: 0000-0002-3945-198X AD - Internal Medicine Department, Division of Rheumatology and Immunology, Faculty of Medicine, Ain Shams University, Cairo, Egypt. FAU - Adham El-Mohamdy, Marwa AU - Adham El-Mohamdy M AD - Clinical Pathology Department, Faculty of medicine, Ain Shams University, Cairo, Egypt. FAU - Abdellah Abdullah Mahmoud, Fatma AU - Abdellah Abdullah Mahmoud F AD - Clinical Pathology Department, Faculty of medicine, Ain Shams University, Cairo, Egypt. FAU - Mohammed Badr, Fatma AU - Mohammed Badr F AD - Internal Medicine Department, Division of Rheumatology and Immunology, Faculty of Medicine, Ain Shams University, Cairo, Egypt. LA - eng PT - Journal Article DEP - 20210716 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC8291800 OTO - NOTNLM OT - anti-ENA OT - clinical manifestations OT - disease activity score OT - juvenile systemic lupus erythematosus COIS- We declare no conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2021/07/24 06:00 MHDA- 2021/07/24 06:01 PMCR- 2021/07/16 CRDT- 2021/07/23 06:45 PHST- 2021/05/05 00:00 [received] PHST- 2021/07/02 00:00 [accepted] PHST- 2021/07/23 06:45 [entrez] PHST- 2021/07/24 06:00 [pubmed] PHST- 2021/07/24 06:01 [medline] PHST- 2021/07/16 00:00 [pmc-release] AID - 317315 [pii] AID - 10.2147/OARRR.S317315 [doi] PST - epublish SO - Open Access Rheumatol. 2021 Jul 16;13:201-212. doi: 10.2147/OARRR.S317315. eCollection 2021. PMID- 32740671 OWN - NLM STAT- MEDLINE DCOM- 20201221 LR - 20201221 IS - 1432-1971 (Electronic) IS - 0172-0643 (Linking) VI - 41 IP - 8 DP - 2020 Dec TI - Effect of General Anesthesia on Cardiac Magnetic Resonance-Derived Cardiac Function in Repaired Tetralogy of Fallot. PG - 1660-1666 LID - 10.1007/s00246-020-02425-9 [doi] AB - Cardiac magnetic resonance imaging (CMR)-derived ejection fraction (EF) predicts adverse outcomes in repaired tetralogy of Fallot (rTOF) and drives timing of pulmonary valve replacement. Certain patient populations require sedation for successful CMR image acquisition. General anesthesia (GA) has been shown to depress EF and heart rate (HR) in animal models, however, its effect on congenital heart disease is unknown. A retrospective review was conducted of all CMR patients referred with rTOF between January 2011 and May 2019. The cohort was separated into GA and non-GA groups. Propensity score matching (PSM) adjusted for selection bias. A kernel matching algorithm was used to match subjects and the differences in mean treatment effect on the treated were computed for left ventricular (LV) and right ventricular (RV) EF, HR, and cardiac index (CI). 143 patients met criteria, 37 patients under GA (mean age 15 years, range 2-45, 59% male), and 106 patients without GA (mean age 21 years, range 10-53, 50% male). Unmatched analysis showed significant depression of LV EF (50 vs. 57%, p < 0.001) and RV EF (42 vs. 48%, p < 0.001) in the GA group compared to the non-GA group. There was no significant difference in HR or CI. After matching and PSM adjustment, the GA group had a significant decrease in LV EF (49 vs. 56%, p < 0.001), RV EF (41 vs. 48%, p < 0.001), CI (2728 vs. 3701 ml/min/m(2), p < 0.001), and HR (72 vs. 79 bpm, p = 0.04). General anesthesia with sevoflurane results in depressed CMR-derived EF. FAU - Muyskens, Steve AU - Muyskens S AUID- ORCID: 0000-0001-9515-4050 AD - Department of Pediatric Cardiology, Cook Children's Medical Center, 1500 Cooper Street, 3rd Floor, Fort Worth, TX, 76104, USA. steve.muyskens@cookchildrens.org. FAU - Roshan, Tony AU - Roshan T AD - University of North Texas Health Science Center, Texas College of Osteopathic Medicine, Fort Worth, TX, USA. FAU - Honan, Kevin AU - Honan K AD - University of North Texas Health Science Center, Texas College of Osteopathic Medicine, Fort Worth, TX, USA. FAU - Umejiego, Johnbosco AU - Umejiego J AD - Department of Research Operations, Cook Children's Medical Center, Fort Worth, TX, USA. FAU - Raynaud, Scott AU - Raynaud S AD - Department of Research Operations, Cook Children's Medical Center, Fort Worth, TX, USA. FAU - Ogunyankin, Fadeke AU - Ogunyankin F AD - Department of Research Operations, Cook Children's Medical Center, Fort Worth, TX, USA. LA - eng PT - Journal Article DEP - 20200801 PL - United States TA - Pediatr Cardiol JT - Pediatric cardiology JID - 8003849 RN - 0 (Anesthetics, Inhalation) RN - 38LVP0K73A (Sevoflurane) SB - IM MH - Adolescent MH - Adult MH - Anesthesia, General/*methods MH - Anesthetics, Inhalation/therapeutic use MH - Cardiac Surgical Procedures/methods MH - Child MH - Child, Preschool MH - Female MH - Heart Ventricles/diagnostic imaging MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Male MH - Middle Aged MH - Propensity Score MH - Retrospective Studies MH - Sevoflurane/*therapeutic use MH - Stroke Volume/*drug effects MH - Tetralogy of Fallot/*diagnostic imaging/surgery MH - Ventricular Function, Left/drug effects MH - Ventricular Function, Right/drug effects MH - Young Adult OTO - NOTNLM OT - Cardiac MRI OT - General anesthesia OT - Tetralogy of fallot EDAT- 2020/08/03 06:00 MHDA- 2020/12/22 06:00 CRDT- 2020/08/03 06:00 PHST- 2020/04/15 00:00 [received] PHST- 2020/07/21 00:00 [accepted] PHST- 2020/08/03 06:00 [pubmed] PHST- 2020/12/22 06:00 [medline] PHST- 2020/08/03 06:00 [entrez] AID - 10.1007/s00246-020-02425-9 [pii] AID - 10.1007/s00246-020-02425-9 [doi] PST - ppublish SO - Pediatr Cardiol. 2020 Dec;41(8):1660-1666. doi: 10.1007/s00246-020-02425-9. Epub 2020 Aug 1. PMID- 19666869 OWN - NLM STAT- MEDLINE DCOM- 20091027 LR - 20260518 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 114 IP - 15 DP - 2009 Oct 8 TI - TET2 mutation is an independent favorable prognostic factor in myelodysplastic syndromes (MDSs). PG - 3285-91 LID - 10.1182/blood-2009-04-215814 [doi] AB - Oncogenic pathways underlying in the development of myelodysplastic syndromes (MDS) remain poorly characterized, but mutations of the ten-eleven translocation 2 (TET2) gene are frequently observed. In the present work, we evaluated the prognostic impact of TET2 mutations in MDS. Frameshift, nonsense, missense mutations, or defects in gene structure were identified in 22 (22.9%) of 96 patients (95% confidence interval [CI], 14.5-31.3 patients). Mutated and unmutated patients did not significantly differ in initial clinical or hematologic parameters. The 5-year OS was 76.9% (95% CI, 49.2%-91.3%) in mutated versus 18.3% (95% CI, 4.2%-41.1%) in unmutated patients (P = .005). The 3-year leukemia-free survival was 89.3% (95% CI, 63.1%-97.0%) in mutated versus 63.7% (95% CI, 48.2%-75.4%) in unmutated patients (P = .035). In univariate analysis (Cox proportional hazard model), the absence of TET2 mutation was associated with a 4.1-fold (95% CI, 1.4-12.0-fold) increased risk of death (P = .009). In multivariate analysis adjusted for age, International Prognostic Scoring System, and transfusion requirement, the presence of TET2 mutation remained an independent factor of favorable prognosis (hazard ratio, 5.2; 95% CI, 1.6-16.3; P = .005). These results indicate that TET2 mutations observed in approximately 20% of patients, irrespective of the World Health Organization or French-American-British subtype, represent a molecular marker for good prognosis in MDS. FAU - Kosmider, Olivier AU - Kosmider O AD - Assistance Publique-Hôpitaux de Paris (AP-HP), Service d'Hématologie Biologique, Hôpital Cochin-Hôtel-Dieu, Paris, France. FAU - Gelsi-Boyer, Véronique AU - Gelsi-Boyer V FAU - Cheok, Meyling AU - Cheok M FAU - Grabar, Sophie AU - Grabar S FAU - Della-Valle, Véronique AU - Della-Valle V FAU - Picard, Françoise AU - Picard F FAU - Viguié, Franck AU - Viguié F FAU - Quesnel, Bruno AU - Quesnel B FAU - Beyne-Rauzy, Odile AU - Beyne-Rauzy O FAU - Solary, Eric AU - Solary E FAU - Vey, Norbert AU - Vey N FAU - Hunault-Berger, Mathilde AU - Hunault-Berger M FAU - Fenaux, Pierre AU - Fenaux P FAU - Mansat-De Mas, Véronique AU - Mansat-De Mas V FAU - Delabesse, Eric AU - Delabesse E FAU - Guardiola, Philippe AU - Guardiola P FAU - Lacombe, Catherine AU - Lacombe C FAU - Vainchenker, William AU - Vainchenker W FAU - Preudhomme, Claude AU - Preudhomme C FAU - Dreyfus, François AU - Dreyfus F FAU - Bernard, Olivier A AU - Bernard OA FAU - Birnbaum, Daniel AU - Birnbaum D FAU - Fontenay, Michaëla AU - Fontenay M CN - Groupe Francophone des Myélodysplasies LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20090807 PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (DNA-Binding Proteins) RN - 0 (Genetic Markers) RN - 0 (Proto-Oncogene Proteins) RN - EC 1.13.11.- (Dioxygenases) RN - EC 1.13.11.- (TET2 protein, human) SB - IM MH - Aged MH - Aged, 80 and over MH - DNA-Binding Proteins/*genetics/metabolism MH - Dioxygenases MH - Disease-Free Survival MH - Female MH - Follow-Up Studies MH - Genetic Markers MH - Humans MH - Male MH - Middle Aged MH - *Mutation MH - Myelodysplastic Syndromes/*genetics/*mortality MH - Predictive Value of Tests MH - Proto-Oncogene Proteins/*genetics/metabolism MH - Risk Factors MH - Survival Rate FIR - Guerci, A IR - Guerci A FIR - Maynadié, M IR - Maynadié M FIR - Lafond, I IR - Lafond I FIR - Slama, B IR - Slama B FIR - Gardembas-Pain, M IR - Gardembas-Pain M FIR - Raynaud, S IR - Raynaud S FIR - Legros, L IR - Legros L EDAT- 2009/08/12 09:00 MHDA- 2009/10/29 06:00 CRDT- 2009/08/12 09:00 PHST- 2009/08/12 09:00 [entrez] PHST- 2009/08/12 09:00 [pubmed] PHST- 2009/10/29 06:00 [medline] AID - S0006-4971(20)36804-X [pii] AID - 10.1182/blood-2009-04-215814 [doi] PST - ppublish SO - Blood. 2009 Oct 8;114(15):3285-91. doi: 10.1182/blood-2009-04-215814. Epub 2009 Aug 7. PMID- 23885934 OWN - NLM STAT- MEDLINE DCOM- 20130926 LR - 20130726 IS - 1558-3171 (Electronic) IS - 0039-6109 (Linking) VI - 93 IP - 4 DP - 2013 Aug TI - Nonarteriosclerotic vascular disease. PG - 833-75, viii LID - S0039-6109(13)00053-4 [pii] LID - 10.1016/j.suc.2013.04.003 [doi] AB - Thromboangiitis obliterans, or Buerger disease, is a chronic nonatherosclerotic endarteritis manifesting as inflammation and thrombosis of distal extremity small and medium-sized arteries resulting in relapsing episodes of distal extremity ischemia. Takayasu arteritis is a rare syndrome characterized by inflammation of the aortic arch, pulmonary, coronary, and cerebral vessels, presenting with cerebrovascular symptoms, myocardial ischemia, or upper extremity claudication in young, often female, patients. Kawasaki disease is a small- and medium-vessel acute systemic vasculitis of young children, with morbidity and mortality stemming from coronary artery aneurysms. Microscopic polyangiitis, Churg-Strauss syndrome, and Wegener granulomatosis are systemic small-vessel vasculitides, affecting arterioles, capillary beds and venules, and each presenting with variable effects on the pulmonary, renal and gastrointestinal systems. CI - Copyright © 2013 Elsevier Inc. All rights reserved. FAU - Wu, William AU - Wu W AD - Division of Vascular Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA. FAU - Chaer, Rabih A AU - Chaer RA LA - eng PT - Journal Article PT - Review DEP - 20130617 PL - United States TA - Surg Clin North Am JT - The Surgical clinics of North America JID - 0074243 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Cardiovascular Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Biopsy/methods MH - Blood Vessel Prosthesis Implantation/methods MH - Cardiovascular Agents/therapeutic use MH - Collateral Circulation/physiology MH - Diagnostic Imaging/methods MH - Female MH - Humans MH - Male MH - Reperfusion/methods MH - Risk Factors MH - Treatment Outcome MH - *Vasculitis/diagnosis/etiology/therapy OTO - NOTNLM OT - Giant cell arteritis OT - Kawasaki disease OT - Polyarteritis nodosa OT - Radiation arteritis OT - Raynaud phenomenon OT - Small-vessel vasculitides OT - Takayasu autoimmune arteritis OT - Thromboangiitis obliterans EDAT- 2013/07/28 06:00 MHDA- 2013/09/27 06:00 CRDT- 2013/07/27 06:00 PHST- 2013/07/27 06:00 [entrez] PHST- 2013/07/28 06:00 [pubmed] PHST- 2013/09/27 06:00 [medline] AID - S0039-6109(13)00053-4 [pii] AID - 10.1016/j.suc.2013.04.003 [doi] PST - ppublish SO - Surg Clin North Am. 2013 Aug;93(4):833-75, viii. doi: 10.1016/j.suc.2013.04.003. Epub 2013 Jun 17. PMID- 35225803 OWN - NLM STAT- MEDLINE DCOM- 20220302 LR - 20220302 IS - 0363-6771 (Print) IS - 0363-6771 (Linking) VI - 70 IP - 2 DP - 2022 Mar-Apr TI - Scleroderma and multiple teeth with invasive cervical resorption: is there a connection? PG - 41-44 AB - Scleroderma is a chronic connective tissue disease generally classified as an autoimmune rheumatic disease. Symptoms may include thickening of the skin, calcifications, Raynaud syndrome, and esophageal problems. Invasive cervical resorption is an aggressive form of external resorption localized in the cervical part of the tooth. Its etiology remains uncertain. This case report describes invasive cervical resorption affecting 4 teeth in a 44-year-old woman with scleroderma and speculates on the possible relationships between these disease entities. FAU - Journo, Jonathan E AU - Journo JE FAU - Woodmansey, Karl F AU - Woodmansey KF FAU - Walsh, Ryan M AU - Walsh RM LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Gen Dent JT - General dentistry JID - 7610466 MH - Adult MH - *Autoimmune Diseases/complications MH - Female MH - Humans MH - *Root Resorption/etiology MH - *Tooth Resorption OTO - NOTNLM OT - autoimmune diseases OT - invasive cervical resorption OT - scleroderma EDAT- 2022/03/01 06:00 MHDA- 2022/03/03 06:00 CRDT- 2022/02/28 12:15 PHST- 2022/02/28 12:15 [entrez] PHST- 2022/03/01 06:00 [pubmed] PHST- 2022/03/03 06:00 [medline] PST - ppublish SO - Gen Dent. 2022 Mar-Apr;70(2):41-44. PMID- 36862558 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230328 IS - 1929-073X (Print) IS - 1929-073X (Electronic) IS - 1929-073X (Linking) VI - 12 DP - 2023 Mar 8 TI - Acute Spontaneous Colonic Perforation in a Case of Newly Confirmed Scleroderma: Case Report. PG - e43295 LID - 10.2196/43295 [doi] LID - e43295 AB - Scleroderma is a group of autoimmune diseases that principally affects the skin, blood vessels, muscles, and viscera. One of the more well-known subgroups of scleroderma is the limited cutaneous form of the multisystem connective tissue disorder known as CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasis) syndrome. In this report, we present a case of a spontaneous colonic bowel perforation in a patient with incomplete features of CREST. Our patient underwent a complicated hospital course involving broad-spectrum antibiotic coverage, surgical hemicolectomy, and immunosuppressives. She was eventually discharged home with a return to functional baseline status after esophageal dysmotility confirmation via manometry. Physicians managing patients with scleroderma ensuing to an emergency department encounter must anticipate the multitude of complications that can occur, as was seen in our patient. The threshold for pursuing imaging and additional tests, in addition to admission, should be relatively low, given the extremely high rates of complications and mortality. Early multidisciplinary involvement with infectious disease, rheumatology, surgery, and other respective specialties is crucial for patient outcome optimization. CI - ©Glenn Goodwin, Christian Ryckeley, Davide Fox, Michael Ashley, Laurence Dubensky, Mauricio Danckers, Todd Slesinger. Originally published in the Interactive Journal of Medical Research (https://www.i-jmr.org/), 08.03.2023. FAU - Goodwin, Glenn AU - Goodwin G AUID- ORCID: 0000-0003-4940-7605 AD - Emergency Department, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Ryckeley, Christian AU - Ryckeley C AUID- ORCID: 0000-0001-7348-9968 AD - Emergency Department, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Fox, Davide AU - Fox D AD - Department of Critical Care, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Ashley, Michael AU - Ashley M AD - Department of Radiology, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Dubensky, Laurence AU - Dubensky L AD - Emergency Department, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Danckers, Mauricio AU - Danckers M AUID- ORCID: 0000-0002-6387-1060 AD - Department of Critical Care, Aventura Hospital and Medical Center, Aventura, FL, United States. FAU - Slesinger, Todd AU - Slesinger T AUID- ORCID: 0000-0002-5861-6989 AD - Emergency Department, Aventura Hospital and Medical Center, Aventura, FL, United States. LA - eng PT - Case Reports PT - Journal Article DEP - 20230308 PL - Canada TA - Interact J Med Res JT - Interactive journal of medical research JID - 101598421 PMC - PMC10034614 OTO - NOTNLM OT - CREST syndrome OT - autoimmune OT - calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasis OT - case report OT - connective tissue OT - dermatology OT - emergency OT - esophageal OT - esophagus OT - gastroenterology OT - gastrointestinal OT - internal medicine OT - multisystem connective tissue disorder OT - perforation OT - scleroderma OT - sclerosis OT - skin OT - spontaneous bowel perforation OT - systemic sclerosis COIS- Conflicts of Interest: None declared. EDAT- 2023/03/03 06:00 MHDA- 2023/03/03 06:01 PMCR- 2023/03/08 CRDT- 2023/03/02 12:42 PHST- 2022/10/07 00:00 [received] PHST- 2023/02/24 00:00 [accepted] PHST- 2023/02/06 00:00 [revised] PHST- 2023/03/03 06:00 [pubmed] PHST- 2023/03/03 06:01 [medline] PHST- 2023/03/02 12:42 [entrez] PHST- 2023/03/08 00:00 [pmc-release] AID - v12i1e43295 [pii] AID - 10.2196/43295 [doi] PST - epublish SO - Interact J Med Res. 2023 Mar 8;12:e43295. doi: 10.2196/43295. PMID- 28967806 OWN - NLM STAT- MEDLINE DCOM- 20190201 LR - 20190201 IS - 1543-2165 (Electronic) IS - 0003-9985 (Linking) VI - 142 IP - 2 DP - 2018 Feb TI - Pulmonary Pathologic Manifestations of Anti-Alanyl-tRNA Synthetase (Anti-PL-12)-Related Inflammatory Myopathy. PG - 191-197 LID - 10.5858/arpa.2017-0010-OA [doi] AB - CONTEXT: - Patients with anti-aminoacyl-tRNA synthetase syndrome (ARS), a subset of idiopathic inflammatory myopathy, have a high prevalence of lung involvement. Autoantibodies directed against alanyl-tRNA synthetase (anti-PL-12 Abs) represent 1 of the 8 autoantibodies currently described under the rubric of ARS. OBJECTIVE: - To describe the clinical, radiographic, and pulmonary histopathologic findings in patients possessing anti-PL-12 autoantibodies. DESIGN: - Patients with anti-PL-12 ARS were identified in the University of Pittsburgh Idiopathic Inflammatory Myopathy registry. Lung biopsies from 10 patients and lung explants from 2 patients with anti-PL-12 ARS were reviewed, together with chest computed tomography and clinical records. RESULTS: - Patients primarily presented with dyspnea and variable combinations of cough, fever, mechanic's hands, Raynaud phenomenon, and skin and muscle involvement. Chest computed tomography most commonly showed lower lung zone-predominant reticular infiltrates and traction bronchiectasis, with or without honeycomb change. Surgical lung biopsies and pneumonectomies for lung transplantation revealed usual interstitial pneumonia in 8 of 12 cases (67%), nonspecific interstitial pneumonia in 2 of 12 cases (17%), and organizing pneumonia in 2 of 12 cases (17%). Lymphoplasmacytic interstitial inflammation with lymphoid aggregates was common. CONCLUSIONS: - Lung disease is often the first manifestation of anti-PL-12 ARS. There are no pathognomonic histopathologic features to distinguish anti-PL-12 ARS-related lung disease from idiopathic variants of diffuse interstitial lung disease. Increased inflammation, lymphoid aggregates, and nonspecific interstitial pneumonia-like areas in a biopsy, as well as clinical features of mechanic's hands, Raynaud phenomenon, arthritis, and fever, should prompt pathologists to suggest involvement by ARS. FAU - Schneider, Frank AU - Schneider F FAU - Yousem, Samuel A AU - Yousem SA FAU - Oddis, Chester V AU - Oddis CV FAU - Aggarwal, Rohit AU - Aggarwal R LA - eng PT - Journal Article DEP - 20171002 PL - United States TA - Arch Pathol Lab Med JT - Archives of pathology & laboratory medicine JID - 7607091 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - EC 6.1.1.7 (Alanine-tRNA Ligase) SB - IM MH - Adolescent MH - Adult MH - Alanine-tRNA Ligase/immunology MH - Autoantibodies/immunology MH - Autoantigens/immunology MH - Female MH - Humans MH - Lung Diseases/*immunology/*pathology MH - Male MH - Middle Aged MH - Myositis/*complications MH - Young Adult EDAT- 2017/10/03 06:00 MHDA- 2019/02/02 06:00 CRDT- 2017/10/03 06:00 PHST- 2017/10/03 06:00 [pubmed] PHST- 2019/02/02 06:00 [medline] PHST- 2017/10/03 06:00 [entrez] AID - 10.5858/arpa.2017-0010-OA [doi] PST - ppublish SO - Arch Pathol Lab Med. 2018 Feb;142(2):191-197. doi: 10.5858/arpa.2017-0010-OA. Epub 2017 Oct 2. PMID- 27550299 OWN - NLM STAT- MEDLINE DCOM- 20170612 LR - 20191210 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 55 IP - 11 DP - 2016 Nov TI - An easy prediction rule for diffuse cutaneous systemic sclerosis using only the timing and type of first symptoms and auto-antibodies: derivation and validation. PG - 2023-2032 AB - OBJECTIVE: DcSSc is associated with high morbidity related to widespread skin disease and poor prognosis due to earlier and more severe organ involvement. The objective of this study is to derive and validate a simple prediction rule for identifying patients at the time of initial diagnosis of SSc who are likely to progress to dcSSc. METHODS: The Nijmegen cohort consists of 619 SSc patients. Logistic regression was used for predictive modelling. A prediction rule was created by rounding regression coefficients. Patients were stratified as being at low risk (<1) or high risk (⩾1) of progression to dcSSc. Performance was analysed in 445 SSc patients from Madrid. RESULTS: One hundred and seventy-four out of 535 patients were classified as dcSSc. The final model consisted of gender, time between RP and non-RP, sclerodactyly (first non-Raynaud symptom) and SSc-specific auto-antibodies. The model performed well in the derivation cohort [area under the curve = 0.78 (95% CI: 0.74, 0.82)] and validation cohort [area under the curve  = 0.78 (95% CI: 0.74, 0.83)]. At the optimal cut point (1) for the prediction rule, sensitivity was 87% and specificity 61% in the derivation cohort, compared with 78% and 65% in the validation cohort. Upon application of the prediction rule to 392 lcSSc patients at initial diagnosis, 32 out of 34 patients were correctly classified as dcSSc. CONCLUSION: A simple prediction rule was designed to attribute a low/high risk category for development of dcSSc.This method is suited for assigning intensified screening at the time of initial diagnosis of SSc to patients most at risk for dcSSc. It provides the opportunity for early identification of potential dcSSc patients for enrolment into clinical trials. CI - © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - van den Hombergh, Wieneke M T AU - van den Hombergh WM AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands Wieneke.vandenHombergh@radboudumc.nl. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital 12 de Octubre, Madrid, Spain. FAU - Knaapen-Hans, Hanneke K A AU - Knaapen-Hans HK AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - van den Hoogen, Frank H J AU - van den Hoogen FH AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Fransen, Jaap AU - Fransen J AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands. LA - eng PT - Journal Article PT - Observational Study PT - Validation Study DEP - 20160821 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Autoantibodies) SB - IM MH - Autoantibodies/*metabolism MH - Decision Support Systems, Clinical/*standards MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prospective Studies MH - Scleroderma, Diffuse/*diagnosis MH - Time Factors OTO - NOTNLM OT - disease subtype OT - first non-Raynaud symptom OT - prediction OT - systemic sclerosis EDAT- 2016/08/24 06:00 MHDA- 2017/06/13 06:00 CRDT- 2016/08/24 06:00 PHST- 2016/03/04 00:00 [received] PHST- 2016/07/13 00:00 [revised] PHST- 2016/08/24 06:00 [pubmed] PHST- 2017/06/13 06:00 [medline] PHST- 2016/08/24 06:00 [entrez] AID - kew305 [pii] AID - 10.1093/rheumatology/kew305 [doi] PST - ppublish SO - Rheumatology (Oxford). 2016 Nov;55(11):2023-2032. doi: 10.1093/rheumatology/kew305. Epub 2016 Aug 21. PMID- 31655256 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231014 IS - 2589-0042 (Electronic) IS - 2589-0042 (Linking) VI - 21 DP - 2019 Nov 22 TI - Dynamic Emergence of Observed and Hidden Intra-tumor Heterogeneity. PG - 157-167 LID - S2589-0042(19)30397-9 [pii] LID - 10.1016/j.isci.2019.10.018 [doi] AB - Intra-tumor heterogeneity is frequently observed in cancer patients, and it is associated with therapeutic resistance and disease relapse. However, its systematic assessment is still limited and often unfeasible. Here, we use a mathematical model of tumor progression to decipher how multiple clones emerge and organize into complex architectures. We found a trade-off between cancer cell alteration and proliferation rates that defines a transition between low and high heterogeneity, the latter characterized by branching tumor phylogenies. We predict the existence of observed and hidden intra-tumor heterogeneity, which challenges the correct estimation of intrinsic tumor complexity. Although the numbers of observed and hidden clones do not always correlate, we demonstrate that population frequencies of observed clones can be used to estimate the extent of hidden heterogeneity in both simulated and human tumors. The characterization of complex clonal architectures is a critical first step toward understanding their organizing principles and predicting their emergence. CI - Copyright © 2019 The Author(s). Published by Elsevier Inc. All rights reserved. FAU - Raynaud, Franck AU - Raynaud F AD - Department of Computational Biology, University of Lausanne, Lausanne, 1015 Lausanne, Switzerland; Swiss Institute of Bioinformatics, Lausanne, 1015 Lausanne, Switzerland; Department of Computer Science, University of Geneva, 1205, Geneva, Switzerland. Electronic address: franck.raynaud@unige.ch. FAU - Mina, Marco AU - Mina M AD - Department of Computational Biology, University of Lausanne, Lausanne, 1015 Lausanne, Switzerland; Swiss Institute of Bioinformatics, Lausanne, 1015 Lausanne, Switzerland. FAU - Ciriello, Giovanni AU - Ciriello G AD - Department of Computational Biology, University of Lausanne, Lausanne, 1015 Lausanne, Switzerland; Swiss Institute of Bioinformatics, Lausanne, 1015 Lausanne, Switzerland. Electronic address: giovanni.ciriello@unil.ch. LA - eng PT - Journal Article DEP - 20191010 PL - United States TA - iScience JT - iScience JID - 101724038 PMC - PMC6820272 OTO - NOTNLM OT - Bioinformatics OT - Cancer OT - Cancer Systems Biology OT - Mathematical Biosciences OT - Systems Biology COIS- The authors declare no competing interests. EDAT- 2019/10/28 06:00 MHDA- 2019/10/28 06:01 PMCR- 2019/10/10 CRDT- 2019/10/27 06:00 PHST- 2018/07/05 00:00 [received] PHST- 2019/07/30 00:00 [revised] PHST- 2019/10/07 00:00 [accepted] PHST- 2019/10/28 06:00 [pubmed] PHST- 2019/10/28 06:01 [medline] PHST- 2019/10/27 06:00 [entrez] PHST- 2019/10/10 00:00 [pmc-release] AID - S2589-0042(19)30397-9 [pii] AID - 10.1016/j.isci.2019.10.018 [doi] PST - ppublish SO - iScience. 2019 Nov 22;21:157-167. doi: 10.1016/j.isci.2019.10.018. Epub 2019 Oct 10. PMID- 41833335 OWN - NLM STAT- Publisher LR - 20260415 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) DP - 2026 Apr 15 TI - Systemic Sclerosis Without Raynaud Phenomenon in Children: The Fibrotic Subtype. LID - jrheum.2025-1104 [pii] LID - 10.3899/jrheum.2025-1104 [doi] AB - OBJECTIVE: Juvenile-onset systemic sclerosis (JSSc) is characterized by vascular manifestations, including Raynaud phenomenon (RP) and vascular disease-related internal organ damage. We describe the clinical features of a subgroup of JSSc patients without clinical vascular manifestations, as a hypothesis-generating analysis of a possible new subtype. METHODS: This was a single-center cohort study of consecutive patients with JSSc diagnosed since 2004. Data on demographics, clinical features, autoantibody profile, and treatment were collected in a standardized method. Disease severity was periodically evaluated by the Juvenile Systemic Sclerosis Severity Score (J4S). Outcome was categorized as clinical remission (CR), clinical remission on medication (CRM), or active/progressive disease (AP). RESULTS: Of 45 patients, 38 with at least 4 years of follow-up were included in the study. The mean age at onset was 10.3 years, mean follow-up time was 8.2 years, and 60.5% were female. Thirty-two (84.2%) patients presented with RP and 5 (13%) patients, all with diffuse cutaneous involvement, did not. The latter subgroup, which we term "fibrotic JSSc" (fJSSc), showed predominant skin involvement and mild internal organ involvement (gastrointestinal in all 5 patients, pulmonary in 2 patients). Whereas antinuclear antibodies were positive in all patients with fJSSc, none had SSc-specific autoantibodies and 2 had SSc-associated autoantibodies. SSc pattern on nailfold videocapillaroscopy was detected in 4/5 patients. At the last follow-up visit, no patient with fJSSc had AP, 3 were in CR, and 2 were in CRM for mild pulmonary disease. CONCLUSION: This proof-of-concept study describes a potential new clinical phenotype, termed fJSSc, characterized by predominant skin involvement, absence of SSc-specific autoantibodies, and favorable outcome. FAU - Zulian, Francesco AU - Zulian F AUID- ORCID: 0000-0002-2479-3485 AD - F. Zulian, MD, Pediatric Rheumatology Unit, University of Padova, Department of Women's and Children's Health, Padua, Italy. FAU - Tirelli, Francesca AU - Tirelli F AD - F. Tirelli, MD, PhD, Pediatric Rheumatology Unit, University of Padova, Department of Women's and Children's Health, Padua, Italy. FAU - Mastrangelo, Greta AU - Mastrangelo G AD - G. Mastrangelo, MD, Pediatric Rheumatology Unit, University of Padova, Department of Women's and Children's Health, Padua, Italy. FAU - Vittadello, Fabio AU - Vittadello F AD - F. Vittadello, MSc, DrPh, Explora - Research and Statistical Analysis, Vigodarzere, Padua, Italy. FAU - Zanatta, Elisabetta AU - Zanatta E AD - E. Zanatta, MD, PhD, Rheumatology Division, Department of Medicine-DIMED, University of Padova, Padua, Italy. FAU - Meneghel, Alessandra AU - Meneghel A AD - A. Meneghel, MD, Pediatric Rheumatology Unit, University of Padova, Department of Women's and Children's Health, Padua, Italy. LA - eng PT - Journal Article DEP - 20260415 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM EDAT- 2026/03/16 00:30 MHDA- 2026/03/16 00:30 CRDT- 2026/03/15 20:43 PHST- 2026/03/16 00:30 [pubmed] PHST- 2026/03/16 00:30 [medline] PHST- 2026/03/15 20:43 [entrez] AID - jrheum.2025-1104 [pii] AID - 10.3899/jrheum.2025-1104 [doi] PST - aheadofprint SO - J Rheumatol. 2026 Apr 15:jrheum.2025-1104. doi: 10.3899/jrheum.2025-1104. PMID- 21884608 OWN - NLM STAT- MEDLINE DCOM- 20111114 LR - 20220310 IS - 1465-993X (Electronic) IS - 1465-9921 (Print) IS - 1465-9921 (Linking) VI - 12 IP - 1 DP - 2011 Sep 1 TI - Interactions between HIV infection and chronic obstructive pulmonary disease: Clinical and epidemiological aspects. PG - 117 LID - 10.1186/1465-9921-12-117 [doi] AB - INTRODUCTION: An association between HIV infection and chronic obstructive pulmonary disease (COPD) has been observed in several studies. OBJECTIVE AND METHODS: we conducted a review of the literature linking HIV infection to COPD, focusing on clinical and epidemiological data published before and during widespread highly active antiretroviral therapy (HAART). RESULTS: Interactions between HIV infection and COPD appear to be influenced by multiple factors. In particular, the bronchopulmonary tract can be damaged by HIV infection, the immunodeficiency it induces, and the resulting increase in the risk of pulmonary infections. In addition, the prevalence of smoking and intravenous drug use is higher in HIV-infected populations, also increasing the risk of COPD. Before the advent of HAART, respiratory tract infections probably played a major role. Since the late 1990s and the widespread use of HAART, the frequency of opportunistic infections has fallen but new complications have emerged as life expectancy has increased. CONCLUSION: given the high prevalence of smoking among HIV-infected patients, COPD may contribute significantly to morbidity and mortality in this setting. FAU - Raynaud, Christine AU - Raynaud C AD - Service de pneumologie, Centre Hospitalier Victor Dupouy, 69 rue du L,C, Prud'hon, 95100 Argenteuil, France. christine.raynaud@ch-argenteuil.fr FAU - Roche, Nicolas AU - Roche N FAU - Chouaid, Christos AU - Chouaid C LA - eng PT - Journal Article PT - Review DEP - 20110901 PL - England TA - Respir Res JT - Respiratory research JID - 101090633 SB - IM MH - Age Factors MH - Antiretroviral Therapy, Highly Active MH - HIV Infections/diagnosis/drug therapy/*epidemiology MH - Humans MH - Prevalence MH - Pulmonary Disease, Chronic Obstructive/diagnosis/*epidemiology MH - Risk Assessment MH - Risk Factors MH - Smoking/epidemiology PMC - PMC3175461 EDAT- 2011/09/03 06:00 MHDA- 2011/11/15 06:00 PMCR- 2011/09/01 CRDT- 2011/09/03 06:00 PHST- 2011/03/30 00:00 [received] PHST- 2011/09/01 00:00 [accepted] PHST- 2011/09/03 06:00 [entrez] PHST- 2011/09/03 06:00 [pubmed] PHST- 2011/11/15 06:00 [medline] PHST- 2011/09/01 00:00 [pmc-release] AID - 1465-9921-12-117 [pii] AID - 10.1186/1465-9921-12-117 [doi] PST - epublish SO - Respir Res. 2011 Sep 1;12(1):117. doi: 10.1186/1465-9921-12-117. PMID- 29359173 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220314 IS - 2332-7812 (Print) IS - 2332-7812 (Electronic) IS - 2332-7812 (Linking) VI - 5 IP - 2 DP - 2018 Mar TI - Immune myopathies with perimysial pathology: Clinical and laboratory features. PG - e434 LID - 10.1212/NXI.0000000000000434 [doi] LID - e434 AB - OBJECTIVE: Immune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP. METHODS: This is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP). RESULTS: Compared with DM-VP, IMPP patients more commonly had interstitial lung disease (ILD) (p < 0.01), Raynaud phenomenon (p < 0.05), mechanic's hands (p < 0.05), arthralgias (p < 0.001), and a sustained response to immunomodulatory therapy (p < 0.05), and less frequently had a concurrent malignancy (p < 0.01). IMPP patients had higher serum creatine kinase values (p < 0.05), more frequent serum Jo-1 (p < 0.03) or SSA/SSA52 autoantibodies (p < 0.05), and less frequent antinuclear antibodies (p < 0.01). IMPP patients with serum Jo-1/antisynthetase antibodies were more likely to have ILD (p < 0.05) and inflammatory arthritis (p < 0.05) than IMPP patients without these antibodies. CONCLUSIONS: IMPP myopathology is associated with an increased risk of ILD, Raynaud phenomenon, mechanic's hands, and inflammatory arthritis when compared with another immune myopathy (DM-VP). IMPP patients require regular screening for ILD, particularly those with antisynthetase antibodies. The absence of myositis-specific autoantibodies in a large percentage of IMPP patients emphasizes the important role for myopathology in identifying patients at higher risk of severe comorbid conditions such as ILD. FAU - Bucelli, Robert C AU - Bucelli RC AD - Department of Neurology, Washington University School of Medicine, St. Louis, MO. FAU - Pestronk, Alan AU - Pestronk A AD - Department of Neurology, Washington University School of Medicine, St. Louis, MO. LA - eng PT - Journal Article DEP - 20180117 PL - United States TA - Neurol Neuroimmunol Neuroinflamm JT - Neurology(R) neuroimmunology & neuroinflammation JID - 101636388 PMC - PMC5773856 EDAT- 2018/01/24 06:00 MHDA- 2018/01/24 06:01 PMCR- 2018/01/17 CRDT- 2018/01/24 06:00 PHST- 2017/10/09 00:00 [received] PHST- 2017/11/17 00:00 [accepted] PHST- 2018/01/24 06:00 [entrez] PHST- 2018/01/24 06:00 [pubmed] PHST- 2018/01/24 06:01 [medline] PHST- 2018/01/17 00:00 [pmc-release] AID - NEURIMMINFL2017014530 [pii] AID - 10.1212/NXI.0000000000000434 [doi] PST - epublish SO - Neurol Neuroimmunol Neuroinflamm. 2018 Jan 17;5(2):e434. doi: 10.1212/NXI.0000000000000434. eCollection 2018 Mar. PMID- 24012055 OWN - NLM STAT- MEDLINE DCOM- 20150528 LR - 20131127 IS - 1532-1940 (Electronic) IS - 0266-4356 (Linking) VI - 51 IP - 8 DP - 2013 Dec TI - Facial calcinosis: case report. PG - e319-20 LID - S0266-4356(13)00355-0 [pii] LID - 10.1016/j.bjoms.2013.07.003 [doi] AB - Antisynthetase syndrome is a subgroup of the idiopathic inflammatory muscle diseases and is characterised by myositis, interstitial pulmonary disease, arthritis, and Raynaud phenomenon. We report the case of a 34-year-old woman with known antisynthetase syndrome who presented with subcutaneous calcinosis bilaterally in the submandibular region. CI - Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved. FAU - Craggs, Lorna AU - Craggs L AD - Croydon University Hospital, United Kingdom. Electronic address: lornacraggs@hotmail.com. FAU - Misir, Ahmet Ferhat AU - Misir AF FAU - Manisali, Mehmet AU - Manisali M LA - eng PT - Case Reports PT - Journal Article DEP - 20130903 PL - Scotland TA - Br J Oral Maxillofac Surg JT - The British journal of oral & maxillofacial surgery JID - 8405235 RN - Antisynthetase syndrome SB - IM MH - Adult MH - Calcinosis/*diagnosis MH - Facial Dermatoses/*diagnosis MH - Female MH - Humans MH - Myositis/*diagnosis MH - Skin Diseases/*diagnosis MH - Subcutaneous Tissue/pathology OTO - NOTNLM OT - Antisynthetase syndrome OT - Calcinosis OT - Face OT - Mandible EDAT- 2013/09/10 06:00 MHDA- 2015/05/29 06:00 CRDT- 2013/09/10 06:00 PHST- 2013/03/20 00:00 [received] PHST- 2013/07/12 00:00 [accepted] PHST- 2013/09/10 06:00 [entrez] PHST- 2013/09/10 06:00 [pubmed] PHST- 2015/05/29 06:00 [medline] AID - S0266-4356(13)00355-0 [pii] AID - 10.1016/j.bjoms.2013.07.003 [doi] PST - ppublish SO - Br J Oral Maxillofac Surg. 2013 Dec;51(8):e319-20. doi: 10.1016/j.bjoms.2013.07.003. Epub 2013 Sep 3. PMID- 23864866 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130718 LR - 20211021 IS - 1687-966X (Print) IS - 1687-9678 (Electronic) VI - 2013 DP - 2013 TI - The Necessity of a Systematic Approach for the Use of MSCs in the Clinical Setting. PG - 892340 LID - 10.1155/2013/892340 [doi] LID - 892340 AB - Cell therapy has emerged as a potential therapeutic strategy in regenerative disease. Among different cell types, mesenchymal stem/stromal cells have been wildly studied in vitro, in vivo in animal models and even used in clinical trials. However, while clinical applications continue to increase markedly, the understanding of their physiological properties and interactions raises many questions and drives the necessity of more caution and supervised strategy in their use. FAU - Raynaud, Christophe Michel AU - Raynaud CM AD - Qatar Cardiovascular Research Center, Qatar Foundation, Qatar Science and Technology Park, Doha, Qatar. FAU - Rafii, Arash AU - Rafii A LA - eng PT - Journal Article DEP - 20130623 PL - United States TA - Stem Cells Int JT - Stem cells international JID - 101535822 PMC - PMC3705875 EDAT- 2013/07/19 06:00 MHDA- 2013/07/19 06:01 PMCR- 2013/06/23 CRDT- 2013/07/19 06:00 PHST- 2013/04/21 00:00 [received] PHST- 2013/05/26 00:00 [revised] PHST- 2013/06/05 00:00 [accepted] PHST- 2013/07/19 06:00 [entrez] PHST- 2013/07/19 06:00 [pubmed] PHST- 2013/07/19 06:01 [medline] PHST- 2013/06/23 00:00 [pmc-release] AID - 10.1155/2013/892340 [doi] PST - ppublish SO - Stem Cells Int. 2013;2013:892340. doi: 10.1155/2013/892340. Epub 2013 Jun 23. PMID- 30500118 OWN - NLM STAT- MEDLINE DCOM- 20190128 LR - 20190128 IS - 1876-8784 (Electronic) IS - 0028-2162 (Linking) VI - 162 DP - 2018 Nov 27 TI - [Life-threatening respiratory insufficiency; rare primary manifestation of the antisynthetase syndrome]. LID - D2837 [pii] AB - The antisynthetase syndrome (AS) is a rare auto-immune disease characterised by inflammatory myopathies, interstitial lung disease, inflammatory arthritis, Mechanic's Hands and Raynaud phenomenon. AS infrequently presents with life-threatening lung disease as its primary or sole manifestation. By means of two clinical case reports, an overview is given of recent advances in diagnosis and treatment of AS-related interstitial lung disease. We emphasise the importance of considering AS as a differential diagnosis if lung disease does not respond to standard treatment in a relatively young patient, certainly if there is even a mild suggestion of myopathy, arthritis or cutaneous involvement. FAU - Betrains, Albrecht AU - Betrains A AD - Universitaire Ziekenhuizen Leuven, afd. Algemene Interne Geneeskunde, België. FAU - De Langhe, Ellen AU - De Langhe E AD - Universitaire Ziekenhuizen Leuven, afd. Reumatologie, België. FAU - De Saedeleer, Laurens AU - De Saedeleer L AD - Universitaire Ziekenhuizen Leuven, afd. Pneumologie, België. FAU - Vanderschueren, Steven AU - Vanderschueren S AD - Universitaire Ziekenhuizen Leuven, afd. Algemene Interne Geneeskunde, België. AD - Contact: S. Vanderschueren (steven.vanderschueren@uzleuven.be). LA - dut PT - Case Reports PT - Journal Article TT - Levensbedreigende respiratoire insufficiëntie. DEP - 20181127 PL - Netherlands TA - Ned Tijdschr Geneeskd JT - Nederlands tijdschrift voor geneeskunde JID - 0400770 RN - Antisynthetase syndrome SB - IM MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung/pathology MH - Male MH - Middle Aged MH - Myositis/complications/*diagnosis/therapy MH - Respiratory Insufficiency/diagnosis/*etiology/therapy EDAT- 2018/12/01 06:00 MHDA- 2019/01/29 06:00 CRDT- 2018/12/01 06:00 PHST- 2018/12/01 06:00 [entrez] PHST- 2018/12/01 06:00 [pubmed] PHST- 2019/01/29 06:00 [medline] AID - D2837 [pii] PST - epublish SO - Ned Tijdschr Geneeskd. 2018 Nov 27;162:D2837. PMID- 17035427 OWN - NLM STAT- MEDLINE DCOM- 20061120 LR - 20070502 IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 130 IP - 4 DP - 2006 Oct TI - Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. PG - 976-81 AB - STUDY OBJECTIVES: To describe the characteristics of systemic sclerosis sine scleroderma (ssSSc)-associated interstitial lung disease (ILD) presenting as idiopathic interstitial pneumonia (IIP). DESIGN: Retrospective review of six patients with ssSSc-associated ILD diagnosed after referral for evaluation of IIP. MEASUREMENT AND RESULTS: All patients were white, their mean age was 56 years (range, 37 to 86), and gender was evenly divided. Sclerodactyly, skin thickening, and digital edema were absent in all patients. All patients had scattered telangiectasia, and four patients had Raynaud phenomenon with abnormal nailfold capillaroscopy findings. All described gastroesophageal reflux, and three patients had esophageal dysmotility by esophagography. All had restrictive pulmonary physiology and a reduced diffusion capacity. High-resolution CT revealed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) radiographic patterns. Of the three patients who underwent surgical lung biopsy, two patients had NSIP and one patient had UIP pathologic patterns. Five patients had asymptomatic pericardial effusions and elevated pulmonary artery pressures by echocardiography. All patients had nucleolar-staining anti-nuclear antibodies (ANAs), and one patient was anti-Scl-70 positive. All five anti-Scl-70-negative patients were anti-Th/To positive, and the anti-Scl-70-positive patient was anti-Th/To negative. CONCLUSIONS: In the presentation of an IIP, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis. These findings should aid clinicians in the evaluation and treatment of patients with otherwise undefined ILD. FAU - Fischer, Aryeh AU - Fischer A AD - Division of Rheumatology, National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206, USA. fischera@njc.org FAU - Meehan, Richard T AU - Meehan RT FAU - Feghali-Bostwick, Carol A AU - Feghali-Bostwick CA FAU - West, Sterling G AU - West SG FAU - Brown, Kevin K AU - Brown KK LA - eng PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Autoantibodies) SB - IM CIN - Chest. 2007 Mar;131(3):940-941. doi: 10.1016/S0012-3692(15)38926-1. PMID: 17356120 MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibody Specificity/immunology MH - Autoantibodies/blood MH - Biopsy MH - Female MH - Humans MH - Lung/pathology MH - Lung Diseases, Interstitial/*diagnosis/immunology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/*diagnosis/immunology MH - Tomography, Spiral Computed EDAT- 2006/10/13 09:00 MHDA- 2006/12/09 09:00 CRDT- 2006/10/13 09:00 PHST- 2006/10/13 09:00 [pubmed] PHST- 2006/12/09 09:00 [medline] PHST- 2006/10/13 09:00 [entrez] AID - S0012-3692(15)51129-X [pii] AID - 10.1378/chest.130.4.976 [doi] PST - ppublish SO - Chest. 2006 Oct;130(4):976-81. doi: 10.1378/chest.130.4.976. PMID- 33401302 OWN - NLM STAT- MEDLINE DCOM- 20210823 LR - 20231107 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 60 IP - 8 DP - 2021 Aug 2 TI - Health-related quality of life in patients with systemic sclerosis: evolution over time and main determinants. PG - 3646-3655 LID - 10.1093/rheumatology/keaa827 [doi] AB - OBJECTIVES: In SSc patients, disease specific determinants that influence health-related quality of life (HRQoL) over time have not been described. We aim to, in patients with SSc, (i) evaluate if and how HRQoL changes over time, and (ii) assess how different SSc domains and functional impairments contribute to changes in HRQoL over time. METHODS: All SSc patients from the Leiden SSc cohort were included; patients with disease duration <24 months were classified as incident cases. HRQoL was assessed prospectively on an annual basis using the EQ-5D and the SF36. To assess baseline associations between clinical characteristics and HRQoL, linear regressions were performed. To identify possible associations between SSc characteristics and HRQoL change over time, linear mixed models were performed in both incident and prevalent cases. RESULTS: In total, 492 SSc patients were included (n = 202 incident cases), with a median follow-up duration of 3.4 years. At baseline, presence of organ involvement was independently associated with a worse SF36 physical component score and lower EQ-5D score. Over time, gastrointestinal symptoms, Raynaud and digital ulcers were independently associated with deterioration of HRQoL in both incident and prevalent cases. In prevalent cases, pulmonary arterial hypertension (PAH) was associated with a decrease in HRQoL over time. Worse functioning as measured by six-min walking distance, mouth-opening, finger-to-palm distance and grip-strength contributed significantly to deterioration of HRQoL over time. CONCLUSION: In SSc, key clinical burdens that contribute to worsening of HRQoL over time include digital ulcers, Raynaud and gastrointestinal involvement. In addition, PAH is a significant burden in prevalent disease. CI - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - van Leeuwen, Nina M AU - van Leeuwen NM AD - Rheumatology Department, Leiden University Medical Center, Leiden, The Netherlands. FAU - Ciaffi, Jacopo AU - Ciaffi J AD - Medicine and Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy. FAU - Liem, Sophie I E AU - Liem SIE AD - Rheumatology Department, Leiden University Medical Center, Leiden, The Netherlands. FAU - Huizinga, Tom W J AU - Huizinga TWJ AD - Rheumatology Department, Leiden University Medical Center, Leiden, The Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Rheumatology Department, Leiden University Medical Center, Leiden, The Netherlands. LA - eng PT - Journal Article PT - Observational Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Aged MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Netherlands/epidemiology MH - Prospective Studies MH - *Quality of Life MH - Scleroderma, Systemic/*epidemiology PMC - PMC8328503 OTO - NOTNLM OT - impairment OT - organ involvement OT - quality of life OT - systemic sclerosis EDAT- 2021/01/06 06:00 MHDA- 2021/08/24 06:00 PMCR- 2021/01/05 CRDT- 2021/01/05 20:14 PHST- 2020/07/27 00:00 [received] PHST- 2020/11/12 00:00 [revised] PHST- 2021/01/06 06:00 [pubmed] PHST- 2021/08/24 06:00 [medline] PHST- 2021/01/05 20:14 [entrez] PHST- 2021/01/05 00:00 [pmc-release] AID - 6064847 [pii] AID - keaa827 [pii] AID - 10.1093/rheumatology/keaa827 [doi] PST - ppublish SO - Rheumatology (Oxford). 2021 Aug 2;60(8):3646-3655. doi: 10.1093/rheumatology/keaa827. PMID- 29320840 OWN - NLM STAT- MEDLINE DCOM- 20180904 LR - 20180904 IS - 0048-7449 (Print) IS - 0048-7449 (Linking) VI - 69 IP - 4 DP - 2017 Dec 21 TI - Microvascular damage evaluation in systemic sclerosis: the role of nailfold videocapillaroscopy and laser techniques. PG - 147-155 LID - 10.4081/reumatismo.2017.959 [doi] AB - Microvascular damage and a decrease in peripheral blood perfusion are typical features of systemic sclerosis (SSc) with serious clinical implications, not only for a very early diagnosis, but also for disease progression. Nailfold videocapillaroscopy is a validated and safe imaging technique able to detect peripheral capillary morphology, as well as to classify and to score any nailfold abnormalities into different microangiopathy patterns. Capillaroscopic analysis is now included in the ACR/EULAR classification criteria for SSc. The decrease in peripheral blood perfusion is usually associated with microvascular damage in SSc, which may be studied by different methods. Several of these make use of safe laser technologies. This paper focuses on these new clinical aspects to assess SSc microvascular impairment. FAU - Ruaro, B AU - Ruaro B AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino-IST, Genova. barbara.ruaro@yahoo.it. FAU - Sulli, A AU - Sulli A FAU - Smith, V AU - Smith V FAU - Pizzorni, C AU - Pizzorni C FAU - Paolino, S AU - Paolino S FAU - Alessandri, E AU - Alessandri E FAU - Cutolo, M AU - Cutolo M LA - eng PT - Journal Article PT - Review DEP - 20171221 PL - Italy TA - Reumatismo JT - Reumatismo JID - 0401302 SB - IM MH - Capillaries/*ultrastructure MH - Humans MH - Laser-Doppler Flowmetry MH - Microcirculation MH - Microscopic Angioscopy/*methods MH - Microscopy, Video/*methods MH - Nails/*blood supply MH - Scleroderma, Systemic/diagnostic imaging/*pathology OTO - NOTNLM OT - Laser techniques. OT - Microcirculation OT - Nailfold videocapillaroscopy OT - Raynaud phenomenon OT - Systemic sclerosis EDAT- 2018/01/13 06:00 MHDA- 2018/09/05 06:00 CRDT- 2018/01/12 06:00 PHST- 2017/01/03 00:00 [received] PHST- 2017/07/28 00:00 [accepted] PHST- 2017/07/09 00:00 [revised] PHST- 2018/01/12 06:00 [entrez] PHST- 2018/01/13 06:00 [pubmed] PHST- 2018/09/05 06:00 [medline] AID - 10.4081/reumatismo.2017.959 [doi] PST - epublish SO - Reumatismo. 2017 Dec 21;69(4):147-155. doi: 10.4081/reumatismo.2017.959. PMID- 22973396 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121002 LR - 20211021 IS - 1735-7136 (Electronic) IS - 1735-1995 (Print) IS - 1735-1995 (Linking) VI - 16 IP - 9 DP - 2011 Sep TI - Rosai-Dorfman Disease with nodal and extranodal involvements: A case report. PG - 1251-6 AB - Rosai-Dorfman disease (RDD) is a rare lymphoproliferative disorder with nodal and extranodal involvements. Here we report a case of RDD in a 15-year-old female who presented with epigastric pain, fatigue, Raynaud phenomenon in fingers, submandibular lymphadenopathy, proptosis, hepatosplenomegaly, and round shape painless patches on the extensor surfaces. Histological examination of the submandibular lymph nodes and skin biopsy demonstrated evidences of RDD. Patient was treated with prednisone and thereafter, with azathioprine. After one year, prednisone was discontinued and all of the symptoms and signs, except proptosis, were resolved. This report highlights the extranodal manifestations of RDD. The presentation, differential diagnosis, and treatment are discussed. FAU - Najafi-Sani, Mehri AU - Najafi-Sani M AD - Associate Professor of Pediatric Gastroenterology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran. FAU - Saneian, Hossein AU - Saneian H FAU - Mahjoub, Fatemeh AU - Mahjoub F LA - eng PT - Case Reports PT - Journal Article PL - India TA - J Res Med Sci JT - Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences JID - 101235599 PMC - PMC3430052 OTO - NOTNLM OT - Lymphadenopathy OT - Rosai-Dorfman Disease OT - Sinus Histiocytosis COIS- Conflict of Interests Authors have no conflict of interests. EDAT- 2012/09/14 06:00 MHDA- 2012/09/14 06:01 PMCR- 2011/09/01 CRDT- 2012/09/14 06:00 PHST- 2011/02/10 00:00 [received] PHST- 2011/08/29 00:00 [accepted] PHST- 2012/09/14 06:00 [entrez] PHST- 2012/09/14 06:00 [pubmed] PHST- 2012/09/14 06:01 [medline] PHST- 2011/09/01 00:00 [pmc-release] AID - JRMS-16-1251 [pii] PST - ppublish SO - J Res Med Sci. 2011 Sep;16(9):1251-6. PMID- 38963060 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260526 LR - 20260526 IS - 2042-6313 (Electronic) IS - 2042-6305 (Print) IS - 2042-6305 (Linking) VI - 13 IP - 7 DP - 2024 Jul TI - Evaluation of inpatient and emergency department healthcare resource utilization and costs pre- and post-nusinersen for the treatment of spinal muscular atrophy using United States claims. PG - e230187 LID - 10.57264/cer-2023-0187 [doi] LID - e230187 AB - Aim: Nusinersen, administered by intrathecal injection at a dose of 12 mg, is indicated across all ages for the treatment of spinal muscular atrophy (SMA). Evidence on real-world healthcare resource use (HRU) and costs among patients taking nusinersen remains limited. This study aimed to evaluate real-world HRU and costs associated with nusinersen use through US claims databases. Patients & methods: Using the Merative™ MarketScan(®) Research Databases, patients with SMA receiving nusinersen were identified from commercial (January 2017 to June 2020) and Medicaid claims (January 2017 to December 2019). Those likely to have complete information on the date of nusinersen initiation and continuous enrollment 12 months pre- and post-index (first record of nusinersen treatment) were retained. Number and costs (US$ 2020) of inpatient admissions and emergency department (ED) visits, unrelated to nusinersen administration, were evaluated for 12 months pre- and post-nusinersen initiation and stratified by age: pediatric (<18 years) and adult (≥18 years). Results: Overall, 103 individuals treated with nusinersen were retained: 59 were pediatric (mean age [range]: 9 [1-17] years), and 44 were adults (30 [18-63] years). Inpatient admissions decreased by 41% for pediatrics and 67% for adults in the 12 months post-treatment versus the 12 months pre-treatment. Average inpatient admission costs per patient for the pediatric cohort decreased by 63% ($22,903 vs $8466) and by 79% ($13,997 vs $2899) for the adult cohort when comparing the 12 months pre-index with the 12 months post-index period. Total ED visits and ED visit costs decreased by 8% and 35%, respectively, for the overall cohort over the 12-month period pre- and post-index. Conclusion: Using US claims databases, nusinersen treatment in pediatric and adult patients was associated with reductions in HRU and costs over a 12-month period post-treatment initiation relative to the pre-treatment period. FAU - Zhu, Cong AU - Zhu C AD - Biogen, Cambridge, MA 02142, USA. FAU - Zaidman, Craig AU - Zaidman C AD - Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA. FAU - Youn, Bora AU - Youn B AD - Biogen, Cambridge, MA 02142, USA. FAU - Paradis, Angela D AU - Paradis AD AD - Biogen, Cambridge, MA 02142, USA. FAU - Raynaud, Stephanie AU - Raynaud S AD - Biogen, Cambridge, MA 02142, USA. FAU - Neville, Bridget A AU - Neville BA AD - Biogen, Cambridge, MA 02142, USA. FAU - Johnson, Nicole B AU - Johnson NB AD - Biogen, Cambridge, MA 02142, USA. LA - eng PT - Journal Article DEP - 20240704 PL - England TA - J Comp Eff Res JT - Journal of comparative effectiveness research JID - 101577308 SB - IM PMC - PMC11225157 OAB - What is this article about/what is the aim of the research? Spinal muscular atrophy (SMA) is an inherited disease affecting mainly infants and children, but it can also present in adults. This condition damages nerve cells and muscles that control key activities, such as sitting, walking, speaking, swallowing and breathing. Nusinersen was the first drug approved in the US to help manage SMA. It is administered by injection with a thin needle into a space in the lower back, below the end of the spinal cord, through a medical procedure known as a “lumbar puncture”. There is very little information on the medical management for people with SMA receiving nusinersen. Using insurance claims processed in the US, this study assessed trends in hospital stays, in emergency department (ED) visits and in related medical costs before and after nusinersen use. What were the results? Comparing the 12 months before nusinersen treatment with the 12 months following treatment, the number of hospital stays was 41% lower for children and 67% lower for adults after treatment. The average cost per person associated with hospital stays was 63% lower for children and 79% lower for adults. The number of days spent at the hospital was also reduced for all ages following nusinersen treatment. The number of ED visits and the resulting costs were 8% and 35% lower, respectively, for the overall cohort. What do these results mean? Overall, nusinersen treatment lessens the burden of disease among people with SMA in the US by lowering the rate of hospital stays and ED visits, and by reducing associated costs. OABL- eng OTO - NOTNLM OT - commercial and Medicaid claims OT - healthcare resource utilization OT - medical cost offsets OT - nusinersen OT - patient-level claims OT - spinal muscular atrophy COIS- Competing interests disclosure The authors have no other competing interests or relevant affiliations with any organization or entity with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. EDAT- 2024/07/04 12:42 MHDA- 2024/07/04 12:43 PMCR- 2024/07/04 CRDT- 2024/07/04 06:54 PHST- 2024/07/04 12:43 [medline] PHST- 2024/07/04 12:42 [pubmed] PHST- 2024/07/04 06:54 [entrez] PHST- 2024/07/04 00:00 [pmc-release] AID - 10.57264/cer-2023-0187 [doi] PST - ppublish SO - J Comp Eff Res. 2024 Jul;13(7):e230187. doi: 10.57264/cer-2023-0187. Epub 2024 Jul 4. PMID- 19932330 OWN - NLM STAT- MEDLINE DCOM- 20091215 LR - 20091125 IS - 1557-9859 (Electronic) IS - 0025-7125 (Linking) VI - 93 IP - 6 DP - 2009 Nov TI - Skin manifestations of internal disease. PG - 1265-82 LID - 10.1016/j.mcna.2009.08.010 [doi] AB - Internal diseases can manifest in a myriad of skin dermatoses ranging from single disorders such as calciphylaxis, cryoglobulinemia, amyopathic dermatomyositis, and Raynaud phenomenon, to spectrum disorders such as the neutrophilic dermatoses and morphea. In this article the underlying causes, triggering events, constitutional symptoms, clinical features and presentations, appearance at various stages, and pathogenesis are described. The course of the diseases and probable healing outcomes are outlined. Finally, examination and diagnostic methods, and therapies and treatments are provided. FAU - Franks, Andrew G Jr AU - Franks AG Jr AD - Department of Dermatology, New York University School of Medicine, 550 First Avenue, New York, NY 10016, USA. andrew.franks@nyumc.org LA - eng PT - Journal Article PT - Review PL - United States TA - Med Clin North Am JT - The Medical clinics of North America JID - 2985236R SB - IM MH - Diagnosis, Differential MH - Humans MH - *Skin Diseases/diagnosis/etiology/therapy RF - 66 EDAT- 2009/11/26 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/11/26 06:00 PHST- 2009/11/26 06:00 [entrez] PHST- 2009/11/26 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - S0025-7125(09)00112-6 [pii] AID - 10.1016/j.mcna.2009.08.010 [doi] PST - ppublish SO - Med Clin North Am. 2009 Nov;93(6):1265-82. doi: 10.1016/j.mcna.2009.08.010. PMID- 26260763 OWN - NLM STAT- MEDLINE DCOM- 20160122 LR - 20181202 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 11 IP - 10 DP - 2015 Oct TI - Therapy. Optimized treatment algorithms for digital vasculopathy in SSc. PG - 569-71 LID - 10.1038/nrrheum.2015.111 [doi] AB - Early identification of secondary Raynaud phenomenon is essential to treat the underlying disease—most frequently systemic sclerosis (SSc). Integrated therapeutic approaches and monitoring systems that offer improved modalities of care feature in the new best practice recommendations for the treatment of digital vasculopathy in SSc. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratories and Academic Division of Clinical Rheumatology, Director Postgraduate School on Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. FAU - Sulli, Alberto AU - Sulli A AD - Research Laboratories and Academic Division of Clinical Rheumatology, Director Postgraduate School on Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, 6, 16132 Genova, Italy. LA - eng PT - Comment PT - Journal Article DEP - 20150811 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM CON - Rheumatology (Oxford). 2015 Nov;54(11):2015-24. doi: 10.1093/rheumatology/kev201. PMID: 26116156 MH - Fingers/*blood supply MH - Humans MH - *Practice Patterns, Physicians' MH - Scleroderma, Systemic/*complications MH - Vascular Diseases/*etiology/*therapy EDAT- 2015/08/12 06:00 MHDA- 2016/01/23 06:00 CRDT- 2015/08/12 06:00 PHST- 2015/08/12 06:00 [entrez] PHST- 2015/08/12 06:00 [pubmed] PHST- 2016/01/23 06:00 [medline] AID - nrrheum.2015.111 [pii] AID - 10.1038/nrrheum.2015.111 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2015 Oct;11(10):569-71. doi: 10.1038/nrrheum.2015.111. Epub 2015 Aug 11. PMID- 21066870 OWN - NLM STAT- MEDLINE DCOM- 20101217 LR - 20181201 IS - 1343-3490 (Print) IS - 1343-3490 (Linking) VI - 48 IP - 10 DP - 2010 Oct TI - [Efficacy of combination therapy with bosentan and sildenafil for refractory pulmonary arterial hypertension associated with fibrotic lung in systemic sclerosis]. PG - 786-90 AB - A 54-year-old woman with a 20-year history of Raynaud phenomenon was admitted to our hospital complaining of progressive dyspnea on exertion since 5 years previously. Interstitial lung disease was diagnosed, accompanied by pulmonary arterial hypertension (PAH) associated with systemic sclerosis. After oxygen therapy and treatment with sildenafil, her clinical condition and PAH gradually improved. However, she was readmitted due to deterioration of Raynaud phenomenon and progressive dyspnea in March 2009. Right heart catheterization findings demonstrated that her mean pulmonary arterial pressure (PAP) was elevated, at 48 mmHg. Bosentan was therefore added to an increased dose of sildenafil. Consequently, her dyspnea, 6-min walking distance, serum brain natriuretic peptide level, and PAP improved. Combination therapy with bosentan and sildenafil was effective for this case of refractory PAH associated with fibrotic lung in systemic sclerosis. FAU - Gocho, Kyoko AU - Gocho K AD - Department of Respiratory Medicine, Toho University Omori Medical Center. FAU - Sugino, Keishi AU - Sugino K FAU - Ota, Hiroki AU - Ota H FAU - Kusano, Emiko AU - Kusano E FAU - Takai, Yujiro AU - Takai Y FAU - Homma, Sakae AU - Homma S LA - jpn PT - Case Reports PT - Journal Article PL - Japan TA - Nihon Kokyuki Gakkai Zasshi JT - Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society JID - 9808802 RN - 0 (Antihypertensive Agents) RN - 0 (Piperazines) RN - 0 (Purines) RN - 0 (Sulfonamides) RN - 0 (Sulfones) RN - 0 (Vasodilator Agents) RN - BW9B0ZE037 (Sildenafil Citrate) RN - Q326023R30 (Bosentan) SB - IM MH - Antihypertensive Agents/*administration & dosage MH - Bosentan MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy/etiology MH - Lung Diseases, Interstitial/etiology MH - Middle Aged MH - Piperazines/*administration & dosage MH - Purines/administration & dosage MH - Scleroderma, Systemic/*complications MH - Sildenafil Citrate MH - Sulfonamides/*administration & dosage MH - Sulfones/*administration & dosage MH - Vasodilator Agents/*therapeutic use EDAT- 2010/11/12 06:00 MHDA- 2010/12/18 06:00 CRDT- 2010/11/12 06:00 PHST- 2010/11/12 06:00 [entrez] PHST- 2010/11/12 06:00 [pubmed] PHST- 2010/12/18 06:00 [medline] PST - ppublish SO - Nihon Kokyuki Gakkai Zasshi. 2010 Oct;48(10):786-90. PMID- 22594010 OWN - NLM STAT- MEDLINE DCOM- 20120619 LR - 20161125 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 8 IP - 337 DP - 2012 Apr 18 TI - [Eosinophllic fasciitis (Shulman disease)]. PG - 854-8 AB - Eosinophilic fasciitis is a rare connective tissue disease, described by Shulman in 1974. This syndrome is characterized by a symmetrical swelling of the skin associated with eosinophilia. A progressive induration of the skin replaces the swelling. Arms and legs are the most affected sites. The face and hands are usually not involved and the patients don't complain of Raynaud phenomenon. No circulating autoantibodies are found. Diagnosis is made with history, MRI and histology. MRI detects fascial thickening and contrast enhancement of this fascia. A full thickness skin to muscle biopsy is necessary to confirm the diagnosis. It shows an inflammation and thickening of the fascia with lymphocytes and plasmocytes. High doses of corticosteroids are reported to be effective in more than 70% of the cases. Immunosuppressive drugs are sometimes necessary to induce clinical remission. FAU - Arlettaz, L AU - Arlettaz L AD - Service de génétique et d'immunologie, Institut Central/Hôpital du Valais. lionel.arlettaz@hopitalvs.ch FAU - Abdou, M AU - Abdou M FAU - Pardon, F AU - Pardon F FAU - Dayer, E AU - Dayer E LA - fre PT - Journal Article TT - Fasciite à eosinophiles ou syndrome de Shulman. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - Eosinophilic synovitis SB - IM MH - Eosinophilia MH - Glucocorticoids/therapeutic use MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Prognosis MH - Synovitis/*diagnosis/*drug therapy/etiology EDAT- 2012/05/19 06:00 MHDA- 2012/06/20 06:00 CRDT- 2012/05/19 06:00 PHST- 2012/05/19 06:00 [entrez] PHST- 2012/05/19 06:00 [pubmed] PHST- 2012/06/20 06:00 [medline] PST - ppublish SO - Rev Med Suisse. 2012 Apr 18;8(337):854-8. PMID- 26040161 OWN - NLM STAT- MEDLINE DCOM- 20150619 LR - 20150604 IS - 1660-9379 (Print) IS - 1660-9379 (Linking) VI - 11 IP - 469 DP - 2015 Apr 8 TI - [The anti-synthetases syndrome: diagnostic and treatments]. PG - 808-12 AB - The anti-synthetases syndrome is a rare disease with a specific constellation of clinical symptoms present in a subset of patients with inflammatory myopathy. Besides constitutional symptoms and myositis, it is associated with mechanic's hands, Raynaud phenomenon, and non-erosive arthritis. This syndrome is characterized by the presence of one of eight auto-antibodies to aminoacyl-transfer ribonucleic acid synthetase enzymes in the serum. Interstitial lung disease is more frequent in this subpopulation of inflammatory myopathy and worsens the patient's prognosis. FAU - Allali, D AU - Allali D FAU - Seebach, J D AU - Seebach JD LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Syndrome des antisynthétases: diagnostic et traitements. PL - Switzerland TA - Rev Med Suisse JT - Revue medicale suisse JID - 101219148 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Autoantibodies/*immunology MH - Humans MH - Lung Diseases, Interstitial/epidemiology/*etiology MH - Male MH - Myositis/diagnosis/physiopathology/*therapy MH - Prognosis EDAT- 2015/06/05 06:00 MHDA- 2015/06/20 06:00 CRDT- 2015/06/05 06:00 PHST- 2015/06/05 06:00 [entrez] PHST- 2015/06/05 06:00 [pubmed] PHST- 2015/06/20 06:00 [medline] PST - ppublish SO - Rev Med Suisse. 2015 Apr 8;11(469):808-12. PMID- 25973980 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 1875-533X (Electronic) IS - 0929-8673 (Linking) VI - 22 IP - 16 DP - 2015 May 13 TI - Antisynthetase syndrome: a review of etiopathogenesis, diagnosis and management. PG - 1963 - 75 AB - Antisynthetase syndrome is a group of closely related rare diseases which clinically manifest with inflammatory myopathies, interstitial lung disease, inflammatory arthritis, skin hyperkeratosis (mechanic’s hands) and Raynaud phenomenon. The pathophysiology of antisynthetase syndrome is not entirely understood, but genetic predisposition, viral infections and medication use may play a role. Certain antisynthetase antibodies are associated with various clinical presentations and a lower burden of inflammatory myopathies. Patients with antisynthetase syndrome have a worse prognosis than patients with pure inflammatory myopathies mainly because of interstitial lung disease. Future research should further investigate the pathogenesis of antisynthetase syndrome which could identify new therapeutic targets. It will be also important to study whether patients with AS are at increased risk of cancer and whether certain antisynthetase antibodies have any association with the risk of malignancy. FAU - Mirrakhimov, Aibek E AU - Mirrakhimov AE AD - Saint Joseph Hospital, Department of Internal Medicine, 2900 N. Lake Shore, Chicago, Illinois 60657, USA. amirrakhimov1@gmail.com. LA - eng PT - Journal Article PL - United Arab Emirates TA - Curr Med Chem JT - Current medicinal chemistry JID - 9440157 EDAT- 2015/05/15 06:00 MHDA- 2015/05/15 06:01 CRDT- 2015/05/15 06:00 PHST- 2015/01/14 00:00 [received] PHST- 2015/01/25 00:00 [revised] PHST- 2015/05/05 00:00 [accepted] PHST- 2015/05/15 06:00 [entrez] PHST- 2015/05/15 06:00 [pubmed] PHST- 2015/05/15 06:01 [medline] AID - CMC-EPUB-67284 [pii] PST - ppublish SO - Curr Med Chem. 2015 May 13;22(16):1963 - 75. PMID- 38337811 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240212 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 14 IP - 3 DP - 2024 Jan 30 TI - The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis-Interstitial Lung Disease in the European Scleroderma Trial and Research Database. LID - 10.3390/diagnostics14030295 [doi] LID - 295 AB - BACKGROUND AND OBJECTIVES: In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD. METHODS: Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t(0)) and after 12 (±4) (t(1)) and 24 (±4) (t(2)) months. RESULTS: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t(2). Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t(2) (p = 0.0031). Neither the mean t(0) to t(1) change (Δ) of DLCO nor the mean t(0) to t(1) FVCΔ predicted the appearance of ILD at t(2). Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (±4) months, we observed a moderate predictive capability of t(0) DLCO < 80%, stronger than that of FVC < 80%. CONCLUSIONS: We suggest that an impaired baseline DLCO may be predictive of the appearance of ILD after 2 years of follow-up. This result advances the hypothesis that a reduction in gas exchange may be considered an early sign of lung involvement. However, further rigorous studies are warranted to understand the predictive role of DLCO evaluation in the course of SSc. FAU - Lepri, Gemma AU - Lepri G AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Bruni, Cosimo AU - Bruni C AUID- ORCID: 0000-0003-2813-2083 AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland. FAU - Tofani, Lorenzo AU - Tofani L AUID- ORCID: 0000-0001-9965-7667 AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Moggi-Pignone, Alberto AU - Moggi-Pignone A AD - Division of Internal Medicine, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Orlandi, Martina AU - Orlandi M AUID- ORCID: 0000-0001-6784-2235 AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Tomassetti, Sara AU - Tomassetti S AD - Interventional Pulmonology Unit, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Hughes, Michael AU - Hughes M AD - Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Royal Hallamshire Hospital, Sheffield S10 2JF, UK. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS2 9JT, UK. FAU - Irace, Rosaria AU - Irace R AD - Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", 81100 Naples, Italy. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland. FAU - Riccieri, Valeria AU - Riccieri V AD - Rheumatology Unit, "Sapienza" University, 00185 Rome, Italy. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology Department, Hopital Cochin, University of Paris, 75019 Paris, France. FAU - Gheorghiu, Ana Maria AU - Gheorghiu AM AUID- ORCID: 0000-0002-1292-2964 AD - Internal Medicine & Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania. FAU - Siegert, Elise AU - Siegert E AUID- ORCID: 0000-0002-9594-0446 AD - Rheumatology, Charite University Hospital, 10117 Berlin, Germany. FAU - De Vries-Bouwstra, Jeska AU - De Vries-Bouwstra J AD - Department of Rheumatology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands. FAU - Hachulla, Eric AU - Hachulla E AD - Service de Médecine Interne, Centre Hospitalier Universitaire, 59000 Lille, France. FAU - Tikly, Mohammed AU - Tikly M AD - Department of Internal Medicine, Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg 1864, South Africa. FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, University Belgrade Medical School, 11000 Belgrade, Serbia. FAU - Spertini, Francois AU - Spertini F AD - Service Immunologie et Allergie, CHUV, 1005 Lausanne, Switzerland. FAU - Mouthon, Luc AU - Mouthon L AD - National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, University Paris Descartes, 75006 Paris, France. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AD - Department of Rheumatology, Oslo University Hospital, 0372 Oslo, Norway. FAU - Gabrielli, Armando AU - Gabrielli A AD - Department of Clinical and Molecular Science, Università Politecninca delle Marche, 60121 Ancona, Italy. FAU - Guiducci, Serena AU - Guiducci S AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy. FAU - Furst, Daniel AU - Furst D AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. AD - Division of Rheumatology, University California Los Angeles, Los Angeles, CA 90095, USA. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy. FAU - Eustar Collaborators AU - Eustar Collaborators LA - eng PT - Journal Article DEP - 20240130 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10855256 OTO - NOTNLM OT - computed tomography (CT) OT - interstitial lung disease OT - pulmonary function test OT - scleroderma COIS- Gemma Lepri: none declared. Cosimo Bruni: C.B. is a Guest Editor for Diagnostics (Special Issue “Advances in Identification and Management of Systemic Sclerosis”); C.B. reports personal fees from Boehringer Ingelheim, grants from the European Scleroderma Trial and Research (EUSTAR) group, grants from the New Horizon Fellowship, grants from the Foundation for Research in Rheumatology (FOREUM), grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), and grants from the Scleroderma Clinical Trial Consortium (SCTC) and the Scleroderma Research Foundation (SRF) outside the submitted work. Lorenzo Tofani: none declared. Alberto Moggi Pignone: none declared. Martina Orlandi: none declared. Tomasetti Sara: speaker’s fees from Roche and Boehringer Ingelheim. Michael Hughes: none declared. Francesco Del Galdo: none declared. Rosaria Irace: none declared. Oliver Distler O.D. (last three years) has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143). Valeria Riccieri: none declared. Yannick Allanore received personal fees from Boehringer, Sanofi, Menarini, and Medsenic and grants from Alpine with regard to the management of systemic sclerosis. Ana Maria Gheorghiu: none declared. Elise Siegert: none declared. Jeska de Vries-Bouwstra: none declared. Eric Hachulla: none declared. Mohammed Tikly: none declared. Nemanja Damjanov: none declared. Francois Spertini: none declared. Luc Mouthon: none declared. Anna-Maria Hoffmann-Vold received consulting fees from Actelion, ARXX, Bayer, Boehringer Ingelheim, Lilly, Medscape, Merck Sharp & Dohme, and Roche, and grants from Boehringer Ingelheim. Armando Gabrielli: none declared. Serena Guiducci: none declared. Marco Matucci-Cerinic has received consulting fees or an honorarium from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, and Roche. Daniel Furst: none declared. Silvia Bellando Randone: none declared. EDAT- 2024/02/10 10:42 MHDA- 2024/02/10 10:43 PMCR- 2024/01/30 CRDT- 2024/02/10 01:05 PHST- 2023/12/04 00:00 [received] PHST- 2023/12/27 00:00 [revised] PHST- 2024/01/04 00:00 [accepted] PHST- 2024/02/10 10:43 [medline] PHST- 2024/02/10 10:42 [pubmed] PHST- 2024/02/10 01:05 [entrez] PHST- 2024/01/30 00:00 [pmc-release] AID - diagnostics14030295 [pii] AID - diagnostics-14-00295 [pii] AID - 10.3390/diagnostics14030295 [doi] PST - epublish SO - Diagnostics (Basel). 2024 Jan 30;14(3):295. doi: 10.3390/diagnostics14030295. PMID- 33330522 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240804 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 7 DP - 2020 TI - Genetic Susceptibility to Antisynthetase Syndrome Associated With Single-Nucleotide Variants in the IL1B Gene That Lead Variation in IL-1β Serum Levels. PG - 547186 LID - 10.3389/fmed.2020.547186 [doi] LID - 547186 AB - The antisynthetase syndrome (ASSD) is an autoimmune disorder characterized by myositis, arthritis, mechanic's hands, fever, Raynaud phenomenon, and interstitial lung disease (ILD). We aimed to evaluate single-nucleotide polymorphisms in the interleukin 1B (IL1B) gene and their association between ILD with antisynthetase autoantibodies, as well as IL-1β serum levels. The most frequent antisynthetase autoantibody was anti-Jo1. The most frequent tomographic pattern was non-specific interstitial pneumonia, whereas in the anti-Jo1 subjects, it was organized pneumonia. Anti-Jo1 patients tend to have more significant arthritis, and Raynaud phenomenon have higher levels of creatinine phosphokinase. In the IL1B gene, the GG genotype and G allele of rs1143634 [odds ratio (OR) = 2.21 and OR = 2.60, respectively, p < 0.05] are associated with an increased risk, as well as with the dominant and recessive models (p < 0.05). This finding is maintained after logistic regression analysis adjusting for potential confounding variables (p < 0.05). Subjects with the rs16944/AG heterozygous genotype had higher serum levels of IL-1β compared to homozygous (p < 0.05). In conclusion, rs1143634 is associated with a higher risk of ASSD. Also, the GA genotype is associated with higher levels of IL-1β in ASSD patients. CI - Copyright © 2020 Ponce-Gallegos, Ramos-Martínez, García-Carmona, Mejía, Nava-Quiroz, Pérez-Rubio, Ambrocio-Ortiz, González-Pérez, Buendía-Roldán, Rojas-Serrano and Falfán-Valencia. FAU - Ponce-Gallegos, Marco Antonio AU - Ponce-Gallegos MA AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Ramos-Martínez, Espiridión AU - Ramos-Martínez E AD - Unidad de Medicina Experimental, Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico City, Mexico. FAU - García-Carmona, Adriana AU - García-Carmona A AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Mejía, Mayra AU - Mejía M AD - Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Nava-Quiroz, Karol J AU - Nava-Quiroz KJ AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Pérez-Rubio, Gloria AU - Pérez-Rubio G AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Ambrocio-Ortiz, Enrique AU - Ambrocio-Ortiz E AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - González-Pérez, Montserrat I AU - González-Pérez MI AD - Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Buendía-Roldán, Ivette AU - Buendía-Roldán I AD - Translational Research Laboratory on Aging and Pulmonary Fibrosis, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AD - Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. FAU - Falfán-Valencia, Ramcés AU - Falfán-Valencia R AD - HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico. LA - eng PT - Journal Article DEP - 20201124 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC7732678 OTO - NOTNLM OT - IL1-beta OT - IL1B gene OT - SNP OT - anti-Jo1 OT - antisynthetase syndrome OT - genetic association EDAT- 2020/12/18 06:00 MHDA- 2020/12/18 06:01 PMCR- 2020/11/24 CRDT- 2020/12/17 05:54 PHST- 2020/05/26 00:00 [received] PHST- 2020/10/12 00:00 [accepted] PHST- 2020/12/17 05:54 [entrez] PHST- 2020/12/18 06:00 [pubmed] PHST- 2020/12/18 06:01 [medline] PHST- 2020/11/24 00:00 [pmc-release] AID - 10.3389/fmed.2020.547186 [doi] PST - epublish SO - Front Med (Lausanne). 2020 Nov 24;7:547186. doi: 10.3389/fmed.2020.547186. eCollection 2020. PMID- 40035201 OWN - NLM STAT- MEDLINE DCOM- 20250304 LR - 20250510 IS - 2084-4336 (Electronic) IS - 1509-3492 (Linking) VI - 26 IP - 4 DP - 2024 Aug 31 TI - Hand Arm Vibration Syndrome [HAVS]: What Do We Know So Far? - Journal Review. PG - 121-130 LID - 10.5604/01.3001.0054.7351 [doi] AB - BACKGROUND: Since over century it has been known that hand to arm transmitted vibrations (HAV) can affect health and quality of life. Exposure over certain level and intensity over time are risk causes of Hand Arm Vibration Syndrome (HAVS). Symptoms varies from the damaged structures which most commonly are secondary Raynaud Phenomenon known as Vibration Induced White Finger (VWF), carpal tunnel syndrome, tingling, loss of manual dexterity, osteoarthritis or even Dupuytren's contracture. The usage of pneumatic vibrating tools among physical workers and specialists like dentists, without any doubt has grown significantly since the start of previous technological era. Unfortunately, development of medicine is not sufficient to reverse harmful effects of vibrations on human body but is able to diagnose and treat symptoms faster and with better efficiency than it used to in the past. Brief update and awareness booster of occupational disease which is silently present in our society, giving more insight and knowledge about current possibilities of treatment and diagnosis for medical personnel and everyone interested. MATERIAL AND METHODS: The article presents actual state of knowledge and scientific discoveries collected and researched via databases of platforms: Journal of Education Health and Sport, PubMed, Google Scholar, National Library of Medicine, Elsevier. Used keywords in research: Vibration induced White Finger, HAVS, Hand Arm Vibration Syndrome. CONCLUSION: While full protection from occupational vibrations is not possible, we should advance in spreading knowledge of occupational hazards, updating and regulating work standards policies, as continue research in new aimed therapies and effects of vibrations on human body. FAU - Popiołek, Adam AU - Popiołek A AD - Uniwersytet Medyczny w Łodzi, Polska / Medical University of Lodz, Poland. FAU - Billewicz, Marta AU - Billewicz M AD - Powiatowe Centrum Zdrowia w Brzezinach, Polska / The District Health Center in Brzeziny, Poland. FAU - Lis, Laura AU - Lis L AD - Uniwersytet Medyczny w Łodzi, Polska / Medical University of Lodz, Poland. FAU - Makowska, Karolina AU - Makowska K AD - Szpital Miejski św. Jana Pawła II w Elblągu, Polska / St. John Paul II Municipal Hospital in Elblag, Poland. FAU - Marczyk, Aleksandra AU - Marczyk A AD - Szpital Miejski św. Jana Pawła II w Elblągu, Polska / St. John Paul II Municipal Hospital in Elblag, Poland. FAU - Pietrzykowska, Julia AU - Pietrzykowska J AD - Uniwersytet Medyczny w Łodzi, Polska / Medical University of Lodz, Poland. FAU - Turek, Aleksandra AU - Turek A AD - Powiatowe Centrum Medyczne w Grójcu, Polska / The District Medical Centre in Grójec, Poland. FAU - Zatorska, Oksana AU - Zatorska O AD - Szpital Miejski św. Jana Pawła II w Elblągu, Polska / St. John Paul II Municipal Hospital in Elblag, Poland. LA - eng PT - Journal Article PT - Review PL - Poland TA - Ortop Traumatol Rehabil JT - Ortopedia, traumatologia, rehabilitacja JID - 101240146 SB - IM MH - Humans MH - *Hand-Arm Vibration Syndrome/diagnosis/therapy/etiology/physiopathology MH - *Vibration/adverse effects MH - *Occupational Diseases/etiology MH - *Occupational Exposure/adverse effects MH - Male MH - Female OTO - NOTNLM OT - HAVS OT - HAVS review OT - Raynaud phenomenon OT - Vibration EDAT- 2025/03/04 06:22 MHDA- 2025/03/04 12:24 CRDT- 2025/03/04 05:52 PHST- 2025/03/04 12:24 [medline] PHST- 2025/03/04 06:22 [pubmed] PHST- 2025/03/04 05:52 [entrez] AID - 01.3001.0054.7351 [pii] AID - 10.5604/01.3001.0054.7351 [doi] PST - ppublish SO - Ortop Traumatol Rehabil. 2024 Aug 31;26(4):121-130. doi: 10.5604/01.3001.0054.7351. PMID- 20805294 OWN - NLM STAT- MEDLINE DCOM- 20110318 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Print) IS - 0003-4967 (Linking) VI - 70 IP - 1 DP - 2011 Jan TI - Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. PG - 32-8 LID - 10.1136/ard.2010.130658 [doi] AB - OBJECTIVES: Ischaemic digital ulcers (DUs) are common in patients with systemic sclerosis (SSc) and are a cause of disease-related morbidity. In an earlier trial, treatment with bosentan, an oral endothelin receptor antagonist, reduced the occurrence of new DUs by 48%. The present study (RAPIDS-2, for 'RAndomized, double-blind, Placebo-controlled study with bosentan on healing and prevention of Ischemic Digital ulcers in patients with systemic Sclerosis') was conducted to more fully evaluate the effects of bosentan treatment on DUs associated with SSc. METHODS: This double-blind, placebo-controlled trial conducted at 41 centres in Europe and North America randomised 188 patients with SSc with at least 1 active DU ('cardinal ulcer') to bosentan 62.5 mg twice daily for 4 weeks and 125 mg twice daily thereafter for 20 weeks (n=98) or matching placebo (n=90; total 24 weeks). The two primary end points were the number of new DUs and the time to healing of the cardinal ulcer. Secondary end points included pain, disability and safety. RESULTS: Over 24 weeks, bosentan treatment was associated with a 30% reduction in the number of new DUs compared with placebo (mean ± standard error: 1.9±0.2 vs 2.7±0.3 new ulcers; p=0.04). This effect was greater in patients who entered the trial with more DUs. There was no difference between treatments in healing rate of the cardinal ulcer or secondary end points of pain and disability. Peripheral oedema and elevated aminotransferases were associated with bosentan treatment. CONCLUSIONS: Bosentan treatment reduced the occurrence of new DUs in patients with SSc but had no effect on DU healing. Bosentan was well tolerated and may be a useful adjunct in the management of patients with SSc with recurrent DUs. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Biomedicine, Division of Rheumatology, DENOTHE Center, University of Florence, Florence, Italy. cerinic@unifi.it FAU - Denton, Christopher P AU - Denton CP FAU - Furst, Daniel E AU - Furst DE FAU - Mayes, Maureen D AU - Mayes MD FAU - Hsu, Vivien M AU - Hsu VM FAU - Carpentier, Patrick AU - Carpentier P FAU - Wigley, Fredrick M AU - Wigley FM FAU - Black, Carol M AU - Black CM FAU - Fessler, Barri J AU - Fessler BJ FAU - Merkel, Peter A AU - Merkel PA FAU - Pope, Janet E AU - Pope JE FAU - Sweiss, Nadera J AU - Sweiss NJ FAU - Doyle, Mittie K AU - Doyle MK FAU - Hellmich, Bernhard AU - Hellmich B FAU - Medsger, Thomas A Jr AU - Medsger TA Jr FAU - Morganti, Adele AU - Morganti A FAU - Kramer, Fabrice AU - Kramer F FAU - Korn, Joseph H AU - Korn JH FAU - Seibold, James R AU - Seibold JR LA - eng GR - M01 RR001066/RR/NCRR NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20100830 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Sulfonamides) RN - Q326023R30 (Bosentan) SB - IM MH - Adult MH - Bosentan MH - Double-Blind Method MH - Drug Administration Schedule MH - Endothelin Receptor Antagonists MH - Female MH - Fingers/*blood supply MH - Hand Dermatoses/*drug therapy/etiology/prevention & control MH - Humans MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*complications MH - Skin Ulcer/*drug therapy/etiology/prevention & control MH - Sulfonamides/administration & dosage/adverse effects/*therapeutic use MH - Treatment Outcome MH - Wound Healing PMC - PMC3002766 COIS- Competing interests MMC has received fees for speaking and research grant from Actelion Pharmaceuticals and Pfizer, and has received fees for speaking from Glaxo-Smith-Kline Beecham. CPD has received research grant funding to support clinical research fellows, honoraria for lecturing and been a consultant to Actelion Pharmaceuticals, Encysive Pharmaceuticals, Genzyme, Aspreva Pharmaceuticals, Pfizer, Biovitrum and DiGNA. DEF has received grants for research from Actelion and Gilead, has consulted regarding trial design for the same companies and has received funds for being a Continuing Medical Education speaker from Actelion. MDM has received honoraria from Actelion Pharmaceuticals for speaking to doctor groups, as well as funds to cover travel expenses and research costs to conduct clinical trials of bosentan. MDM has received payments from Novartis to participate in a Data Safety Monitoring Board for a clinical trial of a drug not currently on the market; and to conduct a clinical trial of a new agent for scleroderma. MDM has received funds from MediQuest to conduct a clinical trial of topical nitroglycerin preparation to treat Raynaud's phenomenon. VMH has received honoraria and travel expenses from Actelion Pharmaceuticals for serving on an advisory board. PC has received fees from Actelion Pharmaceuticals as a speaker and moderator at the ‘Journée Française de la Sclérodermie Systémique’ in 2006 and 2007 and for participation in the Scientific Board on Systemic Sclerosis (2003–2008). FMW has been compensated for presenting in lecture format materials and data from studies related to the use of bosentan; FMW was an investigator in the RAPIDS-2 trial supported by Actelion. BJF has received funds for consulting from Actelion, Encysive and Gilead, for research from Actelion, and for speaking from Encysive and Gilead. PAM has received research funding from the following companies, each of which are involved in research related to the topic of this article: Actelion, Encysive, Genentech, Genzyme and Novartis. JEP has been a consultant and speaker for Actelion. Research in this study and other studies was funded by Actelion. MKD has received honoraria and travel expenses from Actelion Pharmaceuticals for serving on an advisory board and was an investigator for the RAPIDS-2 trial supported by Actelion. BH has received speaker honoraria from Actelion Pharmaceuticals, the manufacturer of bosentan. TAM has been paid honoraria by Actelion for giving lectures unrelated to Actelion products. FK is a full-time employee of and holds stock options in Actelion Pharmaceuticals. AM is a full-time employee of Actelion Pharmaceuticals Italia Srl and holds stock options in Actelion Pharmaceuticals. JHK received research funding from the following companies, each of which are involved in research related to the topic of this article: Actelion and Genzyme. JRS has funded research and a consultancy agreement with Actelion Pharmaceuticals, the sponsor of this study. JS, in the past, received honoraria for lectures supported by Actelion. He also has funded research and consultancy relationships with Pfizer, Encysive, United Therapeutics, Centocor, FibroGen, Bristol-Myers Squibb, Lilly, Genzyme and Gilead, all of which have commercial interests in treating scleroderma and its vascular complications. No conflicts of interest are reported by CMB and NJS. EDAT- 2010/09/02 06:00 MHDA- 2011/03/19 06:00 PMCR- 2010/09/28 CRDT- 2010/09/01 06:00 PHST- 2010/09/01 06:00 [entrez] PHST- 2010/09/02 06:00 [pubmed] PHST- 2011/03/19 06:00 [medline] PHST- 2010/09/28 00:00 [pmc-release] AID - S0003-4967(24)14708-5 [pii] AID - annrheumdis130658 [pii] AID - 10.1136/ard.2010.130658 [doi] PST - ppublish SO - Ann Rheum Dis. 2011 Jan;70(1):32-8. doi: 10.1136/ard.2010.130658. Epub 2010 Aug 30. PMID- 30004110 OWN - NLM STAT- MEDLINE DCOM- 20190716 LR - 20260127 IS - 1365-2141 (Electronic) IS - 0007-1048 (Linking) VI - 182 IP - 6 DP - 2018 Sep TI - Myelodysplastic syndrome (MDS) with isolated trisomy 8: a type of MDS frequently associated with myeloproliferative features? A report by the Groupe Francophone des Myélodysplasies. PG - 843-850 LID - 10.1111/bjh.15490 [doi] AB - Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (MP) features were defined by the repeated presence of one of the following: white blood cell count >10 × 10(9) /l, myelemia (presence of circulating immature granulocytes with a predominance of more mature forms) >2%, palpable splenomegaly. Fifty-four patients (39·1%) had MP features: 28 at diagnosis, 26 were acquired during evolution. MP forms had more EZH2 (33·3% vs. 12·0% in non-MP, P = 0·047), ASXL1 (66·7% vs. 42·3%, P = 0·048) and STAG2 mutations (77·8% vs. 21·7%, P = 0·006). Median event-free survival (EFS) and overall survival (OS) were 25 and 27 months for patients with MP features at diagnosis, versus 28 (P = 0·15) and 39 months (P = 0·085) for those without MP features, respectively. Among the 57 patients who received hypomethylating agent (HMA), OS was lower in MP cases (13 months vs. 23 months in non-MP cases, P = 0.02). In conclusion, MP features are frequent in MDS with isolated +8. MP forms had more EZH2, ASXL1 and STAG2 mutations, responded poorly to HMA, and tended to have poorer survival than non-MP forms. CI - © 2018 British Society for Haematology and John Wiley & Sons Ltd. FAU - Drevon, Louis AU - Drevon L AUID- ORCID: 0000-0002-9531-0380 AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. FAU - Marceau, Alice AU - Marceau A AD - Centre Hospitalier Régional Universitaire (CHRU) de Lille, Lille, France. FAU - Maarek, Odile AU - Maarek O AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. FAU - Cuccuini, Wendy AU - Cuccuini W AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. FAU - Clappier, Emmanuelle AU - Clappier E AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. FAU - Eclache, Virginie AU - Eclache V AD - Hôpital Avicenne, APHP, University Paris 13, Bobigny, France. FAU - Cluzeau, Thomas AU - Cluzeau T AD - Centre Hospitalier Universitaire (CHU) de Nice, Nice, France. FAU - Richez, Valentine AU - Richez V AD - Centre Hospitalier Universitaire (CHU) de Nice, Nice, France. FAU - Berkaoui, Inès AU - Berkaoui I AD - Centre Hospitalier Universitaire (CHU) de Nice, Nice, France. FAU - Dimicoli-Salazar, Sophie AU - Dimicoli-Salazar S AD - CHU de Bordeaux, Bordeaux, France. FAU - Bidet, Audrey AU - Bidet A AD - CHU de Bordeaux, Bordeaux, France. FAU - Vial, Jean-Philippe AU - Vial JP AD - CHU de Bordeaux, Bordeaux, France. FAU - Park, Sophie AU - Park S AD - CHU Grenoble Alpes, Grenoble, France. FAU - Vieira Dos Santos, Christina AU - Vieira Dos Santos C AD - CHU Grenoble Alpes, Grenoble, France. FAU - Kaphan, Eléonore AU - Kaphan E AD - CHU Grenoble Alpes, Grenoble, France. FAU - Berthon, Céline AU - Berthon C AD - Centre Hospitalier Régional Universitaire (CHRU) de Lille, Lille, France. FAU - Stamatoullas, Aspasia AU - Stamatoullas A AD - CHU de Rouen, Rouen, France. FAU - Delhommeau, François AU - Delhommeau F AD - Hôpital Saint-Antoine, APHP, University Pierre et Marie Curie (Paris 6), Paris, France. FAU - Abermil, Nassera AU - Abermil N AD - Hôpital Saint-Antoine, APHP, University Pierre et Marie Curie (Paris 6), Paris, France. FAU - Braun, Thorsten AU - Braun T AD - Hôpital Avicenne, APHP, University Paris 13, Bobigny, France. FAU - Sapena, Rosa AU - Sapena R AD - GFM (Groupe Francophone des Myélodysplasies), Hôpital Saint-Louis, Paris, France. FAU - Lusina, Daniel AU - Lusina D AD - Hôpital Avicenne, APHP, University Paris 13, Bobigny, France. FAU - Renneville, Aline AU - Renneville A AD - Centre Hospitalier Régional Universitaire (CHRU) de Lille, Lille, France. FAU - Adès, Lionel AU - Adès L AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Centre Hospitalier Universitaire (CHU) de Nice, Nice, France. FAU - Fenaux, Pierre AU - Fenaux P AUID- ORCID: 0000-0002-0468-3553 AD - Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), University Paris Diderot (Paris 7), Paris, France. LA - eng PT - Journal Article DEP - 20180713 PL - England TA - Br J Haematol JT - British journal of haematology JID - 0372544 RN - 0 (Antigens, Nuclear) RN - 0 (Antimetabolites, Antineoplastic) RN - 0 (Cell Cycle Proteins) RN - EC 2.1.1.43 (Enhancer of Zeste Homolog 2 Protein) RN - 0 (Repressor Proteins) RN - 0 (ASXL1 protein, human) RN - 0 (STAG2 protein, human) RN - 0 (Cohesins) RN - EC 2.1.1.43 (EZH2 protein, human) RN - Chromosome 8, trisomy SB - IM MH - Adult MH - Aged MH - Antigens, Nuclear/genetics MH - Antimetabolites, Antineoplastic/adverse effects/therapeutic use MH - Cell Cycle Proteins MH - Chromosomes, Human, Pair 8/genetics MH - Disease Progression MH - Enhancer of Zeste Homolog 2 Protein/genetics MH - Female MH - Humans MH - Middle Aged MH - Myelodysplastic Syndromes/epidemiology/*genetics MH - Myeloproliferative Disorders/drug therapy/*genetics/mortality MH - Repressor Proteins/genetics MH - Retrospective Studies MH - Survival Analysis MH - Trisomy/*genetics MH - Cohesins OTO - NOTNLM OT - myelodysplastic syndromes OT - myelodysplastic-myeloproliferative diseases OT - trisomy 8 EDAT- 2018/07/14 06:00 MHDA- 2019/07/17 06:00 CRDT- 2018/07/14 06:00 PHST- 2018/03/06 00:00 [received] PHST- 2018/05/25 00:00 [accepted] PHST- 2018/07/14 06:00 [pubmed] PHST- 2019/07/17 06:00 [medline] PHST- 2018/07/14 06:00 [entrez] AID - 10.1111/bjh.15490 [doi] PST - ppublish SO - Br J Haematol. 2018 Sep;182(6):843-850. doi: 10.1111/bjh.15490. Epub 2018 Jul 13. PMID- 36836844 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230301 IS - 2075-1729 (Print) IS - 2075-1729 (Electronic) IS - 2075-1729 (Linking) VI - 13 IP - 2 DP - 2023 Feb 10 TI - Peripheral Macrovascular Involvement in Systemic Sclerosis: A Cohort Study by Color and Spectral Doppler Ultrasonography. LID - 10.3390/life13020487 [doi] LID - 487 AB - OBJECTIVES: Systemic sclerosis (SSc) is a disease characterized by diffuse sclerosis of skin and organs and small vessel vasculopathy. Despite it, large vessels can also be involved with ulnar artery vasculopathy, revealing as a more frequent feature of SSc. The aim of this paper is to assess the macrovascular involvement of SSc patients through an ultrasound (US) evaluation of radial and ulnar arteries. METHODS: Radial and ulnar resistance indices (RIs) and peak systolic velocity (PV) (cm/s) together with clinical features of SSc patients were evaluated. Raynaud phenomenon (RP) and healthy control (HC) groups were used for comparison. RESULTS: Forty-three SSc patients were evaluated. Twelve patients (28%) had ulnar artery occlusions (UAOs). In nine cases (75%), UAOs were bilateral. A high UAO prevalence (42%) was found in SSc patients with late nailfold-video-capillaroscopy (NVC) pattern (p = 0.0264). Patients with UAOs had digital ulcers (DUs) in 10 cases (83.3%). Radial and ulnar PVs were lower in SSc and RP patients than the HC group. Radial and ulnar RIs were higher in SSc and RP patients than the HC group. A decision tree analysis led to the classification of 70% of SSc patients with an ulnar RI > 0.82 and ulnar PV > 2.8 cm/s. The most influential variables on UAO development were interstitial lung disease (ILD) (p = 0.002) and NVC pattern (p = 0.002). A positive correlation was shown between modified Rodnan skin score (mRSS) and ILD (p = 0.283; r = 0.033), mRSS and DU (r = 0.344; p = 0.012) and DU and ILD (r = 0.303; p = 0.024). Male sex was associated with increased UAO frequency (p = 0.042). CONCLUSIONS: UAO is a peculiar feature of severe SSc present in 28% of the cases, particularly associated with the presence of ILD and late NVC pattern. In 75% of the cases, UAOs are bilateral. DUs are very frequent in patients with UAOs (83%). The RI evaluated by US could be useful to distinguish SSc from HC patients. US could be a useful tool for assessing high-risk DU development in patients. FAU - D'Alessandro, Roberto AU - D'Alessandro R AUID- ORCID: 0000-0002-8350-7581 AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Garcia Gonzalez, Estrella AU - Garcia Gonzalez E AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Falsetti, Paolo AU - Falsetti P AUID- ORCID: 0000-0003-2667-4866 AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Conticini, Edoardo AU - Conticini E AUID- ORCID: 0000-0002-3974-6606 AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - d'Alessandro, Miriana AU - d'Alessandro M AUID- ORCID: 0000-0002-2368-5722 AD - Respiratory Diseases and Lung Transplantation Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Selvi, Enrico AU - Selvi E AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Bellisai, Francesca AU - Bellisai F AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Berlengiero, Virginia AU - Berlengiero V AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Vallifuoco, Giulia AU - Vallifuoco G AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Pata, Anna Paola AU - Pata AP AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Bardelli, Marco AU - Bardelli M AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Baldi, Caterina AU - Baldi C AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Cantarini, Luca AU - Cantarini L AUID- ORCID: 0000-0002-7352-1275 AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Bargagli, Elena AU - Bargagli E AUID- ORCID: 0000-0002-8351-3703 AD - Respiratory Diseases and Lung Transplantation Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. FAU - Frediani, Bruno AU - Frediani B AD - Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, Italy. LA - eng PT - Journal Article DEP - 20230210 PL - Switzerland TA - Life (Basel) JT - Life (Basel, Switzerland) JID - 101580444 PMC - PMC9962179 OTO - NOTNLM OT - Raynaud phenomenon OT - digital ulcer OT - fibrosis OT - resistance index OT - systemic sclerosis OT - ulnar artery OT - ulnar artery occlusion OT - ultrasonography OT - vasculopathy COIS- The authors have declared no conflict of interest. EDAT- 2023/02/26 06:00 MHDA- 2023/02/26 06:01 PMCR- 2023/02/10 CRDT- 2023/02/25 03:20 PHST- 2022/11/10 00:00 [received] PHST- 2023/01/23 00:00 [revised] PHST- 2023/02/07 00:00 [accepted] PHST- 2023/02/25 03:20 [entrez] PHST- 2023/02/26 06:00 [pubmed] PHST- 2023/02/26 06:01 [medline] PHST- 2023/02/10 00:00 [pmc-release] AID - life13020487 [pii] AID - life-13-00487 [pii] AID - 10.3390/life13020487 [doi] PST - epublish SO - Life (Basel). 2023 Feb 10;13(2):487. doi: 10.3390/life13020487. PMID- 34350037 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210806 IS - 2090-6382 (Print) IS - 2090-6390 (Electronic) IS - 2090-6390 (Linking) VI - 2021 DP - 2021 TI - Antisynthetase Syndrome with Severe Interstitial Lung Disease in Pregnancy. PG - 1150394 LID - 10.1155/2021/1150394 [doi] LID - 1150394 AB - Antisynthetase syndrome is a rare multisystem autoimmune disorder which clinically manifests with myositis, arthritis, interstitial lung disease, Raynaud phenomenon, and skin hyperkeratosis. Lung involvement represents the most severe form of disease and has rarely been reported in pregnancy. We present the case of a 22-year-old woman with antisynthetase syndrome and severe restrictive pulmonary disease who experienced a successful pregnancy and delivery. We discuss anesthetic considerations and highlight the importance of a multidisciplinary team approach in caring for parturients with multifactorial medical conditions. CI - Copyright © 2021 Catalina I. Dumitrascu et al. FAU - Dumitrascu, Catalina I AU - Dumitrascu CI AUID- ORCID: 0000-0002-4867-3728 AD - Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, 5777 E Mayo Blvd, Phoenix, AZ 85054, USA. FAU - Olsen, David A AU - Olsen DA AUID- ORCID: 0000-0003-2359-6225 AD - Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. FAU - Arendt, Katherine W AU - Arendt KW AUID- ORCID: 0000-0002-4793-9133 AD - Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. FAU - Rose, Carl H AU - Rose CH AD - Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. FAU - Sharpe, Emily E AU - Sharpe EE AUID- ORCID: 0000-0003-4232-0718 AD - Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210726 PL - United States TA - Case Rep Anesthesiol JT - Case reports in anesthesiology JID - 101581025 PMC - PMC8328709 COIS- The authors declare that there are no conflicts of interest. EDAT- 2021/08/06 06:00 MHDA- 2021/08/06 06:01 PMCR- 2021/07/26 CRDT- 2021/08/05 06:28 PHST- 2021/04/29 00:00 [received] PHST- 2021/07/20 00:00 [accepted] PHST- 2021/08/05 06:28 [entrez] PHST- 2021/08/06 06:00 [pubmed] PHST- 2021/08/06 06:01 [medline] PHST- 2021/07/26 00:00 [pmc-release] AID - 10.1155/2021/1150394 [doi] PST - epublish SO - Case Rep Anesthesiol. 2021 Jul 26;2021:1150394. doi: 10.1155/2021/1150394. eCollection 2021. PMID- 39854163 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250130 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 7 IP - 1 DP - 2025 Jan TI - Abnormal Esophageal Scintigraphy Associates With a Distinct Clinical Phenotype in Patients With Systemic Sclerosis. PG - e11796 LID - 10.1002/acr2.11796 [doi] LID - e11796 AB - OBJECTIVE: In systemic sclerosis (SSc), absent contractility (AC) rather than ineffective esophageal motility on manometry is associated with a severe esophageal and extraintestinal phenotype. We sought to determine whether slow esophageal transit on scintigraphy associates with a comparable clinical phenotype to that of AC on manometry, as scintigraphy may serve as a noninvasive approach to risk-stratify patients with SSc. METHODS: Clinical, demographic, and serologic features were compared between patients with and without delayed esophageal transit on scintigraphy. University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract (GIT) 2.0 scores measured GI symptoms, Medsger scores measured physician-assessed SSc disease severity, and the Composite Autonomic Symptom Score 31 survey evaluated dysautonomia symptoms. RESULTS: Of 131 patients, 79 (60%) had delayed esophageal transit by scintigraphy. Patients with delayed esophageal transit were more likely to have diffuse SSc (24 [32%] vs 11 [22%]; P = 0.024), severe lung involvement (22 [41%] vs 7 [19%]; P = 0.034), severe Raynaud (36 [47%] vs 15 [31%]; P = 0.063), and a higher median (interquartile range [IQR]) diarrhea GIT score (0.5 [IQR 0-1] vs 0 [IQR 0-1]; P = 0.050). Lower diffusing capacity of the lungs for carbon monoxide values correlated with a higher esophageal transit time (ρ = -0.32; P = 0.014). After adjusting for disease duration, delayed esophageal transit was significantly associated with severe Medsger lung scores, severe Raynaud phenomenon, and higher modified Rodnan skin scores. CONCLUSION: Patients with delayed esophageal transit by scintigraphy have a more severe SSc phenotype, similar to patients with AC, on esophageal manometry. Further studies should validate esophageal scintigraphy as a tool to identify patients with SSc with AC who may develop specific GI and extraintestinal complications. CI - © 2025 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Salas, Antonio D AU - Salas AD AUID- ORCID: 0000-0002-9023-8586 AD - Johns Hopkins University, Baltimore, Maryland. FAU - Yanek, Lisa R AU - Yanek LR AD - Johns Hopkins University, Baltimore, Maryland. FAU - Hummers, Laura K AU - Hummers LK AUID- ORCID: 0000-0002-4864-4011 AD - Johns Hopkins University, Baltimore, Maryland. FAU - Shah, Ami A AU - Shah AA AD - Johns Hopkins University, Baltimore, Maryland. FAU - McMahan, Zsuzsanna H AU - McMahan ZH AUID- ORCID: 0000-0001-6461-8940 AD - UTHealth Houston, Houston, Texas. LA - eng PT - Journal Article PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC11760989 EDAT- 2025/01/24 18:25 MHDA- 2025/01/24 18:26 PMCR- 2025/01/24 CRDT- 2025/01/24 12:04 PHST- 2024/11/11 00:00 [revised] PHST- 2024/07/13 00:00 [received] PHST- 2024/12/03 00:00 [accepted] PHST- 2025/01/24 18:26 [medline] PHST- 2025/01/24 18:25 [pubmed] PHST- 2025/01/24 12:04 [entrez] PHST- 2025/01/24 00:00 [pmc-release] AID - ACR211796 [pii] AID - 10.1002/acr2.11796 [doi] PST - ppublish SO - ACR Open Rheumatol. 2025 Jan;7(1):e11796. doi: 10.1002/acr2.11796. PMID- 24891607 OWN - NLM STAT- MEDLINE DCOM- 20140912 LR - 20161125 IS - 1472-4146 (Electronic) IS - 0021-9746 (Linking) VI - 67 IP - 8 DP - 2014 Aug TI - Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy. PG - 678-83 LID - 10.1136/jclinpath-2014-202367 [doi] AB - AIMS: Antisynthetase syndromes are a subset of the idiopathic inflammatory myopathies characterised by the presence of autoantibodies to aminoacyl transfer-RNA synthetases (ARS) and monotypic clinical features including Raynaud phenomenon, fever, non-erosive inflammatory arthritis and hyperkeratotic skin changes ('mechanic's hands'). Interstitial lung disease (ILD) is particularly common in ARS syndromes, affecting up to 90% of patients. METHODS: Four patients with ARS syndrome who possessed anti-glycyl-tRNA synthetase (anti-EJ) autoantibodies were retrieved from the University of Pittsburgh database. We report their clinical, radiographic and histopathologic findings. RESULTS: Patients presented with dyspnoea accompanied by Raynaud phenomenon and 'mechanic's hands'. Lung disease was the first manifestation in all four patients (100%) who were all amyopathic. High-resolution CT of the chest showed patchy opacities and consolidations in two patients (50%) whose surgical lung biopsies revealed organising diffuse alveolar damage (DAD), and lower lung zone predominant reticular infiltrates and traction bronchiectasis without honeycomb change in two patients (50%) whose surgical lung biopsies revealed usual interstitial pneumonia (UIP). Mild lymphoplasmacytic inflammation and few scattered lymphoid aggregates were present, but we found no pathognomonic histopathologic features of anti-EJ ARS syndrome. Serologic testing revealed no other autoantibodies. All patients responded to immunosuppressive therapy. CONCLUSIONS: Identifying ARS-associated autoantibodies in ILD patients with or without myopathy is desirable because patients may respond well to immunosuppressive therapy, and their prognosis is better than that of patients with idiopathic forms of DAD or UIP. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. FAU - Schneider, Frank AU - Schneider F AD - Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. FAU - Yousem, Samuel A AU - Yousem SA AD - Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. FAU - Bi, David AU - Bi D AD - Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. FAU - Gibson, Kevin F AU - Gibson KF AD - Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. FAU - Oddis, Chester V AU - Oddis CV AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. FAU - Aggarwal, Rohit AU - Aggarwal R AD - Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140602 PL - England TA - J Clin Pathol JT - Journal of clinical pathology JID - 0376601 RN - 0 (Autoantibodies) RN - EC 6.1.1.14 (Glycine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Aged MH - Autoantibodies/immunology MH - Female MH - Glycine-tRNA Ligase/*immunology MH - Humans MH - Lung/diagnostic imaging/immunology/*pathology MH - Male MH - Middle Aged MH - Myositis/diagnostic imaging/immunology/*pathology MH - Radiography OTO - NOTNLM OT - Autoantibody OT - Inflammation OT - Lung OT - Muscle OT - fibrosis EDAT- 2014/06/04 06:00 MHDA- 2014/09/13 06:00 CRDT- 2014/06/04 06:00 PHST- 2014/06/04 06:00 [entrez] PHST- 2014/06/04 06:00 [pubmed] PHST- 2014/09/13 06:00 [medline] AID - jclinpath-2014-202367 [pii] AID - 10.1136/jclinpath-2014-202367 [doi] PST - ppublish SO - J Clin Pathol. 2014 Aug;67(8):678-83. doi: 10.1136/jclinpath-2014-202367. Epub 2014 Jun 2. PMID- 25981806 OWN - NLM STAT- MEDLINE DCOM- 20160606 LR - 20180605 IS - 1875-533X (Electronic) IS - 0929-8673 (Linking) VI - 22 IP - 16 DP - 2015 TI - Antisynthetase syndrome: a review of etiopathogenesis, diagnosis and management. PG - 1963-75 AB - Antisynthetase syndrome is a group of closely related rare diseases which clinically manifest with inflammatory myopathies, interstitial lung disease, inflammatory arthritis, skin hyperkeratosis (mechanic's hands) and Raynaud phenomenon. The pathophysiology of antisynthetase syndrome is not entirely understood, but genetic predisposition, viral infections and medication use may play a role. Certain antisynthetase antibodies are associated with various clinical presentations and a lower burden of inflammatory myopathies. Patients with antisynthetase syndrome have a worse prognosis than patients with pure inflammatory myopathies mainly because of interstitial lung disease. Future research should further investigate the pathogenesis of antisynthetase syndrome which could identify new therapeutic targets. It will be also important to study whether patients with AS are at increased risk of cancer and whether certain antisynthetase antibodies have any association with the risk of malignancy. FAU - Mirrakhimov, Aibek E AU - Mirrakhimov AE AD - Saint Joseph Hospital, Department of Internal Medicine, 2900 N. Lake Shore, Chicago, Illinois 60657, USA. amirrakhimov1@gmail.com. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Curr Med Chem JT - Current medicinal chemistry JID - 9440157 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/immunology MH - Humans MH - *Myositis/diagnosis/drug therapy/genetics/physiopathology MH - Neoplasms/complications EDAT- 2015/05/20 06:00 MHDA- 2016/06/09 06:00 CRDT- 2015/05/19 06:00 PHST- 2015/01/14 00:00 [received] PHST- 2015/01/25 00:00 [revised] PHST- 2015/05/05 00:00 [accepted] PHST- 2015/05/19 06:00 [entrez] PHST- 2015/05/20 06:00 [pubmed] PHST- 2016/06/09 06:00 [medline] AID - CMC-EPUB-67284 [pii] PST - ppublish SO - Curr Med Chem. 2015;22(16):1963-75. PMID- 30881402 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 1682-024X (Print) IS - 1681-715X (Electronic) IS - 1681-715X (Linking) VI - 35 IP - 1 DP - 2019 Jan-Feb TI - Frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus. PG - 86-89 LID - 10.12669/pjms.35.1.405 [doi] AB - OBJECTIVE: To determine the frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus in a Pakistani population, presenting at a tertiary care hospital. METHODS: This cross-sectional study was conducted at the Department of Rheumatology, Shiekh Zayed Hospital, Lahore from March to June 2018. A total of 97 patients, who fulfilled the Systemic Lupus Erythematosus (SLE) criteria of American College of Rheumatology (ACR) 1992 were enrolled. Pulmonary Arterial Hypertension (PAH) was measured by calculating pulmonary arterial systolic pressure through echocardiography by a single consultant cardiologist. Disease characteristics and demography was collected in a self-administered proforma. PAH was defined as mean pulmonary arterial pressure of 25mmHg or above by calculating with a formula. SPSS version 20 was used for analysis of data. RESULTS: Out of 97 patients, 89.7% (n=87) were females and 10.3% (n=10) were males, with mean age of 31.29± 8.824 years. The mean disease duration was 24.21 ± 30.46 months. PAH was found in 23.3% (n=23) patients, including 19 females and 4 males. On further analysis of data, Raynaud phenomenon, rheumatoid factor and nephritis were assessed as predictors of PAH and all of these showed statistical significance for presence of PAH as per Chi-square test (p<0.05). CONCLUSION: In this study, 23.3% SLE patients showed evidence of PAH and positive statistical significance was found between predictors like Raynaud phenomenon, rheumatoid factor, nephritis and presence of PAH. So it is imperative to detect PAH early and start prompt treatment to achieve better quality of life. FAU - Asif, Sadia AU - Asif S AD - Dr. Sadia Asif, FCPS (Medicine), Fellow Rheumatology, Department of Rheumatology, Sheikh Zayed Hospital, Lahore, Pakistan. FAU - Rasheed, Aflak AU - Rasheed A AD - Dr. Aflak Rasheed, FCPS (Medicine), FCPS Rheumatology, Department of Rheumatology, Sheikh Zayed Hospital, Lahore, Pakistan. FAU - Mahmud, Tafazzul-E-Haque AU - Mahmud TE AD - Dr. Tafazzul-e-Haq Mahmud, MB MRCP (UK) FRCP (London), Department of Rheumatology, Sheikh Zayed Hospital, Lahore, Pakistan. FAU - Asghar, Ammad AU - Asghar A AD - Dr. Ammad Asghar, FCPS (Medicine), PGR Rheumatology, Department of Rheumatology, Sheikh Zayed Hospital, Lahore, Pakistan. LA - eng PT - Journal Article PL - Pakistan TA - Pak J Med Sci JT - Pakistan journal of medical sciences JID - 100913117 PMC - PMC6408637 OTO - NOTNLM OT - Mean Pulmonary Arterial Pressure (MPAP) OT - Pulmonary Arterial Hypertension (PAH) OT - Pulmonary Artery Systolic Pressure (PASP) OT - Systemic Lupus Erythematosus (SLE) EDAT- 2019/03/19 06:00 MHDA- 2019/03/19 06:01 PMCR- 2019/01/01 CRDT- 2019/03/19 06:00 PHST- 2019/03/19 06:00 [entrez] PHST- 2019/03/19 06:00 [pubmed] PHST- 2019/03/19 06:01 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - PJMS-35-86 [pii] AID - 10.12669/pjms.35.1.405 [doi] PST - ppublish SO - Pak J Med Sci. 2019 Jan-Feb;35(1):86-89. doi: 10.12669/pjms.35.1.405. PMID- 33654616 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210305 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 1 DP - 2021 Jan 27 TI - Anti-Synthetase Syndrome-Related Interstitial Lung Disease With Anti-PL-12 Antibodies. PG - e12936 LID - 10.7759/cureus.12936 [doi] LID - e12936 AB - Anti-synthetase syndrome usually comprises interstitial lung disease, myositis, arthralgias, and Raynaud phenomenon. The anti-PL-12 antibody is directed against the enzyme alanyl-tRNA synthetase and has been associated with interstitial lung disease in the absence of inflammatory myositis. We report the case of a 33-year-old woman with complaints of progressive dyspnea, a persistent dry cough, along with intermittent low-grade fever for a few months. A computed tomography (CT) scan of the chest showed the presence of patchy bilateral airspace opacities and infiltrates. It also showed significant mediastinal and hilar lymphadenopathy. Bronchoscopy with transbronchial biopsy was performed, and histopathology changes were consistent with connective tissue disease related to interstitial lung disease. Further workup revealed the presence of anti-PL-12 antibodies. This case illustrates a rare association of interstitial lung disease with the anti-PL-12 antibody. CI - Copyright © 2021, Elferjani et al. FAU - Elferjani, Belqis AU - Elferjani B AD - Internal Medicine, Southeast Health Medical Center, Dothan, USA. FAU - Liaqat, Adnan AU - Liaqat A AD - Internal Medicine, Southeast Health Medical Center, Dothan, USA. FAU - Zaman, Mohammed AU - Zaman M AD - Internal Medicine, Southeast Health Medical Center, Dothan, USA. FAU - Sexton, Marvin AU - Sexton M AD - Pulmonology, Dothan Pulmonary Associates, Dothan, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20210127 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC7916638 OTO - NOTNLM OT - anti-pl-12 antibody OT - anti-synthetase syndrome OT - interstitial lung disease COIS- The authors have declared that no competing interests exist. EDAT- 2021/03/04 06:00 MHDA- 2021/03/04 06:01 PMCR- 2021/01/27 CRDT- 2021/03/03 05:47 PHST- 2021/03/03 05:47 [entrez] PHST- 2021/03/04 06:00 [pubmed] PHST- 2021/03/04 06:01 [medline] PHST- 2021/01/27 00:00 [pmc-release] AID - 10.7759/cureus.12936 [doi] PST - epublish SO - Cureus. 2021 Jan 27;13(1):e12936. doi: 10.7759/cureus.12936. PMID- 27544937 OWN - NLM STAT- MEDLINE DCOM- 20160921 LR - 20260127 IS - 1221-9118 (Print) IS - 1221-9118 (Linking) VI - 111 IP - 3 DP - 2016 May-Jun TI - The. Thoma Ionescu - Victor Gomoiu Procedure: Cervicothoracic Sympathectomy for Angina Pectoris. The First Surgical Attempt to Treat the Coronary Heart Disease. PG - 216-21 AB - Cervicothoracic Sympathectomy is a common indication in the treatment of Raynaud Syndrome, Palmer Hyperhidrosis or Acute Ischemia of the superior limb. Nonetheless, almost a century ago it represented one of the first innovative attempts in curing coronary heart disease. Nowadays, this indication is no more than a footnote in a volume on the History of Medicine, and a trivia fact for medical history enthusiasts. The operation's history is rather conflicting. A young Romaninan surgeon, Victor Gomoiu seems to have come up with the idea, in the early 20th century. However, his contribution remains unknown, after his successful collaboration with the famous surgeon and anatomist, Thoma Ionescu unfortunately turns into a dispute. This procedure was once thought cutting-edge. Furthermore it is the starting point for cardiovascular surgery. Whoever sparked the idea, gains an important place in the hall of fame of international surgery, that is why it is important to know its creator. FAU - Vasilescu, Cătălin AU - Vasilescu C FAU - Salmen, Monica AU - Salmen M FAU - Bobocea, Andrei AU - Bobocea A LA - eng PT - Biography PT - Historical Article PT - Journal Article PT - Portrait PL - Romania TA - Chirurgia (Bucur) JT - Chirurgia (Bucharest, Romania : 1990) JID - 9213031 SB - IM MH - Coronary Disease/*history MH - General Surgery/*history MH - History, 19th Century MH - History, 20th Century MH - Humans MH - *Nobel Prize MH - Romania MH - Sympathectomy/*history PS - Ionescu T FPS - Ionescu, Thoma PS - Gomoiu V FPS - Gomoiu, Victor EDAT- 2016/08/23 06:00 MHDA- 2016/09/23 06:00 CRDT- 2016/08/23 06:00 PHST- 2016/08/23 06:00 [entrez] PHST- 2016/08/23 06:00 [pubmed] PHST- 2016/09/23 06:00 [medline] PST - ppublish SO - Chirurgia (Bucur). 2016 May-Jun;111(3):216-21. PMID- 28613608 STAT- Publisher CTDT- 20230219 PB - StatPearls Publishing DP - 2026 Jan TI - Buerger Disease. BTI - StatPearls AB - Buerger disease, also known as thromboangiitis obliterans (TAO) is a progressive, nonatherosclerotic, segmental, inflammatory disease that most often affects small and medium arteries of the upper and lower extremities. Initially described by von Winiwarter in 1879, although the eponym was given to Leo Buerger who published extensive pathologic findings from the amputated limbs of afflicted patients in 1908. The typical age range for occurrence is 20 to 50 years, and the disorder is more frequently found in men who smoke. Migratory superficial phlebitis can be present in up to 16% of patients and indicates a systemic inflammatory response. Patients initially present with the foot, leg, arm, or hand claudication which may be mistaken for joint or neuromuscular problems. Progression of the disease leads to calf claudication, and eventually, ischemic rest pain and ulcerations on the toes, feet, or fingers. This is also called Raynaud phenomenon. The treatment of TAO revolves around strict smoking cessation. In patients who can abstain, disease remission is impressive, and amputation avoidance is increased. The role of surgical intervention is minimal in Buerger disease as there is often no acceptable target vessel for bypass. Furthermore, autogenous vein conduits are limited secondary to coexisting migratory thrombophlebitis. CI - Copyright © 2026, StatPearls Publishing LLC. FAU - Qaja, Erion AU - Qaja E AD - Wyckoff Heights Medical Center FAU - Muco, Erind AU - Muco E AD - Campbell University School of Osteopathic Medicine FAU - Hashmi, Muhammad F AU - Hashmi MF AD - National Health Service LA - eng PT - Study Guide PT - Book Chapter PL - Treasure Island (FL) COIS- Disclosure: Erion Qaja declares no relevant financial relationships with ineligible companies. Disclosure: Erind Muco declares no relevant financial relationships with ineligible companies. Disclosure: Muhammad Hashmi declares no relevant financial relationships with ineligible companies. EDAT- 2023/02/19 00:00 CRDT- 2023/02/19 00:00 AID - NBK430858 [bookaccession] PMID- 23550408 OWN - NLM STAT- MEDLINE DCOM- 20130708 LR - 20150826 IS - 0443-5117 (Print) IS - 0443-5117 (Linking) VI - 51 IP - 1 DP - 2013 Jan-Feb TI - [Scleroderma: an update on the pathogenesis and treatment]. PG - 50-7 AB - Scleroderma is a multiorganic disease characterized by inflammatory, vascular and sclerotic changes in skin and internal organs. It is considered as a tripartite disease, associated to autoimmune, fibroblast and endothelial defect, due to genetic, environmental and infectious factors. This disease can be classified in systemic and localized form. The Raynaud phenomenon occurs in 90% of the patients with the diagnosis. It explains the microcirculation involvement and the reduction in the number of capillaries. Malformation of nail bed capillaries is readily demonstrated by nail bed microscopy and has been shown to correlate both with disease severity and with degree of internal organ involvement. The MRSS-51 validates the skin involvement and has the main predictive value to determine the patient survival. MRSS-51 should not be considered as an activity disease parameter or used to validate the effectiveness of treatment. Nowadays, multiple treatment alternatives exist for scleroderma disease; however these treatments offer poor results for the cutaneous manifestations. FAU - Garza-Rodríguez, Verónica AU - Garza-Rodríguez V AD - Servicio de Dermatología, Hospital Universitario "Dr. José Eleuterio González, Universidad Autónoma de Nuevo León, Monterrey, México. verogarzardz@hotmail.com FAU - Villarreal-Alarcón, Miguel Angel AU - Villarreal-Alarcón MA FAU - Ocampo-Candiani, Jorge AU - Ocampo-Candiani J LA - spa PT - English Abstract PT - Journal Article TT - Etiopatogenia y tratamiento de la esclerodermia. Conceptos actuales. PL - Mexico TA - Rev Med Inst Mex Seguro Soc JT - Revista medica del Instituto Mexicano del Seguro Social JID - 101243727 SB - IM MH - Humans MH - Scleroderma, Localized/*etiology/*therapy MH - Scleroderma, Systemic/*etiology/*therapy EDAT- 2013/04/05 06:00 MHDA- 2013/07/09 06:00 CRDT- 2013/04/05 06:00 PHST- 2013/04/05 06:00 [entrez] PHST- 2013/04/05 06:00 [pubmed] PHST- 2013/07/09 06:00 [medline] PST - ppublish SO - Rev Med Inst Mex Seguro Soc. 2013 Jan-Feb;51(1):50-7. PMID- 24337198 OWN - NLM STAT- MEDLINE DCOM- 20140813 LR - 20211021 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 72 IP - 10 DP - 2013 Dec TI - [Therapeutic management in early disease stages of systemic sclerosis : early diagnosis - early symptoms - early problems]. PG - 960-9 LID - 10.1007/s00393-013-1270-2 [doi] AB - Increasing knowledge about the rare disease systemic sclerosis and improved diagnostic methods in the course of recent decades has led to the possibility of diagnosing systemic sclerosis at earlier disease stages. However, earlier diagnosis has an impact on routine clinical care of affected patients, and rheumatologists need to know about early symptoms, their diagnosis, and clinical management. In this review, the therapeutic management of early disease stages is described. In particular, we focus on diagnostic tools which should be included in a "basic assessment" of systemic sclerosis and discuss the diagnosis and treatment options of early symptoms such as Raynaud phenomenon, puffy fingers and hand edema, digital ulcers, calcinosis cutis, and cardiopulmonary, renal, and gastrointestinal involvement. Finally, the options of early immunosuppressive treatment and autologous stem cell transplantation for patients with rapid progressive and severe disease are reviewed. FAU - Frerix, M AU - Frerix M AD - Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie, Justus-Liebig-Universität Gießen, Gießen, Deutschland, m.frerix@kerckhoff-klinik.de. FAU - Meier, F M P AU - Meier FM FAU - Hermann, W AU - Hermann W FAU - Müller-Ladner, U AU - Müller-Ladner U LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Therapeutische Strategien im Frühstadium der systemischen Sklerose : Frühe Diagnose - frühe Symptome - frühe Probleme. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 SB - IM MH - Early Diagnosis MH - Humans MH - *Mass Screening MH - *Primary Prevention MH - Scleroderma, Systemic/*diagnosis/*therapy MH - *Secondary Prevention MH - Symptom Assessment/*methods EDAT- 2013/12/18 06:00 MHDA- 2014/08/15 06:00 CRDT- 2013/12/17 06:00 PHST- 2013/12/17 06:00 [entrez] PHST- 2013/12/18 06:00 [pubmed] PHST- 2014/08/15 06:00 [medline] AID - 10.1007/s00393-013-1270-2 [doi] PST - ppublish SO - Z Rheumatol. 2013 Dec;72(10):960-9. doi: 10.1007/s00393-013-1270-2. PMID- 32190815 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210513 IS - 2515-5091 (Electronic) IS - 2515-5091 (Linking) VI - 4 IP - 2 DP - 2020 Apr TI - Adverse Health Outcomes Among US Testicular Cancer Survivors After Cisplatin-Based Chemotherapy vs Surgical Management. PG - pkz079 LID - 10.1093/jncics/pkz079 [doi] LID - pkz079 AB - We evaluated for the first time, to our knowledge, adverse health outcomes (AHOs) among US testicular cancer survivors (TCS) given chemotherapy (n = 381) vs surgery-only patients (n = 98) managed at a single institution, accounting for non-treatment-related risk factors to delineate chemotherapy's impact. Chemotherapy consisted largely of bleomycin-etoposide-cisplatin (BEP) administered in three or four cycles (BEPx3, n = 235; BEPx4, n = 82). Incidence of at least 3 AHOs was lowest in surgery-only TCS and increased with BEPx3, BEPx4, and other cisplatin-based regimens (12.2%, 40.8%, 52.5%, 54.8%; P < .0001). Multivariable modeling assessed associations of risk factors and treatment with hearing impairment, tinnitus, peripheral neuropathy, and Raynaud phenomenon. Risk for each AHO statistically increased with both increasing chemotherapy burden (P < .0001) and selected modifiable risk factors (P < .05): hypertension (odds ratio [OR] = 2.40) and noise exposure (OR ≥ 2.3) for hearing impairment; noise exposure for tinnitus (OR ≥ 1.69); peripheral vascular disease for neuropathy (OR = 8.72); and current smoking for Raynaud phenomenon (OR = 2.41). Clinicians should manage modifiable risk factors for AHOs among TCS. CI - © The Author(s) 2019. Published by Oxford University Press. FAU - Agrawal, Vaibhav AU - Agrawal V AUID- ORCID: 0000-0002-6383-3777 AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. FAU - Dinh, Paul C Jr AU - Dinh PC Jr AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. AD - Department of Epidemiology and Biostatistics, Indiana University of Public Health, Bloomington, IN. FAU - Fung, Chunkit AU - Fung C AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. AD - Division of Hematology and Oncology, Department of Medicine, University of Rochester School of Medicine and Dentistry, James P. Wilmot Cancer Institute, Rochester, NY. FAU - Monahan, Patrick O AU - Monahan PO AD - Department of Biostatistics, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN. FAU - Althouse, Sandra K AU - Althouse SK AD - Department of Biostatistics, Indiana University Melvin and Bren Simon Cancer Center, Indianapolis, IN. FAU - Norton, Kelli AU - Norton K AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. FAU - Cary, Clint AU - Cary C AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. AD - Department of Urology, Indiana University School of Medicine, Indianapolis, IN. FAU - Einhorn, Lawrence AU - Einhorn L AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. FAU - Fossa, Sophie D AU - Fossa SD AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. AD - Department of Oncology, Oslo University Hospital, Radium Hospital, Oslo, Norway. FAU - Adra, Nabil AU - Adra N AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. FAU - Travis, Lois B AU - Travis LB AD - Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN. LA - eng GR - R01 CA157823/CA/NCI NIH HHS/United States PT - Journal Article DEP - 20191008 PL - England TA - JNCI Cancer Spectr JT - JNCI cancer spectrum JID - 101721827 CIN - J Urol. 2020 Dec;204(6):1383. doi: 10.1097/JU.0000000000001284. PMID: 32955976 PMC - PMC7065712 EDAT- 2020/03/20 06:00 MHDA- 2020/03/20 06:01 PMCR- 2019/10/08 CRDT- 2020/03/20 06:00 PHST- 2019/01/31 00:00 [received] PHST- 2019/03/14 00:00 [revised] PHST- 2019/09/27 00:00 [accepted] PHST- 2020/03/20 06:00 [entrez] PHST- 2020/03/20 06:00 [pubmed] PHST- 2020/03/20 06:01 [medline] PHST- 2019/10/08 00:00 [pmc-release] AID - pkz079 [pii] AID - 10.1093/jncics/pkz079 [doi] PST - epublish SO - JNCI Cancer Spectr. 2019 Oct 8;4(2):pkz079. doi: 10.1093/jncics/pkz079. eCollection 2020 Apr. PMID- 30188463 OWN - NLM STAT- MEDLINE DCOM- 20191021 LR - 20191022 IS - 1536-481X (Electronic) IS - 1057-0829 (Linking) VI - 27 IP - 11 DP - 2018 Nov TI - A Phenotype of Primary Open-angle Glaucoma With Systemic Vasospasm. PG - 987-992 LID - 10.1097/IJG.0000000000001083 [doi] AB - PURPOSE: Primary open-angle glaucoma (POAG) patients constitute a heterogenous group. Identification of phenotypic subtypes among these patients may provide a deeper understanding of the disease and aid associations with genotypes. We describe a phenotype of POAG patients associated with a constellation of systemic disorders; patients with this phenotype seem to be vulnerable to optic nerve damage at low intraocular pressures. MATERIALS AND METHODS: In this retrospective study, we evaluated the medical records of active Jules Stein Eye Institute glaucoma patients from January 1996 to 2017 and included subjects with POAG, acquired pits of the optic nerve (APON), and at least one of the following: systolic blood pressure persistently ≤100 mm Hg, history of migraine headaches or migraine variant, and the Raynaud syndrome. RESULTS: Of 87 patients (125 eyes) with APON, 37 patients (55 eyes) met the study criteria. In total, 34 patients were female (92%). The median age at the time of diagnosis was 55 years. Nineteen patients (73%) had low systolic blood pressures, same number had Raynaud syndrome, and 25 (68%) had a history of migraine. CONCLUSIONS: We describe a POAG subtype with APON and systemic vascular instability, predominantly female in their sixth decade of life who demonstrate progressive glaucomatous visual field damage at low intraocular pressure. We suggest that this clinical picture represents an important phenotype of POAG, and that identification and further study of it will help guide diagnosis and development of individualized treatments. FAU - Alizadeh, Reza AU - Alizadeh R AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. FAU - Vickers, Laura AU - Vickers L AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. FAU - Hirunpatravong, Pradtana AU - Hirunpatravong P AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. FAU - Romero, Pablo AU - Romero P AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. FAU - Lin, Mark C AU - Lin MC AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. FAU - Sharifipour, Farideh AU - Sharifipour F AD - Department of Ophthalmology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran. FAU - Caprioli, Joseph AU - Caprioli J AD - Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Glaucoma JT - Journal of glaucoma JID - 9300903 SB - IM MH - Adult MH - Aged MH - Eye Abnormalities/complications MH - Female MH - Glaucoma, Open-Angle/*physiopathology MH - Humans MH - Intraocular Pressure/*physiology MH - Male MH - Middle Aged MH - Ocular Hypotension/*physiopathology MH - Optic Nerve/pathology MH - Phenotype MH - Retrospective Studies MH - Vascular Diseases/*complications MH - Visual Fields/physiology EDAT- 2018/09/07 06:00 MHDA- 2019/10/23 06:00 CRDT- 2018/09/07 06:00 PHST- 2018/09/07 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] PHST- 2018/09/07 06:00 [entrez] AID - 10.1097/IJG.0000000000001083 [doi] PST - ppublish SO - J Glaucoma. 2018 Nov;27(11):987-992. doi: 10.1097/IJG.0000000000001083. PMID- 31241661 OWN - NLM STAT- MEDLINE DCOM- 20191211 LR - 20220629 IS - 1980-5322 (Electronic) IS - 1807-5932 (Print) IS - 1807-5932 (Linking) VI - 74 DP - 2019 TI - The evaluation of the Myxovirus Resistance 1 protein in serum and saliva to monitor disease activation in primary Sjögren's syndrome. PG - e631 LID - 10.6061/clinics/2019/e631 [doi] LID - e631 AB - OBJECTIVE: Primary Sjögren's syndrome (pSjS) is a chronic autoimmune disease that causes dry eye and mouth. No laboratory parameters to monitor the activation of this disease have been identified. Therefore, any possible relationships between salivary and blood myxovirus resistance 1 (MX1) and pSjS must be prospectively studied. METHODS: Thirty female patients with pSjS, 30 women with rheumatoid arthritis (RA) without secondary Sjögren's syndrome (SjS) and 28 healthy control women were enrolled in this investigation. Analyses of MX1 by the enzyme-linked immunosorbent assay (ELISA) method, SS-A (Ro) and SS-B (La) tests by the strip immunoblot method, anti-nuclear antibody (ANA) tests by immunofluorescence and the measurement of serum rheumatoid factor (RF), C3, C4, immunoglobulin A (IgA), immunoglobulin M (IgM), and immunoglobulin G (IgG) were performed. RESULTS: The serum level of MX1 in patients without Raynaud phenomenon was higher than in those with Raynaud phenomenon (p:0.029, p<0.05, statistically significant). There was a statistically significant positive association between hemoglobin levels and MX1 serum levels. No statistically significant association was found among the other parameters. Low MX1 levels were shown to be associated with both a low disease activity score based on the European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and hydroxychloroquine use in all patients. CONCLUSION: MX1 levels have a considerable impact on the assessment of the disease activity in SjS. We believe that more-comprehensive studies should be performed on patients with pSjS who do not use hydroxychloroquine to prove this relationship and that MX1 levels should be used as a routine marker for the assessment of pSjS disease activity. Further studies are needed to create awareness of the role that MX1 has in the diagnosis of pSjS, which may help to uncover novel pathways for new therapeutic modalities. FAU - Aydemir, Yasemin Gul AU - Aydemir YG AUID- ORCID: 0000-0002-8421-7287 AD - Clinic of Internal Medicine, Malkara State Hospital, Tekirdag, Turkey. FAU - Kocakusak, Ahmet AU - Kocakusak A AUID- ORCID: 0000-0001-9685-6660 AD - General Surgery Clinic, Health Ministry Haseki Teaching and Research State Hospital, University of Health Sciences, Istanbul, Turkey. LA - eng PT - Journal Article DEP - 20190619 PL - United States TA - Clinics (Sao Paulo) JT - Clinics (Sao Paulo, Brazil) JID - 101244734 RN - 0 (Antibodies, Antinuclear) RN - 0 (Biomarkers) RN - 0 (Immunoglobulin A) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin Isotypes) RN - 0 (Immunoglobulin M) RN - 0 (Myxovirus Resistance Proteins) SB - IM MH - Adult MH - Antibodies, Antinuclear/blood MH - Biomarkers/analysis MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Immunoglobulin A/blood MH - Immunoglobulin G/blood MH - Immunoglobulin Isotypes/*blood MH - Immunoglobulin M/blood MH - Middle Aged MH - Myxovirus Resistance Proteins/*immunology MH - Saliva/*chemistry MH - Sjogren's Syndrome/diagnosis/immunology/*metabolism PMC - PMC6558995 COIS- No potential conflict of interest was reported. EDAT- 2019/06/27 06:00 MHDA- 2019/12/18 06:00 PMCR- 2019/01/01 CRDT- 2019/06/27 06:00 PHST- 2018/02/08 00:00 [received] PHST- 2019/05/14 00:00 [accepted] PHST- 2019/06/27 06:00 [entrez] PHST- 2019/06/27 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - S1807-5932(22)00627-5 [pii] AID - cln_74p1 [pii] AID - 10.6061/clinics/2019/e631 [doi] PST - ppublish SO - Clinics (Sao Paulo). 2019;74:e631. doi: 10.6061/clinics/2019/e631. Epub 2019 Jun 19. PMID- 19020548 OWN - NLM STAT- MEDLINE DCOM- 20090302 LR - 20260518 IS - 1476-5551 (Electronic) IS - 0887-6924 (Print) IS - 0887-6924 (Linking) VI - 23 IP - 2 DP - 2009 Feb TI - Specific cytogenetic abnormalities are associated with a significantly inferior outcome in children and adolescents with mature B-cell non-Hodgkin's lymphoma: results of the FAB/LMB 96 international study. PG - 323-31 LID - 10.1038/leu.2008.312 [doi] AB - Clinical studies showed that advanced stage, high LDH, poor response to reduction therapy and combined bone marrow and central nervous system disease are significantly associated with a decreased event-free survival (EFS) in pediatric mature B-cell non-Hodgkin's lymphoma (B-NHL) treated on FAB/LMB96. Although rearranged MYC/8q24 (R8q24) is characteristic of Burkitt lymphoma (BL), little information is available on other cytogenetic abnormalities and their prognostic importance. We performed an international review of 238 abnormal karyotypes in childhood mature B-NHL treated on FAB/LMB96: 76% BL, 8% Burkitt-like lymphoma, 13% diffuse large B-cell lymphoma (DLBCL). The main BL R8q24-associated chromosomal aberrations were +1q (29%), +7q and del(13q) (14% each). The DLBCL appeared heterogeneous and more complex. Incidence of R8q24 (34%) was higher than reported in adult DLBCL. The prognostic value of cytogenetic abnormalities on EFS was studied by Cox model controlling for the known risk factors: R8q24, +7q and del(13q) were independently associated with a significant inferior EFS (hazard ratio: 6.1 (P=0.030), 2.5 (P=0.015) and 4.0 (P=0.0003), respectively). The adverse prognosis of R8q24 was observed only in DLBCL, whereas del(13q) and +7q had a similar effect in DLBCL and BL. These results emphasize the significant biological heterogeneity and the development of cytogenetic risk-adapted therapy in childhood mature B-NHL. FAU - Poirel, H A AU - Poirel HA AD - Biological Hematological Department, CHU Avicenne-Université Paris 13, Bobigny, France. helene.antoine-poirel@uclouvain.be FAU - Cairo, M S AU - Cairo MS FAU - Heerema, N A AU - Heerema NA FAU - Swansbury, J AU - Swansbury J FAU - Aupérin, A AU - Aupérin A FAU - Launay, E AU - Launay E FAU - Sanger, W G AU - Sanger WG FAU - Talley, P AU - Talley P FAU - Perkins, S L AU - Perkins SL FAU - Raphaël, M AU - Raphaël M FAU - McCarthy, K AU - McCarthy K FAU - Sposto, R AU - Sposto R FAU - Gerrard, M AU - Gerrard M FAU - Bernheim, A AU - Bernheim A FAU - Patte, C AU - Patte C CN - FAB/LMB 96 International Study Committee LA - eng GR - U10 CA098543/CA/NCI NIH HHS/United States GR - CA 98543/CA/NCI NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20081120 PL - England TA - Leukemia JT - Leukemia JID - 8704895 SB - IM MH - Adolescent MH - Burkitt Lymphoma/genetics/mortality MH - Child MH - Child, Preschool MH - *Chromosome Aberrations MH - Disease-Free Survival MH - Female MH - Humans MH - Lymphoma, B-Cell/epidemiology/*genetics/mortality MH - Lymphoma, Large B-Cell, Diffuse/genetics/mortality MH - Male MH - Prognosis MH - Proportional Hazards Models MH - Randomized Controlled Trials as Topic MH - Young Adult PMC - PMC2988438 MID - NIHMS107551 FIR - Avet-Loiseau, H IR - Avet-Loiseau H FIR - Baranger, L IR - Baranger L FIR - Barin, C IR - Barin C FIR - Bastard, C IR - Bastard C FIR - Bernheim, A IR - Bernheim A FIR - Berthéas, M F IR - Berthéas MF FIR - Bilhou-Nabera, C IR - Bilhou-Nabera C FIR - Borie, C IR - Borie C FIR - Caillet-Bauchu, E IR - Caillet-Bauchu E FIR - Capdano, A M IR - Capdano AM FIR - Collonge-Rame, M A IR - Collonge-Rame MA FIR - Cornillet, P IR - Cornillet P FIR - Couturier, J IR - Couturier J FIR - Dastugue, N IR - Dastugue N FIR - Daudignon, A IR - Daudignon A FIR - Gachard, N IR - Gachard N FIR - Grégoire, M J IR - Grégoire MJ FIR - Heimann, P IR - Heimann P FIR - Henry, C IR - Henry C FIR - Laï, J L IR - Laï JL FIR - Leroux, D IR - Leroux D FIR - Lessard, M IR - Lessard M FIR - Luquet, I IR - Luquet I FIR - Mellink, C H M IR - Mellink CH FIR - Nadal, N IR - Nadal N FIR - Pagès, M P IR - Pagès MP FIR - Penther, D IR - Penther D FIR - Perissel, B IR - Perissel B FIR - Raynaud, S IR - Raynaud S FIR - Talman, P IR - Talman P FIR - Taviaux, S IR - Taviaux S FIR - Tigaud, I IR - Tigaud I FIR - Van den Akker, J IR - Van den Akker J FIR - Beigel, J IR - Beigel J FIR - Benn, P IR - Benn P FIR - Cantu, E IR - Cantu E FIR - Carlson, K IR - Carlson K FIR - Cooley, L IR - Cooley L FIR - Dawson, A IR - Dawson A FIR - Dev, V G IR - Dev VG FIR - Dewald, G IR - Dewald G FIR - Drumheller, T IR - Drumheller T FIR - Fink, J IR - Fink J FIR - Gadi, I IR - Gadi I FIR - Hanna, J IR - Hanna J FIR - Glassman, A IR - Glassman A FIR - Harrison, K IR - Harrison K FIR - Heerema, N IR - Heerema N FIR - Higgins, J IR - Higgins J FIR - Higgins, R IR - Higgins R FIR - Hirsch, B IR - Hirsch B FIR - Horsman, D IR - Horsman D FIR - Kalousek, D IR - Kalousek D FIR - Koduru, P IR - Koduru P FIR - Lebo, R IR - Lebo R FIR - Li, X IR - Li X FIR - Magenis, R E IR - Magenis RE FIR - McFadden, K IR - McFadden K FIR - McGavron, L IR - McGavron L FIR - McMorrow, L IR - McMorrow L FIR - Murch, A IR - Murch A FIR - Opheim, K IR - Opheim K FIR - Panzar, D IR - Panzar D FIR - Pasztor, L IR - Pasztor L FIR - Pettigrew, A IR - Pettigrew A FIR - Philips, C IR - Philips C FIR - Rao, K IR - Rao K FIR - Rao, P N IR - Rao PN FIR - Rouston, D IR - Rouston D FIR - Sanger, W IR - Sanger W FIR - Satya-Prakash, K L IR - Satya-Prakash KL FIR - Schwartz, S IR - Schwartz S FIR - Sekhon, G S IR - Sekhon GS FIR - Shaw, G IR - Shaw G FIR - Shekter-Levin, S IR - Shekter-Levin S FIR - Spinner, N IR - Spinner N FIR - Stanley, W IR - Stanley W FIR - Storto, P IR - Storto P FIR - Thangavelu, M IR - Thangavelu M FIR - Theil, K IR - Theil K FIR - Vance, G IR - Vance G FIR - VanDyke, D IR - VanDyke D FIR - Zadeh, T IR - Zadeh T FIR - Andrews, K IR - Andrews K FIR - Booth, M IR - Booth M FIR - Bown, N IR - Bown N FIR - Davies, T IR - Davies T FIR - Grace, E IR - Grace E FIR - Griffiths, M IR - Griffiths M FIR - Howard, P IR - Howard P FIR - Hughes, D IR - Hughes D FIR - Kempski, H IR - Kempski H FIR - Lillington, D IR - Lillington D FIR - Lowther, G IR - Lowther G FIR - Martin, K IR - Martin K FIR - Roberts, P IR - Roberts P FIR - Ross, F IR - Ross F FIR - Sadler, J IR - Sadler J FIR - Stallings, R IR - Stallings R FIR - Stevenson, D IR - Stevenson D FIR - Swansbury, J IR - Swansbury J FIR - Talley, P IR - Talley P FIR - Telford, N IR - Telford N FIR - Walker, H IR - Walker H EDAT- 2008/11/21 09:00 MHDA- 2009/03/03 09:00 PMCR- 2010/11/19 CRDT- 2008/11/21 09:00 PHST- 2008/11/21 09:00 [pubmed] PHST- 2009/03/03 09:00 [medline] PHST- 2008/11/21 09:00 [entrez] PHST- 2010/11/19 00:00 [pmc-release] AID - leu2008312 [pii] AID - 10.1038/leu.2008.312 [doi] PST - ppublish SO - Leukemia. 2009 Feb;23(2):323-31. doi: 10.1038/leu.2008.312. Epub 2008 Nov 20. PMID- 29494080 STAT- Publisher CTDT- 20240222 PB - StatPearls Publishing DP - 2026 Jan TI - Calcium Channel Blockers. BTI - StatPearls AB - Calcium channel blockers (CCBs), also known as calcium channel antagonists, have been approved by the US Food and Drug Administration (FDA) and are widely used to treat various conditions such as hypertension, coronary heart disease, and chronic stable angina. However, despite their widespread use, this class of cardiovascular drugs is one of the primary contributors to drug-related fatalities. CCBs are often classified into 2 major categories—non-dihydropyridines or dihydropyridines. The non-dihydropyridines include verapamil, classified as a phenylalkylamine, and diltiazem, categorized as a benzothiazepine. Cardiovascular indications include hypertension, coronary spasm, angina pectoris, supraventricular dysrhythmias, hypertrophic cardiomyopathy, and pulmonary hypertension. In addition, CCBs can also be used to treat certain off-label indications, such as Raynaud phenomenon, subarachnoid hemorrhage, and migraine headaches.  CI - Copyright © 2026, StatPearls Publishing LLC. FAU - McKeever, Rita G AU - McKeever RG AD - Drexel University School of Medicine FAU - Patel, Preeti AU - Patel P FAU - Hamilton, Richard J AU - Hamilton RJ AD - Drexel University LA - eng PT - Study Guide PT - Book Chapter PL - Treasure Island (FL) COIS- Disclosure: Rita McKeever declares no relevant financial relationships with ineligible companies. Disclosure: Preeti Patel declares no relevant financial relationships with ineligible companies. Disclosure: Richard Hamilton declares no relevant financial relationships with ineligible companies. EDAT- 2024/02/22 00:00 CRDT- 2024/02/22 00:00 AID - NBK482473 [bookaccession] PMID- 23745457 OWN - NLM STAT- MEDLINE DCOM- 20131217 LR - 20130610 IS - 0374-1338 (Print) IS - 0374-1338 (Linking) VI - 59 IP - 2 DP - 2012 TI - [Anti-synthetase syndrome]. PG - 53-7 AB - Antysynthetase syndrome is considered as a group ofidiopathic inflammatory myositis with charcteristic serologic hallmark--antibodies which recognise the aminoacyl-tRNA synthetasses (ARS). Clinical picture of those patients contains myositis and/or intersticial lung disease (ILD) and/or arthritis and/or fever and/or Raynaud phenomenon and sometimes characteristic look of mechanic's hands. Myositis can be overt, sometimes even absent, while IBP is major cause of morbidity and determines the outcome of the disease. Untill now eight different any-synthetase autoantibodies are recognised, and most frequent are findings of anti-histidyl-tRNa synthetase antibodies. Patients with other ARS autoantibodies usually have severe ILD. Drug of choice are steroids in dosage of 1 mg/kg with immunosupresive agent (azatioprin or methotrexate) while in severe IBP cyclophosphamide is needed. Recently succsesful treatment with rituximab in combination with cyclophosphamide is reported. FAU - Novak, Srdan AU - Novak S AD - Odjel za reumatologiju i klinicku imunologiju, Klinika za internu medicinu, Klinicki bolnicki centar Rijeka, Kresimirova 42, 51000 Rijeka. LA - hrv PT - English Abstract PT - Journal Article TT - Anti-sintetaza sindrom. PL - Croatia TA - Reumatizam JT - Reumatizam JID - 0216650 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/*analysis MH - Humans MH - Myositis/diagnosis/drug therapy/*immunology EDAT- 2012/01/01 00:00 MHDA- 2013/12/18 06:00 CRDT- 2013/06/11 06:00 PHST- 2013/06/11 06:00 [entrez] PHST- 2012/01/01 00:00 [pubmed] PHST- 2013/12/18 06:00 [medline] PST - ppublish SO - Reumatizam. 2012;59(2):53-7. PMID- 17113983 OWN - NLM STAT- MEDLINE DCOM- 20070123 LR - 20131121 IS - 0960-0760 (Print) IS - 0960-0760 (Linking) VI - 102 IP - 1-5 DP - 2006 Dec TI - Prostate cancer risk in testosterone-treated men. PG - 261-6 AB - Men with classical androgen deficiency have reduced prostate volume and blood prostate-specific antigen (PSA) levels compared with their age peers. As it is plausible that androgen deficiency partially protects against prostate disease, and that restoring androgen exposure increases risk to that of eugonadal men of the same age, men using ART should have age-appropriate surveillance for prostate disease. This should comprise rectal examination and blood PSA measurement at regular intervals (determined by age and family history) according to the recommendations, permanently revisited, published by ISSAM, EAU, Endocrine Society.... Testosterone replacement therapy is now being prescribed more often for aging men, the same population in which prostate cancer incidence increases; it has been suggested that administration in men with unrecognised prostate cancer might promote the development of clinically significant disease. In hypogonadal men who were candidates for testosterone therapy, a 14% incidence of occult cancer was found. A percentage (15.2%) of prostate cancer has been found in the placebo group (with normal DRE and PSA) in the prostate cancer prevention study investigating the chemoprevention potential of finasteride. The hypothesis that high levels of circulating androgens is a risk factor for prostate cancer is supported by the dramatic regression, after castration, of tumour symptoms in men with advanced prostate cancer. However these effects, seen at a very late stage of cancer development, may not be relevant to reflect the effects of variations within a physiological range at an earlier stage. Data from all published prospective studies on circulating level of total and free testosterone do not support the hypothesis that high levels of circulating androgens are associated with an increased risk of prostate cancer. A study on a large prospective cohort of 10,049 men, contributes to the gathering evidence that the long standing "androgen hypothesis" of increasing risk with increasing androgen levels can be rejected, suggesting instead that high levels within the reference range of androgens, estrogens and adrenal androgens decrease aggressive prostate cancer risk. Indeed, high-grade prostate cancer has been associated with low plasma level of testosterone. Furthermore, pre-treatment total testosterone was an independent predictor of extraprostatic disease in patients with localized prostate cancer; as testosterone decreases, patients have an increased likelihood of non-organ confined disease and low serum testosterone levels are associated with positive surgical margins in radical retropubic prostatectomy. A clinical implication of these results concerns androgen supplementation which has become easier to administer with the advent of transdermal preparations (patch or gel) that achieve physiological testosterone serum levels without supra physiological escape levels. During the clinical development of a new testosterone patch in more than 200 primary or secondary hypogonadal patients, no prostate cancer was diagnosed. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Université Pierre & Marie Curie, 51 bvd Suchet, Paris 75016, France. jean-pierre@raynaud.ws LA - eng PT - Journal Article PT - Review PL - England TA - J Steroid Biochem Mol Biol JT - The Journal of steroid biochemistry and molecular biology JID - 9015483 RN - 0 (Androgens) RN - 3XMK78S47O (Testosterone) SB - IM MH - Androgens/*adverse effects MH - Hormone Replacement Therapy MH - Humans MH - Male MH - Neoplasms, Hormone-Dependent MH - Prostatic Neoplasms/*chemically induced/physiopathology MH - Risk Factors MH - Testosterone/*adverse effects RF - 36 EDAT- 2006/11/23 09:00 MHDA- 2007/01/24 09:00 CRDT- 2006/11/23 09:00 PHST- 2006/11/23 09:00 [pubmed] PHST- 2007/01/24 09:00 [medline] PHST- 2006/11/23 09:00 [entrez] AID - S0960-0760(06)00274-3 [pii] AID - 10.1016/j.jsbmb.2006.09.032 [doi] PST - ppublish SO - J Steroid Biochem Mol Biol. 2006 Dec;102(1-5):261-6. doi: 10.1016/j.jsbmb.2006.09.032. PMID- 18626304 OWN - NLM STAT- MEDLINE DCOM- 20080813 LR - 20220419 IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 87 IP - 4 DP - 2008 Jul TI - Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. PG - 210-219 LID - 10.1097/MD.0b013e318181e6af [doi] AB - We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. The cohort included 937 women and 73 men (ratio, 13:1), with a mean age of 53 years at diagnosis and 59 years at inclusion in the registry. Multivariate analysis showed that male patients had a lower frequency of thyroiditis, Raynaud phenomenon, and antinuclear antibodies. Young-onset patients had a low degree of sicca involvement (xerostomia and parotid enlargement) and a high frequency of immunologic markers (anti-Ro/SS-A and low C4 levels). Patients with disease duration of more than 10 years had a higher prevalence of xerophthalmia, parotid enlargement, lung involvement, and peripheral neuropathy in comparison with incident cases. The subset of patients with anti-Ro/La antibodies had the highest prevalence of most systemic, hematologic, and immunologic alterations (higher frequency of Raynaud phenomenon, altered parotid scintigraphy, positive salivary gland biopsy, peripheral neuropathy, thrombocytopenia, and rheumatoid factor). Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia.Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement. FAU - Ramos-Casals, Manuel AU - Ramos-Casals M AD - From Laboratory of Autoimmune Diseases "Josep Font," Department of Autoimmune Diseases, IDIBAPS, Hospital Clínic (MRC), Barcelona; Department of Internal Medicine, Hospital Vall d'Hebron (RS), Barcelona; Rheumatology Unit, Hospital de Villajoyosa (JR), Alicante; Department of Internal Medicine, Hospital Carlos Haya (MTC), Malaga; Department of Internal Medicine, Hospital La Paz (AG), Madrid; Department of Internal Medicine and Rheumatology, Hospital Universitario de Salamanca (JdPM), Salamanca; Rheumatology Section, Hospital de Sierrallana (JCA), Torrelavega; Department of Internal Medicine, Hospital Virgen de las Nieves (JJA), Granada; Department of Internal Medicine, Hospital La Fe (MLM), Valencia; Rheumatology Unit, Hospital General de Castellón (JB), Castellón; Rheumatology Unit, Hospital 9 d'Octubre (RB), Valencia; Department of Internal Medicine, Hospital de Son Dureta (LP), Palma de Mallorca; Spain. FAU - Solans, Roser AU - Solans R FAU - Rosas, Jose AU - Rosas J FAU - Camps, María Teresa AU - Camps MT FAU - Gil, Antonio AU - Gil A FAU - Del Pino-Montes, Javier AU - Del Pino-Montes J FAU - Calvo-Alen, Jaime AU - Calvo-Alen J FAU - Jiménez-Alonso, Juan AU - Jiménez-Alonso J FAU - Micó, Maria-Luisa AU - Micó ML FAU - Beltrán, Juan AU - Beltrán J FAU - Belenguer, Rafael AU - Belenguer R FAU - Pallarés, Lucio AU - Pallarés L CN - GEMESS Study Group LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Biomarkers) SB - IM MH - Adolescent MH - Adult MH - Age Factors MH - Aged MH - Aged, 80 and over MH - Biomarkers/analysis MH - Cohort Studies MH - Female MH - Humans MH - Male MH - Middle Aged MH - Sex Factors MH - Sjogren's Syndrome/epidemiology/*immunology/physiopathology MH - Spain/epidemiology EDAT- 2008/07/16 09:00 MHDA- 2008/08/14 09:00 CRDT- 2008/07/16 09:00 PHST- 2008/07/16 09:00 [pubmed] PHST- 2008/08/14 09:00 [medline] PHST- 2008/07/16 09:00 [entrez] AID - 00005792-200807000-00004 [pii] AID - 10.1097/MD.0b013e318181e6af [doi] PST - ppublish SO - Medicine (Baltimore). 2008 Jul;87(4):210-219. doi: 10.1097/MD.0b013e318181e6af. PMID- 18210018 OWN - NLM STAT- MEDLINE DCOM- 20090406 LR - 20211020 IS - 1432-1289 (Electronic) IS - 0020-9554 (Linking) VI - 49 IP - 3 DP - 2008 Mar TI - [Systemic sclerosis]. PG - 278-85 LID - 10.1007/s00108-007-2011-x [doi] AB - Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as well as in Europe (EUSTAR), a detailed profile of the affected patients could be determined. No specific predictive markers for the disease exist in the early phases of the disease although a Raynaud phenomenon can be present up to ten years prior to the first organ manifestations. On the other hand, distinct clinical features and laboratory parameters can be independent predictive markers for the outcome of a given patient. Therefore, monitoring should be performed on a regular basis in the preclinical and early stages of the disease, as early diagnosis can reduce morbidity and mortality of the affected patients significantly, especially with regard to pulmonary hypertension, digital ischemia, reflux esophagitis and sclerodermal renal crisis. FAU - Müller-Ladner, U AU - Müller-Ladner U AD - Lehrstuhl für Innere Medizin mit Schwerpunkt Rheumatologie, Justus-Liebig-Universität, Giessen. u.mueller-ladner@kerckhoff-klinik.de LA - ger PT - English Abstract PT - Journal Article PT - Review TT - Systemische Sklerose. PL - Germany TA - Internist (Berl) JT - Der Internist JID - 0264620 SB - IM MH - Humans MH - Rheumatology/*methods/*trends MH - Scleroderma, Systemic/*diagnosis/*therapy RF - 12 EDAT- 2008/01/23 09:00 MHDA- 2009/04/07 09:00 CRDT- 2008/01/23 09:00 PHST- 2008/01/23 09:00 [pubmed] PHST- 2009/04/07 09:00 [medline] PHST- 2008/01/23 09:00 [entrez] AID - 10.1007/s00108-007-2011-x [doi] PST - ppublish SO - Internist (Berl). 2008 Mar;49(3):278-85. doi: 10.1007/s00108-007-2011-x. PMID- 16924851 OWN - NLM STAT- MEDLINE DCOM- 20061020 LR - 20131106 IS - 0049-1101 (Print) IS - 0049-1101 (Linking) VI - 51 IP - 1 DP - 2006 TI - [Senegalese case of thromboangeitis obliterans or Buerger's disease]. PG - 53-6 AB - INTRODUCTION: Thromboangeitis obliterans (TAO) is an inflammatory, non atheromatous arteriopathy of smoking young adults. It is diagnosed on an association of non specific criteria that we discuss throughout this case. CASE REPORT AND DISCUSSION: A forty years old tabagical, Senegalese black man, had peripheral destructive lesions preceded by Raynaud phenomenon. He was admitted in our Internal Medicine department in November 2002. Actually this clinical presentation was evolving since 11 years. At that time, hypo aesthesia and ulceration of the fingers led to successive amputations in the leprology centre. The diagnosis of Hansen disease had been suspected but there were no evidence of mycobacterium. At the admission in our service, biological tests showed a moderated non-specific inflammatory syndrome. Ultra sound Doppler and arteriography showed a peripheral arterial stenosis without atheromatous lesions, in favour of TAO. To meet all the criteria the patient didn't have any thrombotic or systemic disease. The evolution was favourable after tobacco weaning. CONCLUSION: TAO can bring to difficulties of diagnosis by its way of presentation. Physicians should practice a systematic vascular screening in case of distal arteriopathy. FAU - Pouye, A AU - Pouye A AD - Clinique Médicale I CHU Aristide Le Dantec, Dakar. apouye@refer.sn FAU - Ndong, S AU - Ndong S FAU - Diallo, S AU - Diallo S FAU - Ba Diop, S AU - Ba Diop S FAU - Fall, S AU - Fall S FAU - Leye, A AU - Leye A FAU - Ka, E F AU - Ka EF FAU - Niang, A AU - Niang A FAU - Ka, M M AU - Ka MM FAU - Moreira Diop, T AU - Moreira Diop T LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Premiere observation Senegalaise de thromboangeite obliterante ou maladie de Buerger. PL - Senegal TA - Dakar Med JT - Dakar medical JID - 7907630 SB - IM MH - Adult MH - Humans MH - Male MH - Senegal MH - Smoking/adverse effects MH - Thromboangiitis Obliterans/*diagnosis EDAT- 2006/08/24 09:00 MHDA- 2006/10/21 09:00 CRDT- 2006/08/24 09:00 PHST- 2006/08/24 09:00 [pubmed] PHST- 2006/10/21 09:00 [medline] PHST- 2006/08/24 09:00 [entrez] PST - ppublish SO - Dakar Med. 2006;51(1):53-6. PMID- 24112952 OWN - NLM STAT- MEDLINE DCOM- 20141009 LR - 20200205 IS - 1778-7254 (Electronic) IS - 1297-319X (Linking) VI - 81 IP - 2 DP - 2014 Mar TI - Fibroblastic rheumatism: immunosuppressive therapy is not always required. PG - 178-9 LID - S1297-319X(13)00182-6 [pii] LID - 10.1016/j.jbspin.2013.06.012 [doi] AB - Fibroblastic rheumatism is a very rare cause of distal and bilateral polyarthritis characterized by cutaneous nodules, sclerodactylitis, thickened palmar fascia and Raynaud phenomenon. Physiopathology remains unknown and the diagnosis is histologic. Despite the use of immunosuppressive agents in some isolated cases with a variable efficacy, we report a case of typical fibroblastic rheumatism with severe digital retraction who dramatically improved after intensive physical therapy without immunosuppressive drugs prescription. Such a case illustrates that improvement may be spontaneous and that non pharmacological approach is a cornerstone in the management of this disease. CI - Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved. FAU - Courties, Alice AU - Courties A AD - Service de rhumatologie, université Paris 06, hôpital Saint-Antoine, AP-HP, DHU « inflammation, immunopathologie, biothérapie » I2B, Paris, France. FAU - Guégan, Sarah AU - Guégan S AD - Service de dermatologie, université Paris 06, hôpital Tenon, AP-HP, Paris, France. FAU - Miquel, Anne AU - Miquel A AD - Service d'imagerie médicale, université Paris 06, hôpital Saint-Antoine, AP-HP, Paris, France. FAU - Duriez, Paul AU - Duriez P AD - Service d'anatomopathologie, université Paris 06, hôpital Tenon, AP-HP, Paris, France. FAU - Berenbaum, Francis AU - Berenbaum F AD - Service de rhumatologie, université Paris 06, hôpital Saint-Antoine, AP-HP, DHU « inflammation, immunopathologie, biothérapie » I2B, Paris, France. Electronic address: francis.berenbaum@sat.aphp.fr. FAU - Sellam, Jérémie AU - Sellam J AD - Service de rhumatologie, université Paris 06, hôpital Saint-Antoine, AP-HP, DHU « inflammation, immunopathologie, biothérapie » I2B, Paris, France. LA - eng PT - Case Reports PT - Journal Article DEP - 20131007 PL - France TA - Joint Bone Spine JT - Joint bone spine JID - 100938016 SB - IM MH - Adult MH - Fibroblasts/pathology MH - Fibrosis MH - Humans MH - Male MH - Physical Therapy Modalities MH - Rheumatic Diseases/*diagnosis/*therapy OTO - NOTNLM OT - Fibroblastic rheumatism OT - Immunosuppressive drugs OT - Non-pharmacological treatment OT - Physical therapy EDAT- 2013/10/12 06:00 MHDA- 2014/10/10 06:00 CRDT- 2013/10/12 06:00 PHST- 2013/03/26 00:00 [received] PHST- 2013/06/30 00:00 [accepted] PHST- 2013/10/12 06:00 [entrez] PHST- 2013/10/12 06:00 [pubmed] PHST- 2014/10/10 06:00 [medline] AID - S1297-319X(13)00182-6 [pii] AID - 10.1016/j.jbspin.2013.06.012 [doi] PST - ppublish SO - Joint Bone Spine. 2014 Mar;81(2):178-9. doi: 10.1016/j.jbspin.2013.06.012. Epub 2013 Oct 7. PMID- 20862203 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1687-9279 (Electronic) IS - 1687-9260 (Print) IS - 1687-9260 (Linking) VI - 2010 DP - 2010 TI - Registry evaluation of digital ulcers in systemic sclerosis. LID - 363679 [pii] LID - 10.1155/2010/363679 [doi] AB - Digital ulcers are a very frequent complication of systemic sclerosis affecting about half of the SSc patients, and about 75% of the affected patients have their first DU episode within 5 years from their first non-Raynaud symptom. The lack of adequate classification criteria as well as the lack of knowledge of the development of DU have contributed to the opening of specific registries to better understand the natural history of these lesions. For these reason, specific disease registries play a fundamental role in this field of research. Thanks to the systematic collection of data and their subsequent analysis and comparison between different cohorts, it is possible to improve understanding of the underlying trigger mechanisms of DU development and to determine temporal trends. In the future, the development of recommendations for the management of DU remains of pivotal importance to prevent DU development and obtain rapid healing as well as reduction of pain and disability. FAU - Galluccio, Felice AU - Galluccio F AD - Division of Rheumatology AOUC, Department of Biomedicine, Denothe Centre, University of Florence, Villa Monna Tessa, viale Pieraccini 18, 50139 Firenze, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M LA - eng PT - Journal Article DEP - 20100825 PL - United States TA - Int J Rheumatol JT - International journal of rheumatology JID - 101519204 PMC - PMC2938432 EDAT- 2010/09/24 06:00 MHDA- 2010/09/24 06:01 PMCR- 2010/08/25 CRDT- 2010/09/24 06:00 PHST- 2010/05/17 00:00 [received] PHST- 2010/08/02 00:00 [accepted] PHST- 2010/09/24 06:00 [entrez] PHST- 2010/09/24 06:00 [pubmed] PHST- 2010/09/24 06:01 [medline] PHST- 2010/08/25 00:00 [pmc-release] AID - 363679 [pii] AID - 10.1155/2010/363679 [doi] PST - ppublish SO - Int J Rheumatol. 2010;2010:363679. doi: 10.1155/2010/363679. Epub 2010 Aug 25. PMID- 31050209 OWN - NLM STAT- MEDLINE DCOM- 20200129 LR - 20200129 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 22 IP - 7 DP - 2019 Jul TI - Capillaroscopy changes are associated with disease progression in patients with early systemic sclerosis: A prospective study. PG - 1319-1326 LID - 10.1111/1756-185X.13592 [doi] AB - AIM: After the development of the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis (SSc), there are still a group of patients affected by early SSc who do not meet the new criteria. This study aimed to evaluate capillaroscopy changes and to identify predictors of progression to definite SSc in patients with early SSc over a 3-year follow-up. METHODS: In this prospective study, 44 patients with early SSc (LeRoy and Medsger 2001 criteria) were included. Clinical evaluation and widefield nailfold capillaroscopy were performed at baseline and after 3 years of follow-up. At the end of follow-up, the fulfilment of the 2013 ACR/EULAR criteria was also assessed. RESULTS: After 3 years, 34 patients with early SSc were re-evaluated. Of these, eight patients (23.5%) developed definite SSc. Worsening of capillaroscopy parameters was observed in 55.9% of patients. An increase in the number of giant capillaries and worsening of the avascular score were more frequent in patients who developed SSc than in those who did not (P = 0.02; P = 0.002, respectively). By multivariate analysis, an active or a late pattern at baseline on capillaroscopy was an independent predictor for the development of definite SSc (odds ratio = 30.0, 95% CI 2.1-421.1). CONCLUSIONS: In this prospective study, worsening in capillaroscopy parameters was observed in early SSc patients. An active or a late pattern on capillaroscopy was an independent predictive risk factor for the development of SSc, suggesting that capillaroscopy might be a useful tool to identify patients with early SSc at risk of disease progression. CI - © 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Zumstein Camargo, Cintia AU - Zumstein Camargo C AD - Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Kayser, Cristiane AU - Kayser C AUID- ORCID: 0000-0003-0543-5305 AD - Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil. LA - eng PT - Journal Article DEP - 20190502 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Adult MH - Aged MH - Capillaries/*diagnostic imaging MH - Disease Progression MH - Female MH - Humans MH - Longitudinal Studies MH - Male MH - *Microscopic Angioscopy MH - Middle Aged MH - Nails/*blood supply MH - Predictive Value of Tests MH - Prospective Studies MH - Scleroderma, Systemic/*diagnostic imaging MH - Time Factors OTO - NOTNLM OT - Raynaud phenomenon OT - disease progression OT - nailfold capillaroscopy OT - prospective study OT - systemic sclerosis EDAT- 2019/05/03 06:00 MHDA- 2020/01/30 06:00 CRDT- 2019/05/04 06:00 PHST- 2018/06/20 00:00 [received] PHST- 2019/02/12 00:00 [revised] PHST- 2019/04/07 00:00 [accepted] PHST- 2019/05/03 06:00 [pubmed] PHST- 2020/01/30 06:00 [medline] PHST- 2019/05/04 06:00 [entrez] AID - 10.1111/1756-185X.13592 [doi] PST - ppublish SO - Int J Rheum Dis. 2019 Jul;22(7):1319-1326. doi: 10.1111/1756-185X.13592. Epub 2019 May 2. PMID- 40924593 OWN - NLM STAT- MEDLINE DCOM- 20260203 LR - 20260430 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 65 IP - 1 DP - 2026 Jan 8 TI - Impact of hand function impairment on daily life of patients with systemic sclerosis: a qualitative study. LID - 10.1093/rheumatology/keaf476 [doi] LID - keaf476 AB - OBJECTIVES: Many patients with systemic sclerosis (SSc) experience impaired hand function, yet the precise nature and impact of this impairment remains unclear. In this study, we explored the determinants of hand function impairment in SSc from a patient perspective and its impact on daily life. Additionally, we identified unmet care needs related to hand function impairment. METHODS: Adult patients with SSc were included from the University Medical Centre Utrecht, the Netherlands, and Royal Free Hospital London, United Kingdom (UK). Face-to-face semi-structured interviews were conducted, transcribed verbatim and coded. Thematic analysis was performed to identify key themes. Hand function was evaluated using the modified Hand Mobility in Scleroderma (mHAMIS) and the Cochin Hand Function Scale (CHFS). RESULTS: Thirty-three patients were included (n = 18 in the Netherlands, n = 15 in the UK). Three main themes were identified: symptoms, impact and (un)met needs. The symptoms theme captures the broad range of medical and functional complaints, often co-occurring and leading to significant hand function impairment. The impact theme describes how these symptoms limited daily activities, employment and leisure, and contributed to emotional distress and social isolation. The (un)met needs theme highlights varied coping strategies and experiences with care. While participants felt that patient education was sufficient when healthcare professionals addressed hand impairment, many reported a lack of tailored support and insufficient recognition of hand-related problems. CONCLUSION: Hand function impairment in SSc profoundly affects patients' daily lives and well-being. Addressing this unmet need requires greater clinical awareness and more personalised and symptom-specific management strategies. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Greveling, Mark AU - Greveling M AUID- ORCID: 0000-0003-3811-6511 AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - Rodolfi, Stefano AU - Rodolfi S AUID- ORCID: 0000-0001-8054-2843 AD - Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital and University College London, London, UK. FAU - El Bardai, Nora AU - El Bardai N AUID- ORCID: 0009-0007-9267-5695 AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital and University College London, London, UK. FAU - Ong, Voon H AU - Ong VH AUID- ORCID: 0000-0001-5474-0053 AD - Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital and University College London, London, UK. FAU - Jeffries-Owen, Nick AU - Jeffries-Owen N AD - Scleroderma & Raynaud's UK (SRUK), London, United Kingdom. FAU - Schriemer, Rita AU - Schriemer R AUID- ORCID: 0009-0006-2591-9971 AD - National Association for People with Lupus, Systemic Sclerosis, Antiphospholipid Syndrome, and Mixed Connective Tissue Disease (NVLE), Utrecht, The Netherlands. FAU - Tweehuysen, Lieke AU - Tweehuysen L AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. FAU - Spierings, Julia AU - Spierings J AUID- ORCID: 0000-0002-2546-312X AD - Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands. AD - Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital and University College London, London, UK. LA - eng GR - 22-1-403/The Dutch Arthritis Foundation/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Scleroderma, Systemic/physiopathology/psychology/complications MH - Female MH - Male MH - *Activities of Daily Living MH - Middle Aged MH - Qualitative Research MH - Aged MH - Adult MH - *Hand/physiopathology MH - Quality of Life MH - Netherlands MH - United Kingdom PMC - PMC12862391 OTO - NOTNLM OT - hand function impairment OT - interview studies OT - qualitative research OT - scleroderma OT - systemic sclerosis EDAT- 2025/09/09 18:28 MHDA- 2026/02/03 07:40 PMCR- 2025/09/09 CRDT- 2025/09/09 12:43 PHST- 2025/03/20 00:00 [received] PHST- 2025/08/26 00:00 [accepted] PHST- 2026/02/03 07:40 [medline] PHST- 2025/09/09 18:28 [pubmed] PHST- 2025/09/09 12:43 [entrez] PHST- 2025/09/09 00:00 [pmc-release] AID - 8250126 [pii] AID - keaf476 [pii] AID - 10.1093/rheumatology/keaf476 [doi] PST - ppublish SO - Rheumatology (Oxford). 2026 Jan 8;65(1):keaf476. doi: 10.1093/rheumatology/keaf476. PMID- 30480566 OWN - NLM STAT- MEDLINE DCOM- 20210624 LR - 20210624 IS - 1539-2031 (Electronic) IS - 0192-0790 (Linking) VI - 54 IP - 2 DP - 2020 Feb TI - Pancreatic Hormone Responses to Mixed Meal Test in New-onset Prediabetes/Diabetes After Non-necrotizing Acute Pancreatitis. PG - e11-e20 LID - 10.1097/MCG.0000000000001145 [doi] AB - AIM: To investigate the pancreatic hormone responses to mixed meal test, in particular changes in insulin secretion, insulin sensitivity, and their interrelationship, in individuals with new-onset prediabetes or diabetes after non-necrotizing acute pancreatitis (NODAP) compared with healthy controls. METHODS: Twenty-nine individuals with NODAP and 29 age-and sex-matched healthy controls were recruited. All participants (after fasting for at least 8 h) were given 12 oz. of BOOST drink and blood samples were collected before and after stimulation to measure insulin, C-peptide, glucagon, and pancreatic polypeptide. Indices of insulin sensitivity (HOMA-IS, 1/fasting insulin, Raynaud, and Matsuda) and insulin secretion (HOMA-β, Stumvoll, insulinogenic index 30' and 60') were calculated. Repeated measures analyses were conducted in the unadjusted and adjusted models. RESULTS: Insulin and C-peptide levels were significantly higher in individuals with NODAP compared with controls during mixed meal test in both the unadjusted (P=0.001 for both) and adjusted (P=0.004 and P=0.006, respectively) models. HOMA-IS (P=0.005), 1/fasting insulin (P=0.018), Raynaud index (P=0.018), and Matsuda index (P=0.021) were significantly lower in individuals with NODAP, whereas HOMA-β (P=0.028) and Stumvoll index (P=0.013) were significantly higher. Glucagon and pancreatic polypeptide levels did not differ significantly between NODAP and controls during mixed meal test in both the unadjusted (P=0.345 and P=0.206, respectively) and adjusted (P=0.359 and P=0.158, respectively) models. CONCLUSIONS: Decreased insulin sensitivity, β-cell compensation, and no significant change in postprandial levels of glucagon and pancreatic polypeptide characterize NODAP. The above findings may help develop an evidence-based protocol with a view to optimize control of glucose homeostasis in NODAP. FAU - Pendharkar, Sayali A AU - Pendharkar SA AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Singh, Ruma G AU - Singh RG FAU - Bharmal, Sakina H AU - Bharmal SH FAU - Drury, Marie AU - Drury M FAU - Petrov, Maxim S AU - Petrov MS LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Clin Gastroenterol JT - Journal of clinical gastroenterology JID - 7910017 RN - 0 (Blood Glucose) RN - 0 (Insulin) RN - 0 (Pancreatic Hormones) SB - IM MH - Acute Disease MH - Blood Glucose MH - *Diabetes Mellitus, Type 2 MH - Humans MH - Insulin MH - *Insulin Resistance MH - Pancreatic Hormones MH - *Pancreatitis/diagnosis/etiology MH - *Prediabetic State/diagnosis EDAT- 2018/11/28 06:00 MHDA- 2021/06/25 06:00 CRDT- 2018/11/28 06:00 PHST- 2018/11/28 06:00 [pubmed] PHST- 2021/06/25 06:00 [medline] PHST- 2018/11/28 06:00 [entrez] AID - 00004836-202002000-00002 [pii] AID - 10.1097/MCG.0000000000001145 [doi] PST - ppublish SO - J Clin Gastroenterol. 2020 Feb;54(2):e11-e20. doi: 10.1097/MCG.0000000000001145. PMID- 28613625 STAT- Publisher CTDT- 20240405 PB - StatPearls Publishing DP - 2026 Jan TI - Systemic Sclerosis (Scleroderma). BTI - StatPearls AB - Systemic sclerosis, also known as scleroderma, is a rare connective tissue disorder with an unknown and complex pathogenesis. Scleroderma can be divided into 2 primary forms—localized scleroderma (including morphea, linear scleroderma, and scleroderma en coup de sabre) and systemic sclerosis. Systemic sclerosis can be further classified as limited systemic sclerosis (formerly known as CREST syndrome, characterized by calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) or diffuse systemic sclerosis based on clinical and serological criteria. Although significant progress has been made in understanding the pathophysiology of scleroderma over the past centuries, the disease continues to pose significant morbidity and mortality in patients. Localized scleroderma primarily affects the skin and subcutaneous tissue, leading to patches of thickened skin that, on biopsy, reveal dermal fibrosis similar to the histopathological changes seen in the thickened skin in systemic sclerosis. However, it is not associated with the Raynaud phenomenon, digital ischemic events, or internal organ involvement. Antinuclear antibodies may be present in up to 50% of cases of localized scleroderma; however, more specific autoantibodies such as anti-centromere, anti-Scl-70, and anti-RNA polymerase III are absent in this condition. Notably, localized scleroderma is not associated with increased mortality. On the other hand, systemic sclerosis is associated with several systemic manifestations and internal organ involvement, leading to increased mortality, and its classification is based on skin involvement.  Limited cutaneous systemic sclerosis, previously known as CREST syndrome, is characterized by skin thickening distal to the elbows and knees and/or on the face without trunk involvement. On the other hand, diffuse cutaneous systemic sclerosis involves skin thickening that may affect areas proximal to the elbows, knees, face, and/or trunk. Both limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis are associated with several systemic manifestations and positive autoantibodies. Antinuclear antibodies may be present in more than 90% of cases of systemic sclerosis, and up to 70% of cases exhibit at least one of the more specific autoantibodies (anti-centromere, anti-Scl-70, and anti-RNA polymerase III). Scleroderma most commonly affects the skin, gastrointestinal tract, lungs, kidneys, skeletal muscle, and pericardium among affected organs. The manifestations of scleroderma may overlap extensively with those of other rheumatological or immunological diseases. The severity of the presentation may also vary depending on the timing of the systemic sclerosis diagnosis. CI - Copyright © 2026, StatPearls Publishing LLC. FAU - Adigun, Rotimi AU - Adigun R AD - Touro University College of Osteopathic Medicine FAU - Goyal, Amandeep AU - Goyal A AD - University of Kansas Medical Center FAU - Hariz, Anis AU - Hariz A AD - Charles Nicolle Hospital, Un. of Tunis El Manar LA - eng PT - Study Guide PT - Book Chapter PL - Treasure Island (FL) COIS- Disclosure: Rotimi Adigun declares no relevant financial relationships with ineligible companies. Disclosure: Amandeep Goyal declares no relevant financial relationships with ineligible companies. Disclosure: Anis Hariz declares no relevant financial relationships with ineligible companies. EDAT- 2024/04/05 00:00 CRDT- 2024/04/05 00:00 AID - NBK430875 [bookaccession] PMID- 36760807 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230211 IS - 2090-6889 (Print) IS - 2090-6897 (Electronic) IS - 2090-6897 (Linking) VI - 2023 DP - 2023 TI - Antisynthetase Syndrome in a Patient with Pulmonary Embolism and Nonbacterial Thrombotic Endocarditis. PG - 9068597 LID - 10.1155/2023/9068597 [doi] LID - 9068597 AB - Antisynthetase syndrome is a rare autoimmune disease within the subset of idiopathic inflammatory myopathies. The diagnostic criteria include the presence of an aminoacyl-tRNA synthetase antibody, and typical clinical findings, including myositis, mechanic's hands, Raynaud phenomenon, unexplained fever, and interstitial lung disease. We describe a case of a 59-year-old male who presented with a 1-month history of progressive purplish discoloration and pain of the fingertips, dyspnea, cough, weight loss, fatigue, and who developed progressive proximal muscle weakness and dysphagia. Investigations revealed pulmonic valve and mitral valve marantic endocarditis, pulmonary embolism, myositis, organizing pneumonia, and elevation of anti-OJ antibodies. He was diagnosed with antisynthetase syndrome and treated with high dose corticosteroids and mycophenolate mofetil with a fair response. CI - Copyright © 2023 Anusha Vege et al. FAU - Vege, Anusha AU - Vege A AUID- ORCID: 0000-0002-9671-220X AD - Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA. FAU - Beery, Jesse AU - Beery J AUID- ORCID: 0000-0002-6078-8317 AD - Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA. FAU - Kara, Areeba AU - Kara A AUID- ORCID: 0000-0001-5107-3857 AD - Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20230131 PL - United States TA - Case Rep Rheumatol JT - Case reports in rheumatology JID - 101585353 PMC - PMC9904917 COIS- The authors declare that they have no conflicts of interest. EDAT- 2023/02/11 06:00 MHDA- 2023/02/11 06:01 PMCR- 2023/01/31 CRDT- 2023/02/10 03:01 PHST- 2022/04/05 00:00 [received] PHST- 2023/01/05 00:00 [revised] PHST- 2023/01/11 00:00 [accepted] PHST- 2023/02/10 03:01 [entrez] PHST- 2023/02/11 06:00 [pubmed] PHST- 2023/02/11 06:01 [medline] PHST- 2023/01/31 00:00 [pmc-release] AID - 10.1155/2023/9068597 [doi] PST - epublish SO - Case Rep Rheumatol. 2023 Jan 31;2023:9068597. doi: 10.1155/2023/9068597. eCollection 2023. PMID- 21837935 OWN - NLM STAT- MEDLINE DCOM- 20110906 LR - 20160307 IS - 1784-3286 (Print) IS - 1784-3286 (Linking) VI - 66 IP - 3 DP - 2011 May-Jun TI - Membranous glomerulopathy in a patient with selective IgA deficiency: is there a link? PG - 228-30 AB - We report a 42-year-old woman, who presented with proteinuria (3.85 g/day) and malleolar oedema. She had a medical history of Graves' disease, recurrent upper respiratory tract infections, episodes of Raynaud phenomenon and dysphagia. Biochemistry showed a selective IgA deficiency (SIgAD). Percutaneous renal needle biopsy showed diffuse global thickening of the glomerular basement membranes on light microscopy and granular deposits of IgG and C3 along the glomerular basement membranes on immunofluorescence. The pathological diagnosis was membranous glomerulopathy stage II. A treatment with dietary salt restriction and an angiotensin-converting enzyme inhibitor was initiated, resulting in a reduction of proteinuria. Despite the fact that selective IgA deficiency is associated with various autoimmune disorders, the association with glomerular disease is rather rare and the pathogenesis is not fully understood. FAU - Vanacker, A AU - Vanacker A AD - Department of Nephrology, Heilig Hartziekenhuis Roeselare-Menen, Roeselare, Belgium. FAU - Van Dorpe, J AU - Van Dorpe J FAU - Maes, B AU - Maes B LA - eng PT - Case Reports PT - Journal Article PL - England TA - Acta Clin Belg JT - Acta clinica Belgica JID - 0370306 RN - 0 (Immunoglobulin G) SB - IM MH - Adult MH - Basement Membrane/pathology MH - Comorbidity MH - Female MH - Glomerulonephritis, Membranous/blood/*epidemiology/pathology MH - Humans MH - IgA Deficiency/blood/*epidemiology MH - Immunoglobulin G/metabolism MH - Kidney Glomerulus/pathology EDAT- 2011/08/16 06:00 MHDA- 2011/09/07 06:00 CRDT- 2011/08/16 06:00 PHST- 2011/08/16 06:00 [entrez] PHST- 2011/08/16 06:00 [pubmed] PHST- 2011/09/07 06:00 [medline] AID - 10.2143/ACB.66.3.2062554 [doi] PST - ppublish SO - Acta Clin Belg. 2011 May-Jun;66(3):228-30. doi: 10.2143/ACB.66.3.2062554. PMID- 37132021 OWN - NLM STAT- MEDLINE DCOM- 20230504 LR - 20230614 IS - 2324-7096 (Electronic) IS - 2324-7096 (Linking) VI - 11 DP - 2023 Jan-Dec TI - Non-cirrhotic Portal Hypertension as the Initial Presentation of Limited Cutaneous Scleroderma: A Case Report. PG - 23247096231171251 LID - 10.1177/23247096231171251 [doi] LID - 23247096231171251 AB - Systemic sclerosis (SSc) is an autoimmune disease characterized by progressive skin fibrosis. It has 2 main clinical subtypes-diffuse cutaneous scleroderma and limited cutaneous scleroderma. Non-cirrhotic portal hypertension (NCPH) is defined as presence of elevated portal vein pressures without cirrhosis. It is often a manifestation of an underlying systemic disease. On histopathology, NCPH may be found to be secondary to multiple abnormalities such as nodular regenerative hyperplasia (NRH) and obliterative portal venopathy. There have been reports of NCPH in patients with both subtypes of SSc secondary to NRH. However, simultaneous presence of obliterative portal venopathy has not been reported. We present a case of NCPH due to NRH and obliterative portal venopathy as a presenting sign of limited cutaneous scleroderma. The patient was initially found to have pancytopenia and splenomegaly and was erroneously labeled as cirrhosis. She underwent workup to rule out leukemia, which was negative. She was referred to our clinic and diagnosed with NCPH. Due to pancytopenia, she could not be started on immunosuppressive therapy for her SSc. Our case describes the presence of these unique pathological findings in the liver and highlights the importance of an aggressive search for an underlying condition in all patients diagnosed with NCPH. FAU - Hitawala, Asif Ali AU - Hitawala AA AUID- ORCID: 0000-0002-2888-0172 AD - National Institutes of Health, Bethesda, MD, USA. FAU - Redmond, Christopher AU - Redmond C AD - National Institutes of Health, Bethesda, MD, USA. FAU - Cowen, Edward W AU - Cowen EW AD - National Institutes of Health, Bethesda, MD, USA. FAU - Kleiner, David E AU - Kleiner DE AD - National Institutes of Health, Bethesda, MD, USA. FAU - Hasni, Sarfaraz AU - Hasni S AD - National Institutes of Health, Bethesda, MD, USA. FAU - Heller, Theo AU - Heller T AD - National Institutes of Health, Bethesda, MD, USA. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Intramural PL - United States TA - J Investig Med High Impact Case Rep JT - Journal of investigative medicine high impact case reports JID - 101624758 SB - IM MH - Female MH - Humans MH - Portal Vein/pathology MH - *Pancytopenia/etiology MH - *Hypertension, Portal/etiology/complications MH - Liver Cirrhosis/complications MH - *Vascular Diseases MH - *Scleroderma, Systemic/complications/diagnosis PMC - PMC10161292 OTO - NOTNLM OT - Raynaud phenomenon OT - elastography OT - idiopathic non-cirrhotic portal hypertension OT - systemic sclerosis COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/05/03 06:42 MHDA- 2023/05/04 12:42 PMCR- 2023/05/02 CRDT- 2023/05/03 02:03 PHST- 2023/05/04 12:42 [medline] PHST- 2023/05/03 06:42 [pubmed] PHST- 2023/05/03 02:03 [entrez] PHST- 2023/05/02 00:00 [pmc-release] AID - 10.1177_23247096231171251 [pii] AID - 10.1177/23247096231171251 [doi] PST - ppublish SO - J Investig Med High Impact Case Rep. 2023 Jan-Dec;11:23247096231171251. doi: 10.1177/23247096231171251. PMID- 41399084 OWN - NLM STAT- MEDLINE DCOM- 20251216 LR - 20251221 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 57 IP - 6 DP - 2025 Dec 18 TI - [POEMS syndrome misdiagnosed as systemic sclerosis: A case report]. PG - 1184-1187 AB - This article reports the diagnosis and treatment process of a 52-year-old female patient who was finally diagnosed with POEMS syndrome. Her main clinical manifestations included Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet. The patient was admitted to the Department of Rheumatology and Immunology, Peking University People's Hospital on April 8, 2024, due to "purplish red skin on the neck and chest for 1.5 years, swelling of both hands for 1 year, and numbness of both feet for 8 months". One and a half years ago, she was diagnosed with systemic sclerosis (SSc) in another hospital, based on evidence from a series of clinical manifestations. Her main symptoms were Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet, which met the 2013 classification criteria for SSc by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). During the disease course, the patient received regular treatment with glucocorticoids and disease-modifying antirheumatic drugs (DMARDs), but her condition still progressed continuously. Eight months ago, she developed new-onset numbness in both her feet accompanied by pinprick-like pain, indicating the complexity of the condition and the need for further investigation of the etiology. Differential diagnosis should consider possibilities, such as mixed connective tissue disease, eosinophilic fasciitis, and malignant tumors. Auxiliary examinations showed that the patient's serum antinuclear antibody, anti-topoisomerase Ⅰ(Scl-70) antibody, anti-U1 ribonucleoprotein (RNP) antibody, and antineutrophil cytoplasmic antibody were all negative. Imaging examinations revealed no pulmonary arterial hypertension or pulmonary interstitial fibrosis. In addition, the patient had multiple endocrine abnormalities and responded poorly to treatment with glucocorticoids and DMARDs, suggesting the need to be alert to the possibility of lymphoproliferative diseases. Further examinations including vascular endothelial growth factor detection, whole-body bone scan, and bone marrow aspiration and biopsy were performed, and the final diagnosis of POEMS syndrome was confirmed. The patient was then transferred to the Department of Hemato-logy and received treatment with the pomalidomide combined with dexamethasone regimen, and her clinical symptoms gradually relieved. This case suggests that POEMS syndrome is similar to rheumatological and immunological diseases such as SSc in terms of clinical manifestations. Clinicians should be more vigilant during diagnosis and treatment, and pay attention to differentiation, so as to reduce missed diagnoses and misdiagnoses, thereby formulating more accurate and effective treatment plans for patients. FAU - Liang, Jingyuan AU - Liang J AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. AD - Department of Rheumatology and Immunology, North China University of Science and Technology Affiliated Hospital, Tang-shan 063000, Hebei, China. FAU - Zhang, Xia AU - Zhang X AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Yao, Haihong AU - Yao H AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - Case Reports PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 SB - IM MH - Humans MH - Female MH - *POEMS Syndrome/diagnosis/drug therapy MH - *Scleroderma, Systemic/diagnosis MH - Middle Aged MH - *Diagnostic Errors MH - Diagnosis, Differential PMC - PMC12711426 OTO - NOTNLM OT - POEMS syndrome OT - Peripheral nervous system diseases OT - Skin pigmentation OT - Systemic sclerosis COIS- 利益冲突  所有作者均声明不存在利益冲突。 EDAT- 2025/12/16 06:28 MHDA- 2025/12/16 06:29 PMCR- 2025/12/18 CRDT- 2025/12/16 02:17 PHST- 2025/12/16 06:29 [medline] PHST- 2025/12/16 06:28 [pubmed] PHST- 2025/12/16 02:17 [entrez] PHST- 2025/12/18 00:00 [pmc-release] AID - bjdxxbyxb-57-6-1184 [pii] AID - 10.19723/j.issn.1671-167X.2025.06.025 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2025 Dec 18;57(6):1184-1187. doi: 10.19723/j.issn.1671-167X.2025.06.025. PMID- 40832809 OWN - NLM STAT- MEDLINE DCOM- 20251002 LR - 20251117 IS - 1531-6963 (Electronic) IS - 1040-8711 (Linking) VI - 37 IP - 6 DP - 2025 Nov 1 TI - Targeted therapies in systemic sclerosis: a narrative review of novel drugs in clinical trials. PG - 392-403 LID - 10.1097/BOR.0000000000001121 [doi] AB - PURPOSE OF REVIEW: Systemic sclerosis (SSc) remains a therapeutic challenge, with conventional immunosuppressive strategies showing inconsistent effects and no disease modifying activity. The lack of head-head trials comparing immunosuppressives with emerging antifibrotic agents further complicates treatment decisions in SSc. This review aims to provide an update on the recent advances in targeted therapies for SSc, with a focus on novel biologics and small molecules that specifically modulate key mechanisms. RECENT FINDINGS: Advances in molecular profiling have revealed inflammatory and fibrotic endotypes within SSc while imaging studies support a fibroinflammatory subset, highlighting potential therapeutic targets. SUMMARY: A literature search for clinical trials between January 2020 and April 2025 from PubMed/MEDLINE, clinicaltrials.gov, euclinicaltrials.eu databases for targeted therapies in systemic sclerosis revealed a total of 117 clinical trials, of which we described the design, methods and endpoints from 14 studies (2 conference abstracts, 11 trials and 1 case series). These study results offer hope for patients with systemic sclerosis and pave way for future studies directing the development of patient-specific guidelines. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Emokpae, Morgan AU - Emokpae M AD - Yale School of Medicine, Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, New Haven, Connecticut, USA. FAU - Cheung, Crystal AU - Cheung C FAU - Nadella, Manvitha AU - Nadella M LA - eng PT - Journal Article PT - Review DEP - 20250820 PL - United States TA - Curr Opin Rheumatol JT - Current opinion in rheumatology JID - 9000851 RN - 0 (Immunosuppressive Agents) RN - 0 (Biological Products) SB - IM MH - Humans MH - *Scleroderma, Systemic/drug therapy MH - *Molecular Targeted Therapy/methods MH - Clinical Trials as Topic MH - *Immunosuppressive Agents/therapeutic use MH - *Biological Products/therapeutic use OTO - NOTNLM OT - Raynaud phenomenon OT - lung fibrosis OT - scleroderma renal crisis OT - skin fibrosis OT - systemic sclerosis OT - targeted therapies OT - treatment EDAT- 2025/08/20 12:32 MHDA- 2025/10/02 12:33 CRDT- 2025/08/20 06:13 PHST- 2025/10/02 12:33 [medline] PHST- 2025/08/20 12:32 [pubmed] PHST- 2025/08/20 06:13 [entrez] AID - 00002281-202511000-00008 [pii] AID - 10.1097/BOR.0000000000001121 [doi] PST - ppublish SO - Curr Opin Rheumatol. 2025 Nov 1;37(6):392-403. doi: 10.1097/BOR.0000000000001121. Epub 2025 Aug 20. PMID- 34266986 OWN - NLM STAT- MEDLINE DCOM- 20211021 LR - 20230619 IS - 1499-2752 (Electronic) IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 48 IP - 10 DP - 2021 Oct TI - Impaired Myocardial Flow Reserve on (82)Rubidium Positron Emission Tomography/Computed Tomography in Patients With Systemic Sclerosis. PG - 1574-1582 LID - 10.3899/jrheum.210040 [doi] AB - OBJECTIVE: To investigate the association between Raynaud phenomenon (RP) and coronary microvascular dysfunction, we measured myocardial flow reserve (MFR) using positron emission tomography/computed tomography (PET/CT) in patients with primary and secondary RP and controls. METHODS: Patients with RP, patient controls, and healthy participants who underwent dynamic rest-stress 82-rubidium PET/CT were studied. Differences in heart rate-blood pressure product-corrected MFR and clinical predictors of reduced MFR (< 2.0) were determined. RESULTS: Forty-nine patients with RP (80% female; aged 65 ± 11 yrs; 11 with primary RP, 18 with systemic sclerosis [SSc], and 20 with other autoimmune rheumatic diseases [AIRDs] including 6 with systemic lupus erythematosus, 6 with rheumatoid arthritis, 4 with overlap syndrome, 2 with Sjögren syndrome, and 2 with inflammatory arthritis), 49 matched patients without RP or AIRD (78% female; 64 ± 13 yrs), and 14 healthy participants (50% female; 35 ± 5 yrs) were studied. Patients with primary RP, matched patient controls, and healthy participants had comparable MFR. Patients with SSc-RP had significantly reduced MFR (1.62 ± 0.32) compared to matched patient controls (P = 0.03, 2.06 ± 0.61) and to healthy participants (P = 0.01, 2.22 ± 0.44). In multivariable logistic regression, SSc was an independent predictor of reduced MFR. We identified a correlation between time since AIRD diagnosis and MFR (r = -0.30, 95% CI -0.63 to -0.02, P = 0.04). CONCLUSION: Our findings suggest that only secondary, not primary, RP is associated with reduced MFR, and that patients with SSc-RP have reduced MFR compared to those with primary RP and patients with other AIRDs. Larger prospective studies are warranted to fully elucidate the prognostic value of MFR in patients with secondary RP. CI - Copyright © 2021 by the Journal of Rheumatology. FAU - Feher, Attila AU - Feher A AD - A. Feher, MD, PhD, E.K. Oikonomou, MD, PhD, S. Thorn, PhD, Y.H. Liu, PhD, E.J. Miller, MD, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine. FAU - Boutagy, Nabil E AU - Boutagy NE AD - N.E. Boutagy, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, and Vascular Biology and Therapeutics Program, and Department of Pharmacology, Yale School of Medicine. FAU - Oikonomou, Evangelos K AU - Oikonomou EK AD - A. Feher, MD, PhD, E.K. Oikonomou, MD, PhD, S. Thorn, PhD, Y.H. Liu, PhD, E.J. Miller, MD, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine. FAU - Thorn, Stephanie AU - Thorn S AD - A. Feher, MD, PhD, E.K. Oikonomou, MD, PhD, S. Thorn, PhD, Y.H. Liu, PhD, E.J. Miller, MD, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine. FAU - Liu, Yi-Hwa AU - Liu YH AD - A. Feher, MD, PhD, E.K. Oikonomou, MD, PhD, S. Thorn, PhD, Y.H. Liu, PhD, E.J. Miller, MD, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine. FAU - Miller, Edward J AU - Miller EJ AD - A. Feher, MD, PhD, E.K. Oikonomou, MD, PhD, S. Thorn, PhD, Y.H. Liu, PhD, E.J. Miller, MD, PhD, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine. FAU - Sinusas, Albert J AU - Sinusas AJ AD - A.J. Sinusas, MD, BSc, Section of Cardiovascular Medicine, Department of Internal Medicine, and Department of Radiology and Biomedical Imaging, Yale School of Medicine, and Department of Biomedical Engineering, Yale University. FAU - Hinchcliff, Monique AU - Hinchcliff M AUID- ORCID: 0000-0002-8652-9890 AD - M. Hinchcliff, MD, Section of Rheumatology, Department of Internal Medicine, and Department of Internal Medicine, Clinical and Translational Research Accelerator, Yale School of Medicine, New Haven, Connecticut, USA. monique.hinchcliff@yale.edu. LA - eng GR - R01 AR073270/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20210715 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - MLT4718TJW (Rubidium) SB - IM MH - Female MH - Humans MH - Male MH - *Positron Emission Tomography Computed Tomography MH - Rubidium MH - *Scleroderma, Systemic/complications/diagnostic imaging PMC - PMC10275580 MID - NIHMS1722121 OTO - NOTNLM OT - Raynaud phenomenon OT - cardiovascular disease OT - radionuclide imaging OT - scleroderma OT - systemic sclerosis COIS- Conflict of Interest: MH has received consulting fees from AbbVie and from Boehringer Ingelheim unrelated to the current study. EJM reports grant funding from NIH, Bracco, Eidos, Pfizer, and Alnylam as well as consulting fees from Bracco, Eidos, Pfizer, and Alnylam, all unrelated to the current study. AJS reports grant funding from Siemens and Jubilant as well as consulting fees from MicroVide, LLC, all unrelated to the current study. EDAT- 2021/07/17 06:00 MHDA- 2023/02/25 06:00 PMCR- 2023/06/16 CRDT- 2021/07/16 05:52 PHST- 2021/07/05 00:00 [accepted] PHST- 2021/07/17 06:00 [pubmed] PHST- 2023/02/25 06:00 [medline] PHST- 2021/07/16 05:52 [entrez] PHST- 2023/06/16 00:00 [pmc-release] AID - jrheum.210040 [pii] AID - 10.3899/jrheum.210040 [doi] PST - ppublish SO - J Rheumatol. 2021 Oct;48(10):1574-1582. doi: 10.3899/jrheum.210040. Epub 2021 Jul 15. PMID- 18159855 OWN - NLM STAT- MEDLINE DCOM- 20080130 LR - 20071227 IS - 0033-2240 (Print) IS - 0033-2240 (Linking) VI - 64 IP - 6 DP - 2007 TI - [The clinical picture of morphea]. PG - 438-41 AB - Skin scleroderma (LS) is characterised by stiffness of skin and/or deeper tissues. As opposed to systemic scleroderma, the involvement of internal organs and Raynaud phenomenon are predominately not observed in morphea. LS is quite rare disease, more frequent in women and young people. There are several useful classifications of skin scleroderma in literature, however the classification which concerns the shape and extension of LS lesion and depth of stiffness is concerned to be the most clear. LS is divided into: plaque morphoea, generalised morphoea, blistering morphoea, linear morphoea and deep morphoea. Different types of skin scleroderma lesions can be observed in one patient or can combine linear and deep fibrosis. Presented classification is clinically useful and it has prognostic and therapeutic implications. FAU - Wojas-Pelc, Anna AU - Wojas-Pelc A AD - Katedra i Klinika Dermatologii, Collegium Medicum, Uniwersytetu Jagiellońskiego, Kraków. FAU - Wielowieyska-Szybińska, Dorota AU - Wielowieyska-Szybińska D LA - pol PT - English Abstract PT - Journal Article PT - Review TT - Obraz kliniczny twardziny skórnej. PL - Poland TA - Przegl Lek JT - Przeglad lekarski JID - 19840720R SB - IM MH - Adolescent MH - Child MH - Child, Preschool MH - Diagnosis, Differential MH - Female MH - Humans MH - PUVA Therapy MH - Scleroderma, Localized/*diagnosis/*etiology/physiopathology MH - Scleroderma, Systemic/diagnosis/etiology/physiopathology MH - Skin/metabolism/pathology/physiopathology RF - 58 EDAT- 2007/12/28 09:00 MHDA- 2008/01/31 09:00 CRDT- 2007/12/28 09:00 PHST- 2007/12/28 09:00 [pubmed] PHST- 2008/01/31 09:00 [medline] PHST- 2007/12/28 09:00 [entrez] PST - ppublish SO - Przegl Lek. 2007;64(6):438-41. PMID- 18664993 OWN - NLM STAT- MEDLINE DCOM- 20090130 LR - 20120110 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 14 IP - 5 DP - 2008 Oct TI - Malignancy in the setting of the anti-synthetase syndrome. PG - 285-8 LID - 10.1097/RHU.0b013e31817d116f [doi] AB - Malignancy and interstitial lung disease (ILD) are 2 conditions associated with dermatomyositis (DM) that are responsible for a significant portion of the morbidity and mortality related to this disease; however, they rarely occur in the same patient. The antisynthetase syndrome consists of several characteristics, including ILD, arthritis, Raynaud phenomenon, "mechanic's hands," and positive antibodies to tRNA synthetases, which have each been negatively associated with cancer. When patients with DM present with such characteristics, clinicians may be falsely reassured that a thorough malignancy screen is unnecessary. We describe a patient who presented with the antisynthetase syndrome and was subsequently found to have colon cancer. Removal of the cancer led to resolution of the myositis and lung disease, but the patient's rash and arthritis persisted and ultimately required immunosuppressive therapy. We provide a review of the literature describing the concurrence of both this syndrome and ILD alone, with malignancy. We conclude that a thorough and expedited age-appropriate malignancy work up is indicated in all patients with a new diagnosis of DM, despite the presence of disease characteristics that are usually not associated with cancer. FAU - Rozelle, Andrew AU - Rozelle A AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA. FAU - Trieu, Sandy AU - Trieu S FAU - Chung, Lorinda AU - Chung L LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) SB - IM CIN - J Clin Rheumatol. 2011 Dec;17(8):458. doi: 10.1097/RHU.0b013e31823b1878. PMID: 22126809 MH - Adenocarcinoma/*epidemiology MH - Antibodies, Antinuclear/*immunology MH - Arthritis/*epidemiology MH - Comorbidity MH - Dermatomyositis/*epidemiology MH - Humans MH - Lung Diseases, Interstitial/epidemiology/immunology/pathology MH - Male MH - Middle Aged MH - Polymyositis/*epidemiology MH - Sigmoid Neoplasms/*epidemiology MH - Syndrome EDAT- 2008/07/31 09:00 MHDA- 2009/01/31 09:00 CRDT- 2008/07/31 09:00 PHST- 2008/07/31 09:00 [pubmed] PHST- 2009/01/31 09:00 [medline] PHST- 2008/07/31 09:00 [entrez] AID - 10.1097/RHU.0b013e31817d116f [doi] PST - ppublish SO - J Clin Rheumatol. 2008 Oct;14(5):285-8. doi: 10.1097/RHU.0b013e31817d116f. PMID- 26204208 OWN - NLM STAT- MEDLINE DCOM- 20160413 LR - 20181202 IS - 1536-0229 (Electronic) IS - 0363-9762 (Linking) VI - 40 IP - 10 DP - 2015 Oct TI - IgG4-Related Tubulointerstitial Nephritis Pattern in 18F-FDG PET/CT. PG - 808-9 LID - 10.1097/RLU.0000000000000899 [doi] AB - A 17-year-old adolescent girl was admitted with chronic arthralgia, Raynaud phenomenon, pericarditis, and evidences of chronic diffuse inflammation. F-FDG PET/CT scan was performed to search systemic vasculitis and showed diffuse moderate uptake in the kidneys. We suggested the existence of a nephritis, but the ultrasonography result was normal, and no treatment was introduced. Another F-FDG PET/CT scan was performed 7 months later to explore abdominal pain. It showed again diffuse intense uptake in both kidneys. A proteinuria was highlighted, and renal biopsy allowed to diagnose IgG4-related disease. FAU - Bélissant, Ophélie Jr AU - Bélissant O Jr AD - From the *Service de Médecine Nucléaire, Centre Cardiologique du Nord, Saint Denis; †Service d'Anatomopathologie, Hôpital Pitié Salpétrière, Paris; and ‡Service de Médecine Interne, Hôpital Delafontaine, Saint Denis, France. FAU - Guernou, Mohamed AU - Guernou M FAU - Rouvier, Philippe AU - Rouvier P FAU - Compain, Caroline AU - Compain C FAU - Bonardel, Gérald AU - Bonardel G LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Clin Nucl Med JT - Clinical nuclear medicine JID - 7611109 RN - 0 (Immunoglobulin G) RN - 0 (Radiopharmaceuticals) RN - 0Z5B2CJX4D (Fluorodeoxyglucose F18) SB - IM MH - Adolescent MH - Female MH - *Fluorodeoxyglucose F18 MH - Humans MH - Immunoglobulin G/*immunology MH - *Multimodal Imaging MH - Nephritis, Interstitial/*diagnostic imaging/immunology MH - *Positron-Emission Tomography MH - Radiopharmaceuticals MH - *Tomography, X-Ray Computed EDAT- 2015/07/24 06:00 MHDA- 2016/04/14 06:00 CRDT- 2015/07/24 06:00 PHST- 2015/07/24 06:00 [entrez] PHST- 2015/07/24 06:00 [pubmed] PHST- 2016/04/14 06:00 [medline] AID - 10.1097/RLU.0000000000000899 [doi] PST - ppublish SO - Clin Nucl Med. 2015 Oct;40(10):808-9. doi: 10.1097/RLU.0000000000000899. PMID- 22408871 OWN - NLM STAT- MEDLINE DCOM- 20120501 LR - 20120313 IS - 0035-2640 (Print) IS - 0035-2640 (Linking) VI - 62 IP - 2 DP - 2012 Feb TI - [Evolutive forms of primary Sjögren's syndrome]. PG - 229-31 AB - Primary Sjogrën's syndrome (pSS) could be restricted in 50% of the cases to glandular involvement with a chronic sicca syndrome, with a considerable alteration of quality of life. It could be complicated by systemic involvements, which are responsible of the visceral severity. Thus systemic complications could appear many years after initial pSS diagnosis and justify long-term surveillance. Initial parotid gland enlargement, Raynaud phenomenon, cutaneous vasculitis and immunological abnormalities (anti-SSA and/or SSB positivity, hypergammaglobulinemia, and cryoglobulinemia) are also implicated in systemic complications. FAU - Fauchais, Anne-Laure AU - Fauchais AL AD - Service de médecine interne A, CHU de Limoges, hôpital Dupuytren, 87042 Limoges. anne-laure.fauchais@chu-limoges.fr FAU - Gondran, Guillaume AU - Gondran G FAU - Martel, Clothilde AU - Martel C FAU - Vidal, Elisabeth AU - Vidal E LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Formes ëvolutives du syndrome de Gougerot-Sjögren. PL - France TA - Rev Prat JT - La Revue du praticien JID - 0404334 SB - IM MH - Age Factors MH - Algorithms MH - Asthenia/complications/diagnosis MH - Disease Progression MH - Humans MH - Immunologic Tests MH - Models, Biological MH - Salivary Gland Diseases/diagnosis/etiology MH - Sjogren's Syndrome/*classification/complications/*diagnosis/pathology MH - Watchful Waiting EDAT- 2012/03/14 06:00 MHDA- 2012/05/02 06:00 CRDT- 2012/03/14 06:00 PHST- 2012/03/14 06:00 [entrez] PHST- 2012/03/14 06:00 [pubmed] PHST- 2012/05/02 06:00 [medline] PST - ppublish SO - Rev Prat. 2012 Feb;62(2):229-31. PMID- 30479855 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220330 IS - 2160-9381 (Print) IS - 2160-9381 (Electronic) IS - 2160-9381 (Linking) VI - 8 IP - 4 DP - 2018 Oct TI - New trends in botulinum toxin use in dermatology. PG - 277-282 LID - 10.5826/dpc.0804a05 [doi] AB - BACKGROUND: Recent studies have highlighted new botulinum neurotoxin (BoNT) applications in the field of dermatology. OBJECTIVE: To review current knowledge of BoNT use in dermatology. METHODS: The literature of the last 5 five years has been reviewed. RESULTS: We describe interesting protocols of BoNT treatment for hyperhidrosis (HH), hypertrophic scars and keloids, Raynaud phenomenon, facial flushing, oily skin, psoriasis, Hailey-Hailey disease, and cutaneous lesions like painful lesions and periorbital syringomas. CONCLUSIONS: Several skin conditions eligible for BoNT treatment have been described. After the wide application for HH treatment, scars as well as vascular and inflammatory skin disorders, oily skin and cutaneous lesions represent fields of application of BoNT. FAU - Guida, Stefania AU - Guida S AD - Dermatology Unit, Department of Surgical, Medical, Dental and Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Italy. FAU - Farnetani, Francesca AU - Farnetani F AD - Dermatology Unit, Department of Surgical, Medical, Dental and Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Italy. FAU - Nisticò, Steven P AU - Nisticò SP AD - Department of Health Sciences, University Magna Graecia, Catanzaro, Italy. FAU - Mariarosaria, Caterina Giorgio AU - Mariarosaria CG AD - Department of Dermatology, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Babino, Graziella AU - Babino G AD - Department of Dermatology, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Pellacani, Giovanni AU - Pellacani G AD - Dermatology Unit, Department of Surgical, Medical, Dental and Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Italy. FAU - Fulgione, Elisabetta AU - Fulgione E AD - Department of Dermatology, University of Campania Luigi Vanvitelli, Naples, Italy. LA - eng PT - Journal Article DEP - 20181031 PL - Austria TA - Dermatol Pract Concept JT - Dermatology practical & conceptual JID - 101585990 PMC - PMC6246063 OTO - NOTNLM OT - BoNT-A OT - botulinum toxin OT - dermatology OT - hyperhidrosis OT - scars COIS- Competing interests: The authors have no conflicts of interest to disclose. EDAT- 2018/11/28 06:00 MHDA- 2018/11/28 06:01 PMCR- 2018/10/31 CRDT- 2018/11/28 06:00 PHST- 2018/03/10 00:00 [received] PHST- 2018/05/28 00:00 [accepted] PHST- 2018/11/28 06:00 [entrez] PHST- 2018/11/28 06:00 [pubmed] PHST- 2018/11/28 06:01 [medline] PHST- 2018/10/31 00:00 [pmc-release] AID - dp0804a05 [pii] AID - 10.5826/dpc.0804a05 [doi] PST - epublish SO - Dermatol Pract Concept. 2018 Oct 31;8(4):277-282. doi: 10.5826/dpc.0804a05. eCollection 2018 Oct. PMID- 26621866 OWN - NLM STAT- MEDLINE DCOM- 20160912 LR - 20181113 IS - 1757-790X (Electronic) IS - 1757-790X (Linking) VI - 2015 DP - 2015 Nov 27 TI - Systemic lupus erythaematosus presenting as spontaneous splenic rupture. LID - 10.1136/bcr-2015-212531 [doi] LID - bcr2015212531 AB - Systemic lupus erythaematosus (SLE) is known to involve the reticuloendothelial system, but spontaneous splenic rupture (SSR) in the context of the disease is a very rare complication. We observed a 61-year-old woman with an unremarkable previous medical history who presented with SSR and underwent an emergency splenectomy. The histopathological analysis of the specimen revealed signs of vasculitis. On review of symptoms with the patient, a history of oligoarthralgia, photosensitivity, xerostomia and Raynaud phenomenon was elicited. Laboratory investigations revealed lymphopaenia, mild proteinuria and positive antinuclear and anti-dsDNA antibodies. The patient was started on hydroxychloroquine and the disease has since remained silent. This article addresses the rare association between SLE and SSR. CI - 2015 BMJ Publishing Group Ltd. FAU - Cruz, António José AU - Cruz AJ AD - Serviço Medicina Interna, Centro Hospitalar de Entre o Douro e Vouga-Hospital de São Sebastião, Santa Maria da Feira, Portugal. FAU - Castro, Alexandra AU - Castro A AD - Serviço Medicina Interna, Centro Hospitalar de Entre o Douro e Vouga-Hospital de São Sebastião, Santa Maria da Feira, Portugal. LA - eng PT - Case Reports PT - Journal Article DEP - 20151127 PL - England TA - BMJ Case Rep JT - BMJ case reports JID - 101526291 RN - 4QWG6N8QKH (Hydroxychloroquine) SB - IM MH - Diagnosis, Differential MH - Female MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy MH - Middle Aged MH - Rupture, Spontaneous MH - Splenectomy MH - Splenic Rupture/*etiology/surgery PMC - PMC4680268 EDAT- 2015/12/02 06:00 MHDA- 2016/09/13 06:00 PMCR- 2017/11/27 CRDT- 2015/12/02 06:00 PHST- 2015/12/02 06:00 [entrez] PHST- 2015/12/02 06:00 [pubmed] PHST- 2016/09/13 06:00 [medline] PHST- 2017/11/27 00:00 [pmc-release] AID - bcr-2015-212531 [pii] AID - 10.1136/bcr-2015-212531 [doi] PST - epublish SO - BMJ Case Rep. 2015 Nov 27;2015:bcr2015212531. doi: 10.1136/bcr-2015-212531. PMID- 38333298 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240210 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 86 IP - 2 DP - 2024 Feb TI - Epidermodysplasia verruciformis arising in a female with systemic lupus erythematosus: a rare case from Syria. PG - 1101-1105 LID - 10.1097/MS9.0000000000001602 [doi] AB - INTRODUCTION AND IMPORTANCE: Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis. Clinical manifestations might be helpful in the diagnosis of this disease. However, the final diagnosis is made after a genetic and histological study. Acquired epidermodysplasia verruciformis is a form of epidermodysplasia verruciformis described in patients with compromised cell-mediated immunity. CASE PRESENTATION: A 42-year-old female with a history of a pain and itch on the soles and palms started a year ago. There were multiple flat papules on the dorsal hands, scarring alopecia, malar rash, oral ulcers, Raynaud phenomenon, and palpable purpura. A histological examination confirmed the diagnosis of epidermodysplasia verruciformis. CLINICAL DISCUSSION: Epidermodysplasia verruciformis is an uncommon disease that affects the immune system. The coexistence of systemic lupus erythematosus and epidermodysplasia verruciformis is rarely reported in the medical literature. This paper reports a rare case in which these two diseases have coexisted. CONCLUSION: This publication aims to document this rare case and highlight the ideal criteria in diagnosing and treating epidermodysplasia verruciformis. CI - Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. FAU - Mohammad Deeb, Ahmad AU - Mohammad Deeb A AD - Faculty of Medicine, Tishreen University. FAU - Mohammad Deeb, Eman AU - Mohammad Deeb E AD - Faculty of Medicine, Tishreen University. FAU - Al-Soufi, Lina AU - Al-Soufi L AD - Department of Dermatology, National Hospital of Latakia, Latakia, Syria. LA - eng PT - Case Reports PT - Journal Article DEP - 20240103 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC10849436 OTO - NOTNLM OT - antinuclear antibody OT - case report OT - epidermodysplasia verruciformis OT - human papillomavirus OT - systemic lupus erythematosus COIS- The authors declare that they have no conflicts of interest.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article. EDAT- 2024/02/09 06:42 MHDA- 2024/02/09 06:43 PMCR- 2024/01/03 CRDT- 2024/02/09 03:57 PHST- 2024/09/15 00:00 [received] PHST- 2024/11/29 00:00 [accepted] PHST- 2024/02/09 06:43 [medline] PHST- 2024/02/09 06:42 [pubmed] PHST- 2024/02/09 03:57 [entrez] PHST- 2024/01/03 00:00 [pmc-release] AID - AMSU-D-23-02045 [pii] AID - 10.1097/MS9.0000000000001602 [doi] PST - epublish SO - Ann Med Surg (Lond). 2024 Jan 3;86(2):1101-1105. doi: 10.1097/MS9.0000000000001602. eCollection 2024 Feb. PMID- 27433180 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20160719 LR - 20200930 IS - 1738-3536 (Print) IS - 2005-6184 (Electronic) IS - 1738-3536 (Linking) VI - 79 IP - 3 DP - 2016 Jul TI - Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome. PG - 188-92 LID - 10.4046/trd.2016.79.3.188 [doi] AB - Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids. FAU - Kim, Seo-Hyun AU - Kim SH AD - Department of Internal Medicine, Inje University Seoul Paik Hospital, Seoul, Korea. FAU - Park, I-Nae AU - Park IN AD - Department of Internal Medicine, Inje University Seoul Paik Hospital, Seoul, Korea. LA - eng PT - Journal Article DEP - 20160701 PL - Korea (South) TA - Tuberc Respir Dis (Seoul) JT - Tuberculosis and respiratory diseases JID - 101479418 PMC - PMC4943904 OTO - NOTNLM OT - Antisynthetase Syndrome OT - Immunoglobulins OT - Respiratory Distress Syndrome, Adult COIS- Conflicts of Interest: No potential conflict of interest relevant to this article was reported. EDAT- 2016/07/20 06:00 MHDA- 2016/07/20 06:01 PMCR- 2016/07/01 CRDT- 2016/07/20 06:00 PHST- 2015/06/01 00:00 [received] PHST- 2015/06/24 00:00 [revised] PHST- 2015/09/19 00:00 [accepted] PHST- 2016/07/20 06:00 [entrez] PHST- 2016/07/20 06:00 [pubmed] PHST- 2016/07/20 06:01 [medline] PHST- 2016/07/01 00:00 [pmc-release] AID - 10.4046/trd.2016.79.3.188 [doi] PST - ppublish SO - Tuberc Respir Dis (Seoul). 2016 Jul;79(3):188-92. doi: 10.4046/trd.2016.79.3.188. Epub 2016 Jul 1. PMID- 23107322 OWN - NLM STAT- MEDLINE DCOM- 20130904 LR - 20121030 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 10 IP - 11 DP - 2012 Nov TI - Systemic sclerosis - focus on dermatological aspects. Part 2: diagnostics, therapy. PG - 783-91 LID - 10.1111/j.1610-0387.2012.07998.x [doi] AB - Systemic sclerosis is a chronic inflammatory multiorgan disease which may involve the skin and internal organs to a varying extent. Pathogenetically the vasculature, connective tissue and the immune system are involved in a yet to be defined sequence and impact. Case history and results of physical as well as laboratory examinations will determine individually adapted further organ imaging or invasive procedures. Based on their results therapy is initiated which may include supportive measures such as physiotherapy as well as basic skin care and avoidance of any trauma. Many agents are available for the circulatory problems including Raynaud phenomenon and digital ulcers such as calcium channel blockers, ACE inhibitors and intravenous prostacyclin derivatives, as well as endothelin receptor blockers and phosphodiesterase inhibitors. Immunosuppressive and immunomodulatory agents are of varying efficacy depending on organ involvement. Though various therapeutic measures are available, beneficial effects are limited and associated with various unwanted effects. In any case, the therapy has to be individually adapted to the disease stage and course of the disease. CI - © The Author • Journal compilation © Blackwell Verlag GmbH, Berlin. FAU - Sticherling, Michael AU - Sticherling M AD - Department of Dermatology, University of Erlangen, Germany. michael.sticherling@uk-erlangen.de LA - eng LA - ger PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Vasodilator Agents) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Dermoscopy/*trends MH - Humans MH - Immunoassay/trends MH - Scleroderma, Systemic/*diagnosis/*therapy MH - Stem Cell Transplantation/*trends MH - Ultrasonography/*trends MH - Vasodilator Agents/*therapeutic use EDAT- 2012/10/31 06:00 MHDA- 2013/09/05 06:00 CRDT- 2012/10/31 06:00 PHST- 2012/10/31 06:00 [entrez] PHST- 2012/10/31 06:00 [pubmed] PHST- 2013/09/05 06:00 [medline] AID - 10.1111/j.1610-0387.2012.07998.x [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2012 Nov;10(11):783-91. doi: 10.1111/j.1610-0387.2012.07998.x. PMID- 19429438 OWN - NLM STAT- MEDLINE DCOM- 20090601 LR - 20131121 IS - 1879-1220 (Electronic) IS - 0960-0760 (Linking) VI - 114 IP - 1-2 DP - 2009 Mar TI - Testosterone deficiency syndrome: treatment and cancer risk. PG - 96-105 LID - 10.1016/j.jsbmb.2009.01.014 [doi] AB - Testosterone deficiency syndrome (TDS) can be linked to premature mortality and to a number of co-morbidities (such as sexual disorders, diabetes, metabolic syndrome, ...). Testosterone deficiency occurs mainly in ageing men, at a time when prostate disease (benign or malign) start to emerge. New testosterone preparations via different route of administration appeared during the last decade allowing optimized treatment to these patients. One potential complication of this treatment is the increased risk of prostate and breast cancer. Consequently, the guidelines from the agencies and the institutions, the recommendations of the scientific expert committees and the attitude of the clinicians to who, when and how to treat hypogonadal patients, is very conservative, not to say, highly restrictive. To date, as documented in many reviews on the subject, nothing has been found to support the evidence that restoring testosterone levels within normal range increases the incidence of prostate cancer. In our experience, during a long-term clinical study including 200 hypogonadal patients receiving a patch of testosterone, 50 patients ended 5 years of treatment and no prostate cancer have been reported. In fact, the incidence of prostate cancer in primary or secondary testosterone treated hypogonadal men is lower than the incidence observed in the untreated eugonadal population. However, even if the number of patients treated in well-conducted clinical trials for whom cancer of the prostate has been reported is insignificant (a very few), the observed population is still too small to raise definite conclusions. Low testosterone levels have been reported in patients undergoing radical prostatectomy and the outcomes are of worse diagnostic in this population; at a later stage, testosterone deficiency can be induced by anti hormonal manipulation of patient with a prostate cancer, leading to the symptoms of hypogonadism. The question is to know whether it is justified, in case of profound symptoms, to supplement those patients with testosterone. Some attempts have been made and the results are encouraging: so it is time to re-examine our position and to question about the definite recommendation that patients with prostate cancer should never receive testosterone supplementation therapy; this is already the situation when intermittent androgen blockade is initiated if the biological response is satisfactory. Furthermore, it has been advocated that, under a rigorous surveillance, patients cured of prostate cancer can be treated with testosterone supplementation with beneficial results. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Université Pierre & Marie Curie, 4 Place Jussieu, Paris, France. jeanpierre@raynaud.ws LA - eng PT - Journal Article PT - Review DEP - 20090130 PL - England TA - J Steroid Biochem Mol Biol JT - The Journal of steroid biochemistry and molecular biology JID - 9015483 RN - 0 (Androgens) RN - 3XMK78S47O (Testosterone) SB - IM MH - Aging/physiology MH - Androgens/metabolism/therapeutic use MH - Clinical Trials as Topic MH - Humans MH - Hypogonadism/*drug therapy MH - Male MH - Meta-Analysis as Topic MH - Prostate/pathology MH - Prostatic Neoplasms/*chemically induced/drug therapy/pathology MH - Risk Factors MH - *Testosterone/adverse effects/blood/therapeutic use RF - 90 EDAT- 2009/05/12 09:00 MHDA- 2009/06/02 09:00 CRDT- 2009/05/12 09:00 PHST- 2009/01/11 00:00 [received] PHST- 2009/01/21 00:00 [accepted] PHST- 2009/05/12 09:00 [entrez] PHST- 2009/05/12 09:00 [pubmed] PHST- 2009/06/02 09:00 [medline] AID - S0960-0760(09)00046-6 [pii] AID - 10.1016/j.jsbmb.2009.01.014 [doi] PST - ppublish SO - J Steroid Biochem Mol Biol. 2009 Mar;114(1-2):96-105. doi: 10.1016/j.jsbmb.2009.01.014. Epub 2009 Jan 30. PMID- 16898534 OWN - NLM STAT- MEDLINE DCOM- 20061213 LR - 20131121 IS - 0033-2240 (Print) IS - 0033-2240 (Linking) VI - 63 Suppl 3 DP - 2006 TI - [Assessment of the peripheral circulation in children with nephrotic syndrome treated with cyclosporin A]. PG - 214-6 AB - Cyclosporin A (CsA) is an immunosuppressive agent used in children for the treatment of steroid-dependent idiopathic nephrotic syndrome (INS). Despite its benefitial effect on a course of the disease CsA may exert nephrotoxic effects because of its vasoconstrictive properties. CsA-dependent disorders of the peripheral flow (Raynaud phenomenon--RP) have been recently described. The aim of the study was to assess the effect of CsA on the peripheral circulation. The study group comprised 16 children (12 male, 4 female; mean age 9.8 +/- 4.5 years) treated with CsA for at least 6 months due to INS (mean treatment time 39 +/- 27 months). Thirteen age- and sex-matched individuals served as controls. Peripheral circulation disorders were evaluated by means of a cold stress test (both hands were held in lukewarm water (20 degrees C) for 1 minute and thereafter the changes in the hand temperature were recorded with thermographic camera (Inframetrics SC1000). RP assessment was performed according to the method described by Ammer and Ring. The temperature gradient of 4 degrees C or greater maintained between metacarpal and peripheral areas of a hand after 10 minutes was considered diagnostic for RP. According to these criteria RP was confirmed in only 3 patients from the study group and in 2 controls. However, the time of the temperature increase in the first 5 minutes after cooling was considerably shorter in the children with INS (0.26 +/- 0.26 degrees C/min vs 0.51 +/- 0.29 degrees C/min, p=0.02). No correlation between CsA serum concentration, CsA dose and impairment of the hand temperature increase was found. The study confirmed that in children suffering from INS treated with CsA peripheral blood flow disorders can be seen. It seems that impaired vessel reactivity may result from the vasoconstrictive effect of CsA. FAU - Czupryniak, Aneta AU - Czupryniak A AD - Klinika Nefrologii i Dializoterapii, Instytut Centrum Zdrowia Matki Polki. aczupryniak@wp.pl FAU - Kałuzyńska, Anna AU - Kałuzyńska A FAU - Tkaczyk, Marcin AU - Tkaczyk M FAU - Półtorak-Krawczyk, Anna AU - Półtorak-Krawczyk A FAU - Ostrowski, Bartosz AU - Ostrowski B FAU - Wiecek, Bogusław AU - Wiecek B FAU - Nowicki, Michał AU - Nowicki M LA - pol PT - Controlled Clinical Trial PT - English Abstract PT - Journal Article TT - Ocena zaburzeń krazenia obwodowego u dzieci z idiopatycznym zespołem nerczycowym leczonych cyklosporyna A. PL - Poland TA - Przegl Lek JT - Przeglad lekarski JID - 19840720R RN - 0 (Immunosuppressive Agents) RN - 83HN0GTJ6D (Cyclosporine) SB - IM MH - Adolescent MH - Blood Circulation/*drug effects MH - Child MH - Child, Preschool MH - Cyclosporine/*adverse effects MH - Female MH - Fingers/blood supply/pathology MH - Humans MH - Immunosuppressive Agents/*adverse effects MH - Male MH - Microcirculation/drug effects MH - Nephrotic Syndrome/*drug therapy/*physiopathology MH - Regional Blood Flow/drug effects MH - Risk Assessment MH - Skin Temperature MH - Thermography MH - Vasoconstriction EDAT- 2006/08/11 09:00 MHDA- 2006/12/14 09:00 CRDT- 2006/08/11 09:00 PHST- 2006/08/11 09:00 [pubmed] PHST- 2006/12/14 09:00 [medline] PHST- 2006/08/11 09:00 [entrez] PST - ppublish SO - Przegl Lek. 2006;63 Suppl 3:214-6. PMID- 41400991 OWN - NLM STAT- MEDLINE DCOM- 20260129 LR - 20260414 IS - 1536-481X (Electronic) IS - 1057-0829 (Linking) VI - 35 IP - 2 DP - 2026 Feb 1 TI - Glaucoma Epidemiology in Colombia: Analysis of the Official Ministry of Health Registry. PG - 85-91 LID - 10.1097/IJG.0000000000002669 [doi] AB - PRÉCIS: This registry-based study estimates the prevalence of glaucoma in Colombia to be 1.94%, with higher rates in women. Significant associations with comorbidities like retinal vascular occlusion, myopia, Raynaud syndrome, and obstructive sleep apnea were found. PURPOSE: To estimate the prevalence of glaucoma in Colombia using data from the Individual Records of Health Services Provision (RIPS) between 2018 and 2022 and determine the prevalence ratios with diagnoses that have been linked to the development and progression of glaucoma. MATERIALS AND METHODS: This observational, retrospective study analyzed secondary data from the RIPS database. The study included individuals diagnosed with glaucoma, categorized by ICD-10 codes, and associated comorbidities. The prevalence was calculated for individuals over 40 years old, and prevalence ratios were determined for comorbid conditions. RESULTS: Between 2018 and 2022, 399,923 patients were diagnosed with glaucoma, with a prevalence of 1.94% in individuals over 40 years old. Primary open angle glaucoma was the most common type, with a prevalence of 1.08%. The study found strong associations between glaucoma and comorbidities such as retinal vascular occlusion (prevalence ratio of 9.1 for glaucoma and 8.8 for primary open angle glaucoma), myopia (3.3 and 3.7), Raynaud syndrome (2.4 and 2.5) and obstructive sleep apnea (2.1 and 2.5). A positive but less significant association with migraine (2.0 and 1.9), hypothyroidism (2.1 and 2.2), asthma (1.7 and 1.7), diabetes (1.5 and 1.7), hypertension (1.4 and 1.4), and chronic obstructive pulmonary disease (1.2 and 1.3) was also found. CONCLUSION: The prevalence of glaucoma in Colombia is consistent with global estimates, but with higher rates in women. The association between glaucoma and several comorbidities underscores the importance of an early detection and comprehensive management of these diseases. CI - Copyright © 2025 Wolters Kluwer Health, Inc. All rights reserved. FAU - Mosos, Juan D AU - Mosos JD AUID- ORCID: 0000-0003-3181-179 AD - Escuela Superior de Oftalmología -Instituto Barraquer de América, Colombia. AD - Faculty of Medicine, Pontificia Universidad Javeriana, Colombia. FAU - Barreto, Juan N AU - Barreto JN AD - Faculty of Medicine, Pontificia Universidad Javeriana, Colombia. FAU - Rosero-Silva, Antonia AU - Rosero-Silva A AD - Faculty of Medicine, Pontificia Universidad Javeriana, Colombia. FAU - De Vivero, Clemencia AU - De Vivero C AD - Escuela Superior de Oftalmología -Instituto Barraquer de América, Colombia. AD - Faculty of Medicine, Pontificia Universidad Javeriana, Colombia. FAU - Rosselli, Diego AU - Rosselli D AD - Department of Clinical Epidemiology and Biostatistics, Pontificia Universidad Javeriana, Colombia. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20251201 PL - United States TA - J Glaucoma JT - Journal of glaucoma JID - 9300903 SB - IM MH - Humans MH - Colombia/epidemiology MH - Female MH - Retrospective Studies MH - Male MH - Middle Aged MH - *Registries MH - Prevalence MH - Adult MH - Aged MH - *Glaucoma/epidemiology MH - Aged, 80 and over MH - Sex Distribution MH - Comorbidity MH - *Intraocular Pressure/physiology OTO - NOTNLM OT - Colombia OT - comorbidity OT - epidemiology OT - glaucoma OT - prevalence COIS- Disclosure: The authors declare no conflict of interest. EDAT- 2025/12/16 13:13 MHDA- 2026/01/29 19:11 CRDT- 2025/12/16 11:43 PHST- 2024/12/10 00:00 [received] PHST- 2025/11/23 00:00 [accepted] PHST- 2026/01/29 19:11 [medline] PHST- 2025/12/16 13:13 [pubmed] PHST- 2025/12/16 11:43 [entrez] AID - 00061198-990000000-00627 [pii] AID - 10.1097/IJG.0000000000002669 [doi] PST - ppublish SO - J Glaucoma. 2026 Feb 1;35(2):85-91. doi: 10.1097/IJG.0000000000002669. Epub 2025 Dec 1. PMID- 29173190 OWN - NLM STAT- MEDLINE DCOM- 20180918 LR - 20180918 IS - 2212-3873 (Electronic) IS - 1871-5303 (Linking) VI - 18 IP - 2 DP - 2018 Feb 13 TI - Migraine in Systemic Autoimmune Diseases. PG - 124-134 LID - 10.2174/1871530317666171124124340 [doi] AB - BACKGROUND AND OBJECTIVE: Migraine and systemic autoimmune diseases are 2-3-fold more common in women and various studies have reported an association between the two pathologies. METHODS: This review takes into account epidemiological studies involving migraine and systemic lupus erythematosus, antiphospholipid syndrome, Sjogren's syndrome, and other diffuse connective tissue diseases. This scientific literature analysis consists of the main articles found in Medline with a search up to April 2017. RESULTS: Many epidemiological studies were carried out on patients suffering from systemic lupus erythematosus. Results showed that headache and migraine are more prevalent in systemic lupus erythematosus patients compared to controls, especially migraine with aura. Patients with Lupus and migraine show a higher lupus activity and association with Raynaud and/or antiphospholipids in these populations are contradictory. There are not enough data to establish an association between antiphospholipid syndrome and migraine. However, data are more consistent between antiphospholipid carrier condition and migraine. Systemic sclerosis is a rare disease, for this reason the amount of available data on this disorder are scanty. However, some studies reported an association between headache, migraine and systemic sclerosis, especially where gliotic brain lesions and Raynaud are coexisting. Finally, large propensity cohort population based studies suggested that systemic autoimmune diseases are more frequent in patients suffering from migraine. CONCLUSION: An attempt at explaining the possible link between these disorders and migraine is discussed at the end of the review. Several autoimmune alterations are shared by most autoimmune diseases and headache types. Endothelial dysfunction is the only alteration that is common among all these disorders. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org. FAU - Cavestro, Cinzia AU - Cavestro C AD - Headache Centre, Department of Neurology, "S.Lazzaro", ASL CN2, Alba, Italy. FAU - Ferrero, Marcella AU - Ferrero M AD - Forensic and Conservation Genetics, University of Central Lancashire, Preston, United Kingdom. LA - eng PT - Journal Article PT - Review PL - United Arab Emirates TA - Endocr Metab Immune Disord Drug Targets JT - Endocrine, metabolic & immune disorders drug targets JID - 101269157 SB - IM MH - Animals MH - Antiphospholipid Syndrome/immunology/physiopathology MH - Autoimmune Diseases/immunology/*physiopathology MH - Connective Tissue Diseases/immunology/physiopathology MH - Endothelium, Vascular/immunology/*physiopathology MH - Humans MH - Migraine Disorders/diagnosis/*etiology/immunology/physiopathology MH - Migraine with Aura/diagnosis/etiology/immunology/physiopathology MH - Practice Guidelines as Topic MH - Severity of Illness Index MH - Sjogren's Syndrome/immunology/physiopathology OTO - NOTNLM OT - Migraine OT - Sjogren's syndrome OT - antiphospholipid syndrome OT - epidemiology OT - prevalence. OT - rheumatoid arthritis OT - systemic lupus erythematosus OT - systemic sclerosis EDAT- 2017/11/28 06:00 MHDA- 2018/09/19 06:00 CRDT- 2017/11/28 06:00 PHST- 2017/05/01 00:00 [received] PHST- 2017/09/17 00:00 [revised] PHST- 2017/10/23 00:00 [accepted] PHST- 2017/11/28 06:00 [pubmed] PHST- 2018/09/19 06:00 [medline] PHST- 2017/11/28 06:00 [entrez] AID - EMIDDT-EPUB-87094 [pii] AID - 10.2174/1871530317666171124124340 [doi] PST - ppublish SO - Endocr Metab Immune Disord Drug Targets. 2018 Feb 13;18(2):124-134. doi: 10.2174/1871530317666171124124340. PMID- 22057071 OWN - NLM STAT- MEDLINE DCOM- 20120313 LR - 20111107 IS - 1735-8604 (Electronic) IS - 1735-8582 (Linking) VI - 5 IP - 6 DP - 2011 Nov TI - Association of tumor necrosis factor-alpha gene polymorphisms with juvenile systemic lupus erythematosus nephritis in a cohort of egyptian patients. PG - 392-7 AB - INTRODUCTION: The production of tumor necrosis factor (TNF)-alpha has been deeply deregulated in systemic lupus erythematosus. We evaluated the association of -863C>A and -1031T>C polymorphisms of the TNF gene with susceptibility to and clinical manifestations of juvenile systemic lupus erythematosus. MATERIALS AND METHODS: This study was performed on 70 juvenile patients with systemic lupus erythematosus (mean age, 13.0 ± 4.2 years). Ninety age- and sex-matched children served as a controls. All participants were genotyped for the TNF -863C>A and -1031T>C polymorphisms by polymerase chain reaction-restriction fragment length polymorphism method. Analysis of serum TNF-alpha was done by solid-phase sandwich enzyme immunoassay. RESULTS: The mean serum TNF-alpha was significantly higher in the SLE patients compared to controls (P < .001). Regarding all participants, the mean serum TNF-alpha was significantly higher in children with -863AA genotype compared to carriers of -863C allele (P < .001). The TNF -863AA genotype frequencies were significantly higher in the patients group compared with the controls (P = .005) and were associated with increased risk for SLE development (odds ratio, 4.05; 95% confidence interval, 1.38 to 13.13; P = .005). The -863AA variant was associated with nephritis (P < .001) and Raynaud phenomenon (P = .001). CONCLUSIONS: The -863A allele of the TNF gene can be used as a genetic marker for SLE susceptibility and was associated with high TNF-alpha production, Raynaud phenomenon, and nephritis in juvenile SLE Egyptian patients. FAU - Farid, Tarek M AU - Farid TM AD - Department of Pediatrics, National Research Center, Cairo, Egypt. tarekelshaer67@yahoo.com FAU - Abd El Baky, Abeer Mohamed Nour Eldin AU - Abd El Baky AM FAU - Khalefa, Eman S AU - Khalefa ES FAU - Talaat, Ahmed A AU - Talaat AA FAU - Mohamed, Amal A AU - Mohamed AA FAU - Gheita, Tamer A AU - Gheita TA FAU - Abdel-Salam, Randa F AU - Abdel-Salam RF LA - eng PT - Comparative Study PT - Journal Article PT - Multicenter Study PL - Iran TA - Iran J Kidney Dis JT - Iranian journal of kidney diseases JID - 101316967 RN - 0 (Tumor Necrosis Factor-alpha) RN - 9007-49-2 (DNA) SB - IM MH - Adolescent MH - Alleles MH - DNA/*genetics MH - Egypt/epidemiology MH - Female MH - Gene Frequency MH - Genetic Predisposition to Disease MH - Genotype MH - Humans MH - Incidence MH - Lupus Nephritis/blood/epidemiology/*genetics MH - Male MH - Polymerase Chain Reaction MH - *Polymorphism, Genetic MH - Polymorphism, Restriction Fragment Length MH - Tumor Necrosis Factor-alpha/blood/*genetics EDAT- 2011/11/08 06:00 MHDA- 2012/03/14 06:00 CRDT- 2011/11/08 06:00 PHST- 2011/01/15 00:00 [received] PHST- 2011/06/27 00:00 [accepted] PHST- 2011/11/08 06:00 [entrez] PHST- 2011/11/08 06:00 [pubmed] PHST- 2012/03/14 06:00 [medline] AID - 489/341 [pii] PST - ppublish SO - Iran J Kidney Dis. 2011 Nov;5(6):392-7. PMID- 31485576 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2542-4548 (Electronic) IS - 2542-4548 (Linking) VI - 3 IP - 3 DP - 2019 Sep TI - Twelve-Year Survival in a Patient With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension on Nifedipine Monotherapy. PG - 376-379 LID - 10.1016/j.mayocpiqo.2019.06.002 [doi] AB - Pulmonary arterial hypertension is a progressive vascular disease with a high mortality rate without proper therapy. Identification of the appropriate treatment for each patient is critical in regard to adverse effects, health care costs, ease of treatment, and the potential for prognostication. Treatment strategies typically begin with acute vasoreactivity testing, which is performed during a right heart catherization. If positive, a calcium channel blocker may work; however, another pulmonary arterial hypertension-specific medication is necessary when testing is negative. Acute vasoreactivity testing is currently recommended to be performed only in certain subgroups of pulmonary arterial hypertension, but not when related to connective tissue disease. In this report, we describe a patient who had systemic sclerosis-related pulmonary arterial hypertension with a positive acute vasoreactivity test result. The patient was placed on calcium channel blocker monotherapy that has been well tolerated for 12 years, resulting in improved symptoms and exercise capacity. The long-term response to calcium channel blocker therapy in systemic sclerosis-associated pulmonary arterial hypertension has not been previously described. In addition, pulmonary artery pressures have been well controlled. The absence of genetic smooth muscle variants prevalent in vasoresponsive idiopathic pulmonary arterial hypertension is also unique. FAU - Helgeson, Scott A AU - Helgeson SA AD - Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL. FAU - Enderby, Cher Y AU - Enderby CY AD - Department of Pharmacy, Mayo Clinic, Jacksonville, FL. FAU - Moss, John E AU - Moss JE AD - Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL. FAU - Gass, Jennifer M AU - Gass JM AD - Department of Laboratory Genetics and Genomics, Greenwood Genetic Center, SC. FAU - Zeiger, Tonya K AU - Zeiger TK AD - Department of Pulmonary Medicine, Mayo Clinic, Jacksonville, FL. FAU - Burger, Charles D AU - Burger CD AD - Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL. LA - eng PT - Case Reports PT - Journal Article DEP - 20190719 PL - Netherlands TA - Mayo Clin Proc Innov Qual Outcomes JT - Mayo Clinic proceedings. Innovations, quality & outcomes JID - 101728275 PMC - PMC6713838 OTO - NOTNLM OT - AVT, acute vasoreactivity testing OT - CCB, calcium channel blocker OT - CREST, calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia OT - CTD, connective tissue disease OT - PAH, pulmonary arterial hypertension OT - PAOP, pulmonary arterial occlusion pressure OT - PVR, pulmonary vascular resistance OT - RHC, right heart catherization OT - SSc, systemic sclerosis OT - TTE, transthoracic echocardiogram OT - mPAP, mean pulmonary arterial pressure EDAT- 2019/09/06 06:00 MHDA- 2019/09/06 06:01 PMCR- 2019/07/19 CRDT- 2019/09/06 06:00 PHST- 2019/03/28 00:00 [received] PHST- 2019/05/28 00:00 [revised] PHST- 2019/06/10 00:00 [accepted] PHST- 2019/09/06 06:00 [entrez] PHST- 2019/09/06 06:00 [pubmed] PHST- 2019/09/06 06:01 [medline] PHST- 2019/07/19 00:00 [pmc-release] AID - S2542-4548(19)30075-X [pii] AID - 10.1016/j.mayocpiqo.2019.06.002 [doi] PST - epublish SO - Mayo Clin Proc Innov Qual Outcomes. 2019 Jul 19;3(3):376-379. doi: 10.1016/j.mayocpiqo.2019.06.002. eCollection 2019 Sep. PMID- 21786514 OWN - NLM STAT- MEDLINE DCOM- 20110830 LR - 20141225 VI - 15 IP - 1 DP - 2011 Jan-Mar TI - [Diagnostic problems in a 17-year-old patient with gastrointestinal manifestations of Fabry disease]. PG - 69-72 AB - Fabry disease is a rare X-linked recessive lysosomal storage disorder caused by deficiency of lysosomal enzyme alpha-galactosidase, which leads to accumulation of globotriasylceramides (GL-3) in visceral tissues and vascular endothelium, causing multi-organ failure. We presenta case of Fabry disease in a 17-year-old patient with mainly gastrointestinal manifestations, diagnosed 10 years after the manifestation of first symptoms. Significant and progressive weight loss with abdominal pain and vomiting, leading to cachexia, were observed in early childhood. The patient was investigated for non-inflammatory bowel diseases, Raynaud syndrome, polimyositis, mitochondrial cytopathies, intestinal lypodystrophies and others. The symptoms of intenstinal pseudo-obstruction syndrome were observed and surgical treatment was instituted because of necrosis of the colon. There was progressive cachexia and parenteral nutrition had to be instituted. Finally, plasma alpha-galactosidase was measured, and its deficit confirmed Fabry disease. In conclusion gastrointestinal symptoms in the course of Fabry disease can obscure other characteristic symptoms, may be prodromal and leading. Heart and renal failure may not occur in children. Unexplained abdominal pain and malnutrition may be gastrointestinal manifestations of metabolic disorders. FAU - Buda, Piotr AU - Buda P AD - Klinika Pediatrii i Zywienia Kierownile prof. dr hab. n. med. I. Ksiqiyk Instytut, Pomnik - Centrum Zdrowia Dziecka" w Warszawie. p.buda@czd.pl FAU - Wieteska-Klimczak, Anna AU - Wieteska-Klimczak A FAU - Ksiazyk, Janusz AU - Ksiazyk J FAU - Smorczewska-Kiljan, Anna AU - Smorczewska-Kiljan A FAU - Gietka, Piotr AU - Gietka P FAU - Czartoryska, Barbara AU - Czartoryska B FAU - Tylki-Szymańska, Anna AU - Tylki-Szymańska A LA - pol PT - Case Reports PT - English Abstract PT - Journal Article TT - Rzadka postać choroby Fabry'ego z ciezkimi objawami ze strony przewodu pokarmowego - trudności diagnostyczne. PL - Poland TA - Med Wieku Rozwoj JT - Medycyna wieku rozwojowego JID - 100928610 RN - EC 3.2.1.22 (alpha-Galactosidase) SB - IM MH - Adolescent MH - Diagnosis, Differential MH - Disease Progression MH - Fabry Disease/*blood/*diagnosis MH - Gastrointestinal Diseases/diagnosis/etiology MH - Humans MH - Male MH - alpha-Galactosidase/*blood EDAT- 2011/07/27 06:00 MHDA- 2011/08/31 06:00 CRDT- 2011/07/27 06:00 PHST- 2011/07/27 06:00 [entrez] PHST- 2011/07/27 06:00 [pubmed] PHST- 2011/08/31 06:00 [medline] PST - ppublish SO - Med Wieku Rozwoj. 2011 Jan-Mar;15(1):69-72. PMID- 37363557 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230701 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 85 IP - 6 DP - 2023 Jun TI - Systemic sclerosis with interstitial lung disease and myocardial infarction: a case report. PG - 3043-3046 LID - 10.1097/MS9.0000000000000740 [doi] AB - Systemic sclerosis (SSc) is a rare autoimmune connective tissue disorder that causes fibrosis due to an accelerated inflammatory response. One of the most frequent co-morbidities with SSc is interstitial lung disease (ILD), which is also one of the biggest killers among SSc patients. CASE PRESENTATION: The authors present a rare case of diffuse SSc with ILD and myocardial infarction having a history of Raynaud phenomenon, skin thickening, and shortness of breath. Antinuclear antibody and antitopoisomerase antibody tests were positive. The patient was managed medically and the condition of patient is improving. CLINICAL DISCUSSION: SSC can affect the skin as well as other organs, with the lungs being the most frequently involved and seriously impacted. SSc patients can have multiple organ involvement like the skin, lungs, heart, kidneys, and gastrointestinal tract. Because ILD is the leading cause of death among people with SSC, early diagnosis and high suspicion of lung involvement can reduce mortality. CONCLUSION: The mortality rate for SSC associated with ILD is extremely high. Even though ILD is common in SSc, it might be difficult to identify and detect early for which a high-resolution CT scan can be used. In SSc patients, heart involvement can coexist with ILD. CI - Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. FAU - Adhikari, Sagar AU - Adhikari S AUID- ORCID: 0000-0002-2761-3574 AD - Department of Internal Medicine, Dhulikhel Hospital, Kathmandu University School of Medical Sciences. FAU - Poudel, Priyanka AU - Poudel P AUID- ORCID: 0000-0002-0882-1052 AD - Kathmandu University School of Medical Sciences, Dhulikhel, Nepal. FAU - Pathak, Sujan AU - Pathak S AUID- ORCID: 0000-0002-0518-385X AD - Kathmandu University School of Medical Sciences, Dhulikhel, Nepal. LA - eng PT - Journal Article DEP - 20230504 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC10289606 OTO - NOTNLM OT - case reports OT - diffuse OT - interstitial lung disease OT - systemic sclerosis COIS- There are no conflicts of interest. EDAT- 2023/06/26 19:07 MHDA- 2023/06/26 19:08 PMCR- 2023/05/04 CRDT- 2023/06/26 13:19 PHST- 2022/12/25 00:00 [received] PHST- 2023/04/15 00:00 [accepted] PHST- 2023/06/26 19:08 [medline] PHST- 2023/06/26 19:07 [pubmed] PHST- 2023/06/26 13:19 [entrez] PHST- 2023/05/04 00:00 [pmc-release] AID - AMSU-D-22-03059 [pii] AID - 10.1097/MS9.0000000000000740 [doi] PST - epublish SO - Ann Med Surg (Lond). 2023 May 4;85(6):3043-3046. doi: 10.1097/MS9.0000000000000740. eCollection 2023 Jun. PMID- 36353844 OWN - NLM STAT- MEDLINE DCOM- 20230525 LR - 20230717 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 53 IP - 5 DP - 2023 May TI - Clinical utility of nailfold capillaroscopy. PG - 671-679 LID - 10.1111/imj.15966 [doi] AB - Nailfold capillaroscopy (NFC) is a simple noninvasive microscopic technique used to identify characteristic morphological abnormalities in the nailfold capillaries. The presence of this microvasculopathy appears to be of fundamental importance in the pathological processes that underlie the scleroderma spectrum disorders (including dermatomyositis and antisynthetase myositis). This review discusses the different methodologies and techniques in performing NFC and stresses the diagnostic utility achieved with simple 'bedside' techniques utilising the ophthalmoscope, dermatoscope or smart phone. Recent advances in reporting abnormal microvascular patterns and vascular metrics (e.g. capillary density and dropout) are discussed. The aetiopathogenesis of the microvasculopathy is currently unknown but its close association with Raynaud Phenomena and specific autoantibodies together with recent observations from sequential NFC allows speculations on its possible mechanism. Finally, future developments in the use of NFC as a possible biomarker in the management of the scleroderma spectrum disorders are discussed, with a recommendation that NFC becomes more widely available, particularly in rheumatological, immunological and dermatological practice. NFC provides a clinically accessible window on the pathologic process fundamental to scleroderma-related disease. CI - © 2022 Royal Australasian College of Physicians. FAU - Roberts-Thomson, Peter J AU - Roberts-Thomson PJ AUID- ORCID: 0000-0002-4330-6398 AD - Rheumatology Unit, Department of Medicine, Flinders Medical Centre, Adelaide, South Australia, Australia. FAU - Patterson, Karen A AU - Patterson KA AD - Rheumatology Unit, Department of Medicine, Flinders Medical Centre, Adelaide, South Australia, Australia. FAU - Walker, Jennifer G AU - Walker JG AD - Rheumatology Unit, Department of Medicine, Flinders Medical Centre, Adelaide, South Australia, Australia. LA - eng PT - Journal Article PT - Review DEP - 20221127 PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - Microscopic Angioscopy/methods MH - *Myositis MH - *Rheumatology MH - Capillaries/pathology MH - Autoantibodies OTO - NOTNLM OT - autoantibodies OT - microvasculopathy OT - nailfold capillaroscopy OT - scleroderma OT - systemic sclerosis EDAT- 2022/11/11 06:00 MHDA- 2023/05/25 06:42 CRDT- 2022/11/10 03:12 PHST- 2022/07/28 00:00 [received] PHST- 2022/09/24 00:00 [accepted] PHST- 2023/05/25 06:42 [medline] PHST- 2022/11/11 06:00 [pubmed] PHST- 2022/11/10 03:12 [entrez] AID - 10.1111/imj.15966 [doi] PST - ppublish SO - Intern Med J. 2023 May;53(5):671-679. doi: 10.1111/imj.15966. Epub 2022 Nov 27. PMID- 17030571 OWN - NLM STAT- MEDLINE DCOM- 20070130 LR - 20181113 IS - 0019-9567 (Print) IS - 1098-5522 (Electronic) IS - 0019-9567 (Linking) VI - 75 IP - 1 DP - 2007 Jan TI - Role of the wbt locus of Francisella tularensis in lipopolysaccharide O-antigen biogenesis and pathogenicity. PG - 536-41 AB - Francisella tularensis is a highly infectious bacterial pathogen, responsible for the zoonotic disease tularemia. We screened a bank of transposon insertion mutants of F. tularensis subsp. holarctica LVS for colony morphology alterations and selected a mutant with a transposon insertion in wbtA, the first gene of the predicted lipopolysaccharide O-antigen gene cluster. Inactivation of wbtA led to the complete loss of O antigen, conferred serum sensitivity, impaired intracellular replication, and severely attenuated virulence in the mouse model. Notably, this mutant afforded protection against a challenge against virulent LVS. FAU - Raynaud, Catherine AU - Raynaud C AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. FAU - Meibom, Karin L AU - Meibom KL FAU - Lety, Marie-Annick AU - Lety MA FAU - Dubail, Iharilalao AU - Dubail I FAU - Candela, Thomas AU - Candela T FAU - Frapy, Eric AU - Frapy E FAU - Charbit, Alain AU - Charbit A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20061009 PL - United States TA - Infect Immun JT - Infection and immunity JID - 0246127 RN - 0 (DNA Transposable Elements) RN - 0 (O Antigens) SB - IM MH - Animals MH - DNA Transposable Elements MH - Female MH - Francisella tularensis/genetics/*immunology/*pathogenicity MH - *Genes, Bacterial MH - Mice MH - Mice, Inbred BALB C MH - Multigene Family MH - Mutagenesis MH - O Antigens/*genetics/*immunology MH - Reverse Transcriptase Polymerase Chain Reaction MH - Tularemia/immunology MH - Virulence PMC - PMC1828372 EDAT- 2006/10/13 09:00 MHDA- 2007/01/31 09:00 PMCR- 2007/05/01 CRDT- 2006/10/13 09:00 PHST- 2006/10/13 09:00 [pubmed] PHST- 2007/01/31 09:00 [medline] PHST- 2006/10/13 09:00 [entrez] PHST- 2007/05/01 00:00 [pmc-release] AID - IAI.01429-06 [pii] AID - 1429-06 [pii] AID - 10.1128/IAI.01429-06 [doi] PST - ppublish SO - Infect Immun. 2007 Jan;75(1):536-41. doi: 10.1128/IAI.01429-06. Epub 2006 Oct 9. PMID- 17537038 OWN - NLM STAT- MEDLINE DCOM- 20070627 LR - 20091103 IS - 1610-0387 (Electronic) IS - 1610-0379 (Linking) VI - 5 IP - 6 DP - 2007 Jun TI - Therapeutic options for digital ulcers in patients with systemic sclerosis. PG - 460-5 AB - Digital ulcers (DU) affect up to half of all patients with systemic sclerosis at some point during their disease. These lesions are extremely painful, heal slowly, and lead to substantial disability. DU arise from recurrent ischemic injury and microtrauma. Treatments for DU include non-pharmacologic modalities such as avoiding cold,stress,and trauma,as well as smoking cessation. Possible pharmacologic therapies for the prevention of DU include vasodilating agents to treat Raynaud phenomenon, statins, and oral agents used in the treatment of pulmonary hypertension (endothelin receptor antagonists, phosphodiesterase-5 inhibitors). The treatment of existing DU includes hydrocolloid occlusion, wound care, pain control, antibiotics, and the use of vasodilating medications. Intravenous or subcutaneous prostacyclins and digital or cervical sympathectomy should be considered for severe cases. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA. shauwei@stanford.edu LA - eng LA - ger PT - Journal Article PT - Review PL - Germany TA - J Dtsch Dermatol Ges JT - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JID - 101164708 RN - 0 (Antihypertensive Agents) RN - 0 (Vasodilator Agents) SB - IM MH - Antihypertensive Agents/*therapeutic use MH - *Fingers MH - Humans MH - Scleroderma, Systemic/*complications/*therapy MH - Skin Ulcer/*complications/*therapy MH - Sympathectomy/*methods MH - Vasodilator Agents/therapeutic use RF - 51 EDAT- 2007/06/01 09:00 MHDA- 2007/06/28 09:00 CRDT- 2007/06/01 09:00 PHST- 2007/06/01 09:00 [pubmed] PHST- 2007/06/28 09:00 [medline] PHST- 2007/06/01 09:00 [entrez] AID - DDG06279 [pii] AID - 10.1111/j.1610-0387.2007.06279.x [doi] PST - ppublish SO - J Dtsch Dermatol Ges. 2007 Jun;5(6):460-5. doi: 10.1111/j.1610-0387.2007.06279.x. PMID- 30981561 OWN - NLM STAT- MEDLINE DCOM- 20200427 LR - 20200427 IS - 1768-3122 (Electronic) IS - 0248-8663 (Linking) VI - 40 IP - 8 DP - 2019 Aug TI - [Very early and early systemic sclerosis: An update]. PG - 517-522 LID - S0248-8663(19)30422-9 [pii] LID - 10.1016/j.revmed.2019.03.327 [doi] AB - Classification criteria for systemic sclerosis evolved over the last three decades, allowing an earlier classification. In the late 2000s, the EULAR Scleroderma Trials and Research Group validated very early and early systemic sclerosis criteria. Raynaud phenomenon, anti-nuclear antibody positivity and the puffy fingers are "Red flags" that must lead to refer the patient to a specialist and benefit from a capillaroscopy and the specific autoantibodies. At the stage of very early systemic sclerosis, pulmonary, cardiac and digestive involvements may be present and must be screened. Herein, we detail very early and early systemic sclerosis criteria, as well as the predictive factors of evolution towards a systemic sclerosis. CI - Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved. FAU - Michaud, M AU - Michaud M AD - Service de médecine interne, hôpital Joseph Ducuing, 15 rue de Varsovie, 31076 Toulouse, France. Electronic address: mmichaud@hjd.asso.fr. FAU - Catros, F AU - Catros F AD - Service de médecine interne, hôpital Joseph Ducuing, 15 rue de Varsovie, 31076 Toulouse, France. FAU - Ancellin, S AU - Ancellin S AD - Service de médecine interne, hôpital Joseph Ducuing, 15 rue de Varsovie, 31076 Toulouse, France. FAU - Gaches, F AU - Gaches F AD - Service de médecine interne, hôpital Joseph Ducuing, 15 rue de Varsovie, 31076 Toulouse, France. LA - fre PT - Journal Article TT - Mise au point sur les sclérodermies très précoces et précoces. DEP - 20190410 PL - France TA - Rev Med Interne JT - La Revue de medecine interne JID - 8101383 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Antibodies, Antinuclear/blood MH - Autoantibodies/blood MH - Decision Trees MH - Disease Progression MH - Early Diagnosis MH - Humans MH - Scleroderma, Systemic/*classification/*diagnosis OTO - NOTNLM OT - Classification criteria OT - Critères de classification OT - Early systemic sclerosis OT - Sclérodermie systémique OT - Sclérodermie systémique précoce OT - Sclérodermie systémique très précoce OT - Systemic sclerosis OT - Very early systemic sclerosis EDAT- 2019/04/15 06:00 MHDA- 2020/04/28 06:00 CRDT- 2019/04/15 06:00 PHST- 2019/01/04 00:00 [received] PHST- 2019/03/11 00:00 [revised] PHST- 2019/03/21 00:00 [accepted] PHST- 2019/04/15 06:00 [pubmed] PHST- 2020/04/28 06:00 [medline] PHST- 2019/04/15 06:00 [entrez] AID - S0248-8663(19)30422-9 [pii] AID - 10.1016/j.revmed.2019.03.327 [doi] PST - ppublish SO - Rev Med Interne. 2019 Aug;40(8):517-522. doi: 10.1016/j.revmed.2019.03.327. Epub 2019 Apr 10. PMID- 26675049 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20151217 LR - 20200930 IS - 2084-8404 (Print) IS - 2451-070X (Electronic) IS - 2084-8404 (Linking) VI - 15 IP - 62 DP - 2015 Sep TI - The value of ultrasound in the diagnosis of limited scleroderma - a case report. PG - 326-31 LID - 10.15557/JoU.2015.0029 [doi] AB - Systemic sclerosis, popularly referred to as scleroderma, is a chronic connective tissue disease with present autoantibodies against platelet-derived growth factor receptor. These antibodies activate directly fibroblasts causing the dermis and internal organs' fibrosis and vascular damage. Additionally, calcific collections, including hydroxyapatite crystals, may develop in subcutaneous tissue and juxta-articular soft tissue. Herein, we report a case of a 72-year-old woman, referred by a rheumatologist for plain radiography and ultrasound examination of hands due to pain and swelling of the fourth finger of the left hand. Dermal induration affecting hands, especially fourth finger on the left side and the Raynaud phenomenon were observed on physical examination. Furthermore, the patient had noticed periodic discharge with a toothpaste consistency from a tiny fistula localised in the vicinity of the fourth finger alterations. The paper emphasises a possible application of the twinkling artefact and MicroPure option on ultrasound examination in differential diagnosis of soft tissue calcifications. Making the correct diagnosis can attribute to precise planning of surgical treatment. FAU - Pracoń, Grzegorz AU - Pracoń G AD - Department of Radiology, Institute of Rheumatology, Warsaw, Poland. FAU - Płaza, Mateusz AU - Płaza M AD - Department of Radiology, Institute of Rheumatology, Warsaw, Poland. FAU - Walentowska-Janowicz, Marta AU - Walentowska-Janowicz M AD - Department of Radiology, Institute of Rheumatology, Warsaw, Poland. FAU - Sudoł-Szopińska, Iwona AU - Sudoł-Szopińska I AD - Department of Radiology, Institute of Rheumatology, Warsaw, Poland ; Department of Diagnostic Imaging, Second Faculty, Warsaw Medical University, Poland. LA - eng PT - Case Reports PT - Journal Article DEP - 20150930 PL - Poland TA - J Ultrason JT - Journal of ultrasonography JID - 101622466 PMC - PMC4657401 OTO - NOTNLM OT - calcinosis OT - colour twinkling artifact OT - limited scleroderma OT - systemic sclerosis OT - ultrasonography EDAT- 2015/12/18 06:00 MHDA- 2015/12/18 06:01 PMCR- 2015/09/01 CRDT- 2015/12/18 06:00 PHST- 2014/12/01 00:00 [received] PHST- 2015/01/09 00:00 [revised] PHST- 2015/01/16 00:00 [accepted] PHST- 2015/12/18 06:00 [entrez] PHST- 2015/12/18 06:00 [pubmed] PHST- 2015/12/18 06:01 [medline] PHST- 2015/09/01 00:00 [pmc-release] AID - 0029 [pii] AID - 10.15557/JoU.2015.0029 [doi] PST - ppublish SO - J Ultrason. 2015 Sep;15(62):326-31. doi: 10.15557/JoU.2015.0029. Epub 2015 Sep 30. PMID- 20808169 OWN - NLM STAT- MEDLINE DCOM- 20101216 LR - 20100902 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 16 IP - 6 DP - 2010 Sep TI - Desquamative interstitial pneumonia as the initial manifestation of systemic sclerosis. PG - 284-6 LID - 10.1097/RHU.0b013e3181eed86d [doi] AB - Interstitial lung disease (ILD) is a frequent pulmonary complication of systemic sclerosis (SSc), and nonspecific interstitial pneumonia is the most commonly recognized pattern of lung injury in these patients. In this report, we describe a never-smoker female presenting with Raynaud phenomenon and ILD that demonstrated desquamative interstitial pneumonia (DIP) on surgical lung biopsy. After 8 months, she was diagnosed with pulmonary hypertension at which time clinical examinations and serologic findings established the diagnosis of SSc. This case report expands the spectrum of patterns of ILD seen in association with SSc to include DIP. FAU - Swartz, Justin S AU - Swartz JS AD - Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, USA. FAU - Chatterjee, Soumya AU - Chatterjee S FAU - Parambil, Joseph G AU - Parambil JG LA - eng PT - Case Reports PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Adrenal Cortex Hormones) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Aged MH - Biopsy MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/etiology MH - Lung/pathology MH - Lung Diseases, Interstitial/*diagnosis/drug therapy/*etiology MH - Scleroderma, Systemic/*complications MH - Treatment Outcome EDAT- 2010/09/03 06:00 MHDA- 2010/12/17 06:00 CRDT- 2010/09/03 06:00 PHST- 2010/09/03 06:00 [entrez] PHST- 2010/09/03 06:00 [pubmed] PHST- 2010/12/17 06:00 [medline] AID - 00124743-201009000-00006 [pii] AID - 10.1097/RHU.0b013e3181eed86d [doi] PST - ppublish SO - J Clin Rheumatol. 2010 Sep;16(6):284-6. doi: 10.1097/RHU.0b013e3181eed86d. PMID- 26546402 OWN - NLM STAT- MEDLINE DCOM- 20160712 LR - 20231111 IS - 1471-2407 (Electronic) IS - 1471-2407 (Linking) VI - 15 DP - 2015 Nov 6 TI - Management of malignant pleural mesothelioma: a French multicenter retrospective study (GFPC 0802 study). PG - 857 LID - 10.1186/s12885-015-1881-x [doi] LID - 857 AB - BACKGROUND: Malignant pleural mesothelioma (MPM) is a rare disease with poor prognosis in spite of significant improvement in survival, due to new chemotherapy regimens. We describe here patients' profiles and management in daily practice in France. METHODS: Observational retrospective study. Data were collected from medical files. All patients with histologically proven MPM diagnosed from January 2005 to December 2008 were included in the participating sites. RESULTS: Four hundred and six patients were included in 37 sites: mean age 68.9 ± 9.8 years, male predominance (sex ratio 3.27), latency of the disease 45.7 years, epithelioïd type 83 %. Diagnosis was made using thoracoscopy in 80.8 % of patients. Radical surgery was performed in 6.2 % of cases. Chemotherapy was administered to 74.6 % of patients. First line regimens consisted mainly of platinum + pemetrexed (91 %) or pemetrexed alone (7 %). Objective response rate was 17.2 % and another 41.6 % of patients experienced disease stabilization. Half of these patients underwent second line chemotherapy (platinium + pemetrexed 31.6 %, pemetrexed alone 24.6 %), resulting in a 6 % response rate. Third-line chemotherapy (56 patients) yielded disease control in 5.4 % of cases. CONCLUSIONS: The management of MPM in France is usually in accordance with guidelines. Response rates are somewhat lower than those described in clinical trials. FAU - Raynaud, C AU - Raynaud C AD - Service de Pneumologie, CH Argenteuil, Argenteuil, France. christine.raynaud@ch-argenteuil.fr. FAU - Greillier, L AU - Greillier L AD - Service d'oncologie thoracique, APHM, Marseille, Services de Pneumologie, Marseille, France. laurent.greillier@ap-hm.fr. FAU - Mazieres, J AU - Mazieres J AD - CHU Toulouse, Toulouse, France. mazieres.j@chu-toulouse.fr. FAU - Monnet, I AU - Monnet I AD - Service de pneumologie, CHI Créteil, 40 avenue de verdun, 94010, Créteil, France. isabelle.monnet@chicreteil.fr. FAU - Mastroianni, B AU - Mastroianni B AD - CHU Lyon, Lyon, France. benedicte.mastroianni@hcl.fr. FAU - Robinet, G AU - Robinet G AD - CHU Brest, Brest, France. gilles.robinet@chu-brest.fr. FAU - Fraboulet, G AU - Fraboulet G AD - CH Cergy Pontoise, Cergy Pontoise, France. gislaine.fraboulet@ch-pontoise.fr. FAU - Dixmier, A AU - Dixmier A AD - CH d'Orléans, Orléans, France. adrien.dixmier@chu-orleans.fr. FAU - Berard, H AU - Berard H AD - HIA Toulon, Toulon, France. hlberard@orange.fr. FAU - Lamy, R AU - Lamy R AD - CH Lorient, Lorient, France. regine.lamy@orange.fr. FAU - Letreut, J AU - Letreut J AD - CH Aix en Provence, Aix en Provence, France. jletreut@ch-aix.fr. FAU - Lena, H AU - Lena H AD - CHU Rennes, Rennes, France. herve.lena@chu-rennes.fr. FAU - Oliviero, G AU - Oliviero G AD - CH Longjumeau, Longjumeau, France. gerard.oliviero@ch-longjumeau.fr. FAU - Botta, S AU - Botta S AD - CHU Rouen, Rouen, France. suzanna.botta@chu-rouen.fr. FAU - Vergnenegre, A AU - Vergnenegre A AD - CHU Limoges, Limoges, France. alain.vergnenegre@unilim.fr. FAU - Borget, I AU - Borget I AD - IGR, Villejuif, France. isabelle.borget@igr.fr. FAU - Chouaid, C AU - Chouaid C AD - Service de pneumologie, CHI Créteil, 40 avenue de verdun, 94010, Créteil, France. christos.chouaid@chicreteil.fr. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20151106 PL - England TA - BMC Cancer JT - BMC cancer JID - 100967800 SB - IM MH - Aged MH - Disease Management MH - Female MH - France/epidemiology MH - Humans MH - Lung Neoplasms/*diagnosis/epidemiology/*therapy MH - Male MH - Mesothelioma/*diagnosis/epidemiology/*therapy MH - Mesothelioma, Malignant MH - Middle Aged MH - Outcome Assessment, Health Care MH - Pleural Neoplasms/*diagnosis/epidemiology/*therapy MH - Retrospective Studies MH - Risk Factors PMC - PMC4635998 EDAT- 2015/11/08 06:00 MHDA- 2016/07/13 06:00 PMCR- 2015/11/06 CRDT- 2015/11/08 06:00 PHST- 2015/01/25 00:00 [received] PHST- 2015/10/30 00:00 [accepted] PHST- 2015/11/08 06:00 [entrez] PHST- 2015/11/08 06:00 [pubmed] PHST- 2016/07/13 06:00 [medline] PHST- 2015/11/06 00:00 [pmc-release] AID - 10.1186/s12885-015-1881-x [pii] AID - 1881 [pii] AID - 10.1186/s12885-015-1881-x [doi] PST - epublish SO - BMC Cancer. 2015 Nov 6;15:857. doi: 10.1186/s12885-015-1881-x. PMID- 41384188 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251212 LR - 20251214 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 17 IP - 11 DP - 2025 Nov TI - An Unusual Case of Thromboangiitis Obliterans With Concurrent Deep Vein Thrombosis and Pulmonary Embolism. PG - e96506 LID - 10.7759/cureus.96506 [doi] LID - e96506 AB - Buerger's disease (thromboangiitis obliterans (TAO)) is a rare, smoking-related vasculitis that primarily affects distal extremity vessels but can occasionally involve the venous system. We report a 45-year-old heavy smoker with chronic obstructive pulmonary disease (COPD), Raynaud phenomenon, prior digital autoamputation, and necrotic fingertip lesions who presented with dyspnea, hemoptysis, rib pain, and thigh discomfort. He was tachycardic, tachypneic, and hypoxic, with digital gangrene and synovitis. Laboratory studies showed leukocytosis, elevated inflammatory markers, and positive antinuclear antibody (ANA). Duplex ultrasound revealed extensive bilateral deep vein thrombosis (DVT), and CT angiography confirmed acute pulmonary embolism (PE) with right heart strain. The patient was diagnosed with TAO complicated by systemic thromboembolic disease and treated with anticoagulation alongside strict smoking cessation counseling. This case underscores that although TAO is classically a peripheral arterial disease, venous involvement can predispose patients to DVT and PE, especially in the setting of systemic inflammation. Clinicians should maintain suspicion for PE in TAO patients with unexplained respiratory symptoms and emphasize tobacco cessation as essential for preventing recurrence and progression. CI - Copyright © 2025, Vaezi et al. FAU - Vaezi, Zahra AU - Vaezi Z AD - Internal Medicine, St. Luke's Hospital, Chesterfield, USA. FAU - Baker, Donica L AU - Baker DL AD - Rheumatology, St. Luke's Hospital, Chesterfield, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20251110 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC12695112 OTO - NOTNLM OT - autoamputation OT - buerger’s disease OT - deep vein thrombosis (dvt) OT - pulmonary embolism (pe) OT - thromboangiitis obliterans COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2025/12/12 07:19 MHDA- 2025/12/12 07:20 PMCR- 2025/11/10 CRDT- 2025/12/12 04:37 PHST- 2025/11/10 00:00 [accepted] PHST- 2025/12/12 07:20 [medline] PHST- 2025/12/12 07:19 [pubmed] PHST- 2025/12/12 04:37 [entrez] PHST- 2025/11/10 00:00 [pmc-release] AID - 10.7759/cureus.96506 [doi] PST - epublish SO - Cureus. 2025 Nov 10;17(11):e96506. doi: 10.7759/cureus.96506. eCollection 2025 Nov. PMID- 16966022 OWN - NLM STAT- MEDLINE DCOM- 20061228 LR - 20060912 IS - 0738-081X (Print) IS - 0738-081X (Linking) VI - 24 IP - 5 DP - 2006 Sep-Oct TI - Rheumatoid arthritis in dermatology. PG - 430-7 AB - Rheumatoid arthritis (RA) is a chronic progressive disorder characterized by symmetric inflammatory arthritis in association with systemic symptoms. Although considered a "joint disease," RA is associated with involvement in diverse organ systems, including the skin. Common manifestations include Raynaud phenomenon, rheumatoid nodules, and rheumatoid vasculitis. As with other extra-articular manifestations, dermatologic involvement tends to occur in patients with more severe RA. In addition to manifestations related to the disease, there are also sundry dermatologic reactions related to the medications used to treat RA. Understanding the etiology and therapy for cutaneous manifestations of RA will help optimize patient care. FAU - Hata, Tissa AU - Hata T AD - Department of Medicine, Division of Dermatology, University of California, San Diego School of Medicine, La Jolla, 92093-0943, USA. thata@ucsd.edu FAU - Kavanaugh, Arthur AU - Kavanaugh A LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 RN - 0 (Antirheumatic Agents) RN - 0 (Immunosuppressive Agents) RN - 0 (Tumor Necrosis Factor-alpha) SB - IM MH - Antirheumatic Agents/adverse effects/therapeutic use MH - Arthritis, Rheumatoid/complications/drug therapy/*pathology/physiopathology MH - Humans MH - Immunosuppressive Agents/adverse effects/therapeutic use MH - Skin/pathology/physiopathology MH - Skin Diseases/drug therapy/etiology/*pathology/physiopathology MH - Tumor Necrosis Factor-alpha/antagonists & inhibitors RF - 121 EDAT- 2006/09/13 09:00 MHDA- 2006/12/29 09:00 CRDT- 2006/09/13 09:00 PHST- 2006/09/13 09:00 [pubmed] PHST- 2006/12/29 09:00 [medline] PHST- 2006/09/13 09:00 [entrez] AID - S0738-081X(06)00100-3 [pii] AID - 10.1016/j.clindermatol.2006.07.008 [doi] PST - ppublish SO - Clin Dermatol. 2006 Sep-Oct;24(5):430-7. doi: 10.1016/j.clindermatol.2006.07.008. PMID- 35850975 OWN - NLM STAT- MEDLINE DCOM- 20220721 LR - 20220923 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 8 IP - 2 DP - 2022 Jul TI - Recommendations for the execution and reporting of skin ultrasound in systemic sclerosis: an international collaboration under the WSF skin ultrasound group. LID - 10.1136/rmdopen-2022-002371 [doi] LID - e002371 AB - OBJECTIVE: Ultrasound is a promising tool to foster much-needed improvement of skin assessment in systemic sclerosis (SSc). Our aim was to develop evidence and expert opinion-based recommendations to promote the standardisation and harmonisation of technical execution and reporting of skin ultrasound studies in SSc. METHODS: A multidisciplinary task force of 16 members from five European countries and Japan was convened under the auspices of World Scleroderma Foundation. First, a systematic literature review (SLR) was performed. Then, each member proposed and formulated items to the overarching principles, recommendations and research agenda. Two rounds of mails exchange for consensus as well as an on-line meeting were performed to debate and refine the proposals. Two Delphi rounds of voting resulted in the final recommendations. Levels of evidence and strengths of recommendations were assigned, and task force members voted anonymously on the level of agreement with each of the items. RESULTS: Five overarching principles and seven recommendations were developed, based on an SLR and expert opinion, through consensus procedures. The overarching principles highlight the promising role of skin ultrasound in SSc assessment, the need for standardisation of technical aspects, sufficient training and adequate equipment. The recommendations provide standards for the execution and reporting of skin ultrasound in SSc. The research agenda includes the need for more research into unmet needs according to Outcome Measures in Rheumatology Algorithm requirements. CONCLUSION: These are the first recommendations providing guidance on the execution and reporting of skin ultrasound in SSc patients, aiming at improving the interpretability, reliability and generalisability of skin ultrasound, thus consolidating its role in research and practice. CI - © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Santiago, Tânia AU - Santiago T AUID- ORCID: 0000-0002-1562-4022 AD - Rheumatology, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal tlousasantiago@hotmail.com. AD - Faculty of Medicine, University of Coimbra, Coimbra, Portugal. FAU - Santos, Eduardo José Ferreira AU - Santos EJF AUID- ORCID: 0000-0003-0557-2377 AD - Health Sciences Research Unit: Nursing (UICiSA:E), Coimbra, Portugal. FAU - Ruaro, Barbara AU - Ruaro B AD - Pulmonology, University Hospital of Cattinara, Trieste, Italy. FAU - Lepri, Gemma AU - Lepri G AUID- ORCID: 0000-0003-4141-6937 AD - Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Internal Medicine, University of Florence, Firenze, Italy. FAU - Green, Lorraine AU - Green L AD - Leeds Institute of Molecular Medicine Section of Musculoskeletal Disease, Leeds, UK. FAU - Wildt, Marie AU - Wildt M AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Watanabe, Shinji AU - Watanabe S AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Lescoat, Alain AU - Lescoat A AD - Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Université de Rennes 1, Rennes, France. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - Scleroderma Programme, NIHR Leeds Musculoskeletal Biomedical Research Centre, Leeds, UK. FAU - Pauling, John D AU - Pauling JD AD - Royal National Hospital for Rheumatic Diseases, Royal United Hospital Bath NHS Trust, Bath, UK. AD - Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. FAU - Reeve, Lucy Jean AU - Reeve LJ AD - Scleroderma and Raynaud's, London, UK. FAU - D'Agostino, Maria Antonieta AU - D'Agostino MA AD - Rheumatology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma, Italy. AD - Catholic University of the Sacred Heart Faculty of Medicine and Surgery, Roma, Italy. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - Experimental and Clinical Medicine, University of Florence, Florence, Italy. AD - Department of Internal Medicine, University of Florence, Firenze, Italy. FAU - Iagnocco, Annamaria AU - Iagnocco A AUID- ORCID: 0000-0001-5592-724X AD - Academic Rheumatology Centre, Department of Clinical and Biological Science, University of Turin, Tourin, Italy. FAU - da Silva, Jose Antonio Pereira AU - da Silva JAP AUID- ORCID: 0000-0002-2782-6780 AD - Rheumatology, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal. AD - Rheumatology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review PL - England TA - RMD Open JT - RMD open JID - 101662038 SB - IM MH - Consensus MH - Humans MH - Reproducibility of Results MH - *Rheumatology MH - *Scleroderma, Systemic/diagnostic imaging MH - Skin/diagnostic imaging PMC - PMC9297224 OTO - NOTNLM OT - Outcome and Process Assessment, Health Care OT - Scleroderma, Systemic OT - Ultrasonography COIS- Competing interests: None declared. EDAT- 2022/07/20 06:00 MHDA- 2022/07/22 06:00 PMCR- 2022/07/18 CRDT- 2022/07/19 07:56 PHST- 2022/03/29 00:00 [received] PHST- 2022/06/27 00:00 [accepted] PHST- 2022/07/19 07:56 [entrez] PHST- 2022/07/20 06:00 [pubmed] PHST- 2022/07/22 06:00 [medline] PHST- 2022/07/18 00:00 [pmc-release] AID - rmdopen-2022-002371 [pii] AID - 10.1136/rmdopen-2022-002371 [doi] PST - ppublish SO - RMD Open. 2022 Jul;8(2):e002371. doi: 10.1136/rmdopen-2022-002371. PMID- 36926342 OWN - NLM STAT- MEDLINE DCOM- 20230320 LR - 20230328 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 14 DP - 2023 TI - An external quality assessment feasibility study; cross laboratory comparison of haemagglutination inhibition assay and microneutralization assay performance for seasonal influenza serology testing: A FLUCOP study. PG - 1129765 LID - 10.3389/fimmu.2023.1129765 [doi] LID - 1129765 AB - INTRODUCTION: External Quality Assessment (EQA) schemes are designed to provide a snapshot of laboratory proficiency, identifying issues and providing feedback to improve laboratory performance and inter-laboratory agreement in testing. Currently there are no international EQA schemes for seasonal influenza serology testing. Here we present a feasibility study for conducting an EQA scheme for influenza serology methods. METHODS: We invited participant laboratories from industry, contract research organizations (CROs), academia and public health institutions who regularly conduct hemagglutination inhibition (HAI) and microneutralization (MN) assays and have an interest in serology standardization. In total 16 laboratories returned data including 19 data sets for HAI assays and 9 data sets for MN assays. RESULTS: Within run analysis demonstrated good laboratory performance for HAI, with intrinsically higher levels of intra-assay variation for MN assays. Between run analysis showed laboratory and strain specific issues, particularly with B strains for HAI, whilst MN testing was consistently good across labs and strains. Inter-laboratory variability was higher for MN assays than HAI, however both assays showed a significant reduction in inter-laboratory variation when a human sera pool is used as a standard for normalization. DISCUSSION: This study has received positive feedback from participants, highlighting the benefit such an EQA scheme would have on improving laboratory performance, reducing inter laboratory variation and raising awareness of both harmonized protocol use and the benefit of biological standards for seasonal influenza serology testing. CI - Copyright © 2023 Waldock, Weiss, Wang, Levine, Jefferson, Ho, Hoschler, Londt, Masat, Carolan, Sánchez-Ovando, Fox, Watanabe, Akimoto, Sato, Kishida, Buys, Maake, Fourie, Caillet, Raynaud, Webby, DeBeauchamp, Cox, Lartey, Trombetta, Marchi, Montomoli, Sanz-Muñoz, Eiros, Sánchez-Martínez, Duijsings and Engelhardt. FAU - Waldock, Joanna AU - Waldock J AD - Vaccines, Science Research & Innovation, Medicines and Healthcare Products Regulatory, Potters Bar, United Kingdom. FAU - Weiss, Carol D AU - Weiss CD AD - Center for Biologics Evaluation and Research, US Food and Drug Administration, Silver Spring, MD, United States. FAU - Wang, Wei AU - Wang W AD - Center for Biologics Evaluation and Research, US Food and Drug Administration, Silver Spring, MD, United States. FAU - Levine, Min Z AU - Levine MZ AD - Influenza Division, Centers for Disease Control and Prevention, National Center for Immunization and Respiratory Diseases, Atlanta, GA, United States. FAU - Jefferson, Stacie N AU - Jefferson SN AD - Influenza Division, Centers for Disease Control and Prevention, National Center for Immunization and Respiratory Diseases, Atlanta, GA, United States. FAU - Ho, Sammy AU - Ho S AD - Respiratory Viruses Unit, UK Health Security Agency, Colindale, United Kingdom. FAU - Hoschler, Katja AU - Hoschler K AD - Respiratory Viruses Unit, UK Health Security Agency, Colindale, United Kingdom. FAU - Londt, Brandon Z AU - Londt BZ AD - hVivo The Queen Mary Bioenterprises (QMB) Innovation, London, United Kingdom. FAU - Masat, Elisa AU - Masat E AD - hVivo The Queen Mary Bioenterprises (QMB) Innovation, London, United Kingdom. FAU - Carolan, Louise AU - Carolan L AD - World Health Organisation (WHO) Collaborating Centre for Reference and Research on Influenza, Royal Melbourne Hospital, at the Peter Doherty Institute for Infection and Immunity, Melbourne, VIC, Australia. FAU - Sánchez-Ovando, Stephany AU - Sánchez-Ovando S AD - World Health Organisation (WHO) Collaborating Centre for Reference and Research on Influenza, Royal Melbourne Hospital, at the Peter Doherty Institute for Infection and Immunity, Melbourne, VIC, Australia. AD - Department of Infectious Diseases, University of Melbourne at the Peter Doherty Institute for Infection and Immunity, Melbourne, VIC, Australia. FAU - Fox, Annette AU - Fox A AD - World Health Organisation (WHO) Collaborating Centre for Reference and Research on Influenza, Royal Melbourne Hospital, at the Peter Doherty Institute for Infection and Immunity, Melbourne, VIC, Australia. AD - Department of Infectious Diseases, University of Melbourne at the Peter Doherty Institute for Infection and Immunity, Melbourne, VIC, Australia. FAU - Watanabe, Shinji AU - Watanabe S AD - Center for Influenza and Respiratory Virus Research, National Institute of Infectious Diseases: Musashi-Murayama, Tokyo, Japan. FAU - Akimoto, Miki AU - Akimoto M AD - Center for Influenza and Respiratory Virus Research, National Institute of Infectious Diseases: Musashi-Murayama, Tokyo, Japan. FAU - Sato, Aya AU - Sato A AD - Center for Influenza and Respiratory Virus Research, National Institute of Infectious Diseases: Musashi-Murayama, Tokyo, Japan. FAU - Kishida, Noriko AU - Kishida N AD - Center for Influenza and Respiratory Virus Research, National Institute of Infectious Diseases: Musashi-Murayama, Tokyo, Japan. FAU - Buys, Amelia AU - Buys A AD - Centre for Respiratory Diseases and Meningitis, National Institute for Communicable Diseases (NICD) of the National Health Laboratory Services, Johannesburg, South Africa. FAU - Maake, Lorens AU - Maake L AD - Centre for Respiratory Diseases and Meningitis, National Institute for Communicable Diseases (NICD) of the National Health Laboratory Services, Johannesburg, South Africa. FAU - Fourie, Cardia AU - Fourie C AD - Centre for Respiratory Diseases and Meningitis, National Institute for Communicable Diseases (NICD) of the National Health Laboratory Services, Johannesburg, South Africa. FAU - Caillet, Catherine AU - Caillet C AD - Department of Research and Development, Sanofi, Lyon, France. FAU - Raynaud, Sandrine AU - Raynaud S AD - Department of Research and Development, Sanofi, Lyon, France. FAU - Webby, Richard J AU - Webby RJ AD - Department of Infectious Diseases, St Jude Children's Research Hospital, Memphis, TN, United States. FAU - DeBeauchamp, Jennifer AU - DeBeauchamp J AD - Department of Infectious Diseases, St Jude Children's Research Hospital, Memphis, TN, United States. FAU - Cox, Rebecca J AU - Cox RJ AD - Influenza Centre, Department of Clinical Sciences, University of Bergen, Bergen, Norway. FAU - Lartey, Sarah L AU - Lartey SL AD - Influenza Centre, Department of Clinical Sciences, University of Bergen, Bergen, Norway. FAU - Trombetta, Claudia M AU - Trombetta CM AD - Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy. FAU - Marchi, Serena AU - Marchi S AD - Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy. FAU - Montomoli, Emanuele AU - Montomoli E AD - Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy. FAU - Sanz-Muñoz, Iván AU - Sanz-Muñoz I AD - National Influenza Centre of Valladolid, Instituto de Estudios de Ciencias de la Salud de Castilla y León (ICSCYL), Edificio Rondilla, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. FAU - Eiros, José María AU - Eiros JM AD - National Influenza Centre of Valladolid, Instituto de Estudios de Ciencias de la Salud de Castilla y León (ICSCYL), Edificio Rondilla, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. FAU - Sánchez-Martínez, Javier AU - Sánchez-Martínez J AD - National Influenza Centre of Valladolid, Instituto de Estudios de Ciencias de la Salud de Castilla y León (ICSCYL), Edificio Rondilla, Hospital Clínico Universitario de Valladolid, Valladolid, Spain. FAU - Duijsings, Danny AU - Duijsings D AD - Viroclinics, Clinical Virology Services, Rotterdam, Netherlands. FAU - Engelhardt, Othmar G AU - Engelhardt OG AD - Vaccines, Science Research & Innovation, Medicines and Healthcare Products Regulatory, Potters Bar, United Kingdom. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20230228 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 SB - IM MH - Humans MH - *Influenza, Human MH - Hemagglutination MH - Laboratories MH - Feasibility Studies MH - Seasons PMC - PMC10011125 OTO - NOTNLM OT - external quality assessment (EQA) OT - haemagglutination inhibition (HAI) OT - influenza viruses OT - microneutralization (MN) OT - serology OT - standardization COIS- EMo is Chief Scientific Officer of VisMederi srl and VisMederi Research srl. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/03/18 06:00 MHDA- 2023/03/21 06:00 PMCR- 2023/01/01 CRDT- 2023/03/17 02:48 PHST- 2022/12/22 00:00 [received] PHST- 2023/02/10 00:00 [accepted] PHST- 2023/03/17 02:48 [entrez] PHST- 2023/03/18 06:00 [pubmed] PHST- 2023/03/21 06:00 [medline] PHST- 2023/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2023.1129765 [doi] PST - epublish SO - Front Immunol. 2023 Feb 28;14:1129765. doi: 10.3389/fimmu.2023.1129765. eCollection 2023. PMID- 26039888 OWN - NLM STAT- MEDLINE DCOM- 20161101 LR - 20260128 IS - 2261-2211 (Electronic) IS - 2261-3684 (Linking) VI - 25 IP - 4 DP - 2015 Oct-Dec TI - [Cryoglobulinemia in a Tunisian internal medicine department]. PG - 414-8 LID - 10.1684/mst.2015.0477 [doi] AB - AIM: Cryoglobulinemia is characterized by multiple organ involvement, mainly including the skin, liver, kidneys, and peripheral nerves. Our aim was to investigate the demographic, clinical, and serologic features, as well as survival in a group of 16 Tunisian patients with cryoglobulinemia. RESULTS: The study included 12 women and 4 men, and their mean age was 41 years. In all but two, the cryoglobulinemia was associated with another disease. These included lupus for 9, Sjögren syndrome for 2, and polyarteritis nodosa for one. They also included infectious diseases: 3 patients with hepatitis B virus infection, one with hepatitis C virus infection, one with parvovirus B19, and another with lymph node tuberculosis. Only one case of lymphoproliferative disease was noted. General symptoms were present in 81% of the patients, cutaneous vasculitis in 43%, peripheral vascular-Raynaud phenomenon in 37%, joint polyarthralgia or arthritis in 62%, renal involvement in 68%, neuropathy in 25%, lung involvement in 56%, gastrointestinal involvement in 37%, and finally cardiac involvement in 31%. In some cases it was difficult to determine if the clinical signs were attributable to cryoglobulinemia or the underlying pathology. The course was favorable under treatment for 5 patients, while 7 patients became sicker and 5 finally died. CONCLUSION: Cryoglobulinemia is underdiagnosed. Treatment depends on the severity of the lesions and the underlying disease. FAU - Boukhris, I AU - Boukhris I AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. FAU - Azzabi, S AU - Azzabi S AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. FAU - Chérif, E AU - Chérif E AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. FAU - Ben Hassine, L AU - Ben Hassine L AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. FAU - Kaouech, Z AU - Kaouech Z AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. FAU - Kooli, C AU - Kooli C AD - CHU Charles Nicolle de Tunis, université de Tunis El Manar, service de médecine interne B, hôpital Charles Nicolle, 1006 Tunis, Tunisie. LA - fre PT - Journal Article TT - Cryoglobulinémie : expérience dans un service de médecine interne tunisien. PL - France TA - Med Sante Trop JT - Medecine et sante tropicales JID - 101581406 SB - IM MH - Adolescent MH - Adult MH - Aged MH - *Cryoglobulinemia/diagnosis/therapy MH - Female MH - Humans MH - Internal Medicine MH - Male MH - Middle Aged MH - Retrospective Studies MH - Tunisia MH - Young Adult OTO - NOTNLM OT - Tunisie OT - cryoglobulinemia OT - hepatitis C OT - systemic lupus erythematosus OT - vasculitis EDAT- 2015/06/04 06:00 MHDA- 2016/11/02 06:00 CRDT- 2015/06/04 06:00 PHST- 2015/06/04 06:00 [entrez] PHST- 2015/06/04 06:00 [pubmed] PHST- 2016/11/02 06:00 [medline] AID - mst.2015.0477 [pii] AID - 10.1684/mst.2015.0477 [doi] PST - ppublish SO - Med Sante Trop. 2015 Oct-Dec;25(4):414-8. doi: 10.1684/mst.2015.0477. PMID- 18293684 OWN - NLM STAT- MEDLINE DCOM- 20080623 LR - 20110113 IS - 0048-7848 (Print) IS - 0048-7848 (Linking) VI - 111 IP - 3 DP - 2007 Jul-Sep TI - Extragastric manifestations of Helicobacter pylori infection. PG - 575-83 AB - The role of Helicobacter pylori (HP) in some digestive diseases (gastritis, ulcer, gastric cancer, MALT lymphoma) is well known. It has been suggested relatively recently that infection with HP can be involved in various extra-digestive conditions: respiratory disorders (chronic obstructive pulmonary disease, bronchiectasis, lung cancer, pulmonary tuberculosis, bronchial asthma); vascular disorders (ischaemic heart disease, stroke, primary Raynaud phenomena, primary headache); autoimmune disorders (Sjogren syndrome, Henoch-Schonlein purpura, autoimmune thrombocytopenia, autoimmune thyroiditis, Parkinson's disease, idiopathic chronic urticaria, rosacea, alopecia areata); other disorders (iron deficiency anaemia, growth retardations, liver cirrhosis). Case studies, small patient series and non-randomized trials that have shown a beneficial effect of HP eradication in different conditions are not convincing. According to Mastricht III the only conditions where HP eradication is indicated are immune thrombocytopenic purpura and iron deficiency anaemia. FAU - Prelipcean, Cristina Cijevschi AU - Prelipcean CC AD - Gr. T. Popa University of Medicine and Pharmacy Iaşi, Institute of Gastroenterology and Hepatology Iaşi. FAU - Mihai, Cătălina AU - Mihai C FAU - Gogălniceanu, P AU - Gogălniceanu P FAU - Mitrică, Dana AU - Mitrică D FAU - Drug, V L AU - Drug VL FAU - Stanciu, C AU - Stanciu C LA - eng PT - Journal Article PT - Review PL - Romania TA - Rev Med Chir Soc Med Nat Iasi JT - Revista medico-chirurgicala a Societatii de Medici si Naturalisti din Iasi JID - 0413735 SB - IM MH - Anemia, Iron-Deficiency/microbiology MH - Autoimmune Diseases/microbiology MH - Case-Control Studies MH - Clinical Trials as Topic MH - Growth Disorders/microbiology MH - Helicobacter Infections/*complications/diagnosis/drug therapy MH - *Helicobacter pylori/pathogenicity MH - Humans MH - Liver Cirrhosis/microbiology MH - Respiratory Tract Diseases/microbiology MH - Risk Factors MH - Vascular Diseases/microbiology RF - 64 EDAT- 2008/02/26 09:00 MHDA- 2008/06/24 09:00 CRDT- 2008/02/26 09:00 PHST- 2008/02/26 09:00 [pubmed] PHST- 2008/06/24 09:00 [medline] PHST- 2008/02/26 09:00 [entrez] PST - ppublish SO - Rev Med Chir Soc Med Nat Iasi. 2007 Jul-Sep;111(3):575-83. PMID- 31536185 STAT- Publisher CTDT- 20190919 PB - University of Washington, Seattle DP - 1993 TI - Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations. BTI - GeneReviews(®) AB - CLINICAL CHARACTERISTICS: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a small-vessel disease that affects highly vascularized tissues including the retina, brain, liver, and kidneys. Age of onset is often between 35 and 50 years. The most common presenting finding is decreased visual acuity and/or visual field defects. Neurologic manifestations may include hemiparesis, facial weakness, aphasia, and hemianopsia. Migraines and seizures are less frequently described. Renal manifestations may include mild-to-moderate increase in serum creatinine and mild proteinuria; progression to end-stage renal disease (ESRD) is uncommon. Hepatic manifestations frequently include mildly elevated levels of alkaline phosphatase and gamma-glutamyltransferase (GGT). Less common findings include psychiatric disorders, hypertension, mild-to-moderate anemia, and Raynaud phenomenon. DIAGNOSIS/TESTING: The diagnosis of RVCL-S is established in a proband with suggestive findings and a heterozygous pathogenic variant in TREX1 identified by molecular genetic testing. MANAGEMENT: Treatment of manifestations: Retinal vasculopathy may be treated with laser therapy, which may also prevent and slow the progression of visual impairment; macular edema may respond to bevacizumab; ESRD may require renal replacement therapy (including renal transplantation); corticosteroid therapy may be considered for those with cerebral vasogenic edema; standard treatment for glaucoma, hypertension, migraine headaches, seizure disorders, hypothyroidism, anemia, Raynaud phenomenon, and psychiatric disorders. Surveillance: Ophthalmologic evaluation, blood pressure assessment, renal function tests (serum creatinine, BUN, and urinalysis to include creatinine and protein content), liver function tests (AST, ALT, alkaline phosphatase, GGT, serum albumin), TSH and free T4, and complete blood count annually starting in the fourth decade or as appropriate based on symptoms; annual assessment of cognition and psychiatric manifestations. Agents/circumstances to avoid: Intravenous tissue-type plasminogen activator therapy for acute ischemic stroke is not warranted, as there is no proof that neurologic manifestations are caused by occluded large blood vessels and the risk of complications is assumed to be higher in affected individuals. Evaluation of relatives at risk: It is appropriate to evaluate the genetic status of apparently asymptomatic older and younger at-risk relatives of an affected individual by molecular genetic testing of the TREX1 pathogenic variant in the family in order to identify as early as possible those who would benefit from prompt initiation of treatment and preventive measures. GENETIC COUNSELING: RVCL-S is inherited in an autosomal dominant manner. Most individuals diagnosed with RVCL-S have an affected parent. However, disease onset and severity vary considerably even within the same family. The offspring of an individual with RVCL-S are at a 50% risk of inheriting the TREX1 pathogenic variant. If the pathogenic variant in the family is known, prenatal testing for pregnancies at increased risk for RVCL-S and preimplantation genetic testing are possible; however, such testing for adult-onset disorders is uncommon. CI - Copyright © 1993-2026, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved. Test. FED - Adam, Margaret P ED - Adam MP FED - Bick, Sarah ED - Bick S FED - Mirzaa, Ghayda M ED - Mirzaa GM FED - Pagon, Roberta A ED - Pagon RA FED - Wallace, Stephanie E ED - Wallace SE FED - Amemiya, Anne ED - Amemiya A FAU - de Boer, Irene AU - de Boer I AD - Leiden University Medical Center Leiden, The Netherlands FAU - Pelzer, Nadine AU - Pelzer N AD - Leiden University Medical Center Leiden, The Netherlands FAU - Terwindt, Gisela AU - Terwindt G AD - Leiden University Medical Center Leiden, The Netherlands LA - eng PT - Review PT - Book Chapter PL - Seattle (WA) OTO - NLM OT - RVCL-S OT - Retinal Vasculopathy with Cerebral Leukodystrophy (RVCL) OT - Cerebroretinal Vasculopathy (CRV) OT - Hereditary Vascular Retinopathy (HVR) OT - Hereditary Systemic Angiopathy (HAS) OT - Hereditary Endotheliopathy, Retinopathy, Nephropathy, and Stroke (HERNS) OT - Three prime repair exonuclease 1 OT - TREX1 OT - Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations OT - Cerebroretinal Vasculopathy (CRV) OT - Hereditary Endotheliopathy, Retinopathy, Nephropathy, and Stroke (HERNS) OT - Hereditary Systemic Angiopathy (HSA) OT - Hereditary Vascular Retinopathy (HVR) OT - Retinal Vasculopathy with Cerebral Leukodystrophy (RVCL) OT - RVCL-S EDAT- 2019/09/19 00:00 CRDT- 2019/09/19 00:00 AID - NBK546576 [bookaccession] PMID- 31681774 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 6 DP - 2019 TI - Interstitial Pneumonia With Autoimmune Features (IPAF). PG - 209 LID - 10.3389/fmed.2019.00209 [doi] LID - 209 AB - A significant proportion of patients with interstitial lung disease (ILD) manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease (CTD). In 2015, the European Respiratory Society (ERS) and American Thoracic Society (ATS) "Task Force on undifferentiated Forms of connective tissue disease-associated interstitial lung disease" proposed classification criteria for a so-called research category of Interstitial Pneumonia with Autoimmune Features (IPAF). These classification criteria were based on a combination of features from three domains: a clinical domain consisting of extra-thoracic features; a serologic domain with specific autoantibodies; and a morphologic domain with imaging patterns, histopathological findings or multi-compartment involvement. Patients meeting IPAF criteria tend to have a history of smoking similar to patients with idiopathic pulmonary fibrosis. The most frequent clinical and serological markers of autoimmune features are Raynaud' phenomenon and positive antinuclear antibodies, respectively. Non-specific interstitial pneumonia is the predominant radiologic and histopathologic pattern, although patients meeting IPAF criteria through the clinical and serologic domains may also have a usual interstitial pneumonia pattern. Management should be carefully individualized on a case-by-case basis in keeping with the wide heterogeneity of IPAF and lack of evidence in this particular subgroup of patients. Prognosis is generally intermediate between that of idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease, but substantially variable according to the predominant histologic and radiologic patterns. As acknowledged by the Task Force, the proposed classification scheme of IPAF is a research concept that will need revision and refinement based on data to better inform prognostication and patient care. CI - Copyright © 2019 Fernandes, Nasser, Ahmad and Cottin. FAU - Fernandes, Ligia AU - Fernandes L AD - Departamento do Tórax, Centro Hospitalar Lisboa Norte, Lisbon, Portugal. FAU - Nasser, Mouhamad AU - Nasser M AD - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Lyon, France. FAU - Ahmad, Kais AU - Ahmad K AD - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Lyon, France. FAU - Cottin, Vincent AU - Cottin V AD - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Lyon, France. AD - Claude Bernard Lyon 1 University, University of Lyon, INRA, UMR754, Lyon, France. LA - eng PT - Journal Article PT - Review DEP - 20190927 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC6798044 OTO - NOTNLM OT - antibody OT - autoimmunity OT - classification OT - connective tissue disease OT - pulmonary fibrosis EDAT- 2019/11/05 06:00 MHDA- 2019/11/05 06:01 PMCR- 2019/09/27 CRDT- 2019/11/05 06:00 PHST- 2019/07/31 00:00 [received] PHST- 2019/09/09 00:00 [accepted] PHST- 2019/11/05 06:00 [entrez] PHST- 2019/11/05 06:00 [pubmed] PHST- 2019/11/05 06:01 [medline] PHST- 2019/09/27 00:00 [pmc-release] AID - 10.3389/fmed.2019.00209 [doi] PST - epublish SO - Front Med (Lausanne). 2019 Sep 27;6:209. doi: 10.3389/fmed.2019.00209. eCollection 2019. PMID- 20141231 OWN - NLM STAT- MEDLINE DCOM- 20100427 LR - 20100209 IS - 1175-0561 (Print) IS - 1175-0561 (Linking) VI - 11 IP - 2 DP - 2010 TI - The purple digit: an algorithmic approach to diagnosis. PG - 103-16 LID - 10.2165/11530180-000000000-00000 [doi] AB - The acute onset of purple digits is a concerning manifestation and may represent underlying, potentially life-threatening disease. Correctly identifying the etiology of purple digits is essential to proper management, and can aid in the diagnosis of systemic disease. Multiple causes of purple digits and significant overlap in clinical presentation can make diagnosis difficult. Despite the various causes of acute purple digits in the published literature, an algorithmic approach to the evaluation and management of the most common and alarming etiologies has yet to be established. The initial step in evaluating a patient with purple digits is to determine if the cause is associated with hypoxemia or trauma. If the patient is in a stable condition, the dermatologist needs to determine if the process could be related to cold exposure such as Raynaud phenomenon, acrocyanosis, pernio, cryoglobulinemia or frostbite. If the disease occurs independent of temperature, physical examination and histological evaluation of the skin is recommended. The lack of peripheral pulses are concerning for acute arterial thrombosis from peripheral vascular disease or arterial embolism. Non-blanching skin changes on the digit that lack inflammation and microthrombosis most likely represent a bleeding or platelet abnormality; however, if microthrombi are identified a more life-threatening processes such as purpura fulminans or embolic phenomenon may be occurring. Evidence of blood vessel inflammation suggests a leukocytoclastic vasculitis. The patient with a purple blanching digit and normal pulses requires an extensive historical review to help determine the cause. This review presents an algorithmic approach to assist in the evaluation and management of the purple digit. FAU - Brown, Patrick J AU - Brown PJ AD - Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15213, USA. FAU - Zirwas, Matthew J AU - Zirwas MJ FAU - English, Joseph C 3rd AU - English JC 3rd LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Am J Clin Dermatol JT - American journal of clinical dermatology JID - 100895290 SB - IM MH - Adult MH - Algorithms MH - Cold Temperature MH - Diagnosis, Differential MH - Fingers/blood supply/*pathology MH - Frostbite/diagnosis MH - Humans MH - Pulse MH - Toes/blood supply/*pathology MH - Vascular Diseases/*diagnosis/etiology RF - 155 EDAT- 2010/02/10 06:00 MHDA- 2010/04/28 06:00 CRDT- 2010/02/10 06:00 PHST- 2010/02/10 06:00 [entrez] PHST- 2010/02/10 06:00 [pubmed] PHST- 2010/04/28 06:00 [medline] AID - 4 [pii] AID - 10.2165/11530180-000000000-00000 [doi] PST - ppublish SO - Am J Clin Dermatol. 2010;11(2):103-16. doi: 10.2165/11530180-000000000-00000. PMID- 22517002 OWN - NLM STAT- MEDLINE DCOM- 20130628 LR - 20171116 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 44 IP - 2 DP - 2012 Apr 18 TI - [Analysis of clinical features and the outcome in 91 cases of mixed connective tissue diseases]. PG - 270-4 AB - OBJECTIVE: To investigate the clinical features and prognosis of mixed connective tissue disease (MCTD). METHODS: Clinical, laboratory and instrumental examination information of 91 patients with MCTD,who were diagnosed between 1990 to 2008 in Peking University People's Hospital, were collected and analyzed retrospectively. These patients were following-up, and different outcoms compared. RESULTS: The most common manifestations of MCTD patients were Raynaud phenomenon, arthralgia, arthritis, fever, acratia, positivities of antinuclear antibodies (anti-ANA) and ribosenuclear protein antibodies (anti-RNP), which were 94.5%,78%,46.2%,48.4%,53.9%,100% and 100%, respectively.Six patients died, and 22 patients were lost in the follow-up after discharge. Among the remaining 63 patients, 8 developed into systemic lupus erythomatosus (SLE), and 2 into antineutrophil cytoplasmic antibodies-associated vasculitis (AAV), 1 into primary Sjogren's syndrome (pSS), and 2 into rheumatoid arthritis (RA) at one to six years after diagnosis of MCTD. The patients who initially manifested as alopecia, proteinuria, thrombocytopenia, low complement were more likely to develop into SLE. CONCLUSION: MCTD can develop into various autoimmune diseases, such as SLE, pSS, RA, AAV. Some clinical features can probably predict future outcomes. FAU - Shi, Yu-hong AU - Shi YH AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Ru AU - Li R FAU - Chen, Shi AU - Chen S FAU - Su, Yin AU - Su Y FAU - Jia, Yuan AU - Jia Y LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Autoantibodies/immunology MH - Child MH - Disease Progression MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/*diagnosis/immunology MH - Prognosis MH - Retrospective Studies MH - Young Adult EDAT- 2012/04/21 06:00 MHDA- 2013/07/03 06:00 CRDT- 2012/04/21 06:00 PHST- 2012/04/21 06:00 [entrez] PHST- 2012/04/21 06:00 [pubmed] PHST- 2013/07/03 06:00 [medline] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2012 Apr 18;44(2):270-4. PMID- 33856650 OWN - NLM STAT- MEDLINE DCOM- 20211105 LR - 20251103 IS - 1880-344X (Electronic) IS - 1349-0222 (Linking) VI - 19 IP - 3 DP - 2021 Sep TI - An advanced ultrasound application used to assess peripheral vascular diseases: superb microvascular imaging. PG - 150-157 LID - 10.1007/s12574-021-00527-8 [doi] AB - Over the past several years, a novel ultrasound imaging modality termed superb microvascular imaging (SMI) has enabled visualization of microvessels. SMI ultrasound studies of peripheral artery diseases have significantly extended our knowledge of tissue microcirculation and the arterial microenvironments of atherosclerotic lesions. We here present an overview of current knowledge on the utility of SMI assessment of vascular diseases and highlight certain peripheral microcirculation disorders for which SMI is particularly valuable. The evidence indicates that SMI can detect intraplaque neovascularization and usefully assess carotid plaque vulnerability; vascularization of the carotid arterial wall detected by SMI is a potential marker of disease activity in patients with Takayasu arteritis; SMI reveals the foot microcirculation and yields a quantitative vascular index (in line with the angiosome concept); and, SMI may serve as an auxiliary diagnostic modality for hereditary hemorrhagic telangiectasia and Raynaud syndrome. In general, microcirculatory evaluation by SMI is an attractive field for future research on therapeutic strategies for peripheral vascular diseases. CI - © 2021. Japanese Society of Echocardiography. FAU - Sato, Wakana AU - Sato W AD - Department of Cardiovascular Medicine, Akita University Graduate School of Medicine, 1-1-1, Hondoh, Akita, 010-8543, Japan. FAU - Suto, Yuta AU - Suto Y AD - Department of Cardiovascular Medicine, Akita University Graduate School of Medicine, 1-1-1, Hondoh, Akita, 010-8543, Japan. FAU - Yamanaka, Takayuki AU - Yamanaka T AD - Department of Cardiovascular Medicine, Akita University Graduate School of Medicine, 1-1-1, Hondoh, Akita, 010-8543, Japan. FAU - Watanabe, Hiroyuki AU - Watanabe H AUID- ORCID: 0000-0001-8690-2416 AD - Department of Cardiovascular Medicine, Akita University Graduate School of Medicine, 1-1-1, Hondoh, Akita, 010-8543, Japan. hirow@doc.med.akita-u.ac.jp. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20210415 PL - Japan TA - J Echocardiogr JT - Journal of echocardiography JID - 101263153 SB - IM MH - Carotid Arteries/diagnostic imaging MH - Humans MH - Microcirculation MH - *Peripheral Vascular Diseases/diagnostic imaging MH - *Plaque, Atherosclerotic MH - Ultrasonography OTO - NOTNLM OT - Microcirculation OT - Peripheral vascular disease OT - Superb microvascular imaging OT - Ultrasound EDAT- 2021/04/16 06:00 MHDA- 2021/11/06 06:00 CRDT- 2021/04/15 12:23 PHST- 2021/03/02 00:00 [received] PHST- 2021/03/31 00:00 [accepted] PHST- 2021/03/02 00:00 [revised] PHST- 2021/04/16 06:00 [pubmed] PHST- 2021/11/06 06:00 [medline] PHST- 2021/04/15 12:23 [entrez] AID - 10.1007/s12574-021-00527-8 [pii] AID - 10.1007/s12574-021-00527-8 [doi] PST - ppublish SO - J Echocardiogr. 2021 Sep;19(3):150-157. doi: 10.1007/s12574-021-00527-8. Epub 2021 Apr 15. PMID- 16884489 OWN - NLM STAT- MEDLINE DCOM- 20060920 LR - 20060803 IS - 1742-464X (Print) IS - 1742-464X (Linking) VI - 273 IP - 15 DP - 2006 Aug TI - Implication of calpain in neuronal apoptosis. A possible regulation of Alzheimer's disease. PG - 3437-43 AB - Apoptotic neuronal cell death is the cardinal feature of aging and neurodegenerative diseases, but its mechanisms remain obscure. Caspases, members of the cysteine protease family, are known to be critical effectors in central nervous system cellular apoptosis. More recently, the calcium-dependent proteases, calpains, have been implicated in cellular apoptotic processes. Indeed, several members of the Bcl-2 family of cell death regulators, nuclear transcription factors (p53) and caspases themselves are processed by calpains. Progressive regional loss of neurons underlies the irreversible pathogenesis of various neurodegenerative diseases such as Alzheimer's disease in adult brain. Alzheimer's disease is characterized by extracellular plaques of amyloid-beta peptide aggregates and intracellular neurofibrillary tangles composed of hyperphosphorylated tau leading to apoptotic cell death. In this review, we summarize the arguments showing that calpains modulate processes that govern the function and metabolism of these two key proteins in the pathogenesis of Alzheimer's disease. To conclude, this article reviews our understanding of calpain-dependent apoptotic neuronal cell death and the ability of these proteases to regulate intracellular signaling pathways leading to chronic neurodegenerative disorders such as Alzheimer's disease. Further research on these calpain-dependent mechanisms which promote or prevent cell apoptosis should help us to develop new approaches for preventing and treating neurodegenerative disorders. FAU - Raynaud, F AU - Raynaud F AD - UMR5539, EPHE-CNRS-UM2, cc107, Université de Montpellier II, France. FAU - Marcilhac, A AU - Marcilhac A LA - eng PT - Journal Article PT - Review PL - England TA - FEBS J JT - The FEBS journal JID - 101229646 RN - 0 (Amyloid) RN - 0 (Proto-Oncogene Proteins c-bcl-2) RN - EC 3.4.22.- (Calpain) RN - EC 3.4.22.- (Caspases) SB - IM MH - Alzheimer Disease/*enzymology/pathology MH - Amyloid/biosynthesis MH - *Apoptosis MH - Brain/enzymology MH - Calpain/*physiology MH - Caspases/metabolism MH - Humans MH - Neurons/*enzymology/pathology MH - Proto-Oncogene Proteins c-bcl-2/metabolism RF - 47 EDAT- 2006/08/04 09:00 MHDA- 2006/09/21 09:00 CRDT- 2006/08/04 09:00 PHST- 2006/08/04 09:00 [pubmed] PHST- 2006/09/21 09:00 [medline] PHST- 2006/08/04 09:00 [entrez] AID - EJB5352 [pii] AID - 10.1111/j.1742-4658.2006.05352.x [doi] PST - ppublish SO - FEBS J. 2006 Aug;273(15):3437-43. doi: 10.1111/j.1742-4658.2006.05352.x. PMID- 23263716 OWN - NLM STAT- MEDLINE DCOM- 20130312 LR - 20250529 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 92 IP - 1 DP - 2013 Jan TI - The clinical phenotypes of the juvenile idiopathic inflammatory myopathies. PG - 25-41 LID - 10.1097/MD.0b013e31827f264d [doi] AB - The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features were shared between juvenile and adult IIM subgroups. However, JDM and JPM patients had a lower frequency of interstitial lung disease, Raynaud phenomenon, "mechanic's hands" and carpal tunnel syndrome, and lower mortality than their adult counterparts. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct clinical subgroups, defined by varying clinical and demographic characteristics, laboratory features, and outcomes. FAU - Shah, Mona AU - Shah M AD - From the Environmental Autoimmunity Group (MS, GM, FWM, LGR), Program of Clinical Research, National Institute of Environmental Health Sciences; Center for Information Technology (JDM), National Institutes of Health, DHHS, Bethesda, Maryland; Department of Epidemiology and Biostatistics (MS, MMR) and Division of Rheumatology, Department of Medicine(GM), George Washington University School of Medicine, Washington, DC; IWK Health Center and Dalhousie University (AMH), Halifax, Nova Scotia, Canada; and Veteran's Affairs Medical Center (INT), University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma. FAU - Mamyrova, Gulnara AU - Mamyrova G FAU - Targoff, Ira N AU - Targoff IN FAU - Huber, Adam M AU - Huber AM FAU - Malley, James D AU - Malley JD FAU - Rice, Madeline Murguia AU - Rice MM FAU - Miller, Frederick W AU - Miller FW FAU - Rider, Lisa G AU - Rider LG CN - with the Childhood Myositis Heterogeneity Collaborative Study Group LA - eng GR - R01 DK088114/DK/NIDDK NIH HHS/United States GR - Z01 ES101074/ImNIH/Intramural NIH HHS/United States GR - Z99 ES999999/ImNIH/Intramural NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, N.I.H., Intramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) SB - IM MH - Age of Onset MH - Autoantibodies/analysis MH - Chi-Square Distribution MH - Child MH - Female MH - Humans MH - Logistic Models MH - Male MH - Myositis/*classification/enzymology/epidemiology/immunology MH - Phenotype MH - Prognosis MH - Registries MH - Statistics, Nonparametric PMC - PMC4580479 MID - NIHMS721635 EDAT- 2012/12/25 06:00 MHDA- 2013/03/13 06:00 CRDT- 2012/12/25 06:00 PHST- 2012/12/25 06:00 [entrez] PHST- 2012/12/25 06:00 [pubmed] PHST- 2013/03/13 06:00 [medline] AID - 00005792-201301000-00004 [pii] AID - 10.1097/MD.0b013e31827f264d [doi] PST - ppublish SO - Medicine (Baltimore). 2013 Jan;92(1):25-41. doi: 10.1097/MD.0b013e31827f264d. PMID- 38314593 OWN - NLM STAT- MEDLINE DCOM- 20240724 LR - 20240724 IS - 1875-6360 (Electronic) IS - 1573-3971 (Linking) VI - 20 IP - 5 DP - 2024 TI - Mixed Connective Tissue Disease: The Two Cases Representing the Range of this Illness. PG - 569-573 LID - 10.2174/0115733971263972231124111042 [doi] AB - INTRODUCTION: Mixed connective tissue disease (MCTD) is defined as a systemic rheumatic disease characterized by the presence of high titer anti-U1 ribonucleoprotein (U1 RNP) antibodies in combination with clinical features commonly seen in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), rheumatoid arthritis (RA) and polymyositis (PM). CASE PRESENTATION: The annual incidence of MCTD is 1.9 per 100,000 adults. Any organ system can be involved in MCTD however four clinical features that suggest the presence of MCTD rather than another systemic rheumatic disease are Raynaud phenomenon with swollen hands or puffy fingers, absence of severe kidney disease and central nervous system (CNS) disease at first presentation generally, insidious onset of pulmonary hypertension and presence of autoantibodies anti-U1 ribonucleoprotein (U1 RNP), especially antibodies to the 68 kD protein. MCTD, although initially thought to be a disease with a benign course is not considered a valid argument at present. This connective tissue disorder can present with life-threating organ involvement with rapid progression of disease. CONCLUSION: We report two cases of MCTD, one with mild disease and another with life-threatening illness, describing the range of severity at presentation of this disorder. CI - Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net. FAU - Parrey, Ashaq Hussain AU - Parrey AH AUID- ORCID: 0000-0003-3766-1809 AD - Internal Medicine Government Medical College Srinagar, Srinagar, India. FAU - Koka, Manzoor AU - Koka M AD - Internal Medicine Government Medical College Srinagar, Srinagar, India. FAU - Ismail, Mohd AU - Ismail M AD - Internal Medicine Government Medical College Srinagar, Srinagar, India. LA - eng PT - Case Reports PT - Journal Article PL - United Arab Emirates TA - Curr Rheumatol Rev JT - Current rheumatology reviews JID - 101261938 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Mixed Connective Tissue Disease/complications MH - Female MH - Adult MH - Middle Aged MH - Male MH - Autoantibodies/blood/immunology OTO - NOTNLM OT - Mixed connective tissue disease OT - central nervous system. OT - interstitial lung disease OT - pulmonary arterial hypertension OT - systemic lupus erythematosus (SLE) OT - systemic sclerosis (SSc) EDAT- 2024/02/05 06:43 MHDA- 2024/07/24 06:42 CRDT- 2024/02/05 05:31 PHST- 2023/08/06 00:00 [received] PHST- 2023/09/12 00:00 [revised] PHST- 2023/09/20 00:00 [accepted] PHST- 2024/07/24 06:42 [medline] PHST- 2024/02/05 06:43 [pubmed] PHST- 2024/02/05 05:31 [entrez] AID - CRR-EPUB-137724 [pii] AID - 10.2174/0115733971263972231124111042 [doi] PST - ppublish SO - Curr Rheumatol Rev. 2024;20(5):569-573. doi: 10.2174/0115733971263972231124111042. PMID- 20065562 OWN - NLM STAT- MEDLINE DCOM- 20100316 LR - 20151119 IS - 1590-8577 (Electronic) IS - 1590-8577 (Linking) VI - 11 IP - 1 DP - 2010 Jan 8 TI - Secnidazole-induced acute pancreatitis: a new side-effect for an old drug? PG - 85-6 FAU - Slim, Raoudha AU - Slim R FAU - Ben Salem, Chaker AU - Ben Salem C FAU - Zamy, Michele AU - Zamy M FAU - Fathallah, Neila AU - Fathallah N FAU - Raynaud, Jean-Jacques AU - Raynaud JJ FAU - Bouraoui, Kamel AU - Bouraoui K FAU - Biour, Michel AU - Biour M LA - eng PT - Case Reports PT - Letter DEP - 20100108 PL - England TA - JOP JT - JOP : Journal of the pancreas JID - 101091810 RN - 0 (Anti-Infective Agents) RN - 140QMO216E (Metronidazole) RN - R3459K699K (secnidazole) SB - IM MH - Acute Disease MH - Anti-Infective Agents/adverse effects MH - Female MH - Humans MH - Metronidazole/adverse effects/*analogs & derivatives MH - Pancreatitis/*chemically induced MH - Young Adult EDAT- 2010/01/13 06:00 MHDA- 2010/03/17 06:00 CRDT- 2010/01/13 06:00 PHST- 2010/01/13 06:00 [entrez] PHST- 2010/01/13 06:00 [pubmed] PHST- 2010/03/17 06:00 [medline] AID - v11i01a19 [pii] PST - epublish SO - JOP. 2010 Jan 8;11(1):85-6. PMID- 29469721 OWN - NLM STAT- MEDLINE DCOM- 20180905 LR - 20180905 IS - 1087-2108 (Electronic) IS - 1087-2108 (Linking) VI - 23 IP - 9 DP - 2017 Sep 15 TI - Lupus and scleroderma overlap features in a 28-year-old man with anti-PL-12 anti-synthetase syndrome. LID - 13030/qt5vc6g7b6 [pii] AB - A 28-year-old man with clinically and laboratory diagnosed anti-PL-12 anti-synthetase syndrome (AS) in 2009 developed cutaneous lupus lesions, discoid lupus lesions, and sclerodacytly with finger-tip ulcerations four years following his AS diagnosis. Laboratory tests including +ANA, +anti-dsDNA antibody, +anti-Smith antibody, and +anti-RNP antibody in 2014 confirmed the diagnosis of progression to an overlap syndrome including systemic lupus erythematosus. The patient now also has clinical findings (sclerodacytly, Raynaud phenomenon, finger-tip ulcerations) consistent with scleroderma overlap. In each stage of his evolving connective tissue disease, cutaneous findings have been central to the recognition and monitoring of his overlap syndromes. FAU - Rana, Jasmine AU - Rana J AD - Department of Dermatology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts. jasmine_rana@hms.harvard.edu. FAU - Moy, Andrea Primiani AU - Moy AP FAU - Piris, Adriano AU - Piris A FAU - Smith, Gideon P AU - Smith GP LA - eng PT - Case Reports PT - Journal Article DEP - 20170915 PL - United States TA - Dermatol Online J JT - Dermatology online journal JID - 9610776 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Autoantibodies/*blood MH - Humans MH - Lupus Erythematosus, Systemic/blood/*complications MH - Male MH - Myositis/blood/*complications MH - Scleroderma, Localized/blood/*complications EDAT- 2018/02/23 06:00 MHDA- 2018/09/06 06:00 CRDT- 2018/02/23 06:00 PHST- 2017/09/20 00:00 [received] PHST- 2017/09/20 00:00 [accepted] PHST- 2018/02/23 06:00 [entrez] PHST- 2018/02/23 06:00 [pubmed] PHST- 2018/09/06 06:00 [medline] AID - 13030/qt5vc6g7b6 [pii] PST - epublish SO - Dermatol Online J. 2017 Sep 15;23(9):13030/qt5vc6g7b6. PMID- 41991397 OWN - NLM STAT- Publisher LR - 20260416 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) DP - 2026 Apr 15 TI - Physical rehabilitation interventions for hand function in people with systemic sclerosis. PG - 102122 LID - S1521-6942(26)00007-0 [pii] LID - 10.1016/j.berh.2026.102122 [doi] AB - Systemic sclerosis (SSc) is a complex systemic autoimmune rheumatic disease with marked clinical heterogeneity. Cutaneous manifestations affecting the hands and wrists include vascular insufficiency, early edema (puffy fingers), followed by progressive skin fibrosis and atrophy (sclerodactyly). This progressive skin tightening results in joint stiffness, deformity, functional impairment and reduced quality of life. Beyond skin changes, hand involvement may also include inflammatory arthritis, joint contractures, tendon friction rubs, Raynaud phenomenon, digital ulcers, acro-osteolysis, and calcinosis, all of which can further impair hand function, significantly affecting individuals' ability to perform routine occupational and daily tasks requiring grasping, gripping, and fine motor dexterity. In this article, we synthesize the evidence evaluating manual therapy, prescribed hand exercises, self-administered hand exercise protocols, telerehabilitation, paraffin wax, therapeutic ultrasound, manual lymphatic drainage, and dynamic splinting to improve hand function in people with SSc. CI - Copyright © 2026 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Denton, Michael AU - Denton M AD - Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada. Electronic address: Michael.Denton@sinaihealth.ca. FAU - Steiman, Amanda AU - Steiman A AD - Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, Toronto, Ontario, Canada. Electronic address: Amanda.Steiman@sinaihealth.ca. FAU - Johnson, Sindhu R AU - Johnson SR AD - Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Schroeder Arthritis Institute, Toronto Western Hospital, Mount Sinai Hospital, Institute of Health Policy, Management, and Evaluation, University of Toronto, Toronto, Ontario, Canada. Electronic address: Sindhu.Johnson@uhn.ca. LA - eng PT - Journal Article PT - Review DEP - 20260415 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM OTO - NOTNLM OT - Exercise OT - Hand therapy OT - Manual lymphatic drainage OT - Occupational therapy OT - Paraffin wax OT - Physical therapy OT - Rehabilitation OT - Scleroderma OT - Telerehabilitation COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/04/17 13:25 MHDA- 2026/04/17 13:25 CRDT- 2026/04/16 21:56 PHST- 2025/12/30 00:00 [received] PHST- 2026/01/31 00:00 [revised] PHST- 2026/02/12 00:00 [accepted] PHST- 2026/04/17 13:25 [medline] PHST- 2026/04/17 13:25 [pubmed] PHST- 2026/04/16 21:56 [entrez] AID - S1521-6942(26)00007-0 [pii] AID - 10.1016/j.berh.2026.102122 [doi] PST - aheadofprint SO - Best Pract Res Clin Rheumatol. 2026 Apr 15:102122. doi: 10.1016/j.berh.2026.102122. PMID- 26648774 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20151209 LR - 20200929 IS - 1426-3912 (Print) IS - 1644-4124 (Electronic) IS - 1426-3912 (Linking) VI - 40 IP - 3 DP - 2015 TI - Antiphospholipid antibodies in patients with upper-extremity deep vein thrombosis. PG - 307-10 LID - 10.5114/ceji.2015.54592 [doi] AB - The levels of antibodies to cardiolipin and β2-glycoprotein I and polymorphic variants G1691A of Factor V (factor V Leiden, FVL) and G20210A of prothrombin gene (G20210A) were studied in 16 patients with upper-extremity deep vein thrombosis (UEDVT). Most of patients with this syndrome have elevated values of these antibodies. Two of these patients are heterozygous carriers for G20210A and 1 - for FVL. Three patients with UEDVT and systemic lupus erythematosus (SLE) are positive for ANA and two others (one of them with Raynaud syndrome) have border line titre 1: 80 for ANA. All 3 patients with SLE are women and the interval between the development of the UEDVT and the onset of SLE was 1-4 years. We would like to suggest that: 1) UEDVT could be the first clinical symptom of Antiphospholipid syndrome, and 2) UEDVT may be the first clinical manifestation of SLE preceding the development of the systemic autoimmune disease by several years. FAU - Nikolova-Vlahova, Milena K AU - Nikolova-Vlahova MK AD - University Hospital Alexandrovska, Sofia, Bulgaria. FAU - Nikolov, Krasimir V AU - Nikolov KV AD - University Hospital Alexandrovska, Sofia, Bulgaria. FAU - Baleva, Marta P AU - Baleva MP AD - University Hospital Alexandrovska, Sofia, Bulgaria. FAU - Savov, Alexey S AU - Savov AS AD - National Genetic Laboratory, Medical University Sofia, Sofia, Bulgaria. LA - eng PT - Journal Article DEP - 20151015 PL - Poland TA - Cent Eur J Immunol JT - Central-European journal of immunology JID - 9702239 PMC - PMC4655380 OTO - NOTNLM OT - SLE OT - antiphospholipid antibodies OT - upper-extremity deep vein thrombosis EDAT- 2015/12/10 06:00 MHDA- 2015/12/10 06:01 PMCR- 2015/01/01 CRDT- 2015/12/10 06:00 PHST- 2015/03/06 00:00 [received] PHST- 2015/05/08 00:00 [accepted] PHST- 2015/12/10 06:00 [entrez] PHST- 2015/12/10 06:00 [pubmed] PHST- 2015/12/10 06:01 [medline] PHST- 2015/01/01 00:00 [pmc-release] AID - 25873 [pii] AID - 10.5114/ceji.2015.54592 [doi] PST - ppublish SO - Cent Eur J Immunol. 2015;40(3):307-10. doi: 10.5114/ceji.2015.54592. Epub 2015 Oct 15. PMID- 19735739 OWN - NLM STAT- MEDLINE DCOM- 20110608 LR - 20110117 IS - 1878-5875 (Electronic) IS - 1357-2725 (Linking) VI - 43 IP - 2 DP - 2011 Feb TI - Biliary differentiation and bile duct morphogenesis in development and disease. PG - 245-56 LID - 10.1016/j.biocel.2009.07.020 [doi] AB - The biliary tract consists of a network of intrahepatic and extrahepatic ducts that collect and drain the bile produced by hepatocytes to the gut. The bile ducts are lined by cholangiocytes, a specialized epithelial cell type that has a dual origin. Intrahepatic cholangiocytes derive from the liver precursor cells, whereas extrahepatic cholangiocytes are generated directly from the endoderm. In this review we discuss the mechanisms of cholangiocyte differentiation and bile duct morphogenesis, and describe how developing ducts interact with the hepatic artery. We also present an overview of the mechanisms of biliary dysgenesis in humans. CI - Copyright © 2009 Elsevier Ltd. All rights reserved. FAU - Raynaud, Peggy AU - Raynaud P AD - Université catholique de Louvain, de Duve Institute, Brussels, Belgium. FAU - Carpentier, Rodolphe AU - Carpentier R FAU - Antoniou, Aline AU - Antoniou A FAU - Lemaigre, Frédéric P AU - Lemaigre FP LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20090906 PL - Netherlands TA - Int J Biochem Cell Biol JT - The international journal of biochemistry & cell biology JID - 9508482 RN - 0 (MicroRNAs) SB - IM MH - Animals MH - *Bile Ducts/embryology/growth & development/pathology MH - Biliary Tract/embryology/growth & development MH - Biliary Tract Diseases/genetics/*pathology MH - *Cell Differentiation MH - Gene Expression Regulation, Developmental MH - Hepatocytes/cytology MH - Humans MH - *Liver/embryology/growth & development/pathology MH - Liver Diseases/genetics/*pathology MH - MicroRNAs/physiology MH - Morphogenesis MH - Signal Transduction EDAT- 2009/09/09 06:00 MHDA- 2011/06/09 06:00 CRDT- 2009/09/09 06:00 PHST- 2009/04/22 00:00 [received] PHST- 2009/07/08 00:00 [revised] PHST- 2009/07/08 00:00 [accepted] PHST- 2009/09/09 06:00 [entrez] PHST- 2009/09/09 06:00 [pubmed] PHST- 2011/06/09 06:00 [medline] AID - S1357-2725(09)00241-6 [pii] AID - 10.1016/j.biocel.2009.07.020 [doi] PST - ppublish SO - Int J Biochem Cell Biol. 2011 Feb;43(2):245-56. doi: 10.1016/j.biocel.2009.07.020. Epub 2009 Sep 6. PMID- 28722530 OWN - NLM STAT- MEDLINE DCOM- 20180904 LR - 20181202 IS - 1439-7609 (Electronic) IS - 1439-7595 (Linking) VI - 28 IP - 3 DP - 2018 May TI - Are there risk factors for scleroderma-related calcinosis? PG - 518-522 LID - 10.1080/14397595.2017.1349594 [doi] AB - OBJECTIVES: Pathogenesis and risk factors of scleroderma associated calcinosis is poorly understood and there is no effective treatment. This study was performed to better understand the prevalence and clinical features associated with calcinosis in a cohort of SSc outpatients. METHODS: In this cross-sectional study, we compared clinical characteristics of SSc patients with (SSc-calcinosis) and without calcinosis (SSc-control) seen in the outpatient Rutgers-RWJ Scleroderma Program between 2012 and 2015. Our analysis included clinical characteristics, comorbidities (i.e. osteoporosis), autoantibodies and imaging results. We performed univariable and multivariable regression analyses to determine common factors associated with calcinosis. RESULTS: There were 215 SSc patients, including 65 SSc-calcinosis (81.5% females) and 150 SSc-without calcinosis (controls, 77% females). SSc-calcinosis patients were older (p = .026) with significantly longer disease duration (20 ± 10.5 years versus 12 ± 8 years, p < .0001). Limited cutaneous scleroderma was more common (54%) in the calcinosis group. Longer disease duration from non-Raynaud symptoms (OR 3.77; 95%CI 1.26, 11.27) and osteoporosis (OR 3.43; 95%CI 1.15, 10.36) remained independently associated with calcinosis. CONCLUSIONS: Calcinosis was common in both limited and diffuse systemic sclerosis; both longer disease duration and osteoporosis were independently associated with calcinosis. Prospective studies are needed to confirm our findings. FAU - Pai, Sneha AU - Pai S AD - a Institute for Rheumatic and Autoimmune Diseases, Overlook Hospital and Medical Center , Summit , NJ , USA. FAU - Hsu, Vivien AU - Hsu V AD - b Department of Medicine, Division of Rheumatology and Connective Tissue Research , Rutgers-Robert Wood Johnson Medical School , New Brunswick , NJ , USA. LA - eng PT - Journal Article DEP - 20170719 PL - England TA - Mod Rheumatol JT - Modern rheumatology JID - 100959226 SB - IM MH - Aged MH - Calcinosis/*epidemiology/etiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Prevalence MH - Risk Factors MH - Scleroderma, Systemic/*complications OTO - NOTNLM OT - Calcinosis OT - risk factors OT - systemic sclerosis EDAT- 2017/07/20 06:00 MHDA- 2018/09/05 06:00 CRDT- 2017/07/20 06:00 PHST- 2017/07/20 06:00 [pubmed] PHST- 2018/09/05 06:00 [medline] PHST- 2017/07/20 06:00 [entrez] AID - 10.1080/14397595.2017.1349594 [doi] PST - ppublish SO - Mod Rheumatol. 2018 May;28(3):518-522. doi: 10.1080/14397595.2017.1349594. Epub 2017 Jul 19. PMID- 25051133 OWN - NLM STAT- MEDLINE DCOM- 20140821 LR - 20220223 IS - 1530-6550 (Print) IS - 1530-6550 (Linking) VI - 15 IP - 2 DP - 2014 TI - Therapeutic potentials of phosphodiesterase-5 inhibitors in cardiovascular disease. PG - 158-67 LID - 10.3909/ricm0679 [doi] AB - Phosphodiesterase-5 (PDE5) inhibitors have been approved by the US Food and Drug Administration for the treatment of erectile dysfunction and more recently for pulmonary arterial hypertension (World Health Organization functional class I). PDE5 inhibitors can induce vasodilation; in addition, through a complex pathway involving nitric oxide, cyclic guanosine monophosphate, and protein kinase G, it can reduce apoptosis and suppress cell proliferation. The presence of PDE5 inhibitors in various tissues and systemic vasculature make them potential targets in a variety of cardiovascular diseases. In many in vitro and in vivo studies, PDE5 inhibitors have been shown to have positive effects in systolic and diastolic congestive heart failure, ischemic heart disease, doxorubicin cardiomyopathy, and pulmonary arterial hypertension. They also improved vasoconstriction in Raynaud phenomenon, peripheral artery disease, and hypoxic brain conditions. This article reviews the therapeutic potentials of PDE5 inhibitors in different cardiovascular diseases. FAU - Nguyen, Ha AU - Nguyen H FAU - Amanullah, Aman M AU - Amanullah AM AD - Department of Medicine, Einstein Medical Center, Philadelphia, PA; Department of Medicine and Einstein Institute for Heart and Vascular Health, Einstein Medical Center, and Jefferson College of Medicine, Philadelphia, PA. LA - eng PT - Journal Article PT - Review PL - Singapore TA - Rev Cardiovasc Med JT - Reviews in cardiovascular medicine JID - 100960007 RN - 0 (Cardiovascular Agents) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - EC 3.1.4.35 (Cyclic Nucleotide Phosphodiesterases, Type 5) SB - IM MH - Animals MH - Cardiovascular Agents/*therapeutic use MH - Cardiovascular Diseases/*drug therapy/enzymology MH - Cyclic Nucleotide Phosphodiesterases, Type 5/*metabolism MH - Humans MH - Phosphodiesterase 5 Inhibitors/*therapeutic use MH - Signal Transduction/drug effects MH - Treatment Outcome EDAT- 2014/07/23 06:00 MHDA- 2014/08/22 06:00 CRDT- 2014/07/23 06:00 PHST- 2014/07/23 06:00 [entrez] PHST- 2014/07/23 06:00 [pubmed] PHST- 2014/08/22 06:00 [medline] AID - 10.3909/ricm0679 [doi] PST - ppublish SO - Rev Cardiovasc Med. 2014;15(2):158-67. doi: 10.3909/ricm0679. PMID- 24767252 OWN - NLM STAT- MEDLINE DCOM- 20140729 LR - 20140523 IS - 1532-8392 (Electronic) IS - 0046-8177 (Linking) VI - 45 IP - 6 DP - 2014 Jun TI - The pulmonary histopathologic manifestations of the anti-PL7/antithreonyl transfer RNA synthetase syndrome. PG - 1199-204 LID - S0046-8177(14)00054-9 [pii] LID - 10.1016/j.humpath.2014.01.018 [doi] AB - The pulmonary histopathologic manifestations of the antisynthetase syndromes is poorly understood and reported. Eight cases of interstitial lung disease related to the presence of antitheonyl (PL7) transfer RNA synthetase autoantibodies along with a review of biopsy changes reported in the English literature are described. Most patients presented with dyspnea and cough, with myositis, skin changes including "mechanic's hands," and Raynaud phenomenon. Biopsy patterns of injury included usual interstitial pneumonia (n = 4), organizing pneumonia (n = 2), nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia (n = 1 each), which, in a minority of instances, contrasted with predictions by thoracic radiologists based on high-resolution computed tomographic scans. This study emphasizes the integrated clinical, radiologic, and pathologic approach to interstitial lung disease, especially in connective tissue disorders, and points out the occurrence of usual interstitial pneumonia and organizing pneumonia in this patient group where nonspecific interstitial pneumonia has been the dominant pattern of clinical interest. CI - Copyright © 2014 Elsevier Inc. All rights reserved. FAU - Yousem, Samuel A AU - Yousem SA AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. Electronic address: yousemsa@upmc.edu. FAU - Schneider, Frank AU - Schneider F AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. FAU - Bi, David AU - Bi D AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. FAU - Oddis, Chester V AU - Oddis CV AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. FAU - Gibson, Kevin AU - Gibson K AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. FAU - Aggarwal, Rohit AU - Aggarwal R AD - Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582. LA - eng PT - Journal Article DEP - 20140206 PL - United States TA - Hum Pathol JT - Human pathology JID - 9421547 RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Amino Acyl-tRNA Synthetases/*immunology MH - Female MH - Humans MH - Lung Diseases, Interstitial/etiology/*pathology MH - Male MH - Middle Aged MH - Myositis/complications/*pathology OTO - NOTNLM OT - Antisynthetase syndrome OT - Autoimmune OT - Lung OT - PL7 EDAT- 2014/04/29 06:00 MHDA- 2014/07/30 06:00 CRDT- 2014/04/29 06:00 PHST- 2013/10/29 00:00 [received] PHST- 2014/01/15 00:00 [revised] PHST- 2014/01/24 00:00 [accepted] PHST- 2014/04/29 06:00 [entrez] PHST- 2014/04/29 06:00 [pubmed] PHST- 2014/07/30 06:00 [medline] AID - S0046-8177(14)00054-9 [pii] AID - 10.1016/j.humpath.2014.01.018 [doi] PST - ppublish SO - Hum Pathol. 2014 Jun;45(6):1199-204. doi: 10.1016/j.humpath.2014.01.018. Epub 2014 Feb 6. PMID- 40459886 OWN - NLM STAT- MEDLINE DCOM- 20251003 LR - 20260217 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) VI - 64 IP - 10 DP - 2025 Oct 1 TI - Long-term effects of selexipag in SSc-associated digital ulcers: a case-control multicentre observational study. PG - 5364-5371 LID - 10.1093/rheumatology/keaf292 [doi] AB - OBJECTIVES: Digital ulcers (DUs) affect ∼50% of SSc patients, causing significant pain and functional impairment. Current management involves both systemic and local therapies. However, the burden in terms of pain and quality of life due to refractory DUs still remains heavy. While selexipag is approved for SSc-associated pulmonary arterial hypertension, its potential in treating DUs is unexplored. We aimed to evaluate the long-term efficacy of selexipag compared with iloprost in treating DUs. METHODS: In this multicentre case-control study, we retrospectively evaluated 96 SSc patients with refractory DUs (32 treated with selexipag, 64 with iloprost), matched for gender, disease subset and age. DU number, ischaemic pain and RP severity were assessed at baseline, 6, 12 and 24 months. Pain and RP were evaluated using the Likert Pain Scale (LPS) and Raynaud Condition Score (RCS), respectively. Additionally, DUs recurrence and new onset were recorded. Healing rates were estimated using Kaplan-Meier analysis. RESULTS: Selexipag showed higher efficacy with 87% of DUs healing rate vs 28% for iloprost at 96 weeks (P < 0.001). DUs number, RCS and LPS showed significant improvement in selexipag-treated patients compared with iloprost (P < 0.001 for all) throughout 24 months. Selexipag-treated patients achieved faster healing (75% by week 40) and maintained significantly lower relapse rates (5% vs 45% at 24 months, P < 0.001). New DUs formation remained consistently lower in the selexipag group compared with the iloprost group (5% vs 40% at 24 months, P < 0.001). CONCLUSIONS: This observational study suggests that selexipag may be strongly effective in treating DUs refractory to conventional drugs. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. FAU - Iannone, Claudia AU - Iannone C AUID- ORCID: 0009-0005-5686-0127 AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Di Battista, Marco AU - Di Battista M AUID- ORCID: 0000-0002-4788-5729 AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Pellico, Maria Rosa AU - Pellico MR AUID- ORCID: 0009-0004-1921-7370 AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Magi, Ilaria AU - Magi I AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Minniti, Antonina AU - Minniti A AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. FAU - Armentaro, Giuseppe AU - Armentaro G AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. FAU - Cavalli, Silvia AU - Cavalli S AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Sette, Manuel AU - Sette M AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Giudice, Laura AU - Giudice L AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Bochicchio, Cristina AU - Bochicchio C AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Tavoni, Antonio G AU - Tavoni AG AD - Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AUID- ORCID: 0000-0001-7479-4462 AD - Department of Medicine and Surgery, LUM University "Giuseppe De Gennaro" Casamassima & Rheumatology Service "Miulli" General Hospital Acquaviva delle Fonti, Bari, Italy. AD - Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari, Bari, Italy. FAU - Stano, Stefano AU - Stano S AD - Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari, Bari, Italy. FAU - Orlandi, Martina AU - Orlandi M AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Giuggioli, Dilia AU - Giuggioli D AUID- ORCID: 0000-0002-0041-3695 AD - Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Modena, Italy. FAU - Mosca, Marta AU - Mosca M AD - Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. FAU - Caporali, Roberto AU - Caporali R AUID- ORCID: 0000-0003-3487-8551 AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AD - Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO Institute, Milan, Italy. AD - Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milano, Italy. LA - eng PT - Journal Article PT - Multicenter Study PT - Observational Study PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 5EXC0E384L (selexipag) RN - JED5K35YGL (Iloprost) RN - 0 (Acetamides) RN - 0 (Pyrazines) RN - 0 (Vasodilator Agents) RN - digital ulcers SB - IM CIN - Rheumatology (Oxford). 2026 Feb 4;65(2):keag068. doi: 10.1093/rheumatology/keag068. PMID: 41649467 MH - Humans MH - Male MH - Female MH - Middle Aged MH - *Skin Ulcer/drug therapy/etiology MH - Retrospective Studies MH - Case-Control Studies MH - *Scleroderma, Systemic/complications MH - Iloprost/therapeutic use MH - *Acetamides/therapeutic use MH - *Pyrazines/therapeutic use MH - Aged MH - Treatment Outcome MH - Vasodilator Agents/therapeutic use MH - Fingers MH - Adult OTO - NOTNLM OT - Raynaud condition score OT - SSc OT - digital ulcers OT - iloprost OT - relapse OT - selexipag OT - vasoactive therapy EDAT- 2025/06/03 12:33 MHDA- 2025/10/03 18:28 CRDT- 2025/06/03 11:33 PHST- 2025/02/19 00:00 [received] PHST- 2025/05/13 00:00 [accepted] PHST- 2025/10/03 18:28 [medline] PHST- 2025/06/03 12:33 [pubmed] PHST- 2025/06/03 11:33 [entrez] AID - 8155856 [pii] AID - 10.1093/rheumatology/keaf292 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Oct 1;64(10):5364-5371. doi: 10.1093/rheumatology/keaf292. PMID- 41782907 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260305 LR - 20260305 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 18 DP - 2026 TI - The clinical phenotype of anti-Th/To+ patients in systemic sclerosis: a case-control study within the European Scleroderma Trials and Research cohort. PG - 1759720X261422369 LID - 10.1177/1759720X261422369 [doi] LID - 1759720X261422369 AB - BACKGROUND: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease, where autoantibody profiling plays a central role in defining disease subsets and guiding personalized management. OBJECTIVES: To investigate the clinical phenotype and long-term outcomes of anti-Th/To positive SSc patients in an international cohort, focusing on interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), malignancy association, organ damage accrual, and mortality within a precision medicine framework. DESIGN: Multicenter case-control study. METHODS: Data prospectively collected from 28 European Scleroderma Trial and Research centers were analyzed (CP144). For each anti-Th/To+ case, two anti-Th/To- controls were matched by sex, age at onset, and disease duration to enable detailed phenotypic comparisons. RESULTS: A total of 102 anti-Th/To+ patients were compared to 204 anti-Th/To- matched controls. Anti-Th/To+ patients had a higher prevalence of concomitant anti-Ro52+, lower frequency of diffuse cutaneous involvement, digital ulcers, pitting scars, and telangiectasias. ILD on high-resolution computed tomography and ILD functional progression events were less frequent in anti-Th/To+ patients, and anti-Th/To positivity was not independently associated with ILD in multivariable analysis. Instead, ILD presence was significantly associated with anti-Topoisomerase-1 (anti-Topo1) and anti-Ro52 positivity, and lack of anticentromere antibodies. Similarly, myocarditis was less frequently observed in anti-Th/To+ cases, although myositis had a higher rate than in anti-centromere+ or other lcSSc patients. Other SSc manifestations, including PAH, and malignancies synchronous to SSc onset had similar frequencies between cases and controls. Anti-Th/To+ patients accrued mild organ damage during the disease course, with lower damage index scores than anti-Topo1+ matched controls. No SSc-related deaths occurred in anti-Th/To+ patients, who had survival curves slightly better, although not significantly different, than matched controls. CONCLUSION: Anti-Th/To+ SSc patients are characterized by low prevalence of major organ involvement, including ILD, when compared to matched controls, mild organ damage, and good survival. These results reinforce the ongoing use of autoantibody profiling-including rarer antibodies-in precision medicine for SSc. CI - © The Author(s), 2026. FAU - Moschetti, Liala AU - Moschetti L AUID- ORCID: 0000-0003-4976-9550 AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Piazzale Spedali Civili 1, Brescia 25123, Italy. FAU - Rovaris, Silvia AU - Rovaris S AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Bonomi, Francesco AU - Bonomi F AUID- ORCID: 0000-0001-7744-8549 AD - Division of Rheumatology, Scleroderma Unit, Department of Experimental and Clinical Medicine, AOU Careggi Firenze, University of Florence, AOU Careggi Firenze, Florence, Italy. FAU - Codes-Mendez, Helena AU - Codes-Mendez H AD - Autoimmune Diseases Functional Unit, Department of Rheumatology and Systemic Autoimmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau and Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Martín-López, María AU - Martín-López M AUID- ORCID: 0000-0002-4218-5731 AD - Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AUID- ORCID: 0000-0001-7479-4462 AD - Rheumatology Unit, DiMePReJ, School of Medicine, University of Bari, Bari, Italy. AD - Rheumatology Service, "Miulli" General Hospital-Department of Medicine and Surgery, LUM University, Casamassima, Bari, Italy. FAU - Tonutti, Antonio AU - Tonutti A AUID- ORCID: 0009-0000-9534-6853 AD - Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy. AD - Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy. FAU - Galdo, Francesco Del AU - Galdo FD AD - Leeds Raynaud's and Scleroderma Program, NIHR Biomedical Research Centre, Leeds, UK. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases, San Raffaele Hospital, Vita-Salute San Raffaele University, Milano, Italy. FAU - Truchetet, Marie Elise AU - Truchetet ME AD - Rheumatology Department, CHU de Bordeaux, Bordeaux, France. FAU - Allanore, Yannick AU - Allanore Y AD - Rheumatology Department, Cochin Hospital of Paris, Université Paris Cité, Paris, France. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Nippon Medical School, Tokyo, Japan. FAU - Bruni, Cosimo AU - Bruni C AUID- ORCID: 0000-0003-2813-2083 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Santos, Cristiana Sieiro AU - Santos CS AUID- ORCID: 0000-0003-0889-9877 AD - Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain. FAU - Moroncini, Gianluca AU - Moroncini G AD - Clinica Medica, Azienda Ospedaliero Universitaria delle Marche, Università Politecnica delle Marche, Ancona, Italy. FAU - Caetano, Joana AU - Caetano J AUID- ORCID: 0000-0001-5278-5397 AD - Systemic Autoimmune Diseases Unit, Fernando Fonseca Hospital, Amadora, Portugal. FAU - Granel, Brigitte AU - Granel B AUID- ORCID: 0000-0001-7995-8817 AD - Internal Medicine Department, Hôpital Nord, Assistance Publique des Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France. FAU - Groseanu, Laura AU - Groseanu L AUID- ORCID: 0000-0002-4175-1199 AD - Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Pedretti, Eleonora AU - Pedretti E AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Colombo, Enrico AU - Colombo E AUID- ORCID: 0000-0001-6744-582X AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Guerini, Miriam AU - Guerini M AD - Unit of Rheumatology, Department of Internal Medicine and Therapeutics, University of Pavia and IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy. FAU - Pereira Silva, Mariana AU - Pereira Silva M AUID- ORCID: 0000-0002-4670-2329 AD - Rheumatology Department, Faculdade de Medicina, Universidade de Lisboa and ULS Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal. FAU - Bearzi, Pietro AU - Bearzi P AUID- ORCID: 0000-0002-7621-1192 AD - Rheumatology and Clinical Immunology, Department of Medicine, School of Medicine, University of Rome "Campus Bio-Medico," Rome, Italy. FAU - Belloli, Laura AU - Belloli L AD - Rheumatology Unit, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy. FAU - Alegre-Sancho, Juan Jose AU - Alegre-Sancho JJ AUID- ORCID: 0000-0003-1641-0875 AD - Department of Rheumatology, Doctor Peset University Hospital, Valencia, Spain. FAU - Maglio, Cristina AU - Maglio C AUID- ORCID: 0000-0002-3786-5767 AD - Department of Rheumatology and Inflammation Research, University of Gothenburg, Gothenburg, Sweden. FAU - Cuomo, Giovanna AU - Cuomo G AD - Department of Precision of Medicine, University of Campania-L. Vanvitelli, Naples, Italy. FAU - Parvu, Magda AU - Parvu M AD - Rheumatology Department, Colentina Clinical Hospital, Bucharest, Romania. FAU - Poormoghim, Hadi AU - Poormoghim H AD - Rheumatology Unit, Firoozgar Hospital, University of Medical Sciences, Tehran, Iran. FAU - Idolazzi, Luca AU - Idolazzi L AUID- ORCID: 0000-0002-7254-4686 AD - Section of Rheumatology, Department of Medicine, University of Verona, Verona, Italy. FAU - Andréasson, Kristofer AU - Andréasson K AD - Section of Rheumatology, Department of Clinical Sciences, Lund University, Lund, Sweden. FAU - De Santis, Maria AU - De Santis M AUID- ORCID: 0000-0002-3196-1336 AD - Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy. AD - Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy. FAU - Iannone, Florenzo AU - Iannone F AUID- ORCID: 0000-0003-0474-5344 AD - Rheumatology Unit, DiMePReJ, School of Medicine, University of Bari, Bari, Italy. FAU - Carreira, Patricia Esmeralda AU - Carreira PE AD - Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Castellví, Ivan AU - Castellví I AUID- ORCID: 0000-0002-5410-5807 AD - Autoimmune Diseases Functional Unit, Department of Rheumatology and Systemic Autoimmune Diseases, Hospital Universitari de la Santa Creu i Sant Pau and Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Guiducci, Serena AU - Guiducci S AD - Division of Rheumatology, Scleroderma Unit, Department of Experimental and Clinical Medicine, AOU Careggi Firenze, University of Florence, AOU Careggi Firenze, Florence, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Franceschini, Franco AU - Franceschini F AUID- ORCID: 0000-0003-3678-6124 AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Airò, Paolo AU - Airò P AUID- ORCID: 0000-0001-5241-1918 AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AUID- ORCID: 0000-0002-1860-6866 AD - Scleroderma Unit, Rheumatology Unit, ERN ReCONNET Centre, ASST Spedali Civili and University of Brescia, Brescia, Italy. LA - eng PT - Journal Article DEP - 20260302 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC12953999 OAB - Understanding the features of systemic sclerosis patients with anti-Th/To antibodies: a study comparing patients within the European EUSTAR network Why was the study done? Systemic sclerosis (SSc) is a complex autoimmune disease that affects the skin and internal organs. Doctors use specific antibodies detected in the blood to help predict how the disease might progress. However, some rare antibodies, like anti-Th/To, are not well understood. This study was done to better understand what anti-Th/To antibodies mean for patients with SSc, especially regarding lung and heart complications, cancer risk, and overall health outcomes. What did the researchers do? The researchers collected and analyzed medical data from a large group of patients with systemic sclerosis across multiple centers in Europe. They compared patients who had anti-Th/To antibodies with similar patients who did not have these antibodies, looking closely at differences in lung disease, heart complications, cancer occurrence, organ damage, and survival over time. What did the researchers find? They found that patients with anti-Th/To antibodies had less severe lung disease and fewer major organ complications compared to those without these antibodies. These patients also showed milder organ damage over time and had better overall survival rates. Some differences in other disease features were noted, but the presence of these antibodies was linked to a generally more favorable disease course. What do the findings mean? The findings show that patients with anti-Th/To antibodies tend to have less severe organ involvement, especially less lung disease, and generally better health outcomes compared to similar patients without these antibodies. This information helps doctors better understand and predict disease progression in systemic sclerosis, supporting more personalized care based on patients’ specific antibody profiles. OABL- eng OTO - NOTNLM OT - anti-Th/To antibodies OT - clinical phenotype OT - precision medicine OT - systemic sclerosis COIS- M.-E.T. received honorarium in the field of systemic sclerosis from Abbvie, Astra-Zeneca, Boehringer Ingelheim, Novartis. Y.A. received honorarium or research grants in the field of systemic sclerosis from Abbvie, Alpine ImmunoSciences, Argenx, Astra-Zeneca, Boehringer Ingelheim, Corvus, Merck Serono, Topadur. M.K. received honorarium or research grants in the field of systemic sclerosis from Abbvie, Argenx, AstraZeneca, Boehringer Ingelheim, Chugai, GSK, and MBL, Novartis. C.B. consultant of Boehringer Ingelheim, Grant/research support from: Novartis Foundation for medical–biological research, EMDO Foundation, Iten-Kohaut Foundation, Kurt und Senta Hermann Foundation, Jubileum Foundation of Swisslife. Educational grants from Wellcome Trust. Congress support from Boehringer-Ingelheim, Hartmann-Muller Foundation. L.G. has/had consultancy relationship with and/or has served as a speaker for the following companies: Astra Zeneca, Boehringer Ingelheim, Exopharma, Abbvie, Pfizer, Sandoz, Zentiva, Eli Lilly, Novartis, Stada, UCB, Jansenn. J.J.A.S. received honorarium or research grants in the field of systemic sclerosis from AstraZeneca, Boehringer Ingelheim, Novartis, Johnson&Johnson. P.E.C. has/had consultancy relationship with and/or has served as a speaker for the following companies: Astra-Zeneca, Boehringer Ingelheim, Corbus, Emerald Health Pharmaceuticals, Janssen, Mitsubishi Tanabe, Novartis, Prometheus. I.C. consultant of Boehringer Ingelheim, GSK, Innovaderm, Novartis. Speaker: Boehringer Ingelheim, Johnson&Johnson. M.G.L. speaker: Boehringer Ingelheim, consultant: UCB, Novartis, Boehringer Ingelheim, grant research: Boehringer Ingelheim. EDAT- 2026/03/05 07:09 MHDA- 2026/03/05 07:10 PMCR- 2026/03/02 CRDT- 2026/03/05 04:47 PHST- 2025/09/23 00:00 [received] PHST- 2026/01/23 00:00 [accepted] PHST- 2026/03/05 07:10 [medline] PHST- 2026/03/05 07:09 [pubmed] PHST- 2026/03/05 04:47 [entrez] PHST- 2026/03/02 00:00 [pmc-release] AID - 10.1177_1759720X261422369 [pii] AID - 10.1177/1759720X261422369 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2026 Mar 2;18:1759720X261422369. doi: 10.1177/1759720X261422369. eCollection 2026. PMID- 31020194 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220408 IS - 2514-2119 (Electronic) IS - 2514-2119 (Linking) VI - 2 IP - 4 DP - 2018 Dec TI - A case report of an unusual cause of mitral stenosis in a young woman. PG - yty118 LID - 10.1093/ehjcr/yty118 [doi] LID - yty118 AB - BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease, frequently associated with cardiovascular involvement. One of the most frequent complication is mitral valve regurgitation in more than one-third of the patients. CASE SUMMARY: A 30-year-old woman with arthralgia, butterfly rash, and Raynaud phenomenon presented with a systolic murmur and renal impairment. Based on the kidney biopsy the diagnosis of SLE was made. Echocardiography revealed the presence of pulmonary hypertension, restrictive mitral valve disease with nodular thickening of the anterior leaflet and moderate regurgitation, compatible with Libman Sacks (LS) endocarditis. Immunosuppressive therapy was started and the patient status improved with normalization of systolic pulmonary artery pressure. After 8 years without follow-up, she presented with fatigue and dyspnoea based on a severe mitral valve stenosis. Subsequently, she underwent a minimal invasive mitral valve replacement and the diagnosis of LS endocarditis could be confirmed upon histopathological examination. DISCUSSION: This case demonstrates that LS endocarditis can not only lead to mitral regurgitation but occasionally to mitral stenosis due to chronic inflammation with thickening and fusion of mitral valve leaflets. Hereby, comprehensive echocardiography, inclusive stress echocardiography, plays a critical role. FAU - Senesael, Ellie AU - Senesael E AD - Department of Cardiology, University Hospital of Ghent, C.Heymanslaan 10, Ghent, Belgium. FAU - Plein, Danièle AU - Plein D AD - Department of Cardiology, Centre for Cardiovascular Diseases, University Hospital of Brussels, Laarbeeklaan 101, Jette, Belgium. FAU - La Meir, Mark AU - La Meir M AD - Cardiac Surgery, Centre for Cardiovascular Diseases, University Hospital of Brussels, Laarbeeklaan 101, Jette, Brussels, Belgium. FAU - Droogmans, Steven AU - Droogmans S AD - Department of Cardiology, Centre for Cardiovascular Diseases, University Hospital of Brussels, Laarbeeklaan 101, Jette, Belgium. LA - eng PT - Case Reports PT - Journal Article DEP - 20181030 PL - England TA - Eur Heart J Case Rep JT - European heart journal. Case reports JID - 101730741 PMC - PMC6426048 OTO - NOTNLM OT - Case report OT - Libman Sacks endocarditis OT - Mitral stenosis OT - Stress echocardiography OT - Systemic lupus erythematosus EDAT- 2019/04/26 06:00 MHDA- 2019/04/26 06:01 PMCR- 2018/10/30 CRDT- 2019/04/26 06:00 PHST- 2018/03/01 00:00 [received] PHST- 2018/10/07 00:00 [accepted] PHST- 2019/04/26 06:00 [entrez] PHST- 2019/04/26 06:00 [pubmed] PHST- 2019/04/26 06:01 [medline] PHST- 2018/10/30 00:00 [pmc-release] AID - yty118 [pii] AID - 10.1093/ehjcr/yty118 [doi] PST - epublish SO - Eur Heart J Case Rep. 2018 Oct 30;2(4):yty118. doi: 10.1093/ehjcr/yty118. eCollection 2018 Dec. PMID- 29173135 OWN - NLM STAT- MEDLINE DCOM- 20190104 LR - 20190104 IS - 2295-3337 (Electronic) IS - 1784-3286 (Linking) VI - 73 IP - 5 DP - 2018 Oct TI - A 64-year-old woman with interstitial lung disease and positive antibodies against aminoacyl-transfer RNA synthetases in the absence of myositis: presentation of an anti-PL-12 positive antisynthetase syndrome. PG - 389-392 LID - 10.1080/17843286.2017.1403133 [doi] AB - Introduction The antisynthetase syndrome is a rare autoimmune disease described by the presence of inflammatory myositis, interstitial lung disease and antibodies against aminoacyl-transfer RNA synthetases. Interstitial lung disease can be the only manifestation in the absence of an inflammatory myositis. Other clinical signs are Raynaud phenomenon, hyperkeratotic skin lesions, fever and inflammatory polyarthritis. Case presentation We report the case of a 64-year old woman who complained of a dry cough, progressive dyspnea and arthralgia since 2 years, with no other systemic symptoms. High resolution computed tomography (HRCT) of the thorax showed the presence of bilateral ground glass opacities, reticular opacities and some traction bronchiectasis. Further biochemical testing revealed the presence of anti-PL12 antibodies. Management The diagnosis of antisynthetase syndrome was made and the patient was treated with steroids and azathioprine with a good response. Conclusion The search for antisynthetase antibodies should always be considered in patients with an interstitial lung disease without any other clinical symptoms or signs of an underlying connective tissue disease. FAU - Ghysen, Katrien AU - Ghysen K AD - a Department of Pneumology , AZ Groeninge , Kortrijk , Belgium. FAU - Leys, Mathias AU - Leys M AD - a Department of Pneumology , AZ Groeninge , Kortrijk , Belgium. LA - eng PT - Case Reports PT - Journal Article DEP - 20171127 PL - England TA - Acta Clin Belg JT - Acta clinica Belgica JID - 0370306 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/blood MH - Female MH - Humans MH - Lung/diagnostic imaging/pathology MH - *Lung Diseases, Interstitial MH - Middle Aged MH - *Myositis MH - Radiography, Thoracic OTO - NOTNLM OT - Interstitial lung disease OT - anti-PL12 OT - anti-aminoacyl-tRNA synthetase autoantibodies OT - antisynthetase syndrome OT - interstitial pulmonary disease with auto-immune features (IPAF) EDAT- 2017/11/28 06:00 MHDA- 2019/01/05 06:00 CRDT- 2017/11/28 06:00 PHST- 2017/11/28 06:00 [pubmed] PHST- 2019/01/05 06:00 [medline] PHST- 2017/11/28 06:00 [entrez] AID - 10.1080/17843286.2017.1403133 [doi] PST - ppublish SO - Acta Clin Belg. 2018 Oct;73(5):389-392. doi: 10.1080/17843286.2017.1403133. Epub 2017 Nov 27. PMID- 31415476 OWN - NLM STAT- MEDLINE DCOM- 20210531 LR - 20251204 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 26 IP - 8 DP - 2020 Dec TI - Characterization of Interstitial Lung Disease Associated With Anti-Ribonucleoprotein Antibodies. PG - 327-333 LID - 10.1097/RHU.0000000000001127 [doi] AB - OBJECTIVES: Interstitial lung disease (ILD) is a common feature of mixed connective tissue disease. However, many patients do not meet the criteria for mixed connective tissue disease and thus may be diagnosed as interstitial pneumonia with autoimmune features. The aim of this study was to characterize ILD associated with anti-ribonucleoprotein (RNP) antibodies. METHODS: Chest computed tomography scans of patients with anti-RNP antibody who were seen between January 2011 and October 2015 were reviewed. The underlying disease was classified with international criteria using clinical and serological features. RESULTS: Among 544 patients with anti-RNP antibodies, 188 had a chest computed tomography scan, and 48 (26%) of them had radiological features of ILD. The presence of ILD was significantly associated with dyspnea, crackles, arthritis, Raynaud phenomenon, myositis, and sicca syndrome. The most frequent pattern was nonspecific interstitial pneumonia in 39 patients (81%). Among patients with ILD, 17 (35%) had a radiological pattern consisting of cysts and ground-glass attenuation not fulfilling the lymphoid interstitial pneumonia criteria. In 3 patients, cysts were related to fibrosis; in 14 patients, cysts corresponded to an original ILD pattern. CONCLUSIONS: Interstitial lung disease was found in 26% of patients with anti-RNP antibodies independently of the underlying disease. Anti-RNP-associated ILD mainly corresponds to nonspecific interstitial pneumonia or an original pattern consisting of cysts and ground-glass attenuation. FAU - Lhote, Raphael AU - Lhote R AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. FAU - Grenier, Philippe AU - Grenier P AD - Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Radiologie. FAU - Haroche, Julien AU - Haroche J AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. FAU - Miyara, Makoto AU - Miyara M AD - Assistance Publique-Hôpitaux de Paris, Département d'immunochimie, Hôpital Pitié-Salpêtrière, Paris, France. FAU - Boussouar, Samia AU - Boussouar S AD - Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Radiologie. FAU - Mathian, Alexis AU - Mathian A AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. FAU - Pha, Micheline AU - Pha M AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. FAU - Amoura, Zahir AU - Amoura Z AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. FAU - Cohen Aubart, Fleur AU - Cohen Aubart F AD - From the Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides. LA - eng PT - Journal Article PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Antibodies, Antinuclear) SB - IM MH - *Antibodies, Antinuclear/analysis MH - Humans MH - *Lung Diseases, Interstitial/diagnosis/etiology MH - *Mixed Connective Tissue Disease MH - *Myositis MH - Retrospective Studies MH - Tomography, X-Ray Computed EDAT- 2019/08/16 06:00 MHDA- 2021/06/01 06:00 CRDT- 2019/08/16 06:00 PHST- 2019/08/16 06:00 [pubmed] PHST- 2021/06/01 06:00 [medline] PHST- 2019/08/16 06:00 [entrez] AID - 00124743-202012000-00004 [pii] AID - 10.1097/RHU.0000000000001127 [doi] PST - ppublish SO - J Clin Rheumatol. 2020 Dec;26(8):327-333. doi: 10.1097/RHU.0000000000001127. PMID- 21717394 OWN - NLM STAT- MEDLINE DCOM- 20111201 LR - 20221207 IS - 1098-8947 (Electronic) IS - 0743-684X (Linking) VI - 27 IP - 6 DP - 2011 Jul TI - Case of combined thenar and hypothenar hammer syndrome: case report and brief review of the literature. PG - 373-6 LID - 10.1055/s-0031-1281517 [doi] AB - Acute or chronic arterial thrombose due to repetitive blunt trauma to the palm of the hand is a rare occupational vascular disease. In most of the cases it affects the ulnar artery and its superficial palmar branch. Repetitive crush is pathogenic and the unique anatomy of the superficial branch of the ulnar artery lying next to the hook of hamate is causative. In rare cases it may affect the superficial palmar branch of the radial artery, called thenar hammer syndrome. The combination of both is an absolute rarity. Both syndromes are occupational diseases in workers using the hand as a hammer. Patients typically present with Raynaud phenomenon or complain about ischemic pain, cold intolerance, or cyanosis. The gold standard in diagnosis is the angiography. Surgical or conservative treatment can be performed successfully. We present a case of combined thenar and hypothenar hammer and a brief review of the literature. CI - © Thieme Medical Publishers. FAU - Koulaxouzidis, Georgios AU - Koulaxouzidis G AD - Department of Plastic and Hand Surgery, University Medical Center Freiburg (UMCF), Germany. georgios.koulaxouzidis@uniklinik-freiburg.de FAU - Kalash, Ziad AU - Kalash Z FAU - Zajonc, Horst AU - Zajonc H FAU - Stark, Björn AU - Stark B FAU - Bannasch, Holger AU - Bannasch H LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20110629 PL - United States TA - J Reconstr Microsurg JT - Journal of reconstructive microsurgery JID - 8502670 SB - IM MH - Angiography/methods MH - Arterial Occlusive Diseases/diagnostic imaging/etiology/surgery MH - Cumulative Trauma Disorders/*complications MH - Follow-Up Studies MH - Hand/*blood supply MH - Humans MH - Male MH - Microsurgery/methods MH - Middle Aged MH - Peripheral Arterial Disease/*diagnostic imaging/etiology/*surgery MH - Plastic Surgery Procedures/methods MH - Risk Assessment MH - Syndrome MH - Treatment Outcome MH - Ulnar Artery/diagnostic imaging/*surgery MH - Vascular Patency/physiology MH - Vascular Surgical Procedures/methods EDAT- 2011/07/01 06:00 MHDA- 2011/12/13 00:00 CRDT- 2011/07/01 06:00 PHST- 2011/07/01 06:00 [entrez] PHST- 2011/07/01 06:00 [pubmed] PHST- 2011/12/13 00:00 [medline] AID - 10.1055/s-0031-1281517 [doi] PST - ppublish SO - J Reconstr Microsurg. 2011 Jul;27(6):373-6. doi: 10.1055/s-0031-1281517. Epub 2011 Jun 29. PMID- 16432874 OWN - NLM STAT- MEDLINE DCOM- 20060414 LR - 20121115 IS - 0361-8609 (Print) IS - 0361-8609 (Linking) VI - 81 IP - 2 DP - 2006 Feb TI - Effects of sarpogrelate hydrochloride in a patient with chronic graft-versus-host disease: a case report. PG - 121-3 AB - Chronic graft-versus-host disease (cGVHD) is a frequent complication of allogenic bone marrow transplantation. Even with aggressive treatment, cGVHD is associated with a significant degree of morbidity and mortality. cGVHD resembles autoimmune disorder, particularly systemic sclerosis (SSc). Sarpogrelate hydrochloride (SH) is an antagonist of the 5-hydroxytryptamine2A (5HT2A) receptor and has been reported to be effective in the treatment of systemic sclerosis (SSc) patients with Raynaud phenomenon. We used SH to treat a cGVHD patient, and we measured plasma PDGF and total TGF-beta levels. After SH treatment, his plasma PDGF and total TGF-beta levels decreased, and he noticed improvement in his skin pigmentation. In the present case, SH may have improved the skin lesion by inhibiting the synthesis of PDGF and TGF-beta. CI - 2006 Wiley-Liss, Inc. FAU - Hayashi, Tomoe AU - Hayashi T AD - Cellular Transplantation Biology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan. tomoe11@nifty.com FAU - Morishita, Eriko AU - Morishita E FAU - Ontachi, Yasuo AU - Ontachi Y FAU - Yamazaki, Masahide AU - Yamazaki M FAU - Asakura, Hidesaku AU - Asakura H FAU - Yoshida, Takashi AU - Yoshida T FAU - Nakao, Shinji AU - Nakao S LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Hematol JT - American journal of hematology JID - 7610369 RN - 0 (Serotonin Antagonists) RN - 0 (Succinates) RN - 0 (Transforming Growth Factor beta) RN - 19P708E787 (sarpogrelate) RN - EC 2.7.10.1 (Receptors, Platelet-Derived Growth Factor) SB - IM MH - Adult MH - Bone Marrow Transplantation/adverse effects MH - Chronic Disease MH - Graft vs Host Disease/*drug therapy MH - Humans MH - Male MH - Receptors, Platelet-Derived Growth Factor/blood/drug effects MH - Serotonin Antagonists/*therapeutic use MH - Succinates/*pharmacology/therapeutic use MH - Transforming Growth Factor beta/blood/drug effects EDAT- 2006/01/25 09:00 MHDA- 2006/04/15 09:00 CRDT- 2006/01/25 09:00 PHST- 2006/01/25 09:00 [pubmed] PHST- 2006/04/15 09:00 [medline] PHST- 2006/01/25 09:00 [entrez] AID - 10.1002/ajh.20511 [doi] PST - ppublish SO - Am J Hematol. 2006 Feb;81(2):121-3. doi: 10.1002/ajh.20511. PMID- 42007790 OWN - NLM STAT- Publisher LR - 20260507 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) DP - 2026 Apr 20 TI - Artificial Intelligence in Systemic Sclerosis: Clinical Applications, Challenges, and Future Directions. LID - 10.1002/acr.80068 [doi] AB - Systemic sclerosis (SSc) is a rare autoimmune disease defined by immune dysregulation, vasculopathy, and progressive fibrosis of the skin and internal organs. Despite advances in care, major complications such as interstitial lung disease (ILD) and myocardial involvement remain the leading causes of morbidity and mortality. Current assessment tools, such as the modified Rodnan skin score, pulmonary function tests, and nailfold capillaroscopy, are limited by subjectivity and interobserver variability. Artificial intelligence (AI) is reshaping this landscape. Machine learning, deep learning, and radiomics have shown potential to enhance disease phenotyping, risk stratification, and imaging quantification in SSc. AI-driven high-resolution computed tomography analysis enables automated fibrosis segmentation and radiomic risk modeling in SSc-ILD. Computer vision approaches applied to nailfold capillaroscopy achieve expert-level agreement for microvascular staging, whereas emerging digital tools aim to quantify Raynaud phenomenon dynamics. In skin disease, AI-assisted ultrasonography, optical imaging, and histopathology provide reproducible quantification of dermal remodeling. However, most applications remain exploratory, based on small or single-center data sets with limited external validation. Challenges such as data heterogeneity, annotation burden, and the "small data" constraints of rare diseases limit immediate clinical translation. This narrative review critically examines current AI applications in SSc, highlighting methodologic limitations, translational readiness, and future directions toward robust, multicenter, and ethically governed implementation. CI - © 2026 The Author(s). Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Sieiro Santos, Cristiana AU - Sieiro Santos C AUID- ORCID: 0000-0003-0889-9877 AD - Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine, and Health, University of Manchester, Manchester, United Kingdom. FAU - Sequí-Sabater, José Miguel AU - Sequí-Sabater JM AUID- ORCID: 0000-0001-8437-1623 AD - Rheumatology Department, La Ribera University Hospital, Alzira, Valencia, Spain. AD - Rheumatology Department, La Fe Polytechnic and University Hospital, Valencia, Spain. AD - Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden. FAU - Benavent, Diego AU - Benavent D AUID- ORCID: 0000-0001-9119-5330 AD - Rheumatology Department, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain. LA - eng PT - Journal Article PT - Review DEP - 20260420 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM EDAT- 2026/04/21 13:10 MHDA- 2026/04/21 13:10 CRDT- 2026/04/20 09:33 PHST- 2026/03/09 00:00 [revised] PHST- 2026/01/14 00:00 [received] PHST- 2026/03/31 00:00 [accepted] PHST- 2026/04/21 13:10 [pubmed] PHST- 2026/04/21 13:10 [medline] PHST- 2026/04/20 09:33 [entrez] AID - 10.1002/acr.80068 [doi] PST - aheadofprint SO - Arthritis Care Res (Hoboken). 2026 Apr 20. doi: 10.1002/acr.80068. PMID- 31738830 OWN - NLM STAT- MEDLINE DCOM- 20200908 LR - 20200908 IS - 2473-9537 (Electronic) IS - 2473-9529 (Print) IS - 2473-9529 (Linking) VI - 3 IP - 22 DP - 2019 Nov 26 TI - Non-del(5q) myelodysplastic syndromes-associated loci detected by SNP-array genome-wide association meta-analysis. PG - 3579-3589 LID - 10.1182/bloodadvances.2019000922 [doi] AB - Myelodysplastic syndromes (MDS) are hematopoietic stem cell malignancies. Known predisposing factors to adult MDS include rare germline mutations, cytotoxic therapy, age-related clonal hematopoiesis, and autoimmune or chronic inflammatory disorders. To date, no published studies characterizing MDS-associated germline susceptibility polymorphisms exist. We performed a genome-wide association study of 2 sample sets (555 MDS cases vs 2964 control subjects; 352 MDS cases vs 2640 control subjects) in non-del(5q) MDS cases of European genomic ancestry. Meta-analysis identified 8 MDS-associated loci at 1q31.1 (PLA2G4A), 3p14.1 (FAM19A4), 5q21.3 (EFNA5), 6p21.33, 10q23.1 (GRID1), 12q24.32, 15q26.1, and 20q13.12 (EYA2) that approached genome-wide significance. Gene expression for 5 loci that mapped within or near genes was significantly upregulated in MDS bone marrow cells compared with those of control subjects (P < .01). Higher PLA2G4A expression and lower EYA2 expression were associated with poorer overall survival (P = .039 and P = .037, respectively). Higher PLA2G4A expression is associated with mutations in NRAS (P < .001), RUNX1 (P = .012), ASXL1 (P = .007), and EZH2 (P = .038), all of which are known to contribute to MDS development. EYA2 expression was an independently favorable risk factor irrespective of age, sex, and Revised International Scoring System score (relative risk, 0.67; P = .048). Notably, these genes have regulatory roles in innate immunity, a critical driver of MDS pathogenesis. EYA2 overexpression induced innate immune activation, whereas EYA2 inhibition restored colony-forming potential in primary MDS cells indicative of hematopoietic restoration and possible clinical relevance. In conclusion, among 8 suggestive MDS-associated loci, 5 map to genes upregulated in MDS with functional roles in innate immunity and potential biological relevance to MDS. CI - © 2019 by The American Society of Hematology. FAU - McGraw, Kathy L AU - McGraw KL AD - Department of Malignant Hematology and. FAU - Cheng, Chia-Ho AU - Cheng CH AD - Department of Biostatistics and Bioinformatics, Moffitt Cancer Center, Tampa, FL. FAU - Chen, Y Ann AU - Chen YA AD - Department of Biostatistics and Bioinformatics, Moffitt Cancer Center, Tampa, FL. FAU - Hou, Hsin-An AU - Hou HA AD - Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan. FAU - Nilsson, Björn AU - Nilsson B AD - Department of Laboratory Medicine, Section of Hematology and Transfusion Medicine, Lund University, Lund, Sweden. FAU - Genovese, Giulio AU - Genovese G AD - Stanley Center for Psychiatric Research and. AD - Program in Medical and Population Genetics, Broad Institute of the Massachusetts Institute of Technology and Harvard, Cambridge, MA. AD - Department of Genetics, Harvard Medical School, Boston, MA. FAU - Cluzeau, Thomas AU - Cluzeau T AD - Cote d'Azur University, Centre Hospitalier Universitaire of Nice, Nice, France. FAU - Pellagatti, Andrea AU - Pellagatti A AD - Bloodwise Molecular Haematology Unit, Nuffield Division of Clinical Laboratory Sciences, Radcliffe Department of Medicine, University of Oxford and Oxford Biomedical Research Centre Haematology Theme, Oxford, United Kingdom. FAU - Przychodzen, Bartlomiej P AU - Przychodzen BP AD - Cleveland Clinic, Taussig Cancer Institute, Cleveland, OH. FAU - Mallo, Mar AU - Mallo M AD - Institut de Recerca Contra la Leucèmia Josep Carreras, Institut Catala d'Oncologia-Hospital GermansTrias i Pujol, Universitat Autonòma de Barcelona, Badalona, Barcelona, Spain. FAU - Arenillas, Leonor AU - Arenillas L AD - Laboratori de Citologia Hematòlogica, Servei de Patologia, Hospital del Mar, Barcelona, Spain. FAU - Mohamedali, Azim AU - Mohamedali A AD - Department of Haematological Medicine, Kings College London, London, United Kingdom. FAU - Adès, Lionel AU - Adès L AD - Senior Hematology Department, Hopital Saint Louis, Paris, France. FAU - Sallman, David A AU - Sallman DA AD - Department of Malignant Hematology and. FAU - Padron, Eric AU - Padron E AD - Department of Malignant Hematology and. FAU - Sokol, Lubomir AU - Sokol L AD - Department of Malignant Hematology and. FAU - Moreilhon, Chimene AU - Moreilhon C AD - Cote d'Azur University, Centre Hospitalier Universitaire of Nice, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Cote d'Azur University, Centre Hospitalier Universitaire of Nice, Nice, France. FAU - Tien, Hwei-Fang AU - Tien HF AD - Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan. FAU - Boultwood, Jacqueline AU - Boultwood J AD - Bloodwise Molecular Haematology Unit, Nuffield Division of Clinical Laboratory Sciences, Radcliffe Department of Medicine, University of Oxford and Oxford Biomedical Research Centre Haematology Theme, Oxford, United Kingdom. FAU - Ebert, Benjamin L AU - Ebert BL AD - Departments of Medicine and Medical Oncology, Harvard Medical School and Brigham and Women's Hospital, Boston, MA; and. FAU - Sole, Francesc AU - Sole F AD - Institut de Recerca Contra la Leucèmia Josep Carreras, Institut Catala d'Oncologia-Hospital GermansTrias i Pujol, Universitat Autonòma de Barcelona, Badalona, Barcelona, Spain. FAU - Fenaux, Pierre AU - Fenaux P AD - Senior Hematology Department, Hopital Saint Louis, Paris, France. FAU - Mufti, Ghulam J AU - Mufti GJ AD - Department of Haematological Medicine, Kings College London, London, United Kingdom. FAU - Maciejewski, Jaroslaw P AU - Maciejewski JP AD - Cleveland Clinic, Taussig Cancer Institute, Cleveland, OH. FAU - Kanetsky, Peter A AU - Kanetsky PA AD - Department of Cancer Epidemiology, Moffitt Cancer Center, Tampa, FL. FAU - List, Alan F AU - List AF AD - Department of Malignant Hematology and. LA - eng GR - P30 CA076292/CA/NCI NIH HHS/United States PT - Journal Article PT - Meta-Analysis PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Blood Adv JT - Blood advances JID - 101698425 SB - IM MH - Chromosome Deletion MH - Chromosomes, Human, Pair 5 MH - Gene Expression Regulation MH - *Genetic Predisposition to Disease MH - *Genome-Wide Association Study MH - Genomics/methods MH - Humans MH - Myelodysplastic Syndromes/diagnosis/*genetics MH - *Polymorphism, Single Nucleotide MH - *Quantitative Trait Loci PMC - PMC6880887 COIS- Conflict-of-interest disclosure: The authors declare no competing financial interests. EDAT- 2019/11/19 06:00 MHDA- 2020/09/09 06:00 PMCR- 2019/11/18 CRDT- 2019/11/19 06:00 PHST- 2019/08/30 00:00 [received] PHST- 2019/10/15 00:00 [accepted] PHST- 2019/11/19 06:00 [entrez] PHST- 2019/11/19 06:00 [pubmed] PHST- 2020/09/09 06:00 [medline] PHST- 2019/11/18 00:00 [pmc-release] AID - 428769 [pii] AID - 2019/ADV2019000922 [pii] AID - 10.1182/bloodadvances.2019000922 [doi] PST - ppublish SO - Blood Adv. 2019 Nov 26;3(22):3579-3589. doi: 10.1182/bloodadvances.2019000922. PMID- 27856460 OWN - NLM STAT- MEDLINE DCOM- 20170814 LR - 20260127 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 129 IP - 4 DP - 2017 Jan 26 TI - Architectural and functional heterogeneity of hematopoietic stem/progenitor cells in non-del(5q) myelodysplastic syndromes. PG - 484-496 LID - 10.1182/blood-2016-03-707745 [doi] AB - Myelodysplastic syndromes (MDSs) are hematopoietic stem cell disorders in which recurrent mutations define clonal hematopoiesis. The origin of the phenotypic diversity of non-del(5q) MDS remains unclear. Here, we investigated the clonal architecture of the CD34(+)CD38(-) hematopoietic stem/progenitor cell (HSPC) compartment and interrogated dominant clones for MDS-initiating cells. We found that clones mainly accumulate mutations in a linear succession with retention of a dominant subclone. The clone detected in the long-term culture-initiating cell compartment that reconstitutes short-term human hematopoiesis in xenotransplantation models is usually the dominant clone, which gives rise to the myeloid and to a lesser extent to the lymphoid lineage. The pattern of mutations may differ between common myeloid progenitors (CMPs), granulomonocytic progenitors (GMPs), and megakaryocytic-erythroid progenitors (MEPs). Rare STAG2 mutations can amplify at the level of GMPs, from which it may drive the transformation to acute myeloid leukemia. We report that major truncating BCOR gene mutation affecting HSPC and CMP was beneath the threshold of detection in GMP or MEP. Consistently, BCOR knock-down (KD) in normal CD34(+) progenitors modifies their granulocytic and erythroid differentiation. Clonal architecture of the HSPC compartment and mutations selected during differentiation contribute to the phenotypic heterogeneity of MDS. Defining the hierarchy of driver mutations provides insights into the process of transformation and may guide the search for novel therapeutic strategies. CI - © 2017 by The American Society of Hematology. FAU - Chesnais, Virginie AU - Chesnais V AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. FAU - Arcangeli, Marie-Laure AU - Arcangeli ML AD - INSERM Unité Mixte de Recherche 967, Commissariat à l'Energie Atomique/Département des Sciences de la Vie/Institut de Recherche en Radiobiologie Cellulaire et Moléculaire Laboratory of Hematopoietic Stem Cells and Leukemic Cells, Equipe Labellisée par la Ligue Nationale Contre le Cancer, Université Paris Diderot, Université Paris-Saclay, Commissariat à l'Energie Atomique et aux Energies Alternatives, Fontenay-aux-Roses, France. FAU - Delette, Caroline AU - Delette C AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. FAU - Rousseau, Alice AU - Rousseau A AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Biologique, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Guermouche, Hélène AU - Guermouche H AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Biologique, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Lefevre, Carine AU - Lefevre C AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. FAU - Bondu, Sabrina AU - Bondu S AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. FAU - Diop, M'boyba AU - Diop M AD - INSERM U1170, Equipe Labellisée Ligue Nationale Contre le Cancer, Université Paris-Saclay, Institut Gustave Roussy, Villejuif, France. FAU - Cheok, Meyling AU - Cheok M AD - INSERM, Centre Hospitalier Universitaire Lille, Unité de Mixte de Recherche-Scientifique 1172, Jean-Pierre Aubert Research Center, Lille, France. FAU - Chapuis, Nicolas AU - Chapuis N AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Biologique, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Legros, Laurence AU - Legros L AD - Service de Médecine Interne, Centre Hospitalier Universitaire, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Laboratoire d'Hématologie Centre Hospitalier Universitaire, Nice, France; and. FAU - Willems, Lise AU - Willems L AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Bouscary, Didier AU - Bouscary D AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Lauret, Evelyne AU - Lauret E AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. FAU - Bernard, Olivier A AU - Bernard OA AD - INSERM U1170, Equipe Labellisée Ligue Nationale Contre le Cancer, Université Paris-Saclay, Institut Gustave Roussy, Villejuif, France. FAU - Kosmider, Olivier AU - Kosmider O AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Biologique, Hôpitaux Universitaires Paris Centre, Paris, France. FAU - Pflumio, Françoise AU - Pflumio F AD - INSERM Unité Mixte de Recherche 967, Commissariat à l'Energie Atomique/Département des Sciences de la Vie/Institut de Recherche en Radiobiologie Cellulaire et Moléculaire Laboratory of Hematopoietic Stem Cells and Leukemic Cells, Equipe Labellisée par la Ligue Nationale Contre le Cancer, Université Paris Diderot, Université Paris-Saclay, Commissariat à l'Energie Atomique et aux Energies Alternatives, Fontenay-aux-Roses, France. FAU - Fontenay, Michaela AU - Fontenay M AD - Institut Cochin, Paris, France. AD - Institut National de la Santé et de la Recherche Médicale INSERM U1016, Paris, France. AD - Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8104, Paris, France. AD - Université Paris Descartes, Paris, France. AD - Assistance Publique-Hôpitaux de Paris, Service d'Hématologie Biologique, Hôpitaux Universitaires Paris Centre, Paris, France. LA - eng PT - Journal Article DEP - 20161116 PL - United States TA - Blood JT - Blood JID - 7603509 RN - EC 3.2.2.6 (ADP-ribosyl Cyclase 1) RN - 0 (Antigens, CD34) RN - 0 (Antigens, Nuclear) RN - 0 (Cell Cycle Proteins) RN - 0 (Membrane Glycoproteins) RN - 0 (Proto-Oncogene Proteins) RN - 0 (Repressor Proteins) RN - 0 (BCOR protein, human) RN - 0 (STAG2 protein, human) RN - 0 (Cohesins) RN - EC 3.2.2.5 (CD38 protein, human) SB - IM MH - ADP-ribosyl Cyclase 1/deficiency/genetics MH - Animals MH - Antigens, CD34/genetics/metabolism MH - Antigens, Nuclear/genetics/metabolism MH - Cell Cycle Proteins MH - Cell Differentiation MH - Cell Lineage/genetics MH - *Chromosomes, Human, Pair 5 MH - Clone Cells MH - Disease Progression MH - Female MH - Gene Expression MH - Gene Knockdown Techniques MH - Hematopoietic Stem Cells/*metabolism/pathology MH - Humans MH - Immunophenotyping MH - Leukemia, Myeloid, Acute/complications/*genetics/metabolism/pathology MH - Lymphocytes/*metabolism/pathology MH - Membrane Glycoproteins/deficiency/genetics MH - Mice MH - Mice, Inbred NOD MH - *Mutation MH - Myelodysplastic Syndromes/complications/*genetics/metabolism/pathology MH - Myeloid Cells/*metabolism/pathology MH - Phenotype MH - Proto-Oncogene Proteins/genetics/metabolism MH - Repressor Proteins/genetics/metabolism MH - Transplantation, Heterologous MH - Cohesins EDAT- 2016/11/20 06:00 MHDA- 2017/08/15 06:00 CRDT- 2016/11/19 06:00 PHST- 2016/03/26 00:00 [received] PHST- 2016/11/06 00:00 [accepted] PHST- 2016/11/20 06:00 [pubmed] PHST- 2017/08/15 06:00 [medline] PHST- 2016/11/19 06:00 [entrez] AID - S0006-4971(20)33757-5 [pii] AID - 10.1182/blood-2016-03-707745 [doi] PST - ppublish SO - Blood. 2017 Jan 26;129(4):484-496. doi: 10.1182/blood-2016-03-707745. Epub 2016 Nov 16. PMID- 19581935 OWN - NLM STAT- MEDLINE DCOM- 20091008 LR - 20161125 IS - 1476-5594 (Electronic) IS - 0950-9232 (Linking) VI - 28 IP - 37 DP - 2009 Sep 17 TI - The tyrosine kinase Syk regulates the survival of chronic lymphocytic leukemia B cells through PKCdelta and proteasome-dependent regulation of Mcl-1 expression. PG - 3261-73 LID - 10.1038/onc.2009.179 [doi] AB - B-cell chronic lymphocytic leukemia (B-CLL) is characterized by accumulation of mature monoclonal CD5+ B cells. The disease results mainly from a failure of cells to undergo apoptosis, a process largely influenced by the existence of constitutively activated components of B-cell receptor signaling and the deregulated expression of anti-apoptotic molecules. Recent evidence pointing to a critical role of spleen tyrosine kinase (Syk) in ligand-independent BCR signaling prompted us to examine its role in primary B-CLL cell survival. We demonstrate that pharmacological inhibition of constitutive Syk activity and silencing by siRNA led to a dramatic decrease of cell viability in CLL samples (n=44), regardless of clinical and biological status and induced typical apoptotic cell death with mitochondrial failure followed by caspase 3-dependent cell death. We also provide functional and biochemical evidence that Syk regulated B-CLL cell survival through a novel pathway involving PKCdelta and a proteasome-dependent regulation of the anti-apoptotic protein Mcl-1. Together, our observations are consistent with a model wherein PKCdelta downstream of Syk stabilizes Mcl-1 through inhibitory phosphorylation of GSK3 by Akt. We conclude that Syk constitutes a key regulator of B-CLL cell survival, emphasizing the clinical utility of Syk inhibition in hematopoietic malignancies. FAU - Baudot, A D AU - Baudot AD AD - INSERM UMR576, Nice, France. FAU - Jeandel, P Y AU - Jeandel PY FAU - Mouska, X AU - Mouska X FAU - Maurer, U AU - Maurer U FAU - Tartare-Deckert, S AU - Tartare-Deckert S FAU - Raynaud, S D AU - Raynaud SD FAU - Cassuto, J P AU - Cassuto JP FAU - Ticchioni, M AU - Ticchioni M FAU - Deckert, M AU - Deckert M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090706 PL - England TA - Oncogene JT - Oncogene JID - 8711562 RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (Ligands) RN - 0 (Myeloid Cell Leukemia Sequence 1 Protein) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Proto-Oncogene Proteins c-bcl-2) RN - 0 (RNA, Small Interfering) RN - EC 2.7.10.1 (Protein-Tyrosine Kinases) RN - EC 2.7.10.2 (SYK protein, human) RN - EC 2.7.10.2 (Syk Kinase) RN - EC 2.7.11.13 (Protein Kinase C-delta) RN - EC 3.4.22.- (Caspase 3) RN - EC 3.4.25.1 (Proteasome Endopeptidase Complex) SB - IM MH - Apoptosis/drug effects/genetics MH - B-Lymphocytes/drug effects/metabolism/*pathology MH - Caspase 3/metabolism MH - Cell Survival/drug effects/genetics MH - *Gene Expression Regulation, Neoplastic MH - Humans MH - Intracellular Signaling Peptides and Proteins/antagonists & inhibitors/deficiency/genetics/*metabolism MH - Leukemia, Lymphocytic, Chronic, B-Cell/enzymology/genetics/metabolism/*pathology MH - Ligands MH - Myeloid Cell Leukemia Sequence 1 Protein MH - Phosphorylation MH - Proteasome Endopeptidase Complex/*metabolism MH - Protein Kinase C-delta/*metabolism MH - Protein Kinase Inhibitors/pharmacology MH - Protein-Tyrosine Kinases/antagonists & inhibitors/deficiency/genetics/*metabolism MH - Proto-Oncogene Proteins c-bcl-2/*metabolism MH - RNA Interference MH - RNA, Small Interfering/genetics MH - Signal Transduction/drug effects MH - Syk Kinase EDAT- 2009/07/08 09:00 MHDA- 2009/10/09 06:00 CRDT- 2009/07/08 09:00 PHST- 2009/07/08 09:00 [entrez] PHST- 2009/07/08 09:00 [pubmed] PHST- 2009/10/09 06:00 [medline] AID - onc2009179 [pii] AID - 10.1038/onc.2009.179 [doi] PST - ppublish SO - Oncogene. 2009 Sep 17;28(37):3261-73. doi: 10.1038/onc.2009.179. Epub 2009 Jul 6. PMID- 17913675 OWN - NLM STAT- MEDLINE DCOM- 20071218 LR - 20131121 IS - 0003-3898 (Print) IS - 0003-3898 (Linking) VI - 65 IP - 5 DP - 2007 Sep-Oct TI - [Late diagnosis of a McArdle disease's case (type V glycogenosis)]. PG - 550-4 AB - McArdle's disease is a metabolic myopathy characterized by a myophosphorylase deficiency resulting in an inability to degrade glycogen stores. We report the case of a 48 years old patient who complained since adolescence of rest and exercise myalgias and presented a chronic increased plasma creatine kinase activity. First, a maximal exercise test was performed. This test demonstrated a quasi lack of rise of respiratory exchange ratio and of blood lactate, possibly due to a glycogenolytic/glycolytic pathway deficiency. Second, a biopsy of vastus lateralis muscle was performed using Bergström needle. As expected, the analysis of mitochondrial function was normal. The in vitro screening test of the glycogenolysis/glycolysis pathway showed a lack of lactate production in presence of glycogen substrate. The study of muscular metabolism of glycogen revealed a glycogen accumulation and a decrease of active and total phosphorylase activities. These data allowed us to diagnose a type V glycogenosis, or McArdle's disease. The patient appeared heterozygous for the most frequent mutation (p.R50X). FAU - Flavier, S AU - Flavier S AD - Service central de physiologie clinique, Unité d'exploration métabolique (CERAMM), Hôpital Lapeyronie, CHU de Montpellier et Inserm, équipe ERI 25 / EA 4202 Muscle et pathologies, Montpellier. FAU - Rolland, M O AU - Rolland MO FAU - Eude, M AU - Eude M FAU - Fédou, C AU - Fédou C FAU - Brun, J F AU - Brun JF FAU - Maire, I AU - Maire I FAU - Mercier, J AU - Mercier J FAU - Raynaud, E AU - Raynaud E LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Diagnostic tardif d'une maladie de McArdle (glycogénose de type V). PL - France TA - Ann Biol Clin (Paris) JT - Annales de biologie clinique JID - 2984690R RN - 33X04XA5AT (Lactic Acid) RN - 9005-79-2 (Glycogen) RN - EC 2.4.1.- (Glycogen Phosphorylase, Muscle Form) RN - EC 2.4.1.- (Phosphorylases) RN - EC 2.7.3.2 (Creatine Kinase) SB - IM MH - Creatine Kinase/blood MH - Exercise Test MH - Female MH - Glycogen/metabolism MH - Glycogen Phosphorylase, Muscle Form/genetics MH - Glycogen Storage Disease Type V/*diagnosis/genetics MH - Heterozygote MH - Humans MH - Lactic Acid/blood MH - Middle Aged MH - Muscle, Skeletal/metabolism MH - Mutation/genetics MH - Phosphorylases/analysis MH - Pulmonary Gas Exchange EDAT- 2007/10/05 09:00 MHDA- 2007/12/19 09:00 CRDT- 2007/10/05 09:00 PHST- 2007/05/25 00:00 [received] PHST- 2007/06/23 00:00 [accepted] PHST- 2007/10/05 09:00 [pubmed] PHST- 2007/12/19 09:00 [medline] PHST- 2007/10/05 09:00 [entrez] PST - ppublish SO - Ann Biol Clin (Paris). 2007 Sep-Oct;65(5):550-4. PMID- 32533291 OWN - NLM STAT- MEDLINE DCOM- 20211115 LR - 20211115 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 41 IP - 5 DP - 2021 May TI - Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review. PG - 987-991 LID - 10.1007/s00296-020-04625-1 [doi] AB - Herein, we report a case-based review of the Sneddon Syndrome (SS), a rare chronic condition which affects small to medium blood vessels. It is known by its skin presentation, livedo racemosa (LRC), and the relapsing cerebrovascular events. However, neither LRC nor cerebrovascular events are exclusive to SS. A 36-year-old female with history of mitral valve prolapse, hypothyroidism, Raynaud phenomenon, hypertension, migraines, and four episodes of transient ischemic attacks (TIA), presented to our clinic with new skin findings, suggestive of LRC. Based on her previous history, current presentation and skin biopsy results, she was diagnosed with SS secondary to antiphospholipid syndrome. The present report illustrates the difficulty in recognizing SS and how the heterogeneity of the disease may be contributing to the difficulty making a distinct diagnosis. FAU - Kong, Steve S AU - Kong SS AUID- ORCID: 0000-0002-7678-2825 AD - University of Central Florida College of Medicine, Orlando, FL, USA. FAU - Azarfar, Azin AU - Azarfar A AUID- ORCID: 0000-0003-0170-8011 AD - University of Central Florida HCA Healthcare GME, 6850 Lake Nona Blvd, Orlando, FL, 32827, USA. azin.azarfar@ucf.edu. AD - Department of Internal Medicine, University of Central Florida College of Medicine, Orlando, FL, USA. azin.azarfar@ucf.edu. FAU - Bhanusali, Neha AU - Bhanusali N AUID- ORCID: 0000-0003-1800-3747 AD - Department of Internal Medicine, University of Central Florida College of Medicine, Orlando, FL, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20200612 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antirheumatic Agents) RN - 0 (Factor Xa Inhibitors) RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 9NDF7JZ4M3 (Rivaroxaban) SB - IM MH - Adult MH - Antiphospholipid Syndrome/complications/*diagnosis/drug therapy MH - Antirheumatic Agents/administration & dosage MH - Factor Xa Inhibitors/administration & dosage MH - Female MH - Humans MH - Hydroxychloroquine/administration & dosage MH - Rivaroxaban/administration & dosage MH - Skin/pathology MH - Sneddon Syndrome/complications/*diagnosis/drug therapy OTO - NOTNLM OT - Case report OT - Cerebrovascular disease OT - Literature review OT - Livedo racemosa OT - Sneddon Syndrome EDAT- 2020/06/14 06:00 MHDA- 2021/11/16 06:00 CRDT- 2020/06/14 06:00 PHST- 2020/02/21 00:00 [received] PHST- 2020/06/09 00:00 [accepted] PHST- 2020/06/14 06:00 [pubmed] PHST- 2021/11/16 06:00 [medline] PHST- 2020/06/14 06:00 [entrez] AID - 10.1007/s00296-020-04625-1 [pii] AID - 10.1007/s00296-020-04625-1 [doi] PST - ppublish SO - Rheumatol Int. 2021 May;41(5):987-991. doi: 10.1007/s00296-020-04625-1. Epub 2020 Jun 12. PMID- 32892170 OWN - NLM STAT- MEDLINE DCOM- 20210701 LR - 20210701 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 6 IP - 2 DP - 2020 Sep TI - Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies. LID - 10.1136/rmdopen-2020-001357 [doi] LID - e001357 AB - OBJECTIVE: To describe systemic sclerosis (SSc) with myopathy in patients without classic SSc-specific and SSc-overlap autoantibodies (aAbs), referred to as seronegative scleromyositis. METHODS: Twenty patients with seronegative scleromyositis diagnosed by expert opinion were analysed retrospectively for SSc features at myositis diagnosis and follow-up, and stratified based on HEp-2 nuclear patterns by indirect immunofluorescence (IIF) according to International Consensus of Autoantibody Patterns. Specificities were analysed by protein A-assisted immunoprecipitation. Myopathy was considered an organ involvement of SSc. RESULTS: SSc sine scleroderma was a frequent presentation (45%) at myositis diagnosis. Myositis was the most common first non-Raynaud manifestation of SSc (55%). Lower oesophagal dysmotility was present in 10 of 11 (91%) investigated patients. At follow-up, 80% of the patients met the American College of Rheumatology/EULAR SSc classification criteria. Two-thirds of patients had a positive HEp-2 IIF nuclear pattern (all with titers ≥1/320), defining three novel scleromyositis subsets. First, antinuclear antibody (ANA)-negative scleromyositis was associated with interstitial lung disease (ILD) and renal crisis. Second, a speckled pattern uncovered multiple rare SSc-specific aAbs. Third, the nuclear dots pattern was associated with aAbs to survival of motor neuron (SMN) complex and a novel scleromyositis subset characteriszed by calcinosis but infrequent ILD and renal crisis. CONCLUSIONS: SSc skin involvement is often absent in early seronegative scleromyositis. ANA positivity, Raynaud phenomenon, SSc-type capillaroscopy and/or lower oesophagal dysmotility may be clues for scleromyositis. Using HEp-2 IIF patterns, three novel clinicoserological subsets of scleromyositis emerged, notably (1) ANA-negative, (2) ANA-positive with a speckled pattern and (3) ANA-positive with nuclear dots and anti-SMN aAbs. CI - © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Landon-Cardinal, Océane AU - Landon-Cardinal O AUID- ORCID: 0000-0002-3361-6165 AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada oceane.landon-cardinal@umontreal.ca. FAU - Baril-Dionne, Alexandra AU - Baril-Dionne A AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Hoa, Sabrina AU - Hoa S AUID- ORCID: 0000-0002-9466-5729 AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Meyer, Alain AU - Meyer A AUID- ORCID: 0000-0003-0893-6041 AD - Centre de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. FAU - Leclair, Valérie AU - Leclair V AD - Division of Rheumatology, Department of Medicine, Jewish General Hospital; Department of Medicine, McGill University, Montreal, QC, Canada. FAU - Bourré-Tessier, Josiane AU - Bourré-Tessier J AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Mansour, Anne-Marie AU - Mansour AM AD - Division of Internal Medicine, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, QC, Canada. FAU - Zarka, Farah AU - Zarka F AD - Division of Internal Medicine, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, QC, Canada. FAU - Makhzoum, Jean-Paul AU - Makhzoum JP AD - Division of Internal Medicine, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, QC, Canada. FAU - Nehme, Jessica AU - Nehme J AD - Division of Geriatrics, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, QC, Canada. FAU - Rich, Eric AU - Rich E AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Goulet, Jean-Richard AU - Goulet JR AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Grodzicky, Tamara AU - Grodzicky T AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Koenig, Martial AU - Koenig M AD - Division of Internal Medicine, CHUM; Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Joyal, France AU - Joyal F AD - Division of Internal Medicine, CHUM; Department of Medicine, Université de Montréal, Montreal, QC, Canada. FAU - Richard, Isabelle AU - Richard I AD - Centre intégré de santé et de services sociaux Abitibi Témiscamingue, Rouyn-Noranda, QC, Canada. FAU - Hudson, Marie AU - Hudson M AD - Division of Rheumatology, Department of Medicine, Jewish General Hospital; Department of Medicine, McGill University, Montreal, QC, Canada. AD - Lady Davis Institute for Medical Research, Montreal, QC, Canada. FAU - Targoff, Ira AU - Targoff I AD - University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA. FAU - Satoh, Minoru AU - Satoh M AD - Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Kitakyushu, Japan. FAU - Fritzler, Marvin J AU - Fritzler MJ AD - Cumming School of Medicine, University of Calgary, Calgary, AB, Canada. FAU - Troyanov, Yves AU - Troyanov Y AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. AD - Division of Rheumatology, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, QC, Canada. FAU - Senécal, Jean-Luc AU - Senécal JL AD - Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM); Department of Medicine, Université de Montréal, Montreal, QC, Canada. AD - Autoimmunity Research Laboratory, CHUM Research Center, Montreal, QC, Canada. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (SMN Complex Proteins) SB - IM MH - Antibodies, Antinuclear/blood/*immunology MH - Autoantibodies/blood/*immunology MH - Autoimmunity MH - Disease Susceptibility MH - Female MH - Fluorescent Antibody Technique, Indirect MH - Humans MH - Immunoprecipitation MH - Male MH - Myositis/blood/*diagnosis/*etiology MH - Retrospective Studies MH - SMN Complex Proteins/*immunology MH - Scleroderma, Systemic/blood/*diagnosis/*etiology MH - Serologic Tests PMC - PMC7509989 OTO - NOTNLM OT - Autoimmune Diseases OT - Polymyositis OT - Scleroderma OT - Systemic COIS- Competing interests: None declared. EDAT- 2020/09/07 06:00 MHDA- 2021/07/02 06:00 PMCR- 2020/09/04 CRDT- 2020/09/06 20:35 PHST- 2020/06/08 00:00 [received] PHST- 2020/07/17 00:00 [revised] PHST- 2020/08/10 00:00 [accepted] PHST- 2020/09/06 20:35 [entrez] PHST- 2020/09/07 06:00 [pubmed] PHST- 2021/07/02 06:00 [medline] PHST- 2020/09/04 00:00 [pmc-release] AID - rmdopen-2020-001357 [pii] AID - 10.1136/rmdopen-2020-001357 [doi] PST - ppublish SO - RMD Open. 2020 Sep;6(2):e001357. doi: 10.1136/rmdopen-2020-001357. PMID- 36133926 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240904 IS - 1179-156X (Print) IS - 1179-156X (Electronic) IS - 1179-156X (Linking) VI - 14 DP - 2022 TI - Predicting the Progression of Very Early Systemic Sclerosis: Current Insights. PG - 171-186 LID - 10.2147/OARRR.S285409 [doi] AB - Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc. CI - © 2022 Bellocchi et al. FAU - Bellocchi, Chiara AU - Bellocchi C AUID- ORCID: 0000-0001-8326-7904 AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, University of Milan, Milan, Italy. AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. FAU - Chung, Augustine AU - Chung A AD - Division of Pulmonary and Critical Care, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA. FAU - Volkmann, Elizabeth R AU - Volkmann ER AUID- ORCID: 0000-0003-3750-6569 AD - Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA. LA - eng GR - K23 HL150237/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Review DEP - 20220915 PL - New Zealand TA - Open Access Rheumatol JT - Open access rheumatology : research and reviews JID - 101688698 PMC - PMC9484572 OTO - NOTNLM OT - disease progression OT - early diagnosis OT - scleroderma OT - systemic sclerosis COIS- Dr Elizabeth R Volkmann reports grants, personal fees from Boehringer Ingelheim, grants from Horizon and grants from Kadmon, outside the submitted work; also awarded grants from Prometheus. The authors report no other conflicts of interest in this work. EDAT- 2022/09/23 06:00 MHDA- 2022/09/23 06:01 PMCR- 2022/09/15 CRDT- 2022/09/22 04:03 PHST- 2022/05/24 00:00 [received] PHST- 2022/09/06 00:00 [accepted] PHST- 2022/09/22 04:03 [entrez] PHST- 2022/09/23 06:00 [pubmed] PHST- 2022/09/23 06:01 [medline] PHST- 2022/09/15 00:00 [pmc-release] AID - 285409 [pii] AID - 10.2147/OARRR.S285409 [doi] PST - epublish SO - Open Access Rheumatol. 2022 Sep 15;14:171-186. doi: 10.2147/OARRR.S285409. eCollection 2022. PMID- 36976428 OWN - NLM STAT- MEDLINE DCOM- 20240115 LR - 20240423 IS - 1573-4919 (Electronic) IS - 0300-8177 (Linking) VI - 479 IP - 1 DP - 2024 Jan TI - Cardiac remodeling associated with chronic kidney disease is enhanced in a rat model of metabolic syndrome: Preparation of mesenchymal transition. PG - 29-39 LID - 10.1007/s11010-023-04710-6 [doi] AB - Cardiac alteration due to chronic kidney disease is described by tissue fibrosis. This remodeling involves myofibroblasts of various origins, including epithelial or endothelial to mesenchymal transitions. In addition, obesity and insulin resistance together or separately seem to exacerbate cardiovascular risk in chronic kidney disease (CKD). The main objective of this study was to assess if pre-existing metabolic disease exacerbates CKD-induced cardiac alterations. In addition, we hypothesised that endothelial to mesenchymal transition participates in this enhancement of cardiac fibrosis. Rats fed cafeteria type diet for 6 months underwent a subtotal nephrectomy at 4 months. Cardiac fibrosis was evaluated by histology and qRT-PCR. Collagens and macrophages were quantified by immunohistochemistry. Endothelial to mesenchymal transitions were assessed by qRT-PCR (CD31, VE-cadherin, α-SMA, nestin) and also by CD31 immunofluorescence staining. Rats fed with cafeteria type regimen were obese, hypertensive and insulin resistant. Cardiac fibrosis was predominant in CKD rats and was highly majored by cafeteria regimen. Collagen-1 and nestin expressions were higher in CKD rats, independently of regimen. Interestingly, in rats with CKD and cafeteria diet we found an increase of CD31 and α-SMA co-staining with suggest an implication of endothelial to mesenchymal transition during heart fibrosis. We showed that rats already obese and insulin resistant had an enhanced cardiac alteration to a subsequent renal injury. Cardiac fibrosis process could be supported by a involvement of the endothelial to mesenchymal transition phenomenon. CI - © 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. FAU - Plawecki, Maëlle AU - Plawecki M AD - PHYMEDEXP, Université de Montpellier, INSERM, CNRS, Montpellier, France. AD - Laboratoire de Biochimie et d'hormonologie, CHU Lapeyronie, Montpellier, France. FAU - Gayrard, Nathalie AU - Gayrard N AD - RD Néphrologie, Montpellier, France. FAU - Jeanson, Laura AU - Jeanson L AD - BC2M, Université de Montpellier, Montpellier, France. FAU - Chauvin, Anthony AU - Chauvin A AD - PHYMEDEXP, Université de Montpellier, INSERM, CNRS, Montpellier, France. FAU - Lajoix, Anne-Dominique AU - Lajoix AD AD - BC2M, Université de Montpellier, Montpellier, France. FAU - Cristol, Jean-Paul AU - Cristol JP AD - PHYMEDEXP, Université de Montpellier, INSERM, CNRS, Montpellier, France. AD - Laboratoire de Biochimie et d'hormonologie, CHU Lapeyronie, Montpellier, France. FAU - Jover, Bernard AU - Jover B AD - PHYMEDEXP, Université de Montpellier, INSERM, CNRS, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - PHYMEDEXP, Université de Montpellier, INSERM, CNRS, Montpellier, France. fabrice.raynaud1@umontpellier.fr. LA - eng PT - Journal Article DEP - 20230328 PL - Netherlands TA - Mol Cell Biochem JT - Molecular and cellular biochemistry JID - 0364456 RN - 0 (Nestin) RN - 0 (Insulins) SB - IM MH - Rats MH - Animals MH - Nestin MH - *Metabolic Syndrome/pathology MH - Ventricular Remodeling MH - *Renal Insufficiency, Chronic/pathology MH - Kidney/pathology MH - Fibrosis MH - Obesity/complications/pathology MH - *Insulins MH - Epithelial-Mesenchymal Transition OTO - NOTNLM OT - Cardiac fibrosis OT - Chronic kidney disease OT - Insulin resistance OT - Mesenchymal transition OT - Obesity EDAT- 2023/03/29 06:00 MHDA- 2024/01/15 12:42 CRDT- 2023/03/28 11:19 PHST- 2022/12/12 00:00 [received] PHST- 2023/03/13 00:00 [accepted] PHST- 2024/01/15 12:42 [medline] PHST- 2023/03/29 06:00 [pubmed] PHST- 2023/03/28 11:19 [entrez] AID - 10.1007/s11010-023-04710-6 [pii] AID - 10.1007/s11010-023-04710-6 [doi] PST - ppublish SO - Mol Cell Biochem. 2024 Jan;479(1):29-39. doi: 10.1007/s11010-023-04710-6. Epub 2023 Mar 28. PMID- 25549148 OWN - NLM STAT- MEDLINE DCOM- 20150417 LR - 20141231 IS - 1543-2165 (Electronic) IS - 0003-9985 (Linking) VI - 139 IP - 1 DP - 2015 Jan TI - The pulmonary histopathology of anti-KS transfer RNA synthetase syndrome. PG - 122-5 LID - 10.5858/arpa.2013-0667-OA [doi] AB - CONTEXT: The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. OBJECTIVE: To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. DESIGN: We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. RESULTS: The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. CONCLUSIONS: Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis. FAU - Schneider, Frank AU - Schneider F AD - From the Departments of Pathology (Drs Schneider and Yousem, Mr Bi), Rheumatology (Drs Aggarwal and Oddis), and Pulmonary Medicine (Dr Gibson), UPMC Presbyterian, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. FAU - Aggarwal, Rohit AU - Aggarwal R FAU - Bi, David AU - Bi D FAU - Gibson, Kevin AU - Gibson K FAU - Oddis, Chester AU - Oddis C FAU - Yousem, Samuel A AU - Yousem SA LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Arch Pathol Lab Med JT - Archives of pathology & laboratory medicine JID - 7607091 RN - 0 (Autoantibodies) RN - 0 (RNA, Transfer, Amino Acyl) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - EC 6.1.1.12 (Aspartate-tRNA Ligase) RN - EC 6.1.1.22 (asparaginyl-tRNA synthetase) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Amino Acyl-tRNA Synthetases/*immunology MH - Aspartate-tRNA Ligase/immunology MH - Autoantibodies/immunology MH - Diagnosis, Differential MH - Female MH - Humans MH - Lung/immunology/*pathology MH - Lung Diseases, Interstitial/*diagnosis/immunology MH - Male MH - Middle Aged MH - Myositis/*diagnosis/immunology MH - RNA, Transfer, Amino Acyl/immunology MH - Registries MH - Retrospective Studies MH - Review Literature as Topic EDAT- 2014/12/31 06:00 MHDA- 2015/04/18 06:00 CRDT- 2014/12/31 06:00 PHST- 2014/12/31 06:00 [entrez] PHST- 2014/12/31 06:00 [pubmed] PHST- 2015/04/18 06:00 [medline] AID - 10.5858/arpa.2013-0667-OA [doi] PST - ppublish SO - Arch Pathol Lab Med. 2015 Jan;139(1):122-5. doi: 10.5858/arpa.2013-0667-OA. PMID- 33163874 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201112 IS - 2529-198X (Electronic) IS - 2529-198X (Linking) VI - 31 IP - 3 DP - 2020 Sep TI - Peripheral Microangiopathy in Patients with Precapillary Pulmonary Hypertension: Correlation with Cardiac Function and Patients' Functional Capacity. Study Design and Rationale. PG - 369-373 LID - 10.31138/mjr.31.3.369 [doi] AB - Pulmonary hypertension (PH) is a rare, heterogenous clinical entity characterised by a progressive remodelling of pulmonary arterioles, which leads to obstructive pulmonary arteriopathy, increased pulmonary vascular resistance, and eventually, right heart failure. Inflammation, endothelial dysfunction, and microvascular changes of the pulmonary vasculature constitute the hallmarks of pulmonary arterial hypertension (PAH), explaining much of the pathophysiology and clinical manifestations of the disease. Besides pulmonary vasculature, a systemic component of endothelial dysfunction and microcirculation may be involved in PAH, affecting different vascular beds. Nailfold videocapillaroscopy (NVC) is an established method for the assessment of the microvasculature with clinical implications in the diagnostic assessment of individuals with Raynaud syndrome and systemic sclerosis (SSc). Nowadays, growing amounts of evidence suggest that NVC changes in SSc are correlated with other vascular complications such as PAH, supporting a potential link between peripheral and internal organ vasculopathy. The purpose of the current prospective observational study is to explore: 1. the presence of peripheral microangiopathy in precapillary PH using NVC, 2. possible NVC differences among PH subgroups, 3. a potential relationship between NVC morphological abnormalities and clinical, functional, biochemical, echocardiographic and hemodynamic markers of cardiac dysfunction in precapillary PH. CI - © 2020 The Mediterranean Journal of Rheumatology (MJR). FAU - Arvanitaki, Alexandra AU - Arvanitaki A AD - Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, Greece. AD - First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece. AD - Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany. FAU - Giannakoulas, George AU - Giannakoulas G AD - First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece. FAU - Triantafyllidou, Eva AU - Triantafyllidou E AD - Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, Greece. FAU - Karvounis, Haralambos AU - Karvounis H AD - First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece. FAU - Dimitroulas, Theodoros AU - Dimitroulas T AD - Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, Greece. LA - eng PT - Journal Article DEP - 20200610 PL - Greece TA - Mediterr J Rheumatol JT - Mediterranean journal of rheumatology JID - 101730166 PMC - PMC7641020 OTO - NOTNLM OT - Peripheral microangiopathy OT - nailfold videocapillaroscopy OT - precapillary pulmonary hypertension OT - pulmonary arterial hypertension OT - systemic sclerosis EDAT- 2020/11/10 06:00 MHDA- 2020/11/10 06:01 PMCR- 2020/06/10 CRDT- 2020/11/09 05:35 PHST- 2020/02/10 00:00 [received] PHST- 2020/04/17 00:00 [revised] PHST- 2020/04/20 00:00 [accepted] PHST- 2020/11/09 05:35 [entrez] PHST- 2020/11/10 06:00 [pubmed] PHST- 2020/11/10 06:01 [medline] PHST- 2020/06/10 00:00 [pmc-release] AID - MJR-31-3-369 [pii] AID - 10.31138/mjr.31.3.369 [doi] PST - epublish SO - Mediterr J Rheumatol. 2020 Jun 10;31(3):369-373. doi: 10.31138/mjr.31.3.369. eCollection 2020 Sep. PMID- 33048480 STAT- Publisher PB - Canadian Agency for Drugs and Technologies in Health CTI - CADTH Rapid Response Reports DP - 2020 Jan 21 BTI - Pilocarpine for Sjögren’s Syndrome-Induced Dry Mouth and Dry Eyes: A Review of Clinical Effectiveness, Cost-Effectiveness, and Guidelines AB - Sjögren’s syndrome is an autoimmune disease most frequently affecting women between 30 and 50 years of age. Sjögren’s syndrome is often under-diagnosed but may affect up to 430,000 Canadians. The cause of Sjögren’s syndrome is currently unknown, however, one prevalent theory is that certain genetic factors coupled with an environmental stimulus (i.e., a virus) triggers the disease.(,) There are two main classifications of Sjögren’s syndrome: primary and secondary. Patients are classified as having primary Sjögren’s syndrome when there is no other autoimmune disease present. Patients are classified as having secondary Sjögren’s syndrome when another autoimmune disorder, such as rheumatoid arthritis, systemic lupus erythematosus or systemic sclerosis, is also present. Both types of Sjögren’s syndrome are characterized by damage to exocrine glands such as the salivary, tear and mucous-secreting glands, which can result in dry eyes and dry mouth.(,) Dry eyes can cause discomfort via a scratchy and gritty sensation. In rare, severe cases, vision impairment may occur due to damage to the corneal surface. Dry mouth occurs secondary to a diminished saliva production and can cause difficulty chewing and swallowing, Candida infections, tooth decay and sialolithiasis. Both dry eyes and dry mouth can be managed with a variety of non-pharmacological and non-prescription therapies. Pharmacological therapy with muscarinic agonists (i.e., pilocarpine, cevimeline) which stimulate exocrine glands, can also be used to alleviate the symptoms of dry eyes and dry mouth. Other symptoms of Sjögren’s syndrome may include extraglandular manifestations such as lymphadenopathy, Raynaud phenomenon, and vasculitis. This report aims to summarize the evidence regarding the clinical effectiveness, cost-effectiveness and evidence-based guidelines’ recommendations for the use of pilocarpine in the treatment of Sjögren’s syndrome-induced dry eyes and dry mouth. CI - Copyright © 2020 Canadian Agency for Drugs and Technologies in Health. FAU - Freige, Christopher AU - Freige C FAU - Ford, Caitlyn AU - Ford C LA - eng PT - Review PT - Book PL - Ottawa (ON) EDAT- 2020/01/21 00:00 CRDT- 2020/01/21 00:00 AID - NBK562832 [bookaccession] PMID- 29515069 OWN - NLM STAT- MEDLINE DCOM- 20190211 LR - 20190215 IS - 0485-1439 (Print) IS - 0485-1439 (Linking) VI - 59 IP - 2 DP - 2018 TI - [Mixed connective tissue disease with pulmonary hypertension developing in a chronic myeloid leukemia patient on dasatinib treatment]. PG - 174-177 LID - 10.11406/rinketsu.59.174 [doi] AB - A 37-year-old woman was diagnosed with chronic phase chronic myeloid leukemia. Nilotinib treatment was initiated; however, it had to be discontinued due to an allergic reaction one month later, and dasatinib treatment was provided. Although favorable response was obtained, she started complaining of shortness of breath 7 months after initiating dasatinib treatment. Chest X-ray and echocardiography indicated pulmonary congestion and hypertension. Further, she was diagnosed with mixed connective tissue disease (MCTD) based on Raynaud phenomenon, swollen fingers, sclerodactyly, pancytopenia, hypocomplementemia, and positive anti-U1-RNP antibody. Consequently, dasatinib treatment was discontinued, and she was administered prednisolone (1 mg/kg/day), which was effective and successfully tapered with concomitant administration of cyclophosphamide. This is the first case of MCTD that developed during dasatinib treatment. However, because the present case was a young woman, the development of MCTD could probably be attributed to autoimmune diatheses or it may be a coincidence. However, the possibility of patients receiving dasatinib treatment developing autoimmune diseases needs to be assessed. FAU - Watanuki, Shintaro AU - Watanuki S AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Kikuchi, Taku AU - Kikuchi T AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Toyama, Takaaki AU - Toyama T AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Abe, Ryohei AU - Abe R AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Nakayama, Hitomi AU - Nakayama H AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Karigane, Daiki AU - Karigane D AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Shimizu, Takayuki AU - Shimizu T AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Kikuchi, Jun AU - Kikuchi J AD - Division of Rheumatology, Department of Medicine, Keio University School of Medicine. FAU - Matsumoto, Kotaro AU - Matsumoto K AD - Division of Rheumatology, Department of Medicine, Keio University School of Medicine. FAU - Yasuoka, Hidetaka AU - Yasuoka H AD - Division of Rheumatology, Department of Medicine, Keio University School of Medicine. FAU - Kataoka, Masaharu AU - Kataoka M AD - Division of Cardiology, Department of Medicine, Keio University School of Medicine. FAU - Okamoto, Shinichiro AU - Okamoto S AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. FAU - Mori, Takehiko AU - Mori T AD - Division of Hematology, Department of Medicine, Keio University School of Medicine. LA - jpn PT - Case Reports PT - Journal Article PL - Japan TA - Rinsho Ketsueki JT - [Rinsho ketsueki] The Japanese journal of clinical hematology JID - 2984782R RN - RBZ1571X5H (Dasatinib) SB - IM MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Dasatinib/*adverse effects/therapeutic use MH - Female MH - Humans MH - Hypertension, Pulmonary/*chemically induced MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*drug therapy MH - Mixed Connective Tissue Disease/*chemically induced MH - Treatment Outcome OTO - NOTNLM OT - Chronic myeloid leukemia OT - Dasatinib OT - Mixed connective tissue disease OT - Pulmonary hypertension EDAT- 2018/03/09 06:00 MHDA- 2019/02/12 06:00 CRDT- 2018/03/09 06:00 PHST- 2018/03/09 06:00 [entrez] PHST- 2018/03/09 06:00 [pubmed] PHST- 2019/02/12 06:00 [medline] AID - 10.11406/rinketsu.59.174 [doi] PST - ppublish SO - Rinsho Ketsueki. 2018;59(2):174-177. doi: 10.11406/rinketsu.59.174. PMID- 31725486 OWN - NLM STAT- MEDLINE DCOM- 20201118 LR - 20210202 IS - 1533-0311 (Electronic) IS - 0193-1091 (Linking) VI - 42 IP - 2 DP - 2020 Feb TI - Fibroblastic Rheumatism Versus Variant Disease of Multinucleate Cell Angiohistiocytoma. PG - 136-139 LID - 10.1097/DAD.0000000000001550 [doi] AB - We report an unusual case of a 49-year-old woman who presented with persistent papulonodules over bilateral fingers and inframammary region in conjunction with features of connective tissue disease including symmetrical polyarthritis and Raynaud phenomenon. Skin biopsy showed an upper-to-mid dermal proliferation of bland spindled cells with thickened collagen bundles and occasional multinucleated giant cells. Dermal blood vessels were only marginally increased. On immunohistochemistry, both the spindled cells and multinucleated giant cells stained negatively for smooth muscle actin. Some of the spindled cells stained positively with CD68 and CD163, whereas the multinucleated giant cells stained negatively for both stains. Elastic fibers were absent on elastic Van Gieson. The clinical and histopathologic features raise a diagnostic dilemma between fibroblastic rheumatism and multinucleate cell angiohistiocytoma. The patient responded well to cyclosporine and methotrexate therapy, with gradual improvement of the finger nodules. FAU - Cai, Sophie Carrie Shan AU - Cai SCS AD - National Skin Centre, Singapore. FAU - Tee, Shang Ian AU - Tee SI FAU - Lee, Joyce Siong See AU - Lee JSS FAU - Tan, Lucinda Siyun AU - Tan LS LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Dermatopathol JT - The American Journal of dermatopathology JID - 7911005 RN - 0 (Antirheumatic Agents) RN - 0 (Immunosuppressive Agents) RN - 83HN0GTJ6D (Cyclosporine) RN - YL5FZ2Y5U1 (Methotrexate) SB - IM MH - Antirheumatic Agents/therapeutic use MH - Cyclosporine/therapeutic use MH - Diagnosis, Differential MH - Female MH - Fibroblasts/*pathology MH - Giant Cells/*pathology MH - Hemangioma/diagnosis/drug therapy/pathology MH - Histiocytoma/diagnosis/drug therapy/pathology MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Methotrexate/therapeutic use MH - Middle Aged MH - Rheumatic Diseases/*diagnosis/drug therapy/pathology MH - Skin Diseases/*diagnosis/drug therapy/pathology MH - Skin Neoplasms/*diagnosis/drug therapy/pathology EDAT- 2019/11/15 06:00 MHDA- 2020/11/20 06:00 CRDT- 2019/11/15 06:00 PHST- 2019/11/15 06:00 [pubmed] PHST- 2020/11/20 06:00 [medline] PHST- 2019/11/15 06:00 [entrez] AID - 00000372-202002000-00011 [pii] AID - 10.1097/DAD.0000000000001550 [doi] PST - ppublish SO - Am J Dermatopathol. 2020 Feb;42(2):136-139. doi: 10.1097/DAD.0000000000001550. PMID- 40258255 OWN - NLM STAT- MEDLINE DCOM- 20250421 LR - 20260609 IS - 0363-6771 (Print) IS - 0363-6771 (Linking) VI - 73 IP - 3 DP - 2025 May-Jun TI - A clinical guide to oral manifestations and diagnosis of limited systemic sclerosis: a case report. PG - 42-45 AB - Scleroderma is a rare connective tissue disease involving vascular injury and autoimmunity. It is characterized by the thickening, hardening, and tightening of connective tissues, leading to multifaceted complications. Scleroderma is classified into 2 forms, localized scleroderma and systemic sclerosis (SSc). The systemic form is further subdivided into limited SSc (formerly known as CREST syndrome) or diffuse SSc. This report describes the case of a 41-year-old woman with gingival pain, temporomandibular joint pain, frequent fevers, night sweats, excessive thirst, hunger, frequent urination, and weight gain. Her medical history included Sjögren syndrome and Raynaud phenomenon. Physical examinations revealed petechiae on the palms, lips, tongue, and soft palate; hypochromia of the lips; fibrosis; dry mouth; and active periodontal disease. Serologic test results were positive for anticentromere and antinuclear antibodies and negative for anti-topoisomerase I antibody, confirming the diagnosis of limited SSc. The oral manifestations were critical to the early diagnosis of limited SSc. Telangiectasia, labial fibrosis, and xerostomia were key indicators. Interdisciplinary collaboration between oral healthcare professionals and rheumatologists is essential for optimal patient management. Further research is needed to understand the underlying mechanisms of SSc and refine the diagnostic criteria. FAU - Soto, Vanessa Carvajal AU - Soto VC FAU - Ranthum, Larissa Knysak AU - Ranthum LK FAU - Rosa, Helen Heloene AU - Rosa HH FAU - Campagnoli, Eduardo Bauml AU - Campagnoli EB FAU - Bortoluzzi, Marcelo Carlos AU - Bortoluzzi MC LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Gen Dent JT - General dentistry JID - 7610466 SB - IM MH - Humans MH - Female MH - Adult MH - *Mouth Diseases/diagnosis/etiology MH - *Scleroderma, Limited/diagnosis/complications MH - *Scleroderma, Systemic/diagnosis MH - Diagnosis, Differential MH - Xerostomia/etiology OTO - NOTNLM OT - early diagnosis OT - fibrosis OT - oral manifestations OT - connective tissue OT - scleroderma OT - systemic sclerosis COIS- No conflicts of interest reported. EDAT- 2025/04/21 18:27 MHDA- 2025/04/22 00:26 CRDT- 2025/04/21 16:42 PHST- 2025/04/22 00:26 [medline] PHST- 2025/04/21 18:27 [pubmed] PHST- 2025/04/21 16:42 [entrez] PST - ppublish SO - Gen Dent. 2025 May-Jun;73(3):42-45. PMID- 37476427 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230722 IS - 2233-4718 (Electronic) IS - 2093-940X (Print) IS - 2233-4718 (Linking) VI - 29 IP - 4 DP - 2022 Oct 1 TI - Adenosine Deaminase 2 Deficiency Caused by Biallele Variants Including Splicing Variant: The First Case in Korea. PG - 254-260 LID - 10.4078/jrd.21.0046 [doi] AB - Deficiency of adenosine deaminase 2 (DADA2) is an autoinflammatory disease caused by pathogenic variants of the ADA2 gene and has similar clinical features to polyarteritis nodosa (PAN). We, herein, report a case of DADA2 in Korea that was diagnosed in a patient with childhood-onset PAN. The patient had a truncal ataxia and facial palsy caused by thalamic infarction at 34 months of age. Livedo reticularis with Raynaud phenomenon and abdominal pain with fever were followed. Radiologic examination showed multiple infarctions in brain and kidney. She was diagnosed with PAN using skin biopsy and angiography. She had severe hemorrhagic strokes despite medical treatments. Her disease activity was controlled after adding a tumor necrosis factor-α inhibitor. Molecular analysis revealed compound heterozygous pathogenic variants of ADA2 gene. This is the first case of DADA2 in Korea. Genetic analysis for ADA2 gene should be considered in patients with childhood-onset PAN. CI - Copyright © 2022 by The Korean College of Rheumatology. All rights reserved. FAU - Cho, Sun AU - Cho S AUID- ORCID: 0000-0002-4783-282X AD - Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. FAU - Park, Seongyeol AU - Park S AUID- ORCID: 0000-0002-9236-5853 AD - GENOME INSIGHT Inc., Daejeon, Korea. FAU - Lee, Jeong Seok AU - Lee JS AUID- ORCID: 0000-0001-8261-7044 AD - GENOME INSIGHT Inc., Daejeon, Korea. FAU - Ju, Young Seok AU - Ju YS AUID- ORCID: 0000-0002-5514-4189 AD - GENOME INSIGHT Inc., Daejeon, Korea. AD - Graduate School of Medical Science and Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea. FAU - Choi, Yun Jung AU - Choi YJ AUID- ORCID: 0000-0002-0078-8374 AD - Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. AD - Hospital Medicine Center, Seoul National University Hospital, Seoul, Korea. FAU - Lee, Soyoung AU - Lee S AUID- ORCID: 0000-0001-9627-6607 AD - Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. AD - GENOME INSIGHT Inc., Daejeon, Korea. LA - eng PT - Case Reports PT - Journal Article DEP - 20220705 PL - Korea (South) TA - J Rheum Dis JT - Journal of rheumatic diseases JID - 101571816 PMC - PMC10351412 OTO - NOTNLM OT - ADA2 deficiency OT - ADA2 gene OT - Deficiency of adenosine deaminase 2 OT - Polyarteritis nodosa COIS- CONFLICT OF INTEREST No potential conflict of interest relevant to this article was reported. EDAT- 2023/07/21 06:44 MHDA- 2023/07/21 06:45 PMCR- 2022/07/05 CRDT- 2023/07/21 04:05 PHST- 2021/09/27 00:00 [received] PHST- 2021/10/30 00:00 [revised] PHST- 2021/11/04 00:00 [accepted] PHST- 2023/07/21 06:45 [medline] PHST- 2023/07/21 06:44 [pubmed] PHST- 2023/07/21 04:05 [entrez] PHST- 2022/07/05 00:00 [pmc-release] AID - jrd-29-4-254 [pii] AID - 10.4078/jrd.21.0046 [doi] PST - ppublish SO - J Rheum Dis. 2022 Oct 1;29(4):254-260. doi: 10.4078/jrd.21.0046. Epub 2022 Jul 5. PMID- 23112358 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20121101 LR - 20211021 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 57 IP - 5 DP - 2012 Sep TI - Clinical presentation and evaluation of dermatomyositis. PG - 375-81 LID - 10.4103/0019-5154.100486 [doi] AB - Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50-70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron's papules, Gottron's sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, "mechanic's hands", palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35-40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM. FAU - Marvi, Umaima AU - Marvi U AD - Division of Immunology and Rheumatology, Stanford University, USA. FAU - Chung, Lorinda AU - Chung L FAU - Fiorentino, David F AU - Fiorentino DF LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC3482801 OTO - NOTNLM OT - Cutaneous manifestations OT - dermatomyositis OT - diagnosis COIS- Conflict of Interest: Nil. EDAT- 2012/11/01 06:00 MHDA- 2012/11/01 06:01 PMCR- 2012/09/01 CRDT- 2012/11/01 06:00 PHST- 2012/11/01 06:00 [entrez] PHST- 2012/11/01 06:00 [pubmed] PHST- 2012/11/01 06:01 [medline] PHST- 2012/09/01 00:00 [pmc-release] AID - IJD-57-375 [pii] AID - 10.4103/0019-5154.100486 [doi] PST - ppublish SO - Indian J Dermatol. 2012 Sep;57(5):375-81. doi: 10.4103/0019-5154.100486. PMID- 38362287 OWN - NLM STAT- MEDLINE DCOM- 20240219 LR - 20250918 IS - 1995-7270 (Electronic) IS - 1995-7262 (Print) IS - 1995-7262 (Linking) VI - 35 IP - 3 DP - 2023 Sep TI - The validity and reliability of the Turkish version of the Brief Fear of Negative Evaluation Scale - straight forwardly in patients with systemic sclerosis. PG - 163-169 LID - 10.4314/mmj.v35i3.5 [doi] AB - OBJECTIVE: This study aimed to examine the validity and reliability of Turkish version of Brief Fear of Negative Evaluation Scale-Straightforwardly (BFNE-S (TR)) in patients with Systemic Sclerosis (SSc). MATERIALS AND METHODS: 35 individuals (mean age: 53.3±13.0 years) diagnosed as SSc were included. Data on demographics, were collected via structured interview. All participants were evaluated by same investigator. The disability was evaluated with Scleroderma Health Assessment Questionnaire (SHAQ), disease severity with Medsger's Disease Severity Scale, and skin involvement with Modified Rodnan Skin Score. BFNE-S (TR) was applied to the patients with SSc who did not receive any treatment for test retest at one-week intervals. RESULTS: The one-factor structure was provided for all indices except Chi-Square. Factor loadings were significant. The patient responses to the BFNE-S (TR) demonstrated excellent internal consistency (Cronbach's α: 0.95). The floor effect (20%) percentage of patients who scored at floor level, was observed. Test-retest reliability of the scale was excellent with 0.91 (95%CI: 0.78-0.96). BFNE-S (TR) total score had positive correlation with SHAQ_Digestive (r=0.503) and SHAQ_Raynaud phenomenon (r=0.343)(p<0.05). CONCLUSIONS: The BFNE-S (TR) is a reliable and valid scale and can be used for measurement of fear of negative evaluation in SSc. CI - © 2023 Kamuzu University of Health Sciences. FAU - Kabul, Elif Gur AU - Kabul EG AD - Faculty of Health Sciences, Physiotherapy and Rehabilitation, Usak University, Usak, Turkey. FAU - Demir, Pervin AU - Demir P AD - Department of Biostatistics and Medical Informatics, Ankara Yıldırım Beyazıt University, Ankara, Turkey. FAU - Ulutas, Firdevs AU - Ulutas F AD - Department of Rheumatology, Medical Faculty, Pamukkale University, Denizli, Turkey. FAU - Yenil, Sinem AU - Yenil S AD - Faculty of Physiotherapy and Rehabilitation, Pamukkale University, Denizli, Turkey. FAU - Calik, Bilge Basakci AU - Calik BB AD - Faculty of Physiotherapy and Rehabilitation, Pamukkale University, Denizli, Turkey. FAU - Cobankara, Veli AU - Cobankara V AD - Department of Rheumatology, Medical Faculty, Pamukkale University, Denizli, Turkey. LA - eng PT - Journal Article PL - Malawi TA - Malawi Med J JT - Malawi medical journal : the journal of Medical Association of Malawi JID - 9500170 SB - IM MH - Humans MH - Adult MH - Middle Aged MH - Aged MH - Reproducibility of Results MH - Surveys and Questionnaires MH - *Fear MH - *Scleroderma, Systemic/diagnosis PMC - PMC10865058 OTO - NOTNLM OT - Systemic Sclerosis OT - disease severity OT - fear OT - questionnaire OT - skin disease EDAT- 2024/02/16 06:43 MHDA- 2024/02/19 06:42 PMCR- 2023/09/01 CRDT- 2024/02/16 03:56 PHST- 2024/02/19 06:42 [medline] PHST- 2024/02/16 06:43 [pubmed] PHST- 2024/02/16 03:56 [entrez] PHST- 2023/09/01 00:00 [pmc-release] AID - 10.4314/mmj.v35i3.5 [doi] PST - ppublish SO - Malawi Med J. 2023 Sep;35(3):163-169. doi: 10.4314/mmj.v35i3.5. PMID- 26087970 OWN - NLM STAT- MEDLINE DCOM- 20160615 LR - 20150828 IS - 1873-2496 (Electronic) IS - 1078-1439 (Linking) VI - 33 IP - 9 DP - 2015 Sep TI - Quality of life among testis cancer survivors. PG - 413-9 LID - S1078-1439(15)00242-2 [pii] LID - 10.1016/j.urolonc.2015.05.018 [doi] AB - BACKGROUND: As the most common cancer among males in late adolescence and early adulthood and as a disease with a 5-year relative survival rate of 96%, testis cancer has many survivors who live many years during which chronic toxicities may impair their quality of life. METHODS: In this review, I aimed to summarize the most relevant literature on quality of life among testis cancer survivors identified via PubMed literature search between 1990 and 2015. RESULTS: Survivors of testis cancer experience an overall quality of life (QOL) that is not measurably different from that of men of the same age in the general population. Nonetheless, testis cancer and its treatments can result in a variety of long-term conditions that affect QOL. These include peripheral neuropathy, hearing loss, tinnitus, fatigue, and Raynaud-like phenomenon. Exercise interventions have been shown to improve fatigue and overall QOL in cancer survivors, and there is evidence that psychosocial and mind-body interventions may also be beneficial. Pharmacological interventions have not been shown to be helpful for cancer-related fatigue, hearing loss, or neuropathy. CONCLUSIONS: Testis cancer survivors should be asked about symptoms related to the conditions above and referred to specialists as indicated. Survivors complaining of fatigue should be encouraged to adopt a regular program of aerobic exercise. CI - Copyright © 2015 Elsevier Inc. All rights reserved. FAU - Gilligan, Timothy AU - Gilligan T AD - Cleveland Clinic Taussig Cancer Institute, Cleveland, OH. Electronic address: gilligt@ccf.org. LA - eng PT - Journal Article PT - Review DEP - 20150615 PL - United States TA - Urol Oncol JT - Urologic oncology JID - 9805460 SB - IM MH - Humans MH - Male MH - *Quality of Life MH - Survivors/*psychology MH - Testicular Neoplasms/complications/*psychology OTO - NOTNLM OT - Fatigue OT - Germ cell tumors OT - Neuropathy OT - Quality of life OT - Survivors OT - Survivorship OT - Testis cancer EDAT- 2015/06/20 06:00 MHDA- 2016/06/16 06:00 CRDT- 2015/06/20 06:00 PHST- 2015/01/05 00:00 [received] PHST- 2015/05/10 00:00 [revised] PHST- 2015/05/13 00:00 [accepted] PHST- 2015/06/20 06:00 [entrez] PHST- 2015/06/20 06:00 [pubmed] PHST- 2016/06/16 06:00 [medline] AID - S1078-1439(15)00242-2 [pii] AID - 10.1016/j.urolonc.2015.05.018 [doi] PST - ppublish SO - Urol Oncol. 2015 Sep;33(9):413-9. doi: 10.1016/j.urolonc.2015.05.018. Epub 2015 Jun 15. PMID- 28503297 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220311 IS - 2046-1402 (Print) IS - 2046-1402 (Electronic) IS - 2046-1402 (Linking) VI - 6 DP - 2017 TI - Cardiovascular involvement and manifestations of systemic Chikungunya virus infection: A systematic review. PG - 390 LID - 10.12688/f1000research.11078.2 [doi] LID - 390 AB - BACKGROUND: In the last three years, chikungunya virus disease has been spreading, affecting particularly the Americas, producing more than two million cases. In this setting, not only new disease-related epidemiological patterns have been found, but also new clinical findings have been reported by different research groups. These include findings on the cardiovascular system, including clinical, electrocardiographic and echocardiographic alterations. No previous systemic reviews have been found in major databases about it. METHODS: We performed a systematic review looking for reports about cardiovascular compromise during chikungunya disease. Cardiac compromise is not so common in isolated episodes; but countries where chikungunya virus is an epidemic should be well informed about this condition. We used 6 bibliographical databases as resources: Medline/Pubmed, Embase, ScienceDirect, ClinicalKey, Ovid and SciELO. Dengue reports on cardiovascular compromise were included as well, to compare both arbovirus' organic compromises. Articles that delved mainly into the rheumatic articular and cutaneous complications were not considered, as they were not in line with the purpose of this study. The type of articles included were reviews, meta-analyses, case-controls, cohort studies, case reports and case series. This systematic review does not reach or performed a meta-analysis. RESULTS: Originally based on 737 articles, our reviewed selected 40 articles with 54.2% at least mentioning CHIKV cardiovascular compromise within the systemic compromise. Cardiovascular manifestations can be considered common and have been reported in France, India, Sri Lanka, Malaysia, Colombia, Venezuela and USA, including mainly, but no limited to: hypotension, shock and circulatory collapse, Raynaud phenomenon, arrhythmias, murmurs, myocarditis, dilated cardiomyopathy, congestive insufficiency, heart failure and altered function profile (Troponins, CPK). CONCLUSIONS: Physicians should be encouraged to keep divulgating reports on the cardiovascular involvement of chikungunya virus disease, to raise awareness and ultimately encourage suitable diagnosis and intervention worldwide. More research about cardiovascular involvement and manifestations of systemic Chikungunya virus infection is urgently needed. FAU - Alvarez, María Fernanda AU - Alvarez MF AD - Faculty of Health, Universidad Industrial de Santander, Santander, Colombia. FAU - Bolívar-Mejía, Adrián AU - Bolívar-Mejía A AD - Department of Internal Medicine, Universidad Industrial de Santander, Santander, Colombia. FAU - Rodriguez-Morales, Alfonso J AU - Rodriguez-Morales AJ AUID- ORCID: 0000-0001-9773-2192 AD - Public Health and Infection Research Group, Faculty of Health Sciences, Universidad Tecnológica de Pereira, Pereira, Risaralda, Colombia. AD - Colombian Collaborative Network on Zika and other Arboviruses (RECOLZIKA), Pereira, Risaralda, Colombia. FAU - Ramirez-Vallejo, Eduardo AU - Ramirez-Vallejo E AD - Public Health and Infection Research Group, Faculty of Health Sciences, Universidad Tecnológica de Pereira, Pereira, Risaralda, Colombia. AD - Colombian Collaborative Network on Zika and other Arboviruses (RECOLZIKA), Pereira, Risaralda, Colombia. LA - eng PT - Journal Article DEP - 20170329 PL - England TA - F1000Res JT - F1000Research JID - 101594320 PMC - PMC5405794 OTO - NOTNLM OT - Chikungunya OT - Colombia OT - Latin America OT - cardiovascular OT - clinical COIS- Competing interests: No competing interests were disclosed. EDAT- 2017/05/20 06:00 MHDA- 2017/05/20 06:01 PMCR- 2017/05/02 CRDT- 2017/05/20 06:00 PHST- 2017/04/27 00:00 [accepted] PHST- 2017/05/20 06:00 [entrez] PHST- 2017/05/20 06:00 [pubmed] PHST- 2017/05/20 06:01 [medline] PHST- 2017/05/02 00:00 [pmc-release] AID - 10.12688/f1000research.11078.2 [doi] PST - epublish SO - F1000Res. 2017 Mar 29;6:390. doi: 10.12688/f1000research.11078.2. eCollection 2017. PMID- 40131427 OWN - NLM STAT- MEDLINE DCOM- 20250801 LR - 20260526 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - 8 DP - 2025 Aug 1 TI - Prostaglandin E1 restores endothelial progenitor cell function in systemic sclerosis. PG - 4761-4765 LID - 10.1093/rheumatology/keaf174 [doi] AB - OBJECTIVES: SSc is a chronic autoimmune disease characterized by microvascular injury and impaired angiogenesis, with endothelial progenitor cells (EPCs) playing a key role in vascular repair. EPC subsets, including endothelial colony-forming cells (ECFCs) and colony-forming unit-endothelial cells (CFU-ECs), are known to be dysfunctional in SSc, contributing to disease-associated vasculopathy. Prostaglandin E1 (PGE1) is a vasodilator with potential pro-angiogenic effects, but its impact on EPC numbers and function in SSc remains unexplored. This study aimed to investigate whether PGE1 treatment can modulate EPC numbers, specifically CFU-ECs and ECFCs, in patients with SSc and RP, and evaluate its potential role in promoting vascular repair. METHODS: This study evaluated the effect of PGE1 on EPC levels in 12 SSc patients with RP and five healthy controls (HCs). CFU-EC and ECFC clusters were quantified before and after PGE1 treatment using standardized culture methods. PGE1 was administered intravenously over 8-9 days. Statistical analyses compared EPC counts between the groups and time points. RESULTS: Baseline CFU-EC and ECFC cluster counts were significantly reduced in SSc patients compared with HCs (P = 0.02 and P < 0.01, respectively). Following PGE1 treatment, both CFU-EC and ECFC clusters significantly increased in SSc patients (P = 0.02 and P = 0.001, respectively), reaching levels comparable to HCs. No significant changes were observed in HCs across two time points. A significant delta in cluster counts was observed in SSc patients vs. HCs (CFU-EC: P = 0.03; ECFC: P = 0.01). CONCLUSION: PGE1 treatment restores CFU-EC and ECFC levels in SSc patients, suggesting a potential role in repairing vascular damage. These findings highlight PGE1's therapeutic benefits beyond vasodilation, supporting its use in SSc-associated microvasculopathy. CI - © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Potjewijd, Judith AU - Potjewijd J AD - Department of Internal Medicine, Division of Clinical & Experimental Immunology, CARIM, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Tobal, Rachid AU - Tobal R AD - Department of Internal Medicine, Division of Clinical & Experimental Immunology, CARIM, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Arends, Steven J AU - Arends SJ AD - Department of Internal Medicine, Division of Clinical & Experimental Immunology, CARIM, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Debrus-Palmans, Lucienne AU - Debrus-Palmans L AD - Department of Internal Medicine, Division of Clinical & Experimental Immunology, CARIM, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Damoiseaux, Jan G M C AU - Damoiseaux JGMC AUID- ORCID: 0000-0003-4007-6985 AD - Central Diagnostic Laboratory, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - van Paassen, Pieter AU - van Paassen P AD - Department of Internal Medicine, Division of Clinical & Experimental Immunology, CARIM, Maastricht University Medical Center, Maastricht, The Netherlands. LA - eng GR - Actelion Pharmaceuticals Nederland/ PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - F5TD010360 (Alprostadil) RN - 0 (Vasodilator Agents) SB - IM MH - Humans MH - *Endothelial Progenitor Cells/drug effects/physiology MH - Female MH - *Scleroderma, Systemic/drug therapy/physiopathology/pathology MH - *Alprostadil/pharmacology/therapeutic use MH - Male MH - Middle Aged MH - Adult MH - *Vasodilator Agents/pharmacology/therapeutic use MH - Case-Control Studies MH - Aged MH - Cells, Cultured PMC - PMC12316368 OTO - NOTNLM OT - Raynaud OT - SSc OT - endothelial cells OT - endothelial progenitor cells OT - prostaglandin E1 OT - vascular repair EDAT- 2025/03/25 18:28 MHDA- 2025/08/01 18:34 PMCR- 2025/03/25 CRDT- 2025/03/25 12:14 PHST- 2024/12/01 00:00 [received] PHST- 2025/03/16 00:00 [accepted] PHST- 2025/08/01 18:34 [medline] PHST- 2025/03/25 18:28 [pubmed] PHST- 2025/03/25 12:14 [entrez] PHST- 2025/03/25 00:00 [pmc-release] AID - 8093285 [pii] AID - keaf174 [pii] AID - 10.1093/rheumatology/keaf174 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Aug 1;64(8):4761-4765. doi: 10.1093/rheumatology/keaf174. PMID- 39242112 OWN - NLM STAT- MEDLINE DCOM- 20240906 LR - 20241205 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 10 IP - 3 DP - 2024 Sep 5 TI - Development of a multivariable prediction model for progression of systemic sclerosis-associated interstitial lung disease. LID - 10.1136/rmdopen-2024-004240 [doi] LID - e004240 AB - OBJECTIVE: To develop a multivariable model for predicting the progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD) over 52 weeks. METHODS: We used logistic regression models to analyse associations between candidate predictors assessed at baseline and progression of SSc-ILD (absolute decline in forced vital capacity (FVC) % predicted >5% or death) over 52 weeks in the placebo group of the SENSCIS trial. Analyses were performed in the overall placebo group and in a subgroup with early and/or inflammatory SSc and/or severe skin fibrosis (<18 months since first non-Raynaud symptom, elevated inflammatory markers, and/or modified Rodnan skin score (mRSS) >18) at baseline. Model performance was assessed using the area under the receiver operating characteristic curve (AUC). RESULTS: In the overall placebo group (n=288), the performance of the final multivariable model for predicting SSc-ILD progression was moderate (apparent AUC: 0.63). A stronger model, with an apparent AUC of 0.75, was developed in the subgroup with early and/or inflammatory SSc and/or severe skin fibrosis at baseline (n=155). This model included diffusing capacity of the lung for carbon monoxide (DLco) % predicted, time since first non-Raynaud symptom, mRSS, anti-topoisomerase I antibody status and mycophenolate use. CONCLUSION: Prediction of the progression of SSc-ILD may require different approaches in distinct subgroups of patients. Among patients with SSc-ILD and early and/or inflammatory SSc and/or severe skin fibrosis, a nomogram based on a multivariable model may be of value for identifying patients at risk of short-term progression. CI - © Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan kuwanam@nms.ac.jp. FAU - Avouac, Jerôme AU - Avouac J AUID- ORCID: 0000-0002-2463-218X AD - Service de Rheumatologie, Hôpital Cochin, AP-HP, Centre - Université Paris Cité, Paris, France. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Rheumatology and Internal Medicine, Ghent University Hospital and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. FAU - Toenges, Gerrit AU - Toenges G AD - Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany. FAU - Alves, Margarida AU - Alves M AD - Boehringer Ingelheim International GmbH, Ingelheim, Germany. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. LA - eng PT - Journal Article DEP - 20240905 PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Scleroderma, Systemic/complications MH - *Lung Diseases, Interstitial/etiology/diagnosis/drug therapy MH - *Disease Progression MH - Female MH - Male MH - Middle Aged MH - Adult MH - ROC Curve MH - Prognosis MH - Vital Capacity MH - Biomarkers MH - Logistic Models PMC - PMC11381690 OTO - NOTNLM OT - pulmonary fibrosis OT - risk factors OT - scleroderma, systemic COIS- Competing interests: MK reports grants from Boehringer Ingelheim, Ono, MBL; consulting fees from Argenx, AstraZeneca, Boehringer Ingelheim, Chugai, GlaxoSmithKline, Kissei, Mochida; speaker fees from AbbVie, Asahi Kasei, Boehringer Ingelheim, Chugai, Eisai, Janssen, Ono; royalties/licenses from MBL. JA reports no disclosures. A-MH-V reports grants from Boehringer Ingelheim and Janssen; consulting fees from Arxx Therapeutics, Bristol-Myers Squibb, Boehringer Ingelheim, Genentech, Janssen, Medscape; speaker fees from Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Roche; and support for attending meetings from Boehringer Ingelheim, Medscape, Roche; she is a EULAR study group leader on the lung in rheumatic and musculoskeletal diseases and a convener of the ERS/EULAR CTD-ILD guidelines. VS reports a grant from the Belgian Fund for Scientific Research in Rheumatic Diseases; research support from Boehringer Ingelheim; she is a senior clinical investigator of the Research Foundation—Flanders (Belgium) (FWO) (1.8.029.20N) (which had no involvement with the study reported in this manuscript). VS also reports consulting fees from Argenx BV, Boehringer Ingelheim, Janssen-Cilag, WebMD Global LLC; speaker fees from Boehringer Ingelheim, Galapagos, Janssen-Cilag; payment from BKC Moving Media Makers B.V.; support for attending congresses from Boehringer Ingelheim; participation on an advisory board and being an educational chair for Janssen-Cilag. VS has unpaid roles as chair of the EULAR study group on microcirculation in rheumatic diseases; as co-chair of the ACR study group on microcirculation and SCTC working group on capillaroscopy; and as a member of the Steering Committee of ERN-ReCONNET. GT and MA are employees of Boehringer Ingelheim. OD reports grants from Boehringer Ingelheim, Kymera, Mitsubishi Tanabe; consulting fees from 4P-Pharma, AbbVie, Acceleron, Alcimed, Altavant Sciences, Amgen, AnaMar, Arxx Therapeutics, AstraZeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Corbus Pharmaceuticals, CSL Behring, Galapagos, Glenmark, Gossamer, Horizon Therapeutics, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Orion, Prometheus Biosciences, Redx Pharma, Roivant, Topadur, UCB; speaker fees from Bayer, Boehringer Ingelheim, Janssen, Medscape; and a patent issued for mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143). OD also reports being Chair of the Executive Committee of the FOREUM Foundation, Co-Chair and President of the ERS/EULAR guidelines and EUSTAR, a member of the Board of Trustees of the Swiss Clinical Quality Management in Rheumatic Diseases, and the Hartmann Müller Foundation, a Senat member of the Swiss Academy of Medical Sciences, and Co-Founder of Citus AG. EDAT- 2024/09/07 14:43 MHDA- 2024/09/07 14:44 PMCR- 2024/09/05 CRDT- 2024/09/06 20:43 PHST- 2024/02/19 00:00 [received] PHST- 2024/08/26 00:00 [accepted] PHST- 2024/09/07 14:44 [medline] PHST- 2024/09/07 14:43 [pubmed] PHST- 2024/09/06 20:43 [entrez] PHST- 2024/09/05 00:00 [pmc-release] AID - rmdopen-2024-004240 [pii] AID - 10.1136/rmdopen-2024-004240 [doi] PST - epublish SO - RMD Open. 2024 Sep 5;10(3):e004240. doi: 10.1136/rmdopen-2024-004240. PMID- 32226164 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220413 IS - 0034-6233 (Print) IS - 2084-9834 (Electronic) IS - 0034-6233 (Linking) VI - 57 IP - 6 DP - 2019 TI - Clinical features and outcome of mixed connective tissue disease in developmental age - observational study from one center. PG - 315-319 LID - 10.5114/reum.2019.91275 [doi] AB - OBJECTIVES: Mixed connective tissue disease is a rare systemic connective tissue disease of developmental age and it includes the features of arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus and systemic sclerosis, with presence of anti-ribonucleoprotein antibodies (anti-RNP) in serum. Early diagnosis of the disease is difficult but essential in preventing development of systemic complications, which are often irreversible. International literature does not report many studies on large cohorts of children with this disease. The aim of this retrospective study was to define clinical characteristics and long-term results of treatment of the disease in 60 children with mixed connective tissue disease hospitalized in the period between 1978 and 2018. The diagnosis was established on the basis of Kasukawa's criteria. MATERIAL AND METHODS: It was a group of 60 children (46 girls and 16 boys) aged 10.5 on average (4-16.5). When assessing general symptoms at the onset of the disease according to Kasukawa's criteria, the highest number, over 80% of children, demonstrated symptoms suggesting SLE, about 40% suggesting DM and about 25% suggesting SSC. In the period of observation the number of children with clinical symptoms suggesting SSC increased. The most common clinical symptoms included Raynaud syndrome, arthritis and myositis and the most common irregularities in the test results included presence of anti-RNP antibodies and rheumatoid factor and hematological symptoms such as leukopenia/thrombocytopenia. Restrictive lung function impairment was demonstrated by 20% of children. Treatment most often included combined therapy (glucocorticosteroids + methotrexate/azathioprine). RESULTS: In 70% of the patients stable improvement was observed. Remission concerned 7% of the patients, frequent exacerbations were found in almost 20% of patients, and 2 children (3.5%) died. CONCLUSIONS: The long term observations of patients in developementeal age with mixed connective tissue disease revealed that the majority of them had domination of SLE symptoms, only in 7% achieved remission and 70% remained in stable improvement. Serious infections with septic state were the cause of death in two cases. CI - Copyright: © 2019 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. FAU - Rutkowska-Sak, Lidia AU - Rutkowska-Sak L AD - Clinic of Developmental Age Rheumatology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. FAU - Gietka, Piotr AU - Gietka P AD - Clinic of Developmental Age Rheumatology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland. LA - eng PT - Journal Article DEP - 20191231 PL - Poland TA - Reumatologia JT - Reumatologia JID - 20130190R PMC - PMC7091480 OTO - NOTNLM OT - clinical symptoms OT - mixed connective tissue disease OT - prognosis COIS- The authors declare no conflict of interest. EDAT- 2019/01/01 00:00 MHDA- 2019/01/01 00:01 PMCR- 2019/01/01 CRDT- 2020/04/01 06:00 PHST- 2019/11/18 00:00 [received] PHST- 2019/12/09 00:00 [accepted] PHST- 2020/04/01 06:00 [entrez] PHST- 2019/01/01 00:00 [pubmed] PHST- 2019/01/01 00:01 [medline] PHST- 2019/01/01 00:00 [pmc-release] AID - 91275 [pii] AID - 10.5114/reum.2019.91275 [doi] PST - ppublish SO - Reumatologia. 2019;57(6):315-319. doi: 10.5114/reum.2019.91275. Epub 2019 Dec 31. PMID- 39093747 OWN - NLM STAT- MEDLINE DCOM- 20240802 LR - 20250212 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 103 IP - 31 DP - 2024 Aug 2 TI - Delayed diagnosis of TAFRO syndrome: A case report. PG - e39148 LID - 10.1097/MD.0000000000039148 [doi] LID - e39148 AB - RATIONALE: TAFRO syndrome is a systemic inflammatory disorder, manifesting as thrombocytopenia (t), anasarca (a), fever (f), reticulin myelofibrosis/renal insufficiency (r), and organomegaly (o), and considered as a unique clinical subtype of idiopathic multicentric Castleman disease (iMCD). Such syndrome gave rise to a clinical picture similar to that of either a connective tissue disease or an autoimmune disease. PATIENT CONCERNS: A Chinese young female initially presenting with arthralgia, Raynaud phenomenon, generalized edema, and a positive anti-small nuclear ribonucleoprotein particle antibody was diagnosed as mixed connective tissue disease. The kidney biopsy showed thrombotic microangiopathy. Bone marrow smear showed bone marrow hyperplasia and biopsy revealed suspected light chain restricted expression, megakaryocyte proliferation, and moderate to severe bone marrow fibrosis. A lymph node biopsy was conducted and the histopathological findings were consistent with the subtype of mixed Castleman disease. The clinical symptoms were relieved after regular chemotherapy. DIAGNOSES: After above examination results and clinical manifestations, the final diagnoses was TAFRO syndrome. INTERVENTION: The she was started on chemotherapy with bortezomib, cyclophosphamide, and dexamethasone. OUTCOME: After chemotherapy, symptoms such as thrombocytopenia, hematuria and proteinuria disappeared, lymphadenopathy and VEGF level decreased, and bone marrow fibrosis relieved. LESSONS: Our case illustrated the first cases of shared characteristics of mixed connective tissue disease and iMCD-TAFRO syndrome. Cytokines may play a role in the shared pathogenicity of the iMCD-TAFRO syndrome and systemic autoimmune diseases. Therapy directly against inflammatory factors such as corticosteroids or chemotherapy have an important therapeutic implication. CI - Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Qiao, Yumeng AU - Qiao Y AUID- ORCID: 0009-0000-2182-1180 AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China. FAU - Zhang, Xin AU - Zhang X AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China. FAU - Xu, Rong AU - Xu R AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China. FAU - Jia, Xiaoyu AU - Jia X AD - Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China. AD - Institute of Nephrology, Peking University, Beijing, China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China. FAU - Wang, Qian AU - Wang Q AD - Department of Hematology, Peking University First Hospital, Beijing, China. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 69G8BD63PP (Bortezomib) RN - 8N3DW7272P (Cyclophosphamide) RN - 7S5I7G3JQL (Dexamethasone) RN - Multi-centric Castleman's Disease SB - IM MH - Female MH - Humans MH - Bortezomib/therapeutic use MH - *Castleman Disease/diagnosis/drug therapy/pathology MH - Cyclophosphamide/therapeutic use MH - *Delayed Diagnosis MH - Dexamethasone/therapeutic use MH - Edema/diagnosis/etiology MH - Fever/etiology MH - Syndrome MH - *Thrombocytopenia/diagnosis PMC - PMC11296483 COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2024/08/02 18:42 MHDA- 2024/08/02 18:43 PMCR- 2024/08/02 CRDT- 2024/08/02 13:03 PHST- 2024/08/02 18:43 [medline] PHST- 2024/08/02 18:42 [pubmed] PHST- 2024/08/02 13:03 [entrez] PHST- 2024/08/02 00:00 [pmc-release] AID - 00005792-202408020-00022 [pii] AID - MD-D-24-00430 [pii] AID - 10.1097/MD.0000000000039148 [doi] PST - ppublish SO - Medicine (Baltimore). 2024 Aug 2;103(31):e39148. doi: 10.1097/MD.0000000000039148. PMID- 39476557 OWN - NLM STAT- MEDLINE DCOM- 20241128 LR - 20250605 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 69 DP - 2024 Dec TI - Primary headache in SLE -systematic review and meta-analysis. PG - 152566 LID - S0049-0172(24)00206-3 [pii] LID - 10.1016/j.semarthrit.2024.152566 [doi] AB - OBJECTIVES: To systematically review and synthesize literature on: 1) the overall prevalence of primary headaches and specifically migraines, in patients with lupus since previous systematic review published in 2004; 2) the risk factors associated with primary headaches in patients with lupus; 3) the association of primary headaches with structural brain changes; and 4) "lupus headaches". METHODS: This review used (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) PRISMA guidelines and literature searches in four databases: Ovid-based Medline, Embase, PsycINFO, and Cochrane Database of Systematic Reviews from inception until 4/2022. Papers on primary headaches in patients with lupus were identified. Included studies were critically appraised and analyzed. Since a systematic review on this topic was published in 2004, only papers published in 2004 and later were included in this review. Statistical and publication bias was assessed using funnel plots. RESULTS: A total of 5096 references were identified, 189 were selected for detailed review and 11 papers were included in the final analysis. 1) The pooled prevalence of primary headaches in lupus was 26.8 % (95 %CI 25.1-28.6). The prevalence of primary headaches was similar between patients with lupus and healthy controls, odds ratio (OR) 2.14, 95 %CI 0.97-4.76, p value=0.06, however publication bias was significant according to the Egger test. Lupus patients seem to have a higher prevalence of primary headaches compared to patients with rheumatoid arthritis (RA) and primary Sjogren's syndrome (pSS), OR 2.5, 95 % CI 1.56-4, p < 0.0001. Regarding the prevalence of migraines specifically, no difference was found between patients with lupus compared to healthy controls and patients with RA or pSS. 2) Primary headaches seem to be associated with depression and impaired health related quality of life rather than lupus activity or damage. There is insufficient data to conclude whether specific lupus treatments affected primary headaches in lupus, however, one study did suggest hydroxychloroquine reduced the frequency of primary headaches. Raynaud phenomenon was associated with migraines. 3) One study which examined MRI scans of patients with lupus compared to healthy controls did suggest that larger gray matter volumes reduced the odds for headaches in general (OR 0.98, p = 0.048) and for migraines in particular (OR 0.95, p = 0.004), and larger white matter volumes increased the odds for migraine (OR 1.04, p = 0.007). However, these findings might reflect disease activity or damage, therefore further studies are required to clarify this issue. 4) The only study which specifically addressed the prevalence of "lupus headaches" is Hanly's study from 2013 of the SLICC cohort. In this large cohort, only 1.5 % of patients had "lupus headaches", as defined by SLE Disease Activity Index-2000 (SLEDAI-2 K). Therefore "Lupus headache" is a rare entity in SLE and should be suspected after excluding other possible explanations for headaches, especially when there is no known prior history of primary headaches. CONCLUSIONS: Patients with lupus seem to have a similar prevalence of primary headaches, and specifically migraines, compared to healthy controls. These primary headaches are associated with depression and Raynaud phenomenon and not with disease activity nor damage. Scarce data is published regarding structural changes associated with primary headaches. Currently is seems "lupus headaches" are a distinct rare manifestation of lupus and more data are needed. CI - Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved. FAU - Feld, Joy AU - Feld J AD - Rheumatology unit, Carmel and Zvulun medical centers, Haifa, Israel; Department of Rheumatology, Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. Electronic address: joyf@clalit.org.il. FAU - Tayer-Shifman, Oshrat E AU - Tayer-Shifman OE AD - Rheumatology Unit, Meir Medical Center, Kfar Saba, Israel; Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. FAU - Su, Jiandong AU - Su J AD - University Health Network, Division of Rheumatology, Schroeder Arthritis Institute, Toronto, Ontario, Canada. FAU - Anderson, Melanie AU - Anderson M AD - Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada. FAU - Touma, Zahi AU - Touma Z AD - University of Toronto Lupus Clinic, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Western Hospital, Toronto, Ontario, Canada. Electronic address: zahi.touma@uhn.ca. LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20241028 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/complications/epidemiology MH - Prevalence MH - Migraine Disorders/epidemiology MH - Headache/epidemiology MH - Headache Disorders, Primary/epidemiology OTO - NOTNLM OT - Keyword OT - lupus OT - primary headaches COIS- Declaration of competing interest Joy Feld reports a relationship with Clalit health care, Israel in the form of consulting fees; honoraria for lectures/presentation from Elly-Lilly; support for attending meetings from Abbvie; and stock or stock options with AbbVie. Oshrat Tayer-Shifman reports a relationship with consulting fees from Astra-Zeneca and Abbvie. Zahi Touma is supported by grants from Schroeder Arthritis Institute, Lupus Ontario, Arthritis Society of Canada, Canadian Institutes of Health Research, Physicians’ Services Incorporated, The Province of Ontario (Early Research Award), The Lupus Research Alliance and reports a relationship with consulting fees from AstraZeneca AB, Merck KGaA, GlaxoSmithKline Inc, and UCB Biopharma SRL, and Sarkana Pharma, Inc; Janssen, LuCin, Ampel Biosolultions and AbbVie. EDAT- 2024/10/31 04:20 MHDA- 2024/11/29 05:21 CRDT- 2024/10/30 19:06 PHST- 2023/09/21 00:00 [received] PHST- 2024/08/27 00:00 [revised] PHST- 2024/10/09 00:00 [accepted] PHST- 2024/11/29 05:21 [medline] PHST- 2024/10/31 04:20 [pubmed] PHST- 2024/10/30 19:06 [entrez] AID - S0049-0172(24)00206-3 [pii] AID - 10.1016/j.semarthrit.2024.152566 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2024 Dec;69:152566. doi: 10.1016/j.semarthrit.2024.152566. Epub 2024 Oct 28. PMID- 26651932 OWN - NLM STAT- MEDLINE DCOM- 20170109 LR - 20170110 IS - 1776-2561 (Electronic) IS - 0761-8417 (Linking) VI - 72 IP - 2 DP - 2016 Apr TI - [Epidemiological, clinical and evolutionary peculiarities of interstitial lung disease in systemic sclerosis]. PG - 122-8 LID - S0761-8417(15)00122-4 [pii] LID - 10.1016/j.pneumo.2015.09.003 [doi] AB - Pulmonary involvement during systemic sclerosis (SS) is dominated by interstitial lung disease and arterial pulmonary hypertension. It is about a retrospective study analyzing 65 cases of SS over a period of 13 years. We compared cases with and without interstitial lung disease. The diagnosis of SS was retained according to American College of Rheumatology (ACR)/EULAR 2013 criteria. The diagnosis of interstitial lung disease was retained in TDM and EFR. Pulmonary hypertension is defined by a pulmonary arterial pression higher than 25 mmHg. The mean delay of diagnosis of interstitial lung disease and the diagnosis was of 48 months (extremes 0-78 months). The comparison between both groups according to average age of the patients, prevalence of pulmonary hypertension, frequency of Raynaud phenomenon and trophic disorders did not find any significant difference. Lung involvement was associated with an esophageal involvement in 71% of the cases (P=0.059). Antibodies anti-Scl 70 were noted more frequently in patient's with interstitial lung disease (79% of the cases, P=0.001). Patients were treated with colchicine and vitamin E. A corticotherapy had been indicated at a single patient. The evolution of SS was marked by the stabilisation of the restrictive syndrome in 71.8% of the cases and a worsening in 25% of the cases. Early and appropriate diagnosis of SS and screening of lung involvement are essential for a early care. CI - Copyright © 2015 Elsevier Masson SAS. All rights reserved. FAU - Aydi, Z AU - Aydi Z AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. FAU - Rachdi, I AU - Rachdi I AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. FAU - Ben Dhaou, B AU - Ben Dhaou B AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. Electronic address: besma.bendhaou@yahoo.fr. FAU - Dridi, M AU - Dridi M AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. FAU - Daoud, F AU - Daoud F AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. FAU - Baili, L AU - Baili L AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. FAU - Boussema, F AU - Boussema F AD - Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Montfleury, Tunisie. LA - fre PT - Journal Article TT - Particularités épidémiologiques, cliniques et évolutives des pneumopathies interstitielles au cours de la sclérodermie systémique. DEP - 20151202 PL - France TA - Rev Pneumol Clin JT - Revue de pneumologie clinique JID - 8406312 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Disease Progression MH - Female MH - Humans MH - Lung Diseases, Interstitial/complications/*epidemiology/*pathology MH - Male MH - Middle Aged MH - Respiratory Function Tests MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*epidemiology/*pathology OTO - NOTNLM OT - Biomarkers OT - Biomarqueurs OT - CT scan OT - Explorations fonctionnelles respiratoires OT - Hypertension artérielle pulmonaire OT - Interstitial lung disease OT - Pneumopathie interstitielle diffuse OT - Pulmonary function tests OT - Pulmonary hypertension OT - Scanner OT - Sclérodermie systémique OT - Systemic sclerosis EDAT- 2015/12/15 06:00 MHDA- 2017/01/10 06:00 CRDT- 2015/12/15 06:00 PHST- 2015/01/30 00:00 [received] PHST- 2015/08/25 00:00 [revised] PHST- 2015/09/15 00:00 [accepted] PHST- 2015/12/15 06:00 [entrez] PHST- 2015/12/15 06:00 [pubmed] PHST- 2017/01/10 06:00 [medline] AID - S0761-8417(15)00122-4 [pii] AID - 10.1016/j.pneumo.2015.09.003 [doi] PST - ppublish SO - Rev Pneumol Clin. 2016 Apr;72(2):122-8. doi: 10.1016/j.pneumo.2015.09.003. Epub 2015 Dec 2. PMID- 37172074 OWN - NLM STAT- Publisher LR - 20230512 IS - 1708-539X (Electronic) IS - 1708-5381 (Linking) DP - 2023 May 12 TI - A comprehensive review of the epidemiology and clinical features of 91 cases with Buerger's disease. PG - 17085381231175257 LID - 10.1177/17085381231175257 [doi] AB - BACKGROUND: Thromboangiitis Obliterans (TAO) is a disease of small and medium-sized arteries with an unclear natural course. This study aims to establish a national registry of the disease to gain a better understanding of its epidemiology and clinical course. METHOD: This study was a cohort study of 242 patients with a high probability of TAO admitted to Mashhad University of Medical Sciences (MUMS) hospitals from 2000 to 2015. Of these, 91 patients with a confirmed diagnosis were included in the study (90 males and 1 female) with a mean age of 35 ± 7.8 years. RESULTS: The most common symptom upon onset of the disease was paresthesia (29.7%), followed by cold sensitivity and paresthesia (93.4%) during the progression of the disease and Raynaud syndrome or vasospasm (93.9%) in the active phase. The right lower limb was the most commonly affected limb (46.2%), and presenting ischemic symptoms in 48.4%.Statistics indicated a positive correlation between the duration of Burger's disease and the number of affected limbs (p = 0.001). There was no effect of disease duration on the likelihood of amputations (p = 0.28). CONCLUSION: Some patients may experience mild, subtle symptoms for years before the initial signs and symptoms appear, which can be severe and rapidly progress to the point of requiring amputation.We suggest that the diagnostic criteria for Buerger's disease should be revised in light of the presence of atherosclerosis and its associated risk factors, which present a challenge in terms of diagnosis and treatment. Clinical experience will be of great importance in this regard. FAU - Modaghegh, Mohammad Hadi Saeed AU - Modaghegh MHS AUID- ORCID: 0000-0002-2724-7463 AD - Professor of Vascular Surgery, Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. RINGGOLD: 37552 FAU - Kamyar, Mohammad Mahdi AU - Kamyar MM AD - Assistant Professor of Vascular Surgery, Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. RINGGOLD: 37552 FAU - Shafiei, Ali AU - Shafiei A AD - Assistant Professor of Vascular Surgery, Qom University of Medical Sciences, Qom, Iran. RINGGOLD: 154202 FAU - Shariatmaghani, Somayeh Sadat AU - Shariatmaghani SS AD - Assistant Professor of Internal Medicine, Ghaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. RINGGOLD: 37552 FAU - Saremi, Elena AU - Saremi E AD - Vascular Surgeon, Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran. RINGGOLD: 37552 FAU - Sadeghipour Kermani, Fatemeh AU - Sadeghipour Kermani F AD - Community and Preventive Medicine, Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Science, Mashhad, Iran. LA - eng PT - Journal Article DEP - 20230512 PL - England TA - Vascular JT - Vascular JID - 101196722 SB - IM OTO - NOTNLM OT - Thromboangiitis obliterans OT - clinical feature OT - epidemiology EDAT- 2023/05/12 19:07 MHDA- 2023/05/12 19:07 CRDT- 2023/05/12 13:43 PHST- 2023/05/12 19:07 [medline] PHST- 2023/05/12 19:07 [pubmed] PHST- 2023/05/12 13:43 [entrez] AID - 10.1177/17085381231175257 [doi] PST - aheadofprint SO - Vascular. 2023 May 12:17085381231175257. doi: 10.1177/17085381231175257. PMID- 19444505 OWN - NLM STAT- MEDLINE DCOM- 20091223 LR - 20221207 IS - 1432-086X (Electronic) IS - 0174-1551 (Linking) VI - 32 IP - 5 DP - 2009 Sep TI - Spontaneous dissection of the renal arteries: a misdiagnosed but not infrequent disease! PG - 1101-2; author reply 1103-4 LID - 10.1007/s00270-009-9552-4 [doi] FAU - Pellerin, Olivier AU - Pellerin O FAU - Beyssen, Bernard AU - Beyssen B FAU - Raynaud, Alain AU - Raynaud A FAU - Sapoval, Marc AU - Sapoval M LA - eng PT - Comment PT - Letter DEP - 20090515 PL - United States TA - Cardiovasc Intervent Radiol JT - Cardiovascular and interventional radiology JID - 8003538 SB - IM CON - Cardiovasc Intervent Radiol. 2009 Mar;32(2):329-32. doi: 10.1007/s00270-008-9363-z. PMID: 18509702 MH - Adult MH - Aortic Dissection/*complications/diagnostic imaging MH - Angiography MH - Angioplasty, Balloon MH - Diagnosis, Differential MH - Fibromuscular Dysplasia/*complications/diagnostic imaging MH - Humans MH - Infarction/diagnostic imaging/*etiology/therapy MH - Kidney/*blood supply MH - Kidney Diseases/diagnostic imaging/*etiology/therapy MH - Male MH - Tomography, X-Ray Computed EDAT- 2009/05/16 09:00 MHDA- 2009/12/24 06:00 CRDT- 2009/05/16 09:00 PHST- 2008/12/01 00:00 [received] PHST- 2009/02/03 00:00 [accepted] PHST- 2009/05/16 09:00 [entrez] PHST- 2009/05/16 09:00 [pubmed] PHST- 2009/12/24 06:00 [medline] AID - 10.1007/s00270-009-9552-4 [doi] PST - ppublish SO - Cardiovasc Intervent Radiol. 2009 Sep;32(5):1101-2; author reply 1103-4. doi: 10.1007/s00270-009-9552-4. Epub 2009 May 15. PMID- 20461385 OWN - NLM STAT- MEDLINE DCOM- 20120214 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 31 IP - 11 DP - 2011 Nov TI - Hearing organ disorders in patients with systemic sclerosis. PG - 1423-8 LID - 10.1007/s00296-010-1503-5 [doi] AB - In systemic sclerosis (SSc), there may develop hearing and balance disorders as a result of the immune-mediated vasculitis and fibrosis in the inner ear. The objective of the study was evaluation of the hearing organ function in patients with SSc with relationship to duration of the disease and Raynaud phenomenon and also to type and severity of the disease. Twenty unselected, consecutive patients with SSc diagnosed in compliance with the international diagnostic criteria of the American Rheumatism Association (1982), were enrolled into the study. The control group consisted of 26 otologically healthy persons matched to the SSc group for age and sex. Case history was recorded for all patients from questionnaire data. Otolaryngological examination and battery of audiological tests (pure tone audiometry, speech audiometry, impedance audiometry and auditory brainstem response-ABR) were performed. In the anamnesis 60% of patients reported vertigo, 55% headaches, 50% tinnitus, 40% hyperacusis, 40% hearing loss and 30% ear fullness. It was found that patients with SSc had significantly poorer mean hearing thresholds than the control group for 0.5, 1, 6 and 8 kHz. In ABR there were no differences between SSc and control groups although an increase of latency averages in the group of limited patients with SSc compared with the diffuse patients with SSc was observed. In eight patients (40%) sensorineural hearing loss, mostly bilateral and symmetrical was found. Furthermore, no relation was seen between hearing level and duration, type and severity of the disease. Ear involvement is frequent in systemic sclerosis and should be taken into consideration during diagnostic and therapeutic procedures. FAU - Maciaszczyk, Katarzyna AU - Maciaszczyk K AD - Department of Otolaryngology, Medical University of Lodz, 22, Kopcinskiego St., 90-153 Lodz, Poland. k.maciaszczyk@op.pl FAU - Waszczykowska, Elżbieta AU - Waszczykowska E FAU - Pajor, Anna AU - Pajor A FAU - Bartkowiak-Dziankowska, Bożena AU - Bartkowiak-Dziankowska B FAU - Durko, Tomasz AU - Durko T LA - eng PT - Journal Article DEP - 20100512 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Adult MH - Aged MH - Comorbidity MH - Female MH - Hearing Loss, Sensorineural/diagnosis/*epidemiology/physiopathology MH - Hearing Tests/methods MH - Humans MH - Male MH - Middle Aged MH - Poland/epidemiology MH - Scleroderma, Systemic/diagnosis/*epidemiology/physiopathology MH - Sex Factors MH - Skin/pathology EDAT- 2010/05/13 06:00 MHDA- 2012/02/15 06:00 CRDT- 2010/05/13 06:00 PHST- 2009/11/29 00:00 [received] PHST- 2010/04/27 00:00 [accepted] PHST- 2010/05/13 06:00 [entrez] PHST- 2010/05/13 06:00 [pubmed] PHST- 2012/02/15 06:00 [medline] AID - 10.1007/s00296-010-1503-5 [doi] PST - ppublish SO - Rheumatol Int. 2011 Nov;31(11):1423-8. doi: 10.1007/s00296-010-1503-5. Epub 2010 May 12. PMID- 24679054 OWN - NLM STAT- MEDLINE DCOM- 20160327 LR - 20150619 IS - 2146-8427 (Electronic) IS - 1304-0855 (Linking) VI - 13 IP - 3 DP - 2015 Jun TI - Cryofibrinogenemia After a Liver Transplant: First Reported Case Posttransplant and a Case-Based Review of the Nontransplant Literature. PG - 290-4 LID - 10.6002/ect.2014.0013 [doi] AB - Cryofibrinogenemia is a rare disorder in which plasma, not serum, forms a cryoprecipitate. Patients with cryofibrinogenemia may be asymptomatic, or they may have painful ulcers, purpura, livedo reticularis, Raynaud phenomenon, perniosis of the extremities, thrombosis, and arthralgia. Cryofibrinogenemia may be primary or secondary to an underlying disorder such as connective tissue disease, malignancy, infection, drugs, or thromboembolic disease. Here, we present a 41-year-old woman with a pancreatic neuroendocrine tumor who underwent a Whipple procedure in 2003 followed by 2 liver transplants for hepatic metastases. Three years posttransplant, we discovered a biopsy-proven metastatic lesion in her femur. Five years posttransplant, she developed acute, severe pain in both feet, and was found to have cryofibrinogenemia despite immunosuppression post-transplant. Testing for connective tissue diseases and hematologic malignancy were negative. She was treated with high-dose prednisone, which completely resolved her symptoms. We also conducted a review of the literature via a PubMed search to summarize the association of cryofibrinogenemia with malignancy and treating cryofibrinogenemia with corticosteroids. Our study is the first reported case of cryofibrinogenemia that developed secondary to a neuroendocrine tumor posttransplant. Our report suggests that cryofibrinogenemia may occur despite immunosuppression adequate to prevent graft rejection, and that high-dose corticosteroids are an effective treatment for posttransplant cryofibrinogenemia. FAU - Chen, Yuanyuan AU - Chen Y AD - From the Division of Gastroenterology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada. FAU - Sreenivasan, Gayatri M AU - Sreenivasan GM FAU - Shojania, Kam AU - Shojania K FAU - Yoshida, Eric M AU - Yoshida EM LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20140328 PL - Turkey TA - Exp Clin Transplant JT - Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation JID - 101207333 RN - 0 (Glucocorticoids) RN - 0 (Immunosuppressive Agents) RN - VB0R961HZT (Prednisone) RN - Cryofibrinogenemia SB - IM MH - Adult MH - Cryoglobulinemia/diagnosis/drug therapy/*etiology MH - Female MH - Glucocorticoids/administration & dosage MH - Humans MH - Immunosuppressive Agents/adverse effects MH - Liver Neoplasms/secondary/*surgery MH - Liver Transplantation/*adverse effects MH - Prednisone/administration & dosage MH - Reoperation MH - Risk Factors MH - Time Factors MH - Treatment Outcome EDAT- 2014/04/01 06:00 MHDA- 2016/03/28 06:00 CRDT- 2014/04/01 06:00 PHST- 2014/04/01 06:00 [entrez] PHST- 2014/04/01 06:00 [pubmed] PHST- 2016/03/28 06:00 [medline] AID - 10.6002/ect.2014.0013 [doi] PST - ppublish SO - Exp Clin Transplant. 2015 Jun;13(3):290-4. doi: 10.6002/ect.2014.0013. Epub 2014 Mar 28. PMID- 35864935 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220723 IS - 2090-6609 (Print) IS - 2090-6617 (Electronic) IS - 2090-6617 (Linking) VI - 2022 DP - 2022 TI - Systemic Sclerosis, Reversible Cerebral Vasoconstriction Syndrome, and NeuroMyelitis Optica in a Patient. PG - 8541329 LID - 10.1155/2022/8541329 [doi] LID - 8541329 AB - Systemic sclerosis (SSC) is an autoimmune disease of connective tissue and microvasculature mostly caused by autoantibodies. Likewise, neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system correlating with autoantibodies against aquapourin-4. Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder of brain vasculature resembling Raynaud phenomena in SSC. Despite co-occurrence is not rare in autoimmune disorders, the co-occurrence of NMO and SSC is extremely rare. In this case, we report a 35-year-old female presenting with paraplegia one day after discharge from hospital following surgical carnioplasty. She had a history of scleroderma and optic neuritis for which she was treated with high dose glucocorticoids causing renal crisis and RCVS causing intracranial and intracerebral hemorrhage which required a craniotomy to be performed in February 2020. In her recent admission, magnetic resonance imaging of the spinal cord indicated longitudinally extensive transverse myelitis (LETM) and blood tests revealed a highly positive titer of NMO-IgG. Daily plasmapheresis resulted in satisfactory improvement in her condition. This case highlights the importance of evaluating neurologic manifestations in systemic sclerosis patients considering the NMO and RCVS occurrence. Additionally, in concomitant cases, the treatment strategy should be modified regarding the risk of scleroderma renal crisis. CI - Copyright © 2022 Masoud Etemadifar et al. FAU - Etemadifar, Masoud AU - Etemadifar M AUID- ORCID: 0000-0002-0066-3782 AD - Department of Neurology, Isfahan University of Medical Sciences, Isfahan, Iran. FAU - Shafiei, Mehdi AU - Shafiei M AUID- ORCID: 0000-0003-1231-9222 AD - Department of Neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran. FAU - Salari, Mehri AU - Salari M AUID- ORCID: 0000-0001-7102-821X AD - Functional Neurosurgery Research Center, Shohada Tajrish Comprehensive Neurosurgical Center of Excellence, Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Modares Sadeghi, Ali AU - Modares Sadeghi A AUID- ORCID: 0000-0001-8107-5220 AD - Department of Neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran. FAU - Fakhrolmobasheri, Mohammad AU - Fakhrolmobasheri M AUID- ORCID: 0000-0002-6502-376X AD - Isfahan Cardiovascular Research Center (Heart Failure Research Center), Isfahan, Iran. LA - eng PT - Case Reports PT - Journal Article DEP - 20220712 PL - United States TA - Case Reports Immunol JT - Case reports in immunology JID - 101622188 PMC - PMC9296349 COIS- The authors declare that they have no conflicts of interest. EDAT- 2022/07/23 06:00 MHDA- 2022/07/23 06:01 PMCR- 2022/07/12 CRDT- 2022/07/22 02:17 PHST- 2022/04/01 00:00 [received] PHST- 2022/06/12 00:00 [revised] PHST- 2022/07/01 00:00 [accepted] PHST- 2022/07/22 02:17 [entrez] PHST- 2022/07/23 06:00 [pubmed] PHST- 2022/07/23 06:01 [medline] PHST- 2022/07/12 00:00 [pmc-release] AID - 10.1155/2022/8541329 [doi] PST - epublish SO - Case Reports Immunol. 2022 Jul 12;2022:8541329. doi: 10.1155/2022/8541329. eCollection 2022. PMID- 32513403 OWN - NLM STAT- MEDLINE DCOM- 20200922 LR - 20200922 IS - 1879-1131 (Electronic) IS - 0738-081X (Linking) VI - 38 IP - 2 DP - 2020 Mar-Apr TI - Sclerodermalike syndromes: Great imitators. PG - 235-249 LID - S0738-081X(19)30186-5 [pii] LID - 10.1016/j.clindermatol.2019.10.010 [doi] AB - Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc). In addition, SLSs can be provoked by several drugs, chemicals, or even physical injury (eg, trauma, vibration stress, radiation). In SLSs, the distribution of skin involvement seems to be atypical compared with SSc. The acral skin involvement is usually missing, and lack of Raynaud phenomenon, scleroderma-specific antinuclear antibodies, the absence of scleroderma capillary pattern, and internal organ manifestations indicate the presence of an SLS. Skin involvement is sometimes nodular, and the underlying tissues can also be affected. For the differential diagnosis, a skin biopsy of the deeper layers including fascia and muscle is required. Histology does not always allow differentiation between SSc and SLSs; therefore, the diagnosis is often based on the distribution, quality of cutaneous involvement, and other accompanying clinical features. CI - Copyright © 2019 Elsevier Inc. All rights reserved. FAU - Varjú, Cecília AU - Varjú C AD - Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. FAU - Kumánovics, Gábor AU - Kumánovics G AD - Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. FAU - Czirják, László AU - Czirják L AD - Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, Division of Rheumatology, Florence, Italy. FAU - Minier, Tünde AU - Minier T AD - Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. Electronic address: minier.tunde@pte.hu. LA - eng PT - Journal Article DEP - 20191024 PL - United States TA - Clin Dermatol JT - Clinics in dermatology JID - 8406412 SB - IM MH - Biopsy/methods MH - Diagnosis, Differential MH - Humans MH - Scleroderma, Systemic/*diagnosis/etiology/*pathology MH - Skin/*pathology MH - Syndrome EDAT- 2020/06/10 06:00 MHDA- 2020/09/23 06:00 CRDT- 2020/06/10 06:00 PHST- 2020/06/10 06:00 [entrez] PHST- 2020/06/10 06:00 [pubmed] PHST- 2020/09/23 06:00 [medline] AID - S0738-081X(19)30186-5 [pii] AID - 10.1016/j.clindermatol.2019.10.010 [doi] PST - ppublish SO - Clin Dermatol. 2020 Mar-Apr;38(2):235-249. doi: 10.1016/j.clindermatol.2019.10.010. Epub 2019 Oct 24. PMID- 30477063 OWN - NLM STAT- MEDLINE DCOM- 20190507 LR - 20190507 IS - 0004-5772 (Print) IS - 0004-5772 (Linking) VI - 66 IP - 5 DP - 2018 May TI - Clinical Profile of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis. PG - 69-70 AB - INTRODUCTION: Primary Sjogren's Syndrome (pSS) with Hypokalemic Periodic Paralysis(HPP) whether an association or a different clinical subset needs review. METHODS: Cross-sectional retrospective study of subjects of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis(HPP) identified from database maintained at Centre For Rheumatic Diseases, Pune since 1996 with records of over 50000 patients. The diagnosis was clinical. Clinical and investigations data was extracted pertaining to initial examination and follow up. Standard investigations & ELISA, immunoblot and nephelometry to assay autoantibodies (AAb) were done. RESULTS: 16 patients of Primary Sjogren's Syndrome (pSS) with Hypokalemic Periodic Paralysis (HPP) were identified in the period 2000-2014. Presenting feature was HPP in 86% with Dry eye (4%) and Arthralgias (10%) in remaining. Distal Renal Tubular Acidosis was identified in all. All were females with average age of 26 years. Symptomatic ocular sicca noted in 60% & Oral sicca in 50% patients. Other features - Arthralgias (91%), arthritis (42%), mucositis (38%), Neuropathy (30%), skin rash (20%) cytopenias (19%), Erosive arthritis (10%), interstitial lung disease (10%) and Raynaud Phenomenon (10%). 100% were positive for ANA. SSA was positive in 100%, SSB in 50% of patients & Rheumatoid Factor in 70 %. Hypothyroidism was associated in 70% patients. CONCLUSION: We present a large series of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis(HPP) from India. Prominent features of female dominance, younger age of onset and SSA positivity noted in this cohort of patients on Routine clinical and serology phenotype suggests existence of a distinct subset. HPP was presenting feature in majority. CI - © Journal of the Association of Physicians of India 2011. FAU - Kulkarni, Nachiket AU - Kulkarni N AD - Consultant Rheumatologist. FAU - Chopra, Arvind AU - Chopra A AD - Chief and Consultant Rheumatologist, Center for Rheumatic Diseases, Pune, Maharashtra. LA - eng PT - Journal Article PL - India TA - J Assoc Physicians India JT - The Journal of the Association of Physicians of India JID - 7505585 SB - IM MH - Adult MH - Cross-Sectional Studies MH - Female MH - Humans MH - *Hypokalemic Periodic Paralysis MH - India MH - Retrospective Studies MH - *Sjogren's Syndrome EDAT- 2018/11/28 06:00 MHDA- 2019/05/08 06:00 CRDT- 2018/11/28 06:00 PHST- 2018/11/28 06:00 [entrez] PHST- 2018/11/28 06:00 [pubmed] PHST- 2019/05/08 06:00 [medline] PST - ppublish SO - J Assoc Physicians India. 2018 May;66(5):69-70. PMID- 23812075 OWN - NLM STAT- MEDLINE DCOM- 20140228 LR - 20191112 IS - 1349-7413 (Electronic) IS - 0911-4300 (Linking) VI - 36 IP - 3 DP - 2013 TI - [Rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis : a case report]. PG - 170-4 AB - A 68-year-old female who had Raynaud phenomenon for a decade was admitted to our hospital in January 2012. She complained of sclerodactyly and scleroderma that did not extend past the elbows. She also had fingertip ulcers that repeatedly disappeared and recurred for several years. Blood tests showed that she was anti-centromere antibody positive. Therefore, she was diagnosed with limited cutaneous systemic sclerosis. Two months after diagnosis, she returned to our hospital because she experienced dyspnea on exertion and exacerbation of her fingertip ulcers. Chest X-rays revealed cardiac enlargement, an echocardiography showed tricuspid regurgitation with an increased tricuspid pressure gradient (91 mmHg) and right heart catheterization showed a mean pulmonary arterial pressure of 59 mmHg. Chest computed tomography and lung perfusion scintigraphy showed no abnormalities. She was then diagnosed with pulmonary arterial hypertension associated with systemic sclerosis. She improved rapidly with daily treatments of prednisolone in addition to warfarin, bosentan and beraprost sodium. This is a rare case of rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis that can be markedly improved with early diagnosis and treatment. FAU - Ishida, Motoko AU - Ishida M AD - Department of Internal Medicine and Rheumatology, Clinical Research Institute, National Hospital Organization, Kyushu Medical Center. FAU - Miyamura, Tomoya AU - Miyamura T FAU - Kaieda, Tomoe AU - Kaieda T FAU - Kimura, Daisaku AU - Kimura D FAU - Nakamura, Akihiro AU - Nakamura A FAU - Takahama, Soichiro AU - Takahama S FAU - Kiyasu, Junichi AU - Kiyasu J FAU - Minami, Rumi AU - Minami R FAU - Yamamoto, Masahiro AU - Yamamoto M FAU - Suematsu, Eiichi AU - Suematsu E LA - jpn PT - Case Reports PT - English Abstract PT - Journal Article PL - Japan TA - Nihon Rinsho Meneki Gakkai Kaishi JT - Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology JID - 9505992 RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Aged MH - Disease Progression MH - Female MH - Humans MH - Hypertension, Pulmonary/*complications/drug therapy MH - Prednisolone/therapeutic use MH - Scleroderma, Systemic/*complications/drug therapy EDAT- 2013/07/03 06:00 MHDA- 2014/03/01 06:00 CRDT- 2013/07/02 06:00 PHST- 2013/07/02 06:00 [entrez] PHST- 2013/07/03 06:00 [pubmed] PHST- 2014/03/01 06:00 [medline] AID - DN/JST.JSTAGE/jsci/36.170 [pii] AID - 10.2177/jsci.36.170 [doi] PST - ppublish SO - Nihon Rinsho Meneki Gakkai Kaishi. 2013;36(3):170-4. doi: 10.2177/jsci.36.170. PMID- 28620416 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1687-9627 (Print) IS - 1687-9635 (Electronic) VI - 2017 DP - 2017 TI - Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient-A High Suspicion Is Still Needed. PG - 5797041 LID - 10.1155/2017/5797041 [doi] LID - 5797041 AB - Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity. A lacy, purplish rash was noted on the trunk and upper extremity. MRI of brain showed acute/subacute lacunar infarctions. Laboratory studies revealed an elevated lactate dehydrogenase level, bilirubin and ferritin, decreased haptoglobin, and positive Coombs test. Antinuclear antibody test was negative and antiphospholipid antibody panel revealed positivity for anti-cardiolipin IgG and IgM, antiphosphatidylserine IgG, and anti-β2-glycoprotein IgG. The patient was diagnosed with primary APS. Pancytopenia is relatively rare in primary APS and is more often seen in secondary APS. Our patient demonstrated involvement of multiple organ systems as well as livedo reticularis and autoimmune-related findings such as Raynaud phenomenon and Coombs positive hemolytic anemia. We discuss the various clinical and laboratory findings in patients with APS that aid in diagnosis, as well as important management considerations. FAU - Ibrahim, Uroosa AU - Ibrahim U AUID- ORCID: 0000-0003-0088-5438 AD - Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA. FAU - Kedia, Shiksha AU - Kedia S AD - Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA. FAU - Garcia, Gwenalyn AU - Garcia G AD - Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA. FAU - Atallah, Jean Paul AU - Atallah JP AD - Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20170523 PL - United States TA - Case Rep Med JT - Case reports in medicine JID - 101512910 PMC - PMC5460429 EDAT- 2017/06/18 06:00 MHDA- 2017/06/18 06:01 PMCR- 2017/05/23 CRDT- 2017/06/17 06:00 PHST- 2017/02/01 00:00 [received] PHST- 2017/04/12 00:00 [revised] PHST- 2017/05/04 00:00 [accepted] PHST- 2017/06/17 06:00 [entrez] PHST- 2017/06/18 06:00 [pubmed] PHST- 2017/06/18 06:01 [medline] PHST- 2017/05/23 00:00 [pmc-release] AID - 10.1155/2017/5797041 [doi] PST - ppublish SO - Case Rep Med. 2017;2017:5797041. doi: 10.1155/2017/5797041. Epub 2017 May 23. PMID- 42186646 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260526 LR - 20260526 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 4 DP - 2026 Apr TI - Immune Checkpoint Inhibitor-Induced Scleroderma-Like Pulmonary Fibrosis in a Patient With Advanced Lung Cancer: A Case Report. PG - e107642 LID - 10.7759/cureus.107642 [doi] LID - e107642 AB - We describe a 79-year-old man with stage IV non-small cell lung cancer previously treated with pembrolizumab who presented with progressive dyspnea and hypoxemia. Evaluation demonstrated bilateral ground glass opacities with interstitial changes compatible with a nonspecific interstitial pneumonia pattern, echocardiographic findings concerning for pulmonary hypertension, Raynaud phenomenon, and autoimmune serologies including elevated polymyositis/scleroderma 75 (PM/Scl 75) and small nuclear ribonucleoprotein 70 kilodalton (SNRNP 70Kd) antibodies suggestive of a scleroderma-like autoimmune process. Infectious evaluation, including blood and urine cultures and respiratory pathogen testing, did not identify a causative pathogen, although sputum culture, bronchoscopy with bronchoalveolar lavage, and lung biopsy were not performed, which is an important limitation. The patient was treated with broad-spectrum antibiotics, systemic corticosteroids, and ventilatory support, with planned initiation of additional immunosuppressive therapy. Despite these interventions, his respiratory status worsened, requiring mechanical ventilation, and his course was complicated by renal failure, heparin-induced thrombocytopenia, and multiorgan dysfunction. This case illustrates a rare, likely immune checkpoint inhibitor-associated scleroderma-like pulmonary syndrome in a patient with multiple comorbidities and underscores the need to consider progressive autoimmune disease among several potential etiologies in patients presenting with unexplained respiratory decline after immunotherapy, while acknowledging that a definitive causal relationship cannot be established in the absence of a complete diagnostic evaluation. CI - Copyright © 2026, Rodriguez Padilla et al. FAU - Rodriguez Padilla, Juan O AU - Rodriguez Padilla JO AD - Internal Medicine, Lakeland Regional Health, Lakeland, USA. FAU - Kheti, Yatin AU - Kheti Y AD - Pulmonary and Critical Care Medicine, Lakeland Regional Health, Lakeland, USA. FAU - Carrillo, Felix AU - Carrillo F AD - Hospital Medicine, Lakeland Regional Health, Lakeland, USA. FAU - Villacreses, Camila AU - Villacreses C AD - Internal Medicine, Lakeland Regional Health, Lakeland, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20260424 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13198839 OTO - NOTNLM OT - autoimmune pneumonitis OT - immune checkpoint inhibitors OT - interstitial lung disease OT - pembrolizumab OT - pulmonary fibrosis OT - scleroderma-like syndrome COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/05/26 12:18 MHDA- 2026/05/26 12:19 PMCR- 2026/04/24 CRDT- 2026/05/26 03:57 PHST- 2026/02/10 00:00 [received] PHST- 2026/04/23 00:00 [accepted] PHST- 2026/05/26 12:19 [medline] PHST- 2026/05/26 12:18 [pubmed] PHST- 2026/05/26 03:57 [entrez] PHST- 2026/04/24 00:00 [pmc-release] AID - 10.7759/cureus.107642 [doi] PST - epublish SO - Cureus. 2026 Apr 24;18(4):e107642. doi: 10.7759/cureus.107642. eCollection 2026 Apr. PMID- 31599343 OWN - NLM STAT- MEDLINE DCOM- 20210210 LR - 20210210 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 40 IP - 3 DP - 2020 Mar TI - Restrictive cardiomyopathy in a patient with systemic sclerosis and Fabry disease: a case-based review. PG - 489-497 LID - 10.1007/s00296-019-04453-y [doi] AB - Systemic sclerosis (SSc) is a rare immune-mediated vasculopathy characterized by fibrosis of the skin and internal organs. Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene producing α-galactosidase-A enzyme (α-Gal A) deficiency. Being a systemic disease, cardiac involvement in FD has a high mortality rate due to heart failure and arrhythmia. The coexistence of these two entities has not been reported previously. We describe the case of a female patient with limited SSc (lcSSc), a diagnosis based on the presence of sclerodactyly, Raynaud phenomenon, microvascular involvement, and positive anti-centromere antibodies. On follow-up, she developed chest pain, a second-degree A-V block, and restrictive cardiomyopathy (without cardiovascular risk factors). Although heart involvement is common in these two entities, the abnormal thickening of lateral and inferior wall, the infiltration pattern and the conduction system disorders presented herein are more characteristic in a heterozygous female with a cardiac variant of FD. The diagnosis of FD was confirmed with high globotriaosylsphingosine (Lyso-Gb3) levels and identification of GLA gene mutation. The patient was treated with enzymatic replacement (agalsidase alpha) following mild improvement in ventricular mass at 6th month, without clinical deterioration. The related literature on SSc associated with FD is also reviewed. FAU - Arbeláez-Cortés, Álvaro AU - Arbeláez-Cortés Á AUID- ORCID: 0000-0001-9167-9204 AD - Internal Medicine, Universidad Libre, Cali, Colombia. aarbelaezc@gmail.com. AD - Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia. aarbelaezc@gmail.com. FAU - Quintero-González, Diana C AU - Quintero-González DC AUID- ORCID: 0000-0003-3041-1698 AD - Internal Medicine, Universidad Libre, Cali, Colombia. FAU - Cuesta-Astroz, Yesid AU - Cuesta-Astroz Y AUID- ORCID: 0000-0002-4243-0645 AD - School of Microbiology, Universidad de Antioquia, Medellín, Colombia. AD - Instituto Colombiano de Medicina Tropical, Universidad CES, Sabaneta, Colombia. FAU - Villadiego, Juan S AU - Villadiego JS AD - Cardiology, Clínica Amiga, Cali, Colombia. FAU - González-Buriticá, Herman AU - González-Buriticá H AD - Internal Medicine, Universidad Libre, Cali, Colombia. AD - Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia. FAU - Rueda, Jorge M AU - Rueda JM AD - Internal Medicine, Universidad Libre, Cali, Colombia. AD - Arthritis and Rheumatology Clinic, Centro Médico Imbanaco, Cali, Colombia. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20191010 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Cardiomyopathy, Restrictive/*complications MH - Fabry Disease/*complications MH - Female MH - Humans MH - Middle Aged MH - Scleroderma, Systemic/*complications OTO - NOTNLM OT - Autoimmune diseases OT - Fabry disease OT - Restrictive cardiomyopathy OT - Systemic sclerosis EDAT- 2019/10/11 06:00 MHDA- 2021/02/11 06:00 CRDT- 2019/10/11 06:00 PHST- 2019/08/08 00:00 [received] PHST- 2019/09/26 00:00 [accepted] PHST- 2019/10/11 06:00 [pubmed] PHST- 2021/02/11 06:00 [medline] PHST- 2019/10/11 06:00 [entrez] AID - 10.1007/s00296-019-04453-y [pii] AID - 10.1007/s00296-019-04453-y [doi] PST - ppublish SO - Rheumatol Int. 2020 Mar;40(3):489-497. doi: 10.1007/s00296-019-04453-y. Epub 2019 Oct 10. PMID- 39037917 OWN - NLM STAT- MEDLINE DCOM- 20251210 LR - 20251213 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 64 IP - SI DP - 2025 Dec 1 TI - Preventive effects of early immunosuppressive treatment on the development of interstitial lung disease in systemic sclerosis. PG - SI122-SI130 LID - 10.1093/rheumatology/keae375 [doi] AB - BACKGROUND: Hypothesizing that early treatment yields improved prognosis, we aimed to investigate how the timing of immunosuppressive treatment relates to interstitial lung disease (ILD) development and the course of pulmonary function in systemic sclerosis (SSc). METHODS: A cohort was created using data from the EUSTAR database and Nijmegen Systemic Sclerosis cohort, including adult patients who started their first immunosuppressive treatment (i.e. mycophenolate mofetil, methotrexate, cyclophosphamide, tocilizumab or rituximab) after SSc diagnosis, and no signs of ILD on high-resolution CT. ILD-free survival and the course of forced vital capacity (FVC) % predicted were assessed for up to 5 years' follow-up comparing patients who started early (disease duration ≤3 years) vs late with immunosuppression. RESULTS: 1052 patients met the eligibility criteria. The early treatment group (n = 547, 52%) showed a higher prevalence of male sex, diffuse cutaneous subtype (53.1% vs 36.5%), and anti-topoisomerase-I antibody (ATA, 51.1% vs 42.7%). Most patients were treated with methotrexate (60.1%), whereas only a few patients were treated with biologics (1.7%). The incidence of ILD was 46.6% after mean (s.d.) 3.6 (1.4) years; the hazards ratio for ILD in the early treatment group was 1.13 (95% CI: 0.93, 1.38) after adjustment for confounders. FVC % predicted trajectories were comparable between groups. CONCLUSION: Our findings did not confirm a preventive role of early initiation of immunosuppressive therapy vs late initiation on ILD development. However, our findings should be interpreted with caution, considering the high inflammatory, ATA-positive enriched nature of the cohort, confounding by indication, and that very few patients were treated with biologics. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Velauthapillai, Arthiha AU - Velauthapillai A AUID- ORCID: 0009-0006-0700-7573 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Bootsma, M F R AU - Bootsma MFR AUID- ORCID: 0009-0005-1724-1112 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Bruni, Cosimo AU - Bruni C AUID- ORCID: 0000-0003-2813-2083 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. AD - Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence-Careggi University Hospital, Florence, Italy. FAU - Bergmann, Christina AU - Bergmann C AUID- ORCID: 0000-0001-5257-9171 AD - Department of Internal Medicine 3, Rheumatology and Clinical Immunology, Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. AD - Deutsches Zentrum Immuntherapie (DZI), Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - University Vita Salute San Raffaele, Milano, Italy. AD - Unit of Immunology, Rheumatology, Allergy and Rare Diseases, and Division of Clinical and Experimental Rheumatology, IRCCS San Raffaele Hospital, Milan, Italy. FAU - Launay, David AU - Launay D AUID- ORCID: 0000-0003-1840-1817 AD - Département de Médecine Interne et d'Immunologie Clinique, Centre National de Référence Maladies Systémiques et Auto-immunes Rares Nord et Nord-Ouest de France (CeRAINO), European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNECT), Univ. Lille, CHU Lille, Lille, France. FAU - Riemekasten, Gabriela AU - Riemekasten G AUID- ORCID: 0000-0002-5406-2464 AD - Department of Rheumatology and Clinical Immunology, University of Medical Center Schleswig-Holstein, University Lübeck, Germany. FAU - Garzanova, L AU - Garzanova L AUID- ORCID: 0000-0002-5012-0540 AD - VA Nasonova Research Institute of Rheumatology, Moscow, Russian Federation. FAU - Airò, Paolo AU - Airò P AUID- ORCID: 0000-0001-5241-1918 AD - UOC Reumatologia e Immunologia Clinica, ASST Spedali Civili di Brescia, Brescia, Italy. FAU - Rezus, Elena AU - Rezus E AUID- ORCID: 0000-0002-8175-2583 AD - Department of Rheumatology, 'Grigore T. Popa' University of Medicine and Pharmacy, 16 Universitatii Street, Iasi, Romania. AD - Clinical Rehabilitation Hospital, Iasi, Romania. FAU - da Silva, Jose A P AU - da Silva JAP AUID- ORCID: 0000-0002-2782-6780 AD - Faculty of Medicine, University of Coimbra, Coimbra, Portugal. AD - Department of Rheumatology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Del Galdo, Francesco AU - Del Galdo F AUID- ORCID: 0000-0002-8528-2283 AD - Leeds Raynaud's And Scleroderma Program, NIHR Biomedical Research Centre Leeds Institute Of Rheumatic And Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology, University of Cologne, Köln, Germany. FAU - Chung, Lorinda S AU - Chung LS AUID- ORCID: 0000-0003-0072-6939 AD - Department of Medicine & Dermatology, Division of Immunology & Rheumatology, Stanford University School of Medicine and Palo Alto VA Health Care System, Palo Alto, CA, USA. FAU - Krasowska, Dorota AU - Krasowska D AUID- ORCID: 0000-0002-3176-9870 AD - Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AUID- ORCID: 0000-0002-4352-2824 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AUID- ORCID: 0000-0002-2266-9907 AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. CN - EUSTAR Collaborators LA - eng GR - WT_/Wellcome Trust/United Kingdom GR - Foundation for Research in Rheumatology/ GR - Scleroderma Clinical Trials Consortium/ GR - Scleroderma Research Foundation/ PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Immunosuppressive Agents) RN - YL5FZ2Y5U1 (Methotrexate) RN - HU9DX48N0T (Mycophenolic Acid) SB - IM MH - Humans MH - Male MH - *Lung Diseases, Interstitial/prevention & control/etiology/epidemiology MH - Female MH - *Immunosuppressive Agents/therapeutic use/administration & dosage MH - *Scleroderma, Systemic/drug therapy/complications MH - Middle Aged MH - Adult MH - Methotrexate/therapeutic use MH - Aged MH - Vital Capacity MH - Mycophenolic Acid/therapeutic use PMC - PMC12695046 OTO - NOTNLM OT - early treatment OT - interstitial lung disease OT - systemic sclerosis FIR - Guiducci, Serena IR - Guiducci S FIR - Walker, Ulrich IR - Walker U FIR - Iannone, Florenzo IR - Iannone F FIR - Distler, Oliver IR - Distler O FIR - Becvar, Radim IR - Becvar R FIR - Cutolo, Maurizio IR - Cutolo M FIR - Rednic, Simona IR - Rednic S FIR - Allanore, Yannick IR - Allanore Y FIR - Montecucco, C IR - Montecucco C FIR - Inanc, Murat IR - Inanc M FIR - Carreira, Patricia E IR - Carreira PE FIR - Czirják, László IR - Czirják L FIR - Iudici, Michele IR - Iudici M FIR - Kotyla, Przemyslaw IR - Kotyla P FIR - Zanatta, Elisabetta IR - Zanatta E FIR - Perdan-Pirkmajer, Katja IR - Perdan-Pirkmajer K FIR - Moroncini, Gianluca IR - Moroncini G FIR - Airò, Paolo IR - Airò P FIR - Balbir-Gurman, Alexandra IR - Balbir-Gurman A FIR - Hunzelmann, Nicolas IR - Hunzelmann N FIR - Idolazzi, Luca IR - Idolazzi L FIR - Mitrovic, Josko IR - Mitrovic J FIR - Denton, Christopher IR - Denton C FIR - Vonk, Madelon IR - Vonk M FIR - Colic, Jelena IR - Colic J FIR - Henes, Jörg IR - Henes J FIR - Foeldvari, Ivan IR - Foeldvari I FIR - da Silva, José António Pereira IR - da Silva JAP FIR - Stamenkovic, Bojana IR - Stamenkovic B FIR - De Santis, Maria IR - De Santis M FIR - Ananieva, Lidia P IR - Ananieva LP FIR - Müller-Ladner, Ulf IR - Müller-Ladner U FIR - Szücs, Gabriella IR - Szücs G FIR - Launay, David IR - Launay D FIR - Riccieri, Valeria IR - Riccieri V FIR - Balanescu, Andra IR - Balanescu A FIR - Gheorghiu, Ana Maria IR - Gheorghiu AM FIR - Bergmann, Christina IR - Bergmann C FIR - Mouthon, Luc IR - Mouthon L FIR - Smith, Vanessa IR - Smith V FIR - Cantatore, Francesco Paolo IR - Cantatore FP FIR - Mogensen, Mette IR - Mogensen M FIR - Vanthuyne, Marie IR - Vanthuyne M FIR - Alegre-Sancho, Juan Jose IR - Alegre-Sancho JJ FIR - Anić, Branimir IR - Anić B FIR - Cauli, Alberto IR - Cauli A FIR - Solanki, Kamal IR - Solanki K FIR - Rosato, Edoardo IR - Rosato E FIR - Foti, Rosario IR - Foti R FIR - Maurer, Britta IR - Maurer B FIR - Senet, Patricia IR - Senet P FIR - Chatelus, Emmanuel IR - Chatelus E FIR - Litinsky, Ira IR - Litinsky I FIR - Del Galdo, Francesco IR - Del Galdo F FIR - Castellví, Ivan IR - Castellví I FIR - Limonta, Massimiliano IR - Limonta M FIR - Marcoccia, Antonella IR - Marcoccia A FIR - Martin, Thierry IR - Martin T FIR - Wojteczek, Anna IR - Wojteczek A FIR - Riemekasten, Gabriela IR - Riemekasten G FIR - Rezus, Elena IR - Rezus E FIR - Cuomo, Giovanna IR - Cuomo G FIR - Epis, Oscar Massimiliano IR - Epis OM FIR - Sfikakis, Petros IR - Sfikakis P FIR - Furst, Daniel IR - Furst D FIR - Ramazan, Ana-Maria IR - Ramazan AM FIR - de Vries-Bouwstra, Jeska IR - de Vries-Bouwstra J FIR - Truchetet, Marie-Elise IR - Truchetet ME FIR - Lescoat, Alain IR - Lescoat A FIR - Matucci-Cerinic, Marco IR - Matucci-Cerinic M FIR - Spierings, Julia IR - Spierings J FIR - Kuwana, Masataka IR - Kuwana M FIR - Martin, Mickaël IR - Martin M FIR - Simeón-Aznar, Carmen-Pilar IR - Simeón-Aznar CP FIR - Pârvu, Magda IR - Pârvu M FIR - Boleto, Gonçalo IR - Boleto G FIR - Del Papa, Nicoletta IR - Del Papa N FIR - Kastrati, Kastriot IR - Kastrati K FIR - Selvi, Enrico IR - Selvi E FIR - Geroldinger-Simic, Marija IR - Geroldinger-Simic M FIR - Mosca, Marta IR - Mosca M FIR - Dzhus, Marta IR - Dzhus M FIR - Karadag, Duygu Temiz IR - Karadag DT FIR - Batalov, Anastas IR - Batalov A FIR - Ginosyan, Knarik IR - Ginosyan K FIR - Mukuchyan, Vahan IR - Mukuchyan V FIR - Vardanyan, Valentina IR - Vardanyan V FIR - Haroyan, Armine IR - Haroyan A FIR - Naffaa, Mohammad IR - Naffaa M FIR - Maglio, Cristina IR - Maglio C FIR - Santos, Cristiana Isabel Sieiro IR - Santos CIS FIR - Iwata, Futoshi IR - Iwata F FIR - Hinchcliff, Monique IR - Hinchcliff M FIR - Cordeiro, Ana IR - Cordeiro A FIR - Giacomelli, Roberto IR - Giacomelli R FIR - Benvenuti, Francesco IR - Benvenuti F FIR - Rabaneda, Esther Vicente IR - Rabaneda EV FIR - Györfi, Andrea-Hermina IR - Györfi AH FIR - Nuñez, Lilian Maria Lopez IR - Nuñez LML FIR - De Angelis, Rossella IR - De Angelis R FIR - Brigante, Jorge Alejandro IR - Brigante JA FIR - Miedany, Yasser IR - Miedany Y FIR - Zhang, Lijun IR - Zhang L EDAT- 2024/07/22 18:42 MHDA- 2025/12/11 06:15 PMCR- 2024/07/22 CRDT- 2024/07/22 12:53 PHST- 2024/02/08 00:00 [received] PHST- 2024/07/09 00:00 [accepted] PHST- 2025/12/11 06:15 [medline] PHST- 2024/07/22 18:42 [pubmed] PHST- 2024/07/22 12:53 [entrez] PHST- 2024/07/22 00:00 [pmc-release] AID - 7717972 [pii] AID - keae375 [pii] AID - 10.1093/rheumatology/keae375 [doi] PST - ppublish SO - Rheumatology (Oxford). 2025 Dec 1;64(SI):SI122-SI130. doi: 10.1093/rheumatology/keae375. PMID- 38960318 OWN - NLM STAT- MEDLINE DCOM- 20240805 LR - 20240912 IS - 1095-9319 (Electronic) IS - 0026-2862 (Linking) VI - 155 DP - 2024 Sep TI - Thymic stromal lymphopoietin and digital microvascular damage in systemic sclerosis patients: A pilot study. PG - 104714 LID - S0026-2862(24)00063-3 [pii] LID - 10.1016/j.mvr.2024.104714 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) is a complex autoimmune connective-tissue disease, characterised by vasculopathy and fibrosis of the skin and internal organs. Activation of microvascular endothelial cells (ECs) causes the intimal hyperplasia that characterises the vascular remodelling in SSc. The most frequent complication of SSc is the development of digital ulcers (DUs). Thymic stromal lymphopoietin (TSLP) may trigger fibrosis and sustain vascular damage. Aim of this study was to evaluate the correlation between serum level of TSLP and DUs. METHODS: 75 consecutive SSc patients were enrolled and serum TSLP levels were measured. The presence of history of DUs (HDU) was evaluated. Recurrent new DUs were defined as the presence of at least 3 episodes of DUs in a 12-months follow up period. The risk of developing new DUs was calculated by applying the capillaroscopic skin ulcer risk index (CSURI). RESULTS: The median value of TSLP was higher in patients with HDU than patients without HDU [181.67 pg/ml (IQR 144.67; 265.66) vs 154.67 pg/ml (IQR 110.67; 171.33), p < 0.01]. The median value of TSLP was higher in patients with an increased CSURI index than patients without an increased CSURI [188 pg/ml (IQR 171.33; 246.33) vs 159.33 pg/ml (IQR 128.67; 218), p < 0.01]. Kaplan-Meier curves demonstrated that free survival from new DUs was significantly (p < 0.01) lower in SSc patients with increased TSLP serum levels. CONCLUSION: TSLP might have a key role in digital microvascular damage of SSc patients. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Pellicano, Chiara AU - Pellicano C AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Cusano, Giuseppina AU - Cusano G AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. FAU - Basile, Umberto AU - Basile U AD - UOC of Clinical Pathology DEA II level, Hospital Santa Maria Goretti-ASL Latina, Latina, Italy. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. Electronic address: edoardo.rosato@uniroma1.it. LA - eng PT - Journal Article DEP - 20240701 PL - United States TA - Microvasc Res JT - Microvascular research JID - 0165035 RN - 0 (Cytokines) RN - GT0IL38SP4 (Thymic Stromal Lymphopoietin) RN - 0 (Biomarkers) RN - 0 (TSLP protein, human) SB - IM MH - Humans MH - *Scleroderma, Systemic/blood/pathology MH - Female MH - Male MH - Middle Aged MH - Pilot Projects MH - *Cytokines/blood MH - *Thymic Stromal Lymphopoietin MH - *Microscopic Angioscopy MH - *Skin Ulcer/pathology/etiology/blood MH - Adult MH - Risk Factors MH - *Biomarkers/blood MH - *Fingers/blood supply MH - Aged MH - Microvessels/pathology/metabolism MH - Time Factors MH - Up-Regulation MH - Recurrence MH - Fibrosis MH - Risk Assessment OTO - NOTNLM OT - CSURI OT - Digital ulcers OT - Nailfold videocapillaroscopy OT - Raynaud phenomenon OT - Systemic sclerosis OT - TSLP COIS- Declaration of competing interest All the authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2024/07/04 00:43 MHDA- 2024/08/06 00:42 CRDT- 2024/07/03 19:28 PHST- 2024/06/14 00:00 [received] PHST- 2024/06/26 00:00 [revised] PHST- 2024/06/29 00:00 [accepted] PHST- 2024/08/06 00:42 [medline] PHST- 2024/07/04 00:43 [pubmed] PHST- 2024/07/03 19:28 [entrez] AID - S0026-2862(24)00063-3 [pii] AID - 10.1016/j.mvr.2024.104714 [doi] PST - ppublish SO - Microvasc Res. 2024 Sep;155:104714. doi: 10.1016/j.mvr.2024.104714. Epub 2024 Jul 1. PMID- 34743853 OWN - NLM STAT- MEDLINE DCOM- 20220113 LR - 20220113 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 160 IP - 5 DP - 2021 Nov TI - A 24-Year-Old Woman With Dyspnea, Chest Pain, and Dry Cough. PG - e503-e506 LID - S0012-3692(21)01110-7 [pii] LID - 10.1016/j.chest.2021.05.064 [doi] AB - A 24-year-old woman, a baby-sitter with no known comorbidities, presented to the outpatient department with complaints of modified Medical Research Council grade IV breathlessness for 3 months, chest pain, and dry cough for 2 weeks. There was no known disease history, including respiratory, flu-like illness, or connective tissue disorder. There was no use of chemotherapeutic, oral contraceptive drugs, exposure to toxic substances, or smoking. A review of systems was negative for fever, arthralgia, myalgia, Raynaud phenomenon, skin thickening, rash, or leg swelling. The patient had no family history suggestive of a genetic syndrome. CI - Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. FAU - Fakili, Fusun AU - Fakili F AD - Department of Pulmonary Medicine, Gaziantep University, Sahinbey Research Hospital, Gaziantep, Turkey. Electronic address: fusunfakili@yahoo.com. FAU - Duzen, Irfan Veysel AU - Duzen IV AD - Department of Cardiology, Gaziantep University, Sahinbey Research Hospital, Gaziantep, Turkey. FAU - Kaplan, Mehmet AU - Kaplan M AD - Department of Cardiology, Gaziantep University, Sahinbey Research Hospital, Gaziantep, Turkey. FAU - Bayram, Nazan Gulhan AU - Bayram NG AD - Department of Pulmonary Medicine, Gaziantep University, Sahinbey Research Hospital, Gaziantep, Turkey. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Endothelin A Receptor Antagonists) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Pyrimidines) RN - 0 (Sulfonamides) RN - BW9B0ZE037 (Sildenafil Citrate) RN - EC 2.7.11.1 (EIF2AK4 protein, human) RN - EC 2.7.11.1 (Protein Serine-Threonine Kinases) RN - Z9K9Y9WMVL (macitentan) RN - Hemangiomatosis, familial pulmonary capillary SB - IM MH - Chest Pain/diagnosis/etiology MH - Computed Tomography Angiography/methods MH - Cough/diagnosis/etiology MH - Diagnosis, Differential MH - Dyspnea/diagnosis/etiology MH - Echocardiography/methods MH - Endothelin A Receptor Antagonists/administration & dosage MH - Female MH - Hemangioma, Capillary/*diagnosis MH - Humans MH - Hypertension, Pulmonary/*diagnosis/etiology/physiopathology MH - Lung Neoplasms/*diagnosis MH - Lung Transplantation MH - Mutation MH - Oxygen Inhalation Therapy/methods MH - Phosphodiesterase 5 Inhibitors/administration & dosage MH - Protein Serine-Threonine Kinases/*genetics MH - *Pulmonary Veno-Occlusive Disease/complications/congenital/diagnosis/genetics MH - Pyrimidines/*administration & dosage MH - Respiratory Function Tests/methods MH - Sildenafil Citrate/*administration & dosage MH - Sulfonamides/*administration & dosage MH - Young Adult EDAT- 2021/11/09 06:00 MHDA- 2022/01/14 06:00 CRDT- 2021/11/08 05:41 PHST- 2021/04/12 00:00 [received] PHST- 2021/05/18 00:00 [revised] PHST- 2021/05/21 00:00 [accepted] PHST- 2021/11/08 05:41 [entrez] PHST- 2021/11/09 06:00 [pubmed] PHST- 2022/01/14 06:00 [medline] AID - S0012-3692(21)01110-7 [pii] AID - 10.1016/j.chest.2021.05.064 [doi] PST - ppublish SO - Chest. 2021 Nov;160(5):e503-e506. doi: 10.1016/j.chest.2021.05.064. PMID- 32657190 OWN - NLM STAT- MEDLINE DCOM- 20200916 LR - 20201218 IS - 2374-4243 (Electronic) IS - 2374-4243 (Linking) VI - 52 IP - 11 DP - 2020 Nov TI - Extension of COVID-19 pulmonary parenchyma lesions based on real-life visual assessment on initial chest CT is an independent predictor of poor patient outcome. PG - 838-840 LID - 10.1080/23744235.2020.1792544 [doi] FAU - Grégory, Jules AU - Grégory J AD - Department of Radiology, Assistance Publique des Hôpitaux de Paris Nord, Beaujon Hospital, University of Paris, Clichy, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, Assistance Publique des Hôpitaux de Paris Nord, Beaujon Hospital, University of Paris, Clichy, France. FAU - Galy, Adrien AU - Galy A AD - Department of Internal Medicine, Beaujon Hospital, Paris Nord Val de Seine Hospitals, Assistance Publique des Hôpitaux de Paris, University of Paris, Clichy, France. FAU - Corre, Félix AU - Corre F AD - Department of Internal Medicine, Beaujon Hospital, Paris Nord Val de Seine Hospitals, Assistance Publique des Hôpitaux de Paris, University of Paris, Clichy, France. FAU - Bijot, Jean Charles AU - Bijot JC AD - Department of Radiology, Assistance Publique des Hôpitaux de Paris Nord, Beaujon Hospital, University of Paris, Clichy, France. FAU - Nguyen, Yann AU - Nguyen Y AD - Department of Internal Medicine, Beaujon Hospital, Paris Nord Val de Seine Hospitals, Assistance Publique des Hôpitaux de Paris, University of Paris, Clichy, France. FAU - Vilgrain, Valérie AU - Vilgrain V AD - Department of Radiology, Assistance Publique des Hôpitaux de Paris Nord, Beaujon Hospital, University of Paris, Clichy, France. AD - Centre for Research on Inflammation, Laboratory of Imaging Biomarkers, INSERM U1149, Paris, France. LA - eng PT - Comment PT - Letter DEP - 20200713 PL - England TA - Infect Dis (Lond) JT - Infectious diseases (London, England) JID - 101650235 SB - IM CON - Infect Dis (Lond). 2020 Jul;52(7):498-505. doi: 10.1080/23744235.2020.1759817. PMID: 32370577 MH - Betacoronavirus MH - COVID-19 MH - *Coronavirus Infections MH - Disease Progression MH - Humans MH - *Pandemics MH - *Pneumonia, Viral MH - Retrospective Studies MH - Risk Factors MH - SARS-CoV-2 MH - Tomography, X-Ray Computed EDAT- 2020/07/14 06:00 MHDA- 2020/09/17 06:00 CRDT- 2020/07/14 06:00 PHST- 2020/07/14 06:00 [pubmed] PHST- 2020/09/17 06:00 [medline] PHST- 2020/07/14 06:00 [entrez] AID - 10.1080/23744235.2020.1792544 [doi] PST - ppublish SO - Infect Dis (Lond). 2020 Nov;52(11):838-840. doi: 10.1080/23744235.2020.1792544. Epub 2020 Jul 13. PMID- 38256549 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20241023 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 13 IP - 2 DP - 2024 Jan 11 TI - Thyroid Disorders in Systemic Sclerosis: A Comprehensive Review. LID - 10.3390/jcm13020415 [doi] LID - 415 AB - Systemic sclerosis, also referred to as scleroderma, is a chronic autoimmune disease that affects both internal organs and the skin. Systemic sclerosis predominantly affects female patients and can coexist with other disorders, including those affecting the thyroid gland. Common symptoms such as fatigue and weight changes can be attributed to either systemic sclerosis or thyroid disease. In this comprehensive review, an extensive analysis is conducted using research from 2002 to 2022, sourced from PubMed. The main focus of this exploration is to understand the intricate relationship between thyroid disorders and systemic sclerosis. We obtained these results by analyzing a number of 32285 patients included in 21 original studies. The existing evidence suggests that there is a higher incidence of elevated TSH levels and hypothyroidism in patients with systemic sclerosis, particularly in females, compared to the general population. This remains true even when comparing patients from iodine-deficient regions. Additionally, there is an increased occurrence of hyperthyroidism in the context of systemic sclerosis, which negatively impacts the prognosis of these patients. Furthermore, thyroid antibodies, predominantly anti-thyroid peroxidase (anti-TPO) antibodies, and autoimmune disorders are more commonly observed in individuals with systemic sclerosis. Although thyroid nodules are not specifically linked to the disease, when considering thyroid volume, it is observed that the thyroid gland in systemic sclerosis patients has a decreased volume, possibly due to fibrosis. Conversely, other studies have revealed that patients without autoimmune thyroid diseases (AITDs) are more likely to have a history of digital ulcers, pulmonary fibrosis detected by computed tomography scan, and a requirement for immunosuppressive medication. The majority of the studies did not establish a connection between thyroid disease in these patients and the occurrence of the limited or diffuse forms of systemic sclerosis, as well as the presence of digital ulcers, calcinosis, pulmonary arterial hypertension, scleroderma renal crisis, Raynaud phenomenon, and various other clinical manifestations. FAU - Cherim, Aifer AU - Cherim A AD - Department of Dermatovenerology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania. AD - Internal Medicine 3rd Department, Colentina Clinical Hospital, 020123 Bucharest, Romania. FAU - Petca, Răzvan-Cosmin AU - Petca RC AUID- ORCID: 0000-0003-4496-1535 AD - Department of Urology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania. AD - Department of Urology, 'Prof. Dr. Th. Burghele' Clinical Hospital, 050659 Bucharest, Romania. FAU - Dumitrascu, Mihai-Cristian AU - Dumitrascu MC AD - Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania. AD - Department of Obstetrics and Gynecology, University Emergency Hospital of Bucharest, 050098 Bucharest, Romania. FAU - Petca, Aida AU - Petca A AUID- ORCID: 0000-0002-4890-0978 AD - Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania. AD - Department of Obstetrics and Gynecology, Elias Emergency University Hospital, 011461 Bucharest, Romania. FAU - Candrea, Elisabeta AU - Candrea E AD - Department of Dermatology, University of Medicine and Pharmacy 'I. Hatieganu', 400347 Cluj Napoca, Romania. FAU - Sandru, Florica AU - Sandru F AD - Department of Dermatovenerology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania. AD - Dermatology Department, "Elias" University Emergency Hospital, 011461 Bucharest, Romania. LA - eng PT - Journal Article PT - Review DEP - 20240111 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC10816939 OTO - NOTNLM OT - familial autoimmunity OT - systemic sclerosis OT - thyroid cancer OT - thyroid disorders COIS- The authors declare no conflicts of interest. EDAT- 2024/01/23 06:44 MHDA- 2024/01/23 06:45 PMCR- 2024/01/11 CRDT- 2024/01/23 01:15 PHST- 2023/12/06 00:00 [received] PHST- 2024/01/05 00:00 [revised] PHST- 2024/01/08 00:00 [accepted] PHST- 2024/01/23 06:45 [medline] PHST- 2024/01/23 06:44 [pubmed] PHST- 2024/01/23 01:15 [entrez] PHST- 2024/01/11 00:00 [pmc-release] AID - jcm13020415 [pii] AID - jcm-13-00415 [pii] AID - 10.3390/jcm13020415 [doi] PST - epublish SO - J Clin Med. 2024 Jan 11;13(2):415. doi: 10.3390/jcm13020415. PMID- 31137064 OWN - NLM STAT- MEDLINE DCOM- 20200429 LR - 20200429 IS - 1098-9048 (Electronic) IS - 1069-3424 (Linking) VI - 40 IP - 2 DP - 2019 Apr TI - Lung Diseases in Inflammatory Myopathies. PG - 255-270 LID - 10.1055/s-0039-1685187 [doi] AB - Lung involvement is the leading cause of mortality in inflammatory myopathy. A careful assessment of clinical and serologic manifestations especially myositis-associated autoantibodies allows precise classification of the different phenotypes of inflammatory myopathy and stratification of the risk of lung involvement. About three out of four patients with inflammatory myopathy develop interstitial lung disease (ILD), which represents the main cause of morbidity and mortality. In patients with a confirmed diagnosis of inflammatory myopathy, the approach to the diagnosis of ILD includes assessment of clinical and functional severity, evaluation of the high-resolution computed tomography pattern of disease, which often suggests nonspecific interstitial pneumonia or organizing pneumonia. Bronchoalveolar lavage to rule out infection is often performed; however, video-assisted thoracoscopic lung biopsy is now generally discouraged, unless malignancy is suspected. The so-called antisynthetase syndrome characterized by the combination of mechanics' hands, Raynaud' phenomenon, myositis often mild or absent, and presence of one of the anti-tRNA synthetase antibodies is associated with a 70% risk of ILD, especially in subjects with antibodies other than anti-Jo1 antibodies (i.e., anti-PL7 or -PL12 antibodies). Treatment depends on both severity and progression of ILD, often including a combination of corticosteroids and immunosuppressive therapy. Rituximab-based regimen has showed promising results in retrospective studies for the management of refractory or rapidly progressive forms of ILD. Clinical trials are ongoing to evaluate the actual efficacy of this strategy on mortality related to lung disease. Secondary pulmonary complications of inflammatory myopathy include opportunistic infections, aspiration pneumonia, pneumomediastinum, ventilatory failure due to diaphragmatic muscular weakness, drug-induced pneumonitis, and rarely pulmonary hypertension. CI - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA. FAU - Barba, Thomas AU - Barba T AD - Department of Internal Medicine, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon, France. FAU - Mainbourg, Sabine AU - Mainbourg S AD - Department of Internal Medicine, Hôpital Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon, France. FAU - Nasser, Mouhamad AU - Nasser M AUID- ORCID: 0000-0001-8373-8032 AD - National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR754, Claude Bernard University Lyon 1, Lyon, France. FAU - Lega, Jean-Christophe AU - Lega JC AD - Department of Internal Medicine, Hôpital Lyon Sud, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon, France. FAU - Cottin, Vincent AU - Cottin V AUID- ORCID: 0000-0002-5591-0955 AD - National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR754, Claude Bernard University Lyon 1, Lyon, France. LA - eng PT - Journal Article PT - Review DEP - 20190528 PL - United States TA - Semin Respir Crit Care Med JT - Seminars in respiratory and critical care medicine JID - 9431858 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Autoantibodies/immunology MH - Disease Progression MH - Humans MH - Lung Diseases/diagnosis/*etiology/mortality/therapy MH - Myositis/*complications/immunology COIS- None. EDAT- 2019/05/29 06:00 MHDA- 2020/04/30 06:00 CRDT- 2019/05/29 06:00 PHST- 2019/05/29 06:00 [entrez] PHST- 2019/05/29 06:00 [pubmed] PHST- 2020/04/30 06:00 [medline] AID - 10.1055/s-0039-1685187 [doi] PST - ppublish SO - Semin Respir Crit Care Med. 2019 Apr;40(2):255-270. doi: 10.1055/s-0039-1685187. Epub 2019 May 28. PMID- 27310209 OWN - NLM STAT- MEDLINE DCOM- 20170117 LR - 20260127 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 34 Suppl 100 IP - 5 DP - 2016 Sep-Oct TI - One decade distinct features, morbidity and mortality of scleroderma: a cross-sectional study. PG - 74-78 AB - OBJECTIVES: Conducting an epidemiologic study on scleroderma patients referred to hospitals and tertiary centres of rheumatologic diseases in Shiraz, located in south of Iran. METHODS: A cross-sectional study was done on patients' records registered in scleroderma outpatient clinics as well as hospitals associated with Shiraz University of Medical Sciences. Gathering data in pre-formed data sheets, descriptive analysis plus qualitative comparisons by chi-square test were done using SPSS 15. RESULTS: In 533 medical records, female to male ratio was 7.3:1. The disease is mostly seen in 3rd and 4th decades of life. More patients had negative family histories (56.1%). 37.5% of the patients had diffuse form of the disease, 36.8% had limited one, and 17.3% had overlap syndrome, mostly, by lupus erythematosus (33%). Most common first presentation was Raynaud phenomenon (40.7%). Two most prevalent clinical manifestations were skin thickening (97.2%) and gastrointestinal involvement (68.9%). Clinical presentations were compared between three most common types of the disease plus various stages of life. Among recorded capillaroscopies, active form was the most prevalent one (38.3%). In documented serologic markers, the most common positive one was anti-nuclear antibody (ANA) (75.6%). Two most common etiologies of hospitalisation were digital ulcer (30.9%) and pulmonary fibrosis (5.7%). The most common cause of death (17) was pulmonary fibrosis (35.2%). CONCLUSION: This study is the first epidemiologic survey on Iranian scleroderma patients with significantly large sample size compared to previous studies worldwide. It can thus provide some guidance for further multi-provincial, multinational and interracial studies on scleroderma. FAU - Nazarinia, Mohammad Ali AU - Nazarinia MA AD - Shiraz Geriatric Research Centre, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Esmaeilzadeh, Elmira AU - Esmaeilzadeh E AD - Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Emami, Yasaman AU - Emami Y AD - Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran. FAU - Salehi, Alireza AU - Salehi A AD - Research Centre of Traditional and History of Medicine, Shiraz, Iran. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20160615 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Aged MH - Aged, 80 and over MH - Cause of Death MH - Chi-Square Distribution MH - Child MH - Child, Preschool MH - Cross-Sectional Studies MH - Disease Progression MH - Female MH - Hospitalization MH - Humans MH - Iran/epidemiology MH - Male MH - Middle Aged MH - Prevalence MH - Prognosis MH - Pulmonary Fibrosis/epidemiology MH - Registries MH - Scleroderma, Systemic/diagnosis/*epidemiology/mortality MH - Sex Distribution MH - Skin Ulcer/epidemiology MH - Time Factors MH - Young Adult EDAT- 2016/10/18 06:00 MHDA- 2017/01/18 06:00 CRDT- 2016/06/17 06:00 PHST- 2015/12/25 00:00 [received] PHST- 2016/04/19 00:00 [accepted] PHST- 2016/10/18 06:00 [pubmed] PHST- 2017/01/18 06:00 [medline] PHST- 2016/06/17 06:00 [entrez] AID - 10266 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):74-78. Epub 2016 Jun 15. PMID- 22633167 OWN - NLM STAT- MEDLINE DCOM- 20130522 LR - 20230202 IS - 2152-2669 (Electronic) IS - 2152-2669 (Linking) VI - 12 IP - 5 DP - 2012 Oct TI - Nilotinib-associated vascular events. PG - 337-40 LID - S2152-2650(12)00083-3 [pii] LID - 10.1016/j.clml.2012.04.005 [doi] AB - Anecdotal evidence suggests that nilotinib therapy may be associated with severe peripheral artery occlusive disease (PAOD). The authors describe the experience at M.D. Anderson Cancer Center regarding vascular events associated with nilotinib therapy in patients with chronic myeloid leukemia. Overall, 5 cases of PAOD were identified among 233 patients, for an incidence of 2%. Nilotinib is a highly selective inhibitor of the inactive conformation of ABL1 kinase. An improved topologic fit to the ABL1 protein-binding surface contributes to its increased potency over imatinib. This higher selectivity in vitro translated to an improved tolerability in vivo. In fact, nilotinib therapy in the frontline phase III ENESTnd (Evaluating Nilotinib Efficacy and Safety in Clinical Trials-Newly Diagnosed Patients) study was associated with an improved toxicity profile compared with that of imatinib. Intriguingly, several cases of severe peripheral artery occlusive disease (PAOD) have been reported among patients treated with nilotinib in small series. We have identified 5 patients with chronic myeloid leukemia (CML) in whom vascular events developed that were likely related to nilotinib therapy among 233 (2%) patients treated at our institution: 1 patient had recurrent Raynaud syndrome, a second patient had recurrent cerebrovascular accidents, and 3 other patients had PAOD (2 of them with other vascular events, including coronary artery disease and pulmonary emboli, respectively). Risk factors for vascular disease were present in only 1 patient with a history of diabetes mellitus. Although the incidence of vascular events is low, this potential complication should be taken into account when selecting nilotinib for the treatment of CML. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Quintás-Cardama, Alfonso AU - Quintás-Cardama A AD - Department of Leukemia, the University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA. aquintas@mdanderson.org FAU - Kantarjian, Hagop AU - Kantarjian H FAU - Cortes, Jorge AU - Cortes J LA - eng PT - Case Reports PT - Journal Article DEP - 20120524 PL - United States TA - Clin Lymphoma Myeloma Leuk JT - Clinical lymphoma, myeloma & leukemia JID - 101525386 RN - 0 (Benzamides) RN - 0 (Piperazines) RN - 0 (Pyrimidines) RN - 8A1O1M485B (Imatinib Mesylate) RN - F41401512X (nilotinib) SB - IM MH - Aged MH - Benzamides/therapeutic use MH - Female MH - Humans MH - Imatinib Mesylate MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*chemically induced MH - Male MH - Middle Aged MH - Piperazines/therapeutic use MH - Pyrimidines/*adverse effects/therapeutic use MH - Risk Factors MH - Vascular Diseases/*chemically induced EDAT- 2012/05/29 06:00 MHDA- 2013/05/23 06:00 CRDT- 2012/05/29 06:00 PHST- 2012/02/04 00:00 [received] PHST- 2012/04/15 00:00 [revised] PHST- 2012/04/16 00:00 [accepted] PHST- 2012/05/29 06:00 [entrez] PHST- 2012/05/29 06:00 [pubmed] PHST- 2013/05/23 06:00 [medline] AID - S2152-2650(12)00083-3 [pii] AID - 10.1016/j.clml.2012.04.005 [doi] PST - ppublish SO - Clin Lymphoma Myeloma Leuk. 2012 Oct;12(5):337-40. doi: 10.1016/j.clml.2012.04.005. Epub 2012 May 24. PMID- 26003942 OWN - NLM STAT- MEDLINE DCOM- 20160328 LR - 20180929 IS - 1872-7549 (Electronic) IS - 0166-4328 (Linking) VI - 291 DP - 2015 Sep 15 TI - Chronic variable stress exposure in male Wistar rats affects the first step of olfactory detection. PG - 36-45 LID - S0166-4328(15)00342-3 [pii] LID - 10.1016/j.bbr.2015.05.013 [doi] AB - For most animal species, olfaction plays a paramount role in their perception of the environment. Odours are initially detected in neurons located in the olfactory mucosa. This tissue is regulated by several physiological signals and can be altered in pathology. A number of clinical studies suggest an association between depressive disorders and olfactory sensory loss. In rodents, depressive-like states can be observed in models of chronic stress. We tested the hypothesis that olfactory function might be altered in a rat model of depression, induced by chronic variable stress (CVS). While CVS rats exhibited several symptoms consistent with chronic stress exposure and depressive-like states (increased sucrose intake in sucrose preference test, increased immobility in forced swim test, hyperlocomotion), their odorant responses recorded at the olfactory mucosa level by electro-olfactogram were decreased. In addition we observed increased apoptosis markers in the olfactory mucosa using Western Blot. Our data are consistent with reduced olfactory capacities in a laboratory rat model of chronic stress and depression, in agreement with human clinical data; this warrants further mechanistic studies. Furthermore, this works raises the possibility that altered olfactory function might be a confounding factor in the behavioural testing of chronically stressed or depressed rats. CI - Copyright © 2015 Elsevier B.V. All rights reserved. FAU - Raynaud, Aurélien AU - Raynaud A AD - Institut National de la Recherche Agronomique (INRA), UR 1197 NeuroBiologie de l'Olfaction, Domaine de Vilvert, Jouy-en-Josas, France. FAU - Meunier, Nicolas AU - Meunier N AD - Institut National de la Recherche Agronomique (INRA), UR 1197 NeuroBiologie de l'Olfaction, Domaine de Vilvert, Jouy-en-Josas, France; Université de Versailles St-Quentin en Yvelines, Versailles, France. FAU - Acquistapace, Adrien AU - Acquistapace A AD - Institut National de la Recherche Agronomique (INRA), UR 1197 NeuroBiologie de l'Olfaction, Domaine de Vilvert, Jouy-en-Josas, France. FAU - Bombail, Vincent AU - Bombail V AD - Institut National de la Recherche Agronomique (INRA), UR 1197 NeuroBiologie de l'Olfaction, Domaine de Vilvert, Jouy-en-Josas, France. Electronic address: vincent.bombail@jouy.inra.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150520 PL - Netherlands TA - Behav Brain Res JT - Behavioural brain research JID - 8004872 RN - 0 (Dietary Sucrose) SB - IM MH - Animals MH - Apoptosis/physiology MH - Chronic Disease MH - Depressive Disorder/pathology/*physiopathology MH - Dietary Sucrose MH - Disease Models, Animal MH - Feeding Behavior/physiology MH - Male MH - Motor Activity/physiology MH - Neurons/physiology MH - Olfactory Mucosa/pathology/*physiopathology MH - Rats, Wistar MH - Smell/*physiology MH - Stress, Psychological/pathology/*physiopathology MH - Swimming/physiology OTO - NOTNLM OT - Chronic stress OT - Depression OT - Odorant response OT - Olfactory epithelium EDAT- 2015/05/25 06:00 MHDA- 2016/03/29 06:00 CRDT- 2015/05/25 06:00 PHST- 2015/02/25 00:00 [received] PHST- 2015/05/06 00:00 [revised] PHST- 2015/05/10 00:00 [accepted] PHST- 2015/05/25 06:00 [entrez] PHST- 2015/05/25 06:00 [pubmed] PHST- 2016/03/29 06:00 [medline] AID - S0166-4328(15)00342-3 [pii] AID - 10.1016/j.bbr.2015.05.013 [doi] PST - ppublish SO - Behav Brain Res. 2015 Sep 15;291:36-45. doi: 10.1016/j.bbr.2015.05.013. Epub 2015 May 20. PMID- 41610418 OWN - NLM STAT- In-Process DCOM- 20260423 LR - 20260606 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 147 IP - 17 DP - 2026 Apr 23 TI - Common variation at 1q23.3, 2p23.3, 2q33.3, and 2p21 influences the risk of acute myeloid leukemia. PG - 1958-1969 LID - 10.1182/blood.2025031266 [doi] AB - Acute myeloid leukemia (AML) is a complex hematologic malignancy with multiple disease subgroups defined by somatic mutations and heterogeneous outcomes. Although genome-wide association studies (GWAS) have identified a small number of common genetic variants influencing AML risk, the heritable component of this disease outside of familial susceptibility remains largely undefined. Here, we perform a meta-analysis of 4 published GWAS plus 2 new GWAS, totaling 4710 AML cases and 12 938 controls. We identify a new genome-wide significant risk locus for pan-AML at 2p23.3 (rs4665765; P = 1.35 × 10-8; EFR3B, POMC, DNMT3A, and DNAJC27), which also significantly associates with patient survival (P = 6.09 × 10-3). Our analysis also identifies 3 new genome-wide significant risk loci for disease subgroups, including AML with deletions of chromosome 5 and/or 7 at 1q23.3 (rs12078864; P = 7.0 × 10-10; DUSP23) and cytogenetically complex AML at 2q33.3 (rs12988876; P = 3.28 × 10-8; PARD3B) and 2p21 (rs79918355; P = 1.60 × 10-9; EPCAM). We also investigated loci previously associated with the risk of clonal hematopoiesis (CH) or CH of indeterminate potential and identified several variants associated with the risk of AML. Our results further inform on AML etiology and demonstrate the existence of disease subgroup specific risk loci. CI - © 2026 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies. FAU - Ranasinghe, Diyanath AU - Ranasinghe D AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Lin, Wei-Yu AU - Lin WY AUID- ORCID: 0000-0002-9267-7988 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Fordham, Sarah E AU - Fordham SE AUID- ORCID: 0000-0002-3778-8634 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Alharbi, Abrar AU - Alharbi A AUID- ORCID: 0009-0007-6466-653X AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. AD - Department of Clinical Laboratory Sciences, Faculty of Applied Medical Sciences, Taibah University, Medina, Saudi Arabia. FAU - Sunter, Nicola J AU - Sunter NJ AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Elstob, Claire AU - Elstob C AUID- ORCID: 0000-0003-4252-493X AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Nahari, Mohammed H AU - Nahari MH AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. AD - Department of Clinical Laboratory Sciences, College of Applied Medical Sciences, Najran University, Najran, Saudi Arabia. FAU - Xu, Yaobo AU - Xu Y AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Park, Catherine AU - Park C AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Hungate, Eric AU - Hungate E AD - University of Chicago Comprehensive Cancer Center, Chicago, IL. FAU - Quante, Anne AU - Quante A AUID- ORCID: 0000-0002-4973-3024 AD - Institute of Human Genetics, Technical University of Munich, TUM School of Medicine and Health, Klinikum rechts der Isar, TUM University Hospital, Munich, Germany. FAU - Strauch, Konstantin AU - Strauch K AD - Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany. AD - Chair of Genetic Epidemiology, Faculty of Medicine, Ludwig-Maximilians-Universität München, Munich, Germany. AD - Institute of Medical Biostatistics, Epidemiology and Informatics, University Medical Center, Johannes Gutenberg University, Mainz, Germany. FAU - Gieger, Christian AU - Gieger C AUID- ORCID: 0000-0001-6986-9554 AD - Chair of Genetic Epidemiology, Faculty of Medicine, Ludwig-Maximilians-Universität München, Munich, Germany. FAU - Skol, Andrew AU - Skol A AD - University of Chicago Comprehensive Cancer Center, Chicago, IL. FAU - Rahman, Thahira AU - Rahman T AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Sucheston-Campbell, Lara AU - Sucheston-Campbell L AD - Karmanos Cancer Institute and Department of Oncology, Wayne State University School of Medicine, Detroit, MI. FAU - Hahn, Theresa AU - Hahn T AUID- ORCID: 0000-0002-3835-8855 AD - Department of Cancer Prevention, Roswell Park Cancer Institute, Buffalo, NY. FAU - Clay-Gilmour, Alyssa I AU - Clay-Gilmour AI AD - Department of Epidemiology and Biostatistics, Arnold School of Public Health, University of South Carolina, Columbia, SC. FAU - Jones, Gail L AU - Jones GL AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom. FAU - Marr, Helen J AU - Marr HJ AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom. FAU - Jackson, Graham H AU - Jackson GH AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom. FAU - Menne, Tobias AU - Menne T AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom. FAU - Collin, Matthew AU - Collin M AUID- ORCID: 0000-0001-6585-9586 AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, United Kingdom. FAU - Ivey, Adam AU - Ivey A AD - Department of Pathology, Alfred Hospital, Melbourne, Australia. FAU - Hills, Robert K AU - Hills RK AD - Nuffield Department of Population Health, University of Oxford, Oxford, United Kingdom. FAU - Burnett, Alan K AU - Burnett AK AD - Paul O'Gorman Leukaemia Research Centre, University of Glasgow, Glasgow, United Kingdom. FAU - Russell, Nigel H AU - Russell NH AD - Department of Haematology, Guy's and St Thomas' Hospital, London, United Kingdom. FAU - Fitzgibbon, Jude AU - Fitzgibbon J AUID- ORCID: 0000-0002-9069-1866 AD - Barts Cancer Institute, Queen Mary University of London, London, United Kingdom. FAU - Larson, Richard A AU - Larson RA AUID- ORCID: 0000-0001-9168-3203 AD - University of Chicago Comprehensive Cancer Center, Chicago, IL. FAU - Le Beau, Michelle M AU - Le Beau MM AD - University of Chicago Comprehensive Cancer Center, Chicago, IL. FAU - Stock, Wendy AU - Stock W AD - University of Chicago Comprehensive Cancer Center, Chicago, IL. FAU - Heidenreich, Olaf AU - Heidenreich O AUID- ORCID: 0000-0001-5404-6483 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. AD - Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. AD - Department of Hematology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Enshaei, Amir AU - Enshaei A AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Gunasinghe, Dumni AU - Gunasinghe D AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Hawking, Zoë L AU - Hawking ZL AUID- ORCID: 0000-0002-2348-0516 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Heslop, Holly AU - Heslop H AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Nandana, Devi AU - Nandana D AUID- ORCID: 0009-0001-5031-4273 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Di, Bingjing AU - Di B AUID- ORCID: 0009-0001-3549-3541 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Plokhuta, Anna AU - Plokhuta A AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Brown, Imogen T AU - Brown IT AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Allsup, David J AU - Allsup DJ AUID- ORCID: 0000-0001-6159-6109 AD - Centre for Biomedicine, Hull York Medical School, University of Hull, Hull, United Kingdom. FAU - Houlston, Richard S AU - Houlston RS AD - Division of Genetics and Epidemiology, The Institute of Cancer Research, London, United Kingdom. FAU - Collins, Andrew AU - Collins A AD - School of Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, United Kingdom. FAU - Milne, Paul AU - Milne P AUID- ORCID: 0000-0002-8278-0463 AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Norden, Jean AU - Norden J AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Dickinson, Anne M AU - Dickinson AM AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Lendrem, Clare AU - Lendrem C AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Daly, Ann K AU - Daly AK AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Palm, Louise AU - Palm L AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, United Kingdom. FAU - Piechocki, Kim AU - Piechocki K AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, United Kingdom. FAU - Jeffries, Sally AU - Jeffries S AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, United Kingdom. FAU - Bornhäuser, Martin AU - Bornhäuser M AUID- ORCID: 0000-0002-5916-3029 AD - Department of Haematology, School of Cancer and Pharmaceutical Research, King's College London, London, United Kingdom. AD - National Center for Tumor Diseases, Partner site Dresden, Dresden, Germany. AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Röllig, Christoph AU - Röllig C AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Altmann, Heidi AU - Altmann H AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Ruhnke, Leo AU - Ruhnke L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Kunadt, Desiree AU - Kunadt D AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Wagenführ, Lisa AU - Wagenführ L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Cordell, Heather J AU - Cordell HJ AUID- ORCID: 0000-0002-1879-5572 AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Darlay, Rebecca AU - Darlay R AUID- ORCID: 0000-0002-7526-5527 AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom. FAU - Andersen, Mette K AU - Andersen MK AD - Department of Clinical Genetics, University Hospital Rigshospitalet, Copenhagen, Denmark. FAU - Fontana, Maria C AU - Fontana MC AD - Institute of Hematology "L. and A. Seràgnoli," University of Bologna, Bologna, Italy. AD - Hematology Unit, Ospedale S. Maria delle Croci, University of Bologna, Ravenna, Italy. FAU - Martinelli, Giovanni AU - Martinelli G AUID- ORCID: 0000-0002-1025-4210 AD - Institute of Hematology "L. and A. Seràgnoli," University of Bologna, Bologna, Italy. FAU - Marconi, Giovanni AU - Marconi G AUID- ORCID: 0000-0001-6309-0515 AD - Hematology Unit, Ospedale S. Maria delle Croci, University of Bologna, Ravenna, Italy. FAU - Sanz, Miguel A AU - Sanz MA AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain and CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cervera, José AU - Cervera J AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain and CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Gómez-Seguí, Inés AU - Gómez-Seguí I AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain and CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cluzeau, Thomas AU - Cluzeau T AUID- ORCID: 0000-0002-6745-1127 AD - CHU Pasteur, Service d'Hématologie Biologique, Université Côte d'Azur, Nice, France. FAU - Moreilhon, Chimène AU - Moreilhon C AUID- ORCID: 0009-0004-6573-3667 AD - CHU Pasteur, Service d'Hématologie Biologique, Université Côte d'Azur, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - CHU Pasteur, Service d'Hématologie Biologique, Université Côte d'Azur, Nice, France. FAU - Sill, Heinz AU - Sill H AUID- ORCID: 0000-0003-0993-4371 AD - Division of Hematology, Medical University of Graz, Graz, Austria. FAU - Voso, Maria Teresa AU - Voso MT AUID- ORCID: 0000-0002-6164-4761 AD - Dipartimento di Biomedicina e Prevenzione, Università di Roma Tor Vergata, Rome, Italy. FAU - Dombret, Hervé AU - Dombret H AD - Hôpital Saint-Louis, Institut Universitaire d'Hématologie, Université Paris Diderot, Paris, France. FAU - Cheok, Meyling AU - Cheok M AUID- ORCID: 0000-0002-7820-8026 AD - Laboratory of Haematology, CHU Lille, Lille, France. FAU - Preudhomme, Claude AU - Preudhomme C AUID- ORCID: 0000-0002-1267-9546 AD - Laboratory of Haematology, CHU Lille, Lille, France. FAU - Gale, Rosemary E AU - Gale RE AUID- ORCID: 0000-0002-0103-1787 AD - Department of Haematology, University College London Cancer Institute, London, United Kingdom. FAU - Linch, David AU - Linch D AD - Department of Haematology, University College London Cancer Institute, London, United Kingdom. FAU - Weisinger, Julia AU - Weisinger J AUID- ORCID: 0009-0003-8337-2925 AD - 1st Department of Internal Medicine, Semmelweis University, Budapest, Hungary. FAU - Masszi, Andras AU - Masszi A AD - Hematology and Lymphoma Unit, National Institute of Oncology, Budapest, Hungary. FAU - Nowak, Daniel AU - Nowak D AUID- ORCID: 0000-0001-7130-7921 AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Hofmann, Wolf-Karsten AU - Hofmann WK AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Gilkes, Amanda AU - Gilkes A AD - Department of Haematology, University of Cardiff, Cardiff, United Kingdom. FAU - Porkka, Kimmo AU - Porkka K AUID- ORCID: 0000-0003-4112-5902 AD - Helsinki University Hospital Comprehensive Cancer Center, Hematology Research Unit Helsinki, University of Helsinki, Helsinki, Finland. FAU - Milosevic Feenstra, Jelena D AU - Milosevic Feenstra JD AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Kralovics, Robert AU - Kralovics R AUID- ORCID: 0000-0002-6997-8539 AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Wang, Junke AU - Wang J AD - Department of Systems Biology, UT MD Anderson Cancer Center, Houston TX. FAU - Meggendorfer, Manja AU - Meggendorfer M AD - Munich Leukemia Laboratory, Munich, Germany. FAU - Haferlach, Torsten AU - Haferlach T AD - Munich Leukemia Laboratory, Munich, Germany. FAU - Krizsán, Szilvia AU - Krizsán S AUID- ORCID: 0000-0002-1782-9275 AD - MTA-SE Lendulet Molecular Oncohematology Research Group, Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. AD - Pediatric Center, Semmelweis University, Budapest, Hungary. FAU - Bödör, Csaba AU - Bödör C AD - MTA-SE Lendulet Molecular Oncohematology Research Group, Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. FAU - Parkin, Brian AU - Parkin B AUID- ORCID: 0000-0003-4692-1027 AD - Division of Hematology and Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI. FAU - Malek, Sami N AU - Malek SN AD - Division of Hematology and Oncology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI. FAU - Stölzel, Friedrich AU - Stölzel F AUID- ORCID: 0000-0003-0653-5332 AD - Division of Stem Cell Transplantation and Cellular Immunotherapies, University Hospital Schleswig-Holstein, Kiel, Germany. FAU - Onel, Kenan AU - Onel K AUID- ORCID: 0000-0002-2021-6250 AD - Department of Cancer Prevention and Control, Roswell Park Comprehensive Cancer Center, Buffalo, NY. AD - Department of Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY. AD - Department of Clinical Genomics, Roswell Park Comprehensive Cancer Center, Buffalo, NY. FAU - Allan, James M AU - Allan JM AUID- ORCID: 0000-0002-7580-5087 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom. LA - eng PT - Journal Article PL - United States TA - Blood JT - Blood JID - 7603509 SB - IM CIN - Blood. 2026 Apr 23;147(17):1894-1896. doi: 10.1182/blood.2025032945. PMID: 42024400 MH - Humans MH - *Leukemia, Myeloid, Acute/genetics/mortality MH - Genome-Wide Association Study MH - *Genetic Predisposition to Disease MH - *Polymorphism, Single Nucleotide MH - *Chromosomes, Human, Pair 2/genetics MH - *Chromosomes, Human, Pair 1/genetics EDAT- 2026/01/29 19:11 MHDA- 2026/04/23 12:53 CRDT- 2026/01/29 16:53 PHST- 2025/12/19 00:00 [accepted] PHST- 2025/08/19 00:00 [received] PHST- 2026/04/23 12:53 [medline] PHST- 2026/01/29 19:11 [pubmed] PHST- 2026/01/29 16:53 [entrez] AID - 566332 [pii] AID - 10.1182/blood.2025031266 [doi] PST - ppublish SO - Blood. 2026 Apr 23;147(17):1958-1969. doi: 10.1182/blood.2025031266. PMID- 29198478 OWN - NLM STAT- MEDLINE DCOM- 20200121 LR - 20200121 IS - 1545-004X (Electronic) IS - 0894-1130 (Linking) VI - 32 IP - 3 DP - 2019 Jul-Sep TI - The evaluation of a home-based program for hands in patients with systemic sclerosis. PG - 313-321 LID - S0894-1130(17)30025-X [pii] LID - 10.1016/j.jht.2017.10.013 [doi] AB - STUDY DESIGN: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program. INTRODUCTION: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease. PURPOSE OF THE STUDY: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc. METHODS: A home-based self-management program that consisted of concise instructions about SSc and hand exercises was developed and evaluated in a group of patients with SSc during 8 weeks. Primary outcome measures were hand pain (Visual Analogue Scale) and hand function (Cochin Hand Function Scale). Secondary outcome measures were disability (Scleroderma Health Assessment Questionnaire), finger motion (delta finger-to-palm), grip strength, tip and key pinch strength, Raynaud phenomenon and digital ulcers impact, quality of life (Short Form Health Survey). For comparisons between different times analysis of variance for repeated measures was used. To calculate the effect size (ES), the Cohen's test was performed. To evaluate skin moisturizing and warming habits before and after intervention, the McNemar test was used. Statistical significance was set at P ≤ .05. RESULTS: Twenty-two SSc patients (19 women: 3 men; 16 limited scleroderma: 6 diffuse scleroderma) completed the program. Significant improvements were noted for hand pain (3.97 vs 2.21, ES: 0.69), Cochin Hand Function Scale (19.24 vs 12.48, ES: 0.48), Scleroderma Health Assessment Questionnaire (0.95 vs 0.48, ES: 1.01), delta finger-to-palm (92.86 vs 106.33, ES: 0.40), grip strength (14.43 vs 19, ES: 0.58), tip pinch strength (2.49 vs 4.18, ES: 1.15), key pinch strength (4.01 vs 5.22, ES: 0.76), Raynaud phenomenon impact (0.94 vs 0.47, ES: 0.75), Short Form Health Survey-role physical (47.38 vs 60.14, ES: 0.61), physical functioning (34.62 vs 61.9, ES: 0.18), social functioning (60.71 vs 75.6, ES: 0.64), bodily pain (50.55 vs 63.38, ES: 0.58), vitality (45.95 vs 62, ES: 2.22), mental health (56.62 vs 72.38, ES: 0.84) moisturizing, and cold avoidance habits. Patients considered the program easy to follow with no adverse effects related to exercises. DISCUSSION: We developed a home based hand care program to be offered to SSc patients. Improvements in hand function, strength, disability, motion, and overall quality of life were independent of age, income, education level, disease duration, and skin score. Our findings support those of other studies that reported the benefits of hand exercises in SSc. Some study limitations include the lack of a control group, the small number of subjects and the short-time follow up. CONCLUSIONS: This home-based program for patients with SSc improved hand pain, function, mobility, and strength at the end of 8 weeks. Patient adherence and sustained efficacy is still to be determined. CI - Copyright © 2017 Hanley & Belfus. Published by Elsevier Inc. All rights reserved. FAU - Landim, Sibila Floriano AU - Landim SF AD - Department of Internal Medicine and Rheumatology, Faculty of Medical Sciences, University of Campinas-Unicamp, Campinas, São Paulo, Brazil. FAU - Bertolo, Manoel Barros AU - Bertolo MB AD - Department of Internal Medicine and Rheumatology, Faculty of Medical Sciences, University of Campinas-Unicamp, Campinas, São Paulo, Brazil. FAU - Marcatto de Abreu, Marcos Felipe AU - Marcatto de Abreu MF AD - Department of Orthopedics and Rheumatology, Clinical Hospital of the State University of Campinas - Unicamp, Campinas, São Paulo, Brazil. FAU - Del Rio, Ana Paula AU - Del Rio AP AD - Department of Orthopedics and Rheumatology, Clinical Hospital of the State University of Campinas - Unicamp, Campinas, São Paulo, Brazil. FAU - Mazon, Cecilia Carmen AU - Mazon CC AD - Department of Orthopedics and Rheumatology, Clinical Hospital of the State University of Campinas - Unicamp, Campinas, São Paulo, Brazil. FAU - Marques-Neto, João Francisco AU - Marques-Neto JF AD - Department of Internal Medicine and Rheumatology, Faculty of Medical Sciences, University of Campinas-Unicamp, Campinas, São Paulo, Brazil. FAU - Poole, Janet L AU - Poole JL AD - Department of Pediatrics, Occupational Therapy Graduate Program, School of Medicine, University of New Mexico, Albuquerque, NM, USA. FAU - de Paiva Magalhães, Eduardo AU - de Paiva Magalhães E AD - Department of Orthopedics and Rheumatology, Clinical Hospital of the State University of Campinas - Unicamp, Campinas, São Paulo, Brazil. Electronic address: dreduardomagalhaes@gmail.com. LA - eng PT - Journal Article DEP - 20171201 PL - United States TA - J Hand Ther JT - Journal of hand therapy : official journal of the American Society of Hand Therapists JID - 8806591 SB - IM MH - Adult MH - Aged MH - Disability Evaluation MH - *Exercise Therapy MH - Female MH - Hand/*physiopathology MH - Hand Strength MH - Humans MH - Male MH - Middle Aged MH - Program Evaluation MH - Quality of Life MH - Scleroderma, Diffuse/physiopathology/*rehabilitation MH - Scleroderma, Limited/physiopathology/*rehabilitation MH - *Self-Management MH - Visual Analog Scale OTO - NOTNLM OT - Hand involvement OT - Occupational therapy OT - Self-management OT - Systemic sclerosis EDAT- 2017/12/05 06:00 MHDA- 2020/01/22 06:00 CRDT- 2017/12/05 06:00 PHST- 2017/02/03 00:00 [received] PHST- 2017/10/18 00:00 [revised] PHST- 2017/10/27 00:00 [accepted] PHST- 2017/12/05 06:00 [pubmed] PHST- 2020/01/22 06:00 [medline] PHST- 2017/12/05 06:00 [entrez] AID - S0894-1130(17)30025-X [pii] AID - 10.1016/j.jht.2017.10.013 [doi] PST - ppublish SO - J Hand Ther. 2019 Jul-Sep;32(3):313-321. doi: 10.1016/j.jht.2017.10.013. Epub 2017 Dec 1. PMID- 23557151 OWN - NLM STAT- MEDLINE DCOM- 20141106 LR - 20131029 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 52 IP - 11 DP - 2013 Nov TI - Cutaneous manifestations in patients with POEMS syndrome. PG - 1349-56 LID - 10.1111/j.1365-4632.2012.05648.x [doi] AB - BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia. METHODS: From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. We assessed prevalence of skin findings and associations between dermatologic and other characteristic disease findings. RESULTS: Of the 107 patients, 96 (90%) had a recognized cutaneous manifestation. Hyperpigmentation and hemangioma were most common (47%), followed by hypertrichosis (38%). Vascular skin changes--acrocyanosis (34%), Raynaud phenomenon (20%), hyperemia/erythema (20%), flushing (16%), or rubor (11%)--occurred in 62%; white nails, sclerodermoid changes, and clubbing occurred in 30%, 26%, and 6%, respectively. Mean number of skin findings per patient was 2.9 (median, 3.0; range, 0-7). Presence of cutaneous manifestation was associated with abnormal pulmonary function tests (P < 0.001); immunoglobulin G gammopathy was associated with hyperpigmentation and hypertrichosis. No other significant associations were seen. CONCLUSIONS: The high prevalence of skin findings (90%) shows the value of dermatologic evaluation in diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Our data indicate new associations between skin findings and other disease characteristics. CI - © 2013 The International Society of Dermatology. FAU - Miest, Rachel Y N AU - Miest RY AD - Department of Dermatology, Mayo Clinic, RochesterDepartment of Laboratory Medicine and Pathology, Mayo Clinic, RochesterDivision of Hematology, Mayo Clinic, RochesterDivision of Clinical Biochemistry and Immunology, Mayo Clinic, RochesterDivision of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA. FAU - Comfere, Nneka I AU - Comfere NI FAU - Dispenzieri, Angela AU - Dispenzieri A FAU - Lohse, Christine M AU - Lohse CM FAU - el-Azhary, Rokea A AU - el-Azhary RA LA - eng PT - Journal Article DEP - 20130404 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - Leukonychia totalis SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Female MH - Hemangioma/*epidemiology MH - Humans MH - Hyperpigmentation/*epidemiology MH - Hypertrichosis/*epidemiology MH - Hypopigmentation/epidemiology MH - Male MH - Middle Aged MH - Nail Diseases/congenital/epidemiology MH - Osteoarthropathy, Primary Hypertrophic/epidemiology MH - POEMS Syndrome/*diagnosis MH - Prevalence MH - Skin Diseases, Vascular/*epidemiology MH - Skin Neoplasms/*epidemiology MH - Young Adult EDAT- 2013/04/06 06:00 MHDA- 2014/11/07 06:00 CRDT- 2013/04/06 06:00 PHST- 2013/04/06 06:00 [entrez] PHST- 2013/04/06 06:00 [pubmed] PHST- 2014/11/07 06:00 [medline] AID - 10.1111/j.1365-4632.2012.05648.x [doi] PST - ppublish SO - Int J Dermatol. 2013 Nov;52(11):1349-56. doi: 10.1111/j.1365-4632.2012.05648.x. Epub 2013 Apr 4. PMID- 42220692 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260601 LR - 20260601 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 18 IP - 4 DP - 2026 Apr TI - Coexistence of Proteinase 3 (PR3)-Positive Granulomatosis With Polyangiitis and Genetically Confirmed Alport Syndrome in a 31-Year-Old Female Patient: A Diagnostic and Management Challenge. PG - e107982 LID - 10.7759/cureus.107982 [doi] LID - e107982 AB - Distinguishing autoimmune glomerulonephritis from hereditary nephropathy is critical to avoid unnecessary immunosuppression and ensure appropriate long-term renal monitoring. A 31-year-old Hispanic female patient with a complex medical history, including proteinase 3 (PR3)-positive granulomatosis with polyangiitis (GPA), rheumatoid arthritis, Raynaud phenomenon, seizure disorder, autism spectrum disorder, bipolar disorder with psychotic features, and cochlear implantation in childhood, was found to have genetically confirmed Alport syndrome through Natera testing (Natera, Inc., Austin, Texas, United States). Despite preserved renal function (estimated glomerular filtration rate (eGFR) 107 mL/minute/1.73m²), urinalysis demonstrated persistent proteinuria. Her history of early-onset hearing loss requiring cochlear implantation raised suspicion for hereditary nephropathy. Elevated anti-PR3 antibodies were present without clinical evidence of active vasculitis. The coexistence of autoimmune disease and hereditary collagen IV nephropathy complicated the attribution of urinary abnormalities. This case highlights the importance of genetic evaluation in patients with multisystem disease and urinary abnormalities, particularly when clinical features such as early-onset sensorineural hearing loss are present. Recognition of Alport syndrome in patients with coexisting autoimmune vasculitis has significant implications for renal surveillance, immunosuppressive stewardship, and multidisciplinary management. CI - Copyright © 2026, Valdes et al. FAU - Valdes, Laura AU - Valdes L AD - Department of Internal Medicine, Larkin Community Hospital, South Miami, USA. FAU - Ludena, Angelica AU - Ludena A AD - Department of Internal Medicine, Larkin Community Hospital, South Miami, USA. FAU - Pantoja Miranda, Juan Carlos AU - Pantoja Miranda JC AD - Department of Internal Medicine, Larkin Community Hospital, South Miami, USA. FAU - Ortiz, Joham AU - Ortiz J AD - Department of Internal Medicine, Larkin Community Hospital, South Miami, USA. FAU - Angly, Sohair AU - Angly S AD - Department of Internal Medicine, Larkin Community Hospital, South Miami, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20260429 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC13221106 OTO - NOTNLM OT - alport syndrome OT - genetic test OT - granulomatosis with polyangiitis (gpa) OT - hereditary diseases OT - pr3-anca COIS- Human subjects: Informed consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/06/01 08:04 MHDA- 2026/06/01 08:05 PMCR- 2026/04/29 CRDT- 2026/06/01 05:58 PHST- 2026/04/29 00:00 [accepted] PHST- 2026/06/01 08:05 [medline] PHST- 2026/06/01 08:04 [pubmed] PHST- 2026/06/01 05:58 [entrez] PHST- 2026/04/29 00:00 [pmc-release] AID - 10.7759/cureus.107982 [doi] PST - epublish SO - Cureus. 2026 Apr 29;18(4):e107982. doi: 10.7759/cureus.107982. eCollection 2026 Apr. PMID- 18333373 OWN - NLM STAT- MEDLINE DCOM- 20080422 LR - 20080312 IS - 1220-4749 (Print) IS - 1220-4749 (Linking) VI - 45 IP - 2 DP - 2007 TI - Platelet activation in patients with systemic scleroderma--pattern and significance. PG - 183-91 AB - The vasooclusive features of scleroderma are attributed to the vessel wall anomalies, while platelet's intervention is less studied. AIM: platelet activation markers (PAM) pattern and significance in systemic sclerosis. DESIGN: 20 scleroderma patients with severe Raynaud phenomenon, under aspirin treatment, were evaluated by quantitative flow-cytometry for PAM (P-selectin, GPIIbIIIa, CD40L) in correlation with scleroderma activity and severity, systemic endothelial dysfunction (flow-mediated vasodilatation), systemic inflammation (serum CRP and IL-6) and cold-provocation test. Associated autoantibodies (ANCA, antiTPO, anticardiolipin, antiplatelet, antimitochondrial antibodies) were evaluated in relation to IL-6. RESULTS: PAM were expressed in 11 (55%) cases: P-selectin in 5 (45.45%), GPIIbIIIa in 1 (9.1%), combined P-selectin and GPIIbIIIa in 5 (45.45%), CD40L in 0 cases. Scleroderma patients expressing PAM had increased incidence of disease activity and severity. There was no correlation between PAM and systemic endothelial dysfunction. CRP increased in 14(70%) cases was correlated with P-selectin and GPIIbIIIa expression (r = 0.28). Normal IL6 present in 19 (90.9%) cases was correlated with lack of CD40L expression (r = 0.69) and with low autoantibodies incidence (r = 0.69 for ANCA, 0.55 for anti TPO and antiplatelet, 0.39 for anti cardiolipin, 0.45 for antimitochondrial). After cold provocation test PAM were significantly lost and were not expressed de novo. CONCLUSIONS: In systemic scleroderma platelet activation markers are correlated with disease activity and severity and increased CRP and are not correlated with systemic endothelial dysfunction or exposure to cold. Normal IL-6 was correlated with lack of CD40L expression and with low incidence of associated autoantibodies. FAU - Agache, Ioana AU - Agache I AD - Department of Clinical Immunology, Transylvania University, Faculty of Medicine. ibrumaru@unitbv.ro FAU - Rădoi, Mariana AU - Rădoi M FAU - Duca, Liliana AU - Duca L LA - eng PT - Journal Article PL - Germany TA - Rom J Intern Med JT - Romanian journal of internal medicine = Revue roumaine de medecine interne JID - 9304507 RN - 0 (Autoantibodies) RN - 0 (P-Selectin) RN - 0 (Platelet Glycoprotein GPIIb-IIIa Complex) RN - 147205-72-9 (CD40 Ligand) RN - 9007-41-4 (C-Reactive Protein) SB - IM MH - Adult MH - Aged MH - Autoantibodies/blood MH - C-Reactive Protein/*immunology MH - CD40 Ligand/blood MH - Cohort Studies MH - Female MH - Flow Cytometry MH - Humans MH - Male MH - Middle Aged MH - P-Selectin/*blood MH - *Platelet Activation MH - Platelet Glycoprotein GPIIb-IIIa Complex/*metabolism MH - Scleroderma, Systemic/*blood MH - Severity of Illness Index EDAT- 2008/03/13 09:00 MHDA- 2008/04/23 09:00 CRDT- 2008/03/13 09:00 PHST- 2008/03/13 09:00 [pubmed] PHST- 2008/04/23 09:00 [medline] PHST- 2008/03/13 09:00 [entrez] PST - ppublish SO - Rom J Intern Med. 2007;45(2):183-91. PMID- 38711778 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240508 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 11 DP - 2024 TI - Presenting clinical and imaging features of patients with clinically amyopathic interstitial lung disease associated with myositis-specific autoantibodies. PG - 1392659 LID - 10.3389/fmed.2024.1392659 [doi] LID - 1392659 AB - BACKGROUND: Lung involvement in the context of idiopathic inflammatory myopathies has significant impact on outcome; early and accurate diagnosis is important but can be difficult to achieve. In particular, patients without clinically evident muscle involvement pose a significant diagnostic challenge. METHODS: A computer-assisted search was conducted to identify patients with amyopathic interstitial lung disease associated with the presence of myositis-specific autoantibodies. Medical records and chest imaging studies were reviewed to identify clinical and radiologic features at presentation. RESULTS: Of the 35 patients with amyopathic interstitial lung disease associated with myositis-specific autoantibodies, the median age was 65 years (range 43-78) and 20 were women (57%). Of the patients, 34% had previously visited the rheumatology department. Presenting symptoms consisted of dyspnea (94%), cough (43%), and arthritis (23%). Raynaud phenomenon, "mechanic hands," Gottron papules, and inspiratory crackles were present in 23, 31, 9, and 74% of patients, respectively. After a detailed history, none of the patients reported muscle weakness, while four (11%) exhibited increased CK levels; of these four, two had a concomitant increase in aldolase levels. Median FVC was 79% predicted (range: 49-135) and median DLco was 50% predicted (range: 17-103). HRCT pattern was suggestive of an alternative to UIP pattern in 31/33 (94%) patients; the most common imaging patterns were NSIP (49%) and NSIP/OP (39%). CONCLUSION: In patients with NSIP and NSIP/OP pattern, the presence of amyopathic interstitial lung disease associated with myositis-specific autoantibodies should be considered even in the absence of clinical evident myositis. CI - Copyright © 2024 Tzilas, Tzouvelekis, Sotiropoulou, Panopoulos, Bouros, Avdoula, Ryu and Bouros. FAU - Tzilas, Vasilios AU - Tzilas V AD - 5th Respiratory Department, Chest Diseases Hospital "Sotiria", Athens, Greece. FAU - Tzouvelekis, Argyrios AU - Tzouvelekis A AD - Department of Respiratory Medicine, Medical School, University of Patras, Patras, Greece. FAU - Sotiropoulou, Vasilina AU - Sotiropoulou V AD - Department of Respiratory Medicine, Medical School, University of Patras, Patras, Greece. FAU - Panopoulos, Stylianos AU - Panopoulos S AD - 1st Department of Propaedeutic and Internal Medicine, and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece. FAU - Bouros, Evangelos AU - Bouros E AD - Athens Medical Center, Athens, Greece. FAU - Avdoula, Eleni AU - Avdoula E AD - Athens Medical Center, Athens, Greece. FAU - Ryu, Jay H AU - Ryu JH AD - Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, United States. FAU - Bouros, Demosthenes AU - Bouros D AD - 1st Department of Respiratory Medicine, Medical School, National Kapodistrian University of Athens, and Athens Medical Center, Athens, Greece. LA - eng PT - Journal Article DEP - 20240422 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC11070581 OTO - NOTNLM OT - HRCT OT - amyopathic OT - antisynthetase syndrome OT - idiopathic inflammatory myopathies OT - myositis OT - non specific interstitial pneumonia OT - organizing pneumonia COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2024/05/07 06:42 MHDA- 2024/05/07 06:43 PMCR- 2024/04/22 CRDT- 2024/05/07 03:39 PHST- 2024/02/27 00:00 [received] PHST- 2024/04/09 00:00 [accepted] PHST- 2024/05/07 06:43 [medline] PHST- 2024/05/07 06:42 [pubmed] PHST- 2024/05/07 03:39 [entrez] PHST- 2024/04/22 00:00 [pmc-release] AID - 10.3389/fmed.2024.1392659 [doi] PST - epublish SO - Front Med (Lausanne). 2024 Apr 22;11:1392659. doi: 10.3389/fmed.2024.1392659. eCollection 2024. PMID- 23519183 OWN - NLM STAT- MEDLINE DCOM- 20130926 LR - 20130327 IS - 1536-7355 (Electronic) IS - 1076-1608 (Linking) VI - 19 IP - 3 DP - 2013 Apr TI - Cryofibrinogenemia. PG - 142-8 LID - 10.1097/RHU.0b013e318289e06e [doi] AB - Cryofibrinogenemia is a cryoprotein that was first identified in 1955 by Korst and Kratochvil. Unlike cryoglobulin, the precipitate forms only in plasma and not in the serum. The presence of cryofibrinogen in plasma can be asymptomatic. Cryofibrinogenemia is considered a rare disorder: its prevalence varies from 0% to 7% in healthy subjects and from 8% to 13% in hospitalized patients. Nevertheless, cryofibrinogenemia, when a cryopathy is clinically suspected, has been reported in 12% to 51% of patients. Skin manifestations are usually the first signs and are usually moderate; in addition, cold intolerance, Raynaud phenomenon, purpura, or livedo reticularis often occurs. Skin necrosis, acral ulcers, and gangrene can lead to surgery and amputation. Systemic manifestations are common, and arterial or venous thrombotic events are frequent. Cryofibrinogenemia may be primary (essential) or secondary to other underlying disorders, such as carcinoma, infection, vasculitis, collagen disease, or associated with cryoglobulinemia. The histological features of cryofibrinogenemia can confirm the presence of cryofibrinogen within small and medium arteries, plus occlusive thrombotic diathesis composed of eosinophilic refractile deposits within vessel lumina. Cryofibrinogenemia is a treatable and potentially reversible disease.In moderate forms, it can be treated by simply avoiding cold temperatures. The use of corticosteroids in association with low-dose aspirin is the treatment of choice for moderate forms, although stanozolol is an alternative maintenance therapy. Immunosuppressive therapies, plasmapheresis, and/or intravenous fibrinolysis are useful at treating severe forms of cryofibrinogenemia. The use of anticoagulants is limited to the management of thrombotic events. Treatment of secondary cryofibrinogenemia involves the management of associated diseases. Regular follow-ups are needed because of the high risk of recurrence. Moreover, up to half of patients with cryofibrinogenemia considered as essential may develop lymphomas in the following years. Compared with cryoglobulinemia, less is known about cryofibrinogenemia. Its diagnosis should be considered when suggestive clinical manifestations are present and when there are specific biopsy findings. Although identification of cryofibrinogen in blood samples is simple and inexpensive, cryofibrinogenemia can be asymptomatic, and a lack of diagnosis criteria can make diagnosis difficult to confirm. This review describes the clinical manifestations and the biological and pathological features and discusses the criteria used to diagnose and manage cryofibrinogenemia. FAU - Michaud, Martin AU - Michaud M AD - Departments of Internal Medicine, CHU Toulouse, France. martin.michaud85@gmail.com FAU - Pourrat, Jacques AU - Pourrat J LA - eng PT - Journal Article PT - Review PL - United States TA - J Clin Rheumatol JT - Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases JID - 9518034 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Cryoglobulins) RN - 0 (Fibrinogens, Abnormal) RN - 0 (Immunosuppressive Agents) RN - 0 (cryofibrinogen) RN - Cryofibrinogenemia SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Cold Temperature/adverse effects MH - Cryoglobulinemia/blood/*diagnosis/*pathology MH - Cryoglobulins/metabolism MH - *Disease Management MH - Female MH - Fibrinogens, Abnormal/metabolism MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Plasmapheresis EDAT- 2013/03/23 06:00 MHDA- 2013/09/27 06:00 CRDT- 2013/03/23 06:00 PHST- 2013/03/23 06:00 [entrez] PHST- 2013/03/23 06:00 [pubmed] PHST- 2013/09/27 06:00 [medline] AID - 10.1097/RHU.0b013e318289e06e [doi] PST - ppublish SO - J Clin Rheumatol. 2013 Apr;19(3):142-8. doi: 10.1097/RHU.0b013e318289e06e. PMID- 19228661 OWN - NLM STAT- MEDLINE DCOM- 20090521 LR - 20211020 IS - 0315-162X (Print) IS - 0315-162X (Linking) VI - 36 IP - 3 DP - 2009 Mar TI - Abnormalities in the regulators of angiogenesis in patients with scleroderma. PG - 576-82 LID - 10.3899/jrheum.080516 [doi] AB - OBJECTIVE: To determine plasma levels of regulators of angiogenesis in patients with scleroderma and to correlate those levels with manifestations of scleroderma-related vascular disease. METHODS: Plasma levels of vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), fibroblast growth factor-2 (FGF-2), matrix metalloproteinase-9 (MMP-9), endostatin, pro-MMP-1, hepatocyte growth factor (HGF), placental growth factor (PlGF), and FGF-4 were examined by ELISA in a cross-sectional study of 113 patients with scleroderma and 27 healthy controls. Simple and multivariate regression models were used to look for associations between factor levels and clinical disease characteristics. RESULTS: There were marked differences in the levels of pro-angiogenic growth factors between patients with scleroderma and controls, with significant elevations of VEGF, PDGF, FGF-2, and PlGF among patients with scleroderma (p < 0.0001). Levels of MMP were also higher in scleroderma patients compared to controls (MMP-9 and pro-MMP-1) (p < 0.0001). Levels of the pro-angiogenic and anti-fibrotic factor, HGF, were noted to be lower in patients with scleroderma, but had a positive correlation with right ventricular systolic pressure (RVSP) as measured by echocardiogram (p < 0.0001) and the Raynaud Severity Score (p = 0.05). Endostatin (an anti-angiogenic factor) was notably higher in patients with scleroderma (p < 0.0001) and also correlated positively with RVSP (p = 0.023). CONCLUSION: These results demonstrate striking abnormalities in the circulating regulators of angiogenesis in patients with scleroderma. The levels of some factors correlate with measures of vascular disease among patients with scleroderma. Dysregulated angiogenesis may play a role in the development of scleroderma vascular disease. FAU - Hummers, Laura K AU - Hummers LK AD - Department of Medicine, Johns Hopkins University, Baltimore, MD 21224, USA. lhummers@jhmi.edu FAU - Hall, Amy AU - Hall A FAU - Wigley, Fredrick M AU - Wigley FM FAU - Simons, Michael AU - Simons M LA - eng GR - K23 AR052742/AR/NIAMS NIH HHS/United States GR - R01 HL062289/HL/NHLBI NIH HHS/United States GR - K23AR052742-01/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20090217 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Angiogenic Proteins) SB - IM MH - Angiogenic Proteins/*blood MH - Case-Control Studies MH - Female MH - Humans MH - Hypertension, Pulmonary/blood/complications MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*blood/complications PMC - PMC4020014 MID - NIHMS580619 EDAT- 2009/02/21 09:00 MHDA- 2009/05/22 09:00 PMCR- 2014/05/14 CRDT- 2009/02/21 09:00 PHST- 2009/02/21 09:00 [entrez] PHST- 2009/02/21 09:00 [pubmed] PHST- 2009/05/22 09:00 [medline] PHST- 2014/05/14 00:00 [pmc-release] AID - jrheum.080516 [pii] AID - 10.3899/jrheum.080516 [doi] PST - ppublish SO - J Rheumatol. 2009 Mar;36(3):576-82. doi: 10.3899/jrheum.080516. Epub 2009 Feb 17. PMID- 23964148 OWN - NLM STAT- MEDLINE DCOM- 20140129 LR - 20211021 IS - 2219-2840 (Electronic) IS - 1007-9327 (Print) IS - 1007-9327 (Linking) VI - 19 IP - 31 DP - 2013 Aug 21 TI - Connective tissue diseases in primary biliary cirrhosis: a population-based cohort study. PG - 5131-7 LID - 10.3748/wjg.v19.i31.5131 [doi] AB - AIM: To establish the frequency and clinical features of connective tissue diseases (CTDs) in a cohort of Chinese patients with primary biliary cirrhosis (PBC). METHODS: Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD, and the systemic involvement was assessed. The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented. The diversity of incidence of CTDs in PBC of different countries and areas was discussed. For the comparison of normally distributed data, Student's t test was used, while non-parametric test (Wilcoxon test) for the non-normally distributed data and 2 × 2 χ(2) or Fisher's exact tests for the ratio. RESULTS: One-hundred and fifty (46.6%) PBC patients had one or more CTDs. The most common CTD was Sjögren's syndrome (SS, 121 cases, 36.2%). There were nine cases of systemic sclerosis (SSc, 2.8%), 12 of systemic lupus erythematosus (SLE, 3.7%), nine of rheumatoid arthritis (RA, 2.8%), and 10 of polymyositis (PM, 3.1%) in this cohort. Compared to patients with PBC only, the PBC + SS patients were more likely to have fever and elevated erythrocyte sedimentation rate (ESR), higher serum immunoglobulin G (IgG) levels and more frequent rheumatoid factor (RF) and interstitial lung disease (ILD) incidences; PBC + SSc patients had higher frequency of ILD; PBC + SLE patients had lower white blood cell (WBC) count, hemoglobin (Hb), platelet count, γ-glutamyl transpeptidase and immunoglobulin M levels, but higher frequency of renal involvement; PBC + RA patients had lower Hb, higher serum IgG, alkaline phosphatase, faster ESR and a higher ratio of RF positivity; PBC + PM patients had higher WBC count and a tendency towards myocardial involvement. CONCLUSION: Besides the common liver manifestation of PBC, systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients. When overlapping with other CTDs, PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis. FAU - Wang, Li AU - Wang L AD - Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China. FAU - Zhang, Feng-Chun AU - Zhang FC FAU - Chen, Hua AU - Chen H FAU - Zhang, Xuan AU - Zhang X FAU - Xu, Dong AU - Xu D FAU - Li, Yong-Zhe AU - Li YZ FAU - Wang, Qian AU - Wang Q FAU - Gao, Li-Xia AU - Gao LX FAU - Yang, Yun-Jiao AU - Yang YJ FAU - Kong, Fang AU - Kong F FAU - Wang, Ke AU - Wang K LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - World J Gastroenterol JT - World journal of gastroenterology JID - 100883448 SB - IM MH - Chi-Square Distribution MH - China/epidemiology MH - Connective Tissue Diseases/diagnosis/*epidemiology MH - Humans MH - Incidence MH - Liver Cirrhosis, Biliary/diagnosis/*epidemiology MH - Retrospective Studies PMC - PMC3746386 OTO - NOTNLM OT - Biliary OT - Cirrhosis OT - Connective tissue disease OT - Raynaud phenomenon OT - Sjögren’s syndrome OT - Systemic sclerosis EDAT- 2013/08/22 06:00 MHDA- 2014/01/30 06:00 PMCR- 2013/08/21 CRDT- 2013/08/22 06:00 PHST- 2013/04/06 00:00 [received] PHST- 2013/06/03 00:00 [revised] PHST- 2013/07/18 00:00 [accepted] PHST- 2013/08/22 06:00 [entrez] PHST- 2013/08/22 06:00 [pubmed] PHST- 2014/01/30 06:00 [medline] PHST- 2013/08/21 00:00 [pmc-release] AID - 10.3748/wjg.v19.i31.5131 [doi] PST - ppublish SO - World J Gastroenterol. 2013 Aug 21;19(31):5131-7. doi: 10.3748/wjg.v19.i31.5131. PMID- 23678158 OWN - NLM STAT- MEDLINE DCOM- 20140207 LR - 20260518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 40 IP - 7 DP - 2013 Jul TI - Systemic sclerosis in Canada's North American Native population: assessment of clinical and serological manifestations. PG - 1121-6 LID - 10.3899/jrheum.121212 [doi] AB - OBJECTIVE: Certain North American Native (NAN) populations are known to have higher rates of systemic sclerosis (SSc) compared to non-NAN; however, little is known of the specific disease characteristics in this population in Canada. This study compares the clinical and serological manifestations of SSc in NAN and white patients. METHODS: This cross-sectional, multicenter study included subjects enrolled in the Canadian Scleroderma Research Group registry between September 2004 and June 2012. Subjects were evaluated with complete medical histories, physical examinations, and self-questionnaires. Ethnicity was defined by self-report. Disease characteristics were compared between NAN and white patients and multivariate analyses were performed to determine the independent association between ethnicity and various clinical manifestations. RESULTS: Of 1278 patients, 1038 (81%) were white, 71 (6%) were NAN, and 169 (13%) were classified as non-white/non-NAN. There were important differences between NAN and white subjects with SSc. In multivariate analysis adjusting for socioeconomic differences and smoking status, NAN ethnicity was an independent risk factor for the severity of Raynaud phenomenon and more gastrointestinal symptoms, and was associated with a nonsignificant increase in the presence of digital ulcers. CONCLUSION: NAN patients with SSc have a distinct clinical phenotype. Our study provides a strong rationale to pursue further research into genetic and environmental determinants of SSc. FAU - Bacher, Adrienne AU - Bacher A AD - Department of Internal Medicine, University of Toronto, Toronto, Ontario, Canada. FAU - Mittoo, Shikha AU - Mittoo S FAU - Hudson, Marie AU - Hudson M FAU - Tatibouet, Solène AU - Tatibouet S CN - Canadian Scleroderma Research Group FAU - Baron, Murray AU - Baron M LA - eng GR - FRN 83518/Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130515 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM CIN - J Rheumatol. 2013 Jul;40(7):1031-3. doi: 10.3899/jrheum.130561. PMID: 23818720 MH - Adult MH - Aged MH - Canada/ethnology MH - Female MH - Humans MH - Indians, North American MH - Male MH - Middle Aged MH - Registries MH - Risk Factors MH - Scleroderma, Systemic/blood/diagnosis/*ethnology MH - Severity of Illness Index MH - Surveys and Questionnaires MH - White People OTO - NOTNLM OT - DISEASE SEVERITY OT - NORTH AMERICAN NATIVE OT - SCLERODERMA OT - SYSTEMIC SCLEROSIS FIR - Pope, J IR - Pope J FIR - Markland, J IR - Markland J FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - Khalidi, N IR - Khalidi N FIR - Docherty, P IR - Docherty P FIR - Kaminska, E IR - Kaminska E FIR - Masetto, A IR - Masetto A FIR - Sutton, E IR - Sutton E FIR - Mathieu, J-P IR - Mathieu JP FIR - Ligier, S IR - Ligier S FIR - Grodzicky, T IR - Grodzicky T FIR - Leclercq, S IR - Leclercq S FIR - Thorne, C IR - Thorne C FIR - Gyger, G IR - Gyger G FIR - Smith, D IR - Smith D FIR - Fritzler, M IR - Fritzler M EDAT- 2013/05/17 06:00 MHDA- 2014/02/08 06:00 CRDT- 2013/05/17 06:00 PHST- 2013/05/17 06:00 [entrez] PHST- 2013/05/17 06:00 [pubmed] PHST- 2014/02/08 06:00 [medline] AID - jrheum.121212 [pii] AID - 10.3899/jrheum.121212 [doi] PST - ppublish SO - J Rheumatol. 2013 Jul;40(7):1121-6. doi: 10.3899/jrheum.121212. Epub 2013 May 15. PMID- 41923962 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260402 LR - 20260402 IS - 2690-442X (Electronic) IS - 2690-442X (Linking) VI - 6 IP - 2 DP - 2026 Mar TI - Unexpected systemic sclerosis in a patient with atopic dermatitis receiving dupilumab: a novel case report. PG - 160-163 LID - 10.1093/skinhd/vzaf100 [doi] AB - Dupilumab, an interleukin (IL)-4 receptor alpha antagonist, is widely used in the treatment of atopic dermatitis and has shown potential benefit in certain fibrosing skin conditions. While blockade of IL-4 and IL-13 is generally considered to inhibit fibrosis, emerging reports of localized sclerosing dermatoses paradoxically arising during dupilumab therapy suggest a more complex immunological effect. We report a 37-year-old woman with the first case of systemic sclerosis developing in a patient with atopic dermatitis treated with dupilumab. After 2 years of treatment, she presented with new-onset dyspnoea, Raynaud phenomenon and digital puffiness. Serological tests revealed positive antinuclear and anticentromere antibodies; thoracic computed tomography showed interstitial lung disease. The modified Rodnan skin score was 4, and a skin biopsy from the fingertip demonstrated compact hyperkeratosis, irregular acanthosis and fibrosis extending to the superficial dermis. The patient met the American College of Rheumatology/European Alliance of Associations for Rheumatology 2013 classification criteria for systemic sclerosis, with a total score of 10. Dupilumab was discontinued, and systemic treatments targeting vascular symptoms and pulmonary involvement were initiated, resulting in clinical improvement. This case highlights a significant potential paradoxical reaction associated with cytokine-targeted biologic therapy. It underscores the importance of monitoring for fibrotic and systemic symptoms even when using agents presumed to exert antifibrotic effects. CI - © The Author(s) 2026. Published by Oxford University Press on behalf of British Association of Dermatologists. FAU - Gazal, Ela AU - Gazal E AUID- ORCID: 0000-0002-6368-4804 AD - Department of Dermatology, Mersin University Hospital, Mersin, Türkiye. FAU - Başak, Saffet Burak AU - Başak SB AD - Department of Dermatology, Mersin University Hospital, Mersin, Türkiye. FAU - Yuyucu Karabulut, Yasemin AU - Yuyucu Karabulut Y AD - Department of Pathology, Mersin University Hospital, Mersin, Türkiye. FAU - Türsen, Ümit AU - Türsen Ü AD - Department of Dermatology, Mersin University Hospital, Mersin, Türkiye. LA - eng PT - Case Reports PT - Journal Article DEP - 20260109 PL - England TA - Skin Health Dis JT - Skin health and disease JID - 9918227353706676 PMC - PMC13036728 COIS- The authors declare no conflicts of interest. EDAT- 2026/04/02 06:30 MHDA- 2026/04/02 06:31 PMCR- 2026/01/09 CRDT- 2026/04/02 04:37 PHST- 2025/10/28 00:00 [accepted] PHST- 2026/04/02 06:31 [medline] PHST- 2026/04/02 06:30 [pubmed] PHST- 2026/04/02 04:37 [entrez] PHST- 2026/01/09 00:00 [pmc-release] AID - vzaf100 [pii] AID - 10.1093/skinhd/vzaf100 [doi] PST - epublish SO - Skin Health Dis. 2026 Jan 9;6(2):160-163. doi: 10.1093/skinhd/vzaf100. eCollection 2026 Mar. PMID- 34063287 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210615 IS - 2075-4426 (Print) IS - 2075-4426 (Electronic) IS - 2075-4426 (Linking) VI - 11 IP - 5 DP - 2021 May 2 TI - S100A6, Calumenin and Cytohesin 2 as Biomarkers for Cutaneous Involvement in Systemic Sclerosis Patients: A Case Control Study. LID - 10.3390/jpm11050368 [doi] LID - 368 AB - BACKGROUND: Systemic sclerosis (Ssc) is an autoimmune disease with incomplete known physiopathology. There is a high number of candidate proteomic biomarkers for Ssc that have not yet been confirmed on independent Ssc cohorts. The aim of the study was to confirm circulating S100A6, calumenin, and cytohesin 2 as biomarkers for Ssc. METHODS: 53 Ssc patients and 26 age- and gender-matched controls were included. Serum S100A6, calumenin, and cytohesin 2 were evaluated with commercial ELISA kits. Associations between serum expression and clinical Ssc characteristics were evaluated. RESULTS: Serum calumenin, S100A6, and cytohesin 2 were higher in Ssc patients compared to controls. Calumenin associated with extensive cutaneous fibrosis, frequency of Raynaud phenomenon, and low complement level, and had a tendency to be higher in Ssc patients with pulmonary fibrosis. S100A6 correlated with the number of active digital ulcers. Serum cytohesin 2 levels were higher in patients with teleangiectasia and associated with pulmonary artery pressure. CONCLUSIONS: Serum calumenin, S100A6, and cytohesin 2 were confirmed as biomarkers on an independent group of Ssc patients. Calumenin had the best predictive capacity for cutaneous Ssc manifestations. Future studies are needed to evaluate the prognostic value of these biomarkers and evaluate them as possible therapeutic targets. FAU - Balanescu, Paul AU - Balanescu P AD - Internal Medicine Chair, University of Medicine and Pharmacy Carol Davila, 020021 Bucharest, Romania. AD - Clinical Immunology Laboratory CDPC, Colentina Clinical Hospital, 020125 Bucharest, Romania. AD - Clinical Research Unit RECIF (Reseau d'Epidemiologie Clinique International Francophone), 020125 Bucharest, Romania. FAU - Balanescu, Eugenia AU - Balanescu E AD - Clinical Immunology Laboratory CDPC, Colentina Clinical Hospital, 020125 Bucharest, Romania. FAU - Baicus, Cristian AU - Baicus C AD - Internal Medicine Chair, University of Medicine and Pharmacy Carol Davila, 020021 Bucharest, Romania. AD - Clinical Immunology Laboratory CDPC, Colentina Clinical Hospital, 020125 Bucharest, Romania. AD - Clinical Research Unit RECIF (Reseau d'Epidemiologie Clinique International Francophone), 020125 Bucharest, Romania. FAU - Balanescu, Anca AU - Balanescu A AD - Pediatrics Chair, University of Medicine and Pharmacy Carol Davila, 020021 Bucharest, Romania. LA - eng GR - PN-III-P1-1.1-PD-2019-0118/Romanian Ministry of Education and Research, CNCS - UEFISCDI/ PT - Journal Article DEP - 20210502 PL - Switzerland TA - J Pers Med JT - Journal of personalized medicine JID - 101602269 PMC - PMC8147492 OTO - NOTNLM OT - S100A6 OT - biomarker OT - calumenin OT - cytohesin 2 OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2021/06/03 06:00 MHDA- 2021/06/03 06:01 PMCR- 2021/05/02 CRDT- 2021/06/02 01:06 PHST- 2021/04/11 00:00 [received] PHST- 2021/04/29 00:00 [revised] PHST- 2021/04/30 00:00 [accepted] PHST- 2021/06/02 01:06 [entrez] PHST- 2021/06/03 06:00 [pubmed] PHST- 2021/06/03 06:01 [medline] PHST- 2021/05/02 00:00 [pmc-release] AID - jpm11050368 [pii] AID - jpm-11-00368 [pii] AID - 10.3390/jpm11050368 [doi] PST - epublish SO - J Pers Med. 2021 May 2;11(5):368. doi: 10.3390/jpm11050368. PMID- 33750226 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210726 IS - 1527-1315 (Electronic) IS - 0033-8419 (Linking) VI - 299 IP - 1 DP - 2021 Apr TI - Case 292. PG - 234-236 LID - 10.1148/radiol.2021201712 [doi] AB - History A 24-year-old right-handed woman presented to a neuro-ophthalmology clinic in Massachusetts in the summer with acute binocular diplopia when looking down and to the left, which started about 1 month earlier. Her medical history was notable for Raynaud syndrome, recurrent streptococcal pharyngitis, and an allergy to amoxicillin. Three days prior to developing diplopia, she presented to an outside emergency department due to fever, chills, and back pain. She received ciprofloxacin for presumed urinary tract infection based on urinalysis, which demonstrated few bacteria and was negative for leukocyte esterase, nitrites, and white blood cells. She then presented again to an outside emergency department for diplopia evaluation. Initial MRI and MR angiography of the brain at that time did not demonstrate any relevant findings, and the patient was referred to our department for neuro-ophthalmic evaluation, where she was seen 4 weeks later. Neuro-ophthalmic examination revealed 20/20 visual acuity in both eyes, and a right hypertropia in left gaze, downgaze and right head tilt, with right eye excyclotorsion. There were no ocular signs of myasthenia gravis or thyroid eye disease, nor did the patient report ocular or systemic symptoms. She denied recent travel. High-spatial-resolution MRI of the brain and orbit were performed (Figs 1, 2). FAU - Douglas, Vivian Paraskevi AU - Douglas VP AUID- ORCID: 0000-0002-5300-5468 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Douglas, Konstantinos A A AU - Douglas KAA AUID- ORCID: 0000-0003-2972-2691 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Reinshagen, Katherine L AU - Reinshagen KL AUID- ORCID: 0000-0003-3705-1082 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Chwalisz, Bart K AU - Chwalisz BK AUID- ORCID: 0000-0001-9918-1742 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). LA - eng PT - Journal Article PL - United States TA - Radiology JT - Radiology JID - 0401260 SB - IM EDAT- 2021/03/23 06:00 MHDA- 2021/03/23 06:01 CRDT- 2021/03/22 17:49 PHST- 2021/03/22 17:49 [entrez] PHST- 2021/03/23 06:00 [pubmed] PHST- 2021/03/23 06:01 [medline] AID - 10.1148/radiol.2021201712 [doi] PST - ppublish SO - Radiology. 2021 Apr;299(1):234-236. doi: 10.1148/radiol.2021201712. PMID- 39035301 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240723 IS - 2213-8862 (Electronic) IS - 1991-7902 (Print) IS - 1991-7902 (Linking) VI - 19 IP - 3 DP - 2024 Jul TI - A scientometric and comparative study of Sjogren's syndrome research by rheumatologists and stomatologists. PG - 1499-1505 LID - 10.1016/j.jds.2024.01.020 [doi] AB - BACKGROUND/PURPOSE: The diagnosis and treatment of Sjogren's syndrome (SS) are commonly conducted by rheumatologists and stomatologists. The purpose of this study was to compare the scientometric characteristics of SS publications by rheumatologists and stomatologists. MATERIALS AND METHODS: All the papers on cheilitis were comprehensively retrieved from the Scopus database, and divided into rheumatologists and stomatologists groups. RESULTS: There were 3245 and 1209 papers on SS were published by rheumatologists and stomatologists, respectively. For the most-cited top-200 papers, the total citation count was 29,764 and the h index was 108 for SS publications by rheumatologists; whereas the count is 19,891 and h index is 81 for publications by stomatologists. Interestingly, we observed that accumulated citations of the publications by stomatologists cooperated with rheumatologists were larger than those by stomatologists alone during 2012-2022. The more common keywords such as saliva, salivation, minor salivary glands, parotid gland, submandibular gland, sialography, lip, dental caries, and hyposalivation were reported by stomatologists. The more frequent keywords such as rheumatoid factor, fatigue, lymphoma, interstitial lung disease, arthralgia, Raynaud phenomenon, lymphadenopathy, and vasculitis were reported by rheumatologists. CONCLUSION: This study firstly reports the scientometric characteristics of SS publications by rheumatologists and stomatologists. The scale and citations of rheumatologists' publications greatly outweigh those of stomatologists, suggesting stomatologists can cooperate more with rheumatologists regarding SS research. CI - © 2024 Association for Dental Sciences of the Republic of China. Publishing services by Elsevier B.V. FAU - Zhang, Zuohao AU - Zhang Z AD - Department of Periodontology and Oral Mucosa, Changsha Stomatological Hospital, School of Dental Medicine, Hunan University of Chinese Medicine, Changsha, Hunan, China. FAU - Zhu, Hanyi AU - Zhu H AD - College of Stomatology, Shanghai Jiao Tong University, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai, China. AD - Department of Oral Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Ren, Zhenhu AU - Ren Z AD - College of Stomatology, Shanghai Jiao Tong University, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai, China. AD - Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Shi, Huan AU - Shi H AD - College of Stomatology, Shanghai Jiao Tong University, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai, China. AD - Department of Oral Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. FAU - Liu, Wei AU - Liu W AD - College of Stomatology, Shanghai Jiao Tong University, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai, China. AD - Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. LA - eng PT - Journal Article DEP - 20240207 PL - Netherlands TA - J Dent Sci JT - Journal of dental sciences JID - 101293181 PMC - PMC11259611 OTO - NOTNLM OT - Bibliometric OT - Oral complications OT - Rheumatology OT - Sjögren syndrome OT - Stomatology OT - Systemic comorbidities COIS- The authors have no conflicts of interest relevant to this article. EDAT- 2024/07/22 06:42 MHDA- 2024/07/22 06:43 PMCR- 2024/02/07 CRDT- 2024/07/22 05:19 PHST- 2023/12/18 00:00 [received] PHST- 2024/01/26 00:00 [revised] PHST- 2024/07/22 06:43 [medline] PHST- 2024/07/22 06:42 [pubmed] PHST- 2024/07/22 05:19 [entrez] PHST- 2024/02/07 00:00 [pmc-release] AID - S1991-7902(24)00033-3 [pii] AID - 10.1016/j.jds.2024.01.020 [doi] PST - ppublish SO - J Dent Sci. 2024 Jul;19(3):1499-1505. doi: 10.1016/j.jds.2024.01.020. Epub 2024 Feb 7. PMID- 21951026 OWN - NLM STAT- MEDLINE DCOM- 20111209 LR - 20110928 IS - 1089-5159 (Print) IS - 1089-5159 (Linking) VI - 16 IP - 3 DP - 2011 Sep TI - A case report of a 53-year-old female with rheumatoid arthritis and osteoporosis: focus on lab testing and CAM therapies. PG - 250-62 AB - A 53-year-old female presented with rheumatoid arthritis and osteoporosis. Additional conditions and symptoms included Raynaud syndrome, fatigue, irritable bowel syndrome associated constipation (IBS-C), gastroesophageal reflux (GERD), menopausal symptoms, chronic urinary tract and upper respiratory infections, and weight gain. She was taking Arthrotec (a combination of diclofenac and misoprostol - for pain and inflammation), Fosamax Plus D (alendronate with vitamin D3 - recently prescribed because of low bone density), and Catapres (clonidine - for menopausal symptoms). Against the advice of her rheumatologist, she had recently discontinued taking Plaquenil (hydroxychloroquine), methotrexate, and prednisone due to significant side effects. Lab tests to identify underlying imbalances and to direct treatment were ordered. Treatment included dietary, nutritional, hormonal, and mind/body support. After one year of therapy, the patient experienced improvement with all of her presenting conditions and symptoms, which enabled her to discontinue several medications. She became versed in identifying and avoiding the environmental triggers of her disease, including foods (dairy, wheat, eggs, and soy), molds, and emotional stress. Antinuclear antibodies were normalized. She experienced a 7.5-percent improvement in left trochanteric bone density - comparable to bisphosphonate therapy. Mild improvements were also noted in the spine and bilateral femoral neck. FAU - Fitzgerald, Kara AU - Fitzgerald K AD - Institute for Functional Medicine, USA. kf@drkarafizgerald.com LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Altern Med Rev JT - Alternative medicine review : a journal of clinical therapeutic JID - 9705340 MH - Arthritis, Rheumatoid/blood/complications/*drug therapy MH - Bone Density/drug effects MH - Comorbidity MH - Complementary Therapies/*methods MH - *Dietary Supplements MH - Female MH - Humans MH - Middle Aged MH - Osteoporosis/blood/complications/*drug therapy MH - Treatment Outcome EDAT- 2011/09/29 06:00 MHDA- 2011/12/14 06:00 CRDT- 2011/09/29 06:00 PHST- 2011/09/29 06:00 [entrez] PHST- 2011/09/29 06:00 [pubmed] PHST- 2011/12/14 06:00 [medline] PST - ppublish SO - Altern Med Rev. 2011 Sep;16(3):250-62. PMID- 17669102 OWN - NLM STAT- MEDLINE DCOM- 20071213 LR - 20220331 IS - 1083-4389 (Print) IS - 1083-4389 (Linking) VI - 12 IP - 4 DP - 2007 Aug TI - Reversal of migraine symptoms by Helicobacter pylori eradication therapy in patients with hepatitis-B-related liver cirrhosis. PG - 306-8 AB - Helicobacter pylori infection might be associated with vascular diseases, such as primary Raynaud phenomenon and coronary heart diseases. The possible mechanism might be due to H. pylori antigens causing intermittent vasospasm of arterioles, which also played roles in the development of liver cirrhosis. Migraine, a functional vascular disease, was observed in many patients with cirrhosis in the clinic. This study aimed to assess the effects of H. pylori eradication on migraine symptoms in patients with hepatitis-B-virus-related cirrhosis. The results clearly showed that the intensity, duration, and frequency of attacks of migraine were significantly reduced in all the patients in whom H. pylori has been eradicated. Thus, the study pushed further insight into the mechanisms of migraine pathogenesis. FAU - Hong, Liu AU - Hong L AD - State Key Laboratory of Cancer Biology and Institute of Digestive Diseases, Xijing Hospital, Fourth Military Medical University, Xi'an 710032, Shaanxi Province, China. FAU - Zhao, Yunping AU - Zhao Y FAU - Han, Ying AU - Han Y FAU - Guo, Wei AU - Guo W FAU - Wang, Jun AU - Wang J FAU - Li, Xiaohua AU - Li X FAU - Han, Yu AU - Han Y FAU - Fan, Daiming AU - Fan D LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Helicobacter JT - Helicobacter JID - 9605411 RN - 804826J2HU (Amoxicillin) RN - H1250JIK0A (Clarithromycin) RN - KG60484QX9 (Omeprazole) SB - IM MH - Adult MH - Amoxicillin/therapeutic use MH - Clarithromycin/therapeutic use MH - Drug Therapy, Combination MH - Female MH - Follow-Up Studies MH - Helicobacter Infections/complications/*drug therapy MH - Helicobacter pylori/*drug effects MH - Hepatitis B/*complications MH - Humans MH - Liver Cirrhosis/*complications MH - Male MH - Middle Aged MH - Migraine Disorders/drug therapy/etiology/pathology MH - Omeprazole/therapeutic use MH - Time Factors MH - Treatment Outcome EDAT- 2007/08/03 09:00 MHDA- 2007/12/14 09:00 CRDT- 2007/08/03 09:00 PHST- 2007/08/03 09:00 [pubmed] PHST- 2007/12/14 09:00 [medline] PHST- 2007/08/03 09:00 [entrez] AID - HEL512 [pii] AID - 10.1111/j.1523-5378.2007.00512.x [doi] PST - ppublish SO - Helicobacter. 2007 Aug;12(4):306-8. doi: 10.1111/j.1523-5378.2007.00512.x. PMID- 33613703 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220420 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 13 DP - 2021 TI - Hospital admissions in inflammatory rheumatic diseases during the peak of COVID-19 pandemic: incidence and role of disease-modifying agents. PG - 1759720X20962692 LID - 10.1177/1759720X20962692 [doi] LID - 1759720X20962692 AB - AIMS: In this pandemic, it is essential for rheumatologists and patients to know the relationship between COVID-19 and inflammatory rheumatic diseases (IRDs). We wanted to assess the role of targeted synthetic or biologic disease-modifying antirheumatic drugs (ts/bDMARDs) and other variables in the development of moderate-severe COVID-19 disease in IRD. METHODS: An observational longitudinal study was conducted during the epidemic peak in Madrid (1 March to 15 April 2020). All patients attended at the rheumatology outpatient clinic of a tertiary hospital in Madrid with a medical diagnosis of IRD were included. Main outcome: hospital admission related to COVID-19. Independent variable: ts/bDMARDs. Covariates: sociodemographic, comorbidities, type of IRD diagnosis, glucocorticoids, non-steroidal anti-inflammatory drugs (NSAIDs), and conventional synthetic disease-modifying antirheumatic drugs (csDMARDs). Incidence rate (IR) of hospital admission related to COVID-19 was expressed per 1000 patient-months. Cox multiple regression analysis was run to examine the influence of ts/bDMARDs and other covariates on IR of hospital admission related to COVID-19. RESULTS: A total of 3951 IRD patients were included (5896 patient-months). Methotrexate was the csDMARD most used. Eight hundred and two patients were on ts/bDMARDs, mainly anti-TNF agents, and Rtx. Hospital admissions related to COVID-19 occurred in 54 patients (1.36%) with an IR of 9.15 (95% confidence interval: 7-11.9). In the multivariate analysis, older, male, comorbidities, and specific systemic autoimmune conditions (Sjögren, polychondritis, Raynaud, and mixed connective tissue disease) had more risk of hospital admissions. Exposition to ts/bDMARDs did not achieve statistical significance. Use of glucocorticoids, NSAIDs, and csDMARDs dropped from the final model. CONCLUSION: This study provides additional evidence in IRD patients regarding susceptibility to moderate-severe infection related to COVID-19. CI - © The Author(s), 2021. FAU - Fernandez-Gutierrez, Benjamin AU - Fernandez-Gutierrez B AUID- ORCID: 0000-0002-6126-8786 AD - Rheumatology Department, Hospital Clínico San Carlos, Madrid, Spain. FAU - Leon, Leticia AU - Leon L AUID- ORCID: 0000-0001-7142-0545 AD - Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (IDISSC), Hospital Clínico San Carlos, Martin Lagos s/n, Madrid, 28040, Spain Universidad Camilo Jose Cela, Madrid, Spain. FAU - Madrid, Alfredo AU - Madrid A AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Rodriguez-Rodriguez, Luis AU - Rodriguez-Rodriguez L AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Freites, Dalifer AU - Freites D AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Font, Judit AU - Font J AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Mucientes, Arkaitz AU - Mucientes A AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Culebras, Esther AU - Culebras E AD - Microbiology Department, Hospital Clínico San Carlos, Madrid, Spain. FAU - Colome, Jose Ignacio AU - Colome JI AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. FAU - Jover, Juan Angel AU - Jover JA AD - Rheumatology Department, Hospital Clínico San Carlos, Madrid, and Medicine Department, Universidad Complutense, Madrid, Spain. FAU - Abasolo, Lydia AU - Abasolo L AD - Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain. LA - eng PT - Journal Article DEP - 20210204 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC7869066 OTO - NOTNLM OT - COVID-19 OT - autoimmune diseases OT - disease-modifying antirheumatic drugs OT - rheumatic diseases COIS- Conflict of interest statement: The authors declare that there is no conflict of interest. EDAT- 2021/02/23 06:00 MHDA- 2021/02/23 06:01 PMCR- 2021/02/04 CRDT- 2021/02/22 05:54 PHST- 2020/07/03 00:00 [received] PHST- 2020/09/01 00:00 [accepted] PHST- 2021/02/22 05:54 [entrez] PHST- 2021/02/23 06:00 [pubmed] PHST- 2021/02/23 06:01 [medline] PHST- 2021/02/04 00:00 [pmc-release] AID - 10.1177_1759720X20962692 [pii] AID - 10.1177/1759720X20962692 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2021 Feb 4;13:1759720X20962692. doi: 10.1177/1759720X20962692. eCollection 2021. PMID- 30572454 OWN - NLM STAT- MEDLINE DCOM- 20190107 LR - 20220331 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 97 IP - 51 DP - 2018 Dec TI - Progression of immunoglobulin G4-related disease to systematic lupus erythematosus after gastric cancer surgery: A case report. PG - e13545 LID - 10.1097/MD.0000000000013545 [doi] LID - e13545 AB - RATIONALE: Immunoglobulin G4 related disease (IgG4-RD) rarely coexists with other autoimmune diseases, though we had a patient whose primary clinical problem was shifted from IgG4-RD to systemic lupus erythematosus (SLE) after gastrectomy. The present paper aimed to report pathological findings and clinical course of the patient. PATIENT CONCERNS: The patient was a male aged 74 years old with gastric cancer characterized by the following symptoms: Raynaud phenomenon, polyarthralgia, and swollen parotid glands on both sides. Before gastrectomy, laboratory examination results showed renal dysfunction, hypocomplementemia, antinuclear antibodies (ANAs) positivity, and elevated serum IgG and IgG4 levels. DIAGNOSIS: Based on postoperative renal biopsy showing severe plasma cell infiltration with tubulointerstitial fibrosclerosis, the patient was diagnosed with IgG4-RD. Despite significant improvement in renal function and reduction in parotid gland swelling during the postoperative follow-up period, after 7 months of the gastrectomy, anti-DNA antibody levels were increased and serositis was detected, which indicated the onset of SLE. IgG4-type ANA were also detected in the sera of the patient. INTERVENTIONS: Treatment by oral prednisolone at 30 mg/day was initiated. OUTCOMES: Pericardial fluid, pleural effusions, and thickening of the gallbladder wall improved after 3 months of treatment according to computed tomography. LESSONS: This study presented a rare case of comorbidity, wherein the patient's primary problem progressed from IgG4-type ANA-positive IgG4-RD to SLE after excision of gastric cancer. FAU - Arai, Haruna AU - Arai H AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Hayashi, Hiroki AU - Hayashi H AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Ogata, Soshiro AU - Ogata S AD - Faculty of Nursing, School of Healthcare, Fujita Health University, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi. FAU - Uto, Kenichi AU - Uto K AD - Department of Clinical Laboratory, Kobe University Hospital. FAU - Saegusa, Jun AU - Saegusa J AD - Department of Rheumatology and Clinical Immunology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Japan. FAU - Takahashi, Kazuo AU - Takahashi K AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Koide, Shigehisa AU - Koide S AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Inaguma, Daijyo AU - Inaguma D AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Hasegawa, Midori AU - Hasegawa M AD - Department of Nephrology, Fujita Health University School of Medicine. FAU - Yuzawa, Yukio AU - Yuzawa Y AD - Department of Nephrology, Fujita Health University School of Medicine. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Adenocarcinoma/*complications/*surgery MH - Aged MH - Comorbidity MH - Disease Progression MH - Humans MH - Immunoglobulin G4-Related Disease/*complications/diagnosis/pathology/physiopathology MH - Lupus Erythematosus, Systemic/*complications/diagnosis/pathology/physiopathology MH - Male MH - Postoperative Period MH - Stomach Neoplasms/*complications/*surgery PMC - PMC6320217 COIS- There are no financial interests and there are no conflict of interest to declare. EDAT- 2018/12/24 06:00 MHDA- 2019/01/08 06:00 PMCR- 2018/12/21 CRDT- 2018/12/22 06:00 PHST- 2018/12/22 06:00 [entrez] PHST- 2018/12/24 06:00 [pubmed] PHST- 2019/01/08 06:00 [medline] PHST- 2018/12/21 00:00 [pmc-release] AID - 00005792-201812210-00033 [pii] AID - MD-D-18-04988 [pii] AID - 10.1097/MD.0000000000013545 [doi] PST - ppublish SO - Medicine (Baltimore). 2018 Dec;97(51):e13545. doi: 10.1097/MD.0000000000013545. PMID- 19105419 OWN - NLM STAT- MEDLINE DCOM- 20090206 LR - 20081224 IS - 0040-3660 (Print) IS - 0040-3660 (Linking) VI - 80 IP - 10 DP - 2008 TI - [Endothelial dysfunction in scleroderma systematica: clinicopathogenetic correlations]. PG - 68-72 AB - AIM: To evaluate disturbances of vascular tonicity regulation in scleroderma systematica (SS) patients using laser Doppler flowmetry (LDF) with calculation of amplitude-frequency blood flow fluctuations (variability) basing on the mathematical model wavelet-transformation. MATERIAL AND METHODS: The trial included 101 patients with verified SS aged 21-60 years (mean 52.3 years, 97 females). In addition, 15 and 10 patients with primary and secondary Raynaud syndrome diagnosed by ACR criteria, respectively, entered the trial. The control group consisted of 20 healthy subjects. Cardiovascular patients were not included in the study. Microcirculation was investigated with laser analyzer of capillary circulation in the tip of left hand finger III and external surface of the left arm. Endothelial motor function was studied in the tests with reactive hyperemia and nitroglycerin. RESULTS: SS patients were found to have a significantly higher variability and endothelial rhythm amplitude index in arm skin, reduced microcirculation, the variation index and high neurogenic tonicity in the hand finger tip. A direct correlation was found between severity and area of skin lesion (of the arm, in particular) and their amplitude of endothelial fluctuations (r = 0.38, p = 0.03) of circulation in the arm skin. Persistent correlations between skin count and LDF values were not registered. There was a significant regression of flow-dependent dilation of the brachial artery in SS patients independent of classic cardiovascular risk factors. CONCLUSION: SS patients have defects in regulation of vascular tonicity both in microcirculation and middle-caliber arteries. Vasomotor activity of the middle-caliber vessels is low in the absence of conventional cardiovascular disease risk factors. LDF detects changes in local regulation in cardiovascular patients confirming the role of neuroendothelial disorders in its pathogenesis. The trend to systemic DE in cardiovascular patients is associated with both disease-mediated vascular lesions (ulcers of finger tips, pulmonary hypertension) and cardiovascular disease markers. FAU - Volkov, A V AU - Volkov AV FAU - Mach, E S AU - Mach ES FAU - Guseva, N G AU - Guseva NG LA - rus PT - English Abstract PT - Journal Article PL - Russia (Federation) TA - Ter Arkh JT - Terapevticheskii arkhiv JID - 2984818R SB - IM MH - Adult MH - Cardiovascular Diseases/diagnosis/epidemiology MH - Endothelium/*physiopathology MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/epidemiology MH - Laser-Doppler Flowmetry MH - Male MH - Microcirculation/physiology MH - Middle Aged MH - Risk Factors MH - Scleroderma, Systemic/diagnosis/epidemiology/*physiopathology EDAT- 2008/12/25 09:00 MHDA- 2009/02/07 09:00 CRDT- 2008/12/25 09:00 PHST- 2008/12/25 09:00 [entrez] PHST- 2008/12/25 09:00 [pubmed] PHST- 2009/02/07 09:00 [medline] PST - ppublish SO - Ter Arkh. 2008;80(10):68-72. PMID- 21050538 OWN - NLM STAT- MEDLINE DCOM- 20101221 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 28 IP - 5 Suppl 62 DP - 2010 Sep-Oct TI - Increased alveolar nitric oxide in early systemic sclerosis. PG - S5-9 AB - OBJECTIVES: Assessment of inflammatory activity in interstitial lung disease of systemic sclerosis (SSc) is difficult. Nitric oxide (NO) has gained attention in the pathogenesis of SSc. The aim of the study was to investigate alveolar NO concentration (CA(NO)) in SSc patients with short disease duration and to relate CA(NO) to radiologic findings. METHODS: In a prospective study, 34 consecutive patients with disease duration of less than 2 years from onset of first non-Raynaud symptom and 26 healthy controls were enrolled. Exhaled NO was measured and CA(NO) was calculated. CA(NO) levels were related to the radiologic extent of pulmonary fibrosis measured as the extent of traction bronchiectasis within areas of ground glass opacities and reticulations. RESULTS: CA(NO) levels were increased in patients with early SSc compared to healthy controls (3.52 (2.94-4.09) versus 2.08 (1.6-2.6); p<0.001). Both SSc patients with SSc-ILD (3.56 (3.04-4.73), p<0.001) and SSc patients without SSc-ILD (2.98 (2.68-3.98), p<0.01) had higher CA(NO) levels compared with healthy controls (2.08 (1.6-2.6)). CA(NO) levels did not differ between SSc patients without SSc-ILD and SSc patients with SSC-ILD. CA(NO) levels did not correlate to the extent of pulmonary fibrosis but were associated with the extent of ground glass opacities (rs=0.37, p<0.05) and reticulations (rs=0.37, p<0.05) on HRCT. CA(NO) levels were not correlated to lung function tests. CONCLUSIONS: In patients with early SSc, alveolar NO is increased and may precede radiological changes of SSc-ILD. CA(NO) may therefore be a marker of early lung involvement. FAU - Wuttge, D M AU - Wuttge DM AD - Department of Clinical Sciences, Lund, Sweden. dirk.wuttge@med.lu.se FAU - Bozovic, G AU - Bozovic G FAU - Hesselstrand, R AU - Hesselstrand R FAU - Aronsson, D AU - Aronsson D FAU - Bjermer, L AU - Bjermer L FAU - Scheja, A AU - Scheja A FAU - Tufvesson, E AU - Tufvesson E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20101103 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Biomarkers) RN - 31C4KY9ESH (Nitric Oxide) SB - IM MH - Aged MH - Biomarkers/metabolism MH - Breath Tests MH - Female MH - Humans MH - Male MH - Middle Aged MH - Nitric Oxide/*metabolism MH - Prospective Studies MH - Pulmonary Alveoli/*metabolism/pathology MH - Radiography, Thoracic MH - Respiratory Function Tests MH - Scleroderma, Systemic/diagnostic imaging/*metabolism/pathology MH - Skin/pathology MH - Time Factors MH - Tomography, X-Ray Computed EDAT- 2010/11/26 06:00 MHDA- 2010/12/22 06:00 CRDT- 2010/11/06 06:00 PHST- 2010/06/29 00:00 [received] PHST- 2010/07/21 00:00 [accepted] PHST- 2010/11/06 06:00 [entrez] PHST- 2010/11/26 06:00 [pubmed] PHST- 2010/12/22 06:00 [medline] AID - 4093 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2010 Sep-Oct;28(5 Suppl 62):S5-9. Epub 2010 Nov 3. PMID- 36479532 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240908 IS - 1016-1430 (Print) IS - 2251-6840 (Electronic) IS - 1016-1430 (Linking) VI - 36 DP - 2022 TI - Surgical Treatment Options for Buerger's Disease (Experience with 315 Cases in Iran). PG - 134 LID - 10.47176/mjiri.36.134 [doi] LID - 134 AB - Background: Buerger's disease (thromboangiitis obliterans) may be a rare peripheral vascular disease that sometimes affects young male smokers. This study presents surgical treatment options for 315 Buerger's patients during a period of 18 years from 2002 to 2020. Methods: In this cross-sectional study, 315 newly diagnosed Buerger patients in a period of 18 years (by Census sampling) were evaluated. Data included age, sex, cigarette smoking status, clinical presentation, the affected limb (right or left, upper or lower extremities), and the performed therapeutic procedures such as angiography of limb arteries, amputation, sympathectomy, and vascular bypass surgery, which were collected in a data sheet. Vascular reconstruction was done if there were suitable inflow and outflow arteries. Sympathectomy was performed for the patients who were unsuitable for revascularization. All analyzes were performed using SPSSV.18 software package (SPSS Inc., Chicago, IL). Data are presented as frequency, mean ± variance (SD). Results: The mean age of patients was 42.6±9 years old, ranging from (26-75). There were 309 (98.1%) males and 6 (1.9%) females. The most common symptom was ulcer 252 (80%), and the most commonly involved arteries were the dorsal pedis (N=231; 73.4%) and posterior tibialis (N=225; 71.5%). Vascular bypass surgery, sympathectomy, and amputation were performed for patients who met surgical indications. Aortofemoral (N=9) and femoropopliteal (N=24) bypass procedures were done in 2.8% and 7.6% of patients respectively. Of nine patients who underwent aorto-femoral bypass procedure, 6 cases presented with leg claudication, 3 with an ulcer, and 3 with the Raynaud phenomenon. The digital loss rate was 9.6% (N=9) in toes and 1% (N=3) in fingers. Conclusion: As most of the Buerger patients have multi arterial involvement, bypass surgery or sympathectomy can't help treat these patients more than cigarette smoking or pharmaceutical therapy. CI - © 2022 Iran University of Medical Sciences. FAU - Salimi, Javad AU - Salimi J AD - Department of Vascular Surgery, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran. FAU - Cheraghali, Roozbeh AU - Cheraghali R AD - Department of Vascular Surgery, Faculty of Medicine, Golestan University of Medical Sciences, Gorgan, Iran. FAU - Omrani, Zahra AU - Omrani Z AD - Department General Surgery, Faculty of Medicine, Iran University of Medical Sciences, Tehran, Iran. FAU - Farshidmehr, Pezhman AU - Farshidmehr P AD - Department of Vascular Surgery, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran. FAU - Afghani, Reza AU - Afghani R AD - Department Thoracic Surgery, Faculty of Medicine, Golestan University of Medical Sciences, Gorgan, Iran. LA - eng PT - Journal Article DEP - 20221114 PL - Iran TA - Med J Islam Repub Iran JT - Medical journal of the Islamic Republic of Iran JID - 8910777 PMC - PMC9719581 OTO - NOTNLM OT - Amputation OT - Surgery OT - Sympathectomy OT - Thromboangiitis Obliterans EDAT- 2022/12/09 06:00 MHDA- 2022/12/09 06:01 PMCR- 2022/11/14 CRDT- 2022/12/08 14:08 PHST- 2021/04/21 00:00 [received] PHST- 2022/12/08 14:08 [entrez] PHST- 2022/12/09 06:00 [pubmed] PHST- 2022/12/09 06:01 [medline] PHST- 2022/11/14 00:00 [pmc-release] AID - 10.47176/mjiri.36.134 [doi] PST - epublish SO - Med J Islam Repub Iran. 2022 Nov 14;36:134. doi: 10.47176/mjiri.36.134. eCollection 2022. PMID- 34631469 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20211012 IS - 2220-3230 (Print) IS - 2220-3230 (Electronic) IS - 2220-3230 (Linking) VI - 11 IP - 9 DP - 2021 Sep 18 TI - Journey of a patient with scleroderma from renal failure up to kidney transplantation. PG - 372-387 LID - 10.5500/wjt.v11.i9.372 [doi] AB - The increased awareness of systemic sclerosis (SS) and its pathogenetic background made the management of this disease more amenable than previously thought. However, scleroderma renal crisis (SRC) is a rarely seen as an associated disorder that may involve 2%-15% of SS patients. Patients presented with earlier, rapidly progressing, diffuse cutaneous SS disease, mostly in the first 3-5 years after non-Raynaud clinical manifestations, are more vulnerable to develop SRC. SRC comprises a collection of acute, mostly symptomatic rise in blood pressure, elevation in serum creatinine concentrations, oliguria and thrombotic microangiopathy in almost 50% of cases. The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis. In a scleroderma patient maintained on regular dialysis; every effort should be exerted to declare any possible evidence of renal recovery. A given period of almost two years has been suggested prior to proceeding in a kidney transplant (KTx). Of note, SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease (ESRD). KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients. Compared to other primary renal diseases, SS-related ESRD was considered for a long period of poor patient and allograft survivals. Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death. Recurrence of SRC after transplantation has been observed in some patients. However, an excellent post-transplant patient and graft outcome have been recently reported. Consequently, the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx. CI - ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved. FAU - Abbas, Fedaey AU - Abbas F AD - Department of Nephrology, Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom. FAU - El Kossi, Mohsen AU - El Kossi M AD - Doncaster Renal Unit, Doncaster Royal Infirmary, Doncaster DN2 5LT, United Kingdom. FAU - Shaheen, Ihab Sakr AU - Shaheen IS AD - Department of Paediatric Nephrology, St James's University Hospital, Glasgow G51 4TF, United Kingdom. FAU - Sharma, Ajay AU - Sharma A AD - Department of Transplant Surgery, Royal Liverpool University Hospital, Liverpool L7 8XP, United Kingdom. FAU - Halawa, Ahmed AU - Halawa A AD - Department of Transplant Surgery, Sheffield Teaching Hospital, Sheffield S5 7AU, United Kingdom. ahmed.halawa@sth.nhs.uk. LA - eng PT - Journal Article PT - Review PL - United States TA - World J Transplant JT - World journal of transplantation JID - 101608356 PMC - PMC8465513 OTO - NOTNLM OT - Hemodialysis OT - Kidney transplant OT - Renal failure OT - Risk factors OT - Scleroderma renal crisis OT - Systemic sclerosis COIS- Conflict-of-interest statement: Fedaey Abbas is an employee (under contract) of MOD, Kuwait. EDAT- 2021/10/12 06:00 MHDA- 2021/10/12 06:01 PMCR- 2021/09/18 CRDT- 2021/10/11 06:07 PHST- 2021/01/29 00:00 [received] PHST- 2021/04/10 00:00 [revised] PHST- 2021/08/19 00:00 [accepted] PHST- 2021/10/11 06:07 [entrez] PHST- 2021/10/12 06:00 [pubmed] PHST- 2021/10/12 06:01 [medline] PHST- 2021/09/18 00:00 [pmc-release] AID - 10.5500/wjt.v11.i9.372 [doi] PST - ppublish SO - World J Transplant. 2021 Sep 18;11(9):372-387. doi: 10.5500/wjt.v11.i9.372. PMID- 26728774 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20191113 IS - 2212-4055 (Electronic) IS - 1871-5281 (Linking) VI - 14 IP - 2 DP - 2015 TI - Imaging Patterns of Cardiovascular Involvement in Mixed Connective Tissue Disease Evaluated by Cardiovascular Magnetic Resonance. PG - 111-6 AB - BACKGROUND: To clarify the imaging patterns of cardiovascular lesions in patients with mixed connective tissue disease (MCTD) and cardiovascular symptoms with or/ without abnormal routine non-invasive evaluation. PATIENTS-METHODS: Twenty-two MCTD patients (19F/3M), aged 38±4 yrs with cardiovascular symptoms were evaluated using a 1.5 T scanner. Of them, 8/22 had systemic lupus erythematosus (SLE), 5/22 rheumatoid arthritis (RA), 5/22 scleroderma (SSc) and 4/22 myositis (MY) overlap syndromes; 10/22 patients with MCTD presented with Raynaud phenomenon (RP) and all were positive for Anti-RNP antibodies. The cardiovascular magnetic resonance study (CMR) included evaluation of function, inflammation and fibrosis. Myocardial stress perfusion-fibrosis evaluation was performed only in MCTD patients with RP. RESULTS: A positive CMR study was identified in 4/8 with SLE, 1/5 with RA, 4/5 with SSc and in 1/4 with MY like MCTD. The CMR lesions were subendocardial or transmural LGE following the distribution of coronary arteries, intramyocardial LGE and diffuse subendocardial LGE in SLE-RA, MY and SSc like MCTD, respectively. Although no evidence of fibrosis was identified in patients with RP, adenosine stress myocardial perfusion revealed diffuse subendocardial perfusion defects. No correlation between disease duration and/or inflammatory indices and cardiac lesions was identified. CONCLUSION: CMR can reveal myocardial lesions in MCTD patients with cardiac symptoms including myocardial infarction, inflammation, diffuse subendocardial fibrosis and diffuse perfusion defects, necessitating further cardiac investigation and/or treatment. FAU - Mavrogeni, Sophie AU - Mavrogeni S AD - Onassis Cardiac Surgery Center, 50 Esperou Street, 175 61 P. Faliro, Athens, Greece. soma13@otenet.gr. FAU - Sfikakis, Petros P AU - Sfikakis PP FAU - Dimitroulas, Theodoros AU - Dimitroulas T FAU - Koutsogeorgopoulou, Loukia AU - Koutsogeorgopoulou L FAU - Karabela, Georgia AU - Karabela G FAU - Katsifis, Gikas AU - Katsifis G FAU - Stavropoulos, Efthymios AU - Stavropoulos E FAU - Gialafos, Elias AU - Gialafos E FAU - Spiliotis, George AU - Spiliotis G FAU - Kolovou, Genovefa AU - Kolovou G FAU - Kitas, George D AU - Kitas GD LA - eng PT - Journal Article PL - United Arab Emirates TA - Inflamm Allergy Drug Targets JT - Inflammation & allergy drug targets JID - 101266886 SB - IM MH - Adult MH - Cardiomyopathies/*diagnostic imaging/pathology/physiopathology MH - *Coronary Circulation MH - Echocardiography MH - Electrocardiography MH - Female MH - Fibrosis MH - Heart/*diagnostic imaging MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Middle Aged MH - Mixed Connective Tissue Disease/*diagnostic imaging/pathology/physiopathology MH - Myocardial Perfusion Imaging/*methods MH - Myocardium/*pathology MH - Predictive Value of Tests MH - Prognosis EDAT- 2016/01/06 06:00 MHDA- 2016/12/15 06:00 CRDT- 2016/01/06 06:00 PHST- 2015/10/10 00:00 [received] PHST- 2015/12/13 00:00 [revised] PHST- 2015/12/17 00:00 [accepted] PHST- 2016/01/06 06:00 [entrez] PHST- 2016/01/06 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - IADT-EPUB-72902 [pii] AID - 10.2174/1871528114666160105112758 [doi] PST - ppublish SO - Inflamm Allergy Drug Targets. 2015;14(2):111-6. doi: 10.2174/1871528114666160105112758. PMID- 26102183 OWN - NLM STAT- MEDLINE DCOM- 20150901 LR - 20150624 IS - 1524-4725 (Electronic) IS - 1076-0512 (Linking) VI - 41 IP - 7 DP - 2015 Jul TI - In Vivo Reflectance Confocal Microscopy Combined With the "Spaghetti" Technique: A New Procedure for Defining Surgical Margins of Genital Paget Disease. PG - 862-4 LID - 10.1097/DSS.0000000000000380 [doi] FAU - Terrier, Jean-Etienne AU - Terrier JE AD - Department of Urology, Centre Hospitalier Lyon-Sud, Lyon, France Department of Thoracic Surgery, Hôpital Nord, Saint Etienne, France Department of Urology, Hôpital Nord, Saint Etienne, France Department of Dermatology, Hôpital Nord, Saint Etienne, France. FAU - Tiffet, Olivier AU - Tiffet O FAU - Raynaud, Néli AU - Raynaud N FAU - Cinotti, Elisa AU - Cinotti E LA - eng PT - Case Reports PT - Letter PL - United States TA - Dermatol Surg JT - Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] JID - 9504371 SB - IM MH - Adenocarcinoma/diagnosis/*pathology/surgery MH - Aged MH - Humans MH - Male MH - Microscopy, Confocal/*instrumentation MH - Neoplasm Recurrence, Local/prevention & control MH - Paget Disease, Extramammary/diagnosis/*pathology/*surgery MH - Penile Neoplasms/diagnosis/*pathology/*surgery MH - Prostatic Neoplasms/diagnosis/*pathology/surgery MH - Scrotum/*pathology/*surgery MH - Skin Transplantation MH - Staining and Labeling MH - Surgical Flaps EDAT- 2015/06/24 06:00 MHDA- 2015/09/02 06:00 CRDT- 2015/06/24 06:00 PHST- 2015/06/24 06:00 [entrez] PHST- 2015/06/24 06:00 [pubmed] PHST- 2015/09/02 06:00 [medline] AID - 00042728-201507000-00018 [pii] AID - 10.1097/DSS.0000000000000380 [doi] PST - ppublish SO - Dermatol Surg. 2015 Jul;41(7):862-4. doi: 10.1097/DSS.0000000000000380. PMID- 25727180 OWN - NLM STAT- MEDLINE DCOM- 20161024 LR - 20220419 IS - 1098-1101 (Electronic) IS - 0733-2459 (Linking) VI - 30 IP - 6 DP - 2015 Dec TI - Therapeutic plasma exchange in antisynthetase syndrome with severe interstitial lung disease. PG - 375-9 LID - 10.1002/jca.21387 [doi] AB - Antisynthetase syndrome (ASS) is a rare condition characterized by interstitial lung disease (ILD), inflammatory myositis, fever, Raynaud phenomenon, mechanic's hand, and inflammatory polyarthritis in the setting of antibodies to amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. Prognosis is very poor especially when there is associated ILD. To date, there is no standardized treatment for ILD associated ASS. Therapy is based on the use of steroids alone or in combination with other immunosuppressive agents, especially in severe or refractory cases. The role of therapeutic plasma exchange (TPE) in the management of this rare condition has not been established. Here, we report a case of severe ILD associated ASS in a 41-year-old woman who did not show clinical or laboratory response after six doses of high dose steroids and a dose of IV cyclophosphamide. Because of the aggressive nature of her disease and poor prognostic indices present, a decision was made to add TPE to her treatment. She underwent five sessions of TPE. At the end of the 5th session, the anti-Jo-1 antibody levels dropped to 3.6 AI (antibody index) and her creatinine kinase (CK) level from 875 to 399 U L(-1) (Units per liter) with overall improvement in her respiratory status. This case suggests TPE may be a promising treatment option in patients with ILD associated ASS refractory to steroids and other immunosuppressive therapy, particularly those with severe disease. CI - © 2015 Wiley Periodicals, Inc. FAU - Omotoso, Bolanle A AU - Omotoso BA AD - Division of Nephrology, University of Virginia, Charlottesville, Virginia. AD - Department of Medicine, Obafemi Awolowo University Teaching Hospitals' Complex, Ile - Ife, Osun State, Nigeria. FAU - Ogden, Melissa I AU - Ogden MI AD - School of Medicine, University of Virginia, Charlottesville, Virginia. FAU - Balogun, Rasheed A AU - Balogun RA AD - Division of Nephrology, University of Virginia, Charlottesville, Virginia. LA - eng PT - Case Reports PT - Journal Article DEP - 20150225 PL - United States TA - J Clin Apher JT - Journal of clinical apheresis JID - 8216305 RN - 0 (Antibodies, Antinuclear) RN - 0 (Jo-1 antibody) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - EC 6.1.1.21 (Histidine-tRNA Ligase) RN - Antisynthetase syndrome SB - IM MH - Adult MH - Amino Acyl-tRNA Synthetases/immunology MH - Antibodies, Antinuclear/blood/isolation & purification MH - Female MH - Histidine-tRNA Ligase/immunology MH - Humans MH - Lung Diseases, Interstitial/enzymology/immunology/*therapy MH - Myositis/enzymology/immunology/*therapy MH - *Plasma Exchange MH - Syndrome MH - Treatment Outcome OTO - NOTNLM OT - antisynthetase syndrome OT - interstitial lung disease OT - therapeutic plasma exchange EDAT- 2015/03/03 06:00 MHDA- 2016/10/25 06:00 CRDT- 2015/03/03 06:00 PHST- 2014/10/23 00:00 [received] PHST- 2015/02/09 00:00 [accepted] PHST- 2015/03/03 06:00 [entrez] PHST- 2015/03/03 06:00 [pubmed] PHST- 2016/10/25 06:00 [medline] AID - 10.1002/jca.21387 [doi] PST - ppublish SO - J Clin Apher. 2015 Dec;30(6):375-9. doi: 10.1002/jca.21387. Epub 2015 Feb 25. PMID- 38022564 OWN - NLM STAT- MEDLINE DCOM- 20231201 LR - 20241013 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 14 DP - 2023 TI - Reactome pathway analysis from whole-blood transcriptome reveals unique characteristics of systemic sclerosis patients at the preclinical stage. PG - 1266391 LID - 10.3389/fimmu.2023.1266391 [doi] LID - 1266391 AB - OBJECTIVE: This study aims to characterize differential expressed pathways (DEP) in subjects with preclinical systemic sclerosis (PreSSc) characterized uniquely by Raynaud phenomenon, specific autoantibodies, and/or capillaroscopy positive for scleroderma pattern. METHODS: Whole-blood samples from 33 PreSSc with clinical prospective data (baseline and after 4 years of follow-up) and 16 matched healthy controls (HC) were analyzed for global gene expression transcriptome analysis via RNA sequencing. Functional Analysis of Individual Microarray Expression method annotated Reactome individualized pathways. ANOVA analysis identified DEP whose predictive capability were tested in logistic regression models after extensive internal validation. RESULTS: At 4 years, 42.4% subjects progressed (evolving PreSSc), while the others kept stable PreSSc clinical features (stable PreSSc). At baseline, out of 831 pathways, 541 DEP were significant at a false discovery rate <0.05, differentiating PreSSc versus HC with an AUROC = 0.792 ± 0.242 in regression models. Four clinical groups were identified via unsupervised clustering (HC, HC and PreSSc with HC-like features, PreSSc and HC with PreSSc-like features, and PreSSc). Biological signatures changed with disease progression while remaining unchanged in stable subjects. The magnitude of change was related to the baseline cluster, yet no DEP at baseline was predictive of progression. Disease progression was mostly related to changes in signal transduction pathways especially linked to calcium-related events and inositol 1,4,5-triphosphate metabolism. CONCLUSION: PreSSc had distinguished Reactome pathway signatures compared to HC. Progression to definite SSc was characterized by a shift in biological fingertips. Calcium-related events promoting endothelial damage and vasculopathy may be relevant to disease progression. CI - Copyright © 2023 Bellocchi, Wang, Lyons, Marchini, Lorini, Carbonelli, Montano, Assassi and Beretta. FAU - Bellocchi, Chiara AU - Bellocchi C AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. FAU - Wang, Xuan AU - Wang X AD - Biostatistics, Baylor Institute for Immunology Research, Dallas, TX, United States. FAU - Lyons, Marka A AU - Lyons MA AD - Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX, United States. FAU - Marchini, Maurizio AU - Marchini M AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. FAU - Lorini, Maurizio AU - Lorini M AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. FAU - Carbonelli, Vincenzo AU - Carbonelli V AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. FAU - Montano, Nicola AU - Montano N AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. FAU - Assassi, Shervin AU - Assassi S AD - Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX, United States. FAU - Beretta, Lorenzo AU - Beretta L AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. LA - eng GR - R01 AR073284/AR/NIAMS NIH HHS/United States GR - R61 AR078078/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20231103 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 RN - SY7Q814VUP (Calcium) SB - IM MH - Humans MH - *Transcriptome MH - Prospective Studies MH - Calcium MH - *Scleroderma, Systemic MH - Disease Progression PMC - PMC10654742 OTO - NOTNLM OT - disease progression OT - gene expression OT - pathways OT - preclinical systemic sclerosis OT - systemic sclerosis COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/11/29 18:42 MHDA- 2023/12/01 06:44 PMCR- 2023/01/01 CRDT- 2023/11/29 15:26 PHST- 2023/07/24 00:00 [received] PHST- 2023/10/16 00:00 [accepted] PHST- 2023/12/01 06:44 [medline] PHST- 2023/11/29 18:42 [pubmed] PHST- 2023/11/29 15:26 [entrez] PHST- 2023/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2023.1266391 [doi] PST - epublish SO - Front Immunol. 2023 Nov 3;14:1266391. doi: 10.3389/fimmu.2023.1266391. eCollection 2023. PMID- 20222922 OWN - NLM STAT- MEDLINE DCOM- 20100628 LR - 20100428 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 162 IP - 5 DP - 2010 May TI - Nailfold capillary abnormalities are prevalent in sclerodermoid graft-versus-host disease and readily detected with dermatoscopy. PG - 1076-82 LID - 10.1111/j.1365-2133.2010.09667.x [doi] AB - BACKGROUND: Well-recognized videocapillaroscopic patterns have been described in systemic sclerosis (SS). However, no studies have described the capillary abnormalities of sclerodermoid chronic graft-versus-host disease (Scl GVHD) developed after allogeneic haematopoietic stem cell transplantation (allo-HSCT). OBJECTIVES: The aims of this study were to find the characteristics of nailfold capillary changes in Scl GVHD after allo-HSCT. PATIENTS AND METHODS: Eighteen patients affected by Scl GVHD and a control group of 15 patients with lichenoid GVHD were evaluated. Duration and type of sclerodermoid GVHD, Raynaud phenomenon (RP), dysphagia, joint contractures, antinuclear antibodies (ANA), anti-Scl-70 and anticentromere (ACA) antibodies were investigated parameters. A nailfold capillary examination using a standard dermatoscope was performed on all fingers of each subject. RESULTS: Twelve patients were male and six were female with a mean age of 37 +/- 11.6 years. Joint retractions and dysphagia developed in 27.8% and 38.9% of the patients, respectively. Three (16.7%) patients had RP. Autoimmune markers like anti-Scl-70 and ACA were negative in all. Capillaroscopy was abnormal in 15 patients with Scl GVHD. A regular disposition of the capillary loops along with avascular whitish linear areas at the level of the last row, neovascularization with reticular pattern, capillary disorganization, haemorrhages, enlarged capillaries and avascular areas were the main features. No capillary abnormalities were observed in patients with lichenoid GVHD. There was no statistically significant correlation between ANA positivity, RP, joint retractions, dysphagia, extensiveness of Scl GVHD, duration of sclerodermoid lesions and nailfold capillaroscopy analysis. CONCLUSIONS: This study shows the identification of distinct nailfold capillaroscopy patterns in patients with Scl GVHD but it does not confer special risk for any other specific clinical symptoms of the disease. FAU - Akay, B N AU - Akay BN AD - Departments of Dermatology and Hematology, University of Ankara School of Medicine, Ankara, Turkey. nisaakay15@yahoo.com FAU - Sanli, H AU - Sanli H FAU - Topcuoglu, P AU - Topcuoglu P FAU - Arat, M AU - Arat M FAU - Akyol, A AU - Akyol A LA - eng PT - Journal Article DEP - 20100305 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 SB - IM MH - Adult MH - Capillaries/pathology MH - Female MH - Graft vs Host Disease/etiology/*pathology MH - Hematopoietic Stem Cell Transplantation/adverse effects MH - Humans MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Nails/*blood supply MH - Neovascularization, Pathologic/etiology/pathology MH - Scleroderma, Systemic/etiology/*pathology MH - Young Adult EDAT- 2010/03/13 06:00 MHDA- 2010/06/29 06:00 CRDT- 2010/03/13 06:00 PHST- 2010/03/13 06:00 [entrez] PHST- 2010/03/13 06:00 [pubmed] PHST- 2010/06/29 06:00 [medline] AID - BJD9667 [pii] AID - 10.1111/j.1365-2133.2010.09667.x [doi] PST - ppublish SO - Br J Dermatol. 2010 May;162(5):1076-82. doi: 10.1111/j.1365-2133.2010.09667.x. Epub 2010 Mar 5. PMID- 17591642 OWN - NLM STAT- MEDLINE DCOM- 20080418 LR - 20241219 IS - 0300-5771 (Print) IS - 0300-5771 (Linking) VI - 36 IP - 4 DP - 2007 Aug TI - Circulatory disease and smokeless tobacco in Western populations: a review of the evidence. PG - 789-804 AB - BACKGROUND: Use of oral snuff or 'snus' has risen in Sweden. Sales of snuff in the US have also risen, overtaking sales of chewing tobacco. There is some evidence that nicotine contributes to circulatory disease (CID) from smoking. We therefore reviewed the evidence relating smokeless tobacco (ST) to CID and related risk factors. METHODS: Publications that described relevant cohort, case-control and cross-sectional studies were identified from in-house files, a Medline search in December 2005 and reference lists. Relative risks (RRs) and odds ratios (ORs) for ischaemic heart disease, stroke and all CID for ST use, stratified by smoking habit, were estimated and combined by meta-analysis to provide an overall RR estimate. For diabetes, increased blood pressure, and other risk factors, evidence was qualitatively reviewed, with results from clinical studies also considered. RESULTS: ST use in non-smokers was associated with an increased risk of heart disease (RR 1.12, 95% CI 0.99-1.27, n = 8), stroke (1.42, 1.29-1.57, n = 5) and CID (1.25, 1.14-1.37, n = 3). The increases mainly derived from two large US studies. The Swedish studies provided little evidence of an increase for heart disease (1.06, 0.83-1.37, n = 5) or stroke (1.17, 0.80-1.70, n = 2), although the estimates by country are not notably heterogeneous, even for stroke (P = 0.29). No dose-response was evident. No increase was seen in former users of ST, or in ST users who also smoked. No clear relationship to diabetes was seen. In the US, an acute blood pressure rise following ST use was consistently reported, and isolated reports linked specific risk factors to ST. In Sweden, though one study reported that snuff acutely increased blood pressure, and two linked snuff to Raynaud-type symptoms, the overall evidence for an effect was inconclusive. Swedish studies generally showed no chronic effect of snuff on blood pressure or various risk factors. CONCLUSIONS: Any CID risk from ST appears to be substantially less than from smoking, and no clear risk from Swedish snuff is seen. However, the overall evidence is limited. FAU - Lee, Peter N AU - Lee PN AD - PN Lee Statistics and Computing Ltd., 17 Cedar Road, Sutton, Surrey, SM2 5DA, UK. PeterLee@pnlee.co.uk LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't DEP - 20070625 PL - England TA - Int J Epidemiol JT - International journal of epidemiology JID - 7802871 SB - IM CIN - Int J Epidemiol. 2007 Aug;36(4):805-8. doi: 10.1093/ije/dym101. PMID: 17698883 MH - Cardiovascular Diseases/*etiology MH - Case-Control Studies MH - Cohort Studies MH - Cross-Sectional Studies MH - Humans MH - Odds Ratio MH - Risk Assessment/methods MH - Tobacco, Smokeless/*adverse effects EDAT- 2007/06/27 09:00 MHDA- 2008/04/19 09:00 CRDT- 2007/06/27 09:00 PHST- 2007/06/27 09:00 [pubmed] PHST- 2008/04/19 09:00 [medline] PHST- 2007/06/27 09:00 [entrez] AID - dym039 [pii] AID - 10.1093/ije/dym039 [doi] PST - ppublish SO - Int J Epidemiol. 2007 Aug;36(4):789-804. doi: 10.1093/ije/dym039. Epub 2007 Jun 25. PMID- 33375368 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210218 IS - 2227-9059 (Print) IS - 2227-9059 (Electronic) IS - 2227-9059 (Linking) VI - 9 IP - 1 DP - 2020 Dec 26 TI - Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential. LID - 10.3390/biomedicines9010017 [doi] LID - 17 AB - In 2015 the European Respiratory Society (ERS) and the American Thoracic Society (ATS) "Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" proposed classification criteria for a new research category defined as "Interstitial Pneumonia with Autoimmune Features" (IPAF), to uniformly define patients with interstitial lung disease (ILD) and features of autoimmunity, without a definite connective tissue disease. These classification criteria were based on a variable combination of features obtained from three domains: a clinical domain consisting of extra-thoracic features, a serologic domain with specific autoantibodies, and a morphologic domain with imaging patterns, histopathological findings, or multicompartment involvement. Features suggesting a systemic vasculitis were excluded. Since publication of ERS/ATS IPAF research criteria, various retrospective studies have been published focusing on prevalence; clinical, morphological, and serological features; and prognosis of these patients showing a broad heterogeneity in the results. Recently, two prospective, cohort studies were performed, confirming the existence of some peculiarities for this clinical entity and the possible progression of IPAF to a defined connective tissue disease (CTD) in about 15% of cases. Moreover, a non-specific interstitial pneumonia pattern, an anti-nuclear antibody positivity, and a Raynaud phenomenon were the most common findings. In comparison with idiopathic pulmonary fibrosis (IPF), IPAF patients showed a better performance in pulmonary function tests and less necessity of oxygen delivery. However, at this stage of our knowledge, we believe that further prospective studies, possibly derived from multicenter cohorts and through randomized control trials, to further validate the proposed classification criteria are needed. FAU - Sebastiani, Marco AU - Sebastiani M AUID- ORCID: 0000-0002-1294-6421 AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy. FAU - Faverio, Paola AU - Faverio P AD - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, University of Milan-Bicocca, 20900 Monza, Italy. FAU - Manfredi, Andreina AU - Manfredi A AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy. FAU - Cassone, Giulia AU - Cassone G AUID- ORCID: 0000-0002-3454-401X AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy. FAU - Vacchi, Caterina AU - Vacchi C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy. FAU - Stainer, Anna AU - Stainer A AD - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, University of Milan-Bicocca, 20900 Monza, Italy. FAU - Pozzi, Maria Rosa AU - Pozzi MR AD - Rheumatology Unit, San Gerardo Hospital, 20900 Monza, Italy. FAU - Salvarani, Carlo AU - Salvarani C AD - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy. AD - Rheumatology Unit, Dipartimento Medicina Interna e Specialità Mediche, Azienda Unità Sanitaria Locale di Reggio Emilia-Istituto di Ricerca e Cura a Carattere Scientifico, 42123 Reggio Emilia, Italy. FAU - Pesci, Alberto AU - Pesci A AD - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, University of Milan-Bicocca, 20900 Monza, Italy. FAU - Luppi, Fabrizio AU - Luppi F AUID- ORCID: 0000-0001-5775-5947 AD - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, University of Milan-Bicocca, 20900 Monza, Italy. LA - eng PT - Journal Article PT - Review DEP - 20201226 PL - Switzerland TA - Biomedicines JT - Biomedicines JID - 101691304 PMC - PMC7824155 OTO - NOTNLM OT - antibody OT - autoimmunity OT - classification OT - connective tissue diseases OT - idiopathic interstitial pneumonias OT - interstitial lung diseases OT - interstitial pneumonia with autoimmune features OT - prognosis COIS- The authors declare no conflict of interest. EDAT- 2020/12/31 06:00 MHDA- 2020/12/31 06:01 PMCR- 2020/12/26 CRDT- 2020/12/30 01:04 PHST- 2020/11/13 00:00 [received] PHST- 2020/12/21 00:00 [revised] PHST- 2020/12/24 00:00 [accepted] PHST- 2020/12/30 01:04 [entrez] PHST- 2020/12/31 06:00 [pubmed] PHST- 2020/12/31 06:01 [medline] PHST- 2020/12/26 00:00 [pmc-release] AID - biomedicines9010017 [pii] AID - biomedicines-09-00017 [pii] AID - 10.3390/biomedicines9010017 [doi] PST - epublish SO - Biomedicines. 2020 Dec 26;9(1):17. doi: 10.3390/biomedicines9010017. PMID- 24833757 OWN - NLM STAT- MEDLINE DCOM- 20150223 LR - 20140702 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 41 IP - 7 DP - 2014 Jul TI - Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center. PG - 1304-10 LID - 10.3899/jrheum.130984 [doi] AB - OBJECTIVE: Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE. METHODS: We analyzed 852 patients with SLE who attended our clinic. Seven autoantibodies were selected for cluster analysis: anti-DNA, anti-Sm, anti-RNP, anticardiolipin (aCL) immunoglobulin (Ig)G or IgM, lupus anticoagulant (LAC), anti-Ro, and anti-La. Two-step clustering and Kaplan-Meier survival analyses were used. RESULTS: Five clusters were identified. A cluster consisted of patients with only anti-dsDNA antibodies, a cluster of anti-Sm and anti-RNP, a cluster of aCL IgG/M and LAC, and a cluster of anti-Ro and anti-La antibodies. Analysis revealed 1 more cluster that consisted of patients who did not belong to any of the clusters formed by antibodies chosen for cluster analysis. Sm/RNP cluster had significantly higher incidence of pulmonary hypertension and Raynaud phenomenon. DsDNA cluster had the highest incidence of renal involvement. In the aCL/LAC cluster, there were significantly more patients with neuropsychiatric involvement, antiphospholipid syndrome, autoimmune hemolytic anemia, and thrombocytopenia. According to the Systemic Lupus International Collaborating Clinics damage index, the highest frequency of damage was in the aCL/LAC cluster. Comparison of 10 and 20 years survival showed reduced survival in the aCL/LAC cluster. CONCLUSION: This study supports the existence of autoantibody clusters with distinct clinical features in SLE and shows that forming clinical subsets according to autoantibody clusters may be useful in predicting the outcome of the disease. Autoantibody clusters in SLE may exhibit differences according to the clinical setting or population. FAU - Artim-Esen, Bahar AU - Artim-Esen B AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. bahartimesen@gmail.com. FAU - Çene, Erhan AU - Çene E AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Şahinkaya, Yasemin AU - Şahinkaya Y AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Ertan, Semra AU - Ertan S AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Pehlivan, Özlem AU - Pehlivan Ö AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Kamali, Sevil AU - Kamali S AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Gül, Ahmet AU - Gül A AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Öcal, Lale AU - Öcal L AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Aral, Orhan AU - Aral O AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. FAU - Inanç, Murat AU - Inanç M AD - From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University, Istanbul, Turkey.B. Artim-Esen, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; E. Çene, Fellow, Department of Statistics, Faculty of Arts and Sciences, Yildiz Technical University; Y. Şahinkaya, MD, Fellow; S. Ertan, MD, Fellow; Ö. Pehlivan, MD, Fellow; S. Kamali, MD, Professor; A. Gül, MD, Professor; L. Öcal, MD, Professor; O. Aral, MD, Professor; M. Inanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. LA - eng PT - Journal Article DEP - 20140515 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Autoantibodies/*blood/immunology MH - Cluster Analysis MH - Female MH - Humans MH - Lupus Erythematosus, Systemic/blood/*immunology MH - Male MH - Middle Aged MH - Young Adult OTO - NOTNLM OT - AUTOANTIBODIES OT - CLUSTER OT - DAMAGE OT - DISEASE PATTERN OT - SURVIVAL OT - SYSTEMIC LUPUS ERYTHEMATOSUS EDAT- 2014/05/17 06:00 MHDA- 2015/02/24 06:00 CRDT- 2014/05/17 06:00 PHST- 2014/05/17 06:00 [entrez] PHST- 2014/05/17 06:00 [pubmed] PHST- 2015/02/24 06:00 [medline] AID - jrheum.130984 [pii] AID - 10.3899/jrheum.130984 [doi] PST - ppublish SO - J Rheumatol. 2014 Jul;41(7):1304-10. doi: 10.3899/jrheum.130984. Epub 2014 May 15. PMID- 36796874 OWN - NLM STAT- MEDLINE DCOM- 20230222 LR - 20260127 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 9 IP - 1 DP - 2023 Feb TI - Effect of nintedanib in patients with systemic sclerosis-associated interstitial lung disease and risk factors for rapid progression. LID - 10.1136/rmdopen-2022-002859 [doi] LID - e002859 AB - OBJECTIVE: To investigate the rate of decline in forced vital capacity (FVC), and the effect of nintedanib on the rate of decline in FVC, in subjects with systemic sclerosis-associated interstitial lung disease (SSc-ILD) who had risk factors for rapid decline in FVC. METHODS: The SENSCIS trial enrolled subjects with SSc and fibrotic ILD of ≥10% extent on high-resolution CT. The rate of decline in FVC over 52 weeks was analysed in all subjects and in those with early SSc (<18 months since first non-Raynaud symptom), elevated inflammatory markers (C reactive protein ≥6 mg/L and/or platelets ≥330×10(9)/L) or significant skin fibrosis (modified Rodnan skin score (mRSS) 15-40 or mRSS ≥18) at baseline. RESULTS: In the placebo group, the rate of decline in FVC was numerically greater in subjects with <18 months since first non-Raynaud symptom (-167.8 mL/year), elevated inflammatory markers (-100.7 mL/year), mRSS 15-40 (-121.7 mL/year) or mRSS ≥18 (-131.7 mL/year) than in all subjects (-93.3 mL/year). Nintedanib reduced the rate of FVC decline across subgroups, with a numerically greater effect in patients with these risk factors for rapid FVC decline. CONCLUSION: In the SENSCIS trial, subjects with SSc-ILD who had early SSc, elevated inflammatory markers or extensive skin fibrosis had a more rapid decline in FVC over 52 weeks than the overall trial population. Nintedanib had a numerically greater effect in patients with these risk factors for rapid ILD progression. CI - © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Khanna, Dinesh AU - Khanna D AUID- ORCID: 0000-0003-1412-4453 AD - Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA khannad@umich.edu. FAU - Maher, Toby M AU - Maher TM AUID- ORCID: 0000-0001-7192-9149 AD - Keck School of Medicine, University of Southern California, Los Angeles, California, USA. AD - National Heart and Lung Institute, Imperial College London, London, UK. FAU - Volkmann, Elizabeth R AU - Volkmann ER AUID- ORCID: 0000-0003-3750-6569 AD - Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, California, USA. FAU - Allanore, Yannick AU - Allanore Y AUID- ORCID: 0000-0002-6149-0002 AD - Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France. FAU - Smith, Vanessa AU - Smith V AUID- ORCID: 0000-0001-6271-7945 AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - Division of Rheumatology, University of Texas McGovern Medical School, Houston, Texas, USA. FAU - Kreuter, Michael AU - Kreuter M AUID- ORCID: 0000-0003-4402-2159 AD - Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Care Medicine, Thoraxklinik, University of Heidelberg and German Center for Lung Research, Heidelberg, Germany. AD - Department of Pneumology, RKH Clinic Ludwigsburg, Ludwigsburg, Germany. FAU - Hoffmann-Vold, Anna-Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Inflammatory and Fibrotic Rheumatic Disease Research Area, Oslo University Hospital, Oslo, Norway. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Stock, Christian AU - Stock C AUID- ORCID: 0000-0002-3493-3234 AD - Global Biostatistics and Data Sciences, Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany. FAU - Alves, Margarida AU - Alves M AD - TA Inflammation Med, Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany. FAU - Sambevski, Steven AU - Sambevski S AD - TA Inflammation Med, Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK. LA - eng PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - G6HRD2P839 (nintedanib) RN - 0 (Indoles) SB - IM MH - Humans MH - Fibrosis MH - *Lung Diseases, Interstitial/drug therapy/etiology MH - Risk Factors MH - *Scleroderma, Systemic/complications/drug therapy MH - Indoles PMC - PMC9936273 OTO - NOTNLM OT - Autoimmune Diseases OT - Pulmonary Fibrosis OT - Scleroderma, Systemic OT - Therapeutics COIS- Competing interests: DK reports grants from Bristol Myers Squibb, Horizon Therapeutics and Pfizer; consulting fees from AbbVie, BI, Bristol Myers Squibb, CSL Behring, Genentech, Horizon Therapeutics, Janssen, Prometheus, Talaris and Theraly; fees for presentations from AbbVie, BI, CSL Behring, Genentech, Horizon Therapeutics and Janssen; has a leadership or fiduciary role with Eicos; has received royalties or licences for the University of California Los Angeles Scleroderma Clinical Trials Consortium (SCTC) Gastrointestinal Tract instrument 2.0; and owns stock in Eicos. TMM reports consulting fees from AstraZeneca, Bayer, Blade Therapeutics, BI, Bristol Myers Squibb, Galapagos, Galecto, GlaxoSmithKline, IQVIA, Pliant, Respivant Sciences, Roche/Genentech, Theravance Biopharma and Veracyte; and fees for presentations from BI and Roche/Genentech. ERV reports grants from BI, Forbius, Kadmon and Horizon; and fees from BI for serving on advisory boards and for presentations. YA reports consulting fees from BI and Sanofi; fees for presentations from AbbVie, BI and Janssen; and has participated on Data Safety Monitoring Boards or advisory boards for BI, Chemomab, Curzion, Medsenic, Menarini, Prometheus and Sanofi. VS reports grants paid to her institution from the Belgian Fund for Scientific Research in Rheumatic Diseases, Research Foundation Flanders, BI and Janssen-Cilag; consulting fees and fees for presentations paid to herself and to her institution from BI; consulting fees paid to her institution from Janssen-Cilag; fees for presentations paid to her institution from Janssen-Cilag and UCB; support for travel paid to her institution by BI; and holds unpaid roles with the ACR and EULAR study groups on microcirculation, ERN-ReCONNET and the SCTC working group on capillaroscopy. SA reports grants paid to his institution from BI, Janssen and Momenta; consulting fees from AbbVie, AstraZeneca, BI, Corbus, CSL Behring and Novartis; and fees for presentations from Integrity Continuing Education. MKreuter reports grants, consulting fees and fees for presentations from BI and Roche; and holds leadership or fiduciary roles with Deutsche gesellschaft für Pneumologie, the European Respiratory Society and the German Respiratory Society. A-MH-V reports grants from BI; consulting fees from Arxx Therapeutics, Bayer, BI, Janssen, Lilly, Medscape, Merck Sharp & Dohme and Roche; fees for presentations from Arxx Therapeutics, Bayer, BI, Janssen, Lilly, Medscape, Merck Sharp & Dohme and Roche; support for travel from Actelion, BI, Medscape and Roche; and holds leadership or fiduciary roles with EUSTAR, the Nordic PH vision group and the Norwegian SSc study group. MKuwana reports grants paid to his institution from BI, MBL and Ono Pharmaceutical; consulting fees from BI, Chugai, Corbus and Mochida; fees for presentations from AbbVie, Asahi Kasei, Astellas, Bayer, BI, Chugai, Eisai, Janssen, Mitsubishi Tanabe and Ono Pharmaceutical; and has received royalties or licences from MBL. CS, MA and SS are employees of BI. CPD reports grants from Arxx Therapeutics, CSL Behring, GlaxoSmithKline, Inventiva and Servier; consulting fees from AbbVie, Acceleron, Bayer, BI, Corbus, CSL Behring, GlaxoSmithKline, Horizon Therapeutics, Inventiva, Roche and Sanofi; and fees for presentations from BI, Corbus and Janssen. EDAT- 2023/02/17 06:00 MHDA- 2023/02/22 06:00 PMCR- 2023/02/16 CRDT- 2023/02/16 20:52 PHST- 2022/11/11 00:00 [received] PHST- 2023/02/02 00:00 [accepted] PHST- 2023/02/16 20:52 [entrez] PHST- 2023/02/17 06:00 [pubmed] PHST- 2023/02/22 06:00 [medline] PHST- 2023/02/16 00:00 [pmc-release] AID - rmdopen-2022-002859 [pii] AID - 10.1136/rmdopen-2022-002859 [doi] PST - ppublish SO - RMD Open. 2023 Feb;9(1):e002859. doi: 10.1136/rmdopen-2022-002859. PMID- 37006667 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231204 IS - 2474-1272 (Electronic) IS - 2474-1264 (Print) IS - 2474-1264 (Linking) VI - 7 IP - 2 DP - 2023 Mar-Apr TI - Retinal Vasculopathy With Cerebral Leukoencephalopathy and Systemic Manifestations: Critical Role of Retina Specialists. PG - 171-177 LID - 10.1177/24741264221129095 [doi] AB - PURPOSE: To describe a case of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) to enhance early recognition of this often-missed diagnosis. METHODS: A case report is presented. RESULTS: A 50-year-old woman with a history of Raynaud phenomenon, memory difficulties, and a family history of strokes was referred for evaluation of a bilateral, small-vessel, occlusive disease refractory to immunosuppressive therapy. An extensive workup for treatable causes was unrevealing. Fifteen months after presentation, brain imaging showed white-matter lesions and dystrophic calcification, which led to the discovery of a pathogenic variant in TREX1 and the diagnosis of RVCL-S. CONCLUSIONS: Retina specialists play a critical role in the timely diagnosis of RVCL-S. Although the findings in this condition can mimic those in other common retinal vascular disorders, there are key characteristics that increase the suspicion for RVCL-S. Early recognition might decrease unnecessary therapies and procedures. CI - © The Author(s) 2022. FAU - Houghton, Odette M AU - Houghton OM AUID- ORCID: 0000-0002-9186-9371 AD - Department of Ophthalmology, Mayo Clinic, Scottsdale, AZ, USA. FAU - Carter, Jonathan AU - Carter J AD - Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA. FAU - Dhamija, Radhika AU - Dhamija R AD - Department of Clinical Genomics, Mayo Clinic, Scottsdale, AZ, USA. LA - eng PT - Case Reports PT - Journal Article DEP - 20221203 PL - United States TA - J Vitreoretin Dis JT - Journal of vitreoretinal diseases JID - 101700301 PMC - PMC10037756 OTO - NOTNLM OT - TREX1 OT - cerebroretinal vasculopathy OT - hereditary cerebral vasculopathy OT - hereditary endotheliopathy with retinopathy OT - inherited neurodegenerative disorder OT - nephropathy and stroke and hereditary vascular retinopathy OT - retinal vasculitis OT - retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations OT - white-matter abnormalities COIS- The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/04/04 06:00 MHDA- 2023/04/04 06:01 PMCR- 2023/12/03 CRDT- 2023/04/03 03:49 PHST- 2023/04/04 06:01 [medline] PHST- 2023/04/03 03:49 [entrez] PHST- 2023/04/04 06:00 [pubmed] PHST- 2023/12/03 00:00 [pmc-release] AID - 10.1177_24741264221129095 [pii] AID - 10.1177/24741264221129095 [doi] PST - epublish SO - J Vitreoretin Dis. 2022 Dec 3;7(2):171-177. doi: 10.1177/24741264221129095. eCollection 2023 Mar-Apr. PMID- 23252656 OWN - NLM STAT- MEDLINE DCOM- 20131112 LR - 20150813 IS - 1744-5078 (Electronic) IS - 0927-3948 (Linking) VI - 21 IP - 2 DP - 2013 Apr TI - Corticosteroid-associated osteonecrosis: a rare, but serious, complication in uveitis. PG - 102-7 LID - 10.3109/09273948.2012.740129 [doi] AB - PURPOSE: To examine the incidence and prevalence of osteonecrosis in uveitis patients. METHODS: An electronic medical record database search was conducted to identify uveitis patients with osteonecrosis and the number at risk for corticosteroid-related osteonecrosis from 2003 to 2012. The clinical and ophthalmologic features of the uveitis patients with osteonecrosis were assessed with retrospective chart reviews. RESULTS: Six uveitis patients with osteonecrosis were identified, comprising a prevalence of 1.5%. The incidence density was 0.19 per 100 person-years of follow-up. The uveitides included sarcoidosis, sympathetic ophthalmia, idiopathic retinal vasculitis, idiopathic chronic anterior and intermediate uveitis, Vogt-Koyanagi Harada disease, and Cogan syndrome. The duration of systemic corticosteroid treatment ranged from 6 weeks to 6 years. The potential systemic risk factors were Raynaud phenomenon, antiphospholipid and autoantibodies, sickle cell trait, and thalassemia. CONCLUSIONS: Although osteonecrosis appears to be a rare complication among uveitis patients, physicians should strive to minimize systemic corticosteroid use when appropriate. A higher level of suspicion for osteonecrosis may be warranted in patients with additional systemic risk factors. FAU - Smith, Wendy M AU - Smith WM AD - Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA. FAU - Larson, Theresa A AU - Larson TA FAU - Meleth, A Dhanu AU - Meleth AD FAU - Krishnadev, Nupura AU - Krishnadev N FAU - Nussenblatt, Robert B AU - Nussenblatt RB FAU - Sen, H Nida AU - Sen HN LA - eng GR - Intramural NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Intramural DEP - 20121219 PL - England TA - Ocul Immunol Inflamm JT - Ocular immunology and inflammation JID - 9312169 RN - 0 (Glucocorticoids) SB - IM MH - Adolescent MH - Adult MH - Female MH - Follow-Up Studies MH - Glucocorticoids/*adverse effects/therapeutic use MH - Humans MH - Incidence MH - Male MH - Middle Aged MH - Osteonecrosis/*chemically induced/diagnosis/epidemiology MH - Prevalence MH - Retrospective Studies MH - Risk Factors MH - United States/epidemiology MH - Uveitis/diagnosis/*drug therapy MH - Young Adult EDAT- 2012/12/21 06:00 MHDA- 2013/11/13 06:00 CRDT- 2012/12/21 06:00 PHST- 2012/12/21 06:00 [entrez] PHST- 2012/12/21 06:00 [pubmed] PHST- 2013/11/13 06:00 [medline] AID - 10.3109/09273948.2012.740129 [doi] PST - ppublish SO - Ocul Immunol Inflamm. 2013 Apr;21(2):102-7. doi: 10.3109/09273948.2012.740129. Epub 2012 Dec 19. PMID- 19210885 OWN - NLM STAT- MEDLINE DCOM- 20090406 LR - 20170112 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 26 IP - 6 DP - 2008 Nov-Dec TI - Pregnancy and childbirth after treatment with autologous hematopoietic stem cell transplantation for severe systemic sclerosis requiring parenteral nutrition. Ethical issues. PG - 1122-4 AB - We report a pregnancy in a patient who had undergone autologous hematopoietic stem cell transplantation (AHSCT) for diffuse cutaneous systemic sclerosis (SSc). SSc onset was at age 25, with Raynaud phenomenon and evolved to include diffuse cutaneous, arthritis, pulmonary fibrosis and extensive gastrointestinal involvement. AHSCT (cyclophosphamide/ATG for conditioning) was performed four years later with improvement of all features apart from the gastrointestinal symptoms requiring parenteral nutrition (PN). Forty months after AHSCT, she had a spontaneous miscarriage necessitating curettage. Despite advice to avoid pregnancy because of poor nutritional status and recurring catheter infections from her PN, she fell pregnant one year later. The pregnancy proceeded normally and she delivered at 34 weeks, under cesarean section. The baby girl, (1990g and 4 APGAR score) after initial respiratory distress, is now 4 years old with normal growth and development. Unfortunately, the patient died early in 2008 due to severe disease progression terminating with gastrointestinal obstruction and pericarditis. This first report of a successful pregnancy in a patient with diffuse SSc treated by AHSCT illustrates that despite the possibility for a normal pregnancy, the decision to do so includes aspects of maternal prognosis. FAU - Tyndall, A J AU - Tyndall AJ AD - Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-Louis, Groupe Hospitalier Assistance Publique-Hôpitaux de Paris, Paris France. FAU - Joly, F AU - Joly F FAU - Carbonne, B AU - Carbonne B FAU - Deligny, C N AU - Deligny CN FAU - Farge, D C AU - Farge DC LA - eng PT - Case Reports PT - Journal Article PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Adult MH - Ethics, Medical MH - Fatal Outcome MH - Female MH - *Hematopoietic Stem Cell Transplantation MH - Humans MH - Infant, Newborn MH - *Parenteral Nutrition MH - Pregnancy MH - *Pregnancy Complications MH - *Pregnancy Outcome MH - Prognosis MH - Scleroderma, Systemic/diet therapy/*therapy MH - Severity of Illness Index EDAT- 2009/02/13 09:00 MHDA- 2009/04/07 09:00 CRDT- 2009/02/13 09:00 PHST- 2009/02/13 09:00 [entrez] PHST- 2009/02/13 09:00 [pubmed] PHST- 2009/04/07 09:00 [medline] AID - 2538 [pii] PST - ppublish SO - Clin Exp Rheumatol. 2008 Nov-Dec;26(6):1122-4. PMID- 40295291 OWN - NLM STAT- MEDLINE DCOM- 20250429 LR - 20250904 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 104 IP - 17 DP - 2025 Apr 25 TI - Hematologic malignancies masquerading as rheumatologic diseases: A case series and review. PG - e42251 LID - 10.1097/MD.0000000000042251 [doi] LID - e42251 AB - RATIONALE: Hematologic malignancies can mimic rheumatologic diseases, presenting a significant diagnostic challenge due to overlapping clinical features. This study highlights 5 cases of hematologic malignancies presenting as rheumatologic disorders and discusses the diagnostic complexities involved. PATIENT CONCERNS: The patients, aged 64 to 78, presented with diverse rheumatologic symptoms including polyarthritis, vasculitis, Raynaud phenomenon, and systemic symptoms such as weight loss, fatigue, and night sweats. Initial workups suggested rheumatologic diagnoses, leading to delays in recognizing the underlying malignancies. DIAGNOSES: The diagnostic journey involved extensive laboratory testing, imaging, and, in all cases, bone marrow biopsies, which ultimately revealed hematologic malignancies: angioimmunoblastic T-cell lymphoma (AITL), extranodal marginal zone lymphoma, myelodysplastic syndrome (MDS), and multiple myeloma. Misleading initial findings, such as autoimmune serologies and transient responses to immunosuppressive therapy, complicated the diagnostic process. INTERVENTIONS: Ultimately, the patients included in this case series benefited from hematological malignancy-specific therapies. Delayed diagnosis impacted the treatment course and outcomes. OUTCOMES: Outcomes varied: 2 patients achieved symptom control with targeted therapy, while others experienced complications such as infections or disease progression, ultimately leading to mortality in some cases. Patient frustrations underscored the psychologic toll of diagnostic delays. LESSONS: Hematologic malignancies can present as atypical or refractory rheumatologic diseases, emphasizing the need for vigilance in patients with unusual clinical courses. Early consideration of malignancy in differential diagnoses, especially with atypical serologic or histopathologic findings, is critical to improving outcomes. CI - Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Clark, Julia AU - Clark J AD - Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Kowlessur, Makshada AU - Kowlessur M AD - Arthritis Research Canada, Vancouver, British Columbia, Canada. FAU - VanderVeer, Elysha AU - VanderVeer E AD - British Columbia Cancer Agency, Surrey, British Columbia, Canada. FAU - Shojania, Kamran AU - Shojania K AD - Arthritis Research Canada, Vancouver, British Columbia, Canada. AD - Division of Rheumatology, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Au, Sheila AU - Au S AD - Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Kim, Hyein AU - Kim H AD - Arthritis Research Canada, Vancouver, British Columbia, Canada. AD - Division of Rheumatology, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Wong, Shannon AU - Wong S AD - Division of Nephrology, University of British Columbia, Vancouver, British Columbia, Canada. FAU - Huang, Kun AU - Huang K AUID- ORCID: 0000-0002-0242-6959 AD - Arthritis Research Canada, Vancouver, British Columbia, Canada. AD - Division of Rheumatology, University of British Columbia, Vancouver, British Columbia, Canada. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Humans MH - Middle Aged MH - Diagnosis, Differential MH - *Rheumatic Diseases/diagnosis MH - *Hematologic Neoplasms/diagnosis/complications MH - Male MH - Female MH - Aged MH - Myelodysplastic Syndromes/diagnosis PMC - PMC12039978 OTO - NOTNLM OT - hematologic malignancy OT - multiple myeloma OT - myelodysplastic syndrome OT - rheumatologic syndromes COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2025/04/29 00:28 MHDA- 2025/04/29 06:25 PMCR- 2025/04/25 CRDT- 2025/04/28 23:03 PHST- 2025/04/29 06:25 [medline] PHST- 2025/04/29 00:28 [pubmed] PHST- 2025/04/28 23:03 [entrez] PHST- 2025/04/25 00:00 [pmc-release] AID - 00005792-202504250-00064 [pii] AID - MD-D-25-00025 [pii] AID - 10.1097/MD.0000000000042251 [doi] PST - ppublish SO - Medicine (Baltimore). 2025 Apr 25;104(17):e42251. doi: 10.1097/MD.0000000000042251. PMID- 28024309 OWN - NLM STAT- MEDLINE DCOM- 20170417 LR - 20181202 IS - 1439-1899 (Electronic) IS - 0174-304X (Linking) VI - 48 IP - 2 DP - 2017 Apr TI - Stroke as Initial Manifestation of Adenosine Deaminase 2 Deficiency. PG - 111-114 LID - 10.1055/s-0036-1597611 [doi] AB - Deficiency of adenosine deaminase 2 (ADA2) due to homozygous or compound heterozygous mutations in the cat eye syndrome chromosome region, candidate 1 (CECR1) gene causes an autoimmune phenotype with systemic vasculitis affecting the skin, inner organs, and the central nervous system. Typically, stroke has been reported to follow systemic inflammatory disease and predominantly affects posterior and central brain areas. Here, we describe one of the rare patients in whom acute mesencephalic stroke preceded systemic inflammation and presented as initial clinical symptom. Symptoms typical for ADA2 deficiency such as fever, livedo racemosa, abdominal colics, arthralgias, and Raynaud phenomenon were observed later. Moreover, angiography of cerebral arteries did not reveal typical vasculitic findings supporting the hypothesis that alternative mechanism of vascular occlusion might have caused the stroke. ADA2 deficiency should be considered in patients with childhood stroke despite the absence of systemic inflammation and cerebral vasculitis. CI - Georg Thieme Verlag KG Stuttgart · New York. FAU - Elbracht, Miriam AU - Elbracht M AD - Institute of Human Genetics, Medical Faculty, RWTH Aachen University, Aachen, Germany. FAU - Mull, Michael AU - Mull M AD - Department of Diagnostic and Interventional Neuroradiology, RWTH University Hospital Aachen, Aachen Germany. FAU - Wagner, Norbert AU - Wagner N AD - Department of Pediatrics, RWTH University Hospital Aachen, Aachen, Germany. FAU - Kuhl, Christiane AU - Kuhl C AD - Department of Diagnostic and Interventional Radiology, RWTH University Hospital Aachen, Aachen, Germany. FAU - Abicht, Angela AU - Abicht A AD - Medical Genetics Center (MGZ), Munich, Germany. FAU - Kurth, Ingo AU - Kurth I AD - Institute of Human Genetics, Medical Faculty, RWTH Aachen University, Aachen, Germany. FAU - Tenbrock, Klaus AU - Tenbrock K AD - Department of Pediatrics, RWTH University Hospital Aachen, Aachen, Germany. FAU - Häusler, Martin AU - Häusler M AD - Department of Pediatrics, RWTH University Hospital Aachen, Aachen, Germany. LA - eng PT - Case Reports PT - Journal Article DEP - 20161226 PL - Germany TA - Neuropediatrics JT - Neuropediatrics JID - 8101187 RN - 0 (Intercellular Signaling Peptides and Proteins) RN - EC 3.5.4.4 (ADA2 protein, human) RN - EC 3.5.4.4 (Adenosine Deaminase) SB - IM MH - Adenosine Deaminase/*deficiency/genetics MH - Brain/diagnostic imaging MH - Brain Ischemia/diagnosis/enzymology/*etiology/genetics MH - Diagnosis, Differential MH - Hand/pathology MH - Humans MH - Infant MH - Inflammation/*complications/diagnosis/enzymology/genetics MH - Intercellular Signaling Peptides and Proteins/*deficiency/genetics MH - Leg/pathology MH - Male MH - Mutation, Missense MH - Stroke/diagnosis/enzymology/*etiology/genetics EDAT- 2016/12/27 06:00 MHDA- 2017/04/18 06:00 CRDT- 2016/12/27 06:00 PHST- 2016/12/27 06:00 [pubmed] PHST- 2017/04/18 06:00 [medline] PHST- 2016/12/27 06:00 [entrez] AID - 10.1055/s-0036-1597611 [doi] PST - ppublish SO - Neuropediatrics. 2017 Apr;48(2):111-114. doi: 10.1055/s-0036-1597611. Epub 2016 Dec 26. PMID- 28640077 OWN - NLM STAT- MEDLINE DCOM- 20170717 LR - 20220419 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 96 IP - 25 DP - 2017 Jun TI - Improvement of peripheral artery disease with Sildenafil and Bosentan combined therapy in a patient with limited cutaneous systemic sclerosis: A case report. PG - e6988 LID - 10.1097/MD.0000000000006988 [doi] LID - e6988 AB - RATIONALE: Sildenafil, a phosphodiesterase-5-inhibitor and Bosentan, an endothelin-1-receptor antagonist combined therapy could have beneficial effect in systemic sclerosis (SSc) patients with peripheral artery disease. PATIENT CONCERNS: We report a case of a 48-year-old Black woman, who developed severe left limb claudication and walking limitation following a left femoropopliteal bypass occlusion in 2014. She was a heavy smoker and had a history of right middle cerebral artery ischemic stroke and bilateral Raynaud phenomenon. DIAGNOSES: According to the American College of Rheumatology/European League Against Rheumatism-2013 criteria, diagnosis of limited cutaneous SSc was retained with macrovascular lesions. She was referred for investigation of left limb claudication on treadmill using transcutaneous oxygen pressure measurement during exercise to argue for the vascular origin of the walking impairment. She had a severe left limb ischemia and the maximum walking distance (MWD) she reached was 118 m in March 2015 despite the medical optimal treatment and walking rehabilitation. INTERVENTIONS: Sildenafil, 20 mg tid, was introduced due to active digital ulcers. In July 2015, the MWD increased to 288 m, then to 452 m in December 2015. Adding Bosentan to Sildenafil to prevent recurrent digital ulcers resulted in an MWD of 1576 m. OUTCOMES: Recently, the patient is treated with the combined therapy. She has no more pain during walking and his quality of life has improved. LESSONS: Sildenafil and Bosentan combined therapy was associated in our case with an improvement of MWD without adverse effect. Further clinical trials are necessary to confirm our original observation. FAU - Omarjee, Loukman AU - Omarjee L AD - Unité de Médecine Vasculaire, INSERM CIC 1414, Pôle Imagerie Médicale et Explorations Fonctionnelles, CHU de Rennes, 35033 Rennes Cedex, France. MitoVasc Institute, UMR CNRS 6015-INSERM U1083, Angers University Hospital, Angers Cedex Unité de Médecine Vasculaire, Plateau des Consultations Médicales, Cholet Cedex France. FAU - Fontaine, Cedric AU - Fontaine C FAU - Mahe, Guillaume AU - Mahe G FAU - Jaquinandi, Vincent AU - Jaquinandi V LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Cardiovascular Agents) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Sulfonamides) RN - BW9B0ZE037 (Sildenafil Citrate) RN - Q326023R30 (Bosentan) SB - IM MH - Bosentan MH - Cardiovascular Agents/*therapeutic use MH - Drug Therapy, Combination MH - Endothelin Receptor Antagonists/therapeutic use MH - Female MH - Humans MH - Middle Aged MH - Peripheral Arterial Disease/*complications/*drug therapy MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Scleroderma, Systemic/*complications/drug therapy MH - Sildenafil Citrate/*therapeutic use MH - Sulfonamides/*therapeutic use PMC - PMC5484185 COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2017/06/24 06:00 MHDA- 2017/07/18 06:00 PMCR- 2017/06/23 CRDT- 2017/06/23 06:00 PHST- 2017/06/23 06:00 [entrez] PHST- 2017/06/24 06:00 [pubmed] PHST- 2017/07/18 06:00 [medline] PHST- 2017/06/23 00:00 [pmc-release] AID - 00005792-201706230-00006 [pii] AID - MD-D-17-00699 [pii] AID - 10.1097/MD.0000000000006988 [doi] PST - ppublish SO - Medicine (Baltimore). 2017 Jun;96(25):e6988. doi: 10.1097/MD.0000000000006988. PMID- 21919117 OWN - NLM STAT- MEDLINE DCOM- 20140505 LR - 20151119 IS - 1099-1611 (Electronic) IS - 1057-9249 (Linking) VI - 21 IP - 11 DP - 2012 Nov TI - Fear of recurrence and causal attributions in long-term survivors of testicular cancer. PG - 1222-8 LID - 10.1002/pon.2030 [doi] AB - BACKGROUND: The purpose was to examine the prevalence of fear of recurrence (FoR) in long-term testicular cancer survivors (TCSs) and the association between FoR and causal attributions of cancer. METHODS: Testicular cancer survivors were sampled from a clinical register and were sent a questionnaire assessing FoR, depression using Beck Depression Inventory II (BDI-II), physical symptoms (ototoxicity, neuropathy, and Raynaud-like phenomena), and causal attributions of testicular cancer. RESULTS: There were 316 TCSs who completed the questionnaires (response rate, 65%). The mean age was 47.6 years (standard deviation (SD) = 10.9), and the mean time since diagnosis was 12.0 years (SD = 3.0). Among the TCSs, 27.9% reported FoR. Univariate analyses revealed that FoR was associated with a BDI-II sum score of ≥19 (odds ratio (OR) = 7.07, p < 0.001) and attributing the cancer disease to psychological stress (OR = 2.57, p = 0.002). A multivariate analysis revealed associations between FoR and attributing the cancer disease to psychological stress (OR = 2.35, p = 0.010) and a BDI-II sum score ≥19 (OR = 5.82, p = 0.002). CONCLUSIONS: Fear of recurrence is prevalent in long-term TCSs. The observed relationship between FoR and a psychological causal attribution is probably complex and the direction of causality may be twofold: attributing the disease to a factor that is perceived as uncontrollable in nature could induce loss of control, and high levels of FoR may increase the need to gain control over the situation by pointing out factors that could be responsible for the disease such as psychological stress. CI - Copyright © 2011 John Wiley & Sons, Ltd. FAU - Pedersen, Anette Fischer AU - Pedersen AF AD - The Research Unit for General Practice, Research Center for Cancer Diagnosis in Primary Care, Aarhus University, Aarhus C, Denmark. AFP@alm.au.dk. FAU - Rossen, Philip AU - Rossen P FAU - Olesen, Frede AU - Olesen F FAU - von der Maase, Hans AU - von der Maase H FAU - Vedsted, Peter AU - Vedsted P LA - eng PT - Journal Article DEP - 20110825 PL - England TA - Psychooncology JT - Psycho-oncology JID - 9214524 SB - IM MH - Adult MH - Age Factors MH - Aged MH - Causality MH - Depression/epidemiology/etiology MH - Fear/*psychology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Neoplasm Recurrence, Local/epidemiology/*psychology MH - Personality Inventory MH - Prevalence MH - Psychiatric Status Rating Scales MH - Quality of Life/psychology MH - *Recurrence MH - Regression Analysis MH - Socioeconomic Factors MH - Stress, Psychological MH - Surveys and Questionnaires MH - Survivors/*psychology MH - Testicular Neoplasms/epidemiology/*psychology OTO - NOTNLM OT - cancer OT - causal attributions OT - fear of recurrence OT - long‐term survivorship OT - oncology EDAT- 2011/09/16 06:00 MHDA- 2014/05/06 06:00 CRDT- 2011/09/16 06:00 PHST- 2010/06/02 00:00 [received] PHST- 2011/06/08 00:00 [revised] PHST- 2011/06/08 00:00 [accepted] PHST- 2011/09/16 06:00 [entrez] PHST- 2011/09/16 06:00 [pubmed] PHST- 2014/05/06 06:00 [medline] AID - 10.1002/pon.2030 [doi] PST - ppublish SO - Psychooncology. 2012 Nov;21(11):1222-8. doi: 10.1002/pon.2030. Epub 2011 Aug 25. PMID- 29086923 OWN - NLM STAT- MEDLINE DCOM- 20190923 LR - 20220330 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 178 IP - 5 DP - 2018 May TI - The non-neuronal and nonmuscular effects of botulinum toxin: an opportunity for a deadly molecule to treat disease in the skin and beyond. PG - 1011-1019 LID - 10.1111/bjd.16080 [doi] AB - There is growing evidence that botulinum neurotoxins (BoNTs) exhibit biological effects on various human cell types with a host of associated clinical implications. This review aims to provide an update on the non-neuronal and nonmuscular effects of botulinum toxin. We critically analysed recent reports on the structure and function of cellular signalling systems subserving biological effects of BoNTs. The BoNT receptors and intracellular targets are not unique for neurotransmission. They have been found in both neuronal and non-neuronal cells, but there are differences in how BoNT binds to, and acts on, neuronal vs. non-neuronal cells. The non-neuronal cells that express one or more BoNT/A-binding proteins, and/or cleavage target synaptosomal-associated protein 25, include: epidermal keratinocytes; mesenchymal stem cells from subcutaneous adipose; nasal mucosal cells; urothelial cells; intestinal, prostate and alveolar epithelial cells; breast cell lines; neutrophils; and macrophages. Serotype BoNT/A can also elicit specific biological effects in dermal fibroblasts, sebocytes and vascular endothelial cells. Nontraditional applications of BoNT have been reported for the treatment of the following dermatological conditions: hyperhidrosis, Hailey-Hailey disease, Darier disease, inversed psoriasis, aquagenic palmoplantar keratoderma, pachyonychia congenita, multiple eccrine hydrocystomas, eccrine angiomatous hamartoma, eccrine sweat gland naevi, congenital eccrine naevus, Raynaud phenomenon and cutaneous leiomyomas. Experimental studies have demonstrated the ability of BoNT/A to protect skin flaps, facilitate wound healing, decrease thickness of hypertrophic scars, produce an anti-ageing effect, improve a mouse model of psoriasiform dermatitis, and have also revealed extracutaneous effects of BoNT arising from its anti-inflammatory and anticancer properties. BoNTs have a much wider range of applications than originally understood, and the individual cellular responses to the cholinergic impacts of BoNTs could provide fertile ground for future studies. CI - © 2017 British Association of Dermatologists. FAU - Grando, S A AU - Grando SA AUID- ORCID: 0000-0001-5769-8938 AD - Department of Dermatology, University of California, Irvine, Irvine, CA, U.S.A. FAU - Zachary, C B AU - Zachary CB AD - Department of Dermatology, University of California, Irvine, Irvine, CA, U.S.A. LA - eng SI - GENBANK/NCT00999869 SI - GENBANK/NCT00408798 SI - GENBANK/NCT00997815 SI - GENBANK/NCT02623829 SI - GENBANK/NCT01459666 SI - GENBANK/NCT02168634 SI - GENBANK/NCT00816517 SI - GENBANK/NCT02577185 SI - GENBANK/NCT02165111 SI - GENBANK/NCT01051687 SI - GENBANK/NCT00971620 SI - GENBANK/NCT01225341 SI - GENBANK/NCT00765375 SI - GENBANK/NCT01293552 SI - GENBANK/NCT00936533 SI - GENBANK/NCT02782702 SI - GENBANK/NCT02639052 PT - Journal Article PT - Review DEP - 20180325 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antineoplastic Agents) RN - 0 (Cosmetics) RN - 0 (Neuromuscular Agents) RN - 0 (Neurotoxins) RN - 0Y70779M1F (rimabotulinumtoxinB) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - EC 3.4.24.69 (incobotulinumtoxinA) SB - IM CIN - Br J Dermatol. 2018 May;178(5):999. doi: 10.1111/bjd.16493. PMID: 29785814 MH - Animals MH - Anti-Inflammatory Agents/pharmacology MH - Antineoplastic Agents/pharmacology MH - Botulinum Toxins, Type A/*pharmacology MH - Cells, Cultured MH - Connective Tissue/drug effects MH - Cosmetics/pharmacology MH - Disease Models, Animal MH - Humans MH - Neuromuscular Agents/*pharmacology MH - Neurotoxins/*pharmacology MH - Rabbits MH - Rats MH - Skin/blood supply MH - Skin Diseases/*drug therapy MH - Surgical Flaps/blood supply EDAT- 2017/11/01 06:00 MHDA- 2019/09/24 06:00 CRDT- 2017/11/01 06:00 PHST- 2017/10/16 00:00 [accepted] PHST- 2017/11/01 06:00 [pubmed] PHST- 2019/09/24 06:00 [medline] PHST- 2017/11/01 06:00 [entrez] AID - 10.1111/bjd.16080 [doi] PST - ppublish SO - Br J Dermatol. 2018 May;178(5):1011-1019. doi: 10.1111/bjd.16080. Epub 2018 Mar 25. PMID- 26190189 OWN - NLM STAT- MEDLINE DCOM- 20160523 LR - 20161125 IS - 1535-7732 (Electronic) IS - 1051-0443 (Linking) VI - 26 IP - 9 DP - 2015 Sep TI - Effect of Peripheral Artery Sympathetic Denervation on Muscle Microperfusion and Macroperfusion in an Animal Peripheral Artery Disease Model Using Contrast-Enhanced Ultrasound and Doppler Flow Measurement. PG - 1396-402.e2 LID - S1051-0443(15)00556-4 [pii] LID - 10.1016/j.jvir.2015.06.013 [doi] AB - PURPOSE: To determine the effects of catheter-based peripheral sympathetic denervation (CPSD) on peripheral artery sympathetic tone and peripheral microperfusion (PMP). MATERIALS AND METHODS: The effects of bilateral CPSD in common iliac arteries on PMP of the biceps femoris were determined in pigs using contrast-enhanced ultrasound, and mean transit time (mTT) and wash-in rate (WiR) were calculated during steady-state infusion of INN-sulfur-hexafluoride. Measurements were performed bilaterally at rest and during infusion of adenosine 70 μg/kg/min after unilateral moderate left external iliac artery stenosis. RESULTS: Before CPSD, PMP decreased significantly (P < .05) under adenosine stress compared with resting conditions, with right mTT of 7.5 seconds ± 3.6 versus 16.9 seconds ± 11.9 and WiR of 63.1 arbitrary units (AU) ± 49.0 versus 25.0 AU ± 17.5 and left mTT of 29.2 seconds ± 18.0 versus 56.3 seconds ± 38.7 and WiR of 13.6 AU ± 8.4 versus 6.0 AU ± 4.1. After CPSD, PMP did not differ significantly (P > .05) between conditions of adenosine stress and rest, with right mTT of 19.9 seconds ± 24.7 versus 23.2 seconds ± 21.0 and WiR of 16.2 AU ± 25.0 versus 20.5 AU ± 19.7 and left mTT of 23.3 seconds ± 23.1 versus 25.8 seconds ± 21.7 and WiR of 12.5 AU ± 6.2 versus 20.0 AU ± 12.1. CONCLUSIONS: CPSD reduced peripheral artery sympathetic tone and may be an alternative to surgical or computed tomography-guided sympathectomy for the treatment of end-stage peripheral artery disease and Raynaud phenomenon. CI - Copyright © 2015 SIR. Published by Elsevier Inc. All rights reserved. FAU - Steinberg, Verena A AU - Steinberg VA AD - Departments of Anaesthesiology and Intensive Care Medicine, University of Bonn, Bonn, Germany. FAU - Mommertz, Gottfried AU - Mommertz G AD - Department of Vascular and Endovascular Surgery, Marienhospital Aachen, Aachen, Germany. FAU - Thiesler, Thore AU - Thiesler T AD - Institute of Pathology, University of Bonn, Bonn, Germany. FAU - Kristiansen, Glen AU - Kristiansen G AD - Institute of Pathology, University of Bonn, Bonn, Germany. FAU - Schild, Hans AU - Schild H AD - Radiology, University of Bonn, Bonn, Germany. FAU - Naehle, Claas P AU - Naehle CP AD - Radiology, University of Bonn, Bonn, Germany. Electronic address: cp@naehle.net. LA - eng PT - Journal Article DEP - 20150717 PL - United States TA - J Vasc Interv Radiol JT - Journal of vascular and interventional radiology : JVIR JID - 9203369 RN - 0 (Contrast Media) SB - IM MH - Animals MH - Blood Flow Velocity MH - Contrast Media MH - Muscle, Skeletal/*blood supply/diagnostic imaging/*physiopathology MH - Peripheral Arterial Disease/*physiopathology/*surgery MH - Swine MH - Sympathectomy/*methods MH - Treatment Outcome MH - Ultrasonography, Doppler EDAT- 2015/07/21 06:00 MHDA- 2016/05/24 06:00 CRDT- 2015/07/21 06:00 PHST- 2014/11/20 00:00 [received] PHST- 2015/05/03 00:00 [revised] PHST- 2015/06/08 00:00 [accepted] PHST- 2015/07/21 06:00 [entrez] PHST- 2015/07/21 06:00 [pubmed] PHST- 2016/05/24 06:00 [medline] AID - S1051-0443(15)00556-4 [pii] AID - 10.1016/j.jvir.2015.06.013 [doi] PST - ppublish SO - J Vasc Interv Radiol. 2015 Sep;26(9):1396-402.e2. doi: 10.1016/j.jvir.2015.06.013. Epub 2015 Jul 17. PMID- 38805897 OWN - NLM STAT- MEDLINE DCOM- 20240612 LR - 20240625 IS - 1873-2364 (Electronic) IS - 0960-8966 (Linking) VI - 40 DP - 2024 Jul TI - Further expanding the phenotype of anti-Ku antibody associated disease in children and adolescents. PG - 7-15 LID - S0960-8966(24)00105-6 [pii] LID - 10.1016/j.nmd.2024.05.008 [doi] AB - Anti-Ku autoantibodies are associated with several autoimmune inflammatory diseases. We aimed to review our anti-Ku positive pediatric patients in this study. Four pediatric patients (all female) who had anti-Ku positivity were included (Patients 1-2-3 with idiopathic inflammatory myopathy (IIM); Patient 4 with chronic urticaria). Patient 1 (onset:10.5 years) had proximal muscle weakness, Raynaud phenomenon, sclerodactyly, hyperpigmentation, joint contracture, and tenosynovitis. The disease course was progressive despite treatment with corticosteroids, intravenous immunoglobulin (IVIG), plasma exchange, and 11 different immunosuppressive drugs. Patient 2 (onset:15 years) presented with proximal muscle weakness, fatigue, weight loss. She recovered normal muscle strength after treatment with corticosteroids, IVIG, methotrexate, cyclosporine A, mycophenolate mofetil. Patient 3 (onset:10 years) had juvenile dermatomyositis with proximal muscle weakness, Gottron's papules, and calcinosis. She also had anti-NXP2 positivity. Remission was achieved with corticosteroids, methotrexate, azathioprine, and infliximab. Muscle biopsy findings revealed a variable spectrum of necrosis, regeneration, perifascicular pattern, and inflammation. Patient 4 had only chronic urticaria (onset: 6.5 years). The striking features of this series were heterogeneity in clinical presentations including solely chronic urticaria and IIM; variable response to immunosuppressive treatments; and histopathology revealing a spectrum of necrosis, regeneration and inflammatory infiltration. Expanding the spectrum of anti-Ku positivity will allow better understanding of anti-Ku-associated phenotype clusters. CI - Copyright © 2024. Published by Elsevier B.V. FAU - Batu, Ezgi Deniz AU - Batu ED AD - Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. Electronic address: ezgidenizbatu@yahoo.com. FAU - Şener, Seher AU - Şener S AD - Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Haliloğlu, Göknur AU - Haliloğlu G AD - Department of Pediatrics, Division of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Talim, Beril AU - Talim B AD - Department of Pediatrics, Pathology Unit, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Şener, Burçin AU - Şener B AD - Department of Medical Microbiology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Şahiner, Ümit Murat AU - Şahiner ÜM AD - Department of Pediatrics, Division of Pediatric Allergy and Immunology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Bilginer, Yelda AU - Bilginer Y AD - Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Orhan, Diclehan AU - Orhan D AD - Department of Medical Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Aydıngöz, Üstün AU - Aydıngöz Ü AD - Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Özen, Seza AU - Özen S AD - Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. LA - eng PT - Case Reports PT - Journal Article DEP - 20240517 PL - England TA - Neuromuscul Disord JT - Neuromuscular disorders : NMD JID - 9111470 RN - EC 4.2.99.- (Ku Autoantigen) RN - 0 (Autoantibodies) RN - EC 3.6.4.12 (Xrcc6 protein, human) SB - IM MH - Humans MH - Female MH - Adolescent MH - Child MH - *Phenotype MH - *Ku Autoantigen/immunology MH - *Autoantibodies/blood MH - Myositis/immunology/drug therapy MH - Chronic Urticaria/drug therapy/immunology OTO - NOTNLM OT - Anti-ku antibody OT - Idiopathic inflammatory myopathy OT - Muscle biopsy OT - Scleroderma COIS- Declaration of competing interest Ezgi Deniz Batu received payment for the speakers bureau from Novartis. Seza Özen received consultancy fees and payment for speakers bureau from Novartis and Sobi. Other authors declare no conflicts of interest. EDAT- 2024/05/29 00:49 MHDA- 2024/06/13 00:42 CRDT- 2024/05/28 18:16 PHST- 2023/12/12 00:00 [received] PHST- 2024/04/21 00:00 [revised] PHST- 2024/05/13 00:00 [accepted] PHST- 2024/06/13 00:42 [medline] PHST- 2024/05/29 00:49 [pubmed] PHST- 2024/05/28 18:16 [entrez] AID - S0960-8966(24)00105-6 [pii] AID - 10.1016/j.nmd.2024.05.008 [doi] PST - ppublish SO - Neuromuscul Disord. 2024 Jul;40:7-15. doi: 10.1016/j.nmd.2024.05.008. Epub 2024 May 17. PMID- 35547828 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220516 IS - 2673-5059 (Electronic) IS - 2673-5059 (Linking) VI - 3 DP - 2022 TI - Case Report: Evidence of Migratory Silicone Particles Arising From Cohesive Silicone Breast Implants. PG - 730276 LID - 10.3389/fgwh.2022.730276 [doi] LID - 730276 AB - BACKGROUND: Silicone implants have been used since the 1960s for aesthetic purposes and breast reconstructions. During this period, many women have reported up to 40 similar symptoms, including fatigue, the emergence of autoimmune diseases, Raynaud Phenomenon, arthritis, arthralgias, and hair loss, among others. However, most of the time, these symptoms are neglected by doctors across different specialties and are most often considered a psychosomatic disease. Since 2017, many women suffering from the same complaints have formed social media groups to report their histories and subsequently describe the disease as Breast Implant Illness (BII). The phenomenon of gel bleed and silicone toxicity is known and accepted in literature, but silicone migration into the extracapsular space is still poorly demonstrated, due to the difficulty of monitoring its particles and access to patient data. METHODS: This work demonstrated the presence of silicone through pathological examination in post-explant breast capsules and in the synovial tissue of the right wrist, detected with special Modified Oil Red O (MORO) staining in a patient with a history of BII. The pathological results were compared to the breast MRI imaging files. RESULTS: The MRI images show the permeability change of the implant shell diagnosed as a water-droplet signal. It was also possible to diagnose the gel bleeding as the silicone-induced granuloma of breast implant capsule (SIGBIC) in both implants. Silicone gel bleed and migration of silicone were detected with MORO staining in and outside the capsule and in the synovial tissue of the right wrist. CONCLUSION: In this case study, we showed that silicone migration is possible via cohesive silicone gel breast implant leakage. The accumulation of silicone in the synovial tissue of the right wrist suggests local silicone toxicity and defects. CI - Copyright © 2022 Mustafá, Fleury and Dijkman. FAU - Mustafá, Jessica C R AU - Mustafá JCR AD - UEMS Universidade Do Estado Do Mato Grosso Do Sul, Campo Grande, Brazil. FAU - Fleury, Eduardo de Faria Castro AU - Fleury EFC AD - Centro Universitário São Camilo, Curso de Medicina, São Paulo, Brazil. FAU - Dijkman, Henry B P M AU - Dijkman HBPM AD - Institute of Applied Biosciences and Chemistry, HAN University of Applied Sciences, Nijmegen, Netherlands. LA - eng PT - Case Reports PT - Journal Article DEP - 20220425 PL - Switzerland TA - Front Glob Womens Health JT - Frontiers in global women's health JID - 101776281 PMC - PMC9085291 OTO - NOTNLM OT - cohesive silicone gel OT - histology OT - non-cohesive silicone gel OT - silicone breast implant OT - silicone gel bleed OT - silicone migration COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/05/14 06:00 MHDA- 2022/05/14 06:01 PMCR- 2022/04/25 CRDT- 2022/05/13 11:17 PHST- 2021/06/24 00:00 [received] PHST- 2022/03/15 00:00 [accepted] PHST- 2022/05/13 11:17 [entrez] PHST- 2022/05/14 06:00 [pubmed] PHST- 2022/05/14 06:01 [medline] PHST- 2022/04/25 00:00 [pmc-release] AID - 10.3389/fgwh.2022.730276 [doi] PST - epublish SO - Front Glob Womens Health. 2022 Apr 25;3:730276. doi: 10.3389/fgwh.2022.730276. eCollection 2022. PMID- 30894448 OWN - NLM STAT- MEDLINE DCOM- 20191216 LR - 20200409 IS - 1526-632X (Electronic) IS - 0028-3878 (Print) IS - 0028-3878 (Linking) VI - 92 IP - 15 DP - 2019 Apr 9 TI - Immune myopathy with large histiocyte-related myofiber necrosis. PG - e1763-e1772 LID - 10.1212/WNL.0000000000007260 [doi] AB - OBJECTIVE: To describe the features of a new, pathologically distinctive, acquired myopathy with an unusual pattern of scattered necrotic muscle fibers that are neighbored, surrounded, or invaded, by large, often multinucleated, histiocytic cells. METHODS: Retrospective review of records and muscle pathology of 4 patients. RESULTS: Clinical features common to our patients included muscle pain and proximal, symmetric, moderate to severe, weakness in the arms and legs progressing over 1-4 weeks. Patients had other associated systemic disorders, including anemia in all, and hemophagocytic lymphohistiocytosis, hepatic disease, Raynaud phenomenon, metastatic cancer, and cardiomyopathy, in 1 patient each. Serum creatine kinase (CK) levels at presentation were very high, ranging from 10,000 to 102,000 U/L. Three patients improved within 3 months after treatment. Muscle pathology included scattered necrotic muscle fibers with cytoplasm that stained for C(5b-9) complement, especially around fiber peripheries, pale on nicotinamide adenine dinucleotide and often dark on hematoxylin & eosin. Large, often multinucleated, cells with features of histiocytes, including anatomical features on electron microscopy and immunostaining for major histocompatibility complex Class I and histiocyte markers (HAM56, CD68, CD163, and S100), were usually closely apposed to the surface of, or invaded, necrotic myofibers. CONCLUSIONS: Patients with large-histiocyte-associated myopathy (LHIM) had a subacute onset of proximal predominant weakness, associated systemic disorders, very high serum CK, and a pathologically distinctive pattern of large histiocyte-associated muscle fiber necrosis. LHIM may be caused by an autoimmune, histiocyte-mediated attack directed against muscle fibers. CI - © 2019 American Academy of Neurology. FAU - Pestronk, Alan AU - Pestronk A AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. pestronka@neuro.wustl.edu. FAU - Sinha, Namita AU - Sinha N AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. FAU - Alhumayyd, Ziad AU - Alhumayyd Z AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. FAU - Ly, Cindy AU - Ly C AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. FAU - Schmidt, Robert AU - Schmidt R AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. FAU - Bucelli, Robert AU - Bucelli R AD - From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190320 PL - United States TA - Neurology JT - Neurology JID - 0401060 RN - 0 (Biomarkers) RN - EC 2.7.3.2 (Creatine Kinase) SB - IM MH - Adolescent MH - Adult MH - Biomarkers MH - Creatine Kinase/blood MH - Disease Progression MH - Female MH - Histiocytes/*pathology MH - Humans MH - Immunohistochemistry MH - Male MH - Middle Aged MH - Muscle Fibers, Skeletal/*pathology MH - Muscle Weakness/etiology MH - Muscular Diseases/*immunology/*pathology MH - Musculoskeletal Pain/etiology MH - Necrosis MH - Retrospective Studies PMC - PMC6511085 EDAT- 2019/03/22 06:00 MHDA- 2019/12/18 06:00 PMCR- 2020/04/09 CRDT- 2019/03/22 06:00 PHST- 2018/03/17 00:00 [received] PHST- 2018/12/10 00:00 [accepted] PHST- 2019/03/22 06:00 [pubmed] PHST- 2019/12/18 06:00 [medline] PHST- 2019/03/22 06:00 [entrez] PHST- 2020/04/09 00:00 [pmc-release] AID - WNL.0000000000007260 [pii] AID - NEUROLOGY2018887091 [pii] AID - 10.1212/WNL.0000000000007260 [doi] PST - ppublish SO - Neurology. 2019 Apr 9;92(15):e1763-e1772. doi: 10.1212/WNL.0000000000007260. Epub 2019 Mar 20. PMID- 32101845 OWN - NLM STAT- MEDLINE DCOM- 20200824 LR - 20200824 IS - 1882-0654 (Electronic) IS - 0009-918X (Linking) VI - 60 IP - 3 DP - 2020 Mar 31 TI - [Antisynthetase myopathy]. PG - 175-180 LID - 10.5692/clinicalneurol.cn-001383 [doi] AB - Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients' serum. A cohort study of muscle biopsy entitled "Integrated Diagnosis Project for Inflammatory Myopathies" revealed that of 460 patients with idiopathic inflammatory myopathies, 51 (11%; female:male, 31:20) had antisynthetase myopathy. It is noted that anti-OJ antibodies, one of anti-ARS antibody subtypes, are clearly detected by RNA immunoprecipitation, but not conventional detection methods including line blot and enzyme-linked immunosorbent assays. The combined mean onset age of the patients was 60 years (range 13-85 years). There were no significant HLA-DRB1 alleles associated with anti-ARS antibodies. All patients with antisynthetase myopathy patients presented muscle limb weakness; 14 had severe weakness, 17 neck weakness, 15 dysphagia, and 15 muscle atrophy. Although patients with anti-OJ antibodies showed severe muscle weakness, the clinical presentations defined by anti-ARS antibodies were relatively homogeneous. In muscle pathology, perifascicular necrosis is a distinctive hallmark of antisynthetase myopathy. Patients with antisynthetase myopathy responded to the combination of immunosuppressive therapy, with favorable outcomes. However, interstitial lung disease, found in 41 patients, was more closely related to mortality than myositis. Antisynthetase myopathy has a distinct clinical and histological entity among idiopathic inflammatory myopathies. FAU - Suzuki, Shigeaki AU - Suzuki S AD - Department of Neurology, Keio University School of Medicine. LA - jpn PT - Journal Article PT - Review DEP - 20200226 PL - Japan TA - Rinsho Shinkeigaku JT - Rinsho shinkeigaku = Clinical neurology JID - 0417466 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/*blood MH - Exanthema MH - Female MH - Humans MH - Immunoprecipitation MH - Immunosuppressive Agents/therapeutic use MH - Joint Diseases MH - Lung Diseases, Interstitial MH - Male MH - Middle Aged MH - Muscle Weakness MH - Muscles/pathology MH - *Muscular Diseases/diagnosis/drug therapy/pathology MH - Syndrome MH - Young Adult OTO - NOTNLM OT - aminoacyl transfer RNA synthetase (ARS) OT - anti-OJ antibodies OT - autoantibodies OT - inflammatory myopathies OT - muscle pathology EDAT- 2020/02/27 06:00 MHDA- 2020/08/25 06:00 CRDT- 2020/02/27 06:00 PHST- 2020/02/27 06:00 [pubmed] PHST- 2020/08/25 06:00 [medline] PHST- 2020/02/27 06:00 [entrez] AID - 10.5692/clinicalneurol.cn-001383 [doi] PST - ppublish SO - Rinsho Shinkeigaku. 2020 Mar 31;60(3):175-180. doi: 10.5692/clinicalneurol.cn-001383. Epub 2020 Feb 26. PMID- 16996579 OWN - NLM STAT- MEDLINE DCOM- 20070327 LR - 20070205 IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 36 IP - 4 DP - 2007 Feb TI - The overlap of Sjögren's syndrome with other systemic autoimmune diseases. PG - 246-55 AB - OBJECTIVE: To analyze the main diagnostic problems caused by the overlap between Sjögren's syndrome (SS) and other systemic autoimmune diseases (SAD). METHODS: We performed a MEDLINE search for articles published between January 1966 and December 2005 that specifically analyzed the overlap between SS and other SAD. We identified a list of diagnostic problems in patients with primary SS who had features considered typical of other SAD. RESULTS: Clinically, the main diagnostic problems occur in SS patients presenting with arthritis, Raynaud phenomenon, cutaneous features (subacute cutaneous lupus erythematosus, purpura, livedo reticularis, erythema nodosum), interstitial pulmonary disease, and cytopenias (leukopenia, thrombocytopenia). Immunologically, antiphospholipid antibodies (aPL) and antineutrophil cytoplasmic antibodies (ANCA) are the most frequent atypical autoantibodies found in primary SS, with a prevalence ranging between 10 and 20%. However, coexisting antiphospholipid syndrome or systemic vasculitis is only detected in around 10% of SS patients with aPL or ANCA. Anti-DNA and anticentromere antibodies have a prevalence of 5 to 10%, but are more closely related to clinical and/or laboratory data suggestive of associated systemic lupus erythematosus and limited systemic sclerosis, respectively, leading to the fulfillment of classification criteria for these diseases in more than 25% of cases. CONCLUSION: The wide variety of clinical and immunological manifestations of patients with primary SS often overlap with other SAD, making the differentiation between primary SS, SS associated with SAD, and SS-like presentations of some other SAD difficult. This overlap suggests that the current classification criteria are useful in differentiating between autoimmune and non-autoimmune processes but fail to clearly differentiate among SAD. FAU - Ramos-Casals, Manuel AU - Ramos-Casals M AD - Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain. mramos@clinic.ub.es FAU - Brito-Zerón, Pilar AU - Brito-Zerón P FAU - Font, Josep AU - Font J LA - eng PT - Journal Article PT - Review DEP - 20060922 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (Autoantibodies) RN - 0 (Peptides, Cyclic) RN - 0 (cyclic citrullinated peptide) SB - IM MH - Age Factors MH - Antibodies, Antiphospholipid/blood MH - Autoantibodies/*blood MH - Autoimmune Diseases/classification/*diagnosis MH - Diagnosis, Differential MH - Humans MH - Mixed Connective Tissue Disease/diagnosis MH - Peptides, Cyclic/immunology MH - Sjogren's Syndrome/*diagnosis/immunology MH - Skin Diseases/diagnosis MH - Vasculitis/diagnosis RF - 62 EDAT- 2006/09/26 09:00 MHDA- 2007/03/28 09:00 CRDT- 2006/09/26 09:00 PHST- 2006/05/24 00:00 [received] PHST- 2006/07/19 00:00 [revised] PHST- 2006/08/12 00:00 [accepted] PHST- 2006/09/26 09:00 [pubmed] PHST- 2007/03/28 09:00 [medline] PHST- 2006/09/26 09:00 [entrez] AID - S0049-0172(06)00120-X [pii] AID - 10.1016/j.semarthrit.2006.08.007 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2007 Feb;36(4):246-55. doi: 10.1016/j.semarthrit.2006.08.007. Epub 2006 Sep 22. PMID- 25293445 OWN - NLM STAT- MEDLINE DCOM- 20160204 LR - 20221207 IS - 1346-8138 (Electronic) IS - 0385-2407 (Linking) VI - 41 IP - 11 DP - 2014 Nov TI - Elevated plasma homocysteine level is possibly associated with skin sclerosis in a series of Japanese patients with systemic sclerosis. PG - 986-91 LID - 10.1111/1346-8138.12642 [doi] AB - Homocysteine is a sulfhydryl-containing amino acid that is derived from dietary methionine, and there has been increasing evidence that elevated plasma homocysteine levels are associated with increased risk of cardiovascular diseases, including carotid, coronary and peripheral arterial disease (PAD). The association of plasma homocysteine levels with peripheral vascular involvements, such as Raynaud phenomenon (RP), digital ulcers (DU) in systemic sclerosis (SSc) patients has not been well studied. The objective of this study was to examine plasma homocysteine levels and their clinical associations in patients with SSc. Plasma homocysteine levels in 151 Japanese patients with SSc and 20 healthy controls were examined. No significant differences were observed in plasma homocysteine levels between SSc patients and healthy individuals. Demographic and clinical features of the SSc patients revealed that severe skin sclerosis, anti-topoisomerase I antibody positivity, complications of DU, acro-osteolysis (AO) and interstitial lung disease (ILD) were significantly more prevalent among the patients with elevated plasma homocysteine levels. The plasma homocysteine levels were positively correlated with modified Rodnan total skin score. The plasma homocysteine levels in the SSc patients with DU, AO and ILD were significantly higher than those in the SSc without DU, AO and ILD, respectively. Plasma homocysteine levels did not correlate with either the mean or max intima-media thickness (IMT) or plaque score, suggesting that plasma homocysteine levels might not be associated with carotid artery atherosclerosis in SSc patients. The measurement of plasma homocysteine levels in SSc patients might be useful for the risk stratifications of severe skin sclerosis, DU and AO. CI - © 2014 Japanese Dermatological Association. FAU - Motegi, Sei-Ichiro AU - Motegi S AD - Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. FAU - Toki, Sayaka AU - Toki S FAU - Yamada, Kazuya AU - Yamada K FAU - Uchiyama, Akihiko AU - Uchiyama A FAU - Ishikawa, Osamu AU - Ishikawa O LA - eng PT - Journal Article DEP - 20141008 PL - England TA - J Dermatol JT - The Journal of dermatology JID - 7600545 RN - 0LVT1QZ0BA (Homocysteine) SB - IM MH - Aged MH - Asian People MH - Atherosclerosis/blood/complications MH - Carotid Artery Diseases/blood/complications MH - Carotid Intima-Media Thickness MH - Case-Control Studies MH - Female MH - Homocysteine/*blood MH - Humans MH - Japan MH - Male MH - Middle Aged MH - Scleroderma, Systemic/*blood/complications/pathology MH - Sclerosis MH - Skin/pathology OTO - NOTNLM OT - carotid intima-media thickness OT - digital ulcers OT - homocysteine OT - skin sclerosis OT - systemic sclerosis EDAT- 2014/10/09 06:00 MHDA- 2016/02/05 06:00 CRDT- 2014/10/09 06:00 PHST- 2014/08/01 00:00 [received] PHST- 2014/08/24 00:00 [accepted] PHST- 2014/10/09 06:00 [entrez] PHST- 2014/10/09 06:00 [pubmed] PHST- 2016/02/05 06:00 [medline] AID - 10.1111/1346-8138.12642 [doi] PST - ppublish SO - J Dermatol. 2014 Nov;41(11):986-91. doi: 10.1111/1346-8138.12642. Epub 2014 Oct 8. PMID- 40217731 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250415 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 14 IP - 7 DP - 2025 Mar 27 TI - Correlation Between the Peak of Skin Thickness Progression Rate and Onset of Cardiopulmonary Involvement in Thai Systemic Sclerosis Patients. LID - 10.3390/jcm14072281 [doi] LID - 2281 AB - Background/Objectives: Rapid skin thickness progression assessed using the modified Rodnan skin score (mRSS) is associated with poor outcomes in systemic sclerosis (SSc). However, the correlation between patterns of skin thickness and the onset of internal organ involvement remains unclear. This study aimed to determine the correlation between peak skin thickness progression rate (pSTPR) and the onset of internal organ involvement, particularly interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) among Thai SSc patients. Method: A prospective cohort study with retrospective analysis was conducted on adult SSc patients who experienced the onset of their first non-Raynaud phenomenon symptoms between January 2013 and December 2020 and had at least a 2-year follow-up. Patients with an overlap syndrome were excluded from this study. The pSTPR was calculated by dividing the peak of the mRSS by the duration of disease at the peak of the mRSS. Result: A total of 509 patients were included in this study. The majority of cases were female (351; 69.0%) and comprised diffuse cutaneous SSc subsets (353 cases; 69.4%). The respective mean age and median pSTPR was 48.2 ± 11.6 years and 1.63 points/year (interquartile range 0.5-4.4). The respective median durations of disease at the onset of significant ILD (>20% extent), ILD, and mean duration of disease at the onset of PAH were 3.4 (Q1-Q3 1.4-7.7), 5.4 (Q1-Q3 2.4-9.2), and 8.0 ± 4.9 years. pSTPR was negatively correlated with disease duration at the onset of significant ILD (Rho -0.509, p < 0.001), ILD (Rho -0.480, p < 0.001), PAH (Rho -0.372, p = 0.03), and disease duration from onset to death (Rho -0.367, p = 0.03) after adjusting for age, sex, and anti-topoisomerase I. Conclusion: SSc patients with a high skin thickness progression rate reaching the maximum point of mRSS were at risk of developing early ILD, PAH, and death. The pSTPR may be used to assess individuals at risk of experiencing early onset cardiopulmonary involvement in SSc. FAU - Rujirawinitchai, Piyanart AU - Rujirawinitchai P AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. FAU - Foocharoen, Chingching AU - Foocharoen C AUID- ORCID: 0000-0002-1964-4389 AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. FAU - Mahakkanukrauh, Ajanee AU - Mahakkanukrauh A AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. FAU - Suwannaroj, Siraphop AU - Suwannaroj S AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. FAU - Pongkulkiat, Patnarin AU - Pongkulkiat P AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. FAU - Onchan, Tippawan AU - Onchan T AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand. LA - eng GR - None/Research and Graduate Studies, Khon Kaen University, Thailand/ PT - Journal Article DEP - 20250327 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC11989290 OTO - NOTNLM OT - interstitial lung disease OT - modified Rodnan skin score OT - pulmonary arterial hypertension OT - pulmonary hypertension OT - scleroderma OT - systemic sclerosis COIS- The authors declare no conflict of interest. EDAT- 2025/04/12 21:52 MHDA- 2025/04/12 21:53 PMCR- 2025/03/27 CRDT- 2025/04/12 01:01 PHST- 2025/02/12 00:00 [received] PHST- 2025/03/21 00:00 [revised] PHST- 2025/03/24 00:00 [accepted] PHST- 2025/04/12 21:53 [medline] PHST- 2025/04/12 21:52 [pubmed] PHST- 2025/04/12 01:01 [entrez] PHST- 2025/03/27 00:00 [pmc-release] AID - jcm14072281 [pii] AID - jcm-14-02281 [pii] AID - 10.3390/jcm14072281 [doi] PST - epublish SO - J Clin Med. 2025 Mar 27;14(7):2281. doi: 10.3390/jcm14072281. PMID- 37833760 OWN - NLM STAT- MEDLINE DCOM- 20231101 LR - 20231122 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 21 IP - 1 DP - 2023 Oct 13 TI - Majority of new patient referrals to a large pediatric rheumatology center result in non-rheumatic diagnosis. PG - 120 LID - 10.1186/s12969-023-00910-y [doi] LID - 120 AB - OBJECTIVE: Pediatric rheumatology faces a looming supply-demand crisis. While strategies have been proposed to address the supply shortfall, investigation into the increased demand for pediatric rheumatic care has been limited. Herein, we analyze new patient visits to a large tertiary care pediatric rheumatology center to identify emerging trends in referrals and areas for potential intervention to meet this increased demand. METHODS: All patients referred to and seen by the University of Alabama at Birmingham Pediatric Rheumatology Division between January 2019 and December 2021 for a new patient evaluation were identified. Patient data was retrospectively abstracted, de-identified, and analyzed to develop trends in referrals and frequency of rheumatic disease, non-rheumatic disease, and specific diagnoses. RESULTS: During the study period, 2638 patients were referred to and seen in by the pediatric rheumatology division. Six hundred and ten patients (23.1%) were diagnosed with rheumatic disease. The most common rheumatic disease was juvenile idiopathic arthritis (JIA) at 45.6%, followed by primary Raynaud phenomenon (7.4%), recurrent fever syndromes (6.9%), vasculitides (6.7%), and inflammatory eye disease (6.2%). Of the 2028 patients (76.9%) diagnosed with a non-rheumatic condition, benign musculoskeletal pain was the most common (61.8%), followed by a combination of somatic conditions (11.6%), and non-inflammatory rash (7.7%). CONCLUSION: In this analysis of new patient referrals to a large pediatric rheumatology center, the majority of patients were diagnosed with a non-rheumatic condition. As a worsening supply-demand gap threatens the field of pediatric rheumatology, increased emphasis should be placed on reducing non-rheumatic disease referrals. CI - © 2023. BioMed Central Ltd., part of Springer Nature. FAU - Reiff, Daniel D AU - Reiff DD AUID- ORCID: 0000-0001-8522-0915 AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. daniel.reiff@boystown.org. AD - Department of Pediatric Rheumatology, Boys Town National Research Hospital, Omaha, NE, 68010, USA. daniel.reiff@boystown.org. FAU - Bridges, John M AU - Bridges JM AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Rife, Eileen C AU - Rife EC AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Gennaro, Victoria L AU - Gennaro VL AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - McAllister, Linda AU - McAllister L AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Reed, Annelle AU - Reed A AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Smith, Carolyn AU - Smith C AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Walker, Bethany AU - Walker B AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Weiser, Peter AU - Weiser P AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Smitherman, Emily A AU - Smitherman EA AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Stoll, Matthew L AU - Stoll ML AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Mannion, Melissa L AU - Mannion ML AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. FAU - Cron, Randy Q AU - Cron RQ AD - Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA. LA - eng PT - Journal Article DEP - 20231013 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 SB - IM MH - Child MH - Humans MH - *Rheumatology MH - Retrospective Studies MH - *Rheumatic Diseases/diagnosis/epidemiology MH - *Arthritis, Juvenile/diagnosis MH - Referral and Consultation PMC - PMC10571278 OTO - NOTNLM OT - Juvenile idiopathic arthritis OT - Musculoskeletal joint pain OT - Pediatric Rheumatology OT - Rheumatic disease COIS- None of the above authors report any relationships, conflicts of interest, or commercial interests specifically related to the submitted manuscript. RQC declares consulting fees for Sobi regarding the use of anakinra in clinical trials to treat macrophage activation syndrome; textbook royalties from Springer; speaker fees from Lilly, Sobi, MedStudy, and Arthros Rheum; and consulting or advisory fees from Sironax and CareerPhysician. RQC also serves on adjudication committees and has received personal fees to establish episodes of macrophage activation syndrome among patients enrolled in clinical trials (funded by Pfizer and AbbVie) for therapeutics used to treat juvenile idiopathic arthritis. RQC holds a patent on detecting rare long non-coding RNA mutations in people with COVID-19 unrelated to the content of this report. EAS and MLM are both supported by the Rheumatology Research Foundation Investigator Award. DDR, JMB, ECR, VLG, LM, AR, CS, BW, PW, and MLS declare no conflicts of interest. EDAT- 2023/10/14 10:45 MHDA- 2023/11/01 12:45 PMCR- 2023/10/13 CRDT- 2023/10/14 00:01 PHST- 2023/08/14 00:00 [received] PHST- 2023/10/11 00:00 [accepted] PHST- 2023/11/01 12:45 [medline] PHST- 2023/10/14 10:45 [pubmed] PHST- 2023/10/14 00:01 [entrez] PHST- 2023/10/13 00:00 [pmc-release] AID - 10.1186/s12969-023-00910-y [pii] AID - 910 [pii] AID - 10.1186/s12969-023-00910-y [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2023 Oct 13;21(1):120. doi: 10.1186/s12969-023-00910-y. PMID- 38224979 OWN - NLM STAT- MEDLINE DCOM- 20240403 LR - 20240403 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 51 IP - 4 DP - 2024 Apr 1 TI - Systemic Sclerosis in Individuals With Exposure to World Trade Center Ground Zero Rescue and Recovery Efforts: A Case Series. PG - 390-395 LID - 10.3899/jrheum.2023-0821 [doi] AB - OBJECTIVE: The World Trade Center (WTC) attack in New York resulted in a dust plume containing silica, hydrocarbons, and asbestos. Autoimmune disorders have been reported among those with WTC site exposure. The characteristics of individuals developing systemic sclerosis (SSc) have not been previously described. The purpose of this study was to describe the features of patients with SSc with WTC exposure. METHODS: Data were collected from 11 patients with SSc or SSc spectrum conditions who reported exposure to the WTC site. Seven patients completed an exposure assessment. RESULTS: Of the 11 patients, the majority (n = 8) were female. The median (range) for age at diagnosis was 46 (36-75) years, time between exposure and first non-Raynaud phenomenon SSc symptom was 8 (1-19) years, and time between exposure and diagnosis was 11 (2-18) years. Fifty-five percent had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, 3 patients had diffuse cutaneous SSc, 1 patient with SSc features met criteria for mixed connective tissue disease (CTD), and 2 patients had undifferentiated CTD with features of SSc. Four patients had overlapping features with other CTDs. Interstitial lung disease (ILD) was present in 10 patients. Five of 11 patients had a history of tobacco use. Seven of 7 patients who completed the questionnaire reported other hazardous exposures outside of WTC. Of these, only 2 patients reported personal protective equipment use. CONCLUSION: A high frequency of ILD and overlap features were observed among patients with SSc with WTC exposure. Future studies are needed to characterize this association. CI - Copyright © 2024 by the Journal of Rheumatology. FAU - Own, Maryam AU - Own M AUID- ORCID: 0009-0003-7127-8392 AD - M. Own, MD, Weill Cornell Medicine, Department of Medicine, New York; own.maryam@mayo.edu. FAU - Bloostein, Aliza AU - Bloostein A AD - A. Bloostein, BA, Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York. FAU - Spiera, Robert AU - Spiera R AUID- ORCID: 0000-0003-2911-6800 AD - R. Spiera, MD, J.R. Berman, MD, J.K. Gordon, MD, MS, K.S. Lakin, MD, MS, Weill Cornell Medicine, Department of Medicine, and Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York. FAU - Berman, Jessica R AU - Berman JR AUID- ORCID: 0000-0001-7472-0228 AD - R. Spiera, MD, J.R. Berman, MD, J.K. Gordon, MD, MS, K.S. Lakin, MD, MS, Weill Cornell Medicine, Department of Medicine, and Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York. FAU - Moline, Jacqueline AU - Moline J AUID- ORCID: 0000-0003-2763-9181 AD - J. Moline, MD, MSc, Northwell Health, Department of Occupational Medicine, Epidemiology and Prevention, Great Neck, New York, USA. FAU - Gordon, Jessica K AU - Gordon JK AUID- ORCID: 0000-0001-8068-0592 AD - R. Spiera, MD, J.R. Berman, MD, J.K. Gordon, MD, MS, K.S. Lakin, MD, MS, Weill Cornell Medicine, Department of Medicine, and Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York. FAU - Lakin, Kimberly S AU - Lakin KS AUID- ORCID: 0000-0001-9343-5545 AD - R. Spiera, MD, J.R. Berman, MD, J.K. Gordon, MD, MS, K.S. Lakin, MD, MS, Weill Cornell Medicine, Department of Medicine, and Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York. LA - eng PT - Journal Article DEP - 20240401 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Humans MH - Male MH - Female MH - Middle Aged MH - *Scleroderma, Systemic/epidemiology MH - *Lung Diseases, Interstitial/epidemiology/etiology/diagnosis MH - *Scleroderma, Diffuse MH - *Autoimmune Diseases OTO - NOTNLM OT - interstitial lung disease OT - occupational exposure OT - scleroderma OT - systemic sclerosis EDAT- 2024/01/16 00:42 MHDA- 2024/04/03 06:44 CRDT- 2024/01/15 20:42 PHST- 2023/12/29 00:00 [accepted] PHST- 2024/04/03 06:44 [medline] PHST- 2024/01/16 00:42 [pubmed] PHST- 2024/01/15 20:42 [entrez] AID - jrheum.2023-0821 [pii] AID - 10.3899/jrheum.2023-0821 [doi] PST - epublish SO - J Rheumatol. 2024 Apr 1;51(4):390-395. doi: 10.3899/jrheum.2023-0821. PMID- 34928551 STAT- Publisher CTDT- 20211216 PB - University of Washington, Seattle DP - 1993 TI - CLCN4-Related Neurodevelopmental Disorder. BTI - GeneReviews(®) AB - CLINICAL CHARACTERISTICS: CLCN4-related neurodevelopmental disorder (CLCN4-NDD), an X-linked disorder, is characterized in the 36 males reported to date by developmental delay or intellectual disability, behavioral/mental health issues (e.g., autism spectrum disorder, anxiety, hyperactivity, and bipolar disorder), epilepsy, and gastrointestinal dysfunction. The five heterozygous females with a de novo CLCN4 variant reported to date had findings very similar to those of affected males. Twenty-two of 25 heterozygous females identified in family studies following identification of an affected male were unaffected or had only mild specific learning difficulties and/or mental health concerns, whereas three were more severely affected. DIAGNOSIS/TESTING: The diagnosis of CLCN4-NDD is established in a male proband with suggestive findings and a hemizygous pathogenic variant in CLCN4 identified by molecular genetic testing. The diagnosis of CLCN4-NDD is usually established in a female proband with suggestive findings and a heterozygous pathogenic variant in CLCN4 identified by molecular genetic testing; however, the phenotype in females with a pathogenic variant can range from asymptomatic to severe. MANAGEMENT: Treatment of manifestations: Treatment is supportive and often includes multidisciplinary specialists in neurology, pediatrics, mental health, physiatry, occupational and physical therapy, gastroenterology, feeding therapy, ophthalmology, audiology, and medical genetics. Surveillance: Routine monitoring of neurologic findings (response to anti-seizure medications; emergence of new findings), development and educational progress, psychiatric/behavioral issues (response to medications; emergence of new findings), mobility and self-help skills, growth and gastrointestinal manifestations, ophthalmologic findings, hearing, and family support systems. GENETIC COUNSELING: CLCN4-NDD is inherited in an X-linked manner. The father of an affected male will not have the disorder nor will he be hemizygous for the CLCN4 pathogenic variant. If the mother of a proband has a CLCN4 pathogenic variant, the chance of transmitting it in each pregnancy is 50%: males who inherit the pathogenic variant will be affected; females who inherit the pathogenic variant will be heterozygotes and may be unaffected or have clinical findings ranging from mild learning difficulties and mental health concerns to severe manifestations. If the proband represents a simplex case and if the CLCN4 pathogenic variant cannot be detected in the leukocyte DNA of the mother, the risk to sibs is presumed to be low but greater than that of the general population. Once the CLCN4 pathogenic variant has been identified in an affected family member, prenatal and preimplantation genetic testing are possible. CI - Copyright © 1993-2026, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved. Test. FED - Adam, Margaret P ED - Adam MP FED - Bick, Sarah ED - Bick S FED - Mirzaa, Ghayda M ED - Mirzaa GM FED - Pagon, Roberta A ED - Pagon RA FED - Wallace, Stephanie E ED - Wallace SE FED - Amemiya, Anne ED - Amemiya A FAU - Palmer, Elizabeth Emma AU - Palmer EE AD - School of Women's and Children's Health, University of New South Wales, Randwick, New South Wales AD - Sydney Children's Hospitals Network, Sydney, New South Wales, Australia FAU - Nguyen, Matthew Huu AU - Nguyen MH AD - Department of Pathology, School of Medical Sciences, University of New South Wales, Randwick, New South Wales AD - Liverpool Hospital, Liverpool, New South Wales, Australia FAU - Forwood, Caitlin AU - Forwood C AD - Sydney Children's Hospitals Network, Sydney, New South Wales, Australia FAU - Kalscheuer, Vera AU - Kalscheuer V AD - Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany LA - eng PT - Review PT - Book Chapter PL - Seattle (WA) OTO - NLM OT - Raynaud-Claes Syndrome OT - H(+)/Cl(-) exchange transporter 4 OT - CLCN4 OT - CLCN4-Related Neurodevelopmental Disorder EDAT- 2021/12/16 00:00 CRDT- 2021/12/16 00:00 AID - NBK575836 [bookaccession] PMID- 19884217 OWN - NLM STAT- MEDLINE DCOM- 20100114 LR - 20221207 IS - 1477-0962 (Electronic) IS - 0961-2033 (Linking) VI - 18 IP - 14 DP - 2009 Dec TI - A clinical and epidemiological study of lupus erythematosus at a tertiary referral dermatology clinic in Korea. PG - 1320-6 LID - 10.1177/0961203309345769 [doi] AB - Dermatological examination is critical for the evaluation of lupus erythematosus. However, little is known about the epidemiology and clinical characteristics of the lupus erythematosus patients that visit dermatology clinics with the chief complaint of skin lesions, especially among Asian populations. We performed this study to determine the epidemiology of cutaneous lupus erythematosus for three subtypes: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus, and for lupus erythematosus non-specific skin disease. Also, we sought to determine the relationship between each type of lupus erythematosus, by the skin manifestations and systemic lupus erythematosus. The medical records of lupus erythematosus patients that were diagnosed by their clinical manifestations, skin biopsy results, and laboratory findings from January 1998 through December 2007 were reviewed. A total of 117 patients were diagnosed with lupus erythematosus; 62 cases had chronic cutaneous lupus erythematosus, 11 had subacute cutaneous lupus erythematosus, and 41 had acute cutaneous lupus erythematosus. The remaining three had systemic lupus erythematosus features with lupus erythematosus non-specific skin lesions such as Raynaud phenomenon, livedo reticularis/vasculitis, non-scarring alopecia, and periungual telangiectasia. The acute cutaneous lupus erythematosus subgroup showed extreme female predominance (9.2:1) whereas subacute and chronic cutaneous lupus erythematosus subgroups did not. Patients with chronic cutaneous lupus erythematosus tended to be older than other groups (peak incidence in the fifth decade). Incidence of laboratory abnormalities, including positive connective tissue markers such as antinuclear, double-strand DNA, and Ro/SS-A antibodies, were present in the order acute, subacute, and chronic cutaneous lupus erythematosus. Acute cutaneous lupus erythematosus almost always indicated systemic involvement of lupus erythematosus, whereas chronic cutaneous lupus erythematosus did not predict the development or existence of systemic lupus erythematosus and had a benign clinical course. Careful consideration of lupus erythematosus non-specific skin lesions may help detect systemic lupus erythematosus regardless of the diagnosis of cutaneous lupus erythematosus. FAU - Bae, Y I AU - Bae YI AD - Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea. FAU - Yun, S-J AU - Yun SJ FAU - Lee, J-B AU - Lee JB FAU - Kim, S-J AU - Kim SJ FAU - Won, Y H AU - Won YH FAU - Lee, S-C AU - Lee SC LA - eng PT - Journal Article DEP - 20091102 PL - England TA - Lupus JT - Lupus JID - 9204265 SB - IM MH - Acute Disease MH - Adult MH - Aged MH - Asian People/*statistics & numerical data MH - Child MH - Chronic Disease MH - Dermatology/statistics & numerical data MH - Female MH - Humans MH - Infant MH - Lupus Erythematosus, Discoid/*epidemiology/*pathology MH - Male MH - Middle Aged MH - Outpatient Clinics, Hospital/statistics & numerical data MH - Referral and Consultation/*statistics & numerical data MH - Republic of Korea/epidemiology MH - Retrospective Studies MH - Skin/pathology EDAT- 2009/11/04 06:00 MHDA- 2010/01/15 06:00 CRDT- 2009/11/04 06:00 PHST- 2009/11/04 06:00 [entrez] PHST- 2009/11/04 06:00 [pubmed] PHST- 2010/01/15 06:00 [medline] AID - 0961203309345769 [pii] AID - 10.1177/0961203309345769 [doi] PST - ppublish SO - Lupus. 2009 Dec;18(14):1320-6. doi: 10.1177/0961203309345769. Epub 2009 Nov 2. PMID- 37534955 OWN - NLM STAT- MEDLINE DCOM- 20230807 LR - 20260127 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 41 IP - 8 DP - 2023 Aug TI - Interstitial lung disease in patients with systemic sclerosis: what can we learn from the SENSCIS trial? PG - 1713-1719 LID - 10.55563/clinexprheumatol/trcv91 [doi] AB - The SENSCIS trial of nintedanib versus placebo is the largest trial conducted to date in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). This trial enrolled 576 patients with an extent of fibrotic ILD on high-resolution computed tomography of >10%. Median time since first non-Raynaud symptom was 3.4 years. Almost half of the patients were receiving a stable dose of mycophenolate at baseline. Key findings of the trial included that at baseline, despite having significant lung fibrosis on HRCT and impairment in lung function, 20% of the patients did not have cough and 30% did not have dyspnoea. Over 52 weeks, a marked decline in forced vital capacity (FVC) was observed (-112.0 mL/year in patients with diffuse cutaneous SSc [dcSSc] and -74.5 mL/year in patients with limited cutaneous SSc [lcSSc] in the placebo group). Loss of FVC was associated with an increased risk of SSc-related hospitalisation or death. Although certain subgroups of patients were at higher risk of progression, it was not possible to make a robust prediction of FVC decline based on baseline characteristics. The relative effect of nintedanib versus placebo on reducing the rate of FVC decline was consistent across subgroups based on factors including anti-topoisomerase I antibody (ATA) status, dcSSc vs. lcSSc, and use of mycophenolate at baseline. The side-effects of nintedanib were mainly gastrointestinal events, particularly diarrhoea. Nintedanib did not have a significant effect on skin fibrosis or health-related quality of life. Overall, the results of the SENSCIS trial support the importance of prompt identification and treatment of SSc-ILD and the consideration of nintedanib as a treatment option. FAU - Assassi, Shervin AU - Assassi S AD - Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, USA. shervin.assassi@uth.tmc.edu. FAU - Tumuluri, Sudhakar AU - Tumuluri S AD - Pacific Coast Dermatology and Wellness Center, Redwood City, CA, USA. FAU - Levin, Robert W AU - Levin RW AD - University of South Florida, Tampa, FL, USA. LA - eng PT - Journal Article PT - Review DEP - 20230803 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 RN - 0 (Immunosuppressive Agents) RN - G6HRD2P839 (nintedanib) RN - 0 (Indoles) SB - IM MH - Humans MH - Disease Progression MH - Fibrosis MH - Immunosuppressive Agents/adverse effects MH - *Lung Diseases, Interstitial/drug therapy/etiology/diagnosis MH - Quality of Life MH - *Scleroderma, Systemic/complications/drug therapy/pathology MH - Vital Capacity MH - Clinical Trials as Topic MH - Indoles EDAT- 2023/08/03 13:08 MHDA- 2023/08/04 06:43 CRDT- 2023/08/03 09:42 PHST- 2023/07/19 00:00 [received] PHST- 2023/07/26 00:00 [accepted] PHST- 2023/08/04 06:43 [medline] PHST- 2023/08/03 13:08 [pubmed] PHST- 2023/08/03 09:42 [entrez] AID - 20171 [pii] AID - 10.55563/clinexprheumatol/trcv91 [doi] PST - ppublish SO - Clin Exp Rheumatol. 2023 Aug;41(8):1713-1719. doi: 10.55563/clinexprheumatol/trcv91. Epub 2023 Aug 3. PMID- 35699136 OWN - NLM STAT- MEDLINE DCOM- 20220823 LR - 20250728 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 25 IP - 8 DP - 2022 Aug TI - Systemic sclerosis in Native Americans of the American Southwest. PG - 916-925 LID - 10.1111/1756-185X.14367 [doi] AB - OBJECTIVE: Many indigenous non-Caucasian populations, including Native Americans, have been reported to have higher rates, distinct clinical phenotypes, increased complications, and greater severity of systemic sclerosis (SSc). However, little is known of SSc specifically in Native Americans of the American Southwest. This study compared the clinical and serologic manifestations and outcomes of SSc in Native Americans and non-Native Americans (non-Natives) of this region. METHODS: This cross-sectional retrospective study included 137 SSc patients (109 [80%] were non-Native and 28 [20%] were Native Americans) followed over a mean of 11.5 ± 7.6 years. Participants were repetitively evaluated with medical history, physical examination, echocardiography, chest imaging, and serologic testing. Disease characteristics and outcomes were statistically compared between Native Americans and non-Native patients. RESULTS: The estimated prevalence of SSc in Native Americans was 40.0 cases/100 000 vs 17.1 cases/100 000 for non-Natives (odds ratio 2.34, 95% confidence interval [CI] 1.55-3.55, P < .001). The cohorts were similar in terms age, age of onset, limited vs diffuse cutaneous SSc, telangiectasias, gastroesophageal reflux disease, Raynaud phenomenon, serologies, interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, cancer prevalence, and overall mortality (all P > .05). However, for Native Americans, mortality specifically from fatal infections was 3.94-fold that of non-Natives (hazard ratio 6.88, 95% CI 1.37-34.64; P < .001). CONCLUSION: In Native Americans of the American Southwest, SSc is increased in prevalence but is phenotypically similar to SSc in non-Natives. However, mortality due specifically to infection is increased in Native Americans with SSc. CI - © 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Emil, N Suzanne AU - Emil NS AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Vondenberg, Jaime A AU - Vondenberg JA AD - Department of Medicine, Rheumatology/Immunology, Cleveland Clinic, Cleveland, Ohio, USA. FAU - Waters, Yvonne M AU - Waters YM AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Muruganandam, Maheswari AU - Muruganandam M AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Ariza-Hutchinson, Angie AU - Ariza-Hutchinson A AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Patel, Rosemina A AU - Patel RA AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Nunez, Sharon E AU - Nunez SE AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Gibb, James I AU - Gibb JI AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - McElwee, Matthew K AU - McElwee MK AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Poole, Janet L AU - Poole JL AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - O'Sullivan, Frank X AU - O'Sullivan FX AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Fields, Roderick A AU - Fields RA AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. FAU - Sibbitt, Wilmer L Jr AU - Sibbitt WL Jr AUID- ORCID: 0000-0001-5872-160X AD - Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA. LA - eng PT - Journal Article DEP - 20220614 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 SB - IM MH - Cross-Sectional Studies MH - Humans MH - *Lung Diseases, Interstitial/diagnosis MH - Retrospective Studies MH - *Scleroderma, Diffuse/complications MH - *Scleroderma, Systemic/complications/diagnosis/epidemiology MH - United States/epidemiology OTO - NOTNLM OT - Native American OT - health disparities OT - infections OT - mortality OT - scleroderma, systemic EDAT- 2022/06/15 06:00 MHDA- 2022/08/24 06:00 CRDT- 2022/06/14 06:03 PHST- 2022/05/27 00:00 [revised] PHST- 2022/02/21 00:00 [received] PHST- 2022/05/30 00:00 [accepted] PHST- 2022/06/15 06:00 [pubmed] PHST- 2022/08/24 06:00 [medline] PHST- 2022/06/14 06:03 [entrez] AID - 10.1111/1756-185X.14367 [doi] PST - ppublish SO - Int J Rheum Dis. 2022 Aug;25(8):916-925. doi: 10.1111/1756-185X.14367. Epub 2022 Jun 14. PMID- 34561652 OWN - NLM STAT- MEDLINE DCOM- 20211115 LR - 20230207 IS - 1759-4804 (Electronic) IS - 1759-4790 (Linking) VI - 17 IP - 11 DP - 2021 Nov TI - Detection of microvascular changes in systemic sclerosis and other rheumatic diseases. PG - 665-677 LID - 10.1038/s41584-021-00685-0 [doi] AB - Morphological and functional analysis of the microcirculation are objective outcome measures that are recommended for use in the presence of clinical signs of altered peripheral blood flow (such as Raynaud phenomenon), which can occur in systemic sclerosis (SSc) and other autoimmune rheumatic diseases. Several advanced non-invasive tools are available for monitoring the microcirculation, including nailfold videocapillaroscopy, which is the best-studied and most commonly used method for distinguishing and quantifying microvascular morphological alterations in SSc. Nailfold videocapillaroscopy can also be used alongside laser Doppler techniques to assist in the early diagnosis and follow-up of patients with dermatomyositis or mixed connective tissue disease. Power Doppler ultrasonography, which has been used for many years to evaluate the vascularity of synovial tissue in rheumatoid arthritis, is another promising tool for the analysis of skin and nailbed capillary perfusion in other autoimmune rheumatic diseases. Other emerging methods include raster-scanning optoacoustic mesoscopy, which offers non-invasive high-resolution 3D visualization of capillaries and has been tested in psoriatic arthritis and SSc. The principle functions and operative characteristics of several non-invasive tools for analysing microvascular changes are outlined in this Review, and the clinical roles of validated or tested imaging methods are discussed for autoimmune rheumatic diseases. CI - © 2021. Springer Nature Limited. FAU - Cutolo, Maurizio AU - Cutolo M AD - Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DiMI), University of Genova-IRCCS San Martino Polyclinic Hospital, Genoa, Italy. mcutolo@unige.it. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. AD - Department of Internal Medicine, Ghent University, Ghent, Belgium. AD - Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium. LA - eng PT - Journal Article PT - Review DEP - 20210924 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 SB - IM MH - *Autoimmune Diseases/complications/physiopathology MH - Humans MH - *Microcirculation MH - Rheumatic Diseases/complications/*physiopathology MH - Scleroderma, Systemic/complications/*physiopathology MH - Ultrasonography, Doppler MH - Vascular Diseases/*diagnostic imaging/etiology/physiopathology EDAT- 2021/09/26 06:00 MHDA- 2021/11/16 06:00 CRDT- 2021/09/25 06:18 PHST- 2021/08/13 00:00 [accepted] PHST- 2021/09/26 06:00 [pubmed] PHST- 2021/11/16 06:00 [medline] PHST- 2021/09/25 06:18 [entrez] AID - 10.1038/s41584-021-00685-0 [pii] AID - 10.1038/s41584-021-00685-0 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2021 Nov;17(11):665-677. doi: 10.1038/s41584-021-00685-0. Epub 2021 Sep 24. PMID- 31157951 OWN - NLM STAT- MEDLINE DCOM- 20210310 LR - 20210310 IS - 1445-5994 (Electronic) IS - 1444-0903 (Linking) VI - 50 IP - 4 DP - 2020 Apr TI - Scleroderma in Cairns: an epidemiological study. PG - 445-452 LID - 10.1111/imj.14376 [doi] AB - BACKGROUND: Systemic sclerosis (SSc) refers to an autoimmune fibrosing disorder with high disease burden and mortality. The prevalence of 23/100 000 in South Australia (SA) is among the highest documented, but anecdotally it is higher still in Cairns. AIMS: To ascertain the prevalence of SSc in Cairns and surrounding regions, and to compare the demographic and clinical characteristics of patients with SSc in Cairns with those in SA. METHODS: Patients with SSc in Cairns were ascertained through hospital records and by referrals from specialist physicians in the region. These patients were interviewed and completed a structured questionnaire. Their physical findings and autoantibodies were recorded. These patients were compared with the SA patients enrolled in the Australian Scleroderma Cohort Study. RESULTS: A total of 81 patients was identified in Cairns, giving an estimated cross-sectional prevalence of 33.7/100 000. Among 65 patients interviewed in Cairns, 23 were born in Cairns, 16 had migrated to Cairns to ameliorate their Raynaud phenomenon and 26 for other reasons. The clinical features in both cohorts were similar, although Cairns had a lower prevalence of digital ulcers (30.8% vs 46.6%; odds ratio (OR) = 0.5035, 95% confidence interval (CI): 0.2839-0.8929, P = 0.0271) and higher prevalence of calcinosis (29.2% vs 17.0%; OR = 2.005, 95% CI: 1.055-3.382). CONCLUSIONS: The higher prevalence of SSc in Cairns is partly, but not completely, due to migration. Differences in clinical features are not entirely explained by the warmer climate. There is a need for greater rheumatologic services in the Cairns region. CI - © 2019 Royal Australasian College of Physicians. FAU - Abbot, Samuel AU - Abbot S AUID- ORCID: 0000-0001-8829-862X AD - Cairns Clinical School, James Cook University College of Medicine and Dentistry, Cairns, Queensland, Australia. AD - Department of Medicine, The Queen Elizabeth Hospital, Adelaide, South Australia, Australia. AD - Department of Medicine, Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - McWilliams, Leah AU - McWilliams L AD - Rheumatology Unit, Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - Spargo, Llewellyn AU - Spargo L AD - Rheumatology Unit, Royal Adelaide Hospital, Adelaide, South Australia, Australia. FAU - de Costa, Caroline AU - de Costa C AD - Cairns Clinical School, James Cook University College of Medicine and Dentistry, Cairns, Queensland, Australia. FAU - Ur-Rehman, Zia AU - Ur-Rehman Z AD - Rheumatology Department, Cairns Hospital, Cairns, Queensland, Australia. FAU - Proudman, Susanna AU - Proudman S AD - Rheumatology Unit, Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - Discipline of Medicine, University of Adelaide, Adelaide, South Australia, Australia. FAU - Bossingham, David AU - Bossingham D AD - Cairns Clinical School, James Cook University College of Medicine and Dentistry, Cairns, Queensland, Australia. LA - eng PT - Journal Article PL - Australia TA - Intern Med J JT - Internal medicine journal JID - 101092952 SB - IM MH - Australia/epidemiology MH - Cohort Studies MH - Cross-Sectional Studies MH - Humans MH - Prevalence MH - *Scleroderma, Systemic/diagnosis/epidemiology MH - South Australia/epidemiology OTO - NOTNLM OT - diffuse OT - epidemiology OT - limited OT - prevalence OT - scleroderma OT - systemic sclerosis EDAT- 2019/06/04 06:00 MHDA- 2021/03/11 06:00 CRDT- 2019/06/04 06:00 PHST- 2019/02/07 00:00 [received] PHST- 2019/05/09 00:00 [revised] PHST- 2019/05/26 00:00 [accepted] PHST- 2019/06/04 06:00 [pubmed] PHST- 2021/03/11 06:00 [medline] PHST- 2019/06/04 06:00 [entrez] AID - 10.1111/imj.14376 [doi] PST - ppublish SO - Intern Med J. 2020 Apr;50(4):445-452. doi: 10.1111/imj.14376. PMID- 29151520 OWN - NLM STAT- MEDLINE DCOM- 20180730 LR - 20181202 IS - 1349-7235 (Electronic) IS - 0918-2918 (Print) IS - 0918-2918 (Linking) VI - 57 IP - 6 DP - 2018 Mar 15 TI - Dropped Head Syndrome and the Presence of Rimmed Vacuoles in a Muscle Biopsy in Scleroderma-polymyositis Overlap Syndrome Associated with Anti-Ku Antibody. PG - 887-891 LID - 10.2169/internalmedicine.9363-17 [doi] AB - A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome. The examination of the skin and autoantibody testing help determine the diagnosis and treatment strategy. FAU - Yoshida, Takeshi AU - Yoshida T AD - Department of Internal Medicine, Division of Neurology, Okinawa Chubu Hospital, Japan. FAU - Yoshida, Mai AU - Yoshida M AD - Department of Internal Medicine, Division of Neurology, Okinawa Chubu Hospital, Japan. FAU - Mitsuyo, Kinjo AU - Mitsuyo K AD - Department of Internal Medicine, Division of Rheumatology, Okinawa Chubu Hospital, Japan. FAU - Jonosono, Manabu AU - Jonosono M AD - Department of Internal Medicine, Division of Neurology, Okinawa Chubu Hospital, Japan. FAU - Higuchi, Itsuro AU - Higuchi I AD - School of Health Sciences, Faculty of Medicine, Kagoshima University, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20171120 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - 9PHQ9Y1OLM (Prednisolone) RN - EC 4.2.99.- (Ku Autoantigen) RN - MRK240IY2L (Azathioprine) SB - IM MH - Aged MH - Anti-Inflammatory Agents/therapeutic use MH - Autoantibodies/immunology MH - Azathioprine/*therapeutic use MH - Female MH - Head Movements MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Inclusion Bodies/ultrastructure MH - Ku Autoantigen MH - Lung Diseases, Interstitial/*complications MH - Muscle Weakness/*complications/diagnosis/*drug therapy/physiopathology MH - Myositis, Inclusion Body/*complications/diagnosis/*drug therapy/immunology MH - Neck Muscles/physiopathology MH - Orthopedic Procedures MH - Prednisolone/*therapeutic use MH - Syndrome MH - Treatment Outcome MH - Vacuoles/ultrastructure PMC - PMC5891533 OTO - NOTNLM OT - anti-Ku antibody OT - dropped head syndrome OT - rimmed vacuole OT - scleroderma-polymyositis overlap syndrome EDAT- 2017/11/21 06:00 MHDA- 2018/07/31 06:00 PMCR- 2018/03/15 CRDT- 2017/11/21 06:00 PHST- 2017/11/21 06:00 [pubmed] PHST- 2018/07/31 06:00 [medline] PHST- 2017/11/21 06:00 [entrez] PHST- 2018/03/15 00:00 [pmc-release] AID - 10.2169/internalmedicine.9363-17 [doi] PST - ppublish SO - Intern Med. 2018 Mar 15;57(6):887-891. doi: 10.2169/internalmedicine.9363-17. Epub 2017 Nov 20. PMID- 21916221 OWN - NLM STAT- MEDLINE DCOM- 20111018 LR - 20151029 IS - 0030-6657 (Print) IS - 0030-6657 (Linking) VI - 65 IP - 3 DP - 2011 May-Jun TI - [Vestibular system in patients with systemic sclerosis]. PG - 202-7 LID - 10.1016/S0030-6657(11)70676-X [doi] AB - OBJECTIVES: In systemic sclerosis (SSc) there may occur hearing and balance disorders as a result of the immune-mediated inner ear damage, the etiology being vasculitis and fibrosis. The objective is the vestibular organ evaluation in patients with SSc regarding their prevalence and relationship to duration of the disease and Raynaud phenomenon and also to type and severity of SSc. MATERIAL: Twenty unselected, consecutive patients with diagnosed SSc, complying with international diagnostic criteria of the American Rheumatism Association (1982), were enrolled into the study. The control group consisted of 26 otologically healthy persons matched to the SSc group for age and sex. METHODS: In all patients the questionnaire about audiovestibular history, otolaryngological examination, static and dynamic vestibular tests and the electronystsgmography (ENG) were performed. The patients were investigated with the electronystsgmography (ENG) for spontaneous, positional and caloric-induced nystagmus. Also visual ocular-motor tests were performer. RESULTS: In the anamnesis 65% of patients reported vertigo, 55% - headaches, 50% - tinnitus, 40% - hyperacusis, 40% - hearing loss and 30% - ear fullness. Vertigo, dizziness balance disturbance and uncorrect results of Romberg and Utenberger tests were more frequent in patients with vestibular organ lesion. Abnormalities in vestibular organ in SSc patients were fund in 14 (70%) persons - the central type of lesion - 8 (40%), mixed type in 3 (15%) and peripheral in 3 (15%). CONCLUSIONS: Ear involvement is frequent in systemic sclerosis and should be taken into consideration during diagnostic and therapeutic procedures. FAU - Maciaszczyk, Katarzyna AU - Maciaszczyk K AD - Uniwersytecki Szpital Kliniczny nr 1 im. N. Barlickiego, I Katedra Otolaryngologii Uniwersytetu Medycznego w Łodzi. k.maciaszczyk@op.pl FAU - Durko, Tomasz AU - Durko T FAU - Waszczykowska, Elzbieta AU - Waszczykowska E FAU - Bartkowiak-Dziankowska, Bozena AU - Bartkowiak-Dziankowska B FAU - Pajor, Anna AU - Pajor A FAU - Erkiert-Polguj, Anna AU - Erkiert-Polguj A FAU - Józefowicz-Korczyńska, Magdalena AU - Józefowicz-Korczyńska M LA - pol PT - English Abstract PT - Journal Article TT - Stan układu przedsionkowego u pacjentów z twardzina układowa. PL - Poland TA - Otolaryngol Pol JT - Otolaryngologia polska = The Polish otolaryngology JID - 0404453 SB - IM MH - Adult MH - Audiometry, Pure-Tone MH - Case-Control Studies MH - Dizziness/diagnosis/etiology MH - Electronystagmography MH - Female MH - Headache/diagnosis/etiology MH - Humans MH - Male MH - Middle Aged MH - Reference Values MH - *Reflex, Vestibulo-Ocular MH - Scleroderma, Systemic/*complications/*physiopathology MH - Tinnitus/diagnosis/etiology MH - Vertigo/diagnosis/etiology MH - Vestibule, Labyrinth/*physiopathology EDAT- 2011/09/16 06:00 MHDA- 2011/10/19 06:00 CRDT- 2011/09/16 06:00 PHST- 2011/09/16 06:00 [entrez] PHST- 2011/09/16 06:00 [pubmed] PHST- 2011/10/19 06:00 [medline] AID - S0030-6657(11)70676-X [pii] AID - 10.1016/S0030-6657(11)70676-X [doi] PST - ppublish SO - Otolaryngol Pol. 2011 May-Jun;65(3):202-7. doi: 10.1016/S0030-6657(11)70676-X. PMID- 27608975 OWN - NLM STAT- MEDLINE DCOM- 20171020 LR - 20181113 IS - 1479-5876 (Electronic) IS - 1479-5876 (Linking) VI - 14 IP - 1 DP - 2016 Sep 8 TI - Salivary psoriasin (S100A7) correlates with diffusion capacity of carbon monoxide in a large cohort of systemic sclerosis patients. PG - 262 LID - 10.1186/s12967-016-1023-5 [doi] LID - 262 AB - BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease characterized by progressive fibrosis of the skin and the internal organs. In a previous work we suggested a correlation between levels of salivary psoriasin (S100A7) and pulmonary involvement in SSc patients. The goals of this study are to determine the distribution characteristics of psoriasin in whole saliva (WS) of SSc and healthy donor populations and define its predictive value on diffusion capacity of carbon monoxide (DLCO), along with others clinical parameters. METHODS: Salivary level of psoriasin was determined by ELISA kit in 134 SSc patients, 63 Raynaud syndrome patients, 40 patients affected by other connective diseases (non-case) and 74 healthy control subjects. RESULTS: A significant increase of salivary psoriasin was observed in SSc patients when compared with other healthy and pathological controls. Moreover, we confirmed the efficacy of salivary psoriasin to correlate with DLCO in a large cohort of SSc patients. CONCLUSIONS: Overall our results suggest a rapid, non invasive and low costing method which can help clinicians in the evaluation of SSc pulmonary involvement. FAU - Giusti, Laura AU - Giusti L AUID- ORCID: 0000-0001-7366-1932 AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. laura.giusti@unipi.it. FAU - Sernissi, Francesca AU - Sernissi F AD - Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy. FAU - Donadio, Elena AU - Donadio E AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. FAU - Ciregia, Federica AU - Ciregia F AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. FAU - Giacomelli, Camillo AU - Giacomelli C AD - Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy. FAU - Giannaccini, Gino AU - Giannaccini G AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. FAU - Mazzoni, Maria Rosa AU - Mazzoni MR AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. FAU - Lucacchini, Antonio AU - Lucacchini A AD - Department of Pharmacy, University of Pisa, Via Bonanno 6, 56126, Pisa, Italy. FAU - Bazzichi, Laura AU - Bazzichi L AD - Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy. LA - eng PT - Journal Article DEP - 20160908 PL - England TA - J Transl Med JT - Journal of translational medicine JID - 101190741 RN - 0 (S100 Calcium Binding Protein A7) RN - 0 (S100 Proteins) RN - 0 (S100A7 protein, human) RN - 7U1EE4V452 (Carbon Monoxide) SB - IM MH - Carbon Monoxide/*metabolism MH - Case-Control Studies MH - Cohort Studies MH - Diffusion MH - Female MH - Humans MH - Male MH - Middle Aged MH - ROC Curve MH - S100 Calcium Binding Protein A7 MH - S100 Proteins/*metabolism MH - Saliva/*metabolism MH - Scleroderma, Systemic/*metabolism PMC - PMC5015208 OTO - NOTNLM OT - Biomarker OT - Diffusion capacity of carbon monoxide (DLCO) OT - Psoriasin OT - S100A7 OT - Systemic sclerosis (SSc) OT - Whole saliva EDAT- 2016/09/14 06:00 MHDA- 2017/10/21 06:00 PMCR- 2016/09/08 CRDT- 2016/09/10 06:00 PHST- 2016/05/31 00:00 [received] PHST- 2016/08/29 00:00 [accepted] PHST- 2016/09/10 06:00 [entrez] PHST- 2016/09/14 06:00 [pubmed] PHST- 2017/10/21 06:00 [medline] PHST- 2016/09/08 00:00 [pmc-release] AID - 10.1186/s12967-016-1023-5 [pii] AID - 1023 [pii] AID - 10.1186/s12967-016-1023-5 [doi] PST - epublish SO - J Transl Med. 2016 Sep 8;14(1):262. doi: 10.1186/s12967-016-1023-5. PMID- 19449187 OWN - NLM STAT- MEDLINE DCOM- 20100325 LR - 20211020 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 30 IP - 4 DP - 2010 Feb TI - A case of SLE with bilateral osteonecrosis of femoral heads and bone infarct in distal of femur. PG - 527-9 LID - 10.1007/s00296-009-0956-x [doi] AB - Osteonecrosis of bone is a major cause of morbidity in lupus patients, and is most common in the femoral head. It has been reported in wide range of patients (2-30%). In different studies presence of arthritis, Raynaud phenomenon, vasculitis, pleuritis, antiphospholipid and other factors were associated with this occurrence. Bone infarcts were also associated with these factors. We report a 21-year-old patient who was diagnosed as SLE about 3 years ago. When the patient was stable with hydroxychloroquine and prednisolone referred to rheumatologic clinic for mechanical knee pain, in evaluation she had bone infarct in distal femur. Two months later she came back with bilateral hip pain, and in evaluation she had bilateral osteonecrosis of femoral heads. There are many reports of femoral head osteonecrosis in lupus patients, and also one report of multiple bone infarct and pain in SLE, but we did not find any report of these two phenomena together in a patient whose disease was controlled and she took minimum of steroid and DMARD in the about 2-month follow-up, and this was very interesting for us. FAU - Salesi, Mansour AU - Salesi M AD - Rheumatology Ward, Alzahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran. salesi@med.mui.ac.ir FAU - Karimifar, Mansoor AU - Karimifar M FAU - Mottaghi, Peyman AU - Mottaghi P FAU - Sayedbonakdar, Zahra AU - Sayedbonakdar Z FAU - Karimzadeh, Hadi AU - Karimzadeh H LA - eng PT - Case Reports PT - Journal Article DEP - 20090518 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antirheumatic Agents) RN - 4QWG6N8QKH (Hydroxychloroquine) RN - 9PHQ9Y1OLM (Prednisolone) SB - IM MH - Antirheumatic Agents/therapeutic use MH - Drug Therapy, Combination MH - Female MH - Femur/diagnostic imaging/*pathology MH - Femur Head Necrosis/complications/diagnostic imaging/*pathology MH - Humans MH - Hydroxychloroquine/therapeutic use MH - Infarction/complications/diagnostic imaging/*pathology MH - Lupus Erythematosus, Systemic/complications/drug therapy/*pathology MH - Magnetic Resonance Imaging MH - Prednisolone/therapeutic use MH - Radiography MH - Young Adult EDAT- 2009/05/19 09:00 MHDA- 2010/03/26 06:00 CRDT- 2009/05/19 09:00 PHST- 2009/01/06 00:00 [received] PHST- 2009/04/28 00:00 [accepted] PHST- 2009/05/19 09:00 [entrez] PHST- 2009/05/19 09:00 [pubmed] PHST- 2010/03/26 06:00 [medline] AID - 10.1007/s00296-009-0956-x [doi] PST - ppublish SO - Rheumatol Int. 2010 Feb;30(4):527-9. doi: 10.1007/s00296-009-0956-x. Epub 2009 May 18. PMID- 34310228 OWN - NLM STAT- MEDLINE DCOM- 20210924 LR - 20210924 IS - 1527-1315 (Electronic) IS - 0033-8419 (Linking) VI - 300 IP - 2 DP - 2021 Aug TI - Case 292: Lyme Neuroborreliosis. PG - 484-488 LID - 10.1148/radiol.2021201715 [doi] AB - History A 24-year-old right-handed woman presented to a neuro-ophthalmology clinic in Massachusetts in the summer with acute binocular diplopia when looking down and to the left, which started about 1 month earlier. Her medical history was notable for Raynaud syndrome, recurrent streptococcal pharyngitis, and an allergy to amoxicillin. Three days prior to developing diplopia, she presented to an outside emergency department due to fever, chills, and back pain. She received ciprofloxacin for presumed urinary tract infection based on urinalysis, which demonstrated few bacteria and was negative for leukocyte esterase, nitrites, and white blood cells. She then presented again to an outside emergency department for diplopia evaluation. Initial MRI and MR angiography of the brain at that time did not demonstrate any relevant findings, and the patient was referred to our department for neuro-ophthalmic evaluation, where she was seen 4 weeks later. Neuro-ophthalmic examination revealed 20/20 visual acuity in both eyes, and a right hypertropia in left gaze, downgaze and right head tilt, with right eye excyclotorsion. There were no ocular signs of myasthenia gravis or thyroid eye disease, nor did the patient report ocular or systemic symptoms. She denied recent travel. High-spatial-resolution MRI of the brain and orbit were performed. FAU - Douglas, Vivian Paraskevi AU - Douglas VP AUID- ORCID: 0000-0002-5300-5468 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Douglas, Konstantinos A A AU - Douglas KAA AUID- ORCID: 0000-0003-2972-2691 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Reinshagen, Katherine L AU - Reinshagen KL AUID- ORCID: 0000-0003-3705-1082 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). FAU - Chwalisz, Bart K AU - Chwalisz BK AUID- ORCID: 0000-0001-9918-1742 AD - From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.). LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Radiology JT - Radiology JID - 0401260 RN - 0 (Anti-Bacterial Agents) RN - N12000U13O (Doxycycline) SB - IM MH - Anti-Bacterial Agents/therapeutic use MH - Diagnosis, Differential MH - Doxycycline/therapeutic use MH - Female MH - Humans MH - Lyme Neuroborreliosis/*diagnostic imaging/drug therapy MH - Magnetic Resonance Imaging/*methods MH - Young Adult EDAT- 2021/07/27 06:00 MHDA- 2021/09/25 06:00 CRDT- 2021/07/26 17:17 PHST- 2021/07/26 17:17 [entrez] PHST- 2021/07/27 06:00 [pubmed] PHST- 2021/09/25 06:00 [medline] AID - 10.1148/radiol.2021201715 [doi] PST - ppublish SO - Radiology. 2021 Aug;300(2):484-488. doi: 10.1148/radiol.2021201715. PMID- 25894644 OWN - NLM STAT- MEDLINE DCOM- 20160707 LR - 20161018 IS - 1437-4331 (Electronic) IS - 1434-6621 (Linking) VI - 53 IP - 11 DP - 2015 Oct TI - Increased plasma soluble urokinase plasminogen activator receptor levels in systemic sclerosis: possible association with microvascular abnormalities and extent of fibrosis. PG - 1799-805 LID - 10.1515/cclm-2015-0079 [doi] AB - BACKGROUND: Urokinase plasminogen activator receptor (uPAR) is a key component of the fibrinolytic system involved in extracellular matrix remodeling and angiogenesis. Novel animal models supported the key role of uPAR not only in fibrosis but also in systemic sclerosis (SSc)-related microvascular abnormalities. The aim of this study was to investigate plasma soluble uPAR (suPAR) levels in SSc, and their association with organ-specific involvement. METHODS: suPAR concentrations were measured by ELISA in SSc patient (n=83) and in healthy controls (n=29). Simultaneously, CRP and ESR were assessed. Detailed clinical data including skin, lung, heart and microvascular characteristics were evaluated at sampling. RESULTS: suPAR values were higher in SSc patients than in controls. Subgroup analysis showed higher suPAR values in diffuse cutaneous- than in limited cutaneous SSc and correlated with anti-Scl-70+. suPAR levels also associated with pulmonary function test parameters of fibrosis, presence of microvascular lesions (e.g., Raynaud phenomenon, naifold capillaroscopic abnormalities and digital ulcers) and arthritis. CONCLUSIONS: Our data indicate that suPAR might be a valuable early diagnostic marker of SSc which also correlates with disease severity. FAU - Legány, Nóra AU - Legány N FAU - Toldi, Gergely AU - Toldi G FAU - Distler, Jörg H W AU - Distler JH FAU - Beyer, Christian AU - Beyer C FAU - Szalay, Balázs AU - Szalay B FAU - Kovács, László AU - Kovács L FAU - Vásárhelyi, Barna AU - Vásárhelyi B FAU - Balog, Attila AU - Balog A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Germany TA - Clin Chem Lab Med JT - Clinical chemistry and laboratory medicine JID - 9806306 RN - 0 (Receptors, Urokinase Plasminogen Activator) SB - IM MH - Adult MH - Aged MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Fibrosis MH - Humans MH - Male MH - Microvessels/*abnormalities MH - Middle Aged MH - Receptors, Urokinase Plasminogen Activator/*blood/deficiency/genetics MH - Scleroderma, Systemic/*blood/complications/*pathology MH - Solubility EDAT- 2015/04/22 06:00 MHDA- 2016/07/09 06:00 CRDT- 2015/04/21 06:00 PHST- 2015/01/22 00:00 [received] PHST- 2015/03/05 00:00 [accepted] PHST- 2015/04/21 06:00 [entrez] PHST- 2015/04/22 06:00 [pubmed] PHST- 2016/07/09 06:00 [medline] AID - /j/cclm.ahead-of-print/cclm-2015-0079/cclm-2015-0079.xml [pii] AID - 10.1515/cclm-2015-0079 [doi] PST - ppublish SO - Clin Chem Lab Med. 2015 Oct;53(11):1799-805. doi: 10.1515/cclm-2015-0079. PMID- 17916985 OWN - NLM STAT- MEDLINE DCOM- 20080411 LR - 20211020 IS - 1080-0549 (Print) IS - 1080-0549 (Linking) VI - 32 IP - 2 DP - 2007 Apr TI - Pulmonary hypertension, antiphospholipid antibodies, and syndromes. PG - 153-8 AB - Antiphospholipid antibodies have been associated with two types of pulmonary hypertension (PHT), the thromboembolic type, after deep venous thromboses in the lower limbs complicated by pulmonary embolism and the "primary" plexogenic type. The PHT may occur in the absence of any other manifestations of the antiphospholipid syndrome (APS), and cases have been recorded with very high levels of antiphospholipid antibodies. It may also accompany systemic lupus erythematosus (SLE) and may manifest with or without other features of the APS. It may also form part of the clinical presentation of a "primary" APS. Its prevalence is of the order of 1.8-3.5% of the manifestations of the APS depending on the series. Primary "idiopathic" PHT has long been regarded as an "immunological" disorder. Its manifestations are essentially to the primary type seen with the connective tissue disorders such as SLE, APS, mixed connective tissue disease, calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia variety of systemic sclerosis and Sjögren's syndrome. The high prevalence of PHT in patients with human immunodeficiency virus infection who demonstrate low CD4 counts points to a close relationship between the T regulatory cells (Treg) and the development of PHT, and this hypothesis is discussed in this review. Genetic and chromosomal aspects of PHT are also discussed. FAU - Asherson, Ronald A AU - Asherson RA AD - Division of Immunology, School of Pathology, University of the Witwatersrand and the Rosebank Clinic, Johannesburg, 2000, South Africa. ashron@icon.co.za FAU - Cervera, Ricard AU - Cervera R LA - eng PT - Journal Article PT - Review PL - United States TA - Clin Rev Allergy Immunol JT - Clinical reviews in allergy & immunology JID - 9504368 RN - 0 (Antibodies, Antiphospholipid) RN - 0 (CD4 Antigens) SB - IM MH - Antibodies, Antiphospholipid/*immunology MH - CD4 Antigens MH - CD4-Positive T-Lymphocytes/immunology MH - Connective Tissue Diseases/immunology/physiopathology MH - Female MH - Humans MH - Hypertension, Pulmonary/genetics/*immunology/physiopathology MH - Immune System Diseases/etiology/physiopathology MH - Lupus Erythematosus, Systemic/physiopathology MH - Pregnancy MH - Prevalence MH - Pulmonary Embolism/immunology/physiopathology MH - Sjogren's Syndrome/immunology/physiopathology MH - Syndrome MH - Thrombosis/immunology/physiopathology RF - 47 EDAT- 2007/10/06 09:00 MHDA- 2008/04/12 09:00 CRDT- 2007/10/06 09:00 PHST- 1999/11/30 00:00 [received] PHST- 1999/11/30 00:00 [revised] PHST- 1999/11/30 00:00 [accepted] PHST- 2007/10/06 09:00 [pubmed] PHST- 2008/04/12 09:00 [medline] PHST- 2007/10/06 09:00 [entrez] AID - CRIAI:32:2:153 [pii] AID - 10.1007/s12016-007-0012-0 [doi] PST - ppublish SO - Clin Rev Allergy Immunol. 2007 Apr;32(2):153-8. doi: 10.1007/s12016-007-0012-0. PMID- 34983928 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220510 IS - 2041-1723 (Electronic) IS - 2041-1723 (Linking) VI - 13 IP - 1 DP - 2022 Jan 4 TI - Author Correction: Genome-wide association study identifies susceptibility loci for acute myeloid leukemia. PG - 2 LID - 10.1038/s41467-021-27679-6 [doi] LID - 2 FAU - Lin, Wei-Yu AU - Lin WY AUID- ORCID: 0000-0002-9267-7988 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Fordham, Sarah E AU - Fordham SE AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Hungate, Eric AU - Hungate E AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Sunter, Nicola J AU - Sunter NJ AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Elstob, Claire AU - Elstob C AUID- ORCID: 0000-0003-4252-493X AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Xu, Yaobo AU - Xu Y AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Park, Catherine AU - Park C AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Quante, Anne AU - Quante A AD - Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany. AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Strauch, Konstantin AU - Strauch K AD - Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany. AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Gieger, Christian AU - Gieger C AUID- ORCID: 0000-0001-6986-9554 AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Skol, Andrew AU - Skol A AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Rahman, Thahira AU - Rahman T AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Sucheston-Campbell, Lara AU - Sucheston-Campbell L AD - College of Pharmacy, The Ohio State University, Columbus, OH, USA. FAU - Wang, Junke AU - Wang J AD - College of Pharmacy, The Ohio State University, Columbus, OH, USA. FAU - Hahn, Theresa AU - Hahn T AUID- ORCID: 0000-0002-3835-8855 AD - Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USA. FAU - Clay-Gilmour, Alyssa I AU - Clay-Gilmour AI AD - Arnold School of Public Health, Department of Epidemiology & Biostatistics, University of South Carolina, Greenville, SC, USA. FAU - Jones, Gail L AU - Jones GL AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Marr, Helen J AU - Marr HJ AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Jackson, Graham H AU - Jackson GH AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Menne, Tobias AU - Menne T AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Collin, Mathew AU - Collin M AUID- ORCID: 0000-0001-6585-9586 AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Ivey, Adam AU - Ivey A AD - Department of Medical and Molecular Genetics, King's College Medical School, London, UK. FAU - Hills, Robert K AU - Hills RK AD - Nuffield Department of Population Health, University of Oxford, Oxford, UK. FAU - Burnett, Alan K AU - Burnett AK AUID- ORCID: 0000-0003-1734-5817 AD - Paul O'Gorman Leukaemia Research Centre, University of Glasgow, Glasgow, UK. FAU - Russell, Nigel H AU - Russell NH AD - Department of Haematology, Nottingham University Hospitals NHS Trust, Nottingham, UK. FAU - Fitzgibbon, Jude AU - Fitzgibbon J AUID- ORCID: 0000-0002-9069-1866 AD - Barts Cancer Institute, Queen Mary University of London, London, UK. FAU - Larson, Richard A AU - Larson RA AUID- ORCID: 0000-0001-9168-3203 AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Le Beau, Michelle M AU - Le Beau MM AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Stock, Wendy AU - Stock W AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Heidenreich, Olaf AU - Heidenreich O AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Alharbi, Abrar AU - Alharbi A AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Allsup, David J AU - Allsup DJ AUID- ORCID: 0000-0001-6159-6109 AD - Centre for Atherothrombosis and Metabolic Disease, Hull York Medical School, Hull, UK. FAU - Houlston, Richard S AU - Houlston RS AUID- ORCID: 0000-0002-5268-0242 AD - Division of Genetics and Epidemiology, The Institute of Cancer Research, London, UK. FAU - Norden, Jean AU - Norden J AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Dickinson, Anne M AU - Dickinson AM AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Douglas, Elisabeth AU - Douglas E AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Lendrem, Clare AU - Lendrem C AUID- ORCID: 0000-0002-9435-7398 AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Daly, Ann K AU - Daly AK AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Palm, Louise AU - Palm L AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Piechocki, Kim AU - Piechocki K AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Jeffries, Sally AU - Jeffries S AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Bornhäuser, Martin AU - Bornhäuser M AD - Department of Haematological Medicine, The Rayne Institute, King's College London, London, UK. AD - National Center for Tumor Diseases NCT, Partner site Dresden, Dresden, Germany. AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Röllig, Christoph AU - Röllig C AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Altmann, Heidi AU - Altmann H AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Ruhnke, Leo AU - Ruhnke L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Kunadt, Desiree AU - Kunadt D AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Wagenführ, Lisa AU - Wagenführ L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Cordell, Heather J AU - Cordell HJ AUID- ORCID: 0000-0002-1879-5572 AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK. FAU - Darlay, Rebecca AU - Darlay R AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK. FAU - Andersen, Mette K AU - Andersen MK AD - Department of Clinical Genetics, University Hospital Rigshospitalet, Copenhagen, Denmark. FAU - Fontana, Maria C AU - Fontana MC AD - Institute of Hematology "L. and A. Seràgnoli", University of Bologna, Bologna, Italy. AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Martinelli, Giovanni AU - Martinelli G AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Marconi, Giovanni AU - Marconi G AUID- ORCID: 0000-0001-6309-0515 AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Sanz, Miguel A AU - Sanz MA AUID- ORCID: 0000-0003-1489-1177 AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cervera, José AU - Cervera J AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Gómez-Seguí, Inés AU - Gómez-Seguí I AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cluzeau, Thomas AU - Cluzeau T AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Moreilhon, Chimène AU - Moreilhon C AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Sill, Heinz AU - Sill H AUID- ORCID: 0000-0003-0993-4371 AD - Division of Hematology, Medical University of Graz, Graz, Austria. FAU - Voso, Maria Teresa AU - Voso MT AUID- ORCID: 0000-0002-6164-4761 AD - Università di Roma Tor Vergata, Dipartimento di Biomedicina e Prevenzione, Rome, Italy. FAU - Lo-Coco, Francesco AU - Lo-Coco F AD - Università di Roma Tor Vergata, Dipartimento di Biomedicina e Prevenzione, Rome, Italy. FAU - Dombret, Hervé AU - Dombret H AD - Hôpital Saint-Louis, Institut Universitaire d'Hématologie, Université Paris Diderot, Paris, France. FAU - Cheok, Meyling AU - Cheok M AUID- ORCID: 0000-0002-7820-8026 AD - Univ. Lille, Inserm, CHU Lille, UMR-S 1172 - JPArc - Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer, F-59000, Lille, France. FAU - Preudhomme, Claude AU - Preudhomme C AD - Univ. Lille, Inserm, CHU Lille, UMR-S 1172 - JPArc - Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer, F-59000, Lille, France. FAU - Gale, Rosemary E AU - Gale RE AD - Department of Haematology, University College London Cancer Institute, London, UK. FAU - Linch, David AU - Linch D AD - Department of Haematology, University College London Cancer Institute, London, UK. FAU - Gaal-Wesinger, Julia AU - Gaal-Wesinger J AD - 1st Department of Internal Medicine, Semmewleis University, Budapest, Hungary. FAU - Masszi, Andras AU - Masszi A AD - 3rd Department of Internal Medicine, Semmewleis University, Budapest, Hungary. FAU - Nowak, Daniel AU - Nowak D AUID- ORCID: 0000-0001-7130-7921 AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Hofmann, Wolf-Karsten AU - Hofmann WK AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Gilkes, Amanda AU - Gilkes A AD - Department of Haematology, University of Cardiff, Cardiff, UK. FAU - Porkka, Kimmo AU - Porkka K AD - Helsinki University Hospital Comprehensive Cancer Center, Hematology Research Unit Helsinki, University of Helsinki, Helsinki, Finland. FAU - Milosevic Feenstra, Jelena D AU - Milosevic Feenstra JD AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Kralovics, Robert AU - Kralovics R AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Grimwade, David AU - Grimwade D AD - Department of Medical and Molecular Genetics, King's College Medical School, London, UK. FAU - Meggendorfer, Manja AU - Meggendorfer M AD - MLL Munich Leukemia Laboratory, Munich, Germany. FAU - Haferlach, Torsten AU - Haferlach T AD - MLL Munich Leukemia Laboratory, Munich, Germany. FAU - Krizsán, Szilvia AU - Krizsán S AUID- ORCID: 0000-0002-1782-9275 AD - HCEMM-SE Molecular Oncohematology Research Group, 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. FAU - Bödör, Csaba AU - Bödör C AUID- ORCID: 0000-0002-0729-692X AD - HCEMM-SE Molecular Oncohematology Research Group, 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. FAU - Stölzel, Friedrich AU - Stölzel F AUID- ORCID: 0000-0003-0653-5332 AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. friedrich.stoelzel@uniklinikum-dresden.de. FAU - Onel, Kenan AU - Onel K AUID- ORCID: 0000-0002-2021-6250 AD - Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA. kenan.onel@mssm.edu. FAU - Allan, James M AU - Allan JM AUID- ORCID: 0000-0002-7580-5087 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. james.allan@newcastle.ac.uk. LA - eng GR - R01 HL102278/HL/NHLBI NIH HHS/United States GR - R03 CA188733/CA/NCI NIH HHS/United States PT - Published Erratum DEP - 20220104 PL - England TA - Nat Commun JT - Nature communications JID - 101528555 SB - IM EFR - Nat Commun. 2021 Oct 29;12(1):6233. doi: 10.1038/s41467-021-26551-x. PMID: 34716350 PMC - PMC8727612 EDAT- 2022/01/06 06:00 MHDA- 2022/01/06 06:01 PMCR- 2022/01/04 CRDT- 2022/01/05 05:51 PHST- 2022/01/05 05:51 [entrez] PHST- 2022/01/06 06:00 [pubmed] PHST- 2022/01/06 06:01 [medline] PHST- 2022/01/04 00:00 [pmc-release] AID - 10.1038/s41467-021-27679-6 [pii] AID - 27679 [pii] AID - 10.1038/s41467-021-27679-6 [doi] PST - epublish SO - Nat Commun. 2022 Jan 4;13(1):2. doi: 10.1038/s41467-021-27679-6. PMID- 36637943 OWN - NLM STAT- MEDLINE DCOM- 20230117 LR - 20250503 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 102 IP - 2 DP - 2023 Jan 13 TI - Critical digital ischemia and biliary cholangitis related to graft versus host disease: A case report and systematic literature review. PG - e32495 LID - 10.1097/MD.0000000000032495 [doi] LID - e32495 AB - RATIONALE: Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis. PATIENT CONCERNS: A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern. DIAGNOSIS: Biliary cholangitis-like and digital ischemia related to cGVHD. INTERVENTIONS: The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome. OUTCOMES: After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function. LESSONS: cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association. CI - Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Hidalgo Calleja, Cristina AU - Hidalgo Calleja C AUID- ORCID: 0000-0001-5505-7539 AD - Rheumatology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain. FAU - Montilla Morales, Carlos Alberto AU - Montilla Morales CA AD - Rheumatology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain. FAU - Sánchez González, María Dolores AU - Sánchez González MD AD - Rheumatology Department, Hospital Clínico Universitario de Valladolid, Spain. FAU - Pastor Navarro, Sonia AU - Pastor Navarro S AD - Rheumatology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain. FAU - Ibáñez Martínez, Marta AU - Ibáñez Martínez M AD - Rheumatology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain. FAU - Conde Ferreiros, Alberto AU - Conde Ferreiros A AD - Dermatology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain. FAU - López Corral, Lucía AU - López Corral L AD - Haematology Department, Complejo Asistencial Universitario de Salamanca-IBSAL, Centro de Investigación del Cáncer-IBMCC, Salamanca, Spain. LA - eng PT - Case Reports PT - Journal Article PT - Systematic Review PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Humans MH - Female MH - Middle Aged MH - Transplantation, Homologous/adverse effects MH - *Hematopoietic Stem Cell Transplantation/adverse effects MH - *Graft vs Host Disease/complications/diagnosis MH - *Cholangitis/complications MH - *Bronchiolitis Obliterans Syndrome MH - *Scleroderma, Systemic/complications/therapy MH - Ischemia/complications MH - Chronic Disease PMC - PMC9839287 COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2023/01/14 06:00 MHDA- 2023/01/18 06:00 PMCR- 2023/01/13 CRDT- 2023/01/13 12:03 PHST- 2023/01/13 12:03 [entrez] PHST- 2023/01/14 06:00 [pubmed] PHST- 2023/01/18 06:00 [medline] PHST- 2023/01/13 00:00 [pmc-release] AID - 00005792-202301130-00028 [pii] AID - 10.1097/MD.0000000000032495 [doi] PST - ppublish SO - Medicine (Baltimore). 2023 Jan 13;102(2):e32495. doi: 10.1097/MD.0000000000032495. PMID- 38438774 OWN - NLM STAT- MEDLINE DCOM- 20240419 LR - 20240605 IS - 1573-2568 (Electronic) IS - 0163-2116 (Linking) VI - 69 IP - 4 DP - 2024 Apr TI - Insulin Resistance/Sensitivity Measures as Screening Indicators of Metabolic-Associated Fatty Liver Disease and Liver Fibrosis. PG - 1430-1443 LID - 10.1007/s10620-024-08309-9 [doi] AB - BACKGROUND: Measures of insulin resistance (IR)/sensitivity (IS) are emerging tools to identify metabolic-associated fatty liver disease (MAFLD). However, the comprehensive assessment of the performance of various indicators is limited. Moreover, the utility of measures of IR/IS in detecting liver fibrosis remains unclear. AIMS: To evaluate the predictive ability of seventeen IR/IS and two beta cell function indices to identify MAFLD and liver fibrosis. METHODS: A cross-sectional study was conducted on individuals aged 25-75 years. Transient elastography was used to estimate liver stiffness and controlled attenuation parameter. The following measures were computed: homeostatic model assessment (HOMA/HOMA2) for IR, IS, and beta cell function; QUICKI; Bennett index; glucose/insulin; FIRI; McAuley index; Reynaud index; SPISE index; TyG; TyG-BMI; TyG-WC; TyG-WHtR; TG/HDL; and METS-IR. Subgroup analyses were performed according to age, gender, diabetes status, and body weight. RESULTS: A total of 644 individuals were included in our analysis. MAFLD and significant liver fibrosis were detected in 320 (49.7%) and 80 (12.4%) of the participants, respectively. All measures of IR/IS identified MAFLD and liver fibrosis. However, TyG-WC, TyG-BMI, and TyG-WHtR were the top three indicators that identified MAFLD. Measures that include insulin level in their mathematical calculation, namely, Raynaud index, HOMA-IR, HOMA 2-IR, FIRI, and QUICKI had the best performance in identifying liver fibrosis in the entire population, as well as among the study subgroups. CONCLUSIONS: TyG-WC, TyG-BMI, and TyG-WHtR were the best predictors of MAFLD. Insulin-based measures had better performances in the detection of advanced fibrosis. This was independent of age, gender, obesity, or diabetes status. CI - © 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. FAU - Khamseh, Mohammad E AU - Khamseh ME AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Malek, Mojtaba AU - Malek M AD - Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Jahangiri, Soodeh AU - Jahangiri S AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Nobarani, Sohrab AU - Nobarani S AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Hekmatdoost, Azita AU - Hekmatdoost A AD - Department of Clinical Nutrition and Dietetics, Faculty of Nutrition and Food Technology, Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Salavatizadeh, Marieh AU - Salavatizadeh M AD - Department of Clinical Nutrition and Dietetics, Faculty of Nutrition and Food Technology, Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Soltanieh, Samira AU - Soltanieh S AD - Department of Clinical Nutrition and Dietetics, Faculty of Nutrition and Food Technology, Shahid Beheshti University of Medical Sciences, Tehran, Iran. FAU - Chehrehgosha, Haleh AU - Chehrehgosha H AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Taheri, Hoda AU - Taheri H AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Montazeri, Zeinab AU - Montazeri Z AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Attaran, Fereshteh AU - Attaran F AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. FAU - Ismail-Beigi, Faramarz AU - Ismail-Beigi F AD - Department of Medicine, Case Western Reserve University, University Hospitals Cleveland Medical Center, Cleveland, OH, 44106, USA. FAU - Alaei-Shahmiri, Fariba AU - Alaei-Shahmiri F AUID- ORCID: 0000-0002-6401-8803 AD - Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran. alaeishahmiri.f@iums.ac.ir. LA - eng PT - Journal Article DEP - 20240304 PL - United States TA - Dig Dis Sci JT - Digestive diseases and sciences JID - 7902782 RN - 0 (Biomarkers) RN - 0 (Blood Glucose) RN - 0 (Triglycerides) RN - 0 (Insulin) RN - IY9XDZ35W2 (Glucose) SB - IM MH - Humans MH - *Insulin Resistance/physiology MH - Cross-Sectional Studies MH - Biomarkers MH - Blood Glucose MH - Triglycerides MH - Insulin MH - Liver Cirrhosis MH - *Liver Diseases MH - *Diabetes Mellitus MH - Glucose OTO - NOTNLM OT - Beta cell function OT - Fatty liver OT - Insulin resistance OT - Insulin sensitivity OT - Liver fibrosis OT - MAFLD EDAT- 2024/03/05 00:45 MHDA- 2024/04/19 06:43 CRDT- 2024/03/04 23:38 PHST- 2023/10/05 00:00 [received] PHST- 2024/01/19 00:00 [accepted] PHST- 2024/04/19 06:43 [medline] PHST- 2024/03/05 00:45 [pubmed] PHST- 2024/03/04 23:38 [entrez] AID - 10.1007/s10620-024-08309-9 [pii] AID - 10.1007/s10620-024-08309-9 [doi] PST - ppublish SO - Dig Dis Sci. 2024 Apr;69(4):1430-1443. doi: 10.1007/s10620-024-08309-9. Epub 2024 Mar 4. PMID- 41238307 OWN - NLM STAT- MEDLINE DCOM- 20251114 LR - 20251114 IS - 2173-5743 (Electronic) IS - 2173-5743 (Linking) VI - 21 IP - 9 DP - 2025 Nov TI - Impact of the IL18 -137 G/C (rs187238) polymorphism on susceptibility and clinical manifestations in women systemic lupus erythematosus. PG - 501972 LID - S2173-5743(25)00162-5 [pii] LID - 10.1016/j.reumae.2025.501972 [doi] AB - INTRODUCTION AND OBJECTIVES: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, inflammation processes, and tissue damage. There are several genetic factors associated with the disease, many of them single nucleotide polymorphisms (SNPs). Interleukin-18 is a pro-inflammatory cytokine encoded by the IL18 gene, and the SNP -137 G/C (rs187238) has been studied in several populations. This case control study analyzed whether rs187238 is associated with SLE susceptibility and its clinical manifestations in a Brazilian population. MATERIALS AND METHODS: 153 patients fulfilling the American College of Rheumatology classification criteria for SLE were recruited, as well as 147 controls. Genotyping was performed by sequence-specific polymerase chain reaction (SSP-PCR). To assess SLE susceptibility a logistic regression test was conducted. Clinical aspects were tested through Poisson regression and clustered by Principal Component Analysis. RESULTS: An association between the rs187238*C_ carriers genotypes and SLE was found, these genotypes were associated with a 127% increased chance of developing the disease (OR=2.27, 95% CI=1.32-3.98, p=0.003). The *C_ genotypes were also associated with photosensitivity (PR=1.39, 95% CI=1.1-1.8, p=0.017), malar rash (PR=1.37, 95% CI=1.1-1.8, p=0.014) and Raynaud phenomenon (PR=1.37, 95% IC=1.1-1.8, p=0.015). DISCUSSION AND CONCLUSIONS: These findings suggest the potential of rs187238 as a genetic marker for SLE risk and clinical stratification in admixed Latin American populations. CI - Copyright © 2025 Sociedad Española de Reumatología (SER), Colegio Mexicano de Reumatología (CMR) and Elsevier España, S.L.U. All rights reserved. FAU - Magri, Danton AU - Magri D AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. FAU - Staffen, Clisten Fátima AU - Staffen CF AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. FAU - Farias, Ticiana Della Justina AU - Farias TDJ AD - Department of Biological Sciences, University of North Carolina at Charlotte, Charlotte, NC, USA. FAU - de Souza, Ilíada Rainha AU - de Souza IR AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. FAU - Muniz, Yara Costa Netto AU - Muniz YCN AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. FAU - Pereira, Ivânio Alves AU - Pereira IA AD - Rheumatology Division, University Hospital Polydoro Ernani de Sao Thiago, Federal University of Santa Catarina, Florianopolis, Brazil. FAU - Back, Lia Kubelka de Carlos AU - Back LKC AD - Biogenetika Individualized Medicine, Florianopolis, Brazil. FAU - Guimarães, Luciano Santos Pinto AU - Guimarães LSP AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. FAU - Lindenau, Juliana Dal-Ri AU - Lindenau JD AD - Genetic Polymorphisms Laboratory, Department of Cell Biology, Embryology and Genetics - Universidade Federal de Santa Catarina, Florianópolis, Brazil. Electronic address: juliana.lindenau@ufsc.br. LA - eng PT - Journal Article PL - Spain TA - Reumatol Clin (Engl Ed) JT - Reumatologia clinica JID - 101717526 RN - 0 (Interleukin-18) RN - 0 (IL18 protein, human) SB - IM MH - Humans MH - *Lupus Erythematosus, Systemic/genetics/diagnosis MH - Female MH - *Polymorphism, Single Nucleotide MH - *Genetic Predisposition to Disease MH - Adult MH - Case-Control Studies MH - *Interleukin-18/genetics MH - Middle Aged MH - Brazil MH - Genotype OTO - NOTNLM OT - Association study OT - Autoimmunity OT - Autoinmunidad OT - Citoquina OT - Cytokine OT - Estudio de asociación OT - Immunogenetics OT - Inmunogenética OT - Interleucina OT - Interleukin EDAT- 2025/11/15 00:29 MHDA- 2025/11/15 00:30 CRDT- 2025/11/14 20:55 PHST- 2025/05/26 00:00 [received] PHST- 2025/07/24 00:00 [revised] PHST- 2025/09/02 00:00 [accepted] PHST- 2025/11/15 00:30 [medline] PHST- 2025/11/15 00:29 [pubmed] PHST- 2025/11/14 20:55 [entrez] AID - S2173-5743(25)00162-5 [pii] AID - 10.1016/j.reumae.2025.501972 [doi] PST - ppublish SO - Reumatol Clin (Engl Ed). 2025 Nov;21(9):501972. doi: 10.1016/j.reumae.2025.501972. PMID- 26233502 OWN - NLM STAT- MEDLINE DCOM- 20160714 LR - 20151002 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 42 IP - 10 DP - 2015 Oct TI - Work Disability in Early Systemic Sclerosis: A Longitudinal Population-based Cohort Study. PG - 1794-800 LID - 10.3899/jrheum.150023 [doi] AB - OBJECTIVE: To study work disability (WD) with reference to levels of sick leave and disability pension in early systemic sclerosis (SSc). METHODS: Patients with SSc living in the southern part of Sweden with onset of their first non-Raynaud symptom between 2003 and 2009 and with a followup of 36 months were included in a longitudinal study. Thirty-two patients (26 women, 24 with limited SSc) with a median age of 47.5 years (interquartile range 43-53) were identified. WD was calculated in 30-day intervals from 12 months prior to disease onset until 36 months after, presented as the prevalence of WD per year (0-3) and as the period prevalence of mean net days per month (± SD). Comparisons were made between patients with different disease severity and sociodemographic characteristics, and between patients and a reference group (RG) from the general population. RESULTS: Seventy-eight percent had no WD 1 year prior to disease onset, which decreased to 47% after 3 years. The relative risk for WD in patients with SSc compared with RG was 0.95 (95% CI 0.39-2.33) at diagnosis, and increased to 2.41 (1.28-4.55) after 3 years. There were no significant correlations between WD and disease severity, but between WD and years at workplace (rs = -0.72; p = 0.002), education (rs = -0.51; p = 0.004), and sickness absence the month before disease onset (rs = 0.58; p = 0.001), respectively. CONCLUSION: Considerable increase in WD was noted 3 years after disease onset. Limited education, fewer years at workplace, and sickness absence before disease onset may be risk factors for sustained WD. FAU - Sandqvist, Gunnel AU - Sandqvist G AD - From the Department of Clinical Sciences, Section of Rheumatology, and Orthopedics, Department of Clinical Sciences, Lund University; Epidemiology and Register Centre South, Skåne University Hospital, Lund, Sweden; Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital, Bispebjerg and Frederiksberg, Denmark.G. Sandqvist, RegOT, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; I.F. Petersson, MD, PhD, Orthopedics, Department of Clinical Sciences, Lund University, and Epidemiology and Register Centre South, Skåne University Hospital; L.E. Kristensen, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University, and Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital. gunnel.sandqvist@med.lu.se. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - From the Department of Clinical Sciences, Section of Rheumatology, and Orthopedics, Department of Clinical Sciences, Lund University; Epidemiology and Register Centre South, Skåne University Hospital, Lund, Sweden; Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital, Bispebjerg and Frederiksberg, Denmark.G. Sandqvist, RegOT, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; I.F. Petersson, MD, PhD, Orthopedics, Department of Clinical Sciences, Lund University, and Epidemiology and Register Centre South, Skåne University Hospital; L.E. Kristensen, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University, and Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital. FAU - Petersson, Ingemar F AU - Petersson IF AD - From the Department of Clinical Sciences, Section of Rheumatology, and Orthopedics, Department of Clinical Sciences, Lund University; Epidemiology and Register Centre South, Skåne University Hospital, Lund, Sweden; Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital, Bispebjerg and Frederiksberg, Denmark.G. Sandqvist, RegOT, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; I.F. Petersson, MD, PhD, Orthopedics, Department of Clinical Sciences, Lund University, and Epidemiology and Register Centre South, Skåne University Hospital; L.E. Kristensen, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University, and Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital. FAU - Kristensen, Lars Erik AU - Kristensen LE AD - From the Department of Clinical Sciences, Section of Rheumatology, and Orthopedics, Department of Clinical Sciences, Lund University; Epidemiology and Register Centre South, Skåne University Hospital, Lund, Sweden; Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital, Bispebjerg and Frederiksberg, Denmark.G. Sandqvist, RegOT, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; I.F. Petersson, MD, PhD, Orthopedics, Department of Clinical Sciences, Lund University, and Epidemiology and Register Centre South, Skåne University Hospital; L.E. Kristensen, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University, and Musculoskeletal Statistics Unit, The Parker Institute, Department of Rheumatology, Copenhagen University Hospital. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150801 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adult MH - Age Factors MH - Cohort Studies MH - Confidence Intervals MH - Early Diagnosis MH - Employment/statistics & numerical data MH - Female MH - Humans MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Risk Assessment MH - Scleroderma, Systemic/*diagnosis/epidemiology/*rehabilitation MH - Severity of Illness Index MH - Sex Factors MH - Sick Leave/statistics & numerical data MH - Statistics, Nonparametric MH - Sweden/epidemiology MH - *Work Capacity Evaluation MH - Workers' Compensation/*statistics & numerical data OTO - NOTNLM OT - DISABILITY OT - LONGITUDINAL STUDIES OT - OCCUPATIONAL THERAPY OT - SYSTEMIC SCLEROSIS OT - WORK EDAT- 2015/08/04 06:00 MHDA- 2016/07/15 06:00 CRDT- 2015/08/03 06:00 PHST- 2015/06/05 00:00 [accepted] PHST- 2015/08/03 06:00 [entrez] PHST- 2015/08/04 06:00 [pubmed] PHST- 2016/07/15 06:00 [medline] AID - jrheum.150023 [pii] AID - 10.3899/jrheum.150023 [doi] PST - ppublish SO - J Rheumatol. 2015 Oct;42(10):1794-800. doi: 10.3899/jrheum.150023. Epub 2015 Aug 1. PMID- 33934078 OWN - NLM STAT- MEDLINE DCOM- 20211021 LR - 20211021 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 48 IP - 10 DP - 2021 Oct TI - Effect of Coping Strategies on Patient and Physician Perceptions of Disease Severity and Disability in Systemic Sclerosis. PG - 1569-1573 LID - 10.3899/jrheum.201612 [doi] AB - OBJECTIVE: Systemic sclerosis (SSc) results in impaired function, disability, and reduced health-related quality of life. We investigated the effect of coping strategies on the patient global assessment of health (PtGA) and Health Assessment Questionnaire-Disability Index (HAQ-DI), after controlling for clinical characteristics and disease activity. We also explored the relationship between coping strategies and the correlation between the PtGA and physician global assessment (PGA) in SSc. METHODS: We undertook posthoc analyses using baseline data obtained from the Raynaud Symptom Study (RSS). The PtGA, Coping Strategies Questionnaire, Pain Catastrophizing Scale, and Scleroderma Health Assessment Questionnaire were collected alongside the PGA, clinical characteristics, and patient demographics. Multivariable linear regression models and correlations were used to evaluate the relationship between coping strategies with the PtGA, HAQ-DI, and PGA. RESULTS: Of the 107 patients with SSc enrolled in the RSS, there were sufficient data available for the analysis of 91 participants. The mean PtGA was 40/100 (SD 27) and the mean HAQ-DI was 0.87/3.0 (SD 0.73). After controlling for clinical and patient demographics, pain catastrophizing and maladaptive coping skills were significantly associated with the PtGA and HAQ-DI scores (P < 0.05 for both), but not the PGA. CONCLUSION: The effect of coping strategies on PtGA and HAQ-DI (but not PGA in SSc) could influence the result of composite measures incorporating these outcome measures. Interventions to improve patient coping skills may support increased resilience and improve patient-perceived functional status and PtGA in SSc. CI - Copyright © 2021 by the Journal of Rheumatology. FAU - DiRenzo, Dana D AU - DiRenzo DD AUID- ORCID: 0000-0001-9350-1821 AD - D.D. DiRenzo, MD, MHS, A.A. Shah, MD, MHS, Johns Hopkins Division of Rheumatology, Baltimore, Maryland, USA. FAU - Smith, Theresa R AU - Smith TR AUID- ORCID: 0000-0002-7085-3864 AD - T.R. Smith, PhD, Department of Mathematical Sciences, University of Bath, Bath, UK. FAU - Frech, Tracy M AU - Frech TM AUID- ORCID: 0000-0002-5472-3840 AD - T.M. Frech, MD, MS, University of Utah, Salt Lake City, Utah, USA. FAU - Shah, Ami A AU - Shah AA AUID- ORCID: 0000-0002-1139-2009 AD - D.D. DiRenzo, MD, MHS, A.A. Shah, MD, MHS, Johns Hopkins Division of Rheumatology, Baltimore, Maryland, USA. FAU - Pauling, John D AU - Pauling JD AUID- ORCID: 0000-0002-2793-2364 AD - J.D. Pauling, BMedSci, BMBS, PhD, FRCP, Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), and Department of Pharmacy and Pharmacology, University of Bath, Bath, UK. JohnPauling@nhs.net. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210501 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Adaptation, Psychological MH - Disability Evaluation MH - Humans MH - Perception MH - *Physicians MH - Quality of Life MH - *Scleroderma, Systemic MH - Severity of Illness Index OTO - NOTNLM OT - coping OT - disability OT - pain OT - quality of life OT - scleroderma OT - systemic sclerosis EDAT- 2021/05/03 06:00 MHDA- 2023/02/25 06:00 CRDT- 2021/05/02 21:02 PHST- 2021/04/14 00:00 [accepted] PHST- 2021/05/03 06:00 [pubmed] PHST- 2023/02/25 06:00 [medline] PHST- 2021/05/02 21:02 [entrez] AID - jrheum.201612 [pii] AID - 10.3899/jrheum.201612 [doi] PST - ppublish SO - J Rheumatol. 2021 Oct;48(10):1569-1573. doi: 10.3899/jrheum.201612. Epub 2021 May 1. PMID- 40527670 OWN - NLM STAT- MEDLINE DCOM- 20260307 LR - 20260505 IS - 1545-004X (Electronic) IS - 0894-1130 (Linking) VI - 39 IP - 1 DP - 2026 Jan-Mar TI - The results of serial casting in the treatment of proximal interphalangeal joint contractures in patients with systemic sclerosis. PG - 198-213 LID - S0894-1130(25)00071-7 [pii] LID - 10.1016/j.jht.2025.04.021 [doi] AB - BACKGROUND: Systemic sclerosis can lead to significant disability. Flexion contractures of the proximal interphalangeal joints are common in systemic sclerosis. Serial casting can be used to correct these contractures. The literature lacks data on the use of this method for this purpose. PURPOSE: To analyze the results of serial casting intervention in the treatment of proximal interphalangeal joint flexion contractures and identify the factors that may affect outcomes. STUDY DESIGN: Retrospective case series. METHODS: The data of 10 patients treated with serial casting for their proximal interphalangeal joint contractures were analyzed. Range of motions of the finger joints, grip pressures, pinch strengths and hand functions at the initial and final evaluations were compared. A finger goniometer, a modified sphygmomanometer, B&L pinchmeter and Duruöz Hand Index were used for the measurements, respectively. RESULTS: A total of 38 fingers were serially casted. The serial casting intervention resulted in significant (p<0.001) reduction in the average proximal interphalangeal joint extension loss. Some flexion decreases in this joint accompanied this gain (p<0.001). Range of motions of the other finger joints increased (p<0.001). The average grip pressures (p=0.005 and 0.007), lateral pinch strengths (p=0.04) and total hand function (p=0.013) improved. The magnitude of the extension loss in the beginning, severity of Raynaud phenomenon, disability and disease durations were all moderately important factors in predicting the gains in the proximal interphalangeal joints. Sixty percent of the gains (adjusted R(2)=0.6) could be explained by these factors. CONCLUSIONS: Serial casting may be an effective method to decrease flexion contractures of proximal interphalangeal joints as an adjunctive method to other rehabilitation modalities in patients with systemic sclerosis. Clinicians should consider disease-specific constraints both in the selection of patients for serial casting application and their follow-up. CI - Copyright © 2025 Elsevier Inc. All rights reserved. FAU - Uğurlu, Ümit AU - Uğurlu Ü AD - Bezmialem Vakıf University, Faculty of Health Sciences, Department of Occupational Therapy, İstanbul, Türkiye. Electronic address: uugurlu@bezmialem.edu.tr. LA - eng PT - Journal Article DEP - 20250616 PL - United States TA - J Hand Ther JT - Journal of hand therapy : official journal of the American Society of Hand Therapists JID - 8806591 SB - IM MH - Humans MH - *Finger Joint/physiopathology MH - Female MH - Retrospective Studies MH - Male MH - *Contracture/etiology/therapy/physiopathology/rehabilitation MH - Range of Motion, Articular/physiology MH - Middle Aged MH - *Casts, Surgical MH - *Scleroderma, Systemic/complications MH - Hand Strength/physiology MH - Adult MH - Treatment Outcome MH - Aged OTO - NOTNLM OT - Contracture OT - Hand OT - Rehabilitation OT - Serial casting OT - Systemic sclerosis COIS- Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/06/18 00:28 MHDA- 2026/03/07 18:38 CRDT- 2025/06/17 21:53 PHST- 2024/11/16 00:00 [received] PHST- 2025/04/20 00:00 [revised] PHST- 2025/04/20 00:00 [accepted] PHST- 2026/03/07 18:38 [medline] PHST- 2025/06/18 00:28 [pubmed] PHST- 2025/06/17 21:53 [entrez] AID - S0894-1130(25)00071-7 [pii] AID - 10.1016/j.jht.2025.04.021 [doi] PST - ppublish SO - J Hand Ther. 2026 Jan-Mar;39(1):198-213. doi: 10.1016/j.jht.2025.04.021. Epub 2025 Jun 16. PMID- 19824738 OWN - NLM STAT- MEDLINE DCOM- 20091215 LR - 20220330 IS - 1175-0561 (Print) IS - 1175-0561 (Linking) VI - 10 IP - 6 DP - 2009 TI - Cutaneous lupus erythematosus: issues in diagnosis and treatment. PG - 365-81 LID - 10.2165/11310780-000000000-00000 [doi] AB - Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or 'butterfly' rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE. FAU - Walling, Hobart W AU - Walling HW AD - Department of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah 84132, USA. FAU - Sontheimer, Richard D AU - Sontheimer RD LA - eng PT - Journal Article PT - Review PL - New Zealand TA - Am J Clin Dermatol JT - American journal of clinical dermatology JID - 100895290 SB - IM MH - Humans MH - *Lupus Erythematosus, Cutaneous/diagnosis/drug therapy/pathology MH - Lupus Erythematosus, Discoid/diagnosis/drug therapy/pathology MH - Lupus Erythematosus, Systemic/diagnosis/drug therapy/pathology RF - 139 EDAT- 2009/10/15 06:00 MHDA- 2009/12/16 06:00 CRDT- 2009/10/15 06:00 PHST- 2009/10/15 06:00 [entrez] PHST- 2009/10/15 06:00 [pubmed] PHST- 2009/12/16 06:00 [medline] AID - 3 [pii] AID - 10.2165/11310780-000000000-00000 [doi] PST - ppublish SO - Am J Clin Dermatol. 2009;10(6):365-81. doi: 10.2165/11310780-000000000-00000. PMID- 25595720 OWN - NLM STAT- MEDLINE DCOM- 20150420 LR - 20240610 IS - 1097-6787 (Electronic) IS - 0190-9622 (Print) IS - 0190-9622 (Linking) VI - 72 IP - 3 DP - 2015 Mar TI - Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis. PG - 449-55 LID - S0190-9622(14)02269-5 [pii] LID - 10.1016/j.jaad.2014.12.009 [doi] AB - BACKGROUND: Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment. OBJECTIVE: We sought to define the clinical phenotype of patients with anti-TIF-1γ DM. METHODS: Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features. RESULTS: In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic ("red on white") patches. LIMITATIONS: This was a retrospective study from a single tertiary referral center. CONCLUSION: TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM. CI - Copyright © 2014 American Academy of Dermatology, Inc. All rights reserved. FAU - Fiorentino, David F AU - Fiorentino DF AD - Department of Dermatology, Stanford University School of Medicine, Redwood City, California. Electronic address: fiorentino@stanford.edu. FAU - Kuo, Karen AU - Kuo K AD - Department of Dermatology, Stanford University School of Medicine, Redwood City, California. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Redwood City, California; Department of Veterans Affairs Palo Alto Health Care System, Palo Alto, California. FAU - Zaba, Lisa AU - Zaba L AD - Department of Dermatology, Stanford University School of Medicine, Redwood City, California. FAU - Li, Shufeng AU - Li S AD - Department of Dermatology, Stanford University School of Medicine, Redwood City, California. FAU - Casciola-Rosen, Livia AU - Casciola-Rosen L AD - Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, Maryland. LA - eng GR - R01 AR044684/AR/NIAMS NIH HHS/United States GR - R56 AR062615/AR/NIAMS NIH HHS/United States GR - T32 AR050942/AR/NIAMS NIH HHS/United States GR - R13 AR053058/AR/NIAMS NIH HHS/United States GR - P30 AR053503/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20150114 PL - United States TA - J Am Acad Dermatol JT - Journal of the American Academy of Dermatology JID - 7907132 RN - 0 (Autoantibodies) RN - 0 (TRIM33 protein, human) RN - 0 (Transcription Factors) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoantibodies/*blood MH - Child MH - Child, Preschool MH - Dermatomyositis/*blood/diagnosis/*immunology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Retrospective Studies MH - Transcription Factors/*immunology MH - Young Adult PMC - PMC4351728 MID - NIHMS667692 OTO - NOTNLM OT - Cutaneous Dermatomyositis Assessment and Severity Index OT - autoantibodies OT - dermatomyositis OT - malignancy OT - phenotype OT - transcriptional intermediary factor-1γ COIS- Conflicts of interest: None declared. EDAT- 2015/01/18 06:00 MHDA- 2015/04/22 06:00 PMCR- 2015/03/06 CRDT- 2015/01/18 06:00 PHST- 2014/07/15 00:00 [received] PHST- 2014/11/30 00:00 [revised] PHST- 2014/12/08 00:00 [accepted] PHST- 2015/01/18 06:00 [entrez] PHST- 2015/01/18 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] PHST- 2015/03/06 00:00 [pmc-release] AID - S0190-9622(14)02269-5 [pii] AID - 10.1016/j.jaad.2014.12.009 [doi] PST - ppublish SO - J Am Acad Dermatol. 2015 Mar;72(3):449-55. doi: 10.1016/j.jaad.2014.12.009. Epub 2015 Jan 14. PMID- 28637716 OWN - NLM STAT- MEDLINE DCOM- 20180717 LR - 20250530 IS - 1538-8514 (Electronic) IS - 1535-7163 (Print) IS - 1535-7163 (Linking) VI - 16 IP - 10 DP - 2017 Oct TI - Modulation of Plasma Metabolite Biomarkers of the MAPK Pathway with MEK Inhibitor RO4987655: Pharmacodynamic and Predictive Potential in Metastatic Melanoma. PG - 2315-2323 LID - 10.1158/1535-7163.MCT-16-0881 [doi] AB - MAPK pathway activation is frequently observed in human malignancies, including melanoma, and is associated with sensitivity to MEK inhibition and changes in cellular metabolism. Using quantitative mass spectrometry-based metabolomics, we identified in preclinical models 21 plasma metabolites including amino acids, propionylcarnitine, phosphatidylcholines, and sphingomyelins that were significantly altered in two B-RAF-mutant melanoma xenografts and that were reversed following a single dose of the potent and selective MEK inhibitor RO4987655. Treatment of non-tumor-bearing animals and mice bearing the PTEN-null U87MG human glioblastoma xenograft elicited plasma changes only in amino acids and propionylcarnitine. In patients with advanced melanoma treated with RO4987655, on-treatment changes of amino acids were observed in patients with disease progression and not in responders. In contrast, changes in phosphatidylcholines and sphingomyelins were observed in responders. Furthermore, pretreatment levels of seven lipids identified in the preclinical screen were statistically significantly able to predict objective responses to RO4987655. The RO4987655 treatment-related changes were greater than baseline physiological variability in nontreated individuals. This study provides evidence of a translational exo-metabolomic plasma readout predictive of clinical efficacy together with pharmacodynamic utility following treatment with a signal transduction inhibitor. Mol Cancer Ther; 16(10); 2315-23. ©2017 AACR. CI - ©2017 American Association for Cancer Research. FAU - Ang, Joo Ern AU - Ang JE AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. AD - Drug Development Unit, The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. FAU - Pal, Akos AU - Pal A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Asad, Yasmin J AU - Asad YJ AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Henley, Alan T AU - Henley AT AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Box, Gary AU - Box G AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - de Haven Brandon, Alexis AU - de Haven Brandon A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Revell, Victoria L AU - Revell VL AD - Faculty of Health and Medical Sciences, University of Surrey, Guildford, United Kingdom. FAU - Skene, Debra J AU - Skene DJ AD - Faculty of Health and Medical Sciences, University of Surrey, Guildford, United Kingdom. FAU - Venturi, Miro AU - Venturi M AD - F. Hoffmann-LaRoche Ltd., Diagnostics Division, DIA Biomarker Group, Basel, Switzerland. FAU - Rueger, Ruediger AU - Rueger R AD - Roche Pharmaceutical Research and Early Development, Translational Medicine Oncology, Roche Innovation Center Penzberg, Penzberg, Germany. FAU - Meresse, Valerie AU - Meresse V AD - Roche Pharmaceutical Research and Early Development, Translational Medicine Oncology, Roche Innovation Center Basel, Basel, Switzerland. FAU - Eccles, Suzanne A AU - Eccles SA AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - de Bono, Johann S AU - de Bono JS AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. AD - Drug Development Unit, The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. FAU - Kaye, Stanley B AU - Kaye SB AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. AD - Drug Development Unit, The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. FAU - Workman, Paul AU - Workman P AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Banerji, Udai AU - Banerji U AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. AD - Drug Development Unit, The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. Florence.Raynaud@icr.ac.uk. AD - Drug Development Unit, The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. LA - eng GR - WT_/Wellcome Trust/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - C309/A8992/CRUK_/Cancer Research UK/United Kingdom GR - 090952/Z/09/Z/WT_/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20170621 PL - United States TA - Mol Cancer Ther JT - Molecular cancer therapeutics JID - 101132535 RN - 0 (Benzamides) RN - 0 (Biomarkers, Tumor) RN - 0 (CH 4987655) RN - 0 (Oxazines) RN - 0 (Protein Kinase Inhibitors) RN - EC 2.7.11.1 (BRAF protein, human) RN - EC 2.7.11.1 (Proto-Oncogene Proteins B-raf) RN - EC 2.7.12.2 (Mitogen-Activated Protein Kinase Kinases) SB - IM MH - Animals MH - Benzamides/*administration & dosage MH - Biomarkers, Tumor/*blood MH - Cell Line, Tumor MH - Humans MH - MAP Kinase Signaling System/drug effects MH - Melanoma/blood/*drug therapy/pathology MH - Mice MH - Mitogen-Activated Protein Kinase Kinases/antagonists & inhibitors/*blood MH - Mutation MH - Neoplasm Metastasis MH - Oxazines/*administration & dosage MH - Protein Kinase Inhibitors/administration & dosage MH - Proto-Oncogene Proteins B-raf/genetics MH - Xenograft Model Antitumor Assays PMC - PMC6112418 MID - EMS79017 EDAT- 2017/06/24 06:00 MHDA- 2018/07/18 06:00 PMCR- 2018/08/28 CRDT- 2017/06/23 06:00 PHST- 2016/12/19 00:00 [received] PHST- 2017/05/31 00:00 [revised] PHST- 2017/06/06 00:00 [accepted] PHST- 2017/06/24 06:00 [pubmed] PHST- 2018/07/18 06:00 [medline] PHST- 2017/06/23 06:00 [entrez] PHST- 2018/08/28 00:00 [pmc-release] AID - 1535-7163.MCT-16-0881 [pii] AID - 10.1158/1535-7163.MCT-16-0881 [doi] PST - ppublish SO - Mol Cancer Ther. 2017 Oct;16(10):2315-2323. doi: 10.1158/1535-7163.MCT-16-0881. Epub 2017 Jun 21. PMID- 36635578 OWN - NLM STAT- MEDLINE DCOM- 20230310 LR - 20260127 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 43 IP - 4 DP - 2023 Apr TI - Central and peripheral mechanisms of pain in fibromyalgia: scoping review protocol. PG - 757-762 LID - 10.1007/s00296-023-05275-9 [doi] AB - Fibromyalgia is characterised by widespread musculoskeletal pain, which may present with fatigue, depression, anxiety, sleep and cognitive disturbances. It is the second most prevalent rheumatic disease. An accurate diagnosis is challenging, since its symptoms may resemble diverse conditions such as carpal tunnel syndrome, Raynaud syndrome, Sjögren syndrome, amongst others. Neuropathic pain and autonomic dysfunction in fibromyalgia suggest the involvement of the nervous system. Ion channels, neurotransmitters and neuromodulators may play a role. Small fibre neuropathy (SFN) may also cause chronic widespread pain. SFN may occur in 50% of fibromyalgia patients, but its role in the disease is unknown. Despite several efforts to synthesise the evidence on the mechanisms for pain in fibromyalgia, there are few studies applying an integrative perspective of neurochemical, immunological, and neuroanatomical characteristics, and their relevance to the disease. This protocol aims to clarify the mechanisms of the central and peripheral nervous system associated with pain in fibromyalgia. We will retrieve published studies from Web of Science, MEDLINE, Scopus, EBSCOhost, Ovid and Google Scholar. All clinical studies or experimental models of fibromyalgia reporting imaging, neurophysiological, anatomical, structural, neurochemical, or immunological characteristics of the central or peripheral nervous systems associated with pain will be included. Exclusion criteria will eliminate studies evaluating pain without a standardised measure, studies written in languages different from Spanish or English that could not be appropriately translated, and studies whose full-text files could not be retrieved after all efforts made. A narrative synthesis will be performed. CI - © 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Pérez-Neri, Iván AU - Pérez-Neri I AUID- ORCID: 0000-0003-0190-7272 AD - Department of Neurochemistry, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Insurgentes Sur 3877, La Fama, Tlalpan, 14269, Ciudad de México, Mexico. FAU - Sandoval, Hugo AU - Sandoval H AUID- ORCID: 0000-0002-9622-1558 AD - General Directorate, National Institute of Rehabilitation Luis Guillermo Ibarra Ibarra, Calzada México-Xochimilco 289, Arenal de Guadalupe, 14389, Ciudad de México, Mexico. FAU - Estêvão, M Dulce AU - Estêvão MD AUID- ORCID: 0000-0002-7151-8363 AD - Escola Superior de Saúde da Universidade do Algarve, Campus de Gambelas, 8005-139, Faro, Portugal. FAU - Vasanthan, Lenny T AU - Vasanthan LT AUID- ORCID: 0000-0003-1776-6304 AD - Physiotherapy Unit, Physical Medicine and Rehabilitation Department, Christian Medical College, Vellore, 632004, India. FAU - Alarcon-Ruiz, Christoper A AU - Alarcon-Ruiz CA AUID- ORCID: 0000-0003-3907-2784 AD - Unidad de Investigación Para la Generación y Síntesis de Evidencias en Salud, Universidad San Ignacio de Loyola, Av. La Fontana 550, La Molina, 15024, Lima, Perú. FAU - Ruszkowski, Jakub AU - Ruszkowski J AUID- ORCID: 0000-0002-9666-9627 AD - Department of Pathophysiology, Faculty of Medicine, Medical University of Gdańsk, Gdańsk, Poland. AD - Department of Nephrology, Transplantology and Internal Medicine. Faculty of Medicine, Medical University of Gdańsk, Dębinki 7, 80-211, Gdańsk, Poland. FAU - Mathangasinghe, Yasith AU - Mathangasinghe Y AUID- ORCID: 0000-0003-4641-5642 AD - Australian Regenerative Medicine Institute, Faculty of Medicine Nursing and Health Sciences, Monash University, Clayton, VIC, 3800, Australia. AD - Department of Anatomy Genetics and Biomedical Informatics, Faculty of Medicine, University of Colombo, 25 Kynsey Road, Colombo, 00800, Sri Lanka. FAU - Ríos, Camilo AU - Ríos C AUID- ORCID: 0000-0002-3216-0119 AD - Department of Neurochemistry, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Insurgentes Sur 3877, La Fama, Tlalpan, 14269, Ciudad de México, Mexico. FAU - Pineda, Carlos AU - Pineda C AUID- ORCID: 0000-0003-0544-7461 AD - General Directorate, National Institute of Rehabilitation Luis Guillermo Ibarra Ibarra, Calzada México-Xochimilco 289, Arenal de Guadalupe, 14389, Ciudad de México, Mexico. carpineda@yahoo.com. LA - eng PT - Journal Article DEP - 20230113 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Humans MH - *Fibromyalgia/diagnosis MH - *Chronic Pain/etiology MH - *Neuralgia MH - *Rheumatic Diseases MH - Scoping Reviews as Topic OTO - NOTNLM OT - Autoimmunity OT - Central sensitisation OT - Fibromyalgia OT - Neuropathic pain OT - Peripheral nerve EDAT- 2023/01/13 06:00 MHDA- 2023/03/11 06:00 CRDT- 2023/01/12 23:27 PHST- 2022/12/16 00:00 [received] PHST- 2023/01/05 00:00 [accepted] PHST- 2023/01/13 06:00 [pubmed] PHST- 2023/03/11 06:00 [medline] PHST- 2023/01/12 23:27 [entrez] AID - 10.1007/s00296-023-05275-9 [pii] AID - 10.1007/s00296-023-05275-9 [doi] PST - ppublish SO - Rheumatol Int. 2023 Apr;43(4):757-762. doi: 10.1007/s00296-023-05275-9. Epub 2023 Jan 13. PMID- 24371972 OWN - NLM STAT- MEDLINE DCOM- 20150821 LR - 20181202 IS - 1330-0164 (Print) IS - 1330-0164 (Linking) VI - 67 Suppl 1 DP - 2013 Oct TI - [List of diagnostic tests and procedures in leg ulcer]. PG - 21-8 AB - Many factors contribute to the pathogenesis of leg ulcer. Most patients have venous leg ulcer due to chronic venous insufficiency. Less often, patients have arterial leg ulcer resulting from peripheral arterial occlusive disease, the most common cause of which is arteriosclerosis. Leg ulcer may be of a mixed arteriovenous origin. In diabetic patients, distal symmetric neuropathy and peripheral vascular disease are probably the most important etiologic factors in the development of diabetic leg ulcer. Other causes of chronic leg ulcers are hematologic diseases, autoimmune diseases, genetic defects, infectious diseases, primary skin diseases, cutaneous malignant diseases, use of some medications and therapeutic procedures, and numerous exogenous factors. Diagnosis of leg ulcer is based on medical history, inspection, palpation of skin temperature, palpation of arteries, fascia holes, presence and degree of edema, firm painful cords, and functional testing to assess peripheral occlusive arterial disease or identify superficial and deep venous reflux of the legs. Knowledge of differential diagnosis is essential for ensuring treatment success in patients with leg ulcer. There are many possible etiologic factors of leg ulcers and sometimes, clinical findings are similar. Additional testing should be performed, e.g., serologic testing such as blood count, C-reactive protein, HBA1c, erythrocyte sedimentation rate, differential blood count, total proteins, electrolytes, coagulation parameters, circulating immune complex, cryoglobulins, homocysteins, AT, PAI-1, APC resistance, proteins C and S, paraproteins, ANA, ENA, ANCA, dsDNA, antiphospholipid antibodies, urea, creatinine, blood lipids, vitamins and trace elements. Also, biopsy of the lesion for histopathology, direct immunofluorescence, bacteriology and mycology should be included. Other tests are Raynaud (cold stimulation) test and pathergy test. Device-based diagnostic testing should be performed for future clarification. Ankle brachial pressure index, color duplex sonography, plethysmography, MSCT and MR angiography, digital subtraction angiography, phlebography, angiography, x-ray, and capillaroscopy in lupus erythematosus are indicated. Except for bacteriologic analyses of wound biopsies, there is no test to provide specific information on the wound condition. FAU - Spoljar, Sanja AU - Spoljar S AD - Klinicki bolnicki centar Sestre milosrdnice, Klinika za kozne i spolne bolesti, Referentni centarza kronicne rane Ministarstva zdravlja RH, Zagreb, Hrvatska. sanja.spoljar3@zg.t-com.hr LA - hrv PT - Journal Article TT - Osnovni dijagnosticki postupci kod bolesnika s venskim ulkusom. PL - Croatia TA - Acta Med Croatica JT - Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti JID - 9208249 SB - IM MH - Aged MH - Biopsy/methods MH - Diabetic Foot/diagnosis MH - Diagnosis, Differential MH - Diagnostic Tests, Routine/*methods MH - Humans MH - Leg Ulcer/blood/*diagnosis/diagnostic imaging/pathology MH - Lower Extremity/blood supply MH - Male MH - Medical History Taking MH - Microscopy/methods MH - Palpation/methods MH - Radiography MH - Ultrasonography, Doppler, Color/methods EDAT- 2014/01/01 06:00 MHDA- 2015/08/22 06:00 CRDT- 2013/12/31 06:00 PHST- 2013/12/31 06:00 [entrez] PHST- 2014/01/01 06:00 [pubmed] PHST- 2015/08/22 06:00 [medline] PST - ppublish SO - Acta Med Croatica. 2013 Oct;67 Suppl 1:21-8. PMID- 37961408 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240616 DP - 2023 Nov 5 TI - MDA5-autoimmunity and Interstitial Pneumonitis Contemporaneous with the COVID-19 Pandemic (MIP-C). LID - 2023.11.03.23297727 [pii] LID - 10.1101/2023.11.03.23297727 [doi] AB - BACKGROUND: Anti-MDA5 (Melanoma differentiation-associated protein-5) positive dermatomyositis (MDA5 (+) -DM) is characterised by rapidly progressive interstitial lung disease (ILD) and high mortality. MDA5 senses single-stranded RNA and is a key pattern recognition receptor for the SARS-CoV-2 virus. METHODS: This is a retrospective observational study of a surge in MDA5 autoimmunity, as determined using a 15 muscle-specific autoantibodies (MSAs) panel, between Janurary 2018-December 2022 in Yorkshire, UK. MDA5-positivity was correlated with clinical features and outcome, and regional SARS-CoV-2 positivity and vaccination rates. Gene expression patterns in COVID-19 were compared with autoimmune lung disease and idiopathic pulmonary fibrosis (IPF) to gain clues into the genesis of the observed MDA5 (+) -DM outbreak. RESULTS: Sixty new anti-MDA5+, but not other MSAs surged between 2020-2022, increasing from 0.4% in 2019 to 2.1% (2020), 4.8% (2021) and 1.7% (2022). Few (8/60) had a prior history of confirmed COVID-19, peak rates overlapped with regional SARS-COV-2 community positivity rates in 2021, and 58% (35/60) had received anti-SARS-CoV-2 RNA vaccines. Few (8/60) had a prior history of COVID-19, whereas 58% (35/60) had received anti-SARS-CoV-2 RNA vaccines. 25/60 cases developed ILD which rapidly progression with death in 8 cases. Among the 35/60 non-ILD cases, 14 had myositis, 17 Raynaud phenomena and 10 had dermatomyositis spectrum rashes. Transcriptomic studies showed strong IFIH1 (gene encoding for MDA5) induction in COVID-19 and autoimmune-ILD, but not IPF, and IFIH1 strongly correlated with an IL-15-centric type-1 interferon response and an activated CD8+ T cell signature that is an immunologic hallmark of progressive ILD in the setting of systemic autoimmune rheumatic diseases. The IFIH1 rs1990760TT variant blunted such response. CONCLUSIONS: A distinct pattern of MDA5-autoimmunity cases surged contemporaneously with circulation of the SARS-COV-2 virus during COVID-19. Bioinformatic insights suggest a shared immunopathology with known autoimmune lung disease mechanisms. FAU - Iqbal, Khizer AU - Iqbal K FAU - Sinha, Saptarshi AU - Sinha S FAU - David, Paula AU - David P FAU - De Marco, Gabriele AU - De Marco G FAU - Taheri, Sahar AU - Taheri S FAU - McLaren, Ella AU - McLaren E FAU - Maisuria, Sheetal AU - Maisuria S FAU - Arumugakani, Gururaj AU - Arumugakani G FAU - Ash, Zoe AU - Ash Z FAU - Buckley, Catrin AU - Buckley C FAU - Coles, Lauren AU - Coles L FAU - Hettiarachchi, Chamila AU - Hettiarachchi C FAU - Smithson, Gayle AU - Smithson G FAU - Slade, Maria AU - Slade M FAU - Shah, Rahul AU - Shah R FAU - Marzo-Ortega, Helena AU - Marzo-Ortega H FAU - Keen, Mansoor AU - Keen M FAU - Lawson, Catherine AU - Lawson C FAU - Mclorinan, Joanna AU - Mclorinan J FAU - Nizam, Sharmin AU - Nizam S FAU - Reddy, Hanu AU - Reddy H FAU - Sharif, Omer AU - Sharif O FAU - Sultan, Shabina AU - Sultan S FAU - Tran, Gui AU - Tran G FAU - Wood, Mark AU - Wood M FAU - Wood, Samuel AU - Wood S FAU - Ghosh, Pradipta AU - Ghosh P AUID- ORCID: 0000-0002-8917-3201 FAU - McGonagle, Dennis AU - McGonagle D LA - eng GR - R01 AI155696/AI/NIAID NIH HHS/United States PT - Journal Article PT - Preprint DEP - 20231105 PL - United States TA - medRxiv JT - medRxiv : the preprint server for health sciences JID - 101767986 UIN - EBioMedicine. 2024 Jun;104:105136. doi: 10.1016/j.ebiom.2024.105136. PMID: 38723554 PMC - PMC10635254 EDAT- 2023/11/14 06:42 MHDA- 2023/11/14 06:43 PMCR- 2023/11/09 CRDT- 2023/11/14 03:57 PHST- 2023/11/14 06:43 [medline] PHST- 2023/11/14 06:42 [pubmed] PHST- 2023/11/14 03:57 [entrez] PHST- 2023/11/09 00:00 [pmc-release] AID - 2023.11.03.23297727 [pii] AID - 10.1101/2023.11.03.23297727 [doi] PST - epublish SO - medRxiv [Preprint]. 2023 Nov 5:2023.11.03.23297727. doi: 10.1101/2023.11.03.23297727. PMID- 20231208 OWN - NLM STAT- MEDLINE DCOM- 20100726 LR - 20161125 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 37 IP - 5 DP - 2010 May TI - Interstitial lung disease associated with anti-PM/Scl or anti-aminoacyl-tRNA synthetase autoantibodies: a similar condition? PG - 1000-9 LID - 10.3899/jrheum.090652 [doi] AB - OBJECTIVE: To compare anti-PM/Scl autoantibody-associated interstitial lung disease (ILD) with anti-aminoacyl-tRNA synthetases (anti-ARS) autoantibody-associated ILD. METHODS: We retrospectively studied 21 patients with ILD from a department of respiratory medicine, including 9 with anti-PM/Scl autoantibodies (6 women, median age 55 yrs, followup 5.5 yrs) and 12 with anti-ARS autoantibodies (6 women, median age 59 yrs, followup 2.3 yrs). RESULTS: Pulmonary manifestations in patients with anti-PM/Scl autoantibody-associated ILD usually followed the extrapulmonary manifestations of the connective tissue disease (CTD) (7/9 cases). The predominant imaging features on initial high resolution computed tomography were ground-glass attenuation and reticular opacities, and mainly suggested nonspecific interstitial pneumonia in both groups. CTD was classified as dermatomyositis (DM; 2), undifferentiated CTD (2), cutaneous limited systemic sclerosis (2), rheumatoid arthritis (RA; 1), and overlap syndrome (1) in the anti-PM/Scl group; and polymyositis (4), undifferentiated CTD (5), DM (1), amyopathic DM (1), and RA (1) in the anti-ARS group. Frequencies of arthralgia, Raynaud phenomenon, cutaneous rash, and mechanic's hands were comparable in both groups. Myalgia or muscle weakness was present in 0/9 PM/Scl and 5/12 ARS patients (p < 0.05). More than 1 autoantibody was present in 11 patients. ILD worsened despite treatment in 4 patients with anti-PM/Scl autoantibodies and 2 with anti-ARS autoantibodies, and included 1 death. CONCLUSION: Anti-PM/Scl and anti-ARS antibodies are associated with similar clinical manifestations, with the exception only of more overt myositis in the latter, therefore challenging the clinical specificity of the antisynthetase syndrome. FAU - Lega, Jean-Christophe AU - Lega JC AD - Department of Respiratory Medicine, Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon I, University of Lyon, Lyon, France. FAU - Cottin, Vincent AU - Cottin V FAU - Fabien, Nicole AU - Fabien N FAU - Thivolet-Béjui, Françoise AU - Thivolet-Béjui F FAU - Cordier, Jean-François AU - Cordier JF LA - eng PT - Journal Article DEP - 20100315 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 0 (Autoantibodies) RN - 0 (Nuclear Proteins) RN - EC 3.1.- (Exoribonucleases) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - EC 3.1.13.- (EXOSC10 protein, human) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Aged MH - Amino Acyl-tRNA Synthetases/*immunology MH - Autoantibodies/*immunology MH - Diagnosis, Differential MH - Exoribonucleases/*immunology MH - Exosome Multienzyme Ribonuclease Complex MH - Female MH - Humans MH - Lung Diseases, Interstitial/diagnostic imaging/*immunology MH - Male MH - Middle Aged MH - Nuclear Proteins/*immunology MH - Radiography MH - Retrospective Studies MH - Severity of Illness Index EDAT- 2010/03/17 06:00 MHDA- 2010/07/27 06:00 CRDT- 2010/03/17 06:00 PHST- 2010/03/17 06:00 [entrez] PHST- 2010/03/17 06:00 [pubmed] PHST- 2010/07/27 06:00 [medline] AID - jrheum.090652 [pii] AID - 10.3899/jrheum.090652 [doi] PST - ppublish SO - J Rheumatol. 2010 May;37(5):1000-9. doi: 10.3899/jrheum.090652. Epub 2010 Mar 15. PMID- 34716350 OWN - NLM STAT- MEDLINE DCOM- 20211130 LR - 20250530 IS - 2041-1723 (Electronic) IS - 2041-1723 (Linking) VI - 12 IP - 1 DP - 2021 Oct 29 TI - Genome-wide association study identifies susceptibility loci for acute myeloid leukemia. PG - 6233 LID - 10.1038/s41467-021-26551-x [doi] LID - 6233 AB - Acute myeloid leukemia (AML) is a hematological malignancy with an undefined heritable risk. Here we perform a meta-analysis of three genome-wide association studies, with replication in a fourth study, incorporating a total of 4018 AML cases and 10488 controls. We identify a genome-wide significant risk locus for AML at 11q13.2 (rs4930561; P = 2.15 × 10(-8); KMT5B). We also identify a genome-wide significant risk locus for the cytogenetically normal AML sub-group (N = 1287) at 6p21.32 (rs3916765; P = 1.51 × 10(-10); HLA). Our results inform on AML etiology and identify putative functional genes operating in histone methylation (KMT5B) and immune function (HLA). CI - © 2021. The Author(s). FAU - Lin, Wei-Yu AU - Lin WY AUID- ORCID: 0000-0002-9267-7988 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Fordham, Sarah E AU - Fordham SE AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Hungate, Eric AU - Hungate E AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Sunter, Nicola J AU - Sunter NJ AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Elstob, Claire AU - Elstob C AUID- ORCID: 0000-0003-4252-493X AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Xu, Yaobo AU - Xu Y AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Park, Catherine AU - Park C AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Quante, Anne AU - Quante A AD - Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany. AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Strauch, Konstantin AU - Strauch K AD - Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany. AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Gieger, Christian AU - Gieger C AUID- ORCID: 0000-0001-6986-9554 AD - Ludwig-Maximilians-Universität München, Chair of Genetic Epidemiology, IBE, Faculty of Medicine, Munich, Germany. FAU - Skol, Andrew AU - Skol A AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Rahman, Thahira AU - Rahman T AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Sucheston-Campbell, Lara AU - Sucheston-Campbell L AD - College of Pharmacy, The Ohio State University, Columbus, OH, USA. FAU - Wang, Junke AU - Wang J AD - College of Pharmacy, The Ohio State University, Columbus, OH, USA. FAU - Hahn, Theresa AU - Hahn T AUID- ORCID: 0000-0002-3835-8855 AD - Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USA. FAU - Clay-Gilmour, Alyssa I AU - Clay-Gilmour AI AD - Arnold School of Public Health, Department of Epidemiology & Biostatistics, University of South Carolina, Greenville, USA. FAU - Jones, Gail L AU - Jones GL AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Marr, Helen J AU - Marr HJ AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Jackson, Graham H AU - Jackson GH AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Menne, Tobias AU - Menne T AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Collin, Mathew AU - Collin M AUID- ORCID: 0000-0001-6585-9586 AD - Department of Haematology, Freeman Hospital, Newcastle upon Tyne Hospitals National Health Service Foundation Trust, Newcastle upon Tyne, UK. FAU - Ivey, Adam AU - Ivey A AD - Department of Medical and Molecular Genetics, King's College Medical School, London, UK. FAU - Hills, Robert K AU - Hills RK AD - Nuffield Department of Population Health, University of Oxford, Oxford, UK. FAU - Burnett, Alan K AU - Burnett AK AUID- ORCID: 0000-0003-1734-5817 AD - Paul O'Gorman Leukaemia Research Centre, University of Glasgow, Glasgow, UK. FAU - Russell, Nigel H AU - Russell NH AD - Department of Haematology, Nottingham University Hospitals NHS Trust, Nottingham, UK. FAU - Fitzgibbon, Jude AU - Fitzgibbon J AUID- ORCID: 0000-0002-9069-1866 AD - Barts Cancer Institute, Queen Mary University of London, London, UK. FAU - Larson, Richard A AU - Larson RA AUID- ORCID: 0000-0001-9168-3203 AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Le Beau, Michelle M AU - Le Beau MM AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Stock, Wendy AU - Stock W AD - Section of Pediatric Hematology and Oncology, University of Chicago, Chicago, IL, USA. FAU - Heidenreich, Olaf AU - Heidenreich O AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Alharbi, Abrar AU - Alharbi A AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Allsup, David J AU - Allsup DJ AUID- ORCID: 0000-0001-6159-6109 AD - Centre for Atherothrombosis and Metabolic Disease, Hull York Medical School, Hull, UK. FAU - Houlston, Richard S AU - Houlston RS AUID- ORCID: 0000-0002-5268-0242 AD - Division of Genetics and Epidemiology, The Institute of Cancer Research, London, UK. FAU - Norden, Jean AU - Norden J AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Dickinson, Anne M AU - Dickinson AM AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Douglas, Elisabeth AU - Douglas E AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Lendrem, Clare AU - Lendrem C AUID- ORCID: 0000-0002-9435-7398 AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Daly, Ann K AU - Daly AK AD - Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. FAU - Palm, Louise AU - Palm L AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Piechocki, Kim AU - Piechocki K AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Jeffries, Sally AU - Jeffries S AD - West Midlands Regional Genetics Laboratory, Birmingham Women's Hospital, Birmingham, UK. FAU - Bornhäuser, Martin AU - Bornhäuser M AD - Department of Haematological Medicine, The Rayne Institute, King's College London, London, UK. AD - National Center for Tumor Diseases NCT, Partner site Dresden, Dresden, Germany. AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Röllig, Christoph AU - Röllig C AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Altmann, Heidi AU - Altmann H AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Ruhnke, Leo AU - Ruhnke L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Kunadt, Desiree AU - Kunadt D AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Wagenführ, Lisa AU - Wagenführ L AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. FAU - Cordell, Heather J AU - Cordell HJ AUID- ORCID: 0000-0002-1879-5572 AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK. FAU - Darlay, Rebecca AU - Darlay R AD - Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK. FAU - Andersen, Mette K AU - Andersen MK AD - Department of Clinical Genetics, University Hospital Rigshospitalet, Copenhagen, Denmark. FAU - Fontana, Maria C AU - Fontana MC AD - Institute of Hematology "L. and A. Seràgnoli", University of Bologna, Bologna, Italy. AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Martinelli, Giovanni AU - Martinelli G AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Marconi, Giovanni AU - Marconi G AUID- ORCID: 0000-0001-6309-0515 AD - IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy. FAU - Sanz, Miguel A AU - Sanz MA AUID- ORCID: 0000-0003-1489-1177 AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cervera, José AU - Cervera J AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Gómez-Seguí, Inés AU - Gómez-Seguí I AD - Hematology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain. AD - CIBERONC, Instituto de Salud Carlos III, Madrid, Spain. FAU - Cluzeau, Thomas AU - Cluzeau T AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Moreilhon, Chimène AU - Moreilhon C AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Raynaud, Sophie AU - Raynaud S AD - Hematology department, Cote d'Azur University, CHU of Nice, Nice, France. FAU - Sill, Heinz AU - Sill H AUID- ORCID: 0000-0003-0993-4371 AD - Division of Hematology, Medical University of Graz, Graz, Austria. FAU - Voso, Maria Teresa AU - Voso MT AUID- ORCID: 0000-0002-6164-4761 AD - Università di Roma Tor Vergata, Dipartimento di Biomedicina e Prevenzione, Rome, Italy. FAU - Lo-Coco, Francesco AU - Lo-Coco F AD - Università di Roma Tor Vergata, Dipartimento di Biomedicina e Prevenzione, Rome, Italy. FAU - Dombret, Hervé AU - Dombret H AD - Hôpital Saint-Louis, Institut Universitaire d'Hématologie, Université Paris Diderot, Paris, France. FAU - Cheok, Meyling AU - Cheok M AUID- ORCID: 0000-0002-7820-8026 AD - Univ. Lille, Inserm, CHU Lille, UMR-S 1172 - JPArc - Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer, F-59000, Lille, France. FAU - Preudhomme, Claude AU - Preudhomme C AD - Univ. Lille, Inserm, CHU Lille, UMR-S 1172 - JPArc - Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer, F-59000, Lille, France. FAU - Gale, Rosemary E AU - Gale RE AD - Department of Haematology, University College London Cancer Institute, London, UK. FAU - Linch, David AU - Linch D AD - Department of Haematology, University College London Cancer Institute, London, UK. FAU - Gaal-Wesinger, Julia AU - Gaal-Wesinger J AD - 1st Department of Internal Medicine, Semmewleis University, Budapest, Hungary. FAU - Masszi, Andras AU - Masszi A AD - 3rd Department of Internal Medicine, Semmewleis University, Budapest, Hungary. FAU - Nowak, Daniel AU - Nowak D AUID- ORCID: 0000-0001-7130-7921 AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Hofmann, Wolf-Karsten AU - Hofmann WK AD - Department of Hematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany. FAU - Gilkes, Amanda AU - Gilkes A AD - Department of Haematology, University of Cardiff, Cardiff, UK. FAU - Porkka, Kimmo AU - Porkka K AD - Helsinki University Hospital Comprehensive Cancer Center, Hematology Research Unit Helsinki, University of Helsinki, Helsinki, Finland. FAU - Milosevic Feenstra, Jelena D AU - Milosevic Feenstra JD AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Kralovics, Robert AU - Kralovics R AD - Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria. FAU - Grimwade, David AU - Grimwade D AD - Department of Medical and Molecular Genetics, King's College Medical School, London, UK. FAU - Meggendorfer, Manja AU - Meggendorfer M AD - MLL Munich Leukemia Laboratory, Munich, Germany. FAU - Haferlach, Torsten AU - Haferlach T AD - MLL Munich Leukemia Laboratory, Munich, Germany. FAU - Krizsán, Szilvia AU - Krizsán S AUID- ORCID: 0000-0002-1782-9275 AD - HCEMM-SE Molecular Oncohematology Research Group, 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. FAU - Bödör, Csaba AU - Bödör C AUID- ORCID: 0000-0002-0729-692X AD - HCEMM-SE Molecular Oncohematology Research Group, 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary. FAU - Stölzel, Friedrich AU - Stölzel F AUID- ORCID: 0000-0003-0653-5332 AD - Medizinische Klinik und Poliklinik I, University Hospital Carl Gustav Carus Dresden, Technical University of Dresden, Dresden, Germany. friedrich.stoelzel@uniklinikum-dresden.de. FAU - Onel, Kenan AU - Onel K AUID- ORCID: 0000-0002-2021-6250 AD - Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA. kenan.onel@mssm.edu. FAU - Allan, James M AU - Allan JM AUID- ORCID: 0000-0002-7580-5087 AD - Translational and Clinical Research Institute, Newcastle University Centre for Cancer, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK. james.allan@newcastle.ac.uk. LA - eng GR - MR/T004231/1/MRC_/Medical Research Council/United Kingdom GR - MR/P002005/1/MRC_/Medical Research Council/United Kingdom GR - G0700052/MRC_/Medical Research Council/United Kingdom GR - R01 HL102278/HL/NHLBI NIH HHS/United States GR - 25643/CRUK_/Cancer Research UK/United Kingdom GR - 24375/CRUK_/Cancer Research UK/United Kingdom GR - R03 CA188733/CA/NCI NIH HHS/United States PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't DEP - 20211029 PL - England TA - Nat Commun JT - Nature communications JID - 101528555 RN - 0 (HLA Antigens) RN - EC 1.1.1.21 (AKR1B1 protein, human) RN - EC 1.1.1.21 (Aldehyde Reductase) SB - IM EIN - Nat Commun. 2022 Jan 4;13(1):2. doi: 10.1038/s41467-021-27679-6. PMID: 34983928 MH - Aldehyde Reductase/genetics MH - Case-Control Studies MH - Genetic Predisposition to Disease MH - Genome-Wide Association Study MH - Genotype MH - HLA Antigens/*genetics MH - Humans MH - Leukemia, Myeloid, Acute/*genetics/mortality MH - Middle Aged MH - *Polymorphism, Single Nucleotide MH - Reproducibility of Results MH - White People/genetics PMC - PMC8556284 COIS- The authors declare no competing interests. EDAT- 2021/10/31 06:00 MHDA- 2021/12/01 06:00 PMCR- 2021/10/29 CRDT- 2021/10/30 05:47 PHST- 2020/01/30 00:00 [received] PHST- 2021/10/01 00:00 [accepted] PHST- 2021/10/30 05:47 [entrez] PHST- 2021/10/31 06:00 [pubmed] PHST- 2021/12/01 06:00 [medline] PHST- 2021/10/29 00:00 [pmc-release] AID - 10.1038/s41467-021-26551-x [pii] AID - 26551 [pii] AID - 10.1038/s41467-021-26551-x [doi] PST - epublish SO - Nat Commun. 2021 Oct 29;12(1):6233. doi: 10.1038/s41467-021-26551-x. PMID- 31876615 OWN - NLM STAT- MEDLINE DCOM- 20210209 LR - 20210209 IS - 1532-0987 (Electronic) IS - 0891-3668 (Linking) VI - 39 IP - 4 DP - 2020 Apr TI - Systematic Review and Meta-analysis of Postlicensure Observational Studies on Human Papillomavirus Vaccination and Autoimmune and Other Rare Adverse Events. PG - 287-293 LID - 10.1097/INF.0000000000002569 [doi] AB - BACKGROUND: Because of the limited number of subjects in prelicensure studies, autoimmune diseases and other rare adverse effects of vaccines may go undetected. Since 2006, millions of human papillomavirus (HPV) vaccine doses have been distributed and a considerable amount of postlicensure safety data has been generated. The objective of this study was to review available HPV postlicensure safety studies and to summarize risk estimates of autoimmune and other rare diseases. METHODS: For this systematic review and meta-analysis, we searched literature databases to identify any postlicensure safety studies related to HPV vaccination and autoimmune adverse events from inception to April 16, 2019. Pooled risk estimates were computed using fixed- or random-effects models if at least 2 estimates per disease and per HPV vaccine were available. RESULTS: Twenty-two studies met our inclusion criteria. The studies applied various methodologies and used different types of data sources and outcome definitions. Quadrivalent HPV vaccine (4vHPV) was most commonly assessed. Type 1 diabetes mellitus, immune thrombocytopenia purpura and thyroiditis diseases were most frequently reported. The meta-analysis was conducted on 35 diseases corresponding to 48 pooled risk estimates. Majority of the pooled estimates showed no significant effect (n = 43). Three negative (paralysis, immune thrombocytopenia purpura and chronic fatigue syndrome) and 2 positive (Hashimoto and Raynaud diseases) associations were detected. CONCLUSION: Our study demonstrated an absence of clear association between HPV vaccines and autoimmune and other rare diseases. The review also highlights the need for more systematic collaborations to monitor rare safety adverse events. FAU - Willame, Corinne AU - Willame C AD - From the Julius Center for Health Sciences and Primary Care, University Medical Center, Utrecht, the Netherlands. FAU - Gadroen, Kartini AU - Gadroen K AD - Department of Medical Informatics, Erasmus MC, University Medical Center, Rotterdam, the Netherlands. FAU - Bramer, Wichor AU - Bramer W AD - Medical Library, Erasmus MC, University Medical Center, Rotterdam, the Netherlands. FAU - Weibel, Daniel AU - Weibel D AD - Department of Medical Informatics, Erasmus MC, University Medical Center, Rotterdam, the Netherlands. FAU - Sturkenboom, Miriam AU - Sturkenboom M AD - From the Julius Center for Health Sciences and Primary Care, University Medical Center, Utrecht, the Netherlands. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review PL - United States TA - Pediatr Infect Dis J JT - The Pediatric infectious disease journal JID - 8701858 RN - 0 (Papillomavirus Vaccines) SB - IM MH - Autoimmune Diseases/*etiology MH - Humans MH - Licensure MH - *Observational Studies as Topic MH - Papillomavirus Infections/prevention & control MH - Papillomavirus Vaccines/*adverse effects MH - Product Surveillance, Postmarketing MH - Vaccination/*adverse effects EDAT- 2019/12/27 06:00 MHDA- 2021/02/10 06:00 CRDT- 2019/12/27 06:00 PHST- 2019/12/27 06:00 [pubmed] PHST- 2021/02/10 06:00 [medline] PHST- 2019/12/27 06:00 [entrez] AID - 00006454-202004000-00006 [pii] AID - 10.1097/INF.0000000000002569 [doi] PST - ppublish SO - Pediatr Infect Dis J. 2020 Apr;39(4):287-293. doi: 10.1097/INF.0000000000002569. PMID- 36707842 OWN - NLM STAT- MEDLINE DCOM- 20230207 LR - 20240911 IS - 1478-6362 (Electronic) IS - 1478-6354 (Print) IS - 1478-6354 (Linking) VI - 25 IP - 1 DP - 2023 Jan 27 TI - Proteomic aptamer analysis reveals serum markers that characterize preclinical systemic sclerosis (SSc) patients at risk for progression toward definite SSc. PG - 15 LID - 10.1186/s13075-023-02989-w [doi] LID - 15 AB - BACKGROUND: The study of molecular mechanisms characterizing disease progression may be relevant to get insights into systemic sclerosis (SSc) pathogenesis and to intercept patients at very early stage. We aimed at investigating the proteomic profile of preclinical systemic sclerosis (PreSSc) via a discovery/validation two-step approach. METHODS: SOMAcan aptamer-based analysis was performed on a serum sample of 13 PreSSc (discovery cohort) according to 2001 LeRoy and Medsger criteria (characterized solely by Raynaud phenomenon plus a positive nailfold capillaroscopy and SSc-specific antibodies without any other sign of definite disease) and 8 healthy controls (HCs) age, gender, and ethnicity matched. Prospective data were available up to 4±0.6 years to determine the progression to definite SSc according to the EULAR/ACR 2013 classification criteria. In proteins with relative fluorescence units (RFU) > |1.5|-fold vs HCs values, univariate analysis was conducted via bootstrap aggregating models to determine the predicting accuracy (progression vs non-progression) of categorized baseline protein values. Gene Ontologies (GO terms) and Reactome terms of significant proteins at the adjusted 0.05 threshold were explored. Significant proteins from the discovery cohort were finally validated via ELISAs in an independent validation cohort of 50 PreSSc with clinical prospective data up to 5 years. Time-to-event analysis for interval-censored data was used to evaluate disease progression. RESULTS: In the discovery cohort, 286 out of 1306 proteins analyzed via SomaScan, were differentially expressed versus HCs. Ten proteins were significantly associated with disease progression; analysis through GO and Reactome showed differentially enriched pathways involving angiogenesis, endothelial cell chemotaxis, and endothelial cell chemotaxis to fibroblast growth factor (FGF). In the validation cohort, endostatin (HR=10.23, CI95=2.2-47.59, p=0.003) was strongly associated with disease progression, as well as bFGF (HR=0.84, CI95=0.709-0.996, p=0.045) and PAF-AHβ (HR=0.372, CI95=0.171-0.809, p=0.013) CONCLUSIONS: A distinct protein profile characterized PreSSc from HCs and proteins associated with hypoxia, vasculopathy, and fibrosis regulation are linked with the progression from preclinical to definite SSc. These proteins, in particular endostatin, can be regarded both as markers of severity and molecules with pathogenetic significance as well as therapeutic targets. CI - © 2023. The Author(s). FAU - Bellocchi, Chiara AU - Bellocchi C AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. FAU - Assassi, Shervin AU - Assassi S AD - Department of Internal Medicine - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, USA. FAU - Lyons, Marka AU - Lyons M AD - Department of Internal Medicine - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, USA. FAU - Marchini, Maurizio AU - Marchini M AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. FAU - Mohan, Chandra AU - Mohan C AD - Department of Biomedical Engineering, University of Houston, Houston, TX, USA. FAU - Santaniello, Alessandro AU - Santaniello A AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. FAU - Beretta, Lorenzo AU - Beretta L AD - Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. lorenzo.beretta@policlinico.mi.it. LA - eng GR - R56 AR078211/AR/NIAMS NIH HHS/United States GR - R61 AR078078/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20230127 PL - England TA - Arthritis Res Ther JT - Arthritis research & therapy JID - 101154438 RN - 0 (Biomarkers) RN - 0 (Endostatins) SB - IM MH - Humans MH - Biomarkers MH - Disease Progression MH - Endostatins MH - Microscopic Angioscopy MH - Prospective Studies MH - *Proteomics MH - *Scleroderma, Systemic/blood/genetics/metabolism/pathology PMC - PMC9881382 OTO - NOTNLM OT - Disease progression OT - Preclinical stage OT - Proteomic OT - Systemic sclerosis OT - Vasculopathy COIS- The authors declare that they have no competing interests. EDAT- 2023/01/29 06:00 MHDA- 2023/02/01 06:00 PMCR- 2023/01/27 CRDT- 2023/01/28 00:10 PHST- 2022/09/11 00:00 [received] PHST- 2023/01/04 00:00 [accepted] PHST- 2023/01/28 00:10 [entrez] PHST- 2023/01/29 06:00 [pubmed] PHST- 2023/02/01 06:00 [medline] PHST- 2023/01/27 00:00 [pmc-release] AID - 10.1186/s13075-023-02989-w [pii] AID - 2989 [pii] AID - 10.1186/s13075-023-02989-w [doi] PST - epublish SO - Arthritis Res Ther. 2023 Jan 27;25(1):15. doi: 10.1186/s13075-023-02989-w. PMID- 34479510 OWN - NLM STAT- MEDLINE DCOM- 20210913 LR - 20250103 IS - 1471-2431 (Electronic) IS - 1471-2431 (Linking) VI - 21 IP - 1 DP - 2021 Sep 3 TI - Novel CLCN4 variant associated with syndromic X-linked intellectual disability in a Chinese girl: a case report. PG - 384 LID - 10.1186/s12887-021-02860-4 [doi] LID - 384 AB - BACKGROUND: The Raynaud-Claes type of X-linked syndromic mental retardation (MRXSRC) is a very rare condition, by intellectual disability ranged from borderline to profound, impaired language development, brain abnormalities, facial dysmorphisms and seizures. MRXSRC is caused by variants in CLCN4 which encodes the 2Cl(-)/H(+) exchanger ClC-4 prominently expressed in brain. CASE PRESENTATION: We present a 3-year-old Chinese girl with intellectual disability, dysmorphic features, brain abnormalities, significant language impairment and autistic features. Brain magnetic resonance imaging (MRI) showed a thin corpus callosum, a mega cisterna magna and ventriculomegaly. Whole exome sequencing (WES) was performed to detect the molecular basis of the disease. It was confirmed that this girl carried a novel heterozygous missense variant (c.1343C > T, p.Ala448Val) of CLCN4 gene, inherited from her mother. This variant has not been registered in public databases and was predicted to be pathogenic by multiple in silico prediction tools. CONCLUSION: Our investigation expands the phenotype spectrum for CLCN4 variants with syndromic X-linked intellectual disability, which help to improve the understanding of CLCN4-related intellectual disability and will help in genetic counselling for this family. CI - © 2021. The Author(s). FAU - Xu, Xin AU - Xu X AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, 210008, Jiangsu Province, China. FAU - Lu, Fen AU - Lu F AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, 210008, Jiangsu Province, China. FAU - Zhang, Li AU - Zhang L AUID- ORCID: 0000-0001-5755-4037 AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, 210008, Jiangsu Province, China. zhangli_0612@163.com. FAU - Li, Hongying AU - Li H AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, 210008, Jiangsu Province, China. FAU - Du, Senjie AU - Du S AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, 210008, Jiangsu Province, China. FAU - Tang, Jian AU - Tang J LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210903 PL - England TA - BMC Pediatr JT - BMC pediatrics JID - 100967804 RN - 0 (CLCN4 protein, human) RN - 0 (Chloride Channels) SB - IM MH - Child, Preschool MH - China MH - Chloride Channels/genetics MH - Female MH - Genes, X-Linked MH - Humans MH - *Intellectual Disability/genetics MH - *X-Linked Intellectual Disability/genetics MH - Mutation MH - Pedigree MH - Phenotype MH - Exome Sequencing PMC - PMC8414764 OTO - NOTNLM OT - CLCN4 gene OT - Case report OT - Exome sequencing OT - Variants OT - X-linked syndromic mental retardation COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2021/09/05 06:00 MHDA- 2021/09/14 06:00 PMCR- 2021/09/03 CRDT- 2021/09/04 05:24 PHST- 2021/05/10 00:00 [received] PHST- 2021/08/26 00:00 [accepted] PHST- 2021/09/04 05:24 [entrez] PHST- 2021/09/05 06:00 [pubmed] PHST- 2021/09/14 06:00 [medline] PHST- 2021/09/03 00:00 [pmc-release] AID - 10.1186/s12887-021-02860-4 [pii] AID - 2860 [pii] AID - 10.1186/s12887-021-02860-4 [doi] PST - epublish SO - BMC Pediatr. 2021 Sep 3;21(1):384. doi: 10.1186/s12887-021-02860-4. PMID- 30187644 OWN - NLM STAT- MEDLINE DCOM- 20190909 LR - 20220408 IS - 1463-1326 (Electronic) IS - 1462-8902 (Linking) VI - 21 IP - 2 DP - 2019 Feb TI - Comprehensive analysis of body composition and insulin traits associated with intra-pancreatic fat deposition in healthy individuals and people with new-onset prediabetes/diabetes after acute pancreatitis. PG - 417-423 LID - 10.1111/dom.13523 [doi] AB - Current knowledge of biomarkers of intra-pancreatic fat deposition (IFD) is limited. We aimed to analyse comprehensively body composition and insulin traits as biomarkers of IFD in healthy normoglycaemic individuals as well as in individuals with new-onset prediabetes or diabetes after acute pancreatitis (NODAP). A total of 29 healthy individuals and 34 individuals with NODAP took part in this cross-sectional study. The studied biomarkers belonged to the following domains: body composition (anthropometric and MRI-derived variables); indices of insulin secretion; indices of insulin sensitivity; incretins and related peptides; and pancreatitis-related factors. All MRI-derived variables (including IFD) were measured using ImageJ software. Univariate and step-wise regression analyses were conducted to determine variables that best explained variance in IFD. Visceral fat volume and oxyntomodulin were the best biomarkers of IFD in normoglycaemic healthy individuals, contributing to 64% variance. The Raynaud index was the best biomarker of IFD in individuals with NODAP, contributing to 20% variance. Longitudinal studies are warranted to investigate the cause and effect relationship between oxyntomodulin and IFD in healthy individuals, as well as insulin sensitivity and IFD in individuals with NODAP. CI - © 2018 John Wiley & Sons Ltd. FAU - Singh, Ruma G AU - Singh RG AD - Department of Surgery, School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Nguyen, Ngoc N AU - Nguyen NN AD - Department of Surgery, School of Medicine, University of Auckland, Auckland, New Zealand. FAU - DeSouza, Steve V AU - DeSouza SV AD - Department of Surgery, School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Pendharkar, Sayali A AU - Pendharkar SA AD - Department of Surgery, School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Petrov, Maxim S AU - Petrov MS AUID- ORCID: 0000-0002-5923-9062 AD - Department of Surgery, School of Medicine, University of Auckland, Auckland, New Zealand. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20181005 PL - England TA - Diabetes Obes Metab JT - Diabetes, obesity & metabolism JID - 100883645 SB - IM MH - Acute Disease MH - Adiposity/physiology MH - Adult MH - Age of Onset MH - Body Composition/*physiology MH - Cross-Sectional Studies MH - Diabetes Complications/metabolism/pathology MH - Diabetes Mellitus/epidemiology/*metabolism/pathology MH - Female MH - Healthy Volunteers MH - Humans MH - *Insulin Resistance MH - Intra-Abdominal Fat/pathology MH - Lipid Metabolism/*physiology MH - Longitudinal Studies MH - Magnetic Resonance Imaging MH - Male MH - Pancreas/diagnostic imaging/*metabolism/pathology MH - Pancreatitis/diagnosis/epidemiology/*metabolism/pathology MH - Prediabetic State/complications/epidemiology/*metabolism/pathology OTO - NOTNLM OT - acute pancreatitis OT - body composition OT - diabetes OT - insulin sensitivity OT - intra-pancreatic fat OT - oxyntomodulin EDAT- 2018/09/07 06:00 MHDA- 2019/09/10 06:00 CRDT- 2018/09/07 06:00 PHST- 2018/07/03 00:00 [received] PHST- 2018/08/21 00:00 [revised] PHST- 2018/09/03 00:00 [accepted] PHST- 2018/09/07 06:00 [pubmed] PHST- 2019/09/10 06:00 [medline] PHST- 2018/09/07 06:00 [entrez] AID - 10.1111/dom.13523 [doi] PST - ppublish SO - Diabetes Obes Metab. 2019 Feb;21(2):417-423. doi: 10.1111/dom.13523. Epub 2018 Oct 5. PMID- 28479486 OWN - NLM STAT- MEDLINE DCOM- 20171129 LR - 20181202 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 16 IP - 7 DP - 2017 Jul TI - Integrated Diagnosis Project for Inflammatory Myopathies: An association between autoantibodies and muscle pathology. PG - 693-700 LID - S1568-9972(17)30123-4 [pii] LID - 10.1016/j.autrev.2017.05.003 [doi] AB - Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. The clinical phenotypes of inflammatory myopathies are characterized by various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, since the correlation between histological features and autoantibodies had not been fully elucidated, we created the "Integrated Diagnosis Project for Inflammatory Myopathies" in October 2010. Based on our work and previous studies, the three major subsets of inflammatory myopathies defined by autoantibodies are immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome, and dermatomyositis. IMNM is the pathological entity, characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration. The detection of autoantibodies against signal recognition particles or 3-hydroxy-3-methylglutaryl-coenzyme A reductase is important for the diagnosis of IMNM. Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is the clinical entity based on the presence of aminoacyl transfer RNA synthetase antibodies. Perifascicular necrosis is a distinctive hallmark of antisynthetase syndrome in muscle pathology. The diagnosis of dermatomyositis is usually based on clinical features of typical skin rash. Several autoantibodies are associated with specific subsets of dermatomyositis. Myxovirus resistance A expression in the myofiber cytoplasm has a better sensitivity for the diagnosis of dermatomyositis compared to perifascicular atrophy. The screening of autoantibodies has clinical relevance for managing patients with inflammatory myopathies. CI - Copyright © 2017 Elsevier B.V. All rights reserved. FAU - Suzuki, Shigeaki AU - Suzuki S AD - Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. Electronic address: sgsuzuki@z3.keio.jp. FAU - Uruha, Akinori AU - Uruha A AD - Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8502, Japan; Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8581, Japan. FAU - Suzuki, Norihiro AU - Suzuki N AD - Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. FAU - Nishino, Ichizo AU - Nishino I AD - Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8502, Japan; Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8581, Japan. LA - eng PT - Journal Article PT - Review DEP - 20170504 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Autoantibodies) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) SB - IM MH - Amino Acyl-tRNA Synthetases/immunology MH - Animals MH - Autoantibodies/*immunology MH - Humans MH - Muscle, Skeletal/immunology/pathology MH - Myositis/diagnosis/*immunology/pathology OTO - NOTNLM OT - Autoantibodies OT - Classification OT - Immune-mediated necrotizing myopathy OT - Immunoprecipitation OT - Inflammatory myopathies OT - Muscle pathology EDAT- 2017/05/10 06:00 MHDA- 2017/12/01 06:00 CRDT- 2017/05/09 06:00 PHST- 2017/03/23 00:00 [received] PHST- 2017/03/25 00:00 [accepted] PHST- 2017/05/10 06:00 [pubmed] PHST- 2017/12/01 06:00 [medline] PHST- 2017/05/09 06:00 [entrez] AID - S1568-9972(17)30123-4 [pii] AID - 10.1016/j.autrev.2017.05.003 [doi] PST - ppublish SO - Autoimmun Rev. 2017 Jul;16(7):693-700. doi: 10.1016/j.autrev.2017.05.003. Epub 2017 May 4. PMID- 37915665 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231103 IS - 2049-0801 (Print) IS - 2049-0801 (Electronic) IS - 2049-0801 (Linking) VI - 85 IP - 11 DP - 2023 Nov TI - Antineutrophil cytoplasmic antibody-associated vasculitis with systemic sclerosis: a fatal case report. PG - 5770-5775 LID - 10.1097/MS9.0000000000001347 [doi] AB - INTRODUCTION AND IMPORTANCE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical manifestations and prognosis of vasculitis in systemic sclerosis depend on organ involvement. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers get along with purpuric vasculitis in some cases reports. Reports had found that survival in those with pulmonary-renal syndrome is poor. However, high-dose corticosteroids and cyclophosphamide increase the survival percent in those patients. CASE PRESENTATION: An 81-year-old female was admitted for newly diagnosed acute renal failure and highly elevated C-reactive protein levels. She was diagnosed with systemic sclerosis 8 years previously, with a 3-year history of interstitial lung disease, and a 2-year history of pulmonary hypertension. Treatment included home oxygen on demand, prednisone 5 mg/day, and azathioprine 75 mg daily. On physical examination, she had sclerodactyly, both extremities ulcers, severe livedo reticularis, and hyperpigmented papules on her hand and feet. Laboratory findings included a markedly positive MPO (p-ANCA), and anti-Scl-70. She was treated with pulse methylprednisolone without any improvement. After a day, she developed anuria and became comatose. Then, she developed cardiac arrest, leading to death. CLINICAL DISCUSSION: The presence of ANCA in systemic sclerosis patients ranges from 2.5 to 9% of systemic sclerosis patients. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers. The treatment with high-dose corticosteroids and cyclophosphamide is benefit. Survival in those with pulmonary-renal syndrome is poor. CONCLUSION: The presence of ANCA-associated vasculitis is rarely reported with scleroderma. It occurs most commonly in women with limited or Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) variants of scleroderma, as well as those with overlap features. Severe manifestations including pulmonary-renal syndrome and death may occur. CI - Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. FAU - Khalayli, Naram AU - Khalayli N AD - Damascus University, Damascus, Syrian Arab Republic. FAU - Ibrahim, Raghad AU - Ibrahim R AD - Damascus University, Damascus, Syrian Arab Republic. FAU - Ibrahim, Rahaf AU - Ibrahim R AD - Damascus University, Damascus, Syrian Arab Republic. FAU - Kudsi, Maysoun AU - Kudsi M AD - Damascus University, Damascus, Syrian Arab Republic. LA - eng PT - Journal Article DEP - 20231004 PL - England TA - Ann Med Surg (Lond) JT - Annals of medicine and surgery (2012) JID - 101616869 PMC - PMC10617915 OTO - NOTNLM OT - ANCA-associated vasculitis OT - pulmonary-renal syndrome OT - sclerodactylia OT - systemic sclerosis COIS- The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/11/02 06:43 MHDA- 2023/11/02 06:44 PMCR- 2023/10/04 CRDT- 2023/11/02 04:03 PHST- 2023/07/28 00:00 [received] PHST- 2023/09/16 00:00 [accepted] PHST- 2023/11/02 06:44 [medline] PHST- 2023/11/02 06:43 [pubmed] PHST- 2023/11/02 04:03 [entrez] PHST- 2023/10/04 00:00 [pmc-release] AID - AMSU-D-23-01633 [pii] AID - 10.1097/MS9.0000000000001347 [doi] PST - epublish SO - Ann Med Surg (Lond). 2023 Oct 4;85(11):5770-5775. doi: 10.1097/MS9.0000000000001347. eCollection 2023 Nov. PMID- 36089600 OWN - NLM STAT- MEDLINE DCOM- 20220913 LR - 20250728 IS - 1546-0096 (Electronic) IS - 1546-0096 (Linking) VI - 20 IP - 1 DP - 2022 Sep 11 TI - Juvenile diabetes and systemic sclerosis: just a coincidence? PG - 81 LID - 10.1186/s12969-022-00741-3 [doi] LID - 81 AB - BACKGROUND: Limited joint mobility (LJM), previously known as cheiroarthropathy, refers to the presence of reduced extension at the finger joints in people with diabetes and may be associated with scleroderma-like syndromes such as diabetic sclerodactyly. While scleroderma-like syndromes and LJM have been observed in patients with long-term diabetes and associated complications, the coexistence of diabetes with Juvenile systemic sclerosis (jSSc) is rarely described. CASE PRESENTATION: We describe the case of a 14-year-old boy with long-lasting type 1 diabetes (T1D) and suspected LJM associated with Raynaud phenomenon, sclerodactyly and tapering of the fingertips. A comprehensive work-up showed positive autoantibodies (ANA, anti-Ro-52, anti-Mi-2b), abnormal nailfold capillaroscopy with a scleroderma pattern, interstitial lung disease and cardiac involvement. The overall clinical picture was consistent with the diagnosis of jSSc. CONCLUSIONS: LJM can be the initial sign of underlying systemic sclerosis. Nailfold capillaroscopy may help differentiate jSSc from classical LJM in pediatric patients with T1D and finger contractures or skin induration of no clear origin. This case report provides a starting point for a novel hypothesis regarding the pathogenesis of jSSc. The association between T1D and jSSc may be more than a coincidence and could suggest a relationship between glucose metabolism, fibrosis and microangiopathy. CI - © 2022. The Author(s). FAU - Mastrangelo, Greta AU - Mastrangelo G AUID- ORCID: 0000-0002-3455-7304 AD - Division of Rheumatology, Department of Paediatrics, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada. greta.mastrangelo@gmail.com. FAU - Meneghel, Alessandra AU - Meneghel A AUID- ORCID: 0000-0002-3606-2436 AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Padova, Italy. FAU - Martini, Giorgia AU - Martini G AUID- ORCID: 0000-0003-3801-8903 AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Padova, Italy. FAU - Moretti, Carlo AU - Moretti C AD - Diabetology Unit, Department of Women's and Children's Health, University of Padova, Padova, Italy. FAU - Zulian, Francesco AU - Zulian F AUID- ORCID: 0000-0002-2479-3485 AD - Pediatric Rheumatology Unit, Department of Woman and Child Health, University of Padova, Padova, Italy. LA - eng PT - Case Reports PT - Journal Article DEP - 20220911 PL - England TA - Pediatr Rheumatol Online J JT - Pediatric rheumatology online journal JID - 101248897 RN - Juvenile systemic scleroderma RN - Juvenile-onset scleroderma SB - IM MH - Adolescent MH - Child MH - *Diabetes Mellitus, Type 1/complications MH - Humans MH - Male MH - Microscopic Angioscopy MH - Scleroderma, Localized MH - *Scleroderma, Systemic/complications/diagnosis PMC - PMC9465903 OTO - NOTNLM OT - Cheiroarthropathy OT - Dipeptidyl peptidase-4 OT - Limited joint mobility OT - Microangiopathy OT - Nailfold capillaroscopy OT - Sclerodactyly COIS- The authors declare that they have no competing interests. EDAT- 2022/09/12 06:00 MHDA- 2022/09/14 06:00 PMCR- 2022/09/11 CRDT- 2022/09/11 23:14 PHST- 2022/06/29 00:00 [received] PHST- 2022/09/06 00:00 [accepted] PHST- 2022/09/11 23:14 [entrez] PHST- 2022/09/12 06:00 [pubmed] PHST- 2022/09/14 06:00 [medline] PHST- 2022/09/11 00:00 [pmc-release] AID - 10.1186/s12969-022-00741-3 [pii] AID - 741 [pii] AID - 10.1186/s12969-022-00741-3 [doi] PST - epublish SO - Pediatr Rheumatol Online J. 2022 Sep 11;20(1):81. doi: 10.1186/s12969-022-00741-3. PMID- 35532771 OWN - NLM STAT- MEDLINE DCOM- 20220511 LR - 20220511 IS - 1751-7125 (Electronic) IS - 1540-9740 (Linking) VI - 20 IP - 2 DP - 2022 TI - Nephrogenic Systemic Fibrosis with Osseous Metaplasia in a Kidney-Pancreas Transplant Patient. PG - 145-148 AB - A French (Caucasian) woman with a history of nonobstructive hypertrophic cardiopathy, type 1 diabetes mellitus, cataract, and ante-hypophysary insufficiency had undergone multiple magnetic resonance imaging (MRI) studies. She had developed end-stage renal disease (ESRD) and had undergone hemodialysis for 10 years before receiving a kidney-pancreas allotransplantation at the age of 48 years. She received antithymocyte globulins as induction immunosuppression and steroids (5 mg/d), mycophenolate mofetil (2 g/d), and tacrolimus (5 mg/d) as maintenance immunosuppression. Following transplantation, she underwent a cerebral MRI with injection of a gadolinium-based contrast agent (GBCA) in the work-up for Schwartz-Bartter syndrome. Shortly thereafter, she progressively developed cutaneous infiltration, sclerosis, and hyperpigmentation on her extremities and back (Figure 1), firm nodules on the thighs and the right hand, and confluent papules on the back, all of which were asymptomatic. She had no facial involvement, sclerodactyly, periungual telangiectasias, Raynaud syndrome, or arthralgias. Histologic examination showed mild epidermal hyperplasia and a thickened dermis containing several fibroblasts and some histiocytes (Figure 2a). The alcian blue stain revealed increased dermal mucin deposits (Figure 3b). Remarkably, several round-to-ovoid, well-limited yellowish collagenous structures containing basophilic (elastic) fibers were seen in the dermis (Figures 2b, 2c, 3a, and 4a). These "elasto-collagenous balls" stained blue with Masson's trichrome stain (Figure 4c); the orcein stain confirmed the presence of elastic fibers within them (Figure 4b). Some orange-yellow elasto-collagenous balls contained osteocytes, indicative of osseous metaplasia (Figure 5); these were von Kossa stain-positive, highlighting calcium deposition (Figure 4d). Immunohistochemically, the dermal fibroblasts were variably CD34-positive. Factor XIIIa+ dermal dendrocytes and histiocytic, occasionally multinucleated, CD68+ cells were also seen. (SKINmed. 2022;20:145-148). FAU - de Lavillandre, Joann Le Borgne AU - de Lavillandre JLB AD - Department of Dermatology, Ed. Herriot Hospital, Lyon, France. FAU - Buron, Fanny AU - Buron F AD - Department of Nephrology, Ed. Herriot Hospital, Lyon, France. FAU - Ducroux, Emilie AU - Ducroux E AD - Department of Dermatology, Ed. Herriot Hospital, Lyon, France. FAU - Kanitakis, Jean AU - Kanitakis J AD - Department of Dermatology, Ed. Herriot Hospital, Lyon, France; jean.kanitakis@univ-lyon1.fr. LA - eng PT - Journal Article DEP - 20220430 PL - United States TA - Skinmed JT - Skinmed JID - 101168327 SB - IM MH - *Calcinosis MH - Female MH - Humans MH - Kidney/pathology MH - Metaplasia/pathology MH - Middle Aged MH - *Nephrogenic Fibrosing Dermopathy/etiology/pathology MH - *Pancreas Transplantation/adverse effects MH - *Skin Diseases/etiology/pathology EDAT- 2022/05/10 06:00 MHDA- 2022/05/12 06:00 CRDT- 2022/05/09 11:13 PHST- 2022/05/09 11:13 [entrez] PHST- 2022/05/10 06:00 [pubmed] PHST- 2022/05/12 06:00 [medline] PST - epublish SO - Skinmed. 2022 Apr 30;20(2):145-148. eCollection 2022. PMID- 31027135 OWN - NLM STAT- MEDLINE DCOM- 20190520 LR - 20221005 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 98 IP - 17 DP - 2019 Apr TI - Hypereosinophilic vasculitis: A case report. PG - e15392 LID - 10.1097/MD.0000000000015392 [doi] LID - e15392 AB - INTRODUCTION: The Revised International Chapel Hill Consensus Conference 2012 subdivides vasculitides based on combinations of features that separate different forms of vasculitis into definable categories. Hypereosinophilic vasculitis with sparing of the respiratory tract and renal system is a rare presentation that is yet to be described in the Revised International Chapel Hill Consensus Conference 2012 report that addresses nomenclature of vasculitides. This is a condition that involves a vascular injury due to either a primary eosinophilic vasculitis or an underlying connective tissue disease and it predisposes patients to a prothrombotic state. PATIENT CONCERNS: A 39-year-old patient presented with left hand digital ischemia, preceded by Raynaud phenomenon, and vasculitic rash. For 3 months, he was having digital ischemia affecting the left 2nd and 3rd digits in the form of pallor and gangrenous discoloration with a preceding history of a pinpoint pruritic rash affecting his lower limbs that extended to involve the trunk and upper limbs over a short period of time and responded to only a tapering dose of oral steroids. Examination revealed a delayed capillary refill in all left-hand digits and a weak left radial pulse but no bruit. The rest of his peripheral vascular examination was unremarkable. DIAGNOSIS: Investigations revealed an absolute eosinophilic count of 4.34 K/μL and erythrocyte sedimentation rate of 44 mm/h. A magnetic resonance angiogram showed a beaded appearance of the left ulnar artery distally and the radial artery branches in the left hand and subsequently was diagnosed with hypereosinophilic vasculitis. INTERVENTIONS: He was started on oral prednisone of 1 mg/kg daily orally tapering done as well as azathioprine for maintenance. OUTCOMES: Two weeks postdischarge, the patient was seen in the outpatient department where his ischemic symptoms improved, and his skin rash healed. Noticed improvement in his splinter hemorrhages was also detected. He continued to do well on 2 years follow-up CONCLUSION:: This case reflects the importance of frequent reevaluation for vasculitic diseases criteria and nomenclature. Hypereosinophilic vasculitis with absent respiratory and renal involvement is a rare presentation with scarce evidence to guide treatment. FAU - Alzayer, Husam AU - Alzayer H AD - Galway University Hospital, Galway, Ireland. FAU - Hasan, Manal Ahmed AU - Hasan MA AD - IAU-KFHU, SCFHS, Khobar, Saudi Arabia. LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Adult MH - Diagnosis, Differential MH - Humans MH - Hypereosinophilic Syndrome/*diagnosis/drug therapy MH - Male MH - Vasculitis/*diagnosis/drug therapy PMC - PMC6831164 EDAT- 2019/04/28 06:00 MHDA- 2019/05/21 06:00 PMCR- 2019/04/26 CRDT- 2019/04/28 06:00 PHST- 2019/04/28 06:00 [entrez] PHST- 2019/04/28 06:00 [pubmed] PHST- 2019/05/21 06:00 [medline] PHST- 2019/04/26 00:00 [pmc-release] AID - 00005792-201904260-00086 [pii] AID - MD-D-18-08075 [pii] AID - 10.1097/MD.0000000000015392 [doi] PST - ppublish SO - Medicine (Baltimore). 2019 Apr;98(17):e15392. doi: 10.1097/MD.0000000000015392. PMID- 29461592 OWN - NLM STAT- MEDLINE DCOM- 20191021 LR - 20191022 IS - 2284-0729 (Electronic) IS - 1128-3602 (Linking) VI - 22 IP - 3 DP - 2018 Feb TI - Health-related quality of life and psychosocial implications in testicular cancer survivors. A literature review. PG - 645-661 LID - 14290 [pii] LID - 10.26355/eurrev_201802_14290 [doi] AB - OBJECTIVE: In this review, we focused our attention on Quality of Life (QoL) of testicular cancer survivors (TCSs), in general and in the most relevant areas. Several key findings have been highlighted in our review. MATERIALS AND METHODS: PubMed, MEDLINE and PsycINFO databases were consulted to find published studies, from 1980 to May 2017, that met our inclusion criteria. RESULTS: The majority of studies investigated older adult TCSs, while few studies on adolescent and young adult patients were available. Many studies indicate that health-related QoL (HRQoL) is similar among the TCSs and the general population. Even if QoL deteriorates so clear at the time of diagnosis and throughout treatment, afterward returns to normal levels, as defined by the matched controls. However, there are numerous chronic conditions consequent to diagnosis and treatment of testicular cancer that plague survivors and affect QoL, like Raynaud-like phenomena, peripheral neuropathy, fatigue, anxiety, sexual, fertility and body image problems. Even if these problems can have no effects on the measures of global QoL, they have an impact on the quality of life. Differences between TCSs with and without a partner bring to different outcomes in the adjustments to cancer. CONCLUSIONS: It is necessary to identify TCSs with higher risks of poorer QoL outcomes, to focus interventions on the areas with the greatest impairments. Further researches should consider the effects of testicular cancer on the impaired areas, collecting more data to better identify survivor's needs and consequent interventions, with a special focus on adolescent and young adult TCSs. Other works are requested on therapies, preventive and ameliorative, to reduce chronic side effects of testicular cancer treatment. FAU - Cappuccio, F AU - Cappuccio F AD - Progetto ONCONET2.0 - Linea progettuale 14 per l'implementazione della prevenzione e diagnosi precoce del tumore alla prostata e testicolo - Regione Campania, Italy. cappuccio.francesca@tiscali.it. FAU - Rossetti, S AU - Rossetti S FAU - Cavaliere, C AU - Cavaliere C FAU - Iovane, G AU - Iovane G FAU - Taibi, R AU - Taibi R FAU - D'Aniello, C AU - D'Aniello C FAU - Imbimbo, C AU - Imbimbo C FAU - Facchini, S AU - Facchini S FAU - Abate, V AU - Abate V FAU - Barberio, D AU - Barberio D FAU - Facchini, G AU - Facchini G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - Italy TA - Eur Rev Med Pharmacol Sci JT - European review for medical and pharmacological sciences JID - 9717360 SB - IM MH - Cancer Survivors/*psychology MH - Chronic Disease MH - Humans MH - Male MH - *Quality of Life MH - Testicular Neoplasms/*complications/*psychology EDAT- 2018/02/21 06:00 MHDA- 2019/10/23 06:00 CRDT- 2018/02/21 06:00 PHST- 2018/02/21 06:00 [entrez] PHST- 2018/02/21 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] AID - 14290 [pii] AID - 10.26355/eurrev_201802_14290 [doi] PST - ppublish SO - Eur Rev Med Pharmacol Sci. 2018 Feb;22(3):645-661. doi: 10.26355/eurrev_201802_14290. PMID- 26419464 OWN - NLM STAT- MEDLINE DCOM- 20160706 LR - 20220311 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 10 DP - 2015 Sep 30 TI - Primary erythromelalgia: a review. PG - 127 LID - 10.1186/s13023-015-0347-1 [doi] LID - 127 AB - Primary erythromelalgia (PE ORPHA90026) is a rare autosomal dominant neuropathy characterized by the combination of recurrent burning pain, warmth and redness of the extremities. The incidence rate of PE ranges from 0.36 to 1.1 per 100,000 persons. Gender ratio differs according to different studies and no evidence showed a gender preference. Clinical onset of PE is often in the first decade of life. Burning pain is the most predominant symptom and is usually caused and precipitated by warmth and physical activities. Reported cases of PE contain both inherited and sporadic forms. Genetic etiology of PE is mutations on SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. Diagnosis of PE is made upon clinical manifestations and screening for mutations on SCN9A. Exclusion of several other treatable diseases/secondary erythromelalgia is also necessary because of the lack of biomarkers specifically for PE. Differential diagnoses can include Fabry disease, cellulites, Raynaud phenomenon, vasculitis and so on. Diagnostic methods often involve complete blood count, imaging studies and thermograph. Treatment for PE is unsatisfactory and highly individualized. Frequently used pain relieving drugs involve sodium channel blockers such as lidocaine, carbamazepine and mexiletine. Novel drugs such as PF-05089771 and TV-45070 could be promising in ameliorating pain symptoms due to their Nav1.7 selectivity. Patients' symptoms often worsen over time and many patients develop ulcerations and gangrenes caused by excessive exposure to low temperature in order to relieve pain. This review mainly focuses on PE and the causative gene SCN9A--its mutations and their effects on Nav1.7 channels' electrophysiological properties. We propose a genotype-channelopathy-phenotype correlation network underlying PE etiology which could provide guidance for future therapeutics. FAU - Tang, Zhaoli AU - Tang Z AD - Department of Neurology, Xiangya Hospital, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. zhaoli.tang@outlook.com. FAU - Chen, Zhao AU - Chen Z AD - Department of Neurology, Xiangya Hospital, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. czcf98@126.com. FAU - Tang, Beisha AU - Tang B AD - Department of Neurology, Xiangya Hospital, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. bstang7398@163.com. AD - Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. bstang7398@163.com. AD - State Key Lab of Medical Genetics, Central South University, 110 Xiangya road, Changsha, 410078, Hunan, China. bstang7398@163.com. FAU - Jiang, Hong AU - Jiang H AD - Department of Neurology, Xiangya Hospital, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. jianghong73868@126.com. AD - Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, 87 Xiangya road, Changsha, 410008, Hunan, China. jianghong73868@126.com. AD - State Key Lab of Medical Genetics, Central South University, 110 Xiangya road, Changsha, 410078, Hunan, China. jianghong73868@126.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20150930 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (NAV1.7 Voltage-Gated Sodium Channel) SB - IM MH - Erythromelalgia/*diagnosis/*genetics/therapy MH - Humans MH - Mutation/*genetics MH - NAV1.7 Voltage-Gated Sodium Channel/*genetics MH - Pain/diagnosis/genetics MH - Pain Management/methods PMC - PMC4589109 EDAT- 2015/10/01 06:00 MHDA- 2016/07/07 06:00 PMCR- 2015/09/30 CRDT- 2015/10/01 06:00 PHST- 2015/07/10 00:00 [received] PHST- 2015/09/24 00:00 [accepted] PHST- 2015/10/01 06:00 [entrez] PHST- 2015/10/01 06:00 [pubmed] PHST- 2016/07/07 06:00 [medline] PHST- 2015/09/30 00:00 [pmc-release] AID - 10.1186/s13023-015-0347-1 [pii] AID - 347 [pii] AID - 10.1186/s13023-015-0347-1 [doi] PST - epublish SO - Orphanet J Rare Dis. 2015 Sep 30;10:127. doi: 10.1186/s13023-015-0347-1. PMID- 20424579 OWN - NLM STAT- MEDLINE DCOM- 20101026 LR - 20100701 IS - 0172-780X (Print) IS - 0172-780X (Linking) VI - 31 IP - 2 DP - 2010 TI - The clinical study of POEMS syndrome in China. PG - 229-37 AB - OBJECTIVE: POEMS syndrome is a unique clinical entity. It was described by the presence of several typical characteristics as paraproteinemia, polyneuropathy, organomegaly, endocrinopathy, and skin changes. Few people reported characteristics of Chinese POEMS patients. PATIENTS AND METHODS: Retrospective evaluation of Chinese patients with POEMS syndrome was carried out to reveal clinical features and compare with foreign series reported previously. In addition to typical characteristics, Chinese patients often were presented with extravascular volume overload (84%), papilledema (44%), bone lesions (41%), raynaud phenomenon (31%) and Clubbing (22%). RESULTS: Clinical laboratory tests found most patients had increased erythrosedimentation rate, hyperlipidemia, liver disorder and renal involvement. 70% patients had hypothyroidism, including overt hypothyroidism (6 patients) and subclinical hypothyroidism (13 patients). High prevalence of positive antimitochondrial antibody (ANA) and positive antineutrophil cytoplasmic antibody (ANCA) was the more common phenomenon. Chinese patients with bone damage in the incidence are lower than in the West and Japanese reports. CONCLUSIONS: In summary, our study demonstrate the POEMS syndrome diagnostic criteria proposed by Dispenzieri et al is also more high applicability in the Chinese population.At the time of diagnosis, we should not pay attention on the typical characteristics of the disease, but also on the changes in thyroid, liver, kidney function and lipid metabolism. FAU - Zhang, Bin AU - Zhang B AD - Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and Department of Neurology, The Second Affiliated Hospital of GuangZhou Medical Universit, China. zhangbin19781130@yahoo.com.cn FAU - Song, XingWang AU - Song X FAU - Liang, Bin AU - Liang B FAU - Hou, QingHua AU - Hou Q FAU - Pu, ShuXiang AU - Pu S FAU - Ying, Jian Rui AU - Ying JR FAU - Gao, Cong AU - Gao C LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Sweden TA - Neuro Endocrinol Lett JT - Neuro endocrinology letters JID - 8008373 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Autoantibodies) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antibodies, Antineutrophil Cytoplasmic/blood MH - Autoantibodies/blood MH - Blood Sedimentation MH - China/epidemiology MH - Female MH - Humans MH - Hyperlipidemias/blood/epidemiology MH - Hypothyroidism/blood/epidemiology MH - Incidence MH - Kidney/physiopathology MH - Liver/physiopathology MH - Male MH - Middle Aged MH - Mitochondria/immunology MH - POEMS Syndrome/blood/*diagnosis/epidemiology/*physiopathology MH - Papilledema/epidemiology/physiopathology MH - Prevalence MH - Retrospective Studies EDAT- 2010/04/29 06:00 MHDA- 2010/10/27 06:00 CRDT- 2010/04/29 06:00 PHST- 2009/05/24 00:00 [received] PHST- 2009/09/27 00:00 [accepted] PHST- 2010/04/29 06:00 [entrez] PHST- 2010/04/29 06:00 [pubmed] PHST- 2010/10/27 06:00 [medline] AID - NEL310210A19 [pii] PST - ppublish SO - Neuro Endocrinol Lett. 2010;31(2):229-37. PMID- 33271402 OWN - NLM STAT- MEDLINE DCOM- 20210104 LR - 20260518 IS - 1879-1360 (Electronic) IS - 0022-3999 (Print) IS - 0022-3999 (Linking) VI - 140 DP - 2021 Jan TI - Factors associated with fears due to COVID-19: A Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 cohort study. PG - 110314 LID - S0022-3999(20)30876-X [pii] LID - 10.1016/j.jpsychores.2020.110314 [doi] AB - INTRODUCTION: No studies have examined factors associated with fear in any group of people vulnerable during COVID-19 due to pre-existing medical conditions. OBJECTIVE: To investigate factors associated with fear of consequences of COVID-19 among people living with a pre-existing medical condition, the autoimmune disease systemic sclerosis (SSc; scleroderma), including country. METHODS: Pre-COVID-19 data from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort were linked to COVID-19 data collected in April 2020. Multivariable linear regression was used to assess factors associated with continuous scores of the 10-item COVID-19 Fears Questionnaire for Chronic Medical Conditions, controlling for pre-COVID-19 anxiety symptoms. RESULTS: Compared to France (N = 156), COVID-19 Fear scores among participants from the United Kingdom (N = 50) were 0.12 SD (95% CI 0.03 to 0.21) higher; scores for Canada (N = 97) and the United States (N = 128) were higher, but not statistically significant. Greater interference of breathing problems was associated with higher fears due to COVID-19 (Standardized regression coefficient = 0.12, 95% CI 0.01 to 0.23). Participants with higher financial resources adequacy scores had lower COVID-19 Fear scores (Standardized coefficient = -0.18, 95% CI -0.28 to -0.09). CONCLUSIONS: Fears due to COVID-19 were associated with clinical and functional vulnerabilities in this chronically ill population. This suggests that interventions may benefit from addressing specific clinical issues that apply to specific populations. Financial resources, health policies and political influences may also be important. The needs of people living with chronic illness during a pandemic may differ depending on the social and political context in which they live. CI - Copyright © 2020 Elsevier Inc. All rights reserved. FAU - Wu, Yin AU - Wu Y AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, the Netherlands. FAU - Henry, Richard S AU - Henry RS AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Gagarine, Maria AU - Gagarine M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Harb, Sami AU - Harb S AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Bourgeault, Angelica AU - Bourgeault A AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Tao, Lydia AU - Tao L AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Carboni-Jiménez, Andrea AU - Carboni-Jiménez A AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Negeri, Zelalem AU - Negeri Z AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Patten, Scott AU - Patten S AD - Department of Community Health Sciences, University of Calgary, Calgary, Alberta, Canada; Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada; O'Brien Institute for Public Health, University of Calgary, Calgary, Alberta, Canada. FAU - Bartlett, Susan J AU - Bartlett SJ AD - Department of Medicine, McGill University, Montreal, Quebec, Canada; Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. FAU - Mouthon, Luc AU - Mouthon L AD - Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France; Service de Médecine Interne, Centre de Reference Maladies Systémiques Autoimmunes Rares d'Ile de France, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Varga, John AU - Varga J AD - Northwestern Scleroderma Program. Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. FAU - Benedetti, Andrea AU - Benedetti A AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Medicine, McGill University, Montreal, Quebec, Canada; Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada; Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Medicine, McGill University, Montreal, Quebec, Canada; Department of Psychology, McGill University, Montreal, Quebec, Canada; Department of Educational and Counselling Psychology, McGill University, Montreal, Quebec, Canada; Biomedical Ethics Unit, McGill University, Montreal, Quebec, Canada. Electronic address: brett.thombs@mcgill.ca. CN - SPIN Patient Advisors CN - SPIN Investigators LA - eng GR - KL2 TR003168/TR/NCATS NIH HHS/United States GR - UL1 TR003167/TR/NCATS NIH HHS/United States PT - Journal Article DEP - 20201125 PL - England TA - J Psychosom Res JT - Journal of psychosomatic research JID - 0376333 SB - IM MH - Adult MH - Aged MH - COVID-19/epidemiology/*psychology MH - Canada/epidemiology MH - Chronic Disease MH - Cohort Studies MH - *Fear MH - Female MH - France/epidemiology MH - Humans MH - Male MH - Middle Aged MH - Patient-Centered Care MH - Risk Factors MH - Scleroderma, Systemic/*therapy MH - Surveys and Questionnaires MH - United Kingdom/epidemiology MH - United States/epidemiology PMC - PMC7685938 OTO - NOTNLM OT - Anxiety OT - COVID-19 OT - Chronic medical condition OT - Fear OT - Mental health OT - Scleroderma OT - Systemic sclerosis COIS- All authors have completed the ICJME uniform disclosure form and declare: no support from any organization for the submitted work; no financial relationships with any organizations that might have an interest in the submitted work in the previous three years. All authors declare no other relationships or activities that could appear to have influenced the submitted work. No funder had any role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. FIR - Fortuné, Catherine IR - Fortuné C IRAD- Scleroderma Society of Ontario, Hamilton, Ontario, Canada. FIR - Gietzen, Amy IR - Gietzen A IRAD- Scleroderma Foundation, Tri-State Chapter, Binghamton, NY, USA. FIR - Guillot, Geneviève IR - Guillot G IRAD- Sclérodermie Québec, Longueuil, Quebec, Canada. FIR - Lewis, Nancy IR - Lewis N IRAD- Toronto, Ontario, Canada. FIR - Richard, Michelle IR - Richard M IRAD- Scleroderma Atlantic, Halifax, Nova Scotia, Canada. FIR - Sauvé, Maureen IR - Sauvé M IRAD- Scleroderma Society of Ontario, Hamilton, Ontario, Canada. FIR - Welling, Joep IR - Welling J IRAD- NVLE Dutch Patient Organization for Systemic Autoimmune Diseases, Utrecht, the Netherlands. FIR - Fligelstone, Kim IR - Fligelstone K IRAD- Scleroderma & Raynaud's UK, London, United Kingdom. FIR - Gottesman, Karen IR - Gottesman K IRAD- Scleroderma Foundation, Tri-State Chapter, Binghamton, NY, USA. FIR - Leite, Catarina IR - Leite C IRAD- University of Minho, Braga, Portugal. FIR - Pérez, Elisabet IR - Pérez E IRAD- Asociación Española de Esclerodermia, Madrid, Spain. FIR - Baron, Murray IR - Baron M IRAD- McGill University, Montreal, Quebec, Canada. FIR - Malcarne, Vanessa IR - Malcarne V IRAD- San Diego State University, San Diego, CA, USA. FIR - Mayes, Maureen D IR - Mayes MD IRAD- University of Texas McGovern School of Medicine, Houston, TX, USA. FIR - Nielson, Warren R IR - Nielson WR IRAD- St. Joseph's Health Care, London, ON, Canada. FIR - Riggs, Robert IR - Riggs R IRAD- San Diego State University, San Diego, CA, USA. FIR - Assassi, Shervin IR - Assassi S IRAD- University of Texas McGovern School of Medicine, Houston, TX, USA. FIR - Ells, Carolyn IR - Ells C IRAD- McGill University, Montreal, Quebec, Canada. FIR - van den Ende, Cornelia IR - van den Ende C IRAD- Sint Maartenskliniek, Nijmegen, the Netherlands. FIR - Frech, Tracy IR - Frech T IRAD- University of Utah, Salt Lake City, Utah, USA. FIR - Harel, Daphna IR - Harel D IRAD- New York University, New York, New York, USA. FIR - Hinchcliff, Monique IR - Hinchcliff M IRAD- Yale School of Medicine, New Haven, CT, USA. FIR - Hudson, Marie IR - Hudson M IRAD- McGill University, Montreal, Quebec, Canada. FIR - Johnson, Sindhu R IR - Johnson SR IRAD- Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Toronto, Ontario, Canada; University of Toronto, Toronto, Ontario, Canada. FIR - Larche, Maggie IR - Larche M IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Nguyen, Christelle IR - Nguyen C IRAD- Université Paris Descartes, Paris, France; Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Pope, Janet IR - Pope J IRAD- University of Western Ontario, London, Ontario, Canada. FIR - Rannou, François IR - Rannou F IRAD- Université Paris Descartes, Paris, France. FIR - Reyna, Tatiana Sofia Rodriguez IR - Reyna TSR IRAD- Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. FIR - Schouffoer, Anne A IR - Schouffoer AA IRAD- Leiden University Medical Center, Leiden, the Netherlands. FIR - Suarez-Almazor, Maria E IR - Suarez-Almazor ME IRAD- University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FIR - Agard, Christian IR - Agard C IRAD- Centre Hospitalier Universitaire - Hôtel-Dieu de Nantes, Nantes, France. FIR - Albert, Alexandra IR - Albert A IRAD- Université Laval, Quebec, Quebec, Canada. FIR - Bernstein, Elana J IR - Bernstein EJ IRAD- Columbia University, New York, NY, USA. FIR - Berthier, Sabine IR - Berthier S IRAD- Centre Hospitalier Universitaire - Hôtel-Dieu de Nantes, Nantes, France. FIR - Bissonnette, Lyne IR - Bissonnette L IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Bruns, Alessandra IR - Bruns A IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Carreira, Patricia IR - Carreira P IRAD- Servicio de Reumatologia del Hospital, 12 de Octubre, Madrid, Spain. FIR - Chaigne, Benjamin IR - Chaigne B IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Chung, Lorinda IR - Chung L IRAD- Stanford University, Stanford, CA, USA. FIR - Correia, Chase IR - Correia C IRAD- Northwestern University, Chicago, IL, USA. FIR - Denton, Christopher IR - Denton C IRAD- Royal Free London Hospital, London, UK. FIR - Domsic, Robyn IR - Domsic R IRAD- University of Pittsburgh, Pittsburgh, PA, USA. FIR - Dunne, James V IR - Dunne JV IRAD- St. Paul's Hospital and University of British Columbia, Vancouver, British Columbia, Canada. FIR - Dunogue, Bertrand IR - Dunogue B IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Farge-Bancel, Dominique IR - Farge-Bancel D IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Fortin, Paul R IR - Fortin PR IRAD- CHU de Québec - Université Laval, Quebec, Quebec, Canada. FIR - Gordon, Jessica IR - Gordon J IRAD- Hospital for Special Surgery, New York City, NY, USA. FIR - Granel-Rey, Brigitte IR - Granel-Rey B IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France; Aix Marseille Université, Marseille, France. FIR - Hatron, Pierre-Yves IR - Hatron PY IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Herrick, Ariane L IR - Herrick AL IRAD- University of Manchester, Salford Royal NHS Foundation Trust, Manchester, UK. FIR - Hoa, Sabrina IR - Hoa S IRAD- Centre hospitalier de l'université de Montréal - CHUM, Montreal, Quebec, Canada. FIR - Jones, Niall IR - Jones N IRAD- University of Alberta, Edmonton, Alberta, Canada. FIR - Fernandes, Artur Jose de B IR - Fernandes AJB IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Kafaja, Suzanne IR - Kafaja S IRAD- University of California, Los Angeles, CA, USA. FIR - Khalidi, Nader IR - Khalidi N IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Launay, David IR - Launay D IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Manning, Joanne IR - Manning J IRAD- Salford Royal NHS Foundation Trust, Salford, UK. FIR - Marie, Isabelle IR - Marie I IRAD- CHU Rouen, Hôpital de Bois-Guillaume, Rouen, France. FIR - Martin, Maria IR - Martin M IRAD- Servicio de Reumatologia del Hospital, 12 de Octubre, Madrid, Spain; François Maurier, Hôpitaux Privés de Metz, Hôpital Belle-Isle, Metz, France. FIR - Mekinian, Arsene IR - Mekinian A IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Melchor, Sheila IR - Melchor S IRAD- Servicio de Reumatologia del Hospital, 12 de Octubre, Madrid, Spain. FIR - Nikpour, Mandana IR - Nikpour M IRAD- St Vincent's Hospital and University of Melbourne, Melbourne, Victoria, Australia. FIR - Olagne, Louis IR - Olagne L IRAD- Centre Hospitalier Universitaire - Hôtel-Dieu de Nantes, Nantes, France. FIR - Proudman, Susanna IR - Proudman S IRAD- Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia, Australia. FIR - Régent, Alexis IR - Régent A IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Rivière, Sébastien IR - Rivière S IRAD- Assistance Publique - Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Robinson, David IR - Robinson D IRAD- University of Manitoba, Winnipeg, Manitoba, Canada. FIR - Rodriguez, Esther IR - Rodriguez E IRAD- Servicio de Reumatologia del Hospital, 12 de Octubre, Madrid, Spain. FIR - Roux, Sophie IR - Roux S IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Sobanski, Vincent IR - Sobanski V IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Steen, Virginia IR - Steen V IRAD- Georgetown University, Washington, DC, USA. FIR - Sutton, Evelyn IR - Sutton E IRAD- Dalhousie University, Halifax, Nova Scotia, Canada. FIR - Thorne, Carter IR - Thorne C IRAD- Southlake Regional Health Centre, Newmarket, Ontario, Canada. FIR - Wilcox, Pearce IR - Wilcox P IRAD- St. Paul's Hospital and University of British Columbia, Vancouver, British Columbia, Canada. FIR - Ayala, Mara Cañedo IR - Ayala MC IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Carboni-Jiménez, Andrea IR - Carboni-Jiménez A IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Gagarine, Maria IR - Gagarine M IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Nordlund, Julia IR - Nordlund J IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Østbø, Nora IR - Østbø N IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Rice, Danielle B IR - Rice DB IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Turner, Kimberly A IR - Turner KA IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Culos-Reed, Nicole IR - Culos-Reed N IRAD- University of Calgary, Alberta, Canada. FIR - Dyas, Laura IR - Dyas L IRAD- Scleroderma Foundation Michigan Chapter, Southfield, Michigan, USA. FIR - El-Baalbaki, Ghassan IR - El-Baalbaki G IRAD- Université du Québec à Montréal, Montreal, Quebec, Canada. FIR - Hebblethwaite, Shannon IR - Hebblethwaite S IRAD- Concordia University, Montreal, Quebec, Canada. FIR - Bustamante, Laura IR - Bustamante L IRAD- Concordia University, Montreal, Quebec, Canada. FIR - Duchek, Delaney IR - Duchek D IRAD- University of Calgary, Alberta, Canada. FIR - Ellis, Kelsey IR - Ellis K IRAD- University of Calgary, Alberta, Canada. EDAT- 2020/12/04 06:00 MHDA- 2021/01/05 06:00 PMCR- 2020/11/25 CRDT- 2020/12/03 20:11 PHST- 2020/10/04 00:00 [received] PHST- 2020/11/18 00:00 [revised] PHST- 2020/11/21 00:00 [accepted] PHST- 2020/12/04 06:00 [pubmed] PHST- 2021/01/05 06:00 [medline] PHST- 2020/12/03 20:11 [entrez] PHST- 2020/11/25 00:00 [pmc-release] AID - S0022-3999(20)30876-X [pii] AID - 110314 [pii] AID - 10.1016/j.jpsychores.2020.110314 [doi] PST - ppublish SO - J Psychosom Res. 2021 Jan;140:110314. doi: 10.1016/j.jpsychores.2020.110314. Epub 2020 Nov 25. PMID- 31422354 OWN - NLM STAT- MEDLINE DCOM- 20200409 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 78 IP - 12 DP - 2019 Dec TI - Revised European Scleroderma Trials and Research Group Activity Index is the best predictor of short-term severity accrual. PG - 1681-1685 LID - 10.1136/annrheumdis-2019-215787 [doi] AB - BACKGROUND: The European Scleroderma Trials and Research Group (EUSTAR) recently developed a preliminarily revised activity index (AI) that performed better than the European Scleroderma Study Group Activity Index (EScSG-AI) in systemic sclerosis (SSc). OBJECTIVE: To assess the predictive value for short-term disease severity accrual of the EUSTAR-AI, as compared with those of the EScSG-AI and of known adverse prognostic factors. METHODS: Patients with SSc from the EUSTAR database with a disease duration from the onset of the first non-Raynaud sign/symptom ≤5 years and a baseline visit between 2003 and 2014 were first extracted. To capture the disease activity variations over time, EUSTAR-AI and EScSG-AI adjusted means were calculated. The primary outcome was disease progression defined as a Δ≥1 in the Medsger's severity score and in distinct items at the 2-year follow-up visit. Logistic regression analysis was carried out to identify predictive factors. RESULTS: 549 patients were enrolled. At multivariate analysis, the EUSTAR-AI adjusted mean was the only predictor of any severity accrual and of that of lung and heart, skin and peripheral vascular disease over 2 years. CONCLUSION: The adjusted mean EUSTAR-AI has the best predictive value for disease progression and development of severe organ involvement over time in SSc. CI - © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Fasano, Serena AU - Fasano S AUID- ORCID: 0000-0002-4718-4551 AD - Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy serena.fasano@unicampania.it. FAU - Riccardi, Antonella AU - Riccardi A AD - Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Messiniti, Valentina AU - Messiniti V AD - Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy. FAU - Caramaschi, Paola AU - Caramaschi P AD - Department of Rheumatology, University of Verona, Verona, Italy. FAU - Rosato, Edoardo AU - Rosato E AUID- ORCID: 0000-0002-7417-8093 AD - Dipartimento di Medicina Traslazionale e di Precisione, Sapienza University of Rome, Roma, Italy. FAU - Maurer, Britta AU - Maurer B AUID- ORCID: 0000-0001-9385-8097 AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Smith, Vanessa AU - Smith V AD - Department of Rheumatology, University Hospital Ghent, Gent, Belgium. FAU - Siegert, Elise AU - Siegert E AD - Department of Rheumatology, Charit University Hospital, Berlin, Germany. FAU - De Langhe, Ellen AU - De Langhe E AD - Department of Development and Regeneration, Laboratory of Tissue Homeostasis and Disease, Skeletal Biology and Engineering Research Center, KU Leuven, Leuven, Belgium. FAU - Riccieri, Valeria AU - Riccieri V AD - Clinical Medicine and Therapy, Sapienza University of Rome, Rome, Italy. FAU - Airó, Paolo AU - Airó P AD - Rheumatology and Clinical Immunology Department, Spedali Civili di Brescia, Brescia, Italy. FAU - Mihai, Carina AU - Mihai C AD - Department of Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucarest, Romania. FAU - Avouac, Jerome AU - Avouac J AD - Department of Rheumatology, Paris Descartes University, Rheumatology A and INSER U1016, Cochin Hospital, Paris, France. FAU - Zanatta, Elisabetta AU - Zanatta E AD - Dipartimento di Medicina, DIMED, Universita degli Studi di Padova, Padova, Italy. FAU - Walker, Ulrich A AU - Walker UA AD - Department of Rheumatology, Basel University, Basel, Switzerland. FAU - Iannone, Florenzo AU - Iannone F AUID- ORCID: 0000-0003-0474-5344 AD - Department of Rheumatology, University of Bari, Bari, Italy. FAU - García De la Peña Lefebvre, Paloma AU - García De la Peña Lefebvre P AD - Department of Rheumatology, Ramon y Cajal University Hospital, Madrid, Spain. FAU - Distler, Jörg H W AU - Distler JHW AUID- ORCID: 0000-0001-7408-9333 AD - Department of Internal Medicine III, University of Erlangen, Erlangen, Germany. FAU - Vacca, Alessandra AU - Vacca A AD - Chair and Rheumatology Unit, University Clinic AOU Cagliari, Monserrato, Italy. FAU - Distler, Oliver AU - Distler O AUID- ORCID: 0000-0002-0546-8310 AD - Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Allanore, Yannick AU - Allanore Y AD - Department of Rheumatology, Paris Descartes University, Rheumatology A and INSER U1016, Cochin Hospital, Paris, France. FAU - Valentini, Gabriele AU - Valentini G AUID- ORCID: 0000-0002-7852-9137 AD - Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20190817 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - Clinical Trials as Topic/*methods MH - Disease Progression MH - Europe MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Prospective Studies MH - Scleroderma, Systemic/*diagnosis MH - Severity of Illness Index MH - Time Factors OTO - NOTNLM OT - autoimmune diseases OT - outcomes research OT - systemic sclerosis COIS- Competing interests: None declared. EDAT- 2019/08/20 06:00 MHDA- 2020/04/10 06:00 CRDT- 2019/08/19 06:00 PHST- 2019/05/28 00:00 [received] PHST- 2019/07/12 00:00 [revised] PHST- 2019/07/19 00:00 [accepted] PHST- 2019/08/20 06:00 [pubmed] PHST- 2020/04/10 06:00 [medline] PHST- 2019/08/19 06:00 [entrez] AID - S0003-4967(24)02088-0 [pii] AID - 10.1136/annrheumdis-2019-215787 [doi] PST - ppublish SO - Ann Rheum Dis. 2019 Dec;78(12):1681-1685. doi: 10.1136/annrheumdis-2019-215787. Epub 2019 Aug 17. PMID- 29728522 OWN - NLM STAT- MEDLINE DCOM- 20190812 LR - 20190812 IS - 1526-632X (Electronic) IS - 0028-3878 (Print) IS - 0028-3878 (Linking) VI - 90 IP - 23 DP - 2018 Jun 5 TI - Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies. PG - e2068-e2076 LID - 10.1212/WNL.0000000000005638 [doi] AB - OBJECTIVE: To define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM). RESULTS: Forty-one anti-PM/Scl-positive, 132 AS, 178 DM, and 135 IMNM patients were included. Although muscle weakness was a presenting feature in just 37% of anti-PM/Scl-positive patients, 93% eventually developed weakness. Unlike the other groups, anti-PM-Scl-positive patients had more severe weakness in arm abductors than hip flexors. Interstitial lung disease was a presenting feature in just 10% of anti-PM/Scl-positive patients, but occurred in 61% during follow-up; fewer patients with DM (13%, p < 0.001) and IMNM (6%, p < 0.001) and more patients with AS (80%, p < 0.05) developed interstitial lung disease during the course of disease. Mechanic's hands (80%), Raynaud syndrome (78%), sclerodactyly (66%), telangiectasias (66%), esophageal reflux disease (61%), subcutaneous edema (46%), puffy hands (39%), and calcinosis (39%) occurred more frequently in anti-PM/Scl-positive patients than in the other groups. Although 30% of anti-PM/Scl-positive patients met criteria for systemic sclerosis, less than 5% had renal crisis or finger ulcerations. No differences were found between patients with only anti-PM/Scl-100 or only anti-PM/Scl-75 autoantibodies. CONCLUSIONS: Unlike patients with DM, AS, or IMNM, anti-PM/Scl-positive patients have weaker arm abductors than hip flexors. Anti-PM/Scl-positive patients also have the most extensive extramuscular features. CI - © 2018 American Academy of Neurology. FAU - De Lorenzo, Rebecca AU - De Lorenzo R AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Pinal-Fernandez, Iago AU - Pinal-Fernandez I AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Huang, Wilson AU - Huang W AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Albayda, Jemima AU - Albayda J AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Tiniakou, Eleni AU - Tiniakou E AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Johnson, Cheilonda AU - Johnson C AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Milisenda, Jose C AU - Milisenda JC AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Casal-Dominguez, Maria AU - Casal-Dominguez M AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Corse, Andrea M AU - Corse AM AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Danoff, Sonye K AU - Danoff SK AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Christopher-Stine, Lisa AU - Christopher-Stine L AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. FAU - Paik, Julie J AU - Paik JJ AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. andrew.mammen@nih.gov jpaik1@jhmi.edu. FAU - Mammen, Andrew L AU - Mammen AL AD - From the National Institute of Arthritis and Musculoskeletal and Skin Diseases (R.D.L., I.P.-F., W.H., J.C.M., M.C.-D., A.L.M.), NIH, Bethesda; and Johns Hopkins University School of Medicine (I.P.-F., J.A., E.T., C.J., M.C.-D., A.M.C., S.K.D., L.C.-S., J.J.P., A.L.M.), Baltimore, MD. andrew.mammen@nih.gov jpaik1@jhmi.edu. LA - eng PT - Journal Article PT - Research Support, N.I.H., Intramural PT - Research Support, Non-U.S. Gov't DEP - 20180504 PL - United States TA - Neurology JT - Neurology JID - 0401060 RN - 0 (Autoantibodies) RN - EC 2.7.3.2 (Creatine Kinase) RN - EC 3.1.- (Exosome Multienzyme Ribonuclease Complex) RN - Antisynthetase syndrome MH - Adult MH - Aged MH - Autoantibodies/*blood MH - Autoimmune Diseases/*blood/diagnostic imaging/therapy MH - Cohort Studies MH - Creatine Kinase/blood MH - Dermatomyositis/*blood/diagnostic imaging/therapy MH - Electromyography MH - Exosome Multienzyme Ribonuclease Complex/*immunology MH - Female MH - Humans MH - Kaplan-Meier Estimate MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Muscle, Skeletal/diagnostic imaging/*pathology/physiopathology MH - Myositis/*blood/diagnostic imaging/therapy MH - Regression Analysis MH - Respiratory Function Tests PMC - PMC5993182 EDAT- 2018/05/08 06:00 MHDA- 2019/08/14 06:00 PMCR- 2019/06/05 CRDT- 2018/05/06 06:00 PHST- 2017/10/09 00:00 [received] PHST- 2018/03/21 00:00 [accepted] PHST- 2018/05/08 06:00 [pubmed] PHST- 2019/08/14 06:00 [medline] PHST- 2018/05/06 06:00 [entrez] PHST- 2019/06/05 00:00 [pmc-release] AID - WNL.0000000000005638 [pii] AID - NEUROLOGY2017855098 [pii] AID - 10.1212/WNL.0000000000005638 [doi] PST - ppublish SO - Neurology. 2018 Jun 5;90(23):e2068-e2076. doi: 10.1212/WNL.0000000000005638. Epub 2018 May 4. PMID- 24715367 OWN - NLM STAT- MEDLINE DCOM- 20150818 LR - 20200930 IS - 1552-4833 (Electronic) IS - 1552-4825 (Linking) VI - 164A IP - 7 DP - 2014 Jul TI - Blepharophimosis, short humeri, developmental delay and hirschsprung disease: expanding the phenotypic spectrum of MED12 mutations. PG - 1821-5 LID - 10.1002/ajmg.a.36539 [doi] AB - We report on two male sibs, a fetus and a newborn, with short humeri and dysmorphic facial features including blepharophimosis. The newborn also had Hirschsprung disease. Goldberg-Shprintzen syndrome and the Say-Barber-Biesecker-Young-Simpson type of Ohdo syndrome were suspected but direct sequencing of KBP and KAT6B failed to identify a mutation. Finally, direct sequencing of MED12, the gene mutated in Opitz-Kaveggia syndrome, Lujan-Fryns syndrome and X-linked Ohdo syndrome identified in the two sibs the missense mutation c.3443G>A (p.Arg1148His) inherited from the mother. This report further expands the phenotypic spectrum of MED12 mutations. CI - © 2014 Wiley Periodicals, Inc. FAU - Isidor, Bertrand AU - Isidor B AD - Service de Génétique Médicale, CHU de Nantes, Nantes, France; INSERM, UMR-S 957, Nantes, France. FAU - Lefebvre, Tiphaine AU - Lefebvre T FAU - Le Vaillant, Claudine AU - Le Vaillant C FAU - Caillaud, Gaëlle AU - Caillaud G FAU - Faivre, Laurence AU - Faivre L FAU - Jossic, Frédéric AU - Jossic F FAU - Joubert, Madeleine AU - Joubert M FAU - Winer, Norbert AU - Winer N FAU - Le Caignec, Cédric AU - Le Caignec C FAU - Borck, Guntram AU - Borck G FAU - Pelet, Anna AU - Pelet A FAU - Amiel, Jeanne AU - Amiel J FAU - Toutain, Annick AU - Toutain A FAU - Ronce, Nathalie AU - Ronce N FAU - Raynaud, Martine AU - Raynaud M FAU - Verloes, Alain AU - Verloes A FAU - David, Albert AU - David A LA - eng PT - Case Reports PT - Journal Article DEP - 20140408 PL - United States TA - Am J Med Genet A JT - American journal of medical genetics. Part A JID - 101235741 RN - 0 (MED12 protein, human) RN - 0 (Mediator Complex) SB - IM MH - Abnormalities, Multiple/*diagnosis/*genetics MH - Aborted Fetus MH - Blepharophimosis MH - Developmental Disabilities MH - *Genetic Association Studies MH - Hirschsprung Disease MH - Humans MH - Humerus/pathology MH - Infant, Newborn MH - Male MH - Mediator Complex/*genetics MH - *Mutation MH - *Phenotype MH - Prenatal Diagnosis MH - Siblings MH - Syndrome OTO - NOTNLM OT - Goldberg-Shprintzen syndrome OT - Hirschsprung Disease OT - MED12 OT - blepharophimosis OT - humerus OT - hypertelorism EDAT- 2014/04/10 06:00 MHDA- 2015/08/19 06:00 CRDT- 2014/04/10 06:00 PHST- 2013/11/14 00:00 [received] PHST- 2014/02/23 00:00 [accepted] PHST- 2014/04/10 06:00 [entrez] PHST- 2014/04/10 06:00 [pubmed] PHST- 2015/08/19 06:00 [medline] AID - 10.1002/ajmg.a.36539 [doi] PST - ppublish SO - Am J Med Genet A. 2014 Jul;164A(7):1821-5. doi: 10.1002/ajmg.a.36539. Epub 2014 Apr 8. PMID- 27283334 OWN - NLM STAT- MEDLINE DCOM- 20170530 LR - 20250214 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 76 IP - 1 DP - 2017 Jan TI - Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study. PG - 159-165 LID - 10.1136/annrheumdis-2016-209522 [doi] AB - OBJECTIVES: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients. METHODS: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations. Organ damage was assessed with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index and the Juvenile Arthritis Damage Index (JADI). Medical records were reviewed for early predictors for outcome, which were assessed by multivariate logistic regression analyses. RESULTS: Three patients developed systemic lupus erythematosus (SLE). Fifty-two patients had continuous JMCTD; the most common manifestations were: Raynaud (100%), arthritis (94%), puffy hands (77%) and pulmonary manifestations (58%). SLE-like, systemic sclerosis (SSc)-like and polymyositis (PM)-like findings were found in 98%, 77% and 48%, respectively. Over time, SLE-like and PM-like manifestations decreased, and SSc-like findings increased. At follow-up, 35 patients (67%) had active disease and 17 (33%) were in remission. In 34 patients (65%), SLICC or JADI≥1 assessments indicated organ damage. Active disease was associated with higher anti-ribonucleoprotein antibody titres at follow-up and positive rheumatoid factor (RF) at diagnosis and follow-up. CONCLUSIONS: Most patients with JMCTD had active disease and organ damage after a mean follow-up of 16.2 years. Active disease was associated with higher anti-ribonucleoprotein antibody levels and positive RF. The presence of RF at diagnosis predicted persistent disease activity. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/. FAU - Hetlevik, Siri Opsahl AU - Hetlevik SO AD - Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. FAU - Flatø, Berit AU - Flatø B AUID- ORCID: 0000-0001-9178-1583 AD - Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. AD - Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo, Norway. FAU - Rygg, Marite AU - Rygg M AD - Department of Pediatrics, St Olavs Hospital, Trondheim, Norway. AD - Department of Laboratory Medicine, Children's and Women's Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. FAU - Nordal, Ellen Berit AU - Nordal EB AD - Department of Pediatrics, University Hospital of North Norway, Tromsø, Norway. AD - Institute of Clinical Medicine, UiT-the Arctic University of Norway, Tromsø, Norway. FAU - Brunborg, Cathrine AU - Brunborg C AD - Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital, Ullevål, Oslo, Norway. FAU - Hetland, Helene AU - Hetland H AD - Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway. FAU - Lilleby, Vibke AU - Lilleby V AD - Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. LA - eng PT - Journal Article DEP - 20160609 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Anti-Inflammatory Agents, Non-Steroidal) RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) RN - 0 (Ribonucleoproteins) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Adolescent MH - Anti-Inflammatory Agents, Non-Steroidal/therapeutic use MH - Antibodies, Antinuclear/blood MH - Child MH - Databases, Factual MH - Female MH - Follow-Up Studies MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Lupus Erythematosus, Systemic/diagnosis/epidemiology MH - Male MH - Mixed Connective Tissue Disease/*diagnosis/drug therapy/epidemiology/immunology MH - Norway/epidemiology MH - Outcome Assessment, Health Care/methods MH - Polymyositis/diagnosis/epidemiology MH - Prognosis MH - Registries MH - Rheumatoid Factor/blood MH - Ribonucleoproteins/immunology MH - Scleroderma, Systemic/diagnosis/epidemiology MH - Severity of Illness Index OTO - NOTNLM OT - Autoimmune Diseases OT - Disease Activity OT - Outcomes research EDAT- 2016/06/11 06:00 MHDA- 2017/05/31 06:00 CRDT- 2016/06/11 06:00 PHST- 2016/03/09 00:00 [received] PHST- 2016/04/17 00:00 [revised] PHST- 2016/05/17 00:00 [accepted] PHST- 2016/06/11 06:00 [pubmed] PHST- 2017/05/31 06:00 [medline] PHST- 2016/06/11 06:00 [entrez] AID - S0003-4967(24)01589-9 [pii] AID - 10.1136/annrheumdis-2016-209522 [doi] PST - ppublish SO - Ann Rheum Dis. 2017 Jan;76(1):159-165. doi: 10.1136/annrheumdis-2016-209522. Epub 2016 Jun 9. PMID- 39492683 OWN - NLM STAT- MEDLINE DCOM- 20250425 LR - 20260518 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 77 IP - 4 DP - 2025 Apr TI - Characterization of Incident Interstitial Lung Disease in Late Systemic Sclerosis. PG - 450-457 LID - 10.1002/art.43051 [doi] AB - OBJECTIVE: Interstitial lung disease (ILD) is a common and potentially lethal complication of systemic sclerosis (SSc). Screening by high-resolution computed tomography (HRCT) is recommended in all patients with risk factors, including early disease. Little is known on late presentations of ILD. This study aimed to characterize the incidence, risk factors, and outcomes of late-onset SSc-ILD. METHODS: Study participants enrolled in the Canadian Scleroderma Research Group cohort from 2004 to 2020 without prevalent ILD were included. Incidence and risk factors for ILD (on HRCT) were compared according to disease duration above (late) and below (earlier) seven years from the first non-Raynaud manifestation. Risk of ILD progression was compared using Kaplan-Meier and multivariable Cox models. RESULTS: Overall, 199 (21%) of 969 patients developed incident ILD over a median of 2.4 (interquartile range 1.2-4.3) years. The incidence rate in late SSc (3.7/100 person-years) was lower than in earlier SSc (relative risk 0.68, 95% confidence interval [CI] 0.51-0.92). Risk factors for incident ILD included male sex, diffuse subtype, myositis, antitopoisomerase I autoantibodies, and higher C-reactive protein levels. Patients with late-onset ILD were also less frequently White and more frequently had arthritis and anti-RNA-polymerase III autoantibodies. Lung disease severity was similar between late- and earlier-onset SSc-ILD (forced vital capacity 88% and 87%, diffusion capacity of the lungs for carbon monoxide 64% and 62%, respectively). Progression rates were also similar between late- and earlier-onset SSc-ILD (log rank P = 0.8, hazard ratio 1.11, 95% CI 0.58-2.10). CONCLUSION: ILD can present in late SSc. Risk factors and progression rates overlapped with earlier-onset SSc-ILD. Surveillance for ILD should continue in longstanding SSc. Frequency and modality of monitoring remain to be defined. CI - © 2024 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Hoa, Sabrina AU - Hoa S AD - Centre Hospitalier de l'Université de Montréal, Montréal, Québec, Canada. FAU - Berger, Claudie AU - Berger C AD - Research Institute of the McGill University Health Center, Montréal, Québec, Canada. FAU - Lahmek, Nouha AU - Lahmek N AD - Centre Hospitalier de l'Université de Montréal, Montréal, Québec, Canada. FAU - Larché, Maggie AU - Larché M AD - McMaster University, Hamilton, Ontario, Canada, and University of Calgary, Calgary, Alberta, Canada. FAU - Osman, Mohammed AU - Osman M AUID- ORCID: 0000-0003-3580-6074 AD - University of Alberta, Edmonton, Alberta, Canada. FAU - Choi, May AU - Choi M AUID- ORCID: 0000-0003-3760-2737 AD - University of Calgary, Calgary, Alberta, Canada. FAU - Pope, Janet AU - Pope J AUID- ORCID: 0000-0003-1479-5302 AD - University of Western Ontario, London, Canada. FAU - Thorne, Carter AU - Thorne C AD - Southlake Regional Health Centre, Newmarket, Ontario, Canada. CN - Canadian Scleroderma Research Group FAU - Hudson, Marie AU - Hudson M AD - McGill University and Lady Davis Institute, Jewish General Hospital, Montréal, Quebec, Canada. LA - eng GR - Université de Montréal Scleroderma Chair/ GR - Sclérodermie Quebec/ GR - Fonds de Recherche du Québec en Santé/ PT - Journal Article DEP - 20250107 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 SB - IM MH - Humans MH - *Lung Diseases, Interstitial/epidemiology/etiology/diagnostic imaging MH - *Scleroderma, Systemic/complications/epidemiology MH - Male MH - Female MH - Incidence MH - Middle Aged MH - Risk Factors MH - Aged MH - Canada/epidemiology MH - Disease Progression MH - Tomography, X-Ray Computed MH - Proportional Hazards Models MH - Adult PMC - PMC11936498 FIR - Baron, M IR - Baron M FIR - Hudson, M IR - Hudson M FIR - Gyger, G IR - Gyger G FIR - Pope, J IR - Pope J FIR - Larché, M IR - Larché M FIR - Khalidi, N IR - Khalidi N FIR - Masetto, A IR - Masetto A FIR - Sutton, E IR - Sutton E FIR - Rodriguez-Reyna, T S IR - Rodriguez-Reyna TS FIR - Maltez, N IR - Maltez N FIR - Thorne, C IR - Thorne C FIR - Fortin, P R IR - Fortin PR FIR - Ikic, A IR - Ikic A FIR - Robinson, D IR - Robinson D FIR - Jones, N IR - Jones N FIR - LeClercq, S IR - LeClercq S FIR - Mathieu, J-P IR - Mathieu JP FIR - Docherty, P IR - Docherty P FIR - Smith, D IR - Smith D FIR - Fritzler, M IR - Fritzler M EDAT- 2024/11/04 06:24 MHDA- 2025/03/26 04:06 PMCR- 2025/03/25 CRDT- 2024/11/04 03:45 PHST- 2024/09/03 00:00 [revised] PHST- 2024/06/08 00:00 [received] PHST- 2024/10/23 00:00 [accepted] PHST- 2025/03/26 04:06 [medline] PHST- 2024/11/04 06:24 [pubmed] PHST- 2024/11/04 03:45 [entrez] PHST- 2025/03/25 00:00 [pmc-release] AID - ART43051 [pii] AID - 10.1002/art.43051 [doi] PST - ppublish SO - Arthritis Rheumatol. 2025 Apr;77(4):450-457. doi: 10.1002/art.43051. Epub 2025 Jan 7. PMID- 26747283 OWN - NLM STAT- MEDLINE DCOM- 20161213 LR - 20181113 IS - 1532-2548 (Electronic) IS - 0032-0889 (Print) IS - 0032-0889 (Linking) VI - 170 IP - 3 DP - 2016 Mar TI - Chloroplast Activity and 3'phosphadenosine 5'phosphate Signaling Regulate Programmed Cell Death in Arabidopsis. PG - 1745-56 LID - 10.1104/pp.15.01872 [doi] AB - Programmed cell death (PCD) is a crucial process both for plant development and responses to biotic and abiotic stress. There is accumulating evidence that chloroplasts may play a central role during plant PCD as for mitochondria in animal cells, but it is still unclear whether they participate in PCD onset, execution, or both. To tackle this question, we have analyzed the contribution of chloroplast function to the cell death phenotype of the myoinositol phosphate synthase1 (mips1) mutant that forms spontaneous lesions in a light-dependent manner. We show that photosynthetically active chloroplasts are required for PCD to occur in mips1, but this process is independent of the redox state of the chloroplast. Systematic genetic analyses with retrograde signaling mutants reveal that 3'-phosphoadenosine 5'-phosphate, a chloroplast retrograde signal that modulates nuclear gene expression in response to stress, can inhibit cell death and compromises plant innate immunity via inhibition of the RNA-processing 5'-3' exoribonucleases. Our results provide evidence for the role of chloroplast-derived signal and RNA metabolism in the control of cell death and biotic stress response. CI - © 2016 American Society of Plant Biologists. All Rights Reserved. FAU - Bruggeman, Quentin AU - Bruggeman Q AUID- ORCID: 0000-0002-2288-7744 AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Mazubert, Christelle AU - Mazubert C AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Prunier, Florence AU - Prunier F AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Lugan, Raphaël AU - Lugan R AUID- ORCID: 0000-0002-1238-844X AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Chan, Kai Xun AU - Chan KX AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Phua, Su Yin AU - Phua SY AUID- ORCID: 0000-0002-7556-1211 AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Pogson, Barry James AU - Pogson BJ AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Krieger-Liszkay, Anja AU - Krieger-Liszkay A AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Delarue, Marianne AU - Delarue M AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Benhamed, Moussa AU - Benhamed M AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Bergounioux, Catherine AU - Bergounioux C AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.). FAU - Raynaud, Cécile AU - Raynaud C AD - Institute of Plant Sciences Paris-Saclay (IPS2), UMR 9213/UMR1403, CNRS, INRA, Université Paris-Sud, Université d'Evry, Université Paris-Diderot, Sorbonne Paris-Cité, Bâtiment 630, 91405 Orsay, France (Q.B., C.M., F.P., M.D., M.B., C.B., C.R.);Institut de Biologie Moléculaire des Plantes, Unité Propre de Recherche 2357 CNRS, Université de Strasbourg, 12 rue du Général Zimmer, 67084 Strasbourg cedex, France (R.L.);Australian Research Council Centre of Excellence in Plant Energy Biology, Research School of Biology, Australian National University, Acton, Australian Capital Territory 2601, Australia (K.X.C., S.Y.P., B.J.P.);Institute for Integrative Biology of the Cell (I2BC), Commissariat à l'Energie Atomique et aux Energies Alternatives Saclay, Centre National de la Recherche Scientifique, Université Paris-Sud, F-91191 Gif-sur-Yvette cedex, France (A.K.-L.); and Division of Biological and Environmental Sciences and Engineering and Center for Desert Agriculture, King Abdullah University of Science and Technology, Thuwal, Kingdom of Saudi Arabia (M.B.) cecile.raynaud@u-psud.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160108 PL - United States TA - Plant Physiol JT - Plant physiology JID - 0401224 RN - 1406-65-1 (Chlorophyll) RN - 61D2G4IYVH (Adenosine Diphosphate) RN - C65F80D52U (adenosine 3'-phosphate-5'-phosphate) RN - EC 5.5.1.4 (Myo-Inositol-1-Phosphate Synthase) SB - IM MH - Adenosine Diphosphate/*metabolism MH - Apoptosis/genetics/*physiology MH - Arabidopsis/genetics/*metabolism/microbiology MH - Chlorophyll/metabolism MH - Chloroplasts/genetics/*metabolism MH - Disease Resistance/genetics MH - Mutation MH - Myo-Inositol-1-Phosphate Synthase/genetics/metabolism MH - Oxidation-Reduction MH - Photosynthesis/genetics/physiology MH - Plant Diseases/genetics/microbiology MH - Plant Immunity/genetics MH - Pseudomonas syringae/physiology MH - Signal Transduction/genetics/*physiology PMC - PMC4775142 EDAT- 2016/01/10 06:00 MHDA- 2016/12/15 06:00 PMCR- 2017/03/01 CRDT- 2016/01/10 06:00 PHST- 2015/11/30 00:00 [received] PHST- 2016/01/05 00:00 [accepted] PHST- 2017/03/01 00:00 [pmc-release] PHST- 2016/01/10 06:00 [entrez] PHST- 2016/01/10 06:00 [pubmed] PHST- 2016/12/15 06:00 [medline] AID - pp.15.01872 [pii] AID - PP201501872DR1 [pii] AID - 10.1104/pp.15.01872 [doi] PST - ppublish SO - Plant Physiol. 2016 Mar;170(3):1745-56. doi: 10.1104/pp.15.01872. Epub 2016 Jan 8. PMID- 24752068 OWN - NLM STAT- MEDLINE DCOM- 20140828 LR - 20140529 IS - 1872-7980 (Electronic) IS - 0304-3835 (Linking) VI - 349 IP - 2 DP - 2014 Jul 28 TI - Structure-based discovery of a small non-peptidic Neuropilins antagonist exerting in vitro and in vivo anti-tumor activity on breast cancer model. PG - 120-7 LID - S0304-3835(14)00210-9 [pii] LID - 10.1016/j.canlet.2014.04.004 [doi] AB - Neuropilin-1/-2 (+33 NRPs), VEGF-A165 co-receptors, are over-expressed during cancer progression. Thus, NRPs targeted drug development is challenged using a multistep in silico/in vitro screening procedure. The first fully non-peptidic VEGF-A165/NRPs protein-protein interaction antagonist (IC50=34 μM) without effect on pro-angiogenic kinases has been identified (compound-1). This hit showed breast cancer cells anti-proliferative activity (IC50=0.60 μM). Compound-1 treated NOG-xenografted mice significantly exerted tumor growth inhibition, which is correlated with Ki-67(low) expression and apoptosis. Furthermore, CD31(+)/CD34(+) vessels are reduced in accordance with HUVEC-tube formation inhibition (IC50=0.20 μM). Taking together, compound-1 is the first fully organic inhibitor targeting NRPs. CI - Copyright © 2014 Elsevier Ireland Ltd. All rights reserved. FAU - Borriello, Lucia AU - Borriello L AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Montès, Matthieu AU - Montès M AD - Conservatoire National des Arts et Métiers, Chaire de Bioinformatique, Laboratoire Génomique, Bioinformatique et Applications, EA 4627, 292 rue Saint Martin, 75003 Paris, France. FAU - Lepelletier, Yves AU - Lepelletier Y AD - INSERM UMR 1163, Laboratory of cellular and molecular basis of normal hematopoiesis and hematological disorders: therapeutical implications, 24 boulevard Montparnasse, 75015 Paris, France; Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, 24 boulevard Montparnasse, 75015 Paris, France; CNRS ERL 8254, 24 boulevard Montparnasse, 75015 Paris, France. FAU - Leforban, Bertrand AU - Leforban B AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Liu, Wang-Qing AU - Liu WQ AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Demange, Luc AU - Demange L AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Delhomme, Brigitte AU - Delhomme B AD - CNRS FRE 3235, Université Paris Descartes, Sorbonne Paris Cité, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Pavoni, Serena AU - Pavoni S AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Jarray, Rafika AU - Jarray R AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Boucher, Jean Luc AU - Boucher JL AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Dufour, Sylvie AU - Dufour S AD - UMR 144 CNRS-Institut Curie, 26 rue d'Ulm, 75005 Paris, France. FAU - Hermine, Olivier AU - Hermine O AD - INSERM UMR 1163, Laboratory of cellular and molecular basis of normal hematopoiesis and hematological disorders: therapeutical implications, 24 boulevard Montparnasse, 75015 Paris, France; Paris Descartes University-Sorbonne Paris Cité, Imagine Institute, 24 boulevard Montparnasse, 75015 Paris, France; CNRS ERL 8254, 24 boulevard Montparnasse, 75015 Paris, France. FAU - Garbay, Christiane AU - Garbay C AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. FAU - Hadj-Slimane, Réda AU - Hadj-Slimane R AD - Tragex Pharma, 29 Rue Marcel Dassault, 92100 Boulogne-Billancourt, France. FAU - Raynaud, Françoise AU - Raynaud F AD - UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, UFR Biomédicale des Saints Pères, 45 rue des Saints Pères, 75270 Paris cedex 06, France. Electronic address: francoise.raynaud@parisdescartes.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140419 PL - Ireland TA - Cancer Lett JT - Cancer letters JID - 7600053 RN - 0 (Antineoplastic Agents) RN - 0 (Ligands) RN - 0 (Neuropilins) RN - 0 (Peptide Fragments) RN - 0 (Small Molecule Libraries) RN - 0 (Vascular Endothelial Growth Factor A) RN - 0 (vascular endothelial growth factor A (138-165)) SB - IM MH - Animals MH - Antineoplastic Agents/chemistry/*pharmacology MH - Apoptosis/drug effects MH - Breast Neoplasms/*drug therapy/pathology MH - Cell Line, Tumor MH - Disease Progression MH - Drug Evaluation, Preclinical MH - Female MH - Human Umbilical Vein Endothelial Cells/drug effects MH - Humans MH - Ligands MH - Mice MH - Mice, Inbred NOD MH - Mice, SCID MH - Mice, Transgenic MH - Models, Molecular MH - Molecular Docking Simulation MH - Neuropilins/*antagonists & inhibitors/chemistry/metabolism MH - Peptide Fragments/antagonists & inhibitors/metabolism MH - Small Molecule Libraries/chemistry/*pharmacology MH - Structure-Activity Relationship MH - Vascular Endothelial Growth Factor A/antagonists & inhibitors/metabolism MH - Xenograft Model Antitumor Assays OTO - NOTNLM OT - Fully organic inhibitors OT - Neuropilin OT - Protein–protein interaction OT - Tumor growth inhibition EDAT- 2014/04/23 06:00 MHDA- 2014/08/29 06:00 CRDT- 2014/04/23 06:00 PHST- 2014/02/27 00:00 [received] PHST- 2014/04/03 00:00 [revised] PHST- 2014/04/06 00:00 [accepted] PHST- 2014/04/23 06:00 [entrez] PHST- 2014/04/23 06:00 [pubmed] PHST- 2014/08/29 06:00 [medline] AID - S0304-3835(14)00210-9 [pii] AID - 10.1016/j.canlet.2014.04.004 [doi] PST - ppublish SO - Cancer Lett. 2014 Jul 28;349(2):120-7. doi: 10.1016/j.canlet.2014.04.004. Epub 2014 Apr 19. PMID- 28749191 OWN - NLM STAT- MEDLINE DCOM- 20180606 LR - 20220331 IS - 1607-842X (Electronic) IS - 0891-6934 (Linking) VI - 50 IP - 7 DP - 2017 Nov TI - Disease-related autoantibody profile in patients with systemic sclerosis. PG - 414-421 LID - 10.1080/08916934.2017.1357699 [doi] AB - BACKGROUND: Autoantibodies (autoAbs) help in diagnosis and predicting clinical phenotypes in systemic sclerosis (SSc). AIM OF THE STUDY: To determine the clinical utility of 13 SSc-related autoAbs in SSc patients. MATERIAL AND METHODS: A total of 131 consecutive patients with SSc (111 female, mean age 58.1 ± 14 years; 49 with diffused cutaneous SSc [dcSSc] and 82 with limited cutaneous SSc [lcSSc]) were analysed by a multiplex line immunoassay (Euroimmun) for autoantibodies (autoAbs) against 13 SSc-related antigens. A total of 22 patients with primary Raynaud phenomenon (RP), and 22 healthy controls were also analysed. RESULTS: ANA by indirect immunofluorescence was present in 128 (97.7%) patients with SSc. Excluding anti-Ro52, 113 (89.3%) SSc patients were positive for at least one autoAb: anti-Topoisomerase I (anti-Topo) I abs in 54 (41.2%), anti-centromere proteins (anti-CENP) in 37 (28.2%, all reactive with centromere protein-A (CENPA) and centromere protein B (CENPB)), anti-RNA polymerase III(RP11) in 19 (14.5%), anti-RNA polymerase III(RP155) in 13 (9.9%), anti-fibrillarin in 4 (3.1%), anti-Ku in 6 (4.6%), anti-nucleolus-organizing region (anti-NOR90) in 8 (6.1%), anti-PM-Scl100 in 2 (1.5%), and anti-PM-Scl75 in 4 (3.1%). There was no immunoreactivity for Th/To or platelet-derived growth factor receptor (PDGFR). Overall, 102 (77.9%) SSc patients had autoAbs against Topo I, CENPA or CENPB, RP11 or RP155. Anti-Topo I abs were strongly associated with dcSSc, interstitial lung disease (ILD) (p < .001), pulmonary hypertension (PH) (p = .019) and ILD-PH (p = .003). Anti-CENPB abs were associated with lcSSc, and negatively associated with ILD. Anti-RP11 and anti-NOR90 abs were associated with male gender, and anti-NOR90 associated with ILD. CONCLUSIONS: Anti-Topo I, anti-CENP, and anti-RNA pol III are the most prevalent autoAbs in SSc. Anti-Topo I and anti-NOR90 abs are associated with ILD and/or PAH. FAU - Liaskos, Christos AU - Liaskos C AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. FAU - Marou, Emmanouela AU - Marou E AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. AD - b Biomedical Section , Institute of Research and Technology Thessaly, Centre for Research and Technology Hellas (CERTH) , Larissa , Greece. FAU - Simopoulou, Theodora AU - Simopoulou T AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. FAU - Barmakoudi, Maria AU - Barmakoudi M AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. AD - b Biomedical Section , Institute of Research and Technology Thessaly, Centre for Research and Technology Hellas (CERTH) , Larissa , Greece. FAU - Efthymiou, Georgios AU - Efthymiou G AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. AD - b Biomedical Section , Institute of Research and Technology Thessaly, Centre for Research and Technology Hellas (CERTH) , Larissa , Greece. FAU - Scheper, Thomas AU - Scheper T AD - c Institute of Immunology affiliated to Euroimmun AG , Lübeck , Germany. FAU - Meyer, Wolfgang AU - Meyer W AD - c Institute of Immunology affiliated to Euroimmun AG , Lübeck , Germany. FAU - Bogdanos, Dimitrios P AU - Bogdanos DP AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. AD - b Biomedical Section , Institute of Research and Technology Thessaly, Centre for Research and Technology Hellas (CERTH) , Larissa , Greece. AD - d Division of Transplantation, Immunology and Mucosal Biology , MRC Centre for Transplantation, King's College London Medical School , London , UK. FAU - Sakkas, Lazaros I AU - Sakkas LI AD - a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece. AD - e Center for Molecular Medicine , Old Dominion University , Norfolk , VA , USA. LA - eng PT - Journal Article DEP - 20170727 PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Epitopes) RN - 0 (Immunoglobulin G) SB - IM MH - Adult MH - Aged MH - Autoantibodies/*immunology MH - Autoantigens/immunology MH - *Autoimmunity MH - Epitopes/immunology MH - Female MH - Humans MH - Immunoassay MH - Immunoglobulin G/immunology MH - Male MH - Middle Aged MH - Prevalence MH - Scleroderma, Systemic/*immunology OTO - NOTNLM OT - Autoimmunity OT - autoantibodies OT - fibrosis OT - line assay OT - scleroderma OT - ulcers EDAT- 2017/07/28 06:00 MHDA- 2018/06/07 06:00 CRDT- 2017/07/28 06:00 PHST- 2017/07/28 06:00 [pubmed] PHST- 2018/06/07 06:00 [medline] PHST- 2017/07/28 06:00 [entrez] AID - 10.1080/08916934.2017.1357699 [doi] PST - ppublish SO - Autoimmunity. 2017 Nov;50(7):414-421. doi: 10.1080/08916934.2017.1357699. Epub 2017 Jul 27. PMID- 20301768 STAT- Publisher DRDT- 20160707 CTDT- 20090625 PB - University of Washington, Seattle DP - 1993 TI - COL4A1-Related Disorders. BTI - GeneReviews(®) AB - CLINICAL CHARACTERISTICS: The spectrum of COL4A1-related disorders includes: small-vessel brain disease of varying severity including porencephaly, variably associated with eye defects (retinal arterial tortuosity, Axenfeld-Rieger anomaly, cataract) and systemic findings (kidney involvement, muscle cramps, cerebral aneurysms, Raynaud phenomenon, cardiac arrhythmia, and hemolytic anemia). On imaging studies, small-vessel brain disease is manifest as diffuse periventricular leukoencephalopathy, lacunar infarcts, microhemorrhage, dilated perivascular spaces, and deep intracerebral hemorrhages. Clinically, small-vessel brain disease manifests as infantile hemiparesis, seizures, single or recurrent hemorrhagic stroke, ischemic stroke, and isolated migraine with aura. Porencephaly (fluid-filled cavities in the brain detected by CT or MRI) is typically manifest as infantile hemiparesis, seizures, and intellectual disability; however, on occasion it can be an incidental finding. HANAC (hereditary angiopathy with nephropathy, aneurysms, and muscle cramps) syndrome usually associates asymptomatic small-vessel brain disease, cerebral large vessel involvement (i.e., aneurysms), and systemic findings involving the kidney, muscle, and small vessels of the eye. Two additional phenotypes include isolated retinal artery tortuosity and nonsyndromic autosomal dominant congenital cataract. DIAGNOSIS/TESTING: Diagnosis is based on clinical findings and molecular genetic testing of COL4A1. MANAGEMENT: Treatment of manifestations: Supportive care tailored to the individual’s specific medical needs and including practical help and emotional support for affected individuals and their families. Hypertension should be treated to reduce the overall risk of stroke. Prevention of primary and secondary complications: Avoiding head trauma and anticoagulant exposure may decrease the risk for intracerebral hemorrhage. Surveillance: Depends on the severity and type of symptoms. Agents/circumstances to avoid: Smoking and hypertension because these factors increase the risk for stroke; sustained head pressure or physical activities that may cause head trauma; anticoagulant use. GENETIC COUNSELING: COL4A1-related disorders are inherited in an autosomal dominant manner. Most individuals diagnosed with a COL4A1-related disorder have an affected parent. The proportion of cases caused by a de novo pathogenic variant is estimated to be at least 27%. Each child of an individual with a COL4A1-related disorder has a 50% chance of inheriting the pathogenic variant. Prenatal testing for a pregnancy at increased risk is possible if the pathogenic variant in the family is known. CI - Copyright © 1993-2026, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved. Test. FED - Adam, Margaret P ED - Adam MP FED - Bick, Sarah ED - Bick S FED - Mirzaa, Ghayda M ED - Mirzaa GM FED - Pagon, Roberta A ED - Pagon RA FED - Wallace, Stephanie E ED - Wallace SE FED - Amemiya, Anne ED - Amemiya A FAU - Plaisier, Emmanuelle AU - Plaisier E AD - Department of Nephrology and Dialysis INSERM UMR S1155 Hôpital Tenon Université Pierre et Marie Curie Paris, France FAU - Ronco, Pierre AU - Ronco P AD - Department of Nephrology and Dialysis INSERM UMR S1155 Hôpital Tenon Université Pierre et Marie Curie Paris, France LA - eng PT - Review PT - Book Chapter PL - Seattle (WA) OTO - NLM OT - Autosomal Dominant Familial Porencephaly OT - Autosomal Dominant Brain Small-Vessel Disease with Hemorrhage OT - Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) Syndrome OT - Tortuosity of Retinal Arteries OT - Nonsyndromic Autosomal Dominant Congenital Cataract OT - Collagen alpha-1(IV) chain OT - COL4A1 OT - COL4A1-Related Disorders EDAT- 2016/07/07 00:00 CRDT- 2016/07/07 00:00 AID - NBK7046 [bookaccession] PMID- 41972746 OWN - NLM STAT- Publisher LR - 20260504 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) DP - 2026 Apr 13 TI - Characteristics and Outcomes of Male Participants in a Multicenter Longitudinal Australian Study Cohort. LID - 10.1002/acr.80060 [doi] AB - OBJECTIVE: The aim of this study was to determine the differences in demographic, serologic, and clinical characteristics between male and female patients with systemic sclerosis (SSc) in an Australian cohort. METHODS: This was a retrospective observational study using data from the Australian Scleroderma Cohort Study. Male patients were compared to female patients using chi-square test, Wilcoxon rank-sum test, and Student's t-test. Survival analysis from time of diagnosis to all-cause mortality was presented on Kaplan-Meier curves, and a multivariate Cox proportional hazards model was used to evaluate independent predictors of all-cause mortality. A subanalysis of patients with incident SSc, defined as disease duration less than four years from onset of first non-Raynaud phenomenon manifestation to recruitment, was performed. RESULTS: Of the 2,033 participants, 299 were male, with a female-to-male ratio of 5.8:1. Male patients were more likely to present with diffuse skin involvement (43.7% vs 23.6%, P < 0.001) and ever be positive for digital ulcers, digital pitting, gangrene, and tendon friction rubs as well as myositis and interstitial lung disease. Male patients were more likely to have anti-Scl-70, anti-Jo-1, anti-PM/Scl and anti-RNA polymerase III antibodies compared to female patients. Male patients reported more exposure to silica dust, organic solvents, vinyl chloride, and epoxy resin compared with female patients. In the incident cohort, male patients had statistically significantly higher mortality, with 60% of male patients compared to 82% of female patients surviving 10 years from diagnosis (P < 0.001). CONCLUSION: Male patients with SSc have more extractable nuclear antigen antibody positivity and reduced survival in comparison to female patients. The significant difference between occupational exposures recorded between sexes warrants further investigation into the role of industrial exposures in the pathogenesis of SSc in male patients. CI - © 2026 The Author(s). Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Lin, Emily AU - Lin E AUID- ORCID: 0009-0003-2134-5278 AD - Monash University, Melbourne, Victoria, Australia. AD - Monash Medical Centre, Clayton, Victoria, Australia. FAU - Hansen, Dylan AU - Hansen D AUID- ORCID: 0000-0001-5777-7476 AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. FAU - Ross, Laura AU - Ross L AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. AD - University of Melbourne, Fitzroy, Melbourne, Australia. FAU - Ngian, Gene-Siew AU - Ngian GS AD - Monash University, Melbourne, Victoria, Australia. AD - Monash Medical Centre, Clayton, Victoria, Australia. FAU - Proudman, Susanna AU - Proudman S AD - Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - University of Adelaide, Adelaide, South Australia, Australia. FAU - Major, Gabor AU - Major G AUID- ORCID: 0000-0003-3464-7438 AD - John Hunter Hospital, New Lambton Heights, New South Wales, Australia. AD - University of Newcastle, Callaghan, New South Wales, Australia. FAU - Tabesh, Maryam AU - Tabesh M AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. FAU - Nikpour, Mandana AU - Nikpour M AUID- ORCID: 0000-0002-6585-5611 AD - University of Sydney, Sydney, New South Wales, Australia. AD - Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. FAU - Walker, Jennifer AU - Walker J AD - Royal Adelaide Hospital, Adelaide, South Australia, Australia. AD - Flinders Medical Centre, Bedford Park, South Australia, Australia. FAU - Host, Lauren AU - Host L AUID- ORCID: 0000-0003-3480-7251 AD - Fiona Stanley Hospital, Murdoch, Western Australia, Australia. FAU - Ferdowsi, Nava AU - Ferdowsi N AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. FAU - Quinlivan, Alannah AU - Quinlivan A AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. AD - University of Melbourne, Fitzroy, Melbourne, Australia. FAU - Stevens, Wendy AU - Stevens W AD - St Vincent's Hospital, Fitzroy, Melbourne, Australia. AD - University of Melbourne, Fitzroy, Melbourne, Australia. FAU - Sahhar, Joanne AU - Sahhar J AD - Monash University, Melbourne, Victoria, Australia. AD - Monash Medical Centre, Clayton, Victoria, Australia. FAU - Apostolopoulos, Diane AU - Apostolopoulos D AUID- ORCID: 0000-0003-1078-6732 AD - Monash University, Melbourne, Victoria, Australia. AD - Monash Medical Centre, Clayton, Victoria, Australia. LA - eng PT - Journal Article DEP - 20260413 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 SB - IM EDAT- 2026/04/13 12:37 MHDA- 2026/04/13 12:37 CRDT- 2026/04/13 08:55 PHST- 2026/03/03 00:00 [revised] PHST- 2025/11/04 00:00 [received] PHST- 2026/04/03 00:00 [accepted] PHST- 2026/04/13 12:37 [pubmed] PHST- 2026/04/13 12:37 [medline] PHST- 2026/04/13 08:55 [entrez] AID - 10.1002/acr.80060 [doi] PST - aheadofprint SO - Arthritis Care Res (Hoboken). 2026 Apr 13. doi: 10.1002/acr.80060. PMID- 28536935 OWN - NLM STAT- MEDLINE DCOM- 20190909 LR - 20190909 IS - 1435-1250 (Electronic) IS - 0340-1855 (Linking) VI - 77 IP - 1 DP - 2018 Feb TI - [Rituximab for the treatment of poly- and dermatomyositis : Results from the GRAID-2 registry]. PG - 40-45 LID - 10.1007/s00393-017-0327-z [doi] AB - INTRODUCTION: In the treatment of poly- and dermatomyositis, only a limited number of treatment modalities are established. OBJECTIVE: The goal of the GRAID-2 registry was to study off-label use of biologic drugs for this indication in Germany. PATIENTS AND METHODS: Analysis of the data of the GRAID-2 registry for poly- and dermatomyositis. RESULTS: In 22 of the 23 patients in the GRAID-2 registry, rituximab (RIX) was administered, while 1 patient was given tocilizumab as off-label therapy. The 22 patients who received RIX treatment were analyzed. At the start of treatment, the following active manifestations were present: myositis (n = 18), lung involvement (mainly interstitial lung disease; n = 10), arthritis (n = 10), skin manifestation (n = 9), and Raynaud syndrome (n = 5). Nine of the patients were Jo-1-antibody positive. All patients had previous treatments with multiple conventional immunosuppressive drugs. Treatment with RIX was given as infusions of 1 g i. v., which were repeated after 2 weeks. Patients received a mean of 3.09 ± 2.27 infusions (equivalent to 1.5 cycles of 2 × 1 g, max. 5 cycles). Tolerability of RIX treatment was rated as very good in 16 of 22 patients (72%), good in 5 (23%), and moderate in 1 (5%). In all, 27 adverse events were documented, with the majority being infections, whereby 2 severe infections occurred (6.59 per 100 patient-years). Eighty six percent of the patients showed complete remission of their myositis and 79% of their arthritis. The mean value of creatinine kinase in plasma fell from 1505 ± 2534 U/l before the start of treatment to 39 ± 134 U/l at the last visit. Regarding lung involvement, 1 of 10 of the patients showed complete and 6 of 10 partial remissions. In 2 of 10 patients, lung disease was stable during treatment. CONCLUSION: RIX is the preferred off-label biologic drug for poly- and dermatomyositis in Germany. In spite of a strongly pretreated group of patients, the tolerability is acceptable, although the patient number in this investigation is small. Moreover, the results lead to the assumption that the majority of the patients had a good or even very good therapeutic response to RIX. FAU - Fiehn, C AU - Fiehn C AD - Praxis für Rheumatologie, Tätigkeitsschwerpunkt Klinische Immunologie, Beethovenstr. 2, 76530, Baden-Baden, Deutschland. c.fiehn@rheuma-badenbaden.de. FAU - Unger, L AU - Unger L AD - Städtisches Klinikum Dresden-Friedrichstadt, Dresden, Deutschland. FAU - Schulze-Koops, H AU - Schulze-Koops H AD - Sektion Rheumatologie und Klinische Immunologie, Medizinische Klinik IV, Klinikum der Universität München, München, Deutschland. FAU - Proft, F AU - Proft F AD - Sektion Rheumatologie und Klinische Immunologie, Medizinische Klinik IV, Klinikum der Universität München, München, Deutschland. AD - Charité Universitätsmedizin Berlin, Berlin, Deutschland. FAU - Henes, J C AU - Henes JC AD - Zentrum für Interdisziplinäre Klinische Immunologie, Rheumatologie und Autoimmunerkrankungen (INDIRA) und Innere Medizin II, Universitätsklinik Tübingen, Tübingen, Deutschland. FAU - Jacobi, A AU - Jacobi A AD - Sektion Rheumatologie und Klinische Immunologie, Medizinische Klinik D, Universität Münster, Münster, Deutschland. FAU - Dörner, T AU - Dörner T AD - Charité Universitätsmedizin Berlin, Berlin, Deutschland. LA - ger PT - Journal Article TT - Rituximab zur Behandlung von Poly- und Dermatomyositis : Die Ergebnisse des GRAID-2-Registers. PL - Germany TA - Z Rheumatol JT - Zeitschrift fur Rheumatologie JID - 0414162 RN - 0 (Antineoplastic Agents, Immunological) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - *Antineoplastic Agents, Immunological/therapeutic use MH - *Dermatomyositis/drug therapy MH - Germany MH - Humans MH - Registries MH - *Rituximab/therapeutic use MH - Treatment Outcome OTO - NOTNLM OT - Alveolitis OT - Dermatomyositis OT - Infections OT - Polymyositis OT - Rituximab EDAT- 2017/05/26 06:00 MHDA- 2019/09/10 06:00 CRDT- 2017/05/25 06:00 PHST- 2017/05/26 06:00 [pubmed] PHST- 2019/09/10 06:00 [medline] PHST- 2017/05/25 06:00 [entrez] AID - 10.1007/s00393-017-0327-z [pii] AID - 10.1007/s00393-017-0327-z [doi] PST - ppublish SO - Z Rheumatol. 2018 Feb;77(1):40-45. doi: 10.1007/s00393-017-0327-z. PMID- 18051224 OWN - NLM STAT- MEDLINE DCOM- 20080922 LR - 20211203 IS - 0025-7680 (Print) IS - 0025-7680 (Linking) VI - 67 IP - 5 DP - 2007 TI - [Lung involvement in systemic sclerosis]. PG - 429-35 AB - The objective of this study was to determine clinical predictors of interstitial lung disease in patients with systemic sclerosis (SSc) and pulmonary involvement as defined by presence of a decreased diffusing capacity for carbon monoxide (DLCO). Forty subjects with SSc were retrospectively evaluated. Patients were categorized according to their level of DLCO (< o > or = 80% of predicted). Sensitivity of dyspnea to detect a decreased DLCO was 46.6% and specificity 90%, whereas oxygen desaturation showed a sensitivity of 71.4% and a specificity of 80%. Patients with decreased DLCO (n = 18) were not different in age (51.1 +/- 13.5 vs. 53.5 +/- 9.3 y, p = 0.5182), sex (male 13.6%, p = 0.6088), prevalence of Raynaud (86.6% vs. 85%, p = 0.6272), sicca syndrome (6.2% vs. 10.5% p = 1.0000) diffuse cutaneous involvement (94.1% vs. 83.3%, p = 0.6026) or esophageal dilatation. The duration of symptoms since diagnosis was no different. Prevalence of pulmonary hypertension assessed by Doppler echocardiography or abnormal nailfold capillaroscopic findings were identical in both populations. Patients with low DLCO had a significatly higher prevalence of anti topoisomerase antibodies. (5/9 vs. 0/11, p = 0.0081) and restrictive lung disease. Patients with low DLCO showed a significantly higher prevalence of abnormal HRCT findings suggestive of ILD (82.3% vs. 5.8%, p < or = 0.0001). We conclude that a low DLCO is a frequent finding in SSc patients, strongly associated with HRCT signs of ILD. We have not found clinical factors predictive for a low DLCO. FAU - Quadrelli, Silvia AU - Quadrelli S AD - Instituto de Enfermedades Intersticiales del Hospital Británico. silvia_quadrelli@hotmail.com FAU - Ciallella, Lorena AU - Ciallella L FAU - Catalán Pellet, Antonio C AU - Catalán Pellet AC FAU - Molinari, Luciana AU - Molinari L FAU - Salvado, Alejandro AU - Salvado A FAU - Auad, Carolina AU - Auad C FAU - Spina, Juan Carlos AU - Spina JC LA - spa PT - Journal Article TT - Compromiso pulmonar en esclerosis sistémica. PL - Argentina TA - Medicina (B Aires) JT - Medicina JID - 0204271 RN - 0 (Biomarkers) RN - 0 (Nuclear Proteins) RN - 0 (Scl 70 antigen, human) RN - 7U1EE4V452 (Carbon Monoxide) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Biomarkers/analysis MH - Carbon Monoxide/analysis/metabolism MH - Child MH - DNA Topoisomerases, Type I MH - Female MH - Humans MH - Hypertension, Pulmonary/*diagnosis/etiology MH - Lung Diseases, Interstitial/diagnosis/*etiology/physiopathology MH - Male MH - Middle Aged MH - Nuclear Proteins/analysis MH - Pulmonary Diffusing Capacity/*physiology MH - Radiography MH - Retrospective Studies MH - Risk Factors MH - Scleroderma, Systemic/*complications/diagnostic imaging/immunology MH - Sensitivity and Specificity MH - Total Lung Capacity/physiology EDAT- 2007/12/07 09:00 MHDA- 2008/09/23 09:00 CRDT- 2007/12/07 09:00 PHST- 2007/12/07 09:00 [pubmed] PHST- 2008/09/23 09:00 [medline] PHST- 2007/12/07 09:00 [entrez] PST - ppublish SO - Medicina (B Aires). 2007;67(5):429-35. PMID- 20471130 OWN - NLM STAT- MEDLINE DCOM- 20100920 LR - 20120824 IS - 1600-0641 (Electronic) IS - 0168-8278 (Linking) VI - 53 IP - 1 DP - 2010 Jul TI - Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis. PG - 162-9 LID - 10.1016/j.jhep.2010.02.019 [doi] AB - BACKGROUND & AIMS: Primary biliary cirrhosis (PBC) is believed to result from the interaction of genetic and environmental factors. The controlled studies aiming to assess risk factors for PBC are still limited. Our aim was to identify risk factors and co-morbidities associated with PBC in a large monocentric cohort. METHODS: We enrolled 222 patients with PBC and 509 controls matched for age, gender, and residential location. Standardized questionnaire data, including more than 200 questions regarding demographic and anthropometric features, lifestyle, individual and familial medical history, and reproductive history, were prospectively collected and examined. Risk factors with odds ratio (OR) and confidence intervals (CI) were determined using conditional logistic regression analyses. RESULTS: Family history of PBC (OR 6.8, 95% CI 2.8-16.4) or autoimmune thyroid disease (AITD) (OR 7.1, 95% CI 3.5-14.5) in first-degree relatives, and individual history of active or passive smoking (OR 3.1, 95% CI 2.0-5.0), recurrent urinary tract infections (UTI) (OR 2.7; 95% CI 2.0-3.7), AITD (OR 7.7, 95% CI 4.8-12.3), Sjögren syndrome (OR 11.9, 95% CI 5.4-26.3), Raynaud syndrome (OR 7.2, 95% CI 4.3-12.1), pruritus during pregnancy (OR 3.9, 95% CI 2.8-5.3), or abortion (OR 2.0, 95% CI 1.6-2.5) were significantly associated with increased risk of PBC, while use of oral contraceptives (OR 0.6; 95% CI 0.5-0.8) was associated with decreased risk. CONCLUSION: This study confirms some of the previously reported risk factors for PBC, namely family history of disease and individual history of smoking, UTI, and autoimmune conditions, and further identifies the use of oral contraceptives as a putative protective factor. CI - Copyright 2010 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. FAU - Corpechot, Christophe AU - Corpechot C AD - Service d'Hépatologie, Centre de référence des Maladies Inflammatoires des Voies Biliaires, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France. christophe.corpechot@sat.aphp.fr FAU - Chrétien, Yves AU - Chrétien Y FAU - Chazouillères, Olivier AU - Chazouillères O FAU - Poupon, Raoul AU - Poupon R LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100331 PL - Netherlands TA - J Hepatol JT - Journal of hepatology JID - 8503886 SB - IM MH - Aged MH - Autoimmune Diseases/genetics MH - Case-Control Studies MH - Cohort Studies MH - Confidence Intervals MH - Female MH - France MH - Humans MH - Life Style MH - Liver Cirrhosis, Biliary/*etiology/genetics MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Odds Ratio MH - Pregnancy MH - Reproductive History MH - Risk Factors EDAT- 2010/05/18 06:00 MHDA- 2010/09/21 06:00 CRDT- 2010/05/18 06:00 PHST- 2009/12/24 00:00 [received] PHST- 2010/02/03 00:00 [revised] PHST- 2010/02/03 00:00 [accepted] PHST- 2010/05/18 06:00 [entrez] PHST- 2010/05/18 06:00 [pubmed] PHST- 2010/09/21 06:00 [medline] AID - S0168-8278(10)00182-0 [pii] AID - 10.1016/j.jhep.2010.02.019 [doi] PST - ppublish SO - J Hepatol. 2010 Jul;53(1):162-9. doi: 10.1016/j.jhep.2010.02.019. Epub 2010 Mar 31. PMID- 32333004 OWN - NLM STAT- MEDLINE DCOM- 20210122 LR - 20210122 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 59 IP - 11 DP - 2020 Nov 1 TI - Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets. PG - 3380-3389 LID - 10.1093/rheumatology/keaa127 [doi] AB - OBJECTIVES: Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (lcSSc, dcSSc and SSc-overlap syndromes). METHODS: Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (<40 years, 40-60 years, >60 years). RESULTS: Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40 years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the lcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (>60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. CONCLUSION: In this large registry, ∼25% of patients developed SSc at an age above 60 years with an increased frequency of lcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications. CI - © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Moinzadeh, Pia AU - Moinzadeh P AD - Department of Dermatology and Venereology, University Hospital Cologne. FAU - Kuhr, Kathrin AU - Kuhr K AD - Institute of Medical Statistics and Computational Biology, Faculty of Medicine, University of Cologne, Cologne. FAU - Siegert, Elise AU - Siegert E AD - Department of Rheumatology and Clinical Immunology, Charité - University Medicine Berlin and Berlin Institute of Health (BIH), Berlin. FAU - Mueller-Ladner, Ulf AU - Mueller-Ladner U AD - Department of Rheumatology, Justus Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology, University Medical Center-UKSH, Luebeck. FAU - Günther, Claudia AU - Günther C AD - Department of Dermatology, University Hospital Carl Gustav Carus, Dresden. FAU - Kötter, Ina AU - Kötter I AD - Department for Internal Medicine, Rheumatology, Immunology and Nephrology, Asklepios Clinic Altona, Hamburg. FAU - Henes, Jörg AU - Henes J AD - Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases and Department of Internal Medicine II (Oncology, Hematology, Immunology, Rheumatology, Pulmonology), University Hospital Tuebingen, Tuebingen. FAU - Blank, Norbert AU - Blank N AD - Department of Rheumatology, University Hospital Heidelberg, Heidelberg. FAU - Zeidler, Gabriele AU - Zeidler G AD - Department of Rheumatology, Johanniter-Hospital Treuenbrietzen, Treuenbrietzen. FAU - Pfeiffer, Christiane AU - Pfeiffer C AD - Department of Dermatology, University Hospital Ulm, Ulm. FAU - Juche, Aaron AU - Juche A AD - Department of Rheumatology, Immanuel Hospital Berlin-Buch, Berlin. FAU - Jandova, Ilona AU - Jandova I AD - Department of Rheumatology, University Hospital Freiburg, Freiburg. FAU - Ehrchen, Jan AU - Ehrchen J AD - Department of Dermatology and Venereology, University Hospital Muenster, Muenster. FAU - Schmalzing, Marc AU - Schmalzing M AD - Department of Internal Medicine, Rheumatology and Clinical Immunology, University Clinic of Wuerzburg, Wuerzburg. FAU - Susok, Laura AU - Susok L AD - Department of Dermatology and Venereology, Ruhr-University-Bochum, Bochum. FAU - Schmeiser, Tim AU - Schmeiser T AD - Department of Rheumatology and Immunology, St. Josef Hospital Wuppertal, Wuppertal. FAU - Sunderkoetter, Cord AU - Sunderkoetter C AD - Department of Dermatology and Venereology, University Hospital Halle, Halle. FAU - Distler, Jörg H W AU - Distler JHW AD - Department of Rheumatology, University Hospital Erlangen, Erlangen. FAU - Worm, Margitta AU - Worm M AD - Department of Dermatology and Allergology, Charité - University Medicine Berlin, Berlin. FAU - Kreuter, Alexander AU - Kreuter A AD - Department of Dermatology, Venereology and Allergology, Helios St. Elisabeth Hospital Oberhausen, University Witten-Herdecke, Oberhausen, Germany. FAU - Krieg, Thomas AU - Krieg T AD - Department of Dermatology and Venereology, University Hospital Cologne. FAU - Hunzelmann, Nicolas AU - Hunzelmann N AD - Department of Dermatology and Venereology, University Hospital Cologne. CN - Registry of the German Network for Systemic Scleroderma LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Age of Onset MH - Disease Progression MH - Female MH - Fingers MH - Germany/epidemiology MH - Humans MH - Hypertension, Pulmonary/etiology MH - Immunosuppressive Agents/therapeutic use MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Phenotype MH - Scleroderma, Systemic/drug therapy/epidemiology/*etiology MH - Skin Ulcer/etiology MH - Symptom Assessment PMC - PMC7590407 OTO - NOTNLM OT - German Network for Systemic Scleroderma OT - SSc OT - age at disease onset OT - older age OT - scleroderma OT - systemic sclerosis EDAT- 2020/04/26 06:00 MHDA- 2021/01/23 06:00 PMCR- 2020/04/24 CRDT- 2020/04/26 06:00 PHST- 2019/12/01 00:00 [received] PHST- 2020/01/27 00:00 [revised] PHST- 2020/04/26 06:00 [pubmed] PHST- 2021/01/23 06:00 [medline] PHST- 2020/04/26 06:00 [entrez] PHST- 2020/04/24 00:00 [pmc-release] AID - 5824949 [pii] AID - keaa127 [pii] AID - 10.1093/rheumatology/keaa127 [doi] PST - ppublish SO - Rheumatology (Oxford). 2020 Nov 1;59(11):3380-3389. doi: 10.1093/rheumatology/keaa127. PMID- 32687656 OWN - NLM STAT- MEDLINE DCOM- 20211012 LR - 20211012 IS - 1365-2230 (Electronic) IS - 0307-6938 (Linking) VI - 46 IP - 2 DP - 2021 Mar TI - Analysis of the first 5 years of an interdisciplinary Rheumatology-Dermatology clinic. PG - 270-275 LID - 10.1111/ced.14390 [doi] AB - BACKGROUND: Multispeciality clinics, such as combined psoriasis-psoriatic arthritis clinics, have shown improved outcomes in various diseases. At Massachusetts General Hospital, we are entering our ninth year of having an interdisciplinary Rheumatology-Dermatology (R-D) clinic. AIM: To evaluate the contribution of an R-D clinic by comparing care of patients pre- and post-evaluation in the combined clinic. As proxies of care, rates and comprehensiveness of evaluations (capillaroscopic examination, skin and joint examination) were compared between the combined clinic and standard Rheumatology or Dermatology clinic. METHODS: This was a retrospective chart review of patients at the R-D clinic in Massachusetts General Hospital during the period November 2012 to December 2017. RESULTS: Prior to the patients visiting the R-D only 5% of capillaroscopic examinations were documented, only 5% of rheumatologists specifically described a rash even when present, and pruritus was documented in only 6% of rheumatology notes. By contrast, in the R-D clinic, capillaroscopic, skin and joint examinations were documented in 100% of visits, and 19% of patients were given a different or a refined diagnosis. Although all our patients had cutaneous manifestations of their disease (hair loss, rash, itch, Raynaud phenomenon, ulcerations, calcinosis) only 34% had seen a dermatologist prior to the combined clinic and only 5% of those had had their concerns addressed by the rheumatologist. This suggests that 95% had a more complete evaluation and management of all aspects of their disease by attendance at the R-D clinic. CONCLUSION: Despite this study being limited by its retrospective nature, we found that it is an efficient model to achieve more comprehensive and potentially lower medication costs. Collaboration between dermatologists and rheumatologists in a combined clinic led to more complete skin and joint examinations, consistent tracking of capillaroscopic examination, better description of rash and improved management. Having this clinic helped in reaching a diagnosis and overall better disease control and outcome. CI - © 2020 British Association of Dermatologists. FAU - Argobi, Y AU - Argobi Y AUID- ORCID: 0000-0001-5343-5372 AD - Department of Dermatology, King Khalid University, Abha, Saudi Arabia. AD - Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA. FAU - Smith, G P AU - Smith GP AD - Department of Dermatology, King Khalid University, Abha, Saudi Arabia. AD - Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA. LA - eng PT - Comparative Study PT - Journal Article PT - Review DEP - 20201104 PL - England TA - Clin Exp Dermatol JT - Clinical and experimental dermatology JID - 7606847 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Ambulatory Care Facilities/*organization & administration/statistics & numerical data MH - Arthritis, Psoriatic/*diagnosis MH - Comprehensive Health Care/*methods/statistics & numerical data MH - Cooperative Behavior MH - Dermatology/standards MH - Female MH - Hospitals, General/organization & administration MH - Humans MH - Interdisciplinary Communication MH - Joint Diseases/diagnosis/pathology/therapy MH - Male MH - Massachusetts MH - Microscopic Angioscopy/methods/*statistics & numerical data MH - Middle Aged MH - Outcome Assessment, Health Care MH - Retrospective Studies MH - Rheumatologists/statistics & numerical data MH - Rheumatology/standards MH - Skin Diseases/diagnosis/pathology/therapy MH - Young Adult EDAT- 2020/07/21 06:00 MHDA- 2021/10/13 06:00 CRDT- 2020/07/21 06:00 PHST- 2020/06/29 00:00 [received] PHST- 2020/07/15 00:00 [accepted] PHST- 2020/07/21 06:00 [pubmed] PHST- 2021/10/13 06:00 [medline] PHST- 2020/07/21 06:00 [entrez] AID - 10.1111/ced.14390 [doi] PST - ppublish SO - Clin Exp Dermatol. 2021 Mar;46(2):270-275. doi: 10.1111/ced.14390. Epub 2020 Nov 4. PMID- 30567687 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230502 IS - 1929-073X (Print) IS - 1929-073X (Electronic) IS - 1929-073X (Linking) VI - 7 IP - 2 DP - 2018 Dec 19 TI - Extrahepatic Autoimmune Diseases are Prevalent in Autoimmune Hepatitis Patients and Their First-Degree Relatives: Survey Study. PG - e18 LID - 10.2196/ijmr.9625 [doi] LID - e18 AB - BACKGROUND: Concurrent autoimmune illnesses contribute to increased medical burden and reduced quality of life in patients with autoimmune hepatitis (AIH). The frequency of coexisting autoimmune conditions among North American patients with AIH and their families remains incomplete. Challenges associated with disease capture in the electronic medical record, high study costs, and geographic spread of patients are formidable barriers to understanding the extent of concurrent autoimmune conditions in these groups. OBJECTIVE: This objective of this study was to examine the frequency of extrahepatic autoimmune diseases (EHAD) among AIH cases and healthy controls as well as their first-degree relatives using social networking sites (SNS). METHODS: We developed a 53-question survey detailing the history of autoimmune diseases. A survey link was posted at routine intervals within specific Web-based cohorts on SNS. Healthy controls, without self-reported autoimmune liver disease, were recruited from Amazon's Mechanical Turk. Continuous variables were summarized using medians and P values obtained with the Wilcoxon rank-sum test. Categorical variables were compared using the chi-square test. RESULTS: Compared with controls (n=1162), cases (n=306) were more likely to be older (median age: 49 vs 33 years), female (284/306, 92.81% vs 955/1162, 82.18%), and have an EHAD (128/306, 41.83% vs 218/1162, 18.76%; P=.001). The most frequent EHADs among cases were thyroid disease (49/306, 16.01% ), Sjögren syndrome (27/306, 8.82%), Raynaud phenomenon (23/306, 7.52%), and psoriasis (22/306, 7.19%). Overall, 55.88% (171/306) of cases and 35.71% (1601/4484) of controls reported at least 1 first-degree relative (FDR) with a history of EHAD (P=.001). Cases had a significantly higher risk of EHAD than controls after the adjustment for age, sex, race, and body mass index: odds ratio 2.46 (95% CI 1.8-3.3); P=.001. CONCLUSIONS: Patients with AIH report higher prevalence of coexistent EHAD than healthy controls, and their FDRs are also more likely to have autoimmune disorders. CI - ©Rachel Fogel, Megan Comerford, Prianka Chilukuri, Eric Orman, Naga Chalasani, Craig Lammert. Originally published in the Interactive Journal of Medical Research (http://www.i-jmr.org/), 19.12.2018. FAU - Fogel, Rachel AU - Fogel R AUID- ORCID: 0000-0002-0251-1547 AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. FAU - Comerford, Megan AU - Comerford M AUID- ORCID: 0000-0003-3891-1451 AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. FAU - Chilukuri, Prianka AU - Chilukuri P AUID- ORCID: 0000-0003-0792-9720 AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. FAU - Orman, Eric AU - Orman E AUID- ORCID: 0000-0002-8295-9076 AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. FAU - Chalasani, Naga AU - Chalasani N AUID- ORCID: 0000-0003-4082-3178 AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. FAU - Lammert, Craig AU - Lammert C AUID- ORCID: 0000-0003-3809-640X AD - Division of Gastroenterology and Hepatology, Indiana University, Indianapolis, IN, United States. LA - eng GR - K23 DK109202/DK/NIDDK NIH HHS/United States GR - K23 DK114561/DK/NIDDK NIH HHS/United States GR - KL2 TR001106/TR/NCATS NIH HHS/United States GR - UL1 TR001108/TR/NCATS NIH HHS/United States PT - Journal Article DEP - 20181219 PL - Canada TA - Interact J Med Res JT - Interactive journal of medical research JID - 101598421 PMC - PMC6315230 OTO - NOTNLM OT - autoimmune hepatitis OT - first-degree relatives OT - social media COIS- Conflicts of Interest: None declared. EDAT- 2018/12/21 06:00 MHDA- 2018/12/21 06:01 PMCR- 2018/12/19 CRDT- 2018/12/21 06:00 PHST- 2017/12/11 00:00 [received] PHST- 2018/07/17 00:00 [accepted] PHST- 2018/07/11 00:00 [revised] PHST- 2018/12/21 06:00 [entrez] PHST- 2018/12/21 06:00 [pubmed] PHST- 2018/12/21 06:01 [medline] PHST- 2018/12/19 00:00 [pmc-release] AID - v7i2e18 [pii] AID - 10.2196/ijmr.9625 [doi] PST - epublish SO - Interact J Med Res. 2018 Dec 19;7(2):e18. doi: 10.2196/ijmr.9625. PMID- 36906866 OWN - NLM STAT- MEDLINE DCOM- 20231025 LR - 20250801 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 43 IP - 8 DP - 2023 Aug TI - Pediatric mixed connective tissue disease versus other overlap syndromes: a retrospective multicenter cohort study. PG - 1485-1495 LID - 10.1007/s00296-023-05300-x [doi] AB - Pediatric mixed connective tissue disease (MCTD) is a subgroup of overlap syndromes. We aimed to compare the characteristics and outcomes in children with MCTD and other overlap syndromes. All MCTD patients met either Kasukawa or Alarcon-Segovia and Villareal criteria. The patients with other overlap syndromes had the features of ≥ 2 autoimmune rheumatic diseases but did not meet MCTD diagnostic criteria. Thirty MCTD (F/M = 28/2) and thirty (F/M = 29/1) overlap patients were included (disease onset < 18 years). The most prominent phenotype at disease onset and the last visit was systemic lupus erythematosus (SLE) in the MCTD group; juvenile idiopathic arthritis and dermatomyositis/polymyositis, respectively, in the overlap group. At the last visit, systemic sclerosis (SSc) phenotype was more frequent among MCTD than overlap patients (60% vs. 33.3%; p = 0.038). The frequency of the predominant SLE phenotype had decreased (60% to 36.7%), while predominant SSc phenotype had increased (13.3% to 33.3%) during follow-up in MCTD patients. Weight loss (36.7% vs. 13.3%), digital ulcers (20% vs. 0), swollen hands (60% vs. 20%), Raynaud phenomenon (86.7% vs. 46.7%), hematologic involvement (70% vs. 26.7%), and anti-Sm positivity (29% vs. 3.3%) were more common, while Gottron papules (16.7% vs. 40%) were less frequent among MCTD than overlap patients (p < 0.05). A higher percentage of overlap patients achieved complete remission than MCTD patients (51.7% vs. 24.1%; p = 0.047). The disease phenotype and outcome differ between pediatric MCTD and other overlap syndromes where MCTD may be regarded as a more severe disease. Analyzing these patients could pave the way for early and effective treatment. CI - © 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Batu, Ezgi Deniz AU - Batu ED AUID- ORCID: 0000-0003-1065-2363 AD - Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. ezgidenizbatu@yahoo.com. AD - Çocuk Romatoloji Bölümü, Hacettepe Üniversitesi İhsan Doğramacı Çocuk Hastanesi, Kat: 3 Sıhhiye, 06100, Ankara, Turkey. ezgidenizbatu@yahoo.com. FAU - Günalp, Aybüke AU - Günalp A AUID- ORCID: 0000-0003-0137-0460 AD - Cerrahpasa Medical School, Department of Pediatrics, Division of Rheumatology, Istanbul University Cerrahpasa, Istanbul, Turkey. FAU - Şahin, Sezgin AU - Şahin S AUID- ORCID: 0000-0002-5365-3457 AD - Cerrahpasa Medical School, Department of Pediatrics, Division of Rheumatology, Istanbul University Cerrahpasa, Istanbul, Turkey. FAU - Özdel, Semanur AU - Özdel S AUID- ORCID: 0000-0001-5602-4595 AD - Dr. Sami Ulus Maternity and Child Health and Diseases Research and Training Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Ankara, Turkey. FAU - Kızıldağ, Zehra AU - Kızıldağ Z AUID- ORCID: 0000-0002-7938-2560 AD - Department of Pediatrics, Division of Rheumatology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey. FAU - Pac Kısaarslan, Aysenur AU - Pac Kısaarslan A AUID- ORCID: 0000-0002-1800-9922 AD - Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Erciyes University, Kayseri, Turkey. FAU - Bağrul, İlknur AU - Bağrul İ AUID- ORCID: 0000-0002-5585-0198 AD - Dr. Sami Ulus Maternity and Child Health and Diseases Research and Training Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Ankara, Turkey. FAU - Kasap Cuceoglu, Muserref AU - Kasap Cuceoglu M AUID- ORCID: 0000-0002-9957-8894 AD - Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Tanatar, Ayşe AU - Tanatar A AUID- ORCID: 0000-0002-1386-4575 AD - Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Istanbul University, Istanbul, Turkey. FAU - Sonmez, Hafize Emine AU - Sonmez HE AUID- ORCID: 0000-0002-9186-3068 AD - Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Kocaeli University, Kocaeli, Turkey. FAU - Sag, Erdal AU - Sag E AUID- ORCID: 0000-0002-6542-2656 AD - Ankara Research and Training Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Ankara, Turkey. FAU - Demir, Selcan AU - Demir S AUID- ORCID: 0000-0002-4320-8632 AD - Department of Pediatrics, Division of Rheumatology, Erzurum Regional Research and Training Hospital, Erzurum, Turkey. FAU - Çelikel, Elif AU - Çelikel E AUID- ORCID: 0000-0003-0129-4410 AD - Ankara City Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Ankara, Turkey. FAU - Cağlayan, Sengul AU - Cağlayan S AUID- ORCID: 0000-0003-3014-5692 AD - Umraniye Research and Training Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Istanbul, Turkey. FAU - Çelikel Acar, Banu AU - Çelikel Acar B AUID- ORCID: 0000-0002-1808-3655 AD - Ankara City Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Ankara, Turkey. FAU - Sözeri, Betül AU - Sözeri B AUID- ORCID: 0000-0002-5079-5644 AD - Umraniye Research and Training Hospital, Department of Pediatrics, Division of Rheumatology, University of Health Sciences, Istanbul, Turkey. FAU - Aktay Ayaz, Nuray AU - Aktay Ayaz N AUID- ORCID: 0000-0003-3594-7387 AD - Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Istanbul University, Istanbul, Turkey. FAU - Bilginer, Yelda AU - Bilginer Y AUID- ORCID: 0000-0002-6232-0072 AD - Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. FAU - Poyrazoğlu, M Hakan AU - Poyrazoğlu MH AUID- ORCID: 0000-0002-5142-8432 AD - Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Erciyes University, Kayseri, Turkey. FAU - Ünsal, Erbil AU - Ünsal E AUID- ORCID: 0000-0002-8800-0800 AD - Department of Pediatrics, Division of Rheumatology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey. FAU - Kasapçopur, Özgür AU - Kasapçopur Ö AUID- ORCID: 0000-0002-1125-7720 AD - Cerrahpasa Medical School, Department of Pediatrics, Division of Rheumatology, Istanbul University Cerrahpasa, Istanbul, Turkey. FAU - Özen, Seza AU - Özen S AUID- ORCID: 0000-0003-2883-7868 AD - Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20230312 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 SB - IM MH - Retrospective Studies MH - Humans MH - Child MH - *Mixed Connective Tissue Disease MH - *Scleroderma, Systemic MH - *Lupus Erythematosus, Systemic MH - Cohort Studies MH - Autoimmune Diseases OTO - NOTNLM OT - Adolescent OT - Autoimmune diseases OT - Child OT - Mixed connective tissue disease EDAT- 2023/03/13 06:00 MHDA- 2023/06/14 06:42 CRDT- 2023/03/12 14:14 PHST- 2023/01/07 00:00 [received] PHST- 2023/02/27 00:00 [accepted] PHST- 2023/06/14 06:42 [medline] PHST- 2023/03/13 06:00 [pubmed] PHST- 2023/03/12 14:14 [entrez] AID - 10.1007/s00296-023-05300-x [pii] AID - 10.1007/s00296-023-05300-x [doi] PST - ppublish SO - Rheumatol Int. 2023 Aug;43(8):1485-1495. doi: 10.1007/s00296-023-05300-x. Epub 2023 Mar 12. PMID- 29596085 OWN - NLM STAT- MEDLINE DCOM- 20190918 LR - 20190918 IS - 1536-3708 (Electronic) IS - 0148-7043 (Linking) VI - 80 IP - 4 Suppl 4 DP - 2018 Apr TI - The Influence of Connective Tissue Disease in Breast Reconstruction: A National Database Analysis. PG - S182-S188 LID - 10.1097/SAP.0000000000001387 [doi] AB - BACKGROUND: Patients with connective tissue diseases (CTD), or collagen vascular diseases, are at risk of potentially higher morbidity after surgical procedures. We aimed to investigate the complication profile in CTD versus non-CTD patients who underwent breast reconstruction on a national scale. METHODS: A retrospective analysis of the Healthcare Cost and Utilization Project NIS Database between 2010 and 2014 was conducted for patients 18 years or older admitted for immediate autologous or implant breast reconstruction. Connective tissue disease was defined as systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, scleroderma, Raynaud phenomenon, psoriatic arthritis, or sarcoidosis. Independent t test/Wilcoxon-Mann-Whitney was used to compare continuous variables and Pearson χ/Fischer exact test was used for categorical variables. Outcomes of interest were assessed using multivariable linear regressions for continuous variables and multivariable logistic regressions for categorical variables. RESULTS: There were 19,496 immediate autologous breast reconstruction patients, with 357 CTD and 19,139 non-CTD patients (2010-2014). The CTD patients had higher postoperative complication rates for infection (2.8% vs 0.8%, P < 0.001), wound dehiscence (1.4% vs 0.4%, P = 0.019), and bleeding (hemorrhage and hematoma) (6.7% vs 3.5%, P < 0.001). After multivariable analysis, CTD remained an independent risk factor for bleeding (odds ratio [OR], 1.568; 95% confidence interval [CI], 1.019-2.412). There were a total of 23,048 immediate implant breast reconstruction patients, with 431 CTD and 22,617 non-CTD patients (2010-2014). The CTD patients had a higher postoperative complication rate for wound dehiscence/complication (2.3% vs 0.6%, P < 0.001). They also experienced a longer length of stay (2.31 days vs 2.07 days, P < 0.001). After multivariable analysis, CTD remained an independent risk factor for wound dehiscence (OR, 4.084; 95% CI, 2.101-7.939) and increased length of stay by 0.050 days (95% CI, -0.081 to 0.181). CONCLUSIONS: Connective tissue disease patients who underwent autologous breast reconstruction had significantly higher infection, wound dehiscence, and bleeding rates, and those who underwent implant breast reconstruction had significantly higher wound dehiscence rates. Connective tissue diseases appear to be an independent risk factor for bleeding and wound dehiscence in autologous and implant breast reconstruction, respectively. This information may help clinicians be aware of this increased risk when determining patients for reconstruction. FAU - Chen, Austin D AU - Chen AD AD - From the Division of Plastic and Reconstructive Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA. FAU - Chi, David AU - Chi D FAU - Wu, Winona W AU - Wu WW FAU - Egeler, Sabine A AU - Egeler SA FAU - Chattha, Anmol S AU - Chattha AS FAU - Bucknor, Alexandra AU - Bucknor A FAU - Lee, Bernard T AU - Lee BT FAU - Lin, Samuel J AU - Lin SJ LA - eng PT - Journal Article PL - United States TA - Ann Plast Surg JT - Annals of plastic surgery JID - 7805336 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Connective Tissue Diseases/*complications MH - Databases, Factual MH - Female MH - Follow-Up Studies MH - Humans MH - Linear Models MH - Logistic Models MH - *Mammaplasty MH - Middle Aged MH - Postoperative Complications/epidemiology/*etiology MH - Retrospective Studies MH - Risk Factors MH - Young Adult EDAT- 2018/03/30 06:00 MHDA- 2019/09/19 06:00 CRDT- 2018/03/30 06:00 PHST- 2018/03/30 06:00 [pubmed] PHST- 2019/09/19 06:00 [medline] PHST- 2018/03/30 06:00 [entrez] AID - 10.1097/SAP.0000000000001387 [doi] PST - ppublish SO - Ann Plast Surg. 2018 Apr;80(4 Suppl 4):S182-S188. doi: 10.1097/SAP.0000000000001387. PMID- 36849541 OWN - NLM STAT- MEDLINE DCOM- 20230329 LR - 20241001 IS - 1759-4804 (Electronic) IS - 1759-4790 (Print) IS - 1759-4790 (Linking) VI - 19 IP - 4 DP - 2023 Apr TI - State-of-the-art evidence in the treatment of systemic sclerosis. PG - 212-226 LID - 10.1038/s41584-023-00909-5 [doi] AB - Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evident from randomized clinical trials. Treatments for early dcSSc include immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab and tocilizumab. Patients with rapidly progressive early dcSSc might be eligible for autologous haematopoietic stem cell transplantation, which can improve survival. Morbidity from interstitial lung disease and pulmonary arterial hypertension is improving with the use of proven therapies. Mycophenolate mofetil has surpassed cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Nintedanib and possibly perfinidone can be considered in SSc pulmonary fibrosis. Pulmonary arterial hypertension is frequently treated with initial combination therapy (for example, with phosphodiesterase 5 inhibitors and endothelin receptor antagonists) and, if necessary, the addition of a prostacyclin analogue. Raynaud phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers (especially nifedipine), then phosphodiesterase 5 inhibitors or intravenous iloprost. Bosentan can reduce the development of new digital ulcers. Trial data for other manifestations are mostly lacking. Research is needed to develop targeted and highly effective treatments, best practices for organ-specific screening and early intervention, and sensitive outcome measurements. CI - © 2023. Springer Nature Limited. FAU - Pope, Janet E AU - Pope JE AUID- ORCID: 0000-0003-1479-5302 AD - Division of Rheumatology, St Joseph's Health Care, London, ON, Canada. janet.pope@sjhc.london.on.ca. AD - Department of Medicine, Schulich School of Medicine & Dentistry, University of Western Ontario, London, ON, Canada. janet.pope@sjhc.london.on.ca. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - Division of Medicine, University College London, London, UK. FAU - Johnson, Sindhu R AU - Johnson SR AUID- ORCID: 0000-0003-0591-2976 AD - Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital, Toronto, ON, Canada. AD - Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada. FAU - Fernandez-Codina, Andreu AU - Fernandez-Codina A AD - Division of Rheumatology, St Joseph's Health Care, London, ON, Canada. AD - General Internal Medicine, Windsor Regional Hospital, Windsor, ON, Canada. AD - Critical Care, Emergency and Systemic Autoimmune Diseases, Hospital Clinic, Barcelona, Spain. FAU - Hudson, Marie AU - Hudson M AD - Department of Medicine, McGill University, Montreal, QC, Canada. AD - Division of Rheumatology and Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada. FAU - Nevskaya, Tatiana AU - Nevskaya T AD - Division of Rheumatology, St Joseph's Health Care, London, ON, Canada. LA - eng PT - Journal Article PT - Review DEP - 20230227 PL - United States TA - Nat Rev Rheumatol JT - Nature reviews. Rheumatology JID - 101500080 RN - HU9DX48N0T (Mycophenolic Acid) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 8N3DW7272P (Cyclophosphamide) RN - digital ulcers SB - IM CIN - Nat Rev Rheumatol. 2023 Oct;19(10):675. doi: 10.1038/s41584-023-01013-4. PMID: 37605003 CIN - Nat Rev Rheumatol. 2023 Oct;19(10):676. doi: 10.1038/s41584-023-01014-3. PMID: 37605004 MH - Mycophenolic Acid/therapeutic use MH - *Pulmonary Arterial Hypertension/drug therapy MH - Skin Ulcer MH - Humans MH - *Scleroderma, Systemic/drug therapy MH - Phosphodiesterase 5 Inhibitors/therapeutic use MH - Cyclophosphamide/therapeutic use MH - *Lung Diseases, Interstitial/drug therapy PMC - PMC9970138 COIS- J.E.P. declares that she has research grants from AbbVie, Bayer, BI, BMS, Frensenius Kabi, Lilly, Mallinckrodt Pharmaceuticals, Merck, Roche, Seattle Genetics; that she has consulted for AbbVie, Amgen, BI, BMS, Celltrion, EMERALD, Frensenius Kabi, Galapagos, Gilead, Janssen, Lilly, Mallinckrodt Pharmaceuticals, Medexus, Merck, Mitsubishi Tanabe Pharma, Novartis, Pfizer, Roche, Sandoz, Samsung, Sanofi, Sobi, Teva, Viatris; and that she has been a speaker or attended an advisory board for AbbVie, Amgen, BI, BMS, Frensenius Kabi, Galapagos, Gilead, Janssen, Lilly, Merck, Novartis, Pfizer, Sandoz, Sanofi, UCB. C.P.D. declares that he has received consultancy or speaker fees from GlaxoSmithKline, Roche, Boehringer Ingelheim, Sanofi-Aventis, Galapagos, Inventiva, Corbus, Acceleron, Horizon, Gesynta; and that he has received research grants to his institution from GlaxoSmithKline, ARXX Therapeutics, Servier and Horizon Therapeutics. S.R.J. declares that he has been a site investigator for clinical trials sponsored by Bayer, Boehringer Ingelheim, Corbus, GlaxoSmithKline; that he has served on advisory boards for Boehringer Ingelheim, Corbus and Ikaria; and that he has been supported by the Oscar and Eleonor Markovitz Scleroderma Research Fund and the Gurmej Kaur Dhanda Scleroderma Research Fund. A.F.-C. declares that he has received grant support from the Scleroderma Society of Ontario and honoraria from Actelion, Bayer, Boehringer-Ingelheim. M.H. declares that she has received research grants from Boehringer Ingelheim and Bristol Myers Squibb and that she has participated in advisory boards for Boehringer Ingelheim, Alexion and Mallinckrodt. T.N. declares no competing interests. EDAT- 2023/02/28 06:00 MHDA- 2023/03/29 06:05 PMCR- 2023/02/27 CRDT- 2023/02/27 23:49 PHST- 2023/01/06 00:00 [accepted] PHST- 2023/03/29 06:05 [medline] PHST- 2023/02/28 06:00 [pubmed] PHST- 2023/02/27 23:49 [entrez] PHST- 2023/02/27 00:00 [pmc-release] AID - 10.1038/s41584-023-00909-5 [pii] AID - 909 [pii] AID - 10.1038/s41584-023-00909-5 [doi] PST - ppublish SO - Nat Rev Rheumatol. 2023 Apr;19(4):212-226. doi: 10.1038/s41584-023-00909-5. Epub 2023 Feb 27. PMID- 32278576 OWN - NLM STAT- MEDLINE DCOM- 20210312 LR - 20210312 IS - 1097-6809 (Electronic) IS - 0741-5214 (Linking) VI - 72 IP - 4 DP - 2020 Oct TI - Safety and feasibility of transradial infrainguinal peripheral arterial disease interventions. PG - 1237-1246.e1 LID - S0741-5214(20)30288-3 [pii] LID - 10.1016/j.jvs.2020.02.016 [doi] AB - OBJECTIVE: Transradial access (TRA) has traditionally been favored for coronary interventions. Tools with up to 200 cm length now allow operators to treat infrainguinal peripheral arterial disease (PAD) using TRA. This study aims to assess the safety and feasibility of TRA infrainguinal interventions. METHODS: Patients with infrainguinal PAD who underwent intervention via TRA from July 2013 through June 2019 were retrospectively reviewed. Exclusion criteria included Barbeau D waveform, a radial artery diameter of greater than 2 mm, radial artery occlusion, Raynaud syndrome, or peripheral vasculitis. Procedural success (adequate inline flow to the foot), TRA alone failure (crossover or use of an additional access site), clinical success (defined as improvement in ankle brachial index, clinical symptoms, or wound healing) and adverse events were recorded from procedure notes and follow-up visits. RESULTS: Thirty-six procedures were attempted using TRA in 32 patients (mean age, 65.8 years; range, 29-86; 22 male, 14 female) with mean height of 65.8 inches (range, 59.0-72.0 inches) and a body mass index of 28.7 (range, 19.1-43.9). Preprocedure Rutherford classification (II/III/IV/V/VI) was 8/15/2/7/4, respectively. The left radial artery was used for 35 of 36 procedures (97.2%). Treated vessels included the common femoral (n = 4), superficial femoral (n = 25), deep femoral (n = 1), popliteal (n = 10), tibioperoneal trunk (n = 2), tibial (n = 4), and plantar (n = 1) arteries. Interventions included angioplasty (n = 32, 100%), atherectomy (n = 8, 25%), and stenting (n = 13, 41%). Procedural success was 100%, the TRA alone failure rate was 11.1%, and clinical success was 89.3%. The median follow-up was 286.5 days (range, 0-919 days). Adverse events included radial artery pseudoaneurysm (n = 1), access site hematoma/bleeding (n = 3), radial artery occlusion (n = 1), groin hematoma (n = 1), popliteal artery dissection treated with stenting (n = 2), and a small superficial femoral artery perforation (n = 1) treated with prolonged balloon tamponade. No patients experienced signs of cerebrovascular events or distal embolism. CONCLUSIONS: TRA is a useful option for treating patients with PAD; however, several limitations still exist. CI - Copyright © 2020 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved. FAU - Sher, Alex AU - Sher A AD - Division of Interventional Radiology, The Mount Sinai Medical Center, New York, NY. FAU - Posham, Raghuram AU - Posham R AD - Division of Interventional Radiology, The Mount Sinai Medical Center, New York, NY. FAU - Vouyouka, Ageliki AU - Vouyouka A AD - Division of Vascular Surgery, The Mount Sinai Medical Center, New York, NY. FAU - Patel, Rahul AU - Patel R AD - Division of Interventional Radiology, The Mount Sinai Medical Center, New York, NY. FAU - Lookstein, Robert AU - Lookstein R AD - Division of Interventional Radiology, The Mount Sinai Medical Center, New York, NY. FAU - Faries, Peter L AU - Faries PL AD - Division of Vascular Surgery, The Mount Sinai Medical Center, New York, NY. FAU - Fischman, Aaron AU - Fischman A AD - Division of Interventional Radiology, The Mount Sinai Medical Center, New York, NY. FAU - Tadros, Rami AU - Tadros R AD - Division of Vascular Surgery, The Mount Sinai Medical Center, New York, NY. Electronic address: rami.tadros@mountsinai.org. LA - eng PT - Journal Article DEP - 20200408 PL - United States TA - J Vasc Surg JT - Journal of vascular surgery JID - 8407742 SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Angioplasty/*adverse effects/instrumentation/methods MH - Case-Control Studies MH - Feasibility Studies MH - Female MH - Femoral Artery/surgery MH - Follow-Up Studies MH - Humans MH - Lower Extremity/blood supply MH - Male MH - Middle Aged MH - Peripheral Arterial Disease/*surgery MH - Popliteal Artery/surgery MH - Postoperative Complications/*epidemiology/etiology MH - Radial Artery/*surgery MH - Retrospective Studies MH - Stents MH - Tibial Arteries/surgery MH - Treatment Outcome OTO - NOTNLM OT - Endovascular procedures OT - Peripheral arterial disease OT - Radial artery EDAT- 2020/04/13 06:00 MHDA- 2021/03/13 06:00 CRDT- 2020/04/13 06:00 PHST- 2019/10/15 00:00 [received] PHST- 2020/02/06 00:00 [accepted] PHST- 2020/04/13 06:00 [pubmed] PHST- 2021/03/13 06:00 [medline] PHST- 2020/04/13 06:00 [entrez] AID - S0741-5214(20)30288-3 [pii] AID - 10.1016/j.jvs.2020.02.016 [doi] PST - ppublish SO - J Vasc Surg. 2020 Oct;72(4):1237-1246.e1. doi: 10.1016/j.jvs.2020.02.016. Epub 2020 Apr 8. PMID- 40360431 OWN - NLM STAT- MEDLINE DCOM- 20250514 LR - 20260512 IS - 2056-5933 (Electronic) IS - 2056-5933 (Linking) VI - 11 IP - 2 DP - 2025 May 13 TI - Association of mitochondrial RNA expression levels in saliva and plasma with interferon signature gene expression and disease activity in patients with Sjögren disease. LID - 10.1136/rmdopen-2024-005166 [doi] LID - e005166 AB - OBJECTIVE: To unveil the clinical implications of mitochondrial RNAs (mt-RNAs) in Sjögren disease (SjD), this study evaluated mt-RNA expression levels in the plasma and saliva of patients with SS and their association with SjD-related features. METHODS: Plasma, saliva and/or peripheral blood mononuclear cells (PBMCs) were collected from 111 patients with SjD and 35 healthy controls (HCs), with 40 rheumatoid arthritis (RA) and 40 systemic lupus erythematosus (SLE) disease controls. The expression levels of mt-RNAs and interferon-stimulated genes (ISGs) were quantified by real-time PCR. Composite mt-RNA and ISG scores were calculated using logistic regression models. Their discriminative power was evaluated using receiver operating characteristic curve analyses, and correlations with clinical data were explored. RESULTS: Altered mt-RNA expression in saliva or plasma and ISG expression in PBMCs were detected in patients with SjD, compared with HCs. Saliva and plasma mt-RNA scores showed better discriminative ability (area under the curve values=0.847 and 0.789, respectively) than ISG scores in distinguishing SjD from HCs. Plasma mt-RNA scores were significantly higher in patients with SjD than in those with RA and SLE (p<0.05). Saliva mt-RNA scores were positively associated with objective disease activity measures and Raynaud phenomenon in patients with SjD, whereas plasma mt-RNA scores did not show this association. RA and SLE disease activity correlated with plasma mt-RNA scores. CONCLUSIONS: Extracellular mt-RNA burden is elevated in SjD, and mt-RNA scores effectively discriminated patients with SjD from HCs. Saliva mt-RNA levels were associated with SjD disease activity, suggesting their potential utility in disease monitoring and stratification of SjD. CI - © Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY. Published by BMJ Group. FAU - Ha, You-Jung AU - Ha YJ AUID- ORCID: 0000-0001-6107-9523 AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). AD - Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea (the Republic of). FAU - Choi, Yong Seok AU - Choi YS AD - Medical Science Research Institute, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). FAU - Choi, Se Rim AU - Choi SR AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). FAU - Yoon, Jimin AU - Yoon J AD - Department of Chemical and Biomolecular Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea (the Republic of). FAU - Ku, Doyeong AU - Ku D AD - Department of Chemical and Biomolecular Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea (the Republic of). FAU - Kim, Yoosik AU - Kim Y AD - Department of Chemical and Biomolecular Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea (the Republic of). FAU - Kang, Eun Ha AU - Kang EH AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). AD - Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea (the Republic of). FAU - Kim, Keun-Suh AU - Kim KS AD - Department of Periodontology, Section of Dentistry, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). FAU - Jeong, Woo-Jin AU - Jeong WJ AD - Department of Otorhinolaryngology - Head & Neck Surgery, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). FAU - Hyon, Joon Young AU - Hyon JY AD - Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of). FAU - Cha, Seunghee AU - Cha S AUID- ORCID: 0000-0003-3772-1832 AD - Division of Oral Medicine, Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida, Gainesville, Florida, USA. FAU - Lee, Yun Jong AU - Lee YJ AUID- ORCID: 0000-0001-7615-8611 AD - Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Gyeonggi, Korea (the Republic of) yn35@snu.ac.kr. AD - Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea (the Republic of). AD - Department of Medical Device Development, Seoul National University College of Medicine, Seoul, Republic of Korea. LA - eng GR - R01 DE032707/DE/NIDCR NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20250513 PL - England TA - RMD Open JT - RMD open JID - 101662038 RN - 0 (RNA, Mitochondrial) RN - 9008-11-1 (Interferons) RN - 0 (Biomarkers) SB - IM MH - Humans MH - *Sjogren's Syndrome/genetics/diagnosis/blood/metabolism MH - *Saliva/metabolism MH - Female MH - Male MH - Middle Aged MH - *RNA, Mitochondrial/genetics/blood/metabolism MH - Adult MH - *Interferons/genetics/metabolism MH - Biomarkers MH - Leukocytes, Mononuclear/metabolism MH - ROC Curve MH - Gene Expression Regulation MH - Case-Control Studies MH - Arthritis, Rheumatoid MH - Aged PMC - PMC12083325 OTO - NOTNLM OT - Autoimmune Diseases OT - Biomarkers OT - Sjogren's Syndrome COIS- Competing interests: None declared. EDAT- 2025/05/14 11:42 MHDA- 2025/05/14 11:43 PMCR- 2025/05/13 CRDT- 2025/05/13 22:12 PHST- 2024/10/21 00:00 [received] PHST- 2025/04/23 00:00 [accepted] PHST- 2025/05/14 11:43 [medline] PHST- 2025/05/14 11:42 [pubmed] PHST- 2025/05/13 22:12 [entrez] PHST- 2025/05/13 00:00 [pmc-release] AID - rmdopen-2024-005166 [pii] AID - 10.1136/rmdopen-2024-005166 [doi] PST - epublish SO - RMD Open. 2025 May 13;11(2):e005166. doi: 10.1136/rmdopen-2024-005166. PMID- 32521358 OWN - NLM STAT- MEDLINE DCOM- 20200717 LR - 20260518 IS - 1879-1360 (Electronic) IS - 0022-3999 (Print) IS - 0022-3999 (Linking) VI - 135 DP - 2020 Aug TI - Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients. PG - 110132 LID - S0022-3999(20)30400-1 [pii] LID - 10.1016/j.jpsychores.2020.110132 [doi] AB - OBJECTIVE: Contagious disease outbreaks and related restrictions can lead to negative psychological outcomes, particularly in vulnerable populations at risk due to pre-existing medical conditions. No randomised controlled trials (RCTs) have tested interventions to reduce mental health consequences of contagious disease outbreaks. The primary objective of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Trial is to evaluate the effect of a videoconference-based program on symptoms of anxiety. Secondary objectives include evaluating effects on symptoms of depression, stress, loneliness, boredom, physical activity, and social interaction. METHODS: The SPIN-CHAT Trial is a pragmatic RCT that will be conducted using the SPIN-COVID-19 Cohort, a sub-cohort of the SPIN Cohort. Eligible participants will be SPIN-COVID-19 Cohort participants without a positive COVID-19 test, with at least mild anxiety (PROMIS Anxiety 4a v1.0 T-score ≥ 55), not working from home, and not receiving current counselling or psychotherapy. We will randomly assign 162 participants to intervention groups of 7 to 10 participants each or waitlist control. We will use a partially nested RCT design to reflect dependence between individuals in training groups but not in the waitlist control. The SPIN-CHAT Program includes activity engagement, education on strategies to support mental health, and mutual participant support. Intervention participants will receive the 4-week (3 sessions per week) SPIN-CHAT Program via videoconference. The primary outcome is PROMIS Anxiety 4a score immediately post-intervention. ETHICS AND DISSEMINATION: The SPIN-CHAT Trial will test whether a brief videoconference-based intervention will improve mental health outcomes among at-risk individuals during contagious disease outbreak. CI - Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Thombs, Brett D AU - Thombs BD AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Medicine, McGill University, Montreal, Quebec, Canada; Department of Psychology, McGill University, Montreal, Quebec, Canada; Department of Educational and Counselling Psychology, McGill University, Montreal, Quebec, Canada; Biomedical Ethics Unit, McGill University, Montreal, Quebec, Canada. Electronic address: brett.thombs@mcgill.ca. FAU - Kwakkenbos, Linda AU - Kwakkenbos L AD - Department of Clinical Psychology, Behavioural Science Institute, Radboud University, Nijmegen, the Netherlands. FAU - Carrier, Marie-Eve AU - Carrier ME AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Bourgeault, Angelica AU - Bourgeault A AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Tao, Lydia AU - Tao L AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Harb, Sami AU - Harb S AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Gagarine, Maria AU - Gagarine M AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Rice, Danielle AU - Rice D AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychology, McGill University, Montreal, Quebec, Canada. FAU - Bustamante, Laura AU - Bustamante L AD - Department of Applied Human Sciences, Concordia University, Montreal, Quebec, Canada. FAU - Ellis, Kelsey AU - Ellis K AD - Faculty of Kinesiology, University of Calgary, Calgary, Alberta, Canada. FAU - Duchek, Delaney AU - Duchek D AD - Faculty of Kinesiology, University of Calgary, Calgary, Alberta, Canada. FAU - Wu, Yin AU - Wu Y AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Bhandari, Parash Mani AU - Bhandari PM AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Neupane, Dipika AU - Neupane D AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada. FAU - Carboni-Jiménez, Andrea AU - Carboni-Jiménez A AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Henry, Richard S AU - Henry RS AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada. FAU - Krishnan, Ankur AU - Krishnan A AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Sun, Ying AU - Sun Y AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Levis, Brooke AU - Levis B AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Centre for Prognosis Research, School of Primary, Community and Social Care, Keele University, Staffordshire, UK. FAU - He, Chen AU - He C AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Turner, Kimberly A AU - Turner KA AD - Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada. FAU - Benedetti, Andrea AU - Benedetti A AD - Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Medicine, McGill University, Montreal, Quebec, Canada; Respiratory Epidemiology and Clinical Research Unit, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Culos-Reed, Nicole AU - Culos-Reed N AD - Department of Applied Human Sciences, Concordia University, Montreal, Quebec, Canada; Department of Oncology, Cumming School of Medicine, Calgary, Canada; Department of Psychosocial Resources, Tom Baker Cancer Centre, Alberta Health Services, Calgary, Alberta, Canada. FAU - El-Baalbaki, Ghassan AU - El-Baalbaki G AD - Department of Psychology, Université du Québec à Montréal, Montreal, Quebec, Canada. FAU - Hebblethwaite, Shannon AU - Hebblethwaite S AD - Department of Applied Human Sciences, Concordia University, Montreal, Quebec, Canada. FAU - Bartlett, Susan J AU - Bartlett SJ AD - Department of Medicine, McGill University, Montreal, Quebec, Canada; Research Institute, McGill University Health Centre, Montreal, Quebec, Canada. FAU - Dyas, Laura AU - Dyas L AD - Scleroderma Foundation Michigan Chapter, Southfield, MI, USA. FAU - Patten, Scott AU - Patten S AD - Department of Community Health Sciences, University of Calgary, Calgary, Alberta, Canada; Hotchkiss Brain Institute and O'Brien Institute for Public Health, University of Calgary, Calgary, Alberta, Canada. FAU - Varga, John AU - Varga J AD - Northwestern Scleroderma Program, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. CN - Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 Patient Advisory Team CN - SPIN Investigators LA - eng GR - KL2 TR003168/TR/NCATS NIH HHS/United States PT - Clinical Trial Protocol PT - Journal Article DEP - 20200514 PL - England TA - J Psychosom Res JT - Journal of psychosomatic research JID - 0376333 SB - IM MH - Humans MH - *Anxiety/prevention & control MH - *Coronavirus Infections/epidemiology/prevention & control/psychology MH - COVID-19 MH - *Health Promotion/methods MH - Pandemics/prevention & control MH - Patient-Centered Care MH - *Pneumonia, Viral/epidemiology/prevention & control/psychology MH - Program Evaluation MH - Research Design MH - Risk Assessment MH - *Scleroderma, Systemic/therapy MH - Social Isolation/psychology MH - Videoconferencing MH - Randomized Controlled Trials as Topic MH - Pragmatic Clinical Trials as Topic PMC - PMC7224675 OTO - NOTNLM OT - Anxiety OT - COVID-19 OT - Coronavirus OT - Mental health OT - RCT OT - Scleroderma OT - Systemic sclerosis OT - Trial COIS- Declaration of Competing Interest All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf and declare that they have no competing interests. FIR - Fortuné, Catherine IR - Fortuné C IRAD- Ottawa Scleroderma Support Group, Ottawa, Ontario, Canada. FIR - Gietzen, Amy IR - Gietzen A IRAD- Scleroderma Foundation, Danvers, Massachusetts, USA. FIR - Guillot, Geneviève IR - Guillot G IRAD- Sclérodermie Québec, Longueuil, Quebec, Canada. FIR - Lewis, Nancy IR - Lewis N IRAD- Toronto, Ontario, Canada. FIR - Nielsen, Karen IR - Nielsen K IRAD- Scleroderma Society of Ontario, Hamilton, Ontario, Canada. FIR - Richard, Michelle IR - Richard M IRAD- Past-president of Scleroderma Canada, Halifax, Nova Scotia, Canada. FIR - Sauvé, Maureen IR - Sauvé M IRAD- Scleroderma Society of Ontario and Scleroderma Canada, Hamilton, Ontario, Canada. FIR - Welling, Joep IR - Welling J IRAD- NVLE Dutch patient organization for systemic autoimmune diseases, Utrecht, The Netherlands. FIR - Baron, Murray IR - Baron M IRAD- McGill University, Montreal, Quebec, Canada. FIR - Furst, Daniel E IR - Furst DE IRAD- Division of Rheumatology, Geffen School of Medicine, University of California, Los Angeles, CA, USA. FIR - Gottesman, Karen IR - Gottesman K IRAD- Scleroderma Foundation, Los Angeles, CA, USA. FIR - Malcarne, Vanessa IR - Malcarne V IRAD- San Diego State University, San Diego, CA, USA. FIR - Mayes, Maureen D IR - Mayes MD IRAD- University of Texas McGovern School of Medicine, Houston, TX, USA. FIR - Mouthon, Luc IR - Mouthon L IRAD- Université Paris Descartes, Paris, France. FIR - Nielson, Warren R IR - Nielson WR IRAD- St. Joseph's Health Care, London, Ontario, Canada. FIR - Riggs, Robert IR - Riggs R IRAD- Scleroderma Foundation, Danvers, MA, USA. FIR - Wigley, Fredrick IR - Wigley F IRAD- Johns Hopkins University School of Medicine, Baltimore, MD, USA. FIR - Assassi, Shervin IR - Assassi S IRAD- University of Texas McGovern School of Medicine, Houston, TX, USA. FIR - Boutron, Isabelle IR - Boutron I IRAD- Université Paris Descartes, Hôpitaux de Paris, Paris, France. FIR - Ells, Carolyn IR - Ells C IRAD- McGill University, Montreal, Quebec, Canada. FIR - van den Ende, Cornelia IR - van den Ende C IRAD- Sint Maartenskliniek, Nijmegen, The Netherlands. FIR - Fligelstone, Kim IR - Fligelstone K IRAD- Scleroderma & Raynaud's UK, London, UK. FIR - Frech, Tracy IR - Frech T IRAD- University of Utah, Salt Lake City, UT, USA. FIR - Godard, Dominique IR - Godard D IRAD- Association des Sclérodermiques de France, Sorel-Moussel, France. FIR - Harel, Daphna IR - Harel D IRAD- New York University, New York, NY, USA. FIR - Hinchcliff, Monique IR - Hinchcliff M IRAD- Yale School of Medicine, New Haven, CT, USA. FIR - Hudson, Marie IR - Hudson M IRAD- McGill University, Montreal, Quebec, Canada. FIR - Johnson, Sindhu R IR - Johnson SR IRAD- Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada. FIR - Larche, Maggie IR - Larche M IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Leite, Catarina IR - Leite C IRAD- University of Minho, Braga, Portugal. FIR - Nguyen, Christelle IR - Nguyen C IRAD- Université Paris Descartes, Hôpitaux de Paris, Paris, France. FIR - Pope, Janet IR - Pope J IRAD- University of Western Ontario, London, Ontario, Canada. FIR - Portales, Alexandra IR - Portales A IRAD- Asociación Española de Esclerodermia, Madrid, Spain. FIR - Rannou, François IR - Rannou F IRAD- Université Paris Descartes, Hôpitaux de Paris, Paris, France. FIR - Reyna, Tatiana Sofia Rodriguez IR - Reyna TSR IRAD- Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. FIR - Schouffoer, Anne A IR - Schouffoer AA IRAD- Leiden University Medical Center, Leiden, The Netherlands. FIR - Suarez-Almazor, Maria E IR - Suarez-Almazor ME IRAD- University of Texas MD Anderson Cancer Center, Houston, TX, USA. FIR - Agard, Christian IR - Agard C IRAD- Centre Hospitalier Universitaire, Hôtel-Dieu de Nantes, Nantes, France. FIR - Albert, Alexandra IR - Albert A IRAD- Université Laval, Quebec, Quebec, Canada. FIR - André, Marc IR - André M IRAD- Centre Hospitalier Universitaire Gabriel-Montpied, Clermont-Ferrand, France. FIR - Arsenault, Guylaine IR - Arsenault G IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Benzidia, Ilham IR - Benzidia I IRAD- Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Bernstein, Elana J IR - Bernstein EJ IRAD- Columbia University, New York, NY, USA. FIR - Berthier, Sabine IR - Berthier S IRAD- Centre Hospitalier Universitaire Dijon Bourgogne, Dijon, France. FIR - Bissonnette, Lyne IR - Bissonnette L IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Boire, Gilles IR - Boire G IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Bruns, Alessandra IR - Bruns A IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Carreira, Patricia IR - Carreira P IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Casadevall, Marion IR - Casadevall M IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Chaigne, Benjamin IR - Chaigne B IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Chung, Lorinda IR - Chung L IRAD- Stanford University, Stanford, CA, USA. FIR - Cohen, Pascal IR - Cohen P IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Correia, Chase IR - Correia C IRAD- Northwestern University, Chicago, IL, USA. FIR - Dagenais, Pierre IR - Dagenais P IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Denton, Christopher IR - Denton C IRAD- Royal Free London Hospital, London, UK. FIR - Domsic, Robyn IR - Domsic R IRAD- University of Pittsburgh, Pittsburgh, PA, USA. FIR - Dubois, Sandrine IR - Dubois S IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Dunne, James V IR - Dunne JV IRAD- St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. FIR - Dunogue, Bertrand IR - Dunogue B IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Fare, Regina IR - Fare R IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Farge-Bancel, Dominique IR - Farge-Bancel D IRAD- Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Fortin, Paul R IR - Fortin PR IRAD- CHU de Québec, Université Laval, Quebec, Quebec, Canada. FIR - Gill, Anna IR - Gill A IRAD- Royal Free London Hospital, London, UK. FIR - Gordon, Jessica IR - Gordon J IRAD- Hospital for Special Surgery, New York City, NY, USA. FIR - Granel-Rey, Brigitte IR - Granel-Rey B IRAD- Aix Marseille Université, Hôpitaux de Marseille, Hôpital Nord, Marseille, France. FIR - Gyger, Genevieve IR - Gyger G IRAD- Jewish General Hospital, McGill University, Montreal, Quebec, Canada. FIR - Hachulla, Eric IR - Hachulla E IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Hatron, Pierre-Yves IR - Hatron PY IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Herrick, Ariane L IR - Herrick AL IRAD- University of Manchester, Salford Royal NHS Foundation Trust, Manchester, UK. FIR - Hij, Adrian IR - Hij A IRAD- Hôpitaux de Paris, Hôpital St-Louis, Paris, France. FIR - Hoa, Sabrina IR - Hoa S IRAD- Centre hospitalier de l'université de Montréal - CHUM, Montreal, Quebec, Canada. FIR - Ikic, Alena IR - Ikic A IRAD- Université Laval, Quebec, Quebec, Canada. FIR - Jones, Niall IR - Jones N IRAD- University of Alberta, Edmonton, Alberta, Canada. FIR - de B Fernandes, Artur Jose IR - de B Fernandes AJ IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Kafaja, Suzanne IR - Kafaja S IRAD- University of California, Los Angeles, CA, USA. FIR - Khalidi, Nader IR - Khalidi N IRAD- McMaster University, Hamilton, Ontario, Canada. FIR - Lambert, Marc IR - Lambert M IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Launay, David IR - Launay D IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Liang, Patrick IR - Liang P IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Maillard, Hélène IR - Maillard H IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Maltez, Nancy IR - Maltez N IRAD- University of Ottawa, Ottawa, Ontario, Canada. FIR - Manning, Joanne IR - Manning J IRAD- Salford Royal NHS Foundation Trust, Salford, UK. FIR - Marie, Isabelle IR - Marie I IRAD- CHU Rouen, Hôpital de Bois-Guillaume, Rouen, France. FIR - Martin, Maria IR - Martin M IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Martin, Thierry IR - Martin T IRAD- Les Hôpitaux Universitaires de Strasbourg, Nouvel Hôpital Civil, Strasbourg, France. FIR - Masetto, Ariel IR - Masetto A IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Maurier, François IR - Maurier F IRAD- Hôpitaux Privés de Metz, Hôpital Belle-Isle, Metz, France. FIR - Mekinian, Arsene IR - Mekinian A IRAD- Hôpitaux de Paris, Hôpital St-Antoine, Paris, France. FIR - Melchor, Sheila IR - Melchor S IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Nikpour, Mandana IR - Nikpour M IRAD- St Vincent's Hospital, University of Melbourne, Melbourne, Victoria, Australia. FIR - Olagne, Louis IR - Olagne L IRAD- Centre Hospitalier Universitaire Gabriel-Montpied, Clermont-Ferrand, France. FIR - Poindron, Vincent IR - Poindron V IRAD- Les Hôpitaux Universitaires de Strasbourg, Nouvel Hôpital Civil, Strasbourg, France. FIR - Proudman, Susanna IR - Proudman S IRAD- Royal Adelaide Hospital, University of Adelaide, Adelaide, South Australia, Australia. FIR - Régent, Alexis IR - Régent A IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Rivière, Sébastien IR - Rivière S IRAD- Hôpitaux de Paris, Hôpital St-Antoine, Paris, France. FIR - Robinson, David IR - Robinson D IRAD- University of Manitoba, Winnipeg, Manitoba, Canada. FIR - Rodriguez, Esther IR - Rodriguez E IRAD- Servicio de Reumatologia del Hospital 12 de Octubre, Madrid, Spain. FIR - Roux, Sophie IR - Roux S IRAD- Université de Sherbrooke, Sherbrooke, Quebec, Canada. FIR - Smets, Perrine IR - Smets P IRAD- Centre Hospitalier Universitaire Gabriel-Montpied, Clermont-Ferrand, France. FIR - Smith, Doug IR - Smith D IRAD- University of Ottawa, Ottawa, Ontario, Canada. FIR - Sobanski, Vincent IR - Sobanski V IRAD- Centre Hospitalier Régional Universitaire de Lille, Hôpital Claude Huriez, Lille, France. FIR - Spiera, Robert IR - Spiera R IRAD- Hospital for Special Surgery, New York City, NY, USA. FIR - Steen, Virginia IR - Steen V IRAD- Georgetown University, Washington, DC, USA. FIR - Stevens, Wendy IR - Stevens W IRAD- St Vincent's Hospital, University of Melbourne, Melbourne, Victoria, Australia. FIR - Sutton, Evelyn IR - Sutton E IRAD- Dalhousie University, Halifax, Nova Scotia, Canada. FIR - Terrier, Benjamin IR - Terrier B IRAD- Hôpitaux de Paris, Hôpital Cochin, Paris, France. FIR - Thorne, Carter IR - Thorne C IRAD- Southlake Regional Health Centre, Newmarket, Ontario, Canada. FIR - Varga, John IR - Varga J IRAD- Northwestern University, Chicago, IL, USA. FIR - Wilcox, Pearce IR - Wilcox P IRAD- St. Paul's Hospital and University of British Columbia, Vancouver, British Columbia, Canada. FIR - Ayala, Mara Cañedo IR - Ayala MC IRAD- Jewish General Hospital, Montreal, Quebec, Canada. FIR - Ostbo, Nora IR - Ostbo N IRAD- Jewish General Hospital, Montreal, Quebec, Canada. EDAT- 2020/06/11 06:00 MHDA- 2020/07/18 06:00 PMCR- 2020/05/14 CRDT- 2020/06/11 06:00 PHST- 2020/04/04 00:00 [received] PHST- 2020/05/03 00:00 [revised] PHST- 2020/05/04 00:00 [accepted] PHST- 2020/06/11 06:00 [pubmed] PHST- 2020/07/18 06:00 [medline] PHST- 2020/06/11 06:00 [entrez] PHST- 2020/05/14 00:00 [pmc-release] AID - S0022-3999(20)30400-1 [pii] AID - 110132 [pii] AID - 10.1016/j.jpsychores.2020.110132 [doi] PST - ppublish SO - J Psychosom Res. 2020 Aug;135:110132. doi: 10.1016/j.jpsychores.2020.110132. Epub 2020 May 14. PMID- 40214648 OWN - NLM STAT- MEDLINE DCOM- 20250513 LR - 20260428 IS - 1469-7793 (Electronic) IS - 0022-3751 (Print) IS - 0022-3751 (Linking) VI - 603 IP - 9 DP - 2025 May TI - Heart dysfunction in a rat model with autosomal recessive polycystic kidney disease. PG - 2551-2567 LID - 10.1113/JP286364 [doi] AB - Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic pathology and is one of the most significant childhood nephropathies leading to chronic kidney disease (CKD). While kidney damage has been well studied in this pathology, only a few studies have investigated specific cardiac damage during ARPKD. This study aimed to conduct a large analysis of heart dysfunction during the progression of CKD. ARPKD rats with the Pkhd1 gene mutation (IVS35-2A>T) were monitored for CKD progression and heart dysfunction via echocardiography. Heart fibrosis was assessed using Sirius red staining, and cardiokines mRNA expressions in heart tissue were analysed. ARPKD rats exhibited increased blood pressure, cardiac hypertrophy and thickening of the left ventricular posterior wall, correlated with elevated plasma creatinine levels. Diastolic dysfunction was evident, shown by altered E/A and E/e' ratios, which worsened with CKD severity. Heart fibrosis correlated with renal dysfunction, and fibrosis signalling pathways were activated, marked by increased galectin-3, collagen-1, fibroblast growth factor 23, suppressor of tumorigenicity 2 (ST2) and soluble ST2 expression. Growth differentiation factor 15 levels rose with CKD progression, while Irisin levels decreased, negatively correlating with the E/e' ratio. This study highlights diastolic dysfunction, heart fibrosis, and hypertrophy in ARPKD rats with severe CKD. These cardiac changes are linked to dysregulation in cardiokine signalling, providing new insights into uraemia-induced heart failure in ARPKD KEY POINTS: Autosomal recessive polycystic kidney disease (ARPKD) is a rare genetic disorder with a reduced life expectancy and a broad clinical spectrum including systemic hypertension, renal failure, portal hypertension, and renal and hepatic fibrosis. PCK rats have a spontaneous mutation in the Pkhd1 gene, the same gene affected in human ARPKD. PCK rats develop renal and hepatic cysts and other manifestations of human ARPKD. Changes in heart tissue (fibrosis, hypertrophy) and diastolic dysfunction (decreasing E/A and increasing E/e' ratios) worsen with renal dysfunction in PCK rats. Heart dysfunctions are associated with a dysregulation of cardiokine signalling such as Irisin, in PCK rats. Irisin could be a new marker of diastolic function in ARPKD and chronic kidney disease patients. CI - © 2025 The Author(s). The Journal of Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society. FAU - Gayrard, Nathalie AU - Gayrard N AUID- ORCID: 0000-0001-8283-8429 AD - RD Néphrologie, Montpellier, France. FAU - Plawecki, Maëlle AU - Plawecki M AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. AD - Department of Biochemistry and Hormonology, University Hospital (CHU Lapeyronie) of Montpellier, Montpellier, France. FAU - Lauret, Céline AU - Lauret C AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. FAU - Fila, Marc AU - Fila M AUID- ORCID: 0000-0001-8857-7100 AD - Department of Pediatric Nephrology, CHU Arnaud de Villeneuve, SORARE Montpellier University, Montpellier, France. FAU - Sicard, Pierre AU - Sicard P AUID- ORCID: 0000-0001-5837-3916 AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. FAU - Duranton, Flore AU - Duranton F AD - RD Néphrologie, Montpellier, France. FAU - Boukhaled, Juliana H AU - Boukhaled JH AD - RD Néphrologie, Montpellier, France. FAU - Cortijo-Tejero, Irene AU - Cortijo-Tejero I AD - RD Néphrologie, Montpellier, France. FAU - Lotierzo, Manuela AU - Lotierzo M AUID- ORCID: 0000-0002-7555-5129 AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. AD - Department of Biochemistry and Hormonology, University Hospital (CHU Lapeyronie) of Montpellier, Montpellier, France. FAU - Jover, Bernard AU - Jover B AUID- ORCID: 0000-0001-5826-5755 AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. FAU - Cristol, Jean-Paul AU - Cristol JP AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. AD - Department of Biochemistry and Hormonology, University Hospital (CHU Lapeyronie) of Montpellier, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AUID- ORCID: 0000-0003-3055-0524 AD - PHYMEDEXP, Montpellier University, INSERM, CNRS, Montpellier, France. LA - eng GR - IRCT dialysis 2023/SFNDT/ PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20250411 PL - England TA - J Physiol JT - The Journal of physiology JID - 0266262 SB - IM MH - Animals MH - *Polycystic Kidney, Autosomal Recessive/physiopathology/complications/genetics MH - Rats MH - Male MH - Fibrosis MH - Disease Models, Animal MH - Myocardium/metabolism/pathology MH - Rats, Sprague-Dawley MH - Renal Insufficiency, Chronic/physiopathology PMC - PMC12072246 OTO - NOTNLM OT - ARPKD OT - chronic kidney disease OT - diastolic function OT - heart fibrosis and cardiokines COIS- The authors declare that there is no conflict of interest regarding the publication of this paper. EDAT- 2025/04/11 12:28 MHDA- 2025/05/13 13:49 PMCR- 2025/04/11 CRDT- 2025/04/11 10:42 PHST- 2024/01/31 00:00 [received] PHST- 2025/03/18 00:00 [accepted] PHST- 2025/05/13 13:49 [medline] PHST- 2025/04/11 12:28 [pubmed] PHST- 2025/04/11 10:42 [entrez] PHST- 2025/04/11 00:00 [pmc-release] AID - TJP16682 [pii] AID - 10.1113/JP286364 [doi] PST - ppublish SO - J Physiol. 2025 May;603(9):2551-2567. doi: 10.1113/JP286364. Epub 2025 Apr 11. PMID- 16484933 OWN - NLM STAT- MEDLINE DCOM- 20060531 LR - 20190922 IS - 0221-0363 (Print) IS - 0221-0363 (Linking) VI - 87 IP - 2 Pt 1 DP - 2006 Feb TI - [Non traumatic high flow priapism: arterial embolization treatment]. PG - 115-9 AB - PURPOSE: To report cases of non traumatic high flow priapism treated by arterial embolization. MATERIAL AND METHODS: Six men presented with non traumatic high flow priapism, the diagnosis was based on colour Doppler ultrasound, cavernous blood gas analysis with arterial blood saturation levels and failed medical or surgical therapy. Four patients had sickle cell disease. The embolization was performed with Gelfoam and was unilateral in one case, bilateral in the other cases. RESULTS: Detumescence occurred in a few hours in all cases. One patient had recurrent priapism two years after and was treated by embolization. Transient erectile dysfunction was observed in five cases, permanent in one case. CONCLUSION: Arterial embolization is the treatment of choice in high flow priapism with low rate of permanent erectile dysfunction. FAU - Poey, C AU - Poey C AD - Service de Radiologie, Clinique St Paul, 97200 Fort de France. claudes.poey@wanadoo.fr FAU - Guy, F AU - Guy F FAU - Rabia, N AU - Rabia N FAU - Vergnolle, M AU - Vergnolle M FAU - Khadji, A AU - Khadji A FAU - Raynaud, M AU - Raynaud M FAU - Dutheil, A AU - Dutheil A LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Priapisme à haut débit non traumatique: traitement par embolisation artérielle. PL - France TA - J Radiol JT - Journal de radiologie JID - 7906266 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Blood Flow Velocity MH - *Embolization, Therapeutic MH - Humans MH - Male MH - Priapism/physiopathology/*therapy EDAT- 2006/02/18 09:00 MHDA- 2006/06/01 09:00 CRDT- 2006/02/18 09:00 PHST- 2006/02/18 09:00 [pubmed] PHST- 2006/06/01 09:00 [medline] PHST- 2006/02/18 09:00 [entrez] AID - MDOI-JR-02-2006-87-2-C1-0221-0363-101019-200508748 [pii] AID - 10.1016/s0221-0363(06)73981-6 [doi] PST - ppublish SO - J Radiol. 2006 Feb;87(2 Pt 1):115-9. doi: 10.1016/s0221-0363(06)73981-6. PMID- 41399061 OWN - NLM STAT- MEDLINE DCOM- 20251216 LR - 20251221 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 57 IP - 6 DP - 2025 Dec 18 TI - [Rheumatic disease spectrum and immunological profile of anti-PM/Scl antibodies in idiopathic inflammatory myopathies]. PG - 1018-1023 AB - OBJECTIVE: To systematically investigate the rheumatic disease spectrum associated with anti-PM/Scl antibodies and to clarify their clinical significance in idiopathic inflammatory myopathies (IIM). METHODS: Patients who were tested positive for anti-PM/Scl antibodies by immunoblotting at Peking University People' s Hospital from January 2016 to December 2024 were enrolled. Clinical and immunolo gical data were systematically collected and compared across the subgroups defined by anti-PM/Scl75, anti-PM/Scl100, or dual antibody positivity. RESULTS: A total of 422 anti-PM/Scl-positive patients were enrolled. Among them, 83.2% (351/422) were diagnosed with connective tissue disease (CTD), 7.8% (33/422) were not diagnosed with CTD, and 9.0% (38/422) had an undetermined clinical diagnosis. Among 422 patients, most commonly represented by IIM (19.7%), systemic sclerosis (SSc, 14.2%), overlap syndrome (11.8%), undifferentiated CTD (UCTD, 10.4%), rheumatoid arthritis (6.9%), Sjögren syndrome (6.4%), and systemic lupus erythematosus (6.2%), the remaining diseases accounting for 24.4%. Within the IIM subgroup, dermatomyositis predominated (74.7%), followed next by anti-synthetase syndrome (21.7%) and immune-mediated necrotizing myopathy (3.6%). Anti-PM/Scl75 antibodies were detected in 52.1% (220/422) of the total patients, anti-PM/Scl100 in 43.6% (184/422), and both in 4.3% (18/422). In the subsequent detailed analysis of the anti-PM/ Scl-positive subgroup, double-positive patients showed a significantly higher prevalence of SSc (38.9% vs. 14.1% vs. 12.0%, P=0.015) and interstitial lung disease (ILD, 70.6% vs. 28.8% vs. 35.4%, P=0.002) than those individuals with single antibody positivity alone. Raynaud phenomenon was observed more frequently in both the double-positive and anti-PM/Scl75-positive groups than in the anti-PM/Scl100-positive group (29.4% vs. 21.3% vs. 10.9%, P=0.007). The measured proportion of peri-pheral CD8(+) T cells was also higher in double-positive patients (35.9%±14.1% vs. 30.4%±11.2% vs. 26.5%±9.7%, P= 0.008), whereas absolute regulatory T-cell levels were lower in the anti-PM/Scl75-positive group compared directly with the anti-PM/Scl100-positive group [7.6% (5.4%, 10.9%) vs. 9.0% (7.9%, 12.0%) vs. 8.8% (5.2%, 9.7%), P=0.017]. Additionally, co-positivity for anti-PM/Scl and other myositis- specific or myositis-associated antibodies was strongly associated with an increased frequency of ILD (P < 0.05). CONCLUSION: Anti-PM/Scl antibodies define a broad disease spectrum encompassing IIM, SSc, overlap syndromes, and UCTD. Dual positivity for anti-PM/Scl75 and anti-PM/Scl100 identifies patients prone to systemic sclerosis and pulmonary involvement, suggesting additive pathogenic effects of the two antibody specificities. FAU - Lian, Yirui AU - Lian Y AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 1000444, China. FAU - Liu, Jingxuan AU - Liu J AD - Department of Rheumatology and Immunology, Peking University People' s Hospital Qingdao Hospital, Qingdao 266000, Shandong, China. FAU - Zhao, Liang AU - Zhao L AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 1000444, China. FAU - Zhao, Jing AU - Zhao J AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 1000444, China. FAU - Zang, Sitian AU - Zang S AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 1000444, China. FAU - Li, Yuhui AU - Li Y AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 1000444, China. AD - Department of Rheumatology and Immunology, Peking University People' s Hospital Qingdao Hospital, Qingdao 266000, Shandong, China. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Myositis/immunology MH - *Autoantibodies/immunology/blood MH - Female MH - Male MH - Middle Aged MH - Adult MH - *Rheumatic Diseases/immunology MH - Aged MH - Connective Tissue Diseases/immunology MH - Scleroderma, Systemic/immunology MH - Young Adult PMC - PMC12711398 OTO - NOTNLM OT - Anti-PM/Scl antibody OT - CD8+ T cells OT - Connective tissue disease OT - Idiopathic inflammatory myopathy COIS- 利益冲突  所有作者均声明不存在利益冲突。 EDAT- 2025/12/16 06:28 MHDA- 2025/12/16 06:29 PMCR- 2025/12/18 CRDT- 2025/12/16 02:17 PHST- 2025/12/16 06:29 [medline] PHST- 2025/12/16 06:28 [pubmed] PHST- 2025/12/16 02:17 [entrez] PHST- 2025/12/18 00:00 [pmc-release] AID - bjdxxbyxb-57-6-1018 [pii] AID - 10.19723/j.issn.1671-167X.2025.06.002 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2025 Dec 18;57(6):1018-1023. doi: 10.19723/j.issn.1671-167X.2025.06.002. PMID- 36183479 OWN - NLM STAT- MEDLINE DCOM- 20221122 LR - 20221129 IS - 1532-866X (Electronic) IS - 0049-0172 (Linking) VI - 57 DP - 2022 Dec TI - The clinical features of juvenile dermatomyositis: A single-centre inception cohort. PG - 152104 LID - S0049-0172(22)00155-X [pii] LID - 10.1016/j.semarthrit.2022.152104 [doi] AB - INTRODUCTION: Juvenile Dermatomyositis (JDM), a severe and rare autoimmune disease, is the most common idiopathic inflammatory myopathy in children. We describe the clinical features of a large single-centre cohort. METHODS: We studied an inception cohort (0-18 years old) referred for diagnosis to the JDM clinic at The Hospital for Sick Children (SickKids), between January 1989 and September 2017. Probable or definite diagnosis of JDM was done according to the 2017 ACR/EULAR Criteria. We excluded children who had treatment started at another hospital. The data were collected retrospectively from clinical charts and the SickKids JDM database. RESULTS: 172/230 (74.8%) patients were included. They were most often female (female:male = 1.8:1); the age at diagnosis was 8.5±4.3 years. There was a positive family history for autoimmune disease in 52%, mainly rheumatoid arthritis. No patient died. The most common signs at inception were muscle weakness (85.5%), nailfold capillary abnormalities (83.4%), Gottron papules (78.5%), heliotrope rash (66.3%), abnormal gait (55.8%), and malar/facial rash (54.7%). The prevalence of Gottron papules, heliotrope rash, facial/malar rash, nailfold capillary abnormalities, Raynaud phenomenon, dysphonia/dysphagia (a frequent cause of hospitalization), mouth ulcers, calcinosis, eye problems, joint involvement, acanthosis nigricans and lipodystrophy increased during follow-up. Muscle enzymes, namely CK, ALT, AST, were often normal or only slightly raised despite active muscle disease; conversely LD was often high. Anti-Nuclear Autoantibodies were positive in 49.7% of patients at diagnosis. The course of the disease was: 29.1% monocyclic, 5.3% polycyclic, 33.1% chronic. The course of 56 patients (32.5%) was not classifiable due to length of follow-up. Corticosteroids were used as treatment in almost all our patients and 30% required intravenous therapy due to the severity of the presentation; methotrexate was added in 64%, more often in recent years. Unresponsive patients were treated mostly with intravenous immunoglobulins (IVIG). CONCLUSIONS: The information obtained from this relatively large number of patients adds to the growing knowledge base of this rare disease. TRIAL REGISTRATION: SickKids Research Ethics Board approved the study. CI - Copyright © 2022. Published by Elsevier Inc. FAU - Cancarini, Paola AU - Cancarini P AD - Operative Unit of Pediatrics, ASST del Garda, Desenzano del Garda (BS), Italy. FAU - Nozawa, Tomo AU - Nozawa T AD - Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Yokohama, Kanagawa, Japan; Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada. FAU - Whitney, Kristi AU - Whitney K AD - Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada; Department of Rehabilitation, The Hospital for Sick Children, Toronto, Canada. FAU - Bell-Peter, Audrey AU - Bell-Peter A AD - Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada. FAU - Marcuz, Jo-Anne AU - Marcuz JA AD - Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada; Department of Rehabilitation, The Hospital for Sick Children, Toronto, Canada. FAU - Taddio, Andrea AU - Taddio A AD - Institute for Maternal and Child Health-IRCCS "Burlo Garofolo" and University of Trieste, Trieste, Italy. FAU - Guo, Jessica AU - Guo J AD - Child Health Evaluative Sciences, Hospital for Sick Children Research Institute, Toronto, Canada. FAU - Dover, Saunya AU - Dover S AD - Child Health Evaluative Sciences, Hospital for Sick Children Research Institute, Toronto, Canada. FAU - Feldman, Brian M AU - Feldman BM AD - Child Health Evaluative Sciences, Hospital for Sick Children Research Institute, Toronto, Canada; Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada; Department of Pediatrics, Faculty of Medicine, University of Toronto, Toronto, ON, Canada; Institute of Health Policy, Management & Evaluation, The Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada. Electronic address: brian.feldman@sickkids.ca. LA - eng PT - Journal Article DEP - 20220925 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 SB - IM MH - Humans MH - Male MH - Female MH - Child, Preschool MH - Child MH - Infant, Newborn MH - Infant MH - Adolescent MH - *Dermatomyositis/diagnosis/drug therapy MH - Retrospective Studies MH - *Myositis MH - *Autoimmune Diseases MH - *Exanthema OTO - NOTNLM OT - Autoimmune disease OT - CHAQ child health assessment questionnaire OT - CMAS childhood myositis assessment scale OT - Gottron's sign/papules OT - Heliotrope rash OT - Juvenile dermatomyositis OT - MMT manual muscle testing OT - Myositis COIS- Declaration of Competing Interest T. Nozawa: Scholarships from Japan Society of Allergology and Gushinkai, Fellowship from Mochida Memorial Foundation; Advisory Board member for Bristol Myers Squibb Company. The remaining authors have no competing interests to declare. EDAT- 2022/10/03 06:00 MHDA- 2022/11/23 06:00 CRDT- 2022/10/02 18:07 PHST- 2022/01/24 00:00 [received] PHST- 2022/08/30 00:00 [revised] PHST- 2022/09/21 00:00 [accepted] PHST- 2022/10/03 06:00 [pubmed] PHST- 2022/11/23 06:00 [medline] PHST- 2022/10/02 18:07 [entrez] AID - S0049-0172(22)00155-X [pii] AID - 10.1016/j.semarthrit.2022.152104 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2022 Dec;57:152104. doi: 10.1016/j.semarthrit.2022.152104. Epub 2022 Sep 25. PMID- 36436115 OWN - NLM STAT- MEDLINE DCOM- 20230829 LR - 20230829 IS - 1591-9528 (Electronic) IS - 1591-8890 (Linking) VI - 23 IP - 5 DP - 2023 Sep TI - Role of kinurenic acid in the systemic sclerosis renal involvement. PG - 1713-1719 LID - 10.1007/s10238-022-00962-6 [doi] AB - Systemic sclerosis (SSc) subclinical renal vasculopathy is characterized by progressive increase of intrarenal stiffness and reduction of parenchymal thickness due to post ischemic fibrosis secondary to the renal Raynaud phenomenon. Aims of this study were to evaluate kinurenic acid (KYNA) serum level in SSc patients and healthy controls (HC) and to assess the role of KYNA in SSc subclinical nephropathy. Serum level of KYNA was evaluated in 52 SSc patients and 20 HC, matched for sex and age. Renal function was calculated using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation to estimate glomerular filtration rate (eGFR) and renal doppler ultrasound was performed to evaluate kidneys' morphology and indices of intrarenal stiffness. The parameters registered were renal longitudinal length, atrophy index (AI), renal sinus, parenchymal thickness, renal resistive index (RRI), pulsatile index (PI) and systolic/diastolic ratio (S/D). SSc patients had lower median value of KYNA than HC [54.43 ng/ml (IQR 44.44-63.64) vs 61.94 ng/ml (IQR 55.23-88.75), p < 0.001]. SSc patients with AI ≥ 0.70 had lower KYNA than SSc patients with AI < 0.70 [47.85 ng/ml (IQR 41.16-59.91) vs 55.5 ng/ml (IQR 49.99-67.33), p < 0.05] and a slightly significant negative linear correlation was found between KYNA and AI (r =  - 0.249, p < 0.05). SSc patient with RRI ≥ 0.70 had higher KYNA than SSc patients with RRI < 0.70 [58.25 ng/ml (IQR 50.49-69.68) vs 50.07 ng/ml (IQR 42.70-56.31), p < 0.05] and a significant positive correlation was found between KYNA and RRI (r = 0.318, p < 0.05). KYNA may be used as a marker to evaluate the renal involvement in SSc patients. CI - © 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG. FAU - Pellicano, Chiara AU - Pellicano C AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. FAU - Vaiarello, Valentina AU - Vaiarello V AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. FAU - Colalillo, Amalia AU - Colalillo A AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. FAU - Gigante, Antonietta AU - Gigante A AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. FAU - Iannazzo, Francesco AU - Iannazzo F AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. FAU - Rosato, Edoardo AU - Rosato E AD - Department of Translational and Precision Medicine, Sapienza University, Viale Dell'Università 37, 00185, Rome, Italy. edoardo.rosato@uniroma1.it. LA - eng PT - Journal Article DEP - 20221127 PL - Italy TA - Clin Exp Med JT - Clinical and experimental medicine JID - 100973405 SB - IM MH - Humans MH - Kidney/diagnostic imaging MH - *Scleroderma, Systemic/complications/diagnostic imaging MH - *Renal Insufficiency, Chronic/complications MH - Ultrasonography MH - Ultrasonography, Doppler OTO - NOTNLM OT - Atrophic index OT - Kynurenic acid OT - Renal resistive index OT - Systemic sclerosis EDAT- 2022/11/28 06:00 MHDA- 2023/08/29 12:44 CRDT- 2022/11/27 14:05 PHST- 2022/11/10 00:00 [received] PHST- 2022/11/22 00:00 [accepted] PHST- 2023/08/29 12:44 [medline] PHST- 2022/11/28 06:00 [pubmed] PHST- 2022/11/27 14:05 [entrez] AID - 10.1007/s10238-022-00962-6 [pii] AID - 10.1007/s10238-022-00962-6 [doi] PST - ppublish SO - Clin Exp Med. 2023 Sep;23(5):1713-1719. doi: 10.1007/s10238-022-00962-6. Epub 2022 Nov 27. PMID- 21925064 OWN - NLM STAT- MEDLINE DCOM- 20120126 LR - 20141120 IS - 1879-0828 (Electronic) IS - 0953-6205 (Linking) VI - 22 IP - 5 DP - 2011 Oct TI - Long-term effects of intermittent Iloprost infusion on pulmonary arterial pressure in connective tissue disease. PG - 518-21 LID - 10.1016/j.ejim.2011.02.005 [doi] AB - BACKGROUND: Intravenous periodic Iloprost is proven effective in the treatment of Raynaud phenomenon (RP) related to connective tissue disorder (CTD). It's well known that synthetic prostaglandins are effective drugs for the treatment of pulmonary arterial hypertension (PAH), and that PAH is frequently associated with CTD. OBJECTIVE: The aim of the study is to evaluate in the chronic effect of cyclic intravenous Iloprost on pulmonary arterial pressure. METHODS: We studied 17 consecutive patients with CTD (14 systemic sclerosis, 3 mixed CTD) and RP, at the entry and after at least 6months of treatment of RP with cyclic Iloprost. On both occasions, in all patients we performed transthoracic Doppler echocardiography and we determined NT-proBNP plasma levels, NYHA functional class, 6 Minute-Walk Distance (6MWD). RESULTS: At follow-up (8.2±1.9months; range 6-12) mean values of pulmonary arterial systolic pressure (PASP) significantly decreased (from 32.2±9.2 to 29.2±7.6mmHg, p<0.04) and mean values of 6MWD significantly increased (from 407.5±101.5 to 448.3±89.9m, p<0.01). Moreover, we observed a significant direct correlation between PASP and NT-proBNP values and a significant inverse correlation both between NT-proBNP and 6MWD values and between PASP and 6MWD values. CONCLUSION: Our results suggest that cyclic intravenous Iloprost may protect against the development or worsening of PAH in patients with CTD and RP. CI - Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. FAU - Caravita, Sergio AU - Caravita S AD - U.O.C. Medicina Interna, Ospedale Bassini, Azienda Ospedaliera Istituti Clinici di Perfezionamento, Milano, Italy. FAU - Wu, Sheng Chin AU - Wu SC FAU - Secchi, Maria Beatrice AU - Secchi MB FAU - Dadone, Viola AU - Dadone V FAU - Bencini, Chiara AU - Bencini C FAU - Pierini, Simona AU - Pierini S LA - eng PT - Comparative Study PT - Journal Article DEP - 20110315 PL - Netherlands TA - Eur J Intern Med JT - European journal of internal medicine JID - 9003220 RN - 0 (Cardiovascular Agents) RN - JED5K35YGL (Iloprost) SB - IM MH - Adult MH - Aged MH - Cardiovascular Agents/administration & dosage/therapeutic use MH - Connective Tissue Diseases/*complications/physiopathology MH - Dose-Response Relationship, Drug MH - Familial Primary Pulmonary Hypertension MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/etiology/physiopathology/*prevention & control MH - Iloprost/*administration & dosage/therapeutic use MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Pulmonary Wedge Pressure/*drug effects/physiology MH - Retrospective Studies MH - Time Factors MH - Treatment Outcome EDAT- 2011/09/20 06:00 MHDA- 2012/01/27 06:00 CRDT- 2011/09/20 06:00 PHST- 2010/11/15 00:00 [received] PHST- 2011/01/16 00:00 [revised] PHST- 2011/02/07 00:00 [accepted] PHST- 2011/09/20 06:00 [entrez] PHST- 2011/09/20 06:00 [pubmed] PHST- 2012/01/27 06:00 [medline] AID - S0953-6205(11)00036-7 [pii] AID - 10.1016/j.ejim.2011.02.005 [doi] PST - ppublish SO - Eur J Intern Med. 2011 Oct;22(5):518-21. doi: 10.1016/j.ejim.2011.02.005. Epub 2011 Mar 15. PMID- 19164337 OWN - NLM STAT- MEDLINE DCOM- 20090520 LR - 20151119 IS - 0004-5632 (Print) IS - 0004-5632 (Linking) VI - 46 IP - Pt 2 DP - 2009 Mar TI - Serum insulin-like growth factor binding protein-1: an improvement over other simple indices of insulin sensitivity in the assessment of subjects with normal glucose tolerance. PG - 109-13 LID - 10.1258/acb.2008.008160 [doi] AB - BACKGROUND: Insulin resistance is associated with an increased risk of cardiovascular disease and diabetes. It can be assessed using complex reference techniques, such as clamp or frequently sampled intravenous glucose tolerance test (FSIVGTT). Therefore, simple indices derived from fasting insulin and glucose concentrations have been proposed. The aim of this study is to assess fasting serum insulin-like growth factor binding protein-1 (IGFBP-1) as a simple index of insulin sensitivity compared with other simple indices and FSIVGTT. METHODS: Fasting serum IGFBP-1, fasting plasma insulin (FPI), homeostasis model assessment (HOMA-IR), quantitative insulin check index (QUICKI), fasting glucose to insulin ratio (FGIR), Raynaud and insulin glycaemic index (ISI-gly) were correlated with FSIVGTT (Si) in 22 subjects with normal glucose tolerance (NGT) and nine with impaired fasting glucose (IFG). RESULTS: In NGT individuals, IGFBP-1 correlated more strongly with Si than did any other index both before (r = 0.76) and after (r = 0.79) natural logarithm (ln) transformation. In subjects with IFG, IGFBP-1 was weakly correlated with Si before and after ln-transformation (r = 0.55, r = 0.56, respectively), but ISI-gly was the index most strongly correlated with Si (r = 0.77, r = 0.85, respectively). CONCLUSIONS: In subjects with NGT, fasting serum IGFBP-1 could be used as a simple reliable marker of insulin sensitivity. For more accurate estimation of insulin sensitivity in normal subjects and those with IFG, ln-transformation is preferred over raw data. FAU - Borai, Anwar AU - Borai A AD - Faculty of Health and Medical Sciences, University of Surrey, Guildford, UK. FAU - Livingstone, Callum AU - Livingstone C FAU - Zarif, Hawazen AU - Zarif H FAU - Ferns, Gordon AU - Ferns G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20090122 PL - England TA - Ann Clin Biochem JT - Annals of clinical biochemistry JID - 0324055 RN - 0 (Biomarkers) RN - 0 (IGFBP1 protein, human) RN - 0 (Insulin) RN - 0 (Insulin-Like Growth Factor Binding Protein 1) SB - IM MH - Adult MH - Biomarkers/blood MH - Fasting MH - Female MH - Glucose Tolerance Test MH - Humans MH - Insulin/blood MH - Insulin Resistance/*physiology MH - Insulin-Like Growth Factor Binding Protein 1/*blood MH - Male MH - Middle Aged EDAT- 2009/01/24 09:00 MHDA- 2009/05/21 09:00 CRDT- 2009/01/24 09:00 PHST- 2009/01/24 09:00 [entrez] PHST- 2009/01/24 09:00 [pubmed] PHST- 2009/05/21 09:00 [medline] AID - acb.2008.008160 [pii] AID - 10.1258/acb.2008.008160 [doi] PST - ppublish SO - Ann Clin Biochem. 2009 Mar;46(Pt 2):109-13. doi: 10.1258/acb.2008.008160. Epub 2009 Jan 22. PMID- 39615258 OWN - NLM STAT- MEDLINE DCOM- 20250205 LR - 20250711 IS - 1473-0502 (Print) IS - 1473-0502 (Linking) VI - 64 IP - 1 DP - 2025 Feb TI - Evaluating the safety and efficacy of plasma therapy/plasmapheresis for systemic sclerosis - A comprehensive systematic review. PG - 104036 LID - S1473-0502(24)00217-9 [pii] LID - 10.1016/j.transci.2024.104036 [doi] AB - INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disorder with fibrosis in multiple organs, autoantibodies, and microvascular abnormalities. Its origin is unclear, but it may result from circulatory damage, collagen metabolism disruption, and modifications in immunoregulation. The disease affects various organs and has high morbidity and mortality rates. SSc-related complications are managed using immunosuppressive medications that target autoantibodies. The main objective of this study was to assess the safety and efficacy of plasma therapy/plasmapheresis in managing SSc. METHODS: This systematic review followed PRISMA and IMRAD guidelines, using PICO framework for study selection based on MeSH terms and Boolean operators. It included cross-sectional, randomized control trials, and clinical studies on plasma therapy for SSc. Standardized protocols were used for data extraction and risk of bias assessment. DISCUSSION: Plasma therapy is a growing treatment option for managing SSc with reported benefits, especially in early stages and specific organ complications. However, further investigation and standardized protocols are needed. This review explores the potential of plasma therapy in improving the quality of life for SSc patients and in combination with other treatments. RESULT: The review analyzed 15 articles, including research papers, controlled trials, and case reports. Plasma therapy, involving Plasmapheresis and therapeutic plasma exchange (TPE), improved symptoms of SSc like Raynaud phenomenon, vasculitis, muscle dysfunction, and digital ulcers. However, outcomes varied among studies, and some advanced cases showed limited benefits. CONCLUSION: Plasma therapy can be an effective way of managing the symptoms of systemic sclerosis with low incidence of adverse events. However, the exact mechanism behind this treatment is still unclear. Therefore, additional studies are required. CI - Copyright © 2024 Elsevier Ltd. All rights reserved. FAU - Bano, Saira AU - Bano S AD - Bahria University, Medical and Dental College, Karachi 44000, Pakistan. Electronic address: sairabano56326@gmail.com. FAU - Jawed, Inshal AU - Jawed I AD - Dow University of Health sciences, Karachi 74200, Pakistan. Electronic address: inshaljwd@gmail.com. FAU - Abdul Qadir, Muhammad Umair AU - Abdul Qadir MU AD - Dow University of Health sciences, Karachi 74200, Pakistan. Electronic address: Umairqadir_19@outlook.com. FAU - Abbas Rizvi, Syed Ali Farhan AU - Abbas Rizvi SAF AD - Jinnah Medical and Dental College, Karachi 75510, Pakistan. Electronic address: Dr.syedalifarhan@gmail.com. FAU - Karmani, Vikash Kumar AU - Karmani VK AD - Jinnah Sindh Medical University, Karachi 75510, Pakistan. Electronic address: vikashkarmani@gmail.com. FAU - Alam, Farah AU - Alam F AD - Dow University of Health sciences, Karachi 74200, Pakistan. Electronic address: farahalam10@yahoo.com. FAU - Haseeb, Abdul AU - Haseeb A AD - Jinnah Sindh Medical University, Karachi 75510, Pakistan. Electronic address: abdulhaseebg96@gmail.com. FAU - Khan, Hina AU - Khan H AD - Jinnah Postgraduate Medical Centre, Karachi 75510, Pakistan. Electronic address: Khina0914@gmail.com. FAU - Mirza, Agha Muhammad Wali AU - Mirza AMW AD - Jinnah Medical and Dental College, Karachi 75510, Pakistan. Electronic address: walimirza110@hotmail.com. FAU - Akhtar, Naheed AU - Akhtar N AD - Bahria University, Medical and Dental College, Karachi 44000, Pakistan. Electronic address: naheedmasood@gmail.com. FAU - Bin Gulzar, Abu Huraira AU - Bin Gulzar AH AD - Services Institute of Medical Sciences, Lahore, Pakistan. Electronic address: abu.huraira.bin.gulzar@gmail.com. FAU - Hussien Mohamed Ahmed, Khabab Abbasher AU - Hussien Mohamed Ahmed KA AD - Faculty of Medicine, University of Khartoum, Khartoum 11111, Sudan. Electronic address: khabab9722@gmail.com. LA - eng PT - Journal Article PT - Systematic Review DEP - 20241123 PL - England TA - Transfus Apher Sci JT - Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis JID - 101095653 SB - IM MH - Humans MH - *Scleroderma, Systemic/therapy MH - *Plasmapheresis/methods MH - *Plasma Exchange/methods OTO - NOTNLM OT - Diffuse Sclerosis OT - Plasma Therapy OT - Plasmapheresis OT - Systemic Sclerosis OT - Treatment EDAT- 2024/12/01 15:22 MHDA- 2025/02/06 00:21 CRDT- 2024/11/30 18:07 PHST- 2024/06/14 00:00 [received] PHST- 2024/11/15 00:00 [revised] PHST- 2024/11/18 00:00 [accepted] PHST- 2025/02/06 00:21 [medline] PHST- 2024/12/01 15:22 [pubmed] PHST- 2024/11/30 18:07 [entrez] AID - S1473-0502(24)00217-9 [pii] AID - 10.1016/j.transci.2024.104036 [doi] PST - ppublish SO - Transfus Apher Sci. 2025 Feb;64(1):104036. doi: 10.1016/j.transci.2024.104036. Epub 2024 Nov 23. PMID- 28937580 OWN - NLM STAT- MEDLINE DCOM- 20180614 LR - 20180614 IS - 1558-2035 (Electronic) IS - 1558-2027 (Linking) VI - 18 IP - 11 DP - 2017 Nov TI - Diagnosis of systemic inflammatory diseases among patients admitted for acute pericarditis with pericardial effusion. PG - 875-880 LID - 10.2459/JCM.0000000000000576 [doi] AB - AIMS: Acute pericarditis may be the heralding manifestation of various systemic inflammatory diseases (SIDs). The aim of this study was to identify clinical indicators for SIDs in patients admitted for acute pericarditis with pericardial effusion. METHODS: All consecutive adult patients hospitalized in a Department of Internal Medicine over a 10-year period for acute pericarditis with pericardial effusion were retrospectively reviewed. Patients with cancer and tuberculosis were excluded. A structured clinical panel for extra-cardiac symptoms of SIDs was applied. SIDs were classified using current international criteria. RESULTS: Ninety-nine patients were admitted for acute pericarditis with pericardial effusion. After exclusion, 74 (49.7 ± 19.7 years, 56.7% women) patients were analyzed. Among them, 23 (23.2%) patients had a SID that was revealed by pericarditis in 12 cases. Systemic lupus erythematosus, undifferentiated connective-tissue disease, and Sjogren's syndrome accounted for 75% of the SID. Patients with SIDs were younger (P < 0.001), more frequently of female sex (P = 0.025), and had a higher frequency of extra-cardiac symptoms (P < 0.001) including arthralgia, myalgia, Raynaud phenomenon, and skin rash, as compared with patients with idiopathic pericarditis (n = 51). Overall, after exclusion of neoplasm and tuberculosis, the probability of SIDs in patients admitted with an acute pericarditis with pericardial effusion was 89.7% in patients younger than 50 who had extra-cardiac symptoms. Conversely, the probability fell to 8.4% in patients older than 50 with no extra-cardiac symptoms. CONCLUSION: Both age and extra-cardiac symptoms suggest an underlying SID as a possible cause of acute pericarditis. FAU - Assayag, Maureen AU - Assayag M AD - aDépartement de Médecine Interne bDépartement d'Epidémiologie et Recherche Clinique cDépartement d'Information Médicale dDépartement de Cardiologie, Hôpital Bichat, Université Paris Diderot, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris eINSERM U1149 fDépartement Hospitalo-Universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), Paris, France. FAU - Abbas, Rachid AU - Abbas R FAU - Chanson, Noémie AU - Chanson N FAU - Perozziello, Anne AU - Perozziello A FAU - Ducrocq, Gregory AU - Ducrocq G FAU - Alexandra, Jean-François AU - Alexandra JF FAU - Dossier, Antoine AU - Dossier A FAU - Papo, Thomas AU - Papo T FAU - Sacre, Karim AU - Sacre K LA - eng PT - Journal Article PL - United States TA - J Cardiovasc Med (Hagerstown) JT - Journal of cardiovascular medicine (Hagerstown, Md.) JID - 101259752 SB - IM CIN - J Cardiovasc Med (Hagerstown). 2018 Jul;19(7):391-392. doi: 10.2459/JCM.0000000000000639. PMID: 29846285 MH - Adult MH - Aged MH - Autoimmune Diseases/*diagnosis MH - Echocardiography MH - Female MH - Humans MH - Logistic Models MH - Male MH - Middle Aged MH - Pericardial Effusion/*etiology MH - Pericarditis/*diagnostic imaging/*physiopathology MH - Retrospective Studies EDAT- 2017/09/25 06:00 MHDA- 2018/06/15 06:00 CRDT- 2017/09/23 06:00 PHST- 2017/09/25 06:00 [pubmed] PHST- 2018/06/15 06:00 [medline] PHST- 2017/09/23 06:00 [entrez] AID - 10.2459/JCM.0000000000000576 [doi] PST - ppublish SO - J Cardiovasc Med (Hagerstown). 2017 Nov;18(11):875-880. doi: 10.2459/JCM.0000000000000576. PMID- 41467108 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20251230 LR - 20260101 IS - 2405-6308 (Electronic) IS - 2405-6308 (Linking) VI - 57 DP - 2026 Mar TI - Benefit of prophylactic pelvic irradiation in intermediate-risk prostate cancer: A multicenter retrospective study (iPPAPI). PG - 101093 LID - 10.1016/j.ctro.2025.101093 [doi] LID - 101093 AB - Context.Prostate cancer (PCa) is the most common urologic malignancy in men, with most cases diagnosed at a localized stage. The benefit of whole-pelvic radiotherapy (WPRT) to eradicate subclinical nodal disease remains debated, particularly in intermediate-risk PCa. This study assessed the impact of WPRT in this population. METHODS: We conducted a multicenter retrospective study within the AP-HP GRRAP program across five radiotherapy departments. Biopsy-proven intermediate-risk PCa (d'Amico classification) treated with conformational external beam radiotherapy between 2010 and 2019 were included. The primary endpoint was recurrence-free survival (RFS), defined as time from diagnosis to biochemical, local, metastatic recurrence, or death. Secondary endpoints were overall survival (OS) and acute (<6 months) or late (≥6 months) genitourinary (GU), gastrointestinal (GI), and sexual toxicities (CTCAE v4.03/v5.0). Survival outcomes were assessed using univariate and multivariate Cox models. RESULTS: Three hundred patients (60 per center) were included; 94 % received IMRT and 6 % 3D-RT. After a median follow-up of 77 months, univariate analysis showed no significant association between WPRT and RFS (HR; 0.61; 95 % CI, 0.26-1.42; p = 0.25). Multivariable analysis adjusted for clinical and treatment factors yielded similar results (HR, 0.70; 95 % CI, 0.27 to 1.81; p = 0.46). OS results were also comparable. Rates of grade 2 acute or late toxicities were similar, but grade ≥ 3 late GI toxicity was higher with WPRT (14.3 % vs. 5.4 %; p = 0.045; OR 2.90). CONCLUSION: In intermediate-risk PCa, WPRT did not improve RFS or OS compared with prostate-only RT but was associated with an increased risk of severe GI toxicity. CI - © 2025 The Author(s). FAU - Raynaud, Charles AU - Raynaud C AD - Radiation Oncology Department, Hôpital Européen Georges Pompidou, Paris, France. FAU - Nebbache, Rafik AU - Nebbache R AD - Radiation Oncology Department, Institut Gustave Roussy, Villejuif, France. FAU - Belkacemi, Yazid AU - Belkacemi Y AD - Radiation Oncology Department, Hôpital Mondor, Creteil, France. FAU - Chargari, Cyrus AU - Chargari C AD - Radiation Oncology Department, Hôpital Pitié-Salpêtrière, Paris, France. FAU - Durdux, Catherine AU - Durdux C AD - Radiation Oncology Department, Hôpital Européen Georges Pompidou, Paris, France. FAU - Hennequin, Christophe AU - Hennequin C AD - Radiation Oncology Department, Hôpital Saint Louis, Paris, France. FAU - Huguet, Florence AU - Huguet F AD - Radiation Oncology Department, Hôpital Tenon, Paris, France. FAU - Quero, Laurent AU - Quero L AD - Radiation Oncology Department, Hôpital Saint Louis, Paris, France. FAU - Bibault, Jean-Emmanuel AU - Bibault JE AD - Radiation Oncology Department, Hôpital Européen Georges Pompidou, Paris, France. AD - INSERM UMR 1138, Centre de Recherche des Cordeliers, Paris, France. LA - eng PT - Journal Article DEP - 20251206 PL - Ireland TA - Clin Transl Radiat Oncol JT - Clinical and translational radiation oncology JID - 101713416 PMC - PMC12744236 OTO - NOTNLM OT - GI toxicity OT - Intermediate risk OT - Prostate cancer OT - Radiotherapy OT - Whole Pelvic Radiotherapy COIS- The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2025/12/30 07:40 MHDA- 2025/12/30 07:41 PMCR- 2025/12/06 CRDT- 2025/12/30 04:22 PHST- 2025/09/11 00:00 [received] PHST- 2025/12/03 00:00 [revised] PHST- 2025/12/05 00:00 [accepted] PHST- 2025/12/30 07:41 [medline] PHST- 2025/12/30 07:40 [pubmed] PHST- 2025/12/30 04:22 [entrez] PHST- 2025/12/06 00:00 [pmc-release] AID - S2405-6308(25)00185-5 [pii] AID - 101093 [pii] AID - 10.1016/j.ctro.2025.101093 [doi] PST - epublish SO - Clin Transl Radiat Oncol. 2025 Dec 6;57:101093. doi: 10.1016/j.ctro.2025.101093. eCollection 2026 Mar. PMID- 40961376 OWN - NLM STAT- MEDLINE DCOM- 20251206 LR - 20251208 IS - 1522-2594 (Electronic) IS - 0740-3194 (Print) IS - 0740-3194 (Linking) VI - 95 IP - 2 DP - 2026 Feb TI - ISME-incoherent sampling of multi-echo data to minimize cardiac-induced noise in brain maps of R2* and magnetic susceptibility. PG - 897-911 LID - 10.1002/mrm.70087 [doi] AB - PURPOSE: Maps of the MRI parameters R2* and magnetic susceptibility ( χ ) enable the investigation of microscopic tissue changes in brain disease. However, cardiac-induced signal instabilities increase the variability of brain maps of R2* and χ . In this study, we introduce incoherent sampling of multi-echo data (ISME)-a sampling strategy that minimizes the level of cardiac-induced instabilities in brain maps of R2* and χ . METHODS: ISME uses phase-encoding gradients to shift the k-space frequency of the acquired data between consecutive readouts of a multi-echo train. As a result, the multi-echo data at a given k-space index are acquired at different phases of the cardiac cycle. We compare the variability of R2* and χ maps acquired with ISME and with standard multi-echo trajectories in N = 10 healthy volunteers. We investigate the effect of both trajectories on the spatial aliasing of pulsating MR signals and propose a weighted least-squares approach for the estimation of R2* that accounts for the increase of the residuals with echo time. RESULTS: ISME reduces the variability of R2* and χ maps across repetitions by 25%/26%/21% and 24%/32%/23% in the cerebellum/brainstem/whole brain, respectively. With ISME, the spatial aliasing of pulsating MR signals is incoherent between raw echo images, leading to visually sharper R2* maps. The proposed weighted least-squares approach for the estimation of R2* reduces the dependence of the fitting residuals on echo time and the variability of R2* by an additional 3%/2%/1% in the cerebellum/brainstem/whole brain. CONCLUSION: ISME allows the mitigation of cardiac-induced signal instabilities in brain maps of R2* and χ , improving reproducibility. CI - © 2025 The Author(s). Magnetic Resonance in Medicine published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine. FAU - Raynaud, Quentin AU - Raynaud Q AUID- ORCID: 0000-0002-6143-2873 AD - Laboratory for Research in Neuroimaging, Department for Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. FAU - Oliveira, Rita AU - Oliveira R AUID- ORCID: 0000-0002-7597-6919 AD - Laboratory for Research in Neuroimaging, Department for Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. FAU - Corbin, Nadège AU - Corbin N AUID- ORCID: 0000-0003-3296-4544 AD - Centre de Résonance Magnétique des Systèmes Biologiques, UMR5536, CNRS/University Bordeaux, Bordeaux, France. FAU - Balbastre, Yaël AU - Balbastre Y AD - Department of Experimental Psychology, University College London, London, UK. FAU - van Heeswijk, Ruud B AU - van Heeswijk RB AUID- ORCID: 0000-0001-5028-4521 AD - Department of Diagnostic and Interventional Radiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. FAU - Lutti, Antoine AU - Lutti A AD - Laboratory for Research in Neuroimaging, Department for Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. LA - eng GR - 320030_184784/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ GR - CR00I5_235940/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ GR - 32003B_182615/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ GR - CRSII5_202276/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ GR - Royal Society/ PT - Journal Article DEP - 20250917 PL - United States TA - Magn Reson Med JT - Magnetic resonance in medicine JID - 8505245 SB - IM MH - Humans MH - *Brain/diagnostic imaging MH - *Magnetic Resonance Imaging/methods MH - *Heart/diagnostic imaging MH - Algorithms MH - *Image Processing, Computer-Assisted/methods MH - Adult MH - *Brain Mapping/methods MH - Male MH - Signal-To-Noise Ratio MH - Female MH - Reproducibility of Results PMC - PMC12681315 OTO - NOTNLM OT - MRI relaxometry OT - QSM OT - R2* OT - neuroimaging OT - physiological noise OT - quantitative MRI EDAT- 2025/09/17 18:31 MHDA- 2025/12/06 21:31 PMCR- 2025/12/06 CRDT- 2025/09/17 15:33 PHST- 2025/08/29 00:00 [revised] PHST- 2025/02/24 00:00 [received] PHST- 2025/08/30 00:00 [accepted] PHST- 2025/12/06 21:31 [medline] PHST- 2025/09/17 18:31 [pubmed] PHST- 2025/09/17 15:33 [entrez] PHST- 2025/12/06 00:00 [pmc-release] AID - MRM70087 [pii] AID - 10.1002/mrm.70087 [doi] PST - ppublish SO - Magn Reson Med. 2026 Feb;95(2):897-911. doi: 10.1002/mrm.70087. Epub 2025 Sep 17. PMID- 40176384 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250829 IS - 2578-5745 (Electronic) IS - 2578-5745 (Linking) VI - 7 IP - 4 DP - 2025 Apr TI - Osteoclastogenesis in Patients With Systemic Sclerosis With and Without Calcinosis Cutis. PG - e70029 LID - 10.1002/acr2.70029 [doi] LID - e70029 AB - OBJECTIVE: We aimed to assess whether the presence of radiographically confirmed calcinosis of the hands in patients with systemic sclerosis (SSc) is associated with increased osteoclastogenesis. METHODS: We recruited 20 patients with SSc (10 with calcinosis and 10 without calcinosis) and 10 age- and gender-matched healthy controls. Hand radiographs were scored using the validated Scleroderma Clinical Trials Consortium (SCTC) radiographic severity score for calcinosis. To evaluate osteoclastogenesis, peripheral blood mononuclear cells (PBMCs) were cultured with RANKL and macrophage colony-stimulating factor; osteoclasts were identified using tartrate-resistant acid phosphatase staining. Measures of bone resorption (RANKL, osteoprotegerin [OPG]) and ischemia or endothelial function (vascular endothelial growth factor, angiopoietin-1, and angiopoietin-2 [Ang-2]) were also evaluated. RESULTS: Patients with SSc were all women and Hispanic, and the majority (n = 12, 60%) had limited SSc skin type. Mean ± SD age was 55.2 ± 14.8 years; mean ± SD disease duration was 9.5 ± 6.5 years from first non-Raynaud phenomenon symptom. Patients with SSc with calcinosis had more digital ischemia than patients without calcinosis. Median SCTC score in patients with SSc with calcinosis was 11.1 (range 0.7-286). After 9 days in culture, PBMCs from patients with calcinosis originated a significantly higher number of osteoclasts (33.0 ± 20.3 cells/well) than patients without calcinosis (15.3 ± 6.9 cells/well) and healthy individuals (11.2 ± 2.6 cells/well) (P = 0.001). The severity of calcinosis was not correlated with the number of osteoclasts per well (r = 0.27, P = 0.5); however, it was correlated with RANKL (r = 0.82, P = 0.004), RANKL/OPG ratio (r = 0.86, P = 0.002), and Ang-2 levels (r = 0.86, P = 0.002). CONCLUSION: Calcinosis in patients with SSc is associated with an increased propensity of peripheral blood cells to form osteoclasts. Targeted inhibition of osteoclastogenesis may provide a specific therapeutic option for patients with SSc-associated calcinosis. CI - © 2025 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Valenzuela, Antonia AU - Valenzuela A AUID- ORCID: 0000-0003-3357-9402 AD - Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Pérez, Guillermo AU - Pérez G AD - Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Chung, Lorinda AU - Chung L AD - Stanford University School of Medicine, Palo Alto, California. FAU - Sánchez, Felipe AU - Sánchez F AD - Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Iturriaga, Carolina AU - Iturriaga C AD - Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Montalva, Rebeca AU - Montalva R AD - Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Borzutzky, Arturo AU - Borzutzky A AUID- ORCID: 0000-0002-7904-262X AD - Pontificia Universidad Católica de Chile, Santiago, Chile. LA - eng GR - 11190426/Agencia Nacional de Investigación y Desarrollo/ GR - Scleroderma Clinical Trials Consortium/ PT - Journal Article PL - United States TA - ACR Open Rheumatol JT - ACR open rheumatology JID - 101740025 PMC - PMC11965697 EDAT- 2025/04/03 09:06 MHDA- 2025/04/03 09:07 PMCR- 2025/04/02 CRDT- 2025/04/03 02:03 PHST- 2025/02/14 00:00 [revised] PHST- 2024/12/06 00:00 [received] PHST- 2025/03/07 00:00 [accepted] PHST- 2025/04/03 09:07 [medline] PHST- 2025/04/03 09:06 [pubmed] PHST- 2025/04/03 02:03 [entrez] PHST- 2025/04/02 00:00 [pmc-release] AID - ACR270029 [pii] AID - 10.1002/acr2.70029 [doi] PST - ppublish SO - ACR Open Rheumatol. 2025 Apr;7(4):e70029. doi: 10.1002/acr2.70029. PMID- 26300023 OWN - NLM STAT- MEDLINE DCOM- 20170103 LR - 20240324 IS - 1873-6513 (Electronic) IS - 0885-3924 (Print) IS - 0885-3924 (Linking) VI - 51 IP - 1 DP - 2016 Jan TI - Symptom Interference Severity and Health-Related Quality of Life in Pulmonary Arterial Hypertension. PG - 25-32 LID - S0885-3924(15)00407-8 [pii] LID - 10.1016/j.jpainsymman.2015.07.012 [doi] AB - CONTEXT: While assessing symptom severity is an important component of evaluating symptoms, understanding those symptoms that interfere with patients' lives is also key. Pulmonary arterial hypertension (PAH) is a chronic disease resulting in right heart failure and increased mortality. Patients with PAH experience multiple symptoms but we do not know which symptoms and to what extent their symptoms interfere with daily life. OBJECTIVES: To: (1) describe the prevalence of those symptoms that interfere with life; (2) describe the severity of symptom interference; and (3) determine those sociodemographic and clinical characteristics and interfering symptoms associated with health-related quality of life (HRQOL) in patients with PAH. METHODS: A convenience sample of 191 patients with PAH completed a sociodemographic form, the Pulmonary Arterial Hypertension Symptom Interference Scale (PAHSIS) and the Medical Outcomes Survey Short Form-36 to measure HRQOL. Hierarchical multiple linear regression was used to analyze demographic and medical characteristics along with symptom interference from the PAHSIS as predictors of HRQOL from the composite mental and physical health summary scores of the Short Form-36. RESULTS: The most interfering symptoms reported were fatigue, shortness of breath with exertion, and difficulty sleeping. Age, gender, functional class, oxygen use, fatigue, dizziness, and Raynaud phenomenon were associated with the HRQOL physical health summary scores. The symptoms fatigue and SOB while lying down were associated with the HRQOL mental health summary scores. CONCLUSION: Patients with PAH are experiencing multiple symptoms that are interfering with their HRQOL and ability to function. CI - Copyright © 2016 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved. FAU - Matura, Lea Ann AU - Matura LA AD - University of Pennsylvania School of Nursing, Philadelphia, Pennsylvania, USA. Electronic address: matura@nursing.upenn.edu. FAU - McDonough, Annette AU - McDonough A AD - University of Massachusetts, Lowell, Massachusetts, USA. FAU - Carroll, Diane L AU - Carroll DL AD - Yvonne L. Munn Center for Nursing Research, Institute for Patient Care, Massachusetts General Hospital, Boston, Massachusetts, USA. LA - eng GR - K23 NR014885/NR/NINR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150820 PL - United States TA - J Pain Symptom Manage JT - Journal of pain and symptom management JID - 8605836 SB - IM MH - Cross-Sectional Studies MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnosis/*epidemiology/*physiopathology/therapy MH - Male MH - Mental Disorders/epidemiology MH - Middle Aged MH - Multivariate Analysis MH - Prevalence MH - *Quality of Life MH - Regression Analysis MH - Severity of Illness Index MH - Sex Factors PMC - PMC4698220 MID - NIHMS717608 OTO - NOTNLM OT - Symptoms OT - interference OT - pulmonary arterial hypertension OT - quality of life COIS- Disclosures The authors declare no conflicts of interest. EDAT- 2015/08/25 06:00 MHDA- 2017/01/04 06:00 PMCR- 2017/01/01 CRDT- 2015/08/25 06:00 PHST- 2015/03/24 00:00 [received] PHST- 2015/07/08 00:00 [revised] PHST- 2015/07/23 00:00 [accepted] PHST- 2015/08/25 06:00 [entrez] PHST- 2015/08/25 06:00 [pubmed] PHST- 2017/01/04 06:00 [medline] PHST- 2017/01/01 00:00 [pmc-release] AID - S0885-3924(15)00407-8 [pii] AID - 10.1016/j.jpainsymman.2015.07.012 [doi] PST - ppublish SO - J Pain Symptom Manage. 2016 Jan;51(1):25-32. doi: 10.1016/j.jpainsymman.2015.07.012. Epub 2015 Aug 20. PMID- 24772147 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20140428 LR - 20211021 IS - 1682-024X (Print) IS - 1681-715X (Electronic) IS - 1681-715X (Linking) VI - 30 IP - 2 DP - 2014 Mar TI - Determination of Anti-nuclear Antibody Pattern Distribution and Clinical Relationship. PG - 380-3 AB - BACKGROUND AND OBJECTIVES: Autoantibodies are immunglobulins occurred directly against autoantigens that are known as endogen antigens. Autoimmune disease is an occasion that the body begins a fight against its own cells and tissues. The antibodies that are created by the body against its own cell nuclei are called as anti-nuclear antibodies (ANA), and one of the methods used for detection and pattern of ANA is indirect immunofluorescence test (IIF). In the present study, it was aimed to determine the rate of ANA positivity and patterns of the positive specimens, and to investigate the relationship between ANA positivity and diseases in patients. METHODS: ANA test results of a total of 3127 patients admitted during March 2010 to December 2012 were evaluated retrospectively. ANA test (HEp 20-10, EUROIMMUN, Germany) was used in dilution of 1:100 in IIF test. RESULTS: A total of 494 (15.8%) resulted as ANA positive. ANA positivity rate was significantly higher in female patients than the male ones (p<0.001). The most frequent ANA patterns were coarse speckled pattern (154 patients, 31.2%), nucleolar pattern (89 patients, 18.0%), fine speckled pattern (57 patients, 11.5%), and speckled pattern (48 patients, 9.7%). ANA positivity was most commonly determined in rheumatoid arthritis (RA) (42 patients, 8.5%), systemic lupus erythematosus (SLE) (29 patients, 5.9%), and rheumatoid vasculitis (RV) (28 patients, 5.7%). The most frequent symptoms or findings were joint pain (127 patients, 26.0%) and anemia (28 patients, 5.7%). ANA positivity rates were found to be significantly higher in patients with RA (p<0.001), with SLE (p<0.001), and with Raynaud phenomenon (p=0.001) in comparison to the controls. Amongst the most frequent diseases evaluated, no significant differences were found between the control groups and the groups of RV (p=0.089), multiple sclerosis (p=0.374), and Sjögren syndrome (p=0.311) in terms of ANA positivity rates. CONCLUSIONS: The present study is the first study reporting the positivity rate and distribution of ANA in Bolu located in northwestern Turkey. Information about the pattern types and the distribution of the patterns according to the diseases and symptoms contribute in diagnosis of autoimmune diseases. It is observed that clinical diagnosis has been supported significantly by ANA test according to data of our study. FAU - Mengeloglu, Zafer AU - Mengeloglu Z AD - Zafer Mengeloglu, MD, Assistant Professor, Department of Medical Microbiology, Abant Izzet Baysal University School of Medicine, Bolu, Turkey. FAU - Tas, Tekin AU - Tas T AD - Tekin Tas, MD, Assistant Professor, Department of Medical Microbiology, Abant Izzet Baysal University School of Medicine, Bolu, Turkey. FAU - Kocoglu, Esra AU - Kocoglu E AD - Esra Kocoglu, MD, Associate Professor, Department of Medical Microbiology, Abant Izzet Baysal University School of Medicine, Bolu, Turkey. FAU - Aktas, Gülali AU - Aktas G AD - Gülali Aktas, MD, Assistant Professor, Department of Internal Diseases, Abant Izzet Baysal University School of Medicine, Bolu, Turkey. FAU - Karabörk, Seyda AU - Karabörk S AD - Seyda Karabörk, Specialist of Medical Microbiology, Department of Medical Microbiology, Abant Izzet Baysal University School of Medicine, Bolu, Turkey. LA - eng PT - Journal Article PL - Pakistan TA - Pak J Med Sci JT - Pakistan journal of medical sciences JID - 100913117 PMC - PMC3999014 OTO - NOTNLM OT - ANA OT - Anti-nuclear antibody OT - IIF OT - Immunfluorescence EDAT- 2014/04/29 06:00 MHDA- 2014/04/29 06:01 PMCR- 2014/03/01 CRDT- 2014/04/29 06:00 PHST- 2013/08/22 00:00 [received] PHST- 2013/12/28 00:00 [revised] PHST- 2013/12/28 00:00 [accepted] PHST- 2014/04/29 06:00 [entrez] PHST- 2014/04/29 06:00 [pubmed] PHST- 2014/04/29 06:01 [medline] PHST- 2014/03/01 00:00 [pmc-release] AID - pjms-30-380 [pii] AID - 10.12669/pjms.302.4276 [doi] PST - ppublish SO - Pak J Med Sci. 2014 Mar;30(2):380-3. doi: 10.12669/pjms.302.4276. PMID- 38457093 OWN - NLM STAT- MEDLINE DCOM- 20240426 LR - 20240502 IS - 2107-0180 (Electronic) IS - 0378-7966 (Linking) VI - 49 IP - 3 DP - 2024 May TI - Anti-Calcitonin Gene-Related Peptide Monoclonal Antibodies in Migraine: Focus on Drug Interactions. PG - 263-275 LID - 10.1007/s13318-024-00887-3 [doi] AB - Calcitonin gene-related peptide neurotransmission was the target for recent development of monoclonal antibodies that effectively prevent attacks of both episodic and chronic migraine. The aim of this narrative review was to offer deeper insight into drug-drug, drug-food and drug-disease interactions of monoclonal antibodies approved for prevention of migraine attacks. For this narrative review, relevant literature was searched for in MEDLINE and Google Scholar databases, covering the 1966-2023 and 2006-2023 periods, respectively. The ClinicalTrials.gov database was also searched for relevant clinical studies whose results had not been published previously in medical journals, covering 2000-2023. Monoclonal antibodies (erenumab, fremanezumab, galcanezumab and eptinezumab) augment prophylactic action of gepants and onabotulinumtoxin A and somewhat increase efficacy of triptans used to abort migraine attacks; however, their adverse reactions may also be augmented. Pharmacokinetic interactions and interactions in general with drugs used for other indications except migraine are negligible, as are drug-food interactions. However, monoclonal antibodies may worsen diseases with already weakened CGRP neurotransmission, Raynaud phenomenon and constipation. Monoclonal antibodies used for prevention of migraine do not engage in significant pharmacokinetic interactions with other drugs; however, they do engage in pharmacodynamic interactions with other anti-migraine drugs, additively augmenting their prophylactic action, but also increasing frequency and severity of adverse reactions, which are a consequence of the CGRP neurotransmission interruption. CI - © 2024. The Author(s), under exclusive licence to Springer Nature Switzerland AG. FAU - Janković, Slobodan M AU - Janković SM AUID- ORCID: 0000-0002-1519-8828 AD - Department of Pharmacology and Toxicology, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovića Street, 69, 34000, Kragujevac, Serbia. slobnera@gmail.com. FAU - Janković, Snežana V AU - Janković SV AD - Department of Pharmacology and Toxicology, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovića Street, 69, 34000, Kragujevac, Serbia. LA - eng GR - Grant no. 175007/contract No 451-03-47/2023-01/200111/Ministarstvo Prosvete, Nauke i Tehnološkog Razvoja/ PT - Journal Article PT - Review DEP - 20240308 PL - France TA - Eur J Drug Metab Pharmacokinet JT - European journal of drug metabolism and pharmacokinetics JID - 7608491 RN - 0 (Antibodies, Monoclonal) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) SB - IM MH - *Migraine Disorders/drug therapy MH - Humans MH - *Antibodies, Monoclonal/therapeutic use/pharmacokinetics/pharmacology/adverse effects MH - *Calcitonin Gene-Related Peptide/immunology/antagonists & inhibitors MH - *Drug Interactions MH - Calcitonin Gene-Related Peptide Receptor Antagonists/pharmacology/therapeutic use MH - Food-Drug Interactions MH - Animals EDAT- 2024/03/08 12:42 MHDA- 2024/04/26 13:21 CRDT- 2024/03/08 11:13 PHST- 2024/02/26 00:00 [accepted] PHST- 2024/04/26 13:21 [medline] PHST- 2024/03/08 12:42 [pubmed] PHST- 2024/03/08 11:13 [entrez] AID - 10.1007/s13318-024-00887-3 [pii] AID - 10.1007/s13318-024-00887-3 [doi] PST - ppublish SO - Eur J Drug Metab Pharmacokinet. 2024 May;49(3):263-275. doi: 10.1007/s13318-024-00887-3. Epub 2024 Mar 8. PMID- 37789889 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231005 IS - 1664-2295 (Print) IS - 1664-2295 (Electronic) IS - 1664-2295 (Linking) VI - 14 DP - 2023 TI - Novel variants in the CLCN4 gene associated with syndromic X-linked intellectual disability. PG - 1096969 LID - 10.3389/fneur.2023.1096969 [doi] LID - 1096969 AB - OBJECTIVE: The dysfunction of the CLCN4 gene can lead to X-linked intellectual disability and Raynaud-Claes syndrome (MRXSRC), characterized by severe cognitive impairment and mental disorders. This study aimed to investigate the genetic defects and clinical features of Chinese children with CLCN4 variants and explore the effect of mutant ClC-4 on the protein expression level and subcellular localization through in vitro experiments. METHODS: A total of 401 children with intellectual disabilities were screened for genetic variability using whole-exome sequencing (WES). Clinical data, including age, sex, perinatal conditions, and environmental exposure, were collected. Cognitive, verbal, motor, and social behavioral abilities were evaluated. Candidate variants were verified using Sanger sequencing, and their pathogenicity and conservation were analyzed using in silico prediction tools. Protein expression and localization of mutant ClC-4 were measured using Western blotting (WB) and immunofluorescence microscopy. The impact of a splice site variant was assessed with a minigene assay. RESULTS: Exome analysis identified five rare CLCN4 variants in six unrelated patients with intellectual disabilities, including two recurrent heterozygous de novo missense variants (p.D89N and p.A555V) in three female patients, and two hemizygous missense variants (p.N141S and p.R694Q) and a splicing variant (c.1390-12T > G) that are maternally inherited in three male patients. The p.N141S variant and the splicing variant c.1390-12(T > G were novel, while p.R694Q was identified in two asymptomatic heterozygous female patients. The six children with CLCN4 variants exhibited a neurodevelopmental spectrum disease characterized by intellectual disability (ID), delayed speech, autism spectrum disorders (ASD), microcephaly, hypertonia, and abnormal imaging findings. The minigene splicing result indicated that the c.1390-12T > G did not affect the splicing of CLCN4 mRNA. In vitro experiments showed that the mutant protein level and localization of mutant protein are similar to the wild type. CONCLUSION: The study identified six probands with CLCN4 gene variants associated with X-linked ID. It expanded the gene and phenotype spectrum of CLCN4 variants. The bioinformatic analysis supported the pathogenicity of CLCN4 variants. However, these CLCN4 gene variants did not affect the ClC-4 expression levels and protein location, consistent with previous studies. Further investigations are necessary to investigate the pathogenetic mechanism. CI - Copyright © 2023 Li, Zhang, Liang, Zhu, Zheng, Zhou, Wang and Zhao. FAU - Li, Sinan AU - Li S AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Zhang, Wenxin AU - Zhang W AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Liang, Piao AU - Liang P AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Zhu, Min AU - Zhu M AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Zheng, Bixia AU - Zheng B AD - Nanjing Key Laboratory of Pediatrics, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Zhou, Wei AU - Zhou W AD - Nanjing Key Laboratory of Pediatrics, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Wang, Chunli AU - Wang C AD - Nanjing Key Laboratory of Pediatrics, Children's Hospital of Nanjing Medical University, Nanjing, China. FAU - Zhao, Xiaoke AU - Zhao X AD - Department of Rehabilitation, Children's Hospital of Nanjing Medical University, Nanjing, China. LA - eng PT - Journal Article DEP - 20230915 PL - Switzerland TA - Front Neurol JT - Frontiers in neurology JID - 101546899 PMC - PMC10542403 OTO - NOTNLM OT - CLCN4 gene OT - MRXSRC OT - intellectual disability OT - missense variant OT - splice site variant COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/10/04 06:43 MHDA- 2023/10/04 06:44 PMCR- 2023/09/15 CRDT- 2023/10/04 03:55 PHST- 2022/11/13 00:00 [received] PHST- 2023/08/15 00:00 [accepted] PHST- 2023/10/04 06:44 [medline] PHST- 2023/10/04 06:43 [pubmed] PHST- 2023/10/04 03:55 [entrez] PHST- 2023/09/15 00:00 [pmc-release] AID - 10.3389/fneur.2023.1096969 [doi] PST - epublish SO - Front Neurol. 2023 Sep 15;14:1096969. doi: 10.3389/fneur.2023.1096969. eCollection 2023. PMID- 35169064 OWN - NLM STAT- MEDLINE DCOM- 20220603 LR - 20240801 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 49 IP - 6 DP - 2022 Jun TI - Impaired Fibrinolysis Is Linked With Digital Vasculopathy and Onset of New Digital Ulcers in Systemic Sclerosis. PG - 598-606 LID - 10.3899/jrheum.210931 [doi] AB - OBJECTIVE: To assess thrombin generation, fibrin formation, and structure together with the fibrinolytic status in patients with systemic sclerosis (SSc) in relation to the occurrence of digital ulcers (DUs) during the course of disease. METHODS: We studied variables of endothelial dysfunction, thrombin generation, overall hemostatic potential, and fibrin clot turbidity in plasma from 58 patients with SSc (39 with DU history and 19 DU-naïve) and 46 matched healthy controls (HCs). Fibrin structure was visualized using scanning electron microscopy (SEM). Finally, 39 patients with a history of DUs were followed for 1.5 years and the predictive value of all investigated markers for new DU onset was explored. RESULTS: Significantly enhanced endogenous thrombin potential (ETP) and prolonged clot lysis time (CLT) were found in patients with DUs compared to HCs. CLT was prolonged in patients with DUs compared to those without, showing good validity in identifying DUs with an area under the curve of 0.7 (95% CI 0.6-0.8). The levels of ETP and intercellular adhesion molecule 1 were independently associated with CLT. Over the follow-up period, 20 patients developed new DUs. CLT was prolonged (P < 0.001) in patients with new DU episodes, especially those with recurrent DUs. Regression analysis showed that the Raynaud phenomenon visual analog scale and CLT were predictors of new DUs (OR 1.1, 95% CI 1.0-1.1 and OR 1.2, 95% CI 1.1-1.3, respectively). SEM confirmed denser fibrin clots in patients with new DUs. CONCLUSION: Our results suggest that impaired fibrinolysis might have an emerging role in underlying digital vasculopathy and its progression in SSc. CI - Copyright © 2022 by the Journal of Rheumatology. FAU - Colic, Jelena AU - Colic J AUID- ORCID: 0000-0002-8418-3056 AD - J. Colic, MD, PhDc, Institute of Rheumatology, Belgrade, Serbia; jcolicster@gmail.com. FAU - Pruner, Iva AU - Pruner I AD - I. Pruner, PhD, Karolinska Institutet, Department of Molecular Medicine and Surgery, Stockholm, Sweden. FAU - Damjanov, Nemanja AU - Damjanov N AD - N. Damjanov, MD, PhD, M. Sefik-Bukilica, MD, PhD, Institute of Rheumatology, Belgrade, Serbia School of Medicine University of Belgrade, Belgrade, Serbia. FAU - Pekmezovic, Tatjana AU - Pekmezovic T AUID- ORCID: 0000-0001-7978-1409 AD - T. Pekmezovic, MD, PhD, Institute of Epidemiology, School of Medicine University of Belgrade, Belgrade, Serbia. FAU - Sefik-Bukilica, Mirjana AU - Sefik-Bukilica M AUID- ORCID: 0000-0003-3421-6232 AD - N. Damjanov, MD, PhD, M. Sefik-Bukilica, MD, PhD, Institute of Rheumatology, Belgrade, Serbia School of Medicine University of Belgrade, Belgrade, Serbia. FAU - Antovic, Aleksandra AU - Antovic A AUID- ORCID: 0000-0002-7421-5069 AD - A. Antovic, MD, PhD, Karolinska Institutet, Department of Medicine Division Solna of Rheumatology, Karolinska University Hospital, Rheumatology, Stockholm, Sweden. LA - eng PT - Journal Article DEP - 20220215 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 RN - 9001-31-4 (Fibrin) RN - EC 3.4.21.5 (Thrombin) SB - IM EIN - J Rheumatol. 2022 May;49(5):547. doi: 10.3899/jrheum.210931.C1. PMID: 35491017 MH - Fibrin MH - Fibrin Clot Lysis Time MH - Fibrinolysis MH - Humans MH - *Scleroderma, Systemic/complications MH - *Skin Ulcer/complications MH - Thrombin MH - *Thrombosis MH - Ulcer OTO - NOTNLM OT - clot lysis time OT - digital ulcers OT - fibrin structure OT - hemostasis OT - systemic sclerosis EDAT- 2022/02/17 06:00 MHDA- 2022/06/07 06:00 CRDT- 2022/02/16 05:46 PHST- 2022/02/02 00:00 [accepted] PHST- 2022/02/17 06:00 [pubmed] PHST- 2022/06/07 06:00 [medline] PHST- 2022/02/16 05:46 [entrez] AID - jrheum.210931 [pii] AID - 10.3899/jrheum.210931 [doi] PST - ppublish SO - J Rheumatol. 2022 Jun;49(6):598-606. doi: 10.3899/jrheum.210931. Epub 2022 Feb 15. PMID- 32982946 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 1664-2295 (Print) IS - 1664-2295 (Electronic) IS - 1664-2295 (Linking) VI - 11 DP - 2020 TI - Myasthenia Gravis Coexisting With Primary Sjögren's Syndrome: Report of Three Cases and Literature Review. PG - 939 LID - 10.3389/fneur.2020.00939 [doi] LID - 939 AB - Objective: The coexistence of myasthenia gravis (MG) and primary Sjögren's syndrome (pSS) is rarely reported. This study aims to describe the clinical features, treatment and outcome of MG coexisting with pSS. Materials and Methods: Herein we reported three cases with the two coexisting diseases, and also searched the PubMed, Medline databases, and China Wanfang databases for the relevant case reports written in English, Chinese, or Japanese with detailed data. Results: We reviewed a total of 17 patients with both diseases. Fifteen patients were female. The median age at onset was 48 years (range 28-78 years). MG was the initial disease in nine of 17 cases. The median interval between the onsets of the two diseases was 30 months (range 7 months to 20 years). The symptoms of MG included fatigable ptosis (64.7%), bulbar symptoms (58.8%), muscle fatigability (64.7%), diplopia (64.7%), dyspnea (23.5%), and facial paralysis (5.9%). Anti-acetylcholine receptor antibody was positive in 70.6% patients. All the patients had sicca symptoms. Manifestations of pSS also included swollen exocrine glands (23.5%), joint pain (23.5%), hair loss (11.8%), leukopenia (11.8%), recurrent oral ulcers (5.9%), Raynaud phenomenon (5.9%), and fever (5.9%). ANA positivity was present in 70.6% patients, anti-SSA positivity in 47.1%, and double positivity of anti-SSA and anti-SSB in 17.6%. There were 12 patients (70.6%) with two autoimmune diseases (pSS and MG), and five patients with more than two autoimmune diseases. Cholinesterase inhibitors were the most commonly prescribed drugs (82.4%). Seven patients received thymectomy and one patient improved after the operation. Two patients were given intravenous methylprednisolone pulse therapy, and four patients oral steroids combined with immunosuppressants initially. Intravenous immunoglobulin and plasma exchange were used in two patients, respectively, for the respiratory failure. All the patients improved following treatment except one patient who died of MG crisis due to medication withdrawal. Conclusion: The coexistence of SS with MG is quite rare. The onset of MG may occur before or after the diagnosis of SS. Co-morbidity with MG does not seem to adversely affect the course of SS. Thus, controlling the progress of MG is the critical aspect of treatment. CI - Copyright © 2020 Li, Zhao, Liao and Da. FAU - Li, Xia AU - Li X AD - Department of Rheumatology & Allergy, Xuanwu Hospital, Capital Medical University, Beijing, China. FAU - Zhao, Yi AU - Zhao Y AD - Department of Rheumatology & Allergy, Xuanwu Hospital, Capital Medical University, Beijing, China. FAU - Liao, Qiuju AU - Liao Q AD - Department of Rheumatology & Allergy, Xuanwu Hospital, Capital Medical University, Beijing, China. FAU - Da, Yuwei AU - Da Y AD - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China. LA - eng PT - Journal Article DEP - 20200902 PL - Switzerland TA - Front Neurol JT - Frontiers in neurology JID - 101546899 PMC - PMC7492206 OTO - NOTNLM OT - autoimmune diseases OT - coexistence OT - myasthenia gravis OT - outcome OT - primary Sjögren's syndrome EDAT- 2020/09/29 06:00 MHDA- 2020/09/29 06:01 PMCR- 2020/09/02 CRDT- 2020/09/28 05:40 PHST- 2020/03/23 00:00 [received] PHST- 2020/07/20 00:00 [accepted] PHST- 2020/09/28 05:40 [entrez] PHST- 2020/09/29 06:00 [pubmed] PHST- 2020/09/29 06:01 [medline] PHST- 2020/09/02 00:00 [pmc-release] AID - 10.3389/fneur.2020.00939 [doi] PST - epublish SO - Front Neurol. 2020 Sep 2;11:939. doi: 10.3389/fneur.2020.00939. eCollection 2020. PMID- 32587654 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220415 IS - 1918-3003 (Print) IS - 1918-3011 (Electronic) IS - 1918-3003 (Linking) VI - 12 IP - 6 DP - 2020 Jun TI - Associations Between Cannabis Use, Abdominal Fat Phenotypes and Insulin Traits. PG - 377-388 LID - 10.14740/jocmr4165 [doi] AB - BACKGROUND: General obesity has been linked to dysregulation of the endocannabinoid system in humans. However, there is a lack of studies on the relationship between cannabis use and specific abdominal fat phenotypes. The aim was to investigate the associations between cannabis use and magnetic resonance imaging-derived fat phenotypes, as well as indices of insulin sensitivity and insulin secretion. METHODS: In this cross-sectional study, magnetic resonance imaging was used to quantify subcutaneous fat volume (SFV), visceral fat volume (VFV), intra-hepatic fat deposition (IHFD), intra-pancreatic fat deposition (IPFD) and skeletal muscle fat deposition (SMFD) by two independent observers. Insulin sensitivity was determined based on HOMA-IS, Raynaud index and Matsuda index, whereas insulin secretion was determined based on HOMA-β, insulinogenic index 30' and insulinogenic index 60'. A validated questionnaire was used to ascertain participants' cannabis use. Linear regression models were constructed, adjusting for demographics, glycated hemoglobin, physical activity, tobacco smoking and alcohol consumption. RESULTS: A total of 120 individuals were included. Cannabis use explained 9.2% of variance in IHFD, 4.4% in SMFD, 3.4% in VFV, 0.4% in SFV and 0.2% in IPFD. Regular cannabis users had significantly greater IHFD compared with never users, in both the unadjusted (P = 0.002) and all adjusted (P = 0.002; P = 0.008) analyses. The other fat phenotypes did not differ significantly between either regular or non-regular users compared with never users. Regular cannabis users had significantly greater insulin secretion (as defined by the insulinogenic index 60') compared with never users, in both the unadjusted (P = 0.049) and all adjusted (P = 0.003; P = 0.004) analyses. Cannabis use explained 20.3% of variance in the insulinogenic index 60', but was not significantly associated with the other indices of insulin secretion. There were no significant differences in indices of insulin sensitivity in either regular or non-regular cannabis users compared with never users. CONCLUSION: Regular cannabis use may be a risk factor for non-alcoholic fatty liver disease (but not IPFD) and may alter the neuromodulation of insulin secretion. Further investigations are now warranted to elucidate the mechanisms underlying these associations. CI - Copyright 2020, Stuart et al. FAU - Stuart, Charlotte E AU - Stuart CE AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Ko, Juyeon AU - Ko J AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Alarcon Ramos, Gisselle C AU - Alarcon Ramos GC AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Modesto, Andre E AU - Modesto AE AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Cho, Jaelim AU - Cho J AD - School of Medicine, University of Auckland, Auckland, New Zealand. FAU - Petrov, Maxim S AU - Petrov MS AD - School of Medicine, University of Auckland, Auckland, New Zealand. LA - eng PT - Journal Article DEP - 20200604 PL - Canada TA - J Clin Med Res JT - Journal of clinical medicine research JID - 101538301 PMC - PMC7295553 OTO - NOTNLM OT - Cannabis use OT - Insulin traits OT - Intra-hepatic fat OT - Intra-pancreatic fat OT - Magnetic resonance imaging OT - Skeletal muscle fat OT - Subcutaneous fat OT - Visceral fat COIS- None to declare. EDAT- 2020/06/27 06:00 MHDA- 2020/06/27 06:01 PMCR- 2020/06/04 CRDT- 2020/06/27 06:00 PHST- 2020/04/14 00:00 [received] PHST- 2020/05/04 00:00 [accepted] PHST- 2020/06/27 06:00 [entrez] PHST- 2020/06/27 06:00 [pubmed] PHST- 2020/06/27 06:01 [medline] PHST- 2020/06/04 00:00 [pmc-release] AID - 10.14740/jocmr4165 [doi] PST - ppublish SO - J Clin Med Res. 2020 Jun;12(6):377-388. doi: 10.14740/jocmr4165. Epub 2020 Jun 4. PMID- 21208200 OWN - NLM STAT- MEDLINE DCOM- 20151026 LR - 20191210 IS - 1399-0004 (Electronic) IS - 0009-9163 (Linking) VI - 79 IP - 3 DP - 2011 Mar TI - Twenty-five novel mutations including duplications in the ATP7A gene. PG - 243-53 LID - 10.1111/j.1399-0004.2010.01461.x [doi] AB - Twenty-five novel mutations including duplications in the ATP7A gene. Menkes disease (MD) and occipital horn syndrome (OHS) are allelic X-linked recessive copper deficiency disorders resulting from ATP7A gene mutations. MD is a severe condition leading to progressive neurological degeneration and death in early childhood, whereas OHS has a milder phenotype with mainly connective tissue abnormalities. Until now, molecular analyses have revealed only deletions and point mutations in both diseases. This study reports new molecular data in a series of 40 patients referred for either MD or OHS. We describe 23 point mutations (9 missense mutations, 7 splice site variants, 4 nonsense mutations, and 3 small insertions or deletions) and 7 intragenic deletions. Of these, 18 point mutations and 3 deletions are novel. Furthermore, our finding of four whole exon duplications enlarges the mutation spectrum in the ATP7A gene. ATP7A alterations were found in 85% of cases. Of these alterations, two thirds were point mutations and the remaining one third consisted of large rearrangements. We found that 66.6% of point mutations resulted in impaired ATP7A transcript splicing, a phenomenon more frequent than expected. This finding enabled us to confirm the pathogenic role of ATP7A mutations, particularly in missense and splice site variants. CI - © 2010 John Wiley & Sons A/S. FAU - Moizard, M-P AU - Moizard MP AD - CHRU de Tours, Service de Génétique, Tours, F-37044, France INSERM U930, Tours, F-37044, France CHU Hôpital Purpan, Service de Génétique médicale, Toulouse, F-31059, France CHU Hôpital d'Enfants Armand-Trousseau, AP-HP, Service de Génétique et Embryologie médicales, Paris, F-75571, France CHU Hôpital d'Enfants Armand-Trousseau, AP-HP, Service de Neuropédiatrie, Paris, F-75012, France Genetica Medica, Università di Pavia, Fondazione IRCCS S. Matteo, Pavia, I-27100, Italie Centre de Référence des Maladies Héréditaires du Métabolisme, INSERM U954. Hôpital d'Enfants, Vandoeuvre les Nancy, F-54511, France CHRU de Tours, Service de Neuropédiatrie, Tours, F-37044 France; Université François Rabelais Tours, F-37044, France. FAU - Ronce, N AU - Ronce N FAU - Blesson, S AU - Blesson S FAU - Bieth, E AU - Bieth E FAU - Burglen, L AU - Burglen L FAU - Mignot, C AU - Mignot C FAU - Mortemousque, I AU - Mortemousque I FAU - Marmin, N AU - Marmin N FAU - Dessay, B AU - Dessay B FAU - Danesino, C AU - Danesino C FAU - Feillet, F AU - Feillet F FAU - Castelnau, P AU - Castelnau P FAU - Toutain, A AU - Toutain A FAU - Moraine, C AU - Moraine C FAU - Raynaud, M AU - Raynaud M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Denmark TA - Clin Genet JT - Clinical genetics JID - 0253664 RN - 0 (Cation Transport Proteins) RN - 0 (RNA Splice Sites) RN - EC 3.6.1.- (Adenosine Triphosphatases) RN - EC 7.2.2.8 (ATP7A protein, human) RN - EC 7.2.2.8 (Copper-Transporting ATPases) RN - Occipital horn syndrome SB - IM MH - Adenosine Triphosphatases/*genetics MH - Cation Transport Proteins/*genetics MH - Copper-Transporting ATPases MH - Cutis Laxa/*genetics/pathology MH - Ehlers-Danlos Syndrome/*genetics/pathology MH - Exons/genetics MH - Female MH - Gene Duplication/*genetics MH - Gene Expression Profiling MH - Gene Rearrangement/genetics MH - Humans MH - Male MH - Menkes Kinky Hair Syndrome/*genetics/pathology MH - Multiplex Polymerase Chain Reaction MH - Mutation, Missense/genetics MH - Point Mutation/*genetics MH - RNA Splice Sites/genetics MH - Sequence Deletion/*genetics EDAT- 2011/01/07 06:00 MHDA- 2015/10/27 06:00 CRDT- 2011/01/07 06:00 PHST- 2011/01/07 06:00 [entrez] PHST- 2011/01/07 06:00 [pubmed] PHST- 2015/10/27 06:00 [medline] AID - 10.1111/j.1399-0004.2010.01461.x [doi] PST - ppublish SO - Clin Genet. 2011 Mar;79(3):243-53. doi: 10.1111/j.1399-0004.2010.01461.x. PMID- 19785596 OWN - NLM STAT- MEDLINE DCOM- 20100524 LR - 20100406 IS - 1365-2133 (Electronic) IS - 0007-0963 (Linking) VI - 162 IP - 1 DP - 2010 Jan TI - Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patients. PG - 91-101 LID - 10.1111/j.1365-2133.2009.09472.x [doi] AB - Background Lupus erythematosus (LE) is a chronic, autoimmune disease resulting from an interaction of genetic, environmental and hormonal factors and characterized by a spectrum of clinical forms with variable evolution from a localized cutaneous form to a life-threatening systemic form. Objective To analyse and compare the prevalence and characteristics of the main clinical and immunological manifestations of subacute cutaneous LE (SCLE) and chronic CLE (CCLE). Methods A total of 270 patients with CLE (112 patients with SCLE and 158 patients with CCLE) were studied retrospectively. The clinical and serological characteristics of all the patients were collected in a chart review. Results The patients with SCLE had a higher prevalence of annular and papulosquamous lesions, Raynaud phenomenon, mucous membrane ulcers, malar rashes, photosensitivity, vasculitis and a lower frequency of discoid lesions and alopecia compared with patients with CCLE. Patients with SCLE had a higher prevalence of arthralgias (P < 0.001), xerophthalmia (P = 0.045), arthritis (P < 0.001), nephropathy (P = 0.003) and systemic LE (SLE) (P < 0.001) compared with patients with CCLE. Patients with SCLE also had a higher frequency of laboratory and serological abnormalities than patients with CCLE. Generalized discoid LE (DLE) was associated with a higher prevalence of photosensitivity (P < 0.001), panniculitis (P = 0.009) and SLE (P = 0.003) than localized DLE. In patients with SCLE and those with CCLE, photosensitivity, arthralgias, arthritis, nephropathy and xerophthalmia were associated with SLE. In patients with SCLE, significant correlations existed between clinical and immunological data. Conclusions In our series, differences in the expression of CCLE and SCLE were found with respect to the distribution and type of lesions, systemic features and immunological findings. FAU - Vera-Recabarren, M A AU - Vera-Recabarren MA AD - Department of Dermatology, Hospital Clínic, University of Barcelona, Villarroel 170, 08036 Barcelona, Catalonia, Spain. mavera@aedv.es FAU - García-Carrasco, M AU - García-Carrasco M FAU - Ramos-Casals, M AU - Ramos-Casals M FAU - Herrero, C AU - Herrero C LA - eng PT - Journal Article DEP - 20090928 PL - England TA - Br J Dermatol JT - The British journal of dermatology JID - 0004041 RN - 0 (Autoantibodies) RN - 9009-79-4 (Rheumatoid Factor) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Alopecia/epidemiology MH - Arthralgia/epidemiology MH - Arthritis/epidemiology MH - Autoantibodies/immunology MH - Blood Sedimentation MH - Child MH - Female MH - Humans MH - Kidney Diseases/epidemiology MH - *Lupus Erythematosus, Cutaneous/complications/immunology/pathology MH - *Lupus Erythematosus, Discoid/complications/immunology/pathology MH - Lupus Erythematosus, Systemic/epidemiology MH - Male MH - Middle Aged MH - Panniculitis/epidemiology MH - Photosensitivity Disorders/epidemiology MH - Retrospective Studies MH - Rheumatoid Factor/analysis MH - Vasculitis/epidemiology MH - Xerophthalmia/epidemiology MH - Young Adult EDAT- 2009/09/30 06:00 MHDA- 2010/05/25 06:00 CRDT- 2009/09/30 06:00 PHST- 2009/09/30 06:00 [entrez] PHST- 2009/09/30 06:00 [pubmed] PHST- 2010/05/25 06:00 [medline] AID - BJD9472 [pii] AID - 10.1111/j.1365-2133.2009.09472.x [doi] PST - ppublish SO - Br J Dermatol. 2010 Jan;162(1):91-101. doi: 10.1111/j.1365-2133.2009.09472.x. Epub 2009 Sep 28. PMID- 32781106 OWN - NLM STAT- MEDLINE DCOM- 20210621 LR - 20210621 IS - 1523-1755 (Electronic) IS - 0085-2538 (Linking) VI - 99 IP - 1 DP - 2021 Jan TI - Trajectories of glomerular filtration rate and progression to end stage kidney disease after kidney transplantation. PG - 186-197 LID - S0085-2538(20)30937-6 [pii] LID - 10.1016/j.kint.2020.07.025 [doi] AB - Although the gold standard of monitoring kidney transplant function relies on glomerular filtration rate (GFR), little is known about GFR trajectories after transplantation, their determinants, and their association with outcomes. To evaluate these parameters we examined kidney transplant recipients receiving care at 15 academic centers. Patients underwent prospective monitoring of estimated GFR (eGFR) measurements, with assessment of clinical, functional, histological and immunological parameters. Additional validation took place in seven randomized controlled trials that included a total of 14,132 patients with 403,497 eGFR measurements. After a median follow-up of 6.5 years, 1,688 patients developed end-stage kidney disease. Using unsupervised latent class mixed models, we identified eight distinct eGFR trajectories. Multinomial regression models identified seven significant determinants of eGFR trajectories including donor age, eGFR, proteinuria, and several significant histological features: graft scarring, graft interstitial inflammation and tubulitis, microcirculation inflammation, and circulating anti-HLA donor specific antibodies. The eGFR trajectories were associated with progression to end stage kidney disease. These trajectories, their determinants and respective associations with end stage kidney disease were similar across cohorts, as well as in diverse clinical scenarios, therapeutic eras and in the seven randomized control trials. Thus, our results provide the basis for a trajectory-based assessment of kidney transplant patients for risk stratification and monitoring. CI - Copyright © 2020 International Society of Nephrology. Published by Elsevier Inc. All rights reserved. FAU - Raynaud, Marc AU - Raynaud M AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France. FAU - Aubert, Olivier AU - Aubert O AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Reese, Peter P AU - Reese PP AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Renal Electrolyte and Hypertension Division, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA. FAU - Bouatou, Yassine AU - Bouatou Y AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France. FAU - Naesens, Maarten AU - Naesens M AD - Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium. FAU - Kamar, Nassim AU - Kamar N AD - Université Paul Sabatier, INSERM, Department of Nephrology and Organ Transplantation, CHU Rangueil & Purpan, Toulouse, France. FAU - Bailly, Élodie AU - Bailly É AD - Nephrology and Immunology Department, Bretonneau Hospital, Tours, France. FAU - Giral, Magali AU - Giral M AD - Department of Nephrology, Centre Hospitalier Universitaire, Nantes, France. FAU - Ladrière, Marc AU - Ladrière M AD - Nephrology Dialysis Transplantation Department, University of Lorraine, Centre Hospitalier Universitaire, Nancy, France. FAU - Le Quintrec, Moglie AU - Le Quintrec M AD - Department of Nephrology, Centre Hospitalier Universitaire, Montpellier, France. FAU - Delahousse, Michel AU - Delahousse M AD - Department of Transplantation, Nephrology and Clinical Immunology, Foch Hospital, Suresnes, France. FAU - Juric, Ivana AU - Juric I AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Basic-Jukic, Nikolina AU - Basic-Jukic N AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Gupta, Gaurav AU - Gupta G AD - Division of Nephrology, Department of Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, Virginia, USA. FAU - Akalin, Enver AU - Akalin E AD - Albert Einstein College of Medicine, Renal Division Montefiore Medical Centre, Kidney Transplantation Program, Bronx, New York, USA. FAU - Yoo, Daniel AU - Yoo D AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France. FAU - Chin, Chen-Shan AU - Chin CS AD - Deep Learning in Medicine and Genomics, DNAnexus, San Francisco, California, USA. FAU - Proust-Lima, Cécile AU - Proust-Lima C AD - Population Health Research Centre, INSERM U1219, Bordeaux, France. FAU - Böhmig, Georg AU - Böhmig G AD - Division of Nephrology and Dialysis, Department of Medicine III, General Hospital Vienna, Vienna, Austria. FAU - Oberbauer, Rainer AU - Oberbauer R AD - Medical University of Vienna, Nephrology, Vienna, Austria. FAU - Stegall, Mark D AU - Stegall MD AD - William J. von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, Minnesota, USA. FAU - Bentall, Andrew J AU - Bentall AJ AD - William J. von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, Minnesota, USA. FAU - Jordan, Stanley C AU - Jordan SC AD - Department of Medicine, Division of Nephrology, Comprehensive Transplant Centre, Cedars Sinai Medical Centre, Los Angeles, California, USA. FAU - Huang, Edmund AU - Huang E AD - Department of Medicine, Division of Nephrology, Comprehensive Transplant Centre, Cedars Sinai Medical Centre, Los Angeles, California, USA. FAU - Glotz, Denis AU - Glotz D AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Legendre, Christophe AU - Legendre C AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Montgomery, Robert A AU - Montgomery RA AD - New York University Langone Transplant Institute, New York, New York, USA. FAU - Segev, Dorry L AU - Segev DL AD - Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. FAU - Empana, Jean-Philippe AU - Empana JP AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France. FAU - Grams, Morgan E AU - Grams ME AD - Johns Hopkins Medical Institutions, George W. Comstock Centre for Public Health Research and Prevention, Baltimore, Maryland, USA. FAU - Coresh, Josef AU - Coresh J AD - Johns Hopkins Medical Institutions, George W. Comstock Centre for Public Health Research and Prevention, Baltimore, Maryland, USA. FAU - Jouven, Xavier AU - Jouven X AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AD - Université de Paris, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. Electronic address: alexandreloupy@inserm.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200808 PL - United States TA - Kidney Int JT - Kidney international JID - 0323470 SB - IM CIN - Kidney Int. 2021 Jan;99(1):24-26. doi: 10.1016/j.kint.2020.07.031. PMID: 33390230 MH - Glomerular Filtration Rate MH - Humans MH - *Kidney Failure, Chronic/diagnosis/surgery MH - *Kidney Transplantation/adverse effects MH - Prospective Studies OTO - NOTNLM OT - end-stage renal disease OT - glomerular filtration rate OT - kidney function OT - kidney transplantation OT - mortality OT - trajectories EDAT- 2020/08/12 06:00 MHDA- 2021/06/22 06:00 CRDT- 2020/08/12 06:00 PHST- 2020/02/19 00:00 [received] PHST- 2020/06/09 00:00 [revised] PHST- 2020/07/02 00:00 [accepted] PHST- 2020/08/12 06:00 [pubmed] PHST- 2021/06/22 06:00 [medline] PHST- 2020/08/12 06:00 [entrez] AID - S0085-2538(20)30937-6 [pii] AID - 10.1016/j.kint.2020.07.025 [doi] PST - ppublish SO - Kidney Int. 2021 Jan;99(1):186-197. doi: 10.1016/j.kint.2020.07.025. Epub 2020 Aug 8. PMID- 21391226 OWN - NLM STAT- MEDLINE DCOM- 20110829 LR - 20211020 IS - 1527-3350 (Electronic) IS - 0270-9139 (Print) IS - 0270-9139 (Linking) VI - 53 IP - 6 DP - 2011 Jun TI - A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. PG - 1959-66 LID - 10.1002/hep.24292 [doi] AB - Ductal plate malformations (DPMs) are developmental anomalies considered to result from lack of ductal plate remodeling during bile duct morphogenesis. In mice, bile duct development is initiated by the formation of primitive ductal structures lined by two cell types, namely ductal plate cells and hepatoblasts. During ductal plate remodeling, the primitive ductal structures mature to ducts as a result from differentiation of the ductal plate cells and hepatoblasts to cholangiocytes. Here, we report this process is conserved in human fetal liver. These findings prompted us to evaluate how DPMs develop in three mouse models, namely mice with livers deficient in hepatocyte nuclear factor 6 (HNF6), HNF1β, or cystin-1 (cpk [congenital polycystic kidney] mice). Human liver from a patient with a HNF1B/TCF2 mutation, and from fetuses affected with autosomal recessive polycystic kidney disease (ARPKD) were also analyzed. Despite the epistatic relationship between HNF6, HNF1β, and cystin-1, the three mouse models displayed distinct morphogenic mechanisms of DPM. They all developed biliary cysts lined by cells with abnormal apicobasal polarity. However, the absence of HNF6 led to an early defect in ductal plate cell differentiation. In HNF1β-deficient liver, maturation of the primitive ductal structures was impaired. Normal differentiation and maturation but abnormal duct expansion was apparent in cpk mouse livers and in human fetal ARPKD. CONCLUSION: DPM is the common endpoint of distinct defects initiated at distinct stages of bile duct morphogenesis. Our observations provide a new pathogenic classification of DPM. CI - Copyright © 2011 American Association for the Study of Liver Diseases. FAU - Raynaud, Peggy AU - Raynaud P AD - de Duve Institute, Université Catholique de Louvain, Brussels, Belgium. FAU - Tate, Joshua AU - Tate J FAU - Callens, Céline AU - Callens C FAU - Cordi, Sabine AU - Cordi S FAU - Vandersmissen, Patrick AU - Vandersmissen P FAU - Carpentier, Rodolphe AU - Carpentier R FAU - Sempoux, Christine AU - Sempoux C FAU - Devuyst, Olivier AU - Devuyst O FAU - Pierreux, Christophe E AU - Pierreux CE FAU - Courtoy, Pierre AU - Courtoy P FAU - Dahan, Karin AU - Dahan K FAU - Delbecque, Katty AU - Delbecque K FAU - Lepreux, Sébastien AU - Lepreux S FAU - Pontoglio, Marco AU - Pontoglio M FAU - Guay-Woodford, Lisa M AU - Guay-Woodford LM FAU - Lemaigre, Frédéric P AU - Lemaigre FP LA - eng GR - P30 DK074038/DK/NIDDK NIH HHS/United States GR - R01 DK055534/DK/NIDDK NIH HHS/United States GR - DK55534/DK/NIDDK NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20110502 PL - United States TA - Hepatology JT - Hepatology (Baltimore, Md.) JID - 8302946 RN - 0 (Biomarkers) RN - 0 (Cys1 protein, mouse) RN - 0 (HNF1B protein, human) RN - 0 (Hepatocyte Nuclear Factor 6) RN - 0 (Hnf1b protein, mouse) RN - 0 (Membrane Proteins) RN - 0 (Onecut1 protein, mouse) RN - 138674-15-4 (Hepatocyte Nuclear Factor 1-beta) SB - IM CIN - Hepatology. 2011 Jun;53(6):1795-7. doi: 10.1002/hep.24404. PMID: 21557279 MH - Animals MH - Bile Ducts, Intrahepatic/*abnormalities/*embryology/pathology MH - Biomarkers/metabolism MH - Cell Differentiation/physiology MH - Congenital Abnormalities/*classification/*etiology/physiopathology MH - Disease Models, Animal MH - Hepatocyte Nuclear Factor 1-beta/metabolism MH - Hepatocyte Nuclear Factor 6/metabolism MH - Humans MH - Liver/embryology/metabolism/pathology MH - Membrane Proteins/metabolism MH - Mice MH - Mice, Mutant Strains MH - Morphogenesis/*physiology MH - Polycystic Kidney Diseases/congenital/metabolism/physiopathology MH - Polycystic Kidney, Autosomal Recessive/metabolism/physiopathology PMC - PMC4271518 MID - NIHMS277404 EDAT- 2011/03/11 06:00 MHDA- 2011/08/30 06:00 PMCR- 2014/12/19 CRDT- 2011/03/11 06:00 PHST- 2010/09/11 00:00 [received] PHST- 2011/02/24 00:00 [accepted] PHST- 2011/03/11 06:00 [entrez] PHST- 2011/03/11 06:00 [pubmed] PHST- 2011/08/30 06:00 [medline] PHST- 2014/12/19 00:00 [pmc-release] AID - 10.1002/hep.24292 [doi] PST - ppublish SO - Hepatology. 2011 Jun;53(6):1959-66. doi: 10.1002/hep.24292. Epub 2011 May 2. PMID- 40420438 OWN - NLM STAT- MEDLINE DCOM- 20251230 LR - 20260114 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 77 IP - 12 DP - 2025 Dec TI - Peripheral Blood Gene Expression Profiling and Prognostic Significance for the Course of Interstitial Lung Disease in Patients With Systemic Sclerosis. PG - 1749-1756 LID - 10.1002/art.43262 [doi] AB - OBJECTIVE: We used data from the placebo arm of the Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial to determine the prognostic/predictive significance of peripheral blood cell (PBC) transcript modules for the course of forced vital capacity (FVC) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) with and without mycophenolate mofetil (MMF) treatment. METHODS: Patients had SSc-ILD with first non-Raynaud symptom within 7 years. MMF treatment was permitted if taken at a stable dose for ≥6 months. PBC RNA samples were taken at baseline. Global RNA sequencing was performed, followed by a modular analysis using 62 curated whole blood modules. The prognostic significance of baseline composite modular scores for decline in FVC% predicted at week 52 was analyzed using mixed models for repeated measures. RESULTS: Among patients taking MMF (n = 120), higher baseline lymphoid lineage and mitochondrial/protein synthesis modules were associated with a better course of FVC% predicted, whereas higher baseline myeloid lineage and inflammation modules were associated with a faster decline in FVC% predicted. Among patients not taking MMF (n = 118), only myeloid lineage and inflammation modules were associated with a faster decline in FVC% predicted. CONCLUSION: Among patients with SSc-ILD in the SENSCIS trial, PBC modules involved in myeloid lineage were associated with a faster decline in FVC regardless of MMF treatment. Higher baseline lymphoid, protein synthesis, and mitochondrial module scores were associated with a better course of SSc-ILD among patients receiving MMF treatment. Blood gene expression profiles might be useful prognostic/predictive biomarkers in patients with SSc-ILD. CI - © 2025 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. FAU - Assassi, Shervin AU - Assassi S AUID- ORCID: 0000-0002-8059-9978 AD - University of Texas Health Science Center at Houston. FAU - Denton, Christopher P AU - Denton CP AUID- ORCID: 0000-0003-3975-8938 AD - University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom. FAU - Zwick, Matthias AU - Zwick M AD - Boehringer Ingelheim Pharma GmbH & Co KG, Biberach, Germany. FAU - Schmid, Ramona AU - Schmid R AD - Boehringer Ingelheim Pharma GmbH & Co KG, Biberach, Germany. FAU - Ittrich, Carina AU - Ittrich C AD - Boehringer Ingelheim Pharma GmbH & Co KG, Biberach, Germany. FAU - Litzenburger, Tobias AU - Litzenburger T AD - Boehringer Ingelheim Pharma GmbH & Co KG, Biberach, Germany. FAU - Visvanathan, Sudha AU - Visvanathan S AD - Boehringer Ingelheim Pharmaceuticals, Inc, Ridgefield, Connecticut. LA - eng GR - Boehringer Ingelheim/ PT - Clinical Trial, Phase III PT - Journal Article PT - Randomized Controlled Trial DEP - 20250729 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Immunosuppressive Agents) RN - 0 (Indoles) RN - HU9DX48N0T (Mycophenolic Acid) RN - G6HRD2P839 (nintedanib) SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Gene Expression Profiling MH - Immunosuppressive Agents/therapeutic use MH - Indoles/therapeutic use MH - *Lung Diseases, Interstitial/drug therapy/genetics/etiology/blood/physiopathology MH - Mycophenolic Acid/therapeutic use MH - Prognosis MH - *Scleroderma, Systemic/complications/drug therapy/genetics/blood MH - Vital Capacity PMC - PMC12750129 EDAT- 2025/05/27 06:27 MHDA- 2025/12/30 13:08 PMCR- 2025/12/30 CRDT- 2025/05/27 01:12 PHST- 2025/04/14 00:00 [revised] PHST- 2024/09/23 00:00 [received] PHST- 2025/05/16 00:00 [accepted] PHST- 2025/12/30 13:08 [medline] PHST- 2025/05/27 06:27 [pubmed] PHST- 2025/05/27 01:12 [entrez] PHST- 2025/12/30 00:00 [pmc-release] AID - ART43262 [pii] AID - 10.1002/art.43262 [doi] PST - ppublish SO - Arthritis Rheumatol. 2025 Dec;77(12):1749-1756. doi: 10.1002/art.43262. Epub 2025 Jul 29. PMID- 28586844 OWN - NLM STAT- MEDLINE DCOM- 20170822 LR - 20220409 IS - 2168-6157 (Electronic) IS - 2168-6149 (Print) IS - 2168-6149 (Linking) VI - 74 IP - 8 DP - 2017 Aug 1 TI - Skeletal Muscle Involvement in Antisynthetase Syndrome. PG - 992-999 LID - 10.1001/jamaneurol.2017.0934 [doi] AB - IMPORTANCE: Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients' serum. However, antisynthetase syndrome is not included in the histological subsets of idiopathic inflammatory myopathies. OBJECTIVE: To elucidate the clinical features of myositis in patients with antisynthetase syndrome. DESIGN, SETTING, AND PARTICIPANTS: In this cohort study, muscle biopsy and blood samples were collected from 460 patients with idiopathic inflammatory myositis from various regional referral centers throughout Japan between October 2010 and December 2014. Data were analyzed in March 2016. EXPOSURES: Six different anti-ARS antibodies were detected in serum by RNA immunoprecipitation. Line blot assay and protein immunoprecipitation were also performed. HLA-DRB1 alleles were genotyped. MAIN OUTCOMES AND MEASURES: The main outcomes were muscle manifestations and histological findings. Predisposing factors, extramuscular symptoms, and follow-up information were also studied. RESULTS: Of 460 patients with idiopathic inflammatory myopathies, 51 (11.1%) had anti-ARS antibodies. Of this subset, 31 (61%) were women, with a mean (SD) age at disease onset of 60.2 (16.1) years. Among 6 different anti-ARS antibodies, only 1-the anti-OJ antibody-was not detected by line blot assay but by RNA immunoprecipitation. There were no significant HLA-DRB1 alleles associated with anti-ARS antibodies. All 51 patients presented with muscle limb weakness; 14 (27%) had severe limb weakness, 17 (33%) had neck muscle weakness, 15 (29%) had dysphagia, and 15 (29%) had muscle atrophy. Although patients with anti-OJ antibodies showed severe muscle weakness, the clinical presentations of antisynthetase syndrome were relatively homogeneous. In histology, perifascicular necrosis, the characteristic finding of antisynthetase syndrome, was found in 24 patients (47%). Myositis with anti-ARS antibodies responded to the combination of immunosuppressive therapy with favorable outcomes. Interstitial lung disease, found in 41 patients (80%), was more closely associated with mortality than myositis. CONCLUSIONS AND RELEVANCE: Although clinical presentations of antisynthetase syndrome were relatively homogeneous, anti-OJ antibodies were associated with severe muscle involvement. Antisynthetase syndrome is a clinical and histological subset among idiopathic inflammatory myopathies. FAU - Noguchi, Eri AU - Noguchi E AD - Department of Neurology, Keio University School of Medicine, Tokyo, Japan. FAU - Uruha, Akinori AU - Uruha A AD - Department of Neuromuscular Research, National Institute of Neuroscience, and Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, Tokyo, Japan. AD - Pierre and Marie Curie University-Paris VI (UPMC), National Institute of Health and Medical Research (INSERM), Mixed Research Unit (UMR) 974, Center of Research in Myology, Institute of Myology, Pitié-Salpêtrière University Hospital, Paris, France. FAU - Suzuki, Shigeaki AU - Suzuki S AD - Department of Neurology, Keio University School of Medicine, Tokyo, Japan. FAU - Hamanaka, Kohei AU - Hamanaka K AD - Department of Neuromuscular Research, National Institute of Neuroscience, and Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, Tokyo, Japan. FAU - Ohnuki, Yuko AU - Ohnuki Y AD - Department of Molecular Life Science, Basic Medical Science and Molecular Medicine, Tokai University School of Medicine, Isehara-shi, Kanagawa, Japan. FAU - Tsugawa, Jun AU - Tsugawa J AD - Department of Neurology, Fukuoka University School of Medicine, Fukuoka, Japan. FAU - Watanabe, Yurika AU - Watanabe Y AD - Department of Neurology, Keio University School of Medicine, Tokyo, Japan. FAU - Nakahara, Jin AU - Nakahara J AD - Department of Neurology, Keio University School of Medicine, Tokyo, Japan. FAU - Shiina, Takashi AU - Shiina T AD - Department of Molecular Life Science, Basic Medical Science and Molecular Medicine, Tokai University School of Medicine, Isehara-shi, Kanagawa, Japan. FAU - Suzuki, Norihiro AU - Suzuki N AD - Department of Neurology, Keio University School of Medicine, Tokyo, Japan. FAU - Nishino, Ichizo AU - Nishino I AD - Department of Neuromuscular Research, National Institute of Neuroscience, and Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, Tokyo, Japan. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - JAMA Neurol JT - JAMA neurology JID - 101589536 RN - 0 (Autoantibodies) RN - 0 (HLA-DRB1 Chains) RN - EC 6.1.1.- (Amino Acyl-tRNA Synthetases) RN - Antisynthetase syndrome SB - IM CIN - JAMA Neurol. 2018 Feb 1;75(2):258-259. doi: 10.1001/jamaneurol.2017.3872. PMID: 29255888 CIN - JAMA Neurol. 2018 Feb 1;75(2):259-260. doi: 10.1001/jamaneurol.2017.3875. PMID: 29255891 MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Amino Acyl-tRNA Synthetases/immunology MH - Autoantibodies/blood MH - Cohort Studies MH - Electromyography MH - Female MH - Genotype MH - HLA-DRB1 Chains/genetics MH - Humans MH - Immunoprecipitation MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Muscle, Skeletal/diagnostic imaging/*metabolism/physiopathology MH - Myositis/*complications/genetics/immunology/pathology MH - Statistics, Nonparametric MH - Young Adult PMC - PMC5710328 COIS- Conflict of Interest Disclosures: None reported. EDAT- 2017/06/07 06:00 MHDA- 2017/08/23 06:00 PMCR- 2018/08/14 CRDT- 2017/06/07 06:00 PHST- 2017/06/07 06:00 [pubmed] PHST- 2017/08/23 06:00 [medline] PHST- 2017/06/07 06:00 [entrez] PHST- 2018/08/14 00:00 [pmc-release] AID - 2629722 [pii] AID - noi170028 [pii] AID - 10.1001/jamaneurol.2017.0934 [doi] PST - ppublish SO - JAMA Neurol. 2017 Aug 1;74(8):992-999. doi: 10.1001/jamaneurol.2017.0934. PMID- 27371996 OWN - NLM STAT- MEDLINE DCOM- 20171109 LR - 20211204 IS - 1532-866X (Electronic) IS - 0049-0172 (Print) IS - 0049-0172 (Linking) VI - 46 IP - 3 DP - 2016 Dec TI - Calcinosis is associated with digital ulcers and osteoporosis in patients with systemic sclerosis: A Scleroderma Clinical Trials Consortium study. PG - 344-349 LID - S0049-0172(16)30060-9 [pii] LID - 10.1016/j.semarthrit.2016.05.008 [doi] AB - OBJECTIVES: We sought to identify the clinical factors associated with calcinosis in an international multicenter collaborative effort with the Scleroderma Clinical Trials Consortium (SCTC). METHODS: This is a retrospective cohort study of 5218 patients with systemic sclerosis (SSc). Logistic regression was used to obtain odds ratios (OR) relating calcinosis to various clinical features in multivariate analyses. RESULTS: A total of 1290 patients (24.7%) had calcinosis. In univariate analyses, patients with calcinosis were older than patients without calcinosis, more likely to be female, and had longer disease duration from the first non-Raynaud phenomenon symptom. Patients with calcinosis were more likely to have digital ulcers, telangiectasias, acro-osteolysis, cardiac disease, pulmonary hypertension, gastrointestinal involvement, arthritis, and osteoporosis, but less likely to have muscle disease. Anti-Scl-70, RNA-polymerase-III, and U1-RNP autoantibodies were significantly less common in patients with calcinosis, while anticentromere (ACA), anti-PM/Scl, and anticardiolipin antibodies were more frequent. In multivariate analysis, the strongest associations with calcinosis were digital ulcers (OR = 3.9; 95% CI: 2.7-5.5; p < 0.0001) and osteoporosis (OR = 4.2; 95% CI: 2.3-7.9; p < 0.0001). CONCLUSION: One quarter of patients with SSc have calcinosis at some time during their illness. Our data confirm a strong association of calcinosis with digital ulcers, and support a novel association with osteoporosis. CI - Copyright © 2016 Elsevier Inc. All rights reserved. FAU - Valenzuela, Antonia AU - Valenzuela A AD - Department of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, Suite 203 MC 5755, Palo Alto, CA 94304. FAU - Baron, Murray AU - Baron M AD - Department of Rheumatology, Jewish General Hospital McGill University, Montreal, Canada. CN - Canadian Scleroderma Research Group FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Proudman, Susanna AU - Proudman S AD - Rheumatology Unit, Royal Adelaide Hospital, North Terrace, Australia; Discipline of Medicine, University of Adelaide, Adelaide, Australia; Department of Rheumatology, St. Vincent׳s Hospital Melbourne, Fitzroy, Australia. FAU - Stevens, Wendy AU - Stevens W AD - Discipline of Medicine, University of Adelaide, Adelaide, Australia; Department of Rheumatology, St. Vincent׳s Hospital Melbourne, Fitzroy, Australia. CN - Australian Scleroderma Interest Group FAU - Rodriguez-Reyna, Tatiana S AU - Rodriguez-Reyna TS AD - Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, DF, Mexico, Mexico. FAU - Vacca, Alessandra AU - Vacca A AD - Unit and Chair of Rheumatology, University Hospital of Cagliari, Cagliari, Italy. FAU - Medsger, Thomas A Jr AU - Medsger TA Jr AD - Department of Medicine/Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, PA. FAU - Hinchcliff, Monique AU - Hinchcliff M AD - Department of Rheumatology, Northwestern University, Chicago, IL. FAU - Hsu, Vivien AU - Hsu V AD - Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ. FAU - Wu, Joy Y AU - Wu JY AD - Department of Endocrinology, Stanford University School of Medicine, Palo Alto, CA. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Dermatology, Stanford University School of Medicine, Palo Alto, CA. FAU - Chung, Lorinda AU - Chung L AD - Department of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, Suite 203 MC 5755, Palo Alto, CA 94304. Electronic address: shauwei@stanford.edu. LA - eng GR - K23 AR059763/AR/NIAMS NIH HHS/United States PT - Journal Article DEP - 20160602 PL - United States TA - Semin Arthritis Rheum JT - Seminars in arthritis and rheumatism JID - 1306053 RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) RN - 0 (Nuclear Proteins) RN - 0 (Ribonucleoprotein, U1 Small Nuclear) RN - 0 (Scl 70 antigen, human) RN - 0 (anticentromere antibody) RN - EC 2.7.7.6 (RNA Polymerase III) RN - EC 5.99.1.2 (DNA Topoisomerases, Type I) SB - IM MH - Acro-Osteolysis/epidemiology MH - Adult MH - Aged MH - Antibodies, Antinuclear/immunology MH - Autoantibodies/immunology MH - Calcinosis/*epidemiology/immunology MH - Cohort Studies MH - DNA Topoisomerases, Type I MH - Female MH - *Fingers MH - Heart Diseases/epidemiology MH - Humans MH - Hypertension, Pulmonary/epidemiology MH - Logistic Models MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Nuclear Proteins/immunology MH - Odds Ratio MH - Osteoporosis/*epidemiology MH - RNA Polymerase III/immunology MH - Retrospective Studies MH - Ribonucleoprotein, U1 Small Nuclear/immunology MH - Scleroderma, Systemic/*epidemiology/immunology MH - Skin Ulcer/*epidemiology/immunology MH - Telangiectasis/epidemiology PMC - PMC5500288 MID - NIHMS858649 OTO - NOTNLM OT - Calcinosis OT - Digital ulcers OT - Osteoporosis OT - Systemic sclerosis EDAT- 2016/07/04 06:00 MHDA- 2017/11/10 06:00 PMCR- 2017/12/01 CRDT- 2016/07/04 06:00 PHST- 2016/02/02 00:00 [received] PHST- 2016/05/20 00:00 [revised] PHST- 2016/05/27 00:00 [accepted] PHST- 2016/07/04 06:00 [pubmed] PHST- 2017/11/10 06:00 [medline] PHST- 2016/07/04 06:00 [entrez] PHST- 2017/12/01 00:00 [pmc-release] AID - S0049-0172(16)30060-9 [pii] AID - 10.1016/j.semarthrit.2016.05.008 [doi] PST - ppublish SO - Semin Arthritis Rheum. 2016 Dec;46(3):344-349. doi: 10.1016/j.semarthrit.2016.05.008. Epub 2016 Jun 2. PMID- 41790634 OWN - NLM STAT- In-Process DCOM- 20260307 LR - 20260609 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 105 IP - 10 DP - 2026 Mar 6 TI - A retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease. PG - e47876 LID - 10.1097/MD.0000000000047876 [doi] LID - e47876 AB - Systemic sclerosis (SSc) is a rare, complex, chronic, progressive, severe, often life-threatening, fibrosing, heterogeneous autoimmune connective tissue disease with immune dysfunction and limited treatment options. Interstitial lung disease (ILD) is common in SSc. To study the clinical characteristics of systemic sclerosis-associated interstitial lung disease (SSc-ILD) will provide evidence for the early diagnosis, treatment decision-making, and prognosis of SSc-ILD patients. One hundred and seventy-five patients with SSc were retrospectively enrolled and divided into SSc-ILD group and SSc-non-ILD group. The general clinical characteristics, autoantibodies, laboratory test, thorax high-resolution computed tomography (HRCT), pulmonary function testing, stratification, and treatment options were compared between the 2 groups. The risk factors of SSc-ILD were also analyzed. One hundred and twenty-four SSc-ILD patients (70.9%) and 51 SSc-non-ILD patients (29.1%) were in 175 SSc patients. Raynaud phenomenon, skin swelling, and joint involvement were the top 3 initial symptoms. Sixty-seven SSc-ILD patients (54.1%) showed new onset or progression of ILD on HRCT, which were heterogeneous. The significantly increased indicators in SSc-ILD group were gastrointestinal involvement (P = .003), antinuclear antibody (ANA) (P = .006), anti-Scl-70 (P <.001), anti-SSA (P = .008), and rheumatoid factor (P = .04), erythrocyte sedimentation rate (ESR) (P = .005), CRP (P = .000), IL-6 (P = .001), globulin (P = .006), IgG (P = .002), IgA (P = .001), while the decreased indicators were mean corpusular hemoglobin concerntration (P = .036), albumin (P = .046), liver dysfunction (P = .018), and FVC% (P = .006). Positive anti-Scl-70 antibody and decreased FVC% were independent risk factors for SSc-ILD. The incidence of ILD occurrence and progression was significantly higher in patients with anti-Scl-70 antibody, diffuse cutaneous systemic sclerosis (dcSSc) plus anti-Scl-70 antibody, ESR ≥50 mm/h, and CRP ≥5.00 mg/L (P <.05). Based on the predictive factors (gastrointestinal involvement, joint involvement, positive anti-Scl-70 antibody, liver dysfunction, duration of disease, FVC%, mean corpusular hemoglobin concerntration, ESR, CRP, IgG, IgA, globulin, and albumin), independent risk factors (anti-Scl-70 antibody and decreased FVC%), clinical phenotypic and autoantibody stratification, HRCT heterogeneity, and atypical initial symptoms, it may be seized the "window of opportunity" with early diagnosis may exist for preventing the irreversible progression of pulmonary fibrosis of SSc-ILD patients. CI - Copyright © 2026 the Author(s). Published by Wolters Kluwer Health, Inc. FAU - Qi, Xin AU - Qi X AUID- ORCID: 0000-0002-3669-998 AD - Department of Rheumatology, Qilu Hospital, Shandong University, Jinan, Shandong Province, China. AD - Department of Rheumatology, Jinan Fourth People's Hospital, Jinan, Shandong Province, China. FAU - Chen, Xiangzhi AU - Chen X AD - Department of Rheumatology, Qilu Hospital, Shandong University, Jinan, Shandong Province, China. FAU - Dai, Xinyue AU - Dai X AD - Department of Nephrology, Northeast International Hospital Houde Hospital, Shenyang, Liaoning Province, China. FAU - Xie, Xiaowen AU - Xie X AD - Department of Rheumatology, Weifang People's Hospital, Weifang, Shandong Province, China. FAU - Liu, Huaxiang AU - Liu H AD - Department of Rheumatology, Qilu Hospital, Shandong University, Jinan, Shandong Province, China. FAU - Zhao, Zhang AU - Zhao Z AUID- ORCID: 0000-0003-3928-201 AD - Department of Orthopedic Surgery, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, Shandong Province, China. LA - eng PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) SB - IM MH - Humans MH - *Lung Diseases, Interstitial/etiology/diagnosis/physiopathology MH - *Scleroderma, Systemic/complications MH - Male MH - Female MH - Retrospective Studies MH - Middle Aged MH - Adult MH - Tomography, X-Ray Computed MH - Risk Factors MH - Aged MH - Autoantibodies/blood MH - Antibodies, Antinuclear/blood MH - Respiratory Function Tests MH - Disease Progression PMC - PMC12975168 OTO - NOTNLM OT - clinical features OT - interstitial lung disease OT - risk factors OT - systemic sclerosis OT - therapeutic strategy COIS- The authors have no funding and conflicts of interest to disclose. EDAT- 2026/03/06 19:43 MHDA- 2026/03/07 06:39 PMCR- 2026/03/06 CRDT- 2026/03/06 13:33 PHST- 2025/06/23 00:00 [received] PHST- 2026/02/10 00:00 [accepted] PHST- 2026/03/07 06:39 [medline] PHST- 2026/03/06 19:43 [pubmed] PHST- 2026/03/06 13:33 [entrez] PHST- 2026/03/06 00:00 [pmc-release] AID - 00005792-202603060-00014 [pii] AID - MD-D-25-07693 [pii] AID - 10.1097/MD.0000000000047876 [doi] PST - ppublish SO - Medicine (Baltimore). 2026 Mar 6;105(10):e47876. doi: 10.1097/MD.0000000000047876. PMID- 39761027 OWN - NLM STAT- MEDLINE DCOM- 20250430 LR - 20260107 IS - 2168-6157 (Electronic) IS - 2168-6149 (Print) IS - 2168-6149 (Linking) VI - 82 IP - 2 DP - 2025 Feb 1 TI - Cardiovascular Safety of Anti-CGRP Monoclonal Antibodies in Older Adults or Adults With Disability With Migraine. PG - 132-141 LID - 10.1001/jamaneurol.2024.4537 [doi] AB - IMPORTANCE: Monoclonal antibodies (mAbs) targeting calcitonin gene-related peptide (CGRP) or its receptor (anti-CGRP mAbs) offer effective migraine-specific preventive treatment. However, concerns exist about their potential cardiovascular risks due to CGRP blockade. OBJECTIVE: To compare the incidence of cardiovascular disease (CVD) between Medicare beneficiaries with migraine who initiated anti-CGRP-mAbs vs onabotulinumtoxinA in the US. DESIGN, SETTING, AND PARTICIPANTS: This retrospective, sequential cohort study was conducted among a nationally representative population-based sample of Medicare claims from May 2018 through December 2020. Data analysis was performed from August to December 2023. This study included fee-for-service Medicare beneficiaries aged 18 years or older with migraine who initiated either anti-CGRP mAbs or onabotulinumtoxinA. Beneficiaries who had a history of myocardial infarction (MI), stroke, cluster headache, malignant cancer, or hospice service within a 1-year baseline period prior to treatment initiation were excluded. To minimize channeling bias from new drug introductions and time-related bias due to the COVID-19 pandemic, 5 cohorts were established, representing sequential 6-month calendar intervals based on the initial prescription or date of index anti-CGRP mAbs or onabotulinumtoxinA use. EXPOSURE: Anti-CGRP mAbs vs onabotulinumtoxinA. MAIN OUTCOMES AND MEASURES: The primary outcome was time to first MI or stroke. Secondary outcomes included hypertensive crisis, peripheral revascularization, and Raynaud phenomenon. The inverse probability of treatment-weighted Cox proportional hazards models were used to compare outcomes between the 2 treatment groups. RESULTS: Among 266 848 eligible patients with migraine, 5153 patients initiated anti-CGRP mAbs (mean [SD] age, 57.8 [14.0] years; 4308 female patients [83.6%]) and 4000 patients initiated onabotulinumtoxinA (mean [SD] age, 61.9 [13.7] years; 3353 female patients [83.8%]). Use of anti-CGRP mAbs was not associated with an increased risk of composite CVD events (adjusted hazard ratio [aHR], 0.88; 95% CI, 0.44-1.77), hypertensive crisis (aHR, 0.46; 95% CI, 0.14-1.55), peripheral revascularization (aHR, 1.50; 95% CI, 0.48-4.73), or Raynaud phenomenon (aHR, 0.75; 95% CI, 0.45-1.24) compared with onabotulinumtoxinA. Subgroup analyses by age group and presence of established non-MI or stroke CVD showed similar findings. CONCLUSIONS AND RELEVANCE: In this cohort study, despite initial concerns regarding the cardiovascular effects of CGRP blockade, anti-CGRP mAbs were not associated with an increased risk of CVD compared with onabotulinumtoxinA among adult Medicare beneficiaries with migraine, who were predominantly older adults or individuals with disability. Future studies with longer follow-up periods and in other populations are needed to confirm these findings. FAU - Yang, Seonkyeong AU - Yang S AD - Department of Pharmaceutical Outcomes and Policy, College of Pharmacy, University of Florida, Gainesville. FAU - Orlova, Yulia AU - Orlova Y AD - Mayo Clinic, Jacksonville, Florida. FAU - Park, Haesuk AU - Park H AD - Department of Pharmaceutical Outcomes and Policy, College of Pharmacy, University of Florida, Gainesville. FAU - Smith, Steven M AU - Smith SM AD - Department of Pharmaceutical Outcomes and Policy, College of Pharmacy, University of Florida, Gainesville. AD - Center For Integrative Cardiovascular and Metabolic Disease, University of Florida, Gainesville. FAU - Guo, Yi AU - Guo Y AD - Department of Health Outcomes and Biomedical Informatics, College of Medicine, University of Florida, Gainesville. FAU - Chapin, Benjamin A AU - Chapin BA AD - Department of Anesthesiology, School of Medicine, University of Florida, Gainesville. AD - Geriatric Research Education and Clinical Center, North Florida/South Georgia Veterans Health System, Gainesville, Florida. FAU - Wilson, Debbie L AU - Wilson DL AD - Department of Pharmaceutical Outcomes and Policy, College of Pharmacy, University of Florida, Gainesville. FAU - Lo-Ciganic, Wei-Hsuan AU - Lo-Ciganic WH AD - Geriatric Research Education and Clinical Center, North Florida/South Georgia Veterans Health System, Gainesville, Florida. AD - Division of General Internal Medicine, School of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania. AD - Center for Pharmaceutical Policy and Prescribing, University of Pittsburgh, Pittsburgh, Pennsylvania. LA - eng PT - Journal Article PL - United States TA - JAMA Neurol JT - JAMA neurology JID - 101589536 RN - 0 (Antibodies, Monoclonal) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) RN - EC 3.4.24.69 (onabotulinum toxin A) RN - EC 3.4.24.69 (Botulinum Toxins, Type A) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Calcitonin Gene-Related Peptide Receptor Antagonists) SB - IM MH - Humans MH - Female MH - Male MH - *Migraine Disorders/drug therapy/epidemiology MH - Aged MH - Retrospective Studies MH - United States/epidemiology MH - *Cardiovascular Diseases/epidemiology/chemically induced MH - Middle Aged MH - *Antibodies, Monoclonal/adverse effects/therapeutic use MH - *Calcitonin Gene-Related Peptide/antagonists & inhibitors/immunology MH - Medicare MH - *Botulinum Toxins, Type A/therapeutic use/adverse effects MH - Cohort Studies MH - Antibodies, Monoclonal, Humanized/adverse effects MH - *Calcitonin Gene-Related Peptide Receptor Antagonists/adverse effects/therapeutic use MH - Incidence PMC - PMC11811796 COIS- Conflict of Interest Disclosures: Dr Park reported grants from Arista Networks, the National Human Genome Research Institute, the National Heart, Lung, and Blood Institute (NHLBI), the National Institute on Drug Abuse (NIDA), the Sleep Foundation, and the University of Florida Clinical and Translational Science Institute (UF CTSI) outside the submitted work. Dr Smith reported grants from the NHLBI, the Patient-Centered Outcomes Research Institute (PCORI), and the US Department of Defense outside the submitted work. Dr Wilson reported grants from the Bristol Myers Squibb/Pfizer Alliance American Thrombosis Investigator Initiated Research Program, Merck Sharp & Dohme, the NIDA (R01DA050676 and 1R01DA057886), the National Institute on Aging (NIA) (R21AG060308), the Sleep Research Society Foundation, and the US Department of Veterans Affairs (AGR DTD 04-26-2023) outside the submitted work and serving on the editorial board of the Journal of Pharmacy Technology. Dr Lo-Ciganic reported grants from Bristol Myers Squibb, Merck Sharp & Dohme, the NIA, the NIDA, the National Institute of Mental Health, the PCORI, the Pharmaceutical Research and Manufacturers of America Foundation, the Richard King Mellon Foundation, the Sleep Foundation, the UF CTSI, and the US Department of Veterans Affairs outside the submitted work; a pending patent for U1195.70174US00; uncompensated board membership by the Pharmacy Quality Alliance; and compensated consulting service for Teva Pharmaceuticals. No other disclosures were reported. EDAT- 2025/01/06 12:23 MHDA- 2025/02/10 18:16 PMCR- 2026/01/06 CRDT- 2025/01/06 11:33 PHST- 2025/02/10 18:16 [medline] PHST- 2025/01/06 12:23 [pubmed] PHST- 2025/01/06 11:33 [entrez] PHST- 2026/01/06 00:00 [pmc-release] AID - 2828333 [pii] AID - noi240083 [pii] AID - 10.1001/jamaneurol.2024.4537 [doi] PST - ppublish SO - JAMA Neurol. 2025 Feb 1;82(2):132-141. doi: 10.1001/jamaneurol.2024.4537. PMID- 38101775 OWN - NLM STAT- MEDLINE DCOM- 20231218 LR - 20231219 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 55 IP - 6 DP - 2023 Dec 18 TI - [Significance of anti-Jo-1 antibody's clinical stratification in idiopathic inflammatory myopathy and disease spectrum]. PG - 958-965 AB - OBJECTIVE: To investigate the significance of anti-histidyl tRNA synthetase (Jo-1) antibody in idiopathic inflammatory myopathies (IIM) and its diseases spectrum. METHODS: We enrolled all the patients who were tested positive for anti-Jo-1 antibody by immunoblotting in Peking University People's Hospital between 2016 and 2022. And the patients diagnosed with anti-synthetase antibody syndrome (ASS) with negative serum anti-Jo-1 antibody were enrolled as controls. We analyzed the basic information, clinical characteristics, and various inflammatory and immunological indicators of the patients at the onset of illness. RESULTS: A total of 165 patients with positive anti-Jo-1 antibody were enrolled in this study. Among them, 80.5% were diagnosed with connective tissue disease. And 57.6% (95/165) were diagnosed with IIM, including ASS (84/165, 50.9%), immune-mediated necrotizing myopathy (7/165, 4.2%) and dermatomyositis (4/165, 2.4%). There were 23.0% (38/165) diagnosed with other connective tissue disease, mainly including rheumatoid arthritis (11/165, 6.7%), undifferentiated connective tissue disease (5/165, 3.0%), interstitial pneumonia with autoimmune features (5/165, 3.0%), undifferentiated arthritis (4/165, 2.4%), Sjögren's syndrome (3/165, 1.8%), systemic lupus erythematosus (3/165, 1.8%), systemic vasculitis (3/165, 1.8%), and so on. Other cases included 3 (1.8%) malignant tumor patients, 4 (2.4%) infectious cases and so on. The diagnoses were not clear in 9.1% (15 /165) of the cohort. In the analysis of ASS subgroups, the group with positive serum anti-Jo-1 antibody had a younger age of onset than those with negative serum anti-Jo-1 antibody (49.9 years vs. 55.0 years, P=0.026). Clinical manifestations of arthritis (60.7% vs. 33.3%, P=0.002) and myalgia (47.1% vs. 22.2%, P=0.004) were more common in the ASS patients with positive anti-Jo-1 antibody. With the increase of anti-Jo-1 antibody titer, the incidence of the manifestations of arthritis, mechanic hands, Gottron sign and Raynaud phenomenon increased, and the proportion of abnormal creatine kinase and α-hydroxybutyric dehydrogenase index increased in the ASS patients. The incidence of myalgia and myasthenia were significantly more common in this cohort when anti-Jo-1 antibody-positive ASS patients were positive for one and more myositis specific antibodies/myositis associated autoantibodies (P < 0.05). CONCLUSION: The disease spectrum in patients with positive serum anti-Jo-1 antibody includes a variety of diseases, mainly ASS. And anti-Jo-1 antibody can also be found in many connective tissue diseases, malignant tumor, infection and so on. FAU - Li, Jia Chen AU - Li JC AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Lai, Zhan Hong AU - Lai ZH AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Shao, Miao AU - Shao M AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Jin, Yue Bo AU - Jin YB AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Gao, Xiao Juan AU - Gao XJ AD - Department of Rheumatology and Immunology, Ningde Hospital Affiliated to Ningde Normal University, Ningde 352199, Fujian, China. FAU - Zhang, Ke AU - Zhang K AD - Department of Endocrinology, 80th Group Army Hospital of Chinese PLA, Weifang 261000, Shandong, China. FAU - Hou, Jing AU - Hou J AD - Department of Nephrology, Zhangjiakou First Hospital, Zhangjiakou 075041, Hebei, China. FAU - Zhang, Yan Ying AU - Zhang YY AD - Department of Rheumatology, Shenzhen Hospital of Traditional Chinese Medicine, Shenzhen 518033, China. FAU - Li, Zhan Guo AU - Li ZG AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. FAU - Li, Yu Hui AU - Li YH AD - Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Humans MH - Middle Aged MH - Myalgia MH - *Myositis/diagnosis/epidemiology MH - Autoantibodies MH - *Connective Tissue Diseases MH - *Arthritis, Rheumatoid MH - *Neoplasms PMC - PMC10723990 OTO - NOTNLM OT - Anti-histidyl tRNA synthetase antibody OT - Anti-synthetase syndrome OT - Connective tissue disease EDAT- 2023/12/16 11:43 MHDA- 2023/12/18 06:42 PMCR- 2023/12/18 CRDT- 2023/12/15 19:53 PHST- 2023/12/18 06:42 [medline] PHST- 2023/12/16 11:43 [pubmed] PHST- 2023/12/15 19:53 [entrez] PHST- 2023/12/18 00:00 [pmc-release] AID - bjdxxbyxb-55-6-958 [pii] AID - 10.19723/j.issn.1671-167X.2023.06.002 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2023 Dec 18;55(6):958-965. doi: 10.19723/j.issn.1671-167X.2023.06.002. PMID- 17533548 OWN - NLM STAT- MEDLINE DCOM- 20080110 LR - 20161124 IS - 0174-1551 (Print) IS - 0174-1551 (Linking) VI - 30 IP - 5 DP - 2007 Sep-Oct TI - Percutaneous manual aspiration embolectomy of renal vein thrombosis due to acute pyelonephritis. PG - 1075-8 AB - We report the case of a 50-year-old man who presented to our institution with septic thrombosis of the renal vein which had not resolved despite several days of antibiotic therapy. Optimal restoration of renal vein flow was obtained by percutaneous manual aspiration embolectomy (PMAE) in this patient with contraindication to fibrinolytic therapy and surgery. FAU - Novelli, Luigi AU - Novelli L AD - Radiologie Cardiovasculaire, HEGP, Paris, France. luigi.novelli@egp.aphp.fr FAU - Raynaud, Alain AU - Raynaud A FAU - Pellerin, Olivier AU - Pellerin O FAU - Carreres, Thierry AU - Carreres T FAU - Sapoval, Marc AU - Sapoval M LA - eng PT - Case Reports PT - Journal Article DEP - 20070529 PL - United States TA - Cardiovasc Intervent Radiol JT - Cardiovascular and interventional radiology JID - 8003538 RN - 0 (Anti-Bacterial Agents) SB - IM MH - Acute Disease MH - Anti-Bacterial Agents/therapeutic use MH - Combined Modality Therapy MH - Embolectomy/*methods MH - Humans MH - Male MH - Middle Aged MH - Phlebography MH - Pyelonephritis/*complications/diagnostic imaging/drug therapy/physiopathology/surgery MH - Renal Veins/diagnostic imaging/physiopathology/*surgery MH - Suction MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Vascular Patency MH - Venous Thrombosis/diagnostic imaging/drug therapy/etiology/physiopathology/*surgery EDAT- 2007/05/30 09:00 MHDA- 2008/01/11 09:00 CRDT- 2007/05/30 09:00 PHST- 2006/09/29 00:00 [received] PHST- 2006/11/07 00:00 [accepted] PHST- 2007/05/30 09:00 [pubmed] PHST- 2008/01/11 09:00 [medline] PHST- 2007/05/30 09:00 [entrez] AID - 10.1007/s00270-007-9035-4 [doi] PST - ppublish SO - Cardiovasc Intervent Radiol. 2007 Sep-Oct;30(5):1075-8. doi: 10.1007/s00270-007-9035-4. Epub 2007 May 29. PMID- 23397042 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20130212 LR - 20211021 IS - 1752-4571 (Print) IS - 1752-4571 (Electronic) IS - 1752-4571 (Linking) VI - 6 IP - 1 DP - 2013 Jan TI - Applying ecological and evolutionary theory to cancer: a long and winding road. PG - 1-10 LID - 10.1111/eva.12021 [doi] AB - Since the mid 1970s, cancer has been described as a process of Darwinian evolution, with somatic cellular selection and evolution being the fundamental processes leading to malignancy and its many manifestations (neoangiogenesis, evasion of the immune system, metastasis, and resistance to therapies). Historically, little attention has been placed on applications of evolutionary biology to understanding and controlling neoplastic progression and to prevent therapeutic failures. This is now beginning to change, and there is a growing international interest in the interface between cancer and evolutionary biology. The objective of this introduction is first to describe the basic ideas and concepts linking evolutionary biology to cancer. We then present four major fronts where the evolutionary perspective is most developed, namely laboratory and clinical models, mathematical models, databases, and techniques and assays. Finally, we discuss several of the most promising challenges and future prospects in this interdisciplinary research direction in the war against cancer. FAU - Thomas, Frédéric AU - Thomas F AD - MIVEGEC (UMR CNRS/IRD/UM1) 5290 Montpellier Cedex 5, France ; CREEC Montpellier Cedex 5, France. FAU - Fisher, Daniel AU - Fisher D FAU - Fort, Philippe AU - Fort P FAU - Marie, Jean-Pierre AU - Marie JP FAU - Daoust, Simon AU - Daoust S FAU - Roche, Benjamin AU - Roche B FAU - Grunau, Christoph AU - Grunau C FAU - Cosseau, Céline AU - Cosseau C FAU - Mitta, Guillaume AU - Mitta G FAU - Baghdiguian, Stephen AU - Baghdiguian S FAU - Rousset, François AU - Rousset F FAU - Lassus, Patrice AU - Lassus P FAU - Assenat, Eric AU - Assenat E FAU - Grégoire, Damien AU - Grégoire D FAU - Missé, Dorothée AU - Missé D FAU - Lorz, Alexander AU - Lorz A FAU - Billy, Frédérique AU - Billy F FAU - Vainchenker, William AU - Vainchenker W FAU - Delhommeau, François AU - Delhommeau F FAU - Koscielny, Serge AU - Koscielny S FAU - Itzykson, Raphael AU - Itzykson R FAU - Tang, Ruoping AU - Tang R FAU - Fava, Fanny AU - Fava F FAU - Ballesta, Annabelle AU - Ballesta A FAU - Lepoutre, Thomas AU - Lepoutre T FAU - Krasinska, Liliana AU - Krasinska L FAU - Dulic, Vjekoslav AU - Dulic V FAU - Raynaud, Peggy AU - Raynaud P FAU - Blache, Philippe AU - Blache P FAU - Quittau-Prevostel, Corinne AU - Quittau-Prevostel C FAU - Vignal, Emmanuel AU - Vignal E FAU - Trauchessec, Hélène AU - Trauchessec H FAU - Perthame, Benoit AU - Perthame B FAU - Clairambault, Jean AU - Clairambault J FAU - Volpert, Vitali AU - Volpert V FAU - Solary, Eric AU - Solary E FAU - Hibner, Urszula AU - Hibner U FAU - Hochberg, Michael E AU - Hochberg ME LA - eng PT - Journal Article DEP - 20121116 PL - England TA - Evol Appl JT - Evolutionary applications JID - 101461828 PMC - PMC3567465 OTO - NOTNLM OT - cancer, disease biology OT - evolutionary medicine OT - evolutionary theory EDAT- 2013/02/12 06:00 MHDA- 2013/02/12 06:01 PMCR- 2013/01/01 CRDT- 2013/02/12 06:00 PHST- 2012/06/13 00:00 [received] PHST- 2012/09/07 00:00 [accepted] PHST- 2013/02/12 06:00 [entrez] PHST- 2013/02/12 06:00 [pubmed] PHST- 2013/02/12 06:01 [medline] PHST- 2013/01/01 00:00 [pmc-release] AID - 10.1111/eva.12021 [doi] PST - ppublish SO - Evol Appl. 2013 Jan;6(1):1-10. doi: 10.1111/eva.12021. Epub 2012 Nov 16. PMID- 18589289 OWN - NLM STAT- MEDLINE DCOM- 20100402 LR - 20080630 IS - 1776-2561 (Electronic) IS - 0761-8417 (Linking) VI - 64 IP - 2 DP - 2008 Apr TI - [Thrombosis and lung cancer]. PG - 85-91 LID - 10.1016/j.pneumo.2008.04.003 [doi] AB - This relation is sometimes described as a double association: venous thromboembolism (VTE) can reveal cancer (so-called Trousseau syndrome), but cancer and its treatment are also risk factors for VTE. Lung cancer, frequent and serious, is one of the greatest purveyors of VTE, a disease that pneumologists and oncologists must often confront in diagnosis, prevention, and treatment. This article investigates the epidemiological, prevention, and treatment aspects of VTE in cancer patients, particularly those with lung cancer, but also discusses diagnostic specificities and, briefly, the possible antitumor effect of heparins. FAU - Girard, P AU - Girard P AD - Département Thoracique, Institut Mutualiste Montsouris, 75014 Paris, France. philippe.girard@imm.fr FAU - Raynaud, C AU - Raynaud C FAU - Meyer, G AU - Meyer G FAU - Parent, F AU - Parent F FAU - Besse, B AU - Besse B LA - fre PT - English Abstract PT - Journal Article PT - Review TT - Thrombose et cancer bronchique. DEP - 20080605 PL - France TA - Rev Pneumol Clin JT - Revue de pneumologie clinique JID - 8406312 RN - 0 (Anticoagulants) RN - 0 (Antineoplastic Agents) RN - 9005-49-6 (Heparin) SB - IM MH - Anticoagulants/adverse effects/therapeutic use MH - Antineoplastic Agents/adverse effects/therapeutic use MH - Carcinoma, Bronchogenic/mortality/*physiopathology MH - Heparin/adverse effects/therapeutic use MH - Humans MH - Lung Neoplasms/mortality/*physiopathology MH - Palliative Care/*methods MH - Survival Rate MH - Thrombosis/drug therapy/*etiology/mortality MH - Venous Thromboembolism/drug therapy/*etiology/mortality RF - 37 EDAT- 2008/07/01 09:00 MHDA- 2010/04/03 06:00 CRDT- 2008/07/01 09:00 PHST- 2008/07/01 09:00 [pubmed] PHST- 2010/04/03 06:00 [medline] PHST- 2008/07/01 09:00 [entrez] AID - S0761-8417(08)00008-4 [pii] AID - 10.1016/j.pneumo.2008.04.003 [doi] PST - ppublish SO - Rev Pneumol Clin. 2008 Apr;64(2):85-91. doi: 10.1016/j.pneumo.2008.04.003. Epub 2008 Jun 5. PMID- 35570033 OWN - NLM STAT- MEDLINE DCOM- 20220608 LR - 20220608 IS - 1776-2588 (Electronic) IS - 0761-8425 (Linking) VI - 39 IP - 5 DP - 2022 May TI - [Aspergillus mediastinitis following post-immunotherapy lobectomy for oligometastatic lung]. PG - 498-501 LID - S0761-8425(22)00165-6 [pii] LID - 10.1016/j.rmr.2022.04.002 [doi] AB - The relationship between infectious disease and therapy with immune checkpoint inhibitors remains unknown. We report the case of a 50-year-old woman with metastatic lung adenocarcinoma who responded remarkably well to immunotherapy and underwent upper right lobectomy. Three weeks after hospital discharge, she was readmitted for severe dyspnea due to mainstem bronchus compression by mediastinal mass. Histological analysis of transbronchial needle aspiration revealed A. fumigatus. After six months of voriconazole regimen, her symptoms improved with the regression of bronchial compression. Postoperative progression of pseudo-tumoral mass in patients treated with long-term immunotherapy may be related to opportunistic infectious disease and requires investigation. CI - Copyright © 2022. Published by Elsevier Masson SAS. FAU - Masgnaux, C AU - Masgnaux C AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Boukhiar, H AU - Boukhiar H AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Dakhil, B AU - Dakhil B AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Raynaud, C AU - Raynaud C AD - Service de pneumologie et d'oncologie thoracique, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Zaimi, R AU - Zaimi R AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Kashi-Dakhil, M AU - Kashi-Dakhil M AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. FAU - Bagan, P AU - Bagan P AD - Service de chirurgie thoracique et vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutnant-Colonel Prudhon, 95107 Argenteuil, France. Electronic address: patrick.bagan@ch-argenteuil.fr. LA - fre PT - Case Reports PT - Journal Article TT - Médiastinite à Aspergillus après lobectomie post immunothérapie pour un cancer du poumon oligométastatique. DEP - 20220512 PL - France TA - Rev Mal Respir JT - Revue des maladies respiratoires JID - 8408032 SB - IM MH - Aspergillus MH - Female MH - Humans MH - Immunotherapy MH - Lung/pathology MH - *Lung Neoplasms/complications/pathology/surgery MH - *Mediastinitis/diagnosis/etiology/therapy MH - Middle Aged OTO - NOTNLM OT - A. fumigatus OT - Cancer bonchique non à petites cellules OT - Immunotherapy OT - Immunothérapie OT - Mediastinitis OT - Médiastinite OT - Non-small cell lung cancer EDAT- 2022/05/16 06:00 MHDA- 2022/06/09 06:00 CRDT- 2022/05/15 22:03 PHST- 2021/06/24 00:00 [received] PHST- 2022/03/28 00:00 [accepted] PHST- 2022/05/16 06:00 [pubmed] PHST- 2022/06/09 06:00 [medline] PHST- 2022/05/15 22:03 [entrez] AID - S0761-8425(22)00165-6 [pii] AID - 10.1016/j.rmr.2022.04.002 [doi] PST - ppublish SO - Rev Mal Respir. 2022 May;39(5):498-501. doi: 10.1016/j.rmr.2022.04.002. Epub 2022 May 12. PMID- 21111289 OWN - NLM STAT- MEDLINE DCOM- 20110330 LR - 20101129 IS - 1776-2588 (Electronic) IS - 0761-8425 (Linking) VI - 27 IP - 9 DP - 2010 Nov TI - [Pleuropulmonary involvement leading to bilateral pneumothorax in a patient being treated for rheumatoid arthritis]. PG - 1119-23 LID - 10.1016/j.rmr.2010.04.015 [doi] AB - INTRODUCTION: Rheumatoid arthritis (RA) is a systemic illness where the development of pulmonary nodule has been described in from 4 to 20% of patients. Symptomatic pleural manifestations occur in 3 to 5% of cases. Rarely, pulmonary nodules become necrotic and lead to pleural complications. Bilateral pneumothorax has only rarely been described. CASE REPORT: We report the case of a 64-year-old woman, who had been treated for RA for several years and presented with bilateral pneumothorax secondary to necrobiosis of one or several pulmonary rheumatoid nodules. The management of the pneumothorax was very prolonged and difficult, and despite surgical pleurodesis, the lung did not reexpand fully. Pathological examination of the pleura revealed a noncaseating granulomatous pattern. The diagnosis of a sarcoidosis like disease, possibly induced by anti-TNFα, or of pleural tuberculosis were suggested, but we concluded that the final diagnosis was of pleural rheumatoid involvement. CONCLUSION: Bilateral pneumothorax secondary to rheumatoid nodule is a rare entity. The management of such a complication is difficult, particularly in patients who receive an immunosuppressant regimen. A granulomatous pattern has been described rarely in the pleural tissue of these patients. Specific RA pleural involvement has to be taken in consideration when other diagnoses are eliminated, especially tuberculosis or sarcoidosis-like disease. CI - Copyright © 2010 SPLF. Published by Elsevier Masson SAS. All rights reserved. FAU - N'Gabou, D AU - N'Gabou D AD - Département thoracique, institut mutualiste Montsouris, 42, boulevard Jourdan, 75014 Paris, France. FAU - Magdeleinat, P AU - Magdeleinat P FAU - Weber, N AU - Weber N FAU - Raynaud, C AU - Raynaud C FAU - Gossot, D AU - Gossot D FAU - Mechouek, A AU - Mechouek A FAU - Stern, J-B AU - Stern JB LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Atteinte pleuropulmonaire conduisant à un pneumothorax bilatéral chez une patiente traitée pour une polyarthrite rhumatoïde. DEP - 20101027 PL - France TA - Rev Mal Respir JT - Revue des maladies respiratoires JID - 8408032 SB - IM MH - Arthritis, Rheumatoid/*complications MH - Female MH - Humans MH - Lung Diseases/*complications MH - Middle Aged MH - Pleural Diseases/*complications MH - Pneumothorax/*etiology MH - Rheumatoid Nodule/*complications EDAT- 2010/11/30 06:00 MHDA- 2011/03/31 06:00 CRDT- 2010/11/30 06:00 PHST- 2009/10/13 00:00 [received] PHST- 2010/04/17 00:00 [accepted] PHST- 2010/11/30 06:00 [entrez] PHST- 2010/11/30 06:00 [pubmed] PHST- 2011/03/31 06:00 [medline] AID - S0761-8425(10)00415-8 [pii] AID - 10.1016/j.rmr.2010.04.015 [doi] PST - ppublish SO - Rev Mal Respir. 2010 Nov;27(9):1119-23. doi: 10.1016/j.rmr.2010.04.015. Epub 2010 Oct 27. PMID- 35452855 OWN - NLM STAT- MEDLINE DCOM- 20220524 LR - 20250623 IS - 1873-0183 (Electronic) IS - 1568-9972 (Linking) VI - 21 IP - 6 DP - 2022 Jun TI - Sjögren syndrome overlapping with ANCA-associated vasculitis: Four additional cases and systematic literature review. PG - 103099 LID - S1568-9972(22)00069-6 [pii] LID - 10.1016/j.autrev.2022.103099 [doi] AB - OBJECTIVES: Sjögren's syndrome (SS) and ANCA-associated vasculitis (AAV) have distinct clinical presentation and evolution, with paucity of reports on overlap syndrome. We aimed to better characterize this entity. METHODS: We report four additional cases from the Montpellier university hospital. We also performed a systematic literature review, according to PRISMA guidelines, in Medline, Embase, Web of science, Cochrane Library, and grey literature. Demographic, clinical, and paraclinical data on SS and AAV were analysed. RESULTS: A total of 3133 articles was identified in databases, with 2695 articles screened for eligibility. After exclusion, we had 30 articles on 40 patients to analyse, in addition to 4 patients from our local recruitment (44 patients overall). Patients were female in 81.8%, with median age at AAV onset of 63.5 years. All patients but one presented with SS before, or concomitantly to the diagnosis of AAV, with a median delay of 12 months between both diagnoses. AAV predominantly had renal involvement (35/44 patients, 79.5%), anti-MPO antibodies being the most frequent (35 patients), even in patients presenting with granulomatosis with polyangiitis. We observed significantly more Raynaud phenomenon and associated auto-immune diseases in the group of non-granulomatous AAV (10 patients versus 1, p = 0.015 and 8 patients versus 0, p = 0.013, respectively). CONCLUSIONS: This is the largest descriptive study on the association between SS and AAV, providing information on this challenging diagnosis and interplay between these two diseases. Particular attention should be paid in the first months after diagnosis, given the specific complications and outcomes of each disease. CI - Copyright © 2022 Elsevier B.V. All rights reserved. FAU - Coustal, Cyrille AU - Coustal C AD - Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France. FAU - Guillope, Béatrice AU - Guillope B AD - Department of nephrology, Université de Montpellier, CHU de Montpellier, Montpellier, France. FAU - Serrand, Chris AU - Serrand C AD - Department of Epidemiology and Biostatistics, Montpellier University Hospital, Montpellier, France. FAU - Morel, Jacques AU - Morel J AD - Department of Rheumatology, Montpellier University Hospital, University of Montpellier, PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR, 9214 Montpellier, France. FAU - Taieb, Guillaume AU - Taieb G AD - Department of Neurology, University Hospital of Montpellier, Montpellier, France. FAU - Castille, Elodie AU - Castille E AD - Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France. FAU - Meliani, Kaoutar AU - Meliani K AD - Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France. FAU - Darmon, Olivier AU - Darmon O AD - Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France. FAU - Goulabchand, Radjiv AU - Goulabchand R AD - Internal Medicine Department, Nîmes University Hospital, Montpellier University, Nîmes, France. FAU - Guilpain, Philippe AU - Guilpain P AD - Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France; Institute of Regenerative Medicine and Biotherapy, Institut national de la santé et de la recherche médicale U1183, Montpellier, France. Electronic address: p-guilpain@chu-montpellier.fr. LA - eng PT - Journal Article PT - Systematic Review DEP - 20220419 PL - Netherlands TA - Autoimmun Rev JT - Autoimmunity reviews JID - 101128967 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) SB - IM MH - *Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis MH - Antibodies, Antineutrophil Cytoplasmic MH - Female MH - *Granulomatosis with Polyangiitis MH - Humans MH - Male MH - Retrospective Studies MH - *Sjogren's Syndrome/complications OTO - NOTNLM OT - ANCA-associated vasculitis OT - Granulomatous vasculitis OT - Overlap syndrome OT - Sjögren's syndrome OT - Systematic review EDAT- 2022/04/23 06:00 MHDA- 2022/05/25 06:00 CRDT- 2022/04/22 20:10 PHST- 2022/03/17 00:00 [received] PHST- 2022/04/18 00:00 [accepted] PHST- 2022/04/23 06:00 [pubmed] PHST- 2022/05/25 06:00 [medline] PHST- 2022/04/22 20:10 [entrez] AID - S1568-9972(22)00069-6 [pii] AID - 10.1016/j.autrev.2022.103099 [doi] PST - ppublish SO - Autoimmun Rev. 2022 Jun;21(6):103099. doi: 10.1016/j.autrev.2022.103099. Epub 2022 Apr 19. PMID- 33163501 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201112 IS - 2296-858X (Print) IS - 2296-858X (Electronic) IS - 2296-858X (Linking) VI - 7 DP - 2020 TI - High-Frequency Ultrasound of Multiple Arterial Areas Reveals Increased Intima Media Thickness, Vessel Wall Appearance, and Atherosclerotic Plaques in Systemic Lupus Erythematosus. PG - 581336 LID - 10.3389/fmed.2020.581336 [doi] LID - 581336 AB - Introduction: Despite improved therapies and management, patients with systemic lupus erythematosus (SLE) still have increased risks of cerebrovascular and cardiovascular disease. High-frequency ultrasound (US) provides an opportunity to distinguish atherosclerosis from inflammation in the vessels. We hypothesized that an extended US protocol may add information regarding vascular affection in SLE. Methods: Sixty patients (52 women, 8 men; mean age 43.2 ± 11.3 years) with SLE characterized by either lupus nephritis (LN; n = 20), antiphospholipid syndrome (APS; n = 20), or skin and joint involvement (n = 20) as well as matched healthy controls (n = 60) were included. Intima-media thickness (IMT), assessment of vessel walls, and plaque occurrence were recorded using high-frequency US (GE Logic E9) in common carotid, internal carotid, brachiocephalic, subclavian, axillary, common femoral, and proximal superficial femoral arteries as well as in the aortic arch. Results: For the entire SLE group, IMT was increased in the internal carotid artery (0.52 ± 0.17 vs. 0.45 ± 0.09 mm, p = 0.004), the common femoral artery (0.57 ± 0.23 vs. 0.49 ± 0.11 mm, p < 0.01), the subclavian artery (0.58 ± 0.19 vs. 0.53 ± 0.13 mm, p = 0.02), and the aortic arch (1.21 ± 0.63 vs. 0.98 ± 0.25 mm, p = 0.002) compared to controls. These differences were primarily observed in the APS and LN groups compared to controls. Vessels with increased IMT ≥0.9 mm had a smooth, medium echogenic appearance in areas free of atherosclerotic plaques. Atherosclerotic plaques were detected in 15/60 patients (25%) as compared to 2/60 of the controls (3%). Plaques were predominantly (67%) located in the carotid bifurcation. Multivariate analysis revealed influence of age on IMT in all vessel areas. Furthermore, in the common femoral artery, sagittal abdominal diameter, diastolic blood pressure, and cholesterol all showed association with increased IMT. In the internal carotid artery, male sex and presence of Raynaud phenomenon influenced IMT. Conclusion: Among SLE patients without presence of plaques, an extended US protocol revealed increased wall thickness with predominantly medium echogenic appearance highlighting possibly inflammation or early atherosclerosis. The appearance of vessel walls has not previously been studied in detail. An increased number of plaques were found in SLE compared to age- and sex-matched healthy controls. We found similar risk factors for increased IMT and occurrence of plaques, possibly indicating atherosclerotic mechanisms rather than inflammation. CI - Copyright © 2020 Svensson, Eriksson, Zachrisson and Sjöwall. FAU - Svensson, Christina AU - Svensson C AD - Department of Clinical Physiology, University Hospital, Linköping, Sweden. AD - Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden. FAU - Eriksson, Per AU - Eriksson P AD - Division of Inflammation and Infection, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden. FAU - Zachrisson, Helene AU - Zachrisson H AD - Department of Clinical Physiology, University Hospital, Linköping, Sweden. AD - Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden. FAU - Sjöwall, Christopher AU - Sjöwall C AD - Division of Inflammation and Infection, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden. LA - eng PT - Journal Article DEP - 20201009 PL - Switzerland TA - Front Med (Lausanne) JT - Frontiers in medicine JID - 101648047 PMC - PMC7581856 OTO - NOTNLM OT - IMT OT - plaque OT - systemic lupus erythematosus OT - ultrasound OT - vessel wall EDAT- 2020/11/10 06:00 MHDA- 2020/11/10 06:01 PMCR- 2020/10/09 CRDT- 2020/11/09 05:33 PHST- 2020/07/08 00:00 [received] PHST- 2020/09/04 00:00 [accepted] PHST- 2020/11/09 05:33 [entrez] PHST- 2020/11/10 06:00 [pubmed] PHST- 2020/11/10 06:01 [medline] PHST- 2020/10/09 00:00 [pmc-release] AID - 10.3389/fmed.2020.581336 [doi] PST - epublish SO - Front Med (Lausanne). 2020 Oct 9;7:581336. doi: 10.3389/fmed.2020.581336. eCollection 2020. PMID- 28387421 OWN - NLM STAT- MEDLINE DCOM- 20171121 LR - 20181202 IS - 1097-4652 (Electronic) IS - 0021-9541 (Linking) VI - 233 IP - 2 DP - 2018 Feb TI - APN/CD13 is over-expressed by Psoriatic fibroblasts and is modulated by CGRP and IL-4 but not by retinoic acid treatment. PG - 958-967 LID - 10.1002/jcp.25941 [doi] AB - Psoriasis vulgaris is a common skin inflammatory disease characterized by recurrent flare episodes associated with scaly well-demarcated skin plaques. Skin biopsies from psoriatic patients with high PASI score (22.67 ± 8.67) and from HD were used to study APN/CD13. APN/CD13 is over-expressed in LP and nLP compare to HD skins and fibroblasts. This over-expression is positively correlated with specific enzymatic activity enhancement. However, discrepancies between APN/CD13 expression in LP and nLP prompt us to focus our study on APN/CD13 modulation. Calcitonin Gene Related Peptide (CGRP), a neuropeptide, positively modulated expression and activity of APN/CD13. CGRP consistently induced IL4 secretion, which is also involved in the increase of APN/CD13 expression and activity, which is significantly reversed using IL-4 blocking antibody. Surprisingly, retinoic acid altered the APN/CD13 enzymatic activity only in nLP fibroblasts without modification of APN/CD13 expression. APN/CD13 is over-expressed on psoriatic fibroblasts and exerted high level of activity compare to HD fibroblasts. Taken together, several factors such as CGRP and IL-4 acted on positive regulation of APN/CD13 expression and activity. This study highlighted the interest of APN/CD13 as a new potential target, which should be investigated in psoriasis. CI - © 2017 Wiley Periodicals, Inc. FAU - Gerbaud, Pascale AU - Gerbaud P AD - INSERM, Université Paris Descartes, Paris, France. FAU - Guibourdenche, Jean AU - Guibourdenche J AD - Service de Biologie hormonale et métabolique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. FAU - Jarray, Rafika AU - Jarray R AD - Sup'Biotech, Villejuif, France. AD - CEA, Division of Prions and Related Diseases (SEPIA), Institute of Emerging Diseases and Innovative Therapies (iMETI), Fontenay-aux- Roses, France. FAU - Conti, Marc AU - Conti M AD - Service de Biochimie, Hôpital Henri-Mondor, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Est, Créteil, France. FAU - Palmic, Patricia AU - Palmic P AD - Service d'Anatomie et Cytologie Pathologiques, CHU de Martinique, Hôpital Pierre Zobda Quitman, Fort-de-France Cedex, France. AD - Laboratory of Cellular and Molecular Basis of Normal Hematopoiesis and Hematological Disorders, INSERM UMR 1163, Paris, France. AD - Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris, France. AD - CNRS ERL 8254, Paris, France. FAU - Leclerc-Mercier, Stéphanie AU - Leclerc-Mercier S AD - Pathology Department and Reference Center for Rare Skin Diseases (MAGEC), Hôpital Necker-Enfants Malades, Paris, France. FAU - Bruneau, Julie AU - Bruneau J AD - Laboratory of Cellular and Molecular Basis of Normal Hematopoiesis and Hematological Disorders, INSERM UMR 1163, Paris, France. AD - Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris, France. AD - CNRS ERL 8254, Paris, France. AD - Service de Pathologie, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. FAU - Hermine, Olivier AU - Hermine O AD - Laboratory of Cellular and Molecular Basis of Normal Hematopoiesis and Hematological Disorders, INSERM UMR 1163, Paris, France. AD - Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris, France. AD - CNRS ERL 8254, Paris, France. FAU - Lepelletier, Yves AU - Lepelletier Y AUID- ORCID: 0000-0001-7746-5170 AD - Laboratory of Cellular and Molecular Basis of Normal Hematopoiesis and Hematological Disorders, INSERM UMR 1163, Paris, France. AD - Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris, France. AD - CNRS ERL 8254, Paris, France. FAU - Raynaud, Françoise AU - Raynaud F AD - Laboratory of Cellular and Molecular Basis of Normal Hematopoiesis and Hematological Disorders, INSERM UMR 1163, Paris, France. AD - Imagine Institute, Paris Descartes University-Sorbonne Paris Cité, Paris, France. AD - CNRS ERL 8254, Paris, France. AD - Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques (LCBPT), UMR 8601 CNRS, Université Paris Descartes, Sorbonne Paris Cité, UFR Biomédicale des Saints Pères, Paris, France. LA - eng PT - Journal Article DEP - 20170523 PL - United States TA - J Cell Physiol JT - Journal of cellular physiology JID - 0050222 RN - 0 (IL4 protein, human) RN - 207137-56-2 (Interleukin-4) RN - 5688UTC01R (Tretinoin) RN - EC 3.4.11.2 (CD13 Antigens) RN - JHB2QIZ69Z (Calcitonin Gene-Related Peptide) SB - IM MH - Adult MH - Aged MH - CD13 Antigens/genetics/*metabolism MH - Calcitonin Gene-Related Peptide/*pharmacology MH - Case-Control Studies MH - Cells, Cultured MH - Female MH - Fibroblasts/*drug effects/enzymology/pathology MH - Humans MH - Interleukin-4/*pharmacology MH - Male MH - Middle Aged MH - Psoriasis/*enzymology/genetics/pathology MH - Skin/*drug effects/enzymology/pathology MH - Time Factors MH - Tretinoin/*pharmacology MH - Up-Regulation OTO - NOTNLM OT - CGRP OT - aminopeptidase-N OT - fibroblasts OT - interleukin-4 OT - psoriasis EDAT- 2017/04/08 06:00 MHDA- 2017/11/29 06:00 CRDT- 2017/04/08 06:00 PHST- 2016/09/28 00:00 [received] PHST- 2017/03/31 00:00 [accepted] PHST- 2017/04/08 06:00 [pubmed] PHST- 2017/11/29 06:00 [medline] PHST- 2017/04/08 06:00 [entrez] AID - 10.1002/jcp.25941 [doi] PST - ppublish SO - J Cell Physiol. 2018 Feb;233(2):958-967. doi: 10.1002/jcp.25941. Epub 2017 May 23. PMID- 24507585 OWN - NLM STAT- MEDLINE DCOM- 20141028 LR - 20211021 IS - 1756-0500 (Electronic) IS - 1756-0500 (Linking) VI - 7 DP - 2014 Feb 7 TI - Intensive aerobic and muscle endurance exercise in patients with systemic sclerosis: a pilot study. PG - 86 LID - 10.1186/1756-0500-7-86 [doi] AB - BACKGROUND: No previous studies have examined the effect of intensive exercise in systemic sclerosis patients with pulmonary impairment. The objective of this study was to examine the effect of an eight-week intensive aerobic exercise and muscle endurance training program for patients with systemic sclerosis with 50-100% of forced vital capacity. METHODS: A single-subject experimental design with repeated systematic measures during a six week A-phase (non-interventional baseline period) and an eight week B-phase (exercise intervention period) was used. Three women and one man with median age 66 years and median disease duration of 3.5 years completed aerobic exercise corresponding to 15 on the Borg RPE scale (strenuous) and muscular endurance training three times/week. Physical capacity (six-minute walk test), aerobic capacity (submaximal treadmill test) and muscle endurance in shoulder and hip flexion (Functional Index 2) were assessed every other week throughout the 14-week study. Activity limitation (Health Assessment Questionnaire), quality of life (Short Form 36), Raynaud, Fatigue and Global Health during the recent week (Visual Analogue Scales) were assessed at weeks 0, 6, 14. RESULTS: Three participants improved significantly in muscular endurance, and two participants improved significantly or clinically relevant in aerobic capacity. All other variables remained unchanged, except for a trend towards reduced fatigue. CONCLUSIONS: This eight week exercise program was largely successful with positive effects on aerobic capacity and muscle endurance. TRIAL REGISTRATION: Clinicaltrials.gov Identifier: NCT01813578. FAU - Alexanderson, Helene AU - Alexanderson H AD - Department of Neurobiology, Care Science and Society, Division of Physical Therapy, Karolinska Institutet, Stockholm, Sweden. helene.alexanderson@karolinska.se. FAU - Bergegård, Jenny AU - Bergegård J FAU - Björnådal, Lena AU - Björnådal L FAU - Nordin, Annica AU - Nordin A LA - eng SI - ClinicalTrials.gov/NCT01813578 PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140207 PL - England TA - BMC Res Notes JT - BMC research notes JID - 101462768 SB - IM MH - Adult MH - Aged MH - Exercise/*physiology MH - Exercise Therapy/*methods MH - Fatigue/diagnosis/physiopathology MH - Female MH - Hip Joint/physiology MH - Humans MH - Lung Diseases/complications/physiopathology/therapy MH - Male MH - Middle Aged MH - Pain Measurement MH - Physical Fitness/*physiology MH - Pilot Projects MH - Quality of Life MH - Range of Motion, Articular/physiology MH - Scleroderma, Systemic/complications/physiopathology/*therapy MH - Shoulder Joint/physiology MH - Surveys and Questionnaires MH - Time Factors MH - Treatment Outcome MH - Vital Capacity/physiology MH - Walking/physiology PMC - PMC3996139 EDAT- 2014/02/11 06:00 MHDA- 2014/10/29 06:00 PMCR- 2014/02/07 CRDT- 2014/02/11 06:00 PHST- 2013/05/30 00:00 [received] PHST- 2014/01/23 00:00 [accepted] PHST- 2014/02/11 06:00 [entrez] PHST- 2014/02/11 06:00 [pubmed] PHST- 2014/10/29 06:00 [medline] PHST- 2014/02/07 00:00 [pmc-release] AID - 1756-0500-7-86 [pii] AID - 10.1186/1756-0500-7-86 [doi] PST - epublish SO - BMC Res Notes. 2014 Feb 7;7:86. doi: 10.1186/1756-0500-7-86. PMID- 18162130 OWN - NLM STAT- MEDLINE DCOM- 20080328 LR - 20260128 IS - 1479-5876 (Electronic) IS - 1479-5876 (Linking) VI - 5 DP - 2007 Dec 27 TI - Association between long-term neuro-toxicities in testicular cancer survivors and polymorphisms in glutathione-s-transferase-P1 and -M1, a retrospective cross sectional study. PG - 70 AB - BACKGROUND: To assess the impact of polymorphisms in Glutathione S-transferase (GST) -P1, -M1, and -T1 on self-reported chemotherapy-induced long-term toxicities in testicular cancer survivors (TCSs). METHODS: A total of 238 TCSs, who had received cisplatin-based chemotherapy at median twelve years earlier, had participated in a long-term follow-up survey which assessed the prevalence of self-reported paresthesias in fingers/toes, Raynaud-like phenomena in fingers/toes, tinnitus, and hearing impairment. From all TCSs lymphocyte-derived DNA was analyzed for the functional A-->G polymorphism at bp 304 in GSTP1, and deletions in GST-M1 and GST-T1. Evaluation of associations between GST polymorphisms and self-reported toxicities included adjustment for prior treatment. RESULTS: All six evaluated toxicities were significantly associated with the cumulative dose of cisplatin and/or bleomycin. Compared to TCSs with either GSTP1-AG or GSTP1-AA, the 37 TCSs with the genotype GSTP1-GG, were significantly less bothered by paresthesias in fingers and toes (p = 0.039, OR 0.46 [0.22-0.96] and p = 0.023, OR 0.42 [0.20-0.88], respectively), and tinnitus (p = 0.008, OR 0.33 [0.14-0.74]). Furthermore, absence of functional GSTM1 protected against hearing impairment (p = 0.025, OR 1.81 [1.08-3.03]). CONCLUSION: In TCSs long-term self-reported chemotherapy-induced toxicities are associated with functional polymorphisms in GSTP1 and GSTM1. Hypothetically, absence of GST-M1 leaves more glutathione as substrate for the co-expressed GST-P1. Also intracellular inactivation of pro-apoptotic mediators represents a possible explanation of our findings. Genotyping of these GSTs might be a welcomed step towards a more individualized treatment of patients with metastatic testicular cancer. FAU - Oldenburg, Jan AU - Oldenburg J AD - Department of Clinical Cancer Research, The Norwegian Radiumhospital, Oslo, Norway. janolde@ulrik.uio.no FAU - Kraggerud, Sigrid M AU - Kraggerud SM FAU - Brydøy, Marianne AU - Brydøy M FAU - Cvancarova, Milada AU - Cvancarova M FAU - Lothe, Ragnhild A AU - Lothe RA FAU - Fossa, Sophie D AU - Fossa SD LA - eng PT - Journal Article DEP - 20071227 PL - England TA - J Transl Med JT - Journal of translational medicine JID - 101190741 RN - 0 (Antineoplastic Agents) RN - Q20Q21Q62J (Cisplatin) RN - EC 2.5.1.18 (Glutathione S-Transferase pi) RN - EC 2.5.1.18 (Glutathione Transferase) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antineoplastic Agents/therapeutic use/toxicity MH - Cisplatin/therapeutic use/*toxicity MH - Cross-Sectional Studies MH - Genetic Predisposition to Disease MH - Genotype MH - Glutathione S-Transferase pi/*genetics MH - Glutathione Transferase/*genetics MH - Humans MH - Male MH - Middle Aged MH - Polymorphism, Restriction Fragment Length MH - Retrospective Studies MH - Survivors MH - Testicular Neoplasms/drug therapy/enzymology/*genetics PMC - PMC2245909 EDAT- 2007/12/29 09:00 MHDA- 2008/03/29 09:00 PMCR- 2007/12/27 CRDT- 2007/12/29 09:00 PHST- 2007/11/01 00:00 [received] PHST- 2007/12/27 00:00 [accepted] PHST- 2007/12/29 09:00 [pubmed] PHST- 2008/03/29 09:00 [medline] PHST- 2007/12/29 09:00 [entrez] PHST- 2007/12/27 00:00 [pmc-release] AID - 1479-5876-5-70 [pii] AID - 10.1186/1479-5876-5-70 [doi] PST - epublish SO - J Transl Med. 2007 Dec 27;5:70. doi: 10.1186/1479-5876-5-70. PMID- 38812619 OWN - NLM STAT- MEDLINE DCOM- 20240530 LR - 20240718 IS - 1303-6165 (Electronic) IS - 1300-0144 (Print) IS - 1300-0144 (Linking) VI - 54 IP - 1 DP - 2024 TI - Clinical characteristics and disease course before and after SARS-CoV-2 infection in a large cohort of systemic sclerosis patients. PG - 76-85 LID - 10.55730/1300-0144.5768 [doi] AB - BACKGROUND/AIM: The objective of this study is to evaluate the clinical presentations and adverse outcomes of Coronavirus Disease 2019 (COVID-19) in patients with systemic sclerosis (SSc) and assess the impact of SSc features on the clinical course of COVID-19. MATERIALS AND METHODS: In this multicenter, retrospective study, SSc patients with COVID-19 were included. Clinical features of SSc, along with detailed COVID-19 data, were extracted from medical records and patient interviews. RESULTS: The study included 112 patients (mean age 51.4 ± 12.8 years; 90.2% female). SSc-associated interstitial lung disease (ILD) was evident in 57.1% of the patients. The findings revealed hospitalization in 25.5%, respiratory support in 16.3%, intensive care unit admission in 3.6%, and a mortality rate of 2.7% among SSc patients with COVID-19. Risk factors for respiratory failure, identified through univariate analysis, included ILD (OR: 7.49, 95% CI: 1.63-34.46), ≥1 comorbidity (OR: 4.55, 95% CI: 1.39-14.88), a higher physician global assessment score at the last outpatient visit (OR 2.73, 95% CI: 1.22-6.10), and the use of mycophenolate at the time of infection (OR: 5.16, 95 %CI: 1.79-14.99). Notably, ≥1 comorbidity emerged as the sole significant predictor of the need for respiratory support in COVID-19 (OR: 5.78, 95% CI: 1.14-29.23). In the early post-COVID-19 period, 17% of patients reported the progression of the Raynaud phenomenon, and 10.6% developed new digital ulcers. Furthermore, progression or new onset of dyspnea and cough were detected in 28.3% and 11.4% of patients, respectively. CONCLUSION: This study suggests a potential association between adverse outcomes of COVID-19 and SSc-related ILD, severe disease activity, and the use of mycophenolate. Additionally, it highlights that having comorbidities is an independent risk factor for the need for respiratory support in COVID-19 cases. CI - © TÜBİTAK. FAU - Güler, Aslıhan Avanoglu AU - Güler AA AUID- ORCID: 0000-0001-9866-9797 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Özçimen, Büşra AU - Özçimen B AUID- ORCID: 0000-0002-7931-1367 AD - Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkiye. FAU - Aydoğdu, Mesude Seda AU - Aydoğdu MS AUID- ORCID: 0000-0001-7031-4716 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fırat University, Elazığ, Turkiye. FAU - Sari, Alper AU - Sari A AUID- ORCID: 0000-0002-7509-6604 AD - Department of Rheumatology, Etlik City Hospital, Ankara, Turkiye. FAU - Numune, Aliyeva AU - Numune A AUID- ORCID: 0000-0003-2848-4396 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, İstanbul University, İstanbul, Turkiye. FAU - Ersan, Nazife Tüzün AU - Ersan NT AUID- ORCID: 0009-0004-3220-4339 AD - Department of Internal Medicine, Gazi University Hospital, Ankara, Turkiye. FAU - Çolak, Seda AU - Çolak S AUID- ORCID: 0000-0002-5703-6739 AD - Department of Rheumatology, Gülhane Training and Research Hospital, University of Health Sciences, Ankara, Turkiye. FAU - Karadeniz, Hazan AU - Karadeniz H AUID- ORCID: 0000-0003-4665-3421 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Vasi, İbrahim AU - Vasi İ AUID- ORCID: 0000-0003-1900-5752 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Küçük, Hamit AU - Küçük H AUID- ORCID: 0000-0003-1206-4725 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Yalçinkaya, Yasemin AU - Yalçinkaya Y AUID- ORCID: 0000-0001-9357-0456 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, İstanbul University, İstanbul, Turkiye. FAU - Erden, Abdülsamet AU - Erden A AUID- ORCID: 0000-0002-8084-2018 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Kayaalp, Mehmet AU - Kayaalp M AUID- ORCID: 0000-0001-5424-3161 AD - Department of Internal Medicine, Yıldırım Beyazıt University, Ankara, Turkiye. FAU - Öztürk, Mehmet Akif AU - Öztürk MA AUID- ORCID: 0000-0001-7203-3934 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Göker, Berna AU - Göker B AUID- ORCID: 0000-0001-9242-0907 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. FAU - Omma, Ahmet AU - Omma A AUID- ORCID: 0000-0003-2582-7445 AD - Department of Rheumatology, Ankara City Hospital, Health Sciences University, Ankara, Turkiye. FAU - Yilmaz, Sedat AU - Yilmaz S AUID- ORCID: 0000-0002-4691-3417 AD - Department of Rheumatology, Gülhane Training and Research Hospital, University of Health Sciences, Ankara, Turkiye. FAU - Koca, Süleyman Serdar AU - Koca SS AUID- ORCID: 0000-0003-4995-430X AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fırat University, Elazığ, Turkiye. FAU - Inanç, Murat AU - Inanç M AUID- ORCID: 0000-0002-6376-5583 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, İstanbul University, İstanbul, Turkiye. FAU - Akdoğan, Ali AU - Akdoğan A AUID- ORCID: 0000-0001-7592-3844 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkiye. FAU - Tufan, Abdurrahman AU - Tufan A AUID- ORCID: 0000-0001-6244-9362 AD - Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkiye. AD - Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, Maryland, USA. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20231221 PL - Turkey TA - Turk J Med Sci JT - Turkish journal of medical sciences JID - 9441758 SB - IM MH - Humans MH - *COVID-19/complications/epidemiology MH - *Scleroderma, Systemic/complications/epidemiology MH - Female MH - Male MH - Middle Aged MH - Retrospective Studies MH - Adult MH - *SARS-CoV-2 MH - Risk Factors MH - Lung Diseases, Interstitial/epidemiology MH - Hospitalization/statistics & numerical data MH - Comorbidity MH - Aged MH - Respiratory Insufficiency/epidemiology/etiology MH - Disease Progression PMC - PMC11031159 OTO - NOTNLM OT - COVID-19 OT - Systemic sclerosis OT - interstitial lung disease OT - outcome OT - respiratory support EDAT- 2024/05/30 06:35 MHDA- 2024/05/30 06:36 PMCR- 2023/12/21 CRDT- 2024/05/30 03:46 PHST- 2023/08/16 00:00 [received] PHST- 2024/02/15 00:00 [revised] PHST- 2023/12/21 00:00 [accepted] PHST- 2024/05/30 06:36 [medline] PHST- 2024/05/30 06:35 [pubmed] PHST- 2024/05/30 03:46 [entrez] PHST- 2023/12/21 00:00 [pmc-release] AID - tjmed-54-01-0076 [pii] AID - 10.55730/1300-0144.5768 [doi] PST - epublish SO - Turk J Med Sci. 2023 Dec 21;54(1):76-85. doi: 10.55730/1300-0144.5768. eCollection 2024. PMID- 31977850 OWN - NLM STAT- MEDLINE DCOM- 20200210 LR - 20221005 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 99 IP - 4 DP - 2020 Jan TI - Impact of a systematic evaluation of connective tissue disease on diagnosis approach in patients with interstitial lung diseases. PG - e18589 LID - 10.1097/MD.0000000000018589 [doi] LID - e18589 AB - To date, there is no clear agreement regarding which is the best method to detect a connective tissue disease (CTD) during the initial diagnosis of interstitial lung diseases (ILD). The aim of our study was to explore the impact of a systematic diagnostic strategy to detect CTD-associated ILD (CTD-ILD) in clinical practice, and to clarify the significance of interstitial pneumonia with autoimmune features (IPAF) diagnosis in ILD patients.Consecutive patients evaluated in an ILD Diagnostic Program were divided in 3 groups: IPAF, CTD-ILD, and other ILD forms. Clinical characteristics, exhaustive serologic testing, high resolution computed tomography (HRCT) images, lung biopsy specimens, and follow-up were prospectively collected and analyzed.Among 139 patients with ILD, CTD was present in 21 (15.1%), 24 (17.3%) fulfilled IPAF criteria, and 94 (67.6%) were classified as other ILD forms. Specific systemic autoimmune symptoms such as Raynaud phenomenon (19%), inflammatory arthropathy (66.7%), and skin manifestations (38.1%) were more frequent in CTD-ILD patients than in the other groups (all P < .001). Among autoantibodies, antinuclear antibody was the most frequently found in IPAF (42%), and CTD-ILD (40%) (P = .04). Nonspecific interstitial pneumonia, detected by HRCT scan, was the most frequently seen pattern in patients with IPAF (63.5%), or CTD-ILD (57.1%) (P < .001). In multivariate analysis, a suggestive radiological pattern by HRCT scan (odds ratio [OR] 15.1, 95% confidence interval [CI] 4.7-48.3, P < .001) was the strongest independent predictor of CTD-ILD or IPAF, followed by the presence of clinical features (OR 14.6, 95% CI 4.3-49.5, P < .001), and serological features (OR 12.4, 95% CI 3.5-44.0, P < .001).This systematic diagnostic strategy was useful in discriminating an underlying CTD in patients with ILD. The defined criteria for IPAF are fulfilled by a considerable proportion of patients referred for ILD. FAU - Hernandez-Gonzalez, Fernanda AU - Hernandez-Gonzalez F AD - Servei de Pneumologia, Hospital Clínic, IDIBAPS, Universitat de Barcelona. FAU - Prieto-González, Sergio AU - Prieto-González S AD - Servei de Malalties Autoimmunes, Hospital Clínic. FAU - Brito-Zeron, Pilar AU - Brito-Zeron P AD - Servei de Malalties Autoimmunes, Hospital Clínic. FAU - Cuerpo, Sandra AU - Cuerpo S AD - Servei de Pneumologia, Hospital Clínic, IDIBAPS, Universitat de Barcelona. FAU - Sanchez, Marcelo AU - Sanchez M AD - Servicio de Radiodiagnóstico, Hospital Clínic. FAU - Ramirez, Jose AU - Ramirez J AD - Servicio de Anatomía Patológica, Hospital Clínic. FAU - Agustí, Carlos AU - Agustí C AD - Servei de Pneumologia, Hospital Clínic, IDIBAPS, Universitat de Barcelona. FAU - Lucena, Carmen María AU - Lucena CM AD - Servei de Pneumologia, Hospital Clínic, IDIBAPS, Universitat de Barcelona. FAU - Paradela, Marina AU - Paradela M AD - Servei de Cirurgia Toràcica, Hospital Clínic, IDIBAPS, Universitat de Barcelona, Barcelona. FAU - Grafia, Ignacio AU - Grafia I AD - Servei de Malalties Autoimmunes, Hospital Clínic. FAU - Espinosa, Gerard AU - Espinosa G AD - Servei de Malalties Autoimmunes, Hospital Clínic. FAU - Sellares, Jacobo AU - Sellares J AD - Servei de Pneumologia, Hospital Clínic, IDIBAPS, Universitat de Barcelona. AD - Centro de Investigación Biomedica En Red-Enfermedades Respiratorias (CibeRes, CB06/06/0028), Barcelona, Spain. LA - eng PT - Journal Article PT - Observational Study PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Aged, 80 and over MH - Autoantibodies/immunology MH - Biopsy MH - Connective Tissue Diseases/*complications/*diagnosis/pathology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Lung Diseases, Interstitial/*complications/*diagnosis/pathology MH - Male MH - Middle Aged MH - Tomography, X-Ray Computed PMC - PMC7004576 COIS- This work has been financed with the grant SLT008/18/00176 and the support of the Department of Health of Generalitat de Catalunya, in the call for grants 2019–2021, under a competitive regime, for the financing of different programs and instrumental actions included in the Strategic Research and Innovation Plan in Health 2016–2020. It has also been financed by SEPAR, SOCAP, FUCAP and with the PhD4MD Programme of the Institute for Research in Biomedicine (IRB) Barcelona, Hospital Clinic of Barcelona and the Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). The authors have no conflicts of interest to disclose. EDAT- 2020/01/25 06:00 MHDA- 2020/02/11 06:00 PMCR- 2020/01/24 CRDT- 2020/01/25 06:00 PHST- 2020/01/25 06:00 [entrez] PHST- 2020/01/25 06:00 [pubmed] PHST- 2020/02/11 06:00 [medline] PHST- 2020/01/24 00:00 [pmc-release] AID - 00005792-202001240-00009 [pii] AID - MD-D-19-07317 [pii] AID - 10.1097/MD.0000000000018589 [doi] PST - ppublish SO - Medicine (Baltimore). 2020 Jan;99(4):e18589. doi: 10.1097/MD.0000000000018589. PMID- 30968571 OWN - NLM STAT- MEDLINE DCOM- 20200129 LR - 20200129 IS - 1756-185X (Electronic) IS - 1756-1841 (Linking) VI - 22 IP - 8 DP - 2019 Aug TI - Overlap myositis, a distinct entity beyond primary inflammatory myositis: A retrospective analysis of a large cohort from the REMICAM registry. PG - 1393-1401 LID - 10.1111/1756-185X.13559 [doi] AB - BACKGROUND: Inflammatory idiopathic myositis (IIM) comprises a heterogeneous group of systemic muscular diseases that can occur together with other connective tissue diseases (CTD), named overlap myositis (OM). The question of whether OM is a distinct entity still remains controversial. AIM: The present study was conducted to assess the clinical and prognostic differences between patients diagnosed with OM, primary polymyositis (PM) and primary dermatomyositis (DM). METHOD: The study consists of a retrospective longitudinal and multicenter series of IIM patients. Patients were classified as OM, PM and DM. Overlap myositis was defined as patients fulfilling criteria for IIM plus criteria for other CTD (namely systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis and primary Sjögren's syndrome). RESULT: A total of 342 patients were included (98 OM, 137 PM and 107 DM). Overlap myositis patients, in comparison with PM and DM, showed significant differences, with more extramuscular involvement, particularly more arthritis (66%, 34.6% and 48.1%, respectively), puffy fingers (49.5%, 11.1% and 24.3%), sclerodactyly (45.4%, 2.2% and 2%), dysphagia (41.8%, 18.2% and 26.4%), Raynaud phenomenon (65.3%, 16.9% and 19.8%), leucopenia (28.9%, 2.2% and 8.4%), thrombocytopenia (8.2%, 2.2% and 1.9%), interstitial lung disease (ILD) (48%, 35% and 30.8%), renal manifestations (13.4%, 3.7% and 1.9%), and more severe infections (41.3%, 26.7% and 21%). No significant differences were found in survival between groups in log rank test (P = 0.106). Multivariate adjusted survival analyses revealed a worse prognosis for severe infections, ILD and baseline elevation of acute phase reactants. CONCLUSION: Overlap myositis stands out as a distinct entity as compared to PM and DM, featuring more extramuscular involvement and more severe infections. Close monitoring is recommended in this subset for early detection and treatment of possible complications. CI - © 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. FAU - Nuño-Nuño, Laura AU - Nuño-Nuño L AUID- ORCID: 0000-0001-7152-0458 AD - Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. FAU - Joven, Beatriz Esther AU - Joven BE AD - Servicio de Reumatología, Hospital Universitario Doce de Octubre, Madrid, Spain. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatología, Hospital Universitario Doce de Octubre, Madrid, Spain. FAU - Maldonado-Romero, Valentina AU - Maldonado-Romero V AD - Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Larena-Grijalba, Carmen AU - Larena-Grijalba C AD - Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - Llorente Cubas, Irene AU - Llorente Cubas I AD - Servicio de Reumatología, Hospital Universitario La Princesa, Madrid, Spain. FAU - Tomero, Eva AU - Tomero E AD - Servicio de Reumatología, Hospital Universitario La Princesa, Madrid, Spain. FAU - Barbadillo-Mateos, María Carmen AU - Barbadillo-Mateos MC AD - Servicio de Reumatología, Hospital Universitario Puerta de Hierro, Madrid, Spain. FAU - García de la Peña Lefebvre, Paloma AU - García de la Peña Lefebvre P AD - Servicio de Reumatología, Hospital Universitario Madrid Norte Sanchinarro, Madrid, Spain. FAU - Ruiz-Gutiérrez, Lucía AU - Ruiz-Gutiérrez L AD - Servicio de Reumatología, Hospital Universitario Infantil Niño Jesús, Madrid, Spain. FAU - López-Robledillo, Juan Carlos AU - López-Robledillo JC AD - Servicio de Reumatología, Hospital Universitario Infantil Niño Jesús, Madrid, Spain. FAU - Moruno-Cruz, Henry AU - Moruno-Cruz H AD - Servicio de Reumatología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Spain. FAU - Pérez, Ana AU - Pérez A AD - Servicio de Reumatología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Spain. FAU - Cobo-Ibáñez, Tatiana AU - Cobo-Ibáñez T AD - Servicio de Reumatología, Hospital Universitario Infanta Sofía, Universidad Europea de Madrid, Madrid, Spain. FAU - Almodóvar, Raquel AU - Almodóvar R AD - Servicio de Reumatología, Hospital Universitario Fundación Alcorcón, Alcorcón, Madrid, Spain. FAU - Lojo, Leticia AU - Lojo L AD - Servicio de Reumatología, Hospital Universitario Infanta Leonor, Madrid, Spain. FAU - García de Yébenes, María Jesús AU - García de Yébenes MJ AD - Instituto de Salud Musculoesquelética, Madrid, Spain. FAU - López-Longo, Francisco Javier AU - López-Longo FJ AD - Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. LA - eng GR - Merck Sharp and Dohme/ PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20190410 PL - England TA - Int J Rheum Dis JT - International journal of rheumatic diseases JID - 101474930 RN - 0 (Immunologic Factors) SB - IM MH - Adult MH - Aged MH - Dermatomyositis/classification/*diagnosis/drug therapy MH - Diagnosis, Differential MH - Female MH - Humans MH - Immunologic Factors/therapeutic use MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Polymyositis/classification/*diagnosis/drug therapy MH - Predictive Value of Tests MH - Prognosis MH - Registries MH - Retrospective Studies MH - Risk Factors MH - Spain MH - Terminology as Topic OTO - NOTNLM OT - dermatomyositis OT - inflammatory idiopathic myositis OT - mortality OT - overlap myositis OT - polymyositis EDAT- 2019/04/11 06:00 MHDA- 2020/01/30 06:00 CRDT- 2019/04/11 06:00 PHST- 2018/07/25 00:00 [received] PHST- 2019/02/16 00:00 [revised] PHST- 2019/03/04 00:00 [accepted] PHST- 2019/04/11 06:00 [pubmed] PHST- 2020/01/30 06:00 [medline] PHST- 2019/04/11 06:00 [entrez] AID - 10.1111/1756-185X.13559 [doi] PST - ppublish SO - Int J Rheum Dis. 2019 Aug;22(8):1393-1401. doi: 10.1111/1756-185X.13559. Epub 2019 Apr 10. PMID- 30459414 OWN - NLM STAT- MEDLINE DCOM- 20190520 LR - 20200225 IS - 1476-5438 (Electronic) IS - 1018-4813 (Print) IS - 1018-4813 (Linking) VI - 27 IP - 3 DP - 2019 Mar TI - A rare regulatory variant in the MEF2D gene affects gene regulation and splicing and is associated with a SLE sub-phenotype in Swedish cohorts. PG - 432-441 LID - 10.1038/s41431-018-0297-x [doi] AB - Systemic lupus erythematosus (SLE) is an autoimmune disorder with heterogeneous clinical presentation and complex etiology involving the interplay between genetic, epigenetic, environmental and hormonal factors. Many common SNPs identified by genome wide-association studies (GWAS) explain only a small part of the disease heritability suggesting the contribution from rare genetic variants, undetectable in GWAS, and complex epistatic interactions. Using targeted re-sequencing of coding and conserved regulatory regions within and around 215 candidate genes selected on the basis of their known role in autoimmunity and genes associated with canine immune-mediated diseases, we identified a rare regulatory variant rs200395694:G > T located in intron 4 of the MEF2D gene encoding the myocyte-specific enhancer factor 2D transcription factor and associated with SLE in Swedish cohorts (504 SLE patients and 839 healthy controls, p = 0.014, CI = 1.1-10). Fisher's exact test revealed an association between the genetic variant and a triad of disease manifestations including Raynaud, anti-U1-ribonucleoprotein (anti-RNP), and anti-Smith (anti-Sm) antibodies (p = 0.00037) among the patients. The DNA-binding activity of the allele was further studied by EMSA, reporter assays, and minigenes. The region has properties of an active cell-specific enhancer, differentially affected by the alleles of rs200395694:G > T. In addition, the risk allele exerts an inhibitory effect on the splicing of the alternative tissue-specific isoform, and thus may modify the target gene set regulated by this isoform. These findings emphasize the potential of dissecting traits of complex diseases and correlating them with rare risk alleles with strong biological effects. FAU - Farias, Fabiana H G AU - Farias FHG AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. ffarias@wustl.edu. AD - Department of Psychiatry, Washington University School of Medicine, St. Louis, MO, 63110, USA. ffarias@wustl.edu. FAU - Dahlqvist, Johanna AU - Dahlqvist J AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. FAU - Kozyrev, Sergey V AU - Kozyrev SV AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. FAU - Leonard, Dag AU - Leonard D AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Wilbe, Maria AU - Wilbe M AD - Department of Animal Breeding and Genetics, Swedish University of Agricultural Sciences (SLU), Box 7023, SE-750 07, Uppsala, Sweden. AD - Science for Life Laboratory, Department of Immunology, Genetics and Pathology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. FAU - Abramov, Sergei N AU - Abramov SN AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. AD - Institute of Fundamental Medicine and Biology, Kazan Federal University, Kazan, 420008, Russia. FAU - Alexsson, Andrei AU - Alexsson A AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Pielberg, Gerli R AU - Pielberg GR AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. FAU - Hansson-Hamlin, Helene AU - Hansson-Hamlin H AD - Department of Clinical Sciences, Swedish University of Agricultural Sciences (SLU), Box 7054, SE-750 07, Uppsala, Sweden. FAU - Andersson, Göran AU - Andersson G AD - Department of Animal Breeding and Genetics, Swedish University of Agricultural Sciences (SLU), Box 7023, SE-750 07, Uppsala, Sweden. FAU - Tandre, Karolina AU - Tandre K AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Bengtsson, Anders A AU - Bengtsson AA AD - Department of Clinical Sciences Lund, Lund University, Skane University Hospital, SE-221 00, Lund, Sweden. FAU - Sjöwall, Christopher AU - Sjöwall C AUID- ORCID: 0000-0003-0900-2048 AD - Department of Clinical and Experimental Medicine, Rheumatology/Division of Neuro and Inflammation Sciences, Linköping University, SE-581 85, Linköping, Sweden. FAU - Svenungsson, Elisabet AU - Svenungsson E AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, SE-171 76, Stockholm, Sweden. FAU - Gunnarsson, Iva AU - Gunnarsson I AD - Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, SE-171 76, Stockholm, Sweden. FAU - Rantapää-Dahlqvist, Solbritt AU - Rantapää-Dahlqvist S AD - Department of Public Health and Clinical Medicine/Rheumatology, Umeå University, SE-901 85, Umeå, Sweden. FAU - Syvänen, Ann-Christine AU - Syvänen AC AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. AD - Department of Medical Sciences, Molecular Medicine and Science for Life Laboratory, Uppsala University, SE-754 11, Uppsala, Sweden. FAU - Sandling, Johanna K AU - Sandling JK AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Eloranta, Maija-Leena AU - Eloranta ML AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Rönnblom, Lars AU - Rönnblom L AD - Science for Life Laboratory, Department of Medical Sciences, Section of Rheumatology, Uppsala University, SE-751 85, Uppsala, Sweden. FAU - Lindblad-Toh, Kerstin AU - Lindblad-Toh K AD - Science for Life Laboratory, Department of Medical Biochemistry and Microbiology, Uppsala University, Box 582, SE-751 24, Uppsala, Sweden. kersli@broadinstitute.org. AD - Broad Institute, Cambridge, 7 Cambridge Center, Cambridge, MA, 02142, USA. kersli@broadinstitute.org. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20181120 PL - England TA - Eur J Hum Genet JT - European journal of human genetics : EJHG JID - 9302235 RN - 0 (MEF2 Transcription Factors) RN - 0 (MEF2D protein, human) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Child MH - Female MH - HEK293 Cells MH - Humans MH - Lupus Erythematosus, Systemic/*genetics MH - MEF2 Transcription Factors/genetics/metabolism MH - Male MH - Middle Aged MH - *Phenotype MH - Polymorphism, Single Nucleotide MH - Protein Binding MH - *RNA Splicing PMC - PMC6460566 COIS- The authors declare that they have no conflict of interest. EDAT- 2018/11/22 06:00 MHDA- 2019/05/21 06:00 PMCR- 2018/11/20 CRDT- 2018/11/22 06:00 PHST- 2018/04/28 00:00 [received] PHST- 2018/10/09 00:00 [accepted] PHST- 2018/08/26 00:00 [revised] PHST- 2018/11/22 06:00 [pubmed] PHST- 2019/05/21 06:00 [medline] PHST- 2018/11/22 06:00 [entrez] PHST- 2018/11/20 00:00 [pmc-release] AID - 10.1038/s41431-018-0297-x [pii] AID - 297 [pii] AID - 10.1038/s41431-018-0297-x [doi] PST - ppublish SO - Eur J Hum Genet. 2019 Mar;27(3):432-441. doi: 10.1038/s41431-018-0297-x. Epub 2018 Nov 20. PMID- 30206672 OWN - NLM STAT- MEDLINE DCOM- 20191001 LR - 20191001 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 38 IP - 12 DP - 2018 Dec TI - High burden of skin sclerosis is associated with severe organ involvement in patients with systemic sclerosis and systemic sclerosis overlap syndrome. PG - 2279-2288 LID - 10.1007/s00296-018-4156-4 [doi] AB - The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013-November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. TA-mRSS was classified as low, intermediate, or high, and then compared between groups. Correlation between the arithmetic mean of laboratory tests and TA-mRSS was assessed by multiple linear regression analysis. A total of 118 patients, with 81.4% women, median (IQR) age 49.8 (43.8, 55.1) years, disease duration from onset of non-Raynaud symptoms to first visit of 3.3 (1, 6.8) years, 78% dcSSc, and 75.3% anti-Scl-70 positivity, were analyzed. TA-mRSS over 1 year ranged from 0 to 37.44. The high skin sclerosis burden group had a median TA-mRSS > 7.26 (> 67th percentile). Patients with high TA-mRSS were dcSSc, high initial and average mRSS, and had tendon friction rub, digital ischemic complications, usual interstitial pneumonia, diastolic dysfunction, gastrointestinal dysmotility, and low serum albumin. In multiple linear regression analysis, the arithmetic mean of hemoglobin (B = - 1.007, 95% CI - 1.779 to - 0.236), erythrocyte sedimentation rate (B = - 0.078, 95% CI - 0.126 to - 0.029), serum glutamic oxaloacetic transaminase (B = 0.073, 95% CI 0.026-0.12), creatine phosphokinase (B = 0.012, 95% CI 0.003-0.021), and albumin (B = - 4.117, 95% CI - 6.958 to - 1.276) were associated with TA-mRSS. This study found a higher cumulative course of mRSS over a 1-year period to be significantly associated with severe internal organ involvement. FAU - Wannarong, Thapat AU - Wannarong T AUID- ORCID: 0000-0003-1839-1903 AD - Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. FAU - Muangchan, Chayawee AU - Muangchan C AUID- ORCID: 0000-0002-5412-4055 AD - Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand. chayawee.mua@mahidol.ac.th. LA - eng PT - Journal Article PT - Observational Study DEP - 20180911 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Biomarkers/blood MH - Disease Progression MH - Female MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Prospective Studies MH - Remission Induction MH - Scleroderma, Diffuse/blood/*complications/drug therapy/pathology MH - Scleroderma, Limited/blood/*complications/drug therapy/pathology MH - Severity of Illness Index MH - Skin/*pathology MH - Syndrome MH - Thailand MH - Time Factors MH - Treatment Outcome OTO - NOTNLM OT - Modified Rodnan skin score OT - Scleroderma OT - Severe organ involvement OT - Skin sclerosis OT - Systemic EDAT- 2018/09/13 06:00 MHDA- 2019/10/02 06:00 CRDT- 2018/09/13 06:00 PHST- 2018/05/14 00:00 [received] PHST- 2018/09/07 00:00 [accepted] PHST- 2018/09/13 06:00 [pubmed] PHST- 2019/10/02 06:00 [medline] PHST- 2018/09/13 06:00 [entrez] AID - 10.1007/s00296-018-4156-4 [pii] AID - 10.1007/s00296-018-4156-4 [doi] PST - ppublish SO - Rheumatol Int. 2018 Dec;38(12):2279-2288. doi: 10.1007/s00296-018-4156-4. Epub 2018 Sep 11. PMID- 18203194 OWN - NLM STAT- MEDLINE DCOM- 20080313 LR - 20200930 IS - 1552-4833 (Electronic) IS - 1552-4825 (Linking) VI - 146A IP - 4 DP - 2008 Feb 15 TI - Carotid artery dissection in an adult with the Simpson-Golabi-Behmel syndrome. PG - 464-7 LID - 10.1002/ajmg.a.32154 [doi] AB - We report on the case of a 44-year-old man affected with the Simpson-Golabi-Behmel syndrome (SGBS) (OMIM 312870) presenting with ischemic stroke due to a dissection of the right internal carotid. Molecular genetic analysis revealed the p.Gly556Arg mutation in exon 8 of the gene encoding glypican 3 (GPC3). This is the second case of a GPC3 missense mutation to be reported. The only risk factor found in this patient was carotid redundancy, a deformation that is significantly associated with spontaneous carotid dissection. The natural history of SGBS in adults is poorly known, and this case raises the question of a possible vascular risk associated with the disease. FAU - Pénisson-Besnier, Isabelle AU - Pénisson-Besnier I AD - Département de Neurologie, CHU Angers, Angers, France. FAU - Lebouvier, Thibaud AU - Lebouvier T FAU - Moizard, Marie-Pierre AU - Moizard MP FAU - Ferré, Marc AU - Ferré M FAU - Barth, Magalie AU - Barth M FAU - Marc, Guillaume AU - Marc G FAU - Raynaud, Martine AU - Raynaud M FAU - Bonneau, Dominique AU - Bonneau D LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Am J Med Genet A JT - American journal of medical genetics. Part A JID - 101235741 RN - 0 (GPC3 protein, human) RN - 0 (Glypicans) SB - IM MH - Abnormalities, Multiple/*genetics MH - Adult MH - Base Sequence MH - Carotid Artery, Internal, Dissection/*complications/genetics MH - Cleft Palate/complications/genetics MH - Cryptorchidism/complications/genetics MH - DNA Mutational Analysis MH - Fetal Macrosomia/genetics MH - Glypicans/genetics MH - Humans MH - Male MH - Polydactyly/complications/genetics MH - Syndrome EDAT- 2008/01/19 09:00 MHDA- 2008/03/14 09:00 CRDT- 2008/01/19 09:00 PHST- 2008/01/19 09:00 [pubmed] PHST- 2008/03/14 09:00 [medline] PHST- 2008/01/19 09:00 [entrez] AID - 10.1002/ajmg.a.32154 [doi] PST - ppublish SO - Am J Med Genet A. 2008 Feb 15;146A(4):464-7. doi: 10.1002/ajmg.a.32154. PMID- 23877355 OWN - NLM STAT- MEDLINE DCOM- 20130926 LR - 20250529 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 92 IP - 4 DP - 2013 Jul TI - The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. PG - 223-243 LID - 10.1097/MD.0b013e31829d08f9 [doi] AB - The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high frequencies of black race, severe onset, distal weakness, falling episodes, Raynaud phenomenon, cardiac involvement, high CK levels, chronic disease course, frequent hospitalization, and wheelchair use. Characteristic features of the anti-Mi-2 subgroup included Hispanic ethnicity, classic dermatomyositis and malar rashes, high CK levels, and very low mortality. Finally, the most common features of patients without any currently defined MSA or myositis-associated autoantibodies included linear extensor erythema, arthralgia, and a monocyclic disease course. Several demographic and clinical features were shared between juvenile and adult idiopathic inflammatory myopathy subgroups, but with several important differences. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct autoantibody phenotypes defined by varying clinical and demographic characteristics, laboratory features, and outcomes. FAU - Rider, Lisa G AU - Rider LG AD - From Environmental Autoimmunity Group (LGR, MS, GM, FWM), Program of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, DHHS, Bethesda, Maryland; Department of Epidemiology and Biostatistics (MS, MMR) and Division of Rheumatology, Department of Medicine (GM), George Washington University School of Medicine, Washington, DC; Veteran's Affairs Medical Center (INT), University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, United States; and IWK Health Center and Dalhousie University (AMH), Halifax, Nova Scotia, Canada. FAU - Shah, Mona AU - Shah M FAU - Mamyrova, Gulnara AU - Mamyrova G FAU - Huber, Adam M AU - Huber AM FAU - Rice, Madeline Murguia AU - Rice MM FAU - Targoff, Ira N AU - Targoff IN FAU - Miller, Frederick W AU - Miller FW CN - Childhood Myositis Heterogeneity Collaborative Study Group LA - eng GR - R01 DK088114/DK/NIDDK NIH HHS/United States GR - Z01 ES101074/ImNIH/Intramural NIH HHS/United States GR - Z99 ES999999/ImNIH/Intramural NIH HHS/United States GR - ZIA ES101074/ImNIH/Intramural NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Antibodies, Antinuclear) RN - 0 (Autoantibodies) SB - IM MH - Adolescent MH - Adult MH - Antibodies, Antinuclear/analysis MH - Autoantibodies/*genetics/immunology MH - Autoimmune Diseases/genetics/immunology MH - Child MH - Child, Preschool MH - Chronic Disease MH - Female MH - Humans MH - Logistic Models MH - Male MH - Models, Statistical MH - Multivariate Analysis MH - Myositis/*genetics/*immunology MH - Phenotype PMC - PMC3721421 MID - NIHMS442755 EDAT- 2013/07/24 06:00 MHDA- 2013/09/27 06:00 CRDT- 2013/07/24 06:00 PHST- 2013/07/24 06:00 [entrez] PHST- 2013/07/24 06:00 [pubmed] PHST- 2013/09/27 06:00 [medline] AID - 00005792-201307000-00005 [pii] AID - 10.1097/MD.0b013e31829d08f9 [doi] PST - ppublish SO - Medicine (Baltimore). 2013 Jul;92(4):223-243. doi: 10.1097/MD.0b013e31829d08f9. PMID- 32060775 OWN - NLM STAT- MEDLINE DCOM- 20200907 LR - 20200907 IS - 1875-8312 (Electronic) IS - 1569-5794 (Linking) VI - 36 IP - 5 DP - 2020 May TI - The relationship between global longitudinal strain and pulmonary function tests in patients with scleroderma and normal ejection fraction and pulmonary artery pressure: a case-control study. PG - 883-888 LID - 10.1007/s10554-020-01788-7 [doi] AB - This study examined the relationship between global longitudinal strain (GLS) and pulmonary function tests (PFT) in patients with systemic sclerosis (SS) and normal ejection fraction (EF) and pulmonary artery pressure (PAP) and healthy controls. Sixty patients in two groups underwent extensive screening, including echocardiography, physical examination, the modified Rodnan Skin Score, and pulmonary function tests. Pulmonary interstitial disease was diagnosed by the pulmonary function test and by CT scan in case of indication. GLS score was computed as the mean peak systolic strain for 17 segments. The mean GLS score was - 18.36 ± 2.1 in the case group and - 20.66 ± 1.6 in the control group (P value < 0.001). GLS scores had a significant inverse relationship with the forced expiratory volume in one second (FEV1) to forced vital capacity (FVC) ratio (P value = 0.049) and both FEV and FVC in patients younger than 35 years old (P = 0.046 and 0.049, respectively). GLS scores had no significant relationship with time elapsed since the onset of skin manifestations, and Raynaud phenomenon, Rodnan score, EF, systolic PAP, or the six-minute walk test results. The patients' six-minute walk test had a significant positive relationship with FVC and right ventricular end diastolic diameter (P value = 0.018 and 0.047, respectively). According to our findings, GLS is significantly lower in patients with SS (with normal EF & PAP) than in healthy individuals. It is also related with certain pulmonary function indices including FEV1/FVC. The reduction in GLS is associated with reduced pulmonary function strength. FAU - Hajsadeghi, Shokoufeh AU - Hajsadeghi S AD - Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology & Metabolism, Iran University of Medical Sciences, Tehran, Iran. FAU - Mirshafiee, Shayan AU - Mirshafiee S AD - Tehran University of Medical Sciences, Tehran, Iran. FAU - Pazoki, Mahboubeh AU - Pazoki M AD - Department of Cardiovascular Disease, Hazrat-e Rasool General Hospital, Iran University of Medical Sciences, Tehran, Iran. FAU - Moradians, Vahan AU - Moradians V AD - Pulmonology Department, Hazrat-e-Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. FAU - Mansouri, Pejman AU - Mansouri P AD - Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran. hart.researchgroup@gmail.com. FAU - Kianmehr, Nahid AU - Kianmehr N AD - Internal Medicine Department, Iran University of Medical Sciences, Tehran, Iran. FAU - Iranpour, Aida AU - Iranpour A AD - Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology & Metabolism, Iran University of Medical Sciences, Tehran, Iran. LA - eng PT - Journal Article DEP - 20200214 PL - United States TA - Int J Cardiovasc Imaging JT - The international journal of cardiovascular imaging JID - 100969716 SB - IM MH - Adult MH - *Arterial Pressure MH - Case-Control Studies MH - Exercise Tolerance MH - Female MH - Forced Expiratory Volume MH - Humans MH - Lung/*blood supply/physiopathology MH - Lung Diseases, Interstitial/diagnosis/*etiology/physiopathology MH - Male MH - Middle Aged MH - *Myocardial Contraction MH - Prognosis MH - Pulmonary Arterial Hypertension/diagnosis/*etiology/physiopathology MH - Pulmonary Artery/*physiopathology MH - Risk Factors MH - Scleroderma, Systemic/*complications/diagnosis/physiopathology MH - Ventricular Dysfunction, Left/diagnostic imaging/*etiology/physiopathology MH - *Ventricular Function, Left MH - Ventricular Function, Right MH - Vital Capacity OTO - NOTNLM OT - Ejection fraction OT - Global longitudinal strain OT - Pulmonary artery pressure OT - Pulmonary function tests OT - Scleroderma EDAT- 2020/02/16 06:00 MHDA- 2020/09/08 06:00 CRDT- 2020/02/16 06:00 PHST- 2019/10/05 00:00 [received] PHST- 2020/01/31 00:00 [accepted] PHST- 2020/02/16 06:00 [pubmed] PHST- 2020/09/08 06:00 [medline] PHST- 2020/02/16 06:00 [entrez] AID - 10.1007/s10554-020-01788-7 [pii] AID - 10.1007/s10554-020-01788-7 [doi] PST - ppublish SO - Int J Cardiovasc Imaging. 2020 May;36(5):883-888. doi: 10.1007/s10554-020-01788-7. Epub 2020 Feb 14. PMID- 32144182 OWN - NLM STAT- MEDLINE DCOM- 20210802 LR - 20210802 IS - 2332-7812 (Electronic) IS - 2332-7812 (Linking) VI - 7 IP - 3 DP - 2020 May TI - Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis. LID - 10.1212/NXI.0000000000000694 [doi] LID - e694 AB - OBJECTIVE: To describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients. METHODS: We reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. We analyzed the effect of the deregulated cytokines in vitro (fibroblasts, endothelial cells, and muscle cells) and in vivo. RESULTS: All patients with BCIM-SSc presented with muscle weakness involving cervical and proximal muscles of the upper limbs plus Raynaud syndrome, telangiectasia and/or sclerodactilia, hypotonia of the esophagus, and interstitial lung disease. Immunosuppressive treatment stopped the progression of the disease. Muscle biopsy showed pathologic changes including the presence of necrotic fibers, fibrosis, and reduced capillary number and size. Cytokines involved in inflammation, angiogenesis, and fibrosis were deregulated. Thrombospondin-1 (TSP-1), which participates in all these 3 processes, was upregulated in patients with BCIM-SSc. In vitro, TSP-1 and serum of patients with BCIM-SSc promoted proliferation and upregulation of collagen, fibronectin, and transforming growth factor beta in fibroblasts. TSP-1 disrupted vascular network, decreased muscle differentiation, and promoted hypotrophic myotubes. In vivo, TSP-1 increased fibrotic tissue and profibrotic macrophage infiltration in the muscle. CONCLUSIONS: Patients with SSc may present with a clinically and pathologically distinct myopathy. A prompt and correct diagnosis has important implications for treatment. Finally, TSP-1 may participate in the pathologic changes observed in muscle. CI - Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. FAU - Suárez-Calvet, Xavier AU - Suárez-Calvet X AUID- ORCID: 0000-0002-5314-6607 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Alonso-Pérez, Jorge AU - Alonso-Pérez J AUID- ORCID: 0000-0001-8866-5186 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Castellví, Ivan AU - Castellví I AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Carrasco-Rozas, Ana AU - Carrasco-Rozas A AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Fernández-Simón, Esther AU - Fernández-Simón E AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Zamora, Carlos AU - Zamora C AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Martínez-Martínez, Laura AU - Martínez-Martínez L AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Alonso-Jiménez, Alicia AU - Alonso-Jiménez A AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Rojas-García, Ricardo AU - Rojas-García R AUID- ORCID: 0000-0003-1411-5573 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Turón, Joana AU - Turón J AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Querol, Luis AU - Querol L AUID- ORCID: 0000-0002-4289-8264 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - de Luna, Noemi AU - de Luna N AUID- ORCID: 0000-0002-4342-794X AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Milena-Millan, Ana AU - Milena-Millan A AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Corominas, Héctor AU - Corominas H AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Castillo, Diego AU - Castillo D AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Cortés-Vicente, Elena AU - Cortés-Vicente E AUID- ORCID: 0000-0002-1428-1072 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Illa, Isabel AU - Illa I AUID- ORCID: 0000-0002-2186-2684 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. FAU - Gallardo, Eduard AU - Gallardo E AUID- ORCID: 0000-0002-3942-3436 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. jdiazm@santpau.cat egallardo@santpau.cat. FAU - Díaz-Manera, Jordi AU - Díaz-Manera J AUID- ORCID: 0000-0003-2941-7988 AD - From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200306 PL - United States TA - Neurol Neuroimmunol Neuroinflamm JT - Neurology(R) neuroimmunology & neuroinflammation JID - 101636388 RN - 0 (Thrombospondin 1) SB - IM MH - Adult MH - Aged MH - Arm MH - Female MH - Humans MH - Middle Aged MH - *Muscle Weakness/immunology/metabolism/pathology/physiopathology MH - *Muscle, Skeletal/immunology/metabolism/pathology/physiopathology MH - *Myositis/immunology/metabolism/pathology/physiopathology MH - Neck Muscles/immunology/metabolism/pathology/physiopathology MH - *Scleroderma, Systemic/immunology/metabolism/pathology/physiopathology MH - Thrombospondin 1/*metabolism PMC - PMC7136050 EDAT- 2020/03/08 06:00 MHDA- 2021/08/03 06:00 PMCR- 2020/03/06 CRDT- 2020/03/08 06:00 PHST- 2019/09/18 00:00 [received] PHST- 2020/01/02 00:00 [accepted] PHST- 2020/03/08 06:00 [entrez] PHST- 2020/03/08 06:00 [pubmed] PHST- 2021/08/03 06:00 [medline] PHST- 2020/03/06 00:00 [pmc-release] AID - 7/3/e694 [pii] AID - NEURIMMINFL2019024042 [pii] AID - 10.1212/NXI.0000000000000694 [doi] PST - epublish SO - Neurol Neuroimmunol Neuroinflamm. 2020 Mar 6;7(3):e694. doi: 10.1212/NXI.0000000000000694. Print 2020 May. PMID- 39486240 OWN - NLM STAT- MEDLINE DCOM- 20241129 LR - 20241204 IS - 1535-6280 (Electronic) IS - 0146-2806 (Linking) VI - 50 IP - 1 DP - 2025 Jan TI - Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review. PG - 102891 LID - S0146-2806(24)00526-7 [pii] LID - 10.1016/j.cpcardiol.2024.102891 [doi] AB - Juvenile onset systemic sclerosis is a rare chronic multisystem connective tissue disease characterized by skin induration, microangiopathy, autoimmune disturbances and widespread fibrosis of internal organs. Primary cardiac involvement in systemic sclerosis (SSc) is associated with a variable phenotype, including heart failure and arrhythmias, which lead to poor short-term prognosis. Isolated heart transplantation is a rare approach for the treatment of advanced heart failure in patients with systemic sclerosis. We report on two juvenile SSc patients receiving cardiac transplantation due to heart failure with malignant arrhythmias. One patient presented with severe dilated cardiomyopathy with recurrent ventricular tachycardia. Following the appearance of Raynaud phenomenon, he was subsequently diagnosed a rare form of systemic sclerosis sine scleroderma, without cutaneous manifestations or other organs involved. His cardiac condition was unresponsive to antiarrhythmic therapy and immunosuppression used to treat SSc, therefore he underwent successful heart transplantation. The second patient presented diffuse scleroderma with mild pulmonary, esophageal and renal involvement. While extracardiac manifestations were effectively kept under control with immunosuppressive therapy, cardiac involvement rapidly progressed with detection of fibrosis at cardiac magnetic resonance imaging and appearance of severe ventricular arrhythmia. Herein, an extensive multidisciplinary evaluation was pivotal in defining the entity and clinical stability of extracardiac involvement, and thus the patient could profit from heart transplantation. Our experience highlights the importance of considering heart transplantation in carefully selected SSc patients with primary cardiac involvement as a lifesaving procedure. CI - Copyright © 2024 Elsevier Inc. All rights reserved. FAU - Bacich, Daniela AU - Bacich D AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: daniela.bacich@aopd.veneto.it. FAU - Tessari, Chiara AU - Tessari C AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: chiara.tessari@unipd.it. FAU - Andreis, Marco AU - Andreis M AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: marco.andreis@studenti.unipd.it. FAU - Geatti, Veronica AU - Geatti V AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: veronica.geatti@studenti.unipd.it. FAU - Cattapan, Irene AU - Cattapan I AD - Pediatric Cardiology Unit, Department of Women's and Children's Health, Padova University Hospital, via Giustiniani 2, 35128 Padova, Italy. Electronic address: irene.cattapan@aopd.veneto.it. FAU - Pradegan, Nicola AU - Pradegan N AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: nicola.pradegan@aopd.veneto.it. FAU - Fedrigo, Marny AU - Fedrigo M AD - Cardiovascular Pathology, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: marny.fedigo@aopd.veneto.it. FAU - Di Salvo, Giovanni AU - Di Salvo G AD - Pediatric Cardiology Unit, Department of Women's and Children's Health, Padova University Hospital, via Giustiniani 2, 35128 Padova, Italy. Electronic address: giovanni.disalvo@unipd.it. FAU - Toscano, Giuseppe AU - Toscano G AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: giuseppe.toscano@aopd.veneto.it. FAU - Angelini, Annalisa AU - Angelini A AD - Cardiovascular Pathology, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: annalisa.angelini@unipd.it. FAU - Gerosa, Gino AU - Gerosa G AD - Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, Padova University Hospital, via Giustiniani 2, 35128, Padova, Italy. Electronic address: gino.gerosa@unipd.it. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20241031 PL - Netherlands TA - Curr Probl Cardiol JT - Current problems in cardiology JID - 7701802 RN - 0 (Immunosuppressive Agents) RN - Juvenile-onset scleroderma SB - IM MH - Humans MH - *Heart Transplantation/methods MH - Male MH - *Scleroderma, Systemic/complications/diagnosis MH - Cardiomyopathy, Dilated/surgery/etiology/diagnosis/physiopathology MH - Heart Failure/etiology/diagnosis MH - Adolescent MH - Immunosuppressive Agents/therapeutic use MH - Scleroderma, Localized OTO - NOTNLM OT - Heart transplantation OT - Juvenile OT - Scleroderma OT - Systemic sclerosis COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2024/11/02 23:14 MHDA- 2024/11/30 11:17 CRDT- 2024/11/01 19:05 PHST- 2024/10/17 00:00 [received] PHST- 2024/10/17 00:00 [accepted] PHST- 2024/11/30 11:17 [medline] PHST- 2024/11/02 23:14 [pubmed] PHST- 2024/11/01 19:05 [entrez] AID - S0146-2806(24)00526-7 [pii] AID - 10.1016/j.cpcardiol.2024.102891 [doi] PST - ppublish SO - Curr Probl Cardiol. 2025 Jan;50(1):102891. doi: 10.1016/j.cpcardiol.2024.102891. Epub 2024 Oct 31. PMID- 34718447 OWN - NLM STAT- MEDLINE DCOM- 20220602 LR - 20220726 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 61 IP - 6 DP - 2022 May 30 TI - A pilot study to evaluate the safety and efficacy of treprostinil in the treatment of calcinosis in systemic sclerosis. PG - 2441-2449 LID - 10.1093/rheumatology/keab810 [doi] AB - OBJECTIVES: We evaluated the safety and efficacy of oral treprostinil in preventing progression of SSc-associated calcinosis. METHODS: This prospective open-label study enrolled 12 SSc patients meeting 2013 ACR/EULAR classification criteria with confirmed clinical and radiographic evidence of one or more calcinosis deposit in the hands. Patients received oral treprostinil for 1 year. Primary endpoints were safety/tolerability and percentage of patients without radiographic progression of calcinosis at 1 year (<25% increase in Scleroderma Clinical Trials Consortium radiographic score). Secondary endpoints included 1-year changes in Scleroderma HAQ (SHAQ), Cochin Hand Functional Scale, Medical Outcomes Survey Short Form 36 (SF-36), Raynaud Condition Score and patient/physician assessment of calcinosis severity. RESULTS: Twelve female patients were enrolled, half with diffuse cutaneous disease; median age was 55 years (range 35-68 years). Five patients completed the study. Seven patients withdrew due to intolerable adverse effects (n = 3), intercurrent unrelated illness (n = 2, cirrhosis, cancer), progressive SSc (n = 1) and personal reasons (n = 1). Most patients developed headaches and gastrointestinal adverse effects. Four of 11 (36%) patients with 1-year follow-up hand radiographs experienced progression of calcinosis. Of five who completed treatment, calcinosis was stable in four (80%) with progression in one. Based on SF-36 Physical and Mental Component and Domain scores, transition question and SF-6D utility score, all patients who finished the trial reported overall improvement or no change compared with baseline. CONCLUSION: Oral treprostinil was poorly tolerated in SSc patients with calcinosis. Of five patients who completed treatment, most (80%) had documented stability of calcinosis on hand radiographs at 1 year. CLINICALTRIALS.GOV IDENTIFIER: NCT02663895. CI - Published by Oxford University Press on behalf of the British Society for Rheumatology 2021. This work is written by US Government employees and is in the public domain in the US. FAU - Chung, Melody P AU - Chung MP AUID- ORCID: 0000-0003-0221-4910 AD - Division of Rheumatology, Johns Hopkins University, Baltimore, MD, USA. FAU - Valenzuela, Antonia AU - Valenzuela A AD - Division of Clinical Immunology and Rheumatology, Pontificia Universidad Católica de Chile, Santiago, Chile. FAU - Li, Shufeng AU - Li S AD - Department of Dermatology. FAU - Catanese, Benjamin AU - Catanese B AD - Department of Internal Medicine. FAU - Stevens, Kate AU - Stevens K AD - Department of Radiology, Stanford University School of Medicine, Stanford. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Dermatology. FAU - Strand, Vibeke AU - Strand V AUID- ORCID: 0000-0003-4978-4072 AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto. AD - Division of Immunology and Rheumatology, VA Palo Alto Health Care System, Palo Alto, CA, USA. LA - eng SI - ClinicalTrials.gov/NCT02663895 GR - T32 AR050942/AR/NIAMS NIH HHS/United States PT - Clinical Trial PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 RN - DCR9Z582X0 (Epoprostenol) RN - RUM6K67ESG (treprostinil) SB - IM MH - Adult MH - Aged MH - *Calcinosis/diagnostic imaging/drug therapy/etiology MH - *Epoprostenol/adverse effects/analogs & derivatives MH - Female MH - Humans MH - Middle Aged MH - Pilot Projects MH - Prospective Studies MH - *Scleroderma, Systemic/complications PMC - PMC9308380 OTO - NOTNLM OT - SSc OT - calcinosis OT - clinical trial OT - cutis OT - prostacyclin OT - scleroderma OT - treprostinil EDAT- 2021/11/01 06:00 MHDA- 2022/06/03 06:00 PMCR- 2021/10/29 CRDT- 2021/10/31 20:59 PHST- 2021/07/18 00:00 [received] PHST- 2021/10/23 00:00 [revised] PHST- 2021/11/01 06:00 [pubmed] PHST- 2022/06/03 06:00 [medline] PHST- 2021/10/31 20:59 [entrez] PHST- 2021/10/29 00:00 [pmc-release] AID - 6414200 [pii] AID - keab810 [pii] AID - 10.1093/rheumatology/keab810 [doi] PST - ppublish SO - Rheumatology (Oxford). 2022 May 30;61(6):2441-2449. doi: 10.1093/rheumatology/keab810. PMID- 38552313 OWN - NLM STAT- MEDLINE DCOM- 20240903 LR - 20241119 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 63 IP - 9 DP - 2024 Sep 1 TI - Systemic sclerosis-associated pulmonary arterial hypertension is characterized by a distinct peripheral T helper cell profile. PG - 2525-2534 LID - 10.1093/rheumatology/keae190 [doi] AB - OBJECTIVES: Systemic sclerosis (SSc) is characterized by multiple clinical manifestations. Vasculopathy is a main disease hallmark and ranges in severity from an exacerbated Raynaud phenomenon to pulmonary arterial hypertension (PAH). The potential involvement of the immune system in SSc-associated vascular abnormalities is not clear. Here, we set out to study SSc-related immune parameters and determine whether and which peripheral T cell subsets associate with vascular severity in SSc patients. METHODS: Peripheral blood and clinical data were collected from 30 SSc patients, 5 patients with idiopathic PAH and 15 age and sex-matched healthy donors (HD). In this cross-sectional cohort, SSc patients with PAH (n = 15) were matched for their age, sex and medication with SSc patients with no signs of PAH (n = 15). Lymphocyte subsets were quantified by multi-colour flow cytometry. RESULTS: SSc patients exhibited elevated percentages of T peripheral helper cells (Tph), CD4+GZMB+ T cells and decreased levels of Th1 cells compared with HD. Increased presence of both CD4+ and CD8+ exhausted-like (CD28-) T cells, characterized by raised cytokine and cytotoxic signature, was also observed in SSc compared with HD blood. Furthermore, IL-4 expressing CD4+CD8+ T cells were significantly increased in SSc peripheral blood. Interestingly, the presence of PAH in SSc was accompanied by a distinct T helper profile, characterized by raised percentages of Th17 and Tph cells. CONCLUSION: SSc patients with severe vasculopathy (presence of PAH) exhibited a distinct T cell profile, suggesting a potential role of auto-immune inflammation in SSc vascular complications. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Papadimitriou, Theodoros Ioannis AU - Papadimitriou TI AUID- ORCID: 0000-0003-4198-4838 AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. AD - Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands. FAU - Lemmers, Jacqueline M J AU - Lemmers JMJ AUID- ORCID: 0000-0003-1183-5049 AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - van Caam, Arjan P M AU - van Caam APM AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - Vos, Jacqueline L AU - Vos JL AD - Department of Cardiology, Radboudumc, Nijmegen, The Netherlands. FAU - Vitters, Elly L AU - Vitters EL AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - Stinissen, Lizan AU - Stinissen L AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - van Leuven, Sander I AU - van Leuven SI AUID- ORCID: 0000-0003-1432-069X AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - Koenders, Marije I AU - Koenders MI AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - van der Kraan, P M AU - van der Kraan PM AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - Koenen, Hans J P M AU - Koenen HJPM AD - Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands. FAU - Smeets, Ruben L AU - Smeets RL AD - Department of Laboratory Medicine - Medical Immunology, Radboudumc, Nijmegen, The Netherlands. AD - Radboudumc Laboratory for Diagnostics, Department of Laboratory Medicine, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Nijveldt, Robin AU - Nijveldt R AUID- ORCID: 0000-0003-1530-6363 AD - Department of Cardiology, Radboudumc, Nijmegen, The Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. FAU - Thurlings, Rogier M AU - Thurlings RM AD - Department of Rheumatology, Radboudumc, Nijmegen, The Netherlands. LA - eng PT - Journal Article PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Humans MH - *Scleroderma, Systemic/immunology/complications MH - Female MH - Male MH - Middle Aged MH - Cross-Sectional Studies MH - Adult MH - T-Lymphocytes, Helper-Inducer/immunology MH - Pulmonary Arterial Hypertension/immunology/etiology MH - Case-Control Studies MH - Aged MH - Hypertension, Pulmonary/etiology/immunology MH - Flow Cytometry PMC - PMC11371376 OTO - NOTNLM OT - T lymphocytes OT - biomarkers OT - pulmonary arterial hypertension OT - systemic sclerosis OT - vasculopathy EDAT- 2024/03/30 11:42 MHDA- 2024/09/04 05:42 PMCR- 2024/03/29 CRDT- 2024/03/29 19:04 PHST- 2023/11/23 00:00 [received] PHST- 2024/03/17 00:00 [accepted] PHST- 2024/09/04 05:42 [medline] PHST- 2024/03/30 11:42 [pubmed] PHST- 2024/03/29 19:04 [entrez] PHST- 2024/03/29 00:00 [pmc-release] AID - 7637682 [pii] AID - keae190 [pii] AID - 10.1093/rheumatology/keae190 [doi] PST - ppublish SO - Rheumatology (Oxford). 2024 Sep 1;63(9):2525-2534. doi: 10.1093/rheumatology/keae190. PMID- 26265167 OWN - NLM STAT- MEDLINE DCOM- 20160707 LR - 20200206 IS - 1569-8041 (Electronic) IS - 0923-7534 (Linking) VI - 26 IP - 10 DP - 2015 Oct TI - Chronic fatigue in 812 testicular cancer survivors during long-term follow-up: increasing prevalence and risk factors. PG - 2133-40 LID - 10.1093/annonc/mdv328 [doi] AB - BACKGROUND: Chronic fatigue (CF) has been reported to be slightly more prevalent in testicular cancer survivors (TCSs) than in the general population. In this study, we wished to explore possible determinants of CF in TCSs median 12 (survey I) and 19 years (survey II) after treatment, in particular the relation to late effects after treatment. PATIENTS AND METHODS: Overall, 812 TCSs treated between 1980 and 1994 provided blood samples (testosterone and luteinizing hormone) and completed questionnaires at survey I (1998-2002) and survey II (2007-2008). Hormone levels were categorized according to quartile thresholds for decadal age groups of controls. Associations between CF and possible risk factors, including the Hospital Anxiety and Depression Scale (HADS), treatment, physical activity, hormone levels, neurotoxicity, and comorbidity, were analyzed by logistic regression. RESULTS: Prevalence of CF increased from 15% at survey I to 27% at survey II (P < 0.001). At survey II, risk for CF was increased three- to four-fold for high levels of neuropathy compared with no neuropathy, and two- to three-fold for high levels of Raynaud-like phenomena, and having testosterone levels in the lowest quartile, while being moderately and highly physically active, had a protective effect. Risk for CF in TCSs with higher levels of HADS-Anxiety and HADS-Depression was increased two- to five-fold, respectively. CONCLUSIONS: The increasing prevalence of CF in TCSs is a novel finding. Lifestyle interventions, early detection and treatment of depression and anxiety, and possibly testosterone substitution might reduce the risk of CF. Extended long-term follow-up seems to be important. CI - © The Author 2015. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oup.com. FAU - Sprauten, M AU - Sprauten M AD - National Advisory Unit on Late Effects after Cancer Treatment, Oslo University Hospital, Oslo. FAU - Haugnes, H S AU - Haugnes HS AD - Department of Oncology, Institute of Clinical Medicine, University of Tromsø, Tromsø Department of Oncology, University Hospital of North Norway, Tromsø FAU - Brydøy, M AU - Brydøy M AD - Department of Oncology, Haukeland University Hospital, Bergen. FAU - Kiserud, C AU - Kiserud C AD - National Advisory Unit on Late Effects after Cancer Treatment, Oslo University Hospital, Oslo. FAU - Tandstad, T AU - Tandstad T AD - The Cancer Clinic, St Olavs University Hospital, Trondheim. FAU - Bjøro, T AU - Bjøro T AD - Department of Medical Biochemistry, Oslo University Hospital, Oslo Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo. FAU - Bjerner, J AU - Bjerner J AD - Fürst Medical Laboratory, Oslo. FAU - Cvancarova, M AU - Cvancarova M AD - National Advisory Unit on Late Effects after Cancer Treatment, Oslo University Hospital, Oslo. FAU - Fosså, S D AU - Fosså SD AD - National Advisory Unit on Late Effects after Cancer Treatment, Oslo University Hospital, Oslo. FAU - Oldenburg, J AU - Oldenburg J AD - Department of Oncology, Oslo University Hospital, Oslo Department of Oncology, Akershus University Hospital, Lørenskog, Norway jan.oldenburg@medisin.uio.no. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150811 PL - England TA - Ann Oncol JT - Annals of oncology : official journal of the European Society for Medical Oncology JID - 9007735 RN - 3XMK78S47O (Testosterone) RN - Testicular Germ Cell Tumor SB - IM MH - Adolescent MH - Adult MH - Aged MH - Anxiety/*epidemiology/etiology MH - Chronic Disease MH - Comorbidity MH - Depression/*epidemiology/etiology MH - Fatigue/*epidemiology/*etiology MH - Follow-Up Studies MH - Humans MH - Logistic Models MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Neoplasm Grading MH - Neoplasm Staging MH - Neoplasms, Germ Cell and Embryonal/*complications/physiopathology/therapy MH - Norway/epidemiology MH - Prevalence MH - Prognosis MH - Risk Factors MH - Survivors/*statistics & numerical data MH - Testicular Neoplasms/*complications/physiopathology/therapy MH - Testosterone/blood MH - Young Adult OTO - NOTNLM OT - fatigue OT - hypogonadism OT - physical activity OT - survivorship OT - testicular cancer OT - testosterone EDAT- 2015/08/13 06:00 MHDA- 2016/07/09 06:00 CRDT- 2015/08/13 06:00 PHST- 2014/11/28 00:00 [received] PHST- 2015/07/24 00:00 [accepted] PHST- 2015/08/13 06:00 [entrez] PHST- 2015/08/13 06:00 [pubmed] PHST- 2016/07/09 06:00 [medline] AID - S0923-7534(19)35808-9 [pii] AID - 10.1093/annonc/mdv328 [doi] PST - ppublish SO - Ann Oncol. 2015 Oct;26(10):2133-40. doi: 10.1093/annonc/mdv328. Epub 2015 Aug 11. PMID- 39690767 OWN - NLM STAT- MEDLINE DCOM- 20241218 LR - 20250104 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 56 IP - 6 DP - 2024 Dec 18 TI - [COVID-19 vaccines efficacy and infection features in patients with systemic sclerosis: A single-center cohort study]. PG - 1041-1046 AB - OBJECTIVE: To comprehensively understand the COVID-19 vaccination and infection status among patients with systemic sclerosis (SSc). METHODS: We conducted a retrospective analysis of patients diagnosed with SSc who were hospitalized in the Rheumatology and Immunology Department of Peking University People' s Hospital from January 2016 to March 2023. We collected detailed clinical cha-racteristics, vaccination status, and infection details through a systematic review of medical records and telephone follow-ups with the SSc patients. RESULTS: Out of 236 identified patients, 99 SSc patients participated in the follow-up. This cohort included 41 patients with limited SSc, 28 with diffuse SSc, and 30 with SSc overlap syndromes. Treatments varied, with glucocorticoids administered to 57.58% of patients, immunosuppressants to 56.57%, biologic agents to 7.07%, and small molecule targeted therapies to 6.06%. Notably, 49 patients had received the COVID-19 vaccine. Between November 2022 and March 2023, a total of 81 patients contracted COVID-19. The infection rate among those who received three doses or more (19/29, 65.5%) was significantly lower compared with unvaccinated patients (45/50, 90.0%, P=0.007). Fourteen of these patients required hospitalization due to COVID-19. Furthermore, 26 patients reported exacerbation of SSc symptoms post-infection, which included severe manifestations, such as Raynaud phenomenon, skin lesions, fingertip ulcers, pulmonary hypertension, and interstitial lung disease. Compared with healthy cohabitants, the SSc patients exhibited more severe symptoms following COVID-19, including fever (36.71%) and fatigue (35.44%). Multivariate regression analysis identified subcutaneous calcinosis (OR=7.713, 95%CI: 1.142-45.051) and positivity for anti-centromere antibodies (OR=9.210, 95%CI: 1.211-70.028) as independent risk factors for hospitalization due to COVID-19. CONCLUSION: Vaccination is both effective and safe in preventing COVID-19 among SSc patients. Additionally, it underscores that these patients experience exacerbation of their underlying disease and more severe COVID-19 symptoms compared with individuals without underlying conditions. Thus, proactive prevention, continuous monitoring, and early treatment of COVID-19 are of significant importance for the health and well-being of SSc patients. Timely interventions can help mitigate the impact of infections and improve overall patient outcomes. FAU - Pan, Wei AU - Pan W AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. AD - Department of Clinical Laboratory, 923 Hospital of the Chinese People' s Liberation Army Joint Logistic Support Force, Nanning 530021, China. FAU - Li, Yun AU - Li Y AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. FAU - Luo, Junjia AU - Luo J AD - Department of Rheumatism and Immunology, Longhua District People' s Hospital, Shenzhen 518110, Guangdong, China. FAU - Li, Chun AU - Li C AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. FAU - Ye, Hua AU - Ye H AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. FAU - Li, Xue AU - Li X AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. FAU - Jia, Yuan AU - Jia Y AD - Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (COVID-19 Vaccines) RN - 0 (Immunosuppressive Agents) SB - IM MH - Humans MH - *COVID-19/prevention & control/complications/epidemiology MH - *Scleroderma, Systemic/complications MH - *COVID-19 Vaccines/administration & dosage MH - Retrospective Studies MH - Male MH - Female MH - Middle Aged MH - SARS-CoV-2 MH - Vaccination MH - Immunosuppressive Agents/therapeutic use MH - Cohort Studies PMC - PMC11652991 OTO - NOTNLM OT - COVID-19 OT - COVID-19 vaccines OT - Systemic sclerosis COIS- 利益冲突  所有作者均声明不存在利益冲突。 EDAT- 2024/12/18 11:32 MHDA- 2024/12/18 11:33 PMCR- 2024/12/18 CRDT- 2024/12/18 01:43 PHST- 2024/12/18 11:33 [medline] PHST- 2024/12/18 11:32 [pubmed] PHST- 2024/12/18 01:43 [entrez] PHST- 2024/12/18 00:00 [pmc-release] AID - bjdxxbyxb-56-6-1041 [pii] AID - 10.19723/j.issn.1671-167X.2024.06.015 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Dec 18;56(6):1041-1046. doi: 10.19723/j.issn.1671-167X.2024.06.015. PMID- 17873760 OWN - NLM STAT- MEDLINE DCOM- 20080321 LR - 20210108 IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 86 IP - 5 DP - 2007 Sep TI - Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. PG - 299-315 LID - 10.1097/MD.0b013e3181579781 [doi] AB - Primary Sjögren syndrome (pSS) is a fairly common autoimmune disease with glandular and extraglandular manifestations. Pulmonary involvement mainly corresponds to small airways and interstitial lung disease. Pulmonary arterial hypertension (PAH) is rare: to our knowledge, only 32 cases have been reported in pSS patients to date. PAH is a disease of the small pulmonary arteries characterized by vascular proliferation and remodeling, resulting in a progressive increase in pulmonary vascular resistance, and, ultimately, right ventricular failure and death. We report 9 new cases of pSS-associated PAH with a complete assessment including clinical characteristics (of both PAH and pSS), hemodynamic parameters, medical management, and outcome. We also review the 19 fully documented PAH patients with pSS reported in the English-language literature, therefore analyzing a total of 28 cases (27 women; mean age at PAH diagnosis, 50 +/- 11 yr; range, 23-68 yr). Functional impairment at diagnosis was severe, with a New York Heart Association (NYHA) functional class of III or IV in most cases. Seven of 15 (47%) patients for whom data were available had history or evidence of right heart failure at PAH diagnosis. Hemodynamic parameters were moderate to severe with a mean pulmonary artery pressure of 44 +/- 11 mm Hg (range, 24-60 mm Hg) and a cardiac index of 2.91 +/- 0.72 Lmin(-1)m(-2) (range, 1.36-3.88 Lmin(-1)m(-2)). Standard PAH therapy (endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, or prostanoids) was initially effective in some patients but had short-term and long-term failures. Some patients were treated with first-line immunosuppressants alone leading to improvement in some, but second-line standard PAH therapy was added in all cases thereafter. The best treatment strategy remains to be defined. Estimated survival rates were low (73% and 66% at 1 and 3 years, respectively). Compared with pSS patients without PAH, patients with pSS-associated PAH had Raynaud phenomenon, cutaneous vasculitis, and interstitial lung disease significantly more frequently. They also more frequently had antinuclear, anti-Ro/SSA, and anti-RNP autoantibodies, as well as positive rheumatoid factor and hypergammaglobulinemia. These data suggest that systemic vasculopathy, B-cell activation, and autoimmunity could play a role in the pathophysiology of pSS-associated PAH. In conclusion, this report underlines the rarity and severity of PAH in pSS patients. The best therapeutic regimen remains to be defined but should include standard PAH therapy and/or immunosuppressants. FAU - Launay, David AU - Launay D AD - From Centre National de Référence de l'Hypertension Artérielle Pulmonaire, UPRES EA2705, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Assistance Publique Hôpitaux de Paris, Université Paris-Sud 11, Clamart (DL, XJ, GS, MH) and Service de Médecine Interne, Centre National de Référence de la Sclérodermie, Hôpital Claude-Huriez, CHRU Lille, Université Lille 2, Lille (DL, EH, PYH), France. FAU - Hachulla, Eric AU - Hachulla E FAU - Hatron, Pierre-Yves AU - Hatron PY FAU - Jais, Xavier AU - Jais X FAU - Simonneau, Gérald AU - Simonneau G FAU - Humbert, Marc AU - Humbert M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Antibodies, Antinuclear) RN - 0 (Endothelin Receptor Antagonists) RN - 0 (Enzyme Inhibitors) RN - 0 (Immunosuppressive Agents) RN - 0 (Phosphodiesterase 5 Inhibitors) RN - 0 (Prostaglandins) SB - IM MH - Adult MH - Aged MH - Antibodies, Antinuclear/immunology MH - Case-Control Studies MH - Diagnosis, Differential MH - Endothelin Receptor Antagonists MH - Enzyme Inhibitors/therapeutic use MH - Female MH - Follow-Up Studies MH - France MH - Heart Failure/etiology MH - Humans MH - Hypertension, Pulmonary/*diagnosis/drug therapy/*etiology/physiopathology MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Phosphodiesterase 5 Inhibitors MH - Prostaglandins/therapeutic use MH - Pulmonary Wedge Pressure MH - Retrospective Studies MH - Sjogren's Syndrome/*complications/immunology/pathology MH - Survival Analysis MH - Treatment Outcome RF - 80 EDAT- 2007/09/18 09:00 MHDA- 2008/03/22 09:00 CRDT- 2007/09/18 09:00 PHST- 2007/09/18 09:00 [pubmed] PHST- 2008/03/22 09:00 [medline] PHST- 2007/09/18 09:00 [entrez] AID - 00005792-200709000-00006 [pii] AID - 10.1097/MD.0b013e3181579781 [doi] PST - ppublish SO - Medicine (Baltimore). 2007 Sep;86(5):299-315. doi: 10.1097/MD.0b013e3181579781. PMID- 34809561 OWN - NLM STAT- MEDLINE DCOM- 20211203 LR - 20240404 IS - 1471-2288 (Electronic) IS - 1471-2288 (Linking) VI - 21 IP - 1 DP - 2021 Nov 22 TI - Impact of the COVID-19 pandemic on publication dynamics and non-COVID-19 research production. PG - 255 LID - 10.1186/s12874-021-01404-9 [doi] LID - 255 AB - BACKGROUND: The COVID-19 pandemic has severely affected health systems and medical research worldwide but its impact on the global publication dynamics and non-COVID-19 research has not been measured. We hypothesized that the COVID-19 pandemic may have impacted the scientific production of non-COVID-19 research. METHODS: We conducted a comprehensive meta-research on studies (original articles, research letters and case reports) published between 01/01/2019 and 01/01/2021 in 10 high-impact medical and infectious disease journals (New England Journal of Medicine, Lancet, Journal of the American Medical Association, Nature Medicine, British Medical Journal, Annals of Internal Medicine, Lancet Global Health, Lancet Public Health, Lancet Infectious Disease and Clinical Infectious Disease). For each publication, we recorded publication date, publication type, number of authors, whether the publication was related to COVID-19, whether the publication was based on a case series, and the number of patients included in the study if the publication was based on a case report or a case series. We estimated the publication dynamics with a locally estimated scatterplot smoothing method. A Natural Language Processing algorithm was designed to calculate the number of authors for each publication. We simulated the number of non-COVID-19 studies that could have been published during the pandemic by extrapolating the publication dynamics of 2019 to 2020, and comparing the expected number to the observed number of studies. RESULTS: Among the 22,525 studies assessed, 6319 met the inclusion criteria, of which 1022 (16.2%) were related to COVID-19 research. A dramatic increase in the number of publications in general journals was observed from February to April 2020 from a weekly median number of publications of 4.0 (IQR: 2.8-5.5) to 19.5 (IQR: 15.8-24.8) (p < 0.001), followed afterwards by a pattern of stability with a weekly median number of publications of 10.0 (IQR: 6.0-14.0) until December 2020 (p = 0.045 in comparison with April). Two prototypical editorial strategies were found: 1) journals that maintained the volume of non-COVID-19 publications while integrating COVID-19 research and thus increased their overall scientific production, and 2) journals that decreased the volume of non-COVID-19 publications while integrating COVID-19 publications. We estimated using simulation models that the COVID pandemic was associated with a 18% decrease in the production of non-COVID-19 research. We also found a significant change of the publication type in COVID-19 research as compared with non-COVID-19 research illustrated by a decrease in the number of original articles, (47.9% in COVID-19 publications vs 71.3% in non-COVID-19 publications, p < 0.001). Last, COVID-19 publications showed a higher number of authors, especially for case reports with a median of 9.0 authors (IQR: 6.0-13.0) in COVID-19 publications, compared to a median of 4.0 authors (IQR: 3.0-6.0) in non-COVID-19 publications (p < 0.001). CONCLUSION: In this meta-research gathering publications from high-impact medical journals, we have shown that the dramatic rise in COVID-19 publications was accompanied by a substantial decrease of non-COVID-19 research. META-RESEARCH REGISTRATION: https://osf.io/9vtzp/ . CI - © 2021. The Author(s). FAU - Raynaud, Marc AU - Raynaud M AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Goutaudier, Valentin AU - Goutaudier V AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Louis, Kevin AU - Louis K AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Al-Awadhi, Solaf AU - Al-Awadhi S AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Dubourg, Quentin AU - Dubourg Q AD - Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Truchot, Agathe AU - Truchot A AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Brousse, Romain AU - Brousse R AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. AD - Kidney Transplantation Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Saleh, Nouredine AU - Saleh N AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Giarraputo, Alessia AU - Giarraputo A AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Debiais, Charlotte AU - Debiais C AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Demir, Zeynep AU - Demir Z AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. AD - Paediatrics Unit, Necker University Hospital, Paris, France. FAU - Certain, Anaïs AU - Certain A AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Tacafred, Francine AU - Tacafred F AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Cortes-Garcia, Esteban AU - Cortes-Garcia E AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Yanes, Safia AU - Yanes S AD - Kidney Transplantation Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Dagobert, Jessy AU - Dagobert J AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Naser, Sofia AU - Naser S AD - Nephrology, Dialysis and Transplantation Department, Hospital Privado Universitario de Cordoba, Cordoba, Argentina. FAU - Robin, Blaise AU - Robin B AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. FAU - Bailly, Élodie AU - Bailly É AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. AD - Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. FAU - Jouven, Xavier AU - Jouven X AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. AD - Cardiology Departement, Hôpital Européen Georges Pompidou, Paris, France. FAU - Reese, Peter P AU - Reese PP AD - University of Pennsylvania School of Medicine, Philadelphia, PA, USA. FAU - Loupy, Alexandre AU - Loupy A AD - Paris Translational Research Epidemiology and Biostatistics Department, Université de Paris, INSERM U970, PARCC, 56 rue Leblanc, 75015, Paris, France. alexandreloupy@gmail.com. AD - Kidney Transplantation Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. alexandreloupy@gmail.com. LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211122 PL - England TA - BMC Med Res Methodol JT - BMC medical research methodology JID - 100968545 SB - IM MH - *Biomedical Research MH - *COVID-19 MH - Global Health MH - Humans MH - Pandemics MH - SARS-CoV-2 PMC - PMC8607966 OTO - NOTNLM OT - COVID-19 OT - High-impact journals OT - Meta-research OT - Publications COIS- The authors declare that they have no competing interests. EDAT- 2021/11/24 06:00 MHDA- 2021/12/15 06:00 PMCR- 2021/11/22 CRDT- 2021/11/23 05:32 PHST- 2021/02/19 00:00 [received] PHST- 2021/09/17 00:00 [accepted] PHST- 2021/11/23 05:32 [entrez] PHST- 2021/11/24 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/11/22 00:00 [pmc-release] AID - 10.1186/s12874-021-01404-9 [pii] AID - 1404 [pii] AID - 10.1186/s12874-021-01404-9 [doi] PST - epublish SO - BMC Med Res Methodol. 2021 Nov 22;21(1):255. doi: 10.1186/s12874-021-01404-9. PMID- 18267044 OWN - NLM STAT- MEDLINE DCOM- 20090414 LR - 20090105 IS - 1748-5460 (Electronic) IS - 0022-2151 (Linking) VI - 123 IP - 1 DP - 2009 Jan TI - An unusual succinate dehydrogenase gene mutation C in a case of laryngeal paraganglioma. PG - 141-4 LID - 10.1017/S0022215107001570 [doi] AB - OBJECTIVE: To report a rare case of a laryngeal paraganglioma related to succinate dehydrogenase gene mutation C. METHOD: A case report and a review of the world literature concerning succinate dehydrogenase mutations and laryngeal paraganglioma are presented. RESULTS: We identified a laryngeal paraganglioma in a 38-year-old woman, related to a very rare, deleterious in exon 4 of the succinate dehydrogenase mutation C. This mutation was a non-sense mutation: c.183G >A leading to p.Trp61X. No other neuroendocrine tumour was identified in this case, but a thyroid papillary carcinoma was concomitantly discovered and cured. CONCLUSION: To our knowledge, this is the first report in the world literature of laryngeal paraganglioma related to a succinate dehydrogenase mutation C. The case presented underlines the fact that every patient with paraganglioma should be tested for succinate dehydrogenase genetic mutations, even if a family history of paraganglioma is absent, in order to enable appropriate clinical management and to improve our knowledge of familial paraganglioma. FAU - Garrel, R AU - Garrel R AD - Department of Otolaryngology, Montpellier Teaching Hospitals, Montpellier Cedex 4, France. r-garrel@chu-montpellier.fr FAU - Raynaud, P AU - Raynaud P FAU - Raingeard, I AU - Raingeard I FAU - Muyshondt, C AU - Muyshondt C FAU - Gardiner, Q AU - Gardiner Q FAU - Guerrier, B AU - Guerrier B FAU - Pujol, P AU - Pujol P FAU - Coupier, I AU - Coupier I LA - eng PT - Case Reports PT - Journal Article DEP - 20080211 PL - England TA - J Laryngol Otol JT - The Journal of laryngology and otology JID - 8706896 RN - 0 (Membrane Proteins) RN - 0 (SDHC protein, human) RN - EC 1.3.99.1 (Succinate Dehydrogenase) SB - IM MH - Adult MH - Female MH - Genetic Predisposition to Disease/genetics MH - Germ-Line Mutation/*genetics MH - Humans MH - Laryngeal Neoplasms/enzymology/*genetics MH - Membrane Proteins/genetics MH - Paraganglioma/enzymology/*genetics MH - Rare Diseases/genetics MH - Succinate Dehydrogenase/*genetics EDAT- 2008/02/13 09:00 MHDA- 2009/04/15 09:00 CRDT- 2008/02/13 09:00 PHST- 2008/02/13 09:00 [pubmed] PHST- 2009/04/15 09:00 [medline] PHST- 2008/02/13 09:00 [entrez] AID - S0022215107001570 [pii] AID - 10.1017/S0022215107001570 [doi] PST - ppublish SO - J Laryngol Otol. 2009 Jan;123(1):141-4. doi: 10.1017/S0022215107001570. Epub 2008 Feb 11. PMID- 25500708 OWN - NLM STAT- MEDLINE DCOM- 20150420 LR - 20210109 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 93 IP - 24 DP - 2014 Nov TI - An autoimmune myositis-overlap syndrome associated with autoantibodies to nuclear pore complexes: description and long-term follow-up of the anti-Nup syndrome. PG - 383-394 LID - 10.1097/MD.0000000000000223 [doi] LID - 383-394 AB - Autoimmune myositis encompasses various myositis-overlap syndromes, each being identified by the presence of serum marker autoantibodies. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. There was no clinical or laboratory evidence for liver disease. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr).By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes. Reactivity with nuclear pore complexes was confirmed by immunoelectron microscopy. In a cohort of 100 French Canadian patients with autoimmune myositis, the nuclear pore complex fluorescent ANA pattern was restricted to these 4 patients (4%). It was not observed in sera from 393 adult patients with systemic sclerosis (n = 112), mixed connective tissue disease (n = 35), systemic lupus (n = 94), rheumatoid arthritis (n = 45), or other rheumatic diseases (n = 107), nor was it observed in 62 normal adults.Autoantibodies to nuclear pore complexes were predominantly of IgG isotype. No other IgG autoantibody markers for defined connective tissue diseases or overlap syndromes were present, indicating a selective and highly focused immune response. In 3 patients, anti-nuclear pore complex autoantibody titers varied in parallel with myositis activity, suggesting a pathogenic link to pathophysiology. The nuclear pore complex proteins, that is, nucleoporins (nup), recognized by these sera were heterogeneous and included Nup358/RanBP2 (n = 2 patients), Nup90 (n = 1), Nup62 (n = 1), and gp210 (n = 1). Taken together the data suggest that nup autoantigens themselves drive the anti-nup autoimmune response. Immunogenetically, the 4 patients shared the DQA1*0501 allele associated with an increased risk for autoimmune myositis.In conclusion, we report an apparent novel subset of autoimmune myositis in our population of French Canadian patients with connective tissue diseases. This syndrome is recognized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes that react with nups, consistent with an "anti-nup syndrome." FAU - Senécal, Jean-Luc AU - Senécal JL AD - From the Department of Medicine, Divisions of Rheumatology (JLS, CI, JPR, YT) and Internal Medicine (FJ), and Laboratory for Research in Autoimmunity, Research Center of the Centre Hospitalier de l'Université de Montréal, University of Montreal Faculty of Medicine, Montreal, Quebec, Canada; Mitogen Advanced Diagnostics Laboratory (MJF), Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada; Veterans Affairs Medical Center (INT), University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, United States; McGill University (RG), Montreal, Quebec, Canada; Polyclinique Saint-Eustache (MG), Saint-Eustache, Quebec, Canada; Biocenter (MCD), Division of Electron Microscopy, University of Würzburg, Am Hubland, Würzburg, Germany. FAU - Isabelle, Catherine AU - Isabelle C FAU - Fritzler, Marvin J AU - Fritzler MJ FAU - Targoff, Ira N AU - Targoff IN FAU - Goldstein, Rose AU - Goldstein R FAU - Gagné, Michel AU - Gagné M FAU - Raynauld, Jean-Pierre AU - Raynauld JP FAU - Joyal, France AU - Joyal F FAU - Troyanov, Yves AU - Troyanov Y FAU - Dabauvalle, Marie-Christine AU - Dabauvalle MC LA - eng GR - MOP-62687/Canadian Institutes of Health Research/Canada GR - MOP-81252/Canadian Institutes of Health Research/Canada GR - MT-14636/Canadian Institutes of Health Research/Canada PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Antibodies, Antinuclear) RN - 0 (Immunosuppressive Agents) SB - IM EIN - Medicine (Baltimore). 2014 Nov;93(24):414 MH - Adult MH - Antibodies, Antinuclear/*immunology MH - Autoimmune Diseases/diagnosis/drug therapy/*immunology MH - Female MH - Fluorescent Antibody Technique, Indirect MH - Follow-Up Studies MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Myositis/diagnosis/drug therapy/*immunology MH - Nuclear Pore/*immunology MH - Prognosis MH - Syndrome PMC - PMC4602431 COIS- Financial support and conflicts of interest: This study was supported in part by operating grants MT-14636, MOP-62687, and MOP-81252 from the Canadian Institutes of Health Research (to JLS), by Department of Veterans Affairs Medical Research Funds (to INT), and by donations from Sclérodermie Québec and Mrs Gisèle Sarrazin-Locas in support of The Laboratory for Research in Autoimmunity. JLS holds the University of Montreal Scleroderma Research Chair. MJF holds the Arthritis Society Research Chair at the University of Calgary. Authors JLS, CI, MCD, RG, and FJ have no conflicts of interest to disclose. The authors listed below have received financial support (personal or institutional) from the listed institutions or pharmaceutical companies, unrelated to the present work. MJF: EUROIMMUN, ImmunoConcepts, INOVA Diagnostics; INT: Oklahoma Medical Research Foundation Clinical Immunology Laboratory, UpToDate; YT: Abbvie, Amgen, BMS, Janssen, Roche, UCB, Warner-Chilcott; MG: Novartis; JPR: Arthrolab, Amgen, BMS, Cellgene, Janssen, Lilly, Roche, Pfizer. EDAT- 2014/12/17 06:00 MHDA- 2015/04/22 06:00 PMCR- 2014/11/02 CRDT- 2014/12/16 06:00 PHST- 2014/12/16 06:00 [entrez] PHST- 2014/12/17 06:00 [pubmed] PHST- 2015/04/22 06:00 [medline] PHST- 2014/11/02 00:00 [pmc-release] AID - 00005792-201411040-00008 [pii] AID - 10.1097/MD.0000000000000223 [doi] PST - ppublish SO - Medicine (Baltimore). 2014 Nov;93(24):383-394. doi: 10.1097/MD.0000000000000223. PMID- 35687757 OWN - NLM STAT- MEDLINE DCOM- 20220908 LR - 20240903 IS - 1528-0020 (Electronic) IS - 0006-4971 (Print) IS - 0006-4971 (Linking) VI - 140 IP - 9 DP - 2022 Sep 1 TI - Sutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial. PG - 980-991 LID - 10.1182/blood.2021014955 [doi] AB - Sutimlimab, a first-in-class humanized immunoglobulin G4 (IgG4) monoclonal antibody that selectively inhibits the classical complement pathway at C1s, rapidly halted hemolysis in the single-arm CARDINAL study in recently transfused patients with cold agglutinin disease (CAD). CADENZA was a 26-week randomized, placebo-controlled phase 3 study to assess safety and efficacy of sutimlimab in patients with CAD without recent (within 6 months prior to enrollment) transfusion history. Forty-two patients with screening hemoglobin ≤10 g/dL, elevated bilirubin, and ≥1 CAD symptom received sutimlimab (n = 22) or placebo (n = 20) on days 0 and 7 and then biweekly. Composite primary endpoint criteria (hemoglobin increase ≥1.5 g/dL at treatment assessment timepoint [mean of weeks 23, 25, 26], avoidance of transfusion, and study-prohibited CAD therapy [weeks 5-26]) were met by 16 patients (73%) on sutimlimab, and 3 patients (15%) on placebo (odds ratio, 15.9 [95% confidence interval, 2.9, 88.0; P < .001]). Sutimlimab, but not placebo, significantly increased mean hemoglobin and FACIT-Fatigue scores at treatment assessment timepoint. Sutimlimab normalized mean bilirubin by week 1. Improvements correlated with near-complete inhibition of the classical complement pathway (2.3% mean activity at week 1) and C4 normalization. Twenty-one (96%) sutimlimab patients and 20 (100%) placebo patients experienced ≥1 treatment-emergent adverse event. Headache, hypertension, rhinitis, Raynaud phenomenon, and acrocyanosis were more frequent with sutimlimab vs placebo, with a difference of ≥3 patients between groups. Three sutimlimab patients discontinued owing to adverse events; no placebo patients discontinued. These data demonstrate that sutimlimab has potential to be an important advancement in the treatment of CAD. This trial was registered at www.clinicaltrials.gov as #NCT03347422. CI - © 2022 by The American Society of Hematology. FAU - Röth, Alexander AU - Röth A AUID- ORCID: 0000-0003-4414-7699 AD - Department of Hematology and Stem Cell Transplantation, West German Cancer Center, University Hospital Essen, University of Duisburg-Essen, Essen, Germany. FAU - Berentsen, Sigbjørn AU - Berentsen S AD - Department of Research and Innovation, Haugesund Hospital, Haugesund, Norway. FAU - Barcellini, Wilma AU - Barcellini W AD - Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. FAU - D'Sa, Shirley AU - D'Sa S AD - UCLH Centre for Waldenström's Macroglobulinemia and Related Conditions, University College London Hospitals National Health Service (NHS) Foundation Trust, London, United Kingdom. FAU - Jilma, Bernd AU - Jilma B AUID- ORCID: 0000-0001-5652-7977 AD - Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria. FAU - Michel, Marc AU - Michel M AD - Henri-Mondor University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil (UPEC), Créteil, France. FAU - Weitz, Ilene C AU - Weitz IC AD - Jane Anne Nohl Division of Hematology Keck-University of Southern California (USC) School of Medicine, Los Angeles, CA. FAU - Yamaguchi, Masaki AU - Yamaguchi M AD - Department of Hematology, Ishikawa Prefectural Central Hospital, Kanazawa, Japan. FAU - Nishimura, Jun-Ichi AU - Nishimura JI AD - Department of Hematology and Oncology, Osaka University Graduate School of Medicine, Osaka, Japan. FAU - Vos, Josephine M I AU - Vos JMI AD - Department of Hematology, Amsterdam University Medical Centers (UMC) & Sanquin, Amsterdam, The Netherlands. FAU - Storek, Michael AU - Storek M AD - Sanofi, Cambridge, MA. FAU - Wong, Nancy AU - Wong N AD - Sanofi, Cambridge, MA. FAU - Patel, Parija AU - Patel P AD - Sanofi, Cambridge, MA. FAU - Jiang, Xiaoyu AU - Jiang X AD - Sanofi, Cambridge, MA. FAU - Vagge, Deepthi S AU - Vagge DS AD - IQVIA, Bangalore, Karnataka, India. FAU - Wardęcki, Marek AU - Wardęcki M AUID- ORCID: 0000-0003-4188-2647 AD - Sanofi, Warsaw, Poland; and. FAU - Shafer, Frank AU - Shafer F AD - Sanofi, Cambridge, MA. FAU - Lee, Michelle AU - Lee M AD - Sanofi, Cambridge, MA. FAU - Broome, Catherine M AU - Broome CM AD - Division of Hematology, MedStar Georgetown University Hospital, Washington, DC. LA - eng SI - ClinicalTrials.gov/NCT03347422 PT - Clinical Trial, Phase III PT - Journal Article PT - Randomized Controlled Trial PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Hemoglobins) RN - GNWE7KJ995 (sutimlimab) RN - RFM9X3LJ49 (Bilirubin) SB - IM CIN - Blood. 2022 Sep 1;140(9):933-935. doi: 10.1182/blood.2022017284. PMID: 36048476 MH - *Anemia, Hemolytic, Autoimmune/blood/drug therapy MH - *Antibodies, Monoclonal, Humanized/therapeutic use MH - Bilirubin/blood MH - Double-Blind Method MH - Hemoglobins/analysis MH - Humans MH - Treatment Outcome PMC - PMC9437710 EDAT- 2022/06/11 06:00 MHDA- 2022/09/09 06:00 PMCR- 2022/09/01 CRDT- 2022/06/10 15:03 PHST- 2021/12/22 00:00 [received] PHST- 2022/05/30 00:00 [accepted] PHST- 2022/06/11 06:00 [pubmed] PHST- 2022/09/09 06:00 [medline] PHST- 2022/06/10 15:03 [entrez] PHST- 2022/09/01 00:00 [pmc-release] AID - S0006-4971(22)00771-6 [pii] AID - 2022/BLD2021014955 [pii] AID - 10.1182/blood.2021014955 [doi] PST - ppublish SO - Blood. 2022 Sep 1;140(9):980-991. doi: 10.1182/blood.2021014955. PMID- 27748811 OWN - NLM STAT- MEDLINE DCOM- 20170406 LR - 20170406 IS - 1791-3004 (Electronic) IS - 1791-2997 (Linking) VI - 14 IP - 5 DP - 2016 Nov TI - Danggui‑Sayuk‑Ga‑Osuyu‑Senggang‑Tang ameliorates cold‑induced vasoconstriction in vitro and in vivo. PG - 4723-4728 LID - 10.3892/mmr.2016.5805 [doi] AB - Danggui-Sayuk-Ga-Osuyu-Senggang-Tang (DSGOST), one of the traditional Chinese medicines, has long been prescribed for patients suffering from Raynaud phenomenon (RP) in Northeast Asian countries, including China, Japan and Korea. Although a previous in vitro study from our laboratory revealed that DSGOST prevents cold (25˚C)‑induced RhoA activation and endothelin‑1 (ET‑1) production in endothelial cells (ECs), the mechanisms by which DSGOST is able to alleviate the symptoms of RP have yet to be fully elucidated. The present study aimed to demonstrate that DSGOST regulates RhoA‑mediated pathways in cold‑exposed pericytes. In pericytes, DSGOST amplified cold‑induced RhoA activation, while markedly reducing ET‑1‑induced RhoA activation. Additionally, DSGOST‑mediated regulation of RhoA was closely associated with Rho‑associated, coiled‑coil‑containing protein kinase 1 (ROCK1)/testis‑specific kinase 1 (TESK1)/PDXP, but not with LIM domain kinase 1/2 (LIMK1/2), cofilin and myosin light chain (MLC). Thus, DSGOST activation of RhoA/ROCK1/TESK1/PDXP in cold‑exposed pericytes appeared to be crucial for treating vessel contraction. In addition, the DSGOST effect on the RhoA‑mediated pathway in cold‑induced human umbilical vein endothelial cells or human dermal microvascular endothelial cells was similar to that in ET‑1‑treated pericytes, but not in cold‑induced pericytes. The results of the present study further confirmed that DSGOST inhibits cold‑induced contraction of the mouse tail vein in vivo. Furthermore, DSGOST treatment reduced cold‑induced expression of the α2c‑adrenergic receptor in mouse tail vessels. Therefore, the data in the present study suggest that DSGOST may be useful for the treatment of RP‑like disease. FAU - Lee, Kangwook AU - Lee K AD - Department of Science in Korean Medicine, Graduate School, Kyung Hee University, Seoul 130‑701, Republic of Korea. FAU - Cho, Sung-Gook AU - Cho SG AD - Department of Biotechnology, Korea National University of Transportation, Chungbuk 368‑701, Republic of Korea. FAU - Woo, Sang-Mi AU - Woo SM AD - Department of Cancer Preventive Material Development, Graduate School, Kyung Hee University, Seoul 130‑701, Republic of Korea. FAU - Kim, Ah Jeong AU - Kim AJ AD - Department of Science in Korean Medicine, Graduate School, Kyung Hee University, Seoul 130‑701, Republic of Korea. FAU - Lee, Kang Min AU - Lee KM AD - Department of Science in Korean Medicine, Graduate School, Kyung Hee University, Seoul 130‑701, Republic of Korea. FAU - Go, Ho Yeon AU - Go HY AD - Department of Korean Internal Medicine, College of Korean Medicine, Semyung University, Chungju 380‑960, Republic of Korea. FAU - Sun, Seung-Ho AU - Sun SH AD - Department of Oriental Internal Medicine, College of Korean Medicine, Sangji University, Wonju 220‑702, Republic of Korea. FAU - Kim, Tae-Hun AU - Kim TH AD - Korean Medicine Clinical Trial Center, Korean Medicine Hospital, Kyung Hee University, Seoul 130‑701, Republic of Korea. FAU - Jung, Ki-Yong AU - Jung KY AD - Department of Korean Internal Medicine, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Choi, You-Kyung AU - Choi YK AD - Department of Korean Internal Medicine, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Lim, Eun Mee AU - Lim EM AD - Department of Obsterics and Gynecology, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Song, Yun-Kyung AU - Song YK AD - Department of Korean Rehabilitation Medicine, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Park, Jong-Hyeong AU - Park JH AD - Department of Korean Internal Medicine, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Jun, Chan-Yong AU - Jun CY AD - Department of Korean Internal Medicine, College of Korean Medicine, Gachon University, Seongnam 461‑701, Republic of Korea. FAU - Ko, Seong-Gyu AU - Ko SG AD - Department of Preventive Medicine, College of Korean Medicine, Kyung Hee University, Seoul 130‑701, Republic of Korea. LA - eng PT - Journal Article DEP - 20161005 PL - Greece TA - Mol Med Rep JT - Molecular medicine reports JID - 101475259 RN - 0 (Drugs, Chinese Herbal) RN - 0 (Endothelin-1) RN - 0 (Receptors, Adrenergic, alpha-2) SB - IM MH - Animals MH - *Cold Temperature MH - Drugs, Chinese Herbal/*pharmacology MH - Endothelin-1/pharmacology MH - Human Umbilical Vein Endothelial Cells/drug effects/metabolism MH - Humans MH - Mice MH - Pericytes/drug effects/metabolism MH - Receptors, Adrenergic, alpha-2/metabolism MH - Signal Transduction/drug effects MH - Vasoconstriction/*drug effects EDAT- 2016/10/26 06:00 MHDA- 2017/04/07 06:00 CRDT- 2016/10/26 06:00 PHST- 2015/05/26 00:00 [received] PHST- 2016/08/05 00:00 [accepted] PHST- 2016/10/26 06:00 [pubmed] PHST- 2017/04/07 06:00 [medline] PHST- 2016/10/26 06:00 [entrez] AID - 10.3892/mmr.2016.5805 [doi] PST - ppublish SO - Mol Med Rep. 2016 Nov;14(5):4723-4728. doi: 10.3892/mmr.2016.5805. Epub 2016 Oct 5. PMID- 22687175 OWN - NLM STAT- MEDLINE DCOM- 20130219 LR - 20220318 IS - 1479-5876 (Electronic) IS - 1479-5876 (Linking) VI - 10 DP - 2012 Jun 11 TI - Gene expression analysis of matched ovarian primary tumors and peritoneal metastasis. PG - 121 LID - 10.1186/1479-5876-10-121 [doi] AB - BACKGROUND: Ovarian cancer is the most deadly gynecological cancer due to late diagnosis at advanced stage with major peritoneal involvement. To date most research has focused on primary tumor. However the prognosis is directly related to residual disease at the end of the treatment. Therefore it is mandatory to focus and study the biology of metastatic disease that is most frequently localized to the peritoneal cavity in ovarian cancer. METHODS: We used high-density gene expression arrays to investigate gene expression changes between matched primary and metastatic (peritoneal) lesions. RESULTS: Here we show that gene expression profiles in peritoneal metastasis are significantly different than their matched primary tumor and these changes are affected by underlying copy number variation differences among other causes. We show that differentially expressed genes are enriched in specific pathways including JAK/STAT pathway, cytokine signaling and other immune related pathways. We show that underlying copy number variations significantly affect gene expression. Indeed patients with important differences in copy number variation displayed greater gene expression differences between their primary and matched metastatic lesions. CONCLUSIONS: Our analysis shows a very specific targeting at both the genomic and transcriptomic level to upregulate certain pathways in the peritoneal metastasis of ovarian cancer. Moreover, while primary tumors use certain pathways we identify distinct differences with metastatic lesions. The variation between primary and metastatic lesions should be considered in personalized treatment of ovarian cancer. FAU - Malek, Joel A AU - Malek JA AD - Genomics Core, Weill Cornell Medical College in Qatar, Education city, Qatar Foundation, Doha, Qatar PO 241 44. FAU - Martinez, Alejandra AU - Martinez A FAU - Mery, Eliane AU - Mery E FAU - Ferron, Gwenael AU - Ferron G FAU - Huang, Ruby AU - Huang R FAU - Raynaud, Christophe AU - Raynaud C FAU - Jouve, Eva AU - Jouve E FAU - Thiery, Jean-Paul AU - Thiery JP FAU - Querleu, Denis AU - Querleu D FAU - Rafii, Arash AU - Rafii A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120611 PL - England TA - J Transl Med JT - Journal of translational medicine JID - 101190741 SB - IM MH - Female MH - *Gene Expression Profiling MH - Humans MH - Ovarian Neoplasms/*genetics/pathology MH - Peritoneal Neoplasms/genetics/*secondary MH - Prognosis PMC - PMC3477065 EDAT- 2012/06/13 06:00 MHDA- 2013/02/21 06:00 PMCR- 2012/06/11 CRDT- 2012/06/13 06:00 PHST- 2012/03/02 00:00 [received] PHST- 2012/04/26 00:00 [accepted] PHST- 2012/06/13 06:00 [entrez] PHST- 2012/06/13 06:00 [pubmed] PHST- 2013/02/21 06:00 [medline] PHST- 2012/06/11 00:00 [pmc-release] AID - 1479-5876-10-121 [pii] AID - 10.1186/1479-5876-10-121 [doi] PST - epublish SO - J Transl Med. 2012 Jun 11;10:121. doi: 10.1186/1479-5876-10-121. PMID- 16647997 OWN - NLM STAT- MEDLINE DCOM- 20060706 LR - 20061115 IS - 0887-8994 (Print) IS - 0887-8994 (Linking) VI - 34 IP - 5 DP - 2006 May TI - The incidence of Rett syndrome in France. PG - 372-5 AB - Since the description of Rett syndrome, only a handful of epidemiologic studies based only on clinical investigation have been reported. Mutations in the MECP2 gene are associated with Rett syndrome and French laboratories have organized a clinical and molecular network to investigate the incidence of Rett syndrome in France including the results of molecular investigations. The present study, based on a large cohort of 424 patients with Rett syndrome, found that the incidence of this disease with a MECP2 mutation varied between 0.43 to 0.71 per 10,000 females. The total population of females aged 4-15 years in November 2004 in France was estimated to be 4,337,627. The data presented here indicate a prevalence of Rett syndrome of 0.558 per 10,000 females aged 4-15 years in France. The incidence of Rett syndrome is in accordance with other European epidemiologic studies based on clinical examination. Given that this is a minimum incidence because complete inventory was not possible, this study of patients with Rett syndrome reinforces the fact that the great majority of patients with Rett syndrome have a MECP2 mutation. FAU - Bienvenu, Thierry AU - Bienvenu T AD - University Paris 5, Cochin Institute, INSERM U567, Centre National de la Recherche Scientifique/Unitré Mixte de Reserche 8104, Paris, France. bienvenu@cochin.inserm.fr FAU - Philippe, Christophe AU - Philippe C FAU - De Roux, Nicolas AU - De Roux N FAU - Raynaud, Martine AU - Raynaud M FAU - Bonnefond, Jean Paul AU - Bonnefond JP FAU - Pasquier, Laurent AU - Pasquier L FAU - Lesca, Gaetan AU - Lesca G FAU - Mancini, Josette AU - Mancini J FAU - Jonveaux, Philippe AU - Jonveaux P FAU - Moncla, Anne AU - Moncla A FAU - Feingold, Josué AU - Feingold J FAU - Chelly, Jamel AU - Chelly J FAU - Villard, Laurent AU - Villard L LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Pediatr Neurol JT - Pediatric neurology JID - 8508183 RN - 0 (MECP2 protein, human) RN - 0 (Methyl-CpG-Binding Protein 2) SB - IM MH - Adolescent MH - Adult MH - Child MH - Female MH - France/epidemiology MH - Genetic Predisposition to Disease/epidemiology MH - Humans MH - Incidence MH - Mass Screening MH - Methyl-CpG-Binding Protein 2/*genetics MH - Mutation MH - Prevalence MH - Rett Syndrome/*epidemiology/*genetics EDAT- 2006/05/02 09:00 MHDA- 2006/07/11 09:00 CRDT- 2006/05/02 09:00 PHST- 2005/08/01 00:00 [received] PHST- 2005/09/02 00:00 [revised] PHST- 2005/10/06 00:00 [accepted] PHST- 2006/05/02 09:00 [pubmed] PHST- 2006/07/11 09:00 [medline] PHST- 2006/05/02 09:00 [entrez] AID - S0887-8994(05)00629-6 [pii] AID - 10.1016/j.pediatrneurol.2005.10.013 [doi] PST - ppublish SO - Pediatr Neurol. 2006 May;34(5):372-5. doi: 10.1016/j.pediatrneurol.2005.10.013. PMID- 17056592 OWN - NLM STAT- MEDLINE DCOM- 20070206 LR - 20210209 IS - 0021-9258 (Print) IS - 0021-9258 (Linking) VI - 281 IP - 51 DP - 2006 Dec 22 TI - A mouse model for monitoring calpain activity under physiological and pathological conditions. PG - 39672-80 AB - Calpains are Ca(2+)-dependent cysteine proteases known to be important for the regulation of cell functions and which aberrant activation causes cell death in a number of degenerative disorders. To provide a tool for monitoring the status of calpain activity in vivo under physiological and pathological conditions, we created a mouse model that expresses ubiquitously a fluorescent reporter consisting of eCFP and eYFP separated by a linker cleavable by the ubiquitous calpains. We named this mouse CAFI for calpain activity monitored by FRET imaging. Our validation studies demonstrated that the level of calpain activity correlates with a decrease in FRET (fluorescence resonance energy transfer) between the two fluorescent proteins. Using this model, we observed a small level of activity after denervation and fasting, a high level of activity during muscle regeneration and ischemia, and local activity in damaged myofibers after exercise. Finally, we crossed the CAFI mouse with the alpha-sarcoglycan-deficient model, demonstrating an increase of calpain activity at the steady state. Altogether, our results present evidence that CAFI mice could be a valuable tool in which to follow calpain activity at physiological levels and in disease states. FAU - Bartoli, Marc AU - Bartoli M AD - Généthon/CNRS-UMR8115, 1 rue de l'Internationale 91000 Evry, France. FAU - Bourg, Nathalie AU - Bourg N FAU - Stockholm, Daniel AU - Stockholm D FAU - Raynaud, Fabrice AU - Raynaud F FAU - Delevacque, Antony AU - Delevacque A FAU - Han, Yang AU - Han Y FAU - Borel, Perrine AU - Borel P FAU - Seddik, Kenza AU - Seddik K FAU - Armande, Nasser AU - Armande N FAU - Richard, Isabelle AU - Richard I LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20061020 PL - United States TA - J Biol Chem JT - The Journal of biological chemistry JID - 2985121R RN - 0 (Bacterial Proteins) RN - 0 (Luminescent Proteins) RN - 0 (yellow fluorescent protein, Bacteria) RN - EC 3.4.22.- (Calpain) RN - SY7Q814VUP (Calcium) SB - IM MH - Animals MH - Bacterial Proteins/chemistry MH - Calcium/metabolism MH - Calpain/*chemistry/metabolism MH - Disease Models, Animal MH - Female MH - Fluorescence Resonance Energy Transfer MH - Genes, Reporter MH - Humans MH - Luminescent Proteins/chemistry MH - Male MH - Mice MH - Mice, Inbred C57BL MH - Models, Chemical MH - Models, Genetic MH - Neurodegenerative Diseases/metabolism EDAT- 2006/10/24 09:00 MHDA- 2007/02/07 09:00 CRDT- 2006/10/24 09:00 PHST- 2006/10/24 09:00 [pubmed] PHST- 2007/02/07 09:00 [medline] PHST- 2006/10/24 09:00 [entrez] AID - S0021-9258(19)33833-5 [pii] AID - 10.1074/jbc.M608803200 [doi] PST - ppublish SO - J Biol Chem. 2006 Dec 22;281(51):39672-80. doi: 10.1074/jbc.M608803200. Epub 2006 Oct 20. PMID- 19013120 OWN - NLM STAT- MEDLINE DCOM- 20090716 LR - 20141120 IS - 1769-7255 (Print) IS - 1769-7255 (Linking) VI - 5 IP - 2 DP - 2009 Apr TI - [A preemptive combined liver-kidney transplantation in Aalpha fibrinogen chain renal amyloidosis]. PG - 139-43 LID - 10.1016/j.nephro.2008.08.015 [doi] AB - The predominant cause of hereditary renal amyloidosis is a mutation of the fibrinogen Aalpha chain (AFib), the most common being the E526V mutation. The evolution towards terminal renal insufficiency is constant and raises the question of renal transplantation and the risk of recurrence. We describe the case of a Portuguese woman with the E526V mutation without any renal or hepatic history in her family which developed a nephrotic syndrome at the age of 35, followed by stage 5 renal insufficiency. Because of the risk of recurrence of amyloidosis on its transplant, we carried out a combined transplantation liver-kidney despite the absence of clinical or biological hepatic abnormalities. Four years later, the result is excellent and there is no sign of the disease on the new organs. This successful experience as well as the five other published cases of combined liver-kidney transplantation in Aalpha fibrinogen chain amyloidosis, demonstrates the feasibility and efficacy of this treatment in AFib amyloidosis. FAU - Delabre, Jean-Philippe AU - Delabre JP AD - Service de néphrologie, transplantation et dialyse péritonéale, hôpital Lapeyronie, 371, avenue G-Giraud, 34295 Montpellier cedex 05, France. FAU - Pageaux, Georges-Philippe AU - Pageaux GP FAU - Le Quellec, Alain AU - Le Quellec A FAU - Raynaud, Pierre AU - Raynaud P FAU - Grateau, Gilles AU - Grateau G FAU - Mourad, Georges AU - Mourad G LA - fre PT - Case Reports PT - English Abstract PT - Journal Article PT - Review TT - Transplantation préemptive foie-rein pour une amylose rénale à fibrinogène Aalpha. DEP - 20081113 PL - France TA - Nephrol Ther JT - Nephrologie & therapeutique JID - 101248950 RN - 0 (fibrinogen Aalpha) RN - 9001-32-5 (Fibrinogen) SB - IM MH - Adult MH - Amyloidosis, Familial/*complications/genetics MH - Female MH - Fibrinogen/*genetics MH - Humans MH - Kidney Failure, Chronic/etiology/*surgery MH - *Kidney Transplantation MH - *Liver Transplantation MH - Mutation, Missense MH - Pedigree MH - Point Mutation MH - Secondary Prevention RF - 18 EDAT- 2008/11/18 09:00 MHDA- 2009/07/17 09:00 CRDT- 2008/11/18 09:00 PHST- 2007/12/20 00:00 [received] PHST- 2008/08/26 00:00 [revised] PHST- 2008/08/28 00:00 [accepted] PHST- 2008/11/18 09:00 [pubmed] PHST- 2009/07/17 09:00 [medline] PHST- 2008/11/18 09:00 [entrez] AID - S1769-7255(08)00214-9 [pii] AID - 10.1016/j.nephro.2008.08.015 [doi] PST - ppublish SO - Nephrol Ther. 2009 Apr;5(2):139-43. doi: 10.1016/j.nephro.2008.08.015. Epub 2008 Nov 13. PMID- 37571269 OWN - NLM STAT- MEDLINE DCOM- 20230814 LR - 20230815 IS - 2072-6643 (Electronic) IS - 2072-6643 (Linking) VI - 15 IP - 15 DP - 2023 Jul 26 TI - Cafeteria Diet-Induced Obesity Worsens Experimental CKD. LID - 10.3390/nu15153331 [doi] LID - 3331 AB - Obesity is a significant risk factor for chronic kidney disease (CKD). This study aimed to evaluate the impact of obesity on the development of kidney fibrosis in a model of cafeteria diet rats undergoing 5/6th nephrectomy (SNx). Collagen 1, 3, and 4 expression, adipocyte size, macrophage number, and the expression of 30 adipokines were determined. Collagen 1 expression in kidney tissue was increased in Standard-SNx and Cafeteria-SNx (7.1 ± 0.6% and 8.9 ± 0.9 tissue area, respectively). Renal expression of collagen 3 and 4 was significantly increased (p < 0.05) in Cafeteria-SNx (8.6 ± 1.5 and 10.9 ± 1.9% tissue area, respectively) compared to Cafeteria (5.2 ± 0.5 and 6.3 ± 0.6% tissue area, respectively). Adipocyte size in eWAT was significantly increased by the cafeteria diet. In Cafeteria-SNx, we observed a significant increase in macrophage number in the kidney (p = 0.01) and a consistent tendency in eWAT. The adipokine level was higher in the Cafeteria groups. Interleukin 11, dipeptidyl peptidase 4, and serpin 1 were increased in Cafeteria-SNx. In the kidney, collagen 3 and 4 expressions and the number of macrophages were increased in Cafeteria-SNx, suggesting an exacerbation by preexisting obesity of CKD-induced renal inflammation and fibrosis. IL11, DPP4, and serpin 1 can act directly on fibrosis and participate in the observed worsening CKD. FAU - Laget, Jonas AU - Laget J AUID- ORCID: 0000-0002-9095-7409 AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Cortijo, Irene AU - Cortijo I AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Boukhaled, Juliana H AU - Boukhaled JH AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Muyor, Karen AU - Muyor K AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Duranton, Flore AU - Duranton F AUID- ORCID: 0000-0003-3202-0551 AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Jover, Bernard AU - Jover B AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - PhyMedExp, INSERM, CNRS, Université de Montpellier, 34090 Montpellier, France. FAU - Lajoix, Anne-Dominique AU - Lajoix AD AUID- ORCID: 0000-0002-5912-5233 AD - Biocommunication in Cardio-Metabolism (BC2M), University of Montpellier, 34090 Montpellier, France. FAU - Argilés, Àngel AU - Argilés À AUID- ORCID: 0000-0001-7029-4532 AD - RD-Néphrologie, 34090 Montpellier, France. FAU - Gayrard, Nathalie AU - Gayrard N AUID- ORCID: 0000-0001-8283-8429 AD - RD-Néphrologie, 34090 Montpellier, France. LA - eng PT - Journal Article DEP - 20230726 PL - Switzerland TA - Nutrients JT - Nutrients JID - 101521595 RN - 0 (Serpins) RN - 9007-34-5 (Collagen) SB - IM MH - Rats MH - Animals MH - *Serpins MH - *Renal Insufficiency, Chronic/etiology/metabolism MH - Nephrectomy/adverse effects MH - Fibrosis MH - Obesity/complications MH - Diet/adverse effects MH - Collagen PMC - PMC10421241 OTO - NOTNLM OT - adipokine OT - chronic kidney disease OT - collagen OT - fibrosis OT - inflammation OT - macrophage OT - obesity COIS- The authors declare no conflict of interest. EDAT- 2023/08/12 10:49 MHDA- 2023/08/14 06:42 PMCR- 2023/07/26 CRDT- 2023/08/12 01:21 PHST- 2023/06/30 00:00 [received] PHST- 2023/07/20 00:00 [revised] PHST- 2023/07/21 00:00 [accepted] PHST- 2023/08/14 06:42 [medline] PHST- 2023/08/12 10:49 [pubmed] PHST- 2023/08/12 01:21 [entrez] PHST- 2023/07/26 00:00 [pmc-release] AID - nu15153331 [pii] AID - nutrients-15-03331 [pii] AID - 10.3390/nu15153331 [doi] PST - epublish SO - Nutrients. 2023 Jul 26;15(15):3331. doi: 10.3390/nu15153331. PMID- 32487712 OWN - NLM STAT- MEDLINE DCOM- 20200928 LR - 20240403 IS - 1526-632X (Electronic) IS - 0028-3878 (Print) IS - 0028-3878 (Linking) VI - 95 IP - 1 DP - 2020 Jul 7 TI - Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. PG - e70-e78 LID - 10.1212/WNL.0000000000009727 [doi] AB - OBJECTIVES: The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody, as well as the homogeneity of the disease. METHODS: To characterize the anti-MDA5+ phenotype, an unsupervised analysis was performed on anti-MDA5+ patients (n = 83/121) and compared to a group of patients with myositis without anti-MDA5 antibody (anti-MDA5-; n = 190/201) based on selected variables, collected retrospectively, without any missing data. RESULTS: Within anti-MDA5+ patients (n = 83), 3 subgroups were identified. One group (18.1%) corresponded to patients with a rapidly progressive ILD (93.3%; p < 0.0001 across all) and a very high mortality rate. The second subgroup (55.4%) corresponded to patients with pure dermato-rheumatologic symptoms (arthralgia; 82.6%; p < 0.01) and a good prognosis. The third corresponded to patients, mainly male (72.7%; p < 0.0001), with severe skin vasculopathy, frequent signs of myositis (proximal weakness: 68.2%; p < 0.0001), and an intermediate prognosis. Raynaud phenomenon, arthralgia/arthritis, and sex permit the cluster appurtenance (83.3% correct estimation). Nevertheless, an unsupervised analysis confirmed that anti-MDA5 antibody delineates an independent group of patients (e.g., dermatomyositis skin rash, skin ulcers, calcinosis, mechanic's hands, ILD, arthralgia/arthritis, and high mortality rate) distinct from anti-MDA5- patients with myositis. CONCLUSION: Anti-MDA5+ patients have a systemic syndrome distinct from other patients with myositis. Three subgroups with different prognosis exist. CI - © 2020 American Academy of Neurology. FAU - Allenbach, Yves AU - Allenbach Y AUID- ORCID: 0000-0002-3185-7993 AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. yves.allenbach@aphp.fr. FAU - Uzunhan, Yurdagul AU - Uzunhan Y AUID- ORCID: 0000-0002-1607-1407 AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Toquet, Ségolène AU - Toquet S AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Leroux, Gaëlle AU - Leroux G AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Gallay, Laure AU - Gallay L AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Marquet, Alicia AU - Marquet A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Meyer, Alain AU - Meyer A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Guillaud, Constance AU - Guillaud C AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Limal, Nicolas AU - Limal N AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Gagnadoux, Frédéric AU - Gagnadoux F AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Hervier, Baptiste AU - Hervier B AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Borie, Raphaël AU - Borie R AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Deligny, Christophe AU - Deligny C AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Terrier, Benjamin AU - Terrier B AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Berezne, Alice AU - Berezne A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Audia, Sylvain AU - Audia S AUID- ORCID: 0000-0003-1772-1392 AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Champtiaux, Nicolas AU - Champtiaux N AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Devilliers, Hervé AU - Devilliers H AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Voermans, Nicol AU - Voermans N AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Diot, Elizabeth AU - Diot E AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Servettaz, Amélie AU - Servettaz A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Marhadour, Thierry AU - Marhadour T AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Castelain, Vincent AU - Castelain V AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Humbert, Sébastien AU - Humbert S AUID- ORCID: 0000-0003-0454-0840 AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Blanchard-Delaunay, Claire AU - Blanchard-Delaunay C AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Tieulie, Nathalie AU - Tieulie N AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Charles, Pierre AU - Charles P AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Gerin, Magdalena AU - Gerin M AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Mekinian, Arsène AU - Mekinian A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Priou, Pascaline AU - Priou P AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Meurice, Jean Claude AU - Meurice JC AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Tazi, Abdellatif AU - Tazi A AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Cottin, Vincent AU - Cottin V AUID- ORCID: 0000-0002-5591-0955 AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Miyara, Makoto AU - Miyara M AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Grange, Benjamin AU - Grange B AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Israël-Biet, Dominique AU - Israël-Biet D AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Phin-Huynh, Sophie AU - Phin-Huynh S AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Bron, Camille AU - Bron C AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - De Saint Martin, Luc AU - De Saint Martin L AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Fabien, Nicole AU - Fabien N AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Mariampillai, Kubéraka AU - Mariampillai K AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Nunes, Hilario AU - Nunes H AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. FAU - Benveniste, Olivier AU - Benveniste O AD - From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France. CN - French Myositis Network LA - eng PT - Journal Article DEP - 20200602 PL - United States TA - Neurology JT - Neurology JID - 0401060 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - EC 3.6.1.- (IFIH1 protein, human) RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) SB - IM CIN - Front Immunol. 2024 Jan 08;14:1319957. doi: 10.3389/fimmu.2023.1319957. PMID: 38259447 MH - Adult MH - Autoantibodies/immunology MH - Autoantigens/immunology MH - *Biological Variation, Population MH - Dermatomyositis/*classification/complications/*immunology MH - Female MH - Humans MH - Interferon-Induced Helicase, IFIH1/*immunology MH - Lung Diseases/etiology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Rheumatic Diseases/etiology MH - Vascular Diseases/etiology PMC - PMC7371381 EDAT- 2020/06/04 06:00 MHDA- 2020/09/29 06:00 PMCR- 2020/07/07 CRDT- 2020/06/04 06:00 PHST- 2019/02/14 00:00 [received] PHST- 2019/12/12 00:00 [accepted] PHST- 2020/06/04 06:00 [pubmed] PHST- 2020/09/29 06:00 [medline] PHST- 2020/06/04 06:00 [entrez] PHST- 2020/07/07 00:00 [pmc-release] AID - WNL.0000000000009727 [pii] AID - NEUROLOGY2019975805 [pii] AID - 10.1212/WNL.0000000000009727 [doi] PST - ppublish SO - Neurology. 2020 Jul 7;95(1):e70-e78. doi: 10.1212/WNL.0000000000009727. Epub 2020 Jun 2. PMID- 35790961 OWN - NLM STAT- MEDLINE DCOM- 20220707 LR - 20220716 IS - 1465-993X (Electronic) IS - 1465-9921 (Print) IS - 1465-9921 (Linking) VI - 23 IP - 1 DP - 2022 Jul 5 TI - Decline in forced vital capacity in subjects with systemic sclerosis-associated interstitial lung disease in the SENSCIS trial compared with healthy reference subjects. PG - 178 LID - 10.1186/s12931-022-02095-6 [doi] LID - 178 AB - BACKGROUND: The forced vital capacity (FVC) of healthy individuals depends on their age, sex, ethnicity and height. Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterised by loss of FVC. We compared FVC values in the subjects with SSc-ILD in the SENSCIS trial of nintedanib versus placebo with values from hypothetical matched healthy references. METHODS: The SENSCIS trial enrolled subjects with SSc with first non-Raynaud symptom in the prior ≤ 7 years, extent of fibrotic ILD on HRCT ≥ 10%, and FVC ≥ 40% predicted. FVC at baseline and decline in FVC over 52 weeks were compared with FVC values in hypothetical healthy reference subjects matched 1:1 to the subjects in the trial for age, sex, ethnicity and height, determined using equations published by the European Respiratory Society Global Lung Function Initiative. RESULTS: At baseline, mean (SD) FVC was 2460 (737) mL in the nintedanib group (n = 287) compared with 3403 (787) mL in the hypothetical matched healthy references. Mean (SD) FVC was 2544 (817) mL in the placebo group (n = 286) compared with 3516 (887) mL in the hypothetical matched healthy references. Mean (SE) changes in FVC at week 52, i.e., age-related loss of lung function, in the hypothetical healthy references matched to the nintedanib and placebo groups, respectively, were - 26.3 (0.5) mL and - 25.8 (0.5) mL. The difference in the change in FVC at week 52 between the nintedanib group and the hypothetical healthy references was 26.6 mL (95% CI: 1.2, 52.0; p = 0.04). The difference in the change in FVC at week 52 between the placebo group and the hypothetical healthy references was 77.5 mL (95% CI: 51.4, 103.7; p < 0.0001). CONCLUSIONS: Subjects with SSc-ILD in the SENSCIS trial had impaired lung function at baseline and experienced further deterioration over 52 weeks. The decline in FVC in the placebo group was four-fold greater than in a hypothetical group of matched healthy references, whereas the decline in FVC in patients who received nintedanib was two-fold greater than in hypothetical healthy references. These data highlight the clinical relevance of the slowing of FVC decline provided by nintedanib. Trial registration Registered 5 November 2015, https://clinicaltrials.gov/ct2/show/NCT02597933 . CI - © 2022. The Author(s). FAU - Maher, Toby M AU - Maher TM AD - Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. Toby.Maher@med.usc.edu. AD - National Heart and Lung Institute, Imperial College, London, UK. Toby.Maher@med.usc.edu. FAU - Bourdin, Arnaud AU - Bourdin A AD - PhyMedExp, University of Montpellier, INSERM U1046, CNRS, UMR 9214, Montpellier, France. AD - Department of Respiratory Diseases, University of Montpellier, CHU Montpellier, Montpellier, France. FAU - Volkmann, Elizabeth R AU - Volkmann ER AD - Department of Medicine, Division of Rheumatology, David Geffen School of Medicine, University of California, Los Angeles, CA, USA. FAU - Vettori, Serena AU - Vettori S AD - UOC Di Fisiopatologia E Riabilitazione Respiratoria, Ospedale Monaldi, Naples, Italy. FAU - Distler, Jörg H W AU - Distler JHW AD - Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Universitätsklinikum Erlangen, Erlangen, Germany. FAU - Alves, Margarida AU - Alves M AD - TA Inflammation Med, Boehringer Ingelheim International GmbH, Ingelheim, Germany. FAU - Stock, Christian AU - Stock C AD - Global Biostatistics and Data Sciences, Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim Am Rhein, Germany. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. LA - eng SI - ClinicalTrials.gov/NCT02597933 PT - Comparative Study PT - Journal Article DEP - 20220705 PL - England TA - Respir Res JT - Respiratory research JID - 101090633 SB - IM MH - Humans MH - Lung MH - *Lung Diseases, Interstitial/diagnosis/drug therapy/etiology MH - *Scleroderma, Systemic/complications/diagnosis/drug therapy MH - Vital Capacity PMC - PMC9258095 OTO - NOTNLM OT - Connective tissue diseases OT - Pulmonary fibrosis OT - Scleroderma, systemic OT - Vital capacity COIS- TMM reports consulting fees from AstraZeneca, Bayer, Blade Therapeutics, BI, Bristol Myers Squibb, Galapagos, Galecto, GlaxoSmithKline, IQVIA, Pliant, Respivant Sciences, Roche/Genentech, Theravance Biopharma, Veracyte and payment for presentations from BI and Roche/Genentech. AB reports grants from AstraZeneca and BI; consulting fees from Amgen, AstraZeneca, BI, Chiesi, GlaxoSmithKline, Novartis, Regeneron, Sanofi; payment for presentations, speakers bureaus, or educational events, and support for attending meetings from AstraZeneca, BI, Chiesi, GlaxoSmithKline, Novartis, Regeneron; and participation on a Data Safety Monitoring Board or advisory board for AB Science. ERV reports grants from BI, Forbius, Kadmon, Horizon and fees from BI for serving on advisory boards and for presentations. SV reports consulting fees from BI. JHWD reports grants, consulting fees and payment for presentations, speakers bureaus, or educational events from BI and stock in 4D Science. MA and CS are employees of Boehringer Ingelheim. OD has received grants BI, Kymera, Mitsubishi Tanabe; consultancy fees from AbbVie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Bayer, Beacon, Blade Therapeutics, BI, Corbus, CSL Behring, 4P Science, Galapagos, Glenmark Pharmaceuticals, Horizon Therapeutics (Curzion), Inventiva, Kymera, Lupin, Merck Sharp & Dohme, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Pfizer, Prometheus Biosciences, Roivant Sciences, Sanofi, Topadur; speaking fees from Bayer, BI, Janssen, Medscape; he holds patent US8247389 for the treatment of SSc; he is Chair of the Executive Committee of the FOREUM Foundation, co-chair of a ERS/EULAR guidelines committee, President of EUSTAR, a Member of the Board of Trustees for the Swiss Clinical Quality Management in Rheumatic Diseases and the Hartmann Müller Foundation, and a Senate member of the Swiss Academy of Medical Sciences. EDAT- 2022/07/06 06:00 MHDA- 2022/07/08 06:00 PMCR- 2022/07/05 CRDT- 2022/07/05 23:32 PHST- 2022/03/02 00:00 [received] PHST- 2022/06/19 00:00 [accepted] PHST- 2022/07/05 23:32 [entrez] PHST- 2022/07/06 06:00 [pubmed] PHST- 2022/07/08 06:00 [medline] PHST- 2022/07/05 00:00 [pmc-release] AID - 10.1186/s12931-022-02095-6 [pii] AID - 2095 [pii] AID - 10.1186/s12931-022-02095-6 [doi] PST - epublish SO - Respir Res. 2022 Jul 5;23(1):178. doi: 10.1186/s12931-022-02095-6. PMID- 38251534 OWN - NLM STAT- MEDLINE DCOM- 20240123 LR - 20260331 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 5 IP - 11 DP - 2023 Nov TI - Beyond very early systemic sclerosis: deciphering pre‑scleroderma and its trajectories to open new avenues for preventive medicine. PG - e683-e694 LID - S2665-9913(23)00212-6 [pii] LID - 10.1016/S2665-9913(23)00212-6 [doi] AB - The identification of individuals with systemic sclerosis in an oligosymptomatic phase preceding the very early manifestations of the disease represents a challenge in the search for a new window of opportunity in systemic sclerosis. This phase could be identified in a clinical scenario as the pre-scleroderma phase, in which the disease would still be far from systemic sclerosis-related fibrotic or irreversible manifestations in skin or organs. In this Personal View, we discuss parameters and candidate definitions for a conceptual framework of pre-scleroderma, from the identification of populations at risk to autoantibodies and their potential functional activities. We discuss how this new paradigm of pre-scleroderma could represent a game-changing approach in the management of systemic sclerosis, allowing the treatment of patients at high risk of organ involvement or skin fibrosis before such events occur. CI - Copyright © 2023 Elsevier Ltd. All rights reserved. FAU - Lescoat, Alain AU - Lescoat A AD - Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France; Institut de Recherche en Sante, Environnement, et Travail, CHU Rennes, University of Rennes, Inserm, EHESP, Rennes, France. Electronic address: alain.lescoat@chu-rennes.fr. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy. FAU - Campochiaro, Corrado AU - Campochiaro C AD - Unit of Immunology, Rheumatology, Allergy, and Rare diseases, IRCCS San Raffaele Hospital, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy. FAU - Del Galdo, Francesco AU - Del Galdo F AD - Department of Rheumatology, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology, Division of Medicine, Royal Free Campus, University College London, London, UK. FAU - Farrington, Sue AU - Farrington S AD - Scleroderma & Raynaud Society UK, London, UK; Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK. FAU - Galetti, Ilaria AU - Galetti I AD - Federation of European Scleroderma Associations, Brussels, Belgium. FAU - Khanna, Dinesh AU - Khanna D AD - University of Michigan Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan. FAU - Truchetet, Marie-Elise AU - Truchetet ME AD - Department of Rheumatology, UMR5164 ImmunoConcept, Bordeaux University, Bordeaux University Hospital, CNRS, Bordeaux, France. FAU - Allanore, Yannick AU - Allanore Y AD - INSERM U1016 UMR 8104, Université Paris Cité, Hôpital Cochin, Paris, France. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Division of Rheumatology, AOUC, Florence, Italy; Unit of Immunology, Rheumatology, Allergy, and Rare diseases, IRCCS San Raffaele Hospital, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy. LA - eng PT - Journal Article PT - Review DEP - 20230926 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 RN - 0 (Autoantibodies) SB - IM CIN - Lancet Rheumatol. 2024 Apr;6(4):e200. doi: 10.1016/S2665-9913(24)00057-2. PMID: 38508818 MH - Humans MH - *Scleroderma, Systemic/diagnosis MH - *Scleroderma, Localized/diagnosis MH - Skin MH - Autoantibodies MH - *Basidiomycota COIS- Declaration of interests FDG declares grants and contracts from AbbVie and Boehringer Ingelheim. CPD declares grants and contracts from GSK, Horizon, Servier, and Arxx Therapeutics; consulting fees from Boehringer Ingelheim, Roche, CSL Behring, Acceleron, Horizon, Arxx Therapeutics, Centessa, AstraZeneca, Lilly, and Novartis; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Boehringer Ingelheim and Janssen. DK declares grants and contracts from BMS, Bayer, and Pfizer; consulting fees from AbbVie, Genentech, GSK, Boehringer Ingelheim, AstraZeneca, Horizon, UCB, and Prometheus; and participation on a data safety monitoring board or advisory board for AbbVie. MK declares grants and contracts from Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, AstraZeneca, and GSK; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Boehringer Ingelheim, Chugai, and Janssen; and participation on a data safety monitoring board or advisory board for Argenx. M-ET declares consulting fees from Boehringer Ingelheim, AbbVie, and UCB; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Lilly, AbbVie, and UCB; and support for attending meetings and travel from Novartis and AbbVie. MMC declared payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Boehringer Ingelheim, Biogen, and Lilly. All other authors declare no competing interests. EDAT- 2024/01/22 12:42 MHDA- 2024/01/23 06:42 CRDT- 2024/01/22 07:24 PHST- 2023/05/26 00:00 [received] PHST- 2023/07/09 00:00 [revised] PHST- 2023/08/01 00:00 [accepted] PHST- 2024/01/23 06:42 [medline] PHST- 2024/01/22 12:42 [pubmed] PHST- 2024/01/22 07:24 [entrez] AID - S2665-9913(23)00212-6 [pii] AID - 10.1016/S2665-9913(23)00212-6 [doi] PST - ppublish SO - Lancet Rheumatol. 2023 Nov;5(11):e683-e694. doi: 10.1016/S2665-9913(23)00212-6. Epub 2023 Sep 26. PMID- 23106313 OWN - NLM STAT- MEDLINE DCOM- 20141014 LR - 20140219 IS - 1468-3083 (Electronic) IS - 0926-9959 (Linking) VI - 28 IP - 3 DP - 2014 Mar TI - Familial benign chronic pemphigus and doxycycline: a review of 6 cases. PG - 370-3 LID - 10.1111/jdv.12016 [doi] AB - BACKGROUND: Hailey-Hailey disease (HHD) or familial benign chronic pemphigus is a rare autosomal dominant inherited skin disorder, characterized by flaccid vesicles and erosions on the intertriginous areas. Current treatments are not particularly effective. We report 6 cases dramatically improving with doxycycline. CASE REPORTS: 6 patients, aged from 33 to 77 years old, presented with a variable 4 to 40 year history of severe treatment-resistant HHD. All 6 patients were then treated successfully with doxycycline 100 mg per day for at least 3 months. DISCUSSION: An improvement was observed in all 6 patients from 1 week to 3 months after the beginning of treatment. Relapses were observed after various periods. Maintenance half-dose therapy seemed to be beneficial in patients experiencing recurrence. Only one patient developed gastro-intestinal intolerance. No other side effects were reported. Currently, 2 patients have improved and present a decreased number of exacerbations, 2 others are in complete remission after more than 5 years of follow-up. Treatment efficiency is difficult to evaluate in HHD as it is a rare condition. No controlled studies have been published. Local treatments may improve inflammation but do not treat the underlying cause, targeted systemic therapies exist but there is little evidence supporting their use, physical treatments are cumbersome. Besides their antibiotic potential, tetracycline antibiotics also have anti-inflammatory properties and anticollagenase activity via inhibition of matrix metalloproteinases. CONCLUSIONS:   Doxycycline appears to be an interesting therapeutic option in Hailey-Hailey disease. CI - © 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology. FAU - Le Saché-de Peufeilhoux, L AU - Le Saché-de Peufeilhoux L AD - Department of Dermatology, Hôpital Necker-Enfants Malades, APHP, Université Paris DescartesNational Reference Centre for Genodermatosis, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, ParisDepartment of Dermatology, Centre de Santé Irène Lézine, ArgenteuilMedical Office of Dermatology, Beaumont-sur-OiseDepartment of Pathology, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France. FAU - Raynaud, E AU - Raynaud E FAU - Bouchardeau, A AU - Bouchardeau A FAU - Fraitag, S AU - Fraitag S FAU - Bodemer, C AU - Bodemer C LA - eng PT - Journal Article PT - Review DEP - 20121027 PL - England TA - J Eur Acad Dermatol Venereol JT - Journal of the European Academy of Dermatology and Venereology : JEADV JID - 9216037 RN - N12000U13O (Doxycycline) SB - IM MH - Adult MH - Aged MH - Doxycycline/*therapeutic use MH - Female MH - Humans MH - Male MH - Middle Aged MH - Pemphigus, Benign Familial/*drug therapy EDAT- 2012/10/31 06:00 MHDA- 2014/10/15 06:00 CRDT- 2012/10/31 06:00 PHST- 2012/10/31 06:00 [entrez] PHST- 2012/10/31 06:00 [pubmed] PHST- 2014/10/15 06:00 [medline] AID - 10.1111/jdv.12016 [doi] PST - ppublish SO - J Eur Acad Dermatol Venereol. 2014 Mar;28(3):370-3. doi: 10.1111/jdv.12016. Epub 2012 Oct 27. PMID- 22672190 OWN - NLM STAT- MEDLINE DCOM- 20130225 LR - 20121002 IS - 1540-8183 (Electronic) IS - 0896-4327 (Linking) VI - 25 IP - 5 DP - 2012 Oct TI - Simultaneous dual vascular access site for the treatment of coronary artery bifurcation lesions by complex 2-stent technique. PG - 439-46 LID - 10.1111/j.1540-8183.2012.00745.x [doi] AB - OBJECTIVES: To propose an original approach based on simultaneous dual vascular access site (DAS) using 2 small-size guiding catheters to easily perform complex 2-stent techniques for bifurcation coronary lesions (BL). BACKGROUND: Simultaneous kissing stenting and classic crush technique require large 7 or 8Fr guiding catheters leading to large amounts of contrast medium, vascular access site complications, and sometimes frictions or criss-cross of the 2-stent delivery systems. METHODS: DAS was used in 30 patients with BL (11 radio-radial, 16 radio-femoral, and 3 femoro-femoral). Among 60 guiding catheters, the size was 5Fr in 28, 6Fr in 30, and 7Fr in 2 cases of double adjacent BL. When 2 different size catheters were used, contrast medium injections were done using the smallest size catheter. DAS patients were compared with a group of 30 BL patients treated using a single femoral vascular access site (SAS) with 7 or 8Fr catheters. RESULTS: Success rate was 100% in all patients. Contrast volume used was smaller in DAS than in SAS patients (277 ± 156 cc vs. 380 ± 165 cc,P = 0.01). No vascular access site complication occurred in the sub-group of the 11 DAS radio-radial patients. Postintervention hospitalization duration was shorter in DAS than in SAS (1.9 ± 2 vs. 2.8 ± 2 days,P = 0.048). CONCLUSION: DAS allows to successfully perform complex stenting technique of BL using small-size guiding catheters leading to decreased contrast medium volume, decreased vascular access site complications rates, and shortened hospitalization duration. CI - ©2012, Wiley Periodicals, Inc. FAU - Isaaz, Karl AU - Isaaz K AD - Division of Cardiology, University of Saint Etienne, Saint Etienne, France. isaaz@univ-st-etienne.fr FAU - Mayaud, Norbert AU - Mayaud N FAU - Lamaud, Michel AU - Lamaud M FAU - Raynaud, Amélie AU - Raynaud A FAU - Cerisier, Alexis AU - Cerisier A FAU - Sabry, Mohammed Hassan AU - Sabry MH FAU - Richard, Laure AU - Richard L FAU - Khamis, Hazem AU - Khamis H FAU - Abd-Alaziz, Ahmad AU - Abd-Alaziz A FAU - DA Costa, Antoine AU - DA Costa A LA - eng PT - Journal Article DEP - 20120607 PL - United States TA - J Interv Cardiol JT - Journal of interventional cardiology JID - 8907826 RN - 0 (Contrast Media) SB - IM MH - Aged MH - Analysis of Variance MH - Cardiac Catheterization/*methods MH - Contrast Media MH - Coronary Artery Disease/diagnosis/*therapy MH - Coronary Vessels/*pathology MH - Female MH - Humans MH - Male MH - Retrospective Studies MH - *Stents MH - Time Factors MH - *Vascular Access Devices EDAT- 2012/06/08 06:00 MHDA- 2013/02/26 06:00 CRDT- 2012/06/08 06:00 PHST- 2012/06/08 06:00 [entrez] PHST- 2012/06/08 06:00 [pubmed] PHST- 2013/02/26 06:00 [medline] AID - 10.1111/j.1540-8183.2012.00745.x [doi] PST - ppublish SO - J Interv Cardiol. 2012 Oct;25(5):439-46. doi: 10.1111/j.1540-8183.2012.00745.x. Epub 2012 Jun 7. PMID- 20336324 OWN - NLM STAT- MEDLINE DCOM- 20101102 LR - 20211020 IS - 1432-198X (Electronic) IS - 0931-041X (Linking) VI - 25 IP - 9 DP - 2010 Sep TI - Favorable outcome in a case of Mycoplasma pneumoniae-associated crescentic glomerulonephritis. PG - 1765-9 LID - 10.1007/s00467-010-1491-4 [doi] AB - Mycoplasma pneumoniae-associated nephritis has been reported in children with various pathological findings. It nevertheless remains an uncommon disease and, within this clinical context, endo-and extracapillary glomerulonephritis in a child has never been described. We report here a case of a 3-year-old girl diagnosed with severe crescentic glomerulonephritis associated with M. pneumoniae infection who presented with nephrotic syndrome and impaired renal function. The serum C3 complement level was initially low but returned to normal after 1 month. Two courses of three methylprednisolone pulses were administered in association with plasmapheresis and, secondarily, mycophenolate mophetil. This treatment regimen led to disease remission and a favorable renal outcome at the 6-month follow-up. However, the treatment guidelines in this situation remain debatable. FAU - Adra, Anne-Laure AU - Adra AL AD - Néphrologie Pédiatrique-Centre de Référence des Maladies Rénales Rares du Sud-Ouest-CHU Montpellier, Université Montpellier I, Montpellier, France. FAU - Vigue, Marie-Gabrielle AU - Vigue MG FAU - Dalla Vale, Fabienne AU - Dalla Vale F FAU - Ichay, Lydia AU - Ichay L FAU - Raynaud, Pierre AU - Raynaud P FAU - Mariani, Aude AU - Mariani A FAU - Morin, Denis AU - Morin D LA - eng PT - Case Reports PT - Journal Article DEP - 20100325 PL - Germany TA - Pediatr Nephrol JT - Pediatric nephrology (Berlin, Germany) JID - 8708728 RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) RN - EC 3.4.21.- (Complement C3-C5 Convertases) RN - HU9DX48N0T (Mycophenolic Acid) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Biomarkers/blood MH - Child, Preschool MH - Complement C3-C5 Convertases/metabolism MH - Drug Administration Schedule MH - Female MH - Glomerulonephritis/immunology/microbiology/*therapy MH - Humans MH - Immunosuppressive Agents/*administration & dosage MH - Methylprednisolone/*administration & dosage MH - Mycophenolic Acid/administration & dosage/*analogs & derivatives MH - Mycoplasma pneumoniae/*isolation & purification MH - Nephrotic Syndrome/microbiology/therapy MH - *Plasmapheresis MH - Pneumonia, Mycoplasma/*complications MH - Proteinuria/microbiology/therapy MH - Pulse Therapy, Drug MH - Severity of Illness Index MH - Time Factors MH - Treatment Outcome EDAT- 2010/03/26 06:00 MHDA- 2010/11/03 06:00 CRDT- 2010/03/26 06:00 PHST- 2009/07/28 00:00 [received] PHST- 2010/01/28 00:00 [accepted] PHST- 2010/01/26 00:00 [revised] PHST- 2010/03/26 06:00 [entrez] PHST- 2010/03/26 06:00 [pubmed] PHST- 2010/11/03 06:00 [medline] AID - 10.1007/s00467-010-1491-4 [doi] PST - ppublish SO - Pediatr Nephrol. 2010 Sep;25(9):1765-9. doi: 10.1007/s00467-010-1491-4. Epub 2010 Mar 25. PMID- 30053890 OWN - NLM STAT- MEDLINE DCOM- 20190312 LR - 20240329 IS - 1757-6512 (Electronic) IS - 1757-6512 (Linking) VI - 9 IP - 1 DP - 2018 Jul 27 TI - The march of pluripotent stem cells in cardiovascular regenerative medicine. PG - 201 LID - 10.1186/s13287-018-0947-5 [doi] LID - 201 AB - Cardiovascular disease (CVD) continues to be the leading cause of global morbidity and mortality. Heart failure remains a major contributor to this mortality. Despite major therapeutic advances over the past decades, a better understanding of molecular and cellular mechanisms of CVD as well as improved therapeutic strategies for the management or treatment of heart failure are increasingly needed. Loss of myocardium is a major driver of heart failure. An attractive approach that appears to provide promising results in reducing cardiac degeneration is stem cell therapy (SCT). In this review, we describe different types of stem cells, including embryonic and adult stem cells, and we provide a detailed discussion of the properties of induced pluripotent stem cells (iPSCs). We also present and critically discuss the key methods used for converting somatic cells to pluripotent cells and iPSCs to cardiomyocytes (CMs), along with their advantages and limitations. Integrating and non-integrating reprogramming methods as well as characterization of iPSCs and iPSC-derived CMs are discussed. Furthermore, we critically present various methods of differentiating iPSCs to CMs. The value of iPSC-CMs in regenerative medicine as well as myocardial disease modeling and cardiac regeneration are emphasized. FAU - Abou-Saleh, Haissam AU - Abou-Saleh H AD - Department of Biological and Environmental Sciences, Qatar University, Doha, Qatar. FAU - Zouein, Fouad A AU - Zouein FA AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - El-Yazbi, Ahmed AU - El-Yazbi A AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. AD - Department of Pharmacology and Toxicology, Alexandria University, Alexandria, Egypt. FAU - Sanoudou, Despina AU - Sanoudou D AD - Clinical Genomics and Pharmacogenomics Unit, 4th Department of Internal Medicine, "Attikon" Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece. FAU - Raynaud, Christophe AU - Raynaud C AD - Sidra Medical and Research Center, Doha, Qatar. FAU - Rao, Christopher AU - Rao C AD - Department of Surgery, Queen Elizabeth Hospital, Woolwich, London, UK. FAU - Pintus, Gianfranco AU - Pintus G AD - Department of Biomedical Sciences, College of Health Sciences, Qatar University, Doha, Qatar. FAU - Dehaini, Hassan AU - Dehaini H AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. FAU - Eid, Ali H AU - Eid AH AD - Department of Biological and Environmental Sciences, Qatar University, Doha, Qatar. ae81@aub.edu.lb. AD - Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon. ae81@aub.edu.lb. AD - Department of Biomedical Sciences, College of Health Sciences, Qatar University, Doha, Qatar. ae81@aub.edu.lb. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20180727 PL - England TA - Stem Cell Res Ther JT - Stem cell research & therapy JID - 101527581 SB - IM MH - Cardiovascular Diseases/*genetics MH - Cell Differentiation MH - Cell- and Tissue-Based Therapy/*methods MH - Humans MH - Myocytes, Cardiac/*metabolism MH - Pluripotent Stem Cells/metabolism MH - Regenerative Medicine/*methods PMC - PMC6062943 OTO - NOTNLM OT - Cardiomyocytes OT - Cardiovascular disease OT - Heart failure OT - Regenerative medicine OT - Stem cell therapy OT - iPSCs COIS- ETHICS APPROVAL: Not applicable. CONSENT FOR PUBLICATION: Not applicable. COMPETING INTERESTS: The authors declare that they have no competing interest. PUBLISHER’S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2018/07/29 06:00 MHDA- 2019/03/13 06:00 PMCR- 2018/07/27 CRDT- 2018/07/29 06:00 PHST- 2018/07/29 06:00 [entrez] PHST- 2018/07/29 06:00 [pubmed] PHST- 2019/03/13 06:00 [medline] PHST- 2018/07/27 00:00 [pmc-release] AID - 10.1186/s13287-018-0947-5 [pii] AID - 947 [pii] AID - 10.1186/s13287-018-0947-5 [doi] PST - epublish SO - Stem Cell Res Ther. 2018 Jul 27;9(1):201. doi: 10.1186/s13287-018-0947-5. PMID- 32121447 OWN - NLM STAT- MEDLINE DCOM- 20201207 LR - 20221207 IS - 1420-3049 (Electronic) IS - 1420-3049 (Linking) VI - 25 IP - 5 DP - 2020 Feb 29 TI - Use of a Pleurotus ostreatus Complex Cell Wall Extract as Elicitor of Plant Defenses: From Greenhouse to Field Trial. LID - 10.3390/molecules25051094 [doi] LID - 1094 AB - Fungi constitute an abundant source of natural polysaccharides, some of them harboring original structures which can induce responses in mammalian or plant cells. An alkaline extract from the edible mushroom Pleurotus ostreatus has been obtained and called Pleuran complex cell wall extract (CCWE). It consists of a glucan-peptide complex whose components fall in a quite broad range of molecular weights, from 30 to 80 kDa. Pleuran extract has been tested on cultivated plants in laboratory conditions and also during field trial for its capacity to stimulate plant defenses in response to pathogen attack. Following Pleuran CCWE treatment, enhanced levels of various biochemical markers associated with plant responses have been observed, including enzymatic activities (e.g., peroxidase) or expression of some pathogenesis-related genes. In addition, during field experiments, we have noticed significant reductions in disease symptom levels in relation to different plant/pathogen systems (wheat/septoria, vine/mildew). These results confirmed that Pleuran CCWE could be used as an elicitor of plant defenses and could help in reducing pesticide applications against plant pathogens. FAU - Faugeron-Girard, Céline AU - Faugeron-Girard C AD - Université de Limoges, Laboratoire Peirene (EA7500), Faculté des Sciences et Techniques, 123, avenue Albert Thomas, 87060 Limoges CEDEX, France. FAU - Gloaguen, Vincent AU - Gloaguen V AD - Université de Limoges, Laboratoire Peirene (EA7500), Faculté des Sciences et Techniques, 123, avenue Albert Thomas, 87060 Limoges CEDEX, France. FAU - Koçi, Rromir AU - Koçi R AD - Université de Limoges, Laboratoire Peirene (EA7500), Faculté des Sciences et Techniques, 123, avenue Albert Thomas, 87060 Limoges CEDEX, France. FAU - Célérier, Julien AU - Célérier J AD - COVERTIS SAS, Technopole d'ESTER, 1 avenue d'Ester, 87069 Limoges CEDEX, France. FAU - Raynaud, Anaïs AU - Raynaud A AD - COVERTIS SAS, Technopole d'ESTER, 1 avenue d'Ester, 87069 Limoges CEDEX, France. FAU - Moine, Charlotte AU - Moine C AD - COVERTIS SAS, Technopole d'ESTER, 1 avenue d'Ester, 87069 Limoges CEDEX, France. LA - eng GR - Phytodev/Région Limousin/ GR - Reduphyt/Région Nouvelle Aquitaine/ PT - Journal Article DEP - 20200229 PL - Switzerland TA - Molecules JT - Molecules (Basel, Switzerland) JID - 100964009 RN - 0 (Complex Mixtures) RN - 0 (Fungal Polysaccharides) SB - IM MH - Cell Wall/*chemistry MH - *Complex Mixtures/chemistry/pharmacology MH - *Fungal Polysaccharides/chemistry/pharmacology MH - Solanum lycopersicum/*growth & development/microbiology MH - Plant Diseases/*prevention & control MH - Pleurotus/*chemistry PMC - PMC7179115 OTO - NOTNLM OT - Pleurotus ostreatus OT - glucan-rich complex OT - plant defenses COIS- The authors declare no conflict of interest. EDAT- 2020/03/04 06:00 MHDA- 2020/12/15 06:00 PMCR- 2020/02/29 CRDT- 2020/03/04 06:00 PHST- 2020/01/30 00:00 [received] PHST- 2020/02/25 00:00 [revised] PHST- 2020/02/26 00:00 [accepted] PHST- 2020/03/04 06:00 [entrez] PHST- 2020/03/04 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2020/02/29 00:00 [pmc-release] AID - molecules25051094 [pii] AID - molecules-25-01094 [pii] AID - 10.3390/molecules25051094 [doi] PST - epublish SO - Molecules. 2020 Feb 29;25(5):1094. doi: 10.3390/molecules25051094. PMID- 25554662 OWN - NLM STAT- MEDLINE DCOM- 20151125 LR - 20150209 IS - 0151-9638 (Print) IS - 0151-9638 (Linking) VI - 142 IP - 2 DP - 2015 Feb TI - [Allgrove syndrome]. PG - 121-4 LID - S0151-9638(14)01291-5 [pii] LID - 10.1016/j.annder.2014.11.012 [doi] AB - BACKGROUND: Allgrove syndrome or "Triple A syndrome" involves adrenal insufficiency as a result of resistance to adrenocorticotropic hormone (ACTH), achalasia and alacrima, often associated with neurological signs. Herein, we report a new case of this rare genetic disease, which is of interest because of its dermatological mode of discovery. PATIENTS AND METHODS: A 4-year-old child, born to parents related by first-degree consanguinity, presented oral hyperpigmentation and diffused acquired melanoderma, as well as long-standing dry-eye syndrome. Laboratory tests confirmed low adrenal insufficiency. The combination of alacrima and adrenal insufficiency prompted screening for Allgrove syndrome, which was confirmed by genetic analysis showing homozygous c.1331+1G>A mutation within intron 14 of the gene encoding for ALADIN protein. Both parents were heterozygous for the same mutation. Two years later, the onset of vomiting raised concerns about achalasia, which was confirmed by oesophageal manometry. The child received symptomatic treatment consisting of supplementary hydrocortisone and oesophageal dilatation. DISCUSSION: The present case serves as a reminder that Allgrove syndrome may be diagnosed by dermatologists. Therapy is cross-disciplinary, being based upon medical treatment for adrenal insufficiency with prescription of artificial tears in the event of alacrima. Achalasia is treated by oesophageal dilatation or by surgery. CI - Copyright © 2014 Elsevier Masson SAS. All rights reserved. FAU - Alakeel, A AU - Alakeel A AD - Dermatologie pédiatrique, service de pédiatrie-pneumologie, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France. Electronic address: dr_alakeel@hotmail.com. FAU - Raynaud, C AU - Raynaud C AD - Service d'endocrinologie, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France. FAU - Rossi, M AU - Rossi M AD - Service de génétique médicale, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France. FAU - Reix, P AU - Reix P AD - Service de pédiatrie-pneumologie, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France. FAU - Jullien, D AU - Jullien D AD - Service de dermatologie, hôpital Édouard-Herriot, pavillon R, 5, place d'Arsonval, 69003 Lyon, France. FAU - Souillet, A-L AU - Souillet AL AD - Dermatologie pédiatrique, service de pédiatrie-pneumologie, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France. LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Syndrome d'Allgrove. DEP - 20141230 PL - France TA - Ann Dermatol Venereol JT - Annales de dermatologie et de venereologie JID - 7702013 RN - Achalasia Addisonianism Alacrimia syndrome SB - IM MH - Adrenal Insufficiency/genetics/*pathology MH - Child, Preschool MH - Esophageal Achalasia/genetics/*pathology MH - Female MH - Humans MH - Phenotype OTO - NOTNLM OT - Achalasia OT - Achalasie OT - Addison (maladie d’) OT - Adrenal insufficiency OT - Alacrima OT - Alacrymie OT - Allgrove OT - Insuffisance surrénalienne OT - Triple A EDAT- 2015/01/03 06:00 MHDA- 2015/12/15 06:00 CRDT- 2015/01/03 06:00 PHST- 2014/07/01 00:00 [received] PHST- 2014/10/07 00:00 [revised] PHST- 2014/11/07 00:00 [accepted] PHST- 2015/01/03 06:00 [entrez] PHST- 2015/01/03 06:00 [pubmed] PHST- 2015/12/15 06:00 [medline] AID - S0151-9638(14)01291-5 [pii] AID - 10.1016/j.annder.2014.11.012 [doi] PST - ppublish SO - Ann Dermatol Venereol. 2015 Feb;142(2):121-4. doi: 10.1016/j.annder.2014.11.012. Epub 2014 Dec 30. PMID- 39527968 OWN - NLM STAT- MEDLINE DCOM- 20250226 LR - 20250623 IS - 2665-9913 (Electronic) IS - 2665-9913 (Linking) VI - 7 IP - 3 DP - 2025 Mar TI - Detection of decline in pulmonary function in patients with systemic sclerosis-associated interstitial lung disease using home monitoring in the Netherlands (DecreaSSc): a prospective, observational study. PG - e178-e186 LID - S2665-9913(24)00236-4 [pii] LID - 10.1016/S2665-9913(24)00236-4 [doi] AB - BACKGROUND: In patients with systemic sclerosis, interstitial lung disease (ILD) is the leading cause of mortality. Early detection of progressive ILD associated with systemic sclerosis is warranted for timely adjustment of management strategies and improved prognosis. We aimed to investigate the validity of home spirometry to detect a decline in pulmonary function in patients with systemic sclerosis-associated ILD. METHODS: DecreaSSc was a prospective, observational study done in two tertiary referral centres in the Netherlands. Eligible patients were aged 18 years or older, fulfilled the American College of Rheumatology-European Alliance of Associations for Rheumatology criteria for systemic sclerosis, had a disease duration from first non-Raynaud phenomenon symptom of 5 years or less, had high-resolution CT-confirmed diagnosis of ILD, and had a maximum immunosuppressive treatment duration of 8 weeks at baseline. Patients took weekly home spirometry measurements using a handheld spirometer for 1 year. At baseline and at semi-annual study visits, patients pulmonary function testing was done in the hospital and patients completed questionnaires on patient-reported outcome measurements. The primary outcome was the κ agreement between home and hospital measurements after 1 year to detect a decline in force vital capacity (FVC) of 5% or more, estimated using separate linear regression analyses for home-based and hospital-based FVC% predicted in individual patients. The sensitivity and specificity of home spirometry to detect an absolute decline in FVC% predicted of 5% or more was assessed using the hospital pulmonary function test as the gold standard. The longitudinal correlation between hospital and home measurements was assessed with regression analysis, whereas the cross-sectional correlation was assessed with the intraclass coefficient. People with lived experience were involved at several stages of the study. FINDINGS: Between Jan 26, 2021, and Feb 27, 2023, 43 patients were enrolled, 35 of whom completed 6 months of follow-up and 31 of whom completed 12 months of follow-up. The last follow-up visit took place on March 28, 2024. 20 (57%) of patients were women and 15 (43%) were men; 32 (91%) were White. Mean age was 57·7 years (SD 10·7). The agreement between hospital and home measurements had a κ value of 0·40 (95% CI 0·01-0·79). The sensitivity of home spirometry to detect a decline in FVC% predicted of 5% or more was 60% (95% CI 44-76) and specificity was 87% (75-98). Regression analysis showed that the course of pulmonary function was not different between hospital and home assessment as the interaction term was not significant (-0·0003 [95% CI -0·0006 to 0·000008]; p=0·057) with a longitudinal correlation of 0·55 (95% CI 0·26-0·74; p=0·0070). The intraclass coefficient between both measurements was 0·85 (95% CI 0·73-0·92; p<0·0001) at baseline, 0·84 (0·71-0·92; p<0·0001) at 6 months, and 0·72 (0·50-0·86; p<0·0001) at 12 months. INTERPRETATION: Home spirometry has the potential to detect a decline in pulmonary function in patients with systemic sclerosis-associated ILD earlier when used in addition to regular health care management. Future research could reveal whether home spirometry can contribute to improved outcomes of patients with systemic sclerosis-associated ILD. FUNDING: Galapagos and Boehringer Ingelheim. CI - Copyright © 2024 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Velauthapillai, Arthiha AU - Velauthapillai A AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. Electronic address: arthiha.velauthapillai@radboudumc.nl. FAU - Moor, Catharina C AU - Moor CC AD - Department of Pulmonology, Erasmus Medical Center, Rotterdam, Netherlands. FAU - de Vries-Bouwstra, Jeska K AU - de Vries-Bouwstra JK AD - Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands. FAU - Wijsenbeek-Lourens, Marlies S AU - Wijsenbeek-Lourens MS AD - Department of Pulmonology, Erasmus Medical Center, Rotterdam, Netherlands. FAU - van den Ende, Cornelia H M AU - van den Ende CHM AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands. LA - eng PT - Journal Article PT - Observational Study DEP - 20241108 PL - England TA - Lancet Rheumatol JT - The Lancet. Rheumatology JID - 101765308 SB - IM MH - Humans MH - Female MH - *Lung Diseases, Interstitial/physiopathology/etiology/diagnosis MH - Male MH - Middle Aged MH - Prospective Studies MH - *Scleroderma, Systemic/complications/physiopathology MH - *Spirometry/methods MH - Netherlands MH - Aged MH - Vital Capacity MH - Adult MH - *Lung/physiopathology MH - Respiratory Function Tests COIS- Declaration of interests CCM declares grants and consultancy fees from Boehringer Ingelheim, AstraZeneca, and Daiichi Sankyo, outside of the submitted work, paid to the institution. JKdV-B received consulting fees from AbbVie, Janssen, and Boehringer Ingelheim, and received research grants from Roche, Galapagos Janssen, National Association for People with Lupus, APA, Scleroderma, and MCTD, and ReumaNederland; all payments were made to her institution. MSW-L received consulting or speakers fees from AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, CSL Behring, Galapagos, Galecto, Hoffmann-La Roche, Horizon Therapeutics, Kinevant Sciences, Molecure, NeRRe, Novartis, PureTech, Thyron, Trevi, and Vicore; and grants from The Dutch Pulmonary Fibrosis Patients Association, The Dutch Lung Foundation, The Netherlands Organisation for Health Research and Development, The Thorax Foundation, Sarcoidosis.nl, AstraZeneca/DaiichiSankyo, Boehringer Ingelheim, and Hoffmann-La Roche; all fees and grants were paid to her institution. MCV received consulting fees or speakers fees from Boehringer Ingelheim, GSK, Janssen, MSD, Novartis, Boehringer Ingelheim, and Janssen; and unrestricted research grants from Boehringer Ingelheim, Ferrer, and Galapagos; all fees and grants were paid to her institution. All other authors declare no competing interests. EDAT- 2024/11/13 13:49 MHDA- 2025/02/27 05:00 CRDT- 2024/11/11 18:53 PHST- 2024/04/26 00:00 [received] PHST- 2024/07/28 00:00 [revised] PHST- 2024/07/29 00:00 [accepted] PHST- 2025/02/27 05:00 [medline] PHST- 2024/11/13 13:49 [pubmed] PHST- 2024/11/11 18:53 [entrez] AID - S2665-9913(24)00236-4 [pii] AID - 10.1016/S2665-9913(24)00236-4 [doi] PST - ppublish SO - Lancet Rheumatol. 2025 Mar;7(3):e178-e186. doi: 10.1016/S2665-9913(24)00236-4. Epub 2024 Nov 8. PMID- 38723554 OWN - NLM STAT- MEDLINE DCOM- 20240616 LR - 20250220 IS - 2352-3964 (Electronic) IS - 2352-3964 (Linking) VI - 104 DP - 2024 Jun TI - MDA5-autoimmunity and interstitial pneumonitis contemporaneous with the COVID-19 pandemic (MIP-C). PG - 105136 LID - S2352-3964(24)00171-3 [pii] LID - 10.1016/j.ebiom.2024.105136 [doi] LID - 105136 AB - BACKGROUND: Anti-MDA5 (Melanoma differentiation-associated protein-5) positive dermatomyositis (MDA5(+)-DM) is characterised by rapidly progressive interstitial lung disease (ILD) and high mortality. MDA5 is an RNA sensor and a key pattern recognition receptor for the SARS-CoV-2 virus. METHODS: This is a retrospective observational study of a surge in MDA5 autoimmunity, as determined using a 15 muscle-specific autoantibodies (MSAs) panel, between Janurary 2018 and December 2022 in Yorkshire, UK. MDA5-positivity was correlated with clinical features and outcome, and regional SARS-CoV-2 positivity and vaccination rates. Gene expression patterns in COVID-19 were compared with autoimmune lung disease and idiopathic pulmonary fibrosis (IPF) to gain clues into the genesis of the observed MDA5(+)-DM outbreak. FINDINGS: Sixty new anti-MDA5+, but not other MSAs surged between 2020 and 2022, increasing from 0.4% in 2019 to 2.1% (2020), 4.8% (2021) and 1.7% (2022). Few (8/60) had a prior history of confirmed COVID-19, peak rates overlapped with regional SARS-COV-2 community positivity rates in 2021, and 58% (35/60) had received anti-SARS-CoV-2 vaccines. 25/60 cases developed ILD which rapidly progression with death in 8 cases. Among the 35/60 non-ILD cases, 14 had myositis, 17 Raynaud phenomena and 10 had dermatomyositis spectrum rashes. Transcriptomic studies showed strong IFIH1 (gene encoding for MDA5) induction in COVID-19 and autoimmune-ILD, but not IPF, and IFIH1 strongly correlated with an IL-15-centric type-1 interferon response and an activated CD8+ T cell signature that is an immunologic hallmark of progressive ILD in the setting of systemic autoimmune rheumatic diseases. The IFIH1 rs1990760TT variant blunted such response. INTERPRETATION: A distinct pattern of MDA5-autoimmunity cases surged contemporaneously with circulation of the SARS-COV-2 virus during COVID-19. Bioinformatic insights suggest a shared immunopathology with known autoimmune lung disease mechanisms. FUNDING: This work was supported in part by the National Institute for Health Research (NIHR) Leeds Biomedical Research Centre (BRC), and in part by the National Institutes of Health (NIH) grant R01-AI155696 and pilot awards from the UC Office of the President (UCOP)-RGPO (R00RG2628, R00RG2642 and R01RG3780) to P.G. S.S was supported in part by R01-AI141630 (to P.G) and in part through funds from the American Association of Immunologists (AAI) Intersect Fellowship Program for Computational Scientists and Immunologists. CI - Copyright © 2024. Published by Elsevier B.V. FAU - David, Paula AU - David P AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom; University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom; Internal Medicine B, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel. FAU - Sinha, Saptarshi AU - Sinha S AD - Department of Cellular and Molecular Medicine, School of Medicine, University of California San Diego, La Jolla, CA, 92093, USA. FAU - Iqbal, Khizer AU - Iqbal K AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - De Marco, Gabriele AU - De Marco G AD - University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom; Mid Yorkshire Teaching NHS Trust, Rheumatology, Wakefield, United Kingdom; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. FAU - Taheri, Sahar AU - Taheri S AD - Department of Computer Science and Engineering, Jacob's School of Engineering, University of California San Diego, La Jolla, CA, 92093, USA. FAU - McLaren, Ella AU - McLaren E AD - Department of Cellular and Molecular Medicine, School of Medicine, University of California San Diego, La Jolla, CA, 92093, USA. FAU - Maisuria, Sheetal AU - Maisuria S AD - Leeds Teaching Hospitals NHS Trust, Pathology, Leeds, United Kingdom. FAU - Arumugakani, Gururaj AU - Arumugakani G AD - Leeds Teaching Hospitals NHS Trust, Pathology, Leeds, United Kingdom; University of Leeds, Immunology, Leeds, United Kingdom. FAU - Ash, Zoe AU - Ash Z AD - Bradford Teaching Hospitals NHS Foundation Trust, Rheumatology, Bradford, United Kingdom. FAU - Buckley, Catrin AU - Buckley C AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Coles, Lauren AU - Coles L AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Hettiarachchi, Chamila AU - Hettiarachchi C AD - Mid Yorkshire Teaching NHS Trust, Rheumatology, Wakefield, United Kingdom. FAU - Payne, Emma AU - Payne E AD - Leeds Teaching Hospitals NHS Trust, Pathology, Leeds, United Kingdom. FAU - Savic, Sinisa AU - Savic S AD - University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom; Department of Clinical Immunology and Allergy, Leeds Teaching Hospitals, NHS Trust, Leeds, UK. FAU - Smithson, Gayle AU - Smithson G AD - Mid Yorkshire Teaching NHS Trust, Rheumatology, Wakefield, United Kingdom. FAU - Slade, Maria AU - Slade M AD - Mid Yorkshire Teaching NHS Trust, Rheumatology, Wakefield, United Kingdom. FAU - Shah, Rahul AU - Shah R AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Marzo-Ortega, Helena AU - Marzo-Ortega H AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom; University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. FAU - Keen, Mansoor AU - Keen M AD - Bradford Teaching Hospitals NHS Foundation Trust, Rheumatology, Bradford, United Kingdom. FAU - Lawson, Catherine AU - Lawson C AD - Harrogate and District NHS Foundation Trust, Rheumatology, Harrogate, United Kingdom. FAU - Mclorinan, Joanna AU - Mclorinan J AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Nizam, Sharmin AU - Nizam S AD - Mid Yorkshire Teaching NHS Trust, Rheumatology, Wakefield, United Kingdom. FAU - Reddy, Hanu AU - Reddy H AD - Airedale NHS Foundation Trust, Rheumatology, Steeton with Eastburn, United Kingdom. FAU - Sharif, Omer AU - Sharif O AD - Calderdale and Huddersfield NHS Foundation Trust, Rheumatology, Huddersfield and Halifax, United Kingdom. FAU - Sultan, Shabina AU - Sultan S AD - Airedale NHS Foundation Trust, Rheumatology, Steeton with Eastburn, United Kingdom. FAU - Tran, Gui AU - Tran G AD - Harrogate and District NHS Foundation Trust, Rheumatology, Harrogate, United Kingdom. FAU - Wood, Mark AU - Wood M AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Wood, Samuel AU - Wood S AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom. FAU - Ghosh, Pradipta AU - Ghosh P AD - Department of Cellular and Molecular Medicine, School of Medicine, University of California San Diego, La Jolla, CA, 92093, USA; Department of Medicine, School of Medicine, and Veterans Affairs Medical Center, University of University of California San Diego, La Jolla, CA, 92093, USA. Electronic address: prghosh@ucsd.edu. FAU - McGonagle, Dennis AU - McGonagle D AD - Leeds Teaching Hospitals NHS Trust, Rheumatology Department, Leeds, United Kingdom; University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom. Electronic address: d.g.mcgonagle@leeds.ac.uk. LA - eng GR - R01 AI141630/AI/NIAID NIH HHS/United States GR - R01 AI155696/AI/NIAID NIH HHS/United States PT - Journal Article PT - Observational Study DEP - 20240508 PL - Netherlands TA - EBioMedicine JT - EBioMedicine JID - 101647039 RN - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1) RN - EC 3.6.1.- (IFIH1 protein, human) RN - 0 (Autoantibodies) SB - IM UOF - medRxiv. 2023 Nov 05:2023.11.03.23297727. doi: 10.1101/2023.11.03.23297727. PMID: 37961408 MH - Humans MH - *COVID-19/immunology MH - *Interferon-Induced Helicase, IFIH1/genetics/immunology MH - *Lung Diseases, Interstitial/immunology/genetics MH - *SARS-CoV-2/immunology MH - Male MH - Female MH - *Autoimmunity MH - Middle Aged MH - *Autoantibodies/immunology MH - Aged MH - Retrospective Studies MH - Pandemics MH - Dermatomyositis/immunology/genetics MH - Adult PMC - PMC11090026 OTO - NOTNLM OT - Autoimmune Raynauds OT - Autoimmune rashes OT - Coronavirus-19 (Covid-19) OT - Interstitial lung disease (ILD) OT - MDA5-autoimmunity and interstitial pneumonitis contemporaneous with the COVID-19 (MIP-C) OT - Melanoma differentiation-associated protein-5 (MDA5) COIS- Declaration of interests The authors declare that they have no financial conflict of interests for this study. EDAT- 2024/05/10 00:43 MHDA- 2024/06/17 00:42 PMCR- 2024/05/08 CRDT- 2024/05/09 18:12 PHST- 2023/11/03 00:00 [received] PHST- 2024/04/15 00:00 [revised] PHST- 2024/04/16 00:00 [accepted] PHST- 2024/06/17 00:42 [medline] PHST- 2024/05/10 00:43 [pubmed] PHST- 2024/05/09 18:12 [entrez] PHST- 2024/05/08 00:00 [pmc-release] AID - S2352-3964(24)00171-3 [pii] AID - 105136 [pii] AID - 10.1016/j.ebiom.2024.105136 [doi] PST - ppublish SO - EBioMedicine. 2024 Jun;104:105136. doi: 10.1016/j.ebiom.2024.105136. Epub 2024 May 8. PMID- 17296514 OWN - NLM STAT- MEDLINE DCOM- 20070403 LR - 20220321 IS - 1262-3636 (Print) IS - 1262-3636 (Linking) VI - 32 IP - 6 DP - 2006 Dec TI - Substrate oxidation during exercise: type 2 diabetes is associated with a decrease in lipid oxidation and an earlier shift towards carbohydrate utilization. PG - 604-10 AB - OBJECTIVES: Exercise is a recommended treatment for type 2 diabetes but the actual pattern of metabolic adaptation to exercise in this disease is poorly known and not taken in account in the protocols used. Metabolic defects involved in the pathways of substrate oxidation were described in type 2 diabetes. We hypothesized that type 2 diabetes, regardless of age, gender, training status and weight, could influence by its own the balance of substrates at exercise. METHODS: 30 sedentary type 2 diabetic subjects and 38 sedentary matched control subjects were recruited. We used exercise calorimetry to determine lipid and carbohydrate oxidation rates. We calculated two parameters quantifying the balance of substrates induced by increasing exercise intensity: the maximal lipid oxidation point (PLipoxMax) and the Crossover point (COP), intensity from which the part of carbohydrate utilization providing energy becomes predominant on lipid oxidation. RESULTS: Lipid oxidation was lower in the diabetic group, independent of exercise intensity. PLipoxMax and COP were lower in the diabetic group [PLipoxMax=25.3+/-1.4% vs. 36.6+/-1.7% %Wmax (P<0.0001)] - COP =24.2+/-2.2% vs. 38.8+/-1.9% %Wmax (P<0.0001). CONCLUSIONS: Type 2 diabetes is associated with a decrease in lipid oxidation at exercise and a shift towards a predominance of carbohydrate oxidation for exercise intensities lower than in control subjects. Taking into account these alterations could provide a basis for personalizing training intensity. FAU - Ghanassia, E AU - Ghanassia E AD - CHU de Montpellier, Service central de Physiologie Clinique, Unité d'Exploration Métabolique (CERAMM), Hôpital Lapeyronie, 34000 Montpellier, France. eghanassia@aol.com FAU - Brun, J F AU - Brun JF FAU - Fedou, C AU - Fedou C FAU - Raynaud, E AU - Raynaud E FAU - Mercier, J AU - Mercier J LA - eng PT - Journal Article PL - France TA - Diabetes Metab JT - Diabetes & metabolism JID - 9607599 RN - 0 (Dietary Carbohydrates) RN - 0 (Lipids) SB - IM MH - Adult MH - Aged MH - Diabetes Mellitus, Type 2/*blood/*physiopathology MH - *Dietary Carbohydrates MH - *Exercise MH - Exercise Test MH - Humans MH - Lipids/*blood MH - Middle Aged MH - Overweight MH - Oxidation-Reduction MH - Reference Values EDAT- 2007/02/14 09:00 MHDA- 2007/04/04 09:00 CRDT- 2007/02/14 09:00 PHST- 2006/03/28 00:00 [received] PHST- 2006/06/12 00:00 [accepted] PHST- 2007/02/14 09:00 [pubmed] PHST- 2007/04/04 09:00 [medline] PHST- 2007/02/14 09:00 [entrez] AID - S1262-3636(07)70315-4 [pii] AID - 10.1016/S1262-3636(07)70315-4 [doi] PST - ppublish SO - Diabetes Metab. 2006 Dec;32(6):604-10. doi: 10.1016/S1262-3636(07)70315-4. PMID- 32776767 OWN - NLM STAT- MEDLINE DCOM- 20210519 LR - 20210519 IS - 1536-0210 (Electronic) IS - 0020-9996 (Linking) VI - 55 IP - 9 DP - 2020 Sep TI - Current and Future MR Contrast Agents: Seeking a Better Chemical Stability and Relaxivity for Optimal Safety and Efficacy. PG - 578-588 LID - 10.1097/RLI.0000000000000684 [doi] AB - This review summarizes 30 years of experience in the development and clinical use of magnetic resonance (MR) contrast agents. Despite their undisputable usefulness for disease diagnosis, gadolinium (Gd)-based contrast agents (GBCAs) have gone through 2 major safety crises. Approximately 10 years ago, the regulatory agencies decided to restrict the use of GBCAs to minimize the risk of nephrogenic systemic fibrosis in patients with severe renal insufficiency. Yet, following the recent discovery of Gd retention in brain, the same agencies adopted different positions ranging from suspension of marketing authorizations, changes in GBCA safety labeling, and performing preclinical and clinical studies to assess the potential long-term consequences of Gd accumulation on motor and cognitive functions. Besides, magnetic resonance imaging (MRI) has benefited from MR technological advances, which provide alternative solutions to increase the MR signal, generate new contrasts on MRI scans, and accelerate their acquisition and analysis. Altogether, GBCAs in combination with new MR techniques have found their place in the diagnostic pathway of various diseases. Despite the large research efforts to identify and develop alternative Gd-free MR agents, manganese- and iron-based contrast agents have failed to reach market approval. In this context, the development of next-generation MR contrast agents should focus on high-stability and high-relaxivity GBCAs, such as gadopiclenol, which offer the possibility to adapt the administered Gd dose to each indication while ensuring an optimal patient safety. FAU - Lancelot, Eric AU - Lancelot E AD - From Guerbet, Roissy CDG Cedex, France. FAU - Raynaud, Jean-Sébastien AU - Raynaud JS FAU - Desché, Pierre AU - Desché P LA - eng PT - Journal Article PT - Review PL - United States TA - Invest Radiol JT - Investigative radiology JID - 0045377 RN - 0 (Contrast Media) SB - IM CIN - Invest Radiol. 2020 Sep;55(9):589-591. doi: 10.1097/RLI.0000000000000710. PMID: 32732647 MH - Brain/diagnostic imaging MH - Contrast Media/adverse effects/*chemistry MH - Humans MH - Magnetic Resonance Imaging/adverse effects/*methods MH - *Safety EDAT- 2020/08/11 06:00 MHDA- 2021/05/20 06:00 CRDT- 2020/08/11 06:00 PHST- 2020/08/11 06:00 [entrez] PHST- 2020/08/11 06:00 [pubmed] PHST- 2021/05/20 06:00 [medline] AID - 00004424-202009000-00007 [pii] AID - 10.1097/RLI.0000000000000684 [doi] PST - ppublish SO - Invest Radiol. 2020 Sep;55(9):578-588. doi: 10.1097/RLI.0000000000000684. PMID- 17893248 OWN - NLM STAT- MEDLINE DCOM- 20081114 LR - 20260518 IS - 1468-2060 (Electronic) IS - 0003-4967 (Print) IS - 0003-4967 (Linking) VI - 67 IP - 5 DP - 2008 May TI - Development of a provisional core set of response measures for clinical trials of systemic sclerosis. PG - 703-9 LID - 10.1136/ard.2007.078923 AB - OBJECTIVE: To develop a provisional core set of response measures for clinical trials of systemic sclerosis (SSc). METHODS: The Scleroderma Clinical Trials Consortium (SCTC) conducted a structured, 3-round Delphi exercise to reach consensus on a core set of measures for clinical trials of SSc. Round 1 asked the SCTC investigators to list items in 11 pre-defined domains (skin, musculoskeletal, cardiac, pulmonary, cardio-pulmonary, gastrointestinal, renal, Raynaud phenomenon and digital ulcers, health-related quality of life and function, global health, and biomarkers) for SSc clinical trials. Round 2 asked respondents to rate the importance of the chosen items and was followed by a meeting, during which the Steering Committee discussed the feasibility, reliability, redundancy and validity of the items. Round 3 sought to obtain broader consensus on the core set measures. Members also voted on items that had data on feasibility but lacked data on reliability and validity, but may still be useful research outcome measures for future trials. RESULTS: A total of 50 SCTC investigators participated in round 1, providing 212 unique items for the 11 domains. In all, 46 (92%) participants responded in round 2 and rated 177 items. The ratings of 177 items were reviewed by the Steering Committee and 31 items from the 11 domains were judged to be appropriate for inclusion in a 1-year multi-centre clinical trial. In total, 40 SCTC investigators completed round 3 and ranked 30 of 31 items as acceptable for inclusion in the core set. The Steering Committee also proposed 14 items for a research agenda. CONCLUSION: Using a Delphi exercise, we have developed a provisional core set of measures for assessment of disease activity and severity in clinical trials of SSc. FAU - Khanna, D AU - Khanna D AD - Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California at Los Angeles, California 90095, USA. dkhanna@mednet.ucla.edu FAU - Lovell, D J AU - Lovell DJ FAU - Giannini, E AU - Giannini E FAU - Clements, P J AU - Clements PJ FAU - Merkel, P A AU - Merkel PA FAU - Seibold, J R AU - Seibold JR FAU - Matucci-Cerinic, M AU - Matucci-Cerinic M FAU - Denton, C P AU - Denton CP FAU - Mayes, M D AU - Mayes MD FAU - Steen, V D AU - Steen VD FAU - Varga, J AU - Varga J FAU - Furst, D E AU - Furst DE CN - Scleroderma Clinical Trials Consortium co-authors LA - eng GR - U01 AR055057-01/AR/NIAMS NIH HHS/United States GR - P50 AR054144/AR/NIAMS NIH HHS/United States GR - K24 AR002224/AR/NIAMS NIH HHS/United States GR - K23 AR053858/AR/NIAMS NIH HHS/United States GR - U01 AR055057/AR/NIAMS NIH HHS/United States GR - K23 AR053858-01A1/AR/NIAMS NIH HHS/United States GR - K24 AR2224-01A1/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20070924 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 SB - IM MH - *Clinical Trials as Topic MH - *Consensus MH - *Delphi Technique MH - Endpoint Determination MH - Humans MH - Multicenter Studies as Topic MH - Scleroderma, Systemic/*therapy MH - Treatment Outcome PMC - PMC3887552 MID - NIHMS541710 FIR - Khanna, Dinesh IR - Khanna D FIR - Furst, Daniel E IR - Furst DE FIR - Clements, Philip J IR - Clements PJ FIR - Denton, Christopher P IR - Denton CP FIR - Giannini, Edward IR - Giannini E FIR - Lovell, Daniel E IR - Lovell DE FIR - Mayes, Maureen D IR - Mayes MD FIR - Matucci-Cerinic, Marco IR - Matucci-Cerinic M FIR - Merkel, Peter A IR - Merkel PA FIR - Seibold, James R IR - Seibold JR FIR - Steen, Virginia D IR - Steen VD FIR - Baron, Murray IR - Baron M FIR - Csuka, Mary Ellen IR - Csuka ME FIR - Berezne, Alice IR - Berezne A FIR - Briet, Samuel N IR - Briet SN FIR - Brühlmann, Pius IR - Brühlmann P FIR - Buch, Maya H IR - Buch MH FIR - Catoggio, Luis IR - Catoggio L FIR - Collier, David IR - Collier D FIR - Crofford, Leslie IR - Crofford L FIR - Czirják, László IR - Czirják L FIR - Derk, Chris T IR - Derk CT FIR - Distler, Oliver IR - Distler O FIR - Doyle, Mittie Kelleher IR - Doyle MK FIR - Farge-Bancel, Domonique IR - Farge-Bancel D FIR - Fessler, Barri IR - Fessler B FIR - Foeldvari, Ivan IR - Foeldvari I FIR - Goldberg, Avram IR - Goldberg A FIR - Gran, Jan Tore IR - Gran JT FIR - Grau, Raffael IR - Grau R FIR - Griffing, W Leroy IR - Griffing WL FIR - Hayat, Samina IR - Hayat S FIR - Herrick, Ariane L IR - Herrick AL FIR - Hsu, Vivien IR - Hsu V FIR - Hummers, Laura K IR - Hummers LK FIR - Inanç, Murat IR - Inanç M FIR - Johnson, Sindhu IR - Johnson S FIR - Kahaleh, M Bashar IR - Kahaleh MB FIR - Lafyatis, Robert A IR - Lafyatis RA FIR - Lee, Peter IR - Lee P FIR - Mahmud, Tafazzul H IR - Mahmud TH FIR - Malcarne, Vanessa IR - Malcarne V FIR - McHugh, Neil J IR - McHugh NJ FIR - Martin, Richard W IR - Martin RW FIR - McKown, Kevin IR - McKown K FIR - Medsger, Thomas A Jr IR - Medsger TA Jr FIR - Moreland, Larry IR - Moreland L FIR - Pope, Janet E IR - Pope JE FIR - Rich, Eric IR - Rich E FIR - Rothfield, Naomi F IR - Rothfield NF FIR - Schiopu, Elena IR - Schiopu E FIR - Scorza, Raffaella IR - Scorza R FIR - Senécal, Jean-Luc IR - Senécal JL FIR - Shanahan, Joseph IR - Shanahan J FIR - Simms, Robert W IR - Simms RW FIR - Strand, Vibeke IR - Strand V FIR - Silver, Richard M IR - Silver RM FIR - Sweiss, Nadera IR - Sweiss N FIR - Valentini, Gabriele IR - Valentini G FIR - van den Hoogen, Frank H J IR - van den Hoogen FH FIR - Veale, Douglas IR - Veale D FIR - Voskuyl, Alexander E IR - Voskuyl AE FIR - Wigley, Fred IR - Wigley F FIR - Wollheim, Frank A IR - Wollheim FA EDAT- 2007/09/26 09:00 MHDA- 2008/11/15 09:00 PMCR- 2014/01/10 CRDT- 2007/09/26 09:00 PHST- 2007/09/26 09:00 [pubmed] PHST- 2008/11/15 09:00 [medline] PHST- 2007/09/26 09:00 [entrez] PHST- 2014/01/10 00:00 [pmc-release] AID - S0003-4967(24)21814-8 [pii] AID - 10.1136/ard.2007.078923 [doi] PST - ppublish SO - Ann Rheum Dis. 2008 May;67(5):703-9. doi: 10.1136/ard.2007.078923. Epub 2007 Sep 24. PMID- 23307483 OWN - NLM STAT- MEDLINE DCOM- 20131113 LR - 20260128 IS - 1573-3297 (Electronic) IS - 0001-8244 (Linking) VI - 43 IP - 2 DP - 2013 Mar TI - Xq27 FRAXA locus is a strong candidate for dyslexia: evidence from a genome-wide scan in French families. PG - 132-40 LID - 10.1007/s10519-012-9575-5 [doi] AB - Dyslexia is a frequent neurodevelopmental learning disorder. To date, nine susceptibility loci have been identified, one of them being DYX9, located in Xq27. We performed the first French SNP linkage study followed by candidate gene investigation in dyslexia by studying 12 multiplex families (58 subjects) with at least two children affected, according to categorical restrictive criteria for phenotype definition. Significant results emerged on Xq27.3 within DYX9. The maximum multipoint LOD score reached 3,884 between rs12558359 and rs454992. Within this region, seven candidate genes were investigated for mutations in exonic sequences (CXORF1, CXORF51, SLITRK2, FMR1, FMR2, ASFMR1, FMR1NB), all having a role during brain development. We further looked for 5'UTR trinucleotide repeats in FMR1 and FMR2 genes. No mutation or polymorphism co-segregating with dyslexia was found. This finding in French families with Dyslexia showed significant linkage on Xq27.3 enclosing FRAXA, and consequently confirmed the DYX9 region as a robust susceptibility locus. We reduced the previously described interval from 6.8 (DXS1227-DXS8091) to 4 Mb also disclosing a higher LOD score. FAU - Huc-Chabrolle, M AU - Huc-Chabrolle M AD - UMR INSERM U930, François Rabelais University, Tours, France. m.huc-chabrolle@chu-tours.fr FAU - Charon, C AU - Charon C FAU - Guilmatre, A AU - Guilmatre A FAU - Vourc'h, P AU - Vourc'h P FAU - Tripi, G AU - Tripi G FAU - Barthez, M A AU - Barthez MA FAU - Sizaret, E AU - Sizaret E FAU - Thepault, R A AU - Thepault RA FAU - Le Gallic, S AU - Le Gallic S FAU - Hager, J AU - Hager J FAU - Toutain, A AU - Toutain A FAU - Raynaud, M AU - Raynaud M FAU - Andres, C AU - Andres C FAU - Campion, D AU - Campion D FAU - Laumonnier, F AU - Laumonnier F FAU - Bonnet-Brilhault, F AU - Bonnet-Brilhault F LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130111 PL - United States TA - Behav Genet JT - Behavior genetics JID - 0251711 RN - 139135-51-6 (Fragile X Messenger Ribonucleoprotein 1) RN - 0 (FMR1 protein, human) SB - IM MH - Child MH - Chromosomes, Human, X/*genetics MH - Dyslexia/*genetics MH - Female MH - Fragile X Messenger Ribonucleoprotein 1/*genetics MH - France MH - Genes, X-Linked MH - Genetic Loci MH - Genetic Predisposition to Disease/*genetics MH - Genome-Wide Association Study MH - Genotype MH - Humans MH - Lod Score MH - Male MH - Pedigree MH - Polymorphism, Single Nucleotide EDAT- 2013/01/12 06:00 MHDA- 2013/11/14 06:00 CRDT- 2013/01/12 06:00 PHST- 2012/08/22 00:00 [received] PHST- 2012/12/08 00:00 [accepted] PHST- 2013/01/12 06:00 [entrez] PHST- 2013/01/12 06:00 [pubmed] PHST- 2013/11/14 06:00 [medline] AID - 10.1007/s10519-012-9575-5 [doi] PST - ppublish SO - Behav Genet. 2013 Mar;43(2):132-40. doi: 10.1007/s10519-012-9575-5. Epub 2013 Jan 11. PMID- 20181063 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20110714 LR - 20211020 IS - 1755-8417 (Electronic) IS - 1755-8417 (Linking) VI - 3 IP - 1 DP - 2010 Feb 2 TI - A distinctive gene expression fingerprint in mentally retarded male patients reflects disease-causing defects in the histone demethylase KDM5C. PG - 2 LID - 10.1186/1755-8417-3-2 [doi] AB - BACKGROUND: Mental retardation is a genetically heterogeneous disorder, as more than 90 genes for this disorder has been found on the X chromosome alone. In addition the majority of patients are non-syndromic in that they do not present with clinically recognisable features. This makes it difficult to determine the molecular cause of this disorder on the basis of the phenotype alone. Mutations in KDM5C (previously named SMCX or JARID1C), a gene that encodes a transcriptional regulator with histone demethylase activity specific for dimethylated and trimethylated H3K4, are a comparatively frequent cause of non-syndromic X-linked mental retardation (NS-XLMR). Specific transcriptional targets of KDM5C, however, are still unknown and the effects of KDM5C deficiency on gene expression have not yet been investigated. RESULTS: By whole-mount in situ hybridisation we showed that the mouse homologue of KDM5C is expressed in multiple tissues during mouse development.We present the results of gene expression profiling performed on lymphoblastoid cell lines as well as blood from patients with mutations in KDM5C. Using whole genome expression arrays and quantitative reverse transcriptase polymerase chain reaction (QRT-PCR) experiments, we identified several genes, including CMKOR1, KDM5B and KIAA0469 that were consistently deregulated in both tissues. CONCLUSIONS: Our findings shed light on the pathological mechanisms underlying mental retardation and have implications for future diagnostics of this heterogeneous disorder. FAU - Jensen, Lars R AU - Jensen LR AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. kuss_a@molgen.mpg.de. FAU - Bartenschlager, Heinz AU - Bartenschlager H FAU - Rujirabanjerd, Sinitdhorn AU - Rujirabanjerd S FAU - Tzschach, Andreas AU - Tzschach A FAU - Nümann, Astrid AU - Nümann A FAU - Janecke, Andreas R AU - Janecke AR FAU - Spörle, Ralf AU - Spörle R FAU - Stricker, Sigmar AU - Stricker S FAU - Raynaud, Martine AU - Raynaud M FAU - Nelson, John AU - Nelson J FAU - Hackett, Anna AU - Hackett A FAU - Fryns, Jean-Pierre AU - Fryns JP FAU - Chelly, Jamel AU - Chelly J FAU - de Brouwer, Arjan Pm AU - de Brouwer AP FAU - Hamel, Ben AU - Hamel B FAU - Gecz, Jozef AU - Gecz J FAU - Ropers, Hans-Hilger AU - Ropers HH FAU - Kuss, Andreas W AU - Kuss AW LA - eng PT - Journal Article DEP - 20100202 PL - England TA - Pathogenetics JT - PathoGenetics JID - 101469516 PMC - PMC2830949 EDAT- 2010/02/26 06:00 MHDA- 2010/02/26 06:01 PMCR- 2010/02/02 CRDT- 2010/02/26 06:00 PHST- 2009/11/13 00:00 [received] PHST- 2010/02/02 00:00 [accepted] PHST- 2010/02/26 06:00 [entrez] PHST- 2010/02/26 06:00 [pubmed] PHST- 2010/02/26 06:01 [medline] PHST- 2010/02/02 00:00 [pmc-release] AID - 1755-8417-3-2 [pii] AID - 10.1186/1755-8417-3-2 [doi] PST - epublish SO - Pathogenetics. 2010 Feb 2;3(1):2. doi: 10.1186/1755-8417-3-2. PMID- 16783569 OWN - NLM STAT- MEDLINE DCOM- 20070410 LR - 20250103 IS - 0340-6717 (Print) IS - 0340-6717 (Linking) VI - 120 IP - 2 DP - 2006 Sep TI - A novel X-linked recessive mental retardation syndrome comprising macrocephaly and ciliary dysfunction is allelic to oral-facial-digital type I syndrome. PG - 171-8 AB - We report on a large family in which a novel X-linked recessive mental retardation (XLMR) syndrome comprising macrocephaly and ciliary dysfunction co-segregates with a frameshift mutation in the OFD1 gene. Mutations of OFD1 have been associated with oral-facial-digital type 1 syndrome (OFD1S) that is characterized by X-chromosomal dominant inheritance and lethality in males. In contrast, the carrier females of our family were clinically inconspicuous, and the affected males suffered from severe mental retardation, recurrent respiratory tract infections and macrocephaly. All but one of the affected males died from respiratory problems in infancy; and impaired ciliary motility was confirmed in the index patient by high-speed video microscopy examination of nasal epithelium. This family broadens the phenotypic spectrum of OFD1 mutations in an unexpected way and sheds light on the complexity of the underlying disease mechanisms. FAU - Budny, Bartlomiej AU - Budny B AD - Max Planck Institute for Molecular Genetics, Ihnestr. 73, 14195, Berlin, Germany. FAU - Chen, Wei AU - Chen W FAU - Omran, Heymut AU - Omran H FAU - Fliegauf, Manfred AU - Fliegauf M FAU - Tzschach, Andreas AU - Tzschach A FAU - Wisniewska, Marzena AU - Wisniewska M FAU - Jensen, Lars R AU - Jensen LR FAU - Raynaud, Martine AU - Raynaud M FAU - Shoichet, Sarah A AU - Shoichet SA FAU - Badura, Magda AU - Badura M FAU - Lenzner, Steffen AU - Lenzner S FAU - Latos-Bielenska, Anna AU - Latos-Bielenska A FAU - Ropers, Hans-Hilger AU - Ropers HH LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20060617 PL - Germany TA - Hum Genet JT - Human genetics JID - 7613873 RN - 0 (DNA, Complementary) RN - 0 (OFD1 protein, human) RN - 0 (Proteins) SB - IM MH - Alleles MH - Blotting, Northern MH - Child MH - Chromosome Mapping MH - Ciliary Motility Disorders/*genetics/pathology MH - Craniofacial Abnormalities/*genetics/pathology MH - DNA, Complementary MH - Female MH - Frameshift Mutation MH - Genes, Recessive MH - Heterozygote MH - Humans MH - Male MH - X-Linked Intellectual Disability/*genetics/pathology MH - Pedigree MH - Proteins/*genetics MH - Syndrome EDAT- 2006/06/20 09:00 MHDA- 2007/04/11 09:00 CRDT- 2006/06/20 09:00 PHST- 2006/05/15 00:00 [received] PHST- 2006/05/15 00:00 [accepted] PHST- 2006/06/20 09:00 [pubmed] PHST- 2007/04/11 09:00 [medline] PHST- 2006/06/20 09:00 [entrez] AID - 10.1007/s00439-006-0210-5 [doi] PST - ppublish SO - Hum Genet. 2006 Sep;120(2):171-8. doi: 10.1007/s00439-006-0210-5. Epub 2006 Jun 17. PMID- 33397292 OWN - NLM STAT- MEDLINE DCOM- 20210125 LR - 20210125 IS - 1471-2288 (Electronic) IS - 1471-2288 (Linking) VI - 21 IP - 1 DP - 2021 Jan 4 TI - COVID-19-related medical research: a meta-research and critical appraisal. PG - 1 LID - 10.1186/s12874-020-01190-w [doi] LID - 1 AB - BACKGROUND: Since the start of the COVID-19 outbreak, a large number of COVID-19-related papers have been published. However, concerns about the risk of expedited science have been raised. We aimed at reviewing and categorizing COVID-19-related medical research and to critically appraise peer-reviewed original articles. METHODS: The data sources were Pubmed, Cochrane COVID-19 register study, arXiv, medRxiv and bioRxiv, from 01/11/2019 to 01/05/2020. Peer-reviewed and preprints publications related to COVID-19 were included, written in English or Chinese. No limitations were placed on study design. Reviewers screened and categorized studies according to i) publication type, ii) country of publication, and iii) topics covered. Original articles were critically appraised using validated quality assessment tools. RESULTS: Among the 11,452 publications identified, 10,516 met the inclusion criteria, among which 7468 (71.0%) were peer-reviewed articles. Among these, 4190 publications (56.1%) did not include any data or analytics (comprising expert opinion pieces). Overall, the most represented topics were infectious disease (n = 2326, 22.1%), epidemiology (n = 1802, 17.1%), and global health (n = 1602, 15.2%). The top five publishing countries were China (25.8%), United States (22.3%), United Kingdom (8.8%), Italy (8.1%) and India (3.4%). The dynamic of publication showed that the exponential growth of COVID-19 peer-reviewed articles was mainly driven by publications without original data (mean 261.5 articles ± 51.1 per week) as compared with original articles (mean of 69.3 ± 22.3 articles per week). Original articles including patient data accounted for 713 (9.5%) of peer-reviewed studies. A total of 576 original articles (80.8%) showed intermediate to high risk of bias. Last, except for simulation studies that mainly used large-scale open data, the median number of patients enrolled was of 102 (IQR = 37-337). CONCLUSIONS: Since the beginning of the COVID-19 pandemic, the majority of research is composed by publications without original data. Peer-reviewed original articles with data showed a high risk of bias and included a limited number of patients. Together, these findings underscore the urgent need to strike a balance between the velocity and quality of research, and to cautiously consider medical information and clinical applicability in a pressing, pandemic context. SYSTEMATIC REVIEW REGISTRATION: https://osf.io/5zjyx/. FAU - Raynaud, Marc AU - Raynaud M AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Zhang, Huanxi AU - Zhang H AD - The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China. FAU - Louis, Kevin AU - Louis K AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Goutaudier, Valentin AU - Goutaudier V AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. AD - Montpellier University Hospital, Montpellier, France. FAU - Wang, Jiali AU - Wang J AD - The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China. FAU - Dubourg, Quentin AU - Dubourg Q AD - Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Wei, Yongcheng AU - Wei Y AD - The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China. FAU - Demir, Zeynep AU - Demir Z AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. AD - Paediatrics Unit, Necker University Hospital, Paris, France. FAU - Debiais, Charlotte AU - Debiais C AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Aubert, Olivier AU - Aubert O AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Bouatou, Yassine AU - Bouatou Y AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Immunology and Nephrology Department, Saint Louis Hospital, Paris, France. FAU - Jabre, Patricia AU - Jabre P AD - Cochrane Pre-hospital and Emergency Care, Necker University Hospital, Paris, France. FAU - Liu, Longshan AU - Liu L AD - The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China. FAU - Wang, Changxi AU - Wang C AD - The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China. FAU - Jouven, Xavier AU - Jouven X AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Reese, Peter AU - Reese P AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. AD - University of Pennsylvania School of Medicine, Philadelphia, PA, USA. FAU - Empana, Jean-Philippe AU - Empana JP AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AD - Paris Translational Research Epidemiology and Biostatistics Department, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France. alexandre.loupy@inserm.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20210104 PL - England TA - BMC Med Res Methodol JT - BMC medical research methodology JID - 100968545 SB - IM MH - Biomedical Research/*statistics & numerical data MH - COVID-19/*epidemiology/*prevention & control/virology MH - China/epidemiology MH - Humans MH - India/epidemiology MH - Italy/epidemiology MH - *Pandemics MH - SARS-CoV-2/*isolation & purification/physiology MH - United Kingdom/epidemiology MH - United States/epidemiology PMC - PMC7780085 OTO - NOTNLM OT - COVID-19 OT - Critical appraisal OT - Quality of research OT - Systematic review COIS- We report no relationships or activities that could appear to have influenced the present work. EDAT- 2021/01/06 06:00 MHDA- 2021/01/26 06:00 PMCR- 2021/01/04 CRDT- 2021/01/05 05:38 PHST- 2020/09/29 00:00 [received] PHST- 2020/12/08 00:00 [accepted] PHST- 2021/01/05 05:38 [entrez] PHST- 2021/01/06 06:00 [pubmed] PHST- 2021/01/26 06:00 [medline] PHST- 2021/01/04 00:00 [pmc-release] AID - 10.1186/s12874-020-01190-w [pii] AID - 1190 [pii] AID - 10.1186/s12874-020-01190-w [doi] PST - epublish SO - BMC Med Res Methodol. 2021 Jan 4;21(1):1. doi: 10.1186/s12874-020-01190-w. PMID- 22813950 OWN - NLM STAT- MEDLINE DCOM- 20130418 LR - 20220408 IS - 1095-9572 (Electronic) IS - 1053-8119 (Linking) VI - 63 IP - 2 DP - 2012 Nov 1 TI - Ultra-sensitive molecular MRI of cerebrovascular cell activation enables early detection of chronic central nervous system disorders. PG - 760-70 LID - S1053-8119(12)00728-8 [pii] LID - 10.1016/j.neuroimage.2012.07.018 [doi] AB - Since endothelial cells can be targeted by large contrast-carrying particles, molecular imaging of cerebrovascular cell activation is highly promising to evaluate the underlying inflammation of the central nervous system (CNS). In this study, we aimed to demonstrate that molecular magnetic resonance imaging (MRI) of cerebrovascular cell activation can reveal CNS disorders in the absence of visible lesions and symptoms. To this aim, we optimized contrast carrying particles targeting vascular cell adhesion molecule-1 and MRI protocols through both in vitro and in vivo experiments. Although, pre-contrast MRI images failed to reveal the ongoing pathology, contrast-enhanced MRI revealed hypoperfusion-triggered CNS injury in vascular dementia, unmasked amyloid-induced cerebrovascular activation in Alzheimer's disease and allowed monitoring of disease activity during experimental autoimmune encephalomyelitis. Moreover, contrast-enhanced MRI revealed the cerebrovascular cell activation associated with known risk factors of CNS disorders such as peripheral inflammation, ethanol consumption, hyperglycemia and aging. By providing a dramatically higher sensitivity than previously reported methods and molecular contrast agents, the technology described in the present study opens new avenues of investigation in the field of neuroinflammation. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Montagne, Axel AU - Montagne A AD - INSERM, INSERM U919 Serine Protease and Pathophysiology of the Neurovascular Unit, University Caen Basse-Normandie, GIP Cyceron, Bd Becquerel, BP5229, 14074 Caen, France. FAU - Gauberti, Maxime AU - Gauberti M FAU - Macrez, Richard AU - Macrez R FAU - Jullienne, Amandine AU - Jullienne A FAU - Briens, Aurélien AU - Briens A FAU - Raynaud, Jean-Sébastien AU - Raynaud JS FAU - Louin, Gaelle AU - Louin G FAU - Buisson, Alain AU - Buisson A FAU - Haelewyn, Benoit AU - Haelewyn B FAU - Docagne, Fabian AU - Docagne F FAU - Defer, Gilles AU - Defer G FAU - Vivien, Denis AU - Vivien D FAU - Maubert, Eric AU - Maubert E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120717 PL - United States TA - Neuroimage JT - NeuroImage JID - 9215515 RN - 0 (Ferric Compounds) RN - 1K09F3G675 (ferric oxide) SB - IM MH - Animals MH - Blotting, Western MH - Central Nervous System Diseases/*diagnosis MH - Endothelial Cells/*metabolism MH - *Ferric Compounds MH - Immunohistochemistry MH - Magnetic Resonance Imaging/*methods MH - Male MH - Metal Nanoparticles MH - Mice MH - Mice, Inbred C57BL MH - Molecular Imaging/*methods MH - Real-Time Polymerase Chain Reaction MH - Reverse Transcriptase Polymerase Chain Reaction EDAT- 2012/07/21 06:00 MHDA- 2013/04/20 06:00 CRDT- 2012/07/21 06:00 PHST- 2012/04/27 00:00 [received] PHST- 2012/07/04 00:00 [revised] PHST- 2012/07/10 00:00 [accepted] PHST- 2012/07/21 06:00 [entrez] PHST- 2012/07/21 06:00 [pubmed] PHST- 2013/04/20 06:00 [medline] AID - S1053-8119(12)00728-8 [pii] AID - 10.1016/j.neuroimage.2012.07.018 [doi] PST - ppublish SO - Neuroimage. 2012 Nov 1;63(2):760-70. doi: 10.1016/j.neuroimage.2012.07.018. Epub 2012 Jul 17. PMID- 40261602 OWN - NLM STAT- MEDLINE DCOM- 20250930 LR - 20251003 IS - 1724-6059 (Electronic) IS - 1121-8428 (Print) IS - 1121-8428 (Linking) VI - 38 IP - 7 DP - 2025 Sep TI - Learning from the implementation phase of the new French capitation payment model for chronic kidney disease care: a qualitative study. PG - 1877-1887 LID - 10.1007/s40620-025-02284-8 [doi] AB - BACKGROUND: France introduced a new payment model for care providers of patients with Chronic Kidney Disease (CKD) Grades 4 and 5: the CKD-Capitation Payment model. The model aims to financially incentivise multidisciplinary care for patients. We performed a qualitative study among participating providers to identify obstacles and facilitators of the model implementation as well as the initial benefits and potential policy improvements. METHODS: From March to July 2023, we collected data through semi-structured interviews with medical and managerial staff of facilities participating in the new model in France. We purposely selected a sample of facilities based on ownership status and CKD-Capitation Payment model activity data, including the number of patients reported. We performed a thematic analysis of the interview transcripts. RESULTS: We interviewed 22 staff from 14 facilities. Interviews revealed that adapting the information systems to the model requirements was a major obstacle to implementation, undermining efficient medical time allocation and data quality. Securing facility management support and organising the care amid workforce shortages were additional obstacles. Despite these challenges, staff reported positively on the model, noting the increased time spent by nurses with patients and the assertion of dietitians' role. Interviewees reported the need for greater flexibility in visit requirements to better align with patient needs. CONCLUSIONS: This study demonstrates how the new capitation payment model introduced in France can enable multidisciplinary and coordinated care for patients with CKD. However, supporting facilities in adopting interoperable information systems and increasing the flexibility of the model appear essential for long-term adoption. CI - © 2025. The Author(s). FAU - Raffray, Maxime AU - Raffray M AD - CNRS, Inserm, Arènes-UMR 6051, RSMS-U1309, École des hautes études en sante publique, Rennes, France. maxime.raffray@ki.se. AD - Clinical Epidemiology Division, Department of Medicine Solna, Karolinska Institutet, Maria Aspmans Gata 22A, 171 64, Solna, Sweden. maxime.raffray@ki.se. FAU - Campéon, Arnaud AU - Campéon A AD - Arènes, CNRS, UMR 6051, École des hautes études en sante publique, Rennes, France. FAU - Bricard, Damien AU - Bricard D AD - Institut de Recherche et Documentation en Économie de la Santé, Paris, France. FAU - Augé, Estelle AU - Augé E AD - Institut de Recherche et Documentation en Économie de la Santé, Paris, France. FAU - Raynaud, Denis AU - Raynaud D AD - Institut de Recherche et Documentation en Économie de la Santé, Paris, France. FAU - Couchoud, Cécile AU - Couchoud C AD - Agence de la Biomédecine, Registre REIN, Saint-Denis, France. FAU - Frimat, Luc AU - Frimat L AD - Néphrologie, CHU de Nancy-Hôpitaux de Brabois, Vandœuvre-lès-Nancy, France. FAU - Bayat, Sahar AU - Bayat S AD - CNRS, Inserm, Arènes-UMR 6051, RSMS-U1309, École des hautes études en sante publique, Rennes, France. LA - eng PT - Journal Article DEP - 20250422 PL - Italy TA - J Nephrol JT - Journal of nephrology JID - 9012268 SB - IM MH - Humans MH - France MH - *Renal Insufficiency, Chronic/therapy/economics MH - Qualitative Research MH - *Capitation Fee MH - Patient Care Team/economics PMC - PMC12484326 OTO - NOTNLM OT - Capitation payment OT - Chronic kidney disease OT - Health policy OT - Multidisciplinary care COIS- Declarations. Conflict of interest: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. Ethical approval: This study was approved by the French School of Public Health data protection officer. Human and animal rights: The interviews performed with the human participants were performed in accordance with the ethical standard of the French national regulations and the institution, French School of Public Health. Informed consent to participate: The participants' consent was sought and obtained before each interview, with information provided about the purposes of the study. EDAT- 2025/04/22 16:26 MHDA- 2025/09/30 19:23 PMCR- 2025/04/22 CRDT- 2025/04/22 11:29 PHST- 2024/11/12 00:00 [received] PHST- 2025/03/17 00:00 [accepted] PHST- 2025/09/30 19:23 [medline] PHST- 2025/04/22 16:26 [pubmed] PHST- 2025/04/22 11:29 [entrez] PHST- 2025/04/22 00:00 [pmc-release] AID - 10.1007/s40620-025-02284-8 [pii] AID - 2284 [pii] AID - 10.1007/s40620-025-02284-8 [doi] PST - ppublish SO - J Nephrol. 2025 Sep;38(7):1877-1887. doi: 10.1007/s40620-025-02284-8. Epub 2025 Apr 22. PMID- 41698327 OWN - NLM STAT- In-Process DCOM- 20260313 LR - 20260608 IS - 1540-1413 (Electronic) IS - 1540-1405 (Linking) VI - 24 IP - 3 DP - 2026 Feb 13 TI - Renal Impairment and Late Toxicities Comparing Contemporary Chemotherapy Regimens for Testicular Cancer in a Real-World Setting. PG - 81-90 LID - 10.6004/jnccn.2025.7120 [doi] AB - BACKGROUND: This study aimed to quantify, for the first time, cumulative burden of morbidity (CBM) scores-including renal function-in long-term testicular cancer survivors (TCS) treated with contemporary NCCN-endorsed regimens of 4 cycles of etoposide/cisplatin (EPx4) or 3 or 4 cycles of bleomycin/etoposide/cisplatin (BEPx3/BEPx4). PATIENTS AND METHODS: A total of 798 TCS underwent baseline clinical examinations and completed follow-up questionnaires. Severity grades for adverse health outcomes (AHOs) and CBM scores were calculated. Adjusted ordinal logistic regression estimated odds ratios (ORs) for AHOs and CBM scores by treatment regimen. Baseline estimated glomerular filtration rate (eGFR) was analyzed for associations with cumulative cisplatin dose and selected follow-up AHOs. RESULTS: Median age at follow-up was 45 years (median, 11 years postchemotherapy); 516 (65%) TCS survived ≥10 years. Chemotherapy consisted largely of BEPx3 (n=317; 39.7%), EPx4 (n=198; 24.8%), or BEPx4 (n=99; 12.4%); 27 (3.4%) received etoposide/ifosfamide/cisplatin (VIPx4). TCS receiving EPx4 (vs BEPx3) had significantly increased odds of worse renal impairment (adjusted OR [aOR], 1.55; P=.035), ototoxicity (aOR, 1.48; P=.04), and neuropathy (aOR, 1.77; P=.002). Reduced eGFR (<90 mL/min/1.73 m2), observed in 41% of TCS, was associated with cumulative cisplatin dose (r = -0.149; P<.0001) and with 2- to 20-fold increased odds of developing hypertension (60-89 mL/min/1.73 m2: OR, 2.01; P=.001; 45-59 mL/min/1.73 m2: OR, 2.84; P=.040; 30-44 mL/min/1.73 m2: OR, 20.0; P=.001). Moderate-to-severe eGFR reductions (30-44 mL/min/1.73 m2) were also associated with significantly increased odds of developing hyperlipidemia (OR, 6.10; P=.032) and/or cardiovascular disease (CVD) (OR, 7.09; P=.023). Significantly increased odds of Raynaud phenomenon were associated with β-blocker use (OR, 2.17; P=.036), peripheral artery disease (OR, 3.14; P=.002), reduced eGFR (OR per 10 mL/min/1.73 m2 increase in eGFR, 0.90; P=.027), and BEPx4 (OR vs EPx4, 2.18; P=.003). CBM scores were similar after EPx4 compared with BEPx3 (aOR, 1.04; P=.83) but worse after BEPx4 (aOR, 1.77; P=.016) or VIPx4 (aOR, 2.24; P=.038). Self-reported global physical health correlated strongly with CBM score (P<.001) and chemotherapy regimen (P<.001). CONCLUSIONS: This multicenter, real-world study shows that long-term CBM scores after NCCN-endorsed EPx4 or BEPx3 are comparable, but statistically significant differences in cisplatin-related toxicities exist. Cisplatin dose-dependent reductions in eGFR are followed by significant excesses of hypertension, hyperlipidemia, and CVD. FAU - Kerns, Sarah L AU - Kerns SL AD - 1Department of Radiation Oncology, The Medical College of Wisconsin, Milwaukee, WI. FAU - Dinh, Paul C Jr AU - Dinh PC Jr AD - 2Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN. FAU - Monahan, Patrick O AU - Monahan PO AD - 3Department of Biostatistics and Health Data Science, Indiana University, Indianapolis, IN. FAU - Fung, Chunkit AU - Fung C AD - 4J.P. Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY. FAU - Sesso, Howard D AU - Sesso HD AD - 5Division of Preventive Medicine, Brigham and Women's Hospital, Boston, MA. FAU - Feldman, Darren R AU - Feldman DR AD - 6Memorial Sloan Kettering Cancer Center, New York, NY. AD - 7Department of Medicine, Weill Cornell Medical College, New York, NY. FAU - Hamilton, Robert J AU - Hamilton RJ AD - 8Division of Urology, Department of Surgical Oncology, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, Canada. FAU - Vaughn, David J AU - Vaughn DJ AD - 9Department of Medicine, University of Pennsylvania, Philadelphia, PA. FAU - Kollmannsberger, Christian AU - Kollmannsberger C AD - 10Division of Medical Oncology, University of British Columbia, Vancouver, Canada. FAU - Martin, Neil E AU - Martin NE AD - 11Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, MA. FAU - Einhorn, Lawrence H AU - Einhorn LH AD - 2Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN. FAU - Travis, Lois B AU - Travis LB AD - 2Division of Medical Oncology, Indiana University School of Medicine, Indianapolis, IN. LA - eng PT - Journal Article DEP - 20260213 PL - United States TA - J Natl Compr Canc Netw JT - Journal of the National Comprehensive Cancer Network : JNCCN JID - 101162515 RN - Q20Q21Q62J (Cisplatin) RN - 6PLQ3CP4P3 (Etoposide) RN - 11056-06-7 (Bleomycin) SB - IM MH - Humans MH - Male MH - *Testicular Neoplasms/drug therapy/mortality/complications/pathology MH - *Antineoplastic Combined Chemotherapy Protocols/adverse effects/therapeutic use MH - Middle Aged MH - Cisplatin/adverse effects/administration & dosage MH - Adult MH - Etoposide/adverse effects/administration & dosage MH - Bleomycin/adverse effects/administration & dosage MH - Glomerular Filtration Rate/drug effects MH - *Renal Insufficiency/epidemiology/chemically induced/diagnosis MH - Young Adult MH - Follow-Up Studies MH - Cancer Survivors EDAT- 2026/02/17 00:53 MHDA- 2026/03/14 00:31 CRDT- 2026/02/16 18:23 PHST- 2025/06/19 00:00 [received] PHST- 2025/10/14 00:00 [accepted] PHST- 2026/03/14 00:31 [medline] PHST- 2026/02/17 00:53 [pubmed] PHST- 2026/02/16 18:23 [entrez] AID - 10.6004/jnccn.2025.7120 [doi] PST - epublish SO - J Natl Compr Canc Netw. 2026 Feb 13;24(3):81-90. doi: 10.6004/jnccn.2025.7120. PMID- 27149136 OWN - NLM STAT- MEDLINE DCOM- 20170331 LR - 20210103 IS - 1847-6538 (Electronic) IS - 1330-027X (Linking) VI - 24 IP - 1 DP - 2016 Apr TI - Paraneoplastic Scleroderma: Are There Any Clues? PG - 78-80 AB - Dear Editor, Scleroderma associated with neoplasia is rare, with only a small number of cases reported. We describe 4 patients with paraneoplastic scleroderma who were treated at the I. Department of Dermatovenereology, St. Anna Hospital, during the period between 2004 and 2014. The patients were diagnosed with cholangiogenic carcinoma, endometrial carcinoma, prostatic adenocarcinoma, and adenoma of the suprarenal gland. In the case of concurrent scleroderma and tumor, four situations may occur: they can develop independently of each other; scleroderma may be induced by the tumor; the tumor can develop in the scleroderma; or the tumor can be induced by immunosuppressive therapy. Sclerotization of the skin was described in association with lung cancer, carcinoid, plasma cell dyscrasia, cancer of the ovary, cervix, breast, esophagus, stomach, nasopharynx, melanoma, and sarcoma (1,2,5,7,10). Symptoms may be induced by substances secreted by the tumor (hormones, cytokines, etc.) (9). Tumorous cells further induce cytotoxic and autoantibody response. Scleroderma is characterized by immunological dysregulation, vasculopathy, and hyperproduction of the extracellular matrix by activated fibroblasts. Endothelial, inflammatory, and mesenchymal cells produce cytokines, chemokines, and growth factors e.g. Interleukin-1 (IL1), Interleukin-6 (IL6), tumor necrosis factor alpha (TNF α), collagen alpha 1, connective tissue growth factor (CTGF) (3), and basic fibroblast growth factor (bFGF). This factor is also produced by lung cancer cells (4). The clinical picture of scleroderma and paraneoplastic scleroderma is similar. Diffuse thickening of the skin and/or sclerodermatous plaques can be seen. The histological picture is consistent with scleroderma. Capillaroscopy changes, antinuclear antibodies (ANA), sclerodactyly, and Raynaud phenomenon suggest the diagnosis of systemic scleroderma (SS) (4). Our patients did not fulfill enough of the criteria for SS. Both diffuse and localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented with diffusely tough skin on her extremities and trunk, present for 2 months. Examination revealed cervicitis with a benign endometric polyp, cholecystolithiasis, borderline pulmonary hypertension, and a hormonally inactive suprarenal adenoma. She was given prednisone 40 mg/day and penicillamine with effect. In the 3rd year of therapy she has residual induration of her lower legs and a scleroderma plaque in the lumbar region. She is monitored for her suprarenal adenoma. Two patients had scleroderma at the same time as a malignant tumor; in one patient the localized scleroderma worsened rapidly at the time of the tumor diagnosis, and in one patient a clinically silent adenoma was found. Adrenal tissue can secrete molecules such as serotonine or bFGF involved in fibroplasia (3,6). One patient died of a metastatic disease, two patients after the successful treatment of the tumor, and the patient with suprarenal adenoma experienced softening of the skin and regression of scleroderma. Although paraneoplastic scleroderma is often classified as a pseudoscleroderma, we regard neoplasia as a distinct triggering impulse for scleroderma. Recently, an association between RNA polymerase I/III antibodies in systemic scleroderma and cancer was suggested (8). Such studies may confirm the true link between scleroderma and malignancy. These patients are characterized by older age, sudden onset, diffuse thickening of the skin, and/or generalized morphea with a concurrent neoplastic process. In the case of a successful tumor treatment, skin changes regress. FAU - Jedlickova, Hana AU - Jedlickova H AD - Prof. Hana Jedlickova, MD, PhD, St. Anna University Hospital, Masaryk University, Brno, Czech Republic; hana.jedlickova@fnusa.cz. FAU - Durčanská, Veronika AU - Durčanská V FAU - Vašků, Vladimír AU - Vašků V LA - eng PT - Case Reports PT - Journal Article PL - Croatia TA - Acta Dermatovenerol Croat JT - Acta dermatovenerologica Croatica : ADC JID - 9433781 SB - IM MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Male MH - Middle Aged MH - Paraneoplastic Syndromes/complications/*diagnosis MH - Scleroderma, Localized/complications/*diagnosis EDAT- 2016/05/07 06:00 MHDA- 2017/04/01 06:00 CRDT- 2016/05/06 06:00 PHST- 2016/05/06 06:00 [entrez] PHST- 2016/05/07 06:00 [pubmed] PHST- 2017/04/01 06:00 [medline] PST - ppublish SO - Acta Dermatovenerol Croat. 2016 Apr;24(1):78-80. PMID- 19377476 OWN - NLM STAT- MEDLINE DCOM- 20090629 LR - 20250529 IS - 1546-1718 (Electronic) IS - 1061-4036 (Print) IS - 1061-4036 (Linking) VI - 41 IP - 5 DP - 2009 May TI - A systematic, large-scale resequencing screen of X-chromosome coding exons in mental retardation. PG - 535-43 LID - 10.1038/ng.367 [doi] AB - Large-scale systematic resequencing has been proposed as the key future strategy for the discovery of rare, disease-causing sequence variants across the spectrum of human complex disease. We have sequenced the coding exons of the X chromosome in 208 families with X-linked mental retardation (XLMR), the largest direct screen for constitutional disease-causing mutations thus far reported. The screen has discovered nine genes implicated in XLMR, including SYP, ZNF711 and CASK reported here, confirming the power of this strategy. The study has, however, also highlighted issues confronting whole-genome sequencing screens, including the observation that loss of function of 1% or more of X-chromosome genes is compatible with apparently normal existence. FAU - Tarpey, Patrick S AU - Tarpey PS AD - Wellcome Trust Sanger Institute, Hinxton, Cambridge, UK. FAU - Smith, Raffaella AU - Smith R FAU - Pleasance, Erin AU - Pleasance E FAU - Whibley, Annabel AU - Whibley A FAU - Edkins, Sarah AU - Edkins S FAU - Hardy, Claire AU - Hardy C FAU - O'Meara, Sarah AU - O'Meara S FAU - Latimer, Calli AU - Latimer C FAU - Dicks, Ed AU - Dicks E FAU - Menzies, Andrew AU - Menzies A FAU - Stephens, Phil AU - Stephens P FAU - Blow, Matt AU - Blow M FAU - Greenman, Chris AU - Greenman C FAU - Xue, Yali AU - Xue Y FAU - Tyler-Smith, Chris AU - Tyler-Smith C FAU - Thompson, Deborah AU - Thompson D FAU - Gray, Kristian AU - Gray K FAU - Andrews, Jenny AU - Andrews J FAU - Barthorpe, Syd AU - Barthorpe S FAU - Buck, Gemma AU - Buck G FAU - Cole, Jennifer AU - Cole J FAU - Dunmore, Rebecca AU - Dunmore R FAU - Jones, David AU - Jones D FAU - Maddison, Mark AU - Maddison M FAU - Mironenko, Tatiana AU - Mironenko T FAU - Turner, Rachel AU - Turner R FAU - Turrell, Kelly AU - Turrell K FAU - Varian, Jennifer AU - Varian J FAU - West, Sofie AU - West S FAU - Widaa, Sara AU - Widaa S FAU - Wray, Paul AU - Wray P FAU - Teague, Jon AU - Teague J FAU - Butler, Adam AU - Butler A FAU - Jenkinson, Andrew AU - Jenkinson A FAU - Jia, Mingming AU - Jia M FAU - Richardson, David AU - Richardson D FAU - Shepherd, Rebecca AU - Shepherd R FAU - Wooster, Richard AU - Wooster R FAU - Tejada, M Isabel AU - Tejada MI FAU - Martinez, Francisco AU - Martinez F FAU - Carvill, Gemma AU - Carvill G FAU - Goliath, Rene AU - Goliath R FAU - de Brouwer, Arjan P M AU - de Brouwer AP FAU - van Bokhoven, Hans AU - van Bokhoven H FAU - Van Esch, Hilde AU - Van Esch H FAU - Chelly, Jamel AU - Chelly J FAU - Raynaud, Martine AU - Raynaud M FAU - Ropers, Hans-Hilger AU - Ropers HH FAU - Abidi, Fatima E AU - Abidi FE FAU - Srivastava, Anand K AU - Srivastava AK FAU - Cox, James AU - Cox J FAU - Luo, Ying AU - Luo Y FAU - Mallya, Uma AU - Mallya U FAU - Moon, Jenny AU - Moon J FAU - Parnau, Josef AU - Parnau J FAU - Mohammed, Shehla AU - Mohammed S FAU - Tolmie, John L AU - Tolmie JL FAU - Shoubridge, Cheryl AU - Shoubridge C FAU - Corbett, Mark AU - Corbett M FAU - Gardner, Alison AU - Gardner A FAU - Haan, Eric AU - Haan E FAU - Rujirabanjerd, Sinitdhorn AU - Rujirabanjerd S FAU - Shaw, Marie AU - Shaw M FAU - Vandeleur, Lucianne AU - Vandeleur L FAU - Fullston, Tod AU - Fullston T FAU - Easton, Douglas F AU - Easton DF FAU - Boyle, Jackie AU - Boyle J FAU - Partington, Michael AU - Partington M FAU - Hackett, Anna AU - Hackett A FAU - Field, Michael AU - Field M FAU - Skinner, Cindy AU - Skinner C FAU - Stevenson, Roger E AU - Stevenson RE FAU - Bobrow, Martin AU - Bobrow M FAU - Turner, Gillian AU - Turner G FAU - Schwartz, Charles E AU - Schwartz CE FAU - Gecz, Jozef AU - Gecz J FAU - Raymond, F Lucy AU - Raymond FL FAU - Futreal, P Andrew AU - Futreal PA FAU - Stratton, Michael R AU - Stratton MR LA - eng GR - 11022/CRUK_/Cancer Research UK/United Kingdom GR - R01 HD026202/HD/NICHD NIH HHS/United States GR - HD26202/HD/NICHD NIH HHS/United States GR - 077012/WT_/Wellcome Trust/United Kingdom GR - 10118/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20090419 PL - United States TA - Nat Genet JT - Nature genetics JID - 9216904 SB - IM CIN - Nat Genet. 2009 May;41(5):510-2. doi: 10.1038/ng0509-510. PMID: 19399033 CIN - Pediatr Res. 2009 Jul;66(1):2. doi: 10.1203/PDR.0b013e3181aebf63. PMID: 19542828 CIN - Clin Genet. 2010 Jan;77(1):35-6. doi: 10.1111/j.1399-0004.2009.01299.x. PMID: 19912263 CIN - Clin Genet. 2009 Dec;76(6):497-9. doi: 10.1111/j.1399-0004.2009.01295.x. PMID: 19930150 MH - Chromosome Mapping MH - Chromosomes, Human, X/*genetics MH - Exons/*genetics MH - Female MH - Genetic Variation MH - Humans MH - Male MH - X-Linked Intellectual Disability/*genetics MH - Pedigree MH - Sequence Analysis, DNA/*methods PMC - PMC2872007 MID - UKMS30579 OID - NLM: UKMS30579 EDAT- 2009/04/21 09:00 MHDA- 2009/06/30 09:00 PMCR- 2010/05/18 CRDT- 2009/04/21 09:00 PHST- 2008/12/08 00:00 [received] PHST- 2009/02/02 00:00 [accepted] PHST- 2009/04/21 09:00 [entrez] PHST- 2009/04/21 09:00 [pubmed] PHST- 2009/06/30 09:00 [medline] PHST- 2010/05/18 00:00 [pmc-release] AID - ng.367 [pii] AID - 10.1038/ng.367 [doi] PST - ppublish SO - Nat Genet. 2009 May;41(5):535-43. doi: 10.1038/ng.367. Epub 2009 Apr 19. PMID- 35757612 OWN - NLM STAT- MEDLINE DCOM- 20220628 LR - 20260518 IS - 2296-2565 (Electronic) IS - 2296-2565 (Linking) VI - 10 DP - 2022 TI - Social Isolation Among Older Adults in the Time of COVID-19: A Gender Perspective. PG - 840940 LID - 10.3389/fpubh.2022.840940 [doi] LID - 840940 AB - We aimed to analyze inequalities in social isolation among older adults in a time of COVID-19 social restrictions, using a gender perspective. A random population-based survey, including 21,543 older adults (65+) was conducted during and post COVID-19 lockdown in France. Our main outcome was a three-dimension indicator of social isolation based on living conditions, i.e., living alone (i) and not having gone out in the past week (ii), completed by an indicator measuring Internet use i.e., never using the Internet (iii). Logistic regressions were used to identify factors associated with isolation for women and men. Women were more likely to live alone (aOR = 2.72 [2.53; 2.92]), not to have gone out in the past week (aOR = 1.53 [1.39; 1.68]), and not to use the Internet (aOR = 1.30 [1.20; 1.44]). In addition to gender effects, being older, at the bottom of the social hierarchy, and from an ethno-racial minority was also associated with social isolation. Preventive policies should take into account these inequalities when addressing the issue of social isolation among older women and men, so as to enable all social groups to maintain social contacts, and access health information. CI - Copyright © 2022 Silberzan, Martin, Bajos and EpiCov Study Group. FAU - Silberzan, Léna AU - Silberzan L AD - IRIS, Inserm, Aubervilliers, France. FAU - Martin, Claude AU - Martin C AD - Arènes (UMR 6051), CNRS, EHESP, Rennes, France. FAU - Bajos, Nathalie AU - Bajos N AD - IRIS, Inserm/EHESS, Aubervilliers, France. CN - EpiCov Study Group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220609 PL - Switzerland TA - Front Public Health JT - Frontiers in public health JID - 101616579 SB - IM MH - Aged MH - *COVID-19/epidemiology MH - Communicable Disease Control MH - Female MH - France/epidemiology MH - Humans MH - Male MH - Social Isolation PMC - PMC9228032 OTO - NOTNLM OT - COVID-19 OT - gender OT - older adults OT - social contacts OT - social inequalities COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. FIR - Bajos, Nathalie IR - Bajos N FIR - Warszawski, Josiane IR - Warszawski J FIR - Bagein, Guillaume IR - Bagein G FIR - Barlet, Muriel IR - Barlet M FIR - Beck, François IR - Beck F FIR - Counil, Emilie IR - Counil E FIR - Jusot, Florence IR - Jusot F FIR - Leduc, Aude IR - Leduc A FIR - Lydie, Nathalie IR - Lydie N FIR - Martin, Claude IR - Martin C FIR - Meyer, Laurence IR - Meyer L FIR - Raynaud, Philippe IR - Raynaud P FIR - Rouquette, Alexandra IR - Rouquette A FIR - Pailhé, Ariane IR - Pailhé A FIR - Paliod, Nicolas IR - Paliod N FIR - Rahib, Delphine IR - Rahib D FIR - Sillard, Patrick IR - Sillard P FIR - Slama, Rémy IR - Slama R FIR - Spire, Alexis IR - Spire A EDAT- 2022/06/28 06:00 MHDA- 2022/06/29 06:00 PMCR- 2022/06/09 CRDT- 2022/06/27 04:29 PHST- 2021/12/21 00:00 [received] PHST- 2022/05/16 00:00 [accepted] PHST- 2022/06/27 04:29 [entrez] PHST- 2022/06/28 06:00 [pubmed] PHST- 2022/06/29 06:00 [medline] PHST- 2022/06/09 00:00 [pmc-release] AID - 10.3389/fpubh.2022.840940 [doi] PST - epublish SO - Front Public Health. 2022 Jun 9;10:840940. doi: 10.3389/fpubh.2022.840940. eCollection 2022. PMID- 19246760 OWN - NLM STAT- MEDLINE DCOM- 20090409 LR - 20200826 IS - 1350-0872 (Print) IS - 1350-0872 (Linking) VI - 155 IP - Pt 3 DP - 2009 Mar TI - lmo1273, a novel gene involved in Listeria monocytogenes virulence. PG - 891-902 LID - 10.1099/mic.0.022277-0 [doi] AB - Listeria monocytogenes is a foodborne pathogen able to infect humans and many other mammalian species, leading to serious, often fatal disease. We have previously identified a five-gene locus in the genome of L. monocytogenes EGD-e which comprised three contiguous genes encoding paralogous type I signal peptidases. In the present study, we focused on the two distal genes of the locus (lmo1272 and lmo1273), encoding proteins sharing significant similarities with the YlqF and RnhB proteins, respectively, of Bacillus subtilis. lmo1273 could complement an Escherichia coli rnhA-rnhB thermosensitive growth phenotype, suggesting that it encodes a functional RNase H. Strikingly, inactivation of lmo1273 provoked a strong attenuation of virulence in the mouse model, and kinetic studies in infected mice revealed that multiplication of the lmo1273 mutant in target organs was significantly impaired. However, the mutation did not impair L. monocytogenes intracellular multiplication or cell-to-cell spread in cell culture models. Transcriptional profiles obtained with an lmo1273-overexpressing strain were compared to those of the wild-type strain, using microarray analyses. The data obtained suggest a pleiotropic regulatory role of Lmo1273 and possible links with amino acid uptake. FAU - Bigot, Armelle AU - Bigot A AD - Inserm, U570, Unité de Pathogénie des Infections Systémiques, Paris F-75015, France. AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. FAU - Raynaud, Catherine AU - Raynaud C AD - Inserm, U570, Unité de Pathogénie des Infections Systémiques, Paris F-75015, France. AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. FAU - Dubail, Iharilalao AU - Dubail I AD - Inserm, U570, Unité de Pathogénie des Infections Systémiques, Paris F-75015, France. AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. FAU - Dupuis, Marion AU - Dupuis M AD - Inserm, U570, Unité de Pathogénie des Infections Systémiques, Paris F-75015, France. AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. FAU - Hossain, Hamid AU - Hossain H AD - Institute for Medical Microbiology, Justus-Liebig-University, Frankfurter Strasse 107, D-35392 Giessen, Germany. FAU - Hain, Torsten AU - Hain T AD - Institute for Medical Microbiology, Justus-Liebig-University, Frankfurter Strasse 107, D-35392 Giessen, Germany. FAU - Chakraborty, Trinad AU - Chakraborty T AD - Institute for Medical Microbiology, Justus-Liebig-University, Frankfurter Strasse 107, D-35392 Giessen, Germany. FAU - Charbit, Alain AU - Charbit A AD - Inserm, U570, Unité de Pathogénie des Infections Systémiques, Paris F-75015, France. AD - Université Paris Descartes, Faculté de Médecine René Descartes, Paris F-75015, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Microbiology (Reading) JT - Microbiology (Reading, England) JID - 9430468 RN - 0 (Bacterial Proteins) RN - 0 (RNA, Bacterial) RN - EC 3.1.26.4 (Ribonuclease H) SB - IM MH - Animals MH - Bacterial Proteins/genetics/*metabolism MH - Cell Line MH - Escherichia coli/genetics MH - Female MH - Gene Deletion MH - Gene Expression Profiling MH - Gene Expression Regulation, Bacterial MH - Genes, Bacterial MH - Genetic Complementation Test MH - Listeria monocytogenes/*genetics/metabolism/*pathogenicity MH - Listeriosis/microbiology MH - Mice MH - Oligonucleotide Array Sequence Analysis MH - RNA, Bacterial/genetics MH - Ribonuclease H/genetics/metabolism MH - *Virulence EDAT- 2009/02/28 09:00 MHDA- 2009/04/10 09:00 CRDT- 2009/02/28 09:00 PHST- 2009/02/28 09:00 [entrez] PHST- 2009/02/28 09:00 [pubmed] PHST- 2009/04/10 09:00 [medline] AID - 10.1099/mic.0.022277-0 [doi] PST - ppublish SO - Microbiology (Reading). 2009 Mar;155(Pt 3):891-902. doi: 10.1099/mic.0.022277-0. PMID- 18803640 OWN - NLM STAT- MEDLINE DCOM- 20090326 LR - 20090107 IS - 1475-097X (Electronic) IS - 1475-0961 (Linking) VI - 29 IP - 1 DP - 2009 Jan TI - Abnormal respiratory-related evoked potentials in untreated awake patients with severe obstructive sleep apnoea syndrome. PG - 10-7 LID - 10.1111/j.1475-097X.2008.00830.x [doi] AB - AIM: Obstructive sleep apnoeas generate an intense afferent traffic leading to arousal and apnoea termination. Yet a decrease in the sensitivity of the afferents has been described in patients with obstructive sleep apnoea, and could be a determinant of disease severity. How mechanical changes within the respiratory system are processed in the brain can be studied through the analysis of airway occlusion-related respiratory-related evoked potentials. Respiratory-related evoked potentials have been found altered during sleep in mild and moderate obstructive sleep apnoea syndrome, with contradictory results during wake. We hypothesized that respiratory-related evoked potentials' alterations during wake, if indeed a feature of the obstructive sleep apnoea syndrome, should be present in untreated severe patients. METHODS: Ten untreated patients with severe obstructive sleep apnoea syndrome and eight matched controls were studied. Respiratory-related evoked potentials were recorded in Cz-C3 and Cz-C4, and described in terms of the amplitudes and latencies of their components P1, N1, P2 and N2. RESULTS: Components amplitudes were similar in both groups. There was no significant difference in P1 latencies. This was also the case for N1 in Cz-C3. In contrast, N1 latencies in Cz-C4 were significantly longer in patients with obstructive sleep apnoea syndrome [median 98 ms (interquartile range 16.00) versus 79.5 ms (5.98), P = 0.015]. P2 and N2 were also significantly delayed, on both sides. CONCLUSIONS: The cortical processing of airway occlusion-related afferents seems abnormal in untreated patients with severe obstructive sleep apnoea syndrome. This could be either a severity marker and/or an aggravating factor. FAU - Donzel-Raynaud, Christine AU - Donzel-Raynaud C AD - AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Laboratoire de Physiopathologie Respiratoire, Paris, France. christine.raynaud@imm.fr FAU - Redolfi, Stefania AU - Redolfi S FAU - Arnulf, Isabelle AU - Arnulf I FAU - Similowski, Thomas AU - Similowski T FAU - Straus, Christian AU - Straus C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080916 PL - England TA - Clin Physiol Funct Imaging JT - Clinical physiology and functional imaging JID - 101137604 SB - IM MH - Adult MH - Afferent Pathways/physiopathology MH - Case-Control Studies MH - Electroencephalography MH - Electromyography MH - Electrooculography MH - *Evoked Potentials, Somatosensory MH - Female MH - Humans MH - Male MH - Middle Aged MH - Polysomnography MH - Reaction Time MH - *Respiration MH - Severity of Illness Index MH - Sleep Apnea, Obstructive/*physiopathology MH - *Wakefulness EDAT- 2008/09/23 09:00 MHDA- 2009/03/27 09:00 CRDT- 2008/09/23 09:00 PHST- 2008/09/23 09:00 [pubmed] PHST- 2009/03/27 09:00 [medline] PHST- 2008/09/23 09:00 [entrez] AID - CPF830 [pii] AID - 10.1111/j.1475-097X.2008.00830.x [doi] PST - ppublish SO - Clin Physiol Funct Imaging. 2009 Jan;29(1):10-7. doi: 10.1111/j.1475-097X.2008.00830.x. Epub 2008 Sep 16. PMID- 21836662 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20111110 LR - 20211020 IS - 1877-6566 (Electronic) IS - 1877-6558 (Print) IS - 1877-6558 (Linking) VI - 3 IP - 1-4 DP - 2009 Dec TI - Mutation screening in 86 known X-linked mental retardation genes by droplet-based multiplex PCR and massive parallel sequencing. PG - 41-9 LID - 10.1007/s11568-010-9137-y [doi] AB - Massive parallel sequencing has revolutionized the search for pathogenic variants in the human genome, but for routine diagnosis, re-sequencing of the complete human genome in a large cohort of patients is still far too expensive. Recently, novel genome partitioning methods have been developed that allow to target re-sequencing to specific genomic compartments, but practical experience with these methods is still limited. In this study, we have combined a novel droplet-based multiplex PCR method and next generation sequencing to screen patients with X-linked mental retardation (XLMR) for mutations in 86 previously identified XLMR genes. In total, affected males from 24 large XLMR families were analyzed, including three in whom the mutations were already known. Amplicons corresponding to functionally relevant regions of these genes were sequenced on an Illumina/Solexa Genome Analyzer II platform. Highly specific and uniform enrichment was achieved: on average, 67.9% unambiguously mapped reads were derived from amplicons, and for 88.5% of the targeted bases, the sequencing depth was sufficient to reliably detect variations. Potentially disease-causing sequence variants were identified in 10 out of 24 patients, including the three mutations that were already known, and all of these could be confirmed by Sanger sequencing. The robust performance of this approach demonstrates the general utility of droplet-based multiplex PCR for parallel mutation screening in hundreds of genes, which is a prerequisite for the diagnosis of mental retardation and other disorders that may be due to defects of a wide variety of genes. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s11568-010-9137-y) contains supplementary material, which is available to authorized users. FAU - Hu, Hao AU - Hu H FAU - Wrogemann, Klaus AU - Wrogemann K FAU - Kalscheuer, Vera AU - Kalscheuer V FAU - Tzschach, Andreas AU - Tzschach A FAU - Richard, Hugues AU - Richard H FAU - Haas, Stefan A AU - Haas SA FAU - Menzel, Corinna AU - Menzel C FAU - Bienek, Melanie AU - Bienek M FAU - Froyen, Guy AU - Froyen G FAU - Raynaud, Martine AU - Raynaud M FAU - Van Bokhoven, Hans AU - Van Bokhoven H FAU - Chelly, Jamel AU - Chelly J FAU - Ropers, Hilger AU - Ropers H FAU - Chen, Wei AU - Chen W LA - eng PT - Journal Article DEP - 20100325 PL - Netherlands TA - Hugo J JT - The HUGO journal JID - 101530565 EIN - Hugo J. 2009 Dec;3(1-4):83. doi: 10.1007/s11568-010-9142-1. PMID: 20535404 PMC - PMC2882650 OTO - NOTNLM OT - Droplet-based multiplex PCR OT - Massive parallel sequencing OT - Mutation screening OT - X-linked mental retardation EDAT- 2009/12/01 00:00 MHDA- 2009/12/01 00:01 PMCR- 2010/03/25 CRDT- 2011/08/13 06:00 PHST- 2010/01/13 00:00 [received] PHST- 2010/02/24 00:00 [revised] PHST- 2010/03/12 00:00 [accepted] PHST- 2011/08/13 06:00 [entrez] PHST- 2009/12/01 00:00 [pubmed] PHST- 2009/12/01 00:01 [medline] PHST- 2010/03/25 00:00 [pmc-release] AID - 9137 [pii] AID - 10.1007/s11568-010-9137-y [doi] PST - ppublish SO - Hugo J. 2009 Dec;3(1-4):41-9. doi: 10.1007/s11568-010-9137-y. Epub 2010 Mar 25. PMID- 37105444 OWN - NLM STAT- MEDLINE DCOM- 20230731 LR - 20230731 IS - 1535-7732 (Electronic) IS - 1051-0443 (Linking) VI - 34 IP - 8 DP - 2023 Aug TI - The Safety and Effectiveness of Hepatic Transarterial Embolic Locoregional Therapy in Patients with Contraindications to Hepatectomy after Portal Vein Embolization. PG - 1324-1330.e6 LID - S1051-0443(23)00298-1 [pii] LID - 10.1016/j.jvir.2023.04.016 [doi] AB - The safety and effectiveness of hepatic transarterial embolic locoregional therapy (LRT) was assessed, including transarterial chemoembolization (TACE) and transarterial radioembolization (TARE), in patients who underwent portal vein embolization (PVE) before major hepatectomy in whom surgery was then contraindicated. Adverse events (AEs) were graded according to the Society of Interventional Radiology classification of AEs. Tumor response was assessed based on the Response Evaluation Criteria In Solid Tumors 1.1. Overall survival (OS) and progression-free survival (PFS) were estimated. Fifteen patients underwent 37 transarterial LRTs (25 TACEs, 11 TAREs, and 1 bland embolization), most (73%) with hepatocellular carcinoma. Eleven AEs occurred in 7 patients, including 2 Grade 3/5 (severe) and 2 Grade 4/5 (life-threatening) events. The best response was partial response in 4 (27%) and stable disease in 10 (66%) patients. The median OS and PFS were 42 (95% CI, 35-49 months) and 33 months (95% CI, 24-42 months), respectively. In conclusion, hepatic transarterial LRT can be considered as a therapeutic option in patients with contraindicated liver surgery after PVE. CI - Copyright © 2023 SIR. Published by Elsevier Inc. All rights reserved. FAU - Bacquet, Raphaël AU - Bacquet R AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France. FAU - Dioguardi Burgio, Marco AU - Dioguardi Burgio M AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France; Université Paris Cité, INSERM U1149 "Centre de Recherche sur l'Inflammation," CRI, Paris, France. FAU - Gregory, Jules AU - Gregory J AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France; Université Paris Cité, INSERM U1149 "Centre de Recherche sur l'Inflammation," CRI, Paris, France. FAU - Bouattour, Mohamed AU - Bouattour M AD - Liver Cancer Unit, Hôpital Beaujon, AP-HP.Nord, Paris, France. FAU - Cauchy, François AU - Cauchy F AD - Department of HPB Surgery and Liver Transplantation, Hôpital Beaujon, AP-HP.Nord, Paris, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France. FAU - Paulatto, Luisa AU - Paulatto L AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France. FAU - Lebtahi, Rachida AU - Lebtahi R AD - Department of Nuclear Medicine, Hôpital Beaujon, AP-HP.Nord, Paris, France; Université Paris Cité, INSERM U1149 "Centre de Recherche sur l'Inflammation," CRI, Paris, France. FAU - Vilgrain, Valerie AU - Vilgrain V AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France; Université Paris Cité, INSERM U1149 "Centre de Recherche sur l'Inflammation," CRI, Paris, France. FAU - Ronot, Maxime AU - Ronot M AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France; Université Paris Cité, INSERM U1149 "Centre de Recherche sur l'Inflammation," CRI, Paris, France. Electronic address: Maxime.ronot@aphp.fr. LA - eng PT - Journal Article DEP - 20230425 PL - United States TA - J Vasc Interv Radiol JT - Journal of vascular and interventional radiology : JVIR JID - 9203369 SB - IM MH - Humans MH - *Carcinoma, Hepatocellular/diagnostic imaging/therapy MH - *Liver Neoplasms/diagnostic imaging/therapy MH - Hepatectomy/adverse effects MH - *Chemoembolization, Therapeutic/adverse effects MH - Portal Vein/diagnostic imaging/pathology MH - Treatment Outcome MH - *Embolism/etiology MH - Contraindications EDAT- 2023/04/28 00:42 MHDA- 2023/07/31 06:43 CRDT- 2023/04/27 19:26 PHST- 2022/09/14 00:00 [received] PHST- 2023/04/03 00:00 [revised] PHST- 2023/04/19 00:00 [accepted] PHST- 2023/07/31 06:43 [medline] PHST- 2023/04/28 00:42 [pubmed] PHST- 2023/04/27 19:26 [entrez] AID - S1051-0443(23)00298-1 [pii] AID - 10.1016/j.jvir.2023.04.016 [doi] PST - ppublish SO - J Vasc Interv Radiol. 2023 Aug;34(8):1324-1330.e6. doi: 10.1016/j.jvir.2023.04.016. Epub 2023 Apr 25. PMID- 33972252 OWN - NLM STAT- MEDLINE DCOM- 20210809 LR - 20231107 IS - 1098-6596 (Electronic) IS - 0066-4804 (Print) IS - 0066-4804 (Linking) VI - 65 IP - 8 DP - 2021 Jul 16 TI - Biological and Biochemical Evaluation of Isatin-Isoniazid Hybrids as Bactericidal Candidates against Mycobacterium tuberculosis. PG - e0001121 LID - 10.1128/AAC.00011-21 [doi] LID - e00011-21 AB - Tuberculosis remains a leading cause of mortality among infectious diseases worldwide, prompting the need to discover new drugs to fight this disease. We report here the design, synthesis, and antimycobacterial activity of isatin-mono/bis-isoniazid hybrids. Most of the compounds exhibited very high activity against Mycobacterium tuberculosis, with MICs in the range of 0.195 to 0.39 μg/ml, and exerted a more potent bactericidal effect than the standard antitubercular drug isoniazid (INH). Importantly, these compounds were found to be well tolerated at high doses (>200 μg/ml) on Vero kidney cells, leading to high selectivity indices. Two of the most promising hybrids were evaluated for activity in THP-1 macrophages infected with M. tuberculosis, among which compound 11e was found to be slightly more effective than INH. Overexpression of InhA along with cross-resistance determination of the most potent compounds, selection of resistant mutants, and biochemical analysis, allowed us to decipher their mode of action. These compounds effectively inhibited mycolic acid biosynthesis and required KatG to exert their biological effects. Collectively, this suggests that the synthesized isatin-INH hybrids are promising antitubercular molecules for further evaluation in preclinical settings. FAU - Johansen, Matt D AU - Johansen MD AD - Centre National de la Recherche Scientifique UMR 9004, Institut de Recherche en Infectiologie de Montpellier (IRIM), Université de Montpellier, Montpellier, France. AD - Centre for Inflammation, Centenary Institute and University of Technology Sydney, Faculty of Science, Sydney, NSW, Australia. FAU - Shalini AU - Shalini AD - Department of Chemistry, Guru Nanak Dev University, Punjab, India. FAU - Kumar, Sumit AU - Kumar S AD - Department of Chemistry, Guru Nanak Dev University, Punjab, India. FAU - Raynaud, Clément AU - Raynaud C AD - Centre National de la Recherche Scientifique UMR 9004, Institut de Recherche en Infectiologie de Montpellier (IRIM), Université de Montpellier, Montpellier, France. FAU - Quan, Diana H AU - Quan DH AD - Tuberculosis Research Program, Centenary Institute, The University of Sydney, Camperdown, NSW, Australia. FAU - Britton, Warwick J AU - Britton WJ AD - Centenary Institute, The University of Sydney, Camperdown, NSW, Australia. AD - Department of Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. FAU - Hansbro, Philip M AU - Hansbro PM AD - Centre for Inflammation, Centenary Institute and University of Technology Sydney, Faculty of Science, Sydney, NSW, Australia. FAU - Kumar, Vipan AU - Kumar V AD - Department of Chemistry, Guru Nanak Dev University, Punjab, India. FAU - Kremer, Laurent AU - Kremer L AD - Centre National de la Recherche Scientifique UMR 9004, Institut de Recherche en Infectiologie de Montpellier (IRIM), Université de Montpellier, Montpellier, France. AD - INSERM, IRIM, Montpellier, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210716 PL - United States TA - Antimicrob Agents Chemother JT - Antimicrobial agents and chemotherapy JID - 0315061 RN - 0 (Antitubercular Agents) RN - 0 (Bacterial Proteins) RN - 82X95S7M06 (Isatin) RN - EC 1.11.1.6 (Catalase) RN - V83O1VOZ8L (Isoniazid) SB - IM MH - Antitubercular Agents/pharmacology/therapeutic use MH - Bacterial Proteins/genetics MH - Catalase/genetics MH - Humans MH - *Isatin/pharmacology MH - Isoniazid/pharmacology MH - Microbial Sensitivity Tests MH - Mutation MH - *Mycobacterium tuberculosis PMC - PMC8284457 OTO - NOTNLM OT - InhA OT - KatG OT - Mycobacterium tuberculosis OT - bactericidal activity OT - drug resistance OT - isatin-isoniazid hybrids OT - mycolic acids EDAT- 2021/05/12 06:00 MHDA- 2021/08/10 06:00 PMCR- 2022/01/16 CRDT- 2021/05/11 05:55 PHST- 2021/05/12 06:00 [pubmed] PHST- 2021/08/10 06:00 [medline] PHST- 2021/05/11 05:55 [entrez] PHST- 2022/01/16 00:00 [pmc-release] AID - AAC.00011-21 [pii] AID - 00011-21 [pii] AID - 10.1128/AAC.00011-21 [doi] PST - ppublish SO - Antimicrob Agents Chemother. 2021 Jul 16;65(8):e0001121. doi: 10.1128/AAC.00011-21. Epub 2021 Jul 16. PMID- 40592371 OWN - NLM STAT- MEDLINE DCOM- 20251024 LR - 20251203 IS - 1557-3117 (Electronic) IS - 1053-2498 (Linking) VI - 44 IP - 11 DP - 2025 Nov TI - Trajectories of FEV(1) after lung transplantation and patient outcomes. PG - 1749-1762 LID - S1053-2498(25)02036-4 [pii] LID - 10.1016/j.healun.2025.06.011 [doi] AB - BACKGROUND: The forced expiratory volume in one second (FEV(1)) is the standard measure used to monitor the lung function after lung transplantation. However, little is known about the FEV(1) trajectories post transplantation, and their associations with clinical outcomes. METHODS: Adult patients who received a bilateral lung transplant between January 1, 2010 and January 1, 2021 were included from 15 centers in France, Belgium, Austria and the US. Three French centers formed the development cohort and the remaining centers formed the external validation cohorts. All centers performed routine spirometry measurements commencing shortly after lung transplantation and continuing at regular intervals of maximum three months afterwards. Recipient, donor and transplant characteristics were collected. Latent class mixed models were used to identify FEV(1) trajectories. The number of FEV(1) trajectories was defined according to the Akaike Information Criterion, Bayesian Information Criterion, the discrimination, the entropy and the interpretability of the model. FINDINGS: A total of 2305 patients were included with 59 034 FEV(1) measurements collected. The median follow-up post-transplantation was 4.3 years (IQR 2.3-6.2). In the development cohort (n=605), the best latent class mixed model identified seven clinically meaningful FEV(1) trajectories. Trajectory #1 (25.6%) was characterized by patients with moderate and stable lung function (3-year patient survival = 79.9%); trajectory #2 (24.0%) and #3 (27.3%) were characterized by patients with moderate/high and stable lung function (3-year patient survival = 96.6% and 95.7% respectively); trajectory #4 (3.8%) was characterized by patients with increasing lung function (3-year patient survival = 69.6%); trajectory #5 (11.7%) was characterized by patients with a slow decline (3-year patient survival = 90.1%); and trajectory #6 (3.0%) and #7 (4.6%) were characterized by patients with accelerated decline (3-year patient survival = 33.3% and 59.5% respectively). Patients belonging to trajectories were associated with gender (p=0.020) and underlying disease (p=0.004). Similar FEV(1) trajectories and patient outcomes were identified in the external validation cohorts on the basis of independent analyses. The trajectories were also confirmed in a series of subpopulations. Last, FEV(1) value and slope at 1 year post transplant were strongly associated with FEV(1) trajectories, in both the development cohort and the external validation cohorts. INTERPRETATION: We identified and validated FEV(1) trajectories that capture different clinical scenarios associated with post-transplant recipient outcomes. Our results may provide the basis for a trajectory-based risk assessment of lung transplant recipients. FUNDING: "Vaincre la mucoviscidose" grant, the association Grégory Lemarchal, ANR grant, PHRC grant, the FP7 collaborative project, IRSRPL grant, the Fondation du Souffle and PLUTO DITCAP grant from Vaincre la Mucoviscidose and Association Grégory Lemarchal. CI - Copyright © 2025. Published by Elsevier Inc. FAU - Raynaud, Marc AU - Raynaud M AD - Université de Paris Cité, INSERM, PARCC, Paris Institute for Transplantation and Organ Regeneration, F-75015 Paris, France. FAU - Belousova, Natalia AU - Belousova N AD - Department of Pneumology, Foch hospital, Suresnes, France. FAU - Glorion, Matthieu AU - Glorion M AD - Deparment of thoracic surgery and lung transplantation, Foch hospital, Suresnes, France; UMR 0892 VIM, INRAE/U de Versailles, St-Quentin-en-Yvelines, France. FAU - Hillebrand, Caroline AU - Hillebrand C AD - Department of Thoracic Surgery, Medical University of Vienna, Vienna, Austria. FAU - Picard, Clément AU - Picard C AD - Department of Pneumology, Foch hospital, Suresnes, France. FAU - Dhanani, Zehra Nizar AU - Dhanani ZN AD - Temple University, Lewis Katz School of Medicine, Philadelphia, PA. FAU - Beaumont-Azuar, Laurence AU - Beaumont-Azuar L AD - Department of Pneumology, Foch hospital, Suresnes, France. FAU - de Verdière, Sylvie Colin AU - de Verdière SC AD - Department of Pneumology, Foch hospital, Suresnes, France. FAU - Sage, Édouard AU - Sage É AD - Deparment of thoracic surgery and lung transplantation, Foch hospital, Suresnes, France; UMR 0892 VIM, INRAE/U de Versailles, St-Quentin-en-Yvelines, France. FAU - Messika, Jonathan AU - Messika J AD - Deparment of thoracic surgery and lung transplantation, Foch hospital, Suresnes, France; INSERM UMR 1149, InnaLung, Université Paris Cité, Paris, France. FAU - Bunel, Vincent AU - Bunel V AD - AP-HP, Nord-Université Paris Cité, Service de Pneumologie B et Transplantation Pulmonaire, Hôpital Bichat, Paris, France; INSERM UMR 1152 PHERE, Université Paris Cité, Paris, France. FAU - Tissot, Adrien AU - Tissot A AD - Nantes Université, CHU Nantes, INSERM, Service de Pneumologie, l'institut du thorax, Center for Research in Transplantation and Translational Immunology (CR2TI), UMR 1064, Nantes, France. FAU - Levine, Deborah AU - Levine D AD - Stanford University, Stanford, California. FAU - Le Pavec, Jérôme AU - Le Pavec J AD - Department of thoracic pneumology and oncology, Marie-Lannelongue hospital, Le Plessis-Robinson, France. FAU - Macey, Julie AU - Macey J AD - Department of pneumology, Bordeaux hospital, Bordeaux, France. FAU - Coiffard, Benjamin AU - Coiffard B AD - Deparment of thoracic surgery and lung transplantation, Marseille hospital, Marseille, France. FAU - Villeneuve, Thomas AU - Villeneuve T AD - Department of pneumology, Toulouse hospital, Toulouse, France. FAU - Renaud-Picard, Benjamin AU - Renaud-Picard B AD - Department of pneumology, Strasbourg hospital, Strasbourg, France. FAU - Falque, Loïc AU - Falque L AD - Department of pneumology, Grenoble hospital, Grenoble, France. FAU - Carlier, Nicolas AU - Carlier N AD - APHP, Centre-Université Paris Cité, Service de Pneumologie, Hôpital Cochin, Paris, France. FAU - Merveilleux, Claire AU - Merveilleux C AD - Department of pneumology, Hospices Civils de Lyon, Lyon, France. FAU - Anjum, Fatima AU - Anjum F AD - Temple University, Lewis Katz School of Medicine, Philadelphia, PA. FAU - Magnan, Antoine AU - Magnan A AD - Department of Pneumology, Foch hospital, Suresnes, France; UMR 0892 VIM, INRAE/U de Versailles, St-Quentin-en-Yvelines, France. FAU - Benazzo, Alberto AU - Benazzo A AD - Department of Thoracic Surgery, Medical University of Vienna, Vienna, Austria. FAU - Loupy, Alexandre AU - Loupy A AD - Université de Paris Cité, INSERM, PARCC, Paris Institute for Transplantation and Organ Regeneration, F-75015 Paris, France. FAU - Roux, Antoine AU - Roux A AD - Université de Paris Cité, INSERM, PARCC, Paris Institute for Transplantation and Organ Regeneration, F-75015 Paris, France; Department of Pneumology, Foch hospital, Suresnes, France; UMR 0892 VIM, INRAE/U de Versailles, St-Quentin-en-Yvelines, France. Electronic address: rouxantoine@hotmail.com. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20250629 PL - United States TA - J Heart Lung Transplant JT - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation JID - 9102703 SB - IM MH - Humans MH - *Lung Transplantation MH - Male MH - Female MH - Forced Expiratory Volume/physiology MH - Middle Aged MH - Adult MH - Retrospective Studies MH - Follow-Up Studies MH - Survival Rate/trends MH - Treatment Outcome OTO - NOTNLM OT - FEV1 OT - Lung OT - Outcomes OT - Trajectories OT - Transplantation EDAT- 2025/07/02 00:27 MHDA- 2025/10/25 00:30 CRDT- 2025/07/01 19:10 PHST- 2025/01/30 00:00 [received] PHST- 2025/05/22 00:00 [revised] PHST- 2025/06/03 00:00 [accepted] PHST- 2025/10/25 00:30 [medline] PHST- 2025/07/02 00:27 [pubmed] PHST- 2025/07/01 19:10 [entrez] AID - S1053-2498(25)02036-4 [pii] AID - 10.1016/j.healun.2025.06.011 [doi] PST - ppublish SO - J Heart Lung Transplant. 2025 Nov;44(11):1749-1762. doi: 10.1016/j.healun.2025.06.011. Epub 2025 Jun 29. PMID- 32363949 OWN - NLM STAT- MEDLINE DCOM- 20210823 LR - 20211005 IS - 1524-4539 (Electronic) IS - 0009-7322 (Linking) VI - 141 IP - 24 DP - 2020 Jun 16 TI - Identification and Characterization of Trajectories of Cardiac Allograft Vasculopathy After Heart Transplantation: A Population-Based Study. PG - 1954-1967 LID - 10.1161/CIRCULATIONAHA.119.044924 [doi] AB - BACKGROUND: Cardiac allograft vasculopathy (CAV) is a major contributor of heart transplant recipient mortality. Little is known about the prototypes of CAV trajectories at the population level. We aimed to identify the different evolutionary profiles of CAV and to determine the respective contribution of immune and nonimmune factors in CAV development. METHODS: Heart transplant recipients were from 4 academic centers (Pitié-Salpêtrière and Georges Pompidou Hospital, Paris, Katholieke Universiteit Leuven, and Cedars-Sinai, Los Angeles; 2004-2016). Patients underwent prospective, protocol-based monitoring consisting of repeated coronary angiographies together with systematic assessments of clinical, histological, and immunologic parameters. The main outcome was a prediction for CAV trajectory. We identified CAV trajectories by using unsupervised latent class mixed models. We then identified the independent predictive variables of the CAV trajectories and their association with mortality. RESULTS: A total of 1301 patients were included (815 and 486 in the European and US cohorts, respectively). The median follow-up after transplantation was 6.6 (interquartile range, 4-9.1) years with 4710 coronary angiographies analyzed. We identified 4 distinct profiles of CAV trajectories over 10 years. The 4 trajectories were characterized by (1) patients without CAV at 1 year and nonprogression over time (56.3%), (2) patients without CAV at 1 year and late-onset slow CAV progression (7.6%), (3) patients with mild CAV at 1 year and mild progression over time (23.1%), and (4) patients with mild CAV at 1 year and accelerated progression (13.0%). This model showed good discrimination (0.92). Among candidate predictors assessed, 6 early independent predictors of these trajectories were identified: donor age (P<0.001), donor male sex (P<0.001), donor tobacco consumption (P=0.001), recipient dyslipidemia (P=0.009), class II anti-human leukocyte antigen donor-specific antibodies (P=0.004), and acute cellular rejection ≥2R (P=0.028). The 4 CAV trajectories manifested consistently in the US independent cohort with similar discrimination (0.97) and in different clinical scenarios, and showed gradients for overall-cause mortality (P<0.001). CONCLUSIONS: In a large multicenter and highly phenotyped prospective cohort of heart transplant recipients, we identified 4 CAV trajectories and their respective independent predictive variables. Our results provide the basis for a trajectory-based assessment of patients undergoing heart transplantation for early risk stratification, patient monitoring, and clinical trials. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04117152. FAU - Loupy, Alexandre AU - Loupy A AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Kidney Transplant Department (A.L., O.A.), Necker Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Coutance, Guillaume AU - Coutance G AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Department of Cardiac and Thoracic Surgery, Cardiology Institute, Pitié-Salpêtrière Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Sorbonne University Medical School, France (G.C., S.V., P.L.). FAU - Bonnet, Guillaume AU - Bonnet G AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Cardiology and Heart Transplant Department (G.B., M.-C.B., R.G., J.-P.E., X.J.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Van Keer, Jan AU - Van Keer J AD - Departments of Cardiology (J.V.K.), University Hospitals Leuven, Katholieke Universiteit Leuven, Belgium. FAU - Raynaud, Marc AU - Raynaud M AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). FAU - Aubert, Olivier AU - Aubert O AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Kidney Transplant Department (A.L., O.A.), Necker Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Bories, Marie-Cécile AU - Bories MC AD - Cardiology and Heart Transplant Department (G.B., M.-C.B., R.G., J.-P.E., X.J.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Racapé, Maud AU - Racapé M AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). FAU - Yoo, Daniel AU - Yoo D AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). FAU - Duong Van Huyen, Jean-Paul AU - Duong Van Huyen JP AD - Pathology Department (J.P.D.V.H.), Necker Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Bruneval, Patrick AU - Bruneval P AD - Pathology Department (P.B.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Taupin, Jean-Luc AU - Taupin JL AD - Laboratory of Immunology and Histocompatibility, AP-HP, Saint Louis Hospital, Paris, France (J.-L.T.). FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). FAU - Varnous, Shaida AU - Varnous S AD - Department of Cardiac and Thoracic Surgery, Cardiology Institute, Pitié-Salpêtrière Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Sorbonne University Medical School, France (G.C., S.V., P.L.). AD - INSERM, UMRS 1166-ICAN, Institute of Cardiometabolism and Nutrition, Paris, France (S.V., P.L.). FAU - Leprince, Pascal AU - Leprince P AD - Department of Cardiac and Thoracic Surgery, Cardiology Institute, Pitié-Salpêtrière Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Sorbonne University Medical School, France (G.C., S.V., P.L.). AD - INSERM, UMRS 1166-ICAN, Institute of Cardiometabolism and Nutrition, Paris, France (S.V., P.L.). FAU - Guillemain, Romain AU - Guillemain R AD - Cardiology and Heart Transplant Department (G.B., M.-C.B., R.G., J.-P.E., X.J.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Empana, Jean-Philippe AU - Empana JP AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Cardiology and Heart Transplant Department (G.B., M.-C.B., R.G., J.-P.E., X.J.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Levine, Ryan AU - Levine R AD - Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA (R.L., J.K.P., J.K.). FAU - Naesens, Maarten AU - Naesens M AD - Microbiology, Immunology, and Transplantation and of Nephrology(M.N.), University Hospitals Leuven, Katholieke Universiteit Leuven, Belgium. AD - Renal Transplantation (M.N.), University Hospitals Leuven, Katholieke Universiteit Leuven, Belgium. FAU - Patel, Jigneh K AU - Patel JK AD - Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA (R.L., J.K.P., J.K.). FAU - Jouven, Xavier AU - Jouven X AD - Université de Paris, INSERM, Paris Translational Research Centre for Organ Transplantation, France (A.L., G.C., G.B., M. Raynaud, O.A., M. Racapé, D.Y., C.L., J.-P.E., X.J.). AD - Cardiology and Heart Transplant Department (G.B., M.-C.B., R.G., J.-P.E., X.J.), Georges Pompidou Hospital, Assistance Publique-Hôpitaux de Paris, France. FAU - Kobashigawa, Jon AU - Kobashigawa J AD - Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA (R.L., J.K.P., J.K.). LA - eng SI - ClinicalTrials.gov/NCT04117152 PT - Clinical Trial PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20200504 PL - United States TA - Circulation JT - Circulation JID - 0147763 SB - IM CIN - Circulation. 2020 Jun 16;141(24):1968-1970. doi: 10.1161/CIRCULATIONAHA.120.047571. PMID: 32539613 CIN - Circulation. 2020 Dec 8;142(23):e409-e410. doi: 10.1161/CIRCULATIONAHA.120.050849. PMID: 33284650 CIN - Circulation. 2020 Dec 8;142(23):e407-e408. doi: 10.1161/CIRCULATIONAHA.120.049661. PMID: 33284654 MH - Adult MH - Allografts MH - Belgium/epidemiology MH - Cardiovascular Diseases/diagnosis/*epidemiology/physiopathology/*surgery MH - Cohort Studies MH - Female MH - Follow-Up Studies MH - Graft Rejection/diagnosis/*epidemiology/physiopathology MH - Heart Transplantation/adverse effects/*trends MH - Humans MH - Los Angeles/epidemiology MH - Male MH - Middle Aged MH - Paris/epidemiology MH - *Population Surveillance/methods MH - Postoperative Complications/diagnosis/*epidemiology/physiopathology MH - Transplantation, Homologous/adverse effects/trends MH - Young Adult OTO - NOTNLM OT - allografts OT - antibodies OT - coronary artery disease OT - graft rejection OT - heart transplantation OT - latent class analysis EDAT- 2020/05/05 06:00 MHDA- 2021/08/24 06:00 CRDT- 2020/05/05 06:00 PHST- 2020/05/05 06:00 [pubmed] PHST- 2021/08/24 06:00 [medline] PHST- 2020/05/05 06:00 [entrez] AID - 10.1161/CIRCULATIONAHA.119.044924 [doi] PST - ppublish SO - Circulation. 2020 Jun 16;141(24):1954-1967. doi: 10.1161/CIRCULATIONAHA.119.044924. Epub 2020 May 4. PMID- 18258820 OWN - NLM STAT- MEDLINE DCOM- 20080317 LR - 20151119 IS - 1524-4636 (Electronic) IS - 1079-5642 (Linking) VI - 28 IP - 3 DP - 2008 Mar TI - Evaluation of matrix metalloproteinases in atherosclerosis using a novel noninvasive imaging approach. PG - 425-32 LID - 10.1161/ATVBAHA.107.149666 [doi] AB - OBJECTIVE: Despite great advances in our knowledge, atherosclerosis continues to kill more people than any other disease in the Western world. This is because our means of identifying truly vulnerable patients is limited. Prediction of atherosclerotic plaque rupture may be addressed by MRI of activated matrix metalloproteinases (MMPs), a family of enzymes that have been implicated in the vulnerability of plaques prone to rupture. This study evaluated the ability of the novel gadolinium-based MRI contrast agent P947 to target MMPs in atherosclerotic plaques. METHODS AND RESULTS: The affinity of P947 toward activated MMPs was demonstrated in vitro. The affinity and specificity of P947 toward matrix metalloproteinase (MMP)-rich plaques was evaluated both in vivo using ApoE-/- mice and ex vivo in hyperlipidemic rabbits. Gadolinium content quantification and MRI showed a preferential accumulation of P947 in atherosclerotic lesions compared with the nontargeted reference compound, Gd-DOTA. The ex vivo assay on rabbit plaques revealed a higher uptake of P947. Moreover, using human carotid artery endarterectomy specimens, P947 facilitated discrimination between histologically defined MMP-rich and MMP-poor plaques. An in vivo MRI investigation in mice revealed that P947 greatly improved the ability to visualize and delineate atherosclerotic plaques. CONCLUSIONS: P947 may be a useful tool for the detection and characterization of the MMP-rich atherosclerotic plaques. FAU - Lancelot, Eric AU - Lancelot E AD - Department of Radiology, the Zena and Michael A. Wiener Cardiovascular Institute, Mount Sinai School of Medicine, Box 1234, One Gustave L. Levy Place, New York, NY 10029, USA. FAU - Amirbekian, Vardan AU - Amirbekian V FAU - Brigger, Irène AU - Brigger I FAU - Raynaud, Jean-Sébastien AU - Raynaud JS FAU - Ballet, Sébastien AU - Ballet S FAU - David, Christelle AU - David C FAU - Rousseaux, Olivier AU - Rousseaux O FAU - Le Greneur, Soizic AU - Le Greneur S FAU - Port, Marc AU - Port M FAU - Lijnen, Henri R AU - Lijnen HR FAU - Bruneval, Patrick AU - Bruneval P FAU - Michel, Jean-Baptiste AU - Michel JB FAU - Ouimet, Tanja AU - Ouimet T FAU - Roques, Bernard AU - Roques B FAU - Amirbekian, Smbat AU - Amirbekian S FAU - Hyafil, Fabien AU - Hyafil F FAU - Vucic, Esad AU - Vucic E FAU - Aguinaldo, Juan Gilberto S AU - Aguinaldo JG FAU - Corot, Claire AU - Corot C FAU - Fayad, Zahi A AU - Fayad ZA LA - eng GR - HL78667/HL/NHLBI NIH HHS/United States GR - R01 HL71021/HL/NHLBI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20080207 PL - United States TA - Arterioscler Thromb Vasc Biol JT - Arteriosclerosis, thrombosis, and vascular biology JID - 9505803 RN - 0 (Apolipoproteins E) RN - 0 (Biomarkers) RN - 0 (Contrast Media) RN - AU0V1LM3JT (Gadolinium) RN - EC 3.4.24.- (Matrix Metalloproteinases) SB - IM MH - Animals MH - Aorta, Thoracic/metabolism/*pathology MH - Apolipoproteins E/pharmacology MH - Atherosclerosis/*diagnosis MH - Biomarkers/analysis MH - Carotid Arteries/metabolism/*pathology MH - Contrast Media MH - Disease Models, Animal MH - Gadolinium MH - Humans MH - In Vitro Techniques MH - Magnetic Resonance Imaging/*methods MH - Matrix Metalloproteinases/analysis/*metabolism MH - Mice MH - Mice, Inbred C57BL MH - Probability MH - Rabbits MH - Sensitivity and Specificity MH - Statistics, Nonparametric EDAT- 2008/02/09 09:00 MHDA- 2008/03/18 09:00 CRDT- 2008/02/09 09:00 PHST- 2008/02/09 09:00 [pubmed] PHST- 2008/03/18 09:00 [medline] PHST- 2008/02/09 09:00 [entrez] AID - ATVBAHA.107.149666 [pii] AID - 10.1161/ATVBAHA.107.149666 [doi] PST - ppublish SO - Arterioscler Thromb Vasc Biol. 2008 Mar;28(3):425-32. doi: 10.1161/ATVBAHA.107.149666. Epub 2008 Feb 7. PMID- 33887439 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20250530 IS - 1464-3405 (Electronic) IS - 0960-894X (Print) IS - 0960-894X (Linking) VI - 42 DP - 2021 Jun 15 TI - Investigating the phosphinic acid tripeptide mimetic DG013A as a tool compound inhibitor of the M1-aminopeptidase ERAP1. PG - 128050 LID - S0960-894X(21)00276-6 [pii] LID - 10.1016/j.bmcl.2021.128050 [doi] LID - 128050 AB - ERAP1 is a zinc-dependent M1-aminopeptidase that trims lipophilic amino acids from the N-terminus of peptides. Owing to its importance in the processing of antigens and regulation of the adaptive immune response, dysregulation of the highly polymorphic ERAP1 has been implicated in autoimmune disease and cancer. To test this hypothesis and establish the role of ERAP1 in these disease areas, high affinity, cell permeable and selective chemical probes are essential. DG013A 1, is a phosphinic acid tripeptide mimetic inhibitor with reported low nanomolar affinity for ERAP1. However, this chemotype is a privileged structure for binding to various metal-dependent peptidases and contains a highly charged phosphinic acid moiety, so it was unclear whether it would display the high selectivity and passive permeability required for a chemical probe. Therefore, we designed a new stereoselective route to synthesize a library of DG013A 1 analogues to determine the suitability of this compound as a cellular chemical probe to validate ERAP1 as a drug discovery target. CI - Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Wilding, Birgit AU - Wilding B AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Pasqua, A Elisa AU - Pasqua AE AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - E A Chessum, Nicola AU - E A Chessum N AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Pierrat, Olivier A AU - Pierrat OA AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Hahner, Tamas AU - Hahner T AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Tomlin, Kathy AU - Tomlin K AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Shehu, Erald AU - Shehu E AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Burke, Rosemary AU - Burke R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Richards, G Meirion AU - Richards GM AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Whitton, Bradleigh AU - Whitton B AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Arwert, Esther N AU - Arwert EN AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Thapaliya, Arjun AU - Thapaliya A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK; Division of Structural Biology, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Salimraj, Ramya AU - Salimraj R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK; Division of Structural Biology, The Institute of Cancer Research, London SW7 3RP, UK. FAU - van Montfort, Rob AU - van Montfort R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK; Division of Structural Biology, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Skawinska, Agi AU - Skawinska A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Hayes, Angela AU - Hayes A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Raynaud, Florence AU - Raynaud F AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Chopra, Rajesh AU - Chopra R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Jones, Keith AU - Jones K AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Newton, Gary AU - Newton G AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. FAU - Cheeseman, Matthew D AU - Cheeseman MD AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London SW7 3RP, UK. Electronic address: Matthew.Cheeseman@icr.ac.uk. LA - eng GR - 22897/CRUK_/Cancer Research UK/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - C2739/A22897/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210420 PL - England TA - Bioorg Med Chem Lett JT - Bioorganic & medicinal chemistry letters JID - 9107377 RN - 0 (Enzyme Inhibitors) RN - 0 (Minor Histocompatibility Antigens) RN - 0 (Oligopeptides) RN - 0 (Phosphinic Acids) RN - EC 3.4.11.- (Aminopeptidases) RN - EC 3.4.11.- (ERAP1 protein, human) SB - IM MH - Aminopeptidases/*antagonists & inhibitors/metabolism MH - Dose-Response Relationship, Drug MH - Enzyme Inhibitors/chemical synthesis/chemistry/*pharmacology MH - Humans MH - Minor Histocompatibility Antigens/metabolism MH - Models, Molecular MH - Molecular Structure MH - Oligopeptides/chemical synthesis/chemistry/*pharmacology MH - Phosphinic Acids/chemical synthesis/chemistry/*pharmacology MH - Structure-Activity Relationship PMC - PMC8188423 OTO - NOTNLM OT - Chemical probe OT - DG013A OT - ERAP1 OT - M1-aminopeptidase OT - Permeability COIS- The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: The authors are employees of ICR and ICR has a commercial interest in inhibitors of ERAP1. The ICR operates a Rewards to Discoverers scheme, which provides financial rewards to inventors in the event of commercial licensing. EDAT- 2021/04/23 06:00 MHDA- 2021/11/03 06:00 PMCR- 2021/06/15 CRDT- 2021/04/22 20:15 PHST- 2021/02/19 00:00 [received] PHST- 2021/03/26 00:00 [revised] PHST- 2021/04/13 00:00 [accepted] PHST- 2021/04/23 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] PHST- 2021/04/22 20:15 [entrez] PHST- 2021/06/15 00:00 [pmc-release] AID - S0960-894X(21)00276-6 [pii] AID - 128050 [pii] AID - 10.1016/j.bmcl.2021.128050 [doi] PST - ppublish SO - Bioorg Med Chem Lett. 2021 Jun 15;42:128050. doi: 10.1016/j.bmcl.2021.128050. Epub 2021 Apr 20. PMID- 30293206 OWN - NLM STAT- MEDLINE DCOM- 20200407 LR - 20230722 IS - 1868-8500 (Electronic) IS - 1868-8497 (Print) IS - 1868-8497 (Linking) VI - 10 IP - 1 DP - 2019 Feb TI - Aggressiveness of Localized Prostate Cancer: the Key Value of Testosterone Deficiency Evaluated by Both Total and Bioavailable Testosterone: AndroCan Study Results. PG - 36-44 LID - 10.1007/s12672-018-0351-8 [doi] AB - Failure rates after first-line treatment of localized prostate cancer (PCa) treatment remain high. Improvements to patient selection and identification of at-risk patients are central to reducing mortality. We aimed to determine if cancer aggressiveness correlates with androgen levels in patients undergoing radical prostatectomy for localized PCa. We performed a prospective, multicenter cohort study between June 2013 and June 2016, involving men with localized PCa scheduled to undergo radical prostatectomy. Clinical and hormonal patient data (testosterone deficiency, defined by total testosterone (TT) levels < 300 ng/dL and/or bioavailable testosterone (BT) levels < 80 ng/dL) were prospectively collected, along with pathological assessment of preoperative biopsy and subsequent radical prostatectomy specimens, using predominant Gleason pattern (prdGP) 3/4 grading. Of 1343 patients analyzed, 912 (68%) had prdGP3 PCa and 431 (32%) had high-grade (prdGP4, i.e., ISUP ≥ 3) disease on prostatectomy specimens. Only moderate concordance in prdGP scores between prostate biopsies and prostatectomy specimens was found. Compared with patients with prdGP3 tumors (i.e., ISUP ≤ 2), significantly more patients with prdGP4 cancers had demonstrable hypogonadism, characterized either by BT levels (17.4% vs. 10.7%, p < 0.001) or TT levels (14.2% vs. 9.7%, p = 0.020). BT levels were also lower in patients with prdGP4 tumors compared to those with prdGP3 disease. Testosterone deficiency (defined by TT and/or BT levels) was independently associated with higher PCa aggressiveness. BT is a predictive factor for prdGP4 disease, and evaluating both TT and BT to define hypogonadism is valuable in preoperative assessment of PCa (AndroCan Trial: NCT02235142). FAU - Neuzillet, Yann AU - Neuzillet Y AD - Department of Urology, Hôpital Foch, University of Versailles-Saint-Quentin-en-Yvelines, 40 Rue Worth, 92150, Suresnes, France. y.neuzillet@hopital-foch.org. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Sorbonne University, Paris, France. FAU - Dreyfus, Jean-François AU - Dreyfus JF AD - Department of Clinical Research and Innovation, Foch Hospital, University of Versailles-Saint-Quentin-en-Yvelines, Suresnes, France. FAU - Radulescu, Camélia AU - Radulescu C AD - Department of Pathology, Foch Hospital, Suresnes, France. FAU - Rouanne, Mathieu AU - Rouanne M AD - Department of Urology, Hôpital Foch, University of Versailles-Saint-Quentin-en-Yvelines, 40 Rue Worth, 92150, Suresnes, France. FAU - Schneider, Marc AU - Schneider M AD - Department of Urology, Louis Pasteur Hospital, Colmar, France. FAU - Krish, Sylvie AU - Krish S AD - Department of Pathology, Louis Pasteur Hospital, Colmar, France. FAU - Rouprêt, Morgan AU - Rouprêt M AD - Department of Urology, Pitié Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Drouin, Sarah J AU - Drouin SJ AD - Department of Urology, Pitié Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Comperat, Eva AU - Comperat E AD - Department of Pathology, Tenon Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Galiano, Marc AU - Galiano M AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Cathelineau, Xavier AU - Cathelineau X AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Validire, Pierre AU - Validire P AD - Department of Pathology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Molinié, Vincent AU - Molinié V AD - Department of Pathology, Centre Hospitalier de Martinique, Le Lamentin, France. FAU - Fiet, Jean AU - Fiet J AD - Inserm U955, Eq07, Recherches Translationnelles en oncogenèse génitale, Créteil, France. FAU - Giton, Franck AU - Giton F AD - Inserm U955, Eq07, Recherches Translationnelles en oncogenèse génitale, Créteil, France. FAU - Lebret, Thierry AU - Lebret T AD - Department of Urology, Hôpital Foch, University of Versailles-Saint-Quentin-en-Yvelines, 40 Rue Worth, 92150, Suresnes, France. FAU - Botto, Henry AU - Botto H AD - Department of Urology, Hôpital Foch, University of Versailles-Saint-Quentin-en-Yvelines, 40 Rue Worth, 92150, Suresnes, France. LA - eng SI - ClinicalTrials.gov/NCT02235142 PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20181006 PL - United States TA - Horm Cancer JT - Hormones & cancer JID - 101518427 RN - 0 (Androgens) RN - 3XMK78S47O (Testosterone) RN - EC 3.4.21.77 (Prostate-Specific Antigen) SB - IM MH - Aged MH - Androgens/metabolism MH - Biopsy MH - France MH - Humans MH - Male MH - Middle Aged MH - Neoplasm Grading MH - Neoplasm Invasiveness MH - Prospective Studies MH - Prostate/pathology MH - Prostate-Specific Antigen/blood MH - Prostatectomy/methods MH - Prostatic Neoplasms/blood/*drug therapy MH - Risk Factors MH - Testosterone/*blood/*deficiency PMC - PMC10355706 OTO - NOTNLM OT - Androgens OT - Gleason OT - Hypogonadism OT - ISUP OT - Prostate cancer OT - Testosterone EDAT- 2018/10/08 06:00 MHDA- 2020/04/09 06:00 PMCR- 2018/10/06 CRDT- 2018/10/08 06:00 PHST- 2018/07/04 00:00 [received] PHST- 2018/09/28 00:00 [accepted] PHST- 2018/10/08 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2018/10/08 06:00 [entrez] PHST- 2018/10/06 00:00 [pmc-release] AID - 10.1007/s12672-018-0351-8 [pii] AID - 351 [pii] AID - 10.1007/s12672-018-0351-8 [doi] PST - ppublish SO - Horm Cancer. 2019 Feb;10(1):36-44. doi: 10.1007/s12672-018-0351-8. Epub 2018 Oct 6. PMID- 40526091 OWN - NLM STAT- MEDLINE DCOM- 20250804 LR - 20250806 IS - 2211-5463 (Electronic) IS - 2211-5463 (Linking) VI - 15 IP - 8 DP - 2025 Aug TI - Gut alterations in a chronic kidney disease rat model with diet-induced vascular calcification. PG - 1219-1231 LID - 10.1002/2211-5463.70043 [doi] AB - Intestinal disorders and vascular calcification (VC) are often associated with chronic kidney disease (CKD). While gut barrier alterations have been reported in CKD (such as abnormal intestinal permeability, bacterial overgrowth, and inflammation), it is not clear if vascular calcification influences these alterations. To investigate whether the bidirectional relationships between VC and gut dysfunction could be mediated by increased inflammation and uremic toxin generation, we used the SNx-VC model of uremic vascular calcification (rats undergoing subtotal 5/6th nephrectomy and fed a procalcifying high-phosphate and vitamin D diet). We confirmed the presence of CKD and VC by von Kossa staining and observed increased gut-origin uremic toxin, indoxyl sulfate (IS), in SNx-VC animals compared to controls. In SNx-VC rats, we observed decreased mucus production (Alcian blue, Mucin 2 staining) in the colon and ileum which was correlated with the level of calcification. There was no change in inflammation markers or tight junction protein expression. We assessed intestinal levels in the NOD-like receptor family pyrin domain containing 6 (NLRP6) protein, known to regulate mucus secretion, and found no change in the colon or ileum. Nlrp6 mRNA was, however, decreased in the colon of SNx-VC rats, along with other mRNA (Ly96, Sod1), while Tlr2 was increased compared to controls. Our observations of low mucus, low Nlrp6 mRNA, and high IS in SNx-VC rats confirm a link between gut barrier alterations and uremic VC. This suggests that alterations in the mucus layer could favor the generation of gut-origin uremic toxins and promote VC in CKD. Thus, improving the gut mucus barrier function in the context of uremic VC could be considered as a possible therapeutic strategy in CKD patients. CI - © 2025 The Author(s). FEBS Open Bio published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies. FAU - Bartochowski, Piotr AU - Bartochowski P AD - RD Néphrologie SAS, Montpellier, France. AD - BC2M, University of Montpellier, France. FAU - Cortijo, Irene AU - Cortijo I AD - RD Néphrologie SAS, Montpellier, France. FAU - Bhargava, Shruti AU - Bhargava S AD - Institute of Molecular Cardiovascular Research, Medical Faculty, RWTH Aachen University, Germany. FAU - Jover, Bernard AU - Jover B AD - RD Néphrologie SAS, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - PhyMedExp, INSERM, CNRS, University of Montpellier, France. FAU - Boukhaled, Juliana H AU - Boukhaled JH AD - RD Néphrologie SAS, Montpellier, France. AD - Université Paul Sabatier, Toulouse, France. FAU - Lajoix, Anne-Dominique AU - Lajoix AD AD - BC2M, University of Montpellier, France. FAU - Jankowski, Vera AU - Jankowski V AD - Institute of Molecular Cardiovascular Research, Medical Faculty, RWTH Aachen University, Germany. FAU - Jankowski, Joachim AU - Jankowski J AD - Institute of Molecular Cardiovascular Research, Medical Faculty, RWTH Aachen University, Germany. FAU - Cordaillat-Simmons, Magali AU - Cordaillat-Simmons M AD - Pharmabiotic Research Institute, European Microbiome Regulatory Science Center, Narbonne, France. AD - UMRF, Université Clermont Auvergne, Aurillac, France. FAU - Argilés, Àngel AU - Argilés À AD - RD Néphrologie SAS, Montpellier, France. AD - BC2M, University of Montpellier, France. FAU - Gayrard, Nathalie AU - Gayrard N AD - RD Néphrologie SAS, Montpellier, France. AD - BC2M, University of Montpellier, France. FAU - Duranton, Flore AU - Duranton F AUID- ORCID: 0000-0003-3202-0551 AD - RD Néphrologie SAS, Montpellier, France. AD - BC2M, University of Montpellier, France. FAU - Laget, Jonas AU - Laget J AUID- ORCID: 0000-0002-9095-7409 AD - RD Néphrologie SAS, Montpellier, France. AD - BC2M, University of Montpellier, France. LA - eng GR - MSCA-DN ID-2021-101072828/H2020 Marie Skłodowska-Curie Actions/ GR - MSCA-ITN-2019-860329/H2020 Marie Skłodowska-Curie Actions/ GR - ANR-21-CE18-0031/Agence Nationale de la Recherche/ PT - Journal Article DEP - 20250617 PL - England TA - FEBS Open Bio JT - FEBS open bio JID - 101580716 RN - N187WK1Y1J (Indican) SB - IM MH - Animals MH - *Renal Insufficiency, Chronic/metabolism/physiopathology MH - Rats MH - Disease Models, Animal MH - *Vascular Calcification/metabolism/etiology/pathology MH - Male MH - Diet/adverse effects MH - Indican/metabolism MH - Intestinal Mucosa/metabolism MH - Uremia MH - Rats, Sprague-Dawley MH - Inflammation/metabolism PMC - PMC12319703 OTO - NOTNLM OT - NLRP6 OT - chronic kidney disease OT - gut OT - mucus OT - vascular calcification COIS- The authors declare no conflict of interest. EDAT- 2025/06/17 14:36 MHDA- 2025/08/04 12:27 PMCR- 2025/06/17 CRDT- 2025/06/17 10:43 PHST- 2025/03/24 00:00 [revised] PHST- 2025/01/06 00:00 [received] PHST- 2025/04/11 00:00 [accepted] PHST- 2025/08/04 12:27 [medline] PHST- 2025/06/17 14:36 [pubmed] PHST- 2025/06/17 10:43 [entrez] PHST- 2025/06/17 00:00 [pmc-release] AID - FEB470043 [pii] AID - 10.1002/2211-5463.70043 [doi] PST - ppublish SO - FEBS Open Bio. 2025 Aug;15(8):1219-1231. doi: 10.1002/2211-5463.70043. Epub 2025 Jun 17. PMID- 37257905 OWN - NLM STAT- MEDLINE DCOM- 20230602 LR - 20240808 IS - 1756-1833 (Electronic) IS - 0959-8138 (Print) IS - 0959-8138 (Linking) VI - 381 DP - 2023 May 31 TI - Race-free estimated glomerular filtration rate equation in kidney transplant recipients: development and validation study. PG - e073654 LID - 10.1136/bmj-2022-073654 [doi] LID - e073654 AB - OBJECTIVE: To compare the performance of a newly developed race-free kidney recipient specific glomerular filtration rate (GFR) equation with the three current main equations for measuring GFR in kidney transplant recipients. DESIGN: Development and validation study SETTING: 17 cohorts in Europe, the United States, and Australia (14 transplant centres, three clinical trials). PARTICIPANTS: 15 489 adults (3622 in development cohort (Necker, Saint Louis, and Toulouse hospitals, France), 11 867 in multiple external validation cohorts) who received kidney transplants between 1 January 2000 and 1 January 2021. MAIN OUTCOME MEASURE: The main outcome measure was GFR, measured according to local practice. Performance of the GFR equations was assessed using P(30) (proportion of estimated GFR (eGFR) within 30% of measured GFR (mGFR)) and correct classification (agreement between eGFR and mGFR according to GFR stages). The race-free equation, based on creatinine level, age, and sex, was developed using additive and multiplicative linear regressions, and its performance was compared with the three current main GFR equations: Modification of Diet in Renal Disease (MDRD) equation, Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) 2009 equation, and race-free CKD-EPI 2021 equation. RESULTS: The study included 15 489 participants, with 50 464 mGFR and eGFR values. The mean GFR was 53.18 mL/min/1.73m(2) (SD 17.23) in the development cohort and 55.90 mL/min/1.73m(2) (19.69) in the external validation cohorts. Among the current GFR equations, the race-free CKD-EPI 2021 equation showed the lowest performance compared with the MDRD and CKD-EPI 2009 equations. When race was included in the kidney recipient specific GFR equation, performance did not increase. The race-free kidney recipient specific GFR equation showed significantly improved performance compared with the race-free CKD-EPI 2021 equation and performed well in the external validation cohorts (P(30) ranging from 73.0% to 91.3%). The race-free kidney recipient specific GFR equation performed well in several subpopulations of kidney transplant recipients stratified by race (P(30) 73.0-91.3%), sex (72.7-91.4%), age (70.3-92.0%), body mass index (64.5-100%), donor type (58.5-92.9%), donor age (68.3-94.3%), treatment (78.5-85.2%), creatinine level (72.8-91.3%), GFR measurement method (73.0-91.3%), and timing of GFR measurement post-transplant (72.9-95.5%). An online application was developed that estimates GFR based on recipient's creatinine level, age, and sex (https://transplant-prediction-system.shinyapps.io/eGFR_equation_KTX/). CONCLUSION: A new race-free kidney recipient specific GFR equation was developed and validated using multiple, large, international cohorts of kidney transplant recipients. The equation showed high accuracy and outperformed the race-free CKD-EPI 2021 equation that was developed in individuals with native kidneys. TRIAL REGISTRATION: ClinicalTrials.gov NCT05229939. CI - © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Raynaud, Marc AU - Raynaud M AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France. FAU - Al-Awadhi, Solaf AU - Al-Awadhi S AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France. FAU - Juric, Ivana AU - Juric I AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Divard, Gillian AU - Divard G AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France. FAU - Lombardi, Yannis AU - Lombardi Y AD - Department of Nephrology and Acute Kidney Intensive Care, Tenon Hospital, Paris, France. FAU - Basic-Jukic, Nikolina AU - Basic-Jukic N AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Aubert, Olivier AU - Aubert O AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France. AD - Department of Nephrology and Kidney Transplantation, Necker Hospital, Paris, France. FAU - Dubourg, Laurence AU - Dubourg L AD - Centre de Référence des Maladies Rénales Rares, Service de Néphrologie et Rhumatologie Pédiatriques, Hospices Civils de Lyon, Lyon, France. FAU - Masson, Ingrid AU - Masson I AD - Department of Nephrology, Dialysis and Renal Transplantation, Nord Hospital, Jean Monnet University, Saint-Etienne, France. FAU - Mariat, Christophe AU - Mariat C AD - Department of Nephrology, Dialysis and Renal Transplantation, Nord Hospital, Jean Monnet University, Saint-Etienne, France. FAU - Prié, Dominique AU - Prié D AD - Department of Nephrology and Kidney Transplantation, Necker Hospital, Paris, France. FAU - Pernin, Vincent AU - Pernin V AD - Department of Nephrology, University Hospital Centre, Montpellier, France. FAU - Le Quintrec, Moglie AU - Le Quintrec M AD - Department of Nephrology, University Hospital Centre, Montpellier, France. FAU - Larson, Timothy S AU - Larson TS AD - William J von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, Minnesota, USA. FAU - Stegall, Mark D AU - Stegall MD AD - William J von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, Minnesota, USA. FAU - Bikbov, Boris AU - Bikbov B AD - Department of Health Policy, Instituto di Ricerche Farmacologiche Mario Negri IRCCS, Milan, Italy. FAU - Ruggenenti, Piero AU - Ruggenenti P AD - Department of Renal Medicine, Clinical Research Centre for Rare Diseases "Aldo e Cele Daccò": Instituto di Ricerche Farmacologiche Mario Negri IRCCS, Ranica, Bergamo, Italy. AD - Unit of Nephrology and Dialysis, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy. FAU - Mesnard, Laurent AU - Mesnard L AD - Department of Nephrology and Acute Kidney Intensive Care, Tenon Hospital, Paris, France. FAU - Ibrahim, Hassan N AU - Ibrahim HN AD - University of Texas Health Sciences Centre at Houston, Texas, USA. FAU - Nielsen, Marie Bodilsen AU - Nielsen MB AD - Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark. FAU - Matas, Arthur J AU - Matas AJ AD - Division of Transplantation, Department of Surgery, University of Minnesota, Minneapolis, Minnesota, USA. FAU - Nankivell, Brian J AU - Nankivell BJ AD - Westmead Hospital Transplant Unit, Sydney, Australia. FAU - Benjamens, Stan AU - Benjamens S AD - Department of Surgery, University of Groningen and University Medical Centre Groningen, Groningen, Netherlands. FAU - Pol, Robert A AU - Pol RA AD - Department of Surgery, University of Groningen and University Medical Centre Groningen, Groningen, Netherlands. FAU - Bakker, Stephan J L AU - Bakker SJL AD - Division of Nephrology, Department of Internal Medicine, University of Groningen and University Medical Centre Groningen, Groningen, Netherlands. FAU - Jouven, Xavier AU - Jouven X AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France. FAU - Legendre, Christophe AU - Legendre C AD - Department of Nephrology and Kidney Transplantation, Necker Hospital, Paris, France. FAU - Kamar, Nassim AU - Kamar N AD - Department of Nephrology and Organ Transplantation, Paul Sabatier University, INSERM, Toulouse, France. FAU - Smith, Byron H AU - Smith BH AD - Division of Nephrology and Hypertension, Mayo Clinic, Jacksonville, Florida, USA. FAU - Wadei, Hani M AU - Wadei HM AD - Division of Nephrology and Hypertension, Mayo Clinic, Jacksonville, Florida, USA. FAU - Durrbach, Antoine AU - Durrbach A AD - Department of Nephrology and Renal Transplantation, Henri-Mondor Hospital, Paris-Saclay University, Creteil, France. FAU - Vincenti, Flavio AU - Vincenti F AD - Department of Surgery, Kidney Transplant Service, University of California San Francisco, San Francisco, California, USA. FAU - Remuzzi, Giuseppe AU - Remuzzi G AD - Department of Renal Medicine, Clinical Research Centre for Rare Diseases "Aldo e Cele Daccò": Instituto di Ricerche Farmacologiche Mario Negri IRCCS, Ranica, Bergamo, Italy. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Department of Kidney Transplantation, Saint Louis University Hospital, Paris, France. FAU - Bentall, Andrew J AU - Bentall AJ AD - William J von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, Minnesota, USA. FAU - Loupy, Alexandre AU - Loupy A AUID- ORCID: 0000-0003-3388-7747 AD - Université de Paris Cité, INSERM, PARCC, Paris Translational Research Centre for Organ Transplantation, F-75015 Paris, France alexandre.loupy@inserm.fr. AD - Department of Nephrology and Kidney Transplantation, Necker Hospital, Paris, France. LA - eng SI - ClinicalTrials.gov/NCT05229939 PT - Journal Article DEP - 20230531 PL - England TA - BMJ JT - BMJ (Clinical research ed.) JID - 8900488 RN - AYI8EX34EU (Creatinine) SB - IM CIN - Am J Kidney Dis. 2024 Feb;83(2):257-259. doi: 10.1053/j.ajkd.2023.08.020. PMID: 37844726 MH - Adult MH - Humans MH - Glomerular Filtration Rate MH - *Kidney Transplantation MH - Creatinine MH - Kidney MH - *Renal Insufficiency, Chronic/diagnosis/surgery/epidemiology PMC - PMC10231444 COIS- Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf and declare: support from the French government through the National Research Agency and the European Union’s Horizon Europe programme; AL holds shares in Cibiltech, a company that develops software; No other relationships or activities that could appear to have influenced the submitted work. EDAT- 2023/06/01 01:08 MHDA- 2023/06/02 06:42 PMCR- 2023/05/31 CRDT- 2023/05/31 20:43 PHST- 2023/06/02 06:42 [medline] PHST- 2023/06/01 01:08 [pubmed] PHST- 2023/05/31 20:43 [entrez] PHST- 2023/05/31 00:00 [pmc-release] AID - bmj-2022-073654.R2 [pii] AID - raym073654 [pii] AID - 10.1136/bmj-2022-073654 [doi] PST - epublish SO - BMJ. 2023 May 31;381:e073654. doi: 10.1136/bmj-2022-073654. PMID- 32533309 OWN - NLM STAT- MEDLINE DCOM- 20210405 LR - 20210405 IS - 1432-086X (Electronic) IS - 0174-1551 (Linking) VI - 43 IP - 11 DP - 2020 Nov TI - Factors Associated with Tumor Progression After Percutaneous Ablation of Hepatocellular Carcinoma: Comparison Between Monopolar Radiofrequency and Microwaves. Results of a Propensity Score Matching Analysis. PG - 1608-1618 LID - 10.1007/s00270-020-02549-8 [doi] AB - PURPOSE: To identify risk factors for local and distant intrahepatic tumor progression after percutaneous ablation of HCC and to compare MWA with monopolar RFA. MATERIALS AND METHODS: Consecutive patients with early or very early HCC who underwent percutaneous monopolar RFA or MWA were included. Factors associated with local and distant tumor progression were identified. Propensity score matching (PSM) was used to limit bias. Statistical analyses were performed with the Kaplan-Meier method using the log-rank test and Cox regression models. RESULTS: One hundred ninety HCC (mean diameter 23 ± 8.6 mm) were treated by RFA (n = 90, 47%) or MWA (n = 100, 53%) in 152 patients (mean age 63 ± 11, 79% men) between 2009 and 2016. The technical success rate was 97.4% (n = 185 HCC). After a median follow-up of 24.6 months (IQR: 9.7-37.2), 43 (23%), HCC showed local tumor progression [after a median of 13.4 months (IQR: 5.8-24.3)] and 91 (63%) patients had distant intrahepatic tumor progression (after a median of 10.4 months (IQR: 5.7-22). The cox model after PSM identified treatment by RFA (HR, 2.89; P = 0.005), HCC size ≥ 30 mm (HR, 3.12; P = 0.007) and vascular contact (HR, 3.43; P = 0.005) as risk factors for local progression. Factors associated with distant intrahepatic progression were HCC ≥ 30 mm (HR, 1.94; P = 0.013), serum AFP > 100 ng/mL (HR, 2.56; p = 0.002), and hepatitis B carrier (HR, 0.51; p = 0.047). CONCLUSION: The rate of local HCC progression was lower after MWA than monopolar RFA, regardless of tumor size and vascular contact. The ablation technique did not influence the risk of distant intrahepatic tumor progression. FAU - Bouda, Damien AU - Bouda D AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Barrau, Vincent AU - Barrau V AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. AD - University Paris Diderot, Sorbonne Paris Cité, Paris, France. FAU - Dioguardi Burgio, Marco AU - Dioguardi Burgio M AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Paulatto, Luisa AU - Paulatto L AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Roche, Vincent AU - Roche V AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Sibert, Annie AU - Sibert A AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. AD - University Paris Diderot, Sorbonne Paris Cité, Paris, France. AD - INSERM U1149, Centre de Recherche biomédicale Bichat-Beaujon, CRB3, Paris, France. FAU - Moussa, Nadia AU - Moussa N AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. FAU - Vilgrain, Valérie AU - Vilgrain V AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. AD - University Paris Diderot, Sorbonne Paris Cité, Paris, France. AD - INSERM U1149, Centre de Recherche biomédicale Bichat-Beaujon, CRB3, Paris, France. FAU - Ronot, Maxime AU - Ronot M AUID- ORCID: 0000-0001-7464-3939 AD - Department of Radiology, Beaujon Hospital, APHP, University Hospitals Paris Nord Val de Seine, 100 Boulevard du Général Leclerc, 92118, Clichy, Hauts-de-Seine, France. maxime.ronot@aphp.fr. AD - University Paris Diderot, Sorbonne Paris Cité, Paris, France. maxime.ronot@aphp.fr. AD - INSERM U1149, Centre de Recherche biomédicale Bichat-Beaujon, CRB3, Paris, France. maxime.ronot@aphp.fr. LA - eng PT - Comparative Study PT - Journal Article DEP - 20200612 PL - United States TA - Cardiovasc Intervent Radiol JT - Cardiovascular and interventional radiology JID - 8003538 SB - IM CIN - Cardiovasc Intervent Radiol. 2020 Nov;43(11):1619-1620. doi: 10.1007/s00270-020-02635-x. PMID: 32909065 MH - Ablation Techniques/*methods MH - Carcinoma, Hepatocellular/diagnosis/*therapy MH - Catheter Ablation/methods MH - Disease Progression MH - Female MH - Humans MH - Liver Neoplasms/diagnosis/*therapy MH - Male MH - Microwaves/*therapeutic use MH - Middle Aged MH - *Propensity Score MH - Risk Factors MH - Treatment Outcome OTO - NOTNLM OT - Carcinoma OT - Disease progression OT - Hepatocellular OT - Propensity score EDAT- 2020/06/14 06:00 MHDA- 2021/04/07 06:00 CRDT- 2020/06/14 06:00 PHST- 2020/01/14 00:00 [received] PHST- 2020/05/30 00:00 [accepted] PHST- 2020/06/14 06:00 [pubmed] PHST- 2021/04/07 06:00 [medline] PHST- 2020/06/14 06:00 [entrez] AID - 10.1007/s00270-020-02549-8 [pii] AID - 10.1007/s00270-020-02549-8 [doi] PST - ppublish SO - Cardiovasc Intervent Radiol. 2020 Nov;43(11):1608-1618. doi: 10.1007/s00270-020-02549-8. Epub 2020 Jun 12. PMID- 39446878 OWN - NLM STAT- MEDLINE DCOM- 20241024 LR - 20241027 IS - 1545-7885 (Electronic) IS - 1544-9173 (Print) IS - 1544-9173 (Linking) VI - 22 IP - 10 DP - 2024 Oct TI - Individualistic reward-seeking strategies that predict response to nicotine emerge among isogenic male mice living in a micro-society. PG - e3002850 LID - 10.1371/journal.pbio.3002850 [doi] LID - e3002850 AB - Individual animals differ in their traits and preferences, which shape their social interactions, survival, and susceptibility to disease, including addiction. Nicotine use is highly heterogenous and has been linked to the expression of personality traits. Although these relationships are well documented, we have limited understanding of the neurophysiological mechanisms that give rise to distinct behavioral profiles and their connection to nicotine susceptibility. To address this question, we conducted a study using a semi-natural and social environment called "Souris-City" to observe the long-term behavior of individual male mice. Souris-City provided both a communal living area and a separate test area where mice engaged in a reward-seeking task isolated from their peers. Mice developed individualistic reward-seeking strategies when choosing between water and sucrose in the test compartment, which, in turn, predicted how they adapted to the introduction of nicotine as a reinforcer. Moreover, the profiles mice developed while isolated in the test area correlated with their behavior within the social environment, linking decision-making strategies to the expression of behavioral traits. Neurophysiological markers of adaptability within the dopamine system were apparent upon nicotine challenge and were associated with specific profiles. Our findings suggest that environmental adaptations influence behavioral traits and sensitivity to nicotine by acting on dopaminergic reactivity in the face of nicotine exposure, potentially contributing to addiction susceptibility. These results further emphasize the importance of understanding interindividual variability in behavior to gain insight into the mechanisms of decision-making and addiction. CI - Copyright: © 2024 Fayad et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. FAU - Fayad, Sophie L AU - Fayad SL AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Reynolds, Lauren M AU - Reynolds LM AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Torquet, Nicolas AU - Torquet N AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Tolu, Stefania AU - Tolu S AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Mondoloni, Sarah AU - Mondoloni S AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Nguyen, Claire AU - Nguyen C AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Siriphanh, Amy AU - Siriphanh A AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Justo, Robin AU - Justo R AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Didienne, Steve AU - Didienne S AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Debray, Nicolas AU - Debray N AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Viollet, Cécile AU - Viollet C AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Raynaud, Louis AU - Raynaud L AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Layadi, Yasmine AU - Layadi Y AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Fouquet, Coralie AU - Fouquet C AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Hannesse, Bernadette AU - Hannesse B AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. FAU - Capaz, Ana-Marta AU - Capaz AM AD - Sorbonne University, ICM Institut du Cerveau et de la Moelle Epinière, Laboratoire de Plasticité Structurale INSERM U1127, CNRS UMR7225, Paris, France. FAU - Topilko, Thomas AU - Topilko T AD - Sorbonne University, ICM Institut du Cerveau et de la Moelle Epinière, Laboratoire de Plasticité Structurale INSERM U1127, CNRS UMR7225, Paris, France. FAU - Renier, Nicolas AU - Renier N AD - Sorbonne University, ICM Institut du Cerveau et de la Moelle Epinière, Laboratoire de Plasticité Structurale INSERM U1127, CNRS UMR7225, Paris, France. FAU - Mourot, Alexandre AU - Mourot A AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Marti, Fabio AU - Marti F AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. FAU - Faure, Philippe AU - Faure P AD - Sorbonne University, INSERM, CNRS UMR8246, Neuroscience Paris Seine-Institut de Biologie Paris Seine (NPS-IBPS), Paris, France. AD - ESPCI Paris, PSL Research University, Brain Plasticity laboratory, CNRS UMR8249, Paris, France. LA - eng PT - Journal Article DEP - 20241024 PL - United States TA - PLoS Biol JT - PLoS biology JID - 101183755 RN - 6M3C89ZY6R (Nicotine) RN - VTD58H1Z2X (Dopamine) SB - IM MH - Animals MH - Male MH - *Nicotine/pharmacology MH - *Reward MH - Mice MH - *Behavior, Animal/drug effects/physiology MH - Mice, Inbred C57BL MH - Social Environment MH - Dopamine/metabolism MH - Social Behavior PMC - PMC11501037 COIS- The authors have declared that no competing interests exist. EDAT- 2024/10/25 07:20 MHDA- 2024/10/25 07:21 PMCR- 2024/10/24 CRDT- 2024/10/24 14:03 PHST- 2024/08/07 00:00 [received] PHST- 2024/09/18 00:00 [accepted] PHST- 2024/10/25 07:21 [medline] PHST- 2024/10/25 07:20 [pubmed] PHST- 2024/10/24 14:03 [entrez] PHST- 2024/10/24 00:00 [pmc-release] AID - PBIOLOGY-D-24-02296 [pii] AID - 10.1371/journal.pbio.3002850 [doi] PST - epublish SO - PLoS Biol. 2024 Oct 24;22(10):e3002850. doi: 10.1371/journal.pbio.3002850. eCollection 2024 Oct. PMID- 35115219 OWN - NLM STAT- MEDLINE DCOM- 20220404 LR - 20240824 IS - 1769-664X (Electronic) IS - 0929-693X (Print) IS - 0929-693X (Linking) VI - 29 IP - 3 DP - 2022 Apr TI - The psychological effects of COVID-19-related containment in children: The E-COCCON French study. PG - 188-193 LID - S0929-693X(22)00013-6 [pii] LID - 10.1016/j.arcped.2022.01.011 [doi] AB - The first containment of the Sars-Cov2 pandemic had the potential to generate posttraumatic stress (PTS) symptoms in children. OBJECTIVE: The main objective of the study was to determine the prevalence of PTS symptoms within 6 weeks of the end of lockdown, in children contained between March 17, 2020 and May 11, 2020 in France. MATERIAL AND METHODS: This was a French prospective cross-sectional study between May 15 and July 2, 2020 conducted via telephone survey. Parents of children aged between 8 and 15 years were eligible. The invitation to participate was proposed through social networks (Instagram and Facebook), various local and national media, and by e-mail to the staff of our University Hospital Center. The PTS symptoms were assessed using the CRIES-13. A score of 30 and over has been confirmed as the cut-off for screening cases. RESULTS: During the study period, 379 children (male, n = 207) were included, their mean age was 10.8±2.1 years. Symptoms of PTSD were identified in 17% of the children (girls 20.5%, boys 13.5%). These children were younger (p = 0.04), lacked access to a private outdoor space (p < 0.0001; OR: 7.8), had parents whose profession exposed them more to the coronavirus, and had parents who were more afraid of COVID-19. CONCLUSION: After the first lockdown related to the pandemic crisis, children developed PTSD symptoms. The onset of such symptoms is correlated with gender, age, lockdown conditions, and parental perceptions. These last considerations were worse for pink- or blue-collar families, attesting to the subsequent intensification of health inequalities. CI - Copyright © 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved. FAU - Claudet, I AU - Claudet I AD - Pediatric Emergency Department, Children's Hospital, CHU Toulouse, France; UMR 1295, Inserm, Paul Sabatier University, UPS, Toulouse III, France. Electronic address: claudet.i@chu-toulouse.fr. FAU - Marchand-Tonel, C AU - Marchand-Tonel C AD - Director of the Health Division, IFRASS, Toulouse, France. FAU - Kelly-Irving, M AU - Kelly-Irving M AD - UMR 1295, Inserm, Paul Sabatier University, UPS, Toulouse III, France. FAU - Gaudron, C Zaouche AU - Gaudron CZ AD - UMR 5193, Laboratoire Interdisciplinaire Solidarités, Sociétés, Territoires (LISST), Jean-Jaurès University, UT2J, Toulouse II, France. FAU - Raynaud, J-P AU - Raynaud JP AD - UMR 1295, Inserm, Paul Sabatier University, UPS, Toulouse III, France; Child and Adolescent Psychiatry Department (SUPEA), CHU Toulouse, France. FAU - Delpierre, C AU - Delpierre C AD - UMR 1295, Inserm, Paul Sabatier University, UPS, Toulouse III, France. FAU - Bréhin, C AU - Bréhin C AD - Pediatric Emergency Department, Children's Hospital, CHU Toulouse, France; UMR 1416, Inserm, IRSD, Toulouse, France. LA - eng PT - Journal Article DEP - 20220119 PL - France TA - Arch Pediatr JT - Archives de pediatrie : organe officiel de la Societe francaise de pediatrie JID - 9421356 RN - 0 (RNA, Viral) SB - IM MH - Adolescent MH - *COVID-19/epidemiology/prevention & control MH - Child MH - Communicable Disease Control MH - Cross-Sectional Studies MH - Female MH - Humans MH - Male MH - Prospective Studies MH - RNA, Viral MH - SARS-CoV-2 PMC - PMC8768449 OTO - NOTNLM OT - COVID-19 OT - Children OT - Containment OT - Lockdown OT - Posttraumatic stress syndrome COIS- Conflict of interest The authors have no conflicts of interests to disclose. EDAT- 2022/02/05 06:00 MHDA- 2022/04/05 06:00 PMCR- 2022/01/19 CRDT- 2022/02/04 05:38 PHST- 2021/09/28 00:00 [received] PHST- 2021/12/03 00:00 [revised] PHST- 2022/01/13 00:00 [accepted] PHST- 2022/02/05 06:00 [pubmed] PHST- 2022/04/05 06:00 [medline] PHST- 2022/02/04 05:38 [entrez] PHST- 2022/01/19 00:00 [pmc-release] AID - S0929-693X(22)00013-6 [pii] AID - 10.1016/j.arcped.2022.01.011 [doi] PST - ppublish SO - Arch Pediatr. 2022 Apr;29(3):188-193. doi: 10.1016/j.arcped.2022.01.011. Epub 2022 Jan 19. PMID- 17182728 OWN - NLM STAT- MEDLINE DCOM- 20070621 LR - 20200930 IS - 0363-6143 (Print) IS - 0363-6143 (Linking) VI - 292 IP - 5 DP - 2007 May TI - Differential localization of autolyzed calpains 1 and 2 in slow and fast skeletal muscles in the early phase of atrophy. PG - C1723-31 AB - Calpains have been proposed to be involved in the cytoskeletal remodeling and wasting of skeletal muscle. However, limited data are available about the specific involvement of each calpain in the early stages of muscle atrophy. The aims of this study were to determine whether calpains 1 and 2 are autolyzed after a short period of muscle disuse, and, if so, where in the myofibers the autolyzed products are localized. In the rat soleus muscle, 5 days of immobilization increased autolyzed calpain 1 in the particulate and not the soluble fraction. Conversely, autolyzed calpain 2 was not found in the particulate fraction, whereas it was increased in the soluble fraction after immobilization. In the less atrophied plantaris muscle, no difference was noted between the control and immobilized groups whatever the fraction or calpain. Other proteolytic pathways were also investigated. The ubiquitin-proteasome pathway was activated in both skeletal muscles, and caspase 3 was activated only in the soleus muscle. Taken together, our data suggest that calpains 1 and 2 are involved in atrophy development in slow type muscle exclusively and that they have different regulation and protein targets. Moreover, the activation of proteolytic pathways appears to differ in slow and fast muscles, and the proteolytic mechanisms involved in fast-type muscle atrophy remain unclear. FAU - Vermaelen, Marianne AU - Vermaelen M AD - INSERM, ERI 25, Hôpitol Arnaud de Villneuve, 34295 Montpellier Cedex 5, France. mvermaelen@netcourrier.com FAU - Sirvent, Pascal AU - Sirvent P FAU - Raynaud, Fabrice AU - Raynaud F FAU - Astier, Catherine AU - Astier C FAU - Mercier, Jacques AU - Mercier J FAU - Lacampagne, Alain AU - Lacampagne A FAU - Cazorla, Olivier AU - Cazorla O LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20061220 PL - United States TA - Am J Physiol Cell Physiol JT - American journal of physiology. Cell physiology JID - 100901225 RN - 0 (Ubiquitin) RN - EC 3.4.22.- (Calpain) RN - EC 3.4.22.- (Caspase 3) RN - EC 3.4.25.1 (Proteasome Endopeptidase Complex) SB - IM MH - Animals MH - Autolysis MH - Calpain/*metabolism MH - Caspase 3/metabolism MH - Disease Models, Animal MH - Enzyme Activation MH - Hindlimb Suspension MH - Male MH - Muscle Fibers, Fast-Twitch/enzymology MH - Muscle Fibers, Skeletal/*enzymology/pathology MH - Muscle Fibers, Slow-Twitch/enzymology MH - Muscle, Skeletal/*enzymology/pathology MH - Muscular Atrophy/*enzymology/pathology MH - Myofibrils/enzymology MH - Phenotype MH - Proteasome Endopeptidase Complex/metabolism MH - Rats MH - Rats, Wistar MH - Time Factors MH - Ubiquitin/metabolism EDAT- 2006/12/22 09:00 MHDA- 2007/06/22 09:00 CRDT- 2006/12/22 09:00 PHST- 2006/12/22 09:00 [pubmed] PHST- 2007/06/22 09:00 [medline] PHST- 2006/12/22 09:00 [entrez] AID - 00398.2006 [pii] AID - 10.1152/ajpcell.00398.2006 [doi] PST - ppublish SO - Am J Physiol Cell Physiol. 2007 May;292(5):C1723-31. doi: 10.1152/ajpcell.00398.2006. Epub 2006 Dec 20. PMID- 34296319 OWN - NLM STAT- MEDLINE DCOM- 20210922 LR - 20220218 IS - 1420-9071 (Electronic) IS - 1420-682X (Print) IS - 1420-682X (Linking) VI - 78 IP - 17-18 DP - 2021 Sep TI - Cellular and molecular actors of myeloid cell fusion: podosomes and tunneling nanotubes call the tune. PG - 6087-6104 LID - 10.1007/s00018-021-03875-x [doi] AB - Different types of multinucleated giant cells (MGCs) of myeloid origin have been described; osteoclasts are the most extensively studied because of their importance in bone homeostasis. MGCs are formed by cell-to-cell fusion, and most types have been observed in pathological conditions, especially in infectious and non-infectious chronic inflammatory contexts. The precise role of the different MGCs and the mechanisms that govern their formation remain poorly understood, likely due to their heterogeneity. First, we will introduce the main populations of MGCs derived from the monocyte/macrophage lineage. We will then discuss the known molecular actors mediating the early stages of fusion, focusing on cell-surface receptors involved in the cell-to-cell adhesion steps that ultimately lead to multinucleation. Given that cell-to-cell fusion is a complex and well-coordinated process, we will also describe what is currently known about the evolution of F-actin-based structures involved in macrophage fusion, i.e., podosomes, zipper-like structures, and tunneling nanotubes (TNT). Finally, the localization and potential role of the key fusion mediators related to the formation of these F-actin structures will be discussed. This review intends to present the current status of knowledge of the molecular and cellular mechanisms supporting multinucleation of myeloid cells, highlighting the gaps still existing, and contributing to the proposition of potential disease-specific MGC markers and/or therapeutic targets. CI - © 2021. The Author(s). FAU - Dufrançais, Ophélie AU - Dufrançais O AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. FAU - Mascarau, Rémi AU - Mascarau R AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Toulouse, France. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Buenos Aires, Argentina. FAU - Poincloux, Renaud AU - Poincloux R AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. FAU - Maridonneau-Parini, Isabelle AU - Maridonneau-Parini I AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Toulouse, France. FAU - Raynaud-Messina, Brigitte AU - Raynaud-Messina B AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. raynaud@ipbs.fr. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Toulouse, France. raynaud@ipbs.fr. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Buenos Aires, Argentina. raynaud@ipbs.fr. FAU - Vérollet, Christel AU - Vérollet C AUID- ORCID: 0000-0002-1079-9085 AD - Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France. verollet@ipbs.fr. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Toulouse, France. verollet@ipbs.fr. AD - International Associated Laboratory (LIA) CNRS "IM-TB/HIV" (1167), Buenos Aires, Argentina. verollet@ipbs.fr. LA - eng PT - Journal Article PT - Review DEP - 20210723 PL - Switzerland TA - Cell Mol Life Sci JT - Cellular and molecular life sciences : CMLS JID - 9705402 RN - 0 (Integrins) RN - 0 (Receptors, Immunologic) SB - IM MH - *Cell Adhesion MH - Giant Cells/cytology/*metabolism MH - Humans MH - Integrins/metabolism MH - Macrophages/cytology/metabolism MH - Myeloid Cells/cytology/*metabolism/ultrastructure MH - Osteoclasts/cytology/metabolism MH - Osteogenesis MH - Podosomes/*metabolism MH - Receptors, Immunologic/metabolism PMC - PMC8429379 OTO - NOTNLM OT - Adhesion OT - Cell-to-cell fusion OT - Multinucleated giant cells (MGCs) OT - Osteoclasts (OCs) OT - Podosomes OT - Tunneling nanotubes (TNTs) COIS- The authors declare that they have no competing interests. EDAT- 2021/07/24 06:00 MHDA- 2021/09/23 06:00 PMCR- 2021/07/23 CRDT- 2021/07/23 07:01 PHST- 2021/01/28 00:00 [received] PHST- 2021/06/05 00:00 [accepted] PHST- 2021/05/25 00:00 [revised] PHST- 2021/07/24 06:00 [pubmed] PHST- 2021/09/23 06:00 [medline] PHST- 2021/07/23 07:01 [entrez] PHST- 2021/07/23 00:00 [pmc-release] AID - 10.1007/s00018-021-03875-x [pii] AID - 3875 [pii] AID - 10.1007/s00018-021-03875-x [doi] PST - ppublish SO - Cell Mol Life Sci. 2021 Sep;78(17-18):6087-6104. doi: 10.1007/s00018-021-03875-x. Epub 2021 Jul 23. PMID- 22038543 OWN - NLM STAT- MEDLINE DCOM- 20120306 LR - 20211020 IS - 1468-201X (Electronic) IS - 1355-6037 (Print) IS - 1355-6037 (Linking) VI - 98 IP - 4 DP - 2012 Feb TI - The evolution of infrahissian conduction time in myotonic dystrophy patients: clinical implications. PG - 291-6 LID - 10.1136/heartjnl-2011-300143 [doi] AB - BACKGROUND: Myotonic dystrophy (MD1) is a hereditary autosomal dominant disease with variable penetrance. Cardiac conduction disturbances are frequent and may be responsible for sudden death, but its progression was heretofore unknown. AIMS: The aim of the study was to analyse the natural history of infrahissian conduction time in patients with a normal first electrophysiological test, and to identify the predictive value of the clinical and ECG factors accompanying an alteration of infrahissian conduction. METHODS: Among 127 consecutive screened MD patients, 25 were enrolled and underwent a second electrophysiological testing. The second electrophysiological test was carried out on patients showing new symptoms, new atrioventricular conduction disturbances on ECG, or significant modifications of signal-averaged (SA)-ECG, and on asymptomatic patients with a follow-up of at least 60 months since the first electrophysiological test. RESULTS: Among the 25 patients, four had new clinical symptoms, four others developed new atrioventricular conduction abnormalities on ECG and six had significant modifications of the SA-ECG. The mean His-ventricle (HV) interval increased significantly between the two electrophysiological studies (initial HV interval 52.1 ms±1.6 ms, final HV interval 61.4 ms±2.2 ms, p<0.005), with a mean increase of 1.2 ms/year. The five patients with HV interval of 70 ms or greater were implanted with a prophylactic dual-chamber pacemaker. Modifications of resting ECG and SA-ECG were strongly associated with HV interval prolongation. CONCLUSION: In patients with a normal initial electrophysiological study, modifications on the resting ECG and/or SA-ECG, on annual check-up, were associated with an alteration of infrahissian conduction. FAU - Lallemand, Bénédicte AU - Lallemand B AD - Cardiology Department, Hospital Trousseau, François Rabelais University, 37044 Tours, France. FAU - Clementy, Nicolas AU - Clementy N FAU - Bernard-Brunet, Anne AU - Bernard-Brunet A FAU - Pierre, Bertrand AU - Pierre B FAU - Corcia, Philippe AU - Corcia P FAU - Fauchier, Laurent AU - Fauchier L FAU - Raynaud, Martine AU - Raynaud M FAU - Pellieux, Sybille AU - Pellieux S FAU - Babuty, Dominique AU - Babuty D LA - eng PT - Comparative Study PT - Journal Article DEP - 20111029 PL - England TA - Heart JT - Heart (British Cardiac Society) JID - 9602087 SB - IM CIN - Heart. 2012 Mar;98(6):433-4. doi: 10.1136/heartjnl-2011-301407. PMID: 22350028 MH - Adult MH - Aged MH - Arrhythmias, Cardiac/diagnosis/etiology/*physiopathology MH - Electrocardiography/*methods MH - Female MH - Follow-Up Studies MH - Heart Conduction System/*physiopathology MH - Heart Rate MH - Humans MH - Male MH - Middle Aged MH - Myotonic Dystrophy/complications/diagnosis/*physiopathology MH - Predictive Value of Tests MH - Young Adult PMC - PMC3262987 COIS- Competing interests: None. EDAT- 2011/11/01 06:00 MHDA- 2012/03/07 06:00 PMCR- 2011/10/29 CRDT- 2011/11/01 06:00 PHST- 2011/11/01 06:00 [entrez] PHST- 2011/11/01 06:00 [pubmed] PHST- 2012/03/07 06:00 [medline] PHST- 2011/10/29 00:00 [pmc-release] AID - heartjnl-2011-300143 [pii] AID - 10.1136/heartjnl-2011-300143 [doi] PST - ppublish SO - Heart. 2012 Feb;98(4):291-6. doi: 10.1136/heartjnl-2011-300143. Epub 2011 Oct 29. PMID- 30594563 OWN - NLM STAT- MEDLINE DCOM- 20190311 LR - 20190311 IS - 1873-7544 (Electronic) IS - 0306-4522 (Linking) VI - 399 DP - 2019 Feb 10 TI - LIMK2-1 is a Hominidae-Specific Isoform of LIMK2 Expressed in Central Nervous System and Associated with Intellectual Disability. PG - 199-210 LID - S0306-4522(18)30826-1 [pii] LID - 10.1016/j.neuroscience.2018.12.017 [doi] AB - LIMK2 is involved in neuronal functions by regulating actin dynamics. Different isoforms of LIMK2 are described in databanks. LIMK2a and LIMK2b are the most characterized. A few pieces of evidence suggest that LIMK2 isoforms might not have overlapping functions. In this study, we focused our attention on a less studied human LIMK2 isoform, LIMK2-1. Compared to the other LIMK2 isoforms, LIMK2-1 contains a supplementary C-terminal phosphatase 1 inhibitory domain (PP1i). We found out that this isoform was hominidae-specific and showed that it was expressed in human fetal brain and faintly in adult brain. Its coding sequence was sequenced in 173 patients with sporadic non-syndromic intellectual disability (ID), and we observed an association of a rare missense variant in the PP1i domain (rs151191437, p.S668P) with ID. Our results also suggest an implication of LIMK2-1 in neurite outgrowth and neurons arborization which appears to be affected by the p.S668P variation. Therefore our results suggest that LIMK2-1 plays a role in the developing brain, and that a rare variation of this isoform is a susceptibility factor in ID. CI - Copyright © 2018 IBRO. Published by Elsevier Ltd. All rights reserved. FAU - Tastet, Julie AU - Tastet J AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CNRS UPR 4301, CBM, Orléans, France; Department of Translational Neuroscience, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, Netherlands. FAU - Cuberos, Hélène AU - Cuberos H AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CNRS UPR 4301, CBM, Orléans, France. FAU - Vallée, Béatrice AU - Vallée B AD - CNRS UPR 4301, CBM, Orléans, France. FAU - Toutain, Annick AU - Toutain A AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CHRU de Tours, Service de Génétique, Tours, France. FAU - Raynaud, Martine AU - Raynaud M AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CHRU de Tours, Service de Génétique, Tours, France. FAU - Marouillat, Sylviane AU - Marouillat S AD - UMR INSERM U1253, Université François Rabelais, Tours, France. FAU - Thépault, Rose-Anne AU - Thépault RA AD - UMR INSERM U1253, Université François Rabelais, Tours, France. FAU - Laumonnier, Frédéric AU - Laumonnier F AD - UMR INSERM U1253, Université François Rabelais, Tours, France. FAU - Bonnet-Brilhault, Frédérique AU - Bonnet-Brilhault F AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CHRU de Tours, Service de Pédopsychiatrie, Tours, France. FAU - Vourc'h, Patrick AU - Vourc'h P AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CHRU de Tours, Service de Biochimie et de Biologie Moléculaire, Tours, France. FAU - Andres, Christian R AU - Andres CR AD - UMR INSERM U1253, Université François Rabelais, Tours, France; CHRU de Tours, Service de Biochimie et de Biologie Moléculaire, Tours, France. FAU - Bénédetti, Hélène AU - Bénédetti H AD - CNRS UPR 4301, CBM, Orléans, France. Electronic address: helene.benedetti@cnrs-orleans.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20181227 PL - United States TA - Neuroscience JT - Neuroscience JID - 7605074 RN - 0 (Protein Isoforms) RN - EC 2.7.11.1 (LIMK2 protein, human) RN - EC 2.7.11.1 (Lim Kinases) SB - IM MH - Amino Acid Sequence MH - Animals MH - Cells, Cultured MH - Central Nervous System/cytology/*growth & development/*metabolism MH - Genetic Predisposition to Disease MH - Hominidae MH - Intellectual Disability/genetics/*metabolism MH - Lim Kinases/genetics/*metabolism MH - Mice MH - Models, Molecular MH - Mutation, Missense MH - Neuronal Outgrowth/physiology MH - Neurons/cytology/metabolism MH - Protein Isoforms MH - Rats MH - Sequence Homology OTO - NOTNLM OT - LIM kinase OT - cytoskeleton remodeling OT - hominidae-specific OT - intellectual deficiency OT - neuron morphology OT - patient mutation EDAT- 2018/12/31 06:00 MHDA- 2019/03/12 06:00 CRDT- 2018/12/31 06:00 PHST- 2018/11/19 00:00 [received] PHST- 2018/12/12 00:00 [revised] PHST- 2018/12/13 00:00 [accepted] PHST- 2018/12/31 06:00 [pubmed] PHST- 2019/03/12 06:00 [medline] PHST- 2018/12/31 06:00 [entrez] AID - S0306-4522(18)30826-1 [pii] AID - 10.1016/j.neuroscience.2018.12.017 [doi] PST - ppublish SO - Neuroscience. 2019 Feb 10;399:199-210. doi: 10.1016/j.neuroscience.2018.12.017. Epub 2018 Dec 27. PMID- 29617189 OWN - NLM STAT- MEDLINE DCOM- 20190725 LR - 20190725 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 36 IP - 15 DP - 2018 May 20 TI - Cumulative Burden of Morbidity Among Testicular Cancer Survivors After Standard Cisplatin-Based Chemotherapy: A Multi-Institutional Study. PG - 1505-1512 LID - 10.1200/JCO.2017.77.0735 [doi] AB - Purpose In this multicenter study, we evaluated the cumulative burden of morbidity (CBM) among > 1,200 testicular cancer survivors and applied factor analysis to determine the co-occurrence of adverse health outcomes (AHOs). Patients and Methods Participants were ≤ 55 years of age at diagnosis, finished first-line chemotherapy ≥ 1 year previously, completed a comprehensive questionnaire, and underwent physical examination. Treatment data were abstracted from medical records. A CBM score encompassed the number and severity of AHOs, with ordinal logistic regression used to assess associations with exposures. Nonlinear factor analysis and the nonparametric dimensionality evaluation to enumerate contributing traits procedure determined which AHOs co-occurred. Results Among 1,214 participants, approximately 20% had a high (15%) or very high/severe (4.1%) CBM score, whereas approximately 80% scored medium (30%) or low/very low (47%). Increased risks of higher scores were associated with four cycles of either ifosfamide, etoposide, and cisplatin (odds ratio [OR], 1.96; 95% CI, 1.04 to 3.71) or bleomycin, etoposide, and cisplatin (OR, 1.44; 95% CI, 1.04 to 1.98), older attained age (OR, 1.18; 95% CI, 1.10 to 1.26), current disability leave (OR, 3.53; 95% CI, 1.57 to 7.95), less than a college education (OR, 1.44; 95% CI, 1.11 to 1.87), and current or former smoking (OR, 1.28; 95% CI, 1.02 to 1.63). CBM score did not differ after either chemotherapy regimen ( P = .36). Asian race (OR, 0.41; 95% CI, 0.23 to 0.72) and vigorous exercise (OR, 0.68; 95% CI, 0.52 to 0.89) were protective. Variable clustering analyses identified six significant AHO clusters (χ(2) P < .001): hearing loss/damage, tinnitus (OR, 16.3); hyperlipidemia, hypertension, diabetes (OR, 9.8); neuropathy, pain, Raynaud phenomenon (OR, 5.5); cardiovascular and related conditions (OR, 5.0); thyroid disease, erectile dysfunction (OR, 4.2); and depression/anxiety, hypogonadism (OR, 2.8). Conclusion Factors associated with higher CBM may identify testicular cancer survivors in need of closer monitoring. If confirmed, identified AHO clusters could guide the development of survivorship care strategies. FAU - Kerns, Sarah L AU - Kerns SL AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Fung, Chunkit AU - Fung C AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Monahan, Patrick O AU - Monahan PO AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Ardeshir-Rouhani-Fard, Shirin AU - Ardeshir-Rouhani-Fard S AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Abu Zaid, Mohammad I AU - Abu Zaid MI AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Williams, AnnaLynn M AU - Williams AM AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Stump, Timothy E AU - Stump TE AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Sesso, Howard D AU - Sesso HD AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Feldman, Darren R AU - Feldman DR AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Hamilton, Robert J AU - Hamilton RJ AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Vaughn, David J AU - Vaughn DJ AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Beard, Clair AU - Beard C AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Huddart, Robert A AU - Huddart RA AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Kim, Jeri AU - Kim J AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Kollmannsberger, Christian AU - Kollmannsberger C AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Sahasrabudhe, Deepak M AU - Sahasrabudhe DM AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Cook, Ryan AU - Cook R AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Fossa, Sophie D AU - Fossa SD AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Einhorn, Lawrence H AU - Einhorn LH AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. FAU - Travis, Lois B AU - Travis LB AD - Sarah L. Kerns, Chunkit Fung, AnnaLynn M. Williams, and Deepak M. Sahasrabudhe, University of Rochester Medical Center, Rochester; Darren R. Feldman, Memorial Sloan Kettering Cancer Center, New York, NY; Patrick O. Monahan, Shirin Ardeshir-Rouhani-Fard, Mohammad I. Abu Zaid, Timothy E. Stump, Ryan Cook, Lawrence H. Einhorn, and Lois B. Travis, Indiana University, Indianapolis, IN; Howard D. Sesso, Brigham and Women's Hospital; Clair Beard, Dana-Farber Cancer Institute, Boston, MA; Robert J. Hamilton, Princess Margaret Cancer Centre, Toronto, Ontario; Christian Kollmannsberger, University of British Columbia, Vancouver, British Columbia, Canada; David J. Vaughn, University of Pennsylvania, Philadelphia, PA; Robert A. Huddart, The Royal Marsden Hospital, London, United Kingdom; Jeri Kim, MD Anderson Cancer Center, Houston, TX; and Sophie D. Fossa, Oslo University Hospital, Oslo, Norway. CN - Platinum Study Group LA - eng GR - K07 CA187546/CA/NCI NIH HHS/United States GR - P30 CA008748/CA/NCI NIH HHS/United States GR - R01 CA157823/CA/NCI NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural DEP - 20180404 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - Q20Q21Q62J (Cisplatin) RN - Testicular Germ Cell Tumor CIN - J Urol. 2019 Jan;201(1):32-33. doi: 10.1097/01.ju.0000550157.48897.08. PMID: 30577377 MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/*adverse effects MH - Cancer Survivors/*statistics & numerical data MH - Cisplatin/administration & dosage/adverse effects MH - Exercise MH - Factor Analysis, Statistical MH - *Health Status MH - Humans MH - Long Term Adverse Effects/chemically induced/epidemiology MH - Male MH - Middle Aged MH - Neoplasms, Germ Cell and Embryonal/*drug therapy MH - Physical Examination MH - Risk Factors MH - Severity of Illness Index MH - Surveys and Questionnaires MH - Testicular Neoplasms/*drug therapy PMC - PMC5959198 EDAT- 2018/04/05 06:00 MHDA- 2019/07/26 06:00 PMCR- 2019/05/20 CRDT- 2018/04/05 06:00 PHST- 2018/04/05 06:00 [pubmed] PHST- 2019/07/26 06:00 [medline] PHST- 2018/04/05 06:00 [entrez] PHST- 2019/05/20 00:00 [pmc-release] AID - 770735 [pii] AID - 10.1200/JCO.2017.77.0735 [doi] PST - ppublish SO - J Clin Oncol. 2018 May 20;36(15):1505-1512. doi: 10.1200/JCO.2017.77.0735. Epub 2018 Apr 4. PMID- 34756569 OWN - NLM STAT- MEDLINE DCOM- 20211231 LR - 20211231 IS - 2589-7500 (Electronic) IS - 2589-7500 (Linking) VI - 3 IP - 12 DP - 2021 Dec TI - Dynamic prediction of renal survival among deeply phenotyped kidney transplant recipients using artificial intelligence: an observational, international, multicohort study. PG - e795-e805 LID - S2589-7500(21)00209-0 [pii] LID - 10.1016/S2589-7500(21)00209-0 [doi] AB - BACKGROUND: Kidney allograft failure is a common cause of end-stage renal disease. We aimed to develop a dynamic artificial intelligence approach to enhance risk stratification for kidney transplant recipients by generating continuously refined predictions of survival using updates of clinical data. METHODS: In this observational study, we used data from adult recipients of kidney transplants from 18 academic transplant centres in Europe, the USA, and South America, and a cohort of patients from six randomised controlled trials. The development cohort comprised patients from four centres in France, with all other patients included in external validation cohorts. To build deeply phenotyped cohorts of transplant recipients, the following data were collected in the development cohort: clinical, histological, immunological variables, and repeated measurements of estimated glomerular filtration rate (eGFR) and proteinuria (measured using the proteinuria to creatininuria ratio). To develop a dynamic prediction system based on these clinical assessments and repeated measurements, we used a Bayesian joint models-an artificial intelligence approach. The prediction performances of the model were assessed via discrimination, through calculation of the area under the receiver operator curve (AUC), and calibration. This study is registered with ClinicalTrials.gov, NCT04258891. FINDINGS: 13 608 patients were included (3774 in the development cohort and 9834 in the external validation cohorts) and contributed 89 328 patient-years of data, and 416 510 eGFR and proteinuria measurements. Bayesian joint models showed that recipient immunological profile, allograft interstitial fibrosis and tubular atrophy, allograft inflammation, and repeated measurements of eGFR and proteinuria were independent risk factors for allograft survival. The final model showed accurate calibration and very high discrimination in the development cohort (overall dynamic AUC 0·857 [95% CI 0·847-0·866]) with a persistent improvement in AUCs for each new repeated measurement (from 0·780 [0·768-0·794] to 0·926 [0·917-0·932]; p<0·0001). The predictive performance was confirmed in the external validation cohorts from Europe (overall AUC 0·845 [0·837-0·854]), the USA (overall AUC 0·820 [0·808-0·831]), South America (overall AUC 0·868 [0·856-0·880]), and the cohort of patients from randomised controlled trials (overall AUC 0·857 [0·840-0·875]). INTERPRETATION: Because of its dynamic design, this model can be continuously updated and holds value as a bedside tool that could refine the prognostic judgements of clinicians in everyday practice, hence enhancing precision medicine in the transplant setting. FUNDING: MSD Avenir, French National Institute for Health and Medical Research, and Bettencourt Schueller Foundation. CI - Copyright © 2021 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY-NC-ND 4.0 license. Published by Elsevier Ltd.. All rights reserved. FAU - Raynaud, Marc AU - Raynaud M AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France. FAU - Aubert, Olivier AU - Aubert O AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Divard, Gillian AU - Divard G AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Reese, Peter P AU - Reese PP AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Renal Electrolyte and Hypertension Division, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. FAU - Kamar, Nassim AU - Kamar N AD - Université Paul Sabatier, INSERM, Department of Nephrology and Organ Transplantation, CHU Rangueil & Purpan, Toulouse, France. FAU - Yoo, Daniel AU - Yoo D AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France. FAU - Chin, Chen-Shan AU - Chin CS AD - Deep Learning in Medicine and Genomics, DNAnexus, San Francisco, CA, USA. FAU - Bailly, Élodie AU - Bailly É AD - Nephrology and Immunology Department, Bretonneau Hospital, Tours, France. FAU - Buchler, Matthias AU - Buchler M AD - Nephrology and Immunology Department, Bretonneau Hospital, Tours, France. FAU - Ladrière, Marc AU - Ladrière M AD - Nephrology Dialysis Transplantation Department, University of Lorraine, Centre Hospitalier Universitaire Nancy, Nancy, France. FAU - Le Quintrec, Moglie AU - Le Quintrec M AD - Department of Nephrology, Centre Hospitalier Universitaire Montpellier, Montpellier, France. FAU - Delahousse, Michel AU - Delahousse M AD - Department of Transplantation, Nephrology and Clinical Immunology, Foch Hospital, Suresnes, France. FAU - Juric, Ivana AU - Juric I AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia. FAU - Basic-Jukic, Nikolina AU - Basic-Jukic N AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia. FAU - Crespo, Marta AU - Crespo M AD - Department of Nephrology, Hospital del Mar Barcelona, Spain. FAU - Silva, Helio Tedesco Jr AU - Silva HT Jr AD - Hospital do Rim, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Linhares, Kamilla AU - Linhares K AD - Hospital do Rim, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, Brazil. FAU - Ribeiro de Castro, Maria Cristina AU - Ribeiro de Castro MC AD - Renal Transplantation Service, Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. FAU - Soler Pujol, Gervasio AU - Soler Pujol G AD - Unidad de Trasplante Renopancreas, Centro de Educacion Medica e Investigaciones Clinicas Buenos Aires, Buenos Aires, Argentina. FAU - Empana, Jean-Philippe AU - Empana JP AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France. FAU - Ulloa, Camilo AU - Ulloa C AD - Kidney Transplantation Department, Clinica Alemana de Santiago, Santiago, Chile. FAU - Akalin, Enver AU - Akalin E AD - Renal Division Montefiore Medical Centre, Kidney Transplantation Program, Albert Einstein College of Medicine, NY, USA. FAU - Böhmig, Georg AU - Böhmig G AD - Division of Nephrology and Dialysis, Department of Medicine III, General Hospital Vienna, Vienna, Austria. FAU - Huang, Edmund AU - Huang E AD - Department of Medicine, Division of Nephrology, Comprehensive Transplant Centre, Cedars Sinai Medical Centre, Los Angeles, CA, USA. FAU - Stegall, Mark D AU - Stegall MD AD - William J von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, MN, USA. FAU - Bentall, Andrew J AU - Bentall AJ AD - William J von Liebig Centre for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, MN, USA. FAU - Montgomery, Robert A AU - Montgomery RA AD - New York University Langone Transplant Institute, New York, NY, USA. FAU - Jordan, Stanley C AU - Jordan SC AD - Department of Medicine, Division of Nephrology, Comprehensive Transplant Centre, Cedars Sinai Medical Centre, Los Angeles, CA, USA. FAU - Oberbauer, Rainer AU - Oberbauer R AD - Nephrology, Medical University of Vienna, Vienna, Austria. FAU - Segev, Dorry L AU - Segev DL AD - Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA. FAU - Friedewald, John J AU - Friedewald JJ AD - Kidney Transplantation Department, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. FAU - Jouven, Xavier AU - Jouven X AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Cardiology Department, European Georges Pompidou Hospital, Paris, France. FAU - Legendre, Christophe AU - Legendre C AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AD - Paris Translational Research Centre for Organ Transplantation, INSERM, PARCC, Université de Paris, Paris, France; Kidney Transplant Department, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France. Electronic address: alexandre.loupy@inserm.fr. LA - eng SI - ClinicalTrials.gov/NCT04258891 PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Validation Study DEP - 20211028 PL - England TA - Lancet Digit Health JT - The Lancet. Digital health JID - 101751302 SB - IM MH - Adult MH - *Allografts MH - Area Under Curve MH - *Artificial Intelligence MH - Bayes Theorem MH - Female MH - Glomerular Filtration Rate MH - Humans MH - Kidney/*surgery MH - *Kidney Transplantation MH - Male MH - Middle Aged MH - *Models, Biological MH - *Postoperative Complications MH - Prognosis MH - Proteinuria MH - Renal Insufficiency/*diagnosis/surgery MH - Reproducibility of Results MH - Risk Assessment MH - Transplant Recipients COIS- Declaration of interests AL holds shares in Cibiltech, a company that develops software and IT solutions. C-SC is affiliated with Deep Learning in Medicine and Genomics, DNAnexus. All other authors declare no competing interests. EDAT- 2021/11/11 06:00 MHDA- 2022/01/01 06:00 CRDT- 2021/11/10 14:00 PHST- 2021/03/25 00:00 [received] PHST- 2021/07/22 00:00 [revised] PHST- 2021/08/17 00:00 [accepted] PHST- 2021/11/11 06:00 [pubmed] PHST- 2022/01/01 06:00 [medline] PHST- 2021/11/10 14:00 [entrez] AID - S2589-7500(21)00209-0 [pii] AID - 10.1016/S2589-7500(21)00209-0 [doi] PST - ppublish SO - Lancet Digit Health. 2021 Dec;3(12):e795-e805. doi: 10.1016/S2589-7500(21)00209-0. Epub 2021 Oct 28. PMID- 33521883 OWN - NLM STAT- MEDLINE DCOM- 20220110 LR - 20220110 IS - 1433-8726 (Electronic) IS - 0724-4983 (Linking) VI - 39 IP - 8 DP - 2021 Aug TI - PSA and obesity among men with localized prostate cancer: results of the ANDROCAN study. PG - 2945-2951 LID - 10.1007/s00345-020-03557-6 [doi] AB - PURPOSE: PSA is known to be lowered in obese patients. There is a lack of data regarding patients with prostate cancer. Our objective was to prospectively assess the relationship PSA concentration, PSA mass and BMI in a cohort of patients with localized prostate cancer. METHODS: A prospective, multicenter cohort study was conducted including patients undergoing radical prostatectomy. Clinical and biological data were collected for each patient before surgery. RESULTS: A total of 1343 patients were analyzed. Mean age was 64.0 years. Mean weight was 82.2 kg and mean BMI was 26.8 kg/m(2). Mean PSA concentration was 8.7 ng/mL and mean PSA mass 29.3 ng. On univariate analysis, an association was found between PSA mass and either BMI, weight and waist circumference. No association was found between PSA concentration and each weight parameters. On multivariate analysis, obesity was not an independent predictor of PSA concentration (p = 0.73). Independent predictors of PSA concentration were cardiovascular disease (negative association, p = 0.034), predominant Gleason 4 (positive association, p < 0.001) and pT3a (positive association, p < 0.001). BMI was an independent predictor of PSA mass (positive association, p = 0.009). PSA mass was negatively associated with TT (p = 0.015) and cardiovascular disease (p = 0.003), and positively associated with BT (p = 0.032), Gleason grade ≥ 4 + 3 (p < 0.001) and pT3a (p < 0.001). CONCLUSION: In this prospective study of patients with localized prostate cancer, higher BMI was associated with higher PSA mass but not with higher PSA concentration. Screening obese patients with a specific PSA method does not appear to be critical. CI - © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature. FAU - Meunier, Matthias E AU - Meunier ME AUID- ORCID: 0000-0001-6205-3333 AD - Department of Urology, Université de Versailles-Saint-Quentin-en-Yvelines, Hôpital Foch, Service d'urologie40 rue Worth, 92150, Suresnes, France. matthias.meunier@yahoo.fr. AD - University of Versailles-Saint-Quentin-en-Yvelines, Versailles, France. matthias.meunier@yahoo.fr. FAU - Neuzillet, Yann AU - Neuzillet Y AD - Department of Urology, Université de Versailles-Saint-Quentin-en-Yvelines, Hôpital Foch, Service d'urologie40 rue Worth, 92150, Suresnes, France. AD - University of Versailles-Saint-Quentin-en-Yvelines, Versailles, France. FAU - Dreyfus, Jean-François AU - Dreyfus JF AD - Department of Clinical Research and Innovations, University of Versailles-Saint-Quentin-en-Yvelines, Versailles, France. FAU - Schneider, Marc AU - Schneider M AD - Department of Urology, Hôpital Louis Pasteur, Colmar, France. FAU - Rouprêt, Morgan AU - Rouprêt M AD - Department of Urology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Cathelineau, Xavier AU - Cathelineau X AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Sorbonne University, Paris, France. FAU - Lebret, Thierry AU - Lebret T AD - Department of Urology, Université de Versailles-Saint-Quentin-en-Yvelines, Hôpital Foch, Service d'urologie40 rue Worth, 92150, Suresnes, France. AD - University of Versailles-Saint-Quentin-en-Yvelines, Versailles, France. FAU - Botto, Henry AU - Botto H AD - Department of Urology, Université de Versailles-Saint-Quentin-en-Yvelines, Hôpital Foch, Service d'urologie40 rue Worth, 92150, Suresnes, France. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20210201 PL - Germany TA - World J Urol JT - World journal of urology JID - 8307716 RN - EC 3.4.21.77 (Prostate-Specific Antigen) SB - IM MH - Body Mass Index MH - Cohort Studies MH - Comorbidity MH - Correlation of Data MH - France/epidemiology MH - Humans MH - Male MH - Middle Aged MH - Neoplasm Grading MH - Neoplasm Staging MH - *Obesity/blood/diagnosis/epidemiology MH - Predictive Value of Tests MH - Prospective Studies MH - *Prostate-Specific Antigen/analysis/blood MH - Prostatectomy/methods/statistics & numerical data MH - *Prostatic Neoplasms/blood/epidemiology/pathology/surgery OTO - NOTNLM OT - Obesity OT - PSA OT - Prostate cancer EDAT- 2021/02/02 06:00 MHDA- 2022/01/11 06:00 CRDT- 2021/02/01 06:05 PHST- 2020/09/25 00:00 [received] PHST- 2020/12/07 00:00 [accepted] PHST- 2021/02/02 06:00 [pubmed] PHST- 2022/01/11 06:00 [medline] PHST- 2021/02/01 06:05 [entrez] AID - 10.1007/s00345-020-03557-6 [pii] AID - 10.1007/s00345-020-03557-6 [doi] PST - ppublish SO - World J Urol. 2021 Aug;39(8):2945-2951. doi: 10.1007/s00345-020-03557-6. Epub 2021 Feb 1. PMID- 20850284 OWN - NLM STAT- MEDLINE DCOM- 20110323 LR - 20131121 IS - 1769-664X (Electronic) IS - 0929-693X (Linking) VI - 17 IP - 11 DP - 2010 Nov TI - [Acute inflammatory polyradiculoneuropathy and membranous glomerulonephritis following Epbstein-Barr virus primary infection in a 12-year-old girl]. PG - 1535-9 LID - 10.1016/j.arcped.2010.08.007 [doi] AB - Acute inflammatory polyradiculoneuropathy, or Guillain-Barré syndrome (GBS), is characterized by peripheral nerve demyelination, which leads to rapidly progressive weakness, loss of sensation, and loss of deep tendon reflexes. It is a prototype of postinfectious autoimmune disease, whose pathophysiology is well described in the forms provoked by certain bacteria (molecular mimicry with Campylobacter jejuni), but remains unclear for the forms related to other organisms (cytomegalovirus, Epstein-Barr virus and other herpes group viruses, Mycoplasma pneumoniae). Glomerular lesions can be associated with the neurological symptoms and have also been described after various infections, independently of any signs of polyradiculoneuropathy. We report the observation of a 12-year-old girl who presented with Guillain-Barré syndrome with facial diplegia, ataxia, and intracranial hypertension following Epstein-Barr virus (EBV) primary infection. During the course of the neurological disease, membranous glomerulonephritis (MGN) was diagnosed. The neurological impairment was regressive within 6 months after intravenous immunoglobulin treatment followed by intravenous then oral corticosteroid administration. Viremia remained high more than 6 months after the onset of symptoms. Glomerulopathy progressed independently and finally required immunosuppressant medication with cyclosporine. EBV might be the factor that triggered the autoimmune disorders, as previously reported for systemic lupus erythematosus and multiple sclerosis in children. To the best of our knowledge, this association of 3 conditions (GBS, MGN, and EBV primary infection) has never been reported in the literature. CI - Copyright © 2010 Elsevier Masson SAS. All rights reserved. FAU - Meyer, P AU - Meyer P AD - Service de neuropédiatrie, hôpital Gui-de-Chauliac, CHU de Montpellier, université Montpellier 1, 80, avenue Fliche, 34295 Montpellier cedex 5, France. FAU - Soëte, S AU - Soëte S FAU - Raynaud, P AU - Raynaud P FAU - Henry, V AU - Henry V FAU - Morin, D AU - Morin D FAU - Rodière, M AU - Rodière M FAU - Rivier, F AU - Rivier F FAU - Roubertie, A AU - Roubertie A LA - fre PT - Case Reports PT - English Abstract PT - Journal Article TT - Polyradiculonévrite aiguë et glomérulonéphrite extramembraneuse au décours d'une primo-infection à Epstein-Barr virus chez une patiente de 12 ans. DEP - 20100917 PL - France TA - Arch Pediatr JT - Archives de pediatrie : organe officiel de la Societe francaise de pediatrie JID - 9421356 RN - 0 (Glucocorticoids) RN - 0 (Immunoglobulins) RN - 0 (Immunologic Factors) RN - 0 (Immunosuppressive Agents) RN - 83HN0GTJ6D (Cyclosporine) SB - IM MH - Ataxia/virology MH - Child MH - Cyclosporine/therapeutic use MH - Drug Therapy, Combination MH - Epstein-Barr Virus Infections/*complications/diagnosis/drug therapy/immunology MH - Facial Paralysis/virology MH - Female MH - Glomerulonephritis, Membranous/diagnosis/drug therapy/immunology/*virology MH - Glucocorticoids/therapeutic use MH - Guillain-Barre Syndrome/diagnosis/drug therapy/immunology/*virology MH - *Herpesvirus 4, Human/immunology MH - Humans MH - Immunoglobulins/therapeutic use MH - Immunologic Factors/therapeutic use MH - Immunosuppressive Agents/therapeutic use MH - Intracranial Hypertension/virology MH - Treatment Outcome EDAT- 2010/09/21 06:00 MHDA- 2011/03/24 06:00 CRDT- 2010/09/21 06:00 PHST- 2009/04/21 00:00 [received] PHST- 2009/09/21 00:00 [revised] PHST- 2010/08/05 00:00 [accepted] PHST- 2010/09/21 06:00 [entrez] PHST- 2010/09/21 06:00 [pubmed] PHST- 2011/03/24 06:00 [medline] AID - S0929-693X(10)00343-X [pii] AID - 10.1016/j.arcped.2010.08.007 [doi] PST - ppublish SO - Arch Pediatr. 2010 Nov;17(11):1535-9. doi: 10.1016/j.arcped.2010.08.007. Epub 2010 Sep 17. PMID- 33123789 OWN - NLM STAT- MEDLINE DCOM- 20210415 LR - 20210731 IS - 1432-1084 (Electronic) IS - 0938-7994 (Linking) VI - 31 IP - 5 DP - 2021 May TI - Predictive factors of severe abdominal pain during and after transarterial chemoembolization for hepatocellular carcinoma. PG - 3267-3275 LID - 10.1007/s00330-020-07404-5 [doi] AB - OBJECTIVES: To prospectively assess the frequency of severe abdominal pain during and after transarterial chemoembolization (TACE) for hepatocellular carcinoma (HCC) using the visual analog scale (VAS), and to identify predictive factors. METHODS: Ninety-eight TACE performed in 80 patients (mean 65 ± 12 years old, 60 men) were consecutively and prospectively included. Abdominal pain was considered severe if the VAS ≥ 30/100 after treatment administration, or if opioid analgesic (grades 2-3) intake was required during hospitalization. Patient and tumor characteristics as well as technical factors associated with severe pain were identified by binary logistic regression. RESULTS: The criterion for severe pain was met in 41/98 (42%) of procedures (peri-procedural pain 30/98 [31%] and opioid consumption during hospitalization 24/98 [25%]). Multivariate analysis identified age (odds ratio [OR] = 0.943 (95% confidence interval 0.895-0.994), p = 0.029), cirrhosis (OR = 0.284 (0.083-0.971), p = 0.045), and alcoholic liver disease (OR = 0.081 (0.010-0.659), p = 0.019) as negative predictive factors of severe abdominal pain. Severe abdominal pain occurred in or after 1/13 (8%), 8/34 (24%), 22/41 (54%), and 10/10 (100%) TACE sessions when none, one, two, and three of the protective factors were absent, respectively (p < 0.001). The area under the ROC curve of the combination of factors for the prediction of severe abdominal pain was 0.779 (CI 0.687-0.871). CONCLUSION: Severe abdominal pain was frequent during and after TACE revealing a clinically relevant and underestimated problem. A predictive model based on three readily available clinical variables suggests that young patients without alcoholic liver disease or cirrhosis could benefit from reinforced analgesia. KEY POINTS: • Severe abdominal pain occurs in 43% of TACE for HCC. • Younger age, absence of cirrhosis, and absence of alcoholic liver disease were identified as independent predictive factors of severe abdominal pain. • A simple combination of the three abovementioned features helped predict the occurrence of severe abdominal pain. FAU - Pachev, Atanas AU - Pachev A AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Paulatto, Luisa AU - Paulatto L AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Dioguardi Burgio, Marco AU - Dioguardi Burgio M AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. AD - INSERM U1149, CRI, Paris, France. FAU - Roche, Vincent AU - Roche V AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. AD - Université de Paris, Paris, France. FAU - Garcia Alba, Carmela AU - Garcia Alba C AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Sibert, Annie AU - Sibert A AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Lagadec, Matthieu AU - Lagadec M AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Kavafyan-Lasserre, Juliette AU - Kavafyan-Lasserre J AD - Department of Anaesthesiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Paugam-Burtz, Catherine AU - Paugam-Burtz C AD - Université de Paris, Paris, France. AD - Department of Anaesthesiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. FAU - Vilgrain, Valérie AU - Vilgrain V AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. AD - INSERM U1149, CRI, Paris, France. AD - Université de Paris, Paris, France. FAU - Ronot, Maxime AU - Ronot M AUID- ORCID: 0000-0001-7464-3939 AD - Department of Radiology, APHP.Nord, Hôpital Beaujon, Clichy, Hauts-de-Seine, France. maxime.ronot@aphp.fr. AD - INSERM U1149, CRI, Paris, France. maxime.ronot@aphp.fr. AD - Université de Paris, Paris, France. maxime.ronot@aphp.fr. LA - eng PT - Journal Article DEP - 20201029 PL - Germany TA - Eur Radiol JT - European radiology JID - 9114774 SB - IM EIN - Eur Radiol. 2021 Jun;31(6):4405. doi: 10.1007/s00330-020-07506-0. PMID: 33245497 MH - Abdominal Pain/epidemiology/etiology MH - Aged MH - *Carcinoma, Hepatocellular/complications/therapy MH - *Chemoembolization, Therapeutic/adverse effects MH - Humans MH - *Liver Neoplasms/therapy MH - Male MH - Middle Aged MH - Retrospective Studies MH - Risk Factors MH - Treatment Outcome OTO - NOTNLM OT - Analgesics, opioid OT - Chemoembolization, therapeutic OT - Liver cirrhosis OT - Pain, measurement OT - Pain, procedural EDAT- 2020/10/31 06:00 MHDA- 2021/04/16 06:00 CRDT- 2020/10/30 05:57 PHST- 2020/05/27 00:00 [received] PHST- 2020/10/09 00:00 [accepted] PHST- 2020/09/03 00:00 [revised] PHST- 2020/10/31 06:00 [pubmed] PHST- 2021/04/16 06:00 [medline] PHST- 2020/10/30 05:57 [entrez] AID - 10.1007/s00330-020-07404-5 [pii] AID - 10.1007/s00330-020-07404-5 [doi] PST - ppublish SO - Eur Radiol. 2021 May;31(5):3267-3275. doi: 10.1007/s00330-020-07404-5. Epub 2020 Oct 29. PMID- 39899882 OWN - NLM STAT- MEDLINE DCOM- 20250501 LR - 20250501 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 145 IP - 18 DP - 2025 May 1 TI - Reactive oxygen species regulate early development of the intestinal macrophage-microbiome interface. PG - 2025-2040 LID - 10.1182/blood.2024025240 [doi] AB - The controlled development of cellular intestinal immunity in the face of dynamic microbiota emergence constitutes a major challenge in very early life and is a bottleneck for sustained growth and well-being. Early-onset inflammatory bowel disease (IBD) represents an extreme disturbance of intestinal immunity. It is a hallmark and often the first manifestation of chronic granulomatous disease (CGD), caused by inborn defects in the nicotinamide adenine dinucleotide phosphate oxidase 2 (NOX2) in phagocytes and thus the failure to produce reactive oxygen species (ROS). However, in contrast to the known role of ROS in antimicrobial defense, the mechanisms underlying intestinal immunopathology in CGD remain enigmatic. This is partly due to the incomplete recapitulation of the CGD-IBD phenotype in established mouse models. We found that mice deficient in the NOX2 subunits p47phox or gp91phox showed similar baseline disturbances in lamina propria macrophage differentiation but responded differently to chemically induced colitis. Although p47phox- and gp91phox-deficient mice differed markedly in microbiota composition, crossfostering failed to equalize discrepant IBD phenotypes and microbiota, pointing at extremely early and functionally important microbiota fixation under specific pathogen-free housing conditions. In contrast, neonatal acquisition of a complex wild-mouse microbiota triggered spontaneous IBD, granuloma formation, and secondary sepsis with intestinal pathogens in both NOX2-deficient mouse lines, which was in part dependent on NOX2 in intestinal macrophages. Thus, in experimental CGD, the aberrant development of tissue immunity and microbiota are closely intertwined immediately after birth. CI - © 2025 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies. FAU - Mansoori Moghadam, Zohreh AU - Mansoori Moghadam Z AUID- ORCID: 0009-0000-9751-7740 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Faculty of Biology, University of Freiburg, Freiburg, Germany. FAU - Zhao, Bei AU - Zhao B AUID- ORCID: 0000-0002-2888-4472 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Department of Microbiome Research, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany. AD - Department of Medicine II, Faculty of Medicine, Medical Center-University of Freiburg, Freiburg, Germany. FAU - Raynaud, Candice AU - Raynaud C AUID- ORCID: 0009-0000-9383-5581 AD - Functional Microbiome Research Group, Institute of Medical Microbiology, Rheinisch-Westfälische Technische Hochschule Aachen University Hospital, Aachen, Germany. FAU - Strohmeier, Valentina AU - Strohmeier V AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Neuber, Jana AU - Neuber J AUID- ORCID: 0009-0009-5928-2421 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Faculty of Biology, University of Freiburg, Freiburg, Germany. FAU - Lösslein, Anne Kathrin AU - Lösslein AK AUID- ORCID: 0000-0002-0570-5902 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Qureshi, Sabrina AU - Qureshi S AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Gres, Vitka AU - Gres V AUID- ORCID: 0000-0002-5034-4691 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Ziegelbauer, Tara AU - Ziegelbauer T AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Baasch, Sebastian AU - Baasch S AUID- ORCID: 0000-0003-4378-3474 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Freiburg Institute for Advanced Studies, University of Freiburg, Freiburg, Germany. FAU - Schell, Christoph AU - Schell C AD - Institute of Surgical Pathology, Faculty of Medicine, University Medical Center Freiburg, Freiburg, Germany. FAU - Warnatz, Klaus AU - Warnatz K AUID- ORCID: 0000-0002-1172-865X AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Department of Rheumatology and Clinical Immunology, Medical Center-University of Freiburg, Freiburg, Germany. AD - Department of Clinical Immunology, University Hospital Zurich, Zurich, Switzerland. FAU - Inohara, Naohiro AU - Inohara N AD - Department of Pathology and Rogel Cancer Center, University of Michigan, Ann Arbor, MI. FAU - Nuñez, Gabriel AU - Nuñez G AD - Department of Pathology and Rogel Cancer Center, University of Michigan, Ann Arbor, MI. FAU - Clavel, Thomas AU - Clavel T AUID- ORCID: 0000-0002-7229-5595 AD - Functional Microbiome Research Group, Institute of Medical Microbiology, Rheinisch-Westfälische Technische Hochschule Aachen University Hospital, Aachen, Germany. FAU - Rosshart, Stephan P AU - Rosshart SP AUID- ORCID: 0000-0002-6989-8686 AD - Department of Microbiome Research, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany. AD - Department of Medicine II, Faculty of Medicine, Medical Center-University of Freiburg, Freiburg, Germany. AD - Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany. FAU - Kolter, Julia AU - Kolter J AUID- ORCID: 0000-0002-3268-8124 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Henneke, Philipp AU - Henneke P AUID- ORCID: 0000-0001-7314-7984 AD - Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. AD - Institute for Infection Control and Prevention, Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany. LA - eng PT - Journal Article PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (Reactive Oxygen Species) RN - EC 1.6.3.- (NADPH Oxidase 2) RN - EC 1.6.3.- (NADPH Oxidases) RN - EC 1.6.3.- (Cybb protein, mouse) RN - EC 1.6.3.1 (neutrophil cytosolic factor 1) SB - IM CIN - Blood. 2025 May 1;145(18):1966-1967. doi: 10.1182/blood.2025028350. PMID: 40310662 MH - Animals MH - *Reactive Oxygen Species/metabolism/immunology MH - Mice MH - NADPH Oxidase 2/genetics MH - *Macrophages/immunology/metabolism/pathology MH - NADPH Oxidases/genetics MH - *Gastrointestinal Microbiome/immunology MH - Granulomatous Disease, Chronic/immunology/genetics/microbiology/pathology MH - Mice, Knockout MH - Inflammatory Bowel Diseases/immunology/microbiology/genetics/pathology MH - Mice, Inbred C57BL MH - *Intestines/microbiology/immunology MH - Colitis/chemically induced/immunology/microbiology/pathology/genetics EDAT- 2025/02/03 18:20 MHDA- 2025/05/01 12:30 CRDT- 2025/02/03 17:13 PHST- 2025/01/01 00:00 [accepted] PHST- 2024/05/02 00:00 [received] PHST- 2025/05/01 12:30 [medline] PHST- 2025/02/03 18:20 [pubmed] PHST- 2025/02/03 17:13 [entrez] AID - 535387 [pii] AID - 10.1182/blood.2024025240 [doi] PST - ppublish SO - Blood. 2025 May 1;145(18):2025-2040. doi: 10.1182/blood.2024025240. PMID- 38750824 OWN - NLM STAT- MEDLINE DCOM- 20250127 LR - 20250127 IS - 1097-6825 (Electronic) IS - 0091-6749 (Linking) VI - 154 IP - 3 DP - 2024 Sep TI - Loss of the TRPM4 channel in humans causes immune dysregulation with defective monocyte migration. PG - 792-806 LID - S0091-6749(24)00466-4 [pii] LID - 10.1016/j.jaci.2024.02.026 [doi] AB - BACKGROUND: TRPM4 is a broadly expressed, calcium-activated, monovalent cation channel that regulates immune cell function in mice and cell lines. Clinically, however, partial loss- or gain-of-function mutations in TRPM4 lead to arrhythmia and heart disease, with no documentation of immunologic disorders. OBJECTIVE: To characterize functional cellular mechanisms underlying the immune dysregulation phenotype in a proband with a mutated TRPM4 gene. METHODS: We employed a combination of biochemical, cell biological, imaging, omics analyses, flow cytometry, and gene editing approaches. RESULTS: We report the first human cases to our knowledge with complete loss of the TRPM4 channel, leading to immune dysregulation with frequent bacterial and fungal infections. Single-cell and bulk RNA sequencing point to altered expression of genes affecting cell migration, specifically in monocytes. Inhibition of TRPM4 in T cells and the THP-1 monocyte cell line reduces migration. More importantly, primary T cells and monocytes from TRPM4 patients migrate poorly. Finally, CRISPR knockout of TRPM4 in THP-1 cells greatly reduces their migration potential. CONCLUSION: Our results demonstrate that TRPM4 plays a critical role in regulating immune cell migration, leading to increased susceptibility to infections. CI - Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Yu, Fang AU - Yu F AD - Calcium Signaling Group, Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar; Department of Physiology and Biophysics, Weill Cornell Medicine, New York, NY. FAU - Hubrack, Satanay AU - Hubrack S AD - Research Department, Sidra Medicine, Doha, Qatar. FAU - Raynaud, Christophe M AU - Raynaud CM AD - Research Department, Sidra Medicine, Doha, Qatar. FAU - Elmi, Asha AU - Elmi A AD - Research Department, Sidra Medicine, Doha, Qatar. FAU - Mackeh, Rafah AU - Mackeh R AD - Research Department, Sidra Medicine, Doha, Qatar. FAU - Agrebi, Nourhen AU - Agrebi N AD - Research Department, Sidra Medicine, Doha, Qatar. FAU - Thareja, Gaurav AU - Thareja G AD - Department of Physiology and Biophysics, Weill Cornell Medicine, New York, NY; Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar. FAU - Belkadi, Abdelaziz AU - Belkadi A AD - Department of Physiology and Biophysics, Weill Cornell Medicine, New York, NY; Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar. FAU - Al Saloos, Hesham AU - Al Saloos H AD - Division of Cardiology, Sidra Medicine, Doha, Qatar. FAU - Ahmed, Ayeda Abdulsalam AU - Ahmed AA AD - Genomics Core, Weill Cornell Medicine-Qatar, Doha, Qatar. FAU - Purayil, Saleema C AU - Purayil SC AD - Allergy & Immunology Division, Department of Medicine, Hamad Medical Corporation, Doha, Qatar. FAU - Mohamoud, Yasmin A AU - Mohamoud YA AD - Genomics Core, Weill Cornell Medicine-Qatar, Doha, Qatar. FAU - Suhre, Karsten AU - Suhre K AD - Department of Physiology and Biophysics, Weill Cornell Medicine, New York, NY; Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar. FAU - Abi Khalil, Charbel AU - Abi Khalil C AD - Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar; Heart Hospital, Hamad Medical Corporation, Doha, Qatar. FAU - Schmidt, Frank AU - Schmidt F AD - Research Department, Sidra Medicine, Doha, Qatar; Department of Biochemistry, Weill Cornell Medicine, New York, NY. FAU - Lo, Bernice AU - Lo B AD - Research Department, Sidra Medicine, Doha, Qatar; College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar. Electronic address: blo@sidra.org. FAU - Hassan, Amel AU - Hassan A AD - Pediatric Allergy and Immunology Department, Sidra Medicine, Doha, Qatar. Electronic address: ahassan2@sidra.org. FAU - Machaca, Khaled AU - Machaca K AD - Calcium Signaling Group, Research Department, Weill Cornell Medicine-Qatar, Doha, Qatar; Department of Physiology and Biophysics, Weill Cornell Medicine, New York, NY. Electronic address: khm2002@qatar-med.cornell.edu. LA - eng PT - Case Reports PT - Journal Article DEP - 20240514 PL - United States TA - J Allergy Clin Immunol JT - The Journal of allergy and clinical immunology JID - 1275002 RN - 0 (TRPM4 protein, human) RN - 0 (TRPM Cation Channels) RN - SY7Q814VUP (Calcium) SB - IM MH - Humans MH - Child, Preschool MH - Child MH - Male MH - Female MH - *Cell Movement MH - *Monocytes/cytology/immunology/metabolism MH - *TRPM Cation Channels/genetics/immunology/metabolism MH - Mutation MH - Pedigree MH - Calcium/metabolism MH - Single-Cell Gene Expression Analysis MH - THP-1 Cells MH - T-Lymphocytes/metabolism MH - Homozygote MH - Infections/genetics/immunology OTO - NOTNLM OT - TRPM4 OT - immune dysregulation OT - infections OT - loss of function OT - migration OT - monocyte COIS- Disclosure statement The Weill Cornell Medicine Qatar Cores are supported by the Biomedical Research Program at Weill Cornell Medicine Qatar, a program funded by Qatar Foundation. This publication was made possible by Path Towards Precision Medicine fourth Cycle grant PPM 04-0128-200015 from the Qatar National Research Fund (a member of Qatar Foundation). The findings herein reflect the work and are solely the responsibility of the authors. This work was also supported by Sidra Medicine and the Biomedical Research Program at Weill Cornell Medicine–Qatar, a program funded by Qatar Foundation. Disclosure of potential conflicts of interest: The authors declare that they have no relevant conflicts of interest. EDAT- 2024/05/16 06:42 MHDA- 2024/09/07 14:43 CRDT- 2024/05/16 00:29 PHST- 2023/06/07 00:00 [received] PHST- 2024/01/30 00:00 [revised] PHST- 2024/02/06 00:00 [accepted] PHST- 2024/09/07 14:43 [medline] PHST- 2024/05/16 06:42 [pubmed] PHST- 2024/05/16 00:29 [entrez] AID - S0091-6749(24)00466-4 [pii] AID - 10.1016/j.jaci.2024.02.026 [doi] PST - ppublish SO - J Allergy Clin Immunol. 2024 Sep;154(3):792-806. doi: 10.1016/j.jaci.2024.02.026. Epub 2024 May 14. PMID- 38937956 OWN - NLM STAT- MEDLINE DCOM- 20240801 LR - 20260518 IS - 1464-360X (Electronic) IS - 1101-1262 (Print) IS - 1101-1262 (Linking) VI - 34 IP - 4 DP - 2024 Aug 1 TI - Social inequalities in medical appointment cancellations and reschedulings at the onset of the COVID-19 epidemic in France. PG - 652-659 LID - 10.1093/eurpub/ckae101 [doi] AB - Inconsistent results are found regarding social inequalities related to healthcare appointment cancellations during the COVID-19 crisis. Whether rescheduling was associated with social status is unknown. By studying both cancellations and rescheduling, we comprehensively describe which social groups were affected by care disruption. First follow-up of a random population-based cohort was used, including 95 118 people aged 18 or older at baseline and who live in France. Poisson and multinomial regressions were used to study social factors associated with experiencing both medical appointment cancellation by health professionals during the first COVID-19 lockdown, and rescheduling within six months. Among all individuals (including those without scheduled appointment), 21.1% reported cancellations initiated by healthcare professionals. Women, the richest, and those with a chronic disease were the most affected by these cancellations. Although 78.1% who had their appointment cancelled obtained a new appointment within six months, 6.6% failed to reschedule and 15.2% did not want to reschedule. While the oldest were more likely to reschedule, regardless of their health status, the poorest and those with multiple chronic diseases were less likely to do so. Difficulties in rescheduling revealed certain social groups were ultimately more penalized by the restriction of access to care during the first wave of the COVID-19 pandemic. Given that the poorest people, a social group that is in poorer health condition compared to other groups, were the most affected, our results raise questions about the ability of the healthcare system to reduce social health inequalities during a major health crisis. CI - © The Author(s) 2024. Published by Oxford University Press on behalf of the European Public Health Association. FAU - Pousson, Jeanna-Eve AU - Pousson JE AUID- ORCID: 0000-0002-3585-8643 AD - INSERM-IRIS (UMR8156 - U997), INSERM, Aubervilliers, France. FAU - Jusot, Florence AU - Jusot F AD - Health Department, Université Paris Dauphine, 8 Boulevard Lannes, Paris, 75116, France. FAU - Silberzan, Léna AU - Silberzan L AD - INSERM-IRIS (UMR8156 - U997), INSERM, Aubervilliers, France. FAU - Bajos, Nathalie AU - Bajos N AD - INSERM-IRIS (UMR8156 - U997), INSERM, Aubervilliers, France. AD - IRIS, EHESS, 25 Rue du Pilier, Aubervilliers, 93322, France. CN - EpiCov Study Group LA - eng GR - INSERM/ GR - French Ministry of Research/ GR - Institut National de la Santé et de la Recherche Médicale/ GR - French Ministry of Research, by Drees-Direction de la Recherche/ GR - des Etudes, de l'Évaluation et des Statistiques/ GR - French Ministry of Health/ GR - ERC-2019-SyG/Gender and Health Inequalities/ GR - ERC_/European Research Council/International GR - 856478/European Union's Horizon 2020/ GR - Connecting European Cohorts to Increase Common and Effective Response/ PT - Journal Article PL - England TA - Eur J Public Health JT - European journal of public health JID - 9204966 SB - IM MH - Humans MH - *COVID-19/epidemiology MH - France/epidemiology MH - Female MH - Male MH - Middle Aged MH - *Appointments and Schedules MH - Adult MH - *SARS-CoV-2 MH - Aged MH - *Socioeconomic Factors MH - Adolescent MH - Young Adult MH - Pandemics MH - Healthcare Disparities/statistics & numerical data MH - Health Services Accessibility/statistics & numerical data PMC - PMC11293806 COIS- None declared. FIR - Bagein, Guillaume IR - Bagein G FIR - Counil, Emilie IR - Counil E FIR - Jusot, Florence IR - Jusot F FIR - Lydie, Nathalie IR - Lydie N FIR - Meyer, Laurence IR - Meyer L FIR - Raynaud, Philippe IR - Raynaud P FIR - Rouquette, Alexandra IR - Rouquette A FIR - Pailhé, Ariane IR - Pailhé A FIR - Rahib, Delphine IR - Rahib D FIR - Sillard, Patrick IR - Sillard P FIR - Spire, Alexis IR - Spire A EDAT- 2024/06/28 06:41 MHDA- 2024/08/01 18:43 PMCR- 2024/06/27 CRDT- 2024/06/28 01:33 PHST- 2024/08/01 18:43 [medline] PHST- 2024/06/28 06:41 [pubmed] PHST- 2024/06/28 01:33 [entrez] PHST- 2024/06/27 00:00 [pmc-release] AID - 7700795 [pii] AID - ckae101 [pii] AID - 10.1093/eurpub/ckae101 [doi] PST - ppublish SO - Eur J Public Health. 2024 Aug 1;34(4):652-659. doi: 10.1093/eurpub/ckae101. PMID- 33762475 OWN - NLM STAT- MEDLINE DCOM- 20211203 LR - 20211214 IS - 1745-7262 (Electronic) IS - 1008-682X (Print) IS - 1008-682X (Linking) VI - 23 IP - 5 DP - 2021 Sep-Oct TI - Relationship of preoperative androgen levels and metabolic syndrome with quality of life and erectile function in patients who are to undergo radical prostatectomy. PG - 520-526 LID - 10.4103/aja.aja_3_21 [doi] AB - This study aims to investigate whether clinical and biological preoperative characteristics of patients who were to undergo radical prostatectomy were associated with impairment in patient-reported quality of life (QoL) and erectile dysfunction immediately before intervention. We evaluated patient-reported outcomes among 1019 patients (out of 1343) of the AndroCan study, willing to score the Aging Male Symptom (AMS) and the International Index of Erectile Function 5-item (IIEF-5) auto-questionnaires. Univariate linear regression and robust multiple regression were used to ascertain the relationship between demographic, clinical, and hormonal parameters and global AMS or IIEF-5 scores. As a result, most patients (85.1') of the Androcan cohort agreed to complete questionnaires. Significantly higher IIEF-5 global scores were found in non-Caucasian and obese patients, with larger waist circumference, metabolic syndrome, diabetes mellitus, cardiovascular disease, hypertension, high blood sugar, concomitant medications, and hypogonadism, while the AMS global score was significantly higher in patients with larger waist circumference, metabolic syndrome, high blood pressure, raised glycemia, and concomitant medication. The IIEF-5 global score was correlated to age, dehydroepiandrosterone (DHEA), fat mass percentage, and androstenediol (D5). The AMS global score was significantly correlated to DHEA, D5, and DHEA sulfate. Finally, the multivariate models showed that QoL and erectile function were significantly affected, before surgery, by symptoms and signs that are usually considered as pertaining to the metabolic syndrome, while sexual hormones are essentially correlated to erectile dysfunction. FAU - Neuzillet, Yann AU - Neuzillet Y AD - Department of Urology, Hôpital Foch, UVSQ-Paris-Saclay University, Suresnes 92150, France. FAU - Dreyfus, Jean-François AU - Dreyfus JF AD - Department of Clinical Research and Innovation, Hôpital Foch, UVSQ-Paris-Saclay University, Suresnes 92150, France. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Sorbonne University, Paris 75013, France. FAU - Rouanne, Mathieu AU - Rouanne M AD - Department of Urology, Hôpital Foch, UVSQ-Paris-Saclay University, Suresnes 92150, France. FAU - Schneider, Marc AU - Schneider M AD - Department of Urology, Hôpital Louis Pasteur, Colmar 68000, France. FAU - Roupret, Morgan AU - Roupret M AD - Department of Urology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris 75013, France. FAU - Drouin, Sarah AU - Drouin S AD - Department of Urology, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris 75013, France. FAU - Galiano, Marc AU - Galiano M AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris 75014, France. FAU - Cathelineau, Xavier AU - Cathelineau X AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris 75014, France. FAU - Lebret, Thierry AU - Lebret T AD - Department of Urology, Hôpital Foch, UVSQ-Paris-Saclay University, Suresnes 92150, France. FAU - Botto, Henry AU - Botto H AD - Department of Urology, Hôpital Foch, UVSQ-Paris-Saclay University, Suresnes 92150, France. LA - eng PT - Journal Article PL - China TA - Asian J Androl JT - Asian journal of andrology JID - 100942132 RN - 0 (Androgens) SB - IM MH - Adult MH - Aged MH - Androgens/*analysis/blood MH - Erectile Dysfunction/*etiology/physiopathology MH - Humans MH - Male MH - Metabolic Syndrome/*complications/physiopathology MH - Middle Aged MH - Preoperative Period MH - Prostatectomy/methods/*standards/statistics & numerical data MH - Quality of Life/psychology MH - Severity of Illness Index MH - Surveys and Questionnaires PMC - PMC8451490 OTO - NOTNLM OT - erectile function OT - metabolic syndrome OT - quality of life OT - radical prostatectomy OT - testosterone COIS- None EDAT- 2021/03/26 06:00 MHDA- 2021/12/15 06:00 PMCR- 2021/03/05 CRDT- 2021/03/25 05:53 PHST- 2021/03/26 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/03/25 05:53 [entrez] PHST- 2021/03/05 00:00 [pmc-release] AID - 310859 [pii] AID - AJA-23-520 [pii] AID - 10.4103/aja.aja_3_21 [doi] PST - ppublish SO - Asian J Androl. 2021 Sep-Oct;23(5):520-526. doi: 10.4103/aja.aja_3_21. PMID- 30755657 OWN - NLM STAT- MEDLINE DCOM- 20200923 LR - 20260127 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 9 IP - 1 DP - 2019 Feb 12 TI - IL-24 contributes to skin inflammation in Para-Phenylenediamine-induced contact hypersensitivity. PG - 1852 LID - 10.1038/s41598-018-38156-4 [doi] LID - 1852 AB - Para-Phenylenediamine (PPD) is an aromatic amine used in hair dyes and in temporary black henna tattoos, which is a frequent cause of allergic contact dermatitis (ACD). ACD is a skin inflammatory reaction characterized by modifications such as spongiosis, exocytosis and acanthosis. The aim of this study is to characterize the expression and the role of IL-20-related cytokines, including IL-19, IL-20, IL-22 and IL-24, in ACD. The expression of IL19, IL20, IL22 and IL24 is increased in affected skin from PPD allergic patients compared with uninvolved skin. In addition, the expression of these cytokines positively correlates with clinical symptoms. To assess their role in ACD, we set up a mouse model of PPD-induced allergic contact dermatitis and we showed that, in contrast to Il22-deficient mice, Il22ra1-, Il20rb- and Il24-deficient mice are partially protected against development of PPD-induced contact hypersensitivity. These mice have decreased ear thickening and less acanthosis compared with WT mice after PPD treatment. In addition, the absence of IL-22R, IL-20R2 or IL-24 affects the recruitment of neutrophils into the skin but not the total IgE production. Taken together, these results demonstrate the implication of IL-24 via the IL-20R type II receptor in the inflammatory process of ACD. FAU - Van Belle, Astrid B AU - Van Belle AB AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Cochez, Perrine M AU - Cochez PM AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - de Heusch, Magali AU - de Heusch M AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Pointner, Lisa AU - Pointner L AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Opsomer, Remi AU - Opsomer R AD - Institut de Neurosciences, Université catholique de Louvain, Brussels, Belgium. FAU - Raynaud, Peggy AU - Raynaud P AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Achouri, Younes AU - Achouri Y AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Hendrickx, Emilie AU - Hendrickx E AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Cheou, Pamela AU - Cheou P AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Warnier, Guy AU - Warnier G AD - Ludwig Institute for Cancer Research, Brussels branch, Brussels, Belgium. FAU - Renauld, Jean-Christophe AU - Renauld JC AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. FAU - Baeck, Marie AU - Baeck M AD - Department of Dermatology, Cliniques Universitaires Saint-Luc, UCL, B-1200, Brussels, Belgium. AD - Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium. FAU - Dumoutier, Laure AU - Dumoutier L AD - de Duve Institute, Université catholique de Louvain, Brussels, Belgium. laure.dumoutier@uclouvain.be. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190212 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Coloring Agents) RN - 0 (Cytokines) RN - 37341-29-0 (Immunoglobulin E) RN - 0 (Interleukins) RN - EC 3.1.3.48 (Leukocyte Common Antigens) RN - 0 (Phenylenediamines) RN - 0 (Receptors, Interleukin) RN - 0 (Interleukin-22) RN - 0 (Interleukin-24) RN - 0 (Il24 protein, mouse) RN - 0 (interleukin-20 receptor) RN - 0 (interleukin-22 receptor) RN - U770QIT64J (4-phenylenediamine) RN - U91R7IMG8U (interleukin 20) SB - IM MH - Adult MH - Aged MH - Animals MH - Biopsy MH - Coloring Agents MH - Cytokines/*metabolism MH - Dermatitis, Allergic Contact/*metabolism MH - Disease Models, Animal MH - Humans MH - Immunoglobulin E/metabolism MH - Inflammation/*chemically induced/metabolism MH - Interleukins/*metabolism MH - Leukocyte Common Antigens/metabolism MH - Mice MH - Mice, Inbred BALB C MH - Mice, Inbred C57BL MH - Middle Aged MH - Phenylenediamines MH - Receptors, Interleukin/metabolism MH - Skin/*drug effects/metabolism MH - Interleukin-22 MH - Interleukin-24 PMC - PMC6372603 COIS- The authors declare no competing interests. EDAT- 2019/02/14 06:00 MHDA- 2020/09/24 06:00 PMCR- 2019/02/12 CRDT- 2019/02/14 06:00 PHST- 2018/06/28 00:00 [received] PHST- 2018/12/19 00:00 [accepted] PHST- 2019/02/14 06:00 [entrez] PHST- 2019/02/14 06:00 [pubmed] PHST- 2020/09/24 06:00 [medline] PHST- 2019/02/12 00:00 [pmc-release] AID - 10.1038/s41598-018-38156-4 [pii] AID - 38156 [pii] AID - 10.1038/s41598-018-38156-4 [doi] PST - epublish SO - Sci Rep. 2019 Feb 12;9(1):1852. doi: 10.1038/s41598-018-38156-4. PMID- 34154955 OWN - NLM STAT- MEDLINE DCOM- 20211210 LR - 20211214 IS - 1166-7087 (Print) IS - 1166-7087 (Linking) VI - 31 IP - 12 DP - 2021 Oct TI - Comparison between MRI and choline-PET trans-perineal target biopsies and saturation biopsies for detection and topography of intra-prostatic recurrence after primary radiation therapy for prostate cancer. PG - 683-691 LID - S1166-7087(21)00116-0 [pii] LID - 10.1016/j.purol.2021.04.008 [doi] AB - INTRODUCTION: Biochemical recurrence of prostate cancer after radiation therapy occurs in 5 to 50% of cases depending on the radiation technique used. The diagnosis of recurrence of prostate adenocarcinoma must be made accurately. The aim of this study was to compare transperineal saturation and target biopsies to index lesion (IL) as defined on MRI and 18FCholine PET-CT (PETc) for detection of intra-prostatic recurrence after primary radiation therapy for prostate cancer. MATERIALS AND METHODS: Thirty-eight patients with an history of prostate radiation for prostate cancer and biochemical recurrence were retrospectively included between March 2013 and June 2017. All patients had PETc and multiparametric MRI (MRI) defining IL. All patients had transperineal saturation biopsies and target biopsies the IL. RESULTS: Among 38 patients with biochemical recurrence, 33 (87%) had biopsy proven recurrence in the prostate. The sensitivity and specificity of MRI were 32% (SD:19%) and 91% respectively (SD:7%). The sensitivity and specificity of PETc were 33% (SD:22%) and 78% respectively (SD:12%). Saturation trans-perineal and target biopsies allowed detection of disease recurrence in 79% and 84% of patients, respectively. CONCLUSION: In case of positive imaging, both trans-perineal prostate saturation and target biopsies offer good performance to confirm intraprostatic recurrence. However, MRI and PETc low sensitivity to detect all sites of local recurrence of prostate cancer after radiation still justify the completion of systematic saturation biopsies. LEVEL OF EVIDENCE: 3. CI - Copyright © 2021 Elsevier Masson SAS. All rights reserved. FAU - Biscans, C AU - Biscans C AD - Service d'urologie et de transplantation rénale, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. Electronic address: cbiscans@gmail.com. FAU - Vallée, M AU - Vallée M AD - Service d'urologie et de transplantation rénale, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France; Inserm U1070, "Pharmacologie des Anti-Infectieux", UFR Médecine-Pharmacie, Université de Poitiers, Pôle Biologie Sante, 1, rue Georges Bonnet, Bâtiment B36 TSA 51106, 86073 Poitiers Cedex 9, France. FAU - Ingrand, P AU - Ingrand P AD - Service de biostatistiques, faculté de médecine de Potiers CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. FAU - Debiais-Delpech, C AU - Debiais-Delpech C AD - Service d'Anatomopathologie, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. FAU - Raynaud, N AU - Raynaud N AD - Service de radiologie, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. FAU - Chalhoub, K AU - Chalhoub K AD - Service d'urologie et de transplantation rénale, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. FAU - Guérif, S AU - Guérif S AD - Service d'oncologie radiothérapie, unité de curiethérapie, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France. LA - eng PT - Journal Article DEP - 20210618 PL - France TA - Prog Urol JT - Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologie JID - 9307844 RN - 0 (Radiopharmaceuticals) RN - N91BDP6H0X (Choline) SB - IM MH - Biopsy MH - *Choline MH - Humans MH - Magnetic Resonance Imaging MH - Male MH - Neoplasm Recurrence, Local/diagnostic imaging MH - Positron Emission Tomography Computed Tomography MH - Prostate/diagnostic imaging MH - *Prostatic Neoplasms/diagnostic imaging/radiotherapy MH - Radiopharmaceuticals MH - Retrospective Studies OTO - NOTNLM OT - Biochemical recurrence OT - Biopsie prostate OT - Cancer de la prostate OT - IRM OT - MRI OT - Post-irradiation OT - Prostate biopsy OT - Prostate cancer OT - Récidive biologique OT - Salvage treatment OT - TEP Choline OT - TEP choline OT - Traitement de rattrapge OT - Trans-perineal OT - Trans-périénale EDAT- 2021/06/23 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/06/22 05:51 PHST- 2020/07/20 00:00 [received] PHST- 2021/04/07 00:00 [revised] PHST- 2021/04/30 00:00 [accepted] PHST- 2021/06/23 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/06/22 05:51 [entrez] AID - S1166-7087(21)00116-0 [pii] AID - 10.1016/j.purol.2021.04.008 [doi] PST - ppublish SO - Prog Urol. 2021 Oct;31(12):683-691. doi: 10.1016/j.purol.2021.04.008. Epub 2021 Jun 18. PMID- 34217607 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20231107 IS - 1532-1770 (Electronic) IS - 1521-6942 (Print) IS - 1521-6942 (Linking) VI - 35 IP - 3 DP - 2021 Sep TI - Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: An introduction by The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS). PG - 101695 LID - S1521-6942(21)00037-1 [pii] LID - 10.1016/j.berh.2021.101695 [doi] AB - Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being. Physical activity and exercise are globally recommended; however, for connective tissue diseases, this guidance carries greater impact on inflammatory disease manifestations, recovery, and cardiovascular health. Exercise, through myogenic and vascular phenomena, naturally targets key pathogenic drivers by downregulating multiple inflammatory and fibrotic pathways in serum and tissue, while increasing circulation and vascular repair. G-FoRSS, The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis recognizes the scientific basis of and advocates for education and research of exercise as a systemic and targeted SSc disease-modifying treatment. An overview of biophysiological mechanisms of physical activity and exercise are herein imparted for patients, clinicians, and researchers, and applied to SSc disease mechanisms, manifestations, and impairment. A preliminary guidance on exercise in SSc, a research agenda, and the current state of research and outcome measures are set forth. CI - Copyright © 2021 Elsevier Ltd. All rights reserved. FAU - Pettersson, Henrik AU - Pettersson H AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Division of Rheumatology, Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Alexanderson, Helene AU - Alexanderson H AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Division of Rheumatology, Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Poole, Janet L AU - Poole JL AD - Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, USA. FAU - Varga, Janos AU - Varga J AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Regardt, Malin AU - Regardt M AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Occupational Therapy, Karolinska Institutet, Stockholm, Sweden. FAU - Russell, Anne-Marie AU - Russell AM AD - University of Exeter, College of Medicine and Health, Exeter, UK; National Institute of Health Research, Senior Nurse Research Leader, London, UK. FAU - Salam, Yasser AU - Salam Y AD - Department of Physical Therapy, University of North Texas Health Science Center, Fort Worth, TX, USA. FAU - Jensen, Kelly AU - Jensen K AD - Oregon Health and Science University, Portland, OR, USA; New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA. FAU - Mansour, Jennifer AU - Mansour J AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA. FAU - Frech, Tracy AU - Frech T AD - Vanderbilt University, Division of Rheumatology, Nashville, TN, USA. FAU - Feghali-Bostwick, Carol AU - Feghali-Bostwick C AD - Department of Medicine, Medical University of South Carolina, Charleston, SC, USA. FAU - Varjú, Cecília AU - Varjú C AD - Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. FAU - Baldwin, Nancy AU - Baldwin N AD - Scleroderma Foundation, Chicago, IL, USA. FAU - Heenan, Matty AU - Heenan M AD - Scleroderma Foundation/Pulmonary Hypertension Association, Tucson, AZ, USA. FAU - Fligelstone, Kim AU - Fligelstone K AD - Scleroderma & Raynaud Society UK (SRUK), London, UK; Royal Free Hospital, London, UK. FAU - Holmner, Monica AU - Holmner M AD - The Swedish Rheumatism Association National Association for Systemic Sclerosis, Sweden. FAU - Lammi, Matthew R AU - Lammi MR AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA. FAU - Scholand, Mary Beth AU - Scholand MB AD - University of Utah, Division of Pulmonary Medicine, Pulmonary Fibrosis Center, Salt Lake City, UT, USA. FAU - Shapiro, Lee AU - Shapiro L AD - Division of Rheumatology, Albany Medical Center, Albany, NY, USA; Steffens Scleroderma Foundation, Albany, NY, USA. FAU - Volkmann, Elizabeth R AU - Volkmann ER AD - University of California, David Geffen School of Medicine, UCLA Scleroderma Program and UCLA CTD-ILD Program, Division of Rheumatology, Department of Medicine, Los Angeles, CA, USA. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA. Electronic address: lsaketk@tulane.edu. LA - eng GR - K23 HL150237/HL/NHLBI NIH HHS/United States GR - K24 AR060297/AR/NIAMS NIH HHS/United States GR - L30 HL129466/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20210701 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Activities of Daily Living MH - Exercise MH - *Fellowships and Scholarships MH - Fibrosis MH - Humans MH - *Scleroderma, Systemic/therapy PMC - PMC8478716 MID - NIHMS1709700 OTO - NOTNLM OT - Disability OT - Exercise OT - Health-related quality of life OT - Interstitial lung disease OT - Muscle OT - Myokine OT - Physical activity OT - Pulmonary hypertension OT - Pulmonary rehabilitation OT - Scleroderma COIS- Declaration of competing interest None of the authors have conflicts to disclose in relation to this publication. EDAT- 2021/07/05 06:00 MHDA- 2021/11/03 06:00 PMCR- 2022/09/01 CRDT- 2021/07/04 20:35 PHST- 2021/07/05 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] PHST- 2021/07/04 20:35 [entrez] PHST- 2022/09/01 00:00 [pmc-release] AID - S1521-6942(21)00037-1 [pii] AID - 10.1016/j.berh.2021.101695 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101695. doi: 10.1016/j.berh.2021.101695. Epub 2021 Jul 1. PMID- 26502155 OWN - NLM STAT- MEDLINE DCOM- 20160412 LR - 20250529 IS - 1552-4469 (Electronic) IS - 1552-4450 (Print) IS - 1552-4450 (Linking) VI - 11 IP - 12 DP - 2015 Dec TI - A selective chemical probe for exploring the role of CDK8 and CDK19 in human disease. PG - 973-980 LID - 10.1038/nchembio.1952 [doi] AB - There is unmet need for chemical tools to explore the role of the Mediator complex in human pathologies ranging from cancer to cardiovascular disease. Here we determine that CCT251545, a small-molecule inhibitor of the WNT pathway discovered through cell-based screening, is a potent and selective chemical probe for the human Mediator complex-associated protein kinases CDK8 and CDK19 with >100-fold selectivity over 291 other kinases. X-ray crystallography demonstrates a type 1 binding mode involving insertion of the CDK8 C terminus into the ligand binding site. In contrast to type II inhibitors of CDK8 and CDK19, CCT251545 displays potent cell-based activity. We show that CCT251545 and close analogs alter WNT pathway-regulated gene expression and other on-target effects of modulating CDK8 and CDK19, including expression of genes regulated by STAT1. Consistent with this, we find that phosphorylation of STAT1(SER727) is a biomarker of CDK8 kinase activity in vitro and in vivo. Finally, we demonstrate in vivo activity of CCT251545 in WNT-dependent tumors. FAU - Dale, Trevor AU - Dale T AD - School of Bioscience, Cardiff University, Cardiff, UK. FAU - Clarke, Paul A AU - Clarke PA AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Esdar, Christina AU - Esdar C AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Waalboer, Dennis AU - Waalboer D AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Adeniji-Popoola, Olajumoke AU - Adeniji-Popoola O AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Ortiz-Ruiz, Maria-Jesus AU - Ortiz-Ruiz MJ AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Mallinger, Aurélie AU - Mallinger A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Samant, Rahul S AU - Samant RS AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Czodrowski, Paul AU - Czodrowski P AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Musil, Djordje AU - Musil D AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Schwarz, Daniel AU - Schwarz D AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Schneider, Klaus AU - Schneider K AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Stubbs, Mark AU - Stubbs M AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Ewan, Ken AU - Ewan K AD - School of Bioscience, Cardiff University, Cardiff, UK. FAU - Fraser, Elizabeth AU - Fraser E AD - School of Bioscience, Cardiff University, Cardiff, UK. FAU - TePoele, Robert AU - TePoele R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Court, Will AU - Court W AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Box, Gary AU - Box G AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - de Haven Brandon, Alexis AU - de Haven Brandon A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Gowan, Sharon AU - Gowan S AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Rohdich, Felix AU - Rohdich F AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Raynaud, Florence AU - Raynaud F AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Schneider, Richard AU - Schneider R AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Poeschke, Oliver AU - Poeschke O AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Blaukat, Andree AU - Blaukat A AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Workman, Paul AU - Workman P AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Schiemann, Kai AU - Schiemann K AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Eccles, Suzanne A AU - Eccles SA AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. FAU - Wienke, Dirk AU - Wienke D AD - Merck KGaA, Merck Serono, Darmstadt, Germany. FAU - Blagg, Julian AU - Blagg J AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, SW7 3RP. LA - eng SI - PubChem-Substance/252819210 SI - PubChem-Substance/252819211 SI - PubChem-Substance/252819212 SI - PubChem-Substance/252819213 SI - PubChem-Substance/252819214 SI - PubChem-Substance/252819215 SI - PubChem-Substance/252819216 SI - PubChem-Substance/252819217 SI - PubChem-Substance/252819218 SI - PubChem-Substance/252819219 SI - PubChem-Substance/252819220 SI - PubChem-Substance/252819221 GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - BB/D00117X/1/BB_/Biotechnology and Biological Sciences Research Council/United Kingdom GR - C368/A6743/CRUK_/Cancer Research UK/United Kingdom GR - 15937/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - A11566/CRUK_/Cancer Research UK/United Kingdom GR - A368/A7990/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20151026 PL - United States TA - Nat Chem Biol JT - Nature chemical biology JID - 101231976 RN - 0 (CCT251545) RN - 0 (Molecular Probes) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Pyridines) RN - 0 (Spiro Compounds) RN - EC 2.7.11.22 (CDK19 protein, human) RN - EC 2.7.11.22 (CDK8 protein, human) RN - EC 2.7.11.22 (Cyclin-Dependent Kinase 8) RN - EC 2.7.11.22 (Cyclin-Dependent Kinases) SB - IM MH - Cell Line, Tumor MH - Colonic Neoplasms/*drug therapy/*metabolism MH - Cyclin-Dependent Kinase 8/*antagonists & inhibitors/genetics/*metabolism MH - Cyclin-Dependent Kinases/*antagonists & inhibitors/genetics/*metabolism MH - Humans MH - Models, Molecular MH - Molecular Probes/chemistry/*pharmacology MH - Molecular Structure MH - Protein Kinase Inhibitors/chemistry/*pharmacology MH - Pyridines/chemistry/*pharmacology MH - Spiro Compounds/chemistry/*pharmacology PMC - PMC4677459 MID - EMS65392 OID - NLM: EMS65392 EDAT- 2015/10/27 06:00 MHDA- 2016/04/14 06:00 PMCR- 2016/05/18 CRDT- 2015/10/27 06:00 PHST- 2015/04/08 00:00 [received] PHST- 2015/10/01 00:00 [accepted] PHST- 2015/10/27 06:00 [entrez] PHST- 2015/10/27 06:00 [pubmed] PHST- 2016/04/14 06:00 [medline] PHST- 2016/05/18 00:00 [pmc-release] AID - 10.1038/nchembio.1952 [doi] PST - ppublish SO - Nat Chem Biol. 2015 Dec;11(12):973-980. doi: 10.1038/nchembio.1952. Epub 2015 Oct 26. PMID- 20683637 OWN - NLM STAT- MEDLINE DCOM- 20120508 LR - 20250529 IS - 1573-0646 (Electronic) IS - 0167-6997 (Linking) VI - 30 IP - 1 DP - 2012 Feb TI - A Phase II trial of 17-allylamino, 17-demethoxygeldanamycin (17-AAG, tanespimycin) in patients with metastatic melanoma. PG - 341-9 LID - 10.1007/s10637-010-9493-4 [doi] AB - PURPOSE: A Phase II study to screen for anti-melanoma activity of the heat shock protein 90 (HSP90) inhibitor, 17-AAG (17-allylamino-17-demethoxygeldanamycin) was performed. The primary endpoint was the rate of disease stabilisation in patients with progressive, metastatic melanoma treated with 17-AAG. Secondary endpoints were to determine: the toxicity of 17-AAG, the duration of response(s), median survival and further study the pharmacokinetics and pharmacodynamics of 17-AAG. PATIENTS AND METHODS: Patients with metastatic melanoma (progressive disease documented ≤6 months of entering study) were treated with weekly, intravenous 17-AAG. A Simon one sample two stage minimax design was used. A stable disease rate of ≥25% at 6 months was considered compatible with 17-AAG having activity. RESULTS: Fourteen patients (8 male: 6 female) were entered, eleven received 17-AAG (performance status 0 or 1). Median age was 60 (range 29-81) years. The majority (93%) received prior chemotherapy and had stage M1c disease (71%). Toxicity was rarely ≥ Grade 2 in severity and commonly included fatigue, headache and gastrointestinal disturbances. One of eleven patients treated with 17-AAG had stable disease for 6 months and median survival for all patients was 173 days. The study was closed prematurely prior to completion of the first stage of recruitment and limited planned pharmacokinetic and pharmacodynamic analyses. CONCLUSION: Some evidence of 17-AAG activity was observed although early study termination meant study endpoints were not reached. Stable disease rates can be incorporated into trials screening for anti-melanoma activity and further study of HSP90 inhibitors in melanoma should be considered. FAU - Pacey, Simon AU - Pacey S AD - Cancer Research UK Centre for Cancer Therapeutics, The Institute of Cancer Research, Sutton, Surrey SM2 5NG, UK. FAU - Gore, Martin AU - Gore M FAU - Chao, David AU - Chao D FAU - Banerji, Udai AU - Banerji U FAU - Larkin, James AU - Larkin J FAU - Sarker, Sarah AU - Sarker S FAU - Owen, Karen AU - Owen K FAU - Asad, Yasmin AU - Asad Y FAU - Raynaud, Florence AU - Raynaud F FAU - Walton, Mike AU - Walton M FAU - Judson, Ian AU - Judson I FAU - Workman, Paul AU - Workman P FAU - Eisen, Tim AU - Eisen T LA - eng SI - ClinicalTrials.gov/NCT00104897 GR - C309/A8274/CRUK_/Cancer Research UK/United Kingdom GR - C212/A11342/CRUK_/Cancer Research UK/United Kingdom GR - C212/A7324/CRUK_/Cancer Research UK/United Kingdom GR - C212/A5720/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial, Phase II PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20100805 PL - United States TA - Invest New Drugs JT - Investigational new drugs JID - 8309330 RN - 0 (Antineoplastic Agents) RN - 0 (Benzoquinones) RN - 0 (HSP90 Heat-Shock Proteins) RN - 0 (Lactams, Macrocyclic) RN - 4GY0AVT3L4 (tanespimycin) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antineoplastic Agents/administration & dosage/adverse effects/pharmacokinetics/*therapeutic use MH - Benzoquinones/administration & dosage/adverse effects/pharmacokinetics/*therapeutic use MH - Drug Administration Schedule MH - Early Termination of Clinical Trials MH - England MH - Female MH - HSP90 Heat-Shock Proteins/antagonists & inhibitors/metabolism MH - Humans MH - Kaplan-Meier Estimate MH - Lactams, Macrocyclic/administration & dosage/adverse effects/pharmacokinetics/*therapeutic use MH - Male MH - Melanoma/*drug therapy/metabolism/mortality/secondary MH - Middle Aged MH - Skin Neoplasms/*drug therapy/metabolism/mortality/pathology MH - Treatment Outcome EDAT- 2010/08/05 06:00 MHDA- 2012/05/09 06:00 CRDT- 2010/08/05 06:00 PHST- 2010/06/11 00:00 [received] PHST- 2010/07/07 00:00 [accepted] PHST- 2010/08/05 06:00 [entrez] PHST- 2010/08/05 06:00 [pubmed] PHST- 2012/05/09 06:00 [medline] AID - 10.1007/s10637-010-9493-4 [doi] PST - ppublish SO - Invest New Drugs. 2012 Feb;30(1):341-9. doi: 10.1007/s10637-010-9493-4. Epub 2010 Aug 5. PMID- 31848526 OWN - NLM STAT- MEDLINE DCOM- 20191226 LR - 20200917 IS - 1671-167X (Print) IS - 1671-167X (Linking) VI - 51 IP - 6 DP - 2019 Dec 18 TI - [Adrenal hemorrhage in a patient with systemic lupus erythematosus]. PG - 1178-1181 AB - A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1:10 000, anti-dsDNA antibody 1:3 200; anti-Smith antibody, anti-U1-snRNP/Sm antibody were positive, blood complement 3(C3) 0.43 g/L, complement 4(C4) 0.07 g /L. Anticardiolipin antibody (ACL), anti-β2-GP1, lupus anticoagulant (LA) were negative; HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 μg/L, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodinecontaining contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. CONCLUSION of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned. FAU - Wang, Y H AU - Wang YH AD - Department of Rheumatology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China. FAU - Zhang, G H AU - Zhang GH AD - Department of Rheumatology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China. FAU - Zhang, L L AU - Zhang LL AD - Department of Rheumatology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China. FAU - Luo, J L AU - Luo JL AD - Department of Rheumatology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China. FAU - Gao, L AU - Gao L AD - Department of Rheumatology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China. LA - chi PT - Case Reports PT - Journal Article PL - China TA - Beijing Da Xue Xue Bao Yi Xue Ban JT - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences JID - 101125284 RN - 0 (Antibodies, Anticardiolipin) SB - IM MH - Antibodies, Anticardiolipin MH - *Antiphospholipid Syndrome MH - Aorta, Abdominal MH - Female MH - Hemorrhage MH - Humans MH - *Lupus Erythematosus, Systemic MH - Middle Aged PMC - PMC7433578 EDAT- 2019/12/19 06:00 MHDA- 2019/12/27 06:00 PMCR- 2019/12/18 CRDT- 2019/12/19 06:00 PHST- 2019/12/19 06:00 [entrez] PHST- 2019/12/19 06:00 [pubmed] PHST- 2019/12/27 06:00 [medline] PHST- 2019/12/18 00:00 [pmc-release] AID - bjdxxbyxb-51-6-1178 [pii] AID - 10.19723/j.issn.1671-167X.2019.06.036 [doi] PST - ppublish SO - Beijing Da Xue Xue Bao Yi Xue Ban. 2019 Dec 18;51(6):1178-1181. doi: 10.19723/j.issn.1671-167X.2019.06.036. PMID- 25533335 OWN - NLM STAT- MEDLINE DCOM- 20150313 LR - 20250529 IS - 1878-3686 (Electronic) IS - 1535-6108 (Print) IS - 1535-6108 (Linking) VI - 27 IP - 1 DP - 2015 Jan 12 TI - Combined MYC and P53 defects emerge at medulloblastoma relapse and define rapidly progressive, therapeutically targetable disease. PG - 72-84 LID - S1535-6108(14)00453-X [pii] LID - 10.1016/j.ccell.2014.11.002 [doi] AB - We undertook a comprehensive clinical and biological investigation of serial medulloblastoma biopsies obtained at diagnosis and relapse. Combined MYC family amplifications and P53 pathway defects commonly emerged at relapse, and all patients in this group died of rapidly progressive disease postrelapse. To study this interaction, we investigated a transgenic model of MYCN-driven medulloblastoma and found spontaneous development of Trp53 inactivating mutations. Abrogation of p53 function in this model produced aggressive tumors that mimicked characteristics of relapsed human tumors with combined P53-MYC dysfunction. Restoration of p53 activity and genetic and therapeutic suppression of MYCN all reduced tumor growth and prolonged survival. Our findings identify P53-MYC interactions at medulloblastoma relapse as biomarkers of clinically aggressive disease that may be targeted therapeutically. CI - Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Hill, Rebecca M AU - Hill RM AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Kuijper, Sanne AU - Kuijper S AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Lindsey, Janet C AU - Lindsey JC AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Petrie, Kevin AU - Petrie K AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Schwalbe, Ed C AU - Schwalbe EC AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Barker, Karen AU - Barker K AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Boult, Jessica K R AU - Boult JK AD - Division of Radiotherapy and Imaging, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Williamson, Daniel AU - Williamson D AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Ahmad, Zai AU - Ahmad Z AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Hallsworth, Albert AU - Hallsworth A AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Ryan, Sarra L AU - Ryan SL AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Poon, Evon AU - Poon E AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Robinson, Simon P AU - Robinson SP AD - Division of Radiotherapy and Imaging, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Ruddle, Ruth AU - Ruddle R AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Raynaud, Florence I AU - Raynaud FI AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Howell, Louise AU - Howell L AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Kwok, Colin AU - Kwok C AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. FAU - Joshi, Abhijit AU - Joshi A AD - Department of Cellular Pathology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK. FAU - Nicholson, Sarah Leigh AU - Nicholson SL AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Crosier, Stephen AU - Crosier S AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Ellison, David W AU - Ellison DW AD - St. Jude Children's Research Hospital, Memphis, TN 38105, USA. FAU - Wharton, Stephen B AU - Wharton SB AD - Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield S10 2HQ, UK. FAU - Robson, Keith AU - Robson K AD - Children's Brain Tumour Research Centre, Queen's Medical Centre, University of Nottingham, Nottingham NG7 2RD, UK. FAU - Michalski, Antony AU - Michalski A AD - Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK. FAU - Hargrave, Darren AU - Hargrave D AD - Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK. FAU - Jacques, Thomas S AU - Jacques TS AD - Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK; Neural Development Unit, UCL Institute of Child Health, London WC1N 1EH, UK. FAU - Pizer, Barry AU - Pizer B AD - Oncology Unit, Alder Hey Children's Hospital, Liverpool L12 2AP, UK. FAU - Bailey, Simon AU - Bailey S AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. FAU - Swartling, Fredrik J AU - Swartling FJ AD - Department of Immunology, Genetics and Pathology, Science for Life Laboratory, Rudbeck Laboratory, Uppsala University, Uppsala 751 85, Sweden. FAU - Weiss, William A AU - Weiss WA AD - Department of Pediatrics, UCSF Benioff Children's Hospital, University of California, San Francisco, San Francisco, CA 94158, USA; Departments of Neurology and Neurological Surgery, University of California, San Francisco, San Francisco, CA 94158, USA; Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, San Francisco, CA 94158, USA. FAU - Chesler, Louis AU - Chesler L AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton SM2 5NG, UK. Electronic address: louis.chesler@icr.ac.uk. FAU - Clifford, Steven C AU - Clifford SC AD - Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne NE1 4LP, UK. Electronic address: steve.clifford@ncl.ac.uk. LA - eng SI - GEO/GSE62618 SI - GEO/GSE62625 GR - 18779/CRUK_/Cancer Research UK/United Kingdom GR - C1060/A10334/CRUK_/Cancer Research UK/United Kingdom GR - 18339/CRUK_/Cancer Research UK/United Kingdom GR - 13457/CRUK_/Cancer Research UK/United Kingdom GR - C34648/A12054/CRUK_/Cancer Research UK/United Kingdom GR - 18777/CRUK_/Cancer Research UK/United Kingdom GR - C8464/A13457/CRUK_/Cancer Research UK/United Kingdom GR - 16464/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20141218 PL - United States TA - Cancer Cell JT - Cancer cell JID - 101130617 RN - 0 (Antineoplastic Agents) RN - 0 (MYC protein, human) RN - 0 (MYCN protein, human) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (Nuclear Proteins) RN - 0 (Oncogene Proteins) RN - 0 (Proto-Oncogene Proteins c-myc) RN - 0 (TP53 protein, human) RN - 0 (Tumor Suppressor Protein p53) SB - IM MH - Adolescent MH - Adult MH - Animals MH - Antineoplastic Agents/therapeutic use MH - Cerebellar Neoplasms/drug therapy/*genetics/pathology MH - Child MH - Child, Preschool MH - Female MH - Gene Amplification MH - Humans MH - Infant MH - Male MH - Medulloblastoma/drug therapy/*genetics/pathology MH - Mice MH - Molecular Sequence Data MH - Mutation MH - N-Myc Proto-Oncogene Protein MH - Neoplasm Recurrence, Local/drug therapy/*genetics MH - Neoplasms, Experimental MH - Nuclear Proteins/genetics/metabolism MH - Oncogene Proteins/genetics/metabolism MH - Proto-Oncogene Proteins c-myc/*genetics/metabolism MH - Signal Transduction MH - Tumor Suppressor Protein p53/*genetics/metabolism MH - Young Adult PMC - PMC4297293 EDAT- 2014/12/24 06:00 MHDA- 2015/03/17 06:00 PMCR- 2015/01/12 CRDT- 2014/12/24 06:00 PHST- 2014/04/25 00:00 [received] PHST- 2014/09/02 00:00 [revised] PHST- 2014/11/05 00:00 [accepted] PHST- 2014/12/24 06:00 [entrez] PHST- 2014/12/24 06:00 [pubmed] PHST- 2015/03/17 06:00 [medline] PHST- 2015/01/12 00:00 [pmc-release] AID - S1535-6108(14)00453-X [pii] AID - 10.1016/j.ccell.2014.11.002 [doi] PST - ppublish SO - Cancer Cell. 2015 Jan 12;27(1):72-84. doi: 10.1016/j.ccell.2014.11.002. Epub 2014 Dec 18. PMID- 34238985 OWN - NLM STAT- MEDLINE DCOM- 20210802 LR - 20210802 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 11 IP - 1 DP - 2021 Jul 8 TI - In vitro Interleukin-7 treatment partially rescues MAIT cell dysfunction caused by SARS-CoV-2 infection. PG - 14090 LID - 10.1038/s41598-021-93536-7 [doi] LID - 14090 AB - MAIT cells have been shown to be activated upon several viral infections in a TCR-independent manner by responding to inflammatory cytokines secreted by antigen-presenting cells. Recently, a few studies have shown a similar activation of MAIT cells in response to severe acute respiratory coronavirus 2 (SARS-CoV-2) infection. In this study, we investigate the effect of SARS-CoV-2 infection on the frequency and phenotype of MAIT cells by flow cytometry, and we test in vitro stimulation conditions on the capacity to enhance or rescue the antiviral function of MAIT cells from patients with coronavirus disease 2019 (COVID-19). Our study, in agreement with recently published studies, confirmed the decline in MAIT cell frequency of hospitalized donors in comparison to healthy donors. MAIT cells of COVID-19 patients also had lower expression levels of TNF-alpha, perforin and granzyme B upon stimulation with IL-12 + IL-18. 24 h' incubation with IL-7 successfully restored perforin expression levels in COVID-19 patients. Combined, our findings support the growing evidence that SARS-CoV-2 is dysregulating MAIT cells and that IL-7 treatment might improve their function, rendering them more effective in protecting the body against the virus. FAU - Hubrack, Satanay AU - Hubrack S AUID- ORCID: 0000-0003-1095-7386 AD - Department of Human Genetics, Research Branch, Sidra Medicine, 26999, Doha, Qatar. Shubrack@sidra.org. FAU - Al-Nesf, Maryam Ali AU - Al-Nesf MA AD - Allergy and Clinical Immunology Section, Hamad Medical Corporation, 3050, Doha, Qatar. AD - Center of Metabolism and Inflammation, Division of Medicine, Royal Free Campus, University College London, Rowland Hill Road, London, NW3 2PF, UK. FAU - Agrebi, Nourhen AU - Agrebi N AD - Department of Human Genetics, Research Branch, Sidra Medicine, 26999, Doha, Qatar. FAU - Raynaud, Christophe AU - Raynaud C AD - Department of Human Genetics, Research Branch, Sidra Medicine, 26999, Doha, Qatar. FAU - Khattab, Mohammed Abu AU - Khattab MA AD - Division of Infectious Disease, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Thomas, Merlin AU - Thomas M AD - Pulmonary Division, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Ibrahim, Tayseer AU - Ibrahim T AD - Allergy and Clinical Immunology Section, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Taha, Salma AU - Taha S AD - Allergy and Clinical Immunology Section, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Dermime, Said AU - Dermime S AUID- ORCID: 0000-0002-5526-7496 AD - Translational Cancer Research Facility, National Center for Cancer Care and Research, Translational Research Institute, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Merhi, Maysaloun AU - Merhi M AD - Translational Cancer Research Facility, National Center for Cancer Care and Research, Translational Research Institute, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Kulinski, Michal AU - Kulinski M AD - Translational Research Institute, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Steinhoff, Martin AU - Steinhoff M AD - Translational Research Institute, Hamad Medical Corporation, 3050, Doha, Qatar. AD - Weill Cornell Medicine, 24144, Doha, Qatar. AD - Weill Cornell Medicine, New York, NY, 10021, USA. AD - Department of Dermatology, Hamad Medical Corporation, 3050, Doha, Qatar. FAU - Tang, Patrick AU - Tang P AUID- ORCID: 0000-0003-1583-5484 AD - Department of Pathology, Sidra Medicine, 26999, Doha, Qatar. FAU - Lo, Bernice AU - Lo B AD - Department of Human Genetics, Research Branch, Sidra Medicine, 26999, Doha, Qatar. blo@sidra.org. AD - College of Health and Life Sciences, Hamad Bin Khalifa University, 34110, Doha, Qatar. blo@sidra.org. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210708 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Interleukin-7) RN - 0 (Tumor Necrosis Factor-alpha) RN - 126465-35-8 (Perforin) RN - EC 3.4.21.- (Granzymes) SB - IM MH - COVID-19/*prevention & control/*virology MH - Cells, Cultured MH - Female MH - Granzymes/metabolism MH - Humans MH - Interleukin-7/*pharmacology MH - Male MH - Mucosal-Associated Invariant T Cells/metabolism/*physiology/*virology MH - Perforin/metabolism MH - SARS-CoV-2/*pathogenicity MH - Tumor Necrosis Factor-alpha/metabolism PMC - PMC8266862 COIS- The authors declare no competing interests. EDAT- 2021/07/10 06:00 MHDA- 2021/08/03 06:00 PMCR- 2021/07/08 CRDT- 2021/07/09 06:34 PHST- 2021/04/04 00:00 [received] PHST- 2021/06/24 00:00 [accepted] PHST- 2021/07/09 06:34 [entrez] PHST- 2021/07/10 06:00 [pubmed] PHST- 2021/08/03 06:00 [medline] PHST- 2021/07/08 00:00 [pmc-release] AID - 10.1038/s41598-021-93536-7 [pii] AID - 93536 [pii] AID - 10.1038/s41598-021-93536-7 [doi] PST - epublish SO - Sci Rep. 2021 Jul 8;11(1):14090. doi: 10.1038/s41598-021-93536-7. PMID- 37026591 OWN - NLM STAT- MEDLINE DCOM- 20230822 LR - 20260127 IS - 1520-4804 (Electronic) IS - 0022-2623 (Print) IS - 0022-2623 (Linking) VI - 66 IP - 8 DP - 2023 Apr 27 TI - Discovery of an In Vivo Chemical Probe for BCL6 Inhibition by Optimization of Tricyclic Quinolinones. PG - 5892-5906 LID - 10.1021/acs.jmedchem.3c00155 [doi] AB - B-cell lymphoma 6 (BCL6) is a transcriptional repressor and oncogenic driver of diffuse large B-cell lymphoma (DLBCL). Here, we report the optimization of our previously reported tricyclic quinolinone series for the inhibition of BCL6. We sought to improve the cellular potency and in vivo exposure of the non-degrading isomer, CCT373567, of our recently published degrader, CCT373566. The major limitation of our inhibitors was their high topological polar surface areas (TPSA), leading to increased efflux ratios. Reducing the molecular weight allowed us to remove polarity and decrease TPSA without considerably reducing solubility. Careful optimization of these properties, as guided by pharmacokinetic studies, led to the discovery of CCT374705, a potent inhibitor of BCL6 with a good in vivo profile. Modest in vivo efficacy was achieved in a lymphoma xenograft mouse model after oral dosing. FAU - Harnden, Alice C AU - Harnden AC AUID- ORCID: 0000-0002-4092-705X FAU - Davis, Owen A AU - Davis OA FAU - Box, Gary M AU - Box GM FAU - Hayes, Angela AU - Hayes A FAU - Johnson, Louise D AU - Johnson LD FAU - Henley, Alan T AU - Henley AT FAU - de Haven Brandon, Alexis K AU - de Haven Brandon AK FAU - Valenti, Melanie AU - Valenti M FAU - Cheung, Kwai-Ming J AU - Cheung KJ FAU - Brennan, Alfie AU - Brennan A FAU - Huckvale, Rosemary AU - Huckvale R FAU - Pierrat, Olivier A AU - Pierrat OA FAU - Talbot, Rachel AU - Talbot R FAU - Bright, Michael D AU - Bright MD FAU - Akpinar, Hafize Aysin AU - Akpinar HA FAU - Miller, Daniel S J AU - Miller DSJ FAU - Tarantino, Dalia AU - Tarantino D FAU - Gowan, Sharon AU - Gowan S FAU - de Klerk, Selby AU - de Klerk S FAU - McAndrew, Peter Craig AU - McAndrew PC AUID- ORCID: 0000-0002-1366-088X FAU - Le Bihan, Yann-Vaï AU - Le Bihan YV AUID- ORCID: 0000-0002-6850-9706 FAU - Meniconi, Mirco AU - Meniconi M FAU - Burke, Rosemary AU - Burke R FAU - Kirkin, Vladimir AU - Kirkin V FAU - van Montfort, Rob L M AU - van Montfort RLM FAU - Raynaud, Florence I AU - Raynaud FI AUID- ORCID: 0000-0003-0957-6279 FAU - Rossanese, Olivia W AU - Rossanese OW FAU - Bellenie, Benjamin R AU - Bellenie BR AUID- ORCID: 0000-0001-9987-3079 FAU - Hoelder, Swen AU - Hoelder S AUID- ORCID: 0000-0001-8636-1488 LA - eng GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20230407 PL - United States TA - J Med Chem JT - Journal of medicinal chemistry JID - 9716531 RN - 0 (Proto-Oncogene Proteins c-bcl-6) RN - 0 (Quinolones) RN - 0 (Transcription Factors) RN - 0 (BCL6 protein, human) RN - 0 (Bcl6 protein, mouse) RN - 0 (CCT373566) RN - 0 (Fluorine Compounds) SB - IM MH - Animals MH - Humans MH - Mice MH - Cell Line, Tumor MH - Disease Models, Animal MH - *Lymphoma, Large B-Cell, Diffuse/pathology MH - Proto-Oncogene Proteins c-bcl-6/chemistry MH - *Quinolones MH - Transcription Factors MH - Fluorine Compounds PMC - PMC10150366 COIS- The authors declare the following competing financial interest(s): All authors are current or previous employees of The Institute of Cancer Research (ICR), which has a commercial interest in a range of drug targets and operates a Rewards to Discoverers scheme, through which employees may receive financial bene-fits following the commercial licensing of a project. EDAT- 2023/04/08 06:00 MHDA- 2023/05/03 06:42 PMCR- 2023/05/01 CRDT- 2023/04/07 07:32 PHST- 2023/05/03 06:42 [medline] PHST- 2023/04/08 06:00 [pubmed] PHST- 2023/04/07 07:32 [entrez] PHST- 2023/05/01 00:00 [pmc-release] AID - 10.1021/acs.jmedchem.3c00155 [doi] PST - ppublish SO - J Med Chem. 2023 Apr 27;66(8):5892-5906. doi: 10.1021/acs.jmedchem.3c00155. Epub 2023 Apr 7. PMID- 35788243 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220716 IS - 2050-2974 (Print) IS - 2050-2974 (Electronic) IS - 2050-2974 (Linking) VI - 10 IP - 1 DP - 2022 Jul 4 TI - Adult and child and adolescent psychiatrists' experiences of transition in anorexia nervosa: a qualitative study. PG - 92 LID - 10.1186/s40337-022-00610-0 [doi] LID - 92 AB - BACKGROUND: Young patients suffering from anorexia nervosa (AN) frequently need further treatment in Adult Mental Health Services (AMHS). The transition period from Child and Adolescent Mental Health Services (CAMHS) to AMHS is a critical time, with a high risk of disengagement from healthcare. We explored physicians' perspectives of the transition to triangulate the multiple perspectives of physicians, parents and those with a lived AN experience to more comprehensively characterize the challenges in this process of treatment transition. METHODS: Using purposive sampling, we recruited 16 physicians confronted with transition in AN (adult psychiatrists, child and adolescent psychiatrists and pediatrician) and conducted semi-structured interviews, which were anonymized, transcribed, and analyzed following the reflexive thematic analysis framework. RESULTS: Our analysis produced three main themes. First, a shared agreement on the transition's malfunction, where participants depicted transition as a dissatisfying, violent event. Second, the conception of AN as a disorder with specific needs, challenging the transition process especially regarding physicians' engagement. Finally, the ideal transition conceived as a serene experience of separation, with unanimous agreement on the necessity to start the transition depending on patients' needs rather than their age, in order to turn transitions into moments of care. CONCLUSION: Our results are in line with other qualitative research studying transition in AN and in other chronic diseases, either focusing on the experience of healthcare workers, families, or patients. Our research shows transition in AN as an anxiety-inducing experience for physicians, patients and families alike. Moreover, we highlight a gap in the way physicians perceive and assist the patient's greater autonomy, depending on their specialty. Helping physicians to manage their patient's autonomy, which is a cornerstone of the transition readiness concept, could be a very efficient way to improve transitions in AN. Anorexia Nervosa (AN) is a severe disease, which most of the time starts during adolescence. Transition from Child and Adolescent Mental Health Services to Adult Mental Health Services is at risk of disengagement from healthcare. In order to better understand this process, we interviewed expert physicians about their experiences of transition in AN using a qualitative thematic analysis which highlighted three main themes. First, a shared agreement on the transition's malfunction. Second, the conception of AN as a disorder with specific needs challenging the transition process. Finally, the ideal transition conceived as a serene experience of separation, which needs to be started depending on patients' needs rather than their age. We also show differences in the way physicians perceive and assist the patient's greater autonomy acquired during the transition. Helping physicians to support their patients in acquiring autonomy, which is a cornerstone of the transition readiness concept, could be a very efficient way to improve transitions in AN. CI - © 2022. The Author(s). FAU - Stocker, Antoine AU - Stocker A AUID- ORCID: 0000-0002-7573-3853 AD - Service Universitaire de Psychiatrie de l'Enfant et de l'Adolescent, CHU de Toulouse, Hôpital Purpan, Place du Dr Baylac, TSA 40031, 31059, Toulouse cedex 9, France. stocker.a@chu-toulouse.fr. AD - Fédération Régionale de Recherche en Psychiatrie Et Santé Mentale Occitanie, FERREPSY Occitanie, 31000, Toulouse, France. stocker.a@chu-toulouse.fr. FAU - Rosenthal, Lucie AU - Rosenthal L AD - Service Universitaire de Psychiatrie de l'Enfant et de l'Adolescent, CHU de Toulouse, Hôpital Purpan, Place du Dr Baylac, TSA 40031, 31059, Toulouse cedex 9, France. FAU - Mesquida, Laure AU - Mesquida L AD - Service Universitaire de Psychiatrie de l'Enfant et de l'Adolescent, CHU de Toulouse, Hôpital Purpan, Place du Dr Baylac, TSA 40031, 31059, Toulouse cedex 9, France. FAU - Raynaud, Jean-Philippe AU - Raynaud JP AD - Service Universitaire de Psychiatrie de l'Enfant et de l'Adolescent, CHU de Toulouse, Hôpital Purpan, Place du Dr Baylac, TSA 40031, 31059, Toulouse cedex 9, France. AD - Fédération Régionale de Recherche en Psychiatrie Et Santé Mentale Occitanie, FERREPSY Occitanie, 31000, Toulouse, France. AD - CERPOP, Université de Toulouse, Inserm, UPS, Toulouse, France. FAU - Revet, Alexis AU - Revet A AD - Service Universitaire de Psychiatrie de l'Enfant et de l'Adolescent, CHU de Toulouse, Hôpital Purpan, Place du Dr Baylac, TSA 40031, 31059, Toulouse cedex 9, France. AD - Fédération Régionale de Recherche en Psychiatrie Et Santé Mentale Occitanie, FERREPSY Occitanie, 31000, Toulouse, France. AD - CERPOP, Université de Toulouse, Inserm, UPS, Toulouse, France. LA - eng PT - Journal Article DEP - 20220704 PL - England TA - J Eat Disord JT - Journal of eating disorders JID - 101610672 PMC - PMC9252565 OTO - NOTNLM OT - Adolescent OT - Anorexia nervosa OT - Mental health services OT - Psychiatrists OT - Qualitative research OT - Transition to adult care OT - Young adult COIS- The authors declare they have no competing interests. EDAT- 2022/07/06 06:00 MHDA- 2022/07/06 06:01 PMCR- 2022/07/04 CRDT- 2022/07/05 18:20 PHST- 2021/12/12 00:00 [received] PHST- 2022/06/10 00:00 [accepted] PHST- 2022/07/05 18:20 [entrez] PHST- 2022/07/06 06:00 [pubmed] PHST- 2022/07/06 06:01 [medline] PHST- 2022/07/04 00:00 [pmc-release] AID - 10.1186/s40337-022-00610-0 [pii] AID - 610 [pii] AID - 10.1186/s40337-022-00610-0 [doi] PST - epublish SO - J Eat Disord. 2022 Jul 4;10(1):92. doi: 10.1186/s40337-022-00610-0. PMID- 34331007 OWN - NLM STAT- MEDLINE DCOM- 20220314 LR - 20221027 IS - 1476-5578 (Electronic) IS - 1359-4184 (Linking) VI - 26 IP - 12 DP - 2021 Dec TI - Restoring glutamate receptosome dynamics at synapses rescues autism-like deficits in Shank3-deficient mice. PG - 7596-7609 LID - 10.1038/s41380-021-01230-x [doi] AB - Shank3 monogenic mutations lead to autism spectrum disorders (ASD). Shank3 is part of the glutamate receptosome that physically links ionotropic NMDA receptors to metabotropic mGlu5 receptors through interactions with scaffolding proteins PSD95-GKAP-Shank3-Homer. A main physiological function of the glutamate receptosome is to control NMDA synaptic function that is required for plasticity induction. Intact glutamate receptosome supports glutamate receptors activation and plasticity induction, while glutamate receptosome disruption blocks receptors activity, preventing the induction of subsequent plasticity. Despite possible impact on metaplasticity and cognitive behaviors, scaffold interaction dynamics and their consequences are poorly defined. Here, we used mGlu5-Homer interaction as a biosensor of glutamate receptosome integrity to report changes in synapse availability for plasticity induction. Combining BRET imaging and electrophysiology, we show that a transient neuronal depolarization inducing NMDA-dependent plasticity disrupts glutamate receptosome in a long-lasting manner at synapses and activates signaling pathways required for the expression of the initiated neuronal plasticity, such as ERK and mTOR pathways. Glutamate receptosome disruption also decreases the NMDA/AMPA ratio, freezing the sensitivity of the synapse to subsequent changes of neuronal activity. These data show the importance of a fine-tuning of protein-protein interactions within glutamate receptosome, driven by changes of neuronal activity, to control plasticity. In a mouse model of ASD, a truncated mutant form of Shank3 prevents the integrity of the glutamate receptosome. These mice display altered plasticity, anxiety-like, and stereotyped behaviors. Interestingly, repairing the integrity of glutamate receptosome and its sensitivity to the neuronal activity rescued synaptic transmission, plasticity, and some behavioral traits of Shank3∆C mice. Altogether, our findings characterize mechanisms by which Shank3 mutations cause ASD and highlight scaffold dynamics as new therapeutic target. CI - © 2021. The Author(s), under exclusive licence to Springer Nature Limited. FAU - Moutin, Enora AU - Moutin E AUID- ORCID: 0000-0001-8250-703X AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. enora.moutin@igf.cnrs.fr. FAU - Sakkaki, Sophie AU - Sakkaki S AUID- ORCID: 0000-0003-2333-951X AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Compan, Vincent AU - Compan V AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Bouquier, Nathalie AU - Bouquier N AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Giona, Federica AU - Giona F AD - Cnr Neuroscience Institute, 3220129, Milan, Italy. FAU - Areias, Julie AU - Areias J AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Goyet, Elise AU - Goyet E AUID- ORCID: 0000-0002-0162-8663 AD - Interdisciplinary Institute for NeuroScience, CNRS, UMR 5297, Centre Broca Nouvelle-Aquitaine, 33076, Bordeaux, France. FAU - Hemonnot-Girard, Anne-Laure AU - Hemonnot-Girard AL AUID- ORCID: 0000-0003-0784-3927 AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Seube, Vincent AU - Seube V AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Glasson, Bastien AU - Glasson B AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Benac, Nathan AU - Benac N AD - Interdisciplinary Institute for NeuroScience, CNRS, UMR 5297, Centre Broca Nouvelle-Aquitaine, 33076, Bordeaux, France. FAU - Chastagnier, Yan AU - Chastagnier Y AUID- ORCID: 0000-0002-1239-0048 AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. AD - PhyMedExp, Univ Montpellier, INSERM, CNRS, CHU de Montpellier, France. FAU - Audinat, Etienne AU - Audinat E AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. FAU - Groc, Laurent AU - Groc L AD - Interdisciplinary Institute for NeuroScience, CNRS, UMR 5297, Centre Broca Nouvelle-Aquitaine, 33076, Bordeaux, France. FAU - Maurice, Tangui AU - Maurice T AD - MMDN, Univ Montpellier, EPHE, INSERM, Montpellier, France. FAU - Sala, Carlo AU - Sala C AUID- ORCID: 0000-0003-0662-9523 AD - Cnr Neuroscience Institute, 3220129, Milan, Italy. FAU - Verpelli, Chiara AU - Verpelli C AUID- ORCID: 0000-0003-2949-9725 AD - Cnr Neuroscience Institute, 3220129, Milan, Italy. FAU - Perroy, Julie AU - Perroy J AUID- ORCID: 0000-0001-6199-0448 AD - IGF, University of Montpellier, CNRS, INSERM, Montpellier, France. julie.perroy@igf.cnrs.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210730 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (Microfilament Proteins) RN - 0 (Nerve Tissue Proteins) RN - 0 (Shank3 protein, mouse) RN - 3KX376GY7L (Glutamic Acid) SB - IM MH - Animals MH - *Autistic Disorder/genetics/metabolism MH - Disease Models, Animal MH - Endosomes/metabolism MH - Glutamic Acid/metabolism MH - Mice MH - *Microfilament Proteins/metabolism MH - *Nerve Tissue Proteins/metabolism MH - Synapses/metabolism EDAT- 2021/08/01 06:00 MHDA- 2022/03/15 06:00 CRDT- 2021/07/31 07:23 PHST- 2020/09/11 00:00 [received] PHST- 2021/07/06 00:00 [accepted] PHST- 2021/07/02 00:00 [revised] PHST- 2021/08/01 06:00 [pubmed] PHST- 2022/03/15 06:00 [medline] PHST- 2021/07/31 07:23 [entrez] AID - 10.1038/s41380-021-01230-x [pii] AID - 10.1038/s41380-021-01230-x [doi] PST - ppublish SO - Mol Psychiatry. 2021 Dec;26(12):7596-7609. doi: 10.1038/s41380-021-01230-x. Epub 2021 Jul 30. PMID- 33212917 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201201 IS - 2072-6694 (Print) IS - 2072-6694 (Electronic) IS - 2072-6694 (Linking) VI - 12 IP - 11 DP - 2020 Nov 17 TI - Real Life Prospective Evaluation of New Drug-Eluting Platform for Chemoembolization of Patients with Hepatocellular Carcinoma: PARIS Registry. LID - 10.3390/cancers12113405 [doi] LID - 3405 AB - BACKGROUND AND AIM: Transarterial chemoembolization with drug-eluting microspheres (DEM-TACE) is recommended for patients with BCLC stage B hepatocellular carcinoma (HCC) and stage 0-A unsuitable for curative treatments. We assessed efficacy and safety along with hepatobiliary toxicities (HBT) of DEM-TACE using a novel microsphere, LifePearl(TM), loaded with anthracyclines. MATERIALS AND METHODS: 97 patients diagnosed with HCC were prospectively enrolled and treated using LifePearl(TM) loaded with doxorubicin (77%) or idarubicin (23%). Safety and tolerability were assessed using CTCAE, HBT by CT/MRI scans, and tumor response by applying modified Response Evaluation Criteria in Solid Tumors (mRECIST). Follow-up was after 2 years. RESULTS: Adverse events (AE) were reported in 73.2% of patients, majority being Grade 1-2. Grade ≥ 3 AE reported in 13.4% of patients were mainly related to postembolization syndrome. HBT were observed after 15.5% (29/187) of the DEM-TACEs. Objective response and disease control rates were 81% and 99%, respectively, as the best responses. Survival rates at one and two years were 81% and 66%, respectively, while the median overall survival (OS) was not reached. Median progression free survival was 13.7 months (95% CI: 11.3; 15.6) and median time to TACE untreatable progression was 16.7 months (95% CI: 12.7; not estimable (n.e.)). CONCLUSIONS: DEM-TACE using LifePearl(TM) provides a high tumor response rate in HCC patients. HBT rates within or below previously reported results for cTACE and DEM-TACE indicate a good safety profile for LifePearl(TM). The trial was registered in ClinicalTrials.gov National Library of Medicine (ID: NCT03053596). FAU - de Baere, Thierry AU - de Baere T AUID- ORCID: 0000-0002-4556-7663 AD - Department of Interventional Radiology, Gustave Roussy Cancer Center, 114 rue Edouard Vaillant, 94805 Villejuif, France. AD - UFR Médecine Le Kremlin-Bicêtre, Université Paris-Saclay, 94250 Le Kremlin-Bicêtre, France. FAU - Guiu, Boris AU - Guiu B AD - Imagerie Médicale St Eloi, 34000 Montpellier, France. FAU - Ronot, Maxime AU - Ronot M AD - Service de Radiologie, Hôpital Beaujon, APHP Nord, Clichy & Université de Paris, 92210 Clichy, France. FAU - Chevallier, Patrick AU - Chevallier P AD - Service d'Imagerie Médicale Diagnostique et Interventionnelle, Hôpital L'Archet II, 06200 Nice, France. FAU - Sergent, Géraldine AU - Sergent G AD - Radiologie & Imagerie Digestive, CHRU Lille, 59037 Lille, France. FAU - Tancredi, Illario AU - Tancredi I AD - Gastroentérologie Médicale, Hôpital Erasme, Université Libre de Bruxelles, 1070 Brussels, Belgium. FAU - Tselikas, Lambros AU - Tselikas L AD - Department of Interventional Radiology, Gustave Roussy Cancer Center, 114 rue Edouard Vaillant, 94805 Villejuif, France. FAU - Dioguardi Burgio, Marco AU - Dioguardi Burgio M AUID- ORCID: 0000-0002-3243-6699 AD - Service de Radiologie, Hôpital Beaujon, APHP Nord, Clichy & Université de Paris, 92210 Clichy, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Service de Radiologie, Hôpital Beaujon, APHP Nord, Clichy & Université de Paris, 92210 Clichy, France. FAU - Deschamps, Frederic AU - Deschamps F AD - Department of Interventional Radiology, Gustave Roussy Cancer Center, 114 rue Edouard Vaillant, 94805 Villejuif, France. FAU - Verset, Gontran AU - Verset G AD - Gastroentérologie Médicale, Hôpital Erasme, Université Libre de Bruxelles, 1070 Brussels, Belgium. LA - eng SI - ClinicalTrials.gov/NCT03053596 GR - NCT03053596/Terumo/ PT - Journal Article DEP - 20201117 PL - Switzerland TA - Cancers (Basel) JT - Cancers JID - 101526829 PMC - PMC7698357 OTO - NOTNLM OT - DEM-TACE OT - doxorubicin OT - hepatobiliary toxicities OT - hepatocellular carcinoma OT - idarubicin COIS- The authors declare following conflict of interest: Thierry de Baere is consultant for Astra-Zeneca, Boston-Scientific, Guerbet, Medtronic, Terumo; Boris Guiu is consultant for Boston-Scientific, Terumo, Guerbet, Roche, Quantum Surgical. Maxime Ronot is consultant for Quantum Surgical; Lambros Tselikas is consultant for BMS, Boston Scientific, General Electric and received grants from BMS and Terumo; Frederick Deschamps is consultant for GE Healthcare, Medtronic, Terumo, Ablatech; Gontran Verset is consultant for Bayer, BTG, Terumo, Eisai and Roche; other authors do not have any conflict of interest to declare. The study sponsor has contributed to study conception, study management and source data verification. However, the study sponsor did not influence the decision to publish data, nor the interpretation of the results. The corresponding author, the coordinating investigator of the study, had full access to data and takes final responsibility for submission of the manuscript. EDAT- 2020/11/21 06:00 MHDA- 2020/11/21 06:01 PMCR- 2020/11/17 CRDT- 2020/11/20 01:01 PHST- 2020/10/05 00:00 [received] PHST- 2020/11/07 00:00 [revised] PHST- 2020/11/10 00:00 [accepted] PHST- 2020/11/20 01:01 [entrez] PHST- 2020/11/21 06:00 [pubmed] PHST- 2020/11/21 06:01 [medline] PHST- 2020/11/17 00:00 [pmc-release] AID - cancers12113405 [pii] AID - cancers-12-03405 [pii] AID - 10.3390/cancers12113405 [doi] PST - epublish SO - Cancers (Basel). 2020 Nov 17;12(11):3405. doi: 10.3390/cancers12113405. PMID- 41952067 OWN - NLM STAT- MEDLINE DCOM- 20260409 LR - 20260409 IS - 1365-2982 (Electronic) IS - 1350-1925 (Linking) VI - 38 IP - 4 DP - 2026 Apr TI - Impact of Early-Onset IBS on Schooling, Illness in Adulthood, and Career Prospects. PG - e70308 LID - 10.1111/nmo.70308 [doi] AB - INTRODUCTION: Irritable bowel syndrome (IBS) is a disorder of gut-brain interaction that significantly affects patients' quality of life, particularly in educational and professional settings. This study aimed to assess the impact of IBS on schooling, higher education, and future professional life. METHODS: An online survey was conducted among members of a French patient association and visitors to its website. Adult participants provided demographic data, details on disease severity, and feedback on their educational and professional experiences. RESULTS: Among 365 participants with IBS, 42% reported having IBS during their schooling or higher education. The most commonly reported difficulties included restricted access to toilets during class (40%), problems with school-provided meals (39%), and lack of privacy in restrooms (50%). Additionally, 62% of the participants reported that IBS had a direct negative impact on their academic performance, leading to frequent late arrivals at class (59%), absenteeism (70%), and repeating a year (23%). Irritable bowel syndrome was rarely addressed in academic settings (27%), though 52% of adaptation requests were granted. Finally, IBS was associated with lower income levels and reduced career prospects compared with individuals without IBS during their education (p < 0.005). CONCLUSION: Irritable bowel syndrome has a significant impact on schooling and career development. Greater awareness and recognition of this condition in educational and professional environments are necessary to improve affected individuals' quality of life and professional integration. CI - © 2026 John Wiley & Sons Ltd. FAU - Wuestenberghs, Fabien AU - Wuestenberghs F AUID- ORCID: 0000-0001-6100-6017 AD - Department of Gastroenterology and Digestive Oncology, Hôpital Avicenne, AP-HP, Sorbonne Paris Nord University, Bobigny, France. FAU - Jouët, Pauline AU - Jouët P AD - Department of Gastroenterology and Digestive Oncology, Hôpital Avicenne, AP-HP, Sorbonne Paris Nord University, Bobigny, France. FAU - Piche, Thierry AU - Piche T AUID- ORCID: 0009-0003-4676-8277 AD - Department of Gastroenterology & Hepatology, Oncology and Functional Bowel Unit Explorations, CHU Nice, Archet 2 University Hospital, Nice, France. FAU - Mion, François AU - Mion F AUID- ORCID: 0000-0002-2908-1591 AD - Department of Digestive Functional Explorations, Hospices Civils de Lyon, Lyon 1 University, Lyon, France. FAU - Raynaud, Jean-Jacques AU - Raynaud JJ AUID- ORCID: 0000-0002-8215-4403 AD - Department of Gastroenterology and Digestive Oncology, Hôpital Avicenne, AP-HP, Sorbonne Paris Nord University, Bobigny, France. FAU - Melchior, Chloé AU - Melchior C AUID- ORCID: 0000-0002-8146-1540 AD - Univ Rouen Normandie, INSERM, Normandie Univ, ADEN UMR1073, Nutrition, Inflammation and Microbiota-Gut-Brain Axis, CHU Rouen, CIC-CRB 1404, Department of Gastroenterology, Rouen, France. FAU - Zerbib, Frank AU - Zerbib F AUID- ORCID: 0000-0002-6802-2121 AD - Department of Gastroenterology, CHU de Bordeaux, Centre Médico-Chirurgical Magellan, Hôpital Haut-Lévêque, Université de Bordeaux, Bordeaux, France. FAU - Macaigne, Gilles AU - Macaigne G AUID- ORCID: 0000-0003-0080-1994 AD - Department of Hepatogastroenterology, GHI Le Raincy-Montfermeil, Montfermeil, France. FAU - Iglicki, Franck AU - Iglicki F AUID- ORCID: 0009-0005-8125-5359 AD - Gastroenterologist, Paris, France. FAU - Facon, Suzelle AU - Facon S AD - APSSII Patient Association, Hôpital Avicenne, AP-HP, Bobigny, France. FAU - Renoul, Patricia AU - Renoul P AD - APSSII Patient Association, Hôpital Avicenne, AP-HP, Bobigny, France. FAU - Dapoigny, Michel AU - Dapoigny M AD - Université Clermont Auvergne, Clermont-Ferrand, France. FAU - Mosca, Alexis AU - Mosca A AUID- ORCID: 0000-0001-9504-5516 AD - Service de Gastroentérologie et Nutrition Pédiatriques, Hôpital Robert-Debré, APHP, Paris, France. FAU - Entremont, Alexandre AU - Entremont A AD - Primary Care Physician, Paris, France. FAU - Sabaté, Jean-Marc AU - Sabaté JM AUID- ORCID: 0000-0001-5591-3489 AD - Department of Gastroenterology and Digestive Oncology, Hôpital Avicenne, AP-HP, Sorbonne Paris Nord University, Bobigny, France. LA - eng PT - Journal Article PL - England TA - Neurogastroenterol Motil JT - Neurogastroenterology and motility JID - 9432572 SB - IM MH - Humans MH - *Irritable Bowel Syndrome/psychology/epidemiology MH - Male MH - Adult MH - Female MH - Middle Aged MH - Quality of Life MH - Surveys and Questionnaires MH - Young Adult MH - *Educational Status MH - Age of Onset OTO - NOTNLM OT - higher education OT - irritable bowel syndrome OT - professional impact OT - quality of life OT - schooling EDAT- 2026/04/09 00:34 MHDA- 2026/04/09 06:30 CRDT- 2026/04/09 00:01 PHST- 2026/02/26 00:00 [revised] PHST- 2025/11/28 00:00 [received] PHST- 2026/03/09 00:00 [accepted] PHST- 2026/04/09 06:30 [medline] PHST- 2026/04/09 00:34 [pubmed] PHST- 2026/04/09 00:01 [entrez] AID - 10.1111/nmo.70308 [doi] PST - ppublish SO - Neurogastroenterol Motil. 2026 Apr;38(4):e70308. doi: 10.1111/nmo.70308. PMID- 35478253 OWN - NLM STAT- MEDLINE DCOM- 20220803 LR - 20260518 IS - 1464-360X (Electronic) IS - 1101-1262 (Print) IS - 1101-1262 (Linking) VI - 32 IP - 4 DP - 2022 Aug 1 TI - Higher risk, higher protection: COVID-19 risk among immigrants in France-results from the population-based EpiCov survey. PG - 655-663 LID - 10.1093/eurpub/ckac046 [doi] AB - BACKGROUND: Immigrants and ethnic/racialized minorities have been identified as being at higher risk of coronavirus disease-19 (COVID-19) infection, but few studies report on their exposures and prevention behaviours. This study aims to examine the social distribution of COVID-19 exposure (overcrowding, working outside the home, use of public transport to go to work) and prevention behaviours (use of face masks, washing hands, respect for physical distance) in France during the first wave of the epidemic. METHODS: We used the EpiCov population-based survey from a random sample of individuals aged 15 years or more. We determined the distribution of the self-reported outcomes according to migratory status and sex, using χ2 tests. We modelled the probability of outcomes with logistic regression. Finally, we focused the analysis on the Greater Paris area and accounted for neighbourhood characteristics. RESULTS: A total of 111 824 participants were included in the study. Overall, immigrant groups from non-European countries were more exposed to COVID-19-related factors and more respectful of prevention measures. The probability of overcrowding and the use of public transport was higher for immigrants from sub-Saharan Africa [adjusted odds ratio (aOR) = 3.71 (3.19; 4.32), aOR = 6.36 (4.86; 8.32)] than for the majority population. Immigrant groups were less likely to have a non-systematic use of face masks and to breach physical distancing than the majority population [for immigrants from sub-Saharan Africa, aOR = 0.32 (0.28; 0.37) and aOR = 0.71 (0.61; 0.81), respectively]. Living in a neighbourhood with a higher share of immigrants was associated with higher exposure and better prevention behaviours. CONCLUSIONS: In France, immigrants had a higher exposure to COVID-19-related factors and more systematic prevention behaviours. CI - © The Author(s) 2022. Published by Oxford University Press on behalf of the European Public Health Association. FAU - Gosselin, Anne AU - Gosselin A AUID- ORCID: 0000-0002-6876-8249 AD - French Institute for Demographic Studies (INED), Mortality, Health, Epidemiology Unit, Aubervilliers, France. AD - French Collaborative Institute on Migrations/CNRS, Aubervilliers, France. FAU - Warszawski, Josiane AU - Warszawski J AD - INSERM CESP U1018, Université Paris-Saclay, Le Kremlin-Bicêtre, France. AD - AP-HP Epidemiology and Public Health Service, Hôpitaux Universitaires Paris-Saclay, Le Kremlin-Bicêtre, France. FAU - Bajos, Nathalie AU - Bajos N AD - Iris, Inserm, Aubervilliers, France. AD - Ecole des Hautes Etudes en Sciences Sociales, Paris, France. CN - EpiCov Study Group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Eur J Public Health JT - European journal of public health JID - 9204966 SB - IM MH - *COVID-19/prevention & control MH - *Emigrants and Immigrants MH - Humans MH - Odds Ratio MH - Physical Distancing MH - Self Report PMC - PMC9341671 FIR - Bajos, Nathalie IR - Bajos N FIR - Warszawski, Josiane IR - Warszawski J FIR - Bagein, Guillaume IR - Bagein G FIR - Beck, François IR - Beck F FIR - Counil, Emilie IR - Counil E FIR - Jusot, Florence IR - Jusot F FIR - Lydié, Nathalie IR - Lydié N FIR - Martin, Claude IR - Martin C FIR - Meyer, Laurence IR - Meyer L FIR - Raynaud, Philippe IR - Raynaud P FIR - Rouquette, Alexandra IR - Rouquette A FIR - Pailhé, Ariane IR - Pailhé A FIR - Rahib, Delphine IR - Rahib D FIR - Sillard, Patrick IR - Sillard P FIR - Slama, Rémy IR - Slama R FIR - Spire, Alexis IR - Spire A EDAT- 2022/04/29 06:00 MHDA- 2022/08/04 06:00 PMCR- 2022/04/27 CRDT- 2022/04/28 05:51 PHST- 2022/04/29 06:00 [pubmed] PHST- 2022/08/04 06:00 [medline] PHST- 2022/04/28 05:51 [entrez] PHST- 2022/04/27 00:00 [pmc-release] AID - 6574954 [pii] AID - ckac046 [pii] AID - 10.1093/eurpub/ckac046 [doi] PST - ppublish SO - Eur J Public Health. 2022 Aug 1;32(4):655-663. doi: 10.1093/eurpub/ckac046. PMID- 18645193 OWN - NLM STAT- MEDLINE DCOM- 20081021 LR - 20250529 IS - 1527-7755 (Electronic) IS - 0732-183X (Linking) VI - 26 IP - 28 DP - 2008 Oct 1 TI - Phase I clinical trial of a selective inhibitor of CYP17, abiraterone acetate, confirms that castration-resistant prostate cancer commonly remains hormone driven. PG - 4563-71 LID - 10.1200/JCO.2007.15.9749 [doi] AB - PURPOSE: Studies indicate that castration-resistant prostate cancer (CRPC) remains driven by ligand-dependent androgen receptor (AR) signaling. To evaluate this, a trial of abiraterone acetate-a potent, selective, small-molecule inhibitor of cytochrome P (CYP) 17, a key enzyme in androgen synthesis-was pursued. PATIENTS AND METHODS: Chemotherapy-naïve men (n = 21) who had prostate cancer that was resistant to multiple hormonal therapies were treated in this phase I study of once-daily, continuous abiraterone acetate, which escalated through five doses (250 to 2,000 mg) in three-patient cohorts. RESULTS: Abiraterone acetate was well tolerated. The anticipated toxicities attributable to a syndrome of secondary mineralocorticoid excess-namely hypertension, hypokalemia, and lower-limb edema-were successfully managed with a mineralocorticoid receptor antagonist. Antitumor activity was observed at all doses; however, because of a plateau in pharmacodynamic effect, 1,000 mg was selected for cohort expansion (n = 9). Abiraterone acetate administration was associated with increased levels of adrenocorticotropic hormone and steroids upstream of CYP17 and with suppression of serum testosterone, downstream androgenic steroids, and estradiol in all patients. Declines in prostate-specific antigen >or= 30%, 50%, and 90% were observed in 14 (66%), 12 (57%), and 6 (29%) patients, respectively, and lasted between 69 to >or= 578 days. Radiologic regression, normalization of lactate dehydrogenase, and improved symptoms with a reduction in analgesic use were documented. CONCLUSION: CYP17 blockade by abiraterone acetate is safe and has significant antitumor activity in CRPC. These data confirm that CRPC commonly remains dependent on ligand-activated AR signaling. FAU - Attard, Gerhardt AU - Attard G AD - Royal Marsden NHS Foundation Trust, Sutton, Surrey, United Kingdom. FAU - Reid, Alison H M AU - Reid AH FAU - Yap, Timothy A AU - Yap TA FAU - Raynaud, Florence AU - Raynaud F FAU - Dowsett, Mitch AU - Dowsett M FAU - Settatree, Sarah AU - Settatree S FAU - Barrett, Mary AU - Barrett M FAU - Parker, Christopher AU - Parker C FAU - Martins, Vanessa AU - Martins V FAU - Folkerd, Elizabeth AU - Folkerd E FAU - Clark, Jeremy AU - Clark J FAU - Cooper, Colin S AU - Cooper CS FAU - Kaye, Stan B AU - Kaye SB FAU - Dearnaley, David AU - Dearnaley D FAU - Lee, Gloria AU - Lee G FAU - de Bono, Johann S AU - de Bono JS LA - eng GR - G0501019/MRC_/Medical Research Council/United Kingdom GR - CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080721 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - 0 (Androstenes) RN - 0 (Androstenols) RN - G819A456D0 (abiraterone) SB - IM EIN - J Clin Oncol. 2012 May 20;30(15):1896 CIN - J Clin Oncol. 2008 Oct 1;26(28):4535-6. doi: 10.1200/JCO.2008.18.3145. PMID: 18626003 CIN - Nat Clin Pract Oncol. 2009 Jan;6(1):12-3. doi: 10.1038/ncponc1262. PMID: 18957947 CIN - Eur Urol. 2008 Dec;54(6):1438-9. doi: 10.1016/j.eururo.2008.08.043. PMID: 19189435 CIN - Eur Urol. 2009 Jul;56(1):220. doi: 10.1016/j.eururo.2009.04.017. PMID: 19995520 CIN - Eur Urol. 2009 Jan;55(1):248. doi: 10.1016/j.eururo.2008.09.035. PMID: 20050017 MH - Adenocarcinoma/*drug therapy MH - Administration, Oral MH - Aged MH - Aged, 80 and over MH - Androstenes MH - Androstenols/administration & dosage/pharmacokinetics/*therapeutic use MH - Castration MH - Disease Progression MH - Humans MH - Male MH - Middle Aged MH - Neoplasms, Hormone-Dependent/*drug therapy MH - Prospective Studies MH - Prostatic Neoplasms/*drug therapy MH - Treatment Outcome EDAT- 2008/07/23 09:00 MHDA- 2008/10/22 09:00 CRDT- 2008/07/23 09:00 PHST- 2008/07/23 09:00 [pubmed] PHST- 2008/10/22 09:00 [medline] PHST- 2008/07/23 09:00 [entrez] AID - JCO.2007.15.9749 [pii] AID - 10.1200/JCO.2007.15.9749 [doi] PST - ppublish SO - J Clin Oncol. 2008 Oct 1;26(28):4563-71. doi: 10.1200/JCO.2007.15.9749. Epub 2008 Jul 21. PMID- 16622046 OWN - NLM STAT- MEDLINE DCOM- 20060921 LR - 20201117 IS - 1350-0872 (Print) IS - 1350-0872 (Linking) VI - 152 IP - Pt 5 DP - 2006 May TI - Exploring the role of the CTL epitope region of listeriolysin O in the pathogenesis of Listeria monocytogenes. PG - 1287-1296 LID - 10.1099/mic.0.28754-0 [doi] AB - Listeria monocytogenes is a facultative intracellular bacterial pathogen responsible for severe opportunistic infections in humans and animals. The secreted cholesterol-dependent cytolysin, listeriolysin O (LLO), mediates phagosomal escape and allows bacterial growth in the cytosol of infected cells. In order to identify new LLO determinants participating in bacterial pathogenesis, this study focused on a major target of LLO proteolytic cleavage in vitro, the CTL epitope region (residues 91-99). Mutations were generated by site-directed mutagenesis in the epitope or in the two clusters of positive charges flanking the epitope. Two LLO mutants (a single mutation K103A and a double mutation R89G, K90G) were normally and stably secreted by L. monocytogenes. In contrast, a mutant carrying four amino acid substitutions in the epitope itself (Y92K, D94A, E97K, Y98F) was highly susceptible to proteolytic degradation. While these three LLO mutant proteins showed a reduced haemolytic activity, they all promoted efficient phagosomal escape and intracellular multiplication in different cell types, and were non-cytotoxic. The deletion of the epitope (Delta91-99), as well as the substitution of two, three or four of the four lysine residues (K103 to K106) by alanine residues did not lead to the production of a detectable protein. These results confirm the lack of correlation between haemolytic activity and phagosomal membrane disruption. They reveal the importance of the 91-99 region in the production of a stable and functional LLO. LD(50) determinations in the mouse model suggest a possible link between LLO stability and virulence. FAU - Lety, Marie-Annick AU - Lety MA AD - Faculté de Médecine Necker-Enfants Malades, INSERM U-570, 156, rue de Vaugirard, 75730 Paris Cedex 15, France. FAU - Frehel, Claude AU - Frehel C AD - Faculté de Médecine Necker-Enfants Malades, INSERM U-570, 156, rue de Vaugirard, 75730 Paris Cedex 15, France. FAU - Raynaud, Catherine AU - Raynaud C AD - Faculté de Médecine Necker-Enfants Malades, INSERM U-570, 156, rue de Vaugirard, 75730 Paris Cedex 15, France. FAU - Dupuis, Marion AU - Dupuis M AD - Faculté de Médecine Necker-Enfants Malades, INSERM U-570, 156, rue de Vaugirard, 75730 Paris Cedex 15, France. FAU - Charbit, Alain AU - Charbit A AD - Faculté de Médecine Necker-Enfants Malades, INSERM U-570, 156, rue de Vaugirard, 75730 Paris Cedex 15, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Microbiology (Reading) JT - Microbiology (Reading, England) JID - 9430468 RN - 0 (Bacterial Toxins) RN - 0 (Epitopes, T-Lymphocyte) RN - 0 (Heat-Shock Proteins) RN - 0 (Hemolysin Proteins) RN - 0 (Virulence Factors) RN - R06ZRQ1YX9 (hlyA protein, Listeria monocytogenes) SB - IM MH - Amino Acid Sequence MH - Amino Acid Substitution MH - Animals MH - Bacterial Toxins/chemistry/genetics/*immunology/metabolism MH - Disease Models, Animal MH - Epitopes, T-Lymphocyte/genetics/*immunology MH - Female MH - Heat-Shock Proteins/chemistry/genetics/*immunology/metabolism MH - Hemolysin Proteins MH - Hemolysis MH - Lethal Dose 50 MH - Listeria monocytogenes/genetics/*pathogenicity MH - Listeriosis/microbiology MH - Macrophages/microbiology MH - Mice MH - Molecular Sequence Data MH - Mutagenesis, Site-Directed MH - Mutation, Missense MH - Phagosomes/microbiology MH - Sequence Deletion MH - Survival Analysis MH - Virulence Factors/chemistry/genetics/*immunology/metabolism EDAT- 2006/04/20 09:00 MHDA- 2006/09/22 09:00 CRDT- 2006/04/20 09:00 PHST- 2006/04/20 09:00 [pubmed] PHST- 2006/09/22 09:00 [medline] PHST- 2006/04/20 09:00 [entrez] AID - 10.1099/mic.0.28754-0 [doi] PST - ppublish SO - Microbiology (Reading). 2006 May;152(Pt 5):1287-1296. doi: 10.1099/mic.0.28754-0. PMID- 41176151 OWN - NLM STAT- MEDLINE DCOM- 20251128 LR - 20260130 IS - 1878-3511 (Electronic) IS - 1201-9712 (Linking) VI - 161 DP - 2025 Dec TI - Cytomegalovirus infection prevention counseling during pregnancy in France: A national population-based study. PG - 108167 LID - S1201-9712(25)00389-3 [pii] LID - 10.1016/j.ijid.2025.108167 [doi] AB - OBJECTIVES: Congenital cytomegalovirus (CMV) infection can cause sensorineural impairment in children. Our primary objective was to estimate the proportion of women in France who received counseling from health care providers about preventing CMV transmission during pregnancy, and our secondary objective was to identify factors associated with receipt of such counseling. METHODS: Using data collected at childbirth in France from women included in the 2021 national perinatal survey (ENP), we first estimated the proportion of women who received counseling about preventing CMV infection during pregnancy, and then used univariate and multivariate analyses to compare maternal characteristics between women who reported such counseling and those who reported not being counseled or not remembering. RESULTS: Among the 10,866 women interviewed, 1737 (16.0%) reported being counseled about CMV during pregnancy. Those born in North African or sub-Saharan countries were less likely to have been counseled (respectively, adjusted odds ratio 0.49, 95% confidence interval [CI] 0.34-0.71 and 0.58, 95% CI 0.37-0.89) compared with women born in France, as were those with a low educational level (0.56, 95% CI, 0.48-0.66 for women with high school level, compared with women with ≥3-year post-secondary schooling). Compared with multiparous women, primiparous women were less likely to have been counseled (adjusted odds ratio 0.61, 95% CI 0.53-0.69). CONCLUSIONS: Too few women receive counseling about CMV prevention during pregnancy. The women least informed were less educated and more socially vulnerable. They are probably also the women who have the least access to preventive health information. CI - Copyright © 2025 The Authors. Published by Elsevier Ltd.. All rights reserved. FAU - Anselem, Olivia AU - Anselem O AD - Maternité Port Royal, Groupe Hospitalier Paris Centre, AP-HP, FHU Prema, Paris, France. Electronic address: olivia.anselem@aphp.fr. FAU - Charlier, Caroline AU - Charlier C AD - Equipe Mobile d'Infectiologie, Groupe Hospitalier Paris Centre, AP-HP, FHU Prema, Paris, France. FAU - Vaux, Sophie AU - Vaux S AD - Santé Publique France, Saint Maurice, France. FAU - Lelong, Nathalie AU - Lelong N AD - Université Paris Cité, INSERM U1153, OPPaLE (Obstetric, Perinatal, Paediatric Life Course Epidemiology) Research Team, Center for Research in Epidemiology and Statistics (CRESS), Paris, France. FAU - Le Ray, Camille AU - Le Ray C AD - Maternité Port Royal, Groupe Hospitalier Paris Centre, AP-HP, FHU Prema, Paris, France; Université Paris Cité, INSERM U1153, OPPaLE (Obstetric, Perinatal, Paediatric Life Course Epidemiology) Research Team, Center for Research in Epidemiology and Statistics (CRESS), Paris, France. CN - ENP2021 Study Group LA - eng PT - Journal Article DEP - 20251030 PL - Canada TA - Int J Infect Dis JT - International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases JID - 9610933 SB - IM MH - Humans MH - Female MH - Pregnancy MH - *Cytomegalovirus Infections/prevention & control/epidemiology MH - France/epidemiology MH - Adult MH - *Pregnancy Complications, Infectious/prevention & control MH - *Infectious Disease Transmission, Vertical/prevention & control MH - *Counseling/statistics & numerical data MH - Young Adult MH - Cytomegalovirus OTO - NOTNLM OT - CMV OT - Infection OT - Information OT - Pregnancy OT - Prevention COIS- Declaration of competing interest The authors have no competing interests to declare. FIR - Lelong, Nathalie IR - Lelong N IRAD- Inserm EPOPé. FIR - Cinelli, Hélène IR - Cinelli H IRAD- Inserm EPOPé. FIR - Blondel, Béatrice IR - Blondel B IRAD- Inserm EPOPé. FIR - Regnault, Nolwenn IR - Regnault N IRAD- Santé publique France. FIR - Demiguel, Virginie IR - Demiguel V IRAD- Santé publique France. FIR - Lebreton, Elodie IR - Lebreton E IRAD- Santé publique France. FIR - Salanave, Benoit IR - Salanave B IRAD- Santé publique France. FIR - Fresson, Jeanne IR - Fresson J IRAD- Direction de la Recherche, des Etudes, de l'Evaluation et des Statistiques. FIR - Vilain, Annick IR - Vilain A IRAD- Direction de la Recherche, des Etudes, de l'Evaluation et des Statistiques. FIR - Deroyon, Thomas IR - Deroyon T IRAD- Direction de la Recherche, des Etudes, de l'Evaluation et des Statistiques. FIR - Raynaud, Philippe IR - Raynaud P IRAD- Direction de la Recherche, des Etudes, de l'Evaluation et des Statistiques. FIR - Rey, Sylvie IR - Rey S IRAD- Direction de la Recherche, des Etudes, de l'Evaluation et des Statistiques. FIR - Chemlal, Khadoudja IR - Chemlal K IRAD- Direction Générale de la Santé. FIR - Rabier-Thoreau, Nathalie IR - Rabier-Thoreau N IRAD- Direction Générale de la Santé. FIR - Collombet-Migeon, Frédérique IR - Collombet-Migeon F IRAD- Direction Générale de l'Offre de Soin. EDAT- 2025/11/02 00:33 MHDA- 2025/11/29 02:21 CRDT- 2025/11/01 20:14 PHST- 2025/09/22 00:00 [received] PHST- 2025/10/24 00:00 [revised] PHST- 2025/10/27 00:00 [accepted] PHST- 2025/11/29 02:21 [medline] PHST- 2025/11/02 00:33 [pubmed] PHST- 2025/11/01 20:14 [entrez] AID - S1201-9712(25)00389-3 [pii] AID - 10.1016/j.ijid.2025.108167 [doi] PST - ppublish SO - Int J Infect Dis. 2025 Dec;161:108167. doi: 10.1016/j.ijid.2025.108167. Epub 2025 Oct 30. PMID- 26443594 OWN - NLM STAT- MEDLINE DCOM- 20160920 LR - 20260127 IS - 1460-2083 (Electronic) IS - 0964-6906 (Linking) VI - 24 IP - 25 DP - 2015 Dec 20 TI - Increased STAG2 dosage defines a novel cohesinopathy with intellectual disability and behavioral problems. PG - 7171-81 LID - 10.1093/hmg/ddv414 [doi] AB - Next generation genomic technologies have made a significant contribution to the understanding of the genetic architecture of human neurodevelopmental disorders. Copy number variants (CNVs) play an important role in the genetics of intellectual disability (ID). For many CNVs, and copy number gains in particular, the responsible dosage-sensitive gene(s) have been hard to identify. We have collected 18 different interstitial microduplications and 1 microtriplication of Xq25. There were 15 affected individuals from 6 different families and 13 singleton cases, 28 affected males in total. The critical overlapping region involved the STAG2 gene, which codes for a subunit of the cohesin complex that regulates cohesion of sister chromatids and gene transcription. We demonstrate that STAG2 is the dosage-sensitive gene within these CNVs, as gains of STAG2 mRNA and protein dysregulate disease-relevant neuronal gene networks in cells derived from affected individuals. We also show that STAG2 gains result in increased expression of OPHN1, a known X-chromosome ID gene. Overall, we define a novel cohesinopathy due to copy number gain of Xq25 and STAG2 in particular. CI - © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com. FAU - Kumar, Raman AU - Kumar R AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Corbett, Mark A AU - Corbett MA AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Van Bon, Bregje W M AU - Van Bon BW AD - Radboud University Medical Center, 6525 GA, Nijmegen, The Netherlands. FAU - Gardner, Alison AU - Gardner A AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Woenig, Joshua A AU - Woenig JA AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Jolly, Lachlan A AU - Jolly LA AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Douglas, Evelyn AU - Douglas E AD - Genetics and Molecular Pathology, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Friend, Kathryn AU - Friend K AD - Genetics and Molecular Pathology, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Tan, Chuan AU - Tan C AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia. FAU - Van Esch, Hilde AU - Van Esch H AD - Center for Human Genetics, University Hospitals Leuven, 3000 Leuven, Belgium. FAU - Holvoet, Maureen AU - Holvoet M AD - Center for Human Genetics, University Hospitals Leuven, 3000 Leuven, Belgium. FAU - Raynaud, Martine AU - Raynaud M AD - Centre Hospitalier Régional Universitaire, Service de Génétique, 37000 Tours, France. FAU - Field, Michael AU - Field M AD - Genetics of Learning Disability Service, Hunter Genetics, Waratah, NSW 2298, Australia. FAU - Leffler, Melanie AU - Leffler M AD - Genetics of Learning Disability Service, Hunter Genetics, Waratah, NSW 2298, Australia. FAU - Budny, Bartłomiej AU - Budny B AD - Department of Endocrinology, Metabolism and Internal Diseases and. FAU - Wisniewska, Marzena AU - Wisniewska M AD - Department of Medical Genetics, Poznan University of Medical Sciences, Poznan 60-355, Poland. FAU - Badura-Stronka, Magdalena AU - Badura-Stronka M AD - Department of Medical Genetics, Poznan University of Medical Sciences, Poznan 60-355, Poland. FAU - Latos-Bieleńska, Anna AU - Latos-Bieleńska A AD - Department of Medical Genetics, Poznan University of Medical Sciences, Poznan 60-355, Poland. FAU - Batanian, Jacqueline AU - Batanian J AD - Department of Pediatrics, Saint Louis University, St Louis, MO 63104, USA. FAU - Rosenfeld, Jill A AU - Rosenfeld JA AD - Signature Genomic Laboratories, Spokane, WA 99207, USA, Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. FAU - Basel-Vanagaite, Lina AU - Basel-Vanagaite L AD - Raphael Recanati Genetic Institute and Felsenstein Medical Research Center, Rabin Medical Center, Beilinson Campus, Petah Tikva 49100, Israel. FAU - Jensen, Corinna AU - Jensen C AD - Department of Human Molecular Genetics and. FAU - Bienek, Melanie AU - Bienek M AD - Department of Human Molecular Genetics and. FAU - Froyen, Guy AU - Froyen G AD - Human Genome Laboratory, Department of Human Genetics, KU Leuven, 3000 Leuven, Belgium and. FAU - Ullmann, Reinhard AU - Ullmann R AD - Department of Human Molecular Genetics and, Bundeswehr Institute of Radiobiology, 80937 Munich, Germany. FAU - Hu, Hao AU - Hu H AD - Department of Human Molecular Genetics and. FAU - Love, Michael I AU - Love MI AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Ihnestrasse 73, 14195 Berlin, Germany. FAU - Haas, Stefan A AU - Haas SA AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Ihnestrasse 73, 14195 Berlin, Germany. FAU - Stankiewicz, Pawel AU - Stankiewicz P AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. FAU - Cheung, Sau Wai AU - Cheung SW AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. FAU - Baxendale, Anne AU - Baxendale A AD - South Australian Clinical Genetics Service, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Nicholl, Jillian AU - Nicholl J AD - Genetics and Molecular Pathology, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Thompson, Elizabeth M AU - Thompson EM AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia, South Australian Clinical Genetics Service, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Haan, Eric AU - Haan E AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia, South Australian Clinical Genetics Service, SA Pathology, North Adelaide, SA 5006, Australia. FAU - Kalscheuer, Vera M AU - Kalscheuer VM AD - Department of Human Molecular Genetics and. FAU - Gecz, Jozef AU - Gecz J AD - School of Medicine, and the Robinson Research Institute, The University of Adelaide, Adelaide, SA 5000, Australia, jozef.gecz@adelaide.edu.au. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20151006 PL - England TA - Hum Mol Genet JT - Human molecular genetics JID - 9208958 RN - 0 (Antigens, Nuclear) RN - 0 (Cell Cycle Proteins) RN - 0 (STAG2 protein, human) RN - 0 (Cohesins) SB - IM MH - Antigens, Nuclear/*genetics MH - Cell Cycle Proteins MH - Chromosomes, Human, X/genetics MH - DNA Copy Number Variations/genetics MH - Humans MH - Intellectual Disability/*genetics MH - Male MH - Problem Behavior MH - Reverse Transcriptase Polymerase Chain Reaction MH - Cohesins EDAT- 2015/10/08 06:00 MHDA- 2016/09/22 06:00 CRDT- 2015/10/08 06:00 PHST- 2015/08/10 00:00 [received] PHST- 2015/09/28 00:00 [accepted] PHST- 2015/10/08 06:00 [entrez] PHST- 2015/10/08 06:00 [pubmed] PHST- 2016/09/22 06:00 [medline] AID - ddv414 [pii] AID - 10.1093/hmg/ddv414 [doi] PST - ppublish SO - Hum Mol Genet. 2015 Dec 20;24(25):7171-81. doi: 10.1093/hmg/ddv414. Epub 2015 Oct 6. PMID- 22789543 OWN - NLM STAT- MEDLINE DCOM- 20120917 LR - 20250529 IS - 1878-3686 (Electronic) IS - 1535-6108 (Print) IS - 1535-6108 (Linking) VI - 22 IP - 1 DP - 2012 Jul 10 TI - The ALK(F1174L) mutation potentiates the oncogenic activity of MYCN in neuroblastoma. PG - 117-30 LID - 10.1016/j.ccr.2012.06.001 [doi] AB - The ALK(F1174L) mutation is associated with intrinsic and acquired resistance to crizotinib and cosegregates with MYCN in neuroblastoma. In this study, we generated a mouse model overexpressing ALK(F1174L) in the neural crest. Compared to ALK(F1174L) and MYCN alone, co-expression of these two oncogenes led to the development of neuroblastomas with earlier onset, higher penetrance, and enhanced lethality. ALK(F1174L)/MYCN tumors exhibited increased MYCN dosage due to ALK(F1174L)-induced activation of the PI3K/AKT/mTOR and MAPK pathways, coupled with suppression of MYCN pro-apoptotic effects. Combined treatment with the ATP-competitive mTOR inhibitor Torin2 overcame the resistance of ALK(F1174L)/MYCN tumors to crizotinib. Our findings demonstrate a pathogenic role for ALK(F1174L) in neuroblastomas overexpressing MYCN and suggest a strategy for improving targeted therapy for ALK-positive neuroblastoma. CI - Copyright © 2012 Elsevier Inc. All rights reserved. FAU - Berry, Teeara AU - Berry T AD - Division of Clinical Studies, The Institute of Cancer Research, Sutton, Surrey SM2 5NG, UK. FAU - Luther, William AU - Luther W FAU - Bhatnagar, Namrata AU - Bhatnagar N FAU - Jamin, Yann AU - Jamin Y FAU - Poon, Evon AU - Poon E FAU - Sanda, Takaomi AU - Sanda T FAU - Pei, Desheng AU - Pei D FAU - Sharma, Bandana AU - Sharma B FAU - Vetharoy, Winston R AU - Vetharoy WR FAU - Hallsworth, Albert AU - Hallsworth A FAU - Ahmad, Zai AU - Ahmad Z FAU - Barker, Karen AU - Barker K FAU - Moreau, Lisa AU - Moreau L FAU - Webber, Hannah AU - Webber H FAU - Wang, Wenchao AU - Wang W FAU - Liu, Qingsong AU - Liu Q FAU - Perez-Atayde, Antonio AU - Perez-Atayde A FAU - Rodig, Scott AU - Rodig S FAU - Cheung, Nai-Kong AU - Cheung NK FAU - Raynaud, Florence AU - Raynaud F FAU - Hallberg, Bengt AU - Hallberg B FAU - Robinson, Simon P AU - Robinson SP FAU - Gray, Nathanael S AU - Gray NS FAU - Pearson, Andrew D J AU - Pearson AD FAU - Eccles, Suzanne A AU - Eccles SA FAU - Chesler, Louis AU - Chesler L FAU - George, Rani E AU - George RE LA - eng SI - GEO/GSE35560 GR - A10294/CRUK_/Cancer Research UK/United Kingdom GR - CA136851-01A1/CA/NCI NIH HHS/United States GR - 091763Z/10/Z/WT_/Wellcome Trust/United Kingdom GR - C1060/A10334/CRUK_/Cancer Research UK/United Kingdom GR - G1000121/1/NC3RS_/National Centre for the Replacement, Refinement and Reduction of Animals in Research/United Kingdom GR - R01 CA148688/CA/NCI NIH HHS/United States GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - R01 CA136851/CA/NCI NIH HHS/United States GR - C309/A8274/CRUK_/Cancer Research UK/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - A14610/CRUK_/Cancer Research UK/United Kingdom GR - DH_/Department of Health/United Kingdom GR - NC3R-G1000121/94513/MRC_/Medical Research Council/United Kingdom GR - WT_/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Cancer Cell JT - Cancer cell JID - 101130617 RN - 0 (MYCN protein, mouse) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (Proto-Oncogene Proteins) RN - 0 (RNA, Messenger) RN - EC 2.7.10.1 (Alk protein, mouse) RN - EC 2.7.10.1 (Anaplastic Lymphoma Kinase) RN - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases) SB - IM MH - Anaplastic Lymphoma Kinase MH - Animals MH - Disease Models, Animal MH - Mice MH - Mice, Transgenic MH - *Mutation MH - N-Myc Proto-Oncogene Protein MH - Neuroblastoma/*genetics/pathology MH - *Oncogenes MH - Proto-Oncogene Proteins/genetics/*physiology MH - RNA, Messenger/genetics MH - Receptor Protein-Tyrosine Kinases/*genetics MH - Signal Transduction PMC - PMC3417812 MID - NIHMS386630 EDAT- 2012/07/14 06:00 MHDA- 2012/09/18 06:00 PMCR- 2013/07/10 CRDT- 2012/07/14 06:00 PHST- 2012/01/14 00:00 [received] PHST- 2012/03/18 00:00 [revised] PHST- 2012/06/05 00:00 [accepted] PHST- 2012/07/14 06:00 [entrez] PHST- 2012/07/14 06:00 [pubmed] PHST- 2012/09/18 06:00 [medline] PHST- 2013/07/10 00:00 [pmc-release] AID - S1535-6108(12)00253-X [pii] AID - 10.1016/j.ccr.2012.06.001 [doi] PST - ppublish SO - Cancer Cell. 2012 Jul 10;22(1):117-30. doi: 10.1016/j.ccr.2012.06.001. PMID- 19703881 OWN - NLM STAT- MEDLINE DCOM- 20090915 LR - 20220409 IS - 1527-1315 (Electronic) IS - 0033-8419 (Linking) VI - 252 IP - 2 DP - 2009 Aug TI - Rapid-clearance iron nanoparticles for inflammation imaging of atherosclerotic plaque: initial experience in animal model. PG - 401-9 LID - 10.1148/radiol.2522081484 [doi] AB - PURPOSE: To evaluate the use of a recently developed fast-clearing ultrasmall superparamagnetic iron oxide (USPIO) for detection of vascular inflammation in atherosclerotic plaque. MATERIALS AND METHODS: The study protocol was approved by the animal experimentation ethics committee. A recently introduced USPIO, P904, and a reference-standard USPIO, ferumoxtran-10, were tested in a rabbit model of induced aortic atherosclerosis. In vivo magnetic resonance (MR) angiography and T2*-weighted plaque MR imaging were performed at baseline and after administration of P904 and ferumoxtran-10 (administered dose for both, 1000 micromol of iron per kilogram of body weight) in 26 hyperlipidemic New Zealand white rabbits. The variation in vessel wall area over time was evaluated with nonparametric testing. Ex vivo MR imaging findings were compared with iron content at linear regression analysis. RESULTS: With in vivo MR imaging, plaque analysis was possible as early as 24 hours after P904 injection. The authors observed a 27.75% increase in vessel wall area due to susceptibility artifacts on day 2 (P = .04) and a 38.81% increase on day 3 (P = .04) after P904 administration compared with a 44.5% increase in vessel wall area on day 7 (P = .04) and a 34.8% increase on day 10 (P = .22) after ferumoxtran-10 administration. These susceptibility artifacts were correlated with intraplaque iron uptake in the corresponding histologic slices. The number of pixels with signal loss on the ex vivo MR images was linearly correlated with the logarithm of the iron concentration (P = .0001; R(2) = 0.93). CONCLUSION: Plaque inflammation in rabbits can be detected earlier with P904 than with ferumoxtran-10 owing to the faster blood pharmacokinetics and the early uptake of P904 in the reticuloendothelial system. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/252/2/401/DC1. FAU - Sigovan, Monica AU - Sigovan M AD - Université Lyon 1, CREATIS-LRMN, UMR CNRS 5220, U630 INSERM, ESCPE, La Doua, 43 Boulevard du 11 Novembre 1918, 69622 Villeurbanne Cedex, France. FAU - Boussel, Loic AU - Boussel L FAU - Sulaiman, Abdulrazzaq AU - Sulaiman A FAU - Sappey-Marinier, Dominique AU - Sappey-Marinier D FAU - Alsaid, Hasan AU - Alsaid H FAU - Desbleds-Mansard, Catherine AU - Desbleds-Mansard C FAU - Ibarrola, Danielle AU - Ibarrola D FAU - Gamondès, Delphine AU - Gamondès D FAU - Corot, Claire AU - Corot C FAU - Lancelot, Eric AU - Lancelot E FAU - Raynaud, Jean-Sebastian AU - Raynaud JS FAU - Vives, Véronique AU - Vives V FAU - Laclédère, Christine AU - Laclédère C FAU - Violas, Xavier AU - Violas X FAU - Douek, Philippe C AU - Douek PC FAU - Canet-Soulas, Emmanuelle AU - Canet-Soulas E LA - eng PT - Comparative Study PT - Evaluation Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Radiology JT - Radiology JID - 0401260 RN - 0 (Contrast Media) RN - 0 (Dextrans) RN - 0 (Magnetite Nanoparticles) RN - 0 (Oxides) RN - 0 (ferumoxtran-10) RN - E1UOL152H7 (Iron) RN - XM0M87F357 (Ferrosoferric Oxide) SB - IM MH - Animals MH - Aortitis/*metabolism/*pathology MH - Atherosclerosis/*metabolism/*pathology MH - Contrast Media/pharmacokinetics MH - Dextrans MH - Disease Models, Animal MH - Ferrosoferric Oxide MH - Humans MH - Iron/*pharmacokinetics MH - Magnetic Resonance Angiography/*methods MH - Magnetite Nanoparticles MH - Metabolic Clearance Rate MH - Oxides/*pharmacokinetics MH - Pilot Projects MH - Rabbits MH - Reproducibility of Results MH - Sensitivity and Specificity EDAT- 2009/08/26 09:00 MHDA- 2009/09/16 06:00 CRDT- 2009/08/26 09:00 PHST- 2009/08/26 09:00 [entrez] PHST- 2009/08/26 09:00 [pubmed] PHST- 2009/09/16 06:00 [medline] AID - 252/2/401 [pii] AID - 10.1148/radiol.2522081484 [doi] PST - ppublish SO - Radiology. 2009 Aug;252(2):401-9. doi: 10.1148/radiol.2522081484. PMID- 40107904 OWN - NLM STAT- MEDLINE DCOM- 20250706 LR - 20250706 IS - 1468-2060 (Electronic) IS - 0003-4967 (Linking) VI - 84 IP - 7 DP - 2025 Jul TI - The role of multicriteria decision analysis in the development of candidate classification criteria for antisynthetase syndrome: analysis from the CLASS project. PG - 1207-1220 LID - S0003-4967(25)00204-3 [pii] LID - 10.1016/j.ard.2025.01.050 [doi] AB - OBJECTIVES: To develop and evaluate the performance of multicriteria decision analysis (MCDA)-driven candidate classification criteria for antisynthetase syndrome (ASSD). METHODS: A list of variables associated with ASSD was developed using a systematic literature review and then refined into an ASSD key domains and variables list by myositis and interstitial lung disease (ILD) experts. This list was used to create preferences surveys in which experts were presented with pairwise comparisons of clinical vignettes and asked to select the case that was more likely to represent ASSD. Experts' answers were analysed using the Potentially All Pairwise RanKings of all possible Alternatives method to determine the weights of the key variables to formulate the MCDA-based classification criteria. Clinical vignettes scored by the experts as consensus cases or controls and real-world data collected in participating centres were used to test the performance of candidate classification criteria using receiver operating characteristic curves and diagnostic accuracy metrics. RESULTS: Positivity for antisynthetase antibodies had the highest weight for ASSD classification. The highest-ranked clinical manifestation was ILD, followed by myositis, mechanic's hands, joint involvement, inflammatory rashes, Raynaud phenomenon, fever, and pulmonary hypertension. The candidate classification criteria achieved high areas under the curve when applied to the consensus cases and controls and real-world patient data. Sensitivities, specificities, and positive and negative predictive values were >80%. CONCLUSIONS: The MCDA-driven candidate classification criteria were consistent with published ASSD literature and yielded high accuracy and validity. CI - Copyright © 2025. Published by Elsevier B.V. FAU - Zanframundo, Giovanni AU - Zanframundo G AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Dourado, Eduardo AU - Dourado E AD - Rheumatology Department, Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal; Aveiro Rheumatology Research Centre, Egas Moniz Health Alliance, Aveiro, Portugal; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal. FAU - Bauer-Ventura, Iazsmin AU - Bauer-Ventura I AD - Section of Rheumatology, Department of Medicine, University of Chicago, Chicago, IL, USA. FAU - Faghihi-Kashani, Sara AU - Faghihi-Kashani S AD - University of Pittsburgh School of Medicine, Pittsburgh, PA, USA; Division of Clinical Informatics and Digital Transformation, University of California San Francisco, San Francisco, CA, USA. FAU - Yoshida, Akira AU - Yoshida A AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Loganathan, Aravinthan AU - Loganathan A AD - Royal National Hospital for Rheumatic Diseases, Bath, UK; Department of Life Sciences, University of Bath, Bath, UK; Arthritis Australia, Broadway, Glebe, NSW, Australia. FAU - Rivero-Gallegos, Daphne AU - Rivero-Gallegos D AD - Rheumatology Clinic, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico. FAU - Lim, Darosa AU - Lim D AD - Department of Dermatology, Perelman School of Medicine & Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center, Philadelphia, PA, USA. FAU - Bozán, Francisca AU - Bozán F AD - Section of Rheumatology, Department of Medicine, Hospital Clínico Universidad de Chile, Santiago de Chile, Chile. FAU - Sambataro, Gianluca AU - Sambataro G AD - Department of Medicine and Surgery, University of Enna "Kore", Enna, Italy. FAU - Bae, Sangmee Sharon AU - Bae SS AD - Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA. FAU - Yamano, Yasuhiko AU - Yamano Y AD - Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan. FAU - Bonella, Francesco AU - Bonella F AD - Center for Interstitial and Rare Lung Disease at Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany. FAU - Corte, Tamera J AU - Corte TJ AD - Royal Prince Alfred Hospital and University of Sydney, Sydney, NSW, Australia. FAU - Doyle, Tracy Jennifer AU - Doyle TJ AD - Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA. FAU - Fiorentino, David AU - Fiorentino D AD - Department of Dermatology, Stanford University School of Medicine, Redwood City, CA, USA. FAU - Gonzalez-Gay, Miguel Angel AU - Gonzalez-Gay MA AD - Division of Rheumatology, IIS-Fundación Jiménez Díaz, Madrid, Spain; Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain. FAU - Hudson, Marie AU - Hudson M AD - McGill University, Montreal, QC, Canada. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Lundberg, Ingrid E AU - Lundberg IE AD - Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Mammen, Andrew AU - Mammen A AD - Departments of Medicine and Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA; National Institute of Arthritis and Musculoskeletal and Skin Disorders, National Institutes of Health, Bethesda, MD, USA. FAU - McHugh, Neil AU - McHugh N AD - Department of Life Sciences, University of Bath, Bath, UK. FAU - Miller, Frederick W AU - Miller FW AD - Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Durham, NC, USA. FAU - Montecucco, Carlomaurizio AU - Montecucco C AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. FAU - Oddis, Chester V AU - Oddis CV AD - University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AD - Rheumatology Clinic, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico. FAU - Schmidt, Jens AU - Schmidt J AD - Department of Neurology, Neuromuscular Center, University Medical Center Göttingen, Göttingen, Germany; Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel Klinik Rüdersdorf, University Hospital of the Brandenburg Medical School; and Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AD - Systemic Autoimmune Diseases Unit, Internal Medicine Department, Vall d'Hebron University Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Werth, Victoria P AU - Werth VP AD - Department of Dermatology, Perelman School of Medicine & Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center, Philadelphia, PA, USA. FAU - Hansen, Paul AU - Hansen P AD - University of Otago, Dunedin, New Zealand. FAU - Rozza, Davide AU - Rozza D AD - Epidemiology Unit, Italian Society of Rheumatology, Milan, Italy. FAU - Scirè, Carlo A AU - Scirè CA AD - School of Medicine, University of Milano Bicocca, Milan, Italy; Rheumatology Unit, IRCCS San Gerardo dei Tintori Foundation, Monza, Italy. FAU - Sakellariou, Garifallia AU - Sakellariou G AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy. FAU - Kaneko, Yuko AU - Kaneko Y AD - Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. FAU - Triantafyllias, Konstantinos AU - Triantafyllias K AD - Rheumatology Center Rhineland-Palatinate, Bad Kreuznach, Germany. FAU - Castañeda, Santos AU - Castañeda S AD - Division of Rheumatology, Hospital Universitario de la Princesa, IIS-Princesa, Madrid, Spain. FAU - Alberti, Maria Laura AU - Alberti ML AD - Hospital de Rehabilitación Respiratoria "María Ferrer", Ciudad de Buenos Aires, Buenos Aires, Argentina. FAU - Merino, Martín Gerardo Greco AU - Merino MGG AD - Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, Spain. FAU - Fiehn, Christopher AU - Fiehn C AD - Rheumatology Baden-Baden, Baden-Baden, Germany. FAU - Molad, Yair AU - Molad Y AD - Institute of Rheumatology, Rabin Medical Center, Beilinson Hospital, and Faculty of Medicine, Tel Aviv University, Petach Tikva, Israel. FAU - Govoni, Marcello AU - Govoni M AD - Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy. FAU - Nakashima, Ran AU - Nakashima R AD - Graduate School of Medicine, Kyoto University, Kyoto, Japan. FAU - Alpsoy, Erkan AU - Alpsoy E AD - Akdeniz University, School of Medicine, Antalya, Turkey. FAU - Giannini, Margherita AU - Giannini M AD - Explorations Fonctionnelles Musculaires, Service de Physiologie, Centre de Référence des Maladies Autoimmunes Rares (RESO), Hôpitaux Universitaires de Strasbourg, UR3072 Centre de Recherche en Biomédecine, Université de Strasbourg, Strasbourg, France. FAU - Chinoy, Hector AU - Chinoy H AD - Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK. FAU - Gallay, Laure AU - Gallay L AD - CHU Lyon, Lyon, France. FAU - Ebstein, Esther AU - Ebstein E AD - Bichat-Claude Bernard Hospital, Paris, France. FAU - Campagne, Julien AU - Campagne J AD - Robert Schuman Hospital, Metz, France. FAU - Saraiva, André Pinto AU - Saraiva AP AD - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. FAU - Conticini, Edoardo AU - Conticini E AD - University of Siena, Siena, Italy. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AD - Rheumatology Unit, San Camillo Hospital, Roma, Italy. FAU - Nuño-Nuño, Laura AU - Nuño-Nuño L AD - Hospital La Paz, Madrid, Spain. FAU - Scarpato, Salvatore AU - Scarpato S AD - Ospedale "Scarlato" Scafati, Scafati, Italy. FAU - Schiopu, Elena AU - Schiopu E AD - Medical College of Georgia at Augusta University, Augusta, GA, USA. FAU - Parker, Matthew AU - Parker M AD - Royal Prince Alfred Hospital and University of Sydney, Sydney, NSW, Australia. FAU - Limonta, Massimiliano AU - Limonta M AD - ASST Papa Giovanni XXIII, Bergamo, Lombardy, Italy. CN - CLASS project participating investigators FAU - Cavagna, Lorenzo AU - Cavagna L AD - Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. Electronic address: lorenzo.cavagna@unipv.it. FAU - Aggarwal, Rohit AU - Aggarwal R AD - University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. LA - eng PT - Journal Article DEP - 20250318 PL - United States TA - Ann Rheum Dis JT - Annals of the rheumatic diseases JID - 0372355 RN - 0 (Autoantibodies) RN - Antisynthetase syndrome SB - IM MH - Humans MH - *Myositis/classification/diagnosis/immunology MH - *Decision Support Techniques MH - Lung Diseases, Interstitial/diagnosis MH - ROC Curve MH - Autoantibodies/blood COIS- Competing interests The authors report the following potentially relevant financial activities: GS has received speaking fees from Boehringer Ingelheim. FB has received consulting fees from Boehringer Ingelheim, Sanofi, Bristol Myers Squibb, and Savara Pharma; speaking fees from Boehringer Ingelheim and Sanofi; and support for attending meetings from Boehringer Ingelheim, AstraZeneca, Atyr, and Savara Pharma. TC has received consulting fees from 4D, Avalyn Therapeutics, Boehringer Ingleheim, Bridge Biotherapeutics, Bristol Myers Squibb, Cincera, DevPro, Endeavour BioMedicine, Pharmaxis, Pliant, and Roche; and speaking fees from Boehringer Ingleheim, Bristol Myers Squibb, and Roche. TD has received grants from Bayer, Genentech, and Sanofi. DFF has received consulting fees from Annexon, Argenx, Biogen, Bristol Meyers Squibb, IgM, Kyverna, Nuvig, Octapharma, Paragon, Pfizer, and Priovant. MH has received grants from AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, and Pfizer; and speaking fees from AstraZeneca and Boehringer Ingelheim. MK has received research grants from Boehringer Ingelheim, MBL, and Ono Pharmaceuticals; speaking fees from Asahi Kasei Parma, Boehringer Ingelheim, Chugai, Eisai, Janssen, Kissei, Mochida, Nippon Shinyaku, and Ono Pharmaceuticals; and consulting fees from AstraZeneca, Boehringer Ingelheim, Chugai, GSK, Kissei, and Mochida. IL has received consulting fees from Argenx, Bristol Myers Squibb, Chugai, Corbus Pharmaceutical, EMD Serono Research & Development Institute, Galapagos, and Pfizer; speaking fees from Boehringer Ingelheim; and has stock or stock options from Novartis and Roche. JS has received grants from Kezar; consulting fees from CSL Behring, DSMB, Grifols, Janssen, and Takeda; speaking fees from CSL Behring, Takeda, and UCB; and support for attending meetings from CSL Behring. VW has received grants from Amgen, AstraZeneca, Biogen, Bristol Myers Squibb, Caribou, Corbus Pharmaceuticals, CSL Behring, Gilead, Horizon, Pfizer, Priovant, Regeneron, Rome Pharmaceuticals, Ventus, and Viela; and consulting fees from AbbVie, Alpine Immune Sciences, Amgen, Anaptysbio, Argenx, AstraZeneca, Biogen, Bristol Myers Squibb, Cabaletta Bio, Calyx, Crisalis, CSL Behring, Cugene, EMD Sorona, Evommune, Gilead, GSK, Horizon, Immunovant, Innovaderm, Janssen, Kwoya Kirin, Lilly, Merck, Nektar, Nuvig Pharmaceuticals, Pfizer, Regeneron, Rome Pharmaceuticals, Sanofi, Takeda, UCB, Ventus, Viela Bio, and Xencor. CS has received grants from AbbVie, Galapagos, and Lilly; and consulting fees from AbbVie. GS has received speaking fees from Boehringer Ingelheim. SC has received speaking fees from Eli Lilly, GSK, Novartis, and UCB; support for attending meetings from Eli Lilly, Janssen, and Pfizer; and has received equipment, materials, drugs, medical writing, gifts or other services from Grunenthal and UCB. MLA has received speaking fees from Boehringer Ingelheim and Tecnofarma; and support for attending meetings from Boehringer Ingelheim, Tuteur, and Raffo. MG has received consulting fees from AstraZeneca and UCB; and speaking fees from AbbVie, AstraZeneca, Eli Lilly, and GSK; support for attending meetings from AstraZeneca. RN has received speaking fees from Astellas Pharma, Boehringer Ingelheim, Daiichi Sankyo, Medical & Biological Laboratories, and Mitsubishi Tanabe Pharma Corporation. EA has received speaking fees from AbbVie, Johnson & Johnson, Lilly, and UCB. HC has received grants from Pfizer; and consulting fees from AstraZeneca, Horizon Therapeutics, PTC Therapeutics, and Pfizer; speaking fees from GSK, and UCB. EE has received consulting fees from Bristol Myers Squibb, Galapagos, and Novartis; and support for attending meetings from AbbVie, Novartis, and UCB. APS has received speaking fees from Nordic Pharma; and has stock or stock options from Pfizer. EC has received consulting fees from GSK; and speaking fees from Eli Lilly and GSK. LNN has received support for attending meetings from AbbVie, Nordic Pharma, and Novartis. MP has received speaking fees from Boehringer Ingelheim. LC has received consulting fees from Boehringer Ingelheim; and speaking fees from Alexion, Boehringer Ingelheim, and GSK. RA has received grants from Boehringer Ingelheim, Bristol Myers Squibb, EMD Serono, Janssen, Mallinckrodt, Pfizer, and Q32; and consulting fees from Actigraph, Alexion, ANI Pharmaceuticals, Argenx, AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, Cabaletta Bio, Capella Bioscience, Corbus, CSL Behring, EMD Serono, Galapagos, Horizon Therapeutics, I-Cell, Janssen, Kezar, Kyverna, Merck, Novartis, Nuvig Therapeutics, Octapharma, Pfizer, Regeneron, Roivant, Sanofi, Teva, Artsome, Capstanx, and Manta. The remaining authors have no conflicts of interest to disclose. EDAT- 2025/03/20 00:33 MHDA- 2025/07/07 00:26 CRDT- 2025/03/19 23:42 PHST- 2024/10/26 00:00 [received] PHST- 2025/01/17 00:00 [revised] PHST- 2025/01/21 00:00 [accepted] PHST- 2025/07/07 00:26 [medline] PHST- 2025/03/20 00:33 [pubmed] PHST- 2025/03/19 23:42 [entrez] AID - S0003-4967(25)00204-3 [pii] AID - 10.1016/j.ard.2025.01.050 [doi] PST - ppublish SO - Ann Rheum Dis. 2025 Jul;84(7):1207-1220. doi: 10.1016/j.ard.2025.01.050. Epub 2025 Mar 18. PMID- 42157907 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20260520 LR - 20260520 IS - 2754-0413 (Electronic) IS - 2754-0413 (Linking) VI - 5 IP - 1 DP - 2026 TI - Qualified prediction system for allograft failure in real world settings: extended validation study. PG - e002088 LID - 10.1136/bmjmed-2025-002088 [doi] LID - e002088 AB - OBJECTIVE: To perform comprehensive validations of the integrative Box (iBox) system, a prediction model for long term risk of kidney allograft failure, for extension of its context of use in clinical trials as well as for its wider implementation in clinical practice. DESIGN: Extended validation study. SETTING: Paris Transplant Group database (comprising kidney recipients with transplantations between 1 January 2005 and 1 January 2014) and European, North American, and South American hospitals (comprising recipients of kidneys transplanted beween 1 January 2000 and 1 January 2022). Patients were followed until 1 November 2024. PARTICIPANTS: 12 683 kidney tranplant recipients from 21 academic centres in Europe, North America, and South America; 4000 patients in the derivation cohort and 8683 in the validation cohorts. MAIN OUTCOME MEASURES: Performance of the iBox, including flexible iBox versions in specific clinical contexts (race-free estimated glomerular filtration rate (eGFR) equations (ie, without including race as a factor in the calculation), in specific clinical contexts (initial nephropathy recurrence, BK virus associated nephropathy, and different immunosuppressive strategies), and over-extended follow-up periods. Predictive performance was assessed by discrimination, calibration, overall fit, and clinical utility. RESULTS: 12 683 kidney transplant recipients were included in the study (n=4000 in the derivation cohort and n=8683 in the validation cohorts). Median follow-up time after risk evaluation was 5.78 years (interquartile range (IQR) 3.51-7.00) in the derivation cohort and 4.68 years (2.48-7.00) in the validation cohorts. 549 (13.7%) and 991 (11.4%) patients had graft loss in the derivation and validation cohorts, respectively. All versions of the iBox algorithm maintained good discrimination and overall fit performance in the derivation and validation cohorts (C index range 0.79-0.87, Brier scores 0.08-0.11). Calibration was adequate in some but not all external validation cohorts, with trends toward overestimation or underestimation of predicted risks. Decision curve analysis showed positive and comparable net benefit for all iBox algorithms across decision thresholds up to 40% in the derivation cohort (net benefit 0.07-0.08 at 20% threshold) and validation cohorts (net benefit 0.03-0.11 at 20% threshold). Accounting for the competing risk of death with a functioning graft resulted in similar performance, except for calibration which varied across cohorts, without any model consistently outperforming any other model. The model performed well with different race-free eGFR equations (C index 0.81), in various clinical scenarios, including disease recurrence and BK virus nephropathy, with different immunosuppressive strategies, such as calcineurin inhibitors and mTOR (mechanistic target of rapamycin) inhibitors (C index range 0.74-0.87), and when extending the prediction period to 10 years after risk evaluation (C index 0.79). The iBox predictive performance was not modified when various histological indices were used. The iBox was also superior to eGFR slope (C index 0.81 v 0.62) and circulating anti-HLA donor specific antibodies (C index 0.81 v 0.57) in its predictive ability. CONCLUSIONS: In this study, the robust predictive performance of the iBox system across diverse real world settings and clinical scenarios was shown. These results highlight the versatility and reliability of the iBox system, and support its use for risk stratification in routine clinical practice and as a surrogate endpoint for clinical trials. CI - Copyright © Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. FAU - Raynaud, Marc AU - Raynaud M AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. FAU - Truchot, Agathe AU - Truchot A AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. FAU - Naser, Sofia AU - Naser S AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. FAU - Aubert, Olivier AU - Aubert O AUID- ORCID: 0000-0001-6284-3757 AD - Kidney Transplant Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Divard, Gillian AU - Divard G AUID- ORCID: 0000-0002-3450-0033 AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. AD - Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Thalamas, Thibaut AU - Thalamas T AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. FAU - Lombardi, Yannis AU - Lombardi Y AUID- ORCID: 0000-0001-9031-1862 AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. AD - Department of Nephrology, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Legendre, Christophe AU - Legendre C AD - Kidney Transplant Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Bailly, Elodie AU - Bailly E AD - Nephrology and Immunology Department, Bretonneau Hospital, Tours, France. FAU - Buchler, Mathias AU - Buchler M AD - Nephrology and Immunology Department, Bretonneau Hospital, Tours, France. FAU - Crespo, Marta AU - Crespo M AUID- ORCID: 0000-0001-6992-6379 AD - Department of Nephrology, Hospital del Mar, Barcelona, Spain. FAU - Redondo, Dolores AU - Redondo D AD - Department of Nephrology, Hospital del Mar, Barcelona, Spain. FAU - Astor, Brad C AU - Astor BC AD - Division of Nephrology, Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. AD - Department of Population Health Sciences, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. FAU - Mandelbrot, Didier AU - Mandelbrot D AD - Division of Nephrology, Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. AD - Department of Population Health Sciences, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. FAU - Parajuli, Sandesh AU - Parajuli S AD - Division of Nephrology, Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. AD - Department of Population Health Sciences, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. FAU - Juric, Ivana AU - Juric I AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Basic-Jukic, Nikolina AU - Basic-Jukic N AD - Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. FAU - Helanterä, Ilkka AU - Helanterä I AD - Department of Transplantation and Liver Surgery, Helsinki University Central Hospital, Helsinki, Finland. FAU - Evans, Rhys D R AU - Evans RDR AD - Centre for Kidney and Bladder Health, University College London, Royal Free Hospital, London, UK. FAU - Sanghera, Aruna AU - Sanghera A AD - Centre for Kidney and Bladder Health, University College London, Royal Free Hospital, London, UK. FAU - Javed, Maryam AU - Javed M AD - Centre for Kidney and Bladder Health, University College London, Royal Free Hospital, London, UK. FAU - Molnar, Miklos Z AU - Molnar MZ AUID- ORCID: 0000-0002-9665-330X AD - Department of Internal Medicine, Division of Nephrology and Hypertension, Spencer Fox Eccles School of Medicine at the University of Utah, Salt Lake City, Utah, USA. FAU - Yamauchi, Junji AU - Yamauchi J AD - Department of Internal Medicine, Division of Nephrology and Hypertension, Spencer Fox Eccles School of Medicine at the University of Utah, Salt Lake City, Utah, USA. FAU - Fornadi, Katalin AU - Fornadi K AUID- ORCID: 0000-0003-3679-4503 AD - Department of Surgery, Division of Transplantation and Advanced Hepatobiliary Surgery, Spencer Fox Eccles School of Medicine at the University of Utah, Salt Lake City, Utah, USA. FAU - Linhares, Kamilla AU - Linhares K AD - Universidade Federal de Sao Paulo, Hospital do Rim, Escola Paulista de Medicina, Sao Paulo, Brazil. FAU - Baujard, Michel AU - Baujard M AD - Association "Le Flambeau de la Vie", Paris, France. FAU - Fowler, Kevin J AU - Fowler KJ AD - The Voice of the Patient, Saint Louis, Missouri, USA. FAU - Akalin, Enver AU - Akalin E AD - Albert Einstein College of Medicine, Renal Division Montefiore Medical Center, Kidney Transplantation Program, Bronx, New York, New York, USA. FAU - Gupta, Gaurav AU - Gupta G AD - Division of Nephrology, Department of Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, Virginia, USA. FAU - Soler Pujol, Gervacio AU - Soler Pujol G AD - Unidad de Trasplante Renopancreas, Centro de Educacion Medica e Investigaciones Clinicas, Buenos Aires, Argentina. FAU - Tedesco-Silva, Helio AU - Tedesco-Silva H AD - Universidade Federal de Sao Paulo, Hospital do Rim, Escola Paulista de Medicina, Sao Paulo, Brazil. FAU - Orandi, Babak J AU - Orandi BJ AD - NYU Grossman School of Medicine, Departments of Surgery and Medicine, New York, New York, USA. FAU - Naesens, Maarten AU - Naesens M AD - Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium. FAU - Budde, Klemens AU - Budde K AD - Department of Nephrology and Internal Intensive Care Medicine, Charité Universitätsmedizin Berlin, Berlin, Germany. FAU - Naik, Marcel AU - Naik M AD - Department of Nephrology and Internal Intensive Care Medicine, Charité Universitätsmedizin Berlin, Berlin, Germany. FAU - Hertig, Alexandre AU - Hertig A AD - Department of Transplantation, Nephrology, and Clinical Immunology, Foch Hospital, Suresnes, France. FAU - Anglicheau, Dany AU - Anglicheau D AD - Kidney Transplant Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Kamar, Nassim AU - Kamar N AD - Department of Nephrology and Organ Transplantation, Toulouse Rangueil University Hospital, INSERM UMR 1291, Toulouse Institute for Infectious and Inflammatory Diseases (Infinity), University Paul Sabatier, Toulouse, France. FAU - Segev, Dorry L AU - Segev DL AUID- ORCID: 0000-0002-1924-4801 AD - NYU Grossman School of Medicine, Departments of Surgery and Population Health, New York, New York, USA. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. AD - Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AUID- ORCID: 0000-0003-3388-7747 AD - Université de Paris Cité, INSERM, PARCC U970, Paris Institute for Transplantation and Organ Regeneration (PITOR), Paris, France. AD - Kidney Transplant Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. LA - eng PT - Journal Article DEP - 20260511 PL - England TA - BMJ Med JT - BMJ medicine JID - 9918487584306676 PMC - PMC13182363 OTO - NOTNLM OT - Kidney transplantation OT - Prognosis OT - Statistics COIS- All authors have completed the ICMJE uniform disclosure form at www.icmje.org/disclosure-of-interest/ and declare: support from INSERM-Action thematique incitative sur programme Avenir and OrganX for the submitted work; AL holds minor shares in Okeiro, a company that builds medical devices; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work. EDAT- 2026/05/20 06:31 MHDA- 2026/05/20 06:32 PMCR- 2026/05/11 CRDT- 2026/05/20 04:34 PHST- 2025/09/02 00:00 [received] PHST- 2026/04/09 00:00 [accepted] PHST- 2026/05/20 06:32 [medline] PHST- 2026/05/20 06:31 [pubmed] PHST- 2026/05/20 04:34 [entrez] PHST- 2026/05/11 00:00 [pmc-release] AID - bmjmed-2025-002088 [pii] AID - 10.1136/bmjmed-2025-002088 [doi] PST - epublish SO - BMJ Med. 2026 May 11;5(1):e002088. doi: 10.1136/bmjmed-2025-002088. eCollection 2026. PMID- 32435753 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231103 IS - 2589-5559 (Electronic) IS - 2589-5559 (Linking) VI - 2 IP - 3 DP - 2020 Jun TI - Hepatobiliary MR contrast agents are useful to diagnose hepatocellular carcinoma in patients with Budd-Chiari syndrome. PG - 100097 LID - 10.1016/j.jhepr.2020.100097 [doi] LID - 100097 AB - BACKGROUND & AIMS: Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. METHODS: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features. RESULTS: Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 vs. 33 ± 9 years old, p = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] vs. 1/22 [5%] with AFP >15 ng/ml, p <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions (p <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs. CONCLUSION: Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS. LAY SUMMARY: Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome. CI - © 2020 The Author(s). FAU - Van Wettere, Morgane AU - Van Wettere M AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Paulatto, Luisa AU - Paulatto L AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Bruno, Onorina AU - Bruno O AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Payancé, Audrey AU - Payancé A AD - Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. FAU - Plessier, Aurélie AU - Plessier A AD - Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. FAU - Rautou, Pierre-Emmanuel AU - Rautou PE AD - Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. AD - Inserm, U970, Paris Cardiovascular Research Center - PARCC, University of Paris, Paris, France. AD - DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France. FAU - Paradis, Valérie AU - Paradis V AD - University of Paris, Paris, France. AD - Department of Pathology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Cazals-Hatem, Dominique AU - Cazals-Hatem D AD - Department of Pathology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. FAU - Valla, Dominique AU - Valla D AD - Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. AD - DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France. FAU - Vilgrain, Valérie AU - Vilgrain V AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. AD - INSERM U1149, CRI, Paris, France. FAU - Ronot, Maxime AU - Ronot M AD - Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France. AD - University of Paris, Paris, France. AD - INSERM U1149, CRI, Paris, France. LA - eng PT - Journal Article DEP - 20200309 PL - Netherlands TA - JHEP Rep JT - JHEP reports : innovation in hepatology JID - 101761237 PMC - PMC7232085 OTO - NOTNLM OT - AFP, alpha-fetoprotein OT - APHE, arterial phase hyperenhancement OT - BCS, Budd-Chiari syndrome OT - FNH, focal nodular hyperplasia-like OT - HBP, hepatobiliary phase OT - HCC OT - HCC, hepatocellular carcinoma OT - Imaging OT - LR, likelihood ratio OT - MRI OT - OATP, organic anionic transporting polypeptides OT - T1-w, T1-weighted imaging OT - TIPS, transjugular intrahepatic portosystemic shunt OT - WO, washout OT - liver cancer OT - non-invasive OT - tumor OT - vascular liver disease COIS- The authors declare no conflicts of interest that pertain to this work. Please refer to the accompanying ICMJE disclosure forms for further details. EDAT- 2020/05/22 06:00 MHDA- 2020/05/22 06:01 PMCR- 2020/03/09 CRDT- 2020/05/22 06:00 PHST- 2020/02/11 00:00 [received] PHST- 2020/02/24 00:00 [accepted] PHST- 2020/05/22 06:00 [entrez] PHST- 2020/05/22 06:00 [pubmed] PHST- 2020/05/22 06:01 [medline] PHST- 2020/03/09 00:00 [pmc-release] AID - S2589-5559(20)30031-8 [pii] AID - 100097 [pii] AID - 10.1016/j.jhepr.2020.100097 [doi] PST - epublish SO - JHEP Rep. 2020 Mar 9;2(3):100097. doi: 10.1016/j.jhepr.2020.100097. eCollection 2020 Jun. PMID- 39854688 OWN - NLM STAT- MEDLINE DCOM- 20250409 LR - 20250409 IS - 1528-1175 (Electronic) IS - 0003-3022 (Linking) VI - 142 IP - 5 DP - 2025 May 1 TI - Impact of Underassisted Ventilation on Diaphragm Function and Structure in a Porcine Model. PG - 896-906 LID - 10.1097/ALN.0000000000005390 [doi] AB - BACKGROUND: Long-term controlled mechanical ventilation in the intensive care unit induces ventilator-induced diaphragm dysfunction (VIDD). The transition from controlled mechanical ventilation to assisted mechanical ventilation is a challenge that requires clinicians to balance overassistance and underassistance. While the effects of overassistance on the diaphragm are well known, the authors aimed to assess the impact of underassistance on diaphragm function and structure in a piglet model with preexisting VIDD (after long-term controlled mechanical ventilation) or without VIDD (short-term controlled mechanical ventilation). METHODS: Twenty-two Large White female piglets were anesthetized, ventilated, and separated into two groups: a VIDD group (n = 10) with long-term 72-h controlled mechanical ventilation, and a no-VIDD group (n = 12) with short-term 2-h controlled mechanical ventilation. After sedation reduction at the end of the controlled mechanical ventilation period, each piglet was switched to underassisted ventilation for 2 h. Diaphragm function (supramaximal diaphragm pressure-generating capacity assessed by negative tracheal pressure after transvenous phrenic nerve stimulation) and diaphragm structure (mini-invasive in vivo biopsies) were assessed before and after underassisted ventilation. RESULTS: In the VIDD group, supramaximal diaphragm pressure-generating capacity decreased by 22% from (mean ± SD) 69.9 ± 12.7 to 54.9 ± 19.7 cm H 2 O ( P = 0.04) after 72 h of controlled mechanical ventilation evidencing VIDD, then dropped by a further 29% from 54.9 ± 19.7 to 38.9 ± 15.5 cm H 2 O ( P < 0.01) after 2 h of underassisted ventilation. Diaphragm pressure-generating capacity remains stable from 55.3 ± 22.7 to 58.2 ± 24 cm H 2 O ( P = 0.24) in the no-VIDD group. Diaphragm structure showed that sarcomeric injuries increase from 13 ± 10% to 24 ± 19% ( P < 0.01) and lipid droplets decrease from 14 ± 8% to 11 ± 6% ( P = 0.03) of the total micrograph area after 2 h of underassisted ventilation in the VIDD group. Sarcomeric injuries and lipid droplets accounted, respectively, for 17 ± 16% and 2 ± 3% of the total micrograph area after underassisted ventilation in the no-VIDD group. CONCLUSIONS: In this porcine model, a short 2-h exposure of underassisted ventilation induces impairment of diaphragm function with damage to the diaphragm structure in intensive care unit condition with preexisting VIDD. CI - Copyright © 2025 American Society of Anesthesiologists. All Rights Reserved. FAU - Capdevila, Mathieu AU - Capdevila M AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Pensier, Joris AU - Pensier J AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - De Jong, Audrey AU - De Jong A AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Jung, Boris AU - Jung B AD - PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France; Department of Intensive Care Medicine, Lapeyronie Hospital, University Teaching Hospital of Montpellier, Montpellier, France. FAU - Beghin, July AU - Beghin J AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Laumon, Thomas AU - Laumon T AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Aarab, Yassir AU - Aarab Y AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Deffontis, Lucas AU - Deffontis L AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France. FAU - Sfara, Thomas AU - Sfara T AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France. FAU - Cuny, Ambre AU - Cuny A AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France. FAU - Carr, Julie AU - Carr J AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France. FAU - Molinari, Nicolas AU - Molinari N AD - Department of Statistics, Lapeyronie Hospital, University Teaching Hospital of Montpellier, UMR 729 MISTEA, Montpellier, France. FAU - Le Guennec, Jean-Yves AU - Le Guennec JY AD - PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Matecki, Stefan AU - Matecki S AD - PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Brochard, Laurent AU - Brochard L AD - Keenan Research Centre, St. Michael's Hospital, Unity Health Toronto, Toronto, Canada; Interdepartmental Division of Critical Care Medicine, University of Toronto, Toronto, Canada. FAU - Lacampagne, Alain AU - Lacampagne A AD - PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. FAU - Jaber, Samir AU - Jaber S AD - Department of Anesthesiology and Critical Care Medicine B, Saint-Eloi Hospital, University Teaching Hospital of Montpellier, Montpellier, France; PhyMedExp, Montpellier University, INSERM U1046, CNRS UMR9214, Montpellier, France. LA - eng PT - Journal Article DEP - 20250124 PL - United States TA - Anesthesiology JT - Anesthesiology JID - 1300217 SB - IM MH - Animals MH - *Diaphragm/pathology/physiopathology/physiology MH - Swine MH - *Respiration, Artificial/adverse effects/methods MH - Female MH - Disease Models, Animal EDAT- 2025/01/24 18:25 MHDA- 2025/04/08 13:56 CRDT- 2025/01/24 16:23 PHST- 2025/04/08 13:56 [medline] PHST- 2025/01/24 18:25 [pubmed] PHST- 2025/01/24 16:23 [entrez] AID - 00000542-202505000-00022 [pii] AID - 10.1097/ALN.0000000000005390 [doi] PST - ppublish SO - Anesthesiology. 2025 May 1;142(5):896-906. doi: 10.1097/ALN.0000000000005390. Epub 2025 Jan 24. PMID- 34139466 OWN - NLM STAT- MEDLINE DCOM- 20211206 LR - 20211214 IS - 1532-1983 (Electronic) IS - 0261-5614 (Linking) VI - 40 IP - 6 DP - 2021 Jun TI - Effect of age, stress and protein supply on plasma amino acids during continuous enteral nutrition; a pragmatic study in rats. PG - 3931-3939 LID - S0261-5614(21)00237-5 [pii] LID - 10.1016/j.clnu.2021.04.045 [doi] AB - BACKGROUND & AIMS: As life expectancy increases, an increasing older population may require surgery with perioperative nutritional management. While little is known about the combined effect of age and stress on amino acid metabolism during enteral nutrition, we hypothesized that blood amino acid bioavailability may be influenced not only by the characteristics of the ingested protein but also by intestinal ageing and splanchnic sequestration of amino acids. Plasma amino acid kinetics were thus evaluated in aged and adult rats receiving continuous enteral nutrition before and after standardized surgical stress. METHODS: Sixteen 5-month-old and sixteen 21-month-old male rats were used. After a gastrostomy, the insertion of a jugular vein catheter and a one-week recovery, the animals were enterally fed with commercially available formulas containing whole milk proteins or a whey hydrolysate for 24 h before (healthy state) and 18 h after a standardized laparotomy (surgical stress). Data were analyzed by 3-factor ANOVA. RESULTS: In all rats, enteral nutrition was associated with a marked increase in plasma alanine, threonine, lysine and proline (+50 to +150 μmol/L; p < 0.001), and a decrease in glycine (≈-80 μmol/L; p < 0.01). For most amino acids, their availability depended first on the amino acid composition of each protein and second on surgical stress. Aging was only associated with higher tyrosine and threonine availability (p < 0.001). There was only limited statistical interaction between age and surgical stress. CONCLUSION: In rats, plasma amino acid availability during continuous enteral nutrition is determined by the nature of the protein source and the occurrence of stress. The effects of aging on plasma amino acid availability seem very limited. Commonly used formulas therefore appear to be as suitable for elderly patients as for adult patients. CI - Copyright © 2021 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved. FAU - Ventura, G AU - Ventura G AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France. Electronic address: g.ventura@synadiet.org. FAU - Le Plenier, S AU - Le Plenier S AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France. Electronic address: servane.le-plenier@parisdescartes.fr. FAU - Neveux, N AU - Neveux N AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France; Clinical Chemistry Department, Cochin Hospital, AP-HP.Centre - Université de Paris, France. Electronic address: nathalie.neveux@aphp.fr. FAU - Sarfati, G AU - Sarfati G AD - Clinical Chemistry Department, Cochin Hospital, AP-HP.Centre - Université de Paris, France. Electronic address: gilles.sarfati-ext@aphp.fr. FAU - Cynober, L AU - Cynober L AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France; Clinical Chemistry Department, Cochin Hospital, AP-HP.Centre - Université de Paris, France. Electronic address: luc.cynober@parisdescartes.fr. FAU - Raynaud-Simon, A AU - Raynaud-Simon A AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France; Geriatry Unit, Bichat Hospital, Paris Nord Val de Seine University Hospitals, AP-HP, Paris, France. Electronic address: agathe.raynaud-simon@aphp.fr. FAU - De Bandt, J P AU - De Bandt JP AD - EA 4466 PRETRAM, Faculty of Pharmacy, Paris University, France; Clinical Chemistry Department, Cochin Hospital, AP-HP.Centre - Université de Paris, France. Electronic address: jean-pascal.de-bandt@parisdescartes.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210507 PL - England TA - Clin Nutr JT - Clinical nutrition (Edinburgh, Scotland) JID - 8309603 RN - 0 (Amino Acids) RN - 0 (Dietary Proteins) SB - IM MH - Age Factors MH - Amino Acids/*blood MH - Animals MH - Dietary Proteins MH - Disease Models, Animal MH - *Enteral Nutrition MH - Male MH - Malnutrition/*diet therapy/prevention & control MH - Rats MH - Rats, Sprague-Dawley MH - Stress, Physiological OTO - NOTNLM OT - Aging OT - Amino acids availability OT - Enteral nutrition COIS- Conflicts of interest LC received honorarium from Nestlé Health Science. EDAT- 2021/06/18 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/06/17 20:17 PHST- 2020/10/27 00:00 [received] PHST- 2021/03/18 00:00 [revised] PHST- 2021/04/28 00:00 [accepted] PHST- 2021/06/18 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/06/17 20:17 [entrez] AID - S0261-5614(21)00237-5 [pii] AID - 10.1016/j.clnu.2021.04.045 [doi] PST - ppublish SO - Clin Nutr. 2021 Jun;40(6):3931-3939. doi: 10.1016/j.clnu.2021.04.045. Epub 2021 May 7. PMID- 26055424 OWN - NLM STAT- MEDLINE DCOM- 20161114 LR - 20181113 IS - 1476-5578 (Electronic) IS - 1359-4184 (Linking) VI - 21 IP - 3 DP - 2016 Mar TI - GABA/Glutamate synaptic pathways targeted by integrative genomic and electrophysiological explorations distinguish autism from intellectual disability. PG - 411-8 LID - 10.1038/mp.2015.75 [doi] AB - Phenotypic and genetic heterogeneity is predominant in autism spectrum disorders (ASD), for which the molecular and pathophysiological bases are still unclear. Significant comorbidity and genetic overlap between ASD and other neurodevelopmental disorders are also well established. However, little is understood regarding the frequent observation of a wide phenotypic spectrum associated with deleterious mutations affecting a single gene even within multiplex families. We performed a clinical, neurophysiological (in vivo electroencephalography-auditory-evoked related potentials) and genetic (whole-exome sequencing) follow-up analysis of two families with known deleterious NLGN4X gene mutations (either truncating or overexpressing) present in individuals with ASD and/or with intellectual disability (ID). Complete phenotypic evaluation of the pedigrees in the ASD individuals showed common specific autistic behavioural features and neurophysiological patterns (abnormal MisMatch Negativity in response to auditory change) that were absent in healthy parents as well as in family members with isolated ID. Whole-exome sequencing in ASD patients from each family identified a second rare inherited genetic variant, affecting either the GLRB or the ANK3 genes encoding NLGN4X interacting proteins expressed in inhibitory or in excitatory synapses, respectively. The GRLB and ANK3 mutations were absent in relatives with ID as well as in control databases. In summary, our findings provide evidence of a double-hit genetic model focused on excitatory/inhibitory synapses in ASD, that is not found in isolated ID, associated with an atypical in vivo neurophysiological pattern linked to predictive coding. FAU - Bonnet-Brilhault, F AU - Bonnet-Brilhault F AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Centre Universitaire de pédopsychiatrie, Tours, France. FAU - Alirol, S AU - Alirol S AUID- ORCID: 0000-0003-4156-5959 AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. FAU - Blanc, R AU - Blanc R AD - Centre Hospitalier Régional Universitaire, Centre Universitaire de pédopsychiatrie, Tours, France. AD - Université Paris Descartes, Sorbonne Paris Cité, Institut de Psychologie, Laboratoire de Psychopathologie et Processus de Santé (EA 4057), Paris, France. FAU - Bazaud, S AU - Bazaud S AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. FAU - Marouillat, S AU - Marouillat S AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. FAU - Thépault, R-A AU - Thépault RA AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. FAU - Andres, C R AU - Andres CR AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Biochimie et de Biologie moléculaire, Tours, France. FAU - Lemonnier, É AU - Lemonnier É AD - Centre Hospitalier Universitaire, Centre Expert Autisme, Limoges, France. AD - Universite de Bretagne occidentale, Laboratoire de neuroscience, Brest, France. FAU - Barthélémy, C AU - Barthélémy C AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Centre Universitaire de pédopsychiatrie, Tours, France. FAU - Raynaud, M AU - Raynaud M AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Toutain, A AU - Toutain A AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Gomot, M AU - Gomot M AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. FAU - Laumonnier, F AU - Laumonnier F AD - INSERM, U930, Tours, France. AD - Université François-Rabelais, UMR « Imaging and Brain », Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. LA - eng GR - 1RC2MH089921-01/MH/NIMH NIH HHS/United States GR - 1RC2MH089929-01/MH/NIMH NIH HHS/United States GR - 1RC2MH090047-01/MH/NIMH NIH HHS/United States PT - Journal Article PT - Research Support, American Recovery and Reinvestment Act PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20150609 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (Cell Adhesion Molecules, Neuronal) RN - 0 (NLGN4X protein, human) RN - 3KX376GY7L (Glutamic Acid) RN - 56-12-2 (gamma-Aminobutyric Acid) SB - IM MH - Acoustic Stimulation MH - Autistic Disorder/*complications/*genetics MH - Cell Adhesion Molecules, Neuronal/*genetics MH - Child, Preschool MH - Electroencephalography MH - Evoked Potentials, Auditory/genetics/*physiology MH - Family Health MH - Female MH - Follow-Up Studies MH - Genetic Predisposition to Disease MH - *Genomics MH - Glutamic Acid MH - Humans MH - Intellectual Disability/*etiology MH - Male MH - Severity of Illness Index MH - Signal Transduction/genetics MH - gamma-Aminobutyric Acid EDAT- 2015/06/10 06:00 MHDA- 2016/11/15 06:00 CRDT- 2015/06/10 06:00 PHST- 2015/01/23 00:00 [received] PHST- 2015/03/19 00:00 [revised] PHST- 2015/04/27 00:00 [accepted] PHST- 2015/06/10 06:00 [entrez] PHST- 2015/06/10 06:00 [pubmed] PHST- 2016/11/15 06:00 [medline] AID - mp201575 [pii] AID - 10.1038/mp.2015.75 [doi] PST - ppublish SO - Mol Psychiatry. 2016 Mar;21(3):411-8. doi: 10.1038/mp.2015.75. Epub 2015 Jun 9. PMID- 40325973 OWN - NLM STAT- MEDLINE DCOM- 20250506 LR - 20250508 IS - 1099-1492 (Electronic) IS - 0952-3480 (Print) IS - 0952-3480 (Linking) VI - 38 IP - 6 DP - 2025 Jun TI - In Vivo Characterization of Magnetic Inclusions in the Subcortex From Nonexponential Transverse Relaxation Decay. PG - e70051 LID - 10.1002/nbm.70051 [doi] LID - e70051 AB - According to theoretical studies, MRI signal decay due to transverse relaxation in brain tissue with magnetic inclusions (e.g., blood vessels and iron-rich cells) is expected to follow a transition from Gaussian behaviour at short echo times to exponential behaviour at longer times. The decay parameters carry information about the inclusions (e.g., size and volume fraction) and provide unique insights into brain microstructure. However, gradient-echo decays typically only capture the long-time exponential behaviour. We provide experimental evidence of nonexponential transverse relaxation decay in human subcortical grey matter from in vivo MRI data acquired at 3 T, allowing the subsequent characterization of the magnetic inclusions. Gradient-echo data were collected with short interecho spacings, minimal echo time (1.25 ms) and novel acquisition strategies to mitigate motion and cardiac-induced effects. The data were fitted using exponential and nonexponential models that describe the impact of magnetic inclusions on the MRI signal. Nonexponential models provided superior fits. The strongest deviations from exponential were detected in the substantia nigra and globus pallidus. Numerical simulations of the signal decay from histological maps of iron concentration in the substantia nigra replicated the experimental data, highlighting that non-haem iron can be at the source of the nonexponential decay. To investigate the potential of nonexponential decays to characterize brain microstructure, we estimated the properties of the underlying inclusions using two analytical models. Under the static dephasing regime, the magnetic susceptibility and volume fractions of the inclusions ranged between 1.8-4 and 0.02-0.04 ppm, respectively. Alternatively, under the diffusion narrowing regime, the typical inclusion size was ~2.4 μm. Both simulations and experimental data suggest an intermediate regime with a non-negligible effect of water diffusion. Nonexponential transverse relaxation decay allows to characterize the spatial distribution of magnetic material within subcortical tissue with increased specificity, with potential applications for Parkinson's disease and other pathologies. CI - © 2025 The Author(s). NMR in Biomedicine published by John Wiley & Sons Ltd. FAU - Oliveira, Rita AU - Oliveira R AUID- ORCID: 0000-0002-7597-6919 AD - Laboratory for Research in Neuroimaging, Department of Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. FAU - Raynaud, Quentin AU - Raynaud Q AUID- ORCID: 0000-0002-6143-2873 AD - Laboratory for Research in Neuroimaging, Department of Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. FAU - Jelescu, Ileana AU - Jelescu I AUID- ORCID: 0000-0002-3664-0195 AD - Department of Radiology, Lausanne University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland. FAU - Kiselev, Valerij G AU - Kiselev VG AUID- ORCID: 0000-0002-5349-5399 AD - Division of Medical Physics, Department of Radiology, Faculty of Medicine, University of Freiburg, Freiburg, Germany. FAU - Kirilina, Evgeniya AU - Kirilina E AUID- ORCID: 0000-0003-1304-4363 AD - Department of Neurophysics, Max Planck Institute for Human Cognitive and Brain Sciences, Leipzig, Germany. FAU - Lutti, Antoine AU - Lutti A AUID- ORCID: 0000-0003-3281-5477 AD - Laboratory for Research in Neuroimaging, Department of Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland. LA - eng GR - 320030 184784/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ GR - PCEFP2_194260/Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung/ PT - Journal Article PL - England TA - NMR Biomed JT - NMR in biomedicine JID - 8915233 SB - IM MH - Humans MH - *Magnetic Resonance Imaging/methods MH - Male MH - *Brain MH - Female MH - Computer Simulation MH - Adult PMC - PMC12053162 OTO - NOTNLM OT - R 2 * $$ {R}_2^{\ast } $$ OT - brain iron OT - dopaminergic neurons OT - nonexponential decay OT - quantitative MRI OT - relaxometry OT - substantia nigra OT - transverse relaxation COIS- The authors declare no conflicts of interest. EDAT- 2025/05/06 06:30 MHDA- 2025/05/06 06:31 PMCR- 2025/05/06 CRDT- 2025/05/06 02:43 PHST- 2025/04/16 00:00 [revised] PHST- 2024/10/28 00:00 [received] PHST- 2025/04/17 00:00 [accepted] PHST- 2025/05/06 06:31 [medline] PHST- 2025/05/06 06:30 [pubmed] PHST- 2025/05/06 02:43 [entrez] PHST- 2025/05/06 00:00 [pmc-release] AID - NBM70051 [pii] AID - 10.1002/nbm.70051 [doi] PST - ppublish SO - NMR Biomed. 2025 Jun;38(6):e70051. doi: 10.1002/nbm.70051. PMID- 36805801 OWN - NLM STAT- MEDLINE DCOM- 20230308 LR - 20230308 IS - 2211-5684 (Electronic) IS - 2211-5684 (Linking) VI - 104 IP - 3 DP - 2023 Mar TI - Improving pain control during transarterial chemoembolization for hepatocellular carcinoma performed under local anesthesia with multimodal analgesia. PG - 123-132 LID - S2211-5684(22)00218-2 [pii] LID - 10.1016/j.diii.2022.10.013 [doi] AB - PURPOSE: The purpose of this study was to assess the performance of a reinforced analgesic protocol (RAP) on pain control in patients undergoing conventional trans-arterial chemoembolization (cTACE) for hepatocellular carcinoma (HCC). MATERIALS AND METHODS: Eighty-one consecutive patients (57 men, 24 women) with a mean age of 69 ± 10 (standard deviation) years (age range: 49-92 years) underwent 103 cTACEs. Standard antalgic protocol (50 mg hydroxyzine, 10 mg oxycodone, 8 mg ondansetron, and lidocaine for local anesthesia) was prospectively compared to a RAP (standard + 40 mg 2-h infusion nefopam and 50 mg tramadol). The individual pain risk was stratified based on age, the presence of cirrhosis and alcoholic liver disease, and patients were assigned to a low-risk group (standard protocol) or high-risk group (RAP). The primary endpoint was severe periprocedural abdominal pain (SAP), defined as a visual analog scale score ≥30/100. A predefined intermediate analysis was performed to monitor the benefit-risk of the RAP. Based on the intermediate analysis, all patients were treated with the RAP. RESULTS: The intermediate analysis performed after 52 cTACE showed that 2/17 (12%) high-risk patients (i.e., those receiving the RAP) experienced SAP compared to 15/35 (43%) low-risk patients (odds ratio [OR] = 0.18; 95% confidence interval [CI]: 0.02-0.98; P = 0.03). Analysis of all procedures showed that 12/67 (18%) patients in cTACE receiving the RAP experienced SAP compared to 15/36 (42%) patients who did not receive it (OR = 3.27; 95% CI: 1.32-8.14; P = 0.01). There were no statistical differences in adverse events, particularly for nausea, between groups. CONCLUSION: Reinforcing the analgesic protocol by combining non-opioid and opioid molecules reduces perioperative pain in patients undergoing cTACE for HCC. CI - Copyright © 2022 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved. FAU - Vanderbecq, Quentin AU - Vanderbecq Q AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Grégory, Jules AU - Grégory J AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France; Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation", CRI, 75018 Paris, France; FHU MOSAIC, 92110 Clichy, France. FAU - Dana, Jeremy AU - Dana J AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Dioguardi Burgio, Marco AU - Dioguardi Burgio M AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France; Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation", CRI, 75018 Paris, France. FAU - Garzelli, Lorenzo AU - Garzelli L AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France; Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation", CRI, 75018 Paris, France. FAU - Raynaud, Lucas AU - Raynaud L AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Frémy, Sébastien AU - Frémy S AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Paulatto, Luisa AU - Paulatto L AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Bouattour, Mohamed AU - Bouattour M AD - FHU MOSAIC, 92110 Clichy, France. FAU - Kavafyan-Lasserre, Juliette AU - Kavafyan-Lasserre J AD - Liver Cancer Unit, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. FAU - Vilgrain, Valérie AU - Vilgrain V AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France; Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation", CRI, 75018 Paris, France. FAU - Ronot, Maxime AU - Ronot M AD - Department of Radiology, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France; Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation", CRI, 75018 Paris, France; Department of Anesthesiology and Intensive Care, Hôpital Beaujon, AP-HP.Nord, 92110 Clichy, France. Electronic address: maxime.ronot@aphp.fr. LA - eng PT - Journal Article DEP - 20221118 PL - France TA - Diagn Interv Imaging JT - Diagnostic and interventional imaging JID - 101568499 SB - IM MH - Male MH - Humans MH - Female MH - Middle Aged MH - Aged MH - Aged, 80 and over MH - *Carcinoma, Hepatocellular/therapy/pathology MH - *Liver Neoplasms/therapy/pathology MH - Anesthesia, Local MH - *Chemoembolization, Therapeutic/methods MH - Abdominal Pain/etiology MH - *Analgesia MH - Treatment Outcome MH - Retrospective Studies OTO - NOTNLM OT - Analgesics opioid OT - Chemoembolization OT - Hepatocellular carcinoma OT - Liver cirrhosis OT - Non-narcotic analgesics OT - Pain measurement COIS- Declaration of Competing Interest The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article. EDAT- 2023/02/23 06:00 MHDA- 2023/03/09 06:00 CRDT- 2023/02/22 10:04 PHST- 2022/07/28 00:00 [received] PHST- 2022/10/21 00:00 [revised] PHST- 2022/10/25 00:00 [accepted] PHST- 2023/02/23 06:00 [pubmed] PHST- 2023/03/09 06:00 [medline] PHST- 2023/02/22 10:04 [entrez] AID - S2211-5684(22)00218-2 [pii] AID - 10.1016/j.diii.2022.10.013 [doi] PST - ppublish SO - Diagn Interv Imaging. 2023 Mar;104(3):123-132. doi: 10.1016/j.diii.2022.10.013. Epub 2022 Nov 18. PMID- 34850536 OWN - NLM STAT- MEDLINE DCOM- 20220411 LR - 20250530 IS - 1878-0261 (Electronic) IS - 1574-7891 (Print) IS - 1574-7891 (Linking) VI - 16 IP - 6 DP - 2022 Mar TI - FGF7-FGFR2 autocrine signaling increases growth and chemoresistance of fusion-positive rhabdomyosarcomas. PG - 1272-1289 LID - 10.1002/1878-0261.13145 [doi] AB - Rhabdomyosarcomas are aggressive pediatric soft-tissue sarcomas and include high-risk PAX3-FOXO1 fusion-gene-positive cases. Fibroblast growth factor receptor 4 (FGFR4) is known to contribute to rhabdomyosarcoma progression; here, we sought to investigate the involvement and potential for therapeutic targeting of other FGFRs in this disease. Cell-based screening of FGFR inhibitors with potential for clinical repurposing (NVP-BGJ398, nintedanib, dovitinib, and ponatinib) revealed greater sensitivity of fusion-gene-positive versus fusion-gene-negative rhabdomyosarcoma cell lines and was shown to be correlated with high expression of FGFR2 and its specific ligand, FGF7. Furthermore, patient samples exhibit higher mRNA levels of FGFR2 and FGF7 in fusion-gene-positive versus fusion-gene-negative rhabdomyosarcomas. Sustained intracellular mitogen-activated protein kinase (MAPK) activity and FGF7 secretion into culture media during serum starvation of PAX3-FOXO1 rhabdomyosarcoma cells together with decreased cell viability after genetic silencing of FGFR2 or FGF7 was in keeping with a novel FGF7-FGFR2 autocrine loop. FGFR inhibition with NVP-BGJ398 reduced viability and was synergistic with SN38, the active metabolite of irinotecan. In vivo, NVP-BGJ398 abrogated xenograft growth and warrants further investigation in combination with irinotecan as a therapeutic strategy for fusion-gene-positive rhabdomyosarcomas. CI - © 2021 The Authors. Molecular Oncology published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies. FAU - Milton, Christopher I AU - Milton CI AUID- ORCID: 0000-0002-1744-6071 AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Selfe, Joanna AU - Selfe J AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Aladowicz, Ewa AU - Aladowicz E AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Man, Stella Y K AU - Man SYK AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Bernauer, Carolina AU - Bernauer C AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Missiaglia, Edoardo AU - Missiaglia E AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Walters, Zoë S AU - Walters ZS AUID- ORCID: 0000-0002-1835-5868 AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Gatz, Susanne A AU - Gatz SA AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Kelsey, Anna AU - Kelsey A AD - Department of Paediatric Histopathology, Manchester University NHS Foundation Trust, Royal Manchester Children's Hospital, UK. FAU - Generali, Melanie AU - Generali M AUID- ORCID: 0000-0001-8396-3256 AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Box, Gary AU - Box G AD - Cancer Pharmacology and Stress Response Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Pharmacology and Stress Response Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - de Haven-Brandon, Alexis AU - de Haven-Brandon A AD - Cancer Pharmacology and Stress Response Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Galiwango, David AU - Galiwango D AD - Drug Metabolism and Pharmacokinetics Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Hayes, Angela AU - Hayes A AD - Drug Metabolism and Pharmacokinetics Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Clarke, Matthew AU - Clarke M AD - Glioma Team, Division of Molecular Pathology, The Institute of Cancer Research, London, UK. FAU - Izquierdo, Elisa AU - Izquierdo E AUID- ORCID: 0000-0002-3054-5832 AD - Glioma Team, Division of Molecular Pathology, The Institute of Cancer Research, London, UK. FAU - Gonzalez De Castro, David AU - Gonzalez De Castro D AD - Molecular Haematology, Division of Molecular Pathology, The Institute of Cancer Research, London, UK. FAU - Raynaud, Florence I AU - Raynaud FI AD - Drug Metabolism and Pharmacokinetics Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Kirkin, Vladimir AU - Kirkin V AD - Cancer Pharmacology and Stress Response Team, Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Shipley, Janet M AU - Shipley JM AUID- ORCID: 0000-0001-6748-8678 AD - Sarcoma Molecular Pathology Team, Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, UK. LA - eng GR - 22897/CRUK_/Cancer Research UK/United Kingdom GR - C5066/A1099/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211218 PL - United States TA - Mol Oncol JT - Molecular oncology JID - 101308230 RN - 0 (FGF7 protein, human) RN - 0 (Protein Kinase Inhibitors) RN - 126469-10-1 (Fibroblast Growth Factor 7) RN - 7673326042 (Irinotecan) RN - EC 2.7.10.1 (FGFR2 protein, human) RN - EC 2.7.10.1 (Receptor, Fibroblast Growth Factor, Type 2) SB - IM MH - *Autocrine Communication MH - Cell Line, Tumor MH - Child MH - Drug Resistance, Neoplasm MH - Fibroblast Growth Factor 7 MH - Humans MH - Irinotecan MH - Protein Kinase Inhibitors/pharmacology MH - Receptor, Fibroblast Growth Factor, Type 2 MH - *Rhabdomyosarcoma/drug therapy/genetics PMC - PMC8936514 OTO - NOTNLM OT - FGF7 OT - FGFR2 OT - NVP-BGJ398 OT - autocrine loop OT - fibroblast growth factor receptor OT - rhabdomyosarcoma COIS- The authors declare no conflict of interest. EDAT- 2021/12/02 06:00 MHDA- 2022/04/12 06:00 PMCR- 2022/03/01 CRDT- 2021/12/01 07:48 PHST- 2021/09/30 00:00 [revised] PHST- 2021/05/29 00:00 [received] PHST- 2021/11/29 00:00 [accepted] PHST- 2021/12/02 06:00 [pubmed] PHST- 2022/04/12 06:00 [medline] PHST- 2021/12/01 07:48 [entrez] PHST- 2022/03/01 00:00 [pmc-release] AID - MOL213145 [pii] AID - 10.1002/1878-0261.13145 [doi] PST - ppublish SO - Mol Oncol. 2022 Mar;16(6):1272-1289. doi: 10.1002/1878-0261.13145. Epub 2021 Dec 18. PMID- 34764173 OWN - NLM STAT- MEDLINE DCOM- 20211118 LR - 20260518 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 11 IP - 11 DP - 2021 Nov 11 TI - Social inequalities and dynamics of the early COVID-19 epidemic: a prospective cohort study in France. PG - e052888 LID - 10.1136/bmjopen-2021-052888 [doi] LID - e052888 AB - OBJECTIVE: Although social inequalities in COVID-19 mortality by race, gender and socioeconomic status are well documented, less is known about social disparities in infection rates and their shift over time. We aim to study the evolution of social disparities in infection at the early stage of the epidemic in France with regard to the policies implemented. DESIGN: Random population-based prospective cohort. SETTING: From May to June 2020 in France. PARTICIPANTS: Adults included in the Epidémiologie et Conditions de Vie cohort (n=77 588). MAIN OUTCOME MEASURES: Self-reported anosmia and/or ageusia in three categories: no symptom, during the first epidemic peak (in March 2020) or thereafter (during lockdown). RESULTS: In all, 2052 participants (1.53%) reported anosmia/ageusia. The social distribution of exposure factors (density of place of residence, overcrowded housing and working outside the home) was described. Multinomial regressions were used to identify changes in social variables (gender, class and race) associated with symptoms of anosmia/ageusia. Women were more likely to report symptoms during the peak and after. Racialised minorities accumulated more exposure risk factors than the mainstream population and were at higher risk of anosmia/ageusia during the peak and after. By contrast, senior executive professionals were the least exposed to the virus with the lower rate of working outside the home during lockdown. They were more affected than lower social classes at the peak of the epidemic, but this effect disappeared after the peak. CONCLUSION: The shift in the social profile of the epidemic was related to a shift in exposure factors under the implementation of a stringent stay-at-home order. Our study shows the importance to consider in a dynamic way the gender, socioeconomic and race direct and indirect effects of the COVID-19 pandemic, notably to implement policies that do not widen health inequalities. CI - © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Bajos, Nathalie AU - Bajos N AUID- ORCID: 0000-0001-8073-9056 AD - Institut de Recherche Interdisciplinaire sur les enjeux Sociaux - Sciences sociales, politique, santé, IRIS (UMR 8156 CNRS - EHESS - U997 INSERM), Aubervilliers, France nathalie.bajos@inserm.fr. FAU - Counil, Emilie AU - Counil E AUID- ORCID: 0000-0002-8527-4662 AD - INED, Paris, France. FAU - Franck, Jeanna-Eve AU - Franck JE AUID- ORCID: 0000-0002-3585-8643 AD - Institut de Recherche Interdisciplinaire sur les enjeux Sociaux - Sciences sociales, politique, santé, IRIS (UMR 8156 CNRS - EHESS - U997 INSERM), Aubervilliers, France. FAU - Jusot, Florence AU - Jusot F AD - Université Paris Dauphine, Paris, France. FAU - Pailhé, Ariane AU - Pailhé A AD - INED, Paris, France. FAU - Spire, Alexis AU - Spire A AD - Institut de Recherche Interdisciplinaire sur les enjeux Sociaux - Sciences sociales, politique, santé, IRIS (UMR 8156 CNRS - EHESS - U997 INSERM), Aubervilliers, France. FAU - Martin, Claude AU - Martin C AD - ARENES UMR 6051, CNRS, EHESP, Rennes, France. FAU - Lydie, Nathalie AU - Lydie N AD - Santé publique France, Saint-Maurice, France. FAU - Slama, Remy AU - Slama R AD - University Grenoble Alpes, INSERM, CNRS, Institute for Advanced Biosciences, Grenoble, France. FAU - Meyer, Laurence AU - Meyer L AD - INSERM, Université Paris-Saclay, AP-HP, Paris, France. FAU - Warszawski, Josiane AU - Warszawski J AD - INSERM, Université Paris-Saclay, AP-HP, Paris, France. CN - EpiCoV study group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211111 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - *COVID-19 MH - Cohort Studies MH - Communicable Disease Control MH - Female MH - France/epidemiology MH - Humans MH - Pandemics MH - Prospective Studies MH - SARS-CoV-2 MH - Socioeconomic Factors PMC - PMC8587531 OTO - NOTNLM OT - COVID-19 OT - public health OT - social medicine COIS- Competing interests: None declared. FIR - Bajos, Nathalie IR - Bajos N FIR - Warszawski, Josiane IR - Warszawski J FIR - Bagein, Guillaume IR - Bagein G FIR - Barlet, Muriel IR - Barlet M FIR - Beck, François IR - Beck F FIR - Counil, Emilie IR - Counil E FIR - FlorenceJusot, Aude Leduc IR - FlorenceJusot AL FIR - Lydie, Nathalie IR - Lydie N FIR - Martin, Claude IR - Martin C FIR - Meyer, Laurence IR - Meyer L FIR - Pailhé, Ariane IR - Pailhé A FIR - NicolasPaliod, Delphine Rahib IR - NicolasPaliod DR FIR - Raynaud, Philippe IR - Raynaud P FIR - Rouquette, Alexandra IR - Rouquette A FIR - Sicard, Patrick IR - Sicard P FIR - Slama, Rémy IR - Slama R FIR - Spire, Alexis IR - Spire A EDAT- 2021/11/13 06:00 MHDA- 2021/11/19 06:00 PMCR- 2021/11/10 CRDT- 2021/11/12 06:00 PHST- 2021/11/12 06:00 [entrez] PHST- 2021/11/13 06:00 [pubmed] PHST- 2021/11/19 06:00 [medline] PHST- 2021/11/10 00:00 [pmc-release] AID - bmjopen-2021-052888 [pii] AID - 10.1136/bmjopen-2021-052888 [doi] PST - epublish SO - BMJ Open. 2021 Nov 11;11(11):e052888. doi: 10.1136/bmjopen-2021-052888. PMID- 32282883 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210925 IS - 2473-9537 (Electronic) IS - 2473-9529 (Print) IS - 2473-9529 (Linking) VI - 4 IP - 7 DP - 2020 Apr 14 TI - Quizartinib-resistant FLT3-ITD acute myeloid leukemia cells are sensitive to the FLT3-Aurora kinase inhibitor CCT241736. PG - 1478-1491 LID - 10.1182/bloodadvances.2019000986 [doi] AB - Internal tandem duplication of FLT3 (FLT3-ITD) is one of the most common somatic mutations in acute myeloid leukemia (AML); it causes constitutive activation of FLT3 kinase and is associated with high relapse rates and poor survival. Small-molecule inhibition of FLT3 represents an attractive therapeutic strategy for this subtype of AML, although resistance from secondary FLT3 tyrosine kinase domain (FLT3-TKD) mutations is an emerging clinical problem. CCT241736 is an orally bioavailable, selective, and potent dual inhibitor of FLT3 and Aurora kinases. FLT3-ITD+ cells with secondary FLT3-TKD mutations have high in vitro relative resistance to the FLT3 inhibitors quizartinib and sorafenib, but not to CCT241736. The mechanism of action of CCT241736 results in significant in vivo efficacy, with inhibition of tumor growth observed in efficacy studies in FLT3-ITD and FLT3-ITD-TKD human tumor xenograft models. The efficacy of CCT241736 was also confirmed in primary samples from AML patients, including those with quizartinib-resistant disease, which induces apoptosis through inhibition of both FLT3 and Aurora kinases. The unique combination of CCT241736 properties based on robust potency, dual selectivity, and significant in vivo activity indicate that CCT241736 is a bona fide clinical drug candidate for FLT3-ITD and TKD AML patients with resistance to current drugs. CI - © 2020 by The American Society of Hematology. FAU - Moore, Andrew S AU - Moore AS AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. AD - Paediatric Oncology, Royal Marsden Hospital, Royal Marsden National Health Service Foundation Trust, Sutton, United Kingdom. FAU - Faisal, Amir AU - Faisal A AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Mak, Grace W Y AU - Mak GWY AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Miraki-Moud, Farideh AU - Miraki-Moud F AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Bavetsias, Vassilios AU - Bavetsias V AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Box, Gary AU - Box G AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Hallsworth, Albert AU - Hallsworth A AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - de Haven Brandon, Alexis AU - de Haven Brandon A AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Xavier, Cristina P R AU - Xavier CPR AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Stronge, Randal AU - Stronge R AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. AD - Haemato-Oncology Section, Royal Marsden Hospital, Sutton, United Kingdom; and. FAU - Pearson, Andrew D J AU - Pearson ADJ AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. AD - Paediatric Oncology, Royal Marsden Hospital, Royal Marsden National Health Service Foundation Trust, Sutton, United Kingdom. FAU - Blagg, Julian AU - Blagg J AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Chopra, Rajesh AU - Chopra R AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Eccles, Suzanne A AU - Eccles SA AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. FAU - Taussig, David C AU - Taussig DC AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. AD - Haemato-Oncology Section, Royal Marsden Hospital, Sutton, United Kingdom; and. FAU - Linardopoulos, Spiros AU - Linardopoulos S AD - Cancer Research UK Cancer Therapeutics Unit, Division of Cancer Therapeutics, The Institute of Cancer Research, Sutton, United Kingdom. AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. LA - eng GR - C309/A8274/CRUK_/Cancer Research UK/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - C1178/A10294/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Blood Adv JT - Blood advances JID - 101698425 RN - 0 (Benzothiazoles) RN - 0 (Phenylurea Compounds) RN - 0 (Protein Kinase Inhibitors) RN - 7LA4O6Q0D3 (quizartinib) RN - EC 2.7.10.1 (FLT3 protein, human) RN - EC 2.7.10.1 (fms-Like Tyrosine Kinase 3) RN - EC 2.7.11.1 (Aurora Kinases) SB - IM MH - Aurora Kinases MH - Benzothiazoles MH - Humans MH - *Leukemia, Myeloid, Acute/drug therapy/genetics MH - *Phenylurea Compounds/pharmacology/therapeutic use MH - Protein Kinase Inhibitors/pharmacology/therapeutic use MH - fms-Like Tyrosine Kinase 3/genetics PMC - PMC7160287 COIS- Conflict-of-interest disclosure: All authors are employees of The Institute of Cancer Research, which has a commercial interest in drug development programs (www.icr.ac.uk). Please note that all authors who are or have been employed by The Institute of Cancer Research are subject to a Rewards to Inventors Scheme which may reward contributors to a program that is subsequently licensed. J.B. is a stockholder in Azeria Therapeutics and NeoPhore Ltd. EDAT- 2020/04/14 06:00 MHDA- 2021/05/15 06:00 PMCR- 2020/04/13 CRDT- 2020/04/14 06:00 PHST- 2019/09/17 00:00 [received] PHST- 2020/02/11 00:00 [accepted] PHST- 2020/04/14 06:00 [entrez] PHST- 2020/04/14 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/04/13 00:00 [pmc-release] AID - S2473-9529(20)31409-9 [pii] AID - 2019/ADV2019000986 [pii] AID - 10.1182/bloodadvances.2019000986 [doi] PST - ppublish SO - Blood Adv. 2020 Apr 14;4(7):1478-1491. doi: 10.1182/bloodadvances.2019000986. PMID- 22529373 OWN - NLM STAT- MEDLINE DCOM- 20120727 LR - 20250529 IS - 1091-6490 (Electronic) IS - 0027-8424 (Print) IS - 0027-8424 (Linking) VI - 109 IP - 20 DP - 2012 May 15 TI - Dependence of Wilms tumor cells on signaling through insulin-like growth factor 1 in an orthotopic xenograft model targetable by specific receptor inhibition. PG - E1267-76 LID - 10.1073/pnas.1105034109 [doi] AB - We have previously demonstrated an increased DNA copy number and expression of IGF1R to be associated with poor outcome in Wilms tumors. We have now tested whether inhibiting this receptor may be a useful therapeutic strategy by using a panel of Wilms tumor cell lines. Both genetic and pharmacological targeting resulted in inhibition of downstream signaling through PI3 and MAP kinases, G(1) cell cycle arrest, and cell death, with drug efficacy dependent on the levels of phosphorylated IGF1R. These effects were further associated with specific gene expression signatures reflecting pathway inhibition, and conferred synergistic chemosensitisation to doxorubicin and topotecan. In the in vivo setting, s.c. xenografts of WiT49 cells resembled malignant rhabdoid tumors rather than Wilms tumors. Treatment with an IGF1R inhibitor (NVP-AEW541) showed no discernable antitumor activity and no downstream pathway inactivation. By contrast, Wilms tumor cells established orthotopically within the kidney were histologically accurate and exhibited significantly elevated insulin-like growth factor-mediated signaling, and growth was significantly reduced on treatment with NVP-AEW541 in parallel with signaling pathway ablation. As a result of the paracrine effects of enhanced IGF2 expression in Wilms tumor, this disease may be acutely dependent on signaling through the IGF1 receptor, and thus treatment strategies aimed at its inhibition may be useful in the clinic. Such efficacy may be missed if only standard ectopic models are considered as a result of an imperfect recapitulation of the specific tumor microenvironment. FAU - Bielen, Aleksandra AU - Bielen A AD - Department of Pediatric Oncology, Institute of Cancer Research, Sutton SM2 5NG, United Kingdom. FAU - Box, Gary AU - Box G FAU - Perryman, Lara AU - Perryman L FAU - Bjerke, Lynn AU - Bjerke L FAU - Popov, Sergey AU - Popov S FAU - Jamin, Yann AU - Jamin Y FAU - Jury, Alexa AU - Jury A FAU - Valenti, Melanie AU - Valenti M FAU - Brandon, Alexis de Haven AU - Brandon Ade H FAU - Martins, Vanessa AU - Martins V FAU - Romanet, Vincent AU - Romanet V FAU - Jeay, Sebastien AU - Jeay S FAU - Raynaud, Florence I AU - Raynaud FI FAU - Hofmann, Francesco AU - Hofmann F FAU - Robinson, Simon P AU - Robinson SP FAU - Eccles, Suzanne A AU - Eccles SA FAU - Jones, Chris AU - Jones C LA - eng GR - C1060/A10334/CRUK_/Cancer Research UK/United Kingdom GR - DH_/Department of Health/United Kingdom GR - MRC_/Medical Research Council/United Kingdom GR - C13468/A6718/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - C309/A8274/CRUK_/Cancer Research UK/United Kingdom GR - 11736/CRUK_/Cancer Research UK/United Kingdom PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20120423 PL - United States TA - Proc Natl Acad Sci U S A JT - Proceedings of the National Academy of Sciences of the United States of America JID - 7505876 RN - 0 (NVP-AEW541) RN - 0 (Pyrimidines) RN - 0 (Pyrroles) RN - 67763-96-6 (Insulin-Like Growth Factor I) RN - EC 2.7.10.1 (Receptor, IGF Type 1) SB - IM MH - Analysis of Variance MH - Animals MH - Cell Line, Tumor MH - Electrochemistry MH - Gene Expression Profiling MH - HEK293 Cells MH - Humans MH - Insulin-Like Growth Factor I/*metabolism MH - Kidney Neoplasms/*physiopathology MH - Magnetic Resonance Imaging MH - Mice MH - Paracrine Communication/physiology MH - Phosphorylation MH - Pyrimidines/pharmacology MH - Pyrroles/pharmacology MH - Receptor, IGF Type 1/antagonists & inhibitors MH - Signal Transduction/drug effects/*physiology MH - Transplantation, Heterologous MH - Wilms Tumor/*physiopathology PMC - PMC3356645 COIS- Conflict of interest statement: V.R., S.J., and F.H. are employees of Novartis Pharma. EDAT- 2012/04/25 06:00 MHDA- 2012/07/28 06:00 PMCR- 2012/11/15 CRDT- 2012/04/25 06:00 PHST- 2012/04/25 06:00 [entrez] PHST- 2012/04/25 06:00 [pubmed] PHST- 2012/07/28 06:00 [medline] PHST- 2012/11/15 00:00 [pmc-release] AID - 1105034109 [pii] AID - 201105034 [pii] AID - 10.1073/pnas.1105034109 [doi] PST - ppublish SO - Proc Natl Acad Sci U S A. 2012 May 15;109(20):E1267-76. doi: 10.1073/pnas.1105034109. Epub 2012 Apr 23. PMID- 32510913 OWN - NLM STAT- MEDLINE DCOM- 20210720 LR - 20210720 IS - 1534-6080 (Electronic) IS - 0041-1337 (Linking) VI - 105 IP - 3 DP - 2021 Mar 1 TI - Impact of Belatacept Conversion on Renal Function, Histology, and Gene Expression in Kidney Transplant Patients With Chronic Active Antibody-mediated Rejection. PG - 660-667 LID - 10.1097/TP.0000000000003278 [doi] AB - BACKGROUND: Here, we present our initial experience with a prospective protocol of belatacept conversion in patients with chronic active antibody-mediated rejection (caAMR) and a high degree of chronicity at the time of diagnosis. METHODS: We converted 19 patients (mean age, 45 ± 12 y) with biopsy-proven caAMR from tacrolimus to belatacept at a median of 44 months post-kidney transplant. RESULTS: At a median of 29 months (interquartile range, 16-46 mo) postconversion, death-censored graft and patient survivals were 89% and 95%, respectively. When compared to a 1:2 propensity-matched control cohort from the INSERM U970 registry maintained on calcineurin inhibitor, the belatacept group had progressive improvement (P = 0.02) in estimated glomerular filtration rate from a mean of 33.9 ± 10 at baseline to 37.8 ± 13 at 6 months and 38.5 ± 12 mL/min/1.73 m2 at 12 months postconversion, as compared to a steady decline noted in the controls (36.2 [baseline] → 33.1 [6 mo] → 32.7 mL/min/1.73 m2 [12 mo] of follow-up). A paired histologic comparison of preconversion and postconversion (performed at median 9.5 mo postconversion) biopsies showed no worsening in microvascular inflammation or chronicity. The paired tissue gene expression analysis showed improved mean total rejection score (0.68 ± 0.26-0.56 ± 0.33; P = 0.02) and a trend toward improved antibody-mediated rejection score (0.64 ± 0.34-0.56 ± 0.39; P = 0.06). CONCLUSIONS: Here, we report that in patients diagnosed with caAMR who were not subjected to intensive salvage immunosuppressive therapies, isolated belatacept conversion alone was associated with stabilization in renal function. These results are bolstered by molecular evidence of improved inflammation. CI - Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved. FAU - Kumar, Dhiren AU - Kumar D AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Raynaud, Marc AU - Raynaud M AD - Institut National de la Santé et de la Recherche Médicale, Unit 970 (INSERM U970), Paris, France. FAU - Chang, Jessica AU - Chang J AD - Alberta Transplant Applied Genomics Center, Edmonton, Canada. FAU - Reeve, Jeff AU - Reeve J AD - Alberta Transplant Applied Genomics Center, Edmonton, Canada. FAU - Yakubu, Idris AU - Yakubu I AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Kamal, Layla AU - Kamal L AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Levy, Marlon AU - Levy M AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Bhati, Chandra AU - Bhati C AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Kimball, Pamela AU - Kimball P AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - King, Anne AU - King A AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Massey, Davis AU - Massey D AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. FAU - Halloran, Philip AU - Halloran P AD - Alberta Transplant Applied Genomics Center, Edmonton, Canada. FAU - Gupta, Gaurav AU - Gupta G AD - Hume-Lee Transplant Center, Virginia Commonwealth University, Richmond, VA. LA - eng PT - Journal Article PL - United States TA - Transplantation JT - Transplantation JID - 0132144 RN - 0 (Immunosuppressive Agents) RN - 7D0YB67S97 (Abatacept) RN - WM0HAQ4WNM (Tacrolimus) SB - IM CIN - Transplantation. 2021 Mar 1;105(3):478-479. doi: 10.1097/TP.0000000000003279. PMID: 32732617 MH - Abatacept/*pharmacology MH - Biopsy MH - Chronic Disease MH - Drug Substitution MH - Female MH - Gene Expression/*drug effects MH - Glomerular Filtration Rate/*drug effects MH - Graft Rejection/diagnosis/*drug therapy/genetics MH - Graft Survival MH - Humans MH - Immunosuppressive Agents/pharmacology MH - Kidney/*pathology/physiopathology MH - Kidney Transplantation/*adverse effects MH - Male MH - Middle Aged MH - Prospective Studies MH - Tacrolimus/*pharmacology COIS- P.H. has received ownership interest from transcriptome Sciences. G.G. has received honoraria CareDx, Mallinckrodt, Thermo Fisher. The other authors declare no conflicts of interest. EDAT- 2020/06/09 06:00 MHDA- 2021/07/21 06:00 CRDT- 2020/06/09 06:00 PHST- 2020/06/09 06:00 [pubmed] PHST- 2021/07/21 06:00 [medline] PHST- 2020/06/09 06:00 [entrez] AID - 00007890-202103000-00029 [pii] AID - 10.1097/TP.0000000000003278 [doi] PST - ppublish SO - Transplantation. 2021 Mar 1;105(3):660-667. doi: 10.1097/TP.0000000000003278. PMID- 17179992 OWN - NLM STAT- MEDLINE DCOM- 20070222 LR - 20181201 IS - 0007-0920 (Print) IS - 1532-1827 (Electronic) IS - 0007-0920 (Linking) VI - 96 IP - 1 DP - 2007 Jan 15 TI - A phase I trial of the selective oral cyclin-dependent kinase inhibitor seliciclib (CYC202; R-Roscovitine), administered twice daily for 7 days every 21 days. PG - 29-37 AB - Seliciclib (CYC202; R-roscovitine) is the first selective, orally bioavailable inhibitor of cyclin-dependent kinases 1, 2, 7 and 9 to enter clinical trial. Preclinical studies showed antitumour activity in a broad range of human tumour xenografts. A phase I trial was performed with a 7-day b.i.d. p.o. schedule. Twenty-one patients (median age 62 years, range: 39-73 years) were treated with doses of 100, 200 and 800 b.i.d. Dose-limiting toxicities were seen at 800 mg b.i.d.; grade 3 fatigue, grade 3 skin rash, grade 3 hyponatraemia and grade 4 hypokalaemia. Other toxicities included reversible raised creatinine (grade 2), reversible grade 3 abnormal liver function and grade 2 emesis. An 800 mg portion was investigated further in 12 patients, three of whom had MAG3 renograms. One patient with a rapid increase in creatinine on day 3 had a reversible fall in renal perfusion, with full recovery by day 14, and no changes suggestive of renal tubular damage. Further dose escalation was precluded by hypokalaemia. Seliciclib reached peak plasma concentrations between 1 and 4 h and elimination half-life was 2-5 h. Inhibition of retinoblastoma protein phosphorylation was not demonstrated in peripheral blood mononuclear cells. No objective tumour responses were noted, but disease stabilisation was recorded in eight patients; this lasted for a total of six courses (18 weeks) in a patient with ovarian cancer. FAU - Benson, C AU - Benson C AD - Cancer Research UK Centre for Cancer Therapeutics, The Institute of Cancer Research, Sutton, Surrey, UK. FAU - White, J AU - White J FAU - De Bono, J AU - De Bono J FAU - O'Donnell, A AU - O'Donnell A FAU - Raynaud, F AU - Raynaud F FAU - Cruickshank, C AU - Cruickshank C FAU - McGrath, H AU - McGrath H FAU - Walton, M AU - Walton M FAU - Workman, P AU - Workman P FAU - Kaye, S AU - Kaye S FAU - Cassidy, J AU - Cassidy J FAU - Gianella-Borradori, A AU - Gianella-Borradori A FAU - Judson, I AU - Judson I FAU - Twelves, C AU - Twelves C LA - eng PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20061219 PL - England TA - Br J Cancer JT - British journal of cancer JID - 0370635 RN - 0 (Antineoplastic Agents) RN - 0 (Enzyme Inhibitors) RN - 0 (Purines) RN - 0ES1C2KQ94 (Roscovitine) RN - EC 2.7.11.22 (Cyclin-Dependent Kinases) SB - IM MH - Administration, Oral MH - Adult MH - Aged MH - Antineoplastic Agents/*administration & dosage/adverse effects MH - Cyclin-Dependent Kinases/antagonists & inhibitors MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Enzyme Inhibitors/*administration & dosage/adverse effects MH - Female MH - Humans MH - Hyperglycemia/chemically induced MH - Male MH - Maximum Tolerated Dose MH - Middle Aged MH - Neoplasm Staging MH - Neoplasms/*drug therapy MH - Purines/*administration & dosage/adverse effects MH - Roscovitine MH - Treatment Outcome PMC - PMC2360206 EDAT- 2006/12/21 09:00 MHDA- 2007/02/23 09:00 PMCR- 2008/01/15 CRDT- 2006/12/21 09:00 PHST- 2006/12/21 09:00 [pubmed] PHST- 2007/02/23 09:00 [medline] PHST- 2006/12/21 09:00 [entrez] PHST- 2008/01/15 00:00 [pmc-release] AID - 6603509 [pii] AID - 10.1038/sj.bjc.6603509 [doi] PST - ppublish SO - Br J Cancer. 2007 Jan 15;96(1):29-37. doi: 10.1038/sj.bjc.6603509. Epub 2006 Dec 19. PMID- 36115540 OWN - NLM STAT- MEDLINE DCOM- 20230404 LR - 20260518 IS - 1527-3296 (Electronic) IS - 0196-6553 (Print) IS - 0196-6553 (Linking) VI - 51 IP - 4 DP - 2023 Apr TI - Decrease of hospital- and community-acquired bloodstream infections due to Streptococcus pneumoniae and Streptococcus pyogenes during the first year of the COVID-19 pandemic: A time-series analysis in Paris region. PG - 475-477 LID - S0196-6553(22)00669-1 [pii] LID - 10.1016/j.ajic.2022.09.002 [doi] AB - The impact of the COVID-19 pandemic on bloodstream infections (BSIs) due to Streptococcus pneumoniae and Streptococcus pyogenes was assessed in 25 university hospitals of Paris. Monthly BSIs incidence rates that appeared stable in 2018 and 2019, decreased for the 2 pathogens during the 2 COVID-19 lockdown periods of 2020. Containment policies, including social distancing, masking and hand hygiene strengthening in both community and hospital settings are likely to reduce BSIs due to these pathogens. CI - Copyright © 2022 Association for Professionals in Infection Control and Epidemiology, Inc. Published by Elsevier Inc. All rights reserved. FAU - Amarsy, Rishma AU - Amarsy R AD - Groupe hospitalo-universitaire APHP.Nord-Université de Paris, Site Lariboisière et Fernand Widal, Infection Prevention and Control Team and CIMI-Paris, Inserm U1135, Sorbonne Université, Paris, France. Electronic address: rishma.amarsy@aphp.fr. FAU - Fournier, Sandra AU - Fournier S AD - Central Infection Control Team, Assistance Publique - hôpitaux de Paris, Paris, France. FAU - Trystram, David AU - Trystram D AD - Groupe hospitalo-universitaire APHP.Sorbonne Université, Site Pitié-Salpêtrière, Laboratoire de Bactériologie-Hygiène, Paris, France and Direction des Systèmes d'Information de l'Assistance Publique - hôpitaux de Paris, Paris, France. FAU - Monteil, Catherine AU - Monteil C AD - Central Infection Control Team, Assistance Publique - hôpitaux de Paris, Paris, France. FAU - Raynaud, Xavier AU - Raynaud X AD - Sorbonne Université, Université Paris-Cité, UPEC, IRD, CNRS, INRA, Institute of Ecology and Environmental Sciences, iEES Paris, Paris, France. FAU - Jarlier, Vincent AU - Jarlier V AD - Groupe hospitalo-universitaire APHP.Sorbonne Université, Site Pitié-Salpêtrière, Laboratoire de Bactériologie-Hygiène and CIMI-Paris, Inserm U1135, Sorbonne Université, Paris, France. FAU - Robert, Jérôme AU - Robert J AD - Groupe hospitalo-universitaire APHP.Sorbonne Université, Site Pitié-Salpêtrière, Laboratoire de Bactériologie-Hygiène and CIMI-Paris, Inserm U1135, Sorbonne Université, Paris, France. CN - la Collégiale de Bactériologie-Virologie-Hygiène de l'Assistance Publique-Hôpitaux de Paris LA - eng PT - Journal Article DEP - 20220915 PL - United States TA - Am J Infect Control JT - American journal of infection control JID - 8004854 SB - IM MH - Humans MH - Streptococcus pneumoniae MH - Streptococcus pyogenes MH - Pandemics MH - *Bacteremia/epidemiology MH - *COVID-19/epidemiology MH - Communicable Disease Control MH - *Community-Acquired Infections/epidemiology MH - Hospitals PMC - PMC9474397 OTO - NOTNLM OT - Bloodstream infection incidence OT - COVID-19 OT - Streptococcus pneumoniae OT - Streptococcus pyogenes FIR - Arlet, Guillaume IR - Arlet G FIR - Lefevre, LaurenceArmand IR - Lefevre L FIR - Aubry, Alexandra IR - Aubry A FIR - Belec, Laurent IR - Belec L FIR - Bercot, Béatrice IR - Bercot B FIR - Bonacorsi, Stéphane IR - Bonacorsi S FIR - Calvez, Vincent IR - Calvez V FIR - Cambau, Emmanuelle IR - Cambau E FIR - Carbonnelle, Etienne IR - Carbonnelle E FIR - Chevaliez, Stéphane IR - Chevaliez S FIR - Decousser, Jean-Winoc IR - Decousser JW FIR - Delaugerre, Constance IR - Delaugerre C FIR - Descamps, Diane IR - Descamps D FIR - Doucet-Populaire, Florence IR - Doucet-Populaire F FIR - Gaillard, Jean-Louis IR - Gaillard JL FIR - Garbarg-Chenon, Antoine IR - Garbarg-Chenon A FIR - Gault, Elyanne IR - Gault E FIR - Herrmann, Jean-Louis IR - Herrmann JL FIR - Jarlier, Vincent IR - Jarlier V FIR - Le Goff, Jérôme IR - Le Goff J FIR - Lucet, Jean-Christophe IR - Lucet JC FIR - Mainardi, Jean-Luc IR - Mainardi JL FIR - Marcellin, Anne-Geneviève IR - Marcellin AG FIR - Morand-Joubert, Laurence IR - Morand-Joubert L FIR - Nassif, Xavier IR - Nassif X FIR - Pawlotsky, Jean-Michel IR - Pawlotsky JM FIR - Robert, Jérôme IR - Robert J FIR - Afonso, Anne-Marie Roque IR - Afonso AR FIR - Rottman, Martin IR - Rottman M FIR - Rouzioux, Christine IR - Rouzioux C FIR - Rozenberg, Flore IR - Rozenberg F FIR - Simon, François IR - Simon F FIR - Veziris, Nicolas IR - Veziris N FIR - Skurnik, David IR - Skurnik D FIR - Zahar, Jean-Ralph IR - Zahar JR FIR - Barnaud, Guilene IR - Barnaud G FIR - Pomares, Typhaine Billard IR - Pomares TB FIR - Cuzon, Gaëlle IR - Cuzon G FIR - Decré, Dominique IR - Decré D FIR - Doloy, Alexandra IR - Doloy A FIR - Donay, Jean-Luc IR - Donay JL FIR - Drieux-Rouzet, Laurence IR - Drieux-Rouzet L FIR - Durand, Isabelle IR - Durand I FIR - Ferroni, Agnès IR - Ferroni A FIR - Fihman, Vincent IR - Fihman V FIR - Fortineau, Nicolas IR - Fortineau N FIR - Gomart, Camille IR - Gomart C FIR - Grall, Nathalie IR - Grall N FIR - Caruba, Christelle Guillet IR - Caruba CG FIR - Jaureguy, Françoise IR - Jaureguy F FIR - Lalande, Valérie IR - Lalande V FIR - Landraud, Luce IR - Landraud L FIR - Leflon, Véronique IR - Leflon V FIR - Mariani, Patricia IR - Mariani P FIR - Mihaila, Liliana IR - Mihaila L FIR - Moissenet, Didier IR - Moissenet D FIR - Noussair, Latifa IR - Noussair L FIR - Podglajen, Isabelle IR - Podglajen I FIR - Poilane, Isabelle IR - Poilane I FIR - Poupet, Hélène IR - Poupet H FIR - Rondinaud, Emilie IR - Rondinaud E FIR - Tardy, Valérie Sivadon IR - Tardy VS FIR - Trystram, David IR - Trystram D FIR - Verdet, Charlotte IR - Verdet C FIR - Vigier, Emmanuelle IR - Vigier E FIR - Billarant, Sophie Vimont IR - Billarant SV EDAT- 2022/09/18 06:00 MHDA- 2023/04/04 06:42 PMCR- 2022/09/15 CRDT- 2022/09/17 19:25 PHST- 2022/06/21 00:00 [received] PHST- 2022/08/31 00:00 [revised] PHST- 2022/09/01 00:00 [accepted] PHST- 2023/04/04 06:42 [medline] PHST- 2022/09/18 06:00 [pubmed] PHST- 2022/09/17 19:25 [entrez] PHST- 2022/09/15 00:00 [pmc-release] AID - S0196-6553(22)00669-1 [pii] AID - 10.1016/j.ajic.2022.09.002 [doi] PST - ppublish SO - Am J Infect Control. 2023 Apr;51(4):475-477. doi: 10.1016/j.ajic.2022.09.002. Epub 2022 Sep 15. PMID- 34527807 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250530 IS - 2366-3987 (Electronic) IS - 2366-3987 (Linking) VI - 4 IP - 2 DP - 2021 Feb TI - Peritumoral Delivery of Docetaxel-TIPS Microparticles for Prostate Cancer Adjuvant Therapy. PG - 2000179 LID - 10.1002/adtp.202000179 [doi] LID - 2000179 AB - Recurrence of prostate cancer after radical prostatectomy is a consequence of incomplete tumor resection. Systemic chemotherapy after surgery is associated with significant toxicity. Improved delivery methods for toxic drugs capable of targeting positive resection margins can reduce tumor recurrence and avoid their known toxicity. This study evaluates the effectiveness and toxicity of docetaxel (DTX) release from highly porous biodegradable microparticles intended for delivery into the tissue cavity created during radical prostatectomy to target residual tumor cells. The microparticles, composed of poly(dl-lactide-co-glycolide) (PLGA), are processed using thermally induced phase separation (TIPS) and loaded with DTX via antisolvent precipitation. Sustained drug release and effective toxicity in vitro are observed against PC3 human prostate cells. Peritumoral injection in a PC3 xenograft tumor model results in tumor growth inhibition equivalent to that achieved with intravenous delivery of DTX. Unlike intravenous delivery of DTX, implantation of DTX-TIPS microparticles is not accompanied by toxicity or elevated systemic levels of DTX in organ tissues or plasma. DTX-TIPS microparticles provide localized and sustained release of nontoxic therapeutic amounts of DTX. This may offer novel therapeutic strategies for improving management of patients with clinically localized high-risk disease requiring radical prostatectomy and other solid cancers at high risk of positive resection margins. CI - © 2020 The Authors. Advanced Therapeutics published by Wiley‐VCH GmbH. FAU - Paliashvili, Ketevan AU - Paliashvili K AD - Centre for Precision Healthcare UCL Division of Medicine University College London Gower Street London WC1E 6BT UK. FAU - Popov, Alexander AU - Popov A AD - Centre for Precision Healthcare UCL Division of Medicine University College London Gower Street London WC1E 6BT UK. FAU - Kalber, Tammy L AU - Kalber TL AD - Centre for Advanced Biomedical Imaging UCL Division of Medicine University College London Gower Street London WC1E 6BT UK. FAU - Patrick, P Stephen AU - Patrick PS AD - Centre for Advanced Biomedical Imaging UCL Division of Medicine University College London Gower Street London WC1E 6BT UK. FAU - Hayes, Angela AU - Hayes A AD - Drug Metabolism Pharmacokinetics and Metabolomics Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research Division of Cancer Therapeutics 15 Cotswold Road Sutton London SM2 5NG UK. FAU - Henley, Alan AU - Henley A AD - Drug Metabolism Pharmacokinetics and Metabolomics Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research Division of Cancer Therapeutics 15 Cotswold Road Sutton London SM2 5NG UK. FAU - Raynaud, Florence I AU - Raynaud FI AD - Drug Metabolism Pharmacokinetics and Metabolomics Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research Division of Cancer Therapeutics 15 Cotswold Road Sutton London SM2 5NG UK. FAU - Ahmed, Hashim U AU - Ahmed HU AD - Division of Surgery Department of Surgery and Cancer Imperial College London South Kensington Campus London SW7 2AZ UK. FAU - Day, Richard M AU - Day RM AUID- ORCID: 0000-0002-3124-2294 AD - Centre for Precision Healthcare UCL Division of Medicine University College London Gower Street London WC1E 6BT UK. LA - eng GR - 22105/CRUK_/Cancer Research UK/United Kingdom GR - 22897/CRUK_/Cancer Research UK/United Kingdom GR - FS/15/33/31608/BHF_/British Heart Foundation/United Kingdom GR - MR/R026416/1/MRC_/Medical Research Council/United Kingdom PT - Journal Article DEP - 20201019 PL - Germany TA - Adv Ther (Weinh) JT - Advanced therapeutics JID - 101724632 PMC - PMC8427470 OTO - NOTNLM OT - adjuvant chemotherapy OT - docetaxel OT - microparticles OT - positive resection margin OT - prostate cancer OT - radical prostatectomy COIS- A patent application has been submitted by UCL Business covering the combination of DTX and TIPS microspheres. EDAT- 2021/09/17 06:00 MHDA- 2021/09/17 06:01 PMCR- 2021/09/09 CRDT- 2021/09/16 07:19 PHST- 2020/08/11 00:00 [received] PHST- 2020/10/05 00:00 [revised] PHST- 2021/09/16 07:19 [entrez] PHST- 2021/09/17 06:00 [pubmed] PHST- 2021/09/17 06:01 [medline] PHST- 2021/09/09 00:00 [pmc-release] AID - ADTP202000179 [pii] AID - 10.1002/adtp.202000179 [doi] PST - ppublish SO - Adv Ther (Weinh). 2021 Feb;4(2):2000179. doi: 10.1002/adtp.202000179. Epub 2020 Oct 19. PMID- 33306128 OWN - NLM STAT- MEDLINE DCOM- 20211125 LR - 20220531 IS - 1460-2385 (Electronic) IS - 0931-0509 (Linking) VI - 36 IP - 10 DP - 2021 Sep 27 TI - Dynapaenia and sarcopaenia in chronic haemodialysis patients: do muscle weakness and atrophy similarly influence poor outcome? PG - 1908-1918 LID - 10.1093/ndt/gfaa353 [doi] AB - BACKGROUND: Sarcopaenia, defined as a decline in both muscle mass and function, has been recognized as a major determinant of poor outcome in haemodialysis (HD) patients. It is generally assumed that sarcopaenia is driven by muscle atrophy related to protein-energy wasting. However, dynapaenia, defined as weakness without atrophy, has been characterized by a different disease phenotype from sarcopaenia. The aim of this study was to compare the characteristics and prognosis of sarcopaenic and dynapaenic patients among a prospective cohort of chronic HD (CHD) patients. METHODS: Two hundred and thirty-two CHD patients were enrolled from January to July 2016 and then followed prospectively until December 2018. At inclusion, weakness and atrophy were, respectively, evaluated by maximal voluntary force (MVF) and creatinine index (CI). Sarcopaenia was defined as the association of weakness and atrophy (MVF and CI below the median) while dynapaenia was defined as weakness not related to atrophy (MVF below the median, and CI above the median). RESULTS: From a total of 187 prevalent CHD patients [65% of men, age 65.3 (49.7-82.0) years], 44 died during the follow-up period of 23.7 (12.4-34.9) months. Sarcopaenia and dynapaenia were observed in 33.7 and 16% of the patients, respectively. Compared with patients with sarcopaenia, patients with dynapaenia were younger and with a lower Charlson score. In contrast, mortality rate was similar in both groups (38 and 27%, respectively). After adjustment for age, sex, lean tissue index, serum albumin, high-sensitivity C-reactive protein (hs-CRP), haemoglobin (Hb), normalized protein catabolic rate (nPCR), dialysis vintage and Charlson score, only patients with dynapaenia were at increased risk of death [hazard ratio (HR) = 2.99, confidence interval 1.18-7.61; P = 0.02]. CONCLUSIONS: Screening for muscle functionality is highly warranted to identify patients with muscle functional impairment without muscle atrophy. In contrast to sarcopaenia, dynapaenia should appear as a phenotype induced by uraemic milieu, characterized by young patients with low Charlson score and poor prognosis outcome independently of serum albumin, hs-CRP, Hb, nPCR and dialysis vintage. CI - © The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. FAU - Souweine, Jean-Sébastien AU - Souweine JS AD - Department of Biochemistry, University Hospital of Montpellier, University of Montpellier, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. FAU - Pasquier, Grégoire AU - Pasquier G AD - Department of Biochemistry, University Hospital of Montpellier, University of Montpellier, Montpellier, France. FAU - Kuster, Nils AU - Kuster N AD - Department of Biochemistry, University Hospital of Montpellier, University of Montpellier, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. FAU - Rodriguez, Annie AU - Rodriguez A AD - AIDER, Montpellier, France. FAU - Patrier, Laure AU - Patrier L AD - AIDER, Montpellier, France. FAU - Morena, Marion AU - Morena M AD - Department of Biochemistry, University Hospital of Montpellier, University of Montpellier, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. FAU - Badia, Eric AU - Badia E AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. FAU - Chalabi, Lotfi AU - Chalabi L AD - AIDER, Montpellier, France. FAU - Raynal, Nathalie AU - Raynal N AD - AIDER, Montpellier, France. FAU - Ohresser, Isabelle AU - Ohresser I AD - AIDER, Montpellier, France. FAU - Hayot, Maurice AU - Hayot M AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, University of Montpellier, Montpellier, France. FAU - Mercier, Jacques AU - Mercier J AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, University of Montpellier, Montpellier, France. FAU - Quintrec, Moglie Le AU - Quintrec ML AD - Department of Nephrology, University Hospital of Montpellier, University of Montpellier, Montpellier, France. FAU - Gouzi, Fares AU - Gouzi F AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, University of Montpellier, Montpellier, France. FAU - Cristol, Jean-Paul AU - Cristol JP AD - Department of Biochemistry, University Hospital of Montpellier, University of Montpellier, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, CNRS, Montpellier, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Nephrol Dial Transplant JT - Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association JID - 8706402 RN - AYI8EX34EU (Creatinine) SB - IM MH - Aged MH - Creatinine MH - Humans MH - *Kidney Failure, Chronic/complications/therapy MH - Male MH - *Muscle Weakness/diagnosis/etiology MH - Muscular Atrophy/diagnosis/etiology MH - Prospective Studies MH - Renal Dialysis/adverse effects MH - *Sarcopenia/diagnosis/etiology OTO - NOTNLM OT - chronic haemodialysis OT - dynapaenia OT - muscle mass OT - muscle strength OT - sarcopaenia EDAT- 2020/12/12 06:00 MHDA- 2021/11/26 06:00 CRDT- 2020/12/11 12:08 PHST- 2020/07/09 00:00 [received] PHST- 2020/12/12 06:00 [pubmed] PHST- 2021/11/26 06:00 [medline] PHST- 2020/12/11 12:08 [entrez] AID - 6030952 [pii] AID - 10.1093/ndt/gfaa353 [doi] PST - ppublish SO - Nephrol Dial Transplant. 2021 Sep 27;36(10):1908-1918. doi: 10.1093/ndt/gfaa353. PMID- 20404006 OWN - NLM STAT- MEDLINE DCOM- 20100812 LR - 20211203 IS - 1557-3265 (Electronic) IS - 1078-0432 (Linking) VI - 16 IP - 11 DP - 2010 Jun 1 TI - Telomere maintenance and DNA damage responses during lung carcinogenesis. PG - 2979-88 LID - 10.1158/1078-0432.CCR-10-0142 [doi] AB - PURPOSE: Telomere shortening is an early event in bronchial carcinogenesis, preceding P53/Rb pathway inactivation and telomerase reactivation, and leading to DNA damage responses (DDR). As their inactivation in cancer increases genetic instability, our objective was to identify the chronology of telomere machinery critical events for malignant progression. EXPERIMENTAL DESIGN: We have evaluated telomere length by fluorescence in situ hybridization and analyzed DDR proteins p-CHK2, p-ATM, and p-H2AX, and telomeric maintenance proteins TRF1 and TRF2 expression by immunohistochemistry in normal bronchial/bronchiolar epithelium, and in 109 bronchial preneoplastic lesions, in comparison with 32 squamous invasive carcinoma (SCC), and in 27 atypical alveolar hyperplasia (AAH) in comparison with 6 adenocarcinoma in situ (AIS; formerly bronchiolo-alveolar carcinoma) and 24 invasive adenocarcinoma (ADC). RESULTS: Telomere length critically shortened at bronchial metaplasia stage to increase gradually from dysplasia to invasive SCC; in bronchiolo-alveolar lesions, telomere length decreased from normal to AIS and increased from stage I to II to stage III to IV ADC. Expression of TRF1 and TRF2 increased progressively from dysplasia to SCC and from AAH to invasive ADC. The expression of concomitant DDR proteins increased significantly from low- to high-grade dysplasia and from AAH to AIS and stage I to II ADC. P-CHK2 and p-H2AX expressions were highly correlated and both decreased, along with p-ATM, in SCC and advanced ADC. CONCLUSION: Telomere attrition occurs at the earliest stage of lung carcinogenesis as an initiating event, preceding TRF1 and TRF2 overexpression for telomere stabilization. In contrast, dismiss of DDR, through p-H2AX and p-CHK2 downregulation, represents a late progressing event associated with SCC and ADC progression. CI - Copyright 2010 AACR. FAU - Lantuejoul, Sylvie AU - Lantuejoul S AD - Department of Pathology, CHU A Michallon, Grenoble, France. SLantuejoul@chu-grenoble.fr FAU - Raynaud, Christophe AU - Raynaud C FAU - Salameire, Dimitri AU - Salameire D FAU - Gazzeri, Sylvie AU - Gazzeri S FAU - Moro-Sibilot, Denis AU - Moro-Sibilot D FAU - Soria, Jean-Charles AU - Soria JC FAU - Brambilla, Christian AU - Brambilla C FAU - Brambilla, Elizabeth AU - Brambilla E LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20100419 PL - United States TA - Clin Cancer Res JT - Clinical cancer research : an official journal of the American Association for Cancer Research JID - 9502500 RN - 0 (Cell Cycle Proteins) RN - 0 (DNA-Binding Proteins) RN - 0 (H2AX protein, human) RN - 0 (Histones) RN - 0 (TERF2 protein, human) RN - 0 (Telomeric Repeat Binding Protein 1) RN - 0 (Telomeric Repeat Binding Protein 2) RN - 0 (Tumor Suppressor Proteins) RN - EC 2.7.1.11 (Checkpoint Kinase 2) RN - EC 2.7.11.1 (ATM protein, human) RN - EC 2.7.11.1 (Ataxia Telangiectasia Mutated Proteins) RN - EC 2.7.11.1 (CHEK2 protein, human) RN - EC 2.7.11.1 (Protein Serine-Threonine Kinases) SB - IM MH - Adenocarcinoma/*genetics/metabolism MH - Adenocarcinoma, Bronchiolo-Alveolar/*genetics/metabolism MH - Ataxia Telangiectasia Mutated Proteins MH - Carcinoma, Squamous Cell/genetics MH - Cell Cycle Proteins/metabolism MH - Checkpoint Kinase 2 MH - *DNA Damage MH - DNA-Binding Proteins/metabolism MH - Disease Progression MH - Histones/metabolism MH - Hyperplasia/pathology MH - Immunohistochemistry MH - Lung/metabolism/pathology MH - Lung Neoplasms/*genetics/metabolism MH - Precancerous Conditions/*genetics/metabolism MH - Protein Serine-Threonine Kinases/metabolism MH - Telomere/*ultrastructure MH - Telomeric Repeat Binding Protein 1/metabolism MH - Telomeric Repeat Binding Protein 2/metabolism MH - Tumor Suppressor Proteins/metabolism EDAT- 2010/04/21 06:00 MHDA- 2010/08/13 06:00 CRDT- 2010/04/21 06:00 PHST- 2010/04/21 06:00 [entrez] PHST- 2010/04/21 06:00 [pubmed] PHST- 2010/08/13 06:00 [medline] AID - 1078-0432.CCR-10-0142 [pii] AID - 10.1158/1078-0432.CCR-10-0142 [doi] PST - ppublish SO - Clin Cancer Res. 2010 Jun 1;16(11):2979-88. doi: 10.1158/1078-0432.CCR-10-0142. Epub 2010 Apr 19. PMID- 30872323 OWN - NLM STAT- MEDLINE DCOM- 20191218 LR - 20200528 IS - 1533-3450 (Electronic) IS - 1046-6673 (Print) IS - 1046-6673 (Linking) VI - 30 IP - 4 DP - 2019 Apr TI - Archetype Analysis Identifies Distinct Profiles in Renal Transplant Recipients with Transplant Glomerulopathy Associated with Allograft Survival. PG - 625-639 LID - 10.1681/ASN.2018070777 [doi] AB - BACKGROUND: Transplant glomerulopathy, a common glomerular lesion observed after kidney transplant that is associated with poor prognosis, is not a specific entity but rather the end stage of overlapping disease pathways. Its heterogeneity has not been precisely characterized to date. METHODS: Our study included consecutive kidney transplant recipients from three centers in France and one in Canada who presented with a diagnosis of transplant glomerulopathy (Banff cg score ≥1 by light microscopy), on the basis of biopsies performed from January of 2004 through December of 2014. We used an unsupervised archetype analysis of comprehensive pathology findings and clinical, immunologic, and outcome data to identify distinct groups of patients. RESULTS: Among the 8207 post-transplant allograft biopsies performed during the inclusion period, we identified 552 biopsy samples (from 385 patients) with transplant glomerulopathy (incidence of 6.7%). The median time from transplant to transplant glomerulopathy diagnosis was 33.18 months. Kidney allograft survival rates at 3, 5, 7, and 10 years after diagnosis were 69.4%, 57.1%, 43.3%, and 25.5%, respectively. An unsupervised learning method integrating clinical, functional, immunologic, and histologic parameters revealed five transplant glomerulopathy archetypes characterized by distinct functional, immunologic, and histologic features and associated causes and distinct allograft survival profiles. These archetypes showed significant differences in allograft outcomes, with allograft survival rates 5 years after diagnosis ranging from 88% to 22%. Based on those results, we built an online application, which can be used in clinical practice on the basis of real patients. CONCLUSIONS: A probabilistic data-driven archetype analysis approach applied in a large, well defined multicenter cohort refines the diagnostic and prognostic features associated with cases of transplant glomerulopathy. Reducing heterogeneity among such cases can improve disease characterization, enable patient-specific risk stratification, and open new avenues for archetype-based treatment strategies and clinical trials optimization. CI - Copyright © 2019 by the American Society of Nephrology. FAU - Aubert, Olivier AU - Aubert O AUID- ORCID: 0000-0001-6284-3757 AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Paris Cite and Kidney Transplantation Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris Descartes University Sorbonne, Paris, France. FAU - Higgins, Sarah AU - Higgins S AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Nephrology, Sherbrooke University, Sherbrooke, Québec, Canada. FAU - Bouatou, Yassine AU - Bouatou Y AUID- ORCID: 0000-0003-3697-3886 AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Pathology, Academic Medical Center-University of Amsterdam, Amsterdam, The Netherlands. AD - Department of Nephrology and Organ Transplantation, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Yoo, Daniel AU - Yoo D AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. FAU - Raynaud, Marc AU - Raynaud M AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. FAU - Viglietti, Denis AU - Viglietti D AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Nephrology and Organ Transplantation, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Rabant, Marion AU - Rabant M AD - Department of Pathology, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Hidalgo, Luis AU - Hidalgo L AD - Department of Laboratory Medicine and Pathology and. FAU - Glotz, Denis AU - Glotz D AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Nephrology and Organ Transplantation, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Legendre, Christophe AU - Legendre C AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Paris Cite and Kidney Transplantation Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris Descartes University Sorbonne, Paris, France. FAU - Delahousse, Michel AU - Delahousse M AD - Department of Transplantation, Nephrology and Clinical Immunology, Hôpital Foch, Suresnes, France; and. FAU - Shah, Nikhil AU - Shah N AD - Division of Nephrology and Transplant Immunology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada. FAU - Sis, Banu AU - Sis B AD - Department of Laboratory Medicine and Pathology and. FAU - Campbell, Patricia AU - Campbell P AD - Department of Laboratory Medicine and Pathology and. AD - Division of Nephrology and Transplant Immunology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada. FAU - Mengel, Michael AU - Mengel M AD - Department of Laboratory Medicine and Pathology and. FAU - Jouven, Xavier AU - Jouven X AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Cardiology, Georges Pompidou European Hospital, Paris, France. FAU - Duong Van Huyen, Jean-Paul AU - Duong Van Huyen JP AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Pathology, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France. AD - Department of Nephrology and Organ Transplantation, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AUID- ORCID: 0000-0003-3388-7747 AD - Paris Translational Research Center for Organ Transplantation, Institut national de la santé et de la recherche médicale, Unité mixte de recherche-S970, Paris, France; alexandre.loupy@inserm.fr. AD - Paris Cite and Kidney Transplantation Department, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris Descartes University Sorbonne, Paris, France. LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20190314 PL - United States TA - J Am Soc Nephrol JT - Journal of the American Society of Nephrology : JASN JID - 9013836 RN - 9007-36-7 (Complement System Proteins) SB - IM MH - Adult MH - Allografts/*pathology MH - Biopsy MH - Complement System Proteins/metabolism MH - Female MH - Glomerulonephritis/etiology/*pathology/*physiopathology MH - Graft Rejection/*pathology/*physiopathology MH - Graft Survival MH - Humans MH - Kidney/pathology/physiopathology MH - Kidney Failure, Chronic/surgery MH - Kidney Transplantation/*adverse effects MH - Male MH - Middle Aged MH - Phenotype MH - Risk Assessment MH - Severity of Illness Index MH - Software MH - Survival Rate MH - Time Factors MH - Unsupervised Machine Learning MH - Young Adult PMC - PMC6442337 OTO - NOTNLM OT - Archetype OT - antibody-mediated rejection OT - donor-specific anti-HLA antibody OT - kidney transplantation OT - transplant glomerulopathy OT - transplant outcomes EDAT- 2019/03/16 06:00 MHDA- 2019/12/19 06:00 PMCR- 2020/04/01 CRDT- 2019/03/16 06:00 PHST- 2018/07/30 00:00 [received] PHST- 2019/01/24 00:00 [accepted] PHST- 2019/03/16 06:00 [pubmed] PHST- 2019/12/19 06:00 [medline] PHST- 2019/03/16 06:00 [entrez] PHST- 2020/04/01 00:00 [pmc-release] AID - ASN.2018070777 [pii] AID - 2018070777 [pii] AID - 10.1681/ASN.2018070777 [doi] PST - ppublish SO - J Am Soc Nephrol. 2019 Apr;30(4):625-639. doi: 10.1681/ASN.2018070777. Epub 2019 Mar 14. PMID- 36796836 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20260609 IS - 1399-3003 (Electronic) IS - 0903-1936 (Linking) VI - 61 IP - 5 DP - 2023 May TI - The French compassionate programme of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant. LID - 2202437 [pii] LID - 10.1183/13993003.02437-2022 [doi] AB - BACKGROUND: The European Medicines Agency has approved the cystic fibrosis transmembrane conductance regulator (CFTR) modulator combination elexacaftor/tezacaftor/ivacaftor (ETI) for people with cystic fibrosis (CF) carrying at least one F508del variant. The United States Food and Drug Administration (FDA) also approved ETI for people with CF carrying one of 177 rare variants. METHODS: An observational study was conducted to evaluate the effectiveness of ETI in people with CF with advanced lung disease who were not eligible for ETI in Europe. All patients with no F508del variant and advanced lung disease (defined as having a percent predicted forced expiratory volume (ppFEV(1)) <40% and/or being under evaluation for lung transplantation) and enrolled in the French compassionate programme initiated ETI at recommended doses. Effectiveness was evaluated by a centralised adjudication committee at 4-6 weeks in terms of clinical manifestations, sweat chloride concentration and ppFEV(1). RESULTS: Among the first 84 people with CF included in the programme, ETI was effective in 45 (54%), and 39 (46%) were considered to be nonresponders. Among the responders, 22 (49%) out of 45 carried a CFTR variant that is not currently approved by the FDA for ETI eligibility. Important clinical benefits, including suspending the indication for lung transplantation, a significant decrease in sweat chloride concentration by a median (interquartile range (IQR)) -30 (-14--43) mmol·L(-1) (n=42; p<0.0001) and an improvement in ppFEV(1) by +10.0 (6.0-20.5) percentage points (n=44; p<0.0001), were observed in those for whom treatment was effective. CONCLUSION: Clinical benefits were observed in a large subset of people with CF with advanced lung disease and CFTR variants not currently approved for ETI. CI - Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org. FAU - Burgel, Pierre-Régis AU - Burgel PR AUID- ORCID: 0000-0003-0903-9828 AD - Université Paris-Cité, Institut Cochin, Inserm U1016, Paris, France pierre-regis.burgel@aphp.fr. AD - Respiratory Medicine and Cystic Fibrosis National Reference Centre, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France. AD - ERN-Lung CF Network, Frankfurt, Germany. FAU - Sermet-Gaudelus, Isabelle AU - Sermet-Gaudelus I AUID- ORCID: 0000-0001-5537-9482 AD - ERN-Lung CF Network, Frankfurt, Germany. AD - Centre de de Référence Maladies Rares, Mucoviscidose et affections liées à CFTR, Pneumologie Pédiatrique et Allergologie, Hôpital Necker Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. AD - Université Paris-Cité, Institut Necker Enfants Malades, INSERM U1151, Paris, France. FAU - Durieu, Isabelle AU - Durieu I AD - ERN-Lung CF Network, Frankfurt, Germany. AD - Centre de Référence Adulte de la Mucoviscidose, Service de Médecine Interne, Hospices Civils de Lyon, Pierre Bénite, France. AD - Université de Lyon, Équipe d'Accueil Health Services and Performance Research (HESPER) 7425, Lyon, France. FAU - Kanaan, Reem AU - Kanaan R AD - Respiratory Medicine and Cystic Fibrosis National Reference Centre, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France. AD - ERN-Lung CF Network, Frankfurt, Germany. FAU - Macey, Julie AU - Macey J AD - Respiratory Medicine and Cystic Fibrosis Center, CHU de Bordeaux, Bordeaux, France. FAU - Grenet, Dominique AU - Grenet D AD - CRCM -‌ Centre de Transplantation Pulmonaire, Service de Pneumologie, Hôpital Foch, Suresnes, France. FAU - Porzio, Michele AU - Porzio M AD - Department of Respiratory Medicine and Cystic Fibrosis Centre, Federation of Translational Medicine of Strasbourg (FMTS), University Hospitals, Strasbourg, France. FAU - Coolen-Allou, Nathalie AU - Coolen-Allou N AD - Centre Hospitalier Universitaire Félix Guyon, Saint Denis, La Réunion, France. FAU - Chiron, Raphael AU - Chiron R AD - Cystic Fibrosis Center, Hôpital Arnaud de Villeneuve, Centre Hospitalier Universitaire de Montpellier, Montpellier, France. FAU - Marguet, Christophe AU - Marguet C AD - Pediatric Respiratory Disease and Cystic Fibrosis Centre, Hospital, UNIROUEN, Inserm EA 2656, Rouen University Hospital, Normandie Univ, Rouen, France. FAU - Douvry, Benoit AU - Douvry B AD - Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, Créteil, France. FAU - Dufeu, Nadine AU - Dufeu N AD - Department of Respiratory Medicine and Lung Transplantation, Aix Marseille Univ, APHM, Hôpital Nord, Marseille, France. FAU - Danner-Boucher, Isabelle AU - Danner-Boucher I AD - Service de Pneumologie, L'Institut Du Thorax, CHU Nantes, Nantes, France. FAU - Foucaud, Pierre AU - Foucaud P AD - Association Vaincre la Mucoviscidose, Paris, France. FAU - Lemonnier, Lydie AU - Lemonnier L AD - Association Vaincre la Mucoviscidose, Paris, France. FAU - Girodon, Emmanuelle AU - Girodon E AUID- ORCID: 0000-0001-7913-4682 AD - APHP Centre-Université de Paris Cité, Service de Médecine Génomique des Maladies de Système et d'Organe, Hôpital Cochin, Paris, France. FAU - Da Silva, Jennifer AU - Da Silva J AD - Respiratory Medicine and Cystic Fibrosis National Reference Centre, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France. AD - ERN-Lung CF Network, Frankfurt, Germany. FAU - Martin, Clémence AU - Martin C AUID- ORCID: 0000-0003-4385-5888 AD - Université Paris-Cité, Institut Cochin, Inserm U1016, Paris, France. AD - Respiratory Medicine and Cystic Fibrosis National Reference Centre, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France. AD - ERN-Lung CF Network, Frankfurt, Germany. CN - French CF Reference Network study group FAU - Andrejak, Claire AU - Andrejak C FAU - Priou, Pascaline AU - Priou P FAU - Richaud-Thiriez, Bénédicte AU - Richaud-Thiriez B FAU - Macey, Julie AU - Macey J FAU - Montcouquiol, Sylvie AU - Montcouquiol S FAU - Douvry, Benoit AU - Douvry B FAU - Remus, Natacha AU - Remus N FAU - Fanton, Annlyse AU - Fanton A FAU - Coolen-Allou, Nathalie AU - Coolen-Allou N FAU - Gachelin, Elsa AU - Gachelin E FAU - Vuillard, Constance AU - Vuillard C FAU - Audousset, Camille AU - Audousset C FAU - Duthoit, Louise AU - Duthoit L FAU - Bellet-Fraysse, Elisabeth AU - Bellet-Fraysse E FAU - Languepin, Jeanne AU - Languepin J FAU - Durieu, Isabelle AU - Durieu I FAU - Nove-Josserand, Raphaele AU - Nove-Josserand R FAU - Ohlmann, Camille AU - Ohlmann C FAU - Raynaud, Quitterie AU - Raynaud Q FAU - Dufeu, Nadine AU - Dufeu N FAU - Chiron, Raphael AU - Chiron R FAU - Billon, Yves AU - Billon Y FAU - Danner-Boucher, Isabelle AU - Danner-Boucher I FAU - Tissot, Adrien AU - Tissot A FAU - Leroy, Sylvie AU - Leroy S FAU - Aubourg, Frédérique AU - Aubourg F FAU - Burnet, Espérie AU - Burnet E FAU - Burgel, Pierre-Régis AU - Burgel PR FAU - Carlier, Nicolas AU - Carlier N FAU - Da Silva, Jennifer AU - Da Silva J FAU - Fajac, Isabelle AU - Fajac I FAU - Girodon, Emmanuelle AU - Girodon E FAU - Kanaan, Reem AU - Kanaan R FAU - Honoré, Isabelle AU - Honoré I FAU - Martin, Clémence AU - Martin C FAU - Le Bourgeois, Muriel AU - Le Bourgeois M FAU - Sermet-Gaudelus, Isabelle AU - Sermet-Gaudelus I FAU - Le Clainche-Viala, Laurence AU - Le Clainche-Viala L FAU - Corvol, Harriet AU - Corvol H FAU - Dehillotte, Clémence AU - Dehillotte C FAU - Foucaud, Pierre AU - Foucaud P FAU - Lemonnier, Lydie AU - Lemonnier L FAU - Brinchault, Graziella AU - Brinchault G FAU - Ravoninjatovo, Bruno AU - Ravoninjatovo B FAU - Le Bihan, Jean AU - Le Bihan J FAU - Ramel, Sophie AU - Ramel S FAU - Marguet, Christophe AU - Marguet C FAU - Porzio, Michele AU - Porzio M FAU - Weiss, Laurence AU - Weiss L FAU - Grenet, Dominique AU - Grenet D FAU - de Miranda, Sandra AU - de Miranda S FAU - Cosson, Laure AU - Cosson L FAU - Mankikian, Julie AU - Mankikian J FAU - Pouradier, Delphine AU - Pouradier D LA - eng PT - Journal Article DEP - 20230505 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 SB - IM CIN - Eur Respir J. 2024 Feb 29;63(2):2400233. doi: 10.1183/13993003.00233-2024. PMID: 38423591 CIN - Eur Respir J. 2024 Feb 29;63(2):2301392. doi: 10.1183/13993003.01392-2023. PMID: 38423592 COIS- Conflict of interest: P-R. Burgel reports support for the present work from Vaincre la Mucoviscidose. P-R. Burgel reports grants from Boehringer Ingelheim, GSK, Vertex; consulting fees and lecture honoraria from Boehringer Ingelheim, GSK, AstraZeneca, Vertex, Chiesi, Pfizer, Novartis, Zambon, Insmed; outside the submitted work. I. Sermet-Gaudelus reports support from the present manuscript from French CF association for primary nasal cell generation and Institut Necker Enfants Malades. I. Sermet-Gaudelus reports grants from Institut Necker Enfants Malades; is a member of scientific advisory board and received an innovation award from Vertex; travel support from North American Cystic Fibrosis Conference, Société Francais de Mucoviscidose; outside the submitted work. C. Marguet reports payment for expert testimony from Vertex; travel support from Sanofi; advisory board participation with Vertex, Zambon, Viatris, Sanofi; leadership role with French Paediatric Pulmonology and Allergy Society; outside the submitted work. B. Douvry reports grants and payment for expert testimony from Vertex, outside the submitted work. C. Martin reports lecture honoraria from AstraZeneca, Chiesi, Zambon; travel support from Zambon, Sanofi; advisory board membership from Vertex, Zambon, GSK; outside the submitted work. All other authors have nothing to disclose. EDAT- 2023/02/17 06:00 MHDA- 2023/02/17 06:01 CRDT- 2023/02/16 20:33 PHST- 2022/12/20 00:00 [received] PHST- 2023/02/09 00:00 [accepted] PHST- 2023/02/17 06:01 [medline] PHST- 2023/02/17 06:00 [pubmed] PHST- 2023/02/16 20:33 [entrez] AID - 13993003.02437-2022 [pii] AID - 10.1183/13993003.02437-2022 [doi] PST - epublish SO - Eur Respir J. 2023 May 5;61(5):2202437. doi: 10.1183/13993003.02437-2022. Print 2023 May. PMID- 34782448 OWN - NLM STAT- MEDLINE DCOM- 20220502 LR - 20260518 IS - 1499-2752 (Electronic) IS - 0315-162X (Linking) VI - 49 IP - 2 DP - 2022 Feb TI - Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology. PG - 176-185 LID - 10.3899/jrheum.210794 [doi] AB - OBJECTIVE: There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics. METHODS: A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared. RESULTS: The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. CONCLUSION: Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex. CI - © 2022 by the Journal of Rheumatology. FAU - De Angelis, Rossella AU - De Angelis R AUID- ORCID: 0000-0001-5169-3511 AD - R. De Angelis, MD, A.M. Risa, MD, Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona; r.deangelis@staff.univpm.it. FAU - Giuggioli, Dilia AU - Giuggioli D AD - D. Giuggioli, MD, F. Lumetti, MD, A. Spinella, MD, C. Ferri, MD, Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena. FAU - Bajocchi, Gianluigi AU - Bajocchi G AUID- ORCID: 0000-0002-6430-800X AD - G. Bajocchi, MD, L. Magnani, MD, C. Salvarani, MD, Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia. FAU - Dagna, Lorenzo AU - Dagna L AUID- ORCID: 0000-0002-7428-315X AD - L. Dagna, MD, C. Campochiaro, MD, G. De Luca, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan. FAU - Zanframundo, Giovanni AU - Zanframundo G AUID- ORCID: 0000-0001-5042-1282 AD - G. Zanframundo, MD, V. Codullo, MD, Department of Rheumatology, Policlinico San Matteo, Pavia. FAU - Foti, Rosario AU - Foti R AD - R. Foti, MD, E. Visalli, MD, AOU Policlinico Vittorio Emanuele, Catania. FAU - Cacciapaglia, Fabio AU - Cacciapaglia F AUID- ORCID: 0000-0001-7479-4462 AD - F. Cacciapaglia, MD, F. Iannone, MD, Rheumatology Unit, Department of Emergency Surgery and Organ Transplantations, University of Bari, Bari. FAU - Cuomo, Giovanna AU - Cuomo G AUID- ORCID: 0000-0002-4292-3589 AD - G. Cuomo, MD, F. Masini, MD, Luigi Vanvitelli University, Naples. FAU - Ariani, Alarico AU - Ariani A AUID- ORCID: 0000-0003-1428-6102 AD - A. Ariani, MD, Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma. FAU - Rosato, Edoardo AU - Rosato E AUID- ORCID: 0000-0002-7417-8093 AD - E. Rosato, MD, A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Guiducci, Serena AU - Guiducci S AUID- ORCID: 0000-0003-2722-6475 AD - S. Guiducci, MD, S. Bellando-Randone, MD, Department of Rheumatology, University of Florence, Florence. FAU - Girelli, Francesco AU - Girelli F AD - F. Girelli, MD, Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni-L Pierantoni, Forlì. FAU - Riccieri, Valeria AU - Riccieri V AD - V. Riccieri, MD, G. Pellegrino, MD, Department of Rheumatology, Sapienza University of Rome, Rome. FAU - Zanatta, Elisabetta AU - Zanatta E AD - E. Zanatta, MD, A. Doria, MD, Department of Rheumatology, University of Padua, Padova. FAU - Bosello, Silvia AU - Bosello S AUID- ORCID: 0000-0002-4837-447X AD - S. Bosello, MD, Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart, Rome. FAU - Cavazzana, Ilaria AU - Cavazzana I AUID- ORCID: 0000-0002-2757-7120 AD - I. Cavazzana, MD, F. Dall'Ara, MD, M.G. Lazzaroni, MD, Department of Rheumatology, Spedali Civili di Brescia, Brescia. FAU - Ingegnoli, Francesca AU - Ingegnoli F AUID- ORCID: 0000-0002-6727-1273 AD - F. Ingegnoli, MD, Division of Clinical Rheumatology, ASST Pini, Dept. of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan. FAU - Santis, Maria De AU - Santis M AUID- ORCID: 0000-0002-3196-1336 AD - M. De Santis, MD, E.Generali, MD, Humanitas Clinical and Research Center IRCCS, Milan. FAU - Murdaca, Giuseppe AU - Murdaca G AD - G. Murdaca, MD, S. Martino Hospital-University of Genoa, Genoa. FAU - Abignano, Giuseppina AU - Abignano G AUID- ORCID: 0000-0002-1479-0133 AD - G. Abignano, MD, G. Mennillo, MD, S. D'Angelo, MD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Romeo, Nicoletta AU - Romeo N AD - N. Romeo, MD, S. Croce e Carle Hospital, Cuneo. FAU - Della Rossa, Alessandra AU - Della Rossa A AD - A. Della Rossa, MD, S. Barsotti, MD, Department of Rheumatology, University of Pisa, Pisa. FAU - Caminiti, Maurizio AU - Caminiti M AD - M. Caminiti, MD, G. Pagano Mariano, MD, F. Calabrese, MD, Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria. FAU - Iuliano, Annamaria AU - Iuliano A AD - A. Iuliano, MD, G.D. Sebastiani, MD, Rheumatology Unit, San Camillo-Forlanini Hospital, Rome. FAU - Ciano, Giovanni AU - Ciano G AD - G. Ciano, MD, Local Health Department, Avellino. FAU - Beretta, Lorenzo AU - Beretta L AD - L. Beretta, MD, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan. FAU - Bagnato, Gianluca AU - Bagnato G AUID- ORCID: 0000-0002-7594-8520 AD - G. Bagnato, MD, Department of Clinical and Experimental Medicine, University of Messina, Messina. FAU - Lubrano, Ennio AU - Lubrano E AUID- ORCID: 0000-0001-6189-5328 AD - E. Lubrano, MD, Department of Rheumatology, University of Molise, Campobasso. FAU - De Andres, Ilenia AU - De Andres I AD - I. De Andres, MD, Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione Garibaldi, Catania. FAU - Giollo, Alessandro AU - Giollo A AUID- ORCID: 0000-0001-9355-7673 AD - A. Giollo, MD, Rheumatology Section, Department of Medicine, University of Verona, Verona. FAU - Saracco, Marta AU - Saracco M AD - M. Saracco, MD, Rheumatology Unit, Mauriziano-Umberto I Hospital, Turin. FAU - Agnes, Cecilia AU - Agnes C AD - C. Agnes, MD, San Lorenzo Hospital. FAU - Lumetti, Federica AU - Lumetti F AUID- ORCID: 0000-0003-4671-7982 AD - D. Giuggioli, MD, F. Lumetti, MD, A. Spinella, MD, C. Ferri, MD, Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena. FAU - Spinella, Amelia AU - Spinella A AD - D. Giuggioli, MD, F. Lumetti, MD, A. Spinella, MD, C. Ferri, MD, Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena. FAU - Magnani, Luca AU - Magnani L AUID- ORCID: 0000-0002-7534-0785 AD - G. Bajocchi, MD, L. Magnani, MD, C. Salvarani, MD, Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia. FAU - Campochiaro, Corrado AU - Campochiaro C AUID- ORCID: 0000-0001-6806-3794 AD - L. Dagna, MD, C. Campochiaro, MD, G. De Luca, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan. FAU - De Luca, Giacomo AU - De Luca G AUID- ORCID: 0000-0002-5306-7714 AD - L. Dagna, MD, C. Campochiaro, MD, G. De Luca, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan. FAU - Codullo, Veronica AU - Codullo V AUID- ORCID: 0000-0003-2557-8514 AD - G. Zanframundo, MD, V. Codullo, MD, Department of Rheumatology, Policlinico San Matteo, Pavia. FAU - Visalli, Elisa AU - Visalli E AUID- ORCID: 0000-0002-8213-5528 AD - R. Foti, MD, E. Visalli, MD, AOU Policlinico Vittorio Emanuele, Catania. FAU - Masini, Francesco AU - Masini F AD - G. Cuomo, MD, F. Masini, MD, Luigi Vanvitelli University, Naples. FAU - Gigante, Antonietta AU - Gigante A AD - E. Rosato, MD, A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Bellando-Randone, Silvia AU - Bellando-Randone S AD - S. Guiducci, MD, S. Bellando-Randone, MD, Department of Rheumatology, University of Florence, Florence. FAU - Pellegrino, Greta AU - Pellegrino G AD - V. Riccieri, MD, G. Pellegrino, MD, Department of Rheumatology, Sapienza University of Rome, Rome. FAU - Pigatto, Erika AU - Pigatto E AD - E. Pigatto, MD, F. Cozzi, MD, Department of Medicine, Villa Salus Hospital, Venice. FAU - Dall'Ara, Francesca AU - Dall'Ara F AD - I. Cavazzana, MD, F. Dall'Ara, MD, M.G. Lazzaroni, MD, Department of Rheumatology, Spedali Civili di Brescia, Brescia. FAU - Lazzaroni, Maria Grazia AU - Lazzaroni MG AD - I. Cavazzana, MD, F. Dall'Ara, MD, M.G. Lazzaroni, MD, Department of Rheumatology, Spedali Civili di Brescia, Brescia. FAU - Generali, Elena AU - Generali E AUID- ORCID: 0000-0002-5898-1767 AD - M. De Santis, MD, E.Generali, MD, Humanitas Clinical and Research Center IRCCS, Milan. FAU - Mennillo, Gianna AU - Mennillo G AD - G. Abignano, MD, G. Mennillo, MD, S. D'Angelo, MD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Barsotti, Simone AU - Barsotti S AUID- ORCID: 0000-0002-4864-4505 AD - A. Della Rossa, MD, S. Barsotti, MD, Department of Rheumatology, University of Pisa, Pisa. FAU - Mariano, Giuseppa Pagano AU - Mariano GP AD - M. Caminiti, MD, G. Pagano Mariano, MD, F. Calabrese, MD, Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria. FAU - Calabrese, Francesca AU - Calabrese F AD - M. Caminiti, MD, G. Pagano Mariano, MD, F. Calabrese, MD, Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria. FAU - Furini, Federica AU - Furini F AUID- ORCID: 0000-0002-0295-1913 AD - F. Furini, MD, L. Vultaggio, MD, M. Govoni, MD, Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara. FAU - Vultaggio, Licia AU - Vultaggio L AD - F. Furini, MD, L. Vultaggio, MD, M. Govoni, MD, Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara. FAU - Parisi, Simone AU - Parisi S AD - S. Parisi, MD, C.L. Peroni, MD, E. Fusaro, MD, Rheumatology Unit, Città della Salute e della Scienza, Turin. FAU - Peroni, Clara Lisa AU - Peroni CL AD - S. Parisi, MD, C.L. Peroni, MD, E. Fusaro, MD, Rheumatology Unit, Città della Salute e della Scienza, Turin. FAU - Risa, Anna Maria AU - Risa AM AD - R. De Angelis, MD, A.M. Risa, MD, Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona. FAU - Rozza, Davide AU - Rozza D AD - D.Rozza, MS, A. Zanetti, MS, G. Carrara, MS, G. Landolfi, MS, Epidemiology Unit, Italian Society for Rheumatology, Milan. FAU - Zanetti, Anna AU - Zanetti A AUID- ORCID: 0000-0001-8408-451X AD - D.Rozza, MS, A. Zanetti, MS, G. Carrara, MS, G. Landolfi, MS, Epidemiology Unit, Italian Society for Rheumatology, Milan. FAU - Carrara, Greta AU - Carrara G AD - D.Rozza, MS, A. Zanetti, MS, G. Carrara, MS, G. Landolfi, MS, Epidemiology Unit, Italian Society for Rheumatology, Milan. FAU - Landolfi, Giampiero AU - Landolfi G AD - D.Rozza, MS, A. Zanetti, MS, G. Carrara, MS, G. Landolfi, MS, Epidemiology Unit, Italian Society for Rheumatology, Milan. FAU - Scirè, Carlo Alberto AU - Scirè CA AUID- ORCID: 0000-0001-7451-0271 AD - C.A. Scirè, MD, Epidemiology Unit, Italian Society for Rheumatology, Milan, and Rheumatology Unit, University of Ferrara-S. Anna Hospital, Ferrara. FAU - Bianchi, Gerolamo AU - Bianchi G AD - G. Bianchi, MD, Rheumatology Unit, Department of Musculoskeletal Sciences, Local Health Trust 3, La Colletta Hospital, Genoa. FAU - Fusaro, Enrico AU - Fusaro E AD - S. Parisi, MD, C.L. Peroni, MD, E. Fusaro, MD, Rheumatology Unit, Città della Salute e della Scienza, Turin. FAU - Sebastiani, Gian Domenico AU - Sebastiani GD AUID- ORCID: 0000-0003-2969-6649 AD - A. Iuliano, MD, G.D. Sebastiani, MD, Rheumatology Unit, San Camillo-Forlanini Hospital, Rome. FAU - Govoni, Marcello AU - Govoni M AUID- ORCID: 0000-0003-0499-5773 AD - F. Furini, MD, L. Vultaggio, MD, M. Govoni, MD, Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara. FAU - D'Angelo, Salvatore AU - D'Angelo S AUID- ORCID: 0000-0002-7442-1110 AD - G. Abignano, MD, G. Mennillo, MD, S. D'Angelo, MD, Department of Translational and Precision Medicine, Sapienza University of Rome. FAU - Cozzi, Franco AU - Cozzi F AUID- ORCID: 0000-0003-3627-3927 AD - E. Pigatto, MD, F. Cozzi, MD, Department of Medicine, Villa Salus Hospital, Venice. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - E. Zanatta, MD, A. Doria, MD, Department of Rheumatology, University of Padua, Padova. FAU - Iannone, Florenzo AU - Iannone F AUID- ORCID: 0000-0003-0474-5344 AD - F. Cacciapaglia, MD, F. Iannone, MD, Rheumatology Unit, Department of Emergency Surgery and Organ Transplantations, University of Bari, Bari. FAU - Salvarani, Carlo AU - Salvarani C AUID- ORCID: 0000-0001-5426-5133 AD - G. Bajocchi, MD, L. Magnani, MD, C. Salvarani, MD, Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AUID- ORCID: 0000-0002-9324-3161 AD - M. Matucci-Cerinic, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, and Department of Rheumatology, University of Florence, Florence, Italy. FAU - Ferri, Clodoveo AU - Ferri C AUID- ORCID: 0000-0002-0840-5817 AD - D. Giuggioli, MD, F. Lumetti, MD, A. Spinella, MD, C. Ferri, MD, Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena. CN - Systemic sclerosis PRogression INvestiGation group (SPRING) LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20211115 PL - Canada TA - J Rheumatol JT - The Journal of rheumatology JID - 7501984 SB - IM MH - Cross-Sectional Studies MH - Female MH - Humans MH - Italy/epidemiology MH - Male MH - Registries MH - *Rheumatology MH - *Scleroderma, Systemic/diagnosis MH - Sex Characteristics MH - *Sjogren's Syndrome MH - Stroke Volume MH - Ventricular Function, Left OTO - NOTNLM OT - scleroderma OT - sex OT - systemic sclerosis FIR - Ferri, Clodoveo IR - Ferri C FIR - Matucci-Cerinic, Marco IR - Matucci-Cerinic M FIR - Abignano, Giuseppina IR - Abignano G FIR - Agnes, Cecilia IR - Agnes C FIR - Amato, Giorgio IR - Amato G FIR - Ariani, Alarico IR - Ariani A FIR - Bagnato, Gianluca IR - Bagnato G FIR - Bajoicchi, Gianluigi IR - Bajoicchi G FIR - Barsotti, Simone IR - Barsotti S FIR - Bellando-Randone, Silvia IR - Bellando-Randone S FIR - Benenati, Alessia IR - Benenati A FIR - Beretta, Lorenzo IR - Beretta L FIR - Bianchi, Gerolamo IR - Bianchi G FIR - Bosello, Silvia IR - Bosello S FIR - Cacciapaglia, Fabio IR - Cacciapaglia F FIR - Calabrese, Francesca IR - Calabrese F FIR - Caminiti, Maurizio IR - Caminiti M FIR - Campochiaro, Corrado IR - Campochiaro C FIR - Carignola, Renato IR - Carignola R FIR - Cavazzana, Ilaria IR - Cavazzana I FIR - Ciano, Giovanni IR - Ciano G FIR - Codullo, Veronica IR - Codullo V FIR - Cozzi, Franco IR - Cozzi F FIR - Giovanna, Cuomo IR - Giovanna C FIR - D'Angelo, Salvatore IR - D'Angelo S FIR - Dagna, Lorenzo IR - Dagna L FIR - Francesca, Dall'Ara IR - Francesca D FIR - De Andres, Ilenia IR - De Andres I FIR - De Angelis, Rossella IR - De Angelis R FIR - De Cata, Angelo IR - De Cata A FIR - De Luca, Giacomo IR - De Luca G FIR - De Santis, Maria IR - De Santis M FIR - Rossa, Alessandra Della IR - Rossa AD FIR - Doria, Andrea IR - Doria A FIR - Marica, Doveri IR - Marica D FIR - Foti, Rosario IR - Foti R FIR - Furini, Federica IR - Furini F FIR - Fusaro, Enrico IR - Fusaro E FIR - Generali, Elena IR - Generali E FIR - Gigante, Antonietta IR - Gigante A FIR - Giollo, Alessandro IR - Giollo A FIR - Girelli, Francesco IR - Girelli F FIR - Giuggioli, Dilia IR - Giuggioli D FIR - Govoni, Marcello IR - Govoni M FIR - Guiducci, Serena IR - Guiducci S FIR - Iannone, Florenzo IR - Iannone F FIR - Ingegnoli, Francesca IR - Ingegnoli F FIR - Iuliano, Anna Maria IR - Iuliano AM FIR - Lazzaroni, Maria Grazia IR - Lazzaroni MG FIR - Lubrano, Ennio IR - Lubrano E FIR - Lumetti, Federica IR - Lumetti F FIR - Magnani, Luca IR - Magnani L FIR - Masini, Francesco IR - Masini F FIR - Mennillo, Gianna IR - Mennillo G FIR - Murdaca, Giuseppe IR - Murdaca G FIR - Mariano, Giuseppa Pagano IR - Mariano GP FIR - Parisi, Simone IR - Parisi S FIR - Pellegrino, Greta IR - Pellegrino G FIR - Peroni, Clara Lisa IR - Peroni CL FIR - Pigatto, Erika IR - Pigatto E FIR - Riccieri, Valeria IR - Riccieri V FIR - Risa, Anna Maria IR - Risa AM FIR - Romeo, Nicoletta IR - Romeo N FIR - Rosato, Edoardo IR - Rosato E FIR - Sambataro, Gianluca IR - Sambataro G FIR - Saracco, Marta IR - Saracco M FIR - Sebastiani, Giandomenico IR - Sebastiani G FIR - Spinella, Amelia IR - Spinella A FIR - Talotta, Rossella IR - Talotta R FIR - Visalli, Elisa IR - Visalli E FIR - Vultaggio, Licia IR - Vultaggio L FIR - Zanatta, Elisabetta IR - Zanatta E FIR - Zanframundo, Giovanni IR - Zanframundo G EDAT- 2021/11/17 06:00 MHDA- 2022/05/03 06:00 CRDT- 2021/11/16 06:13 PHST- 2021/10/27 00:00 [accepted] PHST- 2021/11/17 06:00 [pubmed] PHST- 2022/05/03 06:00 [medline] PHST- 2021/11/16 06:13 [entrez] AID - jrheum.210794 [pii] AID - 10.3899/jrheum.210794 [doi] PST - ppublish SO - J Rheumatol. 2022 Feb;49(2):176-185. doi: 10.3899/jrheum.210794. Epub 2021 Nov 15. PMID- 36324736 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20221104 IS - 1758-8340 (Print) IS - 1758-8359 (Electronic) IS - 1758-8340 (Linking) VI - 14 DP - 2022 TI - Comorbidity between lung cancer and COVID-19 pneumonia: role of immunoregulatory gene transcripts in high ACE2-expressing normal lung. PG - 17588359221133893 LID - 10.1177/17588359221133893 [doi] LID - 17588359221133893 AB - BACKGROUND: SARS-CoV-2 (COVID-19) elicits a T-cell antigen-mediated immune response of variable efficacy. To understand this variability, we explored transcriptomic expression of angiotensin-converting enzyme 2 (ACE2, the SARS-CoV-2 receptor) and of immunoregulatory genes in normal lung tissues from patients with non-small cell lung cancer (NSCLC). METHODS: This study used the transcriptomic and the clinical data for NSCLC patients generated during the CHEMORES study [n = 123 primary resected (early-stage) NSCLC] and the WINTHER clinical trial (n = 32 metastatic NSCLC). RESULTS: We identified patient subgroups with high and low ACE2 expression (p = 1.55 × 10(-19)) in normal lung tissue, presumed to be at higher and lower risk, respectively, of developing severe COVID-19 should they become infected. ACE2 transcript expression in normal lung tissues (but not in tumor tissue) of patients with NSCLC was higher in individuals with more advanced disease. High-ACE2 expressors had significantly higher levels of CD8+ cytotoxic T lymphocytes and natural killer cells but with presumably impaired function by high Thymocyte Selection-Associated High Mobility Group Box Protein TOX (TOX) expression. In addition, immune checkpoint-related molecules - PD-L1, CTLA-4, PD-1, and TIGIT - are more highly expressed in normal (but not tumor) lung tissues; these molecules might dampen immune response to either viruses or cancer. Importantly, however, high inducible T-cell co-stimulator (ICOS), which can amplify immune and cytokine reactivity, significantly correlated with high ACE2 expression in univariable analysis of normal lung (but not lung tumor tissue). CONCLUSIONS: We report a normal lung immune-tolerant state that may explain a potential comorbidity risk between two diseases - NSCLC and susceptibility to COVID-19 pneumonia. Further, a NSCLC patient subgroup has normal lung tissue expressing high ACE2 and high ICOS transcripts, the latter potentially promoting a hyperimmune response, and possibly leading to severe COVID-19 pulmonary compromise. CI - © The Author(s), 2022. FAU - Lazar, Vladimir AU - Lazar V AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, 9 rue Guy Moquet, Villejuif, 94800, France. FAU - Raynaud, Jacques AU - Raynaud J AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Magidi, Shai AU - Magidi S AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Bresson, Catherine AU - Bresson C AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Martini, Jean-François AU - Martini JF AD - Pfizer Inc., San Diego, CA, USA. FAU - Galbraith, Susan AU - Galbraith S AD - AstraZeneca, Gaithersburg, MD, USA. FAU - Wunder, Fanny AU - Wunder F AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Onn, Amir AU - Onn A AD - Sheba Medical Center, Tel-Hashomer, Israel. FAU - Batist, Gerald AU - Batist G AD - Segal Cancer Centre, Jewish General Hospital, McGill University, Montréal, Canada. FAU - Girard, Nicolas AU - Girard N AD - Institut Curie, Paris, France. FAU - Lassen, Ulrik AU - Lassen U AD - Rigshospitalet, Copenhagen, Denmark. FAU - Pramesh, C S AU - Pramesh CS AD - Tata Memorial Hospital, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India. FAU - Al-Omari, Amal AU - Al-Omari A AD - King Hussein Cancer Center, Amman, Jordan. FAU - Ikeda, Sadakatsu AU - Ikeda S AD - Tokyo Medical and Dental University, Tokyo, Japan. FAU - Berchem, Guy AU - Berchem G AD - Centre Hospitalier Luxembourg and Luxembourg Institute of Health, Luxembourg, Luxembourg. FAU - Blay, Jean-Yves AU - Blay JY AD - Centre Leon Bérard, University Lyon 1, LYRICAN & NETSARC+, Lyon, France. FAU - Solomon, Benjamin AU - Solomon B AD - Avera Cancer Institute, Sioux Falls, SD, USA. FAU - Felip, Enriqueta AU - Felip E AD - Vall d'Hebron Hospital Campus and Institute of Oncology, UVic-UCC, Barcelona, Spain. FAU - Tabernero, Josep AU - Tabernero J AD - Sheba Medical Center, Tel-Hashomer, Israel. FAU - Rubin, Eitan AU - Rubin E AD - Faculty of Health Sciences Ben-Gurion University of the Negev, Beer-Sheeva, Israel. FAU - Philip, Thierry AU - Philip T AD - Institut Curie, Paris, France. FAU - Porgador, Angel AU - Porgador A AD - Faculty of Health Sciences Ben-Gurion University of the Negev, Beer-Sheeva, Israel. FAU - Berindan-Neagoe, Ioana AU - Berindan-Neagoe I AD - Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania. FAU - Schilsky, Richard L AU - Schilsky RL AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Kurzrock, Razelle AU - Kurzrock R AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. LA - eng PT - Journal Article DEP - 20221028 PL - England TA - Ther Adv Med Oncol JT - Therapeutic advances in medical oncology JID - 101510808 PMC - PMC9618916 OTO - NOTNLM OT - ACE2 expression OT - COVID-19 OT - cancer OT - normal lung OT - transcriptomics EDAT- 2022/11/04 06:00 MHDA- 2022/11/04 06:01 PMCR- 2022/10/28 CRDT- 2022/11/03 02:32 PHST- 2022/05/04 00:00 [received] PHST- 2022/10/03 00:00 [accepted] PHST- 2022/11/03 02:32 [entrez] PHST- 2022/11/04 06:00 [pubmed] PHST- 2022/11/04 06:01 [medline] PHST- 2022/10/28 00:00 [pmc-release] AID - 10.1177_17588359221133893 [pii] AID - 10.1177/17588359221133893 [doi] PST - epublish SO - Ther Adv Med Oncol. 2022 Oct 28;14:17588359221133893. doi: 10.1177/17588359221133893. eCollection 2022. PMID- 34011278 OWN - NLM STAT- MEDLINE DCOM- 20210601 LR - 20231111 IS - 1471-2334 (Electronic) IS - 1471-2334 (Linking) VI - 21 IP - 1 DP - 2021 May 19 TI - Lessons learned from the investigation of a COVID-19 cluster in Creil, France: effectiveness of targeting symptomatic cases and conducting contact tracing around them. PG - 457 LID - 10.1186/s12879-021-06166-9 [doi] LID - 457 AB - BACKGROUND: This study presents the methods and results of the investigation into a SARS-CoV-2 outbreak in a professional community. Due to the limited testing capacity available in France at the time, we elaborated a testing strategy according to pre-test probability. METHODS: The investigation design combined active case finding and contact tracing around each confirmed case with testing of at-risk contact persons who had any evocative symptoms (n = 88). One month later, we performed serology testing to test and screen symptomatic and asymptomatic cases again (n = 79). RESULTS: Twenty-four patients were confirmed (14 with RT-PCR and 10 with serology). The attack rate was 29% (24/83). Median age was 40 (24 to 59), and the sex ratio was 15/12. Only three cases were asymptomatic (= no symptoms at all, 13%, 95% CI, 3-32). Nineteen symptomatic cases (79%, 95% CI, 63-95) presented a respiratory infection, two of which were severe. All the RT-PCR confirmed cases acquired protective antibodies. Median incubation was 4 days (from 1 to 13 days), and the median serial interval was 3 days (0 to 15). We identified pre-symptomatic transmission in 40% of this cluster, but no transmission from asymptomatic to symptomatic cases. CONCLUSION: We report the effective use of targeted testing according to pre-test probability, specifically prioritizing symptomatic COVID-19 diagnosis and contact tracing. The asymptomatic rate raises questions about the real role of asymptomatic infected people in transmission. Conversely, pre-symptomatic contamination occurred frequently in this cluster, highlighting the need to identify, test, and quarantine asymptomatic at-risk contact persons (= contact tracing). The local lockdown imposed helped reduce transmission during the investigation period. FAU - de Laval, Franck AU - de Laval F AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. f_de_laval@hotmail.com. AD - Aix-Marseille University, INSERM, IRD, SESSTIM (Economic and Social Sciences, Health Systems, and Medical Informatics), Marseille, France. f_de_laval@hotmail.com. FAU - Grosset-Janin, Anaïs AU - Grosset-Janin A AD - SSA (French Military Health Service), 1st Armed Forces Medical Center, Paris, France. FAU - Delon, François AU - Delon F AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. AD - Aix-Marseille University, INSERM, IRD, SESSTIM (Economic and Social Sciences, Health Systems, and Medical Informatics), Marseille, France. FAU - Allonneau, Alexandre AU - Allonneau A AD - SSA (French Military Health Service), 1st Armed Forces Medical Center, Paris, France. FAU - Tong, Christelle AU - Tong C AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. FAU - Letois, Flavie AU - Letois F AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. FAU - Couderc, Anne AU - Couderc A AD - SSA (French Military Health Service), 1st Armed Forces Medical Center, Paris, France. FAU - Sanchez, Marc-Antoine AU - Sanchez MA AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. FAU - Destanque, César AU - Destanque C AD - SSA (French Military Health Service), 1st Armed Forces Medical Center, Paris, France. FAU - Biot, Fabrice AU - Biot F AD - SSA (French Military Health Service), Armed Forces Biomedical Research Institute, Brétigny-sur-Orge, France. FAU - Raynaud, Françoise AU - Raynaud F AD - Direction Générale de l'Armement, Maîtrise NRBC, Vert-le-Petit, France. FAU - Bigaillon, Christine AU - Bigaillon C AD - SSA (French Military Health Service), Bégin Military Teaching Hospital, Saint-Mandé, France. FAU - Ferraris, Olivier AU - Ferraris O AD - SSA (French Military Health Service), Armed Forces Biomedical Research Institute, Brétigny-sur-Orge, France. FAU - Simon-Loriere, Etienne AU - Simon-Loriere E AD - G5 Evolutionary genomics of RNA viruses, Institut Pasteur, Paris, France. FAU - Enouf, Vincent AU - Enouf V AD - National Reference Center for Respiratory Viruses, Molecular Genetics of RNA Viruses, Institut Pasteur, CNRS-UMR 3569, University of Paris, Paris, France. AD - Mutualized Platform of Microbiology, Pasteur International Bioresources Network, Institut Pasteur, Paris, France. FAU - Andriamanantena, Dinaherisoa AU - Andriamanantena D AD - SSA (French Military Health Service), Bégin Military Teaching Hospital, Saint-Mandé, France. FAU - de Santi, Vincent Pommier AU - de Santi VP AD - SSA (French Military Health Service), CESPA (French Armed Forces Center for Epidemiology and Public Health), Epidemiological Surveillance and Investigations Unit, BdD Marseille-Aubagne, 111 Avenue de la Corse, Marseille, 13568, France. AD - Aix-Marseille University, IRD, AP-HM, SSA (French Military Health Service), VITROME, Marseille, France. FAU - Javelle, Emilie AU - Javelle E AD - Aix-Marseille University, IRD, AP-HM, SSA (French Military Health Service), VITROME, Marseille, France. AD - SSA (French Military Health Service), Laveran Military Teaching Hospital, Marseille, France. FAU - Mérens, Audrey AU - Mérens A AD - SSA (French Military Health Service), Bégin Military Teaching Hospital, Saint-Mandé, France. LA - eng PT - Journal Article DEP - 20210519 PL - England TA - BMC Infect Dis JT - BMC infectious diseases JID - 100968551 RN - 0 (RNA, Viral) SB - IM MH - Adult MH - COVID-19/diagnosis/epidemiology/*prevention & control/virology MH - COVID-19 Testing MH - *Contact Tracing MH - Disease Outbreaks MH - France/epidemiology MH - Humans MH - Male MH - Middle Aged MH - RNA, Viral/analysis/metabolism MH - Reverse Transcriptase Polymerase Chain Reaction MH - SARS-CoV-2/genetics/isolation & purification MH - Young Adult PMC - PMC8133048 OTO - NOTNLM OT - Asymptomatic OT - COVID-19 OT - Cluster OT - RT-PCR OT - SARS-CoV-2 COIS- The authors declare that they have no competing interests. EDAT- 2021/05/21 06:00 MHDA- 2021/06/02 06:00 PMCR- 2021/05/19 CRDT- 2021/05/20 05:35 PHST- 2020/12/18 00:00 [received] PHST- 2021/05/06 00:00 [accepted] PHST- 2021/05/20 05:35 [entrez] PHST- 2021/05/21 06:00 [pubmed] PHST- 2021/06/02 06:00 [medline] PHST- 2021/05/19 00:00 [pmc-release] AID - 10.1186/s12879-021-06166-9 [pii] AID - 6166 [pii] AID - 10.1186/s12879-021-06166-9 [doi] PST - epublish SO - BMC Infect Dis. 2021 May 19;21(1):457. doi: 10.1186/s12879-021-06166-9. PMID- 40425534 OWN - NLM STAT- MEDLINE DCOM- 20250527 LR - 20250730 IS - 2041-1723 (Electronic) IS - 2041-1723 (Linking) VI - 16 IP - 1 DP - 2025 May 27 TI - WNK1 signalling regulates amino acid transport and mTORC1 activity to sustain acute myeloid leukaemia growth. PG - 4920 LID - 10.1038/s41467-025-59969-8 [doi] LID - 4920 AB - The lack of curative therapies for acute myeloid leukaemia (AML) remains an ongoing challenge despite recent advances in the understanding of the molecular basis of the disease. Here we identify the WNK1-OXSR1/STK39 pathway as a previously uncharacterised dependency in AML. We show that genetic depletion and pharmacological inhibition of WNK1 or its downstream phosphorylation targets OXSR1 and STK39 strongly reduce cell proliferation and induce apoptosis in leukaemia cells in vitro and in vivo. Furthermore, we show that the WNK1-OXSR1/STK39 pathway controls mTORC1 signalling via regulating amino acid uptake through a mechanism involving the phosphorylation of amino acid transporters, such as SLC38A2. Our findings underscore an important role of the WNK1-OXSR1/STK39 pathway in regulating amino acid uptake and driving AML progression. CI - © 2025. The Author(s). FAU - Duan, Shunlei AU - Duan S AUID- ORCID: 0000-0003-0548-5150 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. FAU - Agger, Karl AU - Agger K AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. FAU - Messling, Jan-Erik AU - Messling JE AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. FAU - Nishimura, Koutarou AU - Nishimura K AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. AD - Cell Biology Program and Center for Epigenetics Research, Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Han, Xuerui AU - Han X AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. FAU - Peña-Rømer, Isabel AU - Peña-Rømer I AUID- ORCID: 0000-0002-2987-6538 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. FAU - Shliaha, Pavel AU - Shliaha P AD - Microchemistry and Proteomics Core Facility, Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Damhofer, Helene AU - Damhofer H AUID- ORCID: 0000-0003-4459-7534 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. AD - Cell Biology Program and Center for Epigenetics Research, Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Douglas, Max AU - Douglas M AUID- ORCID: 0000-0003-1186-7163 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. FAU - Kohli, Manas AU - Kohli M AUID- ORCID: 0000-0002-3904-2158 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. FAU - Pal, Akos AU - Pal A AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Asad, Yasmin AU - Asad Y AUID- ORCID: 0000-0002-3112-8694 AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Van Dyke, Aaron AU - Van Dyke A AUID- ORCID: 0000-0002-6989-4394 AD - Department of Chemistry & Biochemistry, Fairfield University, Fairfield, CT, USA. FAU - Reilly, Raquel AU - Reilly R AD - Department of Chemistry & Biochemistry, Fairfield University, Fairfield, CT, USA. FAU - Köchl, Robert AU - Köchl R AUID- ORCID: 0000-0003-3370-5501 AD - The Francis Crick Institute, London, UK. FAU - Tybulewicz, Victor L J AU - Tybulewicz VLJ AUID- ORCID: 0000-0003-2439-0798 AD - The Francis Crick Institute, London, UK. FAU - Hendrickson, Ronald C AU - Hendrickson RC AD - Microchemistry and Proteomics Core Facility, Memorial Sloan Kettering Cancer Center, New York, NY, USA. FAU - Raynaud, Florence I AU - Raynaud FI AUID- ORCID: 0000-0003-0957-6279 AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, UK. FAU - Gallipoli, Paolo AU - Gallipoli P AUID- ORCID: 0000-0001-7254-2253 AD - Centre for Haemato-Oncology, Barts Cancer Institute, Queen Mary University of London, London, UK. FAU - Poulogiannis, George AU - Poulogiannis G AUID- ORCID: 0000-0002-0529-8614 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. FAU - Helin, Kristian AU - Helin K AUID- ORCID: 0000-0003-1975-6097 AD - Division of Cell and Molecular Biology, The Institute of Cancer Research, Londo, UK. kristian.helin@icr.ac.uk. AD - Biotech Research and Innovation Centre, University of Copenhagen, Copenhagen, Denmark. kristian.helin@icr.ac.uk. AD - Cell Biology Program and Center for Epigenetics Research, Memorial Sloan Kettering Cancer Center, New York, NY, USA. kristian.helin@icr.ac.uk. LA - eng GR - R223-A13071/Kræftens Bekæmpelse (Danish Cancer Society)/ GR - NNF17CC0027852/Novo Nordisk Fonden (Novo Nordisk Foundation)/ GR - R167-A10877/Kræftens Bekæmpelse (Danish Cancer Society)/ GR - P30 CA008748/CA/NCI NIH HHS/United States GR - C57799/A27964/Cancer Research UK (CRUK)/ PT - Journal Article DEP - 20250527 PL - England TA - Nat Commun JT - Nature communications JID - 101528555 RN - EC 2.7.11.1 (Mechanistic Target of Rapamycin Complex 1) RN - EC 2.7.11.1 (WNK Lysine-Deficient Protein Kinase 1) RN - 0 (Amino Acids) RN - EC 2.7.11.1 (WNK1 protein, human) RN - EC 2.7.11.1 (Protein Serine-Threonine Kinases) RN - EC 2.7.11.1 (STK39 protein, human) SB - IM MH - *Mechanistic Target of Rapamycin Complex 1/metabolism/genetics MH - *Leukemia, Myeloid, Acute/metabolism/pathology/genetics MH - Humans MH - Signal Transduction MH - *WNK Lysine-Deficient Protein Kinase 1/metabolism/genetics MH - Animals MH - Cell Proliferation MH - *Amino Acids/metabolism MH - Cell Line, Tumor MH - Mice MH - *Protein Serine-Threonine Kinases/metabolism/genetics MH - Phosphorylation MH - Apoptosis PMC - PMC12116911 COIS- Competing interests: K.H. and K.A. are co-founders of Dania Therapeutics. K.H. is a scientific advisor for Hannibal Innovation and was recently a scientific advisor for Inthera Bioscience AG and for MetaboMed Inc. The remaining authors declare no competing interests. EDAT- 2025/05/28 00:27 MHDA- 2025/05/28 00:28 PMCR- 2025/05/27 CRDT- 2025/05/27 23:13 PHST- 2024/05/13 00:00 [received] PHST- 2025/05/08 00:00 [accepted] PHST- 2025/05/28 00:28 [medline] PHST- 2025/05/28 00:27 [pubmed] PHST- 2025/05/27 23:13 [entrez] PHST- 2025/05/27 00:00 [pmc-release] AID - 10.1038/s41467-025-59969-8 [pii] AID - 59969 [pii] AID - 10.1038/s41467-025-59969-8 [doi] PST - epublish SO - Nat Commun. 2025 May 27;16(1):4920. doi: 10.1038/s41467-025-59969-8. PMID- 34435556 OWN - NLM STAT- MEDLINE DCOM- 20220124 LR - 20220124 IS - 1469-8978 (Electronic) IS - 0033-2917 (Linking) VI - 51 IP - 14 DP - 2021 Oct TI - Preventive interventions in offspring of parents with mental illness: a systematic review and meta-analysis of randomized controlled trials. PG - 2321-2336 LID - 10.1017/S0033291721003366 [doi] AB - Children with parents suffering from a psychiatric disorder are at higher risk for developing a mental disorder themselves. This systematic review and meta-analysis of randomized controlled trials aims to evaluate the efficacy of psychosocial interventions to prevent negative mental health outcomes in the offspring of parents with mental illness. Eight electronic databases, grey literature and a journal hand-search identified 14 095 randomized controlled trials with no backward limit to June 2021. Outcomes in children included incidence of mental disorders (same or different from parental ones) and internalizing and externalizing symptoms at post-test, short-term and long-term follow-up. Relative risks and standardized mean differences (SMD) for symptom severity were generated using random-effect meta-analyses. Twenty trials were selected (pooled n = 2689 children). The main therapeutic approaches found were cognitive-behavioural therapy and psychoeducation. A significant effect of interventions on the incidence of mental disorders in children was found with a risk reduction of almost 50% [combined relative risk = 0.53, 95% confidence interval (CI) 0.34-0.84]. Interventions also had a small but significant effect on internalizing symptoms at post-test (SMD = -0.25, 95% CI -0.37 to -0.14) and short-term follow-up (-0.20, 95% CI -0.37 to -0.03). For externalizing symptoms, a decreasing slope was observed at post-test follow-up, without reaching the significance level (-0.11, 95% CI -0.27 to 0.04). Preventive interventions targeting the offspring of parents with mental disorders showed not only a significant reduction of the incidence of mental illness in children, but also a diminution of internalizing symptoms in the year following the intervention. FAU - Lannes, Alice AU - Lannes A AUID- ORCID: 0000-0003-3031-1443 AD - Department of Child and Adolescent Psychiatry, Toulouse University Hospital, Toulouse, France. FAU - Bui, Eric AU - Bui E AUID- ORCID: 0000-0002-1413-6473 AD - Department of Psychiatry, Massachusetts General Hospital, Boston, MA, USA. AD - University of Caen Normandy & Caen University Hospital, Caen, France. FAU - Arnaud, Catherine AU - Arnaud C AUID- ORCID: 0000-0002-4002-802X AD - CERPOP, UMR 1295, Inserm, University Paul Sabatier Toulouse, Toulouse, France. AD - Clinical Epidemiology Unit, Toulouse University Hospital, Toulouse, France. FAU - Raynaud, Jean-Philippe AU - Raynaud JP AUID- ORCID: 0000-0002-6101-7370 AD - Department of Child and Adolescent Psychiatry, Toulouse University Hospital, Toulouse, France. AD - CERPOP, UMR 1295, Inserm, University Paul Sabatier Toulouse, Toulouse, France. FAU - Revet, Alexis AU - Revet A AUID- ORCID: 0000-0002-8051-1657 AD - Department of Child and Adolescent Psychiatry, Toulouse University Hospital, Toulouse, France. AD - CERPOP, UMR 1295, Inserm, University Paul Sabatier Toulouse, Toulouse, France. AD - CIC 1436, Team PEPSS, « Pharmacologie En Population cohorteS et biobanqueS », Toulouse University Hospital, Toulouse, France. LA - eng PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20210826 PL - England TA - Psychol Med JT - Psychological medicine JID - 1254142 SB - IM MH - Child MH - Child of Impaired Parents/*psychology MH - *Cognitive Behavioral Therapy MH - Genetic Predisposition to Disease MH - Humans MH - *Mental Disorders/diagnosis/prevention & control MH - Parents/*psychology MH - *Psychosocial Intervention MH - *Randomized Controlled Trials as Topic MH - Risk Reduction Behavior OTO - NOTNLM OT - Meta-analysis OT - internalizing and externalizing symptoms OT - mentally ill parents OT - offspring OT - preventive intervention EDAT- 2021/08/27 06:00 MHDA- 2022/01/27 06:00 CRDT- 2021/08/26 08:44 PHST- 2021/08/27 06:00 [pubmed] PHST- 2022/01/27 06:00 [medline] PHST- 2021/08/26 08:44 [entrez] AID - S0033291721003366 [pii] AID - 10.1017/S0033291721003366 [doi] PST - ppublish SO - Psychol Med. 2021 Oct;51(14):2321-2336. doi: 10.1017/S0033291721003366. Epub 2021 Aug 26. PMID- 24607389 OWN - NLM STAT- MEDLINE DCOM- 20140501 LR - 20211021 IS - 1537-6605 (Electronic) IS - 0002-9297 (Print) IS - 0002-9297 (Linking) VI - 94 IP - 3 DP - 2014 Mar 6 TI - Mutations in USP9X are associated with X-linked intellectual disability and disrupt neuronal cell migration and growth. PG - 470-8 LID - S0002-9297(14)00062-7 [pii] LID - 10.1016/j.ajhg.2014.02.004 [doi] AB - With a wealth of disease-associated DNA variants being recently reported, the challenges of providing their functional characterization are mounting. Previously, as part of a large systematic resequencing of the X chromosome in 208 unrelated families with nonsyndromic X-linked intellectual disability, we identified three unique variants (two missense and one protein truncating) in USP9X. To assess the functional significance of these variants, we took advantage of the Usp9x knockout mouse we generated. Loss of Usp9x causes reduction in both axonal growth and neuronal cell migration. Although overexpression of wild-type human USP9X rescued these defects, all three USP9X variants failed to rescue axonal growth, caused reduced USP9X protein localization in axonal growth cones, and (in 2/3 variants) failed to rescue neuronal cell migration. Interestingly, in one of these families, the proband was subsequently identified to have a microdeletion encompassing ARID1B, a known ID gene. Given our findings it is plausible that loss of function of both genes contributes to the individual's phenotype. This case highlights the complexity of the interpretations of genetic findings from genome-wide investigations. We also performed proteomics analysis of neurons from both the wild-type and Usp9x knockout embryos and identified disruption of the cytoskeleton as the main underlying consequence of the loss of Usp9x. Detailed clinical assessment of all three families with USP9X variants identified hypotonia and behavioral and morphological defects as common features in addition to ID. Together our data support involvement of all three USP9X variants in ID in these families and provide likely cellular and molecular mechanisms involved. CI - Copyright © 2014 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved. FAU - Homan, Claire C AU - Homan CC AD - School of Molecular and Biomedical Science, University of Adelaide, Adelaide, SA 5005, Australia. FAU - Kumar, Raman AU - Kumar R AD - Women's and Children's Health Research Institute, North Adelaide, SA 5006, Australia; Discipline of Medicine, University of Adelaide, Adelaide, SA 5005, Australia; School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, SA 5005, Australia. FAU - Nguyen, Lam Son AU - Nguyen LS AD - School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, SA 5005, Australia. FAU - Haan, Eric AU - Haan E AD - School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, SA 5005, Australia; South Australian Clinical Genetics Service, SA Pathology at Women's and Children's Hospital, North Adelaide, SA 5006, Australia. FAU - Raymond, F Lucy AU - Raymond FL AD - Cambridge Institute for Medical Research, University of Cambridge, Cambridge CB2 0XY, UK. FAU - Abidi, Fatima AU - Abidi F AD - J.C. Self Research Institute, Greenwood Genetics Centre, Greenwood, SC 29646, USA. FAU - Raynaud, Martine AU - Raynaud M AD - CHRU de Tours, Service de Génétique, Tours 37000, France; Inserm U930, UMR Imagerie et Cerveau, Tours 37000, France. FAU - Schwartz, Charles E AU - Schwartz CE AD - J.C. Self Research Institute, Greenwood Genetics Centre, Greenwood, SC 29646, USA. FAU - Wood, Stephen A AU - Wood SA AD - Eskitis Institute for Drug Discovery, Griffith University, Brisbane, QLD 4111, Australia. FAU - Gecz, Jozef AU - Gecz J AD - School of Molecular and Biomedical Science, University of Adelaide, Adelaide, SA 5005, Australia; Women's and Children's Health Research Institute, North Adelaide, SA 5006, Australia; School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, SA 5005, Australia; Robinson Institute, University of Adelaide, Adelaide, SA 5005, Australia. Electronic address: jozef.gecz@adelaide.edu.au. FAU - Jolly, Lachlan A AU - Jolly LA AD - School of Paediatrics and Reproductive Health, University of Adelaide, Adelaide, SA 5005, Australia; Robinson Institute, University of Adelaide, Adelaide, SA 5005, Australia. Electronic address: lachlan.jolly@adelaide.edu.au. LA - eng GR - 1R01NS73854/NS/NINDS NIH HHS/United States GR - R01 NS073854/NS/NINDS NIH HHS/United States GR - R01 HD026202/HD/NICHD NIH HHS/United States GR - 2R01HD026202/HD/NICHD NIH HHS/United States GR - 100140/Wellcome Trust/United Kingdom PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Am J Hum Genet JT - American journal of human genetics JID - 0370475 RN - 0 (ARID1B protein, human) RN - 0 (DNA-Binding Proteins) RN - 0 (Transcription Factors) RN - 0 (USP9X protein, human) RN - EC 3.4.19.12 (Ubiquitin Thiolesterase) SB - IM MH - Animals MH - Cell Movement MH - Cell Proliferation MH - *Chromosomes, Human, X MH - Cytoskeleton/metabolism MH - DNA-Binding Proteins/genetics MH - Family Health MH - Female MH - Genes, X-Linked MH - Genetic Variation MH - Humans MH - Intellectual Disability/*genetics MH - Male MH - Mice MH - Mice, Knockout MH - *Mutation MH - Mutation, Missense MH - Neurogenesis/genetics MH - Neurons/*metabolism MH - Phenotype MH - Time Factors MH - Transcription Factors/genetics MH - Ubiquitin Thiolesterase/*genetics/*physiology PMC - PMC3951929 EDAT- 2014/03/13 06:00 MHDA- 2014/05/03 06:00 PMCR- 2014/09/06 CRDT- 2014/03/11 06:00 PHST- 2013/09/03 00:00 [received] PHST- 2014/02/13 00:00 [accepted] PHST- 2014/03/11 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2014/05/03 06:00 [medline] PHST- 2014/09/06 00:00 [pmc-release] AID - S0002-9297(14)00062-7 [pii] AID - 10.1016/j.ajhg.2014.02.004 [doi] PST - ppublish SO - Am J Hum Genet. 2014 Mar 6;94(3):470-8. doi: 10.1016/j.ajhg.2014.02.004. PMID- 23971940 OWN - NLM STAT- MEDLINE DCOM- 20141209 LR - 20260518 IS - 1476-4954 (Electronic) IS - 1476-4954 (Linking) VI - 27 IP - 7 DP - 2014 May TI - Obstetric management does not influence vertical transmission of HCV infection: results of the ALHICE group study. PG - 664-70 LID - 10.3109/14767058.2013.829813 [doi] AB - OBJECTIVE: To investigate the impact of variation in obstetric practice during labor and childbirth upon the rate of neonatal transmission of HCV. METHODS: Pregnant mothers were included in this prospective study from six hospitals in Southern France on the basis of positive HCV serology. Data recorded for the study included maternal factors, delivery details and laboratory data concerning mother and child. Pediatric follow-up was documented for a minimum of 1 year and for up to 2 years for children with circulating HCV RNA. RESULTS: Two hundred and fourteen mother-child pairs were investigated. HIV/HCV co-infected mothers had a rate of HCV transmission significantly higher (11%) than that observed for mono-infected mothers (3.8%) (odds ratio=3.08 [95% CI:0.95 to 9.99] p=0.05). When the HCV viral load was greater than or equal to 6 log copies/ml, the transmission rate was 14.3% [95% CI:5.4-28.5], this representing a risk of transmission four times higher than for women with a lower viral load (OR=4 [95% CI:1.3-12.4]). Among co-infected mothers, the risk of transmission was significantly increased even when the load was less than 6 log copies/ml (p=0.006). Risk factors were identified related to labor (duration and induction type); the birth process (rupture of the amniotic sac, complete opening of the sac, appearance of the amniotic fluid); fetal characteristics (prematurity) and obstetric maneuvers (instrumental extractions, spontaneous or induced perineal trauma) and none of these factors were associated with an increased rate of HCV maternal-fetal transmission. CONCLUSIONS: HCV infection does not appear to be a legitimate indication for modifying obstetric practices with regards to type of induction, monitoring of labor, route of delivery, fetal and perineal obstetric maneuvers or care of the newborn in the delivery room. FAU - Delotte, Jérôme AU - Delotte J AD - Department of Obstetrics, Gynecology, Reproduction and Fetal Medicine . FAU - Barjoan, Eugènia Mariné AU - Barjoan EM FAU - Berrébi, Alain AU - Berrébi A FAU - Laffont, Catherine AU - Laffont C FAU - Benos, Paul AU - Benos P FAU - Pradier, Christian AU - Pradier C FAU - Bongain, André AU - Bongain A CN - ALHICE study group LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130823 PL - England TA - J Matern Fetal Neonatal Med JT - The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians JID - 101136916 SB - IM MH - Adult MH - Coinfection MH - Delivery, Obstetric/*statistics & numerical data MH - Female MH - HIV Infections/complications MH - Hepatitis C/complications/*transmission MH - Humans MH - Infectious Disease Transmission, Vertical/*statistics & numerical data MH - Pregnancy MH - *Pregnancy Complications, Infectious MH - Prospective Studies FIR - Duforestel, T IR - Duforestel T FIR - Flamand, G IR - Flamand G FIR - Magnaldo, S IR - Magnaldo S FIR - Moreigne, M IR - Moreigne M FIR - Raynaud, F IR - Raynaud F FIR - Badetti, L IR - Badetti L FIR - Bébin, B IR - Bébin B FIR - Boismond, H IR - Boismond H FIR - Carmagnole, F IR - Carmagnole F FIR - Sebag, F IR - Sebag F FIR - Azuar, P IR - Azuar P FIR - Court, F IR - Court F FIR - Martin, H IR - Martin H FIR - Ménéguz, C IR - Ménéguz C FIR - Tomassi, C IR - Tomassi C FIR - Sassi, C IR - Sassi C FIR - Boulot, P IR - Boulot P FIR - Benos, P IR - Benos P FIR - Chanal, C IR - Chanal C FIR - Ducos, J IR - Ducos J FIR - Guigue, N IR - Guigue N FIR - Rieu, D IR - Rieu D FIR - Saillard, A IR - Saillard A FIR - Fournier-Favre, S IR - Fournier-Favre S FIR - Mazurier, E IR - Mazurier E FIR - Picaud, J C H IR - Picaud JC FIR - Bongain, A IR - Bongain A FIR - Boutté, P IR - Boutté P FIR - Checchi, C IR - Checchi C FIR - Dellamonica, P IR - Dellamonica P FIR - Ejnès, L IR - Ejnès L FIR - Fafin, A IR - Fafin A FIR - Galiba, E IR - Galiba E FIR - Gillet, J Y IR - Gillet JY FIR - Gilly, V IR - Gilly V FIR - Haas, H IR - Haas H FIR - Laffont, C IR - Laffont C FIR - Malerba, S IR - Malerba S FIR - Mariné Barjoan, E IR - Mariné Barjoan E FIR - Markarian, A IR - Markarian A FIR - Monpoux, F IR - Monpoux F FIR - Pradier, C IR - Pradier C FIR - Ricard, I IR - Ricard I FIR - Trastour, C IR - Trastour C FIR - Tran, A IR - Tran A FIR - Triolo, V IR - Triolo V FIR - Berrebi, A IR - Berrebi A FIR - Izopet, J IR - Izopet J FIR - Tricoire, J IR - Tricoire J FIR - Antras, M IR - Antras M EDAT- 2013/08/27 06:00 MHDA- 2014/12/15 06:00 CRDT- 2013/08/27 06:00 PHST- 2013/08/27 06:00 [entrez] PHST- 2013/08/27 06:00 [pubmed] PHST- 2014/12/15 06:00 [medline] AID - 10.3109/14767058.2013.829813 [doi] PST - ppublish SO - J Matern Fetal Neonatal Med. 2014 May;27(7):664-70. doi: 10.3109/14767058.2013.829813. Epub 2013 Aug 23. PMID- 20159109 OWN - NLM STAT- MEDLINE DCOM- 20100319 LR - 20250529 IS - 1537-6605 (Electronic) IS - 0002-9297 (Print) IS - 0002-9297 (Linking) VI - 86 IP - 2 DP - 2010 Feb 12 TI - Mutations in the small GTPase gene RAB39B are responsible for X-linked mental retardation associated with autism, epilepsy, and macrocephaly. PG - 185-95 LID - 10.1016/j.ajhg.2010.01.011 [doi] AB - Human Mental Retardation (MR) is a common and highly heterogeneous pediatric disorder affecting around 3% of the general population; at least 215 X-linked MR (XLMR) conditions have been described, and mutations have been identified in 83 different genes, encoding proteins with a variety of function, such as chromatin remodeling, synaptic function, and intracellular trafficking. The small GTPases of the RAB family, which play an essential role in intracellular vesicular trafficking, have been shown to be involved in MR. We report here the identification of mutations in the small GTPase RAB39B gene in two male patients. One mutation in family X (D-23) introduced a stop codon seven amino acids after the start codon (c.21C > A; p.Y7X). A second mutation, in the MRX72 family, altered the 5' splice site (c.215+1G > A) and normal splicing. Neither instance produced a protein. Mutations segregate with the disease in the families, and in some family members intellectual disabilities were associated with autism spectrum disorder, epileptic seizures, and macrocephaly. We show that RAB39B, a novel RAB GTPase of unknown function, is a neuronal-specific protein that is localized to the Golgi compartment. Its downregulation leads to an alteration in the number and morphology of neurite growth cones and a significant reduction in presynaptic buttons, suggesting that RAB39B is required for synapse formation and maintenance. Our results demonstrate developmental and functional neuronal alteration as a consequence of downregulation of RAB39B and emphasize the critical role of vesicular trafficking in the development of neurons and human intellectual abilities. CI - Copyright (c) 2010 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved. FAU - Giannandrea, Maila AU - Giannandrea M AD - Dulbecco Telethon Institute at Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy. FAU - Bianchi, Veronica AU - Bianchi V FAU - Mignogna, Maria Lidia AU - Mignogna ML FAU - Sirri, Alessandra AU - Sirri A FAU - Carrabino, Salvatore AU - Carrabino S FAU - D'Elia, Errico AU - D'Elia E FAU - Vecellio, Matteo AU - Vecellio M FAU - Russo, Silvia AU - Russo S FAU - Cogliati, Francesca AU - Cogliati F FAU - Larizza, Lidia AU - Larizza L FAU - Ropers, Hans-Hilger AU - Ropers HH FAU - Tzschach, Andreas AU - Tzschach A FAU - Kalscheuer, Vera AU - Kalscheuer V FAU - Oehl-Jaschkowitz, Barbara AU - Oehl-Jaschkowitz B FAU - Skinner, Cindy AU - Skinner C FAU - Schwartz, Charles E AU - Schwartz CE FAU - Gecz, Jozef AU - Gecz J FAU - Van Esch, Hilde AU - Van Esch H FAU - Raynaud, Martine AU - Raynaud M FAU - Chelly, Jamel AU - Chelly J FAU - de Brouwer, Arjan P M AU - de Brouwer AP FAU - Toniolo, Daniela AU - Toniolo D FAU - D'Adamo, Patrizia AU - D'Adamo P LA - eng GR - R01 HD026202/HD/NICHD NIH HHS/United States GR - TCR09001/TI_/Telethon/Italy GR - HD26202/HD/NICHD NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Am J Hum Genet JT - American journal of human genetics JID - 0370475 RN - 0 (RNA, Small Interfering) RN - EC 3.6.1.- (Rab39B protein, human) RN - EC 3.6.5.2 (rab GTP-Binding Proteins) SB - IM MH - Animals MH - Autistic Disorder/*complications/genetics MH - Base Sequence MH - Brain/metabolism/pathology MH - Cell Differentiation MH - Craniofacial Abnormalities/*complications/genetics MH - DNA Mutational Analysis MH - Down-Regulation/genetics MH - Epilepsy/*complications/genetics MH - Female MH - Golgi Apparatus/metabolism MH - HeLa Cells MH - Humans MH - Male MH - X-Linked Intellectual Disability/*complications/*genetics MH - Mice MH - Molecular Sequence Data MH - Mutation/*genetics MH - Neurons/metabolism/pathology MH - Organ Specificity/genetics MH - Pedigree MH - Protein Transport MH - RNA, Small Interfering/metabolism MH - Synapses/genetics MH - rab GTP-Binding Proteins/*genetics PMC - PMC2820185 EDAT- 2010/02/18 06:00 MHDA- 2010/03/20 06:00 PMCR- 2010/08/12 CRDT- 2010/02/18 06:00 PHST- 2009/10/25 00:00 [received] PHST- 2009/12/28 00:00 [revised] PHST- 2010/01/11 00:00 [accepted] PHST- 2010/02/18 06:00 [entrez] PHST- 2010/02/18 06:00 [pubmed] PHST- 2010/03/20 06:00 [medline] PHST- 2010/08/12 00:00 [pmc-release] AID - S0002-9297(10)00014-5 [pii] AID - AJHG558 [pii] AID - 10.1016/j.ajhg.2010.01.011 [doi] PST - ppublish SO - Am J Hum Genet. 2010 Feb 12;86(2):185-95. doi: 10.1016/j.ajhg.2010.01.011. PMID- 41765245 OWN - NLM STAT- In-Process LR - 20260609 IS - 1873-4596 (Electronic) IS - 0891-5849 (Linking) VI - 248 DP - 2026 May TI - Personalized antioxidant supplementation improves muscle strength, physical activity, and quality of life in patients with FSHD1: A real-world longitudinal study. PG - 424-435 LID - S0891-5849(26)00175-9 [pii] LID - 10.1016/j.freeradbiomed.2026.02.071 [doi] AB - Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant myopathy characterized by progressive muscle weakness. Oxidative stress plays a central role in its pathogenesis. This longitudinal, real-world study evaluated the effect of personalized antioxidant supplementation on quadriceps muscle strength (maximum voluntary contraction; MVC(Q)), physical activity, and quality of life (QoL) in patients with FSHD1. Patients (aged 15-76) at Montpellier Hospital received tailored antioxidant supplementation (vitamins C and E, zinc, copper, and selenomethionine) based on blood analyses during annual visits: 107 completed one year and 74 continued for two more years of supplementation. Linear mixed model analyses showed a significant annual increase in the MVC of both dominant and non-dominant quadriceps muscles (+0.83 kg/year, +1.25 kg/year, respectively), improvement in physical activity, especially sports, and in QoL domains, notably physical role. Sex and age influenced outcomes: supplementation benefits were more pronounced in men and younger patients. Supplementation withdrawal resulted in the benefit loss, while attrition was unrelated to disease severity or treatment response. Overall, our findings suggest that personalized antioxidant supplementation may slow FSHD progression, improve muscle strength and quality of life. Baseline muscle strength and clinical scores, including the Brooke upper extremity scale score, allowed stratifying patients in responders and non-responders. These parameters underscore the importance of baseline assessments to predict outcomes and highlight the need of controlled studies to confirm these preliminary results. CI - Copyright © 2026 The Authors. Published by Elsevier Inc. All rights reserved. FAU - Laoudj-Chenivesse, Dalila AU - Laoudj-Chenivesse D AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France; Department of Clinical Physiology, CHU of Montpellier, Montpellier, France. Electronic address: dalila.laoudj-chenivesse@inserm.fr. FAU - Arbogast, Sandrine AU - Arbogast S AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France. Electronic address: sandrine.arbogast@inserm.fr. FAU - Raynaud De Mauverger, Eric AU - Raynaud De Mauverger E AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France; Department of Clinical Physiology, CHU of Montpellier, Montpellier, France. Electronic address: eric.raynaud-de-mauverger@chu-montpellier.fr. FAU - Fedou, Christine AU - Fedou C AD - Department of Clinical Physiology, CHU of Montpellier, Montpellier, France. Electronic address: christine@fedou.fr. FAU - Debroize, Emma AU - Debroize E AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France. Electronic address: emmadebroize30@gmail.com. FAU - Hugon, Gérald AU - Hugon G AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France. Electronic address: gerald.hugon@inserm.fr. FAU - Pincemail, Joël AU - Pincemail J AD - Department of CREDEC, Department of Medical Chemistry, University Hospital of Liege, Sart Tilman, Liege, Belgium. Electronic address: j.pincemail@chuliege.be. FAU - Picot, Marie-Christine AU - Picot MC AD - Department of Biostatistics and Epidemiology, University Hospital, and CIC 1001-INSERM University of Montpellier, Montpellier, France. Electronic address: mc-picot@chu-montpellier.fr. FAU - Cristol, Jean-Paul AU - Cristol JP AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France; Department of Biochemistry, CHU of Montpellier, Montpellier, France. Electronic address: jp-cristol@chu-montpellier.fr. FAU - Mercier, Jacques AU - Mercier J AD - PhyMedExp, Univ Montpellier, CNRS, INSERM, Montpellier, France; Department of Clinical Physiology, CHU of Montpellier, Montpellier, France. Electronic address: jacques.mercier@umontpellier.fr. FAU - Portet, Florence AU - Portet F AD - Department of Clinical Physiology, CHU of Montpellier, Montpellier, France; U1061 INSERM, CHU of Montpellier, Montpellier, France. Electronic address: floportet@orange.fr. LA - eng PT - Journal Article DEP - 20260228 PL - United States TA - Free Radic Biol Med JT - Free radical biology & medicine JID - 8709159 RN - 0 (Antioxidants) SB - IM MH - Humans MH - *Quality of Life MH - Male MH - *Muscular Dystrophy, Facioscapulohumeral/drug therapy/physiopathology/diet therapy MH - Female MH - *Muscle Strength/drug effects MH - Middle Aged MH - Adult MH - *Antioxidants/administration & dosage/therapeutic use MH - *Dietary Supplements MH - *Exercise MH - Aged MH - Longitudinal Studies MH - Adolescent MH - Young Adult OTO - NOTNLM OT - Antioxidant supplementation (vitamins and trace elements) OT - Efficacy OT - Facioscapulohumeral muscular dystrophy (FSHD) OT - Follow-up OT - France OT - Longitudinal OT - Personalized approach OT - Real-world experience COIS- Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. EDAT- 2026/03/02 00:38 MHDA- 2026/04/09 00:33 CRDT- 2026/03/01 19:14 PHST- 2025/09/16 00:00 [received] PHST- 2026/02/19 00:00 [revised] PHST- 2026/02/27 00:00 [accepted] PHST- 2026/04/09 00:33 [medline] PHST- 2026/03/02 00:38 [pubmed] PHST- 2026/03/01 19:14 [entrez] AID - S0891-5849(26)00175-9 [pii] AID - 10.1016/j.freeradbiomed.2026.02.071 [doi] PST - ppublish SO - Free Radic Biol Med. 2026 May;248:424-435. doi: 10.1016/j.freeradbiomed.2026.02.071. Epub 2026 Feb 28. PMID- 21278242 OWN - NLM STAT- MEDLINE DCOM- 20110722 LR - 20250529 IS - 1557-3265 (Electronic) IS - 1078-0432 (Print) IS - 1078-0432 (Linking) VI - 17 IP - 6 DP - 2011 Mar 15 TI - A phase I study of the heat shock protein 90 inhibitor alvespimycin (17-DMAG) given intravenously to patients with advanced solid tumors. PG - 1561-70 LID - 10.1158/1078-0432.CCR-10-1927 [doi] AB - PURPOSE: A phase I study to define toxicity and recommend a phase II dose of the HSP90 inhibitor alvespimycin (17-DMAG; 17-dimethylaminoethylamino-17-demethoxygeldanamycin). Secondary endpoints included evaluation of pharmacokinetic profile, tumor response, and definition of a biologically effective dose (BED). PATIENTS AND METHODS: Patients with advanced solid cancers were treated with weekly, intravenous (i.v.) 17-DMAG. An accelerated titration dose escalation design was used. The maximum tolerated dose (MTD) was the highest dose at which ≤ 1/6 patients experienced dose limiting toxicity (DLT). Dose de-escalation from the MTD was planned with mandatory, sequential tumor biopsies to determine a BED. Pharmacokinetic and pharmacodynamic assays were validated prior to patient accrual. RESULTS: Twenty-five patients received 17-DMAG (range 2.5-106 mg/m(2)). At 106 mg/m(2) of 17-DMAG 2/4 patients experienced DLT, including one treatment-related death. No DLT occurred at 80 mg/m(2). Common adverse events were gastrointestinal, liver function changes, and ocular. Area under the curve and mean peak concentration increased proportionally with 17-DMAG doses 80 mg/m(2) or less. In peripheral blood mononuclear cells significant (P < 0.05) HSP72 induction was detected (≥ 20 mg/m(2)) and sustained for 96 hours (≥ 40 mg/m(2)). Plasma HSP72 levels were greatest in the two patients who experienced DLT. At 80 mg/m(2) client protein (CDK4, LCK) depletion was detected and tumor samples from 3 of 5 patients confirmed HSP90 inhibition. Clinical activity included complete response (castration refractory prostate cancer, CRPC 124 weeks), partial response (melanoma, 159 weeks), and stable disease (chondrosarcoma, CRPC, and renal cancer for 28, 59, and 76 weeks, respectively). CONCLUSIONS: The recommended phase II dose of 17-DMAG is 80 mg/m(2) weekly i.v. CI - ©2011 AACR. FAU - Pacey, Simon AU - Pacey S AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, Sutton, Surrey, United Kingdom. FAU - Wilson, Richard H AU - Wilson RH FAU - Walton, Mike AU - Walton M FAU - Eatock, Martin M AU - Eatock MM FAU - Hardcastle, Anthea AU - Hardcastle A FAU - Zetterlund, Anna AU - Zetterlund A FAU - Arkenau, Hendrik-Tobias AU - Arkenau HT FAU - Moreno-Farre, Javier AU - Moreno-Farre J FAU - Banerji, Udai AU - Banerji U FAU - Roels, Belle AU - Roels B FAU - Peachey, Heidi AU - Peachey H FAU - Aherne, Wynne AU - Aherne W FAU - de Bono, Johan S AU - de Bono JS FAU - Raynaud, Florence AU - Raynaud F FAU - Workman, Paul AU - Workman P FAU - Judson, Ian AU - Judson I LA - eng GR - C309/A8274/CRUK_/Cancer Research UK/United Kingdom GR - C212/A11342/CRUK_/Cancer Research UK/United Kingdom GR - C212/A7324/CRUK_/Cancer Research UK/United Kingdom GR - C212/A5720/CRUK_/Cancer Research UK/United Kingdom GR - CRUKD/06/052/CRUK_/Cancer Research UK/United Kingdom GR - A5847/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20110128 PL - United States TA - Clin Cancer Res JT - Clinical cancer research : an official journal of the American Association for Cancer Research JID - 9502500 RN - 0 (Benzoquinones) RN - 0 (HSP90 Heat-Shock Proteins) RN - 0 (Lactams, Macrocyclic) RN - 001L2FE0M3 (17-(dimethylaminoethylamino)-17-demethoxygeldanamycin) SB - IM EIN - Clin Cancer Res. 2018 Nov 1;24(21):5488. doi: 10.1158/1078-0432.CCR-18-2742. PMID: 30385660 MH - Adult MH - Aged MH - Benzoquinones/*pharmacology MH - Biopsy MH - Blotting, Western MH - Cohort Studies MH - Enzyme-Linked Immunosorbent Assay/methods MH - Female MH - HSP90 Heat-Shock Proteins/metabolism MH - Humans MH - Lactams, Macrocyclic/*pharmacology MH - Leukocytes, Mononuclear/cytology MH - Male MH - Maximum Tolerated Dose MH - Middle Aged MH - Neoplasms/*drug therapy PMC - PMC3060938 MID - UKMS33491 OID - NLM: UKMS33491 EDAT- 2011/02/01 06:00 MHDA- 2011/07/23 06:00 PMCR- 2011/09/01 CRDT- 2011/02/01 06:00 PHST- 2011/02/01 06:00 [entrez] PHST- 2011/02/01 06:00 [pubmed] PHST- 2011/07/23 06:00 [medline] PHST- 2011/09/01 00:00 [pmc-release] AID - 1078-0432.CCR-10-1927 [pii] AID - 10.1158/1078-0432.CCR-10-1927 [doi] PST - ppublish SO - Clin Cancer Res. 2011 Mar 15;17(6):1561-70. doi: 10.1158/1078-0432.CCR-10-1927. Epub 2011 Jan 28. PMID- 23378603 OWN - NLM STAT- MEDLINE DCOM- 20130903 LR - 20260518 IS - 1468-6244 (Electronic) IS - 0022-2593 (Linking) VI - 50 IP - 4 DP - 2013 Apr TI - CFTR p.Arg117His associated with CBAVD and other CFTR-related disorders. PG - 220-7 LID - 10.1136/jmedgenet-2012-101427 [doi] AB - BACKGROUND: The high frequency of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene mutation p.Arg117His in patients with congenital bilateral absence of the vas deferens (CBAVD) and in newborns screened for CF has created a dilemma. METHODS: Phenotypic and genotypic data were retrospectively collected in 179 non-newborn French individuals carrying p.Arg117His and a second CFTR mutation referred for symptoms or family history, by all French molecular genetics laboratories, referring physicians, CF care centres and infertility clinics. RESULTS: 97% of the patients had the intronic T7 normal variant in cis with p.Arg117His. 89% patients were male, with CBAVD being the reason for referral in 76%. In 166/179 patients with available detailed clinical features, final diagnoses were: four late-onset marked pulmonary disease, 83 isolated CBAVD, 67 other CFTR-related phenotypes, including 44 CBAVD with pulmonary and/or pancreatic symptoms and 12 asymptomatic cases. Respiratory symptoms were observed in 30% of the patients, but the overall phenotype was mild. No correlation was observed between sweat chloride concentrations and disease severity. Five couples at risk of CF offspring were identified and four benefited from prenatal or preimplantation genetic diagnoses (PND or PGD). Eight children were born, including four who were compound heterozygous for p.Arg117His and one with a severe CF mutation. CONCLUSIONS: Patients with CBAVD carrying p.Arg117His and a severe CF mutation should benefit from a clinical evaluation and follow-up. Depending on the CBAVD patients' genotype, a CFTR analysis should be considered in their partners in order to identify CF carrier couples and offer PND or PGD. FAU - Thauvin-Robinet, Christel AU - Thauvin-Robinet C AD - Centre de Génétique, Hôpital d'Enfants, 10 Bd du Maréchal de Lattre de Tassigny, Dijon cedex 21034, France. christel.thauvin@chu-dijon.fr FAU - Munck, Anne AU - Munck A FAU - Huet, Frédéric AU - Huet F FAU - de Becdelièvre, Alix AU - de Becdelièvre A FAU - Jimenez, Clément AU - Jimenez C FAU - Lalau, Guy AU - Lalau G FAU - Gautier, Elodie AU - Gautier E FAU - Rollet, Jacques AU - Rollet J FAU - Flori, Jean AU - Flori J FAU - Nové-Josserand, Raphaëlle AU - Nové-Josserand R FAU - Soufir, Jean-Claude AU - Soufir JC FAU - Haloun, Alain AU - Haloun A FAU - Hubert, Dominique AU - Hubert D FAU - Houssin, Elise AU - Houssin E FAU - Bellis, Gil AU - Bellis G FAU - Rault, Gilles AU - Rault G FAU - David, Albert AU - David A FAU - Janny, Laurent AU - Janny L FAU - Chiron, Raphaël AU - Chiron R FAU - Rives, Nathalie AU - Rives N FAU - Hairion, Dominique AU - Hairion D FAU - Collignon, Patrick AU - Collignon P FAU - Valeri, Antoine AU - Valeri A FAU - Karsenty, Gilles AU - Karsenty G FAU - Rossi, Annick AU - Rossi A FAU - Audrézet, Marie-Pierre AU - Audrézet MP FAU - Férec, Claude AU - Férec C FAU - Leclerc, Julie AU - Leclerc J FAU - Georges, Marie des AU - Georges Md FAU - Claustres, Mireille AU - Claustres M FAU - Bienvenu, Thierry AU - Bienvenu T FAU - Gérard, Bénédicte AU - Gérard B FAU - Boisseau, Pierre AU - Boisseau P FAU - Cabet-Bey, Faïza AU - Cabet-Bey F FAU - Cheillan, David AU - Cheillan D FAU - Feldmann, Delphine AU - Feldmann D FAU - Clavel, Christine AU - Clavel C FAU - Bieth, Eric AU - Bieth E FAU - Iron, Albert AU - Iron A FAU - Simon-Bouy, Brigitte AU - Simon-Bouy B FAU - Izard, Vincent AU - Izard V FAU - Steffann, Julie AU - Steffann J FAU - Viville, Stéphane AU - Viville S FAU - Costa, Catherine AU - Costa C FAU - Drouineaud, Véronique AU - Drouineaud V FAU - Fauque, Patricia AU - Fauque P FAU - Binquet, Christine AU - Binquet C FAU - Bonithon-Kopp, Claire AU - Bonithon-Kopp C FAU - Morris, Mike A AU - Morris MA FAU - Faivre, Laurence AU - Faivre L FAU - Goossens, Michel AU - Goossens M FAU - Roussey, Michel AU - Roussey M FAU - Girodon, Emmanuelle AU - Girodon E CN - collaborating working group on p.Arg117His LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130201 PL - England TA - J Med Genet JT - Journal of medical genetics JID - 2985087R RN - 0 (CFTR protein, human) RN - 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator) RN - Congenital bilateral aplasia of vas deferens SB - IM MH - Child MH - Child, Preschool MH - Cystic Fibrosis/complications/*genetics/pathology MH - Cystic Fibrosis Transmembrane Conductance Regulator/*genetics MH - Female MH - Heterozygote MH - Humans MH - Infant MH - Infant, Newborn MH - Infertility, Male/complications/genetics MH - Male MH - Male Urogenital Diseases/complications/*genetics/pathology MH - Mutation MH - Mutation Rate MH - Phenotype MH - *Prenatal Diagnosis MH - Sweat/chemistry MH - Vas Deferens/abnormalities/pathology FIR - Bazin, A IR - Bazin A FIR - Blayau, M IR - Blayau M FIR - Bonnefont, Jp IR - Bonnefont J FIR - Bouligand, J IR - Bouligand J FIR - Chéry, M IR - Chéry M FIR - Chevalier-Porst, F IR - Chevalier-Porst F FIR - Costa, Jm IR - Costa J FIR - Coude, M IR - Coude M FIR - Creveaux, I IR - Creveaux I FIR - Dalstein, V IR - Dalstein V FIR - Gerson, F IR - Gerson F FIR - Gobin-Limballe, S IR - Gobin-Limballe S FIR - Gouget, Am IR - Gouget A FIR - Kitzis, A IR - Kitzis A FIR - Lagier-Tourenne, C IR - Lagier-Tourenne C FIR - Magdelaine, C IR - Magdelaine C FIR - Malinge, Mc IR - Malinge M FIR - Malzac, P IR - Malzac P FIR - Mittre, H IR - Mittre H FIR - Petit, V IR - Petit V FIR - Philippe, C IR - Philippe C FIR - Ray, P IR - Ray P FIR - Raynaud, M IR - Raynaud M FIR - Ronsin, C IR - Ronsin C FIR - Schmitt, S IR - Schmitt S FIR - Albert, M IR - Albert M FIR - Bassinet, L IR - Bassinet L FIR - Bellon, G IR - Bellon G FIR - Bonnefoy, S IR - Bonnefoy S FIR - Bourouillou, G IR - Bourouillou G FIR - Bremont, F IR - Bremont F FIR - Brechard, Mp IR - Brechard M FIR - Chardot, C IR - Chardot C FIR - Chevalier, Mc IR - Chevalier M FIR - Chiesa, J IR - Chiesa J FIR - Ciolkovitch, A IR - Ciolkovitch A FIR - Clement, A IR - Clement A FIR - Corvol, H IR - Corvol H FIR - Counil, F IR - Counil F FIR - Costa, P IR - Costa P FIR - David, V IR - David V FIR - Delacourt, C IR - Delacourt C FIR - Delafontaine, D IR - Delafontaine D FIR - Delepoulle, F IR - Delepoulle F FIR - Delrue, Ma IR - Delrue M FIR - Deneuville, E IR - Deneuville E FIR - Derelle, J IR - Derelle J FIR - Desrues, B IR - Desrues B FIR - Dominique, S IR - Dominique S FIR - Fanton, Al IR - Fanton A FIR - Foucaud, P IR - Foucaud P FIR - Freour, T IR - Freour T FIR - Froment, S IR - Froment S FIR - Gaillard, D IR - Gaillard D FIR - Gérardin, M IR - Gérardin M FIR - Giacomini, P IR - Giacomini P FIR - Gambert, C IR - Gambert C FIR - Gautier, E IR - Gautier E FIR - Ginies, Jl IR - Ginies J FIR - Ginglinger, E IR - Ginglinger E FIR - Gottrand, F IR - Gottrand F FIR - Guichet, A IR - Guichet A FIR - Guillot, M IR - Guillot M FIR - Heraud, Mc IR - Heraud M FIR - Houriez-Bertolo, E IR - Houriez-Bertolo E FIR - Jeandidier, E IR - Jeandidier E FIR - Journel, H IR - Journel H FIR - Labarière IR - Labarière FIR - Lahsinat, K IR - Lahsinat K FIR - Langlais, S IR - Langlais S FIR - Languepin, J IR - Languepin J FIR - Laurans, M IR - Laurans M FIR - Lauton, D IR - Lauton D FIR - Layet, V IR - Layet V FIR - Le Bourgeois, M IR - Le Bourgeois M FIR - Le Lannou, D IR - Le Lannou D FIR - Lejeune, H IR - Lejeune H FIR - Lenoir, G IR - Lenoir G FIR - Leroy, S IR - Leroy S FIR - Lestrade, F IR - Lestrade F FIR - Llerena, C IR - Llerena C FIR - Marc, B IR - Marc B FIR - Marchand, S IR - Marchand S FIR - Marguet, C IR - Marguet C FIR - Marteletti, O IR - Marteletti O FIR - Massat, G IR - Massat G FIR - Masurel-Paulet, A IR - Masurel-Paulet A FIR - Mely, L IR - Mely L FIR - Menetrey, C IR - Menetrey C FIR - Moisan-Petit, V IR - Moisan-Petit V FIR - Montcouquiol, S IR - Montcouquiol S FIR - Moreau, L IR - Moreau L FIR - Odent, S IR - Odent S FIR - Pagenault, M IR - Pagenault M FIR - Parent, P IR - Parent P FIR - Pautard, J C IR - Pautard JC FIR - Perez-Martin, S IR - Perez-Martin S FIR - Peter, M O IR - Peter MO FIR - Pierre, D IR - Pierre D FIR - Pin, I IR - Pin I FIR - Plessis, G IR - Plessis G FIR - Ramel, S IR - Ramel S FIR - Rembert-Sagot, F IR - Rembert-Sagot F FIR - Roux, C IR - Roux C FIR - Royere, D IR - Royere D FIR - Sardet, A IR - Sardet A FIR - Sarles, J IR - Sarles J FIR - Sermet-Gaudelus, I IR - Sermet-Gaudelus I FIR - Siffroi, J P IR - Siffroi JP FIR - Saulnier, J P IR - Saulnier JP FIR - Sehabiague, J IR - Sehabiague J FIR - Sinet, P M IR - Sinet PM FIR - Tassin, E IR - Tassin E FIR - Terro, F IR - Terro F FIR - Turck, D IR - Turck D FIR - Vodoff, M V IR - Vodoff MV FIR - Wagner, L IR - Wagner L FIR - Weiss, L IR - Weiss L EDAT- 2013/02/05 06:00 MHDA- 2013/09/04 06:00 CRDT- 2013/02/05 06:00 PHST- 2013/02/05 06:00 [entrez] PHST- 2013/02/05 06:00 [pubmed] PHST- 2013/09/04 06:00 [medline] AID - jmedgenet-2012-101427 [pii] AID - 10.1136/jmedgenet-2012-101427 [doi] PST - ppublish SO - J Med Genet. 2013 Apr;50(4):220-7. doi: 10.1136/jmedgenet-2012-101427. Epub 2013 Feb 1. PMID- 34750160 OWN - NLM STAT- MEDLINE DCOM- 20220308 LR - 20230203 IS - 1555-905X (Electronic) IS - 1555-9041 (Print) IS - 1555-9041 (Linking) VI - 16 IP - 11 DP - 2021 Nov TI - Skeletal Muscle Phenotype in Patients Undergoing Long-Term Hemodialysis Awaiting Kidney Transplantation. PG - 1676-1685 LID - 10.2215/CJN.02390221 [doi] AB - BACKGROUND AND OBJECTIVES: Age and comorbidity-related sarcopenia represent a main cause of muscle dysfunction in patients on long-term hemodialysis. However, recent findings suggest muscle abnormalities that are not associated with sarcopenia. The aim of this study was to isolate functional and cellular muscle abnormalities independently of other major confounding factors, including malnutrition, age, comorbidity, or sedentary lifestyle, which are common in patients on maintenance hemodialysis. To overcome these confounding factors, alterations in skeletal muscle were analyzed in highly selected patients on long-term hemodialysis undergoing kidney transplantation. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In total, 22 patients on long-term hemodialysis scheduled for kidney transplantation with few comorbidities, but with a long-term uremic milieu exposure, and 22 age, sex, and physical activity level frequency-matched control participants were recruited. We compared biochemical, functional, and molecular characteristics of the skeletal muscle using maximal voluntary force and endurance of the quadriceps, 6-minute walking test, and muscle biopsy of vastus lateralis. For statistical analysis, mean comparison and multiple regression tests were used. RESULTS: In patients on long-term hemodialysis, muscle endurance was lower, whereas maximal voluntary force was not significantly different. We observed a transition from type I (oxidative) to type II (glycolytic) muscle fibers, and an alteration of mitochondrial structure (swelling) without changes in DNA content, genome replication (peroxisome proliferator activator receptor γ coactivator-1α and mitochondrial transcription factor A), regulation of fusion (mitofusin and optic atrophy 1), or fission (dynamin-related protein 1). Notably, there were autophagosome structures containing glycogen along with mitochondrial debris, with a higher expression of light chain 3 (LC3) protein, indicating phagophore formation. This was associated with a greater conversion of LC3-I to LC3-II and the expression of Gabaralp1 and Bnip3l genes involved in mitophagy. CONCLUSIONS: In this highly selected long-term hemodialysis population, a low oxidative phenotype could be defined by a poor endurance, a fiber-type switch, and an alteration of mitochondria structure, without evidence of sarcopenia. This phenotype could be related to uremia through the activation of autophagy/mitophagy. CLINICAL TRIAL REGISTRATION NUMBERS: NCT02794142 and NCT02040363. CI - Copyright © 2021 by the American Society of Nephrology. FAU - Souweine, Jean-Sébastien AU - Souweine JS AD - Department of Biochemistry, University Hospital of Montpellier and Department of Biochemistry and Hormonology, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. FAU - Gouzi, Fares AU - Gouzi F AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, Montpellier, France. FAU - Badia, Éric AU - Badia É AUID- ORCID: 0000-0002-8922-9834 AD - Department of Biochemistry, University Hospital of Montpellier and Department of Biochemistry and Hormonology, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. FAU - Pomies, Pascal AU - Pomies P AD - Department of Physiology, University Hospital of Montpellier, Montpellier, France. FAU - Garrigue, Valérie AU - Garrigue V AD - Department of Nephrology, University Hospital of Montpellier, Montpellier, France. FAU - Morena, Marion AU - Morena M AD - Department of Biochemistry, University Hospital of Montpellier and Department of Biochemistry and Hormonology, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. FAU - Hayot, Maurice AU - Hayot M AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, Montpellier, France. FAU - Mercier, Jacques AU - Mercier J AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, Montpellier, France. FAU - Ayoub, Bronia AU - Ayoub B AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. AD - Department of Physiology, University Hospital of Montpellier, Montpellier, France. FAU - Quintrec, Moglie Le AU - Quintrec ML AD - Department of Nephrology, University Hospital of Montpellier, Montpellier, France. FAU - Raynaud, Fabrice AU - Raynaud F AD - Department of Biochemistry, University Hospital of Montpellier and Department of Biochemistry and Hormonology, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. FAU - Cristol, Jean-Paul AU - Cristol JP AD - Department of Biochemistry, University Hospital of Montpellier and Department of Biochemistry and Hormonology, Montpellier, France. AD - PhyMedExp, University of Montpellier, INSERM, Montpellier, France. LA - eng SI - ClinicalTrials.gov/NCT02794142 SI - ClinicalTrials.gov/NCT02040363 PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin J Am Soc Nephrol JT - Clinical journal of the American Society of Nephrology : CJASN JID - 101271570 RN - 0 (Adaptor Proteins, Signal Transducing) RN - 0 (BNIP3L protein, human) RN - 0 (GABARAPL1 protein, human) RN - 0 (MAP1LC3A protein, human) RN - 0 (MAP1LC3B protein, human) RN - 0 (Membrane Proteins) RN - 0 (Microtubule-Associated Proteins) RN - 0 (Proto-Oncogene Proteins) RN - 0 (Tumor Suppressor Proteins) SB - IM CIN - Clin J Am Soc Nephrol. 2021 Nov;16(11):1613-1614. doi: 10.2215/CJN.12550921. PMID: 34750157 MH - Adaptor Proteins, Signal Transducing/genetics MH - Autophagosomes/pathology MH - Biopsy MH - Case-Control Studies MH - Female MH - Humans MH - Kidney Transplantation MH - Male MH - Membrane Proteins/genetics MH - Microtubule-Associated Proteins/genetics/metabolism MH - Middle Aged MH - Mitochondria/pathology MH - Mitophagy MH - Muscle Fibers, Skeletal/metabolism/*pathology MH - Muscle Strength MH - Phenotype MH - Physical Endurance MH - Proto-Oncogene Proteins/genetics MH - Quadriceps Muscle/*pathology/*physiopathology MH - *Renal Dialysis MH - Signal Transduction MH - Time Factors MH - Tumor Suppressor Proteins/genetics MH - Waiting Lists MH - Walk Test PMC - PMC8729424 OTO - NOTNLM OT - cell signaling OT - chronic hemodialysis OT - chronic kidney disease OT - mitochondria OT - phenotype OT - signal transduction OT - transplantation EDAT- 2021/11/10 06:00 MHDA- 2022/03/09 06:00 PMCR- 2022/11/01 CRDT- 2021/11/09 06:13 PHST- 2021/02/16 00:00 [received] PHST- 2021/09/08 00:00 [accepted] PHST- 2021/11/09 06:13 [entrez] PHST- 2021/11/10 06:00 [pubmed] PHST- 2022/03/09 06:00 [medline] PHST- 2022/11/01 00:00 [pmc-release] AID - 01277230-202111000-00010 [pii] AID - 02390221 [pii] AID - 10.2215/CJN.02390221 [doi] PST - ppublish SO - Clin J Am Soc Nephrol. 2021 Nov;16(11):1676-1685. doi: 10.2215/CJN.02390221. PMID- 39450752 OWN - NLM STAT- MEDLINE DCOM- 20250219 LR - 20250521 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 392 IP - 8 DP - 2025 Feb 20 TI - Microvascular Inflammation of Kidney Allografts and Clinical Outcomes. PG - 763-776 LID - 10.1056/NEJMoa2408835 [doi] AB - BACKGROUND: The heterogeneous clinical presentation of graft microvascular inflammation poses a major challenge to successful kidney transplantation. The effect of microvascular inflammation on allograft outcomes is unclear. METHODS: We conducted a cohort study that included kidney-transplant recipients from more than 30 transplantation centers in Europe and North America who had undergone allograft biopsy between 2004 and 2023. We integrated clinical and pathological data to classify biopsy specimens according to the 2022 Banff Classification of Renal Allograft Pathology, which includes two new diagnostic categories: probable antibody-mediated rejection and microvascular inflammation without evidence of an antibody-mediated response. We then assessed the association between the newly recognized microvascular inflammation phenotypes and allograft survival and disease progression. RESULTS: A total of 16,293 kidney-transplant biopsy specimens from 6798 patients were assessed. We identified the newly recognized microvascular inflammation phenotypes in 788 specimens, of which 641 were previously categorized as specimens with no evidence of rejection. As compared with patients without rejection, the hazard ratio for graft loss was 2.1 (95% confidence interval [CI], 1.5 to 3.1) among patients with microvascular inflammation without evidence of an antibody-mediated response and 2.7 (95% CI, 2.2 to 3.3) among patients with antibody-mediated rejection. Patients with a diagnosis of probable antibody-mediated rejection had a higher risk of graft failure beyond year 5 after biopsy than those without rejection (hazard ratio, 1.7; 95% CI, 0.8 to 3.5). Patients with a diagnosis of either newly recognized microvascular inflammation phenotype had a higher risk of progression of transplant glomerulopathy during follow-up than patients without microvascular inflammation. CONCLUSIONS: Microvascular inflammation in kidney allografts includes distinct phenotypes, with various disease progression and allograft outcomes. Our findings support the clinical use of additional rejection phenotypes to standardize diagnostics for kidney allografts. (Funded by OrganX. ClinicalTrials.gov number, NCT06496269.). CI - Copyright © 2024 Massachusetts Medical Society. FAU - Sablik, Marta AU - Sablik M AUID- ORCID: 0009-0007-4662-4010 AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. FAU - Sannier, Aurélie AU - Sannier A AUID- ORCID: 0000-0003-3973-8793 AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Department of Pathology, Bichat Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris. FAU - Raynaud, Marc AU - Raynaud M AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. FAU - Goutaudier, Valentin AU - Goutaudier V AUID- ORCID: 0000-0001-6191-6889 AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. FAU - Divard, Gillian AU - Divard G AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Kidney Transplant Department, Saint-Louis Hospital, AP-HP, Paris. FAU - Astor, Brad C AU - Astor BC AD - Division of Nephrology, Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison. FAU - Weng, Patricia AU - Weng P AD - Pediatric Nephrology, David Geffen School of Medicine at UCLA, UCLA Mattel Children's Hospital, Los Angeles. FAU - Smith, Jodi AU - Smith J AD - Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, Seattle. FAU - Garro, Rouba AU - Garro R AD - Division of Pediatric Nephrology, Emory University School of Medicine, Children's Pediatric Institute, Atlanta. FAU - Warady, Bradley A AU - Warady BA AD - Division of Pediatric Nephrology, University of Kansas City, Children's Mercy Hospital, Kansas City, MO. FAU - Zahr, Rima S AU - Zahr RS AD - Division of Pediatric Nephrology and Hypertension, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis. FAU - Twombley, Katherine AU - Twombley K AD - Acute Dialysis Units, Pediatric Kidney Transplant, Medical University of South Carolina, Charleston. FAU - Dharnidharka, Vikas R AU - Dharnidharka VR AD - Division of Pediatric Nephrology, Hypertension, and Apheresis, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis. AD - Department of Pediatrics, Robert Wood Johnson Medical School at Rutgers University, New Brunswick, NJ. FAU - Dandamudi, Raja S AU - Dandamudi RS AD - Division of Pediatric Nephrology, Hypertension, and Apheresis, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis. FAU - Fila, Marc AU - Fila M AUID- ORCID: 0000-0001-8857-7100 AD - Department of Pediatric Nephrology, Centre Hospitalier Universitaire (CHU) Montpellier, Montpellier, France. FAU - Huang, Edmund AU - Huang E AD - Cedars-Sinai Comprehensive Transplant Center, Los Angeles. FAU - Sellier-Leclerc, Anne-Laure AU - Sellier-Leclerc AL AD - Pediatric Nephrology Department, Hôpital Universitaire Mère-Enfant, Hospices Civils de Lyon (HCL), Lyon, France. FAU - Tönshoff, Burkhard AU - Tönshoff B AD - Department of Pediatrics I, University Children Hospital Heidelberg, Heidelberg, Germany. FAU - Rabant, Marion AU - Rabant M AD - Department of Pathology, Necker Hospital, AP-HP, Paris. FAU - Verine, Jérôme AU - Verine J AD - Department of Pathology, Saint-Louis Hospital, AP-HP, Paris. FAU - Del Bello, Arnaud AU - Del Bello A AD - Department of Nephrology-Dialysis-Transplantation, CHU de Toulouse, Toulouse, France. FAU - Berney, Thierry AU - Berney T AD - Division of Abdominal and Transplantation Surgery, Department of Surgery, Faculty of Medicine, Geneva University Hospitals, Geneva. FAU - Boyer, Olivia AU - Boyer O AUID- ORCID: 0000-0002-3957-1359 AD - Division of Pediatric Nephrology, Necker Hospital, AP-HP, Université Paris Cité, Paris. FAU - Catar, Rusan Ali AU - Catar RA AD - Department of Nephrology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Freie Universität Berlin, Berlin Institute of Health, Berlin. FAU - Danger, Richard AU - Danger R AD - Nantes Université, CHU Nantes, INSERM, Center for Research in Transplantation and Translational Immunology, Unité Mixte de Recherche 1064, Institute of Urology-Nephrology Transplantation of the University Hospital of Nantes, Nantes, France. FAU - Giral, Magali AU - Giral M AD - Nantes Université, CHU Nantes, INSERM, Center for Research in Transplantation and Translational Immunology, Unité Mixte de Recherche 1064, Institute of Urology-Nephrology Transplantation of the University Hospital of Nantes, Nantes, France. FAU - Yoo, Daniel AU - Yoo D AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. FAU - Girardin, François R AU - Girardin FR AD - Division of Clinical Pharmacology, Department of Medicine and Department of Laboratory Medicine and Pathology, Lausanne University Hospital, Faculty of Medicine, University of Lausanne, Lausanne, Switzerland. FAU - Alsadi, Alaa AU - Alsadi A AD - Department of Pathology, University of Wisconsin, Madison. FAU - Gourraud, Pierre-Antoine AU - Gourraud PA AD - Nantes Université, CHU Nantes, INSERM, Center for Research in Transplantation and Translational Immunology, Unité Mixte de Recherche 1064, Institute of Urology-Nephrology Transplantation of the University Hospital of Nantes, Nantes, France. FAU - Morelon, Emmanuel AU - Morelon E AD - Department of Transplantation, Edouard Herriot University Hospital, HCL, University of Lyon I, Lyon, France. FAU - Le Quintrec, Moglie AU - Le Quintrec M AD - Department of Nephrology, CHU Montpellier, Montpellier, France. FAU - Try, Mélanie AU - Try M AD - Department of Kidney Transplantation, Necker Hospital, AP-HP, Paris. FAU - Villard, Jean AU - Villard J AD - Division of Transplantation Immunology, University Hospital of Geneva, Geneva. FAU - Zhong, Weixiong AU - Zhong W AD - Nantes Université, CHU Nantes, INSERM, Center for Research in Transplantation and Translational Immunology, Unité Mixte de Recherche 1064, Institute of Urology-Nephrology Transplantation of the University Hospital of Nantes, Nantes, France. FAU - Bestard, Oriol AU - Bestard O AD - Department of Nephrology and Kidney Transplantation, Vall d'Hebrón University Hospital, Barcelona. FAU - Budde, Klemens AU - Budde K AD - Department of Nephrology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Freie Universität Berlin, Berlin Institute of Health, Berlin. FAU - Chauveau, Bertrand AU - Chauveau B AD - Department of Pathology, CHU Bordeaux, Bordeaux, France. FAU - Couzi, Lionel AU - Couzi L AD - Department of Nephrology, Transplantation, Dialysis and Apheresis, CHU Bordeaux, Bordeaux, France. FAU - Brouard, Sophie AU - Brouard S AD - Nantes Université, CHU Nantes, INSERM, Center for Research in Transplantation and Translational Immunology, Unité Mixte de Recherche 1064, Institute of Urology-Nephrology Transplantation of the University Hospital of Nantes, Nantes, France. FAU - Hogan, Julien AU - Hogan J AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Division of Pediatric Nephrology, Robert Debré Hospital, AP-HP, Paris. FAU - Legendre, Christophe AU - Legendre C AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Department of Kidney Transplantation, Necker Hospital, AP-HP, Paris. FAU - Anglicheau, Dany AU - Anglicheau D AD - Department of Kidney Transplantation, Necker Hospital, AP-HP, Paris. FAU - Aubert, Olivier AU - Aubert O AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Department of Kidney Transplantation, Necker Hospital, AP-HP, Paris. FAU - Kamar, Nassim AU - Kamar N AUID- ORCID: 0000-0003-1930-8964 AD - Department of Nephrology-Dialysis-Transplantation, CHU de Toulouse, Toulouse, France. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Kidney Transplant Department, Saint-Louis Hospital, AP-HP, Paris. FAU - Loupy, Alexandre AU - Loupy A AUID- ORCID: 0000-0003-3388-7747 AD - Université Paris Cité, INSERM Unité 970, Paris Institute for Transplantation and Organ Regeneration, Paris. AD - Department of Kidney Transplantation, Necker Hospital, AP-HP, Paris. LA - eng SI - ClinicalTrials.gov/NCT06496269 GR - 754995/European Commission/ GR - ANR-17-RHUS-0010/Agence Nationale de la Recherche/ PT - Journal Article PT - Multicenter Study PT - Observational Study DEP - 20241024 PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM CIN - Transpl Int. 2024 Nov 26;37:14032. doi: 10.3389/ti.2024.14032. PMID: 39659965 MH - Adult MH - Female MH - Humans MH - Male MH - Middle Aged MH - *Allografts/blood supply/immunology/pathology MH - Biopsy MH - Cohort Studies MH - Disease Progression MH - *Graft Rejection/diagnosis/immunology/pathology MH - Graft Survival MH - *Inflammation/diagnosis/pathology MH - *Kidney/blood supply/immunology/pathology MH - *Kidney Transplantation/adverse effects MH - *Microvessels/immunology/pathology MH - Phenotype MH - Child MH - Adolescent MH - Young Adult EDAT- 2024/10/25 12:23 MHDA- 2025/02/19 18:20 CRDT- 2024/10/25 07:22 PHST- 2025/02/19 18:20 [medline] PHST- 2024/10/25 12:23 [pubmed] PHST- 2024/10/25 07:22 [entrez] AID - 10.1056/NEJMoa2408835 [doi] PST - ppublish SO - N Engl J Med. 2025 Feb 20;392(8):763-776. doi: 10.1056/NEJMoa2408835. Epub 2024 Oct 24. PMID- 24740809 OWN - NLM STAT- MEDLINE DCOM- 20140812 LR - 20260518 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 123 IP - 24 DP - 2014 Jun 12 TI - Oncogenetics and minimal residual disease are independent outcome predictors in adult patients with acute lymphoblastic leukemia. PG - 3739-49 LID - 10.1182/blood-2014-01-547695 [doi] AB - With intensified pediatric-like therapy and genetic disease dissection, the field of adult acute lymphoblastic leukemia (ALL) has evolved recently. In this new context, we aimed to reassess the value of conventional risk factors with regard to new genetic alterations and early response to therapy, as assessed by immunoglobulin/T-cell receptor minimal residual disease (MRD) levels. The study was performed in 423 younger adults with Philadelphia chromosome-negative ALL in first remission (265 B-cell precursor [BCP] and 158 T-cell ALL), with cumulative incidence of relapse (CIR) as the primary end point. In addition to conventional risk factors, the most frequent currently available genetic alterations were included in the analysis. A higher specific hazard of relapse was independently associated with postinduction MRD level ≥10(-4) and unfavorable genetic characteristics (ie, MLL gene rearrangement or focal IKZF1 gene deletion in BCP-ALL and no NOTCH1/FBXW7 mutation and/or N/K-RAS mutation and/or PTEN gene alteration in T-cell ALL). These 2 factors allowed definition of a new risk classification that is strongly associated with higher CIR and shorter relapse-free and overall survival. These results indicate that genetic abnormalities are important predictors of outcome in adult ALL not fully recapitulated by early response to therapy. Patients included in this study were treated in the multicenter GRAALL-2003 and GRAALL-2005 trials. Both trials were registered at http://www.clinicaltrials.gov as #NCT00222027 and #NCT00327678, respectively. CI - © 2014 by The American Society of Hematology. FAU - Beldjord, Kheira AU - Beldjord K AD - Department of Hematology and. FAU - Chevret, Sylvie AU - Chevret S AD - Department of Biostatistics, University Paris Diderot, Institut Universitaire d'Hématologie, EA-3518 and UMR-S-717, University Hospital Saint-Louis, Assistance Publique - Hôpitaux de Paris, Paris, France; FAU - Asnafi, Vahid AU - Asnafi V AD - Laboratory of Oncohematology, AP-HP, Hôpital Necker Enfants-Malades, University Paris Descartes Sorbonne Cité, Institut Necker-Enfants Malades, Institut National de Recherche Médicale U1151, Paris, France; FAU - Huguet, Françoise AU - Huguet F AD - Department of Hematology, University Hospital Purpan, Toulouse, France; FAU - Boulland, Marie-Laure AU - Boulland ML AD - Department of Hematology, University Hospital, Rennes, France; FAU - Leguay, Thibaut AU - Leguay T AD - Department of Hematology, University Hospital Haut-Lévèque, Bordeaux, France; FAU - Thomas, Xavier AU - Thomas X AD - Department of Hematology, University Hospital Lyon Sud, Lyon, France; FAU - Cayuela, Jean-Michel AU - Cayuela JM AD - Department of Hematology and. FAU - Grardel, Nathalie AU - Grardel N AD - Department of Hematology, University Hospital Claude Huriez, Lille, France; FAU - Chalandon, Yves AU - Chalandon Y AD - Department of Hematology, University Hospital, Geneva, Switzerland; FAU - Boissel, Nicolas AU - Boissel N AD - Department of Hematology and. FAU - Schaefer, Beat AU - Schaefer B AD - Department of Hematology, University Hospital, Zürich, Switzerland; FAU - Delabesse, Eric AU - Delabesse E AD - Department of Hematology, University Hospital Purpan, Toulouse, France; FAU - Cavé, Hélène AU - Cavé H AD - Department of Hematology, University Hospital Robert Debré, Assistance Publique - Hôpitaux de Paris, Paris, France; FAU - Chevallier, Patrice AU - Chevallier P AD - Department of Hematology, University Hospital, Nantes, France; FAU - Buzyn, Agnès AU - Buzyn A AD - Laboratory of Oncohematology, AP-HP, Hôpital Necker Enfants-Malades, University Paris Descartes Sorbonne Cité, Institut Necker-Enfants Malades, Institut National de Recherche Médicale U1151, Paris, France; FAU - Fest, Thierry AU - Fest T AD - Department of Hematology, University Hospital, Rennes, France; FAU - Reman, Oumedaly AU - Reman O AD - Department of Hematology, University Hospital, Caen, France; FAU - Vernant, Jean-Paul AU - Vernant JP AD - University Hospital Pitié-Salpêtrière, Assistance Publique - Hôpitaux de Paris, Paris, France; FAU - Lhéritier, Véronique AU - Lhéritier V AD - GRAALL Coordination Office, University Hospital Lyon Sud, Lyon, France; FAU - Béné, Marie C AU - Béné MC AD - Department of Hematology, University Hospital, Nantes, France; FAU - Lafage, Marina AU - Lafage M AD - Department of Genetics, Aix-Marseille University, Marseille, France; and. FAU - Macintyre, Elizabeth AU - Macintyre E AD - Laboratory of Oncohematology, AP-HP, Hôpital Necker Enfants-Malades, University Paris Descartes Sorbonne Cité, Institut Necker-Enfants Malades, Institut National de Recherche Médicale U1151, Paris, France; FAU - Ifrah, Norbert AU - Ifrah N AD - Department of Hematology, University Hospital, INSERM U892/CNRS 6299, Angers, France. FAU - Dombret, Hervé AU - Dombret H AD - Department of Hematology and. CN - Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL) LA - eng SI - ClinicalTrials.gov/NCT00222027 SI - ClinicalTrials.gov/NCT00327678 PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140416 PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (Biomarkers, Tumor) SB - IM MH - Adolescent MH - Adult MH - Biomarkers, Tumor/*genetics MH - DNA Mutational Analysis MH - Female MH - Humans MH - Male MH - Middle Aged MH - Multicenter Studies as Topic MH - Neoplasm, Residual MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/genetics/mortality/pathology MH - Prognosis MH - Recurrence MH - Retrospective Studies MH - Survival Analysis MH - Young Adult FIR - Caillères IR - Caillères FIR - Chaib IR - Chaib FIR - Blanc IR - Blanc FIR - Brunel IR - Brunel FIR - Da Silva IR - Da Silva FIR - Cuvelier IR - Cuvelier FIR - Marolleau IR - Marolleau FIR - Damaj IR - Damaj FIR - Vaida IR - Vaida FIR - Royer IR - Royer FIR - Gruson IR - Gruson FIR - Merlusca IR - Merlusca FIR - Copin IR - Copin FIR - Capiod IR - Capiod FIR - Dubus IR - Dubus FIR - Mpari IR - Mpari FIR - Hunault IR - Hunault FIR - Ifrah IR - Ifrah FIR - Schmidt IR - Schmidt FIR - Dib IR - Dib FIR - Francois IR - Francois FIR - Moles IR - Moles FIR - Foussard IR - Foussard FIR - Guardiola IR - Guardiola FIR - Zandecki IR - Zandecki FIR - Blanchet IR - Blanchet FIR - Baranger IR - Baranger FIR - Chassevent IR - Chassevent FIR - Genevieve IR - Genevieve FIR - Marie IR - Marie FIR - Parry IR - Parry FIR - Orsini-Piocelle IR - Orsini-Piocelle FIR - Corront IR - Corront FIR - Daguindau IR - Daguindau FIR - Cony-Makhoul IR - Cony-Makhoul FIR - Reynes IR - Reynes FIR - Cadoux IR - Cadoux FIR - Vittet IR - Vittet FIR - Sutton IR - Sutton FIR - Al Jijakli IR - Al Jijakli FIR - Genet IR - Genet FIR - Chaoui IR - Chaoui FIR - Mesbah IR - Mesbah FIR - Morel IR - Morel FIR - Touahri IR - Touahri FIR - Mossafa IR - Mossafa FIR - Fourcade IR - Fourcade FIR - Guerekobaya IR - Guerekobaya FIR - Zerazhi IR - Zerazhi FIR - Azzedine IR - Azzedine FIR - Boulat IR - Boulat FIR - Lepeu IR - Lepeu FIR - Touchais IR - Touchais FIR - Derre IR - Derre FIR - Beyrne IR - Beyrne FIR - Araujo IR - Araujo FIR - Bauduer IR - Bauduer FIR - Banos IR - Banos FIR - Burtin IR - Burtin FIR - Renoux IR - Renoux FIR - Menard IR - Menard FIR - Labarrère IR - Labarrère FIR - Legrand IR - Legrand FIR - Berceanu IR - Berceanu FIR - Daguindau IR - Daguindau FIR - Deconinck IR - Deconinck FIR - Larosa IR - Larosa FIR - Ferrand IR - Ferrand FIR - Brion IR - Brion FIR - Collonge-Rame IR - Collonge-Rame FIR - Garnache Ottou IR - Garnache Ottou FIR - Peria IR - Peria FIR - Rodon IR - Rodon FIR - Elyamadi IR - Elyamadi FIR - Kadiri IR - Kadiri FIR - Gardin IR - Gardin FIR - Ades IR - Ades FIR - Fenaux IR - Fenaux FIR - Braun IR - Braun FIR - Boulalam IR - Boulalam FIR - Eclache IR - Eclache FIR - Letestu IR - Letestu FIR - Nloga IR - Nloga FIR - Beve IR - Beve FIR - Leguay IR - Leguay FIR - Milpied IR - Milpied FIR - Lascaux IR - Lascaux FIR - Dilhuydy IR - Dilhuydy FIR - Dimicoli IR - Dimicoli FIR - Pigneux IR - Pigneux FIR - Tabrizi IR - Tabrizi FIR - Dumas IR - Dumas FIR - Lacombe IR - Lacombe FIR - Lippert IR - Lippert FIR - Boiron IR - Boiron FIR - Bilhou-Nabera IR - Bilhou-Nabera FIR - Marit IR - Marit FIR - Sauvezie IR - Sauvezie FIR - Perry IR - Perry FIR - Choufi IR - Choufi FIR - Kadiata IR - Kadiata FIR - Barry IR - Barry FIR - Voronina IR - Voronina FIR - Brument IR - Brument FIR - Nouvel IR - Nouvel FIR - Guillerm IR - Guillerm FIR - Berthou IR - Berthou FIR - Dalbies IR - Dalbies FIR - Tempescul IR - Tempescul FIR - Ianotto IR - Ianotto FIR - Couturier IR - Couturier FIR - Eveillard IR - Eveillard FIR - Ugo IR - Ugo FIR - De Braekeleer IR - De Braekeleer FIR - Le Calvez IR - Le Calvez FIR - Gillet IR - Gillet FIR - Reman IR - Reman FIR - Leporrier IR - Leporrier FIR - Chantepie IR - Chantepie FIR - Johnson IR - Johnson FIR - Ansah IR - Ansah FIR - Gac IR - Gac FIR - Macro IR - Macro FIR - Benabed IR - Benabed FIR - Cheze IR - Cheze FIR - Naguib IR - Naguib FIR - Plessis IR - Plessis FIR - Salaun IR - Salaun FIR - Lepesant IR - Lepesant FIR - Marin IR - Marin FIR - Malfuson IR - Malfuson FIR - Konopacki IR - Konopacki FIR - Souleau IR - Souleau FIR - De Revel IR - De Revel FIR - Fagot IR - Fagot FIR - Foissaud IR - Foissaud FIR - Samson IR - Samson FIR - Desangles IR - Desangles FIR - Harrouche IR - Harrouche FIR - Cacheux IR - Cacheux FIR - Bay IR - Bay FIR - Tournilhac IR - Tournilhac FIR - Hermet IR - Hermet FIR - Guieze IR - Guieze FIR - Bailly IR - Bailly FIR - Chaleteix IR - Chaleteix FIR - De Renzis IR - De Renzis FIR - Chabrot IR - Chabrot FIR - Calvet IR - Calvet FIR - Sapin IR - Sapin FIR - Tchirkov IR - Tchirkov FIR - Périssel IR - Périssel FIR - Veronese IR - Veronese FIR - Chassagne Berger IR - Chassagne Berger FIR - Villemagne IR - Villemagne FIR - Latiere IR - Latiere FIR - Giollant IR - Giollant FIR - Roy IR - Roy FIR - Auduy IR - Auduy FIR - Raby IR - Raby FIR - Kohser IR - Kohser FIR - Humbrecht IR - Humbrecht FIR - Barats IR - Barats FIR - Husseini IR - Husseini FIR - Moskovtchenko IR - Moskovtchenko FIR - Mazurier IR - Mazurier FIR - Bauly IR - Bauly FIR - Camara IR - Camara FIR - Salanoubat IR - Salanoubat FIR - Haiat IR - Haiat FIR - Petitdidier IR - Petitdidier FIR - Cereja IR - Cereja FIR - Joly IR - Joly FIR - Devidas IR - Devidas FIR - Quillet IR - Quillet FIR - Boutant IR - Boutant FIR - Maury IR - Maury FIR - Cordonnier IR - Cordonnier FIR - Pautas IR - Pautas FIR - Toma IR - Toma FIR - Hichri IR - Hichri FIR - Kuentz IR - Kuentz FIR - Bories IR - Bories FIR - Jouault IR - Jouault FIR - Wagner-Ballon IR - Wagner-Ballon FIR - Perot IR - Perot FIR - Plonquet IR - Plonquet FIR - Beaune IR - Beaune FIR - Reyes IR - Reyes FIR - Cabanne IR - Cabanne FIR - Caillot IR - Caillot FIR - Lafon IR - Lafon FIR - Casasnovas IR - Casasnovas FIR - Ferrant IR - Ferrant FIR - Bastié IR - Bastié FIR - Teyssier IR - Teyssier FIR - Mugneret IR - Mugneret FIR - Menadier IR - Menadier FIR - Favre-Audry IR - Favre-Audry FIR - Grandjean IR - Grandjean FIR - Pignon IR - Pignon FIR - Bemba IR - Bemba FIR - Wetterwald IR - Wetterwald FIR - Roumier IR - Roumier FIR - Paquez IR - Paquez FIR - Cahn IR - Cahn FIR - Thiebaut IR - Thiebaut FIR - Gressin IR - Gressin FIR - Bulabois IR - Bulabois FIR - Simon IR - Simon FIR - Pégourie IR - Pégourie FIR - Courby IR - Courby FIR - Molina IR - Molina FIR - Callanan IR - Callanan FIR - Lefebvre IR - Lefebvre FIR - Jacob IR - Jacob FIR - Garban IR - Garban FIR - Rolland IR - Rolland FIR - Leroux IR - Leroux FIR - Pittet IR - Pittet FIR - Agapé IR - Agapé FIR - Belkaid IR - Belkaid FIR - Henni IR - Henni FIR - Berg IR - Berg FIR - Pequin IR - Pequin FIR - Stalnikiewicz IR - Stalnikiewicz FIR - Dupriez IR - Dupriez FIR - Morel IR - Morel FIR - Poulain IR - Poulain FIR - Declercq IR - Declercq FIR - Berthon IR - Berthon FIR - Quesnel IR - Quesnel FIR - Preudhomme IR - Preudhomme FIR - Grardel IR - Grardel FIR - Roche IR - Roche FIR - Lestienne IR - Lestienne FIR - Lai IR - Lai FIR - Lepelley IR - Lepelley FIR - Darre IR - Darre FIR - de Botton IR - de Botton FIR - Soenen IR - Soenen FIR - Bauters IR - Bauters FIR - Djeda IR - Djeda FIR - Turlure IR - Turlure FIR - Bordessoule IR - Bordessoule FIR - Touati IR - Touati FIR - Remenieras IR - Remenieras FIR - Abraham IR - Abraham FIR - Gourin IR - Gourin FIR - Girault IR - Girault FIR - Moreau IR - Moreau FIR - Jaccard IR - Jaccard FIR - Chaury IR - Chaury FIR - Penot IR - Penot FIR - Trimoreau IR - Trimoreau FIR - Gachard IR - Gachard FIR - Feuillard IR - Feuillard FIR - Tisseuil IR - Tisseuil FIR - Philippon IR - Philippon FIR - Bosselut IR - Bosselut FIR - Nlend IR - Nlend FIR - Thomas IR - Thomas FIR - Chelghoum IR - Chelghoum FIR - Michallet IR - Michallet FIR - Salles IR - Salles FIR - Coiffier IR - Coiffier FIR - Espinouse IR - Espinouse FIR - Karlin IR - Karlin FIR - Bouafia-Sauvy IR - Bouafia-Sauvy FIR - Broussais-Guillaumot IR - Broussais-Guillaumot FIR - Traullé IR - Traullé FIR - Callet-Bochu IR - Callet-Bochu FIR - Tigaud IR - Tigaud FIR - Hayette IR - Hayette FIR - Nicolini IR - Nicolini FIR - Ducastelle IR - Ducastelle FIR - Labussière IR - Labussière FIR - Detrait IR - Detrait FIR - Plesa IR - Plesa FIR - Le IR - Le FIR - Tavernier IR - Tavernier FIR - Thiebaut IR - Thiebaut FIR - Girard IR - Girard FIR - Wattel IR - Wattel FIR - Boucher IR - Boucher FIR - Maire IR - Maire FIR - Etienne IR - Etienne FIR - Prebet IR - Prebet FIR - Charbonnier IR - Charbonnier FIR - Brunelle IR - Brunelle FIR - d'Incan IR - d'Incan FIR - Vey IR - Vey FIR - Rey IR - Rey FIR - Mozziconacci IR - Mozziconacci FIR - Sainty IR - Sainty FIR - Laibes IR - Laibes FIR - Arnoulet IR - Arnoulet FIR - Stoppa IR - Stoppa FIR - Mouton IR - Mouton FIR - Lafage-Pochitaloff IR - Lafage-Pochitaloff FIR - Blaise IR - Blaise FIR - Dridi IR - Dridi FIR - Redortier IR - Redortier FIR - Frayfer IR - Frayfer FIR - Allard IR - Allard FIR - Mossafa IR - Mossafa FIR - Matis IR - Matis FIR - Dorvaux IR - Dorvaux FIR - Guibaud IR - Guibaud FIR - Zamfir IR - Zamfir FIR - Christian IR - Christian FIR - Staal IR - Staal FIR - Benet IR - Benet FIR - Hagopian IR - Hagopian FIR - Vincent IR - Vincent FIR - Quittet IR - Quittet FIR - Navarro IR - Navarro FIR - Ceballos IR - Ceballos FIR - Fegueux IR - Fegueux FIR - Cartron IR - Cartron FIR - Legouffe IR - Legouffe FIR - Rossi IR - Rossi FIR - Tondeur IR - Tondeur FIR - Cacheux IR - Cacheux FIR - Bret IR - Bret FIR - Taviaux IR - Taviaux FIR - Portales IR - Portales FIR - Lavabre IR - Lavabre FIR - Taib IR - Taib FIR - Eliaou IR - Eliaou FIR - Grosjean IR - Grosjean FIR - Dupont IR - Dupont FIR - Leroux IR - Leroux FIR - Theis IR - Theis FIR - Ojeda-Uribe IR - Ojeda-Uribe FIR - Drenou IR - Drenou FIR - Eisenmann IR - Eisenmann FIR - Arkam IR - Arkam FIR - Rimelen IR - Rimelen FIR - Jeandidier IR - Jeandidier FIR - Debliquis IR - Debliquis FIR - Isaac IR - Isaac FIR - Iglarz IR - Iglarz FIR - Haby IR - Haby FIR - Bonmati IR - Bonmati FIR - Witz IR - Witz FIR - Witz IR - Witz FIR - Feugier IR - Feugier FIR - Randa IR - Randa FIR - Namour IR - Namour FIR - Grégoire IR - Grégoire FIR - Monhoven IR - Monhoven FIR - Léotard IR - Léotard FIR - Jonveaux IR - Jonveaux FIR - Béné IR - Béné FIR - Schwartz IR - Schwartz FIR - Harousseau IR - Harousseau FIR - Milpied IR - Milpied FIR - Moreau IR - Moreau FIR - Mohty IR - Mohty FIR - Le Gouill IR - Le Gouill FIR - Chevallier IR - Chevallier FIR - Guillaume IR - Guillaume FIR - Delaunay IR - Delaunay FIR - Gastinne IR - Gastinne FIR - Mahé IR - Mahé FIR - Peterlin IR - Peterlin FIR - Vigouroux IR - Vigouroux FIR - Lodé IR - Lodé FIR - Garand IR - Garand FIR - Robillard IR - Robillard FIR - Talmant IR - Talmant FIR - Avet-Loiseau IR - Avet-Loiseau FIR - Saulquin IR - Saulquin FIR - Le Houerou IR - Le Houerou FIR - Sirvent IR - Sirvent FIR - Mannone IR - Mannone FIR - Gratecos IR - Gratecos FIR - Legros IR - Legros FIR - Raynaud IR - Raynaud FIR - Philip IR - Philip FIR - Ticchioni IR - Ticchioni FIR - Touitou IR - Touitou FIR - Bouskila IR - Bouskila FIR - Jourdan IR - Jourdan FIR - Richard IR - Richard FIR - Gaillard IR - Gaillard FIR - Arnaud IR - Arnaud FIR - Chiesa IR - Chiesa FIR - Brun IR - Brun FIR - Nicolas IR - Nicolas FIR - Alexis IR - Alexis FIR - Boulet IR - Boulet FIR - Carp IR - Carp FIR - Benbrahim IR - Benbrahim FIR - Schoenwald IR - Schoenwald FIR - Legac IR - Legac FIR - Michel IR - Michel FIR - Dreyfus IR - Dreyfus FIR - Bouscary IR - Bouscary FIR - Al Nawakil IR - Al Nawakil FIR - Picard IR - Picard FIR - Vigue IR - Vigue FIR - Khelfaoui IR - Khelfaoui FIR - Mekdour IR - Mekdour FIR - Buzyn IR - Buzyn FIR - Suarez IR - Suarez FIR - Delarue IR - Delarue FIR - Sibon IR - Sibon FIR - Cheminant IR - Cheminant FIR - Marcais IR - Marcais FIR - Frenzel IR - Frenzel FIR - Brignier IR - Brignier FIR - Asnafi IR - Asnafi FIR - Radford-Weiss IR - Radford-Weiss FIR - MacIntyre IR - MacIntyre FIR - Couderc IR - Couderc FIR - Valensi IR - Valensi FIR - Varet IR - Varet FIR - Berdous Saheb IR - Berdous Saheb FIR - Isnard IR - Isnard FIR - Legrand IR - Legrand FIR - Lapusan IR - Lapusan FIR - Marzac IR - Marzac FIR - Perot IR - Perot FIR - Feger IR - Feger FIR - Negadi IR - Negadi FIR - Van den Akker IR - Van den Akker FIR - Laporte IR - Laporte FIR - Gorin IR - Gorin FIR - Ikhlef IR - Ikhlef FIR - Dombret IR - Dombret FIR - Raffoux IR - Raffoux FIR - Lengline IR - Lengline FIR - Boissel IR - Boissel FIR - Cluzeau IR - Cluzeau FIR - Micléa IR - Micléa FIR - Zini IR - Zini FIR - Cayuela IR - Cayuela FIR - Maarek IR - Maarek FIR - de Labarthe IR - de Labarthe FIR - Beldjord IR - Beldjord FIR - Daniel IR - Daniel FIR - Soulier IR - Soulier FIR - Dhédin IR - Dhédin FIR - Treilhou IR - Treilhou FIR - Klein IR - Klein FIR - Vernant IR - Vernant FIR - Leblond IR - Leblond FIR - Dhedin IR - Dhedin FIR - NGuyen IR - NGuyen FIR - Merle-Beral IR - Merle-Beral FIR - Davi IR - Davi FIR - Aliammar IR - Aliammar FIR - Nguyen-Khac IR - Nguyen-Khac FIR - Sanhes IR - Sanhes FIR - Karangwa IR - Karangwa FIR - Burcheri IR - Burcheri FIR - Roland IR - Roland FIR - Vallantin IR - Vallantin FIR - Andary IR - Andary FIR - Gueudet IR - Gueudet FIR - Barbier IR - Barbier FIR - Touhami IR - Touhami FIR - Randriamalala IR - Randriamalala FIR - Roy IR - Roy FIR - Guilhot IR - Guilhot FIR - Chomel IR - Chomel FIR - Brizard IR - Brizard FIR - Lecron IR - Lecron FIR - Turhan IR - Turhan FIR - Renaud IR - Renaud FIR - Larchee IR - Larchee FIR - Cividin IR - Cividin FIR - Larchée IR - Larchée FIR - Fouillard IR - Fouillard FIR - Gonzales IR - Gonzales FIR - Mallet IR - Mallet FIR - Guignedoux IR - Guignedoux FIR - Amirault IR - Amirault FIR - Himberlin IR - Himberlin FIR - Le IR - Le FIR - Kolb IR - Kolb FIR - Delmer IR - Delmer FIR - Quinquenel IR - Quinquenel FIR - Carcelle IR - Carcelle FIR - Cornillet-Lefebvre IR - Cornillet-Lefebvre FIR - Daliphard IR - Daliphard FIR - Luquet IR - Luquet FIR - Lafon IR - Lafon FIR - Baury IR - Baury FIR - Daliphard IR - Daliphard FIR - Haby IR - Haby FIR - Meur IR - Meur FIR - Escoffre-Barbe IR - Escoffre-Barbe FIR - Doncker IR - Doncker FIR - Houot IR - Houot FIR - Lamy de la Chapelle IR - Lamy de la Chapelle FIR - De Guibert IR - De Guibert FIR - Bernard IR - Bernard FIR - Nimubona IR - Nimubona FIR - Dauriac IR - Dauriac FIR - Fest IR - Fest FIR - Boulland IR - Boulland FIR - Henry IR - Henry FIR - Roussel IR - Roussel FIR - Ly Sunnaram IR - Ly Sunnaram FIR - Picouleau IR - Picouleau FIR - Plantier IR - Plantier FIR - Detourmignies IR - Detourmignies FIR - Fawaz IR - Fawaz FIR - Dervite IR - Dervite FIR - Thiriez IR - Thiriez FIR - Leprêtre IR - Leprêtre FIR - Lanic IR - Lanic FIR - Lenain IR - Lenain FIR - Tilly IR - Tilly FIR - Contentin IR - Contentin FIR - Stamatoullas IR - Stamatoullas FIR - Lemasle IR - Lemasle FIR - Jardin IR - Jardin FIR - Brehar IR - Brehar FIR - Bastard IR - Bastard FIR - Etancelin IR - Etancelin FIR - Boutet IR - Boutet FIR - Penther IR - Penther FIR - Lenormand IR - Lenormand FIR - Bastard IR - Bastard FIR - Simon IR - Simon FIR - Tallon IR - Tallon FIR - Janvier IR - Janvier FIR - Fabre IR - Fabre FIR - Samieh IR - Samieh FIR - Elias IR - Elias FIR - Al Jassem IR - Al Jassem FIR - Bourguignat IR - Bourguignat FIR - Glaisner IR - Glaisner FIR - Soussain IR - Soussain FIR - Malak IR - Malak FIR - Vargaftig IR - Vargaftig FIR - Bennani IR - Bennani FIR - Vavasseur IR - Vavasseur FIR - Tavernier IR - Tavernier FIR - Guyotat IR - Guyotat FIR - Jaubert IR - Jaubert FIR - Cornillon IR - Cornillon FIR - Mounier IR - Mounier FIR - Flandrin-Gresta IR - Flandrin-Gresta FIR - Nadal IR - Nadal FIR - Vasselon IR - Vasselon FIR - Solly IR - Solly FIR - Campos IR - Campos FIR - Marchand IR - Marchand FIR - Marchand IR - Marchand FIR - Bilger IR - Bilger FIR - Lioure IR - Lioure FIR - Herbrecht IR - Herbrecht FIR - Bories IR - Bories FIR - Fornecker IR - Fornecker FIR - Fohrer IR - Fohrer FIR - Moulin IR - Moulin FIR - Gaub IR - Gaub FIR - Gervais IR - Gervais FIR - Mauvieux IR - Mauvieux FIR - Oudet IR - Oudet FIR - Eischen IR - Eischen FIR - Ame IR - Ame FIR - Leymarie IR - Leymarie FIR - Aubry IR - Aubry FIR - Magnier IR - Magnier FIR - Kravanja IR - Kravanja FIR - De Jaurreguiberry IR - De Jaurreguiberry FIR - Huguet IR - Huguet FIR - Huynh IR - Huynh FIR - Roussel IR - Roussel FIR - Tavitian IR - Tavitian FIR - Ysebaert IR - Ysebaert FIR - Recher IR - Recher FIR - Borel IR - Borel FIR - Hebraud IR - Hebraud FIR - Oberic IR - Oberic FIR - Laurent IR - Laurent FIR - Nouvel IR - Nouvel FIR - Delabesse IR - Delabesse FIR - Kuhlein IR - Kuhlein FIR - Dastugue IR - Dastugue FIR - Demas IR - Demas FIR - Luquet IR - Luquet FIR - Vergez IR - Vergez FIR - Attal IR - Attal FIR - Daniel IR - Daniel FIR - Leclerc IR - Leclerc FIR - Lissandre IR - Lissandre FIR - Dartigeas IR - Dartigeas FIR - Gyan IR - Gyan FIR - Renaud IR - Renaud FIR - Monjanel IR - Monjanel FIR - Ertault de la Bretonniere IR - Ertault de la Bretonniere FIR - Benboubker IR - Benboubker FIR - Estienne IR - Estienne FIR - Barin IR - Barin FIR - Watier IR - Watier FIR - Delain IR - Delain FIR - Delepine IR - Delepine FIR - Degene IR - Degene FIR - Colombat IR - Colombat FIR - Nollet IR - Nollet FIR - Fernandes IR - Fernandes FIR - Pollet IR - Pollet FIR - Tricot IR - Tricot FIR - Simon IR - Simon FIR - Bremeault IR - Bremeault FIR - Daudignon IR - Daudignon FIR - Bisiau IR - Bisiau FIR - Poulain IR - Poulain FIR - Crametz IR - Crametz FIR - Rousselot IR - Rousselot FIR - Castaigne IR - Castaigne FIR - Salmeron IR - Salmeron FIR - Lambert IR - Lambert FIR - Taksin IR - Taksin FIR - Rigaudeau IR - Rigaudeau FIR - Farhat IR - Farhat FIR - Merabet IR - Merabet FIR - Ghez IR - Ghez FIR - Spentchian IR - Spentchian FIR - Terre IR - Terre FIR - Garcia IR - Garcia FIR - Pousset IR - Pousset FIR - Henri IR - Henri FIR - de Botton IR - de Botton FIR - Bourhis IR - Bourhis FIR - Arnaud IR - Arnaud FIR - Micol IR - Micol FIR - Garnier IR - Garnier FIR - Willekens IR - Willekens FIR - Bennaceur IR - Bennaceur FIR - Auger IR - Auger FIR - Saada IR - Saada FIR - Bayle IR - Bayle FIR - Bernheim IR - Bernheim FIR - Ferrant IR - Ferrant FIR - Costantini IR - Costantini FIR - Libouton IR - Libouton FIR - Poirel IR - Poirel FIR - Mineur IR - Mineur FIR - Delannoy IR - Delannoy FIR - Tacal IR - Tacal FIR - Rack IR - Rack FIR - Bosly IR - Bosly FIR - Crasson IR - Crasson FIR - Michaux IR - Michaux FIR - Vandenbeghe IR - Vandenbeghe FIR - Hagemeijer IR - Hagemeijer FIR - Bargetzi IR - Bargetzi FIR - Heizmann IR - Heizmann FIR - Sigle IR - Sigle FIR - Gurtner IR - Gurtner FIR - Gratwohl IR - Gratwohl FIR - Arber IR - Arber FIR - Stern IR - Stern FIR - Heim IR - Heim FIR - Pabst IR - Pabst FIR - Rettich IR - Rettich FIR - Chalandon IR - Chalandon FIR - Passweg IR - Passweg FIR - Tirefort IR - Tirefort FIR - Salamanczuk IR - Salamanczuk FIR - Trembleau IR - Trembleau FIR - Lambert IR - Lambert FIR - Spertini IR - Spertini FIR - Jotterand IR - Jotterand FIR - Schoumans Pouw IR - Schoumans Pouw FIR - Kunkel IR - Kunkel FIR - Quarroz IR - Quarroz FIR - Mulhematter IR - Mulhematter FIR - Porter IR - Porter FIR - Abbal IR - Abbal FIR - Jeckelmann IR - Jeckelmann FIR - Voegtin IR - Voegtin FIR - Gregor IR - Gregor FIR - Wuillemin IR - Wuillemin FIR - Nagler IR - Nagler FIR - Fahrni IR - Fahrni FIR - Driessen IR - Driessen FIR - Hess IR - Hess FIR - Hitz IR - Hitz FIR - Weder IR - Weder FIR - Raess IR - Raess FIR - Demmer IR - Demmer FIR - Jacky IR - Jacky FIR - Reiner IR - Reiner FIR - Schanz IR - Schanz FIR - Sasselli IR - Sasselli FIR - Schafer IR - Schafer FIR - Lhéritier IR - Lhéritier FIR - Genton IR - Genton FIR - Largiader IR - Largiader FIR - Kuenzle IR - Kuenzle FIR - Keunecke IR - Keunecke EDAT- 2014/04/18 06:00 MHDA- 2014/08/13 06:00 CRDT- 2014/04/18 06:00 PHST- 2014/04/18 06:00 [entrez] PHST- 2014/04/18 06:00 [pubmed] PHST- 2014/08/13 06:00 [medline] AID - S0006-4971(20)40108-9 [pii] AID - 10.1182/blood-2014-01-547695 [doi] PST - ppublish SO - Blood. 2014 Jun 12;123(24):3739-49. doi: 10.1182/blood-2014-01-547695. Epub 2014 Apr 16. PMID- 36313333 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20250530 IS - 1663-9812 (Print) IS - 1663-9812 (Electronic) IS - 1663-9812 (Linking) VI - 13 DP - 2022 TI - World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease. PG - 979788 LID - 10.3389/fphar.2022.979788 [doi] LID - 979788 AB - Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF. Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included. Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94-100% for the three additional PROMs. Thirty-four new 'Personal Factors' emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. 'pf_embarrassed by cough' or 'pf_panic/afraid when can't get a breath'. Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11. CI - Copyright © 2022 Saketkoo, Escorpizo, Varga, Keen, Fligelstone, Birring, Alexanderson, Pettersson, Chaudhry, Poole, Regardt, LeSage, Sarver, Lanario, Renzoni, Scholand, Lammi, Kowal-Bielecka, Distler, Frech, Shapiro, Varju, Volkmann, Bernstein, Drent, Obi, Patterson, Russell and The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS). FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, United States. AD - University Medical Center-Comprehensive Pulmonary Hypertension Center & Interstitial Lung Disease Clinic Programs, New Orleans, LA, United States. AD - Tulane University School of Medicine, New Orleans, LA, United States. AD - Louisiana State University Health Sciences Center, Division of Pulmonary Medicine-New Orleans, New Orleans, LA, United States. FAU - Escorpizo, Reuben AU - Escorpizo R AD - Department of Rehabilitation and Movement Science, The University of Vermont, Burlington, VT, United States. AD - Swiss Paraplegic Research, Nottwil, Switzerland. FAU - Varga, Janos AU - Varga J AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Keen, Kevin John AU - Keen KJ AD - Department of Mathematics and Statistics and Health Research Institute, University of Northern British Columbia, Prince George, BC, Canada. AD - Department of Medicine, University of British Columbia & Centre for Heart Lung Innovation, Providence Research, Vancouver, BC, Canada. FAU - Fligelstone, Kim AU - Fligelstone K AD - Patient Research Partner Scleroderma & Raynaud Society, UK (SRUK) and Federation of European Scleroderma Associations, London, United Kingdom. AD - Royal Free Hospital Scleroderma Unit, London, United Kingdom. FAU - Birring, Surinder S AU - Birring SS AD - Division of Asthma, Allergy and Lung Biology, King's College London, London, United Kingdom. FAU - Alexanderson, Helene AU - Alexanderson H AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden. AD - Department of Medicin, Division of Rheumatology, Karolinska Institutet, Stockholm, Sweden. FAU - Pettersson, Henrik AU - Pettersson H AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden. AD - Department of Medicin, Division of Rheumatology, Karolinska Institutet, Stockholm, Sweden. FAU - Chaudhry, Humza Ahmad AU - Chaudhry HA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, United States. AD - University Medical Center-Comprehensive Pulmonary Hypertension Center & Interstitial Lung Disease Clinic Programs, New Orleans, LA, United States. AD - Tulane University School of Medicine, New Orleans, LA, United States. FAU - Poole, Janet L AU - Poole JL AD - Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, United States. FAU - Regardt, Malin AU - Regardt M AD - Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden. AD - Department of Medicin, Division of Rheumatology, Karolinska Institutet, Stockholm, Sweden. FAU - LeSage, Daphne AU - LeSage D AD - Patient Research Partner, New Orleans, LA, United States. FAU - Sarver, Catherine AU - Sarver C AD - Patient Research Partner, Baltimore, MD, United States. FAU - Lanario, Joseph AU - Lanario J AD - Research Fellow in Respiratory Health-Exeter Respiratory Institute Royal Devon University Hospitals NHS Foundation Trust, Exeter, United Kingdom. FAU - Renzoni, Elisabetta AU - Renzoni E AD - Royal Brompton Hospital, National Heart and Lung Institute, London, United Kingdom. FAU - Scholand, Mary Beth AU - Scholand MB AD - Pulmonary Medicine, University of Utah, Salt Lake City, UT, United States. FAU - Lammi, Matthew R AU - Lammi MR AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, United States. AD - University Medical Center-Comprehensive Pulmonary Hypertension Center & Interstitial Lung Disease Clinic Programs, New Orleans, LA, United States. AD - Louisiana State University Health Sciences Center, Division of Pulmonary Medicine-New Orleans, New Orleans, LA, United States. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - University of Bialystok, Bialystok, Poland. FAU - Distler, Oliver AU - Distler O AD - Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland. FAU - Frech, Tracy AU - Frech T AD - Division of Rheumatology Vanderbilt University School of Medicine, Nashville, TN, United States. AD - Pulmonary Medicine, University of Utah, Salt Lake City, UT, United States. FAU - Shapiro, Lee AU - Shapiro L AD - Division of Rheumatology, Albany Medical Center, Albany, NY, United States. AD - Steffens Scleroderma Foundation, Albany, NY, United States. FAU - Varju, Cecilia AU - Varju C AD - Department of Rheumatology and Immunology, Medical School, University of Pécs, Pecs, Hungary. FAU - Volkmann, Elizabeth R AU - Volkmann ER AD - Department of Medicine, David Geffen School of Medicine, UCLA Scleroderma Program and UCLA CTD-ILD Program, Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, United States. FAU - Bernstein, Elana J AU - Bernstein EJ AD - Department of Medicine, Columbia University/New York-Presbyterian Scleroderma Program, Division of Rheumatology, Columbia University College of Physician2s and Surgeons, New York, NY, United States. FAU - Drent, Marjolein AU - Drent M AD - Department of Pulmonology, Interstitial Lung Diseases (ILD) Center of Excellence, St. Antonius Hospital, Nieuwegein, Netherlands. AD - Department of Pharmacology and Toxicology, Faculty of Health and Life Sciences, Maastricht University, Nieuwegein, Netherlands. FAU - Obi, Ogugua Ndili AU - Obi ON AD - Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Brody School of Medicine, East Carolina University, Greenville, NC, United States. FAU - Patterson, Karen C AU - Patterson KC AD - Department of Clinical & Experimental Medicine, Brighton & Sussex Medical School, Falmer, United Kingdom. AD - Division Pulmonary, Allergy, and Critical Care Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States. FAU - Russell, Anne-Marie AU - Russell AM AD - Respiratory Institute to Exeter Respiratory Innovation Center, University of Exeter, Exeter, United Kingdom. AD - Respiratory Medicine, Royal Devon University Healthcare NHS Foundation Trust, London, United Kingdom. CN - Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS) LA - eng GR - 20719/VAC_/Versus Arthritis/United Kingdom PT - Journal Article DEP - 20221014 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC9615472 OTO - NOTNLM OT - CTD-ILD OT - ICD-11 OT - connective tissue OT - cough OT - fibrosis OT - patient-reported outcomes COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/11/01 06:00 MHDA- 2022/11/01 06:01 PMCR- 2022/10/14 CRDT- 2022/10/31 04:55 PHST- 2022/06/27 00:00 [received] PHST- 2022/07/13 00:00 [accepted] PHST- 2022/10/31 04:55 [entrez] PHST- 2022/11/01 06:00 [pubmed] PHST- 2022/11/01 06:01 [medline] PHST- 2022/10/14 00:00 [pmc-release] AID - 979788 [pii] AID - 10.3389/fphar.2022.979788 [doi] PST - epublish SO - Front Pharmacol. 2022 Oct 14;13:979788. doi: 10.3389/fphar.2022.979788. eCollection 2022. PMID- 20159824 OWN - NLM STAT- MEDLINE DCOM- 20100504 LR - 20250529 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 28 IP - 9 DP - 2010 Mar 20 TI - Phase I clinical trial of the CYP17 inhibitor abiraterone acetate demonstrating clinical activity in patients with castration-resistant prostate cancer who received prior ketoconazole therapy. PG - 1481-8 LID - 10.1200/JCO.2009.24.1281 [doi] AB - PURPOSE: Abiraterone acetate is a prodrug of abiraterone, a selective inhibitor of CYP17, the enzyme catalyst for two essential steps in androgen biosynthesis. In castration-resistant prostate cancers (CRPCs), extragonadal androgen sources may sustain tumor growth despite a castrate environment. This phase I dose-escalation study of abiraterone acetate evaluated safety, pharmacokinetics, and effects on steroidogenesis and prostate-specific antigen (PSA) levels in men with CPRC with or without prior ketoconazole therapy. PATIENTS AND METHODS: Thirty-three men with chemotherapy-naïve progressive CRPC were enrolled. Nineteen patients (58%) had previously received ketoconazole for CRPC. Bone metastases were present in 70% of patients, and visceral involvement was present in 18%. Three patients (9%) had locally advanced disease without distant metastases. Fasted or fed cohorts received abiraterone acetate doses of 250, 500, 750, or 1,000 mg daily. Single-dose pharmacokinetic analyses were performed before continuous daily dosing. RESULTS: Adverse events were predominantly grade 1 or 2. No dose-limiting toxicities were observed. Hypertension (grade 3, 12%) and hypokalemia (grade 3, 6%; grade 4, 3%) were the most frequent serious toxicities and responded to medical management. Confirmed > or = 50% PSA declines at week 12 were seen in 18 (55%) of 33 patients, including nine (47%) of 19 patients with prior ketoconazole therapy and nine (64%) of 14 patients without prior ketoconazole therapy. Substantial declines in circulating androgens and increases in mineralocorticoids were seen with all doses. CONCLUSION: Abiraterone acetate was well tolerated and demonstrated activity in CRPC, including in patients previously treated with ketoconazole. Continued clinical study is warranted. FAU - Ryan, Charles J AU - Ryan CJ AD - Urologic Oncology Program, University of California, San Francisco, San Francisco, CA 94115, USA. ryanc@medicine.ucsf.edu FAU - Smith, Matthew R AU - Smith MR FAU - Fong, Lawrence AU - Fong L FAU - Rosenberg, Jonathan E AU - Rosenberg JE FAU - Kantoff, Philip AU - Kantoff P FAU - Raynaud, Florence AU - Raynaud F FAU - Martins, Vanessa AU - Martins V FAU - Lee, Gloria AU - Lee G FAU - Kheoh, Thian AU - Kheoh T FAU - Kim, Jennifer AU - Kim J FAU - Molina, Arturo AU - Molina A FAU - Small, Eric J AU - Small EJ LA - eng GR - K23 CA115775/CA/NCI NIH HHS/United States GR - K24 CA121990/CA/NCI NIH HHS/United States GR - K23CA115775/CA/NCI NIH HHS/United States PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. DEP - 20100216 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 RN - 0 (Androgen Antagonists) RN - 0 (Androgens) RN - 0 (Androstenes) RN - 0 (Androstenols) RN - 0 (Antineoplastic Agents) RN - EC 1.14.14.19 (Steroid 17-alpha-Hydroxylase) RN - EC 3.4.21.77 (Prostate-Specific Antigen) RN - G819A456D0 (abiraterone) RN - R9400W927I (Ketoconazole) SB - IM CIN - J Clin Oncol. 2010 Mar 20;28(9):1447-9. doi: 10.1200/JCO.2009.25.3781. PMID: 20159817 CIN - J Clin Oncol. 2010 Oct 10;28(29):e560-1; author reply e562. doi: 10.1200/JCO.2010.29.5170. PMID: 20805462 MH - Aged MH - Aged, 80 and over MH - Androgen Antagonists/pharmacokinetics/*therapeutic use MH - Androgens/metabolism MH - Androstenes MH - Androstenols/pharmacokinetics/*therapeutic use MH - Antineoplastic Agents/pharmacokinetics/*therapeutic use MH - Dose-Response Relationship, Drug MH - Humans MH - Ketoconazole/therapeutic use MH - Male MH - Middle Aged MH - Orchiectomy MH - Prostate-Specific Antigen/blood MH - Prostatic Neoplasms/blood/*drug therapy/metabolism/surgery MH - Steroid 17-alpha-Hydroxylase/antagonists & inhibitors MH - Treatment Outcome PMC - PMC2849769 COIS- Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article. EDAT- 2010/02/18 06:00 MHDA- 2010/05/05 06:00 PMCR- 2011/03/20 CRDT- 2010/02/18 06:00 PHST- 2010/02/18 06:00 [entrez] PHST- 2010/02/18 06:00 [pubmed] PHST- 2010/05/05 06:00 [medline] PHST- 2011/03/20 00:00 [pmc-release] AID - JCO.2009.24.1281 [pii] AID - 41281 [pii] AID - 10.1200/JCO.2009.24.1281 [doi] PST - ppublish SO - J Clin Oncol. 2010 Mar 20;28(9):1481-8. doi: 10.1200/JCO.2009.24.1281. Epub 2010 Feb 16. PMID- 26796641 OWN - NLM STAT- MEDLINE DCOM- 20160712 LR - 20250529 IS - 1520-4804 (Electronic) IS - 0022-2623 (Print) IS - 0022-2623 (Linking) VI - 59 IP - 3 DP - 2016 Feb 11 TI - Discovery of Potent, Selective, and Orally Bioavailable Small-Molecule Modulators of the Mediator Complex-Associated Kinases CDK8 and CDK19. PG - 1078-101 LID - 10.1021/acs.jmedchem.5b01685 [doi] AB - The Mediator complex-associated cyclin-dependent kinase CDK8 has been implicated in human disease, particularly in colorectal cancer where it has been reported as a putative oncogene. Here we report the discovery of 109 (CCT251921), a potent, selective, and orally bioavailable inhibitor of CDK8 with equipotent affinity for CDK19. We describe a structure-based design approach leading to the discovery of a 3,4,5-trisubstituted-2-aminopyridine series and present the application of physicochemical property analyses to successfully reduce in vivo metabolic clearance, minimize transporter-mediated biliary elimination while maintaining acceptable aqueous solubility. Compound 109 affords the optimal compromise of in vitro biochemical, pharmacokinetic, and physicochemical properties and is suitable for progression to animal models of cancer. FAU - Mallinger, Aurélie AU - Mallinger A AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Schiemann, Kai AU - Schiemann K AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Rink, Christian AU - Rink C AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Stieber, Frank AU - Stieber F AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Calderini, Michel AU - Calderini M AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Crumpler, Simon AU - Crumpler S AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Stubbs, Mark AU - Stubbs M AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Adeniji-Popoola, Olajumoke AU - Adeniji-Popoola O AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Poeschke, Oliver AU - Poeschke O AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Busch, Michael AU - Busch M AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Czodrowski, Paul AU - Czodrowski P AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Musil, Djordje AU - Musil D AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Schwarz, Daniel AU - Schwarz D AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Ortiz-Ruiz, Maria-Jesus AU - Ortiz-Ruiz MJ AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Schneider, Richard AU - Schneider R AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Thai, Ching AU - Thai C AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - de Haven Brandon, Alexis AU - de Haven Brandon A AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Burke, Rosemary AU - Burke R AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Workman, Paul AU - Workman P AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Dale, Trevor AU - Dale T AD - School of Bioscience, Cardiff University , Cardiff, CF10 3AX, U.K. FAU - Wienke, Dirk AU - Wienke D AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Clarke, Paul A AU - Clarke PA AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Esdar, Christina AU - Esdar C AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Raynaud, Florence I AU - Raynaud FI AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Eccles, Suzanne A AU - Eccles SA AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. FAU - Rohdich, Felix AU - Rohdich F AD - Merck KGaA , Darmstadt, 64293, Germany. FAU - Blagg, Julian AU - Blagg J AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London, SW7 3RP, U.K. LA - eng GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160121 PL - United States TA - J Med Chem JT - Journal of medicinal chemistry JID - 9716531 RN - 0 (Aminopyridines) RN - 0 (Small Molecule Libraries) RN - EC 2.7.11.22 (CDK19 protein, human) RN - EC 2.7.11.22 (CDK8 protein, human) RN - EC 2.7.11.22 (Cyclin-Dependent Kinase 8) RN - EC 2.7.11.22 (Cyclin-Dependent Kinases) RN - WSX981HEWU (alpha-aminopyridine) SB - IM MH - Administration, Oral MH - Aminopyridines/administration & dosage/chemistry/*pharmacology MH - Animals MH - Biological Availability MH - Caco-2 Cells MH - Cyclin-Dependent Kinase 8/*antagonists & inhibitors/metabolism MH - Cyclin-Dependent Kinases/*antagonists & inhibitors/metabolism MH - Dogs MH - Dose-Response Relationship, Drug MH - *Drug Discovery MH - Female MH - Humans MH - Male MH - Mice MH - Models, Molecular MH - Molecular Structure MH - Neoplasms, Experimental/drug therapy/pathology MH - Rats MH - Rats, Wistar MH - Small Molecule Libraries/administration & dosage/chemistry/*pharmacology MH - Solubility MH - Structure-Activity Relationship MH - Xenograft Model Antitumor Assays PMC - PMC5362750 COIS- The authors declare the following competing financial interest(s): A.M., C.R., S.C., M.S., O.A-P., M.-J.O.-R., C.T., M.V., A.d.H.B., R.B., P.W., T.D., P.A.C., F.I.R., S.A.E., and J.B. are current or former employees of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. K.S., F.S., M.C., O.P., M.B., P.C., D.M., D.S., R.S., D.W., C.E. and F.R. are current employees of Merck. EDAT- 2016/01/23 06:00 MHDA- 2016/07/13 06:00 PMCR- 2017/03/23 CRDT- 2016/01/23 06:00 PHST- 2016/01/23 06:00 [entrez] PHST- 2016/01/23 06:00 [pubmed] PHST- 2016/07/13 06:00 [medline] PHST- 2017/03/23 00:00 [pmc-release] AID - 10.1021/acs.jmedchem.5b01685 [doi] PST - ppublish SO - J Med Chem. 2016 Feb 11;59(3):1078-101. doi: 10.1021/acs.jmedchem.5b01685. Epub 2016 Jan 21. PMID- 27197232 OWN - NLM STAT- MEDLINE DCOM- 20170724 LR - 20250530 IS - 1538-7445 (Electronic) IS - 0008-5472 (Linking) VI - 76 IP - 10 DP - 2016 May 15 TI - p53 Loss in MYC-Driven Neuroblastoma Leads to Metabolic Adaptations Supporting Radioresistance. PG - 3025-35 LID - 10.1158/0008-5472.CAN-15-1939 [doi] AB - Neuroblastoma is the most common childhood extracranial solid tumor. In high-risk cases, many of which are characterized by amplification of MYCN, outcome remains poor. Mutations in the p53 (TP53) tumor suppressor are rare at diagnosis, but evidence suggests that p53 function is often impaired in relapsed, treatment-resistant disease. To address the role of p53 loss of function in the development and pathogenesis of high-risk neuroblastoma, we generated a MYCN-driven genetically engineered mouse model in which the tamoxifen-inducible p53ER(TAM) fusion protein was expressed from a knock-in allele (Th-MYCN/Trp53(KI)). We observed no significant differences in tumor-free survival between Th-MYCN mice heterozygous for Trp53(KI) (n = 188) and Th-MYCN mice with wild-type p53 (n = 101). Conversely, the survival of Th-MYCN/Trp53(KI/KI) mice lacking functional p53 (n = 60) was greatly reduced. We found that Th-MYCN/Trp53(KI/KI) tumors were resistant to ionizing radiation (IR), as expected. However, restoration of functional p53ER(TAM) reinstated sensitivity to IR in only 50% of Th-MYCN/Trp53(KI/KI) tumors, indicating the acquisition of additional resistance mechanisms. Gene expression and metabolic analyses indicated that the principal acquired mechanism of resistance to IR in the absence of functional p53 was metabolic adaptation in response to chronic oxidative stress. Tumors exhibited increased antioxidant metabolites and upregulation of glutathione S-transferase pathway genes, including Gstp1 and Gstz1, which are associated with poor outcome in human neuroblastoma. Accordingly, glutathione depletion by buthionine sulfoximine together with restoration of p53 activity resensitized tumors to IR. Our findings highlight the complex pathways operating in relapsed neuroblastomas and the need for combination therapies that target the diverse resistance mechanisms at play. Cancer Res; 76(10); 3025-35. ©2016 AACR. CI - ©2016 American Association for Cancer Research. FAU - Yogev, Orli AU - Yogev O AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Barker, Karen AU - Barker K AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Sikka, Arti AU - Sikka A AD - Department of Surgery and Cancer, Imperial College, London, United Kingdom. FAU - Almeida, Gilberto S AU - Almeida GS AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, United Kingdom. FAU - Hallsworth, Albert AU - Hallsworth A AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Smith, Laura M AU - Smith LM AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Jamin, Yann AU - Jamin Y AD - Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, United Kingdom. FAU - Ruddle, Ruth AU - Ruddle R AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Koers, Alexander AU - Koers A AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Webber, Hannah T AU - Webber HT AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Popov, Sergey AU - Popov S AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. Department of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom. FAU - Jones, Chris AU - Jones C AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. Department of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom. FAU - Petrie, Kevin AU - Petrie K AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Robinson, Simon P AU - Robinson SP AD - Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, United Kingdom. FAU - Keun, Hector C AU - Keun HC AD - Department of Surgery and Cancer, Imperial College, London, United Kingdom. FAU - Chesler, Louis AU - Chesler L AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. louis.chesler@icr.ac.uk. LA - eng GR - C1060/A16464/MRC_/Medical Research Council/United Kingdom GR - 091763Z/10/Z/WT_/Wellcome Trust/United Kingdom GR - DH_/Department of Health/United Kingdom GR - 18339/CRUK_/Cancer Research UK/United Kingdom GR - C1060/A10334/MRC_/Medical Research Council/United Kingdom GR - 16464/CRUK_/Cancer Research UK/United Kingdom GR - MR/J015938/1/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160329 PL - United States TA - Cancer Res JT - Cancer research JID - 2984705R RN - 0 (MYCN protein, mouse) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (RNA, Messenger) RN - 0 (Tumor Suppressor Protein p53) SB - IM CIN - Ann Transl Med. 2016 Dec;4(24):522. doi: 10.21037/atm.2016.12.40. PMID: 28149884 MH - Adaptation, Physiological/*radiation effects MH - Animals MH - Apoptosis/*radiation effects MH - Blotting, Western MH - Cell Proliferation/radiation effects MH - Female MH - Humans MH - Immunoenzyme Techniques MH - Male MH - Mice MH - Mice, Transgenic MH - N-Myc Proto-Oncogene Protein/*physiology MH - Neuroblastoma/*metabolism/*pathology/radiotherapy MH - RNA, Messenger/genetics MH - *Radiation Tolerance MH - Radiation, Ionizing MH - Real-Time Polymerase Chain Reaction MH - Reverse Transcriptase Polymerase Chain Reaction MH - Tumor Suppressor Protein p53/*physiology EDAT- 2016/05/20 06:00 MHDA- 2017/07/25 06:00 CRDT- 2016/05/20 06:00 PHST- 2015/07/20 00:00 [received] PHST- 2016/02/09 00:00 [accepted] PHST- 2016/05/20 06:00 [entrez] PHST- 2016/05/20 06:00 [pubmed] PHST- 2017/07/25 06:00 [medline] AID - 0008-5472.CAN-15-1939 [pii] AID - 10.1158/0008-5472.CAN-15-1939 [doi] PST - ppublish SO - Cancer Res. 2016 May 15;76(10):3025-35. doi: 10.1158/0008-5472.CAN-15-1939. Epub 2016 Mar 29. PMID- 34049571 OWN - NLM STAT- MEDLINE DCOM- 20210921 LR - 20260518 IS - 1466-609X (Electronic) IS - 1364-8535 (Print) IS - 1364-8535 (Linking) VI - 25 IP - 1 DP - 2021 May 28 TI - Factors associated with death in children with purpura fulminans: a French national prospective cohort study. PG - 181 LID - 10.1186/s13054-021-03603-8 [doi] LID - 181 FAU - Madhi, Fouad AU - Madhi F AUID- ORCID: 0000-0001-6816-5272 AD - Service de Pédiatrie Générale, Centre Hospitalier Intercommunal de Créteil, 40, avenue de Verdun, 94000, Créteil, France. fouad.madhi@chicreteil.fr. AD - GPIP, Groupe de Pathologie Infectieuse Pédiatrique, Paris, France. fouad.madhi@chicreteil.fr. AD - Université Paris Est, IMRB-GRC GEMINI, Créteil, France. fouad.madhi@chicreteil.fr. FAU - Ouldali, Naim AU - Ouldali N AD - GPIP, Groupe de Pathologie Infectieuse Pédiatrique, Paris, France. AD - ACTIV, Association Clinique et Thérapeutique Infantile du Val-de-Marne, Saint Maur-des-Fossés, France. AD - Department of General Pediatrics, Pediatric Infectious Disease and Internal Medicine, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. AD - Université de Paris, INSERM UMR 1123, ECEVE, Paris, France. FAU - Levy, Corinne AU - Levy C AD - GPIP, Groupe de Pathologie Infectieuse Pédiatrique, Paris, France. AD - Université Paris Est, IMRB-GRC GEMINI, Créteil, France. AD - ACTIV, Association Clinique et Thérapeutique Infantile du Val-de-Marne, Saint Maur-des-Fossés, France. AD - Clinical Research Center (CRC), Centre Hospitalier Intercommunal de Créteil, Créteil, France. FAU - Taha, Muhamed-Kheir AU - Taha MK AD - Institut Pasteur, Unit of Invasive Bacterial Infections & National Reference Center for Meningococci, 75724, Paris, Cedex 15, France. FAU - Cohen, Robert AU - Cohen R AD - GPIP, Groupe de Pathologie Infectieuse Pédiatrique, Paris, France. AD - Université Paris Est, IMRB-GRC GEMINI, Créteil, France. AD - ACTIV, Association Clinique et Thérapeutique Infantile du Val-de-Marne, Saint Maur-des-Fossés, France. AD - Unité Court Séjour, Petits nourrissons, Service de Néonatalogie, Centre Hospitalier Intercommunal de Créteil, Paris, France. CN - French Pediatric Meningitis Network LA - eng PT - Journal Article DEP - 20210528 PL - England TA - Crit Care JT - Critical care (London, England) JID - 9801902 SB - IM MH - Child MH - Child, Preschool MH - Cohort Studies MH - Female MH - France MH - Humans MH - Male MH - Multivariate Analysis MH - Prospective Studies MH - Purpura Fulminans/*mortality/physiopathology MH - Risk Factors PMC - PMC8164283 COIS- The authors declare that they have no competing interests. FIR - Abdelhadi IR - Abdelhadi FIR - Aberrane IR - Aberrane FIR - Warde, Abi IR - Warde A FIR - Tara, Abou IR - Tara A FIR - Abrudan IR - Abrudan FIR - Adam IR - Adam FIR - Adamon IR - Adamon FIR - Akitani IR - Akitani FIR - Alba-Sauviat IR - Alba-Sauviat FIR - Chaar, Al IR - Chaar A FIR - Albertini IR - Albertini FIR - Aljazayri IR - Aljazayri FIR - Aljumaidi IR - Aljumaidi FIR - Allali IR - Allali FIR - Allia IR - Allia FIR - Mazini, Al IR - Mazini A FIR - Amama IR - Amama FIR - Amara IR - Amara FIR - Amira IR - Amira FIR - Amirault IR - Amirault FIR - Andriantahina IR - Andriantahina FIR - Ansoborlo IR - Ansoborlo FIR - Arlet IR - Arlet FIR - Armengaud IR - Armengaud FIR - Armouche IR - Armouche FIR - Arnault IR - Arnault FIR - Asensio IR - Asensio FIR - Aubert IR - Aubert FIR - Auburtin IR - Auburtin FIR - Audry IR - Audry FIR - Aujard IR - Aujard FIR - Autret IR - Autret FIR - Azemar IR - Azemar FIR - Bachelier IR - Bachelier FIR - Badran IR - Badran FIR - Bajolle IR - Bajolle FIR - Baleine IR - Baleine FIR - Bandin IR - Bandin FIR - Banegas IR - Banegas FIR - Banerjee IR - Banerjee FIR - Banige IR - Banige FIR - Barbier IR - Barbier FIR - Bardou IR - Bardou FIR - Baret IR - Baret FIR - Barnaud IR - Barnaud FIR - Baron-Joly IR - Baron-Joly FIR - Barrans IR - Barrans FIR - Barraud IR - Barraud FIR - Barré IR - Barré FIR - Barrey IR - Barrey FIR - Barsotti IR - Barsotti FIR - Barthez IR - Barthez FIR - Bartizel IR - Bartizel FIR - Baruteau IR - Baruteau FIR - Basmaci IR - Basmaci FIR - Bassil IR - Bassil FIR - Battaglini IR - Battaglini FIR - Bayle IR - Bayle FIR - Bednarek IR - Bednarek FIR - Belgaid IR - Belgaid FIR - Belivier IR - Belivier FIR - Bellulo IR - Bellulo FIR - Benabdelmalek IR - Benabdelmalek FIR - Benchekroun IR - Benchekroun FIR - Benezech IR - Benezech FIR - Bengrina IR - Bengrina FIR - Benmahammed IR - Benmahammed FIR - Benmoulai IR - Benmoulai FIR - Benoist IR - Benoist FIR - Benoit IR - Benoit FIR - Bensaid IR - Bensaid FIR - Benseddik IR - Benseddik FIR - Benzaim IR - Benzaim FIR - Berche IR - Berche FIR - Berrahma IR - Berrahma FIR - Beretta-Salaun IR - Beretta-Salaun FIR - Bergounioux IR - Bergounioux FIR - Berrahma IR - Berrahma FIR - Berterottiere IR - Berterottiere FIR - Berthier IR - Berthier FIR - Bertrou IR - Bertrou FIR - Besson-Leaud IR - Besson-Leaud FIR - Biendo IR - Biendo FIR - Biessy IR - Biessy FIR - Billaud IR - Billaud FIR - Billion IR - Billion FIR - Bina IR - Bina FIR - Bineau IR - Bineau FIR - Biran IR - Biran FIR - Bitar-Obeid IR - Bitar-Obeid FIR - Blanc IR - Blanc FIR - Blasquez IR - Blasquez FIR - Blondel IR - Blondel FIR - Blondin IR - Blondin FIR - Boileau IR - Boileau FIR - Boivin IR - Boivin FIR - Boize IR - Boize FIR - Bolot IR - Bolot FIR - Bonacorsi IR - Bonacorsi FIR - Boniface IR - Boniface FIR - Boina, Bongo IR - Boina B FIR - Bonnin IR - Bonnin FIR - Boquet IR - Boquet FIR - Bordes IR - Bordes FIR - Born IR - Born FIR - Bosdure IR - Bosdure FIR - Bosi IR - Bosi FIR - Bost-Bru IR - Bost-Bru FIR - Bouainane IR - Bouainane FIR - Bouderlique IR - Bouderlique FIR - Boukezia IR - Boukezia FIR - Boulard IR - Boulard FIR - Bour IR - Bour FIR - Bourennane IR - Bourennane FIR - Bourgeois-Nicolaos IR - Bourgeois-Nicolaos FIR - Boussadia IR - Boussadia FIR - Boussicault IR - Boussicault FIR - Boutros IR - Boutros FIR - Boutte IR - Boutte FIR - Bouvet IR - Bouvet FIR - Bouziges IR - Bouziges FIR - Bovero IR - Bovero FIR - Boyer IR - Boyer FIR - Branca IR - Branca FIR - Branger IR - Branger FIR - Bresson IR - Bresson FIR - Breton IR - Breton FIR - Breuil IR - Breuil FIR - Briand IR - Briand FIR - Brieu IR - Brieu FIR - Brizard IR - Brizard FIR - Brocard IR - Brocard FIR - Brouard IR - Brouard FIR - Bruna IR - Bruna FIR - Brunel IR - Brunel FIR - Bruno IR - Bruno FIR - Bruyas IR - Bruyas FIR - Bucher IR - Bucher FIR - Bucur IR - Bucur FIR - Buisson-Touati IR - Buisson-Touati FIR - Bulteau-Cowan IR - Bulteau-Cowan FIR - Cabaret IR - Cabaret FIR - Cadot IR - Cadot FIR - Caillon IR - Caillon FIR - Callamand IR - Callamand FIR - Calvez IR - Calvez FIR - Cambonie IR - Cambonie FIR - Campet IR - Campet FIR - Canis IR - Canis FIR - Canzi IR - Canzi FIR - Capdeville IR - Capdeville FIR - Carbajal IR - Carbajal FIR - Carbonnelle IR - Carbonnelle FIR - Carre IR - Carre FIR - Carre-Cavellier IR - Carre-Cavellier FIR - Carrer IR - Carrer FIR - Carriere IR - Carriere FIR - Carroger IR - Carroger FIR - Cartier IR - Cartier FIR - Cartier-Riviere IR - Cartier-Riviere FIR - Cascarigny IR - Cascarigny FIR - Castella IR - Castella FIR - Castelnau IR - Castelnau FIR - Cathenoz IR - Cathenoz FIR - Cattoen IR - Cattoen FIR - Cattoir IR - Cattoir FIR - Cau IR - Cau FIR - Cavalier IR - Cavalier FIR - Cecille IR - Cecille FIR - Chabrol IR - Chabrol FIR - Chace IR - Chace FIR - Chaix IR - Chaix FIR - Chale IR - Chale FIR - Chalumeau IR - Chalumeau FIR - Demersay, Chalvon IR - Demersay C FIR - Chami IR - Chami FIR - Chamouilli IR - Chamouilli FIR - Chamoux IR - Chamoux FIR - Champion IR - Champion FIR - Chandesris IR - Chandesris FIR - Chantelat IR - Chantelat FIR - Chantepie IR - Chantepie FIR - Chaplain IR - Chaplain FIR - Chappet IR - Chappet FIR - Charachon IR - Charachon FIR - Charbonneau IR - Charbonneau FIR - Chardon IR - Chardon FIR - Charras IR - Charras FIR - Chenaud IR - Chenaud FIR - Chenel IR - Chenel FIR - Cheron IR - Cheron FIR - Cheuret IR - Cheuret FIR - Chevallier IR - Chevallier FIR - Chevrel IR - Chevrel FIR - Chomienne IR - Chomienne FIR - Chrisment IR - Chrisment FIR - Ciupek IR - Ciupek FIR - Clair IR - Clair FIR - Claris IR - Claris FIR - Cloix IR - Cloix FIR - Coche IR - Coche FIR - Coinde IR - Coinde FIR - Cointin IR - Cointin FIR - Gorski, Colin IR - Gorski C FIR - Collignon IR - Collignon FIR - Colombani IR - Colombani FIR - Combe IR - Combe FIR - Constanty IR - Constanty FIR - Cordier IR - Cordier FIR - Cormier IR - Cormier FIR - Corniau IR - Corniau FIR - Cosson IR - Cosson FIR - Costa IR - Costa FIR - Cottin IR - Cottin FIR - Coumenges IR - Coumenges FIR - Coupe IR - Coupe FIR - Courcol IR - Courcol FIR - Courdavault IR - Courdavault FIR - Courouble IR - Courouble FIR - Courtade IR - Courtade FIR - Craiu IR - Craiu FIR - Crepet IR - Crepet FIR - Croizé IR - Croizé FIR - Cuzzi IR - Cuzzi FIR - D'albignac IR - D'albignac FIR - Dagorne IR - Dagorne FIR - Dalmon IR - Dalmon FIR - Daltroff IR - Daltroff FIR - Danan IR - Danan FIR - Danekova IR - Danekova FIR - Danjean-Deguin IR - Danjean-Deguin FIR - Danjoux IR - Danjoux FIR - Dao-Dubremptz IR - Dao-Dubremptz FIR - Daoud IR - Daoud FIR - Daoudi IR - Daoudi FIR - Darai IR - Darai FIR - Darras IR - Darras FIR - Dassieu IR - Dassieu FIR - Dauger IR - Dauger FIR - Debriel IR - Debriel FIR - Dechamps IR - Dechamps FIR - Decobert IR - Decobert FIR - Decoster IR - Decoster FIR - Decousser IR - Decousser FIR - Deforche IR - Deforche FIR - Degas IR - Degas FIR - Degrange IR - Degrange FIR - Delacour IR - Delacour FIR - Delamare IR - Delamare FIR - Delaporte IR - Delaporte FIR - Delarbre IR - Delarbre FIR - Delattre IR - Delattre FIR - Delavelle IR - Delavelle FIR - Delbeke IR - Delbeke FIR - Delesalle IR - Delesalle FIR - Deligne IR - Deligne FIR - Delisle IR - Delisle FIR - Delvart IR - Delvart FIR - Delorme IR - Delorme FIR - Deluca IR - Deluca FIR - Demachy IR - Demachy FIR - Demarcq IR - Demarcq FIR - Demarque IR - Demarque FIR - Demay-Legros IR - Demay-Legros FIR - Demersay IR - Demersay FIR - Demonchy IR - Demonchy FIR - De Barbentane IR - De Barbentane FIR - De Champs IR - De Champs FIR - De Decker IR - De Decker FIR - De Montclos IR - De Montclos FIR - De Mongolfier IR - De Mongolfier FIR - De Pontual IR - De Pontual FIR - Denis IR - Denis FIR - De Ricaud IR - De Ricaud FIR - Desbois IR - Desbois FIR - Desfrere IR - Desfrere FIR - Desprez IR - Desprez FIR - Dessein IR - Dessein FIR - Dessioux IR - Dessioux FIR - Desvigne IR - Desvigne FIR - Deutscher IR - Deutscher FIR - Devictor IR - Devictor FIR - Deville IR - Deville FIR - Devouge IR - Devouge FIR - Dhaoui IR - Dhaoui FIR - Dieckmann IR - Dieckmann FIR - Dinard, Dit IR - Dinard D FIR - Djafari IR - Djafari FIR - Djaghri IR - Djaghri FIR - Doit IR - Doit FIR - Fiette, Dolfi IR - Fiette D FIR - Dolhem IR - Dolhem FIR - Dommergues IR - Dommergues FIR - Dorangeon IR - Dorangeon FIR - Doucet IR - Doucet FIR - Doucet-Populaire IR - Doucet-Populaire FIR - Douchain IR - Douchain FIR - Dubois IR - Dubois FIR - Dubos IR - Dubos FIR - Dubourdieu IR - Dubourdieu FIR - Dubus IR - Dubus FIR - Duchaine IR - Duchaine FIR - Duchene IR - Duchene FIR - Ducrocq IR - Ducrocq FIR - Dufillot IR - Dufillot FIR - Dufour IR - Dufour FIR - Dugain IR - Dugain FIR - Dumont IR - Dumont FIR - Dumoulard IR - Dumoulard FIR - Dupre IR - Dupre FIR - Duquesne IR - Duquesne FIR - Durand IR - Durand FIR - Duthilly IR - Duthilly FIR - Dutron IR - Dutron FIR - Duval IR - Duval FIR - Arnould, Duval IR - Arnould D FIR - Eb IR - Eb FIR - Eicher IR - Eicher FIR - Eitenschenck IR - Eitenschenck FIR - Hamri, El IR - Hamri E FIR - Elbez IR - Elbez FIR - Elharrif IR - Elharrif FIR - Elias IR - Elias FIR - Emond IR - Emond FIR - Enchery IR - Enchery FIR - Enoch IR - Enoch FIR - Epaud IR - Epaud FIR - Escarguel IR - Escarguel FIR - Estapa IR - Estapa FIR - Esteve IR - Esteve FIR - Mathiaud, Estournet IR - Mathiaud E FIR - Estrangin IR - Estrangin FIR - Etienne IR - Etienne FIR - Evers IR - Evers FIR - Evrard IR - Evrard FIR - Evreux IR - Evreux FIR - Eyer IR - Eyer FIR - Eyssette-Gayraud IR - Eyssette-Gayraud FIR - Ezzedine IR - Ezzedine FIR - Fabbro IR - Fabbro FIR - Faibis IR - Faibis FIR - Fargeot IR - Fargeot FIR - Farges IR - Farges FIR - Farhat IR - Farhat FIR - Farrugia IR - Farrugia FIR - Fasquelle IR - Fasquelle FIR - Faul IR - Faul FIR - Favaretto IR - Favaretto FIR - Faye IR - Faye FIR - Feldmann IR - Feldmann FIR - Ferey IR - Ferey FIR - Ferroni IR - Ferroni FIR - Fevre IR - Fevre FIR - Fieschi IR - Fieschi FIR - Fiette IR - Fiette FIR - Fihman IR - Fihman FIR - Filippi IR - Filippi FIR - Fischbach IR - Fischbach FIR - Flevin IR - Flevin FIR - Flipo IR - Flipo FIR - Flurin IR - Flurin FIR - Forget IR - Forget FIR - Fortin IR - Fortin FIR - Fos IR - Fos FIR - Foskett IR - Foskett FIR - Foucaud IR - Foucaud FIR - Franc IR - Franc FIR - Francois-Chervet IR - Francois-Chervet FIR - Francoise IR - Francoise FIR - Frey IR - Frey FIR - Gagliardone IR - Gagliardone FIR - Gaillard IR - Gaillard FIR - Gallet IR - Gallet FIR - Gallou IR - Gallou FIR - Ganivala IR - Ganivala FIR - Garandeau IR - Garandeau FIR - Garbarg-Chenon IR - Garbarg-Chenon FIR - Garcera IR - Garcera FIR - Garnier IR - Garnier FIR - Garrec IR - Garrec FIR - Gaschet IR - Gaschet FIR - Gaschignard IR - Gaschignard FIR - Gaudelus IR - Gaudelus FIR - Gauduchon IR - Gauduchon FIR - Gaugler IR - Gaugler FIR - Gavignet IR - Gavignet FIR - Gbadamassi IR - Gbadamassi FIR - Geffroy IR - Geffroy FIR - Gendrel IR - Gendrel FIR - Geniez IR - Geniez FIR - Georget IR - Georget FIR - Geraudel IR - Geraudel FIR - Gerony-Laffitte IR - Gerony-Laffitte FIR - Gilles IR - Gilles FIR - Gillet IR - Gillet FIR - Giorgi IR - Giorgi FIR - Girard IR - Girard FIR - Gire IR - Gire FIR - Girier IR - Girier FIR - Glastre IR - Glastre FIR - Glatz IR - Glatz FIR - Gleize IR - Gleize FIR - Goguelin IR - Goguelin FIR - Goissen IR - Goissen FIR - Goldstein IR - Goldstein FIR - Goudeau IR - Goudeau FIR - Gougeon IR - Gougeon FIR - Goumy IR - Goumy FIR - Gouraud IR - Gouraud FIR - Gouriet IR - Gouriet FIR - Goux IR - Goux FIR - Graber IR - Graber FIR - Graff IR - Graff FIR - Grailles IR - Grailles FIR - Graine IR - Graine FIR - Grancher IR - Grancher FIR - Grando IR - Grando FIR - Granier IR - Granier FIR - Granry IR - Granry FIR - Greco IR - Greco FIR - Gremeaux IR - Gremeaux FIR - Gremillet IR - Gremillet FIR - Gressier IR - Gressier FIR - Grimprel IR - Grimprel FIR - Grise IR - Grise FIR - Grollmuss IR - Grollmuss FIR - Guerin IR - Guerin FIR - Guibert IR - Guibert FIR - Guichard IR - Guichard FIR - Guiet IR - Guiet FIR - Guigonis IR - Guigonis FIR - Guilbert IR - Guilbert FIR - Baudet, Guillaume IR - Baudet G FIR - Fromentin, Guillermet IR - Fromentin G FIR - Guillois IR - Guillois FIR - Guillot IR - Guillot FIR - Guilluy IR - Guilluy FIR - Guitton IR - Guitton FIR - Guyon IR - Guyon FIR - Haas IR - Haas FIR - Hachani IR - Hachani FIR - Hachart IR - Hachart FIR - Hachem IR - Hachem FIR - Haegy-Doehring IR - Haegy-Doehring FIR - Hage IR - Hage FIR - Halfatuhi IR - Halfatuhi FIR - Hallage IR - Hallage FIR - Hallalel IR - Hallalel FIR - Halna IR - Halna FIR - Halphen IR - Halphen FIR - Hamdad IR - Hamdad FIR - Haouisee IR - Haouisee FIR - Harchaoui IR - Harchaoui FIR - Hasselmann IR - Hasselmann FIR - Hautefort IR - Hautefort FIR - Hedjem IR - Hedjem FIR - Hees IR - Hees FIR - Heidt IR - Heidt FIR - Hentgen IR - Hentgen FIR - Heraud IR - Heraud FIR - Herve IR - Herve FIR - Heurte IR - Heurte FIR - Heusse IR - Heusse FIR - Martin, Hevin IR - Martin H FIR - Heyman IR - Heyman FIR - Hidri IR - Hidri FIR - Hochart IR - Hochart FIR - Hoffmann IR - Hoffmann FIR - Hombrouck-Alet IR - Hombrouck-Alet FIR - Honore IR - Honore FIR - Horea IR - Horea FIR - Hubert IR - Hubert FIR - Hudebine IR - Hudebine FIR - Huet IR - Huet FIR - Huguet IR - Huguet FIR - Huin IR - Huin FIR - Hureaux IR - Hureaux FIR - Huvenne IR - Huvenne FIR - Ikounga IR - Ikounga FIR - Issa-Brunet IR - Issa-Brunet FIR - Izopet IR - Izopet FIR - Jacob IR - Jacob FIR - Jalloul IR - Jalloul FIR - Jan IR - Jan FIR - Jaouen IR - Jaouen FIR - Jarlier IR - Jarlier FIR - Jarreau IR - Jarreau FIR - Jaulhac IR - Jaulhac FIR - Javouhey IR - Javouhey FIR - Jeannoel IR - Jeannoel FIR - Jeannot IR - Jeannot FIR - Jehan IR - Jehan FIR - Jensen IR - Jensen FIR - Jeny IR - Jeny FIR - Jeudy IR - Jeudy FIR - Jokic IR - Jokic FIR - Joly-Guillou IR - Joly-Guillou FIR - Joly-Sanchez IR - Joly-Sanchez FIR - Joram IR - Joram FIR - Jorion IR - Jorion FIR - Julienne IR - Julienne FIR - Jullian IR - Jullian FIR - Juvin IR - Juvin FIR - Kalach IR - Kalach FIR - Kalkas IR - Kalkas FIR - Kayal IR - Kayal FIR - Kayemba IR - Kayemba FIR - Khaled IR - Khaled FIR - Khalfi IR - Khalfi FIR - Khazaal IR - Khazaal FIR - Khorsi IR - Khorsi FIR - Kitzis IR - Kitzis FIR - Klein IR - Klein FIR - Klink IR - Klink FIR - Klosowski IR - Klosowski FIR - Kone-Paut IR - Kone-Paut FIR - Kouame IR - Kouame FIR - Kovacs IR - Kovacs FIR - Kozisek IR - Kozisek FIR - Kretz IR - Kretz FIR - Kucerova IR - Kucerova FIR - Kuntzel IR - Kuntzel FIR - Labarthe IR - Labarthe FIR - Labbe IR - Labbe FIR - Labenne IR - Labenne FIR - Laborie IR - Laborie FIR - Labrune IR - Labrune FIR - Lafargue IR - Lafargue FIR - Lafendi IR - Lafendi FIR - Lagier IR - Lagier FIR - Lagree IR - Lagree FIR - Lahrach IR - Lahrach FIR - Laidj IR - Laidj FIR - Laisney IR - Laisney FIR - Lakhdari IR - Lakhdari FIR - Lamarcq IR - Lamarcq FIR - Lambert IR - Lambert FIR - Lamoureux IR - Lamoureux FIR - Landragin IR - Landragin FIR - Lanotte IR - Lanotte FIR - Lapeyre IR - Lapeyre FIR - Larchet IR - Larchet FIR - Laugel IR - Laugel FIR - Launay IR - Launay FIR - Lavigne IR - Lavigne FIR - Layadi IR - Layadi FIR - Lazarro IR - Lazarro FIR - Le Bail IR - Le Bail FIR - Le Bideau IR - Le Bideau FIR - Le Coustumier IR - Le Coustumier FIR - Le Galloudec IR - Le Galloudec FIR - Le Luyer IR - Le Luyer FIR - Leblanc IR - Leblanc FIR - Leboucher IR - Leboucher FIR - Lebrun IR - Lebrun FIR - Lecarpentier IR - Lecarpentier FIR - Lecine IR - Lecine FIR - Leclerc IR - Leclerc FIR - Leclercq IR - Leclercq FIR - Lecomte IR - Lecomte FIR - Ledru IR - Ledru FIR - Lefevre IR - Lefevre FIR - Lefrand-Crepin IR - Lefrand-Crepin FIR - Legagneur IR - Legagneur FIR - Legros IR - Legros FIR - Leheup IR - Leheup FIR - Lehnert IR - Lehnert FIR - Lehours IR - Lehours FIR - Lejri IR - Lejri FIR - Lelioux IR - Lelioux FIR - Lelorier IR - Lelorier FIR - Leluan IR - Leluan FIR - Lemarié IR - Lemarié FIR - Lemble IR - Lemble FIR - Lemeland IR - Lemeland FIR - Lemonnier IR - Lemonnier FIR - Lenclen IR - Lenclen FIR - Leneveu IR - Leneveu FIR - Leotard IR - Leotard FIR - Lepage IR - Lepage FIR - Leret IR - Leret FIR - Lesage IR - Lesage FIR - Lescanne IR - Lescanne FIR - Letellier IR - Letellier FIR - Leturdu IR - Leturdu FIR - Levy IR - Levy FIR - Lienhardt IR - Lienhardt FIR - Lorrot IR - Lorrot FIR - Louf IR - Louf FIR - Lounis IR - Lounis FIR - Loznewski IR - Loznewski FIR - Lureau IR - Lureau FIR - M'bamba IR - M'bamba FIR - Maakaroun-Vermesse IR - Maakaroun-Vermesse FIR - Macchi IR - Macchi FIR - Madhi IR - Madhi FIR - Mager IR - Mager FIR - Magrhraoui IR - Magrhraoui FIR - Mahisatra IR - Mahisatra FIR - Maisonneuve IR - Maisonneuve FIR - Malbrunot IR - Malbrunot FIR - Malige IR - Malige FIR - Mallet IR - Mallet FIR - Mammeri IR - Mammeri FIR - Mammes IR - Mammes FIR - Mancini IR - Mancini FIR - Mandjee IR - Mandjee FIR - Mangeol IR - Mangeol FIR - Manin IR - Manin FIR - Mansir IR - Mansir FIR - Manteau IR - Manteau FIR - Marani IR - Marani FIR - Marchand IR - Marchand FIR - Marcou IR - Marcou FIR - Marguet IR - Marguet FIR - Mariette IR - Mariette FIR - Marin IR - Marin FIR - Marmonier IR - Marmonier FIR - Marmouset IR - Marmouset FIR - Marret IR - Marret FIR - Martha IR - Martha FIR - Martin IR - Martin FIR - Martinat IR - Martinat FIR - Ozenda, Martinez IR - Ozenda M FIR - Martinot IR - Martinot FIR - Marty IR - Marty FIR - Mathieu IR - Mathieu FIR - Matray IR - Matray FIR - Maugard IR - Maugard FIR - Maurage IR - Maurage FIR - Maurin IR - Maurin FIR - May IR - May FIR - Mazataud IR - Mazataud FIR - Mellier IR - Mellier FIR - Melon IR - Melon FIR - Menager IR - Menager FIR - Menget IR - Menget FIR - Menouar IR - Menouar FIR - Menouni-Foray IR - Menouni-Foray FIR - Merlot IR - Merlot FIR - Jeanvoine, Mermet IR - Jeanvoine M FIR - Meslin IR - Meslin FIR - Meunier IR - Meunier FIR - Meyer IR - Meyer FIR - Mezgueldi IR - Mezgueldi FIR - Micaelo IR - Micaelo FIR - Mignard IR - Mignard FIR - Mignot IR - Mignot FIR - Mikail IR - Mikail FIR - Milcent IR - Milcent FIR - Milh IR - Milh FIR - Milleret-Proyart IR - Milleret-Proyart FIR - Mitanchez IR - Mitanchez FIR - Mondaud IR - Mondaud FIR - Monier IR - Monier FIR - Monin IR - Monin FIR - Montagnon IR - Montagnon FIR - Moquet IR - Moquet FIR - Morales-Gineste IR - Morales-Gineste FIR - Moreigne IR - Moreigne FIR - Morice IR - Morice FIR - Moriette IR - Moriette FIR - Morin IR - Morin FIR - Morisot IR - Morisot FIR - Morville IR - Morville FIR - Motte IR - Motte FIR - Mougin-Joubert IR - Mougin-Joubert FIR - Moulene IR - Moulene FIR - Moulin IR - Moulin FIR - Mouly IR - Mouly FIR - Mounzer IR - Mounzer FIR - Mselati IR - Mselati FIR - Muller IR - Muller FIR - Muller IR - Muller FIR - Murbach IR - Murbach FIR - Nacer IR - Nacer FIR - Naudion IR - Naudion FIR - Naudot IR - Naudot FIR - Navarro IR - Navarro FIR - Nelson IR - Nelson FIR - Schiavini, Nerl IR - Schiavini N FIR - Nerome IR - Nerome FIR - Netter IR - Netter FIR - Neuwirth IR - Neuwirth FIR - Nevine IR - Nevine FIR - Nguyen IR - Nguyen FIR - Nicola IR - Nicola FIR - Nordmann IR - Nordmann FIR - Noulard IR - Noulard FIR - Nsimba IR - Nsimba FIR - Oprea IR - Oprea FIR - Orzechowski IR - Orzechowski FIR - Osman IR - Osman FIR - Hocine, Ould IR - Hocine O FIR - Oules IR - Oules FIR - Ozanne IR - Ozanne FIR - Pages IR - Pages FIR - Paget IR - Paget FIR - Palette IR - Palette FIR - Pancher IR - Pancher FIR - Pangon IR - Pangon FIR - Pannecouck IR - Pannecouck FIR - Paon IR - Paon FIR - Duchene, Parisi IR - Duchene P FIR - Pateyron IR - Pateyron FIR - Patoz IR - Patoz FIR - Paul IR - Paul FIR - Paut IR - Paut FIR - Pejoan IR - Pejoan FIR - Pellerin IR - Pellerin FIR - Pelloux IR - Pelloux FIR - Penaud IR - Penaud FIR - Penel-Capelle IR - Penel-Capelle FIR - Peralta IR - Peralta FIR - Pere IR - Pere FIR - Perez IR - Perez FIR - Perrin IR - Perrin FIR - Perroud IR - Perroud FIR - Pesenti IR - Pesenti FIR - Petit IR - Petit FIR - Petitboulanger IR - Petitboulanger FIR - Phan IR - Phan FIR - Philippon IR - Philippon FIR - Picherot IR - Picherot FIR - Pierre IR - Pierre FIR - Pina IR - Pina FIR - Pierrejean IR - Pierrejean FIR - Pietrement IR - Pietrement FIR - Pigeon IR - Pigeon FIR - Pina IR - Pina FIR - Pinchi IR - Pinchi FIR - Pindi IR - Pindi FIR - Pinquier IR - Pinquier FIR - Pinturier IR - Pinturier FIR - Pladys IR - Pladys FIR - Planchenault IR - Planchenault FIR - Plassart IR - Plassart FIR - Plasse IR - Plasse FIR - Plouvier IR - Plouvier FIR - Poilane IR - Poilane FIR - Poilly IR - Poilly FIR - Pollet IR - Pollet FIR - Pons IR - Pons FIR - Popelard IR - Popelard FIR - Porcheret IR - Porcheret FIR - Poulain IR - Poulain FIR - Poyard IR - Poyard FIR - Poynard IR - Poynard FIR - Pradeaux IR - Pradeaux FIR - Prere IR - Prere FIR - Priqueler IR - Priqueler FIR - Prudent IR - Prudent FIR - Py IR - Py FIR - Queinnec IR - Queinnec FIR - Questiaux IR - Questiaux FIR - Quinet IR - Quinet FIR - Raber IR - Raber FIR - Rafin IR - Rafin FIR - Raignoux IR - Raignoux FIR - Raoult IR - Raoult FIR - Rault IR - Rault FIR - Raymond IR - Raymond FIR - Raynaud IR - Raynaud FIR - Rebaud IR - Rebaud FIR - Renault IR - Renault FIR - Vialet, Renaud IR - Vialet R FIR - Rennes IR - Rennes FIR - Renolleau IR - Renolleau FIR - Retali IR - Retali FIR - Retornaz IR - Retornaz FIR - Rey IR - Rey FIR - Reynaud IR - Reynaud FIR - Richardin IR - Richardin FIR - Rimet IR - Rimet FIR - Rimpici IR - Rimpici FIR - Rio IR - Rio FIR - Rivaux IR - Rivaux FIR - Riviere IR - Riviere FIR - Roche IR - Roche FIR - Rodiere IR - Rodiere FIR - Rolland IR - Rolland FIR - Romain IR - Romain FIR - Romeo IR - Romeo FIR - Rosellini IR - Rosellini FIR - Rossignol IR - Rossignol FIR - Roth IR - Roth FIR - Rottman IR - Rottman FIR - Roubin IR - Roubin FIR - Roudaut IR - Roudaut FIR - Roudière IR - Roudière FIR - Rougeoreille IR - Rougeoreille FIR - Rougier IR - Rougier FIR - Roullaud IR - Roullaud FIR - Rousseau IR - Rousseau FIR - Roussellier IR - Roussellier FIR - Roux IR - Roux FIR - Roybet IR - Roybet FIR - Roze IR - Roze FIR - Sachs IR - Sachs FIR - Sadik IR - Sadik FIR - Sadki IR - Sadki FIR - Saf IR - Saf FIR - Said-Menthon IR - Said-Menthon FIR - Sakr IR - Sakr FIR - Saliba IR - Saliba FIR - Salomon IR - Salomon FIR - Sanchez IR - Sanchez FIR - Santerne IR - Santerne FIR - Sanyas IR - Sanyas FIR - Sarlangue IR - Sarlangue FIR - Sarthou IR - Sarthou FIR - Sartre IR - Sartre FIR - Saumureau IR - Saumureau FIR - Saunier IR - Saunier FIR - Savoy IR - Savoy FIR - Scanvic IR - Scanvic FIR - Scat IR - Scat FIR - Schaefer IR - Schaefer FIR - Schneider IR - Schneider FIR - Seaume IR - Seaume FIR - Sebag IR - Sebag FIR - Secher IR - Secher FIR - See IR - See FIR - Sevin IR - Sevin FIR - Sfez IR - Sfez FIR - Simonin IR - Simonin FIR - Siouala IR - Siouala FIR - Sirot IR - Sirot FIR - Sivadon-Tardy IR - Sivadon-Tardy FIR - Smati IR - Smati FIR - Smets IR - Smets FIR - Sommabere IR - Sommabere FIR - Soto IR - Soto FIR - Soustelle IR - Soustelle FIR - Spicq IR - Spicq FIR - Stefaniuk IR - Stefaniuk FIR - Stephan IR - Stephan FIR - Sweertvaegher IR - Sweertvaegher FIR - Tahiri IR - Tahiri FIR - Tarral IR - Tarral FIR - Tchanga IR - Tchanga FIR - Terki IR - Terki FIR - Texier IR - Texier FIR - Therond IR - Therond FIR - Thevenot IR - Thevenot FIR - Thibault IR - Thibault FIR - Thiriez IR - Thiriez FIR - Thore IR - Thore FIR - Thouvenin IR - Thouvenin FIR - Tiry IR - Tiry FIR - Tixier IR - Tixier FIR - Tommasi IR - Tommasi FIR - Toubiana IR - Toubiana FIR - Touroult-Jupin IR - Touroult-Jupin FIR - Tourrand IR - Tourrand FIR - Tran IR - Tran FIR - Trioche IR - Trioche FIR - Troller IR - Troller FIR - Tronc IR - Tronc FIR - Trouilloud IR - Trouilloud FIR - Tuypens IR - Tuypens FIR - Tytgat IR - Tytgat FIR - Ursulescu IR - Ursulescu FIR - Uzan IR - Uzan FIR - Vachee IR - Vachee FIR - Vaillant IR - Vaillant FIR - Valade IR - Valade FIR - Valayer IR - Valayer FIR - Valensi IR - Valensi FIR - Vallee IR - Vallee FIR - Vallet IR - Vallet FIR - Vandenesch IR - Vandenesch FIR - Van de Perre IR - Van de Perre FIR - Contamin, Vanel IR - Contamin V FIR - Varengue IR - Varengue FIR - Vasselon-Raina IR - Vasselon-Raina FIR - Vaucel IR - Vaucel FIR - Venot IR - Venot FIR - Verdan IR - Verdan FIR - Verdet IR - Verdet FIR - Vergerond IR - Vergerond FIR - Vergnaud IR - Vergnaud FIR - Vergne IR - Vergne FIR - Vernet-Garnier IR - Vernet-Garnier FIR - Vervel IR - Vervel FIR - Viala IR - Viala FIR - Vialette IR - Vialette FIR - Vic IR - Vic FIR - Vignaud IR - Vignaud FIR - Vigneron IR - Vigneron FIR - Villeneuve IR - Villeneuve FIR - Violette IR - Violette FIR - Vittecoq IR - Vittecoq FIR - Vodoff IR - Vodoff FIR - Vogt IR - Vogt FIR - Vorlet IR - Vorlet FIR - Vray IR - Vray FIR - Vrillon IR - Vrillon FIR - Thien, Vu IR - Thien V FIR - Warin IR - Warin FIR - Wasels IR - Wasels FIR - Weber IR - Weber FIR - Weisse IR - Weisse FIR - Wemeau IR - Wemeau FIR - Worcel IR - Worcel FIR - Ting, Yang IR - Ting Y FIR - Ygout IR - Ygout FIR - Youssef IR - Youssef FIR - Ythier IR - Ythier FIR - Zaoui IR - Zaoui FIR - Zarzour IR - Zarzour FIR - Zehani IR - Zehani FIR - Zelinsky-Gurung IR - Zelinsky-Gurung FIR - Zenkhri IR - Zenkhri FIR - Zimmermann IR - Zimmermann FIR - Zitvogel IR - Zitvogel FIR - Zumbo IR - Zumbo EDAT- 2021/05/30 06:00 MHDA- 2021/09/22 06:00 PMCR- 2021/05/28 CRDT- 2021/05/29 05:28 PHST- 2021/04/22 00:00 [received] PHST- 2021/05/12 00:00 [accepted] PHST- 2021/05/29 05:28 [entrez] PHST- 2021/05/30 06:00 [pubmed] PHST- 2021/09/22 06:00 [medline] PHST- 2021/05/28 00:00 [pmc-release] AID - 10.1186/s13054-021-03603-8 [pii] AID - 3603 [pii] AID - 10.1186/s13054-021-03603-8 [doi] PST - epublish SO - Crit Care. 2021 May 28;25(1):181. doi: 10.1186/s13054-021-03603-8. PMID- 32109418 OWN - NLM STAT- MEDLINE DCOM- 20200505 LR - 20210604 IS - 1537-6605 (Electronic) IS - 0002-9297 (Print) IS - 0002-9297 (Linking) VI - 106 IP - 3 DP - 2020 Mar 5 TI - Evaluation of DNA Methylation Episignatures for Diagnosis and Phenotype Correlations in 42 Mendelian Neurodevelopmental Disorders. PG - 356-370 LID - S0002-9297(20)30019-7 [pii] LID - 10.1016/j.ajhg.2020.01.019 [doi] AB - Genetic syndromes frequently present with overlapping clinical features and inconclusive or ambiguous genetic findings which can confound accurate diagnosis and clinical management. An expanding number of genetic syndromes have been shown to have unique genomic DNA methylation patterns (called "episignatures"). Peripheral blood episignatures can be used for diagnostic testing as well as for the interpretation of ambiguous genetic test results. We present here an approach to episignature mapping in 42 genetic syndromes, which has allowed the identification of 34 robust disease-specific episignatures. We examine emerging patterns of overlap, as well as similarities and hierarchical relationships across these episignatures, to highlight their key features as they are related to genetic heterogeneity, dosage effect, unaffected carrier status, and incomplete penetrance. We demonstrate the necessity of multiclass modeling for accurate genetic variant classification and show how disease classification using a single episignature at a time can sometimes lead to classification errors in closely related episignatures. We demonstrate the utility of this tool in resolving ambiguous clinical cases and identification of previously undiagnosed cases through mass screening of a large cohort of subjects with developmental delays and congenital anomalies. This study more than doubles the number of published syndromes with DNA methylation episignatures and, most significantly, opens new avenues for accurate diagnosis and clinical assessment in individuals affected by these disorders. CI - Copyright © 2020 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved. FAU - Aref-Eshghi, Erfan AU - Aref-Eshghi E AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada. FAU - Kerkhof, Jennifer AU - Kerkhof J AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada. FAU - Pedro, Victor P AU - Pedro VP AD - Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON N6A5C1, Canada. CN - Groupe DI France AD - Groupe DI, French Rare Disease Network filière AnDDI-Rare France. FAU - Barat-Houari, Mouna AU - Barat-Houari M AD - Autoinflammatory and Rare Diseases Unit, Medical Genetic Department for Rare Diseases and Personalized Medicine, Centre Hospitalier Universitaire de Montpellier, 34090 Montpellier, France. FAU - Ruiz-Pallares, Nathalie AU - Ruiz-Pallares N AD - Autoinflammatory and Rare Diseases Unit, Medical Genetic Department for Rare Diseases and Personalized Medicine, Centre Hospitalier Universitaire de Montpellier, 34090 Montpellier, France. FAU - Andrau, Jean-Christophe AU - Andrau JC AD - Institut de Génétique Moléculaire de Montpellier (IGMM), University Montpellier, CNRS-UMR5535, 34090 Montpellier, France. FAU - Lacombe, Didier AU - Lacombe D AD - Medical Genetics Department, Inserm U1211, Reference Center AD SOOR, AnDDI-RARE, Bordeaux University, Centre Hospitalier Universitaire de Bordeaux, 33076 Bordeaux, France. FAU - Van-Gils, Julien AU - Van-Gils J AD - Medical Genetics Department, Inserm U1211, Reference Center AD SOOR, AnDDI-RARE, Bordeaux University, Centre Hospitalier Universitaire de Bordeaux, 33076 Bordeaux, France. FAU - Fergelot, Patricia AU - Fergelot P AD - Medical Genetics Department, Inserm U1211, Reference Center AD SOOR, AnDDI-RARE, Bordeaux University, Centre Hospitalier Universitaire de Bordeaux, 33076 Bordeaux, France. FAU - Dubourg, Christèle AU - Dubourg C AD - Service de Génétique Moléculaire et Génomique, Centre Hospitalier Universitaire de Rennes, 35000 Rennes, France. FAU - Cormier-Daire, Valerie AU - Cormier-Daire V AD - Department of Medical Genetics, Paris Descartes University, INSERM UMR 1163, Imagine Institute, Necker Enfants Malades Hospital, 75015 Paris, France. FAU - Rondeau, Sophie AU - Rondeau S AD - Department of Medical Genetics, Paris Descartes University, INSERM UMR 1163, Imagine Institute, Necker Enfants Malades Hospital, 75015 Paris, France. FAU - Lecoquierre, François AU - Lecoquierre F AD - Normandie University, UNIROUEN, Inserm U1245 and Rouen University Hospital, Department of Genetics and Reference Center for Developmental Disorders, F 76000, Normandy Center for Genomic and Personalized Medicine, 76183 Rouen, France. FAU - Saugier-Veber, Pascale AU - Saugier-Veber P AD - Normandie University, UNIROUEN, Inserm U1245 and Rouen University Hospital, Department of Genetics and Reference Center for Developmental Disorders, F 76000, Normandy Center for Genomic and Personalized Medicine, 76183 Rouen, France. FAU - Nicolas, Gaël AU - Nicolas G AD - Normandie University, UNIROUEN, Inserm U1245 and Rouen University Hospital, Department of Genetics and Reference Center for Developmental Disorders, F 76000, Normandy Center for Genomic and Personalized Medicine, 76183 Rouen, France. FAU - Lesca, Gaetan AU - Lesca G AD - Department of Medical Genetics, University Hospital of Lyon, 69007 Lyon, France. FAU - Chatron, Nicolas AU - Chatron N AD - Department of Medical Genetics, University Hospital of Lyon, 69007 Lyon, France. FAU - Sanlaville, Damien AU - Sanlaville D AD - Department of Medical Genetics, University Hospital of Lyon, 69007 Lyon, France. FAU - Vitobello, Antonio AU - Vitobello A AD - Inserm, UMR1231, Equipe GAD, Bâtiment B3, Université de Bourgogne Franche Comté, 15 boulevard du Maréchal de Lattre de Tassigny, 21000, Dijon Cedex, France; Unité Fonctionnelle Innovation en Diagnostic génomique des maladies rares, FHU-TRANSLAD, Dijon University Hospital, 21000 Dijon, France. FAU - Faivre, Laurence AU - Faivre L AD - Inserm, UMR1231, Equipe GAD, Bâtiment B3, Université de Bourgogne Franche Comté, 15 boulevard du Maréchal de Lattre de Tassigny, 21000, Dijon Cedex, France; Centre de Référence Déficiences Intellectuelles de Causes Rares, CHU Dijon, 21000, Dijon, France. FAU - Thauvin-Robinet, Christel AU - Thauvin-Robinet C AD - Inserm, UMR1231, Equipe GAD, Bâtiment B3, Université de Bourgogne Franche Comté, 15 boulevard du Maréchal de Lattre de Tassigny, 21000, Dijon Cedex, France; Centre de Référence Déficiences Intellectuelles de Causes Rares, CHU Dijon, 21000, Dijon, France. FAU - Laumonnier, Frederic AU - Laumonnier F AD - UMR 1253, iBrain, Universite de Tours, Inserm, 37200 Tours, France; Centre Hospitalier Universitaire de Tours, Service de Genetique, 37000 Tours, France. FAU - Raynaud, Martine AU - Raynaud M AD - UMR 1253, iBrain, Universite de Tours, Inserm, 37200 Tours, France; Centre Hospitalier Universitaire de Tours, Service de Genetique, 37000 Tours, France. FAU - Alders, Mariëlle AU - Alders M AD - Amsterdam University Medical Center, University of Amsterdam, Department of Clinical Genetics, Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands. FAU - Mannens, Marcel AU - Mannens M AD - Amsterdam University Medical Center, University of Amsterdam, Department of Clinical Genetics, Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands. FAU - Henneman, Peter AU - Henneman P AD - Amsterdam University Medical Center, University of Amsterdam, Department of Clinical Genetics, Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands. FAU - Hennekam, Raoul C AU - Hennekam RC AD - Department of Pediatrics, Academic Medical Center, University of Amsterdam, Amsterdam, 1012 WX, the Netherlands. FAU - Velasco, Guillaume AU - Velasco G AD - Université de Paris, Epigénétique et Destin Cellulaire, CNRS, 75013 Paris, France. FAU - Francastel, Claire AU - Francastel C AD - Université de Paris, Epigénétique et Destin Cellulaire, CNRS, 75013 Paris, France. FAU - Ulveling, Damien AU - Ulveling D AD - Université de Paris, Epigénétique et Destin Cellulaire, CNRS, 75013 Paris, France. FAU - Ciolfi, Andrea AU - Ciolfi A AD - Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00146 Rome, Italy. FAU - Pizzi, Simone AU - Pizzi S AD - Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00146 Rome, Italy. FAU - Tartaglia, Marco AU - Tartaglia M AD - Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00146 Rome, Italy. FAU - Heide, Solveig AU - Heide S AD - Department of Genetics, Referral Center for Intellectual Disabilities, APHP Sorbonne University, Pitié Salpêtrière Hospital, 75013 Paris, France. FAU - Héron, Delphine AU - Héron D AD - Department of Genetics, Referral Center for Intellectual Disabilities, APHP Sorbonne University, Pitié Salpêtrière Hospital, 75013 Paris, France. FAU - Mignot, Cyril AU - Mignot C AD - Department of Genetics, Referral Center for Intellectual Disabilities, APHP Sorbonne University, Pitié Salpêtrière Hospital, 75013 Paris, France. FAU - Keren, Boris AU - Keren B AD - Department of Genetics, Referral Center for Intellectual Disabilities, APHP Sorbonne University, Pitié Salpêtrière Hospital, 75013 Paris, France. FAU - Whalen, Sandra AU - Whalen S AD - Unit of Genetics, APHP Sorbonne University, Trousseau Hospital, 75012 Paris, France. FAU - Afenjar, Alexandra AU - Afenjar A AD - Unit of Genetics, APHP Sorbonne University, Trousseau Hospital, 75012 Paris, France. FAU - Bienvenu, Thierry AU - Bienvenu T AD - Department of Molecular Genetics, Cochin Hospital, 75014 Paris, France. FAU - Campeau, Philippe M AU - Campeau PM AD - Department of Pediatrics, University of Montreal, Montreal, QC H3T1J4, Canada. FAU - Rousseau, Justine AU - Rousseau J AD - Department of Pediatrics, University of Montreal, Montreal, QC H3T1J4, Canada. FAU - Levy, Michael A AU - Levy MA AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada; Department of Pathology and Laboratory Medicine, Western University, London, ON N6A3K7, Canada. FAU - Brick, Lauren AU - Brick L AD - Genetic Division, Department of Pediatrics, McMaster University, Hamilton, ON L8S4K1, Canada. FAU - Kozenko, Mariya AU - Kozenko M AD - Genetic Division, Department of Pediatrics, McMaster University, Hamilton, ON L8S4K1, Canada. FAU - Balci, Tugce B AU - Balci TB AD - Department of Pediatrics, Division of Medical Genetics, Western University, London, ON N6A 3K7; Medical Genetics Program of Southwestern Ontario, London Health Sciences Centre, London, ON N6A5W9, Canada. FAU - Siu, Victoria Mok AU - Siu VM AD - Department of Pediatrics, Division of Medical Genetics, Western University, London, ON N6A 3K7; Medical Genetics Program of Southwestern Ontario, London Health Sciences Centre, London, ON N6A5W9, Canada. FAU - Stuart, Alan AU - Stuart A AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada. FAU - Kadour, Mike AU - Kadour M AD - Department of Pathology and Laboratory Medicine, London Health Sciences Centre, London, ON N6A5W9 Canada; St. Joseph's Health Care London, London, ON N6A5W9 Canada. FAU - Masters, Jennifer AU - Masters J AD - Department of Pathology and Laboratory Medicine, London Health Sciences Centre, London, ON N6A5W9 Canada; St. Joseph's Health Care London, London, ON N6A5W9 Canada. FAU - Takano, Kyoko AU - Takano K AD - Center for Medical Genetics, Shinshu University Hospital, 3-1-1 Asahi, Matsumoto, Nagano 3908621, Japan. FAU - Kleefstra, Tjitske AU - Kleefstra T AD - Department of Human Genetics, Radboud University Medical Center, 6525 GA Nijmegen, the Netherlands; Donders Center for Medical Neuroscience, 6525 GA Nijmegen, the Netherlands. FAU - de Leeuw, Nicole AU - de Leeuw N AD - Department of Human Genetics, Radboud University Medical Center, 6525 GA Nijmegen, the Netherlands; Donders Center for Medical Neuroscience, 6525 GA Nijmegen, the Netherlands. FAU - Field, Michael AU - Field M AD - Genetics of Learning Disability Service, Hunter Genetics, Waratah, NSW 2298, Australia. FAU - Shaw, Marie AU - Shaw M AD - School of Medicine, Robinson Research Institute, University of Adelaide, Adelaide, SA 5005, Australia. FAU - Gecz, Jozef AU - Gecz J AD - School of Medicine, Robinson Research Institute, University of Adelaide, Adelaide, SA 5005, Australia; South Australian Health and Medical Research Institute, Adelaide, SA 5005, Australia. FAU - Ainsworth, Peter J AU - Ainsworth PJ AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada; Department of Pathology and Laboratory Medicine, Western University, London, ON N6A3K7, Canada. FAU - Lin, Hanxin AU - Lin H AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada; Department of Pathology and Laboratory Medicine, Western University, London, ON N6A3K7, Canada. FAU - Rodenhiser, David I AU - Rodenhiser DI AD - Children's Health Research Institute, London, ON N6A3K7, Canada; Department of Biochemistry, Western University, London, ON N6A3K7, Canada. FAU - Friez, Michael J AU - Friez MJ AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Tedder, Matt AU - Tedder M AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Lee, Jennifer A AU - Lee JA AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - DuPont, Barbara R AU - DuPont BR AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Stevenson, Roger E AU - Stevenson RE AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Skinner, Steven A AU - Skinner SA AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Schwartz, Charles E AU - Schwartz CE AD - Greenwood Genetic Center, Greenwood, SC 29646, USA. FAU - Genevieve, David AU - Genevieve D AD - Medical Genetic Department for Rare Diseases and Personalized Medicine, Reference Center AD SOOR, AnDDI-RARE, Groupe DI, Inserm U1183-Institute for Regenerative Medicine and Biotherapy, Montpellier University, Centre Hospitalier Universitaire de Montpellier, 34090 Montpellier, France. FAU - Sadikovic, Bekim AU - Sadikovic B AD - Molecular Genetics Laboratory, Molecular Diagnostics Division, London Health Sciences Centre, London, ON N6A5W9, Canada; Department of Pathology and Laboratory Medicine, Western University, London, ON N6A3K7, Canada. Electronic address: Bekim.Sadikovic@lhsc.on.ca. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200227 PL - United States TA - Am J Hum Genet JT - American journal of human genetics JID - 0370475 SB - IM EIN - Am J Hum Genet. 2021 Jun 3;108(6):1161-1163. doi: 10.1016/j.ajhg.2021.04.022. PMID: 34087165 MH - Cohort Studies MH - *DNA Methylation MH - Genetic Heterogeneity MH - Humans MH - Neurodevelopmental Disorders/*genetics MH - *Phenotype MH - Syndrome PMC - PMC7058829 OTO - NOTNLM OT - DNA methylation OT - EpiSign OT - VUS classification OT - episignature OT - molecular diagnostics OT - uncertain clinical cases COIS- The authors declare no competing interests. EDAT- 2020/02/29 06:00 MHDA- 2020/05/06 06:00 PMCR- 2020/09/05 CRDT- 2020/02/29 06:00 PHST- 2019/11/08 00:00 [received] PHST- 2020/01/27 00:00 [accepted] PHST- 2020/02/29 06:00 [pubmed] PHST- 2020/05/06 06:00 [medline] PHST- 2020/02/29 06:00 [entrez] PHST- 2020/09/05 00:00 [pmc-release] AID - S0002-9297(20)30019-7 [pii] AID - 10.1016/j.ajhg.2020.01.019 [doi] PST - ppublish SO - Am J Hum Genet. 2020 Mar 5;106(3):356-370. doi: 10.1016/j.ajhg.2020.01.019. Epub 2020 Feb 27. PMID- 33326457 OWN - NLM STAT- MEDLINE DCOM- 20201224 LR - 20231110 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 15 IP - 12 DP - 2020 TI - Glucocorticoids with low-dose anti-IL1 anakinra rescue in severe non-ICU COVID-19 infection: A cohort study. PG - e0243961 LID - 10.1371/journal.pone.0243961 [doi] LID - e0243961 AB - BACKGROUND: The optimal treatment for patients with severe coronavirus-19 disease (COVID-19) and hyper-inflammation remains debated. MATERIAL AND METHODS: A cohort study was designed to evaluate whether a therapeutic algorithm using steroids with or without interleukin-1 antagonist (anakinra) could prevent death/invasive ventilation. Patients with a ≥5-day evolution since symptoms onset, with hyper-inflammation (CRP≥50mg/L), requiring 3-5 L/min oxygen, received methylprednisolone alone. Patients needing ≥6 L/min received methylprednisolone + subcutaneous anakinra daily either frontline or in case clinical deterioration upon corticosteroids alone. Death rate and death or intensive care unit (ICU) invasive ventilation rate at Day 15, with Odds Ratio (OR) and 95% CIs, were determined according to logistic regression and propensity scores. A Bayesian analysis estimated the treatment effects. RESULTS: Of 108 consecutive patients, 70 patients received glucocorticoids alone. The control group comprised 63 patients receiving standard of care. In the corticosteroid±stanakinra group (n = 108), death rate was 20.4%, versus 30.2% in the controls, indicating a 30% relative decrease in death risk and a number of 10 patients to treat to avoid a death (p = 0.15). Using propensity scores a per-protocol analysis showed an OR for COVID-19-related death of 0.9 (95%CI [0.80-1.01], p = 0.067). On Bayesian analysis, the posterior probability of any mortality benefit with corticosteroids+/-anakinra was 87.5%, with a 7.8% probability of treatment-related harm. Pre-existing diabetes exacerbation occurred in 29 of 108 patients (26.9%). CONCLUSION: In COVID-19 non-ICU inpatients at the cytokine release phase, corticosteroids with or without anakinra were associated with a 30% decrease of death risk on Day 15. FAU - Borie, Raphael AU - Borie R AUID- ORCID: 0000-0002-9906-0024 AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Savale, Laurent AU - Savale L AD - Pulmonology Department, Kremlin-Bicêtre University Hospital, AP-HP, Paris-Saclay University, Kremlin-Bicêtre, France. FAU - Dossier, Antoine AU - Dossier A AD - Internal Medicine Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Ghosn, Jade AU - Ghosn J AD - Infectious Disease Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Taillé, Camille AU - Taillé C AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Visseaux, Benoit AU - Visseaux B AD - Virology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Jebreen, Kamel AU - Jebreen K AD - Biostatistics and Clinical Research Department, University Hospital Lariboisière, AP-HP, Université de Paris, Paris, France. FAU - Diallo, Abourahmane AU - Diallo A AD - Biostatistics and Clinical Research Department, University Hospital Lariboisière, AP-HP, Université de Paris, Paris, France. FAU - Tesmoingt, Chloe AU - Tesmoingt C AD - Pharmacy Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Morer, Lise AU - Morer L AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Goletto, Tiphaine AU - Goletto T AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Faucher, Nathalie AU - Faucher N AD - Geriatrics Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Hajouji, Linda AU - Hajouji L AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Neukirch, Catherine AU - Neukirch C AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Phillips, Mathilde AU - Phillips M AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Stelianides, Sandrine AU - Stelianides S AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Bouadma, Lila AU - Bouadma L AD - Medical and infectious Diseases ICU, Intensive Care Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Brosseau, Solenn AU - Brosseau S AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Ottaviani, Sébastien AU - Ottaviani S AD - Rheumatology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Pluvy, Johan AU - Pluvy J AUID- ORCID: 0000-0002-0129-5803 AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Le Pluart, Diane AU - Le Pluart D AD - Infectious Disease Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Debray, Marie-Pierre AU - Debray MP AD - Radiology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Raynaud-Simon, Agathe AU - Raynaud-Simon A AD - Pharmacy Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Descamps, Diane AU - Descamps D AD - Virology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Khalil, Antoine AU - Khalil A AD - Radiology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Timsit, Jean Francois AU - Timsit JF AD - Medical and infectious Diseases ICU, Intensive Care Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Lescure, Francois-Xavier AU - Lescure FX AD - Infectious Disease Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Descamps, Vincent AU - Descamps V AD - Dermatology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Papo, Thomas AU - Papo T AD - Internal Medicine Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Humbert, Marc AU - Humbert M AD - Pulmonology Department, Kremlin-Bicêtre University Hospital, AP-HP, Paris-Saclay University, Kremlin-Bicêtre, France. FAU - Crestani, Bruno AU - Crestani B AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Dieude, Philippe AU - Dieude P AD - Rheumatology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. FAU - Vicaut, Eric AU - Vicaut E AD - Biostatistics and Clinical Research Department, University Hospital Lariboisière, AP-HP, Université de Paris, Paris, France. FAU - Zalcman, Gérard AU - Zalcman G AD - Pulmonology and Thoracic Oncology Department, University Hospital Bichat-Claude Bernard, AP-HP, Université de Paris, Paris, France. CN - Bichat & Kremlin-Bicêtre AP-HP COVID teams LA - eng PT - Journal Article DEP - 20201216 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - 0 (Glucocorticoids) RN - 0 (Interleukin 1 Receptor Antagonist Protein) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Aged MH - Bayes Theorem MH - COVID-19/mortality/pathology/virology MH - Case-Control Studies MH - Cohort Studies MH - Comorbidity MH - Drug Therapy, Combination MH - Female MH - Glucocorticoids/*therapeutic use MH - Humans MH - Intensive Care Units MH - Interleukin 1 Receptor Antagonist Protein/*therapeutic use MH - Kaplan-Meier Estimate MH - Male MH - Methylprednisolone/*therapeutic use MH - Middle Aged MH - Odds Ratio MH - Risk Factors MH - SARS-CoV-2/isolation & purification MH - Severity of Illness Index MH - *COVID-19 Drug Treatment PMC - PMC7743937 COIS- Raphael Borie, Laurent Savalle, Antoine Dossier, Camille Taillé, Benoit Visseaux, Kamel Jebreen, Sébastien Ottaviani, Chloe Tesmoingt, Lise Morer, Tiphaine Goletto, Nathalie Faucher, Linda Hajouji, Catherine Neukirch, Mathilde Phillips, Sandrine Stelianides, Solenn Brosseau, Johan Pluvy, Marie Pierre Debray, Raynaud-Simon Agathe, Antoine Khalil, Vincent Descamps, Thomas Papo, Marc Humbert, Bruno Crestani, Eric Vicaut, Gérard Zalcman have nothing to disclose. Jean Francois Timsit reported participation to an advisory board from Gilead. Is the principal investigator of PHRC-N 'Covidicus' (Dexamethasone vs. Placebo on Covid-19 pneumonia in ICUs) granted by the French Ministry of Health. Jade Ghosn reported receiving Jade Ghosn reported receiving travel grants and fundings from Gilead Sciences, ViiV Healthcare and MSD. Benoit Visseaux reported grants from QIAGEN outside the scope of the current work Xavier Lescure reported travel grants and fundings from from Gilead, MSD, Astellas, Eumedica. This does not alter our adherence to PLOS ONE policies on sharing data and materials. EDAT- 2020/12/17 06:00 MHDA- 2020/12/29 06:00 PMCR- 2020/12/16 CRDT- 2020/12/16 17:14 PHST- 2020/08/18 00:00 [received] PHST- 2020/12/02 00:00 [accepted] PHST- 2020/12/16 17:14 [entrez] PHST- 2020/12/17 06:00 [pubmed] PHST- 2020/12/29 06:00 [medline] PHST- 2020/12/16 00:00 [pmc-release] AID - PONE-D-20-25884 [pii] AID - 10.1371/journal.pone.0243961 [doi] PST - epublish SO - PLoS One. 2020 Dec 16;15(12):e0243961. doi: 10.1371/journal.pone.0243961. eCollection 2020. PMID- 31451536 OWN - NLM STAT- MEDLINE DCOM- 20200615 LR - 20260518 IS - 1468-6244 (Electronic) IS - 0022-2593 (Print) IS - 0022-2593 (Linking) VI - 56 IP - 10 DP - 2019 Oct TI - Estimating the effect size of the 15Q11.2 BP1-BP2 deletion and its contribution to neurodevelopmental symptoms: recommendations for practice. PG - 701-710 LID - 10.1136/jmedgenet-2018-105879 [doi] AB - BACKGROUND: The 15q11.2 deletion is frequently identified in the neurodevelopmental clinic. Case-control studies have associated the 15q11.2 deletion with neurodevelopmental disorders, and clinical case series have attempted to delineate a microdeletion syndrome with considerable phenotypic variability. The literature on this deletion is extensive and confusing, which is a challenge for genetic counselling. The aim of this study was to estimate the effect size of the 15q11.2 deletion and quantify its contribution to neurodevelopmental disorders. METHODS: We performed meta-analyses on new and previously published case-control studies and used statistical models trained in unselected populations with cognitive assessments. We used new (n=241) and previously published (n=150) data from a clinically referred group of deletion carriers. 15q11.2 duplications (new n=179 and previously published n=35) were used as a neutral control variant. RESULTS: The deletion decreases IQ by 4.3 points. The estimated ORs and respective frequencies in deletion carriers for intellectual disabilities, schizophrenia and epilepsy are 1.7 (3.4%), 1.5 (2%) and 3.1 (2.1%), respectively. There is no increased risk for heart malformations and autism. In the clinically referred group, the frequency and nature of symptoms in deletions are not different from those observed in carriers of the 15q11.2 duplication suggesting that most of the reported symptoms are due to ascertainment bias. CONCLUSIONS: We recommend that the deletion should be classified as 'pathogenic of mild effect size'. Since it explains only a small proportion of the phenotypic variance in carriers, it is not worth discussing in the developmental clinic or in a prenatal setting. CI - © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Jønch, Aia Elise AU - Jønch AE AD - Department of Clinical Genetics, Odense University Hospital, Odense, Denmark. AD - Human Genetics, Department of Clinical Research, University of Southern Denmark, Odense, Denmark. FAU - Douard, Elise AU - Douard E AD - Department of Pediatrics, University of Montreal, Montreal, Québec, Canada. AD - Center Hospitalier Universitaire Sainte-Justine Research Center, Montreal, Québec, Canada. FAU - Moreau, Clara AU - Moreau C AD - Department of Pediatrics, University of Montreal, Montreal, Québec, Canada. AD - Center Hospitalier Universitaire Sainte-Justine Research Center, Montreal, Québec, Canada. FAU - Van Dijck, Anke AU - Van Dijck A AD - Department of Medical Genetics, University of Antwerp, Antwerp, Belgium. AD - Department of Neurology, University Hospital Antwerp, Antwerp, Belgium. FAU - Passeggeri, Marzia AU - Passeggeri M AD - Service of Medical Genetics, CHUV Lausanne, Lausanne, Switzerland. FAU - Kooy, Frank AU - Kooy F AUID- ORCID: 0000-0003-2024-0485 AD - Department of Medical Genetics, University of Antwerp, Antwerp, Belgium. AD - Department of Neurology, University Hospital Antwerp, Antwerp, Belgium. FAU - Puechberty, Jacques AU - Puechberty J AD - Département de Génétique Médicale, Maladies rares et Médecine personnalisée, Université Montpelier, Hôpital Arnaud de Villeneuve, CHU de Montpellier, Montpellier, France. FAU - Campbell, Carolyn AU - Campbell C AD - Oxford Medical Genetics Laboratories, Oxford University Hospitals NHS Foundation Trust, Oxford, UK. FAU - Sanlaville, Damien AU - Sanlaville D AD - Service de Génétique, Hospices Civils de Lyon, CHU de Lyon, Bron, France. AD - Centre de Recherche en Neurosciences de Lyon, GENDEV Team, INSERM U1028, CNRS UMR5292, Université Claude Bernard Lyon, Bron, France. FAU - Lefroy, Henrietta AU - Lefroy H AD - Oxford Centre for Genomic Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, UK. FAU - Richetin, Sonia AU - Richetin S AD - Service of Medical Genetics, CHUV Lausanne, Lausanne, Switzerland. FAU - Pain, Aurelie AU - Pain A AD - Service of Medical Genetics, CHUV Lausanne, Lausanne, Switzerland. AD - Centre Cantonal Autisme, CHUV Lausanne, Lausanne, Switzerland. FAU - Geneviève, David AU - Geneviève D AD - Département de Génétique Médicale, Hôpital Arnaud de Villeneuve, CHU de Montpellier, Montpellier, France. AD - INSERM, U1183, IRMB, Hôpital Saint Eloi, CHU de Montpellier, Montpellier, France. FAU - Kini, Usha AU - Kini U AD - Oxford Centre for Genomic Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, UK. AD - The Spires Cleft Centre, John Radcliffe Hospital, Oxford, UK. FAU - Le Caignec, Cédric AU - Le Caignec C AD - Service de Génétique Médical, CHU Nantes, Nantes, France. FAU - Lespinasse, James AU - Lespinasse J AD - Service de Cytogenetique, Centre Hospitalier de Chambéry, Chambéry, France. FAU - Skytte, Anne-Bine AU - Skytte AB AD - Department of Clinical Genetics, Aarhus University Hospital, Aarhus, Denmark. AD - Department of Clinical epidemiology, Aarhus University, Aarhus, Denmark. FAU - Isidor, Bertrand AU - Isidor B AD - Service de Génétique Médical, CHU Nantes, Nantes, France. FAU - Zweier, Christiane AU - Zweier C AD - Institute of Human Genetics, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany. FAU - Caberg, Jean-Hubert AU - Caberg JH AD - Department of Human Genetics, CHU de Liège, Liège, Belgium. FAU - Delrue, Marie-Ange AU - Delrue MA AD - Department of Pediatrics, University of Montreal, Montreal, Québec, Canada. AD - Center Hospitalier Universitaire Sainte-Justine Research Center, Montreal, Québec, Canada. FAU - Møller, Rikke Steensbjerre AU - Møller RS AD - Danish Epilepsy Center, Filadelfia, Dianalund, Denmark. FAU - Bojesen, Anders AU - Bojesen A AD - Department of Clinical Genetics, Sygehus Lillebalt Vejle Sygehus, Vejle, Denmark. FAU - Hjalgrim, Helle AU - Hjalgrim H AD - Danish Epilepsy Center, Filadelfia, Dianalund, Denmark. FAU - Brasch-Andersen, Charlotte AU - Brasch-Andersen C AD - Department of Clinical Genetics, Odense University Hospital, Odense, Denmark. AD - Human Genetics, Department of Clinical Research, University of Southern Denmark, Odense, Denmark. FAU - Lemyre, Emmanuelle AU - Lemyre E AD - Department of Pediatrics, University of Montreal, Montreal, Québec, Canada. AD - Center Hospitalier Universitaire Sainte-Justine Research Center, Montreal, Québec, Canada. FAU - Ousager, Lilian Bomme AU - Ousager LB AD - Department of Clinical Genetics, Odense University Hospital, Odense, Denmark. AD - Human Genetics, Department of Clinical Research, University of Southern Denmark, Odense, Denmark. FAU - Jacquemont, Sébastien AU - Jacquemont S AUID- ORCID: 0000-0001-6838-8767 AD - Department of Pediatrics, University of Montreal, Montreal, Québec, Canada sebastien.jacquemont@umontreal.ca. AD - Center Hospitalier Universitaire Sainte-Justine Research Center, Montreal, Québec, Canada. CN - 15q11.2 Working Group LA - eng GR - WT_/Wellcome Trust/United Kingdom PT - Journal Article PT - Meta-Analysis PT - Research Support, Non-U.S. Gov't DEP - 20190826 PL - England TA - J Med Genet JT - Journal of medical genetics JID - 2985087R SB - IM MH - Autistic Disorder/*genetics MH - Case-Control Studies MH - Cohort Studies MH - *DNA Copy Number Variations MH - Epilepsy/*genetics MH - Female MH - Heart Diseases/congenital/*genetics MH - Humans MH - Intellectual Disability/*genetics MH - Loss of Function Mutation MH - Male MH - Neurodevelopmental Disorders/*genetics MH - Sequence Deletion PMC - PMC6817694 OTO - NOTNLM OT - 15q11.2 copy-number variants OT - congenital heart disease OT - epilepsy OT - loss-of-function intolerance OT - neurodevelopmental disorders COIS- Competing interests: None declared. FIR - Andrieux, Joris IR - Andrieux J FIR - Barnicoat, Angela IR - Barnicoat A FIR - Blanchet, Patricia IR - Blanchet P FIR - Blesson, Sophie IR - Blesson S FIR - Bütschi, Florence Niel IR - Bütschi FN FIR - Campeau, Philippe M IR - Campeau PM FIR - Chelloug, Nora IR - Chelloug N FIR - Debray, François-Guillaume IR - Debray FG FIR - Fellmann, Florence IR - Fellmann F FIR - Ferrarini, Alessandra IR - Ferrarini A FIR - Gibbons, Richard IR - Gibbons R FIR - Gregersen, Pernille Axel IR - Gregersen PA FIR - Hoyer, Juliane IR - Hoyer J FIR - Hüffmeier, Ulrike IR - Hüffmeier U FIR - Kjelgaard, Ditte IR - Kjelgaard D FIR - Krumbiegel, Mandy IR - Krumbiegel M FIR - Lebon, Sébastien IR - Lebon S FIR - Lesca, Gaetan IR - Lesca G FIR - Marignier, Stéphanie IR - Marignier S FIR - Mercier, Sandra IR - Mercier S FIR - Michaud, Jacques IR - Michaud J FIR - Mitchell, Grant IR - Mitchell G FIR - Mortemousque, Isabelle IR - Mortemousque I FIR - Møller, Rikke S IR - Møller RS FIR - Nizon, Mathilde IR - Nizon M FIR - Pierquin, Genevieve IR - Pierquin G FIR - Sørensen, Kristina Pilekær IR - Sørensen KP FIR - Price, Sue IR - Price S FIR - Pujol, Pascal H IR - Pujol PH FIR - Ramaekers, Vincent IR - Ramaekers V FIR - Raynaud, Martine IR - Raynaud M FIR - Reis, André IR - Reis A FIR - Rossi, Massimiliano IR - Rossi M FIR - Sarda, Pierre IR - Sarda P FIR - Stanzial, Franco IR - Stanzial F FIR - Stewart, Helen IR - Stewart H FIR - Svaneby, Dea IR - Svaneby D FIR - Theil, Christian T IR - Theil CT FIR - Till, Marianne IR - Till M FIR - Trakadis, Yannis IR - Trakadis Y FIR - Ville, Dorothée IR - Ville D FIR - Vonwill, Sandrine IR - Vonwill S FIR - Wilkie, Andrew IR - Wilkie A FIR - Wiessner, Antje IR - Wiessner A EDAT- 2019/08/28 06:00 MHDA- 2020/06/17 06:00 PMCR- 2019/10/29 CRDT- 2019/08/28 06:00 PHST- 2018/11/14 00:00 [received] PHST- 2019/04/12 00:00 [revised] PHST- 2019/05/27 00:00 [accepted] PHST- 2019/08/28 06:00 [pubmed] PHST- 2020/06/17 06:00 [medline] PHST- 2019/08/28 06:00 [entrez] PHST- 2019/10/29 00:00 [pmc-release] AID - jmedgenet-2018-105879 [pii] AID - 10.1136/jmedgenet-2018-105879 [doi] PST - ppublish SO - J Med Genet. 2019 Oct;56(10):701-710. doi: 10.1136/jmedgenet-2018-105879. Epub 2019 Aug 26. PMID- 39209681 OWN - NLM STAT- MEDLINE DCOM- 20250918 LR - 20250918 IS - 2588-9311 (Electronic) IS - 2588-9311 (Linking) VI - 8 IP - 4 DP - 2025 Aug TI - Final Results of the ANDROCAN Study: Histopathological Characteristics and Biochemical Recurrence at 5 Years of Localized Prostate Cancer According to Preoperative Gonadal Status. PG - 1111-1117 LID - S2588-9311(24)00191-3 [pii] LID - 10.1016/j.euo.2024.08.003 [doi] AB - BACKGROUND AND OBJECTIVE: Failure rates after first-line treatment of localized prostate cancer (PCa) treatment remain high; therefore, it is essential to improve the selection and identification of at-risk patients to reduce mortality. The aim of the ANDROCAN study was to evaluate the biochemical recurrence (BCR) in patients with localized PCa treated by total prostatectomy at 5 yr after surgery, according to their presurgery gonadal status. METHODS: A prospective cohort study was conducted including 1318 patients undergoing total prostatectomy for localized PCa with a 5-yr postoperative follow-up. Clinical and hormonal data (assays of total testosterone [TT], bioavailable testosterone [BT], dihydrotestosterone, estrone, and estradiol were performed by gas chromatography/mass spectrometry) as well as metabolic syndrome parameters were collected at baseline before surgery. Pathological data (predominant Gleason grade 4 and stage) were collected and cross-referenced centrally. Factors associated with BCR were assessed by a multivariate analysis, and BCR-free survival was assessed by a Kaplan-Meier analysis. KEY FINDINGS AND LIMITATIONS: Among the 1318 patients, 237 had BCR of PCa. Considering demographic characteristics, populations with and without BCR were similar. However, patients with BCR had cancers with a higher Gleason score (p = 0.0001) and higher prostate-specific antigen (PSA) values (p = 0.0005) at baseline. Gleason score, pT >3a, and PSA level at baseline were positively correlated with BCR (p < 0.0001, p < 0.0001, and p = 0.0048, respectively), while BT and TT levels were not associated with BCR. This study includes patients with varying clinical characteristics, such as cancer history and metabolic syndrome, introducing variability that makes it challenging to isolate the specific effects of gonadal status on BCR. Another limitation is the lack of evaluation of long-term BCR beyond 5 yr, potentially overlooking recurrences that occur between 5 and 15 yr after surgery. This could lead to an underestimation of the actual long-term recurrence rates. CONCLUSIONS AND CLINICAL IMPLICATIONS: Overall, PSA levels, high Gleason score, and pT >3a are associated with a greater likelihood of disease recurrence following initial treatment and could serve as important prognostic indicators for predicting the risk of BCR. In this prospective study, biochemical hypogonadism was not associated with a higher occurrence of BCR within 5 yr of prostatectomy. The biological gonadal status of preoperative patients could potentially be useful for therapeutic decisions but does not provide an indication for the oncological follow-up. PATIENT SUMMARY: Five-year follow up of patients after surgery showed that there is no association between hypogonadism (low levels of total testosterone and bioavailable testosterone) and cancer recurrence. However, cancer recurrence seems to be more associated with aggressiveness of cancer at the time of detection. CI - Copyright © 2024 European Association of Urology. Published by Elsevier B.V. All rights reserved. FAU - Neuzillet, Yann AU - Neuzillet Y AD - Department of Urology, University of Versailles-Saint-Quentin-en-Yvelines, Foch Hospital, Suresnes, France. Electronic address: y.neuzillet@hopital-foch.org. FAU - Raynaud, Jean-Pierre AU - Raynaud JP AD - Sorbonne University, Paris, France. FAU - Dreyfus, Jean-François AU - Dreyfus JF AD - Department of Clinical Research and Innovation, University of Versailles-Saint-Quentin-en-Yvelines, Foch Hospital, Suresnes, France. FAU - Radulescu, Camélia AU - Radulescu C AD - Department of Pathology, Foch Hospital, Suresnes, France. FAU - Rouanne, Mathieu AU - Rouanne M AD - Department of Urology, University of Versailles-Saint-Quentin-en-Yvelines, Foch Hospital, Suresnes, France. FAU - Schneider, Marc AU - Schneider M AD - Department of Urology, Louis Pasteur Hospital, Colmar, France. FAU - Krish, Sylvie AU - Krish S AD - Department of Pathology, Louis Pasteur Hospital, Colmar, France. FAU - Rouprêt, Morgan AU - Rouprêt M AD - Department of Urology, Pitié Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Drouin, Sarah J AU - Drouin SJ AD - Department of Urology, Pitié Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Comperat, Eva AU - Comperat E AD - Department of Pathology, Tenon Hospital, Assistance Publique - Hôpitaux de Paris, Sorbonne University, Paris, France. FAU - Galiano, Marc AU - Galiano M AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Cathelineau, Xavier AU - Cathelineau X AD - Department of Urology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Validire, Pierre AU - Validire P AD - Department of Pathology, Institut Mutualiste Montsouris, Paris-Descartes University, Paris, France. FAU - Molinié, Vincent AU - Molinié V AD - Department of Pathology, Centre Hospitalier de Martinique, Le Lamentin, France. FAU - Fiet, Jean AU - Fiet J AD - Inserm U955, Eq07, Recherches Translationnelles en oncogenèse génitale, Créteil, France. FAU - Giton, Franck AU - Giton F AD - Inserm U955, Eq07, Recherches Translationnelles en oncogenèse génitale, Créteil, France. FAU - Lebret, Thierry AU - Lebret T AD - Department of Urology, University of Versailles-Saint-Quentin-en-Yvelines, Foch Hospital, Suresnes, France. FAU - Botto, Henry AU - Botto H AD - Department of Urology, University of Versailles-Saint-Quentin-en-Yvelines, Foch Hospital, Suresnes, France. LA - eng PT - Journal Article DEP - 20240828 PL - Netherlands TA - Eur Urol Oncol JT - European urology oncology JID - 101724904 RN - EC 3.4.21.77 (Prostate-Specific Antigen) RN - 3XMK78S47O (Testosterone) SB - IM MH - Humans MH - Male MH - *Prostatic Neoplasms/surgery/pathology/blood MH - Middle Aged MH - Aged MH - *Neoplasm Recurrence, Local/blood/pathology MH - Prostatectomy MH - Prospective Studies MH - Prostate-Specific Antigen/blood MH - Neoplasm Grading MH - Testosterone/blood MH - Follow-Up Studies MH - Preoperative Period OTO - NOTNLM OT - Biochemical recurrence OT - Eugonadic OT - Hypogonadism OT - Prostate cancer OT - Prostatectomy OT - Testosterone EDAT- 2024/08/31 09:51 MHDA- 2025/09/19 00:36 CRDT- 2024/08/29 21:58 PHST- 2024/05/13 00:00 [received] PHST- 2024/07/24 00:00 [revised] PHST- 2024/08/06 00:00 [accepted] PHST- 2025/09/19 00:36 [medline] PHST- 2024/08/31 09:51 [pubmed] PHST- 2024/08/29 21:58 [entrez] AID - S2588-9311(24)00191-3 [pii] AID - 10.1016/j.euo.2024.08.003 [doi] PST - ppublish SO - Eur Urol Oncol. 2025 Aug;8(4):1111-1117. doi: 10.1016/j.euo.2024.08.003. Epub 2024 Aug 28. PMID- 42141162 OWN - NLM STAT- Publisher LR - 20260515 IS - 1573-0646 (Electronic) IS - 0167-6997 (Linking) DP - 2026 May 16 TI - A Phase I trial of the anti-IL23 monoclonal antibody tildrakizumab, in combination with abiraterone acetate, for the treatment of metastatic castration-resistant prostate cancer. LID - 10.1007/s10637-026-01619-x [doi] AB - BACKGROUND: Interleukin-23 (IL23) has been reported to drive androgen receptor (AR) and JAK2-STAT3 signaling, promoting treatment resistance and disease progression in advanced prostate cancer (PC). We evaluated the safety, tolerability, and antitumor activity of the anti-IL23 monoclonal antibody tildrakizumab in combination with the AR pathway inhibitor (ARPI) abiraterone acetate (AA) in men with ARPI-resistant metastatic castration-resistant PC (mCRPC). METHODS: mCRPC patients of ECOG performance status (PS) ≤ 2, who had previously progressed on first-line ARPI therapy, were treated with tildrakizumab (100 mg, 300 mg, 600 mg; 4-weekly) in combination with AA (YonsaTM, 500 mg daily). The primary objective was to determine the recommended phase 2 dose. Secondary endpoints were elucidation of pharmacokinetics (PK), pharmacodynamics (PD), and antitumor activity. RESULTS: No dose limiting toxicities (DLTs) were observed, nor any grade ≥ 3 adverse effects (AEs). The most common treatment-related AEs attributable to tildrakizumab were grade 1-2 fatigue (n = 3/12; 25.0%) and grade 1 nausea (n = 2/12; 16.7%). PK analyses showed no obvious drug-drug interactions. Overall, no objective responses were observed. Median progression free survival (PFS) was 3.7 months (n = 10; 95% CI 1.6-not reached) with median overall survival (OS) of 9.7 months (n = 10; 95% CI 6.9-not reached). CONCLUSIONS: Tildrakizumab and abiraterone acetate combination therapy is well tolerated but did not show clinical efficacy in this small, unselected, cohort of ARPI-resistant mCRPC patients. Further study into the causes of primary resistance to IL23 targeting in mCRPC, and development of biomarkers of downstream IL23-IL23R activity, are now needed to translate IL23 blockade into the clinic. CI - © 2026. The Author(s). FAU - Chandran, Khobe AU - Chandran K AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Guo, Christina AU - Guo C AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Pacey, Simon AU - Pacey S AD - Department of Oncology, School of Clinical Medicine, University of Cambridge, Cambridge, UK. AD - Cambridge University Hospitals NHS Foundation Trust, Addenbrookes Hospital, Cambridge, UK. FAU - Villacampa, Guillermo AU - Villacampa G AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Matthews, Ruth AU - Matthews R AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Prout, Toby AU - Prout T AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Fernandes, Joan AU - Fernandes J AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Turner, Alison AU - Turner A AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Parmar, Mona AU - Parmar M AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Riisnaes, Ruth AU - Riisnaes R AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Ferreira, Ana AU - Ferreira A AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Carreira, Suzanne AU - Carreira S AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Bertan, Claudia AU - Bertan C AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Crespo, Mateus AU - Crespo M AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Figueiredo, Ines AU - Figueiredo I AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Yuan, Wei AU - Yuan W AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Bogdan, Denisa AU - Bogdan D AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Raynaud, Florence AU - Raynaud F AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Ruddle, Ruth AU - Ruddle R AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Calì, Bianca AU - Calì B AD - Institute of Oncology Research (IOR), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Lugano, Switzerland. FAU - Maddalena, Martino AU - Maddalena M AD - Institute of Oncology Research (IOR), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Lugano, Switzerland. FAU - Calcinotto, Arianna AU - Calcinotto A AD - Institute of Oncology Research (IOR), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Lugano, Switzerland. FAU - Yap, Christina AU - Yap C AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. FAU - Sharp, Adam AU - Sharp A AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Alimonti, Andrea AU - Alimonti A AD - Oncology Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland. AD - Institute of Oncology Research (IOR), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Lugano, Switzerland. AD - Department of Health Sciences and Technology, Eidgenössische Technische Hochschule Zürich (ETH), Zurich, Switzerland. AD - Department of Medicine, University of Padova, Padua, Italy. FAU - De Bono, Johann AU - De Bono J AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. Johann.DeBono@icr.ac.uk. AD - The Royal Marsden NHS Foundation Trust, London, UK. Johann.DeBono@icr.ac.uk. FAU - Paschalis, Alec AU - Paschalis A AD - Institute of Cancer Research, 15Cotswold RoadSM2 5NG, London, UK. Alec.paschalis@icr.ac.uk. AD - The Royal Marsden NHS Foundation Trust, London, UK. Alec.paschalis@icr.ac.uk. LA - eng GR - NIHR203312/NIHR Cambridge Biomedical Research Centre/ GR - 21A009/Novartis Foundation/ GR - 19CHAL08/Prostate Cancer Foundation/ GR - 20YOUN17/John Black Charitable Foundation/ PT - Journal Article DEP - 20260516 PL - United States TA - Invest New Drugs JT - Investigational new drugs JID - 8309330 SB - IM OTO - NOTNLM OT - Androgen Receptor Signaling OT - Interleukin-23 OT - Metastatic Castration-Resistant Prostate Cancer OT - Phase I trial OT - Prostate Cancer OT - Treatment Resistance COIS- Declarations. Conflicts Of interest: KC, CG, RR, AF, SC, CB, MC, IF, AS, AP and JDB are all employees of The Institute of Cancer Research (ICR), which has a commercial interest in abiraterone, PARP inhibition in DNA repair–defective cancers, and PI3K/AKT pathway inhibitors (no personal income). The ICR operates a Rewards to Inventors scheme through which employees of the ICR may receive financial benefit following commercial licensing. AP has been the CI/PI of industry-sponsored clinical trials and has received honoraria from Boehringer Ingelheim. AS has received travel support from Sanofi, Roche/Genentech, and Nurix, and speaker honoraria from Astellas Pharma and Merck Sharp & Dohme. He has served as an advisor to DE Shaw Research, CHARM Therapeutics, Ellipses Pharma, and Droia Ventures. JDB has served on advisory boards and received fees from many companies including Abbvie, Acai Therapeutics, Amgen, Amunix, Astellas Nordic, Bayer, Bioxcel Therapeutics, Celcuity, Crescendo, Daiichi, Dark Blue Therapeutics, Duke Street Bio Limited, Dunad Therapeutics, Endeavor Biomedicines INC, Genentech/Roche, GSK, Macrogenics, Merck Serono, MetaCurUm, Moma, Myricx, Novartis, Nurix Therapeutics, Nuvation Bio, One-Carbon Therapeutics Inc, Oncternal, Orion, Page Therapeutics, Pfizer, Takeda, Tango Therapeutics, Tubulis GmbH. He is an employee of The ICR, which have received funding or other support for his research work from AstraZeneca, Cellcentric, Crescendo, Daiichi, Immunic Therapeutics, MetaCurUm, Myricx, Nurix Therapeutics, Oncternal, Orion, Sanofi Aventis. JDB was named as an inventor, with no financial interest for patent 8,822,438, submitted by Janssen that covers the use of abiraterone acetate with corticosteroids. He has been the CI/PI of many industry sponsored clinical trials. SP received research funding from AstraZeneca, Ferring, Jannsen, Guardant Health and has consultancy in Astra Zeneca, Roche, Elsevier and Bayer. CY received consultancy fees from FARON Pharmaceuticals unrelated to this work. AA is a co-founder of and owns stock in OncoSense and MicThera. He has served as an advisor to IBSA Institut Biochimique, Relmada Therapeutics, MicThera, BIOREK, Debiopharm International, ONO Pharma UK. He is an employee of The Institute of Oncology Research (IOR) in Bellinzona (Switzerland), which have received funding for his research work from AlpENa Biosciences, Astellas Pharma, AstraZeneca, IBSA Institut Biochimique, Macomics, Novartis, Peptone, Sun Pharma Global, MGGM, NovaRock Biotherapeutics. He was named as an inventor, with no financial interest for patent US20200095314A1 and US11168134B2, that cover the use of an IL23 inhibitor for treating of prostate cancer. BC, MM, and AC are all employees of IOR. EDAT- 2026/05/16 00:36 MHDA- 2026/05/16 00:36 CRDT- 2026/05/15 23:21 PHST- 2026/02/04 00:00 [received] PHST- 2026/05/03 00:00 [accepted] PHST- 2026/05/16 00:36 [medline] PHST- 2026/05/16 00:36 [pubmed] PHST- 2026/05/15 23:21 [entrez] AID - 10.1007/s10637-026-01619-x [pii] AID - 10.1007/s10637-026-01619-x [doi] PST - aheadofprint SO - Invest New Drugs. 2026 May 16. doi: 10.1007/s10637-026-01619-x. PMID- 40261978 OWN - NLM STAT- MEDLINE DCOM- 20250423 LR - 20250812 IS - 1534-6080 (Electronic) IS - 0041-1337 (Linking) VI - 109 IP - 5 DP - 2025 May 1 TI - Archetypal Analysis of Kidney Allograft Biopsies Using Next-generation Sequencing Technology. PG - 871-880 LID - 10.1097/TP.0000000000005181 [doi] AB - BACKGROUND: In kidney transplantation, molecular diagnostics may be a valuable approach to improve the precision of the diagnosis. Using next-generation sequencing (NGS), we aimed to identify clinically relevant archetypes. METHODS: We conducted an Illumina bulk RNA sequencing on 770 kidney biopsies (540 kidney recipients) collected between 2006 and 2021 from 11 European centers. Differentially expressed genes were determined for 11 Banff lesions. An ElasticNet model was used for feature selection, and 4 machine learning classifiers were trained to predict the probability of presence of the lesions. NGS-based classifiers were used in an unsupervised archetypal analysis to different archetypes. The association of the archetypes with allograft survival was assessed using the iBox risk prediction score. RESULTS: The ElasticNet feature selection reduced the number of the genes from a range of 859-10 830 to a range of 52-867 genes. NGS-based classifiers demonstrated robust performances (precision-recall area under the curves 0.708-0.980) in predicting the Banff lesions. Archetypal analysis revealed 8 distinct phenotypes, each characterized by distinct clinical, immunological, and histological features. Although the archetypes confirmed the well-defined Banff rejection phenotypes for T cell-mediated rejection and antibody-mediated rejection, equivocal histologic antibody-mediated rejection, and borderline diagnoses were reclassified into different archetypes based on their molecular signatures. The 8 NGS-based archetypes displayed distinct allograft survival profiles with incremental graft loss rates between archetypes, ranging from 90% to 56% rates 7 y after evaluation ( P < 0.0001). CONCLUSIONS: Using molecular phenotyping, 8 archetypes were identified. These NGS-based archetypes might improve disease characterization, reclassify ambiguous Banff diagnoses, and enable patient-specific risk stratification. CI - Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved. FAU - Cortes Garcia, Esteban AU - Cortes Garcia E AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Giarraputo, Alessia AU - Giarraputo A AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Racapé, Maud AU - Racapé M AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Goutaudier, Valentin AU - Goutaudier V AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Ursule-Dufait, Cindy AU - Ursule-Dufait C AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - de la Grange, Pierre AU - de la Grange P AD - GenoSplice, Paris, France. FAU - Letourneur, Franck AU - Letourneur F AD - Université Paris Cité, CNRS, INSERM, Institut Cochin, Paris, France. FAU - Raynaud, Marc AU - Raynaud M AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Couderau, Clément AU - Couderau C AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Mezine, Fariza AU - Mezine F AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Dagobert, Jessie AU - Dagobert J AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. FAU - Bestard, Oriol AU - Bestard O AD - Department of Nephrology and Kidney Transplantation, Vall d'Hebron Hospital Universitari, Vall d'Hebron Institut de Recerca, Vall d'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Moreso, Francesc AU - Moreso F AD - Department of Nephrology and Kidney Transplantation, Vall d'Hebron Hospital Universitari, Vall d'Hebron Institut de Recerca, Vall d'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Villard, Jean AU - Villard J AD - Transplantation Immunology Unit and National Reference Laboratory for Histocompatibility, Department of Diagnostic, Geneva University Hospitals, Geneva, Switzerland. FAU - Halleck, Fabian AU - Halleck F AD - Department of Nephrology and Intensive Care, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. FAU - Giral, Magali AU - Giral M AD - Nantes Université, INSERM, CRT2I-Center for Research in Transplantation and Translational Immunology, Nantes, France. FAU - Brouard, Sophie AU - Brouard S AD - Nantes Université, INSERM, CRT2I-Center for Research in Transplantation and Translational Immunology, Nantes, France. FAU - Danger, Richard AU - Danger R AD - Nantes Université, INSERM, CRT2I-Center for Research in Transplantation and Translational Immunology, Nantes, France. FAU - Gourraud, Pierre-Antoine AU - Gourraud PA AD - Nantes Université, CHU de Nantes, Pôle Hospitalo-Universitaire 11: Santé Publique, Clinique des données, INSERM, Nantes, France. FAU - Rabant, Marion AU - Rabant M AD - Department of Pathology, Necker-Enfants Malades Hospital, APHP, Paris, France. AD - Université Paris Cité, Paris, France. FAU - Couzi, Lionel AU - Couzi L AD - Department of Nephrology, Transplantation, Dialysis and Apheresis, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France. FAU - Le Quintrec, Moglie AU - Le Quintrec M AD - Department of Nephrology Dialysis and Kidney Transplantation, Centre Hospitalier Universitaire de Montpellier, Montpellier, France. FAU - Kamar, Nassim AU - Kamar N AD - Department of Nephrology and Organ Transplantation, Toulouse Rangueil University Hospital, INSERM UMR 1291, Toulouse Institute for Infectious and Inflammatory Diseases (Infinity), University Paul Sabatier, Toulouse, France. FAU - Morelon, Emmanuel AU - Morelon E AD - Department of Transplantation, Nephrology and Clinical Immunology, Hospices Civils de Lyon, Lyon, France. FAU - Vrtovsnik, François AU - Vrtovsnik F AD - Department of Kidney Transplantation, Bichat Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.. FAU - Taupin, Jean-Luc AU - Taupin JL AD - Laboratory of Immunology and Histocompatibility, Hôpital Saint-Louis APHP, Paris, France.. FAU - Snanoudj, Renaud AU - Snanoudj R AD - Department of Nephrology and Transplantation, Kremlin-Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Kremlin-Bicêtre, France. FAU - Legendre, Christophe AU - Legendre C AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. AD - Department of Kidney Transplantation, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Anglicheau, Dany AU - Anglicheau D AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. AD - Department of Kidney Transplantation, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Budde, Klemens AU - Budde K AD - Department of Nephrology and Intensive Care, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. FAU - Lefaucheur, Carmen AU - Lefaucheur C AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. AD - Kidney Transplant Department, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Loupy, Alexandre AU - Loupy A AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. AD - Department of Kidney Transplantation, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. FAU - Aubert, Olivier AU - Aubert O AD - Université Paris Cité, INSERM U970, Paris Institute for Transplantation and Organ Regeneration, Paris, France. AD - Department of Kidney Transplantation, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. LA - eng GR - KTD_innov, EU-TRAIN/ PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20241023 PL - United States TA - Transplantation JT - Transplantation JID - 0132144 SB - IM MH - Humans MH - *Kidney Transplantation/adverse effects MH - *Graft Rejection/genetics/immunology/pathology/diagnosis MH - Biopsy MH - *High-Throughput Nucleotide Sequencing MH - Male MH - Female MH - Middle Aged MH - Graft Survival/genetics MH - Allografts/pathology MH - Adult MH - *Kidney/pathology/immunology MH - Predictive Value of Tests MH - Gene Expression Profiling MH - Machine Learning MH - Risk Assessment MH - Phenotype MH - Aged MH - Risk Factors MH - Treatment Outcome MH - Transcriptome COIS- The authors declare no conflicts of interest. EDAT- 2025/04/22 16:24 MHDA- 2025/04/23 06:26 CRDT- 2025/04/22 14:23 PHST- 2025/04/23 06:26 [medline] PHST- 2025/04/22 16:24 [pubmed] PHST- 2025/04/22 14:23 [entrez] AID - 00007890-202505000-00021 [pii] AID - 10.1097/TP.0000000000005181 [doi] PST - ppublish SO - Transplantation. 2025 May 1;109(5):871-880. doi: 10.1097/TP.0000000000005181. Epub 2024 Oct 23. PMID- 25587040 OWN - NLM STAT- MEDLINE DCOM- 20150720 LR - 20260518 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 125 IP - 16 DP - 2015 Apr 16 TI - Role of allogeneic stem cell transplantation in adult patients with Ph-negative acute lymphoblastic leukemia. PG - 2486-96; quiz 2586 LID - 10.1182/blood-2014-09-599894 [doi] AB - Because a pediatric-inspired Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL) protocol yielded a markedly improved outcome in adults with Philadelphia chromosome-negative ALL, we aimed to reassess the role of allogeneic stem cell transplantation (SCT) in patients treated in the GRAALL-2003 and GRAALL-2005 trials. In all, 522 patients age 15 to 55 years old and presenting with at least 1 conventional high-risk factor were candidates for SCT in first complete remission. Among these, 282 (54%) received a transplant in first complete remission. At 3 years, posttransplant cumulative incidences of relapse, nonrelapse mortality, and relapse-free survival (RFS) were estimated at 19.5%, 15.5%, and 64.7%, respectively. Time-dependent analysis did not reveal a significant difference in RFS between SCT and no-SCT cohorts. However, SCT was associated with longer RFS in patients with postinduction minimal residual disease (MRD) ≥10(-3) (hazard ratio, 0.40) but not in good MRD responders. In B-cell precursor ALL, SCT also benefitted patients with focal IKZF1 gene deletion (hazard ratio, 0.42). This article shows that poor early MRD response, in contrast to conventional ALL risk factors, is an excellent tool to identify patients who may benefit from allogeneic SCT in the context of intensified adult ALL therapy. Trial GRAALL-2003 was registered at www.clinicaltrials.gov as #NCT00222027; GRAALL-2005 was registered as #NCT00327678. CI - © 2015 by The American Society of Hematology. FAU - Dhédin, Nathalie AU - Dhédin N AD - Department of Hematology and University Paris Diderot, Institut Universitaire d'Hématologie, EA-3518, University Hospital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France; FAU - Huynh, Anne AU - Huynh A AD - Department of Hematology, University Institute of Cancer, Toulouse, France; FAU - Maury, Sébastien AU - Maury S AD - Department of Hematology, University Hospital Henri Mondor, AP-HP, Créteil, France; FAU - Tabrizi, Reza AU - Tabrizi R AD - Department of Hematology, University Hospital, Bordeaux, France; FAU - Beldjord, Kheira AU - Beldjord K AD - Department of Hematology and University Paris Diderot, Institut Universitaire d'Hématologie, EA-3518, University Hospital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France; FAU - Asnafi, Vahid AU - Asnafi V AD - Department of Hematology, University Paris Descartes Sorbonne Cité, Institut Necker-Enfants Malades, INSERM U1151, and Onco-Hematology Laboratory, Assistance Publique Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Paris, France; FAU - Thomas, Xavier AU - Thomas X AD - Department of Hematology, Hospital Lyon Sud, Pierre Bénite, France; FAU - Chevallier, Patrice AU - Chevallier P AD - Department of Hematology, University Hospital, Nantes, France; FAU - Nguyen, Stéphanie AU - Nguyen S AD - Department of Hematology, University Hospital Pitié-Salpêtrière, Assistance Publique Hôpitaux de Paris, Paris, France; FAU - Coiteux, Valérie AU - Coiteux V AD - Department of Hematology, University Hospital, Lille, France; FAU - Bourhis, Jean-Henri AU - Bourhis JH AD - Department of Hematology, Institut Gustave Roussy, Villejuif, France; FAU - Hichri, Yosr AU - Hichri Y AD - Department of Hematology, University Hospital, Montpellier, France; FAU - Escoffre-Barbe, Martine AU - Escoffre-Barbe M AD - Department of Hematology, University Hospital, Rennes, France; FAU - Reman, Oumedaly AU - Reman O AD - Department of Hematology, University Hospital, Caen, France; FAU - Graux, Carlos AU - Graux C AD - Department of Hematology, University Hospital, Yvoir, Belgium; FAU - Chalandon, Yves AU - Chalandon Y AD - Division of Hematology, Department of Medical Specialties, University Hospital, Geneva, and Faculty of Medicine, University of Geneva Switzerland and Swiss Group for Clinical Cancer Research, Bern, Switzerland; FAU - Blaise, Didier AU - Blaise D AD - Department of Hematology, Institut Paoli Calmettes, Marseille, France; FAU - Schanz, Urs AU - Schanz U AD - Division of Hematology, University Hospital of Zurich, Zurich, Switzerland; FAU - Lhéritier, Véronique AU - Lhéritier V AD - Group for Research on Adult Acute Lymphoblastic Leukemia, Coordination Office, Pierre Bénite, France; FAU - Cahn, Jean-Yves AU - Cahn JY AD - Department of Hematology, University Hospital, Grenoble, France; and. FAU - Dombret, Hervé AU - Dombret H AD - Department of Hematology and University Paris Diderot, Institut Universitaire d'Hématologie, EA-3518, University Hospital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France; FAU - Ifrah, Norbert AU - Ifrah N AD - Department of Hematology and INSERM U 892/CNRS 6299, University Hospital, Angers, France. CN - GRAALL group LA - eng SI - ClinicalTrials.gov/NCT00222027 SI - ClinicalTrials.gov/NCT00327678 PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150113 PL - United States TA - Blood JT - Blood JID - 7603509 SB - IM CIN - Blood. 2015 Apr 16;125(16):2453-4. doi: 10.1182/blood-2015-02-623793. PMID: 25883226 MH - Adolescent MH - Adult MH - *Clinical Trials as Topic MH - Disease-Free Survival MH - Female MH - Humans MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Neoplasm, Residual MH - Outcome Assessment, Health Care/*methods MH - Philadelphia Chromosome MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics/*therapy MH - Remission Induction MH - Stem Cell Transplantation/*methods MH - Transplantation, Homologous MH - Young Adult FIR - Caillères IR - Caillères FIR - Chaib IR - Chaib FIR - Blanc IR - Blanc FIR - Brunel IR - Brunel FIR - Da Silva IR - Da Silva FIR - Cuvelier IR - Cuvelier FIR - Marolleau IR - Marolleau FIR - Damaj IR - Damaj FIR - Vaida IR - Vaida FIR - Royer IR - Royer FIR - Gruson IR - Gruson FIR - Merlusca IR - Merlusca FIR - Copin IR - Copin FIR - Capiod IR - Capiod FIR - Dubus IR - Dubus FIR - Mpari IR - Mpari FIR - Hunault IR - Hunault FIR - Ifrah IR - Ifrah FIR - Schmidt IR - Schmidt FIR - Dib IR - Dib FIR - Francois IR - Francois FIR - Moles IR - Moles FIR - Foussard IR - Foussard FIR - Guardiola IR - Guardiola FIR - Zandecki IR - Zandecki FIR - Blanchet IR - Blanchet FIR - Baranger IR - Baranger FIR - Chassevent IR - Chassevent FIR - Genevieve IR - Genevieve FIR - Marie IR - Marie FIR - Parry IR - Parry FIR - Orsini-Piocelle IR - Orsini-Piocelle FIR - Corront IR - Corront FIR - Daguindau IR - Daguindau FIR - Cony-Makhoul IR - Cony-Makhoul FIR - Reynes IR - Reynes FIR - Cadoux IR - Cadoux FIR - Vittet IR - Vittet FIR - Sutton IR - Sutton FIR - Al Jijakli IR - Al Jijakli FIR - Genet IR - Genet FIR - Chaoui IR - Chaoui FIR - Mesbah IR - Mesbah FIR - Morel IR - Morel FIR - Touahri IR - Touahri FIR - Mossafa IR - Mossafa FIR - Fourcade IR - Fourcade FIR - Guerekobaya IR - Guerekobaya FIR - Zerazhi IR - Zerazhi FIR - Azzedine IR - Azzedine FIR - Boulat IR - Boulat FIR - Lepeu IR - Lepeu FIR - Touchais IR - Touchais FIR - Derre IR - Derre FIR - Beyrne IR - Beyrne FIR - Araujo IR - Araujo FIR - Bauduer IR - Bauduer FIR - Banos IR - Banos FIR - Burtin IR - Burtin FIR - Renoux IR - Renoux FIR - Menard IR - Menard FIR - Labarrère IR - Labarrère FIR - Legrand IR - Legrand FIR - Berceanu IR - Berceanu FIR - Daguindau IR - Daguindau FIR - Deconinck IR - Deconinck FIR - Larosa IR - Larosa FIR - Ferrand IR - Ferrand FIR - Brion IR - Brion FIR - Collonge-Rame IR - Collonge-Rame FIR - Garnache Ottou IR - Garnache Ottou FIR - Peria IR - Peria FIR - Rodon IR - Rodon FIR - Elyamadi IR - Elyamadi FIR - Kadiri IR - Kadiri FIR - Gardin IR - Gardin FIR - Ades IR - Ades FIR - Fenaux IR - Fenaux FIR - Braun IR - Braun FIR - Boulalam IR - Boulalam FIR - Eclache IR - Eclache FIR - Letestu IR - Letestu FIR - Nloga IR - Nloga FIR - Beve IR - Beve FIR - Leguay IR - Leguay FIR - Milpied IR - Milpied FIR - Lascaux IR - Lascaux FIR - Dilhuydy IR - Dilhuydy FIR - Dimicoli IR - Dimicoli FIR - Pigneux IR - Pigneux FIR - Tabrizi IR - Tabrizi FIR - Dumas IR - Dumas FIR - Lacombe IR - Lacombe FIR - Lippert IR - Lippert FIR - Boiron IR - Boiron FIR - Bilhou-Nabera IR - Bilhou-Nabera FIR - Marit IR - Marit FIR - Sauvezie IR - Sauvezie FIR - Perry IR - Perry FIR - Choufi IR - Choufi FIR - Kadiata IR - Kadiata FIR - Barry IR - Barry FIR - Voronina IR - Voronina FIR - Brument IR - Brument FIR - Nouvel IR - Nouvel FIR - Guillerm IR - Guillerm FIR - Berthou IR - Berthou FIR - Dalbies IR - Dalbies FIR - Tempescul IR - Tempescul FIR - Ianotto IR - Ianotto FIR - Couturier IR - Couturier FIR - Eveillard IR - Eveillard FIR - Ugo IR - Ugo FIR - De Braekeleer IR - De Braekeleer FIR - Le Calvez IR - Le Calvez FIR - Gillet IR - Gillet FIR - Reman IR - Reman FIR - Leporrier IR - Leporrier FIR - Chantepie IR - Chantepie FIR - Johnson IR - Johnson FIR - Ansah IR - Ansah FIR - Gac IR - Gac FIR - Macro IR - Macro FIR - Benabed IR - Benabed FIR - Cheze IR - Cheze FIR - Naguib IR - Naguib FIR - Plessis IR - Plessis FIR - Salaun IR - Salaun FIR - Lepesant IR - Lepesant FIR - Marin IR - Marin FIR - Malfuson IR - Malfuson FIR - Konopacki IR - Konopacki FIR - Souleau IR - Souleau FIR - De Revel IR - De Revel FIR - Fagot IR - Fagot FIR - Foissaud IR - Foissaud FIR - Samson IR - Samson FIR - Desangles IR - Desangles FIR - Harrouche IR - Harrouche FIR - Cacheux IR - Cacheux FIR - Bay IR - Bay FIR - Tournilhac IR - Tournilhac FIR - Hermet IR - Hermet FIR - Guieze IR - Guieze FIR - Bailly IR - Bailly FIR - Chaleteix IR - Chaleteix FIR - De Renzis IR - De Renzis FIR - Chabrot IR - Chabrot FIR - Calvet IR - Calvet FIR - Sapin IR - Sapin FIR - Tchirkov IR - Tchirkov FIR - Périssel IR - Périssel FIR - Veronese IR - Veronese FIR - Chassagne Berger IR - Chassagne Berger FIR - Villemagne IR - Villemagne FIR - Latiere IR - Latiere FIR - Giollant IR - Giollant FIR - Roy IR - Roy FIR - Auduy IR - Auduy FIR - Raby IR - Raby FIR - Kohser IR - Kohser FIR - Humbrecht IR - Humbrecht FIR - Barats IR - Barats FIR - Husseini IR - Husseini FIR - Moskovtchenko IR - Moskovtchenko FIR - Mazurier IR - Mazurier FIR - Bauly IR - Bauly FIR - Camara IR - Camara FIR - Salanoubat IR - Salanoubat FIR - Haiat IR - Haiat FIR - Petitdidier IR - Petitdidier FIR - Cereja IR - Cereja FIR - Joly IR - Joly FIR - Devidas IR - Devidas FIR - Quillet IR - Quillet FIR - Boutant IR - Boutant FIR - Maury IR - Maury FIR - Cordonnier IR - Cordonnier FIR - Pautas IR - Pautas FIR - Toma IR - Toma FIR - Hichri IR - Hichri FIR - Kuentz IR - Kuentz FIR - Bories IR - Bories FIR - Jouault IR - Jouault FIR - Wagner-Ballon IR - Wagner-Ballon FIR - Perot IR - Perot FIR - Plonquet IR - Plonquet FIR - Beaune IR - Beaune FIR - Reyes IR - Reyes FIR - Cabanne IR - Cabanne FIR - Caillot IR - Caillot FIR - Lafon IR - Lafon FIR - Casasnovas IR - Casasnovas FIR - Ferrant IR - Ferrant FIR - Bastié IR - Bastié FIR - Teyssier IR - Teyssier FIR - Mugneret IR - Mugneret FIR - Menadier IR - Menadier FIR - Favre-Audry IR - Favre-Audry FIR - Grandjean IR - Grandjean FIR - Pignon IR - Pignon FIR - Bemba IR - Bemba FIR - Wetterwald IR - Wetterwald FIR - Roumier IR - Roumier FIR - Paquez IR - Paquez FIR - Cahn IR - Cahn FIR - Thiebaut IR - Thiebaut FIR - Gressin IR - Gressin FIR - Bulabois IR - Bulabois FIR - Simon IR - Simon FIR - Pégourie IR - Pégourie FIR - Courby IR - Courby FIR - Molina IR - Molina FIR - Callanan IR - Callanan FIR - Lefebvre IR - Lefebvre FIR - Jacob IR - Jacob FIR - Garban IR - Garban FIR - Rolland IR - Rolland FIR - Leroux IR - Leroux FIR - Pittet IR - Pittet FIR - Agapé IR - Agapé FIR - Belkaid IR - Belkaid FIR - Henni IR - Henni FIR - Berg IR - Berg FIR - Pequin IR - Pequin FIR - Stalnikiewicz IR - Stalnikiewicz FIR - Dupriez IR - Dupriez FIR - Morel IR - Morel FIR - Poulain IR - Poulain FIR - Declercq IR - Declercq FIR - Berthon IR - Berthon FIR - Quesnel IR - Quesnel FIR - Preudhomme IR - Preudhomme FIR - Grardel IR - Grardel FIR - Roche IR - Roche FIR - Lestienne IR - Lestienne FIR - Lai IR - Lai FIR - Lepelley IR - Lepelley FIR - Darre IR - Darre FIR - de Botton IR - de Botton FIR - Soenen IR - Soenen FIR - Bauters IR - Bauters FIR - Djeda IR - Djeda FIR - Turlure IR - Turlure FIR - Bordessoule IR - Bordessoule FIR - Touati IR - Touati FIR - Remenieras IR - Remenieras FIR - Abraham IR - Abraham FIR - Gourin IR - Gourin FIR - Girault IR - Girault FIR - Moreau IR - Moreau FIR - Jaccard IR - Jaccard FIR - Chaury IR - Chaury FIR - Penot IR - Penot FIR - Trimoreau IR - Trimoreau FIR - Gachard IR - Gachard FIR - Feuillard IR - Feuillard FIR - Tisseuil IR - Tisseuil FIR - Philippon IR - Philippon FIR - Bosselut IR - Bosselut FIR - Nlend IR - Nlend FIR - Thomas IR - Thomas FIR - Chelghoum IR - Chelghoum FIR - Michallet IR - Michallet FIR - Salles IR - Salles FIR - Coiffier IR - Coiffier FIR - Espinouse IR - Espinouse FIR - Karlin IR - Karlin FIR - Bouafia-Sauvy IR - Bouafia-Sauvy FIR - Broussais-Guillaumot IR - Broussais-Guillaumot FIR - Traullé IR - Traullé FIR - Callet-Bochu IR - Callet-Bochu FIR - Tigaud IR - Tigaud FIR - Hayette IR - Hayette FIR - Nicolini IR - Nicolini FIR - Ducastelle IR - Ducastelle FIR - Labussière IR - Labussière FIR - Detrait IR - Detrait FIR - Plesa IR - Plesa FIR - Le Tavernier IR - Le Tavernier FIR - Thiebaut IR - Thiebaut FIR - Girard IR - Girard FIR - Wattel IR - Wattel FIR - Boucher IR - Boucher FIR - Maire IR - Maire FIR - Etienne IR - Etienne FIR - Prebet IR - Prebet FIR - Charbonnier IR - Charbonnier FIR - Brunelle IR - Brunelle FIR - d'Incan IR - d'Incan FIR - Vey IR - Vey FIR - Rey IR - Rey FIR - Mozziconacci IR - Mozziconacci FIR - Sainty IR - Sainty FIR - Laibes IR - Laibes FIR - Arnoulet IR - Arnoulet FIR - Stoppa IR - Stoppa FIR - Mouton IR - Mouton FIR - Lafage-Pochitaloff IR - Lafage-Pochitaloff FIR - Blaise IR - Blaise FIR - Dridi IR - Dridi FIR - Redortier IR - Redortier FIR - Frayfer IR - Frayfer FIR - Allard IR - Allard FIR - Mossafa IR - Mossafa FIR - Matis IR - Matis FIR - Dorvaux IR - Dorvaux FIR - Guibaud IR - Guibaud FIR - Zamfir IR - Zamfir FIR - Christian IR - Christian FIR - Staal IR - Staal FIR - Benet IR - Benet FIR - Hagopian IR - Hagopian FIR - Vincent IR - Vincent FIR - Quittet IR - Quittet FIR - Navarro IR - Navarro FIR - Ceballos IR - Ceballos FIR - Fegueux IR - Fegueux FIR - Cartron IR - Cartron FIR - Legouffe IR - Legouffe FIR - Rossi IR - Rossi FIR - Tondeur IR - Tondeur FIR - Cacheux IR - Cacheux FIR - Bret IR - Bret FIR - Taviaux IR - Taviaux FIR - Portales IR - Portales FIR - Lavabre IR - Lavabre FIR - Taib IR - Taib FIR - Eliaou IR - Eliaou FIR - Grosjean IR - Grosjean FIR - Dupont IR - Dupont FIR - Leroux IR - Leroux FIR - Theis IR - Theis FIR - Mulhouse IR - Mulhouse FIR - Ojeda-Uribe IR - Ojeda-Uribe FIR - Drenou IR - Drenou FIR - Eisenmann IR - Eisenmann FIR - Arkam IR - Arkam FIR - Rimelen IR - Rimelen FIR - Jeandidier IR - Jeandidier FIR - Debliquis IR - Debliquis FIR - Isaac IR - Isaac FIR - Iglarz IR - Iglarz FIR - Haby IR - Haby FIR - Bonmati IR - Bonmati FIR - Witz IR - Witz FIR - Witz IR - Witz FIR - Feugier IR - Feugier FIR - Randa IR - Randa FIR - Namour IR - Namour FIR - Grégoire IR - Grégoire FIR - Monhoven IR - Monhoven FIR - Léotard IR - Léotard FIR - Jonveaux IR - Jonveaux FIR - Béné IR - Béné FIR - Schwartz IR - Schwartz FIR - Harousseau IR - Harousseau FIR - Milpied IR - Milpied FIR - Moreau IR - Moreau FIR - Mohty IR - Mohty FIR - Le Gouill IR - Le Gouill FIR - Chevallier IR - Chevallier FIR - Guillaume IR - Guillaume FIR - Delaunay IR - Delaunay FIR - Gastinne IR - Gastinne FIR - Mahé IR - Mahé FIR - Peterlin IR - Peterlin FIR - Vigouroux IR - Vigouroux FIR - Lodé IR - Lodé FIR - Garand IR - Garand FIR - Robillard IR - Robillard FIR - Talmant IR - Talmant FIR - Avet-Loiseau IR - Avet-Loiseau FIR - Saulquin IR - Saulquin FIR - Le Houerou IR - Le Houerou FIR - Sirvent IR - Sirvent FIR - Mannone IR - Mannone FIR - Gratecos IR - Gratecos FIR - Legros IR - Legros FIR - Raynaud IR - Raynaud FIR - Philip IR - Philip FIR - Ticchioni IR - Ticchioni FIR - Touitou IR - Touitou FIR - Bouskila IR - Bouskila FIR - Jourdan IR - Jourdan FIR - Richard IR - Richard FIR - Gaillard IR - Gaillard FIR - Arnaud IR - Arnaud FIR - Chiesa IR - Chiesa FIR - Brun IR - Brun FIR - Nicolas IR - Nicolas FIR - Alexis IR - Alexis FIR - Boulet IR - Boulet FIR - Carp IR - Carp FIR - Benbrahim IR - Benbrahim FIR - Schoenwald IR - Schoenwald FIR - Legac IR - Legac FIR - Michel IR - Michel FIR - Dreyfus IR - Dreyfus FIR - Bouscary IR - Bouscary FIR - Al Nawakil IR - Al Nawakil FIR - Picard IR - Picard FIR - Vigue IR - Vigue FIR - Khelfaoui IR - Khelfaoui FIR - Mekdour IR - Mekdour FIR - Buzyn IR - Buzyn FIR - Suarez IR - Suarez FIR - Delarue IR - Delarue FIR - Sibon IR - Sibon FIR - Cheminant IR - Cheminant FIR - Marcais IR - Marcais FIR - Frenzel IR - Frenzel FIR - Brignier IR - Brignier FIR - Asnafi IR - Asnafi FIR - Radford-Weiss IR - Radford-Weiss FIR - MacIntyre IR - MacIntyre FIR - Couderc IR - Couderc FIR - Valensi IR - Valensi FIR - Varet IR - Varet FIR - Berdous Saheb IR - Berdous Saheb FIR - Isnard IR - Isnard FIR - Legrand IR - Legrand FIR - Lapusan IR - Lapusan FIR - Marzac IR - Marzac FIR - Perot IR - Perot FIR - Feger IR - Feger FIR - Negadi IR - Negadi FIR - Van den Akker IR - Van den Akker FIR - Laporte IR - Laporte FIR - Gorin IR - Gorin FIR - Ikhlef IR - Ikhlef FIR - Dombret IR - Dombret FIR - Raffoux IR - Raffoux FIR - Lengline IR - Lengline FIR - Boissel IR - Boissel FIR - Cluzeau IR - Cluzeau FIR - Micléa IR - Micléa FIR - Zini IR - Zini FIR - Cayuela IR - Cayuela FIR - Maarek IR - Maarek FIR - de Labarthe IR - de Labarthe FIR - Beldjord IR - Beldjord FIR - Daniel IR - Daniel FIR - Soulier IR - Soulier FIR - Dhédin IR - Dhédin FIR - Treilhou IR - Treilhou FIR - Klein IR - Klein FIR - Vernant IR - Vernant FIR - Leblond IR - Leblond FIR - Dhedin IR - Dhedin FIR - Nguyen IR - Nguyen FIR - Merle-Beral IR - Merle-Beral FIR - Davi IR - Davi FIR - Aliammar IR - Aliammar FIR - Nguyen-Khac IR - Nguyen-Khac FIR - Sanhes IR - Sanhes FIR - Karangwa IR - Karangwa FIR - Burcheri IR - Burcheri FIR - Roland IR - Roland FIR - Vallantin IR - Vallantin FIR - Andary IR - Andary FIR - Gueudet IR - Gueudet FIR - Barbier IR - 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De Jaurreguiberry IR - De Jaurreguiberry FIR - Huguet IR - Huguet FIR - Huynh IR - Huynh FIR - Roussel IR - Roussel FIR - Tavitian IR - Tavitian FIR - Ysebaert IR - Ysebaert FIR - Recher IR - Recher FIR - Borel IR - Borel FIR - Hebraud IR - Hebraud FIR - Oberic IR - Oberic FIR - Laurent IR - Laurent FIR - Nouvel IR - Nouvel FIR - Delabesse IR - Delabesse FIR - Kuhlein IR - Kuhlein FIR - Dastugue IR - Dastugue FIR - Demas IR - Demas FIR - Luquet IR - Luquet FIR - Vergez IR - Vergez FIR - Attal IR - Attal FIR - Daniel IR - Daniel FIR - Leclerc IR - Leclerc FIR - Lissandre IR - Lissandre FIR - Dartigeas IR - Dartigeas FIR - Gyan IR - Gyan FIR - Renaud IR - Renaud FIR - Monjanel IR - Monjanel FIR - Ertault de la Bretonniere IR - Ertault de la Bretonniere FIR - Benboubker IR - Benboubker FIR - Estienne IR - Estienne FIR - Barin IR - Barin FIR - Watier IR - Watier FIR - Delain IR - Delain FIR - Delepine IR - Delepine FIR - Degene IR - Degene FIR - Colombat IR - Colombat FIR - Nollet IR - Nollet FIR - Fernandes IR - Fernandes FIR - Pollet IR - Pollet FIR - Tricot IR - Tricot FIR - Simon IR - Simon FIR - Bremeault IR - Bremeault FIR - Daudignon IR - Daudignon FIR - Bisiau IR - Bisiau FIR - Poulain IR - Poulain FIR - Crametz IR - Crametz FIR - Rousselot IR - Rousselot FIR - Castaigne IR - Castaigne FIR - Salmeron IR - Salmeron FIR - Lambert IR - Lambert FIR - Taksin IR - Taksin FIR - Rigaudeau IR - Rigaudeau FIR - Farhat IR - Farhat FIR - Merabet IR - Merabet FIR - Ghez IR - Ghez FIR - Spentchian IR - Spentchian FIR - Terre IR - Terre FIR - Garcia IR - Garcia FIR - Pousset IR - Pousset FIR - Henri IR - Henri FIR - de Botton IR - de Botton FIR - Bourhis IR - Bourhis FIR - Arnaud IR - Arnaud FIR - Micol IR - Micol FIR - Garnier IR - Garnier FIR - Willekens IR - Willekens FIR - Bennaceur IR - Bennaceur FIR - Auger IR - Auger FIR - Saada IR - Saada FIR - Bayle IR - Bayle FIR - Bernheim IR - Bernheim FIR - Ferrant IR - Ferrant FIR - Costantini IR - Costantini FIR - Libouton IR - Libouton FIR - Poirel IR - Poirel FIR - Mineur IR - Mineur FIR - Delannoy IR - Delannoy FIR - Tacal IR - Tacal FIR - Rack IR - Rack FIR - Bosly IR - Bosly FIR - Crasson IR - Crasson FIR - Michaux IR - Michaux FIR - Vandenbeghe IR - Vandenbeghe FIR - Hagemeijer IR - Hagemeijer FIR - Bargetzi IR - Bargetzi FIR - Heizmann IR - Heizmann FIR - Sigle IR - Sigle FIR - Gurtner IR - Gurtner FIR - Gratwohl IR - Gratwohl FIR - Arber IR - Arber FIR - Stern IR - Stern FIR - Heim IR - Heim FIR - Meili IR - Meili FIR - Gerulls IR - Gerulls FIR - Servida IR - Servida FIR - Marongui IR - Marongui FIR - Christinat IR - Christinat FIR - Steffanoni IR - Steffanoni FIR - Taborelli IR - Taborelli FIR - Cresto IR - Cresto FIR - Busi IR - Busi FIR - Pabst IR - Pabst FIR - Rettich IR - Rettich FIR - Chalandon IR - Chalandon FIR - Passweg IR - Passweg FIR - Tirefort IR - Tirefort FIR - Salamanczuk IR - Salamanczuk FIR - Trembleau IR - Trembleau FIR - Lambert IR - Lambert FIR - Spertini IR - Spertini FIR - Jotterand IR - Jotterand FIR - Schoumans Pouw IR - Schoumans Pouw FIR - Kunkel IR - Kunkel FIR - Quarroz IR - Quarroz FIR - Mulhematter IR - Mulhematter FIR - Porter IR - Porter FIR - Abbal IR - Abbal FIR - Mme Jeckelmann IR - Mme Jeckelmann FIR - Voegtin IR - Voegtin FIR - Gregor IR - Gregor FIR - Wuillemin IR - Wuillemin FIR - Nagler IR - Nagler FIR - Fahrni IR - Fahrni FIR - Driessen IR - Driessen FIR - Hess IR - Hess FIR - Hitz IR - Hitz FIR - Weder IR - Weder FIR - Raess IR - Raess FIR - Demmer IR - Demmer FIR - Jacky IR - Jacky FIR - Reiner IR - Reiner FIR - Schanz IR - Schanz FIR - Sasselli IR - Sasselli EDAT- 2015/01/15 06:00 MHDA- 2015/07/21 06:00 CRDT- 2015/01/15 06:00 PHST- 2014/09/08 00:00 [received] PHST- 2014/12/25 00:00 [accepted] PHST- 2015/01/15 06:00 [entrez] PHST- 2015/01/15 06:00 [pubmed] PHST- 2015/07/21 06:00 [medline] AID - S0006-4971(20)31788-2 [pii] AID - 10.1182/blood-2014-09-599894 [doi] PST - ppublish SO - Blood. 2015 Apr 16;125(16):2486-96; quiz 2586. doi: 10.1182/blood-2014-09-599894. Epub 2015 Jan 13. PMID- 29425244 OWN - NLM STAT- MEDLINE DCOM- 20180703 LR - 20181113 IS - 1553-7374 (Electronic) IS - 1553-7366 (Print) IS - 1553-7366 (Linking) VI - 14 IP - 2 DP - 2018 Feb TI - Initiation, extension, and termination of RNA synthesis by a paramyxovirus polymerase. PG - e1006889 LID - 10.1371/journal.ppat.1006889 [doi] LID - e1006889 AB - Paramyxoviruses represent a family of RNA viruses causing significant human diseases. These include measles virus, the most infectious virus ever reported, in addition to parainfluenza virus, and other emerging viruses. Paramyxoviruses likely share common replication machinery but their mechanisms of RNA biosynthesis activities and details of their complex polymerase structures are unknown. Mechanistic and functional details of a paramyxovirus polymerase would have sweeping implications for understanding RNA virus replication and for the development of new antiviral medicines. To study paramyxovirus polymerase structure and function, we expressed an active recombinant Nipah virus (NiV) polymerase complex assembled from the multifunctional NiV L protein bound to its phosphoprotein cofactor. NiV is an emerging highly pathogenic virus that causes severe encephalitis and has been declared a global public health concern due to its high mortality rate. Using negative-stain electron microscopy, we demonstrated NiV polymerase forms ring-like particles resembling related RNA polymerases. We identified conserved sequence elements driving recognition of the 3'-terminal genomic promoter by NiV polymerase, and leading to initiation of RNA synthesis, primer extension, and transition to elongation mode. Polyadenylation resulting from NiV polymerase stuttering provides a mechanistic basis for transcription termination. It also suggests a divergent adaptation in promoter recognition between pneumo- and paramyxoviruses. The lack of available antiviral therapy for NiV prompted us to identify the triphosphate forms of R1479 and GS-5734, two clinically relevant nucleotide analogs, as substrates and inhibitors of NiV polymerase activity by delayed chain termination. Overall, these findings provide low-resolution structural details and the mechanism of an RNA polymerase from a previously uncharacterized virus family. This work illustrates important functional differences yet remarkable similarities between the polymerases of nonsegmented negative-strand RNA viruses. FAU - Jordan, Paul C AU - Jordan PC AUID- ORCID: 0000-0003-3265-2911 AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. FAU - Liu, Cheng AU - Liu C AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. FAU - Raynaud, Pauline AU - Raynaud P AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. FAU - Lo, Michael K AU - Lo MK AD - Centers for Disease Control and Prevention, Atlanta, Georgia, United States of America. FAU - Spiropoulou, Christina F AU - Spiropoulou CF AUID- ORCID: 0000-0001-8406-3161 AD - Centers for Disease Control and Prevention, Atlanta, Georgia, United States of America. FAU - Symons, Julian A AU - Symons JA AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. FAU - Beigelman, Leo AU - Beigelman L AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. FAU - Deval, Jerome AU - Deval J AUID- ORCID: 0000-0001-8369-907X AD - Alios BioPharma, Inc. a Janssen Pharmaceutical Company of Johnson & Johnson, South San Francisco, California, United States of America. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180209 PL - United States TA - PLoS Pathog JT - PLoS pathogens JID - 101238921 RN - 0 (P protein, Nipah virus) RN - 0 (Phosphoproteins) RN - 0 (RNA, Viral) RN - 0 (Viral Proteins) RN - EC 2.7.7.- (L protein, Nipah virus) RN - EC 2.7.7.6 (DNA-Directed RNA Polymerases) SB - IM MH - Amino Acid Sequence MH - DNA-Directed RNA Polymerases/chemistry/genetics/*metabolism MH - Nipah Virus/enzymology/*genetics MH - Paramyxovirinae/enzymology/genetics/metabolism MH - Phosphoproteins/chemistry/genetics/*metabolism MH - RNA, Viral/genetics/metabolism MH - *Transcription Elongation, Genetic MH - *Transcription Initiation, Genetic MH - *Transcription Termination, Genetic MH - Viral Proteins/chemistry/genetics/*metabolism MH - Virus Replication PMC - PMC5823471 COIS- I have read the journal's policy and the authors of this manuscript have the following competing interests: PCJ, CL, PR, JAS, LB, and JD are past or current employees of Alios BioPharma, Inc., a Janssen Pharmaceutical Company of Johnson & Johnson EDAT- 2018/02/10 06:00 MHDA- 2018/07/04 06:00 PMCR- 2018/02/09 CRDT- 2018/02/10 06:00 PHST- 2017/11/07 00:00 [received] PHST- 2018/01/21 00:00 [accepted] PHST- 2018/02/22 00:00 [revised] PHST- 2018/02/10 06:00 [pubmed] PHST- 2018/07/04 06:00 [medline] PHST- 2018/02/10 06:00 [entrez] PHST- 2018/02/09 00:00 [pmc-release] AID - PPATHOGENS-D-17-02404 [pii] AID - 10.1371/journal.ppat.1006889 [doi] PST - epublish SO - PLoS Pathog. 2018 Feb 9;14(2):e1006889. doi: 10.1371/journal.ppat.1006889. eCollection 2018 Feb. PMID- 27935476 OWN - NLM STAT- MEDLINE DCOM- 20171101 LR - 20250530 IS - 2050-084X (Electronic) IS - 2050-084X (Linking) VI - 5 DP - 2016 Dec 9 TI - Assessing the mechanism and therapeutic potential of modulators of the human Mediator complex-associated protein kinases. LID - e20722 [pii] LID - 10.7554/eLife.20722 [doi] AB - Mediator-associated kinases CDK8/19 are context-dependent drivers or suppressors of tumorigenesis. Their inhibition is predicted to have pleiotropic effects, but it is unclear whether this will impact on the clinical utility of CDK8/19 inhibitors. We discovered two series of potent chemical probes with high selectivity for CDK8/19. Despite pharmacodynamic evidence for robust on-target activity, the compounds exhibited modest, though significant, efficacy against human tumor lines and patient-derived xenografts. Altered gene expression was consistent with CDK8/19 inhibition, including profiles associated with super-enhancers, immune and inflammatory responses and stem cell function. In a mouse model expressing oncogenic beta-catenin, treatment shifted cells within hyperplastic intestinal crypts from a stem cell to a transit amplifying phenotype. In two species, neither probe was tolerated at therapeutically-relevant exposures. The complex nature of the toxicity observed with two structurally-differentiated chemical series is consistent with on-target effects posing significant challenges to the clinical development of CDK8/19 inhibitors. FAU - Clarke, Paul A AU - Clarke PA AUID- ORCID: 0000-0001-9342-1290 AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Ortiz-Ruiz, Maria-Jesus AU - Ortiz-Ruiz MJ AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - TePoele, Robert AU - TePoele R AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Adeniji-Popoola, Olajumoke AU - Adeniji-Popoola O AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Box, Gary AU - Box G AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Court, Will AU - Court W AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Czasch, Stephanie AU - Czasch S AD - Merck KGaA, Darmstadt, Germany. FAU - El Bawab, Samer AU - El Bawab S AD - Merck KGaA, Darmstadt, Germany. FAU - Esdar, Christina AU - Esdar C AD - Merck KGaA, Darmstadt, Germany. FAU - Ewan, Ken AU - Ewan K AD - School of Bioscience, Cardiff University, Cardiff, United Kingdom. FAU - Gowan, Sharon AU - Gowan S AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - De Haven Brandon, Alexis AU - De Haven Brandon A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Hewitt, Phillip AU - Hewitt P AD - Merck KGaA, Darmstadt, Germany. FAU - Hobbs, Stephen M AU - Hobbs SM AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Kaufmann, Wolfgang AU - Kaufmann W AD - Merck KGaA, Darmstadt, Germany. FAU - Mallinger, Aurélie AU - Mallinger A AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Raynaud, Florence AU - Raynaud F AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Roe, Toby AU - Roe T AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Rohdich, Felix AU - Rohdich F AD - Merck KGaA, Darmstadt, Germany. FAU - Schiemann, Kai AU - Schiemann K AD - Merck KGaA, Darmstadt, Germany. FAU - Simon, Stephanie AU - Simon S AD - Merck KGaA, Darmstadt, Germany. FAU - Schneider, Richard AU - Schneider R AD - Merck KGaA, Darmstadt, Germany. FAU - Valenti, Melanie AU - Valenti M AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Weigt, Stefan AU - Weigt S AD - Merck KGaA, Darmstadt, Germany. FAU - Blagg, Julian AU - Blagg J AUID- ORCID: 0000-0002-7409-0323 AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Blaukat, Andree AU - Blaukat A AD - Merck KGaA, Darmstadt, Germany. FAU - Dale, Trevor C AU - Dale TC AD - School of Bioscience, Cardiff University, Cardiff, United Kingdom. FAU - Eccles, Suzanne A AU - Eccles SA AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Hecht, Stefan AU - Hecht S AD - Merck KGaA, Darmstadt, Germany. FAU - Urbahns, Klaus AU - Urbahns K AD - Merck KGaA, Darmstadt, Germany. FAU - Workman, Paul AU - Workman P AUID- ORCID: 0000-0003-1659-3034 AD - Cancer Research UK Cancer Therapeutics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Wienke, Dirk AU - Wienke D AD - Merck KGaA, Darmstadt, Germany. LA - eng GR - 2008MAYPR16/BBC_/Breast Cancer Now/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - 15937/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - C368/A6743/CRUK_/Cancer Research UK/United Kingdom GR - A368/A7990/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20161209 PL - England TA - Elife JT - eLife JID - 101579614 RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antineoplastic Agents) RN - 0 (Mediator Complex) RN - 0 (Protein Kinase Inhibitors) RN - EC 2.7.11.22 (CDK19 protein, human) RN - EC 2.7.11.22 (CDK8 protein, human) RN - EC 2.7.11.22 (Cyclin-Dependent Kinase 8) RN - EC 2.7.11.22 (Cyclin-Dependent Kinases) SB - IM MH - Animals MH - Anti-Inflammatory Agents/*administration & dosage/adverse effects/toxicity MH - Antineoplastic Agents/*administration & dosage/adverse effects/toxicity MH - Cyclin-Dependent Kinase 8/*antagonists & inhibitors MH - Cyclin-Dependent Kinases/*antagonists & inhibitors MH - Disease Models, Animal MH - Heterografts MH - Humans MH - Hyperplasia/drug therapy MH - Mediator Complex/*antagonists & inhibitors MH - Mice MH - Neoplasms/drug therapy MH - Protein Kinase Inhibitors/*administration & dosage/adverse effects/toxicity MH - Treatment Outcome PMC - PMC5224920 OTO - NOTNLM OT - CDK8 OT - Mediator complex OT - Wnt OT - cancer biology OT - human OT - human biology OT - inhibitor OT - medicine OT - super-enhancer COIS- PAC: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. M-JO-R: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. RT: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. OA-P: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. GB: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. WC: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. SC: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. SEB: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. CE: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. SG: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. ADHB: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. PH: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. SMH: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. WK: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. AM: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. FRa: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. TR: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. FRo: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. KS: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. SS: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. RS: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. MV: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. SW: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. JB: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. AB: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. SAE: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. SH: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. KU: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. PW: Current or former employee of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. DW: Current or former employee of Merck KGaA (Darmstadt, Germany), which has a commercial interest in the development of WNT pathway inhibitors. The other authors declare that no competing interests exist. EDAT- 2016/12/10 06:00 MHDA- 2017/11/02 06:00 PMCR- 2016/12/10 CRDT- 2016/12/10 06:00 PHST- 2016/08/21 00:00 [received] PHST- 2016/11/29 00:00 [accepted] PHST- 2016/12/10 06:00 [pubmed] PHST- 2017/11/02 06:00 [medline] PHST- 2016/12/10 06:00 [entrez] PHST- 2016/12/10 00:00 [pmc-release] AID - e20722 [pii] AID - 20722 [pii] AID - 10.7554/eLife.20722 [doi] PST - epublish SO - Elife. 2016 Dec 9;5:e20722. doi: 10.7554/eLife.20722. PMID- 39232581 OWN - NLM STAT- Publisher LR - 20260306 IS - 1878-3686 (Electronic) IS - 1535-6108 (Print) IS - 1535-6108 (Linking) DP - 2024 Aug 27 TI - GABAergic neuronal lineage development determines clinically actionable targets in diffuse hemispheric glioma, H3G34-mutant. LID - S1535-6108(24)00305-2 [pii] LID - 10.1016/j.ccell.2024.08.006 [doi] AB - Diffuse hemispheric gliomas, H3G34R/V-mutant (DHG-H3G34), are lethal brain tumors lacking targeted therapies. They originate from interneuronal precursors; however, leveraging this origin for therapeutic insights remains unexplored. Here, we delineate a cellular hierarchy along the interneuron lineage development continuum, revealing that DHG-H3G34 mirror spatial patterns of progenitor streams surrounding interneuron nests, as seen during human brain development. Integrating these findings with genome-wide CRISPR-Cas9 screens identifies genes upregulated in interneuron lineage progenitors as major dependencies. Among these, CDK6 emerges as a targetable vulnerability: DHG-H3G34 tumor cells show enhanced sensitivity to CDK4/6 inhibitors and a CDK6-specific degrader, promoting a shift toward more mature interneuron-like states, reducing tumor growth, and prolonging xenograft survival. Notably, a patient with progressive DHG-H3G34 treated with a CDK4/6 inhibitor achieved 17 months of stable disease. This study underscores interneuronal progenitor-like states, organized in characteristic niches, as a distinct vulnerability in DHG-H3G34, highlighting CDK6 as a promising clinically actionable target. CI - Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved. FAU - Liu, Ilon AU - Liu I AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA; Department of Neurology with Experimental Neurology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin und Humboldt-Universität zu Berlin, 10117 Berlin, Germany; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, BIH Charité Digital Clinician Scientist Program, 10117 Berlin, Germany. FAU - Alencastro Veiga Cruzeiro, Gustavo AU - Alencastro Veiga Cruzeiro G AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Bjerke, Lynn AU - Bjerke L AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Rogers, Rebecca F AU - Rogers RF AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Grabovska, Yura AU - Grabovska Y AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Beck, Alexander AU - Beck A AD - Center for Neuropathology, Ludwig-Maximilians-University, 81377 Munich, Germany. FAU - Mackay, Alan AU - Mackay A AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Barron, Tara AU - Barron T AD - Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Hack, Olivia A AU - Hack OA AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Quezada, Michael A AU - Quezada MA AD - Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA. FAU - Molinari, Valeria AU - Molinari V AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Shaw, McKenzie L AU - Shaw ML AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Perez-Somarriba, Marta AU - Perez-Somarriba M AD - Children & Young People's Unit, Royal Marsden Hospital NHS Trust, Sutton, Surrey SM2 5 NG, UK. FAU - Temelso, Sara AU - Temelso S AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. FAU - Raynaud, Florence AU - Raynaud F AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London SW7 3RK, UK. FAU - Ruddle, Ruth AU - Ruddle R AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London SW7 3RK, UK. FAU - Panditharatna, Eshini AU - Panditharatna E AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Englinger, Bernhard AU - Englinger B AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA; Department of Urology, Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria; Center for Cancer Research, Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria. FAU - Mire, Hafsa M AU - Mire HM AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Jiang, Li AU - Jiang L AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Nascimento, Andrezza AU - Nascimento A AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - LaBelle, Jenna AU - LaBelle J AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Haase, Rebecca AU - Haase R AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Rozowsky, Jacob AU - Rozowsky J AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Neyazi, Sina AU - Neyazi S AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Baumgartner, Alicia-Christina AU - Baumgartner AC AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Castellani, Sophia AU - Castellani S AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Hoffman, Samantha E AU - Hoffman SE AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. FAU - Cameron, Amy AU - Cameron A AD - Lurie Family Imaging Center, Center for Biomedical Imaging in Oncology, Dana-Farber Cancer Institute, Boston, MA 02215, USA. FAU - Morrow, Murry AU - Morrow M AD - Lurie Family Imaging Center, Center for Biomedical Imaging in Oncology, Dana-Farber Cancer Institute, Boston, MA 02215, USA. FAU - Nguyen, Quang-De AU - Nguyen QD AD - Lurie Family Imaging Center, Center for Biomedical Imaging in Oncology, Dana-Farber Cancer Institute, Boston, MA 02215, USA. FAU - Pericoli, Giulia AU - Pericoli G AD - Department of Onco-haematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital-IRCCS, 00165 Rome, Italy. FAU - Madlener, Sibylle AU - Madlener S AD - Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics and Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria. FAU - Mayr, Lisa AU - Mayr L AD - Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics and Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria. FAU - Dorfer, Christian AU - Dorfer C AD - Department of Neurosurgery, Medical University of Vienna, 1090 Vienna, Austria. FAU - Geyeregger, Rene AU - Geyeregger R AD - Clinical Cell Biology, Children's Cancer Research Institute (CCRI), Vienna 1090, Austria. FAU - Rota, Christopher AU - Rota C AD - Department of Neurobiology, Harvard Medical School, Boston, MA 02215, USA. FAU - Ricken, Gerda AU - Ricken G AD - Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna 1090, Austria. FAU - Ligon, Keith L AU - Ligon KL AD - Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA; Department of Oncologic Pathology, Dana-Farber Cancer Institute, Boston, MA 02215, USA; Department of Pathology, Brigham and Women's Hospital, Boston, MA 02215, USA; Department of Pathology, Boston Children's Hospital, Boston, MA 02115, USA. FAU - Alexandrescu, Sanda AU - Alexandrescu S AD - Department of Pathology, Boston Children's Hospital, Boston, MA 02115, USA. FAU - Cartaxo, Rodrigo T AU - Cartaxo RT AD - Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA. FAU - Lau, Benison AU - Lau B AD - Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA. FAU - Uphadhyaya, Santhosh AU - Uphadhyaya S AD - Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA. FAU - Koschmann, Carl AU - Koschmann C AD - Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA. FAU - Braun, Emelie AU - Braun E AD - Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Danan-Gotthold, Miri AU - Danan-Gotthold M AD - Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Hu, Lijuan AU - Hu L AD - Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Siletti, Kimberly AU - Siletti K AD - Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Sundström, Erik AU - Sundström E AD - Division of Neurodegeneration, Department of Neurobiology, Care Sciences and Society, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Hodge, Rebecca AU - Hodge R AD - Allen Institute for Brain Science, Seattle, WA 98109, USA. FAU - Lein, Ed AU - Lein E AD - Allen Institute for Brain Science, Seattle, WA 98109, USA. FAU - Agnihotri, Sameer AU - Agnihotri S AD - Departments of Neurosurgery and Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA 15224, USA. FAU - Eisenstat, David D AU - Eisenstat DD AD - Murdoch Children's Research Institute, Department of Paediatrics, University of Melbourne, Parkville, VIC 3052, Australia. FAU - Stapleton, Simon AU - Stapleton S AD - Department of Neurosurgery, St George's Hospital NHS Trust, London SW17 0QT, UK. FAU - King, Andrew AU - King A AD - Department of Neuropathology, King's College Hospital NHS Trust, London SE5 9RS, UK. FAU - Bleil, Cristina AU - Bleil C AD - Department of Neurosurgery, King's College Hospital NHS Trust, London SE5 9RS, UK. FAU - Mastronuzzi, Angela AU - Mastronuzzi A AD - Department of Onco-haematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital-IRCCS, 00165 Rome, Italy. FAU - Cole, Kristina A AU - Cole KA AD - Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. FAU - Waanders, Angela J AU - Waanders AJ AD - Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA. FAU - Montero Carcaboso, Angel AU - Montero Carcaboso A AD - Laboratory of Molecular Oncology, Hospital Sant Joan de Déu, 08950 Barcelona, Spain. FAU - Schüller, Ulrich AU - Schüller U AD - Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, 20251 Hamburg, Germany. FAU - Hargrave, Darren AU - Hargrave D AD - University College London Great Ormond Street Institute for Child Health, London WC1N 1EH, UK. FAU - Vinci, Maria AU - Vinci M AD - Department of Onco-haematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital-IRCCS, 00165 Rome, Italy. FAU - Carceller, Fernando AU - Carceller F AD - Children & Young People's Unit, Royal Marsden Hospital NHS Trust, Sutton, Surrey SM2 5 NG, UK; Division of Clinical Studies, The Institute of Cancer Research, London SW7 3RK, UK. FAU - Haberler, Christine AU - Haberler C AD - Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna 1090, Austria. FAU - Slavc, Irene AU - Slavc I AD - Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics and Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria. FAU - Linnarsson, Sten AU - Linnarsson S AD - Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, 17177 Stockholm, Sweden. FAU - Gojo, Johannes AU - Gojo J AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics and Comprehensive Cancer Center, Medical University of Vienna, 1090 Vienna, Austria. FAU - Monje, Michelle AU - Monje M AD - Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA; Howard Hughes Medical Institute, Stanford, CA, USA. FAU - Jones, Chris AU - Jones C AD - Division of Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey SM2 5 NG, UK. Electronic address: chris.jones@icr.ac.uk. FAU - Filbin, Mariella G AU - Filbin MG AD - Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. Electronic address: mariella.filbin@childrens.harvard.edu. LA - eng GR - T32 GM144273/GM/NIGMS NIH HHS/United States GR - R01 NS119231/NS/NINDS NIH HHS/United States GR - DP2 NS127705/NS/NINDS NIH HHS/United States GR - T32 CA272386/CA/NCI NIH HHS/United States GR - P50 CA165962/CA/NCI NIH HHS/United States PT - Journal Article DEP - 20240827 PL - United States TA - Cancer Cell JT - Cancer cell JID - 101130617 SB - IM PMC - PMC11865364 MID - NIHMS2017911 COIS- Declaration of interests M.G.F. is a consultant for Twentyeight-Seven Therapeutics and Blueprint Medicines. M.M. is an SAB member for Cygnal Therapeutics. K.L.L. is founder and equity holder of Travera Inc. and receives consulting fees from BMS, Integragen, and Rarecyte, and research support from Lilly, BMS, and Amgen. D.H. has acted as an advisor for Novartis in relation to ribociclib. EDAT- 2024/09/05 00:50 MHDA- 2024/09/05 00:50 PMCR- 2026/02/27 CRDT- 2024/09/04 23:52 PHST- 2023/07/11 00:00 [received] PHST- 2024/05/24 00:00 [revised] PHST- 2024/08/07 00:00 [accepted] PHST- 2024/09/05 00:50 [medline] PHST- 2024/09/05 00:50 [pubmed] PHST- 2024/09/04 23:52 [entrez] PHST- 2026/02/27 00:00 [pmc-release] AID - S1535-6108(24)00305-2 [pii] AID - 10.1016/j.ccell.2024.08.006 [doi] PST - aheadofprint SO - Cancer Cell. 2024 Aug 27:S1535-6108(24)00305-2. doi: 10.1016/j.ccell.2024.08.006. PMID- 34551970 OWN - NLM STAT- MEDLINE DCOM- 20220314 LR - 20250530 IS - 2159-8290 (Electronic) IS - 2159-8274 (Linking) VI - 12 IP - 2 DP - 2022 Feb TI - Repurposing Vandetanib plus Everolimus for the Treatment of ACVR1-Mutant Diffuse Intrinsic Pontine Glioma. PG - 416-431 LID - 10.1158/2159-8290.CD-20-1201 [doi] AB - Somatic mutations in ACVR1 are found in a quarter of children with diffuse intrinsic pontine glioma (DIPG), but there are no ACVR1 inhibitors licensed for the disease. Using an artificial intelligence-based platform to search for approved compounds for ACVR1-mutant DIPG, the combination of vandetanib and everolimus was identified as a possible therapeutic approach. Vandetanib, an inhibitor of VEGFR/RET/EGFR, was found to target ACVR1 (K (d) = 150 nmol/L) and reduce DIPG cell viability in vitro but has limited ability to cross the blood-brain barrier. In addition to mTOR, everolimus inhibited ABCG2 (BCRP) and ABCB1 (P-gp) transporters and was synergistic in DIPG cells when combined with vandetanib in vitro. This combination was well tolerated in vivo and significantly extended survival and reduced tumor burden in an orthotopic ACVR1-mutant patient-derived DIPG xenograft model. Four patients with ACVR1-mutant DIPG were treated with vandetanib plus an mTOR inhibitor, informing the dosing and toxicity profile of this combination for future clinical studies. SIGNIFICANCE: Twenty-five percent of patients with the incurable brainstem tumor DIPG harbor somatic activating mutations in ACVR1, but there are no approved drugs targeting the receptor. Using artificial intelligence, we identify and validate, both experimentally and clinically, the novel combination of vandetanib and everolimus in these children based on both signaling and pharmacokinetic synergies.This article is highlighted in the In This Issue feature, p. 275. CI - ©2021 The Authors; Published by the American Association for Cancer Research. FAU - Carvalho, Diana M AU - Carvalho DM AD - Division of Molecular Pathology, Institute of Cancer Research, London, United Kingdom. FAU - Richardson, Peter J AU - Richardson PJ AUID- ORCID: 0000-0001-7813-041X AD - BenevolentAI, London, United Kingdom. FAU - Olaciregui, Nagore AU - Olaciregui N AD - Laboratory of Molecular Oncology, Hospital Sant Joan de Déu, Barcelona, Spain. FAU - Stankunaite, Reda AU - Stankunaite R AUID- ORCID: 0000-0003-3977-7020 AD - Molecular Diagnostics, Royal Marsden Hospital NHS Trust, Sutton, United Kingdom. FAU - Lavarino, Cinzia AU - Lavarino C AUID- ORCID: 0000-0002-9630-3676 AD - Laboratory of Molecular Oncology, Hospital Sant Joan de Déu, Barcelona, Spain. FAU - Molinari, Valeria AU - Molinari V AD - Division of Molecular Pathology, Institute of Cancer Research, London, United Kingdom. FAU - Corley, Elizabeth A AU - Corley EA AUID- ORCID: 0000-0002-8660-9938 AD - Children & Young People's Unit, Royal Marsden Hospital NHS Trust, Sutton, United Kingdom. FAU - Smith, Daniel P AU - Smith DP AD - BenevolentAI, London, United Kingdom. FAU - Ruddle, Ruth AU - Ruddle R AUID- ORCID: 0000-0003-0025-8872 AD - Division of Cancer Therapeutics, Institute of Cancer Research, London, United Kingdom. FAU - Donovan, Adam AU - Donovan A AD - Division of Cancer Therapeutics, Institute of Cancer Research, London, United Kingdom. FAU - Pal, Akos AU - Pal A AD - Division of Cancer Therapeutics, Institute of Cancer Research, London, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - Division of Cancer Therapeutics, Institute of Cancer Research, London, United Kingdom. FAU - Temelso, Sara AU - Temelso S AUID- ORCID: 0000-0001-6017-8503 AD - Division of Molecular Pathology, Institute of Cancer Research, London, United Kingdom. FAU - Mackay, Alan AU - Mackay A AD - Division of Molecular Pathology, Institute of Cancer Research, London, United Kingdom. FAU - Overington, John P AU - Overington JP AUID- ORCID: 0000-0002-5859-1064 AD - Medicines Discovery Catapult, Alderley Edge, United Kingdom. FAU - Phelan, Anne AU - Phelan A AD - BenevolentAI, London, United Kingdom. FAU - Sheppard, David AU - Sheppard D AD - BenevolentAI, London, United Kingdom. FAU - Mackinnon, Andrew AU - Mackinnon A AD - Children & Young People's Unit, Royal Marsden Hospital NHS Trust, Sutton, United Kingdom. AD - Atkinson Morley Regional Neuroscience Centre, St George's Hospital NHS Trust, London, United Kingdom. FAU - Zebian, Bassel AU - Zebian B AD - Department of Neurosurgery, Kings College Hospital NHS Trust, London, United Kingdom. FAU - Al-Sarraj, Safa AU - Al-Sarraj S AD - Department of Clinical Neuropathology, Kings College Hospital NHS Trust, London, United Kingdom. FAU - Merve, Ashirwad AU - Merve A AUID- ORCID: 0000-0001-9715-1849 AD - Institute of Neurology, University College London Hospitals, London, United Kingdom. FAU - Pryce, Jeremy AU - Pryce J AD - South West London Pathology, St George's Hospital NHS Trust, London, United Kingdom. FAU - Grill, Jacques AU - Grill J AUID- ORCID: 0000-0003-4773-3001 AD - Department of Pediatric and Adolescent Oncology and INSERM Unit U891, Team "Genomics and Oncogenesis of Pediatric Brain Tumors," Gustave Roussy and University Paris-Saclay, Villejuif, France. FAU - Hubank, Michael AU - Hubank M AUID- ORCID: 0000-0002-2901-0742 AD - Molecular Diagnostics, Royal Marsden Hospital NHS Trust, Sutton, United Kingdom. FAU - Cruz, Ofelia AU - Cruz O AUID- ORCID: 0000-0001-7102-3283 AD - Paediatric Oncology, Neuro-Oncology Unit, Hospital Sant Joan de Déu, Barcelona, Spain. FAU - Morales La Madrid, Andres AU - Morales La Madrid A AD - Paediatric Oncology, Neuro-Oncology Unit, Hospital Sant Joan de Déu, Barcelona, Spain. FAU - Mueller, Sabine AU - Mueller S AD - University Children's Hospital, Zurich, Switzerland. AD - University of California, San Francisco, San Francisco, California. FAU - Carcaboso, Angel M AU - Carcaboso AM AUID- ORCID: 0000-0002-8485-426X AD - Laboratory of Molecular Oncology, Hospital Sant Joan de Déu, Barcelona, Spain. FAU - Carceller, Fernando AU - Carceller F AUID- ORCID: 0000-0003-3094-3758 AD - Children & Young People's Unit, Royal Marsden Hospital NHS Trust, Sutton, United Kingdom. chris.jones@icr.ac.uk fernando.carceller@icr.ac.uk. AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Jones, Chris AU - Jones C AD - Division of Molecular Pathology, Institute of Cancer Research, London, United Kingdom. chris.jones@icr.ac.uk fernando.carceller@icr.ac.uk. LA - eng GR - A23536/CRUK_/Cancer Research UK/United Kingdom GR - 13982/CRUK_/Cancer Research UK/United Kingdom GR - 22897/CRUK_/Cancer Research UK/United Kingdom GR - 23536/CRUK_/Cancer Research UK/United Kingdom GR - G0902001/MRC_/Medical Research Council/United Kingdom PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210922 PL - United States TA - Cancer Discov JT - Cancer discovery JID - 101561693 RN - 0 (Piperidines) RN - 0 (Quinazolines) RN - 9HW64Q8G6G (Everolimus) RN - EC 2.7.11.30 (ACVR1 protein, human) RN - EC 2.7.11.30 (Activin Receptors, Type I) RN - YO460OQ37K (vandetanib) SB - IM MH - Activin Receptors, Type I/*genetics MH - Animals MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*therapeutic use MH - Brain Stem Neoplasms/*drug therapy/mortality MH - Child MH - Child, Preschool MH - Drug Repositioning MH - Everolimus/administration & dosage/*therapeutic use MH - Female MH - Glioma/*drug therapy/mortality MH - Humans MH - Male MH - Mice MH - Mice, SCID MH - Piperidines/administration & dosage/*therapeutic use MH - Quinazolines/administration & dosage/*therapeutic use MH - Rats MH - Treatment Outcome PMC - PMC7612365 MID - EMS135882 EDAT- 2021/09/24 06:00 MHDA- 2022/03/15 06:00 CRDT- 2021/09/23 05:57 PHST- 2020/08/15 00:00 [received] PHST- 2021/05/17 00:00 [revised] PHST- 2021/09/10 00:00 [accepted] PHST- 2021/09/24 06:00 [pubmed] PHST- 2022/03/15 06:00 [medline] PHST- 2021/09/23 05:57 [entrez] AID - 2159-8290.CD-20-1201 [pii] AID - 10.1158/2159-8290.CD-20-1201 [doi] PST - ppublish SO - Cancer Discov. 2022 Feb;12(2):416-431. doi: 10.1158/2159-8290.CD-20-1201. Epub 2021 Sep 22. PMID- 32317163 OWN - NLM STAT- MEDLINE DCOM- 20211124 LR - 20211124 IS - 0013-7006 (Print) IS - 0013-7006 (Linking) VI - 46 IP - 6 DP - 2020 Dec TI - [Suicidal risk prevention in schizophrenia: Importance of family psychoeducation]. PG - 450-454 LID - S0013-7006(20)30039-7 [pii] LID - 10.1016/j.encep.2020.02.002 [doi] AB - OBJECTIVES: Although mortality by suicide in schizophrenia seems to have decreased in some countries over the last 30 years, it remains much higher than in the general population. Studies have shown this risk to impact around 5% of patients, corresponding to a risk almost 2.5 times higher than in the general population. Family psychoeducation in schizophrenia has been demonstrated to lead to symptom reductions and to an improvement of the quality of life, two factors that should contribute to decreasing the suicidal risk. Therefore, if families attend an efficient psychoeducation program, we can expect a decrease in the patient suicidal risk. Attending a family psychoeducation program at the beginning of the disease would also be associated with a stronger preventive effect on suicidal mortality. The objective of this study is to describe the suicide attempt rate of patients who suffer from schizophrenia before and one year after one of their relatives participated to the family psychoeducation program Profamille. METHOD: We performed a retrospective study on 1209 people who attended the Profamille (V3.2 version) Family Psychoeducation Program. This program has 2 modules: an initial training module of 14 weekly or fortnightly sessions, and a consolidation module of 4 sessions over 2 years. Sessions last 4 hours and follow a precise and structured course. Data were collected from 40 different centers in France, Belgium and Switzerland and were based on participants assessed at the beginning and one year after the first module. Self-assessment from the relatives participating in the program provided the measure of patients' suicide attempts. An assessment at T0 explored the attempts over the 12 months before the beginning of the program while the assessment at T1 analyzed those during the 12 months following the end of the Program. The Chi(2) test was used to compare the suicide attempt rates for each period, using a significance threshold of 0.05. Since the risk of suicide is greater in the first years of the illness, rates of attempts are also calculated according to the age of disorder. The analysis was carried out with the statistical software R. RESULTS: The number of participants reporting that their relative had attempted suicide in the previous 12 months decreased from 41 to 21. The annual attempts rate was evaluated at 6.4 % before the Profamille program and decreased to 2.4 % a year after the end of the program (P=0.0003). The reduction of the attempt rate was observed even for patients with schizophrenia for more than 10 years. CONCLUSION: This study shows the positive impact of Profamille on reducing the rate of suicide attempts in patients with schizophrenia. It has been shown that the risk is highest at the beginning of the disorder. Therefore, based on our results, it would seem appropriate to propose the Profamille program at an early stage. CI - Copyright © 2020. Published by Elsevier Masson SAS. FAU - Hodé, Y AU - Hodé Y AD - Institut de psychiatrie GDR 3557 Paris, 1, rue Cabanis, 75014 Paris, France. Electronic address: yann.hode@yahoo.fr. FAU - Dubreucq, J AU - Dubreucq J AD - C3r CH Alpes-Isère, 3, rue de la Gare, 38120 Saint-Égrève, France. FAU - Valladier, E AU - Valladier E AD - Service hospitalo-universitaire-S14, centre référent en remédiation cognitive et réhabilitation psychosociale (C3RP), centre hospitalier Sainte-Anne, 75014 Paris, France. FAU - Guillard Bouhet, N AU - Guillard Bouhet N AD - CREATIV, centre hospitalier H. Laborit, Poitiers, 370, avenue Jacques-Cœur, 86021 Poitiers, France. FAU - Lemestré, S AU - Lemestré S AD - ASBL Similes Wallonie, rue Lairesse, 15, 4020 Liège, Belgique. FAU - Attal, J AU - Attal J AD - Service universitaire de psychiatrie adulte, hôpital la Colombière, CHRU Montpellier, 191, avenue du Doyen Gaston-Giraud, 34295 Montpellier cedex 5, France. FAU - Canceil, O AU - Canceil O AD - Pôle Paris 12 - Secteur 75G10/11 hôpitaux de Saint-Maurice, 12-4, rue du Val d'Osne, 94410 Saint-Maurice, France. FAU - Biotteau, M AU - Biotteau M AD - Département de psychiatrie, CHU de Tours, 37044 Tours cedex 9, France. FAU - Laffond, P AU - Laffond P AD - Hôpital de Malévoz, route de Morgins, 10, 1870 Monthey, Suisse. FAU - Raynaud, A AU - Raynaud A AD - Filière ambulatoire, centre hospitalier Esquirol, 15, rue Doct Raymond-Marcland, 87000 Limoges, France. FAU - Chéreau-Boudet, I AU - Chéreau-Boudet I AD - Service de psychiatrie de l'adulte, CHU de Clermont-Ferrand, rue Montalembert, BP 69, 63003 Clermont-Ferrand, France. FAU - Montagne Larmurier, A AU - Montagne Larmurier A AD - Service de psychiatrie adulte, CHU de Caen, avenue de la Côte de Nacre CS 30001, 14033 Caen cedex 9, France. FAU - Giordana, J-Y AU - Giordana JY AD - CHS Sainte-Marie, 87, avenue Joseph-Raybaud, 06200 Nice, France. FAU - Saingery, B AU - Saingery B AD - EPSM des Ardennes CHS Bélair, 1, rue Pierre-Hallali, 08000 Charleville-Mézières, France. FAU - d'Amato, T AU - d'Amato T AD - SHU pôle est, centre hospitalier Le-Vinatier, BP 30039 - 95, boulevard Pinel, 69678 Bron, France. FAU - Willard, D AU - Willard D AD - C3R-P/CJAAD, service hospitalo-universitaire, GHU Paris psychiatrie et neurosciences, 1, rue Cabanis, 75014 Paris, France. LA - fre PT - Journal Article TT - Prévention du risque suicidaire dans la schizophrénie : importance de la psychoéducation des familles. DEP - 20200418 PL - France TA - Encephale JT - L'Encephale JID - 7505643 SB - IM MH - Humans MH - Quality of Life MH - Retrospective Studies MH - *Schizophrenia MH - Suicidal Ideation MH - Suicide, Attempted OTO - NOTNLM OT - Behavioral cognitive therapy OT - Family intervention OT - Intervention familiale OT - Prevention OT - Prévention OT - Psychoeducation OT - Psychoéducation OT - Schizophrenia OT - Schizophrénie OT - Suicide OT - Thérapie comportementale et cognitive EDAT- 2020/04/23 06:00 MHDA- 2021/11/25 06:00 CRDT- 2020/04/23 06:00 PHST- 2019/06/15 00:00 [received] PHST- 2020/02/03 00:00 [revised] PHST- 2020/02/07 00:00 [accepted] PHST- 2020/04/23 06:00 [pubmed] PHST- 2021/11/25 06:00 [medline] PHST- 2020/04/23 06:00 [entrez] AID - S0013-7006(20)30039-7 [pii] AID - 10.1016/j.encep.2020.02.002 [doi] PST - ppublish SO - Encephale. 2020 Dec;46(6):450-454. doi: 10.1016/j.encep.2020.02.002. Epub 2020 Apr 18. PMID- 31164419 OWN - NLM STAT- MEDLINE DCOM- 20200406 LR - 20250530 IS - 1091-6490 (Electronic) IS - 0027-8424 (Print) IS - 0027-8424 (Linking) VI - 116 IP - 25 DP - 2019 Jun 18 TI - Ancient Yersinia pestis genomes from across Western Europe reveal early diversification during the First Pandemic (541-750). PG - 12363-12372 LID - 10.1073/pnas.1820447116 [doi] AB - The first historically documented pandemic caused by Yersinia pestis began as the Justinianic Plague in 541 within the Roman Empire and continued as the so-called First Pandemic until 750. Although paleogenomic studies have previously identified the causative agent as Y. pestis, little is known about the bacterium's spread, diversity, and genetic history over the course of the pandemic. To elucidate the microevolution of the bacterium during this time period, we screened human remains from 21 sites in Austria, Britain, Germany, France, and Spain for Y. pestis DNA and reconstructed eight genomes. We present a methodological approach assessing single-nucleotide polymorphisms (SNPs) in ancient bacterial genomes, facilitating qualitative analyses of low coverage genomes from a metagenomic background. Phylogenetic analysis on the eight reconstructed genomes reveals the existence of previously undocumented Y. pestis diversity during the sixth to eighth centuries, and provides evidence for the presence of multiple distinct Y. pestis strains in Europe. We offer genetic evidence for the presence of the Justinianic Plague in the British Isles, previously only hypothesized from ambiguous documentary accounts, as well as the parallel occurrence of multiple derived strains in central and southern France, Spain, and southern Germany. Four of the reported strains form a polytomy similar to others seen across the Y. pestis phylogeny, associated with the Second and Third Pandemics. We identified a deletion of a 45-kb genomic region in the most recent First Pandemic strains affecting two virulence factors, intriguingly overlapping with a deletion found in 17th- to 18th-century genomes of the Second Pandemic. CI - Copyright © 2019 the Author(s). Published by PNAS. FAU - Keller, Marcel AU - Keller M AUID- ORCID: 0000-0001-9668-6817 AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany; marcel.keller@ut.ee harbeck@snsb.de herbig@shh.mpg.de krause@shh.mpg.de. AD - State Collection of Anthropology and Palaeoanatomy Munich, Staatliche Naturwissenschaftliche Sammlungen Bayerns, 80333 Munich, Germany. FAU - Spyrou, Maria A AU - Spyrou MA AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany. FAU - Scheib, Christiana L AU - Scheib CL AD - Department of Archaeology, University of Cambridge, Cambridge CB2 3ER, United Kingdom. AD - Institute of Genomics, University of Tartu, 51010 Tartu, Estonia. FAU - Neumann, Gunnar U AU - Neumann GU AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany. FAU - Kröpelin, Andreas AU - Kröpelin A AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany. AD - Friedrich Schiller University Jena, 07743 Jena, Germany. FAU - Haas-Gebhard, Brigitte AU - Haas-Gebhard B AD - Archaeological Collection of the Bavarian State, 80538 Munich, Germany. FAU - Päffgen, Bernd AU - Päffgen B AD - Institute for Pre- and Protohistoric Archaeology and Archaeology of the Roman Provinces, Ludwig Maximilian University Munich, 80799 Munich, Germany. FAU - Haberstroh, Jochen AU - Haberstroh J AD - Bavarian State Department of Monuments and Sites, 80539 Munich, Germany. FAU - Ribera I Lacomba, Albert AU - Ribera I Lacomba A AD - Department for Municipal Archaeology, Valencia City Council, 46014 Valencia, Spain. FAU - Raynaud, Claude AU - Raynaud C AD - CNRS, UMR5140, Archéologie des Sociétés Méditerranéennes, 34199 Montpellier, France. FAU - Cessford, Craig AU - Cessford C AD - Department of Archaeology, University of Cambridge, Cambridge CB2 3ER, United Kingdom. FAU - Durand, Raphaël AU - Durand R AD - Service d'Archéologie Préventive de l'Agglomération de Bourges Plus, 18023 Bourges Cedex, France. FAU - Stadler, Peter AU - Stadler P AD - Department of Pre- and Protohistory, University of Vienna, 1190 Vienna, Austria. FAU - Nägele, Kathrin AU - Nägele K AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany. FAU - Bates, Jessica S AU - Bates JS AD - Department of Archaeology, University of Cambridge, Cambridge CB2 3ER, United Kingdom. FAU - Trautmann, Bernd AU - Trautmann B AD - State Collection of Anthropology and Palaeoanatomy Munich, Staatliche Naturwissenschaftliche Sammlungen Bayerns, 80333 Munich, Germany. FAU - Inskip, Sarah A AU - Inskip SA AD - McDonald Institute for Archaeological Research, University of Cambridge, Cambridge CB2 3ER, United Kingdom. FAU - Peters, Joris AU - Peters J AD - State Collection of Anthropology and Palaeoanatomy Munich, Staatliche Naturwissenschaftliche Sammlungen Bayerns, 80333 Munich, Germany. AD - ArchaeoBioCenter, Ludwig Maximilian University Munich, 80539 Munich, Germany. AD - Department of Veterinary Sciences, Institute of Palaeoanatomy, Domestication Research and the History of Veterinary Medicine, Ludwig Maximilian University Munich, 80539 Munich, Germany. FAU - Robb, John E AU - Robb JE AD - Department of Archaeology, University of Cambridge, Cambridge CB2 3ER, United Kingdom. FAU - Kivisild, Toomas AU - Kivisild T AUID- ORCID: 0000-0002-6297-7808 AD - Department of Archaeology, University of Cambridge, Cambridge CB2 3ER, United Kingdom. AD - Department of Human Genetics, Katholieke Universiteit Leuven, 3000 Leuven, Belgium. FAU - Castex, Dominique AU - Castex D AD - UMR 5199, PACEA, CNRS Institute, 33615 Pessac Cedex, France. FAU - McCormick, Michael AU - McCormick M AD - Initiative for the Science of the Human Past, Department of History, Harvard University, Cambridge, MA 02138. AD - Max Planck-Harvard Research Center for the Archaeoscience of the Ancient Mediterranean, 07745 Jena, Germany. FAU - Bos, Kirsten I AU - Bos KI AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany. FAU - Harbeck, Michaela AU - Harbeck M AD - State Collection of Anthropology and Palaeoanatomy Munich, Staatliche Naturwissenschaftliche Sammlungen Bayerns, 80333 Munich, Germany; marcel.keller@ut.ee harbeck@snsb.de herbig@shh.mpg.de krause@shh.mpg.de. FAU - Herbig, Alexander AU - Herbig A AUID- ORCID: 0000-0003-1176-1166 AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany; marcel.keller@ut.ee harbeck@snsb.de herbig@shh.mpg.de krause@shh.mpg.de. FAU - Krause, Johannes AU - Krause J AUID- ORCID: 0000-0001-9144-3920 AD - Department of Archaeogenetics, Max Planck Institute for the Science of Human History, 07745 Jena, Germany; marcel.keller@ut.ee harbeck@snsb.de herbig@shh.mpg.de krause@shh.mpg.de. AD - Max Planck-Harvard Research Center for the Archaeoscience of the Ancient Mediterranean, 07745 Jena, Germany. LA - eng GR - 200368/Z/15/Z/WT_/Wellcome Trust/United Kingdom GR - 2000368/Z/15/Z/WT_/Wellcome Trust/United Kingdom PT - Historical Article PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190604 PL - United States TA - Proc Natl Acad Sci U S A JT - Proceedings of the National Academy of Sciences of the United States of America JID - 7505876 SB - IM MH - Disease Outbreaks/*history MH - Europe/epidemiology MH - *Genome, Bacterial MH - History, Medieval MH - Humans MH - Plague/epidemiology/history/*microbiology MH - Yersinia pestis/*genetics/pathogenicity PMC - PMC6589673 OTO - NOTNLM OT - Anglo-Saxons OT - Justinianic Plague OT - Merovingians OT - ancient DNA OT - bacterial evolution COIS- The authors declare no conflict of interest. EDAT- 2019/06/06 06:00 MHDA- 2020/04/09 06:00 PMCR- 2019/06/04 CRDT- 2019/06/06 06:00 PHST- 2019/06/06 06:00 [pubmed] PHST- 2020/04/09 06:00 [medline] PHST- 2019/06/06 06:00 [entrez] PHST- 2019/06/04 00:00 [pmc-release] AID - 1820447116 [pii] AID - 201820447 [pii] AID - 10.1073/pnas.1820447116 [doi] PST - ppublish SO - Proc Natl Acad Sci U S A. 2019 Jun 18;116(25):12363-12372. doi: 10.1073/pnas.1820447116. Epub 2019 Jun 4. PMID- 25680029 OWN - NLM STAT- MEDLINE DCOM- 20150507 LR - 20250529 IS - 1520-4804 (Electronic) IS - 0022-2623 (Print) IS - 0022-2623 (Linking) VI - 58 IP - 4 DP - 2015 Feb 26 TI - Discovery of potent, orally bioavailable, small-molecule inhibitors of WNT signaling from a cell-based pathway screen. PG - 1717-35 LID - 10.1021/jm501436m [doi] AB - WNT signaling is frequently deregulated in malignancy, particularly in colon cancer, and plays a key role in the generation and maintenance of cancer stem cells. We report the discovery and optimization of a 3,4,5-trisubstituted pyridine 9 using a high-throughput cell-based reporter assay of WNT pathway activity. We demonstrate a twisted conformation about the pyridine-piperidine bond of 9 by small-molecule X-ray crystallography. Medicinal chemistry optimization to maintain this twisted conformation, cognisant of physicochemical properties likely to maintain good cell permeability, led to 74 (CCT251545), a potent small-molecule inhibitor of WNT signaling with good oral pharmacokinetics. We demonstrate inhibition of WNT pathway activity in a solid human tumor xenograft model with evidence for tumor growth inhibition following oral dosing. This work provides a successful example of hypothesis-driven medicinal chemistry optimization from a singleton hit against a cell-based pathway assay without knowledge of the biochemical target. FAU - Mallinger, Aurélie AU - Mallinger A AD - Cancer Research UK Cancer Therapeutics Unit at The Institute of Cancer Research, London SW7 3RP, U.K. FAU - Crumpler, Simon AU - Crumpler S FAU - Pichowicz, Mark AU - Pichowicz M FAU - Waalboer, Dennis AU - Waalboer D FAU - Stubbs, Mark AU - Stubbs M FAU - Adeniji-Popoola, Olajumoke AU - Adeniji-Popoola O FAU - Wood, Bozena AU - Wood B FAU - Smith, Elizabeth AU - Smith E FAU - Thai, Ching AU - Thai C FAU - Henley, Alan T AU - Henley AT FAU - Georgi, Katrin AU - Georgi K FAU - Court, William AU - Court W FAU - Hobbs, Steve AU - Hobbs S FAU - Box, Gary AU - Box G FAU - Ortiz-Ruiz, Maria-Jesus AU - Ortiz-Ruiz MJ FAU - Valenti, Melanie AU - Valenti M FAU - De Haven Brandon, Alexis AU - De Haven Brandon A FAU - TePoele, Robert AU - TePoele R FAU - Leuthner, Birgitta AU - Leuthner B FAU - Workman, Paul AU - Workman P FAU - Aherne, Wynne AU - Aherne W FAU - Poeschke, Oliver AU - Poeschke O FAU - Dale, Trevor AU - Dale T FAU - Wienke, Dirk AU - Wienke D FAU - Esdar, Christina AU - Esdar C FAU - Rohdich, Felix AU - Rohdich F FAU - Raynaud, Florence AU - Raynaud F FAU - Clarke, Paul A AU - Clarke PA FAU - Eccles, Suzanne A AU - Eccles SA FAU - Stieber, Frank AU - Stieber F FAU - Schiemann, Kai AU - Schiemann K FAU - Blagg, Julian AU - Blagg J LA - eng GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - 15937/CRUK_/Cancer Research UK/United Kingdom GR - BB/D00117X/1/BB_/Biotechnology and Biological Sciences Research Council/United Kingdom GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150213 PL - United States TA - J Med Chem JT - Journal of medicinal chemistry JID - 9716531 RN - 0 (Antineoplastic Agents) RN - 0 (CCT251545) RN - 0 (Pyridines) RN - 0 (Small Molecule Libraries) RN - 0 (Spiro Compounds) RN - EC 1.13.12.- (Luciferases) SB - IM MH - Administration, Oral MH - Animals MH - Antineoplastic Agents/administration & dosage/chemistry/*pharmacology MH - Biological Assay/methods MH - Biological Availability MH - Cell Line, Tumor MH - Cell Proliferation/drug effects MH - Colorectal Neoplasms/*drug therapy/metabolism/pathology MH - Crystallography, X-Ray MH - Disease Models, Animal MH - Dose-Response Relationship, Drug MH - Drug Evaluation, Preclinical/*methods MH - Humans MH - Luciferases/*antagonists & inhibitors/metabolism MH - Mice MH - Models, Molecular MH - Molecular Structure MH - Pyridines/administration & dosage/chemistry/*pharmacology MH - Small Molecule Libraries/administration & dosage/chemistry/*pharmacology MH - Spiro Compounds/administration & dosage/chemistry/*pharmacology MH - Structure-Activity Relationship MH - Wnt Signaling Pathway/*drug effects MH - Xenograft Model Antitumor Assays PMC - PMC4767141 COIS- The authors declare the following competing financial interest(s): A.M., S.C., M.P., D.W., M.S., O.A-P., E.S., C.T., A.T.H., W.C., S.H., G.B., M.-J.O.R., M.V., A.D.H.B., R.T., P.W., W.A., F.R., P.A.C., S.A.E., and J.B. are current or former employees of The Institute of Cancer Research, which has a commercial interest in the development of WNT pathway inhibitors. K.G., O.P., D.W., C.E., F.R., F.S., and K.S. are current employees of Merck Serono. EDAT- 2015/02/14 06:00 MHDA- 2015/05/08 06:00 PMCR- 2016/02/25 CRDT- 2015/02/14 06:00 PHST- 2015/02/14 06:00 [entrez] PHST- 2015/02/14 06:00 [pubmed] PHST- 2015/05/08 06:00 [medline] PHST- 2016/02/25 00:00 [pmc-release] AID - 10.1021/jm501436m [doi] PST - ppublish SO - J Med Chem. 2015 Feb 26;58(4):1717-35. doi: 10.1021/jm501436m. Epub 2015 Feb 13. PMID- 34538573 OWN - NLM STAT- MEDLINE DCOM- 20211101 LR - 20240404 IS - 1532-1770 (Electronic) IS - 1521-6942 (Print) IS - 1521-6942 (Linking) VI - 35 IP - 3 DP - 2021 Sep TI - A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc. PG - 101707 LID - S1521-6942(21)00049-8 [pii] LID - 10.1016/j.berh.2021.101707 [doi] AB - Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications. CI - Copyright © 2021 Elsevier Ltd. All rights reserved. FAU - Saketkoo, Lesley Ann AU - Saketkoo LA AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA. Electronic address: lsaketk@tulane.edu. FAU - Frech, Tracy AU - Frech T AD - Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Varjú, Cecília AU - Varjú C AD - Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary. FAU - Domsic, Robyn AU - Domsic R AD - University of Pittsburgh, Pittsburgh, PA, USA. FAU - Farrell, Jessica AU - Farrell J AD - Albany College of Pharmacy and Health Sciences, Albany, NY, USA; Steffens Scleroderma Foundation, Albany, NY, USA. FAU - Gordon, Jessica K AU - Gordon JK AD - Department of Rheumatology at Hospital for Special Surgery, New York, NY, USA. FAU - Mihai, Carina AU - Mihai C AD - Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland; Department of Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. FAU - Sandorfi, Nora AU - Sandorfi N AD - Perelman School of Medicine, Philadelphia, PA, USA. FAU - Shapiro, Lee AU - Shapiro L AD - Steffens Scleroderma Foundation, Albany, NY, USA; Division of Rheumatology, Albany Medical Center, Albany, NY, USA. FAU - Poole, Janet AU - Poole J AD - Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, USA. FAU - Volkmann, Elizabeth R AU - Volkmann ER AD - University of California, David Geffen School of Medicine, UCLA Scleroderma Program and UCLA CTD-ILD Program, Division of Rheumatology, Department of Medicine, Los Angeles, CA, USA. FAU - Lammi, Monika AU - Lammi M AD - Ochsner Medical Center, New Orleans, LA, USA. FAU - McAnally, Kendra AU - McAnally K AD - Norton Thoracic Institute, St. Joseph's Hospital and Medical Centre, Phoenix, AZ, USA. FAU - Alexanderson, Helene AU - Alexanderson H AD - Function Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Pettersson, Henrik AU - Pettersson H AD - Function Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden. FAU - Hant, Faye AU - Hant F AD - Division of Rheumatology, Medical University of South Caroline, SC, USA. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Shah, Ami A AU - Shah AA AD - Department of Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Smith, Vanessa AU - Smith V AD - Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. FAU - Hsu, Vivien AU - Hsu V AD - Rutgers- RWJ Scleroderma Program, New Brunswick, NJ, USA. FAU - Kowal-Bielecka, Otylia AU - Kowal-Bielecka O AD - Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. FAU - Assassi, Shervin AU - Assassi S AD - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, USA. FAU - Cutolo, Maurizio AU - Cutolo M AD - Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Polyclinic San Martino Hospital, Genova, Italy. FAU - Kayser, Cristiane AU - Kayser C AD - Escola Paulista de Medicina, Federal University of São Paulo (UNIFESP) São Paulo, SP, Brazil. FAU - Shanmugam, Victoria K AU - Shanmugam VK AD - Department of Rheumatology, George Washington University, School of Medicine and Health Sciences, Washington, DC, USA. FAU - Vonk, Madelon C AU - Vonk MC AD - Department of the rheumatic diseases, Radboud University Medical Center, Nijmegen, the Netherlands. FAU - Fligelstone, Kim AU - Fligelstone K AD - Patient Research Partner, Scleroderma & Raynaud Society UK (SRUK), London, UK; Royal Free Hospital, London, UK. FAU - Baldwin, Nancy AU - Baldwin N AD - Patient Research Partner, Scleroderma Foundation, Chicago, IL, USA. FAU - Connolly, Kerri AU - Connolly K AD - Scleroderma Foundation, Danvers, MA, USA. FAU - Ronnow, Anneliese AU - Ronnow A AD - Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK. FAU - Toth, Beata AU - Toth B AD - Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK. FAU - Suave, Maureen AU - Suave M AD - Scleroderma Canada, Canada. FAU - Farrington, Sue AU - Farrington S AD - Patient Research Partner, Scleroderma & Raynaud Society UK (SRUK), London, UK; Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK. FAU - Bernstein, Elana J AU - Bernstein EJ AD - Columbia University/New York-Presbyterian Scleroderma Program, Division of Rheumatology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA. FAU - Crofford, Leslie J AU - Crofford LJ AD - Vanderbilt University Medical Center, Nashville, TN, USA. FAU - Czirják, László AU - Czirják L AD - Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary. FAU - Jensen, Kelly AU - Jensen K AD - Tulane University School of Medicine, New Orleans, USA; Oregon Health and Science University, Portland, OR, USA. FAU - Hinchclif, Monique AU - Hinchclif M AD - Yale School of Medicine, Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, USA. FAU - Hudson, Marie AU - Hudson M AD - Division of heumatology and Department of Medicine, Jewish General Hospital and McGill University, Montreal, QC, Canada. FAU - Lammi, Matthew R AU - Lammi MR AD - New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA. FAU - Mansour, Jennifer AU - Mansour J AD - Tulane University School of Medicine, New Orleans, USA. FAU - Morgan, Nadia D AU - Morgan ND AD - Department of Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Mendoza, Fabian AU - Mendoza F AD - Rheumatology Division, Department of Medicine, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Nikpour, Mandana AU - Nikpour M AD - Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Pauling, John AU - Pauling J AD - University of Melbourne, Melbourne at St. Vincent's Hospital Melbourne, Victoria, Australia. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Royal National Hospital for Rheumatic Diseases, Bath, UK; University of Lübeck, University Clinic of Schleswig-Holstein, Dept Rheumatology and Clinical Immunology, Lübeck, Germany. FAU - Russell, Anne-Marie AU - Russell AM AD - University of Exeter, College of Medicine and Health, Exeter, UK. FAU - Scholand, Mary Beth AU - Scholand MB AD - University of Utah, Division of Pulmonary Medicine, Pulmonary Fibrosis Center, Salt Lake City, UT, USA. FAU - Seigart, Elise AU - Seigart E AD - Department of Rheumatology and Clinical Immunology Charité - Universitätsmedizin Berlin and Berlin Institute of Health, Berlin, Germany. FAU - Rodriguez-Reyna, Tatiana Sofia AU - Rodriguez-Reyna TS AD - Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. FAU - Hummers, Laura AU - Hummers L AD - Department of Medicine, Johns Hopkins University, Baltimore, MD, USA. FAU - Walker, Ulrich AU - Walker U AD - Dept. of Rheumatology, Basel University Hospital, Basel, Switzerland. FAU - Steen, Virginia AU - Steen V AD - Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA. LA - eng GR - K23 HL150237/HL/NHLBI NIH HHS/United States GR - R01 AR073270/AR/NIAMS NIH HHS/United States GR - R01 AR050840/AR/NIAMS NIH HHS/United States GR - T32 HL144470/HL/NHLBI NIH HHS/United States GR - L30 HL129466/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20210915 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 SB - IM MH - Humans MH - *Hypertension, Pulmonary/diagnosis/etiology/prevention & control MH - Lung MH - *Lung Diseases, Interstitial MH - Patient Care MH - *Scleroderma, Systemic/complications/diagnosis/therapy PMC - PMC8670736 MID - NIHMS1742238 OTO - NOTNLM OT - Disability OT - Interstitial lung disease OT - Pulmonary fibrosis OT - Pulmonary hypertension OT - Quality of life OT - Renal crisis OT - Scleroderma OT - Symptom burden OT - Systemic sclerosis COIS- Declaration of competing interest None of the authors have conflicts of interest to report that are related to the reported content of this paper. EDAT- 2021/09/21 06:00 MHDA- 2021/11/03 06:00 PMCR- 2022/09/15 CRDT- 2021/09/20 05:41 PHST- 2021/09/21 06:00 [pubmed] PHST- 2021/11/03 06:00 [medline] PHST- 2021/09/20 05:41 [entrez] PHST- 2022/09/15 00:00 [pmc-release] AID - S1521-6942(21)00049-8 [pii] AID - 10.1016/j.berh.2021.101707 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101707. doi: 10.1016/j.berh.2021.101707. Epub 2021 Sep 15. PMID- 35307972 OWN - NLM STAT- MEDLINE DCOM- 20220725 LR - 20251112 IS - 2045-7634 (Electronic) IS - 2045-7634 (Linking) VI - 11 IP - 14 DP - 2022 Jul TI - A WIN Consortium phase I study exploring avelumab, palbociclib, and axitinib in advanced non-small cell lung cancer. PG - 2790-2800 LID - 10.1002/cam4.4635 [doi] AB - BACKGROUND: The Worldwide Innovative Network (WIN) Consortium has developed the Simplified Interventional Mapping System (SIMS) to better define the cancer molecular milieu based on genomics/transcriptomics from tumor and analogous normal tissue biopsies. SPRING is the first trial to assess a SIMS-based tri-therapy regimen in advanced non-small cell lung cancer (NSCLC). METHODS: Patients with advanced NSCLC (no EGFR, ALK, or ROS1 alterations; PD-L1 unrestricted; ≤2 prior therapy lines) received avelumab, axitinib, and palbociclib (3 + 3 dose escalation design). RESULTS: Fifteen patients were treated (five centers, four countries): six at each of dose levels 1 (DL1) and DL2; three at DL3. The most common ≥Grade 3 adverse events were neutropenia, hypertension, and fatigue. The recommended Phase II dose (RP2D) was DL1: avelumab 10 mg/kg IV q2weeks, axitinib 3 mg po bid, and palbociclib 75 mg po daily (7 days off/21 days on). Four patients (27%) achieved a partial response (PR) (progression-free survival [PFS]: 14, 24, 25 and 144+ weeks), including two after progression on pembrolizumab. Four patients attained stable disease (SD) that lasted ≥24 weeks: 24, 27, 29, and 64 weeks. At DL1 (RP2D), four of six patients (66%) achieved stable disease (SD) ≥6 months/PR (2 each). Responders included patients with no detectable PD-L1 expression and low tumor mutational burden. CONCLUSIONS: Overall, eight of 15 patients (53%) achieved clinical benefit (SD ≥ 24 weeks/PR) on the avelumab, axitinib, and palbociclib combination. This triplet showed antitumor activity in NSCLC, including in tumors post-pembrolizumab progression, and was active at the RP2D, which was well tolerated. NCT03386929 clinicaltrial.gov. CI - © 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd. FAU - Solomon, Benjamin AU - Solomon B AUID- ORCID: 0000-0002-6014-1775 AD - Avera Cancer Institute, Sioux Falls, South Dakota, USA. FAU - Callejo, Ana AU - Callejo A AD - Vall d'Hebron Hospital Campus and Institute of Oncology (VHIO), Barcelona, Spain. FAU - Bar, Jair AU - Bar J AD - Chaim Sheba Medical Center, Tel Hashomer, Israel. FAU - Berchem, Guy AU - Berchem G AD - Centre Hospitalier de Luxembourg, Luxembourg Institute of Health, Luxembourg City, Luxemburg. FAU - Bazhenova, Lyudmila AU - Bazhenova L AD - University of California San Diego, Moores Cancer Center, San Diego, California, USA. FAU - Saintigny, Pierre AU - Saintigny P AD - Centre Léon Bérard, Cancer Research Center of Lyon, University of Lyon, Lyon, France. FAU - Wunder, Fanny AU - Wunder F AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Raynaud, Jacques AU - Raynaud J AD - ARC Foundation for cancer research, Villejuif, France. FAU - Girard, Nicolas AU - Girard N AD - Institut Curie, Paris, France. FAU - Lee, J Jack AU - Lee JJ AD - The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Sulaiman, Raed AU - Sulaiman R AD - Avera Cancer Institute, Sioux Falls, South Dakota, USA. FAU - Prouse, Bruce AU - Prouse B AD - Avera Cancer Institute, Sioux Falls, South Dakota, USA. FAU - Bresson, Catherine AU - Bresson C AUID- ORCID: 0000-0002-7523-849X AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Ventura, Hila AU - Ventura H AD - Ben-Gurion University of the Negev, Be'er Sheva, Israel. FAU - Magidi, Shai AU - Magidi S AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Rubin, Eitan AU - Rubin E AD - Ben-Gurion University of the Negev, Be'er Sheva, Israel. FAU - Young, Brandon AU - Young B AD - Bowden Laboratory, Murrieta, California, USA. FAU - Onn, Amir AU - Onn A AD - Chaim Sheba Medical Center, Tel Hashomer, Israel. FAU - Leyland-Jones, Brian AU - Leyland-Jones B AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Schilsky, Richard L AU - Schilsky RL AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Lazar, Vladimir AU - Lazar V AD - Worldwide Innovative Network (WIN) Association - WIN Consortium, Villejuif, France. FAU - Felip, Enriqueta AU - Felip E AD - Vall d'Hebron Hospital Campus and Institute of Oncology (VHIO), Barcelona, Spain. FAU - Kurzrock, Razelle AU - Kurzrock R AUID- ORCID: 0000-0003-4110-1214 AD - University of California San Diego, Moores Cancer Center, San Diego, California, USA. LA - eng SI - ClinicalTrials.gov/NCT03386929 GR - P30 CA023100/CA/NCI NIH HHS/United States PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20220320 PL - United States TA - Cancer Med JT - Cancer medicine JID - 101595310 RN - 0 (Antibodies, Monoclonal) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (B7-H1 Antigen) RN - 0 (Piperazines) RN - 0 (Proto-Oncogene Proteins) RN - 0 (Pyridines) RN - C9LVQ0YUXG (Axitinib) RN - EC 2.7.10.1 (Protein-Tyrosine Kinases) RN - G9ZF61LE7G (palbociclib) RN - KXG2PJ551I (avelumab) SB - IM MH - Antibodies, Monoclonal MH - Antibodies, Monoclonal, Humanized MH - *Antineoplastic Combined Chemotherapy Protocols/adverse effects MH - Axitinib/therapeutic use MH - B7-H1 Antigen/metabolism MH - *Carcinoma, Non-Small-Cell Lung/drug therapy/genetics MH - Humans MH - *Lung Neoplasms/drug therapy/genetics MH - Piperazines MH - Protein-Tyrosine Kinases MH - Proto-Oncogene Proteins MH - Pyridines PMC - PMC9302335 OTO - NOTNLM OT - NSCLC OT - VEGFR OT - CDK4/6 OT - anti-PD-L1 OT - genomics OT - phase I OT - transcriptomics COIS- Dr. Benjamin Solomon discloses advisory board participation with AstraZeneca and Bayer. Dr. Ana Callejo receives advisory role and/or travel compensation from: Bristol‐Myers Squibb, F. Hoffman‐LaRoche, Pfizer, Boehringer Ingelheim, MSD Oncology, Kyowa Kirin, Celgene, Leo Pharma, Medscape, Kern Pharma. Dr. Jair Bar receives advisory board fee from Abbvie, AstraZeneca, Bayer, Bristol‐Myers Squibb, Boehringer Ingelheim, Causalis, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Takeda; Research support (for the institution) from Abbvie, AstraZeneca, ImmuneAI, Merck Sharp & Dohme, Novartis, OncoHost, Roche, Takeda. Dr. Lyudmila Bazhenova has stock and other ownership interests in Epic Sciences; consulting or advisory role in Neuvogen, Janssen, Daichi Sankyo, Boehringer Ingelheim, Merck, Regeneron, Bristol‐Myers Squibb, Novartis; and receives research funding from BeyondSpring. Dr. Pierre Saintigny receives research grants from the following companies: AstraZeneca, Roche, Bristol‐Myers‐Squibb, Healthcare company Hitachi, Ose Immunotherapeutics, HTG Molecular Diagnostcs, Illumina, Cellenion, Vortex Biosceinces and Inivata. Dr. Vladimir Lazar, Catherine Bresson and Fanny Wunder are full time employees of Worldwide Innovative Network (WIN) Association—WIN Consortium. Worldwide Innovative Network (WIN) Association—WIN Consortium is the owner of the patent family entitled “Method for Selecting Personalized Tri‐Therapy for Cancer Treatment.” The inventor is Dr. Vladimir Lazar. Dr. J. Jack Lee receives honorarium from AstraZeneca for participating the AAZPIRE Education Program. Shai Magidi receives consultancy from Worldwide Innovative Network (WIN) Association—WIN Consortium. Dr. Amir Onn receives consulting fees from Roche Israel, MSD Israel, Boehringer Ingelheim and AstraZeneca. Dr. Brian Leyland Jones serves on the Board/ is an officer of NFCR, receives speaker's bureau fees from Puma, Genentech, Exelixis, Bayer. Dr. Richard L. Schilsky receives research grants in support of a clinical trial that he directs for the American Society of Clinical Oncology from the following companies: AstraZeneca, Bayer, Boehringer Ingelheim, Bristol‐Myers Squibb, Genentech, Lilly, Merck, Pfizer, Seattle Genetics. Dr. Schilsky serves as a consultant to: Brylogyx, Cellworks Group, Clarify Precision Oncology, EQRx, Scandion Oncology. Dr. Enriqueta Felip receives advisory board and/or speaker's bureau fee from Amgen, AstraZeneca, Bayer, Beigene, Boehringer Ingelheim, Bristol‐Myers Squibb, Eli Lilly, F. Hoffman‐LaRoche, Glaxi Smith Kline, Janssen, Medical Trends, Medscape, Merck Sharp & Dohme, Merck Serono, Peptomyc, Peervoice, Pfizer, Puma, Regeneron, Sanofi, Springer, Syneos Health, Takeda, Touch Medical; on the board of Grifols, Independent member. Receives research funding from Fundacion Merck Salud, Grant for Oncology Innovation (GOI) EMD Serono. Dr. Razelle Kurzrock receives research funding from Genentech, Merck Serono, Pfizer, Boehringer Ingelheim, TopAlliance, Takeda, Incyte, Debiopharm, Medimmune, Sequenom, Foundation Medicine, Konica Minolta, Grifols, Omniseq, and Guardant, as well as consultant and/or speaker fees and/or advisory board for X‐Biotech, Neomed, Pfizer, Actuate Therapeutics, Roche, Turning Point Therapeutics, TD2/Volastra, Bicara Therapeutics, Inc., has an equity interest in IDbyDNA and CureMatch Inc, serves on the Board of CureMatch and CureMetrix, and is a co‐founder of CureMatch. All other authors declare no potential conflict of interest. EDAT- 2022/03/22 06:00 MHDA- 2022/07/26 06:00 PMCR- 2022/03/20 CRDT- 2022/03/21 05:47 PHST- 2022/01/19 00:00 [revised] PHST- 2021/09/29 00:00 [received] PHST- 2022/01/25 00:00 [accepted] PHST- 2022/03/22 06:00 [pubmed] PHST- 2022/07/26 06:00 [medline] PHST- 2022/03/21 05:47 [entrez] PHST- 2022/03/20 00:00 [pmc-release] AID - CAM44635 [pii] AID - 10.1002/cam4.4635 [doi] PST - ppublish SO - Cancer Med. 2022 Jul;11(14):2790-2800. doi: 10.1002/cam4.4635. Epub 2022 Mar 20. PMID- 39467037 OWN - NLM STAT- MEDLINE DCOM- 20250325 LR - 20260518 IS - 2326-5205 (Electronic) IS - 2326-5191 (Print) IS - 2326-5191 (Linking) VI - 77 IP - 4 DP - 2025 Apr TI - Clinical Characteristics of Anti-Synthetase Syndrome: Analysis From the Classification Criteria for Anti-Synthetase Syndrome Project. PG - 477-489 LID - 10.1002/art.43038 [doi] AB - OBJECTIVE: Anti-synthetase syndrome (ASSD) is a rare systemic autoimmune rheumatic disease (SARD) with significant heterogeneity and no shared classification criteria. We aimed to identify clinical and serological features associated with ASSD that may be suitable for inclusion in the data-driven classification criteria for ASSD. METHODS: We used a large, international, multicenter "Classification Criteria for Anti-synthetase Syndrome" (CLASS) project database, which includes both patients with ASSD and controls with mimicking conditions, namely, SARDs and/or interstitial lung disease (ILD). The local diagnoses of ASSD and controls were confirmed by project team members. We employed univariable logistic regression and multivariable Ridge regression to evaluate clinical and serological features associated with an ASSD diagnosis in a randomly selected subset of the cohort. RESULTS: Our analysis included 948 patients with ASSD and 1,077 controls. Joint, muscle, lung, skin, and cardiac involvement were more prevalent in patients with ASSD than in controls. Specific variables associated with ASSD included arthritis, diffuse myalgia, muscle weakness, muscle enzyme elevation, ILD, mechanic's hands, secondary pulmonary hypertension due to ILD, Raynaud phenomenon, and unexplained fever. In terms of serological variables, Jo-1 and non-Jo-1 anti-synthetase autoantibodies, antinuclear antibodies with cytoplasmic pattern, and anti-Ro52 autoantibodies were associated with ASSD. In contrast, isolated arthralgia, dysphagia, electromyography/magnetic resonance imaging/muscle biopsy findings suggestive of myopathy, inflammatory rashes, myocarditis, and pulmonary arterial hypertension did not differentiate between patients with ASSD and controls or were inversely associated with ASSD. CONCLUSION: We identified key clinical and serological variables associated with ASSD, which will help clinicians and offer insights into the development of data-driven classification criteria for ASSD. CI - © 2024 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA. FAU - Faghihi-Kashani, Sara AU - Faghihi-Kashani S AUID- ORCID: 0000-0001-5948-0297 AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Yoshida, Akira AU - Yoshida A AUID- ORCID: 0000-0003-3590-1637 AD - Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - Bozan, Francisca AU - Bozan F AUID- ORCID: 0009-0004-2711-0434 AD - Hospital Clínico Universidad de Chile, Santiago, Chile. FAU - Zanframundo, Giovanni AU - Zanframundo G AUID- ORCID: 0000-0001-5042-1282 AD - Università di Pavia and Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy. FAU - Rozza, Davide AU - Rozza D AUID- ORCID: 0000-0003-2648-742X AD - Italian Society of Rheumatology, Milan, Italy. FAU - Loganathan, Aravinthan AU - Loganathan A AUID- ORCID: 0000-0003-3576-7204 AD - Royal National Hospital for Rheumatic Diseases and University of Bath, Bath, UK, and Arthritis Australia, Broadway, Glebe, New South Wales, Australia. FAU - Dourado, Eduardo AU - Dourado E AUID- ORCID: 0000-0003-2186-2833 AD - Centro Hospitalar do Baixo Vouga and Egas Moniz Health Alliance, Aveiro, and Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal. FAU - Sambataro, Gianluca AU - Sambataro G AUID- ORCID: 0000-0001-9933-1202 AD - Policlinico G. Rodolico-San Marco, University of Catania, Catania, Italy. FAU - Bauer-Ventura, Iazsmin AU - Bauer-Ventura I AUID- ORCID: 0000-0003-1306-4241 AD - University of Chicago, Chicago, Illinois. FAU - Bae, Sangmee Sharon AU - Bae SS AUID- ORCID: 0000-0002-7216-7219 AD - David Geffen School of Medicine, University of California, Los Angeles, California. FAU - Lim, Darosa AU - Lim D AUID- ORCID: 0000-0003-2707-8134 AD - Perelman School of Medicine & Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center, Philadelphia, Pennsylvania. FAU - Rivero-Gallegos, Daphne AU - Rivero-Gallegos D AUID- ORCID: 0009-0003-9918-3459 AD - Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico. FAU - Yamano, Yasuhiko AU - Yamano Y AUID- ORCID: 0000-0002-7779-9215 AD - Tosei General Hospital, Seto, Japan. FAU - Selva-O'Callaghan, Albert AU - Selva-O'Callaghan A AUID- ORCID: 0000-0003-2823-9761 AD - Vall d'Hebron University Hospital, Universitat Autònoma de Barcelona, Barcelona, Spain. FAU - Mammen, Andrew L AU - Mammen AL AUID- ORCID: 0000-0003-3732-3252 AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, and Johns Hopkins University School of Medicine, Baltimore, Maryland. FAU - Scirè, Carlo A AU - Scirè CA AUID- ORCID: 0000-0001-7451-0271 AD - University of Milano Bicocca, Milan, and Istituto di Ricovero e Cura a Carattere Scientifico San Gerardo dei Tintori Foundation, Monza, Italy. FAU - Montecucco, Carlomaurizio AU - Montecucco C AUID- ORCID: 0000-0001-8263-3925 AD - Università di Pavia and Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy. FAU - Oddis, Chester V AU - Oddis CV AUID- ORCID: 0000-0002-9529-3111 AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Fiorentino, David AU - Fiorentino D AUID- ORCID: 0000-0001-7951-3674 AD - Stanford University School of Medicine, Redwood City, California. FAU - Bonella, Francesco AU - Bonella F AUID- ORCID: 0000-0001-7579-9767 AD - Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany. FAU - Miller, Frederick W AU - Miller FW AUID- ORCID: 0000-0003-2831-9593 AD - National Institute of Environmental Health Sciences, NIH, Durham, North Carolina. FAU - Lundberg, Ingrid E AU - Lundberg IE AUID- ORCID: 0000-0002-6068-9212 AD - Karolinska Institutet, Stockholm, Sweden. FAU - Schmidt, Jens AU - Schmidt J AUID- ORCID: 0000-0002-5589-2371 AD - University Medical Center Göttingen, Göttingen, Germany, and Immanuel Klinik Rüdersdorf, University Hospital of the Brandenburg Medical School and Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany. FAU - Rojas-Serrano, Jorge AU - Rojas-Serrano J AUID- ORCID: 0000-0001-6980-7898 AD - Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico. FAU - Hudson, Marie AU - Hudson M AUID- ORCID: 0000-0001-6718-2468 AD - McGill University, Montreal, Quebec, Canada. FAU - Kuwana, Masataka AU - Kuwana M AUID- ORCID: 0000-0001-8352-6136 AD - Nippon Medical School Graduate School of Medicine, Tokyo, Japan. FAU - González-Gay, Miguel Angel AU - González-Gay MA AUID- ORCID: 0000-0002-7924-7406 AD - Instituto de Investigación Sanitaria-Fundación Jiménez Díaz, Madrid, and University of Cantabria, Santander, Spain. FAU - McHugh, Neil AU - McHugh N AUID- ORCID: 0000-0003-2765-658X AD - University of Bath, Bath, UK. FAU - Corte, Tamera J AU - Corte TJ AUID- ORCID: 0000-0002-5076-8929 AD - Royal Prince Alfred Hospital, University of Sydney, Sydney, New South Wales, Australia. FAU - Doyle, Tracy Jennifer AU - Doyle TJ AUID- ORCID: 0000-0002-0770-1059 AD - Brigham and Women's Hospital, Boston, Massachusetts. FAU - Werth, Victoria P AU - Werth VP AUID- ORCID: 0000-0003-3030-5369 AD - Perelman School of Medicine & Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center, Philadelphia, Pennsylvania. FAU - Gupta, Latika AU - Gupta L AUID- ORCID: 0000-0003-2753-2990 AD - Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, and The University of Manchester, Manchester, UK. FAU - Perez Roman, Diana Isabel AU - Perez Roman DI AUID- ORCID: 0000-0003-3325-9214 AD - Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Mexico City, Mexico. FAU - Bianchessi, Lorenzo M AU - Bianchessi LM AD - Università di Pavia, Pavia, Italy. FAU - Devarasetti, Phani Kumar AU - Devarasetti PK AUID- ORCID: 0000-0002-6251-7828 AD - The University of Manchester, Manchester, UK. FAU - Shinjo, Samuel Katsuyuki AU - Shinjo SK AUID- ORCID: 0000-0002-3682-4517 AD - Faculdade de Medicina Faculdade de Medicina, Universidade de São Paulo, Universidade de Sao Paulo, Sao Paulo, Brazil. FAU - Luppi, Fabrizio AU - Luppi F AUID- ORCID: 0000-0001-5775-5947 AD - University of Milano Bicocca, Monza, Italy. FAU - Cavazzana, Ilaria AU - Cavazzana I AUID- ORCID: 0000-0002-2757-7120 AD - University and Azienda Ospedaliera Spedali Civili, Brescia, Italy. FAU - Moghadam-Kia, Siamak AU - Moghadam-Kia S AUID- ORCID: 0000-0002-0994-1590 AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Fornaro, Marco AU - Fornaro M AUID- ORCID: 0000-0003-1716-7432 AD - University of Bari, Bari, Italy. FAU - Volkmann, Elizabeth R AU - Volkmann ER AUID- ORCID: 0000-0003-3750-6569 AD - David Geffen School of Medicine, University of California, Los Angeles, California. FAU - Piga, Matteo AU - Piga M AUID- ORCID: 0000-0002-1126-8315 AD - University of Cagliari and Azienda Ospedaliera Universitaria Cagliari, Cagliari, Italy. FAU - Loarce-Martos, Jesus AU - Loarce-Martos J AUID- ORCID: 0000-0003-1352-9539 AD - Hospital Universitario Ramón y Cajal, Madrid, Spain. FAU - De Luca, Giacomo AU - De Luca G AUID- ORCID: 0000-0002-5306-7714 AD - Vita-Salute San Raffaele University & Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele Hospital, Milan, Italy. FAU - Knitza, Johannes AU - Knitza J AUID- ORCID: 0000-0001-9695-0657 AD - University Hospital of Giessen and Marburg, Philipps-University Marburg, Marburg, and Friedrich-Alexander-Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. FAU - Wolff-Cecchi, Veronica AU - Wolff-Cecchi V AUID- ORCID: 0000-0001-9924-4950 AD - Instituto Nacional del Torax and Universidad de Chile, Santiago, Chile. FAU - Sebastiani, Marco AU - Sebastiani M AUID- ORCID: 0000-0002-1294-6421 AD - Guglielmo da Saliceto Hospital of Piacenza, University of Parma, Parma, Italy. FAU - Schiffenbauer, Adam AU - Schiffenbauer A AUID- ORCID: 0000-0001-9964-9966 AD - National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Rider, Lisa G AU - Rider LG AUID- ORCID: 0000-0002-6912-2458 AD - National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland. FAU - Campanilho-Marques, Raquel AU - Campanilho-Marques R AUID- ORCID: 0000-0002-1894-8516 AD - Faculdade de Medicina, Universidade de Lisboa and Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal. FAU - Marts, Lucian AU - Marts L AUID- ORCID: 0000-0003-3210-8051 AD - Emory University School of Medicine, Atlanta, Georgia. FAU - Bravi, Elena AU - Bravi E AUID- ORCID: 0000-0002-4416-2568 AD - Ospedale Guglielmo da Saliceto, Piacenza, Italy. FAU - Gunawardena, Harsha AU - Gunawardena H AUID- ORCID: 0009-0001-2197-5582 AD - North Bristol NHS Trust, Bristol, UK. FAU - Aggarwal, Rohit AU - Aggarwal R AUID- ORCID: 0000-0001-7531-8038 AD - University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania. FAU - Cavagna, Lorenzo AU - Cavagna L AUID- ORCID: 0000-0003-3292-1528 AD - Università di Pavia and Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy. CN - Classification Criteria for Anti‐Synthetase Syndrome Project participating investigators LA - eng GR - American College of Rheumatology Research and Education Foundation/ GR - ZIA ES101081/ImNIH/Intramural NIH HHS/United States GR - European League Against Rheumatism/ GR - ZIA ES101081/ES/NIEHS NIH HHS/United States GR - R01 AR076766/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Multicenter Study DEP - 20241217 PL - United States TA - Arthritis Rheumatol JT - Arthritis & rheumatology (Hoboken, N.J.) JID - 101623795 RN - 0 (Autoantibodies) RN - 0 (Antibodies, Antinuclear) RN - Antisynthetase syndrome SB - IM MH - Humans MH - Male MH - Female MH - Middle Aged MH - *Myositis/immunology/diagnosis/classification/physiopathology MH - Adult MH - Lung Diseases, Interstitial/immunology MH - Aged MH - *Autoimmune Diseases/immunology/diagnosis/classification MH - Autoantibodies/blood/immunology MH - Antibodies, Antinuclear/immunology MH - Muscle Weakness/immunology MH - Hypertension, Pulmonary/etiology MH - Case-Control Studies MH - Arthritis/immunology PMC - PMC11936500 FIR - Anuja, Anamika IR - Anuja A FIR - Sambataro, Domenico IR - Sambataro D FIR - Colaci, Michele IR - Colaci M FIR - Vancheri, Carlo IR - Vancheri C FIR - Mejia, Mayra IR - Mejia M FIR - Rivera Matias, Pedro Abisay IR - Rivera Matias PA FIR - Bottazzi, Francesca IR - Bottazzi F FIR - Alpini, Claudia IR - Alpini C FIR - Rajasekhar, Liza IR - Rajasekhar L FIR - Agarwal, Vikas IR - Agarwal V FIR - De Souza, Fernando Henrique Carlos IR - De Souza FHC FIR - Miossi, Renata IR - Miossi R FIR - Pozzi, Maria Rosa IR - Pozzi MR FIR - Faverio, Paola IR - Faverio P FIR - Franco, Giovanni IR - Franco G FIR - Dei, Giulia IR - Dei G FIR - Franceschini, Franco IR - Franceschini F FIR - Lacomis, David IR - Lacomis D FIR - Iannone, Florenzo IR - Iannone F FIR - Girolamo, Francesco IR - Girolamo F FIR - Trallero-Araguás, Ernesto IR - Trallero-Araguás E FIR - Gil-Vila, Albert IR - Gil-Vila A FIR - Villar, Ana IR - Villar A FIR - Charles-Schoeman, Christina IR - Charles-Schoeman C FIR - Bukiri, Heather IR - Bukiri H FIR - Murru, Chiara IR - Murru C FIR - Cauli, Alberto IR - Cauli A FIR - Bachiller-Corral, Javier IR - Bachiller-Corral J FIR - Dagna, Lorenzo IR - Dagna L FIR - Campochiaro, Corrado IR - Campochiaro C FIR - Distler, Jorg IR - Distler J FIR - Schett, George IR - Schett G FIR - Florenzano, Matias IR - Florenzano M FIR - Reyes, Felipe IR - Reyes F FIR - Amato, Antony IR - Amato A FIR - Dellaripa, Paul IR - Dellaripa P FIR - Saavedra, Silvana IR - Saavedra S FIR - Vergara, Karen IR - Vergara K FIR - Manfredi, Andreina IR - Manfredi A FIR - Vacchi, Caterina IR - Vacchi C FIR - Melo, Teresa IR - Melo T FIR - Gandiga, Prateek IR - Gandiga P FIR - Amratia, Dhruv IR - Amratia D FIR - Arrigoni, Eugenio IR - Arrigoni E FIR - Kaneko, Yuko IR - Kaneko Y FIR - Triantafyllias, Konstantinos IR - Triantafyllias K FIR - Mandel, Anna IR - Mandel A FIR - Castañeda, Santos IR - Castañeda S FIR - Vicente-Rabaneda, Esther Francisca IR - Vicente-Rabaneda EF FIR - Landon-Cardinal, Océane IR - Landon-Cardinal O FIR - Alberti, María Laura IR - Alberti ML FIR - Caro, Fabian IR - Caro F FIR - Greco Merino, Martín Gerardo IR - Greco Merino MG FIR - Rua-Figueroa, Iñigo IR - Rua-Figueroa I FIR - Fiehn, Christoph IR - Fiehn C FIR - Bauhammer, Jutta IR - Bauhammer J FIR - Molad, Yair IR - Molad Y FIR - Klein, Osnat IR - Klein O FIR - Govoni, Marcello IR - Govoni M FIR - Nakashima, Ran IR - Nakashima R FIR - Alpsoy, Erkan IR - Alpsoy E FIR - Meyer, Alain IR - Meyer A FIR - Giannini, Margherita IR - Giannini M FIR - Chinoy, Hector IR - Chinoy H FIR - Lilleker, James IR - Lilleker J FIR - Gallay, Laure IR - Gallay L FIR - Cottin, Vincent IR - Cottin V FIR - Dieudè, Philippe IR - Dieudè P FIR - Campagne, Julien IR - Campagne J FIR - Saraiva, André Pinto IR - Saraiva AP FIR - Conticini, Edoardo IR - Conticini E FIR - Sebastiani, Giandomenco IR - Sebastiani G FIR - Nuno, Laura IR - Nuno L FIR - Scarpato, Salvatore IR - Scarpato S FIR - Schiopu, Elena IR - Schiopu E FIR - Sainaghi, Pier Paolo IR - Sainaghi PP FIR - Jovani, Vega IR - Jovani V FIR - Barsotti, Simone IR - Barsotti S FIR - Umezawa, Natsuka IR - Umezawa N FIR - Needham, Merrilee IR - Needham M FIR - Cagnotto, Giovanni IR - Cagnotto G FIR - Danieli, Maria Giovanna IR - Danieli MG FIR - Tomietto, Paola IR - Tomietto P FIR - Gomez, Nair Perez IR - Gomez NP FIR - Limonta, Massimiliano IR - Limonta M FIR - Barausse, Giovanni IR - Barausse G FIR - Kondo, Yasuhiro IR - Kondo Y FIR - Ghetie, Daniela IR - Ghetie D FIR - Tamirou, Farah IR - Tamirou F FIR - Basharat, Pari IR - Basharat P FIR - Tomaras, Stylianos IR - Tomaras S FIR - Voll, Reinhard Edmund IR - Voll RE FIR - Andersson, Helena IR - Andersson H FIR - Barrio, Julia Martinez IR - Barrio JM FIR - Schneider, Matthias IR - Schneider M FIR - Parronchi, Paola IR - Parronchi P FIR - Notarnicola, Antonella IR - Notarnicola A FIR - Cifrian, Jose IR - Cifrian J EDAT- 2024/10/28 18:23 MHDA- 2025/03/26 04:06 PMCR- 2025/03/25 CRDT- 2024/10/28 14:34 PHST- 2024/09/19 00:00 [revised] PHST- 2024/06/07 00:00 [received] PHST- 2024/10/08 00:00 [accepted] PHST- 2025/03/26 04:06 [medline] PHST- 2024/10/28 18:23 [pubmed] PHST- 2024/10/28 14:34 [entrez] PHST- 2025/03/25 00:00 [pmc-release] AID - ART43038 [pii] AID - 10.1002/art.43038 [doi] PST - ppublish SO - Arthritis Rheumatol. 2025 Apr;77(4):477-489. doi: 10.1002/art.43038. Epub 2024 Dec 17. PMID- 31011205 OWN - NLM STAT- MEDLINE DCOM- 20190710 LR - 20210109 IS - 1546-170X (Electronic) IS - 1078-8956 (Print) IS - 1078-8956 (Linking) VI - 25 IP - 5 DP - 2019 May TI - Genomic and transcriptomic profiling expands precision cancer medicine: the WINTHER trial. PG - 751-758 LID - 10.1038/s41591-019-0424-4 [doi] AB - Precision medicine focuses on DNA abnormalities, but not all tumors have tractable genomic alterations. The WINTHER trial ( NCT01856296 ) navigated patients to therapy on the basis of fresh biopsy-derived DNA sequencing (arm A; 236 gene panel) or RNA expression (arm B; comparing tumor to normal). The clinical management committee (investigators from five countries) recommended therapies, prioritizing genomic matches; physicians determined the therapy given. Matching scores were calculated post-hoc for each patient, according to drugs received: for DNA, the number of alterations matched divided by the total alteration number; for RNA, expression-matched drug ranks. Overall, 303 patients consented; 107 (35%; 69 in arm A and 38 in arm B) were evaluable for therapy. The median number of previous therapies was three. The most common diagnoses were colon, head and neck, and lung cancers. Among the 107 patients, the rate of stable disease ≥6 months and partial or complete response was 26.2% (arm A: 23.2%; arm B: 31.6% (P = 0.37)). The patient proportion with WINTHER versus previous therapy progression-free survival ratio of >1.5 was 22.4%, which did not meet the pre-specified primary end point. Fewer previous therapies, better performance status and higher matching score correlated with longer progression-free survival (all P < 0.05, multivariate). Our study shows that genomic and transcriptomic profiling are both useful for improving therapy recommendations and patient outcome, and expands personalized cancer treatment. FAU - Rodon, Jordi AU - Rodon J AD - Vall d'Hebron Institute of Oncology (VHIO), Barcelona, Spain. AD - Department of Investigational Cancer Therapeutics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Soria, Jean-Charles AU - Soria JC AD - Gustave Roussy, Villejuif, France. FAU - Berger, Raanan AU - Berger R AD - Chaim Sheba Medical Center, Tel Hashomer, Israel. FAU - Miller, Wilson H AU - Miller WH AD - Segal Cancer Centre, Jewish General Hospital, QCROC-Quebec Cancer Consortium and Rossy Cancer Network, McGill University, Montreal, Québec, Canada. FAU - Rubin, Eitan AU - Rubin E AUID- ORCID: 0000-0002-7807-4005 AD - Ben-Gurion University of the Negev, Beersheva, Israel. FAU - Kugel, Aleksandra AU - Kugel A AD - Ben-Gurion University of the Negev, Beersheva, Israel. FAU - Tsimberidou, Apostolia AU - Tsimberidou A AD - Department of Investigational Cancer Therapeutics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Saintigny, Pierre AU - Saintigny P AD - Centre Léon-Bérard, Lyon, France. FAU - Ackerstein, Aliza AU - Ackerstein A AD - Chaim Sheba Medical Center, Tel Hashomer, Israel. FAU - Braña, Irene AU - Braña I AD - Vall d'Hebron Institute of Oncology (VHIO), Barcelona, Spain. FAU - Loriot, Yohann AU - Loriot Y AD - Gustave Roussy, Villejuif, France. FAU - Afshar, Mohammad AU - Afshar M AD - Ariana Pharmaceuticals, Paris, France. FAU - Miller, Vincent AU - Miller V AD - Foundation Medicine Inc., Cambridge, MA, USA. FAU - Wunder, Fanny AU - Wunder F AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. FAU - Bresson, Catherine AU - Bresson C AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. FAU - Martini, Jean-François AU - Martini JF AD - Pfizer Inc., San Diego, CA, USA. FAU - Raynaud, Jacques AU - Raynaud J AD - ARC Foundation for Cancer Research, Villejuif, France. FAU - Mendelsohn, John AU - Mendelsohn J AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. AD - Sheikh Khalifa Bin Zayad Al Nahyan Institute for Personalized Cancer Therapy (IPCT), The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Batist, Gerald AU - Batist G AD - Segal Cancer Centre, Jewish General Hospital, QCROC-Quebec Cancer Consortium and Rossy Cancer Network, McGill University, Montreal, Québec, Canada. FAU - Onn, Amir AU - Onn A AD - Chaim Sheba Medical Center, Tel Hashomer, Israel. FAU - Tabernero, Josep AU - Tabernero J AUID- ORCID: 0000-0002-2495-8139 AD - Vall d'Hebron Institute of Oncology (VHIO), Barcelona, Spain. FAU - Schilsky, Richard L AU - Schilsky RL AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. AD - American Society of Clinical Oncology (ASCO), Alexandria, VA, USA. FAU - Lazar, Vladimir AU - Lazar V AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. FAU - Lee, J Jack AU - Lee JJ AUID- ORCID: 0000-0001-5469-9214 AD - Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Kurzrock, Razelle AU - Kurzrock R AUID- ORCID: 0000-0003-4110-1214 AD - Worldwide Innovative Network (WIN) Association-WIN Consortium, Villejuif, France. rkurzrock@ucsd.edu. AD - University of California San Diego, Moores Cancer Center, San Diego, CA, USA. rkurzrock@ucsd.edu. LA - eng GR - P30 CA016672/CA/NCI NIH HHS/United States GR - P30 CA023100/CA/NCI NIH HHS/United States PT - Clinical Trial PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20190422 PL - United States TA - Nat Med JT - Nature medicine JID - 9502015 RN - 0 (Biomarkers, Tumor) SB - IM CIN - Nat Med. 2019 May;25(5):711-712. doi: 10.1038/s41591-019-0442-2. PMID: 31036881 CIN - Cancer Cell. 2019 Jun 10;35(6):825-826. doi: 10.1016/j.ccell.2019.05.009. PMID: 31185208 CIN - Cancer Discov. 2019 Aug;9(8):OF8. doi: 10.1158/2159-8290.CD-ND2019-008. PMID: 31242995 MH - Adult MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Biomarkers, Tumor/genetics MH - Combined Modality Therapy MH - Female MH - Gene Expression Profiling MH - Genomics MH - High-Throughput Nucleotide Sequencing MH - Humans MH - Kaplan-Meier Estimate MH - Lung Neoplasms/genetics/therapy MH - Male MH - Middle Aged MH - Molecular Targeted Therapy MH - Neoplasms/*genetics/*therapy MH - Precision Medicine MH - Progression-Free Survival MH - Sequence Analysis, DNA PMC - PMC6599610 MID - NIHMS1036639 COIS- Competing interests J.Rodon reports non-financial support and reasonable reimbursement for travel from the European Journal of Cancer, Vall d’Hebron Institut of Oncology, the Chinese University of Hong Kong, SOLTI, Elsevier, GlaxoSmithKline; receives consulting and travel fees from Novartis, Eli Lilly, Orion Pharmaceuticals, Servier Pharmaceuticals, Peptomyc, Merck Sharp & Dohme, Kelun Pharmaceutical/Klus Pharma, Spectrum Pharmaceuticals, Pfizer, Roche Pharmaceuticals and Ellipses Pharma (including serving on the scientific advisory board from 2015 to present); receives research funding from Bayer and Novartis; and serves as investigator in clinical trials with Spectrum Pharmaceuticals, Tocagen, Symphogen, BioAtla, Pfizer, GenMab, CytomX, Kelun Pharmaceutical/Klus Pharma, Takeda-Millenium, GlaxoSmithKline and Ipsen. J.-C.S. received consultancy fees from AstraZeneca, Roche, Sanofi, Servier, Pierre Fabre and is a full-time employee of Medimmune/AstraZeneca since September 2017. W.H.M. receives speaking and/or consulting fees from BMS, Merck, Roche, Novartis and Amgen. E.R. received consultant fees from Teva, Carmentix and Hinoman, is receiving consultant fees from Equinom and has ownership interest in Carmentix. A.O. receives consulting fees from Roche Israel, MSD Israel, Boehringer Ingelheim and AstraZeneca. A.T. received consultant fees from Roche and receives research funding from EMD Serono, Baxter, Foundation Medicine, ONYX and Bayer. PS. collaborates in research with Roche, AstraZeneca, BMS and Novartis. I.B. receives consultant fees from Orion Pharma, receives speaking fees from BMS, AstraZeneca and Merck Serono, and is principal investigator and receives funding for clinical trials from AstraZeneca, BMS, Celgene, Gliknik, GSK, Janssen, KURA, MSD, Novartis, Orion Pharma and Pfizer. Y.L. collaborates in research with Merck, Roche, AstraZeneca, Sanofi, Pfizer, Janssen, Astellas and BMS. M.A. is an employee and shareholder of Ariana Pharmaceuticals. VM. is an employee (salary and equity) of Foundation Medicine. J.-F.M. is a full-time employee and stockholder of Pfizer. G.B. collaborates in formal clinical trial contracts, investigator initiated trials (IITs) and in joint grants funded by the Canadian and Quebec governments with Roche, Merck, Novartis, AstraZeneca, Bayer, Esperas, Aurka, Caprion and MRM-P. J.T. declares a scientific consultancy role for Array Biopharma, AstraZeneca, Bayer, BeiGene, Boehringer Ingelheim, Chugai, Genentech, Genmab A/S, Halozyme, Imugene, Inflection Biosciences, Ipsen, Kura Oncology, Lilly, MSD, Menarini, Merck Serono, Merrimack, Merus, Molecular Partners, Novartis, Peptomyc, Pfizer, Pharmacyclics, ProteoDesign SL, Rafael Pharmaceuticals, F. Hoffmann-La Roche, Sanofi, SeaGen, Seattle Genetics, Servier, Symphogen, Taiho, VCN Biosciences, Biocartis, Foundation Medicine, HalioDX SAS and Roche Diagnostics. R.K. has research funding from Incyte, Genentech, Merck Serono, Pfizer, Sequenom, Foundation Medicine, Guardant Health, Grifols, Konica Minolta and OmniSeq, as well as consultant fees from LOXO, X-Biotech, Actuate Therapeutics, Roche and NeoMed. She serves as an advisor to Soluventis. She receives speaker fees from Roche and also has equity in IDbyDNA, CureMatch and Soluventis. F.W., C.B. and V.L. are full-time employees of WIN Association-WIN Consortium. WIN Association-WIN Consortium is the owner of the patent family entitled ‘Method for predicting efficacy of drugs in a patient’ (WINTHER). The inventors are V.L., J.-C.S., Michel Ducreux and Thomas Tursz. EDAT- 2019/04/24 06:00 MHDA- 2019/07/11 06:00 PMCR- 2019/11/01 CRDT- 2019/04/24 06:00 PHST- 2018/11/08 00:00 [received] PHST- 2019/03/14 00:00 [accepted] PHST- 2019/04/24 06:00 [pubmed] PHST- 2019/07/11 06:00 [medline] PHST- 2019/04/24 06:00 [entrez] PHST- 2019/11/01 00:00 [pmc-release] AID - 10.1038/s41591-019-0424-4 [pii] AID - 10.1038/s41591-019-0424-4 [doi] PST - ppublish SO - Nat Med. 2019 May;25(5):751-758. doi: 10.1038/s41591-019-0424-4. Epub 2019 Apr 22. PMID- 32532747 OWN - NLM STAT- MEDLINE DCOM- 20211119 LR - 20250530 IS - 2159-8290 (Electronic) IS - 2159-8274 (Print) IS - 2159-8274 (Linking) VI - 10 IP - 10 DP - 2020 Oct TI - Phase I Trial of the PARP Inhibitor Olaparib and AKT Inhibitor Capivasertib in Patients with BRCA1/2- and Non-BRCA1/2-Mutant Cancers. PG - 1528-1543 LID - 10.1158/2159-8290.CD-20-0163 [doi] AB - Preclinical studies have demonstrated synergy between PARP and PI3K/AKT pathway inhibitors in BRCA1 and BRCA2 (BRCA1/2)-deficient and BRCA1/2-proficient tumors. We conducted an investigator-initiated phase I trial utilizing a prospective intrapatient dose- escalation design to assess two schedules of capivasertib (AKT inhibitor) with olaparib (PARP inhibitor) in 64 patients with advanced solid tumors. Dose expansions enrolled germline BRCA1/2-mutant tumors, or BRCA1/2 wild-type cancers harboring somatic DNA damage response (DDR) or PI3K-AKT pathway alterations. The combination was well tolerated. Recommended phase II doses for the two schedules were: olaparib 300 mg twice a day with either capivasertib 400 mg twice a day 4 days on, 3 days off, or capivasertib 640 mg twice a day 2 days on, 5 days off. Pharmacokinetics were dose proportional. Pharmacodynamic studies confirmed phosphorylated (p) GSK3β suppression, increased pERK, and decreased BRCA1 expression. Twenty-five (44.6%) of 56 evaluable patients achieved clinical benefit (RECIST complete response/partial response or stable disease ≥ 4 months), including patients with tumors harboring germline BRCA1/2 mutations and BRCA1/2 wild-type cancers with or without DDR and PI3K-AKT pathway alterations. SIGNIFICANCE: In the first trial to combine PARP and AKT inhibitors, a prospective intrapatient dose- escalation design demonstrated safety, tolerability, and pharmacokinetic-pharmacodynamic activity and assessed predictive biomarkers of response/resistance. Antitumor activity was observed in patients harboring tumors with germline BRCA1/2 mutations and BRCA1/2 wild-type cancers with or without somatic DDR and/or PI3K-AKT pathway alterations.This article is highlighted in the In This Issue feature, p. 1426. CI - ©2020 American Association for Cancer Research. FAU - Yap, Timothy A AU - Yap TA AUID- ORCID: 0000-0002-2154-3309 AD - Royal Marsden Hospital, London, United Kingdom. tyap@mdanderson.org. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Kristeleit, Rebecca AU - Kristeleit R AUID- ORCID: 0000-0003-3825-1326 AD - University College London, London, United Kingdom. FAU - Michalarea, Vasiliki AU - Michalarea V AD - Royal Marsden Hospital, London, United Kingdom. FAU - Pettitt, Stephen J AU - Pettitt SJ AUID- ORCID: 0000-0003-3313-3857 AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. AD - The CRUK Gene Function Laboratory, The Institute of Cancer Research, London, United Kingdom. FAU - Lim, Joline S J AU - Lim JSJ AD - Royal Marsden Hospital, London, United Kingdom. FAU - Carreira, Suzanne AU - Carreira S AD - The Institute of Cancer Research, London, United Kingdom. FAU - Roda, Desamparados AU - Roda D AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Miller, Rowan AU - Miller R AD - University College London, London, United Kingdom. FAU - Riisnaes, Ruth AU - Riisnaes R AD - The Institute of Cancer Research, London, United Kingdom. FAU - Miranda, Susana AU - Miranda S AUID- ORCID: 0000-0002-5936-2706 AD - The Institute of Cancer Research, London, United Kingdom. FAU - Figueiredo, Ines AU - Figueiredo I AD - The Institute of Cancer Research, London, United Kingdom. FAU - Rodrigues, Daniel Nava AU - Rodrigues DN AD - The Institute of Cancer Research, London, United Kingdom. FAU - Ward, Sarah AU - Ward S AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Matthews, Ruth AU - Matthews R AUID- ORCID: 0000-0003-1697-3109 AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Parmar, Mona AU - Parmar M AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Turner, Alison AU - Turner A AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Tunariu, Nina AU - Tunariu N AUID- ORCID: 0000-0001-6656-3699 AD - Royal Marsden Hospital, London, United Kingdom. FAU - Chopra, Neha AU - Chopra N AD - Royal Marsden Hospital, London, United Kingdom. AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Gevensleben, Heidrun AU - Gevensleben H AD - The Institute of Cancer Research, London, United Kingdom. FAU - Turner, Nicholas C AU - Turner NC AD - Royal Marsden Hospital, London, United Kingdom. AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Ruddle, Ruth AU - Ruddle R AUID- ORCID: 0000-0003-0025-8872 AD - The Institute of Cancer Research, London, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - The Institute of Cancer Research, London, United Kingdom. FAU - Decordova, Shaun AU - Decordova S AUID- ORCID: 0000-0002-0091-0587 AD - The Institute of Cancer Research, London, United Kingdom. FAU - Swales, Karen E AU - Swales KE AUID- ORCID: 0000-0002-6572-1293 AD - The Institute of Cancer Research, London, United Kingdom. FAU - Finneran, Laura AU - Finneran L AD - The Institute of Cancer Research, London, United Kingdom. FAU - Hall, Emma AU - Hall E AUID- ORCID: 0000-0001-5999-5020 AD - The Institute of Cancer Research, London, United Kingdom. FAU - Rugman, Paul AU - Rugman P AD - Oncology R&D, AstraZeneca, Cambridge, United Kingdom. FAU - Lindemann, Justin P O AU - Lindemann JPO AUID- ORCID: 0000-0003-1531-9754 AD - Oncology R&D, AstraZeneca, Cambridge, United Kingdom. FAU - Foxley, Andrew AU - Foxley A AD - Oncology R&D, AstraZeneca, Cambridge, United Kingdom. FAU - Lord, Christopher J AU - Lord CJ AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. AD - The CRUK Gene Function Laboratory, The Institute of Cancer Research, London, United Kingdom. FAU - Banerji, Udai AU - Banerji U AUID- ORCID: 0000-0003-1503-3123 AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Plummer, Ruth AU - Plummer R AD - Clinical and Translational Research Institute, Newcastle University, Newcastle, United Kingdom. FAU - Basu, Bristi AU - Basu B AUID- ORCID: 0000-0002-3562-2868 AD - Department of Oncology, University of Cambridge, Cambridge, United Kingdom. FAU - Lopez, Juanita S AU - Lopez JS AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. FAU - Drew, Yvette AU - Drew Y AUID- ORCID: 0000-0001-6676-9224 AD - Clinical and Translational Research Institute, Newcastle University, Newcastle, United Kingdom. FAU - de Bono, Johann S AU - de Bono JS AD - Royal Marsden Hospital, London, United Kingdom. AD - The Institute of Cancer Research, London, United Kingdom. LA - eng GR - P30 CA016672/CA/NCI NIH HHS/United States GR - RP-2016-07-028/DH_/Department of Health/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom GR - 24439/CRUK_/Cancer Research UK/United Kingdom GR - 14276/CRUK_/Cancer Research UK/United Kingdom GR - 22897/CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial, Phase I PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20200612 PL - United States TA - Cancer Discov JT - Cancer discovery JID - 101561693 RN - 0 (BRCA1 Protein) RN - 0 (BRCA1 protein, human) RN - 0 (BRCA2 Protein) RN - 0 (BRCA2 protein, human) RN - 0 (Phthalazines) RN - 0 (Piperazines) RN - 0 (Poly(ADP-ribose) Polymerase Inhibitors) RN - 0 (Pyrimidines) RN - 0 (Pyrroles) RN - WFR23M21IE (capivasertib) RN - WOH1JD9AR8 (olaparib) SB - IM MH - Adult MH - Aged MH - Antineoplastic Combined Chemotherapy Protocols/pharmacology/*therapeutic use MH - BRCA1 Protein/*metabolism MH - BRCA2 Protein/*metabolism MH - Female MH - Humans MH - Middle Aged MH - Phthalazines/pharmacology/*therapeutic use MH - Piperazines/pharmacology/*therapeutic use MH - Poly(ADP-ribose) Polymerase Inhibitors/pharmacology/*therapeutic use MH - Pyrimidines/pharmacology/*therapeutic use MH - Pyrroles/pharmacology/*therapeutic use PMC - PMC7611385 MID - EMS128585 EDAT- 2020/06/14 06:00 MHDA- 2021/11/20 06:00 PMCR- 2021/07/27 CRDT- 2020/06/14 06:00 PHST- 2020/02/15 00:00 [received] PHST- 2020/05/20 00:00 [revised] PHST- 2020/06/09 00:00 [accepted] PHST- 2020/06/14 06:00 [pubmed] PHST- 2021/11/20 06:00 [medline] PHST- 2020/06/14 06:00 [entrez] PHST- 2021/07/27 00:00 [pmc-release] AID - 2159-8290.CD-20-0163 [pii] AID - 10.1158/2159-8290.CD-20-0163 [doi] PST - ppublish SO - Cancer Discov. 2020 Oct;10(10):1528-1543. doi: 10.1158/2159-8290.CD-20-0163. Epub 2020 Jun 12. PMID- 26350554 OWN - NLM STAT- MEDLINE DCOM- 20161031 LR - 20260127 IS - 0013-7006 (Print) IS - 0013-7006 (Linking) VI - 42 IP - 1 DP - 2016 Feb TI - [Perstimulus asystole during electroconvulsive therapy: Clinical case and critical literature review]. PG - 59-66 LID - S0013-7006(15)00121-9 [pii] LID - 10.1016/j.encep.2015.06.004 [doi] AB - INTRODUCTION: Electroconvulsive therapy (ECT) is most frequently indicated for episodes of melancholic depression, but is also useful in the treatment of maniac syndrome and some schizophrenia subtypes. ECT is part of the treatment of movement disorders, neuroleptic malignant syndrome and even in the treatment of severe conversions. Although the therapeutic results are excellent when used appropriately, the mortality rate is estimated between 2 and 4 for 100,000 shocks. Despite this mortality rate, the benefit-risk ratio remains very positive and serious complications are extremely rare. ECT results in a biphasic cardiological effect: firstly a perstimulus parasympathetic hypertonia contemporary to the seizure's tonic phase, then a phase of contemporary sympathetic hypertonia during the epileptic clonic movement. We will focus on the perstimulus asystole as it is by far the most frequent. Very few cases and even less studies have been referenced in the literature; here, we present a clinical case followed by a discussion. CLINICAL CASE: The patient is in his fifties and has been treated for many years for a unipolar mood disorder with recurrent melancholic depressive episodes. With each new depressive episode, the clinical evolution is rapidly positive after a few sessions of ECT. Maintenance ECT was not retained due to the supra-annual periodicity of the melancholic depressive episodes and rapid recovery after electric treatment. Then, this patient developed another depressive decline in mood comparable to the previous one, despite adapted blood lithium levels associated with a new generation antidepressant treatment. According to his history, a hospitalisation was programmed to carry out a new course of ECT. Considering the short duration of the first seizures, the intensity of the stimulus was progressively increased. At 180 joules, the patient presented an immediate per-stimulus asystole of 20seconds which ceased spontaneously. The specialized cardiologic consultation following the rhythmic episode was reassuring: the patient's cardiac condition remained stable. However, after discussion with the patient and his family, we decided to stop the ECT. Was this a reasonable decision? DISCUSSION: According to the literature, the patient's medical history, sex, psychiatric diagnosis, the shock parameters (level of energy applied, duration of the stimulus, number of shocks) and clinical results, are not predictive factors in the occurrence of an asystole. Concerning the ECT protocol, the vagus nerve seems less stimulated during bifrontal stimulations in opposition to unilateral stimulations. Perasystolic patients are younger and have less prior history of cardiovascular disease or ECG abnormalities. Although the patients receiving ECT are often taking several medications (antipsychotics, benzodiazepines, antidepressants, anticholinergic correctors, calcium channel blockers, loop diuretics, converting enzyme inhibitors), these drugs are not considered as facilitating asystoles. No increase in the frequency of asystole had been observed when taking an average dose psychotropic treatment allowing the continuation of an antidepressant treatment at the recommended dose. Differently, lithium is regularly stopped during the shock phase as it could - even a few days after being stopped - potentiate the effects of succinylcholine and increase the vagal tone. Succinylcholine seems to promote asystole, whilst caffeine, methohexital and trimethaphan do not. The hypersympathetic phase can be controlled by a betablocker (propranolol, esmolol, labetalol) that does not increase the prior risk of asystole. Anticholinergic premedication using atropine does not appear to be systematic and could even potentially induce tachy-dysarrhythmia. However, in the case of perstimulus asystole, most authors recommend continuing the shocks with doses of atropine around 0.4 to 1mg. PHYSIOPATHOLOGY: Vagal stimulation is preferentially central and directly linked to the electric excitation of the lateral dorsal motor nucleus of the vagus nerve. Younger patients with no cardiac history are more at risk. This could be explained by the fact that juvenile tissue conducts electricity more rapidly than senescent (the difference being probably due to the fibrosis and adipose tissue which reduce its conductive capacity). Finally, it is appropriate to question the direct therapeutic aspect of vagal stimulation which constitutes an experimental treatment of resistant depression. CONCLUSIONS: The occurrence of perstimulus asystole is not considered as a serious complication of ECT and therefore as a contra-indication to any future sessions. On the contrary, most authors are campaigning for the continuation of shocks with the possibility of adding prophylactic intravenous atropine. Cardiac arrest reminds us that ECT requires a special attention to its cardiovascular effect, which emphasizes the role of interdisciplinarity between anaesthesiologists and psychiatrists. CI - Copyright © 2015 L’Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved. FAU - Roche, N C AU - Roche NC AD - Service de cardiologie, Saint-Mandé Armées, hôpital d'instruction des Armées Bégin, 69, avenue de Paris, 94163 Saint-Mandé, France. FAU - Raynaud, L AU - Raynaud L AD - Service d'anesthésie et de réanimation, hôpital d'instuction des Armées du Val-de-Grâce, 74, boulevard Port-Royal, 75230 Paris cedex 5, France. FAU - Bompaire, F AU - Bompaire F AD - Service de neurologie, hôpital d'instruction des Armées du Val-de-Grâce, 74, boulevard Port-Royal, 75230 Paris cedex 5, France. FAU - Lucas, J-J AU - Lucas JJ AD - Service de psychiatrie et de psychologie clinique, hôpital d'instruction des Armées Legouest, 27, avenue de Plantières, BP 90001, 57077 Metz cedex 3, France. FAU - Auxéméry, Y AU - Auxéméry Y AD - Service de psychiatrie et de psychologie clinique, hôpital d'instruction des Armées Legouest, 27, avenue de Plantières, BP 90001, 57077 Metz cedex 3, France. Electronic address: yann.auxemery@hotmail.fr. LA - fre PT - Case Reports PT - Journal Article TT - Asystolie perstimulus résultant d'une hypertonie parasympathique au cours des électroconvulsivothérapies : cas clinique, brève revue de la littérature et discussion. DEP - 20150906 PL - France TA - Encephale JT - L'Encephale JID - 7505643 RN - 0 (Anti-Arrhythmia Agents) RN - 7C0697DR9I (Atropine) SB - IM MH - Aging MH - Anesthesia MH - Anti-Arrhythmia Agents/therapeutic use MH - Atropine/therapeutic use MH - Contraindications MH - Major Depressive Disorder/*complications/*therapy MH - Electric Conductivity MH - Electroconvulsive Therapy/*adverse effects MH - Heart Arrest/*etiology/prevention & control MH - Humans MH - Male MH - Middle Aged MH - Risk Factors MH - Vagus Nerve Stimulation OTO - NOTNLM OT - Asystole OT - Asystolie OT - Electroconvulsive therapy OT - Guidelines OT - Physiopathologie OT - Physiopathology OT - Recommandations OT - Électroconvulsivothérapie EDAT- 2015/09/10 06:00 MHDA- 2016/11/01 06:00 CRDT- 2015/09/10 06:00 PHST- 2013/05/02 00:00 [received] PHST- 2014/06/19 00:00 [accepted] PHST- 2015/09/10 06:00 [entrez] PHST- 2015/09/10 06:00 [pubmed] PHST- 2016/11/01 06:00 [medline] AID - S0013-7006(15)00121-9 [pii] AID - 10.1016/j.encep.2015.06.004 [doi] PST - ppublish SO - Encephale. 2016 Feb;42(1):59-66. doi: 10.1016/j.encep.2015.06.004. Epub 2015 Sep 6. PMID- 32859654 OWN - NLM STAT- MEDLINE DCOM- 20211203 LR - 20211214 IS - 1557-3265 (Electronic) IS - 1078-0432 (Linking) VI - 26 IP - 23 DP - 2020 Dec 1 TI - Circulating Tumor DNA Genomics Reveal Potential Mechanisms of Resistance to BRAF-Targeted Therapies in Patients with BRAF-Mutant Metastatic Non-Small Cell Lung Cancer. PG - 6242-6253 LID - 10.1158/1078-0432.CCR-20-1037 [doi] AB - PURPOSE: The limited knowledge on the molecular profile of patients with BRAF-mutant non-small cell lung cancer (NSCLC) who progress under BRAF-targeted therapies (BRAF-TT) has hampered the development of subsequent therapeutic strategies for these patients. Here, we evaluated the clinical utility of circulating tumor DNA (ctDNA)-targeted sequencing to identify canonical BRAF mutations and genomic alterations potentially related to resistance to BRAF-TT, in a large cohort of patients with BRAF-mutant NSCLC. EXPERIMENTAL DESIGN: This was a prospective study of 78 patients with advanced BRAF-mutant NSCLC, enrolled in 27 centers across France. Blood samples (n = 208) were collected from BRAF-TT-naïve patients (n = 47), patients nonprogressive under treatment (n = 115), or patients at disease progression (PD) to BRAF-TT (24/46 on BRAF monotherapy and 22/46 on BRAF/MEK combination therapy). ctDNA sequencing was performed using InVisionFirst-Lung. In silico structural modeling was used to predict the potential functional effect of the alterations found in ctDNA. RESULTS: BRAF(V600E) ctDNA was detected in 74% of BRAF-TT-naïve patients, where alterations in genes related with the MAPK and PI3K pathways, signal transducers, and protein kinases were identified in 29% of the samples. ctDNA positivity at the first radiographic evaluation under treatment, as well as BRAF-mutant ctDNA positivity at PD were associated with poor survival. Potential drivers of resistance to either BRAF-TT monotherapy or BRAF/MEK combination were identified in 46% of patients and these included activating mutations in effectors of the MAPK and PI3K pathways, as well as alterations in U2AF1, IDH1, and CTNNB1. CONCLUSIONS: ctDNA sequencing is clinically relevant for the detection of BRAF-activating mutations and the identification of alterations potentially related to resistance to BRAF-TT in BRAF-mutant NSCLC. CI - ©2020 American Association for Cancer Research. FAU - Ortiz-Cuaran, Sandra AU - Ortiz-Cuaran S AD - Univ Lyon, Claude Bernard Lyon 1 University, INSERM 1052, CNRS 5286, Centre Léon Bérard, Cancer Research Center of Lyon, Lyon, France. david.planchard@gustaveroussy.fr sandra.ortiz-cuaran@lyon.unicancer.fr pierre.saintigny@lyon.unicancer.fr. FAU - Mezquita, Laura AU - Mezquita L AD - Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. AD - Department of Medical Oncology, Hospital Clinic, Laboratory of Translational Genomics and Targeted Therapeutics in Solid Tumors, IDIBAPS, Barcelona, Spain. FAU - Swalduz, Aurélie AU - Swalduz A AD - Univ Lyon, Claude Bernard Lyon 1 University, INSERM 1052, CNRS 5286, Centre Léon Bérard, Cancer Research Center of Lyon, Lyon, France. AD - Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I/Université de Lyon, Lyon, France. FAU - Aldea, Mihalea AU - Aldea M AD - Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Mazieres, Julien AU - Mazieres J AUID- ORCID: 0000-0002-5921-7613 AD - Department of Respiratory Disease, Larrey Hospital, University Hospital of Toulouse, Paul Sabatier University, Toulouse, France. FAU - Leonce, Camille AU - Leonce C AD - Univ Lyon, Claude Bernard Lyon 1 University, INSERM 1052, CNRS 5286, Centre Léon Bérard, Cancer Research Center of Lyon, Lyon, France. FAU - Jovelet, Cecile AU - Jovelet C AD - Translational Research Laboratory, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Pradines, Anne AU - Pradines A AD - Institut Claudius Regaud, Toulouse, France. FAU - Avrillon, Virginie AU - Avrillon V AD - Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I/Université de Lyon, Lyon, France. FAU - Chumbi Flores, Washington R AU - Chumbi Flores WR AD - Hospices Civils de Lyon, Lyon, France. FAU - Lacroix, Ludovic AU - Lacroix L AUID- ORCID: 0000-0003-2535-1010 AD - Translational Research Laboratory, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Loriot, Yohann AU - Loriot Y AD - Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Westeel, Virginie AU - Westeel V AD - University Hospital of Besancon, Besançon, France. FAU - Ngo-Camus, Maud AU - Ngo-Camus M AD - Department of Early Drug Development, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Tissot, Claire AU - Tissot C AD - University Hospital of Saint-Etienne, Saint-Etienne, France. FAU - Raynaud, Christine AU - Raynaud C AD - Centre Hospitalier Argenteuil, Argenteuil, France. FAU - Gervais, Radj AU - Gervais R AD - Centre François Baclesse, Caen, France. FAU - Brain, Etienne AU - Brain E AUID- ORCID: 0000-0003-0881-9371 AD - Hôpital René Huguenin, Saint-Cloud, France. FAU - Monnet, Isabelle AU - Monnet I AD - Centre Hospitalier Intercommunal de Créteil, Creteil, France. FAU - Giroux Leprieur, Etienne AU - Giroux Leprieur E AD - APHP-Hôpital Ambroise Paré and Université Paris Saclay, Paris, France. FAU - Caramella, Caroline AU - Caramella C AD - Department of Radiology, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Mahier-Aït Oukhatar, Celine AU - Mahier-Aït Oukhatar C AD - Unicancer, Paris, France. FAU - Hoog-Labouret, Natalie AU - Hoog-Labouret N AD - Institut National du Cancer, Boulogne-Billancourt, France. FAU - de Kievit, Frank AU - de Kievit F AD - Inivata Ltd, Cambridge, United Kingdom. FAU - Howarth, Karen AU - Howarth K AD - Inivata Ltd, Cambridge, United Kingdom. FAU - Morris, Clive AU - Morris C AD - Inivata Ltd, Cambridge, United Kingdom. FAU - Green, Emma AU - Green E AD - Inivata Ltd, Cambridge, United Kingdom. FAU - Friboulet, Luc AU - Friboulet L AUID- ORCID: 0000-0002-1129-4978 AD - Université Paris-Saclay, Gustave Roussy Cancer Campus, Inserm, Biomarqueurs Prédictifs et Nouvelles Stratégies Thérapeutiques en Oncologie, Villejuif, France. FAU - Chabaud, Sylvie AU - Chabaud S AD - Department of Clinical Research, Centre Léon Bérard, Lyon, France. FAU - Guichou, Jean-François AU - Guichou JF AD - Centre de Biochimie Structurale (CBS), INSERM, CNRS, Université de Montpellier, Montpellier, France. FAU - Perol, Maurice AU - Perol M AUID- ORCID: 0000-0002-3296-423X AD - Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I/Université de Lyon, Lyon, France. FAU - Besse, Benjamin AU - Besse B AD - Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. FAU - Blay, Jean-Yves AU - Blay JY AUID- ORCID: 0000-0001-7190-120X AD - Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I/Université de Lyon, Lyon, France. FAU - Saintigny, Pierre AU - Saintigny P AD - Univ Lyon, Claude Bernard Lyon 1 University, INSERM 1052, CNRS 5286, Centre Léon Bérard, Cancer Research Center of Lyon, Lyon, France. david.planchard@gustaveroussy.fr sandra.ortiz-cuaran@lyon.unicancer.fr pierre.saintigny@lyon.unicancer.fr. AD - Department of Medical Oncology, Centre Léon Bérard & Université Claude Bernard Lyon I/Université de Lyon, Lyon, France. FAU - Planchard, David AU - Planchard D AD - Department of Cancer Medicine, Gustave Roussy Cancer Campus, Villejuif, France. david.planchard@gustaveroussy.fr sandra.ortiz-cuaran@lyon.unicancer.fr pierre.saintigny@lyon.unicancer.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200828 PL - United States TA - Clin Cancer Res JT - Clinical cancer research : an official journal of the American Association for Cancer Research JID - 9502500 RN - 0 (Biomarkers, Tumor) RN - 0 (Circulating Tumor DNA) RN - 0 (Protein Kinase Inhibitors) RN - EC 2.7.11.1 (BRAF protein, human) RN - EC 2.7.11.1 (Proto-Oncogene Proteins B-raf) SB - IM MH - Biomarkers, Tumor/genetics MH - Carcinoma, Non-Small-Cell Lung/*drug therapy/genetics/secondary MH - Circulating Tumor DNA/analysis/*genetics MH - *Drug Resistance, Neoplasm MH - Follow-Up Studies MH - Genomics/methods MH - Humans MH - Lung Neoplasms/drug therapy/genetics/pathology MH - Molecular Targeted Therapy/*methods MH - *Mutation MH - Prognosis MH - Prospective Studies MH - Protein Kinase Inhibitors/*pharmacology MH - Proto-Oncogene Proteins B-raf/*genetics MH - Survival Rate EDAT- 2020/08/30 06:00 MHDA- 2021/12/15 06:00 CRDT- 2020/08/30 06:00 PHST- 2020/03/18 00:00 [received] PHST- 2020/06/11 00:00 [revised] PHST- 2020/08/20 00:00 [accepted] PHST- 2020/08/30 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2020/08/30 06:00 [entrez] AID - 1078-0432.CCR-20-1037 [pii] AID - 10.1158/1078-0432.CCR-20-1037 [doi] PST - ppublish SO - Clin Cancer Res. 2020 Dec 1;26(23):6242-6253. doi: 10.1158/1078-0432.CCR-20-1037. Epub 2020 Aug 28. PMID- 35984704 OWN - NLM STAT- MEDLINE DCOM- 20221102 LR - 20250530 IS - 1557-3265 (Electronic) IS - 1078-0432 (Print) IS - 1078-0432 (Linking) VI - 28 IP - 21 DP - 2022 Nov 1 TI - A Phase I Trial of CT900, a Novel α-Folate Receptor-Mediated Thymidylate Synthase Inhibitor, in Patients with Solid Tumors with Expansion Cohorts in Patients with High-Grade Serous Ovarian Cancer. PG - 4634-4641 LID - 10.1158/1078-0432.CCR-22-1268 [doi] AB - PURPOSE: CT900 is a novel small molecule thymidylate synthase inhibitor that binds to α-folate receptor (α-FR) and thus is selectively taken up by α-FR-overexpressing tumors. PATIENTS AND METHODS: A 3+3 dose escalation design was used. During dose escalation, CT900 doses of 1-6 mg/m2 weekly and 2-12 mg/m2 every 2 weeks (q2Wk) intravenously were evaluated. Patients with high-grade serous ovarian cancer were enrolled in the expansion cohorts. RESULTS: 109 patients were enrolled: 42 patients in the dose escalation and 67 patients in the expansion cohorts. At the dose/schedule of 12 mg/m2/q2Wk (with and without dexamethasone, n = 40), the most common treatment-related adverse events were fatigue, nausea, diarrhea, cough, anemia, and pneumonitis, which were predominantly grade 1 and grade 2. Levels of CT900 more than 600 nmol/L needed for growth inhibition in preclinical models were achieved for >65 hours at a dose of 12 mg/m2. In the expansion cohorts, the overall response rate (ORR), was 14/64 (21.9%). Thirty-eight response-evaluable patients in the expansion cohorts receiving 12 mg/m2/q2Wk had tumor evaluable for quantification of α-FR. Patients with high or medium expression had an objective response rate of 9/25 (36%) compared with 1/13 (7.7%) in patients with negative/very low or low expression of α-FR. CONCLUSIONS: The dose of 12 mg/m2/q2Wk was declared the recommended phase II dose/schedule. At this dose/schedule, CT900 exhibited an acceptable side effect profile with clinical benefit in patients with high/medium α-FR expression and warrants further investigation. CI - ©2022 The Authors; Published by the American Association for Cancer Research. FAU - Banerjee, Susana AU - Banerjee S AUID- ORCID: 0000-0002-8840-7934 AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. AD - Gynaecology Unit, The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Michalarea, Vasiliki AU - Michalarea V AUID- ORCID: 0000-0002-3102-3534 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Ang, Joo Ern AU - Ang JE AUID- ORCID: 0000-0003-2103-996X AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Ingles Garces, Alvaro AU - Ingles Garces A AUID- ORCID: 0000-0002-0073-4237 AD - Gynaecology Unit, The Royal Marsden NHS Foundation Trust, London, United Kingdom. AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Biondo, Andrea AU - Biondo A AUID- ORCID: 0000-0003-0599-254X AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Funingana, Ionut-Gabriel AU - Funingana IG AUID- ORCID: 0000-0002-1197-2652 AD - Cambridge University Hospitals NHS Foundation Trust and University of Cambridge, Cambridge, United Kingdom. FAU - Little, Martin AU - Little M AUID- ORCID: 0000-0003-2592-1570 AD - Experimental Cancer Medicine Team, The Christie NHS Foundation Trust, Manchester, United Kingdom. FAU - Ruddle, Ruth AU - Ruddle R AUID- ORCID: 0000-0003-0025-8872 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Raynaud, Florence AU - Raynaud F AUID- ORCID: 0000-0003-0957-6279 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Riisnaes, Ruth AU - Riisnaes R AUID- ORCID: 0000-0002-8924-302X AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Gurel, Bora AU - Gurel B AUID- ORCID: 0000-0002-5018-8078 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Chua, Sue AU - Chua S AUID- ORCID: 0000-0001-5369-8156 AD - Radiology and Nuclear Medicine Department, The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Tunariu, Nina AU - Tunariu N AUID- ORCID: 0000-0001-6656-3699 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. AD - Radiology and Nuclear Medicine Department, The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Porter, Joanna C AU - Porter JC AUID- ORCID: 0000-0002-7307-169X AD - UCL Respiratory, University College London and Interstitial Lung Disease Service, University College London NHS Foundation Trust, London, United Kingdom. FAU - Prout, Toby AU - Prout T AUID- ORCID: 0000-0002-6465-4578 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Parmar, Mona AU - Parmar M AUID- ORCID: 0000-0001-7818-4100 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Zachariou, Anna AU - Zachariou A AUID- ORCID: 0000-0002-7867-8327 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Turner, Alison AU - Turner A AUID- ORCID: 0000-0003-2915-2756 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Jenkins, Ben AU - Jenkins B AUID- ORCID: 0000-0002-2517-3595 AD - Clinical Trials and Statistics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - McIntosh, Stuart AU - McIntosh S AUID- ORCID: 0000-0002-7379-4505 AD - Carrick Therapeutics, Dublin, Ireland. FAU - Ainscow, Ed AU - Ainscow E AUID- ORCID: 0000-0002-3119-8422 AD - Carrick Therapeutics, Dublin, Ireland. FAU - Minchom, Anna AU - Minchom A AUID- ORCID: 0000-0002-9339-7101 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Lopez, Juanita AU - Lopez J AUID- ORCID: 0000-0001-8321-4212 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - de Bono, Johann AU - de Bono J AUID- ORCID: 0000-0002-2034-595X AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Jones, Robert AU - Jones R AUID- ORCID: 0000-0003-3576-9496 AD - Cardiff University, School of Medicine, Velindre University NHS Trust, Cardiff, United Kingdom. FAU - Hall, Emma AU - Hall E AUID- ORCID: 0000-0001-5999-5020 AD - Clinical Trials and Statistics Unit, The Institute of Cancer Research, London, United Kingdom. FAU - Cook, Natalie AU - Cook N AUID- ORCID: 0000-0003-2606-1082 AD - Experimental Cancer Medicine Team, The Christie NHS Foundation Trust, Manchester, United Kingdom. AD - Division of Cancer Sciences, The University of Manchester, Manchester, United Kingdom. FAU - Basu, Bristi AU - Basu B AUID- ORCID: 0000-0002-3562-2868 AD - Cambridge University Hospitals NHS Foundation Trust and University of Cambridge, Cambridge, United Kingdom. FAU - Banerji, Udai AU - Banerji U AUID- ORCID: 0000-0003-1503-3123 AD - Drug Development Unit, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London, United Kingdom. LA - eng SI - ClinicalTrials.gov/NCT02360345 SI - EudraCT/2013-000569-34 GR - 22897/CRUK_/Cancer Research UK/United Kingdom GR - 23819/CRUK_/Cancer Research UK/United Kingdom PT - Clinical Trial, Phase I PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Clin Cancer Res JT - Clinical cancer research : an official journal of the American Association for Cancer Research JID - 9502500 RN - EC 2.1.1.45 (Thymidylate Synthase) RN - 0 (Enzyme Inhibitors) RN - 935E97BOY8 (Folic Acid) SB - IM CIN - 1078-0432. doi: 10.1158/1078-0432.CCR-28-21-HI MH - Humans MH - Female MH - Thymidylate Synthase/genetics MH - Maximum Tolerated Dose MH - *Neoplasms/drug therapy MH - Enzyme Inhibitors/therapeutic use MH - *Ovarian Neoplasms/drug therapy/genetics/pathology MH - Folic Acid PMC - PMC9623233 EDAT- 2022/08/20 06:00 MHDA- 2022/11/03 06:00 PMCR- 2022/11/01 CRDT- 2022/08/19 12:03 PHST- 2022/05/06 00:00 [received] PHST- 2022/07/07 00:00 [revised] PHST- 2022/08/17 00:00 [accepted] PHST- 2022/08/20 06:00 [pubmed] PHST- 2022/11/03 06:00 [medline] PHST- 2022/08/19 12:03 [entrez] PHST- 2022/11/01 00:00 [pmc-release] AID - 708154 [pii] AID - CCR-22-1268 [pii] AID - 10.1158/1078-0432.CCR-22-1268 [doi] PST - ppublish SO - Clin Cancer Res. 2022 Nov 1;28(21):4634-4641. doi: 10.1158/1078-0432.CCR-22-1268. PMID- 27550844 OWN - NLM STAT- MEDLINE DCOM- 20190215 LR - 20190215 IS - 1476-5578 (Electronic) IS - 1359-4184 (Print) IS - 1359-4184 (Linking) VI - 23 IP - 2 DP - 2018 Feb TI - De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females. PG - 222-230 LID - 10.1038/mp.2016.135 [doi] AB - Variants in CLCN4, which encodes the chloride/hydrogen ion exchanger CIC-4 prominently expressed in brain, were recently described to cause X-linked intellectual disability and epilepsy. We present detailed phenotypic information on 52 individuals from 16 families with CLCN4-related disorder: 5 affected females and 2 affected males with a de novo variant in CLCN4 (6 individuals previously unreported) and 27 affected males, 3 affected females and 15 asymptomatic female carriers from 9 families with inherited CLCN4 variants (4 families previously unreported). Intellectual disability ranged from borderline to profound. Behavioral and psychiatric disorders were common in both child- and adulthood, and included autistic features, mood disorders, obsessive-compulsive behaviors and hetero- and autoaggression. Epilepsy was common, with severity ranging from epileptic encephalopathy to well-controlled seizures. Several affected individuals showed white matter changes on cerebral neuroimaging and progressive neurological symptoms, including movement disorders and spasticity. Heterozygous females can be as severely affected as males. The variability of symptoms in females is not correlated with the X inactivation pattern studied in their blood. The mutation spectrum includes frameshift, missense and splice site variants and one single-exon deletion. All missense variants were predicted to affect CLCN4's function based on in silico tools and either segregated with the phenotype in the family or were de novo. Pathogenicity of all previously unreported missense variants was further supported by electrophysiological studies in Xenopus laevis oocytes. We compare CLCN4-related disorder with conditions related to dysfunction of other members of the CLC family. FAU - Palmer, E E AU - Palmer EE AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, Australia. AD - School of Women and Children's Health, University of New South Wales, NSW, Australia. FAU - Stuhlmann, T AU - Stuhlmann T AD - Department Physiology and Pathology of Ion Transport, Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Department Physiology and Pathology of Ion Transport, Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Weinert, S AU - Weinert S AD - Department Physiology and Pathology of Ion Transport, Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Department Physiology and Pathology of Ion Transport, Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Haan, E AU - Haan E AD - School of Medicine, The University of Adelaide, Adelaide, SA, Australia. AD - South Australian Clinical Genetics Service, SA Pathology (at Women's and Children's Hospital), Adelaide, SA, Australia. FAU - Van Esch, H AU - Van Esch H AD - Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium. FAU - Holvoet, M AU - Holvoet M AD - Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium. FAU - Boyle, J AU - Boyle J AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Leffler, M AU - Leffler M AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Raynaud, M AU - Raynaud M AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Moraine, C AU - Moraine C AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - van Bokhoven, H AU - van Bokhoven H AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behavior, Nijmegen, The Netherlands. FAU - Kleefstra, T AU - Kleefstra T AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behavior, Nijmegen, The Netherlands. FAU - Kahrizi, K AU - Kahrizi K AD - Genetics Research Center, University of Social Welfare and Rehabilitation Sciences, Tehran, Iran. FAU - Najmabadi, H AU - Najmabadi H AD - Genetics Research Center, University of Social Welfare and Rehabilitation Sciences, Tehran, Iran. FAU - Ropers, H-H AU - Ropers HH AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Delgado, M R AU - Delgado MR AD - Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX, USA. AD - Texas Scottish Rite Hospital for Children, Dallas, TX, USA. FAU - Sirsi, D AU - Sirsi D AD - Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX, USA. FAU - Golla, S AU - Golla S AD - Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX, USA. FAU - Sommer, A AU - Sommer A AD - Department of Molecular and Human Genetics, Nationwide Children's Hospital, Columbus, OH, USA. FAU - Pietryga, M P AU - Pietryga MP AD - Department of Molecular and Human Genetics, Nationwide Children's Hospital, Columbus, OH, USA. FAU - Chung, W K AU - Chung WK AD - Columbia University, New York, NY, USA. FAU - Wynn, J AU - Wynn J AD - Columbia University, New York, NY, USA. FAU - Rohena, L AU - Rohena L AD - Department of Pediatrics, San Antonio Military Medical Center, Fort Sam, Houston, TX, USA. FAU - Bernardo, E AU - Bernardo E AD - Department of Pediatrics, San Antonio Military Medical Center, Fort Sam, Houston, TX, USA. FAU - Hamlin, D AU - Hamlin D AD - Department of Pediatrics, San Antonio Military Medical Center, Fort Sam, Houston, TX, USA. FAU - Faux, B M AU - Faux BM AD - Department of Pediatrics, San Antonio Military Medical Center, Fort Sam, Houston, TX, USA. FAU - Grange, D K AU - Grange DK AD - Division of Genetics and Genomic Medicine, Department of Pediatrics, Washington University School of Medicine, St Louis Children's Hospital, St Louis, MO, USA. FAU - Manwaring, L AU - Manwaring L AD - Division of Genetics and Genomic Medicine, Department of Pediatrics, Washington University School of Medicine, St Louis Children's Hospital, St Louis, MO, USA. FAU - Tolmie, J AU - Tolmie J AD - West of Scotland Clinical Genetics Service, Southern General Hospital, Glasgow, UK. FAU - Joss, S AU - Joss S AD - West of Scotland Clinical Genetics Service, Southern General Hospital, Glasgow, UK. CN - DDD Study FAU - Cobben, J M AU - Cobben JM AD - Department of Pediatrics, AMC University Hospital Amsterdam, Amsterdam, The Netherlands. FAU - Duijkers, F A M AU - Duijkers FAM AD - Department of Clinical Genetics, AMC University Hospital Amsterdam, Amsterdam, The Netherlands. FAU - Goehringer, J M AU - Goehringer JM AD - Autism and Developmental Medicine Institute, Geisinger Health System, Lewisburg, PA, USA. FAU - Challman, T D AU - Challman TD AD - Autism and Developmental Medicine Institute, Geisinger Health System, Lewisburg, PA, USA. FAU - Hennig, F AU - Hennig F AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Fischer, U AU - Fischer U AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Grimme, A AU - Grimme A AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Suckow, V AU - Suckow V AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Musante, L AU - Musante L AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Nicholl, J AU - Nicholl J AD - SA Pathology, Women's and Children's Hospital, North Adelaide, SA, Australia. FAU - Shaw, M AU - Shaw M AD - School of Medicine, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Lodh, S P AU - Lodh SP AD - School of Women and Children's Health, University of New South Wales, NSW, Australia. FAU - Niu, Z AU - Niu Z AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA. FAU - Rosenfeld, J A AU - Rosenfeld JA AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA. FAU - Stankiewicz, P AU - Stankiewicz P AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA. FAU - Jentsch, T J AU - Jentsch TJ AD - Department Physiology and Pathology of Ion Transport, Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Department Physiology and Pathology of Ion Transport, Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Gecz, J AU - Gecz J AD - School of Medicine, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Field, M AU - Field M AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Kalscheuer, V M AU - Kalscheuer VM AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, Germany. LA - eng GR - Wellcome Trust/United Kingdom GR - R01 HD057036/HD/NICHD NIH HHS/United States GR - UL1 RR024156/RR/NCRR NIH HHS/United States GR - WT098051/Wellcome Trust/United Kingdom GR - Department of Health/United Kingdom PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20160823 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (CLCN4 protein, human) RN - 0 (Chloride Channels) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Animals MH - Child MH - Child, Preschool MH - Chloride Channels/*genetics/metabolism MH - Epilepsy/genetics MH - Epileptic Syndromes/*genetics/physiopathology MH - Family MH - Female MH - Genes, X-Linked MH - Genetic Diseases, X-Linked/genetics MH - Germ-Line Mutation MH - Humans MH - Intellectual Disability/*genetics/metabolism MH - Male MH - Middle Aged MH - Mutation MH - Oocytes MH - Pedigree MH - Phenotype MH - Syndrome MH - White Matter/physiopathology MH - Xenopus laevis PMC - PMC5794876 COIS- WK Chung is a consultant for BioReference Laboratories. The remaining authors declare no conflict of interest. EDAT- 2016/08/24 06:00 MHDA- 2019/02/16 06:00 PMCR- 2018/02/02 CRDT- 2016/08/24 06:00 PHST- 2016/02/08 00:00 [received] PHST- 2016/05/14 00:00 [revised] PHST- 2016/06/20 00:00 [accepted] PHST- 2016/08/24 06:00 [pubmed] PHST- 2019/02/16 06:00 [medline] PHST- 2016/08/24 06:00 [entrez] PHST- 2018/02/02 00:00 [pmc-release] AID - mp2016135 [pii] AID - 10.1038/mp.2016.135 [doi] PST - ppublish SO - Mol Psychiatry. 2018 Feb;23(2):222-230. doi: 10.1038/mp.2016.135. Epub 2016 Aug 23. PMID- 19082699 OWN - NLM STAT- MEDLINE DCOM- 20090511 LR - 20260518 IS - 1573-7241 (Electronic) IS - 0920-3206 (Linking) VI - 23 IP - 2 DP - 2009 Apr TI - The rationale and design of the PERindopril GENEtic association study (PERGENE): a pharmacogenetic analysis of angiotensin-converting enzyme inhibitor therapy in patients with stable coronary artery disease. PG - 171-81 LID - 10.1007/s10557-008-6156-1 [doi] AB - BACKGROUND: Angiotensin-converting enzyme (ACE) inhibitors reduce clinical symptoms and improve outcome in patients with hypertension, heart failure, and stable coronary artery disease (CAD) and are among the most frequently used drugs in these patient groups. For hypertension, treatment is guided by the level of blood pressure. In the secondary prevention setting, there are no means of guiding therapy. Prior attempts to target ACE-inhibitors to those patients that are most likely to benefit have not been successful, mainly due to the consistency in the treatment effect in clinical subgroups. Still, for prolonged prophylactic treatment with ACE-inhibitors it would be best to target treatment to only those patients most likely to benefit, which would considerably lower the number needed to treat and increase cost-effectiveness. A new approach for such "tailored-therapy" may be to integrate information on the genetic variation between patients. Until now, pharmacogenetic research of the efficacy of ACE-inhibitor therapy in CAD patients is still in a preliminary stage. METHODS: The PERindopril GENEtic association study (PERGENE) is a substudy of the EUROPA trial, a randomized double-blind placebo-controlled multicentre clinical trial which demonstrated a beneficial effect of the ACE-inhibitor perindopril in reducing cardiovascular morbidity and mortality in 12.218 patients with stable coronary artery disease (mean follow-up 4.2 years). Blood tubes were received from patients at the beginning of the EUROPA trial and buffy coats were stored at -40 degrees C at the central core laboratory. Candidate genes were selected in the renin-angiotensin-system and bradykinin pathways. Polymorphisms were selected based on haplotype tagging principles using the HapMap genome project, Seattle and other up-to-date genetic database platforms to comprehensively cover all common genetic variation within the genes. Selection also took into consideration the functionality of SNP's, location within the gene (promoter) and existing relevant literature. The main outcome measure of PERGENE is the effect of genetic factors on the treatment benefit with ACE-inhibitors. The size of this pharmacogenetic substudy allows detection with a statistical power of 98% to detect a difference in hazard ratios (treatment effect) of 20% between genotypes with minor allele frequency of 0.20 (two-sided alpha 0.05). CONCLUSION: The PERGENE study is a large cardiovascular pharmacogenetic study aimed to assess the feasibility of pharmacogenetic profiling of the treatment effect of ACE-inhibitor use with the perspective to individualize treatment in patients with stable coronary artery disease. FAU - Brugts, J J AU - Brugts JJ AD - Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands. j.brugts@erasmusmc.nl FAU - de Maat, M P M AU - de Maat MP FAU - Boersma, E AU - Boersma E FAU - Witteman, J C M AU - Witteman JC FAU - van Duijn, C AU - van Duijn C FAU - Uitterlinden, A G AU - Uitterlinden AG FAU - Bertrand, M AU - Bertrand M FAU - Remme, W AU - Remme W FAU - Fox, K AU - Fox K FAU - Ferrari, R AU - Ferrari R FAU - Danser, A H J AU - Danser AH FAU - Simoons, M L AU - Simoons ML CN - EUROPA-PERGENE investigators LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20081210 PL - United States TA - Cardiovasc Drugs Ther JT - Cardiovascular drugs and therapy JID - 8712220 RN - 0 (Angiotensin-Converting Enzyme Inhibitors) RN - Y5GMK36KGY (Perindopril) SB - IM MH - Angiotensin-Converting Enzyme Inhibitors/*pharmacology MH - Cardiovascular Diseases/mortality/prevention & control MH - Coronary Artery Disease/*drug therapy/genetics MH - Double-Blind Method MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Perindopril/*pharmacology MH - *Pharmacogenetics MH - Polymorphism, Single Nucleotide MH - Randomized Controlled Trials as Topic MH - Research Design FIR - Brugts, J J IR - Brugts JJ FIR - de Maat, M P M IR - de Maat MP FIR - Boersma, E IR - Boersma E FIR - Witteman, J C M IR - Witteman JC FIR - van Duijn, C IR - van Duijn C FIR - Uitterlinden, A G IR - Uitterlinden AG FIR - Danser, A H J IR - Danser AH FIR - Simoons, M L IR - Simoons ML FIR - Bertrand, M IR - Bertrand M FIR - Ferrari, R IR - Ferrari R FIR - Fox, K IR - Fox K FIR - Remme, W J IR - Remme WJ FIR - Simoons, M L IR - Simoons ML FIR - Aldershville, J IR - Aldershville J FIR - Hildebrandt, P IR - Hildebrandt P FIR - Bassand, J P IR - Bassand JP FIR - Cokkinos, D IR - Cokkinos D FIR - Toutouzas, P IR - Toutouzas P FIR - Eha, J IR - Eha J FIR - Erhardt, L IR - Erhardt L FIR - Erikssen, J IR - Erikssen J FIR - Grybauskas, R IR - Grybauskas R FIR - Kalnins, U IR - Kalnins U FIR - Karsch, K IR - Karsch K FIR - Sechtem, U IR - Sechtem U FIR - Keltai, M IR - Keltai M FIR - Klein, W IR - Klein W FIR - Luescher, T IR - Luescher T FIR - Mulcahy, D IR - Mulcahy D FIR - Nieminen, M IR - Nieminen M FIR - Oto, A IR - Oto A FIR - Ozsaruhan, O IR - Ozsaruhan O FIR - Paulus, W IR - Paulus W FIR - Providencia, L IR - Providencia L FIR - Riecansky, I IR - Riecansky I FIR - Ruzyllo, W IR - Ruzyllo W FIR - Santini, U IR - Santini U FIR - Tavazzi, L IR - Tavazzi L FIR - Soler-Soler, J IR - Soler-Soler J FIR - Widimsky, P IR - Widimsky P FIR - Julian, D IR - Julian D FIR - Dargie, H IR - Dargie H FIR - Kobler, W IR - Kobler W FIR - Duprez, D IR - Duprez D FIR - Steg, G IR - Steg G FIR - Thygesen, K IR - Thygesen K FIR - Drexel, H IR - Drexel H FIR - Gombotz, G IR - Gombotz G FIR - Klein, W IR - Klein W FIR - Stoeckl, G IR - Stoeckl G FIR - Duprez, D IR - Duprez D FIR - Heyndrickx, G H IR - Heyndrickx GH FIR - Legrand, V IR - Legrand V FIR - Materne, P IR - Materne P FIR - Van Mieghem, W IR - Van Mieghem W FIR - Bocek, P IR - Bocek P FIR - Branny, M IR - Branny M FIR - Cech, M IR - Cech M FIR - Charouzek, J IR - Charouzek J FIR - Drazka, J IR - Drazka J FIR - Fabik, L IR - Fabik L FIR - Florian, J IR - Florian J FIR - Francek, L IR - Francek L FIR - Groch, L IR - Groch L FIR - Havranek, P IR - Havranek P FIR - Herman, A IR - Herman A FIR - Hradec, J IR - Hradec J FIR - Jansky, P IR - Jansky P FIR - Jirmar, R IR - Jirmar R FIR - Jokl, I IR - Jokl I FIR - Krejcova, H IR - Krejcova H FIR - Kvasnak, M IR - Kvasnak M FIR - Maratka, T IR - Maratka T FIR - Marcinek, G IR - Marcinek G FIR - Moravcova, J IR - Moravcova J FIR - Nedbal, P IR - Nedbal P FIR - Peterka, K IR - Peterka K FIR - Povolny, J IR - Povolny J FIR - Rosolova, H IR - Rosolova H FIR - Semrad, B IR - Semrad B FIR - Sochor, K IR - Sochor K FIR - Spacek, R IR - Spacek R FIR - Spinar, J IR - Spinar J FIR - Stipal, R IR - Stipal R FIR - Stuchlik, K IR - Stuchlik K FIR - Sulda, M IR - Sulda M FIR - Ulman, J IR - Ulman J FIR - Vaclavicek, A IR - Vaclavicek A FIR - Vojtisek, P IR - Vojtisek P FIR - Bjerregard Andersen, H IR - Bjerregard Andersen H FIR - Dorff, B IR - Dorff B FIR - Hildebrandt, P IR - Hildebrandt P FIR - Kristensen, K IR - Kristensen K FIR - Madsen, J K IR - Madsen JK FIR - Markenvard, J IR - Markenvard J FIR - Meibom, J IR - Meibom J FIR - Norgaard, A IR - Norgaard A FIR - Scheibel, M IR - Scheibel M FIR - Eha, J IR - Eha J FIR - Leht, A IR - Leht A FIR - Teesalu, R IR - Teesalu R FIR - Vahulaa, V IR - Vahulaa V FIR - Itkonen, A IR - Itkonen A FIR - Juvonen, J IR - Juvonen J FIR - Karmakoski, J IR - Karmakoski J FIR - Kilkki, E IR - Kilkki E FIR - Koskela, E IR - Koskela E FIR - Kotila, M J IR - Kotila MJ FIR - Melin, J IR - Melin J FIR - Nieminen, M S IR - Nieminen MS FIR - Savola, R IR - Savola R FIR - Terho, T IR - Terho T FIR - Voipio Pulkki, L M IR - Voipio Pulkki LM FIR - Apffel, F IR - Apffel F FIR - Attali, P IR - Attali P FIR - Baron, B IR - Baron B FIR - Bassand, J P IR - Bassand JP FIR - Berthier, Y IR - Berthier Y FIR - Bertrand, M IR - Bertrand M FIR - Dambrine, P IR - Dambrine P FIR - Danchin, N IR - Danchin N FIR - Decoulx, E IR - Decoulx E FIR - Deshayes, P IR - Deshayes P FIR - Fouche, R IR - Fouche R FIR - Genest, M IR - Genest M FIR - Godard, S IR - Godard S FIR - Guillot, J P IR - Guillot JP FIR - Hanania, G IR - Hanania G FIR - Lelguen, C IR - Lelguen C FIR - Leroy, F IR - Leroy F FIR - Mansourati, J IR - Mansourati J FIR - Mery, D IR - Mery D FIR - Michel IR - Michel FIR - Quiret, J C IR - Quiret JC FIR - Raynaud, P IR - Raynaud P FIR - Rondepierre, D IR - Rondepierre D FIR - Roynard, J L IR - Roynard JL FIR - Van Belle, E IR - Van Belle E FIR - Veyrat, A IR - Veyrat A FIR - Gaudron, P IR - Gaudron P FIR - Karsch, K R IR - Karsch KR FIR - Lauer, B IR - Lauer B FIR - Rettig Stsrmer, G IR - Rettig Stsrmer G FIR - Riessen, R IR - Riessen R FIR - Rutsch, W IR - Rutsch W FIR - Sechtem, U IR - Sechtem U FIR - Sigel, H A IR - Sigel HA FIR - Simon, R IR - Simon R FIR - Stork, S IR - Stork S FIR - Von Schacky, C IR - Von Schacky C FIR - Winkelmann, B R IR - Winkelmann BR FIR - Christakos, S IR - Christakos S FIR - Cokkinos, D IR - Cokkinos D FIR - Feggos, S IR - Feggos S FIR - Geleris, P IR - Geleris P FIR - Georgiadis, S IR - Georgiadis S FIR - Gialafos, J IR - Gialafos J FIR - Goudevenos, I IR - Goudevenos I FIR - Kardara, D IR - Kardara D FIR - Kardaras, F IR - Kardaras F FIR - Karidis, C IR - Karidis C FIR - Kelesides, C IR - Kelesides C FIR - Kyriakidis, M IR - Kyriakidis M FIR - Koliopoulos, N IR - Koliopoulos N FIR - Kremastinos, D IR - Kremastinos D FIR - Liberi, S IR - Liberi S FIR - Manolis, A N IR - Manolis AN FIR - Pyrgakis, V IR - Pyrgakis V FIR - Papasteriadis, E IR - Papasteriadis E FIR - Papazoglou, N IR - Papazoglou N FIR - Skoufas, P IR - Skoufas P FIR - Stamatelopoulos, S IR - Stamatelopoulos S FIR - Stambola, S IR - Stambola S FIR - Stavridis, A IR - Stavridis A FIR - Syribeis, S IR - Syribeis S FIR - Vardas, P IR - Vardas P FIR - Vassiliadis, I IR - Vassiliadis I FIR - Voudris, V IR - Voudris V FIR - Zacharoulis, A IR - Zacharoulis A FIR - Zobolos, S IR - Zobolos S FIR - Zouras, C IR - Zouras C FIR - Berenyi, I IR - Berenyi I FIR - Bocsa, Z IR - Bocsa Z FIR - Csendes, E IR - Csendes E FIR - Edes, I IR - Edes I FIR - Gelesz, E IR - Gelesz E FIR - Janosi, A IR - Janosi A FIR - Kalo, E IR - Kalo E FIR - Karpati, P IR - Karpati P FIR - Kornel, S IR - Kornel S FIR - Pap, I IR - Pap I FIR - Pinter, I IR - Pinter I FIR - Polak, G IR - Polak G FIR - Reiber, I IR - Reiber I FIR - Rusznak, M IR - Rusznak M FIR - Simon, A IR - Simon A FIR - Tarjan, J IR - Tarjan J FIR - Tihanyi, L IR - Tihanyi L FIR - Timar, S IR - Timar S FIR - Toth, K IR - Toth K FIR - Veress, G IR - Veress G FIR - Barton, J IR - Barton J FIR - Crean, P IR - Crean P FIR - Daly, K IR - Daly K FIR - Kearney, P IR - Kearney P FIR - Meany, T B IR - Meany TB FIR - Mulcahy, D IR - Mulcahy D FIR - Quigley, P IR - Quigley P FIR - Azzolini, P IR - Azzolini P FIR - Barone, G IR - Barone G FIR - Barsotti, A IR - Barsotti A FIR - Bellone, E IR - Bellone E FIR - Borghetti, A IR - Borghetti A FIR - Branzi, A IR - Branzi A FIR - Brunelli, C IR - Brunelli C FIR - Capponi, E IR - Capponi E FIR - Capucci, A IR - Capucci A FIR - Casaccia, M IR - Casaccia M FIR - Casali, G IR - Casali G FIR - Cecchetti, E IR - Cecchetti E FIR - Ceci, V IR - Ceci V FIR - Celegon, L IR - Celegon L FIR - Chimini, C IR - Chimini C FIR - Colombo, A IR - Colombo A FIR - Corsini, G IR - Corsini G FIR - Cucchini, F IR - Cucchini F FIR - Dalla Volta, S IR - Dalla Volta S FIR - De Luca, I IR - De Luca I FIR - De Servi, S IR - De Servi S FIR - Delise, P IR - Delise P FIR - Di Donato, M IR - Di Donato M FIR - Di Giacomo, U IR - Di Giacomo U FIR - Di Pasquale, G IR - Di Pasquale G FIR - Fiorentini, C IR - Fiorentini C FIR - Gaddi, O IR - Gaddi O FIR - Giannetto, M IR - Giannetto M FIR - Giannuzzi, P IR - Giannuzzi P FIR - Giordano, A IR - Giordano A FIR - Giovannini, E IR - Giovannini E FIR - Iacono, A IR - Iacono A FIR - Inama, G IR - Inama G FIR - Ippoliti, G IR - Ippoliti G FIR - Leghissa, R IR - Leghissa R FIR - Lorusso, R IR - Lorusso R FIR - Marzilli, M IR - Marzilli M FIR - Minutiello, L IR - Minutiello L FIR - Moretti, G IR - Moretti G FIR - Mosele, G M IR - Mosele GM FIR - Pasotti, C IR - Pasotti C FIR - Pettinati, G IR - Pettinati G FIR - Pezzano, A IR - Pezzano A FIR - Polimeni, M R IR - Polimeni MR FIR - Portaluppi, F IR - Portaluppi F FIR - Proto, C IR - Proto C FIR - Riva, S IR - Riva S FIR - Sanguinetti, M IR - Sanguinetti M FIR - Santini, M IR - Santini M FIR - Severi, S IR - Severi S FIR - Sinagra, G IR - Sinagra G FIR - Tantalo, L IR - Tantalo L FIR - Tavazzi, L IR - Tavazzi L FIR - Vajola, S F IR - Vajola SF FIR - Vincenzi, M IR - Vincenzi M FIR - Volterrani, M IR - Volterrani M FIR - Zavatteri, G IR - Zavatteri G FIR - Zogno, M IR - Zogno M FIR - Gailiss, E IR - Gailiss E FIR - Gersamija, A IR - Gersamija A FIR - Kalnins, U IR - Kalnins U FIR - Ozolina, M A IR - Ozolina MA FIR - Skards, J IR - Skards J FIR - Baubiniene, A IR - Baubiniene A FIR - Berukstis, E IR - Berukstis E FIR - Grigoniene, L IR - Grigoniene L FIR - Grybauskas IR - Grybauskas FIR - Kibarskis, A IR - Kibarskis A FIR - Kirkutis, A IR - Kirkutis A FIR - Marcinkus, R IR - Marcinkus R FIR - Milvidaite, I IR - Milvidaite I FIR - Vasiliauskas, D IR - Vasiliauskas D FIR - Aalders, J C A IR - Aalders JC FIR - Bruggeling, W A J IR - Bruggeling WA FIR - Bucx, J J J IR - Bucx JJ FIR - De Feyter, P J IR - De Feyter PJ FIR - De Leeuw, M J IR - De Leeuw MJ FIR - De Waard, D E P IR - De Waard DE FIR - De Weerd, G J IR - De Weerd GJ FIR - De Zwaan, C IR - De Zwaan C FIR - Dijkgraaf, R IR - Dijkgraaf R FIR - Droste, H T IR - Droste HT FIR - Freericks, M P IR - Freericks MP FIR - Hagoort Kok, A W IR - Hagoort Kok AW FIR - Jap, W T J IR - Jap WT FIR - Jochemsen, G M IR - Jochemsen GM FIR - Kiemeney, K IR - Kiemeney K FIR - Kuijer, P J P IR - Kuijer PJ FIR - Mannaerts, H F J IR - Mannaerts HF FIR - Piek, J J IR - Piek JJ FIR - Saelman, J P M IR - Saelman JP FIR - Simoons, M L IR - Simoons ML FIR - Slob, F D IR - Slob FD FIR - Smits, W C G IR - Smits WC FIR - Spierenburg, H A M IR - Spierenburg HA FIR - Suttorp, M J IR - Suttorp MJ FIR - Tan, T B IR - Tan TB FIR - Van Beek, G J IR - Van Beek GJ FIR - Van Daele, M E R M IR - Van Daele ME FIR - Van Den Merkhof, L F M IR - Van Den Merkhof LF FIR - Van Den Toren, E W IR - Van Den Toren EW FIR - Van Hessen, M W J IR - Van Hessen MW FIR - Van Langeveld, R A M IR - Van Langeveld RA FIR - Van Loo, L W H IR - Van Loo LW FIR - Van Nierop, P R IR - Van Nierop PR FIR - Van Rey, F J W IR - Van Rey FJ FIR - Van Straalen, M J IR - Van Straalen MJ FIR - Vos, J IR - Vos J FIR - Werner, H A IR - Werner HA FIR - Westendorp, J J C IR - Westendorp JJ FIR - Zwiers, G IR - Zwiers G FIR - Erikssen, J IR - Erikssen J FIR - Achremczyk, P IR - Achremczyk P FIR - Adamus, J IR - Adamus J FIR - Baska, J IR - Baska J FIR - Bolinska Soltysiak, H IR - Bolinska Soltysiak H FIR - Bubinski, R IR - Bubinski R FIR - Ceremuzynski, L IR - Ceremuzynski L FIR - Cieslinski, A IR - Cieslinski A FIR - Dariusz, D IR - Dariusz D FIR - Deptulski, T IR - Deptulski T FIR - Drewla, P IR - Drewla P FIR - Drozdowski, P IR - Drozdowski P FIR - Dubiel, J S IR - Dubiel JS FIR - Dudek, D IR - Dudek D FIR - Galewicz, M IR - Galewicz M FIR - Ghlebus, K IR - Ghlebus K FIR - Halawa, B IR - Halawa B FIR - Jakubowska Majnigier, M IR - Jakubowska Majnigier M FIR - Janion, M IR - Janion M FIR - Jaworska, K IR - Jaworska K FIR - Kaszewska, I IR - Kaszewska I FIR - Kleinrok, A IR - Kleinrok A FIR - Kornacewicz Jach, Z IR - Kornacewicz Jach Z FIR - Krawczyk, W IR - Krawczyk W FIR - Krynicki, R IR - Krynicki R FIR - Krzciuk, M IR - Krzciuk M FIR - Krzeminska Pakula, M IR - Krzeminska Pakula M FIR - Kuch, J IR - Kuch J FIR - Kuzniar, J IR - Kuzniar J FIR - Legutko, J IR - Legutko J FIR - Liszewska Pfejfer, D IR - Liszewska Pfejfer D FIR - Loboz Grudzien, K IR - Loboz Grudzien K FIR - Musial, W IR - Musial W FIR - Opolski, G IR - Opolski G FIR - Pasyk, S IR - Pasyk S FIR - Piwowarska, W IR - Piwowarska W FIR - Pulkowski, G IR - Pulkowski G FIR - Radziszewski, B IR - Radziszewski B FIR - Romanski, B IR - Romanski B FIR - Ruzyllo, W IR - Ruzyllo W FIR - Rynkiewicz, A IR - Rynkiewicz A FIR - Skura, M IR - Skura M FIR - Slowinski, S IR - Slowinski S FIR - Smielak Korombel, W IR - Smielak Korombel W FIR - Targonski, R IR - Targonski R FIR - Templin, W IR - Templin W FIR - Tendera, M IR - Tendera M FIR - Tracz, W IR - Tracz W FIR - Trusz Gluza, M IR - Trusz Gluza M FIR - Turek, P IR - Turek P FIR - Tyminska Sedek, K IR - Tyminska Sedek K FIR - Wodniecki, J IR - Wodniecki J FIR - Zalewski, M IR - Zalewski M FIR - Zinka, E IR - Zinka E FIR - Carrageta, M IR - Carrageta M FIR - Coelho, J IR - Coelho J FIR - Ferreira Gil, R IR - Ferreira Gil R FIR - Leitao Marques, A IR - Leitao Marques A FIR - Santos Andrade, C M IR - Santos Andrade CM FIR - Seabra Gomes, R IR - Seabra Gomes R FIR - Bada, V IR - Bada V FIR - Belicova, M IR - Belicova M FIR - Buksarova, E IR - Buksarova E FIR - Dukat, A IR - Dukat A FIR - Gonsorcik, J IR - Gonsorcik J FIR - Jonas, P IR - Jonas P FIR - Kamensky, G IR - Kamensky G FIR - Karvaj, M IR - Karvaj M FIR - Micko, K IR - Micko K FIR - Mikes, Z IR - Mikes Z FIR - Palinsky, M IR - Palinsky M FIR - Pella, D IR - Pella D FIR - Renker, B IR - Renker B FIR - Riecansky, I IR - Riecansky I FIR - Sefara, P IR - Sefara P FIR - Sojka, G IR - Sojka G FIR - Sulej, P IR - Sulej P FIR - Szakacs, M IR - Szakacs M FIR - Szentivanyi, M IR - Szentivanyi M FIR - Aguirre Salcedo, J M IR - Aguirre Salcedo JM FIR - Alonso Orcajo, N IR - Alonso Orcajo N FIR - Ancillo Garcia, P IR - Ancillo Garcia P FIR - Auge Sanpera, J M IR - Auge Sanpera JM FIR - Ayuela Azcarate, J IR - Ayuela Azcarate J FIR - Bardaji Mayor, J L IR - Bardaji Mayor JL FIR - Bertomeu Martinez, V IR - Bertomeu Martinez V FIR - Blanco Coronado, J L IR - Blanco Coronado JL FIR - Bros Caimari, R IR - Bros Caimari R FIR - Bruguera Cortada, J IR - Bruguera Cortada J FIR - Caparros Valderrama, J IR - Caparros Valderrama J FIR - DeArmas Trujillo, D IR - DeArmas Trujillo D FIR - Del Rio Ligorit, A IR - Del Rio Ligorit A FIR - Espinosa Caliani, J S IR - Espinosa Caliani JS FIR - Fernandez Aviles, F IR - Fernandez Aviles F FIR - Garcia Guerrero, J J IR - Garcia Guerrero JJ FIR - Garcia Lopez, D IR - Garcia Lopez D FIR - Gonzalez Cocina, E IR - Gonzalez Cocina E FIR - Guallar Urena, C IR - Guallar Urena C FIR - Jodar Lorente, L IR - Jodar Lorente L FIR - Lopez Garcia, Aranda V IR - Lopez Garcia AV FIR - MacayaDeMiguel, C IR - MacayaDeMiguel C FIR - Maroto Montero, J IR - Maroto Montero J FIR - Martinez Romero, P IR - Martinez Romero P FIR - Navarro Lopez, F IR - Navarro Lopez F FIR - Noriega Peiro, F IR - Noriega Peiro F FIR - Olague De Ros, J IR - Olague De Ros J FIR - Orellana Mas, J IR - Orellana Mas J FIR - Paz Bermejo, M A IR - Paz Bermejo MA FIR - Placer Peralta, L J IR - Placer Peralta LJ FIR - Rodriguez Padial, L IR - Rodriguez Padial L FIR - Salvador Sanz, A IR - Salvador Sanz A FIR - Segui Bonnin, J IR - Segui Bonnin J FIR - Simarro Martin, E IR - Simarro Martin E FIR - Sobrino Daza, J IR - Sobrino Daza J FIR - Valles Belsue, F IR - Valles Belsue F FIR - Ekdahl, S IR - Ekdahl S FIR - Erhardt, L IR - Erhardt L FIR - Forslund, L IR - Forslund L FIR - Ohlin, H IR - Ohlin H FIR - Persson, S IR - Persson S FIR - Pieper, M IR - Pieper M FIR - Moccetti, T IR - Moccetti T FIR - Acartsrk, E IR - Acartsrk E FIR - Guzelsoy, D IR - Guzelsoy D FIR - Oto, A IR - Oto A FIR - TMZsaruhan, O IR - TMZsaruhan O FIR - Ozturk, M IR - Ozturk M FIR - Sansoy, V IR - Sansoy V FIR - Turkoglu, C IR - Turkoglu C FIR - Ysksel, H IR - Ysksel H FIR - Adgey, A A J IR - Adgey AA FIR - Ahsan, A IR - Ahsan A FIR - Al Khafaji, M IR - Al Khafaji M FIR - Ball, S G IR - Ball SG FIR - Birkhead, J IR - Birkhead J FIR - Boon, N IR - Boon N FIR - Bowker, T IR - Bowker T FIR - Brack, M IR - Brack M FIR - Bridges, A IR - Bridges A FIR - Buchalter, M IR - Buchalter M FIR - Buchalter, B IR - Buchalter B FIR - Calder, B IR - Calder B FIR - Cooke, R A IR - Cooke RA FIR - Corr, L IR - Corr L FIR - Cowell, R IR - Cowell R FIR - Curzen, N P IR - Curzen NP FIR - Davidson, C IR - Davidson C FIR - Davies, E IR - Davies E FIR - Davies, J IR - Davies J FIR - De Belder, M IR - De Belder M FIR - Dhiya, L IR - Dhiya L FIR - Doig, J C IR - Doig JC FIR - Findlay, I N IR - Findlay IN FIR - Fox, K IR - Fox K FIR - Francis, C M IR - Francis CM FIR - Glancy, J M IR - Glancy JM FIR - Glen, S IR - Glen S FIR - Greenwood, T W IR - Greenwood TW FIR - Groves, P IR - Groves P FIR - Hall, A S IR - Hall AS FIR - Hamilton, G IR - Hamilton G FIR - Hillman, R IR - Hillman R FIR - Holdright, D IR - Holdright D FIR - Hubbard, W IR - Hubbard W FIR - Travill, C IR - Travill C FIR - Hutton, I IR - Hutton I FIR - Ilsley, C IR - Ilsley C FIR - Innes, M IR - Innes M FIR - James, M IR - James M FIR - Jennings, K IR - Jennings K FIR - Jones, C J H IR - Jones CJ FIR - Joy, M IR - Joy M FIR - Keeling, P IR - Keeling P FIR - Kooner, J IR - Kooner J FIR - Lawson, C IR - Lawson C FIR - Levy, R D IR - Levy RD FIR - Lip, G IR - Lip G FIR - Lorimer, A R IR - Lorimer AR FIR - Marshall, H IR - Marshall H FIR - Mclachlan, B IR - Mclachlan B FIR - Montgomery, H IR - Montgomery H FIR - Morley, C IR - Morley C FIR - Murdoch, D L IR - Murdoch DL FIR - Muthusamy, R IR - Muthusamy R FIR - Oakley, G D IR - Oakley GD FIR - Penny, W IR - Penny W FIR - Pohl, J E F IR - Pohl JE FIR - Purvis, J IR - Purvis J FIR - Pye, M IR - Pye M FIR - Ramsdale, D IR - Ramsdale D FIR - Reid, D IR - Reid D FIR - Roberts, D H IR - Roberts DH FIR - Rowlands, D IR - Rowlands D FIR - Rozkovec, A IR - Rozkovec A FIR - Saltissi, S IR - Saltissi S FIR - Schofield, P M IR - Schofield PM FIR - Scott, M IR - Scott M FIR - Shapiro, L M IR - Shapiro LM FIR - Silke, B IR - Silke B FIR - Stephens, J IR - Stephens J FIR - Sutherland, S IR - Sutherland S FIR - Swan, J W IR - Swan JW FIR - Shakespeare, C IR - Shakespeare C FIR - Tildesley, G IR - Tildesley G FIR - Travill, C IR - Travill C FIR - Watson, R D S IR - Watson RD FIR - Wilkinson, P IR - Wilkinson P EDAT- 2008/12/17 09:00 MHDA- 2009/05/12 09:00 CRDT- 2008/12/17 09:00 PHST- 2008/10/13 00:00 [received] PHST- 2008/11/10 00:00 [accepted] PHST- 2008/12/17 09:00 [entrez] PHST- 2008/12/17 09:00 [pubmed] PHST- 2009/05/12 09:00 [medline] AID - 10.1007/s10557-008-6156-1 [doi] PST - ppublish SO - Cardiovasc Drugs Ther. 2009 Apr;23(2):171-81. doi: 10.1007/s10557-008-6156-1. Epub 2008 Dec 10. PMID- 23918606 OWN - NLM STAT- MEDLINE DCOM- 20140609 LR - 20260128 IS - 1557-3265 (Electronic) IS - 1078-0432 (Print) IS - 1078-0432 (Linking) VI - 19 IP - 21 DP - 2013 Nov 1 TI - Dual blockade of the PI3K/AKT/mTOR (AZD8055) and RAS/MEK/ERK (AZD6244) pathways synergistically inhibits rhabdomyosarcoma cell growth in vitro and in vivo. PG - 5940-51 LID - 10.1158/1078-0432.CCR-13-0850 [doi] AB - PURPOSE: To provide rationale for using phosphoinositide 3-kinase (PI3K) and/or mitogen-activated protein kinase (MAPK) pathway inhibitors to treat rhabdomyosarcomas, a major cause of pediatric and adolescent cancer deaths. EXPERIMENTAL DESIGN: The prevalence of PI3K/MAPK pathway activation in rhabdomyosarcoma clinical samples was assessed using immunohistochemistry. Compensatory signaling and cross-talk between PI3K/MAPK pathways was determined in rhabdomyosarcoma cell lines following p110α short hairpin RNA-mediated depletion. Pharmacologic inhibition of reprogrammed signaling in stable p110α knockdown lines was used to determine the target-inhibition profile inducing maximal growth inhibition. The in vitro and in vivo efficacy of inhibitors of TORC1/2 (AZD8055), MEK (AZD6244), and P13K/mTOR (NVP-BEZ235) was evaluated alone and in pairwise combinations. RESULTS: PI3K pathway activation was seen in 82.5% rhabdomyosarcomas with coactivated MAPK in 36% and 46% of alveolar and embryonal subtypes, respectively. p110α knockdown in cell lines over the short and long term was associated with compensatory expression of other p110 isoforms, activation of the MAPK pathway, and cross-talk to reactivate the PI3K pathway. Combinations of PI3K pathway and MAP-ERK kinase (MEK) inhibitors synergistically inhibited cell growth in vitro. Treatment of RD cells with AZD8055 plus AZD6244 blocked reciprocal pathway activation, as evidenced by reduced AKT/ERK/S6 phosphorylation. In vivo, the synergistic effect on growth and changes in pharmacodynamic biomarkers was recapitulated using the AZD8055/AZD6244 combination but not NVP-BEZ235/AZD6244. Pharmacokinetic analysis provided evidence of drug-drug interaction with both combinations. CONCLUSIONS: Dual PI3K/MAPK pathway activation and compensatory signaling in both rhabdomyosarcoma subtypes predict a lack of clinical efficacy for single agents targeting either pathway, supporting a therapeutic strategy combining a TORC1/2 with a MEK inhibitor. FAU - Renshaw, Jane AU - Renshaw J AD - Authors' Affiliations: Divisions of Clinical Studies, Cancer Therapeutics, and Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey; Histopathology Department, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom; and Department of Neuropathology, University of Bonn, Bonn, Germany. FAU - Taylor, Kathryn R AU - Taylor KR FAU - Bishop, Ryan AU - Bishop R FAU - Valenti, Melanie AU - Valenti M FAU - De Haven Brandon, Alexis AU - De Haven Brandon A FAU - Gowan, Sharon AU - Gowan S FAU - Eccles, Suzanne A AU - Eccles SA FAU - Ruddle, Ruth R AU - Ruddle RR FAU - Johnson, Louise D AU - Johnson LD FAU - Raynaud, Florence I AU - Raynaud FI FAU - Selfe, Joanna L AU - Selfe JL FAU - Thway, Khin AU - Thway K FAU - Pietsch, Torsten AU - Pietsch T FAU - Pearson, Andrew D AU - Pearson AD FAU - Shipley, Janet AU - Shipley J LA - eng GR - C309/A11566/CRUK_/Cancer Research UK/United Kingdom GR - C5066/A10399/CRUK_/Cancer Research UK/United Kingdom GR - A11566/CRUK_/Cancer Research UK/United Kingdom GR - A10399/CRUK_/Cancer Research UK/United Kingdom GR - 11566/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130805 PL - United States TA - Clin Cancer Res JT - Clinical cancer research : an official journal of the American Association for Cancer Research JID - 9502500 RN - 0 (Antineoplastic Agents) RN - 0 (Benzimidazoles) RN - EC 2.7.1.137 (Class I Phosphatidylinositol 3-Kinases) RN - EC 2.7.11.24 (Mitogen-Activated Protein Kinases) RN - 0 (Morpholines) RN - EC 2.7.1.- (Phosphatidylinositol 3-Kinases) RN - EC 2.7.11.1 (Proto-Oncogene Proteins c-akt) RN - EC 3.6.5.2 (Proto-Oncogene Proteins p21(ras)) RN - EC 2.7.11.1 (TOR Serine-Threonine Kinases) RN - 0 (AZD 6244) RN - 970JJ37FPW ((5-(2,4-bis((3S)-3-methylmorpholin-4-yl)pyrido(2,3-d)pyrimidin-7-yl)-2-methoxyphenyl)methanol) RN - EC 2.7.1.137 (PIK3CA protein, human) SB - IM CIN - Clin Cancer Res. 2013 Nov 1;19(21):5811-3. doi: 10.1158/1078-0432.CCR-13-2177. PMID: 24097859 MH - Animals MH - Antineoplastic Agents/administration & dosage/pharmacology MH - Benzimidazoles/administration & dosage/pharmacology MH - Cell Line, Tumor MH - Class I Phosphatidylinositol 3-Kinases MH - Disease Models, Animal MH - Drug Synergism MH - Enzyme Activation/drug effects MH - Female MH - Gene Knockdown Techniques MH - Humans MH - Mitogen-Activated Protein Kinases/*metabolism MH - Morpholines/administration & dosage/pharmacology MH - Phosphatidylinositol 3-Kinases/genetics/*metabolism MH - Proto-Oncogene Proteins c-akt/*metabolism MH - Proto-Oncogene Proteins p21(ras)/*metabolism MH - Rhabdomyosarcoma/drug therapy/*metabolism/*pathology MH - *Signal Transduction/drug effects MH - TOR Serine-Threonine Kinases/*metabolism MH - Xenograft Model Antitumor Assays PMC - PMC3818134 MID - EMS54323 OID - NLM: EMS54323 EDAT- 2013/08/07 06:00 MHDA- 2014/06/10 06:00 PMCR- 2014/05/01 CRDT- 2013/08/07 06:00 PHST- 2013/08/07 06:00 [entrez] PHST- 2013/08/07 06:00 [pubmed] PHST- 2014/06/10 06:00 [medline] PHST- 2014/05/01 00:00 [pmc-release] AID - 1078-0432.CCR-13-0850 [pii] AID - 10.1158/1078-0432.CCR-13-0850 [doi] PST - ppublish SO - Clin Cancer Res. 2013 Nov 1;19(21):5940-51. doi: 10.1158/1078-0432.CCR-13-0850. Epub 2013 Aug 5. PMID- 29728705 OWN - NLM STAT- MEDLINE DCOM- 20200504 LR - 20250103 IS - 1476-5578 (Electronic) IS - 1359-4184 (Linking) VI - 24 IP - 11 DP - 2019 Nov TI - Pathogenic variants in E3 ubiquitin ligase RLIM/RNF12 lead to a syndromic X-linked intellectual disability and behavior disorder. PG - 1748-1768 LID - 10.1038/s41380-018-0065-x [doi] AB - RLIM, also known as RNF12, is an X-linked E3 ubiquitin ligase acting as a negative regulator of LIM-domain containing transcription factors and participates in X-chromosome inactivation (XCI) in mice. We report the genetic and clinical findings of 84 individuals from nine unrelated families, eight of whom who have pathogenic variants in RLIM (RING finger LIM domain-interacting protein). A total of 40 affected males have X-linked intellectual disability (XLID) and variable behavioral anomalies with or without congenital malformations. In contrast, 44 heterozygous female carriers have normal cognition and behavior, but eight showed mild physical features. All RLIM variants identified are missense changes co-segregating with the phenotype and predicted to affect protein function. Eight of the nine altered amino acids are conserved and lie either within a domain essential for binding interacting proteins or in the C-terminal RING finger catalytic domain. In vitro experiments revealed that these amino acid changes in the RLIM RING finger impaired RLIM ubiquitin ligase activity. In vivo experiments in rlim mutant zebrafish showed that wild type RLIM rescued the zebrafish rlim phenotype, whereas the patient-specific missense RLIM variants failed to rescue the phenotype and thus represent likely severe loss-of-function mutations. In summary, we identified a spectrum of RLIM missense variants causing syndromic XLID and affecting the ubiquitin ligase activity of RLIM, suggesting that enzymatic activity of RLIM is required for normal development, cognition and behavior. FAU - Frints, Suzanna G M AU - Frints SGM AD - Department of Clinical Genetics, Maastricht University Medical Center+, azM, Maastricht, 6202 AZ, The Netherlands. s.frints@mumc.nl. AD - Department of Genetics and Cell Biology, School for Oncology and Developmental Biology, GROW, FHML, Maastricht University, Maastricht, 6200 MD, The Netherlands. s.frints@mumc.nl. FAU - Ozanturk, Aysegul AU - Ozanturk A AD - Center for Human Disease Modeling and Departments of Pediatrics and Psychiatry, Duke University, Durham, NC, 27710, USA. FAU - Rodríguez Criado, Germán AU - Rodríguez Criado G AD - Unidad de Genética Clínica, Hospital Virgen del Rocío, Sevilla, 41920, Spain. FAU - Grasshoff, Ute AU - Grasshoff U AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - de Hoon, Bas AU - de Hoon B AD - Department of Developmental Biology, Erasmus University Medical Center, Rotterdam, 3015 CN, Rotterdam, The Netherlands. AD - Department of Gynaecology and Obstetrics, Erasmus University Medical Center, Rotterdam, 3015 CN, The Netherlands. FAU - Field, Michael AU - Field M AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, 2298, Australia. FAU - Manouvrier-Hanu, Sylvie AU - Manouvrier-Hanu S AD - Clinique de Génétique médicale Guy Fontaine, Centre de référence maladies rares Anomalies du développement Hôpital Jeanne de Flandre, Lille, 59000, France. AD - EA 7364 RADEME Maladies Rares du Développement et du Métabolisme, Faculté de Médecine, Université de Lille, Lille, 59000, France. FAU - E Hickey, Scott AU - E Hickey S AD - Division of Molecular & Human Genetics, Nationwide Children's Hospital, Columbus, OH, 43205, USA. AD - Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, 43205, USA. FAU - Kammoun, Molka AU - Kammoun M AD - Center for Human Genetics, University Hospitals Leuven, Leuven, 3000, Belgium. FAU - Gripp, Karen W AU - Gripp KW AD - Alfred I. duPont Hospital for Children Nemours, Wilmington, DE, 19803, USA. FAU - Bauer, Claudia AU - Bauer C AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - Schroeder, Christopher AU - Schroeder C AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - Toutain, Annick AU - Toutain A AUID- ORCID: 0000-0002-5999-5300 AD - Service de Génétique, Hôpital Bretonneau, CHU de Tours, Tours, 37044, France. AD - UMR 1253, iBrain, Université de Tours, Inserm, Tours, 37032, France. FAU - Mihalic Mosher, Theresa AU - Mihalic Mosher T AUID- ORCID: 0000-0003-3697-5590 AD - Division of Molecular & Human Genetics, Nationwide Children's Hospital, Columbus, OH, 43205, USA. AD - Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, 43205, USA. AD - The Institute for Genomic Medicine, Nationwide Children's Hospital, Columbus, OH, 43205, USA. FAU - Kelly, Benjamin J AU - Kelly BJ AD - The Institute for Genomic Medicine, Nationwide Children's Hospital, Columbus, OH, 43205, USA. FAU - White, Peter AU - White P AUID- ORCID: 0000-0002-5218-5903 AD - Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, 43205, USA. AD - The Institute for Genomic Medicine, Nationwide Children's Hospital, Columbus, OH, 43205, USA. FAU - Dufke, Andreas AU - Dufke A AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - Rentmeester, Eveline AU - Rentmeester E AD - Department of Developmental Biology, Erasmus University Medical Center, Rotterdam, 3015 CN, Rotterdam, The Netherlands. FAU - Moon, Sungjin AU - Moon S AD - Center for Human Disease Modeling and Departments of Pediatrics and Psychiatry, Duke University, Durham, NC, 27710, USA. FAU - Koboldt, Daniel C AU - Koboldt DC AD - Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, 43205, USA. AD - The Institute for Genomic Medicine, Nationwide Children's Hospital, Columbus, OH, 43205, USA. FAU - van Roozendaal, Kees E P AU - van Roozendaal KEP AD - Department of Clinical Genetics, Maastricht University Medical Center+, azM, Maastricht, 6202 AZ, The Netherlands. AD - Department of Genetics and Cell Biology, School for Oncology and Developmental Biology, GROW, FHML, Maastricht University, Maastricht, 6200 MD, The Netherlands. FAU - Hu, Hao AU - Hu H AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, 14195, Germany. FAU - Haas, Stefan A AU - Haas SA AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, 14195, Germany. FAU - Ropers, Hans-Hilger AU - Ropers HH AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, 14195, Germany. FAU - Murray, Lucinda AU - Murray L AD - GOLD (Genetics of Learning and Disability) Service, Hunter Genetics, Waratah, NSW, 2298, Australia. FAU - Haan, Eric AU - Haan E AUID- ORCID: 0000-0002-7310-5124 AD - Adelaide Medical School and Robinson Research Institute, The University of Adelaide, Adelaide, SA, 5000, Australia. AD - South Australian Clinical Genetics Service, SA Pathology (at Women's and Children's Hospital), North Adelaide, SA, 5006, Australia. FAU - Shaw, Marie AU - Shaw M AD - Adelaide Medical School and Robinson Research Institute, The University of Adelaide, Adelaide, SA, 5000, Australia. FAU - Carroll, Renee AU - Carroll R AD - Adelaide Medical School and Robinson Research Institute, The University of Adelaide, Adelaide, SA, 5000, Australia. FAU - Friend, Kathryn AU - Friend K AD - Genetics and Molecular Pathology, SA Pathology, Adelaide, SA, 5006, Australia. FAU - Liebelt, Jan AU - Liebelt J AD - South Australian Clinical Genetics Service, SA Pathology (at Women's and Children's Hospital), North Adelaide, SA, 5006, Australia. FAU - Hobson, Lynne AU - Hobson L AD - Genetics and Molecular Pathology, SA Pathology, Adelaide, SA, 5006, Australia. FAU - De Rademaeker, Marjan AU - De Rademaeker M AD - Centre for Medical Genetics, Reproduction and Genetics, Reproduction Genetics and Regenerative Medicine, Vrije Universiteit Brussel (VUB), UZ Brussel, 1090, Brussels, Belgium. FAU - Geraedts, Joep AU - Geraedts J AD - Department of Clinical Genetics, Maastricht University Medical Center+, azM, Maastricht, 6202 AZ, The Netherlands. AD - Department of Genetics and Cell Biology, School for Oncology and Developmental Biology, GROW, FHML, Maastricht University, Maastricht, 6200 MD, The Netherlands. FAU - Fryns, Jean-Pierre AU - Fryns JP AD - Center for Human Genetics, University Hospitals Leuven, Leuven, 3000, Belgium. FAU - Vermeesch, Joris AU - Vermeesch J AD - Center for Human Genetics, University Hospitals Leuven, Leuven, 3000, Belgium. FAU - Raynaud, Martine AU - Raynaud M AD - Service de Génétique, Hôpital Bretonneau, CHU de Tours, Tours, 37044, France. AD - UMR 1253, iBrain, Université de Tours, Inserm, Tours, 37032, France. FAU - Riess, Olaf AU - Riess O AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - Gribnau, Joost AU - Gribnau J AD - Department of Developmental Biology, Erasmus University Medical Center, Rotterdam, 3015 CN, Rotterdam, The Netherlands. FAU - Katsanis, Nicholas AU - Katsanis N AD - Center for Human Disease Modeling and Departments of Pediatrics and Psychiatry, Duke University, Durham, NC, 27710, USA. FAU - Devriendt, Koen AU - Devriendt K AD - Center for Human Genetics, University Hospitals Leuven, Leuven, 3000, Belgium. FAU - Bauer, Peter AU - Bauer P AD - Institute of Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, 72076, Germany. FAU - Gecz, Jozef AU - Gecz J AUID- ORCID: 0000-0002-7884-6861 AD - Adelaide Medical School and Robinson Research Institute, The University of Adelaide, Adelaide, SA, 5000, Australia. AD - South Australian Health and Medical Research Institute, Adelaide, SA, 5000, Australia. FAU - Golzio, Christelle AU - Golzio C AD - Center for Human Disease Modeling and Departments of Pediatrics and Psychiatry, Duke University, Durham, NC, 27710, USA. AD - Institut de Génétique et de Biologie Moléculaire et Cellulaire, Department of Translational Medicine and Neurogenetics; Centre National de la Recherche Scientifique, UMR7104; Institut National de la Santé et de la Recherche Médicale, U964, Université de Strasbourg, 67400, Illkirch, France. FAU - Gontan, Cristina AU - Gontan C AD - Department of Developmental Biology, Erasmus University Medical Center, Rotterdam, 3015 CN, Rotterdam, The Netherlands. FAU - Kalscheuer, Vera M AU - Kalscheuer VM AUID- ORCID: 0000-0001-6898-3259 AD - Research Group Development and Disease, Max Planck Institute for Molecular Genetics, Berlin, 14195, Germany. kalscheu@molgen.mpg.de. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180504 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (Transcription Factors) RN - 0 (Zebrafish Proteins) RN - EC 2.3.2.27 (RLIM protein, human) RN - EC 2.3.2.27 (Rlim protein, mouse) RN - EC 2.3.2.27 (Ubiquitin-Protein Ligases) RN - EC 2.3.2.27 (rlim protein, zebrafish) SB - IM MH - Adolescent MH - Adult MH - Animals MH - Child MH - Child, Preschool MH - Conduct Disorder/genetics MH - Female MH - Genes, X-Linked MH - HEK293 Cells MH - Humans MH - Infant, Newborn MH - Intellectual Disability/genetics/metabolism MH - Male MH - X-Linked Intellectual Disability/*genetics/metabolism MH - Mice MH - Middle Aged MH - Mutation MH - Pedigree MH - Transcription Factors/genetics MH - Ubiquitin-Protein Ligases/*genetics/*metabolism MH - Ubiquitination MH - X Chromosome Inactivation MH - Zebrafish MH - Zebrafish Proteins/genetics/metabolism EDAT- 2018/05/08 06:00 MHDA- 2020/05/06 06:00 CRDT- 2018/05/06 06:00 PHST- 2017/11/23 00:00 [received] PHST- 2018/02/28 00:00 [accepted] PHST- 2018/05/08 06:00 [pubmed] PHST- 2020/05/06 06:00 [medline] PHST- 2018/05/06 06:00 [entrez] AID - 10.1038/s41380-018-0065-x [pii] AID - 10.1038/s41380-018-0065-x [doi] PST - ppublish SO - Mol Psychiatry. 2019 Nov;24(11):1748-1768. doi: 10.1038/s41380-018-0065-x. Epub 2018 May 4. PMID- 25878120 OWN - NLM STAT- MEDLINE DCOM- 20150825 LR - 20260518 IS - 1528-0020 (Electronic) IS - 0006-4971 (Linking) VI - 125 IP - 24 DP - 2015 Jun 11 TI - Randomized study of reduced-intensity chemotherapy combined with imatinib in adults with Ph-positive acute lymphoblastic leukemia. PG - 3711-9 LID - 10.1182/blood-2015-02-627935 [doi] AB - In this study, we randomly compared high doses of the tyrosine kinase inhibitor imatinib combined with reduced-intensity chemotherapy (arm A) to standard imatinib/hyperCVAD (cyclophosphamide/vincristine/doxorubicin/dexamethasone) therapy (arm B) in 268 adults (median age, 47 years) with Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL). The primary objective was the major molecular response (MMolR) rate after cycle 2, patients being then eligible for allogeneic stem cell transplantation (SCT) if they had a donor, or autologous SCT if in MMolR and no donor. With fewer induction deaths, the complete remission (CR) rate was higher in arm A than in arm B (98% vs 91%; P = .006), whereas the MMolR rate was similar in both arms (66% vs 64%). With a median follow-up of 4.8 years, 5-year event-free survival and overall survival (OS) rates were estimated at 37.1% and 45.6%, respectively, without difference between the arms. Allogeneic transplantation was associated with a significant benefit in relapse-free survival (hazard ratio [HR], 0.69; P = .036) and OS (HR, 0.64; P = .02), with initial white blood cell count being the only factor significantly interacting with this SCT effect. In patients achieving MMolR, outcome was similar after autologous and allogeneic transplantation. This study validates an induction regimen combining reduced-intensity chemotherapy and imatinib in Ph+ ALL adult patients and suggests that SCT in first CR is still a good option for Ph+ ALL adult patients. This trial was registered at www.clinicaltrials.gov as #NCT00327678. CI - © 2015 by The American Society of Hematology. FAU - Chalandon, Yves AU - Chalandon Y AD - Division of Hematology, Department of Medical Specialties, University Hospital and University of Geneva, Geneva, Switzerland; Swiss Group for Clinical Cancer Research, Bern, Switzerland; FAU - Thomas, Xavier AU - Thomas X AD - Division of Hematology, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre Bénite, France; FAU - Hayette, Sandrine AU - Hayette S AD - Division of Hematology, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre Bénite, France; FAU - Cayuela, Jean-Michel AU - Cayuela JM AD - Division of Hematology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Paris, France; FAU - Abbal, Claire AU - Abbal C AD - Division of Hematology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; FAU - Huguet, Françoise AU - Huguet F AD - Division of Hematology, Institut Universitaire de Cancérologie, Toulouse, France; FAU - Raffoux, Emmanuel AU - Raffoux E AD - Division of Hematology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Paris, France; FAU - Leguay, Thibaut AU - Leguay T AD - Division of Hematology, Hôpital Haut-Levêque, Pessac, France; FAU - Rousselot, Philippe AU - Rousselot P AD - Division of Hematology, Hôpital Mignot, Versailles, France; FAU - Lepretre, Stéphane AU - Lepretre S AD - Division of Hematology, Centre Henri Becquerel, Rouen, France; FAU - Escoffre-Barbe, Martine AU - Escoffre-Barbe M AD - Division of Hematology, Hôpital Pontchailloux, Rennes, France; FAU - Maury, Sébastien AU - Maury S AD - Division of Hematology, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, University Paris Est Créteil, Créteil, France; FAU - Berthon, Céline AU - Berthon C AD - Division of Hematology, Hôpital Claude Huriez, Lille, France; FAU - Tavernier, Emmanuelle AU - Tavernier E AD - Division of Hematology, Institut de Cancérologie Lucien-Neuwirth, Saint-Priest-en-Jarez, France; FAU - Lambert, Jean-François AU - Lambert JF AD - Swiss Group for Clinical Cancer Research, Bern, Switzerland; Division of Hematology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; FAU - Lafage-Pochitaloff, Marina AU - Lafage-Pochitaloff M AD - Division of Hematology, Hôpital de la Timone, Marseille, France; FAU - Lhéritier, Véronique AU - Lhéritier V AD - Group for Research on Adult Acute Lymphoblastic Leukemia, Lyon, France; FAU - Chevret, Sylvie AU - Chevret S AD - Division of Biostatistics, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Paris, France; and. FAU - Ifrah, Norbert AU - Ifrah N AD - Division of Hematology, Centre Hospitalier Universitaire d'Angers and INSERM U892/Centre National de la Recherche Scientifique 6299, Angers, France. FAU - Dombret, Hervé AU - Dombret H AD - Division of Hematology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Paris, France; CN - Group for Research on Adult Acute Lymphoblastic Leukemia (GRAALL) LA - eng SI - ClinicalTrials.gov/NCT00327678 PT - Journal Article PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't DEP - 20150415 PL - United States TA - Blood JT - Blood JID - 7603509 RN - 0 (Benzamides) RN - 0 (Piperazines) RN - 0 (Protein Kinase Inhibitors) RN - 0 (Pyrimidines) RN - 8A1O1M485B (Imatinib Mesylate) SB - IM EIN - Blood. 2015 Sep 3;126(10):1261 CIN - Blood. 2015 Jun 11;125(24):3674-5. doi: 10.1182/blood-2015-04-641704. PMID: 26069331 MH - Adolescent MH - Adult MH - Antineoplastic Combined Chemotherapy Protocols/administration & dosage/*therapeutic use/toxicity MH - Benzamides/administration & dosage/*therapeutic use/toxicity MH - Disease-Free Survival MH - Female MH - Humans MH - Imatinib Mesylate MH - Male MH - Middle Aged MH - *Philadelphia Chromosome MH - Piperazines/administration & dosage/*therapeutic use/toxicity MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*drug therapy/*genetics/therapy MH - Protein Kinase Inhibitors/administration & dosage/*therapeutic use/toxicity MH - Pyrimidines/administration & dosage/*therapeutic use/toxicity MH - Stem Cell Transplantation MH - Treatment Outcome MH - Young Adult FIR - Caillères IR - Caillères FIR - Chaib IR - Chaib FIR - Blanc IR - Blanc FIR - Brunel IR - Brunel FIR - Cuvelier IR - Cuvelier FIR - Marolleau IR - Marolleau FIR - Damaj IR - Damaj FIR - Vaida IR - Vaida FIR - Royer IR - Royer FIR - Gruson IR - Gruson FIR - Merlusca IR - Merlusca FIR - Copin IR - Copin FIR - Capiod IR - Capiod FIR - Dubus IR - Dubus FIR - Mpari IR - Mpari FIR - Hunault IR - Hunault FIR - Ifrah IR - Ifrah FIR - Schmidt IR - Schmidt FIR - Dib IR - Dib FIR - Francois IR - Francois FIR - Foussard IR - Foussard FIR - Guardiola IR - Guardiola FIR - Zandecki IR - Zandecki FIR - Blanchet IR - Blanchet FIR - Baranger IR - Baranger FIR - Chassevent IR - Chassevent FIR - Genevieve IR - Genevieve FIR - Marie IR - Marie FIR - Parry IR - Parry FIR - Orsini-Piocelle IR - Orsini-Piocelle FIR - Corront IR - Corront FIR - Daguindau IR - Daguindau FIR - Cony-Makhoul IR - Cony-Makhoul FIR - Reynes IR - Reynes FIR - Cadoux IR - Cadoux FIR - Vittet IR - Vittet FIR - Sutton IR - Sutton FIR - Al Jijakli IR - Al Jijakli FIR - Genet IR - Genet FIR - Chaoui IR - Chaoui FIR - Mesbah IR - Mesbah FIR - Morel IR - Morel FIR - Touahri IR - Touahri FIR - Mossafa IR - Mossafa FIR - Fourcade IR - Fourcade FIR - Guerekobaya IR - Guerekobaya FIR - Zerazhi IR - Zerazhi FIR - Azzedine IR - Azzedine FIR - Boulat IR - Boulat FIR - Lepeu IR - Lepeu FIR - Touchais IR - Touchais FIR - Derre IR - Derre FIR - Beyrne IR - Beyrne FIR - Araujo IR - Araujo FIR - Bauduer IR - Bauduer FIR - Banos IR - Banos FIR - Renoux IR - Renoux FIR - Menard IR - Menard FIR - Labarrère IR - Labarrère FIR - Legrand IR - Legrand FIR - Berceanu IR - Berceanu FIR - Daguindau IR - Daguindau FIR - Deconinck IR - Deconinck FIR - Larosa IR - Larosa FIR - Ferrand IR - Ferrand FIR - Brion IR - Brion FIR - Collonge-Rame IR - Collonge-Rame FIR - Garnache Ottou IR - Garnache Ottou FIR - Peria IR - Peria FIR - Rodon IR - Rodon FIR - Elyamadi IR - Elyamadi FIR - Kadiri IR - Kadiri FIR - Gardin IR - Gardin FIR - Ades IR - Ades FIR - Fenaux IR - Fenaux FIR - Braun IR - Braun FIR - Cayuela IR - Cayuela FIR - Eclache IR - Eclache FIR - Letestu IR - Letestu FIR - Nloga IR - Nloga FIR - Leguay IR - Leguay FIR - Milpied IR - Milpied FIR - Lascaux IR - Lascaux FIR - Dilhuydy IR - Dilhuydy FIR - Dimicoli IR - Dimicoli FIR - Pigneux IR - Pigneux FIR - Tabrizi IR - Tabrizi FIR - Dumas IR - Dumas FIR - Lacombe IR - Lacombe FIR - Lippert IR - Lippert FIR - Sauvezie IR - Sauvezie FIR - Choufi IR - Choufi FIR - Kadiata IR - Kadiata FIR - Barry IR - Barry FIR - Voronina IR - Voronina FIR - Brument IR - Brument FIR - Nouvel IR - Nouvel FIR - Guillerm IR - Guillerm FIR - Berthou IR - Berthou FIR - Dalbies IR - Dalbies FIR - Tempescul IR - Tempescul FIR - Ianotto IR - Ianotto FIR - Couturier IR - Couturier FIR - Eveillard IR - Eveillard FIR - Ugo IR - Ugo FIR - De Braekeleer IR - De Braekeleer FIR - Le Calvez IR - Le Calvez FIR - Gillet IR - Gillet FIR - Reman IR - Reman FIR - Leporrier IR - Leporrier FIR - Chantepie IR - Chantepie FIR - Johnson Ansah IR - Johnson Ansah FIR - Gac IR - Gac FIR - Macro IR - Macro FIR - Benabed IR - Benabed FIR - Cheze IR - Cheze FIR - Naguib IR - Naguib FIR - Plessis IR - Plessis FIR - Salaun IR - Salaun FIR - Marin IR - Marin FIR - Malfuson IR - Malfuson FIR - Konopacki IR - Konopacki FIR - Souleau IR - Souleau FIR - De Revel IR - De Revel FIR - Fagot IR - Fagot FIR - Foissaud IR - Foissaud FIR - Harrouche IR - Harrouche FIR - Cacheux IR - Cacheux FIR - Bay IR - Bay FIR - Tournilhac IR - Tournilhac FIR - Hermet IR - Hermet FIR - Guieze IR - Guieze FIR - Bailly IR - Bailly FIR - Chaleteix IR - Chaleteix FIR - De Renzis IR - De Renzis FIR - Chabrot IR - Chabrot FIR - Calvet IR - Calvet FIR - Sapin IR - Sapin FIR - Tchirkov IR - Tchirkov FIR - Périssel IR - Périssel FIR - Veronese IR - Veronese FIR - Chassagne Berger IR - Chassagne Berger FIR - Roy IR - Roy FIR - Auduy IR - Auduy FIR - Raby IR - Raby FIR - Kohser IR - Kohser FIR - Humbrecht IR - Humbrecht FIR - Barats IR - Barats FIR - Husseini IR - Husseini FIR - Moskovtchenko IR - Moskovtchenko FIR - Mazurier IR - Mazurier FIR - Camara IR - Camara FIR - Salanoubat IR - Salanoubat FIR - Haiat IR - Haiat FIR - Petitdidier IR - Petitdidier FIR - Cereja IR - Cereja FIR - Joly IR - Joly FIR - Devidas IR - Devidas FIR - Quillet IR - Quillet FIR - Boutant IR - Boutant FIR - Maury IR - Maury FIR - Cordonnier IR - Cordonnier FIR - Pautas IR - Pautas FIR - Toma IR - Toma FIR - Hichri IR - Hichri FIR - Kuentz IR - Kuentz FIR - Bories IR - Bories FIR - Jouault IR - Jouault FIR - Wagner-Ballon IR - Wagner-Ballon FIR - Perot IR - Perot FIR - Plonquet IR - Plonquet FIR - Cabanne IR - Cabanne FIR - Caillot IR - Caillot FIR - Lafon IR - Lafon FIR - Casasnovas IR - Casasnovas FIR - Ferrant IR - Ferrant FIR - Bastié IR - Bastié FIR - Teyssier IR - Teyssier FIR - Mugneret IR - Mugneret FIR - Grandjean IR - Grandjean FIR - Pignon IR - Pignon FIR - Bemba IR - Bemba FIR - Wetterwald IR - Wetterwald FIR - Roumier IR - Roumier FIR - Paquez IR - Paquez FIR - Cahn IR - Cahn FIR - Thiebaut IR - Thiebaut FIR - Gressin IR - Gressin FIR - Bulabois IR - Bulabois FIR - Simon IR - Simon FIR - Pégourie IR - Pégourie FIR - Courby IR - Courby FIR - Molina IR - Molina FIR - Callanan IR - Callanan FIR - Lefebvre IR - Lefebvre FIR - Jacob IR - Jacob FIR - Pittet IR - Pittet FIR - Agapé IR - Agapé FIR - Belkaid IR - Belkaid FIR - Henni IR - Henni FIR - Berg IR - Berg FIR - Pequin IR - Pequin FIR - Stalnikiewicz IR - Stalnikiewicz FIR - Dupriez IR - Dupriez FIR - Morel IR - Morel FIR - Declercq IR - Declercq FIR - Berthon IR - Berthon FIR - Quesnel IR - Quesnel FIR - Preudhomme IR - Preudhomme FIR - Grardel IR - Grardel FIR - Roche Lestienne IR - Roche Lestienne FIR - Lai IR - Lai FIR - Lepelley IR - Lepelley FIR - Djeda IR - Djeda FIR - Turlure IR - Turlure FIR - Bordessoule IR - Bordessoule FIR - Touati IR - Touati FIR - Remenieras IR - Remenieras FIR - Abraham IR - Abraham FIR - Gourin IR - Gourin FIR - Girault IR - Girault FIR - Moreau IR - Moreau FIR - Jaccard IR - Jaccard FIR - Chaury IR - Chaury FIR - Penot IR - Penot FIR - Trimoreau IR - Trimoreau FIR - Gachard IR - Gachard FIR - Feuillard IR - Feuillard FIR - Tisseuil IR - Tisseuil FIR - Philippon IR - Philippon FIR - Bosselut IR - Bosselut FIR - Nlend IR - Nlend FIR - Salles IR - Salles FIR - Coiffier IR - Coiffier FIR - Espinouse IR - Espinouse FIR - Michallet IR - Michallet FIR - Karlin IR - Karlin FIR - Bouafia-Sauvy IR - Bouafia-Sauvy FIR - Broussais-Guillaumot IR - Broussais-Guillaumot FIR - Traullé IR - Traullé FIR - Callet-Bochu IR - Callet-Bochu FIR - Tigaud IR - Tigaud FIR - Hayette IR - Hayette FIR - Maire IR - Maire FIR - Thomas IR - Thomas FIR - Chelghoum IR - Chelghoum FIR - Nicolini IR - Nicolini FIR - Ducastelle IR - Ducastelle FIR - Labussière IR - Labussière FIR - Detrait IR - Detrait FIR - Plesa IR - Plesa FIR - Boucher IR - Boucher FIR - Etienne IR - Etienne FIR - Prebet IR - Prebet FIR - Charbonnier IR - Charbonnier FIR - Brunelle IR - Brunelle FIR - D'Incan IR - D'Incan FIR - Vey IR - Vey FIR - Rey IR - Rey FIR - Mozziconacci IR - Mozziconacci FIR - Sainty IR - Sainty FIR - Laibes IR - Laibes FIR - Arnoulet IR - Arnoulet FIR - Dridi IR - Dridi FIR - Redortier IR - Redortier FIR - Frayfer IR - Frayfer FIR - Allard IR - Allard FIR - Matis IR - Matis FIR - Dorvaux IR - Dorvaux FIR - Guibaud IR - Guibaud FIR - Zamfir IR - Zamfir FIR - Christian IR - Christian FIR - Staal IR - Staal FIR - Benet IR - Benet FIR - Hagopian IR - Hagopian FIR - Vincent IR - Vincent FIR - Quittet IR - Quittet FIR - Navarro IR - Navarro FIR - Ceballos IR - Ceballos FIR - Fegueux IR - Fegueux FIR - Cartron IR - Cartron FIR - Legouffe IR - Legouffe FIR - Rossi IR - Rossi FIR - Tondeur IR - Tondeur FIR - Cacheux IR - Cacheux FIR - Bret IR - Bret FIR - Taviaux IR - Taviaux FIR - Portales IR - Portales FIR - Lavabre IR - Lavabre FIR - Taib IR - Taib FIR - Eliaou IR - Eliaou FIR - Leroux IR - Leroux FIR - Theis IR - Theis FIR - Ojeda-Uribe IR - Ojeda-Uribe FIR - Drenou IR - Drenou FIR - Eisenmann IR - Eisenmann FIR - Arkam IR - Arkam FIR - Rimelen IR - Rimelen FIR - Jeandidier IR - Jeandidier FIR - Debliquis IR - Debliquis FIR - Iglarz IR - Iglarz FIR - Haby IR - Haby FIR - Bonmati IR - Bonmati FIR - Witz IR - Witz FIR - Feugier IR - Feugier FIR - Randa IR - Randa FIR - Namour IR - Namour FIR - Grégoire IR - Grégoire FIR - Monhoven IR - Monhoven FIR - Léotard IR - Léotard FIR - Jonveaux IR - Jonveaux FIR - Béné IR - Béné FIR - Schwartz IR - Schwartz FIR - Chevallier IR - Chevallier FIR - Mohty IR - Mohty FIR - Harousseau IR - Harousseau FIR - Le Gouill IR - Le Gouill FIR - Gastinne IR - Gastinne FIR - Peterlin IR - Peterlin FIR - Guillaume IR - Guillaume FIR - Delaunay IR - Delaunay FIR - Vigouroux IR - Vigouroux FIR - Mahé IR - Mahé FIR - Lodé IR - Lodé FIR - Garand IR - Garand FIR - Robillard IR - Robillard FIR - Talmant IR - Talmant FIR - Avet-Loiseau IR - Avet-Loiseau FIR - Le Houerou IR - Le Houerou FIR - Sirvent IR - Sirvent FIR - Mannone IR - Mannone FIR - Gratecos IR - Gratecos FIR - Legros IR - Legros FIR - Raynaud IR - Raynaud FIR - Philip IR - Philip FIR - Ticchioni IR - Ticchioni FIR - Bouskila IR - Bouskila FIR - Jourdan IR - Jourdan FIR - Richard IR - Richard FIR - Gaillard IR - Gaillard FIR - Arnaud IR - Arnaud FIR - Chiesa IR - Chiesa FIR - Brun IR - Brun FIR - Nicolas IR - Nicolas FIR - Alexis IR - Alexis FIR - Boulet IR - Boulet FIR - Carp IR - Carp FIR - Benbrahim IR - Benbrahim FIR - Schoenwald IR - Schoenwald FIR - Legac IR - Legac FIR - Michel IR - Michel FIR - Dreyfus IR - Dreyfus FIR - Bouscary IR - Bouscary FIR - Al Nawakil IR - Al Nawakil FIR - Picard IR - Picard FIR - Vigue IR - Vigue FIR - Mekdour IR - Mekdour FIR - Buzyn IR - Buzyn FIR - Suarez IR - Suarez FIR - Delarue IR - Delarue FIR - Sibon IR - Sibon FIR - Cheminant IR - Cheminant FIR - Marcais IR - Marcais FIR - Frenzel IR - Frenzel FIR - Brignier IR - Brignier FIR - Asnafi IR - Asnafi FIR - Radford-Weiss IR - Radford-Weiss FIR - MacIntyre IR - MacIntyre FIR - Berdous Saheb IR - Berdous Saheb FIR - Isnard IR - Isnard FIR - Legrand IR - Legrand FIR - Lapusan IR - Lapusan FIR - Marzac IR - Marzac FIR - Perot IR - Perot FIR - Feger IR - Feger FIR - Ikhlef IR - Ikhlef FIR - Dombret IR - Dombret FIR - Raffoux IR - Raffoux FIR - Lengline IR - Lengline FIR - 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Stamatoullas IR - Stamatoullas FIR - Lemasle IR - Lemasle FIR - Jardin IR - Jardin FIR - Brehar IR - Brehar FIR - Bastard IR - Bastard FIR - Etancelin IR - Etancelin FIR - Boutet IR - Boutet FIR - Penther IR - Penther FIR - Lenormand IR - Lenormand FIR - Simon IR - Simon FIR - Janvier IR - Janvier FIR - Fabre IR - Fabre FIR - Samieh IR - Samieh FIR - Elias IR - Elias FIR - Al Jassem IR - Al Jassem FIR - Bourguignat IR - Bourguignat FIR - Glaisner IR - Glaisner FIR - Soussain IR - Soussain FIR - Malak IR - Malak FIR - Vargaftig IR - Vargaftig FIR - Bennani IR - Bennani FIR - Vavasseur IR - Vavasseur FIR - Tavernier IR - Tavernier FIR - Guyotat IR - Guyotat FIR - Jaubert IR - Jaubert FIR - Cornillon IR - Cornillon FIR - Mounier IR - Mounier FIR - Flandrin-Gresta IR - Flandrin-Gresta FIR - Nadal IR - Nadal FIR - Vasselon IR - Vasselon FIR - Solly IR - Solly FIR - Campos IR - Campos FIR - Marchand IR - Marchand FIR - Bilger IR - Bilger FIR - Lioure IR - Lioure FIR - Herbrecht IR - Herbrecht FIR - Bories IR - Bories FIR - Fornecker IR - Fornecker FIR - Fohrer IR - Fohrer FIR - Moulin IR - Moulin FIR - Gaub IR - Gaub FIR - Gervais IR - Gervais FIR - Mauvieux IR - Mauvieux FIR - Oudet IR - Oudet FIR - Eischen IR - Eischen FIR - Aubry IR - Aubry FIR - Magnier IR - Magnier FIR - Kravanja IR - Kravanja FIR - Huguet IR - Huguet FIR - Huynh IR - Huynh FIR - Roussel IR - Roussel FIR - Tavitian IR - Tavitian FIR - Ysebaert IR - Ysebaert FIR - Recher IR - Recher FIR - Borel IR - Borel FIR - Hebraud IR - Hebraud FIR - Oberic IR - Oberic FIR - Laurent IR - Laurent FIR - Nouvel IR - Nouvel FIR - Delabesse IR - Delabesse FIR - Kuhlein IR - Kuhlein FIR - Dastugue IR - Dastugue FIR - Demas IR - Demas FIR - Luquet IR - Luquet FIR - Vergez IR - Vergez FIR - Daniel IR - Daniel FIR - Leclerc IR - Leclerc FIR - Lissandre IR - Lissandre FIR - Dartigeas IR - Dartigeas FIR - Gyan IR - Gyan FIR - Renaud IR - Renaud FIR - Monjanel IR - Monjanel FIR - Ertault de la Bretonniere IR - Ertault de la Bretonniere FIR - Benboubker IR - Benboubker FIR - Estienne IR - Estienne FIR - Barin IR - Barin FIR - Watier IR - Watier FIR - Nollet IR - Nollet FIR - Fernandes IR - Fernandes FIR - Pollet IR - Pollet FIR - Tricot IR - Tricot FIR - Simon IR - Simon FIR - Bremeault IR - Bremeault FIR - Daudignon IR - Daudignon FIR - Bisiau IR - Bisiau FIR - Crametz IR - Crametz FIR - Rousselot IR - Rousselot FIR - Castaigne IR - Castaigne FIR - Salmeron IR - Salmeron FIR - Lambert IR - Lambert FIR - Taksin IR - Taksin FIR - Rigaudeau IR - Rigaudeau FIR - Farhat IR - Farhat FIR - Merabet IR - Merabet FIR - Ghez IR - Ghez FIR - Spentchian IR - Spentchian FIR - Terré IR - Terré FIR - Garcia IR - Garcia FIR - Henri IR - Henri FIR - De Botton IR - De Botton FIR - Bourhis IR - Bourhis FIR - Arnaud IR - Arnaud FIR - Micol IR - Micol FIR - Garnier IR - Garnier FIR - Willekens IR - Willekens FIR - Bennaceur IR - Bennaceur FIR - Auger IR - Auger FIR - Saada IR - Saada FIR - Bargetzi IR - Bargetzi FIR - Heizmann IR - Heizmann FIR - Gurtner IR - Gurtner FIR - Gratwohl IR - Gratwohl FIR - Medinger IR - Medinger FIR - Arber IR - Arber FIR - Stern IR - Stern FIR - Heim IR - Heim FIR - Buser IR - Buser FIR - Halter IR - Halter FIR - Cantoni IR - Cantoni FIR - Gerull IR - Gerull FIR - Villa Hohler IR - Villa Hohler FIR - Wannesson IR - Wannesson FIR - Servida IR - Servida FIR - Christinat IR - Christinat FIR - Steffanoni IR - Steffanoni FIR - Cresto IR - Cresto FIR - Busi IR - Busi FIR - Pabst IR - Pabst FIR - Klingenberg-Rettich IR - Klingenberg-Rettich FIR - Chalandon IR - Chalandon FIR - Passweg IR - Passweg FIR - Robert IR - Robert FIR - Bernimoulin IR - Bernimoulin FIR - Levrat IR - Levrat FIR - Chigrinova IR - Chigrinova FIR - Verholen IR - Verholen FIR - Matthes IR - Matthes FIR - Salamanchuck IR - Salamanchuck FIR - Trembleau IR - Trembleau FIR - Lambert IR - Lambert FIR - Spertini IR - Spertini FIR - Jotterand IR - Jotterand FIR - Schoumans Pouw IR - Schoumans Pouw FIR - Abbal IR - Abbal FIR - Quarroz IR - Quarroz FIR - Porter IR - Porter FIR - Jeckelmann IR - Jeckelmann FIR - Voegtlin IR - Voegtlin FIR - Gregor IR - Gregor FIR - Nagler IR - Nagler FIR - Fahrni IR - Fahrni FIR - Saint Gallen IR - Saint Gallen FIR - Hess IR - Hess FIR - Driessen IR - Driessen FIR - Hitz IR - Hitz FIR - Weder IR - Weder FIR - Mark IR - Mark FIR - Schnitter IR - Schnitter FIR - Mme Raess IR - Mme Raess FIR - Demmer IR - Demmer FIR - de Wolf-Linder IR - de Wolf-Linder FIR - Schanz IR - Schanz FIR - Gemünden IR - Gemünden FIR - Nair IR - Nair FIR - Stüssi IR - Stüssi FIR - Delaloye IR - Delaloye FIR - Müller IR - Müller FIR - Solana IR - Solana FIR - Schäfer IR - Schäfer FIR - Reiner IR - Reiner FIR - Sasselli IR - Sasselli FIR - Ghielmetti IR - Ghielmetti FIR - Wolf IR - Wolf FIR - Genton IR - Genton FIR - Largiader IR - Largiader FIR - Kuenzle IR - Kuenzle FIR - Keunecke IR - Keunecke EDAT- 2015/04/17 06:00 MHDA- 2015/08/26 06:00 CRDT- 2015/04/17 06:00 PHST- 2015/02/09 00:00 [received] PHST- 2015/04/11 00:00 [accepted] PHST- 2015/04/17 06:00 [entrez] PHST- 2015/04/17 06:00 [pubmed] PHST- 2015/08/26 06:00 [medline] AID - S0006-4971(20)31546-9 [pii] AID - 10.1182/blood-2015-02-627935 [doi] PST - ppublish SO - Blood. 2015 Jun 11;125(24):3711-9. doi: 10.1182/blood-2015-02-627935. Epub 2015 Apr 15. PMID- 32432994 OWN - NLM STAT- MEDLINE DCOM- 20200706 LR - 20260518 IS - 1524-4628 (Electronic) IS - 0039-2499 (Linking) VI - 51 IP - 7 DP - 2020 Jul TI - Mechanical Thrombectomy for Acute Ischemic Stroke Amid the COVID-19 Outbreak: Decreased Activity, and Increased Care Delays. PG - 2012-2017 LID - 10.1161/STROKEAHA.120.030373 [doi] AB - BACKGROUND AND PURPOSE: The efficiency of prehospital care chain response and the adequacy of hospital resources are challenged amid the coronavirus disease 2019 (COVID-19) outbreak, with suspected consequences for patients with ischemic stroke eligible for mechanical thrombectomy (MT). METHODS: We conducted a prospective national-level data collection of patients treated with MT, ranging 45 days across epidemic containment measures instatement, and of patients treated during the same calendar period in 2019. The primary end point was the variation of patients receiving MT during the epidemic period. Secondary end points included care delays between onset, imaging, and groin puncture. To analyze the primary end point, we used a Poisson regression model. We then analyzed the correlation between the number of MTs and the number of COVID-19 cases hospitalizations, using the Pearson correlation coefficient (compared with the null value). RESULTS: A total of 1513 patients were included at 32 centers, in all French administrative regions. There was a 21% significant decrease (0.79; [95%CI, 0.76-0.82]; P<0.001) in MT case volumes during the epidemic period, and a significant increase in delays between imaging and groin puncture, overall (mean 144.9±SD 86.8 minutes versus 126.2±70.9; P<0.001 in 2019) and in transferred patients (mean 182.6±SD 82.0 minutes versus 153.25±67; P<0.001). After the instatement of strict epidemic mitigation measures, there was a significant negative correlation between the number of hospitalizations for COVID and the number of MT cases (R(2) -0.51; P=0.04). Patients treated during the COVID outbreak were less likely to receive intravenous thrombolysis and to have unwitnessed strokes (both P<0.05). CONCLUSIONS: Our study showed a significant decrease in patients treated with MTs during the first stages of the COVID epidemic in France and alarming indicators of lengthened care delays. These findings prompt immediate consideration of local and regional stroke networks preparedness in the varying contexts of COVID-19 pandemic evolution. FAU - Kerleroux, Basile AU - Kerleroux B AD - Neuroradiology Department, CH Sainte-Anne, Paris, France (B.K., W.B.H., G.B.). FAU - Fabacher, Thibaut AU - Fabacher T AD - Public Health, CHRU Strasbourg, France (T.F.). FAU - Bricout, Nicolas AU - Bricout N AD - Interventional Neuroradiology Department, CHRU Lille, France (N.B., M.M.). FAU - Moïse, Martin AU - Moïse M AD - Interventional Neuroradiology Department, CHRU Lille, France (N.B., M.M.). FAU - Testud, Benoit AU - Testud B AD - Interventional Neuroradiology Department, CHRU Marseille La Timone, France (B.T., S.V.). FAU - Vingadassalom, Sivadji AU - Vingadassalom S AD - Interventional Neuroradiology Department, CHRU Marseille La Timone, France (B.T., S.V.). FAU - Ifergan, Héloïse AU - Ifergan H AD - Interventional Neuroradiology Department, CHRU Tours, France (H.I., K.J.). FAU - Janot, Kévin AU - Janot K AD - Interventional Neuroradiology Department, CHRU Tours, France (H.I., K.J.). FAU - Consoli, Arturo AU - Consoli A AD - Interventional Neuroradiology Department, CH Foch, France (A.C.). FAU - Ben Hassen, Wagih AU - Ben Hassen W AD - Neuroradiology Department, CH Sainte-Anne, Paris, France (B.K., W.B.H., G.B.). FAU - Shotar, Eimad AU - Shotar E AD - Department of Neuroradiology, Pitié-Salpêtrière Hospital, Paris, France (E.S.). FAU - Ognard, Julien AU - Ognard J AD - Interventional Neuroradiology Department, CHRU Brest, France (J.O.). FAU - Charbonnier, Guillaume AU - Charbonnier G AD - Interventional Neuroradiology Department, CHRU Besançon, France (G.C.). FAU - L'Allinec, Vincent AU - L'Allinec V AD - Radiology Department, CHU Angers, France (V.L.). FAU - Guédon, Alexis AU - Guédon A AD - Department of Neuroradiology, Lariboisière Hospital, Paris, France (A.G.). FAU - Bolognini, Federico AU - Bolognini F AD - Interventional Neuroradiology Department, CHRU Colmar, France (F.B.). FAU - Marnat, Gaultier AU - Marnat G AD - Interventional Neuroradiology Department, CHRU Bordeaux, France (G.M., J.B.). FAU - Forestier, Géraud AU - Forestier G AD - Interventional Neuroradiology Department, CHU Limoges, France (G.F., A.R.). FAU - Rouchaud, Aymeric AU - Rouchaud A AD - Interventional Neuroradiology Department, CHU Limoges, France (G.F., A.R.). FAU - Pop, Raoul AU - Pop R AD - Interventional Neuroradiology Department, CHRU Strasbourg, France (R.P.). FAU - Raynaud, Nicolas AU - Raynaud N AD - Interventional Neuroradiology Department, CHRU Poitiers, France (N.R.). FAU - Zhu, François AU - Zhu F AD - Interventional Neuroradiology Department, CHRU Nancy, France (F.Z.). FAU - Cortese, Jonathan AU - Cortese J AD - Interventional Neuroradiology Department, Kremlin Bicêtre Hospital, Bicêtre, France (J.C., V.C.). FAU - Chalumeau, Vanessa AU - Chalumeau V AD - Interventional Neuroradiology Department, Kremlin Bicêtre Hospital, Bicêtre, France (J.C., V.C.). FAU - Berge, Jérome AU - Berge J AD - Interventional Neuroradiology Department, CHRU Bordeaux, France (G.M., J.B.). FAU - Escalard, Simon AU - Escalard S AD - Interventional Neuroradiology Department, Fondation Rothschild, Paris, France (S.E.). FAU - Boulouis, Grégoire AU - Boulouis G AD - Neuroradiology Department, CH Sainte-Anne, Paris, France (B.K., W.B.H., G.B.). CN - SFNR, the ETIS registry, and the JENI-Research Collaborative LA - eng PT - Journal Article DEP - 20200520 PL - United States TA - Stroke JT - Stroke JID - 0235266 SB - IM MH - Aged MH - Aged, 80 and over MH - *Betacoronavirus MH - Brain Ischemia/epidemiology/*surgery MH - COVID-19 MH - *Coronavirus Infections MH - *Delivery of Health Care MH - Female MH - France/epidemiology MH - Hospitalization/statistics & numerical data MH - Humans MH - Male MH - Mechanical Thrombolysis/methods/*statistics & numerical data MH - Middle Aged MH - *Pandemics MH - Patient Admission/statistics & numerical data MH - *Pneumonia, Viral MH - Procedures and Techniques Utilization MH - Prospective Studies MH - SARS-CoV-2 MH - Stroke/epidemiology/*surgery MH - Time-to-Treatment/statistics & numerical data OTO - NOTNLM OT - COVID-19 OT - coronavirus OT - groin OT - hospitalization OT - thrombectomy FIR - Chivot, Cyril IR - Chivot C FIR - Hanafi, Riyad IR - Hanafi R FIR - Pasco, Anne IR - Pasco A FIR - Girot, Jean-Baptiste IR - Girot JB FIR - Biondi, Alessandra IR - Biondi A FIR - Di Caterino, Fortunato IR - Di Caterino F FIR - Primikiris, Panagiotis IR - Primikiris P FIR - Vitale, Giovanni IR - Vitale G FIR - Bonnet, Louise IR - Bonnet L FIR - Gariel, Florent IR - Gariel F FIR - Barreau, Xavier IR - Barreau X FIR - Debruxelles, Sabrina IR - Debruxelles S FIR - Lucas, Ludovic IR - Lucas L FIR - Menegon, Patrice IR - Menegon P FIR - Olindo, Stéphane IR - Olindo S FIR - Poli, Mathilde IR - Poli M FIR - Renou, Pauline IR - Renou P FIR - Sagnier, Sharmila IR - Sagnier S FIR - Sibon, Igor IR - Sibon I FIR - Veunac, Louis IR - Veunac L FIR - Gentric, Jean-Christophe IR - Gentric JC FIR - Barbier, Charlotte IR - Barbier C FIR - Boulanger, Marion IR - Boulanger M FIR - Cogez, Julien IR - Cogez J FIR - Guettier, Sophie IR - Guettier S FIR - Schneckenburger, Romain IR - Schneckenburger R FIR - Touze, Emmanuel IR - Touze E FIR - Delaitre, Mariette IR - Delaitre M FIR - Lebendinsky, Pablo IR - Lebendinsky P FIR - Musacchio, Mariano IR - Musacchio M FIR - Ricolfi, Frédéric IR - Ricolfi F FIR - Thouant, Pierre IR - Thouant P FIR - Caparros, François IR - Caparros F FIR - Casolla, Barbara IR - Casolla B FIR - Della Schiava, Lucie IR - Della Schiava L FIR - Dequatre, Nelly IR - Dequatre N FIR - Henon, Hilde IR - Henon H FIR - Pasi, Marco IR - Pasi M FIR - Kazemi, Apolline IR - Kazemi A FIR - Bala, Fouzi IR - Bala F FIR - Estrade, Laurent IR - Estrade L FIR - Mounayer, Charbel IR - Mounayer C FIR - Saleme, Susanna IR - Saleme S FIR - Macian-Montoro, Francisco IR - Macian-Montoro F FIR - Eker, Omer IR - Eker O FIR - Cotton, François IR - Cotton F FIR - Blanc-Lasserre, Karine IR - Blanc-Lasserre K FIR - Cakmak, Serkan IR - Cakmak S FIR - Cho, Tae-Hee IR - Cho TH FIR - Derex, Laurent IR - Derex L FIR - Lukaszewicz, Anne-Claire IR - Lukaszewicz AC FIR - Mechtouff, Laura IR - Mechtouff L FIR - Nighoghossian, Norbert IR - Nighoghossian N FIR - Philippeau, Frédéric IR - Philippeau F FIR - Riva, Roberto IR - Riva R FIR - Turjman, Francis IR - Turjman F FIR - Vallet, Anne-Evelyne IR - Vallet AE FIR - Carle, Xavier IR - Carle X FIR - Dory-Lautrec, Philippe IR - Dory-Lautrec P FIR - Reyre, Anthony IR - Reyre A FIR - Hak, Jean-François IR - Hak JF FIR - Brunel, Hervé IR - Brunel H FIR - Benali, Amel IR - Benali A FIR - Collemiche, François-Louis IR - Collemiche FL FIR - Dargazanli, Cyril IR - Dargazanli C FIR - Cagnazzo, Frederico IR - Cagnazzo F FIR - Derraz, Imad IR - Derraz I FIR - Arquizan, Caroline IR - Arquizan C FIR - Corti, Lucas IR - Corti L FIR - Costalat, Vincent IR - Costalat V FIR - Gaillard, Nicolas IR - Gaillard N FIR - Gascou, Grégory IR - Gascou G FIR - Lefèvre, Pierre-Henri IR - Lefèvre PH FIR - Mourand, Isabelle IR - Mourand I FIR - Riquelme, Carlos IR - Riquelme C FIR - Derelle, Anne Laure IR - Derelle AL FIR - Gory, Benjamin IR - Gory B FIR - Liao, Liang IR - Liao L FIR - Tonnelet, Romain IR - Tonnelet R FIR - Anxionnat, René IR - Anxionnat R FIR - Bonnerot, Mathieu IR - Bonnerot M FIR - Bracard, Serge IR - Bracard S FIR - Braun, Marc IR - Braun M FIR - Humbertjean, Lisa IR - Humbertjean L FIR - Lacour, Jean-Christophe IR - Lacour JC FIR - Mione, Gioia IR - Mione G FIR - Planel, Sophie IR - Planel S FIR - Richard, Sébastien IR - Richard S FIR - Riou-Comte, Nolwenn IR - Riou-Comte N FIR - Schmitt, Emmanuelle IR - Schmitt E FIR - Bourcier, Romain IR - Bourcier R FIR - Detraz, Lili IR - Detraz L FIR - Desal, Hubert IR - Desal H FIR - Alexandre, Pierre-Louis IR - Alexandre PL FIR - Daumas-Duport, Benjamin IR - Daumas-Duport B FIR - Lenoble, Cédric IR - Lenoble C FIR - Roy, Monica IR - Roy M FIR - Coskun, Oghuzan IR - Coskun O FIR - Di Maria, Frederico IR - Di Maria F FIR - Lapergue, Bertrand IR - Lapergue B FIR - Rodesch, Georges IR - Rodesch G FIR - Wang, Adrien IR - Wang A FIR - Weisenburger-Lile, David IR - Weisenburger-Lile D FIR - Zimatore, Sergio IR - Zimatore S FIR - Ajili, Nadia IR - Ajili N FIR - Buard, Géraldine IR - Buard G FIR - Evrard, Serge IR - Evrard S FIR - Gorza, Lucas IR - Gorza L FIR - Gratieux, Julie IR - Gratieux J FIR - Leguen, Morgan IR - Leguen M FIR - Marinier, Sylvie IR - Marinier S FIR - Pico, Fernando IR - Pico F FIR - Poll, Roxanna IR - Poll R FIR - Rakotoharinandrasana, Haja IR - Rakotoharinandrasana H FIR - Tassan, Philippe IR - Tassan P FIR - Tchikviladze, Maya IR - Tchikviladze M FIR - Delvoye, François IR - Delvoye F FIR - Hebert, Solène IR - Hebert S FIR - Blanc, Raphaël IR - Blanc R FIR - Ciccio, Gabriele IR - Ciccio G FIR - Desilles, Jean-Philippe IR - Desilles JP FIR - Maier, Benjamin IR - Maier B FIR - Mazighi, Mikael IR - Mazighi M FIR - Piotin, Michel IR - Piotin M FIR - Redjem, Hocine IR - Redjem H FIR - Smajda, Stanislas IR - Smajda S FIR - Ben Maacha, Malek IR - Ben Maacha M FIR - Corabianu, Ovide IR - Corabianu O FIR - De Broucker, Thomas IR - De Broucker T FIR - Ille, Olivier IR - Ille O FIR - Manchon, Eric IR - Manchon E FIR - Obadia, Michael IR - Obadia M FIR - Obadia, Mickael IR - Obadia M FIR - Raynouard, Igor IR - Raynouard I FIR - Peres, Roxanne IR - Peres R FIR - Sabben, Candice IR - Sabben C FIR - Smadja, Didier IR - Smadja D FIR - Taylor, Guillaume IR - Taylor G FIR - Thion, Laurie-Anne IR - Thion LA FIR - Lecler, Augustin IR - Lecler A FIR - Spelle, Laurent IR - Spelle L FIR - Denier, Christian IR - Denier C FIR - Caroff, Jildaz IR - Caroff J FIR - Chassin, Olivier IR - Chassin O FIR - Spelle, Laurent IR - Spelle L FIR - Venditti, Laura IR - Venditti L FIR - Aymard, Armand IR - Aymard A FIR - Betty, Jean IR - Betty J FIR - Civelli, Vittorio IR - Civelli V FIR - Eliezer, Michael IR - Eliezer M FIR - Fantoni, Matteo IR - Fantoni M FIR - Houdart, Emmanuel IR - Houdart E FIR - Labeyrie, Marc-Antoine IR - Labeyrie MA FIR - Saint Maurice, Jean-Pierre IR - Saint Maurice JP FIR - Kalsoum, Erwah IR - Kalsoum E FIR - Pacini, Aurelien IR - Pacini A FIR - Ramadane, Chawkat IR - Ramadane C FIR - Tuilier, Titien IR - Tuilier T FIR - Villain, Adrien IR - Villain A FIR - Clarencon, Frédéric IR - Clarencon F FIR - Degos, Vincent IR - Degos V FIR - Elhfnawy, Ahmed IR - Elhfnawy A FIR - Elhorany, Mahmoud IR - Elhorany M FIR - Lenck, Stéphanie IR - Lenck S FIR - Premat, Kevin IR - Premat K FIR - Sourour, Nader-Antoine IR - Sourour NA FIR - Alamowitch, Sonia IR - Alamowitch S FIR - Baronnet, Flore IR - Baronnet F FIR - Crozier, Sophie IR - Crozier S FIR - Deltour, Sandrine IR - Deltour S FIR - Leger, Anne IR - Leger A FIR - Rosso, Charlotte IR - Rosso C FIR - Deltour, Sandrine IR - Deltour S FIR - Leger, Anne IR - Leger A FIR - Rosso, Charlotte IR - Rosso C FIR - Pyatigorskaya, Nadya IR - Pyatigorskaya N FIR - Rodriguez Regent, Christine IR - Rodriguez Regent C FIR - Trystram, Denis IR - Trystram D FIR - Naggara, Olivier IR - Naggara O FIR - Seners, Pierre IR - Seners P FIR - Turc, Guillaume IR - Turc G FIR - Edjlali, Myriam IR - Edjlali M FIR - Agbonon, Rémi IR - Agbonon R FIR - Alotaibi, Mohammed IR - Alotaibi M FIR - Sonchet, Antoine IR - Sonchet A FIR - Oppenheim, Catherine IR - Oppenheim C FIR - Meder, Jean François IR - Meder JF FIR - Benzakoun, Joseph IR - Benzakoun J FIR - Legrand, Laurence IR - Legrand L FIR - Fauché, Cédric IR - Fauché C FIR - Velasco, Stéphane IR - Velasco S FIR - Manceau, Pierre François IR - Manceau PF FIR - Moulin, Sebastien Solène Soize IR - Moulin SSS FIR - Eugene, François IR - Eugene F FIR - Ferre, Jean-Christophe IR - Ferre JC FIR - Paya, Christophe IR - Paya C FIR - Eugene, François IR - Eugene F FIR - Gauvrit, Jean-Yves IR - Gauvrit JY FIR - Langnier-Lemercier, Sophie IR - Langnier-Lemercier S FIR - Lassale, Maria IR - Lassale M FIR - Raoult, Helene IR - Raoult H FIR - Ronziere, Thomas IR - Ronziere T FIR - Tracol, Clément IR - Tracol C FIR - Vannier, Stéphane IR - Vannier S FIR - Burel, Julien IR - Burel J FIR - Le Moal, Julien IR - Le Moal J FIR - Papagiannaki, Chrysanthi IR - Papagiannaki C FIR - Aggour, Mohamed IR - Aggour M FIR - Sachet, Marina IR - Sachet M FIR - Boutet, Claire IR - Boutet C FIR - Beaujeux, Remy IR - Beaujeux R FIR - Hasiu, Anca IR - Hasiu A FIR - Manisor, Monica IR - Manisor M FIR - Mihoc, Dan IR - Mihoc D FIR - Kremer, Stéphane IR - Kremer S FIR - Arteaga, Charles IR - Arteaga C FIR - Gazzola, Sébastien IR - Gazzola S FIR - Darcourt, Jean IR - Darcourt J FIR - Cognard, Christophe IR - Cognard C FIR - Bonneville, Fabrice IR - Bonneville F FIR - Christine Januel, Anne IR - Christine Januel A FIR - Olivot, Jean-Marc IR - Olivot JM FIR - Raposo, Nicolas IR - Raposo N FIR - Viguier, Alain IR - Viguier A FIR - Bibi, Richard IR - Bibi R FIR - Boustia, Fakhreddine IR - Boustia F FIR - Herbreteau, Denis IR - Herbreteau D FIR - Maldonado, Igor IR - Maldonado I FIR - Narata, Ana-Paula IR - Narata AP EDAT- 2020/05/21 06:00 MHDA- 2020/07/07 06:00 CRDT- 2020/05/21 06:00 PHST- 2020/05/21 06:00 [pubmed] PHST- 2020/07/07 06:00 [medline] PHST- 2020/05/21 06:00 [entrez] AID - 10.1161/STROKEAHA.120.030373 [doi] PST - ppublish SO - Stroke. 2020 Jul;51(7):2012-2017. doi: 10.1161/STROKEAHA.120.030373. Epub 2020 May 20. PMID- 32958578 OWN - NLM STAT- MEDLINE DCOM- 20210604 LR - 20260127 IS - 2159-8290 (Electronic) IS - 2159-8274 (Linking) VI - 11 IP - 1 DP - 2021 Jan TI - Triplet Therapy with Palbociclib, Taselisib, and Fulvestrant in PIK3CA-Mutant Breast Cancer and Doublet Palbociclib and Taselisib in Pathway-Mutant Solid Cancers. PG - 92-107 LID - 10.1158/2159-8290.CD-20-0553 [doi] AB - Cyclin-dependent kinase 4/6 (CDK4/6) and PI3K inhibitors synergize in PIK3CA-mutant ER-positive HER2-negative breast cancer models. We conducted a phase Ib trial investigating the safety and efficacy of doublet CDK4/6 inhibitor palbociclib plus selective PI3K inhibitor taselisib in advanced solid tumors, and triplet palbociclib plus taselisib plus fulvestrant in 25 patients with PIK3CA-mutant, ER-positive HER2-negative advanced breast cancer. The triplet therapy response rate in PIK3CA-mutant, ER-positive HER2-negative cancer was 37.5% [95% confidence interval (CI), 18.8-59.4]. Durable disease control was observed in PIK3CA-mutant ER-negative breast cancer and other solid tumors with doublet therapy. Both combinations were well tolerated at pharmacodynamically active doses. In the triplet group, high baseline cyclin E1 expression associated with shorter progression-free survival (PFS; HR = 4.2; 95% CI, 1.3-13.1; P = 0.02). Early circulating tumor DNA (ctDNA) dynamics demonstrated high on-treatment ctDNA association with shorter PFS (HR = 5.2; 95% CI, 1.4-19.4; P = 0.04). Longitudinal plasma ctDNA sequencing provided genomic evolution evidence during triplet therapy. SIGNIFICANCE: The triplet of palbociclib, taselisib, and fulvestrant has promising efficacy in patients with heavily pretreated PIK3CA-mutant ER-positive HER2-negative advanced breast cancer. A subset of patients with PIK3CA-mutant triple-negative breast cancer derived clinical benefit from palbociclib and taselisib doublet, suggesting a potential nonchemotherapy targeted approach for this population.This article is highlighted in the In This Issue feature, p. 1. CI - ©2020 American Association for Cancer Research. FAU - Pascual, Javier AU - Pascual J AUID- ORCID: 0000-0003-3874-2782 AD - Breast Unit, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Lim, Joline S J AU - Lim JSJ AUID- ORCID: 0000-0001-8979-8597 AD - National University Cancer Institute, Singapore (NCIS), National University Hospital, Singapore. FAU - Macpherson, Iain R AU - Macpherson IR AUID- ORCID: 0000-0003-4295-8885 AD - Institute of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom. FAU - Armstrong, Anne C AU - Armstrong AC AUID- ORCID: 0000-0002-0774-7006 AD - Department of Medical Oncology, Christie Hospital NHS Foundation Trust and Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom. FAU - Ring, Alistair AU - Ring A AD - Breast Unit, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. FAU - Okines, Alicia F C AU - Okines AFC AUID- ORCID: 0000-0002-2068-2593 AD - Breast Unit, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. FAU - Cutts, Rosalind J AU - Cutts RJ AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Herrera-Abreu, Maria Teresa AU - Herrera-Abreu MT AUID- ORCID: 0000-0002-6953-3919 AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Garcia-Murillas, Isaac AU - Garcia-Murillas I AUID- ORCID: 0000-0001-8183-7969 AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Pearson, Alex AU - Pearson A AUID- ORCID: 0000-0002-5854-193X AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Hrebien, Sarah AU - Hrebien S AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. FAU - Gevensleben, Heidrun AU - Gevensleben H AD - University Hospital Bonn, Bonn, Germany. FAU - Proszek, Paula Z AU - Proszek PZ AD - Centre for Molecular Pathology, The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Hubank, Michael AU - Hubank M AUID- ORCID: 0000-0002-2901-0742 AD - Centre for Molecular Pathology, The Royal Marsden NHS Foundation Trust, London, United Kingdom. FAU - Hills, Margaret AU - Hills M AD - Ralph Lauren Centre for Breast Cancer Research, Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. FAU - King, Jenny AU - King J AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Parmar, Mona AU - Parmar M AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Prout, Toby AU - Prout T AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Finneran, Laura AU - Finneran L AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Malia, Jason AU - Malia J AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Swales, Karen E AU - Swales KE AUID- ORCID: 0000-0002-6572-1293 AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Ruddle, Ruth AU - Ruddle R AUID- ORCID: 0000-0003-0025-8872 AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Raynaud, Florence I AU - Raynaud FI AD - Division of Cancer Therapeutics, The Institute of Cancer Research, London, United Kingdom. FAU - Turner, Alison AU - Turner A AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Hall, Emma AU - Hall E AUID- ORCID: 0000-0001-5999-5020 AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Yap, Timothy A AU - Yap TA AUID- ORCID: 0000-0002-2154-3309 AD - Department of Investigational Cancer Therapeutics (Phase I Program), The University of Texas MD Anderson Cancer Center, Houston, Texas. FAU - Lopez, Juanita S AU - Lopez JS AD - Division of Clinical Studies, The Institute of Cancer Research, London, United Kingdom. FAU - Turner, Nicholas C AU - Turner NC AD - Breast Unit, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. nick.turner@icr.ac.uk. AD - The Breast Cancer Now Toby Robins Research Centre, The Institute of Cancer Research, London, United Kingdom. AD - Ralph Lauren Centre for Breast Cancer Research, Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom. LA - eng GR - 22897/CRUK_/Cancer Research UK/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20200921 PL - United States TA - Cancer Discov JT - Cancer discovery JID - 101561693 RN - EC 2.7.1.137 (Class I Phosphatidylinositol 3-Kinases) RN - 22X328QOC4 (Fulvestrant) RN - 0 (Imidazoles) RN - 0 (Oxazepines) RN - EC 2.7.1.- (Phosphatidylinositol 3-Kinases) RN - 0 (Piperazines) RN - 0 (Pyridines) RN - EC 2.7.10.1 (Erb-b2 Receptor Tyrosine Kinases) RN - 0 (2-(3-(2-(1-isopropyl-3-methyl-1H-1,2-4-triazol-5-yl)-5,6-dihydrobenzo(f)imidazo(1,2-d)(1,4)oxazepin-9-yl)-1H-pyrazol-1-yl)-2-methylpropanamide) RN - EC 2.7.1.137 (PIK3CA protein, human) RN - G9ZF61LE7G (palbociclib) SB - IM MH - Antineoplastic Combined Chemotherapy Protocols/adverse effects MH - *Breast Neoplasms/drug therapy/genetics MH - Class I Phosphatidylinositol 3-Kinases/genetics MH - Fulvestrant MH - Humans MH - Imidazoles MH - Oxazepines MH - Phosphatidylinositol 3-Kinases MH - Piperazines MH - Pyridines MH - Erb-b2 Receptor Tyrosine Kinases/genetics EDAT- 2020/09/23 06:00 MHDA- 2021/06/05 06:00 CRDT- 2020/09/22 05:37 PHST- 2020/04/30 00:00 [received] PHST- 2020/08/11 00:00 [revised] PHST- 2020/09/16 00:00 [accepted] PHST- 2020/09/23 06:00 [pubmed] PHST- 2021/06/05 06:00 [medline] PHST- 2020/09/22 05:37 [entrez] AID - 2159-8290.CD-20-0553 [pii] AID - 10.1158/2159-8290.CD-20-0553 [doi] PST - ppublish SO - Cancer Discov. 2021 Jan;11(1):92-107. doi: 10.1158/2159-8290.CD-20-0553. Epub 2020 Sep 21. PMID- 36385166 OWN - NLM STAT- MEDLINE DCOM- 20230210 LR - 20240912 IS - 1476-5578 (Electronic) IS - 1359-4184 (Print) IS - 1359-4184 (Linking) VI - 28 IP - 2 DP - 2023 Feb TI - Functional and clinical studies reveal pathophysiological complexity of CLCN4-related neurodevelopmental condition. PG - 668-697 LID - 10.1038/s41380-022-01852-9 [doi] AB - Missense and truncating variants in the X-chromosome-linked CLCN4 gene, resulting in reduced or complete loss-of-function (LOF) of the encoded chloride/proton exchanger ClC-4, were recently demonstrated to cause a neurocognitive phenotype in both males and females. Through international clinical matchmaking and interrogation of public variant databases we assembled a database of 90 rare CLCN4 missense variants in 90 families: 41 unique and 18 recurrent variants in 49 families. For 43 families, including 22 males and 33 females, we collated detailed clinical and segregation data. To confirm causality of variants and to obtain insight into disease mechanisms, we investigated the effect on electrophysiological properties of 59 of the variants in Xenopus oocytes using extended voltage and pH ranges. Detailed analyses revealed new pathophysiological mechanisms: 25% (15/59) of variants demonstrated LOF, characterized by a "shift" of the voltage-dependent activation to more positive voltages, and nine variants resulted in a toxic gain-of-function, associated with a disrupted gate allowing inward transport at negative voltages. Functional results were not always in line with in silico pathogenicity scores, highlighting the complexity of pathogenicity assessment for accurate genetic counselling. The complex neurocognitive and psychiatric manifestations of this condition, and hitherto under-recognized impacts on growth, gastrointestinal function, and motor control are discussed. Including published cases, we summarize features in 122 individuals from 67 families with CLCN4-related neurodevelopmental condition and suggest future research directions with the aim of improving the integrated care for individuals with this diagnosis. CI - © 2022. The Author(s). FAU - Palmer, Elizabeth E AU - Palmer EE AUID- ORCID: 0000-0003-1844-215X AD - Centre for Clinical Genetics, Sydney Children's Hospital Network, Randwick, NSW, Australia. elizabeth.palmer@unsw.edu.au. AD - Discipline of Paediatrics and Child Health, Faculty of Medicine and Health, University of New South Wales, Randwick, NSW, Australia. elizabeth.palmer@unsw.edu.au. FAU - Pusch, Michael AU - Pusch M AUID- ORCID: 0000-0002-8644-8847 AD - Istituto di Biofisica, CNR, Genova, Italy. michael.pusch@ibf.cnr.it. FAU - Picollo, Alessandra AU - Picollo A AD - Istituto di Biofisica, CNR, Genova, Italy. FAU - Forwood, Caitlin AU - Forwood C AD - Centre for Clinical Genetics, Sydney Children's Hospital Network, Randwick, NSW, Australia. FAU - Nguyen, Matthew H AU - Nguyen MH AD - Discipline of Paediatrics and Child Health, Faculty of Medicine and Health, University of New South Wales, Randwick, NSW, Australia. AD - Department of Clinical Genetics, Liverpool Hospital, Liverpool, NSW, Australia. FAU - Suckow, Vanessa AU - Suckow V AD - Max Planck Institute for Molecular Genetics, Group Development and Disease, Berlin, Germany. FAU - Gibbons, Jessica AU - Gibbons J AD - Max Planck Institute for Molecular Genetics, Group Development and Disease, Berlin, Germany. FAU - Hoff, Alva AU - Hoff A AD - Istituto di Biofisica, CNR, Genova, Italy. AD - Department of Biomedical and Clinical Sciences, Linköping University, Linköping, 581 83, Sweden. FAU - Sigfrid, Lisa AU - Sigfrid L AD - Istituto di Biofisica, CNR, Genova, Italy. AD - Department of Biomedical and Clinical Sciences, Linköping University, Linköping, 581 83, Sweden. FAU - Megarbane, Andre AU - Megarbane A AD - Department of Human Genetics, Gilbert and Rose-Marie Chagoury School of Medicine, Lebanese American University, Byblos, Lebanon. AD - Institut Jerome Lejeune, Paris, France. FAU - Nizon, Mathilde AU - Nizon M AD - Service de Génétique Médicale, CHU de Nantes, Nantes Université, Nantes, France. AD - Nantes Université, CNRS, INSERM, l'Institut du Thorax, Nantes, France. FAU - Cogné, Benjamin AU - Cogné B AD - Service de Génétique Médicale, CHU de Nantes, Nantes Université, Nantes, France. AD - Nantes Université, CNRS, INSERM, l'Institut du Thorax, Nantes, France. FAU - Beneteau, Claire AU - Beneteau C AUID- ORCID: 0000-0002-1682-523X AD - Service de Génétique Médicale, CHU de Nantes, Nantes Université, Nantes, France. FAU - Alkuraya, Fowzan S AU - Alkuraya FS AUID- ORCID: 0000-0003-4158-341X AD - Department of Translational Genomics, Center for Genomic Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. FAU - Chedrawi, Aziza AU - Chedrawi A AD - Department of Neurosciences, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. FAU - Hashem, Mais O AU - Hashem MO AD - Department of Translational Genomics, Center for Genomic Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. FAU - Stamberger, Hannah AU - Stamberger H AD - Applied and Translational Neurogenomics Group, VIB Center for Molecular Neurology, VIB, Antwerp, Belgium. AD - Neurology Department, Antwerp University Hospital, Antwerp, Belgium. FAU - Weckhuysen, Sarah AU - Weckhuysen S AD - Applied and Translational Neurogenomics Group, VIB Center for Molecular Neurology, VIB, Antwerp, Belgium. AD - Neurology Department, Antwerp University Hospital, Antwerp, Belgium. AD - Translational Neurosciences, Faculty of Medicine and Health Science, University of Antwerp, Antwerp, Belgium. FAU - Vanlander, Arnaud AU - Vanlander A AD - Department of Child Neurology & Metabolism, Ghent University Hospital, Ghent, Belgium. FAU - Ceulemans, Berten AU - Ceulemans B AD - Department of Pediatric Neurology, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium. FAU - Rajagopalan, Sulekha AU - Rajagopalan S AD - Department of Clinical Genetics, Liverpool Hospital, Liverpool, NSW, Australia. FAU - Nunn, Kenneth AU - Nunn K AD - Children's Hospital at Westmead, Sydney Children's Hospitals Network, Sydney, Australia. FAU - Arpin, Stéphanie AU - Arpin S AD - Service de Génétique Clinique, Centre Hospitalier Régional Universitaire de Tours, Tours, France. FAU - Raynaud, Martine AU - Raynaud M AD - Service de Génétique Clinique, Centre Hospitalier Régional Universitaire de Tours, Tours, France. FAU - Motter, Constance S AU - Motter CS AD - Genetic Center, Akron Children's Hospital, Akron, OH, USA. FAU - Ward-Melver, Catherine AU - Ward-Melver C AD - Genetic Center, Akron Children's Hospital, Akron, OH, USA. FAU - Janssens, Katrien AU - Janssens K AD - Center of Medical Genetics, University Hospital Antwerp/University of Antwerp, Edegem, Belgium. FAU - Meuwissen, Marije AU - Meuwissen M AD - Center of Medical Genetics, University Hospital Antwerp/University of Antwerp, Edegem, Belgium. FAU - Beysen, Diane AU - Beysen D AD - Department of Pediatric Neurology, University Hospital Antwerp/University of Antwerp, Edegem, Belgium. FAU - Dikow, Nicola AU - Dikow N AD - Institute of Human Genetics, Heidelberg University, Heidelberg, Germany. FAU - Grimmel, Mona AU - Grimmel M AD - Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tuebingen, Germany. FAU - Haack, Tobias B AU - Haack TB AD - Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tuebingen, Germany. FAU - Clement, Emma AU - Clement E AD - Department of Clinical Genetics, Great Ormond Street Hospital for Children, London, UK. FAU - McTague, Amy AU - McTague A AUID- ORCID: 0000-0002-0334-2909 AD - Developmental Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK. AD - Department of Neurology, Great Ormond Street Hospital, London, UK. FAU - Hunt, David AU - Hunt D AD - Wessex Clinical Genetics Service, Princess Anne Hospital, Southampton, UK. FAU - Townshend, Sharron AU - Townshend S AD - Genetic Services of WA, King Edward Memorial Hospital, Subiaco, WA, Australia. FAU - Ward, Michelle AU - Ward M AD - Genetic Services of WA, King Edward Memorial Hospital, Subiaco, WA, Australia. FAU - Richards, Linda J AU - Richards LJ AD - Department of Neuroscience, Washington University in St Louis School of Medicine, St Louis, MI, USA. AD - The University of Queensland, Queensland Brain Institute, St Lucia, QLD, Australia. FAU - Simons, Cas AU - Simons C AD - Centre for Population Genomics, Murdoch Children's Research Institute, Melbourne, Australia. AD - Garvan Institute of Medical Research, UNSW, Sydney, NSW, Australia. FAU - Costain, Gregory AU - Costain G AD - Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, ON, Canada. FAU - Dupuis, Lucie AU - Dupuis L AUID- ORCID: 0000-0002-6068-8199 AD - Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, ON, Canada. FAU - Mendoza-Londono, Roberto AU - Mendoza-Londono R AD - Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, ON, Canada. FAU - Dudding-Byth, Tracy AU - Dudding-Byth T AD - Genetics of Learning Disability Service, Newcastle, NSW, Australia. AD - University of Newcastle Grow Up Well Priority Research Centre, Newcastle, NSW, Australia. FAU - Boyle, Jackie AU - Boyle J AD - Genetics of Learning Disability Service, Newcastle, NSW, Australia. FAU - Saunders, Carol AU - Saunders C AD - Department of Pathology and Laboratory Medicine, Children's Mercy Hospital and Clinics, MI, Kansas City, USA. AD - Kansas City School of Medicine, University of Missouri, Kansas City, MI, USA. FAU - Fleming, Emily AU - Fleming E AD - Division of Clinical Genetics, Children's Mercy Hospital and Clinics, Kansas City, MI, USA. FAU - El Chehadeh, Salima AU - El Chehadeh S AUID- ORCID: 0000-0003-1613-6570 AD - Service de Génétique Médicale, Institut de Génétique Médicale d'Alsace (IGMA), Hôpitaux Universitaires de Strasbourg, Strasbourg, France. AD - Laboratoire de Génétique Médicale, UMRS_1112, Institut de Génétique Médicale d'Alsace (IGMA), Université de Strasbourg et INSERM, Strasbourg, France. FAU - Spitz, Marie-Aude AU - Spitz MA AD - Service de Pédiatrie, Hôpital de Hautepierre, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. FAU - Piton, Amelie AU - Piton A AD - Laboratoires de Diagnostic Génétique, Institut de Génétique Médicale d'Alsace (IGMA), Hôpitaux Universitaires de Strasbourg, Nouvel Hôpital Civil, Strasbourg, France. FAU - Gerard, Bénédicte AU - Gerard B AD - Laboratoires de Diagnostic Génétique, Institut de Génétique Médicale d'Alsace (IGMA), Hôpitaux Universitaires de Strasbourg, Nouvel Hôpital Civil, Strasbourg, France. FAU - Abi Warde, Marie-Thérèse AU - Abi Warde MT AD - Service de Pédiatrie, Hôpital de Hautepierre, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. AD - Pediatric Neurology Department, CHU de Strasbourg, Strasbourg, France. FAU - Rea, Gillian AU - Rea G AD - Northern Ireland Regional Genetics Service, Belfast, Northern Ireland. FAU - McKenna, Caoimhe AU - McKenna C AD - Northern Ireland Regional Genetics Service, Belfast, Northern Ireland. FAU - Douzgou, Sofia AU - Douzgou S AD - Department of Medical Genetics, Haukeland University Hospital, Bergen, Norway. AD - Division of Evolution, Infection and Genomics, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. FAU - Banka, Siddharth AU - Banka S AUID- ORCID: 0000-0002-8527-2210 AD - Division of Evolution, Infection and Genomics, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. AD - Manchester Centre for Genomic Medicine, Saint Mary's Hospital, Manchester University NHS Foundation Trust, Manchester, UK. FAU - Akman, Cigdem AU - Akman C AD - Department of Neurology, Division of Child Neurology, Columbia University Irving Medical Center, New York, USA. FAU - Bain, Jennifer M AU - Bain JM AUID- ORCID: 0000-0002-6642-8902 AD - Department of Neurology, Division of Child Neurology, Columbia University Irving Medical Center, New York, USA. FAU - Sands, Tristan T AU - Sands TT AUID- ORCID: 0000-0001-7285-1195 AD - Department of Neurology, Division of Child Neurology, Columbia University Irving Medical Center, New York, USA. FAU - Wilson, Golder N AU - Wilson GN AD - Texas Tech Health Sciences Center Lubbock and KinderGenome Medical Genetics, Dallas, TX, USA. FAU - Silvertooth, Erin J AU - Silvertooth EJ AD - Texas Sports Psychiatry and Integrative Health, Austin, TX, USA. FAU - Miller, Lauren AU - Miller L AD - Hillcrest Internal Medicine, Waco, TX, USA. FAU - Lederer, Damien AU - Lederer D AD - Centre de Génétique Humaine, Institut de Pathologie et de Génétique ASBL, Gosselies, Belgium. FAU - Sachdev, Rani AU - Sachdev R AD - Centre for Clinical Genetics, Sydney Children's Hospital Network, Randwick, NSW, Australia. AD - Discipline of Paediatrics and Child Health, Faculty of Medicine and Health, University of New South Wales, Randwick, NSW, Australia. FAU - Macintosh, Rebecca AU - Macintosh R AD - Centre for Clinical Genetics, Sydney Children's Hospital Network, Randwick, NSW, Australia. AD - Discipline of Paediatrics and Child Health, Faculty of Medicine and Health, University of New South Wales, Randwick, NSW, Australia. FAU - Monestier, Olivier AU - Monestier O AUID- ORCID: 0000-0002-3236-0807 AD - Centre de Génétique Humaine, Institut de Pathologie et de Génétique ASBL, Gosselies, Belgium. FAU - Karadurmus, Deniz AU - Karadurmus D AUID- ORCID: 0000-0001-8663-6357 AD - Centre de Génétique Humaine, Institut de Pathologie et de Génétique ASBL, Gosselies, Belgium. FAU - Collins, Felicity AU - Collins F AD - Department of Medical Genomics/Clinical Genetics, Royal Prince Alfred Hospital, Camperdown, Sydney, NSW, Australia. FAU - Carter, Melissa AU - Carter M AD - Department of Genetics, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. FAU - Rohena, Luis AU - Rohena L AD - Division of Medical Genetics, Department of Pediatrics, San Antonio Military Medical Center, San Antonio, TX, USA. AD - Department of Pediatrics, Long School of Medicine-UT Health San Antonio, San Antonio, TX, USA. FAU - Willemsen, Marjolein H AU - Willemsen MH AD - Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Ockeloen, Charlotte W AU - Ockeloen CW AD - Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Pfundt, Rolph AU - Pfundt R AD - Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Kroft, Sanne D AU - Kroft SD AD - Pluryn, Residential Care Setting, Groesbeek, The Netherlands. FAU - Field, Michael AU - Field M AUID- ORCID: 0000-0001-7455-934X AD - Genetics of Learning Disability Service, Newcastle, NSW, Australia. FAU - Laranjeira, Francisco E R AU - Laranjeira FER AD - Centro de Genética Médica Jacinto Magalhães, Centro Hospitalar Universitário do Porto, Porto, Portugal. FAU - Fortuna, Ana M AU - Fortuna AM AD - Unit for Multidisciplinary Research in Biomedicine, School of Medicine and Biomedical Sciences, Porto University, Porto, Portugal. FAU - Soares, Ana R AU - Soares AR AUID- ORCID: 0000-0001-7817-9889 AD - Unit for Multidisciplinary Research in Biomedicine, School of Medicine and Biomedical Sciences, Porto University, Porto, Portugal. FAU - Michaud, Vincent AU - Michaud V AUID- ORCID: 0000-0002-5788-392X AD - Service de Génétique Médicale, CHU Bordeaux, Bordeaux, France. AD - INSERM U1211, Laboratoire Maladies Rares: Génétique et Métabolisme, Bordeaux, Univ., Bordeaux, France. FAU - Naudion, Sophie AU - Naudion S AD - Service de Génétique Médicale, CHU Bordeaux, Bordeaux, France. FAU - Golla, Sailaja AU - Golla S AD - Child Neurology and Neurodevelopmental Medicine Thompson Autism Center, CHOC Hospital, Orange County, CA, USA. FAU - Weaver, David D AU - Weaver DD AD - Indiana University School of Medicine, Indianapolis, USA. FAU - Bird, Lynne M AU - Bird LM AUID- ORCID: 0000-0003-4833-3747 AD - University of California, San Diego, Rady Children's Hospital San Diego, San Diego, CA, USA. FAU - Friedman, Jennifer AU - Friedman J AD - University of California, San Diego, Rady Children's Hospital San Diego, San Diego, CA, USA. FAU - Clowes, Virginia AU - Clowes V AD - North West Thames Regional Genetics Service, London North West University Healthcare NHS Trust, Harrow, London, UK. AD - Imperial College London, London, UK. FAU - Joss, Shelagh AU - Joss S AD - West of Scotland Centre for Genomic Medicine, Queen Elizabeth University Hospital, Glasgow, UK. FAU - Pölsler, Laura AU - Pölsler L AUID- ORCID: 0000-0002-1796-2287 AD - Centrum Medische Genetica, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel (VUB), Brussels, Belgium. FAU - Campeau, Philippe M AU - Campeau PM AUID- ORCID: 0000-0001-9713-7107 AD - CHU Sainte-Justine Research Center, University of Montreal, Montreal, QC, Canada. FAU - Blazo, Maria AU - Blazo M AD - Division Clinical Genetics Texas A&M University Health Science Center, College Station, TX, USA. FAU - Bijlsma, Emilia K AU - Bijlsma EK AD - Department of Clinical Genetics, Leiden University Medical Centre, Leiden, The Netherlands. FAU - Rosenfeld, Jill A AU - Rosenfeld JA AD - Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA. AD - Baylor Genetics Laboratories, Houston, TX, USA. FAU - Beetz, Christian AU - Beetz C AD - Centogene GmbH, Rostock, Germany. FAU - Powis, Zöe AU - Powis Z AD - Clinical Genomics, Ambry Genetics, Aliso Viejo, CA, USA. FAU - McWalter, Kirsty AU - McWalter K AUID- ORCID: 0000-0002-1654-9036 AD - GeneDx LLC, Gaithersburg, MA, USA. FAU - Brandt, Tracy AU - Brandt T AD - GeneDx LLC, Gaithersburg, MA, USA. FAU - Torti, Erin AU - Torti E AD - GeneDx LLC, Gaithersburg, MA, USA. FAU - Mathot, Mikaël AU - Mathot M AD - Neuropediatric Unit, CHU UCL-Namur, Namur, Belgium. FAU - Mohammad, Shekeeb S AU - Mohammad SS AUID- ORCID: 0000-0003-1219-4781 AD - Children's Hospital at Westmead, Sydney Children's Hospitals Network, Sydney, Australia. AD - Children's Hospital at Westmead, Sydney Children's Hospitals Network, Sydney, NSW, Australia. FAU - Armstrong, Ruth AU - Armstrong R AD - East Anglian Medical Genetics Service, Clinical Genetics, Addenbrooke's Treatment Centre, Addenbrooke's Hospital, Cambridge, UK. FAU - Kalscheuer, Vera M AU - Kalscheuer VM AUID- ORCID: 0000-0001-6898-3259 AD - Max Planck Institute for Molecular Genetics, Group Development and Disease, Berlin, Germany. kalscheu@molgen.mpg.de. LA - eng GR - WT_/Wellcome Trust/United Kingdom GR - DH_/Department of Health/United Kingdom GR - MR/T007087/1/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20221116 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (CLCN4 protein, human) RN - 0 (Chloride Channels) SB - IM MH - Male MH - Female MH - Humans MH - *Neurodevelopmental Disorders/genetics MH - Mutation, Missense MH - Genes, X-Linked MH - Phenotype MH - Chloride Channels/genetics PMC - PMC9908558 COIS- SW received consultancy fees from UCB, Biocodex, Xenon Pharmaceuticals, Zogenix, Lundbeck, Knopp Biosciences, and Encoded Therapeutics. KMc, TB, and ET are employees of GeneDx. ZP is an employee of Ambry. CB is an employee of Centogene, GmbH. The Department of Molecular and Human Genetics at Baylor College of Medicine receives revenue from clinical genetic testing completed at Baylor Genetics Laboratories. All other authors declare no competing interests. EDAT- 2022/11/18 06:00 MHDA- 2023/02/11 06:00 PMCR- 2022/11/16 CRDT- 2022/11/17 10:36 PHST- 2022/04/05 00:00 [received] PHST- 2022/10/21 00:00 [accepted] PHST- 2022/10/10 00:00 [revised] PHST- 2022/11/18 06:00 [pubmed] PHST- 2023/02/11 06:00 [medline] PHST- 2022/11/17 10:36 [entrez] PHST- 2022/11/16 00:00 [pmc-release] AID - 10.1038/s41380-022-01852-9 [pii] AID - 1852 [pii] AID - 10.1038/s41380-022-01852-9 [doi] PST - ppublish SO - Mol Psychiatry. 2023 Feb;28(2):668-697. doi: 10.1038/s41380-022-01852-9. Epub 2022 Nov 16. PMID- 25644381 OWN - NLM STAT- MEDLINE DCOM- 20160916 LR - 20250103 IS - 1476-5578 (Electronic) IS - 1359-4184 (Print) IS - 1359-4184 (Linking) VI - 21 IP - 1 DP - 2016 Jan TI - X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes. PG - 133-48 LID - 10.1038/mp.2014.193 [doi] AB - X-linked intellectual disability (XLID) is a clinically and genetically heterogeneous disorder. During the past two decades in excess of 100 X-chromosome ID genes have been identified. Yet, a large number of families mapping to the X-chromosome remained unresolved suggesting that more XLID genes or loci are yet to be identified. Here, we have investigated 405 unresolved families with XLID. We employed massively parallel sequencing of all X-chromosome exons in the index males. The majority of these males were previously tested negative for copy number variations and for mutations in a subset of known XLID genes by Sanger sequencing. In total, 745 X-chromosomal genes were screened. After stringent filtering, a total of 1297 non-recurrent exonic variants remained for prioritization. Co-segregation analysis of potential clinically relevant changes revealed that 80 families (20%) carried pathogenic variants in established XLID genes. In 19 families, we detected likely causative protein truncating and missense variants in 7 novel and validated XLID genes (CLCN4, CNKSR2, FRMPD4, KLHL15, LAS1L, RLIM and USP27X) and potentially deleterious variants in 2 novel candidate XLID genes (CDK16 and TAF1). We show that the CLCN4 and CNKSR2 variants impair protein functions as indicated by electrophysiological studies and altered differentiation of cultured primary neurons from Clcn4(-/-) mice or after mRNA knock-down. The newly identified and candidate XLID proteins belong to pathways and networks with established roles in cognitive function and intellectual disability in particular. We suggest that systematic sequencing of all X-chromosomal genes in a cohort of patients with genetic evidence for X-chromosome locus involvement may resolve up to 58% of Fragile X-negative cases. FAU - Hu, H AU - Hu H AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Haas, S A AU - Haas SA AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Chelly, J AU - Chelly J AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Van Esch, H AU - Van Esch H AD - Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium. FAU - Raynaud, M AU - Raynaud M AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - de Brouwer, A P M AU - de Brouwer AP AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands. FAU - Weinert, S AU - Weinert S AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Froyen, G AU - Froyen G AD - Human Genome Laboratory, VIB Center for the Biology of Disease, Leuven, Belgium. AD - Human Genome Laboratory, Department of Human Genetics, K.U. Leuven, Leuven, Belgium. FAU - Frints, S G M AU - Frints SG AD - Department of Clinical Genetics, Maastricht University Medical Center, azM, Maastricht, The Netherlands. AD - School for Oncology and Developmental Biology, GROW, Maastricht University, Maastricht, The Netherlands. FAU - Laumonnier, F AU - Laumonnier F AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. FAU - Zemojtel, T AU - Zemojtel T AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Love, M I AU - Love MI AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Richard, H AU - Richard H AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Emde, A-K AU - Emde AK AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Bienek, M AU - Bienek M AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Jensen, C AU - Jensen C AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Hambrock, M AU - Hambrock M AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Fischer, U AU - Fischer U AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Langnick, C AU - Langnick C AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. FAU - Feldkamp, M AU - Feldkamp M AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. FAU - Wissink-Lindhout, W AU - Wissink-Lindhout W AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands. FAU - Lebrun, N AU - Lebrun N AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Castelnau, L AU - Castelnau L AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Rucci, J AU - Rucci J AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Montjean, R AU - Montjean R AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Dorseuil, O AU - Dorseuil O AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Billuart, P AU - Billuart P AD - University Paris Descartes, Paris, France. AD - Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut National de la Santé et de la Recherche Médicale Unité 1016, Institut Cochin, Paris, France. FAU - Stuhlmann, T AU - Stuhlmann T AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Shaw, M AU - Shaw M AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Corbett, M A AU - Corbett MA AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Gardner, A AU - Gardner A AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Willis-Owen, S AU - Willis-Owen S AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - National Heart and Lung Institute, Imperial College London, London, UK. FAU - Tan, C AU - Tan C AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. FAU - Friend, K L AU - Friend KL AD - SA Pathology, Women's and Children's Hospital, Adelaide, SA, Australia. FAU - Belet, S AU - Belet S AD - Human Genome Laboratory, VIB Center for the Biology of Disease, Leuven, Belgium. AD - Human Genome Laboratory, Department of Human Genetics, K.U. Leuven, Leuven, Belgium. FAU - van Roozendaal, K E P AU - van Roozendaal KE AD - Department of Clinical Genetics, Maastricht University Medical Center, azM, Maastricht, The Netherlands. AD - School for Oncology and Developmental Biology, GROW, Maastricht University, Maastricht, The Netherlands. FAU - Jimenez-Pocquet, M AU - Jimenez-Pocquet M AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Moizard, M-P AU - Moizard MP AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Ronce, N AU - Ronce N AD - Inserm U930 'Imaging and Brain', Tours, France. AD - University François-Rabelais, Tours, France. AD - Centre Hospitalier Régional Universitaire, Service de Génétique, Tours, France. FAU - Sun, R AU - Sun R AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - O'Keeffe, S AU - O'Keeffe S AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Chenna, R AU - Chenna R AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - van Bömmel, A AU - van Bömmel A AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Göke, J AU - Göke J AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Hackett, A AU - Hackett A AD - Genetics of Learning and Disability Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Field, M AU - Field M AD - Genetics of Learning and Disability Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Christie, L AU - Christie L AD - Genetics of Learning and Disability Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Boyle, J AU - Boyle J AD - Genetics of Learning and Disability Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Haan, E AU - Haan E AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - SA Pathology, Women's and Children's Hospital, Adelaide, SA, Australia. FAU - Nelson, J AU - Nelson J AD - Genetic Services of Western Australia, King Edward Memorial Hospital, Perth, WA, Australia. FAU - Turner, G AU - Turner G AD - Genetics of Learning and Disability Service, Hunter Genetics, Waratah, NSW, Australia. FAU - Baynam, G AU - Baynam G AD - Genetic Services of Western Australia, King Edward Memorial Hospital, Perth, WA, Australia. AD - School of Paediatrics and Child Health, University of Western Australia, Perth, WA, Australia. AD - Institute for Immunology and Infectious Diseases, Murdoch University, Perth, WA, Australia. AD - Telethon Kids Institute, Perth, WA, Australia. FAU - Gillessen-Kaesbach, G AU - Gillessen-Kaesbach G AD - Institut für Humangenetik, Universität zu Lübeck, Lübeck, Germany. FAU - Müller, U AU - Müller U AD - Institut für Humangenetik, Justus-Liebig-Universität Giessen, Giessen, Germany. AD - bio.logis Center for Human Genetics, Frankfurt a. M., Germany. FAU - Steinberger, D AU - Steinberger D AD - Institut für Humangenetik, Justus-Liebig-Universität Giessen, Giessen, Germany. AD - bio.logis Center for Human Genetics, Frankfurt a. M., Germany. FAU - Budny, B AU - Budny B AD - Chair and Department of Endocrinology, Metabolism and Internal Diseases, Ponzan University of Medical Sciences, Poznan, Poland. FAU - Badura-Stronka, M AU - Badura-Stronka M AD - Chair and Department of Medical Genetics, Poznan University of Medical Sciences, Poznan, Poland. FAU - Latos-Bieleńska, A AU - Latos-Bieleńska A AD - Chair and Department of Medical Genetics, Poznan University of Medical Sciences, Poznan, Poland. FAU - Ousager, L B AU - Ousager LB AD - Department of Clinical Genetics, Odense University Hospital, Odense, Denmark. FAU - Wieacker, P AU - Wieacker P AD - Institut für Humangenetik, Universitätsklinikum Münster, Muenster, Germany. FAU - Rodríguez Criado, G AU - Rodríguez Criado G AD - Unidad de Genética Clínica, Hospital Virgen del Rocío, Sevilla, España. FAU - Bondeson, M-L AU - Bondeson ML AD - Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden. FAU - Annerén, G AU - Annerén G AD - Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden. FAU - Dufke, A AU - Dufke A AD - Institut für Medizinische Genetik und Angewandte Genomik, Tübingen, Germany. FAU - Cohen, M AU - Cohen M AD - Kinderzentrum München, München, Germany. FAU - Van Maldergem, L AU - Van Maldergem L AD - Centre de Génétique Humaine, Université de Franche-Comté, Besançon, France. FAU - Vincent-Delorme, C AU - Vincent-Delorme C AD - Service de Génétique, Hôpital Jeanne de Flandre CHRU de Lilles, Lille, France. FAU - Echenne, B AU - Echenne B AD - Service de Neuro-Pédiatrie, CHU Montpellier, Montpellier, France. FAU - Simon-Bouy, B AU - Simon-Bouy B AD - Laboratoire SESEP, Centre hospitalier de Versailles, Le Chesnay, France. FAU - Kleefstra, T AU - Kleefstra T AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands. FAU - Willemsen, M AU - Willemsen M AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands. FAU - Fryns, J-P AU - Fryns JP AD - Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium. FAU - Devriendt, K AU - Devriendt K AD - Center for Human Genetics, University Hospitals Leuven, Leuven, Belgium. FAU - Ullmann, R AU - Ullmann R AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Vingron, M AU - Vingron M AD - Department of Computational Molecular Biology, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Wrogemann, K AU - Wrogemann K AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. AD - Department of Biochemistry and Medical Genetics, University of Manitoba, Winnipeg, MB, Canada. FAU - Wienker, T F AU - Wienker TF AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Tzschach, A AU - Tzschach A AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - van Bokhoven, H AU - van Bokhoven H AD - Department of Human Genetics, Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands. FAU - Gecz, J AU - Gecz J AUID- ORCID: 0000-0002-7884-6861 AD - School of Paediatrics and Reproductive Health, The University of Adelaide, Adelaide, SA, Australia. AD - Robinson Research Institute, The University of Adelaide, Adelaide, SA, Australia. FAU - Jentsch, T J AU - Jentsch TJ AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. AD - Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. FAU - Chen, W AU - Chen W AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. AD - Max-Delbrück-Centrum für Molekulare Medizin, Berlin, Germany. FAU - Ropers, H-H AU - Ropers HH AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. FAU - Kalscheuer, V M AU - Kalscheuer VM AD - Department of Human Molecular Genetics, Max Planck Institute for Molecular Genetics, Berlin, Germany. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150203 PL - England TA - Mol Psychiatry JT - Molecular psychiatry JID - 9607835 RN - 0 (Adaptor Proteins, Signal Transducing) RN - 0 (CLC-5 chloride channel) RN - 0 (CLCN4 protein, human) RN - 0 (CNKSR2 protein, human) RN - 0 (Chloride Channels) RN - 0 (FRMPD4 protein, human) RN - 0 (Intracellular Signaling Peptides and Proteins) RN - 0 (KLHL15 protein, human) RN - 0 (Las1L protein, human) RN - 0 (Microfilament Proteins) RN - 0 (Nuclear Proteins) RN - 0 (RNA, Messenger) RN - 0 (TATA-Binding Protein Associated Factors) RN - 0 (Transcription Factor TFIID) RN - EC 2.3.1.48 (Histone Acetyltransferases) RN - EC 2.3.2.27 (RLIM protein, human) RN - EC 2.3.2.27 (Ubiquitin-Protein Ligases) RN - EC 2.7.11.1 (TATA-binding protein associated factor 250 kDa) RN - EC 2.7.11.22 (Cyclin-Dependent Kinases) RN - EC 2.7.11.22 (PCTAIRE-1 protein kinase) SB - IM MH - Adaptor Proteins, Signal Transducing/genetics MH - Adolescent MH - Adult MH - Animals MH - Cells, Cultured MH - Chloride Channels/genetics/metabolism MH - Cohort Studies MH - Cyclin-Dependent Kinases/genetics MH - *Genetic Variation MH - High-Throughput Nucleotide Sequencing MH - Histone Acetyltransferases/genetics MH - Humans MH - Intracellular Signaling Peptides and Proteins/genetics MH - Male MH - X-Linked Intellectual Disability/*genetics MH - Mice, Knockout MH - Microfilament Proteins/genetics MH - Neurons/metabolism/pathology MH - Nuclear Proteins/genetics MH - RNA, Messenger/metabolism MH - TATA-Binding Protein Associated Factors/genetics MH - Transcription Factor TFIID/genetics MH - Ubiquitin-Protein Ligases/genetics PMC - PMC5414091 COIS- The authors declare no conflict of interest. EDAT- 2015/02/04 06:00 MHDA- 2016/09/17 06:00 PMCR- 2017/05/03 CRDT- 2015/02/04 06:00 PHST- 2014/08/04 00:00 [received] PHST- 2014/11/17 00:00 [revised] PHST- 2014/12/08 00:00 [accepted] PHST- 2015/02/04 06:00 [entrez] PHST- 2015/02/04 06:00 [pubmed] PHST- 2016/09/17 06:00 [medline] PHST- 2017/05/03 00:00 [pmc-release] AID - mp2014193 [pii] AID - 10.1038/mp.2014.193 [doi] PST - ppublish SO - Mol Psychiatry. 2016 Jan;21(1):133-48. doi: 10.1038/mp.2014.193. Epub 2015 Feb 3. PMID- 36108261 OWN - NLM STAT- MEDLINE DCOM- 20220919 LR - 20221222 IS - 2473-4284 (Electronic) IS - 2473-4284 (Linking) VI - 6 DP - 2022 Sep TI - Transcriptomics in Tumor and Normal Lung Tissues Identify Patients With Early-Stage Non-Small-Cell Lung Cancer With High Risk of Postsurgery Recurrence Who May Benefit From Adjuvant Therapies. PG - e2200072 LID - 10.1200/PO.22.00072 [doi] LID - e2200072 AB - PURPOSE: The prognosis of patients with non-small-cell lung cancer (NSCLC), traditionally determined by anatomic histology and TNM staging, neglects the biological features of the tumor that may be important in determining patient outcome and guiding therapeutic interventions. Identifying patients with NSCLC at increased risk of recurrence after curative-intent surgery remains an important unmet need so that known effective adjuvant treatments can be offered to those at highest risk of recurrence. METHODS: Relative gene expression level in the primary tumor and normal bronchial tissues was used to retrospectively assess their association with disease-free survival (DFS) in a cohort of 120 patients with NSCLC who underwent curative-intent surgery. RESULTS: Low versus high Digital Display Precision Predictor (DDPP) score (a measure of relative gene expression) was significantly associated with shorter DFS (highest recurrence risk; P = .006) in all patients and in patients with TNM stages 1-2 (P = .00051; n = 83). For patients with stages 1-2 and low DDPP score (n = 29), adjuvant chemotherapy was associated with improved DFS (P = .0041). High co-overexpression of CTLA-4, PD-L1, and ICOS in normal lung (28 of 120 patients) was also significantly associated with decreased DFS (P = .0013), suggesting an immune tolerance to tumor neoantigens in some patients. Patients with DDPP low and immunotolerant normal tissue had the shortest DFS (P = 2.12E-11). CONCLUSION: TNM stage, DDPP score, and immune competence status of normal lung are independent prognostic factors in multivariate analysis. Our findings open new avenues for prospective prognostic assessment and treatment assignment on the basis of transcriptomic profiling of tumor and normal lung tissue in patients with NSCLC. FAU - Lazar, Vladimir AU - Lazar V AUID- ORCID: 0000-0003-2375-1088 AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. FAU - Girard, Nicolas AU - Girard N AD - Institut Curie, Paris, France. AD - Institut du Thorax Curie-Institut Montsouris, Paris, France. FAU - Raymond, Eric AU - Raymond E AD - Groupe Hospitalier Saint-Joseph, Paris, France. FAU - Martini, Jean-François AU - Martini JF AUID- ORCID: 0000-0001-8570-1401 AD - Pfizer Inc, San Diego, CA. FAU - Galbraith, Susan AU - Galbraith S AD - AstraZeneca Plc, Cambridge, United Kingdom. FAU - Raynaud, Jacques AU - Raynaud J AUID- ORCID: 0000-0002-9854-8124 AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. FAU - Bresson, Catherine AU - Bresson C AUID- ORCID: 0000-0002-7523-849X AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. FAU - Solomon, Benjamin AU - Solomon B AUID- ORCID: 0000-0002-6014-1775 AD - Avera Cancer Institute, Sioux Falls, SD. FAU - Magidi, Shai AU - Magidi S AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. FAU - Nechushtan, Hovav AU - Nechushtan H AD - Hadassah Sharett Institute of Oncology, Jerusalem, Israel. FAU - Onn, Amir AU - Onn A AUID- ORCID: 0000-0002-6602-0405 AD - Sheba Medical Center, Tel-Hashomer, Israel. FAU - Berger, Raanan AU - Berger R AD - Sheba Medical Center, Tel-Hashomer, Israel. FAU - Chen, Haiquan AU - Chen H AUID- ORCID: 0000-0001-7846-257X AD - Fudan University Shanghai Cancer Center, Shanghai, China. FAU - Al-Omari, Amal AU - Al-Omari A AUID- ORCID: 0000-0001-7571-3097 AD - King Hussein Cancer Center, Amman, Jordan. FAU - Ikeda, Sadakatsu AU - Ikeda S AD - Tokyo Medical and Dental University, Tokyo, Japan. FAU - Lassen, Ulrik AU - Lassen U AUID- ORCID: 0000-0002-3865-4574 AD - Rigshospitalet, Copenhagen, Denmark. FAU - Sekacheva, Marina AU - Sekacheva M AD - Sechenov First State Medical University, Moscow, Russia. FAU - Felip, Enriqueta AU - Felip E AUID- ORCID: 0000-0002-7620-0098 AD - Vall d'Hebron Hospital Campus and Institute of Oncology (VHIO), UVic-UCC, Barcelona, Spain. FAU - Tabernero, Josep AU - Tabernero J AUID- ORCID: 0000-0002-2495-8139 AD - Vall d'Hebron Hospital Campus and Institute of Oncology (VHIO), UVic-UCC, Barcelona, Spain. FAU - Batist, Gerald AU - Batist G AD - Segal Cancer Center, Jewish General Hospital, McGill University, Montréal, Canada. FAU - Spatz, Alan AU - Spatz A AUID- ORCID: 0000-0003-3020-1420 AD - Segal Cancer Center, Jewish General Hospital, McGill University, Montréal, Canada. FAU - Pramesh, C S AU - Pramesh CS AUID- ORCID: 0000-0002-3635-0083 AD - Tata Memorial Hospital, Tata Memorial Center, Homi Bhabha National Institute, Mumbai, India. FAU - Girard, Philippe AU - Girard P AUID- ORCID: 0000-0002-1559-8055 AD - Institut Mutualiste Montsouris, Paris, France. FAU - Blay, Jean-Yves AU - Blay JY AUID- ORCID: 0000-0001-7190-120X AD - Center Leon-Bérard, Lyon, France. AD - Unicancer, Paris, France. FAU - Philip, Thierry AU - Philip T AD - Pfizer Inc, San Diego, CA. FAU - Berindan-Neagoe, Ioana AU - Berindan-Neagoe I AUID- ORCID: 0000-0001-5828-1325 AD - Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania. FAU - Porgador, Angel AU - Porgador A AD - Ben-Gurion University of the Negev, Be'er Sheva, Israel. FAU - Rubin, Eitan AU - Rubin E AUID- ORCID: 0000-0002-7807-4005 AD - Ben-Gurion University of the Negev, Be'er Sheva, Israel. FAU - Kurzrock, Razelle AU - Kurzrock R AUID- ORCID: 0000-0003-4110-1214 AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. FAU - Schilsky, Richard L AU - Schilsky RL AUID- ORCID: 0000-0003-4474-4303 AD - Worldwide Innovative Network-WIN Consortium, Villejuif, France. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - JCO Precis Oncol JT - JCO precision oncology JID - 101705370 RN - 0 (B7-H1 Antigen) RN - 0 (CTLA-4 Antigen) SB - IM MH - B7-H1 Antigen/analysis MH - CTLA-4 Antigen/genetics MH - *Carcinoma, Non-Small-Cell Lung/genetics MH - Humans MH - Lung/chemistry MH - *Lung Neoplasms/drug therapy MH - Prospective Studies MH - Retrospective Studies MH - *Small Cell Lung Carcinoma MH - Transcriptome PMC - PMC9489166 COIS- Vladimir LazarPatents, Royalties, Other Intellectual Property: I am inventor on patentes filed by WIN Consortium I do not receive money Nicolas GirardEmployment: AstraZeneca (I)Consulting or Advisory Role: Roche, Lilly, AstraZeneca, Novartis, Pfizer, Bristol Myers Squibb, MSD, Takeda, Janssen, Sanofi, AmgenResearch Funding: Roche (Inst), AstraZeneca (Inst), BMS (Inst)Travel, Accommodations, Expenses: Roche Eric RaymondEmployment: Genoscience Pharma, SCOR, StromaCare, Onward TherapeuticsLeadership: AFR-OncologyStock and Other Ownership Interests: Neuronax, Oncoethix, Genoscience Pharma, SCORConsulting or Advisory Role: Lilly, Pfizer, Merck Serono, PharmaEngine, Novartis/lpsen, Celgene Jean-François MartiniEmployment: PfizerStock and Other Ownership Interests: Pfizer Susan GalbraithEmployment: AstraZenecaLeadership: BB Biotech VenturesStock and Other Ownership Interests: AstraZeneca Benjamin SolomonConsulting or Advisory Role: BayerResearch Funding: AstraZeneca/Merck (Inst)Open Payments Link: https://openpaymentsdata.cms.gov/physician/358846 Hovav NechushtanResearch Funding: AstraZeneca (Inst), Spectrum Pharmaceuticals (Inst), Lilly (Inst), Merck KGaA (Inst)Uncompensated Relationships: MSD Amir OnnHonoraria: Boehringer Ingelheim, Roche, MSD, AmgenConsulting or Advisory Role: Boehringer Ingelheim, MSDSpeakers' Bureau: AstraZeneca Raanan BergerStock and Other Ownership Interests: BelongHonoraria: Bristol Myers Squibb, Roche Israel, AstraZeneca, Pfizer, MSDConsulting or Advisory Role: Belong, MSD Oncology, BMSSpeakers' Bureau: Mitra Biotech, BMS, MSD OncologyTravel, Accommodations, Expenses: Mitra Biotech, Bristol Myers Squibb, MSD, AstraZeneca Sadakatsu IkedaHonoraria: Chugai Pharma, Novartis, AstraZeneca, Taiho Pharmaceutical, Guardant Health, Bristol Myers Squibb-Ono Pharmaceutical, MSD, ACT Genomics, Roche, Canon Medical System, ACT MedConsulting or Advisory Role: Genodive Pharma (Inst)Research Funding: ACT Genomics (Inst), Hitachi (Inst), Canon Medical System (Inst) Ulrik LassenHonoraria: Bayer, Pfizer, NovartisConsulting or Advisory Role: Bayer, PfizerResearch Funding: BMS (Inst), Roche (Inst), Pfizer (Inst), GlaxoSmithKline (Inst), Incyte (Inst), Lilly (Inst) Enriqueta FelipConsulting or Advisory Role: Amgen, AstraZeneca, Bayer, Bristol Myers Squibb, Lilly, GlaxoSmithKline, Janssen, Merck Serono, Novartis, Pfizer, Sanofi, Takeda, Peptomyc, Daiichi Sankyo Europe GmbH, F. Hoffmann La Roche, Merck Sharp & DohmeSpeakers’ Bureau: AstraZeneca, Bristol Myers Squibb, Lilly, Medscape, Merck Sharp & Dohme, PeerVoice, Pfizer, Takeda, Amgen, F. Hoffmann La Roche, Janssen, Medical Trends, Merck Serono, Sanofi, TouchONCOLOGYResearch Funding: Merck (Inst), Merck KGaA (Inst)Other Relationship: GRiFOLS Josep TaberneroStock and Other Ownership Interests: Oniria TherapeuticsConsulting or Advisory Role: Bayer, Boehringer Ingelheim, Lilly, MSD, Merck Serono, Novartis, Sanofi, Taiho Pharmaceutical, Peptomyc, Chugai Pharma, Pfizer, Seattle Genetics, Array BioPharma, AstraZeneca, Genentech, Menarini, Servier, HalioDx, F. Hoffmann La Roche, Mirati Therapeutics, Pierre Fabre, Tessa Therapeutics, TheraMyc, Daiichi Sankyo, Samsung Bioepis, IQvia, Ikena Oncology, Merus, Neophore, Orion Biotechnology, Hutchison MediPharma, Scandion Oncology, Ona Therapeutics, Sotio, Inspirna, Scorpion TherapeuticsOther Relationship: Imedex, Medscape, MJH Life Sciences, Peerview, Physicans' Education Resource Alan SpatzConsulting or Advisory Role: Janssen, Bristol Myers Squibb, Pfizer/EMD Serono, Merck, Roche, AstraZeneca, Novartis, Bayer, AbbVieResearch Funding: Merck (Inst), Bristol Myers Squibb (Inst) C.S. PrameshStock and Other Ownership Interests: Aurobindo Philippe GirardHonoraria: Bayer, LEO Pharma, BMS/PfizerConsulting or Advisory Role: LEO Pharma, Bayer, BMS/PfizerTravel, Accommodations, Expenses: Bayer, LEO Pharma, BMS/Pfizer Jean-Yves BlayLeadership: Innate PharmaHonoraria: Roche, AstraZeneca, PharmaMar, MSD, BMS, Bayer, Ignyta, DecipheraConsulting or Advisory Role: Roche, Pharmamar, Deciphera, Blueprint Medicines, Bayer, Karyopharm TherapeuticsResearch Funding: GlaxoSmithKline (Inst), Pharmamar (Inst), Novartis (Inst), Bayer (Inst), Roche (Inst), BMS (Inst), MSD (Inst), Deciphera (Inst), AstraZeneca (Inst), OSE Pharma (Inst)Travel, Accommodations, Expenses: Roche Angel PorgadorStock and Other Ownership Interests: PooldiConsulting or Advisory Role: PiNKPatents, Royalties, Other Intellectual Property: Ben-Gurion University, Israel, several patents Eitan RubinStock and Other Ownership Interests: Pfizer Razelle KurzrockLeadership: CureMatch, CureMetrixStock and Other Ownership Interests: CureMatch, IDbyDNA, CureMetrixHonoraria: Roche, EUSA Pharma, NeoGenomics Laboratories, Biocom, NeoMed, Advanced Therapeutics, LEK, AACR, Chugai Pharma USA, Wiley, Merck, Pfizer, Meyer Consulting, Foundation Medicine, Turning Point Therapeutics, Bicara TherapeuticsConsulting or Advisory Role: Actuate Therapeutics, Loxo, XBiotech, NeoMed, Roche, Gaido, Soluventis, Pfizer, Merck, Turning Point Therapeutics, TD2/Volastra, Bicara Therapeutics, AstraZeneca, Biological Dynamics, Daiichi Sankyo, Eisai, EOM Pharmaceuticals, lylon, ProsperdtxSpeakers' Bureau: RocheResearch Funding: Guardant Health (Inst), Sequenom (Inst), Merck Serono (Inst), Genentech (Inst), Pfizer (Inst), Foundation Medicine (Inst), Incyte (Inst), Konica Minolta (Inst), Grifols (Inst), OmniSeq (Inst), Debiopharm Group (Inst), Boehringer Ingelheim (Inst), Top Alliance BioScience (Inst), Takeda (Isnt), Medlmmune (Inst), Biological Dynamics (Inst)Travel, Accommodations, Expenses: Roche, EUSA Pharma, NeoGenomics Laboratories, Biocom, NeoMed, Advanced Therapeutics, LEK, AACR, Chugai Pharma USA, Wiley Richard L. SchilskyLeadership: Clarified Precision MedicineStock and Other Ownership Interests: EQRxConsulting or Advisory Role: Cellworks, Scandion Oncology, Bryologyx, IIIumina, EQRx, Syapse, ZephyrAIResearch Funding: AstraZeneca (Inst), Bayer (Inst), Bristol Myers Squibb (Inst), Genentech/Roche (Inst), Lilly (Inst), Merck (Inst), Pfizer (Inst), Boehringer Ingelheim (Inst), Seattle Genetics (Inst)Open Payments Link: https://openpaymentsdata.cms.gov/physician/I138818/summaryNo other potential conflicts of interest were reported. EDAT- 2022/09/16 06:00 MHDA- 2022/09/20 06:00 PMCR- 2022/09/15 CRDT- 2022/09/15 16:03 PHST- 2022/09/15 16:03 [entrez] PHST- 2022/09/16 06:00 [pubmed] PHST- 2022/09/20 06:00 [medline] PHST- 2022/09/15 00:00 [pmc-release] AID - PO.22.00072 [pii] AID - 10.1200/PO.22.00072 [doi] PST - ppublish SO - JCO Precis Oncol. 2022 Sep;6:e2200072. doi: 10.1200/PO.22.00072. PMID- 41062500 OWN - NLM STAT- MEDLINE DCOM- 20251008 LR - 20251012 IS - 2041-1723 (Electronic) IS - 2041-1723 (Linking) VI - 16 IP - 1 DP - 2025 Oct 8 TI - Elucidating molecularly stratified single agent, and combination, therapeutic strategies targeting MCL1 for lethal prostate cancer. PG - 8806 LID - 10.1038/s41467-025-64042-5 [doi] LID - 8806 AB - Metastatic castration-resistant prostate cancer (mCRPC) is a lethal disease requiring additional therapeutic strategies. MCL1, an anti-apoptotic BCL2 family member, promotes cancer-cell survival, but its role in mCRPC remains poorly understood. Here, we characterise MCL1 in multiple mCRPC biopsy cohorts and patient-derived models, assessing responses to MCL1 inhibition. MCL1 copy number gain (14%-34%) correlates with increased MCL1 expression and worse outcomes. MCL1 inhibition exhibits anti-tumour effects in MCL1-gained mCRPC models. Co-inhibition of MCL1 and AKT induces cancer-specific cell death in PTEN-loss/PI3K-activated models in vitro and in vivo, modulating BAD-BCLXL and BIM-MCL1 interactions, with durable anti-tumour activity in models with AKT inhibitor acquired resistance. Finally, CDK9-mediated MCL1 downregulation combined with AKT inhibition recapitulates these findings, providing further opportunities for clinical translation. These data support early phase clinical trials targeting MCL1, both as monotherapy for MCL1-gained mCRPC, and in combination with AKT inhibition for PTEN-loss/PI3K-activated mCRPC. CI - © 2025. The Author(s). FAU - Jiménez-Vacas, Juan M AU - Jiménez-Vacas JM AD - The Institute of Cancer Research, London, UK. FAU - Westaby, Daniel AU - Westaby D AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden Hospital, London, UK. FAU - Figueiredo, Ines AU - Figueiredo I AUID- ORCID: 0009-0007-2754-5608 AD - The Institute of Cancer Research, London, UK. FAU - De Haven Brandon, Alexis AU - De Haven Brandon A AD - The Institute of Cancer Research, London, UK. FAU - Padilha, Ana AU - Padilha A AD - The Institute of Cancer Research, London, UK. FAU - Yuan, Wei AU - Yuan W AUID- ORCID: 0000-0001-8004-1846 AD - The Institute of Cancer Research, London, UK. FAU - Seed, George AU - Seed G AUID- ORCID: 0000-0002-1105-1781 AD - The Institute of Cancer Research, London, UK. FAU - Bogdan, Denisa AU - Bogdan D AD - The Institute of Cancer Research, London, UK. FAU - Gurel, Bora AU - Gurel B AUID- ORCID: 0000-0002-5018-8078 AD - The Institute of Cancer Research, London, UK. FAU - Bertan, Claudia AU - Bertan C AD - The Institute of Cancer Research, London, UK. FAU - Miranda, Susana AU - Miranda S AUID- ORCID: 0000-0002-5936-2706 AD - The Institute of Cancer Research, London, UK. FAU - Lambros, Maryou AU - Lambros M AD - The Institute of Cancer Research, London, UK. FAU - Montero-Hidalgo, Antonio J AU - Montero-Hidalgo AJ AUID- ORCID: 0000-0002-1948-7512 AD - The Institute of Cancer Research, London, UK. AD - Maimonides Institute for Biomedical Research of Cordoba (IMIBIC); Department of Cell Biology, Physiology, and Immunology, University of Cordoba; Reina Sofia University Hospital; CIBER Physiopathology of Obesity and Nutrition (CIBERobn), Cordoba, Spain. FAU - Coleman, Ilsa AU - Coleman I AD - Divisions of Human Biology and Clinical Research, Fred Hutchinson Cancer Center, Seattle, WA, USA. FAU - Yu, Ivan Pak Lok AU - Yu IPL AD - Vancouver Prostate Centre, University of British Columbia, Vancouver, BC, Canada. FAU - Buroni, Lorenzo AU - Buroni L AD - The Institute of Cancer Research, London, UK. FAU - Zeng, Wanting AU - Zeng W AUID- ORCID: 0000-0002-4101-1502 AD - The Institute of Cancer Research, London, UK. FAU - Neeb, Antje J AU - Neeb AJ AD - The Institute of Cancer Research, London, UK. FAU - Welti, Jon AU - Welti J AD - The Institute of Cancer Research, London, UK. FAU - Rekowski, Jan AU - Rekowski J AUID- ORCID: 0000-0002-5207-3864 AD - The Institute of Cancer Research, London, UK. FAU - Paravati, Roberta AU - Paravati R AD - The Institute of Cancer Research, London, UK. FAU - Gabel, Florian AU - Gabel F AD - The Institute of Cancer Research, London, UK. FAU - Pandell, Nicole AU - Pandell N AD - The Institute of Cancer Research, London, UK. FAU - Ferreira, Ana AU - Ferreira A AD - The Institute of Cancer Research, London, UK. FAU - Crespo, Mateus AU - Crespo M AUID- ORCID: 0000-0003-4043-8296 AD - The Institute of Cancer Research, London, UK. FAU - Riisnaes, Ruth AU - Riisnaes R AD - The Institute of Cancer Research, London, UK. FAU - Das, Souvik AU - Das S AD - The Institute of Cancer Research, London, UK. FAU - Taylor, Joe AU - Taylor J AD - The Institute of Cancer Research, London, UK. FAU - Waldron, Nick AU - Waldron N AD - The Institute of Cancer Research, London, UK. FAU - Hobern, Emily AU - Hobern E AD - The Institute of Cancer Research, London, UK. FAU - Valenti, Melanie AU - Valenti M AD - The Institute of Cancer Research, London, UK. FAU - Ning, Jian AU - Ning J AD - The Institute of Cancer Research, London, UK. FAU - Bernett, Ilona AU - Bernett I AD - The Institute of Cancer Research, London, UK. FAU - Liodaki, Kate AU - Liodaki K AD - The Institute of Cancer Research, London, UK. FAU - Persse, Thomas AU - Persse T AD - Divisions of Human Biology and Clinical Research, Fred Hutchinson Cancer Center, Seattle, WA, USA. FAU - Galipeau, Patricia AU - Galipeau P AUID- ORCID: 0000-0001-7961-7430 AD - Divisions of Human Biology and Clinical Research, Fred Hutchinson Cancer Center, Seattle, WA, USA. FAU - Wilkinson, Scott AU - Wilkinson S AUID- ORCID: 0000-0002-2613-8425 AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Trostel, Shana Y AU - Trostel SY AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Karzai, Fatima AU - Karzai F AUID- ORCID: 0000-0002-3244-1332 AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Chau, Cindy H AU - Chau CH AUID- ORCID: 0000-0002-6928-3833 AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Beatson, Erica L AU - Beatson EL AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Zhang, Xiaohu AU - Zhang X AD - Division of Preclinical Innovation, National Center for Advancing Translational Sciences, National Institute of Health, Rockville, MD, USA. FAU - Klumpp-Thomas, Carleen AU - Klumpp-Thomas C AUID- ORCID: 0000-0002-6646-6132 AD - Division of Preclinical Innovation, National Center for Advancing Translational Sciences, National Institute of Health, Rockville, MD, USA. FAU - Varkaris, Andreas AU - Varkaris A AD - Hematology-Oncology Division, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - Luque, Raul M AU - Luque RM AD - Maimonides Institute for Biomedical Research of Cordoba (IMIBIC); Department of Cell Biology, Physiology, and Immunology, University of Cordoba; Reina Sofia University Hospital; CIBER Physiopathology of Obesity and Nutrition (CIBERobn), Cordoba, Spain. FAU - Swain, Amanda AU - Swain A AUID- ORCID: 0000-0001-8666-1608 AD - The Institute of Cancer Research, London, UK. FAU - Raynaud, Florence AU - Raynaud F AUID- ORCID: 0000-0003-0957-6279 AD - The Institute of Cancer Research, London, UK. FAU - Lack, Nathan A AU - Lack NA AUID- ORCID: 0000-0001-7399-5844 AD - Vancouver Prostate Centre, University of British Columbia, Vancouver, BC, Canada. AD - Department of Medical Pharmacology, School of Medicine, Koc University, Istanbul, Turkey. FAU - Thomas, Craig J AU - Thomas CJ AUID- ORCID: 0000-0001-9386-9001 AD - Division of Preclinical Innovation, National Center for Advancing Translational Sciences, National Institute of Health, Rockville, MD, USA. AD - Lymphoid Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Ha, Gavin AU - Ha G AUID- ORCID: 0000-0001-7578-7272 AD - Divisions of Human Biology and Clinical Research, Fred Hutchinson Cancer Center, Seattle, WA, USA. FAU - Figg, William D AU - Figg WD AUID- ORCID: 0000-0003-2428-5613 AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Bezzi, Marco AU - Bezzi M AD - The Institute of Cancer Research, London, UK. FAU - Sowalsky, Adam G AU - Sowalsky AG AUID- ORCID: 0000-0003-2760-1853 AD - Genitourinary Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. FAU - Nelson, Peter S AU - Nelson PS AUID- ORCID: 0000-0002-5451-5726 AD - Divisions of Human Biology and Clinical Research, Fred Hutchinson Cancer Center, Seattle, WA, USA. FAU - Carreira, Suzanne AU - Carreira S AUID- ORCID: 0000-0002-5077-5379 AD - The Institute of Cancer Research, London, UK. FAU - Balk, Steven P AU - Balk SP AUID- ORCID: 0000-0002-4546-7371 AD - Hematology-Oncology Division, Beth Israel Deaconess Medical Center, Boston, MA, USA. FAU - de Bono, Johann S AU - de Bono JS AUID- ORCID: 0000-0002-2034-595X AD - The Institute of Cancer Research, London, UK. johann.de-bono@icr.ac.uk. AD - The Royal Marsden Hospital, London, UK. johann.de-bono@icr.ac.uk. FAU - Sharp, Adam AU - Sharp A AUID- ORCID: 0000-0002-3740-1612 AD - The Institute of Cancer Research, London, UK. adam.sharp@icr.ac.uk. AD - The Royal Marsden Hospital, London, UK. adam.sharp@icr.ac.uk. LA - eng GR - IES\R3\213131/Royal Society of Medicine (RSM)/ GR - Clinical Research Career Development Fellowship/Wellcome Trust (Wellcome)/ GR - R21 CA277368/CA/NCI NIH HHS/United States GR - R50 CA274336/CA/NCI NIH HHS/United States GR - WT_/Wellcome Trust/United Kingdom GR - P01 CA163227/CA/NCI NIH HHS/United States GR - Challenge Award/Prostate Cancer Foundation (PCF)/ PT - Journal Article DEP - 20251008 PL - England TA - Nat Commun JT - Nature communications JID - 101528555 RN - 0 (Myeloid Cell Leukemia Sequence 1 Protein) RN - 0 (MCL1 protein, human) RN - EC 2.7.11.1 (Proto-Oncogene Proteins c-akt) RN - EC 3.1.3.67 (PTEN Phosphohydrolase) RN - EC 3.1.3.67 (PTEN protein, human) RN - EC 2.7.1.- (Phosphatidylinositol 3-Kinases) RN - 0 (bcl-Associated Death Protein) RN - 0 (Bcl-2-Like Protein 11) SB - IM MH - *Myeloid Cell Leukemia Sequence 1 Protein/genetics/metabolism/antagonists & inhibitors MH - Male MH - Humans MH - *Prostatic Neoplasms, Castration-Resistant/drug therapy/genetics/pathology/metabolism MH - Animals MH - Cell Line, Tumor MH - Mice MH - Proto-Oncogene Proteins c-akt/antagonists & inhibitors/metabolism MH - Xenograft Model Antitumor Assays MH - PTEN Phosphohydrolase/metabolism/genetics MH - Phosphatidylinositol 3-Kinases/metabolism MH - Gene Expression Regulation, Neoplastic/drug effects MH - Apoptosis/drug effects MH - bcl-Associated Death Protein/metabolism MH - Bcl-2-Like Protein 11/metabolism PMC - PMC12508096 COIS- Competing interests: J.M.J.-V., D.W., I.F., A.d.H.V., A.P., W.Y., G.S., D.B., B.G., C.B., S.M., L.B., W.Z., A.J.N., J.W., J.R., R.P., A.F., M.C., R.R., S.D., J.T., E.H., M.V., J.N., K.L., A.S., F.R., S.C., J.S.d.B. and A.Sharp are employees of the ICR, which has a commercial interest in abiraterone, PARP inhibition in DNA repair defective cancers, and PI3K/AKT pathway inhibitors (no personal income). A.G.S. reports that the National Cancer Institute (NCI) has a Cooperative Research and Development Agreement (CRADA) with Astellas. Resources are provided by this CRADA to the NCI. A.G.S. gets no personal funding from this CRADA but is the primary investigator of the CRADA. J.S.d.B. has served on advisory boards and received fees from many companies, including Amgen, Astra Zeneca, Bayer, Bioxcel Therapeutics, Daiichi, Genentech/Roche, GSK, Merck Serono, Merck Sharp & Dohme, Pfizer, and Sanofi Aventis. He is an employee of the ICR, which has received funding or other support for his research work from AZ, Astellas, Bayer, Cellcentric, Daiichi, Genentech, Genmab, GSK, Janssen, Merck Serono, MSD, Menarini/Silicon Biosystems, Orion, Sanofi Aventis, Sierra Oncology, Taiho, Pfizer, Vertex. J.S.d.B. was named as an inventor, with no financial interest, for patent 8,822,438, submitted by Janssen, that covers the use of abiraterone acetate with corticosteroids. J.S.d.B. has been the CI/PI of many industry-sponsored clinical trials. A.Sharp has received travel support from Sanofi, Roche-Genentech and Nurix, and speaker honoraria from Astellas Pharma and Merck Sharp & Dohme. He has served as an advisor to DE Shaw Research, CHARM Therapeutics, Ellipses Pharma and Droia Ventures. A.Sharp has been the CI/PI of industry-sponsored clinical trials. The remaining authors declare no conflicts of interest. EDAT- 2025/10/09 00:30 MHDA- 2025/10/09 00:31 PMCR- 2025/10/08 CRDT- 2025/10/08 23:17 PHST- 2024/03/07 00:00 [received] PHST- 2025/09/04 00:00 [accepted] PHST- 2025/10/09 00:31 [medline] PHST- 2025/10/09 00:30 [pubmed] PHST- 2025/10/08 23:17 [entrez] PHST- 2025/10/08 00:00 [pmc-release] AID - 10.1038/s41467-025-64042-5 [pii] AID - 64042 [pii] AID - 10.1038/s41467-025-64042-5 [doi] PST - epublish SO - Nat Commun. 2025 Oct 8;16(1):8806. doi: 10.1038/s41467-025-64042-5. PMID- 37844613 OWN - NLM STAT- MEDLINE DCOM- 20231204 LR - 20260127 IS - 1476-4687 (Electronic) IS - 0028-0836 (Print) IS - 0028-0836 (Linking) VI - 623 IP - 7989 DP - 2023 Nov TI - Targeting myeloid chemotaxis to reverse prostate cancer therapy resistance. PG - 1053-1061 LID - 10.1038/s41586-023-06696-z [doi] AB - Inflammation is a hallmark of cancer(1). In patients with cancer, peripheral blood myeloid expansion, indicated by a high neutrophil-to-lymphocyte ratio, associates with shorter survival and treatment resistance across malignancies and therapeutic modalities(2-5). Whether myeloid inflammation drives progression of prostate cancer in humans remain unclear. Here we show that inhibition of myeloid chemotaxis can reduce tumour-elicited myeloid inflammation and reverse therapy resistance in a subset of patients with metastatic castration-resistant prostate cancer (CRPC). We show that a higher blood neutrophil-to-lymphocyte ratio reflects tumour myeloid infiltration and tumour expression of senescence-associated mRNA species, including those that encode myeloid-chemoattracting CXCR2 ligands. To determine whether myeloid cells fuel resistance to androgen receptor signalling inhibitors, and whether inhibiting CXCR2 to block myeloid chemotaxis reverses this, we conducted an investigator-initiated, proof-of-concept clinical trial of a CXCR2 inhibitor (AZD5069) plus enzalutamide in patients with metastatic CRPC that is resistant to androgen receptor signalling inhibitors. This combination was well tolerated without dose-limiting toxicity and it decreased circulating neutrophil levels, reduced intratumour CD11b(+)HLA-DR(lo)CD15(+)CD14(-) myeloid cell infiltration and imparted durable clinical benefit with biochemical and radiological responses in a subset of patients with metastatic CRPC. This study provides clinical evidence that senescence-associated myeloid inflammation can fuel metastatic CRPC progression and resistance to androgen receptor blockade. Targeting myeloid chemotaxis merits broader evaluation in other cancers. CI - © 2023. The Author(s). FAU - Guo, Christina AU - Guo C AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Sharp, Adam AU - Sharp A AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Gurel, Bora AU - Gurel B AUID- ORCID: 0000-0002-5018-8078 AD - The Institute of Cancer Research, London, UK. FAU - Crespo, Mateus AU - Crespo M AUID- ORCID: 0000-0003-4043-8296 AD - The Institute of Cancer Research, London, UK. FAU - Figueiredo, Ines AU - Figueiredo I AD - The Institute of Cancer Research, London, UK. FAU - Jain, Suneil AU - Jain S AD - Northern Ireland Cancer Centre, Belfast, UK. AD - Patrick G Johnston Centre for Cancer Research, Queen's University Belfast, Belfast, UK. FAU - Vogl, Ursula AU - Vogl U AD - Oncology Institute of Southern Switzerland, Ente Ospedaliero Cantonale (EOC), Bellinzona, Switzerland. FAU - Rekowski, Jan AU - Rekowski J AUID- ORCID: 0000-0002-5207-3864 AD - The Institute of Cancer Research, London, UK. FAU - Rouhifard, Mahtab AU - Rouhifard M AD - The Institute of Cancer Research, London, UK. FAU - Gallagher, Lewis AU - Gallagher L AUID- ORCID: 0000-0003-2694-3157 AD - The Institute of Cancer Research, London, UK. FAU - Yuan, Wei AU - Yuan W AD - The Institute of Cancer Research, London, UK. FAU - Carreira, Suzanne AU - Carreira S AUID- ORCID: 0000-0002-5077-5379 AD - The Institute of Cancer Research, London, UK. FAU - Chandran, Khobe AU - Chandran K AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Paschalis, Alec AU - Paschalis A AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Colombo, Ilaria AU - Colombo I AUID- ORCID: 0000-0002-0602-8667 AD - Oncology Institute of Southern Switzerland, Ente Ospedaliero Cantonale (EOC), Bellinzona, Switzerland. FAU - Stathis, Anastasios AU - Stathis A AD - Oncology Institute of Southern Switzerland, Ente Ospedaliero Cantonale (EOC), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università della Svizzera Italiana (USI), Lugano, Switzerland. FAU - Bertan, Claudia AU - Bertan C AD - The Institute of Cancer Research, London, UK. FAU - Seed, George AU - Seed G AUID- ORCID: 0000-0002-1105-1781 AD - The Institute of Cancer Research, London, UK. FAU - Goodall, Jane AU - Goodall J AD - The Institute of Cancer Research, London, UK. FAU - Raynaud, Florence AU - Raynaud F AUID- ORCID: 0000-0003-0957-6279 AD - The Institute of Cancer Research, London, UK. FAU - Ruddle, Ruth AU - Ruddle R AUID- ORCID: 0000-0003-0025-8872 AD - The Institute of Cancer Research, London, UK. FAU - Swales, Karen E AU - Swales KE AUID- ORCID: 0000-0002-6572-1293 AD - The Institute of Cancer Research, London, UK. FAU - Malia, Jason AU - Malia J AD - The Institute of Cancer Research, London, UK. FAU - Bogdan, Denisa AU - Bogdan D AUID- ORCID: 0000-0002-3017-4832 AD - The Institute of Cancer Research, London, UK. FAU - Tiu, Crescens AU - Tiu C AUID- ORCID: 0000-0002-0515-6130 AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Caldwell, Reece AU - Caldwell R AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Aversa, Caterina AU - Aversa C AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Ferreira, Ana AU - Ferreira A AD - The Institute of Cancer Research, London, UK. FAU - Neeb, Antje AU - Neeb A AD - The Institute of Cancer Research, London, UK. FAU - Tunariu, Nina AU - Tunariu N AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Westaby, Daniel AU - Westaby D AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Carmichael, Juliet AU - Carmichael J AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Fenor de la Maza, Maria Dolores AU - Fenor de la Maza MD AD - The Institute of Cancer Research, London, UK. AD - The Royal Marsden NHS Foundation Trust, London, UK. FAU - Yap, Christina AU - Yap C AUID- ORCID: 0000-0002-6715-2514 AD - The Institute of Cancer Research, London, UK. FAU - Matthews, Ruth AU - Matthews R AD - The Institute of Cancer Research, London, UK. FAU - Badham, Hannah AU - Badham H AD - The Institute of Cancer Research, London, UK. FAU - Prout, Toby AU - Prout T AD - The Institute of Cancer Research, London, UK. FAU - Turner, Alison AU - Turner A AD - The Institute of Cancer Research, London, UK. FAU - Parmar, Mona AU - Parmar M AUID- ORCID: 0000-0001-7818-4100 AD - The Institute of Cancer Research, London, UK. FAU - Tovey, Holly AU - Tovey H AD - The Institute of Cancer Research, London, UK. FAU - Riisnaes, Ruth AU - Riisnaes R AD - The Institute of Cancer Research, London, UK. FAU - Flohr, Penny AU - Flohr P AD - The Institute of Cancer Research, London, UK. FAU - Gil, Jesus AU - Gil J AUID- ORCID: 0000-0002-4303-6260 AD - MRC London Institute of Medical Sciences (LMS), London, UK. AD - Institute of Clinical Sciences (ICS), Faculty of Medicine, Imperial College London, London, UK. FAU - Waugh, David AU - Waugh D AD - Patrick G Johnston Centre for Cancer Research, Queen's University Belfast, Belfast, UK. AD - Centre for Cancer Biology, University of South Australia, Adelaide, South Australia, Australia. FAU - Decordova, Shaun AU - Decordova S AD - The Institute of Cancer Research, London, UK. FAU - Schlag, Anna AU - Schlag A AD - The Institute of Cancer Research, London, UK. FAU - Calì, Bianca AU - Calì B AD - Institute of Oncology Research, Bellinzona, Switzerland. FAU - Alimonti, Andrea AU - Alimonti A AUID- ORCID: 0000-0002-9362-2313 AD - Oncology Institute of Southern Switzerland, Ente Ospedaliero Cantonale (EOC), Bellinzona, Switzerland. AD - Faculty of Biomedical Sciences, Università della Svizzera Italiana (USI), Lugano, Switzerland. AD - Institute of Oncology Research, Bellinzona, Switzerland. AD - Department of Health Sciences and Technology, Eidgenössische Technische Hochschule Zürich (ETH), Zurich, Switzerland. AD - Department of Medicine, Veneto Institute of Molecular Medicine, University of Padova, Padua, Italy. FAU - de Bono, Johann S AU - de Bono JS AUID- ORCID: 0000-0002-2034-595X AD - The Institute of Cancer Research, London, UK. johann.debono@icr.ac.uk. AD - The Royal Marsden NHS Foundation Trust, London, UK. johann.debono@icr.ac.uk. LA - eng GR - MR/M018318/1/MRC_/Medical Research Council/United Kingdom GR - MR/W018217/1/MRC_/Medical Research Council/United Kingdom GR - 28647/CRUK_/Cancer Research UK/United Kingdom GR - WT_/Wellcome Trust/United Kingdom GR - MC_U120085810/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20231016 PL - England TA - Nature JT - Nature JID - 0410462 RN - 0 (Lewis X Antigen) RN - 0 (Receptors, Androgen) RN - 0 (Androgen Receptor Antagonists) RN - 0 (Antineoplastic Agents) RN - 0 (CD14 protein, human) RN - 0 (Lipopolysaccharide Receptors) RN - 93T0T9GKNU (enzalutamide) RN - 0 (Benzamides) RN - 0 (Nitriles) RN - 2010-15-3 (Phenylthiohydantoin) RN - 0 (ITGAM protein, human) RN - 0 (CD11b Antigen) RN - 0 (N-(2-(2,3-difluoro-6-benzylthio)-6-(3,4-dihydroxybutan-2-yloxy)pyrimidin-4-yl)azetidine-1-sulfonamide) RN - 0 (Pyrimidines) RN - 0 (Sulfonamides) SB - IM MH - Humans MH - Male MH - *Chemotaxis/drug effects MH - Disease Progression MH - *Drug Resistance, Neoplasm MH - Inflammation/drug therapy/pathology MH - Lewis X Antigen/metabolism MH - *Myeloid Cells/drug effects/pathology MH - Neoplasm Metastasis MH - Prostate/drug effects/metabolism/pathology MH - *Prostatic Neoplasms, Castration-Resistant/drug therapy/metabolism/pathology MH - Receptors, Androgen/metabolism MH - *Androgen Receptor Antagonists/pharmacology/therapeutic use MH - *Antineoplastic Agents/pharmacology/therapeutic use MH - Lipopolysaccharide Receptors MH - Benzamides MH - Nitriles MH - Phenylthiohydantoin MH - CD11b Antigen MH - Pyrimidines MH - Sulfonamides PMC - PMC10686834 COIS- A. Sharp, B.G., M.C., I.F., J.R., M.R., L.G., W.Y., S.C., K.C., A.P., C.B., G.S., J. Goodall, F.R., R. Ruddle, K.E.S., J.M., D.B., C.T., A.N., N.T., D. Westaby, J.C., M.D.F., C.Y., R.M., H.B., T.P., A.T., M.P., H.T., R. Riisnaes, A.F., P.F., S.D., A. Schlag and J.S.d.B. are employees of The ICR, which has a commercial interest in abiraterone, PARP inhibition in DNA-repair-defective cancers and PI3K–AKT pathway inhibitors. C.G. was an employee of The ICR during the conduct of this work, as well as submission, review, and revision of the manuscript, and is now an employee of Roche-Genentech. A. Sharp has received travel support from Sanofi, Roche-Genentech and Nurix, and speaker honoraria from Astellas Pharma and Merck Sharp & Dohme; has served as an advisor to DE Shaw Research and CHARM Therapeutics; and has been the chief or principal investigator of industry-sponsored clinical trials. J.S.d.B. has served on advisory boards and received fees from companies including Amgen, AstraZeneca, Astellas, Bayer, Bioxcel Therapeutics, Boehringer Ingelheim, Cellcentric, Daiichi, Eisai, Genentech/Roche, Genmab, GSK, Harpoon, Janssen, Merck Serono, Merck Sharp & Dohme, Menarini/Silicon Biosystems, Orion, Pfizer, Qiagen, Sanofi Aventis, Sierra Oncology, Taiho, Terumo and Vertex Pharmaceuticals. J.S.d.B. receives funding or other support for his research work from Daiich Sankyo, AstraZeneca, Astellas, Bayer, Cellcentric, Daiichi, Roche-Genentech, Genmab, GSK, Janssen, Merck Serono, MSD, Menarini/Silicon Biosystems, Orion, Sanofi Aventis, Sierra Oncology, Taiho, Pfizer and Vertex. J.S.d.B. was named as an inventor, with no financial interest, on US patent 8,822,438. J.S.d.B. has been the chief or principal investigator of many industry-sponsored clinical trials. S.J. has served on advisory boards and received fees from companies including Accord, AstraZeneca, Astellas, Bayer, Boston Scientific, Janssen and Pfizer; and reports research funding from Boston Scientific for other research projects. I.C. reports speaker, consultancy or advisory role activities for GSK, AstraZeneca, Novartis and MSD; travel grants from Tesaro, GSK, AstraZeneca and Janssen; and research funding (to institution as the principal investigator) from MSD, Bayer, Incyte, AstraZeneca and Vivesto. U.V. reports an advisory role (institutional) to Janssen, Astellas, Merck, MSD, Pfizer, Roche, Bayer, BMS and Novartis AAA; travel support from Janssen, Merck and Ipsen; being part of the speaker bureau for (compensated, institutional) Janssen, Astellas, Pfizer, Roche, SAMO, BMS and Ipsen; and grant funding from Fond’Action. A. Stathis serves as a principal investigator and receives institutional funding for clinical trials sponsored by AstraZeneca, Bayer, Incyte, Roche, Abbvie, ADC Therapeutics, Amgen, Cellestia, Loxo Oncology, Merck MSD, Novartis, Pfizer, Philogen and Roche; received travel grants from AstraZeneca and Incyte; served on advisory boards for Janssen and Roche; provided expert testimony to Bayer and Eli Lilly. M.D.F. received travel funding from Astellas, AstraZeneca, Pfizer, Pierre Fabre, Roche, Bristol Meiers Squibb, Novartis, MSD, Janssen and Bayer, and personal fees from Janssen, Pierre Fabre and Roche (all funding and fees were outside the submitted work). J. Gil has acted as a consultant for Unity Biotechnology, Geras Bio, Myricx Pharma and Merck KGaA. Pfizer and Unity Biotechnology have financially supported research in J. Gil’s laboratory (unrelated to the work presented here). J. Gil owns equity in Geras Bio. J. Gil is a named inventor on MRC and Imperial College patents, both related to senolytic therapies (the patents are not related to the work presented here). A.A. has been the principal investigator of industry-sponsored clinical trials sponsored by Astellas Pharma Inc., AstraZeneca, Sun Pharma Global FZE; has received consulting fees from Debiopharm; and owns shares in Oncosence. B.C., D. Waugh., R.C. and C.A. do not have any competing interest to declare. EDAT- 2023/10/17 00:42 MHDA- 2023/12/01 06:44 PMCR- 2023/10/16 CRDT- 2023/10/16 19:03 PHST- 2023/05/23 00:00 [received] PHST- 2023/09/28 00:00 [accepted] PHST- 2023/12/01 06:44 [medline] PHST- 2023/10/17 00:42 [pubmed] PHST- 2023/10/16 19:03 [entrez] PHST- 2023/10/16 00:00 [pmc-release] AID - 10.1038/s41586-023-06696-z [pii] AID - 6696 [pii] AID - 10.1038/s41586-023-06696-z [doi] PST - ppublish SO - Nature. 2023 Nov;623(7989):1053-1061. doi: 10.1038/s41586-023-06696-z. Epub 2023 Oct 16. PMID- 38718358 OWN - NLM STAT- MEDLINE DCOM- 20240508 LR - 20260518 IS - 1533-4406 (Electronic) IS - 0028-4793 (Linking) VI - 390 IP - 18 DP - 2024 May 9 TI - Trial of Thrombectomy for Stroke with a Large Infarct of Unrestricted Size. PG - 1677-1689 LID - 10.1056/NEJMoa2314063 [doi] AB - BACKGROUND: The use of thrombectomy in patients with acute stroke and a large infarct of unrestricted size has not been well studied. METHODS: We assigned, in a 1:1 ratio, patients with proximal cerebral vessel occlusion in the anterior circulation and a large infarct (as defined by an Alberta Stroke Program Early Computed Tomographic Score of ≤5; values range from 0 to 10) detected on magnetic resonance imaging or computed tomography within 6.5 hours after symptom onset to undergo endovascular thrombectomy and receive medical care (thrombectomy group) or to receive medical care alone (control group). The primary outcome was the score on the modified Rankin scale at 90 days (scores range from 0 to 6, with higher scores indicating greater disability). The primary safety outcome was death from any cause at 90 days, and an ancillary safety outcome was symptomatic intracerebral hemorrhage. RESULTS: A total of 333 patients were assigned to either the thrombectomy group (166 patients) or the control group (167 patients); 9 were excluded from the analysis because of consent withdrawal or legal reasons. The trial was stopped early because results of similar trials favored thrombectomy. Approximately 35% of the patients received thrombolysis therapy. The median modified Rankin scale score at 90 days was 4 in the thrombectomy group and 6 in the control group (generalized odds ratio, 1.63; 95% confidence interval [CI], 1.29 to 2.06; P<0.001). Death from any cause at 90 days occurred in 36.1% of the patients in the thrombectomy group and in 55.5% of those in the control group (adjusted relative risk, 0.65; 95% CI, 0.50 to 0.84), and the percentage of patients with symptomatic intracerebral hemorrhage was 9.6% and 5.7%, respectively (adjusted relative risk, 1.73; 95% CI, 0.78 to 4.68). Eleven procedure-related complications occurred in the thrombectomy group. CONCLUSIONS: In patients with acute stroke and a large infarct of unrestricted size, thrombectomy plus medical care resulted in better functional outcomes and lower mortality than medical care alone but led to a higher incidence of symptomatic intracerebral hemorrhage. (Funded by Montpellier University Hospital; LASTE ClinicalTrials.gov number, NCT03811769.). CI - Copyright © 2024 Massachusetts Medical Society. FAU - Costalat, Vincent AU - Costalat V AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Jovin, Tudor G AU - Jovin TG AUID- ORCID: 0000-0002-2619-6975 AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Albucher, J F AU - Albucher JF AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Cognard, Christophe AU - Cognard C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Henon, Hilde AU - Henon H AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Nouri, Nasreddine AU - Nouri N AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Gory, Benjamin AU - Gory B AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Richard, Sebastien AU - Richard S AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Marnat, Gaultier AU - Marnat G AUID- ORCID: 0000-0002-7611-7753 AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Sibon, Igor AU - Sibon I AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Di Maria, Federico AU - Di Maria F AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Annan, Mariam AU - Annan M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Boulouis, Grégoire AU - Boulouis G AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Cardona, Pere AU - Cardona P AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Obadia, Michael AU - Obadia M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Piotin, Michel AU - Piotin M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Bourcier, Romain AU - Bourcier R AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Guillon, Benoit AU - Guillon B AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Godard, Sophie AU - Godard S AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Pasco-Papon, Anne AU - Pasco-Papon A AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Eker, Omer F AU - Eker OF AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Cho, Tae-Hee AU - Cho TH AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Turc, Guillaume AU - Turc G AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Naggara, Olivier AU - Naggara O AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Velasco, Stéphane AU - Velasco S AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Lamy, Matthias AU - Lamy M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Clarençon, Frédéric AU - Clarençon F AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Alamowitch, Sonia AU - Alamowitch S AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Renu, Arturo AU - Renu A AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Suissa, Laurent AU - Suissa L AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Brunel, Hervé AU - Brunel H AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Gentric, Jean-Christophe AU - Gentric JC AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Timsit, Serge AU - Timsit S AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Lamy, Chantal AU - Lamy C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Chivot, Cyril AU - Chivot C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Macian-Montoro, Francisco AU - Macian-Montoro F AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Mounayer, Charbel AU - Mounayer C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Ozkul-Wermester, Ozlem AU - Ozkul-Wermester O AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Papagiannaki, Chrysanthi AU - Papagiannaki C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Wolff, Valérie AU - Wolff V AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Pop, Raoul AU - Pop R AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Ferrier, Anna AU - Ferrier A AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Chabert, Emmanuel AU - Chabert E AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Ricolfi, Frédéric AU - Ricolfi F AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Béjot, Yannick AU - Béjot Y AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Lopez-Cancio, Elena AU - Lopez-Cancio E AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Vega, Pedro AU - Vega P AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Spelle, Laurent AU - Spelle L AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Denier, Christian AU - Denier C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Millán, Mònica AU - Millán M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Arenillas, Juan F AU - Arenillas JF AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Mazighi, Mikael AU - Mazighi M AUID- ORCID: 0000-0003-0911-8999 AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Houdart, Emmanuel AU - Houdart E AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Del Mar Freijo, Maria AU - Del Mar Freijo M AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Duhamel, Alain AU - Duhamel A AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Sanossian, Nerses AU - Sanossian N AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Liebeskind, David S AU - Liebeskind DS AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Labreuche, Julien AU - Labreuche J AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Lapergue, Bertrand AU - Lapergue B AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. FAU - Arquizan, Caroline AU - Arquizan C AD - From the Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier (V.C.), the Departments of Neurology (J.F. Albucher) and Neuroradiology (C. Cognard), Hôpital Pierre Paul Riquet, and Toulouse Clinical Investigations Centers 1436 (J.F. Albucher, C. Cognard), Toulouse, the Departments of Neurology (H.H.) and Neuroradiology (N.N.), Hôpital Salengro, and the Department of Biostatistics, Centre Hospitalier Universitaire (CHU) Lille (A.D., J.L.), Lille, the Department of Neuroradiology, Hôpital central, L'unité d'Imagerie Adaptative Diagnostique et Interventionnelle, INSERM Unité 1254 (B. Gory), and the Department of Neurology, Hôpital central, Centre d'investigation clinique Plurithématique 1433, INSERM Unité 1116 (S.R.), Nancy, the Departments of Neuroradiology (G.M.) and Neurology (I.S.), Hôpital Pellegrin, Bordeaux, the Department of Neuroradiology, Hôpital Foch, Suresnes (F.D.M.), the Departments of Neurology (M.A.) and Neuroradiology (G.B.), Hôpital Bretonneau, Tours, the Departments of Neurology (M.O.) and Neuroradiology (M.P.), Hôpital Fondation Adolphe de Rothschild, the Departments of Neurology (G.T.) and Neuroradiology (O.N.), Groupe Hospitalier Universitaire Paris, Centre Hospitalier Sainte-Anne, INSERM Unité 1266, the Departments of Neuroradiology (F.C.) and Neurology (S.A.), Hôpital La Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), the Departments of Neuroradiology (L. Spelle) and Neurology (C.D.), Hôpital Bicêtre, AP-HP, the Departments of Neurology (M. Mazighi) and Neuroradiology (E.H.), Hôpital Lariboisière AP-HP, and INSERM Unité 1266 (C.A.), Paris, the Department of Neuroradiology, Nantes Université, CHU Nantes (R.B.), INSERM Unité Mixte de Recherche 1087, Centre National de la Recherche Scientifique, University of Nantes, L'institut du Thorax (R.B.), and Clinique Neurologique, Hôpital G.R. Laennec CHU Nantes (B. Guillon), Nantes, the Departments of Neurology (S.G.) and Neuroradiology (A.P.-P.), CHU d'Angers, Angers, the Departments of Neuroradiology (O.F.E.) and Neurology (T.-H.C.), Hospices Civils de Lyon, Groupement Hospitalier Est Hôpital Pierre Wertheimer, Lyon, the Departments of Neuroradiology (S.V.) and Neurology (M.L.), CHU Poitiers, Site de La Milétrie, Poitiers, the Stroke Unit (L. Suissa) and the Department of Neuroradiology (H.B.), Assistance Publique-Hôpitaux de Marseille, Hôpital de la Timone, Marseille, the Departments of Neuroradiology (J.-C.G.) and Neurology (S.T.), Centre Hospitalier Régional Universitaire (CHRU) Brest, Hôpital de la Cavale Blanche, Brest, the Departments of Neurology (C.L.) and Neuroradiology (C. Chivot), CHU Amiens-Picardie, Amiens, the Departments of Neurology (F.M.-M.) and Neuroradiology (C.M.), CHU Limoges, Dupuytren, Limoges, the Departments of Neurology (O.O.-W.) and Neuroradiology (C.P.), CHU Rouen, Rouen, the Departments of Neurology (V.W.) and Neuroradiology (R.P.), CHRU Strasbourg, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, the Departments of Neurology (A.F.) and Neuroradiology (E.C.), CHU Clermont-Ferrand, Hôpital Gabriel Montpied, Clermont-Ferrand, the Departments of Neuroradiology (F.R.) and Neurology (Y.B.), CHU Dijon-Bourgogne, Hôpital François Mitterrand, Dijon, and the Department of Neurology, Hôpital Foch, Suresnes (B.L.), and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier (C.A.) - all in France; Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ (T.G.J.); the Department of Neurology, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat (P.C.), and the Department of Neurology, Hospital Clínic de Barcelona (A.R.), Barcelona, the Department of Radiology, Hospital Universitario Central de Asturias, Oviedo (E.L.-C., P.V.), the Department of Neurology Hospital Germans Trias i Pujol, Badalona (M. Millán), Hospital Clínico Universitario de Valladolid, Valladolid (J.F. Arenillas), and the Department of Neurology, Hospital Universitario Cruces, Baracaldo (M.M.F.) - all in Spain; and the Department of Neurology, University of Southern California (N.S.), and the Department of Neurology, UCLA (D.S.L.) - both in Los Angeles. CN - LASTE Trial Investigators LA - eng SI - ClinicalTrials.gov/NCT03811769 PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, Non-U.S. Gov't PL - United States TA - N Engl J Med JT - The New England journal of medicine JID - 0255562 SB - IM CIN - N Engl J Med. 2024 Jul 25;391(4):378-379. doi: 10.1056/NEJMc2406965. PMID: 39047249 CIN - N Engl J Med. 2024 Jul 25;391(4):379. doi: 10.1056/NEJMc2406965. PMID: 39047250 CIN - AJR Am J Roentgenol. 2025 Apr;224(4):e2431849. doi: 10.2214/AJR.24.31849. PMID: 39109679 MH - Aged MH - Aged, 80 and over MH - Female MH - Humans MH - Male MH - Cerebral Hemorrhage/etiology MH - Combined Modality Therapy MH - Endovascular Procedures MH - Magnetic Resonance Imaging MH - *Stroke/diagnostic imaging/etiology/therapy MH - *Thrombectomy MH - *Thrombolytic Therapy/adverse effects/methods MH - Tomography, X-Ray Computed MH - Brain Infarction/diagnostic imaging/etiology/therapy MH - Acute Disease MH - Cerebral Arteries/diagnostic imaging/surgery MH - Cerebral Arterial Diseases/complications/diagnostic imaging/pathology/surgery MH - *Infarction, Anterior Cerebral Artery/diagnostic imaging/pathology/surgery FIR - Ayrignac, Xavier IR - Ayrignac X FIR - Arquizan, Caroline IR - Arquizan C FIR - Charif, Mahmoud IR - Charif M FIR - Costalat, Vincent IR - Costalat V FIR - Corti, Lucas IR - Corti L FIR - Dargazanli, Cyril IR - Dargazanli C FIR - Derraz, Imad IR - Derraz I FIR - Gaillard, Nicolas IR - Gaillard N FIR - Gascou, Grégory IR - Gascou G FIR - Lefevre, Pierre-Henri IR - 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Neau, Jean-Philippe IR - Neau JP FIR - Palazzo, Paola IR - Palazzo P FIR - Raynaud, Nicolas IR - Raynaud N FIR - Aghetti, Agnès IR - Aghetti A FIR - Houdart, Emmanuel IR - Houdart E FIR - Dimitrovic, Anna IR - Dimitrovic A FIR - Mazighi, Mikael IR - Mazighi M FIR - Gueden, Alexis IR - Gueden A FIR - Reiner, Peggy IR - Reiner P FIR - Benhassen, Wagih IR - Benhassen W FIR - Naggara, Olivier IR - Naggara O FIR - Kerleroux, Basile IR - Kerleroux B FIR - Turc, Guillaume IR - Turc G FIR - Rodriguez Regent, Christine IR - Rodriguez Regent C FIR - Allard, Julien IR - Allard J FIR - Clarencon, Frédéric IR - Clarencon F FIR - Baronnet, Flore IR - Baronnet F FIR - Alamowitch, Sonia IR - Alamowitch S FIR - Crozier, Sophie IR - Crozier S FIR - Delorme, Stephen IR - Delorme S FIR - Deltour, Sandrine IR - Deltour S FIR - Gerschenfeld, Gaspard IR - Gerschenfeld G FIR - Leger, Anne IR - Leger A FIR - Premat, Kevin IR - Premat K FIR - Rosso, Charlotte IR - Rosso C FIR - Samson, Yves IR - Samson Y FIR - Shotar, Eimad IR - Shotar E FIR - Caroff, Jildaaz IR - Caroff J FIR - Spelle, Laurent IR - Spelle L FIR - Chalumeau, Vanessa IR - Chalumeau V FIR - Chassin, Olivier IR - Chassin O FIR - Gallas, Sophie IR - Gallas S FIR - Ikka, Léon IR - Ikka L FIR - Legris, Nicolas IR - Legris N FIR - Sarov Riviere, Mariana IR - Sarov Riviere M FIR - Venditti, Laura IR - Venditti L FIR - Denier, Christian IR - Denier C FIR - Anxionnat, René IR - Anxionnat R FIR - Bonnerot, Mathieu IR - Bonnerot M FIR - Liao, Liang IR - Liao L FIR - Riou-Comte, Nolwenn IR - Riou-Comte N FIR - Gory, Benjamin IR - Gory B FIR - Richard, Sébastien IR - Richard S FIR - Alexandre, Pierre-Louis IR - Alexandre PL FIR - Daumas-Duport, Benjamin IR - Daumas-Duport B FIR - Desal, Hubert IR - Desal H FIR - Detraz, Lili IR - Detraz L FIR - Preterre, Cécile IR - Preterre C FIR - Bourcier, Romain IR - Bourcier R FIR - Guillon, Benoît IR - Guillon B FIR - Wolff, Valérie IR - Wolff V FIR - Pop, Raoul IR - Pop R FIR - Baptiste, Laura IR - Baptiste L FIR - Comby, Pierre-Olivier IR - Comby PO FIR - Lemogne, Brivael IR - Lemogne B FIR - Thouant, Pierre IR - Thouant P FIR - Bejot, Yannick IR - Bejot Y FIR - Ricolfi, Frédéric IR - Ricolfi F FIR - Merrien, François-Mathias IR - Merrien FM FIR - Nico, Lorena IR - Nico L FIR - Ognard, Julien IR - Ognard J FIR - Simoni, Laurina IR - Simoni L FIR - Gentric, Jean-Christophe IR - Gentric JC FIR - Timsit, Serge IR - Timsit S FIR - Triquenot Bagan, Aude IR - Triquenot Bagan A FIR - Papagiannaki, Chrysanthi IR - Papagiannaki C FIR - Ozkul, Ozlem IR - Ozkul O FIR - Seners, Pierre IR - Seners P FIR - Piotin, Michel IR - Piotin M FIR - Obadia, Michael IR - Obadia M FIR - Bodenant, Marie IR - Bodenant M FIR - Bricout, Nicolas IR - Bricout N FIR - Casolla, Barbara IR - Casolla B FIR - Dequatre, Nelly IR - Dequatre N FIR - Della Shiava, Lucie IR - Della Shiava L FIR - Pasi, Maco IR - Pasi M FIR - Caparros, François IR - Caparros F FIR - Puy, Laurent IR - Puy L FIR - Karam, Arnaud IR - Karam A FIR - Kaaouana, Olfa IR - Kaaouana O FIR - Watel, Kaelig IR - Watel K FIR - Scopelliti, Giuseppe IR - Scopelliti G FIR - Nouri, Nasreddine IR - Nouri N FIR - Henon, Hilde IR - Henon H FIR - Bibi, Richard IR - Bibi R FIR - Bretonniere, Arnaud IR - Bretonniere A FIR - Gaudron, Marie IR - Gaudron M FIR - Herbreteau, Denis IR - Herbreteau D FIR - Ifergan, Héloise IR - Ifergan H FIR - Jannot, Kevin IR - Jannot K FIR - Molinier, Elisabeth IR - Molinier E FIR - Annan, Mariam IR - Annan M FIR - Boulouis, Grégoire IR - Boulouis G FIR - Wiener, Emmanuel IR - Wiener E FIR - Arnoux Courselle, Audrey IR - Arnoux Courselle A FIR - Canaple, Sandrine IR - Canaple S FIR - Ouin, Elisa IR - Ouin E FIR - Chivot, Cyril IR - Chivot C FIR - Lamy, Chantal IR - Lamy C FIR - Saleme, Suzana IR - Saleme S FIR - Mounayer, Charbel IR - Mounayer C FIR - Macian Montoro, Francisco IR - Macian Montoro F FIR - Laubacher, Morgane IR - Laubacher M FIR - Riva, Roberto IR - Riva R FIR - Eker, Omer IR - Eker O FIR - Cho, Tae-Hee IR - 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Dorado Bouix, Laura IR - Dorado Bouix L FIR - Calib, Christopher IR - Calib C FIR - Carbonell, Jaime IR - Carbonell J FIR - Garcia Amor, Gema Eugenia IR - Garcia Amor GE FIR - Ezcurra, Garbiñe IR - Ezcurra G FIR - Flores, Belén IR - Flores B FIR - Gómez Choco, Manuel IR - Gómez Choco M FIR - Gomis Cortina, Meritxell IR - Gomis Cortina M FIR - Hernández Pérez, María IR - Hernández Pérez M FIR - Hernández, Marina IR - Hernández M FIR - Larrañaga, Clara IR - Larrañaga C FIR - Domínguez Lizarbe, Manuel IR - Domínguez Lizarbe M FIR - Martínez, Marina IR - Martínez M FIR - Menéndez Albarracín, Alex IR - Menéndez Albarracín A FIR - Pérez de la Ossa, Natalia IR - Pérez de la Ossa N FIR - Rabaneda, Neus IR - Rabaneda N FIR - Ramos Pachón, Anna IR - Ramos Pachón A FIR - Rodríguez, Luis Alejandro IR - Rodríguez LA FIR - Rubio, Sara IR - Rubio S FIR - Puig, Isabel IR - Puig I FIR - Santana, Daniel 1st IR - Santana D 1st FIR - Valls, Adrian IR - Valls A FIR - Yugueros, Barbara IR - Yugueros B FIR - Wenger, Denisse IR - Wenger D FIR - Millán, Mònica IR - Millán M FIR - Amaro, Sergio IR - Amaro S FIR - Balasa, Mircea IR - Balasa M FIR - Cabrera, Jose Maria IR - Cabrera JM FIR - Chamorro, Ángel IR - Chamorro Á FIR - Conde, Estefania IR - Conde E FIR - Contador, Jose Miguel IR - Contador JM FIR - Doncel-Mariano, Antonio IR - Doncel-Mariano A FIR - Renú, Arturo IR - Renú A FIR - Guasp, Mar IR - Guasp M FIR - Llansó, Laura IR - Llansó L FIR - Llull, Laura IR - Llull L FIR - Mayà Casalprim, Gerard IR - Mayà Casalprim G FIR - Montejo, Carmen IR - Montejo C FIR - Rodríguez, Alejandro IR - Rodríguez A FIR - Rudilosso, Salvatore IR - Rudilosso S FIR - Sánchez, Almudena IR - Sánchez A FIR - Santana, Daniel IR - Santana D FIR - Nuin, Xabier Urra IR - Nuin XU FIR - Vera Monge, Victor Augusto IR - Vera Monge VA FIR - De Lera, Mercedes IR - De Lera M FIR - Ramos, María Esther IR - Ramos ME FIR - Aguirre, Garazi IR - Aguirre G FIR - Cabral, Laura IR - Cabral L FIR - Díaz, Irene IR - Díaz I FIR - Durán Lozano, Alejandro IR - Durán Lozano A FIR - Luna, Alain IR - Luna A FIR - Martín, Jon IR - Martín J FIR - Moreno Estébanez, Ana IR - Moreno Estébanez A FIR - Sifontes, Walter IR - Sifontes W FIR - Ugarriza, Iratxe IR - Ugarriza I FIR - Benavente, Lorena IR - Benavente L FIR - Calleja Puerta, Sergio IR - Calleja Puerta S FIR - Castañón Apilánez, María IR - Castañón Apilánez M FIR - Criado, Álvaro IR - Criado Á FIR - Fuentes, David IR - Fuentes D FIR - García-Cabo, Carmen IR - García-Cabo C FIR - González Delgado, Montserrat IR - González Delgado M FIR - González, Gemma María IR - González GM FIR - Molina Gil, Javier IR - Molina Gil J FIR - Larrosa Campo, Davinia IR - Larrosa Campo D FIR - López López, Begoña IR - López López B FIR - Reguera Acuña, Antía IR - Reguera Acuña A FIR - Rico Santos, María IR - Rico Santos M FIR - Zunzunegui, Patricia IR - Zunzunegui P EDAT- 2024/05/08 18:50 MHDA- 2024/05/08 18:51 CRDT- 2024/05/08 16:23 PHST- 2024/05/08 18:51 [medline] PHST- 2024/05/08 18:50 [pubmed] PHST- 2024/05/08 16:23 [entrez] AID - 10.1056/NEJMoa2314063 [doi] PST - ppublish SO - N Engl J Med. 2024 May 9;390(18):1677-1689. doi: 10.1056/NEJMoa2314063. PMID- 40113367 OWN - NLM STAT- MEDLINE DCOM- 20250320 LR - 20260518 IS - 2352-4650 (Electronic) IS - 2352-4642 (Linking) VI - 9 IP - 4 DP - 2025 Apr TI - Adjunctive dexamethasone and 30-day all-cause death after hospital admission in paediatric pneumococcal meningitis: a propensity score analysis. PG - 255-261 LID - S2352-4642(25)00029-X [pii] LID - 10.1016/S2352-4642(25)00029-X [doi] AB - BACKGROUND: Pneumococcal meningitis is a leading cause of bacterial meningitis and the most deadly pneumococcal disease in children worldwide. There is a paucity of evidence concerning the benefit of dexamethasone to prevent death in paediatric pneumococcal meningitis. We aimed to compare the effect of early adjunctive therapy with dexamethasone versus no dexamethasone on death in children with pneumococcal meningitis. METHODS: We did a non-randomised, comparative, multicentre, retrospective, quasi-experimental, propensity score-based study using data from a French national surveillance system of pneumococcal meningitis in children that collates data for 238 French paediatric wards working with 168 microbiology laboratories. We compared outcomes of adjunctive therapy with dexamethasone treatment (0·15 mg/kg every 6 h, for 4 days, per national guidelines) given within 12 h of antibiotic treatment versus no dexamethasone among all children aged 0-17 years with confirmed pneumococcal meningitis who had been hospitalised in one of the participating centres between Jan 1, 2005, and Nov 1, 2022. The primary outcome was 30-day all-cause death after hospital admission. The main propensity score analysis was based on inverse probability treatment weighting (IPTW), allowing adjustment for initial severity and baseline characteristics. Sensitivity analyses, such as propensity score matching, were done to assess the robustness of the results. FINDINGS: Between Jan 1, 2005, and Nov 1, 2022, 1765 cases of pneumococcal meningitis were reported to the National Surveillance System of Paediatric Bacterial Meningitis. 534 were excluded from the analysis and 1231 were included, with a median age of 1·1 years (IQR 0·5-5·0, range 0-17·9). 495 (40%) of 1231 patients were female, 716 (58%) were male, and 20 (1%) were missing data for sex. 650 (53%) of 1231 children received dexamethasone and 581 (47%) children did not receive dexamethasone. 108 (9%) of 1231 patients died. Within 30 days of hospitalisation, 105 (9%) patients died, 36 (6%) of 650 in the dexamethasone group and 69 (12%) of 581 in the no dexamethasone group. After IPTW, the adjusted 30-day death rate was 6% in the dexamethasone group and 12% in the no dexamethasone group (marginal odds ratio 0·39, 95% CI 0·23-0·65). All sensitivity analyses gave similar results. INTERPRETATION: Adjunctive dexamethasone within 12 h of starting antibiotic treatment was associated with a reduced 30-day risk of death in children hospitalised with pneumococcal meningitis. Our findings support the use of dexamethasone to reduce the risk of death in paediatric pneumococcal meningitis. FUNDING: Pfizer, ACTIV, and National Institute of Health and Medical Research (Inserm) Centre. CI - Copyright © 2025 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Giolito, Anna AU - Giolito A AD - Department of General Paediatrics, Paediatric Infectious Disease and Internal Medicine, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France. FAU - Levy, Corinne AU - Levy C AD - Association Clinique et Thérapeutique Infantile du Val-de-Marne, Créteil, France; IMRB-GRC GEMINI, Centre Hospitalier Intercommunal, Research Centre, Université Paris Est, Créteil, France; French Group of Paediatric Infectious Diseases, Paris, France. FAU - Varon, Emmanuelle AU - Varon E AD - National Reference Centre for Pneumococci, Centre de Recherche Clinique et Biologique, Centre Hospitalier Intercommunal de Créteil, Créteil, France. FAU - Cohen, Robert AU - Cohen R AD - Association Clinique et Thérapeutique Infantile du Val-de-Marne, Créteil, France; IMRB-GRC GEMINI, Centre Hospitalier Intercommunal, Research Centre, Université Paris Est, Créteil, France; French Group of Paediatric Infectious Diseases, Paris, France. FAU - Hanna, Sidonie AU - Hanna S AD - Paediatric Intensive Care Unit, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France. FAU - Assad, Zein AU - Assad Z AD - Department of General Paediatrics, Paediatric Infectious Disease and Internal Medicine, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France; French Group of Paediatric Infectious Diseases, Paris, France. FAU - Lenglart, Léa AU - Lenglart L AD - Paediatric Emergency Department, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France. FAU - Bechet, Stephane AU - Bechet S AD - Association Clinique et Thérapeutique Infantile du Val-de-Marne, Créteil, France; IMRB-GRC GEMINI, Centre Hospitalier Intercommunal, Research Centre, Université Paris Est, Créteil, France; French Group of Paediatric Infectious Diseases, Paris, France. FAU - Bonacorsi, Stephane AU - Bonacorsi S AD - Department of Microbiology, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France; INSERM UMR 1137, Infection, Antimicrobials, Modelling, Evolution, Université Paris Cité, Paris, France. FAU - Dubos, François AU - Dubos F AD - French Group of Paediatric Infectious Diseases, Paris, France; Paediatric Emergency Unit and Infectious Diseases, Centre Hospitalier Universitaire Lille, Université de Lille, Lille, France. FAU - Launay, Elise AU - Launay E AD - French Group of Paediatric Infectious Diseases, Paris, France; Department of Paediatrics, Centre Hospitalier Universitaire Nantes, Université de Nantes, Nantes, France. FAU - Pelleter, Morgane AU - Pelleter M AD - Department of Paediatrics, Centre Hospitalier Universitaire Nantes, Université de Nantes, Nantes, France. FAU - Rybak, Alexis AU - Rybak A AD - Association Clinique et Thérapeutique Infantile du Val-de-Marne, Créteil, France; Department of Paediatrics, Department Woman-Mother-Child, Lausanne University Hospital (Centre Hospitalier Universitaire Vaudois), Lausanne, Vaud, Switzerland. FAU - Angoulvant, Francois AU - Angoulvant F AD - Association Clinique et Thérapeutique Infantile du Val-de-Marne, Créteil, France; Department of Paediatrics, Department Woman-Mother-Child, Lausanne University Hospital (Centre Hospitalier Universitaire Vaudois), Lausanne, Vaud, Switzerland. FAU - Levy, Michael AU - Levy M AD - Paediatric Intensive Care Unit, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France. FAU - Ouldali, Naïm AU - Ouldali N AD - Department of General Paediatrics, Paediatric Infectious Disease and Internal Medicine, Robert Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France; French Group of Paediatric Infectious Diseases, Paris, France; INSERM UMR 1137, Infection, Antimicrobials, Modelling, Evolution, Université Paris Cité, Paris, France. Electronic address: naim.ouldali@aphp.fr. CN - French Paediatric Meningitis Network LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PL - England TA - Lancet Child Adolesc Health JT - The Lancet. Child & adolescent health JID - 101712925 RN - 7S5I7G3JQL (Dexamethasone) RN - 0 (Anti-Bacterial Agents) RN - 0 (Glucocorticoids) SB - IM CIN - Lancet Child Adolesc Health. 2025 Apr;9(4):213-215. doi: 10.1016/S2352-4642(25)00066-5. PMID: 40113362 MH - Humans MH - *Dexamethasone/therapeutic use/administration & dosage MH - *Meningitis, Pneumococcal/mortality/drug therapy MH - Child, Preschool MH - Male MH - Propensity Score MH - Female MH - Child MH - Infant MH - Retrospective Studies MH - Adolescent MH - Anti-Bacterial Agents/therapeutic use MH - France/epidemiology MH - Hospitalization MH - Infant, Newborn MH - Cause of Death MH - *Glucocorticoids/therapeutic use COIS- Declaration of interests NO reports travel grants from Pfizer, GSK, Merck, and Sanofi. CL reports travel grants from Pfizer and personal fees from Pfizer and MSD. AR reports travels grants from Pfizer and personal fees from MSD and Sanofi. All other authors declare no competing interests. FIR - Abadie, Véronique IR - Abadie V FIR - Abdanne, Mohamed IR - Abdanne M FIR - Abdelhadi, Mohamed IR - Abdelhadi M FIR - Aberrane, Said IR - Aberrane S FIR - Ackermann, Steffen IR - Ackermann S FIR - Adam, Marie-Noëlle IR - Adam MN FIR - Adamon, Latif IR - Adamon L FIR - Adi, Ghassan IR - Adi G FIR - Akitani, Sylvia IR - Akitani S FIR - Al Jazayri, Ziad IR - Al Jazayri Z FIR - Al Junaidi, Ashraf IR - Al Junaidi A FIR - Al Mazini, Céline IR - Al Mazini C FIR - Alba-Sauviat, Catherine IR - Alba-Sauviat C FIR - Albertini, Marie-Thérèse IR - Albertini MT FIR - Allia, Abdelatif IR - Allia A FIR - Allouche, Chantal IR - Allouche C FIR - Amama, Habib IR - Amama H FIR - Amara, Marlene IR - Amara M FIR - Amirault, Patrick IR - Amirault P FIR - Amsallem, Daniel IR - Amsallem D FIR - Andre, Maud IR - Andre M FIR - Andriantahina, Isabelle IR - Andriantahina I FIR - Anxionnat, Raphaël IR - Anxionnat R FIR - Arlet, Guillaume IR - Arlet G FIR - Armouche, Samar IR - Armouche S FIR - Astruc, Dominique IR - 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Chahine, Jamilé IR - Chahine J FIR - Chaix, Yves IR - Chaix Y FIR - Chalumeau, Martin IR - Chalumeau M FIR - Chalvon Demersay, Arnaud IR - Chalvon Demersay A FIR - Chamouilli, Jean-Marc IR - Chamouilli JM FIR - Champion, Valérie IR - Champion V FIR - Chantelat, Pascal IR - Chantelat P FIR - Chantreuil, Julie IR - Chantreuil J FIR - Chaplain, Chantal IR - Chaplain C FIR - Charachon, Sylvie IR - Charachon S FIR - Charbonneau, Anne IR - Charbonneau A FIR - Chardon, Hubert IR - Chardon H FIR - Charpentier, Sabine IR - Charpentier S FIR - Charreton, Claire IR - Charreton C FIR - Chellun, Pamela IR - Chellun P FIR - Chenel, Claude IR - Chenel C FIR - Cheron, Mireille IR - Cheron M FIR - Cheuret, Emmanuel IR - Cheuret E FIR - Chevallier, Bertrand IR - Chevallier B FIR - Chevrel, Jean IR - Chevrel J FIR - Chevret, Laurent IR - Chevret L FIR - Chognot, Didier IR - Chognot D FIR - Chomienne, Francis IR - Chomienne F FIR - Chosidow, Anais IR - Chosidow A FIR - Chrisment, Delphine IR - Chrisment D FIR - Ciupek, Catherine IR - Ciupek C FIR - Clair, Perrine IR - Clair P FIR - Claris, Olivier IR - Claris O FIR - Clavel, Catherine IR - Clavel C FIR - Cochat, Pierre IR - Cochat P FIR - Coche, Delphine IR - Coche D FIR - Coinde, Edeline IR - Coinde E FIR - Colombani, Jean-Claude IR - Colombani JC FIR - Combe, Jean Charles IR - Combe JC FIR - Cordier, Anne-Marie IR - Cordier AM FIR - Cormier, Philippe IR - Cormier P FIR - Corniau, Francoise IR - Corniau F FIR - Costa, Guillaume IR - Costa G FIR - Costa, Yannick IR - Costa Y FIR - Cottin, Jane IR - Cottin J FIR - Coumenges, Pascal IR - Coumenges P FIR - Courcol, René IR - Courcol R FIR - Courdavault, Laurence IR - Courdavault L FIR - Courouble, Gery IR - Courouble G FIR - Courtade, Henri IR - Courtade H FIR - Coutable, Audrey IR - Coutable A FIR - Craiu, Irina IR - Craiu I FIR - Crepet, Françoise IR - Crepet F FIR - DA, Edwige IR - DA E FIR - Daire-Chabanon, Isabelle IR - Daire-Chabanon I FIR - Dalmon, Fabienne IR - Dalmon F FIR - Danan, Claude IR - Danan C FIR - Danekova, Nevena IR - Danekova N FIR - Danjean-Deguin, Marie-Pierre IR - Danjean-Deguin MP FIR - Danquigny, Anne Laure IR - Danquigny AL FIR - Dao-Dubremetz, Anne IR - Dao-Dubremetz A FIR - Daoud, Patrick IR - Daoud P FIR - Daoudi, Florence IR - Daoudi F FIR - Darai, Emile IR - Darai E FIR - Darnaud, Cécile IR - Darnaud C FIR - Darras, Jean Philippe IR - Darras JP FIR - Dassieu, Gilles IR - Dassieu G FIR - Dauger, Stéphane IR - Dauger S FIR - DE Barbentane, Marie-Christine IR - DE Barbentane MC FIR - DE Champs, Christophe IR - DE Champs C FIR - DE Decker, Laurent IR - DE Decker L FIR - DE Montclos, Henri IR - DE Montclos H FIR - DE Pontual, Loïc IR - DE Pontual L FIR - Debriel, Dominique IR - Debriel D FIR - Decoster, Anne IR - Decoster A FIR - Degas, Vanessa IR - Degas V FIR - Delacour, Thierry IR - Delacour T FIR - Delafay, Marie IR - Delafay M FIR - Delamare, Catherine IR - Delamare C FIR - Delaporte, Gilles IR - Delaporte G FIR - Delarbre, Jean-Marie IR - Delarbre JM FIR - Delavelle, Chantal IR - Delavelle C FIR - Delbeke, Emmanuel IR - Delbeke E FIR - Delesalle, Sophie IR - Delesalle S FIR - Deligne, Delphine IR - Deligne D FIR - Delimele, Marylin IR - Delimele M FIR - Delorme, Delphine IR - Delorme D FIR - Deluca, Daniele IR - Deluca D FIR - Delvart, Céline IR - Delvart C FIR - Demachy, Marie-Claude IR - Demachy MC FIR - Demarcq, Marie-Joelle IR - Demarcq MJ FIR - Demarque, Nathalie IR - Demarque N FIR - Demayer, Nadia IR - Demayer N FIR - Demay-Legros, Gaelle IR - Demay-Legros G FIR - Demonchy, Diane IR - Demonchy D FIR - Desbois, Delphine IR - Desbois D FIR - Deschamps, Nina IR - Deschamps N FIR - Desfrere, Luc IR - Desfrere L FIR - Deshayes DE Cambronne, Romain IR - Deshayes DE Cambronne R FIR - Desse, Blandine IR - Desse B FIR - Dessein, Rodrigue IR - Dessein R FIR - Dessioux, Emmanuelle IR - Dessioux E FIR - Desson, Julienne IR - Desson J FIR - Desvigne, Ghislaine IR - Desvigne G FIR - Deville, E IR - Deville E FIR - Dewitte, Aude IR - Dewitte A FIR - Dhaoui, Tahar IR - Dhaoui T FIR - Didier-Wright, Catherine IR - Didier-Wright C FIR - Dieckmann, Katherine IR - Dieckmann K FIR - Doit, Catherine IR - Doit C FIR - Dolfi Fiette, Hélène IR - Dolfi Fiette H FIR - Dolhem, Philippe IR - Dolhem P FIR - Dommergues, Marie-Aliette IR - Dommergues MA FIR - Dorangeon, Elodie IR - Dorangeon E FIR - Doucet-Populaire, Florence IR - Doucet-Populaire F FIR - Dubois, Franck IR - Dubois F FIR - Dubourdieu, Béatrice IR - Dubourdieu B FIR - Ducrocq, Sarah IR - Ducrocq S FIR - Dudin, Anwar IR - Dudin A FIR - Dufour, Marie-José IR - Dufour MJ FIR - Dumoulard, Bruno IR - Dumoulard B FIR - Dupre, Jean-Michel IR - Dupre JM FIR - Durand, Marie-France IR - Durand MF FIR - Duthilly, Arnaud IR - Duthilly A FIR - Dutron, Sarah IR - Dutron S FIR - Duval, Véronique IR - Duval V FIR - Eicher, Emmanuel IR - Eicher E FIR - Eitenschenck, Laurence IR - Eitenschenck L FIR - El Hamri, Mohamed IR - El Hamri M FIR - Elena-Daumas, Martine IR - Elena-Daumas M FIR - Elharrif, Zoubida IR - Elharrif Z FIR - Elias, Ossam IR - Elias O FIR - Emond, Jean-Philippe IR - Emond JP FIR - Enchery, Sophie IR - Enchery S FIR - Epaud, Ralph IR - Epaud R FIR - Escoba, Simon IR - Escoba S FIR - Estapa, Laurence IR - Estapa L FIR - Esteve, Vincent IR - Esteve V FIR - Estevez, Cyrielle IR - Estevez C FIR - Etienne, Jerôme IR - Etienne J FIR - Evers, Annie IR - Evers A FIR - Evrard, Dominique IR - Evrard D FIR - Evreux, Françoise IR - Evreux F FIR - Eyer, Didier IR - Eyer D FIR - Eyssette-Guerreau, Stéphanie IR - Eyssette-Guerreau S FIR - Fabbro, Christelle IR - Fabbro C FIR - Faibis, Frédéric IR - Faibis F FIR - Fargeot Espaliat, Anne IR - Fargeot Espaliat A FIR - Farges, Anne IR - Farges A FIR - Farhat, Skander IR - Farhat S FIR - Faul, Valérie IR - Faul V FIR - Favaretto, Giovanni IR - Favaretto G FIR - Favier, Marion IR - Favier M FIR - Faye, Albert IR - Faye A FIR - Feldmann, Marc IR - Feldmann M FIR - Ferey, Janine IR - Ferey J FIR - Ferroni, Agnès IR - Ferroni A FIR - Fiette, Hélène IR - Fiette H FIR - Filippi, Jean-François IR - Filippi JF FIR - Filleron, Anne IR - Filleron A FIR - Flipo, Jean-Louis IR - Flipo JL FIR - Flurin, Vincent IR - Flurin V FIR - Foca, Michaela IR - Foca M FIR - Foix, Laurence IR - Foix L FIR - Fos, Marie Pierre IR - Fos MP FIR - Francois-Chervet, Catherine IR - Francois-Chervet C FIR - Frey, Ulrike IR - Frey U FIR - Gaillard, Jean-Louis IR - Gaillard JL FIR - Gaillot, Théophile IR - Gaillot T FIR - Gajdos, Vincent IR - Gajdos V FIR - Gallet, Serge IR - Gallet S FIR - Gallou, Gildas IR - Gallou G FIR - Ganivala, Idris IR - Ganivala I FIR - Garandeau, Caroline IR - Garandeau C FIR - Garbarg-Chenon, Antoine IR - Garbarg-Chenon A FIR - Garcera, Yves IR - Garcera Y FIR - Garcia Sanchez, Claudio IR - Garcia Sanchez C FIR - Garnier, Fabien IR - Garnier F FIR - Garrabe, Eulalie IR - Garrabe E FIR - Gaschet, Anne IR - Gaschet A FIR - Gaschignard, Jean IR - Gaschignard J FIR - Gascoin, Géraldine IR - Gascoin G FIR - Gaudelus, Joël IR - Gaudelus J FIR - Gauduchon, Valérie IR - Gauduchon V FIR - Gavignet, Martine IR - Gavignet M FIR - Geffroy, Françoise IR - Geffroy F FIR - Geniez, Laurent IR - Geniez L FIR - Georget, Emilie IR - Georget E FIR - Gerard, Sophie IR - Gerard S FIR - Geraud, Justine IR - Geraud J FIR - Ghosn, Ezat IR - Ghosn E FIR - Giacobbi Milet, Vannina IR - Giacobbi Milet V FIR - Gilles, Isabelle IR - Gilles I FIR - Gillet, Yves IR - Gillet Y FIR - Giorgi, Catherine IR - Giorgi C FIR - Girard, Florent IR - Girard F FIR - Girard, Juliette IR - Girard J FIR - Giraudon, Adeline IR - Giraudon A FIR - Giroux, Nathan IR - Giroux N FIR - Glatz, Isabelle IR - Glatz I FIR - Gleize, Elodie IR - Gleize E FIR - Gobet, Sophie IR - Gobet S FIR - Goguelin, Annie IR - Goguelin A FIR - Goissen, Celine IR - Goissen C FIR - Goudeau, Alain IR - Goudeau A FIR - Gougeon, Anne IR - Gougeon A FIR - Goumy, Pierre IR - Goumy P FIR - Gouraud, François IR - Gouraud F FIR - Gouriet, Frédérique IR - Gouriet F FIR - Goux, Alain IR - Goux A FIR - Grailles, Aurélie IR - Grailles A FIR - Graine, Houria IR - Graine H FIR - Grancher, Céline IR - Grancher C FIR - Grando, Anna IR - Grando A FIR - Gras-LE-Guen, Christelle IR - Gras-LE-Guen C FIR - Greco, Magali IR - Greco M FIR - Gressier, Bernard IR - Gressier B FIR - Grimal, Isabelle IR - Grimal I FIR - Grimprel, Emmanuel IR - Grimprel E FIR - Grise, Geneviève IR - Grise G FIR - Gruss, Richard IR - Gruss R FIR - Guerin, François IR - Guerin F FIR - Guibert, Sophie IR - Guibert S FIR - Guichard, Henriette IR - Guichard H FIR - Guiddir, Tamazoust IR - Guiddir T FIR - Guiet, Pascal IR - Guiet P FIR - Guigonis, Vincent IR - Guigonis V FIR - Guilbert, Julia IR - Guilbert J FIR - Guillermet Fromentin, Christine IR - Guillermet Fromentin C FIR - Guilluy, Olivier IR - Guilluy O FIR - Guitton, Corinne IR - Guitton C FIR - Haas, Andreas IR - Haas A FIR - Haas, Hervé IR - Haas H FIR - Habib, Alexandre IR - Habib A FIR - Hachani, Abdelatif IR - Hachani A FIR - Hachem, Guilda IR - Hachem G FIR - Hadjadj, Sarah IR - Hadjadj S FIR - Haegy-Doehring, Isabelle IR - Haegy-Doehring I FIR - Hage, Mahmad IR - Hage M FIR - Hage Chehade, Mohamed IR - Hage Chehade M FIR - Hallage, Houria IR - Hallage H FIR - Hallalel, Fazia IR - Hallalel F FIR - Halna, Muriel IR - Halna M FIR - Hamdad, Farida IR - Hamdad F FIR - Haouisee, Severine IR - Haouisee S FIR - Harchaoui, Samir IR - Harchaoui S FIR - Hatahet, Rihad IR - Hatahet R FIR - Hay, Fréderique IR - Hay F FIR - Hedjem, Noureddine IR - Hedjem N FIR - Hees, Laure IR - Hees L FIR - Heidt, Annie IR - Heidt A FIR - Hentgen, Véronique IR - Hentgen V FIR - Heraud, Marie-Christine IR - Heraud MC FIR - Herve, Christian IR - Herve C FIR - Heurte, Joelle IR - Heurte J FIR - Heusse, Emmanuelle IR - Heusse E FIR - Heyman, Rachel IR - Heyman R FIR - Hivert, Céline IR - Hivert C FIR - Hochart, Anne-Cécile IR - Hochart AC FIR - Hombrouck-Alet, Cécile IR - Hombrouck-Alet C FIR - Honoré, Stéphanie IR - Honoré S FIR - Horea, Cosmina IR - Horea C FIR - Hubert, Nathalie IR - Hubert N FIR - Hubert, Philippe IR - Hubert P FIR - Huet, Frédéric IR - Huet F FIR - Huin, Nicole IR - Huin N FIR - Huvenne, Hélène IR - Huvenne H FIR - Idres, Nourredine IR - Idres N FIR - Ikounga, Patrick IR - Ikounga P FIR - Ingrao, Tara IR - Ingrao T FIR - Irakotoh, Jacques IR - Irakotoh J FIR - Jacob, Jean Louis IR - Jacob JL FIR - Jacquet, Coralie IR - Jacquet C FIR - Jalloul, Mohamad IR - Jalloul M FIR - Jan, Didier IR - Jan D FIR - Jaouen, Anne-Christinne IR - Jaouen AC FIR - Jarlier, Vincent IR - Jarlier V FIR - Jarreau, Pierre-Henri IR - Jarreau PH FIR - Jaulhac, Benoit IR - Jaulhac B FIR - Javouhey, Etienne IR - Javouhey E FIR - Jehan, Josette IR - Jehan J FIR - Jensen, Cecile IR - Jensen C FIR - Jeziorski, Eric IR - Jeziorski E FIR - Jokic, Mikael IR - Jokic M FIR - Joly-Guillou, Marie-Laure IR - Joly-Guillou ML FIR - Joly-Sanchez, Lydie IR - Joly-Sanchez L FIR - Joram, Nicolas IR - Joram N FIR - Jorion, Claude IR - Jorion C FIR - Jullian, Eric IR - Jullian E FIR - Juvin, Marie-Emmanuelle IR - Juvin ME FIR - Kalach, Nicolas IR - Kalach N FIR - Kayal, Samer IR - Kayal S FIR - Khaled, Mohamed IR - Khaled M FIR - Khalfi, Ali IR - Khalfi A FIR - Khorsi, Slimane IR - Khorsi S FIR - Kieffer, Francois IR - Kieffer F FIR - Kitzis, Marie-Dominique IR - Kitzis MD FIR - Kom, Rémi IR - Kom R FIR - Kone-Paut, Isabelle IR - Kone-Paut I FIR - Kovacs, Tamas IR - Kovacs T FIR - Kozisek, Stanislas IR - Kozisek S FIR - Kucerova, Johana IR - Kucerova J FIR - Kuntzel, Bernard IR - Kuntzel B FIR - Labarthe, Francois IR - Labarthe F FIR - Labenne, Marc IR - Labenne M FIR - Laborie, Marie-Jose IR - Laborie MJ FIR - Labrune, Philippe IR - Labrune P FIR - Lacroix, Delphine IR - Lacroix D FIR - Lafendi, Abdelhamid IR - Lafendi A FIR - Lagier, Evelyne IR - Lagier E FIR - Lagier, Jeremy IR - Lagier J FIR - Lagree, Marion IR - Lagree M FIR - Lahrach, Hakim IR - Lahrach H FIR - Laidj IR - Laidj FIR - Laisney, Norbert IR - Laisney N FIR - Lakhdari, Mustapha IR - Lakhdari M FIR - Lamant, Marie IR - Lamant M FIR - Lamarcq, Mélanie IR - Lamarcq M FIR - Lambert, Christine IR - Lambert C FIR - Lamoureux, Sylvie IR - Lamoureux S FIR - Landragin, Danielle IR - Landragin D FIR - Lanotte, Philippe IR - Lanotte P FIR - Laoudi, Yacine IR - Laoudi Y FIR - Lapeyre, Delphine IR - Lapeyre D FIR - Lapeyre, Fabrice IR - Lapeyre F FIR - Lapostolle, Claire IR - Lapostolle C FIR - Laugel, Vincent IR - Laugel V FIR - Laulan, Afaf IR - Laulan A FIR - Launay, Elise IR - Launay E FIR - Lavigne, Jean-Philippe IR - Lavigne JP FIR - LE Bideau, Marc IR - LE Bideau M FIR - LE Coustumier, Alain IR - LE Coustumier A FIR - Leboucher, Bertrand IR - Leboucher B FIR - Lecine, Thierry IR - Lecine T FIR - Ledru, Sylvie IR - Ledru S FIR - Lefevre, Fabrice IR - Lefevre F FIR - Legagneur, Michel IR - Legagneur M FIR - Legalle, Marie-Ange IR - Legalle MA FIR - Leger, Pierre-Louis IR - Leger PL FIR - Legros, Antoine IR - Legros A FIR - Lehours, Philippe IR - Lehours P FIR - Lelievre, Nathalie IR - Lelievre N FIR - Lelioux, Jean IR - Lelioux J FIR - Leluan, Philippe IR - Leluan P FIR - Lemaitre, Nadine IR - Lemaitre N FIR - Lemarié, Carole IR - Lemarié C FIR - Lemble, Chantal IR - Lemble C FIR - Lemenand, Olivier IR - Lemenand O FIR - Leneveu, Michel IR - Leneveu M FIR - Leotard, Sophie IR - Leotard S FIR - Lepage, Jm IR - Lepage J FIR - Leret, Nathalie IR - Leret N FIR - Lerouge-Bailhache, Marion IR - Lerouge-Bailhache M FIR - Leroux, Pascal IR - Leroux P FIR - Lesage, Fabrice IR - Lesage F FIR - Lescanne, Emmanuel IR - Lescanne E FIR - Letellier, Claire IR - Letellier C FIR - Leteurtre, Stéphane IR - Leteurtre S FIR - Letreust, Muriel IR - Letreust M FIR - Leuvrey, Maeva IR - Leuvrey M FIR - Levast, Marion IR - Levast M FIR - Levy, Marc IR - Levy M FIR - Levy, Michael IR - Levy M FIR - Levy, Pierre-Yves IR - Levy PY FIR - Lienhardt, Anne IR - Lienhardt A FIR - Ligios, Agnès IR - Ligios A FIR - Long, Laurence IR - Long L FIR - Lorrot, Mathie IR - Lorrot M FIR - Louf, Sylvie IR - Louf S FIR - Loznewski, Alain IR - Loznewski A FIR - Lureau, Pierre IR - Lureau P FIR - Maakaroun-Vermesse, Zoha IR - Maakaroun-Vermesse Z FIR - Macchi, Pascal IR - Macchi P FIR - Madhi, Fouad IR - Madhi F FIR - Mager, Guy IR - Mager G FIR - Magny, Jean-François IR - Magny JF FIR - Malige, Laurence IR - Malige L FIR - Mammes, Olivier IR - Mammes O FIR - Mandelcwajg, Alexis IR - Mandelcwajg A FIR - Mandjee, Aziza IR - Mandjee A FIR - Mangeol, Alain IR - Mangeol A FIR - Manin, Cécile IR - Manin C FIR - Mansir, Thierry IR - Mansir T FIR - Manteau, Céline IR - Manteau C FIR - Marcou, Valérie IR - Marcou V FIR - Marguet, Christophe IR - Marguet C FIR - Mariette, Sylvie IR - Mariette S FIR - Marin, Mariana IR - Marin M FIR - Marmonier, Alain IR - Marmonier A FIR - Marret, Stéphane IR - Marret S FIR - Martha, Sandrine Anne IR - Martha SA FIR - Martin, Claude IR - Martin C FIR - Martinat, Laurence IR - Martinat L FIR - Martinez Ozenda, Muriel IR - Martinez Ozenda M FIR - Marty, Sophie IR - Marty S FIR - Masserot, Caroline IR - Masserot C FIR - Mathieu, Laurent IR - Mathieu L FIR - Mathieu, Pierre IR - Mathieu P FIR - Matoussi, Zied IR - Matoussi Z FIR - Maugard, Thierry IR - Maugard T FIR - Maurin, Damien IR - Maurin D FIR - Mazataud, Richard IR - Mazataud R FIR - Mazeghrane, Mustapha IR - Mazeghrane M FIR - M'bamba, Célestin IR - M'bamba C FIR - Mellier, Joelle IR - Mellier J FIR - Melon, Monique IR - Melon M FIR - Menager, Cedric IR - Menager C FIR - Menguy, Anne-Claude IR - Menguy AC FIR - Menouar, Mohamed IR - Menouar M FIR - Menouni-Foray, Odile IR - Menouni-Foray O FIR - Merlin, Etienne IR - Merlin E FIR - Merlot, Elodie IR - Merlot E FIR - Mermet Jeanvoine, Fabienne IR - Mermet Jeanvoine F FIR - Mermond, Sylvain IR - Mermond S FIR - Meslin, Pauline IR - Meslin P FIR - Meunier, Patricia IR - Meunier P FIR - Mezgueldi, Ellia IR - Mezgueldi E FIR - Micaelo, Maite IR - Micaelo M FIR - Michel, Fabrice IR - Michel F FIR - Mignard, Sophie IR - Mignard S FIR - Mignot, Loïc IR - Mignot L FIR - Mikail, Izzat IR - Mikail I FIR - Milcent, Karen IR - Milcent K FIR - Milh, Mathieu IR - Milh M FIR - Milleret-Proyart, Marie-José IR - Milleret-Proyart MJ FIR - Millou, Catherine IR - Millou C FIR - Minodier, Philippe IR - Minodier P FIR - Monceau, Luc IR - Monceau L FIR - Moquet, Olivier IR - Moquet O FIR - Moreigne, Michel IR - Moreigne M FIR - Morisot, Cyril IR - Morisot C FIR - Morle, Sophie IR - Morle S FIR - Mornand, Pierre IR - Mornand P FIR - Moulene, Eric IR - Moulene E FIR - Moulin, Pierre IR - Moulin P FIR - Mouly, Laurence IR - Mouly L FIR - Mowendabeka, Audrey IR - Mowendabeka A FIR - Muller, Stéphanie IR - Muller S FIR - Murbach, Valérie IR - Murbach V FIR - Nakhleh, Jean IR - Nakhleh J FIR - Nathanson, Sylvie IR - Nathanson S FIR - Naudot, Xavier IR - Naudot X FIR - Navarro, Caroline IR - Navarro C FIR - Nemes, Raluca IR - Nemes R FIR - Neri Schiavini, Dominique IR - Neri Schiavini D FIR - Nerome, Simone IR - Nerome S FIR - Neuwirth, Catherine IR - Neuwirth C FIR - Ngalula, Guy IR - Ngalula G FIR - Nguyen, Dzu Joel IR - Nguyen DJ FIR - Nicola, Nawal IR - Nicola N FIR - Ninot, Alain IR - Ninot A FIR - Nobili-Dubarry, Céline IR - Nobili-Dubarry C FIR - Noulard, Marie-Noëlle IR - Noulard MN FIR - Osman, Hatem IR - Osman H FIR - Osman, Zafer IR - Osman Z FIR - Ould Hocine, Houria IR - Ould Hocine H FIR - Ouldali, Naim IR - Ouldali N FIR - Oulebsir, Abdelaziz IR - Oulebsir A FIR - Oules, Olivier IR - Oules O FIR - Ouziel, Antoine IR - Ouziel A FIR - Pages, Anne-Sophie IR - Pages AS FIR - Pages, Marie-Christine IR - Pages MC FIR - Paget, Corinne IR - Paget C FIR - Palette, Xavier IR - Palette X FIR - Pancher, Marie IR - Pancher M FIR - Pantalone, Letitia IR - Pantalone L FIR - Paon, Jean-Christophe IR - Paon JC FIR - Pateyron, Fabienne IR - Pateyron F FIR - Patoz, Pierre IR - Patoz P FIR - Paul, Gérard IR - Paul G FIR - Paul, Jean-Gabriel IR - Paul JG FIR - Pauquet, Emilie IR - Pauquet E FIR - Peigne, Chantal IR - Peigne C FIR - Pejoan, Hélène IR - Pejoan H FIR - Pellegrino, Béatrice IR - Pellegrino B FIR - Pelloux, Isabelle IR - Pelloux I FIR - Penaud, Antoni IR - Penaud A FIR - Penel, Delphine IR - Penel D FIR - Pere, Bastien IR - Pere B FIR - Perez, Noémie IR - Perez N FIR - Perrier, Coline IR - Perrier C FIR - Pesenti, Delphine IR - Pesenti D FIR - Petitboulanger, Nelly IR - Petitboulanger N FIR - Phan, Florence IR - Phan F FIR - Pharaon, Isabelle IR - Pharaon I FIR - Pierre, Marie-Hélène IR - Pierre MH FIR - Pierrejean, Denys IR - Pierrejean D FIR - Pietrement, Christine IR - Pietrement C FIR - Pina, Patrick IR - Pina P FIR - Pindi, Béatrice IR - Pindi B FIR - Pinlou, Elisabeth IR - Pinlou E FIR - Pinquier, Didier IR - Pinquier D FIR - Pinturier, Marie-Frédérique IR - Pinturier MF FIR - Plaisant, Franck IR - Plaisant F FIR - Planchenault, Deborah IR - Planchenault D FIR - Plassart, Claire IR - Plassart C FIR - Ploton, Marie-Caroline IR - Ploton MC FIR - Plouvier, Emmanuel IR - Plouvier E FIR - Poggioli, Isabelle IR - Poggioli I FIR - Poilane, Isabelle IR - Poilane I FIR - Pons, Charlotte IR - Pons C FIR - Porcheret Huong, Thi Ngoc IR - Porcheret Huong TN FIR - 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Tronc, Frédéric IR - Tronc F FIR - Truong, Jeanne IR - Truong J FIR - Ursulescu, Nicoletta IR - Ursulescu N FIR - Vachee, Anne IR - Vachee A FIR - Vaillant, Christine IR - Vaillant C FIR - Valade, Aurélie IR - Valade A FIR - Valayer, Patrick IR - Valayer P FIR - Valensi, Mickaël IR - Valensi M FIR - Vallee, Eric IR - Vallee E FIR - Vallet, Christelle IR - Vallet C FIR - VAN DE Perre, Philippe IR - VAN DE Perre P FIR - Vandenesch, François IR - Vandenesch F FIR - Vanel, Noémie IR - Vanel N FIR - Vaucel, Jacques IR - Vaucel J FIR - Veauvy-Juven, Agnès IR - Veauvy-Juven A FIR - Venot, Christine IR - Venot C FIR - Verdan, Matthieu IR - Verdan M FIR - Verdet, Charlotte IR - Verdet C FIR - Vergnaud, Michel IR - Vergnaud M FIR - Vergne, Christiane IR - Vergne C FIR - Vernet-Garnier, Véronique IR - Vernet-Garnier V FIR - Vervel, Christine IR - Vervel C FIR - Viala, Céline IR - Viala C FIR - Vialette, Véronique IR - Vialette V FIR - Vic, Philippe IR - Vic P FIR - Vignaud, Olivier IR - Vignaud O FIR - Violette, Jérémie IR - Violette J FIR - Vittecoq, Catherine IR - Vittecoq C FIR - Vu Thien, Hoang IR - Vu Thien H FIR - Wasels, Richard IR - Wasels R FIR - Wiedemann-Fode, Arnaud IR - Wiedemann-Fode A FIR - Worcel, Isabelle IR - Worcel I FIR - Yangui, Mohamed Amine IR - Yangui MA FIR - Youssef William, William Azzam IR - Youssef William WA FIR - Ythier, Hubert IR - Ythier H FIR - Zarzour, Ali IR - Zarzour A FIR - Zelinsky-Gurung, Arianne IR - Zelinsky-Gurung A FIR - Zemouri, Neila IR - Zemouri N FIR - Zenkhri, Ferielle IR - Zenkhri F FIR - Zimmermann, Brigitte IR - Zimmermann B FIR - Zini, Justine IR - Zini J EDAT- 2025/03/21 00:22 MHDA- 2025/03/21 00:23 CRDT- 2025/03/20 21:57 PHST- 2024/09/04 00:00 [received] PHST- 2025/01/29 00:00 [revised] PHST- 2025/02/03 00:00 [accepted] PHST- 2025/03/21 00:23 [medline] PHST- 2025/03/21 00:22 [pubmed] PHST- 2025/03/20 21:57 [entrez] AID - S2352-4642(25)00029-X [pii] AID - 10.1016/S2352-4642(25)00029-X [doi] PST - ppublish SO - Lancet Child Adolesc Health. 2025 Apr;9(4):255-261. doi: 10.1016/S2352-4642(25)00029-X. PMID- 42107392 OWN - NLM STAT- MEDLINE DCOM- 20260521 LR - 20260527 IS - 1474-547X (Electronic) IS - 0140-6736 (Linking) VI - 407 IP - 10543 DP - 2026 May 23 TI - Endovascular thrombectomy for patients with large-core ischaemic stroke presenting up to 24 h after onset (ATLAS): a systematic review and individual patient data meta-analysis with central imaging adjudication. PG - 2015-2026 LID - S0140-6736(26)00876-7 [pii] LID - 10.1016/S0140-6736(26)00876-7 [doi] AB - BACKGROUND: Patients with extensive ischaemic change are often excluded from endovascular thrombectomy. We aimed to synthesise the evidence from recent trials in these patients by performing a systematic review and individual patient data meta-analysis to estimate treatment benefit, including within clinical and imaging subgroups. METHODS: In this systematic review and meta-analysis, we searched PubMed and Embase for randomised trials published between March 1, 2018, and March 1, 2025, that evaluated efficacy and safety of endovascular thrombectomy compared with medical management in patients with large-core ischaemic stroke (based on an Alberta Stroke Program Early CT Score [ASPECTS] of ≤5 or estimated ischaemic core ≥50 mL) presenting within 24 h of onset. Individual patient-level data from all eligible trials were obtained. A central imaging core laboratory readjudicated ASPECTS and reanalysed ischaemic core volume. A two-stage meta-analysis with random-effects model was used to evaluate the distribution of 90-day modified Rankin Scale (mRS) scores (the primary outcome) using adjusted pooled generalised odds ratios (aGenORs). Missing data were handled by multiple imputation. Safety outcomes were all-cause mortality within 90-day follow-up and neurological worsening within 24-48 h of randomisation, reported as adjusted pooled relative risk (aRR); and symptomatic intracerebral haemorrhage within 36 h of randomisation (reported as risk difference). Subgroup analyses based on clinical and imaging characteristics were done, including subgroups defined by ischaemic core volume, ASPECTS, and time window from onset to randomisation. The meta-analysis was registered with PROSPERO (CRD420251058584). FINDINGS: We included 1886 patients (944 assigned to endovascular thrombectomy and 942 assigned to medical management) from six trials. Baseline characteristics were similar between treatment groups. At day 90, the distribution of mRS scores was improved in patients in the endovascular thrombectomy group (median score 4 [IQR 3-6]; n=940) versus those in the medical management group (5 [4-6]; n=931; aGenOR 1·63 [95% CI 1·42-1·88], p<0·0001). The endovascular thrombectomy group also had reduced mortality (292 [31·1%]) compared with the medical management group (347 [37·3%]; aRR 0·82 [95% CI 0·70-0·97], p=0·022). No significant differences were observed in symptomatic intracranial haemorrhage (ten [1·1%] of 944 vs nine [1·0%] of 942 patients; pooled unadjusted risk difference -0·17 percentage points [95% CI -1·01 to 0·67], p=0·69) or neurological worsening (197 [22·0%] of 896 patients vs 161 [17·9%] of 899; aRR 1·19 [0·87-1·62], p=0·27). Improved functional outcomes with endovascular thrombectomy were consistent across clinical and imaging subgroups, except for those with an estimated ischaemic core volume of 150 mL or greater, in whom point estimates favoured endovascular thrombectomy, particularly in the early time window (0-6 h), but wide 95% CIs limited interpretation. INTERPRETATION: Endovascular thrombectomy was associated with improved functional outcomes and reduced mortality versus medical management in patients with large-core ischaemic stroke presenting within 24 h of onset. With the exception of very extensive ischaemic changes (core volume ≥150 mL) presenting beyond 6 h, where evidence remains limited, benefit was sustained across ASPECTS and ischaemic core strata for patients presenting up to 24 h after onset. FUNDING: None. CI - Copyright © 2026 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies. FAU - Sarraj, Amrou AU - Sarraj A AD - Department of Neurology, University Hospitals Cleveland Medical Center, Cleveland, OH, USA; Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. Electronic address: amrou.sarraj@uhhospitals.org. FAU - Thomalla, Götz AU - Thomalla G AD - Klinik und Poliklinik für Neurologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. FAU - Yoshimura, Shinichi AU - Yoshimura S AD - Department of Neurosurgery, Hyogo Medical University, Nishinomiya, Japan. FAU - Huo, Xiaochuan AU - Huo X AD - Neurological Disease Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China. FAU - Costalat, Vincent AU - Costalat V AD - Department of Neuroradiology, Hôpital Gui de Chauliac, Montpellier, France. FAU - Zaidat, Osama O AU - Zaidat OO AD - Neuroscience Institute, Bon Secours Mercy Hospital, Toledo, OH, USA. FAU - Arquizan, Caroline AU - Arquizan C AD - Department of Neurology, Hôpital Gui de Chauliac, Montpellier, France; Institute of Psychiatry and Neuroscience of Paris, INSERM U1266, Team Turc, Paris, France. FAU - Johns, Hannah AU - Johns H AD - Department of Medicine, University of Melbourne, Melbourne, VIC, Australia. FAU - Yogendrakumar, Vignan AU - Yogendrakumar V AD - Division of Neurology, Ottawa Hospital Research Institute, University of Ottawa, Ottawa, ON, Canada; Department of Medicine and Neurology, Melbourne Brain Centre at The Royal Melbourne Hospital, University of Melbourne, Melbourne, VIC, Australia. FAU - Churilov, Leonid AU - Churilov L AD - Department of Medicine, University of Melbourne, Melbourne, VIC, Australia. FAU - Pujara, Deep AU - Pujara D AD - Department of Neurology, University Hospitals Cleveland Medical Center, Cleveland, OH, USA. FAU - Sitton, Clark W AU - Sitton CW AD - Department of Neuroradiology, McGovern Medical School, Houston, TX, USA. FAU - Liebeskind, David S AU - Liebeskind DS AD - Departments of Neurology, Neurological Surgery & Radiology, University of Southern California, Los Angeles, CA, USA. FAU - Inoue, Manabu AU - Inoue M AD - Division of Stroke Care Unit, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan. FAU - Majoie, Charles AU - Majoie C AD - Department of Radiology and Nuclear Medicine, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, Netherlands. FAU - Beenen, Ludo AU - Beenen L AD - Department of Radiology and Nuclear Medicine, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, Netherlands. FAU - Abdulrazzak, Mohammad Ammar AU - Abdulrazzak MA AD - Cerebrovascular Center, Cleveland Clinic, Cleveland, OH, USA. FAU - Chaturvedi, Aditya AU - Chaturvedi A AD - Department of Medicine, University of Melbourne, Melbourne, VIC, Australia. FAU - Singh, Sarthak AU - Singh S AD - Department of Medicine, University of Melbourne, Melbourne, VIC, Australia. FAU - Parsons, Mark W AU - Parsons MW AD - Department of Neurology, Liverpool Hospital, University of New South Wales School of Clinical Medicine, Ingham Institute of Applied Medical Research, Sydney, NSW, Australia. FAU - Denis, Angelique AU - Denis A AD - Service de Biostatistique, Hospices Civils de Lyon, Lyon, France; Université Claude Bernard Lyon 1, Laboratoire de Biométrie et Biologie Evolutive, UMR 5558, Centre National de Recherche Scientifique, VetAgroSup, Villeurbanne, France. FAU - Sakai, Nobuyuki AU - Sakai N AD - Department of Neurosurgery, Seijinkai Shimizu Hospital, Kyoto, Japan. FAU - Nguyen, Thanh N AU - Nguyen TN AD - Departments of Neurology and Radiology, Boston Medical Center, Boston, MA, USA. FAU - Albucher, Jean François AU - Albucher JF AD - Department of Neurology, Hôpital Pierre Paul Riquet, Toulouse, France. FAU - Kasab, Sami Al AU - Kasab SA AD - Department of Neurology and Neurosurgery, Medical University of South Carolina, Charleston, SC, USA. FAU - Hassan, Ameer E AU - Hassan AE AD - Department of Neurology, University of Texas Rio Grande Valley, Harlingen, TX, USA. FAU - Abraham, Michael AU - Abraham M AD - Department of Neurology and Radiology, University of Kansas Medical Center, Kansas City, KS, USA. FAU - Rai, Ansar AU - Rai A AD - Department of Neuroradiology, West Virginia University, Morgantown, WV, USA. FAU - Henon, Hilde AU - Henon H AD - Department of Neurology, Hôpital Salengro, Lille, France. FAU - Sun, Dapeng AU - Sun D AD - Interventional Neuroradiology, Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; China National Clinical Research Center for Neurological Diseases, Beijing, China. FAU - Yamagami, Hiroshi AU - Yamagami H AD - Division of Stroke Prevention and Treatment, Institute of Medicine, University of Tsukuba, Tsukuba, Japan. FAU - Subtil, Fabien AU - Subtil F AD - Service de Biostatistique, Hospices Civils de Lyon, Lyon, France; Université Claude Bernard Lyon 1, Laboratoire de Biométrie et Biologie Evolutive, UMR 5558, Centre National de Recherche Scientifique, VetAgroSup, Villeurbanne, France. FAU - Bonekamp, Susanne AU - Bonekamp S AD - Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany. FAU - Uchida, Kazutaka AU - Uchida K AD - Department of Neurosurgery, Hyogo Medical University, Nishinomiya, Japan. FAU - Yuan, Guangxiong AU - Yuan G AD - Department of Emergency, Xiangtan Central Hospital (The Affiliated Hospital of Hunan University), Xiangtan, China. FAU - Gory, Benjamin AU - Gory B AD - Department of Diagnostic and Therapeutic Neuroradiology, CHRU-Nancy, Nancy, France; Université de Lorraine, INSERM, IADI U1254, Nancy, France. FAU - Smith, Wade AU - Smith W AD - Department of Neurology, University of California-San Francisco, San Francisco, CA, USA. FAU - Ortega-Gutierrez, Santiago AU - Ortega-Gutierrez S AD - Departments of Neurology, Neurosurgery, and Radiology, University of Iowa, Iowa City, IA, USA. FAU - Blackburn, Spiros AU - Blackburn S AD - Department of Neurosurgery, UTHealth Houston, McGovern Medical School, Houston, TX, USA. FAU - Sheth, Sunil A AU - Sheth SA AD - Department of Neurology, UTHealth Houston, McGovern Medical School, Houston, TX, USA. FAU - Marnat, Gaultier AU - Marnat G AD - Department of Neuroradiology, Hôpital Pellegrin, Bordeaux University Hospital, Bordeaux, France. FAU - Liu, Liping AU - Liu L AD - China National Clinical Research Center for Neurological Diseases, Beijing, China; Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. FAU - Shirakawa, Manabu AU - Shirakawa M AD - Department of Neurosurgery, Hyogo Medical University, Nishinomiya, Japan. FAU - Fiehler, Jens AU - Fiehler J AD - Klinik und Poliklinik für Neuroradiologische Diagnostik und Intervention, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. FAU - Jensen, Märit AU - Jensen M AD - Klinik und Poliklinik für Neurologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. FAU - Shindo, Seigo AU - Shindo S AD - Department of Neurology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan. FAU - Nogueira, Raul G AU - Nogueira RG AD - UPMC Stroke Institute, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. FAU - Grandhi, Ramesh AU - Grandhi R AD - Department of Neurosurgery, University of Utah, Salt Lake City, UT, USA. FAU - Jabbour, Pascal AU - Jabbour P AD - Department of Neurological Surgery, Thomas Jefferson University, Philadelphia, PA, USA. FAU - Hussain, Muhammad Shazam AU - Hussain MS AD - Cerebrovascular Center, Cleveland Clinic, Cleveland, OH, USA. FAU - Dippel, Diederik AU - Dippel D AD - Department of Neurology, Erasmus MC University Medical Center, Rotterdam, Netherlands. FAU - Wang, Yilong AU - Wang Y AD - China National Clinical Research Center for Neurological Diseases, Beijing, China; Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. FAU - Yazawa, Yukako AU - Yazawa Y AD - Department of Stroke Neurology, Kohnan Hospital, Sendai, Japan. FAU - Simonsen, Claus Z AU - Simonsen CZ AD - Department of Neurology, Aarhus University Hospital, Aarhus, Denmark. FAU - Wang, Yongjun AU - Wang Y AD - China National Clinical Research Center for Neurological Diseases, Beijing, China; Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China. FAU - Chen, Michael AU - Chen M AD - Department of Neurological Surgery, Rush University Medical Center, Chicago, IL, USA. FAU - Hill, Michael D AU - Hill MD AD - Department of Clinical Neuroscience, University of Calgary, Calgary, AB, Canada. FAU - Grotta, James C AU - Grotta JC AD - Mobile Stroke Unit, Memorial Hermann Hospital, Houston, TX, USA. FAU - Lapergue, Bertrand AU - Lapergue B AD - Department of Neurology, Hôpital Foch, Suresnes, France. FAU - Jovin, Tudor G AU - Jovin TG AD - Cooper Neurological Institute and Cooper Medical School of Rowan University, Camden, NJ, USA. FAU - Yoo, Albert J AU - Yoo AJ AD - California Neurointerventional Surgeons, HCA Healthcare, Riverside, CA, USA. FAU - Bendszus, Martin AU - Bendszus M AD - Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany. FAU - Campbell, Bruce C V AU - Campbell BCV AD - Department of Medicine and Neurology, Melbourne Brain Centre at The Royal Melbourne Hospital, University of Melbourne, Melbourne, VIC, Australia; Florey Institute for Neuroscience and Mental Health, Melbourne, VIC, Australia. FAU - Morimoto, Takeshi AU - Morimoto T AD - Department of Data Science, Hyogo Medical University, Nishinomiya, Japan. FAU - Miao, Zhongrong AU - Miao Z AD - Interventional Neuroradiology, Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; China National Clinical Research Center for Neurological Diseases, Beijing, China. CN - ATLAS Investigators LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20260507 PL - England TA - Lancet JT - Lancet (London, England) JID - 2985213R SB - IM MH - Humans MH - *Endovascular Procedures/adverse effects/methods MH - *Ischemic Stroke/complications/drug therapy/mortality/surgery MH - Randomized Controlled Trials as Topic MH - *Thrombectomy/methods/adverse effects MH - Time Factors MH - Treatment Outcome MH - Follow-Up Studies MH - *Intracranial Hemorrhages/epidemiology/etiology/prevention & control COIS- Declaration of interests AJY declares grants and contracts paid to their institution from Medtronic, Cerenovus, Penumbra, Stryker, Genentech, Balt, and Route 92; consulting fees from Penumbra, Vesalio, and NIH/NINDS; participation on a data safety and monitoring board or advisory board with Route 92 and HCA Neurovascular Research Advisory Board; and stock or stock options in Insera Therapeutics, Galaxy Medical, Gravity Medical, and Nicolab. AEH declares consulting fees from Medtronic, Microvention, Stryker, Penumbra, J&J MedTech, Genentech, GE Healthcare, Scientia, Balt, Viz.ai, Insera therapeutics, Proximie, NeuroVasc, NovaSignal, Vesalio, Rapid Medical, Imperative Care, Galaxy Therapeutics, Route 92, Perfuze, CorTech, Imago, Shockwave, Toro, NeuroVasX, Xcath, Kaneka, Plaga, Project Neu, Piraeus, BioPhiliQ, Arbor Medical, Medinol, Navigantis, and Magnisity; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Medtronic, Microvention, Stryker, Penumbra, J&J MedTech, Genentech, GE Healthcare, Scientia, Balt, Viz.ai, Insera therapeutics, Proximie, NeuroVasc, NovaSignal, Vesalio, Rapid Medical, Imperative Care, Galaxy Therapeutics, Route 92, Perfuze, CorTech, Imago, Shockwave, Toro, NeuroVasX, Xcath, Kaneka, Plaga, Project Neu, Piraeus, BioPhiliQ, Arbor Medical, Medinol, Navigantis, and Magnisity; participation on a data safety and monitoring board for the COMAND trial and FOCUS trial; and being Past President of the Society of Vascular and Interventional Neurology. AS declares grant support as a global principal investigator (PI) for SELECT LATE (Patient-Centered Outcomes Research Institute; paid to their institution) and as a PI for SELECT, SELECT2, and Stryker Neurovascular (paid to their institution). AR declares consulting fees from Stryker NV, Cerenovous, Route 92; and participation on a data safety and monitoring board for ATHENA and TORNADO. BG declares grant support from the French Ministry of Health as a PI for TITAN, DIRECT ANGIO, and IA-SUCCESS; consulting fees from Air Liquide, MIVI, Medtronic, Microvention, Perfuze, and Penumbra; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim, Acticor Biotech, Medtronic, and Penumbra. BL declares grants or contracts from Balt, Microvention, and Phenox; and consulting fees from Olea Medical. CA declares consulting fees from Medtronic; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Medtronic. CM declares grants or contracts from the European Union, CVON/Dutch Heart Foundation, Boehringer Ingelheim, and Stryker (paid to their institution); and stock or stock options from Nicolab (minority interest). CZS declares grants or contracts from the Health Research Foundation of Central Denmark Region (research grant from hospital); and participation on a data safety monitoring board or advisory board for EAST-STROKE. DSL declares consulting fees for imaging core laboratory work from Johnson & Johnson and Rapid Medical. DD declares participation on a data and safety monitoring committee for ACT Global, and, in the past, data and safety monitoring committee for TESLA; and role as Vice Chair of the data access committee of CONTRAST consortium. GM declares consulting fees from Stryker, Terumo Neuro, and Sim & Cure; payment for lectures for Penumbra, Medtronic, Wallaby Phenox, Cerenovus (Johnson & Johnson), and Bracco; and participation on an advisory board for the MAESTRO study. GT declares (related to the current manuscript) receipt of funding for the TENSION trial from the EU Horizon 2020 Research and Innovation Programme under grant agreement number 754640; consulting fees from Acandis, Bayer, Boehringer Ingelheim, and TarGED; payment or honoraria from Acandis, Boehringer Ingelheim, and Lilly; and role as Chair of the Board of Directors of the European Stroke Organisation. HY declares payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Daiichi-Sankyo, Otsuka Pharmaceutical, Stryker, Boston Scientific, Abbott Medical (lecture fee, personal). JCG declares research grants from Genentech (supplies investigational product); consulting fees from Frazer (consulting on mobile stroke unit); payment for expert testimony from various law firms (legal malpractice cases) three times in 2026; support for attending meetings and/or travel from Perosphere (consulting meeting); participation as chair of a data safety monitoring board for Prolong Pharma. JF declares, for the current project, role as managing director of EppData (completed imaging core laboratory work for TENSION trial); consulting fees (personal) from Medtronic, Stryker, Microvention/Terumo, Cerenovus/JuJ Medtech, Acandis, Phenox, and Penumbra; support for attending meetings and/or travel from Microport; participation on a data safety monitoring board or advisory board for Stryker and Phenox; role as Past President of ESMINT; and stock or stock options for Eppdata and Vastrax. MJ declares consulting fees for advisory boards for BMS and Bayer. MB declares grants from EU Horizon 2020 for the TENSION trial, the EU for the VALIDATE trial, and the DFG for SFB project C02; payment or honoraria for educational talks for Novartis and Boehringer Ingelheim; and role as Editor in Chief for Clinical Neuroradiology (Springer). MC declares consulting fees from RapidPulse and Route 92; payment for expert testimony from various law firms; participation on a data safety monitoring board or advisory board for STEM; and role as Editor of Journal of NeuroInterventional Surgery. MDH declares grants or contracts from NoNO (grant to the University of Calgary for the ESCAPE-NA1 trial, ESCAPE NEXT trial), Canadian Institutes for Health Research (grant to the University of Calgary for the ESCAPE-NA1 trial, ESCAPE NEXT trial, and ACT-GLOBAL trial), Medtronic (grant to the University of Calgary for the ESCAPE-MeVO study), and Boehringer Ingelheim (grant to the University of Calgary for ACT-GLOBAL trials); consulting fees from Sun Pharma (for adjudication of clinical trials outcomes); patents planned, issued, or pending (US Patent 62/086,077, licensed to Circle NVI; and US Patent 10 916,346, licensed to Circle NVI); participation as data and safety monitoring committee chair for ONCOVIR Hiltonel trial, DUMAS trial, EMAGINE trial, EVERLAST trial, ILLUMINATE trial, and POTENTIAL trial; participation as data safety monitoring board member for the ARTESIA trial, BRAIN-AF trial, and LAOOS trial; role as President of the Canadian Neurological Sciences Federation (not for profit); stock or stock options in Circle Inc and Basking Biosciences (private stock or stock options). RG declares consulting fees from Medtronic, Balt, Cerenovus, Kaneka, and Stryker Neurovascular. SY declares payment or honoraria for lectures from Daiichi Sankyo, Bristol Myers Squibb, Otsuka Pharmaceutical, Stryker, Medtronic, Johnson & Johnson, Kaneka Medics, Terumo, Medicos Hirata, Nxera Pharma, and Eisai. SAS declares consulting fees (personal) from Penumbra, Route 92, Perfuze, Balt, and Kaneka; support for attending meetings and/or travel from the American Heart Association and the German Neuroradiology Society; and participation on a data safety monitoring board or advisory board for Vesalio (clinical events committee participation). SBo declares funding for the present manuscript from EU Horizon 2020 and EU Horizon 2.1. TM declares other financial interests, outside the submitted work, in Abbott, Boehringer Ingelheim, Bristol Myers Squibb, Daiichi Sankyo, Eisai, GlaxoSmithKline, Integra LifeSciences, Pfizer, UCB, Japan Lifeline. TGJ declares grants or contracts from NIH (STEP), PCORI, and Stryker; consulting fees from Medtronic, Johnson & Johnson, and Contego Medical; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Medtronic; participation on a data safety monitoring board or advisory board for Johnson and Johnson; and stock or stock options in Route 92, Viz.ai, Methinks, Gravity, Galaxy, Anaconda, Kandu, FreeOx Biotech, and Vastrax. WS declares participation on a data safety monitoring board for the PICASSO Trial, SEGA Trial, TESLA Trial, TESSERACT-2 Trial, and role as safety adjudicator for the SISTER trial. RGN reports research grants from Cerenovus and Stryker Neurovascular to the University of Pittsburgh; role as a principal investigator of the “Endovascular Therapy for Low NIHSS Ischemic Strokes (ENDOLOW)” trial (funding provided by Cerenovus); role as a principal investigator of the “Combined Thrombectomy for Distal MediUm Vessel Occlusion StroKe (DUSK)” trial (funding provided by Stryker Neurovascular); consulting fees for advisory roles with Anaconda, Biogen, Boehringer Ingelheim, Bristol Myers Squibb, J&J, Cerenovus, Genentech, Philips, Hybernia, Hyperfine, Imperative Care, Medtronic, Merck, Phenox, Prolong Pharmaceuticals, Stryker Neurovascular, Shanghai Wallaby, Synchron, Takeda Pharmaceutical; stock options for advisory roles with Astrocyte, Brainomix, Cerebrotech, Ceretrieve, Corindus Vascular Robotics, CrestecBio, Euphrates Vascular, Vesalio, Viz.ai, RapidPulse, Sensome, and Perfuze; and being an investor in Viz.ai, Perfuze, Cerebrotech, Reist/Q’Apel Medical, Truvic, Tulavi Therapeutics, Vastrax, Piraeus Medical, Brain4Care, Quantanosis AI, Unity Medical, and Viseon. SOG reports research grants from NIH/NINDS, PCORI, Stryker, Medtronic, and Methinks; consulting fees from Stryker and Medtronic; payment or honoraria from Stryker and Medtronic; appointment as president of the Society of Vascular and Interventional Neurology; and stock options and equity in Motif, Gravity, and Clotter. TNN reports consulting fees as an advisor for Bayer, Route 92, and Medtronic; and appointment as Associate Editor for Stroke. All other authors declare no competing interests. FIR - Yoshimura, Shinichi IR - Yoshimura S FIR - Sakai, Nobuyuki IR - Sakai N FIR - Yamagami, Hiroshi IR - Yamagami H FIR - Morimoto, Takeshi IR - Morimoto T FIR - Toyoda, Kazunori IR - Toyoda K FIR - Matsumaru, Yuji IR - Matsumaru Y FIR - Matsumoto, Yasushi IR - Matsumoto Y FIR - Kimura, Kazumi IR - Kimura K FIR - Kuwayama, Naoya IR - Kuwayama N FIR - Ogasawara, Kuniaki IR - Ogasawara K FIR - Hirano, Teruyuki IR - Hirano T FIR - Ishikura, Reiichi IR - Ishikura R FIR - Inoue, Manabu IR - Inoue M FIR - Sakakibara, Fumihiro IR - Sakakibara F FIR - Kinjo, Norito IR - Kinjo N FIR - Saito, Takuya IR - Saito T FIR - Uchida, Kazutaka IR - Uchida K FIR - Beppu, Mikiya IR - Beppu M FIR - Uchio, Akito IR - Uchio A FIR - Takeuchi, Masataka IR - Takeuchi M FIR - Matsumoto, Yasushi IR - Matsumoto Y FIR - Kimura, Naoto IR - Kimura N FIR - Shigeta, Keigo IR - Shigeta K FIR - Shindo, Seigo IR - Shindo S FIR - Imamura, Hirotoshi IR - Imamura H FIR - Suzuki, Ichiro IR - Suzuki I FIR - Enomoto, Yukiko IR - 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Yassi N FIR - Pavlin-Premrl, Davor IR - Pavlin-Premrl D FIR - Beharry, James IR - Beharry J FIR - Balabanski, Anna IR - Balabanski A FIR - Santos, Angela Dos IR - Santos AD FIR - Sharobeam, Angelos IR - Sharobeam A FIR - Valente, Michael IR - Valente M FIR - Park, Ashley IR - Park A FIR - Wong, Joseph IR - Wong J FIR - McDonald, Amy IR - McDonald A FIR - Jackson, David IR - Jackson D FIR - Kleinig, Timothy J IR - Kleinig TJ FIR - Scroop, Rebecca IR - Scroop R FIR - Kurunawai, Craig IR - Kurunawai C FIR - Mahadevan, Joshua IR - Mahadevan J FIR - Goh, Rudy IR - Goh R FIR - Drew, Roy IR - Drew R FIR - Cordato, Dennis IR - Cordato D FIR - Miller, Megan IR - Miller M FIR - Manning, Nathan IR - Manning N FIR - Parsons, Mark IR - Parsons M FIR - McQuinn, Alex IR - McQuinn A FIR - Wenderoth, Jason IR - Wenderoth J FIR - Cheung, Andrew IR - Cheung A FIR - McDougall, Alan IR - McDougall A FIR - Thomas, James IR - Thomas J FIR - Cappelen-Smith, Cecilia IR - Cappelen-Smith C FIR - Blair, Christopher IR - Blair C FIR - Edwards, Leon IR - Edwards L FIR - Williams, Cameron IR - Williams C FIR - Whitely, Justin IR - Whitely J FIR - Helou, Jacob IR - Helou J FIR - Wu, Teddy Y IR - Wu TY FIR - Collecutt, Wayne IR - Collecutt W FIR - Colgan, Frances IR - Colgan F FIR - Krauss, Martin IR - Krauss M FIR - Laing, Andrew IR - Laing A FIR - Wilson, Duncan IR - Wilson D FIR - Fink, John IR - Fink J FIR - LeHeron, Campbell IR - LeHeron C FIR - Mason, Deborah IR - Mason D FIR - Green, Rosemary IR - Green R FIR - Bremner, Kathleen IR - Bremner K FIR - Ribo, Marc IR - Ribo M FIR - Tomasello, Alejandro IR - Tomasello A FIR - Requena, Manuel IR - Requena M FIR - Gadea, Marta Olive IR - Gadea MO FIR - Sanjuan, Estela IR - Sanjuan E FIR - Cendrero, Judith IR - Cendrero J FIR - Blasco, Jordi IR - Blasco J FIR - Renu, Arturo IR - Renu A FIR - Roman, Luis San IR - Roman LS FIR - Urra, Xabier IR - Urra X FIR - Santana, Daniel IR - Santana D FIR - Cabrera, Jose Maria IR - Cabrera JM FIR - Llull, Laura IR - Llull L FIR - López-Rueda, Antonio IR - López-Rueda A FIR - Cabero, Andrea IR - Cabero A FIR - Serrano, Elena IR - Serrano E FIR - Perez de la Ossa, Natalia IR - Perez de la Ossa N FIR - Hernández-Pérez, Maria IR - Hernández-Pérez M FIR - Millán, Mònica IR - Millán M FIR - Gomis, Meritxell IR - Gomis M FIR - Bustamante, Alejandro IR - Bustamante A FIR - Dorado, Laura IR - Dorado L FIR - Remollo, Sebastià IR - Remollo S FIR - Castaño, Carlos IR - Castaño C FIR - Werner, Mariano IR - Werner M FIR - Boix, Martí IR - Boix M FIR - Muñoz-Narbona, Lucia IR - Muñoz-Narbona L FIR - Cardona, Pere IR - Cardona P FIR - Quesada, Helena IR - Quesada H FIR - Lara, Blanca IR - Lara B FIR - Paipa, Andres IR - Paipa A FIR - Nuñez, Anna IR - Nuñez A FIR - Cuba, Victor IR - Cuba V FIR - Chirife, Oscar IR - Chirife O FIR - Aja, Lucia IR - Aja L FIR - Angeles de Miquel, Maria IR - Angeles de Miquel M FIR - Arenillas, Juan F IR - Arenillas JF FIR - De Lera Alfonso, Mercedes IR - De Lera Alfonso M FIR - Esther Ramos Araque, Maria IR - Esther Ramos Araque M FIR - Calleja, Ana I IR - Calleja AI FIR - Cortijo, Elisa IR - Cortijo E FIR - Martínez-Galdámez, Mario IR - Martínez-Galdámez M FIR - Schüller, Miguel IR - Schüller M FIR - Galván, Jorge IR - Galván J FIR - Muñoz, Javier Reyes IR - Muñoz JR FIR - Vicente, Beatriz Gómez IR - Vicente BG FIR - Psychogios, Marios IR - Psychogios M FIR - Brehm, Alex IR - Brehm A FIR - Albers, Gregory W IR - Albers GW FIR - Pereira, Vitor Mendes IR - Pereira VM FIR - Wechsler, Lawrence IR - Wechsler L FIR - Davis, Stephen IR - Davis S FIR - Nguyen, Thanh IR - Nguyen T FIR - Fifi, Johanna IR - Fifi J FIR - Hamilton, Steve IR - Hamilton S FIR - Hill, Michael IR - Hill M FIR - Churilov, Leonid IR - Churilov L FIR - Lavori, Philip IR - Lavori P FIR - Cai, Chunyan IR - Cai C FIR - Johns, Hannah IR - Johns H FIR - Sitton, Clark W IR - Sitton CW FIR - Olenko, Liudmyla IR - Olenko L FIR - Riascos, Roy IR - Riascos R FIR - Sharma, Gagan IR - Sharma G FIR - Ng, Felix IR - Ng F FIR - Yogendrakumar, Vignan IR - Yogendrakumar V FIR - Guha, Prodipta IR - Guha P FIR - Rokaha, Birendra IR - Rokaha B FIR - Chaturvedi, Aditya IR - Chaturvedi A FIR - Singh, Sarthak IR - Singh S FIR - Dhimal, Niruta IR - Dhimal N FIR - Rahbar, Mohammad Hossain IR - Rahbar MH FIR - Hessabi, Manouchehr IR - Hessabi M FIR - Tahanan, Amirali IR - Tahanan A FIR - Bomben, Valerie IR - Bomben V FIR - Blanks, Abigail IR - Blanks A FIR - Pohlman, McKenzie IR - Pohlman M FIR - Sutek, Broc IR - Sutek B FIR - Aamodt, Anne Hege IR - Aamodt AH FIR - Adamczewski, Olaf IR - Adamczewski O FIR - Alektoror, Kirill IR - Alektoror K FIR - Alexander, März IR - Alexander M FIR - Alexandrou, Maria IR - Alexandrou M FIR - Alias, Quentin IR - Alias Q FIR - Al-Kuzae, Fadha Elawi IR - Al-Kuzae FE FIR - Allard, Julien IR - Allard J FIR - Al-Schameri, Rahman IR - Al-Schameri R FIR - Álvarez, Alberto IR - Álvarez A FIR - Andersen, Grethe IR - Andersen G FIR - AnkerlundBlaufeldt, Rolf IR - AnkerlundBlaufeldt R FIR - Antje, Riedel IR - Antje R FIR - Appelbohm, Hannes IR - Appelbohm H FIR - Argren, Maria IR - Argren M FIR - Assmann, Anne IR - Assmann A FIR - Augustin, Michael IR - Augustin M FIR - Bach, Elke IR - Bach E FIR - Bar, Michal IR - Bar M FIR - Barleben, Maria IR - Barleben M FIR - Baronnet, Flore IR - Baronnet F FIR - Barrios, Andrés IR - Barrios A FIR - Bavúzová, Xénia IR - Bavúzová X FIR - BayThomsen, Rikke IR - BayThomsen R FIR - Becker, Sandra IR - Becker S FIR - Beer, Sylvia IR - Beer S FIR - Behme, Daniel IR - Behme D FIR - Bellut, Maximilian IR - Bellut M FIR - Bendszus, Martin IR - Bendszus M FIR - Berkefeld, Joachim IR - Berkefeld J FIR - Bester, Maximillian IR - Bester M FIR - Bode, Felix J IR - Bode FJ FIR - Boehme, Christian IR - Boehme C FIR - Boese, Ramona IR - Boese R FIR - Bohmann, Ferdinand IR - Bohmann F FIR - Bonekamp, Susanne IR - Bonekamp S FIR - Borggrefe, Jan IR - Borggrefe J FIR - Boss, Erendira Gabriela IR - Boss EG FIR - Boutchakova, Maria IR - Boutchakova M FIR - Boxhammer, Elfi IR - Boxhammer E FIR - Brandhofe, Annemarie IR - Brandhofe A FIR - Breckwoldt, Michael IR - Breckwoldt M FIR - Brekenfeld, Casper IR - Brekenfeld C FIR - Brehm, Alex IR - Brehm A FIR - Brem, Christian IR - Brem C FIR - Breuer, Stella IR - Breuer S FIR - Breyer, Tobias IR - Breyer T FIR - Brodová, Petra IR - Brodová P FIR - Broocks, Gabriel IR - Broocks G FIR - Brosinski, Christoph IR - Brosinski C FIR - Bubel, Nele IR - Bubel N FIR - Búřil, Jiří IR - Búřil J FIR - Čábal, Martin IR - Čábal M FIR - Casado, Laura IR - Casado L FIR - Celis, Elena de IR - Celis E FIR - Chabert, Emmanuel IR - Chabert E FIR - Charisse, Daniel IR - Charisse D FIR - Cheng, Bastian IR - Cheng B FIR - Chovanec, Vendelín IR - Chovanec V FIR - Cidlinsky, Peter IR - Cidlinsky P FIR - Cisár, Juraj IR - Cisár J FIR - Clarençon, Fréderic IR - Clarençon F FIR - Crozier, Sophie IR - Crozier S FIR - Čurdová, Nina IR - Čurdová N FIR - Damaskinos, Michele IR - Damaskinos M FIR - Damgaard, Dorte IR - Damgaard D FIR - Daniš, Martin IR - Daniš M FIR - Dazinger, Florian IR - Dazinger F FIR - Deb-Chatterji, Milani IR - Deb-Chatterji M FIR - Delalic, Asima IR - Delalic A FIR - Delekta, Agnieszka IR - Delekta A FIR - Delorme, Stephen IR - Delorme S FIR - Deutschmann, Hannes IR - Deutschmann H FIR - Diamandis, Elie IR - Diamandis E FIR - Diedrichsen, Tove IR - Diedrichsen T FIR - Doležalová, Irena IR - Doležalová I FIR - Dorn, Franziska IR - Dorn F FIR - Dorn, Franziska IR - Dorn F FIR - du Mesnil de Rochemont, Richard IR - du Mesnil de Rochemont R FIR - DupontHougaard, Kristina IR - DupontHougaard K FIR - Ebrahimi, Taraneh IR - Ebrahimi T FIR - Eff, Florian IR - Eff F FIR - Eliášová, Ilona IR - Eliášová I FIR - Enriquez, Brian IR - Enriquez B FIR - Ergawy, Mostafa IR - Ergawy M FIR - Essig, Fabian IR - Essig F FIR - Falkesgaard, Maiken IR - Falkesgaard M FIR - Fandler-Höfler, Simon IR - Fandler-Höfler S FIR - Fernández, Andrés IR - Fernández A FIR - Ferré, Jean-Christophe IR - Ferré JC FIR - Ferrier, Anna IR - Ferrier A FIR - Fiehler, Jens IR - Fiehler J FIR - Figlewski, Krystian IR - Figlewski K FIR - Fischer, Sebastian IR - Fischer S FIR - Fischer, Urs IR - Fischer U FIR - Flottmann, Fabian IR - Flottmann F FIR - Forbrig, Robert IR - Forbrig R FIR - Förch, Christian IR - Förch C FIR - Fromm, Annette IR - Fromm A FIR - Fuentes, Blanca IR - Fuentes B FIR - Gaedke, Ines IR - Gaedke I FIR - Galczak, Romana IR - Galczak R FIR - Galijasevic, Malik IR - Galijasevic M FIR - Ganser, Bernhard IR - Ganser B FIR - Gattringer, Thomas IR - Gattringer T FIR - Gawlitza, Matthias IR - Gawlitza M FIR - Gelhard, Sarah IR - Gelhard S FIR - Gellißen, Susanne IR - Gellißen S FIR - Gerber, Johannes IR - Gerber J FIR - Giannakakis, Michail Panagiotis IR - Giannakakis MP FIR - Gindlhuber, Karin IR - Gindlhuber K FIR - Gizewski, Elke R IR - Gizewski ER FIR - Glodny, Bernhard IR - Glodny B FIR - Godel, Tim IR - Godel T FIR - Goebell, Einar IR - Goebell E FIR - Goldemund, David IR - Goldemund D FIR - Görtler, Michael IR - Görtler M FIR - Goyal, Mayank IR - Goyal M FIR - Grams, Astrid E IR - Grams AE FIR - Gruber, Joachim IR - Gruber J FIR - Gruber, Katharina IR - Gruber K FIR - Günthner-Lengsfeld, Thomas IR - Günthner-Lengsfeld T FIR - Haase, Kathrin IR - Haase K FIR - Hacker-Ivan, Floriana IR - Hacker-Ivan F FIR - Hallerstig, Erika IR - Hallerstig E FIR - Hametner, Christian IR - Hametner C FIR - Hanning, Uta IR - Hanning U FIR - Haring, Jozef IR - Haring J FIR - Haršány, Michal IR - Haršány M FIR - Haršány, Ján IR - Haršány J FIR - Hartmann, Christian IR - Hartmann C FIR - Hassler, Eva Maria IR - Hassler EM FIR - Hauptmann, Kristina IR - Hauptmann K FIR - Haeusler, Karl Georg IR - Haeusler KG FIR - Hecker, Constantin IR - Hecker C FIR - Hellstern, Victoria IR - Hellstern V FIR - Henkes, Hans IR - Henkes H FIR - Hernández, Victoria IR - Hernández V FIR - Herweh, Christian IR - Herweh C FIR - Hilgenfeld, Tim IR - Hilgenfeld T FIR - Hill, Michael D IR - Hill MD FIR - Hjort, Niels IR - Hjort N FIR - HjortJensen, Nina IR - HjortJensen N FIR - Hoelter, Maya IR - Hoelter M FIR - Hoffmann, Karl-Titus IR - Hoffmann KT FIR - Holst, Brigitte IR - Holst B FIR - Holtmannspoetter, Markus IR - Holtmannspoetter M FIR - Hopf-Jensen, Silke IR - Hopf-Jensen S FIR - Hoppe, Julia IR - Hoppe J FIR - Horner, Susanne IR - Horner S FIR - HougaardSoerensen, Leif IR - HougaardSoerensen L FIR - Hua, Vi Tuan IR - Hua VT FIR - Hubert, Alexander IR - Hubert A FIR - Hurtíková, Eva IR - Hurtíková E FIR - Jakubíček, Stanislava IR - Jakubíček S FIR - Janjic, Tanja IR - Janjic T FIR - Jaramillo, Kirsten IR - Jaramillo K FIR - Jedlitschka, Angela IR - Jedlitschka A FIR - Jensen, Schiela IR - Jensen S FIR - Jensen, Märit IR - Jensen M FIR - Jesser, Jessica IR - Jesser J FIR - Jestaedt, Leonie IR - Jestaedt L FIR - Johnson, Sabine IR - Johnson S FIR - Jonszta, Tomáš IR - Jonszta T FIR - Kalmar, Peter IR - Kalmar P FIR - Karabegovic, Sanja IR - Karabegovic S FIR - Karen, Kollo IR - Karen K FIR - Kastrup, Andreas IR - Kastrup A FIR - Katja, Hopp IR - Katja H FIR - Keeba, Natalia IR - Keeba N FIR - Keese, Petra IR - Keese P FIR - Kefaloykos, Christina IR - Kefaloykos C FIR - Keil, Fee IR - Keil F FIR - Kellert, Lars IR - Kellert L FIR - Kellinghaus, Christoph IR - Kellinghaus C FIR - Kestner, Roxane-Isabelle IR - Kestner RI FIR - Kiechl, Stefan IR - Kiechl S FIR - Killer-Oberpfalzer, Monika IR - Killer-Oberpfalzer M FIR - Klepanec, Andrej IR - Klepanec A FIR - Knispel, Casjupea IR - Knispel C FIR - Knoflach, Michael IR - Knoflach M FIR - Kohler, Sabine IR - Kohler S FIR - Kohlhase, Konstantin IR - Kohlhase K FIR - Kollikowski, Alexander Marco IR - Kollikowski AM FIR - Kovář, Martin IR - Kovář M FIR - Krajina, Antonín IR - Krajina A FIR - Kral, Michael IR - Kral M FIR - Krastev, Georgi IR - Krastev G FIR - Krause, Lars Udo IR - Krause LU FIR - Kreidenhuber, Rudolf IR - Kreidenhuber R FIR - Křivka, Tomáš IR - Křivka T FIR - Krkoška, Adam IR - Krkoška A FIR - Kröger, Jan Robert IR - Kröger JR FIR - Kronlage, Moritz IR - Kronlage M FIR - Krukowski, Pawel IR - Krukowski P FIR - Kühn, Julia IR - Kühn J FIR - Kurča, Egon IR - Kurča E FIR - Kurka, Natalia IR - Kurka N FIR - Kuschnerow, Michael IR - Kuschnerow M FIR - Lachmund, Rita IR - Lachmund R FIR - Lamprecht, Susanne IR - Lamprecht S FIR - Lange, Rüdiger IR - Lange R FIR - Lauer, Monika IR - Lauer M FIR - Leciñana, Alonso de IR - Leciñana A FIR - Leder, Sara IR - Leder S FIR - Leger, Anne IR - Leger A FIR - Lehnen, Nils IR - Lehnen N FIR - Lehrieder, Dominik IR - Lehrieder D FIR - Leißner, Maximilian IR - Leißner M FIR - Leitinger, Markus IR - Leitinger M FIR - Leitner, Ursula IR - Leitner U FIR - Lenck, Stéphanie IR - Lenck S FIR - Lenzenweger, Eva IR - Lenzenweger E FIR - Liebig, Thomas IR - Liebig T FIR - Lowens, Stephan IR - Lowens S FIR - Lunzer, Manuel IR - Lunzer M FIR - Maegerlein, Christian IR - Maegerlein C FIR - Magyar, Marton IR - Magyar M FIR - Marques, Leonardo IR - Marques L FIR - Matyáš, David IR - Matyáš D FIR - Maurer, Gabriele IR - Maurer G FIR - Mauritz, Matthias IR - Mauritz M FIR - Maximilian, Thormann IR - Maximilian T FIR - Mayer-Süß, Lukas IR - Mayer-Süß L FIR - Meckel, Stephan IR - Meckel S FIR - Medek, Oldřich IR - Medek O FIR - Meissner, Julius N IR - Meissner JN FIR - Mencl, Pavel IR - Mencl P FIR - Merkle, Andrea IR - Merkle A FIR - Mesche, Birte IR - Mesche B FIR - Michalski, Dominik IR - Michalski D FIR - Mikulík, Robert IR - Mikulík R FIR - Modrau, Boris IR - Modrau B FIR - Möhlenbruch, Markus A IR - Möhlenbruch MA FIR - Mohr, Alexander IR - Mohr A FIR - Mönninghoff, Christoph IR - Mönninghoff C FIR - Moser, Tobias IR - Moser T FIR - Mücke, Ramona IR - Mücke R FIR - Müller-Hülsbeck, Stefan IR - Müller-Hülsbeck S FIR - Müller-Thies-Broussalis, Erasmia IR - Müller-Thies-Broussalis E FIR - Mutzenbach, Sebastian IR - Mutzenbach S FIR - Navia, Pedro IR - Navia P FIR - Neuberger, Ulf IR - Neuberger U FIR - Neugebauer, Hermann IR - Neugebauer H FIR - Neumann, Jens IR - Neumann J FIR - Nguyên, Anh IR - Nguyên A FIR - Niederkorn, Kurt IR - Niederkorn K FIR - Nosál', Vladimír IR - Nosál' V FIR - Novobilský, Richard IR - Novobilský R FIR - Ntoulias, Nikos IR - Ntoulias N FIR - Nussbaum, Lukas IR - Nussbaum L FIR - Oder, Joanna IR - Oder J FIR - Oldag, Andreas IR - Oldag A FIR - Ondrejkovič, Marián IR - Ondrejkovič M FIR - Otto, Ferdinand IR - Otto F FIR - Otto, Dagmar IR - Otto D FIR - Paech, Daniel IR - Paech D FIR - Pagano, Paolo IR - Pagano P FIR - Pallesen, Lars-Peder IR - Pallesen LP FIR - Panský, Michal IR - Panský M FIR - Papanagiotou, Panagiotis IR - Papanagiotou P FIR - Patrick, Samp IR - Patrick S FIR - Paukisch, Harald IR - Paukisch H FIR - Pelz, Johann IR - Pelz J FIR - Petersen, Inga IR - Petersen I FIR - Petersen, Martina IR - Petersen M FIR - Petzold, Gabor C IR - Petzold GC FIR - Pfaff, Johannes IR - Pfaff J FIR - Pfaff, Johannes IR - Pfaff J FIR - Pfeilschifter, Waltraud IR - Pfeilschifter W FIR - Pham, Mirko IR - Pham M FIR - Pichler, Alexander IR - Pichler A FIR - Pierot, Laurent IR - Pierot L FIR - Pikija, Slaven IR - Pikija S FIR - PlougmannPovlsen, Jan IR - PlougmannPovlsen J FIR - Polkowski, Christoph IR - Polkowski C FIR - Polomac, Nenad IR - Polomac N FIR - Portugaller, Rupert Horst IR - Portugaller RH FIR - Poulsen, Marika IR - Poulsen M FIR - Preiß, Michael IR - Preiß M FIR - Prestsæter, Sjur IR - Prestsæter S FIR - Prieto-Pérez, Rocio IR - Prieto-Pérez R FIR - Psychogios, Marios IR - Psychogios M FIR - Puetz, Volker IR - Puetz V FIR - Purrucker, Jan IR - Purrucker J FIR - Rai, Heike IR - Rai H FIR - Rauch, Maximilian IR - Rauch M FIR - Raupach, Jan IR - Raupach J FIR - Reimann, Gernot IR - Reimann G FIR - Reimann, Georg IR - Reimann G FIR - Reitz, Sarah IR - Reitz S FIR - Renc, Ondřej IR - Renc O FIR - Retzlaff, Jasmin IR - Retzlaff J FIR - Rigual, Ricardo IR - Rigual R FIR - Ringleb, Peter Arthur IR - Ringleb PA FIR - Rivera-Bengoa, Carlota IR - Rivera-Bengoa C FIR - Rodríguez, Jorge IR - Rodríguez J FIR - Rohde, Stefan IR - Rohde S FIR - Rohler, Siegfried IR - Rohler S FIR - Rosso, Charlotte IR - Rosso C FIR - Roth, Christian IR - Roth C FIR - Röttcher, Thomas IR - Röttcher T FIR - Roubec, Martin IR - Roubec M FIR - Roztočilová, Milada IR - Roztočilová M FIR - Rudnicka, Svetlana IR - Rudnicka S FIR - Ruiz, Gerardo IR - Ruiz G FIR - Ryan, Stephen IR - Ryan S FIR - Ryckborst, Karla J IR - Ryckborst KJ FIR - Sandvik, Simen IR - Sandvik S FIR - Schäfer, Jan-Hendrik IR - Schäfer JH FIR - Schaller-Paule, Martin IR - Schaller-Paule M FIR - Schell, Maximillian IR - Schell M FIR - Schellinger, Peter IR - Schellinger P FIR - Schlemm, Eckhard IR - Schlemm E FIR - Schmid, Florian IR - Schmid F FIR - Schmidt, Christoph IR - Schmidt C FIR - Schmitz, Marie Louise IR - Schmitz ML FIR - Schneider, Claus IR - Schneider C FIR - Scholtz, Jan-Erik IR - Scholtz JE FIR - Schönenberger, Silvia IR - Schönenberger S FIR - Schröter, Andreas IR - Schröter A FIR - Schwarz, Daniel IR - Schwarz D FIR - Schwarz, Stephan IR - Schwarz S FIR - Schwarzenhofer, Daniel IR - Schwarzenhofer D FIR - Seifert-Held, Thomas IR - Seifert-Held T FIR - Seiler, Alexander IR - Seiler A FIR - Seker, Fatih IR - Seker F FIR - Shotar, Eimad IR - Shotar E FIR - Simonsen, Claus Z IR - Simonsen CZ FIR - Simonsen, Maria Theresa IR - Simonsen MT FIR - Sivák, Jozef IR - Sivák J FIR - Skagen, Karolina IR - Skagen K FIR - Skjelland, Mona IR - Skjelland M FIR - Šnajdrová, Alena IR - Šnajdrová A FIR - Solymosi, Lazlo IR - Solymosi L FIR - Sømark, Jesper IR - Sømark J FIR - Sonnberger, Michael IR - Sonnberger M FIR - Soršák, Jakub IR - Soršák J FIR - Sourour, Nader IR - Sourour N FIR - Søyland, Jogrim IR - Søyland J FIR - Spitzer, Daniel IR - Spitzer D FIR - Sporns, Peter IR - Sporns P FIR - Städt, Michael IR - Städt M FIR - Steidl, Eike IR - Steidl E FIR - Størdal, Anne Margrethe Kaalaas IR - Størdal AMK FIR - Stösser, Sebastian IR - Stösser S FIR - Strickmann, Sarah IR - Strickmann S FIR - Strýček, Ondřej IR - Strýček O FIR - Suškevič, Igor IR - Suškevič I FIR - Sýkora, Jan IR - Sýkora J FIR - Tennøe, Bjørn IR - Tennøe B FIR - Thaler, Daniela IR - Thaler D FIR - Theisen, Sara IR - Theisen S FIR - Thomalla, Götz IR - Thomalla G FIR - Trendafilov, Petar IR - Trendafilov P FIR - Trenkler, Johannes IR - Trenkler J FIR - Trumm, Christoph IR - Trumm C FIR - Tsogkas, Ioannis IR - Tsogkas I FIR - Tunold, Jon-Anders IR - Tunold JA FIR - Tveit, Lars IR - Tveit L FIR - Ulfert, Christian IR - Ulfert C FIR - Vališ, Kateřina IR - Vališ K FIR - Vaníček, Jiří IR - Vaníček J FIR - Vassilev, Christine IR - Vassilev C FIR - Vítková, Eva IR - Vítková E FIR - Voit-Höhne, Heinz-Leonhard IR - Voit-Höhne HL FIR - Vojtíšek, Bohuslav IR - Vojtíšek B FIR - Volderauer, Karoline IR - Volderauer K FIR - Vollherbst, Dominik IR - Vollherbst D FIR - Vollmuth, Christoph IR - Vollmuth C FIR - Volna, Kamila IR - Volna K FIR - Volný, Ondřej IR - Volný O FIR - VonWeitzel-Mudersbach, Poul IR - VonWeitzel-Mudersbach P FIR - Vorčák, Martin IR - Vorčák M FIR - Wagner, Marlies IR - Wagner M FIR - Wathle, Gaute Kjellevold IR - Wathle GK FIR - Weber, Werner IR - Weber W FIR - Weber, Anushe IR - Weber A FIR - Weiss, Viktor IR - Weiss V FIR - Weller, Johannes M IR - Weller JM FIR - Wenger-Alakmeh, Katharina IR - Wenger-Alakmeh K FIR - Weyland, Cyrill IR - Weyland C FIR - Weymayr, Friedrich IR - Weymayr F FIR - Wießpeiner, Ulrike IR - Wießpeiner U FIR - Willeit, Johannes IR - Willeit J FIR - Wittwer, Aymeric IR - Wittwer A FIR - Wollenweber, Frank IR - Wollenweber F FIR - Wortmann, Ginette IR - Wortmann G FIR - Wunderlich, Silke IR - Wunderlich S FIR - Xiong, Yanyan IR - Xiong Y FIR - You, Se-Jong IR - You SJ FIR - ZachoSpeiser, Lasse IR - ZachoSpeiser L FIR - Zamani, Mahtab IR - Zamani M FIR - Zelenak, Kamil IR - Zelenak K FIR - Zeleňáková, Jana IR - Zeleňáková J FIR - Zubel, Seraphine IR - Zubel S FIR - Ayrignac, Xavier IR - Ayrignac X FIR - Charif, Mahmoud IR - Charif M FIR - Corti, Lucas IR - Corti L FIR - Dargazanli, Cyril IR - Dargazanli C FIR - Derraz, Imad IR - Derraz I FIR - Gaillard, Nicolas IR - Gaillard N FIR - Gascou, Grégory IR - Gascou G FIR - Lefevre, Pierre-Henri IR - Lefevre PH FIR - Lippi, Anaïs IR - Lippi A FIR - Mourand, Isabelle IR - Mourand I FIR - Prin, Pauline IR - Prin P FIR - Schmitt, Perrine IR - Schmitt P FIR - Savenier, Florian IR - Savenier F FIR - Schiphorst, Adrien Ter IR - Schiphorst AT FIR - Arquizan, Caroline IR - Arquizan C FIR - Costalat, Vincent IR - Costalat V FIR - Briau, Pierre IR - Briau P FIR - Gariel, Florent IR - Gariel F FIR - Liegey, Jean-Sébastien IR - Liegey JS FIR - Milnerowicz, Malgorzata IR - Milnerowicz M FIR - Papaxanthos, Jean IR - Papaxanthos J FIR - Papillon, Lisa IR - Papillon L FIR - Sibon, Igor IR - Sibon I FIR - Marnat, Gaultier IR - Marnat G FIR - Corfu, Alice IR - Corfu A FIR - Girot, Jean-Baptiste IR - Girot JB FIR - L'allinec, Vincent IR - L'allinec V FIR - Lecluse, Alderic IR - Lecluse A FIR - Godard, Sophie IR - Godard S FIR - Pasco-Papon, Anne IR - Pasco-Papon A FIR - Beydoun, Nadeem IR - Beydoun N FIR - Boucebci, Samy IR - Boucebci S FIR - Dumas, Victor IR - Dumas V FIR - Bin Khunayfir, Cédric IR - Bin Khunayfir C FIR - Neau, Jean-Philippe IR - Neau JP FIR - Palazzo, Paola IR - Palazzo P FIR - Raynaud, Nicolas IR - Raynaud N FIR - Lamy, Matthias IR - Lamy M FIR - Velasco, Stéphane IR - Velasco S FIR - Aghetti, Agnès IR - Aghetti A FIR - Dimitrovic, Anna IR - Dimitrovic A FIR - Guedon, Alexis IR - Guedon A FIR - Reiner, Peggy IR - Reiner P FIR - Houdart, Emmanuel IR - Houdart E FIR - Mazighi, Mikael IR - Mazighi M FIR - Benhassen, Wagih IR - Benhassen W FIR - Kerleroux, Basile IR - Kerleroux B FIR - Rodriguez Regent, Christine IR - Rodriguez Regent C FIR - Naggara, Olivier IR - Naggara O FIR - Turc, Guillaume IR - Turc G FIR - Allard, Julien IR - Allard J FIR - Baronnet, Flore IR - Baronnet F FIR - Crozier, Sophie IR - Crozier S FIR - Delorme, Stephen IR - Delorme S FIR - Deltour, Sandrine IR - Deltour S FIR - Gerschenfeld, Gaspard IR - Gerschenfeld G FIR - Leger, Anne IR - Leger A FIR - Premat, Kevin IR - Premat K FIR - Rosso, Charlotte IR - Rosso C FIR - Samson, Yves IR - Samson Y FIR - Shotar, Eimad IR - Shotar E FIR - Alamowitch, Sonia IR - Alamowitch S FIR - Spelle, Laurent IR - Spelle L FIR - Chalumeau, Vanessa IR - Chalumeau V FIR - Chassin, Olivier IR - Chassin O FIR - Gallas, Sophie IR - Gallas S FIR - Ikka, Léon IR - Ikka L FIR - Legris, Nicolas IR - Legris N FIR - Sarov Riviere, Mariana IR - Sarov Riviere M FIR - Venditti, Laura IR - Venditti L FIR - Denier, Christian IR - Denier C FIR - Anxionnat, René IR - Anxionnat R FIR - Bonnerot, Mathieu IR - Bonnerot M FIR - Liao, Liang IR - Liao L FIR - Riou-Comte, Nolwenn IR - Riou-Comte N FIR - Gory, Benjamin IR - Gory B FIR - Richard, Sébastien IR - Richard S FIR - Alexandre, Pierre-Louis IR - Alexandre PL FIR - Daumasduport, Benjamin IR - Daumasduport B FIR - Desal, Hubert IR - Desal H FIR - Detraz, Lili IR - Detraz L FIR - Preterre, Cécile IR - Preterre C FIR - Bourcier, Romain IR - Bourcier R FIR - Guillon, Benoît IR - Guillon B FIR - Wolff, Valérie IR - Wolff V FIR - Pop, Raoul IR - Pop R FIR - Baptiste, Laura IR - Baptiste L FIR - Comby, Pierre-Olivier IR - Comby PO FIR - Lemogne, Brivael IR - Lemogne B FIR - Thouant, Pierre IR - Thouant P FIR - Bejot, Yannick IR - Bejot Y FIR - Ricolfi, Frédéric IR - Ricolfi F FIR - Merrien, François-Mathias IR - Merrien FM FIR - Nico, Lorena IR - Nico L FIR - Ognard, Julien IR - Ognard J FIR - Simoni, Laurina IR - Simoni L FIR - Gentric, Jean-Christophe IR - Gentric JC FIR - Timsit, Serge IR - Timsit S FIR - Triquenot Bagan, Aude IR - Triquenot Bagan A FIR - Papagiannaki, Chrysanthi IR - Papagiannaki C FIR - Ozkul, Ozlem IR - Ozkul O FIR - Seners, Pierre IR - Seners P FIR - Piotin, Michel IR - Piotin M FIR - Obadia, Michael IR - Obadia M FIR - Bodenant, Marie IR - Bodenant M FIR - Bricout, Nicolas IR - Bricout N FIR - Casolla, Barbara IR - Casolla B FIR - Dequatre, Nelly IR - Dequatre N FIR - Della Shiava, Lucie IR - Della Shiava L FIR - Pasi, Maco IR - Pasi M FIR - Caparros, François IR - Caparros F FIR - Puy, Laurent IR - Puy L FIR - Karam, Arnaud IR - Karam A FIR - Kaaouana, Olfa IR - Kaaouana O FIR - Watel, Kaelig IR - Watel K FIR - Scopelliti, Giuseppe IR - Scopelliti G FIR - Nouri, Nasreddine IR - Nouri N FIR - Henon, Hilde IR - Henon H FIR - Bibi, Richard IR - Bibi R FIR - Bretonniere, Arnaud IR - Bretonniere A FIR - Gaudron, Marie IR - Gaudron M FIR - Herbreteau, Denis IR - Herbreteau D FIR - Ifergan, Héloise IR - Ifergan H FIR - Jannot, Kevin IR - Jannot K FIR - Molinier, Elisabeth IR - Molinier E FIR - Pasi, Maco IR - Pasi M FIR - Annan, Mariam IR - Annan M FIR - Boulouis, Grégoire IR - Boulouis G FIR - Wiener, Emmanuel IR - Wiener E FIR - Arnoux Courselle, Audrey IR - Arnoux Courselle A FIR - Canaple, Sandrine IR - Canaple S FIR - Ouin, Elisa IR - Ouin E FIR - Chivot, Cyril IR - Chivot C FIR - Lamy, Chantal IR - Lamy C FIR - Saleme, Suzana IR - Saleme S FIR - Mounayer, Charbel IR - Mounayer C FIR - Macian Montoro, Francisco IR - Macian Montoro F FIR - Laubacher, Morgane IR - Laubacher M FIR - Riva, Roberto IR - Riva R FIR - Eker, Omer IR - Eker O FIR - Cho, Tae-Hee IR - Cho TH FIR - Calviere, Lionel IR - Calviere L FIR - Fontaine, Louis IR - Fontaine L FIR - Olivot, Jean-Marc IR - Olivot JM FIR - Raposo, Nicolas IR - Raposo N FIR - Viguier, Alain IR - Viguier A FIR - Cognard, Christophe IR - Cognard C FIR - Albucher, Jean-François IR - Albucher JF FIR - Doche, Emilie IR - Doche E FIR - Laksiri, Nadia IR - Laksiri N FIR - Machado, Stephanie IR - Machado S FIR - Osman, Ophelie IR - Osman O FIR - Peres, Roxane IR - Peres R FIR - Robinet, Emanuelle IR - Robinet E FIR - Rey, Caroline IR - Rey C FIR - Suissa, Laurent IR - Suissa L FIR - Brunel, Hervé IR - Brunel H FIR - Maria, Federico DI IR - Maria FD FIR - Lapergue, Bertrand IR - Lapergue B FIR - Lteif, Elie IR - Lteif E FIR - Chabert, Emmanuel IR - Chabert E FIR - Ferrier, Anna IR - Ferrier A FIR - Blanca, Lara IR - Blanca L FIR - Quesada, Helena IR - Quesada H FIR - Núñez, Ana IR - Núñez A FIR - Cardona, Pere IR - Cardona P FIR - Bustamante, Alejandro IR - Bustamante A FIR - Dorado Bouix, Laura IR - Dorado Bouix L FIR - Calib, Christopher IR - Calib C FIR - Carbonell, Jaime IR - Carbonell J FIR - Eugenia, Gema IR - Eugenia G FIR - Amor, García IR - Amor G FIR - Ezcurra, Garbiñe IR - Ezcurra G FIR - Flores, Belén IR - Flores B FIR - Gómez Choco, Manuel IR - Gómez Choco M FIR - Gomis Cortina, Meritxell IR - Gomis Cortina M FIR - Hernández, Marina IR - Hernández M FIR - Larrañaga, Clara IR - Larrañaga C FIR - Domínguez Lizarbe, Manuel IR - Domínguez Lizarbe M FIR - Martínez, Marina IR - Martínez M FIR - Menéndez Albarracín, Alex IR - Menéndez Albarracín A FIR - Rabaneda, Neus IR - Rabaneda N FIR - Ramos Pachón, Anna IR - Ramos Pachón A FIR - Alejandro Rodríguez, Luis IR - Alejandro Rodríguez L FIR - Rubio, Sara IR - Rubio S FIR - Puig, Isabel IR - Puig I FIR - Santana L, Daniel IR - Santana L D FIR - Valls, Adrian IR - Valls A FIR - Yugueros, Barbara IR - Yugueros B FIR - Wenger, Denisse IR - Wenger D FIR - Millán, Mònica IR - Millán M FIR - Amaro, Sergio IR - Amaro S FIR - Balasa, Mircea IR - Balasa M FIR - Maria Cabrera, Jose IR - Maria Cabrera J FIR - Chamorro, Ángel IR - Chamorro Á FIR - Conde, Estefania IR - Conde E FIR - Miguel Contador, Jose IR - Miguel Contador J FIR - Doncel-Mariano, Antonio IR - Doncel-Mariano A FIR - Guasp, Mar IR - Guasp M FIR - Llansó, Laura IR - Llansó L FIR - Llull, Laura IR - Llull L FIR - Mayà Casalprim, Gerard IR - Mayà Casalprim G FIR - Montejo, Carmen IR - Montejo C FIR - Rodríguez, Alejandro IR - Rodríguez A FIR - Rudilosso, Salvatore IR - Rudilosso S FIR - Sánchez, Almudena IR - Sánchez A FIR - Santana, Daniel IR - Santana D FIR - Urra Nuin, Xabier IR - Urra Nuin X FIR - Augusto Vera Monge, Víctor IR - Augusto Vera Monge V FIR - De Lera, Mercedes IR - De Lera M FIR - Esther Ramos, María IR - Esther Ramos M FIR - F Arenillas, Juan IR - F Arenillas J FIR - Aguirre, Garazi IR - Aguirre G FIR - Cabral, Laura IR - Cabral L FIR - Díaz, Irene IR - Díaz I FIR - Durán Lozano, Alejandro IR - Durán Lozano A FIR - Luna, Alain IR - Luna A FIR - Martín, Jon IR - Martín J FIR - Moreno Estébanez, Ana IR - Moreno Estébanez A FIR - Sifontes, Walter IR - Sifontes W FIR - Ugarriza, Iratxe IR - Ugarriza I FIR - Del Mar Freijo, María IR - Del Mar Freijo M FIR - Benavente, Lorena IR - Benavente L FIR - Calleja Puerta, Sergio IR - Calleja Puerta S FIR - Castañón Apilánez, María IR - Castañón Apilánez M FIR - Criado, Álvaro IR - Criado Á FIR - Fuentes, David IR - Fuentes D FIR - García-Cabo, Carmen IR - García-Cabo C FIR - González Delgado, Montserrat IR - González Delgado M FIR - María González, Gemma IR - María González G FIR - Molina Gil, Javier IR - Molina Gil J FIR - Larrosa Campo, Davinia IR - Larrosa Campo D FIR - López López, Begoña IR - López López B FIR - Reguera Acuña, Antía IR - Reguera Acuña A FIR - Rico Santos, María IR - Rico Santos M FIR - Zunzunegui, Patricia IR - Zunzunegui P FIR - López-Cancio, Elena IR - López-Cancio E FIR - Vega, Pedro IR - Vega P FIR - VAN Zwam, Wim IR - VAN Zwam W FIR - Chatellier, Gilles IR - Chatellier G FIR - Fisher, Urs IR - Fisher U FIR - Detante, Olivier IR - Detante O FIR - Arteaga, Charles IR - Arteaga C FIR - Pico, Fernando IR - Pico F FIR - Heck, Olivier IR - Heck O FIR - Zapata, Elena IR - Zapata E FIR - Flores, Alan IR - Flores A FIR - Ayo, Oscar IR - Ayo O FIR - Labreuche, Julien IR - Labreuche J FIR - Duhamel, Alain IR - Duhamel A FIR - Chouiki, Hajar IR - Chouiki H FIR - Liebeskind, David S IR - Liebeskind DS FIR - Sanossian, Nerses IR - Sanossian N FIR - Yoo, Albert J IR - Yoo AJ FIR - Zaidat, Osama O IR - Zaidat OO FIR - Al Kasab, Sami IR - Al Kasab S FIR - Sheth, Sunil A IR - Sheth SA FIR - Rai, Ansaar T IR - Rai AT FIR - Ortega-Gutierrez, Santiago IR - Ortega-Gutierrez S FIR - Given, Curtis A IR - Given CA FIR - Zaidi, Syed F IR - Zaidi SF FIR - Grandhi, Ramesh IR - Grandhi R FIR - Cuellar, Hugo IR - Cuellar H FIR - Mokin, Maxim IR - Mokin M FIR - Katz, Jeffrey M IR - Katz JM FIR - Alshekhlee, Amer IR - Alshekhlee A FIR - Taqi, Muhammad A IR - Taqi MA FIR - Ansari, Sameer A IR - Ansari SA FIR - Siddiqui, Adnan H IR - Siddiqui AH FIR - Barazangi, Nobl IR - Barazangi N FIR - English, Joey D IR - English JD FIR - Maud, Alberto IR - Maud A FIR - Kirmani, Jawad IR - Kirmani J FIR - Gupta, Rishi IR - Gupta R FIR - Yavagal, Dileep R IR - Yavagal DR FIR - Tarpley, Jason IR - Tarpley J FIR - Pandya, Dhruvil J IR - Pandya DJ FIR - Cress, Marshall C IR - Cress MC FIR - Dharmadhikari, Sushrut IR - Dharmadhikari S FIR - Asif, Kaiz S IR - Asif KS FIR - Kass-Hout, Tareq IR - Kass-Hout T FIR - Puri, Ajit S IR - Puri AS FIR - Janjua, Nazli IR - Janjua N FIR - Majjhoo, Aniel Q IR - Majjhoo AQ FIR - Badruddin, Aamir IR - Badruddin A FIR - Edgell, Randall C IR - Edgell RC FIR - Khatri, Rakesh IR - Khatri R FIR - Morgan, Larry IR - Morgan L FIR - Razak, Anmar IR - Razak A FIR - Zha, Alicia IR - Zha A FIR - Khandelwal, Priyank IR - Khandelwal P FIR - Mueller-Kronast, Nils IR - Mueller-Kronast N FIR - Rivet, Dennis J IR - Rivet DJ FIR - Wolfe, Thomas IR - Wolfe T FIR - Snelling, Brian IR - Snelling B FIR - Sultan-Qurraie, Ali IR - Sultan-Qurraie A FIR - Lin, Shao-Pow IR - Lin SP FIR - Khangura, Rajkamal IR - Khangura R FIR - Spiotta, Alejandro M IR - Spiotta AM FIR - Bhuva, Parita IR - Bhuva P FIR - Salazar-Marioni, Sergio IR - Salazar-Marioni S FIR - Lin, Eugene IR - Lin E FIR - Tarabishy, Abdul R IR - Tarabishy AR FIR - Samaniego, Edgar A IR - Samaniego EA FIR - Kolikonda, Murali K IR - Kolikonda MK FIR - Jumaa, Mouhammad A IR - Jumaa MA FIR - Reddy, Vivek K IR - Reddy VK FIR - Sharma, Pankaj IR - Sharma P FIR - Berkhemer, Olvert A IR - Berkhemer OA FIR - van Doormaal, Pieter-Jan IR - van Doormaal PJ FIR - van Es, Adriaan C G M IR - van Es ACGM FIR - van Zwam, Wim H IR - van Zwam WH FIR - Emmer, Bart J IR - Emmer BJ FIR - Beenen, Ludo F IR - Beenen LF FIR - Majoie, Charles B L M IR - Majoie CBLM FIR - Buderer, Nancy IR - Buderer N FIR - Detry, Michelle A IR - Detry MA FIR - Bosse, Anna IR - Bosse A FIR - Graves, Todd L IR - Graves TL FIR - Saunders, Christina IR - Saunders C FIR - Elijovich, Lucas IR - Elijovich L FIR - Jadhav, Ashutosh IR - Jadhav A FIR - Brown, Scott IR - Brown S FIR - Nguyen, Thanh N IR - Nguyen TN FIR - Gress, Daryl IR - Gress D FIR - Dippel, Diederik W J IR - Dippel DWJ FIR - Smith, Wade S IR - Smith WS FIR - Olvany, Jasmine M IR - Olvany JM FIR - Ibrahim, Laila A IR - Ibrahim LA FIR - Henricks, Alexia IR - Henricks A FIR - Tariq, Zain IR - Tariq Z FIR - Geraghty, Scott IR - Geraghty S FIR - Hannon, Peter IR - Hannon P FIR - Chung, Lee IR - Chung L FIR - Tekiela, Peter IR - Tekiela P FIR - Hoversten, Knut IR - Hoversten K FIR - DeWitt, Dana IR - DeWitt D FIR - Wold, Jana IR - Wold J FIR - Kilburg, Craig IR - Kilburg C FIR - Taussky, Phil IR - Taussky P FIR - Alexander, Matthew IR - Alexander M FIR - Renner, David IR - Renner D FIR - Chauhan, Muhammed IR - Chauhan M FIR - Ansari, Safdar IR - Ansari S FIR - Mittal, Manoj IR - Mittal M FIR - Barnard, Zachary IR - Barnard Z FIR - Park, Joseph IR - Park J FIR - Park, Yong IR - Park Y FIR - Hughes, Rodger IR - Hughes R FIR - Dhand, Sabeen IR - Dhand S FIR - Oconnor, Paul IR - Oconnor P FIR - Hsu, Mike IR - Hsu M FIR - Darkhabani, Ziad IR - Darkhabani Z FIR - Dandapat, Sudeepta IR - Dandapat S FIR - Shah, Kavit IR - Shah K FIR - Guerrero, Waldo IR - Guerrero W FIR - Thanki, Shail IR - Thanki S FIR - Vakharia, Kunal IR - Vakharia K FIR - Paradise, Scott IR - Paradise S FIR - Cajavilca, Christian IR - Cajavilca C FIR - Hansen, Paul IR - Hansen P FIR - Ajiboye, Norman IR - Ajiboye N FIR - Soomro, Jazba IR - Soomro J FIR - Fares, Mary-Ann IR - Fares MA FIR - Turabova, Charos IR - Turabova C FIR - Levy, Elad IR - Levy E FIR - Davies, Jason IR - Davies J FIR - Snyder, Kenneth IR - Snyder K FIR - Tso, Michael IR - Tso M FIR - Horn, Chris IR - Horn C FIR - Owada, Kumiko IR - Owada K FIR - Sharma, Pankaj IR - Sharma P FIR - Ansari, Junaid IR - Ansari J FIR - DeAlba, Luis IR - DeAlba L FIR - Adeeb, Nimer IR - Adeeb N FIR - Savardekar, Amey IR - Savardekar A FIR - Kelly, Roger IR - Kelly R FIR - Chernyshev, Oleg IR - Chernyshev O FIR - Ansari, Junaid IR - Ansari J FIR - Tan, Benedict IR - Tan B FIR - Bushnaq, Saif IR - Bushnaq S FIR - Ezzledin, Mohamad IR - Ezzledin M FIR - Aleuzi, Bader IR - Aleuzi B FIR - Osman, Mohamed IR - Osman M FIR - Kassen, Mohammad IR - Kassen M FIR - Liaw, Nicholas IR - Liaw N FIR - Khan, Shoeb IR - Khan S FIR - Chaubal, Varun IR - Chaubal V FIR - Hou, Samuel IR - Hou S FIR - Ovando, Renee IR - Ovando R FIR - Teitelbaum, George IR - Teitelbaum G FIR - Putman, Christopher IR - Putman C FIR - Szweda, Kamila IR - Szweda K FIR - Jackson, Robert IR - Jackson R FIR - Arnold, Lisa IR - Arnold L FIR - Nimchinsky, Esther IR - Nimchinsky E FIR - Aroor, Sushanth IR - Aroor S FIR - Bach, Ivo IR - Bach I FIR - Hannawi, Yousef IR - Hannawi Y FIR - Culati, Deepak IR - Culati D FIR - Lakhani, Sushil IR - Lakhani S FIR - Lee, Vivien IR - Lee V FIR - Newman, Ben IR - Newman B FIR - Ramsey, Christian IR - Ramsey C FIR - Villeli, Nicholas IR - Villeli N FIR - Modak, Janhavi M IR - Modak JM FIR - Mazudar, Avi IR - Mazudar A FIR - Shownkeen, Harish IR - Shownkeen H FIR - Panezai, Spozhmy IR - Panezai S FIR - Strauss, Sara IR - Strauss S FIR - Mehta, Siddhart IR - Mehta S FIR - Zacharatos, Haralabos IR - Zacharatos H FIR - Fourcand, Farah IR - Fourcand F FIR - Singh, Amrinder IR - Singh A FIR - Amireh, Abdallah IR - Amireh A FIR - Gadallah, Nancy IR - Gadallah N FIR - Ali, Nasar IR - Ali N FIR - Mansour, Ali IR - Mansour A FIR - Previti, Micahel IR - Previti M FIR - Foreman, Paul IR - Foreman P FIR - Vachhani, Jay IR - Vachhani J FIR - Hirschl, Robert IR - Hirschl R FIR - Peterson, Eric IR - Peterson E FIR - Malik, Amer IR - Malik A FIR - Starke, Robert IR - Starke R FIR - Balousek, Pete IR - Balousek P FIR - Agonafer, Makeda IR - Agonafer M FIR - Wang, Sophia IR - Wang S FIR - Boo, Sohyun IR - Boo S FIR - Tarabishy, Abdul IR - Tarabishy A FIR - Alvi, Muhammad IR - Alvi M FIR - Sukhumaran, Madhav IR - Sukhumaran M FIR - Jahromi, Babak IR - Jahromi B FIR - Cantrell, Donald IR - Cantrell D FIR - Abdalla, Ramez IR - Abdalla R FIR - Potts, Matthew IR - Potts M FIR - Shaibani, Ali IR - Shaibani A FIR - Turabova, Charoskhon IR - Turabova C FIR - Rayes, Mahmoud IR - Rayes M FIR - Naravetla, Bharath IR - Naravetla B FIR - Linares, Guillermo IR - Linares G FIR - Ramiro, Joanna IR - Ramiro J FIR - Gordon, Wes IR - Gordon W FIR - Miremadi, Brian IR - Miremadi B FIR - Kim, Warren IR - Kim W FIR - Settecase, Fabio IR - Settecase F FIR - Spokoyny, Ilana IR - Spokoyny I FIR - Liu, Zuolu IR - Liu Z FIR - Giannakidis, Dimitri IR - Giannakidis D FIR - Kim, Jaehyun IR - Kim J FIR - Gajjar, Ashish IR - Gajjar A FIR - Kim, Song IR - Kim S FIR - Reyes, Dennys IR - Reyes D FIR - Chen, Sherman IR - Chen S FIR - Amit, Sanghvi IR - Amit S FIR - Burgess, Richard IR - Burgess R FIR - Oliver, Marion IR - Oliver M FIR - Ramesh Rao, Rahul IR - Ramesh Rao R FIR - Ming Kung, Vieh IR - Ming Kung V FIR - Hendricks-Jones, Melinda IR - Hendricks-Jones M FIR - Shawver, Julie IR - Shawver J FIR - Hajjar, Monica IR - Hajjar M FIR - Robbins, Sarah IR - Robbins S FIR - Sedlak, Emily IR - Sedlak E FIR - Chen, Penn Roc IR - Chen PR FIR - Dannenbaum, Mark IR - Dannenbaum M FIR - Jones, Welsey IR - Jones W FIR - Blackburn, Spiros IR - Blackburn S FIR - Ezepue, Chizoba IR - Ezepue C FIR - Rodriguez, Gustavo IR - Rodriguez G FIR - Liu, Kenneth IR - Liu K FIR - Harning, Julie IR - Harning J FIR - Dee, Alicia IR - Dee A FIR - Woo, Henry IR - Woo H FIR - Teron Molina, Ina IR - Teron Molina I FIR - Libman, Richard IR - Libman R FIR - Schwartz, Erin IR - Schwartz E FIR - Kapoor, Guarav IR - Kapoor G FIR - Koul, Prateeka IR - Koul P FIR - Link, Thomas IR - Link T FIR - Arora, Rohan IR - Arora R FIR - Ambro, Sandra IR - Ambro S FIR - Kelly, Christina IR - Kelly C FIR - Modaber, Morteza IR - Modaber M FIR - Batanero, Daphne IR - Batanero D FIR - Thomas, Judi IR - Thomas J FIR - Scicutella, Thomas IR - Scicutella T FIR - Vincent, Emily IR - Vincent E FIR - Kaur, Navneet IR - Kaur N FIR - Ballout, Ahmad IR - Ballout A FIR - Reavey-Cantwell, John IR - Reavey-Cantwell J FIR - Samaniego, Edgar IR - Samaniego E FIR - Shim, Hyung-Sub IR - Shim HS FIR - Shaban, Amir IR - Shaban A FIR - Leira, Enrique IR - Leira E FIR - Hayakawa, Minako IR - Hayakawa M FIR - Maljaars, Jason IR - Maljaars J FIR - Ghannam, Malik IR - Ghannam M FIR - Zevallos, Cynthia IR - Zevallos C FIR - Derdeyn, Colin IR - Derdeyn C FIR - Rossen, James IR - Rossen J FIR - Dlouhy, Kathleen IR - Dlouhy K FIR - Roeder, Hannah IR - Roeder H FIR - Lahoti, Sourabh IR - Lahoti S FIR - Hansen, Megan IR - Hansen M FIR - Koul, Prateeka IR - Koul P FIR - Almajali, Mohammad IR - Almajali M FIR - Fain, Nicholas IR - Fain N FIR - Grewal, Parneet IR - Grewal P FIR - Almallouhi, Eyad IR - Almallouhi E FIR - Ozark, Shelly IR - Ozark S FIR - Hegedus, Mary IR - Hegedus M FIR - Dvoren Baker, Tina IR - Dvoren Baker T FIR - Creed, Sarah IR - Creed S FIR - Folsom, Jamie IR - Folsom J FIR - Holmstedt, Christine IR - Holmstedt C FIR - Lena, Jonathan IR - Lena J FIR - Spiotta, Alejandro IR - Spiotta A FIR - Anadani, Mohammad IR - Anadani M FIR - Kicielinski, Kimberly IR - Kicielinski K FIR - Singh, Jasmeet IR - Singh J FIR - DeMacedo Rodrigues, Katyucia IR - DeMacedo Rodrigues K FIR - Marino Granados, Jose IR - Marino Granados J FIR - Kuhn, Anna Luisa IR - Kuhn AL FIR - Massari, Francesco IR - Massari F FIR - Elnazeir, Marna IR - Elnazeir M EDAT- 2026/05/11 00:30 MHDA- 2026/05/22 00:33 CRDT- 2026/05/10 18:52 PHST- 2026/04/06 00:00 [received] PHST- 2026/04/14 00:00 [revised] PHST- 2026/05/01 00:00 [accepted] PHST- 2026/05/22 00:33 [medline] PHST- 2026/05/11 00:30 [pubmed] PHST- 2026/05/10 18:52 [entrez] AID - S0140-6736(26)00876-7 [pii] AID - 10.1016/S0140-6736(26)00876-7 [doi] PST - ppublish SO - Lancet. 2026 May 23;407(10543):2015-2026. doi: 10.1016/S0140-6736(26)00876-7. Epub 2026 May 7.